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Sample records for diseases radiologic-pathologic correlation

  1. From the radiologic pathology archives: diseases of the male breast: radiologic-pathologic correlation.

    PubMed

    Lattin, Grant E; Jesinger, Robert A; Mattu, Rubina; Glassman, Leonard M

    2013-01-01

    Male breast disease includes a variety of benign and malignant conditions, many of which are hormonally influenced. Gynecomastia and skin lesions account for the majority of conditions in symptomatic men with a palpable abnormality, and these conditions should be accurately recognized. Imaging patterns of gynecomastia include nodular, dendritic, and diffuse patterns. Histopathologically, the nodular and dendritic patterns correlate with the florid and quiescent (fibrotic) phases of gynecomastia, respectively. The diffuse pattern may have features of both phases and is associated with exposure to exogenous estrogen. Benign-appearing palpable masses in male patients should be approached cautiously, given the overlapping morphologic features of benign and malignant tumors. In addition to gynecomastia, other benign male breast tumors include lipoma, pseudoangiomatous stromal hyperplasia, granular cell tumor, fibromatosis, myofibroblastoma, schwannoma, and hemangioma. Male breast cancer accounts for 1% of all breast carcinomas. Invasive ductal carcinoma accounts for the majority of cases in adult males and typically appears as a subareolar mass without calcifications that is eccentric to the nipple. Other epithelial and mesenchymal tumors that may occur, albeit not as commonly as in women, include papillary carcinoma, invasive lobular carcinoma, adenoid cystic carcinoma, liposarcoma, dermatofibrosarcoma, pleomorphic hyalinizing angiectatic tumor, basal cell carcinoma of the nipple, hematopoietic malignancies, and secondary tumors. Knowledge of the natural history, clinical characteristics, and imaging features of tumors that occur in the male breast will help narrow the radiologic differential diagnosis and optimize treatment.

  2. From the radiologic pathology archives: pediatric polycystic kidney disease and other ciliopathies: radiologic-pathologic correlation.

    PubMed

    Chung, Ellen M; Conran, Richard M; Schroeder, Jason W; Rohena-Quinquilla, Ivan R; Rooks, Veronica J

    2014-01-01

    Genetic defects of cilia cause a wide range of diseases, collectively known as ciliopathies. Primary, or nonmotile, cilia function as sensory organelles involved in the regulation of cell growth, differentiation, and homeostasis. Cilia are present in nearly every cell in the body and mutations of genes encoding ciliary proteins affect multiple organs, including the kidneys, liver, pancreas, retina, central nervous system (CNS), and skeletal system. Genetic mutations causing ciliary dysfunction result in a large number of heterogeneous phenotypes that can manifest with a variety of overlapping abnormalities in multiple organ systems. Renal manifestations of ciliopathies are the most common abnormalities and include collecting duct dilatation and cyst formation in autosomal recessive polycystic kidney disease (ARPKD), cyst formation anywhere in the nephron in autosomal dominant polycystic kidney disease (ADPKD), and tubulointerstitial fibrosis in nephronophthisis, as well as in several CNS and skeletal malformation syndromes. Hepatic disease is another common manifestation of ciliopathies, ranging from duct dilatation and cyst formation in ARPKD and ADPKD to periportal fibrosis in ARPKD and several malformation syndromes. The unifying molecular pathogenesis of this emerging class of disorders explains the overlap of abnormalities in disparate organ systems and links diseases of widely varied clinical features. It is important for radiologists to be able to recognize the multisystem manifestations of these syndromes, as imaging plays an important role in diagnosis and follow-up of affected patients.

  3. White Matter Diseases with Radiologic-Pathologic Correlation.

    PubMed

    Sarbu, Nicolae; Shih, Robert Y; Jones, Robert V; Horkayne-Szakaly, Iren; Oleaga, Laura; Smirniotopoulos, James G

    2016-01-01

    White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease). Infectious processes include Lyme disease (neuroborreliosis), progressive multifocal leukoencephalopathy, and human immunodeficiency virus (HIV) encephalopathy. Vascular processes include different types of small-vessel disease: arteriolosclerosis, cerebral amyloid angiopathy, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), primary angiitis of the central nervous system, Susac syndrome, and neurolupus. Toxic-metabolic processes include osmotic myelinolysis, methotrexate leukoencephalopathy, and posterior reversible encephalopathy syndrome. The imaging spectrum can vary widely from small multifocal white matter lesions to confluent or extensive white matter involvement. Understanding the pathologic substrate is fundamental for understanding the radiologic manifestations, and a systematic approach to the radiologic findings, in correlation with clinical and laboratory data, is crucial for narrowing the differential diagnosis. (©)RSNA, 2016. PMID:27618323

  4. Neurocysticercosis: radiologic-pathologic correlation.

    PubMed

    Kimura-Hayama, Eric T; Higuera, Jesús A; Corona-Cedillo, Roberto; Chávez-Macías, Laura; Perochena, Anamari; Quiroz-Rojas, Laura Yadira; Rodríguez-Carbajal, Jesús; Criales, José L

    2010-10-01

    Neurocysticercosis is a neurologic parasitic disease caused by the encysted larva of the tapeworm Taenia solium and is the most important parasitic disease of the human central nervous system. It is the most common cause of acquired epilepsy in endemic settings and constitutes a public health challenge for most of the developing world. Nowadays, however, as a result of globalization, neurocysticercosis is being seen more frequently in developed countries as well. Neurocysticercosis is acquired through fecal-oral contamination, and the disease course is complex, with two intermediate hosts (ie, pigs and humans) and a definitive host (humans). Traditionally, it has been classified into active and nonactive forms according to disease location. Radiologists must be aware of its imaging appearance, which is quite variable, as is the differential diagnosis. Imaging findings depend on several factors, including the stage of the life cycle of T solium at presentation; the number and location (ie, subarachnoid, cisternal, or intraventricular) of parasites; and associated complications such as vascular involvement (ie, arteritis with or without infarction), inflammatory response (ie, edema, gliosis, or arachnoiditis), and, in ventricular forms, degree of obstruction. Thus, the diagnostic approach, management, and prognosis for neurocysticercosis differ widely depending on the type of infection. PMID:21071384

  5. A case of interstitial lung disease associated with clinically amyopathic dermatomyositis: radiologic-pathologic correlation.

    PubMed

    Okubo, Gosuke; Noma, Satoshi; Nishimoto, Yuko; Sada, Ryuichi; Kobashi, Yoichiro

    2013-01-01

    This case report describes a 64-year-old woman with interstitial lung disease associated with clinically amyopathic dermatomyositis. Chest computed tomography revealed consolidations along bronchovascular bundles in the periphery of the lower lungs. Interstitial lung disease developed acutely, and the patient died 3 months after the clinical diagnosis. An autopsy was performed, and a large section of the lung specimen was prepared. Various interstitial lesions including organizing pneumonia, cellular and fibrotic nonspecific interstitial pneumonia, and diffuse alveolar damage were seen in the large section. Correlating the large section and computed tomography images was useful for determining the distribution of diffuse alveolar damage.

  6. Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation: From the Radiologic Pathology Archives.

    PubMed

    Kligerman, Seth J; Auerbach, Aaron; Franks, Teri J; Galvin, Jeffrey R

    2016-01-01

    Castleman disease is a complex lymphoproliferative disease pathologically divided into two subtypes, the hyaline vascular variant (HVV) and the plasma cell variant (PCV). The HVV is the most common, is thought to represent a benign neoplasm of lymph node stromal cells, and is treated with surgical resection. It is most commonly found in the mediastinum, where it classically appears as a unicentric, avidly enhancing mass at computed tomography (CT) and magnetic resonance imaging. This appearance can mimic other avidly enhancing mediastinal masses, and location, clinical history, laboratory data, and nuclear medicine single photon emission CT (SPECT) and positron emission tomography (PET) studies can help narrow the differential diagnosis. Multicentric Castleman disease (MCD), which in the majority of cases is composed of the PCV, is an aggressive lymphoproliferative disorder associated with human herpesvirus infection, interleukin 6 dysregulation, and other systemic disorders. While it can be difficult to differentiate MCD from lymphoma, the presence of avidly enhancing lymph nodes can suggest the diagnosis. The purpose of this article is to review the clinical, immunologic, and pathologic findings associated with both unicentric Castleman disease and MCD and discuss how the imaging findings correlate with the pathophysiology of the disease. PMID:27618318

  7. Spinal Metastases of Extramammary Paget Disease with Radiologic-Pathologic Correlation

    PubMed Central

    Little, Jason T; Lehman, Vance T; Morris, Jonathan M; Lehman, Julia S; Diehn, Felix E

    2016-01-01

    Extramammary Paget disease (EMPD) is an uncommon malignancy. It manifests either in the primary form in the skin as an intraepithelial neoplasm, or in secondary form as pagetoid (intraepithelial) spread of an underlying internal carcinoma to the skin. Although local invasion and recurrence of primary extramammary Paget disease are relatively frequent, widespread metastases are rare. As such, there are very few reports and little characterization of the radiologic features of widespread spinal metastases. To our knowledge, there are no prior reports of a metastatic extramammary Paget disease presenting as a painful pathologic vertebral body compression fracture. We report the radiological features of a case of primary extramammary Paget disease with subsequent spinal metastases presenting as a painful compression fracture. PMID:27761174

  8. From the archives of the AFIP: Lung disease in premature neonates: radiologic-pathologic correlation.

    PubMed

    Agrons, Geoffrey A; Courtney, Sherry E; Stocker, J Thomas; Markowitz, Richard I

    2005-01-01

    Pulmonary disease is the most important cause of morbidity in preterm neonates, whose lungs are often physiologically and morphologically immature. Surfactant deficiency in immature lungs triggers a cascade of alveolar instability and collapse, capillary leak edema, and hyaline membrane formation. The term respiratory distress syndrome (RDS) has come to represent the clinical expression of surfactant deficiency and its nonspecific histologic counterpart, hyaline membrane disease. Historically, chest radiographs of infants with RDS predictably demonstrated decreased pulmonary expansion, symmetric generalized reticulogranular lung opacities, and air bronchograms. Refinements in perinatal medicine, including antenatal glucocorticoid administration, surfactant replacement therapy, and increasingly sophisticated ventilatory strategies have decreased the prevalence of RDS and air leak, altered familiar radiographic features, and lowered the threshold of potential viability to a gestational age of approximately 23 weeks. Alveolar paucity and pulmonary interstitial thickness in these profoundly premature neonates impair normal gas exchange and may necessitate prolonged mechanical ventilation, increasing the risk of lung injury. Bronchopulmonary dysplasia (BPD), alternatively termed chronic lung disease of infancy, is a disorder of lung injury and repair originally ascribed to positive-pressure mechanical ventilation and oxygen toxicity. Before the advent of surfactant replacement therapy, chest radiographs of infants with classic BPD demonstrated coarse reticular lung opacities, cystic lucencies, and markedly disordered lung aeration that reflected alternating regions of alveolar septal fibrosis and hyperinflated normal lung parenchyma. In the current era of surfactant replacement, BPD is increasingly a disorder of very low-birth-weight neonates with arrested alveolar and pulmonary vascular development, minimal alveolar septal fibrosis and inflammation, and more subtle

  9. Pulmonary Alveolar Microlithiasis: AIRP Best Cases in Radiologic-Pathologic Correlation.

    PubMed

    Delic, Joseph A; Fuhrman, Carl R; Trejo Bittar, Humberto E

    2016-01-01

    Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP). PMID:27618319

  10. Immunoglobulin G4 -related Sclerosing Mastitis: AIRP Best Cases in Radiologic-Pathologic Correlation.

    PubMed

    Zalaquett, Eugenio; Razmilic, Dravna; Oddo, David

    2016-01-01

    Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP). PMID:27399235

  11. Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation.

    PubMed

    Walton, William J; Flores, Raina R

    2016-01-01

    Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP). PMID:27618327

  12. Benign Multicystic Peritoneal Mesothelioma: AIRP Best Cases in Radiologic-Pathologic Correlation.

    PubMed

    Lee, Ruri; Tong, Angela; Kurtis, Boaz; Gilet, Anthony G

    2016-01-01

    RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP). PMID:26963453

  13. Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation.

    PubMed

    Walton, William J; Flores, Raina R

    2016-01-01

    Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).

  14. Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 1. The 1st Decade: From the Radiologic Pathology Archives.

    PubMed

    Chung, Ellen M; Graeber, Adam R; Conran, Richard M

    2016-01-01

    Wilms tumor is the second most common pediatric solid tumor and by far the most common renal tumor of infants and young children. As most tumors are large at presentation and are treated with nephrectomy, the role of imaging is primarily in preoperative planning and evaluation for metastatic disease. However, with treatment protocols increasingly involving use of preoperative (neoadjuvant) chemotherapy (the standard in Europe) and consideration of nephron-sparing surgery, the role of imaging is evolving to include providing initial disease staging information and a presumptive diagnosis to guide therapy. Differential diagnostic considerations include lesions that are clinically benign and others that require more intensive therapy than is used to treat Wilms tumor. In part 1 of this article, the unique histologic spectrum of renal neoplasms of infants and young children is reviewed with emphasis on radiologic-pathologic correlation. Part 2 will focus on renal tumors of older children and adolescents.

  15. From the radiologic pathology archives: Adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation.

    PubMed

    Lattin, Grant E; Sturgill, Eric D; Tujo, Charles A; Marko, Jamie; Sanchez-Maldonado, Katherine W; Craig, William D; Lack, Ernest E

    2014-01-01

    Advanced imaging often reveals adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients. When adrenal disease is clinically suspected, cross-sectional imaging can be helpful in evaluating the etiology of the patient's symptoms. When adrenal disease is incidentally identified, what the clinician and patient really want to know is whether the findings are benign or malignant, as this ultimately will affect their next step in management. Using radiologic-pathologic correlation, we broadly classify common, uncommon, and rare tumors and tumor-like conditions that can occur in the adrenal as benign or malignant. This classification follows predominant trends in observed biologic behavior while acknowledging those tumors that may behave in the minority in an unpredictable manner. We review the clinical background and presentation of functional adrenal tumors including Conn syndrome, Cushing syndrome, and catecholamine-secreting tumors, as well as their relationship with adrenal anatomy. We discuss a variety of benign tumors, including adrenal cortical adenoma (including oncocytoma) and pheochromocytoma, as well as uncommonly and rarely encountered tumors such as myelolipoma, hemangioma, lymphangioma, schwannoma, ganglioneuroma, and adenomatoid tumor. A variety of tumefactive but nonneoplastic lesions are addressed, including adrenal cortical hyperplasia, adrenal hemorrhage, adrenal cysts, and infections. Malignant tumors discussed include adrenal cortical carcinoma, the rare malignant pheochromocytoma, lymphoma, metastases, and sarcomas. For each tumor and tumor-like lesion, the clinical presentation, epidemiology, key imaging findings, diagnostic differential considerations, and management options are briefly addressed. Finally, an approach to the workup of suspected or incidentally discovered tumors is presented based on a selected literature survey and our clinical experience. Radiologists play an important role in identification and

  16. From the radiologic pathology archives: Adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation.

    PubMed

    Lattin, Grant E; Sturgill, Eric D; Tujo, Charles A; Marko, Jamie; Sanchez-Maldonado, Katherine W; Craig, William D; Lack, Ernest E

    2014-01-01

    Advanced imaging often reveals adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients. When adrenal disease is clinically suspected, cross-sectional imaging can be helpful in evaluating the etiology of the patient's symptoms. When adrenal disease is incidentally identified, what the clinician and patient really want to know is whether the findings are benign or malignant, as this ultimately will affect their next step in management. Using radiologic-pathologic correlation, we broadly classify common, uncommon, and rare tumors and tumor-like conditions that can occur in the adrenal as benign or malignant. This classification follows predominant trends in observed biologic behavior while acknowledging those tumors that may behave in the minority in an unpredictable manner. We review the clinical background and presentation of functional adrenal tumors including Conn syndrome, Cushing syndrome, and catecholamine-secreting tumors, as well as their relationship with adrenal anatomy. We discuss a variety of benign tumors, including adrenal cortical adenoma (including oncocytoma) and pheochromocytoma, as well as uncommonly and rarely encountered tumors such as myelolipoma, hemangioma, lymphangioma, schwannoma, ganglioneuroma, and adenomatoid tumor. A variety of tumefactive but nonneoplastic lesions are addressed, including adrenal cortical hyperplasia, adrenal hemorrhage, adrenal cysts, and infections. Malignant tumors discussed include adrenal cortical carcinoma, the rare malignant pheochromocytoma, lymphoma, metastases, and sarcomas. For each tumor and tumor-like lesion, the clinical presentation, epidemiology, key imaging findings, diagnostic differential considerations, and management options are briefly addressed. Finally, an approach to the workup of suspected or incidentally discovered tumors is presented based on a selected literature survey and our clinical experience. Radiologists play an important role in identification and

  17. From the radiologic pathology archives: precocious puberty: radiologic-pathologic correlation.

    PubMed

    Chung, Ellen M; Biko, David M; Schroeder, Jason W; Cube, Regino; Conran, Richard M

    2012-01-01

    Precocious puberty represents a unique diagnostic problem in which imaging plays an important role. Development of secondary sex characteristics may result from inappropriate activation of the hypothalamic-pituitary axis with release of gonadotropin, or from gonadotropin-independent secretion of sex steroids by the adrenal glands or gonads. A variety of lesions can manifest with precocious puberty, including various central nervous system (CNS) lesions, adrenal lesions, and sex cord-stromal tumors of the testis or ovary. CNS lesions causing precocious puberty are much more common in boys than in girls and are well evaluated with brain magnetic resonance imaging. Neoplastic (hypothalamic-chiasmatic astrocytoma, suprasellar germinoma) and nonneoplastic (hypothalamic hamartoma, hydrocephalus, trauma, empty sella, infection, congenital midline anomalies) conditions may affect the function of the hypothalamic-pituitary axis. The adrenal cortex may produce sex hormones. Some adrenal cortical neoplasms (ACNs) in patients under 5 years of age are related to a mutation of the tumor suppressor gene p53 and represent a disease that is distinct from ACNs in older children and adults. Adrenal cortical hyperplasia secondary to an enzymatic defect in steroid biosynthesis causes virilization and salt wasting, which usually manifest in the neonatal period; however, milder forms of the disease may manifest in childhood. Female precocious puberty may be caused by an autonomously functioning ovarian cyst or a juvenile granulosa cell tumor of the ovary. Male precocious puberty may be caused by a sex steroid-producing Leydig or Sertoli cell tumor of the testis. Ultrasonography is the primary modality for evaluating the sex organs and may also be used to evaluate for adrenal abnormalities.

  18. From the radiologic pathology archives imaging of osteonecrosis: radiologic-pathologic correlation.

    PubMed

    Murphey, Mark D; Foreman, Kristopher L; Klassen-Fischer, Mary K; Fox, Michael G; Chung, Ellen M; Kransdorf, Mark J

    2014-01-01

    Osteonecrosis is common and represents loss of blood supply to a region of bone. Common sites affected include the femoral head, humeral head, knee, femoral/tibial metadiaphysis, scaphoid, lunate, and talus. Symptomatic femoral head osteonecrosis accounts for 10,000-20,000 new cases annually in the United States. In contradistinction, metadiaphyseal osteonecrosis is often occult and asymptomatic. There are numerous causes of osteonecrosis most commonly related to trauma, corticosteroids, and idiopathic. Imaging of osteonecrosis is frequently diagnostic with a serpentine rim of sclerosis on radiographs, photopenia in early disease at bone scintigraphy, and maintained yellow marrow at MR imaging with a serpentine rim of high signal intensity (double-line sign) on images obtained with long repetition time sequences. These radiologic features correspond to the underlying pathology of osseous response to wall off the osteonecrotic process and attempts at repair with vascularized granulation tissue at the reactive interface. The long-term clinical importance of epiphyseal osteonecrosis is almost exclusively based on the likelihood of overlying articular collapse. MR imaging is generally considered the most sensitive and specific imaging modality both for early diagnosis and identifying features that increase the possibility of this complication. Treatment subsequent to articular collapse and development of secondary osteoarthritis typically requires reconstructive surgery. Malignant transformation of osteonecrosis is rare and almost exclusively associated with metadiaphyseal lesions. Imaging features of this dire sequela include aggressive bone destruction about the lesion margin, cortical involvement, and an associated soft-tissue mass. Recognizing the appearance of osteonecrosis, which reflects the underlying pathology, improves radiologic assessment and is important to guide optimal patient management. PMID:25019438

  19. Age-related alterations in the vertebral spinous processes and intervening soft tissues: radiologic-pathologic correlation.

    PubMed

    Sartoris, D J; Resnick, D; Tyson, R; Haghighi, P

    1985-11-01

    A radiologic-pathologic correlative investigation of the normal age-related alterations in the spinous processes and intervening soft tissues was performed using cadaveric spines and both ancient and modern macerated vertebral specimens. Extreme lordosis in the cervical or lumbar spine results in spinous process apposition, formation of interspinous bursae, eburnation with osteophytosis, and creation of synovial articulations. A concomitant degenerative enthesopathy involves the supraspinous or interspinous ligamentous attachments in any spinal segment. The differential diagnosis of this phenomenon, the clinical significance of which in a given patient is controversial, includes rheumatoid and other inflammatory arthritides as well as crystal deposition disease.

  20. Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives.

    PubMed

    Chung, Ellen M; Biko, David M; Arzamendi, Aaron M; Meldrum, Jaren T; Stocker, J Thomas

    2015-01-01

    Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management.

  1. Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives.

    PubMed

    Chung, Ellen M; Biko, David M; Arzamendi, Aaron M; Meldrum, Jaren T; Stocker, J Thomas

    2015-01-01

    Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management. PMID:25763737

  2. Infantile hemangioendothelioma of the liver: a radiologic-pathologic-clinical correlation

    SciTech Connect

    Dachman, A.H.; Lichtenstein, J.E.; Friedman, A.C.; Hartman, D.S.

    1983-06-01

    Infantile hemangioendothelioma is the most common symptomatic vascular liver tumor of infancy. It is considered a benign tumor; however, aggressive behavior is occasionally seen microscopically, and rarely distant metastases have been reported. The exact incidence of infantile hemangioendothelioma is difficult to determine because often it has been either misdiagnosed or mislabeled as cavernous hemangioma in the literature. Cavernous hemangioma is the most common primary liver tumor in older age groups but is rarely found in infants as a clinically significant tumor. Levick and Rubie were the first to recognize an association between hemangioendothelioma of the liver and congestive heart failure, and there were subsequent reports substantiating this association. However, it is our impression and the finding of others that congestive heart failure is distinctly less common than abdominal mass or hepatomegaly as the presenting sign in infantile hemangioendothelioma. Congestive heart failure is rarely a feature of cavernous hemangioma. Because of the errors in terminology and questions regarding clinical presentation, a radiologic-pathologic-clinical correlation study of infantile hemangioendothelioma and review of the literature was undertaken.

  3. Radiologic-pathologic Correlation-An Advanced Fourth-year Elective: How We Do It.

    PubMed

    Hartman, Matthew; Silverman, Jan; Spruill, Laura; Hill, Jeanne

    2016-07-01

    Traditionally, the radiology elective has been designed to teach medical students the fundamentals of radiologic interpretation. When questioned, many students state that they want to take a radiology elective so they can "interpret images." For the students on radiology, rotation/elective education was often passive, consisting of didactic conferences and observational shadowing of radiologists as they interpreted images. Students had only a superficial appreciation of how radiologists interacted with clinical services, multidisciplinary teams, and pathology. There was very little emphasis on imaging appropriateness or the most efficient and effective imaging for various clinical problems. With the expansion of numerous imaging modalities and the emphasis on patient-centered care, including imaging safety and dose reduction, it is important to change the focus of radiology education from interpretation to the optimal integration of imaging into clinical medicine. Radiology-pathology (rad path) electives were created at Allegheny General Hospital and the Medical University of South Carolina as a new option to provide a high-quality advanced elective for fourth-year medical students. These electives enable students to correlate radiologic images with gross and microscopic pathology specimens, thus increasing their knowledge and understanding of both. The rad path elective combines aspects of surgery, radiology, and pathology and requires students to be active learners. The implementation of this elective is an exciting work in progress that has been evolving over the past 2 and 4 years at Medical University of South Carolina and Allegheny General Hospital, respectively. We will discuss the historical basis for the elective, the advantages and challenges of having such an integrated course, and some different strategies for creating a rad path elective.

  4. Radiologic-pathologic Correlation-An Advanced Fourth-year Elective: How We Do It.

    PubMed

    Hartman, Matthew; Silverman, Jan; Spruill, Laura; Hill, Jeanne

    2016-07-01

    Traditionally, the radiology elective has been designed to teach medical students the fundamentals of radiologic interpretation. When questioned, many students state that they want to take a radiology elective so they can "interpret images." For the students on radiology, rotation/elective education was often passive, consisting of didactic conferences and observational shadowing of radiologists as they interpreted images. Students had only a superficial appreciation of how radiologists interacted with clinical services, multidisciplinary teams, and pathology. There was very little emphasis on imaging appropriateness or the most efficient and effective imaging for various clinical problems. With the expansion of numerous imaging modalities and the emphasis on patient-centered care, including imaging safety and dose reduction, it is important to change the focus of radiology education from interpretation to the optimal integration of imaging into clinical medicine. Radiology-pathology (rad path) electives were created at Allegheny General Hospital and the Medical University of South Carolina as a new option to provide a high-quality advanced elective for fourth-year medical students. These electives enable students to correlate radiologic images with gross and microscopic pathology specimens, thus increasing their knowledge and understanding of both. The rad path elective combines aspects of surgery, radiology, and pathology and requires students to be active learners. The implementation of this elective is an exciting work in progress that has been evolving over the past 2 and 4 years at Medical University of South Carolina and Allegheny General Hospital, respectively. We will discuss the historical basis for the elective, the advantages and challenges of having such an integrated course, and some different strategies for creating a rad path elective. PMID:27311804

  5. Bacterial, Fungal, and Parasitic Infections of the Central Nervous System: Radiologic-Pathologic Correlation and Historical Perspectives.

    PubMed

    Shih, Robert Y; Koeller, Kelly K

    2015-01-01

    Despite remarkable progress in prevention and treatment, infectious diseases affecting the central nervous system remain an important source of morbidity and mortality, particularly in less-developed countries and in immunocompromised persons. Bacterial, fungal, and parasitic pathogens are derived from living organisms and affect the brain, spinal cord, or meninges. Infections due to these pathogens are associated with a variety of neuroimaging patterns that can be appreciated at magnetic resonance imaging in most cases. Bacterial infections, most often due to Streptococcus, Haemophilus, and Neisseria species, cause significant meningitis, whereas the less common cerebritis and subsequent abscess formation have well-documented progression, with increasingly prominent altered signal intensity and corresponding contrast enhancement. Atypical bacterial infections are characterized by the development of a granulomatous response, classically seen in tuberculosis, in which the tuberculoma is the most common parenchymal form of the disease; spirochetal and rickettsial diseases are less common. Fungal infections predominate in immunocompromised hosts and are caused by yeasts, molds, and dimorphic fungi. Cryptococcal meningitis is the most common fungal infection, whereas candidiasis is the most common nosocomial infection. Mucormycosis and aspergillosis are characterized by angioinvasiveness and are associated with high morbidity and mortality among immunocompromised patients. In terms of potential exposure in the worldwide population, parasitic infections, including neurocysticercosis, toxoplasmosis, echinococcosis, malaria, and schistosomiasis, are the greatest threat. Rare amebic infections are noteworthy for their extreme virulence and high mortality. The objective of this article is to highlight the characteristic neuroimaging manifestations of bacterial, fungal, and parasitic diseases, with emphasis on radiologic-pathologic correlation and historical perspectives.

  6. Bacterial, Fungal, and Parasitic Infections of the Central Nervous System: Radiologic-Pathologic Correlation and Historical Perspectives.

    PubMed

    Shih, Robert Y; Koeller, Kelly K

    2015-01-01

    Despite remarkable progress in prevention and treatment, infectious diseases affecting the central nervous system remain an important source of morbidity and mortality, particularly in less-developed countries and in immunocompromised persons. Bacterial, fungal, and parasitic pathogens are derived from living organisms and affect the brain, spinal cord, or meninges. Infections due to these pathogens are associated with a variety of neuroimaging patterns that can be appreciated at magnetic resonance imaging in most cases. Bacterial infections, most often due to Streptococcus, Haemophilus, and Neisseria species, cause significant meningitis, whereas the less common cerebritis and subsequent abscess formation have well-documented progression, with increasingly prominent altered signal intensity and corresponding contrast enhancement. Atypical bacterial infections are characterized by the development of a granulomatous response, classically seen in tuberculosis, in which the tuberculoma is the most common parenchymal form of the disease; spirochetal and rickettsial diseases are less common. Fungal infections predominate in immunocompromised hosts and are caused by yeasts, molds, and dimorphic fungi. Cryptococcal meningitis is the most common fungal infection, whereas candidiasis is the most common nosocomial infection. Mucormycosis and aspergillosis are characterized by angioinvasiveness and are associated with high morbidity and mortality among immunocompromised patients. In terms of potential exposure in the worldwide population, parasitic infections, including neurocysticercosis, toxoplasmosis, echinococcosis, malaria, and schistosomiasis, are the greatest threat. Rare amebic infections are noteworthy for their extreme virulence and high mortality. The objective of this article is to highlight the characteristic neuroimaging manifestations of bacterial, fungal, and parasitic diseases, with emphasis on radiologic-pathologic correlation and historical perspectives

  7. Computed tomography of the anterior mediastinum in myasthemia gravis: a radiologic-pathologic correlative study

    SciTech Connect

    Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.

    1982-01-01

    Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented.

  8. Irreversible Electroporation of Hepatic and Pancreatic Malignancies: Radiologic-Pathologic Correlation.

    PubMed

    Gonzalez-Beicos, Aldo; Venkat, Shree; Songrug, Tanakorn; Poveda, Julio; Garcia-Buitrago, Monica; Poozhikunnath Mohan, Prasoon; Narayanan, Govindarajan

    2015-09-01

    Irreversible electroporation (IRE) is a novel therapy that has shown to be a feasible and promising alternative to conventional ablative techniques when treating tumors near vital structures or blood vessels. The clinical efficacy of IRE has been evaluated using established imaging criteria. This study evaluates the histologic and imaging response of hepatic and pancreatic malignancies that were surgically resected after IRE. In total, 12 lesions ablated with IRE were included, including 3 pancreatic carcinomas, 5 primary tumors of the liver, and 4 metastatic tumors of the liver. The rate of complete response to IRE was 25% based on the histologic evaluation of the resected tumors. Although treatment-related vessel wall changes were noted in several cases in histologic findings, there was no evidence of vascular luminal narrowing or obliteration in any of the specimens. The imaging response to IRE before surgical resection usually resulted in underestimation of disease burden when compared with the histologic response seen on the resected specimens.

  9. Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic-pathologic correlation.

    PubMed

    Schieda, N; Kielar, A Z; Al Dandan, O; McInnes, M D F; Flood, T A

    2015-02-01

    Classic (triphasic) renal angiomyolipoma (AML) is currently classified as a neoplasm of perivascular epithelioid cells. For diagnosis of AML, the use of thin-section non-contrast enhanced CT (NECT) improves diagnostic accuracy; however, identifying gross fat within a very small AML is challenging and often better performed with chemical-shift MRI. Although the presence of gross intra-tumoural fat is essentially diagnostic of AML; co-existing intra-tumoural fat and calcification may represent renal cell carcinoma (RCC). Differentiating AML from retroperitoneal sarcoma can be difficult when AML is large; the feeding vessel and claw signs are suggestive imaging findings. AML can haemorrhage, with intra-tumoural aneurysm size >5 mm a more specific predictor of future haemorrhage than tumor size >4 cm. Diagnosis of AML in the setting of acute haemorrhage is complex; comparison studies or follow-up imaging may be required. Not all AML contain gross fat and imaging features of AML without visible fat overlap with RCC; however, homogeneity, hyperdensity at NECT, low T2-weighted signal intensity and, microscopic fat are suggestive features. Patients with tuberous sclerosis often demonstrate a combination of classic and minimal fat AML, but are also at a slightly increased risk for RCC and should be imaged cautiously. Several rare pathological variants of AML exist including AML with epithelial cysts and epithelioid AML, which have distinct imaging characteristics. Classic AML, although benign, can be locally invasive and the rare epithelioid AML can be frankly malignant. The purpose of this review is to highlight the imaging manifestations of 10 uncommon and unusual variants of AML using pathological correlation.

  10. From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation.

    PubMed

    Abbott, Gerald F; Rosado-de-Christenson, Melissa L; Frazier, Aletta Ann; Franks, Teri J; Pugatch, Robert D; Galvin, Jeffrey R

    2005-01-01

    Lymphangioleiomyomatosis (LAM) is an uncommon interstitial lung disease that exclusively affects women, usually during their reproductive years. LAM is characterized pathologically by abnormal proliferation of LAM cells in the lungs and in thoracic and retroperitoneal lymphatics. Thirty-three cases of LAM were reviewed retrospectively for clinical and radiologic findings. Twenty-eight (85%) of 33 women (aged 21-62 years; mean, 37.5 years) were symptomatic. Radiographs (n = 32) demonstrated reticular opacities in 21 (66%) patients, large lung volumes in 17 (53%), pleural effusion in 14 (44%), and pneumothorax in 13 (41%). High-resolution CT (n = 15) and conventional CT (n = 3) showed 2-5-mm bilateral thin-walled cysts in all patients and cysts that were 6-12 mm or larger in patients with severe lung involvement. CT depicted diffuse lung involvement by cysts in nine (50%) patients, relative sparing of lung apices in seven (39%), and relative sparing of lung bases in two (11%). Pleural effusion and pneumothorax were seen at CT in four (22%) and three (17%) patients, respectively. Four cases of tuberous sclerosis complex-associated LAM (TSC-LAM) (women aged 27-50 years; mean, 35.7 years) were similarly reviewed. Three (75%) were symptomatic. Radiographs (n = 4) demonstrated reticular opacities in three (75%) and large lung volumes in two (50%). All high-resolution CT (n = 3) and conventional CT (n = 1) studies showed 2-5-mm bilateral thin-walled cysts and cysts that were 6-12 mm or larger in two patients with severe lung involvement. Pleural effusion and pneumothorax were demonstrated at CT in three (75%) and two (50%) patients, respectively. LAM and TSC-LAM affect symptomatic women who often exhibit reticular opacities and large lung volumes at radiography and bilateral uniform small thin-walled cysts at CT. Large (>12 mm) cysts occur in patients with severe cystic lung involvement. Pneumothorax and pleural effusion are common associated findings.

  11. Primary mixed malignant tumor of bone in an 18-year-old male: Report of a case with radiologic-pathologic correlation

    PubMed Central

    Courtier, Jesse; Robbins, Elizabeth; Soares, Bruno; Horvai, Andrew; Mackenzie, John D.

    2012-01-01

    We report a case of primary malignant mixed tumor (MMT) of bone in an 18-year-old boy with X-ray, CT, MR, scintigraphic, FDG PET, and pathologic correlation. Primary MMT of bone is a highly aggressive tumor and presents both a diagnostic and clinical treatment challenge. This tumor is extremely rare and to the best of our knowledge, this is the first report of the diagnostic imaging findings for primary MMT arising from bone in a patient of this age group. PMID:26909264

  12. Mediastinal Hibernoma: A Rare Case with Radiologic-Pathologic Correlation

    PubMed Central

    Dasyam, Anil; Then, Matthew; Varma, Kavita; Borhani, Amir A.

    2016-01-01

    Hibernomas, especially located in the mediastinum, are extremely rare benign tumors, which are important to consider in the differential diagnosis of a heterogeneously enhancing mass with areas of fat attenuation on imaging of an often incidentally discovered mass. Other common possibilities in the differential include malignant tumors, such as liposarcoma, hence histopathology is usually required to confirm the diagnosis. Hibernomas often follow the distribution of sites of persistence of brown fat in adults, and intrathoracic locations are unusual. We present a very rare case of a mediastinal hibernoma in a 53-year-old woman. She presented to the emergency department with severe, progressive right neck and shoulder pain with radiation down her arm and was found to have a right apical posterior mediastinal mass on imaging. Initial radiographs of the shoulder showed a soft tissue mass within the apical right hemithorax. Further imaging with CT revealed a well circumscribed, heterogeneously enhancing mass with areas of fat attenuation. Pathology confirmed the diagnosis of mediastinal hibernoma, and the mass was completely excised. Fourteen months after surgery, the patient had a normal chest radiograph, and thirty-two months after surgery, she remains asymptomatic. PMID:27651970

  13. Mediastinal Hibernoma: A Rare Case with Radiologic-Pathologic Correlation.

    PubMed

    Darke, Maxine; Dasyam, Anil; Then, Matthew; Varma, Kavita; Borhani, Amir A; Varma, Rakesh

    2016-01-01

    Hibernomas, especially located in the mediastinum, are extremely rare benign tumors, which are important to consider in the differential diagnosis of a heterogeneously enhancing mass with areas of fat attenuation on imaging of an often incidentally discovered mass. Other common possibilities in the differential include malignant tumors, such as liposarcoma, hence histopathology is usually required to confirm the diagnosis. Hibernomas often follow the distribution of sites of persistence of brown fat in adults, and intrathoracic locations are unusual. We present a very rare case of a mediastinal hibernoma in a 53-year-old woman. She presented to the emergency department with severe, progressive right neck and shoulder pain with radiation down her arm and was found to have a right apical posterior mediastinal mass on imaging. Initial radiographs of the shoulder showed a soft tissue mass within the apical right hemithorax. Further imaging with CT revealed a well circumscribed, heterogeneously enhancing mass with areas of fat attenuation. Pathology confirmed the diagnosis of mediastinal hibernoma, and the mass was completely excised. Fourteen months after surgery, the patient had a normal chest radiograph, and thirty-two months after surgery, she remains asymptomatic. PMID:27651970

  14. MR imaging findings of uterine pyomyoma: radiologic-pathologic correlation.

    PubMed

    Ono, Hiromi; Kanematsu, Masayuki; Kato, Hiroki; Toyoki, Hiroshi; Hayasaki, Yoh; Furui, Tatsuro; Morishige, Ken-ichirou; Hatano, Yuichiro

    2014-08-01

    A 69-year-old postmenopausal female with a spontaneously occurring uterine pyomyoma was described with emphasis on the MR imaging findings. On unenhanced T1- and T2-weighted MR images, a huge mottled mass suspected to contain blood products, necrotic tissue, or purulent or viscous fluid was demonstrated within anterior myometrial wall of uterine body. The mass was surrounded by a peripheral rim that was hyperintense on T1-weighted images and hypointense on T2-weighted images. On gadolinium-enhanced MR images, most of the mass was unenhanced, but the peripheral rim was equally enhanced with the surrounding myometrium. Pathological examination revealed an intramural uterine pyomyoma surrounded by fibrous capsules with abundant lymphocytes and neutrophils. Our findings indicate that pyomyoma should be considered when MR images demonstrate a myometrial cystic lesion accompanied by a peripheral rim. PMID:24615512

  15. Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1--Benign Tumors: From the Radiologic Pathology Archives.

    PubMed

    Manning, Maria A; Srivastava, Amogh; Paal, Edina E; Gould, Charles F; Mortele, Koenraad J

    2016-01-01

    Solid and cystic pancreatic neoplasms are being recognized more frequently with increasing utilization and spatial resolution of modern imaging techniques. In addition to the more common primary pancreatic solid (ductal adenocarcinoma) and cystic neoplasms of epithelial origin, nonepithelial neoplasms of the pancreas may appear as well-defined solid or cystic neoplasms. Most of these lesions have characteristic imaging features, such as a well-defined border, which allows differentiation from ductal adenocarcinoma. Solid masses include neurofibroma, ganglioneuroma, leiomyoma, lipoma, and perivascular epithelioid cell tumor (PEComa). Schwannomas and desmoid tumors can be solid or cystic. Cystic tumors include mature cystic teratoma and lymphangioma. Lipoma, PEComa, and mature cystic teratoma can contain fat, and ganglioneuroma and mature cystic teratoma may contain calcification. Although these unusual benign neoplasms are rare, the radiologist should at least consider them in the differential diagnosis of well-defined lesions of the pancreas. The goal of this comprehensive review is to improve understanding of these rare primary pancreatic mesenchymal tumors. PMID:26761535

  16. Review of ovarian tumors in children and adolescents: radiologic-pathologic correlation.

    PubMed

    Heo, Suk Hee; Kim, Jin Woong; Shin, Sang Soo; Jeong, Seo In; Lim, Hyo Soon; Choi, Yoo Duk; Lee, Kyoung Hwa; Kang, Woo Dae; Jeong, Yong Yeon; Kang, Heoung Keun

    2014-01-01

    The incidence, histologic distribution, and clinical manifestations of ovarian tumors in the pediatric population are distinct from those in adults. Although ovarian neoplasms in childhood and adolescence are rare, the diagnosis should be considered in young girls with abdominal pain and a palpable mass. Differential diagnosis in children and adolescents with ovarian tumors should be conducted on the basis of unique clinical manifestations, elevated serum tumor marker levels, and distinctive imaging findings. Although the clinical manifestations are nonspecific and may overlap, they may assist in diagnosis of some types of ovarian tumors. Children who present with a palpable mass or symptoms of precocious puberty have a high likelihood of malignancy. Many ovarian tumors are associated with abnormal hormonal activity and/or abnormal sexual development. Elevated levels of serum tumor markers, including α-fetoprotein, the beta subunit of human chorionic gonadotropin, and CA-125, raise concern for ovarian malignancies. However, negative tumor markers do not exclude the possibility of malignancy. Identification of imaging features at ultrasonography, computed tomography, and magnetic resonance imaging can help differentiate benign from malignant ovarian tumors and, in turn, plays a crucial role in determining treatment options. At imaging, malignant ovarian tumors usually appear predominantly solid or heterogeneous and are larger than benign tumors. Because surgery is the primary treatment for ovarian tumors, ovarian salvage with fertility preservation and use of a minimally invasive surgical technique are important in children and adolescents.

  17. Radiologic-Pathologic Correlation: Metastatic Pulmonary Epithelioid Hemangioendothelioma of Bone Primary

    PubMed Central

    Lee, Christine U; Zreik, Riyam T; Boland, Jennifer M; White, Mariah L

    2015-01-01

    Epithelioid hemangioendothelioma is a rare vascular malignancy often characterized by a clinically indolent course and delayed diagnosis. The authors present the radiologic and pathologic features of a case of pulmonary epithelioid hemangioendothelioma which was initially thought to be calcified granulomas. PMID:26430543

  18. Ileostomy obstruction by ingested apricot stone with clinical-radiological-pathological correlation.

    PubMed

    George, A J; Fallaize, R C; Bennett, J; Shabbir, J

    2015-09-15

    Patients with stomas often present with bowel obstruction, often secondary to adhesions. This case describes the presentation, investigation and management of a 62-year-old woman with an end ileostomy, who presented to hospital with acute abdominal pain and subacute bowel obstruction. Further questioning revealed the recent ingestion of an apricot stone and this was identified by multimodality imaging as the cause of the luminal obstruction in the distal ileum, just proximal to the stoma. After a failed period of conservative management, examination under anaesthesia was performed and digital extraction attempted, but this was unsuccessful. Rather than surgical stoma revision, endoscopic removal was achieved. The patient improved and was discharged the following day. However, her small bowel obstruction relapsed within 48 h. She was readmitted and underwent stoma revision with no further problems.

  19. From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation.

    PubMed

    Murphey, M D; Smith, W S; Smith, S E; Kransdorf, M J; Temple, H T

    1999-01-01

    Numerous neurogenic tumors can affect the musculoskeletal system, including traumatic neuroma, Morton neuroma, neural fibrolipoma, nerve sheath ganglion, neurilemoma, neurofibroma, and malignant peripheral nerve sheath tumors (PNSTs). The diagnosis of neurogenic tumors can be suggested from their imaging appearances, including lesion shape and intrinsic imaging characteristics. It is also important to establish lesion location along a typical nerve distribution (eg, plantar digital nerve in Morton neuroma, median nerve in neural fibrolipoma, large nerve trunk in benign and malignant PNSTs). Traumatic and Morton neuromas are commonly related to an amputation stump or are located in the intermetatarsal space, respectively. Neural fibrolipomas show fat interspersed between nerve fascicles and are often associated with macrodactyly. Nerve sheath ganglion has a cystic appearance and commonly occurs about the knee. Radiologic characteristics of neurilemoma, neurofibroma, and malignant PNST at computed tomography (CT), ultrasonography, and magnetic resonance imaging include fusiform shape, identification of entering and exiting nerve, low attenuation at CT, target sign, fascicular sign, split-fat sign, and associated muscle atrophy. Although differentiation of neurilemoma from neurofibroma and of benign from malignant PNST is problematic, recognition of the radiologic appearances of neurogenic tumors often allows prospective diagnosis and improves clinical management of patients.

  20. CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation.

    PubMed

    Egashira, Ryoko; Kondo, Tetsuya; Hirai, Tetsuyoshi; Kamochi, Noriyuki; Yakushiji, Mai; Yamasaki, Fumio; Irie, Hiroyuki

    2013-01-01

    Primary Sjögren syndrome is an immune-mediated exocrinopathy characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands. Various systemic extraglandular disorders are associated with primary Sjögren syndrome, and the thorax is commonly affected. The pulmonary manifestations of primary Sjögren syndrome may be categorized as airway abnormalities, interstitial pneumonias, and lymphoproliferative disorders; in each category, bronchiectasis or centrilobular nodules, nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia are common. These manifestations do not usually occur in isolation; they are concomitantly seen with other types of lesions. Mucosa-associated lymphoid tissue (MALT) lymphoma and amyloidosis are key components of lymphoproliferative disorders, and MALT lymphoma should always be considered because its morphologic characteristics are similar to those of benign lymphoproliferative disorders. Amyloidosis is rare but important because it carries a risk for underlying MALT lymphoma or plasmacytoma, and it may lead to hemoptysis during biopsy. In addition, thin-walled air cysts are characteristic of primary Sjögren syndrome, irrespective of the main pulmonary manifestations. Lymphadenopathy and multilocular thymic cysts may be seen in the mediastinum. During the follow-up period, there is a risk for acute exacerbation of interstitial pneumonia and development of malignant lymphoma. Often, primary Sjögren syndrome is subclinical, but there are various underlying risks. Thus, imaging findings are important. In addition to the various types of interstitial pneumonia and airway abnormalities, air cysts and mediastinal manifestations may help diagnose primary Sjögren syndrome.

  1. Review of ovarian tumors in children and adolescents: radiologic-pathologic correlation.

    PubMed

    Heo, Suk Hee; Kim, Jin Woong; Shin, Sang Soo; Jeong, Seo In; Lim, Hyo Soon; Choi, Yoo Duk; Lee, Kyoung Hwa; Kang, Woo Dae; Jeong, Yong Yeon; Kang, Heoung Keun

    2014-01-01

    The incidence, histologic distribution, and clinical manifestations of ovarian tumors in the pediatric population are distinct from those in adults. Although ovarian neoplasms in childhood and adolescence are rare, the diagnosis should be considered in young girls with abdominal pain and a palpable mass. Differential diagnosis in children and adolescents with ovarian tumors should be conducted on the basis of unique clinical manifestations, elevated serum tumor marker levels, and distinctive imaging findings. Although the clinical manifestations are nonspecific and may overlap, they may assist in diagnosis of some types of ovarian tumors. Children who present with a palpable mass or symptoms of precocious puberty have a high likelihood of malignancy. Many ovarian tumors are associated with abnormal hormonal activity and/or abnormal sexual development. Elevated levels of serum tumor markers, including α-fetoprotein, the beta subunit of human chorionic gonadotropin, and CA-125, raise concern for ovarian malignancies. However, negative tumor markers do not exclude the possibility of malignancy. Identification of imaging features at ultrasonography, computed tomography, and magnetic resonance imaging can help differentiate benign from malignant ovarian tumors and, in turn, plays a crucial role in determining treatment options. At imaging, malignant ovarian tumors usually appear predominantly solid or heterogeneous and are larger than benign tumors. Because surgery is the primary treatment for ovarian tumors, ovarian salvage with fertility preservation and use of a minimally invasive surgical technique are important in children and adolescents. PMID:25384300

  2. Lymphography in the management of urologic tumors: radiological-pathological correlation

    SciTech Connect

    Strijk, S.P.; Debruyne, F.M.; Herman, C.J.

    1983-01-01

    Of 59 patients undergoing lymphography as part of staging for urologic tumor (bladder carcinoma, prostate carcinoma, seminomatous and nonseminomatous testicular tumors), histological material was obtained in 38. Lymphography was shown to have a specificity of 95%, a sensitivity of 81%, and an overall accuracy of 89%. When lymph node regions were considered, the specificity was 99.2%, sensitivity 59%, and overall accuracy 91%. Causes and discrepancies between radiography and histology are evaluated and receiver operating characteristics curves are demonstrated.

  3. From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.

    PubMed

    Murphey, Mark D; Ruble, Chad M; Tyszko, Sean M; Zbojniewicz, Andrew M; Potter, Benjamin K; Miettinen, Markku

    2009-11-01

    Musculoskeletal fibromatoses represent a wide spectrum of fibroblastic and myofibroblastic neoplasms with similar pathologic appearances and variable clinical behavior. These lesions can be categorized by location (superficial or deep) or by the age group predominantly affected. Superficial fibromatoses in adults (palmar and plantar) and children (calcifying aponeurotic fibroma, lipofibromatosis, and inclusion body fibromatosis) are often small slow-growing lesions; their diagnosis is suggested by location. Deep fibromatoses in adults (desmoid type and abdominal wall) and children (fibromatosis colli and myofibroma and myofibromatosis) are frequently large and more rapidly enlarging; location of these lesions may be nonspecific. Radiographic findings typically are nonspecific. Cross-sectional imaging (ultrasonography, computed tomography, or magnetic resonance [MR] imaging) reveals lesion location, extent, and involvement of adjacent structures for staging and evaluation of local recurrence. MR imaging findings of predominantly low to intermediate signal intensity, nonenhancing bands of low signal intensity on long repetition time MR images that represent collagenized regions, and extension along fascial planes ("fascial tail" sign) add specificity for diagnosis. Additional features that aid in diagnostic specificity include an abdominal wall location related to pregnancy (abdominal wall fibromatosis), a lower neck location in a young child (fibromatosis colli), an adipose component (lipofibromatosis), or multiple lesions in young children (myofibromatosis). Treatment may be conservative or surgical resection, depending on the specific diagnosis. Local recurrence is common after surgical resection owing to the infiltrative growth of these lesions. Recognition that the appearances of the various types of musculoskeletal fibromatoses reflect their pathologic characteristics improves radiologic assessment and helps optimize patient management.

  4. Tomosynthesis-detected Architectural Distortion: Management Algorithm with Radiologic-Pathologic Correlation.

    PubMed

    Durand, Melissa A; Wang, Steven; Hooley, Regina J; Raghu, Madhavi; Philpotts, Liane E

    2016-01-01

    As use of digital breast tomosynthesis becomes increasingly widespread, new management challenges are inevitable because tomosynthesis may reveal suspicious lesions not visible at conventional two-dimensional (2D) full-field digital mammography. Architectural distortion is a mammographic finding associated with a high positive predictive value for malignancy. It is detected more frequently at tomosynthesis than at 2D digital mammography and may even be occult at conventional 2D imaging. Few studies have focused on tomosynthesis-detected architectural distortions to date, and optimal management of these distortions has yet to be well defined. Since implementing tomosynthesis at our institution in 2011, we have learned some practical ways to assess architectural distortion. Because distortions may be subtle, tomosynthesis localization tools plus improved visualization of adjacent landmarks are crucial elements in guiding mammographic identification of elusive distortions. These same tools can guide more focused ultrasonography (US) of the breast, which facilitates detection and permits US-guided tissue sampling. Some distortions may be sonographically occult, in which case magnetic resonance imaging may be a reasonable option, both to increase diagnostic confidence and to provide a means for image-guided biopsy. As an alternative, tomosynthesis-guided biopsy, conventional stereotactic biopsy (when possible), or tomosynthesis-guided needle localization may be used to achieve tissue diagnosis. Practical uses for tomosynthesis in evaluation of architectural distortion are highlighted, potential complications are identified, and a working algorithm for management of tomosynthesis-detected architectural distortion is proposed. PMID:26963448

  5. Spinal osteoblastic meningioma with hematopoiesis: radiologic-pathologic correlation and review of the literature.

    PubMed

    Cochran, Elizabeth J; Schlauderaff, Abraham; Rand, Scott D; Eckardt, Gerald W; Kurpad, Shekar

    2016-10-01

    Spinal meningiomas associated with bone formation and hematopoiesis are rare tumors with only 3 prior case reports in the literature. We describe a case report of a woman who presented with back pain and an isolated event of urinary incontinence. A calcified spinal canal mass at T8 was identified on computed tomographic and magnetic resonance imaging. A gross total resection of the tumor was performed and pathologic examination showed a meningioma, World Health Organization grade 1, containing bone and bone marrow elements. A review of previously reported cases and a discussion of possible mechanisms of bone and hematopoiesis development in meningioma are presented. PMID:27649951

  6. Postmortem computed tomography in victims of military air mishaps: radiological-pathological correlation of CT findings.

    PubMed

    Levy, Gad; Goldstein, Liav; Blachar, Arye; Apter, Sara; Barenboim, Erez; Bar-Dayan, Yaron; Shamis, Ari; Atar, Eli

    2007-10-01

    A thorough medical inquiry is included in every aviation mishap investigation. While the gold standard of this investigation is a forensic pathology examination, numerous reports stress the important role of computed tomography in the postmortem evaluation of trauma victims. To characterize the findings identified by postmortem CT and compare its performance to conventional autopsy in victims of military aviation mishaps, we analyzed seven postmortem CT examinations. Musculoskeletal injuries accounted for 57.8% of the traumatic findings identified by postmortem CT. The most frequent findings were fractures of the rib (47%), skull (9.6%) and facial bones (8.6%). Abnormally located air accounted for 24% of findings, for which CT was superior (3.5% detected by autopsy, 100% by postmortem CT, P < 0.001). The performance of autopsy in detecting injuries was superior (autopsy detected 85.8% of all injuries, postmortem CT detected 53.9%, P < 0.001), especially in the detection of superficial lesions (100% detected by autopsy, 10.5% by postmortem CT, P < 0.001) and solid organ injuries (100% by autopsy, 18.5% by postmortem CT, P < 0.001). Performance in the detection of musculoskeletal injuries was similar (91.3% for autopsy, 90.3% for postmortem CT, P = not significant). Postmortem CT and autopsy have distinct performance profiles, and although the first cannot replace the latter it is a useful complementary examination. PMID:17987755

  7. Ileostomy obstruction by ingested apricot stone with clinical-radiological-pathological correlation.

    PubMed

    George, A J; Fallaize, R C; Bennett, J; Shabbir, J

    2015-01-01

    Patients with stomas often present with bowel obstruction, often secondary to adhesions. This case describes the presentation, investigation and management of a 62-year-old woman with an end ileostomy, who presented to hospital with acute abdominal pain and subacute bowel obstruction. Further questioning revealed the recent ingestion of an apricot stone and this was identified by multimodality imaging as the cause of the luminal obstruction in the distal ileum, just proximal to the stoma. After a failed period of conservative management, examination under anaesthesia was performed and digital extraction attempted, but this was unsuccessful. Rather than surgical stoma revision, endoscopic removal was achieved. The patient improved and was discharged the following day. However, her small bowel obstruction relapsed within 48 h. She was readmitted and underwent stoma revision with no further problems. PMID:26374775

  8. Non-squamous cell neoplasms of the larynx: radiologic-pathologic correlation.

    PubMed

    Becker, M; Moulin, G; Kurt, A M; Dulgerov, P; Vukanovic, S; Zbären, P; Marchal, F; Rüfenacht, D A; Terrier, F

    1998-01-01

    A variety of benign and malignant non-squamous cell neoplasms may affect the larynx. Most of these uncommon laryngeal neoplasms are located beneath an intact mucosa, making diagnosis difficult with endoscopy alone, and sampling errors may occur if only traditional superficial biopsies are performed. In some laryngeal neoplasms, radiologic evaluation allows the correct diagnosis. Hemangiomas have very high signal intensity at T2-weighted magnetic resonance (MR) imaging and strong enhancement at both computed tomography (CT) and MR imaging after administration of contrast material. Phleboliths, which are pathognomonic for hemangiomas, are easily identified at CT. Chondrogenic tumors typically manifest with coarse or stippled calcifications at CT. Because of their high water content, chondrogenic tumors have very high signal intensity on T2-weighted MR images, whereas only moderate enhancement is observed after administration of contrast material. Lipomas typically manifest at both CT and MR imaging as homogeneous nonenhancing lesions. They are isoattenuating to subcutaneous fat at CT and isointense relative to subcutaneous fat with all MR pulse sequences. Metastases from renal adenocarcinoma typically demonstrate strong contrast enhancement and flow voids at MR imaging, and metastases from melanotic melanoma usually have high signal intensity on T1-weighted MR images and low signal intensity on T2-weighted images owing to the paramagnetic properties of melanin. Although radiologic findings are nonspecific in most other non-squamous cell neoplasms of the larynx (eg, Kaposi sarcoma, hematopoietic tumors, tumors of the minor salivary glands, metastases from amelanotic melanoma), cross-sectional imaging can play an important role in the diagnostic work-up of these unusual tumors by delineating the extent of submucosal tumor spread and directing the endoscopist to the appropriate site for the deep, transmucosal biopsies needed to establish the diagnosis. In addition, CT and MR imaging are crucial for posttherapeutic monitoring and early detection of local recurrence.

  9. Radiology-Pathology Conference: pulmonary hyalinizing granuloma associated with lupus-like anticoagulant and Morvan's Syndrome.

    PubMed

    Winger, David I; Spiegler, Peter; Trow, Terence K; Goyal, Amit; Yu, Huiying; Yung, Elizabeth; Katz, Douglas S

    2007-01-01

    Pulmonary hyalinizing granulomata are rare, noninfectious, fibrosing lesions of the lung, which can mimic metastatic disease radiographically. Their etiology is unknown, but they may be caused by an exaggerated immune response. We report the radiology, long clinical course, and pathology of a patient with pulmonary hyalinizing granuloma who presented with initially asymptomatic pulmonary nodules. Over a 10-year period, the patient developed multiple insidious autoimmune phenomena, including lupus anticoagulant, neuromyotonia, demyelinating sensorimotor polyneuropathy, and eventually, Morvan's syndrome. Such an association has not been previously published to our knowledge. PMID:17599621

  10. From the radiologic pathology archives: gastrointestinal lymphoma: radiologic and pathologic findings.

    PubMed

    Lewis, Rachel B; Mehrotra, Anupamjit K; Rodríguez, Pablo; Manning, Maria A; Levine, Marc S

    2014-01-01

    Gastrointestinal (GI) lymphoma encompasses a heterogeneous group of neoplasms that have a common lymphoid origin but variable pathologic and imaging features. Extranodal marginal zone B-cell lymphoma (ENMZL) and diffuse large B-cell lymphoma (DLBCL) are the most common. ENMZL usually occurs in the stomach, where it is associated with chronic infection by Helicobacter pylori, and is typically a superficial spreading lesion that causes mucosal nodularity or ulceration and mild wall thickening. DLBCL may arise de novo or from transformation of ENMZL or other low-grade lymphomas. This form of lymphoma produces extensive wall thickening or a bulky mass, but obstruction is uncommon. Mantle cell lymphoma is the classic cause of lymphomatous polyposis, but multiple polyps or nodules can also be seen with ENMZL and follicular lymphoma. Burkitt lymphoma is usually characterized by an ileocecal mass or wall thickening in the terminal ileum in young children, often in the setting of widespread disease. Primary GI Hodgkin lymphoma, which is rare, may be manifested by a variety of findings, though stenosis is more common than with non-Hodgkin lymphoma. Enteropathy-associated T-cell lymphoma is frequently associated with celiac disease and is characterized by wall thickening, ulceration, and even perforation of the jejunum. Accurate radiologic diagnosis of GI lymphoma requires a multifactorial approach based on the clinical findings, site of involvement, imaging findings, and associated complications. PMID:25384294

  11. From the archives of the AFIP: Pediatric liver masses: radiologic-pathologic correlation part 1. Benign tumors.

    PubMed

    Chung, Ellen M; Cube, Regino; Lewis, Rachel B; Conran, Richard M

    2010-05-01

    Benign hepatic tumors in children include lesions that are unique to the pediatric age group and others that are more common in adults. Infantile hemangioendothelioma, or infantile hepatic hemangioma, is a benign vascular tumor that may cause serious clinical complications. It is composed of vascular channels lined by endothelial cells. At imaging, large feeding arteries and draining veins and early, intense, peripheral nodular enhancement with centripetal filling on delayed images are characteristic features. Mesenchymal hamartoma of the liver occurs in young children and is characterized pathologically by mesenchymal proliferation with fluid-containing cysts of varying size and number. The mesenchymal component or cystic component may predominate; this predominance determines the imaging appearance of the tumor. Benign epithelial tumors that are common in adults may infrequently occur in childhood. These include focal nodular hyperplasia (FNH), hepatocellular adenoma, and nodular regenerative hyperplasia. All are composed of hyperplastic hepatocytes similar to surrounding liver parenchyma and may be difficult to discern at imaging. Preferential hepatic arterial phase enhancement helps distinguish FNH and hepatic adenoma from uninvolved liver. Hepatic adenoma often has intracellular fat and a propensity for intratumoral hemorrhage, neither of which are seen in FNH. Unlike adenoma, FNH often contains enough Kupffer cells to show uptake at sulfur colloid scintigraphy. Nodular regenerative hyperplasia is often associated with portal hypertension, which may be evident at imaging. Knowledge of how the pathologic features of these tumors affect their imaging appearances helps radiologists offer an appropriate differential diagnosis and management plan.

  12. From the archives of the AFIP: tumors and tumorlike lesions of the testis: radiologic-pathologic correlation.

    PubMed

    Woodward, Paula J; Sohaey, Roya; O'Donoghue, Michael J; Green, Douglas E

    2002-01-01

    Testicular carcinoma represents only 1% of all neoplasms in men, but it is the most common malignancy in the 15-34-year-old age group. Germ cell tumors constitute 95% of all testicular tumors. Germ cell tumors are a varied group of neoplasms whose imaging features reflect their underlying histologic characteristics. Seminomas are generally well-defined homogeneous lesions, whereas the nonseminomatous tumors (embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, and mixed germ cell tumor) have a much more varied appearance. Germ cell tumors follow a predictable pattern of spread via the lymphatic drainage to the retroperitoneal nodes. Choriocarcinoma, which has a proclivity for early hematogenous spread, is a notable exception. Testicular tumors may also arise from the sex cords (Sertoli cells) and stroma (Leydig cells). Although 90% of these tumors are benign, there are no reliable imaging criteria to differentiate them from malignant masses. Some benign testicular masses can be recognized, obviating an unwarranted orchiectomy. A dilated rete testis is a normal variant and appears as a series of small tubules near the mediastinum testis. Other benign lesions that can be suspected on the basis of imaging findings and history include intratesticular cysts, epidermoid cysts, congenital adrenal hyperplasia, and sarcoidosis.

  13. Cognitive Correlates of Metamemory in Alzheimer's Disease

    PubMed Central

    Shaked, Danielle; Farrell, Meagan; Huey, Edward; Metcalfe, Janet; Cines, Sarah; Karlawish, Jason; Sullo, Elisabeth; Cosentino, Stephanie

    2014-01-01

    Objective Metamemory, or knowledge of one's memory abilities, is often impaired in individuals with Alzheimer's disease (AD), although the basis of this metacognitive deficit has not been fully articulated. Behavioral and imaging studies have produced conflicting evidence regarding the extent to which specific cognitive domains (i.e., executive functioning (EF), memory) and brain regions contribute to memory awareness. The primary aim of this study was to disentangle the cognitive correlates of metamemory in AD by examining the relatedness of objective metamemory performance to cognitive tasks grouped by domain (EF or memory) as well as by preferential hemispheric reliance defined by task modality (verbal or nonverbal). Method 89 participants with mild AD recruited at Columbia University Medical Center and the University of Pennsylvania underwent objective metamemory and cognitive testing. Partial correlations were used to assess the relationship between metamemory and four cognitive variables, adjusted for recruitment site. Results The significant correlates of metamemory included nonverbal fluency (r = .27 p = .02) and nonverbal memory (r = .24, p = .04). Conclusions Our findings suggest that objectively measured metamemory in a large sample of individuals with mild AD is selectively related to a set of inter-domain nonverbal tasks. The association between metamemory and the nonverbal tasks may implicate a shared reliance on a right-sided cognitive network that spans frontal and temporal regions. PMID:24819066

  14. Correlation between osteoporosis and cardiovascular disease

    PubMed Central

    Sprini, Delia; Rini, Giovam Battista; Di Stefano, Laura; Cianferotti, Luisella; Napoli, Nicola

    2014-01-01

    Summary Several evidences have shown in the last years a possible correlation between cardiovascular diseases and osteoporosis. Patients affected with osteoporosis, for example, have a higher risk of cardiovascular diseases than subjects with normal bone mass. However, the heterogeneous approaches and the different populations that have been studied so far have limited the strength of the findings. Studies conducted in animal models show that vascular calcification is a very complex mechanism that involves similar pathways described in the normal bone calcification. Proteins like BMP, osteopontin, osteoprotegerin play an important role at the bone level but are also highly expressed in the calcified vascular tissue. In particular, it seems that the OPG protect from vascular calcification and elevated levels have been found in patients with CVD. Other factors like oxidative stress, inflammation, free radicals, lipids metabolism are involved in this complex scenario. It is not a case that medications used for treating osteoporosis also inhibit the atherosclerotic process, acting on blood pressure and ventricular hypertrophy. Given the limited amount of available data, further studies are needed to elucidate the underlying mechanisms between osteoporosis and cardiovascular disease which may be important in the future also for preventive and therapeutic approaches of both conditions. PMID:25285139

  15. Putaminal Diffusivity Correlates With Disease Progression in Parkinson's Disease

    PubMed Central

    Chan, Ling-Ling; Ng, Kia-Min; Yeoh, Chooi-Sum; Rumpel, H.; Li, Hui-Hua; Tan, Eng-King

    2016-01-01

    Abstract Diffusion tensor imaging (DTI) is an increasingly used noninvasive imaging tool. However its long-term clinical utility is unclear. Parkinson's disease (PD) is a common neurodegenerative disease. We prospectively examined a cohort of 46 Parkinson's disease (PD) patients who underwent diffusion tensor imaging (DTI) of the brain at baseline and 6 years later on a 1.5 Tesla scanner using a standardized protocol. DTI parameters of mean diffusivity (MD) and fractional anisotrophy (FA) were extracted using regions-of-interest (ROIs) analysis from various brain regions. Compared to the baseline scan, MD increased in all brain regions (P < 0.0001). FA increased in the substantia nigra and posterior putamen, but decreased in the frontal white matter (P < 0.0001). Linear regression analysis demonstrated that the MD in the anterior putamen increased 11.6 units (95% CI = [4.71, 18.43]) (P = 0.0003) for every unit increase of United PD Rating Scale (UPDRS). Our 6-year prospective longitudinal study demonstrated increased diffusivity in all brain regions and that in the anterior putamen correlated with disease progression. Serial diffusion data may be useful as an additional objective in vivo biomarker for motor progression in PD. PMID:26871779

  16. [Initial management of advanced ovarian cancer: What radiological, pathological and surgical information are important for optimal therapeutic strategy?].

    PubMed

    Heudel, Pierre-Etienne; Selle, Frédéric; Morice, Philippe; Rouzier, Roman; Taieb, Sophie; Devouassoux-Shisheboran, Mojgan; Genestie, Catherine; Balleyguier, Corinne; Ray-Coquard, Isabelle

    2015-09-01

    Because the majority of patients present advanced disease at diagnosis, the management of epithelial ovarian cancer needs specialist multidisciplinary teamwork. Expertise in surgery, chemotherapy, imaging and histopathology is essential to achieve optimum outcomes. Computed tomography scans are routinely used to determine the extent of disease and to aid in surgical planning. The histologic classification is crucial to plan the best therapeutic strategy and to define the prognosis of disease. Pathological prognostic factors, such as degree of differentiation, FIGO-stage, and histological type have to be described. This report is fundamental to assessing prognosis and selection of appropriate treatment strategy. An adequate staging procedure is an extensive staging by an experienced gynecological oncologist, exploring the entire upper abdomen, and the pelvic and para-aortic lymph node regions to define the Peritoneal Cancer Index (PCI). The final assessment is the completeness of cytoreduction (CC) score, which is an assessment of residual disease after a maximal surgical effort. Initial management of advanced ovarian cancer is best provided by a specialist multidisciplinary team, including a radiologist, a pathologist, a gynecologic oncologist and a medical oncologist.

  17. Innovations in macroscopic evaluation of pancreatic specimens and radiologic correlation.

    PubMed

    Triantopoulou, Charikleia; Papaparaskeva, Kleo; Agalianos, Christos; Dervenis, Christos

    2016-01-01

    The purpose of this study was to evaluate the feasibility of a novel dissection technique of surgical specimens in different cases of pancreatic tumors and provide a radiologic pathologic correlation. In our hospital, that is a referral center for pancreatic diseases, the macroscopic evaluation of the pancreatectomy specimens is performed by the pathologists using the axial slicing technique (instead of the traditional procedure with longitudinal opening of the main pancreatic and/or common bile duct and slicing along the plane defined by both ducts). The specimen is sliced in an axial plane that is perpendicular to the longitudinal axis of the descending duodenum. The procedure results in a large number of thin slices (3-4 mm). This plane is identical to that of CT or MRI and correlation between pathology and imaging is straightforward. We studied 70 cases of suspected different solid and cystic pancreatic tumors and we correlated the tumor size and location, the structure-consistency (areas of necrosis-hemorrhage-fibrosis-inflammation), the degree of vessels' infiltration, the size of pancreatic and common bile duct and the distance from resection margins. Missed findings by imaging or pitfalls were recorded and we tried to explain all discrepancies between radiology evaluation and the histopathological findings. Radiologic-pathologic correlation is extremely important, adding crucial information on imaging limitations and enabling quality assessment of surgical specimens. The deep knowledge of different pancreatic tumors' consistency and way of extension helps to improve radiologists' diagnostic accuracy and minimize the radiological-surgical mismatching, preventing patients from unnecessary surgery. PMID:27069980

  18. Innovations in macroscopic evaluation of pancreatic specimens and radiologic correlation

    PubMed Central

    Triantopoulou, Charikleia; Papaparaskeva, Kleo; Agalianos, Christos; Dervenis, Christos

    2016-01-01

    The purpose of this study was to evaluate the feasibility of a novel dissection technique of surgical specimens in different cases of pancreatic tumors and provide a radiologic pathologic correlation. In our hospital, that is a referral center for pancreatic diseases, the macroscopic evaluation of the pancreatectomy specimens is performed by the pathologists using the axial slicing technique (instead of the traditional procedure with longitudinal opening of the main pancreatic and/or common bile duct and slicing along the plane defined by both ducts). The specimen is sliced in an axial plane that is perpendicular to the longitudinal axis of the descending duodenum. The procedure results in a large number of thin slices (3–4 mm). This plane is identical to that of CT or MRI and correlation between pathology and imaging is straightforward. We studied 70 cases of suspected different solid and cystic pancreatic tumors and we correlated the tumor size and location, the structure—consistency (areas of necrosis—hemorrhage—fibrosis—inflammation), the degree of vessels’ infiltration, the size of pancreatic and common bile duct and the distance from resection margins. Missed findings by imaging or pitfalls were recorded and we tried to explain all discrepancies between radiology evaluation and the histopathological findings. Radiologic-pathologic correlation is extremely important, adding crucial information on imaging limitations and enabling quality assessment of surgical specimens. The deep knowledge of different pancreatic tumors’ consistency and way of extension helps to improve radiologists’ diagnostic accuracy and minimize the radiological-surgical mismatching, preventing patients from unnecessary surgery. PMID:27069980

  19. Combined Undifferentiated and Neuroendocrine Carcinomas of the Gallbladder Appearing as Two Separate Lesions: A Case Report with Radiological-Pathological Correlation.

    PubMed

    Lee, So Won; Baek, Seung Yon; Sung, Sun Hee

    2015-05-01

    We report herein a rare case of incidentally detected combined undifferentiated and neuroendocrine carcinomas of the gallbladder. An incidental gallbladder malignancy was revealed on abdominal ultrasound and multi-detector computed tomography in a 54-year-old man. A short distance from the main polypoid hypoechoic mass at the fundus of the gallbladder, focal wall thickening was noted with prominently increased power Doppler flow. Extended cholecystectomy was performed, and histology confirmed the main polypoid mass as undifferentiated carcinoma and the separate nodule as neuroendocrine carcinoma. To our knowledge, this is the first report presenting two separate lesions of combined gallbladder carcinomas by radiological features.

  20. Prevalence and correlates of heart disease among adults in Singapore.

    PubMed

    Picco, Louisa; Subramaniam, Mythily; Abdin, Edimansyah; Vaingankar, Janhavi Ajit; Chong, Siow Ann

    2016-02-01

    Heart disease is one of the leading causes of morbidity and mortality worldwide and it has been well established that it is associated with both mental and physical conditions. This paper describes the prevalence of heart disease with mental disorders and other chronic physical conditions among the Singapore resident population. Data were from the Singapore Mental Health Study which was a representative, cross-sectional epidemiological survey undertaken with 6616 Singapore residents, between December 2009 and December 2010. The Composite International Diagnostic Interview Version 3.0 was used to establish the diagnosis of mental disorders, while a chronic medical conditions checklist was used to gather information on 15 physical conditions, including various forms of heart disease. Health-related quality of life was measured using the Euro-Quality of Life Scale (EQ-5D). The lifetime prevalence of heart disease was 2.8%. Socio-demographic correlates of heart disease included older age, Indian ethnicity, secondary education (vs. tertiary) and being economically inactive. After adjusting for socio-demographic variables and other comorbid physical and mental disorders, the prevalence of major depressive disorder and bipolar disorder were significantly higher among those with heart disease, as were diabetes, arthritis, kidney failure and lung disease. These findings highlight important associations between heart disease and various socio-demographic correlates, mental disorders and physical conditions. Given the high prevalence of mood disorders among heart disease patients, timely and appropriate screening and treatment of mental disorders among this group is essential.

  1. Serum Renalase Levels Correlate with Disease Activity in Lupus Nephritis

    PubMed Central

    Zhang, Minfang; Shao, Xinghua; Chang, Xinbei; Fan, Zhuping; Cao, Qin; Mou, Shan; Wang, Qin; Yan, Yucheng; Desir, Gary; Ni, Zhaohui

    2015-01-01

    Introduction Lupus nephritis (LN) is among the most serious complications of systemic lupus erythematosus (SLE), which causes significant morbidity and mortality. Renalase is a novel, kidney-secreted cytokine-like protein that promotes cell survival. Here, we aimed to investigate the relationship of serum renalase levels with LN and its role in the disease progression of LN. Methods For this cross-sectional study, 67 LN patients and 35 healthy controls were enrolled. Seventeen active LN patients who received standard therapies were followed up for six months. Disease activity was determined by the SLE Disease Activity–2000 (SLEDAI-2K) scoring system and serum renalase amounts were determined by ELISA. Predictive value of renalase for disease activity was assessed. Furthermore, the expression of renalase in the kidneys of patients and macrophage infiltration was assessed by immunohistochemistry. Results Serum renalase amounts were significantly higher in LN patients than in healthy controls. Moreover, patients with proliferative LN had more elevated serum renalase levels than Class V LN patients. In proliferative LN patients, serum renalase levels were significantly higher in patients with active LN than those with inactive LN. Serum renalase levels were positively correlated with SLEDAI-2K, 24-h urine protein excretion, ds-DNA and ESR but inversely correlated with serum albumin and C3. Renalase amounts decreased significantly after six-months of standard therapy. The performance of renalase as a marker for diagnosis of active LN was 0.906 with a cutoff value of 66.67 μg/ml. We also observed that the amount of renalase was significantly higher in glomerular of proliferative LN along with the co-expression of macrophages. Conclusion Serum renalase levels were correlated with disease activity in LN. Serum renalase might serve as a potential indicator for disease activity in LN. The marked increase of glomerular renalase and its association with macrophages suggest

  2. Neuroanatomical correlates of cognitive functioning in prodromal Huntington disease

    PubMed Central

    Harrington, Deborah L; Liu, Dawei; Smith, Megan M; Mills, James A; Long, Jeffrey D; Aylward, Elizabeth H; Paulsen, Jane S

    2014-01-01

    Introduction The brain mechanisms of cognitive impairment in prodromal Huntington disease (prHD) are not well understood. Although striatal atrophy correlates with some cognitive abilities, few studies of prHD have investigated whether cortical gray matter morphometry correlates in a regionally specific manner with functioning in different cognitive domains. This knowledge would inform the selection of cognitive measures for clinical trials that would be most sensitive to the target of a treatment intervention. Method In this study, random forest analysis was used to identify neuroanatomical correlates of functioning in five cognitive domains including attention and information processing speed, working memory, verbal learning and memory, negative emotion recognition, and temporal processing. Participants included 325 prHD individuals with varying levels of disease progression and 119 gene-negative controls with a family history of HD. In intermediate analyses, we identified brain regions that showed significant differences between the prHD and the control groups in cortical thickness and striatal volume. Brain morphometry in these regions was then correlated with cognitive functioning in each of the domains in the prHD group using random forest methods. We hypothesized that different regional patterns of brain morphometry would be associated with performances in distinct cognitive domains. Results The results showed that performances in different cognitive domains that are vulnerable to decline in prHD were correlated with regionally specific patterns of cortical and striatal morphometry. Putamen and/or caudate volumes were top-ranked correlates of performance across all cognitive domains, as was cortical thickness in regions related to the processing demands of each domain. Conclusions The results underscore the importance of identifying structural magnetic resonance imaging (sMRI) markers of functioning in different cognitive domains, as their relative

  3. Serum melatonin in juvenile rheumatoid arthritis: correlation with disease activity.

    PubMed

    El-Awady, Hanaa Mahmoud; El-Wakkad, Amany Salah El-Dien; Saleh, Maysa Tawheed; Muhammad, Saadia Ibraheem; Ghaniema, Eiman Mahmoud

    2007-05-01

    The study was conducted to investigate the abnormalities in early morning serum melatonin among patients with Juvenile Rheumatoid Arthritis (JRA) and to outline its relation to disease activity and severity. Twenty one patients with JRA and twenty healthy age and sex matched controls were enrolled in the study. Fifteen patients had polyarticular JRA, 3 had oligoarticular and 3 had systemic onset JRA. Evaluation was carried out clinically, functionally and radiologically by using disease activity score, Juvenile Arthritis Functional Assessment Report for Children (JAFAR-C score) and modified Larsen score, respectively. Laboratory investigations included Complete Blood Picture (CBC), The Erythrocyte Sedimentation Rate (ESR), C-Reactive Protein (CRP), classic IgM Rheumatoid Factor (RF), Anti-nuclear Antibodies (ANA) and melatonin estimation in serum. The serum levels of melatonin were significantly increased in JRA patients (mean +/- SD = 13.9 +/- 8 pg mL(-1)) as compared to healthy controls (mean +/- SD = 8.1 +/- 2.7 pg mL(-1), p < 0.01). A significant positive correlation could link serum melatonin levels to disease activity scores and ESR (r = 0.91, p < 0.001 and r = 0.55, p < 0.01, respectively). No significant correlation was found between melatonin and either Larsen or JAFAR scores (r = 0.19, r = 0.15, respectively). According to melatonin levels, there were 2 groups of patients: Group I with elevated melatonin level (more than 11 pg mL(-1)) (n = 15) and group II with normal melatonin level (less than 11 pg mL(-1)) (n = 6). Patients with elevated melatonin levels had higher ESR (p < 0.05), higher disease activity scores (p < 0.01) and Larsen scores (p < 0.05), than the group of patients with normal serum melatonin. The results of GAFAR scores were comparable between the two groups (p > 0.05). Hence the study conclude that the elevated melatonin levels among JRA patients with active synovitis and its close relation to disease activity rather than disease severity

  4. Regulatory B cells correlate with HIV disease progression.

    PubMed

    Jiao, Yanmei; Wang, Xi; Zhang, Tong; Sun, Lijun; Wang, Rui; Li, Wei; Ji, Yunxia; Wu, Hao; Liu, Cuie

    2014-08-01

    A rare subset of IL-10-producing B cells, named Breg, was recently identified in mice and humans. Currently, there are no unified cell surface markers to identify Breg, and the relationship between the frequency of Breg and HIV disease progression in chronic HIV infection is unclear. In the present study, we determined whether the cell surface markers of Breg reported for other diseases are suitable for identifying Breg in HIV-infected patients. In addition, we examined the relationship between Breg and HIV disease progression. We found that Breg frequency correlated positively with viral load and negatively with CD4 count in chronic HIV infection. Following antiretroviral treatment, the CD4 count increased and the frequency of Breg decreased stepwise. There was no difference in IL-10 expression of CD1d(hi) or CD1d(lo) cells isolated from HIV-infected patients. Therefore, CD1d may not be a marker of Breg in HIV-infected patients.

