Note: This page contains sample records for the topic manifestations rhumatologiques revelatrices from Science.gov.
While these samples are representative of the content of Science.gov,
they are not comprehensive nor are they the most current set.
We encourage you to perform a real-time search of Science.gov
to obtain the most current and comprehensive results.
Last update: August 15, 2014.
1

Commonly Occurring VHL Manifestations  

MedlinePLUS

... Most Common Ages at DX Frequency in Patients CNS Retinal hemangioblastomas 0-68 yrs 12-25 yrs ... Occurring VHL Manifestations Kidney Brain and Spinal Cord / CNS Retina Pancreas Inner Ear Pheochromocytoma / Paraganglioma Reproductive Organs ...

2

Rare manifestations of sarcoidosis  

Microsoft Academic Search

Sarcoidosis may be associated with granulomatous inflammation in any part of the body, usually in more than one area (1). Rare manifestations of sarcoidosis include unusual patterns of organ involvement, or are the result of granulomatous inflammation developing in unusual locations for sarcoidosis. In other rare cases, sarcoidosis is associated with a second disorder. Although the frequency of individual rare

D. R. Moller

2005-01-01

3

Manifest and Latent Variates  

ERIC Educational Resources Information Center

The clue to what latent variable models are, and to a workable account of the basis for the traditional manifest/latent variable distinction, lies in a reconsideration of the indeterminacy property of linear factor structures. In this article, the authors contend that latent variable models are not detectors of unobservable latent structures,…

Maraun, Michael D.; Halpin, Peter F.

2008-01-01

4

Oral manifestations of hyperoxaluria.  

PubMed

Primary hyperoxaluria is a rare, inherited autosomal recessive disease caused by defects in the metabolism of glyoxylate. Oral manifestations of hyperoxaluria are rare. However, bone and tooth resorption may be the result of chronic inflammation and the presence of osteoclastic cells surrounding the oxalate crystal deposit. A deposit of calcium oxalate in the periodontium was identified in a patient with end-stage renal disease. Dental radiographs indicated bone loss and external tooth resorption. Radiolucent image in the inferior incisor region was observed and removed. The tissue showed granulomatous inflammation with foreign body reaction and associated crystalline deposits. When viewed in polarized light, these deposits are green and presented a birefringent aspect, which were interpreted as calcium oxalate crystals compatible with oxaluria. Oral manifestations of hyperoxaluria are of particular interest because of the unusual location of the oxalate crystal deposition, resulting in aggressive tooth resorption and alveolar bone loss, which may be misdiagnosed. PMID:22075817

Guerra, Eliete Neves da Silva; Vianna, Leonora; Sobreira, Maria Nazareth; de Araújo, Flavio Nader Gross; de Melo, Nilce Santos

2011-11-01

5

Diverse manifestations of trichloroethylene.  

PubMed Central

Trichloroethylene, a solvent used in a variety of industrial settings for more than 60 years, has caused adverse health effects on the central and peripheral nervous system, the skin, liver, kidney, and heart. Three men have shown relatively unusual manifestations secondary to exposure to trichloroethylene in degreasing operations in the jewelry industry. Toxic encephalopathy, hepatitis, and carpal spasm occurred among young, healthy workers. Clinical and laboratory data, including measurement of urinary trichloroacetic acid concentrations, are presented.

McCunney, R J

1988-01-01

6

Diverse manifestations of trichloroethylene.  

PubMed

Trichloroethylene, a solvent used in a variety of industrial settings for more than 60 years, has caused adverse health effects on the central and peripheral nervous system, the skin, liver, kidney, and heart. Three men have shown relatively unusual manifestations secondary to exposure to trichloroethylene in degreasing operations in the jewelry industry. Toxic encephalopathy, hepatitis, and carpal spasm occurred among young, healthy workers. Clinical and laboratory data, including measurement of urinary trichloroacetic acid concentrations, are presented. PMID:3342194

McCunney, R J

1988-02-01

7

Deconstructing the manifest dream.  

PubMed

A major part of the analyst's task is to discover the basis for the patient's misidentification of his present life situation with significant but threatening events of his earlier life, now repressed and inaccessible to conscious recall. Reconstructing the patient's history is a crucial step in this process of discovery, but the dynamic relation between the present and the past must be reconstructed as well. The structure of the manifest dream contains the key to this relation. The imagery of the dream is a composite of experimental materials drawn from important drive-related events of the present and the past. The complex formed by the manifest dream and the patient's associations provides the analyst with data about both of these distinct sets of drive-related experiences. As Freud's discussion of his M elusine dream illustrates, one associative thread can be traced to an experience that incorporates a conflicted current wish. Another thread will lead to an experience in which a repressed wish of childhood has been expressed. Where the two associative threads converge, in the composite imagery of the dream, the basis for the identification between the wishes of the present and the past will be exposed. An understanding of the structure of the manifest dream helps to clarify some of the important theoretical issues left unresolved in Freud's writings. These include: the function of the day residue and the mechanism through which it is formed, the relation of the screen memory to the associative process, and the differing roles of condensation and displacement in dream construction and free association. A simple procedure is described for enhancing the recovery of the significant childhood memories whose details have been incorporated into the composite imagery of the manifest dream. PMID:6736549

Palombo, S R

1984-01-01

8

CUTANEOUS MANIFESTATIONS IN BRUCELLOSIS  

PubMed Central

Brucellosis is a common worldwide zoonotic disease. Cutaneous manifestations are not specific and affect 1–14% of patients with brucellosis. Here, we describe 49-year-old female with fever and a diffuse maculopapular rash due to Brucella melitensis infection. Histopathology of skin biopsy revealed leukocytoclastic vasculitis; positive blood cultures for B. melitensis established the diagnosis of brucellosis. We provide a review of the relevant literature.

Karaali, Zeynep; Baysal, Birol; Poturoglu, Sule; Kendir, Mehmet

2011-01-01

9

Nonpulmonary manifestations of sarcoidosis  

Microsoft Academic Search

Sarcoidosis is one in a heterogeneous family of granulomatous disorders. The clinical manifestations of sarcoidosis can vary\\u000a widely, depending on the patient and the tissues involved. Recent advances in our understanding of the immunologic steps leading\\u000a to granuloma formation and persistence have yet to translate into more effective care for patients with this disease. This\\u000a review discusses the immunology of

Stephen J. Oliver

2002-01-01

10

Subtle manifestations of AIDS.  

PubMed

The current AIDS epidemic continues unabated. The steadily increasing number of HIV-infected persons in United States, and the prolonged latent period from time of infection to the development of clinical signs and symptoms, increase the likelihood of the undiagnosed patient arriving a t the dental office for treatment. Keen examination skills are required to recognize the early manifestations of infection. The purpose of this article is to present the clinical features of HIV infection and indicator oral diseases, and to illustrate the importance of the dental hygienist in providing early recognition of the very subtle clinical findings often associated with AIDS Related Complex. PMID:2370582

Sobieralski, M F; Miller, C S; Jones, S E

1990-02-01

11

Rheumatologic Manifestations of Sarcoidosis  

PubMed Central

Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis.

Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.

2012-01-01

12

[Rheumatologic manifestations of sarcoidosis].  

PubMed

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It has various clinical features. The most commonly affected organs are the lung, the lymph nodes, the eyes and the skin. Involvement of the musculoskeletal system is far less common and may be inaugural. Articular involvement is dominated by Lofgren syndrome and acute polyarthritis. Abarticular manifestations are often confounded with arthritis. Bone locations are dominated by sarcoidosis dactylitis and osteolysis. Muscular involvement is often unknown and can appear as 3 clinical features: spread form, myositique form or pseudotumoral form. Calcium balance disturbances are dominated by hypercalcemia which is often asymptomatique, but sometimes it reveal the sarcoidosis. Treatment of rheumatologic disorders often involves non steroidal antiinflammatory drugs, corticosteroids and methotrexate. Biological therapies such as the anti-TNFa and the anti-CD20 were showed to be effective in some case reports of severe and refractory disease. PMID:21049404

Hamdi, Wafa; Néji, Olfa; Ghannouchi, Mohamed Mehdi; Kaffel, Dhia; Kchir, Mohamed Montacer

2010-11-01

13

Manifesting the Quantum World  

NASA Astrophysics Data System (ADS)

In resisting attempts to explain the unity of a whole in terms of a multiplicity of interacting parts, quantum mechanics calls for an explanatory concept that proceeds in the opposite direction: from unity to multiplicity. Being part of the Scientific Image of the world, the theory concerns the process by which (the physical aspect of) what Sellars called the Manifest Image of the world comes into being. This process consists in the progressive differentiation of an intrinsically undifferentiated entity. By entering into reflexive spatial relations, this entity gives rise to (i) what looks like a multiplicity of relata if the reflexive quality of the relations is not taken into account, and (ii) what looks like a substantial expanse if the spatial quality of the relations is reified. If there is a distinctly quantum domain, it is a non-spatial and non-temporal dimension across which the transition from the unity of this entity to the multiplicity of the world takes place. Instead of being constituents of the physical world, subatomic particles, atoms, and molecules are instrumental in its manifestation. These conclusions are based on the following interpretive principle and its more direct consequences: whenever the calculation of probabilities calls for the addition of amplitudes, the distinctions we make between the alternatives lack objective reality. Applied to alternatives involving distinctions between regions of space, this principle implies that, owing to the indefiniteness of positions, the spatiotemporal differentiation of the physical world is incomplete: the existence of a real-valued spatiotemporal background is an unrealistic idealization. This guarantees the existence of observables whose values are real per se, as against "real by virtue of being indicated by the values of observables that are real per se." Applied to alternatives involving distinctions between things, it implies that, intrinsically, all fundamental particles are numerically identical and thus identifiable with the aforementioned undifferentiated entity.

Mohrhoff, Ulrich

2014-06-01

14

Gastrointestinal manifestations of leukemia.  

PubMed

Gastrointestinal (GI) manifestations of leukemia occur in up to 25% of patients at autopsy, generally during relapse. Its presence varies with the type of leukemia and has been decreasing over time due to improved chemotherapy. Gross leukemic lesions are most common in the stomach, ileum, and proximal colon. Leukemia in the esophagus and stomach includes hemorrhagic lesions from petechiae to ulcers, leukemic infiltrates, pseudomembranous esophagitis, and fungal esophagitis. Lesions in the small and large bowel are usually hemorrhagic or infiltrative. Infiltration of lymphoreticular organs, mainly spleen, liver, and lymph nodes, is more prominent in chronic than acute leukemia. Neutropenic enterocolitis, a necrotizing process involving the cecum, ascending colon, and terminal ileum, is increasing in incidence due to greater intensity of chemotherapy. Distension of bowel leads to mucosal breaches, permitting entry of organisms that grow profusely in the absence of neutrophils. Ischemic necrosis follows, leading to perforation and/or peritonitis. Patients present with fever, abdominal pain, diarrhea, nausea, vomiting, abdominal distension and tenderness. Ultrasound and computed tomography scans show thickening of the bowel wall. Treatment is supportive with surgery for necrosis and perforation. The main GI causes of death in leukemia are hemorrhage, infection, and necrotizing enterocolitis. PMID:21913980

Ebert, Ellen C; Hagspiel, Klaus D

2012-03-01

15

Skin manifestations of sarcoidosis.  

PubMed

The skin manifestations of sarcoidosis are classified as specific, where biopsy reveals non-caseating granulomas, and non-specific, typically erythema nodosum. The most frequent specific (granulomatous) skin lesions are maculopapules, subcutaneous nodules, scar sarcoidosis, plaques and lupus pernio. Skin biopsy allows early diagnosis of sarcoidosis through a non-aggressive procedure. In sarcoidosis, erythema nodosum is usually associated with bilateral hilar lymphadenopathy on the chest radiograph, this being known as Löfgren's syndrome. Cutaneous lesions have prognostic significance. Löfgren's syndrome is usually associated with good prognosis and spontaneous resolution. Maculopapular lesions and subcutaneous nodules are more often associated with remission of the systemic disease at two years, while plaques and, mainly, lupus pernio are hallmarks of chronic disease. Most cutaneous lesions of sarcoidosis are only mildly symptomatic and do not require treatment. However, chronic skin lesions, particularly lupus pernio, are disfiguring and can have a strong psychological and social impact. Treatment of these lesions is a challenge since they do not respond well to conventional treatments. The introduction of biological agents has been an important although not definitive advance in the treatment of cutaneous sarcoidosis. PMID:22579238

Mañá, Juan; Marcoval, Joaquim

2012-06-01

16

Ocular Manifestations of Trichothiodystrophy  

PubMed Central

Objective Trichothiodystrophy (TTD) is a rare autosomal recessive disorder characterized by sulfur-deficient brittle hair and multisystem abnormalities. Many TTD patients have a defect in known DNA repair genes. This report systematically evaluates the ocular manifestations of the largest-to-date cohort of TTD patients and xeroderma pigmentosum (XP)/TTD patients. Design Case Series Participants Thirty-two participants, ages 1 to 30 years, referred to the National Eye Institute for examination from 2001 to 2010: Twenty-five had TTD, and 7 had XP/TTD. Methods Complete, age- and developmental stage-appropriate ophthalmic examination. Main Outcome Measures Visual acuity (VA), best corrected VA, ocular motility, state of the ocular surface and corneal endothelial cell density, corneal diameter, lens assessment. Results Developmental abnormalities included microcornea (44% TTD), microphthalmia (8% TTD, 14% XP/TTD), nystagmus (40% TTD), and infantile cataracts (56% TTD and 86% XP/TTD). Corrective lenses were required by 65% of the participants, and decreased best-corrected VA was present in 28% of TTD patients and 71% of XP/TTD patients. Degenerative changes included dry eye (32% TTD, 57% XP/TTD) and ocular surface disease identified by ocular surface staining with fluorescein (32% TTD), that usually are exhibited by much older patients in the general population. The two oldest TTD patients exhibited clinical signs of retinal/macular degeneration. Four XP/TTD patients presented with corneal neovascularization. Conclusions TTD and XP/TTD study participants had a wide variety of ocular findings including refractive error, infantile cataracts, microcornea, nystagmus, and dry eye/ocular surface disease. While many of these can be ascribed to abnormal development—likely due to abnormalities in basal transcription of critical genes—patients may also have a degenerative course.

Brooks, Brian P; Thompson, Amy H; Clayton, Janine A; Chan, Chi-Chao; Tamura, Deborah; Zein, Wadih M; Blain, Delphine; Hadsall, Casey; Rowan, John; Bowles, Kristen E; Khan, Sikandar G.; Ueda, Takahiro; Boyle, Jennifer; Oh, Kyu-Seon; DiGiovanna, John J; Kraemer, Kenneth H

2011-01-01

17

Abdominal manifestations of neurologic disorders.  

PubMed

A variety of disorders-including infectious, inflammatory, hereditary, and metabolic diseases-may affect both the brain and abdominal cavity, and the findings in one region may help establish the diagnosis or limit the differential diagnosis. Establishing an accurate early diagnosis enables clinicians to adequately manage these unusual diseases and potentially avert life-threatening complications. For example, an early diagnosis of Gardner syndrome enables annual sigmoid- or colonoscopy and ultrasonography. In many conditions, abdominal manifestations precede neurologic manifestations and may have prognostic significance. Patients with celiac disease more often present with abdominal manifestations such as duodenitis, slow transit time, reversal of the jejunal-ileal fold pattern, and transient small bowel intussusception than with intracranial manifestations. In other conditions, the neurologic manifestations may be the same as the presenting symptoms. For example, patients with Gardner syndrome may initially present with multiple mandibular or sinonasal osteomas. In addition, sarcoidosis may manifest with multifocal enhancing dural masses. Abdominal and neurologic manifestations may even occur simultaneously, as in several of the phakomatoses such as neurofibromatosis type 1, tuberous sclerosis complex, and von Hippel-Lindau syndrome. Ultimately, familiarity with the appearances of these conditions allows radiologists to pinpoint a diagnosis, even when imaging findings in either location are nonspecific. PMID:23322834

Bhavsar, Anil S; Verma, Sadhna; Lamba, Ramit; Lall, Chandana G; Koenigsknecht, Vincent; Rajesh, Arumugam

2013-01-01

18

Ocular manifestations of drug abuse.  

PubMed

Abuse of drugs, including some used clinically and others with no legal or clinical use, can cause ocular injury and disease. Ocular manifestations of drug abuse may be due to the substances themselves, to invasive methods of administration, or to injury suffered during states of altered consciousness. Grouping the drugs into five categories (opiates, marijuana, stimulants, depressants and hallucinogens), the authors describe the pharmacologic, congenital, toxic, infectious, embolic, and psychological ocular manifestations of their abuse. PMID:2872731

McLane, N J; Carroll, D M

1986-01-01

19

Gastrointestinal Manifestations of Systemic Sclerosis  

Microsoft Academic Search

Systemic sclerosis is a chronic disorder of connective tissue that affects the gastrointestinal tract in more than 80% of\\u000a patients. Changes in neuromuscular function with progressive fibrosis of smooth muscle within the muscularis propria impair\\u000a normal motor function, which may secondarily alter transit and nutrient absorption. Esophageal manifestations with gastroesophageal\\u000a reflux and dysphagia are the most common visceral manifestation of

Robyn Domsic; Kenneth Fasanella; Klaus Bielefeldt

2008-01-01

20

The protean manifestations of pheochromocytoma.  

PubMed

The treacherous and deceptive nature of pheochromocytoma makes it crucial to detect and treat it promptly; otherwise it will almost certainly be fatal from cardiovascular complications or metastases. Hypertension occurring in patients with pheochromocytomas is sustained in about 50% and paroxysmal in the remainder; however, many patients remain normotensive. Hypertension attacks may be precipitated by physical activity, postural changes, anxiety, certain foods or wine, some drugs, operative procedures, etc. Cardinal manifestations are paroxysmal hypertension, headache, palpitations +/- tachycardia, inappropriate sweating; anxiety, tremulousness, pallor (rarely flushing), chest and abdominal pains; nausea and vomiting often occur. Hypercatecholaminemia manifestations are more common and pronounced when paroxysmal hypertension occurs, but persons with familial pheochromocytoma may be asymptomatic. Protean manifestations of pheochromocytoma may simulate many conditions, some of which may have elevated plasma and urine catecholamines and their metabolites. Baro-reflex failure, postural tachycardia syndrome, sleep apnea, carcinoid, renal failure, and pseudopheochromocytoma may be diagnostic challenges. The history, physical examination, biochemical testing (after eliminating interfering drugs, when possible) for plasma and urinary metanephrines can usually establish or exclude presence of pheochromocytomas. Occasionally a clonidine suppression test is needed to differentiate neurogenic from pheochromocytic hypertension. Manifestations suggesting hypercatecholaminemia without hypertension are highly atypical of pheochromocytoma. Pheochromocytoma may present as panic attacks, pre-eclampsia, cardiomyopathy, infection with fever and leucocytosis, diabetes, migraine, shock, Cushing's syndrome, multiple organ failure with lactic acidosis, neurological manifestations, transitory electrocardiogram abnormalities, constipation, intestinal obstruction, visual impairment, convulsions, etc. The key to diagnosis is always to think of pheochromocytoma in the differential diagnosis of hypertension. PMID:19242899

Manger, W M

2009-09-01

21

[Oral manifestations of endocrine dysfunction].  

PubMed

Oral manifestations of endocrine dysfunction often may be observed initially by the dentist. Objective manifestations, such as ophtalmos in hyperthyroidism, signs of hypersecretion of GH in acromegaly, are easily recognized. Dentists should have some knowledge of many other diseases in this category that occasionally come in our attention. The present article will discuss the effects of over and under-secretion of each endocrine gland separately, showing its influence on the development and maintenance of the health of the teeth and supporting structures. Diabetes mellitus is the most common endocrinological disease, with an incidence of 3%. Periodontitis risk is three time greater in diabetic patients than in general population and it may worsen the diabetes evolution. Periodontitis in diabetic patients needs an rapid diagnosis and treatment. We also presents the oral aspects of thyroid, parathyroid, suprarenalian, growth hormone and female hormones pathology. The incidence of these troubles is less important, but oral manifestations may reveal an endocrine disfunction. PMID:15550888

Ionescu, O; Sonnet, E; Roudaut, N; Prédine-Hug, F; Kerlan, V

2004-10-01

22

Breast manifestations of systemic diseases  

PubMed Central

Although much emphasis has been placed on the primary presentations of breast cancer, little focus has been placed on how systemic illnesses may affect the breast. In this article, we discuss systemic illnesses that can manifest in the breast. We summarize the clinical features, imaging, histopathology, and treatment recommendations for endocrine, vascular, systemic inflammatory, infectious, and hematologic diseases, as well as for the extramammary malignancies that can present in the breast. Despite the rarity of these manifestations of systemic disease, knowledge of these conditions is critical to the appropriate evaluation and treatment of patients presenting with breast symptoms.

Dilaveri, Christina A; Mac Bride, Maire Brid; Sandhu, Nicole P; Neal, Lonzetta; Ghosh, Karthik; Wahner-Roedler, Dietlind L

2012-01-01

23

Cutaneous manifestations in celiac disease  

PubMed Central

Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of intestinal villi that improves after gluten-free diet (GFD). CD is often associated with extra-intestinal manifestations; among them, several skin diseases are described in CD patients. The present review reports all CD-associated skin manifestations described in the literature and tries to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed.

Abenavoli, L; Proietti, I; Leggio, L; Ferrulli, A; Vonghia, L; Capizzi, R; Rotoli, M; Amerio, PL; Gasbarrini, G; Addolorato, G

2006-01-01

24

Psychiatric manifestations in cerebrotendinous xanthomatosis.  

PubMed

Cerebrotendinous xanthomatosis (CTX) is a rare and severe, but treatable, inborn disorder of bile acid biosynthesis and sterol storage with autosomal recessive inheritance and variable clinical presentation. CTX treatment consists of chenodeoxycholic acid and must be started as early as possible to prevent permanent disability. Psychiatric manifestations are rare and non-specific, and often lead to significant diagnostic and treatment delay. Therefore, better recognition of the gamut of psychiatric manifestations in CTX can diminish the risk of misdiagnosis and irreversible neurological deterioration. We hereby describe the psychiatric features in CTX. A complete review of all published cases of CTX in the medical literature was undertaken and the case reports with psychiatric presentation were collected and analyzed. We also describe the psychiatric features in relation to the neurological semeiology in six patients with CTX diagnosed at the La Salpêtrière Hospital. We conclude that psychiatric manifestations in CTX follow a bimodal/bitemporal pattern, appearing early in the disease course in the form of a behavioral/personality disorder associated with learning difficulties or mental retardation, or manifesting in advanced disease in the setting of dementia as rich neuropsychiatric syndromes, such as frontal, orbitofrontal or frontotemporal syndromes of cortico-subcortical dementia encompassing behavioral/personality disturbance, affective/mood disorders or psychotic disorders. Behavioral/personality disturbance in childhood or adolescence, especially when accompanied by learning difficulties, should therefore lead to further investigation to exclude CTX, as early diagnosis and treatment is critical for prognosis. PMID:24002088

Fraidakis, M J

2013-01-01

25

[Neurological manifestations of Behçet's disease].  

PubMed

Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: "parenchymal" lesions, which include mainly meningoencephalitis as opposed to "extra-parenchymal" lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death. PMID:24290030

Noel, N; Drier, A; Wechsler, B; Piette, J-C; De Paz, R; Dormont, D; Cacoub, P; Saadoun, D

2014-02-01

26

Unusual Manifestations after Malathion Poisoning  

Microsoft Academic Search

We report a case of organophosphate poisoning with a commercial preparation of malathion (deliberate ingestion of Malathane Garden Spray: malathion 15% in isopropyl alcohol) in which the initial cholinergic crisis was followed by cardiac, pulmonary, neurological and renal manifestations. They occurred when erythrocyte and plasma cholinesterases were reactivating. A chemical analysis of the pesticide preparation revealed, apart from malathion itself,

A. Dive; P. Mahieu; R. Van Binst; A. Hassoun; D. Lison; H. De Bisschop; B. Nemery; R. Lauwerys

1994-01-01

27

The neuromuscular manifestations of amyloidosis.  

PubMed

Amyloidosis is a systemic disease that may be acquired or hereditary and which results in the deposition of amyloid fibrils in a variety of tissues causing their progressive dysfunction. Although the clinical presentation often is dominated by cardiac or renal failure, peripheral neuropathy may be a significant or the initial manifestation, resulting in presentation to the neurologist. Diagnosis often is challenging and may require multiple diagnostic procedures, including more than one biopsy. Acquired and hereditary amyloidosis can be definitively distinguished from one another only by immunohistochemical staining or molecular genetic testing. Treatment remains a challenge, although chemotherapy and autologous stem cell transplantation offer hope for those with primary systemic amyloidosis, whereas liver transplantation is effective for some forms of hereditary amyloid neuropathy. Much less commonly, myopathy may be a clinically significant manifestation of amyloidosis. PMID:20215989

Simmons, Zachary; Specht, Charles S

2010-03-01

28

Cardiopulmonary Manifestations of Ankylosing Spondylitis  

PubMed Central

Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications.

Momeni, Mahnaz; Taylor, Nora; Tehrani, Mahsa

2011-01-01

29

Severe pulmonary manifestation of leptospirosis.  

PubMed

Based on increasing incidence and the occurrence of worldwide outbreaks, leptospirosis is recognised as an emerging zoonosis. Severe manifestations are associated with high morbidity and mortality rates and may therefore pose an important risk to public health, especially in certain high prevalence areas. A considerable number of infections progress to a severe form, which can present as the well-known triad of jaundice, impaired renal function and haemorrhage, known as Weil's disease. The severe pulmonary form of leptospirosis (SPFL) is a less known entity and is characterised by intra-alveolar haemorrhage and can lead to acute respiratory failure and death when adequate treatment fails. Prognostic factors correlating with severity and survival of leptospirosis include indicators of renal failure, pulmonary involvement and electrolyte imbalances. We report an imported case of SPFL in a returning traveller, and review the literature discussing epidemiology, clinical manifestations, prognostic factors and treatment of this resurgent disease. PMID:22744922

Helmerhorst, H J F; van Tol, E N; Tuinman, P R; de Vries, P J; Hartskeerl, R A; Grobusch, M P; Hovius, J W

2012-06-01

30

Cardiac manifestations in Fabry disease  

Microsoft Academic Search

Fabry disease is an X-linked recessive genetic disorder of glycosphingolipid metabolism, due to deficiency of the lysosomal\\u000a enzyme?-galactosidase A. The disease is characterized by the progressive intracellular lysosomal accumulation of neutral glycosphingolipids\\u000a throughout the body, including the cardiovascular system.\\u000a \\u000a It has been reported that cardiac involvement could be the sole manifestation of the disease in some patients. Myocardial\\u000a abnormalities are

A. Linhart; J.-C. Lubanda; T. Palecek; J. Bultas; D. Karetová; J. Ledvinová; M. Elleder; M. Aschermann

2001-01-01

31

MUCOCUTANEOUS MANIFESTATIONS OF DENGUE FEVER  

PubMed Central

Dengue viral infection is a cause of considerable morbidity and mortality and may be associated with a variety of mucocutaneous manifestations that may provide important early clues to the diagnosis of this condition. Cutaneous and mucosal findings like confluent erythema, morbilliform eruptions, and hemorrhagic lesions may figure prominently in the clinical features of dengue. The differential diagnoses include a large number of bacterial and viral exanthems as well as drug rash.

Thomas, Emy Abi; John, Mary; Kanish, Bimal

2010-01-01

32

Neurologic Manifestations of Lyme Disease  

Microsoft Academic Search

Borrelia burgdorferi infection, the tick-borne spirochetosis known as Lyme disease or Lyme borreliosis, involves the nervous system (neuroborreliosis)\\u000a in 10% to 15% of patients. Common manifestations include lymphocytic meningitis, cranial neuritis, mononeuropathy multiplex,\\u000a and painful radiculoneuritis. Rare patients develop inflammation in the brain or spinal cord. Regardless of the form of involvement,\\u000a neuroborreliosis can be microbiologically cured in virtually all

John J. Halperin

2011-01-01

33

Hepatic Manifestations in Hematological Disorders  

PubMed Central

Liver involvement is often observed in several hematological disorders, resulting in abnormal liver function tests, abnormalities in liver imaging studies, or clinical symptoms presenting with hepatic manifestations. In hemolytic anemia, jaundice and hepatosplenomegaly are often seen mimicking liver diseases. In hematologic malignancies, malignant cells often infiltrate the liver and may demonstrate abnormal liver function test results accompanied by hepatosplenomegaly or formation of multiple nodules in the liver and/or spleen. These cases may further evolve into fulminant hepatic failure.

Murakami, Jun

2013-01-01

34

Atypical manifestations of dengue Fever.  

