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1

Successful treatment of Schamberg's disease with pentoxifylline.  

PubMed

Three patients with Schamberg's disease were treated with pentoxifylline, 300 mg daily for 8 weeks. A significant response was observed within 2 to 3 weeks. One patient had recurrence after discontinuation of this treatment; but promptly responded to resumption of therapy. No adverse effects were noted in any patients. We investigated the expression pattern of adhesion molecules in relation to perivascular accumulation of lymphocytes in specimens obtained before and after treatment with pentoxifylline in these patients. Our results suggest that pentoxifylline exerts its therapeutic effects on Schamberg's disease at the level of T-cell adherence to endothelial cells and keratinocytes. PMID:9146559

Kano, Y; Hirayama, K; Orihara, M; Shiohara, T

1997-05-01

2

Graves' disease as an uncommon cause of acute pericarditis.  

PubMed

Acute pericarditis is either dry, fibrinous or effusive, independent of its aetiology. A case is presented involving a 44-year-old man with acute pericarditis. The cause was established to be an aggravation of Graves' disease due to non-compliance with treatment. Pericarditis is an uncommon cardiac complication of Graves' disease and is associated with more recurrent episodes when not detected. Pharmacological treatment should include anti-inflammatory drugs in combination with treatment for hyperthyroidism. The specific pathophysiological link between the two conditions is still to be elucidated. PMID:24769665

Kortekaas, Kirsten A; Kortekaas, Kirsten; van der Lienden, Bas; Jong, Simone; Riezebos, Robert

2014-01-01

3

Just a sore throat? Uncommon causes of significant respiratory disease.  

PubMed

We present two uncommon underlying causes of a sore throat which, if missed or delayed in diagnosis, can lead to disastrous consequences. Our first case is of Lemierre's syndrome diagnosed in a 21-year-old man presenting with a 5-day history of sore throat, fever, right-sided pleuritic chest pain and bilateral pulmonary nodules on CT imaging. Fusobacterium necrophorum cultured from peripheral blood and an occluded left internal jugular vein on ultrasound lead to an eventual diagnosis. Our second case presents a 29-year-old woman with a 5-day history of sore throat, fever and right-sided pleuritic chest pain. A left-sided quinsy was diagnosed and aspirated and the patient was discharged home. She represented shortly with worsening pleuritic pain and was found to have a right-sided pleural effusion with descending mediastinitis originating from the tonsillar abscess. Delayed diagnosis resulted in open thoracotomy, decortication and prolonged intravenous antibiotics. PMID:23632177

Wahab, Dalia; Bichard, Julia; Shah, Anand; Mann, Bhupinder

2013-01-01

4

Recovery of uncommon bacteria from blood: association with neoplastic disease.  

PubMed Central

Table 6 is a summary of the organisms discussed with a listing of the environmental source, the endogenous source, the predisposing factors including neoplasms, and the postulated mechanisms by which the organism can gain access to the circulation. The evidence considered indicates that the entrance of one of these microorganisms into the bloodstream of a human being depends on the presence of multiplicity of predisposing factors. In the majority of cases of bacteremia due to one of these unusual organisms, two or more predisposing factors are present. Certain predisposing factors, such as cancer chemotherapy or intravenous catheterization, often provide a barrier break, while others, such as liver disease, may render the host immune system less capable of clearing organisms from the circulation. For organisms such as Campy-lobacter, Listeria, and Salmonella spp., attributes that allow the invasion of a healthy host are present and seem to be enhanced by the simultaneous presence of a predisposing condition, such as liver disease, in the host. Although somewhat fragmentary, a number of individual case reports describe bacteremia due to one of these organisms occurring weeks to years after surgery and after other therapeutic measures had effected a supposed cure of a cancer. It may be speculated that cancer patients, even after a cure, are still susceptible to bloodstream invasion by one of the aforementioned organisms by virtue of the presence of one or more predisposing metabolic, physiologic, or immunologic factors, even though these factors may be cryptic. The predominance of hematologic malignancies among cases of bacteremia due to these unusual organisms is also apparent. Although, as pointed out by Keusch (169), the reduction in the performance of immune function in hematologic malignancies compared with solid tumors is likely to be responsible, other associations of certain organisms with specific neoplasms warrant further examination. The frequency of bloodstream infections of Salmonella typhimurium and Capno-cytophaga canimorsus in Hodgkin's disease patients seems likely due to a particular mechanism which infection by these species is favored. The specific nature of these mechanisms remains to be determined. The recovery of any unusual bacterium from blood should warrant a careful consideration of the possibility of underlying disease, especially cancer. Microbiologists should advise clinicians of the unusual nature of the identified organism and provide the counsel that certain neoplastic processes, often accompanied by neutropenia, render the human host susceptible to invasion by almost any bacterium. The recovery of such organisms as C. septicum or S. bovis should prompt the clinician to aggressively seek to identify an occult neoplasm if one has not yet been diagnosed. PMID:7553569

Beebe, J L; Koneman, E W

1995-01-01

5

Bartonella henselae Infection: An Uncommon Mimicker of Autoimmune Disease  

PubMed Central

We present a case of a seven-year-old immunocompetent female patient who developed systemic symptoms mimicking an autoimmune rather than an infectious disease. The patient presented with rash, biquotidian fever, night sweats, and arthralgias. There was no antecedent history of cat contact. Investigations showed increased inflammatory markers, leukocytosis, thrombocytosis, hypercalcemia, and raised angiotensin-converting enzyme. Interferon-gamma releasing assay for tuberculosis infection was negative. Abdominal imaging demonstrated multifocal lesions of the liver and spleen (later proved to be granulomata), chest X-ray showed enlarged hilar lymph nodes, and ophthalmology review revealed uveitis. Clinical, laboratory, and imaging features pointed towards sarcoidosis. Subsequently, raised titers (IgM 1 : 32, IgG 1 : 256) against Bartonella confirmed the diagnosis of B. henselae infection. She was treated with gentamycin followed by ciprofloxacin; repeat investigations showed complete resolution of findings. The presence of hepatic and splenic lesions in children with bartonellosis is well documented. Our case, however, exhibited certain unusual findings such as the coexistence of acute ocular and systemic involvement in an immunocompetent host. Serological testing is an inexpensive and effective way to diagnose bartonellosis in immunocompetent patients; we suggest that bartonella serology is included in the baseline tests performed on children with prolonged fever even in the absence of contact with cats in countries where bartonellosis is prevalent. PMID:23424700

Maritsi, Despoina N.; Zarganis, Diagoras; Metaxa, Zoi; Papaioannou, Georgia; Vartzelis, George

2013-01-01

6

A review of uncommon cytopathologic diagnoses of pleural effusions from a chest diseases center in Turkey  

PubMed Central

Background: After pneumonia, cancer involving the pleura is the leading cause of exudative pleural effusion. Cytologic examination of pleural effusions is an important initial step in management of malignant effusions. The aim of this study is to evaluate the spectrum of uncommon malignant pleural effusions in a chest disease center in Turkey. Materials and Methods: A retrospective study of samples of pleural effusions submitted to Ataturk Chest Diseases and Chest Surgery Education and Research Hospital Department of Pathology between March 2005 and November 2008 was performed. Results: Out of a total of 4684 samples reviewed 364 (7.8%) were positive for cancer cells. Of the malignant pleural effusions 295 (81%) were classified as adenocarcinoma or carcinoma not otherwise specified (NOS). Pleural effusion specimens revealing a diagnosis other than adenocarcinoma/carcinoma NOS were: 32 (8.8%) malignant mesotheliomas, 14 (3.8%) small cell carcinomas, 13 (3.5%) hematolymphoid malignancies and 10 (2.7%) squamous cell carcinoma. Hematolymphoid malignancies included non- Hodgkin lymphoma (diffuse B large cell lymphoma, mantle cell lymphoma), multiple myeloma, chronic myeloid leukemia, and acute myeloid leukemia. Conclusions: Despite that adenocarcinoma is the most common cause of malignant pleural effusions, there is a significant number of hematological and non-hematological uncommon causes of such effusions. Cytopathologists and clinicians must keep in mind these uncommon entities in routine practice for an accurate diagnosis. PMID:21799700

Cakir, Ebru; Demirag, Funda; Aydin, Mehtap; Erdogan, Yurdanur

2011-01-01

7

A Case of Hemophagocytic Syndrome due to Tuberculosis: Uncommon Manifestation of a Common Disease  

PubMed Central

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is the manifestation of immune dysregulation. It is associated with ineffective but exaggerated immune response and infiltration of active lymphocytes and histiocytes in various organs. This devastating clinical condition has myriad of clinical and biochemical manifestations such as fever, splenomegaly, pancytopenia, hypertrygliceridemia, and hyperferritinemia. It can be either primary or secondary. Primary HLH usually presents in childhood. Secondary HLH occurs due to infection mostly viruses but other aetiologies are also important as early detection and treatment may improve survival. Hemophagocytosis due to tuberculosis is uncommon. Only handful of cases have been reported mostly in immunocompromised patients. We report a case of hemophagocytic syndrome associated with disseminated tuberculosis in an immunocompetent women highlighting early diagnosis and treatment is a demanding need in this devastating disease. PMID:25404945

Singha, Arijit; Mukherjee, Adreesh; Dasgupta, Riddhi; Das, Tapas

2014-01-01

8

A common presentation to an uncommon disease. Penile Mondor’s disease: a case report and literature review  

PubMed Central

Penile Mondor’s disease, or superficial thrombophlebitis of the dorsal vein of the penis, is a relatively uncommon but potentially anxiety-inducing self-limiting condition that should be easily recognizable by any primary care practitioner. It typically presents with a cord-like mass and pain to the dorsal penis and has a myriad of causes, including trauma, excessive sexual activity, excessive exercise, or malignancy. Although Penile Mondor’s disease is typically a clinical diagnosis, Doppler ultrasound is the initial imaging modality of choice if there is question or doubt about the diagnosis. Accurate diagnosis and reassurance about the condition’s benign and self-limiting nature assuages most patients’ fears. Treatment is primarily symptomatic but may vary depending on possible underlying disease processes. PMID:25382986

Walsh, John C; Poimboeuf, Sabré; Garvin, Daniel S

2014-01-01

9

Erdheim-Chester disease: an unusual presentation of an uncommon disease.  

PubMed

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with classic radiographic findings of patchy or diffuse osteosclerosis predominantly involving the long bones in a bilaterally symmetrical pattern. A 49-year-old woman presented with diffuse lymphadenopathy, painful skin lesions, and constitutional symptoms. Recent history was significant for a nontraumatic fracture of the tibia 3 weeks prior to admission. Physical examination and laboratory studies were notable for lower extremity pain and swelling, nodular lesions on the skin, and normocytic, normochromic anemia. Plain radiographs showed a lytic pattern of destruction with a superimposed fracture in the left proximal tibia. MRI showed focal bone marrow replacement extending from the subchondral bone to the tibial diaphysis. Excisional lymph node and skin biopsies of the lesions demonstrated a CD-68 positive, S-100 variable, and CD1a-negative histiocytic cell proliferation filling the dermis and completely replacing the sampled lymph node with an accompanying chronic inflammatory infiltrate and fibrosis, pathognomonic for ECD. We report an unusual case of ECD presenting initially as diffuse, painful lymphadenopathy, and subsequently demonstrating a lytic lesion of the tibia underlying a nontraumatic fracture. PMID:24366632

Bindra, Jasjeet; Lam, Alexander; Lamba, Ramit; VanNess, Michael; Boutin, Robert D

2014-06-01

10

Nodular fasciitis: an uncommon disease with common medical management challenges at a remote Naval Hospital.  

PubMed

Nodular fasciitis is a rare benign soft tissue lesion that is often confused with malignant sarcoma, which can make management of this pathology challenging. We present here a case of head and neck nodular fasciitis that was managed at a remote U.S. Naval Hospital with limited diagnostic and therapeutic resources. The aim of this article is to bring to light this uncommon pathology so that it can be given its due consideration in the differential diagnosis from both a clinical and pathologic perspective. However, the more important purpose of this article is to highlight the complex decision-making process that sometimes occurs when evaluating patients within a medical infrastructure that is significantly less than that of a typical military treatment facility or U.S. hospital. Young surgeons and other providers may find this discussion useful before deploying, whether it be to a remote land-based facility or to an aircraft carrier. PMID:24005559

Spinelli, Nicholas; Khorassani, Nima

2013-09-01

11

Erdheim-Chester disease: an uncommon cause of upper urinary tract obstruction.  

PubMed

Erdheim-Chester disease is a rare non-Langerhans form of systemic histiocytosis of unknown origin. We describe a 45-year-old man presenting with bilateral hydronephrosis suggestive of extrinsic urinary tract obstruction. Computed tomography revealed extensive hypodense soft tissue infiltration in the retroperitoneum surrounding the kidneys. Needle biopsy of the retroperitoneal soft tissue revealed aggregates of lipid-laden histiocytes expressing CD68 but negative for CD1a and S100 protein. The diagnosis of Erdheim-Chester disease was supported by typical radionuclide bone scinitigraphic findings. Treatment with prednisolone, sirolimus, and regular ureteric stent revision was initiated to achieve adequate urinary tract drainage. To our knowledge, this is the second patient with Erdheim-Chester disease reported in Hong Kong. A high index of suspicion is required to avoid delay in the diagnosis of this rare disease. PMID:24088590

Tsu, J H L; Yuen, S K; Cheung, H; Lee, Y W; Liu, P L

2013-10-01

12

Crohn’s disease and Takayasu’s arteritis: An uncommon association  

PubMed Central

Takayasu’s arteritis (TA) and Crohn’s disease (CD) are two rare autoimmune disorders; however some reports describe the presence of both diseases in the same patient. This finding has suggested the possibility that both diseases could share some common etiologic origin. We describe a case of a 13-year-old male affected by CD characterized by fever, diarrhea, weight loss, abdominal pain and elevation of inflammatory markers. Clinical and histological features from colonic specimens were consistent with CD. Treatment with steroids and azathioprine was started, however disease flared every time steroids were tapered. One year later, while still on treatment, he came back to our attention for dyspnea at rest and at night, tiredness and weakness. At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit. A transthoracic echocardiography showed mild aortic valve insufficiency, left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch. A computed tomography scan showed abdominal aorta thickening, dilated thoracic aorta and the presence of a thoracic aortic aneurysm. TA associated with CD was diagnosed and medical treatment with cyclophosphamide, steroids and aminosalicylic acid was started, with good clinical response at 6 mo follow-up. We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease. PMID:24124342

Taddio, Andrea; Maschio, Massimo; Martelossi, Stefano; Barbi, Egidio; Ventura, Alessandro

2013-01-01

13

Uncommon Lives  

NSDL National Science Digital Library

The Uncommon Lives series on the National Archives of Australia website takes an approach to Australian history that not only encompasses the well-known history-makers, but also lesser known people's role in shaping Australian history. One of the stated goals of the Uncommon Lives series is to show how amateur historians and researchers alike can use the archives to find biographical resources. There are five stories the visitor can discover by simply clicking on the image of the person or people next to the brief description of their story including, "Muslim Journeys", "Charles and Ruth Lane Poole", "Jessie Street", "Wolf Klaphake", and "Dhakiyarr Wirrpanda". By clicking on any choice, visitors will find each story divided into subsections. Explanatory text accompanies the thumbnails of each image and these can be expanded into a high quality image by clicking on them. Each of these stories provides a unique and compelling look into Australian history. For instance in Wolf Klaphake's story you can listen to or read the transcript of segments of the ABC radio play "A Doubtful Character" which is about Klaphake's life and in Dhakiyarr Wirrpanda's story, you can view the actual court records of his case, which was the first for an Aboriginal Australian in the High Court.

2007-01-01

14

Celiac disease and gastric hyperplastic polyps: a case series of an uncommon association.  

PubMed

Gastric polypoid lesions are found in ?1-4% of patients who undergo esophagogastroduodenoscopy. The hyperplastic lesions are considered non-neoplastic polyps that are distributed randomly in the stomach and they are usually related to chronic gastritis as a result of the exaggerated mucosal healing response. Although several conditions have been associated with celiac disease (CD), such as thyroiditis, Addison's disease, type 1 diabetes mellitus, and autoimmune hepatitis, the association with gastric polyps is rare. In this case series, we present seven newly diagnosed patients (six women) with CD and hyperplastic gastric polyps. Helicobacter pylori infection was ruled out through histology in all the patients and two out of seven had a history of occasional proton pump inhibitor use. An unusual association was found between CD and hyperplastic polyps in this case series. PMID:24901825

Galvez-Ríos, Shareni; Arano-Acua, Miguel; Meixueiro-Daza, Arturo; Grube-Pagola, Peter; Remes-Troche, José M

2014-07-01

15

A common anesthesiology procedure for a patient with an uncommon combination of diseases: a case report.  

PubMed

Administering neuraxial anesthesia to a patient with an underlying neurological disease and a combination of four other pathological disorders can be challenging. We report in this paper the case of a 45-year-old woman with neurological deficit due to ischemic brain infarct, multiple sclerosis, antiphospholipid syndrome, and ?-heterozygous thalassemia that was subjected to abdominal hysterectomy and bilateral salpingoophorectomy under epidural anesthesia for ovarian cancer. PMID:23304562

Tympa, Aliki; Hassiakos, Dimitrios; Salakos, Nikolaos; Melemeni, Aikaterini

2012-01-01

16

Pulmonary veno-occlusive disease: an uncommon cause of pulmonary hypertension  

PubMed Central

Pulmonary veno-occlusive disease (PVOD) is a rare and challenging cause of pulmonary hypertension. Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. Our case involves a healthy 21-year-old man who developed dyspnoea on exertion at an elevated altitude during deployment to Afghanistan. His work-up included an echocardiogram, a high-resolution CT scan, V/Q scan, pulmonary function tests with diffusion capacity, and a cardiac catheterisation with vasodilator challenge. Initially diagnosed with vasodilator responsive pulmonary arterial hypertension, an oral vasodilator was given with subsequent development of non-cardiogenic pulmonary oedema, thus confirming a clinical diagnosis of PVOD. He was medically stabilised with diuretic therapy, but developed progressive right-ventricular failure. For definitive treatment, he underwent a successful bilateral lung transplant. Explanted lung histology confirmed the diagnosis of PVOD. PMID:23378546

Masters, Kyle; Bennett, Steven

2013-01-01

17

Diurnal and twenty-four hour patterning of human diseases: acute and chronic common and uncommon medical conditions.  

PubMed

The symptom intensity and mortality of human diseases, conditions, and syndromes exhibit diurnal or 24 h patterning, e.g., skin: atopic dermatitis, urticaria, psoriasis, and palmar hyperhidrosis; gastrointestinal: esophageal reflux, peptic ulcer (including perforation and hemorrhage), cyclic vomiting syndrome, biliary colic, hepatic variceal hemorrhage, and proctalgia fugax; infection: susceptibility, fever, and mortality; neural: frontal, parietal, temporal, and occipital lobe seizures, Parkinson's and Alzheimer's disease, hereditary progressive dystonia, and pain (cancer, post-surgical, diabetic neuropathic and foot ulcer, tooth caries, burning mouth and temporomandibular syndromes, fibromyalgia, sciatica, intervertebral vacuum phenomenon, multiple sclerosis muscle spasm, and migraine, tension, cluster, hypnic, and paroxysmal hemicranial headache); renal: colic and nocturnal enuresis and polyuria; ocular: bulbar conjunctival redness, keratoconjunctivitis sicca, intraocular pressure and anterior ischemic optic neuropathy, and recurrent corneal erosion syndrome; psychiatric/behavioral: major and seasonal affective depressive disorders, bipolar disorder, parasuicide and suicide, dementia-associated agitation, and addictive alcohol, tobacco, and heroin cravings and withdrawal phenomena; plus autoimmune and musculoskeletal: rheumatoid arthritis, osteoarthritis, axial spondylarthritis, gout, Sjögren's syndrome, and systemic lupus erythematosus. Knowledge of these and other 24 h patterns of human pathophysiology informs research of their underlying circadian and other endogenous mechanisms, external temporal triggers, and more effective patient care entailing clinical chronopreventive and chronotherapeutic strategies. PMID:25129839

Smolensky, Michael H; Portaluppi, Francesco; Manfredini, Roberto; Hermida, Ramon C; Tiseo, Ruana; Sackett-Lundeen, Linda L; L Haus, Erhard

2014-07-18

18

Detecting Uncommon Trajectories  

Microsoft Academic Search

An effective video surveillance system relies on detection of suspicious activities. In recent times, there has been an increasing focus on detecting anomalies in human behaviour using surveillance cameras as they provide a clue to preventing breaches in security. Human behaviour can be termed as suspicious when it is uncommon in occurrences and deviates from commonly understood behaviour within a

Arnold Wiliem; Vamsi Krishna Madasu; Wageeh Boles; Prasad K. D. V. Yarlagadda

2008-01-01

19

Hoover Institution: Uncommon Knowledge  

NSDL National Science Digital Library

The Hoover Institution has placed a wide array of multimedia content online for over a decade, and recently they created a site for their "Uncommon Knowledge" program. Hosted by Hoover fellow Peter Robinson, the program features interviews with political leaders, distinguished scholars, and leading journalists. First-time visitors to the site can browse the archives by topic, date, or guest. Currently, the online archive contains programs from 1997 to 2005, along with webcasts from 2006 to the present. Recent conversations added to the site include a discussion with Shelby Steele and a talk with Michael Barone about tax reform and various health-care proposals.

2008-01-01

20

Bacterial infections: uncommon presentations  

Microsoft Academic Search

The essence of dermatology is morphology. The most important instrument in the practice of dermatology has always been, and still is, the naked eye; however, “We see only what we are ready to see, what we have been taught to see” (Jean Martin Charcot). Although most practitioners will easily correctly diagnose common bacterial skin diseases (such as cellulitis, erysipelas, impetigo,

Hagit Matz; Edith Orion; Ronni Wolf

2005-01-01

21

Uncommon and/or bizarre features of dementia.  

PubMed

This study aimed at describing uncommon or bizarre symptoms observed in patients suffering from dementia. Medline and Google scholar searches were conducted for relevant articles, chapters, and books published since 1967. Search terms used included uncommon presentation, behavioural and psychological symptoms, dementia, Alzheimer's disease, and fronto-temporal dementia. Publications found through this indexed search were reviewed for further relevant references. The uncommon symptoms are described as case-reports and there are no systematic investigations. Bizarre behaviours arising late in life should be thoroughly investigated as symptoms of dementia. PMID:24854147

Cipriani, Gabriele; Lucetti, Claudio; Danti, Sabrina; Ulivi, Martina; Nuti, Angelo

2014-05-23

22

Detection of Uncommon Deletions in Alpha-Thalassemia Using the PCR-Reverse Dot-Blot Method for Prenatal Diagnosis of Nondeletional Hemoglobin H Disease  

Microsoft Academic Search

Nondeletional hemoglobin (Hb) H disease is caused by a deletion of both ?-globin genes on one chromosome 16 and of an ?+-thalassemia point mutation on the other chromosome 16. Patients with nondeletional Hb H disease are usually more anemic, more symptomatic, and are often transfused. Counseling and prenatal diagnosis ought to be offered to couples at risk of nondeletional Hb

Yu Yang; Dong-Zhi Li

2010-01-01

23

Diabetic myonecrosis: uncommon complications in common diseases.  

PubMed

We report a case of sudden thigh pain from spontaneous quadriceps necrosis, also known as diabetic myonecrosis, in a 28-year-old patient with poorly controlled diabetes mellitus. Diabetic muscle infarction is a rare end-organ complication seen in patients with poor glycemic control and advanced chronic microvascular complications. Proposed mechanisms involve atherosclerotic microvascular occlusion, ischemia-reperfusion related injury, vasculitis with microthrombi formation, and an acquired antiphospholipid syndrome. Diabetic myonecrosis most commonly presents as sudden thigh pain with swelling and should be considered in any patient who has poorly controlled diabetes mellitus. PMID:24716004

Sran, Sisira; Sran, Manpreet; Ferguson, Nicole; Anand, Prachi

2014-01-01

24

Diabetic Myonecrosis: Uncommon Complications in Common Diseases  

PubMed Central

We report a case of sudden thigh pain from spontaneous quadriceps necrosis, also known as diabetic myonecrosis, in a 28-year-old patient with poorly controlled diabetes mellitus. Diabetic muscle infarction is a rare end-organ complication seen in patients with poor glycemic control and advanced chronic microvascular complications. Proposed mechanisms involve atherosclerotic microvascular occlusion, ischemia-reperfusion related injury, vasculitis with microthrombi formation, and an acquired antiphospholipid syndrome. Diabetic myonecrosis most commonly presents as sudden thigh pain with swelling and should be considered in any patient who has poorly controlled diabetes mellitus. PMID:24716004

Sran, Manpreet; Ferguson, Nicole

2014-01-01

25

Uncommon presentations of tinea versicolor.  

PubMed

Tinea versicolor (TV) is a common cutaneous fungal infection characterized by superficial scaling and a mild disturbance of skin pigmentation. It typically affects the chest, upper back, and shoulders. However, involvement of more unusual regions of the body such as the face and scalp, arms and legs, intertriginous sites, genitalia, areolae, and palms and soles has been reported. This report details two such cases observed at our institution: a 32-year-old woman with involvement of the popliteal fossa and a 16-year-old boy with involvement of the groin. The clinician must be aware of these variations in location and perform the appropriate diagnostic workup when lesions have the characteristic morphology of TV despite an unusual location. The etiology, pathophysiology, and epidemiology of TV are reviewed and current literature describing other instances of TV in uncommon locations is discussed. PMID:25126470

Varada, Sowmya; Dabade, Tushar; Loo, Daniel S

2014-07-01

26

Uncommon presentations of tinea versicolor  

PubMed Central

Tinea versicolor (TV) is a common cutaneous fungal infection characterized by superficial scaling and a mild disturbance of skin pigmentation. It typically affects the chest, upper back, and shoulders. However, involvement of more unusual regions of the body such as the face and scalp, arms and legs, intertriginous sites, genitalia, areolae, and palms and soles has been reported. This report details two such cases observed at our institution: a 32-year-old woman with involvement of the popliteal fossa and a 16-year-old boy with involvement of the groin. The clinician must be aware of these variations in location and perform the appropriate diagnostic workup when lesions have the characteristic morphology of TV despite an unusual location. The etiology, pathophysiology, and epidemiology of TV are reviewed and current literature describing other instances of TV in uncommon locations is discussed. PMID:25126470

Varada, Sowmya; Dabade, Tushar; Loo, Daniel S.

2014-01-01

27

Uncommon Locations and Presentations of Hydatid Cyst  

PubMed Central

Background: Hydatid disease (HD) is an ancient disease and even was known to Hippocrates. This disease involves all human parts and most common affected organs are liver and lungs. Incidence of unusual site is about 8-10%. The clinical picture depends upon the involved organs, its effects on adjacent structures, complications due to secondary infection, rupture, and anaphylaxis caused by hydatid cysts. Aim: The aim of this study was to find out incidence of unusual location of hydatid cyst in the human body. Materials and Methods: A retrospective study of HD was carried in a medical college between July 2007 and June 2012. A total 79 cases of HD were treated during this period. Information on clinical presentation and management were reviewed, and results presented as summary statistics. Results: Sixty one cases were of liver HD, and 11 were with hydatid lung disease. Fifty cases were with right lobe involvement, and rest 11 were with both lobe involvement. Out of 11 lung hydatid only one case was with bilateral lung involvement. Only eight cases of HD of uncommon locations and presentations were encountered during this period. First case presented with left hypochondriac mass as splenic HD, second with pelvic HD along with obstructive uropathy, third with non-functioning right kidney with bilateral psoas muscles HD, fourth with HD involving mesentery, fifth with pelvic pain due to right ovary HD, sixth with simultaneous involvement of the liver and right subdiaphragmatic region, seventh with HD of right inguinal region, and eighth with hydatid cyst of the left kidney. Even though, there was no mortality found in these patients, there was high morbidity. Conclusion: We conclude that Echinococcus granulosus can affect any organ in the body from head to toe, and a high suspicion of this disease is justified in endemic regions. Moreover, medical treatment should be given in the pre-operative period as well as in the post-operative period for 4-6 weeks. PMID:24971224

Sachar, S; Goyal, S; Goyal, S; Sangwan, S

2014-01-01

28

[Uncommon clinical manifestations of cutaneous leishmaniasis].  

PubMed

Cutaneous leishmaniasis is one of the most common dermatoses of the tropics. A major focus of this disease is the Syrian city of Aleppo, after which it was named in many textbooks ("Aleppo boil"). The first cases of cutaneous leishmaniasis were reported from Aleppo particularly more than 100 years ago. Syria is one of the most affected countries worldwide. This disease used to be well documented until the onset of the war in Syria in 2012, which is also supported by the numbers of the World Health Organisation (WHO), and Aleppo used to be the most affected Syrian city. Since 2012, the documentation of cutaneous leishmaniasis in Syria is no longer possible. An outbreak of cutaneous leishmaniasis has been detected especially in the besieged regions due to missing prevention measures against the sandflies and a lack of medical care. A short summary of the epidemiologic situation in Syria as well as outstanding and uncommon clinical manifestations of cutaneous leishmaniasis in Aleppo are presented. PMID:25115981

Hayani, K; Dandashli, A; Weisshaar, E

2014-10-01

29

Prerequisite for Common Learning: An Uncommon Faculty.  

ERIC Educational Resources Information Center

Perspectives on having an "uncommon" faculty teach general education courses are offered, based in part on the experience of Pennsylvania State University. An uncommon faculty members is one who is willing to spend time with students, to learn in areas outside his or her "major" field of mastered interest, to be a fellow learner instead of a…

Kiffer, Theodore E.

30

Uncommon Success: A Conversation with Brett Peiser  

ERIC Educational Resources Information Center

This article introduces a conversation with Brett Peiser, named chief executive officer of "Uncommon Schools" in July of 2012, along with the principal of North Star Academy Vailsburg Middle School, a charter school in Newark, New Jersey's West Ward. There is no doubt that "Uncommon Schools" has given thousands of…

Meyer, Peter

2014-01-01

31

Solidago multiradiata Uncommon -meadows. Blooms in  

E-print Network

asl. Flowers present starting in June. Antennaria monocephala Pussytoes ? - status of this species in July. #12;Antennaria dioeca Pussytoes Uncommon - dry rocky slopes, heaths, disturbed areas, 25 - 100 m

Jones, Ian L.

32

Catalog of Uncommon Facilities in Southern Universities.  

ERIC Educational Resources Information Center

This catalog of uncommon facilities in southern universities was developed in an effort to bring about more effective use of existing facilities, to reduce the need for duplicating certain facilities, and to increase learning and research opportunities. All of the facilities listed are available, under certain conditions, for use by students and…

Southern Regional Education Board, Atlanta, GA.

33

Uncommon Flaps for Chest Wall Reconstruction  

PubMed Central

The omentum, external oblique musculocutaneous, and thoracoepigastric flaps are uncommonly used for chest wall reconstruction. Nevertheless, awareness and knowledge of these flaps is essential for reconstructive surgeons because they fill specific niche indications or serve as lifeboats when workhorse flaps are unavailable. The current report describes the anatomic basis, technical aspects of flap elevation, and indications for these unusual flaps. PMID:22294943

Matros, Evan; Disa, Joseph J.

2011-01-01

34

Allergy to Uncommon Pets: New Allergies but the Same Allergens  

PubMed Central

The prevalence of exotic pet allergies has been increasing over the last decade. Years ago, the main allergy-causing domestic animals were dogs and cats, although nowadays there is an increasing number of allergic diseases related to insects, rodents, amphibians, fish, and birds, among others. The current socio-economic situation, in which more and more people have to live in small apartments, might be related to this tendency. The main allergic symptoms related to exotic pets are the same as those described for dog and cat allergy: respiratory symptoms. Animal allergens are therefore, important sensitizing agents and an important risk factor for asthma. There are three main protein families implicated in these allergies, which are the lipocalin superfamily, serum albumin family, and secretoglobin superfamily. Detailed knowledge of the characteristics of allergens is crucial to improvement treatment of uncommon-pet allergies. PMID:24416032

Díaz-Perales, Araceli; González-de-Olano, David; Pérez-Gordo, Marina; Pastor-Vargas, Carlos

2013-01-01

35

Allergy to uncommon pets: new allergies but the same allergens.  

PubMed

The prevalence of exotic pet allergies has been increasing over the last decade. Years ago, the main allergy-causing domestic animals were dogs and cats, although nowadays there is an increasing number of allergic diseases related to insects, rodents, amphibians, fish, and birds, among others. The current socio-economic situation, in which more and more people have to live in small apartments, might be related to this tendency. The main allergic symptoms related to exotic pets are the same as those described for dog and cat allergy: respiratory symptoms. Animal allergens are therefore, important sensitizing agents and an important risk factor for asthma. There are three main protein families implicated in these allergies, which are the lipocalin superfamily, serum albumin family, and secretoglobin superfamily. Detailed knowledge of the characteristics of allergens is crucial to improvement treatment of uncommon-pet allergies. PMID:24416032

Díaz-Perales, Araceli; González-de-Olano, David; Pérez-Gordo, Marina; Pastor-Vargas, Carlos

2013-01-01

36

A Content Analysis of Cognitive and Affective Uses of Patient Support Groups for Rare and Uncommon Vascular Diseases: Comparisons of May Thurner, Thoracic Outlet, and Superior Mesenteric Artery Syndrome.  

PubMed

Rare disease patients are the predominant group of patients who are now connecting online to patient support groups, yet research on their uses of support groups has received little attention. This is a content analysis of three vascular diseases of differing degrees of rarity. Wall posts from Facebook patient support groups for May Thurner syndrome, thoracic outlet syndrome, and superior mesenteric artery syndrome were analyzed over a period of two years. Using Uses and Gratifications as the theoretical framework, the study purpose was to assess how variations in health condition and rarity of condition affect online support group user needs. Results indicated common main cognitive and affective uses across conditions, indicating a consistent pattern of needs communicated by all patients. However, there were nuanced differences in subcategories of cognitive and affective uses between the most and least rare disorders, which inform areas for tailored support mechanisms. Additionally, these vascular patients used their respective support groups primarily for cognitive reasons, especially for the rarest conditions, which informs of basic medical informational needs these patients face related to tests, treatment, surgery, and diagnoses. PMID:24877701

Walker, Kimberly K

2014-05-30

37

An uncommon cause of transient neurological dysfunction.  

PubMed

Transient neurological dysfunction may be associated with uncommon disorders and should prompt consideration of a broad differential diagnosis when assessing patients with episodic symptoms. The most common causes of transient neurological dysfunction include transient ischemic attack (TIA), seizure disorder, and migraine and its variants. However, underlying unusual pathophysiological processes such as brain tumors can also cause transient neurological dysfunction. Here we present a case of a 68-year-old male with oligodendroglial gliomatosis cerebri (OGC) who presented with TIA-like symptoms. Brain magnetic resonance imaging revealed multiple diffuse T2 hyperintensities within the white and gray matter. Magnetic resonance spectroscopy was suggestive of gliomatosis cerebri and was particularly helpful in this case. The diagnosis of OGC was confirmed by histopathology and molecular genetic studies on brain biopsy tissue. In this report, we discuss the clinical and radiological characteristics of OGC and highlight the unusual presentation of this case. PMID:24982718

Farooq, Muhammad U; Bhatt, Archit; Chang, Howard T

2014-07-01

38

An Uncommon Cause of Transient Neurological Dysfunction  

PubMed Central

Transient neurological dysfunction may be associated with uncommon disorders and should prompt consideration of a broad differential diagnosis when assessing patients with episodic symptoms. The most common causes of transient neurological dysfunction include transient ischemic attack (TIA), seizure disorder, and migraine and its variants. However, underlying unusual pathophysiological processes such as brain tumors can also cause transient neurological dysfunction. Here we present a case of a 68-year-old male with oligodendroglial gliomatosis cerebri (OGC) who presented with TIA-like symptoms. Brain magnetic resonance imaging revealed multiple diffuse T2 hyperintensities within the white and gray matter. Magnetic resonance spectroscopy was suggestive of gliomatosis cerebri and was particularly helpful in this case. The diagnosis of OGC was confirmed by histopathology and molecular genetic studies on brain biopsy tissue. In this report, we discuss the clinical and radiological characteristics of OGC and highlight the unusual presentation of this case. PMID:24982718

Bhatt, Archit; Chang, Howard T.

2014-01-01

39

Unusual Development of Iatrogenic Complex, Mixed Biliary and Duodenal Fistulas Complicating Roux-en-Y Antrectomy for Stenotic Peptic Disease of the Supraampullary Duodenum Requiring Whipple Procedure: An Uncommon Clinical Dilemma  

PubMed Central

Complex fistulas of the duodenum and biliary tree are severe complications of gastric surgery. The association of duodenal and major biliary fistulas occurs rarely and is a major challenge for treatment. They may occur during virtually any kind of operation, but they are more frequent in cases complicated by the presence of difficult duodenal ulcers or cancer, with a mortality rate of up to 35%. Options for treatment are many and range from simple drainage to extended resections and difficult reconstructions. Conservative treatment is the choice for well-drained fistulas, but some cases require reoperation. Very little is known about reoperation techniques and technical selection of the right patients. We present the case of a complex iatrogenic duodenal and biliary fistula. A 42-year-old Caucasian man with a diagnosis of postoperative peritonitis had been operated on 3 days earlier; an antrectomy with a Roux-en-Y reconstruction for stenotic peptic disease was performed. Conservative treatment was attempted with mixed results. Two more operations were required to achieve a definitive resolution of the fistula and related local complications. The decision was made to perform a pancreatoduodenectomy with subsequent reconstruction on a double jejunal loop. The patient did well and was discharged on postoperative day 17. In our experience pancreaticoduodenectomy may be an effective treatment of refractory and complex iatrogenic fistulas involving both the duodenum and the biliary tree. PMID:21103208

Polistina, Francesco A.; Costantin, Giorgio; Settin, Alessandro; Lumachi, Franco; Ambrosino, Giovanni

2010-01-01

40

Uncommon Sense - The Heretical Nature of Science  

NASA Astrophysics Data System (ADS)

Most people believe that science arose as a natural end-product of our innate intelligence and curiosity, as an inevitable stage in human intellectual development. But physicist and educator Alan Cromer disputes this belief. Cromer argues that science is not the natural unfolding of human potential, but the invention of a particular culture, Greece, in a particular historical period. Indeed, far from being natural, scientific thinking goes so far against the grain of conventional human thought that if it hadn't been discovered in Greece, it might not have been discovered at all.In Uncommon Sense , Alan Cromer develops the argument that science represents a radically new and different way of thinking. Using Piaget's stages of intellectual development, he shows that conventional thinking remains mired in subjective, "egocentric" ways of looking at the world--most people even today still believe in astrology, ESP, UFOs, ghosts and other paranormal phenomena--a mode of thought that science has outgrown. He provides a fascinating explanation of why science began in Greece, contrasting the Greek practice of debate to the Judaic reliance on prophets for acquiring knowledge. Other factors, such as a maritime economy and wandering scholars (both of which prevented parochialism) and an essentially literary religion not dominated by priests, also promoted in Greece an objective, analytical way of thinking not found elsewhere in the ancient world. He examines India and China and explains why science could not develop in either country. In China, for instance, astronomy served only the state, and the private study of astronomy was forbidden. Cromer also provides a perceptive account of science in Renaissance Europe and of figures such as Copernicus, Galileo, and Newton. Along the way, Cromer touches on many intriguing topics, arguing, for instance, that much of science is essential complete; there are no new elements yet to be discovered. He debunks the vaunted SETI (Search for Extraterrestrial Intelligence) project, which costs taxpayers millions each year, showing that physical limits--such as the melting point of metal--put an absolute limit on the speed of space travel, making trips to even the nearest star all but impossible. Finally, Cromer discusses the deplorable state of science education in America and suggests several provocative innovations to improve high school education, including a radical proposal to give all students an intensive eighth and ninth year program, eliminating the last two years of high school.Uncommon Sense is an illuminating look at science, filled with provocative observations. Whether challenging Thomas Kuhn's theory of scientific revolutions, or extolling the virtues of Euclid's Elements , Alan Cromer is always insightful, outspoken, and refreshingly original.

Cromer, Alan

1995-08-01

41

[Merkel cell carcinoma: an uncommon neuroendocrine cancer].  

PubMed

Merkel cell carcinoma (MCC), firstly described by Torker in 1972, is an uncommon and aggressive neuroendocrine cancer of the skin. MCC tends to recur and precociously spread to lymph nodes. Five-year survival rate is between 35 and 75%. In literature there are not univocal criteria regarding the diagnosis and therapy of MCC, probably due to its rarity. Surgery plays an important role in the therapeutic strategy of this cancer. Surgical excision must be wide and guarantee at least 2-3 cm of free tumor margins. Sentinel lymph node biopsy is useful to identify those patients in which extensive lymph node dissection and/or adjuvant therapies (radio- and/or chemotherapy) are advisable. We hereby report a case of MCC of the left arm in a 48 year-old male. A wide excision was performed with sentinel lymph node biopsy that did not show any metastasis. Adjuvant radio therapy was administered. The patient was healthy at one year follow-up. PMID:19505415

Negro, P; Gossetti, F; Brugiotti, C; Viarengo, M A; Vitolo, D; D'Amore, L

2009-05-01

42

Syntheses and biological activities of daunorubicin analogs with uncommon sugars  

Microsoft Academic Search

To study the effects of the sugar structure on the activity of anthracycline against cancer cells, six daunorubicin analogs containing different uncommon sugars were synthesized. Their cytotoxicities were tested against colon cancer cells by MTS assay. The results showed that the aglycon without sugar moiety has 70–100-fold lower activity against cancer cells than daunorubicin derivatives with various uncommon sugars. It

Lizhi Zhu; Xianhua Cao; Wenlan Chen; Guisheng Zhang; Duxin Sun; Peng George Wang

2005-01-01

43

Pyramid heart: uncommon sequel of a common disease.  

PubMed

A 40-year-old woman presented 19 years after ring-annuloplastyfor rheumatic mitral regurgitation. Long-standing rheumatic heartdisease resulted in an unusual finding on her chest radiograph. PMID:22964650

Patil, Nikhil Prakash; Katti, Karuna; Yadav, Niraj; Satsangi, Deepak Kumar

2015-01-01

44

Unilateral nevoid acanthosis nigricans: Uncommon variant of a common disease  

PubMed Central

Acanthosis nigricans (AN) is a fairly common dermatosis characterized by hyperpigmented velvety plaques, having a predilection for the intertriginous areas. We herein present a case of unilateral nevoid acanthosis nigricans over the left lateral chest, in an adult male. The rarity of documentation of this entity in the world literature prompted us to report the case. PMID:25506563

Das, Anupam; Bhattacharya, Sabari; Kumar, Piyush; Gayen, Tirthankar; Roy, Kunal; Das, Nilay K.; Gharami, Ramesh C.

2014-01-01

45

Unilateral nevoid acanthosis nigricans: Uncommon variant of a common disease.  

PubMed

Acanthosis nigricans (AN) is a fairly common dermatosis characterized by hyperpigmented velvety plaques, having a predilection for the intertriginous areas. We herein present a case of unilateral nevoid acanthosis nigricans over the left lateral chest, in an adult male. The rarity of documentation of this entity in the world literature prompted us to report the case. PMID:25506563

Das, Anupam; Bhattacharya, Sabari; Kumar, Piyush; Gayen, Tirthankar; Roy, Kunal; Das, Nilay K; Gharami, Ramesh C

2014-11-01

46

Erythema nodosum - a review of an uncommon panniculitis.  

PubMed

Panniculitis, inflammation of the subcutaneous fat, is a relatively uncommon condition that usually presents with inflammatory nodules or plaques. Erythema nodosum (EN) is clinically the most frequent form of panniculitis and is considered a reactive process that may be triggered by a wide variety of stimuli. Whilst up to 55% of EN is considered idiopathic, the most common causes include infections, drugs, systemic illnesses such as sarcoidosis and inflammatory bowel disease, pregnancy, and malignancy. EN typically presents in the teens and 20s, and is seen more commonly in females. It is often preceded by a non-specific prodrome of one to three weeks, which may include fever, malaise, and symptoms of an upper respiratory tract infection. Cutaneous lesions then follow, typically localized on the extensor aspect of the limbs. The lesions are painful rounded or oval, slightly raised, non-ulcerative red nodules. The exact pathogenesis of EN is not understood, although is thought to result from deposition of immune complexes in the venules of the septae in subcutaneous fat, causing a neutrophilic panniculitis. The classical histopathological picture is of a septal panniculitis without vasculitis. However, the pathological features vary with the chronology of the lesions. Even without specific therapy for a causative condition, EN typically resolves without treatment. Therefore, symptomatic support is adequate for the majority of patients. PMID:24746312

Blake, Tristan; Manahan, Melissa; Rodins, Karl

2014-01-01

47

Uncommon CT Findings in Relapsing Polychondritis  

Microsoft Academic Search

Summary: Relapsing polychondritis is a rare inflammatory disorder of cartilage with well-established clinical features and imaging characteristics. Abnormal calcification and erosion of cartilaginous structures are the traditional ra- diographic findings. As with any disease, aberrancies of the expected clinical presentation may lead to a delayed (or missed) diagnosis. We discuss a rare case of relapsing polychondritis in which the diagnosis

Laura E. Faix; Barton F. Branstetter

48

Arsenic Compound Improves Survival of Adults with Uncommon Form of Leukemia  

Cancer.gov

Positive results of a phase III cancer clinical trial in an uncommon form of leukemia were released today. The results showed that adult patients with previously untreated acute promyelocytic leukemia who had standard chemotherapy to induce remission of their disease, and then received the chemotherapy drug arsenic trioxide to maintain remission, had a significantly better event-free survival (more patients free of leukemia) and better overall survival than those who received only standard chemotherapy. The trial was sponsored by the NCI, and was led by one of its Cooperative Clinical Trials Groups -- the Cancer and Leukemia Group B.

49

Clinical and Radiologic Review of Uncommon Cause of Profound Iron Deficiency Anemia: Median Arcuate Ligament Syndrome  

PubMed Central

Median arcuate ligament syndrome is an anatomic and clinical entity characterized by dynamic compression of the proximal celiac artery by the median arcuate ligament, which leads to postprandial epigastric pain, vomiting, and weight loss. These symptoms are usually nonspecific and are easily misdiagnosed as functional dyspepsia, peptic ulcer disease, or gastropathy. In this report, we presented a 72-year-old male patient with celiac artery compression syndrome causing recurrent abdominal pain associated with gastric ulcer and iron deficiency anemia. This association is relatively uncommon and therefore not well determined. In addition, we reported the CT angiography findings and three-dimensional reconstructions of this rare case. PMID:25053902

Asil, K?yasettin; Aksoy, Yakup Ersel; Tatl? Ayhan, Laçin

2014-01-01

50

Thoracoscopic surgery for pulmonary oncocytoma, an uncommon neoplasm.  

PubMed

Oncocytoma is an uncommon neoplasm with a characteristic histologic feature of abundant eosinophilic granular cytoplasm. It almost always occurs in kidney, thyroid, or salivary gland. Pulmonary oncocytoma is a rare pulmonary neoplasm, with fewer than 10 cases reported so far in the available English literature. We encountered one such case that was successfully managed by thoracoscopic lobectomy. PMID:24523615

Zhang, B; Jin, J; Ye, Z; Zheng, H

2014-02-01

51

Thoracoscopic surgery for pulmonary oncocytoma, an uncommon neoplasm  

PubMed Central

Oncocytoma is an uncommon neoplasm with a characteristic histologic feature of abundant eosinophilic granular cytoplasm. It almost always occurs in kidney, thyroid, or salivary gland. Pulmonary oncocytoma is a rare pulmonary neoplasm, with fewer than 10 cases reported so far in the available English literature. We encountered one such case that was successfully managed by thoracoscopic lobectomy. PMID:24523615

Zhang, B.; Jin, J.; Ye, Z.; Zheng, H.

2014-01-01

52

Uncommon aetiological agents of catheter-related bloodstream infections.  