  5. Correlations of clinical, neuroimaging, and electrophysiological features in Hirayama disease

    PubMed Central

    Liao, Ming-Feng; Chang, Hong-Shiu; Chang, Kuo-Hsuan; Ro, Long-Sun; Chu, Chun-Che; Kuo, Hung-Chou; Lyu, Rong-Kuo

    2016-01-01

    Abstract Hirayama disease (HD) is characterized by development of asymmetric forearm muscle atrophy during adolescence with or without focal cervical spinal cord atrophy. The purpose of this study is to assess the correlation of clinical symptoms, disease progression, and electrophysiological findings with cervical spine magnetic resonance imaging (MRI) findings. The medical records, cervical spine MRIs, and electrophysiological findings of 44 HD patients were retrospectively reviewed and analyzed. Denervation changes in any single C5 to C7 root-innervated muscle (deltoid, biceps, triceps, or extensor digitorum communis) occurred more frequently in the 25 patients with cord atrophy than the 19 patients without cord atrophy (88% vs 53%, P = 0.02). Onset age, duration of disease progression, neurological examinations, nerve conduction study, and electromyographic findings from individual muscles were similar between patient groups. Compared with HD patients without cord atrophy, HD patients with cord atrophy experience a more severe denervation change in C5 to C7 root-innervated muscles. PMID:27428223

  6. Dyspepsia and gastroesophageal reflux disease (GERD): is there any correlation?

    PubMed

    Simadibrata, Marcellus

    2009-10-01

    Dyspepsia is a syndrome characterized by symptoms and signs of upper gastrointestinal tract and the adjacent organs. It is estimated that 25% of the community have symptoms of dyspepsia syndrome. One-third of patients who visit general physician practices are patients with dyspepsia syndrome; and half of patients who visit gastroenterologists are also patients with dyspepsia syndrome. Dyspepsia syndrome and gastroesophageal reflux disease (GERD) are very prevalent in the community throughout the world.Gastroesophageal reflux disease (GERD) is more and more commonly found in daily medical practice. Until now,the natural history of disease on GERD and dyspepsia is hardly understood, even though many scientists studied both conditions and there are frequently overlapping. In an individual, GERD and dyspepsia may occur simultaneously and therefore they are hardly to be discriminated.The management of GERD is performed in keeping with Indonesia and Asia Pacific consensus, life-style modification and administering the acid suppression agents (Proton pump inhibitor (drug of choice), H2-receptor antagonist, etc),prokinetic agents (Cisapride, domperidone, etc). Life-style modification shall be performed as follows, i.e. sleep with 30-45 degree elevated head or upper chest, do not avoid sour beverages, chocolate, coffee or alcohol, avoid fat and various fried foods, sour food, less stress, stop smoking, small but frequent feeding, etc. There is a correlation between dyspepsia syndrome and gastroesophageal reflux disease(GERD), particularly between the functional dyspepsia and non-erosive gastroesophageal reflux (NERD). More appropriate definition is necessary to differentiate the dyspepsia syndrome and GERD. Further studies are needed to establish distinct definition and criteria between dyspepsia syndrome and GERD.

  7. Weak detrusor contractility correlates with motor disorders in Parkinson's disease.

    PubMed

    Terayama, Keiichiro; Sakakibara, Ryuji; Ogawa, Akihiro; Haruta, Hiroyuki; Akiba, Takashi; Nagao, Takeki; Takahashi, Osamu; Sugiyama, Megumi; Tateno, Akihiko; Tateno, Fuyuki; Yano, Masashi; Kishi, Masahiko; Tsuyusaki, Yohei; Uchiyama, Tomoyuki; Yamamoto, Tatsuya

    2012-12-01

    Limited attention has been paid to the relationship between urinary symptoms or urodynamic findings and motor disorders in Parkinson's disease (PD). We aimed to correlate pressure-flow urodynamic parameters with video-gait analysis parameters in PD. We recruited 41 patients with PD (25 men and 16 women; age, 70.6 ± 8.5 years; H & Y motor grading: 2 [range, 1-3]; disease duration: 4 years [range, 1-7]; taking levodopa 300 mg/day [range, 100-400]). All patients underwent pressure-flow urodynamics (parameters: first sensation, bladder capacity, detrusor overactivity [noted in 24 patients], and Watts factor [WF]) and video-gait analysis (parameters: time and number of strides for 5-m gait [simple task] and time for timed up and go [complex task]). Statistical analysis was made by Mann-Whitney's U-test for analyzing the relation between detrusor overactivity and gait as well as Spearman's rank-correlation coefficient test for analyzing the relation between the remaining parameters and gait. We found no relation between filling-phase urodynamics (detrusor overactivity, first sensation, and bladder capacity) and video-gait analysis parameters. By contrast, we found a significant relation between voiding-phase urodynamics (WF, reflecting detrusor power) and all three video-gait analysis parameters (reflecting lower-half bradykinesia and loss of postural reflex) in our PD patients (P < 0.01). The close relation between the WF and motor disorders in the present study suggests that, though clinically mild, a weak detrusor in PD might have a central origin. We should follow postvoid residual volume carefully in PD patients with advanced gait disorder, because postvoid residual volume might increase in such patients. PMID:23080035

  8. Cholinergic activity correlates with reserve proxies in Alzheimer's disease.

    PubMed

    Garibotto, Valentina; Tettamanti, Marco; Marcone, Alessandra; Florea, Ioana; Panzacchi, Andrea; Moresco, Rosamaria; Virta, Jere R; Rinne, Juha; Cappa, Stefano F; Perani, Daniela

    2013-11-01

    The clinical expression of Alzheimer's disease (AD) occurs as neuropathology exceeds the brain "reserve capacity." A possible association between the cholinergic system and reserve is suggested by preclinical observations that the cholinergic system allows cortical plasticity and by clinical observations of variable responses to cholinergic treatments depending on the patient's educational level. The aim of this study was to investigate the association of reserve proxies, that is, education and occupation, with acetylcholinesterase (AChE) activity, measured voxelwise by [(11)C]-MP4A and positron emission tomography (PET), in 9 healthy controls (HC), 7 patients with early probable AD, and 9 subjects with mild cognitive impairment (MCI) at the time of PET imaging, who progressed to AD at follow-up (prodromal AD). The analysis of prodromal and early AD showed positive correlations between education and AChE activity in the hippocampus, bilaterally, and between occupation and AChE activity in the right posterior cingulate gyrus. The significant correlation between AChE activity in structures belonging to the memory network and reserve proxies suggests that the brain reserve in AD is associated with a preserved/stimulated cholinergic neurotransmission.

  9. Biochemical correlates of neuropsychiatric illness in maple syrup urine disease

    PubMed Central

    Muelly, Emilie R.; Moore, Gregory J.; Bunce, Scott C.; Mack, Julie; Bigler, Don C.; Morton, D. Holmes; Strauss, Kevin A.

    2013-01-01

    Maple syrup urine disease (MSUD) is an inherited disorder of branched chain amino acid metabolism presenting with neonatal encephalopathy, episodic metabolic decompensation, and chronic amino acid imbalances. Dietary management enables survival and reduces risk of acute crises. Liver transplantation has emerged as an effective way to eliminate acute decompensation risk. Psychiatric illness is a reported MSUD complication, but has not been well characterized and remains poorly understood. We report the prevalence and characteristics of neuropsychiatric problems among 37 classical MSUD patients (ages 5–35 years, 26 on dietary therapy, 11 after liver transplantation) and explore their underlying mechanisms. Compared with 26 age-matched controls, MSUD patients were at higher risk for disorders of cognition, attention, and mood. Using quantitative proton magnetic resonance spectroscopy, we found lower brain glutamate, N-acetylaspartate (NAA), and creatine concentrations in MSUD patients, which correlated with specific neuropsychiatric outcomes. Asymptomatic neonatal course and stringent longitudinal biochemical control proved fundamental to optimizing long-term mental health. Neuropsychiatric morbidity and neurochemistry were similar among transplanted and nontransplanted MSUD patients. In conclusion, amino acid dysregulation results in aberrant neural networks with neurochemical deficiencies that persist after transplant and correlate with neuropsychiatric morbidities. These findings may provide insight into general mechanisms of psychiatric illness. PMID:23478409

  10. Angiogenic growth factors correlate with disease severity in young patients with autosomal dominant polycystic kidney disease

    PubMed Central

    Reed, Berenice; Masoumi, Amirali; Elhassan, Elwaleed; McFann, Kim; Cadnapaphornchai, Melissa; Maahs, David; Snell-Bergeon, Janet; Schrier, Robert W.

    2013-01-01

    Renal cysts, pain and hematuria are common presentations of autosomal dominant polycystic kidney disease (ADPKD) in children. Renal function, however, is typically preserved in these patients despite increased renal volume. Since angiogenesis has been implicated in promotion of renal cyst growth in ADPKD we measured the serum level of various angiogenic factors and early renal structural changes and cardiovascular parameters in 71 patients with ADPKD with a mean age of 16 years. Renal structure and left ventricular mass index were measured by magnetic resonance imaging or by echocardiogram. Renal function was assessed by creatinine clearance, and urinary protein excretion. Serum growth factor levels were measured by enzyme-linked immunosorbent assay. Because of skewed distributions, the various parameters are reported as log10. Serum Log10 vascular endothelial growth factor was positively correlated with renal and cardiac structure, but negatively correlated with creatinine clearance. Serum angiopoietin 1 levels significantly correlated with structural change in both the kidney and the heart and with urinary protein. Thus, the correlation between angiogenic growth factors with both renal and cardiac disease severity is compatible with a possible role for angiogenesis in the early progression of disease in ADPKD. PMID:20881939

  11. [Correlations between the patient's genotype and the course of the disease in hereditary cancer diseases].

    PubMed

    Bisgaard, Marie Luise

    2006-06-12

    The majority of hereditary cancers demonstrate extended variation of phenotype, not only between families, but also within a particular family, who supposedly have inherited the same pathogenic mutation. The present paper reviews genotype-phenotype correlations with examples mainly from the two largest hereditary cancer groups, namely hereditary colorectal cancer and hereditary breast (and ovarian) cancer. It is concluded that knowledge about genotype-phenotype correlations is essential in the cancer genetic clinic; otherwise, the patient tends to base decisions on personal experience, not realising that the course of disease might be different in other family members. PMID:16822416

  12. Angiogenic growth factors correlate with disease severity in young patients with autosomal dominant polycystic kidney disease.

    PubMed

    Reed, Berenice Y; Masoumi, Amirali; Elhassan, Elwaleed; McFann, Kim; Cadnapaphornchai, Melissa A; Maahs, David M; Snell-Bergeon, Janet K; Schrier, Robert W

    2011-01-01

    Renal cysts, pain, and hematuria are common presentations of autosomal dominant polycystic kidney disease (ADPKD) in children. Renal function, however, is typically preserved in these patients despite increased renal volume. Since angiogenesis has been implicated in promotion of renal cyst growth in ADPKD, we measured the serum level of various angiogenic factors and early renal structural changes and cardiovascular parameters in 71 patients with ADPKD, with a mean age of 16 years. Renal structure and left ventricular mass index were measured by magnetic resonance imaging or by echocardiogram. Renal function was assessed by creatinine clearance and urinary protein excretion. Serum growth factor levels were measured by enzyme-linked immunosorbent assay. Because of skewed distributions, the various parameters are reported as log(10). Serum log(10) vascular endothelial growth factor was positively correlated with renal and cardiac structure, but negatively with creatinine clearance. Serum angiopoietin 1 levels significantly correlated with structural change in both the kidney and the heart and with urinary protein. Thus, the correlation between angiogenic growth factors with both renal and cardiac disease severity is compatible with a possible role for angiogenesis in the early progression of disease in ADPKD. PMID:20881939

  13. Correlating Photoreceptor Mosaic Structure to Clinical Findings in Stargardt Disease

    PubMed Central

    Razeen, Moataz M.; Cooper, Robert F.; Langlo, Christopher S.; Goldberg, Mara R.; Wilk, Melissa A.; Han, Dennis P.; Connor, Thomas B.; Fishman, Gerald A.; Collison, Frederick T.; Sulai, Yusufu N.; Dubra, Alfredo; Carroll, Joseph; Stepien, Kimberly E.

    2016-01-01

    Purpose To demonstrate a method for correlating photoreceptor mosaic structure with optical coherence tomography (OCT) and microperimetry findings in patients with Stargardt disease. Methods A total of 14 patients with clinically diagnosed Stargardt disease were imaged using confocal and split-detection adaptive optics scanning light ophthalmoscopy. Cone photoreceptors were identified manually in a band along the temporal meridian. Resulting values were compared to a normative database (n = 9) to generate cone density deviation (CDD) maps. Manual measurement of outer nuclear layer plus Henle fiber layer (ONL+HFL) thickness was performed, in addition to determination of the presence of ellipsoid zone (EZ) and interdigitation zone (IZ) bands on OCT. These results, along with microperimetry data, were overlaid with the CDD maps. Results Wide variation in foveal structure and CDD maps was seen within this small group. Disruption of ONL+HFL and/or IZ band was seen in all patients, with EZ band preservation in regions with low cone density in 38% of locations analyzed. Normality of retinal lamellar structure on OCT corresponded with cone density and visual function at 50/78 locations analyzed. Outer retinal tubulations containing photoreceptor-like structures were observed in 3 patients. Conclusions The use of CDD color-coded maps enables direct comparison of cone mosaic local density with other measures of retinal structure and function. Larger normative datasets and improved tools for automation of image alignment are needed. Translational Relevance The approach described facilitates comparison of complex multimodal data sets from patients with inherited retinal degeneration, and can be expanded to incorporate other structural imaging or functional testing. PMID:26981328

  14. Radiological-Pathological Analysis of WHO, RECIST, EASL, mRECIST and DWI: Imaging Analysis from a Prospective Randomized Trial of Y90 +/− Sorafenib

    PubMed Central

    Vouche, Michael; Kulik, Laura; Atassi, Rohi; Memon, Khairuddin; Hickey, Ryan; Ganger, Daniel; Miller, Frank H.; Yaghmai, Vahid; Abecassis, Michael; Baker, Talia; Mulcahy, Mary; Nayar, Ritu; Lewandowski, Robert J; Salem, Riad

    2013-01-01

    Aim To compare radiological and pathological changes and test the adjunct efficacy of Sorafenib to Y90 as a bridge to transplantation in HCC. Methods 15 patients with 16 HCC lesions randomized to Y90 without (Group A, n=9) or with Sorafenib (Group B, n=7). Size (WHO, RECIST), enhancement (EASL, mRECIST) and diffusion-weighted imaging criteria (ADC) measurements were obtained at baseline, 1 and every 3 months after treatment until transplantation. Percentage necrosis in explanted tumors was correlated with imaging findings. Results 100%, 50-99% and <50% pathological necrosis was observed in 6 (67%), 1 (11%) and 2 (22%) tumors in Group A and 3 (42%), 2 (28%) and 2 (28%) in Group B, respectively (p=0.81). While ADC (p=0.46) did not change after treatment, WHO (p=0.06) and RECIST (p=0.08) response at 1 month failed to reach significance, but significant responses by EASL (p<0.01/0.03) and mRECIST (p<0.01/0.03) at 1 and 3 months were observed. Response was equivalent by EASL or mRECIST. No difference in response rates were observed between groups A and B at 1 and 3 months by WHO, RECIST, EASL, mRECIST or ADC measurements. Despite failing to reach significance, smaller baseline size was associated with CPN (RECIST: p=0.07; WHO: p=0.05). However, a cut-off size of 35 mm was predictive of CPN (p=0.005). CPN could not be predicted by WHO (p=0.25 and 0.62), RECIST (p=0.35 and 0.54), EASL (p=0.49 and 0.46), mRECIST (p=0.49 and 0.60) or ADC (p=0.86 and 0.93). Conclusion The adjunct of Sorafenib did not augment radiological or pathological response to Y90 therapy for HCC. Equivalent significant reduction in enhancement at 1 and 3 months by EASL/mRECIST were noted. Neither EASL nor mRECIST could reliably predict CPN. PMID:23703789

  15. Neuroanatomical correlates of cognitive self-appraisal in neurodegenerative disease.

    PubMed

    Rosen, Howard J; Alcantar, Oscar; Rothlind, Johannes; Sturm, Virginia; Kramer, Joel H; Weiner, Michael; Miller, Bruce L

    2010-02-15

    Self-appraisal is a critical cognitive function, which helps us to choose tasks based on an accurate assessment of our abilities. The neural mechanisms of self-appraisal are incompletely understood, although a growing body of literature suggests that several frontal and subcortical regions are important for self-related processing. Anosognosia, or lack of awareness of one's deficits, is common in neurodegenerative dementias, offering an important window onto the brain systems involved in self-appraisal. We examined the neuroanatomical basis of self-appraisal in a mixed group of 39 individuals, including 35 with cognitive impairment due to one of several probable neurodegenerative diseases, using voxel-based morphometry and an objective, neuropsychologically-based measure of self-appraisal accuracy. Self-appraisal accuracy was correlated with tissue content in the right ventromedial prefrontal cortex (vmPFC). We hypothesize that emotional/physiological processing carried out by vmPFC is an important factor mediating self-appraisal accuracy in dementia. PMID:19961939

  16. Neuroanatomical correlates of cognitive self-appraisal in neurodegenerative disease

    PubMed Central

    Rosen, Howard J.; Alcantar, Oscar; Rothlind, Johannes; Sturm, Virginia; Kramer, Joel H.; Weiner, Michael; Miller, Bruce L.

    2009-01-01

    Self-appraisal is a critical cognitive function, which helps us to choose tasks based on an accurate assessment of our abilities. The neural mechanisms of self-appraisal are incompletely understood, although a growing body of literature suggests that several frontal and subcortical regions are important for self-related processing. Anosognosia, or lack of awareness of one's deficits, is common in neurodegenerative dementias, offering an important window onto the brain systems involved in self-appraisal. We examined the neuroanatomical basis of self-appraisal in a mixed group of 39 individuals, including 35 with cognitive impairment due to one of several probable neurodegenerative diseases, using voxel-based morphometry and an objective, neuropsychologically-based measure of self-appraisal accuracy. Self-appraisal accuracy was correlated with tissue content in the right ventromedial prefrontal cortex (vmPFC). We hypothesize that emotional/physiological processing carried out by vmPFC is an important factor mediating self-appraisal accuracy in dementia. PMID:19961939

  17. Decreased RECQL5 correlated with disease progression of osteosarcoma.

    PubMed

    Wu, Junlong; Zhi, Liqiang; Dai, Xin; Cai, Qingchun; Ma, Wei

    2015-11-27

    Human RecQ helicase family, consisting of RECQL, RECQL4, RECQL5, BLM and WRN, has critical roles in genetic stability and tumorigenesis. Although RECQL5 has been reported to correlate with the susceptibility to malignances including osteosarcoma, the specific effect on tumor genesis and progression is not yet clarified. Here we focused on the relationship between RECQL5 expression and osteosarcoma disease progression, and further investigated the function of RECQL5 on MG-63 cell proliferation and apoptosis. By immunohistochemical analysis, qRT-PCR and western blot, we found that RECQL5 expression was downregulated in osteosarcoma tissues and cells. Patients with advanced tumor stage and low grade expressed lower RECQL5. To construct a stable RECQL5 overexpression osteosarcoma cell line (MG-63-RECQL5), RECQL5 gene was inserted into the human AAVS1 safe harbor by CRISPR/Cas9 system. The overexpression of RECQL5 was verified by qRT-PCR and western blot. Cell proliferation, cell cycle and apoptosis assay revealed that RECQL5 overexpression inhibited proliferation, induced G1-phase arrest and promoted apoptosis in MG-63 cells. Collectively, our results suggested RECQL5 as a tumor suppressor in osteosarcoma and may be a potential therapeutic target for osteosarcoma treatment.

  18. Coats' disease: CT-pathologic correlation in two cases

    SciTech Connect

    Sherman, J.L.; McLean, I.W.; Brallier, D.R.

    1983-01-01

    Computerized axial tomography (CT) of the orbit in two children with Coats' disease demonstrated increased density in the globe. This finding was attributed to the subretinal accumulation of exudate that is a characteristic of this disease. The CT appearance of Coats' disease cannot yet be differentiated from retinoblastoma or other diseases with retinal telangiectasis.

  19. Cervical Castleman disease: CT study with angiographic correlation.

    PubMed

    Koslin, D B; Berland, L L; Sekar, B C

    1986-07-01

    The computed tomographic (CT) appearance, angiographic appearance, and clinical features of a patient with cervical Castleman disease, an uncommon disease of benign lymph node hyperplasia, are reported. CT scans showed a densely enhancing cervical mass. On external carotid angiography, the mass was seen as hypervascular with a capillary blush. Differential diagnosis included carotid body chemodectoma, vagal neuroma, tumor of the salivary gland, tuberculous adenitis and other granulomatous diseases, inflammatory lymph nodes, metastatic disease, and lymphoma.

  20. Human keratin diseases: the increasing spectrum of disease and subtlety of the phenotype-genotype correlation.

    PubMed

    Irvine, A D; McLean, W H

    1999-05-01

    Keratins are obligate heterodimer proteins that form the intermediate filament cytoskeleton of all epithelial cells. Keratins are tissue and differentiation specific and are expressed in pairs of types I and II proteins. The spectrum of inherited human keratin diseases has steadily increased since the causative role of mutations in the basal keratinocyte keratins 5 and 14 in epidermolysis bullosa simplex (EBS) was first reported in 1991. At the time of writing, mutations in 15 epithelial keratins and two trichocyte keratins have been associated with human diseases which include EBS, bullous congenital ichthyosiform erythroderma, epidermolytic palmoplantar keratoderma, ichthyosis bullosa of Siemens, diffuse and focal non-epidermolytic palmoplantar keratoderma, pachyonychia congenita and monilethrix. Mutations in extracutaneous keratins have been reported in oral white sponge naevus and Meesmann's corneal dystrophy. New subtleties of phenotype-genotype correlation are emerging within the keratin diseases with widely varying clinical presentations attributable to similar mutations within the same keratin. Mutations in keratin-associated proteins have recently been reported for the first time. This article reviews clinical, ultrastructural and molecular aspects of all the keratin diseases described to date and delineates potential future areas of research in this field.

  1. Putaminal Diffusivity Correlates With Disease Progression in Parkinson's Disease: Prospective 6-Year Study.

    PubMed

    Chan, Ling-Ling; Ng, Kia-Min; Yeoh, Chooi-Sum; Rumpel, H; Li, Hui-Hua; Tan, Eng-King

    2016-02-01

    Diffusion tensor imaging (DTI) is an increasingly used noninvasive imaging tool. However its long-term clinical utility is unclear. Parkinson's disease (PD) is a common neurodegenerative disease.We prospectively examined a cohort of 46 Parkinson's disease (PD) patients who underwent diffusion tensor imaging (DTI) of the brain at baseline and 6 years later on a 1.5 Tesla scanner using a standardized protocol. DTI parameters of mean diffusivity (MD) and fractional anisotrophy (FA) were extracted using regions-of-interest (ROIs) analysis from various brain regions.Compared to the baseline scan, MD increased in all brain regions (P < 0.0001). FA increased in the substantia nigra and posterior putamen, but decreased in the frontal white matter (P < 0.0001). Linear regression analysis demonstrated that the MD in the anterior putamen increased 11.6 units (95% CI = [4.71, 18.43]) (P = 0.0003) for every unit increase of United PD Rating Scale (UPDRS).Our 6-year prospective longitudinal study demonstrated increased diffusivity in all brain regions and that in the anterior putamen correlated with disease progression. Serial diffusion data may be useful as an additional objective in vivo biomarker for motor progression in PD.

  2. Rainbow Trout (Oncorhynchus mykiss) resistance to columnaris disease is heritable and favorably correlated with bacterial cold water disease resistance

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Columnaris disease (CD) is an emerging disease affecting rainbow trout aquaculture. Objectives were to estimate heritability of CD resistance in a line (ARS-Fp-R) selected 4 generations for improved bacterial cold water disease (BCWD) resistance; estimate genetic correlations among CD resistance, BC...

  3. Disease activity in idiopathic pulmonary fibrosis: CT and pathologic correlation.

    PubMed

    Müller, N L; Staples, C A; Miller, R R; Vedal, S; Thurlbeck, W M; Ostrow, D N

    1987-12-01

    Computed tomography (CT) scans were compared with pathologic determinants of disease activity in 12 patients with idiopathic pulmonary fibrosis. The theory was that intraalveolar and interstitial cellularity would result in areas of opacification of air spaces on CT scans. All patients underwent open lung biopsy, and disease activity was assessed with a pathologic grading system. Seven patients had mild disease activity, five had moderate to marked disease activity. Opacification of air spaces was patchy in distribution, predominantly peripheral, and seen better on 1.5-mm rather than 10-mm collimation scans. Disease activity on CT scans was graded independently from 0 to 3 based on the presence and relative density of the areas of air space consolidation compared with the surrounding parenchyma. The pathologic score was significantly greater in the patients with high CT scores than in those with low CT scores (P = .001). Five patients with marked disease activity and five of seven patients with mild disease activity were correctly identified. CT may be useful in the assessment of disease activity in idiopathic pulmonary fibrosis.

  4. Phenotype-genotype correlations in patients with Wilson's disease.

    PubMed

    Ferenci, Peter

    2014-05-01

    There is considerable phenotypic variation in Wilson's disease (WD). Some patients present with hepatic disease during the first decade of life and some with neurological degeneration in adolescence or adult life, with or without overt liver disease. Although the absence of neurologic disease in patients with liver disease in childhood or adolescence can be explained by the limited time exposure of the central nervous system to copper toxicity, it is surprising that late-onset neurologic WD can occur without any evidence of liver involvement. This huge variability in the clinical presentation of WD in general reflects our limited knowledge on the natural history of WD. Genetic association studies require the phenotype to be defined as accurately as possible.

  5. Progressive diaphyseal dysplasia (Engelmann disease): scintigraphic-radiographic-clinical correlations

    SciTech Connect

    Kumar, B.; Murphy, W.A.; Whyte, M.P.

    1981-07-01

    Four patients (2 males, 2 females; ages 15-47 yrs.) with variable clinical, radiographic, and scintigraphic manifestations of progressive diaphyseal dysplasia (PDD) or Engelmann disease were studied with 99mTc methylene diphosphonate bone imaging and radiographic skeletal surveys. Comparison of the results of the two imaging procedures showed that some affected bones were scintigraphically normal but radiographically abnormal and vice versa. These findings suggest that the lesions of PDD may mature, causing a significant decrease in disease activity, and that abnormally increased radiopharmaceutical accumulation during bone scintigraphy appears to be a sensitive indicator of disease activity.

  6. Cerebral correlates of psychotic syndromes in neurodegenerative diseases

    PubMed Central

    Jellinger, Kurt A

    2012-01-01

    Abstract Psychosis has been recognized as a common feature in neurodegenerative diseases and a core feature of dementia that worsens most clinical courses. It includes hallucinations, delusions including paranoia, aggressive behaviour, apathy and other psychotic phenomena that occur in a wide range of degenerative disorders including Alzheimer’s disease, synucleinopathies (Parkinson’s disease, dementia with Lewy bodies), Huntington’s disease, frontotemporal degenerations, motoneuron and prion diseases. Many of these psychiatric manifestations may be early expressions of cognitive impairment, but often there is a dissociation between psychotic/behavioural symptoms and the rather linear decline in cognitive function, suggesting independent pathophysiological mechanisms. Strictly neuropathological explanations are likely to be insufficient to explain them, and a large group of heterogeneous factors (environmental, neurochemical changes, genetic factors, etc.) may influence their pathogenesis. Clinico-pathological evaluation of behavioural and psychotic symptoms (PS) in the setting of neurodegenerative and dementing disorders presents a significant challenge for modern neurosciences. Recognition and understanding of these manifestations may lead to the development of more effective preventive and therapeutic options that can serve to delay long-term progression of these devastating disorders and improve the patients’ quality of life. A better understanding of the pathophysiology and distinctive pathological features underlying the development of PS in neurodegenerative diseases may provide important insights into psychotic processes in general. PMID:21418522

  7. Chronic Pancreatitis Correlates With Increased Risk of Cerebrovascular Disease

    PubMed Central

    Wong, Tuck-Siu; Liao, Kuan-Fu; Lin, Chi-Ming; Lin, Cheng-Li; Chen, Wen-Chi; Lai, Shih-Wei

    2016-01-01

    Abstract The aim of this study is to explore whether there is a relationship between chronic pancreatitis and cerebrovascular disease in Taiwan. Using the claims data of the Taiwan National Health Insurance Program, we identified 16,672 subjects aged 20 to 84 years with a new diagnosis of chronic pancreatitis from 2000 to 2010 as the chronic pancreatitis group. We randomly selected 65,877 subjects aged 20 to 84 years without chronic pancreatitis as the nonchronic pancreatitis group. Both groups were matched by sex, age, comorbidities, and the index year of diagnosing chronic pancreatitis. The incidence of cerebrovascular disease at the end of 2011 was measured. The multivariable Cox proportional hazards regression model was used to measure the hazard ratio (HR) and 95% confidence interval (CI) for cerebrovascular disease risk associated with chronic pancreatitis and other comorbidities. The overall incidence of cerebrovascular disease was 1.24-fold greater in the chronic pancreatitis group than that in the nonchronic pancreatitis group (14.2 vs. 11.5 per 1000 person-years, 95% CI = 1.19–1.30). After controlling for confounding factors, the adjusted HR of cerebrovascular disease was 1.27 (95% CI = 1.19–1.36) for the chronic pancreatitis group as compared with the nonchronic pancreatitis group. Woman (adjusted HR = 1.41, 95% CI = 1.31–1.51), age (every 1 year, HR = 1.04, 95% CI = 1.04–1.05), atrial fibrillation (adjusted HR = 1.23, 95% CI = 1.02–1.48), chronic kidney disease (adjusted HR = 1.48, 95% CI = 1.31–1.67), chronic obstructive pulmonary disease (adjusted HR = 1.27, 95% CI = 1.16–1.40), diabetes mellitus (adjusted HR = 1.82, 95% CI = 1.72–1.92), hypertension (adjusted HR = 1.66, 95% CI = 1.56–1.76), and peripheral atherosclerosis (adjusted HR = 1.26, 95% CI = 1.06–1.51) were other factors significantly associated with cerebrovascular disease. Chronic pancreatitis is

  8. Clinical and radiological correlations in patients with gestational trophoblastic disease*

    PubMed Central

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Câmara Medeiros; Maestá, Izildinha; Amim Junior, Joffre; de Rezende Filho, Jorge Fonte; Montenegro, Carlos Antonio Barbosa; Braga, Antônio

    2016-01-01

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging. PMID:27777478

  9. Linguistic Correlates of Asymmetric Motor Symptom Severity in Parkinson's Disease

    ERIC Educational Resources Information Center

    Holtgraves, Thomas; McNamara, Patrick; Cappaert, Kevin; Durso, Raymond

    2010-01-01

    Asymmetric motor severity is common in Parkinson's Disease (PD) and provides a method for examining the neurobiologic mechanisms underlying cognitive and linguistic deficits associated with the disorder. In the present research, PD participants (N = 31) were assessed in terms of the asymmetry of their motor symptoms. Interviews with the…

  10. An Examination of the Demographic and Environmental Variables Correlated with Lyme Disease Emergence in Virginia.

    PubMed

    Seukep, Sara E; Kolivras, Korine N; Hong, Yili; Li, Jie; Prisley, Stephen P; Campbell, James B; Gaines, David N; Dymond, Randel L

    2015-12-01

    Lyme disease is the United States' most significant vector-borne illness. Virginia, on the southern edge of the disease's currently expanding range, has experienced an increase in Lyme disease both spatially and temporally, with steadily increasing rates over the past decade and disease spread from the northern to the southwestern part of the state. This study used a Geographic Information System and a spatial Poisson regression model to examine correlations between demographic and land cover variables, and human Lyme disease from 2006 to 2010 in Virginia. Analysis indicated that herbaceous land cover is positively correlated with Lyme disease incidence rates. Areas with greater interspersion between herbaceous and forested land were also positively correlated with incidence rates. In addition, income and age were positively correlated with incidence rates. Levels of development, interspersion of herbaceous and developed land, and population density were negatively correlated with incidence rates. Abundance of forest fragments less than 2 hectares in area was not significantly correlated. Our results support some findings of previous studies on ecological variables and Lyme disease in endemic areas, but other results have not been found in previous studies, highlighting the potential contribution of new variables as Lyme disease continues to emerge southward. PMID:26163019

  11. An Examination of the Demographic and Environmental Variables Correlated with Lyme Disease Emergence in Virginia.

    PubMed

    Seukep, Sara E; Kolivras, Korine N; Hong, Yili; Li, Jie; Prisley, Stephen P; Campbell, James B; Gaines, David N; Dymond, Randel L

    2015-12-01

    Lyme disease is the United States' most significant vector-borne illness. Virginia, on the southern edge of the disease's currently expanding range, has experienced an increase in Lyme disease both spatially and temporally, with steadily increasing rates over the past decade and disease spread from the northern to the southwestern part of the state. This study used a Geographic Information System and a spatial Poisson regression model to examine correlations between demographic and land cover variables, and human Lyme disease from 2006 to 2010 in Virginia. Analysis indicated that herbaceous land cover is positively correlated with Lyme disease incidence rates. Areas with greater interspersion between herbaceous and forested land were also positively correlated with incidence rates. In addition, income and age were positively correlated with incidence rates. Levels of development, interspersion of herbaceous and developed land, and population density were negatively correlated with incidence rates. Abundance of forest fragments less than 2 hectares in area was not significantly correlated. Our results support some findings of previous studies on ecological variables and Lyme disease in endemic areas, but other results have not been found in previous studies, highlighting the potential contribution of new variables as Lyme disease continues to emerge southward.

  12. Symptoms and their correlates in chronic kidney disease.

    PubMed

    Weisbord, Steven D

    2007-10-01

    While there is a significant body of literature documenting the impairments in health-related quality of life (HRQOL) experienced by patients with end-stage renal disease (ESRD), recent work has helped to elucidate the mediators of impaired well-being in this patient group. Physical and emotional symptoms have been shown to be common, frequently severe, and directly linked with impaired HRQOL. The following review explores the process of symptom assessment in patients with chronic kidney disease (CKD), presents an overview of the composite burden and importance of symptoms in patients with ESRD, highlights particularly common and distressing symptoms for which existing treatment strategies may be applicable, and discusses future directions for efforts to address and alleviate symptoms in the growing population of patients who suffer from CKD.

  13. Psychosocial correlates of survival in advanced malignant disease?

    PubMed

    Cassileth, B R; Lusk, E J; Miller, D S; Brown, L L; Miller, C

    1985-06-13

    Prospective studies of the general population have isolated specific social and psychological factors as independent predictors of longevity. This study assesses the ability of these factors, plus two others said to influence survival in patients with cancer, to predict survival and the time to relapse after a diagnosis of cancer. Patients with unresectable cancers (n = 204) were followed to determine the length of survival. Patients with Stage I or II melanoma or Stage II breast cancer (n = 155) were followed to determine the time to relapse. Analysis of data on these 359 patients indicates that social and psychological factors individually or in combination do not influence the length of survival or the time to relapse (P less than 0.10). The specific diagnosis (F = 2.0, P = 0.06), performance status (F = 0.66, P = 0.62), extent of disease (F = 1.12, P = 0.89), and therapy (F = 1.08, P = 0.35) were also unrelated to the psychosocial factors studied. Although these factors may contribute to the initiation of morbidity, the biology of the disease appears to predominate and to override the potential influence of life-style and psychosocial variables once the disease process is established.

  14. Cerebral correlates of psychotic symptoms in Alzheimer's disease

    PubMed Central

    Mega, M.; Lee, L.; Dinov, I.; Mishkin, F.; Toga, A.; Cummings, J.

    2000-01-01

    BACKGROUND—Psychotic symptoms are produced by distributed neuronal dysfunction. Abnormalities of reality testing and false inference implicate frontal lobe abnormalities.
OBJECTIVES—To identify the functional imaging profile of patients with Alzheimer's disease manifesting psychotic symptoms as measured by single photon emission computed tomography (SPECT).
METHODS—Twenty patients with Alzheimer's disease who had SPECT and clinical evaluations were divided into two equal groups with similar mini mental status examination (MMSE), age, sex, and the range of behaviours documented by the neuropsychiatric inventory (NPI), except delusions and hallucinations. SPECT studies, registered to a probabilistic anatomical atlas, were normalised across the combined group mean intensity level, and subjected to a voxel by voxel subtraction of the non-psychotic minus psychotic groups. Subvolume thresholding (SVT) corrected random lobar noise to produce a three dimensional functional significance map.
RESULTS—The significance map showed lower regional perfusion in the right and left dorsolateral frontal, left anterior cingulate, and left ventral striatal regions along with the left pulvinar and dorsolateral parietal cortex, in the psychotic versus non-psychotic group.
CONCLUSION—Patients with Alzheimer's disease who manifest psychosis may have disproportionate dysfunction of frontal lobes and related subcortical and parietal structures.

 PMID:10896687

  15. [Refsum's disease. Epidemiologic, clinical and biological correlation. 6 cases].

    PubMed

    Petit, H; Leys, D; Skjeldal, O H; Caron, J C; Lambert, P; Lehembre, P; Hache, J C

    1986-01-01

    Nine patients with symptoms and signs of Refsum's disease are reported. In 6 a systemic accumulation of phytanic acid was demonstrated, together with low phytanic acid oxidase activity in skin fibroblasts in 5 of them. In 3, no disorder of phytanic acid metabolism was demonstrated. In 3, the diagnosis was made during the pre-clinical period. The disease seems more frequent in Northern France, which agrees with the hypothesis of a genetic mutation which would have taken place in Scandinavia some centuries ago and was subsequently spread by the Vikings. The effects of a dietary treatment on serum phytanic acid levels and clinical disorders are reported. The general condition of the patients improved remarkably but only partially. The diet is unpalatable and in some patients the level of serum phytanic acid increased, due to the mobilization of body fat. Patients with very high levels of phytanic acid might be initially treated by plasmapheresis. For the same reason, the diet should supply enough calories to keep body weight unchanged, and body weight loss whatever its cause should be avoided.

  16. Linguistic Correlates of Asymmetric Motor Symptom Severity in Parkinson's Disease

    PubMed Central

    Holtgraves, Thomas; McNamara, Patrick; Cappaert, Kevin; Durso, Raymond

    2009-01-01

    Asymmetric motor severity is common in Parkinson's Disease (PD) and provides a method for examining the neurobiologic mechanisms underlying cognitive and linguistic deficits associated with the disorder. In the present research, PD participants (N = 31) were assessed in terms of the asymmetry of their motor symptoms. Interviews with the participants were analyzed with the Linguistic Inquiry and Word Count (LIWC) program. Three measures of linguistic complexity – the proportion of verbs, proportion of function words, and sentence length – were found to be affected by symptom asymmetry. Greater left-side motor severity (and hence greater right hemisphere dysfunction) was associated with the production of significantly fewer verbs, function words, and shorter sentences. Hence, the production of linguistic complexity in a natural language context was associated with relatively greater right hemisphere involvement. The potential neurobiological mechanisms underlying this effect are discussed. PMID:19751960

  17. Exploring homogeneity of correlation structures of gene expression datasets within and between etiological disease categories.

    PubMed

    Jong, Victor L; Novianti, Putri W; Roes, Kit C B; Eijkemans, Marinus J C

    2014-12-01

    The literature shows that classifiers perform differently across datasets and that correlations within datasets affect the performance of classifiers. The question that arises is whether the correlation structure within datasets differ significantly across diseases. In this study, we evaluated the homogeneity of correlation structures within and between datasets of six etiological disease categories; inflammatory, immune, infectious, degenerative, hereditary and acute myeloid leukemia (AML). We also assessed the effect of filtering; detection call and variance filtering on correlation structures. We downloaded microarray datasets from ArrayExpress for experiments meeting predefined criteria and ended up with 12 datasets for non-cancerous diseases and six for AML. The datasets were preprocessed by a common procedure incorporating platform-specific recommendations and the two filtering methods mentioned above. Homogeneity of correlation matrices between and within datasets of etiological diseases was assessed using the Box's M statistic on permuted samples. We found that correlation structures significantly differ between datasets of the same and/or different etiological disease categories and that variance filtering eliminates more uncorrelated probesets than detection call filtering and thus renders the data highly correlated.

  18. Correlations of regional postmortem enzyme activities with premortem local glucose metabolic rates in Alzheimer's disease.

    PubMed

    McGeer, E G; McGeer, P L; Harrop, R; Akiyama, H; Kamo, H

    1990-12-01

    Correlations were sought between local cerebral metabolic rates (LCMRs) for glucose in various regions of the cortex, determined in premortem PET scans, with the regional activities of choline acetyltransferase (ChAT), acetylcholinesterase (AChE), beta-glucuronidase (Gluc, a probable index of reactive gliosis), and phosphate-activated glutaminase (PAG, a possible indice of the large pyramidal neurons) measured on postmortem tissue. Significant negative correlations between LCMRs and Gluc activities were found in 6 PET-scanned cases of Alzheimer disease (AD), and positive correlations of LCMRs with PAG were found in 5. By contrast, a positive correlation with ChAT and AChE was found in only 1. The results are consistent with the metabolic deficits in AD being primarily a reflection of local neuronal loss and gliosis. Similar data on two cases of Huntington's disease showed no significant correlations, while 1 patient with Parkinson dementia showed a significant (negative) correlation only with Gluc.

  19. Neural correlates of anosognosia for cognitive impairment in Alzheimer's disease.

    PubMed

    Salmon, Eric; Perani, Daniela; Herholz, Karl; Marique, Patricia; Kalbe, Elke; Holthoff, Vjera; Delbeuck, Xavier; Beuthien-Baumann, Bettina; Pelati, Oriana; Lespagnard, Solange; Collette, Fabienne; Garraux, Gaëtan

    2006-07-01

    We explored the neural substrate of anosognosia for cognitive impairment in Alzheimer's disease (AD). Two hundred nine patients with mild to moderate dementia and their caregivers assessed patients' cognitive impairment by answering a structured questionnaire. Subjects rated 13 cognitive domains as not impaired or associated with mild, moderate, severe, or very severe difficulties, and a sum score was calculated. Two measures of anosognosia were derived. A patient's self assessment, unconfounded by objective measurements of cognitive deficits such as dementia severity and episodic memory impairment, provided an estimate of impaired self-evaluative judgment about cognition in AD. Impaired self-evaluation was related to a decrease in brain metabolism measured with 18F-2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) in orbital prefrontal cortex and in medial temporal structures. In a cognitive model of anosognosia, medial temporal dysfunction might impair a comparison mechanism between current information on cognition and personal knowledge. Hypoactivity in orbitofrontal cortex may not allow AD patients to update the qualitative judgment associated with their impaired cognitive abilities. Caregivers perceived greater cognitive impairments than patients did. The discrepancy score between caregiver's and patient's evaluations, an other measure of anosognosia, was negatively related to metabolic activity located in the temporoparietal junction, consistent with an impairment of self-referential processes and perspective taking in AD. PMID:16247783

  20. Neural correlates of spelling difficulties in Alzheimer's disease.

    PubMed

    Rodríguez-Ferreiro, Javier; Martínez, Carmen; Pérez-Carbajal, Ana-Julia; Cuetos, Fernando

    2014-12-01

    Alzheimer's disease (AD) is associated with a general cognitive decline that affects the memory and language domains. Thus, an oral production deficit with a lexical-semantic origin has been widely observed in these patients. Their written production capacities, however, have been much less studied. We assessed the spelling abilities of 22 AD patients and a group of matched healthy controls with a test battery including written picture naming and word and pseudoword dictation tests, as well as text dictation and spontaneous writing tasks. The results of the AD patients in the discriminative tasks were then entered into voxel-based morphometry analyses along with their grey matter volumes. The patient group presented a selective impairment for word dictation, which contrasted with a spared capacity to spell pseudowords, and showed more difficulties for words with arbitrary and rule-based orthography. Moreover, they also produced less complete syntactic units in the spontaneous writing task. These results point out the lexical-semantic, as opposed to sublexical, nature of the spelling deficit associated to AD. In addition, we recognized a mainly left-lateralized cortical network, including areas in the posterior inferior temporal lobe and the superior region of the parietal cortex, which might be responsible for this impairment. Other regions, such as the putamen, were also associated to the deficit. The results of this study, hence, improve our understanding of the neuropsychological and neuroanatomical mechanisms that underlie the cognitive symptoms associated to AD. PMID:25447060

  1. Hemodynamic Correlates of Proteinuria in Chronic Kidney Disease

    PubMed Central

    Townsend, Raymond R.; Fink, Jeffrey C.; Teal, Valerie; Anderson, Cheryl; Appel, Lawrence; Chen, Jing; He, Jiang; Litbarg, Natasha; Ojo, Akinlolu; Rahman, Mahboob; Rosen, Leigh; Sozio, Stephen M.; Steigerwalt, Susan; Strauss, Louise; Joffe, Marshall M.

    2011-01-01

    Summary Background and objectives Brachial artery measures of BP are associated with increasing degrees of proteinuria. Whether central measures of BP or vascular stiffness are associated with increased risk of proteinuria in patients with chronic kidney disease (CKD) is unknown. Design, setting, participants, & measurements Measurements of central and brachial artery BP, and aortic pulse wave velocity (PWV) were performed in a cross-sectional cohort of patients with CKD (n = 2144) from the Chronic Renal Insufficiency Cohort (CRIC) study to determine factors which predict increased risk of proteinuria. Multivariate analysis stratified by diabetes included age, ethnicity, gender, estimated glomerular filtration rate (GFR), waistline, smoking, heart rate, and medications to evaluate the relationship of hemodynamic factors and proteinuria. Results Brachial artery systolic BP (SBP) was important as an explanatory factor for variations in proteinuria among both diabetics (R2 = 0.40, P < 0.0001) and non diabetics (R2 = 0.38, P < 0.001). Measures of peripheral pulse pressure (PP), central SBP, and central pulse pressure added little to the explained variation in proteinuria beyond brachial artery SBP, whereas PWV as a measure of vascular stiffness incrementally accounted for a significant portion of variation in proteinuria beyond that explained by brachial artery SBP in diabetics (R2 = 0.42, P < 0.001) but not non diabetics. Conclusions Brachial artery SBP and PWV are both associated with variations in proteinuria in patients with CKD. PMID:21852669

  2. Examination of the Spatial Correlation of Statistics Information in the Ultrasonic Echo from Diseased Liver

    NASA Astrophysics Data System (ADS)

    Yamaguchi, Tadashi; Hachiya, Hiroyuki; Kamiyama, Naohisa; Moriyasu, Fuminori

    2002-05-01

    To realize a quantitative diagnosis of liver cirrhosis, we have been analyzing the characteristics of echo amplitude in B-mode images. Realizing the distinction between liver diseases such as liver cirrhosis and chronic hepatitis is required in the field of medical ultrasonics. In this study, we examine the spatial correlation, with the coefficient of correlation between the frames and the amplitude characteristics of each frame, using the volumetric data of RF echo signals from normal and diseased liver. It is found that there is a relationship between the tissue structure of liver and the spatial correlation of echo information.

  3. No Geographic Correlation between Lyme Disease and Death Due to 4 Neurodegenerative Disorders, United States, 2001-2010.

    PubMed

    Forrester, Joseph D; Kugeler, Kiersten J; Perea, Anna E; Pastula, Daniel M; Mead, Paul S

    2015-11-01

    Associations between Lyme disease and certain neurodegenerative diseases have been proposed, but supportive evidence for an association is lacking. Similar geographic distributions would be expected if 2 conditions were etiologically linked. Thus, we compared the distribution of Lyme disease cases in the United States with the distributions of deaths due to Alzheimer disease, amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), and Parkinson disease; no geographic correlations were identified. Lyme disease incidence per US state was not correlated with rates of death due to ALS, MS, or Parkinson disease; however, an inverse correlation was detected between Lyme disease and Alzheimer disease. The absence of a positive correlation between the geographic distribution of Lyme disease and the distribution of deaths due to Alzheimer disease, ALS, MS, and Parkinson disease provides further evidence that Lyme disease is not associated with the development of these neurodegenerative conditions.

  4. The Correlation of Serum IL-12B Expression With Disease Activity in Patients With Inflammatory Bowel Disease

    PubMed Central

    Lee, Hye Won; Chung, Sook Hee; Moon, Chang Mo; Che, Xiumei; Kim, Seung Won; Park, Soo Jung; Hong, Sung Pil; Kim, Tae Il; Kim, Won Ho; Cheon, Jae Hee

    2016-01-01

    Abstract Genetic variants in IL12B, encoding the p40 subunit common in interleukin-12 (IL-12) and interleukin-23, were identified as the susceptibility loci for inflammatory bowel disease (IBD). This study aimed to identify the correlation of serum IL-12B expression with disease activity in patients with IBD and evaluate the possibility of IL-12B as a biomarker for assessing inflammatory status in IBD. A total of 102 patients with IBD, including 38, 32, and 32 patients with Crohn's disease (CD), ulcerative colitis (UC), and intestinal Behçet's disease (intestinal BD), respectively, were included. The clinical and laboratory data from the patients were collected at the time of serum IL-12B measurement. Serum IL-12B levels were measured using an enzyme-linked immunosorbent assay. The median IL-12B levels in patients with CD, UC, and intestinal BD were significantly higher than those in controls (1.87, 2.74, and 2.73 pg/mL, respectively, vs. 1.42 pg/mL, all P <0.05). IL-12B concentrations were associated with disease activity in patients with UC and intestinal BD but not in those with CD. IL-12B levels were increased with increasing disease activity in patients with UC (P <0.001). Likewise, patients with active intestinal BD had higher IL-12B levels than those without active disease (P = 0.008). IL-12B levels were correlated with the endoscopic disease activity of UC (P = 0.002) and intestinal BD (P = 0.001) but not that of CD. Serum IL-12B levels were significantly correlated with clinical and endoscopic disease activity in patients with UC and intestinal BD, suggesting its potential use as a biomarker for assessing disease activity in these patients. PMID:27281077

  5. Does APO ε4 correlate with MRI changes in Alzheimer's disease?

    PubMed Central

    Doody, R; Azher, S; Haykal, H; Dunn, J; Liao, T; Schneider, L

    2000-01-01

    OBJECTIVE—To assess the relation between APO E genotype and MRI white matter changes in Alzheimer's disease. The APO ε4 allele is correlated with amyloid angiopathy and other neuropathologies in Alzheimer's disease and could be associated with white matter changes. If so, there should be a dose effect.
METHODS—104 patients with probable Alzheimer's disease (NINCDS-ADRDA criteria) in this Alzheimer's Disease Research Centre were studied. Patients received MRI and APO E genotyping by standardised protocols. Axial MRI was scored (modified Schelten's scale) for the presence and degree of white matter changes and atrophy in several regions by a neuroradiologist blinded to genotype. Total white matter and total atrophy scores were also generated. Data analysis included Pearson's correlation for regional and total imaging scores and analysis of variance (ANOVA) (or Kruskal-Wallis) and χ2 for demographic and disease related variables.
RESULTS—30 patients had no ε4, 53 patients were heterozygous, and 21 patients were homozygous. The three groups did not differ in sex distribution, age of onset, age at MRI, MMSE, clinical dementia rating, or modified Hachinski ischaemia scores. There were no significant correlations between total or regional white matter scores and APO E genotype (Pearson correlation).
CONCLUSIONS—No correlation between total or regional white matter scores and APO E genotype was found. The pathogenesis of white matter changes in Alzheimer's disease may be independent of APO E genotype.

 PMID:11032626

  6. Altered fractal dynamics of gait: reduced stride-interval correlations with aging and Huntington's disease

    NASA Technical Reports Server (NTRS)

    Hausdorff, J. M.; Mitchell, S. L.; Firtion, R.; Peng, C. K.; Cudkowicz, M. E.; Wei, J. Y.; Goldberger, A. L.

    1997-01-01

    Fluctuations in the duration of the gait cycle (the stride interval) display fractal dynamics and long-range correlations in healthy young adults. We hypothesized that these stride-interval correlations would be altered by changes in neurological function associated with aging and certain disease states. To test this hypothesis, we compared the stride-interval time series of 1) healthy elderly subjects and young controls and of 2) subjects with Huntington's disease and healthy controls. Using detrended fluctuation analysis we computed alpha, a measure of the degree to which one stride interval is correlated with previous and subsequent intervals over different time scales. The scaling exponent alpha was significantly lower in elderly subjects compared with young subjects (elderly: 0.68 +/- 0.14; young: 0.87 +/- 0.15; P < 0.003). The scaling exponent alpha was also smaller in the subjects with Huntington's disease compared with disease-free controls (Huntington's disease: 0.60 +/- 0.24; controls: 0.88 +/-0.17; P < 0.005). Moreover, alpha was linearly related to degree of functional impairment in subjects with Huntington's disease (r = 0.78, P < 0.0005). These findings demonstrate that strike-interval fluctuations are more random (i.e., less correlated) in elderly subjects and in subjects with Huntington's disease. Abnormal alterations in the fractal properties of gait dynamics are apparently associated with changes in central nervous system control.

  7. Quantitative EEG parameters correlate with the progression of human prion diseases

    PubMed Central

    Wehner, Tim; Lowe, Jessica; Porter, Marie-Claire; Kenny, Joanna; Thompson, Andrew; Rudge, Peter; Collinge, John; Mead, Simon

    2016-01-01

    Background Prion diseases are universally fatal and often rapidly progressive neurodegenerative diseases. EEG has long been used in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic waveforms do not occur in all types of prion diseases. Here, we re-evaluate the utility of EEG by focusing on the development of biomarkers. We test whether abnormal quantitative EEG parameters can be used to measure disease progression in prion diseases or predict disease onset in healthy individuals at risk of disease. Methods In the National Prion Monitoring Cohort study, we did quantitative encephalography on 301 occasions in 29 healthy controls and 67 patients with prion disease. The patients had either inherited prion disease or sporadic Creutzfeldt-Jakob disease. We computed the main background frequency, the α and θ power and the α/θ power ratio, then averaged these within 5 electrode groups. These measurements were then compared among participant groups and correlated with functional and cognitive scores cross-sectionally and longitudinally. Results We found lower main background frequency, α power and α/θ power ratio and higher θ power in patients compared to control participants. The main background frequency, the power in the α band and the α/θ power ratio also differed in a consistent way among the patient groups. Moreover, the main background frequency and the α/θ power ratio correlated significantly with functional and cognitive scores. Longitudinally, change in these parameters also showed significant correlation with the change in clinical and cognitive scores. Conclusions Our findings support the use of quantitative EEG to follow the progression of prion disease, with potential to help evaluate the treatment effects in future clinical-trials. PMID:27413165

  8. A correlation study between ankle brachial pressure index and the severity of coronary artery disease.

    PubMed

    Benyakorn, Thoetphum; Kuanprasert, Sarun; Rerkasem, Kittipan

    2012-06-01

    Previous studies have shown that there was a correlation between low ankle brachial pressure index (ABPI) and the presence of the coronary artery disease (CAD). However, few studies have investigated the correlation between ABPI and the severity of CAD by using a scoring system. The authors aimed to investigate this correlation by using ABPI and CAD diagnosed by coronary angiography (CAG). A total of 213 consecutive patients awaiting CAG in Maharaj Nakorn Chiang Mai Hospital from July 2009 to November 2009 were enrolled in this study. The ABPI was measured before CAG. The severity of CAD was graded on CAG by using SYNTAX scores. The authors found a significantly negative correlation between ABPI and SYNTAX scores (correlation coefficient = -.172, P = .01). The authors concluded that ABPI appeared to correlate negatively with the severity of CAD in the Thai population. PMID:22561522

  9. Correlation between automated writing movements and striatal dopaminergic innervation in patients with Wilson's disease.

    PubMed

    Hermann, Wieland; Eggers, Birk; Barthel, Henryk; Clark, Daniel; Villmann, Thomas; Hesse, Swen; Grahmann, Friedrich; Kühn, Hans-Jürgen; Sabri, Osama; Wagner, Armin

    2002-08-01

    Handwriting defects are an early sign of motor impairment in patients with Wilson's disease. The basal ganglia being the primary site of copper accumulation in the brain suggests a correlation with lesions in the nigrostiatal dopaminergic system. We have analysed and correlated striatal dopaminergic innervation using [(123)I]beta-CIT-SPECT and automated handwriting movements in 37 patients with Wilson's disease. There was a significant correlation of putaminal dopaminergic innervation with fine motor ability (p < 0,05 for NIV [number of inversion in velocity], NIA [number of inversion in acceleration], frequency). These data suggest that loss of dorsolateral striatal dopaminergic innervation has a pathophysiological function for decreased automated motor control in Wilson's disease. Furthermore analysis of automated handwriting movements could be useful for therapy monitoring and evaluation of striatal dopaminergic innervation. PMID:12195459

  10. Specificity of immunoglobulin E in coccidioidomycosis and correlation with disease involvement.

    PubMed Central

    Cox, R A; Baker, B S; Stevens, D A

    1982-01-01

    Serum immunoglobulin E (IgE) antibodies were quantitated in 26 patients with active pulmonary coccidioidomycosis, 59 patients with active disseminated disease, 12 patients in clinical remission, and 91 healthy subjects. Significant differences were obtained in IgE serum levels of patients with active disease versus healthy subjects (P less than 0.0001). Patients with pulmonary coccidioidomycosis did not differ in their IgE levels when compared with patients with disseminated disease. However, serum IgE levels were significantly increased in patients with disease involving two or more organ systems when compared with patients with pulmonary disease or extrapulmonary disease involving a single organ system (P less than 0.02). Total serum IgE correlated with anti-Coccidioides IgE (P less than 0.001), but with only six exceptions, patients with anti-Coccidioides IgE also exhibited IgE antibodies to 1 or more of 12 common allergens. The correlation between hyperproduction of IgE and disease severity coupled with the depressed cell-mediated immune status of patients with this disease suggests a defect(s) in the T-lymphocyte population which functions to regulate IgE synthesis. PMID:7118252

  11. Neuroanatomical Correlates of Recognizing Face Expressions in Mild Stages of Alzheimer's Disease.

    PubMed

    Sapey-Triomphe, Laurie-Anne; Heckemann, Rolf A; Boublay, Nawele; Dorey, Jean-Michel; Hénaff, Marie-Anne; Rouch, Isabelle; Padovan, Catherine; Hammers, Alexander; Krolak-Salmon, Pierre

    2015-01-01

    Early Alzheimer's disease can involve social disinvestment, possibly as a consequence of impairment of nonverbal communication skills. This study explores whether patients with Alzheimer's disease at the mild cognitive impairment or mild dementia stage have impaired recognition of emotions in facial expressions, and describes neuroanatomical correlates of emotion processing impairment. As part of the ongoing PACO study (personality, Alzheimer's disease and behaviour), 39 patients with Alzheimer's disease at the mild cognitive impairment or mild dementia stage and 39 matched controls completed tests involving discrimination of four basic emotions-happiness, fear, anger, and disgust-on photographs of faces. In patients, automatic volumetry of 83 brain regions was performed on structural magnetic resonance images using MAPER (multi-atlas propagation with enhanced registration). From the literature, we identified for each of the four basic emotions one brain region thought to be primarily associated with the function of recognizing that emotion. We hypothesized that the volume of each of these regions would be correlated with subjects' performance in recognizing the associated emotion. Patients showed deficits of basic emotion recognition, and these impairments were correlated with the volumes of the expected regions of interest. Unexpectedly, most of these correlations were negative: better emotional facial recognition was associated with lower brain volume. In particular, recognition of fear was negatively correlated with the volume of amygdala, disgust with pallidum, and happiness with fusiform gyrus. Recognition impairment in mild stages of Alzheimer's disease for a given emotion was thus associated with less visible atrophy of functionally responsible brain structures within the patient group. Possible explanations for this counterintuitive result include neuroinflammation, regional β-amyloid deposition, or transient overcompensation during early stages of

  12. Neuroanatomical Correlates of Recognizing Face Expressions in Mild Stages of Alzheimer's Disease.

    PubMed

    Sapey-Triomphe, Laurie-Anne; Heckemann, Rolf A; Boublay, Nawele; Dorey, Jean-Michel; Hénaff, Marie-Anne; Rouch, Isabelle; Padovan, Catherine; Hammers, Alexander; Krolak-Salmon, Pierre

    2015-01-01

    Early Alzheimer's disease can involve social disinvestment, possibly as a consequence of impairment of nonverbal communication skills. This study explores whether patients with Alzheimer's disease at the mild cognitive impairment or mild dementia stage have impaired recognition of emotions in facial expressions, and describes neuroanatomical correlates of emotion processing impairment. As part of the ongoing PACO study (personality, Alzheimer's disease and behaviour), 39 patients with Alzheimer's disease at the mild cognitive impairment or mild dementia stage and 39 matched controls completed tests involving discrimination of four basic emotions-happiness, fear, anger, and disgust-on photographs of faces. In patients, automatic volumetry of 83 brain regions was performed on structural magnetic resonance images using MAPER (multi-atlas propagation with enhanced registration). From the literature, we identified for each of the four basic emotions one brain region thought to be primarily associated with the function of recognizing that emotion. We hypothesized that the volume of each of these regions would be correlated with subjects' performance in recognizing the associated emotion. Patients showed deficits of basic emotion recognition, and these impairments were correlated with the volumes of the expected regions of interest. Unexpectedly, most of these correlations were negative: better emotional facial recognition was associated with lower brain volume. In particular, recognition of fear was negatively correlated with the volume of amygdala, disgust with pallidum, and happiness with fusiform gyrus. Recognition impairment in mild stages of Alzheimer's disease for a given emotion was thus associated with less visible atrophy of functionally responsible brain structures within the patient group. Possible explanations for this counterintuitive result include neuroinflammation, regional β-amyloid deposition, or transient overcompensation during early stages of

  13. Serum Paraoxonase Levels are Correlated with Impaired Aortic Functions in Patients with Chronic Kidney Disease

    PubMed Central

    Efe, Tolga H; Ertem, Ahmet G; Altunoglu, Alpaslan; Koseoglu, Cemal; Erayman, Ali; Bilgin, Murat; Kurmuş, Özge; Aslan, Turgay; Bilge, Mehmet

    2016-01-01

    Background The correlation between aortic functions and paraoxonase levels has been previously demonstrated by several earlier studies. In this study, we aimed to investigate the correlation between serum paraoxonase levels and aortic functions among patients with chronic kidney disease. Methods Our study enrolled 46 chronic kidney disease patients and 45 healthy controls. From these patients, serum cholesterol, creatinine, hemoglobin, and paraoxonase-1 levels were analyzed. Results Paraoxonase-1 levels were significantly lower in patients with chronic kidney disease compared to the controls (p < 0.001). Additionally, the extent of aortic stiffness index (%) was significantly higher in chronic kidney disease patients, but aortic strain and aortic distensibility were significantly higher in healthy controls (p < 0.001, p < 0.001, and p < 0.001, respectively). We further found that paraoxonase-1 levels were correlated with aortic stiffness index, aortic strain, and aortic distensibility (p < 0.001, p < 0.001, and p < 0.001, respectively). Conclusions Our study demonstrated that serum paraoxonase-1 levels were significantly correlated with impaired aortic functions. The results of this study highlight the impact of serum paraoxonase-1 activity on atherosclerosis and cardiovascular adverse events. PMID:27122934

  14. Synchrotron radiation analysis of possible correlations between metal status in human cementum and periodontal disease

    SciTech Connect

    Martin, R.R.; Naftel, S.J.; Nelson, A.J.; Edwards, M.; Mithoowani, H.; Stakiw, J.

    2010-03-16

    Periodontitis is a serious disease that affects up to 50% of an adult population. It is a chronic condition involving inflammation of the periodontal ligament and associated tissues leading to eventual tooth loss. Some evidence suggests that trace metals, especially zinc and copper, may be involved in the onset and severity of periodontitis. Thus we have used synchrotron X-ray fluorescence imaging on cross sections of diseased and healthy teeth using a microbeam to explore the distribution of trace metals in cementum and adhering plaque. The comparison between diseased and healthy teeth indicates that there are elevated levels of zinc, copper and nickel in diseased teeth as opposed to healthy teeth. This preliminary correlation between elevated levels of trace metals in the cementum and plaque of diseased teeth suggests that metals may play a role in the progress of periodontitis.

  15. Voxel-based analysis in neuroferritinopathy expands the phenotype and determines radiological correlates of disease severity.

    PubMed

    Keogh, M J; Aribisala, B S; He, J; Tulip, E; Butteriss, D; Morris, C; Gorman, G; Horvath, R; Chinnery, P F; Blamire, Andrew M

    2015-10-01

    Neuroferritinopathy is an autosomal dominant adult-onset movement disorder which occurs due to mutations in the ferritin light chain gene (FTL). Extensive iron deposition and cavitation are observed post-mortem in the basal ganglia, but whether more widespread pathological changes occur, and whether they correlate with disease severity is unknown. 3D-T1w and quantitative T2 whole brain MRI scans were performed in 10 clinically symptomatic patients with the 460InsA FTL mutation and 10 age-matched controls. Voxel-based morphometry (VBM) and voxel-based relaxometry (VBR) were subsequently performed. Clinical assessment using the Unified Dystonia Rating Scale (UDRS) and Unified Huntington's Disease Rating Scale (UHDRS) was undertaken in all patients. VBM detected significant tissue changes within the substantia nigra, midbrain and dentate together with significant cerebellar atrophy in patients (FWE, p < 0.05). Iron deposition in the caudate head and cavitation in the lateral globus pallidus correlated with UDRS score (p < 0.001). There were no differences between groups with VBR. Our data show that progressive iron accumulation in the caudate nucleus, and cavitation of the globus pallidus correlate with disease severity in neuroferritinopathy. We also confirm sub-clinical cerebellar atrophy as a feature of the disease. We suggest that VBM is an effective technique to detect regions of iron deposition and cavitation, with potential wider utility to determine radiological markers of disease severity for all NBIA disorders. PMID:26142024

  16. Genome-wide analysis of genetic correlation in dementia with Lewy bodies, Parkinson's and Alzheimer's diseases

    PubMed Central

    Guerreiro, Rita; Escott-Price, Valentina; Darwent, Lee; Parkkinen, Laura; Ansorge, Olaf; Hernandez, Dena G.; Nalls, Michael A.; Clark, Lorraine; Honig, Lawrence; Marder, Karen; van der Flier, Wiesje; Holstege, Henne; Louwersheimer, Eva; Lemstra, Afina; Scheltens, Philip; Rogaeva, Ekaterina; St George-Hyslop, Peter; Londos, Elisabet; Zetterberg, Henrik; Ortega-Cubero, Sara; Pastor, Pau; Ferman, Tanis J.; Graff-Radford, Neill R.; Ross, Owen A.; Barber, Imelda; Braae, Anne; Brown, Kristelle; Morgan, Kevin; Maetzler, Walter; Berg, Daniela; Troakes, Claire; Al-Sarraj, Safa; Lashley, Tammaryn; Compta, Yaroslau; Revesz, Tamas; Lees, Andrew; Cairns, Nigel J.; Halliday, Glenda M.; Mann, David; Pickering-Brown, Stuart; Powell, John; Lunnon, Katie; Lupton, Michelle K.; Dickson, Dennis; Hardy, John; Singleton, Andrew; Bras, Jose

    2016-01-01

    The similarities between dementia with Lewy bodies (DLB) and both Parkinson's disease (PD) and Alzheimer's disease (AD) are many and range from clinical presentation, to neuropathological characteristics, to more recently identified, genetic determinants of risk. Because of these overlapping features, diagnosing DLB is challenging and has clinical implications since some therapeutic agents that are applicable in other diseases have adverse effects in DLB. Having shown that DLB shares some genetic risk with PD and AD, we have now quantified the amount of sharing through the application of genetic correlation estimates, and show that, from a purely genetic perspective, and excluding the strong association at the APOE locus, DLB is equally correlated to AD and PD. PMID:26643944

  17. Genome-wide analysis of genetic correlation in dementia with Lewy bodies, Parkinson's and Alzheimer's diseases.

    PubMed

    Guerreiro, Rita; Escott-Price, Valentina; Darwent, Lee; Parkkinen, Laura; Ansorge, Olaf; Hernandez, Dena G; Nalls, Michael A; Clark, Lorraine; Honig, Lawrence; Marder, Karen; van der Flier, Wiesje; Holstege, Henne; Louwersheimer, Eva; Lemstra, Afina; Scheltens, Philip; Rogaeva, Ekaterina; St George-Hyslop, Peter; Londos, Elisabet; Zetterberg, Henrik; Ortega-Cubero, Sara; Pastor, Pau; Ferman, Tanis J; Graff-Radford, Neill R; Ross, Owen A; Barber, Imelda; Braae, Anne; Brown, Kristelle; Morgan, Kevin; Maetzler, Walter; Berg, Daniela; Troakes, Claire; Al-Sarraj, Safa; Lashley, Tammaryn; Compta, Yaroslau; Revesz, Tamas; Lees, Andrew; Cairns, Nigel J; Halliday, Glenda M; Mann, David; Pickering-Brown, Stuart; Powell, John; Lunnon, Katie; Lupton, Michelle K; Dickson, Dennis; Hardy, John; Singleton, Andrew; Bras, Jose

    2016-02-01

    The similarities between dementia with Lewy bodies (DLB) and both Parkinson's disease (PD) and Alzheimer's disease (AD) are many and range from clinical presentation, to neuropathological characteristics, to more recently identified, genetic determinants of risk. Because of these overlapping features, diagnosing DLB is challenging and has clinical implications since some therapeutic agents that are applicable in other diseases have adverse effects in DLB. Having shown that DLB shares some genetic risk with PD and AD, we have now quantified the amount of sharing through the application of genetic correlation estimates, and show that, from a purely genetic perspective, and excluding the strong association at the APOE locus, DLB is equally correlated to AD and PD. PMID:26643944

  18. Polygenic risk of Parkinson disease is correlated with disease age at onset

    PubMed Central

    Escott‐Price, Valentina; Nalls, Mike A.; Morris, Huw R.; Lubbe, Steven; Brice, Alexis; Gasser, Thomas; Heutink, Peter; Wood, Nicholas W.; Hardy, John; Singleton, Andrew B.

    2015-01-01

    Objective We have investigated the polygenic architecture of Parkinson disease (PD) and have also explored the potential relationship between an individual's polygenic risk score and their disease age at onset. Methods This study used genotypic data from 4,294 cases and 10,340 controls obtained from the meta‐analysis of PD genome‐wide association studies. Polygenic score analysis was performed as previously described by the International Schizophrenia Consortium, testing whether the polygenic score alleles identified in 1 association study were significantly enriched in the cases relative to the controls of 3 independent studies. Linear regression was used to investigate the relationship between an individual's polygenic score for PD risk alleles and disease age at onset. Results Our polygenic score analysis has identified significant evidence for a polygenic component enriched in the cases of each of 3 independent PD genome‐wide association cohorts (minimum p = 3.76 × 10−6). Further analysis identified compelling evidence that the average polygenic score in patients with an early disease age at onset was significantly higher than in those with a late age at onset (p = 0.00014). Interpretation This provides strong support for a large polygenic contribution to the overall heritable risk of PD and also suggests that early onset forms of the illness are not exclusively caused by highly penetrant Mendelian mutations, but can also be contributed to by an accumulation of common polygenic alleles with relatively low effect sizes. Ann Neurol 2015;77:582–591 PMID:25773351

  19. Structural Imaging Changes and Behavioral Correlates in Patients with Crohn’s Disease in Remission

    PubMed Central

    Nair, Veena A.; Beniwal-Patel, Poonam; Mbah, Ifeanyi; Young, Brittany M.; Prabhakaran, Vivek; Saha, Sumona

    2016-01-01

    Background: Crohn’s disease (CD) is a subtype of inflammatory bowel disease caused by immune-mediated inflammation in the gastrointestinal tract. The extent of morphologic brain alterations and their associated cognitive and affective impairments remain poorly characterized. Aims: We used magnetic resonance imaging to identify structural brain differences between patients with Crohn’s disease in remission compared to age-matched healthy controls and evaluated for structural-behavioral correlates. Methods: Nineteen patients and 20 healthy, age-matched controls were recruited in the study. Group differences in brain morphometric measures and correlations between brain measures and performance on a cognitive task, the verbal fluency (VF) task, were examined. Correlations between brain measures and cognitive measures as well as self-reported measures of depression, personality, and affective scales were examined. Results: Patients showed significant cortical thickening in the left superior frontal region compared to controls. Significant group differences were observed in sub-cortical volume measures in both hemispheres. Investigation of brain-behavior correlations revealed significant group differences in the correlation between cortical surface area and VF performance, although behavioral performance was equivalent between the two groups. The left middle temporal surface area was a significant predictor of VF performance with controls showing a significant positive correlation between these measures, and patients showing the opposite effect. Conclusion: Our results indicate key differences in structural brain measures in patients with CD compared to controls. Additionally, correlation between brain measures and behavioral responses suggest there may be a neural basis to the alterations in patients’ cognitive and affective responses. PMID:27695405

  20. Structural Imaging Changes and Behavioral Correlates in Patients with Crohn’s Disease in Remission

    PubMed Central

    Nair, Veena A.; Beniwal-Patel, Poonam; Mbah, Ifeanyi; Young, Brittany M.; Prabhakaran, Vivek; Saha, Sumona

    2016-01-01

    Background: Crohn’s disease (CD) is a subtype of inflammatory bowel disease caused by immune-mediated inflammation in the gastrointestinal tract. The extent of morphologic brain alterations and their associated cognitive and affective impairments remain poorly characterized. Aims: We used magnetic resonance imaging to identify structural brain differences between patients with Crohn’s disease in remission compared to age-matched healthy controls and evaluated for structural-behavioral correlates. Methods: Nineteen patients and 20 healthy, age-matched controls were recruited in the study. Group differences in brain morphometric measures and correlations between brain measures and performance on a cognitive task, the verbal fluency (VF) task, were examined. Correlations between brain measures and cognitive measures as well as self-reported measures of depression, personality, and affective scales were examined. Results: Patients showed significant cortical thickening in the left superior frontal region compared to controls. Significant group differences were observed in sub-cortical volume measures in both hemispheres. Investigation of brain-behavior correlations revealed significant group differences in the correlation between cortical surface area and VF performance, although behavioral performance was equivalent between the two groups. The left middle temporal surface area was a significant predictor of VF performance with controls showing a significant positive correlation between these measures, and patients showing the opposite effect. Conclusion: Our results indicate key differences in structural brain measures in patients with CD compared to controls. Additionally, correlation between brain measures and behavioral responses suggest there may be a neural basis to the alterations in patients’ cognitive and affective responses.

  1. Localization of coronary artery disease with exercise electrocardiography: correlation with thallium-201 myocardial perfusion scanning

    SciTech Connect

    Dunn, R.F.; Freedman, B.; Bailey, I.K.; Uren, R.F.; Kelly, D.T.

    1981-11-01

    In 61 patients with single vessel coronary artery disease (70 percent or greater obstruction of luminal diameter in only one vessel) and no previous myocardial infarction, the sites of ischemic changes on 12 lead exercise electrocardiography and on thallium-201 myocardial perfusion scanning were related to the obstructed coronary artery. The site of exercise-induced S-T segment depression did not identify which coronary artery was obstructed. In the 37 patients with left anterior descending coronary artery disease S-T depression was most often seen in the inferior leads and leads V4 to V6, and in the 18 patients with right coronary artery disease and in the 6 patients with left circumflex artery disease S-T depression was most often seen in leads V5 and V6. Although S-T segment elevation was uncommon in most leads, it occurred in lead V1 or a VL, or both, in 51 percent of the patients with left anterior descending coronary artery disease. A reversible anterior defect on exercise thallium scanning correlated with left anterior descending coronary artery disease (probability (p) less than 0.0001) and a reversible inferior thallium defect correlated with right coronary or left circumflex artery disease (p less than 0.0001). In patients with single vessel disease, the site of S-T segment depression does not identify the obstructed coronary artery; S-T segment elevation in lead V1 or aVL, or both, identifies left anterior descending coronary artery disease; and the site of reversible perfusion defect on thallium scanning identifies the site of myocardial ischemia and the obstructed coronary artery.

  2. Seasonal Distribution and Climatic Correlates of Dengue Disease in Dhaka, Bangladesh.

    PubMed

    Morales, Ivonne; Salje, Henrik; Saha, Samir; Gurley, Emily S

    2016-06-01

    Dengue has been regularly reported in Dhaka, Bangladesh, since a large outbreak in 2000. However, to date, we have limited information on the seasonal distribution of dengue disease and how case distribution correlates with climate. Here, we analyzed dengue cases detected at a private diagnostic facility in Dhaka during 2010-2014. We calculated Pearson cross-correlation coefficients to examine the relationship between the timing of cases and both rainfall and temperature. There were 2,334 cases diagnosed during the study period with 76% over the age of 15 years. Cases were reported in every month of the study; however, 90% of cases occurred between June and November. Increases in rainfall were correlated with increases in cases 2 months later (correlation of 0.7). The large proportion of adult cases is consistent with substantial population susceptibility and suggests Dhaka remains at risk for outbreaks. Although cases occurred year-round, public health preparedness should be focused during peak months.

  3. Disease activity in Graves' ophthalmopathy: diagnosis with orbital MR imaging and correlation with clinical score.

    PubMed

    Tortora, Fabio; Cirillo, Mario; Ferrara, Marco; Belfiore, Maria Paola; Carella, Carlo; Caranci, Ferdinando; Cirillo, Sossio

    2013-10-01

    In Graves' ophthalmopathy (GO) it is important to distinguish acute inflammation at an early stage, responsive to immunosuppressive treatment, from inactive fibrotic end stage disease, unresponsive to the same treatment. The purpose of this study was to identify the most relevant signal intensities on orbital MR imaging with contrast administration both to classify patients according to their clinical activity score (defined by a cut-off value of 3) and to make a prediction of patient's CAS. Such threshold was considered as widely used in literature. Sixteen consecutive patients with a diagnosis of GO in different phases of thyroid disease based on clinical and orbital MR imaging signs, and six normal volunteers were examined. Orbital MR imaging was performed on a 1.5 Tesla MR Unit. MR scans were assessed by an experienced neuroradiologist, blinded to the clinical examinations. We found a statistical correlation between CAS and both STIR and contrast enhanced T1-weighted sequences. There was also a statistically significant correlation between STIR and contrast-enhanced T1 images disclosing the possibility of avoiding the injection of contrast medium. Our study proved that signal intensity values on STIR sequence increase in the inflammatory oedematous phase of disease. We confirmed the correlation between signal intensities on this sequence and CAS, showing an increase in signal intensity proportional to the CAS value. So we validated MRI use to establish the activity phase of disease more sensitively than CAS alone.

  4. Disease Activity in Graves' Ophthalmopathy: Diagnosis with Orbital MR Imaging and Correlation with Clinical Score

    PubMed Central

    Tortora, Fabio; Cirillo, Mario; Ferrara, Marco; Belfiore, Maria Paola; Carella, Carlo; Caranci, Ferdinando; Cirillo, Sossio

    2013-01-01

    Summary In Graves' ophthalmopathy (GO) it is important to distinguish acute inflammation at an early stage, responsive to immunosuppressive treatment, from inactive fibrotic end stage disease, unresponsive to the same treatment. The purpose of this study was to identify the most relevant signal intensities on orbital MR imaging with contrast administration both to classify patients according to their clinical activity score (defined by a cut-off value of 3) and to make a prediction of patient's CAS. Such threshold was considered as widely used in literature. Sixteen consecutive patients with a diagnosis of GO in different phases of thyroid disease based on clinical and orbital MR imaging signs, and six normal volunteers were examined. Orbital MR imaging was performed on a 1.5 Tesla MR Unit. MR scans were assessed by an experienced neuroradiologist, blinded to the clinical examinations. We found a statistical correlation between CAS and both STIR and contrast enhanced T1-weighted sequences. There was also a statistically significant correlation between STIR and contrast-enhanced T1 images disclosing the possibility of avoiding the injection of contrast medium. Our study proved that signal intensity values on STIR sequence increase in the inflammatory oedematous phase of disease. We confirmed the correlation between signal intensities on this sequence and CAS, showing an increase in signal intensity proportional to the CAS value. So we validated MRI use to establish the activity phase of disease more sensitively than CAS alone. PMID:24199816

  5. Diffusion-weighted MRI findings and clinical correlations in sporadic Creutzfeldt-Jakob disease.

    PubMed

    Gao, Ting; Lyu, Jin-Hao; Zhang, Jia-Tang; Lou, Xin; Zhao, Wei; Xing, Xiao-Wei; Yang, Ming; Yao, Yan; Tan, Qing-Che; Tian, Cheng-Lin; Huang, Xu-Sheng; Ma, Lin; Yu, Sheng-Yuan

    2015-06-01

    The objective of this study is to investigate the hyperintense lesions on diffusion-weighted magnetic resonance imaging (DWI) and its clinical correlation in sporadic Creutzfeldt-Jakob disease (sCJD). Patients who suffered from sCJD and followed up at the Department of Neurology at the General Hospital of the People's Liberation Army during the period of June 1, 2007 to July 1, 2014 were reviewed. The location of the hyperintense lesions on DWI, apparent diffusion coefficient (ADC) values of the hyperintense lesions were correlated with symptoms and clinical course. A total of 58 sCJD patients and ten healthy controls were included. Hyperintense lesions on DWI were observed in all the patients. The patients with basal ganglia (BG) hyperintense lesions on DWI had shorter disease duration and higher incidence of myoclonus (92 versus 44 %) than those without BG hyperintense lesions. The patients with occipital cortex hyperintense lesions on DWI had shorter disease duration between symptom onset and akinetic mutism than those without these lesions. The lower of the BG ADC value the faster presence of akinetic mutism and the shorter disease duration the patients will have. The presence of BG and occipital cortex hyperintense lesions on DWI and BG ADC values is correlated with the clinical course and clinical symptoms. PMID:25860342

  6. Nonalcoholic fatty liver disease: updates in noninvasive diagnosis and correlation with cardiovascular disease.

    PubMed

    Hu, Kuang-Chun; Wang, Horng-Yuan; Liu, Sung-Chen; Liu, Chuan-Chuan; Hung, Chung-Lieh; Bair, Ming-Jong; Liu, Chun-Jen; Wu, Ming-Shiang; Shih, Shou-Chuan

    2014-06-28

    Nonalcoholic fatty liver disease (NAFLD) refers to the accumulation of fat (mainly triglycerides) within hepatocytes. Approximately 20%-30% of adults in the general population in developed countries have NAFLD; this trend is increasing because of the pandemicity of obesity and diabetes, and is becoming a serious public health burden. Twenty percent of individuals with NAFLD develop chronic hepatic inflammation [nonalcoholic steatohepatitis (NASH)], which can be associated with the development of cirrhosis, portal hypertension, and hepatocellular carcinoma in a minority of patients. And thus, the detection and diagnosis of NAFLD is important for general practitioners. Liver biopsy is the gold standard for diagnosing NAFLD and confirming the presence of NASH. However, the invasiveness of this procedure limits its application to screening the general population or patients with contraindications for liver biopsy. The development of noninvasive diagnostic methods for NAFLD is of paramount importance. This review focuses on the updates of noninvasive diagnosis of NAFLD. Besides, we review clinical evidence supporting a strong association between NAFLD and the risk of cardiovascular disease because of the cross link between these two disorders. PMID:24976709

  7. Serotonin content of platelets in inflammatory rheumatic diseases. Correlation with clinical activity.

    PubMed

    Zeller, J; Weissbarth, E; Baruth, B; Mielke, H; Deicher, H

    1983-04-01

    Significantly decreased platelet serotonin contents were measured in rheumatoid arthritis, systemic lupus erythematosus (SLE), progressive systemic sclerosis, and mixed connective tissue disease. An inverse relationship between platelet serotonin levels and clinical disease activity was observed in both rheumatoid arthritis and systemic lupus erythematosus. SLE patients with multiple organ involvement showed the lowest platelet serotonin values. No correlation was observed between platelet serotonin contents and nonsteroidal antiinflammatory drug treatment, presence of circulating platelet reactive IgG, or the amount of circulating immune complexes. The results are interpreted as indicating platelet release occurring in vivo during inflammatory episodes of the rheumatic disorders investigated. PMID:6838676

  8. Automated Gait and Balance Parameters Diagnose and Correlate with Severity in Parkinson Disease

    PubMed Central

    Dewey, Daniel C.; Miocinovic, Svjetlana; Bernstein, Ira; Khemani, Pravin; Dewey, Richard B.; Querry, Ross; Chitnis, Shilpa; Dewey, Richard B.