PubMed

We reviewed case records of 40 in-patients (22 boys) with serologically confirmed dengue fever between 1st October and 30th November, 2013. Severe dengue was seen in 30, out of which 12 (30%) had compensated shock. Splenomegaly (6,15%) and encephalopathy (4,10%) were the commonest atypical features. Atypical manifestations of dengue fever were more common than that reported in the past. PMID:24986292

Pawaria, Arti; Mishra, Devendra; Juneja, Monica; Meena, Jagdish

2014-06-01

35

40 CFR 267.72 - Manifest discrepancies.  

Code of Federal Regulations, 2013 CFR

...or type of hazardous waste designated on the manifest or shipping paper, and the quantity or type of hazardous waste a facility actually...solvent substituted for waste acid, or toxic constituents...manifest or shipping paper. (b) Upon...

2013-07-01

36

Dermatitis Herpetiformis: Skin Manifestation of Celiac Disease  

MedlinePLUS

... a chronic, intensely itchy, blistering skin manifestation of gluten-sensitive enteropathy, commonly known as celiac disease. DH ... external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin ...

37

Clinical manifestations of food allergy.  

PubMed

Adverse reactions to foods are a diverse group of clinical syndromes resulting from immunologic and non-immunologic responses to food ingestion. Symptoms can range from mild, self-limiting reactions to severe, life-threatening reactions depending on the mechanism. This review primarily focuses on the clinical manifestations of immunologically derived adverse food reactions or food allergies.The true prevalence of food allergy is unknown. Up to 25% of the general population believes that they may be allergic to some food; however, the actual prevalence of food allergy diagnosed by a provider appears to be 1.5% to 2% of the adult population and approximately 6% to 8% of children. This discrepancy makes it imperative that clinicians are aware of the different food allergy syndromes. With a clear understanding of the clinical manifestations of food allergies, an accurate diagnosis and treatment plan can be formulated. Failing to do so may result in unnecessary dietary restrictions that may adversely affect nutritional status, growth, and quality of life.Most food allergic reactions are secondary to a limited number of foods, and the most common foods causing allergic reactions in children include milk, egg, peanuts, tree nuts, and fish. In adolescents and adults, allergies to peanuts, tree nuts, fish, and shellfish are most prevalent. Food allergies can result from immunoglobulin E (IgE)-mediated, non-IGE-mediated, or mixed IgE/non-IgE mechanisms. The purpose of this review is to discuss the clinical manifestations of each of these types of food allergy. PMID:23718237

Perry, Tamara T; Pesek, Robbie D

2013-06-01

38

Otolaryngologic manifestations of phenytoin toxicity.  

PubMed

DPH may result in protean head and neck manifestations (Table 1). In some cases these effects are dose related and in others they appear to be idiosyncratic; some occur only at toxic levels (Table 2). Many of the conditions are reversible and withdrawal of DPH or re-establishment of therapeutic levels is the only treatment necessary. Failure to recognize this fact can result in expensive workups or, worse, in aggressive treatment, both of which are unnecessary. The otolaryngologist should therefore be aware of these conditions and consider them in the differential diagnosis. PMID:1587038

Grillone, G; Myssiorek, D

1992-04-01

39

Neurological manifestation of colonic adenocarcinoma  

PubMed Central

Paraneoplastic neurologic disorders are extremely rare in cancer patients and are most commonly associated with certain tumors, such as ovarian cancer, small cell lung cancer, and breast cancer. We report here a paraneoplastic neurological syndrome in a 53-year-old man with colonic adenocarcinoma with a solitary liver metastasis. His paraneoplastic syndrome was successfully treated by methylprednisolone and primary oncologic therapies including neoadjuvant chemotherapy and definitive surgery. This is also the first documented case of simultaneous manifestation of a sensory neuropathy and limbic encephalitis with colon cancer.

Sio, Terence T.; Paredes, Mercedes; Uzair, Chaudhary

2012-01-01

40

Unusual manifestations after malathion poisoning.  

PubMed

We report a case of organophosphate poisoning with a commercial preparation of malathion (deliberate ingestion of Malathane Garden Spray: malathion 15% in isopropyl alcohol) in which the initial cholinergic crisis was followed by cardiac, pulmonary, neurological and renal manifestations. They occurred when erythrocyte and plasma cholinesterases were reactivating. A chemical analysis of the pesticide preparation revealed, apart from malathion itself, the presence of isopropylmalathion and O,O,S-trimethylphosphorothioate. Although pure malathion is regarded as one of the safest organophosphate insecticides, this observation underlines the possibility of severe complications after exposure to a preparation which has been stored for a long period of time. PMID:8204314

Dive, A; Mahieu, P; Van Binst, R; Hassoun, A; Lison, D; De Bisschop, H; Nemery, B; Lauwerys, R

1994-04-01

41

Rheumatic manifestations in diabetic patients  

PubMed Central

Diabetes mellitus (DM), a worldwide high prevalence disease, is associated with a large variety of rheumatic manifestations. For most of these affections, pathophysiologic correlations are not well established. Some of them, such as diabetic cheiroarthropathy, neuropathic arthritis, diabetic amyotrophy, diabetic muscle infraction, are considered intrinsic complications of DM. For others, like diffuse idiopathic skeletal hyperostosis or reflex sympathetic dystrophy, DM is considered a predisposing condition. In most cases, these affections cause pain and disability, affecting the quality of life of diabetic patients, but once correctly diagnosed, they often respond to the treatment, that generally requires a multidisciplinary team. This article reviews some epidemiological, clinical, diagnostic and therapeutic aspects of these conditions.

Serban, AL; Udrea, GF

2012-01-01

42

Musculoskeletal manifestations of endocrine disorders.  

PubMed

Endocrine disorders can lead to disturbances in numerous systems within the body, including the musculoskeletal system. Radiological evaluation of these conditions can demonstrate typical appearances of the bones and soft tissues. Knowledge of these patterns can allow the radiologist to suggest a diagnosis that may not be clinically apparent. This review will highlight the typical musculoskeletal findings of acromegaly, hypercortisolism, hyperthyroidism, hypothyroidism, hyperparathyroidism, pseudo- and pseudopseudohypoparathyroidism, and diabetes mellitus. The radiological manifestations of each of these endocrine disorders, along with a brief discussion of the pathophysiology and clinical implications, will be discussed. PMID:24642251

Boswell, Stephanie B; Patel, Dakshesh B; White, Eric A; Gottsegen, Christopher J; Forrester, Deborah M; Masih, Sulabha; Matcuk, George R

2014-01-01

43

Extraintestinal Manifestations of Inflammatory Bowel Disease  

PubMed Central

Extraintestinal manifestations of inflammatory bowel disease are prevalent in both ulcerative colitis and Crohn's disease. The most common manifestations involve the musculoskeletal and dermatologic systems. Other manifestations involve the hepatopan-creatobiliary system (eg, primary sclerosing cholangitis) as well as the ocular, renal, and pulmonary systems. A multidisciplinary team approach is often needed for effective management, and emergency situations require prompt evaluation.

Burakoff, Robert

2011-01-01

44

The Extrarenal Manifestations of Hypernephroma  

PubMed Central

The extrarenal manifestations of hypernephroma in 34 medical patients are described, and the literature on this subject is reviewed. One case presented with hematuria and 11 other cases with metastases. Presentation was not obviously related to metastases in 22 patients. Eight were first seen because of fever or general weakness. Vascular disturbances in six included hypertension, thrombophlebitis, inferior vena caval obstruction and varicocele. Four patients had gastrointestinal complaints; one had hypercalcemia and another musculoskeletal symptoms. Two cases had neurological syndromes, one of which proved to be due to metastases. The overall incidence of systemic features was greater and included anemia, eosinophilia, a leukemoid reaction and thrombocytosis. Polycythemia, amyloidosis and hyponatremia were not encountered. Urographic procedures were performed in half of the patients, most of whom had an abdominal mass. Calcium was visible radiologically in the tumour in five cases. The diagnosis was not made in 19 and was an unexpected finding at autopsy in 10. Appreciation of the extrarenal manifestations of hypernephroma might lead to earlier diagnosis.

Gordon, Duncan A.

1963-01-01

45

[AIDS--mucous membrane manifestations].  

PubMed

In the course of the infection with the human immunodeficiency virus (HIV), we frequently observe disorders of the mucous membranes and, occasionally, they present the first manifestation of HIV-induced immunodeficiency. Like in other organs, opportunistic infections and malignant tumors prevail as a result of the impaired immune system. Opportunistic infections are characterized by frequency (candidiasis), aggressive expansion, persistence, frequent recurrences, and resistance to therapy (gingivitis, parodontitis, herpes simplex, warts). Oral hairy leucoplakia is considered a specific lesion of HIV infection. Malignant tumors, such as Kaposi's sarcoma, non-Hodgkin's lymphoma, and squamous cell carcinoma, may cause marked morbidity in AIDS patients; occasionally, the clinical picture of Kaposi's sarcoma and non-Hodgkin's lymphoma is rather uncharacteristic. Other manifestations on the mucous membranes may arise in association with systemic reactions, such as drug eruptions, thrombocytopenic purpura, or acute HIV infection. The etiology of still other lesions of the mucous membranes (e.g. chronic recurrent ulcers, xerostomia, disorders of pigmentation) is incompletely understood. The awareness of these disorders of the mucous membranes in HIV infection is of diagnostic, therapeutic and epidemiological importance. PMID:2205062

Pichler, E; Zangerle, R; Puelacher, W; Waldhart, E; Hintner, H; Fritsch, P

1990-07-01

46

Genital manifestations of tropical diseases  

PubMed Central

Genital symptoms in tropical countries and among returned travellers can arise from a variety of bacterial, protozoal, and helminthic infections which are not usually sexually transmitted. The symptoms may mimic classic sexually transmitted infections (STIs) by producing ulceration (for example, amoebiasis, leishmaniasis), wart-like lesions (schistosomiasis), or lesions of the upper genital tract (epididymo-orchitis caused by tuberculosis, leprosy, and brucellosis; salpingitis as a result of tuberculosis, amoebiasis, and schistosomiasis). A variety of other genital symptoms less suggestive of STI are also seen in tropical countries. These include hydrocele (seen with filariasis), which can be no less stigmatising than STI, haemospermia (seen with schistosomiasis), and hypogonadism (which may occur in lepromatous leprosy). This article deals in turn with genital manifestations of filariasis, schistosomiasis, amoebiasis, leishmaniasis, tuberculosis and leprosy and gives clinical presentation, diagnosis, and treatment.

Richens, J

2004-01-01

47

Neuroophthalmologic manifestations of systemic disease.  

PubMed

Significant contributions on the neuroophthalmologic manifestations of systemic disease have been published in the past year. The first part of this paper is devoted to practical guidelines that may help in the diagnosis and the management of oculomotor disorders, especially in connection with systemic diseases; the second part is focused on specific entities. Including idiopathic intracranial hypertension and ophthalmoplegic migraine in this review might be debatable; however, although the cause of these conditions is still unclear, relevant findings suggest that they may be the consequence of other adjacent processes rather than the cause itself. We believe that the diagnosis of idiopathic intracranial hypertension as well as that of ophthalmoplegic migraine remains a challenging issue for the ophthalmologist and may easily be overlooked. PMID:10621544

Spiritus, M; Boschi, A

1999-10-01

48

Hematologic manifestations of celiac disease  

PubMed Central

Celiac disease is a common systemic disorder that can have multiple hematologic manifestations. Patients with celiac disease may present to hematologists for evaluation of various hematologic problems prior to receiving a diagnosis of celiac disease. Anemia secondary to malabsorption of iron, folic acid, and/or vitamin B12 is a common complication of celiac disease and many patients have anemia at the time of diagnosis. Celiac disease may also be associated with thrombocytosis, thrombocytopenia, leukopenia, venous thromboembolism, hyposplenism, and IgA deficiency. Patients with celiac disease are at increased risk of being diagnosed with lymphoma, especially of the T-cell type. The risk is highest for enteropathy-type T-cell lymphoma (ETL) and B-cell lymphoma of the gut, but extraintestinal lymphomas can also be seen. ETL is an aggressive disease with poor prognosis, but strict adherence to a gluten-free diet may prevent its occurrence.

Halfdanarson, Thorvardur R.; Litzow, Mark R.; Murray, Joseph A.

2007-01-01

49

Gastrointestinal manifestations of systemic mastocytosis  

PubMed Central

Systemic mastocytosis (SM) is a rare disease with abnormal proliferation and infiltration of mast cells in the skin, bone marrow, and viscera including the mucosal surfaces of the digestive tract. Gastrointestinal (GI) symptoms occur in 14%-85% of patients with systemic mastocytosis. The GI symptoms may be as frequent as the better known pruritus, urticaria pigmentosa, and flushing. In fact most recent studies show that the GI symptoms are especially important clinically due to the severity and chronicity of the effects that they produce. GI symptoms may include abdominal pain, diarrhea, nausea, vomiting, and bloating. A case of predominantly GI systemic mastocytosis with unique endoscopic images and pathologic confirmation is herein presented, as well as a current review of the GI manifestations of this disease including endoscopic appearances. Issues such as treatment and prognosis will not be discussed for the purposes of this paper.

Lee, Jason K; Whittaker, Scott J; Enns, Robert A; Zetler, Peter

2008-01-01

50

Caudal Cingulate Infarction Manifesting Astasia  

PubMed Central

Introduction Astasia is a rare presenting symptom of stroke, usually known as ‘thalamic astasia’, induced by a lesion in the ventrolateral thalamus. We report a case of caudal cingulate infarction manifesting astasia. Case Presentation A 58-year-old male presented with inability to sit, stand and walk (astasia). No apparent motor weakness was noticed in the extremities. MRI revealed cerebral infarction in the caudal cingulate gyrus, which was located between the vertical commissure anterior (VCA) line and vertical commissure posterior (VPC) line. His symptoms persisted for 1 year to a lesser degree. Conclusion Lesions in the caudal cingulate gyrus can present with astasia. The responsible lesion is located in the cingulate gyrus between the VCA and VPC line, which might correspond to the caudal cingulate zone in humans. We should keep in mind that astasia can be a presenting symptom of stroke.

Satow, Takeshi; Komuro, Taro; Kobayashi, Akira

2014-01-01

51

Severe Oxalosis With Systemic Manifestations  

PubMed Central

Ethylene glycol toxicity can have various clinical presentations with different organ system involvements. These presentations are independent of the level of toxicity. We describe a 31 years old male who presented with ethylene glycol toxicity manifesting as anuric renal failure who subsequently developed neurological sequela of its toxicity. Ethylene glycol is known to be metabolized to various metabolites and is ultimately converted to oxalate which results in crystal deposition the renal parenchyma causing renal failure. Oxalate deposition can occur in various organs including the nervous system as seen in our patient. The majority of patients do not recover from severe oxalosis despite the supportive care of hemodialysis in removing the parent compounds. Despite severe oxalosis, our patient was fortunate enough to be left with minimal neurological sequelae, and eventually was able to cease hemodialysis treatments. Keywords Ethylene glycol; Oxalate; Oxalosis; Glyoxylic acid

Samarneh, Majed Mark; Shtaynberg, Norbert; Goldman, Michael; Epstein, Edward; Kleiner, Morton; El-Sayegh, Suzanne

2012-01-01

52

Neurodevelopmental Manifestations of Mitochondrial Disease  

PubMed Central

Mitochondrial disease is an increasingly recognized but widely heterogeneous group of multi-systemic disorders that commonly involve severe neurodevelopmental manifestations in childhood. This article will review the presentation, genetic basis, and diagnostic evaluation of primary mitochondrial disease. Emphasis will be placed on neurodevelopmental findings that may be encountered by a Developmental Pediatrician that should provoke consideration of a mitochondrial disorder. The inheritance patterns and mechanisms by which mutations in genes located in either the nuclear or mitochondrial genomes can cause mitochondrial diseases will be discussed. A general overview of the current diagnostic evaluation that can be readily initiated by the Developmental Pediatrician will be provided, along with a summary of currently available treatment options.

Falk, Marni J.

2014-01-01

53

[Ophthalmological manifestations of arterial hypertension].  

PubMed

Systemic Arterial hypertension (AHT) is one of the most frequent diseases in the industrialised countries, with an incidence reaching 30%, a figure that is rising due to the greater life expectancy of the population. This rise in arterial tension causes, or accelerates, changes in the vascular wall of the target organs such as the kidney, brain, heart and eye. At the ocular level, AHT produces lesions in the retina, the choroids and optic nerve head; this can include a wide range of lesions, from slight vascular narrowing to severe visual loss due to ischaemic optical neuropathy. The primary response of the retinal arteries to systemic arterial hypertension is vascular narrowing and the manifestations that appear at the back of the eye in hypertensive retinopathy are diffuse or focal vasoconstriction, extravasation due to increased vascular permeability and arteriosclerosis with swelling of the wall of the vessels. These three entities are responsible for the appearance of different lesions that characterise the stages of the retinal disease, which are: arteriovenous crossings, hard and cotton-like exudates, thrombosis, embolisms, haemorrhages in the retinal parenchyma, vitreous detachment from the retina, papilla edema and ischaemic optical neuropathy in the more severe cases, such as in case malign arterial hypertension. PMID:19169291

Rodríguez, N A; Zurutuza, A

2008-01-01

54

[Cardiac manifestations of muscular dystrophies].  

PubMed

Muscular dystrophies (MD) are a clinically and genetically heterogeneous disease group. In the last few years, remarkable progress has been made in understanding the close und various relations between skeletal muscle disease and heart muscle disease. Cardiac involvement has been documented in a number of primary MDs and is even the dominant feature in some of them. The myocardium can be affected in the form of a dilated cardiomyopathy while the conduction system can be affected resulting in arrhythmias and conduction defects. Many patients with MD die because of cardiac complications like sudden cardiac death or congestive heart failure. Detailed clinical data about cardiac involvement are available for Duchenne/Becker MD, Emery-Dreifuss MD, myotonic dystrophy, and the different limb girdle MDs. Cardiac manifestations were also found in congenital MD, central core disease, proximal myotonic myopathy, and nemaline myopathy. No data about cardiac abnormalities are available in oculopharyngeal MD and rippling muscle disease. The heart of patients with primary MD should be carefully investigated because of the life-threatening events caused by cardiac complications. There is a strong need for a close collaboration between neurologists and cardiologists in order to provide optimal disease management for the affected patients. PMID:15868359

Perrot, A; Spuler, S; Geier, C; Dietz, R; Osterziel, K J

2005-05-01

55

Ocular manifestations of HIV infection.  

PubMed Central

OBJECTIVE: To evaluate the frequency of ocular complications and the clinical outcomes of these complications in patients with various stages of HIV infection. METHODS: Retrospective review of all HIV-infected patients seen in an AIDS ophthalmology clinic from November 1983 through December 31, 1992. RESULTS: Eleven-hundred sixty-three patients were seen for ophthalmologic evaluation. Of these, 781 had the acquired immune deficiency syndrome (AIDS), 226 had symptomatic HIV infection (AIDs-related complex [ARC]), and 156 had asymptomatic HIV infection. Non-infectious HIV retinopathy was the most common ocular complication, affecting 50% of the patients with AIDS, 34% of the patients with ARC, and 3% of the patients with asymptomatic HIV infection. Cytomegalovirus (CMV) retinitis was the most common opportunistic ocular infection, affecting 37% of the patients with AIDS. Other opportunistic ocular infections, including ocular toxoplasmosis, varicella zoster virus retinitis, and Pneumocystis choroidopathy were all much less common, each occurring in < or = 1% of the patients with AIDS. Treatment of CMV retinitis with either foscarnet or ganciclovir was successful in initially controlling the retinitis. However, relapse represented a significant problem and required frequent re-inductions. As a consequence of the retinal damage associated with relapse, loss of visual acuity occurred. The median time to a visual acuity of 20/200 or worse for all eyes with CMV retinitis was 13.4 months, and the median time to a visual acuity of 20/200 or worse in the better eye was 21.1 months. At last follow-up, 75% of the patients had a final visual acuity of 20/40 or better in at least one eye. Retinal detachments were a frequent ophthalmologic complication of CMV retinitis with a cumulative probability of a retinal detachment in at least one eye of 57% at 12 months after the diagnosis of CMV retinitis. Herpes zoster ophthalmicus developed in 3% of the overall series and was seen in all stages of HIV infection. Fifty-six percent of the cases of ocular toxoplasmosis had simultaneous toxoplasmic cerebritis. Ocular toxoplasmosis responded to standard anti-microbial therapy. Varicella zoster virus retinitis, when manifested by the acute retinal necrosis (ARN) syndrome, responded to intravenous acyclovir therapy. Conversely, in a limited number of patients with the progressive outer retinal necrosis syndrome, the disease responded poorly to intravenous acyclovir therapy, but appeared to respond to combination foscarnet and acyclovir therapy. Neuro-ophthalmic lesions were present in 6% of the patients with AIDS. The most common cause of a neuro-ophthalmic lesion was cryptococcal meningitis, and 25% of the patients with cryptococcal meningitis developed a neuro-ophthalmic complication. CONCLUSIONS: Ocular manifestations are common in patients with AIDS. CMV retinitis represented a major vision-threatening problem in these patients. While available therapy was successful in initially controlling the retinitis, the phenomenon of relapse resulted in some degree of long-term visual loss. Preservation of the patient's visual acuity in at least one eye was generally successful. Other opportunistic ocular infections were substantially less common than CMV retinitis but require aggressive therapy. Images FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6

Jabs, D A

1995-01-01

56

Protean Manifestations of Neonatal Hyperinsulinism  

PubMed Central

Endogenous hyperinsulinism is the leading cause of persistent hypoglycemia in children under one year of age. Classically, the symptoms of neonatal hypoglycemia have been referable to central nervous system dysfunction, with seizures described in nearly all patients. Our experience with eight neonates emphasizes the protean manifestations of this disease. One patient presented with a maternal history of diuretic use, and developed asymptomatic hyperinsulinism documented by provocative testing. The hyperinsulinism cleared after two weeks of medical therapy. This transient hyperinsulinism may have been secondary to use of a thiazide-type diuretic. A second patient presented, as a neonate, with a large abdominal mass but no seizure activity. Exploratory laparotomy revealed an 11 x 5 x 3 cm pancreatic tumor, which required splenectomy, 60% gastrectomy and duodenectomy for removal. Histologic examination demonstrated an insulin-secreting hamartoma. A third patient died suddenly without prior symptoms, and was found to have striking nesidioblastosis on pathologic examination. One infant presented with absence of the abdominal musculature (prune belly syndrome) and features of the Beck-with-Wiedeman syndrome, as well as profound hypoglycemia. Only three patients had seizures, and an additional patient had jitteriness. Pathologic diagnoses were: nesidioblastosis (n = 2); islet cell hyperplasia (n = 1); adenoma (n = 1); hamartoma (n = 1); transient hyperinsulinism (n = 1). One patient's pancreas showed areas of nesidioblastosis, islet cell hyperplasia, and a discrete adenoma in the region of the common bile duct. Careful diagnostic testing is essential in these patients, inasmuch as hypoglycemia is poorly tolerated by neonates and infants. Using the diagnostic algorithm presented here, all patients' endogenous hyperinsulinism was documented quickly and efficiently. Recognition of the broad spectrum of symptoms with which these patients may present is essential if serious neurologic sequelae are to be avoided.

Mayer, Thom; Matlak, Michael E.; Lowry, Stephen F.; Gooch, W. Manford; Johnson, Dale G.

1981-01-01

57

Shoulder manifestations of diabetes mellitus.  

PubMed

The musculoskeletal system can be affected by diabetes in a number of ways. The shoulder is one of the frequently affected sites. One of the rheumatic conditions caused by diabetes is frozen shoulder (adhesive capsulitis), which is characterized by pain and severe limited active and passive range of motion of the glenohumeral joint, particularly external rotation. This disorder has a clinical diagnosis and the treatment is based on physiotherapy, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroid injections and, in refractory cases, surgical resolution. As with adhesive capsulitis, calcific periarthritis of the shoulder causes pain and limited joint mobility, although usually it has a better prognosis than frozen shoulder. Reflex sympathetic dystrophy, also known as shoulder-hand syndrome, is a painful syndrome associated with vasomotor and sudomotor changes in the affected member. Diabetic amyotrophy usually affects the peripheral nerves of lower limbs. However, when symptoms involve the shoulder girdle, it must be considered in the differential diagnosis of shoulder painful conditions. Osteoarthritis is the most common rheumatic condition. There are many risk factors for shoulder osteoarthritis including age, genetics, sex, weight, joint infection, history of shoulder dislocation, and previous injury, in older age patients, diabetes is a risk factor for shoulder OA. Treatment options include acetaminophen, NSAIDs, short term opiate, glucosamine and chondroitin. Corticosteroid injections and/or injections of hyaluronans could also be considered. Patients with continued disabling pain that is not responsive to conservative measures may require surgical referral. The present review will focus on practice points of view about shoulder manifestations in patients with diabetes. PMID:20701586

Garcilazo, Cintia; Cavallasca, Javier A; Musuruana, Jorge L

2010-09-01

58

78 FR 11877 - Announcement of Requirements Gathering Meetings for the Electronic Manifest (e-Manifest) System  

Federal Register 2010, 2011, 2012, 2013

...with manifest data and processes as well as staff that have specialized understanding of the manifest program and business process. Potentially interested parties may include but are not limited to: IT staff personnel supporting hazardous...

2013-02-20

59

Pulmonary manifestations of systemic autoimmune disease  

Microsoft Academic Search

Patients with systemic autoimmune disease may present with a number of different pulmonary manifestations. In order to recognise, diagnose and manage these manifestations, it is necessary to have a working knowledge of the anatomy and physiology of the thorax. This chapter will describe the clinical symptoms and clinical examination findings in patients who may have underlying pulmonary disease. It will

Sisa Grubnic

2004-01-01

60

40 CFR 761.215 - Manifest discrepancies.  

Code of Federal Regulations, 2013 CFR

...quantity or type of PCB waste designated on the manifest or shipping paper, and the quantity and type of PCB waste a facility actually receives...manifest or shipping paper at issue. (d)(1) Upon rejecting the PCB waste, the facility must...

2013-07-01

61

40 CFR 265.72 - Manifest discrepancies.  

Code of Federal Regulations, 2013 CFR

...quantity or type of hazardous waste designated on the manifest or shipping paper, and the quantity and type of hazardous waste a facility actually receives...the manifest or shipping paper at issue. (d)(1) Upon rejecting waste or identifying a...

2013-07-01

62

40 CFR 264.72 - Manifest discrepancies.  

Code of Federal Regulations, 2013 CFR

...quantity or type of hazardous waste designated on the manifest or shipping paper, and the quantity and type of hazardous waste a facility actually receives...the manifest or shipping paper at issue. (d)(1) Upon rejecting waste or identifying a...

2013-07-01

63

Clinical manifestations of zinc deficiency.  

PubMed

The essentiality of zinc for humans was recognized in the early 1960s. The causes of zinc deficiency include malnutrition, alcoholism, malabsorption, extensive burns, chronic debilitating disorders, chronic renal diseases, following uses of certain drugs such as penicillamine for Wilson's disease and diuretics in some cases, and genetic disorders such as acrodermatitis enteropathica and sickle cell disease. In pregnancy and during periods of growth the requirement of zinc is increased. The clinical manifestations in severe cases of zinc deficiency include bullous-pustular dermatitis, alopecia, diarrhea, emotional disorder, weight loss, intercurrent infections, hypogonadism in males; it is fatal if unrecognized and untreated. A moderate deficiency of zinc is characterized by growth retardation and delayed puberty in adolescents, hypogonadism in males, rough skin, poor appetite, mental lethargy, delayed wound healing, taste abnormalities, and abnormal dark adaptation. In mild cases of zinc deficiency in human subjects, we have observed oligospermia, slight weight loss, and hyperammonemia. Zinc is a growth factor. Its deficiency adversely affects growth in many animal species and humans. Inasmuch as zinc is needed for protein and DNA synthesis and for cell division, it is believed that the growth effect of zinc is related to its effect on protein synthesis. Whether or not zinc is required for the metabolism of somatomedin needs to be investigated in the future. Testicular functions are affected adversely as a result of zinc deficiency in both humans and experimental animals. This effect of zinc is at the end organ level; the hypothalamic-pituitary axis is intact in zinc-deficient subjects. Inasmuch as zinc is intimately involved in cell division, its deficiency may adversely affect testicular size and thus affect its functions. Zinc is required for the functions of several enzymes and whether or not it has an enzymatic role in steroidogenesis is not known at present. Thymopoeitin, a hormone needed for T-cell maturation, has also been shown to be zinc dependent. Zinc deficiency affects T-cell functions and chemotaxis adversely. Disorders of cell-mediated immune functions are commonly observed in patients with zinc deficiency. Zinc is beneficial for wound healing in zinc-deficient subjects. In certain zinc-deficient subjects, abnormal taste and abnormal dark adaptation have been noted to reverse with zinc supplementation. PMID:3896271

Prasad, A S

1985-01-01

64

Oral manifestations of coeliac disease.  