PubMed

SUMMARY The clinical and microbiological characteristics of catheter-related bloodstream infection (CR-BSI) due to uncommon microorganisms was assessed in a retrospective case-control study over a 9-year period in a tertiary teaching hospital. Uncommon microorganisms were defined as those representing <0·5% of all CR-BSI. Diagnosis of CR-BSI required that the same microorganism was grown from at least one peripheral venous blood culture and a catheter tip culture. Thirty-one episodes of CR-BSI were identified due to 13 different genera and these accounted for 2·3% of all CR-BSI in the hospital. Although these infections were not associated with increased mortality, they occurred in patients with more severe underlying conditions who were receiving prolonged antibiotic therapy. PMID:24887020

Reigadas, E; Rodríguez-Créixems, M; Sánchez-Carrillo, C; Martín-Rabadán, P; Bouza, E

2014-06-01

53

The gallbladder: uncommon gallbladder conditions and unusual presentations of the common gallbladder pathological processes.  

PubMed

This article reviews a spectrum of gallbladder conditions that are either uncommon or represent unusual manifestations of common diseases. These conditions are divided into four major categories: (a) congenital anomalies and normal variants including duplication, ectopia, and lymphangioma; (b) inflammatory processes and stone-related diseases and complications including adenomyomatosis, emphysematous cholecystitis, xanthogranulomatous cholecystitis, gangrenous and hemorrhagic cholecystitis, perforation, gallstone ileus, and Bouveret and Mirizzi syndromes; (c) gallbladder neoplasms including adenocarcinoma with associated porcelain gallbladder, squamous cell carcinoma, lymphoma, melanoma, and neurofibroma. A thorough understanding of the imaging characteristics of each condition can help the radiologist to make a timely and accurate diagnosis, thus avoiding potentially harmful delays in patient management and decreasing morbidity and mortality rates. PMID:25063238

Revzin, Margarita V; Scoutt, Leslie; Smitaman, Edward; Israel, Gary M

2015-02-01

54

Necrotizing sarcoid granulomatosis with an uncommon manifestation: clinicopathological features and review of literature.  

PubMed

We report a rare case of an incidental diagnosis of necrotizing sarcoid granulomatosis (NSG) in a 60-y-old non-smoking male. The patient was admitted to the hospital for sudden back pain. Chest x-ray revealed areas of parenchymal consolidation and high-resolution computed tomography demonstrated a pulmonary nodular pattern with no lymph node enlargement. All laboratory and pulmonary function tests were normal. Bronchoscopy with bronchoalveolar lavage showed no sign of infection or specific inflammation. The diagnosis of NSG was made by histopathological examination of a surgical lung biopsy and by excluding other causes of granulomatous disease. In paucisymptomatic/asymptomatic patients, as in our case, therapy is not necessary, with a good prognosis and complete recovery. NSG is a rare systemic disease similar to sarcoidosis and Wegener's granulomatosis with a benign clinical course and should always be considered for patients with nodular pulmonary lesions even with subclinical or uncommon features. PMID:24327742

Giraudo, Chiara; Nannini, Nazarena; Balestro, Elisabetta; Meneghin, Alessia; Lunardi, Francesca; Polverosi, Roberta; Calabrese, Fiorella

2014-09-01

55

Metabolic Bone Diseases  

Microsoft Academic Search

\\u000a The current chapter provides an overview of the important imaging features observed in metabolic bone diseases [1, 2]. Common and uncommon imaging findings observed in insufficiency stress fractures are reviewed and illustrated.

BrunoVande Berg; Frederic Lecouvet; Paolo Simoni; Jacques Malghem

56

Acute Esophageal Necrosis: An Uncommon Cause of Hematemesis  

PubMed Central

Acute esophageal necrosis or black esophagus is an uncommon clinical entity, diagnosed at the upper gastrointestinal endoscopy with the presence of strikingly black necrotic esophagus. Very often no definite etiology will be identified even though a large list of potential associations has been postulated. Upper gastrointestinal bleeding is the most common clinical presentation, others being epigastric pain, retrosternal chest discomfort and dysphagia. Only about a hundred cases of acute esophageal necrosis have been described in medical literature till this date. We report a case of acute esophageal necrosis in an elderly female who had presented with hematemesis. PMID:25170416

Zacharia, George Sarin; Sandesh, K; Ramachandran, TM

2014-01-01

57

Common variable immunodeficiency disorder - An uncommon cause for bronchiectasis  

PubMed Central

Bronchiectasis continues to be a common respiratory problem of varied etiology. Common variable immunodeficiency disorder (CVID) is an uncommon cause for bronchiectasis. However, the prevalence of bronchiectasis remains very high in patients with CVID. This remains largely an underdiagnosed entity as primary immunodeficiency is not suspected in adults as a cause of bronchiectasis and hence, serum immunoglobulin (Ig) levels are not measured routinely. In addition to bronchiectasis, patients with CVID usually present with various extrapulmonary symptoms. I report here a case of young man who presented with bronchiectasis and multisystem complains who was diagnosed as CVID. PMID:25378851

Panigrahi, Manoj Kumar

2014-01-01

58

Solar Elastosis in Its Papular Form: Uncommon, Mistakable  

PubMed Central

Solar elastosis is a degenerative condition of elastic tissue in the dermis due to prolonged sun exposure. There are a variety of clinical manifestations of solar elastosis. In its most common form, solar elastosis manifests as yellow, thickened, coarsely wrinkled skin. We report two uncommon cases of severe solar elastosis with a papular morphology. Its presentation can closely mimic a host of cutaneous disorders and thus, although it is helpful to be cognizant of this entity, it is still crucial to biopsy these lesions to avoid missing a more sinister condition. PMID:24926253

Heng, Jun Khee; Aw, Derrick Chen Wee; Tan, Kong Bing

2014-01-01

59

Primary Ewing's sarcoma of cervical vertebra: An uncommon presentation  

PubMed Central

Ewing's sarcoma is a malignant primary bone tumor primarily seen in the long bones. Primary Ewing's sarcoma of the cranium is quite uncommon occurring in 1% of the cases. We report the occurrence of this rare lesion in a 24-year-old male presenting with progressively increasing swelling in left mastoid region mimicking a mastoid abscess which was later diagnosed on Fine needle aspiration cytology (FNAC) as a small round cell tumor as Ewing's sarcoma. Contrast enhanced computed tomography (CECT) revealed a typical moth eaten appearance in the first and second cervical vertebra. PMID:25126127

Chhabra, Sonia; Singh, Sunita; Sethi, Divya; Mahapatra, Qury Sabita

2014-01-01

60

European and German food legislation facing uncommon foodstuffs.  

PubMed

In Europe, uncommon foodstuff (UFS, i.e., traditional foods from specific European regions and uncommon ethnic foods from non-EU countries) have been contributing to a diversification of the food supply. E-commerce and specialized retail shops are the main sources for UFS. This article discusses the legal bases for UFS introduction and evaluation. By means of 35 representative UFS, this article analyses the possibilities of trade and veterinary inspection of these products in Germany, comparing European Union and national food legislation with the many idiosyncrasies the UFS presents. Conservatory legislation bans the trade with endangered species (primates, cetaceans, songbirds), but for many other species, this is a complex matter that may ban only subpopulations from trade. Although introduction of legal UFS is regulated (yet complicated), the lack of appropriate definitions, intra-European trade harmonization, and of sufficient scientific knowledge hampers a satisfactory evaluation of many UFSs, for example, reptile meat or terrestrial insects. In these cases, official inspection would only be very basic. PMID:23768143

Grabowski, Nils Th; Klein, Günter; López, Antonio Martínez

2013-01-01

61

Venous thromboembolism at uncommon sites in neonates and children.  

PubMed

We retrospectively analyzed the data of 24 children (whereof 11 neonates), with non-central venous line-related and nonmalignancy-related venous thromboembolism (VTE) at uncommon sites, referred to our Unit from January 1999 to January 2012. Thirty patients who also suffered deep vein thrombosis, but in upper/low extremities, were not included in the analysis. The location of rare site VTE was: portal (n=7), mesenteric (n=2) and left facial vein (n=1), spleen (n=3), lung (n=3), whereas 10 neonates developed renal venous thrombosis. The majority of patients (91.7%) had at least 1 risk factor for thrombosis. Identified thrombophilic factors were: antiphospholipid antibodies (n=2), FV Leiden heterozygosity (n=6), MTHFR C677T homozygosity (n=4), protein S deficiency (n=2), whereas all neonates had age-related low levels of protein C and protein S. All but 6 patients received low-molecular-weight heparin, followed by warfarin in 55% of cases, for 3 to 6 months. Prolonged anticoagulation was applied in selected cases. During a median follow-up period of 6 years, the clinical outcome was: full recovery in 15 patients, evolution to both chronic portal hypertension and esophageal varices in 2 children, and progression to renal failure in 7 of 10 neonates. Neonates are greatly vulnerable to complications after VTE at uncommon sites, particularly renal. Future multicentre long-term studies on neonatal and pediatric VTE at unusual sites are considered worthwhile. PMID:24517966

Pergantou, Helen; Avgeri, Maria; Komitopoulou, Anna; Xafaki, Panagiota; Kapsimali, Zoey; Mazarakis, Michail; Adamtziki, Eftychia; Platokouki, Helen

2014-11-01

62

Lobulated capillary haemangioma: a common lesion in an uncommon site  

PubMed Central

Pyogenic granuloma (PG) is a well-known localised granulation tissue overgrowth. It remains an aetiopathological enigma, with trauma, inflammatory and infectious agents being the suspected causative factors. It is a relatively common benign mucocutaneous lesion occurring intraorally or extraorally and is more common in women in the second decade of their lives than in men. Although it is a common lesion it may present with varying clinical features that sometimes may mimic more serious lesions such as malignancies. The clinical diagnosis of such lesion can be quite challenging. This case report drives attention towards the uncommon location of PG of lobular capillary haemangioma type occurring on anterior palate. Surgical excision of the lesion was planned because of the discomfort attributed to large size of the lesion and hindrance in mastication. PMID:23417947

Varma, Siddhartha; Gangavati, Rashmi; Sundaresh, K J; Mallikarjuna, Rachappa

2013-01-01

63

Recognizing Uncommon Presentations of Psychogenic (Functional) Movement Disorders  

PubMed Central

Background Psychogenic or functional movement disorders (PMDs) pose a challenge in clinical diagnosis. There are several clues, including sudden onset, incongruous symptoms, distractibility, suggestibility, entrainment of symptoms, and lack of response to otherwise effective pharmacological therapies, that help identify the most common psychogenic movements such as tremor, dystonia, and myoclonus. Methods In this manuscript, we review the frequency, distinct clinical features, functional imaging, and neurophysiological tests that can help in the diagnosis of uncommon presentations of PMDs, such as psychogenic parkinsonism, tics, and chorea; facial, palatal, and ocular movements are also reviewed. In addition, we discuss PMDs at the extremes of age and mass psychogenic illness. Results Psychogenic parkinsonism (PP) is observed in less than 10% of the case series about PMDs, with a female–male ratio of roughly 1:1. Lack of amplitude decrement in repetitive movements and of cogwheel rigidity help to differentiate PP from true parkinsonism. Dopamine transporter imaging with photon emission tomography can also help in the diagnostic process. Psychogenic movements resembling tics are reported in about 5% of PMD patients. Lack of transient suppressibility of abnormal movements helps to differentiate them from organic tics. Psychogenic facial movements can present with hemifacial spasm, blepharospasm, and other movements. Some patients with essential palatal tremor have been shown to be psychogenic. Convergence ocular spasm has demonstrated a high specificity for psychogenic movements. PMDs can also present in the context of mass psychogenic illness or at the extremes of age. Discussion Clinical features and ancillary studies are helpful in the diagnosis of patients with uncommon presentations of psychogenic movement disorders.

Baizabal-Carvallo, José Fidel; Fekete, Robert

2015-01-01

64

Ewing’s Sarcoma: An Uncommon Breast Tumor  

PubMed Central

Ewing’s sarcoma/primitive neuroectodermal tumors (EWS/PNET) are rare malignant and aggressive tumors, usually seen in the trunk and lower limbs of children and young adults. They are uncommon in the breast. We report a case of a 43-year-old woman who developed a painless breast mass. An initial core needle biopsy concluded to a fibrocystic dystrophy contrasting with a rapidly growing mass; thus a large lumpectomy was done. Diagnosis of primary PNET of the breast was established, based on both histopathological examination and immunohistochemical findings. Surgical margins were positive, therefore, left modified radical mastectomy with axillary lymph nodes dissection was performed. The patient was given 6 cycles of adjuvant chemotherapy containing cyclophosphamide, adriamycin and vincristine. Twenty months later, she is in life without recurrence or metastasis. EWS/PNET may impose a diagnostic challenge. Indeed, mammography and ultrasonography features are non specific. The histopathological pattern is variable depending on the degree of neuroectodermal differentiation. Immuno-phenotyping is necessary and genetic study is the only confirmatory tool of diagnosis showing a characteristic cytogenetic anomaly; t (11; 22) translocation. PMID:25332765

Meddeb, Sawsen; Rhim, Mohamed Salah; Kouira, Mouna; Mestiri, Sarra; Bibi, Mohamed; Yacoubi, Mohamed Tahar

2014-01-01

65

Severe and uncommon involuntary movement disorders due to psychotropic drugs.  

PubMed

Disorders of involuntary movement due to psychotropic drugs pose a major problem when treating mentally ill patients. These adverse drug reactions (ADR) frequently undermine the patients' compliance and may have serious consequences as well. The drug safety program in psychiatry AMSP (Arzneimittelsicherheit in der Psychiatrie) surveyed a population of 122,562 patients between 1993 and 2000, and documented 129 especially severe or uncommon involuntary movement disorders (IMD): 9 episodes of severe acute dyskinesia, 32 of severe Parkinsonism, 5 of especially severe akathisia, 16 of 'atypical dyskinesia', 38 of Pisa syndrome, 6 of catatonic neuroleptic syndrome, 15 of neuroleptic malignant syndrome, and 8 of tardive dyskinesia. The epidemiological data for this population were systematically analyzed as regards the patient's history of medication, comedication, and clinical course. In those cases, in which a certain drug was imputed to cause an ADR alone and the causal relationship was rated as definite or probable, typical neuroleptics with mainly antipsychotic effects showed a relatively high incidence of 0.1047 %, those with hypnotic-sedative effect a lower incidence of 0.0198 %, and the atypical neuroleptics an incidence of 0.0567 %. This difference was highly significant in an chi (2)-analysis (chi (2) = 18.81, df = 2, p < 0.0001). Our data provide important information on the frequency, severity, and the consequences of ARD for the patients' compliance, and thus are of clinical interest. PMID:15052515

Stübner, S; Rustenbeck, E; Grohmann, R; Wagner, G; Engel, R; Neundörfer, G; Möller, H-J; Hippius, H; Rüther, E

2004-03-01

66

Epidemiology of Peyronie's disease  

Microsoft Academic Search

Francois Gigot de la Peyronie, surgeon to Louis XV of France, has become synonymous with the rather enigmatic though not uncommon condition of Peyronie's disease (PD), a localized connective tissue disorder of the penile tunica albuginea. The true prevalence of Peyronie's disease is unknown. Therefore, we decided to perform an evaluation of existing epidemiological data. A prevalence rate of 3.2%

F Sommer; U Schwarzer; G Wassmer; W Bloch; M Braun; T Klotz; U Engelmann

2002-01-01

67

[Fabry disease].  

PubMed

Fabry disease is an uncommon, X-linked lysosomal storage disorder, caused by partial or complete deficiency of the enzyme a-galactosidase A. The defect leads to accumulation of uncleaved globotriaosylceramide on the vascular endothelium and visceral tissues, being the skin, heart, kidneys and central nervous system the most affected organs. We performed review of the literature related to the disease and emphasized that early recognition of angiokeratomas and hypohidrosis are key diagnostic signs of this serious disease. We also addressed the need of multidisciplinary assessment of these patients. PMID:19851669

Boggio, Paula; Luna, Paula Carolina; Abad, María Eugenia; Larralde, Margarita

2009-01-01

68

Orchestration of an uncommon maturation cascade of the house dust mite protease allergen quartet.  

PubMed

In more than 20% of the world population, sensitization to house dust mite allergens triggers typical allergic diseases such as allergic rhinitis and asthma. Amongst the 23 mite allergen groups hitherto identified, group 1 is cysteine proteases belonging to the papain-like family whereas groups 3, 6, and 9 are serine proteases displaying trypsin, chymotrypsin, and collagenolytic activities, respectively. While these proteases are more likely to be involved in the mite digestive system, they also play critical roles in the initiation and in the chronicity of the allergic response notably through the activation of innate immune pathways. All these allergenic proteases are expressed in mite as inactive precursor form. Until recently, the exact mechanisms of their maturation into active proteases remained to be fully elucidated. Recent breakthroughs in the understanding of the activation mechanisms of mite allergenic protease precursors have highlighted an uncommon and unique maturation pathway orchestrated by group 1 proteases that tightly regulates the proteolytic activities of groups 1, 3, 6, and 9 through complex intra- or inter-molecular mechanisms. This review presents and discusses the currently available knowledge of the activation mechanisms of group 1, 3, 6, and 9 allergens of Dermatophagoides pteronyssinus laying special emphasis on their localization, regulation, and interconnection. PMID:24744761

Dumez, Marie-Eve; Herman, Julie; Campizi, Vincenzo; Galleni, Moreno; Jacquet, Alain; Chevigné, Andy

2014-01-01

69

Orchestration of an Uncommon Maturation Cascade of the House Dust Mite Protease Allergen Quartet  

PubMed Central

In more than 20% of the world population, sensitization to house dust mite allergens triggers typical allergic diseases such as allergic rhinitis and asthma. Amongst the 23 mite allergen groups hitherto identified, group 1 is cysteine proteases belonging to the papain-like family whereas groups 3, 6, and 9 are serine proteases displaying trypsin, chymotrypsin, and collagenolytic activities, respectively. While these proteases are more likely to be involved in the mite digestive system, they also play critical roles in the initiation and in the chronicity of the allergic response notably through the activation of innate immune pathways. All these allergenic proteases are expressed in mite as inactive precursor form. Until recently, the exact mechanisms of their maturation into active proteases remained to be fully elucidated. Recent breakthroughs in the understanding of the activation mechanisms of mite allergenic protease precursors have highlighted an uncommon and unique maturation pathway orchestrated by group 1 proteases that tightly regulates the proteolytic activities of groups 1, 3, 6, and 9 through complex intra- or inter-molecular mechanisms. This review presents and discusses the currently available knowledge of the activation mechanisms of group 1, 3, 6, and 9 allergens of Dermatophagoides pteronyssinus laying special emphasis on their localization, regulation, and interconnection. PMID:24744761

Dumez, Marie-Eve; Herman, Julie; Campizi, Vincenzo; Galleni, Moreno; Jacquet, Alain; Chevigné, Andy

2014-01-01

70

Uncommon case of brain metastasis in a patient with a history of heavy smoking.  

PubMed

Primary sarcomas of the aorta are extremely uncommon. Depending on histomorphology and immunohistochemical pattern, intimal sarcomas can show angiosarcomatous differentiation. Here, we describe the case of a 60-year-old woman with a primary intimal sarcoma of the aortic arch and signs of cerebral metastatic disease as the initial manifestation. After the patient experienced the onset of severe headaches, ataxia, and left-sided weakness, magnetic resonance imaging showed several brain lesions. Histologic assessment of a brain biopsy specimen revealed a malignant tumour composed of large pleomorphic cells that were positive for pancytokeratin and CD10. Radiation to the brain did not significantly improve the patient's symptoms, and cranial computed tomography (ct) imaging revealed several metastases, indicating lack of response. Because of the patient's smoking history, the presence of central nervous system and skeletal metastases on combined positron-emission tomography and ct imaging, and the focal pan-cytokeratin positivity of the tumour, carcinoma of the lung was favoured as the primary tumour. Despite chemotherapy with cisplatin and etoposide, the patient's neurologic symptoms and general condition deteriorated rapidly, and she died within a few days. At autopsy, an undifferentiated intimal sarcoma of the aortic arch was diagnosed. The primary tumour in the aorta consisted of large pleomorphic cells. Immunohistochemical analysis of the aortic tumour and brain metastases demonstrated diffuse positivity for vimentin and p53 and focal S-100 staining. In summary, we report a challenging case of advanced intimal sarcoma of the aortic arch with brain and bone metastases at initial presentation. Our report demonstrates the difficulties in diagnosing and treating this disease, and the need for multicentre studies to accrue more patients for investigations of optimal therapy. PMID:25302044

Scharl, M; Bode, B; Rushing, E; Knuth, A; Rordorf, T

2014-10-01

71

Contribution of endosonography in an uncommon case of pancreatic cysts  

PubMed Central

Here we present the case of a 35-year-old female patient with long standing dyspepsia and imaging studies showing the presence of multiple cysts in the head and tail of the pancreas. The patient underwent endosonography that confirmed the presence of multiple simple cysts throughout the entirety of the pancreas without dilation of the pancreatic duct. The majority of the cysts were less than one centimeter in size, and the largest cyst showed a honeycomb appearance. Cytology of aspirates from the two largest cysts was compatible with benign pancreatic cysts. Endosonography also revealed cysts within the left kidney and spleen. Genetic testing confirmed Von Hippel-Lindau disease. We highlight this case because it is unusual for Von Hippel-Lindau disease, a rare clinical entity, to present solely with cysts in the absence of more common manifestations, such as hemangioblastomas in the central nervous system and malignancy. PMID:24147197

Sousa, Ana Lúcia; Sousa, Diamantino; Figueiredo, Pedro; Marques, Pedro Pinto; Guerreiro, Horácio

2013-01-01

72

Gene Duplication Identified in an Uncommon Form of Bone Cancer  

Cancer.gov

Scientists have discovered that a familial form of a rare bone cancer called chordoma is explained not by typical types of changes or mutations in the sequence of DNA in a gene, but rather by the presence of a second copy of an entire gene. Inherited large structural changes, known as copy number variations (CNVs), have been implicated in some hereditary diseases but have seldom been reported as the underlying basis for a familial cancer.

73

Death in pediatric Cushing syndrome is uncommon but still occurs.  

PubMed

Cushing syndrome (CS) in children is rare. Delayed diagnosis and treatment of CS may be associated with increased morbidity and, unfortunately, mortality. We performed a retrospective review of all patients with CS under the age of 18 years referred to the National Institutes of Health (NIH) from 1998 to 2013 in order to describe deceased patients among cases of pediatric CS referred to the National Institutes of Health (NIH). The deaths of four children (three females and one male), aged 7.5-15.5 years (mean age 11.2 years) with length of disease 2-4 years, were recorded among 160 (2.5 %) children seen at or referred to the NIH over the last 15 years. All died at different institutions, prior to coming to the NIH (two) or after leaving NIH (two). Presenting symptoms included increasing weight and decreasing height gain, facial plethora, dorsocervical fat pad (webbed neck), striae, headache, vision disturbances, and depression and other mood or behavior changes; there were no differences between how these patients presented and the others in our cohort. The causes of CS in the deceased patients were also not different, in fact, they spanned the entire spectrum of CS: pituitary disease (one), ectopic corticotropin production (one), and primary adrenal hyperplasia (one). In one patient, the cause of CS could not be verified. Three died of sepsis and one due to residual disease and complications of the primary tumor. Conclusions: Despite the advances in early diagnosis and treatment of pediatric CS, a 2.5 % mortality rate was identified in a large cohort of patients with this condition referred to an experienced, tertiary care referral center (although these deaths occurred elsewhere). Pediatricians need to recognize the possibility of death, primarily due to sepsis, in a patient with pediatric CS and treat accordingly. PMID:25241829

Gkourogianni, Alexandra; Lodish, Maya B; Zilbermint, Mihail; Lyssikatos, Charalampos; Belyavskaya, Elena; Keil, Margaret F; Stratakis, Constantine A

2014-09-23

74

Chronic Cough and Eosinophilic Esophagitis: An Uncommon Association  

PubMed Central

An increasing number of children, usually with gastrointestinal symptoms, is diagnosed with eosinophilic esophagitis (EE), and a particular subset of these patients complains of airway manifestations. We present the case of a 2-year-old child with chronic dry cough in whom EE was found after a first diagnosis of gastroesophageal reflux disease (GERD) due to pathological 24-hour esophageal pH monitoring. Traditional allergologic tests were negative, while patch tests were diagnostic for cow's milk allergy. We discuss the intriguing relationship between GERD and EE and the use of patch test for the allergologic screening of patients. PMID:21960955

Orizio, Paolo; Cinquini, Massimo; Minetti, Stefano; Alberti, Daniele; Paolo, Camilla Di; Villanacci, Vincenzo; Torri, Fabio; Crispino, Paola; Facchetti, Susanna; Rizzini, Fabio Lodi; Bassotti, Gabrio; Tosoni, Cinzia

2011-01-01

75

American tegumentary leishmaniasis: an uncommon clinical and histopathological presentation.  

PubMed

We report a case of an unusual presentation of American Tegumentary Leishmaniasis involving a male patient with a solitary lesion on the ear lobe, persisting with minimal increase for at least six months without ulceration or related symptoms. The histological sections showed epithelial atrophy and a large number of structures consistent with Leishmania sp. amastigotes within macrophages. Treatment commenced with meglumine antimoniate resulting in regression of the condition. This report is of importance given the unusual clinical manifestation and histopathological findings in this case and the fact that there was low correlation with the extended duration of the disease. PMID:23739691

Adriano, Adrilena Lopes; Leal, Paula Azevedo Borges; Breckenfeld, Marcelle Parente; Costa, Igor dos Santos; Almeida, Clarisse; Sousa, Antônio Renę Diógenes de

2013-01-01

76

Sciatic nerve tumor and tumor-like lesions - uncommon pathologies.  

PubMed

Sciatic nerve mass-like enlargement caused by peripheral nerve sheath tumors or neurocutaneous syndromes such as neurofibromatosis or schwannomatosis has been widely reported. Other causes of enlargement, such as from perineuroma, fibromatosis, neurolymphoma, amyloidosis, endometriosis, intraneural ganglion cyst, Charcot-Marie-Tooth disease, and chronic inflammatory demyelinating polyneuropathy are relatively rare. High-resolution magnetic resonance imaging (MRI) is an excellent non-invasive tool for the evaluation of such lesions. In this article, the authors discuss normal anatomy of the sciatic nerve and MRI findings of the above-mentioned lesions. PMID:22410805

Wadhwa, Vibhor; Thakkar, Rashmi S; Maragakis, Nicholas; Höke, Ahmet; Sumner, Charlotte J; Lloyd, Thomas E; Carrino, John A; Belzberg, Allan J; Chhabra, Avneesh

2012-07-01

77

Lupus Flare: An Uncommon Presentation of Disseminated Gonorrhea  

PubMed Central

Gonorrhea is one of the most common sexually transmitted diseases in the US with 700,000 annual cases. Although most cases of gonorrhea are localized, approximately 0.5–3% become disseminated. Here we discuss a rare case of a patient with systemic lupus erythematosus (SLE) who developed septic shock from disseminated gonorrhea infection (DGI). Our patient is a 24-year-old woman with SLE, mixed connective tissue disease with cutaneous vasculitis, and lupus nephritis who presented with several weeks of malaise and generalized body aches associated with a diffuse rash along her fingers, palms, and trunk. Infectious workup was unrevealing with the exception of a positive gonorrhea test obtained from a cervical swab. Given her symptoms of tenosynovitis, the appearance of her skin lesions, and her positive gonorrhea test, she was diagnosed with septic shock secondary to DGI. With antibiotic treatment, the patient reported a dramatic improvement of the pain in her swollen joints and her rash receded. Patients diagnosed with SLE carry an increased risk of gonorrhea regardless of whether or not they are being treated for their SLE. Although it is well-documented that SLE is associated with severe DGI, few describe it resulting in overt septic shock. PMID:25024709

Kim, Joyce

2014-01-01

78

Uncommon and rarely reported adverse events of endoscopic retrograde cholangiopancreatography.  

PubMed

Endoscopic retrograde cholangiopancreatography (ERCP) has become a primary tool for the treatment of biliary and pancreatic ductal diseases. It is essential for the endoscopist carrying out the ERCP to have a thorough understanding of the potential adverse events. Typically, endoscopists are well familiar with common adverse events such as post-ERCP pancreatitis, cholangitis, post-sphincterotomy bleeding, post-sphincterotomy perforation, and sedation-related cardiopulmonary compromises. However, there are other less common adverse events that arecritical to promptly recognize in order to provide appropriate therapy and prevent disastrous outcomes. This review focuses on the presentation and management of the less common and rare adverse events of an ERCP from the perspective of the practicing endoscopist. PMID:24118211

Chavalitdhamrong, Disaya; Donepudi, Suman; Pu, Liping; Draganov, Peter V

2014-01-01

79

Renal abscesses in childhood: report of two uncommon cases.  

PubMed

Renal abscesses are rare conditions in children, but they must be remembered in differential diagnosis of fever and abdominal pain. The authors report two paediatric cases with unusual presentation. Case 1: a 15-year-old girl was admitted following a period of fever, vomiting and left hypochondrium pain which became more localised to the left lower ribs. Blood tests suggested bacterial infection, but urinalysis and culture were negative. Renal CT scan presented features of bilateral pyelonephritis and left renal abscesses, while ultrasound remained normal until the ninth day of disease. Case 2: a 2-year-old girl, with diagnosis of ?-thalassemia minor, had intermittent diffuse abdominal pain with 2 weeks of evolution. Renal ultrasonography and CT scan showed a heterogeneous mass compatible with Willms tumour. Intraoperative diagnosis was compatible with renal abscess with isolation of Proteus mirabilis in the fluid. Both responded well to long-term antibiotics and to surgical drainage (in the second case). PMID:24671317

Cancelinha, Candida; Santos, Lea; Ferreira, Carmen; Gomes, Clara

2014-01-01

80

Congenital Lobar Emphysema and Intercostal Drainage Tube Insertion: The Common Fate of an Uncommon Disease  

PubMed Central

Congenital Lobar Emphysema (CLE) is one of the rare cystic malformations of the lung. This malformation is generally confused with pneumothorax of the lung and most often, the placement of an intercostal drainage tube is entertained in an emergency situation, but the non- improvement of the distress and non-expansion of the lung are eye openers for a treating paediatrician. We are describing a case of a 26 days old baby who presented with respiratory distress in the Emergency Department. On the basis of the chest X-ray (CXR), we suspected it to be a case of pneumothorax and an intercostal drainage tube was inserted. But as he did not improve, CT of the chest was done and the baby was diagnosed to have congenital lobar emphysema. In spite of the advanced diagnostic techniques, the diagnosis of CLE may present a diagnostic challenge and a high index of suspicion is needed if the diagnosis has to be made promptly. PMID:23285462

Kumar, Saurabh; Debata, P.K.; Gupta, R.

2012-01-01

81

Torsion of the Retroperitoneal Kidney: Uncommon or Underreported?  

PubMed Central

Vascular torsion in a renal allograft after placement in the retroperitoneum is rare and has only been reported twice in the literature. It is an extrinsically mediated process that occurs at the vascular pedicle resulting in graft compromise and potential loss. Rapid diagnosis and immediate surgical intervention may salvage allograft function. Herein, we present a unique case of a 42-year-old male that developed renal allograft torsion following a second kidney transplant placed in the retroperitoneum. Immediate detorsion did not resolve allograft dysfunction, and a biopsy revealed acute cellular mediated rejection. After antithymocyte globulin treatment, allograft function was salvaged. A review of the current literature shows that the incidence, morbidity, and long term allograft function of intraperitoneal and extraperitoneal torsion are different. As such, torsion of the retroperitoneal kidney demonstrates encouraging allograft salvage rates. Only the third case reported to date, this serves as a contribution to the growing body of literature in retroperitoneal renal torsion and reviews the risks, medication considerations, diagnostic tests, and treatment modalities in a unique disease process. PMID:24551473

Sosin, Michael; Lumeh, Wuya; Cooper, Matthew

2014-01-01

82

Dysphonia, an uncommon symptom of systemic neurotoxic envenomation by Vipera aspis bite. Report of two cases.  

PubMed

Two cases of Vipera aspis bites with severe envenomation in which, among other neurotoxic signs, dysphonia was observed, with alteration of the pitch of the voice are described. This uncommon symptom has never been reported in envenomation by European adders. It is pointed out that bites of European vipers should never be underestimated as severe envenomation may develop. PMID:9655630

Beer, E; Putorti, F

1998-05-01

83

Uncommon acute neurologic presentation of canine distemper in 4 adult dogs.  

PubMed

Four uncommon cases of canine distemper (CD) were diagnosed in vaccinated adult dogs. All dogs had acute onset of neurologic signs, including seizures, abnormal mentation, ataxia, and proprioceptive deficits. Polymerase chain reaction for CD virus was positive on cerebrospinal fluid in 2 cases. Due to rapid deterioration the dogs were euthanized and CD was confirmed by postmortem examination. PMID:24688139

Galán, Alba; Gamito, Araceli; Carletti, Beatrice E; Guisado, Alicia; de las Mulas, Juana Martín; Pérez, José; Martín, Eva M

2014-04-01

84

Uncommon acute neurologic presentation of canine distemper in 4 adult dogs  

PubMed Central

Four uncommon cases of canine distemper (CD) were diagnosed in vaccinated adult dogs. All dogs had acute onset of neurologic signs, including seizures, abnormal mentation, ataxia, and proprioceptive deficits. Polymerase chain reaction for CD virus was positive on cerebrospinal fluid in 2 cases. Due to rapid deterioration the dogs were euthanized and CD was confirmed by postmortem examination. PMID:24688139

Galán, Alba; Gamito, Araceli; Carletti, Beatrice E.; Guisado, Alicia; de las Mulas, Juana Martín; Pérez, José; Martín, Eva M.

2014-01-01

85

Autism is uncommon in 22q: the how and why of wrong diagnoses  

E-print Network

Autism is uncommon in 22q: the how and why of wrong diagnoses Kathleen Angkustsiri, Khyati Disclosures · Co-investigator on clinical trials in autism and fragile X syndrome ­Novartis ­Roche ­Seaside Pharmaceuticals ­Forest Laboratories #12;22q and Autism Spectrum Disorders · Autism Spectrum Disorders

Nguyen, Danh

86

Uncommon breeding birds in North Dakota: Population estimates and frequencies of occurrence  

USGS Publications Warehouse

Breeding bird populations were surveyed on 128 randomly selected quarter-sections throughout North Dakota in 1967, 1992, and 1993. Population estimates and frequencies of occurrence are reported for 92 uncommon breeding bird species with statewide frequencies of less than 10%.

Igl, L.D.; Johnson, D.H.; Kantrud, H.A.

1999-01-01

87

An uncommon location of Meckel's diverticulum or small intestine duplication? Case report and literature review.  

PubMed

Meckel's diverticulum is the most common congenital abnormality of alimentary tract. The antimesenteric location is one of the cardinal attribiutes of this pathology. We report case which tries to verify this dogma. The literature regarding uncommon location of Meckel's diverticulum was also reviewed. PMID:22166720

Walczak, Dominik A; Fa?ek, Wojciech; Zakrzewski, Jacek

2011-08-01

88

Spontaneous Tooth Exfoliation after Trigeminal Herpes Zoster: A Case Series of an Uncommon Complication  

PubMed Central

The most significant and debilitating complication of herpes zoster (HZ) is herpetic neuralgia that accompanies and may persist in 10-15% of all zoster patients, particularly those over 60 years of age. The described 3 cases had an uncommon complication of spontaneous tooth exfoliation after trigeminal HZ that rarely finds mention in dermatology literature. PMID:23723511

Mahajan, Vikram K; Ranjan, Nitin; Sharma, Sangeet; Sharma, Nand Lal

2013-01-01

89

Mesenteric Inflammatory Venoocclusive Disease in a Patient with Sjögren's Syndrome  

PubMed Central

Mesenteric inflammatory venoocclusive disease is an uncommon cause of intestinal ischemia. Certain diseases, such as hypercoagulation disorders, autoimmune diseases, or drugs have been associated with the pathogenesis of mesenteric inflammatory venoocclusive disease. Here, we report a patient with Sjögren's syndrome who underwent surgery for suspected acute appendicitis with a subsequent pathological diagnosis of mesenteric inflammatory venoocclusive disease. PMID:25477970

Rios-Fernández, Raquel; Callejas-Rubio, José-Luis; Caba-Molina, Mercedes; Ríos-Peregrina, Rosa; Ortego-Centeno, Norberto

2014-01-01

90

A Rare Case of Unrecognized and Uncommon Bladder Perforation after Transobturator Tape Procedure  

PubMed Central

The transobturator tape (TOT) procedure has become practically widespread worldwide. Complications seem to be rare, but recognizing them intraoperatively is the most significant step because some of the complications which may appear in postoperative period can be challenging for both physicians and patients. The purpose of this case, with this patient who was operated on with open surgery, is to evaluate this rarely seen unrecognized and uncommon bladder perforation after TOT procedure and thus make some contribution to the literature. Here, we present a case report about the treatment of a 48-year-old woman patient with unrecognized and uncommon bladder perforation after TOT procedure, 5 months postoperatively. Cystoscopic evaluation is not recommended routinely, but it must be performed if the patient is complicated enough to create doubt and also the surgeon's skill and ability are not sufficient enough to operate decently.

K?l?nç, Ercüment; Akpak, Ya?am Kemal

2015-01-01

91

Distribution Patterns of Ohio Stoneflies, with an Emphasis on Rare and Uncommon Species  

PubMed Central

Presently, 102 stonefly species (Plecoptera) have been reported from Ohio. All 9 Nearctic families are represented. Over 90% of the fauna exhibit a combination of broad Nearctic-widespread, eastern Nearctic-widespread, Appalachian, and eastern Nearctic-unglaciated distributions. In contrast, only 2 species display a central Nearctic-Prairie distribution. Seven species of Perlidae are likely no longer present (Acroneuria evoluta Klapálek, A. perplexa Frison, Attaneuria ruralis (Hagen), and Neoperla mainensis Banks) or have experienced marked range reductions (Acroneuria abnormis (Newman), A. frisoni Stark and Brown, and A. filicis Frison). Another nearly 31% of the fauna (32 species) are rare, uncommon, or have highly-limited distributions within the state. Twelve of these species have Appalachian distributions, and an additional 8 have eastern Nearctic-unglaciated distributions. The distributional status for each of the 32 rare/uncommon species is discussed. PMID:24219390

Grubbs, Scott A.; Pessimo, Massimo; DeWalt, R. Edward

2013-01-01

92

Bilateral claw hand: an uncommon presentation of regional Guillain-Barré syndrome.  

PubMed

We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day of hospitalization the patient presented symmetrical distal leg weakness and was treated with intravenous immunoglobulin. PMID:23927940

Tsivgoulis, Georgios; Tsakaldimi, Soultana; Vadikolias, Konstantinos; Mantatzis, Michalis; Katsanos, Aristeidis H; Heliopoulos, Ioannis; Piperidou, Charitomeni

2013-11-15

93

Uncommon causes of anterior urethral diverticula in children: Two cases and review of literature  

PubMed Central

Anterior urethral diverticula are rare in children. Anterior urethral valves and associated diverticulum is the commonly discussed pathological entity in children. There is a lack of awareness among clinicians regarding less common presentations of anterior urethral diverticula in children; which can have a diverse involvement of the urinary tract. This report describes two uncommon presentations of anterior urethral diverticula in children, their diagnoses and management. A systematic differential diagnosis and review of anterior urethral diverticula in children is also presented. PMID:24669129

Smith, Grahame H. H.; Deshpande, Aniruddh V.; Tang, Robert W. K.

2014-01-01

94

Common and uncommon adult unilateral renal masses other than renal cell carcinoma  

PubMed Central

Abstract Many different masses can involve the kidney other than the commonly encountered renal cell carcinoma (RCC). The purpose of this article is to review the characteristic clinical and imaging findings of common and uncommon masses that predominantly present unilaterally in the adult patient, other than RCC. Awareness of such lesions and knowing the clinical scenario is important for appropriate diagnosis and management, especially in a multidisciplinary care setting. PMID:22752221

Le, Ott; Roy, Anjali; Silverman, Paul M.; Kundra, Vikas

2012-01-01

95

Uncommon Alu-mediated NF1 microdeletion with a breakpoint inside the NF1 gene.  

PubMed

Neurofibromatosis type 1 (NF1) microdeletion syndrome is caused by haploinsufficiency of the NF1 gene and of gene(s) located in adjacent flanking regions. Most of the NF1 deletions originate by nonallelic homologous recombination between repeated sequences (REP-P and -M) mapped to 17q11.2, while a few uncommon deletions show unusual breakpoints. We characterized an uncommon 1.5-Mb deletion of an NF1 patient displaying a mild phenotype. We applied high-resolution FISH analysis allowing us to obtain the sequence of the first junction fragment of an uncommon deletion showing the telomeric breakpoint inside the IVS23a of the NF1 gene. Sequence analysis of the centromeric and telomeric boundaries revealed that the breakpoints were present in the AluJb and AluSx regions, respectively, showing 85% homology. The centromeric breakpoint is localized inside a chi-like element; a few copies of this sequence are also located very close to both breakpoints. The in silico analysis of the breakpoint intervals, aimed at identifying consensus sequences of several motifs usually involved in deletions and translocations, suggests that Alu sequences, probably associated with the chi-like element, might be the only recombinogenic motif directly mediating this large deletion. PMID:15676286

Gervasini, Cristina; Venturin, Marco; Orzan, Francesca; Friso, Alessandra; Clementi, Maurizio; Tenconi, Romano; Larizza, Lidia; Riva, Paola

2005-02-01

96

Characterization of uncommon portosystemic collateral circulations in patients with hepatic cirrhosis.  

PubMed

The purpose of the present study was to characterize uncommon portosystemic collateral circulation in hepatic cirrhosis. Portosystemic uncommon collateral circulation (UCC) was detected, characterized and evaluated by a combination of spiral computed tomography angiography, three-dimensional imaging angiography and electronic gastroscopy in patients diagnosed with hepatic cirrhosis. In total, 118 cases with UCC were detected from a pool of 700 hepatic cirrhosis patients with portal hypertension. The incidence was 16.86% and included cases with splenic-renal, gastro-renal, paravertebral, retroperitoneal, gastric-splenic and cardio-phrenic angle vein shunts. The occurrence rate of UCC formation increased with the Child-Pugh grade. Compared with common collateral circulations, the incidence of severe esophageal or gastric fundus varicose veins, severe portal hypertensive gastropathy and the incidence of a large quantity of ascites was much lower in the patients with UCC (P<0.01), whereas the incidence of hepatic encephalopathy and chronic elevated blood ammonia levels was significantly higher (P<0.01). The incidence of uncommon portosystemic collateral circulation is extremely common in patients with liver cirrhosis and is associated with the Child-Pugh grades of hepatic function. UCC can aid in the relief of the complications derived from portal hypertension, but it may increase the incidence of hepatic encephalopathy and chronic elevated blood ammonia levels. PMID:25435990

Wu, Qin; Shen, Lijun; Chu, Jindong; Ma, Xuemei; Jin, Bo; Meng, Fanping; Chen, Jinpin; Wang, Yanling; Wu, Libing; Han, Jun; Zhang, Wenhui; Ma, Wei; Wang, Huaming; Li, Hanwei

2015-01-01

97

Management of multiple valve disease.  

PubMed

Multivalvular heart disease is not an uncommon situation, but the paucity of data for each specific situation does not allow the proposal of a standardised, evidence-based management strategy. This paper aims at reviewing the available evidence on the management of multivalvular disease, taking into account the interactions between different valve lesions, the diagnostic pitfalls and the strategies that should be considered in the presence of multiple valvular disease. PMID:21156677

Unger, Philippe; Rosenhek, Raphael; Dedobbeleer, Chantal; Berrebi, Alain; Lancellotti, Patrizio

2011-02-01

98

Intestinal pseudoobstruction in Kawasaki disease.  

PubMed

Intestinal pseudoobstruction is an uncommon but important manifestation of Kawasaki disease. Its occurrence at the onset or during the course of the disease may confuse the clinical picture and cause delay in diagnosis and treatment. This delay may be responsible for the high rate of coronary artery abnormalities that have been reported in patients with this complication. We suggest that Kawasaki disease be considered in the differential diagnosis of any child presenting with intestinal pseudoobstruction and fever without definable cause. PMID:15121996

Akikusa, Jonathan D; Laxer, Ronald M; Friedman, Jeremy N

2004-05-01

99

AN EXCITING OPPORTUNITY TO EXPERIENCE UNCOMMON LEARNING The Department of Cell Biology and Neuroscience at Montana State University offers an  

E-print Network

AN EXCITING OPPORTUNITY TO EXPERIENCE UNCOMMON LEARNING The Department of Cell Biology. Students graduating in Cell Biology and Neuroscience are well prepared for a successful career in research requirements. For additional information, contact: Department of Cell Biology & Neuroscience Montana State

Maxwell, Bruce D.

100

Rare Association: Chagas' Disease and Hypertrophic Cardiomyopathy.  

PubMed

A woman (49 years) with Chagas' disease showed: ECG, right bundle-branch block and left anterior-superior fascicular block; V1 has unusual R > R', and elevated ST segment from V2 to V6 . Additional imaging revealed concomitant HCM and Chagas, which is uncommon. Overlapping of ECG findings can be explained by this rare association of diseases. PMID:25367861

Pastore, Carlos Alberto; Samesima, Nelson; Filho, Horácio Gomes Pereira; Varoni, Leonardo Paschoal Camacho; Rochitte, Carlos Eduardo; Vieira, Marcelo Luiz; de Ávila, Luiz Francisco Rodrigues; Melo, Rodrigo de Jesus Louzeiro; Pereira, Alexandre da Costa; Daheer, Julia; Carlo, Carlos Henrique Del

2014-11-01

101

Relationship Between Uncommon Computed Tomography Findings and Clinical Aspects in Patients With Acute Pyelonephritis  

PubMed Central

Purpose Computed tomography (CT) has become popular in the diagnosis of acute pyelonephritis (APN) and its related complications in adults. The aim of this study was to investigate the relationship between uncommon CT findings and clinical and laboratory data in patients with APN. Materials and Methods From July 2009 to July 2012, CT findings and clinical data were collected from 125 female patients with APN. The six uncommon CT findings (excluding a wedge-shaped area of hypoperfusion in the renal parenchyma) studied were perirenal fat infiltration, ureteral wall edema, renal abscess formation, pelvic ascites, periportal edema, and renal scarring. The clinical parameters analyzed were the age and body mass index of the patients as well as the degree and duration of fever. Laboratory parameters related to inflammation and infection included white blood cell count, C-reactive protein (CRP) level, erythrocyte sedimentation rate, pyuria, and bacteriuria. Results The most common CT finding was perirenal fat infiltration (69 cases, 55%). A longer duration of fever, higher CRP level, and grade of pyuria were related with perirenal fat infiltration (p=0.010, p=0.003, and p=0.049, respectively). The CRP level was significantly higher in patients with renal abscess and ureteral wall edema (p=0.005 and p=0.015, respectively). Conclusions The uncommon CT findings that were related to aggravated clinical and laboratory parameters of APN patients were perirenal fat infiltration, ureteral wall edema, and renal abscess formation. The inflammatory reaction and tissue destruction may be more aggressive in patients with these CT findings. PMID:25045448

Kim, Jang Sik; Lee, Sangwook; Lee, Kwang Woo; Kim, Young Ho; Kim, Min Eui

2014-01-01

102

Superior mesenteric artery syndrome: an uncommon cause of abdominal pain mimicking gastric outlet obstruction.  

PubMed

Upper gastrointestinal symptoms like vomiting, abdominal pain, abdominal distention may be caused by many conditions like complicated peptic/duodenal ulcer, gastritis or hiatal hernia. However, these symptoms are uncommonly produced by superior mesenteric artery (SMA) syndrome. SMA syndrome is triggered when there is narrowing of the mesenteric angle and shortening of the aortomesenteric distance. We report a case of 42-year-old female who presented with features of gastric outlet obstruction which on further investigation was revealed as SMA syndrome. The aetiology, presentation, diagnosis and management of this unusual condition are discussed. PMID:24349841

Shah, D; Naware, S; Thind, S; Kuber, R

2013-11-01

103

Adder bite: an uncommon cause of compartment syndrome in northern hemisphere  

PubMed Central

Snakebite envenomation is an uncommon condition in the northern hemisphere, but requires high vigilance with regard to both the systemic effects of the venom and the locoregional impact on the soft tissues. Bites from the adder, Vipera Berus, may have serious clinical consequences due to systemic effects. A case of a 44-year-old man is reported. The patient was bitten in the right hand. He developed fasciotomy-requiring compartment syndrome of the upper limb. Recognition of this most seldom complication of an adder bite is vital to save the limb. We recommend that the classical signs and symptoms of compartment syndrome serve as indication for surgical decompression. PMID:20854675

2010-01-01

104

Adder bite: an uncommon cause of compartment syndrome in northern hemisphere.  