    2014-01-01

    Objective To assess the suitability of instrumented gait and balance measures for diagnosis and estimation of disease severity in PD. Methods Each subject performed iTUG (instrumented Timed-Up-and-Go) and iSway (instrumented Sway) using the APDM® Mobility Lab. MDS-UPDRS parts II and III, a postural instability and gait disorder (PIGD) score, the mobility subscale of the PDQ-39, and Hoehn & Yahr stage were measured in the PD cohort. Two sets of gait and balance variables were defined by high correlation with diagnosis or disease severity and were evaluated using multiple linear and logistic regressions, ROC analyses, and t-tests. Results 135 PD subjects and 66 age-matched controls were evaluated in this prospective cohort study. We found that both iTUG and iSway variables differentiated PD subjects from controls (area under the ROC curve was 0.82 and 0.75 respectively) and correlated with all PD severity measures (R2 ranging from 0.18 to 0.61). Objective exam-based scores correlated more strongly with iTUG than iSway. The chosen set of iTUG variables was abnormal in very mild disease. Age and gender influenced gait and balance parameters and were therefore controlled in all analyses. Interpretation Our study identified sets of iTUG and iSway variables which correlate with PD severity measures and differentiate PD subjects from controls. These gait and balance measures could potentially serve as markers of PD progression and are under evaluation for this purpose in the ongoing NIH Parkinson Disease Biomarker Program. PMID:25082782

  9. Histomorphometry of feline chronic kidney disease and correlation with markers of renal dysfunction.

    PubMed

    Chakrabarti, S; Syme, H M; Brown, C A; Elliott, J

    2013-01-01

    Chronic kidney disease is common in geriatric cats, but most cases have nonspecific renal lesions, and few studies have correlated these lesions with clinicopathological markers of renal dysfunction. The aim of this study was to identify the lesions best correlated with renal function and likely mediators of disease progression in cats with chronic kidney disease. Cats were recruited through 2 first-opinion practices between 1992 and 2010. When postmortem examinations were authorized, renal tissues were preserved in formalin. Sections were evaluated by a pathologist masked to all clinicopathological data. They were scored semiquantitatively for the severity of glomerulosclerosis, interstitial inflammation, and fibrosis. Glomerular volume was measured using image analysis; the percentage of glomeruli that were obsolescent was recorded. Sections were assessed for hyperplastic arteriolosclerosis and tubular mineralization. Kidneys from 80 cats with plasma biochemical data from the last 2 months of life were included in the study. Multivariable linear regression (P < .05) was used to assess the association of lesions with clinicopathological data obtained close to death. Interstitial fibrosis was the lesion best correlated with the severity of azotemia, hyperphosphatemia, and anemia. Proteinuria was associated with interstitial fibrosis and glomerular hypertrophy, whereas higher time-averaged systolic blood pressure was associated with glomerulosclerosis and hyperplastic arteriolosclerosis. PMID:22773469

  10. Self-reported dysphagia and its correlates within a prevalent population of people with Parkinson's disease.

    PubMed

    Walker, Richard W; Dunn, Janet R; Gray, William K

    2011-03-01

    Many people with Parkinson's disease (PD) experience dysphagia; however, the prevalence of dysphagia in people with PD is unknown. We studied a prevalent population of PD cases. All of those who consented to participate were assessed for anxiety, depression, cognitive function, and quality of life using standard rating scales. Anyone who answered "yes" to either one of the two questions: Do you have difficulty swallowing food/liquid or tablets? and Do you cough after eating/drinking? was considered to have dysphagia. Question 7 of the Unified Parkinson's Disease Rating Scale (UPDRS) was also used to identify dysphagia. Of 106 prevalent PD cases, 75 (38 males) patients consented to examination and assessment. The prevalence of dysphagia was 32.0% (n=24; 11 males). Using the response to UPDRS Question 7 as an indicator of the impact of swallowing problems on the patient, there were significant correlations with cognitive function, anxiety, depression, quality of life, and UPDRS-reported gait disturbance, postural instability and problems with falling. There was no correlation with disease duration, age, or gender. Almost one third of the participants reported dysphagia. There was a strong correlation between dysphagia and gross motor skills; patients reporting such problems should be screened for swallowing problems.

  11. Is there any correlation between high sensitive CRP and disease activity in systemic lupus erythematosus?

    PubMed

    Rezaieyazdi, Z; Sahebari, M; Hatef, M R; Abbasi, B; Rafatpanah, H; Afshari, J Tavakol; Esmaily, H

    2011-12-01

    The role of C-reactive protein (CRP) in systemic lupus erythematosus (SLE) as an inflammatory marker is still controversial. Recently, more sensitive methods, such as high sensitive CRP (hs-CRP) have been used to detect micro-inflammation. The role of hs-CRP in lupus flare has not been documented well. We conducted this study to examine the correlation between hs-CRP serum concentrations and disease activity in lupus. Ninety-two SLE patients and 49 healthy controls contributed to our study. Most confounding factors influencing the hs-CRP values were excluded. Disease activity was estimated using the SLE Disease Activity Index (SLEDAI-2K). hs-CRP values were determined using an enzyme-linked immunosorbent assay (ELISA) kit. Serum values of hs-CRP were significantly higher (p < 0.001, z = 3.29) in patients compared with healthy controls. The cutoff point for hs-CRP between patients and controls was 0.93 mg/L (Youden's Index = 0.39). There was no correlation between hs-CRP serum levels and disease activity. Furthermore, hs-CRP values did not correlate with any of the laboratory parameters, except for C3 (p = 0.003, r(s)  = -0.2) and C4 (p = 0.02, r(s) = -0.1). Although hs-CRP serum levels were significantly higher in lupus patients compared with healthy controls, it seems that this marker is not a good indicator for disease activity.

  12. Respiratory Syncytial Virus (RSV) RNA loads in peripheral blood correlates with disease severity in mice

    PubMed Central

    2010-01-01

    Background Respiratory Syncytial Virus (RSV) infection is usually restricted to the respiratory epithelium. Few studies have documented the presence of RSV in the systemic circulation, however there is no consistent information whether virus detection in the blood correlates with disease severity. Methods Balb/c mice were inoculated with live RSV, heat-inactivated RSV or medium. A subset of RSV-infected mice was treated with anti-RSV antibody 72 h post-inoculation. RSV RNA loads were measured by PCR in peripheral blood from day 1-21 post-inoculation and were correlated with upper and lower respiratory tract viral loads, the systemic cytokine response, lung inflammation and pulmonary function. Immunohistochemical staining was used to define the localization of RSV antigens in the respiratory tract and peripheral blood. Results RSV RNA loads were detected in peripheral blood from day 1 to 14 post-inoculation, peaked on day 5 and significantly correlated with nasal and lung RSV loads, airway obstruction, and blood CCL2 and CXCL1 expression. Treatment with anti-RSV antibody reduced blood RSV RNA loads and improved airway obstruction. Immunostaining identified RSV antigens in alveolar macrophages and peripheral blood monocytes. Conclusions RSV RNA was detected in peripheral blood upon infection with live RSV, followed a time-course parallel to viral loads assessed in the respiratory tract and was significantly correlated with RSV-induced airway disease. PMID:20843364

  13. Stressful life events and psychosocial correlates of pediatric inflammatory bowel disease activity

    PubMed Central

    Giannakopoulos, George; Chouliaras, George; Margoni, Daphne; Korlou, Sophia; Hantzara, Vassiliki; Panayotou, Ioanna; Roma, Eleftheria; Liakopoulou, Magda; Anagnostopoulos, Dimitris C

    2016-01-01

    AIM To investigate the association of psychiatric and psychosocial correlates with inflammatory bowel disease (IBD) activity in children and adolescents. METHODS A total of 85 pediatric IBD patients (in remission or active state of the disease) and their parents completed a series of questionnaires and semi-structured interviews measuring life events, depression, anxiety, family dysfunction, and parent mental health. Differences between the remission and the IBD active group and the association of any significant variable with the disease activity state were examined. RESULTS Parents of children being in active state of the disease reported more life events (P = 0.005) and stressful life events (P = 0.048) during the past year and more mental health symptoms (P < 0.001), while the children themselves reported higher levels of anxiety symptoms (P = 0.017) compared to the remission group. In the logistic regression multivariate analysis, the only predictor which had a significant positive effect on the probability of the patients being in active state was parent mental health symptoms (OR = 4.8; 95%CI: 1.2-25.8). CONCLUSION Life events, child anxiety and parent mental health symptoms may be important correlates of pediatric IBD activity and targets of thorough assessment and treatment. PMID:27679771

  14. Stressful life events and psychosocial correlates of pediatric inflammatory bowel disease activity

    PubMed Central

    Giannakopoulos, George; Chouliaras, George; Margoni, Daphne; Korlou, Sophia; Hantzara, Vassiliki; Panayotou, Ioanna; Roma, Eleftheria; Liakopoulou, Magda; Anagnostopoulos, Dimitris C

    2016-01-01

    AIM To investigate the association of psychiatric and psychosocial correlates with inflammatory bowel disease (IBD) activity in children and adolescents. METHODS A total of 85 pediatric IBD patients (in remission or active state of the disease) and their parents completed a series of questionnaires and semi-structured interviews measuring life events, depression, anxiety, family dysfunction, and parent mental health. Differences between the remission and the IBD active group and the association of any significant variable with the disease activity state were examined. RESULTS Parents of children being in active state of the disease reported more life events (P = 0.005) and stressful life events (P = 0.048) during the past year and more mental health symptoms (P < 0.001), while the children themselves reported higher levels of anxiety symptoms (P = 0.017) compared to the remission group. In the logistic regression multivariate analysis, the only predictor which had a significant positive effect on the probability of the patients being in active state was parent mental health symptoms (OR = 4.8; 95%CI: 1.2-25.8). CONCLUSION Life events, child anxiety and parent mental health symptoms may be important correlates of pediatric IBD activity and targets of thorough assessment and treatment.

  15. Low Serum Lysosomal Acid Lipase Activity Correlates with Advanced Liver Disease

    PubMed Central

    Shteyer, Eyal; Villenchik, Rivka; Mahamid, Mahmud; Nator, Nidaa; Safadi, Rifaat

    2016-01-01

    Fatty liver has become the most common liver disorder and is recognized as a major health burden in the Western world. The causes for disease progression are not fully elucidated but lysosomal impairment is suggested. Here we evaluate a possible role for lysosomal acid lipase (LAL) activity in liver disease. To study LAL levels in patients with microvesicular, idiopathic cirrhosis and nonalcoholic fatty liver disease (NAFLD). Medical records of patients with microvesicular steatosis, cryptogenic cirrhosis and NAFLD, diagnosed on the basis of liver biopsies, were included in the study. Measured serum LAL activity was correlated to clinical, laboratory, imaging and pathological data. No patient exhibited LAL activity compatible with genetic LAL deficiency. However, serum LAL activity inversely predicted liver disease severity. A LAL level of 0.5 was the most sensitive for detecting both histologic and noninvasive markers for disease severity, including lower white blood cell count and calcium, and elevated γ-glutamyltransferase, creatinine, glucose, glycated hemoglobin, uric acid and coagulation function. Serum LAL activity <0.5 indicates severe liver injury in patients with fatty liver and cirrhosis. Further studies should define the direct role of LAL in liver disease severity and consider the possibility of replacement therapy. PMID:26927097

  16. Neural correlates of attention‐executive dysfunction in lewy body dementia and Alzheimer's disease

    PubMed Central

    Kobeleva, Xenia; Cherry, George; Killen, Alison; Gallagher, Peter; Burn, David J.; Thomas, Alan J.; O'Brien, John T.; Taylor, John‐Paul

    2015-01-01

    Abstract Attentional and executive dysfunction contribute to cognitive impairment in both Lewy body dementia and Alzheimer's disease. Using functional MRI, we examined the neural correlates of three components of attention (alerting, orienting, and executive/conflict function) in 23 patients with Alzheimer's disease, 32 patients with Lewy body dementia (19 with dementia with Lewy bodies and 13 with Parkinson's disease with dementia), and 23 healthy controls using a modified Attention Network Test. Although the functional MRI demonstrated a similar fronto‐parieto‐occipital network activation in all groups, Alzheimer's disease and Lewy body dementia patients had greater activation of this network for incongruent and more difficult trials, which were also accompanied by slower reaction times. There was no recruitment of additional brain regions or, conversely, regional deficits in brain activation. The default mode network, however, displayed diverging activity patterns in the dementia groups. The Alzheimer's disease group had limited task related deactivations of the default mode network, whereas patients with Lewy body dementia showed heightened deactivation to all trials, which might be an attempt to allocate neural resources to impaired attentional networks. We posit that, despite a common endpoint of attention‐executive disturbances in both dementias, the pathophysiological basis of these is very different between these diseases. Hum Brain Mapp 37:1254–1270, 2016. © 2015 The Authors Human Brain Mapping Published by Wiley Periodicals, Inc. PMID:26705763

  17. Assessment of subclinical atherosclerosis in ankylosing spondylitis: correlations with disease activity indices.

    PubMed

    Perrotta, F M; Scarno, A; Carboni, A; Bernardo, V; Montepaone, M; Lubrano, E; Spadaro, A

    2013-01-01

    The aim of the study was to evaluate atherosclerosis in ankylosing spondylitis (AS) through the assessment of morphological and functional measures of subclinical atherosclerosis. Twenty patients [M/F=12/8, age (median/range) 43.5/28-69 years; disease duration (median/range) 9.7/1-36) years] with AS classified according to modified New York criteria and twenty age and sex related healthy controls with negative past medical history for cardiovascular events were enrolled in the study. In all patients and controls, the intima-media thickness (IMT) of common carotid artery, carotid bulb and internal carotid artery, and the flow-mediated dilatation (FMD) of non-dominant arm brachial artery were determined, using a sonographic probe Esaote GPX (Genoa, Italy). Furthermore, we assess the main disease activity and disability indices [bath ankylosing spondylitis disease activity index, ankylosing spondylitis disease activity score-eritrosedimentation rate (ASDAS-ESR), ASDAS-C-reactive protein (CRP), bath ankylosing spondylitis metrology index, bath ankylosing spondylitis functional index) and acute phase reactants. Plasmatic values of total cholesterol, low-density lipoprotein, high-density lipoprotein, triglyceride and homocysteine were carried out in all twenty patients. IMT at carotid bulb was significant higher in patients than in controls (0.67 mm vs 0.54 mm; P=0.03). FMD did not statistically differ between patients and controls (12.5% vs 15%; P>0.05). We found a correlation between IMT at carotid bulb and ESR (rho 0.43; P=0.04). No correlation was found between FMD and disease activity and disability indices. This study showed that in AS patients, without risk factors for cardiovascular disease, carotid bulb IMT, morphological index of subclinical atherosclerosis, is higher than in controls.

  18. The correlation between Toxoplasma gondii infection and Parkinson's disease: a case-control study.

    PubMed

    Mahami Oskouei, Mahmoud; Hamidi, Faezeh; Talebi, Mahnaz; Farhoudi, Mehdi; Taheraghdam, Ali Akbar; Kazemi, Tohid; Sadeghi-Bazargani, Homayoun; Fallah, Esmaeil

    2016-09-01

    Toxoplasma gondii is an obligate intracellular parasite that infects all nucleate cells of vertebrates. Human infected by vertical transmission and also using raw or undercooked meat or food and water that contaminated with mature oocysts. Parkinson's disease as neurodegenerative disease affects people above 60 years. Due to high prevalence of toxoplasmosis in Iran and evidence about effects of T. gondii on neurodegenerative diseases, this study has been conducted to investigate possible correlation between Toxoplasma and Parkinson's disease in Iran. Seventy five Parkinson's patients and equal healthy volunteers were enrolled. After obtaining informed consent and sociodemographic features, 5 ml blood sample were collected and then anti-Toxoplasma IgG and IgM levels were examined by ELISA method. Data was analyzed with Chi-squre and Fisher's test by usig stata 11 software. Binary logistic regression was used for multivariate analysis in assessing the correlation between toxoplasmosis and Parkinson. Eighty five percent of Parkinson's group and 90.3 % of control group were positive for anti-Toxoplasma IgG antibody. In this investigation no statically differences were observed between groups and age, gender, residency and using raw or undercooked meat. There is no significant association between IgG positive titer and Parkinson's disease. However, statistically significant association was found between Parkinson and keeping cat (P = 0.03) as well as the using of undercooked egg (P = 0.004). Although there is high level of anti-Toxoplasma IgG antibody in Parkinson's patients which reflects chronic Toxoplasma infection; we couldn't detect any statistical association between T. gondii infection and Parkinson's disease.

  19. The correlation between Toxoplasma gondii infection and Parkinson's disease: a case-control study.

    PubMed

    Mahami Oskouei, Mahmoud; Hamidi, Faezeh; Talebi, Mahnaz; Farhoudi, Mehdi; Taheraghdam, Ali Akbar; Kazemi, Tohid; Sadeghi-Bazargani, Homayoun; Fallah, Esmaeil

    2016-09-01

    Toxoplasma gondii is an obligate intracellular parasite that infects all nucleate cells of vertebrates. Human infected by vertical transmission and also using raw or undercooked meat or food and water that contaminated with mature oocysts. Parkinson's disease as neurodegenerative disease affects people above 60 years. Due to high prevalence of toxoplasmosis in Iran and evidence about effects of T. gondii on neurodegenerative diseases, this study has been conducted to investigate possible correlation between Toxoplasma and Parkinson's disease in Iran. Seventy five Parkinson's patients and equal healthy volunteers were enrolled. After obtaining informed consent and sociodemographic features, 5 ml blood sample were collected and then anti-Toxoplasma IgG and IgM levels were examined by ELISA method. Data was analyzed with Chi-squre and Fisher's test by usig stata 11 software. Binary logistic regression was used for multivariate analysis in assessing the correlation between toxoplasmosis and Parkinson. Eighty five percent of Parkinson's group and 90.3 % of control group were positive for anti-Toxoplasma IgG antibody. In this investigation no statically differences were observed between groups and age, gender, residency and using raw or undercooked meat. There is no significant association between IgG positive titer and Parkinson's disease. However, statistically significant association was found between Parkinson and keeping cat (P = 0.03) as well as the using of undercooked egg (P = 0.004). Although there is high level of anti-Toxoplasma IgG antibody in Parkinson's patients which reflects chronic Toxoplasma infection; we couldn't detect any statistical association between T. gondii infection and Parkinson's disease. PMID:27605800

  20. Cognitive and neuropsychiatric correlates of EEG dynamic complexity in patients with Alzheimer's disease.

    PubMed

    Yang, Albert C; Wang, Shuu-Jiun; Lai, Kuan-Lin; Tsai, Chia-Fen; Yang, Cheng-Hung; Hwang, Jen-Ping; Lo, Men-Tzung; Huang, Norden E; Peng, Chung-Kang; Fuh, Jong-Ling

    2013-12-01

    This study assessed the utility of multiscale entropy (MSE), a complexity analysis of biological signals, to identify changes in dynamics of surface electroencephalogram (EEG) in patients with Alzheimer's disease (AD) that was correlated to cognitive and behavioral dysfunction. A total of 108 AD patients were recruited and their digital EEG recordings were analyzed using MSE methods. We investigate the appropriate parameters and time scale factors for MSE calculation from EEG signals. We then assessed the within-subject consistency of MSE measures in different EEG epochs and correlations of MSE measures to cognitive and neuropsychiatric symptoms of AD patients. Increased severity of AD was associated with decreased MSE complexity as measured by short-time scales, and with increased MSE complexity as measured by long-time scales. MSE complexity in EEGs of the temporal and occipitoparietal electrodes correlated significantly with cognitive function. MSE complexity of EEGs in various brain areas was also correlated to subdomains of neuropsychiatric symptoms. MSE analysis revealed abnormal EEG complexity across short- and long-time scales that were correlated to cognitive and neuropsychiatric assessments. The MSE-based EEG complexity analysis may provide a simple and cost-effective method to quantify the severity of cognitive and neuropsychiatric symptoms in AD patients. PMID:23954738

  1. D-dimer assay in Egyptian patients with Gaucher disease: correlation with bone and lung involvement.

    PubMed

    Sherif, Eman M; Tantawy, Azza A G; Adly, Amira A M; Kader, Hossam A; Ismail, Eman A R

    2011-04-01

    Gaucher disease is the most frequent lysosomal storage disorder. Bone and lung involvement are two major causes of morbidity in this disease. D-dimer is a reliable indicator of active microvascular thrombosis, even in patients without overt hypercoagulation. This study aimed to assess D-dimer levels in Gaucher disease, correlating this marker to clinical characteristics and radiological parameters to investigate its role as a potential predictor for the occurrence and severity of skeletal and pulmonary manifestations. The study population consisted of 56 Egyptian patients with Gaucher disease, 36 had type 1 Gaucher disease (64.3%) and 20 had type 3 Gaucher disease (35.7%). Thirty healthy individuals were enrolled as a control group. D-dimer levels were significantly higher in all patients with Gaucher disease compared with controls (P < 0.001). Patients with type 3 showed significantly higher D-dimer concentrations compared with type 1 (P < 0.001). Pulmonary involvement was present in a significant proportion among type 3 Gaucher patients (P < 0.05), whereas bone changes were present in a higher percentage in type 1 compared with type 3 Gaucher patients. D-dimers were significantly higher in patients with abnormal MRI findings of the long bones and in those with ground glass appearance on high-resolution computerized tomography of the chest compared with patients with normal radiology (P < 0.001). Splenectomized patients displayed significantly higher D-dimer levels compared with nonsplenectomized patients (P < 0.001). Our results suggest that D-dimer is significantly elevated in Gaucher disease, particularly type 3, and may be considered as a potential marker of risk prediction of bone and lung involvement that could be used to monitor treatment response.

  2. Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function

    PubMed Central

    Figueroa-Bonaparte, Sebastián; Segovia, Sonia; Llauger, Jaume; Belmonte, Izaskun; Pedrosa, Irene; Alejaldre, Aída; Mayos, Mercè; Suárez-Cuartín, Guillermo; Gallardo, Eduard; Illa, Isabel; Díaz-Manera, Jordi

    2016-01-01

    Objectives Enzyme replacement therapy has shown to be effective for childhood/adult onset Pompe disease (AOPD). The discovery of biomarkers useful for monitoring disease progression is one of the priority research topics in Pompe disease. Muscle MRI could be one possible test but the correlation between muscle MRI and muscle strength and function has been only partially addressed so far. Methods We studied 34 AOPD patients using functional scales (Manual Research Council scale, hand held myometry, 6 minutes walking test, timed to up and go test, time to climb up and down 4 steps, time to walk 10 meters and Motor Function Measure 20 Scale), respiratory tests (Forced Vital Capacity seated and lying, Maximun Inspiratory Pressure and Maximum Expiratory Pressure), daily live activities scales (Activlim) and quality of life scales (Short Form-36 and Individualized Neuromuscular Quality of Life questionnaire). We performed a whole body muscle MRI using T1w and 3-point Dixon imaging centered on thighs and lower trunk region. Results T1w whole body muscle MRI showed a homogeneous pattern of muscle involvement that could also be found in pre-symptomatic individuals. We found a strong correlation between muscle strength, muscle functional scales and the degree of muscle fatty replacement in muscle MRI analyzed using T1w and 3-point Dixon imaging studies. Moreover, muscle MRI detected mild degree of fatty replacement in paraspinal muscles in pre-symptomatic patients. Conclusion Based on our findings, we consider that muscle MRI correlates with muscle function in patients with AOPD and could be useful for diagnosis and follow-up in pre-symptomatic and symptomatic patients under treatment. Take home message Muscle MRI correlates with muscle function in patients with AOPD and could be useful to follow-up patients in daily clinic. PMID:27711114

  3. Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study

    PubMed Central

    Piano, Carla; Losurdo, Anna; Della Marca, Giacomo; Solito, Marcella; Calandra-Buonaura, Giovanna; Provini, Federica; Bentivoglio, Anna Rita; Cortelli, Pietro

    2015-01-01

    Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P < 0.02). UHMDRS correlated positively with the ESS score (P < 0.005), and negatively with the percentage of REM sleep. Conclusions: Patients with Huntington disease showed a severe sleep disruption and a high prevalence of periodic limb movements, but no evidence of sleep disordered breathing or REM sleep behavior disorder. Citation: Piano C, Losurdo A, Della Marca G, Solito M

  4. Correlation of CT perfusion and CT volumetry in patients with Alzheimer’s disease

    PubMed Central

    Czarnecka, Anna; Zimny, Anna; Sąsiadek, Marek

    2010-01-01

    Summary Background: Both brain atrophy and decrease of perfusion are observed in dementive diseases. The aim of the study was to correlate the results of brain perfusion CT (pCT) and CT volumetry in patients with Alzheimer’s disease (AD). Material/Methods: Forty-eight patients with AD (mean age of 71.3 years) underwent brain pCT and CT volumetry. The pCT was performed at the level of basal ganglia after the injection of contrast medium (50 ml, 4 ml/sec.) with serial scanning (delay 7 sec, 50 scans, 1 scan/sec). Volumetric measurements were carried out on the basis of source images, with the use of a dedicated CT software combined with manual outlining of the regions of interest in extracerebral and intraventricular CSF spaces. Perfusion parameters of the cerebral blood flow (CBF) and cerebral blood volume (CBV) from the grey matter of frontal and temporal as well as basal ganglia were compared statistically with the volumetric measurements of frontal and temporal cortical atrophy as well as subcortical atrophy. Results: A statistically significant positive correlation was found between the values of CBF and CBV in the basal ganglia and the volumes of the lateral and third ventricles. The comparison of CBF and CBV results with the volumetric measurements in the areas of the frontal and temporal lobes showed mostly negative correlations, but none of them was of statistical significance. Conclusions: In patients with AD, the degree of cortical atrophy is not correlated with the decrease of perfusion in the grey matter and subcortical atrophy is not correlated with the decrease of perfusion in the basal ganglia region. It suggests that functional and structural changes in AD are not related to each other. PMID:22802771

  5. Distinctive gene expression signatures in rheumatoid arthritis synovial tissue fibroblast cells: correlates with disease activity.

    PubMed

    Galligan, C L; Baig, E; Bykerk, V; Keystone, E C; Fish, E N

    2007-09-01

    Gene expression profiling of rheumatoid arthritis (RA) and osteoarthritis (OA) joint tissue samples provides a unique insight into the gene signatures involved in disease development and progression. Fibroblast-like synovial (FLS) cells were obtained from RA, OA and control trauma joint tissues (non-RA, non-OA) and RNA was analyzed by Affymetrix microarray. Thirty-four genes specific to RA and OA FLS cells were identified (P<0.05). HOXD10, HOXD11, HOXD13, CCL8 and LIM homeobox 2 were highly and exclusively expressed in RA and CLU, sarcoglycan-gamma, GPR64, POU3F3, peroxisome proliferative activated receptor-gamma and tripartite motif-containing 2 were expressed only in OA. The data also revealed expression heterogeneity for patients with the same disease. To address disease heterogeneity in RA FLS cells, we examined the effects of clinical disease parameters (Health Assessment Questionnaire (HAQ) score, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF)) and drug therapies (methotrexate/prednisone) on RA FLS cell gene expression. Eight specific and unique correlations were identified: human leukocyte antigen (HLA)-DQA2 with HAQ score; Clec12A with RF; MAB21L2, SIAT7E, HAPLN1 and BAIAP2L1 with CRP level; RGMB and OSAP with ESR. Signature RA FLS cell gene expression profiles may provide insights into disease pathogenesis and have utility in diagnosis, prognosis and drug responsiveness. PMID:17568789

  6. N30 Somatosensory Evoked Potential Is Negatively Correlated with Motor Function in Parkinson’s Disease

    PubMed Central

    Kang, Suk Yun; Ma, Hyeo-Il

    2016-01-01

    Objective The aim of this study was to investigate frontal N30 status in Parkinson’s disease (PD) and to examine the correlation between the amplitude of frontal N30 and the severity of motor deficits. Methods The frontal N30 was compared between 17 PD patients and 18 healthy volunteers. Correlations between the amplitude of frontal N30 and the Unified Parkinson’s Disease Rating Scale (UPDRS) motor score of the more severely affected side was examined. Results The mean latency of the N30 was not significantly different between patients and healthy volunteers (p = 0.981), but the mean amplitude was lower in PD patients (p < 0.025). There was a significant negative correlation between the amplitude of N30 and the UPDRS motor score (r = -0.715, p = 0.013). Conclusions The frontal N30 status indicates the motor severity of PD. It can be a useful biomarker reflecting dopaminergic deficits and an objective measurement for monitoring the clinical severity of PD. PMID:26828214

  7. Correlation of coronary plaque characteristics and obstructive stenosis with chronic kidney disease by coronary CT angiography

    PubMed Central

    Wang, Chengming; Duanmu, Yibo; Zhu, Yi; Xu, Lu

    2015-01-01

    Background Chronic kidney disease (CKD) is an independent risk factor for cardiovascular events. We evaluated the correlation of coronary plaque characteristics and obstructive stenosis with CKD by coronary computed tomographic angiography (CCTA). Methods We enrolled 491 subjects who were suspected coronary artery disease (CAD) undergoing CCTA. Estimated glomerular filtration rate (eGFR) was calculated by the modification of diet in renal disease (MDRD) equation. Patients were subdivided into four groups based on their eGFR: normal GFR (n=213, eGFR ≥90 mL/min/1.73 m2), mild renal insufficiency (n=191, eGFR 60-89 mL/min/1.73 m2), moderate renal insufficiency(n=78, eGFR <60 mL/min/1.73 m2, ≥30 mL/min/1.73 m2), and severe renal insufficiency (n=9, eGFR <30 mL/min/1.73 m2, ≥15 mL/min/1.73 m2). Results Spearman correlation regression analysis showed that the prevalence of any plaque, calcified plaque (CP), mixed plaque (MP) were positively correlate with CKD (r=0.173, P<0.001; r=0.127, P=0.005; r=0.171, P<0.001), after adjustment for traditional risk factors the prevalence of any plaque and MP were still positively correlate with CKD (r=0.106, P=002; r=0.178, P<0.001). And the prevalence of any stenosis and severe stenosis were positively correlate with CKD (r=0.13, P<0.001; r=0.149, P<0.001), after adjustment for traditional risk factors were still positively correlate with CKD (r=0.134, P=0.003; r=0.174, P<0.001). Conclusions CKD is closely related with occurrence of CAD. CKD patients from mild renal insufficiency to severe renal insufficiency are the risk factors for CAD. More serious renal function impairment will indicates higher risk of coronary plaque, MP and obstructive stenosis. PMID:26676159

  8. Hope and spirituality among patients with chronic kidney disease undergoing hemodialysis: a correlational study1

    PubMed Central

    Ottaviani, Ana Carolina; Souza, Érica Nestor; Drago, Natália de Camargo; de Mendiondo, Marisa Silvana Zazzetta; Pavarini, Sofia Cristina Iost; Orlandi, Fabiana de Souza

    2014-01-01

    Objective to analyze the relationship between the hope and spirituality of patients with chronic kidney disease undergoing hemodialysis. Method this is a cross-sectional, correlational study. The sample was composed of 127 patients of a Renal Replacement Unit. Data were collected through individual interviews guided by the following instruments: participant characterization, Herth Hope Index (HHI), and Pinto Pais-Ribeiro Spirituality Scale (PP-RSS). Results the average HHI score was 38.06 (±4.32) while the average PP-RSS score was 3.67 (±0.62) for "beliefs" and 3.21 (±0.53) for "hope/optimism". Spearman's coefficient indicated there was a moderate positive correlation between the HHI and PP-RSS dimensions of "beliefs" (r=0.430; p<0.001) and "hope/optimism" (r=0.376; p<0.001). Conclusion Since a relationship between the sense of hope and spirituality of patients with chronic kidney disease was found, these constructs should be taken into account at the time health professionals deliver care to help patients coping with the disease and treatment. PMID:26107832

  9. Appearance of monoclonal plasma cell diseases in whole-body magnetic resonance imaging and correlation with parameters of disease activity.

    PubMed

    Kloth, Jost K; Hillengass, Jens; Listl, Karin; Kilk, Kerstin; Hielscher, Thomas; Landgren, Ola; Delorme, Stefan; Goldschmidt, Hartmut; Kauczor, Hans-Ulrich; Weber, Marc-André

    2014-11-15

    The aim of our study was to assess in which way different infiltration patterns of monoclonal plasma cell diseases in whole-body (wb) magnetic resonance imaging (MRI) are associated with clinical stages, plasma cell content in bone marrow samples and established serum markers of disease activity. Institutional review board approval was obtained. We performed wb-MRI in 547 consecutive, unselected and untreated patients with monoclonal gammopathy of undetermined significance (MGUS, n=138), smoldering myeloma (SMM, n=157) and multiple myeloma (MM, n=252) on two 1.5 T MRI-scanners with body array coils. The studies were evaluated in consensus by two experienced radiologists blinded to the diagnosis. We observed focal lesions in 23.9% (MGUS), 34.4% (SMM) and 81.3% (MM), respectively. A diffuse infiltration pattern was detected in 38.4%, 45.9% and 71%, respectively. The differences between all infiltration patterns were significant (p<0.0001). The presence of focal lesions and the presence of a diffuse bone marrow infiltration was associated with an increased plasma cell percentage in bone marrow samples (median 22% vs. 14%, 26% vs. 10%, both p<0.0001) and monoclonal protein concentration (median 18 g/dl vs. 13 g/dl, p=0.003, 20 g/dl vs. 11 g/dl, p<0.0001). Further categorization of the diffuse infiltration patterns in wb-MRI into "salt-and-pepper," moderate and severe identified significant associations with M-protein (median g/dl for S+P/moderate/severe 23/18/25, p=0.04), plasma cell percentage in the bone marrow (median 25%/24%/40%, p=0.02), and age (median years 67/60/57, p<0.0001). Bone marrow infiltration in wb-MRI is significantly different between the various stages of plasma cell disease and correlates well with established markers of disease activity. PMID:24706394

  10. Raised serum activity of phospholipase A2 immunochemically related to group II enzyme in inflammatory bowel disease: its correlation with disease activity of Crohn's disease and ulcerative colitis.

    PubMed Central

    Minami, T; Tojo, H; Shinomura, Y; Tarui, S; Okamoto, M

    1992-01-01

    Calcium dependent phospholipase A2 activity in the mixed micelles of 1-palmitoyl-2-oleoyl-phosphatidylglycerol and cholate was measured in sera of 39 patients with Crohn's disease, 40 patients with ulcerative colitis, and 40 healthy controls. The phospholipase A2 activity was significantly raised in those sera of the patients with active Crohn's disease and those with moderate and severe ulcerative colitis. The major phospholipase A2 activity derived from the sera was separated into two peaks by reverse phase high performance liquid chromatography. The phospholipase A2 active fractions were immunochemically characterised using specific antibody directed against human group II phospholipase A2 purified from rheumatoid synovial fluid. The results suggest that raised serum phospholipase A2 activity in patients with Crohn's disease and ulcerative colitis was mainly attributed to the two forms of phospholipase A2 immunochemically related to group II enzyme. In patients with Crohn's disease, serum phospholipase A2 activity decreased in parallel with clinical improvement, and correlated with serum C-reactive protein and erythrocyte sedimentation rate. The results suggest that serum phospholipase A2 activity may serve as an additional indicator of disease activity. Serum phospholipase A2 activity in patients with ulcerative colitis tends to increase in relation with endoscopic severity, and may be a more sensitive laboratory index than serum C-reactive protein and erythrocyte sedimentation rate to evaluate disease activity. Images Figure 3 PMID:1644331

  11. Are Long-Range Structural Correlations Behind the Aggregration Phenomena of Polyglutamine Diseases?

    PubMed Central

    Moradi, Mahmoud; Babin, Volodymyr; Roland, Christopher; Sagui, Celeste

    2012-01-01

    We have characterized the conformational ensembles of polyglutamine peptides of various lengths (ranging from to ), both with and without the presence of a C-terminal polyproline hexapeptide. For this, we used state-of-the-art molecular dynamics simulations combined with a novel statistical analysis to characterize the various properties of the backbone dihedral angles and secondary structural motifs of the glutamine residues. For (i.e., just above the pathological length for Huntington's disease), the equilibrium conformations of the monomer consist primarily of disordered, compact structures with non-negligible -helical and turn content. We also observed a relatively small population of extended structures suitable for forming aggregates including - and -strands, and - and -hairpins. Most importantly, for we find that there exists a long-range correlation (ranging for at least residues) among the backbone dihedral angles of the Q residues. For polyglutamine peptides below the pathological length, the population of the extended strands and hairpins is considerably smaller, and the correlations are short-range (at most residues apart). Adding a C-terminal hexaproline to suppresses both the population of these rare motifs and the long-range correlation of the dihedral angles. We argue that the long-range correlation of the polyglutamine homopeptide, along with the presence of these rare motifs, could be responsible for its aggregation phenomena. PMID:22577357

  12. The Geomagnetic Field and Correlations with Multiple Sclerosis: A Possible Etiology of Disease

    NASA Astrophysics Data System (ADS)

    Wade, Brett

    Multiple sclerosis (MS) is a complex autoimmune disease that results in a demyelinating process of the central nervous system. It is the most common, progressive, neurological disease affecting young adults, and there is no cure. A curious feature of MS is its distinct global prevalence with high rates of occurrence between 40 and 60 degrees latitude. While genetics may partially explain this phenomenon, studies have shown that the influence of genetics is modest. Many non-genetic variables, such as viruses, vitamin D, smoking, diet, hormones, etc., have been shown to be related to the expression of MS but none of these variables have been determined to be necessarily strong enough to exclude other factors. The geomagnetic field, which is a non-uniform, three dimensional entity which protects all living things from ionizing radiation, is suggested in this research to be related to global MS prevalence. This study hypothesized that either the total field, the vertical field, or the horizontal field strength of the geomagnetic field will be correlated with MS. Using secondary sources of prevalence studies (N=131) and geomagnetic data, the results supported all three hypotheses with the strongest correlation being an inverse relationship between the horizontal field and MS (r = -.607). The explanation for the inverse relationship being most strongly correlated with MS prevalence is explained by the fact that the horizontal aspect of the geomagnetic field has a protective effect from incoming cosmic radiation. Chronic exposure to high levels of background radiation can have deleterious health effects. This research suggests that living in areas of a weak horizontal field increases a person's exposure to ionizing radiation and therefore increases the risk for developing MS. While it was not the intention of this research, it became clear that an explanation which explained the results of this research and also attempted to unify the mechanisms of all non

  13. Correlates of Excessive Daytime Sleepiness in De Novo Parkinson's Disease: A Case Control Study

    PubMed Central

    Simuni, Tanya; Caspell-Garcia, Chelsea; Coffey, Christopher; Chahine, Lama M.; Lasch, Shirley; Oertel, Wolfgang H.; Mayer, Geert; Högl, Birgit; Postuma, Ron; Videnovic, Aleksandar; Amara, Amy Willis; Marek, Ken

    2016-01-01

    Objective This study was undertaken to determine the frequency and correlates of excessive daytime sleepiness in de novo, untreated Parkinson's disease (PD) patients compared with the matched healthy controls. Methods Data were obtained from the Parkinson's Progression Markers Initiative, an international study of de novo, untreated PD patients and healthy controls. At baseline, participants were assessed with a wide range of motor and nonmotor scales, including the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS). Excessive daytime sleepiness was assessed based on the Epworth Sleepiness scale (ESS), with a cutoff of 10. Results Four hundred twenty-three PD subjects and 196 healthy controls were recruited into the study. Mean ESS (min, max) score was 5.8 (0, 20) for the PD subjects and 5.6 (0, 19) for healthy controls (P = 0.54). Sixty-six (15.6%) PD subjects and 24 (12%) healthy controls had ESS of at least 10 (P = 0.28). No difference was seen in demographic characteristics, age of onset, disease duration, PD subtype, cognitive status, or utilization of sedatives between the PD sleepiness-positive versus the negative group. The sleepiness-positive group had higher MDS-UPDRS Part I and II but not III scores, and higher depression and autonomic dysfunction scores. Sleepiness was associated with a marginal reduction of A-beta (P = 0.05) but not alpha-synuclein spinal fluid levels in PD. Conclusions This largest case control study demonstrates no difference in prevalence of excessive sleepiness in subjects with de novo untreated PD compared with healthy controls. The only clinical correlates of sleepiness were mood and autonomic dysfunction. Ongoing longitudinal analyses will be essential to further examine clinical and biological correlates of sleepiness in PD and specifically the role of dopaminergic therapy. PMID:26095202

  14. Mechanistic effects of IVIg in neuroinflammatory diseases: conclusions based on clinicopathologic correlations.

    PubMed

    Dalakas, Marinos C

    2014-07-01

    The mechanisms of action of IVIg on immunoregulatory and neuroinflammatory network have been predominantly based on in vitro experiments and animal studies, rather than direct effects on human tissues. Based on clinicopathologic correlations and tissues obtained before and after IVIg therapy, the better documented and clinically-relevant in-vivo actions of IVIg include effects on: a) Antibodies. An extracted antigen-specific anti-immunoglobulin (idiotypic) fraction appears partially responsible for its effect in myasthenia gravis and GBS; b) Complement. Sera from Dermatomyositis (DM) patients responding to IVIg, inhibit complement consumption and intercept MAC formation leading to disappearance of MAC deposits in the repeated muscle biopsies and normalization of muscle tissue; c) Genes. In repeated muscle biopsies from DM patients who improved after IVIg, but not from Inclusion-Body-Myositis (IBM) who did not improve, there is a 2-fold alteration of 2206 tissue genes associated with inflammation, fibrosis, tissue remodeling and regeneration; and d) degenerative-proinflammatory molecules and β-amyloid, implicated in neurodegenerative CNS diseases and IBM. In repeated muscle biopsies of IBM patients who did not respond to IVIg, the mRNA or protein expression for chemokines, IFN-γ, TGF-ß, IL-10, Ubiquitin and aB-crystallin is reduced, but not for the key molecules ICOS, ICOSL, IL-6, IL1-β, perforin, APP, nitric oxide synthase and nitrotyrosine, in spite of good IVIg penetration in muscles. Collectively, the selective effectiveness of IVIg in human diseases seems to correlate in vivo with inhibition of causative inflammatory mediators. Study of accessible tissues before and after therapy and clinicopathologic correlations, may help explain the differential effect of IVIg in autoimmune or neuroinflammatory diseases.

  15. Correlation of Alzheimer's disease death rates with historical per capita personal income in the USA.

    PubMed

    Stępkowski, Dariusz; Woźniak, Grażyna; Studnicki, Marcin

    2015-01-01

    Alzheimer's disease (AD) is a progressive degenerating disease of complex etiology. A variety of risk factors contribute to the chance of developing AD. Lifestyle factors, such as physical, mental and social activity, education, and diet all affect the susceptibility to developing AD. These factors are in turn related to the level of personal income. Lower income usually coincides with lower level of education, lesser mental, leisure-social and physical activity, and poorer diet. In the present paper, we have analyzed the correlation of historical (1929-2011) per capita personal income (PCPI) for all states of the USA with corresponding age-adjusted AD death rates (AADR) for years 2000, 2005 and 2008. We found negative correlations in all cases, the highest one (R ≈ -0.65) for the PCPIs in the year 1970 correlated against the AADRs in 2005. From 1929 to 2005 the R value varies in an oscillatory manner, with the strongest correlations in 1929, 1970, 1990 and the weakest in 1950, 1980, 1998. Further analysis indicated that this oscillatory behavior of R is not artificially related to the economic factors but rather to delayed biological consequences associated with personal income. We conclude that the influence of the income level on the AD mortality in 2005 was the highest in the early years of life of the AD victims. Overall, the income had a significant, lifelong, albeit constantly decreasing, influence on the risk of developing AD. We postulate that the susceptibility of a population to late-onset AD (LOAD) is determined to a large extent by the history of income-related modifiable lifestyle risk factors. Among these risk factors, inappropriate diet has a significant contribution.

  16. Excretion of pyridinium crosslinks correlates with disease activity and appendicular bone loss in early rheumatoid arthritis.

    PubMed Central

    Gough, A K; Peel, N F; Eastell, R; Holder, R L; Lilley, J; Emery, P

    1994-01-01

    OBJECTIVE--To establish if urinary excretion rates of the collagen crosslinks pyridinoline and deoxypyridinoline, which are known to be elevated in established rheumatoid arthritis (RA), are useful markers of bone loss in this disease. METHODS--Eight hour urine collections on all patients and 52 controls were performed, and the rates of pyridinoline and deoxypyridinoline excretion were measured. Bone mineral density (BMD), by dual energy x-ray absorption, and full laboratory and clinical assessments were performed. RESULTS--The rates of excretion of pyridinoline and deoxypyridinoline were significantly increased in patients compared with controls (p < 0.001). Pyridinoline excretion was associated with increased disease activity (ESR/CRP) but not disability (HAQ score/Functional Grade), and correlated with BMD loss at the femoral neck (p < 0.01). CONCLUSION--The excretion of collagen crosslinks may be useful as markers of bone and cartilage turnover in patients with RA. PMID:8311548

  17. Correlation of mast cells in different stages of human periodontal diseases: Pilot study

    PubMed Central

    Agrawal, Raina; Gupta, Jagriti; Gupta, Krishna Kumar; Kumar, Vinod

    2016-01-01

    Aims and Objectives: The aim of this study was to evaluate and correlate the relationship between mast cells counts and different stages of human periodontal diseases. Materials and Methods: The study sample comprised 50 patients, which were divided into three groups, consisting of 10 cases of clinically healthy gingival tissues (control group) 20 cases of dental plaque-induced gingivitis with no attachment loss and 20 cases of localized chronic periodontitis (LCP) characterized by the loss of periodontal support. The samples for control group were obtained during tooth extractions for orthodontic reasons. The specimens were immediately fixed in 10% neutral buffered formalin. Conclusion: In this study, LCP cases had higher mast cell counts compared to gingivitis sites or healthy tissues. Increased mast cell counts in the progressing sites of periodontal diseases may indicate the importance of these cells in the progression of chronic periodontitis. PMID:27194868

  18. Prevalence and correlates of vitamin K deficiency in children with inflammatory bowel disease.

    PubMed

    Nowak, Jan K; Grzybowska-Chlebowczyk, Urszula; Landowski, Piotr; Szaflarska-Poplawska, Anna; Klincewicz, Beata; Adamczak, Daria; Banasiewicz, Tomasz; Plawski, Andrzej; Walkowiak, Jaroslaw

    2014-01-01

    Although vitamin K deficiency has been implicated in adult inflammatory bowel disease (IBD), its prevalence in pediatric IBD remains unknown. We carried out a cross-sectional study in 63 children with Crohn's disease (CD) and 48 with ulcerative colitis (UC) to assess the prevalence of vitamin K deficiency and to search for potential correlation between vitamin K status and pediatric IBD activity. Vitamin K status was assessed using protein induced by vitamin K absence-II (PIVKA-II; ELISA). Prevalence of vitamin K deficiency was 54.0% in CD and 43.7% in UC. Vitamin K deficiency was more common in patients with higher CD activity, in CD patients with higher mass Z-scores, and less common among children with CD treated with infliximab. Relation of vitamin K deficiency to pediatric IBD clinical course and treatment demand further research. PMID:24759680

  19. Prevalence and correlates of vitamin K deficiency in children with inflammatory bowel disease.

    PubMed

    Nowak, Jan K; Grzybowska-Chlebowczyk, Urszula; Landowski, Piotr; Szaflarska-Poplawska, Anna; Klincewicz, Beata; Adamczak, Daria; Banasiewicz, Tomasz; Plawski, Andrzej; Walkowiak, Jaroslaw

    2014-04-24

    Although vitamin K deficiency has been implicated in adult inflammatory bowel disease (IBD), its prevalence in pediatric IBD remains unknown. We carried out a cross-sectional study in 63 children with Crohn's disease (CD) and 48 with ulcerative colitis (UC) to assess the prevalence of vitamin K deficiency and to search for potential correlation between vitamin K status and pediatric IBD activity. Vitamin K status was assessed using protein induced by vitamin K absence-II (PIVKA-II; ELISA). Prevalence of vitamin K deficiency was 54.0% in CD and 43.7% in UC. Vitamin K deficiency was more common in patients with higher CD activity, in CD patients with higher mass Z-scores, and less common among children with CD treated with infliximab. Relation of vitamin K deficiency to pediatric IBD clinical course and treatment demand further research.

  20. Radiotherapy as a cause of complete atrioventricular block in Hodgkin's disease: an electrophysiological-pathological correlation

    SciTech Connect

    Cohen, S.I.; Bharati, S.; Glass, J.; Lev, M.

    1981-04-01

    A 20-year-old man contracted Hodgkin's disease and was treated with mantle radiotherapy. Heart block developed 11 years later. Electrocardiograms revealed predominant atrioventricular (AV) block and occasional AV conduction. Intracardiac electrograms demonstrated that the site of AV block was above the level of the His bundle. A permanent transvenous pacemaker was implanted. Seven months later the patient died of complications from cryptococcal meningitis. Pathological study of the heart revealed marked arteriosclerosis with fibrosis of the epicardium, myocardium, and endocardium. Examination of the conduction system revealed extensive arteriolosclerosis of the sinoatrial node and its approaches. In addition, there was marked fibrosis of the approaches to the AV node, the AV bundle, and both bundle branches. There was no evidence of Hodgkin's disease. This case documents the rare occurrence of AV block due to tissue destruction by radiotherapy. There was a good correlation between block proximal to the His bundle recording site and fibrosis of the approaches to the AV node.

  1. Radiotherapy as a cause of complete atrioventricular block in Hodgkin's disease. An electrophysiological-pathological correlation

    SciTech Connect

    Cohen, S.I.; Bharati, S.; Glass, J.; Lev, M.

    1981-04-01

    A 20-year-old man contracted Hodgkin's disease and was treated with mantle radiotherapy. Heart block developed 11 years later. Electrocardiograms revealed predominant atrioventricular (AV) block and occasional AV conduction. Intracardiac electrograms demonstrated that the site of AV block was above the level of the His bundle. A permanent transvenous pacemaker was implanted. Seven months later the patient died of complications from cryptococcal meningitis. Pathological study of the heart revealed marked arteriosclerosis with fibrosis of the epicardium, myocardium, and endocardium. Examination of the conduction system revealed extensive arteriolosclerosis of the sinoatrial node and its approaches. In addition, there was marked fibrosis of the approaches to the AV node, the AV bundle, and both bundle branches. There was no evidence of Hodgkin's disease. This case documents the rare occurrence of AV block due to tissue destruction by radiotherapy. There was a good correlation between block proximal to the His bundle recording site and fibrosis of the approaches to the AV node.

  2. Rainbow trout (Oncorhynchus mykiss) resistance to columnaris disease is heritable and favorably correlated with bacterial cold water disease resistance.

    PubMed

    Evenhuis, J P; Leeds, T D; Marancik, D P; LaPatra, S E; Wiens, G D

    2015-04-01

    Columnaris disease (CD), caused by Flavobacterium columnare, is an emerging disease affecting rainbow trout aquaculture. Objectives of this study were to 1) estimate heritability of CD resistance in a rainbow trout line (ARS-Fp-R) previously selected 4 generations for improved bacterial cold water disease (BCWD) resistance; 2) estimate genetic correlations among CD resistance, BCWD resistance, and growth to market BW; and 3) compare CD resistance among the ARS-Fp-R, ARS-Fp-S (selected 1 generation for increased BCWD susceptibility), and ARS-Fp-C (selection control) lines. Heritability of CD resistance was estimated using data from a waterborne challenge of 44 full-sib ARS-Fp-R families produced using a paternal half-sib mating design, and genetic correlations were estimated using these data and 5 generations of BCWD resistance, 9-mo BW (approximately 0.5 kg), and 12-mo BW (approximately 1.0 kg) data from 405 ARS-Fp-R full-sib families. The CD and BCWD challenges were initiated at approximately 52 and 84 d posthatch, or approximately 650 and 1,050 degree days (°C × d), respectively. Survival of ARS-Fp-R families ranged from 0 to 48% following CD challenge and heritability estimates were similar between CD (0.17 ± 0.09) and BCWD (0.18 ± 0.03) resistance, and the genetic correlation between these 2 traits was favorable (0.35 ± 0.25). Genetic correlations were small and antagonistic (-0.15 ± 0.08 to -0.19 ± 0.24) between the 2 resistance traits and 9- and 12-mo BW. Two challenges were conducted in consecutive years to compare CD resistance among ARS-Fp-R, ARS-Fp-C, and ARS-Fp-S families. In the first challenge, ARS-Fp-R families (83% survival) had greater CD resistance than ARS-Fp-C (73.5%; P = 0.02) and ARS-Fp-S (68%; P < 0.001) families, which did not differ (P = 0.16). In the second challenge, using an approximately 2.5-fold greater challenge dose, ARS-Fp-R families exhibited greater CD resistance (56% survival) than ARS-Fp-S (38% survival; P = 0.02) families

  3. Lipoprotein (a) in childhood: correlations with family history of cardiovascular disease.

    PubMed

    De Simone, M; Verrotti, A; Cappa, M; Iughetti, L; Di Cesare, E; Palumbo, M; Bernabei, R; Rosato, T

    2003-05-01

    The association between lipoprotein (a) [Lp(a)] and cardiovascular diseases is well known. Lp(a) is an independent risk factor for the development of atherosclerosis. Little information concerning Lp(a) during childhood is available. The aim of the present investigation was to determine the Lp(a) concentration in a cohort of children aged between 4 and 15 yr and to correlate Lp(a) with: a) overweight status; b) body fat distribution; c) family history of vascular diseases in their parents and grandparents. Six hundred and eighty-nine children (350 males, 339 females), were enrolled in the study. BMI as index of being overweight was calculated; the waist-to-hip ratio (WHR) and the waist-to-thigh ratio (WTR) were calculated to obtain two anthropometric indexes for the pattern of body fat distribution. The areas of visceral (VAT) and subcutaneous adipose tissue (SAT) were evaluated by MRI at the L4-L5 level in only 30 overweight subjects. The serum of Lp(a), total cholesterol (TC), HDL-cholesterol, LDL-cholesterol and triglycerides were evaluated in the whole population. Moreover, the same biochemical study was performed in 70 children's parents randomly chosen. A structured questionnaire was administered to the children's parents to investigate the presence of cardiovascular disease (CVD) in family stories. Our data show no Lp(a) serum differences between children according to sex, age and body composition. The strong correlation between the children's and the parents' Lp(a) concentrations and the occurrence of CVD in their grandparents suggests that Lp(a) represents an important independent early risk factor for the development of CVD in adulthood. Measurements of Lp(a) in childhood may help to evaluate this risk in subjects with family history of cardiovascular diseases.

  4. Metallothionein-1 and nitric oxide expression are inversely correlated in a murine model of Chagas disease

    PubMed Central

    Gonzalez-Mejia, Martha Elba; Torres-Rasgado, Enrique; Porchia, Leonardo M; Salgado, Hilda Rosas; Totolhua, José-Luis; Ortega, Arturo; Hernández-Kelly, Luisa Clara Regina; Ruiz-Vivanco, Guadalupe; Báez-Duarte, Blanca G; Pérez-Fuentes, Ricardo

    2014-01-01

    Chagas disease, caused by Trypanosoma cruzi, represents an endemic among Latin America countries. The participation of free radicals, especially nitric oxide (NO), has been demonstrated in the pathophysiology of seropositive individuals with T. cruzi. In Chagas disease, increased NO contributes to the development of cardiomyopathy and megacolon. Metallothioneins (MTs) are efficient free radicals scavengers of NO in vitro and in vivo. Here, we developed a murine model of the chronic phase of Chagas disease using endemic T. cruzi RyCH1 in BALB/c mice, which were divided into four groups: infected non-treated (Inf), infected N-monomethyl-L-arginine treated (Inf L-NAME), non-infected L-NAME treated and non-infected vehicle-treated. We determined blood parasitaemia and NO levels, the extent of parasite nests in tissues and liver MT-I expression levels. It was observed that NO levels were increasing in Inf mice in a time-dependent manner. Inf L-NAME mice had fewer T. cruzi nests in cardiac and skeletal muscle with decreased blood NO levels at day 135 post infection. This affect was negatively correlated with an increase of MT-I expression (r = -0.8462, p < 0.0001). In conclusion, we determined that in Chagas disease, an unknown inhibitory mechanism reduces MT-I expression, allowing augmented NO levels. PMID:24676665

  5. Prevalence and correlates of valvular heart diseases in the elderly population in Hubei, China

    PubMed Central

    Shu, Chang; Chen, Si; Qin, Tingting; Fu, Zhen; Sun, Tucheng; Xie, Mingxing; Zhang, Li; Dong, Nianguo; Yin, Ping

    2016-01-01

    We sought to determine the prevalence and correlates of valvular heart diseases (VHD) in the elderly population. The participants’ personal information, medical history, behavioral habits and clinical status were assessed by questionnaire, while the left ventricular dimensions, function and the presence and severity of VHD were evaluated by transthoracic echocardiography. This study analyzed the data of 3948 participants who were older than 60 years. Significant VHD was present in 1.93% of participants; the standardized prevalence of VHD among the elderly population in Hubei was 2.05% (95% CI: 1.61–2.49). The most frequent VHD was aortic regurgitation, followed by tricuspid regurgitation, mitral regurgitation and multiple valve diseases. Univariate analysis results indicated that compared with participants without VHD, those with VHD were older (p < 0.001), with a higher body mass index (BMI) (p < 0.001), were more likely to smoke (p = 0.04), and had higher rates of coronary artery disease (CAD) (p < 0.001) and arrhythmia (p < 0.001). The results of multinomial regression analysis of complex sampling indicated that combined mitral and aortic valve diseases were related to older age, male sex and smoking; CAD was associated with single left-sided VHD. PMID:27250873

  6. Metallothionein-1 and nitric oxide expression are inversely correlated in a murine model of Chagas disease.

    PubMed

    Gonzalez-Mejia, Martha Elba; Torres-Rasgado, Enrique; Porchia, Leonardo M; Salgado, Hilda Rosas; Totolhua, José-Luis; Ortega, Arturo; Hernández-Kelly, Luisa Clara Regina; Ruiz-Vivanco, Guadalupe; Báez-Duarte, Blanca G; Pérez-Fuentes, Ricardo

    2014-04-01

    Chagas disease, caused by Trypanosoma cruzi, represents an endemic among Latin America countries. The participation of free radicals, especially nitric oxide (NO), has been demonstrated in the pathophysiology of seropositive individuals with T. cruzi. In Chagas disease, increased NO contributes to the development of cardiomyopathy and megacolon. Metallothioneins (MTs) are efficient free radicals scavengers of NO in vitro and in vivo. Here, we developed a murine model of the chronic phase of Chagas disease using endemic T. cruzi RyCH1 in BALB/c mice, which were divided into four groups: infected non-treated (Inf), infected N-monomethyl-L-arginine treated (Inf L-NAME), non-infected L-NAME treated and non-infected vehicle-treated. We determined blood parasitaemia and NO levels, the extent of parasite nests in tissues and liver MT-I expression levels. It was observed that NO levels were increasing in Inf mice in a time-dependent manner. Inf L-NAME mice had fewer T. cruzi nests in cardiac and skeletal muscle with decreased blood NO levels at day 135 post infection. This affect was negatively correlated with an increase of MT-I expression (r = -0.8462, p < 0.0001). In conclusion, we determined that in Chagas disease, an unknown inhibitory mechanism reduces MT-I expression, allowing augmented NO levels.

  7. Dengue viremia titer, antibody response pattern, and virus serotype correlate with disease severity.

    PubMed

    Vaughn, D W; Green, S; Kalayanarooj, S; Innis, B L; Nimmannitya, S; Suntayakorn, S; Endy, T P; Raengsakulrach, B; Rothman, A L; Ennis, F A; Nisalak, A

    2000-01-01

    Viremia titers in serial plasma samples from 168 children with acute dengue virus infection who were enrolled in a prospective study at 2 hospitals in Thailand were examined to determine the role of virus load in the pathogenesis of dengue hemorrhagic fever (DHF). The infecting virus serotype was identified for 165 patients (DEN-1, 46 patients; DEN-2, 47 patients; DEN-3, 47 patients, DEN-4, 25 patients). Patients with DEN-2 infections experienced more severe disease than those infected with other serotypes. Eighty-one percent of patients experienced a secondary dengue virus infection that was associated with more severe disease. Viremia titers were determined for 41 DEN-1 and 46 DEN-2 patients. Higher peak titers were associated with increased disease severity for the 31 patients with a peak titer identified (mean titer of 107.6 for those with dengue fever vs. 108.5 for patients with DHF, P=.01). Increased dengue disease severity correlated with high viremia titer, secondary dengue virus infection, and DEN-2 virus type.

  8. Non linear approach to study the dynamics of neurodegenerative diseases by Multifractal Detrended Cross-correlation Analysis-A quantitative assessment on gait disease

    NASA Astrophysics Data System (ADS)

    Dutta, Srimonti; Ghosh, Dipak; Samanta, Shukla

    2016-04-01

    This paper studies the human gait pattern of normal people and patients suffering from Parkinson's disease using the MFDXA (Multifractal Detrended Cross-correlation Analysis) methodology. The auto correlation and cross correlation of the time series of the total force under the left foot and right foot were studied. The study reveals that the degree of multifractality (W) and degree of correlation (γ) are generally more for normal patients than the diseased set. It is also observed that the values of W and γ are nearly same for left foot and right. It is also observed that the study of autocorrelation alone is not sufficient, cross correlations should also be studied to get a better concept of neurodegenerative diseases.

  9. Grey and White Matter Clinico-Anatomical Correlates of Disinhibition in Neurodegenerative Disease

    PubMed Central

    Santillo, Alexander Frizell; Lundblad, Karl; Nilsson, Markus; Landqvist Waldö, Maria; van Westen, Danielle; Lätt, Jimmy; Blennow Nordström, Erik; Vestberg, Susanna; Lindberg, Olof; Nilsson, Christer

    2016-01-01

    Disinhibition is an important symptom in neurodegenerative diseases. However, the clinico-anatomical underpinnings remain controversial. We explored the anatomical correlates of disinhibition in neurodegenerative disease using the perspective of grey and white matter imaging. Disinhibition was assessed with a neuropsychological test and a caregiver information-based clinical rating scale in 21 patients with prefrontal syndromes due to behavioural variant frontotemporal dementia (n = 12) or progressive supranuclear palsy (n = 9), and healthy controls (n = 25). Cortical thickness was assessed using the Freesurfer software on 3T MRI data. The integrity of selected white matter tracts was determined by the fractional anisotropy (FA) from Diffusion Tensor Imaging. Disinhibition correlated with the cortical thickness of the right parahippocampal gyrus, right orbitofrontal cortex and right insula and the FA of the right uncinate fasciculus and right anterior cingulum. Notably, no relationship was seen with the thickness of ventromedial prefrontal cortex. Our results support an associative model of inhibitory control, distributed in a medial temporal lobe-insular-orbitofrontal network, connected by the intercommunicating white matter tracts. This reconciles some of the divergences among previous studies, but also questions the current conceptualisation of the “prefrontal” syndrome and the central role attributed to the ventromedial prefrontal cortex in inhibitory control. PMID:27723823

  10. High Mobility Group Box Protein-1 Correlates with Renal Function in Chronic Kidney Disease (CKD)

    PubMed Central

    Bruchfeld, Annette; Qureshi, Abdul Rashid; Lindholm, Bengt; Barany, Peter; Yang, LiHong; Stenvinkel, Peter; Tracey, Kevin J

    2008-01-01

    Chronic kidney disease (CKD) is associated with inflammation and malnutrition and carries a markedly increased risk of cardiovascular disease (CVD). High Mobility Group Box Protein-1 (HMGB-1) is a 30-kDa nuclear and cytosolic protein known as a transcription and growth factor, recently identified as a proinflammatory mediator of tissue injury. Recent data implicates HMGB-1 in endotoxin lethality, rheumatoid arthritis, and atherosclerosis. The aim of this post-hoc, cross-sectional study was to determine whether HMGB-1 serum levels are elevated in CKD patients. The study groups were categorized as follows: 110 patients starting dialysis defined as CKD 5; 67 patients with moderately to severely reduced renal function or CKD 3–4; and 48 healthy controls. High-sensitivity C-reactive-protein (hs-CRP), interleukin-6 (IL-6), tumor necrosis factor (TNF), serum-albumin (S-albumin), hemoglobin A1c (HbA1c), hemoglobin, subjective global nutritional assessment (SGA), and glomerular filtration rate (GFR) were analyzed. Kruskal-Wallis test was used to compare groups and Spearman’s rank correlation test was used for continuous variables. HMGB-1, measured by Western blot, was significantly (P < 0.001) elevated in CKD 5 (146.7 ± 58.6 ng/mL) and CKD 3–4 (85.6 ± 31.8) compared with controls (10.9 ± 10.5). HMGB-1 levels were correlated positively with TNF (Rho = 0.52; P < 0.001), hs-CRP (Rho = 0.38; P < 0.001), IL-6 (Rho = 0.30; P < 0.001), HbA1c (Rho = 0.14; P = 0.02) and SGA (Rho = 0.21; P = 0.002) and negatively correlated with GFR (Rho = –0.69; P = 0.0001), Hb (Rho = –0.60; P < 0.001), S-albumin (Rho = –0.31; P < 0.001). The current study has revealed that HMGB-1 is elevated significantly in CKD patients and correlates with GFR as well as markers of inflammation and malnutrition. Future studies may delineate whether HMGB-1 is also a marker of disease activity and severity as well as a predictor of outcome in CKD. PMID:18317568

  11. Correlation of Paraoxonase Status with Disease Activity Score and Systemic Inflammation in Rheumatoid Arthritic Patients

    PubMed Central

    Bindal, Usha Dudeja; Siddiqui, Merajul Haque; Sharma, Dilutpal

    2016-01-01

    Introduction Despite, various preventive efforts on conventional cardiovascular disease (CVD) risk factors, the incidence of CVD in rheumatoid arthritis (RA) patients increases continuously. To solve this conundrum one needs more investigations. Aim The present study was conducted to evaluate the plasma paraoxonase (PON) activity along with the markers of systemic inflammation, oxidative stress and disease activity score-28 (DAS28) in RA patients and clarify their role in determining the probability of RA patients to develop future CVD risk. Materials and Methods Plasma PON, total antioxidant activity (TAA), C-reactive protein (CRP), synovial interleukin-6 (IL-6) and erythrocyte malondialdehyde (MDA) levels were estimated in 40 RA patients aged 40-55 years aged and 40 age-matched healthy controls. The data obtained were compared statistically by using Student’s t-test and Pearson correlation test. Results Besides dyslipidaemia, marked reduction in plasma PON and TAA (p< 0.05) were observed in RA patients as compared with that of healthy controls. Erythrocyte MDA, plasma CRP and synovial IL-6 levels were increased significantly (p<0.05) in RA patients. PON was negatively correlated with MDA (r = - 0.672; p < 0.001), CRP (r = -0.458; p<0.05), IL-6 (r = -0.426; p<0.05) and DAS28 (r = -0.598; p < 0.001), and positively correlated with HDL cholesterol (r = 0.648; p<0.001) and TAA (r = 0.608; p< 0.001) levels in RA patients. Conclusion Alteration in PON activity might contribute to the progression of future CVD risk in RA patients, which may result from interplay of several confounding factors, such as inflammation, oxidative stress and dyslipidaemia. Furthermore, plasma PON activity, CRP and TAA levels could be considered as non-traditional factors to predict CVD risk. Thus, it is suggested that future drugs could be developed to target the non-traditional risk factors in RA patients. PMID:27134854

  12. Prediction of Low versus High Recurrence Scores in Estrogen Receptor-Positive, Lymph Node-Negative Invasive Breast Cancer on the Basis of Radiologic-Pathologic Features: Comparison with Oncotype DX Test Recurrence Scores.

    PubMed

    Dialani, Vandana; Gaur, Shantanu; Mehta, Tejas S; Venkataraman, Shambhavi; Fein-Zachary, Valerie; Phillips, Jordana; Brook, Alexander; Slanetz, Priscilla J

    2016-08-01

    Purpose To review mammographic, ultrasonographic (US), and magnetic resonance (MR) imaging features and pathologic characteristics of estrogen receptor (ER)-positive, lymph node-negative invasive breast cancer and to determine the relationship of these characteristics to Oncotype DX (Genomic Health, Redwood City, Calif) test recurrence scores (ODRS) for breast cancer recurrence. Materials and Methods This institutional review board-approved retrospective study was performed in a single large academic medical center. The study population included patients with ER-positive, lymph node-negative invasive breast cancer who underwent genomic testing from January 1, 2009, to December 31, 2013. Imaging features of the tumor were classified according to the Breast Imaging Reporting and Data System lexicon by breast imagers who were blinded to the ODRS. Mammography was performed in 86% of patients, US was performed in 84%, and MR imaging was performed in 33%, including morphologic and kinetic evaluation. Images from each imaging modality were evaluated. Each imaging finding, progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) status, and tumor grade were then individually correlated with ODRS. Analysis of variance was used to determine differences for each imaging feature. Regression analysis was used to calculate prediction of recurrence on the basis of imaging features combined with histopathologic features. Results The 319 patients had a mean age ± standard deviation of 55 years ± 8.7 (range, 31-82 years). Imaging features with a positive correlation with ODRS included a well-circumscribed oval mass (P = .024) at mammography, vascularity (P = .047) and posterior enhancement (P = .004) at US, and lobulated mass (P = .002) at MR imaging. Recurrence scores were predicted by using these features in combination with PR and HER2 status and tumor grade by using the threshold of more than 30 as a high recurrence score. With a regression tree, there

  13. Clinical Correlations With Lewy Body Pathology in LRRK2-Related Parkinson Disease

    PubMed Central

    Kalia, Lorraine V.; Lang, Anthony E.; Hazrati, Lili-Naz; Fujioka, Shinsuke; Wszolek, Zbigniew K.; Dickson, Dennis W.; Ross, Owen A.; Van Deerlin, Vivianna M.; Trojanowski, John Q.; Hurtig, Howard I.; Alcalay, Roy N.; Marder, Karen S.; Clark, Lorraine N.; Gaig, Carles; Tolosa, Eduardo; Ruiz-Martínez, Javier; Marti-Masso, Jose F.; Ferrer, Isidre; de Munain, Adolfo López; Goldman, Samuel M.; Schüle, Birgitt; Langston, J. William; Aasly, Jan O.; Giordana, Maria T.; Bonifati, Vincenzo; Puschmann, Andreas; Canesi, Margherita; Pezzoli, Gianni; De Paula, Andre Maues; Hasegawa, Kazuko; Duyckaerts, Charles; Brice, Alexis; Stoessl, A. Jon; Marras, Connie

    2015-01-01

    IMPORTANCE Mutations in leucine-rich repeat kinase 2 (LRRK2) are the most common cause of genetic Parkinson disease (PD) known to date. The clinical features of manifesting LRRK2 mutation carriers are generally indistinguishable from those of patients with sporadic PD. However, some PD cases associated with LRRK2 mutations lack Lewy bodies (LBs), a neuropathological hallmark of PD. We investigated whether the presence or absence of LBs correlates with different clinical features in LRRK2-related PD. OBSERVATIONS We describe genetic, clinical, and neuropathological findings of 37 cases of LRRK2-related PD including 33 published and 4 unpublished cases through October 2013. Among the different mutations, the LRRK2 p.G2019S mutation was most frequently associated with LB pathology. Nonmotor features of cognitive impairment/dementia, anxiety, and orthostatic hypotension were correlated with the presence of LBs. In contrast, a primarily motor phenotype was associated with a lack of LBs. CONCLUSIONS AND RELEVANCE To our knowledge, this is the first report of clinicopathological correlations in a series of LRRK2-related PD cases. Findings from this selected group of patients with PD demonstrated that parkinsonian motor features can occur in the absence of LBs. However, LB pathology in LRRK2-related PD may be a marker for a broader parkinsonian symptom complex including cognitive impairment. PMID:25401511

  14. Cytotoxic immune responses in the lungs correlate to disease severity in patients with hantavirus infection.

    PubMed

    Rasmuson, J; Pourazar, J; Mohamed, N; Lejon, K; Evander, M; Blomberg, A; Ahlm, C

    2016-04-01

    Hantavirus infections may cause severe and sometime life-threatening lung failure. The pathogenesis is not fully known and there is an urgent need for effective treatment. We aimed to investigate the association between pulmonary viral load and immune responses, and their relation to disease severity. Bronchoscopy with sampling of bronchoalveolar lavage (BAL) fluid was performed in 17 patients with acute Puumala hantavirus infection and 16 healthy volunteers acting as controls. Lymphocyte subsets, granzyme concentrations, and viral load were determined by flow cytometry, enzyme-linked immunosorbent assay (ELISA), and quantitative reverse transcription polymerase chain reaction (RT-PCR), respectively. Analyses of BAL fluid revealed significantly higher numbers of activated CD8(+) T cells and natural killer (NK) cells, as well as higher concentrations of the cytotoxins granzymes A and B in hantavirus-infected patients, compared to controls. In patients, Puumala hantavirus RNA was detected in 88 % of BAL cell samples and correlated inversely to the T cell response. The magnitude of the pulmonary cytotoxic lymphocyte response correlated to the severity of disease and systemic organ dysfunction, in terms of need for supplemental oxygen treatment, hypotension, and laboratory data indicating renal failure, cardiac dysfunction, vascular leakage, and cell damage. Regulatory T cell numbers were significantly lower in patients compared to controls, and may reflect inadequate immune regulation during hantavirus infection. Hantavirus infection elicits a pronounced cytotoxic lymphocyte response in the lungs. The magnitude of the immune response was associated with disease severity. These results give insights into the pathogenesis and possibilities for new treatments. PMID:26873376

  15. Clinicopathological correlation of psychosis and brain vascular changes in Alzheimer’s disease

    PubMed Central

    Ting, Simon Kang Seng; Hao, Ying; Chia, Pei Shi; Tan, Eng-King; Hameed, Shahul

    2016-01-01

    Psychosis is common in Alzheimer’s disease (AD). However, studies on neuropathology in vascular etiology contributing to psychosis in AD is lacking to date. The aim of this study was to investigate neuropathological vascular related changes in Alzheimer’s disease with psychosis. Data of patients with AD from the National Alzheimer’s Coordinating Center between 2005 to September 2013 was accessed and reviewed. Presence of psychosis was determined based on Neuropsychiatric Inventory Questionnaire taken from the last visit within one year prior to death, and patients were divided into psychosis positive and negative group. Comparison of clinical details and neuropathological vascular changes between the groups was performed using Wilcoxon rank sum test and Chi-square/ Fisher’s exact test. Significant variables were further included in a multivariate logistic model. Overall, 145 patients was included. Of these, 50 patients were psychosis positive. Presence of one or more cortical microinfarcts and moderate to severe arteriosclerosis was found to be positively associated with psychosis. Our results suggest vascular changes correlate with psychosis in Alzheimer’s disease. PMID:26868671

  16. Discriminative Learning for Alzheimer's Disease Diagnosis via Canonical Correlation Analysis and Multimodal Fusion

    PubMed Central

    Lei, Baiying; Chen, Siping; Ni, Dong; Wang, Tianfu

    2016-01-01

    To address the challenging task of diagnosing neurodegenerative brain disease, such as Alzheimer's disease (AD) and mild cognitive impairment (MCI), we propose a novel method using discriminative feature learning and canonical correlation analysis (CCA) in this paper. Specifically, multimodal features and their CCA projections are concatenated together to represent each subject, and hence both individual and shared information of AD disease are captured. A discriminative learning with multilayer feature hierarchy is designed to further improve performance. Also, hybrid representation is proposed to maximally explore data from multiple modalities. A novel normalization method is devised to tackle the intra- and inter-subject variations from the multimodal data. Based on our extensive experiments, our method achieves an accuracy of 96.93% [AD vs. normal control (NC)], 86.57 % (MCI vs. NC), and 82.75% [MCI converter (MCI-C) vs. MCI non-converter (MCI-NC)], respectively, which outperforms the state-of-the-art methods in the literature. PMID:27242506

  17. Joint and fascial chronic graft-vs-host disease: correlations with clinical and laboratory parameters

    PubMed Central

    Vukić, Tamara; Smith, Sean Robinson; Ljubas Kelečić, Dina; Desnica, Lana; Prenc, Ema; Pulanić, Dražen; Vrhovac, Radovan; Nemet, Damir; Pavletic, Steven Z.

    2016-01-01

    Aim To determine if there are correlations between joint and fascial chronic graft-vs-host disease (cGVHD) with clinical findings, laboratory parameters, and measures of functional capacity. Methods 29 patients were diagnosed with cGVHD based on National Institutes of Health (NIH) Consensus Criteria at the University Hospital Centre Zagreb from October 2013 to October 2015. Physical examination, including functional measures such as 2-minute walk test and hand grip strength, as well as laboratory tests were performed. The relationship between these evaluations and the severity of joint and fascial cGVHD was tested by logistical regression analysis. Results 12 of 29 patients (41.3%) had joint and fascial cGVHD diagnosed according to NIH Consensus Criteria. There was a significant positive correlation of joint and fascial cGVHD and skin cGVHD (P < 0.001), serum C3 complement level (P = 0.045), and leukocytes (P = 0.032). There was a significant negative correlation between 2-minute walk test (P = 0.016), percentage of cytotoxic T cells CD3+/CD8+ (P = 0.022), serum albumin (P = 0.047), and Karnofsky score (P < 0.001). Binary logistic regression model found that a significant predictor for joint and fascial cGVHD was cGVHD skin involvement (odds ratio, 7.79; 95 confidence interval 1.87-32.56; P = 0.005). Conclusion Joint and fascial cGVHD manifestations correlated with multiple laboratory measurements, clinical features, and cGVHD skin involvement, which was a significant predictor for joint and fascial cGVHD. PMID:27374828

  18. Endoscopic evaluation of celiac disease severity and its correlation with histopathological aspects of the duodenal mucosa

    PubMed Central

    Bonatto, Mauro W.; Kotze, Luiz; Orlandoski, Marcia; Tsuchyia, Ricardo; de Carvalho, Carlos A.; Lima, Doryane; Kurachi, Gustavo; Orso, Ivan R.B.; Kotze, Lorete

    2016-01-01

    Background and study aims: Celiac disease (CD) is a chronic systemic autoimmune disorder affecting genetically predisposed individuals, triggered and maintained by the ingestion of gluten. Triggered and maintained by the ingestion of gluten, celiac disease is a chronic systemic autoimmune disorder affecting genetically predisposed individuals. Persistent related inflammation of the duodenal mucosa causes atrophy architecture detectable on esophagogastroduodenoscopy (EGD) and histopathology. We investigated the association between endoscopic features and histopathological findings (Marsh) for duodenal mucosa in celiac disease patients and propose an endoscopic classification of severity. Patients and methods: Between January 2000 and March 2010, an electronic database containing 34,540 EDGs of patients aged > 14 years was searched for cases of CD. Out of 109 cases, 85 met the inclusion criteria: conventional EGD combined with chromoendoscopy, zoom and biopsy. EGD types 0, I and II corresponds to Marsh grades 0, 1 and 2, respectively, while EGD type III corresponds to Marsh grade 3 and 4. Results: Five patients (5.8 %) were EGD I but not Marsh grade 1; 25 patients (29.4 %) were EGD II, 4 of whom (16 %) were classified as Marsh grade 2; and 55 patients (64.7 %) were EGD III, 51 (92.7 %) of whom were classified as Marsh grades 3 and 4. The Spearman correlation coefficient (r = 0.33) revealed a significant association between the methods (P = 0.002). Conclusions: Changes in the duodenal mucosa detected on EGD were significantly and positively associated with histopathologic findings. The use of chromoendoscopy in addition to conventional EGD enhances changes in the duodenal mucosa and permits diagnosis of CD, even in routine examinations. The proposed endoscopic classification is practical and easily reproducible and provides valuable information regarding disease extension. PMID:27556094

  19. Correlates of dietary energy sources with cardiovascular disease risk markers in Mexican school-age children.

    PubMed

    Perichart-Perera, Otilia; Balas-Nakash, Margie; Rodríguez-Cano, Ameyalli; Muñoz-Manrique, Cinthya; Monge-Urrea, Adriana; Vadillo-Ortega, Felipe

    2010-02-01

    Dietary and lifestyle changes in Mexico have been linked to an increase in chronic diseases such as obesity and cardiovascular disease. Important dietary changes such as an increase in the consumption of energy-dense foods (high in oils, animal or processed fats, and sugars) have been recently reported. The objective of this study was to identify how key dietary energy sources correlated with other indexes of cardiovascular disease in a Mexican school-age population. From 2004 to 2006, a convenience sample (n=228) of 9- to 13-year-olds, 48.2% girls and 51.8% boys, from three public urban schools were included. Anthropometric, blood pressure, and dietary assessment (two multiple pass 24-hour recalls) were done. More than half of children did not meet the fruit and vegetable recommended intake. High-fat dairy foods (14% of total energy intake), refined carbohydrates (13.5%), red/processed meat (8.5%), added sugars/desserts (7%), corn tortilla (6.5%), and soft drinks/sweetened beverages (5%) were the highest dietary energy sources consumed. In a subgroup of children (n=185), a fasting blood sample was collected for biochemical analysis. A positive association was observed between glucose and diastolic blood pressure with the intake of soft drinks/sweetened beverages, insulin concentrations and the intake of white bread, and triglyceride concentrations with the intake of added fats. Unhealthful dietary energy sources are frequently consumed by these children. Culturally competent nutrition counseling should be offered to Mexican-American children and their families with a significant risk of cardiovascular disease. Efforts should be made to design and implement nutrition education and health promotion strategies in schools. PMID:20102853

  20. Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome.

    PubMed

    van den Munckhof, Bart; de Vries, Evelien E; Braun, Kees P J; Boss, H Myrthe; Willemsen, Michèl A; van Royen-Kerkhof, Annet; de Jager, Wilco; Jansen, Floor E

    2016-02-01

    We aimed to study serum cytokine levels in 11 electrical status epilepticus in sleep (ESES) patients and 20 healthy control children. Patients showed significantly higher levels of interleukin (IL)-1α, IL-6, IL-10, chemokine (C-C motif) ligand (CCL)2 and chemokine (C-X-C motif) ligand (CXCL)8/IL-8 than controls, while macrophage migration inhibitory factor (MIF) and CCL3 were significantly lower. Follow-up analyses in five patients revealed a significant decrease of IL-6 levels after immunomodulating treatment. IL-6 changes were accompanied by clear improvement of electroencephalography (EEG) patterns and neuropsychological evaluation. We hypothesize that IL-6 correlates with disease activity and immunomodulating treatment efficacy.