PubMed Central

SUMMARY Aim. The aim of the clinical-statistic study was to evaluate the prevalence of the different oral manifestations in a sample of coeliac patients, in comparison with a control group of healthy subjects. Moreover, a second objective was to determine if the clinical oral examination is useful as a diagnostic tool of screening for atypical forms of coeliac disease (CD). Methods. The enrolment of 300 coeliac patients, aged between 4 and 13 years (mean age 8.16), was carried out at the Pediatric Dentistry Unit in patients sent from the Pediatric Gastroenterology Unit of the PTV Hospital, University of Rome Tor Vergata. The control group was composed of 300 healthy subjects, age-matched (mean age 8.29), enrolled from the Pediatric Dentistry Unit. The patients were examined for hard tissues (enamel hypoplasia, dental caries), soft tissues (recurrent aphthous stomatitis RAS, atrophic glossitis, geographic tongue) and delay dental eruption. Enamel defects were classified according to Aine’s criteria, while dental caries was recorded as dmft/DMFT indices. Statistical analysis was carried out by using SPSS/PC+ Software. Differences between case and control groups were tested using Paired samples T-test, and Chi-Square Test, depending on the variable considered. The minimal level of significance of the differences was fixed at p?0.05 for all the procedures. Results. Statistical differences between groups were observed for the prevalence of enamel defects (p=0.0001), RAS (p=0.005), delay in dental eruption (p=0.0001), but not for the prevalence of atrophic glossitis (p=0.664). Differences in symmetrical distribution and a chronologic coherence of enamel defects were statistically significant between CD and control groups (p=0.0001). Regarding dental caries, the coeliac patients had higher indexes of caries than healthy subjects, both in deciduous teeth (dmft 2.31±1.84 vs 1.42±1.13; p= 0.021) and permanent teeth (DMFT 2.97±1.74 vs 1.74±1.64; p=0.0001). Conclusions. The clinical oral examination should be considered a diagnostic tool for the characterization of subjects affected by silent-atypical forms of CD.

COSTACURTA, M.; MATURO, P.; BARTOLINO, M.; DOCIMO, R.

2010-01-01

65

Genetic alterations in syndromes with oral manifestations  

PubMed Central

Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome.

Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J.

2013-01-01

66

MANIFEST instrument concept and related technologies  

NASA Astrophysics Data System (ADS)

The Australian Astronomical Observatory (AAO) has recently completed a feasibility study for a fiber-positioner facility proposed for the Giant Magellan Telescope (GMT), called MANIFEST (the Many Instrument Fiber System). The MANIFEST instrument takes full advantage of the wide-field focal plane to efficiently feed other instruments. About 2000 individually deployable fiber units are envisaged, with a wide variety of aperture types (single-aperture, image- or pupil-slicing, IFU). MANIFEST allows (a) full use of the GMT's 20' field-of-view, (b) a multiplexed IFU capability, (c) greatly increased spectral resolution via image-slicing, (d) the possibility of OH-suppression in the near-infrared.

Goodwin, Michael; Brzeski, Jurek; Case, Scott; Colless, Matthew; Farrell, Tony; Gers, Luke; Gilbert, James; Heijmans, Jeroen; Hopkins, Andrew; Lawrence, Jon; Miziarski, Stan; Monnet, Guy; Muller, Rolf; Saunders, Will; Smith, Greg; Tims, Julia; Waller, Lewis

2012-09-01

67

Genetic alterations in syndromes with oral manifestations.  

PubMed

Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome. PMID:24379857

Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J

2013-11-01

68

Rheumatic manifestations of dysproteinemias and lymphoproliferative disorders.  

PubMed

Rheumatic manifestations may be the presenting features of dysproteinemias and lymphoproliferative disorders. Disease or therapy-related complications may mimic a number of primary rheumatic syndromes. This article emphasizes clinical aspects pertaining to prompt diagnosis and therapy. PMID:8907064

Duna, G F; Cash, J M

1996-02-01

69

Important cutaneous manifestations of inflammatory bowel disease  

PubMed Central

Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition.

Trost, L; McDonnell, J

2005-01-01

70

Carpal Tunnel Syndrome as Manifestation of Sarcoidosis.  

National Technical Information Service (NTIS)

Two patients with bilateral carpal tunnel syndrome are reported. One had coeistent psoriasis raising the question as to the etiology of his joint, muscle, and carpal tunnel manifestations. The other had mild diabetes mellitus. In both, evidence favored th...

G. E. Shambaugh W. J. Cirksena K. L. Newcomer

1964-01-01

71

Metabolic and hormonal signatures in pre-manifest and manifest Huntington's disease patients  

PubMed Central

Huntington's disease (HD) is an inherited neurodegenerative disorder typified by involuntary body movements, and psychiatric and cognitive abnormalities. Many HD patients also exhibit metabolic changes including progressive weight loss and appetite dysfunction. Here we have investigated metabolic function in pre-manifest and manifest HD subjects to establish an HD subject metabolic hormonal plasma signature. Individuals at risk for HD who have had predictive genetic testing showing the cytosine-adenine-guanine (CAG) expansion causative of HD, but who do not yet present signs and symptoms sufficient for the diagnosis of manifest HD are said to be “pre-manifest.” Pre-manifest and manifest HD patients, as well as both familial and non-familial controls, were evaluated for multiple peripheral metabolism signals including circulating levels of hormones, growth factors, lipids, and cytokines. Both pre-manifest and manifest HD subjects exhibited significantly reduced levels of circulating growth factors, including growth hormone and prolactin. HD-related changes in the levels of metabolic hormones such as ghrelin, glucagon, and amylin were also observed. Total cholesterol, HDL-C, and LDL-C were significantly decreased in HD subjects. C-reactive protein was significantly elevated in pre-manifest HD subjects. The observation of metabolic alterations, even in subjects considered to be in the pre-manifest stage of HD, suggests that in addition, and prior, to overt neuronal damage, HD affects metabolic hormone secretion and energy regulation, which may shed light on pathogenesis, and provide opportunities for biomarker development.

Wang, Rui; Ross, Christopher A.; Cai, Huan; Cong, Wei-Na; Daimon, Caitlin M.; Carlson, Olga D.; Egan, Josephine M.; Siddiqui, Sana; Maudsley, Stuart; Martin, Bronwen

2014-01-01

72

Rheumatic manifestations of inflammatory bowel disease  

Microsoft Academic Search

This article reviews the literature concerning rheu- matic manifestations of inflammatory bowel disease (IBD), including common immune-mediated pathways, frequency, clinical course and therapy. Musculoskel- etal complications are frequent and well-recognized manifestations in IBD, and affect up to 33% of pa- tients with IBD. The strong link between the bowel and the osteo-articular system is suggested by many clinical and experimental

Tatiana Sofía Rodríguez-Reyna; Cynthia Martínez-Reyes; Jesús Kazúo Yamamoto-Furusho

2009-01-01

73

Genetic Diseases and Associated Urologic Manifestations  

Microsoft Academic Search

\\u000a The diagnosis of a genetic disease is usually made through a combination of clinical characteristics and genetic testing.\\u000a It is important that urologists be able to recognize the cardinal features of these disorders and identify the associated\\u000a urologic manifestations. The purpose of this chapter is to provide an overview of the genetic disorders with urologic manifestations.

Jeffrey S. Palmer; Katherine C. Hubert

74

[Under-evaluated extrahepatic manifestations of cholestasis].  

PubMed

Biliary stasis can occur in many different diseases. Pruritus, metabolic bone disease, deficiencies of fat-soluble vitamins, steatorrhea, hyperlipidemia and fatigue represent the major extra-hepatic manifestations of cholestatic liver disease that considerably affect the patient's quality of life. The present article reviews pathogenetic and clinical aspects of and current therapeutic approaches to extra-hepatic manifestations of cholestatic liver disease. PMID:19292075

Prelipcean, Cristina Cijevschi; Mihai, C?t?lina; Gog?lniceanu, P; Drug, V L; Chiril?, N

2006-01-01

75

Unfamiliar Manifestations of Anti-tubercular Therapy  

PubMed Central

Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved.

Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi

2014-01-01

76

Skeletal scintigraphy manifestations of hematologic disorders  

PubMed Central

Skeletal manifestations are common in hematologic disorders. Benign entities such as Sickle cell disease develop microvascular embolization causing skeletal crisis. Leukemia, acute myeloblastic or lymphoblastic may develop bone marrow infarcts. Compromised immunity makes them susceptible to secondary infection leading to osteomyelitis or septic arthritis. Exposure to steroids may lead to osteonecrosis in these cases. Presented here is an atlas of various scintigraphic skeletal manifestations encountered over the past 10 years, in hematologic disorders.

Solav, Shrikant V; Bhandari, Ritu; Solav, Pallavi

2012-01-01

77

8 CFR 251.1 - Arrival manifests and lists.  

Code of Federal Regulations, 2010 CFR

...2009-01-01 2009-01-01 false Arrival manifests and lists. 251.1 Section...SECURITY IMMIGRATION REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.1 Arrival manifests and lists. (a)...

2009-01-01

78

8 CFR 251.1 - Arrival manifests and lists.  

Code of Federal Regulations, 2010 CFR

...2010-01-01 2010-01-01 false Arrival manifests and lists. 251.1 Section...SECURITY IMMIGRATION REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.1 Arrival manifests and lists. (a)...

2010-01-01

79

Autoimmune manifestations in patients with visceral leishmaniasis.  

PubMed

Visceral leishmaniasis (VL) is a vector-borne protozoal infection caused by replication of Leishmania species in macrophages. VL is characterized by fever, hepatosplenomegaly and cytopenia. Apart from those classic clinical characteristics, VL has been associated with autoimmune clinical and laboratory features. Reported herein are 16 consecutive patients with VL who were checked for laboratory autoimmune manifestations. A variety of autoimmune antibodies including elevated titers of antinuclear antibodies and rheumatoid factor were detected in all patients. Of note, no laboratory autoimmune manifestations were detected in the seven patients who were re-evaluated 3 months after therapy. It is concluded that autoimmune laboratory manifestations during VL infection are common. These may mistakenly lead to diagnosis of an autoimmune disorder. PMID:22516744

Liberopoulos, Evangelos; Kei, Anastazia; Apostolou, Fotini; Elisaf, Moses

2013-08-01

80

Oral manifestations of hematologic and nutritional diseases.  

PubMed

Oral manifestations of hematologic and nutritional deficiencies can affect the mucous membranes, teeth, periodontal tissues, salivary glands, and perioral skin. This article reviews common oral manifestations of hematologic conditions starting with disorders of the white blood cells including cyclic hematopoiesis (cyclic neutropenia), leukemias, lymphomas, plasma cell dyscrasias, and mast cell disorders; this is followed by a discussion of the impact of red blood cell disorders including anemias and less common red blood cell dyscrasias (sickle cell disease, hemochromatosis, and congenital erythropoietic porphyria) as well as thrombocytopenia. Several nutritional deficiencies exhibit oral manifestations. The authors specifically discuss the impact of water-soluble vitamins (B2, B3, B6, B9, B12, and C), fat-soluble vitamins (A, D, and K) and the eating disorders anorexia nervosa and bulimia nervosa on the oral mucosa. PMID:21093629

Schlosser, Bethanee J; Pirigyi, Megan; Mirowski, Ginat W

2011-02-01

81

Bodily manifestations in the psychoanalytic process.  

PubMed

The broadening scope of psychoanalysis has brought to the fore patients whose unconscious conflicts tend to be literally played out on the stage of the body. In these cases, the body seems to be predominantly used in a concrete, not symbolic, way in order to express underlying conflicts. In a similar vein, transference and countertransference can be manifested via the body. The authors briefly discuss some of the literature on body-mind issues, and then present an extended case report to illustrate bodily manifestations in the psychoanalytic process. PMID:23038903

Vartzopoulos, Ioannis; Beratis, Stavroula

2012-07-01

82

The Chronic Gastrointestinal Manifestations of Chagas Disease  

PubMed Central

Chagas disease is an infectious disease caused by the protozoan Trypanosoma cruzi. The disease mainly affects the nervous system, digestive system and heart. The objective of this review is to revise the literature and summarize the main chronic gastrointestinal manifestations of Chagas disease. The chronic gastrointestinal manifestations of Chagas disease are mainly a result of enteric nervous system impairment caused by T. cruzi infection. The anatomical locations most commonly described to be affected by Chagas disease are salivary glands, esophagus, lower esophageal sphincter, stomach, small intestine, colon, gallbladder and biliary tree. Chagas disease has also been studied in association with Helicobacter pylori infection, interstitial cells of Cajal and the incidence of gastrointestinal cancer.

Matsuda, Nilce Mitiko; Miller, Steven M.; Evora, Paulo R. Barbosa

2009-01-01

83

Pulmonary manifestations of sickle cell disease  

PubMed Central

Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed.

Siddiqui, A; Ahmed, S

2003-01-01

84

Musculoskeletal manifestations of hemophilia: imaging features.  

PubMed

The purpose of this pictorial assay is to demonstrate the imaging features of the various musculoskeletal manifestations of hemophilia, an X-linked disorder. Depending on the site of recurrent bleeding, musculoskeletal manifestation can be in the form of hemophilic arthropathy and/or soft tissue, intraosseous, or subperiosteal pseudotumors. Radiography, sonography, computed tomography, and especially magnetic resonance imaging help in the evaluation of hemophilic arthropathy and pseudotumors, providing accurate diagnosis, thus avoiding inadvertent procedures and related complications. Treatment includes replacement of clotting factors either continuously or when indicated, radionuclide or open synovectomy in cases of disabling arthropathy. PMID:21787985

Jaganathan, Sriram; Gamanagatti, Shivanand; Goyal, Ankur

2011-01-01

85

Manifestation Determination as a Golden Fleece.  

ERIC Educational Resources Information Center

Manifestation determination is a mandated provision for deciding whether a student's misbehavior is related to his disability and, consequently, whether cessation of services is allowed. This article critiques this provision by reviewing relevant case law and legislation, examining the mandate's social context, and questioning the validity of…

Katsiyannis, Antonis; Maag, John W.

2001-01-01

86

Clinical Manifestations of Superior Semicircular Canal Dehiscence  

Microsoft Academic Search

Objectives\\/Hypotheses: To determine the symp- toms, signs, and findings on diagnostic tests in pa- tients with clinical manifestations of superior canal dehiscence. To investigate hypotheses about the ef- fects of superior canal dehiscence. To analyze the out- comes in patients who underwent surgical repair of the dehiscence. Study Design: Review and analysis of clinical data obtained as a part of

Lloyd B. Minor

2005-01-01

87

Clinical Manifestations of Cerebrovascular Hypoplasias in Childhood  

Microsoft Academic Search

The aim of the study was to analyze cerebrovascular hypoplasia in childhood and its clinical manifestations in a clinical population of 205 children aged 3 to 14 years. Cerebrovascular hypoplasias were diagnosed using angiography (n=63), magnetic resonance angiography, and transcranial Doppler ultrasonography. Hypoplasias were localized in the internal carotid artery in 41.9% of patients, in the middle cerebral artery in

Veneta Bojinova; Petia Dimova; Liliana Belopitova

2000-01-01

88

Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation  

PubMed Central

Propylthiouracil (PTU) is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA) in few patients with Graves’ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves’ disease.

Ayturk, Semra; Demir, Mustafa Volkan; Yaylac?, Selcuk; Tamer, Ali

2013-01-01

89

Oculorenal manifestations in systemic autoimmune diseases  

Microsoft Academic Search

Vasculitides form a heterogeneous group of diseases characterized by blood-vessel inflammation and necrosis. They have a wide spectrum of manifestations because of the involvement of arteries and other vessels of various sizes and locations. Early diagnosis and prompt treatment may decrease the morbidity and mortality associated with these disorders. Examination of the eye and kidney should be performed routinely in

Hassane Izzedine; Bahram Bodaghi; Vincent Launay-Vacher; Gilbert Deray

2004-01-01

90

The musculoskeletal manifestations of Werner's syndrome.  

PubMed

Werner's syndrome is a rare condition usually presenting as premature ageing in adults. Over a period of 30 years we have followed two siblings with extensive musculoskeletal manifestations including a soft-tissue tumour, insufficiency fractures, nonunion and tendonitis, with associated problems of management. The literature is reviewed. PMID:10990317

Walton, N P; Brammar, T J; Coleman, N P

2000-08-01

91

Skin Manifestations in Solid Organ Transplant Recipients  

Microsoft Academic Search

The clinical spectrum of the most significant dermatological complications of solid organ transplantations is presented in an attempt to enhance the awareness among dermatologists and other physicians of the importance of careful dermatological monitoring of organ transplant recipients for early diagnosis and prompt treatment of these manifestations.

D. Tsambaos; G. Badavanis

2001-01-01

92

Osteoarticular manifestations in sickle-cell disorders  

Microsoft Academic Search

Summary  The osteoarticular manifestations of the main sickling disorders (homozygous sickle cell-HbSS anaemia-, double heterozygous-HbS- thalassaemia, double heterozygous HbS-C disease and sickle cell-trait) are reviewed; They may be grouped into 3 main categories: vaso-occlusive, infective and metabolic. The pathophysiology and pathogenesis as well as the treatment are discussed.

Phaedon Kaklamanis

1984-01-01

93

Gestural Manifestation of Knowledge in Conceptual Frames  

ERIC Educational Resources Information Center

Frames are cognitive structures of knowledge grounded in people's social interaction in recurrent sociocultural activities or individual incidences. Discussion concerning the knowledge in frames largely focuses on its linguistic manifestation with regard to roles and role relations in scenes. Little attention has been paid to the scriptal…

Chui, Kawai

2012-01-01

94

Clinical manifestations and evaluation of isolated lissencephaly  

Microsoft Academic Search

Lissencephaly (“smooth brain”) is a brain malformation characterized by a smooth cerebral surface, incomplete neuronal migration, and secondary abnormalities such as mental retardation, seizures, and minor facial dysmorphisms. Recent reports have produced evidence supporting several different causes including submicroscopic deletions in chromosome band 17p13.3, autosomal recessive inheritance, intrauterine infection, and intrauterine perfusion failure. We describe the clinical manifestations in seven

L. Pavone; R. Rizzo; W. B. Dobyns

1993-01-01

95

Unusual manifestation of Marden-Walker syndrome  

PubMed Central

Marden-Walker syndrome (MWS) is characterized by multiple joint contractures, a mask-like face with blepharophimosis, micrognathia, high-arched or cleft palate, low-set ears, decreased muscular bulk, arachnodactyly, and kyphoscoliosis. We report a case of MWS along with unusual manifestation of neurological, cardiovascular, and genitourinary system.

Taksande, Amar M.; Vilhekar, K. Y.

2012-01-01

96

Ocular manifestations of systemic lupus erythematosus  

Microsoft Academic Search

Ocular manifestations of lupus are fairly common, may be the presenting feature of the disease and can be sight-threatening. Almost any part of the eye and visual pathway can be affected by inflammatory or thrombotic processes. Ocular pain and visual impairment require urgent assessment by an ophthalmologist. Infection should be excluded. Optic neuritis and ischaemic optic neuropathy may be difficult

R. R. Sivaraj; O. M. Durrani; A. K. Denniston; P. I. Murray; Caroline Gordon

2007-01-01

97

Hematologic Manifestation of Childhood Celiac Disease  

Microsoft Academic Search

We wanted to describe the hematologic manifestations of celiac disease (CD) in childhood. This study included 22 children with CD in whom the disease remained undiagnosed until they had presented with hematological abnormalities, such as anemia, thrombocytopenia, leukopenia or prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT). Anemia was present alone in 19 (86.3%) patients, and leukopenia coexisted

Tunc Fisgin; Nese Yarali; Feride Duru; Belgin Usta; Abdurrahman Kara

2004-01-01

98

Clinical manifestation of opisthorchiasis and treatment.  

PubMed

This paper presents an overview of recent progress in the clinical study of opisthorchiasis and treatment. Details of clinical manifestations of opisthorchiasis, hepatobiliary diseases and liver functions, community-based ultrasound study, treatment efficacy, and future study are discussed. PMID:14611876

Mairiang, Eimorn; Mairiang, Pisaln

2003-11-01

99

Cardiovascular manifestations of the emerging dengue pandemic.  

PubMed

Dengue is one of the most important emerging viral diseases globally. The majority of symptomatic infections result in a relatively benign disease course. However, a small proportion of patients develop severe clinical manifestations, including bleeding, organ impairment, and endothelial dysfunction with increased capillary permeability causing hypovolaemic shock that can lead to cardiovascular collapse. Evidence is increasing that dengue can also cause myocardial impairment, arrhythmias and, occasionally, fulminant myocarditis. No antiviral agents or vaccines are licensed for dengue, and treatment remains supportive with judicious fluid replacement for patients with severe disease. Defining the role of cardiac dysfunction in the haemodynamic compromise of severe dengue has potentially important management implications. In this Review, we will outline the current understanding of the cardiovascular manifestations of dengue, including myocardial and vascular involvement, and conclude with a discussion of the available therapeutic options and potential future research directions. PMID:24710495

Yacoub, Sophie; Wertheim, Heiman; Simmons, Cameron P; Screaton, Gavin; Wills, Bridget

2014-06-01

100

PATTERN OF CUTANEOUS MANIFESTATIONS IN DIABETES MELLITUS  

PubMed Central

Background: Diabetes mellitus affects individuals of all ages and socioeconomic status. Skin is affected by the acute metabolic derangements as well as by chronic degenerative complications of diabetes. Aims: To evaluate the prevalence of skin manifestations in patients with diabetes mellitus. To analyze the prevalence and pattern of skin disorders among diabetic patients from this region of Western Himalayas. Materials and Methods: One hundred consecutive patients with the diagnosis of diabetes mellitus and having skin lesions, either attending the diabetic clinic or admitted in medical wards were included in this study. Results: The common skin disorders were: Xerosis (44%), diabetic dermopathy (36%), skin tags (32%), cutaneous infections (31%), and seborrheic keratosis (30%). Conclusion: Skin is involved in diabetes quite often and the manifestations are numerous. High prevalence of xerosis in our diabetic population is perhaps due to cold and dry climatic conditions in the region for most of the time in the year.

Goyal, Abhishek; Raina, Sujeet; Kaushal, Satinder S; Mahajan, Vikram; Sharma, Nand Lal

2010-01-01

101

Clinical Manifestations and Diagnosis of Acromegaly  

PubMed Central

Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal.

Lugo, Gloria; Pena, Lara; Cordido, Fernando

2012-01-01

102

Imaging lung manifestations of HIV/AIDS  

PubMed Central

Advances in our understanding of human immunodeficiency virus (HIV) infection have led to improved care and incremental increases in survival. However, the pulmonary manifestations of HIV/acquired immunodeficiency syndrome (AIDS) remain a major cause of morbidity and mortality. Respiratory complaints are not infrequent in patients who are HIV positive. The great majority of lung complications of HIV/AIDS are of infectious etiology but neoplasm, interstitial pneumonias, Kaposi sarcoma and lymphomas add significantly to patient morbidity and mortality. Imaging plays a vital role in the diagnosis and management of lung of complications associated with HIV. Accurate diagnosis is based on an understanding of the pathogenesis of the processes involved and their imaging findings. Imaging also plays an important role in selection of the most appropriate site for tissue sampling, staging of disease and follow-ups. We present images of lung manifestations of HIV/AIDS, describing the salient features and the differential diagnosis.

Allen, Carolyn M.; AL-Jahdali, Hamdan H.; Irion, Klaus L.; Al Ghanem, Sarah; Gouda, Alaa; Khan, Ali Nawaz

2010-01-01

103

Gastrointestinal Manifestations in Systemic Autoimmune Diseases  

PubMed Central

ABSTRACT In an autoimmune disease, the immune system attacks and harms the body's own tissues. The systemic autoimmune diseases include collagen vascular diseases, the systemic vasculitides, Wegener granulomatosis, and Churg-Strauss syndrome. These disorders can involve any part of the gastrointestinal tract, hepatobiliary system and pancreas. They can cause a variety of gastrointestinal manifestations that are influenced by the pathophysiologic characteristics of the underlying disease process. There is a wide variation of gastrointestinal manifestations from these autoimmune disorders including, but not limited to: oral ulcers, dysphagia, gastroesophageal reflux disease, abdominal pain, constipation, diarrhea, fecal incontinence, pseudo-obstruction, perforation and gastrointestinal bleeding. Clinical workup should be initiated by the patient's subjective complaints. In this review, we analyze the effects of autoimmune diseases on the gastrointestinal tract.

COJOCARU, M.; COJOCARU, Inimioara Mihaela; SILOSI, Isabela; VRABIE, Camelia Doina

2011-01-01

104

Oral Crohn's disease without intestinal manifestations.  

PubMed

Crohn's disease is a granulomatous inflammatory bowel disease and was described in 1932 as a chronic granulomatous disorder of the terminal ileum and is now considered a distinct member of the inflammatory bowel disease family. It may affect any part of the gastrointestinal tract. Oral Crohn's disease has been reported frequently in the last three decades with or without intestinal manifestations. In the latter case, it is considered as one of the orofacial granulomatosis. There has been much doubt whether intestinal manifestations of Crohn's disease will eventually develop in the orofacial granulomatosis. We present a female patient aged 22 years with prominent clinical findings such as persistent swelling of lower and upper lip with fissuring and angular cheilitis, granulomatous gingival enlargement, and cobblestone or corrugated appearance of labial mucosa, which are suggestive of Crohn's disease, but with no evidence of other gastrointestinal involvement. The patient underwent surgical treatment with external gingivectomy procedure. A 6-month follow-up showed minimal recurrence. PMID:23066305

Harikishan, Gingisetty; Reddy, Nagate Raghavendra; Prasad, Harikrishnan; Anitha, Subappa

2012-08-01

105

Acute dacryocystitis: another clinical manifestation of sporotrichosis  

PubMed Central

Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis.

Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordao, Livia; Zancope-Oliveira, Rosely Maria; do Valle, Antonio Carlos Francesconi; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

2013-01-01

106

Acute dacryocystitis: another clinical manifestation of sporotrichosis.  

PubMed

Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis. PMID:24810176

Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordão, Livia; Zancopé-Oliveira, Rosely Maria; do Valle, Antonio Carlos Francesconi; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

2014-04-01

107

Senior-Loken syndrome with unusual manifestations.  

PubMed

The Senior-Loken syndrome, also called Hereditary Renal Retinal Dystrophy is a rare disorder that combines a juvenile nephronophthisis-like disease with tapetoretinal degeneration. We are reporting a family in which two, possibly three siblings are affected by this disorder. In addition to these two documented components of the disease, our patients also have unusual manifestations: short stature; skeletal abnormalities in the form of kyphoscoliosis, short metacarpals; and cutis laxa-unreported so far. PMID:11229290

Singh, N P; Anuradha, S; Gupta, S; Rizvi, S N; Arora, R

1998-08-01

108

Drug Hypersensitivity: Clinical Manifestations and Diagnosis  

Microsoft Academic Search

Drug hypersensitivity, including the allergic type, is one of the side effects of drugs and is a daily worry for the clinician.\\u000a Even though urticarial and maculopapular eruptions are the most frequent manifestations, there are many clinical forms, mirroring\\u000a many distinct pathophysiological events. The diagnosis of drug hypersensitivity often relies on clinical histories, skin tests,\\u000a patch tests, and a few

Pascal Demoly; Antonino Romano

109

[Extrahepatic manifestations of hepatitis C virus infection].  

PubMed

HCV virus is associated with various immunological disorders. Some of them like mixed cryoglobulinemia, are proved by molecular biology and virology. Others are presumed auto-immune: auto-antibodies production (antinuclear, anti-smooth muscle, anti-liver-kidney microsomal antibodies...) has generally no pathological significance; however, true auto-immune diseases such as auto-immune hepatitis type 1 or 2, Sjögren's syndrome, lichen planus and auto-immune thyroiditis can be associated with HCV related liver disease. Finally, the association of some extra-hepatic manifestations like porphyria cutanea tarda with hepatitis C virus are only based on epidemiological data. Alpha interferon, the reference treatment of chronic hepatitis C, can be efficient on manifestations such as cryoglobulinemia which are directly linked to the virus. However, because of its immunological effect, the same treatment can severely worsen auto-immunological diseases associated with hepatitis C virus (autoimmune hepatitis, thyroiditis...). In practice, it's of great importance to identify and classify these extra-hepatic manifestations to optimize the treatment of chronic hepatitis C. PMID:10905094

Loustaud-Ratti, V; Lunel, F

2000-05-15

110

Mandibular and Dental Manifestations of Gaucher Disease  

PubMed Central

Gaucher disease is a systemic lysosomal storage disorder with a high prevalence among Ashkenazi Jews. It is caused by an inherited deficiency of the lysosomal enzyme glucocerebrosidase. Common signs and symptoms include hepatosplenomegaly, anemia, thrombocytopenia, and skeletal involvement. Oral and dental manifestations are less commonly seen. These manifestations are often asymptomatic, although they may be detected by routine dental x-rays. There are several case reports and a few larger series published describing patients with Gaucher disease who have mandibulo-maxillofacial involvement. This review aims to examine the oral manifestations observed in Gaucher disease and to suggest practical guidelines for dealing with these often worrisome signs. Among the critical issues are the benign nature of Gaucher cell infiltration of the mandible and the critical importance of being prepared for post-procedure bleeding and/or infections. Therefore, it is essential that dental practitioners be aware of the possible oral and dental complications of Gaucher disease, as well as the available treatment modalities.