PubMed

Snakebite envenomation is an uncommon condition in the northern hemisphere, but requires high vigilance with regard to both the systemic effects of the venom and the locoregional impact on the soft tissues. Bites from the adder, Vipera Berus, may have serious clinical consequences due to systemic effects. A case of a 44-year-old man is reported. The patient was bitten in the right hand. He developed fasciotomy-requiring compartment syndrome of the upper limb. Recognition of this most seldom complication of an adder bite is vital to save the limb. We recommend that the classical signs and symptoms of compartment syndrome serve as indication for surgical decompression. PMID:20854675

Evers, Lars H; Bartscher, Tanja; Lange, Thomas; Mailänder, Peter

2010-01-01

105

An uncommon treatment of totally extruded and lost talus: a case report  

PubMed Central

Introduction Total extrusion and loss of the talus is a rare injury with a wide choice of appropriate treatment, but rarely resulting in a fully functional recovery. We report on an uncommon case, both for the severity of the injury and for the uncommon treatment due to the patient’s rejection of secondary surgery. Case presentation We treated a 16-year-old Caucasian man with the most extreme variant of a totally extruded and lost talus, accompanied with complex injury of the soft tissues of the ankle and foot. The treatment included urgent microvascular foot reimplantation, microvascular muscle free flap transfer, and temporary fixation. This kind of injury should typically be treated by tibiocalcaneal arthrodesis. However, this was not performed, as after the successful early stages of the treatment he strongly objected to another surgery due to his fully functional status and the successful therapeutic results of our early treatment. Conclusions The injury described in this case study would ordinarily be treated by amputation, but due to the well-executed treatment in the early stages after the injury, the outcome was satisfying. Surprisingly and against our expectations, the late results of the treatment were successful even without arthrodesis. He is now 37 years old and has a functional foot 21 years after the injury. PMID:25266945

2014-01-01

106

Journal of Wildlife Diseases, 40(1), 2004, pp. 103109 Wildlife Disease Association 2004  

E-print Network

103 Journal of Wildlife Diseases, 40(1), 2004, pp. 103­109 Wildlife Disease Association 2004: Hantavirus, Heteromyidae, Muridae, Panama, seroprevalence, Sigmodon- tinae. Hantavirus pulmonary syndrome but uncommon viral pneumo- nitis with a mortality rate of about 38%. Hantavirus pulmonary syndrome occurs

107

An uncommon cause of acutely altered mental status in a renal transplant recipient  

PubMed Central

Introduction Neurological complications are quite frequent in patients after solid organ transplantation presenting with focal or generalized neurologic symptoms as well as altered mental status. Posterior reversible encephalopathy syndrome is a rare cliniconeuroradiological entity characterized by headache, altered mental status, cortical blindness, seizures, and other focal neurological signs and a diagnostic magnetic resonance imaging. Case report We present a case of a 57-year-old woman with one episode of seizures and sudden onset of altered mental status (time and person perception) accompanied with headache at the thirtieth postoperative day after renal transplantation. Conclusion Posterior reversible encephalopathy syndrome, although an uncommon post-renal transplantation complication, should be considered in these patients, as several factors surrounding the setting of transplantation have been implicated in its development. Thus, physicians should be aware of this condition in order to establish the diagnosis and offer appropriate treatment. PMID:24079344

Vernadakis, Spiridon; Lionaki, Sofia; Daikos, Georgios; Zavos, Georgios

2014-01-01

108

Apomictic parthenogenesis in a parasitoid wasp Meteorus pulchricornis, uncommon in the haplodiploid order Hymenoptera.  

PubMed

Although apomixis is the most common form of parthenogenesis in diplodiploid arthropods, it is uncommon in the haplodiploid insect order Hymenoptera. We found a new type of spontaneous apomixis in the Hymenoptera, completely lacking meiosis and the expulsion of polar bodies in egg maturation division, on the thelytokous strain of a parasitoid wasp Meteorus pulchricornis (Wesmael) (Braconidae, Euphorinae) on pest lepidopteran larvae Spodoptera litura (Fabricius) (Noctuidae). The absence of the meiotic process was consistent with a non-segregation pattern in the offspring of heterozygous females, and no positive evidence was obtained for the induction of thelytoky by any bacterial symbionts. We discuss the conditions that enable the occurrence of such rare cases of apomictic thelytoky in the Hymenoptera, suggesting the significance of fixed heterosis caused by hybridization or polyploidization, symbiosis with bacterial agents, and occasional sex. Our finding will encourage further genetic studies on parasitoid wasps to use asexual lines more wisely for biological control. PMID:24521569

Tsutsui, Y; Maeto, K; Hamaguchi, K; Isaki, Y; Takami, Y; Naito, T; Miura, K

2014-06-01

109

Laparoscopic management of an uncommon cause for right lower quadrant pain: A case report  

PubMed Central

Introduction Primary segmental infarction of the greater omentum is an infrequent cause for right lower quadrant pain. The exact aetiology is unknown and the right side is more commonly involved. It usually presents like acute appendicitis and the diagnosis is made during exploration. Case report We report such a case which was diagnosed and managed by laparoscopy. A 27-year-old male presented with features suggestive of acute appendicitis. Preoperative imaging failed to diagnose the condition. Laparoscopy showed a segment of oedematous and haemorrhagic greater omentum adherent to the parietal wall over the right lower quadrant. The infarcted segment was excised and removed in a non permeable bag through the umbilical port. A short edited video of the operative findings and the procedure executed is also provided. Conclusion Primary segmental infarction of the greater omentum is an uncommon cause of right lower quadrant pain mimicking appendicitis. Laparoscopy is both diagnostic as well as therapeutic. PMID:18801201

Kavalakat, Alfie J; Varghese, Chalissery J

2008-01-01

110

Complex papillary hyperplasia of the endometrium: An uncommon case report with cytopathological features and diagnostic implications.  

PubMed

Papillary proliferations of the endometrium, without atypia have been uncommonly documented, including on cytology specimens. Herein, we present an uncommon case of a 55-year-old obese lady, on antihypertensive medications, who presented with history of irregular perimenopausal bleeding. A year ago, she was diagnosed with simple cystic hyperplasia on dilation and curettage specimen. Presently, she underwent endometrial aspiration. Cytology smears were prepared from the collected tissue specimen that was further submitted for histopathological analysis. Although the smears were initially diagnosed as negative for malignancy, the tissue sections were reported as a uterine papillary serous carcinoma (UPSC). Review of the smears revealed prominent overlapping clusters and papillary arrangements of relatively banal endometrial cells exhibiting focal metaplasia. Histopathology sections confirmed diagnosis of complex papillary hyperplasia (CPH). Immunohistochemical (IHC) stains reinforced this impression with diffuse estrogen receptor positivity, low Ki-67/MIB1, and lack of diffuse p53 immunostaining. Subsequent hysterectomy, at the time of intraoperative consultation showed a small residual focus of CPH, restricted to endometrium with intramural leiomyomas and adenomyosis. This case is presented to highlight the fact that despite lack of significant atypia, cytological features like overlapping, clustering, and papillary formations are indicators of papillary lesions of the endometrium, including CPH, especially in postmenopausal women. On histopathology, in spite of conspicuous papillary formations, lack of significant nuclear pleomorphism, and tumor invasion are helpful features in avoiding an overdiagnosis of UPSC in such cases. IHC stains are supportive. Correct identification has significant therapeutic implications. Diagn. Cytopathol. 2015;43:163-168. © 2014 Wiley Periodicals, Inc. PMID:24825243

Rekhi, Bharat; Menon, Santosh; Maheshwari, Amita

2015-02-01

111

Kienbock's disease and juvenile idiopathic arthritis  

PubMed Central

Kienbock's disease or osteonecrosis of the lunate is an uncommon cause of wrist pain. . Though there have been several reports of cases in patients with various rheumatologic diseases, the precise etiology has currently not been established. We report a case of Kienbock's disease that occurred in a patient with juvenile idiopathic arthritis. To our knowledge, this is the first case report with an association between these two conditions. PMID:22363188

Desy, Nicholas M.; Bernstein, Mitchell; Harvey, Edward J.; Hazel, Hazel

2011-01-01

112

Juvenile tricuspid stenosis and rheumatic tricuspid valve disease: an echocardiographic study  

Microsoft Academic Search

Tricuspid valve involvement is not uncommon in patients with rheumatic heart disease and is frequently missed on routine clinical examination. We prospectively studied the echocardiographic profile of tricuspid valve disease in 788 consecutive patients with rheumatic heart disease. Out of these patients 9% (70) had tricuspid valve disease and 55.7% (39) of these were of ?20 years of age. Of

Kewal C. Goswami; M. Bhaskara Rao; Vishava Dev; S. Shrivastava

1999-01-01

113

Mapping of the UGT1A locus identifies an uncommon coding variant that affects mRNA expression and protects from bladder cancer  

PubMed Central

A recent genome-wide association study of bladder cancer identified the UGT1A gene cluster on chromosome 2q37.1 as a novel susceptibility locus. The UGT1A cluster encodes a family of UDP-glucuronosyltransferases (UGTs), which facilitate cellular detoxification and removal of aromatic amines. Bioactivated forms of aromatic amines found in tobacco smoke and industrial chemicals are the main risk factors for bladder cancer. The association within the UGT1A locus was detected by a single nucleotide polymorphism (SNP) rs11892031. Now, we performed detailed resequencing, imputation and genotyping in this region. We clarified the original genetic association detected by rs11892031 and identified an uncommon SNP rs17863783 that explained and strengthened the association in this region (allele frequency 0.014 in 4035 cases and 0.025 in 5284 controls, OR = 0.55, 95%CI = 0.44–0.69, P = 3.3 × 10?7). Rs17863783 is a synonymous coding variant Val209Val within the functional UGT1A6.1 splicing form, strongly expressed in the liver, kidney and bladder. We found the protective T allele of rs17863783 to be associated with increased mRNA expression of UGT1A6.1 in in-vitro exontrap assays and in human liver tissue samples. We suggest that rs17863783 may protect from bladder cancer by increasing the removal of carcinogens from bladder epithelium by the UGT1A6.1 protein. Our study shows an example of genetic and functional role of an uncommon protective genetic variant in a complex human disease, such as bladder cancer. PMID:22228101

Tang, Wei; Fu, Yi-Ping; Figueroa, Jonine D.; Malats, Núria; Garcia-Closas, Montserrat; Chatterjee, Nilanjan; Kogevinas, Manolis; Baris, Dalsu; Thun, Michael; Hall, Jennifer L.; De Vivo, Immaculata; Albanes, Demetrius; Porter-Gill, Patricia; Purdue, Mark P.; Burdett, Laurie; Liu, Luyang; Hutchinson, Amy; Myers, Timothy; Tardón, Adonina; Serra, Consol; Carrato, Alfredo; Garcia-Closas, Reina; Lloreta, Josep; Johnson, Alison; Schwenn, Molly; Karagas, Margaret R.; Schned, Alan; Black, Amanda; Jacobs, Eric J.; Diver, W. Ryan; Gapstur, Susan M.; Virtamo, Jarmo; Hunter, David J.; Fraumeni, Joseph F.; Chanock, Stephen J.; Silverman, Debra T.; Rothman, Nathaniel; Prokunina-Olsson, Ludmila

2012-01-01

114

Languages of Sub-Saharan Africa. A Survey of Materials for the Study of the Uncommonly Taught Languages.  

ERIC Educational Resources Information Center

This is an annotated bibliography of basic tools of access for the study of the uncommonly taught languages of Sub-Saharan Africa. It is one of eight fascicles which constitute a revision of "A Provisional Survey of Materials for the Study of the Neglected Languages" (CAL 1969). The emphasis is on materials for the adult learner whose native…

Johnson, Dora E.; And Others

115

Four uncommon nanocage-based Ln-MOFs: highly selective luminescent sensing for Cu˛? ions and selective CO? capture.  

PubMed

Four uncommon isostructural nanocage-based 3D Ln-MOFs, 1-Ln (Ln = Eu, Tb, Gd and Dy), were constructed using a new diisophthalate ligand with active pyridyl sites. 1-Eu exhibits highly efficient luminescent sensing for Cu(2+) ions and selective CO2 capture. PMID:24963971

Liu, Bo; Wu, Wei-Ping; Hou, Lei; Wang, Yao-Yu

2014-08-14

116

Languages of Eastern Europe and the Soviet Union. A Survey of Materials for the Study of the Uncommonly Taught Languages.  

ERIC Educational Resources Information Center

This is an annotated bibliography of basic tools of access for the study of the uncommonly taught languages of Eastern Europe and the Soviet Union. It is one of eight fascicles which constitute a revision of "A Provisional Survey of Materials for the Study of the Neglected Languages" (CAL 1969). Although the focus is on materials for the…

Johnson, Dora E.; And Others

117

Languages of the Middle East and North Africa. A Survey of Materials for the Study of the Uncommonly Taught Languages.  

ERIC Educational Resources Information Center

This is an annotated bibliography of basic tools of access for the study of the uncommonly taught languages of the Middle East and North Africa. It is one of eight fascicles which constitute a revision of "A Provisional Survey of Materials for the Study of the Neglected Languages" (CAL 1969). The emphasis is on materials for the adult learner…

Johnson, Dora E.; And Others

118

Visceral Leishmaniasis with Associated Common, Uncommon, and Atypical Morphological Features on Bone Marrow Aspirate Cytology in Nonendemic Region  

PubMed Central

Objectives. The present study was conducted to categorise the morphological features on bone marrow aspirate cytology into common, uncommon, and atypical features in a nonendemic region which would be helpful in clinching an early and correct diagnosis especially in clinically unsuspected cases. Methods. The morphological features on bone marrow were categorized into common, uncommon, and atypical in cases of leishmaniasis from non endemic region. Results. Out of total 27 cases, 77.7% were residents of places at the height of 500?m or above and fever was the most common presentation followed by hepatosplenomegaly. Plasmacytosis, hemophagocytosis were the common cytological features while dysmyelopoiesis, presence of leishmania bodies in nonhistiocytic cells, and granuloma with necrosis were uncommon features. Aggregates of LD bodies in form of ring, floret, or strap shapes along with giant cells constitute the atypical morphological features. Conclusion. The knowledge of common, uncommon, and atypical features on bone marrow aspirate cytology is helpful in clinching an early and correct diagnosis of leishmaniasis especially in non endemic areas where clinical suspicion is low. These features will guide the pathologist for vigilant search of LD bodies in the marrow for definite diagnosis and thus will also be helpful in preventing unnecessary workups. PMID:24089618

Chandra, Harish; Kaushik, Rajeev Mohan

2013-01-01

119

Uncommon formation of two antiparallel sperm bundles per cyst in tenebrionid beetles (Coleoptera)  

NASA Astrophysics Data System (ADS)

Several species of Tenebrionidae are stored-grain pests. Since they belong to a specious family, the systematics of these beetles is still in doubt. In insects, spermatogenesis and the spermatozoa exhibit great diversity, and are therefore commonly used in phylogenetic and taxonomic analyses. During the spermatogenetic process in these organisms, the cells originating from a single spermatogonium develop synchronically in groups referred to as cysts. At the end of this process, there is usually only one sperm bundle per cyst, with all the cells in the same orientation. This paper details the spermiogenesis of the tenebrionid beetles Tenebrio molitor, Zophobas confusa, Tribolium castaneum and Palembus dermestoides using whole mount and histological sections of the cysts. In these species, spermatogenesis is similar to that which occurs in most insects. However, during spermiogenesis, the nuclei of the spermatids migrate to two opposite regions at the periphery of the cyst, leading to the uncommon formation of two bundles of spermatozoa per cyst. This feature is possibly an apomorphy for Tenebrionidae.

Dias, Glenda; Yotoko, Karla S. C.; Gomes, Luiz F.; Lino-Neto, José

2012-09-01

120

Fulminant myocarditis demonstrating uncommon morphology--a report of two autopsy cases.  

PubMed

Two autopsy cases of fulminant myocarditis demonstrating uncommon morphology were studied. Subjects included two male patients: a 42-year-old (case 1) and a 39-year-old (case 2). Both cases had fever, chest or epigastric pain, electrocardiographic abnormalities, prominent elevation of serum glutamic oxaloacetic transaminase, glutamic pyruvic transaminase, lactic dehydrogenase and creatine phosphokinase. They were treated with intra-aortic balloon pumping and percutaneous cardiopulmonary support, and they died at 3 days and 4 days after admission (total course of 10 days and 9 days), respectively. Case 1 showed focal necrosis, severe myocardial dystrophic calcification positive for Kossa stain, inflammatory edema, lymphocyte and macrophage infiltration and extravasation of erythrocytes. Case 2 showed acute inflammation and severe myocardial necrosis with neutrophilic abscess, lymphocyte and macrophage infiltration, cell debris and purulent exudate. Calcified, degenerative and necrotic cardiac myocytes and macrophages were reacted with anti-Enterovirus antibody (clone 5-D8/1), which recognizes an epitope on the VP1 peptide of most Coxsackievirus, echovirus, poliovirus and enterovirus strains. Therefore, the present two cases may be compatible with fulminant enterovirus-associated myocarditis. Using reverse transcriptase-semi-nested polymerase chain reaction, picornaviral RNA was detected in the amplified products extracted from the paraffin-embedded myocardial sample of case 1 but not in case 2. PMID:15668804

Oka, Kuniyuki; Oohira, Koji; Yatabe, Yasushi; Tanaka, Toshio; Kurano, Kozo; Kosugi, Rie; Murata, Minoru; Hakozaki, Hando; Nishikawa, Toshio; Tsutsumi, Yutaka

2005-03-01

121

Suprasellar Mature Cystic Teratoma: An Unusual Location for an Uncommon Tumor  

PubMed Central

Intracranial germ cell tumors are uncommon and account for only 0.3–3.4% of all intracranial tumors. Teratomas are a subset of these neoplasms, and their finding in brain structures is exceptionally rare, and occurrence within the skull base is quite novel. The authors report the case of a 57-year-old male patient who presented with vision changes, incontinence, ataxia, and altered mental status of 1 week's duration. Imaging revealed a large intrasellar mass with suprasellar extension, involvement of the ventricular system, and marked hydrocephalus with the enlargement of the lateral and third ventricles. The patient underwent a pterional craniotomy/transsylvian approach for resection of the mass. Postoperative histological examination of the resected mass was confirmatory for a mature cystic teratoma. This was followed by radiotherapy, stereotactic radiosurgery, and adjuvant radiotherapy. At the most recent followup, approximately 4 years later, the patient is doing well with improved vision since the operation. This report highlights our experience with a teratoma in a very unusual location, and we review the relevant literature. PMID:24198987

Sweiss, Raed B.; Sweiss, Fadi B.; Dalvin, Lauren; Siddiqi, Javed

2013-01-01

122

[Uncommon etiology of acute abdomen in pediatric age: the torsion of spleen].  

PubMed

The torsion of spleen on its vascular shank represents an uncommon problem, responsible of acute and chronic pain. The mobile spleen is fixed only through hilus vessels the gastrosplenic ligament. The incidence is unknow, greater in the male with an M:F ratio 6.1 in the first ten years of life, even if an episode of intrauterine torsion has been reported. The diagnosis can be performed with ultrasonography, angiography, scintigraphy and CT scan. There are reported two cases: male of 2.5 years female of 14 years who presented with recurrent pain to the left side, vomit diarrhoea and fever. Objectively a palpable mass was present. Ultrasonography and angio-CT scan of abdomen revealed splenomegaly, ptosis of the spleen and malrotation with signs of obstruction of the vessels. The treatment in both cases was splenectomy. The spleen appeared rotated on its shank and increased of volume, deprived of anatomical structures of fixation. The histological report confirmed the haemorrhagic infarction. The excessive mobility of the spleen, from insufficiency or absence of the ligamentous attachments is case of abdominal pain or acute abdomen, that can complicate with the infarction of the spleen. Angio-CT scan, in the cases here reported, has shown to greater sensibility in comparison to the ultrasonography. The Authors believe that the video-laparoscopic splenopexy, when the diagnosis is made of "wandering spleen" with painful repeated episodes, can be finalized, to the preservation of organ. PMID:15847092

Marinaccio, F; Caldarulo, E; Nobili, M; Magistro, D; Marinaccio, M

2005-01-01

123

Epidemiology of Peyronie's disease.  

PubMed

Francois Gigot de la Peyronie, surgeon to Louis XV of France, has become synonymous with the rather enigmatic though not uncommon condition of Peyronie's disease (PD), a localized connective tissue disorder of the penile tunica albuginea. The true prevalence of Peyronie's disease is unknown. Therefore, we decided to perform an evaluation of existing epidemiological data. A prevalence rate of 3.2% was determined in male inhabitants of the greater Cologne area. This is much higher than revealed by the data reported up to now, thus rendering the accepted prevalence rates of 0.3% to 1% untenable. The actual prevalence of Peyronie's disease may be even higher, considering many patients' reluctance to report this embarrassing condition to their physicians. Along these lines, most clinicians note that the number of Peyronie's patients has increased since the advent of oral sildenafil. Comparably high prevalences are known for diabetes and urolithiasis, suggesting a greater frequency of this rare disease than formerly believed. PMID:12454689

Sommer, F; Schwarzer, U; Wassmer, G; Bloch, W; Braun, M; Klotz, T; Engelmann, U

2002-10-01

124

Inflammatory bowel disease in exclusively breast-fed infants.  

PubMed

Inflammatory bowel disease is uncommon in infants. We present the clinical, endoscopic and pathologic findings for two exclusively breast-fed infants with a diagnosis of inflammatory bowel disease. We emphasize that although inflammatory bowel disease is rare in infants, chronic bloody diarrhea must be a sufficiently alarming symptom to consider a differential diagnosis of inflammatory bowel disease in young infants is considered, even when they are exclusively breast-fed. PMID:17060132

Kulo?lu, Zarife; Kansu, Aydan; Kirsaçlio?lu, Ceyda; Ince, Erdal; Ensari, Arzu; Girgin, Nurten

2006-11-01

125

Positioning patients for spine surgery: Avoiding uncommon position-related complications  

PubMed Central

Positioning patients for spine surgery is pivotal for optimal operating conditions and operative-site exposure. During spine surgery, patients are placed in positions that are not physiologic and may lead to complications. Perioperative peripheral nerve injury (PPNI) and postoperative visual loss (POVL) are rare complications related to patient positioning during spine surgery that result in significant patient disability and functional loss. PPNI is usually due to stretch or compression of the peripheral nerve. PPNI may present as a brachial plexus injury or as an isolated injury of single nerve, most commonly the ulnar nerve. Understanding the etiology, mechanism and pattern of injury with each type of nerve injury is important for the prevention of PPNI. Intraoperative neuromonitoring has been used to detect peripheral nerve conduction abnormalities indicating peripheral nerve stress under general anesthesia and to guide modification of the upper extremity position to prevent PPNI. POVL usually results in permanent visual loss. Most cases are associated with prolonged spine procedures in the prone position under general anesthesia. The most common causes of POVL after spine surgery are ischemic optic neuropathy and central retinal artery occlusion. Posterior ischemic optic neuropathy is the most common cause of POVL after spine surgery. It is important for spine surgeons to be aware of POVL and to participate in safe, collaborative perioperative care of spine patients. Proper education of perioperative staff, combined with clear communication and collaboration while positioning patients in the operating room is the best and safest approach. The prevention of uncommon complications of spine surgery depends primarily on identifying high-risk patients, proper positioning and optimal intraoperative management of physiological parameters. Modification of risk factors extrinsic to the patient may help reduce the incidence of PPNI and POVL. PMID:25232519

Kamel, Ihab; Barnette, Rodger

2014-01-01

126

Positioning patients for spine surgery: Avoiding uncommon position-related complications.  

PubMed

Positioning patients for spine surgery is pivotal for optimal operating conditions and operative-site exposure. During spine surgery, patients are placed in positions that are not physiologic and may lead to complications. Perioperative peripheral nerve injury (PPNI) and postoperative visual loss (POVL) are rare complications related to patient positioning during spine surgery that result in significant patient disability and functional loss. PPNI is usually due to stretch or compression of the peripheral nerve. PPNI may present as a brachial plexus injury or as an isolated injury of single nerve, most commonly the ulnar nerve. Understanding the etiology, mechanism and pattern of injury with each type of nerve injury is important for the prevention of PPNI. Intraoperative neuromonitoring has been used to detect peripheral nerve conduction abnormalities indicating peripheral nerve stress under general anesthesia and to guide modification of the upper extremity position to prevent PPNI. POVL usually results in permanent visual loss. Most cases are associated with prolonged spine procedures in the prone position under general anesthesia. The most common causes of POVL after spine surgery are ischemic optic neuropathy and central retinal artery occlusion. Posterior ischemic optic neuropathy is the most common cause of POVL after spine surgery. It is important for spine surgeons to be aware of POVL and to participate in safe, collaborative perioperative care of spine patients. Proper education of perioperative staff, combined with clear communication and collaboration while positioning patients in the operating room is the best and safest approach. The prevention of uncommon complications of spine surgery depends primarily on identifying high-risk patients, proper positioning and optimal intraoperative management of physiological parameters. Modification of risk factors extrinsic to the patient may help reduce the incidence of PPNI and POVL. PMID:25232519

Kamel, Ihab; Barnette, Rodger

2014-09-18

127

Orbital solitary fibrous tumor with multinucleate giant cells: case report of an unusual finding in an uncommon tumor.  

PubMed

Solitary fibrous tumor (SFT) is a rare soft-tissue neoplasm which may occur at any site although it is more frequent in the pleura, mediastinum and lung. Orbital involvement by SFT is uncommon. Giant cells are extremely rare to be seen in a SFT and have been described to be immunoreactive for CD34. We present a case of orbital SFT with multinucleate giant cells expressing CD68 and lacking immunoreactivity for CD34. The differential diagnosis is discussed. PMID:24152511

Mulay, Kaustubh; Honavar, Santosh G

2013-01-01

128

Undulating tongue in Wilson's disease.  

PubMed

We report an unusual occurrence of involuntary movement involving the tongue in a patient with confirmed Wilson's disease (WD). She manifested with slow, hypophonic speech and dysphagia of 4 months duration, associated with pseudobulbar affect, apathy, drooling and dystonia of upper extremities of 1 month duration. Our patient had an uncommon tongue movement which was arrhythmic. There was no feature to suggest tremor, chorea or dystonia. It might be described as athetoid as there was a writhing quality, but of lesser amplitude. Thus, the phenomenology was uncommon in clinical practice and the surface of the tongue was seen to "ripple" like a liquid surface agitated by an object or breeze. Isolated lingual dyskinesias are rare in WD. It is important to evaluate them for WD, a potentially treatable disorder. PMID:25024581

Nagappa, M; Sinha, S; Saini, Js; Bindu, Ps; Taly, Ab

2014-04-01

129

Castleman's Disease of the Porta Hepatis.  

PubMed

Castleman's disease is an uncommon cause of lymph node enlargement that mostly involves the mediastinum and lung hilum. It is divided into 2 types: localized, which is usually asymptomatic and presents with a mass lesion; and multicentric, which is characterized by chills, anemia, generalized lymphadenopathy and hepatosplenomegaly. An extrathoracic site of involvement is very uncommon, and may be located in the mesentery of the intestines, axilla, and pelvis. Hepatic localization of this disease is a rare event. Herein, we report our experience with a symptomatic case of Castleman's disease in the porta hepatis who has been treated successfully by excision of the hilar lymph nodes, but recurred after 2 years with the same clinical picture plus abnormal liver function tests. However, at the time of recurrence ,no lymphadenopathy was detected and liver biopsy showed giant lymphoid follicles with germinal centers. She was treated with steroids and showed a dramatic response. PMID:24829655

Geramizadeh, Bita; Fattahi, Mohammad Reza; Bagheri Lankarani, Kamran

2012-07-01

130

Spinal epidural abscess – a rare complication of inflammatory bowel disease  

PubMed Central

Spinal epidural abscess is an uncommon but highly morbid illness. While it usually afflicts older, immunocompromised patients, this condition has been reported as a result of intestinal perforation in the setting of inflammatory bowel disease. Two cases of spinal epidural abscess in patients with inflammatory bowel disease are reported: one in a patient with Crohn’s disease and one in a patient with ulcerative colitis after restorative proctocolectomy. PMID:18299737

Brown, Carl J; Jaffer, Hussein; Jaffer, Nasir; Burul, Claude; McLeod, Robin S

2008-01-01

131

An uncommon localization of a giant hydatid cyst presenting with cardiac tamponade.  

PubMed

Cardiac involvement in hydatid cyst disease is a rare manifestation and may remain asymptomatic for years. The location of the cyst determines the presentation. In this report, we present a case of hydatid cyst presenting with massive pericardial effusion, right ventricle compression leading to cardiac tamponade. The diagnosis was established with transthoracic, transesophageal echocardiagraphy and computed tomography. PMID:25655857

?ahin, ?rfan; Özkaynak, Berk; Ayça, Burak; Okuyan, Ertu?rul

2015-01-01

132

MR imaging of uncommon recurrence of fibrolipomatous hamartoma of the ulnar nerve.  

PubMed

We present the MR and histopathologic findings of fibrolipomatous hamartoma (FLH) of the ulnar nerve in a 54-year-old woman, a lipomatous process that rarely affects the ulnar nerve. The case illustrated is further unusual as a local soft tissue recurrent mass developed over a remarkably long course of the disease. PMID:12752006

Kakitsubata, Y; Theodorou, S J; Theodorou, D J; Shibata, M; Yuge, M; Yuki, Y; Hatakeyama, K; Yokouchi, T

2003-05-01

133

Extraskeletal osteosarcoma: An uncommon variant with rare metastatic sites detected with FDG PET/CT  

PubMed Central

Extraskeletal osteosarcoma (ESOS) is a rare malignancy, which commonly presents with metastatic disease. Like their osteogenic counterparts, these tumors commonly metastasize to lungs and bones. We report the fluoro-deoxyglucose positron emission tomography findings in a case of ESOS presenting with a combination of rare metastatic sites such as brain, kidney and the bone marrow. PMID:25006295

Puranik, Ameya D.; Purandare, Nilendu C.; Bal, Munita M.; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

2014-01-01

134

Extraskeletal osteosarcoma: An uncommon variant with rare metastatic sites detected with FDG PET/CT.  

PubMed

Extraskeletal osteosarcoma (ESOS) is a rare malignancy, which commonly presents with metastatic disease. Like their osteogenic counterparts, these tumors commonly metastasize to lungs and bones. We report the fluoro-deoxyglucose positron emission tomography findings in a case of ESOS presenting with a combination of rare metastatic sites such as brain, kidney and the bone marrow. PMID:25006295

Puranik, Ameya D; Purandare, Nilendu C; Bal, Munita M; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

2014-01-01

135

Detection and characterization of classical and “uncommon” exon 19 Epidermal Growth Factor Receptor mutations in lung cancer by pyrosequencing  

PubMed Central

Background The management of advanced stage non-small cell lung cancer is increasingly based on diagnostic and predictive analyses performed mostly on limited amounts of tumor tissue. The evaluation of Epidermal Growth Factor Receptor (EGFR) mutations have emerged as the strongest predictor of response to EGFR-tyrosine kinase inhibitors mainly in patients with adenocarcinoma. Several EGFR mutation detection techniques are available, having both sensitivity and specificity issues, being the Sanger sequencing technique the reference standard, with the limitation of a relatively high amount of mutated cells needed for the analysis. Methods A novel nucleotide dispensation order for pyrosequencing was established allowing the identification and characterization of EGFR mutation not definable with commercially and clinically approved kits, and validated in a consecutive series of 321 lung cancer patients (246 biopsies or cytology samples and 75 surgical specimens). Results 61/321 (19%) mutated cases were detected, 17 (27.9%) in exon 21 and 44 (72.1%) in exon 19, these latter corresponding to 32/44 (72.7%) classical and 12/44 (27.3%) uncommon mutations. Furthermore, a novel, never reported, point mutation, was found, which determined a premature stop codon in the aminoacidic sequence that resulted in a truncated protein in the tyrosine kinase domain, thus impairing the inhibitory effect of specific therapy. Conclusions The novel dispensation order allows to detect and characterize both classical and uncommon EGFR mutations. Although several phase III studies in genotypically defined groups of patients are already available, further prospective studies assessing the role of uncommon EGFR mutations are warranted. PMID:23497146

2013-01-01

136

Extensive dural sinus thrombosis and bilateral lateral rectus palsy as an uncommon complication of chronic suppurative otitis media  

PubMed Central

Dural venous sinus thrombosis, especially of the sigmoid sinus, is a known but uncommon intracranial extradural complication of chronic suppurative otitis media. Even rarer is the simultaneous occurrence of bilateral abducens palsy in the same patient. We report the case of an adolescent male who presented with signs of raised intracranial pressure, diplopia and bilateral lateral rectus palsy associated with a history of left ear discharge and neck swelling. Extensive dural sinus thrombosis extending right up to the left internal jugular vein was confirmed on CT imaging. The patient was successfully treated with thrombolytic agents and antibiotic therapy. The pathophysiology of the concurrent complications is discussed. PMID:23355565

Balasubramanian, Anusha; Mohamad, Irfan; Sidek, Dinsuhaimi

2013-01-01

137

Vanishing lung syndrome and HIV infection: an uncommon yet potentially fatal sequela of cigarette smoking.  

PubMed

Persons with HIV infection have a higher risk of infectious pulmonary complications, chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, and pulmonary fibrosis than individuals not infected with HIV. Herein, we describe the clinical course of a patient with longstanding and well-controlled HIV infection and multiple previous pneumothoraces who presented to medical attention with insidious onset of shortness of breath and was diagnosed with vanishing lung syndrome (VLS). The VLS or giant bullous emphysema is a distinct clinical syndrome characterized by large bullae, predominantly in the upper lobes, occupying at least one third of the hemithorax and compressing surrounding normal lung parenchyma. It is a progressive disorder that typically occurs in young men, the majority of whom are smokers. As people with HIV/AIDS are now surviving well into middle age and beyond, clinicians are more likely to encounter VLS and severe obstructive lung disease, which are potentially fatal but preventable conditions. PMID:22564798

Mani, Deepthi; Guinee, Donald G; Aboulafia, David M

2012-01-01

138

Dengue eye disease.  

PubMed

Dengue fever, a viral disease epidemic in some parts of the world, is of considerable international concern, with a growing incidence owing to developing urbanization, tourism, and trade. Ocular manifestations of dengue fever are uncommon, but of great significance. Proposed mechanisms include direct viral infection as well as immunologic phenomena. Common manifestations include subconjunctival, vitreous, and retinal hemorrhages; posterior uveitis; optic neuritis; and maculopathies such as foveolitis, hemorrhage, and edema. Main symptoms include blurring of vision, scotomata, metamorphopsia, and floaters. Diagnostic and monitoring investigations described included optical coherence tomography, fundus fluorescein and indocyanine green angiography, visual field analysis, and electrophysiologic tests. Management is based on clinical presentation and includes active surveillance as well as various anti-inflammatory and immunosuppressive therapies. There have been no prospective, randomized therapeutic trials, and it is unclear if the disease is self-limiting or if treatment is actually beneficial. Prognosis varies, ranging from full resolution to permanent vision loss despite intervention. PMID:25223497

Ng, Aaron W; Teoh, Stephen C

2014-08-12

139

Changes in Semantic Memory in Early Stage Alzheimer's Disease Patients.  

ERIC Educational Resources Information Center

Contrasts changes in semantic memory in elderly normal controls and Alzheimer's disease (AD) patients before patients expressed symptoms. Found that controls generated more uncommon exemplars from closed semantic categories (fruits and vegetables) than did AD patients prior to presumed onset of AD. AD patients were just as productive as controls…

Weingartner, Herbert J.; And Others

1993-01-01

140

Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-a rare association within an uncommon tumor.  

PubMed

PEComa, defined as a perivascular epithelioid cell tumor, displays a wide clinicopathological spectrum. Lately, a sclerosing PEComa has been identified as its distinct variant, but with limited documentation, in view of its rarity. Herein, we describe an uncommon case of a 53-year-old lady, who was referred to us with pain abdomen. Radiological imaging disclosed a well-defined, hypodense retroperitoneal mass. The excised tumor was a round, encapsulated soft tissue mass measuring 7 cm with a tan-brown cut surface. Microscopy showed uniform, epithelioid cells with clear cytoplasm, focal melanin pigmentation and mild nuclear atypia, arranged in sheets and nests around capillary-sized vessels in a dense sclerotic stroma. Additionally, co-existing epithelioid granulomas were noted. On immunohistochemistry (IHC), tumor cells were diffusely positive for HMB45; focally for desmin and smooth muscle actin (SMA), while negative for EMA, CD10, S100-P, Melan A, CD34, AMACR and CK MNF116. This case reinforces sclerosing PEComa as an uncommon, but a distinct clinicopathological entity and exemplifies diagnostic challenge associated with it; necessitating application of IHC markers for its correct identification. Presence of melanin pigment and granulomatous inflammation in the present tumor constitute as novel histopathological findings in a sclerosing PEComa. PMID:23032844

Rekhi, Bharat; Sable, Mukund; Desai, Sangeeta B

2012-01-01

141

An experimental and theoretical study to relate uncommon rock/fluid properties to oil recovery. Final report  

SciTech Connect

Waterflooding is the most commonly used secondary oil recovery technique. One of the requirements for understanding waterflood performance is a good knowledge of the basic properties of the reservoir rocks. This study is aimed at correlating rock-pore characteristics to oil recovery from various reservoir rock types and incorporating these properties into empirical models for Predicting oil recovery. For that reason, this report deals with the analyses and interpretation of experimental data collected from core floods and correlated against measurements of absolute permeability, porosity. wettability index, mercury porosimetry properties and irreducible water saturation. The results of the radial-core the radial-core and linear-core flow investigations and the other associated experimental analyses are presented and incorporated into empirical models to improve the predictions of oil recovery resulting from waterflooding, for sandstone and limestone reservoirs. For the radial-core case, the standardized regression model selected, based on a subset of the variables, predicted oil recovery by waterflooding with a standard deviation of 7%. For the linear-core case, separate models are developed using common, uncommon and combination of both types of rock properties. It was observed that residual oil saturation and oil recovery are better predicted with the inclusion of both common and uncommon rock/fluid properties into the predictive models.

Watson, R.

1995-07-01

142

Molecular and clinical analysis in a series of patients with Pyknodysostosis reveals some uncommon phenotypic findings  

PubMed Central

Pyknodysostosis is a rare autosomal recessive skeletal dysplasia characterized by short stature, deformity of the skull, osteosclerosis, hypoplasia of the clavicle, and bone fragility. Radiographs show increased bone density, osteosclerosis, and acroosteolysis of the terminal phalanges. The pycnodysostosis gene is located on chromosome 1q21 and encodes an enzyme called Cathepsin K. Cathepsin K is a cysteine protease lysosomal protein associated with the degradation of bone and cartilage. In the current study, the authors described the clinical, radiological and molecular features of a group of six Mexican patients, including two familial and two sporadic cases, with Pyknodysostosis. One of the patients presented hypoacusia, an unusual finding in this disease. PMID:25550899

Valdes-Flores, Margarita; Hidalgo-Bravo, Alberto; Casas-Avila, L; Chima-Galan, Carmen; Hazan-Lasri, Eric J; Pineda-Gomez, Ernesto; Lopez-Estrada, Druso; Zenteno, Juan C

2014-01-01

143

The role of fecal elastase-1 in detecting exocrine pancreatic disease  

Microsoft Academic Search

Exocrine pancreatic disease is thought to be uncommon in clinical practice and usually secondary to excess alcohol intake. Although excess alcohol intake does account for many cases of exocrine pancreatic disease, other conditions are associated with exocrine pancreatic insufficiency and such dysfunction perhaps occurs more frequently than conventionally expected. A reliable, patient-friendly, cheap and easy to use test for exocrine

Kofi Oppong; David S. Sanders; John S. Leeds

2011-01-01

144

Melanized Fungi in Human Disease  

PubMed Central

Summary: Melanized or dematiaceous fungi are associated with a wide variety of infectious syndromes. Many are soil organisms and are generally distributed worldwide, though certain species appear to have restricted geographic ranges. Though they are uncommon causes of disease, melanized fungi have been increasingly recognized as important pathogens, with most reports occurring in the past 20 years. The spectrum of diseases with which they are associated has also broadened and includes allergic disease, superficial and deep local infections, pneumonia, brain abscess, and disseminated infection. For some infections in immunocompetent individuals, such as allergic fungal sinusitis and brain abscess, they are among the most common etiologic fungi. Melanin is a likely virulence factor for these fungi. Diagnosis relies on careful microscopic and pathological examination, as well as clinical assessment of the patient, as these fungi are often considered contaminants. Therapy varies depending upon the clinical syndrome. Local infection may be cured with excision alone, while systemic disease is often refractory to therapy. Triazoles such as voriconazole, posaconazole, and itraconazole have the most consistent in vitro activity. Further studies are needed to better understand the pathogenesis and optimal treatment of these uncommon infections. PMID:20930077

Revankar, Sanjay G.; Sutton, Deanna A.

2010-01-01

145

Hemophagocytic syndrome as uncommon presentation of disseminated toxoplasmosis in an immunocompetent adult from Chinese Kunming.  

PubMed

Hemophagocytic syndrome is a rare disease that is often fatal, despite treatment. An immunocompetent patient was presented with fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia, which conformed to a hemophagocytic syndrome diagnosis. Despite broad antibiotic treatment, the patient's clinical condition rapidly deteriorated and he died within 8 days of admission. Blood cultures and a serology test were negative; however, based on morphological characteristics, tissue cysts Toxoplasma gondii were found in the bone marrow. Based on polymerase chain reaction analysis, identity of the parasite was confirmed. Although very rare, T. gondii-associated hemophagocytic syndrome should be suspected in the case of cytopenia or multiorgan failure symptoms. To our knowledge, this is the first fatal toxoplasmosis case reported from China. PMID:23509123

Yang, YanFen; Zuo, WeiWei; Hu, JunJie; Esch, Gerald W; Zuo, YangXian

2013-06-01

146

[Thrombosis of a popliteal pseudoaneurysm secondary to an osteochondroma of the femur: an uncommon clinical case].  

PubMed

Osteochondroma are the bone tumors most frequently found in adolescents or young adults. Most often discovered by accidental findings, they however may be diagnosed while some complications. They are commonly described as solitary findings while multiple exostoses are rather included in an inherited disease. We report the rare case of a 24-year-old patient with acute ischemia of popliteal artery pseudoaneurysm caused by a femur bone exostosis. He presented a severe and supported pain in the left leg. Clinical examination found acute limb ischemia. An angio-CT-scan showed a pseudoaneurysm thrombosis of popliteal artery with distal emboli. An exostosis of the femur bone just above the aneurysm was also found. In this dramatic presentation, a surgical treatment was performed and consisted of thrombectomy, excluding the popliteal aneurysm with a distal saphenous vein graft, excision of the exostosis, fibrinolysis and preventive anterior and posterior compartements fasciotomy. PMID:25672013

Negulescu, M; Arend, P

2014-01-01

147

Primary neuroendocrine tumors of the kidney: morphological and molecular alterations of an uncommon malignancy.  

PubMed

Primary neuroendocrine (NE) tumors of the kidney (PNRTs) are rare and frequently mistaken for other renal and urothelial cancers. We evaluated morphological and molecular findings of 11 PNRTs classified according to the World Health Organization classification of lung NE tumors. Patients included 5 men and 6 women with a median age of 50 years. These tumors occurred in the left (5/11), right (3/11), and horseshoe (1/11) kidney. The histologic patterns were predominantly solid, trabecular, and pseudoglandular. Lymphovascular invasion and calcification were found in 3 and 1 cases, respectively. There were 2 atypical and 9 typical carcinoids. At the time of surgery, 2 patients with atypical carcinoids had hepatic metastasis, and 1 of the typical carcinoid patients had lymph node metastasis. All cases showed <1% proliferative rate, except 2 cases with hepatic metastasis, which showed 3% to 5% with MIB1/Ki-67 immunostaining. Immunostainings were frequently positive for synaptophysin, chromogranin, CD56, CD99, and neuron-specific enolase. Follow-up data (average 4 years) were available for 6 patients. Two patients with distant metastasis were alive with disease, and four patients with no metastasis were alive without disease. We evaluated the association of PNRT and loss of heterozygosity (LOH) on chromosome 3p21 and found LOH in 2 of 3 cases. However, the comparative genomic hybridization study (2/2) did not demonstrate significant chromosomal imbalances. We conclude that PNRTs are positive for NE markers and may have LOH on chromosome 3p21. PNRTs should be classified as NE tumors in other sites, and proliferative rate can be an indicator of aggressive behavior/metastasis. PMID:23199527

Aung, Phyu P; Killian, Keith; Poropatich, Carrie O; Linehan, W Marston; Merino, Maria J

2013-05-01

148

Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement.  

PubMed

Rosai-Dorfman disease, that is, sinus histiocytosis with massive lymphadenopathy is a benign systemic proliferative disorder of histiocytes. The typical clinical presentation of the disease includes bilateral painless massive lymphadenopathy, fever and polyclonal hypergammaglobulinemia. Extranodal involvement is present in only a few cases and skin lesions are the most common form of extranodal disease. However, purely cutaneous Rosai-Dorfman disease is uncommon. In this study, we describe a 10-year-old child presenting with bilateral ocular involvement. PMID:21897550

Kala, Chayanika; Agarwal, Asha; Kala, Sanjay

2011-07-01

149

Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement  

PubMed Central

Rosai-Dorfman disease, that is, sinus histiocytosis with massive lymphadenopathy is a benign systemic proliferative disorder of histiocytes. The typical clinical presentation of the disease includes bilateral painless massive lymphadenopathy, fever and polyclonal hypergammaglobulinemia. Extranodal involvement is present in only a few cases and skin lesions are the most common form of extranodal disease. However, purely cutaneous Rosai-Dorfman disease is uncommon. In this study, we describe a 10-year-old child presenting with bilateral ocular involvement. PMID:21897550

Kala, Chayanika; Agarwal, Asha; Kala, Sanjay

2011-01-01

150

Simultaneous development of Dieterich disease and Freiberg disease.  

PubMed

Dieterich disease is an uncommon arthropathy of the hand, with few studies published. This lesion shares a similar etiopathogenesis with Freiberg disease, although the association of both conditions has only been described once. We report a 65-year-old man consulting for inflammatory pain in his right hand of 1 month's duration and also in his right foot of 4 months' duration. The rheumatology department was consulted to rule out systemic disease because the synovitis had occurred simultaneously in 2 different locations. The plain radiography and magnetic resonance imaging findings supported the diagnosis of Dieterich disease and Freiberg disease, although only increased uptake was found on scintigraphy in the affected zones. Few studies have been published about Dieterich disease, most in case report form. To our knowledge, only 1 study has described the association of Dieterich disease and Freiberg disease. Surgical treatment has been described when conservative management is unsuccessful, with multiple techniques used. The present case is the first in which Dieterich disease and Freiberg disease manifested simultaneously in the initial painful inflammatory phase. PMID:23363812

Conesa, Xavier; González, Xavier; Siles, Eduard; Parals, Fčlix; Novell, Josep

2013-01-01

151

An unusual cause of pancytopenia: Whipple's disease  

PubMed Central

Whipple's disease is a systemic infectious disease caused by the bacteria Tropheryma whipplei. The most common clinical manifestations of Whipple's disease are weight loss (92%), hypoalbuminemia and steatorrhea (91%, respectively), diarrhea (72%), arthralgia (67%), and abdominal pain (55%). Neurological signs and symptoms from dementia to oculomasticatory myorhythmia or oculofacioskeletal myorhythmia (pathognomonic of Whipple's disease), lymphadenopathy, and fatigue can also be present. Pancytopenia is a rare and less recognized clinical feature in Whipple's disease patients. We are describing a case where a middle-aged Caucasian male diagnosed with Whipple's disease was found to have pancytopenia. Etiology of pancytopenia is postulated to be due to the invasion of bone marrow by T. whipplei. It is important to recognize that bone marrow involvement by the Whipple bacillus is not uncommon. In the presence of lymphadenopathy and pancytopenia, clinicians should think of Whipple's disease as a differential diagnosis apart from lymphoma or other non-specific granulomatous reticuloendothelial disorders. PMID:24765256

Tun, Nay T.; Shukla, Shwetanshu; Krishnakurup, Jaykrishnan; Pappachen, Binu; Krishnamurthy, Mahesh; Salib, Hayman

2014-01-01

152

Nutrient composition, phenolic content and free radical scavenging activity of some uncommon vegetables of Pakistan.  