  1. Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome.

    PubMed

    van den Munckhof, Bart; de Vries, Evelien E; Braun, Kees P J; Boss, H Myrthe; Willemsen, Michèl A; van Royen-Kerkhof, Annet; de Jager, Wilco; Jansen, Floor E

    2016-02-01

    We aimed to study serum cytokine levels in 11 electrical status epilepticus in sleep (ESES) patients and 20 healthy control children. Patients showed significantly higher levels of interleukin (IL)-1α, IL-6, IL-10, chemokine (C-C motif) ligand (CCL)2 and chemokine (C-X-C motif) ligand (CXCL)8/IL-8 than controls, while macrophage migration inhibitory factor (MIF) and CCL3 were significantly lower. Follow-up analyses in five patients revealed a significant decrease of IL-6 levels after immunomodulating treatment. IL-6 changes were accompanied by clear improvement of electroencephalography (EEG) patterns and neuropsychological evaluation. We hypothesize that IL-6 correlates with disease activity and immunomodulating treatment efficacy. PMID:26666401

  2. Mobilization of Circulating Endothelial Progenitor Cells Correlates with the Clinical Course of Hantavirus Disease

    PubMed Central

    Grouls, Stephan; Hettwer, David; Rafat, Neysan; Tönshoff, Burkhard; Zeier, Martin

    2014-01-01

    Infections with hemorrhagic fever viruses are characterized by increased permeability leading to capillary leakage. Hantavirus infection is associated with endothelial dysfunction, and the clinical course is related to the degree of vascular injury. Circulating endothelial progenitor cells (cEPCs) play a pivotal role in the repair of the damaged endothelium. Therefore, we analyzed the number of cEPCs and their mobilizing growth factors in patients suffering from hantavirus disease induced by infection with Puumala virus. The numbers of EPCs of 36 hantavirus-infected patients and age- and gender-matched healthy controls were analyzed by flow cytometry. Concentrations of cEPC-mobilizing growth factors in plasma were determined by enzyme-linked immunosorbent assay. Laboratory parameters were correlated with the number of cEPCs. In patients infected with hantavirus, the number of cEPCs was significantly higher than that in healthy controls. Levels of mobilizing cytokines were upregulated in patients, and the mobilization of cEPCs is paralleled with the normalization of clinical parameters. Moreover, higher levels of cEPCs correlated with higher serum albumin levels and platelet concentrations. Our data indicate that cEPCs may play a role in the repair of hantavirus-induced endothelial damage, thereby influencing the clinical course and the severity of symptoms. PMID:24155401

  3. [Genetic characterization and correlation among fragments of HN gene of the field Newcastle disease viruses].

    PubMed

    Qin, Zhuo-Ming; Ma, Bao-Chen; Yuan, Xiao-Yuan; Xu, Huai-Ying; He, Ye-Feng; Cui, Zhi-Zhong

    2007-01-01

    Twenty-four isolates of Newcastle disease virus (NDV) prevailing during 1997 -- 2005 in China were collected. These isolates were purified by CEF plaque assay and replicated in SPF chicken embryos. The hemagglutinin-neuraminidase (HN) genes of these viruses were cloned and sequenced. The HN gene sequences of thirty-six NDV reference strains in GenBank were also used in this study. The amino acid homologing of these viruses were compared and analyzed. The correlations among different fragments of HN gene were also analyzed. The results indicated that the homology of Chinese field NDV strains was 94.4%-99.4%, but 86.9%-89% compared with LaSota and Clone30, 87.9%-89.9% to F48E9, and 87.2%-96.2% to foreign NDV strains. There had the nearest distances among Chinese NDV isolates as compared with that of the LaSota, Clone30 and F48E9 by the phylogenetic tree. However, the distances of seven foreign NDV isolates were very close to Chinese NDV isolates as compared with these of the other foreign NDV isolates. We also found that all the Chinese field isolates were devoid of glycosylation site in position 538 -- 540. There were good correlations between different length amino acid fragments and the genomes of HN, especially the 5'-terminus first 80aa.

  4. Neuroanatomical correlates of impaired decision-making and facial emotion recognition in early Parkinson's disease.

    PubMed

    Ibarretxe-Bilbao, Naroa; Junque, Carme; Tolosa, Eduardo; Marti, Maria-Jose; Valldeoriola, Francesc; Bargallo, Nuria; Zarei, Mojtaba

    2009-09-01

    Decision-making and recognition of emotions are often impaired in patients with Parkinson's disease (PD). The orbitofrontal cortex (OFC) and the amygdala are critical structures subserving these functions. This study was designed to test whether there are any structural changes in these areas that might explain the impairment of decision-making and recognition of facial emotions in early PD. We used the Iowa Gambling Task (IGT) and the Ekman 60 faces test which are sensitive to the integrity of OFC and amygdala dysfunctions in 24 early PD patients and 24 controls. High-resolution structural magnetic resonance images (MRI) were also obtained. Group analysis using voxel-based morphometry (VBM) showed significant and corrected (P < 0.05 FEW-small volume correction) gray matter (GM) loss in the right amygdala and bilaterally in the OFC in PD patients. Volumetric analyses were also performed but did not yield significant differences between groups. Left lateral GM volume in OFC showed a slight correlation with the IGT, and bilateral OFC GM was strongly correlated with Ekman test performance in PD patients. We conclude that: (i) impairment in decision-making and recognition of facial emotions occurs at the early stages of PD, (ii) these neuropsychological deficits are accompanied by degeneration of OFC and amygdala, and (iii) bilateral OFC reductions are associated with impaired recognition of emotions, and GM volume loss in left lateral OFC is related to decision-making impairment in PD.

  5. Subthalamic nucleus phase–amplitude coupling correlates with motor impairment in Parkinson’s disease

    PubMed Central

    van Wijk, Bernadette C.M.; Beudel, Martijn; Jha, Ashwani; Oswal, Ashwini; Foltynie, Tom; Hariz, Marwan I.; Limousin, Patricia; Zrinzo, Ludvic; Aziz, Tipu Z.; Green, Alexander L.; Brown, Peter; Litvak, Vladimir

    2016-01-01

    Objective High-amplitude beta band oscillations within the subthalamic nucleus are frequently associated with Parkinson’s disease but it is unclear how they might lead to motor impairments. Here we investigate a likely pathological coupling between the phase of beta band oscillations and the amplitude of high-frequency oscillations around 300 Hz. Methods We analysed an extensive data set comprising resting-state recordings obtained from deep brain stimulation electrodes in 33 patients before and/or after taking dopaminergic medication. We correlated mean values of spectral power and phase–amplitude coupling with severity of hemibody bradykinesia/rigidity. In addition, we used simultaneously recorded magnetoencephalography to look at functional interactions between the subthalamic nucleus and ipsilateral motor cortex. Results Beta band power and phase–amplitude coupling within the subthalamic nucleus correlated positively with severity of motor impairment. This effect was more pronounced within the low-beta range, whilst coherence between subthalamic nucleus and motor cortex was dominant in the high-beta range. Conclusions We speculate that the beta band might impede pro-kinetic high-frequency activity patterns when phase–amplitude coupling is prominent. Furthermore, results provide evidence for a functional subdivision of the beta band into low and high frequencies. Significance Our findings contribute to the interpretation of oscillatory activity within the cortico-basal ganglia circuit. PMID:26971483

  6. Dynamic correlation between intrahost HIV-1 quasispecies evolution and disease progression.

    PubMed

    Lee, Ha Youn; Perelson, Alan S; Park, Su-Chan; Leitner, Thomas

    2008-12-01

    Quantifying the dynamics of intrahost HIV-1 sequence evolution is one means of uncovering information about the interaction between HIV-1 and the host immune system. In the chronic phase of infection, common dynamics of sequence divergence and diversity have been reported. We developed an HIV-1 sequence evolution model that simulated the effects of mutation and fitness of sequence variants. The amount of evolution was described by the distance from the founder strain, and fitness was described by the number of offspring a parent sequence produces. Analysis of the model suggested that the previously observed saturation of divergence and decrease of diversity in later stages of infection can be explained by a decrease in the proportion of offspring that are mutants as the distance from the founder strain increases rather than due to an increase of viral fitness. The prediction of the model was examined by performing phylogenetic analysis to estimate the change in the rate of evolution during infection. In agreement with our modeling, in 13 out of 15 patients (followed for 3-12 years) we found that the rate of intrahost HIV-1 evolution was not constant but rather slowed down at a rate correlated with the rate of CD4+ T-cell decline. The correlation between the dynamics of the evolutionary rate and the rate of CD4+ T-cell decline, coupled with our HIV-1 sequence evolution model, explains previously conflicting observations of the relationships between the rate of HIV-1 quasispecies evolution and disease progression.

  7. Neuropsychological correlates of theory of mind in patients with early Parkinson's disease.

    PubMed

    Santangelo, Gabriella; Vitale, Carmine; Trojano, Luigi; Errico, Domenico; Amboni, Marianna; Barbarulo, Anna Maria; Grossi, Dario; Barone, Paolo

    2012-01-01

    The theory of mind is the ability to attribute mental states to oneself and others and to understand that others have beliefs, desires and intentions different from one's own. The aim of the study was to explore the neuropsychological correlates of theory of mind in patients affected by early Parkinson's disease (PD). Thirty-three PD patients and 33 age-, sex-, and education-matched control subjects underwent the Frontal Assessment Battery, as well as tasks assessing "cognitive" and "affective" theory of mind, and memory abilities; questionnaires evaluating behavioral disorders and quality of life were also administrated. Although the 2 groups did not differ on neuropsychological tasks, PD patients' performance on tasks assessing cognitive and affective theory of mind was significantly worse than controls. Moreover, PD patients had more behavioral disorders and worse quality of life than controls. After covarying for behavioral and quality of life scores, the differences between patients and controls on theory of mind tasks remained significant. "Cognitive" theory of mind was associated with Frontal Assessment Battery score and 2 domains of quality of life scale, whereas "affective" theory of mind scores correlated only with behavioral scales such as the Frontal Behavioral Inventory and Apathy Evaluation Scale. The results demonstrate that both affective and cognitive aspects of theory of mind are simultaneously impaired in early PD and suggest that deficits in the 2 subcomponents of theory of mind may be linked to dysfunction of different frontosubcortical circuitries in early PD. PMID:21915910

  8. Ventromedial-frontopolar prefrontal cortex atrophy correlates with insight loss in frontotemporal dementia and Alzheimer's disease.

    PubMed

    Hornberger, Michael; Yew, Belinda; Gilardoni, Silvia; Mioshi, Eneida; Gleichgerrcht, Ezequiel; Manes, Facundo; Hodges, John R

    2014-02-01

    Loss in insight is a major feature of frontotemporal dementia (FTD) but has been investigated relatively little. More importantly, the neural basis of insight loss is still poorly understood. The current study investigated insight deficit profiles across a large cohort of neurodegenerative patients (n = 81), including FTD and Alzheimer's disease (AD) patients. We employed a novel insight questionnaire, which tapped into changes across different domains: social interaction, emotion, diagnosis/treatment, language, and motivation. FTD subtypes varied considerably for insight loss, with the behavioral variant worst and the progressive non-fluent variant least affected. All other subtypes and AD showed milder but consistent insight loss. Voxel-based morphometry analysis revealed that overall insight loss correlated with ventromedial and frontopolar prefrontal atrophy, with exception of social interaction and emotion insight loss, which additionally correlated with lateral temporal and amygdala atrophy, respectively. Our results show that patients with neurodegenerative conditions show variable loss of insight, with ventromedial and frontopolar cortex regions appearing to be particularly important for insight.

  9. Transcriptional correlates of disease outcome in anticoagulant-treated non-human primates infected with ebolavirus.

    PubMed

    Garamszegi, Sara; Yen, Judy Y; Honko, Anna N; Geisbert, Joan B; Rubins, Kathleen H; Geisbert, Thomas W; Xia, Yu; Hensley, Lisa E; Connor, John H

    2014-01-01

    Ebola virus (EBOV) infection in humans and non-human primates (NHPs) is highly lethal, and there is limited understanding of the mechanisms associated with pathogenesis and survival. Here, we describe a transcriptomic analysis of NHPs that survived lethal EBOV infection, compared to NHPs that did not survive. It has been previously demonstrated that anticoagulant therapeutics increase the survival rate in EBOV-infected NHPs, and that the characteristic transcriptional profile of immune response changes in anticoagulant-treated NHPs. In order to identify transcriptional signatures that correlate with survival following EBOV infection, we compared the mRNA expression profile in peripheral blood mononuclear cells from EBOV-infected NHPs that received anticoagulant treatment, to those that did not receive treatment. We identified a small set of 20 genes that are highly confident predictors and can accurately distinguish between surviving and non-surviving animals. In addition, we identified a larger predictive signature of 238 genes that correlated with disease outcome and treatment; this latter signature was associated with a variety of host responses, such as the inflammatory response, T cell death, and inhibition of viral replication. Notably, among survival-associated genes were subsets of genes that are transcriptionally regulated by (1) CCAAT/enhancer-binding protein alpha, (2) tumor protein 53, and (3) megakaryoblastic leukemia 1 and myocardin-like protein 2. These pathways merit further investigation as potential transcriptional signatures of host immune response to EBOV infection. PMID:25079789

  10. Proliferating cell nuclear antigen in oesophageal diseases; correlation with transforming growth factor alpha expression.

    PubMed Central

    Jankowski, J; McMenemin, R; Yu, C; Hopwood, D; Wormsley, K G

    1992-01-01

    This study was designed to correlate mucosal proliferation in Barrett's oesophagus with expression of a growth promoting peptide, transforming growth factor alpha (TGF alpha). Oesophageal mucosa was studied from 50 patients with oesophageal disease who had been treated by oesophagectomy. Histological analysis showed a range of oesophageal pathology - 18 patients had gastric type Barrett's mucosa, 18 had intestinal type Barrett's mucosa, and 14 had oesophageal adenocarcinomas. Sections were stained immunohistochemically for proliferating cell nuclear antigen (PCNA) (an index of cellular proliferation) and TGF alpha. PCNA immunostaining was seen mainly in the basal cells of the neck/foveolar epithelial compartment of the glands in Barrett's oesophagus. However, in mucosa with high grade dysplasia, the proliferative compartment extended upwards into the superficial layers of the glands. At least 2000 cells were counted in each patient to determine the proportion with PCNA immunoreactivity (PCNA labelling index). The labelling index was highest in adenocarcinoma (25%) and in Barrett's intestinal type mucosa with high grade dysplasia (26%) compared with intestinal type mucosa with no significant dysplasia (20%) and Barrett's gastric type mucosa (12%). There was a significant positive correlation between PCNA labelling indices and TGF alpha expression in Barrett's mucosa (p less than 0.01). In glands showing high grade dysplasia, TGF alpha immunoreactivity was seen in the same regions of the glands as PCNA immunoreactivity, indicating the possibility of involvement of TGF alpha in (pre) neoplastic proliferation in Barrett's oesophagus. Images Figure 2 Figure 5 PMID:1351861

  11. Brain perfusion correlates of visuoperceptual deficits in Mild Cognitive Impairment and mild Alzheimer’s disease

    PubMed Central

    Alegret, Montserrat; Vinyes-Junqué, Georgina; Boada, Mercè; Martínez-Lage, Pablo; Cuberas, Gemma; Espinosa, Ana; Roca, Isabel; Hernández, Isabel; Valero, Sergi; Rosende-Roca, Maitée; Mauleón, Ana; Becker, James T.; Tárraga, Lluís

    2012-01-01

    Background Visuoperceptual processing is impaired early in the clinical course of Alzheimer’s disease (AD). The 15-Objects Test (15-OT) detects such subtle performance deficits in Mild Cognitive Impairment (MCI) and mild AD. Reduced brain perfusion in the temporal, parietal and prefrontal regions have been found in early AD and MCI patients. Objectives To confirm the role of the 15-OT in the diagnosis of MCI and AD, and to investigate the brain perfusion correlates of visuoperceptual dysfunction (15-OT) in subjects with MCI, AD and normal aging. Methods Forty-two AD, 42 MCI and 42 healthy elderly control (EC) subjects underwent a brain Single Photon Emission Tomography (SPECT) and separately completed the 15-OT. An analysis of variance compared 15-OT scores between groups. SPM5 was used to analyse the SPECT data. Results 15-OT performace was impaired in the MCI and AD patients. In terms of the SPECT scans, AD patients showed reduced perfusion in temporal-parietal regions, while the MCI subjects had decreased perfusion in the middle and posterior cingulate. When MCI and AD groups were compared, a significant brain perfusion reduction was found in temporo-parietal regions. In the whole sample, 15-OT performance was significantly correlated with the clinical dementia rating scores, and with the perfusion in the bilateral posterior cingulate and the right temporal pole, with no significant correlation in each separate group. Conclusion Our findings suggest that the 15-OT performance provides a useful gradation of impairment from normal aging to AD, and it seems to be related to perfusion in the bilateral posterior cingulate and the right temporal pole. PMID:20555146

  12. Buccal Cell Cytokeratin 14 Correlates with Multiple Blood Biomarkers of Alzheimer's Disease Risk.

    PubMed

    Leifert, Wayne R; Nguyen, Tori; Rembach, Alan; Martins, Ralph; Rainey-Smith, Stephanie; Masters, Colin L; Ames, David; Rowe, Christopher C; Macaulay, S Lance; François, Maxime; Fenech, Michael F

    2015-01-01

    Mild cognitive impairment (MCI) may reflect early stages of neurodegenerative disorders such as Alzheimer's disease (AD). Our hypothesis was that cytokeratin 14 (CK14) expression could be used with blood-based biomarkers such as homocysteine, vitamin B12, and folate to identify individuals with MCI or AD from the Australian Imaging, Biomarkers and Lifestyle (AIBL) flagship study of aging. Buccal cells from 54 individuals were analyzed by a newly developed method that is rapid, automated, and quantitative for buccal cell CK14 expression levels. CK14 was negatively correlated with plasma Mg²⁺ and LDL, while positively correlated with vitamin B12, red cell hematocrit/volume, and basophils in the MCI group and positively correlated with insulin and vitamin B12 in the AD group. The combined biomarker panel (CK14 expression, plasma vitamin B12, and homocysteine) was significantly lower in the MCI (p = 0.003) and AD (p = 0.0001) groups compared with controls. Receiver-operating characteristic curves yielded area under the curve (AUC) values of 0.829 for the MCI (p = 0.002) group and 0.856 for the AD (p = 0.0003) group. These complex associations of multiple related parameters highlight the differences between the MCI and AD cohorts and possibly an underlying metabolic pathology associated with the development of early memory impairment. The changes in buccal cell CK14 expression observed in this pilot study supports previous results suggesting the peripheral biomarkers and metabolic changes are not restricted to brain pathology alone in MCI and AD and could prove useful as a potential biomarker in identifying individuals with an increased risk of developing MCI and eventually AD. PMID:26402008

  13. Buccal Cell Cytokeratin 14 Correlates with Multiple Blood Biomarkers of Alzheimer's Disease Risk.

    PubMed

    Leifert, Wayne R; Nguyen, Tori; Rembach, Alan; Martins, Ralph; Rainey-Smith, Stephanie; Masters, Colin L; Ames, David; Rowe, Christopher C; Macaulay, S Lance; François, Maxime; Fenech, Michael F

    2015-01-01

    Mild cognitive impairment (MCI) may reflect early stages of neurodegenerative disorders such as Alzheimer's disease (AD). Our hypothesis was that cytokeratin 14 (CK14) expression could be used with blood-based biomarkers such as homocysteine, vitamin B12, and folate to identify individuals with MCI or AD from the Australian Imaging, Biomarkers and Lifestyle (AIBL) flagship study of aging. Buccal cells from 54 individuals were analyzed by a newly developed method that is rapid, automated, and quantitative for buccal cell CK14 expression levels. CK14 was negatively correlated with plasma Mg²⁺ and LDL, while positively correlated with vitamin B12, red cell hematocrit/volume, and basophils in the MCI group and positively correlated with insulin and vitamin B12 in the AD group. The combined biomarker panel (CK14 expression, plasma vitamin B12, and homocysteine) was significantly lower in the MCI (p = 0.003) and AD (p = 0.0001) groups compared with controls. Receiver-operating characteristic curves yielded area under the curve (AUC) values of 0.829 for the MCI (p = 0.002) group and 0.856 for the AD (p = 0.0003) group. These complex associations of multiple related parameters highlight the differences between the MCI and AD cohorts and possibly an underlying metabolic pathology associated with the development of early memory impairment. The changes in buccal cell CK14 expression observed in this pilot study supports previous results suggesting the peripheral biomarkers and metabolic changes are not restricted to brain pathology alone in MCI and AD and could prove useful as a potential biomarker in identifying individuals with an increased risk of developing MCI and eventually AD.

  14. Clinical Correlates of Apathy in Patients Recently Diagnosed with Parkinson's Disease: The ANIMO Study

    PubMed Central

    Cubo, Esther; Benito-León, Julián; Coronell, Carlos; Armesto, Diana

    2012-01-01

    Objective Little is known about apathy in the early stages of Parkinson's disease (PD). We determined the clinical correlates of apathy in a large representative sample of patients recently diagnosed with PD (ANIMO study). Methods PD patients, diagnosed within 2 years of inclusion, were recruited in 102 outpatient clinics situated in 82 populations throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale (LARS). Clinical comparisons and correlations were performed using nonparametric tests. Regression analyses were used to test the association of clinical variables with apathy. Results We recruited 557 PD patients (60.3% men) with a mean age of 68.8 ± 9.7 years, and UPDRS motor score of 21.1 ± 10.8. Apathy only was diagnosed in 186 (33.4%), and apathy and depression in 215 patients (38.6%). Patients with higher comorbidity (OR = 1.10, 95% CI 1.01−1.20, p = 0.001), motor impairment (OR = 1.07, 95% CI 1.03−1.10, p < 0.0001), and lower education (OR = 2.16, 95% CI 1.21−;3.85, p = 0.009) had higher odds of having apathy, in contrast to patients living in a rural environment (OR = 0.35, 95% CI 0.32–0.85, p = 0.01), and left predominant PD motor laterality (OR = 0.34, 95% CI 0.13–0.88, p = 0.01). LARS scores were significantly correlated with UPDRS motor scores (rs = 0.44, p < 0.001), predominantly with axial score (rs = 0.43, p < 0.001). Conclusions In PD, apathy is a very common and disabling nonmotor symptom separable from depression. Patients living in a rural environment, with lower comorbidity and motor impairment, higher education background, and left predominant PD motor laterality are at lower risk of suffering from apathy. PMID:22236943

  15. Severity of atopic disease inversely correlates with intestinal microbiota diversity and butyrate-producing bacteria.

    PubMed

    Nylund, L; Nermes, M; Isolauri, E; Salminen, S; de Vos, W M; Satokari, R

    2015-02-01

    The reports on atopic diseases and microbiota in early childhood remain contradictory, and both decreased and increased microbiota diversity have been associated with atopic eczema. In this study, the intestinal microbiota signatures associated with the severity of eczema in 6-month-old infants were characterized. Further, the changes in intestinal microbiota composition related to the improvement of this disease 3 months later were assessed. The severity of eczema correlated inversely with microbiota diversity (r = -0.54, P = 0.002) and with the abundance of butyrate-producing bacteria (r = -0.52, P = 0.005). During the 3-month follow-up, microbiota diversity increased (P < 0.001) and scoring atopic dermatitis values decreased (P < 0.001) in all infants. This decrease coincided with the increase in bacteria related to butyrate-producing Coprococcus eutactus (r = -0.59, P = 0.02). In conclusion, the high diversity of microbiota and high abundance of butyrate-producing bacteria were associated with milder eczema, thus suggesting they have a role in alleviating symptoms of atopic eczema.

  16. Depression in end-stage renal disease patients treated with hemodialysis: tools, correlates, outcomes, and needs.

    PubMed

    Kimmel, Paul L; Peterson, Rolf A

    2005-01-01

    Depression has been thought to be the most common psychiatric abnormality in hemodialysis (HD) patients. There are few data using psychiatric diagnostic criteria and a lack of large, well-designed epidemiologic research studies in patients with end-stage renal disease (ESRD) that can render definitive results on this topic. The prevalence of major depression or a defined psychiatric illness in ESRD patients is unknown, but is probably between 5% and 10%. The prevalence of increased levels of depressive affect is greater. Estimates of the prevalence will vary according to the screening techniques used. Depression could affect medical outcomes in ESRD patients through several mechanisms. Correlational analyses suggest stressors and protective factors play roles in mediating the level of depressive affect and associated outcomes. Although early studies suggested a deleterious effect of depression on survival in ESRD patients, more recent studies had failed to confirm such findings. The use of longitudinal analyses and larger samples has confirmed an association of depressive affect and morbidity and mortality in more contemporary ESRD populations. The importance of depressive affect compared with the presence of a defined psychiatric syndrome in mediating clinically important outcomes in patients with chronic kidney disease has not been determined. Studies of interventions designed to reduce levels of depressive affect in ESRD patients are urgently needed.

  17. Pathological and Clinical Correlation between Celiac Disease and Helicobacter Pylori Infection; a Review of Controversial Reports.

    PubMed

    Rostami-Nejad, Mohammad; Javad Ehsani-Ardakani, Mohammad; Assadzadeh, Hamid; Shahbazkhani, Bijan; Ierardi, Enzo; Losurdo, Giuseppe; Zojaji, Homayon; Alizadeh, Amirhoshang Mohammad; Naderi, Nosratollah; Sadeghi, Amir; Zali, Mohammad Reza

    2016-04-01

    There are overwhelming reports and descriptions about celiac associated disorders. Although there is a clear genetic association between celiac disease (CD) and some gastrointestinal disorders, there are controversial reports claiming an association between CD and Helicobacter pylori (H. pylori) infection. Different studies indicated the possible association between lymphocytic gastritis and both CD and H. pylori infection, although this evidence is not consistently accepted. Also it was shown that an increase in intraepithelial lymphocytes count is associated with both H. pylori infection and celiac disease. Therefore the following questions may raise: how far is this infection actually related to CD?, which are the underlying patho-mechanisms for these associations? what are the clinical implications? what is the management? and what would be the role of gluten free diet in treating these conditions? PubMed (PubMed Central), Ovid, ISI of web knowledge, and Google scholar were searched for full text articles published between 1985 and 2015. The associated keywords were used, and papers described particularly the impact of pathological and clinical correlation between CD and H. pylori infection were identified. In this review we tried to answer the above questions and discussed some of the recent developments in the pathological and clinical aspects of CD and H. pylori infection. PMID:27252814

  18. Pathological and Clinical Correlation between Celiac Disease and Helicobacter Pylori Infection; a Review of Controversial Reports

    PubMed Central

    Rostami-Nejad, Mohammad; Javad Ehsani-Ardakani, Mohammad; Assadzadeh, Hamid; Shahbazkhani, Bijan; Ierardi, Enzo; Losurdo, Giuseppe; Zojaji, Homayon; Alizadeh, Amirhoshang Mohammad; Naderi, Nosratollah; Sadeghi, Amir; Zali, Mohammad Reza

    2016-01-01

    There are overwhelming reports and descriptions about celiac associated disorders. Although there is a clear genetic association between celiac disease (CD) and some gastrointestinal disorders, there are controversial reports claiming an association between CD and Helicobacter pylori (H. pylori) infection. Different studies indicated the possible association between lymphocytic gastritis and both CD and H. pylori infection, although this evidence is not consistently accepted. Also it was shown that an increase in intraepithelial lymphocytes count is associated with both H. pylori infection and celiac disease. Therefore the following questions may raise: how far is this infection actually related to CD?, which are the underlying patho-mechanisms for these associations? what are the clinical implications? what is the management? and what would be the role of gluten free diet in treating these conditions? PubMed (PubMed Central), Ovid, ISI of web knowledge, and Google scholar were searched for full text articles published between 1985 and 2015. The associated keywords were used, and papers described particularly the impact of pathological and clinical correlation between CD and H. pylori infection were identified. In this review we tried to answer the above questions and discussed some of the recent developments in the pathological and clinical aspects of CD and H. pylori infection. PMID:27252814

  19. Hippocampal transcriptome-guided genetic analysis of correlated episodic memory phenotypes in Alzheimer's disease

    PubMed Central

    Yan, Jingwen; Kim, Sungeun; Nho, Kwangsik; Chen, Rui; Risacher, Shannon L.; Moore, Jason H.; Saykin, Andrew J.; Shen, Li

    2015-01-01

    As the most common type of dementia, Alzheimer's disease (AD) is a neurodegenerative disorder initially manifested by impaired memory performances. While the diagnosis information indicates a dichotomous status of a patient, memory scores have the potential to capture the continuous nature of the disease progression and may provide more insights into the underlying mechanism. In this work, we performed a targeted genetic study of memory scores on an AD cohort to identify the associations between a set of genes highly expressed in the hippocampal region and seven cognitive scores related to episodic memory. Both main effects and interaction effects of the targeted genetic markers on these correlated memory scores were examined. In addition to well-known AD genetic markers APOE and TOMM40, our analysis identified a new risk gene NAV2 through the gene-level main effect analysis. NAV2 was found to be significantly and consistently associated with all seven episodic memory scores. Genetic interaction analysis also yielded a few promising hits warranting further investigation, especially for the RAVLT list B Score. PMID:25859259

  20. Correlation between CAG Repeat Length and Clinical Features in Machado-Joseph Disease

    PubMed Central

    Maciel, Patrícia; Gaspar, Claudia; DeStefano, Anita L.; Silveira, Isabel; Coutinho, Paula; Radvany, João; Dawson, David M.; Sudarsky, Lewis; Guimarães, João; Loureiro, Jose E. L.; Nezarati, Marjan M.; Corwin, Lee I.; Lopes-Cendes, Iscia; Rooke, Karen; Rosenberg, Roger; MacLeod, Patrick; Farrer, Lindsay A.; Sequeiros, Jorge; Rouleau, Guy A.

    1995-01-01

    Machado-Joseph disease (MJD) is associated with the expansion of a CAG trinucleotide repeat in a novel gene on 14q32.1. We confirmed the presence of this expansion in 156 MJD patients from 33 families of different geographic origins: 15 Portuguese Azorean, 2 Brazilian, and 16 North American of Portuguese Azorean descent. Normal chromosomes contain between 12 and 37 CAG repeats in the MJD gene, whereas MJD gene carriers have alleles within the expanded range of 62–84 CAG units. The distribution of expanded alleles and the gap between normal and expanded allele sizes is either inconsistent with a premutation hypothesis or most (if not all) of the alleles we studied descend from a common ancestor. There is a strong correlation between the expanded repeat size and the age at onset of the disease as well as the clinical presentation. There is mild instability of the CAG tract length with transmission of the expanded alleles; both increase and decrease in size between parents and progeny occur, with larger variations in male than in female transmissions. Together, these effects can partly explain the variability of age at onset and of phenotypic features in MJD; however, other modifying factors must exist. ImagesFigure 3 PMID:7611296

  1. Circulating plasma cells in multiple myeloma: characterization and correlation with disease stage.

    PubMed

    Rawstron, A C; Owen, R G; Davies, F E; Johnson, R J; Jones, R A; Richards, S J; Evans, P A; Child, J A; Smith, G M; Jack, A S; Morgan, G J

    1997-04-01

    The aim of this study was to develop a flow cytometric test to quantitate low levels of circulating myeloma plasma cells, and to determine the relationship of these cells with disease stage. Cells were characterized using five-parameter flow cytometric analysis with a panel of antibodies, and results were evaluated by comparison with fluorescent consensus-primer IgH-PCR. Bone marrow myeloma plasma cells, defined by high CD38 and Syndecan-1 expression, did not express CD10, 23, 30, 34 or 45RO, and demonstrated weak expression of CD37 and CD45. 65% of patients had CD19- 56+ plasma cells, 30% CD19- 56(low), and 5% CD19+ 56+, and these two antigens discriminated myeloma from normal plasma cells, which were all CD19+ 56(low). Peripheral blood myeloma plasma cells had the same composite phenotype, but expressed significantly lower levels of CD56 and Syndecan-1, and were detected in 75% (38/51) of patients at presentation, 92% (11/12) of patients in relapse, and 40% (4/10) of stem cell harvests. Circulating plasma cells were not detectable in patients in CR (n = 9) or normals (n = 10), at a sensitivity of up to 1 in 10,000 cells. There was good correlation between the flow cytometric test and IgH-PCR results: myeloma plasma cells were detectable by flow cytometry in all PCR positive samples, and samples with no detectable myeloma plasma cells were PCR negative. Absolute numbers decreased in patients responding to treatment, remained elevated in patients with refractory disease, and increased in patients undergoing relapse. We conclude that flow cytometry can provide an effective aternative to IgH-PCR that will allow quantitative assessment of low levels of residual disease.

  2. Increased type 1 chemokine expression in experimental Chagas disease correlates with cardiac pathology in beagle dogs.

    PubMed

    Guedes, Paulo M M; Veloso, Vanja M; Talvani, André; Diniz, Livia F; Caldas, Ivo S; Do-Valle-Matta, Maria A; Santiago-Silva, Juliana; Chiari, Egler; Galvão, Lucia M C; Silva, João S; Bahia, Maria T

    2010-11-15

    Chemokines and chemokine receptors interaction have presented important role in leukocyte migration to specific immune reaction sites. Recently, it has been reported that chemokine receptors CXC (CXCR3) and CC (CCR5) were preferentially expressed on Th1 cells while CCR3 and CCR4 were preferentially expressed on Th2 cells. This study evaluated the mRNA expression of type 1 and type 2 chemokine and chemokine receptors in the cardiac tissue of Beagle dogs infected with distinct genetic groups of Trypanosoma cruzi (Y, Berenice-78 and ABC strains) during acute and chronic phases. To analyze the correlation between chemokine and chemokine receptors expression and the development of heart pathology, the chronic infected animals were divided into groups, according to the parasite strain and based on the degree of heart damage: cardiac and indeterminate form of Chagas disease. Our results indicated that cardiac type1/2 chemokines and their receptors were partially dependent on the genetic diversity of parasites as well as the polarization of clinical forms. Also, dogs presenting cardiac form showed lower heart tissue mRNA expression of CCL24 (type 2) and higher expression of CCL5, CCL4 and CXCR3 (type 1) when compared with those with indeterminate form of disease. Together, these data reinforce a close-relation between T. cruzi genetic population and the host specific type 1 immune response and, for the first time, we show the distribution of type 1/2 chemokines associated with the development of cardiac pathology using dogs, a well similar model to study human Chagas disease.

  3. Experiential and Doctrinal Religious Knowledge Categorization in Parkinson's Disease: Behavioral and Brain Correlates

    PubMed Central

    Modestino, Edward J.; O'Toole, Partrick; Reinhofer, AnnaMarie

    2016-01-01

    Recent studies suggest changes in religious cognition in a subgroup of patients with Parkinson's disease (PD e.g., Butler et al., 2011). It is unclear whether this deficit extends to both doctrinal and experiential categorization forms of religious cognition. Kapogiannis et al. (2009b) dissociated experiential and doctrinal religious knowledge to different neural networks using fMRI. We examined Kapogiannis' dissociation against the background of PD side of onset (LOPD, ROPD), assessing performance both On- and Off-medication. In the behavioral portion of the study, we used a statement classification task in combination with scholar derived test sets for experiential and doctrinal religious knowledge categorization in conjunction with neuropsychological measures. In the neuroimaging portion of the study, we expanded on Kapogiannis' study by examining the same networks in PD. The behavioral data revealed that all groups rated (categorized) the scholar derived tests of experiential and doctrinal significantly differently than the scholars. All groups, including the scholars, classified more phrases as doctrinal than experiential. Religious cognition differed in the PD groups: those with PD Off-medication and LOPD Off-medication comprehended scholar defined experiential phrases with more difficulty, making them more likely to be classified as mixed or doctrinal. This was in contrast to the subjective frequency of classification of phrases as experiential paired with a cognitive decline in PD Off-medication; whereas PD On-medication showed a positive correlation with cognitive state and subjective doctrinal classification. For ROPD, cognitive state was associated with subjective experiential and doctrinal frequency of classification. With more intact intellect, there was a greater likelihood of classifying phrases subjectively as mixed, and the converse for experiential. Furthermore, religiosity negatively predicted subjective doctrinal frequency in LOPD, with the

  4. Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

    PubMed Central

    Elias, Darcielle Bruna Dias; Rocha, Lilianne Brito da Silva; Cavalcante, Maritza Barbosa; Pedrosa, Alano Martins; Justino, Izabel Cristina Bandeira; Gonçalves, Romélia Pinheiro

    2012-01-01

    Background Sickle cell disease is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and recurrent vaso-occlusive crises that reduces the quality of life of sufferers. Objective To evaluate the correlation of the levels of lactate dehydrogenase, malonaldehyde and nitrite to fetal hemoglobin in patients with sickle cell disease not under treatment with hydroxyurea in outpatients at a university hospital in Fortaleza, Ceará, Brazil. Methods Forty-four patients diagnosed with sickle cell disease were enrolled at baseline. Diagnosis was confirmed by evaluating the beta globin gene using polymerase chain reaction-restriction fragment length polymorphism. The concentration of fetal hemoglobin was obtained by high-performance liquid chromatography. Serum levels of nitrite, malonaldehyde and lactate dehydrogenase were measured by biochemical methods. Results Significantly higher levels of lactate dehydrogenase, nitrite and malonaldehyde were observed in patients with sickle cell disease compared to a control group. The study of the correlation between fetal hemoglobin levels and these variables showed a negative correlation with nitrite levels. No correlation was found between fetal hemoglobin and malonaldehyde or lactate dehydrogenase. When the study population was stratified according to fetal hemoglobin levels, a decrease in the levels of nitrite was observed with higher levels of fetal hemoglobin (p-value = 0.0415). Conclusion The results show that, similar to fetal hemoglobin levels, the concentration of nitrite can predict the clinical course of the disease, but should not be used alone as a modulator of prognosis in patients with sickle cell disease. PMID:23049438

  5. Toxoplasmosis – A Global Threat. Correlation of Latent Toxoplasmosis with Specific Disease Burden in a Set of 88 Countries

    PubMed Central

    Flegr, Jaroslav; Prandota, Joseph; Sovičková, Michaela; Israili, Zafar H.

    2014-01-01

    Background Toxoplasmosis is becoming a global health hazard as it infects 30–50% of the world human population. Clinically, the life-long presence of the parasite in tissues of a majority of infected individuals is usually considered asymptomatic. However, a number of studies show that this ‘asymptomatic infection’ may also lead to development of other human pathologies. Aims of the Study The purpose of the study was to collect available geoepidemiological data on seroprevalence of toxoplasmosis and search for its relationship with mortality and disability rates in different countries. Methods and Findings Prevalence data published between 1995–2008 for women in child-bearing age were collected for 88 countries (29 European). The association between prevalence of toxoplasmosis and specific disease burden estimated with age-standardized Disability Adjusted Life Year (DALY) or with mortality, was calculated using General Linear Method with Gross Domestic Product per capita (GDP), geolatitude and humidity as covariates, and also using nonparametric partial Kendall correlation test with GDP as a covariate. The prevalence of toxoplasmosis correlated with specific disease burden in particular countries explaining 23% of variability in disease burden in Europe. The analyses revealed that for example, DALY of 23 of 128 analyzed diseases and disease categories on the WHO list showed correlations (18 positive, 5 negative) with prevalence of toxoplasmosis and another 12 diseases showed positive trends (p<0.1). For several obtained significant correlations between the seroprevalence of toxoplasmosis and specific diseases/clinical entities, possible pathophysiological, biochemical and molecular explanations are presented. Conclusions The seroprevalence of toxoplasmosis correlated with various disease burden. Statistical associations does not necessarily mean causality. The precautionary principle suggests however that possible role of toxoplasmosis as a triggering

  6. Decreased Circulating T Regulatory Cells in Egyptian Patients with Nonsegmental Vitiligo: Correlation with Disease Activity

    PubMed Central

    Hegab, Doaa Salah; Attia, Mohamed Attia Saad

    2015-01-01

    Background. Vitiligo is an acquired depigmentary skin disorder resulting from autoimmune destruction of melanocytes. Regulatory T cells (Tregs), specifically CD4+CD25+ and Forkhead box P3+ (FoxP3+) Tregs, acquired notable attention because of their role in a variety of autoimmune pathologies. Dysregulation of Tregs may be one of the factors that can break tolerance to melanocyte self-antigens and contribute to vitiligo pathogenesis. Methods. In order to sustain the role of Tregs in pathogenesis and disease activity of vitiligo, surface markers for CD4+CD25+ and FoxP3+ peripheral Tregs were evaluated by flow cytometry in 80 Egyptian patients with nonsegmental vitiligo in addition to 60 healthy control subjects and correlated with clinical findings. Results. Vitiligo patients had significantly decreased numbers of both peripheral CD4+CD25+ and FoxP3+ T cells compared to control subjects (11.49%  ± 8.58% of CD4+ T cells versus 21.20%  ± 3.08%, and 1.09%  ± 0.96% versus 1.44%  ± 0.24%, resp., P < 0.05 for both). Peripheral numbers of CD4+CD25+ and FoxP3+ Tregs correlated negatively with VIDA score. Conclusion. Treg depletion with impaired immune downregulatory function might play a key role in the autoimmune conditions beyond nonsegmental vitiligo particularly in active cases. Effective Treg cell-based immunotherapies might be a future hope for patients with progressive vitiligo. PMID:26788051

  7. B7-H1 Expression in Wilms Tumor: Correlation With Tumor Biology and Disease Recurrence

    PubMed Central

    Routh, Jonathan C.; Ashley, Richard A.; Sebo, Thomas J.; Lohse, Christine M.; Husmann, Douglas A.; Kramer, Stephen A.; Kwon, Eugene D.

    2009-01-01

    Purpose Despite tremendous gains in improving prognosis, 10% of patients with Wilms tumor will ultimately experience disease recurrence. The identification of novel prognostic markers and tumor associated targets for patients at risk could enable clinicians to treat recurrences more aggressively and, thus, optimize outcomes. We have previously shown that tumor expression of the T cell coregulatory ligand B7-H1 portends a poor prognosis for adults with renal cell carcinoma and represents a promising target to improve therapy. We hypothesize that this finding may be true for Wilms tumor. Materials and Methods We identified 81 patients with Wilms tumor treated at 1 institution between 1968 and 2004. Histopathological features, including Wilms tumor B7-H1 expression, were correlated with clinical observations and outcome. Results Tumor recurrences were noted in 22% of patients with Wilms tumor and 14% died. B7-H1 was expressed in 11 tumors (14%) and was more likely to occur in anaplastic Wilms tumor (p = 0.03). Tumor B7-H1 expression was associated with a 2.7-fold increased risk of recurrence, although this difference did not achieve statistical significance (p = 0.06). However, in favorable histology tumors B7-H1 expression was associated with a 3.7-fold increased risk of recurrence (p = 0.03). Conclusions B7-H1 is expressed by Wilms tumor, correlates with tumor biology and is associated with an increased risk of recurrence in patients with favorable histology tumors. B7-H1 may prove useful in identifying high risk patients who could benefit from more aggressive initial treatment regimens, and may represent a promising therapeutic target. Multi-institutional studies to elucidate the role of B7-H1 in the treatment of Wilms tumor are warranted. PMID:18355839

  8. Correlation between the different pH-metry scores in gastroesophageal reflux disease in children

    PubMed Central

    Lupu, Vasile Valeriu; Ignat, Ancuţa; Paduraru, Gabriela; Ciubara, Anamaria; Moscalu, Mihaela; Marginean, Cristina Oana; Burlea, Marin

    2016-01-01

    Abstract The 24-hour esophageal pH-metry is the most widely used method to diagnose the gastroesophageal reflux disease (GERD). The study compares the different scores obtained during the 24-hour esophageal pH-metry. A retrospective study over 5 years including 234 children (1 month and 18 years old) admitted in a pediatric gastroenterology regional center in Northeast Romania, with suspicion of GERD. They underwent 24- hour esophageal pH-metry, and the scores obtained (Boix-Ochoa, DeMeester, Johnson-DeMeester) were compared. Out of the 234 children, 172 (73.50%) had positive Boix-Ochoa score and 62 (26.50%) had normal Boix-Ochoa score (<11.99). Based on the DeMeester score, 149 children (63.68%) were positive and 85 (36.32%) were negative. The correlation of the Demeester score with the Boix-Ochoa score was very high (r = 0.978, P <  < 0.01, 95% confidence interval). Considering the Johnson-DeMeester score, 120 cases (51.28%) had GERD and 114 (48.72%) did not. The correlation of the Johnson-DeMeester score with the Boix-Ochoa score was still high (r = 0.94, P <  < 0.01, 95% 95% confidence interval). As considered until now, the Boix-Ochoa score is the most accurate score to be used in pediatrics for the diagnosis of GERD. The use of the different scores—Boix-Ochoa, DeMeester, Johnson-DeMeester—showed a high sensitivity and specificity of the pH-metry measurements applied to the study lot, but the last score has a higher risk of false-negative results. PMID:27367982

  9. Type 1 chemokine receptor expression in Chagas' disease correlates with morbidity in cardiac patients.

    PubMed

    Gomes, Juliana A S; Bahia-Oliveira, Lilian M G; Rocha, Manoel Otávio C; Busek, Solange C U; Teixeira, Mauro M; Silva, João Santana; Correa-Oliveira, Rodrigo

    2005-12-01

    Chemokines and chemokine receptors (CKRs) control the migration of leukocytes during the inflammatory process and are important immunological markers of type 1 (CCR5 and CXCR3) and type 2 (CCR3 and CCR4) responses. The coexpression of CKRs (CCR2, CCR3, CCR5, CXCR3, and CXCR4) and intracellular cytokines (interleukin-10 [IL-10], IL-4, tumor necrosis factor alpha [TNF-alpha], and gamma interferon [IFN-gamma]) on T CD4+ and CD8+ peripheral cells from individuals with indeterminate (IND) or cardiac (CARD) clinical forms of Chagas' disease after in vitro stimulation with Trypanosoma cruzi antigens, were evaluated in this study. The percentage of T CD4+ and CD8+ cells coexpressing CCR5 and IFN-gamma, CXCR3 and IFN-gamma, and CXCR3 and TNF-alpha were higher in CARD than in IND individuals; on the other hand, the percentage of T CD4+ or CD8+ cells coexpressing CCR3 and IL-10 or coexpressing CCR3 and IL-4 were lower in CARD individuals than in IND individuals. In addition, a significant positive correlation between the expression of CCR5 or CXCR3 and IFN-gamma was observed in CARD individuals contrasting with a significant positive correlation between the expression of CCR3 and IL-4 and of CCR3 and IL-10 in IND patients. These results reinforce the hypothesis that a T. cruzi-exacerbated specific type 1 immune response developed by CARD chagasic patients is associated with the development of heart pathology.

  10. Decreased Circulating T Regulatory Cells in Egyptian Patients with Nonsegmental Vitiligo: Correlation with Disease Activity.

    PubMed

    Hegab, Doaa Salah; Attia, Mohamed Attia Saad

    2015-01-01

    Background. Vitiligo is an acquired depigmentary skin disorder resulting from autoimmune destruction of melanocytes. Regulatory T cells (Tregs), specifically CD4(+)CD25(+) and Forkhead box P3(+) (FoxP3(+)) Tregs, acquired notable attention because of their role in a variety of autoimmune pathologies. Dysregulation of Tregs may be one of the factors that can break tolerance to melanocyte self-antigens and contribute to vitiligo pathogenesis. Methods. In order to sustain the role of Tregs in pathogenesis and disease activity of vitiligo, surface markers for CD4(+)CD25(+) and FoxP3(+) peripheral Tregs were evaluated by flow cytometry in 80 Egyptian patients with nonsegmental vitiligo in addition to 60 healthy control subjects and correlated with clinical findings. Results. Vitiligo patients had significantly decreased numbers of both peripheral CD4(+)CD25(+) and FoxP3(+) T cells compared to control subjects (11.49%  ± 8.58% of CD4(+) T cells versus 21.20%  ± 3.08%, and 1.09%  ± 0.96% versus 1.44%  ± 0.24%, resp., P < 0.05 for both). Peripheral numbers of CD4(+)CD25(+) and FoxP3(+) Tregs correlated negatively with VIDA score. Conclusion. Treg depletion with impaired immune downregulatory function might play a key role in the autoimmune conditions beyond nonsegmental vitiligo particularly in active cases. Effective Treg cell-based immunotherapies might be a future hope for patients with progressive vitiligo. PMID:26788051

  11. Correlation of Levels of Oncostatin M Cytokine in Crevicular Fluid and Serum in Periodontal Disease

    PubMed Central

    Thorat, ManojKumar; AR, Pradeep; Garg, Garima

    2010-01-01

    Aim The aim of this study was to measure the level of Oncostatin M (OSM) a gp130 cytokine in the gingival crevicular fluid (GCF) and serum of chronic periodontitis patients and to find any correlation between them before and after periodontal therapy (scaling and root planing, SRP). Methodology 60 subjects (age 25–50 years) were enrolled into three groups (n=20 per group), group I (healthy), group II (gingivitis) and group III (chronic periodontitis). Group III subjects were followed for 6–8 weeks after the initial periodontal therapy (SRP) as the group IV (after periodontal therapy). Clinical parameters were assessed as gingival index (GI), probing depth (PD), clinical attachment level (CAL), and radiographic evidence of bone loss. GCF and serum levels of OSM were measured by using Enzyme Linked Immunosorbent Assay (ELISA). Results It was found that mean OSM levels had been elevated in both the GCF and serum of chronic periodontitis subjects (726.65 ± 283.56 and 65.59 ± 12.37 pg·mL−1, respectively) and these levels were decreased proportionally after the periodontal therapy (95.50 ± 38.85 and 39.98 ± 16.69 pg·mL−1 respectively). However, OSM was detected in GCF of healthy subjects (66.15 ± 28.10 pg·mL−1) and gingivitis-suffering subjects (128.33 ± 22.96 pg·mL−1) and was found as below the detectable limit (≈0.0 pg·mL−1) in the serum of same subjects. Significant correlation has been found between clinical parameters and GCF-serum levels of OSM. Conclusion Increased OSM level both in the GCF and serum, and the decreased levels after initial periodontal therapy (SRP) may suggest a use as an inflammatory bio-marker in the periodontal disease. PMID:21404969

  12. Correlation Between Bladder Pain Syndrome/Interstitial Cystitis and Pelvic Inflammatory Disease

    PubMed Central

    Chung, Shiu-Dong; Chang, Chao-Hsiang; Hung, Peir-Haur; Chung, Chi-Jung; Muo, Chih-Hsin; Huang, Chao-Yuan

    2015-01-01

    Abstract Pelvic inflammatory disease (PID) has been investigated in Western countries and identified to be associated with chronic pelvic pain and inflammation. Bladder pain syndrome/interstitial cystitis (BPS/IC) is a complex syndrome that is significantly more prevalent in women than in men. Chronic pelvic pain is a main symptom of BPS/IC, and chronic inflammation is a major etiology of BPS/IC. This study aimed to investigate the correlation between BPS/IC and PID using a population-based dataset. We constructed a case–control study from the Taiwan National Health Insurance program. The case cohort comprised 449 patients with BPS/IC, and 1796 randomly selected subjects (about 1:4 matching) were used as controls. A Multivariate logistic regression model was constructed to estimate the association between BPS/IC and PID. Of the 2245 sampled subjects, a significant difference was observed in the prevalence of PID between BPS/IC cases and controls (41.7% vs 15.4%, P < 0.001). Multivariate logistic regression analysis revealed that the odds ratio (OR) for PID among cases was 3.69 (95% confidence interval [CI]: 2.89–4.71). Furthermore, the ORs for PID among BPS/IC cases were 4.52 (95% CI: 2.55–8.01), 4.31 (95% CI: 2.91–6.38), 3.00 (95% CI: 1.82–4.94), and 5.35 (95% CI: 1.88–15.20) in the <35, 35–49, 50–64, and >65 years age groups, respectively, after adjusting for geographic region, irritable bowel syndrome, and hypertension. Joint effect was also noted, specifically when patients had both PID and irritable bowel disease with OR of 10.5 (95% CI: 4.88–22.50). This study demonstrated a correlation between PID and BPS/IC. Clinicians treating women with PID should be alert to BPS/IC-related symptoms in the population. PMID:26579800

  13. Domains and correlates of clinical balance impairment associated with Huntington's disease.

    PubMed

    Jacobs, Jesse V; Boyd, James T; Hogarth, Penelope; Horak, Fay B

    2015-03-01

    This study sought to (a) determine the domains of clinical balance impairments associated with Huntington's disease (HD), and (b) evaluate associations between balance test scores and other disease-related impairments. Eighteen subjects with genetically definite HD and 17 age-matched control subjects were evaluated on the Mini-BESTest for their clinical balance impairments as well as the Unified HD Rating Scale (UHDRS) motor and total functional capacity scales, Activity-Specific Balance Confidence (ABC) Scale-short form, Montreal Cognitive Assessment (MoCA), and Symbol Digit Modalities Test (SDMT). Results showed that subjects with HD exhibited significantly lower total Mini-BESTest scores than subjects without HD (mean (95% CI)=76 (64-87)% with HD, 98 (96-99)% without HD; p=0.0011). Mini-BESTest item scores were significantly lower for subjects with HD on one-leg stance, postural responses, standing with eyes closed on foam, and dual-task timed up-and-go. Mini-BESTest scores significantly correlated with UHDRS motor (r(2)=0.68; p=0.00003) and total functional capacity (r(2)=0.75; p=0.000006) scores as well as with scores on the ABC short form (r(2)=0.45; p=0.0024), SDMT (r(2)=0.42; p=0.0036), and MoCA (r(2)=0.23; p=0.046) assessments. This study, therefore, demonstrates that balance impairments associated with HD span domains of anticipatory postural adjustments, postural responses, stance in challenging sensory conditions, and gait. Although preliminary, clinical balance impairment appears to be an efficient proxy evaluation of multiple HD-related factors due to associations with functional capacity, other motor impairments, balance confidence, and cognitive abilities. PMID:25797790

  14. Bleeding symptoms and laboratory correlation in patients with severe von Willebrand disease.

    PubMed

    Metjian, A D; Wang, C; Sood, S L; Cuker, A; Peterson, S M; Soucie, J M; Konkle, B A

    2009-07-01

    Type 3 von Willebrand disease (VWD) is a rare bleeding disorder with markedly decreased or absent von Willebrand factor (VWF) protein, accompanied by a parallel decrease in VWF function and factor VIII (FVIII) activity. The goal of this study was to describe the population of patients enrolled in the USA Centers for Disease Control Universal Data Collection (UDC) study with type 3 VWD, defined as a VWF:Ag of <10%, and to correlate bleeding symptoms with VWF and FVIII levels. Data on 150 patients were analysed. Almost all patients experienced bleeding episodes (98%) and required blood and/or factor product treatment (92%). While oral mucosal bleeding (the site of first bleed in 54%) was most common, subsequent muscle and joint bleeds were also seen (28%, 45%, respectively), and intracranial haemorrhage occurred in 8% of individuals. Mean age of first bleed was lower in those with either a FVIII < or =5% or a VWF:Ag <1%. Univariate marginal model analysis showed lower levels of FVIII and VWF:Ag both predicted a higher risk of joint bleeding. Longitudinal multivariate analysis found a lower FVIII level (P = 0.03), increasing age (P < 0.0001), history of joint bleeding (P = 0.001), higher body mass index (BMI) (P < 0.0001), and use of home infusion (P = 0.02) were all negatively associated with joint mobility. Low levels of VWF:Ag (P = 0.003) and male sex (P = 0.007) were also negatively associated with joint function. This study documents the strong bleeding phenotype in severe VWD and provides data to help target therapy, including prophylaxis, for patients most at risk of bleeding complications.

  15. Functional correlates of optic flow motion processing in Parkinson’s disease

    PubMed Central

    Putcha, Deepti; Ross, Robert S.; Rosen, Maya L.; Norton, Daniel J.; Cronin-Golomb, Alice; Somers, David C.; Stern, Chantal E.

    2014-01-01

    The visual input created by the relative motion between an individual and the environment, also called optic flow, influences the sense of self-motion, postural orientation, veering of gait, and visuospatial cognition. An optic flow network comprising visual motion areas V6, V3A, and MT+, as well as visuo-vestibular areas including posterior insula vestibular cortex (PIVC) and cingulate sulcus visual area (CSv), has been described as uniquely selective for parsing egomotion depth cues in humans. Individuals with Parkinson’s disease (PD) have known behavioral deficits in optic flow perception and visuospatial cognition compared to age- and education-matched control adults (MC). The present study used functional magnetic resonance imaging (fMRI) to investigate neural correlates related to impaired optic flow perception in PD. We conducted fMRI on 40 non-demented participants (23 PD and 17 MC) during passive viewing of simulated optic flow motion and random motion. We hypothesized that compared to the MC group, PD participants would show abnormal neural activity in regions comprising this optic flow network. MC participants showed robust activation across all regions in the optic flow network, consistent with studies in young adults, suggesting intact optic flow perception at the neural level in healthy aging. PD participants showed diminished activity compared to MC particularly within visual motion area MT+ and the visuo-vestibular region CSv. Further, activation in visuo-vestibular region CSv was associated with disease severity. These findings suggest that behavioral reports of impaired optic flow perception and visuospatial performance may be a result of impaired neural processing within visual motion and visuo-vestibular regions in PD. PMID:25071484

  16. Mediastinal germ cell tumors: a radiologic-pathologic review.

    PubMed

    Drevelegas, A; Palladas, P; Scordalaki, A

    2001-01-01

    Germ cell tumors of the mediastinum are histologically identical to those found in the testes and ovaries. Early diagnosis and treatment improve the survival rate. Imaging studies of teratoma demonstrate a rounded, often lobulated heterogeneous mass containing soft tissue elements with fluid and fat attenuation. Calcification is present in 20-43% of cases. Seminomas are large masses of homogeneous soft tissue attenuation. Malignant nonseminomatous germ cell tumors are heterogeneous tumors with irregular borders due to invasion of adjacent structures. CT shows the location and extent of the tumors as well as intrinsic elements including soft tissue, fat, fluid, and calcification. CT is the modality of choice for the diagnostic evaluation of these tumors. MRI reveals masses of heterogeneous signal intensity, is more sensitive in depicting infiltration of the adjacent structures by fat plane obliteration, and is performed as an ancillary study.

  17. Circulating tumor cells are correlated with disease progression and treatment response in an orthotopic hepatocellular carcinoma model.

    PubMed

    Yan, Jun; Fan, Zhichao; Wu, Xiufeng; Xu, Min; Jiang, Jiahao; Tan, Changjun; Wu, Weizhong; Wei, Xunbin; Zhou, Jian

    2015-11-01

    Hepatocellular carcinoma (HCC) is a highly malignant tumor characterized by rapid progression, poor prognosis, and frequent hematogenous metastasis. A minimally invasive diagnostic biomarker that can predict disease progression and treatment response would be of extraordinary benefit. Therefore, we have investigated whether the number of circulating tumor cells (CTCs) is correlated with disease progression and treatment response in HCC. Here we report that the number of CTCs, monitored by in vivo flow cytometry (IVFC), is strongly correlated with disease progression and treatment response in a highly metastatic orthotopic nude mouse model of green fluorescent protein (GFP)-labeled HCC. Sorafenib treatment reduces the number of CTCs significantly. The decreased number of CTCs is consistent with low lung metastasis. This study has demonstrated a considerable clinical value of CTCs as a biomarker in predicting disease progression and monitoring therapeutic efficacy in patients with HCC.

  18. Asymmetries of amyloid-β burden and neuronal dysfunction are positively correlated in Alzheimer's disease.

    PubMed

    Frings, Lars; Hellwig, Sabine; Spehl, Timo S; Bormann, Tobias; Buchert, Ralph; Vach, Werner; Minkova, Lora; Heimbach, Bernhard; Klöppel, Stefan; Meyer, Philipp T

    2015-10-01

    Clinical Alzheimer's disease affects both cerebral hemispheres to a similar degree in clinically typical cases. However, in atypical variants like logopenic progressive aphasia, neurodegeneration often presents asymmetrically. Yet, no in vivo imaging study has investigated whether lateralized neurodegeneration corresponds to lateralized amyloid-β burden. Therefore, using combined (11)C-Pittsburgh compound B and (18)F-fluorodeoxyglucose positron emission tomography, we explored whether asymmetric amyloid-β deposition in Alzheimer's disease is associated with asymmetric hypometabolism and clinical symptoms. From our database of patients who underwent positron emission tomography with both (11)C-Pittsburgh compound B and (18)F-fluorodeoxyglucose (n = 132), we included all amyloid-positive patients with prodromal or mild-to-moderate Alzheimer's disease (n = 69). The relationship between (11)C-Pittsburgh compound B binding potential and (18)F-fluorodeoxyglucose uptake was assessed in atlas-based regions of interest covering the entire cerebral cortex. Lateralizations of amyloid-β and hypometabolism were tested for associations with each other and with type and severity of cognitive symptoms. Positive correlations between asymmetries of Pittsburgh compound B binding potential and hypometabolism were detected in 6 of 25 regions (angular gyrus, middle frontal gyrus, middle occipital gyrus, superior parietal gyrus, inferior and middle temporal gyrus), i.e. hypometabolism was more pronounced on the side of greater amyloid-β deposition (range: r = 0.41 to 0.53, all P < 0.001). Stronger leftward asymmetry of amyloid-β deposition was associated with more severe language impairment (P < 0.05), and stronger rightward asymmetry with more severe visuospatial impairment (at trend level, P = 0.073). Similarly, patients with predominance of language deficits showed more left-lateralized amyloid-β burden and hypometabolism than patients with predominant visuospatial impairment

  19. Supervised multi-view canonical correlation analysis: fused multimodal prediction of disease diagnosis and prognosis

    NASA Astrophysics Data System (ADS)

    Singanamalli, Asha; Wang, Haibo; Lee, George; Shih, Natalie; Rosen, Mark; Master, Stephen; Tomaszewski, John; Feldman, Michael; Madabhushi, Anant

    2014-03-01

    While the plethora of information from multiple imaging and non-imaging data streams presents an opportunity for discovery of fused multimodal, multiscale biomarkers, they also introduce multiple independent sources of noise that hinder their collective utility. The goal of this work is to create fused predictors of disease diagnosis and prognosis by combining multiple data streams, which we hypothesize will provide improved performance as compared to predictors from individual data streams. To achieve this goal, we introduce supervised multiview canonical correlation analysis (sMVCCA), a novel data fusion method that attempts to find a common representation for multiscale, multimodal data where class separation is maximized while noise is minimized. In doing so, sMVCCA assumes that the different sources of information are complementary and thereby act synergistically when combined. Although this method can be applied to any number of modalities and to any disease domain, we demonstrate its utility using three datasets. We fuse (i) 1.5 Tesla (T) magnetic resonance imaging (MRI) features with cerbrospinal fluid (CSF) proteomic measurements for early diagnosis of Alzheimer's disease (n = 30), (ii) 3T Dynamic Contrast Enhanced (DCE) MRI and T2w MRI for in vivo prediction of prostate cancer grade on a per slice basis (n = 33) and (iii) quantitative histomorphometric features of glands and proteomic measurements from mass spectrometry for prediction of 5 year biochemical recurrence postradical prostatectomy (n = 40). Random Forest classifier applied to the sMVCCA fused subspace, as compared to that of MVCCA, PCA and LDA, yielded the highest classification AUC of 0.82 +/- 0.05, 0.76 +/- 0.01, 0.70 +/- 0.07, respectively for the aforementioned datasets. In addition, sMVCCA fused subspace provided 13.6%, 7.6% and 15.3% increase in AUC as compared with that of the best performing individual view in each of the three datasets, respectively. For the biochemical recurrence

  20. Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system

    PubMed Central

    Baehring, J; Henchcliffe, C; Ledezma, C; Fulbright, R; Hochberg, F

    2005-01-01

    Background: Intravascular lymphoma (IVL) is a rare non-Hodgkin's lymphoma with relative predilection for the central nervous system. In the absence of extraneural manifestations, the disease is not recognised until autopsy in the majority of cases underlining the need for new clinical markers. Methods: This is a retrospective series of five patients with IVL seen at a single institution over three years. An advanced magnetic resonance imaging (MRI) protocol was performed at various time points prior to diagnosis and during treatment. Results: MRI revealed multiple lesions scattered throughout the cerebral hemispheres; the brainstem, cerebellum, and spinal cord were less frequently involved. On initial presentation, hyperintense lesions were seen on diffusion weighted images suggestive of ischaemia in three of four patients in whom the images were obtained at that time point. In four patients lesions were also identifiable as hyperintense areas on fluid attenuated inversion recovery (FLAIR) sequences. Initial contrast enhancement was encountered in three cases. Diffusion weighted imaging lesions either vanished or followed the typical pattern of an ischaemic small vessel stroke with evolution of abnormal FLAIR signal followed by enhancement with gadolinium in the subacute stage and tissue loss in the chronic stage. Diffusion weighted imaging and FLAIR abnormalities proved to be partially reversible, correlating with the response to chemotherapy. Conclusion: We provide the first detailed description of the dynamic pattern of diffusion weighted MRI in IVL. These patterns in combination with systemic findings may facilitate early diagnosis and serve as a new tool to monitor treatment response. PMID:15774442

  1. Pelvic Inflammatory Disease: Multimodality Imaging Approach with Clinical-Pathologic Correlation.

    PubMed

    Revzin, Margarita V; Mathur, Mahan; Dave, Haatal B; Macer, Matthew L; Spektor, Michael

    2016-01-01

    Pelvic inflammatory disease (PID) is a common medical problem, with almost 1 million cases diagnosed annually. Historically, PID has been a clinical diagnosis supplemented with the findings from ultrasonography (US) or magnetic resonance (MR) imaging. However, the diagnosis of PID can be challenging because the clinical manifestations may mimic those of other pelvic and abdominal processes. Given the nonspecific clinical manifestations, computed tomography (CT) is commonly the first imaging examination performed. General CT findings of early- and late-stage PID include thickening of the uterosacral ligaments, pelvic fat stranding with obscuration of fascial planes, reactive lymphadenopathy, and pelvic free fluid. Recognition of these findings, as well as those seen with cervicitis, endometritis, acute salpingitis, oophoritis, pyosalpinx, hydrosalpinx, tubo-ovarian abscess, and pyometra, is crucial in allowing prompt and accurate diagnosis. Late complications of PID include tubal damage resulting in infertility and ectopic pregnancy, peritonitis caused by uterine and/or tubo-ovarian abscess rupture, development of peritoneal adhesions resulting in bowel obstruction and/or hydroureteronephrosis, right upper abdominal inflammation (Fitz-Hugh-Curtis syndrome), and septic thrombophlebitis. Recognition of these late manifestations at CT can also aid in proper patient management. At CT, careful assessment of common PID mimics, such as endometriosis, adnexal torsion, ruptured hemorrhagic ovarian cyst, adnexal neoplasms, appendicitis, and diverticulitis, is important to avoid misinterpretation, delay in management, and unnecessary surgery. Correlation with the findings from complementary imaging examinations, such as US and MR imaging, is useful for establishing a definitive diagnosis. (©)RSNA, 2016. PMID:27618331

  2. Disease duration of episodic migraine correlates with modified amplitudes and habituation of contingent negative variation.

    PubMed

    Kropp, Peter; Wallasch, Thomas-Martin; Müller, Britta; Meyer, Bianca; Darabaneanu, Stephanie; Bosse, Christoph; Keller, Armin; Meyer, Wolfgang; Gerber, Wolf-Dieter

    2015-06-01

    Cortical habituation in episodic migraine patients without medication overuse headache (MOH), recorded by contingent negative variation (CNV), is often reduced compared with healthy controls. There is evidence that with longer duration of migraine disease (DOD) amplitudes and habituation of CNV become progressively abnormal. The aim of the study was to examine habituation characteristics of contingent negative variation in episodic migraine patients suffering from short- and long-lasting migraine compared to matched healthy controls. 32 migraine patients without aura and without MOH diagnosed according to the revised ICHD-II criteria and 16 age- and sex-matched healthy controls were included. According to DOD, the total sample of migraine patients was divided into two groups (group a: DOD <121 months, n = 17 subjects, group b: DOD >120 months, n = 15 subjects). Both migraine groups did not differ in the number of days of migraine and the duration of attacks. Overall CNV and initial CNV differed significantly between migraine patients and controls, whereas the former produced more negative amplitudes. In the migraine group lack of or deficient habituation occurred, whilst controls showed habituation. There were middle range correlations between the DOD and overall CNV, initial CNV, and y-intercept. Patients suffering from long-lasting migraine produced higher CNV amplitudes with a higher y-intercept. The results are interpreted as "maladaptive plasticity" with a risen intercept in long-lasting migraine. PMID:25432434

  3. Correlation between water hardness and cardiovascular diseases in Mostar city, Bosnia and Herzegovina.

    PubMed

    Knezović, Nevenka J; Memić, Mustafa; Mabić, Mirela; Huremović, Jasna; Mikulić, Ivanka

    2014-12-01

    The aim of this study was to determine the association of cardiovascular disease (CVD) of selected human subjects with the hardness of water they consume. Laboratory testing of physical and chemical parameters of water were made using standardized methods: Standard Methods 19th edn, 1995 (APHA, AWWA & WEF, Washington, DC), and ISO 7888:1985, ISO 10523:1998. Pearson's chi-squared test was used for the statistical analysis of data, with the significance level of 0.05. The obtained data were analysed using the statistical program SPSS 16.0. The study sample consisted of 1,021 individuals divided into two groups: soft water consumers, 603 individuals, and, hard water consumers, 618 individuals. Results indicate that a statistically significant (χ(2) = 5.315; df = 1; p = 0.021) number of individuals with CVD drink soft water. The prevalence of CVD in the age group 45-60 years in the study area where soft water is consumed was 21.3% and in the study area where hard water is consumed the prevalence of CVD was 13.7%. The summary results indicate significant correlation between the prevalence of CVD in the population group who drink soft water. The value of the relative risk is 1.127.

  4. Urinary CYP Eicosanoid Excretion Correlates with Glomerular Filtration in African-Americans with Chronic Kidney Disease

    PubMed Central

    Dreisbach, Albert W; Smith, Stanley V; Kyle, Patrick B; Ramaiah, Manjunath; Amenuke, Margaret; Garrett, Michael R; Lirette, Seth T; Griswold, Michael E; Roman, Richard J

    2015-01-01

    Previous studies have indicated that cytochrome P450 (CYP) metabolites of arachidonic acid (AA), i.e., 20-hydroxyeicosatetraenoic acid (20-HETE) and epoxyeicosatrienoic acids (EETs), play an important role in the regulation of renal tubular and vascular function. The present study for the first time profiled HETEs and epoxygenase derived dihydroxyeicosatetraenoic acid diHETEs levels in spot urines and plasma in 262 African American patients from the University of Mississippi Chronic Kidney Disease Clinic and 31 African American controls. Significant correlations in eGFR and urinary 20-HETE/creatinine and 19-HETE/ creatinine levels were observed. The eGFR increased by 17.47 [p=0.001] and 60.68 [(p=0.005] ml/min/ for each ng/mg increase in 20-HETE and 19-HETE levels, respectively. Similar significant positive associations were found between the other urinary eicosanoids and eGFR and also with 19-HETE/urine creatinine concentration and proteinuria. We found that approximately 80% of plasma HETEs and 30% diHETEs were glucuronidated and the fractional excretion of 20-HETE was less than 1%. These results suggest that there is a significant hepatic source of urinary 20-HETE glucuronide and EETs with extensive renal biotransformation to metabolites which may play a role in the pathogenesis of CKD. PMID:25151892

  5. Urinary CYP eicosanoid excretion correlates with glomerular filtration in African-Americans with chronic kidney disease.

    PubMed

    Dreisbach, Albert W; Smith, Stanley V; Kyle, Patrick B; Ramaiah, Manjunath; Amenuke, Margaret; Garrett, Michael R; Lirette, Seth T; Griswold, Michael E; Roman, Richard J

    2014-10-01

    Previous studies have indicated that cytochrome P450 (CYP) metabolites of arachidonic acid (AA), i.e., 20-hydroxyeicosatetraenoic acid (20-HETE) and epoxyeicosatrienoic acids (EETs), play an important role in the regulation of renal tubular and vascular function. The present study for the first time profiled HETEs and epoxygenase derived dihydroxyeicosatetraenoic acid diHETEs levels in spot urines and plasma in 262 African American patients from the University of Mississippi Chronic Kidney Disease Clinic and 31 African American controls. Significant correlations in eGFR and urinary 20-HETE/creatinine and 19-HETE/creatinine levels were observed. The eGFR increased by 17.47 [p=0.001] and 60.68 [(p=0.005]ml/min/for each ng/mg increase in 20-HETE and 19-HETE levels, respectively. Similar significant positive associations were found between the other urinary eicosanoids and eGFR and also with 19-HETE/urine creatinine concentration and proteinuria. We found that approximately 80% of plasma HETEs and 30% diHETEs were glucuronidated and the fractional excretion of 20-HETE was less than 1%. These results suggest that there is a significant hepatic source of urinary 20-HETE glucuronide and EETs with extensive renal biotransformation to metabolites which may play a role in the pathogenesis of CKD.

  6. Wilson disease: Histopathological correlations with treatment on follow-up liver biopsies

    PubMed Central

    Cope-Yokoyama, Sandy; Finegold, Milton J; Sturniolo, Giacomo Carlo; Kim, Kyoungmi; Mescoli, Claudia; Rugge, Massimo; Medici, Valentina

    2010-01-01

    AIM: To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease (WD) patients. METHODS: We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent multiple follow-up liver biopsies. Demographic, clinical and laboratory data were gathered and all patients underwent an initial biopsy and at least one repeat biopsy. RESULTS: Time to repeat biopsy ranged from 2 to 12 years. Six patients (non-progressors) showed stable hepatic histology or improvement. In one case, we observed improvement of fibrosis from stage 2 to 0. Six patients (progressors) had worsening of fibrosis. There was no significant correlation between the histological findings and serum aminotransferases or copper metabolism parameters. The hepatic copper concentration reached normal levels in only two patients: one from the non-progressors and one from the progressors group. The estimated rate of progression of hepatic fibrosis in the entire group was 0 units per year in the time frame between the first and the second liver biopsy (4 years), and 0.25 between the second and the third (3 years). In the progressors group, the rate of progression of liver fibrosis was estimated at 0.11 fibrosis units per year between the first and second biopsy and, 0.6 fibrosis units between the second and third biopsy. CONCLUSION: The inability of clinical tools to detect fibrosis progression in WD suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered. PMID:20333789

  7. Do executive dysfunction and freezing of gait in Parkinson's disease share the same neuroanatomical correlates?

    PubMed

    Brugger, Florian; Abela, Eugenio; Hägele-Link, Stefan; Bohlhalter, Stephan; Galovic, Marian; Kägi, Georg

    2015-09-15

    Current hypotheses postulate a relationship between executive dysfunction and freezing of gait (FOG) in Parkinson's disease (PD). Hitherto, most evidence comes from entirely clinical approaches, while knowledge about this relationship on the morphological level is sparse. The aim of this study was therefore to assess the overlap of gray matter atrophy associated with FOG and executive dysfunction in PD. We included 18 PD patients with FOG and 20 without FOG in our analysis. A voxel-based morphometry approach was used to reveal voxel clusters in the gray matter which were associated with FOG and executive dysfunction as measured by the Frontal Assessment Battery, respectively. Conjunction analysis was applied to detect overlaps of the associated patterns. FOG correlated with different cortical clusters in the frontal and parietal lobes, whereas those associated with the FAB scores were, although widespread, widely confined to the frontal lobe. Conjunction analysis revealed a significant cluster of gray matter loss in the right dorsolateral prefrontal cortex. We could show that the patterns of neurodegeneration associated with FOG and executive dysfunction (as measured by the FAB) share atrophic changes in the same cortical areas. However, there is also a considerable number of cortical areas where neurodegenerative changes are only unique for either sign. Particularly, the involvement of parietal lobe areas seems to be more specific for FOG.

  8. Optimal control problem in correlation between smoking and epidemic of respiratory diseases

    NASA Astrophysics Data System (ADS)

    Aldila, D.; Apri, M.

    2014-02-01

    Smoking appears to be a risk factor that may increase the number of different pulmonary infections. This link is likely to be mediated by smoking adverse effects on the respiratory defenses. A mathematical model to describe correlation between the number of smokers and its effect on the number of infected people suffer from respiratory disease like influenza is constructed in this paper. Promotion of healthy life is accounted in the model as an optimal control problem to reduce the number of smokers. In this work, the transition rates from smokers to non-smokers and from non-smokers to smokers are regarded as the control variables. Assuming the control variables are constant, equilibrium points of the model can be obtained analytically. The basic reproductive ratio as the endemic threshold is taken from the spectral radius of the next-generation matrix. Using numerical simulation, we show that the healthy life promotion can reduce the number of infected person significantly by reducing the number of smokers. Furthermore, different initial conditions to show different situations in the field are also simulated. It is shown that a large effort to increase the transition rate from smokers to non-smokers and to reduce the transition from non-smokers to smokers should be applied in the endemic reduction scenario.

  9. Growth Patterns of Clostridium difficile – Correlations with Strains, Binary Toxin and Disease Severity: A Prospective Cohort Study

    PubMed Central

    Tschudin-Sutter, Sarah; Braissant, Olivier; Erb, Stefan; Stranden, Anne; Bonkat, Gernot; Frei, Reno; Widmer, Andreas F.

    2016-01-01

    A broad spectrum of symptoms has been associated with C. difficile infection (CDI). Several studies indicate that toxin-production correlates with growth rates of C. difficile. This study aimed to correlate growth rates of C. difficile with disease severity and strain characteristics. From 01/2003 to 10/2011, strains from a prospective cohort of all inpatients with CDI at the University Hospital Basel, Switzerland were analyzed regarding binary toxin, presence of the tcdC deletion and ribotype. Isothermal microcalorimetry was performed to determine growth rates, quantified by the Gompertz function. Ordered logistic regression models were used to correlate disease severity with strain features and clinical characteristics. Among 199 patients, 31 (16%) were infected with binary toxin-producing strains, of which the tcdC gene-deletion nt117 was detected in 9 (4%). Disease severity was classified as mild in 130 patients (65.3%), as severe in 59 patients (29.7%) and as severe/complicated in 10 patients (5.0%). Growth rates were inversely associated with disease severity in univariable (OR 0.514, 95%CI 0.29–0.91, p = 0.023) and multivariable analyses (OR 0.51, 95%CI 0.26–0.97, p = 0.040). While none of the strain characteristics such as presence of the tcdC gene deletion or binary toxin predicted CDI severity, growth rates were inversely correlated with disease severity. Further investigations are needed to analyze growth-regulators and respective correlations with the level of toxin production in C. difficile, which may be important determinants of disease severity. PMID:27598309

  10. Growth Patterns of Clostridium difficile - Correlations with Strains, Binary Toxin and Disease Severity: A Prospective Cohort Study.

    PubMed

    Tschudin-Sutter, Sarah; Braissant, Olivier; Erb, Stefan; Stranden, Anne; Bonkat, Gernot; Frei, Reno; Widmer, Andreas F

    2016-01-01

    A broad spectrum of symptoms has been associated with C. difficile infection (CDI). Several studies indicate that toxin-production correlates with growth rates of C. difficile. This study aimed to correlate growth rates of C. difficile with disease severity and strain characteristics. From 01/2003 to 10/2011, strains from a prospective cohort of all inpatients with CDI at the University Hospital Basel, Switzerland were analyzed regarding binary toxin, presence of the tcdC deletion and ribotype. Isothermal microcalorimetry was performed to determine growth rates, quantified by the Gompertz function. Ordered logistic regression models were used to correlate disease severity with strain features and clinical characteristics. Among 199 patients, 31 (16%) were infected with binary toxin-producing strains, of which the tcdC gene-deletion nt117 was detected in 9 (4%). Disease severity was classified as mild in 130 patients (65.3%), as severe in 59 patients (29.7%) and as severe/complicated in 10 patients (5.0%). Growth rates were inversely associated with disease severity in univariable (OR 0.514, 95%CI 0.29-0.91, p = 0.023) and multivariable analyses (OR 0.51, 95%CI 0.26-0.97, p = 0.040). While none of the strain characteristics such as presence of the tcdC gene deletion or binary toxin predicted CDI severity, growth rates were inversely correlated with disease severity. Further investigations are needed to analyze growth-regulators and respective correlations with the level of toxin production in C. difficile, which may be important determinants of disease severity. PMID:27598309

  11. Increased expression of low density granulocytes in juvenile-onset systemic lupus erythematosus patients correlates with disease activity.

    PubMed

    Midgley, A; Beresford, M W

    2016-04-01

    Neutrophils are implicated in a wide range of non-infectious inflammatory conditions. A subset of neutrophils in the peripheral circulation of systemic lupus erythematosus (SLE) patients has been described and termed low density granulocytes (LDGs). This study investigates the expression of LDG in juvenile-onset SLE (JSLE) patients compared to controls, and any correlations with disease activity.Neutrophils and LDGs were isolated from JSLE (n = 13) and paediatric non-inflammatory control patients (n = 12). Cell populations were assessed and compared using flow cytometry and morphological analysis. Standard clinical data, which included disease activity markers/scores, were collected for each patient.Significantly increased LDG expression (%mean ± SEM, range) was observed in JSLE patients (10.4 ± 3.26, 3.41-36.3) compared to controls (2.4 ± 0.44, 0.36-5.27; p = 0.005). A statistically significant positive correlation was observed between LDG expression and the British Isles Lupus Activity Group (correlation coefficient 0.685; p = 0.010) and SLE Disease Activity Index (correlation coefficient 0.567; p = 0.043) and the biomarker of dsDNA-antibodies (correlation coefficient 0.590; p = 0.043).Here we observe increased expression in LDGs in JSLE patients, which correlate with dsDNA antibody concentration and scores of disease activity. These correlations indicate that the increased LDG expression observed in this study may have a potential role in the pathogenesis of JSLE, and may be a useful biomarker.

  12. Rainbow trout (Oncorhynchus mykiss) resistance to columnaris disease is heritable and favorably correlated with bacterial cold water disease resistance

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Columnaris disease (CD), caused by Flabobacterium columnare, is an emerging disease affecting rainbow trout aquaculture. Objectives of this study were to 1) estimate heritability of innate CD resistance in a rainbow trout line (ARS-Fp-R) previously selected four generations for improved bacterial co...

  13. Tests of dorsolateral frontal function correlate with objective tests of postural stability in early to moderate stage Parkinson's disease.