Saranjam, Hamid R.; Sidransky, Ellen; Levine, William Z.; Zimran, Ari; Elstein, Deborah

2012-01-01

111

Amniotic band sequence and its neurocutaneous manifestations.  

PubMed

Six new cases of the amniotic band sequence with a wide spectrum of defects were studied with respect to neurocutaneous manifestations, including histopathologic characteristics. Fetuses 1 and 2 were stillborn. Despite severe craniofacial involvement, infants 3 and 4 survived 1 yr and 22 months, respectively. Patients 5 and 6 had only limb defects. Craniofacial and neurologic manifestations of our longer-survival patients with severe craniofacial anomalies were microcephaly, encephalocele, micro- or anophthalmia, mental retardation, hydrocephaly, seizures, EEG abnormalities, impaired body temperature control, impaired vision, hypertonia, and other psychomotor deficits. Characteristic cutaneous manifestations seen in our patients were clefting, schisis, fibrous strings, ring constriction, lymphedema. pseudosyndactyly, and intrauterine amputation. Histologic examination of the amniotic bands attaching to the skin showed autolyzed amnion in patient 2, erosion of the band into upper dermis in patient 1, and fibrosis underlying the constricted areas in patient 5. Prenatal diagnosis by ultrasonography is possible in patients with severe craniofacial defects (patient 2). A brief review of the literature is offered. PMID:3425634

Chen, H; Gonzalez, E

1987-11-01

112

Cutaneous manifestations of human papillomaviruses: a review.  

PubMed

Human papillomaviruses (HPVs) are small DNA viruses of the papovavirus family, with more than 100 types already described. Their importance in human disease cannot be overemphasized because these agents are among the most common pathogens in cutaneous infectious diseases and are very important in a subset of predominantly, but not exclusively, genital squamous-cell carcinomas. HPVs can be associated with a variety of cutaneous as well as mucosal manifestations. Some types of HPVs are associated with increased risk of epithelial malignancies; these have been divided into low-risk and high-risk types based on their oncogenic potential. Clinical and histological features of HPV infection vary according to individual susceptibility (e.g., immunosuppressed patients), site of involvement, and type of HPV implicated. The histological features of HPV infection are very easy to identify on sections stained with hematoxylin and eosin. However, many findings usually associated with HPV infection are entirely non-specific. Additional current diagnostic methods for identification of HPV in tissues include techniques based on the detection of viral DNA; namely, in-situ hybridization and polymerase chain reaction (PCR). This article reviews the main clinical and histopathological cutaneous manifestations of HPV infection, including common warts, plantar warts, plane warts, condyloma acuminatum, Bowenoid papulosis, and epidermodysplasia verruciformis. Emphasis is placed on the clinical and histological features of these various manifestations, including a brief discussion about the routinely used laboratory methods for detecting HPV in tissues. PMID:22131115

Cardoso, Jose Carlos; Calonje, Eduardo

2011-09-01

113

Manifestly covariant Jüttner distribution and equipartition theorem.  

PubMed

The relativistic equilibrium velocity distribution plays a key role in describing several high-energy and astrophysical effects. Recently, computer simulations favored Jüttner's as the relativistic generalization of Maxwell's distribution for d=1,2,3 spatial dimensions and pointed to an invariant temperature. In this work, we argue an invariant temperature naturally follows from manifest covariance. We present a derivation of the manifestly covariant Jüttner's distribution and equipartition theorem. The standard procedure to get the equilibrium distribution as a solution of the relativistic Boltzmann's equation, which holds for dilute gases, is here adopted. However, contrary to previous analysis, we use Cartesian coordinates in d+1 momentum space, with d spatial components. The use of the multiplication theorem of Bessel functions turns crucial to regain the known invariant form of Jüttner's distribution. Since equilibrium kinetic-theory results should agree with thermodynamics in the comoving frame to the gas the covariant pseudonorm of a vector entering the distribution can be identified with the reciprocal of temperature in such comoving frame. Then by combining the covariant statistical moments of Jüttner's distribution a form of the equipartition theorem is advanced which also accommodates the invariant comoving temperature and it contains, as a particular case, a previous not manifestly covariant form. PMID:20365549

Chacón-Acosta, Guillermo; Dagdug, Leonardo; Morales-Técotl, Hugo A

2010-02-01

114

Skin Manifestations of Inflammatory Bowel Disease  

PubMed Central

Inflammatory bowel disease (IBD) is a disease that affects the intestinal tract via an inflammatory process. Patients who suffer from IBD often have diseases that affect multiple other organ systems as well. These are called extraintestinal manifestations and can be just as, if not more debilitating than the intestinal inflammation itself. The skin is one of the most commonly affected organ systems in patients who suffer from IBD. The scientific literature suggests that a disturbance of the equilibrium between host defense and tolerance, and the subsequent over-activity of certain immune pathways are responsible for the cutaneous disorders seen so frequently in IBD patients. The purpose of this review article is to give an overview of the types of skin diseases that are typically seen with IBD and their respective pathogenesis, proposed mechanisms, and treatments. These cutaneous disorders can manifest as metastatic lesions, reactive processes to the intestinal inflammation, complications of IBD itself, or side effects from IBD treatments; these can be associated with IBD via genetic linkage, common autoimmune processes, or other mechanisms that will be discussed in this article. Ultimately, it is important for healthcare providers to understand that skin manifestations should always be checked and evaluated for in patients with IBD. Furthermore, skin disorders can predate gastrointestinal symptoms and thus may serve as important clinical indicators leading physicians to earlier diagnosis of IBD.

Huang, Brian L.; Chandra, Stephanie; Shih, David Quan

2012-01-01

115

Neurological manifestations of Erdheim-Chester disease  

PubMed Central

Erdheim-Chester disease is a rare sporadic systemic histiocytic disease of unknown aetiology that affects multiple organ systems. The case records of all patients with Erdheim-Chester disease who had been seen at the Mayo Clinic between 1975 and 1996 were reviewed to assess the neurological manifestations of the disease. Two of 10 patients had neurological involvement. A 42 year old woman developed central diabetes insipidus and a progressive cerebellar syndrome. Brain MRI showed a lesion in the left pons with patchy gadolinium enhancement and T2 weighted signal abnormalities extending into both cerebellar peduncles and the medulla. Biopsy of the brainstem mass showed a xanthogranulomatous lesion. The second patient was a 53 year old man with retroperitoneal fibrosis secondary to xanthogranulomatous infiltration. Although he had no neurological symptoms and a normal neurological examination, MRI of the head showed multiple uniformly enhancing extra-axial masses along the dura of both convexities and the falx, and a mass in the left orbital apex. Both patients had the characteristic radiographic and bone scan findings of Erdheim-Chester disease. Review of the literature disclosed a wide variety of neurological manifestations in Erdheim-Chester disease. The most frequent CNS manifestations are diabetes insipidus, cerebellar syndromes, orbital lesions, and extra-axial masses involving the dura.??

Wright, R; Hermann, R.; Parisi, J.

1999-01-01

116

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2013 CFR

...PLANS Shipping Papers § 172.205 Hazardous waste manifest. ...subchapter for shipping papers, (3) Given...facility receiving the waste, (4) Returned...manifest or shipping paper. (3) When delivering hazardous waste to the...

2013-10-01

117

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2013 CFR

...DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest...chapter. (3) For rail cars transiting Canada, a manifest on Customs Form...

2013-04-01

118

[Vectorcardiographic manifestations of right ventricular enlargement].  

PubMed

The basic criteria for the vectorcardiographic diagnosis of right ventricular enlargement are discussed, in context with the course of myocardial activation. Right ventricular dilatation, secondary to isolated diastolic overloading (atrial septal defect) shows basically different degrees of dextrorotation. The ventricular curve starts to the left on the frontal and horizontal planes, and forward on the last one. Cases with right ventricular hypertrophy, produced by sustained systolic overload, are also evaluated. When the hypertrophy is generalized (pulmonary valvular stenosis), there is an increase in the manifestation of all the resulting vectors of activation of this ventricle: IIs, IIr, and IIIr. As a resultant of these changes, the ventricular curve presents a clockwise rotation in the three planes, and is oriented to the right and forward, with its terminal portions generally located above the E point. When the right ventricular hypertrophy is of the segmentary type, there is an increase of the manifestation of only some of the resulting vectors of the activation of this ventricle. For example, the vector IIr will be increased in cases of tetralogy of Fallot, while the IIIr will be increased in some cases of obstructive chronic pulmonary hypertensive cardiopathy. The T loop, of secondary type, generally opposes the vector IIr on the horizontal plane, and the IIIr on the frontal plane. When an important right ventricular dilatation is associated to a right bundle branch block of intermediate degree, owing to their proximity, the manifestation of the electromotive parietal forces is increased at the expense of the septal ones. This phenomenon produces a characteristic appearance of the SH loop, narrow and with a clockwise rotation. PMID:162477

de Micheli, A; Medrano, G A

1979-01-01

119

Neurologic manifestations of Henoch-Schönlein purpura.  

PubMed

Henoch-Shönlein purpura (HSP) is a systemic small vessel vasculitis. Most patients present during childhood. The characteristic association of purpura, arthralgia, abdominal pain, and nephritis reflects the predominant distribution of vasculitis. Headaches and mild behavioral changes suggest CNS involvement in one-third of HSP patients. Salient central nervous system (CNS) manifestations are rarer and mostly reported in adults and patients with a severe disease course. Diagnosis of CNS vasculitis is rarely confirmed by histopathology and generally relies on "suggestive" imaging showing brain hemorrhages, infarcts and edema, predominantly located in the parieto-occipital regions. Vessel wall friability and thrombogenicity of active vasculitis, antiphospholipid antibody synthesis, and other hemostatic disturbances may contribute to hemorrhagic and thrombotic complications of HSP. Posterior reversible encephalopathy syndrome and hypertensive encephalopathy occur in HSP and can be difficult to differentiate from CNS vasculitis. Some 53% of patients with neurologic complications experience seizures. Cerebral venous thrombosis, subdural hematoma, subarachnoidal hemorrhage, neuro-ophthalmologic complications, myelopathy, and diverse neuromuscular manifestations are also reported. In contrast with other systemic small vessel vasculitides, peripheral nervous system involvement is infrequent in HSP. Systemic involvement of HSP and homeostatic disorders such as hypertension, uremia, and electrolyte disturbances, as well as superimposed infections can affect the nervous system secondarily. Identification of nervous system complications of HSP is often challenging due to prominent systemic manifestations. HSP is usually a self-limiting disease that requires only supportive care. Patients with CNS vasculitis are commonly treated with corticosteroids. One-fifth of patients with CNS involvement remain with sequelae. PMID:24365374

Bérubé, Maxime D; Blais, Normand; Lanthier, Sylvain

2014-01-01

120

Various musculoskeletal manifestations of chronic renal insufficiency.  

PubMed

Musculoskeletal manifestations in chronic renal insufficiency are caused by complex bone metabolism alterations, now described under the umbrella term of chronic kidney disease mineral- and bone-related disorder (CKD-MBD), as well as iatrogenic processes related to renal replacement treatment. Radiological imaging remains the mainstay of disease assessment. This review aims to illustrate the radiological features of CKD-MBD, such as secondary hyperparathyroidism, osteomalacia, adynamic bone disease, soft-tissue calcifications; as well as features associated with renal replacement therapy, such as aluminium toxicity, secondary amyloidosis, destructive spondyloarthropathy, haemodialysis-related erosive arthropathy, tendon rupture, osteonecrosis, and infection. PMID:23522485

Lim, C Y; Ong, K O

2013-07-01

121

Cardiovascular manifestations of Williams syndrome: imaging findings.  

PubMed

Williams syndrome is a relatively common (1 in 10,000 live births) genetic disorder caused by a deletion involving chromosome 7 that results in a variety of clinically significant abnormalities, including developmental delay, behavioral changes, hypercalcemia, and a distinct "elfin" facial appearance. Congenital cardiovascular disease that presents in childhood is responsible for most of the morbidity and mortality associated with this disorder. The purpose of this pictorial essay is to review imaging findings of some of the more common cardiovascular manifestations of Williams syndrome and to highlight some of the unique anatomic variations that can be seen in these patients. PMID:24331936

Gray, J Cranston; Krazinski, Aleksander W; Schoepf, U Joseph; Meinel, Felix G; Pietris, Nicholas P; Suranyi, Pal; Hlavacek, Anthony M

2013-01-01

122

Paraneoplastic dermatological manifestation of gastrointestinal malignancies  

PubMed Central

Numerous dermatological disorders have been associated with underlining malignancies of the gastrointestinal (GI) tract. Such cutaneous manifestations might have an important diagnostic value if they are the sole expressions of otherwise asymptomatic carcinomas. The recognition of some typical paraneoplastic dermatologic disorders can lead to the prompt diagnosis of the underlying malignancy, timely administration of therapy, and ultimately, better prognosis. In this review we discuss the most common paraneoplastic dermatological syndromes from the perspective of the practicing gastroenterologist. We also outline a comprehensive practical approach for the evaluation for occult malignancy in patients presenting with cutaneous findings potentially associated with GI cancers.

Dourmishev, Lyubomir A; Draganov, Peter V

2009-01-01

123

Ocular manifestations of systemic inflammatory diseases.  

PubMed

Inflammation of the eye is often times seen in association with systemic inflammatory diseases. Understanding the various forms of ocular involvement in these conditions is important as untreated ophthalmic involvement can lead to severe vision loss. In addition to providing a basic framework for diagnosis and treatment, this review will highlight the ocular manifestations of the following systemic inflammatory conditions: rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, Sjögren's syndrome, polyarteritisnodosa, primary antiphospholipid syndrome, Behçet's syndrome, Kawasaki disease, Cogan's syndrome and relapsing polychondritis. PMID:23155672

Mohsenin, Amir; Huang, John J

2012-10-01

124

Musculoskeletal manifestations of osteomalacia and rickets.  

PubMed

Osteomalacia (OM) is still an important metabolic bone disease with increased prevalence in certain regions of the world as well as in the urban population of elderly confined. The disease presents with a wide variety of clinical, biochemical and radiographic manifestations mimicking other musculoskeletal disorders, including 'osteoporosis'. In this chapter, we provide the basis for its clinical diagnosis and management. There have been significant recent advances in the understanding of vitamin D deficiency and hypophosphataemic osteomalacia, which can now assist clinicians in the precise diagnosis and treatment of this disease. In this chapter we also review the various underlying aetiologies. The successful management of OM depends on the underlying aetiology. PMID:15123051

Reginato, Antonio J; Coquia, Juliet Arambulo

2003-12-01

125

Neuro-ophthalmologic manifestations of sarcoidosis  

Microsoft Academic Search

Opinion statement  The first-line treatment for the neuro-ophthalmologic manifestations of sarcoidosis is corticosteroid therapy. Prednisone,\\u000a 0.5 to 1 mg\\/kg\\/day, is initially prescribed for 2 to 4 weeks, before a slow taper is begun as the patient’s symptoms and examination\\u000a are monitored. Patients frequently require adjunct therapy, which can be in the form of immunomodulatory drugs such as pentoxyfillin,\\u000a hydroxychloroquine, or thalidomide,

Barney J. Stern; James Corbett

2007-01-01

126

Cardiac tamponade as a manifestation of tuberculosis.  

PubMed

Tuberculosis has been increasing in incidence in recent years. Pericardial involvement and pericardial effusions are well-documented and may result in pericardial tamponade. Despite this, large pericardial effusions are uncommon, and manifestation as cardiac tamponade is rare. We report two cases of tuberculous pericarditis in which the initial feature was tamponade. Since the diagnosis of tuberculosis may be delayed due to the slow-growing nature of the bacterium, physicians need to be aware of this possibility and consider the use of modern diagnostic techniques that may permit an earlier diagnosis. PMID:11372808

Gladych, E; Goland, S; Attali, M; Somin, M; Malnick, S D

2001-05-01

127

An unusual manifestation of renal osteodystrophy.  

PubMed

Besides the more frequent aspects, an unusual manifestation of renal osteodystrophy occurred in a 19-year-old man on regular hemodialysis treatment. About 6 years after the start of hemodialysis, the patient, who had severe osteodystrophy, presented a brown tumor, localized to the jaw, which caused a serious deformity of the face. Pharmacological treatment for hyperparathyroidism did not reduce the volume of the mass. Only total surgical removal gave long-lasting success. Brown tumor is an unusual but serious complication of renal bone disease which can be treated successfully by surgical removal of the tumoral mass and by pharmacological treatment for hyperparathyroidism. PMID:6728088

Catizone, L; Casolino, D; Santoro, A; Casanova, S; Cerasoli, P T; Zucchelli, P

1984-01-01

128

Dermatomal sensory manifestations in lateral medullary infarction.  

PubMed

A 61-year-old man who experienced a sudden onset of unstable gait followed by nuchal pain was admitted to our department. The neurologic examination revealed right-sided limb ataxia, right partial ptosis, and decreased sensation to 50% of the normal side to pinprick and temperature stimuli on the left side below the level of the T-6 dermatome. A lateral medullary infarction caused by spontaneous vertebral artery dissection was diagnosed by magnetic resonance imaging and computed tomography angiography. In conclusion, lateral medullary infarction is an important entity to consider in the differential diagnosis of dermatomal sensory manifestations. PMID:23099044

Hongo, Hiroki; Tanaka, Yasutaka; Shimada, Yoshiaki; Tanaka, Ryota; Hattori, Nobutaka; Urabe, Takao

2014-01-01

129

40 CFR 263.20 - The manifest system.  

Code of Federal Regulations, 2013 CFR

...manifest (or shipping paper for exports by water...exports of hazardous waste subject to the requirements...manifest or the shipping paper; and (4) The...delivering the hazardous waste to the initial water...manifest or shipping paper. (3) When delivering hazardous waste to the...

2013-07-01

130

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2013 CFR

...CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification...with § 262.40(a). (b) The generator must give the transporter the...

2013-07-01

131

AL amyloidosis manifesting as systemic lymphadenopathy.  

PubMed

We report three patients with AL amyloidosis manifesting as systemic lymphadenopathy, mainly in the cervical and supraclavicular regions. Histopathology of lymph nodes showed massive deposition of AL amyloid with no abnormal findings suggestive of lymphoproliferative disorders. Two of the patients were considered to be classifiable as primary systemic AL amyloidosis based on the presence of M-protein in serum and abnormal plasma cells or lymphoplasmacytoid cells in the bone marrow probably producing the precursor immunoglobulin, although no visceral organs were affected. The size of the involved lymph nodes in these two patients increased gradually, and one was treated with rituximab and VAD (vincristine, doxorubicin and dexamethasone) followed by high-dose melphalan with autologous peripheral blood stem cell transplantation (auto-PBSCT). The remaining patient showed no obvious change in the size of lymph nodes or detectable M-protein in serum. The prognosis of AL amyloidosis manifesting as lymphadenopathy is usually good as long as there are no hematological malignancies or rapid increases in the size of lymph nodes, but in cases of the systemic type, intensive chemotherapy, such as high-dose melphalan with auto-PBSCT, should be actively considered in order to avoid possible involvement of visceral organs. PMID:18484338

Matsuda, Masayuki; Gono, Takahisa; Shimojima, Yasuhiro; Yoshida, Takuhiro; Katoh, Nagaaki; Hoshii, Yoshinobu; Yamada, Toshiyuki; Ikeda, Shu-ichi

2008-06-01

132

[Rheumatic manifestations of parvovirus B19 infection].  

PubMed

Parvoviruses are small single-strand DNA viruses. Human parvovirus (PV B19) was isolated in the 1970s and recognized as a pathogen in the 80s. Its role was first demonstrated in acute erythroblastopenia in patients with chronic haemolytic anaemia, then in epidemic megalerythema. More recently PVB19 has been shown to be the aetiologic agent in fetal loss and chronic infection in immunodepressed patients. Further attention has focused on rheumatologic manifestations, particularly in prolonged joint diseases raising the problem of differential diagnosis and a possible pathogenic relationship with rheumatoid arthritis and systemic lupus erythematosus. Clinically, subjects with parvovirus B19 infection have flu-like manifestations of joint pain and myalgia lasting for about 1 week. Generally, the joint pain is symmetrical, of inflammatory type usually involving proximal joints (phalangeal joints in two-thirds of the cases). Acute back pain or associated extra-articular signs are often absent. Signs regress spontaneously in 10 to 15 days although longer periods have been observed. Diagnosis can be confirmed by ELISA identification of specific IgM antibodies. Nonsteroid antiinflammatory drugs are generally effective. PMID:7899371

Wendling, D; Lorge, J F; Kremer, P

1995-01-28

133

[Skin manifestations in patients with HIV infection].  

PubMed

Cutaneous manifestations are common in patients with HIV infection and mainly due to the immunodeficiency. In the initial stage of HIV infection, we frequently observe a rash of macular lesions. During the asymptomatic phase, the patients may typically show the following skin diseases: seborrhoic dermatitis, acneiform folliculitis, persistent herpes simplex, and infections with the human papilloma virus. In ARC and AIDS patients, 3 groups of skin disorders are found: cutaneous infections, skin tumors, and other mixed skin diseases. Herpes simplex and herpes zoster may develop into ulcerating and necrotising forms especially in patients with advanced immunodeficiency. The most frequent skin tumors in AIDS patients are the disseminated Kaposi's sarcoma and non-Hodgkin's lymphoma. More than 50% of the AIDS patients treated with trimethoprim/sulfamethoxazole developed a severe drug eruption. African and Caribbean patients with AIDS frequently suffer from pruritic skin lesions, the pathogenesis of which is not known. Aside from these cutaneous manifestations, a variety of other skin disorders have been reported in patients with HIV infection, ARC, or AIDS; future research will furnish definite proof whether they are correlated with HIV infection. PMID:2205061

Eichmann, A

1990-07-01

134

Oral Crohn's disease without intestinal manifestations  

PubMed Central

Crohn?s disease is a granulomatous inflammatory bowel disease and was described in 1932 as a chronic granulomatous disorder of the terminal ileum and is now considered a distinct member of the inflammatory bowel disease family. It may affect any part of the gastrointestinal tract. Oral Crohn?s disease has been reported frequently in the last three decades with or without intestinal manifestations. In the latter case, it is considered as one of the orofacial granulomatosis. There has been much doubt whether intestinal manifestations of Crohn?s disease will eventually develop in the orofacial granulomatosis. We present a female patient aged 22 years with prominent clinical findings such as persistent swelling of lower and upper lip with fissuring and angular cheilitis, granulomatous gingival enlargement, and cobblestone or corrugated appearance of labial mucosa, which are suggestive of Crohn?s disease, but with no evidence of other gastrointestinal involvement. The patient underwent surgical treatment with external gingivectomy procedure. A 6-month follow-up showed minimal recurrence.

Harikishan, Gingisetty; Reddy, Nagate Raghavendra; Prasad, Harikrishnan; Anitha, Subappa

2012-01-01

135

Classifying functional manifestations of ectodermal dysplasias.  

PubMed

Ectodermal dysplasias (ED) encompass more than 200 conditions involving some combination of disorders of hair, nails, teeth, and sweat glands. The incidence of ED is relatively rare affecting about 7 of 10,000 births [Itin and Fistarol (2004)]. Individuals manifesting ED present with a wide range of disorders involving hair, nails, teeth, and sweat glands and in many cases other characteristics as well. The complex nature of the disorder has presented challenges for clinical practice and required the involvement of multiple approaches and disciplines. It has also resulted in significant research initiatives on cause and symptomatology. A significant challenge has been the search for comprehensive documentation of the varied and complex manifestations associated with ED. Existing classification systems of ED have focused on physiological and structural dimensions. Classification approaches with a broader focus including characteristics of functioning in persons with ED could facilitate clinical work and research initiatives. In this context, the potential utility of available classifications that address functioning and disability would be appropriate to consider in the search for a consensus classification of ED. To that end, the purpose of this article is to (a) review the status of classification of ED, (b) provide a brief overview of the International Classification of Functioning, Disability and Health-Children and Youth, ICF-CY [World Health Organization (2007); International Classification of Functioning, Disability and Health-Children and Youth. Geneva: WHO.], and (c) identify possible contributions of the ICF-CY to classification of ED's. PMID:19681156

Simeonsson, Rune J

2009-09-01

136

Neurologic manifestations of inflammatory bowel diseases.  

PubMed

Inflammatory bowel diseases (IBD) are chronic, relapsing and remitting inflammatory conditions affecting the digestive system, comprising two main distinctive diseases, ulcerative colitis (UC) and Crohn's disease (CD). Besides the classic gastrointestinal manifestations, a variable number of IBD patients present with extraintestinal manifestations, including central and peripheral nervous system involvement. Peripheral neuropathy is one of the most common complications. An inflammatory myopathy has also been found. Cranial neuropathies include the Melkersson-Rosenthal syndrome, optic neuritis, and sensorineural hearing loss. Patients with IBD have a remarkable thromboembolic tendency and are at increased risk of both venous and arterial thrombotic complications. The prothrombotic state in IBD has multiple contributors. Ischemic stroke occurs through several mechanisms, including large artery disease, small vessel disease, paradoxical embolism, endocarditis, vasculitis, and associated with anti-TNF-? therapy. Thrombosis of the dural sinus and cerebral veins are at least as frequent as arterial stroke in IBD. Multiple sclerosis has been repeatedly associated with IBD. Up to 50% of IBD present asymptomatic white matter lesions. Other central nervous system complications include a slowly progressive myelopathy, epidural and subdural spinal empyema secondary to fistulous extension from the rectum, seizures, and encephalopathy. PMID:24365340

Ferro, José M; Oliveira, Sofia N; Correia, Luis

2014-01-01

137

Retropharyngeal pseudoabscess manifesting in nephrotic syndrome.  

PubMed

We describe a case of nephrotic syndrome that manifested as a retropharyngeal pseudoabscess. The patient was a 32-year-old man who presented with a short history of throat discomfort and a choking sensation. Laryngoscopy identified bulging of the posterior pharyngeal wall that partially occluded the laryngeal inlet. A lateral neck x-ray revealed that the prevertebral space was widened, and computed tomography detected fluid in the retropharyngeal and parapharyngeal spaces. Neck exploration revealed that the edema had been caused by nonsuppurative fluid. Biochemical analyses revealed marked hypoalbuminemia and heavy proteinuria suggestive of nephrotic syndrome. Following surgery, the patient's symptoms resolved. Aseptic effusion into the retropharyngeal space is rare; reported etiologies include internal jugular vein thrombosis, neoplasia, radiation therapy, trauma, acute calcific tendinitis, hereditary angioedema, and myxedema of hypothyroidism. To the best of our knowledge, this is the first case of nephrotic syndrome initially manifesting as retropharyngeal pseudoabscess to be reported in the literature. Thrombotic occlusion of the pharyngeal venous plexus secondary to hypercoagulability is a plausible explanation for such isolated retropharyngeal effusion. PMID:24817238

Johari, Shirish; Handa, Pankaj; Siow, Jin Keat

2014-01-01

138

Otolaryngologic manifestations of diffuse idiopathic skeletal hyperostosis.  

PubMed

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by formation of large cervical osteophytes that may compress the posterior wall of the aerodigestive tract. It is a rare cause of dysphagia in the elderly. The aim of this study was to investigate the various otolaryngologic manifestations of DISH. Eleven elderly patients with DISH were included in the study. All patients presented with dysphagia that was graded on the swallowing screening tool (EAT-10), and the diagnosis of DISH was based on computed tomographic criteria. The patients were subjected to otolaryngologic examination and flexible laryngoscopy. Polysomnography was used for patients with excessive daytime sleepiness for detection of obstructive sleep apnea (OSA). In addition to dysphagia of varying severity, OSA was found in nine patients, change of voice in six, globus sensation in seven, aspiration in three, and cervical pain in seven. Flexible laryngoscopy showed bulging of the posterior pharyngeal wall in all patients. DISH may be an unrecognized contributory factor to both dysphagia and OSA in the elderly. Change of voice, aspiration, globus sensation, and cervical pain are other otolaryngologic manifestations that may be encountered symptoms of the disease. An otolaryngologist should be aware of the disease that may be overlooked, and computed tomography is a confirmatory diagnostic method. PMID:24264765

Abdel-Aziz, Mosaad; Azab, Noha A; Rashed, Mohammed; Talaat, Ahmed

2014-06-01

139

Secondary Syphilis: Uncommon Manifestations a Common Disease  

PubMed Central

Reports of cases of primary and secondary syphilis are increasing in the United States, particularly in urban areas and among homosexual men. While primary syphilis poses little diagnostic difficulty, many physicians are unfamiliar with the multisystem nature of secondary lues. Patients who have secondary syphilis commonly present with systemic signs, skin rash, mucous membrane lesions and generalized adenopathy. Less commonly, secondary syphilis may occur as acute meningitis, sensorineural hearing loss, iritis, anterior uveitis, optic neuritis, Bell's palsy, gastropathy, proctitis, hepatitis, pulmonary infiltration, nephrotic syndrome, glomerulonephritis, periostitis, tenosynovitis and polyarthritis. The diagnosis of secondary syphilis is easily confirmed. Its various manifestations are readily treated with penicillin and, if treated early, are entirely reversible. Two recent cases of secondary syphilis, one presenting as nephrotic syndrome and one as chorioretinitis and ptosis, illustrate the usual and unusual features of this common infection. Images

McPhee, Stephen J.