PubMed

Vegetables play a vital role in the prevention of human disease and in the improvement of general health as these contain vitamins, amino acids, fiber, antioxidants and minerals. In the present study, some less familiar vegetables of Pakistan namely chickpea (leaves), chungah (shoots), drumstick tree (inflorescences), radish (fruit pods), mountain ebony (flower buds), mustard (leaves), purslane (leaves) and white goosefoot (leaves and shoots) were evaluated for proximate composition, mineral content, phenolic content and free radical scavenging activity. The protein, fat, fiber, carbohydrate and ash contents of the selected vegetables were in the range of 2.9 to 6.6%, 0.2 to 2.5%, 2.4 to 8.6%, 9.7 to 20.1% and 1.0 to 2.3%, respectively. The concentration of vitamin C ranged between 32.6 to 120.1 mg/100 g. The phosphorus, calcium, iron, zinc, manganese, magnesium and copper were 190 to 3400, 103 to 987, 19 to 93, 12 to 47, 9 to 121, 299 to 1635 and non detectable level to 42 mg/kg, respectively. The amount of total phenolic content varied from 55.3 to 221.0 mg/g in the dry methanolic extracts of the studied plants. The EC(50) values were below 1400 ?g/ml, indicating that all the studied vegetables have good scavenging effect on DPPH radical. PMID:21715260

Khattak, Khanzadi Fatima

2011-07-01

153

Parathyroid carcinoma and oxyphil parathyroid adenoma: an uncommon case of misinterpretation in clinical practice.  

PubMed

A 46 year-old male presented with persistently high level of serum parathyroid hormone (PTH), despite successful resection of an oxyphilic cell parathyroid adenoma of the left lower gland. Renal function and serum calcium were normal, leading to vitamin D deficiency being considered. Tc99m-sestamibi parathyroid scintigraphy showed no capitation, but a cervical ultrasound demonstrated an increase in the lower parathyroids. Surgery confirmed that the right gland was normal but the left corresponded to parathyroid carcinoma. The patient developed severe hypocalcemia, with PTH values being consistent with hypoparathyroidism for a few months. However, a progressive increase in calcium and PTH serum levels indicated recurrence of disease. Tc99m-sestamibi scintigraphy demonstrated hyperfixation in topography of the left inferior parathyroid and the patient was subjected to a third and more extensive surgery, with removal of lymph nodes and adjacent thyroid tissue. Serum calcium and PTH remained elevated, requiring loop diuretics and intravenous bisphosphonates to control hypercalcemia. Cervical radiotherapy was implemented as adjuvant therapy. After two months the patient complained of dyspnea, and a CT scan of the chest demonstrated areas of parenchymal condensation, suggestive of actinic pneumonitis. At the 2-year follow-up no major issues were evident. PMID:23268928

Dytz, Márcio Garrison; Souza, Rodrigo Gomes; Lázaro, Ana Paula Pires; Gonçalves, Manuel Domingos da Cruz; Vidal, Ana Paula Aguiar; dos Santos Teixeira, Patrícia de Fátima; Fleiuss Farias, Maria Lucia

2013-01-01

154

A Pseudo MS3 Approach for Identification of Disulfide-Bonded Proteins: Uncommon Product Ions and Database Search  

NASA Astrophysics Data System (ADS)

It has previously been reported that disulfide and backbone bonds of native intact proteins can be concurrently cleaved using electrospray ionization (ESI) and collision-induced dissociation (CID) tandem mass spectrometry (MS/MS). However, the cleavages of disulfide bonds result in different cysteine modifications in product ions, making it difficult to identify the disulfide-bonded proteins via database search. To solve this identification problem, we have developed a pseudo MS3 approach by combining nozzle-skimmer dissociation (NSD) and CID on a quadrupole time-of-flight (Q-TOF) mass spectrometer using chicken lysozyme as a model. Although many of the product ions were similar to those typically seen in MS/MS spectra of enzymatically derived peptides, additional uncommon product ions were detected including ci-1 ions (the ith residue being aspartic acid, arginine, lysine and dehydroalanine) as well as those from a scrambled sequence. The formation of these uncommon types of product ions, likely caused by the lack of mobile protons, were proposed to involve bond rearrangements via a six-membered ring transition state and/or salt bridge(s). A search of 20 pseudo MS3 spectra against the Gallus gallus (chicken) database using Batch-Tag, a program originally designed for bottom up MS/MS analysis, identified chicken lysozyme as the only hit with the expectation values less than 0.02 for 12 of the spectra. The pseudo MS3 approach may help to identify disulfide-bonded proteins and determine the associated post-translational modifications (PTMs); the confidence in the identification may be improved by incorporating the fragmentation characteristics into currently available search programs.

Chen, Jianzhong; Shiyanov, Pavel; Schlager, John J.; Green, Kari B.

2012-02-01

155

Effectiveness of Gefitinib against Non–Small-Cell Lung Cancer with the Uncommon EGFR Mutations G719X and L861Q  

PubMed Central

Introduction: In non–small-cell lung cancer, an exon 19 deletion and an L858R point mutation in the epidermal growth factor receptor (EGFR) are predictors of a response to EGFR-tyrosine kinase inhibitors. However, it is uncertain whether other uncommon EGFR mutations are associated with sensitivity to EGFR-tyrosine kinase inhibitors. Methods: A post-hoc analysis to assess prognostic factors was performed with the use of patients with EGFR mutations (exon 19 deletion, L858R, G719X, and L861Q) who were treated with gefitinib in the NEJ002 study, which compared gefitinib with carboplatin-paclitaxel as the first-line therapy. Results: In the NEJ002 study, 225 patients with EGFR mutations received gefitinib at any treatment line. The Cox proportional hazards model indicated that performance status, response to chemotherapy, response to gefitinib, and mutation types were significant prognostic factors. Overall survival (OS) was significantly shorter among patients with uncommon EGFR mutations (G719X or L861Q) compared with OS of those with common EGFR mutations (12 versus 28.4 months; p = 0.002). In the gefitinib group (n = 114), patients with uncommon EGFR mutations had a significantly shorter OS (11.9 versus 29.3 months; p < 0.001). By contrast, OS was similar between patients with uncommon mutations and those with common mutations in the carboplatin-paclitaxel group (n = 111; 22.8 versus 28 months; p = 0.358). Conclusions: The post-hoc analyses clearly demonstrated shorter survival for gefitinib-treated patients with uncommon EGFR mutations compared with the survival of those with common mutations and suggest that the first-line chemotherapy may be relatively effective for non–small-cell lung cancer with uncommon EGFR mutations. PMID:24419415

Watanabe, Satoshi; Minegishi, Yuji; Maemondo, Makoto; Inoue, Akira; Sugawara, Shunichi; Isobe, Hiroshi; Harada, Masao; Ishii, Yoshiki; Gemma, Akihiko; Hagiwara, Koichi; Kobayashi, Kunihiko

2014-01-01

156

Imaging findings of hematologic diseases affecting the breast.  

PubMed

The breast is an uncommon manifestation site for malignant hematological diseases. Intramammary relapse can occur as an isolated tumor or as an extramedullary manifestation in systemic disease. Most published data regarding breast involvement in lymphoma or leukemia focus on the histopathologic characteristics of breast lymphoma or leukemia and do not describe radiological findings. Therefore, the purpose of this review is to provide statistical, clinical, and radiological information on hematological malignancies of the breast. PMID:24332206

Surov, Alexey

2013-12-01

157

Rosai-Dorfman disease of the paranasal sinuses and orbit.  

PubMed

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination. PMID:21727770

Khan, Afaq Ahmad; Siraj, Fouzia; Rai, Devinder; Aggarwal, Shyam

2011-01-01

158

Three Cases of Lichen Nitidus Associated with Various Cutaneous Diseases  

PubMed Central

Lichen nitidus (LN) is an uncommon, usually asymptomatic cutaneous eruption characterized by the presence of multiple, small, flesh-colored papules. The epidemiologic and pathophysiologic characteristics of LN have not yet been defined. Furthermore, LN has rarely been described in association with other cutaneous diseases. We herein report 3 cases of LN associated with various cutaneous diseases, including lichen striatus, oral lichen planus, and psoriasis vulgaris. PMID:25143682

Cho, Eun Byul; Kim, Heung Yeol; Park, Eun Joo; Kwon, In Ho; Kim, Kwang Joong

2014-01-01

159

Hypercalciuria and nephrocalcinosis, a feature of Wilson's disease.  

PubMed

Hypercalciuria and nephrocalcinosis are not uncommon in patients with Wilson's disease but have only once been reported as the presenting sign. We diagnosed Wilson's disease in a 17-year-old male patient 6 years after his first episode of gross hematuria and 2 years after detection of hypercalciuria and nephrocalcinosis. Therapy with penicillamine resulted only in a moderate reduction of urinary calcium excretion but oxalate excretion increased. PMID:8290000

Hoppe, B; Neuhaus, T; Superti-Furga, A; Forster, I; Leumann, E

1993-01-01

160

Rapidly progressive congenital rhabdomyosarcoma presenting with multiple cutaneous lesions: an uncommon diagnosis and a therapeutic challenge.  

PubMed

Congenital rhabdomyosarcomas (RMSs) are rare tumors with variable clinical presentations. A 2 month-old, term male neonate (37 weeks, 4 days), weighing 3.2kg, born to a 24 year-old primigravida, by simple vaginal delivery presented with multiple erythematous papulonodular lesions over his trunk that progressed to his whole body, on the first day of delivery. Prior to conception, his mother was treated for polycystic ovarian disease. On the tenth day, his chest computed tomogram scans revealed multiple, heterogeneously enhancing, bilateral pleural-based soft tissue density nodular lesions, along with multiple soft tissue density lesions, involving skeletal muscles of all his body parts. Microsections from two biopsies (on 10th day and after 2 months) revealed a malignant round cell tumor with cells arranged in a diffuse, solid pattern, comprising embryonal and solid alveolar components. Immunohistochemically, the tumor cells were diffusely positive for desmin, myoD1 and myogenin. Diagnosis of embryonal and alveolar (mixed type) RMS was offered. Further molecular cytogenetic analysis was negative for PAX3-FKHR and PAX7-FKHR. The patient was induced on chemotherapy as per intergroup rhabdomyosarcoma study IV protocol. There was treatment response with near total remission after 8 weeks of treatment. Thereafter, new lesions started appearing that also disappeared after modification of the chemotherapy drugs. However, after 16 months, the baby died of brain metastasis. The present case forms the fourth case report of an aggressive form of a congenital RMS with extensive cutaneous involvement and brain metastasis. A review of previously diagnosed cases of congenital RMSs is discussed herewith. PMID:24635971

Rekhi, Bharat; Qureshi, Sajid Shafique; Narula, Gaurav; Gujral, Sumeet; Kurkure, Purna

2014-05-01

161

Sexually transmitted diseases in children in India.  

PubMed

Sexually transmitted diseases (STDs) in children are not uncommon in India, though systematic epidemiological studies to determine the exact prevalence are not available. STDs in children can be acquired via sexual route or, uncommonly, via non-sexual route such as accidental inoculation by a diseased individual. Neonatal infections are almost always acquired intrauterine or during delivery. Voluntary indulgence in sexual activity is also an important factor in acquisition of STDs in childhood. Sexual abuse and sex trafficking remain the important problems in India. Surveys indicate that nearly half of the children are sexually abused. Most at risk children are street-based, homeless or those living in or near brothels. Last two decades have shown an increase in the prevalence of STDs in children, though most of the data is from northern part of the country and from major hospitals. However, due to better availability of antenatal care to majority of women, cases of congenital syphilis have declined consistently over the past two-three decades. Other bacterial STDs are also on decline. On the other hand, viral STDs such as genital herpes and anogenital warts are increasing. This reflects trends of STDs in the adult population. Concomitant HIV infection is uncommon in children. Comprehensive sex education, stringent laws to prevent sex trafficking and child sexual abuse, and antenatal screening of all the women can reduce the prevalence of STDs in children. PMID:20826987

Dhawan, Jyoti; Gupta, Somesh; Kumar, Bhushan

2010-01-01

162

Reconstructors: Uncommon Scents - Episode 1. Students learn the health effects of inhalant abuse and exposure to hazardous household products such as organic solvents  

NSDL National Science Digital Library

In Uncommon Scents Episode 1, students investigate a chemical accident and learn about the health effects of exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, learn how inhaling these product can severely damage the nervous system and other parts of the body, and find ways to protect themselves from exposure to toxic vapors.

Center for Technology in Teaching and Learning

2011-09-28

163

Reconstructors: Uncommon Scents - Episode 3. Students learn the health effects of solvent exposure on memory and selective attention, inhalant abuse, and refusal strategies  

NSDL National Science Digital Library

In Uncommon Scents Episode 3, students investigate exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, and also learn how inhaling these product can severely damage the nervous system and other parts of the body.

Center for Technology in Teaching and Learning

2011-09-28

164

Reconstructors: Uncommon Scents - Episode 2. Students learn the health effects of solvent exposure on memory and selective attention, inhalant abuse, and refusal strategies  

NSDL National Science Digital Library

In Uncommon Scents Episode 2, students investigate exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, and also learn how inhaling these product can severely damage the nervous system and other parts of the body.

Center for Technology in Teaching and Learning

2011-09-28

165

Crohn's disease of the vulva in a 10-year-old girl.  

PubMed

Crohn's disease may involve all parts of the gastrointestinal tract and may often involve other organs as well. These non-intestinal affections are termed extraintestinal manifestations. Vulval involvement is an uncommon extraintestinal manifestation of Crohn's disease, and it is very rare in children. Patients with vulval CD typically present with erythema and edema of the labia majora, which progresses to extensive ulcer formation. Vulval Crohn's disease can appear before or after intestinal problems or it may occur simultaneously. We present a 10-year-old girl with intestinal Crohn's disease complicated with perianal skin tags and asymptomatic unilateral labial hypertrophy. The course of her lesion was independent of the intestinal disease and responded significantly to medical treatment including azathioprine and topical steroid. We emphasize that although vulval involvement in childhood is uncommon, Crohn's disease must be considered in the differential diagnosis of nontender, red, edematous lesions of the genital area. PMID:18664090

Kulo?lu, Zarife; Kansu, Aydan; Demirçeken, Fulya; Bozkir, Mehtap; Kundakçi, Nihal; Bingöl-Kolo?lu, Meltem; Girgin, Nurten

2008-01-01

166

Salivary gland diseases in children  

PubMed Central

Salivary gland diseases in children are rare, apart from viral-induced diseases. Nevertheless, it is essential for the otolaryngologist to recognize these uncommon findings in children and adolescents and to diagnose and initiate the proper treatment. The present work provides an overview of the entire spectrum of congenital and acquired diseases of the salivary glands in childhood and adolescence. The current literature was reviewed and the results discussed and summarized. Besides congenital diseases of the salivary glands in children, the main etiologies of viral and bacterial infections, autoimmune diseases and tumors of the salivary glands were considered. In addition to the known facts, new developments in diagnostics, imaging and therapy, including sialendoscopy in obstructive diseases and chronic recurrent juvenile sialadenitis were taken into account. In addition, systemic causes of salivary gland swelling and the treatment of sialorrhoea were discussed. Although salivary gland diseases in children are usually included in the pathology of the adult, they differ in their incidence and some­times in their symptoms. Clinical diagnostics and especially the surgical treatment are influenced by a stringent indications and a less invasive strategy. Due to the rarity of tumors of the salivary glands in children, it is recommended to treat them in a specialized center with greater surgical experience. Altogether the knowledge of the differential diagnoses in salivary gland diseases in children is important for otolaryngologists, to indicate the proper therapeutic approach. PMID:25587366

Iro, Heinrich; Zenk, Johannes

2014-01-01

167

Rosai-Dorfman disease with diffuse gastrointestinal involvement.  

PubMed

Rosai-Dorfman disease, formerly known as 'sinus histiocytosis with massive lymphadenopathy', is a rare self-limiting histiocytic proliferative disorder typically presenting early in life with cervical lymphadenopathy and nonspecific systemic symptoms. Although it is usually a nodal disease, extranodal lesions may be encountered in some cases. The gastrointestinal tract is uncommonly affected in Rosai-Dorfman disease and its diagnosis depends on clinical suspicion and careful histopathological examination of biopsy samples taken from involved gastrointestinal segments. Here, we report a case of atypical Rosai-Dorfman disease with systemic symptoms and diffuse gastrointestinal involvement that led to a diagnostic and therapeutic challenge. PMID:23510963

Baran, Bulent; Karaca, Cetin; Soyer, Ozlem M; Yonal, Ipek; Isiklar, Aysun D; Cakmak, Ramazan; Nalcaci, Meliha; Dogan, Oner; Kaymakoglu, Sabahattin

2013-07-01

168

Sea Anemone Peptide with Uncommon ?-Hairpin Structure Inhibits Acid-sensing Ion Channel 3 (ASIC3) and Reveals Analgesic Activity*  

PubMed Central

Three novel peptides were isolated from the venom of the sea anemone Urticina grebelnyi. All of them are 29 amino acid peptides cross-linked by two disulfide bridges, with a primary structure similar to other sea anemone peptides belonging to structural group 9a. The structure of the gene encoding the shared precursor protein of the identified peptides was determined. One peptide, ?-AnmTX Ugr 9a-1 (short name Ugr 9-1), produced a reversible inhibition effect on both the transient and the sustained current of human ASIC3 channels expressed in Xenopus laevis oocytes. It completely blocked the transient component (IC50 10 ± 0.6 ?m) and partially (48 ± 2%) inhibited the amplitude of the sustained component (IC50 1.44 ± 0.19 ?m). Using in vivo tests in mice, Ugr 9-1 significantly reversed inflammatory and acid-induced pain. The other two novel peptides, AnmTX Ugr 9a-2 (Ugr 9-2) and AnmTX Ugr 9a-3 (Ugr 9-3), did not inhibit the ASIC3 current. NMR spectroscopy revealed that Ugr 9-1 has an uncommon spatial structure, stabilized by two S-S bridges, with three classical ?-turns and twisted ?-hairpin without interstrand disulfide bonds. This is a novel peptide spatial structure that we propose to name boundless ?-hairpin. PMID:23801332

Osmakov, Dmitry I.; Kozlov, Sergey A.; Andreev, Yaroslav A.; Koshelev, Sergey G.; Sanamyan, Nadezhda P.; Sanamyan, Karen E.; Dyachenko, Igor A.; Bondarenko, Dmitry A.; Murashev, Arkadii N.; Mineev, Konstantin S.; Arseniev, Alexander S.; Grishin, Eugene V.

2013-01-01

169

Zygomycetes in Human Disease  

PubMed Central

The Zygomycetes represent relatively uncommon isolates in the clinical laboratory, reflecting either environmental contaminants or, less commonly, a clinical disease called zygomycosis. There are two orders of Zygomycetes containing organisms that cause human disease, the Mucorales and the Entomophthorales. The majority of human illness is caused by the Mucorales. While disease is most commonly linked to Rhizopus spp., other organisms are also associated with human infection, including Mucor, Rhizomucor, Absidia, Apophysomyces, Saksenaea, Cunninghamella, Cokeromyces, and Syncephalastrum spp. Although Mortierella spp. do cause disease in animals, there is no longer sufficient evidence to suggest that they are true human pathogens. The spores from these molds are transmitted by inhalation, via a variety of percutaneous routes, or by ingestion of spores. Human zygomycosis caused by the Mucorales generally occurs in immunocompromised hosts as opportunistic infections. Host risk factors include diabetes mellitus, neutropenia, sustained immunosuppressive therapy, chronic prednisone use, iron chelation therapy, broad-spectrum antibiotic use, severe malnutrition, and primary breakdown in the integrity of the cutaneous barrier such as trauma, surgical wounds, needle sticks, or burns. Zygomycosis occurs only rarely in immunocompetent hosts. The disease manifestations reflect the mode of transmission, with rhinocerebral and pulmonary diseases being the most common manifestations. Cutaneous, gastrointestinal, and allergic diseases are also seen. The Mucorales are associated with angioinvasive disease, often leading to thrombosis, infarction of involved tissues, and tissue destruction mediated by a number of fungal proteases, lipases, and mycotoxins. If the diagnosis is not made early, dissemination often occurs. Therapy, if it is to be effective, must be started early and requires combinations of antifungal drugs, surgical intervention, and reversal of the underlying risk factors. The Entomophthorales are closely related to the Mucorales on the basis of sexual growth by production of zygospores and by the production of coenocytic hyphae. Despite these similarities, the Entomophthorales and Mucorales have dramatically different gross morphologies, asexual reproductive characteristics, and disease manifestations. In comparison to the floccose aerial mycelium of the Mucorales, the Entomophthorales produce a compact, glabrous mycelium. The asexually produced spores of the Entomophthorales may be passively released or actively expelled into the environment. Human disease with these organisms occurs predominantly in tropical regions, with transmission occurring by implantation of spores via minor trauma such as insect bites or by inhalation of spores into the sinuses. Conidiobolus typically infects mucocutaneous sites to produce sinusitis disease, while Basidiobolus infections occur as subcutaneous mycosis of the trunk and extremities. The Entomophthorales are true pathogens, infecting primarily immunocompetent hosts. They generally do not invade blood vessels and rarely disseminate. Occasional cases of disseminated and angioinvasive disease have recently been described, primarily in immunocompromised patients, suggesting a possible emerging role for this organism as an opportunist. PMID:10756000

Ribes, Julie A.; Vanover-Sams, Carolyn L.; Baker, Doris J.

2000-01-01

170

An Uncommon Cottage  

ERIC Educational Resources Information Center

This article features the Cliffs Cottage, a "showcase home" at Furman University which demonstrates the use of green technology in residential building and teaches about sustainability. Custom-built for the shelter-magazine dreams of "Southern Living," a sponsor of the home, the house seems better suited for a tony subdivision. The Cliffs Cottage…

Carlson, Scott

2008-01-01

171

The University's Uncommon Community  

ERIC Educational Resources Information Center

In the UK, as elsewhere in the world, the global financial crisis has focused attention on the cost of public services and the need to reduce expenditure, not least in respect of higher education. This, however, raises a set of prior questions: What kind of society do we want? What is important to democratic society? What kind of higher education…

Harris, Suzy

2012-01-01

172

Mitogenomes from Two Uncommon Haplogroups Mark Late Glacial/Postglacial Expansions from the Near East and Neolithic Dispersals within Europe  

PubMed Central

The current human mitochondrial (mtDNA) phylogeny does not equally represent all human populations but is biased in favour of representatives originally from north and central Europe. This especially affects the phylogeny of some uncommon West Eurasian haplogroups, including I and W, whose southern European and Near Eastern components are very poorly represented, suggesting that extensive hidden phylogenetic substructure remains to be uncovered. This study expanded and re-analysed the available datasets of I and W complete mtDNA genomes, reaching a comprehensive 419 mitogenomes, and searched for precise correlations between the ages and geographical distributions of their numerous newly identified subclades with events of human dispersal which contributed to the genetic formation of modern Europeans. Our results showed that haplogroups I (within N1a1b) and W originated in the Near East during the Last Glacial Maximum or pre-warming period (the period of gradual warming between the end of the LGM, ?19 ky ago, and the beginning of the first main warming phase, ?15 ky ago) and, like the much more common haplogroups J and T, may have been involved in Late Glacial expansions starting from the Near East. Thus our data contribute to a better definition of the Late and postglacial re-peopling of Europe, providing further evidence for the scenario that major population expansions started after the Last Glacial Maximum but before Neolithic times, but also evidencing traces of diffusion events in several I and W subclades dating to the European Neolithic and restricted to Europe. PMID:23936216

Olivieri, Anna; Pala, Maria; Gandini, Francesca; Kashani, Baharak Hooshiar; Perego, Ugo A.; Woodward, Scott R.; Grugni, Viola; Battaglia, Vincenza; Semino, Ornella; Achilli, Alessandro; Richards, Martin B.; Torroni, Antonio

2013-01-01

173

Recurrent cutaneous necrotizing vasculitis in a patient with Kikuchi's disease: case report.  

PubMed

Kikuchi's disease or histiocytic necrotizing lymphadenitis without granulocytic infilteration, is a clinico-pathologic entity well described in developed countries. We report a case of this disorder presenting with recurrent cutaneous necrotizing vasculitis, with the dual objective of highlighting this condition in developing countries and presenting an uncommon manifestation of this disorder. PMID:9557453

Belagavi, C S; Kubeyinje, E P

1997-11-01

174

Isolated primary hydatid cyst of small intestinal mesentery: an exceptional location of hydatid disease  

PubMed Central

Hydatid disease is an endemic problem in some areas of the world. Common sites include liver and lungs. We report an unusual case of isolated primary Hydatid cyst of small gut mesentery. Characteristics of this uncommon location, mechanism, diagnostic difficulties, and treatment are discussed. PMID:23308322

Najih, Mohammed; Chabni, Ali; Attoulou, Gilles; Yamoul, Rajae; Yakka, Mbarek; Ehirchiou, Abdelkader; AlKandry, Siffedine

2012-01-01

175

Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases  

Microsoft Academic Search

A group of relatively uncommon but important genetic cardiovascular diseases (GCVDs) are associated with increased risk for sudden cardiac death during exercise, including hypertrophic cardiomyopathy, long-QT syndrome, Marfan syndrome, and arrhythmogenic right ventricular cardiomyopathy. These conditions, characterized by diverse phenotypic expression and genetic substrates, account for a substantial proportion of unexpected and usually arrhythmia-based fatal events during adolescence and young

Barry J. Maron; Bernard R. Chaitman; Michael J. Ackerman; Antonio Bayés de Luna; Domenico Corrado; Jane E. Crosson; Barbara J. Deal; David J. Driscoll; N. A. Mark Estes III; Claudio Gil; S. Araújo; David H. Liang; Matthew J. Mitten; Robert J. Myerburg; Antonio Pelliccia; Paul D. Thompson; Jeffrey A. Towbin; Steven P. Van Camp

176

Campylobacter jejuni, an uncommon cause of splenic abscess diagnosed by 16S rRNA gene sequencing.  

PubMed

Splenic abscess is a rare disease that primarily occurs in patients with splenic trauma, endocarditis, sickle cell anemia, or other diseases that compromise the immune system. This report describes a culture-negative splenic abscess in an immunocompetent patient caused by Campylobacter jejuni, as determined by 16S rRNA gene sequencing. PMID:25447730

Seng, Piseth; Quenard, Fanny; Menard, Amélie; Heyries, Laurent; Stein, Andreas

2014-12-01

177

Activity of Linezolid against 3,251 Strains of Uncommonly Isolated Gram-Positive Organisms: Report from the SENTRY Antimicrobial Surveillance Program?  

PubMed Central

Linezolid was tested against 32 species of uncommonly isolated gram-positive organisms (3,251 strains) by reference MIC methods and found to be highly active (MIC50 range, 0.25 to 2 ?g/ml; MIC90 range, 0.25 to 2 ?g/ml). Only one isolate (viridans group streptococcus; 0.03% of tested strains) was resistant to linezolid. PMID:17210770

Jones, Ronald N.; Stilwell, Matthew G.; Hogan, Patricia A.; Sheehan, Daniel J.

2007-01-01

178

Activity of Linezolid against 3,251 Strains of Uncommonly Isolated Gram-Positive Organisms: Report from the SENTRY Antimicrobial Surveillance Program  

Microsoft Academic Search

Linezolid was tested against 32 species of uncommonly isolated gram-positive organisms (3,251 strains) by reference MIC methods and found to be highly active (MIC50 range, 0.25 to 2 g\\/ml; MIC90 range, 0.25 to 2 g\\/ml). Only one isolate (viridans group streptococcus; 0.03% of tested strains) was resistant to linezolid.

Ronald N. Jones; Matthew G. Stilwell; Patricia A. Hogan; Daniel J. Sheehan

2007-01-01

179

Recurrent eosinophilic cystitis in a child with chronic granulomatous disease.  

PubMed

Eosinophilic cystitis is an uncommon disease in children, and its association with chronic granulomatous disease (CGD) has been previously reported in only five patients. In all those patients the disease showed either a self-limited benign course or a rapid response to corticosteroid treatment. The authors describe a child with X-linked CGD who developed eosinophilic cystitis with a recurrent course and difficult therapeutic management. The authors also discuss the pathogenesis of granuloma formation in CGD and review the literature for current therapies for these complications. PMID:15125617

Barese, Cecilia N; Podestá, Miguel; Litvak, Edith; Villa, Mariana; Rivas, Eva María

2004-03-01

180

Localized Castleman disease of plasma cell type in the abdomen.  

PubMed

Castleman disease is a relatively rare entity, with the hyaline-vascular type the predominant form. Although the plasma cell type is uncommon, it still comprises approximately 10% of cases of localized diseases. In addition, the abdomen is a rare site for involvement and localized Castleman disease of the plasma cell type in the abdomen is rare. The radiologic features of localized plasma cell type in the abdomen are mostly limited to case reports. In addition to the conventional imaging findings, we present some new imaging findings of localized plasma cell type in the abdomen. PMID:22040445

Lu, Zhi-hua; Wu, Mei

2011-09-01

181

Rosai-Dorfman disease with isolated laryngeal involvement.  

PubMed

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced in reaching a correct pathologic diagnosis and in deciding on an appropriate treatment regimen. Based on our experience, we believe that Rosai-Dorfman disease should be considered as a differential diagnosis in patients who present with a recurrent inflammatory (histiocytic) mass lesion of the larynx. PMID:23076853

Illing, Elisa A; Halum, Stacey L

2012-10-01

182

Life-threatening retro-pharyngeal bleed in a case of systemic lupus erythematosus – an uncommon but important evil of a common disease  

PubMed Central

Acquired inhibitors of coagulation are antibodies that interfere with the coagulation system either by functional inhibition of the activity of clotting factors or by their increased clearance from the plasma. They can be idiopathic or seen in various physiologic (pregnancy and postpartum) or pathologic states (systemic lupus erythematosus (SLE), malignancies, rheumatoid arthritis) and can result in a variety of haemorrhagic sequelae. SLE can be associated with antibodies to multiple clotting factors and bleeding though rare can be an important haematologic manifestation with life-threatening consequences in these patients. PMID:22675012

Palla, Amruth; Rogers, John; Singh, Sindhu

2011-01-01

183

Epidemiology and clinical management of Legionnaires' disease.  

PubMed

Legionnaires' disease is an important cause of community-acquired and hospital-acquired pneumonia. Although uncommon, Legionnaires' disease continues to cause disease outbreaks of public health significance. The disease is caused by any species of the Gram-negative aerobic bacteria belonging to the genus Legionella; Legionella pneumophila serogroup 1 is the causative agent of most cases in Europe. In this Review we outline the global epidemiology of Legionnaires' disease, summarise its diagnosis and management, and identify research gaps and priorities. Early clinical diagnosis and prompt initiation of appropriate antibiotics for Legionella spp in all patients with community-acquired or hospital-acquired pneumonias is a crucial measure for management of the disease. Progress in typing and sequencing technologies might additionally contribute to understanding the distribution and natural history of Legionnaires' disease, and inform outbreak investigations. Control of Legionnaires' disease outbreaks relies on rapid ascertainment of descriptive epidemiological data, combined with microbiological information to identify the source and implement control measures. Further research is required to define the actual burden of disease, factors that influence susceptibility, key sources of infection, and differences in virulence between strains of Legionella species. Other requirements are improved, specific, sensitive, and rapid diagnostic tests to accurately inform management of Legionnaires' disease, and controlled clinical trials to ascertain the optimum antibiotics for treatment. PMID:24970283

Phin, Nick; Parry-Ford, Frances; Harrison, Timothy; Stagg, Helen R; Zhang, Natalie; Kumar, Kartik; Lortholary, Olivier; Zumla, Alimuddin; Abubakar, Ibrahim

2014-10-01

184

Vegetarian Dietary Patterns as a Means to Achieve Reduction in Cardiovascular Disease and Diabetes Risk Factors  

Microsoft Academic Search

Cardiovascular disease and type 2 diabetes are uncommon in people consuming vegetarian and vegan diets. Vegetarian and vegan\\u000a dietary patterns tend to result in lower body weight and better nutritional profiles than conventional healthy eating patterns\\u000a and have been shown to be an effective tool for management of cardiovascular disease and diabetes risk. The consistency of\\u000a observed beneficial outcomes with

Amy Joy Lanou; Barbara Svenson

2010-01-01

185

Association of Dermal Melanocytosis With Lysosomal Storage Disease Clinical Features and Hypotheses Regarding Pathogenesis  

Microsoft Academic Search

Background: The potential association of dermal me- lanocytosis with lysosomal storage disease in infancy is an uncommonly known and poorly understood entity. Observations:Wedescribe2infantswithextensiveder- mal melanocytosis in association with GM1gangliosido- sis type 1 and Hurler syndrome, respectively. A litera- ture analysis revealed 37 additional cases. Clinically, dermal melanocytosis associated with lysosomal stor- age disease is characterized by extensive, blue cutane- ous

Miriam Hanson; James R. Lupski; John Hicks; Denise Metry; ERMAL MELANOCYTOSIS

2003-01-01

186

Rosai-Dorfman disease with massive intracranial involvement: asymmetric response to conservative therapy.  

PubMed

Rosai-Dorfman Disease (RDD) is a rare, idiopathic lymphoproliferative disorder. Central nervous system (CNS) involvement in this disorder is an uncommon manifestation. The clinical and radiographic appearance of CNS RDD is variable, and may mimic more common diseases. Treatment is controversial, and spontaneous remission is common. Positive outcomes have been reported with radiation therapy, or corticosteroid administration, or surgical excision. Our case is unusual in that the extracranial sites of involvement responded to corticosteroid therapy while the intracranial masses progressed. PMID:19659569

Walker, Robert N; Nickles, Thomas P; Lountzis, Nektarios I; Jacobs, Darren L; Nawaz, Nava K

2011-04-01

187

Paget's Disease of the Vulva in Premenopausal Woman Treated with Only Surgery: A Case Report  

PubMed Central

Paget's disease of the vulva remains a rare condition with only a limited number of cases reported in the literature. It is an uncommon neoplasm usually of postmenopausal white women characterized by controversies in its prevalence, clinical features, treatment strategies, and prognostic. We here report a case of a primary Paget's disease of the vulva in premenopausal woman treated by only surgery with a favorable issue. PMID:23024871

Asmouki, Hamid; Oumouloud, Rachid; Aboulfalah, Abderrahim; Soummani, Abderraouf; Marrat, Abdelouahed

2012-01-01

188

Imatinib Added to Chemotherapy Keeps Disease in Check for Longer in Children with Rare Form of Acute Lymphoblastic Leukemia  

Cancer.gov

The addition of the targeted drug imatinib to chemotherapy for children with an uncommon form of acute lymphoblastic leukemia (ALL) dramatically improved the length of time the disease remained in remission, according to the November 1, 2009, Journal of Clinical Oncology.

189

Myeloproliferative disease in a cat  

SciTech Connect

Myeloproliferative disorders, a complex of cytologic abnormalities arising in the bone marrow, are among domestic animals most frequently recognized in cats but are relatively uncommon. A 4-year-old female Siamese, with splenomegaly and weight loss, was listless, anorectic, pale and dehydrated. A hemogram showed severe, macrocytic normochromic anemia, leukocytosis and reticulocytosis, with abnormally high numbers of nucleated RBC and undifferentiated blast cells. Bone marrow smears contained predominantly undifferentiated blast cells, RBC precursors and myeloblasts. The fluorescent antibody test for FeLV was positive. The cat died 66 days later despite a blood transfusion and chemotherapy. Necropsy confirmed a diagnosis of myeloproliferative disease, with hepatic and splenic invasion. 15 references, 5 figures, 1 table.

Yates, R.W.; Weller, R.E.; Feldman, B.F.

1984-10-01

190

Plastic bronchitis: an old disease revisited.  

PubMed

Expectoration of bronchial casts (plastic bronchitis) is an uncommon but ancient problem. Herein we describe a 40-year-old man, with no prior lung disease, who had dyspnea, cough, and expectoration of long branching bronchial casts. No specific cause was delineated, although special stains for eosinophilic granule major basic protein demonstrated occasional foci of eosinophils and small amounts of extracellular major basic protein in the bronchial casts. Various diseases, such as allergic bronchopulmonary aspergillosis, bronchiectasis, and cystic fibrosis, have been associated with the formation of bronchial casts and should be considered in the differential diagnosis. Although most previously reported cases have been associated with some type of pulmonary disease, our patient had no evidence of an underlying pulmonary disorder. PMID:2002688

Jett, J R; Tazelaar, H D; Keim, L W; Ingrassia, T S

1991-03-01

191

Hirschsprung Disease  

MedlinePLUS

... GI) Inflammatory Bowel Disease Irritable Bowel Syndrome (IBS) Digestive System Irritable Bowel Syndrome Inflammatory Bowel Disease Your Digestive System Irritable Bowel Syndrome Constipation Inflammatory Bowel Disease Digestive ...

192

Biliary cystic disease.  

PubMed

Biliary cystic disease, though uncommon, can present at a wide range of ages with a wide range of symptoms. Choledochal cysts are associated with the development of both cholangiocarcinoma and gallbladder cancer. Thus, most biliary cystic disease is best managed operatively. Many factors should be considered when performing surgery on patients with choledochal cysts, including age, presenting symptoms, cyst type, associated biliary stones, prior biliary surgery, intrahepatic strictures, hepatic atrophy/hypertrophy, biliary cirrhosis, portal hypertension, and associated biliary malignancy. When feasible, surgical treatment should consist of cholecystectomy and complete surgical excision of extrahepatic cysts with Roux-en-Y reconstruction. Because the risk of recurrent cholangitis is significant and additional symptoms and problems are common, the use of long-term soft Silastic biliary stents (Dow Corning Corp., Midland, MI) should be considered when complex intrahepatic and extrahepatic cystic disease is present. Alternatively, the Roux-en-Y jejunal limb can be marked at the fascia for future percutaneous access. Reconstruction via hepaticoduodenostomy and jejunal interposition has been associated with increased postoperative pain due to bile reflux gastritis. Thus, hepaticojejunostomy reconstruction is recommended. For choledochal cysts involving the distal bile duct, the bile duct should be excised at the intrapancreatic portion. Resection of the pancreatic head should be reserved for patients with established malignancies. Surgical excision of the intrahepatic portion of the bile duct should be individualized to include preservation of hepatic parenchyma when the liver is not cirrhotic. If cirrhosis is advanced, hepatic transplantation may be indicated, but this is rare. Oncologic principles should be followed in the presence of a malignancy. Lifelong follow-up is required because of the possibility of a "field" defect increasing susceptibility to cancer throughout the biliary tract epithelium. PMID:16539871

Lipsett, Pamela A; Locke, Jayme E

2006-04-01

193

Rosai-Dorfman disease of the pericardium: a case report and review of literature  

PubMed Central

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disease of unknown etiology. It typically presents as massive lymphadenopathy with a predilection for the cervical lymph nodes of children and young adults. However, extranodal involvement is not uncommon and may cause confusion with other neoplasms or reactive disease. We describe here a unique case of extranodal RDD manifesting as a pericardial mass in a 69-year-old man. The lesion was detected by computed tomography during a periodic examination of the chest. Subsequently positron emission tomography scan showed mild increase of flurodeoxyglucose uptake. Clinically, it was supposed to be a mesothelioma. Histological examination showed the typical features of RDD confirmed by the staining of S100 protein, which highlighted the emperipolesis of the characteristic histiocytes. To the best of our knowledge, pericardial RDD represents an extremely rare condition and should be included in the differential diagnosis of pericardial neoplasms. PMID:25031768

Lao, I-Weng; Dong, Yun; Wang, Jian

2014-01-01

194

Rosai-Dorfman disease of the pericardium: a case report and review of literature.  

PubMed

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disease of unknown etiology. It typically presents as massive lymphadenopathy with a predilection for the cervical lymph nodes of children and young adults. However, extranodal involvement is not uncommon and may cause confusion with other neoplasms or reactive disease. We describe here a unique case of extranodal RDD manifesting as a pericardial mass in a 69-year-old man. The lesion was detected by computed tomography during a periodic examination of the chest. Subsequently positron emission tomography scan showed mild increase of flurodeoxyglucose uptake. Clinically, it was supposed to be a mesothelioma. Histological examination showed the typical features of RDD confirmed by the staining of S100 protein, which highlighted the emperipolesis of the characteristic histiocytes. To the best of our knowledge, pericardial RDD represents an extremely rare condition and should be included in the differential diagnosis of pericardial neoplasms. PMID:25031768

Lao, I-Weng; Dong, Yun; Wang, Jian

2014-01-01

195

Tumour Calcification and Calciphylaxis in End-Stage Renal Disease  

PubMed Central

Although soft tissue and vascular calcifications are common in CKD and progress as an independent risk factor of all-cause mortality, tumour calcification and calciphylaxis are uncommon in patients with end-stage renal disease (ESRD). Here, we discuss a rare case of a patient with tumour calcification complicated with calciphylaxis developed septic shock from infection. Our patient is a 57-year-old man in his late stage of renal disease who presented with a huge mass at the right hip and necrotic cutaneous ulcers on the lower legs followed by local and systemic infection and death due to septic shock. PMID:25477966

Di, Jia; Jiang, Zhenxing; Yang, Min

2014-01-01

196

Primary Testicular Carcinoid Tumor presenting as Carcinoid Heart Disease  

PubMed Central

Primary carcinoid tumors of the testis are very rare, and they seldom present with carcinoid syndrome. We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome. He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease. His urinary 5-hydroxy indole acetic acid level was elevated. He underwent orchidectomy and the histopathology confirmed a testicular carcinoid tumor.

Chikkaraddi, Manjunath L.; Krishna, Sudeep; Shetty, Manjunath

2015-01-01

197

Behcet's Disease  

MedlinePLUS

NINDS Behcet's Disease Information Page Table of Contents (click to jump to sections) What is Behcet's Disease? Is there ... Trials Organizations Additional resources from MedlinePlus What is Behcet's Disease? Behcet's disease is a rare, chronic inflammatory ...

198

Graves' Disease  

MedlinePLUS

... information Autoimmune diseases fact sheet Diabetes fact sheet Hashimoto's disease fact sheet Illnesses and disabilities Lupus fact sheet ... of overactive thyroid. It is closely related to Hashimoto's disease, another autoimmune disease affecting the thyroid. Return to ...

199

Moyamoya Disease  

MedlinePLUS

NINDS Moyamoya Disease Information Page Table of Contents (click to jump to sections) What is Moyamoya Disease? Is there ... is being done? Clinical Trials Organizations What is Moyamoya Disease? Moyamoya disease is a rare, progressive cerebrovascular ...

200

Zoonotic diseases of common pet birds: psittacine, passerine, and columbiform species.  

PubMed

Zoonotic transmission of disease from pet birds is uncommon, but there are some recognized dangers. Most notably, Chlamydophila psittaci can be transmitted from pet birds to humans. Allergic responses to pet birds, including pneumonitis and contact dermatitis, have also been documented. Bite wounds from pet birds are rarely reported but can cause trauma and develop infection. The other diseases discussed here are considered potential zoonotic diseases of pet birds because of either isolated reports of suspected but unconfirmed transmission to humans or from reports of wild conspecifics being reported to have the disease. PMID:21872782

Evans, Erika E

2011-09-01

201

Cutaneous manifestations of gastrointestinal disease: part I.  

PubMed

Cutaneous findings are not uncommonly a concomitant finding in patients afflicted with gastrointestinal (GI) diseases. The dermatologic manifestations may precede clinically evident GI disease. Part I of this 2-part CME review focuses on dermatologic findings as they relate to hereditary and nonhereditary polyposis disorders and paraneoplastic disorders. A number of hereditary GI disorders have an increased risk of colorectal carcinomas. These disorders include familial adenomatous polyposis, Peutz-Jeghers syndrome, and juvenile polyposis syndrome. Each disease has its own cutaneous signature that aids dermatologists in the early diagnosis and detection of hereditary GI malignancy. These disease processes are associated with particular gene mutations that can be used in screening and to guide additional genetic counseling. In addition, there is a group of hamartomatous syndromes, some of which are associated with phosphatase and tensin homolog (PTEN) gene mutations, which present with concurrent skin findings. These include Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, and Cronkhite-Canada syndrome. Finally, paraneoplastic disorders are another subcategory of GI diseases associated with cutaneous manifestations, including malignant acanthosis nigricans, Leser-Trélat sign, tylosis, Plummer-Vinson syndrome, necrolytic migratory erythema, perianal extramammary Paget disease, carcinoid syndrome, paraneoplastic dermatomyositis, and paraneoplastic pemphigus. Each of these disease processes have been shown to be associated with an increased risk of GI malignancy. This underscores the important role of dermatologists in the diagnosis, detection, monitoring, and treatment of these disorders while consulting and interacting with their GI colleagues. PMID:23317980

Shah, Kejal R; Boland, C Richard; Patel, Mahir; Thrash, Breck; Menter, Alan

2013-02-01

202

Commentary: is Alzheimer's disease uniquely human?  

PubMed

That Alzheimer's disease (AD) might be a human-specific disease was hypothesized by Rapoport in 1989. Apes and humans share an identical amyloid beta (A?) peptide amino acid sequence and accumulate considerable A? deposits after age 40 years, an age when amyloid plaques are uncommon in humans. Despite their early A? buildup, ape brains have not shown evidence dystrophic neurites near plaques. Aging great ape brains also have few neurofibrillary tangles, with one exception of 1 obese chimpanzee euthanized after a stroke who displayed abundant neurofibrillary tangles, but without the typical AD distribution. We discuss the need for more exacting evaluation of neuron density with age, and note husbandry issues that may allow great apes to live to greater ages. We remain reserved about expectations for fully developed AD-like pathology in the great apes of advanced ages and cautiously support Rapoport's hypothesis. PMID:25533426

Finch, Caleb E; Austad, Steven N

2015-02-01

203

Alzheimer's Disease  

MedlinePLUS

... version of this page please turn Javascript on. Alzheimer's Disease What Is Alzheimer's Disease? Alzheimer’s disease is a brain disease that slowly ... it has no cure. A Common Cause of Dementia Alzheimer’s disease is the most common cause of ...

204

Controlling distant metastasis and surgical treatment are crucial for improving clinical outcome in uncommon head and neck malignancies, such as non-squamous cell carcinoma  

PubMed Central

The objective of this study was to elucidate the clinical characteristics of uncommon head and neck malignancies, such as non-squamous cell carcinoma (SCC), in order to improve patient outcomes. A total of 463 head and neck malignancies were retrospectively analyzed, with 43 cases (9.3%) diagnosed as non-SCC. The overall survival rate of patients with adenoid cystic carcinoma was significantly worse compared to that of patients with SCC. The 5-year survival rates were <50% for patients with malignant melanoma, adenocarcinoma, small-cell carcinoma and sarcomas. Distant metastasis to the lung was frequently observed in cases with a poor outcome. Non-SCC malignancies treated without surgery were associated with a worse outcome. Some non-SCC patients had a poor prognosis and distant metastasis was associated with an unsatisfactory outcome. Timely treatment and control of distant metastasis are essential and surgical treatment should be prioritized in non-SCC cases to improve patient outcomes. PMID:24940505

SHIIBA, MASASHI; UNOZAWA, MOTOHARU; HIGO, MORIHIRO; KOUZU, YUKINAO; KASAMATSU, ATSUSHI; SAKAMOTO, YOSUKE; OGAWARA, KATSUNORI; UZAWA, KATSUHIRO; TAKIGUCHI, YUICHI; TANZAWA, HIDEKI

2014-01-01

205

A 1:1 pharmaceutical cocrystal of myricetin in combination with uncommon piracetam conformer: X-ray single crystal analysis and mechanochemical synthesis  

NASA Astrophysics Data System (ADS)

Combination of two Active Pharmaceutical Ingredients, myricetin and piracetam, yields a 1:1 cocrystal characterized by X-ray single-crystal and powder diffraction, Raman spectroscopy, 1H NMR, thermal analysis (DSC and TG-DTA) methods. Constituents of the cocrystalline phase were also investigated in terms of Hirshfeld surfaces. Compounds in their neutral forms cocrystallize in the Pna21 space group of orthorhombic system. Notably, piracetam adopts an uncommon conformation, not encountered in its cocrystals previously described. In the crystal lattice, a three-dimensional hydrogen-bonded network is observed, including formation of a 2D molecular scaffolding motif. A scale-up procedure is readily available with use of solvent-drop grinding method, in which application of a variety of common solvents leads to formation of the cocrystal, as confirmed by XRPD and Raman spectroscopy.