    PubMed

    Nocera, Joe R; Price, Catherine; Fernandez, Hubert H; Amano, Shinichi; Vallabhajosula, Srikant; Okun, Michael S; Hwynn, Nelson; Hass, Chris J

    2010-11-01

    A substantial number of individuals with Parkinson's disease who display impaired postural stability experience accelerated cognitive decline and an increased prevalence of dementia. To date, studies suggest that this relationship, believed to be due to involvement of nondopaminergic circuitry, occurs later in the disease process. Research has yet to adequately investigate this cognitive-posturomotor relationship especially when examining earlier disease states. To gain greater understanding of the relationship between postural stability and cognitive function/dysfunction we evaluated a more stringent, objective measure of postural stability (center of pressure displacement), and also more specific measures of cognition in twenty-two patients with early to moderate stage Parkinson's disease. The magnitude of the center of pressure displacement in this cohort was negatively correlated with performance on tests known to activate dorsolateral frontal regions. Additionally, the postural stability item of the UPDRS exhibited poor correlation with the more objective measure of center of pressure displacement and all specific measures of cognition. These results may serve as rationale for a more thorough evaluation of postural stability and cognition especially in individuals with mild Parkinson's disease. Greater understanding of the relationship between motor and cognitive processes in Parkinson's disease will be critical for understanding the disease process and its potential therapeutic possibilities.

  14. Amyloid Beta-Mediated Hypomethylation of Heme Oxygenase 1 Correlates with Cognitive Impairment in Alzheimer's Disease.

    PubMed

    Sung, Hye Youn; Choi, Byung-Ok; Jeong, Jee Hyang; Kong, Kyoung Ae; Hwang, Jinha; Ahn, Jung-Hyuck

    2016-01-01

    To identify epigenetically regulated genes involved in the pathogenesis of Alzheimer's disease (AD) we analyzed global mRNA expression and methylation profiles in amyloid precursor protein (APP)-Swedish mutant-expressing AD model cells, H4-sw and selected heme oxygenase-1 (HMOX1), which is associated with pathological features of AD such as neurofibrillary tangles and senile plaques. We examined the epigenetic regulatory mechanism of HMOX1 and its application as a diagnostic and prognostic biomarker for AD. Our results show that HMOX1 mRNA and protein expression was approximately 12.2-fold and 7.9-fold increased in H4-sw cells, respectively. Increased HMOX1 expression was also detected in the brain, particularly the hippocampus, of AD model transgenic mice. However, the methylation of specific CpG sites within its promoter, particularly at CpG located -374 was significantly decreased in H4-sw cells. Treatment of neuroglioma cells with the demethylating agent 5-aza-2'-deoxycytidine resulted in reduced methylation of HMOX1 promoter accompanied by enhanced HMOX1 expression strongly supporting DNA methylation-dependent transcriptional regulation of HMOX1. Toxic Aβ-induced aberrant hypomethylation of HMOX1 at -374 promoter CpG site was correlated with increased HMOX1 expression. In addition to neuroglioma cells, we also found Aβ-induced epigenetic regulation of HMOX1 in human T lymphocyte Jurkat cells. We evaluated DNA methylation status of HMOX1 at -374 promoter CpG site in blood samples from AD patients, patients with mild cognitive impairment (MCI), and control individuals using quantitative methylation-specific polymerase chain reaction. We observed lower methylation of HMOX1 at the -374 promoter CpG site in AD patients compared to MCI and control individuals, and a correlation between Mini-Mental State Examination score and demethylation level. Receiver operating characteristics analysis revealed good discrimination of AD patients from MCI patients and control

  15. The ratio of N-acetyl aspartate to glutamate correlates with disease duration of amyotrophic lateral sclerosis.

    PubMed

    Sako, Wataru; Abe, Takashi; Izumi, Yuishin; Harada, Masafumi; Kaji, Ryuji

    2016-05-01

    Glutamate (Glu)-induced excitotoxicity has been implicated in the neuronal loss of amyotrophic lateral sclerosis. To test the hypothesis that Glu in the primary motor cortex contributes to disease severity and/or duration, the Glu level was investigated using MR spectroscopy. Seventeen patients with amyotrophic lateral sclerosis were diagnosed according to the El Escorial criteria for suspected, possible, probable or definite amyotrophic lateral sclerosis, and enrolled in this cross-sectional study. We measured metabolite concentrations, including N-acetyl aspartate (NAA), creatine, choline, inositol, Glu and glutamine, and performed partial correlation between each metabolite concentration or NAA/Glu ratio and disease severity or duration using age as a covariate. Considering our hypothesis that Glu is associated with neuronal cell death in amyotrophic lateral sclerosis, we investigated the ratio of NAA to Glu, and found a significant correlation between NAA/Glu and disease duration (r=-0.574, p=0.02). The "suspected" amyotrophic lateral sclerosis patients showed the same tendency as possible, probable and definite amyotrophic lateral sclerosis patients in regard to correlation of NAA/Glu ratio with disease duration. The other metabolites showed no significant correlation. Our findings suggested that glutamatergic neurons are less vulnerable compared to other neurons and this may be because inhibitory receptors are mainly located presynaptically, which supports the notion of Glu-induced excitotoxicity.

  16. Correlation between Helicobacter pylori-associated gastric diseases and colorectal neoplasia

    PubMed Central

    Qing, Ying; Wang, Min; Lin, Ying-Min; Wu, Dong; Zhu, Jing-Yu; Gao, Lang; Liu, Yan-Yan; Yin, Teng-Fei

    2016-01-01

    AIM: To explore the correlation between Helicobacter pylori (H. pylori)-associated gastric diseases and colorectal neoplasia. METHODS: Patients included in this study underwent a colonoscopy and esophago-gastro-duodenoscopy (EGD) along with histopathological measurement between March 2012 and March 2015 at Qi-Lu Hospital of Shandong University, who also had results of H. pylori detection. A total of 233 cases were selected. Demographic data, H. pylori infection status (including results of rapid urease tests and gastric mucosa pathological examinations) and histopathological examination results of gastric and colorectal mucosa were gathered and analyzed. The statistical analysis focused on the prevalence of colorectal neoplasms among patients with various histopathological categories of the stomach. ORs and their 95%CI were calculated to describe the strengths of the associations. RESULTS: The incidence rates of colorectal adenoma without high-grade intraepithelial neoplasia (HGIEN) (OR = 2.400, 95%CI: 0.969-5.941), adenoma with HGIEN (5.333, 1.025-27.758) and adenocarcinoma (1.455, 0.382-5.543) were all higher for patients with H. pylori-associated gastritis than for those in the control group. The incidence rate of colorectal adenoma with HGIEN (3.218, 0.767-13.509) was higher in patients with intestinal metaplasia than in the control group, while the incidence rates of adenoma without HGIEN (0.874, 0.414-1.845) and adenocarcinoma (0.376, 0.096-1.470) were lower in the intestinal metaplasia group than in the control group. The incidence rate of colorectal adenoma without HGIEN (3.111, 1.248-7.753) was significantly higher in the gastric intraepithelial neoplasia group than in the control group, while the rates of adenoma with HGIEN (1.481, 0.138-15.941) and adenocarcinoma (2.020, 0.561-7.272) were higher in the gastric intraepithelial neoplasia group. Incidence rates of colorectal adenoma without HGIEN (1.067, 0.264-4.314), adenoma with HGIEN (2.667, 0

  17. Correlation between serum ferritin levels and liver iron concentration determined by MR imaging: impact of hematologic disease and inflammation.

    PubMed

    Olthof, Allard W; Sijens, Paul E; Kreeftenberg, Herman G; Kappert, Peter; Irwan, Roy; van der Jagt, Eric J; Oudkerk, Matthijs

    2007-02-01

    Liver iron concentration was determined in 28 patients by magnetic resonance imaging using the method of Gandon et al. (Non-invasive assessment of hepatic iron stores by MRI. Lancet 2004;363:357-362). The result showed a significant correlation with blood plasma ferritin content (Spearman's r=.66; P<.001) and a slightly improving correlation coefficient when limited to those patients not known to have inflammation (r=.82; n=17; P<.001). Zooming in on patients with hematologic disease also had a beneficial effect on the correlation between liver iron content and plasma ferritin level (r=.79; n=13; P=.001). It is concluded that in patients without inflammation and in patients with hematologic disease, the content of ferritin in blood is a better predictor of liver iron content than in other patient categories.

  18. Hyperuricemia Inversely Correlates with Disease Severity in Taiwanese Nonalcoholic Steatohepatitis Patients

    PubMed Central

    Huang, Jee-Fu; Yeh, Ming-Lun; Yu, Ming-Lung; Huang, Chung-Feng; Dai, Chia-Yen; Hsieh, Ming-Yen; Hsieh, Meng-Hsuan; Huang, Ching-I; Lin, Zu-Yau; Chen, Shinn-Chern; Hsiao, Pi-Jung; Shin, Shyi-Jang; Chuang, Wan-Long

    2015-01-01

    Background & Aims Asians are more susceptible to non-alcoholic steatohepatitis (NASH) as well as metabolic disorder than other ethnicities. We aimed to assess the interaction between metabolic factors and fibrosis in Taiwanese NASH patients. Methods A total of 130 biopsy-proven Taiwanese NASH patients (94 males, age = 43.0 ± 13.0 years) were consecutively enrolled. Their demographic, metabolic profiles and histopathological manifestations were analyzed. Results Twenty-four (18.5%) NASH patients were non-obese. Thirty-three (25.4%) patients had significant fibrosis (F2) or more: 22 (16.9%) patients were of F2, whilst 11 (8.5%) patients were of advanced fibrosis (F3-4). The prevalence of metabolic syndrome, diabetes and hypertension were 60.8%, 39.4%, and 61.5%, respectively. There was a significant inverse correlation between hyperuricemia and fibrosis stages, ranging from 48.4% of F0-1, 33.3% of F2, and 9.1% of F3-4, respectively (P = 0.01, linear trend). Multivariate logistic regression analysis showed that a decreased serum albumin level (OR = 40.0, 95% CI = 4.5–300, P = 0.001) and normal uric acid level (OR = 5.6, 95% CI = 1.5–21.7, P = 0.01) were the significant factors associated with significant fibrosis. Conclusions Hyperuricemia inversely predicts fibrosis stages. Females might carry a more disease severity than males in Taiwanese NASH patients. PMID:26441244

  19. Rotavirus diarrhea in Bangladeshi children: correlation of disease severity with serotypes.

    PubMed Central

    Bern, C; Unicomb, L; Gentsch, J R; Banul, N; Yunus, M; Sack, R B; Glass, R I

    1992-01-01

    To improve the understanding of the relative importance of serotypes of rotavirus in dehydrating diarrhea, we examined the correlation of clinical characteristics and disease severity with serotype in 2,441 diarrheal episodes among children younger than 2 years of age in rural Bangladesh. Of 764 rotavirus-associated episodes, a single G type (serotype 1, 2, 3, or 4) was determined by oligonucleotide probe in 485 (63%), while 233 episodes were nontypeable. Episodes with G types 2 and 3 were associated with more-severe dehydration than episodes associated with G type 1 or 4 or with nontypeable rotavirus. Episodes did not differ by G type in prevalence of vomiting, copious diarrhea, fever, abdominal pain, or length of treatment center stay. Rotavirus reinfections were detected in seven children, with homologous reinfection (G type 2) in one. Twelve children with diarrhea who died had rotavirus detected in stool specimens within 30 days of death. Children who died were more likely to be malnourished than were surviving children with rotavirus diarrhea. Of 40 specimens tested by polymerase chain reaction, 29 (72.5%) were P type 1, 9 (22.5%) were P type 2, 1 (2.5%) was P type 3, and 1 (2.5%) was nontypeable. One severely symptomatic diarrheal episode was associated with P type 3 rotavirus, a serotype usually found in asymptomatic nursery infections. Although G types 2 and 3 were associated with more-severe dehydration than other serotypes, the differences do not appear to be of major clinical importance. Effective vaccines should protect against all four major G types. PMID:1333490

  20. Olfactory dysfunction: its early temporal relationship and neural correlates in the pathogenesis of Alzheimer's disease.

    PubMed

    Daulatzai, Mak Adam

    2015-10-01

    We interact with the physical world through our senses, and these aid our behavioral performance and various activities of life. Sensory information is transmitted in neuronal networks, and the brain optimally interprets the external and internal milieu/environment. This paper delineates the framework in which the pathogenesis of memory and cognitive dysfunction is underpinned by sensory olfactory dysfunction. ERC is the gateway for olfactory input to the hippocampus, and there is seamless synchronization between sensory function and hippocampal activity. Transmission of olfactory information to the hippocampus is sequential-it is projected from the olfactory receptors to olfactory bulb to the primary olfactory cortex (comprised the anterior olfactory nucleus, the olfactory tubercle, and the piriform cortex) to the entorhinal cortex (ERC). Through perforant pathway ERC enables olfactory inputs to effectively excite hippocampal neurons. One of the earliest pathological changes in Alzheimer's disease (AD) include the olfactory dysfunction and the atrophy in ERC and hippocampus (rate in ERC is higher than in the hippocampus). Olfactory dysfunction negatively impacts the ERC and the deafferenting of the hippocampus from olfactory inputs upregulates memory decline. Olfactory dysfunction, therefore, is an important and early correlate of AD pathology. A number of factors described here may cause olfactory dysfunction; this may lead to hypoperfusion, hypometabolism, impaired synaptic transmission, and variable atrophy in olfaction-related regions. Improvement in olfactory function, therefore, is an important goal in order to attenuate cognitive neuropathology in aging and AD. This article seeks to provide a comprehensive and balanced overview of olfactory neuropathology in incipient AD, and suggests strategies to enhance olfactory function and ameliorate cognitive decline. PMID:25944089

  1. Endogenous nitric oxide in patients with stable COPD: correlates with severity of disease

    PubMed Central

    Clini, E.; Bianchi, L.; Pagani, M.; Ambrosino, N.

    1998-01-01

    BACKGROUND—Increased levels of exhaled nitric oxide (eNO) have been reported in asthmatic subjects but little information is available on eNO in patients with advanced chronic obstructive pulmonary disease (COPD). A study was undertaken to evaluate the levels of eNO in patients with stable COPD of different degrees of severity.
METHODS—Peak and plateau values of eNO (PNO and PLNO, respectively) were evaluated in 53 patients with COPD and analysed according to the level of forced expiratory volume in one second (FEV1) and the presence of cor pulmonale (CP) (group 1, FEV1 <35% predicted with CP, n = 15; group 2, FEV1 <35% predicted without CP, n =15; group 3, FEV1 >35% predicted, n = 23). Seventeen normal subjects served as controls.
RESULTS—All the patients with COPD had reduced levels of PLNO compared with the controls (mean (SD) 6.3 (3.0) and 9.4 (2.8) ppb, respectively). In groups 1 and 2 PLNO levels were significantly lower than in subjects in group 3 (5.5 (2.9), 5.7 (3.5), and 7.1 (2.7) ppb, respectively; p<0.01 ANOVA). In all subjects % predicted FEV1 correlated slightly with PLNO but not with PNO.
CONCLUSION—Patients with severe stable COPD have reduced levels of eNO compared with normal subjects. eNO levels are slightly related to the severity of airflow obstruction.

 PMID:10193378

  2. Plasma Uromodulin Correlates With Kidney Function and Identifies Early Stages in Chronic Kidney Disease Patients.

    PubMed

    Steubl, Dominik; Block, Matthias; Herbst, Victor; Nockher, Wolfgang Andreas; Schlumberger, Wolfgang; Satanovskij, Robin; Angermann, Susanne; Hasenau, Anna-Lena; Stecher, Lynne; Heemann, Uwe; Renders, Lutz; Scherberich, Jürgen

    2016-03-01

    Uromodulin, released from tubular cells of the ascending limb into the blood, may be associated with kidney function. This work studies the relevance of plasma uromodulin as a biomarker for kidney function in an observational cohort of chronic kidney disease (CKD) patients and subjects without CKD (CKD stage 0). It should be further evaluated if uromodulin allows the identification of early CKD stages.Plasma uromodulin, serum creatinine, cystatin C, blood-urea-nitrogen (BUN) concentrations, and estimated glomerular filtration rate (eGFR CKD-EPIcrea-cystatin) were assessed in 426 individuals of whom 71 were CKD stage 0 and 355 had CKD. Besides descriptive statistics, univariate correlations between uromodulin and biomarkers/eGFR were calculated using Pearson-correlation coefficient. Multiple linear regression modeling was applied to establish the association between uromodulin and eGFR adjusted for demographic parameters and pharmacologic treatment. Receiver-operating-characteristic (ROC) analysis adjusted for demographic parameters was performed to test if uromodulin allows differentiation of subjects with CKD stage 0 and CKD stage I.Mean uromodulin plasma levels were 85.7 ± 60.5 ng/mL for all CKD stages combined. Uromodulin was correlated with all biomarkers/eGFR in univariate analysis (eGFR: r = 0.80, creatinine: r = -0.76, BUN: r = -0.72, and cystatin C: r = -0.79). Multiple linear regression modeling showed significant association between uromodulin and eGFR (coefficient estimate β = 0.696, 95% confidence interval [CI] 0.603-0.719, P < 0.001). In ROC analysis uromodulin was the only parameter that significantly improved a model containing demographic parameters to differentiate between CKD 0° and I° (area under the curve [AUC] 0.831, 95% CI 0.746-0.915, P = 0.008) compared to creatinine, cystatin C, BUN, and eGFR (AUC for creatinine: 0.722, P = 0.056, cystatin C: 0.668, P = 0.418, BUN: 0.653, P = 0.811, and e

  3. Plasma Uromodulin Correlates With Kidney Function and Identifies Early Stages in Chronic Kidney Disease Patients

    PubMed Central

    Steubl, Dominik; Block, Matthias; Herbst, Victor; Nockher, Wolfgang Andreas; Schlumberger, Wolfgang; Satanovskij, Robin; Angermann, Susanne; Hasenau, Anna-Lena; Stecher, Lynne; Heemann, Uwe; Renders, Lutz; Scherberich, Jürgen

    2016-01-01

    Abstract Uromodulin, released from tubular cells of the ascending limb into the blood, may be associated with kidney function. This work studies the relevance of plasma uromodulin as a biomarker for kidney function in an observational cohort of chronic kidney disease (CKD) patients and subjects without CKD (CKD stage 0). It should be further evaluated if uromodulin allows the identification of early CKD stages. Plasma uromodulin, serum creatinine, cystatin C, blood-urea-nitrogen (BUN) concentrations, and estimated glomerular filtration rate (eGFR CKD-EPIcrea-cystatin) were assessed in 426 individuals of whom 71 were CKD stage 0 and 355 had CKD. Besides descriptive statistics, univariate correlations between uromodulin and biomarkers/eGFR were calculated using Pearson-correlation coefficient. Multiple linear regression modeling was applied to establish the association between uromodulin and eGFR adjusted for demographic parameters and pharmacologic treatment. Receiver-operating-characteristic (ROC) analysis adjusted for demographic parameters was performed to test if uromodulin allows differentiation of subjects with CKD stage 0 and CKD stage I. Mean uromodulin plasma levels were 85.7 ± 60.5 ng/mL for all CKD stages combined. Uromodulin was correlated with all biomarkers/eGFR in univariate analysis (eGFR: r = 0.80, creatinine: r = −0.76, BUN: r = −0.72, and cystatin C: r = −0.79). Multiple linear regression modeling showed significant association between uromodulin and eGFR (coefficient estimate β = 0.696, 95% confidence interval [CI] 0.603–0.719, P < 0.001). In ROC analysis uromodulin was the only parameter that significantly improved a model containing demographic parameters to differentiate between CKD 0° and I° (area under the curve [AUC] 0.831, 95% CI 0.746–0.915, P = 0.008) compared to creatinine, cystatin C, BUN, and eGFR (AUC for creatinine: 0.722, P = 0.056, cystatin C: 0.668, P = 0.418, BUN: 0.653, P

  4. Real-time quantitative PCR detection of WT1 gene expression in children with AML: prognostic significance, correlation with disease status and residual disease detection by flow cytometry.

    PubMed

    Trka, J; Kalinová, M; Hrusák, O; Zuna, J; Krejcí, O; Madzo, J; Sedlácek, P; Vávra, V; Michalová, K; Jarosová, M; Starý, J

    2002-07-01

    The clinical significance of WT1 gene expression at diagnosis and during therapy of AML has not yet been resolved. We analysed WT1 expression at presentation in an unselected group of 47 childhood AML patients using real-time quantitative reverse-transcription PCR. We also showed that within the first 30 h following aspiration RQ-RT-PCR results were not influenced by transportation time. We observed lower levels of WT1 transcript in AML M5 (P = 0.0015); no association was found between expression levels and sex, initial leukocyte count and karyotype-based prognostic groups. There was significant correlation between very low WT1 expression at presentation and excellent outcome (EFS P = 0.0014). Combined analysis of WT1 levels, three-colour flow cytometry residual disease detection and the course of the disease in 222 samples from 28 children with AML showed remarkable correlation. Fourteen patients expressed high WT1 levels at presentation. In eight of them, who suffered relapse or did not reach complete remission, dynamics of WT1 levels clearly correlated with the disease status and residual disease by flow cytometry. We conclude that very low WT1 levels at presentation represent a good prognostic factor and that RQ-RT-PCR-based analysis of WT1 expression is a promising and rapid approach for monitoring of MRD in approximately half of paediatric AML patients.

  5. Rainbow trout resistance to bacterial cold-water disease is moderately heritable and is not adversely correlated with growth.

    PubMed

    Silverstein, J T; Vallejo, R L; Palti, Y; Leeds, T D; Rexroad, C E; Welch, T J; Wiens, G D; Ducrocq, V

    2009-03-01

    The objectives of this study were to estimate the heritabilities for and genetic correlations among resistance to bacterial cold-water disease and growth traits in a population of rainbow trout (Oncorhynchus mykiss). Bacterial cold-water disease, a chronic disease of rainbow trout, is caused by Flavobacterium psychrophilum. This bacterium also causes acute losses in young fish, known as rainbow trout fry syndrome. Selective breeding for increased disease resistance is a promising strategy that has not been widely used in aquaculture. At the same time, improving growth performance is critical for efficient production. At the National Center for Cool and Cold Water Aquaculture, reducing the negative impact of diseases on rainbow trout culture and improving growth performance are primary objectives. In 2005, when fish averaged 2.4 g, 71 full-sib families were challenged with F. psychrophilum and evaluated for 21 d. Overall survival was 29.3% and family rates of survival varied from 1.5 to 72.5%. Heritability of postchallenge survival, an indicator of disease resistance, was estimated to be 0.35 +/- 0.09. Body weights at 9 and 12 mo posthatch and growth rate from 9 to 12 mo were evaluated on siblings of the fish in the disease challenge study. Growth traits were moderately heritable, from 0.32 for growth rate to 0.61 for 12-mo BW. Genetic and phenotypic correlations between growth traits and resistance to bacterial cold-water disease were not different from zero. These results suggest that genetic improvement can be made simultaneously for growth and bacterial cold-water disease resistance in rainbow trout by using selective breeding.

  6. Macrophage Activation in Pediatric Nonalcoholic Fatty Liver Disease (NAFLD) Correlates with Hepatic Progenitor Cell Response via Wnt3a Pathway

    PubMed Central

    Renzi, Anastasia; De Stefanis, Cristiano; Stronati, Laura; Franchitto, Antonio; Alisi, Anna; Onori, Paolo; De Vito, Rita; Alpini, Gianfranco; Gaudio, Eugenio

    2016-01-01

    Non-alcoholic fatty liver disease is one of the most important causes of liver-related morbidity in children. In non-alcoholic fatty liver disease, the activation of liver resident macrophage pool is a central event in the progression of liver injury. The aims of the present study were to evaluate the polarization of liver macrophages and the possible role of Wnt3a production by macrophages in hepatic progenitor cell response in the progression of pediatric non-alcoholic fatty liver disease. 32 children with biopsy-proven non-alcoholic fatty liver disease were included. 20 out of 32 patients were treated with docosahexaenoic acid for 18 months and biopsies at the baseline and after 18 months were included. Hepatic progenitor cell activation, macrophage subsets and Wnt/β-catenin pathway were evaluated by immunohistochemistry and immunofluorescence. Our results indicated that in pediatric non-alcoholic fatty liver disease, pro-inflammatory macrophages were the predominant subset. Macrophage polarization was correlated with Non-alcoholic fatty liver disease Activity Score, ductular reaction, and portal fibrosis; docosahexaenoic acid treatment determined a macrophage polarization towards an anti-inflammatory phenotype in correlation with the reduction of serum inflammatory cytokines, with increased macrophage apoptosis, and with the up-regulation of macrophage Wnt3a expression; macrophage Wnt3a expression was correlated with β-catenin phosphorylation in hepatic progenitor cells and signs of commitment towards hepatocyte fate. In conclusion, macrophage polarization seems to have a key role in the progression of pediatric non-alcoholic fatty liver disease; the modulation of macrophage polarization could drive hepatic progenitor cell response by Wnt3a production. PMID:27310371

  7. Relations between hypometabolism in the posterior association neocortex and hippocampal atrophy in Alzheimer's disease: a PET/MRI correlative study

    PubMed Central

    Meguro, K; LeMestric, C; Landeau, B; Desgranges, B; Eustache, F; Baron, J

    2001-01-01

    OBJECTIVES—Hippocampal atrophy and hypometabolism in the posterior association neocortex are two well known features of Alzheimer's disease. A correlation between these two features was reported twice previously, suggesting intriguing relations. This question has been reassessed, this time controlling for severity of dementia as well as assessing each side of the brain separately and using a voxel based image analysis in addition to the previously employed regions of interest (ROIs).
PATIENTS AND METHODS—Eleven patients were studied with probable Alzheimer's disease and mild to moderate dementia in whom both volume MRI and PET assessed cerebral glucose consumption (CMRGlc) were available. Hypothesis driven correlations between hippocampal width (an index of atrophy) and CMRGlc were performed for two posterior association regions, the superior temporal and the inferior parietal (angular gyrus) cortices, using ROIs set separately for each hemisphere. To confirm significant correlations from the ROIs approach, if any, and to assess their specificity for the posterior association neocortex, CMRGlc image voxel based analysis of correlations with hippocampal width was then carried out.
RESULTS—There was a significant correlation, in the positive—neurobiologically expected—direction, between right hippocampal width and right angular gyrus metabolism (p< 0.01, Spearman), which remained significant with Kendall partial correlation controlling for dementia severity (estimated by mini mental state scores). Statistical non-parametric mapping (SnPM) confirmed this correlation (p< 0.025), and showed a single additional correlation in the right middle temporal gyrus (p< 0.005), which is also part of the posterior association cortex.
CONCLUSION—The findings with both ROIs and voxel based mapping replicate earlier reports of a relation between hippocampal atrophy and ipsilateral association cortex hypometabolism in Alzheimer's disease, and for the first time

  8. Correlation between decreased CSF α-synuclein and Aβ₁₋₄₂ in Parkinson disease.

    PubMed

    Buddhala, Chandana; Campbell, Meghan C; Perlmutter, Joel S; Kotzbauer, Paul T

    2015-01-01

    Accumulation of misfolded α-synuclein (α-syn) protein in Lewy bodies and neurites is the cardinal pathologic feature of Parkinson disease (PD), but abnormal deposition of other proteins may also play a role. Cerebrospinal fluid (CSF) levels of proteins known to accumulate in PD may provide insight into disease-associated changes in protein metabolism and their relationship to disease progression. We measured CSF α-syn, amyloid β₁₋₄₂ (Aβ₁₋₄₂), and tau from 77 nondemented PD and 30 control participants. CSF α-syn and Aβ₁₋₄₂ were significantly lower in PD compared with controls. In contrast with increased CSF tau in Alzheimer disease, CSF tau did not significantly differ between PD and controls. CSF protein levels did not significantly correlate with ratings of motor function or performance on neuropsychological testing. As expected, CSF Aβ₁₋₄₂ inversely correlated with [(11)C]-Pittsburgh compound B (PiB) mean cortical binding potential, with PiB(+) PD participants having lower CSF Aβ₁₋₄₂ compared with PiB(-) PD participants. Furthermore, CSF α-syn positively correlated with Aβ₁₋₄₂ in PD participants but not in controls, suggesting a pathophysiologic connection between the metabolisms of these proteins in PD.

  9. Correlation of Promis Scales and Clinical Measures Among Chronic Obstructive Pulmonary Disease Patients With and Without Exacerbations

    PubMed Central

    Irwin, Debra E.; Atwood, Charles A.; Hays, Ron D.; Spritzer, Karen; Liu, Honghu; Donohue, James F.; Leidy, Nancy Kline; Yount, Susan E.; DeWalt, Darren A.

    2014-01-01

    Purpose The Patient-Reported Outcomes Measurement Information System (PROMIS®) initiative was developed to advance the methodology of PROs applicable to chronic diseases. Chronic obstructive pulmonary disease (COPD) is a progressive chronic disease associated with poor health. This study was designed to examine the correlation of PROMIS health-related quality of life (HRQOL) scales and clinical measures among COPD patients. Methods A cross-sectional analysis was conducted comparing patients who were stable (n = 100) with those currently experiencing a COPD exacerbation (n=85). All PROMIS measures for adults available at the time of the study (2008), disease-targeted and other HRQOL instruments, health literacy, percent predicted FEV1, and a 6-minute walk distance were assessed when patients were considered clinically stable. Results Stable COPD patients reported significantly (p≤0.05) better health-related quality of life on PROMIS domains than patients experiencing an exacerbation. PROMIS domain scores were significantly (p≤0.01) correlated with each of legacy measures. Six-minute walk scores were most highly correlated with the PROMIS physical function domain scores (r=0.53) followed by the fatigue (r=-0.26), social (r=0.24) and to a lesser extent depression (r=-0.23) and anxiety (r=-0.22) domain scores. Percent predicted FEV1 score was significantly associated with PROMIS physical function scores (r=0.27). Conclusion This study provides support for the validity of the PROMIS measures in COPD patients. PMID:25307510

  10. [Possible correlations between periodontitis and chronic obstructive pulmonary disease. Review of the literature].

    PubMed

    Martos, Renáta; Márton, Ildikó

    2011-09-01

    Chronic obstructive pulmonary disease (COPD) is a complex, multifactorial inflammatory disease of the airways and the pulmonary parenchyme, caused by infection, air pollution and particles. 4-7% of the adult population is involved. COPD is the 4th-6th common cause of death throughout the world. The main aetiological factor is smoking. Bacteria, such as bacteria from the oral cavity, could play a keyrole in the progression of the disease. Epidemiologic studies have noted a relationship between poor oral hygiene or periodontal bone loss and chronic obstructive pulmonary disease. The prevalence and mortality of the disease is increasing worldwide, the treatment is expensive, the efficiency of the present pharmacotherapy is poor, so the importance of prevention should be increasing. Patients with chronic obstructive pulmonary disease (COPD) are prone to frequent exacerbations which are a significant cause of morbidity and mortality. This review is a short summary of studies about the possible relationship between periodontitis and COPD. PMID:22039714

  11. Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease*

    PubMed Central

    Gameiro, Ana; Gouveia, Miguel; Cardoso, José Carlos; Tellechea, Oscar

    2016-01-01

    Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

  12. Correlation of spicule sign on computed tomography scans with peripheral lung cancers associated with interstitial lung disease and chronic obstructive pulmonary disease.

    PubMed

    Chen, L; Gao, L; Wu, W L

    2015-03-27

    The objective of this study was to investigate the correlation between spicular signs on computed tomography (CT) scans and peripheral lung cancer (PLC) that is associated with interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD). We analyzed clinical data from 96 PLC cases and grouped patients based on whether they had interstitial pneumonia into either ILD/COPD group or non-ILD/COPD group. The occurrence rate of spicule sign was 90.3% in the ILD/COPD group and 61.8% in the non-ILD/COPD group, respectively. There was a significant difference between these groups (P < 0.05). There were no significant differences in the occurrence rate of spicular signs among patients with different pathological types of PLC. The severity of ILD affected the spicular morphology on CT scans directly. There was a significant correlation between the appearance of spicule sign on CT scans and PLC that was associated with ILD/COPD.

  13. Increased serum levels of MIP-1alpha correlate with bone disease and angiogenic cytokines in patients with multiple myeloma.

    PubMed

    Tsirakis, George; Roussou, Parascevi; Pappa, Constantina A; Kolovou, Anna; Vasilokonstantaki, Chrysoula; Miminas, Ioannis; Kyriakaki, Stavroula; Alegakis, Athanasios; Alexandrakis, Michael G

    2014-01-01

    Many cytokines possess variable roles in the pathogenesis of multiple myeloma. Macrophage inflammatory protein-1alpha (MIP-1alpha) is an osteoclast-activating factor with a major role in myeloma bone disease. The aim of the study was to examine its participation in the angiogenic process of the disease. We measured, by enzyme-linked immunosorbent assays, its serum levels in 56 newly diagnosed myeloma patients, in several skeletal grades and stages of the disease and in 25 healthy controls. Concurrently, we measured serum levels of the angiogenic cytokines basic-fibroblast growth factor, hepatocyte growth factor and interleukin-18. All the above cytokines were higher in myeloma patients (p < 0.001 for all cases) and were increasing in parallel with disease stage (p < 0.001 for all cases) and skeletal grade (p < 0.04 for MIP-1alpha and p < 0.001 for the other cases). Moreover, positive correlations between MIP-1alpha and all the angiogenic cytokines were noted (p < 0.001 for all cases). MIP-1alpha seems to be a predominant factor responsible for the enhancement of bone resorption and increased angiogenesis. The positive correlation between MIP-1alpha and the angiogenic chemoattractants supports the involvement of these factors in the biology of myeloma cell growth. Moreover, they could be used as possible therapeutic targets as well as markers of disease activity.

  14. Osteopontin is increased in the cerebrospinal fluid of patients with Alzheimer's disease and its levels correlate with cognitive decline.

    PubMed

    Comi, Cristoforo; Carecchio, Miryam; Chiocchetti, Annalisa; Nicola, Stefania; Galimberti, Daniela; Fenoglio, Chiara; Cappellano, Giuseppe; Monaco, Francesco; Scarpini, Elio; Dianzani, Umberto

    2010-01-01

    Inflammation is believed to play a role in Alzheimer's disease (AD). Osteopontin (OPN) is a molecule involved in macrophage recruitment and activation and implicated in neurodegeneration. In order to elucidate the role of OPN in AD, we evaluated its levels in serum and cerebrospinal fluid (CSF) of 67 AD patients, 46 frontotemporal dementia (FTD) patients, and 69 controls. We found that OPN levels: i) are significantly increased in the CSF of AD patients; ii) correlate with MMSE score; and iii) are higher in the early disease phases ( 2 years). These findings support a role of OPN in AD pathogenesis. PMID:20308780

  15. Blood-Borne Activity-Dependent Neuroprotective Protein (ADNP) is Correlated with Premorbid Intelligence, Clinical Stage, and Alzheimer's Disease Biomarkers.

    PubMed

    Malishkevich, Anna; Marshall, Gad A; Schultz, Aaron P; Sperling, Reisa A; Aharon-Peretz, Judith; Gozes, Illana

    2015-01-01

    Biomarkers for Alzheimer's disease (AD) are vital for disease detection in the clinical setting. Discovered in our laboratory, activity-dependent neuroprotective protein (ADNP) is essential for brain formation and linked to cognitive functions. Here, we revealed that blood borne expression of ADNP and its paralog ADNP2 is correlated with premorbid intelligence, AD pathology, and clinical stage. Age adjustment showed significant associations between: 1) higher premorbid intelligence and greater serum ADNP, and 2) greater cortical amyloid and lower ADNP and ADNP2 mRNAs. Significant increases in ADNP mRNA levels were observed in patients ranging from mild cognitive impairment (MCI) to AD dementia. ADNP2 transcripts showed high correlation with ADNP transcripts, especially in AD dementia lymphocytes. ADNP plasma/serum and lymphocyte mRNA levels discriminated well between cognitively normal elderly, MCI, and AD dementia participants. Measuring ADNP blood-borne levels could bring us a step closer to effectively screening and tracking AD.

  16. Human immunodeficiency virus type 1 expression in the central nervous system correlates directly with extent of disease

    SciTech Connect

    Weiser, B.; La Neve, D.; Eilbott, D.J.; Burger, H.; Seidman, R. ); Peress, N. Veterans Administration Medical Center, Northport, NY )

    1990-05-01

    To investigate human immunodeficiency virus type 1 (HIV-1) pathogenesis in infected individuals and examine the correlation of HIV-1 expression with extent of clinical and pathologic disease, the authors studied spinal cords from acquired immunodeficiency syndrome patients with a wide range of spinal cord pathology. By performing in situ hybridization with HIV-1-specific riboprobes, they detected HIV-1 RNA in all 10 cords from acquired immunodeficiency syndrome patients with a common, characteristic pathologic entity called vacuolar myelopathy but not in 10 control cords from HIV-1-infected and uninfected patients. In the cords from individuals with vacuolar myelopathy, the level of HIV-1 RNA expression correlated directly with extent of spinal cord pathology and clinical findings. These data support a role for HIV-1 int he pathogenesis of tissue damage and related clinical disease in infected individuals.

  17. A molecular marker of disease activity in autoimmune liver diseases with histopathological correlation; FoXp3/RORγt ratio.

    PubMed

    Mitra, Suvradeep; Anand, Shashi; Das, Ashim; Thapa, Baburam; Chawla, Yogesh Kumar; Minz, Ranjana Walker

    2015-11-01

    Autoimmune liver diseases (AILDs) encompass a group of diseases with variable clinicopathological manifestations. Th17 and Treg cells have roles in the pathogenesis of AILDs with a balance shifted towards a relative increase in activity of the Th17 cells. In this study, the balance between the transcription factors of Treg and Th17 cells (FoXp3 and RORγt) was sought as a molecular marker of disease activity and to highlight the pathogenesis. The peripheral blood samples of 46 treatment-naive patients were collected and RNA was extracted. Real time PCR was performed and the ratio of gene expression was calculated. Histopathology of 18 patients was obtained and the activity score of these biopsies were also corroborated with their respective molecular (FoXp3/RORγt) (FRGT=FoXp3-ROR Gamma T) ratio. The FRGT ratio in healthy individuals was close to 1 and in disease the ratio changed significantly. This ratio (FRGT) was not significantly different in different varieties of AILD or in adult or paediatric form of the disease. However, the ratio remained consistently below 1 (mean 0.3) in acute disease and high (mean 224.7) in chronic or asymptomatic form of the disease (p < 0.001). The histopathological activity score also significantly correlated with the ratio. This signified the relative excess of Th17 (RORγt) in active disease as compared to Treg (FoXp3) and the reverse in chronic form. This ratio can be an important peripheral molecular marker to assess the disease activity without the necessity of performing a liver biopsy.

  18. Examining the neural correlates of depressive and motor symptoms in Parkinson's disease using Frequency Component Analysis (FCA)

    NASA Astrophysics Data System (ADS)

    Song, Xiaopeng; Hu, Xiao; Zhou, Shuqin; Liu, Weiguo; Liu, Yijun; Zhu, Huaiqiu; Gao, Jia-Hong

    2016-03-01

    Depression is prevalent among patients with Parkinson's disease (PD); however the pathophysiology of depression in PD is not well understood. In order to investigate how depression and motor impairments differentially and interactively affect specific brain regions in Parkinson's disease, we introduced a new data driven approach, namely Frequency Component Analysis (FCA), to decompose the resting-state functional magnetic resonance imaging data of 59 subjects with Parkinson's disease into different frequency bands. We then evaluated the main effects of motor severity and depression, and their interactive effects on the BOLD-fMRI signal oscillation energy in these specific frequency components. Our results show that the severity of motor symptoms is more negatively correlated with energy in the frequency band of 0.10-0.25Hz in the bilateral thalamus (THA), but more positively correlated with energy in the frequency band of 0.01-0.027Hz in the bilateral postcentral gyrus (PoCG). In contrast, the severity of depressive symptoms is more associated with the higher energy of the high frequency oscillations (>0.1Hz) but lower energy of 0.01-0.027Hz in the bilateral subgenual gyrus (SGC). Importantly, the interaction between motor and depressive symptoms is negatively correlated with the energy of high frequency oscillations (>0.1Hz) in the substantia nigra/ventral tegmental area (SN/VTA), left hippocampus (HIPP), left inferior orbital frontal cortex (OFC), and left temporoparietal junction (TPJ), but positively correlated with the energy of 0.02-0.05Hz in the left inferior OFC, left TPJ, left inferior temporal gyrus (ITG), and bilateral cerebellum. These results demonstrated that FCA was a promising method in interrogating the neurophysiological implications of different brain rhythms. Our findings further revealed the neural bases underlying the interactions as well the dissociations between motor and depressive symptoms in Parkinson's disease.

  19. Dopamine-dependent non-linear correlation between subthalamic rhythms in Parkinson's disease

    PubMed Central

    Marceglia, S; Foffani, G; Bianchi, A M; Baselli, G; Tamma, F; Egidi, M; Priori, A

    2006-01-01

    The basic information architecture in the basal ganglia circuit is under debate. Whereas anatomical studies quantify extensive convergence/divergence patterns in the circuit, suggesting an information sharing scheme, neurophysiological studies report an absence of linear correlation between single neurones in normal animals, suggesting a segregated parallel processing scheme. In 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-treated monkeys and in parkinsonian patients single neurones become linearly correlated, thus leading to a loss of segregation between neurones. Here we propose a possible integrative solution to this debate, by extending the concept of functional segregation from the cellular level to the network level. To this end, we recorded local field potentials (LFPs) from electrodes implanted for deep brain stimulation (DBS) in the subthalamic nucleus (STN) of parkinsonian patients. By applying bispectral analysis, we found that in the absence of dopamine stimulation STN LFP rhythms became non-linearly correlated, thus leading to a loss of segregation between rhythms. Non-linear correlation was particularly consistent between the low-beta rhythm (13–20 Hz) and the high-beta rhythm (20–35 Hz). Levodopa administration significantly decreased these non-linear correlations, therefore increasing segregation between rhythms. These results suggest that the extensive convergence/divergence in the basal ganglia circuit is physiologically necessary to sustain LFP rhythms distributed in large ensembles of neurones, but is not sufficient to induce correlated firing between neurone pairs. Conversely, loss of dopamine generates pathological linear correlation between neurone pairs, alters the patterns within LFP rhythms, and induces non-linear correlation between LFP rhythms operating at different frequencies. The pathophysiology of information processing in the human basal ganglia therefore involves not only activities of individual rhythms, but also

  20. Extracellular protein deposition correlates with glial activation and oxidative stress in Creutzfeldt-Jakob and Alzheimer's disease.

    PubMed

    Van Everbroeck, Bart; Dobbeleir, Itte; De Waele, Michèle; De Leenheir, Evelyn; Lübke, Ursula; Martin, Jean-Jacques; Cras, Patrick

    2004-09-01

    The relation of protein deposition with glial cells and oxidative stress was studied in Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD) and neurologically healthy control patients. Three neocortical areas, the hippocampus, and the cerebellum of 20 CJD, 10 AD and 10 control patients were immunohistochemically examined for the presence of astroglia, microglia, and protein depositions. To investigate the level of oxidative stress the percentage of neurons with cytoplasmic hydroxylated DNA was determined. Astroglia, microglia and oxidative stress were located around amyloid-beta depositions and a clear quantitative relation was identified. These markers were only increased in the hippocampus of AD compared to controls. Quantitative analysis in these groups showed a correlation between the oxidative stress level and the number of microglia in the grey matter. All markers were increased in the grey matter and the cerebellum of CJD when compared to AD and controls. The highest numbers of lesions were observed in a CJD population with a rapid disease progression. Quantitative analysis showed a correlation between the oxidative stress level and all glial cells. Further analysis showed that the number of microglia was related to the intensity of the prion depositions. Glial cells in the brain are thought to be the main producers of oxidative stress, resulting in neuronal death. Our results confirm that this close relationship exists in both AD and CJD. We also show that an increased number of glial cells and therefore possibly oxidative stress is associated with the disease progression.

  1. Asthma and infectious respiratory disease in children--correlation to residence near hazardous waste sites.

    PubMed

    Ma, Jing; Kouznetsova, Maria; Lessner, Lawrence; Carpenter, David O

    2007-12-01

    Rates of hospitalization for asthma and respiratory infectious disease in children were modeled as a function of residence: (1) in a zip code containing a hazardous waste site with persistent organic pollutants ('POPs'); (2) in a zip code with a waste site that did not contain POPs ('other'); or (3) in a zip code without any identified waste site ('clean'), as well as other demographic covariates. After adjustment, living in a zip code containing a POPs waste site significantly increased the frequency of hospitalization for asthma and infectious respiratory disease. Living in a zip code with an 'other' waste site also increased hospitalization frequencies for both diseases. The association was strongest for zip codes whose residents were in the lowest quartile of medium family income. This evidence supports the hypothesis that living near a hazardous waste site increases risk of respiratory disease in children.

  2. Thermal conductivity of biological cells at cellular level and correlation with disease state

    NASA Astrophysics Data System (ADS)

    Park, Byoung Kyoo; Woo, Yunho; Jeong, Dayeong; Park, Jaesung; Choi, Tae-Youl; Simmons, Denise Perry; Ha, Jeonghong; Kim, Dongsik

    2016-06-01

    This paper reports the thermal conductivity k of matched pair cell lines: two pairs of a normal and a cancer cell, one pair of a primary and metastatic cell. The 3ω method with a nanoscale thermal sensor was used to measure k at the single-cell level. To observe the difference in k between normal and cancer cells, the measurements were conducted for Hs 578Bst/Hs 578 T (human breast cells) and TE 353.Sk/TE 354.T (human skin cells). Then k of WM-115/WM-266-4, a primary and metastatic pair of human skin cell, was measured to find the effect of disease progression on k. The measured k data for normal and disease cell samples show statistically meaningful differences. In all cases, k decreased as the disease progressed. This work shows that thermal-analysis schemes, such as the 3ω method, have a potential to detect diseases at the cell level.

  3. [Correlation between clinical parameters and quantitative analysis of inflammatory infiltrate. Importance in the diagnosis of active periodontal disease].

    PubMed

    Mendieta Fiter, C

    1989-05-01

    Recent studies have indicated that the destruction in chronic periodontal disease occurs in relatively short periods of time which are followed of prolonged periods of inactivity. These bursts of activity are characterized by an increase in the inflammatory reaction. It has been the purpose of this paper to study the correlation between the clinical measurements of periodontal disease and the histomorphometric quantification of areas of dense inflammatory infiltrate. The results of this study show that the clinical parameters that measure gingival inflammation or loss of periodontal attachment are useful to distinguish pathology from normal (p less than 0.003), but lack sensitivity to detect burst of periodontal disease activity (p greater than 0.05). PMID:2489938

  4. [Correlation between clinical parameters and quantitative analysis of inflammatory infiltrate. Importance in the diagnosis of active periodontal disease].

    PubMed

    Mendieta Fiter, C

    1989-05-01

    Recent studies have indicated that the destruction in chronic periodontal disease occurs in relatively short periods of time which are followed of prolonged periods of inactivity. These bursts of activity are characterized by an increase in the inflammatory reaction. It has been the purpose of this paper to study the correlation between the clinical measurements of periodontal disease and the histomorphometric quantification of areas of dense inflammatory infiltrate. The results of this study show that the clinical parameters that measure gingival inflammation or loss of periodontal attachment are useful to distinguish pathology from normal (p less than 0.003), but lack sensitivity to detect burst of periodontal disease activity (p greater than 0.05).

  5. HLA correlates in a cohort of slow progressors from China: effects on HIV-1 disease progression.

    PubMed

    Rai, Mohammad A; Zhang, Yonghong; Yindom, Louis-M; Holmes, Jane; Yu, Ly-Mee; Hao, Chun; Rostron, Tim; Yan, Huiping; Zhang, Yong Li; Cai, Chao; McMichael, Andrew J; Dong, Tao; Rowland-Jones, Sarah L; Blais, Marie-E; Xu, Ke Yi

    2013-11-13

    We looked at our HIV + slow progressors cohort to determine if there were any human leukocyte antigen (HLA) correlates for protection. No statistically significant allelic differences were found between the HIV + and control cohorts using regression analysis, though trends were noted. Data for Elite Controllers showed an increased frequency of B*57. Likewise, no correlation was inferred with the clinical data of the HIV + cohort. We hypothesize that the protective effect of HLA alleles may have been lost over time. PMID:23942057

  6. Correlation of the serum concentrations of tumour necrosis factor and nitric oxide with disease severity in chronic Chagas disease (American trypanosomiasis).

    PubMed

    Pérez-Fuentes, R; López-Colombo, A; Ordóñez-Toquero, G; Gomez-Albino, I; Ramos, J; Torres-Rasgado, E; Salgado-Rosas, H; Romero-Díaz, M; Pulido-Pérez, P; Sánchez-Guillén, M C

    2007-03-01

    Pro-inflammatory cytokines such as tumour necrosis factor (TNF) and nitric oxide (NO) are believed to play an important role in the severity of chronic disease. When evaluated in 71 patients who were seropositive for Trypanosoma cruzi and 50 apparently healthy controls, the mean (S.D.) serum concentrations of both TNF [7.65 (1.32) nu. 4.24 (1.53) ng/ml; P<0.001] and NO [114 (40) nu. 74 (21) microM; P<0.0001] were found to be significantly higher in the patients than in the controls. In addition, patients with chronic, symptomatic disease affecting their hearts--eight with dilated cardiomyopathy [8.82 (1.47) ng TNF/ml; 142 (45) microM NO] and 17 others with electrocardiographic alterations [8.37 (1.26) ng TNF/ml; 134 (53) microM NO]--had significantly higher serum concentrations of these cytokines than 34 patients who were in the asymptomatic, indeterminate phase of the disease [6.38 (1.35) ng TNF/ml; 99 (28) microM NO]. In those infected with T. cruzi, it therefore appears that serum concentrations of TNF and NO correlate with disease severity, indicating that these cytokines play some role in the pathogenesis of chronic Chagas disease.

  7. Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation.

    PubMed

    Lim, Hyo Soon; Jeong, Su Jin; Lee, Ji Shin; Park, Min Ho; Kim, Jin Woong; Shin, Sang Soo; Park, Jin Gyoon; Kang, Heoung Keun

    2011-01-01

    Paget disease is a rare malignancy of the breast characterized by infiltration of the nipple epidermis by adenocarcinoma cells. The clinical features of Paget disease are characteristic and should increase the likelihood of the diagnosis being made. An important point is that more than 90% of cases of Paget disease are associated with an additional underlying breast malignancy. Paget disease is frequently associated with ductal carcinoma in situ (DCIS) in the underlying lactiferous ducts of the nipple-areolar complex; it may even be associated with DCIS or invasive breast cancer elsewhere in the breast, at least 2 cm from the nipple-areolar complex. Nevertheless, mammographic findings may be negative in up to 50% of cases. Magnetic resonance (MR) imaging can be useful in patients with Paget disease for evaluation of the nipple-areolar complex and identification of an additional underlying malignancy in the breast. The appropriate surgical treatment must be carefully selected and individualized on the basis of radiologic findings, especially those obtained with breast MR imaging.

  8. Differentiated pediatric thyroid cancer: correlates with adult disease, controversies in treatment.

    PubMed

    Parisi, Marguerite T; Mankoff, David

    2007-09-01

    The biologic behavior of differentiated thyroid cancer can differ between adults and children, especially in those children younger than 10 years of age. Unlike adults, young children typically present with advanced disease at diagnosis. Despite this, children respond rapidly to therapy and have an excellent prognosis that is significantly better than that of their adult counterparts with advanced disease. In contradistinction to adults, children with thyroid cancer also have higher local and distant disease recurrences with progression-free survival of only 70% at 5 years, mandating life-long surveillance. Although thyroid cancer is the most common carcinoma in children, overall incidence is low, a factor that has prevented performance of a controlled, randomized, prospective study to determine the most efficacious treatment regimen in this age group. So, although extensively investigated, treatment of pediatric patients with differentiated thyroid cancer remains controversial. This article reviews the current controversies in the treatment of pediatric differentiated thyroid cancer, focusing on issues of optimal initial and subsequent therapy as well as that of long-term follow-up. Our approach to treatment is presented. In so doing, similarities and differences between adults and children with differentiated thyroid cancer as regards unique considerations in epidemiology, diagnosis, staging, treatment, therapy-related late effects, and disease surveillance are presented. The expanding use of and appropriate roles for thyrogen and fluorine-18-fluorodeoxyglucose positron emission tomography in disease evaluation and surveillance will be addressed.

  9. Correlation between Helicobacter pylori infection, gastric diseases and life habits among patients treated at a university hospital in Southeast Brazil.

    PubMed

    Módena, José Luiz Proença; Acrani, Gustavo Olszanski; Micas, André Fernando Ditondo; Castro, Marly de; Silveira, Wanderley Dias da; Módena, José Luiz Pimenta; Oliveira, Ricardo Brandt de; Brocchi, Marcelo

    2007-02-01

    Helicobacter pylori is considered a significant agent in the development of various gastric diseases. However, the diseases caused by this bacterium are known as being multi-factorial, with the genotype, immune system and life habits of the host playing important roles in the establishment of the clinical outcome. Also, H. pylori exhibit a high degree of genetic variability, contributing to the complexity of the host-pathogen relationship. These observations, considered together with the widely-varying origins and social habits of the Brazilian population, lead us to speculate about the influence of these life habits on H. pylori infection and the clinical outcome. Therefore, in this study we evaluated the relationship between H. pylori infection and certain diseases in 172 patients treated at the Hospital das Clínicas of Ribeirão Preto (HCRP), Brazil, taking into account their different life habits, such as non-steroidal anti-inflammatory drugs and alcohol ingestion, and smoking habit. Our analysis indicated that H. pylori infection is not affected by any of the life habits evaluated but is associated with the development of peptic ulcers (gastric and duodenal ulcer) and inverse correlate with gastroesophageal reflux disease (GERD). No correlation was found between the infection with this bacterium and gastritis or intestinal metaplasia. However, gastritis and erosive gastritis were directly correlated with non-steroidal anti-inflammatory drugs (NSAID) ingestion. Moreover, ingestion of alcohol beverages exhibited a protective effect on gastritis development in men. Our data also indicated that to achieve reliable detection of this bacterium in biopsies, two or three detection methods should be used. PMID:17625734

  10. Spatial Correlation Analysis between Particulate Matter 10 (PM10) Hazard and Respiratory Diseases in Chiang Mai Province, Thailand

    NASA Astrophysics Data System (ADS)

    Trang, N. Ha; Tripathi, N. K.

    2014-11-01

    Every year, during dry season, Chiang Mai and other northern provinces of Thailand face the problem of haze which is mainly generated by the burning of agricultural waste and forest fire, contained high percentage of particulate matter. Particulate matter 10 (PM10), being very small in size, can be inhaled easily to the deepest parts of the human lung and throat respiratory functions. Due to this, it increases the risk of respiratory diseases mainly in the case of continuous exposure to this seasonal smog. MODIS aerosol images (MOD04) have been used for four weeks in March 2007 for generating the hazard map by linking to in-situ values of PM10. Simple linear regression model between PM10 and AOD got fair correlation with R2 = 0.7 and was applied to transform PM10 pattern. The hazard maps showed the dominance of PM10 in northern part of Chiang Mai, especially in second week of March when PM10 level was three to four times higher than standard. The respiratory disease records and public health station of each village were collected from Provincial Public Health Department in Chiang Mai province. There are about 300 public health stations out of 2070 villages; hence thiessen polygon was created to determine the representative area of each public health station. Within each thiessen polygon, respiratory disease incident rate (RDIR) was calculated based on the number of patients and population. Global Moran's I was computed for RDIR to explore spatial pattern of diseases through four weeks of March. Moran's I index depicted a cluster pattern of respiratory diseases in 2nd week than other weeks. That made sense for a relationship between PM10 and respiratory diseases infections. In order to examine how PM10 affect the human respiratory system, geographically weighted regression model was used to observe local correlation coefficient between RDIR and PM10 across study area. The result captured a high correlation between respiratory diseases and high level of PM10 in

  11. Nutritional and metabolic correlates of cardiovascular and bone disease in HIV-infected patients1234

    PubMed Central

    Fitch, Kathleen

    2011-01-01

    The treatment of HIV infection has dramatically reduced the incidence of AIDS-related illnesses. At the same time, non-AIDS-related illnesses such as cardiovascular and bone disease are becoming more prevalent in this population. The mechanisms of these illnesses are complex and are related in part to the HIV virus, antiretroviral medications prescribed for HIV infection, traditional risk factors exacerbated by HIV, and lifestyle and nutritional factors. Further prospective research is needed to clarify the mechanisms by which HIV, antiretroviral medications, and nutritional abnormalities contribute to bone and cardiovascular disease in the HIV population. Increasingly, it is being recognized that optimizing the treatment of HIV infection to improve immune function and reduce viral load may also benefit the development of non-AIDS-related illnesses such as cardiovascular and bone disease. PMID:22089442

  12. Nutritional and metabolic correlates of cardiovascular and bone disease in HIV-infected patients.

    PubMed

    Fitch, Kathleen; Grinspoon, Steven

    2011-12-01

    The treatment of HIV infection has dramatically reduced the incidence of AIDS-related illnesses. At the same time, non-AIDS-related illnesses such as cardiovascular and bone disease are becoming more prevalent in this population. The mechanisms of these illnesses are complex and are related in part to the HIV virus, antiretroviral medications prescribed for HIV infection, traditional risk factors exacerbated by HIV, and lifestyle and nutritional factors. Further prospective research is needed to clarify the mechanisms by which HIV, antiretroviral medications, and nutritional abnormalities contribute to bone and cardiovascular disease in the HIV population. Increasingly, it is being recognized that optimizing the treatment of HIV infection to improve immune function and reduce viral load may also benefit the development of non-AIDS-related illnesses such as cardiovascular and bone disease.

  13. Correlation of carotid artery disease severity and vasomotor response of cerebral blood vessels.

    PubMed

    Krdžić, Ivana; Čovičković-Šternić, Nadežda; Katsiki, Niki; Isenović, Esma R; Radak, Đorđe

    2015-05-01

    We assessed reactivity of cerebral vessels on hypercapnia in patients with carotid occlusive disease. The effects of vascular risk factors on carotid atherosclerosis and vasomotor reactivity (VMR) of cerebral arterioles were also examined. Patients (n = 50) with carotid stenosis (≥30% in 1 or both sides) were included; 30 patients acted as controls. Hypertension, hyperlipidemia, diabetes, cardiac diseases, inflammation, and smoking were recorded. Vasomotor reactivity was assessed with the apnea test by transcranial Doppler ultrasonography and estimated by flow velocity changes in the middle cerebral artery before and after hypercapnia induction. Vasomotor reactivity was defined by the breath holding index, and values under 0.69 were considered critical for VMR impairment. Vasomotor reactivity reduction was significant (P = .004) in patients with severe carotid stenosis (>70%) and with symptomatic carotid disease (P < .05). The risk factors did not significantly influence VMR reduction. Severe carotid stenosis impairs VMR and may increase the risk of stroke, especially in symptomatic patients.

  14. Molecular Phylogeny of the Psittacid Herpesviruses Causing Pacheco's Disease: Correlation of Genotype with Phenotypic Expression

    PubMed Central

    Tomaszewski, Elizabeth K.; Kaleta, Erhard F.; Phalen, David N.

    2003-01-01

    Fragments of 419 bp of the UL16 open reading frame from 73 psittacid herpesviruses (PsHVs) from the United States and Europe were sequenced. All viruses caused Pacheco's disease, and serotypes of the European isolates were known. A phylogenetic tree derived from these sequences demonstrated that the PsHVs that cause Pacheco's disease comprised four major genotypes, with each genotype including between two and four variants. With the exception of two viruses, the serotypes of the virus isolates could be predicted by the genotypes. Genotypes 1 and 4 corresponded to serotype 1 isolates, genotype 2 corresponded to serotype 2 isolates, and genotype 3 corresponded to serotype 3 isolates. The single serotype 4 virus mapped to genotype 4. DNA from a virus with a unique serotype could not be amplified with primers that amplified DNA from all other PsHVs, and its classification remains unknown. Viruses representing all four genotypes were found in both the United States and Europe, and it was therefore predicted that serotypes 1, 2, and 3 were present in the United States. Serotype 4 was represented by a single European isolate that could not be genetically distinguished from serotype 1 viruses; therefore, the presence of serotype 4 in the United States could not be predicted. Viruses of genotype 4 were found to be the most commonly associated with Pacheco's disease in macaws and conures and were least likely to be isolated in chicken embryo fibroblasts in the United States. All four genotypes caused deaths in Amazon parrots, but genotype 4 was associated with Pacheco's disease only in Amazons in Europe. Genotypes 2, 3, and 4, but not 1, were found in African grey parrots. Although parrots from the Pacific distribution represent a relatively small percentage of the total number of birds with Pacheco's disease, all four genotypes were found to cause disease in these species. PMID:14512573

  15. Higher minor hemoglobin A2 levels in multiple sclerosis patients correlate with lesser disease severity

    PubMed Central

    Ozcan, Muhammed Emin; Ince, Bahri; Karadeli, Hasan Huseyin; Gedikbasi, Asuman; Asil, Talip; Altinoz, Meric A

    2016-01-01

    Objective To define whether minor adult hemoglobin A2 (HbA2, α2δ2) exerts any protective activity in multiple sclerosis (MS). Methods HbA2 levels were measured in 146 MS patients with high performance liquid chromatography and association with MS Severity Scores (MSSS) were determined. HbA2 associations with blood count parameters were also studied using blood counts evaluated on the same day of high performance liquid chromatography sampling. Routine biochemical parameters were also determined to rule out elusively influential factors, such as anemia and thyroid disorders. Results HbA2 levels negatively correlated with MSSS (Spearman correlation, R: −0.186, P=0.025). Exclusion of confounding factors with a generalized linear model revealed an even stronger negative correlation between HbA2 and MSSS (P<0.001). HbA2 positively correlated with red blood cells (RBCs) (R=0.350, P<0.001) and in turn, RBCs negatively correlated with MSSS (R=−0.180, P=0.031). Average HbA2 levels were highest among patients treated with interferon β1a. Conclusion RBC fragility is increased in MS, and recent data suggest that circulating free Hb contributes to neural injury in MS. HbA2 and its oxidative denaturation product hemichrome A2 enhance RBC membrane stability to a greater extent than do major HbA or hemichrome A. Reductions in ischemic cerebrovascular vascular events are reported in β-thalassemia carriers and HbA2 levels are considerably higher in this population. Episodic declines of cerebral blood flow were shown in bipolar disorder, and we have recently shown a protective role of HbA2 against postpartum episodes in females with bipolar disorder. HbA2’s erythroprotective functions may reduce free Hb and long-term neural injury in MS. PMID:27578976

  16. An overview on the correlation of neurological disorders with cardiovascular disease

    PubMed Central

    Firoz, C.K.; Jabir, Nasimudeen R.; Khan, Mohd Shahnawaz; Mahmoud, Maged; Shakil, Shazi; Damanhouri, Ghazi A.; Zaidi, Syed Kashif; Tabrez, Shams; Kamal, Mohammad A.

    2014-01-01

    Neurological disorders (NDs) are one of the leading causes of death especially in the developed countries. Among those NDs, Alzheimer’s disease (AD) and Parkinson disease (PD) are heading the table. There have been several reports in the scientific literatures which suggest the linkage between cardiovascular disorders (CVDs) and NDs. In the present communication, we have tried to compile NDs (AD and PD) association with CVDs reported in the literature. Based on the available scientific literature, we believe that further comprehensive study needs to be done to elucidate the molecular linking points associated with the above mentioned disorders. PMID:25561878

  17. Plasma levels of tumour necrosis factor and its soluble receptors correlate with clinical features and outcome of Hodgkin's disease patients.

    PubMed Central

    Warzocha, K.; Bienvenu, J.; Ribeiro, P.; Moullet, I.; Dumontet, C.; Neidhardt-Berard, E. M.; Coiffier, B.; Salles, G.

    1998-01-01

    A prospective study was performed to assess the use of plasma measurement of tumour necrosis factor (TNF), lymphotoxin alpha (LT alpha) and their soluble receptors (p55 and p75) for prognostic risk assignment in 61 patients with Hodgkin's disease. Plasma levels of TNF, p55 and p75, but not of LT alpha, were higher in Hodgkin's disease patients than in healthy controls. Plasma levels of TNF, p55 and p75 were associated with several prognostic factors for Hodgkin's disease, including those related to the host (age, performance status) and to the tumour (disease stage, extranodal site involvement, bulky tumour, serum levels of LDH and beta2-microglobulin, histology). Elevated plasma levels of TNF, p55 and p75 were also associated with several parameters reflecting an immune activation, including the presence of B symptoms, elevated serum levels of gammaglobulins, alkaline phosphatase and fibrinogen, as well as peripheral monocytosis, anaemia and low serum albumin levels. Finally, elevated TNF ligand receptor plasma markers were associated with a lower incidence of complete response to therapy and predicted shorter free-from-progression survival and overall survival of the patients. These results indicate that the plasma levels of TNF and its soluble receptors correlate with clinical features and outcome of patients with Hodgkin's disease. PMID:9649158

  18. Cleft Palate, Retrognathia and Congenital Heart Disease in Velo-Cardio-Facial Syndrome: A Phenotype Correlation Study

    PubMed Central

    Friedman, Marcia A.; Miletta, Nathanial; Roe, Cheryl; Wang, Dongliang; Morrow, Bernice E.; Kates, Wendy R.; Higgins, Anne Marie; Shprintzen, Robert J.

    2011-01-01

    Objective Velo-cardio-facial syndrome (VCFS) is caused by a microdeletion of approximately 40 genes from one copy of chromosome 22. Expression of the syndrome is a variable combination of over 190 phenotypic characteristics. As of yet, little is known about how these phenotypes correlate with one another or whether there are predictable patterns of expression. Two of the most common phenotypic categories, congenital heart disease and cleft palate, have been proposed to have a common genetic relationship to the deleted T-box 1 gene (TBX1). The purpose of this study is to determine if congenital heart disease and cleft palate are correlated in a large cohort of human subjects with VCFS. Methods This study is a retrospective chart review including 316 Caucasian non-Hispanic subjects with FISH or CGH microarray confirmed chromosome 22q11.2 deletions. All subjects were evaluated by the interdisciplinary team at the Velo-Cardio-Facial Syndrome International Center at Upstate Medical University, Syracuse, NY. Each combination of congenital heart disease, cleft palates, and retrognathia was analyzed by chi square or Fisher exact test. Results For all categories of congenital heart disease and cleft palate or retrognathia no significant associations were found, with the exception of submucous cleft palate and retrognathia (nominal p=0.0325) and occult submucous cleft palate and retrognathia (nominal p=0.000013). Conclusions Congenital heart disease and cleft palate do not appear to be correlated in human subjects with VCFS despite earlier suggestions from animal models. Possible explanations include modification of the effect of TBX1 by genes outside of the 22q11.2 region that may further influence the formation of the palate or heart, or the presence of epigenetic factors that may effect genes within the deleted region, modifying genes elsewhere, or polymorphisms on the normal copy of chromosome 22. Lastly, it is possible that TBX1 plays a role in palate formation in some

  19. Significant positive correlation between sunshine and lactase nonpersistence in Europe may implicate both in similarly altering risks for some diseases.

    PubMed

    Szilagyi, Andrew; Leighton, Henry; Burstein, Barry; Shrier, Ian

    2011-01-01

    Decreasing latitude and increasing frequency of population lactase nonpersistence have been reported to diminish risks for several diseases, but the reason for overlap has not been explained. We evaluate, relationships between calculated national annual ultraviolet light B (UVB) exposure, latitude, and national lactose digestion frequencies. Annual UVB exposure and latitude were based on weighted averages from several cities in different countries. Lactase distribution status was based on published data that have been used previously to derive relations with diseases. We compare univariate regression analyses (r(2)(adj), slope) of percentage of lactase nonpersistence with UVB or latitude. We determine, differences between European and non-European sources by multiregression analysis of independent variables. Correlation between UVB and latitude is high (r(2) = 0.89), and between percentage of lactase nonpersistence and either latitude or UVB the correlation is moderately strong with r(2) = 0.51 and 0.46, respectively, with P ≤ 0.01 for both. A more detailed analysis shows that correlations between percentage of lactase nonpersistence and UVB are only significant in Europe, r(2) = 0.59, P < 0.001, whereas outside Europe: r(2) = 0.06, P = 0.16. These relationships raise hypothetical explanations to account for the observed overlap in similar risk modification by the 2 variables.

  20. Cytokine and chemokine profiles in multiple myeloma; significance of stromal interaction and correlation of IL-8 production with disease progression.

    PubMed

    Kline, Michael; Donovan, Kathleen; Wellik, Linda; Lust, Christopher; Jin, Wendy; Moon-Tasson, Laurie; Xiong, Yuning; Witzig, Thomas E; Kumar, Shaji; Rajkumar, S Vincent; Lust, John A

    2007-05-01

    Multiple myeloma (MM) is a product of interactions between tumor plasma cells and multiple cell types native to the bone marrow (BM). We have used antibody array technology to examine the proteins produced by BM stromal cells in response to stimulation by BM taken from patients diagnosed with monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), and MM. We observed increased production of the chemokine IL-8 by stromal cells co-cultured with supernatants from bone marrow cells of patients with active myeloma. IL-8 production is correlated with active disease and is dependent upon IL-1beta and NF-kappaB signaling. Consistent with the pro-angiogenic activity of IL-8, increased BM microvessel density (MVD) correlated with stimulation of stromal cell IL-8 production. In addition, the majority of MM cell lines and MM patient plasma cells were found to express IL-8 receptors CXCR1 and CXCR2. We conclude that stromal cell IL-8 production parallels MM disease activity, is IL-1beta induced, and correlates with bone marrow angiogenesis. PMID:16879867

  1. Factors correlated with fatigue in disease-free breast cancer patients: application of the Cancer Fatigue Scale.

    PubMed

    Okuyama, T; Akechi, T; Kugaya, A; Okamura, H; Imoto, S; Nakano, T; Mikami, I; Hosaka, T; Uchitomi, Y

    2000-05-01

    Fatigue is one of the most frequent symptoms in cancer patients. However, the precise causes of this fatigue are still unknown, and this situation makes it difficult to combat the problem. The present study was conducted to investigate factors correlated with fatigue in disease-free breast cancer patients. A group of 134 randomly selected ambulatory breast cancer patients who had undergone successful surgical treatment participated. They completed the Cancer Fatigue Scale, the Hospital Anxiety and Depression Scale, the Mental Adjustment to Cancer Scale, and an ad hoc questionnaire detailing physical symptoms, social support, and demographic variables at home and returned them by mail the following day. Multiple regression analysis revealed that fatigue was significantly correlated with dyspnea, insufficient sleep, and depression, and that these three variables accounted for a total of 46% of variance in fatigue. Factors concerned with the cancer and treatment, such as disease stage, lymph node metastasis, number of days since operation, past intravenous chemotherapy, radiotherapy, current use of fluoropyrimidine compounds, and current use of tamoxifen citrate were not correlated with fatigue. The results suggest that fatigue in this population is determined by current physical and psychological distress rather than by the cancer itself and prior cancer treatments, and that the management of dyspnea, insomnia, and depression might be important in reducing fatigue in this population.

  2. α-Synuclein dimerization in erythrocytes of Gaucher disease patients: correlation with lipid abnormalities and oxidative stress.

    PubMed

    Moraitou, Marina; Dermentzaki, Georgia; Dimitriou, Evangelia; Monopolis, Ioannis; Dekker, Nick; Aerts, Hans; Stefanis, Leonidas; Michelakakis, Helen

    2016-02-01

    Several observations suggest that disturbed homeostasis of α-Synuclein (α-Syn) may provide a link between Gaucher disease (GD) and Parkinson's disease (PD). We recently reported increased dimerization of α-Syn in the red blood cell (RBC) membrane of patients with GD. Several studies indicate a crucial relationship between lipids, oxidative stress and α-Syn status. Here we investigated the relationship between the observed increased dimerization of α-Syn in the cell membranes of RBCs, cells devoid of lysosomes and lacking lysosomal enzyme synthesis, and the lipid abnormalities and oxidative stress already described in GD. Correlation studies showed that in GD the α-Syn dimer/monomer ratio is positively correlated with the levels of glucosylceramide (GlcCer) and the glucosylceramide/ceramide (GlcCer/Cer) ratio and negatively with the levels of malonyldialdehyde (MDA) and plasmalogens. In conclusion, we have shown that the increased tendency of α-Syn to form dimers in the RBC membrane of patients with GD, is correlated with both the level of lipids, including GlcCer, the primary lipid abnormality in GD, and the increased oxidative stress observed in this disorder. The study of other tissues, and in particular brain, will be important in order to elucidate the significance of these findings regarding the link between GD and PD. PMID:26708635

  3. Behavioral and Neurophysiological Correlates of Striatal Dopamine Depletion: A Rodent Model of Parkinson's Disease

    ERIC Educational Resources Information Center

    Plowman, Emily K.; Kleim, Jeffrey A.

    2011-01-01

    Both limb and cranial motor functions are adversely impacted by Parkinson's disease (PD). While current pharmacological and surgical interventions are effective in alleviating general limb motor symptoms of PD, they have failed to provide significant benefit for cranial motor functions. This suggests that the neuropathologies mediating limb and…

  4. Physiological Correlates of Intellectual Function in Children with Sickle Cell Disease: Hypoxaemia, Hyperaemia and Brain Infarction

    ERIC Educational Resources Information Center

    Hogan, Alexandra M.; Pit-ten Cate, Ineke M.; Vargha-Khadem, Faraneh; Prengler, Mara; Kirkham, Fenella J.

    2006-01-01

    Lowered intelligence relative to controls is evident by mid-childhood in children with sickle cell disease. There is consensus that brain infarct contributes to this deficit, but the subtle lowering of IQ in children with normal MRI scans might be accounted for by chronic systemic complications leading to insufficient oxygen delivery to the brain.…

  5. Correlation of Bone Morphogenetic Protein-2 Levels in Serum and Synovial Fluid with Disease Severity of Knee Osteoarthritis

    PubMed Central

    Liu, Yan; Hou, Ruizhi; Yin, Ruofeng; Yin, Weitian

    2015-01-01

    Background This study aimed to investigate the bone morphogenetic protein-2 (BMP-2) levels in serum and synovial fluid (SF) of patients with primary knee osteoarthritis (OA) and to exam its correlation with radiographic and symptomatic severity of the disease. Material/Methods A total of 37 knee OA patients and 20 healthy controls were enrolled in this study. Knee OA radiographic grading was performed according to the Kellgren-Lawrence (KL) grading system by evaluating X-ray changes observed in anteroposterior knee radiography. Symptomatic severity of the disease was evaluated according to the Western Ontario McMaster University Osteoarthritis Index (WOMAC) scores. BMP-2 levels in serum and SF were determined using enzyme-linked immunosorbent assay. Results Serum BMP-2 level in patients with knee OA was higher than that in healthy controls. Knee OA patients with KL grade 4 showed significantly elevated BMP-2 levels in the serum and SF compared with those with KL grade 2 and 3. Knee OA patients with KL grade 3 had significant higher SF levels of BMP-2 than those with KL grade 2. BMP-2 levels in the serum and SF of knee OA patients were both positively correlated with KL grades and WOMAC scores. Conclusions BMP2 levels in serum and SF were closely related to the radiographic and symptomatic severity of knee OA and may serve as an alternative biochemical parameter to determine disease severity of primary knee OA. PMID:25644704

  6. Fractal mechanisms and heart rate dynamics. Long-range correlations and their breakdown with disease

    NASA Technical Reports Server (NTRS)

    Peng, C. K.; Havlin, S.; Hausdorff, J. M.; Mietus, J. E.; Stanley, H. E.; Goldberger, A. L.

    1995-01-01

    Under healthy conditions, the normal cardiac (sinus) interbeat interval fluctuates in a complex manner. Quantitative analysis using techniques adapted from statistical physics reveals the presence of long-range power-law correlations extending over thousands of heartbeats. This scale-invariant (fractal) behavior suggests that the regulatory system generating these fluctuations is operating far from equilibrium. In contrast, it is found that for subjects at high risk of sudden death (e.g., congestive heart failure patients), these long-range correlations break down. Application of fractal scaling analysis and related techniques provides new approaches to assessing cardiac risk and forecasting sudden cardiac death, as well as motivating development of novel physiologic models of systems that appear to be heterodynamic rather than homeostatic.

  7. Vital Capacity Impairment due to Neuromuscular Disease and its Correlation with Diaphragmatic Ultrasound: A Preliminary Study.

    PubMed

    Carrié, Cédric; Bonnardel, Eline; Vally, Rishad; Revel, Philippe; Marthan, Roger; Marthan, Roger

    2016-01-01

    The purpose of this pilot study was to evaluate the correlation between diaphragmatic excursion measured by a right sub-costal ultrasound approach and forced vital capacity in patients with amyotrophic lateral sclerosis (ALS) or myotonic dystrophy (MD). All patients referred for pulmonary function testing underwent ultrasonic measurement of diaphragmatic excursion during quiet breathing, voluntary sniffing (Esniff) and forced breathing (EDEmax). Forty-five patients were included, mainly for amyotrophic lateral sclerosis or myotonic dystrophy. There was a significant correlation between EDEmax values and forced vital capacity (FVC) values (r = 0.68 [0.46–0.90], p < 0.0001) and between EDEmax values and percentage of predicted FVC values (r = 0.75 [0.55–0.95], p < 0.0001). At a threshold of EDEmax < 5.5 cm, the sensitivity and specificity of ultrasonic diaphragmatic excursion in predicting FVC ≤ 50% of theoretical values were 100% [66%–100%] and 69% [52%–84%] respectively, without any significant difference between males and females. There was no statistical correlation between maximal inspiratory pressure and Esniff.

  8. Correlation between global longitudinal peak systolic strain and coronary artery disease severity as assessed by the angiographically derived SYNTAX score

    PubMed Central

    Dawson, David; Grigoratos, Chrysanthos; Nihoyannopoulos, Petros

    2016-01-01

    Background In this study, we investigate the correlation between reduced global longitudinal peak systolic strain (GLPSS) and the SYNTAX score (SS) in patients undergoing coronary angiography. Methods We examined 71 patients undergoing both echocardiogram and coronary angiography within 15 days. All patients had normal global and/or regional wall motion on resting echocardiogram. We calculated GLPSS using two-dimensional speckle-tracking echocardiography. SS was calculated for each group of patients based on the presence and/or the severity of coronary artery disease (CAD): no CAD on angiogram (n=10, control group), low SS (n=36, SS<22) and high SS (n=25, SS≥22). We hypothesised that GLPSS at rest is inversely correlated with the angiographically derived SS. Results Age, sex and most of the risk factors were equally distributed among the groups. There was a significant inverse correlation between GLPSS and SS values (r2=0.3869, P<0.001). This correlation was weaker in the low-SS group (r2=0.1332, P<0.05), whereas it was lost in the high-SS group (r2=0.0002, P=NS). Receiver operating characteristic curve analysis identified that the optimal cut-off for the detection of high-SS patients was 13.95% (sensitivity=71%, specificity=90%, P<0.001). Conclusions The results of our study suggest that GLPSS might be promising for the detection of patients with high SYNTAX score on coronary angiogram. There is an inverse correlation between resting GLPSS and SS as assessed by coronary angiography. In patients with the highest SS, however, the correlation with GLPSS was less significant. PMID:27248153

  9. Analysis of the correlation between serum resistin and the variability of erythropoietin responsiveness in patients with chronic kidney disease

    PubMed Central

    ZHANG, HONGHAO; LI, XIUJIANG; KAN, YANHONG; YANG, FAN; HOU, YUE; DU, YUJUN

    2015-01-01

    Chronic kidney disease (CKD) is commonly accompanied by inflammation and anemia; however, the pathogenesis of CKD is unclear. Expression of resistin, a cysteine-rich secretory plasma protein, is correlated with the expression of tumor necrosis factor (TNF)-α, interleukin (IL)-6 and lipoprotein-associated phospholipase A2, indicating that resistin may be involved in inflammatory events. In addition, inflammation inhibits the activity of erythropoietin (EPO) and, thus, erythropoiesis. The aim of the present study was to analyze the correlation between serum resistin and the variability of EPO responsiveness in CKD patients. The levels of serum creatinine (SCr), C-reactive protein (CRP), total cholesterol, triglycerides, IL-6 and serum resistin were measured in the samples obtained from 138 CKD patients and healthy control subjects. The levels of serum resistin in the CKD groups with and without hemodialysis were significantly higher than those observed in the normal control group (P<0.01) and the levels of serum resistin in the hemodialysis CKD group were higher than those observed in the CKD group without dialysis (P<0.01). The levels of serum resistin in patients in the randomly selected CKD group (with hemodialysis) were positively correlated with the duration of dialysis and the levels of SCr and CRP (P<0.05), however, were negatively correlated with the estimated glomerular filtration rate. The EPO resistance index (ERI) was identified to be associated with body mass index and the levels of CRP and resistin; furthermore, EPO reactivity was correlated with the level of resistin and ERI. The levels of serum resistin were correlated with the variability in EPO responsiveness that was observed in the CKD patients. Therefore, the measurement of serum resistin may aid with understanding the mechanisms, clinical diagnosis and treatment of CKD. PMID:26640574

  10. Correlation between Magnetic Resonance Imaging Characteristics of the Patellar Tendon and Clinical Scores in Osgood-Schlatter Disease

    PubMed Central

    Lee, Dhong Won; Kim, Min Jeong; Kim, Woo Jong; Ha, Jeong Ku

    2016-01-01

    Purpose This study aims to evaluate magnetic resonance imaging (MRI) findings in young adults with symptomatic Osgood-Schlatter disease (OSD) and compare those in young adults without OSD. Materials and Methods We compared MRI findings between young adults with OSD (OS group, n=30) and the equivalent number of young adults without OSD (control group). Visual analog scale scores and Kujala scores were evaluated and correlation analysis was performed in the OS group. Results In the OS group, MRI revealed that the patellar tendon was attached to the tibia more widely, resulting in a reduced free tendon portion, and more proximally to the articular surface (p<0.001). The correlation analysis between MRI findings and clinical scores showed statistically significant correlations (p<0.01). In the OS group, 43% presented with patellar tendinopathy or bone marrow edema at the distal attachments. Conclusions Compared to the control group, the relatively small free portion and relatively proximal attachment of the patellar tendon were observed with MRI in the OS group. The free portion of the patellar tendon was positively correlated with the clinical scores. Patellar tendinopathy was also frequently encountered in the OS group. PMID:26955614

  11. Correlation between Extraocular Muscle Size Measured by Computed Tomography and the Vertical Angle of Deviation in Thyroid Eye Disease.