1984-01-01

140

Olfactory neuroblastoma presenting with exclusive orbital manifestations.  

PubMed

We report a case of an olfactory neuroblastoma revealed by atypical presentation with only ophthalmic symptoms. A 73-year-old woman presented with exophthalmos, left chemosis, decreased visual acuity, and diplopia. CT scan revealed a heterogeneous mass with isolated maxillary sinus, orbital floor, and lower eyelid invasion. This orbital lesion has no major ethmoidal extension (a single anterior ethmoidal cell appears retrospectively invaded). The biopsy sample of the hardened tumefaction revealed after histological examination an olfactory neuroblastoma (or esthesioneuroblastoma). The patient was treated by surgery (resection of the lesion and neck dissection) followed by postoperative irradiation.Olfactory neuroblastoma has no specific symptoms, but habitually presents as an ethmoidal lesion possibly accompanied with ophthalmic manifestations (in 20% to 30% of cases) due to orbital extension. This type of unusual presentation requires our attention, and we discuss the actual knowledge about the pathogenesis, clinical presentation, and treatment of this rare entity. PMID:23524773

Lopez, Raphaël; Mazzoni, Lina; Chaput, Benoit; Jalbert, Florian

2013-03-01

141

[Ictus emeticus. Vomiting as epileptic manifestation].  

PubMed

Autonomic and visceral phenomena are well-known manifestations of epileptic seizures, but recurrent vomiting as ictal epileptic events are less known. Three patients with ictus emeticus, i.e. with nausea and vomiting as their main ictal symptoms, are described. Vomiting is a complex symptom preceded by several phenomena in the gastrointestinal tract and transmitted by the vagal nerve to the vomiting centre in the lateral reticular formation of the medulla oblongata. This autonomic centre in the brain stem is influenced by several cerebral structures. There is both experimental and clinical evidence to support the hypothesis that epileptic disturbances in the insula and the mesial temporal structures are responsible for ictus emeticus. PMID:8638265

Nakken, K O

1996-01-30

142

Ocular manifestations in porphyria cutanea tarda.  

PubMed

A 24-year-old man presented with pain, sticky discharge and loss of vision in the right eye. He has had typical skin manifestations of porphyria cutanea tarda (PCT) since 6 years and ophthalmological symptom for 6 weeks. On ophthalmological examination, visual acuity was light perception in the right eye and 6/12 in the left. There were bilateral, symmetrical temporal scleromalacia along with temporal corneal melting in both eyes and perforation in the right eye. Ultrasonography B-scan (USG B-scan) revealed a retinal detachment in the right eye. Artificial tear instillation was started every hour along with topical antibiotic coverage in both eyes. Additionally, ultraviolet protective sunglasses and hat for photo-protection was advised. The vision in the right eye improved to 5/60 along with subsidence of retinal detachment on repeat USG B-scan after 3 weeks. PMID:24811555

Gogri, Pratik Yeshwant; Misra, Neeta Somen; Misra, Somen

2014-01-01

143

Cerebellar hemangioblastoma manifesting as hearing disturbance.  

PubMed

A 49-year-old man presented with a rare case of cerebellar hemangioblastoma manifesting as only hearing disturbance. He had suffered from hearing difficulty in the right ear for a few months. Magnetic resonance imaging revealed a cystic mass lesion with an internal fluid level and surrounding flow voids in the right cerebellopontine (CP) angle. Cerebral angiography disclosed a vascular-rich tumor fed by both the superior cerebellar and anterior inferior cerebellar arteries. En bloc resection of the tumor was planned under a preoperative diagnosis of cerebellar hemangioblastoma. The tumor protruded into the CP cistern and compressed cranial nerve VIII. The feeding arteries were meticulously coagulated and the tumor was successfully removed. The histological diagnosis was hemangioblastoma. After the operation, the patient's hearing acuity improved dramatically. Cerebellar hemangioblastoma should be considered in the differential diagnosis of CP angle tumors associated with hearing disturbance. PMID:19779288

Amano, Toshiyuki; Tokunaga, So; Shono, Tadahisa; Mizoguchi, Masahiro; Matsumoto, Kenichi; Yoshida, Fumiaki; Sasaki, Tomio

2009-09-01

144

Laryngeal manifestations of gastroesophageal reflux disease.  

PubMed

Chronic laryngeal signs and symptoms associated with gastroesophageal reflux disease (GERD) are often referred to as reflux laryngitis or laryngopharyngeal reflux (LPR). It is estimated that up to 15% of all visits to otolaryngology offices are because of manifestations of LPR. Damage to laryngeal mucosa may be the result of reflux of gastroduodenal contents, whether chronic or a single incident. The most common presenting symptoms of LPR include hoarseness, sore throat, throat clearing, and chronic cough. The diagnosis of LPR is usually made on the basis of presenting symptoms and associated laryngeal signs, including laryngeal edema and erythema. The current recommendation for managing these patients is empiric therapy with twice-daily proton pump inhibitors for 1 to 2 months. Other causes of laryngeal irritation are considered in most of those who are unresponsive to such therapy. Surgical fundoplication is most effective in those who are responsive to acid-suppressive therapy. PMID:18625138

Vaezi, Michael F

2008-06-01

145

Cerebellar Stroke-manifesting as Mania  

PubMed Central

Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed.

Jagadesan, Venkatesan; Thiruvengadam, Kannapiran R.; Muralidharan, Rengarajalu

2014-01-01

146

Imaging Manifestations of Mediastinal Fat Necrosis  

PubMed Central

Mediastinal fat necrosis (MFN) or epipericardial fat necrosis, as it is commonly referred to in the literature, is a rare self-limiting cause of chest pain of unclear etiology. MFN affects previously healthy individuals who present with acute pleuritic chest pain. Characteristic computed tomography (CT) findings include a fat attenuation lesion with intrinsic and surrounding increased attenuation stranding. There is often associated thickening of the adjacent pericardium and/or pleural effusions. We present two cases of MFN manifesting as ovoid fat attenuation lesions demarcated by a soft tissue attenuation rim with intrinsic and surrounding soft tissue attenuation stranding and review the clinical and pathologic features of these lesions. Knowledge of the clinical presentation of patients with MFN and familiarity with the characteristic imaging findings of these lesions should allow radiologists to prospectively establish the correct diagnosis and suggest conservative management and follow-up.

Bhatt, Malay Y.; Martinez-Jimenez, Santiago; Rosado-de-Christenson, Melissa L.; Watson, Kenneth R.; Walker, Christopher M.; Kunin, Jeffrey R.

2013-01-01

147

Imaging manifestations of mediastinal fat necrosis.  

PubMed

Mediastinal fat necrosis (MFN) or epipericardial fat necrosis, as it is commonly referred to in the literature, is a rare self-limiting cause of chest pain of unclear etiology. MFN affects previously healthy individuals who present with acute pleuritic chest pain. Characteristic computed tomography (CT) findings include a fat attenuation lesion with intrinsic and surrounding increased attenuation stranding. There is often associated thickening of the adjacent pericardium and/or pleural effusions. We present two cases of MFN manifesting as ovoid fat attenuation lesions demarcated by a soft tissue attenuation rim with intrinsic and surrounding soft tissue attenuation stranding and review the clinical and pathologic features of these lesions. Knowledge of the clinical presentation of patients with MFN and familiarity with the characteristic imaging findings of these lesions should allow radiologists to prospectively establish the correct diagnosis and suggest conservative management and follow-up. PMID:24369521

Bhatt, Malay Y; Martínez-Jiménez, Santiago; Rosado-de-Christenson, Melissa L; Watson, Kenneth R; Walker, Christopher M; Kunin, Jeffrey R

2013-01-01

148

Ocular and skin manifestations in systemic pseudohypoaldosteronism.  

PubMed

Pseudohypoaldosteronism type-1 is a rare disorder characterised by end-organ resistance to aldosterone resulting in salt-losing crisis with hyponatraemic dehydration, hyperkalaemia and metabolic acidosis. We report two siblings with pseudohypoaldosteronism type-1 who presented early in neonatal period with hyponatraemia, severe hyperkalaemia and metabolic acidosis. Both babies had miliaria like skin rash which flared up during episodes of hyperkalaemia and hyponatraemia. They had visible dilated meibomian glands from which a white material was protruding. The clinical presentation of pseudohypoaldosteronism type-1 mimics congenital adrenal hyperplasia. As there is often a delay in obtaining hormonal assay results, the eye and skin manifestations may give an important diagnostic clue which in turn will influence management. PMID:24654255

Eliwa, Mahmoud Salah; El-Emmawie, Aymen Hussein; Saeed, Mahmood Ahmad

2014-01-01

149

[Digestive manifestations in acquired immunodeficiency disease].  

PubMed

Digestive manifestations of AIDS were studied retrospectively in 87 patients with respect to clinical, microbiological, endoscopic and histological data. Chronic or intermittent diarrhea was observed in 62 p. 100 of patients and frequently associated with major weight loss. The digestive opportunistic infections were: oesophageal candidiasis (42 p. 100), cryptosporidiosis (24 p. 100), cytomegalovirus infections (22 p. 100), isosporiasis (3.4 p. 100), atypical mycobacteriosis (2.2 p. 100), toxoplasmosis (2.2 p. 100). Thirty non-opportunistic agents were detected in 26 patients. Gastrointestinal Kaposi sarcoma occurred in 18 p. 100 of cases. The etiology of diarrhea remained unknown in 16 p. 100 of patients. The value of endoscopy is discussed: esophagogastroduodenal fibroscopy, is usually required to assess oesophageal candidiasis, colonoscopy is required to assess cytomegalovirus colitis on histological data. PMID:2829681

Girard, P M; Marche, C; Maslo, C; Rene, E; Leport, J; Matheron, S; Michon, C; Coulaud, J P; Saimot, A G

1987-01-01

150

Neuro-ophthalmologic Manifestations of Sarcoidosis.  

PubMed

The first-line treatment for the neuro-ophthalmologic manifestations of sarcoidosis is corticosteroid therapy. Prednisone, 0.5 to 1 mg/kg/day, is initially prescribed for 2 to 4 weeks, before a slow taper is begun as the patient's symptoms and examination are monitored. Patients frequently require adjunct therapy, which can be in the form of immunomodulatory drugs such as pentoxyfillin, hydroxychloroquine, or thalidomide, or immunosuppressive drugs such as mycophenolate mofetil, azathioprine, methotrexate, and cyclophosphamide. Individuals with profound visual compromise or progressive disease may benefit from high-dose intravenous methylprednisolone or tumor necrosis factor-alpha antagonists such as infliximab. Attention to the overall medical status of the patient is essential to ensure that an optimal clinical status is achieved. PMID:17288891

Stern, Barney J; Corbett, James

2007-01-01

151

Neurologic manifestations of chronic methamphetamine abuse  

PubMed Central

Summary Chronic methamphetamine abuse has devastating effects on the central nervous system. The degree to which addicts will tolerate the dysfunction in the way they think, feel, move, and even look, is a powerful testimony to the addictive properties of this drug. While the mechanisms behind these disorders are complex, at their heart they involve the recurring increase in the concentrations of central monoamines with subsequent dysfunction in dopaminergic neurotransmission. The mainstay of treatment for the problems associated with chronic methamphetamine abuse is abstinence. However, by recognizing the manifestations of chronic abuse, clinicians will be better able to help their patients get treatment for their addiction and to deal with the neurologic complications related to chronic abuse.

Rusyniak, Daniel E.

2011-01-01

152

Acoustic manifestations of gas hydrate shelled bubbles  

NASA Astrophysics Data System (ADS)

The hydrocarbon seeps emitting buoyant bubble plumes from seafloor vents—gas flares have been actively investigated in different regions of the World Ocean, in particular, on the Sakhalin slope in the Sea of Okhotsk. The gas flares can be easily detected by regular echo sounders, because the scattering cross section of a gas bubble is large. Within the gas-hydrate stability zone—for high hydrostatic pressures and low temperatures, methane-hydrate ice skins are formed on rising seep bubbles which are typically methane. The objective of the present study was to develop a suitable model describing rheological characteristics of gas-hydrate shell and to analyze acoustic manifestations of such bubbles for the frequency range used in marine field experiments.

Maksimov, A. O.; Sosedko, E. V.

2009-11-01

153

Musculoskeletal manifestations in patients with malignant disease.  

PubMed

To detect and describe the incidence of musculoskeletal manifestations in different malignant diseases as well as their relation to the treatment received whether by chemotherapy or radiation therapy. Sixty patients with different malignant diseases were included in this study, 45 with solid tumors and 15 patients with hematological malignancy. The mean age was 46.55 +/- 11.04 years and the mean disease duration was 2 +/- 0.75 years. The patients were fully examined for any rheumatologic involvement, laboratory investigations were performed as well as dual energy X-ray absorptiometry study for bone densitometry. Treatment strategies were assessed including the chemotherapeutics, radiation therapy, and/or surgery. Myalgias and arthralgias were the most frequent followed by flexor tenosynovitis, frozen shoulder, and fibromyalgia syndrome. Hypertrophic osteoarthropathy was seen in five patients, cutaneous vasculitis in two patients as well as arthritis. Osteonecrosis was present in one of the lunate carpal bones of a patient with non-Hodgkin's lymphoma (1.67%) and receiving high dose steroids. Rheumatoid factor was positive in four patients, three of which had hepatitis C virus positivity and cryoglobulins. Anti-neutrophil cytoplasmic antibody was negative in all the studied patients. The bone mineral density was significantly reduced in the patients with malignancy compared to the control. Mild to moderate osteoporosis was present, being more evident in the spine and forearm. The bone loss was higher in those with solid tumors and even more obvious in those receiving aromatase inhibitors. Musculoskeletal manifestations occurring during malignancies and following the treatment represent a significant percentage of symptoms and signs which may raise a clue to differential diagnosis. PMID:19898774

Gheita, Tamer A; Ezzat, Yasser; Sayed, Safaa; El-Mardenly, Ghada; Hammam, Waleed

2010-02-01

154

When does an illness begin: genetic discrimination and disease manifestation.  

PubMed

The Genetic Information Nondiscrimination Act of 2008 (GINA) was passed to encourage patients to seek genetic testing that could improve health outcomes and provide opportunities for preventive measures. GINA protects individuals from discrimination based upon genetic information, but not upon manifested diseases and conditions. Because the manifestation of a disease establishes a threshold of protection for individuals under GINA, the definition of manifestation is crucial to understanding the scope of the bill. This paper examines the range of possible legal definitions of disease manifestation and explores the historical struggle that courts have faced when trying to apply these different definitions. Specifically, the paper examines three frameworks that courts have used in the past to interpret the manifestation of a disease; "manifestation as apparent symptoms,"manifestation as patient action," and "manifestation as physician action." We argue that the "manifestation as physician action" is the best framework to utilize in this law generally, but that the definition of manifestation should be read in the light most favorable to a plaintiff in order to achieve the underlying goals of GINA. PMID:23061591

Prince, Anya E R; Berkman, Benjamin E

2012-01-01

155

Multiple Endocrine Neoplasia Type 1: Clinical Manifestations and Management  

Microsoft Academic Search

\\u000a Multiple endocrine neoplasia type I (MEN-1) is an autosomal dominant syndrome associated with anterior pituitary, parathyroid,\\u000a and enteropancreatic endocrine tumors as well as other endocrine and nonendocrine tumors [1]. MEN-1 is defined as the presence\\u000a of two of three main MEN-1-related manifestations, or at least one manifestation plus a first degree relative with at least\\u000a one MEN-1-related manifestation [1,2]. The

Anathea C. Powell; Steven K. Libutti

156

Simulation of the regional manifestation of asthma.  

PubMed

Asthma presents serious medical problems of global proportions. Clinical data suggest that the disease occurs preferentially at regions designated by large (0 manifestation of asthma as distinct components of smooth muscle constriction and inflammation. We have conducted a systematic analysis of the relative effects of morphology, ventilation, and particle size on aerosol deposition. Different intensities of asthma were simulated by reducing airway diameters by prescribed amounts. To show the real clinical applications of modeling, we have also simulated the performance of a popular nebulizer. Regarding therapeutic implications, it is clear that disease-induced changes in airway morphologies have pronounced effects on the administration of inhaled drugs. Likewise, ventilation affects both the total aerosol mass deposited and its relative spatial distribution among airways. By formulating these effects, the computer code allows drugs (e.g., bronchodilators for constriction, steroids for inflammation) to be selectively deposited. We suggest, therefore, that the code can be used in a complementary manner with clinical studies and can be integrated into aerosol therapy regimens. PMID:15067697

Sbirlea-Apiou, Gabriela; Lemaire, Marc; Katz, Ira; Conway, Joy; Fleming, John; Martonen, Ted

2004-05-01

157

Unusual manifestations of a common gastrointestinal disorder.  

PubMed

We present an instructive case of a 13-year old male who presented with bilateral scrotal redness, swelling and tenderness, but with a normal testicular exam. His scrotal swelling persisted despite treatment with intravenous antibiotics, and on further history he reported 2 years of intermittent upper lip swelling. After a referral to a dermatologist, a lip biopsy showed granulomatous changes and he was referred to the gastroenterology department. A gastroscopy and colonoscopy was performed and histology confirmed non-caseating granulomas consistent with Crohn's disease (CD). Eighteen months after the diagnosis of CD he developed perianal disease with a fistula and distal anal stricture. He was successfully treated with insertion of a seton and escalation of therapy to azathioprine and infliximab. CD is a phenotypically diverse chronic inflammatory condition with an increasing incidence in Australia and other Western countries. Non-typical presentations, such as perianal manifestations or orofacial granulomatosis, can be the only presenting symptom in CD, and this highlights the importance for a high degree of clinical suspicion. Genital involvement is rare, but reported. PMID:23662656

Rajah, Karthik; Oliver, Mark R; McLeod, Liz; Orchard, David; Leal, Marcelo

2014-02-01

158

Ophthalmic manifestation of congenital protein C deficiency.  

PubMed

Under normal conditions activated protein C is a natural anticoagulant that cleaves 2 activated coagulation factors, factor Va and factor VIIIa, thereby inhibiting the conversion of factor X to factor Xa and of prothrombin to thrombin. Additionally, activated protein C enhances tissue-plasminogen activator-mediated fibrinolysis by inhibition of plasminogen activator inhibitor-1. This results in an increase in circulatory plasminogen activator levels. Protein C deficiency, a genetic or acquired thrombophilic abnormality, has been demonstrated to predispose to episodes of potentially blinding and lethal thromboembolic events. Heterozygous-deficient subjects usually remain asymptomatic until adolescence or adulthood. In homozygous-deficient patients, protein C activity is usually less than 1% (reference range, 70%-140%), resulting in thromboembolism as early as in the neonatal period. The major clinical symptoms in affected newborn infants have been purpura fulminans, vitreous hemorrhage, and central nervous system thrombosis. The age of onset of the first symptoms has ranged from a few hours to 2 weeks after birth, usually after an uncomplicated full-term pregnancy and delivery. In contrast to the genetic form, acquired neonatal protein C deficiency occurs particularly in ill preterm babies. Typical complications of prematurity such as respiratory distress syndrome, necrotizing enterocolitis, and neonatal sepsis may also be present. In the medical literature, there are only a few reports of homozygous protein C deficiency in neonates. We present 2 cases of homozygous protein C deficiency with ocular and extraocular manifestation. PMID:10428594

Hattenbach, L O; Beeg, T; Kreuz, W; Zubcov, A

1999-06-01

159

Dermatologic manifestations of polycystic ovary syndrome.  

PubMed

Polycystic ovary syndrome (PCOS) affects 5-10% of reproductive-aged women and is one of the most common endocrine disorders in women. The disorder is commonly characterized by elevated levels of androgen and insulin. Women with PCOS may present with a range of signs and symptoms, and face increased risks of reproductive, metabolic, cardiovascular, psychologic, and neoplastic sequelae, particularly if the condition is left unrecognized or untreated. The clinical definition of PCOS has changed in recent years and includes as one of its cardinal criteria the dermatologic manifestations of hyperandrogenism, chiefly hirsutism, acne vulgaris, and androgenetic alopecia. Acanthosis nigricans, a cutaneous sign of hyperinsulinemia, may also be present. These dermatologic features may provide early clinical clues to recognition of PCOS, and treatment of these cutaneous conditions may improve the patient's quality of life and psychologic well-being. The effects of androgen on pilosebaceous units in the skin can vary by anatomic location, producing pathophysiologic effects on hair growth and differentiation, sebaceous gland size and activity, and follicular keratinization. Treatment modalities may include hormonal therapy intended to modulate androgen production and action as well as non-hormonal therapies directed toward specific dermatologic conditions. PMID:17645376

Lee, Amy T; Zane, Lee T

2007-01-01

160

Rheumatic manifestations in inflammatory bowel disease.  

PubMed

Musculoskeletal symptoms (articular, periarticular and muscular involvement, osteoporosis and related fractures, and fibromyalgia) are the most common frequent extra-intestinal manifestations of inflammatory bowel disease (IBD) and affect 6-46% of patients. IBD-related arthropathy is one of a group of inflammatory arthritides known as seronegative spondyloarthropathies (SpA), which also includes idiopathic ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis, and undifferentiated SpA. The articular involvement in IBD significantly affects the patients' quality of the life. Although magnetic resonance imaging (MRI) is still the gold standard for assessing entheseal involvement, ultrasonography (US) is a non-invasive and easily reproducible means of detecting early pathological changes in SpA patients. It can identify characteristic features of SpA such as enthesitis, bone erosions, synovitis, bursitis, and tenosynovitis and is therefore helpful for diagnostic purposes. Anti-TNF drugs should be used to treat AS patients with axial and peripheral symptoms (arthritis and enthesitis) who have persistently high levels of disease activity despite conventional treatment, and adalimumab and infliximab can also be beneficially used in patients with IBD. PMID:23774109

Atzeni, Fabiola; Defendenti, Caterina; Ditto, Maria Chiara; Batticciotto, Alberto; Ventura, Donatella; Antivalle, Marco; Ardizzone, Sandro; Sarzi-Puttini, Piercarlo

2014-01-01

161

Neurological manifestation of methyl bromide intoxication.  

PubMed

Methyl bromide is a highly toxic gas with poor olfactory warning properties. It is widely used as insecticidal fumigant for dry foodstuffs and can be toxic to central and peripheral nervous systems. Most neurological manifestations of methyl bromide intoxication occur from inhalation. Acute toxicity characterized by headache, dizziness, abdominal pain, nausea, vomiting and visual disturbances. Tremor, convulsion, unconsciousness and permanent brain damage may occur in severe poisoning. Chronic exposure can cause neuropathy, pyramidal and cerebellar dysfunction, as well as neuropsychiatric disturbances. The first case of methyl bromide intoxication in Thailand has been described. The patient was a 24-year-old man who worked in a warehouse of imported vegetables fumigated with methyl bromide. He presented with unstable gait, vertigo and paresthesia of both feet, for two weeks. He had a history of chronic exposure to methyl bromide for three years. His fourteen co-workers also developed the same symptoms but less in severity. Neurological examination revealed ataxic gait, decreased pain and vibratory sense on both feet, impaired cerebellar signs and hyperactive reflex in all extremities. The serum concentration of methyl bromide was 8.18 mg/dl. Electrophysilogical study was normal. Magnetic resonance imaging of the brain (MRI) revealed bilateral symmetrical lesion of abnormal hypersignal intensity on T2 and fluid-attenuation inversion recovery (FLAIR) sequences at bilateral dentate nuclei of cerebellum and periventricular area of the fourth ventricle. This incident stresses the need for improvement of worker education and safety precautions during all stages of methyl bromide fumigation. PMID:18575299

Suwanlaong, Kanokrat; Phanthumchinda, Kammant

2008-03-01

162

Oral manifestations in HIV+ children in Mozambique.  

PubMed

The scope of this study was to identify the prevalence of oral manifestations in HIV+/AIDS patients at the DIA Pediatric Hospital of Maputo. All 90 patients were included in the research. Data on dental caries (dmft/DMFT index), soft tissues and saliva flow were analyzed. Information on diet and hygienic habits was obtained in a semi-structured questionnaire. The t- student and chi-square tests were used for statistical assessment. The most frequent oral lesion was candidiasis (5.5%) and upon further oral examination the prevalence of parotid enlargement was 23.0%. The mean dmft 2.6 (SD 3.6) was considerably higher in relation to DMFT of 0.6 (SD 1.6), and the differences between them were statistically significant (P<0.05). The occurrence of mucosal lesions was higher in children who did not take antiretroviral therapy (ART) (p = 0.026). The use of ART is associated with reduced prevalence of oral lesions in HIV+ patients; however, rampant caries were highest in this group. Primary dentition of HIV+ patients should be considered high risk for caries. PMID:22218539

Sales-Peres, Sílvia Helena de Carvalho; Mapengo, Marta Artemisa Abel; de Moura-Grec, Patrícia Garcia; Marsicano, Juliane Avansine; Sales-Peres, André de Carvalho; Sales-Peres, Arsenio

2012-01-01

163

Monogenic Autoinflammatory Diseases: Concept And Clinical Manifestations  

PubMed Central

The objectives of this review are to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary “periodic fever syndromes”, familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) 6. very rare conditions presenting with autoinflammation and immunodeficiency.

De Jesus, Adriana Almeida; Goldbach-Mansky, Raphaela

2013-01-01

164

Oral manifestations of hepatitis C virus infection  

PubMed Central

Extrahepatic manifestations (EHMs) of hepatitis C virus (HCV) infection can affect a variety of organ systems with significant morbidity and mortality. Some of the most frequently reported EHM of HCV infection, involve the oral region predominantly or exclusively. Oral lichen planus (OLP) is a chronic inflammatory condition that is potentially malignant and represents cell-mediated reaction to a variety of extrinsic antigens, altered self-antigens, or super antigens. Robust epidemiological evidence support the link between OLP and HCV. As the virus may replicate in the oral mucosa and attract HCV-specific T lymphocytes, HCV may be implicated in OLP pathogenesis. Sjögren syndrome (SjS) is an autoimmune exocrinopathy, characterized by dryness of the mouth and eyes and a multitude of other systemic signs and symptoms. SjS patients have also an increased risk of non-Hodgkin lymphoma. Patients with chronic hepatitis C do frequently have histological signs of Sjögren-like sialadenitis with mild or even absent clinical symptoms. However, it is still unclear if HCV may cause a disease mimicking SjS or it is directly responsible for the development of SjS in a specific subset of patients. Oral squamous cell carcinoma is the most common oral malignant tumour and at least in some part of the world could be linked to HCV.

Carrozzo, Marco; Scally, Kara

2014-01-01

165

Hypersensitivity manifestations to the fruit mango  

PubMed Central

The objectives of this study are 1) To review the published data and document the current knowledge on allergic manifestations to the fruit mango 2) To highlight the two distinct clinical presentations of hypersensitivity reactions caused by mango 3) To discuss the role of cross-reactivity 4) To increase awareness of potentially life threatening complications that can be caused by allergy to mango. An extensive search of the literature was performed in Medline/PubMed with the key terms "mango", "anaphylaxis", "contact dermatitis", "cross-reactivity", "food hypersensitivity", "oral allergy syndrome" and "urticaria". The bibliographies of all papers thus located were searched for further relevant articles. A total of 17 reports describing 22 patients were documented, including ten patients with immediate hypersensitivity reaction and twelve patients with delayed hypersensitivity reaction to mango. Ten of these patients (four with immediate reaction; six with delayed reaction) were from geographical areas cultivating mango, whereas twelve patients (six with immediate reaction; six with delayed reaction) were from the countries where large scale mango cultivation does not occur. The clinical features, pathogenesis and diagnostic modalities of both these presentations are highlighted. The fruit mango can cause immediate and delayed hypersensitivity reactions, as also "oral allergy syndrome". Although rare, it can even result in a life threatening event. Reactions may even occur in individuals without prior exposure to mango, owing to cross reactivity. It is imperative to recognize such a phenomenon early so as to avoid potentially severe clinical reactions in susceptible patients.

Sareen, Richa

2011-01-01

166

Gastrointestinal manifestations of Henoch-Schonlein Purpura.  