Sowa, Micha?; ?lepokura, Katarzyna; Matczak-Jon, Ewa

2014-01-01

206

Transitional cell carcinoma of the kidney graft: an extremely uncommon presentation of tumor in renal transplant recipients.  

PubMed

Purpose. Transitional cell carcinoma (TCC) affecting the graft after renal transplantation is a very infrequent way of presentation of this tumor. Our aim is to present our single institution experience with 2 cases, as well as to perform a review of the literature about this tumor after the transplant. Materials and Methods. TCC of the graft developed in 2 of 1365 patients from 1977 to 2010, both cases in women. Data were analyzed for incidence, clinical presentation, treatment, and outcomes. Results. Both cases occurred in 2 mid-age women and resulted to be high grade and locally advanced TCCs, representing an incidence of 0,14% (2/1365). Clinical presentation was urinary obstruction for the first case and incidental ultrasound finding for the second. Preoperative staging was made with CT, cytology, pyelography, ureterorenoscopy, and biopsy. Treatment performed was nephroureterectomy of the graft with bladder cuff and regional lymphadenectomy. Pathological examination showed in both cases a locally advanced and high grade urothelial carcinoma of the pelvis allograft. After 24 and 14 months of followup, both patients are disease free. Conclusions. TCC of the kidney graft is an infrequent tumor that has only been reported in a few cases in the literature. It usually appears at a lower age, more often locally advanced, and with poor differentiation. A multidisciplinary approach to treatment should be required in these cases. PMID:23781380

Hevia, Vital; Gómez, Victoria; Alvarez, Sara; Díez Nicolás, Víctor; Gómez Del Cańizo, Carmen; Orosa, Andrea; Galeano Álvarez, Cristina; Burgos Revilla, F J

2013-01-01

207

Unilateral milia-type intradermal tophi associated with underlying urate subcutaneous deposition: an uncommon cutaneous presentation of gout.  

PubMed

Tophi develop during the most advanced clinical stage of gout, and are usually located on or around the joints. However, unusual skin features caused by intradermal and/or subcutaneous deposition of tophaceous material at locations other than articular regions have been reported. We present the case of a patient with a condition that has been recently termed 'miliarial gout'. which is only the second such case, to our knowledge. A 51-year-old woman, who had a chronic joint disease that had been diagnosed and treated as psoriatic arthritis, presented with multiple asymptomatic, yellowish-white, firm papules (1-3 mm in size) on erythematous areas on the outside of her left leg. On histological examination of a skin biopsy, uric acid crystals were seen in the dermis and subcutis. The patient also had a raised level of serum urate, consistent with a diagnosis of gout. Treatment with allopurinol led to rapid improvement. Intake of corticosteroids and diuretics was a possible triggering factor for the development of cutaneous tophi in this patient. PMID:23837935

Aguayo, R S; Baradad, M; Soria, X; Abal, L; Sanmartín, V; Egido, R; Gallel, P; Casanova, J M; Martí, R M

2013-08-01

208

Parkinson's Disease  

MedlinePLUS

... of movements, and gait problems. DBS requires careful programming of the stimulator device in order to work ... Depression in Parkinson's Disease An NIH disease specific web site to facilitate research on Parkinson's Disease. NINDS, ...

209

Lyme Disease  

MedlinePLUS

... JavaScript on. Read more information on enabling JavaScript. Lyme Disease Top Banner Content Area Skip Content Marketing Share this: Main Content Area Understanding Lyme Disease A History of Lyme Disease, Symptoms, Diagnosis, Treatment ...

210

The Brazilian founder mutation TP53 p.R337H is uncommon in Portuguese women diagnosed with breast cancer.  

PubMed

Since the first studies reporting the TP53 p.R337H mutation as founder mutation in Southern and Southeastern Brazil, there has been controversy on its origin. Preliminary analysis of a small subset of Brazilian mutation carriers revealed that the haplotype incided on a Caucasian background. The vast majority of carriers identified today reside in Brazil or, if identified in other countries, are Brazilian immigrants. To our knowledge, the only two exceptions of carriers without a recognizable link with Brazil are two European families, from Portugal and Germany. Haplotype analysis in the Portuguese family revealed the same haplotype identified in Brazilian individuals, but in the German family, a distinct haplotype was found. Knowing that a significant proportion of women with breast cancer (BC) in Southern Brazil are p.R337H carriers, we analyzed p.R337H in a Portuguese cohort of women diagnosed with this disease. Median age at diagnosis among the first 573 patients tested was 60 years and 100 (17.4%) patients had been diagnosed at or under the age of 45 years. Mutation screening failed to identify the mutation in the 573 patients tested. These results are in contrast with the mutation frequency observed in a study including 815 BC-affected women from Brazil, in which carrier frequencies of 12.1 and 5.1% in pre- and postmenopausal women were observed, respectively. These findings suggest that the Brazilian founder mutation p.R337H, the most frequent germline TP53 mutation reported to date, is not a common germline alteration in Portuguese women diagnosed with BC. PMID:25052705

Giacomazzi, Juliana; Correia, Rudinei Luis; Palmero, Edenir Ines; Gaspar, Jorge Francisco; Almeida, Marta; Portela, Catarina; Camey, Suzi Alves; Monteiro, Augusta; Pinheiro, Manuela; Peixoto, Ana; Teixeira, Manuel R; Reis, Rui Manuel; Ashton-Prolla, Patricia

2014-01-01

211

Meningococcal Disease  

MedlinePLUS

... A Hepatitis B HPV (Human Papillomavirus) Influenza (Flu) Measles Meningococcal Disease Mumps Pertussis (Whooping Cough) Pneumococcal Disease Rubella (German Measles) Shingles (Herpes Zoster) Tetanus (Lockjaw) Professional Resources Adult ...

212

Lyme disease in the United Kingdom.  

PubMed

Lyme disease, while still an uncommon disease in the UK, is on the increase. Case numbers have increased by 3.6-fold since 2001, with over 950 cases reported by the Health Protection Agency (HPA) in 2011, compared with less than 500 cases annually pre-2004. HPA indications of the true incidence are suggested to be closer to 3000 cases/year, of which around 82% of cases are indigenously acquired. Three genospecies, Borrelia burgdorferi sensu stricto, Borrelia afzelli and Borrelia garinii, represent the predominant pathogenic variants in the UK. Erythema migrans is the commonest manifestation, occurring in 60%-91% of cases. In the UK, neuroborelliosis is the most common complication, while myocarditis is unusual, and death from either conduction disease or carditis is extremely rare. The role of Borrelia infection in chronic dilated cardiomyopathy in the UK remains unproven. Controversy over the existence of either 'chronic Lyme disease' and/or 'post-Lyme disease syndrome' continues unabated. National medical societies, patient advocacy groups, insurance companies, lawyers, doctors, the private health medical sector and scientific journals have all become embroiled in this bitter controversy. New developments include diagnostic tests able to detect Lyme disease at an earlier stage, shorter durations of antibiotic therapy and potential advances in vaccines against Borrelia. PMID:24198341

Dubrey, Simon W; Bhatia, Ajay; Woodham, Sarah; Rakowicz, Wojtek

2014-01-01

213

Successful treatment of Kimura's disease with leflunomide and methylprednisolone: a case report.  

PubMed

Kimura's disease (KD) is an uncommon, chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes and increased peripheral blood eosinophil counts and serum immunoglobulin E (IgE). Prednisone is widely used to treat the disease. Here, we reported a 59-year-old KD patient failed to response to prednisone. Leflunomide combined with methylprednisolone (Medrol) were carried out to treat KD and encouraging outcome was obtained during the medication and 1 year follow up period. PMID:25232411

Ma, Xiao-Rong; Xin, Shu-Jia; Ouyang, Tian-Xiang; Ma, Yue-Ting; Chen, Wei-Ying; Chang, Meng-Ling

2014-01-01

214

Successful treatment of Kimura’s disease with leflunomide and methylprednisolone: a case report  

PubMed Central

Kimura’s disease (KD) is an uncommon, chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes and increased peripheral blood eosinophil counts and serum immunoglobulin E (IgE). Prednisone is widely used to treat the disease. Here, we reported a 59-year-old KD patient failed to response to prednisone. Leflunomide combined with methylprednisolone (Medrol) were carried out to treat KD and encouraging outcome was obtained during the medication and 1 year follow up period. PMID:25232411

Ma, Xiao-Rong; Xin, Shu-Jia; Ouyang, Tian-Xiang; Ma, Yue-Ting; Chen, Wei-Ying; Chang, Meng-Ling

2014-01-01

215

Non-communicable diseases--finally on the global agenda.  

PubMed

After the UN Millennium Development Goals were declared in September 2000 (see Table 1), one of the major short-comings recognized world-wide was the lack of mention of non-communicable diseases (NCDs). While AIDS and malaria were included, none of the leading and universal non-communicable causes of death made the list. There was no mention of cardiovascular diseases, cancer or diabetes, even though these place a far greater burden on global health and economic development than the infectious diseases, and are predicted to continue to increase in epidemic proportions. After much public discussion and intense lobbying, a significant-and uncommon-achievement occurred: on May 13th, 2010, the United Nations General Assembly voted in favour of convening a summit on non-communicable diseases, to take place in September 2011. PMID:21733345

Wielgosz, A T

2011-06-01

216

Current Status of Therapy in Autoimmune Liver Disease  

PubMed Central

Therapeutic strategies for autoimmune liver diseases are increasingly established. Although proportionately uncommon, specialist centers have with time refined the best approaches for each disease, based on an improved understanding of the spectrum of presentation. The major treatment aims are to prevent end-stage liver disease and its associated complications. As a result of drugs such as ursodeoxycholic acid, predniso(lo)ne and azathioprine, both primary biliary cirrhosis and autoimmune hepatitis are now less commonly indications for liver transplantation. Unfortunately, the same inroads in treatment efficacy have as yet not been made for primary sclerosing cholangitis, although the recognition that a subset of patients may have a treatable secondary sclerosing cholangitis (IgG4 related) is helping a proportion. With better biological understanding, more specific interventions are expected that will benefit all those with autoimmune liver diseases. PMID:21180531

Al-Harthi, Nadya; Heathcote, E. Jenny

2009-01-01

217

Myxomatous mitral valve disease in dogs: Does size matter?  

PubMed Central

Myxomatous mitral valve disease (MMVD) is the most commonly diagnosed cardiovascular disease in the dog accounting for more than 70% of all cardiovascular disease in dogs. As are most canine diseases with genetic underpinnings, risk of MMVD is greatly increased in a subset of breeds. What is uncommon is that the vast majority of the breeds at elevated risk for MMVD are small or toy breeds with average adult weights under 9 kg. These breeds appear to have little in common other than their diminutive size. In the following review we propose a number of mechanisms by which relatively unrelated small breeds may have developed a predisposition for chronic valvular disorders. Although factors such as age are key in the expression of MMVD, taking a comprehensive look at the commonalities, as well as the differences, between the susceptible breeds may assist in finding the causal variants responsible for MMVD and translating them to improved treatments for both dogs and humans. PMID:22356836

Parker, Heidi G.; Kilroy-Glynn, Paul

2012-01-01

218

Rehabilitation of avulsion fracture of the tibial tuberosity following Osgood-Schlatter disease  

Microsoft Academic Search

A sixteen-year-old boy suffered from sharp pain in the knee during a jump while playing basketball. He had a positive history of Osgood Schlatter disease. Radiographic evaluation demonstrated an avulsion fracture of the tibial tuberosity Type III according to the classification of Watson-Jones. Rehabilitation after avulsion fracture of the tibial tuberosity is an important consideration for this relatively uncommon adolescent

G. Baltaci; H. Özer; V. B. Tunay

2004-01-01

219

Large-scale gene-centric analysis identifies novel variants for coronary artery disease  

Microsoft Academic Search

Coronary artery disease (CAD) has a significant genetic contribution that is incompletely characterized. To complement genome-wide association (GWA) studies, we conducted a large and systematic candidate gene study of CAD susceptibility, including analysis of many uncommon and functional variants. We examined 49,094 genetic variants in approximately 2,100 genes of cardiovascular relevance, using a customised gene array in 15,596 CAD cases

A. S. Butterworth; P. S. Braund; R. J. Hardwick; D. Saleheen; J. F. Peden; N. Soranzo; J. C. Chambers; M. E. Kleber; B. Keating; A. Qasim; N. Klopp; J. Erdmann; H. Basart; J. H. Baumert; C. R. Bezzina; B. O. Boehm; J. Brocheton; P. Bugert; F. Cambien; R. Collins; D. Couper; J. S. de Jong; P. Diemert; K. Ejebe; C. C. Elbers; P. Elliott; M. Fornage; P. Frossard; S. Garner; S. E. Hunt; J. J. Kastelein; O. H. Klungel; H. Kluter; K. Koch; I. R. Konig; A. S. Kooner; K. Liu; R. McPherson; M. D. Musameh; S. Musani; G. Papanicolaou; A. Peters; B. J. Peters; S. Potter; B. M. Psaty; A. Rasheed; J. Scott; U. Seedorf; J. S. Sehmi; N. Sotoodehnia; K. Stark; J. Stephens; C. E. van der Schoot; Y. T. van der Schouw; P. van der Harst; R. S. Vasan; A. A. Wilde; C. Willenborg; B. R. Winkelmann; M. Zaidi; W. Zhang; A. Ziegler; W. Koenig; W. Matz; M. D. Trip; M. P. Reilly; S. Kathiresan; H. Schunkert; A. Hamsten; A. S. Hall; J. S. Kooner; S. G. Thompson; J. R. Thompson; H. Watkins; J. Danesh; T. Barnes; S. Rafelt; V. Codd; N. Bruinsma; L. R. Dekker; J. P. Henriques; R. J. de Winter; M. Alings; C. F. Allaart; A. P. Gorgels; F. W. A. Verheugt; M. Mueller; C. Meisinger; S. DerOhannessian; N. N. Mehta; J. Ferguson; H. Hakonarson; W. Matthai; R. Wilensky; J. C. Hopewell; S. Parish; P. Linksted; J. Notman; H. Gonzalez; A. Young; T. Ostley; A. Munday; N. Goodwin; V. Verdon; S. Shah; C. Edwards; C. Mathews; R. Gunter; J. Benham; C. Davies; M. Cobb; L. Cobb; J. Crowther; A. Richards; M. Silver; S. Tochlin; S. Mozley; S. Clark; M. Radley; K. Kourellias; P. Olsson; S. Barlera; G. Tognoni; S. Rust; G. Assmann; S. Heath; D. Zelenika; I. Gut; F. Green; M. Farrall; A. Goel; H. Ongen; M. G. Franzosi; M. Lathrop; R. Clarke; A. Aly; K. Anner; K. Bjorklund; G. Blomgren; B. Cederschiold; K. Danell-Toverud; P. Eriksson; U. Grundstedt; M. Heinonen; M. L. Hellenius; F. van't Hooft; K. Husman; J. Lagercrantz; A. Larsson; M. Larsson; M. Mossfeldt; A. Malarstig; G. Olsson; M. Sabater-Lleal; B. Sennblad; A. Silveira; R. Strawbridge; B. Soderholm; J. Ohrvik; K. S. Zaman; N. H. Mallick; M. Azhar; A. Samad; M. Ishaq; N. Shah; M. Samuel; T. L. Assimes; H. Holm; M. Preuss; A. F. Stewart; M. Barbalic; C. Gieger; D. Absher; Z. Aherrahrou; H. Allayee; D. Altshuler; S. Anand; K. Andersen; J. L. Anderson; D. Ardissino; S. G. Ball; A. J. Balmforth; L. C. Becker; D. M. Becker; K. Berger; J. C. Bis; S. M. Boekholdt; E. Boerwinkle; M. J. Brown; M. S. Burnett; I. Buysschaert; J. F. Carlquist; L. Chen; R. W. Davies; G. Dedoussis; A. Dehghan; S. Demissie; J. Devaney; A. Doering; N. E. El Mokhtari; S. G. Ellis; R. Elosua; J. C. Engert; S. Epstein; U. de Faire; M. Fischer; A. R. Folsom; J. Freyer; B. Gigante; D. Girelli; S. Gretarsdottir; V. Gudnason; J. R. Gulcher; S. Tennstedt; E. Halperin; N. Hammond; S. L. Hazen; A. Hofman; B. D. Horne; T. Illig; C. Iribarren; G. T. Jones; J. W. Jukema; M. A. Kaiser; L. M. Kaplan; K. T. Khaw; J. W. Knowles; G. Kolovou; A. Kong; R. Laaksonen; D. Lambrechts; K. Leander; M. Li; W. Lieb; G. Lettre; C. Loley; A. J. Lotery; P. M. Mannucci; N. Martinelli; P. P. McKeown; T. Meitinger; O. Melander; P. A. Merlini; V. Mooser; T. Morgan; Muhleisen T. W; J. B. Muhlestein; K. Musunuru; J. Nahrstaedt; M. M. Nothen; O. Olivieri; F. Peyvandi; R. S. Patel; C. C. Patterson; L. Qu; A. A. Quyyumi; D. J. Rader; L. S. Rallidis; C. Rice; F. R. Roosendaal; D. Rubin; V. Salomaa; M. L. Sampietro; M. S. Sandhu; E. Schadt; A. Schafer; A. Schillert; S. Schreiber; J. Schrezenmeir; S. M. Schwartz; D. S. Siscovick; M. Sivananthan; S. Sivapalaratnam; A. V. Smith; T. B. Smith; J. D. Snoep; J. A. Spertus; K. Stefansson; K. Stirrups; M. Stoll; W. H. Tang; G. Thorgeirsson; G. Thorleifsson; M. Tomaszewski; A. G. Uitterlinden; A. M. van Rij; B. F. Voight; N. J. Wareham; G. AWells; H. E. Wichmann; J. C. Witteman; B. J. Wright; S. Ye; L. A. Cupples; T. Quertermous; W. Marz; S. Blankenberg; U. Thorsteinsdottir; R. Roberts; C. J. O'Donnell; N. C. Onland-Moret; J. van Setten; P. I. de Bakker; W. M. Verschuren; J. M. Boer; C. Wijmenga; M. H. Hofker; A. H. Maitland-van der Zee; A. de Boer; D. E. Grobbee; T. Attwood; S. Belz; J. Cooper; A. Crisp-Hihn; P. Deloukas; N. Foad; A. H. Goodall; J. Gracey; E. Gray; R. Gwilliams; S. Heimerl; C. Hengstenberg; J. Jolley; U. Krishnan; H. Lloyd-Jones; I. Lugauer; P. Lundmark; S. Maouche; J. S. Moore; D. Muir; E. Murray; C. P. Nelson; J. Neudert; D. Niblett; K. O'Leary; W. H. Ouwehand; H. Pollard; A. Rankin; H. Sager; N. J. Samani; J. Sambrook; G. Schmitz; M. Scholz; L. Schroeder; A. C. Syvannen; C. Wallace

2011-01-01

220

Pigmented Bowen's disease of the penis and scrotum in a patient with AIDS.  

PubMed

Patients with HIV have higher risk of developing squamous cell carcinoma of the skin given the increased risk of HPV infection, which alters cell proliferation and apoptosis [1]. Pigmented Bowen's disease is an uncommon form of squamous cell carcinoma in-situ characterized by pigmented lesions that can clinically mimic superficial spreading melanoma, pigmented basal cell carcinoma, melanocytic nevus, Bowenoid papulosis, and seborrheic keratosis. PMID:24746300

Al-Dawsari, Najla A; Raslan, Wasim; Dawamneh, M Fawaz

2014-01-01

221

Dercum’s disease (adiposis dolorosa): a new case-report  

Microsoft Academic Search

Dercum’s disease, or adiposa dolorosa, is a rare condition characterized by the development of multiple painful subcutaneous lipomas. Postmenopausal women are selectively affected. The ankle area is an uncommon site of involvement. We report a case with lipomas about the ankle, and we review the relevant literature.Case-report. – This 46-year-old perimenopausal woman receiving follow-up for depression presented in December 1997

Bouchra Amine; Fabienne Leguilchard; Claude Laurent Benhamou

2004-01-01

222

Kennedy's Disease  

MedlinePLUS

NINDS Kennedy's Disease Information Page Synonym(s): Bulbospinal Muscular Atrophy, X-Linked Spinal and Bulbar Muscular Atrophy Table of Contents (click ... is being done? Clinical Trials Organizations What is Kennedy's Disease? Kennedy's disease is an inherited motor neuron disease ...

223

Autonomic dysfunction in Parkinson's disease.  

PubMed

This article is a review of autonomic dysfunction in idiopathic Parkinson's disease (iPD), as well as the clinical features of a specific form of PD, i.e. autonomic failure (AF) with PD, and is based mainly on the results obtained from our recent studies. Since James Parkinson's original discription, the definition of autonomic dysfunctions in iPD and their clinical characteristics have undergone changes. Autonomic dysfunction is considered to be uncommon and rarely severe on one hand, while not infrequent but not as severe as in Shy-Drager syndrome on the other hand. AF with PD is characterized by severe orthostatic hypotension, postprandial hypotension, supersensitivity to noradrenaline, low or absent uptake of m-[123I]iodobenzylguanidine scintigraphy of the limbs, and preserved arginine vasopressin response to head-up tilt, suggesting a postganglionic sympathetic lesion resembling pure AF (PAF). On the other hand, reduced cortical glucose metabolism in positron emission tomography study may indicate that AF with PD has diffuse nervous system lesions resembling diffuse Lewy body disease. PMID:9387797

Koike, Y; Takahashi, A

1997-01-01

224

Prion Diseases  

Microsoft Academic Search

The modern history of the prion diseases is one of novel microbes, anthropological intrigue, and food safety mishaps. The\\u000a prion diseases, also called the transmissible spongiform encephalopathies, are fatal neurodegenerative diseases that can be\\u000a sporadic, inherited, or acquired. These multiple origins are unique among human disease. The basis of all prion diseases is\\u000a the misfolding of the host prion protein

Qingzhong Kong; Richard A. Bessen

225

Threatening auditory hallucinations and Cotard syndrome in Parkinson disease.  

PubMed

Psychotic symptoms are commonly reported in patients with Parkinson disease (PD). In particular, patients experience nonthreatening visual hallucinations that can occur with insight (so called hallucinosis) or without. Auditory hallucinations are uncommon, and schizophrenialike symptoms such as pejorative and threatening auditory hallucinations and delusions that are persecutory, referential, somatic, religious, or grandiose have rarely been reported. The authors present 2 PD patients who experienced threatening auditory hallucinations, without visual hallucinations, and schizophrenialike delusions with detailed description of the clinical phenomenology including 1 patient with Cotard syndrome. PMID:15602098

Factor, Stewart A; Molho, Eric S

2004-01-01

226

Pregnancy and reproduction in autoimmune rheumatic diseases.  

PubMed

Despite evidence for the important role of oestrogens in the aetiology and pathophysiology of chronic immune/inflammatory diseases, the previous view of an unequivocal beneficial effect of oestrogens on RA compared with a detrimental effect on SLE has to be reconsidered. Likewise, the long-held belief that RA remits in the majority of pregnant patients has been challenged, and shows that only half of the patients experience significant improvement when objective disease activity measurements are applied. Pregnancies in patients with SLE are mostly successful when well planned and monitored interdisciplinarily, whereas a small proportion of women with APS still have adverse pregnancy outcomes in spite of the standard treatment. New prospective studies indicate better outcomes for pregnancies in women with rare diseases such as SSc and vasculitis. Fertility problems are not uncommon in patients with rheumatic disease and need to be considered in both genders. Necessary therapy, shortly before or during the pregnancy, demands taking into account the health of both mother and fetus. Long-term effects of drugs on offspring exposed in utero or during lactation is a new area under study as well as late effects of maternal rheumatic disease on children. PMID:21097449

Ostensen, Monika; Brucato, Antonio; Carp, Howard; Chambers, Christina; Dolhain, Radboud J E M; Doria, Andrea; Förger, Frauke; Gordon, Caroline; Hahn, Sinuhe; Khamashta, Munther; Lockshin, Michael D; Matucci-Cerinic, Marco; Meroni, Pierluigi; Nelson, J Lee; Parke, Ann; Petri, Michelle; Raio, Luigi; Ruiz-Irastorza, Guillermo; Silva, Clovis A; Tincani, Angela; Villiger, Peter M; Wunder, Dorothea; Cutolo, Maurizio

2011-04-01

227

Epidemiology of gallbladder disease: cholelithiasis and cancer.  

PubMed

Diseases of the gallbladder are common and costly. The best epidemiological screening method to accurately determine point prevalence of gallstone disease is ultrasonography. Many risk factors for cholesterol gallstone formation are not modifiable such as ethnic background, increasing age, female gender and family history or genetics. Conversely, the modifiable risks for cholesterol gallstones are obesity, rapid weight loss and a sedentary lifestyle. The rising epidemic of obesity and the metabolic syndrome predicts an escalation of cholesterol gallstone frequency. Risk factors for biliary sludge include pregnancy, drugs like ceftiaxone, octreotide and thiazide diuretics, and total parenteral nutrition or fasting. Diseases like cirrhosis, chronic hemolysis and ileal Crohn's disease are risk factors for black pigment stones. Gallstone disease in childhood, once considered rare, has become increasingly recognized with similar risk factors as those in adults, particularly obesity. Gallbladder cancer is uncommon in developed countries. In the U.S., it accounts for only ~ 5,000 cases per year. Elsewhere, high incidence rates occur in North and South American Indians. Other than ethnicity and female gender, additional risk factors for gallbladder cancer include cholelithiasis, advancing age, chronic inflammatory conditions affecting the gallbladder, congenital biliary abnormalities, and diagnostic confusion over gallbladder polyps. PMID:22570746

Stinton, Laura M; Shaffer, Eldon A

2012-04-01

228

Inflammatory ascending aortic disease: Perspectives from pathology.  

PubMed

Inflammatory diseases of the aorta comprise a spectrum of disease with diverse clinical and histopathologic presentations. Broadly, they may be dichotomized into infectious and noninfectious varieties. Although uncommon, infectious forms, caused by bacteria, fungi, or mycobacteria, may result from hematogenous seeding of the aorta or direct spread from a contiguous infectious source. The noninfectious forms include a number of entities, the most common of which is atherosclerosis, a disease that primarily affects the aortic intima but has important secondary effects on the media and adventitia that may result in aneurysm formation. Other important noninfectious inflammatory diseases include giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis (Wegener granulomatosis), sarcoidosis, and lymphoplasmacytic aortitis. Importantly, there is increasing recognition that there is a subset of cases of lymphoplasmacytic aortitis perhaps better classified under the spectrum of so-called IgG4-related sclerosing disease, with important clinical and therapeutic ramifications. This review focuses on the variable and defining characteristics of the inflammatory aortopathies, specifically those affecting the ascending aorta, and discusses areas of important clinical and pathological distinction between them. PMID:25199476

Maleszewski, Joseph J

2014-08-01

229

Evaluation of epidemiological cut-off values indicates that biocide resistant subpopulations are uncommon in natural isolates of clinically-relevant microorganisms.  

PubMed

To date there are no clear criteria to determine whether a microbe is susceptible to biocides or not. As a starting point for distinguishing between wild-type and resistant organisms, we set out to determine the minimal inhibitory concentration (MIC) and minimal bactericidal concentration (MBC) distributions for four common biocides; triclosan, benzalkonium chloride, chlorhexidine and sodium hypochlorite for 3319 clinical isolates, with a particular focus on Staphylococcus aureus (N?=?1635) and Salmonella spp. (N?=?901) but also including Escherichia coli (N?=?368), Candida albicans (N?=?200), Klebsiella pneumoniae (N?=?60), Enterobacter spp. (N?=?54), Enterococcus faecium (N?=?53), and Enterococcus faecalis (N?=?56). From these data epidemiological cut-off values (ECOFFs) are proposed. As would be expected, MBCs were higher than MICs for all biocides. In most cases both values followed a normal distribution. Bimodal distributions, indicating the existence of biocide resistant subpopulations were observed for Enterobacter chlorhexidine susceptibility (both MICs and MBCs) and the susceptibility to triclosan of Enterobacter (MBC), E. coli (MBC and MIC) and S. aureus (MBC and MIC). There is a concern on the potential selection of antibiotic resistance by biocides. Our results indicate however that resistance to biocides and, hence any potential association with antibiotic resistance, is uncommon in natural populations of clinically relevant microorganisms. PMID:24466194

Morrissey, Ian; Oggioni, Marco Rinaldo; Knight, Daniel; Curiao, Tania; Coque, Teresa; Kalkanci, Ayse; Martinez, Jose Luis

2014-01-01

230

Evaluation of Epidemiological Cut-Off Values Indicates that Biocide Resistant Subpopulations Are Uncommon in Natural Isolates of Clinically-Relevant Microorganisms  

PubMed Central

To date there are no clear criteria to determine whether a microbe is susceptible to biocides or not. As a starting point for distinguishing between wild-type and resistant organisms, we set out to determine the minimal inhibitory concentration (MIC) and minimal bactericidal concentration (MBC) distributions for four common biocides; triclosan, benzalkonium chloride, chlorhexidine and sodium hypochlorite for 3319 clinical isolates, with a particular focus on Staphylococcus aureus (N?=?1635) and Salmonella spp. (N?=?901) but also including Escherichia coli (N?=?368), Candida albicans (N?=?200), Klebsiella pneumoniae (N?=?60), Enterobacter spp. (N?=?54), Enterococcus faecium (N?=?53), and Enterococcus faecalis (N?=?56). From these data epidemiological cut-off values (ECOFFs) are proposed. As would be expected, MBCs were higher than MICs for all biocides. In most cases both values followed a normal distribution. Bimodal distributions, indicating the existence of biocide resistant subpopulations were observed for Enterobacter chlorhexidine susceptibility (both MICs and MBCs) and the susceptibility to triclosan of Enterobacter (MBC), E. coli (MBC and MIC) and S. aureus (MBC and MIC). There is a concern on the potential selection of antibiotic resistance by biocides. Our results indicate however that resistance to biocides and, hence any potential association with antibiotic resistance, is uncommon in natural populations of clinically relevant microorganisms. PMID:24466194

Morrissey, Ian; Oggioni, Marco Rinaldo; Knight, Daniel; Curiao, Tania; Coque, Teresa; Kalkanci, Ayse; Martinez, Jose Luis

2014-01-01

231

Revisiting Mednick’s Model on Creativity-Related Differences in Associative Hierarchies. Evidence for a Common Path to Uncommon Thought  

PubMed Central

Fifty years ago, Mednick [Psychological Review, 69 (1962) 220] proposed an elaborate model that aimed to explain how creative ideas are generated and why creative people are more likely to have creative ideas. The model assumes that creative people have flatter associative hierarchies and as a consequence can more fluently retrieve remote associative elements, which can be combined to form creative ideas. This study aimed at revisiting Mednick’s model and providing an extensive test of its hypotheses. A continuous free association task was employed and association performance was compared between groups high and low in creativity, as defined by divergent thinking ability and self-report measures. We found that associative hierarchies do not differ between low and high creative people, but creative people showed higher associative fluency and more uncommon responses. This suggests that creativity may not be related to a special organization of associative memory, but rather to a more effective way of accessing its contents. The findings add to the evidence associating creativity with highly adaptive executive functioning. PMID:24532853

Benedek, Mathias; Neubauer, Aljoscha C.

2014-01-01

232

Grover's Disease  

MedlinePLUS

... No, Keep Private Grover's Disease Share | Grover's disease (transient acantholytic dermatosis) is a condition that appears suddenly ... months (which is why it was originally called "transient"). Unfortunately it may last much longer. The cause ...

233

Alpers' Disease  

MedlinePLUS

NINDS Alpers' Disease Information Page Synonym(s): Progressive Sclerosing Poliodystrophy Table of Contents (click to jump to sections) What is Alpers' Disease? Is there any treatment? What is the prognosis? What research ...

234

Chagas disease  

MedlinePLUS

American trypanosomiasis ... Kirchhoff LV. Trypanosoma species (American trypanosomiasis, Chagas' disease): Biology of trypanosomes. In: Mandell GL, Bennett JE, Dolin R, eds. Principles and Practice of Infectious Diseases . 7th ed. ...

235

Heart Disease  

MedlinePLUS

... blood vessels. You might know someone who has cardiovascular disease because more than 60 million Americans have some ... The blood carries oxygen, which the cells need. Cardiovascular disease is a group of problems that occur when ...

236

Parkinson's Disease  

MedlinePLUS

... You may have seen the actor Michael J. Fox on TV talking about Parkinson's disease. He has ... and help find a cure. Mostly adults (like Fox and boxer Muhammad Ali) get Parkinson's disease. It's ...

237

Mycobacterial Diseases  

MedlinePLUS

... are conducting research on The biology of mycobacteria species that cause human disease How mycobacterial diseases develop in the body Interactions between the human immune system and mycobacteria Immunology ...

238

Lyme Disease  

MedlinePLUS

... Content Marketing Share this: Main Content Area A History of Lyme Disease, Symptoms, Diagnosis, Treatment, and Prevention ... Diagnosis Treatment Prevention Borellia burgdorferi . Credit: NIAID A History of Lyme Disease In the early 1970s, a ...

239

Wildlife Diseases  

E-print Network

Some wildlife diseases can be transmitted to humans. This leaflet explains the causes and symptoms of rabies, giardiasis, bubonic plague, Rocky Mountain spotted fever, Lyme disease, tularemia, leptospirosis and histoplasmosis....

Texas Wildlife Services

2007-03-13

240

Reportable diseases  

MedlinePLUS

... make a report by phone. Examples are rubeola (measles) and pertussis (whooping cough). Report of total number ... related infant deaths Legionellosis Listeriosis Lyme disease Malaria Measles Meningococcal disease Mumps Novel influenza A virus infections ...

241

Alexander Disease  

MedlinePLUS

... no exact animal model for the disease; however, mice have been engineered to produce the same mutant ... GFAP found in individuals with Alexander disease. These mice form Rosenthal fibers and have a predisposition for ...

242

Celiac Disease  

MedlinePLUS

... with celiac disease, a lifelong disorder of the digestive system, these foods aren't always the treats that ... commonly consumed ingredient. What Is Celiac Disease? The digestive system is the set of organs that digest food ...

243

Periodontal Diseases  

MedlinePLUS

... diseases. ? The primary research focus was on oral bacteria. Periodontal diseases were thought to begin when chalky ... small pocket between the tooth and gingiva, allowing bacteria to freely enter and progressively erode the bone ...

244

Whipple's Disease  

MedlinePLUS

... and large intestine. [ Top ] What causes Whipple disease? Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple disease. ... whipplei infects people; however, scientists have noted the bacteria are found in soil and sewage wastewater the ...

245

Whipple's disease  

MedlinePLUS

Whipple's disease is a rare condition that prevents the small intestines from allowing nutrients to pass into ... Whipple's disease is caused by infection with a form of bacteria called Tropheryma whippelii . The disorder mainly ...

246

Heart Disease  

MedlinePLUS

... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ...

247

Binswanger's Disease  

MedlinePLUS

... BD is a progressive disease; there is no cure. Changes may be sudden or gradual and then progress in a stepwise manner. BD can often coexist with Alzheimer's disease. Behaviors that slow the progression of high ...

248

Tickborne Diseases  

MedlinePLUS

... diseases are becoming a serious problem in this country as people increasingly build homes in formerly uninhabited wilderness areas where ticks and their animal hosts live. Tickborne diseases can be caused by viruses, ...

249

Parkinson disease  

MedlinePLUS

Parkinson disease causes certain brain cells to die. These are the cells that help control movement and coordination. ... called dopamine to help control muscle movement. With Parkinson disease, the brain cells that make dopamine slowly die. ...

250

Krabbe Disease  

MedlinePLUS

... better for children who receive umbilical cord blood stem cells prior to disease onset or early bone marrow transplantation. Persons with juvenile- or adult-onset cases of Krabbe disease generally have a ...

251

Lyme Disease  

MedlinePLUS

Lyme disease is a bacterial infection you get from the bite of an infected tick. The first symptom ... Muscle and joint aches A stiff neck Fatigue Lyme disease can be hard to diagnose because you may ...

252

Behçet's disease  

Microsoft Academic Search

Background: Behçet's disease is a multisystem disease that is rare in the United States.Objective: The purpose of our study was to assess the characteristics and treatment of a series of patients with Behçet's disease in the United States.Methods: A retrospective clinical review of 25 patients with Behçet's disease was performed, and histopathologic findings and therapeutic modalities were reviewed.Results: All patients

Heidi C. Mangelsdorf; Wain L. White; Joseph L. Jorizzo

1996-01-01

253

Autoinflammatory Diseases  

MedlinePLUS

... treatment, a synthetic form of human IL-1Ra. Behçet’s Disease Behçet’s disease causes canker sores or ulcers in the ... of the digestive tract, brain, and spinal cord. Behçet’s disease is common in the Middle East, Asia, ...

254

Lyme disease  

MedlinePLUS

... Lyme disease: part II. Management and prevention. J Am Acad Dermatol. 2011;64:639-653. Centers for Disease Control. Lyme disease. Page last updated November 15, 2013. Available at: http://www.cdc.gov/lyme. Accessed February 25, 2014. Halperin JJ, Shapiro ...

255

Lyme Disease.  

ERIC Educational Resources Information Center

This overview of the public health significance of Lyme disease includes the microbiological specifics of the infectious spirochete, the entomology and ecology of the ticks which are the primary disease carrier, the clinical aspects and treatment stages, the known epidemiological patterns, and strategies for disease control and for expanded public…

Taylor, George C.

1991-01-01

256

Alzheimer's Disease  

MedlinePLUS

... years of age and the sixth leading cause of death for all people in the United States. Most people live 4 to 8 years after being diagnosed with Alzheimer’s disease. Some live with the disease for up ... of Alzheimer’s disease? The Alzheimer’s Association has identified 10 ...

257

Introducing Polyautoimmunity: Secondary Autoimmune Diseases No Longer Exist  

PubMed Central

Similar pathophysiological mechanisms within autoimmune diseases have stimulated searches for common genetic roots. Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. When three or more autoimmune diseases coexist, this condition is called multiple autoimmune syndrome (MAS). We analyzed the presence of polyautoimmunity in 1,083 patients belonging to four autoimmune disease cohorts. Polyautoimmunity was observed in 373 patients (34.4%). Autoimmune thyroid disease (AITD) and Sjögren's syndrome (SS) were the most frequent diseases encountered. Factors significantly associated with polyautoimmunity were female gender and familial autoimmunity. Through a systematic literature review, an updated search was done for all MAS cases (January 2006–September 2011). There were 142 articles retrieved corresponding to 226 cases. Next, we performed a clustering analysis in which AITD followed by systemic lupus erythematosus and SS were the most hierarchical diseases encountered. Our results indicate that coexistence of autoimmune diseases is not uncommon and follows a grouping pattern. Polyautoimmunity is the term proposed for this association of disorders, which encompasses the concept of a common origin for these diseases. PMID:22454759

Rojas-Villarraga, Adriana; Amaya-Amaya, Jenny; Rodriguez-Rodriguez, Alberto; Mantilla, Rubén D.; Anaya, Juan-Manuel

2012-01-01

258

Introducing polyautoimmunity: secondary autoimmune diseases no longer exist.  

PubMed

Similar pathophysiological mechanisms within autoimmune diseases have stimulated searches for common genetic roots. Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. When three or more autoimmune diseases coexist, this condition is called multiple autoimmune syndrome (MAS). We analyzed the presence of polyautoimmunity in 1,083 patients belonging to four autoimmune disease cohorts. Polyautoimmunity was observed in 373 patients (34.4%). Autoimmune thyroid disease (AITD) and Sjögren's syndrome (SS) were the most frequent diseases encountered. Factors significantly associated with polyautoimmunity were female gender and familial autoimmunity. Through a systematic literature review, an updated search was done for all MAS cases (January 2006-September 2011). There were 142 articles retrieved corresponding to 226 cases. Next, we performed a clustering analysis in which AITD followed by systemic lupus erythematosus and SS were the most hierarchical diseases encountered. Our results indicate that coexistence of autoimmune diseases is not uncommon and follows a grouping pattern. Polyautoimmunity is the term proposed for this association of disorders, which encompasses the concept of a common origin for these diseases. PMID:22454759

Rojas-Villarraga, Adriana; Amaya-Amaya, Jenny; Rodriguez-Rodriguez, Alberto; Mantilla, Rubén D; Anaya, Juan-Manuel

2012-01-01

259

[Onychomadesis associated with mouth, hand and foot disease].  

PubMed

Onychomadesis is the spontaneous, complete shedding of the nail from its proximal side, without pain or inflammation, following nail matrix arrest. This disorder is uncommon in children and it can occur in fingernails, toenails or both. It may be secondary to systemic disorders, Kawasaki disease, bullous dermatoses, drugs, paronychia, stress and radiotherapy. Since 2000, Hand, Foot, and Mouth Disease (HFMD) has been described as a cause of onychomadesis, and has been associated with outbreaks of this condition in different regions of the world. HFMD is an infection characterized by vesicular and erosive stomatitis in combination with a vesicular eruption in palms and soles. It occurs in small children during summer and autumn months, and it is caused by coxsackie virus. We present a study that reflects the current situation of onychomadesis in Argentinian children and shows a strong association between this disorder and HFMD, suggesting that onychomadesis is a new manifestation of a previously known disease. PMID:24196774

Ferrari, Bruno; Taliercio, Vanina; Hornos, Lorena; Luna, Paula; Abad, María Eugenia; Larralde, Margarita

2013-12-01

260

Early gastric cancer in Menetrier’s disease  

PubMed Central

Uncommon conditions such as pernicious anaemia and hypertrophic gastropathies have been considered as risk factors for gastric cancer; however, the exact increase in risk is unknown. Menetrier’s disease is a rare hyperproliferative disorder of the stomach caused by an overexpression of tumour growth factor ?, a ligand for the tyrokinase epidermal growth factor receptor, resulting in a selective expansion of surface mucous cells in the body and fundus of the stomach. There have been nearly 200 cases of Menetrier’s disease reported in the literature yet less than 15 have been associated with gastric adenocarcinoma. Here, we report an early stage gastric adenocarcinoma detected incidentally in a patient recently diagnosed with Menetrier’s disease. PMID:21686802

Remes-Troche, Jose Maria; Zapata-Colindres, Juan Carlos; Starkman, Ivethe; De Anda, Jazmin; Arista-Nasr, Julian; Valdovinos-Diaz, Miguel Angel

2009-01-01

261

Seizures in Alzheimer Disease  

PubMed Central

Background Transient symptoms in Alzheimer disease (AD) are frequent and include seizures, syncope, and episodes of inattention or confusion. The incidence of seizures in AD and predictors of which patients with AD might be more predisposed to them is based primarily on retrospective studies and is not well established. Objective To determine the incidence and predictors of new-onset unprovoked seizures. Design Prospective cohort study. Setting Three academic centers. Patients Four hundred fifty-three patients with probable AD observed prospectively from mild disease stages since 1992. Main Outcome Measure Informant interviews every 6 months included questions about whether the patient had a seizure (convulsion, fainting, or “funny” spell) and whether diagnosis or treatment for epilepsy or seizure was made. Two epileptologists independently retrospectively reviewed all available medical records for 52 patients with positive responses to either of these questions, and using a specific checklist form, events were diagnosed as to whether they were unprovoked seizures (intrarater concordance, ?=0.67). Diagnosis of unprovoked seizures constituted the event in survival analyses. Potential predictors included sex, age, race/ethnicity, educational achievement, duration of illness, baseline cognition and function, depression, medical comorbidities, and time-dependent use of cholinesterase inhibitors and neuroleptic agents, apolipoprotein E genotype, and previous electroencephalographic findings. Results Over the course of 3518 visit-assessments (per patient: mean, 7.8; maximum, 27), 7 patients (1.5%) developed seizures. Younger age was associated with higher risk (hazard ratio, 1.23; 95% confidence interval, 1.08–1.41; P=.003 for each additional year of age) of seizure incidence. No other predictor was significant. The overall incidence of seizures was low (418 per 100 000 person-years of observation) although significantly higher than expected for idiopathic unprovoked seizures in similar age ranges of the general population (hazard ratio, 8.06; 95% confidence interval, 3.23–16.61). Conclusions Unprovoked seizures are uncommon in AD, but they do occur more frequently than in the general population. Younger age is a risk factor for seizures in AD. PMID:19667221

Scarmeas, Nikolaos; Honig, Lawrence S.; Choi, Hyunmi; Cantero, Julio; Brandt, Jason; Blacker, Deborah; Albert, Marilyn; Amatniek, Joan C.; Marder, Karen; Bell, Karen; Hauser, W. Allen; Stern, Yaakov

2009-01-01

262

Eosinophil-associated lung diseases. A cry for surfactant proteins A and D help?  

PubMed

Surfactant proteins (SP)-A and SP-D (SP-A/-D) play important roles in numerous eosinophil-dominated diseases, including asthma, allergic bronchopulmonary aspergillosis, and allergic rhinitis. In these settings, SP-A/-D have been shown to modulate eosinophil chemotaxis, inhibit eosinophil mediator release, and mediate macrophage clearance of apoptotic eosinophils. Dysregulation of SP-A/-D function in eosinophil-dominated diseases is also not uncommon. Alterations in serum SP-A/-D levels are associated with disease severity in allergic rhinitis and chronic obstructive pulmonary disease. Furthermore, oligimerization of SP-A/-D, necessary for their proper function, can be perturbed by reactive nitrogen species, which are increased in eosinophilic disease. In this review, we highlight the associations of eosinophilic lung diseases with SP-A and SP-D levels and functions. PMID:24960334

Ledford, Julie G; Addison, Kenneth J; Foster, Matthew W; Que, Loretta G

2014-11-01

263

An uncommonly common: Glossopharyngeal neuralgia  

PubMed Central

Glossopharyngeal neuralgia is a relatively rare condition characterized by severe, paroxysmal episodes of pain localized to the external ear canal, the base of the tongue, the tonsil or the area beneath the angle of the jaw. This pain is many a times confused with Trigeminal Neuralgia and mistreated. There are various diagnostic and management dilemmas which are herein addressed in this review. PMID:23661955

Singh, P. M.; Kaur, Manpreet; Trikha, Anjan

2013-01-01

264

Rare species occupy uncommon niches  

NASA Astrophysics Data System (ADS)

The fact that temperate grasslands often contain upwards of 30 vascular plant species per m2 yet these species seem to have relatively similar life histories and resource requirements has made explaining species coexistence in these communities a major focus of research. While the reduction of competition by disturbance has been a popular explanation for species coexistence, in tallgrass prairies any level of disturbance either has no effect, or decreases diversity, since it favors the dominant plants. Although there has long been speculation that grassland species could coexist by niche partitioning the concept received renewed interest when it was shown that soil hydrology could explain species coexistence. One aspect of community structure that has not been explained by niche partitioning is the rareness and commonness of species within communities. There are three classes of explanations for rareness: narrow habitat requirements, low competitive ability combined with frequency dependent fitness and, dispersal ability. However, evidence for these explanations tend to be anecdotal, focusing on particular species. Here I show that in tallgrass prairies common and rare species consistently occupy different parts of niche space, with rare species being restricted by the cover of common species and occupying the rare available niches.