    PubMed

    Lee, Ju-Yeun; Bae, Kunho; Park, Kyung-Ah; Lyu, In Jeong; Oh, Sei Yeul

    2016-01-01

    The aim of this study was to investigate extraocular muscle (EOM) volume and cross-sectional area using computed tomography (CT), and to determine the relationship between EOM size and the vertical angle of deviation in thyroid eye disease (TED). Twenty-nine TED patients (58 orbits) with vertical strabismus were enrolled in the study. All patients underwent complete ophthalmic examination including prism, alternate cover, and Krimsky tests. Orbital CT scans were also performed on each patient. Digital image analysis was used to quantify superior rectus (SR) and inferior rectus (IR) muscle cross-sectional areas and volumes. Measurements were compared with those of controls. The correlation between muscle size and degree of vertical angle deviation was evaluated. The mean vertical angle of deviation was 26.2 ± 4.1 prism diopters. The TED group had a greater maximum cross-sectional area and EOM volume in the SR and IR than the control group (all p<0.001). Area and volume of the IR were correlated with the angle of deviation, but the SR alone did not show a significant correlation. The maximum cross-sectional area and volume of [Right IR + Left SR - Right SR - Left IR] was strongly correlated with the vertical angle of deviation (P<0.001). Quantitative CT of the orbit with evaluation of the area and volume of EOMs may be helpful in anticipating and monitoring vertical strabismus in TED patients.

  12. Correlation of stress with outcome of radioiodine therapy for Graves disease

    SciTech Connect

    Stewart, T.; Rochon, J.; Lenfestey, R.; Wise, P.

    1985-06-01

    Between November 1965 and December 1983, 293 patients were treated for Graves disease using /sup 131/I. All patients were asked to identify a stressful event antedating the onset of overt clinical symptoms. Eighty-one patients were able to do this (27.6%). Two hundred forty-four patients received a single treatment, 49 required two or more treatments. Patients with stress initiating the symptoms of Graves disease became hypothyroid earlier, 50% at 12 mo compared with 36 mo for the nonstress group. At 10 yr 5% of the stress group remained euthyroid compared with 17% nonstress. The authors conclude that stress in the 12 mo or less before the onset of clinical symptoms potentiates the development of hypothyroidism induced by a standard dose of radioiodine.

  13. Radiographic Evaluation of Valvular Heart Disease With Computed Tomography and Magnetic Resonance Correlation.

    PubMed

    Lempel, Jason K; Bolen, Michael A; Renapurkar, Rahul D; Azok, Joseph T; White, Charles S

    2016-09-01

    Valvular heart disease is a group of complex entities with varying etiologies and clinical presentations. There are a number of imaging tools available to supplement clinical evaluation of suspected valvular heart disease, with echocardiography being the most common and clinically established, and more recent emergence of computed tomography and magnetic resonance imaging as additional supportive techniques. Yet even with these newer and more sophisticated modalities, chest radiography remains one of the earliest and most common diagnostic examinations performed during the triage of patients with suspected cardiac dysfunction. Recognizing the anatomic and pathologic features of cardiac radiography including the heart's adaptation to varying hemodynamic changes can provide clues to the radiologist regarding the underlying etiology. In this article, we will elucidate several principles relating to chamber modifications in response to pressure and volume overload as well as radiographic appearances associated with pulmonary fluid status and cardiac dysfunction. We will also present a pattern approach to optimize analysis of the chest radiograph for valvular heart disease, which will help guide the radiologist down a differential diagnostic pathway and create a more meaningful clinical report. PMID:27548877

  14. Assessment of Interleukin 16 Serum Levels and Skin Expression in Psoriasis Patients in Correlation with Clinical Severity of the Disease

    PubMed Central

    Purzycka-Bohdan, Dorota; Szczerkowska-Dobosz, Aneta; Zablotna, Monika; Wierzbicka, Justyna; Piotrowska, Anna; Zmijewski, Michal A.; Nedoszytko, Boguslaw; Nowicki, Roman

    2016-01-01

    Interleukin 16 (IL-16) has been described as a significant cytokine involved in the recruitment of CD4+ cells during inflammation; however, its potential role in psoriasis has not been defined. Our aim was to investigate the IL-16 serum levels and IL-16 mRNA skin expression in psoriasis patients in correlation with disease severity and mRNA skin expression for CD4. Moreover, the IL-16 skin localization was assessed and the -295 T/C IL-16 polymorphism was analyzed. For this exploratory, observational, and cross-sectional study, 97 unrelated patients with chronic plaque type psoriasis and 104 healthy controls were enrolled. IL-16 serum levels were significantly increased in patients compared with controls (P = 0.000022) and positively correlated with Psoriasis Area and Severity Index (r = 0.34, P = 0.0007), Body Surface Area (r = 0.34, P = 0.01) and were significantly higher in individuals with moderate to severe psoriasis (P = 0.0029). There was no significant correlation between IL-16 serum levels and Dermatology Quality of Life Index and no differences in genotype and allele frequencies for -295 T/C IL-16 polymorphism. The expression of IL-16 (mRNA and protein) was elevated in the margin of psoriatic skin while statistically significant increase in IL-16 immunoreactivity, but not in mRNA level, was observed within plaques. Furthermore, the IL-16 mRNA levels within psoriatic lesions positively correlated with the levels of CD4 mRNA, but not with Psoriasis Area and Severity Index. In conclusion, our data revealed an association between circulating IL-16 and severity of psoriasis which indicates that this cytokine could serve as a potential marker of disease activity. However, further investigations are required. PMID:27788245

  15. Human plasma C-peptide immunoreactivity: its correlation with immunoreactive insulin in diabetes, and chronic liver and renal diseases.

    PubMed

    Kajinuma, H; Kanazawa, Y; Sando, H; Hayashi, M; Kawazu, S; Kosaka, K

    1979-02-01

    The correlation between plasma C-peptide immunoreactivity (CPR) and immunoreactive insulin (IRI) was investigated during the oral glucose tolerance test in 20 normals, 127 diabetics, and 39 non-diabetics with chronic liver or renal disorders. When all subjects were included, the increment of CPR 30 minutes after glucose load (deltaCPR) correlated well with that of IRI (deltaIRI) (r = 0.66, p less than 0.001), but the return of CPR towards the basal level was delayed as compared with IRI. The positive correlation was also observed between the sum of 6 IRI and that of 6 CPR values during the glucose tolerance test in diabetics and controls (r = 0.53, p less than 0.001). deltaCPR/deltaBS (30 min.) was also well correlated with deltaIRI/deltaBS (30 min.), and was specifically low in diabetics. Insulin-treated maturity-onset diabetics showed low but considerable CPR responses while no CPR responses were observed in insulin-treated juvenile diabetics. In each plasma sample, CPR always exceeded IRI on the molar basis. At fasting CPR/IRI ratio was 15.6 +/- 1.7 (mean +/- SE) in normals and 14.9 +/- 1.3 approximately 16.9 +/- 1.0 in diabetics. In chronic liver diseases IRI response was augmented while CPR response was not different from that of controls, and the molar ratio of CPR/IRI was significantly low (9.5 +/- 1.1). On the contrary, it exceeded that of normals in chronic renal diseases (35.7 +/- 14.9). It is concluded that, first, the plasma CPR response appears to be a valuable indicator of pancreatic B-cell function, and second, it is, nevertheless, modified in chronic liver or renal disorders.

  16. Decreasing Fertility Rate Correlates with the Chronological Increase and Geographical Variation in Incidence of Kawasaki Disease in Japan

    PubMed Central

    Nagao, Yoshiro

    2013-01-01

    Background Kawasaki disease (KD) is a common cause of acquired paediatric heart disease in developed countries. KD was first identified in the 1960s in Japan, and has been steadily increasing since it was first reported. The aetiology of KD has not been defined, but is assumed to be infection-related. The present study sought to identify the factor(s) that mediate the geographical variation and chronological increase of KD in Japan. Methods and Findings Based upon data reported between 1979 and 2010 from all 47 prefectures in Japan, the incidence and mean patient age at the onset of KD were estimated. Using spatial and time-series analyses, incidence and mean age were regressed against climatic/socioeconomic variables. Both incidence and mean age of KD were inversely correlated with the total fertility rate (TFR; i.e., the number of children that would be born to one woman). The extrapolation of a time-series regressive model suggested that KD emerged in the 1960s because of a dramatic decrease in TFR in the 1940s through the 1950s. Conclusions Mean patient age is an inverse surrogate for the hazard of contracting the aetiologic agent. Therefore, the observed negative correlation between mean patient age and TFR suggests that a higher TFR is associated with KD transmission. This relationship may be because a higher TFR facilitates sibling-to-sibling transmission. Additionally, the observed inverse correlation between incidence and TFR implies a paradoxical “negative” correlation between the incidence and the hazard of contracting the aetiologic agent. It was hypothesized that a decreasing TFR resulted in a reduced hazard of contracting the agent for KD, thereby increasing KD incidence. PMID:23861836

  17. Germ-line mutations in the neurofibromatosis 2 gene: Correlations with disease severity and retinal abnormalities

    SciTech Connect

    Parry, D.M.; Kaiser-Kupfer, M.; Eldridge, R.

    1996-09-01

    Neurofibromatosis 2 (NF2) features bilateral vestibular schwannomas, other benign neural tumors, and cataracts. Patients in some families develop many tumors at an early age and have rapid clinical progression, whereas in other families, patients may not have symptoms until much later and vestibular schwannomas may be the only tumors. The NF2 gene has been cloned from chromosome 22q; most identified germ-line mutations result in a truncated protein and severe NF2. To look for additional mutations and clinical correlations, we used SSCP analysis to screen DNA from 32 unrelated patients. We identified 20 different mutations in 21 patients (66%): 10 nonsense mutations, 2 frameshifts, 7 splice-site mutations, and 1 large in-frame deletion. Clinical information on 47 patients from the 21 families included ages at onset and at diagnosis, numbers of meningiomas, spinal and skin tumors, and presence of cataracts and retinal abnormalities. We compared clinical findings in patients with nonsense or frameshift mutations to those with splice-site mutations. When each patient was considered as an independent random event, the two groups differed (P {le} .05) for nearly every variable. Patients with nonsense or frameshift mutations were younger at onset and at diagnosis and had a higher frequency and mean number of tumors, supporting the correlation between nonsense and frameshift mutations and severe NF2. When each family was considered as an independent random event, statistically significant differences between the two groups were observed only for mean ages at onset and at diagnosis. A larger data set is needed to resolve these discrepancies. We observed retinal hamartomas and/or epiretinal membranes in nine patients from five families with four different nonsense mutations. This finding, which may represent a new genotype-phenotype correlation, merits further study. 58 refs., 2 tabs.

  18. Predicted Mutation Strength of Nontruncating PKD1 Mutations Aids Genotype-Phenotype Correlations in Autosomal Dominant Polycystic Kidney Disease.

    PubMed

    Heyer, Christina M; Sundsbak, Jamie L; Abebe, Kaleab Z; Chapman, Arlene B; Torres, Vicente E; Grantham, Jared J; Bae, Kyongtae T; Schrier, Robert W; Perrone, Ronald D; Braun, William E; Steinman, Theodore I; Mrug, Michal; Yu, Alan S L; Brosnahan, Godela; Hopp, Katharina; Irazabal, Maria V; Bennett, William M; Flessner, Michael F; Moore, Charity G; Landsittel, Douglas; Harris, Peter C

    2016-09-01

    Autosomal dominant polycystic kidney disease (ADPKD) often results in ESRD but with a highly variable course. Mutations to PKD1 or PKD2 cause ADPKD; both loci have high levels of allelic heterogeneity. We evaluated genotype-phenotype correlations in 1119 patients (945 families) from the HALT Progression of PKD Study and the Consortium of Radiologic Imaging Study of PKD Study. The population was defined as: 77.7% PKD1, 14.7% PKD2, and 7.6% with no mutation detected (NMD). Phenotypic end points were sex, eGFR, height-adjusted total kidney volume (htTKV), and liver cyst volume. Analysis of the eGFR and htTKV measures showed that the PKD1 group had more severe disease than the PKD2 group, whereas the NMD group had a PKD2-like phenotype. In both the PKD1 and PKD2 populations, men had more severe renal disease, but women had larger liver cyst volumes. Compared with nontruncating PKD1 mutations, truncating PKD1 mutations associated with lower eGFR, but the mutation groups were not differentiated by htTKV. PKD1 nontruncating mutations were evaluated for conservation and chemical change and subdivided into strong (mutation strength group 2 [MSG2]) and weak (MSG3) mutation groups. Analysis of eGFR and htTKV measures showed that patients with MSG3 but not MSG2 mutations had significantly milder disease than patients with truncating cases (MSG1), an association especially evident in extreme decile populations. Overall, we have quantified the contribution of genic and PKD1 allelic effects and sex to the ADPKD phenotype. Intrafamilial correlation analysis showed that other factors shared by families influence htTKV, with these additional genetic/environmental factors significantly affecting the ADPKD phenotype. PMID:26823553

  19. Muscle histopathology in nebulin-related nemaline myopathy: ultrastrastructural findings correlated to disease severity and genotype.

    PubMed

    Malfatti, Edoardo; Lehtokari, Vilma-Lotta; Böhm, Johann; De Winter, Josine M; Schäffer, Ursula; Estournet, Brigitte; Quijano-Roy, Susana; Monges, Soledad; Lubieniecki, Fabiana; Bellance, Remi; Viou, Mai Thao; Madelaine, Angéline; Wu, Bin; Taratuto, Ana Lía; Eymard, Bruno; Pelin, Katarina; Fardeau, Michel; Ottenheijm, Coen A C; Wallgren-Pettersson, Carina; Laporte, Jocelyn; Romero, Norma B

    2014-01-01

    Nemaline myopathy (NM) is a rare congenital myopathy characterised by hypotonia, muscle weakness, and often skeletal muscle deformities with the presence of nemaline bodies (rods) in the muscle biopsy. The nebulin (NEB) gene is the most commonly mutated and is thought to account for approximately 50% of genetically diagnosed cases of NM. We undertook a detailed muscle morphological analysis of 14 NEB-mutated NM patients with different clinical forms to define muscle pathological patterns and correlate them with clinical course and genotype. Three groups were identified according to clinical severity. Group 1 (n = 5) comprises severe/lethal NM and biopsy in the first days of life. Group 2 (n = 4) includes intermediate NM and biopsy in infancy. Group 3 (n = 5) comprises typical/mild NM and biopsy in childhood or early adult life. Biopsies underwent histoenzymological, immunohistochemical and ultrastructural analysis. Fibre type distribution patterns, rod characteristics, distribution and localization were investigated. Contractile performance was studied in muscle fibre preparations isolated from seven muscle biopsies from each of the three groups. G1 showed significant myofibrillar dissociation and smallness with scattered globular rods in one third of fibres; there was no type 1 predominance. G2 presented milder sarcomeric dissociation, dispersed or clustered nemaline bodies, and type 1 predominance/uniformity. In contrast, G3 had well-delimited clusters of subsarcolemmal elongated rods and type 1 uniformity without sarcomeric alterations. In accordance with the clinical and morphological data, functional studies revealed markedly low forces in muscle bundles from G1 and a better contractile performance in muscle bundles from biopsies of patients from G2, and G3.In conclusion NEB-mutated NM patients present a wide spectrum of morphological features. It is difficult to establish firm genotype phenotype correlation. Interestingly, there was a correlation

  20. Muscle histopathology in nebulin-related nemaline myopathy: ultrastrastructural findings correlated to disease severity and genotype

    PubMed Central

    2014-01-01

    Nemaline myopathy (NM) is a rare congenital myopathy characterised by hypotonia, muscle weakness, and often skeletal muscle deformities with the presence of nemaline bodies (rods) in the muscle biopsy. The nebulin (NEB) gene is the most commonly mutated and is thought to account for approximately 50% of genetically diagnosed cases of NM. We undertook a detailed muscle morphological analysis of 14 NEB-mutated NM patients with different clinical forms to define muscle pathological patterns and correlate them with clinical course and genotype. Three groups were identified according to clinical severity. Group 1 (n = 5) comprises severe/lethal NM and biopsy in the first days of life. Group 2 (n = 4) includes intermediate NM and biopsy in infancy. Group 3 (n = 5) comprises typical/mild NM and biopsy in childhood or early adult life. Biopsies underwent histoenzymological, immunohistochemical and ultrastructural analysis. Fibre type distribution patterns, rod characteristics, distribution and localization were investigated. Contractile performance was studied in muscle fibre preparations isolated from seven muscle biopsies from each of the three groups. G1 showed significant myofibrillar dissociation and smallness with scattered globular rods in one third of fibres; there was no type 1 predominance. G2 presented milder sarcomeric dissociation, dispersed or clustered nemaline bodies, and type 1 predominance/uniformity. In contrast, G3 had well-delimited clusters of subsarcolemmal elongated rods and type 1 uniformity without sarcomeric alterations. In accordance with the clinical and morphological data, functional studies revealed markedly low forces in muscle bundles from G1 and a better contractile performance in muscle bundles from biopsies of patients from G2, and G3. In conclusion NEB-mutated NM patients present a wide spectrum of morphological features. It is difficult to establish firm genotype phenotype correlation. Interestingly, there was a correlation

  1. Disease quantification in dermatology: in vivo near-infrared spectroscopy measures correlate strongly with the clinical assessment of psoriasis severity

    NASA Astrophysics Data System (ADS)

    Greve, Tanja Maria; Kamp, Søren; Jemec, Gregor B. E.

    2013-03-01

    Accurate documentation of disease severity is a prerequisite for clinical research and the practice of evidence-based medicine. The quantification of skin diseases such as psoriasis currently relies heavily on clinical scores. Although these clinical scoring methods are well established and very useful in quantifying disease severity, they require an extensive clinical experience and carry a risk of subjectivity. We explore the opportunity to use in vivo near-infrared (NIR) spectra as an objective and noninvasive method for local disease severity assessment in 31 psoriasis patients in whom selected plaques were scored clinically. A partial least squares (PLS) regression model was used to analyze and predict the severity scores on the NIR spectra of psoriatic and uninvolved skin. The correlation between predicted and clinically assigned scores was R=0.94 (RMSE=0.96), suggesting that in vivo NIR provides accurate clinical quantification of psoriatic plaques. Hence, NIR may be a practical solution to clinical severity assessment of psoriasis, providing a continuous, linear, numerical value of severity.

  2. Fasting Glucose Levels Correlate with Disease Severity of Guillain-Barré Syndrome

    PubMed Central

    Wang, Ying; Guan, Yun; Press, Rayomand; Zhu, Jie; Zhang, Hong-Liang

    2015-01-01

    Objective A potential association between diabetes and Guillain-Barré syndrome (GBS) has been indicated by a few case studies. We retrospectively analyzed the clinical features of a large cohort of GBS patients to explore the relationship between the level of fasting plasma glucose (FPG) obtained in the acute phase at admission and the severity of GBS. Methods Three hundred and four GBS patients were divided into two groups, one with normal FPG and the other with high FPG levels according to the international standards of FPG. Results The GBS disability scale score was positively, the Medical Research Council (MRC) sum score was negatively correlated to the level of FPG, but not to blood HBA1c or CSF glucose concentrations. A relatively higher FPG level was observed in older and younger GBS patients, and more often in those with cranial nerve involvement, autonomic deficit, dyspnea and ventilator dependence than in patients without these clinical characteristics. Importantly, higher levels of FPG at admission were associated with poorer short-term prognosis measured by the MRC sum score and the GBS disability scale at discharge. Conclusions Our data demonstrates that FPG in the acute phase of GBS correlates with the severity of GBS and may predict the short-term prognosis of GBS. PMID:26684748

  3. Complement Effectors of Inflammation in Cystic Fibrosis Lung Fluid Correlate with Clinical Measures of Disease

    PubMed Central

    Sass, Laura A.; Hair, Pamela S.; Perkins, Amy M.; Shah, Tushar A.; Krishna, Neel K.; Cunnion, Kenji M.

    2015-01-01

    In cystic fibrosis (CF), lung damage is mediated by a cycle of obstruction, infection, and inflammation. Here we explored complement inflammatory effectors in CF lung fluid. In this study soluble fractions (sols) from sputum samples of 15 CF patients were assayed for complement effectors and analyzed with clinical measurements. The pro-inflammatory peptide C5a was increased 4.8-fold (P = 0.04) in CF sols compared with controls. Incubation of CF sols with P. aeruginosa or S. aureus increased C5a concentration 2.3-fold (P = 0.02). A peptide inhibitor of complement C1 (PIC1) completely blocked the increase in C5a concentration from P. aeruginosa in CF sol in vitro (P = 0.001). C5a concentration in CF sol correlated inversely with body mass index (BMI) percentile in children (r = -0.77, P = 0.04). C3a, which has anti-inflammatory effects, correlated positively with FEV1% predicted (rs = 0.63, P = 0.02). These results suggest that complement effectors may significantly impact inflammation in CF lung fluid. PMID:26642048

  4. Sonographic assessment of carpal tunnel syndrome in rheumatoid arthritis: prevalence and correlation with disease activity.

    PubMed

    Karadag, Omer; Kalyoncu, Umut; Akdogan, Ali; Karadag, Yesim Sucullu; Bilgen, Sule Apras; Ozbakır, Senay; Filippucci, Emilio; Kiraz, Sedat; Ertenli, Ihsan; Grassi, Walter; Calgüneri, Meral

    2012-08-01

    Carpal tunnel syndrome (CTS) is one of the most frequent extra-articular manifestations of rheumatoid arthritis (RA). High frequency ultrasonography (US) is a sensitive and specific method in diagnosis of CTS. This study is aimed to: firstly assess diameter frequency of CTS in RA with US and compare with a control group; secondly, investigate relationship of CTS with disease activity. One hundred consecutive RA patients (women/men: 78/22) fulfilling ACR 1987 RA criteria and 45 healthy controls (women/control: 34/11) were enrolled into study. Disease activity parameters, RA and CTS patient global assessment and health assessment questionnaire (HAQ-DI) were recorded. Both patient and control group were questioned about secondary causes of CTS, and Katz hand diagram, Boston CTS questionnaire and Phalen ve Tinel tests were applied once for each hand. Wrist joint and carpal tunnel were assessed with US grey scale and power Doppler US, then cross-sectional area of median nerve (CSA) was calculated. Patients with median nerve CSA between 10.0 and 13.0 mm(2) were evaluated with electromyography (EMG). CTS was diagnosed if CSA of median nerve >13.0 mm(2) or CTS was shown with NCS. Although there was no difference between RA patients and controls in age, sex, history of DM (+) and goitre, CTS was more frequent in RA group (respectively, 17.0% vs. 4.4%, P = 0.038). In RA group with CTS, age, history of DM, disease duration, HAQ-DI score, CTS patient global score, Boston symptom severity and functional status scores were elevated compared to without CTS [respectively, 57 (36-73) vs. 50 (24-76), P = 0.041; 35.3% vs. 6.0%, P < 0.001; 108 (12-396) months vs. 72 (6-360) months, P = 0.036; 1.93 (0.75-2.87) vs. 1.125 (0-2.75), P = 0.013; 52 (1-97) vs. 25 (0-91), P = 0.001; 2.81 (1.18-4.17) vs. 2.0 (1.0-4.01), P = 0.01; 3.37 (1.37-5.0) vs. 2.25 (1.0-5.0), P = 0.008]. No difference was found between CTS (+) and (-) RA patients in acute phase reactants, disease activity and US findings

  5. Wnt expression is not correlated with β-catenin dysregulation in Dupuytren's Disease

    PubMed Central

    O'Gorman, David B; Wu, Yan; Seney, Shannon; Zhu, Rebecca D; Gan, Bing Siang

    2006-01-01

    Background Dupuytren's contracture or disease (DD) is a fibro-proliferative disease of the hand that results in finger flexion contractures. Increased cellular β-catenin levels have been identified as characteristic of this disease. As Wnts are the most widely recognized upstream regulators of cellular β-catenin accumulation, we have examined Wnt gene expression in surgical specimens and in DD-derived primary cell cultures grown in two-dimensional monolayer culture or in three-dimensional FPCL collagen lattice cultures. Results The Wnt expression profile of patient-matched DD and unaffected control palmar fascia tissue was determined by a variety of complimentary methods; Affymetrix Microarray analysis, specific Wnt and degenerative primer-based Reverse Transcriptase (RT)-PCR, and Real Time PCR. Microarray analysis identified 13 Wnts associated with DD and control tissues. Degenerate Wnt RT-PCR analysis identified Wnts 10b and 11, and to a lesser extent 5a and 9a, as the major Wnt family members expressed in our patient samples. Competitive RT-PCR analysis identified significant differences between the levels of expression of Wnts 9a, 10b and 11 in tissue samples and in primary cell cultures grown as monolayer or in FPCL, where the mRNA levels in tissue > FPCL cultures > monolayer cultures. Real Time PCR data confirmed the down-regulation of Wnt 11 mRNA in DD while Wnt 10b, the most frequently isolated Wnt in DD and control palmar fascia, displayed widely variable expression between the methods of analysis. Conclusion These data indicate that changes in Wnt expression per se are unlikely to be the cause of the observed dysregulation of β-catenin expression in DD. PMID:16942611

  6. Prevalence and Correlates of Post-traumatic Stress Disorder in Adults With Congenital Heart Disease.

    PubMed

    Deng, Lisa X; Khan, Abigail May; Drajpuch, David; Fuller, Stephanie; Ludmir, Jonathan; Mascio, Christopher E; Partington, Sara L; Qadeer, Ayesha; Tobin, Lynda; Kovacs, Adrienne H; Kim, Yuli Y

    2016-03-01

    Post-traumatic stress disorder (PTSD) is associated with adverse outcomes and increased mortality in cardiac patients. No studies have examined PTSD in the adult congenital heart disease (ACHD) population. The objectives of this study were to assess the prevalence of self-reported symptoms of PTSD in patients with ACHD and explore potential associated factors. Patients were enrolled from an outpatient ACHD clinic and completed several validated measures including the Impact of Event Scale-Revised, PTSD Checklist-Civilian Version, and the Hospital Anxiety and Depression Scale. Clinical data were abstracted through medical data review. A total of 134 participants (mean age 34.6 ± 10.6; 46% men) were enrolled. Of the 127 participants who completed the Impact of Event Scale-Revised, 14 (11%) met criteria for elevated PTSD symptoms specifically related to their congenital heart disease or treatment. Of the 134 patients who completed PTSD Checklist-Civilian Version, 27 (21%) met criteria for global PTSD symptoms. In univariate analyses, patients with congenital heart disease-specific PTSD had their most recent cardiac surgery at an earlier year (p = 0.008), were less likely to have attended college (p = 0.04), had higher rates of stroke or transient ischemic attack (p = 0.03), and reported greater depressive symptoms on the Hospital Anxiety and Depression Scale (7 vs 2, p <0.001). In multivariable analysis, the 2 factors most strongly associated with PTSD were depressive symptoms (p <0.001) and year of most recent cardiac surgery (p <0.03). In conclusion, PTSD is present in 11% to 21% of subjects seen at a tertiary referral center for ACHD. The high prevalence of PTSD in this complex group of patients has important implications for the medical and psychosocial management of this growing population.

  7. Characterising the grey matter correlates of leukoaraiosis in cerebral small vessel disease.

    PubMed

    Lambert, Christian; Sam Narean, Janakan; Benjamin, Philip; Zeestraten, Eva; Barrick, Thomas R; Markus, Hugh S

    2015-01-01

    Cerebral small vessel disease (SVD) is a heterogeneous group of pathological disorders that affect the small vessels of the brain and are an important cause of cognitive impairment. The ischaemic consequences of this disease can be detected using MRI, and include white matter hyperintensities (WMH), lacunar infarcts and microhaemorrhages. The relationship between SVD disease severity, as defined by WMH volume, in sporadic age-related SVD and cortical thickness has not been well defined. However, regional cortical thickness change would be expected due to associated phenomena such as underlying ischaemic white matter damage, and the observation that widespread cortical thinning is observed in the related genetic condition CADASIL (Righart et al., 2013). Using MRI data, we have developed a semi-automated processing pipeline for the anatomical analysis of individuals with cerebral small vessel disease and applied it cross-sectionally to 121 subjects diagnosed with this condition. Using a novel combined automated white matter lesion segmentation algorithm and lesion repair step, highly accurate warping to a group average template was achieved. The volume of white matter affected by WMH was calculated, and used as a covariate of interest in a voxel-based morphometry and voxel-based cortical thickness analysis. Additionally, Gaussian Process Regression (GPR) was used to assess if the severity of SVD, measured by WMH volume, could be predicted from the morphometry and cortical thickness measures. We found significant (Family Wise Error corrected p < 0.05) volumetric decline with increasing lesion load predominately in the parietal lobes, anterior insula and caudate nuclei bilaterally. Widespread significant cortical thinning was found bilaterally in the dorsolateral prefrontal, parietal and posterio-superior temporal cortices. These represent distinctive patterns of cortical thinning and volumetric reduction compared to ageing effects in the same cohort, which exhibited

  8. (Non)Compliance with disease prevention and control messages: communication correlates and psychological predictors.

    PubMed

    Burgoon, M

    1996-07-01

    This article argues that while compliance with disease prevention and control messages should be high given the unique characteristics of this persuasive situation, the data indicate that non compliance is a major social problem. Two communication theories, Language Expectancy Theory and Reinforcement Expectancy Theory, offer promising new conceptualizations of how more compliance can be obtained with health-related messages. Both theories are supported by empirical evidence. Also, ongoing research programs with three psychological predictors (misanthropy, acculturation and sensation-seeking) show promise in providing evidence on how to tailor health-related, compliance-gaining messages to subgroups within the general population.

  9. Sleep/wake problems in Parkinson's disease: pathophysiology and clinicopathologic correlations.

    PubMed

    Ondo, William G

    2014-08-01

    In his initial description of shaking palsy, James Parkinson first noted that sleep became disturbed with advancing paralysis agitans. More recent studies have confirmed that the majority of patients with Parkinson's disease (PD) suffer from some sleep disturbances. This can manifest as difficulty in falling or staying asleep, fractionated sleep, specific parasomnias, and daytime sleepiness. In this article, we will explore the pathophysiology of these varied sleep disorders. In most cases, however, the definitive etiology is debated, and phenotypes are often felt to be multifactorial. Some of these may be associated with dopaminergic dysfunction, some presumed to arise from varied non-dopaminergic PD pathology, and some from PD treatments.

  10. Common Variants in PLD3 and Correlation to Amyloid-Related Phenotypes in Alzheimer's Disease.

    PubMed

    Wang, Chong; Tan, Lan; Wang, Hui-Fu; Yu, Wan-Jiang; Liu, Ying; Jiang, Teng; Tan, Meng-Shan; Hao, Xiao-Ke; Zhang, Dao-Qiang; Yu, Jin-Tai

    2015-01-01

    The phospholipase D3 (PLD3) gene has shown association with Alzheimer's disease (AD). However, the role of PLD3 common variants in amyloid-β (Aβ) pathology remains unclear. We examined the association of thirteen common single nucleotide polymorphisms (SNPs) with cerebrospinal fluid (CSF) Aβ(1- 42) levels and florbetapir retention on florbetapir 18F amyloid positron emission tomography (AV45-PET) in a large population. We found that one SNP (rs11667768) was significantly associated with CSF Aβ(1- 42) levels in the normal cognition group. We did not observe an association of any SNP with florbetapir retention. Our study predicted the potential role of PLD3 variants in Aβ pathology.

  11. Pain in Alzheimer's disease: A study of behavior and neural correlates

    NASA Astrophysics Data System (ADS)

    Beach, Paul Anthony

    Alzheimer's disease (AD) is a devastating neurodegenerative disease characterized by insidious and progressive impairment of cognition, emotion, and memory. Though pain in patients with AD is a major medical concern it is under diagnosed and under treated in patients, compared to cognitively healthy elderly. Further complicating matters, subjective self-report of pain by becomes increasingly compromised with disease progression; this often leaves clinicians and caregivers no choice but to rely on discerning pain from behavior alone. Patients also report pain at a lower frequency and intensity than healthy seniors (HS). These findings, coupled with recognition that AD pathology affects many pain processing brain regions, have prompted examination of whether AD alters pain perception. While there is evidence that AD actually predisposes heightened perception of pain, several issues remain: experimental work is limited to a handful of studies, whose results have been inconsistent; few examinations of pain in AD have included patients with advanced disease; the neural mechanism underlying altered pain in AD is not clear. I addressed these gaps in the literature by examining subjective, behavioral, and autonomic pain responses in 33 HS and 38 patients with varying severities of AD. A subset of these subjects (24 HS and 20 AD) were scanned, using fMRI. I then determined how the functional connectivity of various resting-state networks (RSNs) were associated with measured pain responses. I found that AD patients rated low-level stimuli as more painful than HS. Also, patients, regardless of severity, showed greater degrees of pain behaviors than HS - both with respect to global behaviors as measured by a clinical pain scale and facial responses as measured by an experimental tool. In contrast, autonomic responses were blunted with advancing AD. Altered pain responses in AD were associated with altered function of RSNs involved in attention and internal mentation, affect

  12. Sleep/wake problems in Parkinson's disease: pathophysiology and clinicopathologic correlations.

    PubMed

    Ondo, William G

    2014-08-01

    In his initial description of shaking palsy, James Parkinson first noted that sleep became disturbed with advancing paralysis agitans. More recent studies have confirmed that the majority of patients with Parkinson's disease (PD) suffer from some sleep disturbances. This can manifest as difficulty in falling or staying asleep, fractionated sleep, specific parasomnias, and daytime sleepiness. In this article, we will explore the pathophysiology of these varied sleep disorders. In most cases, however, the definitive etiology is debated, and phenotypes are often felt to be multifactorial. Some of these may be associated with dopaminergic dysfunction, some presumed to arise from varied non-dopaminergic PD pathology, and some from PD treatments. PMID:24858728

  13. Visual dysfunction and its correlation with retinal changes in patients with Parkinson's disease: an observational cross-sectional study

    PubMed Central

    Polo, V; Satue, M; Rodrigo, M J; Otin, S; Alarcia, R; Bambo, M P; Fuertes, M I; Larrosa, J M; Pablo, L E; Garcia-Martin, E

    2016-01-01

    Objectives To evaluate visual dysfunction and its correlation with structural changes in the retina in patients with Parkinson's disease (PD). Methods Patients with PD (n=37) and controls (n=37) were included in an observational cross-sectional study, and underwent visual acuity (VA), colour vision (using the Farnsworth and Lanthony desaturated D15 colour tests) and contrast sensitivity vision (CSV; using the Pelli-Robson chart and CSV 1000E test) evaluation to measure visual dysfunction. Structural measurements of the retinal nerve fibre layer (RNFL), and macular and ganglion cell layer (GCL) thicknesses, were obtained using spectral domain optical coherence tomography (SD-OCT). Comparison of obtained data, and correlation analysis between functional and structural results were performed. Results VA (in all different contrast levels) and all CSV spatial frequencies were significantly worse in patients with PD than in controls. Colour vision was significantly affected based on the Lanthony colour test. Significant GCL loss was observed in the minimum GCL+inner plexiform layer. A clear tendency towards a reduction in several macular sectors (central, outer inferior, outer temporal and superior (inner and outer)) and in the temporal quadrant of the RNFL thickness was observed, although the difference was not significant. CSV was the functional parameter most strongly correlated with structural measurements in PD. Colour vision was associated with most GCL measurements. Macular thickness was strongly correlated with macular volume and functional parameters (r>0.70, p<0.05). Conclusions Patients with PD had visual dysfunction that correlated with structural changes evaluated by SD-OCT. GCL measurements may be reliable indicators of visual impairment in patients with PD. PMID:27154474

  14. Validation of a short disease specific quality of life scale for hemifacial spasm: correlation with SF-36

    PubMed Central

    Tan, E; Fook-Chong, S; Lum, S; Thumboo, J

    2005-01-01

    Background: A short, practical, and validated quality of life (QoL) scale for hemifacial spasm (HFS) is not currently available. Objectives: To examine the reliability and validity of a short self-rating scale (HFS-7) by comparing HFS patients with healthy controls. We also evaluated the correlation of HFS-7 with the physical and mental domains of SF-36, a generic QoL scale. Methods: Seven self-rating items (HFS-7) were administered to HFS patients and healthy controls. In addition, HFS patients answered the SF-36 questionnaire. The validity and reliability of HFS-7 were analysed and correlation between HFS-7 and SF-36 examined. Results: A total of 178 subjects were enrolled in the study, including 85 HFS patients with mean age of 54.8 (SD 11.0) years, of whom 52 (61.2%) were women, and 93 controls with mean age of 51.4 (SD 10.0) years, of whom 59 (63.4%) were women. The test-retest intraclass correlation coefficient for the seven items was between 0.75 and 0.90 and Cronbach's coefficient of reliability for the HFS-7 scale was 0.88. Every item in HFS-7 discriminated between disease and controls (p<0.0001). The HFS-7 summary index correlated with the SF-36 summary score (Spearman's correlation r = –0.28, p = 0.009), in particular the mental health summary score (r = –0.416, p<0.0001) and the emotional domain (r = –0.466, p<0.00001). Conclusion: HFS-7 could prove useful as a simple clinical tool to assess and monitor QoL measures in HFS patients. PMID:16291898

  15. MRI Correlates of Parkinson's Disease Progression: A Voxel Based Morphometry Study

    PubMed Central

    Fioravanti, Valentina; Benuzzi, Francesca; Codeluppi, Luca; Contardi, Sara; Cavallieri, Francesco; Nichelli, Paolo; Valzania, Franco

    2015-01-01

    We investigated structural brain differences between a group of early-mild PD patients at different phases of the disease and healthy subjects using voxel-based morphometry (VBM). 20 mild PD patients compared to 15 healthy at baseline and after 2 years of follow-up. VBM is a fully automated technique, which allows the identification of regional differences in the gray matter enabling an objective analysis of the whole brain between groups of subjects. With respect to controls, PD patients exhibited decreased GM volumes in right putamen and right parietal cortex. After 2 years of disease, the same patients confirmed GM loss in the putamen and parietal cortex; a significant difference was also observed in the area of pedunculopontine nucleus (PPN) and in the mesencephalic locomotor region (MLR). PD is associated with brain morphological changes in cortical and subcortical structures. The first regions to be affected in PD seem to be the parietal cortex and the putamen. A third structure that undergoes atrophy is the part of the inferior-posterior midbrain, attributable to the PPN and MLR. Our findings provide new insight into the brain involvement in PD and could contribute to a better understanding of the sequence of events occurring in these patients. PMID:25628916

  16. Prevalence and correlates of coronary heart disease: first population-based study in Lebanon

    PubMed Central

    Zeidan, Rouba Karen; Farah, Rita; Chahine, Mirna N; Asmar, Roland; Hosseini, Hassan; Salameh, Pascale; Pathak, Atul

    2016-01-01

    Background Lebanon is experiencing a growing epidemic of coronary heart diseases (CHDs), as most low- and middle-income countries currently are. However, this growth can be attenuated if effective preventive strategies are adopted. Purpose To provide the first national population-based prevalence of CHD and to describe the profile of Lebanese adults with prevalent CHD. Methods We carried out a cross-sectional study using a multistage cluster sample across Lebanon. We interviewed residents aged 40 years and older using a questionnaire that captured the presence of CHDs and their risk factors (RFs). Results Our study showed that 13.4% of the Lebanese population aged ≥40 years suffer from a prevalent CHD. CHD seemed to appear more prematurely than in developed countries, and males seemed to be more subject to CHD than females until a certain age. CHD was associated with older age, male sex, a lower economic situation, hypercholesterolemia, hypertension, having a family history of premature cardiovascular diseases, and suffering from diabetes. However, smoking and waist circumference did not seem to have an independent effect on CHD, but rather an effect mediated by biological RFs. Conclusion This is the first nationwide endeavor conducted in Lebanon to assess the prevalence of CHD. This study also confirms the relevance of the classic RFs of CHD and their applicability to the Lebanese population, thus allowing for prevention strategies. PMID:27051290

  17. Historical Mammal Extinction on Christmas Island (Indian Ocean) Correlates with Introduced Infectious Disease

    PubMed Central

    Wyatt, Kelly B.; Campos, Paula F.; Gilbert, M. Thomas P.; Kolokotronis, Sergios-Orestis; Hynes, Wayne H.; DeSalle, Rob; Daszak, Peter; MacPhee, Ross D. E.; Greenwood, Alex D.

    2008-01-01

    It is now widely accepted that novel infectious disease can be a leading cause of serious population decline and even outright extinction in some invertebrate and vertebrate groups (e.g., amphibians). In the case of mammals, however, there are still no well-corroborated instances of such diseases having caused or significantly contributed to the complete collapse of species. A case in point is the extinction of the endemic Christmas Island rat (Rattus macleari): although it has been argued that its disappearance ca. AD 1900 may have been partly or wholly caused by a pathogenic trypanosome carried by fleas hosted on recently-introduced black rats (Rattus rattus), no decisive evidence for this scenario has ever been adduced. Using ancient DNA methods on samples from museum specimens of these rodents collected during the extinction window (AD 1888–1908), we were able to resolve unambiguously sequence evidence of murid trypanosomes in both endemic and invasive rats. Importantly, endemic rats collected prior to the introduction of black rats were devoid of trypanosome signal. Hybridization between endemic and black rats was also previously hypothesized, but we found no evidence of this in examined specimens, and conclude that hybridization cannot account for the disappearance of the endemic species. This is the first molecular evidence for a pathogen emerging in a naïve mammal species immediately prior to its final collapse. PMID:18985148

  18. Neural Correlates of Spatial Navigation Changes in Mild Cognitive Impairment and Alzheimer’s Disease

    PubMed Central

    Vlček, Kamil; Laczó, Jan

    2014-01-01

    Although the memory impairment is a hallmark of Alzheimer’s disease (AD), AD has also been characterized by spatial disorientation, which is present from its early stages. Spatial disorientation in AD manifests itself in getting lost in familiar and unfamiliar places and have been characterized more specifically using spatial navigation tests in both real space and virtual environments as an impairment in multiple spatial abilities, including allocentric and egocentric navigation strategies, visuo-spatial perception, or selection of relevant information for successful navigation. Patients suffering mild cognitive impairment (MCI), who are at a high risk of development of dementia, show impairment in a subset of these abilities, mainly connected with allocentric and egocentric processing. While spatial disorientation in typical AD patients probably reflects neurodegenerative changes in medial and posterior temporal, parietal, and frontal lobes, and retrosplenial cortex, the impairment of spatial navigation in MCI seem to be connected mainly with the medial temporal and also parietal brain changes. In this review, we will summarize the signs of brain disease in most MCI and AD patients showing in various tasks of spatial memory and navigation. PMID:24672452

  19. Nuclear medicine and ultrasound; correlation in diagnosis of disease of liver and biliary tract.

    PubMed

    Lomonaco, A; Kline, P; Halpern, S; Leopold, G

    1975-10-01

    Even though the radiocolloid scan is nonspecific it will be approximately 70%-80% accurate in predicting the presence or absence of liver disease and somewhat less accurate than that in making statements as to the specific type of disease. This compares well with other modalities. The ability of nuclear medicine techniques to provide a correct diagnosis is improved when additional isotopic techniques such as hepatic blood flow studies and 131I-rose bengal and 67Ga scanning are performed. Ultrasound scanning is also non specific. To date, the major application of ultrasound in the study of the liver has been in deciphering puzzling contour abnormalities seen on nuclear medicine scans and in demonstrating fluid-filled abnormalities. Its usefulness in diffuse and solid focal lesions has been less dramatic. More recently, however, the development of gray scale has necessitated a reevaluation of the technique. Gray scale demonstrates a large number of intrahepatic interfaces that were previously invisible, and it has already been shown to demonstrate focal disorders such as metastasis more easily than the nongray-scale method. It can also demonstrate dilated biliary radicals, the gallbladder, and gallstones. In addition, while routinely studying the liver one can evaluate diaphragmatic motion and various retroperitoneal structures such as the pancreas, lymph nodes, and abdominal vascular structures.

  20. Prevalence and Correlates of Peripheral Arterial Disease in Nigerians with Type 2 Diabetes

    PubMed Central

    Ikem, R. T.; Oluwadiya, K. S.; Bolarinwa, R. A.; Adebayo, O. J.

    2016-01-01

    Background. Peripheral arterial disease (PAD) is a major risk factor for nonhealing foot ulcers in people with diabetes. A number of traditional risk factors have been reported to be associated with PAD; however, there may be a need to consider nontraditional risk factors especially in some vulnerable populations. This study determined the prevalence and risk factors associated with PAD in diabetics. Methods. One hundred and fifty type 2 diabetics and an equal number of age- and sex-matched apparently healthy controls were studied. Assessment of PAD was made using history, palpation of lower limb vessels, and measurement of ankle-brachial index (ABI). Statistically significant differences between categorical and continuous variables were determined using Chi square (χ2) and Student t-tests, respectively. Regression analysis was done to determine the associated risk factors for PAD. Results. Prevalence of PAD using ABI was 22.0% and 8.0% among diabetic and nondiabetic populations, respectively. Peripheral arterial disease was associated with age, male gender, waist circumference, and high-sensitivity C-reactive protein. Conclusion. This study highlights the high prevalence of PAD in people with type 2 diabetes mellitus and in apparently healthy controls; age, male gender, abdominal obesity, and high hs-CRP values were the associated risk factors. PMID:27800544

  1. Historical mammal extinction on Christmas Island (Indian Ocean) correlates with introduced infectious disease.

    PubMed

    Wyatt, Kelly B; Campos, Paula F; Gilbert, M Thomas P; Kolokotronis, Sergios-Orestis; Hynes, Wayne H; DeSalle, Rob; Ball, Stanley J; Daszak, Peter; MacPhee, Ross D E; Greenwood, Alex D

    2008-01-01

    It is now widely accepted that novel infectious disease can be a leading cause of serious population decline and even outright extinction in some invertebrate and vertebrate groups (e.g., amphibians). In the case of mammals, however, there are still no well-corroborated instances of such diseases having caused or significantly contributed to the complete collapse of species. A case in point is the extinction of the endemic Christmas Island rat (Rattus macleari): although it has been argued that its disappearance ca. AD 1900 may have been partly or wholly caused by a pathogenic trypanosome carried by fleas hosted on recently-introduced black rats (Rattus rattus), no decisive evidence for this scenario has ever been adduced. Using ancient DNA methods on samples from museum specimens of these rodents collected during the extinction window (AD 1888-1908), we were able to resolve unambiguously sequence evidence of murid trypanosomes in both endemic and invasive rats. Importantly, endemic rats collected prior to the introduction of black rats were devoid of trypanosome signal. Hybridization between endemic and black rats was also previously hypothesized, but we found no evidence of this in examined specimens, and conclude that hybridization cannot account for the disappearance of the endemic species. This is the first molecular evidence for a pathogen emerging in a naïve mammal species immediately prior to its final collapse.

  2. 13C-methacetin breath test correlates with clinical indices of liver disease severity in patients with primary biliary cirrhosis.

    PubMed

    Kochel-Jankowska, A; Hartleb, M; Jonderko, K; Kaminska, M; Kasicka-Jonderko, A

    2013-02-01

    This prospective study intended to ascertain if cytochrome P450 dependent liver function is affected in early and late histological stages of primary biliary cirrhosis (PBC). The study included 32 female PBC patients (mean age 55.4 years, range 33-70) and 16 aged-matched healthy women (mean age 52.6 years, range 38-65). In every subject a 13(C)-methacetin breath test (13(C)-MBT) was applied, and the results were related to histological Ludwig's staging system and several indices of liver disease severity comprising the MAYO-1, MAYO-2, MELD, and Child-Pugh score. The 13(C)-MBT differentiated healthy controls from the patients with Ludwig IV and Ludwig III histopathological stages of PBC. The most significant relationships (i.e. explaining >50% of the variance) were found between measurements of the momentary breath 13(C) elimination from 6 to 18 minutes as well as the 15-min or 30-min cumulative elimination and the MAYO-1 or MAYO-2 scores. The breath test poorly correlated with histopathological features of PBC, however, it accurately discriminated cirrhotic from non-cirrhotic patients (momentary breath 13(C) elimination at 40 min, AUROC 0,958). In conclusion, 13(C)-MBT correlates with clinical scoring systems, especially those specifically designed for PBC (Mayo model) and accurately recognizes the disease at the stage of cirrhosis up to 40 minutes of the test duration.

  3. The Expression of NOX4 in Smooth Muscles of Small Airway Correlates with the Disease Severity of COPD

    PubMed Central

    2016-01-01

    Airway smooth muscle (ASM) remodeling is a hallmark in chronic obstructive pulmonary disease (COPD), and nicotinamide-adenine dinucleotide phosphate (NADPH) oxidases (NOXs) produced reactive oxygen species (ROS) play a crucial role in COPD pathogenesis. In the present study, the expression of NOX4 and its correlation with the ASM hypertrophy/hyperplasia, clinical pulmonary functions, and the expression of transforming growth factor β (TGF-β) in the ASM of COPD small airways were investigated by semiquantitative morphological and/or immunohistochemistry staining methods. The results showed that an elevated expression of NOX4 and TGF-β, along with an increased volume of ASM mass, was found in the ASM of small airways in COPD patients. The abundance of NOX4 protein in the ASM was increased with disease severity and inversely correlated with the pulmonary functions in COPD patients. In addition, the expression of NOX4 and ASM marker α-SMA was colocalized, and the increased NOX4 expression was found to accompany an upregulated expression of TGF-β in the ASM of small airways of COPD lung. These results indicate that NOX4 may be a key regulator in ASM remodeling of small airway, in part through a mechanism interacting with TGF-β signaling in the pathogenesis of COPD, which warrants further investigation. PMID:27656649

  4. Serum Inflammatory Cytokine Levels Correlate with Hand-Foot-Mouth Disease Severity: A Nested Serial Case-Control Study

    PubMed Central

    Sun, Wumei; Sui, Meili; Zhang, Rongguang; Wang, Xinhong; Wang, Fang; Zhang, Weidong; Xi, Yuanlin; Fan, Qingtang

    2014-01-01

    Background Hand-food-mouth disease (HFMD) cases can be fatal. These cases develop rapidly, and it is important to predict the severity of HFMD from mild to fatal and to identify risk factors for mild HFMD. The objective of this study was to correlate the levels of serum inflammatory cytokines with HFMD severity. Methods This study was designed as a nested serial case-control study. The data collected included general information, clinical symptoms and signs, laboratory findings and serum cytokine levels. Results The levels of IL-4, IL-6, IL-10, TNF-α and IFN-γ in patients with severe HFMD were significantly higher than in mild patients during the 2nd to 5th day after disease onset. The levels of IL-4, IL-6, IL-10 and IFN-γ increased from the 2nd day to the 4th day and later decreased. The levels of TNF-α were high on the first two days and subsequently decreased. The changes of IL-10, TNF-α and IFN-γ in the controls were similar for all cases. The levels of IL-4, IL-6 and IL-17 in the controls were not significantly different with the progression of HFMD. Conclusions Our findings indicate that the IL-4, IL-6, IL-10, TNF-α and IFN-γ levels correlate with HFMD severity. PMID:25391156

  5. The Expression of NOX4 in Smooth Muscles of Small Airway Correlates with the Disease Severity of COPD

    PubMed Central

    2016-01-01

    Airway smooth muscle (ASM) remodeling is a hallmark in chronic obstructive pulmonary disease (COPD), and nicotinamide-adenine dinucleotide phosphate (NADPH) oxidases (NOXs) produced reactive oxygen species (ROS) play a crucial role in COPD pathogenesis. In the present study, the expression of NOX4 and its correlation with the ASM hypertrophy/hyperplasia, clinical pulmonary functions, and the expression of transforming growth factor β (TGF-β) in the ASM of COPD small airways were investigated by semiquantitative morphological and/or immunohistochemistry staining methods. The results showed that an elevated expression of NOX4 and TGF-β, along with an increased volume of ASM mass, was found in the ASM of small airways in COPD patients. The abundance of NOX4 protein in the ASM was increased with disease severity and inversely correlated with the pulmonary functions in COPD patients. In addition, the expression of NOX4 and ASM marker α-SMA was colocalized, and the increased NOX4 expression was found to accompany an upregulated expression of TGF-β in the ASM of small airways of COPD lung. These results indicate that NOX4 may be a key regulator in ASM remodeling of small airway, in part through a mechanism interacting with TGF-β signaling in the pathogenesis of COPD, which warrants further investigation.

  6. Correlation of serum hepcidin levels with disease progression in hepatitis B virus-related disease assessed by nanopore film based assay

    PubMed Central

    Wang, Jing; Dong, Ailian; Liu, Gang; Anderson, Gregory J.; Hu, Tony Y.; Shi, Jian; Hu, Yulin; Nie, Guangjun

    2016-01-01

    Chronic hepatitis B virus (HBV) infection often develop into cirrhosis, and both are major risk factors of hepatocellular carcinoma. However, effective approaches for the monitoring of HBV-related disease progress are still in need. Increased iron storage has an important role in HBV-related diseases. Hepcidin is a key regulator of iron homeostasis whose expression changes are often indicative of abnormal iron metabolism. There are few reports of hepcidin levels in patients with HBV infections, and the available results are inconsistent. In this study, using a recently validated nanopore silica film based method, we measured serum hepcidin levels in 46 HBV-related patients and 20 healthy controls. Patients were divided into three groups: chronic hepatitis B without cirrhosis; HBV-related cirrhosis; and HBV-related cirrhosis with hepatocellular carcinoma. Compared to healthy controls, the mean serum hepcidin level was significantly higher in CHB patients without cirrhosis, and in those with hepatocellular carcinoma, but not in those with cirrhosis. Iron-loading, viral infection and liver dysfunction are determined to be the major regulators of hepcidin in these patients. These observations suggest correlations between serum hepcidin and progression of chronic HBV infection, and may shed a new light on the development of biomarkers for HBV-related disease surveillance. PMID:27694815

  7. Plasma and Synovial Fluid TrxR Levels are Correlated With Disease Risk and Severity in Patients With Rheumatoid Arthritis

    PubMed Central

    Xie, Zhijun; Sun, Jing; Li, Haichang; Shao, Tiejuan; Wang, Dawei; Zheng, Qi; Wen, Chengping

    2016-01-01

    Abstract This study was designed and performed to establish the relationship between plasma and synovial fluid (SF) levels of thioredoxin reductase (TrxR) and disease activity in Chinese patients with rheumatoid arthritis (RA). This study consisted of a total of 224 patients diagnosed with RA, 224 age and sex-matched healthy controls, and 156 patient controls. The disease activity of RA patients was calculated as diseases activity score that include 28-joint counts (DAS 28), which was divided into low-diseases activity (LDA) and high-diseases activity (HDA) groups. Increased plasma TrxR was detected in patients with RA than healthy controls (P < 0.0001). With an area under the curve (AUC) of 0.874, plasma TrxR showed a evidently greater discriminatory ability than C-reactive protein (CRP; AUC, 0.815), antistreptolysin-O (ASO; AUC, 0.631), rheumatoid factor (RF, AUC, 0.793), and erythrocyte sedimentation rate (ESR, AUC, 0.789) in diagnosing RA. RA patients with HDA had significantly elevated TrxR levels in plasma and SF than did those with LDA (P < 0.0001). With an AUC of 0.874, plasma TrxR levels as an indicator for screening of HDA showed a significantly greater discriminatory ability than CRP (AUC, 0.690), ASO (AUC, 0.597), RF (AUC, 0.657), and ESR (AUC, 0.603). Similarly, SF TrxR levels as an indicator for screening of HDA also showed a significantly greater discriminatory ability as compared with above biomarkers. TrxR levels in plasma and SF were positively correlated with the severity of RA. TrxR levels may therefore serve as a new biomarker in addition of the traditional biomarkers for assessing the risk and severity of RA. Further analysis of TrxR release machinery may give us a new understanding of pathogenesis of RA. PMID:26871773

  8. Anxiety and Depression Are Better Correlates of Parkinson's Disease Quality of Life Than Apathy.

    PubMed

    Jones, Jacob D; Butterfield, London C; Song, Woojin; Lafo, Jacob; Mangal, Paul; Okun, Michael S; Bowers, Dawn

    2015-01-01

    Due to controversy regarding the influence of apathy on quality of life (QoL), the authors examined the independent influence of apathy, depression, and trait anxiety in a nondemented sample of patients with Parkinson disease (PD). Participants (N=107) completed standard self-report measures of QoL and mood/motivation. Analyses investigated the contribution of these measures and empirically derived factor scores on QoL. QoL was predicted by trait anxiety, dysphoria, and decreased interest, with no independent contribution of apathy. Different patterns emerged with respect to domain-specific QoL, with trait anxiety being the strongest predictor across most domains. Anxiety was most widely related to QoL in PD, with minimal contribution of apathy. Future studies should examine different roles of PD mood/motivation symptoms on caregiver QoL.

  9. Common Variants in PLD3 and Correlation to Amyloid-Related Phenotypes in Alzheimer's Disease.

    PubMed

    Wang, Chong; Tan, Lan; Wang, Hui-Fu; Yu, Wan-Jiang; Liu, Ying; Jiang, Teng; Tan, Meng-Shan; Hao, Xiao-Ke; Zhang, Dao-Qiang; Yu, Jin-Tai

    2015-01-01

    The phospholipase D3 (PLD3) gene has shown association with Alzheimer's disease (AD). However, the role of PLD3 common variants in amyloid-β (Aβ) pathology remains unclear. We examined the association of thirteen common single nucleotide polymorphisms (SNPs) with cerebrospinal fluid (CSF) Aβ(1- 42) levels and florbetapir retention on florbetapir 18F amyloid positron emission tomography (AV45-PET) in a large population. We found that one SNP (rs11667768) was significantly associated with CSF Aβ(1- 42) levels in the normal cognition group. We did not observe an association of any SNP with florbetapir retention. Our study predicted the potential role of PLD3 variants in Aβ pathology. PMID:26402410

  10. Regional serotonin transporter availability and depression are correlated in Wilson's disease.

    PubMed

    Hesse, S; Barthel, H; Hermann, W; Murai, T; Kluge, R; Wagner, A; Sabri, O; Eggers, B

    2003-08-01

    In patients with Wilson's disease (WD), depression is a frequent psychiatric symptom. In vivo neuroimaging studies suggest that depression and other neuropsychiatric disorders are associated with central serotonergic deficits. However, in vivo measurements of serotonergic neurotransmission have not until now been performed in patients with this copper deposition disorder. The present prospective study revealed that depressive symptomatology is related to an alteration of presynaptic serotonin transporters (SERT) availability as measured by [123I]-2beta-carbomethoxy-3beta-(iodophenyl)tropane ([123I]beta-CIT) and high-resolution single-photon emission computed tomography (SPECT). SERT imaging with [123I]beta-CIT-SPECT could therefore become a useful tool for diagnosis and therapy monitoring in depressed WD patients. PMID:12898347

  11. Clinicopathological correlates of chronic kidney disease of unknown etiology in Sri Lanka

    PubMed Central

    Selvarajah, M.; Weeratunga, P.; Sivayoganthan, S.; Rathnatunga, N.; Rajapakse, S.

    2016-01-01

    Chronic kidney disease of unknown etiology (CKDu) is a major healthcare issue in Sri Lanka. This study included 125 consecutive patients with a diagnosis of CKDu undergoing renal biopsy at one hospital from 2008 to 2012. Associations between renal outcome parameters, epidemiological data, and histopathological findings were examined and regression models constructed based on univariate associations with outcome variables as serum creatinine >1.2 and stage of CKD >3. The mean patient age was 46.21 years (standard deviation = 11.64). A marked male predominance was noted. A positive family history of CKD was seen in 35.8%. Prominent histopathological features were glomerular sclerosis (94.8%), interstitial infiltration (76%) with lymphocytic infiltration, interstitial fibrosis (71.2%), and tubular atrophy (70.4%). Importantly, significant histological changes were seen in patients with early CKDu. For CKD stage >3 independent associations were: interstitial fibrosis [P = 0.005; odds ratio (OR) =0.153] and interstitial infiltrate (P = 0.030; OR = 0.2440. For serum creatinine >1.2, independent predictors were >50% glomerular sclerosis (P = 0.041; OR = 0.92), tubular atrophy (P = 0.034; OR = 0.171, and more than 40 residential life years (P = 0.009; OR = 9.229). Chronic tubulointerstitial nephritis (TIN) appears to be the predominant histopathological finding in patients with CKDu, with significant renal pathology established early on in the course of the disease. Interstitial infiltration appears to be an independent association of advancing CKD, CKDu, histopathology, histology, and TIN.

  12. Correlates of disease-specific knowledge in Chinese patients with COPD

    PubMed Central

    Wong, Carlos KH; Yu, WC

    2016-01-01

    Background This study aimed to determine the associations of various sociodemographic factors with the level of disease-specific knowledge among Hong Kong Chinese patients with COPD. Methods A cross-sectional survey of 100 Chinese adults with COPD recruited from outpatient clinics was conducted from September 2009 to September 2010. Data on the knowledge specific to COPD and patients’ sociodemographics were collected from face-to-face interviews. Primary outcome of disease-specific knowledge was measured using 65-item Bristol COPD Knowledge Questionnaire (BCKQ), summing up the 65 items as the BCKQ overall score. Associations of sociodemographic factors with the BCKQ overall score were evaluated using the linear regression model. Results The mean BCKQ overall score of our patients was 41.01 (SD: 10.64). The knowledge in topics of “Smoking” and “Phlegm” achieved the first (3.97, SD: 0.82) and second (3.91, SD: 1.17) highest mean scores, respectively, while the topic of “Oral steroids” returned the lowest mean score of 1.89 (SD: 1.64). The BCKQ overall score progressively declined (P<0.001) with increase in education level, with the highest BCKQ overall score of 46.71 at no formal education among all subgroups. Compared to nondrinkers, current drinkers were associated with lower total BCKQ score. Conclusion We found that among COPD patients in outpatient clinics, impairments in the level of COPD knowledge were evident in patients who were current drinkers or had higher level of education. PMID:27695309

  13. Correlates of disease-specific knowledge in Chinese patients with COPD

    PubMed Central

    Wong, Carlos KH; Yu, WC

    2016-01-01

    Background This study aimed to determine the associations of various sociodemographic factors with the level of disease-specific knowledge among Hong Kong Chinese patients with COPD. Methods A cross-sectional survey of 100 Chinese adults with COPD recruited from outpatient clinics was conducted from September 2009 to September 2010. Data on the knowledge specific to COPD and patients’ sociodemographics were collected from face-to-face interviews. Primary outcome of disease-specific knowledge was measured using 65-item Bristol COPD Knowledge Questionnaire (BCKQ), summing up the 65 items as the BCKQ overall score. Associations of sociodemographic factors with the BCKQ overall score were evaluated using the linear regression model. Results The mean BCKQ overall score of our patients was 41.01 (SD: 10.64). The knowledge in topics of “Smoking” and “Phlegm” achieved the first (3.97, SD: 0.82) and second (3.91, SD: 1.17) highest mean scores, respectively, while the topic of “Oral steroids” returned the lowest mean score of 1.89 (SD: 1.64). The BCKQ overall score progressively declined (P<0.001) with increase in education level, with the highest BCKQ overall score of 46.71 at no formal education among all subgroups. Compared to nondrinkers, current drinkers were associated with lower total BCKQ score. Conclusion We found that among COPD patients in outpatient clinics, impairments in the level of COPD knowledge were evident in patients who were current drinkers or had higher level of education.

  14. Physiopathology of Chagas' heart disease: correlations between clinical and experimental findings*

    PubMed Central

    Anselmi, Alfonso; Moleiro, Federico

    1971-01-01

    In penetrating the heart and developing in it, Trypanosoma cruzi produces an immunoallergic reaction that leads to changes in the histological structure of the myocardium; these changes alter the fundamental properties of the heart, causing fundamental dynamic disorders and morphological changes in the organ. In Chagas' cardiomyopathy, the velocity of impulse propagation diminishes in the auricular and ventricular musculature, altering the activation mechanism, this being shown by changes in the P-wave and in ventricular focal blocks. The functional refractory period (FRP) is shortened in the auricular and ventricular tissue and constitutes, together with changes in conductivity, the physiopathological basis that explains the circus movement—the fundamental factor of the arrhythmias of this stage of the disease. Localization of the inflammation in the A-V conduction system increases the duration of the FRP, producing all types of A-V block. The oedema and the cellular interstitial infiltration seen during this acute phase reduce the distensibility of the fibres; this, in turn, limits their contractility, producing a decrease in systolic volume and an increase in the final diastolic pressure in the chambers of the heart—fundamental factors in reducing kinesia and in increasing the heart's volume. In the chronic phase, destruction of the contractile tissue and fibroblastic proliferation bring into play compensatory mechanisms that maintain the strength of cardiac contractions; the elongation of the fibres and the nature of the dynamic pressure—volume curves explain the dilatation of the chambers of the heart and the dynamic changes seen in this phase of the disease. PMID:5003721

  15. Studies on the correlation with olfactory dysfunction in a transgenic mice model of Alzheimer's disease

    NASA Astrophysics Data System (ADS)

    Rasheed, Ameer; Lee, Ji Hye; Suh, Yoo-Hun; Moon, Cheil

    2013-05-01

    Alzheimer's disease (AD) is a progressively debilitating neurodegenerative disorder characterized by the presence of proteinaceous deposits in the brain. AD often results in olfactory dysfunction and impaired olfactory perceptual acuity may be a potential biomarker for early diagnosis of AD. Until recently, there is no Alzheimer's nanoscope or any other high-end microscope developed to be capable of seeing buried feature of AD clearly. Modern neuroimaging techniques are more effective only after the occurrence of cognitive impairment. Therefore, early detection of Alzheimer's disease is critical in developing effective treatment of AD. H and E (Haematoxyline and Eosin) staining is performed for examining gross morphological changes, while TUNEL (transferase (TdT)-mediated dUTP nick end labeling) staining for monitoring neuronal death in the olfactory epithelium (OE). Furthermore, immunohistochemistry and western blot are performed to examine β-amyloid protein expression. AD model animals were Tg2576 (transgenic mice that overexpress a mutated form of the Aβ precursor protein), and 6 month (before onset of AD symptoms) and 14 month (after onset of AD symptoms) old WT (wild type) and transgenic mice were compared in their olfactory system. We found that in OE of Tg2576 mice, thickness and total number of cells were decreased, while the numbers of TUNEL-positive neurons, caspase-3 activation were significantly increased compared with age-matched WT. Our results demonstrate that the olfactory system may get deteriorated before onset of AD symptoms. Our findings imply that an olfactory biopsy could be served as an early and relatively simple diagnostic tool for potential AD patients.

  16. Correlation between Patient-Reported Symptoms and Ankle-Brachial Index after Revascularization for Peripheral Arterial Disease.

    PubMed

    Je, Hyung Gon; Kim, Bo Hyun; Cho, Kyoung Im; Jang, Jae Sik; Park, Yong Hyun; Spertus, John

    2015-05-18

    Improvement in quality of life (QoL) is a primary treatment goal for patients with peripheral arterial disease (PAD). The current study aimed to quantify improvement in the health status of PAD patients following peripheral revascularization using the peripheral artery questionnaire (PAQ) and ankle-brachial index (ABI), and to evaluate possible correlation between the two methods. The PAQ and ABI were assessed in 149 symptomatic PAD patients before, and three months after peripheral revascularization. Mean PAQ summary scores improved significantly three months after revascularization (+49.3 ± 15 points, p < 0.001). PAQ scores relating to patient symptoms showed the largest improvement following revascularization. The smallest increases were seen in reported treatment satisfaction (all p's < 0.001). As expected the ABI of treated limbs showed significant improvement post-revascularization (p < 0.001). ABI after revascularization correlated with patient-reported changes in the physical function and QoL domains of the PAQ. Twenty-two percent of PAD patients were identified as having a poor response to revascularization (increase in ABI < 0.15). Interestingly, poor responders reported improvement in symptoms on the PAQ, although this was less marked than in patients with an increase in ABI > 0.15 following revascularization. In conclusion, data from the current study suggest a significant correlation between improvement in patient-reported outcomes assessed by PAQ and ABI in symptomatic PAD patients undergoing peripheral revascularization.

  17. The endolymphatic sac in patients with Ménière's disease: correlation between the MRI and the surgical findings.

    PubMed

    Kobayashi, M; Fukaya, T; Noda, M

    2000-10-01

    The endolymphatic sac (ES) is thought to have close relations to pathogenesis of endolymphatic hydrops. Here is reported a retrospective study of 41 patients (42 ears) with Meniere's disease who underwent MRI prior to endolymphatic sac surgery. Based on proton-density weighted image (PDI) and T2-weighted image (T2), the ES including an endolymphatic duct (ED) were estimated whether it was detectable or not. Fourteen ESs were detected on both images (Group A), 14 ESs were detected only on PDI (Group B), and the remaining 14 ESs were not detected on either image (Group D). The actual shape of the sac, obtained from surgical findings, was classified into three (normoplastic, atrophic, invisible). Seventeen ears showed normoplastic ESs and 14 showed atrophic ESs. ES was not detected in 11 ears during surgery, and these findings were compared with image classification. From the study data, normoplastic ESs tend to be observed on both images whereas atrophic or invisible sacs were hardly observed on T2. This classification of ES on MRI was thought to correlate with surgical findings and this correlation was statistically significant (Spearman's rank correlation coefficient. r(s) = 0.58, p < 0.01).

  18. Efficacy of Percutaneous Epidural Neuroplasty Does Not Correlate with Dural Sac Cross-Sectional Area in Single Level Disc Disease

    PubMed Central

    Ji, Gyu Yeul; Oh, Chang Hyun; Moon, Bongju; Choi, Seung Hyun; Yoon, Young Sul; Kim, Keung Nyun

    2015-01-01

    Purpose Percutaneous epidural neuroplasty (PEN) is a minimally invasive treatment. The efficacy of PEN has been relatively well investigated; however, the relationship between the clinical effectiveness of PEN and the severity of spinal canal stenosis by disc material has not yet been established. The purpose of this study was to compare clinical outcomes of PEN according to the dural sac cross-sectional area in single level disc disease. Materials and Methods This study included 363 patients with back pain from single level disc disease with and without radiculopathy. Patients were categorized into groups according to spinal canal compromise by disc material: Category 1, less or more than 50%; and Category 2, three subgroups with lesser than a third, between a third and two thirds, and more than two thirds. Clinical outcomes were assessed according to the Visual Analog Scale (VAS) score for back pain and leg pain and Odom's criteria at 1, 3, 6, 12, and 24 months after treatment. Results The demographic data showed no difference between groups according to spinal canal compromise by disc material except age (older age correlated with more spinal canal compromise). The dural sac cross-sectional area did not correlate with the VAS scores for back and leg pain after PEN in single level disc disease in Groups 1 and 2. Odom's criteria after PEN were also not different according to dural sac cross-sectional area by disc material. Conclusion PEN is an effective procedure in treating single level lumbar disc herniation without affecting dural sac cross-sectional area. PMID:25837174

  19. Susceptibility to Theiler's virus-induced demyelinating disease correlates with astrocyte class II induction and antigen presentation.

    PubMed Central

    Borrow, P; Nash, A A

    1992-01-01

    Theiler's murine encephalomyelitis virus (TMEV) is a picornavirus which induces a chronic demyelinating disease of the central nervous system (CNS) in certain susceptible mouse strains. Demyelination has been shown to result from immunopathological responses mediated by CD4+, major histocompatibility complex (MHC) class II-restricted T cells. As little or no class II is expressed in the normal mouse CNS, the ability of astrocytes to express these proteins and present antigen to T cells from TMEV-infected mice was investigated here. It is shown that astrocytes are capable of presenting TMEV to virus-specific T cells in vitro, and that this ability is dependent on prior induction of MHC class II by interferon-gamma (IFN-gamma) treatment. Unlike other viruses such as murine hepatitis virus-JHM (a coronavirus) and measles, TMEV is not capable of inducing class II on astrocytes directly. There is a correlation between the ease of class II induction on astrocytes from different mouse strains by IFN-gamma and mouse strain susceptibility to TMEV-induced demyelinating disease. These results suggest that following viral infection and initial T-cell infiltration into the CNS, class II induction on astrocytes is a key step allowing local antigen presentation and amplification of immunopathological responses within the CNS and hence the development of demyelinating disease. PMID:1628891

  20. Susceptibility to Theiler's virus-induced demyelinating disease correlates with astrocyte class II induction and antigen presentation.

    PubMed

    Borrow, P; Nash, A A

    1992-05-01

    Theiler's murine encephalomyelitis virus (TMEV) is a picornavirus which induces a chronic demyelinating disease of the central nervous system (CNS) in certain susceptible mouse strains. Demyelination has been shown to result from immunopathological responses mediated by CD4+, major histocompatibility complex (MHC) class II-restricted T cells. As little or no class II is expressed in the normal mouse CNS, the ability of astrocytes to express these proteins and present antigen to T cells from TMEV-infected mice was investigated here. It is shown that astrocytes are capable of presenting TMEV to virus-specific T cells in vitro, and that this ability is dependent on prior induction of MHC class II by interferon-gamma (IFN-gamma) treatment. Unlike other viruses such as murine hepatitis virus-JHM (a coronavirus) and measles, TMEV is not capable of inducing class II on astrocytes directly. There is a correlation between the ease of class II induction on astrocytes from different mouse strains by IFN-gamma and mouse strain susceptibility to TMEV-induced demyelinating disease. These results suggest that following viral infection and initial T-cell infiltration into the CNS, class II induction on astrocytes is a key step allowing local antigen presentation and amplification of immunopathological responses within the CNS and hence the development of demyelinating disease.

  1. Multimodal Image Analysis in Alzheimer’s Disease via Statistical Modelling of Non-local Intensity Correlations

    PubMed Central

    Lorenzi, Marco; Simpson, Ivor J.; Mendelson, Alex F.; Vos, Sjoerd B.; Cardoso, M. Jorge; Modat, Marc; Schott, Jonathan M.; Ourselin, Sebastien

    2016-01-01

    The joint analysis of brain atrophy measured with magnetic resonance imaging (MRI) and hypometabolism measured with positron emission tomography with fluorodeoxyglucose (FDG-PET) is of primary importance in developing models of pathological changes in Alzheimer’s disease (AD). Most of the current multimodal analyses in AD assume a local (spatially overlapping) relationship between MR and FDG-PET intensities. However, it is well known that atrophy and hypometabolism are prominent in different anatomical areas. The aim of this work is to describe the relationship between atrophy and hypometabolism by means of a data-driven statistical model of non-overlapping intensity correlations. For this purpose, FDG-PET and MRI signals are jointly analyzed through a computationally tractable formulation of partial least squares regression (PLSR). The PLSR model is estimated and validated on a large clinical cohort of 1049 individuals from the ADNI dataset. Results show that the proposed non-local analysis outperforms classical local approaches in terms of predictive accuracy while providing a plausible description of disease dynamics: early AD is characterised by non-overlapping temporal atrophy and temporo-parietal hypometabolism, while the later disease stages show overlapping brain atrophy and hypometabolism spread in temporal, parietal and cortical areas. PMID:27064442

  2. Multimodal Image Analysis in Alzheimer’s Disease via Statistical Modelling of Non-local Intensity Correlations

    NASA Astrophysics Data System (ADS)

    Lorenzi, Marco; Simpson, Ivor J.; Mendelson, Alex F.; Vos, Sjoerd B.; Cardoso, M. Jorge; Modat, Marc; Schott, Jonathan M.; Ourselin, Sebastien

    2016-04-01

    The joint analysis of brain atrophy measured with magnetic resonance imaging (MRI) and hypometabolism measured with positron emission tomography with fluorodeoxyglucose (FDG-PET) is of primary importance in developing models of pathological changes in Alzheimer’s disease (AD). Most of the current multimodal analyses in AD assume a local (spatially overlapping) relationship between MR and FDG-PET intensities. However, it is well known that atrophy and hypometabolism are prominent in different anatomical areas. The aim of this work is to describe the relationship between atrophy and hypometabolism by means of a data-driven statistical model of non-overlapping intensity correlations. For this purpose, FDG-PET and MRI signals are jointly analyzed through a computationally tractable formulation of partial least squares regression (PLSR). The PLSR model is estimated and validated on a large clinical cohort of 1049 individuals from the ADNI dataset. Results show that the proposed non-local analysis outperforms classical local approaches in terms of predictive accuracy while providing a plausible description of disease dynamics: early AD is characterised by non-overlapping temporal atrophy and temporo-parietal hypometabolism, while the later disease stages show overlapping brain atrophy and hypometabolism spread in temporal, parietal and cortical areas.

  3. Prominent effects and neural correlates of visual crowding in a neurodegenerative disease population.

    PubMed

    Yong, Keir X X; Shakespeare, Timothy J; Cash, Dave; Henley, Susie M D; Nicholas, Jennifer M; Ridgway, Gerard R; Golden, Hannah L; Warrington, Elizabeth K; Carton, Amelia M; Kaski, Diego; Schott, Jonathan M; Warren, Jason D; Crutch, Sebastian J

    2014-12-01

    Crowding is a breakdown in the ability to identify objects in clutter, and is a major constraint on object recognition. Crowding particularly impairs object perception in peripheral, amblyopic and possibly developing vision. Here we argue that crowding is also a critical factor limiting object perception in central vision of individuals with neurodegeneration of the occipital cortices. In the current study, individuals with posterior cortical atrophy (n=26), typical Alzheimer's disease (n=17) and healthy control subjects (n=14) completed centrally-presented tests of letter identification under six different flanking conditions (unflanked, and with letter, shape, number, same polarity and reverse polarity flankers) with two different target-flanker spacings (condensed, spaced). Patients with posterior cortical atrophy were significantly less accurate and slower to identify targets in the condensed than spaced condition even when the target letters were surrounded by flankers of a different category. Importantly, this spacing effect was observed for same, but not reverse, polarity flankers. The difference in accuracy between spaced and condensed stimuli was significantly associated with lower grey matter volume in the right collateral sulcus, in a region lying between the fusiform and lingual gyri. Detailed error analysis also revealed that similarity between the error response and the averaged target and flanker stimuli (but not individual target or flanker stimuli) was a significant predictor of error rate, more consistent with averaging than substitution accounts of crowding. Our findings suggest that crowding in posterior cortical atrophy can be regarded as a pre-attentive process that uses averaging to regularize the pathologically noisy representation of letter feature position in central vision. These results also help to clarify the cortical localization of feature integration components of crowding. More broadly, we suggest that posterior cortical atrophy

  4. Clinical significance of exercise-induced ST segment elevation. Correlative angiographic study in patients with ischaemic heart disease.

    PubMed Central

    de Feyter, P J; Majid, P A; van Eenige, M J; Wardeh, R; Wempe, F N; Roos, J P

    1981-01-01

    We have examined the relation between electrocardiographic ST elevation during treadmill exercise (greater than or equal to 1 mm, using the conventional 12 leads), the severity of coronary artery disease, and left ventricular wall motion abnormalities in 680 patients. They were divided into three groups: (1) 218 patients with clinically significant coronary artery disease, (2) 178 patients with clinically significant coronary artery disease, and (3) 284 patients with clinically significant coronary artery disease and previous myocardial infarction. ST elevation during exercise (predominantly in lead V2) was seen in two patients (1%) in group 1, three patients (2%) in group 2, and 147 patients (52%) in group 3. Coronary artery disease (number of vessels involved and severity of stenoses) was comparable in groups 2 and 3. All the patients in group 1 showed a normal left ventricular contraction pattern; 64% of the patients in group 2 showed wall motion abnormalities (predominantly hypokinesia) and 95% of group 3 (mainly akinesia, dyskinesia, or aneurysm). A strongly positive correlation was seen between the ST elevation and left ventricular dysfunction in patients belonging to group 3. The overall sensitivity and the specificity of the stress test in detecting wall motion abnormalities was 55% and 100% respectively. The sensitivity increased with deterioration in left ventricular function, reaching 81% and 90% in patients with dyskinesia and aneurysm, respectively. Maximal ST elevation (greater than or equal to 3 mm) was confined to the patients with dyskinesia or aneurysm. The incidence of ST elevation during exercise was also related to the location of previous infarction, showing a positive response in 85% of patients with anterior myocardial infarction and in only 33% with inferior myocardial infarction. We conclude that ST segment elevation during exercise in patients with previous myocardial infarction is a sensitive and a specific indicator of advanced left

  5. Neural correlates of cognitive intervention in persons at risk of developing Alzheimer’s disease

    PubMed Central

    Hosseini, S. M. Hadi; Kramer, Joel H.; Kesler, Shelli R.

    2014-01-01

    Cognitive training is an emergent approach that has begun to receive increased attention in recent years as a non-pharmacological, cost-effective intervention for Alzheimer’s disease (AD). There has been increasing behavioral evidence regarding training-related improvement in cognitive performance in early stages of AD. Although these studies provide important insight about the efficacy of cognitive training, neuroimaging studies are crucial to pinpoint changes in brain structure and function associated with training and to examine their overlap with pathology in AD. In this study, we reviewed the existing neuroimaging studies on cognitive training in persons at risk of developing AD to provide an overview of the overlap between neural networks rehabilitated by the current training methods and those affected in AD. The data suggest a consistent training-related increase in brain activity in medial temporal, prefrontal, and posterior default mode networks, as well as increase in gray matter structure in frontoparietal and entorhinal regions. This pattern differs from the observed pattern in healthy older adults that shows a combination of increased and decreased activity in response to training. Detailed investigation of the data suggests that training in persons at risk of developing AD mainly improves compensatory mechanisms and partly restores the affected functions. While current neuroimaging studies are quite helpful in identifying the mechanisms underlying cognitive training, the data calls for future multi-modal neuroimaging studies with focus on multi-domain cognitive training, network level connectivity, and individual differences in response to training. PMID:25206335

  6. Cystinosis as a lysosomal storage disease with multiple mutant alleles: Phenotypic-genotypic correlations

    PubMed Central

    Al-Haggar, Mohammad

    2013-01-01

    Cystinosis is an autosomal recessive lysosomal storage disease with an unclear enzymatic defect causing lysosomal cystine accumulation with no corresponding elevation of plasma cystine levels leading to multisystemic dysfunction. The systemic manifestations include a proximal renal tubular defect (Fanconi-like), endocrinal disturbances, eye involvements, with corneal, conjunctival and retinal depositions, and neurological manifestations in the form of brain and muscle dysfunction. Most of the long-term ill effects of cystinosis are observed particularly in patients with long survival as a result of a renal transplant. Its responsible CTNS gene that encodes the lysosomal cystine carrier protein (cystinosin) has been mapped on the short arm of chromosome 17 (Ch17 p13). There are three clinical forms based on the onset of main symptoms: nephropathic infantile form, nephropathic juvenile form and non-nephropathic adult form with predominant ocular manifestations. Avoidance of eye damage from sun exposure, use of cystine chelators (cysteamine) and finally renal transplantation are the main treatment lines. Pre-implantation genetic diagnosis for carrier parents is pivotal in the prevention of recurrence. PMID:24255892

  7. Positron emission tomographic scan investigations of Huntington's disease: cerebral metabolic correlates of cognitive function

    SciTech Connect

    Berent, S.; Giordani, B.; Lehtinen, S.; Markel, D.; Penney, J.B.; Buchtel, H.A.; Starosta-Rubinstein, S.; Hichwa, R.; Young, A.B.