PubMed

Henoch-Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. The diagnostic criteria include palpable purpura with at least one other manifestation -- abdominal pain, IgA deposition, arthritis or arthralgia, or renal involvement. Immune complex deposits result in necrosis of the wall of small- and medium-sized arteries with infiltration of tissue by neutrophils and deposition of nuclear fragments, a process called leukocytoclastic vasculitis (LCV). It is often associated with infections, medications, or tumors. It may coexist with or mimic Crohn's disease. Periumbilical and epigastric pain worsens with meals, from bowel angina. Bleeding is usually occult or, less commonly, associated with melena. Intussusception, the most common surgical complication, is usually ileo-ileo or ileo-colic. Perforations, usually ileal, may occur spontaneously or be associated with intussusception. Ultrasound, recommended as the first diagnostic test, and CT scans may show intussusception and asymmetric bowel wall thickening mainly involving the jejunum and ileum. There are a range of endoscopic findings including gastritis, duodenitis, ulceration, and purpura, with the second portion of the duodenum characteristically being involved more than the bulb. Intestinal biopsies show IgA deposition and LCV in the submucosal vessels. Superficial biopsies may show inflammation, ulceration, edema, hemorrhage, and vascular congestion, presumably due to vasculitis-induced mucosal ischemia. The efficacy of corticosteroids in preventing severe complications or relapses is controversial. The majority of patients, however, improve spontaneously. PMID:18351468

Ebert, Ellen C

2008-08-01

167

Observational Manifestation of Chaos in Astrophysical Objects  

NASA Astrophysics Data System (ADS)

This book addresses a broad range of problems related to observed manifestations of chaotic motions in galactic and stellar objects, by invoking basic theory, numerical modeling, and observational evidence. For the first time, methods of stochastic dynamics are applied to actually observed astronomical objects, e.g. the gaseous disc of the spiral galaxy NGC 3631. In the latter case, the existence of chaotic trajectories in the boundary of giant vortices was recently found by the calculation of the Lyapunov characteristic number of these trajectories. The reader will find research results on the peculiarities of chaotic system behaviour; a study of the integrals of motion in self-consistent systems; numerical modeling results of the evolution process of disk systems involving resonance excitation of the density waves in spiral galaxies; a review of specific formations in stars and high-energy sources demonstrating their stochastic nature; a discussion of the peculiarities of the precessional motion of the accretion disk and relativistic jets in the double system SS 433; etc. This book stands out as the first one that deals with the problem of chaos in real astrophysical objects. It is intended for graduate and post-graduate students in the fields of non-linear dynamics, astrophysics, planetary and space physics; specifically for those dealing with computer modeling of the relevant processes. Link: http://www.wkap.nl/prod/b/1-4020-0935-6

Fridman, A.; Marov, M.; Miller, R.

2002-12-01

168

[Vestibular and cochlear manifestations in Fabry's disease].  

PubMed

Anderson-Fabry's disease corresponds to an inherited disorder transmitted by an X-linked recessive gene. The disease is caused by an alpha-galactosidase deficiency leading to an abnormal glycosphingolipid metabolism, resulting in glycosphingolipids deposits all over the body. The disease affects all organs over the body and can be responsible for central nervous system or renal failure, heart attack, which can lead for early death in absence of diagnosis and treatment. In addition to these life-threatening manifestations, other problems which may have a profound impact on quality of life, such as hearing loss, have been relatively neglected. Thus, a large proportion of patients with Fabry's disease suffer from sensorineural hearing loss, with both progressive hearing impairment and sudden deafness, and peripheral vestibular deficits with dizziness and vertigo. The exact pathophysiologic mechanism(s) of those otological complications is still studied, but both cochleo-vestibular disorder and vascular origin seems to be involved. For many years, only symptomatic treatment has been available. For the past ten years, the introduction of enzyme replacement therapy with recombinant agalsidase-? or -? provides new prospect for these patients, decreasing the risk of complications. Still on study, it may also be active both on hearing loss and vestibular disturbances. PMID:21211674

Malinvaud, D; Germain, D P; Benistan, K; Bonfils, P

2010-12-01

169

Behcet's Disease: Systemic and Ocular Manifestations  

PubMed Central

Aim. The aim of this study was to evaluate if patients with Behcet's disease who have ocular involvement have a more severe form of this disease as compared to patients with Behcet's disease alone. Methods. A total of 99 patients were included in the study. 76 patients were used as part of the examined group, and 23 patients formed a control group. Results. The following are the results of examined and control groups, respectively: recurrent oral aphthous ulcers 89.5%, 95.7%; genital ulcers 61.8%, 97.0%; articular involvement 72.4%, 65.2%; vasculitis 81.6%, 60.9%; positive pathergy test 25.0%, 47.8%. Higher frequency of genital ulcerations was noted in control group (P = 0.001). More than two major criteria were met in 100% of the cases. HLA B51 was present in 78.9% of the cases in the examined group and 43.5% of the cases in control group; thus there is significant difference between them (P = 0.001). Visual acuity >0.5 occurred in 76% (examined group). Most frequent ocular manifestations in the examined group were retinal periphlebitis 81.6%, periphlebitis and periarteritis 65%, and serofibrinous uveitis 63.2%. Macular edema as a complication was present in 63.2%. The majority of patients (55.3%) were treated with combined therapy consisting of cyclosporine A and systemic corticosteroids. In 38.2% of patients, laser photocoagulation was used on retinal periphery.

Paovic, Jelena

2013-01-01

170

Association of oral manifestations with ulcerative colitis  

PubMed Central

Aim The study aims to document the oral lesions in patients with UC. Background Inflammatory bowel disease (IBD) is comprised of two chronic, tissue-destructive and clinical entities including Crohn's disease (CD) and ulcerative colitis (UC), both of which are immunologically based. Patients and methods The population of the study includes fifty patients with UC, as the experimental group, and fifty patients without gastrointestinal disease as the control group. All patients had an oral examination and completed the relevant questionnaire. Results There was a significant statistical relationship among tongue coating (<0.0001), halitosis (<0.0001), and oral ulceration (0.001) in patients suffering from severe UC compared to the control group. Also there was a higher prevalence of oral manifestations in patients with moderate UC compared to that of control group; but taste change (0.001) was the only significant factor. Acidic taste and taste change were among the symptoms more commonly found in patients suffering from pancolitis (40% each). Conclusion The results of the present study show that patients with UC had more oral signs and symptoms than the patients in the normal group. Further researches on oral lesions in UC are also recommended so that these diseases can be better understood.

Elahi, Maryam; Samadi, Vahid; Vakili, Hadi

2012-01-01

171

Neurologic Manifestations of Chronic Methamphetamine Abuse  

PubMed Central

COMMENTARY ON METHAMPHETAMINE ABUSE FOR PSYCHIATRIC PRACTICE Every decade seems to have its own unique drug problem. The 1970s had hallucinogens, the 1980s had crack cocaine, the 1990s had designer drugs, the 2000s had methamphetamine (Meth), and in the 2010s we are dealing with the scourge of prescription drug abuse. While each of these drug epidemics has distinctive problems and history, the one with perhaps the greatest impact on the practice of Psychiatry is Meth. By increasing the extracellular concentrations of dopamine while slowly damaging the dopaminergic neurotransmission, Meth is a powerfully addictive drug whose chronic use preferentially causes psychiatric complications. Chronic Meth users have deficits in memory and executive functioning as well as higher rates of anxiety, depression, and most notably psychosis. It is because of addiction and chronic psychosis from Meth abuse that the Meth user is most likely to come to the attention of the practicing Psychiatrist/Psychologist. Understanding the chronic neurologic manifestations of Meth abuse will better arm practitioners with the diagnostic and therapeutic tools needed to make the Meth epidemic one of historical interest only.

Rusyniak, Daniel E.

2013-01-01

172

Cytogenetics of extramedullary manifestations in multiple myeloma.  

PubMed

Extramedullary disease in patients with multiple myeloma is a rare event, occurring mostly in advanced disease or relapse. Outcome is poor and prognostic factors predicting the development of extramedullary disease have not been defined. We investigated cytogenetic alterations of myeloma cells in different extramedullary manifestations by adapting the fluorescence in situ hybridization (FISH) technique in combination with cytoplasmic immunoglobulin staining to study the cytogenetics of plasma cell tumours on paraffin embedded material. Thirty six patients were investigated: 19 with extramedullary disease, 11 with skeletal extramedullary disease and six with solitary extramedullary plasmacytoma. The first two groups showed the following results: del(17p13) 32% vs. 27%, del(13q14) 35% vs. 27%, MYC-overrepresentation 28% vs. 18% and t(4;14) 37% vs. 18%. We detected an overall higher incidence of del(17p13) in both groups compared to data from bone marrow samples of multiple myeloma reported to date (range 7-16%). The solitary extramedullary plasmacytomas presented overall less cytogenetic aberrations than the other groups. Most important, three patients with extramedullary disease and one with skeletal extramedullary disease presented different FISH findings in the extramedullary tumour compared to their bone marrow plasma cells. del(17p13), occurring additional in three of four cases, seems a strong marker for extramedullary progression of myeloma. PMID:23368088

Billecke, Lisa; Murga Penas, Eva M; May, Annette M; Engelhardt, Monika; Nagler, Arnon; Leiba, Merav; Schiby, Ginette; Kröger, Nicolaus; Zustin, Jozef; Marx, Andreas; Matschke, Jakob; Tiemann, Markus; Goekkurt, Eray; Heidtmann, Hans-Heinrich; Vettorazzi, Eik; Dierlamm, Judith; Bokemeyer, Carsten; Schilling, Georgia

2013-04-01

173

Multiple hepatic nodules: rare manifestation of clonorchiasis.  

PubMed

A 38-year-old man was admitted due to intermittent right upper quadrant pain for 1 month. Leukocytosis with marked eosionphilia and elevated serum alkaline phosphatase were noted. Stool examinations revealed no parasites or ova. Ultrasonography and computed tomography disclosed multiple hepatic tumors. Biopsy of the hepatic tumor was performed due to non-conclusive imaging studies and revealed eosinophil infiltration in portal areas only. Endoscopic retrograde cholangiography showed mild dilatation with irregularity of bilateral intrahepatic ducts, compatible with chronic cholangitis. Bile was aspirated and biliary lavage with normal saline was performed during endoscopy-guided biliary cannulation. Microscopic examination of the aspirate showed the characteristic ova of Clonorchis sinensis. The patient received Praziquantel therapy for 1 day. Abdominal pain reduced in intensity gradually. Eosinophilia and multiple hepatic lesions resolved after adequate treatment of Clonorchis sinensis. The rare manifestation of multiple hepatic tumors in Clonorchis sinensis should be differentiated from other primary or metastatic neoplasms, while biliary lavage for parasite ova is a valuable diagnostic tool when stool examination is negative. PMID:16911704

Liao, Wei-Chih; Wang, Hsiu-Po; Chiu, Han-Mo; Chang, Chi-Yang; Lin, Jaw-Town

2006-09-01

174

HCV infection: pathogenesis, clinical manifestations and therapy.  

PubMed

Chronic hepatitis C virus (HCV) infection is a worldwide public health problem with a global prevalence of 2-3%. It is believed that about 170 million people are currently infected (about 3% of the world's population), and a further 3-4 million are infected each year. HCV is the main reason for liver transplantation in the developed world, and the main cause of liver-related morbidity and mortality in a number of countries, including Italy. It is not only a frequent cause of chronic liver diseases such as hepatitis, cirrhosis and hepatocellular carcinoma, but is also involved in the pathogenesis of various autoimmune and rheumatic disorders (arthritis, vasculitis, sicca syndrome, porphyria cutanea tarda, lichen planus, nephropathies, thyroid diseases, and lung fibrosis), as well as in the development of B-cell lymphoproliferative diseases. Furthermore, patients suffering from C hepatitis tend to produce rheumatoid factor, cryoglobulins and a large series of autoantibodies (ANA, anti-SSA/SSB, SAM, ATG, aCL). The use of glucocorticoids or immuno-suppressant agents in HCV infected individuals, which are needed to treat autoimmune and rheumatic disorders, leads to a risk of worsening the clinical outcome of HCV. Under these conditions, the viral infection often needs to be treated with antiviral agents, mainly pegylated interferon combined with ribavirin. However, cyclosporine A seems to be safe and effective in patients with autoimmune disease (AD) and concomitant chronic HCV infection as is documented by the reduction in viremia and transaminases, particularly in patients with high baseline levels. Finally, HCV is the main trigger of mixed cryoglobulinemia. An attempt at viral eradication is therefore indicated in most patients, and is particularly effective in the case of mild or moderate manifestations. In severe cases, rituximab is an apparently safe and effective alternative to conventional immunosuppression and, specifically, it controls B-cell proliferation. PMID:18570753

Antonelli, A; Ferri, C; Galeazzi, M; Giannitti, C; Manno, D; Mieli-Vergani, G; Menegatti, E; Olivieri, I; Puoti, M; Palazzi, C; Roccatello, D; Vergani, D; Sarzi-Puttini, P; Atzeni, F

2008-01-01

175

19 CFR 122.48 - Air cargo manifest.  

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false Air cargo manifest. 122.48 Section 122...HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry...Overflying the United States § 122.48 Air cargo manifest. (a) When...

2013-04-01

176

Orofacial manifestations of hematological disorders: anemia and hemostatic disorders.  

PubMed

The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial signs and symptoms include iron deficiency anemia, Plummer-Vinson syndrome, megaloblastic anemia, sickle cell anemia, thalassaemia and aplastic anemia. The manifestations include conjunctiva and facial pallor, atrophic glossitis, angular stomatitis, dysphagia, magenta tongue, midfacial overgrowth, osteoclerosis, osteomyelitis and paraesthesia/anesthesia of the mental nerve. Orofacial petechiae, conjunctivae hemorrhage, nose-bleeding, spontaneous and post-traumatic gingival hemorrhage and prolonged post-extraction bleeding are common orofacial manifestations of inherited hemostatic disorders such as von Willebrand's disease and hemophilia. A wide array of anemic and hemostatic disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself. PMID:22048588

Adeyemo, Titilope A; Adeyemo, Wasiu L; Adediran, Adewumi; Akinbami, Abd Jaleel A; Akanmu, Alani S

2011-01-01

177

19 CFR 122.48 - Air cargo manifest.  

Code of Federal Regulations, 2011 CFR

...2014-04-01 2014-04-01 false Air cargo manifest. 122.48 Section 122...HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry...Overflying the United States § 122.48 Air cargo manifest. (a) When...

2014-04-01

178

Ontological manifestation of product risk in service oriented environment  

Microsoft Academic Search

Risk is omnipresent in all the interactions that we carry out. Quantifying and making risk-based assessments and decisions is very important especially in the case of business interactions. However in the existing literature there is no ontological manifestation of risk and its related concepts. In this paper, we propose an ontological manifestation of risk. Subsequently it is extended to derive

Sudha Catherine Arathoon; Anhar Fazal; Omar Khadeer Hussain; Farookh Khadeer Hussain; Elizabeth Chang

2008-01-01

179

Clinical Manifestations Associated with Neurocysticercosis: A Systematic Review  

PubMed Central

Background The clinical manifestations of neurocysticercosis (NCC) are poorly understood. This systematic review aims to estimate the frequencies of different manifestations, complications and disabilities associated with NCC. Methods A systematic search of the literature published from January 1, 1990, to June 1, 2008, in 24 different electronic databases and 8 languages was conducted. Meta-analyses were conducted when appropriate. Results A total of 1569 documents were identified, and 21 included in the analysis. Among patients seen in neurology clinics, seizures/epilepsy were the most common manifestations (78.8%, 95%CI: 65.1%–89.7%) followed by headaches (37.9%, 95%CI: 23.3%–53.7%), focal deficits (16.0%, 95%CI: 9.7%–23.6%) and signs of increased intracranial pressure (11.7%, 95%CI: 6.0%–18.9%). All other manifestations occurred in less than 10% of symptomatic NCC patients. Only four studies reported on the mortality rate of NCC. Conclusions NCC is a pleomorphic disease linked to a range of manifestations. Although definitions of manifestations were very rarely provided, and varied from study to study, the proportion of NCC cases with seizures/epilepsy and the proportion of headaches were consistent across studies. These estimates are only applicable to patients who are ill enough to seek care in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases.

Carabin, Helene; Ndimubanzi, Patrick Cyaga; Budke, Christine M.; Nguyen, Hai; Qian, Yingjun; Cowan, Linda Demetry; Stoner, Julie Ann; Rainwater, Elizabeth; Dickey, Mary

2011-01-01

180

[Siblings present different clinical manifestations in white sponge nevus].  

PubMed

White sponge nevus (WSN) is a rare, autosomal-dominant hereditary disease. This article reported two siblings affected by white sponge nevus. Because of smoking or not, they presented different clinical manifestations. PMID:23991589

Lin, Duanxian; Wu, Lanyan; Zhang, Lin; Zhou, Hongmei

2013-08-01

181

Tracheoesophageal fistula as the presenting manifestation of Hodgkin's lymphoma.  

PubMed Central

We present a patient with tracheoesophageal fistula as the initial manifestation of Hodgkin's disease with oesophageal involvement. To our knowledge, this has not been previously reported. The diagnosis of Hodgkin's lymphoma was made at autopsy. Images Figure 1

Alba, D.; Lobato, S. D.; Alvarez-Sala, R.; Villasante, C.; Echevarria, C.

1994-01-01

182

Manifest Anxiety and Self-Concept: Further Investigations  

ERIC Educational Resources Information Center

Manifest Anxiety Scale (MAS), Actual-Self, Ideal-Self, and Self-Ideal Discrepancy results from 134 first-year female nursing students were studied by the multivariate methods of factor analysis and multiple regression.

Furlong, Allannah; LaForge, Hubert

1975-01-01

183

Dimensions of Manifest Anxiety and Their Relationship to College Achievement  

ERIC Educational Resources Information Center

Administration of the Taylor Manifest Anxiety scale to 153 male and 107 female college freshmen and factor analysis of the results, revealed that the only significant correlation was between psychosomatic fitness" and grades for males. (KJ)

Khan, S. B.

1970-01-01

184

Bilateral keratitis as a manifestation of Lyme disease.  

PubMed

An 11-year-old girl developed bilateral keratitis, which we believe was a manifestation of Lyme disease. She had had several attacks of Lyme arthritis and was twice treated with parenteral penicillin. The keratitis developed five years after the initial episode of Lyme arthritis at a time when there were no other manifestations of Lyme disease. It cleared completely in both eyes after topical corticosteroid therapy. PMID:3337196

Baum, J; Barza, M; Weinstein, P; Groden, J; Aswad, M

1988-01-15

185

Superspace with manifest T-duality from type II superstring  

NASA Astrophysics Data System (ADS)

A superspace formulation of type II superstring background with manifest T-duality symmetry is presented. This manifestly T-dual formulation is constructed in a space spanned by two sets of nondegenerate super-Poincaré algebras. Supertorsion constraints are obtained from consistency of the ?-symmetric Virasoro constraints. All superconnections and vielbein fields are solved in terms of a prepotential which is one of the vielbein components. AdS5×S5 background is explained in this formulation.

Hatsuda, Machiko; Kamimura, Kiyoshi; Siegel, Warren

2014-06-01

186

Intracranial fibromuscular dysplasia presenting as various ocular manifestations.  

PubMed

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that mainly affects renal, extracranial carotid, and vertebral arteries. Intracranial FMD is uncommon unlike extracranial or renal FMD, and the primary manifestation of intracranial FMD is intracranial aneurysm. We report an unusual case of intracranial FMD showing various ocular manifestations, including central retinal artery occlusion, transient monocular blindness, and oculomotor nerve palsy without renal involvement. PMID:24360187

Choi, Jae-Hwan; Jung, Jaeho; Park, Kyung-Pil; Baik, Seung Kuk; Choi, Kyung Un; Choi, Kwang-Dong; Choi, Hee-Young; Shin, Jong-Hoon

2014-02-15

187

Rheumatologic manifestations of benign and malignant haematological disorders  

Microsoft Academic Search

Diseases of blood and lymphoreticular system can have multisystem manifestations. Rheumatologic involvement has been reported\\u000a in association with many benign and malignant haematological disorders; these patients are equally likely to present to both\\u000a clinical rheumatologists and haematologists. This review focuses on the well-described rheumatologic features, other occasionally\\u000a reported rheumatologic manifestations and unusual musculoskeletal complications related to the treatment in patients

Vinod Ravindran; Parameswaran Anoop

188

Mucocutaneous manifestations of acquired hypoparathyroidism: An observational study.  

PubMed

Hypoparathyroidism is a disorder of calcium and phosphorus metabolism due to decreased secretion of parathyroid hormone. Hypoparathyroidism can be hereditary and acquired. Acquired hypoparathyroidism usually occurs following neck surgery (thyroid surgery or parathyroid surgery). Along with systemic manifestations, hypoparathyroidism produces some skin manifestations. Lack of study regarding mucocutaneous manifestations of acquired hypoparathyroidism prompted us to undertake this study. To evaluate the mucocutaneous manifestations of acquired hypoparathyroidism. An observational study done in a tertiary care hospital of Kolkata by comprehensive history taking, through clinical examination and relevant laboratory investigations. Twenty-one patients were included in the study. The commonest form of acquired hypoparathyroidism was neck surgery (thyroidectomy and parathyroidectomy operation). Mucocutaneous manifestations were present in 76.19% of patients. The most frequent mucocutaneous manifestation was found in the hairs like the loss of axillary hair (61.9%), loss of pubic hair (52.38%), coarsening of body hair (47.62%), and alopecia areata (9.52%). The nail changes noted were brittle and ridged nail, followed by onycholysis, onychosezia, and onychomedesis. The most common skin features were xerotic skin in 11 patients (52.38%), followed by pellagra-like skin pigmentation, pustular psoriasis and acne form eruption, bullous impetigo, etc. Mucosa was normal in all the cases excepting the one which showed oral candidiasis. PMID:23087872

Sarkar, Somenath; Mondal, Modhuchanda; Das, Kapildev; Shrimal, Arpit

2012-09-01

189

8 CFR 251.5 - Paper arrival and departure manifests for crew.  

Code of Federal Regulations, 2010 CFR

... 2010-01-01 false Paper arrival and departure manifests for crew...SECURITY IMMIGRATION REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS...SUPPORTING DOCUMENTS § 251.5 Paper arrival and departure manifests for...

2010-01-01

190

8 CFR 251.5 - Paper arrival and departure manifests for crew.  

Code of Federal Regulations, 2010 CFR

... 2009-01-01 false Paper arrival and departure manifests for crew...SECURITY IMMIGRATION REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS...SUPPORTING DOCUMENTS § 251.5 Paper arrival and departure manifests for...

2009-01-01

191

78 FR 59365 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...  

Federal Register 2010, 2011, 2012, 2013

...Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...concerning the Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...collection: Title: Entry and Manifest of Merchandise Free of Duty, Carrier's...

2013-09-26

192

76 FR 62086 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...  

Federal Register 2010, 2011, 2012, 2013

...Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...concerning the Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...collection: Title: Entry and Manifest of Merchandise Free of Duty, Carrier's...

2011-10-06

193

76 FR 76983 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...  

Federal Register 2010, 2011, 2012, 2013

...Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...Reduction Act: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...information. Title: Entry and Manifest of Merchandise Free of Duty, Carrier's...

2011-12-09

194

78 FR 76153 - Agency Information Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier...  

Federal Register 2010, 2011, 2012, 2013

...Collection Activities: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...Reduction Act: Entry and Manifest of Merchandise Free of Duty, Carrier's Certificate...information. Title: Entry and Manifest of Merchandise Free of Duty, Carrier's...

2013-12-16

195

Dry cough is a frequent manifestation of giant cell arteritis.  

PubMed

Physicians need to be familiar with the typical manifestations of giant cell arteritis. However, the challenge lies in recognizing atypical cases that lack the more specific manifestations or reflect vasculitis in less frequently involved territories. Among atypical clinical manifestations, dry cough has been reported in recent years. The literature contains sporadic reports mainly single case report. The objective of this study was to determine the frequency of dry cough in patients with giant cell arteritis. Clinical data were collected from 88 patients with giant cell arteritis. Relationships between dry cough and other clinical manifestations or biological data were analyzed. Dry cough of recent appearance was found at initial presentation of giant cell arteritis in 12 patients (13.6%). In 2 cases, dry cough was isolated. The 2 patients sought attention because of chronic dry cough associated with inflammation of unknown origin. In 10 cases, dry cough was associated with typical clinical manifestations of giant cell arteritis. A correlation was found between inflammatory biomarkers and presence of dry cough. The mean CRP was 153.8 mg/l (SD 85.1) in patients with dry cough and 94 mg/l (SD 72.2) in patients without dry cough (p = 0.0131). We conclude that the diagnosis of giant cell arteritis should always be considered in an elderly patient with an unexplained elevation of inflammatory markers and chronic dry cough. Dry cough in giant cell arteritis was not correlated with other clinical manifestations of this vasculitis, including pulmonary manifestations, but was correlated with inflammatory biomarkers. PMID:22451028

Zenone, Thierry; Puget, Marie

2013-08-01

196

Cutaneous Manifestations of Lightning Injury: A Case Report  

PubMed Central

Background: Lightning injuries are relatively uncommon and have been a subject of awe since primitive times. It most significantly affects the cardiorespiratory, nervous, and integumentary systems. Surprisingly, cutaneous burn injuries caused by lightning are usually superficial. Objective: To present the cutaneous manifestations of lightning injuries and the sequelae of improper management. Case report: A 22-year-old woman presented with cutaneous manifestations of lightning-induced burns and bilateral upper limb gangrene after 2 months of improper treatment. She refused amputation after counseling and left the hospital. Conclusion: This is a rare case of burns with cutaneous manifestations peculiar to lightning injury. These features serve as evidence of lightning injury, when in doubt, especially in societies where superstition is rife. Education concerning the nature of lightning and proper management would improve outcome.

Asuquo, M. E.; Ikpeme, I. A.; Abang, I.

2008-01-01

197

Large cerebellar mass lesion: A rare intracranial manifestation of blastomycosis  

PubMed Central

Background: Blastomyces dermatitidis is a dimorphic fungus found endemically in the Mississippi and Ohio River basins and in the Midwestern and Canadian provinces that border the Great Lakes. Unlike other fungal infections, it most commonly affects immunocompetent hosts. Blastomycosis typically manifests as pulmonary infection, but may affect nearly any organ, including the skin, bone, and genitourinary system. Central nervous system (CNS) blastomycosis is rare, but potentially fatal manifestation of this disease. When it does occur, it most commonly presents as acute or chronic meningitis. Case Description: We present a case of a patient who suffered intractable nausea and vomiting for several months before discovery of a large cerebellar blastomycoma causing mass effect and obstructive hydrocephalus. The enhancing lesion with unusual peripheral cystic structures is a unique radiographic appearance of CNS blastomycosis. Conclusion: We review this patient's purely intraparenchymal manifestation of CNS blastomycosis and describe the unique imaging characteristics encountered.

Munich, Stephan A.; Johnson, Andrew K.; Ahuja, Sumeet K.; Venizelos, Alexander; Byrne, Richard W.

2013-01-01

198

Dermatological manifestations of hepatitis C virus infection in Saudi Arabia.  

PubMed

The Saudi Ministry of Health data indicates that almost 32% of viral hepatitis cases were caused by hepatitis C virus (HCV). It has been widely reported that chronic HCV infection is associated with and may trigger or exacerbate many skin manifestations in 20-40% of patients visiting dermatologists. The most commonly encountered dermatological manifestations of HCV infection globally include mixed cryoglobulinemia, porphyria cutanea tarda, cutaneous and/or oral lichen planus, urticaria, pruritus, thrombocytopenic purpura, and psoriasis. The current article indicates that HCV infection is increasing in Saudi Arabia and approximately 12% of the reported dermatological manifestations are caused by HCV infection. We recommend the urgent need for large-scale, case-control studies to understand the impact of HCV infection in patients with skin disease. PMID:24888650

Halawani, Mona R

2014-06-01

199

Database basics help manage hazardous waste manifest data  

SciTech Connect

Hazardous waste generators spend considerable time filling out uniform hazardous waste manifests and such related documents as land disposal forms, profile sheets and laboratory test results. For many environmental professionals, the continual demand for updating and maintaining records is a burdensome task, especially when some records may be outdated, illegible or otherwise difficult to manage. One solution to the paperwork maze is using a commercial software program that allows users to print legible copies of manifests. Some programs create summary reports detailing shipping destinations, annual waste generation totals and other information. Features vary among available programs, and additional capabilities may require customized programming, which can be expensive. For those with reservations about committing to buying or using software specifically designed to produce or manage manifests -- or who have limited budgets -- generic, off-the-shelf database programs designed for personal computers (PCs) or Apple Macintosh equipment offer low-cost alternatives.

Nielsen, E.S. (Varian Associates Inc., Palo Alto, CA (United States))

1993-07-01

200

Gastrointestinal and Hepatic Manifestations of Primary Immune Deficiency Diseases  

PubMed Central

Primary immune deficiency diseases (PIDs) are a heterogeneous group of inherited diseases characterized by variable genetic immune defects, conferring susceptibility to recurrent infections. They have a vast array of manifestations some of which involve the gastrointestinal and hepatobiliary systems. These complications can be the consequence of five different factors, namely, infection, autoimmune process, unregulated inflammation, malignancies and complications of therapeutic intervention. They may precede the PID diagnosis and, once developed, they pose high risk of morbidity. Untrained clinicians may treat these manifestations only at the level of their presentation, leaving the PIDs dangerously undiagnosed. In fact, early diagnosis of PIDs and accompanied gastrointestinal and hepatic complications clearly require appropriate treatment, and in-turn lead to an improved quality of life for the patient. To improve the awareness of gastroenterologists and related health care providers about these diseases, we have reviewed herein the complications of different PIDs focusing on gastrointestinal and hepatic manifestation.