Markham, John

2014-08-01

265

Uncommon Connections with Common Numerators  

ERIC Educational Resources Information Center

Undergraduate students who are pre-service teachers need to make connections between the college mathematics they are learning and the pre-college mathematics they will be teaching. Spanning a broad range of undergraduate curricula, this article describes useful lesser-known connections, explorations, and original proofs involving fractions. In…

Lesser, Lawrence M.; Guthrie, Joe A.

2012-01-01

266

The challenge of treating orphan disease.  

PubMed

Uncommon or orphan diseases are less frequently addressed in mainstream medical journals and, as a consequence, their understanding and clinical recognition may rely on case series or anecdotal data with limited guidelines and management directions. The study of selected underrepresented autoimmune and allergy conditions is the subject of the present issue of Clinical Reviews in Allergy and Immunology to provide peculiar perspectives on common and rare themes. First, allergy remains a major concern for physicians worldwide despite the limited developments over the past years, particularly for antigens such as mite or Alternaria alternata, and due to the increasing incidence of drug hypersensitivity. Second, the female predominance of autoimmune diseases such as systemic sclerosis is well recognized but enigmatic, and a unifying hypothesis remains elusive. Third, the management of conditions triggered by infectious agents as in Guillain-Barre syndrome or mixed cryoglobulinemia is challenging, and clinical guidelines are needed in the setting of infections and autoimmunity. Fourth, gamma-delta T cells represent major players in innate immunity and are the subject of extensive studies in autoimmune diseases to provide new therapeutic targets for disease prevention or modulation in the near future. Ultimately, we acknowledge the major developments in the broad fields of rheumatology and immunology and expect that microbiota definition, epigenetics studies, and microRNA analysis will provide new exciting avenues toward the understanding and treatment of chronic and acute inflammation. PMID:25395247

Dias, Carlos; Selmi, Carlo

2014-12-01

267

Thyroid Diseases and Cerebrovascular Disease  

Microsoft Academic Search

Background and Purpose—Acute cerebral ischemia has been described in different diseases of the thyroid gland, and not only as a result of thyrotoxic atrial fibrillation and cardioembolic stroke. The purpose of this review is to summarize the studies on the relationship between thyroid diseases and cerebrovascular diseases, discussing the main findings for overt hyperthyroidism and hypothyroidism, as well as for

A. Squizzato; V. E. A. Gerdes; D. P. M. Brandjes; H. R. Buller; J. Stam

2010-01-01

268

An uncommon cause of tardy ulnar nerve palsy due to upper extremity prolonged malposition in a comatose child: a case report  

PubMed Central

Abstract: Background: Ulnar nerve neuropathy is one of the most common peripheral nerve dysfunctions. Elbow is the most common area affected by ulnar nerve which is mainly because of fractures or dislocations of this area. Delayed ulnar nerve palsy (Tardy Ulnar Nerve Palsy) in children due to a malpositioning of upper extremity during hospitalization is an uncommon cause of ulnar nerve injury which we have already reported it. Methods: An eight-year-old conscious patient who had weakness, paresthesia and tingling in the right 4th and 5th fingers, as well as right claw hand deformity was evaluated, he had attended once before in 4 months ago due to head trauma in coma state. The child had no clinical and radiological indications of arm or elbow fractures causing nerve compression or entrapment. Elbow malposition had caused ulnar nerve neuropathy during hospitalization. Surgery was attempted, ulnar nerve decompression and anterior transposition done. Results: After three weeks post operatively, active physical therapy was started on the right upper extremity and the hand returned to normal activity after 6 months. Conclusions: In patients with decreased level of consciousness or coma state who need prolonged hospitalization, the limbs must remain in correct position to prevent superficial nerve injuries and neuropathies. Furthermore, careful and scrutinized attention to the traumatic patients and doing on time and targeted imaging, regular follow up of patients, complete and perfect neurological examinations can prevent peripheral nerve injuries or develop on-time treatments which improve the patients' quality of life. Keywords: Ulnar nerve, Elbow malposition, Ulnar nerve decompression

Emamhadi, Mohammad Reza; Mahmoudi, Davood

2012-01-01

269

Diffuse aneurysmal and obstructive coronary artery disease: a do-not-intervene patient.  

PubMed

Coronary artery aneurysms are uncommon but potentially hazardous entities found on a small percentage of coronary angiograms. The best management strategy remains to be determined; the coexistence of obstructive coronary artery disease is the main determinant of prognosis. We report the case of a middle-aged woman with mixed aneurysmal and obstructive coronary artery disease presenting as an acute myocardial infarction, probably due to atherosclerosis. The unusual severity and complexity of the angiographic presentation of this case, with involvement of all major coronary arteries, raises important issues in terms of the patient's treatment options. PMID:23890759

Pereira, Eulália; Melica, Bruno; Primo, Joăo; Mota, Joăo C; Ferreira, Nuno; P Morais, Gustavo; Ponte, Marta; Caeiro, Daniel; Carvalho, Mónica; Bettencourt, Nuno; Vouga, Luís; Gama, Vasco

2013-01-01

270

Rosai-Dorfman disease with exclusive intra-abdominal lymphadenopathy masquerading as Wilkie's syndrome.  

PubMed

Rosai-Dorfman disease is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with nonspecific symptoms and massive cervical lymphadenopathy. Exclusive involvement of intra-abdominal lymph nodes is unusual and presentation mimicking Wilkie's syndrome due to compression of the third part of the duodenum by enlarged retroduodenal lymph nodes is rare. This entity should be included in the differential diagnosis with infectious, granulomatous and malignant causes of intra-abdominal lymphadenopathy. We highlight an uncommon presentation and discuss the challenges in the diagnosis and management of Rosai-Dorfman disease. PMID:24630515

Hussain Ashraf, Tarique; Chandra, Abhijit; Jauhari, Ramendra K; Kumar Singh, Sunil; Noushif, M

2014-03-01

271

[Social diseases, civilization diseases or lifestyle diseases?].  

PubMed

In general, the development of civilization is viewed as a positive step for the well-being of the human species, leading to an increased duration and quality of life. The accelerated progress of civilization (mainly industrialization, urbanization and nutrition) has lead to new possibilities for adverse effects on human health. In former high civilization--like old Egypt, Greece, Roman, Chinese, Indian, Maya civilizations--the "modem civilization diseases" were unknown. Modem science through improved sanitation, vaccination and antibiotics as well as improved social and economical conditions, has eliminated the threat of death from most infectious diseases. In the years after World War II the social, economic and health conditions changed. Most deaths have resulted from heart disease, stroke, cancer and other diseases as a result of an inappropriate relationship of people with their environment and changed lifestyle. Lifestyle diseases are different from other diseases because they are potentially preventable and can be lowered with changes in diet, lifestyle and environment. PMID:18350729

Betlejewski, Stans?aw

2007-01-01

272

Prion Diseases  

NSDL National Science Digital Library

Prion Diseases is one of a set of lecture notes for Virology 335 by Shaun Heaphy of Leicester University (UK). It contains detailed information on its topic, along with selected links. Although prion research has been going on for over 25 years, the scientific and medical communities have only recently acknowledged the existence of prions and there remains serious debate over their role in a variety of neurological diseases. The name "prion" is derived from "proteinaceous infectious particles," and was coined by Dr. Stanley Prusiner, who discovered the agents and who recently received the Nobel Prize for Medicine for his work. Prions are thought to be the first transmissible and heritable disease-causing agents that lack DNA and RNA. They are composed solely of protein and appear to be the cause of such diseases as kuru and Creutzfeldt-Jakob disease in humans, and bovine spongiform encephalopathies, mad cow disease, and scrapie in sheep and goats.

Heaphy, Shaun.

1997-01-01

273

Skin Diseases  

Microsoft Academic Search

In assigning health priorities, skin diseases are sometimes thought of, in planning terms, as small-time players in the global league of illness compared with diseases that cause signif- icant mortality, such as HIV\\/AIDS, community-acquired pneu- monias, and tuberculosis. However, skin problems are generally among the most common diseases seen in primary care settings in tropical areas, and in some regions

Roderick Hay; Sandra E. Bendeck; Suephy Chen; Roberto Estrada; Anne Haddix; Tonya McLeod; Antoine Mahé

274

Lyme Disease  

NSDL National Science Digital Library

A little microorganism called a spirochete causes Lyme disease, which can cause extremely severe symptoms, including neck stiffness, acute headaches, neurological damage, and rheumatoid arthritis-like problems. Lyme disease is transmitted by ticks and so is tied to the ticks' life cycle. Lyme disease is also seen by veterinarians, largely in dogs, for whom it can be fatal. Lyme research is ongoing on numerous fronts.

Dr. Leslie Nader (MSMR)

1992-04-14

275

Huntington's Disease  

PubMed Central

Huntington's disease (HD) is the most common inherited neurodegenerative disease and is characterized by uncontrolled excessive motor movements and cognitive and emotional deficits. The mutation responsible for HD leads to an abnormally long polyglutamine (polyQ) expansion in the huntingtin (Htt) protein, which confers one or more toxic functions to mutant Htt leading to neurodegeneration. The polyQ expansion makes Htt prone to aggregate and accumulate, and manipulations that mitigate protein misfolding or facilitate the clearance of misfolded proteins tend to slow disease progression in HD models. This article will focus on HD and the evidence that it is a conformational disease. PMID:21441583

Finkbeiner, Steven

2011-01-01

276

Comprehensive Carrier Screening and Molecular Diagnostic Testing for Recessive Childhood Diseases  

PubMed Central

Of 7,028 disorders with suspected Mendelian inheritance, 1,139 are recessive and have an established molecular basis. Although individually uncommon, Mendelian diseases collectively account for ~20% of infant mortality and ~18% of pediatric hospitalizations. Molecular diagnostic testing is currently available for only ~300 recessive disorders. Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis. However, extension of preconception screening and molecular diagnostic testing to most recessive disease genes has hitherto been impractical. Recently, we reported a preconception carrier screen / molecular diagnostic test for 448 recessive childhood diseases. The current status of this test is reviewed here. Currently, this reports analytical validity of the comprehensive carrier test. As the clinical validity and clinical utility in the contexts described is ascertained, this article will be updated. PMID:22872815

Kingsmore, Stephen

2012-01-01

277

[Kimura disease. Apropos of a case with review of the literature].  

PubMed

Kimura's disease is an uncommon chronic inflammatory disease of unknown etiology often associated with an important category of reactive lymphadenopathy in Oriental populations. The enlarged nodes are mostly located in the head and neck region. The main pathologic changes in the lymph nodes include florid germinal centers, Warthin-Finkeldey polykaryocytes, vascularization of the germinal centers, increased postcapillary veinules in the paracortex, eosinophilic infiltration and fibrosis. The disease is often confused with angiolymphoid hyperplasia with eosinophilia. Because the adenopathy may be the sole presentation of the disease, the accurate diagnosis may spare the patients unnecessary medical and/or surgical treatment. We report a case of cervical lymphadenopathy as a sole presentation of Kimura's disease. PMID:10881442

Habib, K; Faour, M

2000-01-01

278

Parkinson's disease.  

PubMed

Parkinson's disease is a common movement disorder caused by a deficiency of dopamine in the brain. The disease is chronic and progressive. While there are many treatments for symptomatic relief, there is no cure. This article addresses the etiology, clinical manifestations, standard and experimental therapeutics and role of the nurse in the comprehensive care of the patient and family. PMID:2529323

Vernon, G M

1989-10-01

279

Cardiovascular Disease  

Technology Transfer Automated Retrieval System (TEKTRAN)

Cardiovascular disease (CVD), particularly CHD (coronary heart disease) and stroke, remain the leading causes of death of women in America and most developed countries. In recent years the rate of CVD has declined in men but not in women. This is contributed to by an under-recognition of women’s C...

280

Alzheimer's Disease  

Microsoft Academic Search

The proliferation of information regarding Alzheimer's disease in current years has had a tremendous impact on the literature being generated on this topic. Until fairly recently, Alzheimer's disease was believed to be incurable, and its progress inexorable. In recent years research has come up with encouraging results which give hope that new treatments and even a cure is possible in

Carol Gillen

1993-01-01

281

Prion Diseases  

Technology Transfer Automated Retrieval System (TEKTRAN)

Prion diseases comprise a set of rare fatal neurological diseases found in humans and other mammals. A prion is a protein capable of converting a normal cellular protein (PrPC) into a prion and thereby propagating an infection. A prion and PrPC differ solely in their conformation. There are differen...

282

[Graves' disease].  

PubMed

Genetic and environmental determinants inducing Graves' disease are still poorly defined, especially those leading to the appearance of TSH receptor antibodies, which are both the hallmark of the disease and the keystone of its diagnosis. The treatment of Graves' disease is based on the use of antithyroid drugs and no therapeutic protocol has proven superior to the other. Surgical or radiometabolic treatments, largely restricted to relapses and to patients with comorbidities could be considered earlier provided we had reliable predictive markers for relapse. The treatment of Graves' orbitopathy relies first on a rigorous analysis of severity and activity of the disease. Intravenous steroids appear as a reference treatment in active forms. Evaluation of new therapeutics is in process. Graves' disease during pregnancy requires a multidisciplinary approach and an expert ultrasound evaluation of the fetus. Because of a suspected teratogenicity of carbimazole and methimazole, propylthiouracyl is the preferred drug used during early pregnancy. PMID:25090772

Illouz, Frédéric; Rodien, Patrice

2014-06-01

283

Crohn's disease.  

PubMed

Crohn's disease is a chronic inflammatory disease of the gastrointestinal tract and is an important cause of morbidity in children and adolescents. In India Crohn's disease (CD) was considered a rare disease, however, during the last 10 years CD in adults is being reported from several centers especially in Southern India. CD is characterized by transmural granulomatous inflammation involving any part of the gastrointestinal tract in a discontinuous manner. The peak incidence of Crohn's disease occurs during the adolescent and young adult years. The clinical presentation and complications are varied and several extraintestinal manifestations have been recognized. The understanding of the pathophysiology has opened new avenues in the management. The recognition of this problem in children and adolescents by pediatricians is necessary for proper diagnosis and management. PMID:16936369

Sathiyasekaran, Malathi; Shivbalan, So

2006-08-01

284

Chronic Obstructive Pulmonary Disease (COPD)  

MedlinePLUS

... lungs, but asthma is not included in the definition of COPD. It is not uncommon, however for ... most important treatment in smokers is to stop smoking. Medications are usually prescribed to widen the airways ( ...

285

Inflammatory Bowel Disease in Children of Middle Eastern Descent  

PubMed Central

Increasing rates of inflammatory bowel disease (IBD) are now seen in populations where it was once uncommon. The pattern of IBD in children of Middle Eastern descent in Australia has never been reported. This study aimed to investigate the burden of IBD in children of Middle Eastern descent at the Sydney Children's Hospital, Randwick (SCHR). The SCHR IBD database was used to identify patients of self-reported Middle Eastern ethnicity diagnosed between 1987 and 2011. Demographic, diagnosis, and management data was collected for all Middle Eastern children and an age and gender matched non-Middle Eastern IBD control group. Twenty-four patients of Middle Eastern descent were identified. Middle Eastern Crohn's disease patients had higher disease activity at diagnosis, higher use of thiopurines, and less restricted colonic disease than controls. Although there were limitations with this dataset, we estimated a higher prevalence of IBD in Middle Eastern children and they had a different disease phenotype and behavior compared to the control group, with less disease restricted to the colon and likely a more active disease course. PMID:24987422

Naidoo, Christina Mai Ying; Leach, Steven T.; Day, Andrew S.; Lemberg, Daniel A.

2014-01-01

286

A case of multiple sclerosis and celiac disease.  

PubMed

Objectives. Multiple sclerosis (MS) is an inflammatory autoimmune disorder of the central nervous system (CNS). Since a correlation between gluten intake and incidence of MS had been reported, the relationship of antigliadin antibodies and MS was debated. Case Report. We report the case of a 45-year-old female MS patient who is under interferon treatment. After seven years of monitoring, during her routine gastroenterological assessment, she was diagnosed with celiac disease. Conclusion. Beside the neurological manifestations that have been demonstrated in about 10% of celiac disease (CD) patients, white-matter abnormalities in brain MRI are uncommon and controversial. But in the literature, MS seems to be associated with CD as in our patient. We suggest that MS patients with gastroenterological complaints should undergo an assessment for CD. PMID:23365772

Batur-Caglayan, H Z; Irkec, C; Yildirim-Capraz, I; Atalay-Akyurek, N; Dumlu, S

2013-01-01

287

Rivastigmine in the treatment of hypersexuality in Alzheimer disease.  

PubMed

Inappropriate sexual behaviors (ISB) represent uncommon and often misdiagnosed clinical disorders among patients with Alzheimer disease. So far, no randomized clinical trials regarding the treatment of ISB in demented people have been conducted, but available data from case series and isolated case reports suggest the efficacy of selective serotonin reuptake inhibitors (SSRIs), antipsychotics, antiandrogens, and H2-receptor antagonists. Controversial data exist on the therapeutic influence of cholinesterase inhibitors on sexual disorders. In the present article, we describe the case of an Alzheimer disease patient presenting hypersexuality, successfully treated with rivastigmine. Thus, we perform a revision of the existing literature regarding the therapeutical effect of cholinesterase inhibitors in the treatment of ISB. PMID:22683665

Canevelli, Marco; Talarico, Giuseppina; Tosto, Giuseppe; Troili, Fernanda; Lenzi, Gian Luigi; Bruno, Giuseppe

2013-01-01

288

[Peyronie's disease].  

PubMed

Peyronie's disease is caused by collagen deposits in the tunica albuginea of the corpus cavernosum following microtrauma. Symptoms may include a combination of penile curvature, a palpable plaque, painful erections and erectile dysfunction. Peyronie's disease can have a major impact on the quality of life. In the course of the disease two phases can be discerned. In the first, active phase there is penile curvature with painful erections. The second, stable phase is characterised by painless curvature of the penis. Treatment in the active phase is conservative and supportive. Surgical treatment is useful only in the stable phase and may consist of penile plication surgery or penile graft surgery. PMID:25004781

Ruiter, Annebeth E C; Meuleman, Eric J H

2014-01-01

289

Pericardial diseases.  

PubMed

The pericardium provides an enclosed lubricated space for the beating heart and functions to fix the heart in the chest cavity relative to adjacent organs. Pericardial pathophysiology is often manifested in a spectrum of distinct cardiac and systemic disease states. The pericardial response to injury typically involves a spectrum of inflammation with both acute and chronic features and/or fluid accumulation. Recent advances in imaging methods have refined the diagnosis and therapy of pericardial disease. This article presents the anatomy and physiology of pericardial disease and the clinical approach for diagnosis and treatment. PMID:22289657

Dudzinski, David M; Mak, Gary S; Hung, Judy W

2012-03-01

290

Hirschsprung disease.  

PubMed

Hirschsprung disease is a relatively common condition managed by pediatric surgeons. Significant advances have been made in understanding its etiologies in the last decade, especially with the explosion of molecular genetic techniques and early diagnosis. The surgical management has progressed from a two- or three-stage procedure to a primary operation. More recently, definitive surgery for Hirschsprung disease through minimally invasive techniques has gained popularity. In neonates, the advancement of treatment strategies for Hirschsprung disease continues with reduced patient morbidity and improved outcomes. PMID:19019295

Haricharan, Ramanath N; Georgeson, Keith E

2008-11-01

291

Digestive diseases  

MedlinePLUS

... includes one or more of the following symptoms: Bleeding Bloating Constipation Diarrhea Heartburn Incontinence Nausea and vomiting Pain in the belly Swallowing problems Weight gain or loss A digestive disease is any health problem that occurs in ...

292

Kawasaki Disease  

MedlinePLUS

... Division of Intramural Research Research Resources Scientific Reports Technology Transfer Clinical Trials What Are Clinical Trials? Children & ... any type of blood vessel in the body, including the arteries, veins, and capillaries. Sometimes Kawasaki disease ...

293

Graves disease  

MedlinePLUS

... is called hyperthyroidism. (An underactive thyroid leads to hypothyroidism .) Graves disease is the most common cause of ... radioactive iodine usually will cause an underactive thyroid (hypothyroidism). Without getting the correct dosage of thyroid hormone ...

294

Chagas Disease  

MedlinePLUS

... that cause Chagas disease are in the bug’s feces. People will usually scratch the bite and when this happens, a small amount of the bug’s feces, along with the germs, enter the bloodstream. ? The ...

295

Alzheimer's Disease  

MedlinePLUS

... progressive brain disease that slowly destroys memory and thinking skills, and eventually even the ability to carry ... people. Dementia is the loss of cognitive functioning—thinking, remembering, and reasoning—and behavioral abilities, to such ...

296

Legionnaires' Disease  

MedlinePLUS

... with major differences in "attack rate" (the fraction of exposed persons who become infected) and severity are not known. In the United States, Legionnaires' disease is fairly common and serious. LDB ...

297

Canavan Disease  

MedlinePLUS

... known as axons vulnerable and unable to properly function. Canavan disease is caused by mutation in the gene for an enzyme called aspartoacylase, which acts to break down the concentrated brain chemical known as N-acetyl-aspartate. Symptoms of ...

298

Méničre's disease  

MedlinePLUS

... in one ear, but it may affect both ears. Hearing tends to improve between attacks but gets worse ... disease, or if symptoms get worse. These include hearing loss, ringing in the ears, or dizziness.

299

Gaucher Disease  

MedlinePLUS

... common of the inherited metabolic disorder known as lipid storage diseases. Lipids are fatty materials that include oils, fatty acids, ... research to find ways to treat and prevent lipid storage disorders. This research includes clinical studies by ...

300

Legionnaire disease  

MedlinePLUS

... or diabetes Weakened immune system, such as during cancer treatment or taking steroid medicines Long-term (chronic) lung disease, such as COPD Long-term use of a breathing machine (ventilator) ...

301

Dupuytren's Disease  

MedlinePLUS

Dupuytren’s disease is an abnormal thickening of the tissue just beneath the skin. This thickening occurs in ... Figure 2), which is a condition described as Dupuytren’s contracture. Although the skin may become involved in ...

302

Vascular Diseases  

MedlinePLUS

... and block blood flow to the heart or brain. Weakened blood vessels can burst, causing bleeding inside ... and not smoking can help vascular disease. Other treatments include medicines and surgery.

303

Parkinson's Disease  

MedlinePLUS

... Cognitive impairment Fatigue What are some of the environmental factors researchers believe may be associated with PD? ... PD or slow its progression. Stories from the Environmental Factor (the official newsletter of NIEHS) Parkinson’s disease ...

304

Diarrhoeal Disease  

MedlinePLUS

... of diarrhoeal disease can be prevented through safe drinking-water and adequate sanitation and hygiene. Globally, there are ... organisms. Infection is spread through contaminated food or drinking-water, or from person-to-person as a result ...

305

Zoonotic Diseases  

MedlinePLUS

... wildlife when we clear wooded land for new construction. Because of these interactions, it’s important to be ... Centers for Disease Control and Prevention 1600 Clifton Road Atlanta, GA 30329-4027, USA 800-CDC-INFO ( ...

306

Disease Resources  

Cancer.gov

Key Programs Disease Resources The ASCUS/LSIL Triage Study for Cervical Cancer (ALTS) Human Papillomavirus Cervical Cancer Screening NCI Bethesda System 2001 The Bethesda System Web Atlas National Cervical Cancer Coalition American Social

307

Graves' Disease  

MedlinePLUS

... Graves' disease can cause the following symptoms: Nervousness Insomnia Emotional swings Sweating Hand tremor Palpitations Unexplained weight ... medication such as propanolol (Inderal). For anxiety and insomnia, your doctor may prescribe diazepam (Valium), lorazepam (Ativan) ...

308

Menkes Disease  

MedlinePLUS

... Additional research is being performed by the Eunice Kennedy Shriver National Institute of Child Health and Human Development, in collaboration with the NINDS, that applies gene therapy approaches to Menkes disease. 3 1. Kaler, SG. The neurology of STPAT ...

309

Alzheimer disease  

MedlinePLUS

Senile dementia - Alzheimer type (SDAT); SDAT ... The exact cause of Alzheimer disease (AD) is not known. Research shows that certain changes in the brain lead to AD developing. You are more likely ...

310

Kawasaki Disease  

PubMed Central

Kawasaki disease is an acute febrile, systemic vasculitic syndrome of an unknown etiology that primarily occurs in children younger than five years of age. The principal presentations of Kawasaki disease include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develops in 15% to 25% of untreated children with the disease, which may later lead to myocardial infarction, sudden death, or ischemic heart disease. Treatment with intravenous gamma globulin (IVIG) is effective, but the mode of action is still unclear. The development of a diagnostic test, a more specific therapy, and ultimately the prevention of this potentially fatal illness in children are all dependent upon the continued advances in determining the etiopathogenesis of this fascinating disorder. PMID:17191303

2006-01-01

311

Lentil Diseases  

Microsoft Academic Search

Fungal diseases of lentils are the most important biological constraint to productivity. Ascochyta lentis (ascochyta blight) and Fusarium oxysporum f. sp. lentis (fusarium wilt) are the major fungal pathogens that can cause severe losses in most lentil growing regions of the world.\\u000a Fungal diseases such as botrytis grey mould (Botrytis fabae and B. cinerea), rust (Uromyces viciae-fabae), stemphylium blight (Stemphylium

Paul Taylor; Kurt Lindbeck; Weidong Chen; Rebecca Ford

312

Kidney Disease and Diabetes  

MedlinePLUS

... the chance of getting kidney disease. How are cardiovascular disease (CVD) and kidney disease related? Chronic kidney disease can lead to cardiovascular disease (CVD) . Conversely, CVD can lead to kidney disease, ...

313

Potential treatment of inflammatory bowel disease: a review of helminths therapy  

PubMed Central

An inflammatory bowel disease (IBD) is most common in highly industrialized Western countries but uncommon in less developed areas of the world where helminths are frequent. The hygiene hypothesis proposes that the recent increase in allergic and autoimmune diseases is due to modern highly hygienic life styles and medical conditions. Loss of routine exposure to parasitic helminths, as a result of increasing lifestyle-associated factors, may be one factor leading to the increased disease prevalence. In animal models and clinical trials of IBD, gastrointestinal nematodes colonization suppresses intestinal inflammation through multiple mechanisms including induction of innate and adaptive regulatory circuits. Studies using helminths like Trichuris suis or Necator americanus showed that these helminths are safe and may be effective therapeutic approaches for the control of IBD and other immune diseases. The aim of present review was to exploring the therapeutic use of helminths for the control of IBD. PMID:25436093

Taghipour, Niloofar; Aghdaei, Hamid Asadzadeh; Haghighi, Ali; Mossafa, Nariman; Tabaei, Seyyed Javad Seyyed

2014-01-01

314

Potential treatment of inflammatory bowel disease: a review of helminths therapy.  

PubMed

An inflammatory bowel disease (IBD) is most common in highly industrialized Western countries but uncommon in less developed areas of the world where helminths are frequent. The hygiene hypothesis proposes that the recent increase in allergic and autoimmune diseases is due to modern highly hygienic life styles and medical conditions. Loss of routine exposure to parasitic helminths, as a result of increasing lifestyle-associated factors, may be one factor leading to the increased disease prevalence. In animal models and clinical trials of IBD, gastrointestinal nematodes colonization suppresses intestinal inflammation through multiple mechanisms including induction of innate and adaptive regulatory circuits. Studies using helminths like Trichuris suis or Necator americanus showed that these helminths are safe and may be effective therapeutic approaches for the control of IBD and other immune diseases. The aim of present review was to exploring the therapeutic use of helminths for the control of IBD. PMID:25436093

Taghipour, Niloofar; Aghdaei, Hamid Asadzadeh; Haghighi, Ali; Mossafa, Nariman; Tabaei, Seyyed Javad Seyyed; Rostami-Nejad, Mohammad

2014-01-01

315

Diffuse Parenchymal Diseases Associated With Aluminum Use and Primary Aluminum Production  

PubMed Central

Aluminum use and primary aluminum production results in the generation of various particles, fumes, gases, and airborne materials with the potential for inducing a wide range of lung pathology. Nevertheless, the presence of diffuse parenchymal or interstitial lung disease related to these processes remains controversial. The relatively uncommon occurrence of interstitial lung diseases in aluminum-exposed workers—despite the extensive industrial use of aluminum—the potential for concurrent exposure to other fibrogenic fibers, and the previous use of inhaled aluminum powder for the prevention of silicosis without apparent adverse respiratory effects are some of the reasons for this continuing controversy. Specific aluminum-induced parenchymal diseases described in the literature, including existing evidence of interstitial lung diseases, associated with primary aluminum production are reviewed. PMID:24806728

2014-01-01

316

Adopting orphans: comprehensive genetic testing of Mendelian diseases of childhood by next-generation sequencing  

PubMed Central

Orphan diseases are individually uncommon but collectively contribute significantly to pediatric morbidity, mortality and healthcare costs. Current molecular testing for rare genetic disorders is often a lengthy and costly endeavor, and in many cases a molecular diagnosis is never achieved despite extensive testing. Diseases with locus heterogeneity or overlapping signs and symptoms are especially challenging owing to the number of potential targets. Consequently, there is immense need for scalable, economical, rapid, multiplexed diagnostic testing for rare Mendelian diseases. Recent advances in next-generation sequencing and bioinformatic technologies have the potential to change the standard of care for the diagnosis of rare genetic disorders. These advances will be reviewed in the setting of a recently developed test for 592 autosomal recessive and X-linked diseases. PMID:22022947

Kingsmore, Stephen F; Dinwiddie, Darrell L; Miller, Neil A; Soden, Sarah E; Saunders, Carol J

2011-01-01

317

Atypical radiological findings in achondroplasia with uncommon mutation of the fibroblast growth factor receptor-3 (FGFR-3) gene (Gly to Cys transition at codon 375)  

Microsoft Academic Search

The recent discovery of mutations in the FGFR-3 (fibroblast growth factor receptor-3) gene (FGFR3) as the cause of achondroplasia has provided new insight into understanding genetic diseases. It was surprising from the viewpoint of molecular genetics that most patients with achondroplasia showed the same mutation at nucleotide 1138, leading to a single amino acid substitution from glycine to arginine at

Gen Nishimuri; Yoshimitsu Fukushima; Hirofumi Ohashi; Shiro Ikegawa

1995-01-01

318

Neurodegenerative diseases ranging from Alzheimer disease and polyglutamine diseases to transmissible spongiform enceph-  

E-print Network

with Neurodegenerative Diseases The protein deposits found in Alzheimer disease, Huntington disease and prion diseaseNeurodegenerative diseases ranging from Alzheimer disease and polyglutamine diseases, we focus on three neuro- degenerative diseases, Alzheimer disease, Huntington disease and prion

Lindquist, Susan

319

Rheumatoid-like deformities in Parkinson’s disease with 1-year follow-up: case report and literature review  

Microsoft Academic Search

Rheumatoid-like deformities in joints are uncommon in patients with Parkinson’s disease and easy to be misdiagnosed with rheumatoid\\u000a arthritis. Therefore, unnecessary treatment is often initiated. Here, we report a case of a 60-year-old woman with Parkinson’s\\u000a disease developing a rheumatoid-like joint deformities, and evaluate 1-year follow-up outcome. We also review the literature\\u000a and discuss the clinical characteristics, possible pathogenesis, and

Xiaoming Shu; Guochun Wang; Xin Lu; Yao Xie

2010-01-01

320

[Vulvar diseases].  

PubMed

Vulvar diseases might cause problems in the differential diagnosis, because the clinical presentation of inflammatory, infectious and neoplastic disorders may be quite similar. Itching and pain as well as dysuria and dyspareunia are the most common symptoms of vulvar diseases. Inflammatory dermatoses like atopic and contact dermatitis, lichen planus, lichen sclerosus and atrophic vulvitis account for the majority of cases in specialized clinics. Furthermore, neoplastic conditions such as vulvar carcinoma, vulvar intraepithelial neoplasia (VIN) and vulvar Paget's disease have to be considered. Another frequent group are infections. Candida spp., herpes simplex viruses and human papilloma viruses are common pathogens. Additionally, vulvodynia is a typical problem that is sometimes difficult to treat. Because of the wide spectrum of clinical symptoms, cooperation with gynaecologists and psychosomatic specialists should be considered in difficult cases. The typical clinical presentations, their specific signs and symptoms, and pitfalls in the dermatological consultation are discussed. PMID:25475624

Peckruhn, M; Elsner, P

2015-01-01

321

Celiac disease.  

PubMed

On the basis of strong evidence, gastrointestinal symptoms and failure to thrive are classic presentations of celiac disease, but atypical, nongastrointestinal symptoms are also extremely common, particularly in the older child and adolescent. (3)(4)(8). On the basis of some research evidence and consensus, guidelines recommend celiac testing in symptomatic children with typical and atypical symptoms and consideration of testing in those with associated conditions and first-degree relatives of those with celiac disease. (3)(9). On the basis of strong research evidence, measurement of tTG IgA and total serum IgA level has been reported to be the most cost-effective and accurate means of serologic testing for celiac disease and is the test of choice unless the child is younger than 2 years or IgA deficient. (9). On the basis of strong research evidence, children with elevated titers of celiac antibodies or strong clinical suspicion for celiac disease should be referred to a gastroenterologist for upper endoscopy and biopsy. Until this procedure is performed, the child should continue on a diet with ingestion of gluten. (3)(9). On the basis of strong research evidence, all those with a confirmed diagnosis of celiac disease should follow a strict gluten-free diet for life, with avoidance of all foods that contain wheat, barley, and rye ingredients. (3)(4). Referral to a health care professional with specialized knowledge of celiac disease and the gluten-free diet is critical because of the numerous ways, often hidden, in which gluten may be present in the diet and environment. PMID:25274968

Ediger, Tracy R; Hill, Ivor D

2014-10-01

322

[Neurogastroenterology: focus on pediatric digestive motility diseases].  

PubMed

The enteric nervous system exercises a key role on the gastrointestinal tract (GIT) motility, sensibility, secretions and absorption. This "Little brain of the gut" consists of numerous autonomic neurones located in the GIT, influenced by luminal and intrinsic factors. A new science, the neurogastroenterology, explores the modulation of the GIT functions and the interactions between the central, autonomic and enteric nervous systems forming the brain-gut axis. It works to understand the role of the glial and Cajal's cells, of chemical mediators, hazards of the GIT ontology, influence of inflammation stress and early childhood environment. Motility disorders are congenital or acquired and can persist with more or less severe impairment of quality of life or be a life threatening condition. They are consequences of impaired embryonic development, genetic disorders, systemic diseases, toxic effects, normal or pathologic immunologic reactions acting on the nervous systems or the myocytes. Advances in the understanding of the pathogenesis of uncommon disorders (Hirschsprung disease, achalasia, chronic intestinal pseudo-obstruction) or more prevalent functional disorders (regurgitations, chronic constipation or diarrhoea, functional abdominal pain) contribute to improve the care of such patients. Multidisciplinary team is sometimes mandatory as a holistic approach and the use of sophisticated techniques are important. Improvement of the efficacy of the drugs could by obtained. For clinical works, we need a common language, for this purpose the paediatric Rome III classification of GIT functional disorders is proposed, we need also more consensus on paediatric GI motility exploration protocols. PMID:21818895

Scaillon, M

2006-01-01

323

Ureaplasma urealyticum serotypes in urinary tract disease.  

PubMed Central

Ureaplasma urealyticum cultures from 124 patients with urinary tract disease were serotyped by indirect immunofluorescence, using antisera to serotypes I to VIII. A similar range of serotypes was recovered from first-voided, midstream, and bladder-aspiration (SPA) urine, upper urinary tract samples, and vaginal swabs. Serotype VI was predominant (44/124) among the samples, whereas serotypes V (1/124 samples) and VII (0/124 samples) were uncommon. Twenty of 124 cultures contained more than one serotype, and three cultures were untypeable. Serotypes cultured from bladder urine were also present in vaginal and urethral samples, although these samples often carried additional serotypes. Consecutive SPA samples from the same patient invariably contained the same serotype, whereas some consecutive midstream urine samples showed a loss or gain of serotypes with time. One patient carried the same serotype in SPA urine over a period of 13 months. The pattern of serotypes recovered from the urinary tract was similar irrespective of the sampling site, the site of infection, the clinical diagnosis and renal function of the patient, and the presence or absence of other microorganisms. Colonization above the urethra and association with urinary tract disease appeared to be serotype independent. PMID:3700599

Hewish, M J; Birch, D F; Fairley, K F

1986-01-01

324

Behçet's disease  

PubMed Central

Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.???Keywords: Behçet's disease; oral ulcers; uveitis; immunosuppressants PMID:11009577

Kontogiannis, V; Powell, R

2000-01-01

325

Pilonidal Disease  

PubMed Central

Pilonidal disease presents many therapeutic challenges to surgeons throughout the world. Its varied clinical presentations necessitate a wide range of treatments, thus underscoring the need to tailor the treatment to the patient and the severity of disease. Recent studies confirm the efficacy of smaller, more conservative operations for appropriate indications. When flap closures are performed, every attempt should be directed to placing sutures off (lateral) to the midline gluteal cleft. Meticulous attention to the details of immediate and long-term postoperative care is paramount. PMID:22379405

Khanna, Amit; Rombeau, John L.

2011-01-01

326

Prion Diseases  

MedlinePLUS

... research on developing a better test for the proteins responsible for mad cow disease (Windows Media Player Format, SMIL captioned, 3 MB, Credit: ScienCentral, Inc.) How do I view captions in Windows Media Player? (PDF) Javascript Error Your browser JavaScript is turned off ...

327

Infectious Diseases  

NSDL National Science Digital Library

With the threat of a warmer, wetter world and a larger global population, scientists are researching how climate change may impact the spread of infectious diseases,ťsuch as cholera and dengue fever, and how outbreaks may be prevented.ť "Changing Planet" is produced in partnership with the National Science Foundation.

NBC Learn

2010-10-07

328

Addison's Disease  

MedlinePLUS

... just above your kidneys. They produce hormones that affect how your body responds to stress. In people who have Addison’s disease, the adrenal glands do not produce enough corticosteroid hormones, such as cortisol and ... can occur at any age and affects men and women equally. It is also called ...

329

Morbihan disease.  

PubMed

Morbihan disease, which consists of solid facial edema, is a rare complication of rosacea, a common cutaneous disorder in middle-aged individuals. The characteristic features of Morbihan disease are its chronic course, typical clinical picture, lack of specific laboratory and histopathologic findings, and refractoriness to therapeutic measures. Since its initial description in 1957, only a small number of cases have been reported in the dermatologic literature. We report a 54-year-old man who developed a two-year duration of erythema and edema that affects the upper and mid face, with accentuation in the periorbital region. Patch tests excluded an allergic contact dermatitis and histopathologic investigation showed small, nodular clusters of epithelioid cells in the dermis that were consistent with sarcoidal granulomata. A diagnosis of Morbihan disease was made owing to the combination of clinical and histopathologic findings. Therapeutic options for the disease remain unsatisfactory and treatments reported in the literature include systemic glucocorticoids, oral tetracyclines, thalidomide, isotretinoin, ketotifen, and clofazimine. Our patient failed a six-to-seven months course of minocycline prior to presentation and has since experienced improvement on gradually-increasing doses of isotretinoin. PMID:23286817

Hu, Stephanie W; Robinson, Maria; Meehan, Shane A; Cohen, David E

2012-12-01

330

Pompe Disease  

MedlinePLUS

... by the GAA into glucose, a sugar that fuels muscles. In Pompe disease, mutations in the GAA gene reduce or completely eliminate this essential enzyme. Excessive amounts of lysosomal glycogen accumulate everywhere in the body, but the cells of the heart and skeletal muscles are the ...

331

Lyme Disease  

MedlinePLUS

... Northern California. It has also been reported in China, Europe, Japan, Australia, and the parts of the ... Lyme disease can be treated and cured with one of several oral antibiotics for 3–4 weeks. The skin rash ... diagnosis or treatment provided by a qualified health care provider.

332

Lyme Disease  

MedlinePLUS

... Northern California. It has also been reported in China, Europe, Japan, Australia, and the parts of the ... Lyme disease can be treated and cured with one of several oral antibiotics for 3–4 weeks. The skin rash will ... of Skinsight's terms of service and privacy policy. The material on this site is for informational ...

333

Behçet's disease  

PubMed Central

Definition of the disease Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations. Epidemiology BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population. Clinical description The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations. Etiology The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated. Diagnostic methods Diagnosis is only based on clinical criteria. Differrential diagnosis It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn’s disease, Takayasu’s arteritis, polychondritis or antiphospholipid syndrome need to be considered. Management Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy. Prognosis The prognosis is severe due to the ocular, neurological and arterial involvement. PMID:22497990

2012-01-01

334

Lung Diseases  

MedlinePLUS

When you breathe, your lungs take in oxygen from the air and deliver it to the bloodstream. The cells in your body need oxygen to ... you breathe nearly 25,000 times. People with lung disease have difficulty breathing. Millions of people in ...

335

Winkler's disease.  

PubMed

Winkler's disease otherwise known as chondrodermatitis nodularis chronica helices (CNHC) is characterized by a painful persistent erythematous nodule, mostly located at the rim of helix of pinna. Occurs mostly in men over 40 years of age. Standard therapy is by local excision or carbon dioxide laser vaporization. Recurrence is frequent. PMID:23120207

Jacob K, Joe; Satheesh, S; Menon, P; Saju, K G

2005-10-01

336

Winkler's disease  

Microsoft Academic Search

Winkler's disease otherwise known as chondrodermatitis nodularis chronica helices (CNHC) is characterized by a painful persistent\\u000a erythematous nodule, mostly located at the rim of helix of pinna. Occurs mostly in men over 40 years of age. Standard therapy\\u000a is by local excision or carbon dioxide laser vaporization. Recurrence is frequent.

Joe Jacob. K; S. Satheesh; P. Menon; K. G. Saju

2005-01-01

337

Diverticular Disease  

MedlinePLUS

... or in the toilet. If you notice blood coming from your rectum, you should call your doctor immediately. ... both men and women and is most common in people older than 40 years of age. Diverticular disease may be caused by not eating ...

338

Leigh's Disease  

MedlinePLUS

... group of substances that are important for cell metabolism. This gene is only found on the X chromosome. Is there any treatment? The most common treatment for Leigh's disease is thiamine or Vitamin B1. Oral sodium bicarbonate or sodium citrate may ...

339

Histiocytic necrotising lymphadenitis (Kikuchi-Fujimoto disease) of axillary lymph nodes.  

PubMed

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rare entity, occurring most commonly in young Asian adults. KFD is characterised by fever with tender lymph node enlargement. The cervical group of lymph nodes is most commonly involved, and the diagnosis is conclusively made by lymph node biopsy and histopathology. KFD is a self-limiting condition, which usually resolves over 1-4?months. Symptomatic treatment with antipyretics and/or non-steroidal anti-inflammatory drugs is recommended. Here we describe an uncommon presentation of KFD in a young woman in which only the axillary lymph nodes were enlarged. PMID:25564582

Nagaraju, Santosh; Vaishnav, Sakshi; Burke, Leandra H; Norman, Earl M

2015-01-01

340

The Enigmatic Kikuchi-Fujimoto Disease: A Case Report and Review  

PubMed Central

We report this case of a 33-year-old African American woman who presented to the clinic with preauricular and submandibular masses that she had noticed 6 weeks earlier. She gave a remote history of noticing bilateral cervical masses 3 years prior to this presentation that had not been investigated at the time and resolved spontaneously. Excisional biopsies of the cervical lymph nodes showed morphologic and immunophenotypic findings suggestive of Kikuchi Fujimoto disease (KFD). KFD is an uncommon, self-limited, and perhaps an underdiagnosed entity with an excellent prognosis. It mimics malignant lymphoma in presentation and therefore an accurate clinicopathological differentiation is crucial. PMID:24649384

Gaduputi, Vinaya; Rafiq, Arsalan; Shenoy, Roopalekha

2014-01-01

341

Diabetic Heart Disease  

MedlinePLUS

... from the NHLBI on Twitter. What Is Diabetic Heart Disease? The term "diabetic heart disease" (DHD) refers to ... Kidney Diseases' Introduction to Diabetes Web page. What Heart Diseases Are Involved in Diabetic Heart Disease? DHD may ...

342

Coronary heart disease  

MedlinePLUS

Heart disease, Coronary heart disease, Coronary artery disease; Arteriosclerotic heart disease; CHD; CAD ... al. Effectiveness-Based Guidelines for the Prevention of Cardiovascular Disease in Women--2011 Update:a guideline from the ...

343

Acid Lipase Disease  

MedlinePLUS

NINDS Acid Lipase Disease Information Page Synonym(s): Cholesterol Ester Storage Disease, Wolman’s Disease Table of Contents (click to jump ... research is being done? Clinical Trials What is Acid Lipase Disease ? Acid lipase disease occurs when the ...

344

Understanding Pulmonary Vascular Disease  

MedlinePLUS

... ENews Home > Lung Disease > Pulmonary Vascular Disease Understanding Pulmonary Vascular Disease Pulmonary vascular disease is a category of disorders. ... in a person's pulmonary arteries gets dangerously high. Pulmonary Veno-occlusive Disease This is an extremely rare form of high ...

345

Lyme Disease Transmission  

MedlinePLUS

... Health Officials Veterinarians Tool kit Lyme disease quiz Lyme disease transmission The Lyme disease bacterium, Borrelia burgdorferi , is ... blacklegged ticks Are there other ways to get Lyme disease? There is no evidence that Lyme disease is ...

346

Fabry disease  

PubMed Central

Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal ?-galactosidase A activity. FD is pan-ethnic and the reported annual incidence of 1 in 100,000 may underestimate the true prevalence of the disease. Classically affected hemizygous males, with no residual ?-galactosidase A activity may display all the characteristic neurological (pain), cutaneous (angiokeratoma), renal (proteinuria, kidney failure), cardiovascular (cardiomyopathy, arrhythmia), cochleo-vestibular and cerebrovascular (transient ischemic attacks, strokes) signs of the disease while heterozygous females have symptoms ranging from very mild to severe. Deficient activity of lysosomal ?-galactosidase A results in progressive accumulation of globotriaosylceramide within lysosomes, believed to trigger a cascade of cellular events. Demonstration of marked ?-galactosidase A deficiency is the definitive method for the diagnosis of hemizygous males. Enzyme analysis may occasionnally help to detect heterozygotes but is often inconclusive due to random X-chromosomal inactivation so that molecular testing (genotyping) of females is mandatory. In childhood, other possible causes of pain such as rheumatoid arthritis and 'growing pains' must be ruled out. In adulthood, multiple sclerosis is sometimes considered. Prenatal diagnosis, available by determination of enzyme activity or DNA testing in chorionic villi or cultured amniotic cells is, for ethical reasons, only considered in male fetuses. Pre-implantation diagnosis is possible. The existence of atypical variants and the availability of a specific therapy singularly complicate genetic counseling. A disease-specific therapeutic option - enzyme replacement therapy using recombinant human ?-galactosidase A - has been recently introduced and its long term outcome is currently still being investigated. Conventional management consists of pain relief with analgesic drugs, nephroprotection (angiotensin converting enzyme inhibitors and angiotensin receptors blockers) and antiarrhythmic agents, whereas dialysis or renal transplantation are available for patients experiencing end-stage renal failure. With age, progressive damage to vital organ systems develops and at some point, organs may start to fail in functioning. End-stage renal disease and life-threatening cardiovascular or cerebrovascular complications limit life-expectancy of untreated males and females with reductions of 20 and 10 years, respectively, as compared to the general population. While there is increasing evidence that long-term enzyme therapy can halt disease progression, the importance of adjunctive therapies should be emphasized and the possibility of developing an oral therapy drives research forward into active site specific chaperones. PMID:21092187

2010-01-01

347

Errors and difficulties in the diagnosis of bladder cancer in the 19th century: about an uncommon case reported by Professor Claude-François Lallemand (1790-1854).  