    1988-06-01

    Fifteen drug-free patients with early to mid-stage Huntington's disease (HD) were evaluated with positron emission tomographic (PET) scans of /sup 18/F-2-fluoro-2-deoxy-D-glucose uptake and quantitative measures of neurological function, learning, memory, and general intelligence. In comparison with a group of normal volunteers, the HD patients showed lower metabolism in both caudate (p less than 0.001) and putamen (p less than 0.001) on PET scans. A significant and positive relationship was found between neuropsychological measures of verbal learning and memory and caudate metabolism in the patient group but not in the normal group. Visual-spatial learning did not reflect a similar pattern, but performance intelligence quotient was positively related to both caudate and putamen metabolism in the HD group. Vocabulary level was unrelated to either brain structure. Discussion focuses on these and other observed brain-behavior relationships and on the implications of these findings for general behaviors such as those involved in coping and adaptation.

  8. Correlates of Osteoprotegerin and Association with Aortic Pulse Wave Velocity in Patients with Chronic Kidney Disease

    PubMed Central

    Leonard, Mary B.; Townsend, Raymond R.; Appel, Lawrence; Wolf, Myles; Budoff, Matt J.; Chen, Jing; Lustigova, Eva; Gadegbeku, Crystal A.; Glenn, Melanie; Hanish, Asaf; Raj, Dominic; Rosas, Sylvia E.; Seliger, Stephen L.; Weir, Matthew R.; Parekh, Rulan S.

    2011-01-01

    Summary Background and objectives Osteoprotegerin (OPG), a cytokine that regulates bone resorption, has been implicated in the process of vascular calcification and stiffness. Design, setting, participants, & measurements Serum OPG was measured in 351 participants with chronic kidney disease (CKD) from one site of the Chronic Renal Insufficiency Cohort Study. Cortical bone mineral content (BMC) was measured by quantitative computed tomography in the tibia. Multivariable linear regression was used to test the association between serum OPG and traditional cardiovascular risk factors, measures of abnormal bone and mineral metabolism, and pulse wave velocity. Results Higher serum OPG levels were associated with older age, female gender, greater systolic BP, lower estimated GFR, and lower serum albumin. OPG was not associated with measures of abnormal bone or mineral metabolism including serum phosphorus, albumin-corrected serum calcium, intact parathyroid hormone, bone-specific alkaline phosphatase, or cortical BMC. Among 226 participants with concurrent aortic pulse wave velocity measurements, increasing tertiles of serum OPG were associated with higher aortic pulse wave velocity after adjustment for demographics, traditional vascular risk factors, and nontraditional risk factors such as estimated GFR, albuminuria, serum phosphate, corrected serum calcium, presence of secondary hyperparathyroidism, serum albumin, and C-reactive protein or after additional adjustment for cortical BMC in a subset (n = 161). Conclusions These data support a strong relationship between serum OPG and arterial stiffness independent of many potential confounders including traditional cardiovascular risk factors, abnormal bone and mineral metabolism, and inflammation. PMID:21940840

  9. Clinical-physiologic correlates of Alzheimer's disease and frontal lobe dementia

    SciTech Connect

    Jagust, W.J.; Reed, B.R.; Seab, J.P.; Kramer, J.H.; Budinger, T.F. )

    1989-01-01

    Thirty patients with degenerative dementia underwent clinical evaluation, neuropsychological testing, and single photon emission computed tomography (SPECT) with the blood flow tracer ({sup 123}I)-N-isopropyl-p-iodoamphetamine. Five of these patients were clinically and psychologically different from the others, demonstrating predominant behavioral disturbances with relative preservation of memory function. These five patients, who were felt to have a frontal lobe dementia (FLD), showed SPECT perfusion patterns which differed from the remaining 25 patients, who were diagnosed as having Alzheimer's disease (AD), and from 16 healthy control subjects. The FLD patients showed diminished perfusion in orbitofrontal, dorsolateral frontal, and temporal cortex relative to controls, while the AD patients showed lower perfusion in temporal and parietal cortex than controls. The FLD patients also showed hypoperfusion in both frontal cortical regions relative to AD patients. The pattern of performance on neuropsychological testing paralleled these differences in regional perfusion. These results suggest that clinical evaluation and physiological imaging may enable the differentiation of groups of degenerative dementia patients during life.

  10. Correlation of disease spectrum among four Dengue serotypes: a five years hospital based study from India.

    PubMed

    Kumaria, Rajni

    2010-01-01

    The recognition of DF (DHF Dengue Hemorrhagic Fever) is very complicated due to occurrence of a wide spectrum of clinical signs and symptoms during acute phase of illness. Moreover, presence of four serotypes further complicates the prognosis. To investigate the predictors of disease severity and elucidate the prognostic markers among four dengue serotypes, this study was conducted on 320 inpatients having acute febrile illness clinically suspected as DI, over a period of five years. Dengue serotypes were confirmed by multiplex reverse transcriptase (RT)-PCR. Eighty patients were positive for DI with presence of Den-1, Den-2, Den-3, and Den-4 in 8, 35, 27 and 10 patients, respectively. The severe clinical manifestations, abdominal pain and hepatomegaly, were comparatively higher in Den-2 patients. Liver aminotransferases levels were also higher in Den-2 patients (app. 5 fold). This study clearly indicates the hyperendemicity of all dengue serotypes. Nucleotide sequencing of Envelope region revealed that the presently emerged Den-3 belongs to type III, having high homology with genotype responsible for number of outbreaks in 1980s. The re-emergence of this deadly type can be suspected to cause more outbreaks in future and is a matter of great concern.

  11. Granulomatous vasculitis in Crohn's disease: a clinicopathologic correlate of two unusual cases.

    PubMed

    Burns, Ariel M; Walsh, Noreen; Green, Peter J

    2010-10-01

    Cutaneous complications occur not uncommonly in patients with Crohn's disease (CD). Gastrointestinal CD often shows non-caseating granulomas and a rare cutaneous finding in CD is a sterile granulomatous infiltrate not contiguous with the GI tract, termed extraintestinal CD (ECD). The clinical presentation of ECD is diverse. The most common histopathological presentation is a superficial and deep granulomatous infiltrate that often accompanies a mixed perivascular infiltrate. Here we report two patients with CD and skin lesions characterized on microscopy by granulomatous vasculitis. A 29-year-old female presented with papules and ulcerated nodules above the ankle. The biopsy showed dermal and superficial subcutaneous involvement by a vasocentric infiltrate of mononuclear and multinucleated histiocytes as well as mural fibrin deposition. A 35-year-old male presented with two tender indurated erythematous plaques with punched-out centers on the lower leg. Histopathologically, a granulomatous vasculitis of small and medium-sized vessels in the dermis and subcutis was evident. These two cases represent the rarely described phenomenon of cutaneous granulomatous vasculitis in CD. Previously reported examples of this entity are reviewed.

  12. Behavioral and Electrophysiological Correlates of Memory Binding Deficits in Patients at Different Risk Levels for Alzheimer's Disease.

    PubMed

    Pietto, Marcos; Parra, Mario A; Trujillo, Natalia; Flores, Facundo; García, Adolfo M; Bustin, Julian; Richly, Pablo; Manes, Facundo; Lopera, Francisco; Ibáñez, Agustín; Baez, Sandra

    2016-06-30

    Deficits in visual short-term memory (VSTM) binding have been proposed as an early and specific marker for Alzheimer's disease (AD). However, no studies have explored the neural correlates of this domain in clinical categories involving prodromal stages with different risk levels of conversion to AD. We assessed underlying electrophysiological modulations in patients with mild cognitive impairment (MCI), patients in the MCI stages of familial AD carrying the mutation E280A of the presenilin-1 gene (MCI-FAD), and healthy controls. Moreover, we compared the behavioral performance and neural correlates of both patient groups. Participants completed a change-detection VSTM task assessing recognition of changes between shapes or shape-color bindings, presented in two consecutive arrays (i.e., study and test) while event related potentials (ERPs) were recorded. Changes always occurred in the test array and consisted of new features replacing studied features (shape-only) or features swapping across items (shape-color binding). Both MCI and MCI-FAD patients performed worse than controls in the shape-color binding condition. Early electrophysiological activity (100-250 ms) was significantly reduced in both clinical groups, particularly over fronto-central and parieto-occipital regions. However, shape-color binding performance and their reduced neural correlates were similar between MCI and MCI-FAD. Our results support the validity of the VSTM binding test and their neural correlates in the early detection of AD and highlight the importance of studies comparing samples at different risk for AD conversion. The combined analysis of behavioral and ERP data gleaned with the VSTM binding task can offer a valuable memory biomarker for AD. PMID:27372640

  13. Depressive symptoms in prodromal Huntington's Disease correlate with Stroop-interference related functional connectivity in the ventromedial prefrontal cortex

    PubMed Central

    Unschuld, Paul G.; Joel, Suresh E.; Pekar, James J.; Reading, Sarah A.; Oishi, Kenichi; McEntee, Julie; Shanahan, Megan; Bakker, Arnold; Margolis, Russell L.; Bassett, Susan S.; Rosenblatt, Adam; Mori, Susumu; van Zijl, Peter C.; Ross, Christopher A.; Redgrave, Graham W.

    2012-01-01

    Huntington's Disease (HD) is a neurodegenerative disorder caused by a cytosine-adenine-guanine (CAG) triplet repeat-expansion in the Huntingtin (HTT) gene. Diagnosis of HD is classically defined by the presence of motor symptoms; however cognitive and depressive symptoms frequently precede motor manifestation, and may occur early in the prodromal phase. There are sparse data so far on functional brain correlates of depressive symptoms in prodromal-HD. A Stroop color-naming test was administered to 32 subjects in the prodromal phase of HD and 52 expansion-negative controls while performing functional magnetic resonance imaging at 3 Tesla. Networks of functional connectivity were identified using group independent component analysis, followed by an analysis of functional network interactions. A contrast of temporal regression-based beta-weights was calculated as a reflection of Stroop-interference related activity and correlated with Center for Epidemiologic Studies Depression (CES-D) scores. For secondary analysis, patients were stratified in two subgroups by median split of CAG repeat-length. Stroop-performance was independent of HTT mutation-carrier-status and CES-D score. Stroop-interference related activity of the ventromedial prefrontal cortex-node of the default-mode network, calculated by temporal-regression beta-weights, was more highly correlated with depressive symptoms in subjects in the prodromal phase of HD than in controls, differing significantly. The strength of this correlation and its difference from controls increased when a subgroup of patients with longer CAG repeat lengths was analyzed. These findings suggest that depressive symptoms in prodromal-HD subjects may reflect altered functional brain network activity in the context of early HD related brain alterations. PMID:22974690

  14. Behavioral and Electrophysiological Correlates of Memory Binding Deficits in Patients at Different Risk Levels for Alzheimer's Disease.

    PubMed

    Pietto, Marcos; Parra, Mario A; Trujillo, Natalia; Flores, Facundo; García, Adolfo M; Bustin, Julian; Richly, Pablo; Manes, Facundo; Lopera, Francisco; Ibáñez, Agustín; Baez, Sandra

    2016-06-30

    Deficits in visual short-term memory (VSTM) binding have been proposed as an early and specific marker for Alzheimer's disease (AD). However, no studies have explored the neural correlates of this domain in clinical categories involving prodromal stages with different risk levels of conversion to AD. We assessed underlying electrophysiological modulations in patients with mild cognitive impairment (MCI), patients in the MCI stages of familial AD carrying the mutation E280A of the presenilin-1 gene (MCI-FAD), and healthy controls. Moreover, we compared the behavioral performance and neural correlates of both patient groups. Participants completed a change-detection VSTM task assessing recognition of changes between shapes or shape-color bindings, presented in two consecutive arrays (i.e., study and test) while event related potentials (ERPs) were recorded. Changes always occurred in the test array and consisted of new features replacing studied features (shape-only) or features swapping across items (shape-color binding). Both MCI and MCI-FAD patients performed worse than controls in the shape-color binding condition. Early electrophysiological activity (100-250 ms) was significantly reduced in both clinical groups, particularly over fronto-central and parieto-occipital regions. However, shape-color binding performance and their reduced neural correlates were similar between MCI and MCI-FAD. Our results support the validity of the VSTM binding test and their neural correlates in the early detection of AD and highlight the importance of studies comparing samples at different risk for AD conversion. The combined analysis of behavioral and ERP data gleaned with the VSTM binding task can offer a valuable memory biomarker for AD.

  15. Clinical correlates of suicidality among individuals with HIV infection and AIDS disease in Mbarara, Uganda.

    PubMed

    Rukundo, Godfrey Zari; Kinyanda, Eugene; Mishara, Brian

    2016-09-01

    The association between suicidality and HIV/AIDS has been demonstrated for three decades, but little is know about risk factors that can help understand this association and help identify who is most at risk. Few research studies have been conducted in sub-Saharan Africa, a region that accounts for more than 70% of the HIV global burden. This paper describes clinical risk factors for suicidality among individuals with HIV infection and AIDS disease in Mbarara, Uganda. In this study, suicidality includes both suicidal ideation and suicidal attempts. A cross-sectional survey was conducted with 543 HIV-positive individuals aged 15 years and above, recruited from 2 HIV specialised clinics in Mbarara. Using logistic regression analysis, factors significantly associated with suicidality at 95% confidence interval were identified. The rate of suicidality was 10% (n = 54; 95% CI: 5.00-15.00). Risk factors for suicidality were: perception of poor physical health (OR 2.22, 95% CI 1.23-3.99, p = 0.007), physical pain (OR 1.83, 95% CI 1.01-3.30, p = 0.049), reducing work due to illness (OR = 2.22, 95% CI 1.23-3.99, p = 0.004) and recent HIV diagnosis (OR 1.02, 95% CI 1.01-1.03, p = 0.001). These findings suggest that HIV/AIDS in south-western Uganda is associated with a considerable burden of suicidality. HIV is associated with several clinical factors that increase vulnerability to suicidality. There is need for more appropriate interventions targeting these clinical risk factors, systematic suicide risk assessment and management of suicidal ideation and behaviours in HIV care. PMID:27681146

  16. Vertically acquired hepatitis C virus infection: Correlates of transmission and disease progression

    PubMed Central

    Tovo, Pier-Angelo; Calitri, Carmelina; Scolfaro, Carlo; Gabiano, Clara; Garazzino, Silvia

    2016-01-01

    The worldwide prevalence of hepatitis C virus (HCV) infection in children is 0.05%-0.4% in developed countries and 2%-5% in resource-limited settings, where inadequately tested blood products or un-sterile medical injections still remain important routes of infection. After the screening of blood donors, mother-to-child transmission (MTCT) of HCV has become the leading cause of pediatric infection, at a rate of 5%. Maternal HIV co-infection is a significant risk factor for MTCT and anti-HIV therapy during pregnancy seemingly can reduce the transmission rate of both viruses. Conversely, a high maternal viral load is an important, but not preventable risk factor, because at present no anti-HCV treatment can be administered to pregnant women to block viral replication. Caution is needed in adopting obstetric procedures, such as amniocentesis or internal fetal monitoring, that can favor fetal exposure to HCV contaminated maternal blood, though evidence is lacking on the real risk of single obstetric practices. Mode of delivery and type of feeding do not represent significant risk factors for MTCT. Therefore, there is no reason to offer elective caesarean section or discourage breast-feeding to HCV infected parturients. Information on the natural history of vertical HCV infection is limited. The primary infection is asymptomatic in infants. At least one quarter of infected children shows a spontaneous viral clearance (SVC) that usually occurs within 6 years of life. IL-28B polymorphims and genotype 3 infection have been associated with greater chances of SVC. In general, HCV progression is mild or moderate in children with chronic infection who grow regularly, though cases with marked liver fibrosis or hepatic failure have been described. Non-organ specific autoantibodies and cryoglobulins are frequently found in children with chronic infection, but autoimmune diseases or HCV associated extrahepatic manifestations are rare. PMID:26819507

  17. Neurofunctional correlates of attention rehabilitation in Parkinson's disease: an explorative study.

    PubMed

    Cerasa, Antonio; Gioia, Maria Cecilia; Salsone, Maria; Donzuso, Giulia; Chiriaco, Carmelina; Realmuto, Sabrina; Nicoletti, Alessandra; Bellavia, Gabriele; Banco, Aurelia; D'amelio, Marco; Zappia, Mario; Quattrone, Aldo

    2014-08-01

    The effectiveness of cognitive rehabilitation (CR) in Parkinson's disease (PD) is in its relative infancy, and nowadays there is insufficient information to support evidence-based clinical protocols. This study is aimed at testing a validated therapeutic strategy characterized by intensive computer-based attention-training program tailored to attention deficits. We further investigated the presence of synaptic plasticity by means of functional magnetic resonance imaging (fMRI). Using a randomized controlled study, we enrolled eight PD patients who underwent a CR program (Experimental group) and seven clinically/demographically-matched PD patients who underwent a placebo intervention (Control group). Brain activity was assessed using an 8-min resting state (RS) fMRI acquisition. Independent component analysis and statistical parametric mapping were used to assess the effect of CR on brain function. Significant effects were detected both at a phenotypic and at an intermediate phenotypic level. After CR, the Experimental group, in comparison with the Control group, showed a specific enhanced performance in cognitive performance as assessed by the SDMT and digit span forward. RS fMRI analysis for all networks revealed two significant groups (Experimental vs Control) × time (T0 vs T1) interaction effects on the analysis of the attention (superior parietal cortex) and central executive neural networks (dorsolateral prefrontal cortex). We demonstrated that intensive CR tailored for the impaired abilities impacts neural plasticity and improves some aspects of cognitive deficits of PD patients. The reported neurophysiological and behavioural effects corroborate the benefits of our therapeutic approach, which might have a reliable application in clinical management of cognitive deficits.

  18. Asymmetric dimethylarginine Correlates with Measures of Disease Severity, Major Adverse Cardiovascular Events and All-Cause Mortality in Patients with Peripheral Arterial Disease

    PubMed Central

    Wilson, Andrew M; Shin, David S; Weatherby, Carlton; Harada, Randall K; Ng, Martin K; Nair, Nandini; Kielstein, Jan; Cooke, John P

    2011-01-01

    Background Peripheral arterial disease (PAD) is associated with major cardiovascular morbidity and mortality. Abnormalities in nitric oxide metabolism due to excess of the NO synthase inhibitor asymmetric dimethylarginine (ADMA) may be pathogenic in PAD. We explored the association between ADMA levels and markers of atherosclerosis, function, and prognosis. Methods and Results 133 patients with symptomatic PAD were enrolled. Ankle brachial index (ABI), walking time, vascular function measures (arterial compliance and flow-mediated vasodilatation) and plasma ADMA level were assessed for each patient at baseline. ADMA correlated inversely with ABI (r = −0.238, p=0.003) and walking time (r = −0.255, p = 0.001), independent of other vascular risk factors. We followed up 125 (94%) of our 133 initial subjects with baseline measurements (mean 35 months). Subjects with ADMA levels in the highest quartile (>0.84 μmol/L) showed significantly greater occurrence of MACE compared to those with ADMA levels in the lower 3 quartiles (p = 0.001). Cox proportional-hazards regression analysis revealed that ADMA was a significant predictor of MACE, independent of other risk factors including age, gender, blood pressure, smoking history, diabetes and ABI (Hazard ratio = 5.1, p<0.001). Measures of vascular function, such as compliance, FMVD and blood pressure, as well as markers of PAD severity, including ABI and walking time, were not predictive. Conclusion Circulating levels of ADMA correlate independently with measures of disease severity and major adverse cardiovascular events. Agents that target this pathway may be useful for this patient population. PMID:20484311

  19. Kingella kingae Expresses Four Structurally Distinct Polysaccharide Capsules That Differ in Their Correlation with Invasive Disease

    PubMed Central

    Porsch, Eric A.; Seed, Patrick C.; Heiss, Christian; Naran, Radnaa; Amit, Uri; Yagupsky, Pablo; Azadi, Parastoo; St. Geme, Joseph W.

    2016-01-01

    Kingella kingae is an encapsulated gram-negative organism that is a common cause of osteoarticular infections in young children. In earlier work, we identified a glycosyltransferase gene called csaA that is necessary for synthesis of the [3)-β-GalpNAc-(1→5)-β-Kdop-(2→] polysaccharide capsule (type a) in K. kingae strain 269–492. In the current study, we analyzed a large collection of invasive and carrier isolates from Israel and found that csaA was present in only 47% of the isolates. Further examination of this collection using primers based on the sequence that flanks csaA revealed three additional gene clusters (designated the csb, csc, and csd loci), all encoding predicted glycosyltransferases. The csb locus contains the csbA, csbB, and csbC genes and is associated with a capsule that is a polymer of [6)-α-GlcpNAc-(1→5)-β-(8-OAc)Kdop-(2→] (type b). The csc locus contains the cscA, cscB, and cscC genes and is associated with a capsule that is a polymer of [3)-β-Ribf-(1→2)-β-Ribf-(1→2)-β-Ribf-(1→4)-β-Kdop-(2→] (type c). The csd locus contains the csdA, csdB, and csdC genes and is associated with a capsule that is a polymer of [P-(O→3)[β-Galp-(1→4)]-β-GlcpNAc-(1→3)-α-GlcpNAc-1-] (type d). Introduction of the csa, csb, csc, and csd loci into strain KK01Δcsa, a strain 269–492 derivative that lacks the native csaA gene, was sufficient to produce the type a capsule, type b capsule, type c capsule, and type d capsule, respectively, indicating that these loci are solely responsible for determining capsule type in K. kingae. Further analysis demonstrated that 96% of the invasive isolates express either the type a or type b capsule and that a disproportionate percentage of carrier isolates express the type c or type d capsule. These results establish that there are at least four structurally distinct K. kingae capsule types and suggest that capsule type plays an important role in promoting K. kingae invasive disease. PMID:27760194

  20. Blood-Borne RNA Correlates with Disease Activity and IFN-Stimulated Gene Expression in Systemic Lupus Erythematosus.

    PubMed

    Doedens, John R; Jones, Wendell D; Hill, Kay; Mason, Michael J; Gersuk, Vivian H; Mease, Philip J; Dall'Era, Maria; Aranow, Cynthia; Martin, Richard W; Cohen, Stanley B; Fleischmann, Roy M; Kivitz, Alan J; Burge, Daniel J; Chaussabel, Damien; Elkon, Keith B; Posada, James A

    2016-10-01

    The loss of tolerance and the presence of circulating autoantibodies directed against nuclear Ags is the hallmark of systemic lupus erythematosus (SLE). Many of these Ags are complexed with short, noncoding RNAs, such as U1 and Y1. The amount of U1 and Y1 RNA complexed with SLE patient Abs and immune complexes was measured in a cross-section of 228 SLE patients to evaluate the role of these RNA molecules within the known biochemical framework of SLE. The study revealed that SLE patients had significantly elevated levels of circulating U1 and/or Y1 RNA compared with healthy volunteers. In addition, the blood-borne RNA molecules were correlated with SLE disease activity and increased expression of IFN-inducible genes. To our knowledge, this study provides the first systematic examination of the role of circulating RNA in a large group of SLE patients and provides an important link with IFN dysregulation.

  1. Single nucleotide polymorphisms in the dystroglycan gene do not correlate with disease severity in hereditary inclusion body myopathy.

    PubMed

    Gottlieb, Emily; Ciccone, Carla; Darvish, Daniel; Naiem-Cohen, Shahrouz; Dalakas, Marinos C; Savelkoul, Paul J; Krasnewich, Donna M; Gahl, William A; Huizing, Marjan

    2005-01-01

    Aberrant glycosylation of dystroglycan occurs in certain muscular dystrophies, including hereditary inclusion body myopathy (HIBM). HIBM harbors a widely varying clinical severity and age of onset, which raised the suspicion of the presence of disease modifier genes. We considered the highly polymorphic dystroglycan gene (DAG1) as a feasible candidate modifier gene. DAG1 genomic DNA was sequenced for 32 HIBM patients, mainly of Persian-Jewish descent. Five novel DAG1 single nucleotide polymorphisms (SNPs) were identified, bringing the total number of SNPs to 19. However, no direct correlation between DAG1 SNPs and clinical severity of HIBM could be detected. Several identified SNPs substitute an amino acid and might modulate dystroglycan function or glycosylation status, and deserve further research. These data are valuable for future studies on the role of DAG1 in HIBM and other muscular dystrophies, especially those dystrophies that involve abnormal glycosylation of dystroglycan.

  2. Blood-Borne RNA Correlates with Disease Activity and IFN-Stimulated Gene Expression in Systemic Lupus Erythematosus.

    PubMed

    Doedens, John R; Jones, Wendell D; Hill, Kay; Mason, Michael J; Gersuk, Vivian H; Mease, Philip J; Dall'Era, Maria; Aranow, Cynthia; Martin, Richard W; Cohen, Stanley B; Fleischmann, Roy M; Kivitz, Alan J; Burge, Daniel J; Chaussabel, Damien; Elkon, Keith B; Posada, James A

    2016-10-01

    The loss of tolerance and the presence of circulating autoantibodies directed against nuclear Ags is the hallmark of systemic lupus erythematosus (SLE). Many of these Ags are complexed with short, noncoding RNAs, such as U1 and Y1. The amount of U1 and Y1 RNA complexed with SLE patient Abs and immune complexes was measured in a cross-section of 228 SLE patients to evaluate the role of these RNA molecules within the known biochemical framework of SLE. The study revealed that SLE patients had significantly elevated levels of circulating U1 and/or Y1 RNA compared with healthy volunteers. In addition, the blood-borne RNA molecules were correlated with SLE disease activity and increased expression of IFN-inducible genes. To our knowledge, this study provides the first systematic examination of the role of circulating RNA in a large group of SLE patients and provides an important link with IFN dysregulation. PMID:27534558

  3. Increased Intestinal Permeability Correlates with Sigmoid Mucosa alpha-Synuclein Staining and Endotoxin Exposure Markers in Early Parkinson's Disease

    PubMed Central

    Forsyth, Christopher B.; Shannon, Kathleen M.; Kordower, Jeffrey H.; Voigt, Robin M.; Shaikh, Maliha; Jaglin, Jean A.; Estes, Jacob D.; Dodiya, Hemraj B.; Keshavarzian, Ali

    2011-01-01

    Parkinson's disease (PD) is the second most common neurodegenerative disorder of aging. The pathological hallmark of PD is neuronal inclusions termed Lewy bodies whose main component is alpha-synuclein protein. The finding of these Lewy bodies in the intestinal enteric nerves led to the hypothesis that the intestine might be an early site of PD disease in response to an environmental toxin or pathogen. One potential mechanism for environmental toxin(s) and proinflammatory luminal products to gain access to mucosal neuronal tissue and promote oxidative stress is compromised intestinal barrier integrity. However, the role of intestinal permeability in PD has never been tested. We hypothesized that PD subjects might exhibit increased intestinal permeability to proinflammatory bacterial products in the intestine. To test our hypothesis we evaluated intestinal permeability in subjects newly diagnosed with PD and compared their values to healthy subjects. In addition, we obtained intestinal biopsies from both groups and used immunohistochemistry to assess bacterial translocation, nitrotyrosine (oxidative stress), and alpha-synuclein. We also evaluated serum markers of endotoxin exposure including LPS binding protein (LBP). Our data show that our PD subjects exhibit significantly greater intestinal permeability (gut leakiness) than controls. In addition, this intestinal hyperpermeability significantly correlated with increased intestinal mucosa staining for E. coli bacteria, nitrotyrosine, and alpha-synuclein as well as serum LBP levels in PD subjects. These data represent not only the first demonstration of abnormal intestinal permeability in PD subjects but also the first correlation of increased intestinal permeability in PD with intestinal alpha–synuclein (the hallmark of PD), as well as staining for gram negative bacteria and tissue oxidative stress. Our study may thus shed new light on PD pathogenesis as well as provide a new method for earlier diagnosis of PD and

  4. Slowing of Hippocampal Activity Correlates with Cognitive Decline in Early Onset Alzheimer's Disease. An MEG Study with Virtual Electrodes.

    PubMed

    Engels, Marjolein M A; Hillebrand, Arjan; van der Flier, Wiesje M; Stam, Cornelis J; Scheltens, Philip; van Straaten, Elisabeth C W

    2016-01-01

    Pathology in Alzheimer's disease (AD) starts in the entorhinal cortex and hippocampus. Because of their deep location, activity from these areas is difficult to record with conventional electro- or magnetoencephalography (EEG/MEG). The purpose of this study was to explore hippocampal activity in AD patients and healthy controls using "virtual MEG electrodes". We used resting-state MEG recordings from 27 early onset AD patients [age 60.6 ± 5.4, 12 females, mini-mental state examination (MMSE) range: 19-28] and 26 cognitively healthy age- and gender-matched controls (age 61.8 ± 5.5, 14 females). Activity was reconstructed using beamformer-based virtual electrodes for 78 cortical regions and 6 hippocampal regions. Group differences in peak frequency and relative power in six frequency bands were identified using permutation testing. For the patients, spearman correlations between the MMSE scores and peak frequency or relative power were calculated. Moreover, receiver operator characteristic curves were plotted to estimate the diagnostic accuracy. We found a lower hippocampal peak frequency in AD compared to controls, which, in the patients, correlated positively with MMSE [r(25) = 0.61; p < 0.01] whereas hippocampal relative theta power correlated negatively with MMSE [r(25) = -0.54; p < 0.01]. Cortical peak frequency was also lower in AD in association areas. Furthermore, cortical peak frequency correlated positively with MMSE [r(25) = 0.43; p < 0.05]. In line with this finding, relative theta power was higher in AD across the cortex, and relative alpha and beta power was lower in more circumscribed areas. The average cortical relative theta power was the best discriminator between AD and controls (sensitivity 82%; specificity 81%). Using beamformer-based virtual electrodes, we were able to detect hippocampal activity in AD. In AD, this hippocampal activity is slowed, and correlates better with cognition than the (slowed) activity in cortical areas. On the other

  5. Slowing of Hippocampal Activity Correlates with Cognitive Decline in Early Onset Alzheimer's Disease. An MEG Study with Virtual Electrodes.

    PubMed

    Engels, Marjolein M A; Hillebrand, Arjan; van der Flier, Wiesje M; Stam, Cornelis J; Scheltens, Philip; van Straaten, Elisabeth C W

    2016-01-01

    Pathology in Alzheimer's disease (AD) starts in the entorhinal cortex and hippocampus. Because of their deep location, activity from these areas is difficult to record with conventional electro- or magnetoencephalography (EEG/MEG). The purpose of this study was to explore hippocampal activity in AD patients and healthy controls using "virtual MEG electrodes". We used resting-state MEG recordings from 27 early onset AD patients [age 60.6 ± 5.4, 12 females, mini-mental state examination (MMSE) range: 19-28] and 26 cognitively healthy age- and gender-matched controls (age 61.8 ± 5.5, 14 females). Activity was reconstructed using beamformer-based virtual electrodes for 78 cortical regions and 6 hippocampal regions. Group differences in peak frequency and relative power in six frequency bands were identified using permutation testing. For the patients, spearman correlations between the MMSE scores and peak frequency or relative power were calculated. Moreover, receiver operator characteristic curves were plotted to estimate the diagnostic accuracy. We found a lower hippocampal peak frequency in AD compared to controls, which, in the patients, correlated positively with MMSE [r(25) = 0.61; p < 0.01] whereas hippocampal relative theta power correlated negatively with MMSE [r(25) = -0.54; p < 0.01]. Cortical peak frequency was also lower in AD in association areas. Furthermore, cortical peak frequency correlated positively with MMSE [r(25) = 0.43; p < 0.05]. In line with this finding, relative theta power was higher in AD across the cortex, and relative alpha and beta power was lower in more circumscribed areas. The average cortical relative theta power was the best discriminator between AD and controls (sensitivity 82%; specificity 81%). Using beamformer-based virtual electrodes, we were able to detect hippocampal activity in AD. In AD, this hippocampal activity is slowed, and correlates better with cognition than the (slowed) activity in cortical areas. On the other

  6. The neural correlates of naming and fluency deficits in Alzheimer’s disease: an FDG-PET study

    PubMed Central

    Melrose, Rebecca J.; Campa, Olivia M.; Harwood, Dylan G.; Osato, Sheryl; Mandelkern, Mark A.; Sultzer, David L.

    2009-01-01

    Summary Objective To examine the neural processes associated with language deficits in Alzheimer’s disease (AD), and in particular to elucidate the correlates of confrontation naming and word retrieval impairments. Methods Sixty patients with probable AD were included. Confrontation naming was assessed using the number of words spontaneously named correctly on the Boston Naming Test. We recorded the number of additional words stated following phonemic cuing. We also assessed phonemic (FAS) and semantic (supermarket items) fluency. We then correlated performance on each measure with resting cortical metabolic activity using FDG-PET images. Results We found that poorer ability to spontaneously name an object was associated with hypometabolism of bilateral inferior temporal lobes. In contrast, when a phonemic cue was provided, successful naming under this condition was associated with higher metabolic activity in bilateral inferior frontal gyrus (IFG), right superior frontal gyrus (SFG), left temporal, and occipital regions. Consistent with these findings, we found that poorer semantic fluency was associated with hypometabolism in regions including both IFG and temporal regions, and poorer phonemic fluency was associated with hypometabolism in only left IFG. Across analyses, measures that required cued retrieval were associated with metabolism in the left IFG, whereas measures taxing semantic knowledge were associated with metabolic rate of left temporal cortex. Conclusions Naming deficits in AD reflect compromise to temporal regions involved in the semantic knowledge network, and frontal regions involved in the controlled retrieval of information from that network. PMID:19296551

  7. Accumulation of aspartic acid421- and glutamic acid391-cleaved tau in neurofibrillary tangles correlates with progression in Alzheimer disease.

    PubMed

    Basurto-Islas, Gustavo; Luna-Muñoz, Jose; Guillozet-Bongaarts, Angela L; Binder, Lester I; Mena, Raul; García-Sierra, Francisco

    2008-05-01

    Truncations of tau protein at aspartic acid421 (D421) and glutamic acid391 (E391) residues are associated with neurofibrillary tangles (NFTs) in the brains of Alzheimer disease (AD) patients. Using immunohistochemistry with antibodies to D421- and E391-truncated tau (Tau-C3 and MN423, respectively), we correlated the presence of NFTs composed of these truncated tau proteins with clinical and neuropathologic parameters in 17 AD and 23 non-AD control brains. The densities of NFTs composed of D421- or E391-truncated tau correlated with clinical dementia index and Braak staging in AD. Glutamic acid391 tau truncation was prominent in the entorhinal cortex, whereas D421 truncation was prominent in the subiculum, suggesting that NFTs composed of either D421- or E391-truncated tau may be formed mutually exclusively in these areas. Both truncations were associated with the prevalence of the apolipoprotein E epsilon4 allele. By double labeling, intact tau in NFTs was commonly associated with D421-cleaved tau but not with E391-truncated tau; D421-cleaved tau was never associated with E391-truncated tau. These results indicate that tau is not randomly proteolyzed at different domains, and that proteolysis occurs sequentially from the C-terminus to inner regions of tau in AD progression. Identification of NFTs composed of tau at different stages of truncation may facilitate assessment of neurofibrillary pathology in AD.

  8. Single-nucleotide polymorphisms and haplotypes of non-coding area in the CP gene are correlated with Parkinson's disease.

    PubMed

    Zhao, Na; Xiao, Jianqiu; Zheng, Zhiyong; Fei, Guoqiang; Zhang, Feng; Jin, Lirong; Zhong, Chunjiu

    2015-04-01

    Our previous studies have demonstrated that ceruloplasmin (CP) dysmetabolism is correlated with Parkinson's disease (PD). However, the causes of decreased serum CP levels in PD patients remain to be clarified. This study aimed to explore the potential association between genetic variants of the CP gene and PD. Clinical features, serum CP levels, and the CP gene (both promoter and coding regions) were analyzed in 60 PD patients and 50 controls. A luciferase reporter system was used to investigate the function of promoter single-nucleotide polymorphisms (SNPs). High-density comparative genomic hybridization microarrays were also used to detect large-scale copy-number variations in CP and an additional 47 genes involved in PD and/or copper/iron metabolism. The frequencies of eight SNPs (one intronic SNP and seven promoter SNPs of the CP gene) and their haplotypes were significantly different between PD patients, especially those with lowered serum CP levels, and controls. However, the luciferase reporter system revealed no significant effect of the risk haplotype on promoter activity of the CP gene. Neither these SNPs nor their haplotypes were correlated with the Hoehn and Yahr staging of PD. The results of this study suggest that common genetic variants of CP are associated with PD and further investigation is needed to explore their functions in PD.

  9. Correlation between Serum Lipid Levels and Measured Glomerular Filtration Rate in Chinese Patients with Chronic Kidney Disease

    PubMed Central

    Wang, Caixia; Ye, Zengchun; Li, Shaomin; Liu, Qiong; Lou, Tanqi

    2016-01-01

    Introduction Dyslipidemia is often detected in patients with chronic kidney disease (CKD). Previous studies of the relationship between lipid profiles and kidney function have yielded variable results. We aimed to investigate the correlation between serum lipid levels and kidney function evaluated by measured glomerular filtration rate (mGFR) in Chinese patients with CKD. Methods A cross-sectional study was conducted on 2036 Chinese CKD patients who had mGFR. Linear regression analysis was performed to evaluate the correlation between different serum lipid levels and mGFR, while logistic regression analysis was used to investigate the association between CKD stages and the risk of different types of dyslipidemia. Results The mean age was 55 years and the mean mGFR was 63 mL/min/1.73m2. After adjusting for some confounders (age, gender, body mass index, a history of diabetes, fasting glucose, a history of hypertension, systolic blood pressure, diastolic blood pressure, smoking status, hemoglobin, serum potassium, serum albumin, and serum uric acid), serum triglyceride level showed a negative correlation with mGFR (β = -0.006, P = 0.006) in linear regression analysis, and CKD stages were positively related to the risk of hypertriglyceridemia (odds ratios were 1.329, 1.868, 2.514 and P were 0.046, < 0.001, < 0.001 for CKD stage 2, 3, 4/5, respectively) in logistic regression anlysis. Conclusions Serum triglyceride level is independently association with mGFR. Patients with reduced kidney function are more likely to have higher serum triglyceride levels. Further longitudinal, multicenter and well-conducted studies are needed to provide more evidence. PMID:27695128

  10. Altered Fecal Microbiota Correlates with Liver Biochemistry in Nonobese Patients with Non-alcoholic Fatty Liver Disease

    PubMed Central

    Wang, Baohong; Jiang, Xiangyang; Cao, Min; Ge, Jianping; Bao, Qiongling; Tang, Lingling; Chen, Yu; Li, Lanjuan

    2016-01-01

    Increasing evidence suggests a role of intestinal dysbiosis in obesity and non-alcoholic fatty liver disease (NAFLD). But it remains unknown in nonobese NAFLD. This prospective, cross-sectional study sought to characterize differences in fecal microbiota between nonobese adult individuals with and without NAFLD and their potential association with metabolic markers of disease progression. A total of 126 nonobese subjects were enrolled: 43 NAFLD and 83 healthy controls (HC). The microbial community was profiled by denaturing gradient gel electrophoresis and examined by 454 pyrosequencing of the 16S ribosomal RNA V3 region. Lower diversity and a phylum-level change in the fecal microbiome were found in NAFLD. Compared with HC, patients had 20% more phylum Bacteroidetes (p = 0.005) and 24% less Firmicutes (p = 0.002). Within Firmicutes, four families and their 8 genera, which were short-chain fatty acids-producing and 7α-dehydroxylating bacteria, were significantly decreased. Moreover, Gram-negative (G−) bacteria were prevalent in NAFLD (p = 0.008). Furthermore, a significant correlation with metabolic markers was revealed for disturbed microbiota in NAFLD. This novel study indicated that intestinal dysbiosis was associated with nonobese NAFLD and might increase the risk of NAFLD progression. PMID:27550547

  11. Altered Fecal Microbiota Correlates with Liver Biochemistry in Nonobese Patients with Non-alcoholic Fatty Liver Disease.

    PubMed

    Wang, Baohong; Jiang, Xiangyang; Cao, Min; Ge, Jianping; Bao, Qiongling; Tang, Lingling; Chen, Yu; Li, Lanjuan

    2016-01-01

    Increasing evidence suggests a role of intestinal dysbiosis in obesity and non-alcoholic fatty liver disease (NAFLD). But it remains unknown in nonobese NAFLD. This prospective, cross-sectional study sought to characterize differences in fecal microbiota between nonobese adult individuals with and without NAFLD and their potential association with metabolic markers of disease progression. A total of 126 nonobese subjects were enrolled: 43 NAFLD and 83 healthy controls (HC). The microbial community was profiled by denaturing gradient gel electrophoresis and examined by 454 pyrosequencing of the 16S ribosomal RNA V3 region. Lower diversity and a phylum-level change in the fecal microbiome were found in NAFLD. Compared with HC, patients had 20% more phylum Bacteroidetes (p = 0.005) and 24% less Firmicutes (p = 0.002). Within Firmicutes, four families and their 8 genera, which were short-chain fatty acids-producing and 7α-dehydroxylating bacteria, were significantly decreased. Moreover, Gram-negative (G-) bacteria were prevalent in NAFLD (p = 0.008). Furthermore, a significant correlation with metabolic markers was revealed for disturbed microbiota in NAFLD. This novel study indicated that intestinal dysbiosis was associated with nonobese NAFLD and might increase the risk of NAFLD progression. PMID:27550547

  12. Intra-epidemic evolutionary dynamics of a Dengue virus type 1 population reveal mutant spectra that correlate with disease transmission.

    PubMed

    Hapuarachchi, Hapuarachchige Chanditha; Koo, Carmen; Kek, Relus; Xu, Helen; Lai, Yee Ling; Liu, Lilac; Kok, Suet Yheng; Shi, Yuan; Chuen, Raphael Lee Tze; Lee, Kim-Sung; Maurer-Stroh, Sebastian; Ng, Lee Ching

    2016-01-01

    Dengue virus (DENV) is currently the most prevalent mosquito-borne viral pathogen. DENVs naturally exist as highly heterogeneous populations. Even though the descriptions on DENV diversity are plentiful, only a few studies have narrated the dynamics of intra-epidemic virus diversity at a fine scale. Such accounts are important to decipher the reciprocal relationship between viral evolutionary dynamics and disease transmission that shape dengue epidemiology. In the current study, we present a micro-scale genetic analysis of a monophyletic lineage of DENV-1 genotype III (epidemic lineage) detected from November 2012 to May 2014. The lineage was involved in an unprecedented dengue epidemic in Singapore during 2013-2014. Our findings showed that the epidemic lineage was an ensemble of mutants (variants) originated from an initial mixed viral population. The composition of mutant spectrum was dynamic and positively correlated with case load. The close interaction between viral evolution and transmission intensity indicated that tracking genetic diversity through time is potentially a useful tool to infer DENV transmission dynamics and thereby, to assess the epidemic risk in a disease control perspective. Moreover, such information is salient to understand the viral basis of clinical outcome and immune response variations that is imperative to effective vaccine design. PMID:26940650

  13. Intra-epidemic evolutionary dynamics of a Dengue virus type 1 population reveal mutant spectra that correlate with disease transmission.

    PubMed

    Hapuarachchi, Hapuarachchige Chanditha; Koo, Carmen; Kek, Relus; Xu, Helen; Lai, Yee Ling; Liu, Lilac; Kok, Suet Yheng; Shi, Yuan; Chuen, Raphael Lee Tze; Lee, Kim-Sung; Maurer-Stroh, Sebastian; Ng, Lee Ching

    2016-01-01

    Dengue virus (DENV) is currently the most prevalent mosquito-borne viral pathogen. DENVs naturally exist as highly heterogeneous populations. Even though the descriptions on DENV diversity are plentiful, only a few studies have narrated the dynamics of intra-epidemic virus diversity at a fine scale. Such accounts are important to decipher the reciprocal relationship between viral evolutionary dynamics and disease transmission that shape dengue epidemiology. In the current study, we present a micro-scale genetic analysis of a monophyletic lineage of DENV-1 genotype III (epidemic lineage) detected from November 2012 to May 2014. The lineage was involved in an unprecedented dengue epidemic in Singapore during 2013-2014. Our findings showed that the epidemic lineage was an ensemble of mutants (variants) originated from an initial mixed viral population. The composition of mutant spectrum was dynamic and positively correlated with case load. The close interaction between viral evolution and transmission intensity indicated that tracking genetic diversity through time is potentially a useful tool to infer DENV transmission dynamics and thereby, to assess the epidemic risk in a disease control perspective. Moreover, such information is salient to understand the viral basis of clinical outcome and immune response variations that is imperative to effective vaccine design.

  14. Geographic Variation of Chronic Kidney Disease Prevalence: Correlation with the Incidence of Renal Cell Carcinoma or Urothelial Carcinoma?

    PubMed Central

    Yap, Yit-Sheung; Chuang, Kai-Wen; Chiang, Chun-Ju; Chuang, Hung-Yi; Lu, Sheng-Nan

    2015-01-01

    Background. The aim of this study is to evaluate whether geographic variations in the prevalence of late-stage chronic kidney disease (CKD) exist and are associated with incidence rates of renal cell carcinoma (RCC), upper tract urothelial carcinoma (UTUC), or lower tract urothelial carcinoma (LTUC). Methods. Prevalence rates of late-stage CKD for 366 townships (n > 30) in Taiwan were calculated for 1,518,241 and 1,645,151 subjects aged 40 years or older in years 2010 and 2009, respectively. Late-stage CKD prevalence in year 2010 was used as a training set and its age-adjusted standardized morbidity rates (ASMR) were divided into three groups as defined <1.76%, 1.76% ≤ ASMR < 2.64%, and ≥2.64%, respectively. Year 2009, defined as the validation set, was used to validate the results. Results. The ASMR of late-stage CKD in years 2010 and 2009 were 1.76%, and 2.09%, respectively. Geographic variations were observed, with notably higher rates of disease in areas of the central, southwestern mountainside, and southeastern seaboard. There were no significant differences among different combined risk groups of RCC, UTUC, and LTUC incidence. Conclusion. The substantial geographic variations in the prevalence of late-stage CKD exist, but are not correlated with RCC, UTUC, or LTUC incidence. PMID:26605329

  15. Proximal correlates of metabolic phenotypes during ‘at-risk' and ‘case' stages of the metabolic disease continuum

    PubMed Central

    Haren, M T; Misan, G; Grant, J F; Buckley, J D; Howe, P R C; Taylor, A W; Newbury, J; McDermott, R A

    2012-01-01

    Objective: To examine the social and behavioural correlates of metabolic phenotypes during ‘at-risk' and ‘case' stages of the metabolic disease continuum. Design: Cross-sectional study of a random population sample. Participants: A total of 718 community-dwelling adults (57% female), aged 18–92 years from a regional South Australian city. Measurements: Total body fat and lean mass and abdominal fat mass were assessed by dual energy x-ray absorptiometry. Fasting venous blood was collected in the morning for assessment of glycated haemoglobin, plasma glucose, serum triglycerides, cholesterol lipoproteins and insulin. Seated blood pressure (BP) was measured. Physical activity and smoking, alcohol and diet (96-item food frequency), sleep duration and frequency of sleep disordered breathing (SDB) symptoms, and family history of cardiometabolic disease, education, lifetime occupation and household income were assessed by questionnaire. Current medications were determined by clinical inventory. Results: 36.5% were pharmacologically managed for a metabolic risk factor or had known diabetes (‘cases'), otherwise were classified as the ‘at-risk' population. In both ‘at-risk' and ‘cases', four major metabolic phenotypes were identified using principal components analysis that explained over 77% of the metabolic variance between people: fat mass/insulinemia (FMI); BP; lipidaemia/lean mass (LLM) and glycaemia (GLY). The BP phenotype was uncorrelated with other phenotypes in ‘cases', whereas all phenotypes were inter-correlated in the ‘at-risk'. Over and above other socioeconomic and behavioural factors, medications were the dominant correlates of all phenotypes in ‘cases' and SDB symptom frequency was most strongly associated with FMI, LLM and GLY phenotypes in the ‘at-risk'. Conclusion: Previous research has shown FMI, LLM and GLY phenotypes to be most strongly predictive of diabetes development. Reducing SDB symptom frequency and optimising the duration

  16. Respiratory disease in systemic lupus erythematosus: correlation with results of laboratory tests and histological appearance of muscle biopsy specimens.

    PubMed Central

    Evans, S A; Hopkinson, N D; Kinnear, W J; Watson, L; Powell, R J; Johnston, I D

    1992-01-01

    BACKGROUND: In systemic lupus erythematosus, certain laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis reflect disease activity. A study was designed to determine if such indices predict respiratory lesions, and in particular whether the presence of vasculitis in quadriceps muscle reflects respiratory muscle function. METHODS: Twenty seven 27 patients with systemic lupus erythematosus were studied, ten of whom were consecutive untreated patients and 17 having clinically active disease and being treated. They were prospectively evaluated on the basis of erythrocyte sedimentation rate, lymphocyte count, C3 degradation products, quadriceps muscle biopsy, spirometry, lung volumes, carbon monoxide transfer factor, and mouth pressure during a maximal sniff. RESULTS: Lung function test results were abnormal in 12 patients. Vital capacity was reduced in seven, carbon monoxide transfer factor capacity in five, and mouth pressure was low (< 70% predicted) in ten. Lymphocytic vasculitis was seen in the muscle biopsy specimens of ten patients. No correlation was found between laboratory tests and lung function or mouth pressure, or between the presence of lymphocytic vasculitis and mouth pressure. In untreated patients, those with lymphocytic vasculitis had lower spirometric values. CONCLUSIONS: In systemic lupus erythematosus, evidence from muscle biopsy specimens of lymphocytic vasculitis is not predictive of impaired inspiratory muscle function as measured by mouth pressure. In untreated patients there were relationships between some laboratory test results and respiratory function, but this was not the case for the whole group. In systemic lupus erythematosus, laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis are therefore unlikely to be helpful in the assessment of respiratory disease. Images PMID:1465755

  17. Southern leaf blight disease severity is correlated with decreased maize leaf epiphytic bacterial species richness and the phyllosphere bacterial diversity decline is enhanced by nitrogen fertilization

    PubMed Central

    Manching, Heather C.; Balint-Kurti, Peter J.; Stapleton, Ann E.

    2014-01-01

    Plant leaves are inhabited by a diverse group of microorganisms that are important contributors to optimal growth. Biotic and abiotic effects on plant growth are usually studied in controlled settings examining response to variation in single factors and in field settings with large numbers of variables. Multi-factor experiments with combinations of stresses bridge this gap, increasing our understanding of the genotype-environment-phenotype functional map for the host plant and the affiliated epiphytic community. The maize inbred B73 was exposed to single and combination abiotic and the biotic stress treatments: low nitrogen fertilizer and high levels of infection with southern leaf blight (causal agent Cochliobolus heterostrophus). Microbial epiphyte samples were collected at the vegetative early-season phase and species composition was determined using 16S ribosomal intergenic spacer analysis. Plant traits and level of southern leaf blight disease were measured late-season. Bacterial diversity was different among stress treatment groups (P < 0.001). Lower species richness—alpha diversity—was correlated with increased severity of southern leaf blight disease when disease pressure was high. Nitrogen fertilization intensified the decline in bacterial alpha diversity. While no single bacterial ribotype was consistently associated with disease severity, small sets of ribotypes were good predictors of disease levels. Difference in leaf bacterial-epiphyte diversity early in the season were correlated with plant disease severity, supporting further tests of microbial epiphyte-disease correlations for use in predicting disease progression. PMID:25177328

  18. Uncovering Molecular Biomarkers That Correlate Cognitive Decline with the Changes of Hippocampus' Gene Expression Profiles in Alzheimer's Disease

    PubMed Central

    Gómez Ravetti, Martín; Rosso, Osvaldo A.; Berretta, Regina; Moscato, Pablo

    2010-01-01

    Background Alzheimer's disease (AD) is characterized by a neurodegenerative progression that alters cognition. On a phenotypical level, cognition is evaluated by means of the MiniMental State Examination (MMSE) and the post-morten examination of Neurofibrillary Tangle count (NFT) helps to confirm an AD diagnostic. The MMSE evaluates different aspects of cognition including orientation, short-term memory (retention and recall), attention and language. As there is a normal cognitive decline with aging, and death is the final state on which NFT can be counted, the identification of brain gene expression biomarkers from these phenotypical measures has been elusive. Methodology/Principal Findings We have reanalysed a microarray dataset contributed in 2004 by Blalock et al. of 31 samples corresponding to hippocampus gene expression from 22 AD subjects of varying degree of severity and 9 controls. Instead of only relying on correlations of gene expression with the associated MMSE and NFT measures, and by using modern bioinformatics methods based on information theory and combinatorial optimization, we uncovered a 1,372-probe gene expression signature that presents a high-consensus with established markers of progression in AD. The signature reveals alterations in calcium, insulin, phosphatidylinositol and wnt-signalling. Among the most correlated gene probes with AD severity we found those linked to synaptic function, neurofilament bundle assembly and neuronal plasticity. Conclusions/Significance A transcription factors analysis of 1,372-probe signature reveals significant associations with the EGR/KROX family of proteins, MAZ, and E2F1. The gene homologous of EGR1, zif268, Egr-1 or Zenk, together with other members of the EGR family, are consolidating a key role in the neuronal plasticity in the brain. These results indicate a degree of commonality between putative genes involved in AD and prion-induced neurodegenerative processes that warrants further investigation

  19. NK Cell Genotype and Phenotype at Diagnosis of Acute Lymphoblastic Leukemia Correlate to Post-induction Residual Disease

    PubMed Central

    Sullivan, Erin M.; Jeha, Sima; Kang, Guolian; Cheng, Cheng; Rooney, Barbara; Holladay, Martha; Bari, Rafijul; Schell, Sarah; Tuggle, MaCal; Pui, Ching-Hon; Leung, Wing

    2014-01-01

    Purpose Not all natural killer (NK) cells are equally cytotoxic against leukemia because of differences in receptor gene content and surface expression. We correlated NK cell genotype and phenotype at diagnosis of childhood acute lymphoblastic leukemia (ALL) with minimal residual disease (MRD) after induction chemotherapy. Experimental Design The NK cells and leukemia blasts of 244 patients were analyzed at diagnosis by killer-cell immunoglobulin-like receptor (KIR) typing and immunophenotyping. The results were correlated statistically to post-induction MRD status. Results The odds of being MRD positive in patients with KIR telomeric (Tel)-A/B genotype was 2.85 times the odds in those with Tel-A/A genotype (p=0.035). MRD positive patients were more likely to have KIR2DL5A (p=0.006) and expressed less activating receptor NKp46 and FASL on their NK cells (p=0.0074 and p=0.029, respectively). The odds of being MRD positive increased by 2.01-fold for every percentage increase in NK cells expressing KIR2DL1 in the presence of HLA-C2 ligand (p=0.034). The quantity of granzyme B inhibitor PI-9 in the leukemia blasts was greater in patients who were MRD positive (p=0.038). Collectively, five NK cell-related factors (Tel-B associated KIR2DL5A, NKp46, FASL, Granzyme B, and PI-9) are strongly associated with MRD positivity at the end of induction with 100% sensitivity and 80% specificity. Conclusions Our data support the hypothesis that NK cells with a strong effector phenotype in the setting of decreased leukemia resistance are associated with better leukemia control. PMID:25281696

  20. Neuroimaging correlates of neuropsychiatric symptoms in Alzheimer's disease: a review of 20 years of research.

    PubMed

    Boublay, N; Schott, A M; Krolak-Salmon, P

    2016-10-01

    Assessing morphological, perfusion and metabolic brain changes preceding or associated with neuropsychiatric symptoms (NPSs) will help in the understanding of pathophysiological underlying processes in Alzheimer's disease (AD). This review aimed to highlight the main findings on significant associations between neuroimaging and NPSs, the pathophysiology to elucidate possible underlying mechanisms, and methodological issues to aid future research. Research papers published from January 1990 to October 2015 were identified in the databases PsycInfo, Embase, PubMed and Medline, using key words related to NPSs and imaging techniques. In addition to a semi-systematic search in the databases, we also performed hand searches based on reported citations identified to be of interest. Delusions, apathy and depression symptoms were particularly associated with brain changes in AD. The majority of studies disclosed an association between frontal lobe structural and/or metabolic changes and NPSs, implicating, interestingly, for all 12 NPSs studied, the anterior cingulate cortex although temporal, subcortical and parietal regions, and insula were also involved. Given the high degree of connectivity of these brain areas, frontal change correlates of NPSs may help in the understanding of neural network participation. This review also highlights crucial methodological issues that may reduce the heterogeneity of results to enable progress on the pathophysiological mechanisms and aid research on NPS treatments in AD. Based on a broad review of the current literature, a global brain pattern to support the huge heterogeneity of neuroimaging correlates of NPSs in AD and methodological strategies are suggested to help direct future research. PMID:27435186

  1. Expression of hepatic Fibroblast Growth Factor 19 is enhanced in Primary Biliary Cirrhosis and correlates with severity of the disease

    PubMed Central

    Wunsch, Ewa; Milkiewicz, Małgorzata; Wasik, Urszula; Trottier, Jocelyn; Kempińska-Podhorodecka, Agnieszka; Elias, Elwyn; Barbier, Olivier; Milkiewicz, Piotr

    2015-01-01

    Cholestasis induces adaptive mechanisms protecting the liver against bile acids (BA) toxicity including modulation of BA synthesis. Whether fibroblast growth factor 19 (FGF19) or farnesoid X receptor (FXR) dependent signaling are involved in the regulation of BA homeostasis in primary biliary cirrhosis (PBC) remains unknown. Here we analyzed hepatic expression of FGF19 and other genes relevant to the adaptive response to cholestasis in tissues from non-cirrhotic (n = 24) and cirrhotic (n = 21) patients along with control tissues (n = 21). Moreover we searched for relationships between serum FGF19 and laboratory/clinical findings in 51 patients. Hepatic FGF19 mRNA expression was increased in non-cirrhotic and cirrhotic tissues (9-fold,p = 0.01; 69-fold,p < 0.0001, respectively). Protein levels of FGF19, FGF receptor 4, FXR and short heterodimer partner were increased in cirrhotic livers (9-fold, p < 0.001; 3.5-fold,p = 0.007; 2.4-fold,p < 0.0001; 2.8-fold,p < 0.0001 vs controls, respectively) which was accompanied by down-regulation of CYP7A1 (50% reduction, p = 0.006). Serum and liver levels of FGF19 correlated with worse liver biochemistry, BAs, quality of life and Mayo Risk Score. Serum FGF19 was elevated in UDCA non-responders. We conclude that PBC induces characteristic changes in liver expression of BAs synthesis regulatory molecules. FGF19 correlates with severity of liver disease and can potentially serve as an indicator of chronic cholestatic liver injury. PMID:26293907

  2. Identification of mutations, genotype-phenotype correlation and prenatal diagnosis of maple syrup urine disease in Indian patients.

    PubMed

    Gupta, Deepti; Bijarnia-Mahay, Sunita; Saxena, Renu; Kohli, Sudha; Dua-Puri, Ratna; Verma, Jyotsna; Thomas, E; Shigematsu, Yosuke; Yamaguchi, Seiji; Deb, Roumi; Verma, Ishwar Chander

    2015-09-01

    Maple syrup urine disease (MSUD) is caused by mutations in genes BCKDHA, BCKDHB, DBT encoding E1α, E1β, and E2 subunits of enzyme complex, branched-chain alpha-ketoacid dehydrogenase (BCKDH). BCKDH participates in catabolism of branched-chain amino acids (BCAAs) - leucine, isoleucine and valine in the energy production pathway. Deficiency or defect in the enzyme complex causes accumulation of BCAAs and keto-acids leading to toxicity. Twenty-four patients with MSUD were enrolled in the study for molecular characterization and genotype-phenotype correlation. Molecular studies were carried out by sequencing of the 3 genes by Sanger method. Bioinformatics tools were employed to classify novel variations into pathogenic or benign. The predicted effects of novel changes on protein structure were elucidated by 3D modeling. Mutations were detected in 22 of 24 patients (11, 7 and 4 in BCKDHB, BCKDHA and DBT genes, respectively). Twenty mutations including 11 novel mutations were identified. Protein modeling in novel mutations showed alteration of structure and function of these subunits. Mutations, c.1065 delT (BCKDHB gene) and c.939G > C (DBT gene) were noted to be recurrent, identified in 6 of 22 alleles and 5 of 8 alleles, respectively. Two-third patients were of neonatal classical phenotype (16 of 24). BCKDHB gene mutations were present in 10 of these 16 patients. Prenatal diagnoses were performed in 4 families. Consanguinity was noted in 37.5% families. Although no obvious genotype-phenotype correlation could be found in our study, most cases with mutation in BCKDHB gene presented in neonatal period. Large number of novel mutations underlines the heterogeneity and distinctness of gene pool from India. PMID:26257134

  3. Correlations between immunogenicity, drug levels, and disease activity in an Italian cohort of rheumatoid arthritis patients treated with tocilizumab

    PubMed Central

    Benucci, Maurizio; Meacci, Francesca; Grossi, Valentina; Infantino, Maria; Manfredi, Mariangela; Bellio, Emanuele; Bellio, Valerio; Li Gobbi, Francesca; Bazzichi, Laura; Moscato, Paolo; Caputo, Dario; Saviola, Gianantonio; Talotta, Rossella; Sarzi-Puttini, Piercarlo; Atzeni, Fabiola

    2016-01-01

    The aim of this study was to evaluate the real-life immunogenicity of anti-drug antibodies, drug levels, and disease activity in an Italian cohort of rheumatoid arthritis patients treated with tocilizumab (TCZ). We evaluated 126 TCZ-treated patients with rheumatoid arthritis (16 males and 110 females; mean age 59±12 years, range 26–83; mean disease duration 11±5 years) with inadequate 12-week response to any synthetic and biological disease-modifying anti-rheumatic drugs, in a retrospective analysis. One-hundred and seven patients were treated with methotrexate mean dose 12.6±1.3 mg/week in combination with TCZ, 13 received TCZ monotherapy, and six received leflunomide 20 mg/day plus TCZ; all patients were treated with prednisone mean dose 6.4±1.2 mg/day. They had a 28-joint Disease Activity Score (DAS28) of >3.2, an erythrocyte sedimentation rate (ESR) of >30 mm/hour, and CRP levels of >1.0 mg/dL. We evaluated at baseline and after 6 months of treatment: DAS28; rheumatoid factor (RF) IgM, IgA, and IgG; anti-citrullinated peptide antibody; ESR; CRP; TNF-α; and IL-6. TCZ and anti-TCZ antibodies were detected using LISA-TRACKER Duo TCZ. TCZ levels of <10 µg/mL were considered low and >10 µg/mL high. After 6 months of treatment only one patient was positive for anti-TCZ antibodies. There were correlations between DAS28, ESR, and CRP and IL-6 levels in all patients. Comparison of the 84 patients with TCZ levels of <10 µg/mL and the 42 with TCZ levels of >10 µg/mL showed the following differences: DAS28: 3.09±1.32 vs 2.78±1.32, P=0.0005; ESR: 27±14.8 vs 14±12 mm/hour, P=0.0001; CRP: 1.47±1.05 vs 0.65±0.80 mg/dL, P=0.0086; TNF-α: 10.2±1.2 vs 9.9±1.1 pg/mL, P=0.999; IL-6: 3.65±4.75 vs 3.62±4.41 pg/mL, P=0.97; anti-citrullinated peptide antibody: 85.2±93.7 vs 86.7±90.3 IU/mL, P=0.94; RF IgM: 72.4±62.7 vs 68.3±61.6 IU/mL, P=0.754; RF IgA: 41.7±36.4 vs 47.8±42.1 U/mL, P=0.449; and RF IgG: 46.4±46.1 vs 59.3±58.2 U/mL, P=0.212. These findings show

  4. Role of muscarinic-3 receptor antibody in systemic sclerosis: correlation with disease duration and effects of IVIG.

    PubMed

    Kumar, Sumit; Singh, Jagmohan; Kedika, Ramalinga; Mendoza, Fabian; Jimenez, Sergio A; Blomain, Erik S; DiMarino, Anthony J; Cohen, Sidney; Rattan, Satish

    2016-06-01

    Gastrointestinal dysmotility in systemic sclerosis (SSc) is associated with autoantibodies against muscarinic-3 receptor (M3-R). We investigated the temporal course of the site of action of these autoantibodies at the myenteric neurons (MN) vs. the smooth muscle (SM) M3-R in relation to disease duration, and determined the role of intravenous immunoglobulin (IVIG) in reversing these changes. Immunoglobulins purified from SSc patients (SScIgG) were used to assess their differential binding to MN and SM (from rat colon) employing immunohistochemistry (IHC). Effect of SScIgG on neural and direct muscle contraction was determined by cholinergic nerve stimulation and bethanechol-induced SM contraction. Effects of IVIG and its antigen-binding fragment F(ab')2 on SScIgG binding were studied by enzyme-linked immunosorbent assay (ELISA) of rat colonic longitudinal SM myenteric plexus (LSMMP) lysate and to second extracellular loop peptide of M3-R (M3-RL2). SScIgG from all patients demonstrated significantly higher binding to MN than to SM. With progression of SSc duration, binding at MN and SM increased in a linear fashion with a correlation coefficient of 0.696 and 0.726, respectively (P < 0.05). SScIgG-mediated attenuation of neural and direct SM contraction also increased with disease duration. ELISA analysis revealed that IVIG and F(ab')2 significantly reduced SScIgG binding to LSMMP lysate and M3-RL2. Dysmotility in SSc occurs sequentially, beginning with SScIgG-induced blockage of cholinergic neurotransmission (neuropathy), which progresses to inhibition of acetylcholine action at the SM cell (myopathy). IVIG reverses this cholinergic dysfunction at the neural and myogenic receptors by anti-idiotypic neutralization of SScIgG.

  5. Immunohistochemical and histopathologic correlates of Alzheimer's disease-associated Alz-50 immunoreactivity quantified in homogenates of cerebral tissue.

    PubMed Central

    de la Monte, S. M.; Spratt, R. A.; Chong, J.; Ghanbari, H. A.; Wands, J. R.

    1992-01-01

    Alz-50 is a monoclonal antibody that immunoreacts with neurofibrillary tangles and neurites in brains with Alzheimer's disease (AD). In addition, the levels of Alz-50 immunoreactivity in brain, measured by either enzyme-linked immunosorbent assay or ALZ-enzyme-linked immunosorbant assay (EIA), are increased in AD relative to age-matched controls. The current study compares the distribution and extent of Alz-50 immunostaining with quantified levels of Alz-50 immunoreactivity measured in adjacent frozen blocks of tissue by ALZ-EIA. The brain tissue studied was obtained from individuals with AD, AD + Down's syndrome (AD + DN), Parkinson's disease with dementia (PD), or AD + PD, and from nondemented aged controls. In AD, AD + DN, and AD + PD, there were significantly higher densities of Alz-50-immunoreactive (AFI) neurons, more abundant diffuse AFI neurites, and higher ALZ-EIA values than in aged controls. In PD, the overall mean density of AFI neurons was significantly lower than in AD and AD + DN, but AFI neurites were as abundant as they were in brains with an AD diagnosis. However, PD was readily distinguished from AD and AD + DN by significantly lower mean ALZ-EIA values, and significantly lower densities of neurofilament-immuno-reactive AD lesions. Multiple-regression analysis demonstrated significant correlations between ALZ-EIA levels and the severity of AD lesions, and the density of AFI neurites, but not with the density of AFI neurons. Therefore, ALZ-EIA levels may represent only a portion of the Alz-50 immunoreactivity detectable by immunohistochemical staining. Images Figure 1 PMID:1466403

  6. The Neuropathology of Huntington´s disease: classical findings, recent developments and correlation to functional neuroanatomy.

    PubMed

    Rüb, Udo; Vonsattel, Jean Paul V; Heinsen, Helmut; Korf, Horst-Werner

    2015-01-01

    Huntington’s disease (HD) is a severe, autosomal dominantly inherited, gradually worsening neurological disorder, the clinical features of which were first described in 1863 by Irving W. Lyon and with additional details, in 1872, by George Huntington. Progress in molecular biological research has shown that HD is caused by meiotically unstable CAG-repeats in the mutated HD gene (the so-called IT 15 gene) on chromosome 4p16.3, which encodes the mutated protein huntingtin (Htt). This monograph provides a survey of the stepwise progress in neuropathological HD research made during a time period of more than hundred years, the currently known neuropathological hallmarks of HD, as well as their pathogenic and clinical relevance. Starting with the initial descriptions of the progressive degeneration of the neostriatum (i.e., caudate nucleus and putamen) as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating qualitative and quantitative neuropathological data with characteristics pertaining to the functional neuroanatomy of the human brain, subsequent chapters will highlight the latest neuropathological HD findings: the area- and layer-specifi c neuronal loss in the cerebral neo- and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and the deep cerebellar nuclei, the involvement of distinct brainstem nuclei, and the pathophysiological relevance of these pathologies for the clinical phenotype of HD. Finally, the potential pathophysiological role of axonal transport deficit

  7. Smothering in UK free-range flocks. Part 2: investigating correlations between disease, housing and management practices.

    PubMed

    Rayner, A C; Gill, R; Brass, D; Willings, T H; Bright, A

    2016-09-10

    Smothering, when birds group together in a way that results in death from suffocation, is a welfare and economic concern for the egg industry. This questionnaire-based study explored correlations between disease, housing, management practices and smothering on free-range farms. A binomial logistic regression approach was used to test whether question responses predicted occurrence of nest box smothers (NBS) and panic and recurring smothers (PSRS) on farms. Breed (P=0.008) and nest box manufacturer (P=0.014) predicted NBS. Breed and nest box design have been previously reported to affect nesting behaviour. The affect of nest box manufacturer found in this study may illustrate the effect of nest box design features or house layouts. Nest box manufacturer (P=0.009), feeding oyster grit or grain on the litter (P<0.001) and range use on a sunny day (P<0.001) also predicted PSRS. Implementing some management practices to encourage desirable behaviours (eg ranging) may contribute to smothering, whereas some management practices such as those aimed at occupying birds may be beneficial, illustrating the delicate balance of factors involved in free-range egg production. It is hoped that these results will stimulate further work exploring the suitability of housing design and management of laying hens in light of smothering. PMID:27353872

  8. Smothering in UK free-range flocks. Part 2: investigating correlations between disease, housing and management practices.

    PubMed

    Rayner, A C; Gill, R; Brass, D; Willings, T H; Bright, A

    2016-09-10

    Smothering, when birds group together in a way that results in death from suffocation, is a welfare and economic concern for the egg industry. This questionnaire-based study explored correlations between disease, housing, management practices and smothering on free-range farms. A binomial logistic regression approach was used to test whether question responses predicted occurrence of nest box smothers (NBS) and panic and recurring smothers (PSRS) on farms. Breed (P=0.008) and nest box manufacturer (P=0.014) predicted NBS. Breed and nest box design have been previously reported to affect nesting behaviour. The affect of nest box manufacturer found in this study may illustrate the effect of nest box design features or house layouts. Nest box manufacturer (P=0.009), feeding oyster grit or grain on the litter (P<0.001) and range use on a sunny day (P<0.001) also predicted PSRS. Implementing some management practices to encourage desirable behaviours (eg ranging) may contribute to smothering, whereas some management practices such as those aimed at occupying birds may be beneficial, illustrating the delicate balance of factors involved in free-range egg production. It is hoped that these results will stimulate further work exploring the suitability of housing design and management of laying hens in light of smothering.

  9. Integrative Frequency Power of EEG Correlates with Progression of Mild Cognitive Impairment to Dementia in Parkinson's Disease.

    PubMed

    Gu, Youquan; Chen, Jun; Lu, Yaqin; Pan, Suyue

    2016-04-01

    Clinically, predicting the progression of mild cognitive impairment (MCI) and diagnosing dementia in Parkinson's disease (PD) are difficult. This study aims to explore an integrative electroencephalography (EEG) frequency power that could be used to predict the progression of MCI in PD patients. Twenty-six PD patients, in this study, were divided into the mild cognitive impairment group (PDMCI, 17 patients) and dementia group (PDD, 9 patients) according to cognitive performance. Beta peak frequency, alpha relative power, and alpha/theta power were recorded and analyzed for the prediction. Mini Mental State Examination (MMSE) scores at initiation, in the first year, and in the second year were examined. The sensitivity, specificity, positive predictive value, Matthew correlation coefficient, and positive likelihood ratio were calculated in both the integrative EEG biomarkers and single best biomarker. Of the 17 patients with MCI for 2 years, 6 progressed to dementia. Integrative EEG biomarkers, mainly associated with beta peak frequency, can predict conversion from MCI to dementia. These biomarkers had sensitivity of 82% and specificity of 78%, compared with sensitivity of 61% and specificity of 58% of the beta peak frequency. In conclusion, the integrative EEG frequency powers were more sensitive and specific to MCI progression in PD patients.

  10. Reduced basal and stimulated (isoprenaline, Gpp(NH)p, forskolin) adenylate cyclase activity in Alzheimer's disease correlated with histopathological changes.

    PubMed

    Ohm, T G; Bohl, J; Lemmer, B

    1991-02-01

    Cyclic adenosine monophosphate (cAMP) is an adenylate cyclase borne second messenger involved in basic metabolic events. The beta-adrenoceptor sensitive adenylate cyclase was studied in post-mortem hippocampi of controls and Alzheimer patients. Virtually identical subsets of each hippocampus homogenate were stimulated by 100 mumol isoprenaline, Gpp(NH)p and forskolin, respectively, in presence of an ATP-regenerating system. The determination of cAMP formed was carried out by means of a radioassay. The observed significant 50% reduction in basal as well as in stimulated adenylate cyclase activity in Alzheimer's disease is negatively correlated with semiquantitative evaluations of amyloid plaques (P less than 0.05) but not with neuritic plaques, neurofibrillary tangles or neuropil threads. This reduction in enzyme activity is obviously not due to simple cell loss alone. It is likely that the crucial point of the observed functional disturbance is at the level of the catalytic unit of the adenylate cyclase, since the same degree of reduction is maintained at all steps of the signal cascade. PMID:2054615

  11. [Correlations between angiocardiography and changes of R wave amplitude during effort in patients with coronary heart disease (author's transl)].

    PubMed

    De Caprio, L; Adamo, B; Bellotti, P; Cuomo, S; Meccariello, P; Romano, M; Vigorito, C; Rengo, F

    1980-01-01

    The Authors studied correlations between angiocardiography and changes of R wave amplitude (delta R) during effort in 113 patients. They showed coronarographic evidence: 69 with stenosis greater than or equal to 70% of at least one major coronary vessel and 44 with no significant lesions. delta R values greater or equal than 0 were considered as pathologic. delta R appeared greater than or equal to 0 in 8 of 14 patients (57%) with single stenosis, 4 (28%) with abnormal wall motion (AWM). delta R increase or unchanged in 26 of 31 (84%) patients with double stenoses, 25 of them (81%) with AWM. delta R was greater than or equal to 0 in 22 of 24 (92%) with triple stenoses. In subjects with milk coronary artery disease (CAD) R wave increased or unchanged in 47% (19/44). Pathologic changes of R wave are highly frequent in CAD patients, especially in those with severe impairment. These changes, however, are not specific or costant because they appeared enough frequently in subjects with normal doronary vessels, and, moreover, R wave may decrease also in CAD patients with severe impairment. The Authors consider that evidence of delta R values greater than or equal to 0 may not be considered as a sign of CAD, but it must be evaluated with the other data showed by ergometric tests. PMID:7372032

  12. Beak and feather disease virus: correlation between viral load and clinical signs in wild Cape parrots (Poicepahlus robustus) in South Africa.

    PubMed

    Regnard, Guy L; Boyes, Rutledge S; Martin, Rowan O; Hitzeroth, Inga I; Rybicki, Edward P

    2015-01-01

    Psittacine beak and feather disease (PBFD), the most prevalent viral disease affecting psittacines, is caused by beak and feather disease virus (BFDV). This study assessed viral load using qPCR in a wild Cape parrot population affected by PBFD and compared it to overall physical condition based on clinical signs attributable to PBFD. A significant inverse correlation between viral load and overall physical condition was found, which confirmed that clinical signs may confidently be used to diagnose the relative severity of BFDV infections in wild populations. This is the first assessment of BFDV viral load in a wild psittacine population.

  13. Tests of Dorsolateral Frontal Function Correlate with Objective Tests of Postural Stability in Early to Moderate Stage Parkinson’s Disease

    PubMed Central

    Nocera, Joe R.; Price, Catherine; Fernandez, Hubert H.; Amano, Shinichi; Vallabhajosula, Srikant; Okun, Michael S.; Hwynn, Nelson; Hass, Chris J.

    2010-01-01

    A substantial number of individuals with Parkinson’s disease who display impaired postural stability experience accelerated cognitive decline and an increased prevalence of dementia. To date, studies suggest that this relationship, believed to be due to involvement of nondopaminergic circuitry, occurs later in the disease process. Research has yet to adequately investigate this cognitive-posturomotor relationship especially when examining earlier disease states. To gain greater understanding of the relationship between postural stability and cognitive function/dysfunction we evaluated a more stringent, objective measure of postural stability (center of pressure displacement), and also more specific measures of cognition in twenty-two patients with early to moderate stage Parkinson’s disease. The magnitude of the center of pressure displacement in this cohort was negatively correlated with performance on tests known to activate dorsolateral frontal regions. Additionally, the postural stability item of the UPDRS exhibited poor correlation with the more objective measure of center of pressure displacement and all specific measures of cognition. These results may serve as rationale for a more thorough evaluation of postural stability and cognition especially in individuals with mild Parkinson’s disease. Greater understanding of the relationship between motor and cognitive processes in Parkinson’s disease will be critical for understanding the disease process and its potential therapeutic possibilities. PMID:20829093

  14. Amyloid Beta-Mediated Hypomethylation of Heme Oxygenase 1 Correlates with Cognitive Impairment in Alzheimer’s Disease

    PubMed Central

    Sung, Hye Youn; Choi, Byung-Ok; Jeong, Jee Hyang; Kong, Kyoung Ae; Hwang, Jinha; Ahn, Jung-Hyuck

    2016-01-01

    To identify epigenetically regulated genes involved in the pathogenesis of Alzheimer’s disease (AD) we analyzed global mRNA expression and methylation profiles in amyloid precursor protein (APP)-Swedish mutant-expressing AD model cells, H4-sw and selected heme oxygenase-1 (HMOX1), which is associated with pathological features of AD such as neurofibrillary tangles and senile plaques. We examined the epigenetic regulatory mechanism of HMOX1 and its application as a diagnostic and prognostic biomarker for AD. Our results show that HMOX1 mRNA and protein expression was approximately 12.2-fold and 7.9-fold increased in H4-sw cells, respectively. Increased HMOX1 expression was also detected in the brain, particularly the hippocampus, of AD model transgenic mice. However, the methylation of specific CpG sites within its promoter, particularly at CpG located −374 was significantly decreased in H4-sw cells. Treatment of neuroglioma cells with the demethylating agent 5-aza-2′-deoxycytidine resulted in reduced methylation of HMOX1 promoter accompanied by enhanced HMOX1 expression strongly supporting DNA methylation-dependent transcriptional regulation of HMOX1. Toxic Aβ-induced aberrant hypomethylation of HMOX1 at −374 promoter CpG site was correlated with increased HMOX1expression. In addition to neuroglioma cells, we also found Aβ-induced epigenetic regulation of HMOX1 in human T lymphocyte Jurkat cells. We evaluated DNA methylation status of HMOX1 at −374 promoter CpG site in blood samples from AD patients, patients with mild cognitive impairment (MCI), and control individuals using quantitative methylation-specific polymerase chain reaction. We observed lower methylation of HMOX1 at the −374 promoter CpG site in AD patients compared to MCI and control individuals, and a correlation between Mini-Mental State Examination score and demethylation level. Receiver operating characteristics analysis revealed good discrimination of AD patients from MCI patients and

  15. Increased interleukin-6 correlates with myelin oligodendrocyte glycoprotein antibodies in pediatric monophasic demyelinating diseases and multiple sclerosis.

    PubMed

    Horellou, Philippe; Wang, Min; Keo, Vixra; Chrétien, Pascale; Serguera, Ché; Waters, Patrick; Deiva, Kumaran

    2015-12-15

    Acquired demyelinating syndromes (ADS) in children evolve either as a monophasic disease diagnosed as acute demyelinating encephalomyelitis (ADEM), transverse myelitis (TM) or optic neuritis (ON), or a multiphasic one with several relapses most often leading to the diagnosis of multiple sclerosis (MS) or neuromyelitis optica (NMO). These neuroinflammatory disorders are increasingly associated with autoantibodies against proteins such as aquaporin-4 in rare instances, and more frequently against myelin oligodendrocyte glycoprotein (MOG). Recently, in adult NMO patients, C5a levels were shown to be elevated in cerebrospinal fluid (CSF) during acute exacerbation. We investigated the CSF levels of anaphylatoxins and pro-inflammatory cytokines, and plasma MOG antibodies in onset samples from children with ADS. Thirty four children presenting with a first episode of ADS, 17 with monophasic ADS (9 with ADEM, 4 with TM and 4 with ON) and 17 with MS, who had paired blood and CSF samples at onset were included and compared to 12 patients with other non-inflammatory neurological disorders (OND). Cytokines and anaphylatoxins in CSF were measured by Cytometric Bead Array immunoassay. MOG antibody titers in plasma were tested by flow cytometry using a stable cell line expressing full-length human MOG. We found a significant increase in C5a levels in the CSF of patients with monophasic ADS (n=17) compared to OND (n=12, p=0.0036) and to MS (n=17, p=0.0371). The C5a levels in MS were higher than in OND without reaching significance (p=0.2). CSF IL-6 levels were significantly increased in monophasic ADS compared to OND (p=0.0027) and to MS (p=0.0046). MOG antibody plasma levels were significantly higher in monophasic ADS (p<0.0001) and, to a lesser extent, in MS compared to OND (p=0.0023). Plasma MOG antibodies and CSF IL-6 levels were significantly correlated (r=0.51, p=0.018). CSF C5a and IL-6 levels are increased in monophasic ADS but not in MS when compared to OND, suggesting

  16. CD28⁻ CD8⁺ T cells are significantly reduced and correlate with disease duration in juveniles with type 1 diabetes.

    PubMed

    Yarde, Danielle N; Lorenzo-Arteaga, Kristina; Corley, Kevin P; Cabrera, Monina; Sarvetnick, Nora E

    2014-10-01

    Type 1 diabetes (T1D) is a chronic disease caused by autoimmune destruction of insulin-producing pancreatic β-cells. T1D is typically diagnosed in children, but information regarding immune cell subsets in juveniles with T1D is scarce. Therefore, we studied various lymphocytic populations found in the peripheral blood of juveniles with T1D compared to age-matched controls (ages 2-17). One population of interest is the CD28(-) CD8(+) T cell subset, which are late-differentiated cells also described as suppressors. These cells are altered in a number of disease states and have been shown to be reduced in adults with T1D. We found that the proportion of CD28(-) cells within the CD8(+) T cell population is significantly reduced in juvenile type 1 diabetics. Furthermore, this reduction is not correlated with age in T1D juveniles, although a significant negative correlation between proportion CD28(-) CD8(+) T cells and age was observed in the healthy controls. Finally, correlation analysis revealed a significant and negative correlation between the proportion of CD28(-) CD8(+) T cells and T1D disease duration. These findings show that the CD28(-) CD8(+) T cell population is perturbed following onset of disease and may prove to be a valuable marker for monitoring the progression of T1D.