Al-Muhsen, Saleh Z.

2010-01-01

201

Characteristics of 10 patients with paraneoplastic rheumatologic musculoskeletal manifestations.  

PubMed

Abstract Objectives. To evaluate the possible correlation of malignant neoplasms and paraneoplastic rheumatologic syndromes. Methods. We studied a series of 10 patients with paraneoplastic rheumatological syndromes collected from our Division of Rheumatic Disease between 2006 and 2012. Results. Our series consisted of four males and six females, with a mean age of 65.5 years (range, 57-78 years). Of the 10 patients recruited, six had hematological malignancies and four had solid cancers. Malignancies were diagnosed after rheumatic symptoms were reported in all patients. Compared to solid tumors, hemopathy was diagnosed at a later time point (16.2 vs. 7.3 months). Extra-articular symptoms were associated with rheumatologic musculoskeletal manifestations in 100% of the patients. Polyarthritis was the main rheumatologic musculoskeletal manifestation (50% of the patients). The other manifestations were oligopolyarthritis and polymyalgia rheumatic-like symptoms (20% of the patients). Symmetric arthritis was present in 60% of the patients, and the remaining patients developed asymmetric arthritis. Musculoskeletal manifestations completely regressed in 66.7% of the patients after cancer therapy. When tumor relapse was observed, rheumatic symptoms did not recur in any of our patients (100%). Conclusions. Rheumatic disorders with atypical clinical presentation in older patients, poor response to usual treatment and systemic features such as weight loss and clinical findings compatible with well-recognized paraneoplastic syndromes should alert clinicians to the possible coexistence of an occult malignancy. Especially in cases of paraneoplastic rheumatic/musculoskeletal manifestations associated with hemopathy, the primary disease is unlikely to have manifested yet, making the diagnosis difficult. Thus, caution is required. PMID:24252036

Yamashita, Hiroyuki; Ueda, Yo; Ozaki, Takashi; Tsuchiya, Haruka; Takahashi, Yuko; Kaneko, Hiroshi; Kano, Toshikazu; Mimori, Akio

2014-05-01

202

Use of Biologic Agents in Ocular Manifestations of Rheumatic Disease  

PubMed Central

Biologic agents have dramatically shifted the treatment paradigm for rheumatic disease. Use of these agents can decrease disease burden, allow the patient to be weaned from corticosteroids, and reduce the likelihood of relapse. Eye disease associated with rheumatic conditions may present with a wide range of signs and symptoms. This coexisting pathology should not be overlooked and should be considered a reason for initiation or continuation of biologic therapy. Additionally, many of the ocular manifestations of rheumatic disease respond preferentially to specific targeting molecules. This paper summarizes the available studies on the use, efficacy, and safety of biologic agents in the treatment of ocular manifestations of rheumatic disease.

Kraus, Courtney L.; Culican, Susan M.

2012-01-01

203

Ocular manifestations of the potentially lethal rheumatologic and vasculitic disorders.  

PubMed

Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving. PMID:23688612

Foster, C Stephen

2013-06-01

204

An Unusual Cardiac Manifestation in Autosomal Dominant Polycystic Kidney Disease  

PubMed Central

Autosomal dominant polycystic kidney disease is a common hereditary disorder characterized by renal and extrarenal, cystic and noncystic manifestations. Connective tissue defects, including cerebral aneurysm, meningeal diverticula, abdominal wall hernias, intestinal diverticula, and cardiac valvular abnormalities, are widely known manifestations. Instead intracardiac aneurysms have never been reported in adults with autosomal dominant polycystic kidney disease. We describe a 65-year-old patient with end-stage renal disease due to autosomal dominant polycystic kidney disease and an atrial septum aneurysm associated with platypnoea-orthodeoxia syndrome.

Catapano, Fausta; Pancaldi, Stefano; Napoleone, Carlo Pace; De Sanctis, Lucia Barbara; Gargiulo, Gaetano; Emiliani, Giuseppe; Santoro, Antonio

2012-01-01

205

Psychiatric and behavioral manifestations of lysosomal storage disorders.  

PubMed

The different lysosomal storage disorders (LSDs) manifest with a wide spectrum of clinical presentations. Most of these disorders are typically diagnosed early in life, due to the severity of the associated phenotypes. However, it is important to appreciate that some of the LSDs present later in adolescence or adulthood. The diverse findings triggering the initial diagnosis, as well as the range of manifestations arising later during the disease course, contribute to the complexity of these issues. Clinical presentations occurring at a more advanced age, especially psychiatric and behavioral manifestations, can be overlooked or misdiagnosed. This review describes different psychiatric and behavioral manifestations encountered in individuals with LSDs, including psychosis, schizophrenia, mood disorders, aggressiveness, early-onset dementia, and conduct disorder. Twelve different disorders are presented, including descriptions of their associated biochemical abnormalities, clinical presentations, pathology, epidemiology, and genetics. In addition, discussions of neurocognitive, behavioral, and psychiatric findings are outlined for each disorder. A greater awareness of these features may help to reduce missed diagnoses, to avoid unnecessary, invasive and expensive testing, and to facilitate an earlier detection of these rare disorders. Earlier diagnosis can enable the implementation of appropriate interventions and improve genetic counseling. PMID:20872765

Staretz-Chacham, Orna; Choi, Jae Hyuk; Wakabayashi, Kazuyo; Lopez, Grisel; Sidransky, Ellen

2010-10-01

206

Pathology and pathophysiology of pulmonary manifestations in leptospirosis  

Microsoft Academic Search

Leptospirosis is a re-emerging zoonosis occurring as large outbreaks throughout the world caused by Leptospira interrogans. The incidence of pulmonary involvement in leptospirosis has been reported to be increasing in the last years, affecting up to 70% of the patients. Alveolar hemorrhage presented as dyspnea and hemoptysis is the main pulmonary manifestation. The emergence of massive hemoptysis and acute respiratory

Marisa Dolhnikoff; Thais Mauad; Eduardo P. Bethlem; Carlos Roberto Ribeiro Carvalho

2007-01-01

207

The Development and Manifestation of Altruistic Caring: A Qualitative Inquiry  

ERIC Educational Resources Information Center

Qualitative, phenomenological research provides rich information about the constructive, life span perspectives of the manifestation and development of altruism. Using an interpretive phenomenological approach, this study investigated "altruism" as described by 34 older persons in a continuing care retirement community. The findings identified 13…

Curry, Jennifer R.; Smith, Heather L.; Robinson, Edward H., III

2009-01-01

208

Cricoarytenoiditis as an Initial Manifestation of Systemic Lupus Erythematosus  

PubMed Central

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease encompassing a broadened spectrum of clinical manifestations. Vocal cords involvement in SLE is not a frequent entity but can be life threatening if not treated. We hereby report the case of a patient presenting with cricoarytenoiditis and vocal cord dysfunction revealing SLE.

Hougardy, Jean-Michel; Roper, Nicolas; Michils, Alain; Soyfoo, Muhammad S.

2011-01-01

209

Cardiac tamponade as first manifestation of colonic cancer.  

PubMed

Metastatic cardiac tamponade was the presenting manifestation of a colonic carcinoma. The patient's condition improved after surgical treatment and shows no sign of recurrence of pericardial effusion after more than eight months of systemic chemotherapy. Metastatic pericardial effusions and their treatment are discussed. PMID:6703833

Kacenelenbogen, R; Devriendt, J; De Reuck, M; De Paepe, J; Decoodt, P; De Roy, G; Schmerber, J; Staroukine, M

1984-03-01

210

[Osteitis fibrosa cystica as the initial manifestation of primary hyperparathyroidism].  

PubMed

Primary hyperparathyroidism is a common endocrinological disease and most cases are asymptomatic. We report the case of a patient with primary hyperparathyroidism and symptomatic bone lesions. The possibility of different etiologies (adenoma vs carcinoma) according to the clinical manifestations and treatment are also discussed. PMID:19627728

Aragón Valera, Carmen; Carrasco de la Fuente, Marta; Alpañés Buesa, Macarena; Sanchón Rodríguez, Raúl; Antón Bravo, Teresa; de la Calle Blasco, Hermenegildo

2009-03-01

211

Clinical manifestations of disordered microcirculatory perfusion in severe sepsis  

Microsoft Academic Search

Microcirculatory dysfunction plays a pivotal role in the development of the clinical manifestations of severe sepsis. Prior to the advent of new imaging technologies, clinicians had been limited in their ability to assess the microcirculation at the bedside. Clinical evidence of microcirculatory perfusion has historically been limited to physical examination findings or surrogates that could be derived from global parameters

Stephen Trzeciak; Emanuel P Rivers

2005-01-01

212

Monostotic lumbar manifestation of fibrous dysplasia – a rare entity  

Microsoft Academic Search

Summary ¶Monostotic manifestation of fibrous dysplasia in the spine is exceedingly rare. We report the case of a 30 year-old woman suffering from slowly increasing low back pain. Radiologically a polycyclic, slightly gadolinium-enhancing, solitary lesion within the first lumbar vertebral body was detected. The lesion was removed and stabilized with bone marrow transplant. We describe the radiological and histopathological findings.

F. Hertel; Th. Hopf; W. Feiden; M. Bettag; C. Walter; G. Delling

2003-01-01

213

[Histology of the skin and mucous membrane manifestations of AIDS].  

PubMed

The dermatopathologist must be aware of the wide spectrum of non-specific cutaneous manifestations, cutaneous infections and skin tumors associated with AIDS. We present the histological criteria essential for the diagnosis of early Kaposi's sarcoma, its differential diagnosis including epithelioid angiomatosis, as well as the diagnosis of oral hairy leucoplakia. PMID:2205063

Langer, K; Ordonnez, M; Konrad, K

1990-07-01

214

Biological therapy for dermatological manifestations of inflammatory bowel disease  

PubMed Central

Ulcerative colitis and Crohn’s disease are the two forms of inflammatory bowel disease (IBD). The advent of biological drugs has significantly changed the management of these conditions. Skin manifestations are not uncommon in IBD. Among the reactive lesions (immune-mediated extraintestinal manifestations), erythema nodosum (EN) and pyoderma gangrenosum (PG) are the two major cutaneous ills associated with IBD, while psoriasis is the dermatological comorbidity disease observed more often. In particular, in the last few years, anti-tumor necrosis factor (TNF)-? agents have been successfully used to treat psoriasis, especially these kinds of lesions that may occur during the treatment with biological therapies. The entity of the paradoxical manifestations has been relatively under reported as most lesions are limited and a causal relationship with the treatment is often poorly understood. The reason for this apparent side-effect of the therapy still remains unclear. Although side effects may occur, their clinical benefits are undoubted. This article reviews the therapeutic effects of the two most widely used anti-TNF-? molecules, infliximab (a fusion protein dimer of the human TNF-? receptor) and adalimumab (a fully human monoclonal antibody to TNF-?), for the treatment of the major cutaneous manifestations associated with IBD (EN, PG and psoriasis).

Zippi, Maddalena; Pica, Roberta; De Nitto, Daniela; Paoluzi, Paolo

2013-01-01

215

40 CFR 264.71 - Use of manifest system.  

Code of Federal Regulations, 2013 CFR

...transporter, hazardous waste which is accompanied by a shipping paper containing all...that the hazardous waste covered by the manifest or shipping paper was received; ...13(c) include waste analysis must perform...signing the shipping paper and giving it...

2013-07-01

216

40 CFR 265.71 - Use of manifest system.  

Code of Federal Regulations, 2013 CFR

...transporter, hazardous waste which is accompanied by a shipping paper containing all the...that the hazardous waste covered by the manifest or shipping paper was received; ...13(c) include waste analysis must perform...signing the shipping paper and giving it...

2013-07-01

217

40 CFR 267.71 - Use of the manifest system.  

Code of Federal Regulations, 2013 CFR

...transporter, hazardous waste which is accompanied by a shipping paper containing all...that the hazardous waste covered by the manifest or shipping paper was received; ...13(c) include waste analysis must perform...signing the shipping paper and giving it...

2013-07-01

218

40 CFR 761.211 - Manifest system-Transporter requirements.  

Code of Federal Regulations, 2013 CFR

...or the shipping paper; and (4) The...delivering the PCB waste to the initial...manifest or shipping paper. (3) When delivering PCB waste to the designated...signed shipping paper in accordance with...delivering PCB waste to a...

2013-07-01

219

Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases  

Microsoft Academic Search

OBJECTIVE:Hemophagocytic syndrome has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, or lymph nodes in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. Hepatic dysfunction is often present but the nature of the hepatic lesions and related manifestations have not been fully characterized. The aim of this study was to ascertain

Caroline de Kerguenec; Sophie Hillaire; Vincent Molinié; Claude Gardin; Claude Degott; Serge Erlinger; Dominique Valla

2001-01-01

220

Radiculopathy as a manifestation of Langerhans' cell histiocytosis  

Microsoft Academic Search

Langerhans' cell histiocytosis (LCH) is a rare condition of children and young adults in which Langerhans' cells proliferate. The clinical spectrum ranges from solitary or few focal lesions to multisystem involvement mimicking vasculitis or hematological malignancy. Focal bone lesions, known as eosinophilic granulomas, are the most common manifestations. Eosinophilic granuloma usually presents with a variable combination of pain, swelling, fracture,

Athan Baillet; Laurent Grange; Pierre-André Lafaix; Philippe Gaudin; Robert Juvin

2007-01-01

221

Chronic urticaria: a cutaneous manifestation of celiac disease.  

PubMed

Celiac disease, or gluten-sensitive enteropathy, is an immune-mediated disease of the small bowel that results in malabsorption. It classically presents with gastrointestinal symptoms including chronic diarrhea, weight loss, abdominal bloating and anorexia. It is becoming more frequently identified in asymptomatic patients with a diagnosis of deficiencies related to malabsorption of iron, folic acid, vitamin B12 and vitamin D. It is increasingly identified as a cause for early or refractory osteoporosis. Occasionally, celiac disease presents with cutaneous manifestations alone. Dermatitis herpetiformis is a well-recognized cutaneous manifestation of celiac disease. Other cutaneous manifestations include alopecia, angular stomatitis and aphthous ulcerations. Described here is a case of a 24-year-old woman who presented with intermittent urticaria and gastrointestinal complaints. She was found to have celiac disease on small-bowel biopsy. Both her gastrointestinal symptoms and urticaria resolved when she was put on a gluten-free diet, suggesting that her urticaria was a cutaneous manifestation of celiac disease. PMID:16609761

Haussmann, Jessica; Sekar, Arni

2006-04-01

222

Life as a manifestation of the second law of thermodynamics  

Microsoft Academic Search

We examine the thermodynamic evolution of various evolving systems, from primitivephysical systems to complex living systems, and conclude that they involve similar processeswhich are phenomenological manifestations of the second law of thermodynamics. We take thereformulated second law of thermodynamics of Hatsopoulos and Keenan and Kestin and extendit to nonequilibrium regions, where nonequilibrium is described in terms of gradientsmaintaining systems at

Eric D. Schneider; James J. Kay

1994-01-01

223

Less common clinical manifestations of atopic dermatitis: prevalence by age.  

PubMed

The common manifestations of atopic dermatitis (AD) appear sequentially with involvement of the cheeks in infancy, flexural extremities in childhood, and hands in adulthood. Although less common clinical manifestations are well described, they have not been the subject of epidemiologic studies to describe their prevalence in specific age groups. This observational, cross-sectional, comparative study included 131 children younger than 18 of both sexes with AD who attended the clinics of the Dermatology Department of the National Institute of Pediatrics in Mexico City. Patients were examined to determine the presence of infrequent clinical manifestations of AD during infancy, preschool and school age, and adolescence and stratified according to sex, age, and number of clinical signs. A chi-square test was used to detect differences according to age and sex. Logistic regression analysis was also performed. The main findings according to age were genital dermatitis and papular-lichenoid dermatitis variant in infants; atopic feet, prurigo-like, nummular pattern, and erythroderma in preschool and school-aged children; and eyelid eczema and nipple dermatitis in adolescents. The risk of development of nipple dermatitis and eyelid eczema increased with age, and the development of genital dermatitis decreased with age. The knowledge of the prevalence of less common clinical manifestations of AD according to age in different populations might be helpful in diagnosing incipient cases of AD. PMID:22469300

Julián-Gónzalez, Rolando Elias; Orozco-Covarrubias, Luz; Durán-McKinster, Carola; Palacios-Lopez, Carolina; Ruiz-Maldonado, Ramon; Sáez-de-Ocariz, Marimar

2012-01-01

224

19 CFR 123.3 - Inward foreign manifest required.  

Code of Federal Regulations, 2013 CFR

...HOMELAND SECURITY; DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.3 Inward foreign...vessel of less than 5 net tons arriving otherwise than by sea from Canada or Mexico shall be listed on a manifest as prescribed...

2013-04-01

225

Chronic urticaria: A cutaneous manifestation of celiac disease  

PubMed Central

Celiac disease, or gluten-sensitive enteropathy, is an immune-mediated disease of the small bowel that results in malabsorption. It classically presents with gastrointestinal symptoms including chronic diarrhea, weight loss, abdominal bloating and anorexia. It is becoming more frequently identified in asymptomatic patients with a diagnosis of deficiencies related to malabsorption of iron, folic acid, vitamin B12 and vitamin D. It is increasingly identified as a cause for early or refractory osteoporosis. Occasionally, celiac disease presents with cutaneous manifestations alone. Dermatitis herpetiformis is a well-recognized cutaneous manifestation of celiac disease. Other cutaneous manifestations include alopecia, angular stomatitis and aphthous ulcerations. Described here is a case of a 24-year-old woman who presented with intermittent urticaria and gastrointestinal complaints. She was found to have celiac disease on small-bowel biopsy. Both her gastrointestinal symptoms and urticaria resolved when she was put on a gluten-free diet, suggesting that her urticaria was a cutaneous manifestation of celiac disease.

Haussmann, Jessica; Sekar, Arni

2006-01-01

226

Reliability Generalization of the Revised Children's Manifest Anxiety Scale  

ERIC Educational Resources Information Center

A reliability generalization of the Revised Children's Manifest Anxiety Scale (RCMAS) was conducted using the normative sample. The RCMAS consists of a Total Anxiety scale as well as four subscales. Results suggest that the Total Anxiety scores are typically reliable (median across 48 samples = .81). Subscale scores were less reliable: The median…

Ryngala, Donna J.; Shields, Alan L.; Caruso, John C.

2005-01-01

227

Extra-articular manifestations of seronegative and seropositive rheumatoid arthritis.  

PubMed

Although considered a "joint disease," rheumatoid arthritis is associated with the involvement of extra-articular manifestations. The aim of the study is the investigation and comparison of frequency and type of extra-articular manifestations in a well defined community based cohort of patients with seropositive and seronegative rheumatoid arthritis. Using the ACR (1987) criteria for rheumatoid arthritis, patients have been classified into the 2nd and 3rd functional class (ARA). The studied group consisted of 125 seronegative patients with titters lower than 1:64 as defined by Rose-Waaler test, whereas the control group consisted of 125 seropositive patients with titters of 1:64 or higher. All patients were between 25-60 years of age (Xb=49,96), with disease duration between 1-27 years (Xb=6,41). In order to present the findings of the study, the structure, prevalence, arithmetic mean (Xb), standard deviation (SB), variation quotient (QV%) and variation interval (Rmax-Rmin) have been used. Probability level has been expressed by p<0,01 and p<0,05. Correlation between the number of extra-articular manifestations and duration of the disease has been calculated by means of Pearson linear correlation. Higher presence of diffuse lung fibrosis, central and peripheral nervous system damages have been confirmed in the seropositive group, and osteoporosis in the seronegative; however, no statistical difference has been found. In extra-articular manifestations, "rheumatoid core" in the seropositive subset (chi2=4,80, p<0,05) presented significant statistical difference. Rheumatoid nodules were more frequent in seropositive subset (12%:16%), in both sexes; however, they were not of significant statistical difference. Neuropathy and lung diseases were also frequently present in seropositive group, but no statistical difference has been found regarding the statistical difference. Longer duration of the disease resulted in an increase of the number of extra-articular manifestations. Calculated linear correlation by Pearson, resulted as positive and high correlation in total (r=0,36, p<0,01), and for groups [(r=0,52, p<0,01) seronegative, (r=0,25, p<0,01) seropositive], nevertheless no significant statistical difference was found regarding the sero-status. In conclusion, extra-articular manifestations are more frequent in the seropositive patients. The longer the duration of the disease the larger the number of extra-articular manifestations. Differences with regard to sero-status and sex, with some exceptions, are not observed. PMID:20192927

Sahatciu-Meka, Vjollca; Rexhepi, Sylejman; Manxhuka-Kerliu, Suzana; Rexhepi, Mjellma

2010-02-01

228

Optic Neuritis as Isolated Manifestation of Leptomeningeal Carcinomatosis: A Case Report and Systematic Review of Ocular Manifestations of Neoplastic Meningitis  

PubMed Central

Introduction. Leptomeningeal carcinomatosis occurs in about 5% of cancer patients. Ocular involvement is a common clinical manifestation and often the presenting clinical feature. Materials and Methods. We report the case of a 52-year old lady with optic neuritis as isolated manifestation of neoplastic meningitis and a review of ocular involvement in neoplastic meningitis. Ocular symptoms were the presenting clinical feature in 34 patients (83%) out of 41 included in our review, the unique manifestation of meningeal carcinomatosis in 3 patients (7%). Visual loss was the presenting clinical manifestation in 17 patients (50%) and was the most common ocular symptom (70%). Other ocular signs were diplopia, ptosis, papilledema, anisocoria, exophthalmos, orbital pain, scotomas, hemianopsia, and nystagmus. Associated clinical symptoms were headache, altered consciousness, meningism, limb weakness, ataxia, dizziness, seizures, and other cranial nerves involvement. All patients except five underwent CSF examination which was normal in 1 patient, pleocytosis was found in 11 patients, increased protein levels were observed in 16 patients, and decreased glucose levels were found in 8 patients. Cytology was positive in 29 patients (76%). Conclusion. Meningeal carcinomatosis should be considered in patients with ocular symptoms even in the absence of other suggestive clinical symptoms.

Basilico, Paola; Trezzi, Ilaria; Borellini, Linda; Franco, Giulia; Civelli, Vittorio; Bresolin, Nereo; Baron, Pierluigi

2013-01-01

229

Pretibial myxedema as the initial manifestation of Graves' disease.  

PubMed

Pretibial myxedema (PM) is a localized thickening of the pretibial skin due to accumulation of acid mucopolysacharides (glycosaminoglycans). Its pathogenesis is still under investigation. Pretibial myxedema, exophthalmus and thyroid acropachy are the dassic extrathyroidal manifestations of Graves' disease. Almost invariably, PM follows the onset of ophthalmopathy, developing after the diagnosis and treatment of hyperthyroidism. Pretibial myxedema preceding Graves' ophthalmopathy is rare. We report the case of a 28-year-old Greek woman, who presented with multiple, asymptomatic nodules and plaques of the lower legs in the absence of other physical findings. Histopathologic examination revealed deposition of mucopolysacharides in the lower dermis. Laboratory investigation showed elevated serum T3 and T4 and depressed TSH levels. In our patient, pretibial myxedema was the earliest manifestation, leading to the diagnosis of Graves' disease. PMID:12224697

Georgala, S; Katoulis, A C; Georgala, C; Katoulis, E C; Hatziolou, E; Stavrianeas, N G

2002-07-01

230

[Incontinentia pigmenti. Four patients with different clinical manifestations].  

PubMed

Incontinentia pigmenti (IP) is a rare neurocutaneous disorder with a frequency of 1 in 50,000 newborn, and is associated with mutations in IKBKG gene (NEMO) in Xq28, inherited as an X-linked dominant trait. Clinical manifestations detected since the newborn period are highly variable, with 3 well established sequential or overlapped states and each with a characteristic differential diagnosis. With PCR+RFLPs, we analyzed the IKBKG gene in 4 patients with different clinical manifestations and characteristic skin biopsy. In all 4 patients the same deletion of exons 4 to 10 was identified. In female patients in whom the dermatological lesions lead to the suspicion of an IP diagnosis, it is important to have the complete, multidisciplinary and molecular analysis of their first level female relatives. This should give us a clear diagnosis, which is the first step to complete genetic counselling. PMID:22036144

Llano-Rivas, I; Soler-Sánchez, T; Málaga-Diéguez, I; Fernández-Toral, J

2012-03-01

231

[Spectrum of renal manifestations in sickle cell disease].  

PubMed

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management. PMID:24113202

Cazenave, Maud; Koehl, Bérengère; Nochy, Dominique; Tharaux, Pierre-Louis; Audard, Vincent

2014-02-01

232

Anaphylaxis as a clinical manifestation of clonal mast cell disorders.  

PubMed

Clonal mast cell disorders comprise a heterogeneous group of disorders characterized by the presence of gain of function KIT mutations and a constitutively altered activation-associated mast cell immunophenotype frequently associated with clinical manifestations related to the release of mast cells mediators. These disorders do not always fulfil the World Health Organization (WHO)-proposed criteria for mastocytosis, particularly when low-sensitive diagnostic approaches are performed. Anaphylaxis is a frequent presentation of clonal mast cell disorders, particularly in mastocytosis patients without typical skin lesions. The presence of cardiovascular symptoms, e.g., hypotension, occurring after a hymenoptera sting or spontaneously in the absence of cutaneous manifestations such as urticaria is characteristic and differs from the presentation of anaphylaxis in the general population without mastocytosis. PMID:24947681

Matito, A; Alvarez-Twose, I; Morgado, J M; Sánchez-Muñoz, L; Orfao, A; Escribano, L

2014-08-01

233

Hypomelanosis of Ito: a manifestation of mosaicism or chimerism.  

PubMed Central

We describe three patients with the cutaneous manifestations of hypomelanosis of Ito. Two, with unusual abnormalities of their toes, had a mixture of diploid and triploid cells in cultured skin fibroblasts. The published clinical descriptions of hypomelanosis of Ito and diploid-triploid mosaicism are reviewed. Chromosome heteromorphisms, HLA types, and DNA fingerprints were studied in an attempt to elucidate the origin of the disease in our patients. We conclude that hypomelanosis of Ito is a manifestation of a heterogeneous group of disorders, the common factor being the presence of two genetically different cell lines. It can result from chromosomal mosaicism or chimerism, from a postzygotic mutation, or from X inactivation. The risk of recurrence is negligible if the proband is a male; if the proband is female the risk is also low but an X linked mutation must be considered. Images

Donnai, D; Read, A P; McKeown, C; Andrews, T

1988-01-01

234

Oral manifestations associated with systemic complications of prune belly syndrome.  

PubMed

Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by congenital abnormalities including abdominal wall flaccidity, urinary tract alterations, and bilateral cryptorchidism. The incidence of the syndrome is between 1/35000 and 1/50000 live births and there is little information about the oral findings. The present case describes the oral manifestations of a 15-year-old boy diagnosed with PBS. The findings include enamel hypoplasia associated with generalized hypocalcemic dental lines. In the radiographic exam, pronounced demineralization of the trabecular bone of the jaws, loss of lamina dura in all the teeth, and discontinuity of the mandibular cortical bone were observed. Prune belly syndrome is a rare disease, whose clinical dental aspects are not pathognomonic of the syndrome. The comprehension of the systemic mechanism of PBS and its comorbidities enable an understanding of the systemic etiologic factors associated with oral manifestations. PMID:22901648

Pessoa, Larissa; Galvão, Virgilio

2013-01-01

235

Oral manifestation in inflammatory bowel disease: A review  

PubMed Central

Inflammatory bowel diseases (IBDs), including Crohn’s disease (CD) and ulcerative colitis, not only affect the intestinal tract but also have an extraintestinal involvement within the oral cavity. These oral manifestations may assist in the diagnosis and the monitoring of disease activity, whilst ignoring them may lead to an inaccurate diagnosis and useless and expensive workups. Indurated tag-like lesions, cobblestoning, and mucogingivitis are the most common specific oral findings encountered in CD cases. Aphthous stomatitis and pyostomatitis vegetans are among non-specific oral manifestations of IBD. In differential diagnosis, side effects of drugs, infections, nutritional deficiencies, and other inflammatory conditions should also be considered. Treatment usually involves managing the underlying intestinal disease. In severe cases with local symptoms, topical and/or systemic steroids and immunosuppressive drugs might be used.

Lankarani, Kamran B; Sivandzadeh, Gholam Reza; Hassanpour, Shima

2013-01-01

236

Oral manifestation in inflammatory bowel disease: a review.  