PubMed

Before the official foundation of the specialty of urology in 1870 from Félix Guyon, its exercise was in the hands of general surgeons. One of the most distinguished surgeons interested in urology was Claude-François Lallemand, Professor of Surgery in Montpellier. Despite his enormous experience in the diagnosis and treatment of urinary tract diseases as well as the invention of various surgical instruments for the lower urinary system, Lallemand, however, did not avoid serious diagnostic errors because of the lack of diagnostic tools. Characteristically in the present article we present a serious diagnostic mistake in a patient with bladder cancer with fatal outcome. PMID:20414956

Karamanou, M; Tsoukalas, G; Papandreou, X; Androutsos, G

2010-01-01

348

[Perthes disease].  

PubMed

The Legg-Calve-Perthes disease is an idiopathic avascular necrosis of the hip during early childhood. It is characterized by different stages with the main risk of persisting hip deformation, dysfunction of the joint movement, and the potential for early osteoarthritis. For the evaluation of prognosis and therapy planning patients age and extent of the necrotic area of the epiphysis are important factors. For an early diagnosis and sufficient therapy all radiological efforts have to be performed. MR imaging is an ideal method for the assessment of osteonecrotic changes of the Morbus Perthes. Compared to plain radiography by MR imaging pathologic alterations can be detected earlier and with higher specificity. However, conventional radiograms have to be still used as basic imaging modality. Nowadays x-rays and MR imaging should be the main methods for the evaluation of children suffering from Perthes disease. PMID:12149903

Kramer, J; Hofmann, S; Scheurecker, A; Tschauner, C

2002-06-01

349

Thyroid disease  

SciTech Connect

Presenting a multidisciplinary approach to the diagnosis and treatment of thyroid disease, this volume provides a comprehensive picture of current thyroid medicine and surgery. The book integrates the perspectives of the many disciplines that deal with the clinical manifestations of thyroid disorders. Adding to the clinical usefulness of the book is the state-of-the-art coverage of many recent developments in thyroidology, including the use of highly sensitive two-site TSH immunoradionetric measurements to diagnose thyroid activity; thyroglobulin assays in thyroid cancer and other diseases; new diagnostic applications of MRI and CT; treatment with radionuclides and chemotherapy; new developments in thyroid immunology, pathology, and management of hyperthyroidism; suppressive treatment with thyroid hormone; and management of Graves' ophthalmopathy. The book also covers all aspects of thyroid surgery, including surgical treatment of hyperthyroidism; papillary, follicular, and other carcinomas; thyroidectomy; and prevention and management of complications.

Falk, S.

1990-01-01

350

Stargardt Disease  

Microsoft Academic Search

When the adenosine triphosphate (ATP)-binding cassette (ABC) transporter gene, ABCA4 (originally named ABCR), was cloned and characterized in 1997 as the causal gene for autosomal recessive Stargardt disease (arSTGD or STGD1) (1) it seemed as if just another missing link was added to the extensive table of genetic determinants of rare monogenic retinal\\u000a dystrophies. Now, 9 yr later, the ABCA4

Rando Allikmets

351

Menkes disease  

Microsoft Academic Search

Menkes disease (MD) is a lethal multisystemic disorder of copper metabolism. Progressive neurodegeneration and connective tissue disturbances, together with the peculiar ‘kinky’ hair are the main manifestations. MD is inherited as an X-linked recessive trait, and as expected the vast majority of patients are males. MD occurs due to mutations in the ATP7A gene and the vast majority of ATP7A

Zeynep Tümer; Lisbeth B Mřller

2010-01-01

352

Wilson's disease.  

PubMed

Wilson's disease (WD), an inborn error of copper (Cu) metabolism, is now one of the leading liver diseases in children in India. The clinical presentation can be extremely varied viz.,--all forms of acute and chronic liver disease, minimal to severe neurological disease, psychiatric problems, bony deformities, hemolytic anemia and endocrine manifestations. A high index of suspicion is necessary along with a judicious battery of investigations for diagnosis. Hepatic copper estimation is the most reliable test but is not easily available in India. Liver biopsy may not be possible because of bleeding problems and histological features are often not diagnostic of WD. In the absence of hepatic Cu, a low ceruloplasmin, high 24 hour urinary copper and presence of KF rings aid in making the diagnosis. The mainstay of initial therapy is Cu-chelators like D-Penicillamine, and Trientine for reduction in body copper to sub-toxic levels. Subsequent maintenance therapy is necessarily lifelong with D-Penicillamine, Trientine or Zinc. Children on therapy must be monitored regularly for response, side-effects, compliance and rehabilitation. Response to therapy may be unpredictable, but acute and early presentations like fulminant hepatic failures have a poor outcome. All siblings must be screened for WD as early diagnosis and treatment result in a good outcome. The identification of the WD gene on chromosome 13 has led to the possible use of molecular genetics (haplotype and mutational analyses) in the diagnosis of WD. Parent groups/associations must take active part in holistic management of WD. PMID:12420912

Pandit, Anand; Bavdekar, Ashish; Bhave, Sheila

2002-09-01

353

Beryllium disease  

SciTech Connect

After two workers at the nuclear weapons plant at Oak Ridge National Laboratory in Tennessee were diagnosed earlier this year with chronic beryllium disease (CBD), a rare and sometimes fatal scarring of the lungs, the Department of Energy ordered up a 4-year probe. Now, part of that probe has begun - tests conducted by the Oak Ridge Associated Universities' Center for Epidemiological Research measuring beryllium sensitivity in 3,000 people who've been exposed to the metal's dust since Manhattan Project managers opened the Y-12 plant at Oak Ridge in 1943. Currently, 119 Y-12 employees process beryllium, which has a number of industrial uses, including rocket heat shields and nuclear weapon and electrical components. The disease often takes 20 to 25 years to develop, and the stricken employees haven't worked with beryllium for years. There is no cure for CBD, estimated to strike 2% of people exposed to the metal. Anti-inflammatory steroids alleviate such symptoms as a dry cough, weight loss, and fatigue. Like other lung-fibrosis diseases that are linked to lung cancer, some people suspect CBD might cause some lung cancer. While difficult to diagnose, about 900 cases of CBD have been reported since a Beryllium Case Registry was established in 1952. The Department of Energy (DOE) estimates that about 10,000 DOE employees and 800,000 people in private industry have worked with beryllium.

Not Available

1991-12-20

354

Atypical radiological findings in achondroplasia with uncommon mutation of the fibroblast growth factor receptor-3 (FGFR-3) gene (Gly to Cys transition at codon 375)  

SciTech Connect

The recent discovery of mutations in the FGFR-3 (fibroblast growth factor receptor-3) gene (FGFR3) as the cause of achondroplasia has provided new insight into understanding genetic diseases. It was surprising from the viewpoint of molecular genetics that most patients with achondroplasia showed the same mutation at nucleotide 1138, leading to a single amino acid substitution from glycine to arginine at codon 380 (Gly380Arg). All 39 patients examined by two groups had the Gly380Arg; 38 patients and the other demonstrated a G to A and a G to C transition at nucleotide 1138, respectively. Subsequently another group disclosed a G to A transition at the same nucleotide 1138 in 21/23 patients of diverse ethnic origin, although mutations were not identified in two patients. To date, a total of 193 patients with the mutation of the G380Arg have been reported; a single patient with another mutation resulting in a substitution from glycine to cysteine at codon 375 (Gly375Cys) has been described. The presence of this common mutation is consistent with the clinical fact that achondroplastic individuals show less phenotypic variability than is unusual for autosomal dominant diseases. We encountered a Japanese boy with the Gly375Cys. His mother with achondroplasia has the same mutation. The molecular investigation of these patients was reported elsewhere. Here we report the clinical and radiological findings in this boy who demonstrated some atypical manifestations from those of typical achondroplasia. 8 refs., 1 fig.

Nishimuri, Gen [Dokkyo Univ. School of Medicine, Tochigi (Japan); Fukushima, Yoshimitsu; Ohashi, Hirofumi [Saitama Children`s Medical Center, Saitama (Japan); Ikegawa, Shiro [Tokyo Univ. (Japan)

1995-11-20

355

A child with a long-standing, intensely itching subcutaneous nodule on a thigh: an uncommon (?) reaction to commonly used vaccines  

PubMed Central

A 2-year-old girl presented with an intensely itching subcutaneous nodule on the front of a thigh. The nodule persisted for 10?months until it was excised. Subsequent investigation for malignancy and systemic disease showed no pathological findings. The diagnosis, persistent itching vaccination granuloma, was revealed by hazard almost 2?years after the onset of symptoms. Persistent itching subcutaneous nodules at the injection site for aluminium containing vaccines (mostly diphtheria-tetanus-pertussis combination vaccines for primary immunisation of infants) may appear with a long delay after the vaccination (months), cause prolonged itching (years) and are often associated with contact allergy to aluminium. The condition is poorly recognised in Health Care which may lead to prolonged symptoms and unnecessary investigations. PMID:23354861

Bergfors, Elisabet; Lundmark, Katarzyna; Nyström Kronander, Ulla

2013-01-01

356

Vestibular disease: diseases causing vestibular signs.  

PubMed

Having determined whether a patient has central or peripheral vestibular disease, clinicians must then determine what diseases are likely to result in such a presentation. This article describes the more common diseases causing vestibular disease in dogs and cats. Having formulated a list of potential causes of vestibular disease, clinicians should proceed through a systematic investigation to diagnose the underlying condition. A companion article describes the anatomy, physiology, and clinical signs associated with vestibular disease. PMID:22847321

Lowrie, Mark

2012-07-01

357

Actinomyces in Chronic Granulomatous Disease: An Emerging and Unanticipated Pathogen  

PubMed Central

Background Chronic granulomatous disease (CGD) is a rare inherited disease of the phagocyte NADPH oxidase system that causes defective production of toxic oxygen metabolites, impaired bacterial and fungal killing, and recurrent life-threatening infections, mostly by catalase-producing organisms. We report for the first time, to our knowledge, chronic infections with Actinomyces species in 10 patients with CGD. Actinomycosis is a chronic granulomatous condition that commonly manifests as cervicofacial, pulmonary, or abdominal disease, caused by slowly progressive infection with oral and gastrointestinal commensal Actinomyces species. Treatment of actinomycosis is usually simple in immunocompetent individuals, requiring long-term, high-dose intravenous penicillin, but is more complicated in those with CGD because of delayed diagnosis and an increased risk of chronic invasive or debilitating disease. Methods Actinomyces was identified by culture, staining, 16S ribosomal DNA polymerase chain reaction, and/ or a complement fixation test in 10 patients with CGD. Results All 10 patients presented with a history of fever and elevated inflammatory signs without evident focus. Diagnosis was delayed and clinical course severe and protracted despite high-dose intravenous antibiotic therapy and/or surgery. These results suggest an unrecognized and unanticipated susceptibility to weakly pathogenic Actinomyces species in patients with CGD because these are catalase-negative organisms previously thought to be nonpathogenic in CGD. Conclusions Actinomycosis should be vigorously sought and promptly treated in patients with CGD presenting with uncommon and prolonged clinical signs of infection. Actinomycosis is a catalase-negative infection important to consider in CGD. PMID:19874205

Reichenbach, Janine; Lopatin, Uri; Mahlaoui, Nizar; Beovic, Bojana; Siler, Ulrich; Zbinden, Reinhard; Seger, Reinhard A.; Galmiche, Louise; Brousse, Nicole; Kayal, Samer; Güngör, Tayfun; Blanche, Stéphane; Holland, Steven M.

2014-01-01

358

Demyelinating diseases  

PubMed Central

A diagnosis of demyelination carries important therapeutic and prognostic implications. In most cases the diagnosis is made clinically, and involvement of the histopathologist is largely confined to postmortem confirmation and clinicopathological correlation. However, every now and then, accurate diagnosis of the presence or cause of demyelination before death hinges on the histopathological assessment. Recognition of demyelination depends on an awareness of this as a diagnostic possibility, and on the use of appropriate tinctorial and immunohistochemical stains to identify myelin, axons and inflammatory cells. In biopsy specimens, the critical distinction is usually from ischaemic or neoplastic disease, and the types of demyelinating disease most likely to be encountered are multiple sclerosis, acute?disseminated encephalomyelitis, progressive multifocal leucoencephalopathy and extrapontine myelinolysis. Interpretation of the pathology has to be made in the context of the clinical, radiological and biochemical findings. Freezing of a small amount of fresh tissue allows for later virological studies, and electron microscopy is occasionally helpful for demonstration of viral particles. PMID:17071802

Love, S

2006-01-01

359

Comprehensive MRI diagnosis of sacral osteomyelitis and multiple muscle abscesses as a rare complication of fistulizing Crohn's disease.  

PubMed

Pelvic osteomyelitis is a very uncommon complication of Crohn's disease, usually clinically unsuspected in the setting of acute Crohn's disease relapses. The case of a 21-year old patient is reported, in whom ileo-cecal inflammatory disease was complicated by fistulization to the presacral space and sacral osteomyelitis, plus multiple abscesses involving the iliopsoas, posterior paravertebral and gluteal muscles. As confirmed by surgical and pathological findings, MRI provided comprehensive imaging diagnosis by demonstrating both the pathogenesis and the full extent of the complex, deep pelvic inflammatory process. Low back pain in patients with Crohn's disease should not be underestimated since its differential diagnosis includes serious and potentially life-threatening causes such as osteomyelitis, so prompt assessment with cross sectional imaging, particularly MRI, is necessary. PMID:21939924

Tonolini, Massimo; Ravelli, Anna; Campari, Alessandro; Bianco, Roberto

2011-10-01

360

Quetiapine effective in treatment of inappropriate sexual behavior of lewy body disease with predominant frontal lobe signs.  

PubMed

Dementia of Lewy body disease is the second most common degenerative cause of dementia after Alzheimer's disease, among all the dementias. The core features are a progressive dementia, fluctuations in cognitive functions, visual hallucinations, and spontaneous parkinsonism. Rapid eye movement sleep behavior disorder, severe neuroleptic sensitivity, and low dopamine transporter uptake in basal ganglia are other suggestive features. Behavioral abnormalities are commonly present in the form of aggressive behavior, irritability, and uninhibited behaviors. These are mostly seen in the advanced stages of dementia. However, inappropriate sexual behavior is uncommonly seen in such cases. Three types of inappropriate sexual behaviors commonly found in cases of dementia are sex talks, sexual acts, and implied sexual acts. Such inappropriate sexual behaviors have not been described adequately in dementia of Lewy body disease. We report inappropriate sexual behaviors in a case of dementia of Lewy body disease, which improved rapidly after treatment with quetiapine. PMID:19129547

Prakash, Ravi; Pathak, Amit; Munda, Sanjay; Bagati, Dhruv

2009-01-01

361

Complete response and prolonged disease-free survival in a patient with recurrent duodenal adenocarcinoma treated with bevacizumab plus FOLFOX6.  

PubMed

Small bowel adenocarcinoma is an uncommon gastrointestinal malignancy with limited data on effective chemotherapy in the adjuvant setting, as well as for advanced disease. We present a case report of a patient with recurrent duodenal adenocarcinoma after resection and adjuvant chemotherapy who experienced a complete response to bevacizumab with oxaliplatin and 5FU (FOLFOX) followed by bevacizumab/capecitabine maintenance therapy for 2 years. The patient continues to be disease-free 8 years after his recurrence. This case highlights the potential of vascular endothelial growth factor (VEGF) inhibitors to enhance chemotherapeutic regimens for advanced small bowel adenocarcinoma. PMID:24490045

Nagaraj, Gayathri; Zarbalian, Yousef; Flora, Karin; Tan, Benjamin R

2014-02-01

362

Digestive Diseases Materials  

MedlinePLUS

... for patients and health care professionals to help increase awareness of bowel control problems. Celiac Disease Awareness Campaign ... for patients and health care professionals to help increase awareness of celiac disease. Kidney Disease Kidney Diseases Information ...

363

Depression and Heart Disease  

MedlinePLUS

... see the NIMH booklet on Depression . What is heart disease? Heart disease refers to a number of illnesses ... and save your life. How are depression and heart disease linked? People with heart disease are more likely ...

364

Heart disease and women  

MedlinePLUS

... not consider heart disease a woman's disease.Yet cardiovascular disease is the leading killer of women over age ... al. Effectiveness-based guidelines for the prevention of cardiovascular disease in women--2011 update: A guideline from the ...

365

HIV and Cardiovascular Disease  

MedlinePLUS

... Select a Language: Fact Sheet 652 HIV and Cardiovascular Disease HIV AND CARDIOVASCULAR DISEASE WHY SHOULD PEOPLE WITH HIV CARE ABOUT CVD? ... OF CVD? WHAT ABOUT CHANGING MEDICATIONS? HIV AND CARDIOVASCULAR DISEASE Cardiovascular disease (CVD) includes a group of problems ...

366

Chronic Kidney Disease (CKD)  

MedlinePLUS

... www.kidneyfund.org > Kidney Disease > Chronic Kidney Disease Chronic Kidney Disease (CKD) An estimated 31 million people in the United States are living with chronic kidney disease (CKD). What is CKD? The term “chronic kidney ...

367

Creutzfeldt-Jakob disease  

MedlinePLUS

... rare) Classic CJD is not related to mad cow disease (bovine spongiform encephalitis ). However, variant CJD (vCJD) ... of the disease that is related to mad cow disease. The infection that causes the disease in ...

368

Creutzfeldt-Jakob Disease  

MedlinePLUS

... disease This prion disease is similar to mad cow disease that's been found in wild deer, elk ... from the CDC . Meat or other products from cattle infected with bovine spongiform encephalopathy ("mad cow disease") , ...

369

Lipid Storage Diseases  

MedlinePLUS

NINDS Lipid Storage Diseases Information Page Condensed from Lipid Storage Diseases Fact Sheet Table of Contents (click to jump ... Trials Organizations Additional resources from MedlinePlus What are Lipid Storage Diseases? Lipid storage diseases are a group ...

370

Polycystic Kidney Disease (PKD)  

MedlinePLUS

MENU Return to Web version Polycystic Kidney Disease Overview What is polycystic kidney disease? Polycystic kidney disease (PKD) is an inherited disease that affects the kidneys. Sacs of fluid (called ...

371

Alzheimer's Disease Antimicrobial Peptides  

E-print Network

Keywords Alzheimer's Disease Antimicrobial Peptides Proteomics Posttranslational Modifications collaborations. Current international collaborations focus on Alzheimer's disease (Tau and A), molecular mechanisms of cell aging (glycation, oxidation) and related diseases (diabetes, Alzheimer's disease) as well

SchĂĽler, Axel

372

About Alzheimer's Disease: Symptoms  

MedlinePLUS

... more about other early signs of Alzheimer's » Mild Alzheimer's disease As the disease progresses, memory loss worsens, and ... disease is often diagnosed at this stage. Moderate Alzheimer's disease In this stage, damage occurs in areas of ...

373

Rice Diseases Atlas.  

E-print Network

CONTENTS INTRODUCTION .......... . .. . ........ . SEEDLI NG DISEASES ... . ... . .. . ........ . FOLIAGE DISEASES ................... .. SHEATH AND STEM DISEASES .......... . DISEASES ATT ACKI NG THE KERNEL ..... . PHYSIOLOGICAL DiSORDERS... or middle porti of the leaves. Symptoms are most conspicuouso the flag leaf just before heading. Diseased plan have less vigor and height and produce small panicles. On severely diseased plants the flag Ie and sheath become twisted and hold the panic...

Walla, Walter

1977-01-01

374

Alzheimer's disease: strategies for disease modification  

Microsoft Academic Search

Alzheimer's disease is the largest unmet medical need in neurology. Current drugs improve symptoms, but do not have profound disease-modifying effects. However, in recent years, several approaches aimed at inhibiting disease progression have advanced to clinical trials. Among these, strategies targeting the production and clearance of the amyloid-? peptide — a cardinal feature of Alzheimer's disease that is thought to

Martin Citron

2010-01-01

375

Maintenance with rituximab is safe and not associated with severe or uncommon infections in patients with follicular lymphoma: results from the phase IIIb MAXIMA study.  

PubMed

Previous randomized trials have demonstrated that rituximab maintenance (R-maintenance) can prolong time to progressive disease in patients with follicular lymphoma (FL). The phase IIIb MAXIMA study (NCT00430352) was a large prospective evaluation of R-maintenance in a daily care setting. The primary objective was safety. Secondary objectives included progression-free survival, overall survival, time to next lymphoma treatment, and partial response (PR) to complete response/unconfirmed (CR/CRu) conversion rate. Patients (n = 545) with first-line or relapsed FL who responded to 8 cycles of rituximab-based induction received R-maintenance every 2 months for 2 years. At study entry, 380 patients had CR or CRu, and 165 had PR. The median age was 57.0 years. The most common non-hematologic adverse events (AEs, excluding infusion-related reactions) were cough (9.9 % of patients), fatigue (7.5 %), nasopharyngitis (7.1 %), back pain (6.5 %), diarrhea (6.9 %), arthralgia (6.0 %), headache and hypertension (5.2 % each), and pyrexia (5.1 %). The majority of AEs were grade 1 or 2. Grade 3, 4, and 5 infections occurred in 21 (3.9 %), 2 (0.4 %), and 1 (0.2 %) patient, respectively. Fifty-one hematologic AEs occurred in 6.6 % (n = 35) of patients. Grade 3/4 prolonged neutropenia and hypogammaglobulinemia occurred in 13 (2.4 %) and 5 (0.9 %) patients, respectively. All cases of prolonged neutropenia or hypogammaglobulinemia were manageable and resolved. Fast infusion did not alter the safety profile. Efficacy was comparable with results from previous trials. R-maintenance is safe in a daily care setting for patients with first-line or relapsed FL. PMID:24824768

Witzens-Harig, Mathias; Foá, Robin; Di Rocco, Alice; van Hazel, Guy; Chamone, Dalton F A; Rowe, Jacob M; Arcaini, Luca; Poddubnaya, Irina; Ho, Anthony D; Ivanova, Valentina; Vranovsky, Andrej; Thurley, Dan; Oertel, Stephan

2014-10-01

376

[Osler's disease].  

PubMed

Osler's disease, also known as hereditary hemorrhagic telangiectasia (HHT) and Osler-Weber-Rendu syndrome, is an autosomal dominant disorder leading to abnormal blood vessel formation in the skin, mucous membranes and often in organs, such as the lungs, liver and brain (arteriovenous malformations AVM). Various types are known. Patients may present with epistaxis. Teleangiectasia can be identified by visual inspection during physical examination of the skin or oral cavity or by endoscopy. Diagnosis is made after clinical examination and genetic testing based on the Curacao criteria. Modern imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI) have become more important as they can depict the AVMs. Pulmonary AVMs can be depicted in CT imaging even without the use of a contrast agent while other locations including the central nervous system (CNS) usually require administration of contrast agents. Knowledge of possible clinical manifestations in various organs, possible complications and typical radiological presentation is mandatory to enable adequate therapy of these patients. Interventional procedures are becoming increasingly more important in the treatment of HHT patients. PMID:24276214

Ahlhelm, F; Lieb, J; Schneider, G; Müller, U; Ulmer, S

2013-12-01

377

Fabry disease.  

PubMed

Fabry disease (FD) is an X-linked disorder caused by deficiency of the enzyme alpha-galactosidase A, required for the degradation of globotriaosylceramide. Accumulation of substrate occurs in multiple cell types resulting in a multi-system disorder, affecting both males and females. Clinical features include neuropathic pain and angiokeratoma, with subsequent development of proteinuria, renal failure, left ventricular hypertrophy, arrhythmias and stroke. Beyond palliative therapies for organ involvement and pain control, enzyme replacement therapy directed at the underlying metabolic defect became available in 2001-2003. Knowledge of the pathophysiology and clinical features of FD is vital for assessing the rationale and evidence of efficacy of therapies for FD and their limitations. Whilst ERT improves many of the symptoms of FD, its effect on the natural history of the disorder has yet to be fully demonstrated. Improved understanding of the appropriate use of adjunctive therapies and the development of new treatment strategies, including pharmacologic chaperone therapy and gene therapy, coupled with long term clinical outcome data on the effects of ERT are all key components of optimising treatment for FD. PMID:25345090

Thomas, Atison S; Hughes, Derralynn A

2014-09-01

378

[Pathological gambling and Parkinson disease].  

PubMed

Pathological gambling (PG) is defined by the DSM IV criteria as inappropriate, persistent, and maladaptive gambling behavior that has repercussions on family, personal, and professional life. It is classified as an impulse control disorder and is widely understood as a nonpharmacological addiction. PG has been reported as a complication in the treatment of Parkinson disease (PD). The prevalence of PG in PD has been reported to range between 1.7 and 7% compared to a prevalence of approximately 1% in the general population. Though there is no survey that indicates the prevalence of PG in Japanese PD patients, problematic gambling behaviors in PD are occasionally observed. In addition to PG other impulse control behaviors and punding (repetitive stereotyped behavior) are recognized as components of the dopamine dysregulation syndrome (DDS), which is characterized by the compulsive use of dopaminergic medications, including levodopa and subcutaneous apomorphine. Though PG can occur with DDS it often occurs in isolation. The vast majority of PG seen in PD is related to dopamine agonists (DA). With regard to the administration of oral DA, pramipexole may induce a higher degree of PG than other types of oral DA due to its disproportionate stimulation of dopamine D3 receptors. However, the differences between the observed effects of various classes of oral DA were insignificant. PG associated with levodopa mono-therapy is uncommon, but in the majority of cases levodopa is co-prescribed. Subthalamic nucleus deep brain stimulation (STN-DBS) was recently introduced to treat advanced PD. An improvement in PG symptoms following STN-DBS has been reported due to a marked decrease in dopaminergic medications. However, in some patients, PG developed following STN-DBS despite the significant reduction or discontinuation of DA. STN-DBS per se may be a potential initiator of PG. Younger age of PD onset, novelty seeking personality traits, history of alcohol abuse, and impulsivity traits were significant predictors of PG. Patients especially those with individual susceptibility to PG should be warned of the potential risks of PG before DA treatment is initiated. PMID:18807938

Fujimoto, Ken-Ichi

2008-09-01

379

Alzheimer disease: An interactome of many diseases  

PubMed Central

Alzheimer Disease (AD) is an outcome as well as source of many diseases. Alzheimer is linked with many other diseases like Diabetes type 2, cholesterolemia, hypertension and many more. But how each of these diseases affecting other is still unknown to scientific community. Signaling Pathways of one disease is interlinked with other disease. But to what extent healthy brain is affected when any signaling in human body is disturbed is the question that matters. There is a need of Pathway analysis, Protein-Protein interaction (PPI) and the conserved interactome study in AD and linked diseases. It will be helpful in finding the potent drug or vaccine target in conscious manner. In the present research the Protein-Protein interaction of all the proteins involved in Alzheimer Disease is analyzed using ViSANT and osprey tools and pathway analysis further reveals the significant genes/proteins linking AD with other diseases. PMID:24753659

Rao, Balaji S.; Gupta, Krishna Kant; Karanam, Pujitha; Peruri, Anusha

2014-01-01

380

The global burden of chronic respiratory disease in adults.  

PubMed

With an aging global population, chronic respiratory diseases are becoming a more prominent cause of death and disability. Age-standardised death rates from chronic obstructive pulmonary disease (COPD) are highest in low-income regions of the world, particularly South Asia and sub-Saharan Africa, although airflow obstruction is relatively uncommon in these areas. Airflow obstruction is, by contrast, more common in regions with a high prevalence of cigarette smoking. COPD mortality is much more closely related to the prevalence of a low forced vital capacity which is, in turn, associated with poverty. Mortality from asthma is less common than mortality from COPD, but it is also relatively more common in poorer areas, particularly Oceania, South and South-East Asia, the Middle East and Africa. Again this contrasts with the asthma prevalence among adults, which is highest in high-income regions. In high-income areas, mortality due to asthma, which is predominantly an adult problem, has fallen substantially in recent decades with the spread of new guidelines for treatment that emphasise the use of inhaled steroids to control the disease. Although mortality rates have been falling, the prevalence of atopy has been increasing between generations in Western Europe. Changes in the prevalence of wheeze among adults has been more varied and may have been influenced by the reduction in smoking and the increase in the use of inhaled steroids. PMID:25519785

Burney, P; Jarvis, D; Perez-Padilla, R

2015-01-01

381

Autoimmune thyroid disease in rheumatoid arthritis: a global perspective.  

PubMed

Objective. To determine the prevalence and impact of autoimmune thyroid disease (AITD) in patients with rheumatoid arthritis (RA). Methods. Eight-hundred patients were included. The association between AITD and RA was analyzed was analyzed by bivariate and multivariate analysis. In addition, a literature review was done focusing on geographical variations. Results. In our cohort the prevalence of AITD was 9.8% while the presence of antibodies was 37.8% for antithyroperoxidase enzyme (TPOAb) and 20.8% for antithyroglobulin protein (TgAb). The presence of type 2 diabetes, thrombosis, abnormal body mass index, and a high educational level was positively associated with AITD. The literature review disclosed a geographical variation of AITD in RA ranging from 0.5% to 27%. Autoantibody prevalence ranges from 6% to 31% for TgAb, 5% to 37% for TPOAb, and from 11.4% to 32% for the presence of either of the two. Conclusion. AITD is not uncommon in RA and should be systematically assessed since it is a risk factor for developing diabetes and cardiovascular disease. These results may help to further study the common mechanisms of autoimmune diseases, to improve patients' outcome, and to define public health policies. An international consensus to accurately diagnose AITD is warranted. PMID:23209899

Cárdenas Roldán, Jorge; Amaya-Amaya, Jenny; Castellanos-de la Hoz, Juan; Giraldo-Villamil, Juliana; Montoya-Ortiz, Gladys; Cruz-Tapias, Paola; Rojas-Villarraga, Adriana; Mantilla, Rubén D; Anaya, Juan-Manuel

2012-01-01

382

Autoimmune Thyroid Disease in Rheumatoid Arthritis: A Global Perspective  

PubMed Central

Objective. To determine the prevalence and impact of autoimmune thyroid disease (AITD) in patients with rheumatoid arthritis (RA). Methods. Eight-hundred patients were included. The association between AITD and RA was analyzed was analyzed by bivariate and multivariate analysis. In addition, a literature review was done focusing on geographical variations. Results. In our cohort the prevalence of AITD was 9.8% while the presence of antibodies was 37.8% for antithyroperoxidase enzyme (TPOAb) and 20.8% for antithyroglobulin protein (TgAb). The presence of type 2 diabetes, thrombosis, abnormal body mass index, and a high educational level was positively associated with AITD. The literature review disclosed a geographical variation of AITD in RA ranging from 0.5% to 27%. Autoantibody prevalence ranges from 6% to 31% for TgAb, 5% to 37% for TPOAb, and from 11.4% to 32% for the presence of either of the two. Conclusion. AITD is not uncommon in RA and should be systematically assessed since it is a risk factor for developing diabetes and cardiovascular disease. These results may help to further study the common mechanisms of autoimmune diseases, to improve patients' outcome, and to define public health policies. An international consensus to accurately diagnose AITD is warranted. PMID:23209899

Cárdenas Roldán, Jorge; Amaya-Amaya, Jenny; Castellanos-de la Hoz, Juan; Giraldo-Villamil, Juliana; Montoya-Ortiz, Gladys; Cruz-Tapias, Paola; Rojas-Villarraga, Adriana; Mantilla, Rubén D.; Anaya, Juan-Manuel

2012-01-01

383

Esophageal actinomycosis in a patient with end-stage renal disease.  

PubMed

Actinomycosis of esophagus is uncommon. Herpes simplex virus, cytomegalovirus, candidiasis, tuberculosis, and other fungal infections are the commonly reported infections in both immunocompromised and immunocompetent patients. We report a case of esophageal actinomycosis in an end-stage renal disease patient. A 28-year-old lady, known case of systemic lupus erythematosus, hepatitis B virus infection with end-stage renal disease on regular maintenance hemodialysis since 5 years presented with history of epigastric pain and odynophagia for 1 week. Her upper gastrointestinal endoscopic examination revealed extensive necrotic areas with membrane in the esophagus. Histopathology revealed actinomycotic colonies and bacterial clumps. She was treated with intravenous penicillin followed by oral ampicillin for 6 months. She showed marked clinical improvement, and repeat endoscopy showed healing of ulceration and no evidence of actinomycosis. PMID:24393413

Nagaraju, Shankar Prasad; Kirpalani, Dilip Ashok; Bhabhe, Aditya S; Prasad, Raghvendra; Shah, Hardik; Kirpalani, Ashok L

2014-04-01

384

Rosai-Dorfman Disease in Thoracic Spine: A Rare Case of Compression Fracture  

PubMed Central

Sinus histiocytosis with massive lymphadenopathy known as Rosai-Dorfman disease is characterized by painless bilateral cervical lymphadenopathy. Extranodal manifestations are uncommon and spinal involvement is rare. A 15-year-old man presented with intermittent midthoracic back pain only. He had no specific findings on neurologic examinations, hematologic and biochemical laboratory tests. Radiological examination of thoracic spine revealed collapse of T6 vertebrae with thoracic kyphosis and osteolytic lesion of T12 vertebra body. He underwent a removal of bone tumor, anterior reconstruction with mesh and pedicle screw fixation via posterior approach for pathologic confirmation and stabilization. Histopathologic study of the lesion revealed focal infiltration of large histiocytes showing emperipolesis. Immunochemistry stain of histiocytes was positive for CD68 and S-100 but negative for CD1a. This report presents a rare case and literature review of extranodal Rosai-dorfman disease in thoracic spine. PMID:25346769

Kim, Do Young; Park, Ji Hye; Shin, Dong Ah; Yi, Seung; Ha, Yoon; Yoon, Do Heum; Kim, Keung Nyun

2014-01-01

385

Rosai-dorfman disease in thoracic spine: a rare case of compression fracture.  

PubMed

Sinus histiocytosis with massive lymphadenopathy known as Rosai-Dorfman disease is characterized by painless bilateral cervical lymphadenopathy. Extranodal manifestations are uncommon and spinal involvement is rare. A 15-year-old man presented with intermittent midthoracic back pain only. He had no specific findings on neurologic examinations, hematologic and biochemical laboratory tests. Radiological examination of thoracic spine revealed collapse of T6 vertebrae with thoracic kyphosis and osteolytic lesion of T12 vertebra body. He underwent a removal of bone tumor, anterior reconstruction with mesh and pedicle screw fixation via posterior approach for pathologic confirmation and stabilization. Histopathologic study of the lesion revealed focal infiltration of large histiocytes showing emperipolesis. Immunochemistry stain of histiocytes was positive for CD68 and S-100 but negative for CD1a. This report presents a rare case and literature review of extranodal Rosai-dorfman disease in thoracic spine. PMID:25346769

Kim, Do Young; Park, Ji Hye; Shin, Dong Ah; Yi, Seung; Ha, Yoon; Yoon, Do Heum; Kim, Keung Nyun

2014-09-01

386

Spatial connectedness of plant species: potential links for apparent competition via plant diseases  

E-print Network

. Bouteloua curtipendula was uncommon but occurred in all environments, while Buchloe dactyloides and Bouteloua gracilis were uncommon and only occurred in upland sites. Co-occurrence of plant species

Garrett, Karen A.

387

30/07/2011 00:19The selfish gene: Darwinism is so self-referential now that it sheds light only on itself. | Uncommon Descent Page 1 of 7http://www.uncommondescent.com/darwinism/the-selfish-gene-darwinism-is-so-self-referential-now-that-it-sheds-light-onl  

E-print Network

30/07/2011 00:19The selfish gene: Darwinism is so self-referential now that it sheds light only on itself. | Uncommon Descent Page 1 of 7http://www.uncommondescent.com/darwinism/the-selfish-gene-darwinism Archive Advertisements #12;30/07/2011 00:19The selfish gene: Darwinism is so self-referential now

Gardner, Andy

388

Strategies for disease modification in Alzheimer's disease  

Microsoft Academic Search

Treating Alzheimer's disease (AD) is the biggest unmet medical need in neurology. Current drugs improve symptoms, but do not have profound disease-modifying effects. Three main classes of disease-modification approaches can be defined: one that is broadly neurotrophic or neuroprotective, one that targets specific aspects of AD pathology, and one that is based on epidemiological observation. This review discusses all three

Martin Citron

2004-01-01

389

Cardiovascular Disease and Diabetes  

MedlinePLUS

Cardiovascular Disease & Diabetes Updated:Jan 31,2013 The following statistics speak loud and clear that there is a strong correlation between cardiovascular disease (CVD) and diabetes. Heart diseases and stroke are ...

390

Heart Disease in Women  

MedlinePLUS

... page from the NHLBI on Twitter. How Does Heart Disease Affect Women? Espańol In the United States, 1 ... about coronary MVD and broken heart syndrome. Coronary Heart Disease CHD is a disease in which plaque (plak) ...

391

Sleep and Chronic Disease  

MedlinePLUS

... control in persons with Type 2 diabetes. 1 Cardiovascular Disease Persons with sleep apnea have been found to be at increased risk for a number of cardiovascular diseases. Notably, hypertension, stroke, coronary heart disease and irregular ...

392

Mad Cow Disease  

MedlinePLUS

... Anxiety Disorders Relaxation Exercises The Flu Vaccine Mad Cow Disease KidsHealth > Teens > Infections > Bacterial & Viral Infections > Mad ... are people to get it? What Is Mad Cow Disease? Mad cow disease is an incurable, fatal ...

393

Pregnancy and Rheumatic Disease  

MedlinePLUS

... some rheumatic diseases (SLE, APS, Sjögren’s and, particularly, scleroderma), also warrants mention. Because this severe disease frequently ... period—pregnancy isconsidered inadvisable. Other diseases such as scleroderma (in the absence of pulmonary hypertension or lung ...

394

Chronic kidney disease  

MedlinePLUS

Kidney failure - chronic; Renal failure - chronic; Chronic renal insufficiency; Chronic kidney failure; Chronic renal failure ... kidney disease. If it worsens to end-stage renal disease , and how ... Kidney failure is the last stage of chronic kidney disease. ...

395

Carotid Artery Disease  

MedlinePLUS

... cerebrovascular disease, stroke, transient ischemic attacks (TIA) Carotid artery disease is a form of disease that affects ... to the brain by the 2 large carotid arteries in the front of your neck and by ...

396

Peripheral Artery Disease  

MedlinePLUS

Peripheral Artery Disease • Overview Peripheral artery disease, or P.A.D., refers to arterial disease that occurs outside of the heart or brain. In P.A.D., the arteries that carry oxygenated blood throughout the body become ...

397

Gum Disease in Children  

MedlinePLUS

... types of gum disease in children. Types of periodontal diseases in children Chronic gingivitis is common in ... cause the teeth to become loose. Signs of periodontal disease Four basic signs will alert you to ...

398

American Lyme Disease Foundation  

MedlinePLUS

... A. Jacobs, M.D., Emeritus Professor, Division of Infectious Diseases, University of California - San Francisco [ Video ] Lyme Disease ... on line Find a Local Physician Knowledgeable About Infectious Diseases The best private organization-based site that can ...

399

Understanding Alzheimer's Disease  

MedlinePLUS

... Referral Center Alzheimer's Disease Education and Referral Center Alzheimer's Disease Education and Referral Center Home About Alzheimer’s ... National Alzheimer's Project Act (NAPA) About ADEAR Understanding Alzheimer's Disease: What You Need to Know Introduction Many ...

400

Interstitial Lung Disease  

MedlinePLUS

... MS Dept. of Medicine View full profile Interstitial Lung Disease (ILD): Overview Interstitial lung disease (ILD) is ... they may make informed decisions Learn more. Interstitial Lung Disease Program As a center specializing in the ...

401

Autoimmune liver disease panel  

MedlinePLUS

Liver disease test panel - autoimmune ... Autoimmune disorders are a possible cause of liver disease. The most common of these diseases are autoimmune hepatitis and primary biliary cirrhosis. This group of tests helps your health care provider ...

402

Sickle Cell Disease  

MedlinePLUS

... sickle cell disease? Sickle cell disease, also called sickle cell anemia, is a hereditary problem (which mean it runs ... there is no cure for sickle cell disease. Sickle cell anemia can cause: Swollen hands and feet Jaundice (the ...

403

About Alzheimer's Disease: Treatment  

MedlinePLUS

... being researched? What are clinical trials? How is Alzheimer's disease treated? Alzheimer's disease is complex, and it is unlikely that any ... better to one drug than another. See also: Alzheimer's Disease Medications Fact Sheet Are there treatments available for ...

404

Pelvic Inflammatory Disease (PID)  

MedlinePLUS

MENU Return to Web version Pelvic Inflammatory Disease Overview What is pelvic inflammatory disease (PID)? Pelvic inflammatory disease (PID) is an infection of the female reproductive organs (the uterus, ...

405

Pelvic Inflammatory Disease (PID)  

MedlinePLUS

... STD, and TB Prevention Division of STD Prevention Pelvic Inflammatory Disease (PID) - CDC Fact Sheet Untreated sexually transmitted diseases (STDs) can cause pelvic inflammatory disease (PID), a serious condition, in women. 1 in ...

406

Peripheral Vascular Disease  

MedlinePLUS

... Arterial blockage including peripheral artery disease or PAD Aortic aneurysms Buerger's Disease Raynaud's Phenomenon Disease of the veins ... blood to flow around, or "bypass," the blockage. Aortic Aneurysms An aneurysm is a balloon-like bulge in ...

407

Types of Periodontal Disease  

MedlinePLUS

Types of Periodontal Disease Gingivitis Chronic Periodontitis Aggressive Periodontitis Periodontitis Caused by Conditions of the Body Necrotizing Periodontal Diseases Periodontal disease can refer to any condition that affects the gums and ...

408

About Alzheimer's Disease: Alzheimer's Basics  

MedlinePLUS

... with Alzheimer's disease? What is dementia? What is Alzheimer's disease? Alzheimer’s disease is an irreversible, progressive brain disease ... devastating disease. What happens to the brain in Alzheimer's disease? Although we still don’t know how the ...

409

Atheroembolic renal disease  

MedlinePLUS

Renal disease - atheroembolic; Cholesterol embolization syndrome; Atheroemboli - renal; Atherosclerotic disease - renal ... disorder of the arteries. It occurs when fat, cholesterol, and other substances build up in the walls ...

410

Effect of impulse control disorders on disability and quality of life in Parkinson's disease patients.  

PubMed

Impulse control and related disorders (ICRD) are not uncommon in patients with idiopathic Parkinson's disease (PD). The present study aimed to investigate the effects of ICRD on quality of life (QoL) and disability in PD. From two movement disorder clinics in Sydney, Australia, 100 consecutive patients with PD were included in the trial. The Unified Parkinson's Disease Rating Scale (UPDRS), Mini Mental State Examination and the Parkinson's Disease Questionnaire-39 were used to measure disease severity, cognition and disease-specific QoL. The diagnosis of ICRD was based on face-to-face structured clinical interviews by three psychiatrists with experience in ICRD using the Expanded Structured Clinical Interview for the Diagnostic and Statistical Manual IV for Obsessive-Compulsive Disorder Related/Spectrum Disorders. ICRD were present in 15% of our patient population, and had a negative impact on QoL and Activity of Daily Living (ADL) scores. After adjusting for the presence of major depressive disorders and PD duration, the effect on emotional wellbeing remained statistically significant (p<0.004). Disease duration also correlated with worse QoL and ADL scores. Major depression disorders reduced QoL but not ADL. Patients with ICRD tended to suffer more from depression than those without ICRD. There were no statistically significant differences in age, sex, major depressive disorders, PD duration, total levodopa equivalent daily dose, use of dopamine agonists, or UPDRS motor score between patients with and without ICDR. PMID:24035421

Phu, Amy L; Xu, Zheyu; Brakoulias, Vlasios; Mahant, Neil; Fung, Victor S C; Moore, Gregory De; Martin, Andrew; Starcevic, Vladan; Krause, Martin

2014-01-01

411

Castleman's disease in the pelvic retroperitoneum: A case report and review of the Japanese literature  

PubMed Central

INTRODUCTION Castleman's disease is a fairly rare benign tumor of lymphoid origin. It can develop anywhere lymphoid tissue is found, but the expected origin is mediastinum and rarely pelvic retroperitoneum. PRESENTATION OF CASE A 22-year-old woman was admitted to our hospital for a mass in the pelvic retroperitoneum that was detected incidentally on an ultrasonography during a routine medical checkup with no signs of symptoms. After laboratory examination, ultrasonography, and magnetic resonance imaging (MRI), surgical resection was performed successfully through a lower midline incision. But the patient was needed transfusion because of massive bleeding. Postoperative histopathological diagnosis was hyaline-vascular type of Castleman's disease. The patient is leading an active social life without any signs of sequelae or recurrence. DISCUSSION Through the review of Japanese literature on Castleman's disease in the retroperitoneum, the characteristics of preoperative imaging findings are studied. Castleman's disease is easily misdiagnosed clinically because of its scarcity and no specific imaging findings. And the embolization via angiography should be considered in the hypervasular tumors such as in this case to prevent massive bleeding and transfusion. CONCLUSION Although Castleman's disease is uncommon, it should always be included in the differential diagnosis of pelvic retroperitoneal tumors. A better knowledge of this disease would help surgeon to avoid unnecessarily extensive resection and massive bleeding for transfusion when dealing with retroperitoneal tumors. PMID:23103628

Sato, Atsushi

2012-01-01

412

An uncommon case of inflammatory infiltration of the urinary bladder in the long-term process of the purulent inflammation of the cervix and vaginal fornix, complicated with vesicovaginal fistula of unknown etiology.  

PubMed

We shall discuss the case of a female patient, aged 64 years, who was suffering from long-term purulent inflammation of the vaginal fornix that later involved the vaginal stump. This inflammatory process spread to the bladder trigone and resulted in vesicovaginal fistula (VVF) formation together with a bilateral hydronephrosis that required the placement of a temporary percutaneous nephrostomy. A non-cicatrized inflammatory reaction occurred at the right-sided insertion of the nephrostomy, which has yet to be successfully treated despite intensive dermatological and surgical approaches that included skin grafting. In the course of five-year treatment we observed a gradual regression of the inflammatory infiltration of both the trigone of the bladder and the vagina as well as a gradual closing of the VVF. The extremely long-lasting and uncommon local inflammatory reactions in the vagina, bladder, and dermal layers mandated the application of conservative treatment. The possibility of difficulties and defective healing of tissues that could result from surgical correction of the VVF are discouraging for both the patient and medical staff. PMID:24579004

Starownik, Rados?aw; Michalak, Jerzy; Bar, Krzysztof; P?aza, Pawe?; Muc, Kamil; Rechberger, Tomasz

2013-01-01

413

The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome  

PubMed Central

Background Over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients. Discussion There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome (MAS), adult onset Still’s disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. Ferritin is known to be a pro-inflammatory mediator inducing expression of pro-inflammatory molecules, yet it has opposing actions as a pro-inflammatory and as an immunosuppressant. We propose that the exceptionally high ferritin levels observed in these uncommon clinical conditions are not just the product of the inflammation but rather may contribute to the development of a cytokine storm. Summary Here we review and compare four clinical conditions and the role of ferritin as an immunomodulator. We would like to propose including these four conditions under a common syndrome entity termed “Hyperferritinemic Syndrome”. PMID:23968282

2013-01-01

414

Perianal Crohn's disease  

Microsoft Academic Search

PURPOSE: This study relates our experience with local surgical management of perianal Crohn's disease. METHOD: Of 1,735 patients with Crohn's disease seen between 1980 and 1990, records of 66 patients (3.8 percent) with symptomatic perianal Crohn's disease treated by local operations were retrospectively reviewed to study outcome of local surgical intervention. RESULTS: All patients had intestinal disease that was limited

Yash P. Sangwan; David J. Schoetz; John J. Murray; Patricia L. Roberts; John A. Coller

1996-01-01

415

Kidney Disease of Diabetes  

E-print Network

Kidney Disease of Diabetes National Kidney and Urologic Diseases Information Clearinghouse is the final stage of chronic kidney disease (CKD). Diabetes is the most common cause of kidney failure, accounting for nearly 44 percent of new cases.1 Even when diabetes is controlled, the disease can lead to CKD

Baker, Chris I.

416

[Imaging of aortic disease].  