  17. Soluble Ligands for the NKG2D Receptor Are Released during Endometriosis and Correlate with Disease Severity

    PubMed Central

    González-Foruria, Iñaki; Santulli, Pietro; Chouzenoux, Sandrine; Carmona, Francisco

    2015-01-01

    Background Endometriosis is a benign gynaecological disease. Abundant bulk of evidence suggests that patients with endometriosis have an immunity dysfunction that enables ectopic endometrial cells to implant and proliferate. Previous studies show that natural killer cells have a pivotal role in the immune control of endometriosis. Methods and Findings This is a prospective laboratory study conducted in a tertiary-care university hospital between January 2011 and April 2013. We investigated non-pregnant, younger than 42-year-old patients (n= 202) during surgery for benign gynaecological conditions. After complete surgical exploration of the abdominopelvic cavity, 121 women with histologically proven endometriosis and 81 endometriosis-free controls women were enrolled. Patients with endometriosis were classified according to a surgical classification in three different types of endometriosis: superficial peritoneal endometriosis (SUP), ovarian endometrioma (OMA) and deep infiltrating endometriosis (DIE). Peritoneal fluid samples were obtained from all study participants during the surgery in order to detect soluble NKG2D ligands (MICA, MICB and ULBP-2). When samples with undetectable peritoneal fluid levels of MICA, MICB and ULBP-2 were excluded, MICA ratio levels were significantly higher in endometriosis patients than in controls (median, 1.1 pg/mg; range, 0.1–143.5 versus median, 0.6 pg/mg; range, 0.1–3.5; p=0.003). In a similar manner peritoneal fluid MICB levels were also increased in endometriosis-affected patients compared with disease-free women (median, 4.6 pg/mg; range, 1.2–4702 versus median, 3.4 pg/mg; range, 0.7–20.1; p=0.001). According to the surgical classification, peritoneal fluid soluble MICA, MICB and ULBP-2 ratio levels were significantly increased in DIE as compared to controls (p=0.015, p=0.003 and p=0.045 respectively). MICA ratio levels also correlated with dysmenorrhea (r=0.232; p=0.029), total rAFS score (r=0.221; p=0.031) and

  18. A Laboratory Phenotype/Genotype Correlation of 1167 French Patients From 670 Families With von Willebrand Disease

    PubMed Central

    Veyradier, Agnès; Boisseau, Pierre; Fressinaud, Edith; Caron, Claudine; Ternisien, Catherine; Giraud, Mathilde; Zawadzki, Christophe; Trossaert, Marc; Itzhar-Baïkian, Nathalie; Dreyfus, Marie; d’Oiron, Roseline; Borel-Derlon, Annie; Susen, Sophie; Bezieau, Stéphane; Denis, Cécile V.; Goudemand, Jenny

    2016-01-01

    Abstract von Willebrand disease (VWD) is a genetic bleeding disease due to a defect of von Willebrand factor (VWF), a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels <50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50 IU/dL). The French reference center for VWD performed a laboratory phenotypic and genotypic analysis in 1167 VWD patients (670 families) selected by their basic biologic phenotype: type 3, type 2, and type 1 with VWF levels <30 IU/dL. In these patients indeed, to achieve an accurate diagnosis of VWD type and subtype is crucial for the management (treatment and genetic counseling). A phenotype/genotype correlation was present in 99.3% of cases; 323 distinct VWF sequence variations (58% of novel) were identified (missense 67% versus truncating 33%). The distribution of VWD types was: 25% of type 1, 8% of type 3, 66% of type 2 (2A: 18%, 2B: 17%, 2M: 19%, 2N: 12%), and 1% of undetermined type. Type 1 VWD was related either to a defective synthesis/secretion or to an accelerated clearance of VWF. In type 3 VWD, bi-allelic mutations of VWF were found in almost all patients. In type 2A, the most frequent mechanism was a hyper-proteolysis of VWF. Type 2B showed 85% of patients with deleterious mutations (distinct from type 2B New York). Type 2M was linked to a defective binding of VWF to platelet glycoprotein Ib or to collagen. Type 2N VWD included almost half type 2N/3. This biologic study emphasizes the complex mechanisms for both quantitative and qualitative VWF defects in VWD. In addition, this study provides a new epidemiologic picture of the most bleeding forms of VWD in which

  19. Two systemic lupus erythematosus (SLE) global disease activity indexes--the SLE Disease Activity Index and the Systemic Lupus Activity Measure--demonstrate different correlations with activation of peripheral blood CD4+ T cells.

    PubMed

    Daca, Agnieszka; Czuszyńska, Zenobia; Smoleńska, Zaneta; Zdrojewski, Zbigniew; Witkowski, Jacek M; Bryl, Ewa

    2011-12-01

    Global disease activity measurement in systemic lupus erythematosus (SLE) patients is important for the clinical estimation and adjustment of therapy. By contrast, immune system activation plays a significant role in disease pathogenesis, with CD4+ lymphocytes acting as central cells in the immune response. We investigated which scale better correlates with immunologic changes in the blood of SLE patients, the SLE Disease Activity Index (SLEDAI) or the Systemic Lupus Activity Measure (SLAM) scale. Samples of peripheral blood were obtained from 45 SLE patients with different disease activity as assessed by the SLEDAI and the SLAM scales on the same day. We assessed the percentage of CD4+ T cells with activation-associated receptors: CD69, CD25int, CD95, HLA-DR, and CD4+ T cells with killing properties containing perforin and granzyme B. Our results indicated that the percentage of CD4+CD69+ and CD4+CD25(int) cells did not correlate with either the SLEDAI or the SLAM scale. Significant and positive correlations were observed between percentages of CD4+CD95+ and CD4+HLA-DR+ lymphocytes and SLE activity, but only when activity was measured using the SLAM scale, not with the SLEDAI scale. The percentage of CD4+perforin+ and CD4+granzyme B+ cells also strongly correlated with disease activity measured only with the SLAM scale. We conclude that the SLAM scale better reflects changes of immune system activity in SLE patients compared with the SLEDAI scale.

  20. Plasma zinc levels inversely correlate with vascular cell adhesion molecule-1 concentration in children with sickle cell disease.

    PubMed Central

    Kuvibidila, Solo R.; Sandoval, Manuel; Lao, Juan; Velez, Maria; Yu, Lolie; Ode, David; Gardner, Renée; Lane, Gerald; Warrier, Raj P.

    2006-01-01

    Zinc deficiency has been implicated in impaired cell-mediated immunity of children with sickle cell disease (SCD). However, its influence on the expression of vascular cell-adhesion molecule-1 (VCAM-1) on endothelial cells, a protein involved in vasoocclusion, has not been previously investigated. We therefore measured (soluble) sVCAM-1 and zinc in 76 SCD children and 96 non-SCD children, mean age 7.73 years and 11.24 years, respectively. Although mean zinc levels of both groups were within the normal range (approximately 14.5 micromol/l), 14.5 % of SCD and 11% of non-SCD children (without inflammation) had levels below normal (10.7 micromol/L). Mean sVCAM-1 concentrations of SCD children (837 microg/l) were significantly higher than those of controls (627 microg/l) (p < 0.001). Differences persisted after taking into account age, hemoglobin phenotype, and inflammation (alpha-l acid glycoprotein >l g/l and C-reactive protein >10 mg/I). sVCAM-1 negatively correlated with serum (r = -0.444) and red blood cells zinc (r = -0.242, p < 0.05) but not with acute-phase proteins. Mean sVCAM-1 tended to be higher in SCD children with than in those without a history of a health problem (infection, pain crisis or were transfused; not significant). Data suggest that zinc may modulate the clinical status of SCD children through VCAM-1 expression, and zinc supplementation may be beneficial in these patients. PMID:16916123

  1. The Cerebrospinal Fluid Neurogranin/BACE1 Ratio is a Potential Correlate of Cognitive Decline in Alzheimer’s Disease

    PubMed Central

    De Vos, Ann; Struyfs, Hanne; Jacobs, Dirk; Fransen, Erik; Klewansky, Tom; De Roeck, Ellen; Robberecht, Caroline; Van Broeckhoven, Christine; Duyckaerts, Charles; Engelborghs, Sebastiaan; Vanmechelen, Eugeen

    2016-01-01

    Background: In diagnosing Alzheimer’s disease (AD), ratios of cerebrospinal fluid (CSF) biomarkers, such as CSF Aβ1-42/tau, have an improved diagnostic performance compared to the single analytes, yet, still a limited value to predict cognitive decline. Since synaptic dysfunction/loss is closely linked to cognitive impairment, synaptic proteins are investigated as candidate CSF AD progression markers. Objective: We studied CSF levels of the postsynaptic protein neurogranin and protein BACE1, predominantly localized presynaptically, and their relation to CSF total-tau, Aβ1-42, Aβ1-40, and Aβ1-38. All six analytes were considered as single parameters as well as ratios. Methods: Every ELISA involved was based on monoclonal antibodies, including the BACE1 and neurogranin immunoassay. The latter specifically targets neurogranin C-terminally truncated at P75, a more abundant species of the protein in CSF. We studied patients with MCI due to AD (n = 38) and 50 dementia due to AD patients, as well as age-matched cognitively healthy elderly (n = 20). A significant subset of the patients was followed up by clinical and neuropsychologically (MMSE) examinations for at least one year. Results: The single analytes showed statistically significant differences between the clinical groups, but the ratios of analytes indeed had a higher diagnostic performance. Furthermore, only the ratio of CSF neurogranin trunc P75/BACE1 was significantly correlated with the yearly decline in MMSE scores in patients with MCI and dementia due to AD, pointing toward the prognostic value of the ratio. Conclusion: This is the first study demonstrating that the CSF neurogranin trunc P75/BACE1 ratio, reflecting postsynaptic/presynaptic integrity, is related to cognitive decline. PMID:27392859

  2. Increased O-GlcNAc Levels Correlate with Decreased O-GlcNAcase Levels in Alzheimer Disease Brain

    PubMed Central

    Förster, Sarah; Welleford, Andrew S.; Triplett, Judy C.; Sultana, Rukhsana; Schmitz, Brigitte; Butterfield, D. Allan

    2014-01-01

    The potential role of the posttranslational modification of proteins with O-linked N-acetyl-β-D-glucosamine (O-GlcNAc) in the pathogenesis of Alzheimer disease (AD) has been studied extensively, yet the exact function of O-GlcNAc in AD remains elusive. O-GlcNAc cycling is facilitated by only two highly conserved enzymes: O-GlcNAc transferase (OGT) catalyzes the addition, while O-GlcNAcase (OGA) catalyzes the removal of GlcNAc from proteins. Studies analyzing global O-GlcNAc levels in AD brain have produced inconsistent results and the reasons for altered O-GlcNAcylation in AD are still poorly understood. In this study, we show a 1.2 fold increase in cytosolic protein O-GlcNAc modification in AD brain when compared to age-matched controls. Interestingly, O-GlcNAc changes seem to be attributable to differential modification of a few individual proteins. While our finding of augmented O-GlcNAcylation concurs with some reports, it is contrary to others demonstrating decreased O-GlcNAc levels in AD brain. These conflicting results emphasize the need for further studies providing conclusive evidence on the subject of O-GlcNAcylation in AD. We further demonstrate that, while OGT protein levels are unaffected in AD, OGA protein levels are significantly decreased to 75 % of those in control samples. In addition, augmented protein O-GlcNAc modification correlates to decreased OGA protein levels in AD subjects. While OGA inhibitors are already being tested for AD treatment, our results provide a strong indication that the general subject of O-GlcNAcylation and specifically its regulation by OGA and OGT in AD need further investigation to conclusively elucidate its potential role in AD pathogenesis and treatment. PMID:24859566

  3. Immune response in human visceral leishmaniasis: analysis of the correlation between innate immunity cytokine profile and disease outcome.

    PubMed

    Peruhype-Magalhães, V; Martins-Filho, O A; Prata, A; Silva, L de A; Rabello, A; Teixeira-Carvalho, A; Figueiredo, R M; Guimarães-Carvalho, S F; Ferrari, T C A; Correa-Oliveira, R

    2005-11-01

    We investigated the cytokine profile of cells of the innate immune response and its association with active (ACT), asymptomatic (AS) and cured (CUR) human visceral leishmaniasis (VL), as well as noninfected (NI) subjects. The frequency of cytokine-producing cells was determined after short-term in vitro incubation of whole peripheral blood samples with soluble Leishmania antigen (SLA). Our data demonstrated a predominant type 2 cytokine profile in NI and ACT. In NI, we observed an increase of IL-4+ neutrophils, IL-10+ eosinophils besides a decrease of tumour necrosis factor (TNF)-alpha+ eosinophils/monocytes. Yet in ACT, we observed an increase of IL-4+ neutrophils and natural killer (NK) cells and IL-10+ monocytes, a reduced frequency of IL-12+ and IFN-gamma+ eosinophils and lower levels of TNF-alpha+ and IL-12+ monocytes. AS presented a mixed profile, characterized by an increase of IFN-gamma+ neutrophils/eosinophils and NK cells, of IL-12+ eosinophils/monocytes, as well as increase of IL-4+ neutrophils and NK cells and IL-10+ eosinophils/monocytes. In contrast, CUR was characterized by a type 1 response with an increase of IFN-gamma+ neutrophils/eosinophils and NK cells, associated with an increase in IL-12+ monocytes. In conclusion, we show a correlation between innate immune cytokine patterns and clinical status of VL, suggesting that these cells, in addition to other factors, may contribute to the cytokine microenvironment in which Leishmania-specific T cells are primed and to disease outcome.

  4. Aerobic capacity correlates to self-assessed physical function but not to overall disease activity or organ damage in women with systemic lupus erythematosus with low-to-moderate disease activity and organ damage.

    PubMed

    Boström, C; Dupré, B; Tengvar, P; Jansson, E; Opava, C H; Lundberg, I E

    2008-02-01

    The present aim is to investigate the relationships between aerobic capacity and disease activity, organ damage, health-related quality of life (HRQL) and physical activity in 34 women with systemic lupus erythematosus (SLE) with low-to-moderate disease activity and organ damage. Mean age was 51 (SD 10) years, disease duration 17 (SD 11) years. Aerobic capacity (maximal oxygen uptake/VO2 max) was measured with a bicycle ergometer exercise test. Overall disease activity was assessed with Systemic Lupus Activity Measure (SLAM) and the modified Systemic Lupus Erythematosus-Disease Activity Index (modified SLE-DAI), overall organ damage with the Systemic Lupus International Collaboration Clinics/American College of Rheumatology-Damage Index, [SLICC/(ACR)-DI], HRQL with the 36-item Short-form health-survey (SF-36) and physical activity with a self-assessed question. The women who were low-to-moderately physically active had 89-92% (P < or = 0.001) of VO2 max predicted for sedentary women. Maximal oxygen uptake (L/min, mL/min/kg) correlated to SF-36 physical function (rs = 0.49, rs = 0.72) (P < or = 0.01), but not (rs < or = 0.25) to other HRQL scales, overall disease activity or organ damage or physical activity. The correlation between aerobic capacity and physical function and the absence of correlation between aerobic capacity and physical activity, suggest a possible disease-related factor behind the low aerobic capacity. However, with no correlation between aerobic capacity and overall disease activity and organ damage, low physical activity may contribute to the low aerobic capacity in our sample.

  5. CD38 Expression in a Subset of Memory T Cells Is Independent of Cell Cycling as a Correlate of HIV Disease Progression

    PubMed Central

    Würsch, Daniela; Ormsby, Christopher E.; Romero-Rodríguez, Dámaris P.; Olvera-García, Gustavo; Zúñiga, Joaquín; Jiang, Wei; Pérez-Patrigeon, Santiago

    2016-01-01

    In order to determine if the expression of the activation marker CD38 can correlate with HIV disease progression independently of cycling, we performed a cluster-based multivariate correlation analysis of total circulating CD4+ T cell counts and viral loads with frequencies of CD38 and Ki67 expression on CD4+ lymphocytes from patients with untreated HIV infection, stratified in maturation subpopulations, and subpopulation subsets defined by the expression of CXCR5, CXCR3, and CCR4. The frequencies of the activated phenotypes %CD38+ Ki67− and %CD38+ Ki67+ of the CXCR5− CXCR3− CCR4+ (“pre-Th2”) central memory (TCM) cell subset clustered together, comprising a significant negative correlate of total circulating CD4+ T cell counts and a positive correlate of viral load in multivariate analysis. Frequency of cycling-uncoupled CD38 expression in “pre-Th2” TCM cells was a negative correlate of total circulating CD4+ T cell counts in univariate analysis, which was not the case of their %CD38+ Ki67+. CXCR5+ CXCR3− CCR4−  TCM cells were underrepresented in patients, and their absolute counts correlated negatively with their %CD38+ Ki67− but not with their % CD38+ Ki67+. Our results may imply that CD38 expression either reflects or participates in pathogenic mechanisms of HIV disease independently of cell cycling. PMID:27064238

  6. CD38 Expression in a Subset of Memory T Cells Is Independent of Cell Cycling as a Correlate of HIV Disease Progression.

    PubMed

    Würsch, Daniela; Ormsby, Christopher E; Romero-Rodríguez, Dámaris P; Olvera-García, Gustavo; Zúñiga, Joaquín; Jiang, Wei; Pérez-Patrigeon, Santiago; Espinosa, Enrique

    2016-01-01

    In order to determine if the expression of the activation marker CD38 can correlate with HIV disease progression independently of cycling, we performed a cluster-based multivariate correlation analysis of total circulating CD4(+) T cell counts and viral loads with frequencies of CD38 and Ki67 expression on CD4(+) lymphocytes from patients with untreated HIV infection, stratified in maturation subpopulations, and subpopulation subsets defined by the expression of CXCR5, CXCR3, and CCR4. The frequencies of the activated phenotypes %CD38(+) Ki67(-) and %CD38(+) Ki67(+) of the CXCR5(-) CXCR3(-) CCR4(+) ("pre-Th2") central memory (T(CM)) cell subset clustered together, comprising a significant negative correlate of total circulating CD4(+) T cell counts and a positive correlate of viral load in multivariate analysis. Frequency of cycling-uncoupled CD38 expression in "pre-Th2" T(CM) cells was a negative correlate of total circulating CD4(+) T cell counts in univariate analysis, which was not the case of their %CD38(+) Ki67(+). CXCR5(+) CXCR3(-) CCR4(-) T(CM) cells were underrepresented in patients, and their absolute counts correlated negatively with their %CD38(+) Ki67(-) but not with their % CD38(+) Ki67(+). Our results may imply that CD38 expression either reflects or participates in pathogenic mechanisms of HIV disease independently of cell cycling.

  7. Bovine coronavirus antibody titers at weaning negatively correlate with incidence of bovine respiratory disease in the feed yard

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Bovine respiratory disease complex (BRDC) is a multifactorial disease caused by complex interactions among viral and bacterial pathogens, stressful management practices and host genetic variability. Although vaccines and antibiotic treatments are readily available to prevent and treat infection caus...

  8. Sicca symptoms in Thai patients with rheumatoid arthritis, systemic lupus erythematosus and scleroderma: a comparison with age-matched controls and correlation with disease variables.

    PubMed

    Wangkaew, Suparaporn; Kasitanon, Nuntana; Sivasomboon, Chate; Wichainun, Ramjai; Sukitawut, Waraporn; Louthrenoo, Worawit

    2006-12-01

    This study was performed to determine the prevalence of ocular and oral sicca symptoms in Thai patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma (Scl). The ocular symptoms and sign (the Schirmer's 1 test) and the oral sicca symptoms and sign (the Saxon's test) in each of 50 RA, SLE and Scl patients were compared with their age-matched controls. The correlation between the presence of sicca symptoms and signs with their clinical activity was also determined. Ocular sicca symptoms were found more common in patients with RA (38% vs 18%, p < 0.05), SLE (36% vs 14%, p < 0.05) and Scl (54% vs 16%, p < 0.01), and oral sicca symptoms were found more common in SLE (22% vs 0%, p < 0.01), and Scl (16% vs 4%, p < 0.05) than their controls. However, only RA patients had a significantly higher proportion of positive Schimer-1 test compared with their controls (p < 0.01). There was no strong correlation between sicca symptoms or signs and other clinical or laboratory variables (age, disease duration, disease activity, disease severity, and antibody to Ro and La antigens) in these three groups. In conclusion, sicca symptoms were seen significantly more common in Thai patients with connective tissue diseases, but the symptoms did not show a good correlation with the clinical and laboratory variables.

  9. Assessment of myocardial viability with delayed-enhancement MRI in coronary artery disease: A correlative study with coronary artery stenosis using digital subtraction angiography

    PubMed Central

    Zhao, Xinxiang; Zhang, Yanglin; Sun, Yong; Sun, Lin; Cai, Renhui

    2016-01-01

    The aim of the present study was to investigate the correlation between the degree of coronary artery stenosis determined by digital subtraction angiography (DSA) and infarcted segments detected by delayed enhancement magnetic resonance imaging (DE-MRI). DE-MRI and DSA were performed in 40 patients with coronary artery disease. The number of myocardial segments with infarction, the transmural extent of myocardial infarction, score of myocardial infarction by MRI, degree of coronary artery stenosis and Gensini score of the coronary artery were assessed. The correlation was analyzed using Spearman's rank correlation test. Among the 40 patients, 126 infarcted myocardial segments with a total score of 307 were found by DE-MRI; the total Gensini score for coronary artery stenosis was 587. It was observed that 81.74% of the infarcted segments were at sites with >50% coronary artery stenosis. The correlation coefficient between the Gensini score and myocardial infarction score was 0.786 (P<0.001), indicating a good correlation. However, 18.26% of myocardial infarction segments were found in patients with slight coronary artery stenosis (≤25%). A correlation was identified between DSA detected coronary artery stenosis and infarcted segments detected by DE-MRI; a higher transmural extent of myocardial infarction correlated with more severe stenosis of the coronary artery. The combined use of the two tools may facilitate accurate diagnosis.

  10. Assessment of myocardial viability with delayed-enhancement MRI in coronary artery disease: A correlative study with coronary artery stenosis using digital subtraction angiography

    PubMed Central

    Zhao, Xinxiang; Zhang, Yanglin; Sun, Yong; Sun, Lin; Cai, Renhui

    2016-01-01

    The aim of the present study was to investigate the correlation between the degree of coronary artery stenosis determined by digital subtraction angiography (DSA) and infarcted segments detected by delayed enhancement magnetic resonance imaging (DE-MRI). DE-MRI and DSA were performed in 40 patients with coronary artery disease. The number of myocardial segments with infarction, the transmural extent of myocardial infarction, score of myocardial infarction by MRI, degree of coronary artery stenosis and Gensini score of the coronary artery were assessed. The correlation was analyzed using Spearman's rank correlation test. Among the 40 patients, 126 infarcted myocardial segments with a total score of 307 were found by DE-MRI; the total Gensini score for coronary artery stenosis was 587. It was observed that 81.74% of the infarcted segments were at sites with >50% coronary artery stenosis. The correlation coefficient between the Gensini score and myocardial infarction score was 0.786 (P<0.001), indicating a good correlation. However, 18.26% of myocardial infarction segments were found in patients with slight coronary artery stenosis (≤25%). A correlation was identified between DSA detected coronary artery stenosis and infarcted segments detected by DE-MRI; a higher transmural extent of myocardial infarction correlated with more severe stenosis of the coronary artery. The combined use of the two tools may facilitate accurate diagnosis. PMID:27698725

  11. Leucocyte complement receptor 1 (CR1/CD35) transcript and its correlation with the clinical disease activity in rheumatoid arthritis patients

    PubMed Central

    Anand, D; Kumar, U; Kanjilal, M; Kaur, S; Das, N

    2014-01-01

    In view of the exaggerated complement activation in rheumatoid arthritis (RA) and significance of complement receptor 1 (CR1/CD35) as a complement regulatory protein (CRP), we aimed to determine the leucocyte-complement receptor 1 (L-CR1) transcript levels and the relationship of this protein with the clinical disease activity of RA patients. Sixty-six controls and 45 RA patients were enrolled. L-CR1 transcript levels were correlated with the levels of circulating immune complexes (CIC), C3, C4 and C3d in controls and patients and with disease activity score 28 (DAS28) in patients only. CIC levels were determined by polyethylene glycol (PEG) precipitation, C3 and C4 levels by nephlometry and C3d levels by enzyme-linked immunosorbent assay (ELISA). Eleven patients were recruited for follow-up of L-CR1 and DAS28 levels at weeks 0, 12 and 24. Appropriate statistical methods were used for the data analysis. L-CR1 (P < 0·01) transcript levels were decreased in patients compared to controls. L-CR1 levels correlated negatively with DAS28, CIC and C3d. DAS28 correlated positively with levels of CIC, C3 and C3d. Levels of CIC correlated positively with C3 and C3d. Levels of C3 correlated positively with C3d in patients and with C4 in both controls and patients. Levels of L-CR1 increased with decline in DAS28 scores in follow-up patients. Observations were statistically significant. Lower levels of L-CR1 transcript in patients compared to controls, their correlations with the levels of CIC, C3d and DAS28 at different time-points in RA patients suggest CR1 as a potential disease marker for RA. PMID:24433281

  12. [Correlation between the genetic polymorphisms of apolipoprotein M with the susceptibility to rheumatic diseases of Chinese Han populastion in Lanzhou].

    PubMed

    Li, Meiyong; Guo, Xinling; Li, Qiannan; You, Chongge

    2016-08-01

    Objective To investigate the relationship between the genetic polymorphisms of apolipoprotein M (ApoM) and the susceptibility to rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and ankylosing spondylitis (AS) among Chinese Han population in Lanzhou. Methods Primers for the two single nucleotide polymorphism (SNP) sites (rs805296 and rs805297) in ApoM gene were designed and their genotyping methods of polymerase chain reaction-high resolution melting (PCR-HRM) assay were established. Case-control studies were performed among the 599 cases of RA, 194 cases of SLE, 179 cases of AS and 273 matched healthy controls to analyze the correlations between the two SNPs and the susceptibility to rheumatic diseases. Results The genotype frequencies of rs805296 were AA 87.0%, AG 12.7%, GG 0.3% in RA cases, AA 84.5%, AG 15.0%, GG 0.5% in SLE cases, AA 91.6%, AG 7.3%, GG 1.1% in AS cases, AA 85.0%, AG 15.0%, GG 0% in healthy controls. The ones of rs805297 were GG 38.2%, GT 51.8%, TT 10.0% in RA cases, GG 44.3%, GT 45.4%, TT 10.3% in SLE cases, GG 37.4%, GT 47.5%, TT 15.1% in AS cases, GG 40.7%, GT 46.1%, TT 13.2% in healthy controls. Statistical analyses showed that only the genotype distribution of rs805296 was significantly different between the AS cases and the healthy controls. Under the dominant model, the G allele carriers of rs805296 (AG heterozygote and GG homozygote) were found to significantly decrease the risk for AS development. Conclusion The established PCR-HRM genotyping assays in the present study can successfully achieve the molecular diagnosis of the two SNPs sites (rs805296 and rs805297) from clinical samples, and the study found a significant association between the SNP of rs805296 and the susceptibility to AS among Chinese Han population in Lanzhou. PMID:27412944

  13. Harnessing the power of gene microarrays for the study of brain aging and Alzheimer's disease: statistical reliability and functional correlation.

    PubMed

    Blalock, E M; Chen, K-C; Stromberg, A J; Norris, C M; Kadish, I; Kraner, S D; Porter, N M; Landfield, P W

    2005-11-01

    During normal brain aging, numerous alterations develop in the physiology, biochemistry and structure of neurons and glia. Aging changes occur in most brain regions and, in the hippocampus, have been linked to declining cognitive performance in both humans and animals. Age-related changes in hippocampal regions also may be harbingers of more severe decrements to come from neurodegenerative disorders such as Alzheimer's disease (AD). However, unraveling the mechanisms underlying brain aging, AD and impaired function has been difficult because of the complexity of the networks that drive these aging-related changes. Gene microarray technology allows massively parallel analysis of most genes expressed in a tissue, and therefore is an important new research tool that potentially can provide the investigative power needed to address the complexity of brain aging/neurodegenerative processes. However, along with this new analytic power, microarrays bring several major bioinformatics and resource problems that frequently hinder the optimal application of this technology. In particular, microarray analyses generate extremely large and unwieldy data sets and are subject to high false positive and false negative rates. Concerns also have been raised regarding their accuracy and uniformity. Furthermore, microarray analyses can result in long lists of altered genes, most of which may be difficult to evaluate for functional relevance. These and other problems have led to some skepticism regarding the reliability and functional usefulness of microarray data and to a general view that microarray data should be validated by an independent method. Given recent progress, however, we suggest that the major problem for current microarray research is no longer validity of expression measurements, but rather, the reliability of inferences from the data, an issue more appropriately redressed by statistical approaches than by validation with a separate method. If tested using statistically

  14. Assessment of genetic correlation between bacterial cold water disease resistance and spleen index in a domesticated population of rainbow trout: identification of QTL on chromosome Omy19.

    PubMed

    Wiens, Gregory D; Vallejo, Roger L; Leeds, Timothy D; Palti, Yniv; Hadidi, Sima; Liu, Sixin; Evenhuis, Jason P; Welch, Timothy J; Rexroad, Caird E

    2013-01-01

    Selective breeding of animals for increased disease resistance is an effective strategy to reduce mortality in aquaculture. However, implementation of selective breeding programs is limited by an incomplete understanding of host resistance traits. We previously reported results of a rainbow trout selection program that demonstrated increased survival following challenge with Flavobacterium psychrophilum, the causative agent of bacterial cold water disease (BCWD). Mechanistic study of disease resistance identified a positive phenotypic correlation between post-challenge survival and spleen somatic-index (SI). Herein, we investigated the hypothesis of a genetic correlation between the two traits influenced by colocalizing QTL. We evaluated the inheritance and calculated the genetic correlation in five year-classes of odd- and even-year breeding lines. A total of 322 pedigreed families (n = 25,369 fish) were measured for disease resistance, and 251 families (n = 5,645 fish) were evaluated for SI. Spleen index was moderately heritable in both even-year (h(2)  = 0.56±0.18) and odd-year (h(2)  = 0.60±0.15) lines. A significant genetic correlation between SI and BCWD resistance was observed in the even-year line (rg  = 0.45±0.20, P = 0.03) but not in the odd-year line (rg  = 0.16±0.12, P = 0.19). Complex segregation analyses of the even-year line provided evidence of genes with major effect on SI, and a genome scan of a single family, 2008132, detected three significant QTL on chromosomes Omy19, 16 and 5, in addition to ten suggestive QTL. A separate chromosome scan for disease resistance in family 2008132 identified a significant BCWD QTL on Omy19 that was associated with time to death and percent survival. In family 2008132, Omy19 microsatellite alleles that associated with higher disease resistance also associated with increased spleen size raising the hypothesis that closely linked QTL contribute to the correlation between these

  15. Assessment of genetic correlation between bacterial cold water disease resistance and spleen index in a domesticated population of rainbow trout: identification of QTL on chromosome Omy19.

    PubMed

    Wiens, Gregory D; Vallejo, Roger L; Leeds, Timothy D; Palti, Yniv; Hadidi, Sima; Liu, Sixin; Evenhuis, Jason P; Welch, Timothy J; Rexroad, Caird E

    2013-01-01

    Selective breeding of animals for increased disease resistance is an effective strategy to reduce mortality in aquaculture. However, implementation of selective breeding programs is limited by an incomplete understanding of host resistance traits. We previously reported results of a rainbow trout selection program that demonstrated increased survival following challenge with Flavobacterium psychrophilum, the causative agent of bacterial cold water disease (BCWD). Mechanistic study of disease resistance identified a positive phenotypic correlation between post-challenge survival and spleen somatic-index (SI). Herein, we investigated the hypothesis of a genetic correlation between the two traits influenced by colocalizing QTL. We evaluated the inheritance and calculated the genetic correlation in five year-classes of odd- and even-year breeding lines. A total of 322 pedigreed families (n = 25,369 fish) were measured for disease resistance, and 251 families (n = 5,645 fish) were evaluated for SI. Spleen index was moderately heritable in both even-year (h(2)  = 0.56±0.18) and odd-year (h(2)  = 0.60±0.15) lines. A significant genetic correlation between SI and BCWD resistance was observed in the even-year line (rg  = 0.45±0.20, P = 0.03) but not in the odd-year line (rg  = 0.16±0.12, P = 0.19). Complex segregation analyses of the even-year line provided evidence of genes with major effect on SI, and a genome scan of a single family, 2008132, detected three significant QTL on chromosomes Omy19, 16 and 5, in addition to ten suggestive QTL. A separate chromosome scan for disease resistance in family 2008132 identified a significant BCWD QTL on Omy19 that was associated with time to death and percent survival. In family 2008132, Omy19 microsatellite alleles that associated with higher disease resistance also associated with increased spleen size raising the hypothesis that closely linked QTL contribute to the correlation between these

  16. Assessment of Genetic Correlation between Bacterial Cold Water Disease Resistance and Spleen Index in a Domesticated Population of Rainbow Trout: Identification of QTL on Chromosome Omy19

    PubMed Central

    Hadidi, Sima; Liu, Sixin; Evenhuis, Jason P.; Welch, Timothy J.; Rexroad, Caird E.

    2013-01-01

    Selective breeding of animals for increased disease resistance is an effective strategy to reduce mortality in aquaculture. However, implementation of selective breeding programs is limited by an incomplete understanding of host resistance traits. We previously reported results of a rainbow trout selection program that demonstrated increased survival following challenge with Flavobacterium psychrophilum, the causative agent of bacterial cold water disease (BCWD). Mechanistic study of disease resistance identified a positive phenotypic correlation between post-challenge survival and spleen somatic-index (SI). Herein, we investigated the hypothesis of a genetic correlation between the two traits influenced by colocalizing QTL. We evaluated the inheritance and calculated the genetic correlation in five year-classes of odd- and even-year breeding lines. A total of 322 pedigreed families (n = 25,369 fish) were measured for disease resistance, and 251 families (n = 5,645 fish) were evaluated for SI. Spleen index was moderately heritable in both even-year (h2 = 0.56±0.18) and odd-year (h2 = 0.60±0.15) lines. A significant genetic correlation between SI and BCWD resistance was observed in the even-year line (rg = 0.45±0.20, P = 0.03) but not in the odd-year line (rg = 0.16±0.12, P = 0.19). Complex segregation analyses of the even-year line provided evidence of genes with major effect on SI, and a genome scan of a single family, 2008132, detected three significant QTL on chromosomes Omy19, 16 and 5, in addition to ten suggestive QTL. A separate chromosome scan for disease resistance in family 2008132 identified a significant BCWD QTL on Omy19 that was associated with time to death and percent survival. In family 2008132, Omy19 microsatellite alleles that associated with higher disease resistance also associated with increased spleen size raising the hypothesis that closely linked QTL contribute to the correlation between these traits. To

  17. Use of color Doppler ultrasonography for evaluating vascularity of small intestinal lesions in Crohn's disease: correlation with endoscopic and surgical macroscopic findings.

    PubMed

    Sasaki, Tomohiko; Kunisaki, Reiko; Kinoshita, Hiroto; Yamamoto, Hisae; Kimura, Hideaki; Hanzawa, Akiho; Shibata, Naomi; Yonezawa, Hiromi; Miyajima, Eiji; Sakamaki, Kentaro; Numata, Kazushi; Tanaka, Katsuaki; Maeda, Shin

    2014-03-01

    OBJECTIVE. Ultrasonography (US) is a simple, inexpensive and minimally invasive method. We evaluated the vascularity of small intestinal lesions in Crohn's disease using color Doppler US (CD-US) and retrospectively compared them with endoscopic and surgical macroscopic findings. MATERIAL AND METHODS. In order to compare CD-US and endoscopic findings, 108 Crohn's disease patients who underwent examination of the terminal ileum by both colonoscopy and CD-US were included in the study. Vascularity was evaluated in CD-US using a semiquantitative method, the Limberg score. We analyzed correlations between Limberg score and simple endoscopic score for Crohn's disease (SES-CD), an index reflecting endoscopic activity. Scores of SES-CD 3 and higher were defined as endoscopically active. For comparison with surgical macroscopic findings, 22 Crohn's disease patients who received CD-US and subsequent iliectomies were included. Lesions with apparent open ulcers were defined as active, and those without as non-active. These findings were compared with the Limberg score. RESULTS. A substantial positive correlation was observed between Limberg scores and SES-CD (ρ = 0.709 [p < 0.001]). Notably, all 27 cases with a Limberg score of 3 or 4 were classified as endoscopically active. Compared to surgical macroscopic activity, Limberg scores of active lesions were significantly higher than those of non-active lesions (p = 0.005). In particular, all 11 cases with a Limberg score of 3 or 4 were classified as active lesions. CONCLUSION. Vascularity of small intestinal lesions of Crohn's disease evaluated by CD-US with Limberg score is well correlated with endoscopic and surgical macroscopic findings.

  18. Significance of measuring oxidative stress in lifestyle-related diseases from the viewpoint of correlation between d-ROMs and BAP in Japanese subjects.

    PubMed

    Fukui, Toshiki; Yamauchi, Kazuhiro; Maruyama, Mie; Yasuda, Tadashi; Kohno, Masakazu; Abe, Youichi

    2011-09-01

    In recent years, oxidative stress has been postulated to be an important factor in the pathogenesis and development of lifestyle-related diseases. In this study, we investigated the association between the derivatives of reactive oxygen metabolites (d-ROMs), as an index of products of reactive oxygen species (ROS), and biological antioxidant potential (BAP), as an index of antioxidant potential. We also investigated the associations between d-ROMs or BAP and the risk factors for lifestyle-related diseases or metabolic syndrome-associated factors to evaluate their usefulness in preventive medicine. There were 442 subjects who underwent health checkup examination in our facilities. In addition to standard medical checkup items, we analyzed d-ROMs, BAP, brachial-ankle pulse wave velocity, high-sensitivity C-reactive protein level and visceral fat area (VFA) visualized on a computed tomography scan. The mean d-ROM value in females was significantly higher than that in males. There was a positive correlation between the d-ROM and VFA levels. On correlation analysis, there was a negative correlation between the d-ROM and creatinine levels. As factors that influence d-ROMs, the level of VFA was selected, suggesting the significance of oxidative stress measurement with d-ROMs. In addition, there was a positive correlation between d-ROMs and BAP values. Further research is required to resolve whether increased production of ROS or the antioxidant potential that can compensate for such an increase of ROS is more important in vivo.

  19. Computer-Aided Quantification of Interstitial Lung Disease from High Resolution Computed Tomography Images in Systemic Sclerosis: Correlation with Visual Reader-Based Score and Physiologic Tests

    PubMed Central

    Salaffi, Fausto; Carotti, Marina; Bosello, Silvia; Bichisecchi, Elisabetta; Giuseppetti, Gianmarco; Ferraccioli, Gianfranco

    2015-01-01

    Objective. To evaluate the performance of a computerized-aided method (CaM) for quantification of interstitial lung disease (ILD) in patients with systemic sclerosis and to determine its correlation with the conventional visual reader-based score (CoVR) and the pulmonary function tests (PFTs). Methods. Seventy-nine patients were enrolled. All patients underwent chest high resolution computed tomography (HRCT) scored by two radiologists adopting the CoVR. All HRCT images were then analysed by a CaM using a DICOM software. The relationships among the lung segmentation analysis, the readers, and the PFTs results were calculated using linear regression analysis and Pearson's correlation. Receiver operating curve analysis was performed for determination of CaM extent threshold. Results. A strong correlation between CaM and CoVR was observed (P < 0.0001). The CaM showed a significant negative correlation with forced vital capacity (FVC) (P < 0.0001) and the single breath carbon monoxide diffusing capacity of the lung (DLco) (P < 0.0001). A CaM optimal extent threshold of 20% represented the best compromise between sensitivity (75.6%) and specificity (97.4%). Conclusions. CaM quantification of SSc-ILD can be useful in the assessment of extent of lung disease and may provide reliable tool in daily clinical practice and clinical trials. PMID:25629053

  20. [Correlation of the level of Reg3α protein in plasma with gastrointestinal acute graft-versus-host disease].

    PubMed

    Cai, Cheng-Sen; Chen, Guang-Hua; Sun, Ai-Ning; Qiao, Man; Liu, Hui-Wen; Chen, Feng; Wang, Ying; Qiu, Hui-Ying; Han, Yue; Ma, Xiao; Tang, Xiao-Wen; Jin, Zheng-Ming; Fu, Cheng-Cheng; Wu, De-Pei

    2014-06-01

    This study was purposed to explore the correlation of regenerating Islet-derived 3-alpha(Reg3α) protein level in plasma with the diagnosis and prognosis of the gastrointestinal acute graft-versus-host disease (GI-aGVHD) after all-HSCT, 103 patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) were observed in our hospital from December 2011 to December 2012. Peripheral blood samples were routinely collected at 9 d before allo-HSCT, 0 d, 14 d, 28 d after allo-HSCT as well as in aGVHD and at the 1 and 4 weeks after aGVHD therapy. The plasma concentrations of Reg3α were measured by using ELISA kit. The results indicated that among the 103 patients, 17 cases never developed aGVHD symptoms (no-aGVHD), 27 cases presented with non-aGVHD associated diarrhea, 10 cases presented with isolated skin aGVHD, 17 cases developed grades I-II GI-aGVHD, 32 cases with grades III-IV GI-aGVHD. The plasma concentrations of Reg3α in group of patients with GI-aGVHD and group of non-aGVHD diarrhea were 111.5 (54.7-180.2) and 23.9 (14.5-89.5) ng/ml respectively with significant difference (P < 0.001). The plasma concentrations of Reg3α in 17 patients of grades III-IV GI-aGVHD who experienced a complete or partial response and 7 patients who had no response to therapy at 4 weeks were 137.2(51.7-205.4) and 679.4(122.3-896.8) ng/ml respectively with the significant difference (P = 0.028). All of the patients who had no response to therapy died of aGVHD associated multiple organ failure. The area under the ROC curve was 0.902 when plasma concentration of Reg3α was set at 87.73 ng/ml. The sensitivity was 81.48% and the specificity was 82.86% when the critical value was used in diagnosis of grades III-IV GI-aGVHD. The probability of grades III-IV GI-aGVHD had statistical difference above and below 87.73 ng/ml after allo-HSCT (P < 0.001). It is concluded that the increase of plasma Reg3α level after transplantation suggests the incidence of grades III-IV GI

  1. Studies of correlation between progression of coronary artery disease, as assessed by coronary arteriography, left ventricular end-diastolic pressure, ejection fraction, and employability.

    PubMed

    Nitter-Hauge, S; Noreik, K; Simonsen, S; Storstein, O; Bjorbaek, T; Steen, A

    1977-08-01

    The occupational status of a group of 100 male patients, 35 to 68 years of age, with chronic ischaemic heart disease was studied, and related to the severity and distribution of coronary artery stenosis seen on the arteriograms and to left ventricular function. In this series of patients 31 per cent were working, 31 per cent were recorded as temporarily sick, and 38 per cent as permanently disabled. It appears that while the type of previous occupation and physical activity associated with the job were of importance, there was no correlation between employability on the one hand and severity and distribution of coronary artery disease on the other. 'Blue-collar' workers had a higher rate of unemployment than those in 'white collar' occupations, but this could not be explained by differences in severity of coronary artery disease.

  2. [Current pattern of anthracosilicosis, its complications and correlation with other diseases (evaluation of 300 legal autopsies 1977-1988)].

    PubMed

    Hartung, W; Moon, J S

    1992-10-01

    The spectrum of silicosis of coal miners has changed during the past decades. The life expectancy of the miners suffering from silicosis has been successfully adapted to that of the non-miners as a result of a consistent therapy. Morphologically, the processes involving large callosities have receded markedly; in their place, there has been an increased incidence of generalised focal dust emphysema that are difficult to differentiate clinically from the common chronic obstructive pulmonary diseases. Chronic bronchitis and emphysema are the most important concomitant pulmonary diseases; the incidence of tuberculosis is still enhanced and carcinomas of the lung are only rarely to be acknowledged as so-called carcinomas in scar tissue. Other major diseases that coincide with silicosis are seen in p.m. statistics with customary frequency of incidence (cardiovascular diseases almost 50%, malignant tumours including those of the lung about 25%, other major diseases without respiratory organs about 10%). The main problem in expertising is to differentiate the influence of these diseases from those of silicosis; expertising must employ the legally prescribed terminology. In our own investigations based on 300 postmortem expertises, death as a result of a professionally acquired disease was acknowledged in 48% of all cases of silicosis of severity grades I to III, silicosis being the sole major disease in only half of the cases and in the other cases an essential partial contributor to the cause of death. The significance of the extended generalised dust emphysema as a special type of pneumoconioses that must be classified as grave, is emphasised in contrast to previously compiled statistics.

  3. 3D Osteoarthritic Changes in TMJ Condylar Morphology Correlates with Specific Systemic and Local Biomarkers of Disease

    PubMed Central

    Cevidanes, Lucia H. S.; Walker, David; Schilling, Juan; Sugai, James; Giannobile, William; Paniagua, Beatriz; Benavides, Erika; Zhu, Hongtu; Marron, J. Steve; Jung, Bryan T.; Baranowski, David; Rhodes, Jesse; Nackley, Andrea; Lim, Pei Feng; Ludlow, John B.; Nguyen, Tung; Goncalves, Joao R.; Wolford, Larry; Kapila, Sunil; Styner, Martin

    2014-01-01

    Objective To assess 3D morphological variations and local and systemic biomarker profiles in subjects with a diagnosis of temporomandibular joint osteoarthritis (TMJ OA). Design Twenty-eight patients with long-term TMJ OA (39.9 ± 16 years), 12 patients at initial diagnosis of OA (47.4 ± 16.1 years), and 12 healthy controls (41.8 ± 12.2 years) were recruited. All patients were female and had cone beam CT scans taken. TMJ arthrocentesis and venipuncture were performed on 12 OA and 12 age-matched healthy controls. Serum and synovial fluid levels of 50 biomarkers of arthritic inflammation were quantified by protein microarrays. Shape Analysis MANCOVA tested statistical correlations between biomarker levels and variations in condylar morphology. Results Compared with healthy controls, the OA average condyle was significantly smaller in all dimensions except its anterior surface, with areas indicative of bone resorption along the articular surface, particularly in the lateral pole. Synovial fluid levels of ANG, GDF15, TIMP-1, CXCL16, MMP-3 and MMP-7 were significantly correlated with bone apposition of the condylar anterior surface. Serum levels of ENA-78, MMP-3, PAI-1, VE-Cadherin, VEGF, GM-CSF, TGFβb1, IFNγg, TNFαa, IL-1αa, and IL-6 were significantly correlated with flattening of the lateral pole. Expression levels of ANG were significantly correlated with the articular morphology in healthy controls. Conclusions Bone resorption at the articular surface, particularly at the lateral pole was statistically significant at initial diagnosis of TMJ OA. Synovial fluid levels of ANG, GDF15, TIMP-1, CXCL16, MMP-3 and MMP-7 were correlated with bone apposition. Serum levels of ENA-78, MMP-3, PAI-1, VE-Cadherin, VEGF, GM-CSF, TGFβ1, IFNγ, TNFα, IL-1α, and IL-6 were correlated with bone resorption. PMID:25278075

  4. Cardiovascular risk factors and global risk of fatal cardiovascular disease are positively correlated between partners of 802 married couples from different European countries. Report from the IMMIDIET project.

    PubMed

    Di Castelnuovo, Augusto; Quacquaruccio, Gianni; Arnout, Jozef; Cappuccio, Francesco Paolo; de Lorgeril, Michel; Dirckx, Carla; Donati, Maria Benedetta; Krogh, Vittorio; Siani, Alfonso; van Dongen, Marten C J M; Zito, Francesco; de Gaetano, Giovanni; Iacoviello, Licia

    2007-09-01

    Shared environmental factors may confer to spouses a similar risk for cardiovascular disease. We aimed at investigating in pairs the concordance in risk factors for cardiovascular disease and in global risk of cardiovascular events. In the framework of the IMMIDIET Project, married couples, recruited randomly from general practice, were studied. One thousand six hundred and four apparently healthy subjects aged 25-74 years from three different European populations were enrolled. Individual cardiovascular risks were estimated using SCORE risk equations. Age was strongly correlated within couples (r = 0.86, P < 0.0001). In multivariate model, within-pair correlation was high for social status (r = 0.49; percentage of explained variation = 24%) and percent of calories from lipids (r = 0.34; 12%). Concerning conventional metabolic risk factors, percentage of explained variation varied from 0.5% (triglycerides) to 11% (glucose). Among new risk factors, activated factor VII showed the strongest correlation (r = 0.28) and C-reactive protein the lowest (r = 0.13). Either total, coronary or non-coronary risk estimates at 10 years were strongly correlated within pairs: the risk of a member explained about two thirds of the cardiovascular risk of the partner. Spouse pairs share common lifestyle habits, common and new metabolic risk factors and the predicted global risk of cardiovascular events. If the individual risk of a person is influenced by the risk of his/her partner, decreasing the risk in a member of the pair should also decrease the risk in the partner. These concepts may have important public health consequences in targeting screening or disease prevention measures towards partners of people with cardiovascular risk.

  5. Chronic Pancreatitis Correlates With Increased Risk of Cerebrovascular Disease: A Retrospective Population-Based Cohort Study in Taiwan.

    PubMed

    Wong, Tuck-Siu; Liao, Kuan-Fu; Lin, Chi-Ming; Lin, Cheng-Li; Chen, Wen-Chi; Lai, Shih-Wei

    2016-04-01

    The aim of this study is to explore whether there is a relationship between chronic pancreatitis and cerebrovascular disease in Taiwan. Using the claims data of the Taiwan National Health Insurance Program, we identified 16,672 subjects aged 20 to 84 years with a new diagnosis of chronic pancreatitis from 2000 to 2010 as the chronic pancreatitis group. We randomly selected 65,877 subjects aged 20 to 84 years without chronic pancreatitis as the nonchronic pancreatitis group. Both groups were matched by sex, age, comorbidities, and the index year of diagnosing chronic pancreatitis. The incidence of cerebrovascular disease at the end of 2011 was measured. The multivariable Cox proportional hazards regression model was used to measure the hazard ratio (HR) and 95% confidence interval (CI) for cerebrovascular disease risk associated with chronic pancreatitis and other comorbidities. The overall incidence of cerebrovascular disease was 1.24-fold greater in the chronic pancreatitis group than that in the nonchronic pancreatitis group (14.2 vs. 11.5 per 1000 person-years, 95% CI = 1.19-1.30). After controlling for confounding factors, the adjusted HR of cerebrovascular disease was 1.27 (95% CI = 1.19-1.36) for the chronic pancreatitis group as compared with the nonchronic pancreatitis group. Woman (adjusted HR = 1.41, 95% CI = 1.31-1.51), age (every 1 year, HR = 1.04, 95% CI = 1.04-1.05), atrial fibrillation (adjusted HR = 1.23, 95% CI = 1.02-1.48), chronic kidney disease (adjusted HR = 1.48, 95% CI = 1.31-1.67), chronic obstructive pulmonary disease (adjusted HR = 1.27, 95% CI = 1.16-1.40), diabetes mellitus (adjusted HR = 1.82, 95% CI = 1.72-1.92), hypertension (adjusted HR = 1.66, 95% CI = 1.56-1.76), and peripheral atherosclerosis (adjusted HR = 1.26, 95% CI = 1.06-1.51) were other factors significantly associated with cerebrovascular disease. Chronic pancreatitis is associated with increased

  6. Correlation between infection rate of triatominies and Chagas Disease in Southwest of Bahia, Brazil: a warning sign?

    PubMed

    Silveira, Eliezer A DA; Ribeiro, Israel S; Amorim, Miguel S; Rocha, Dalva V; Coutinho, Helder S; Freitas, Leandro M DE; Tomazi, Laize; Silva, Robson A A DA

    2016-07-11

    Chagas disease, caused by the Trypanosoma cruzi, has a wide distribution in South America, and its main method of control is the elimination of triatomines. It is presented here the geographic distribution and the rate of natural infection by T. cruzi of triatomines collected and evaluated from 2008 to 2013 in southwest of Bahia. Triatomines were captured in the intradomiciliary and peridomiciliary areas of five cities located in the southwest of Bahia state, identified, and analyzed for the presence of trypanosomatids in their feces. During the study period the number of patients suspected for acute Chagas disease was recovered from the Notifiable Diseases Information System (SINAN). 8966 triatomines were captured and identified as belonging to eight species. Twenty-six presented themselves infected, being Triatoma sordida the most abundant and with the highest percentage of infection by T. cruzi. Tremedal was the city with the highest number of cases of acute Chagas' disease reported to SINAN. All cities showed triatomines infected with T. cruzi, so there is considerable risk of vectorial transmission of Chagas disease in the southwestern Bahia state, evidencing the need for vector transmission control programs and preventive surveillance measures.

  7. Correlation between infection rate of triatominies and Chagas Disease in Southwest of Bahia, Brazil: a warning sign?

    PubMed

    Silveira, Eliezer A DA; Ribeiro, Israel S; Amorim, Miguel S; Rocha, Dalva V; Coutinho, Helder S; Freitas, Leandro M DE; Tomazi, Laize; Silva, Robson A A DA

    2016-07-11

    Chagas disease, caused by the Trypanosoma cruzi, has a wide distribution in South America, and its main method of control is the elimination of triatomines. It is presented here the geographic distribution and the rate of natural infection by T. cruzi of triatomines collected and evaluated from 2008 to 2013 in southwest of Bahia. Triatomines were captured in the intradomiciliary and peridomiciliary areas of five cities located in the southwest of Bahia state, identified, and analyzed for the presence of trypanosomatids in their feces. During the study period the number of patients suspected for acute Chagas disease was recovered from the Notifiable Diseases Information System (SINAN). 8966 triatomines were captured and identified as belonging to eight species. Twenty-six presented themselves infected, being Triatoma sordida the most abundant and with the highest percentage of infection by T. cruzi. Tremedal was the city with the highest number of cases of acute Chagas' disease reported to SINAN. All cities showed triatomines infected with T. cruzi, so there is considerable risk of vectorial transmission of Chagas disease in the southwestern Bahia state, evidencing the need for vector transmission control programs and preventive surveillance measures. PMID:27411071

  8. FECAL CALPROTECTIN AND GASTROINTESTINAL (GI) PERMEABILITY CORRELATE WITH DISEASE ACTIVITY INDEX, AND HISTOLOGIC, ENDOSCOPIC, AND RADIOLOGIC FINDINGS IN CHILDREN WITH CROHN DISEASE (CD)

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Fecal calprotectin and permeability are noninvasive measures of GI inflammation and damage, respectively. However, there are scant data as to the possible association between the tests and CD disease activity in children. We hypothesized that levels of fecal calprotectin and permeability would corre...

  9. MicroRNAs Expression in the Ileal Pouch of Patients with Ulcerative Colitis Is Robustly Up-Regulated and Correlates with Disease Phenotypes

    PubMed Central

    Sherman Horev, Hadas; Elad, Hofit; Baram, Liran; Issakov, Ofer; Tulchinsky, Hagit; Pasmanik-Chor, Metsada; Shomron, Noam; Dotan, Iris

    2016-01-01

    Background Gene expression alterations are associated with disease behavior in inflammatory bowel disease (IBD). microRNAs (miRNAs) are dominant in the regulation of gene expression, and may affect IBD phenotype. Our aim was to assess mucosal miRNA expression in IBD and the correlation with intestinal inflammation. Methods We performed a large-scale analysis of ileal mucosal miRNA. Biopsies were retrieved from patients with ileal Crohn’s disease (CD), unoperated ulcerative colitis (UC) patients, UC patients after pouch surgery, and normal controls (NC). Pouch UC patients were classified as having a normal pouch (NP), chronic pouchitis (CP), and Crohn’s-like disease of the pouch (CLDP). miRNA expression was analyzed by parallel massive (next-generation) sequencing (NGS). Bioinformatics tools were applied for clustering and the detection of potential targets. Results Sixty-one subjects were recruited. The ileum of unoperated UC patients was comparable with NC. There were significant miRNA expression alterations (fold change ≥2, corrected P ≤.05) in NP (n = 6), CP (n = 40) and CLDP (n = 139), but only two expression alterations were noted in CD. More than 90% of the altered miRNAs were up-regulated, and many were predicted to be associated with significantly decreased transcripts. miRNAs alterations were generally clustered with disease phenotypes. Conclusions Ileal inflammation causes increased miRNA expression. miRNA alterations correlate with IBD phenotype, apparently by controlling the down-regulation of specific mRNAs. PMID:27536783

  10. Transforming Growth Factor Beta (TGF-β) Is a Muscle Biomarker of Disease Progression in ALS and Correlates with Smad Expression.

    PubMed

    Si, Ying; Kim, Soojin; Cui, Xiangqin; Zheng, Lei; Oh, Shin J; Anderson, Tina; AlSharabati, Mohammad; Kazamel, Mohamed; Volpicelli-Daley, Laura; Bamman, Marcas M; Yu, Shaohua; King, Peter H

    2015-01-01

    We recently identified Smads1, 5 and 8 as muscle biomarkers in human ALS. In the ALS mouse, these markers are elevated and track disease progression. Smads are signal transducers and become activated upon receptor engagement of ligands from the TGF-β superfamily. Here, we sought to characterize ligands linked to activation of Smads in ALS muscle and their role as biomarkers of disease progression. RNA sequencing data of ALS muscle samples were mined for TGF-β superfamily ligands. Candidate targets were validated by qRT-PCR in a large cohort of human ALS muscle biopsy samples and in the G93A SOD1 mouse. Protein expression was evaluated by Western blot, ELISA and immunohistochemistry. C2C12 muscle cells were used to assess Smad activation and induction. TGF-β1, 2 and 3 mRNAs were increased in ALS muscle samples compared to controls and correlated with muscle strength and Smads1, 2, 5 and 8. In the G93A SOD1 mouse, the temporal pattern of TGF-β expression paralleled the Smads and increased with disease progression. TGF-β1 immunoreactivity was detected in mononuclear cells surrounding muscle fibers in ALS samples. In muscle cells, TGF-β ligands were capable of activating Smads. In conclusion, TGF-β1, 2 and 3 are novel biomarkers of ALS in skeletal muscle. Their correlation with weakness in human ALS and their progressive increase with advancing disease in the ALS mouse suggest that they, as with the Smads, can track disease progression. These ligands are capable of upregulating and activating Smads and thus may contribute to the Smad signaling pathway in ALS muscle.

  11. Systemic endothelial activation occurs in both mild and severe malaria. Correlating dermal microvascular endothelial cell phenotype and soluble cell adhesion molecules with disease severity.

    PubMed

    Turner, G D; Ly, V C; Nguyen, T H; Tran, T H; Nguyen, H P; Bethell, D; Wyllie, S; Louwrier, K; Fox, S B; Gatter, K C; Day, N P; Tran, T H; White, N J; Berendt, A R

    1998-06-01

    Fatal Plasmodium falciparum malaria is accompanied by systemic endothelial activation. To study endothelial activation directly during malaria and sepsis in vivo, the expression of cell adhesion molecules on dermal microvascular endothelium was examined in skin biopsies and correlated with plasma levels of soluble (circulating) ICAM-1, E-selectin, and VCAM-1 and the cytokine tumor necrosis factor (TNF)-alpha. Skin biopsies were obtained from 61 cases of severe malaria, 42 cases of uncomplicated malaria, 10 cases of severe systemic sepsis, and 17 uninfected controls. Systemic endothelial activation, represented by the up-regulation of inducible cell adhesion molecules (CAMs) on endothelium and increased levels of soluble CAMs (sCAMs), were seen in both severe and uncomplicated malaria and sepsis when compared with uninfected controls. Plasma levels of sICAM-1, sVCAM-1, and sE-selectin correlated positively with the severity of malaria whereas TNF-alpha was raised nonspecifically in malaria and sepsis. Immunohistochemical evidence of endothelial activation in skin biopsies did not correlate with sCAM levels or disease severity. This indicates a background of systemic endothelial activation, which occurs in both mild and severe malaria and sepsis. The levels of sCAMs in malaria are thus not an accurate reflection of endothelial cell expression of CAMs in a particular vascular bed, and other factors must influence their levels during disease.

  12. Systemic endothelial activation occurs in both mild and severe malaria. Correlating dermal microvascular endothelial cell phenotype and soluble cell adhesion molecules with disease severity.

    PubMed Central

    Turner, G. D.; Ly, V. C.; Nguyen, T. H.; Tran, T. H.; Nguyen, H. P.; Bethell, D.; Wyllie, S.; Louwrier, K.; Fox, S. B.; Gatter, K. C.; Day, N. P.; Tran, T. H.; White, N. J.; Berendt, A. R.

    1998-01-01

    Fatal Plasmodium falciparum malaria is accompanied by systemic endothelial activation. To study endothelial activation directly during malaria and sepsis in vivo, the expression of cell adhesion molecules on dermal microvascular endothelium was examined in skin biopsies and correlated with plasma levels of soluble (circulating) ICAM-1, E-selectin, and VCAM-1 and the cytokine tumor necrosis factor (TNF)-alpha. Skin biopsies were obtained from 61 cases of severe malaria, 42 cases of uncomplicated malaria, 10 cases of severe systemic sepsis, and 17 uninfected controls. Systemic endothelial activation, represented by the up-regulation of inducible cell adhesion molecules (CAMs) on endothelium and increased levels of soluble CAMs (sCAMs), were seen in both severe and uncomplicated malaria and sepsis when compared with uninfected controls. Plasma levels of sICAM-1, sVCAM-1, and sE-selectin correlated positively with the severity of malaria whereas TNF-alpha was raised nonspecifically in malaria and sepsis. Immunohistochemical evidence of endothelial activation in skin biopsies did not correlate with sCAM levels or disease severity. This indicates a background of systemic endothelial activation, which occurs in both mild and severe malaria and sepsis. The levels of sCAMs in malaria are thus not an accurate reflection of endothelial cell expression of CAMs in a particular vascular bed, and other factors must influence their levels during disease. Images Figure 1 PMID:9626052

  13. Cerebrospinal Fluid Levels of sAPPα and sAPPβ in Lewy Body and Alzheimer's Disease: Clinical and Neurochemical Correlates.

    PubMed

    Mulugeta, Ezra; Londos, Elisabet; Hansson, Oskar; Ballard, Clive; Skogseth, Ragnhild; Minthon, Lennart; Blennow, Kaj; Zetterberg, Henrik; Aarsland, Dag

    2011-01-01

    We measured cerebrospinal fluid (CSF) levels of the soluble isoforms of amyloid precursor protein (APP; sAPPα sAPPβ) and other CSF biomarkers in 107 patients with Alzheimer's disease (AD), dementia with Lewy body dementia (DLB), Parkinson's disease dementia (PDD), and normal controls (NC) using commercial kits. DLB and PDD were combined in a Lewy body dementia group (LBD). No differences were observed in sAPPα and sAPPβ levels between the groups. Significant correlations were observed between sAPPα and sAPPβ and between sAPPβ and Mini-Mental State Examination scores in the total group analysis as well as when LBD and AD groups were analyzed separately. sAPPα and sAPPβ levels correlated with Aβ38, Aβ40, Aβ42, and Tau in the LBD group. In AD, sAPPα correlated with p-Tau and sAPPβ with Aβ40. The differential association between sAPPα and sAPPβ with Aβ and Tau species between LBD and AD groups suggests a possible relationship with the underlying pathologies in LBD and AD. PMID:21966597

  14. Expression of VSTM1-v2 Is Increased in Peripheral Blood Mononuclear Cells from Patients with Rheumatoid Arthritis and Is Correlated with Disease Activity.

    PubMed

    Wang, Dashan; Li, Yan; Liu, Yuan; He, Yan; Shi, Guixiu

    2016-01-01

    Rheumatoid arthritis (RA) is a chronic, systematic autoimmune disease that mainly affects joints and bones. Although the precise etiology is still unknown, Th17 cell is being recognized as an important mediator in pathogenesis of RA. VSTM1-v2 is a novel cytokine which has recently been reported to promote the differentiation of Th17 cells. This study is performed to study whether VSTM1-v2 can be recognized as a biomarker of RA, and is correlated to IL-17 expression. We obtained peripheral blood mononuclear cells (PBMCs) from 40 patients with RA and 40 age- and sex-matched healthy controls by standard Ficoll-Paque Plus density centrifugation. The mRNA expression levels of VSTM1-v2 and IL-17A in PBMCs were detected by real time-PCR. Disease activity parameters of RA were measured by routine methods. Our results showed that VSTM1-v2 mRNA expression in PBMCs from RA patients was significantly increased in comparison of that in healthy individuals. The VSTM1-v2 mRNA expression level was positively correlated with IL-17A mRNA expression level, DAS28, CRP and ESR, but was not correlated to RF, Anti-CCP or ANA. VSTM1-v2 might be a biomarker of RA and a novel factor in the pathogenesis of RA.

  15. Evolution to a Chronic Disease Niche Correlates with Increased Sensitivity to Tryptophan Availability for the Obligate Intracellular Bacterium Chlamydia pneumoniae

    PubMed Central

    Huston, Wilhelmina M.; Barker, Christopher J.; Chacko, Anu

    2014-01-01

    The chlamydiae are obligate intracellular parasites that have evolved specific interactions with their various hosts and host cell types to ensure their successful survival and consequential pathogenesis. The species Chlamydia pneumoniae is ubiquitous, with serological studies showing that most humans are infected at some stage in their lifetime. While most human infections are asymptomatic, C. pneumoniae can cause more-severe respiratory disease and pneumonia and has been linked to chronic diseases such as asthma, atherosclerosis, and even Alzheimer's disease. The widely dispersed animal-adapted C. pneumoniae strains cause an equally wide range of diseases in their hosts. It is emerging that the ability of C. pneumoniae to survive inside its target cells, including evasion of the host's immune attack mechanisms, is linked to the acquisition of key metabolites. Tryptophan and arginine are key checkpoint compounds in this host-parasite battle. Interestingly, the animal strains of C. pneumoniae have a slightly larger genome, enabling them to cope better with metabolite restrictions. It therefore appears that as the evolutionarily more ancient animal strains have evolved to infect humans, they have selectively become more “susceptible” to the levels of key metabolites, such as tryptophan. While this might initially appear to be a weakness, it allows these human C. pneumoniae strains to exquisitely sense host immune attack and respond by rapidly reverting to a persistent phase. During persistence, they reduce their metabolic levels, halting progression of their developmental cycle, waiting until the hostile external conditions have passed before they reemerge. PMID:24682324

  16. Modifications of the mouse bone marrow microenvironment favor angiogenesis and correlate with disease progression from asymptomatic to symptomatic multiple myeloma

    PubMed Central

    Calcinotto, Arianna; Ponzoni, Maurilio; Ria, Roberto; Grioni, Matteo; Cattaneo, Elena; Villa, Isabella; Sabrina Bertilaccio, Maria Teresa; Chesi, Marta; Rubinacci, Alessandro; Tonon, Giovanni; Bergsagel, P Leif; Vacca, Angelo; Bellone, Matteo

    2015-01-01

    While multiple myeloma (MM) is almost invariably preceded by asymptomatic monoclonal gammopathy of undetermined significance (MGUS) and/or smoldering MM (SMM), the alterations of the bone marrow (BM) microenvironment that establish progression to symptomatic disease are circumstantial. Here we show that in Vk*MYC mice harboring oncogene-driven plasma cell proliferative disorder, disease appearance associated with substantial modifications of the BM microenvironment, including a progressive accumulation of both CD8+ and CD4+ T cells with a dominant T helper type 1 (Th1) response. Progression from asymptomatic to symptomatic MM was characterized by further BM accrual of T cells with reduced Th1 and persistently increased Th2 cytokine production, which associated with accumulation of CD206+Tie2+ macrophages, and increased pro-angiogenic cytokines and microvessel density (MVD). Notably, MVD was also increased at diagnosis in the BM of MGUS and SMM patients that subsequently progressed to MM when compared with MGUS and SMM that remained quiescent. These findings suggest a multistep pathogenic process in MM, in which the immune system may contribute to angiogenesis and disease progression. They also suggest initiating a large multicenter study to investigate MVD in asymptomatic patients as prognostic factor for the progression and outcome of this disease. PMID:26155424

  17. [Effect of calcium pantothenate on vitamin B6 and C correlation in patients with hypertension and ischemic heart disease].

    PubMed

    Kalkun, D P; Artaeva, L P

    1986-01-01

    The relationship between pyridoxin and ascorbic acid was studied in patients with essential hypertension and coronary heart disease and proved to be antagonistic. To correct negative interrelations it is recommended that each of these vitamins be administered in combination with calcium pantothenate which favours their better utilization in the patient's body. PMID:3765531

  18. Correlations of Hepatic Hemodynamics, Liver Function, and Fibrosis Markers in Nonalcoholic Fatty Liver Disease: Comparison with Chronic Hepatitis Related to Hepatitis C Virus.

    PubMed

    Shigefuku, Ryuta; Takahashi, Hideaki; Nakano, Hiroyasu; Watanabe, Tsunamasa; Matsunaga, Kotaro; Matsumoto, Nobuyuki; Kato, Masaki; Morita, Ryo; Michikawa, Yousuke; Tamura, Tomohiro; Hiraishi, Tetsuya; Hattori, Nobuhiro; Noguchi, Yohei; Nakahara, Kazunari; Ikeda, Hiroki; Ishii, Toshiya; Okuse, Chiaki; Sase, Shigeru; Itoh, Fumio; Suzuki, Michihiro

    2016-01-01

    The progression of chronic liver disease differs by etiology. The aim of this study was to elucidate the difference in disease progression between chronic hepatitis C (CHC) and nonalcoholic fatty liver disease (NAFLD) by means of fibrosis markers, liver function, and hepatic tissue blood flow (TBF). Xenon computed tomography (Xe-CT) was performed in 139 patients with NAFLD and 152 patients with CHC (including liver cirrhosis (LC)). The cutoff values for fibrosis markers were compared between NAFLD and CHC, and correlations between hepatic TBF and liver function tests were examined at each fibrosis stage. The cutoff values for detection of the advanced fibrosis stage were lower in NAFLD than in CHC. Although portal venous TBF (PVTBF) correlated with liver function tests, PVTBF in initial LC caused by nonalcoholic steatohepatitis (NASH-LC) was significantly lower than that in hepatitis C virus (C-LC) (p = 0.014). Conversely, the liver function tests in NASH-LC were higher than those in C-LC (p < 0.05). It is important to recognize the difference between NAFLD and CHC. We concluded that changes in hepatic blood flow occurred during the earliest stage of hepatic fibrosis in patients with NAFLD; therefore, patients with NAFLD need to be followed carefully. PMID:27649152

  19. Correlations of Hepatic Hemodynamics, Liver Function, and Fibrosis Markers in Nonalcoholic Fatty Liver Disease: Comparison with Chronic Hepatitis Related to Hepatitis C Virus.

    PubMed

    Shigefuku, Ryuta; Takahashi, Hideaki; Nakano, Hiroyasu; Watanabe, Tsunamasa; Matsunaga, Kotaro; Matsumoto, Nobuyuki; Kato, Masaki; Morita, Ryo; Michikawa, Yousuke; Tamura, Tomohiro; Hiraishi, Tetsuya; Hattori, Nobuhiro; Noguchi, Yohei; Nakahara, Kazunari; Ikeda, Hiroki; Ishii, Toshiya; Okuse, Chiaki; Sase, Shigeru; Itoh, Fumio; Suzuki, Michihiro

    2016-01-01

    The progression of chronic liver disease differs by etiology. The aim of this study was to elucidate the difference in disease progression between chronic hepatitis C (CHC) and nonalcoholic fatty liver disease (NAFLD) by means of fibrosis markers, liver function, and hepatic tissue blood flow (TBF). Xenon computed tomography (Xe-CT) was performed in 139 patients with NAFLD and 152 patients with CHC (including liver cirrhosis (LC)). The cutoff values for fibrosis markers were compared between NAFLD and CHC, and correlations between hepatic TBF and liver function tests were examined at each fibrosis stage. The cutoff values for detection of the advanced fibrosis stage were lower in NAFLD than in CHC. Although portal venous TBF (PVTBF) correlated with liver function tests, PVTBF in initial LC caused by nonalcoholic steatohepatitis (NASH-LC) was significantly lower than that in hepatitis C virus (C-LC) (p = 0.014). Conversely, the liver function tests in NASH-LC were higher than those in C-LC (p < 0.05). It is important to recognize the difference between NAFLD and CHC. We concluded that changes in hepatic blood flow occurred during the earliest stage of hepatic fibrosis in patients with NAFLD; therefore, patients with NAFLD need to be followed carefully.

  20. Correlations of Hepatic Hemodynamics, Liver Function, and Fibrosis Markers in Nonalcoholic Fatty Liver Disease: Comparison with Chronic Hepatitis Related to Hepatitis C Virus

    PubMed Central

    Shigefuku, Ryuta; Takahashi, Hideaki; Nakano, Hiroyasu; Watanabe, Tsunamasa; Matsunaga, Kotaro; Matsumoto, Nobuyuki; Kato, Masaki; Morita, Ryo; Michikawa, Yousuke; Tamura, Tomohiro; Hiraishi, Tetsuya; Hattori, Nobuhiro; Noguchi, Yohei; Nakahara, Kazunari; Ikeda, Hiroki; Ishii, Toshiya; Okuse, Chiaki; Sase, Shigeru; Itoh, Fumio; Suzuki, Michihiro

    2016-01-01

    The progression of chronic liver disease differs by etiology. The aim of this study was to elucidate the difference in disease progression between chronic hepatitis C (CHC) and nonalcoholic fatty liver disease (NAFLD) by means of fibrosis markers, liver function, and hepatic tissue blood flow (TBF). Xenon computed tomography (Xe-CT) was performed in 139 patients with NAFLD and 152 patients with CHC (including liver cirrhosis (LC)). The cutoff values for fibrosis markers were compared between NAFLD and CHC, and correlations between hepatic TBF and liver function tests were examined at each fibrosis stage. The cutoff values for detection of the advanced fibrosis stage were lower in NAFLD than in CHC. Although portal venous TBF (PVTBF) correlated with liver function tests, PVTBF in initial LC caused by nonalcoholic steatohepatitis (NASH-LC) was significantly lower than that in hepatitis C virus (C-LC) (p = 0.014). Conversely, the liver function tests in NASH-LC were higher than those in C-LC (p < 0.05). It is important to recognize the difference between NAFLD and CHC. We concluded that changes in hepatic blood flow occurred during the earliest stage of hepatic fibrosis in patients with NAFLD; therefore, patients with NAFLD need to be followed carefully. PMID:27649152

  1. Glycated Hemoglobin (HbA1c) Correlation with Severity of Coronary Artery Disease in Non-diabetic Patients - A Hospital based Study from North-Eastern India

    PubMed Central

    Dutta, Bornali; Neginhal, Mahesh

    2016-01-01

    Introduction Glycated Hemoglobin (HbA1c) levels are predictive of cardiovascular disease and mortality in patients with diabetes mellitus, however, association of HbA1c with Coronary Artery Disease (CAD) in non-diabetics is inconsistent. Aim To evaluate the correlation between HbA1c level and severity of CAD in non-diabetic patients using SYNTAX score in a cohort of proven CAD on angiography at Gauhati Medical College, Guwahati, Assam, India, which is a major tertiary care hospital of North-Eastern India. Materials and Methods We prospectively collected data of non-diabetic patients with proven CAD on angiography from June 2014 to June 2015. Patients were divided into four groups (interquartiles) according to HbA1c levels, less than 4.8%, 4.8% to 5.1%, 5.1% to 5.6%, and 5.6% to 6.5%. Severity of CAD was assessed using SYNTAX score and the number of coronary vessels diseased. We compared different quartiles of HbA1c with regard to SYNTAX score and number of diseased vessels. Results A total of 346 patients were included in the study. Mean age was 58.1±10.4 years. Of the total 91.9% (318) were males, 44.8% (155) were hypertensives, 29.2% (101) were smokers and 34.7% (120) were dyslipidemic. We found that CAD severity by SYNTAX score as well as number of vessels involved was significantly different among quartiles (p-values <0.001 and <0.001 respectively). Increase in HbA1c level was strongly correlated with disease severity and higher SYNTAX score. A significant increase was noted in the mean number of diseased vessels (p-value <0.001) as HbA1c level increases. Age, gender, hypertension and dyslipidemia did not show significant difference among quartiles however smoking was found to be an independent predictor of severity of CAD by SYNTAX score (p <0.05). Conclusion From this clinical study, we can conclude that a significant correlation exists between HbA1c and severity of CAD by SYNTAX score as well as number of vessels involved in non- diabetes. PMID:27790487

  2. Correlation between cystatin C-based formulas, Schwartz formula and urinary creatinine clearance for glomerular filtration rate estimation in children with kidney disease

    PubMed Central

    Safaei-Asl, Afshin; Enshaei, Mercede; Heydarzadeh, Abtin; Maleknejad, Shohreh

    2016-01-01

    Introduction: Assessment of glomerular filtration rate (GFR) is an important tool for monitoring renal function. Objectives: Regarding to limitations in available methods, we intended to calculate GFR by cystatin C (Cys C) based formulas and determine correlation rate of them with current methods. Patients and Methods: We studied 72 children (38 boys and 34 girls) with renal disorders. The 24 hour urinary creatinine (Cr) clearance was the gold standard method. GFR was measured with Schwartz formula and Cys C-based formulas (Grubb, Hoek, Larsson and Simple). Then correlation rates of these formulas were determined. Results: Using Pearson correlation coefficient, a significant positive correlation between all formulas and the standard method was seen (R2 for Schwartz, Hoek, Larsson, Grubb and Simple formula was 0.639, 0.722, 0.705, 0.712, 0.722, respectively) (P<0.001). Cys C-based formulas could predict the variance of standard method results with high power. These formulas had correlation with Schwarz formula by R2 0.62-0.65 (intermediate correlation). Using linear regression and constant (y-intercept), it revealed that Larsson, Hoek and Grubb formulas can estimate GFR amounts with no statistical difference compared with standard method; but Schwartz and Simple formulas overestimate GFR. Conclusion: This study shows that Cys C–based formulas have strong relationship with 24 hour urinary Cr clearance. Hence, they can determine GFR in children with kidney injury, easier and with enough accuracy. It helps the physician to diagnosis of renal disease in early stages and improves the prognosis. PMID:27689114

  3. Joint estimation of multiple disease-specific sensitivities and specificities via crossed random effects models for correlated reader-based diagnostic data: application of data cloning.

    PubMed

    Withanage, Niroshan; de Leon, Alexander R; Rudnisky, Christopher J

    2015-12-20

    We present a model for describing correlated binocular data from reader-based diagnostic studies, where the same group of readers evaluates the presence or absence of certain diseases on binocular organs (e.g., fellow eyes) of patients. Multiple random effects are incorporated to meaningfully delineate various associations in the data including crossed random effects to account for reader-specific variability and to incorporate cross correlations. To overcome the computational complexity involved in the evaluation and maximization of the marginal likelihood, we adopt the data cloning approach, which calculates maximum likelihood estimates under the Bayesian paradigm. The bias and efficiency of the estimates are assessed in two simulation studies. We apply our model to data from a diabetic retinopathy study. PMID:26179660

  4. c-erbB-2 oncoprotein detected by automated quantitative immunocytochemistry in breast carcinomas correlates with patients' overall and disease-free survival.

    PubMed Central

    Charpin, C.; Garcia, S.; Bouvier, C.; Martini, F.; Lavaut, M. N.; Allasia, C.; Bonnier, P.; Andrac, L.

    1997-01-01

    The prognostic significance of c-erbB-2 oncoprotein overexpression detected in tumours by immunocytochemical assays (ICAs) was investigated in 148 breast carcinomas. ICAs were performed under optimal technical conditions with frozen tissue sections and included automated immunoperoxidase technique and computer-assisted analysis (densitometry) of digitized coloured microscopic images. Results of quantitative ICAs (expressed in percentages of c-erbB-2-positive surfaces and mean optical densities) were correlated with the patients' follow-up in axillary lymph node-positive (N+) and node-negative (N-) subgroups of patients. Patients' follow-up ranged from 9 months (for the first death) to 101 months (for the 121 alive patients) with a 62.5 months mean overall follow-up. It was shown that marked c-erbB-2 immunocytochemical expression in tumours (cut-off point 35%) significantly correlated with the patients' poor overall survival in N+ and in N- patients (Kaplan-Meier, log-rank test, P = 0.045 and P = 0.015). Also, marked c-erbB-2 immunohistochemical expression correlates with short disease-free (P = 0.005), recurrence-free (P = 0.048) and metastasis-free survival (P = 0.05) (Kaplan-Meier, log-rank test) in N+, but not in N- subgroups. It is concluded that in optimal conditions (automated and quantitative ICAs on frozen sections) c-erbB immunohistochemical expression is a significant prognostic indicator in terms of overall and disease-free survival. The c-erbB-2 protein prognostic significance is independent of node status in terms of overall survival, but not of disease-free survival. Images Figure 1 PMID:9184184

  5. Spatial analysis of the regional variation of hypertensive disease mortality and its socio-economic correlates in South Korea.

    PubMed

    Park, Seong-Yong; Kwak, Jin-Mi; Seo, Eun-Won; Lee, Kwang-Soo

    2016-05-31

    This paper presents a cross-sectional study based on the cause of death statistics in 2011 extracted from all 229 local governments in South Korea. The standardised hypertensive disease mortality rate (SHDMR) was defined by age- and sex-adjusted mortality by hypertensive diseases distinguished by International Classificat