PubMed

Diseases of the aorta are imaged using different modalities according to the cause and clinical situation. Current imaging strategies for the clinically most pertinent aortic diseases are analysed. These disease entities may be differentiated into congenital, acquired and inflammatory diseases. Traumatic and non-traumatic aortic aneurysms and dissections are emphasised in context with endovascular treatment options and subsequent follow-up. PMID:17479238

Reimer, P; Vosshenrich, R; Landwehr, P; Storck, M

2007-05-01

417

Molecular Bioinformatics for Diseases  

E-print Network

. PNAS, 104: 8685 (2007). #12;Mendelian diseases E6V6 Sickle Cell Disease: Autosomal recessive disorder 4. Curr Opin Pediatr, 13: 22 (2001). Leads to sickle cell anemia Manifestation of disease vastly differentPSB 2009 Molecular Bioinformatics for Diseases Tutorial Atul Butte, Maricel Kann, Yves Lussier

Radivojac, Predrag

418

Current and emerging treatment options for Peyronie's disease.  

PubMed

Peyronie's disease (PD) is a condition of the penis, characterized by the presence of localized fibrotic plaque in the tunica albuginea. PD is not an uncommon disorder, with recent epidemiologic studies documenting a prevalence of 3-9% of adult men affected. The actual prevalence of PD may be even higher. It is often associated with penile pain, anatomical deformities in the erect penis, and difficulty with intromission. As the definitive pathophysiology of PD has not been completely elucidated, further basic research is required to make progress in the understanding of this enigmatic condition. Similarly, research on effective therapies is limited. Currently, nonsurgical treatments are used for those men who are in the acute stage of PD, whereas surgical options are reserved for men with established PD who cannot successfully penetrate. Intralesional treatments are growing in clinical popularity as a minimally invasive approach in the initial treatment of PD. A surgical approach should be considered when men with PD do not respond to conservative, medical, or minimally invasive therapies for approximately 1 year and cannot have satisfactory sexual intercourse. As scientific breakthroughs in the understanding of the mechanisms of this disease process evolve, novel treatments for the many men suffering with PD are anticipated. PMID:24400231

Gokce, Ahmet; Wang, Julie C; Powers, Mary K; Hellstrom, Wayne Jg

2013-01-01

419

Current and emerging treatment options for Peyronie’s disease  

PubMed Central

Peyronie’s disease (PD) is a condition of the penis, characterized by the presence of localized fibrotic plaque in the tunica albuginea. PD is not an uncommon disorder, with recent epidemiologic studies documenting a prevalence of 3–9% of adult men affected. The actual prevalence of PD may be even higher. It is often associated with penile pain, anatomical deformities in the erect penis, and difficulty with intromission. As the definitive pathophysiology of PD has not been completely elucidated, further basic research is required to make progress in the understanding of this enigmatic condition. Similarly, research on effective therapies is limited. Currently, nonsurgical treatments are used for those men who are in the acute stage of PD, whereas surgical options are reserved for men with established PD who cannot successfully penetrate. Intralesional treatments are growing in clinical popularity as a minimally invasive approach in the initial treatment of PD. A surgical approach should be considered when men with PD do not respond to conservative, medical, or minimally invasive therapies for approximately 1 year and cannot have satisfactory sexual intercourse. As scientific breakthroughs in the understanding of the mechanisms of this disease process evolve, novel treatments for the many men suffering with PD are anticipated. PMID:24400231

Gokce, Ahmet; Wang, Julie C; Powers, Mary K; Hellstrom, Wayne JG

2013-01-01

420

Connective tissue disease-associated pulmonary arterial hypertension  

PubMed Central

Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sclerosis to PAH-specific therapy appears to be worse than in other forms of PAH. Survival in systemic sclerosis-associated PAH is inferior to that observed in idiopathic PAH. Potential reasons for this include differences in age, the nature of the underlying pulmonary vasculopathy and the ability of the right ventricle to cope with increased afterload between patients with systemic sclerosis-associated PAH and idiopathic PAH, while coexisting cardiac and pulmonary disease is common in systemic sclerosis-associated PAH. Other forms of connective tissue-associated PAH have been less well studied, however PAH associated with systemic lupus erythematosus (SLE) has a better prognosis than systemic sclerosis-associated PAH and likely responds to immunosuppression.

Howard, Luke S.

2015-01-01

421

Motor neurone disease and multiple sclerosis among immigrants to Britain.  

PubMed Central

Hospital discharge rates for various immigrant groups resident in Greater London and the West Midlands were studied to compare the incidence of two diseases of the nervous system-motor neurone disease (MND) and multiple sclerosis (MS). For both these conditions immigrants from Europe, Ireland, America and from the old commonwealth countries of Canada, Australia, and New Zealand had discharge rates that were similar to those for people born in the United Kingdom. In contrast, immigrants from the new commonwealth countries of Asia, Africa, America (including the West Indies), and Europe (Gibraltar, Malta, and Gozo) had a greatly reduced risk of MS but a very similar risk of MND compared with those born in the United Kingdom. MS but not MND was rare among immigrants of Asian or African ethnic origin and uncommon among immigrants from the West Indies. Deaths attributed to MS and MND have also been studied. During a three-year period there were no deaths reported for MS among immigrants of Asian and African ethnic origin and 14 were expected. There were two deaths from MND among these immigrants and five were expected. Among the immigrants from the West Indies two deaths were attributed to MS and 13 were expected, and there were three deaths attributed to MND and four were expected. The contrast between MND and MS in hospital morbidity and mortality confirms that MS among immigrants to Britain is rare in those of Asian and African ethnic origin. PMID:588852

Dean, G; Brady, R; McLoughlin, H

1977-01-01

422

Uncommon regioselectivity in thiacalix[4]arene formylation.  

PubMed

To reveal the alternative ways for upper-rim thiacalixarene derivatization, the formylation reactions (Gross and/or Duff conditions) of the corresponding tetrapropoxythiacalix[4]arene immobilized in the 1,3-alternate conformation were systematically studied. Surprisingly, albeit using an excess of the formylation agent, only two formyl groups were introduced exclusively into the meta positions of thiacalixarene skeleton. Unexpected regioselectivity of these reactions opens the door for a unique substitution pattern in thiacalixarene chemistry. The formation of meta-substituted aldehydes is another illustration showing remarkably different reactivity of the thiacalix[4]arene system compared with that of a classical calyx[4]arene analogue. PMID:19476313

Kundrat, Ondrej; Cisarova, Ivana; Böhm, Stanislav; Pojarova, Michaela; Lhotak, Pavel

2009-06-19

423

Uncommon Teaching in an Unexpected Place  

ERIC Educational Resources Information Center

Continued physical well-being is important, especially within the aging teacher ranks. The story of one veteran exercise instructor emphasizes fitness and weaves best classroom practices throughout the narrative.

Shore, Rebecca

2014-01-01

424

Stomach in a parastomal hernia: uncommon presentation  

PubMed Central

Parastomal herniae are among the most common complications of stoma formation. The authors present an unusual case of a 41-year-old lady who presented with frequent early postprandial vomiting devoid of bile, upper abdominal distension and weight loss in the preceding 4 weeks losing weight for the last 4 weeks. Barium meal revealed herniation of the stomach through the gastric wall causing gastric outlet obstruction. Intraoperatively she was found to have a subcutaneous incarcerated parastomal hernia containing viable stomach and small bowel loops. The hernial contents were reduced back into the peritoneum and the abdominal wall defect repaired using the open sombrero repair technique. PMID:22605584

Bota, Emil; Shaikh, Irshad; Fernandes, Roland; Doughan, Sameer

2012-01-01

425

Uncommon Progression of an Extradural Spinal Meningioma  

PubMed Central

Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is still incomplete and they may be easily confused with malignant neoplasms, much more common in this location. We report a rare case of a purely extradural thoracic spine meningioma in a 70-year-old man, with an unusual progression. In addition we discuss the pathogenesis of these tumors and the potential pitfalls in differential diagnosis and review the relevant literature concerning their treatment and outcome. PMID:25243091

Boughamoura, Mohamed; Mahmoudi, Houda; Kilani, Mohamed; Hattab, Nejib

2014-01-01

426

Uncommon Etiology of Chest Pain: Pulmonary Sequestration  

PubMed Central

Chest pain is a common presenting symptom in the emergency department. After ruling out emergent causes, emergency physicians need to identify and manage less commonly encountered conditions. Pulmonary sequestration (PS) is a rare congenital condition involving pulmonary parenchyma. In PS, a portion of non-functional lung tissue receives systemic blood supply from an anomalous artery. While most individuals with PS present in early life with symptoms of difficulty feeding, cyanosis, and dyspnea, some present later with recurrent pneumonia, hemoptysis, or productive cough. In this report, we present a case of PS in an adult with acute onset pleuritic chest pain. PMID:24381687

Haider, Asghar; Hoonpongsimanont, Wirachin

2013-01-01

427

Uncommon Sense: Core Knowledge in the Classroom.  

ERIC Educational Resources Information Center

This book shares the author's experiences implementing the Core Knowledge Sequence in a small town, offering an understanding of why Core Knowledge has been such a positive experience for learners. As the book describes reasons for using the Core Knowledge Sequence, it substantiates them with authentic experiences from administrators, teachers,…

Mackley, Timm A.

428

Malignancy of parathyroid: An uncommon clinical entity  

PubMed Central

Parathyroid carcinoma is a very rare cause of hyperparathyroidism. The diagnosis is usually established on histopathological grounds of capsular and vascular invasion, but a potential clue to the diagnosis is also offered by the severity of clinical profile, abrupt onset of symptoms, and a high degree of hypercalcemia and raised serum parathyroid hormone (PTH). We report a case of an elderly female with a prolonged history of generalized weakness and bone pain along with bilateral renal calculi, classical bony lesions, and a high serum calcium and PTH level who underwent a right inferior parathyroidectomy considering a parathyroid adenoma as our diagnosis. However, the biopsy report was consistent with a parathyroid carcinoma, and so, she was further subjected to an ipsilateral hemithyroidectomy as a completion procedure. So, we would like to emphasize that its preferable to have a high index of suspicion for parathyroid carcinoma when these clues are present, than to miss the opportunity for surgical cure in the first go by failing to consider it in the differential diagnosis. PMID:23776914

Ali, Kamran; Sarangi, Rathindra; Dhawan, Shashi; Agarwal, Brij B.; Gupta, Manish K.

2013-01-01

429

Adolescent Girls' Zines: Uncommon Pages and Practices.  

ERIC Educational Resources Information Center

Stephen Duncombe has written one of the only book-length studies examining the phenomenon of "zines.""Note from Underground: Zines and the Politics of Alternative Culture" traces the historical rise in zine popularity beginning with fanzines of the 1930s, fueled in the 1970s by the punk movement, and reaching a height in the 1990s. In his…

Sinor, Jennifer

430

The Uncommon Education of Lucretia Mott  

ERIC Educational Resources Information Center

At a time when female education remained, at best, an afterthought, how were nineteenth-century women prepared to assume positions of responsibility in national organizations? An examination of the early life of Lucretia Mott, a spirited Quaker minister of that time, seems to suggest one set of answers. (Author/RK)

Pagliaro, Penny

1977-01-01

431

Ovarian pregnancy: uncommon mode of presentation.  

PubMed

Ovarian pregnancy is very rare, and its incidence is 1 in 3000 live births. In this condition, common risk factors for ectopic pregnancy not usually found. It usually occurs in fertile women and more commonly with in-situ intrauterine device (IUD). Preoperative diagnosis is always not possible although the patient commonly presents with abdomen-pelvic pain, per vaginal bleeding and hypovolemic shock. High degree of suspicion with estimation of serum beta HCG, transvaginal ultrasonography by an experienced sonologist and laparoscopy is required for confirming the diagnosis. Though, the usual treatment is surgery, it can be managed by medical methods only in hemodynamically stable patients. In this case report, we describe the unusual mode of clinical presentation in an elderly woman with ovarian pregnancy. PMID:25478415

Jena, Saubhagya Kumar; Kar, Gayatri; Samal, Soumya; Behera, Basanta Kumar

2014-10-01

432

Xiphoid syndrome: an uncommon occupational disorder.  

PubMed

We report a case of a 45-year-old man, complaining of swelling and pain in his epigastric region for the last 3 years. According to his medical history, he had undergone various investigations and treatments for gastro-oesophageal reflux, without relief. He had had a history of chronic repeated microtraumas to his sternum during 9 years of working as a carpenter, as a result of placing wood against his anterior chest wall and pushing the former into a plank cutting machine. On examination, a tender swelling was palpable as an immobile, hard mass showing minimal protrusion under the skin on the xiphoid process. He was diagnosed as having xiphoid syndrome. We prescribed anti-inflammatory medication and advised him to avoid pressure on his anterior chest wall, especially on the sternum, while cutting wood. At follow-up, the symptoms were relieved. Xiphoid syndrome may be seen in people performing hard physical work who incur sustained pressure or friction on their anterior chest wall. The case emphasizes the importance of the occupational history as well as clinical and radiological investigation of unusual conditions as mentioned above. PMID:24336479

Yapici Ugurlar, O; Ugurlar, M; Ozel, A; Erturk, S M

2014-01-01

433

Bilateral eosinophilic mastitis: An uncommon unheard entity.  

PubMed

We are reporting a case of bilateral eosinophilic mastitis which is rare and hardly heard. It is a mimicker of carcinoma breast both clinically & radiologically. A 30 years old non diabetic female presented with bilateral breast lumps with history of rhinitis off & on and peripheral eosinophilia. Mammography was suspicious while ultrasonography was diagnostic of bilateral mastitis. Aspiration cytology exhibited inflammatory lesion rich in eosinophils. Histopathology revealed the diagnosis of eosinophilic mastitis. Eosinophilic infiltration of the breast is a rare manifestation of tissue involvement in peripheral eosinophilia and bilateralism is even rarer. PMID:25171213

Singh, Aminder; Kaur, Pavneet; Sood, Neena; Puri, Harpreet; Garg, Bhavna

2015-01-01

434

Pott's Puffy Tumor: An Uncommon Clinical Entity  

PubMed Central

Although first described in 1760, Pott's puffy tumor is a lesser known clinical entity. Often seen as a complication of frontal sinusitis, Pott's is a frontal bone osteomyelitis with an associated subperiosteal abscess. Patients present with a fluctuant swelling of the scalp. The diagnosis is often confirmed with computed tomography (CT). Prompt surgical and medical treatments are the rule as there is the potential for significant morbidity if not quickly diagnosed and treated. Herein, we describe the presentation, diagnosis, and treatment of an 8-year-old female presenting with Pott's puffy tumor. PMID:23091765

Suwan, Phillip T.; Mogal, Suvarna; Chaudhary, Subhash

2012-01-01

435

Disease model: LAMP2 enlightens Danon disease  

Microsoft Academic Search

Danon disease (‘lysosomal glycogen storage disease with normal acid maltase’) is characterized by a cardiomyopathy, myopathy and variable mental retardation. Mutations in the coding sequence of the lysosomal-associated membrane protein 2 (LAMP-2) were shown to cause a LAMP-2 deficiency in patients with Danon disease. LAMP-2 deficient mice manifest a similar vacuolar cardioskeletal myopathy. In addition to the patient reports LAMP-2

Paul Saftig; Kurt von Figura; Yshitaka Tanaka; Renate Lüllmann-Rauch

2001-01-01

436

Imaging in chest disease  

Microsoft Academic Search

Imaging in chest disease may be performed:•to investigate symptoms (e.g. high temperature, cough)•to confirm or exclude suspected diagnoses (e.g. pulmonary embolic disease, cancer)•in the further investigation of known diseases (e.g. diffuse lung disease)•to monitor disease status (e.g. consolidation in pneumonia, pleural effusion in empyema, mediastinal lymphadenopathy in lung cancer).A combination of different imaging modalities may be used to aid diagnosis

Fergus V Gleeson

2003-01-01

437

Hypercalcemia in patients with known malignent disease.  

PubMed

In 82 patients, a preoperative diagnosis of primary hyperparathyroidism has been established by means of transfemoral neck vein catheterization and measurement of serum immunoreactive parathyroid hormone (iPTH). Twenty-five of these patients have had cancer in other parts of the body but with no evidence of recurrence or metastasis. One patient had carcinoma of the colon with metastases, and four were members of families with multiple endocrine adenomatosis (MEA, Types I and II). In six other hypercalcemic patients, high levels of iPTH were found also in the effluent blood from cancer sites other than the parathyroid gland, secondary to ectopic hormone production or pseudohyperparathyroidism. In addition, a high serum level of iPTH was found in the superior vena cava of a seventh patient who had carcinoma of the breast but no clinical or radiological signs of recurrence or metastasis with the exception of an enlarged liver. This iPTH finding was interpreted as being, probably, the result of parathyroid adenoma in either the neck or the mediastinum. At the time of operation, a transcervical mediastinal search was made. Four normal cervical parathyroid glands were found; three were removed. Hypercalcemia persisted after operation, and the patient died. At postmortem examination, microscopic study revealed that the disease had metastasized to lungs and hilar lymph nodes. There was massive metastasis in the liver; the liver contained a large amount of iPTH. The results of these investigations suggest that (1) venous catheterization of the neck veins and the effluent blood from extraparathyroid tumors aid in identifying and localizing iPTH production; (2) primary benign hyperparathyroidism is not uncommon in patients with cancer, and its co-existence must be recognized; (3) high serum iPTH level in the superior vena cava may be found in patients with metastatic or primary cancer of the thoracic cavity; and (4) hyperparathyroidism may be the first hint of a familial multiple endocrine syndrome. PMID:960005

Samaan, N A; Hickey, R C; Sethi, M R; Yang, K P; Wallace, S

1976-09-01

438

Pathology of patients with Chagas' disease and acquired immunodeficiency syndrome.  

PubMed

The main pathologic findings in 23 patients with acquired immunodeficiency syndrome (AIDS) and Chagas' disease are reviewed; five are from our own experience and 18 from the literature. The presence of Trypanosoma cruzi parasites and/or T. cruzi antibodies in blood and cerebrospinal fluid was recorded and computerized tomograms of the brain were evaluated. Twenty (87%) of the 23 subjects developed severe, multifocal or diffuse meningoencephalitis with necrosis and hemorrhage associated with numerous tissue parasites. The second most severely affected site was the heart. Seven (30.4%) of the 23 cases had myocarditis on pathologic examination. It was acute in four patients, chronic in two, and simultaneously acute and chronic in one. Acute myocarditis and meningoencephalitis are interpreted as being caused by relapses of chronic T. cruzi infections. An AIDS permissive role is suggested for these conditions since immunologic defense against T. cruzi is mediated mainly by T lymphocytes, whose CD4 subpopulation is depleted in patients with this disease. Consequently, AIDS is a factor that may favor the reactivation of T. cruzi infections. The lesions reported in the association of Chagas' disease with AIDS were compared with those reported from patients without AIDS having fatal, acute, vector-transmitted infections, contaminated blood transfusions, or accidental exposures in the laboratory. For the latter three, meningoencephalitis is uncommon. Only immunosuppressed cases of Chagas' disease have been described as having a pseudotumoral presentation that shows expanding lesions with a mass effect in the cranial cavity that causes intracranial hypertension and simulates neoplasms (tumors such as gliomas, lymphomas, metastases, etc.). PMID:8147485

Rocha, A; de Meneses, A C; da Silva, A M; Ferreira, M S; Nishioka, S A; Burgarelli, M K; Almeida, E; Turcato Júnior, G; Metze, K; Lopes, E R

1994-03-01

439

The A431E mutation in PSEN1 causing Familial Alzheimer’s Disease originating in Jalisco State, Mexico: an additional fifteen families  

PubMed Central

Nine families with autosomal dominant Alzheimer’s disease (AD), all of whom had the Ala431Glu substitution in the PSEN1 gene and came from Jalisco State in Mexico, have been previously reported. As they shared highly polymorphic flanking dinucleotide marker alleles, this strongly suggests that this mutation arose from a common founder. In the current letter, we expand this observation by describing an additional 15 independent families with the Ala431Glu substitution in the PSEN1 gene and conclude that this mutation is not an uncommon cause of early-onset autosomal dominant AD in persons of Mexican origin. PMID:16897084

Murrell, Jill; Ghetti, Bernardino; Cochran, Elizabeth; Macias-Islas, Miguel Angel; Medina, Luis; Varpetian, Arousiak; Cummings, Jeffrey L.; Mendez, Mario F.; Kawas, Claudia; Chui, Helena

2012-01-01

440

The A431E mutation in PSEN1 causing familial Alzheimer's disease originating in Jalisco State, Mexico: an additional fifteen families.  

PubMed

Nine families with autosomal dominant Alzheimer's disease (AD), all of whom had the Ala431Glu substitution in the PSEN1 gene and came from Jalisco State in Mexico, have been previously reported. As they shared highly polymorphic flanking dinucleotide marker alleles, this strongly suggests that this mutation arose from a common founder. In the current letter, we expand this observation by describing an additional 15 independent families with the Ala431Glu substitution in the PSEN1 gene and conclude that this mutation is not an uncommon cause of early-onset autosomal dominant AD in persons of Mexican origin. PMID:16897084

Murrell, Jill; Ghetti, Bernardino; Cochran, Elizabeth; Macias-Islas, Miguel Angel; Medina, Luis; Varpetian, Arousiak; Cummings, Jeffrey L; Mendez, Mario F; Kawas, Claudia; Chui, Helena; Ringman, John M

2006-11-01

441

Challenging complications of treatment – human herpes virus 6 encephalitis and pneumonitis in a patient undergoing autologous stem cell transplantation for relapsed Hodgkin's disease: a case report  

PubMed Central

Background Reactivation of human herpesvirus 6 (HHV-6) occurs frequently in patients after allogeneic stem cell transplantation and is associated with bone-marrow suppression, enteritis, pneumonitis, pericarditis and also encephalitis. After autologous stem cell transplantation or intensive polychemotherapy HHV-6 reactivation is rarely reported. Case report This case demonstrates a severe symptomatic HHV-6 infection with encephalitis and pneumonitis after autologous stem cell transplantation of a patient with relapsed Hodgkin's disease. Conclusion Careful diagnostic work up in patients with severe complications after autologous stem cell transplantation is mandatory to identify uncommon infections. PMID:19619326

Bommer, Martin; Pauls, Sandra; Greiner, Jochen

2009-01-01

442

Emerging and reemerging epidemic-prone diseases among settling nomadic pastoralists in Uganda.  

PubMed

Epidemic-prone diseases have traditionally been uncommon among nomadic pastoralists as mobility allows already dispersed populations to migrate away from epidemic threats. In the Karamoja region of Uganda, nomadic pastoralists are transitioning to an increasingly settled lifestyle due to cattle raiding and associated civil insecurity. In attempts to reduce conflict in the region, the Ugandan government has instituted disarmament campaigns and encouraged sedentism in place of mobility. In Karamoja, this transition to sedentism has contributed to the emergence and reemergence of epidemic-prone diseases such as cholera, hepatitis E, yellow fever, and meningococcal meningitis. The incidence of these diseases remains difficult to measure and several challenges exist to their control. Challenges to communicable disease surveillance and control among settling nomadic pastoralists are related to nomadic mobility, remote geography, vaccination and immunity, and poor sanitation and safe water access. In addition to improving gaps in infrastructure, attracting well-trained government health workers to Karamoja and similar areas with longstanding human resource limitations is critical to address the challenges to epidemic-prone disease surveillance and control among settling nomadic pastoralists. In conjunction with government health workers, community health teams provide a sustainable method by which public health programs can be improved in the austere environments inhabited by mobile and settling pastoralists. PMID:24784434

Cummings, Matthew J; Wamala, Joseph F; Komakech, Innocent; Malimbo, Mugagga; Lukwago, Luswa

2014-09-01

443

Spectrum of high-resolution computed tomography imaging in occupational lung disease  

PubMed Central

Damage to the lungs caused by dusts or fumes or noxious substances inhaled by workers in certain specific occupation is known as occupational lung disease. Recognition of occupational lung disease is especially important not only for the primary worker, but also because of the implications with regard to primary and secondary disease prevention in the exposed co-workers. Although many of the disorders can be detected on chest radiography, high-resolution computed tomography (HRCT) is superior in delineating the lung architecture and depicting pathology. The characteristic radiological features suggest the correct diagnosis in some, whereas a combination of clinical features, occupational history, and radiological findings is essential in establishing the diagnosis in others. In the presence of a history of exposure and consistent clinical features, the diagnosis of even an uncommon occupational lung disease can be suggested by the characteristic described HRCT findings. In this article, we briefly review the HRCT appearance of a wide spectrum of occupational lung diseases. PMID:24604929

Satija, Bhawna; Kumar, Sanyal; Ojha, Umesh Chandra; Gothi, Dipti

2013-01-01

444

Value of routine duodenal biopsy in diagnosing coeliac disease in patients with iron deficiency anaemia  

PubMed Central

Background: Iron deficiency anaemia (IDA) is a recognised feature of coeliac disease in adults and can be its only presentation. Objective: To determine the yield of routine distal duodenal biopsies in diagnosing coeliac disease in adult and elderly patients with IDA whose endoscopy revealed no upper gastrointestinal cause of iron deficiency. Study design: Prospective study in a teaching hospital endoscopy unit. Method: Altogether 504 consecutive patients with IDA, aged 16–80 years, attending for endoscopy were included in this study. At least two distal duodenal biopsies were taken if endoscopy revealed no cause of iron deficiency. Result: In nine (1.8%) patients duodenal biopsies revealed typical histological features of coeliac disease. Of these, five patients were above 65 years old. Conclusion: In adult and elderly patients undergoing endoscopy for IDA, the endoscopist should take distal duodenal biopsies to exclude coeliac disease if no upper gastrointestinal cause of anaemia is found. Coeliac disease is not an uncommon cause of IDA in patients >65 years of age and a history of chronic diarrhoea increases diagnostic yield in this age group. PMID:15299158

Mandal, A; Mehdi, I; Munshi, S; Lo, T

2004-01-01

445

Cardiovascular Diseases (and Oral Health)  

MedlinePLUS

Cardiovascular Diseases Periodontal Disease and Cardiovascular Conditions Heart Disease and Dental Treatment Myocardial Infarction (Heart Attack) High Blood ... it is a current list. Periodontal Disease and Cardiovascular Conditions Periodontal disease can affect your overall health. Over time, it ...

446

Understanding Byssinosis (Brown Lung Disease)  

MedlinePLUS

... Disparities Reports Lung Disease Finder Lung Disease List Lung HelpLine Questions about your lung health? Need help ... ENews Home > Lung Disease > Byssinosis Understanding Byssinosis (Brown Lung Disease) What is Byssinosis? Byssinosis (brown lung disease) ...

447

Obesity and cardiovascular disease.  

PubMed

Cardiovascular disease is the most common cause of mortality in rich countries and today it has the same meaning for health care as the epidemics of past centuries had for medicine in earlier times: 50% of the population in these countries die of cardiovascular disease. The amount of cardiovascular disease is also increasing in the developing countries together with economic growth. By 2015 one in three deaths will globally be due to cardiovascular diseases. Coronary heart disease is a chronic disease that starts in childhood, even if the symptoms first occur in the middle age. The risks for coronary heart disease are well-known: lipid disorders, especially high serum LDL-cholesterol concentration, high blood pressure, tobacco smoking, obesity, diabetes, male gender and physical inactivity. Obesity is both an independent risk factor for cardiovascular disease but is also closely connected with several other risk factors. This review focuses on the connection between overweight or obesity and cardiovascular disease. PMID:25387321

Jokinen, E

2015-03-01

448

Formation and anti-tumor activity of uncommon in vitro and in vivo metabolites of CPI-613, a novel anti-tumor compound that selectively alters tumor energy metabolism.  

PubMed

CPI-613 is a novel anti-tumor compound with a mechanism-of-action which appears distinct from the current classes of anti-cancer agents used in the clinic. CPI-613 demonstrates both in vitro and in vivo anti-tumor activity. In vitro metabolic studies using liver S9 were performed which demonstrated that CPI-613 undergoes both phase 1 (oxidation) and phase 2 (glucuronidation) transformations. Its metabolic half-life varied between species and ranged from 8 minutes (Hanford minipig) to 47 minutes (CD-1 mouse). We performed metabolite mass assessments using selected in vitro incubation samples and demonstrated that +16 amu oxidation with and without +176 amu glucuronidation products were generated by human and animal liver S9. LC/MS/MS fragmentation patterns showed that an uncommon sulfoxide metabolite was formed and the O-glucuronidation occurred at the terminal carboxyl moiety. We observed that the +192 amu sulfoxide/glucuronide was generated only in human liver S9 and not by any of the other species tested. Synthetic metabolites were prepared and compared with the enzymatically-generated metabolites. Both the chromatographic retention times and the LC/MS/MS fragmentation patterns were similar, demonstrating that the synthetic metabolites were virtually identical to the S9-generated products. CYP450 reaction phenotyping and inhibition data both suggested that multiple CYP isozymes (2C8 and 3A4, along with minor contributions by 2C9 and 2C19) were involved in CPI-613 metabolism and sulfoxide formation. Plasma samples from human subjects dosed with CPI-613 also contained the sulfoxide ± glucuronide metabolites. These results show that the in vitro- and in vivo-generated phase 1 and phase 2 metabolites were in good agreement. PMID:21722089

Lee, King C; Shorr, Robert; Rodriguez, Robert; Maturo, Claudia; Boteju, Lakmal W; Sheldon, Adrian

2011-08-01

449

Prion diseases as transmissible zoonotic diseases.  

PubMed

Prion diseases, also called transmissible spongiform encephalopathies (TSEs), lead to neurological dysfunction in animals and are fatal. Infectious prion proteins are causative agents of many mammalian TSEs, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (BSE; in cattle), and Creutzfeldt-Jakob disease (CJD; in humans). BSE, better known as mad cow disease, is among the many recently discovered zoonotic diseases. BSE cases were first reported in the United Kingdom in 1986. Variant CJD (vCJD) is a disease that was first detected in 1996, which affects humans and is linked to the BSE epidemic in cattle. vCJD is presumed to be caused by consumption of contaminated meat and other food products derived from affected cattle. The BSE epidemic peaked in 1992 and decreased thereafter; this decline is continuing sharply owing to intensive surveillance and screening programs in the Western world. However, there are still new outbreaks and/or progression of prion diseases, including atypical BSE, and iatrogenic CJD and vCJD via organ transplantation and blood transfusion. This paper summarizes studies on prions, particularly on prion molecular mechanisms, BSE, vCJD, and diagnostic procedures. Risk perception and communication policies of the European Union for the prevention of prion diseases are also addressed to provide recommendations for appropriate government policies in Korea. PMID:24159531

Lee, Jeongmin; Kim, Su Yeon; Hwang, Kyu Jam; Ju, Young Ran; Woo, Hee-Jong

2013-02-01

450

Coronary Artery Disease - Coronary Heart Disease  

MedlinePLUS

... sudden cardiac death in patients with coronary heart disease. Cigarette smoking also acts with other risk factors to ... but their risk isn't as great as cigarette smokers'. Exposure to other people's smoke increases the risk of heart disease even for nonsmokers. Learn about smoking and cardiovascular ...

451

Prion Diseases as Transmissible Zoonotic Diseases  

PubMed Central

Prion diseases, also called transmissible spongiform encephalopathies (TSEs), lead to neurological dysfunction in animals and are fatal. Infectious prion proteins are causative agents of many mammalian TSEs, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (BSE; in cattle), and Creutzfeldt–Jakob disease (CJD; in humans). BSE, better known as mad cow disease, is among the many recently discovered zoonotic diseases. BSE cases were first reported in the United Kingdom in 1986. Variant CJD (vCJD) is a disease that was first detected in 1996, which affects humans and is linked to the BSE epidemic in cattle. vCJD is presumed to be caused by consumption of contaminated meat and other food products derived from affected cattle. The BSE epidemic peaked in 1992 and decreased thereafter; this decline is continuing sharply owing to intensive surveillance and screening programs in the Western world. However, there are still new outbreaks and/or progression of prion diseases, including atypical BSE, and iatrogenic CJD and vCJD via organ transplantation and blood transfusion. This paper summarizes studies on prions, particularly on prion molecular mechanisms, BSE, vCJD, and diagnostic procedures. Risk perception and communication policies of the European Union for the prevention of prion diseases are also addressed to provide recommendations for appropriate government policies in Korea. PMID:24159531

Lee, Jeongmin; Kim, Su Yeon; Hwang, Kyu Jam; Ju, Young Ran; Woo, Hee-Jong

2013-01-01

452

Metabolic Bone Disease in Chronic Kidney Disease  

Microsoft Academic Search

Metabolic bone disease is a common complication of chronic kidney disease (CKD) and is part of a broad spectrum of disorders of mineral metabolism that occur in this clinical setting and result in both skeletal and extraskeletal consequences. Detailed research in that past 4 decades has uncovered many of the mechanisms that are involved in the initiation and maintenance of

Kevin J. Martin; Esther A. Gonzalez

2007-01-01

453

Working Memory in Mild Alzheimer's Disease and Early Parkinson's Disease  

E-print Network

Working Memory in Mild Alzheimer's Disease and Early Parkinson's Disease Elizabeth A. Kensinger of Technology Alzheimer's disease (AD) and Parkinson's disease (PD) impair working memory (WM). It is unclear an expanding interest in how neurological diseases such as Alzheimer's disease (AD) and Parkinson's disease (PD

Corkin, Suzanne

454

Pelvic Inflammatory Disease  

MedlinePLUS

... disease can lead to serious, long-term problems: • Infertility —One in ten women with PID becomes infertile. ... lead to pelvic inflammatory disease, infertility, and arthritis. Infertility: A condition in which a couple has been ...

455

Chronic obstructive pulmonary disease  

MedlinePLUS

COPD; Chronic obstructive airways disease; Chronic obstructive lung disease; Chronic bronchitis; Emphysema; Bronchitis - chronic ... Smoking is the main cause of COPD. The more a person smokes, the ... develop COPD. But some people smoke for years and never get ...

456

Diabetes and Celiac Disease  

MedlinePLUS

... in every 133 people in the United States. Diabetes and celiac disease: The link • There is a ... blood sugar control. Signs of a malabsorption problem Diabetes Celiac Disease Weight Loss X X GI symptoms ( ...

457

Pregnancy and Fifth Disease  

MedlinePLUS

... Cheek Rash Parvovirus B19 and Other Illnesses References Pregnancy and Fifth Disease Share Compartir On this Page ... with fifth disease. Testing for Parvovirus B19 During Pregnancy A blood test for parvovirus B19 can show ...

458

Menopause and Heart Disease  

MedlinePLUS

... the onset of menopause. Menopause does not cause cardiovascular diseases . However, certain risk factors increase around the time ... t the only reason women face a higher cardiovascular disease risk after reaching menopause, Dr. Goldberg said. “We’ ...

459

Understanding cardiovascular disease  

MedlinePLUS

Cardiovascular disease is the broad term for problems with the heart and blood vessels. These problems are often ... and tissue. High blood pressure (hypertension) is a cardiovascular disease that can lead to other problems, such as ...

460

Office of Rare Diseases  

MedlinePLUS

... Read more... NCATS Expands Rare Diseases Clinical Research Network NIH funds 22 consortia and a data center ... NIH names new clinical sites in Undiagnosed Diseases Network Four-year, $43 million initiative engages broad expertise ...

461

Reproductive Diseases in Cattle  

E-print Network

This publication describes prevention measures, symptoms, diagnosis and treatment options for the most common reproductive diseases in cattle: brucellosis (Bang's disease); leptospirosis; infectious bovine rhinotracheitis (IBR) and bovine diarrhea...

Sprott, L. R.; Field, Bob

1998-12-03

462

Kidney and Urologic Diseases  

MedlinePLUS

... Kidney Failure: Hemodialysis ) Autosomal Dominant Polycystic Kidney Disease (Yesterday, Today & Tomorrow: NIH Research Timelines) (from the National ... care professionals) Chronic Kidney Disease and Kidney Failure (Yesterday, Today & Tomorrow: NIH Research Timelines) (from the National ...

463

Pelvic Inflammatory Disease (PID)  

MedlinePLUS

... Disease Control and Prevention 1600 Clifton Rd Atlanta, GA 30329-4027 800-CDC-INFO (800-232-4636) ... Disease Control and Prevention 1600 Clifton Rd. Atlanta, GA 30329-4027, USA 800-CDC-INFO (800-232- ...

464

Undifferentiated Connective Tissue Disease  

MedlinePLUS

... vessels. Examples of connective tissue diseases include lupus , scleroderma , rheumatoid arthritis , Sjögren's syndrome , myositis , and vasculitis . There ... connective tissue diseases, such as lupus, Sjögren's or scleroderma. More UCTD Information Causes Diagnosis Symptoms Treatment Print ...

465

Hemochromatosis: Iron Storage Disease  

MedlinePLUS

... this? Submit What's this? Submit Button CDC Features Hemochromatosis: Iron Storage Disease Language: English Espańol (Spanish) Share ... iron storage disease, and stay healthy. What Is Hemochromatosis? Hemochromatosis occurs when the body absorbs too much ...

466

Polycystic kidney disease  

MedlinePLUS

Cysts - kidneys; Kidney - polycystic; Autosomal dominant polycystic kidney disease; ADPKD ... Polycystic kidney disease (PKD) is passed down through families (inherited), usually as an autosomal dominant trait. If one parent ...

467

Niemann-Pick Disease  

MedlinePLUS

... a group of inherited metabolic disorders known as lipid storage diseases. Lipids (fatty materials such as waxes, fatty acids, oils, ... body. In Niemann-Pick disease, harmful quantities of lipids accumulate in the spleen, liver, lungs, bone marrow, ...

468

Coronary Heart Disease  

MedlinePLUS

... from the NHLBI on Twitter. What Is Coronary Heart Disease? Espańol Coronary heart disease (CHD) is a ... Red: Eileen's Story 10/14/2014 Celebrating American Heart Month: NIH Advancing Heart Research 10/14/2014 ...

469

Travelers' Health: Meningococcal Disease  

MedlinePLUS

... CDC website: www.cdc.gov/meningitis/bacterial.html Table 3-13. Vaccines to prevent meningococcal disease VACCINE ... Book Contents Chapter 3 (78) Meningococcal Disease more Tables Maps Figures Boxes Updates About Mobile Apps RSS ...

470

Carotid Artery Disease  

MedlinePLUS

... from the NHLBI on Twitter. What Is Carotid Artery Disease? Carotid (ka-ROT-id) artery disease is ... blood to your face, scalp, and neck. Carotid Arteries Figure A shows the location of the right ...

471

Coronary Artery Disease  

MedlinePLUS

Coronary artery disease (CAD) is the most common type of heart disease. It is the leading cause of death ... both men and women. CAD happens when the arteries that supply blood to heart muscle become hardened ...

472

Diabetic Eye Disease  

MedlinePLUS

... de los Ojos Cómo hablarle a su oculista Diabetic Eye Disease Listen View this module and educate yourself, family, and friends about diabetic eye disease. This module includes descriptive audio and ...

473

Sickle Cell Disease  

MedlinePLUS

... message, please visit this page: About CDC.gov . Sickle Cell Disease (SCD) New Study Findings - Sickle Cell Trait in ... SCD in New York found through newborn screening Sickle cell disease (SCD) is a group of inherited red blood ...

474

Chronic Kidney Diseases  

MedlinePLUS

... pressure at a healthy level. Continue Kinds of Kidney Diseases Like any complicated machine, not all kidneys work ... passed down through a person's genes. How Are Kidney Diseases Diagnosed? Kidney problems are often not noticed at ...

475

Eye Disease Simulations  

MedlinePLUS

... Research at NEI Education Programs Training and Jobs Eye Disease Simulations Listen Age-Related Macular Degeneration Cataract ... information page Back to top Diabetic Retinopathy Diabetic Eye Disease information page Back to top Glaucoma Glaucoma ...

476

Blood and Lymph Diseases  

MedlinePLUS

... for Biotechnology Information (US); 1998-. Genes and Disease [Internet]. Show details National Center for Biotechnology Information (US). ... Center for Biotechnology Information (US). Genes and Disease [Internet]. Bethesda (MD): National Center for Biotechnology Information (US); ...

477

Cholesterol and Heart Disease  

MedlinePLUS Videos and Cool Tools

... the lower right-hand corner of the player. Cholesterol and Heart Disease HealthDay February 5, 2015 Related MedlinePlus Pages Cholesterol Heart Diseases Transcript When should you start worrying ...

478

Diseases and Their Management  

Technology Transfer Automated Retrieval System (TEKTRAN)

Important diseases and their management practices of lentil were reviewed. The diseases reveiwed include Ascochyta blight (Ascochyta lentis), Anthracnose (Colletotrichum truncatum), White mold (Sclerotinia sclerotiorum), rust (Uromyces viciae-fabae), Botrytis gray mold (Botrytis cinerea and B. faba...

479

Sickle Cell Disease Quiz  

MedlinePLUS

... cure for sickle cell disease. FALSE Bone marrow/stem cell transplant can cure sickle cell disease. Bone ... where blood cells are made. A bone marrow/stem cell transplant is a procedure that takes healthy ...

480

Women and Heart Disease  

MedlinePLUS

... this? Submit What's this? Submit Button Related CDC Web Sites Heart Disease Stroke High Blood Pressure Salt ... this? Submit What's this? Submit Button Related CDC Web Sites Heart Disease Stroke High Blood Pressure Salt ...

481

Heart Disease Risk Factors  

MedlinePLUS

... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ...

482

Men and Heart Disease  

MedlinePLUS

... this? Submit What's this? Submit Button Related CDC Web Sites Heart Disease Stroke High Blood Pressure Salt ... this? Submit What's this? Submit Button Related CDC Web Sites Heart Disease Stroke High Blood Pressure Salt ...

483

Head Lice: Disease  

MedlinePLUS

... Treatment FAQs Malathion FAQs Epidemiology & Risk Factors Disease Biology Diagnosis Treatment Prevention & Control Resources for Health Professionals ... Frequently Asked Questions (FAQs) Epidemiology & Risk Factors Disease Biology Diagnosis Treatment Prevention & Control Resources for Health Professionals ...

484

Genetics of Alzheimer's Disease  

PubMed Central

Alzheimer's disease is the most common form of dementia and is the only top 10 cause of death in the United States that lacks disease-altering treatments. It is a complex disorder with environmental and genetic components. There are two major types of Alzheimer's disease, early onset and the more common late onset. The genetics of early-onset Alzheimer's disease are largely understood with variants in three different genes leading to disease. In contrast, while several common alleles associated with late-onset Alzheimer's disease, including APOE, have been identified using association studies, the genetics of late-onset Alzheimer's disease are not fully understood. Here we review the known genetics of early- and late-onset Alzheimer's disease. PMID:23984328

Ridge, Perry G.; Ebbert, Mark T. W.; Kauwe, John S. K.

2013-01-01

485

Alcoholic liver disease  

MedlinePLUS

Liver disease due to alcohol; Cirrhosis or hepatitis - alcoholic; Laennec's cirrhosis ... Alcoholic liver disease occurs after years of heavy drinking. Alcohol can cause inflammation in the liver . Over time, scarring ...

486

Progression of Liver Disease  

MedlinePLUS

... Handouts Education Resources Support Services Helpful Links For Liver Health Information Call 1-800-GO-LIVER (1- ... The Progression of Liver Disease The Progression of Liver Disease There are many different types of liver ...

487

Progression of Parkinson's Disease  

MedlinePLUS

... Order Free Materials Today Progression The progression of Parkinson’s disease varies among different individuals. Parkinson's is chronic and ... assess the progression of Parkinson's is the United Parkinson’s Disease Rating Scale (UPDRS). It is more comprehensive than ...

488

Treatments for Alzheimer's Disease  

MedlinePLUS

... 3900 Find your chapter: search by state Home > Alzheimer's Disease > Treatments Overview What Is Dementia? What Is Alzheimer's? ... and move closer to a cure. Treatments for Alzheimer's disease Get weekly e-news Take the Brain Tour ...

489

Depression and Alzheimer's Disease  

MedlinePLUS

... right-hand corner of the player. Depression and Alzheimer's Disease HealthDay January 15, 2015 Related MedlinePlus Pages Alzheimer's Disease Depression Transcript Depression and anxiety are common in ...

490

Lyme Disease in Construction  

MedlinePLUS

... doctor about the vaccine.) (Please turn the page.) Lyme Disease in Construction Hazard Alert Check for tick bites ... carefully. Only 12 of the workers who had Lyme disease on Long Island knew they had been bitten. ...

491

What Is Vascular Disease?  

MedlinePLUS

... when diseased, frequently cause life threatening strokes. Coronary arteries supply blood to the heart and, when diseased, can block ... is primarily managed by heart specialists – cardiologists. Iliac Arteries supply blood to the hip and the legs and, when ...

492

Metabolic Bone Disease  

Microsoft Academic Search

\\u000a Metabolic bone disease may result from genetic, endocrine, nutritional, or biochemical disorders, with variable and often\\u000a inconsistent imaging findings. For the radiologist, the cornerstone of “metabolic bone disease” has been osteoporosis, osteomalacia,\\u000a hyperparathyroidism, and Paget’s disease. Over the past three decades, the diagnosis and therapy of these diseases has changed,\\u000a influenced by biochemical discoveries, imaging advances, and epidemiology studies that

Murali Sundaram

493

Parallelization: Infectious Disease  

NSDL National Science Digital Library

Epidemiology is the study of infectious disease. Infectious diseases are said to be "contagious" among people if they are transmittable from one person to another. Epidemiologists can use models to assist them in predicting the behavior of infectious diseases. This module will develop a simple agent-based infectious disease model, develop a parallel algorithm based on the model, provide a coded implementation for the algorithm, and explore the scaling of the coded implementation on high performance cluster resources.

Aaron Weeden

494

Telomeres in disease  

PubMed Central

Telomeres and telomere repair are basic molecular features of cells possessing linear DNA chromosomes and defects in them result in various diseases. This review examines recent advances in understanding these diseases, particularly at a molecular level, and in relating telomere dysfunction to clinical diseases. We also discuss the potential role of telomere elongation as a therapy in diseases, and more controversially, the prevention/reversal of aging. PMID:22500192

Calado, Rodrigo

2012-01-01

495

Peyronie’s Disease  

Microsoft Academic Search

Peyronie’s disease is an enigmatic disease characterized by penile curvature, penile pain, and erectile dysfunction. The disease\\u000a appears to be more prevalent than previously thought as more men seek medical attention for sexual complaints. The lack of\\u000a a clear pathogenetic mechanism for the disease has led to numerous, diverse treatment options. Inconsistent results from studies\\u000a evaluating these various treatment options,

J. Slade Hubbard; Culley C. Carson

496

Metastatic Crohn's disease  

PubMed Central

Crohn's disease, first described in 1922, is characterized by segmental granulomatous inflammation of the intestinal tract and frequently involves the cutaneous tissues as well. Cutaneous Crohn's disease (CCD) is synonymous with metastatic Crohn's disease (MSD). A case of CCD, without any gastrointestinal involvement is reported for its rarity. PMID:24616854

Lanka, Padmavathy; Lanka, Lakshmana Rao; Sylvester, N.; Lakshmi, M. Dhana; Ethirajan, N.

2014-01-01

497

What's Mad Cow Disease?  

MedlinePLUS

... Main Page The Pink Locker Society What's Mad Cow Disease? KidsHealth > Kids > Q&A > Q & A > What's Mad Cow Disease? Print A A A Text Size What's ... You might have heard news reports about mad cow disease and wondered: What the heck is that? ...

498

Diabetes and Kidney Disease  

MedlinePLUS

... of high blood pressure and kidney disease. Maintaining control of your diabetes can lower your risk of developing severe kidney disease. What are the late signs of kidney disease in patients with diabetes? As your kidneys fail, your blood urea nitrogen (BUN) levels will rise as well as the ...

499

Pelvic Inflammatory Disease  

MedlinePLUS

... inflammatory disease What is pelvic inflammatory disease (PID)? Female reproductive system Pelvic inflammatory disease (PID) is an infection of a ... treatment for you. You must take all your medicine, even if your symptoms go away. This ... and chronic pelvic pain. Any damage done to your pelvic organs ...

500

Dysphagia in systemic disease  

Microsoft Academic Search

Dysphagia, difficulty in swallowing sometimes accompanied by a sensation of food sticking, is a common symptom resulting from structural and\\/or motility disorders of the oropharynx and esophagus which may result from local factors or occur as one of the manifestations of a systemic disease process. In this review, “systemic disease” will include diseases that affect more than one organ or

Bronwyn Jones; William J. Ravich; Martin W. Donner

1993-01-01