PubMed

Inflammatory bowel diseases (IBDs), including Crohn's disease (CD) and ulcerative colitis, not only affect the intestinal tract but also have an extraintestinal involvement within the oral cavity. These oral manifestations may assist in the diagnosis and the monitoring of disease activity, whilst ignoring them may lead to an inaccurate diagnosis and useless and expensive workups. Indurated tag-like lesions, cobblestoning, and mucogingivitis are the most common specific oral findings encountered in CD cases. Aphthous stomatitis and pyostomatitis vegetans are among non-specific oral manifestations of IBD. In differential diagnosis, side effects of drugs, infections, nutritional deficiencies, and other inflammatory conditions should also be considered. Treatment usually involves managing the underlying intestinal disease. In severe cases with local symptoms, topical and/or systemic steroids and immunosuppressive drugs might be used. PMID:24379574

Lankarani, Kamran B; Sivandzadeh, Gholam Reza; Hassanpour, Shima

2013-12-14

237

[Cytomegalovirus. Pathological-anatomical manifestations and detection methods].  

PubMed

Human cytomegalovirus, a double-stranded DNA virus, is a member of the Herpesviridae family with high rates of transmission. Primary infection is often asymptomatic and leads to life-long latency. Reactivation may induce different organ manifestations, particularly in the setting of immunosuppression. Histopathologically, the virus can be detected by light microscopy. Different cell populations in different organs are transformed into"owl's eye" cells, which are pathognomonic. Immunohistochemistry and electron microscopy can be applied as complementary methods. Various PCR approaches in molecular pathology including nested PCR, capture probe ELISA-PCR and real time PCR confer HCMV tests high sensitivity and specificity. The present article discusses the methods of pathological diagnostic approaches and describes organ manifestations of HCMV. PMID:21792604

Drebber, U; Hardt, A; Dienes, H-P; Odenthal, M

2011-09-01

238

Hashimoto's Thyroiditis with Clinical Manifestations Resembling Dermatomyositis: A Case Report.  

PubMed

We report on a 59-year-old man with a 1-year history of forearm erythema, bilateral limb arthralgia, and muscle weakness. During the initial examination we observed infiltrative erythema of the forearm and muscle weakness and atrophy of the limbs. Blood tests revealed marked increases in myogenic enzymes. Because histopathological studies showed lymphocytic infiltration around the small blood vessels in the dermis and mucin deposition, we made a tentative diagnosis of dermatomyositis. However, the specific cutaneous manifestations of dermatomyositis, including heliotrope erythema and Gottron's sign, were absent, and the findings of electromyography were normal. A subsequent detailed examination revealed hypothyroidism and high titers of antithyroglobulin and antimicrosome antibodies, and we made a definitive diagnosis of Hashimoto's thyroiditis. The thyroid function and skin manifestations both improved after treatment with levothyroxine sodium. Dermatomyositis and Hashimoto's thyroiditis can exhibit similar characteristics, and caution is required because of the possibility of misdiagnosis. PMID:24805098

Matayoshi, Takemitsu; Omi, Tokuya; Mayumi, Nobuko; Kawana, Seiji

2014-01-01

239

Ocular manifestations of crush head injury in children  

Microsoft Academic Search

AimsTo review the ocular manifestations of crush head injuries in children.MethodsRetrospective clinical and pathological reviews. Group 1: A total of 16 children admitted with crush head injuries from television tip over. Group 2: Nine autopsy findings in crush head injury.ResultsGroup 1: A total of 11 children had fundus examination: three by neurosurgeons, eight by ophthalmologists. Scattered posterior pole preretinal and

L Gnanaraj; M G F Gilliland; R R Yahya; J T Rutka; J Drake; P Dirks; A V Levin

2007-01-01

240

Premature centromere division: A possible manifestation of chromosome instability  

SciTech Connect

Retrospective analysis of {open_quotes}routine{close_quotes} chromosome preparations from 2 patients with Fanconi anemia and 2 others with ataxia-teleangiectasia showed increased chromosome breakage and a tendency to premature centromere division (PCD) with special reference to early separation of the large acrocentric (13-15) chromosomes. The findings suggest that PCD may be a manifestation of chromosome instability related to potential malignancy. 28 refs., 1 fig., 1 tab.

Mehes, K. [Univ. Medical School, Pecs (Hungary); Buehler, E.M. [Univ. of Children`s Hospital, Basle (Switzerland)

1995-03-13

241

EntomoModel: Understanding and Avoiding Performance Anomaly Manifestations  

Microsoft Academic Search

Subtle implementation errors or mis-configurations in complex Internet services may lead to performance degrada- tions without causing failures. These undiscovered performance anomalies afflict many of today's systems, causing violations of service-level agreements (SLAs), unnecessary resource over- provisioning, or both. In this paper, we re-inserted realistic anomaly causes into a multi-tier Internet service architecture and studied their manifestations. We observed that

Christopher Stewart; Kai Shen; Arun Iyengar; Jian Yin

2010-01-01

242

[Rapidly progressive compromise of cranial pairs as neurosyphilis manifestation].  

PubMed

Syphilis remains a common disease throughout the world, being neurosyphilis a relatively common manifestation. A case of a 34 years old male with HIV and neurosyphilis is presented, characterized by a clinical course evidenced by progressive palsy of cranial nerves. This case is unusual and a rare presentation of progressive cranial involvement with swallowing deficit, have found no similar data in the literature. PMID:23156895

Baccaro, Fernando; Moldes, Sofía; Novelli Poisson, Paola; Arduin, Julieta; Valerga, Mario

2012-01-01

243

Syphilitic hepatitis: an uncommon manifestation of a common disease.  

PubMed

Hepatitis being first manifestation of secondary syphilis is rare. Here in we report a case of 39 years old male who was being treated for hepatitis and presented to us subsequently with itchy maculopapular rash. Venereal disease research laboratory (VDRL) titre was 1:16. Treponema pallidum hemagglutination assay (TPHA) was positive. He was treated with intramuscular Benzathine Penicillin. His hepatitis improved rapidly. PMID:24700957

Baveja, Sukriti; Garg, Shilpa; Rajdeo, Amol

2014-03-01

244

Atypical Clinical Manifestations of Graves' Disease: An Analysis in Depth  

PubMed Central

Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual) manifestations of Graves' disease (GD), that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD.

Hegazi, Mohamed Osama; Ahmed, Sherif

2012-01-01

245

Multi-slice computed tomography manifestations of hepatic epithelioid angiomyolipoma  

PubMed Central

AIM: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate. METHODS: The multi-slice CT manifestations in five patients who were diagnosed with HEA definitely by postoperative pathological examination were analysed retrospectively. Three female patients and two male patients were included. Before operation, four patients received plain CT scanning and dynamic enhancement scanning, and the other patient only received enhancement scanning, with immunohistochemical analysis conducted after postoperative pathological examination. Four patients were misdiagnosed by CT, including three patients misdiagnosed with hepatic cell carcinoma and one patient with focal nodular hyperplasia. RESULTS: Upper abdominal multi-slice spiral CT scanning and three-stage enhancement scanning were conducted in five patients with HEA before operation. HEA had certain characteristic CT manifestations: low density masses, a few relatively high-density masses or fat-density masses diffusely shown in foci, clear boundary, round or oval and large focus, and tumour size ranging from 3.1 cm × 2.5 cm to 7.0 cm × 5.2 cm. During enhancement scanning, the foci were significantly enhanced uniformly or non-uniformly during the arterial phase, while during the venous and equilibrium phases, the foci were enhanced continuously or showed obvious low-density masses. Obviously enhanced and widened vessels could be found adjacent to foci or in the central area of foci during the arterial phase. CONCLUSION: CT manifestations of HEA have certain characteristics. Primary diagnosis can be obtained by combining CT findings with clinical data, but pathological examination is still needed for a definite diagnosis.

Dai, Chun-Lai; Xue, Li-Ping; Li, Yu-Mei

2014-01-01

246

Transient Spinal Cord Ischemia as Presenting Manifestation of Polycythemia Vera  

PubMed Central

Spinal arterial vascularization is supplied by a large anastomotic net, making spinal ischemic events far less common than ischemic cerebral strokes. Polycythemia vera, due to blood hyperviscosity and activated platelet aggregation, is associated with a higher risk of arterial and venous thrombotic events. We report a patient with spinal cord transient ischemic attacks, a rarely presenting manifestation, and polycythemia vera, which highlights the thrombotic potential of this disease, and the requirement of exhaustive diagnostic workout of a spinal ischemic event.

Costa, Sonia; Marques, Joana; Barradas, Anabela; Valverde, Ana

2011-01-01

247

[Apropos of 1 case of ophthalmologic manifestation of bilharziasis].  

PubMed

We present an ocular manifestation of schistosomiasis. We observed the case of a 10 year old young girl living near a flooding canal of the Niger river. She presented a tumor of the left lid associated with an important ptosis. This tumor resulted from numerous polyps of the conjunctiva. The histological analysis of a biopsy of the conjunctiva made the diagnostic. It was confirmed by the analysis of urine and by the important amelioration of inflammation resulting from a specific therapy. PMID:8219789

Kabo, A M; Warter, A

1993-01-01

248

Conditions in subjects with rheumatic diseases: pulmonary manifestations of vasculitides  

Microsoft Academic Search

Pulmonary involvement is a common complication of vasculitides, especially small vessel vasculitides. This review provides\\u000a an overview of vasculitic manifestations of the lung as well as of other organs involved in vasculitides. Furthermore, it\\u000a provides the diagnostic procedures required to asses a patient with vasculitic lung involvement and gives an overview of current\\u000a treatment strategies.

Julia U Holle; Frank Moosig; Klaus Dalhoff; Wolfgang L Gross

2011-01-01

249

Papilledema as a manifestation of a spinal subdural abscess  

Microsoft Academic Search

Papilledema is an uncommon presentation of spinal cord processes. Spinal subdural abscess (SSA) is a rare site of post-operative infection. We report a patient who developed papilledema as the primary manifestation of a post-operative lumbar subdural abscess. A spinal abscess should be considered in the post-operative spinal surgery patient who develops papilledema in the setting of persistent back pain. The

Melissa W. Ko; Benjamin Osborne; Sungmi Jung; Dina A. Jacobs; Paul Marcotte; Steven L. Galetta

2007-01-01

250

Hepatosplenic candidiasis. A manifestation of chronic disseminated candidiasis.  

PubMed

Much progress has been made over the last decade in diagnosing and treating CDC, a chronic and debilitating infection that interferes with the delivery of intensive cytotoxic chemotherapy in patients with leukemia. The use of fluconazole prophylaxis in these patients has decreased the incidence of CDC dramatically. The greatest future challenges are gaining a better understanding of its pathophysiology, and the continued development of effective diagnostic and therapeutic strategies to treat this unusual manifestation of systemic candidiasis. PMID:10987117

Kontoyiannis, D P; Luna, M A; Samuels, B I; Bodey, G P

2000-09-01

251

Panhypopituitarism as first manifestation of a lung cancer  

Microsoft Academic Search

  Pituitary metastases of solid tumours are infrequent, specially as a first manifestation. When they happen, they are usually\\u000a due to breast or lung cancer and are asymptomatic or produce diabetes insipidus. It is very strange that they produce hormonal\\u000a deficiency.\\u000a We present a case report of a bronchogenic adenocarcinoma in a 65-year-old man which began with panhypopituitarism, diabetes\\u000a insipidus and

Carmen Guillén Ponce; María Pilar Garrido López; María José Molina Garrido; Gema Muñoz Molina; Alfredo Carrato

2007-01-01

252

Congenital Stridor: Unusual Manifestation of Coarctation of the Aorta  

Microsoft Academic Search

Coarctation of the aorta is a relatively common congenital condition. Most infantile presentations of aortic coarctation are\\u000a related to the associated congenital cardiac abnormalities leading to congestive heart failure or systemic hypoperfusion.\\u000a We describe a 4-month-old infant who presented with stridor as the sole manifestation of coarctation. Radiologic studies revealed\\u000a enlarged innominate artery due to the aortic coarctation that resulted

J. G. Park; M. E. Wylam

2006-01-01

253

Chronic disorders with episodic manifestations: focus on epilepsy and migraine  

PubMed Central

Epilepsy and migraine are chronic neurological disorders with episodic manifestations that are commonly treated in neurological practice and frequently occur together. In this review we examine similarities and contrasts between these disorders, with focus on epidemiology and classification, temporal coincidence, triggers, and mechanistically based therapeutic overlap. This investigation draws attention to unique aspects of both epilepsy and migraine, while identifying areas of crossover in which each specialty could benefit from the experience of the other.

2006-01-01

254

Ocular manifestations of the Johanson-Blizzard syndrome.  

PubMed

Johanson-Blizzard syndrome is a rare autosomal-recessive congenital disorder characterized by hypoplastic nasal alae, midline scalp defects, deafness, microcephaly, hypothyroidism, absent permanent teeth, malabsorption, and failure to thrive. The literature was reviewed to define the reported spectrum of ocular manifestations, which are not well documented. We found that nasolacrimal system malformations are a common feature of Johanson-Blizzard, whereas intraocular malformations are rare. This report describes the ophthalmologic findings and management of 2 affected children. PMID:19717322

Cheung, Jason C; Thomson, Hugh; Buncic, J Raymond; Héon, Elise; Levin, Alex V

2009-10-01

255

The Gastrointestinal Manifestations of Telomere-Mediated Disease  

PubMed Central

Summary Defects in telomere maintenance genes cause pathological telomere shortening, and manifest in syndromes which have prominent phenotypes in tissues of high turnover: the skin and bone marrow. Because the gastrointestinal (GI) epithelium has rapid turnover, we sought to determine whether telomere syndromes cause GI disease, and to define its prevalence, spectrum and natural history. We queried subjects in the Johns Hopkins Telomere Syndrome Registry for evidence of luminal GI disease. In sixteen percent of Registry subjects (6 of 38), there was a history of significant GI pathology, and 43 additional cases were identified in the literature. Esophageal stenosis, enteropathy and enterocolitis were the recurrent findings. In the intestinal mucosa, there was striking villous atrophy, extensive apoptosis, and anaphase bridging pointing to regenerative defects in the epithelial compartment. GI disease was often the first and most severe manifestation of telomere disease in young children. These findings indicate that telomere dysfunction disrupts the epithelial integrity in the human GI tract manifesting in recognizable disease processes. A high index of suspicion should facilitate diagnosis and management.

Jonassaint, Naudia L.; Guo, Nini; Califano, Joseph A.; Montgomery, Elizabeth A.; Armanios, Mary

2013-01-01

256

Endocrine manifestations and management of Prader-Willi syndrome  

PubMed Central

Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13. In infancy it is characterized by hypotonia with poor suck resulting in failure to thrive. As the child ages, other manifestations such as developmental delay, cognitive disability, and behavior problems become evident. Hypothalamic dysfunction has been implicated in many manifestations of this syndrome including hyperphagia, temperature instability, high pain threshold, sleep disordered breathing, and multiple endocrine abnormalities. These include growth hormone deficiency, central adrenal insufficiency, hypogonadism, hypothyroidism, and complications of obesity such as type 2 diabetes mellitus. This review summarizes the recent literature investigating optimal screening and treatment of endocrine abnormalities associated with PWS, and provides an update on nutrition and food-related behavioral intervention. The standard of care regarding growth hormone therapy and surveillance for potential side effects, the potential for central adrenal insufficiency, evaluation for and treatment of hypogonadism in males and females, and the prevalence and screening recommendations for hypothyroidism and diabetes are covered in detail. PWS is a genetic syndrome in which early diagnosis and careful attention to detail regarding all the potential endocrine and behavioral manifestations can lead to a significant improvement in health and developmental outcomes. Thus, the important role of the provider caring for the child with PWS cannot be overstated.

2013-01-01

257

[Vectorcardiographic manifestations of left ventricular and biventricular enlargement].  

PubMed

The basic criteria for the vectorcardiographic diagnosis of left ventricular and biventricular enlargements are discussed on the basis of the myocardial activation sequence. Left ventricular dilatation, secondary to isolated diastolic overloading, increases the manifestation of all the vectors resulting of the activation of this ventricle. These changes reflect the proximity of the left ventricular walls to the exploring electrodes. The vectors above mentioned project themselves as wide ventricular curves with counterclockwise rotation on the three planes. The T loop, of secondary type, is concordant in its orientation with the R loop. Cases with left ventricular hypertrophy, produced by a sustained systolic overloading, are also described. In the presence of global left ventricular hypertrophy without LBBB, the manifestation of all the vectors resulting from the depolarization of this ventricle (I, IIl, IIIl), is increased. This is due to a prolonged duration of the corresponding activation fronts. These vectors are projected on the different segments of the ventricular curves and they show a counterclockwise rotation on the three planes. When LBBB is also present, the first septal vector is not evident. The T loop, of secondary type, opposes the R loop on the frontal and horizontal planes. The presence of left ventricular hypertrophy of the segmentary type, generally increases the manifestation of the vector I, and sometimes, also that of the vector IIIl. When both ventricles are hypertrophied, the electromotive forces of the chamber more severely affected predominate in the vectorcardiographic records. PMID:162478

de Micheli, A; Medrano, G A

1979-01-01

258

Cutaneous manifestations in renal failure patients: a case series.  

PubMed

Cutaneous involvement in renal disease is due to a host of factors ranging from metabolic disturbances to immunosuppressive drugs. Herein we report a series of six cases of renal failure with varied cutaneous manifestations ranging from infections to neoplasms due to prolonged immunosuppression. Our first case had cutaneous cryptococcosis where skin lesions gave a clue to the diagnosis of altered sensorium and underlying meningitis. The second case initially presented with florid warts and was treated successfully but later presented with an explosive recurrence of skin lesions due to malignant transformation. Our third case had basal cell carcinoma over the presternal region that was successfully treated with liquid nitrogen cryotherapy. Our fourth case had diabetic nephropathy that presented with septicemia and purpura fulminans. The last case had cutaneous manifestations of drug therapy because of heparin infusion. To conclude, cutaneous manifestations in patients with renal failure are varied and a high degree of suspicion is needed for early diagnosis and aggressive treatment to effectively combat mortality and morbidity. PMID:17456917

Banerjee, S

2007-01-01

259

Pulmonary manifestations of Birt-Hogg-Dubé syndrome.  

PubMed

Birt-Hogg-Dubé syndrome (BHD) is a rare, autosomal dominant disorder characterized by the development of hair follicle tumors, renal tumors and pulmonary cysts. BHD is caused by heterozygous, predominantly truncating mutations in the folliculin (FLCN) gene located on chromosome 17, which encodes a highly conserved tumor suppressor protein. Although management of renal tumors of low malignant potential is the primary focus of longitudinal care, pulmonary manifestations including cyst formation and spontaneous pneumothorax are among the most common manifestations in BHD. Due to the lack of awareness, there is commonly a delay in the pulmonary diagnosis of BHD and patients are frequently mislabeled as having chronic obstructive lung disease, emphysema or common bullae/blebs. A family history of pneumothorax is present in 35 % of patients with BHD. Certain imaging characteristics of the cysts, including size, basilar and peripheral predominance, perivascular and periseptal localization, and elliptical or lentiform shape can suggest the diagnosis of BHD based on inspection of the chest CT scan alone. Recurrent pneumothoraces are common and early pleurodesis is recommended. A better understanding of role of FLCN in pulmonary cyst formation and long term studies to define the natural history of the pulmonary manifestations of BHD are needed. PMID:23715758

Gupta, Nishant; Seyama, Kuniaki; McCormack, Francis X

2013-09-01

260

Endocrine manifestations and management of Prader-Willi syndrome.  

PubMed

Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13. In infancy it is characterized by hypotonia with poor suck resulting in failure to thrive. As the child ages, other manifestations such as developmental delay, cognitive disability, and behavior problems become evident. Hypothalamic dysfunction has been implicated in many manifestations of this syndrome including hyperphagia, temperature instability, high pain threshold, sleep disordered breathing, and multiple endocrine abnormalities. These include growth hormone deficiency, central adrenal insufficiency, hypogonadism, hypothyroidism, and complications of obesity such as type 2 diabetes mellitus. This review summarizes the recent literature investigating optimal screening and treatment of endocrine abnormalities associated with PWS, and provides an update on nutrition and food-related behavioral intervention. The standard of care regarding growth hormone therapy and surveillance for potential side effects, the potential for central adrenal insufficiency, evaluation for and treatment of hypogonadism in males and females, and the prevalence and screening recommendations for hypothyroidism and diabetes are covered in detail. PWS is a genetic syndrome in which early diagnosis and careful attention to detail regarding all the potential endocrine and behavioral manifestations can lead to a significant improvement in health and developmental outcomes. Thus, the important role of the provider caring for the child with PWS cannot be overstated. PMID:23962041

Emerick, Jill E; Vogt, Karen S

2013-01-01

261

Orthopedic manifestations and implications for individuals with Costello syndrome.  

PubMed

Costello syndrome is a rare genetic condition caused by heterozygous alterations in HRAS and characterized by multi-system abnormalities. Individuals with Costello syndrome usually present with severe feeding difficulties in infancy, short stature, coarse facial features, increased tumor risks, cardiac and neurological complications, intellectual disability and orthopedic complications. This study further defines the orthopedic manifestations affecting individuals with Costello syndrome. We studied 43 participants and performed medical records review, clinical examinations and orthopedic inquiry forms. In 23 participants, hip and or spinal imaging assessments were completed. Serial radiographs were analyzed when available. A total of 25 orthopedic manifestations were identified. Ten manifestations were seen in the majority of the participants: hypotonia (87%), ligamentous laxity (85%), scoliosis (63%), kyphosis (58%), characteristic hand deformities (85%), ulnar deviation of the wrist (63%), elbow (55%) and shoulder contractures (65%), tight Achilles tendon (73%), and pes planus (53%). Other characteristics of special note were hip dysplasia (45%), foot deformities requiring surgical intervention (38%) and osteopenia/osteoporosis (47%). We also studied the development of the hips and spine. Uni- or bilateral hip dysplasia was congenital in some, while it developed throughout childhood in others. Spinal involvement included scoliosis, kyphosis, lordosis, and curvature reversal (thoracic lordosis and lumbar kyphosis). Based on these findings, we recommend routine referral to an orthopedic surgeon as well as instituting screening protocols for hips and spine for individuals with Costello syndrome. PMID:23813656

Detweiler, Stacey; Thacker, Mihir M; Hopkins, Elizabeth; Conway, Laura; Gripp, Karen W

2013-08-01

262

75 FR 57283 - Agency Information Collection Activities: Passenger and Crew Manifest  

Federal Register 2010, 2011, 2012, 2013

...Passenger and Crew Manifest (Advance Passenger Information System...appropriate automated, electronic, mechanical, or other technological techniques...Passenger and Crew Manifest (Advance Passenger Information System-APIS...Number: None. Abstract: The Advance Passenger Information...

2010-09-20

263

19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.  

Code of Federal Regulations, 2010 CFR

...failure to manifest narcotic drugs or marihuana. 162.65 Section 162.65 ...Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a) Cargo or baggage...

2009-04-01

264

19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.  

Code of Federal Regulations, 2010 CFR

...failure to manifest narcotic drugs or marihuana. 162.65 Section 162.65 ...Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a) Cargo or baggage...

2010-04-01

265

19 CFR 122.113 - Form for transit air cargo manifest procedures.  

Code of Federal Regulations, 2011 CFR

...2014-04-01 2014-04-01 false Form for transit air cargo manifest procedures. 122.113 Section...OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures §...

2014-04-01

266

19 CFR 122.162 - Failure to notify and explain differences in air cargo manifest.  

Code of Federal Regulations, 2011 CFR

...Failure to notify and explain differences in air cargo manifest. 122.162 Section 122...HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Penalties § 122...Failure to notify and explain differences in air cargo manifest. (a)...

2014-04-01

267

19 CFR 162.65 - Penalties for failure to manifest narcotic drugs or marihuana.  

Code of Federal Regulations, 2013 CFR

...failure to manifest narcotic drugs or marihuana. 162.65 Section 162.65 ...Controlled Substances, Narcotics, and Marihuana § 162.65 Penalties for failure to manifest narcotic drugs or marihuana. (a) Cargo or baggage...

2013-04-01

268

19 CFR 4.7a - Inward manifest; information required; alternative forms.  

Code of Federal Regulations, 2013 CFR

...pallets are not acceptable manifested quantities; for example, a container containing 10 pallets with 200 cartons should be manifested as 200 cartons); (vi) The first foreign port where the carrier takes possession of the cargo destined to...

2013-04-01

269

19 CFR 123.5 - Certification and filing of inward foreign manifest.  

Code of Federal Regulations, 2013 CFR

... U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.5 Certification and filing of inward foreign manifest. The manifest...

2013-04-01

270

Glycemic Control and Radiographic Manifestations of Tuberculosis in Diabetic Patients  

PubMed Central

Background Radiographic manifestations of pulmonary tuberculosis (TB) in patients with diabetes mellitus (DM) have previously been reported, with inconsistent results. We conducted a study to investigate whether glycemic control has an impact on radiographic manifestations of pulmonary TB. Methods Consecutive patients with culture-positive pulmonary TB who had DM in three tertiary care hospitals from 2005–2010 were selected for review and compared with a similar number without DM. Glycemic control was assessed by glycated haemoglobin A1C (HbA1C). A pre-treatment chest radiograph was read independently by two qualified pulmonologists blinded to patients’ diabetic status. Films with any discordant reading were read by a third reader. Results 1209 culture positive pulmonary TB patients (581 with DM and 628 without DM) were enrolled. Compared with those without DM, TB patients with DM were significantly more likely to have opacity over lower lung fields, extensive parenchymal lesions, any cavity, multiple cavities and large cavities (>3 cm). The relative risk of lower lung field opacities was 0.80 (95% CI 0.46–1.42) for those with DM with A1C<7%, 2.32 (95% CI 1.36 - 3.98) for A1C 7%–9%, and 1.62 (95% CI 1.12–2.36) for A1C>9%; and that of any cavity over no cavity was 0.87 (95% CI 0.46–1.62) for patients with DM with A1C<7%, 1.84 (95% CI 1.20–2.84) for A1C 7%–9%, and 3.71 (95% CI 2.64–5.22) for A1C>9%, relative to patients without DM. Conclusions Glycemic control significantly influenced radiographic manifestations of pulmonary TB in patients with DM.

Chiang, Chen-Yuan; Lee, Jen-Jyh; Chien, Shun-Tien; Enarson, Donald A.; Chang, You-Cheng; Chen, Yi-Ting; Hu, Ting-Yu; Lin, Chih-Bin; Suk, Chi-Won; Tao, Jui-Ming; Bai, Kuan-Jen

2014-01-01

271

Cutaneous manifestations of serum sickness in patients receiving antithymocyte globulin.  

PubMed

We have prospectively evaluated the cutaneous manifestations of serum sickness in thirty-five patients treated with horse antithymocyte globulin for bone marrow failure. Twenty-one patients (21/35) were treated with antithymocyte globulin (15 mg/kg/day) for 10 days, and fourteen of thirty-five patients were treated with antithymocyte globulin (15 mg/kg/day) for 14 days and then every other day for an additional 14 days. Clinical evidence of serum sickness developed in thirty patients and included fever and malaise (100%), cutaneous eruptions (93%), arthralgias and myalgias (67%), gastrointestinal complaints (67%), and lymphadenopathy (13%). Cutaneous findings consisted of morbilliform eruptions (n = 19), urticaria (n = 1), or a combination of these two reaction patterns (n = 8). Cutaneous manifestations of serum sickness began on day 7 +/- 1 and lasted for 12 +/- 2 days for the group as a whole. Biopsies of lesional skin revealed mild perivascular lymphohistiocytic infiltrates by light microscopy in these leukopenic patients. Direct immunofluorescence microscopy of lesional skin from patients with serum sickness demonstrated immunoreactants in seven of nine subjects (78%). Immunoreactants were confined to the walls of dermal blood vessels and consisted of IgM (7/9), C3 (6/9), IgE (5/9), and IgA (4/9). IgG (horse or human) was not identified in any of these specimens. Twenty-one patients (21/28) also developed an erythematous eruption on the sides of the fingers, toes, palms, and soles 12 to 48 hours prior to their morbilliform eruption. This study describes the cutaneous manifestations of human serum sickness occurring during therapy with horse antithymocyte globulin, documents a cutaneous sign of serum sickness, and suggests that the cutaneous eruptions associated with human serum sickness are immunologically mediated. PMID:3877081

Bielory, L; Yancey, K B; Young, N S; Frank, M M; Lawley, T J

1985-09-01

272

Irreversible papillitis and ophthalmoparesis as a presenting manifestation of neurobrucellosis.  

PubMed

A 35-year-old man presented with a meningeal syndrome and acute onset of visual blurring. Clinical investigations revealed bacterial meningitis with bilateral papillitis and ophthalmoparesis. Serum and cerebrospinal fluid serology confirmed the diagnosis of chronic active neurobrucellosis. Following therapy there was no improvement and he developed optic atrophy. Extensive literature review revealed, one case of bilateral irreversible papillitis resulting from neurobrucellosis. However no cases of neurobrucellosis have been reported with meningitis, irreversible papillitis and ophthalmoparesis. This case demonstrates that in endemic areas, acute meningitis is a potential manifestation of neurobrucellosis and that bilateral irreversible papillitis with ophthalmoparesis can be a potential serious complication. PMID:17320277

Miyares, Francisco Ruiz; Deleu, Dirk; ElShafie, Sittana S; Equia, Frank; Mesraoua, Boulenouar; Al Hail, Hassan; Salim, Khalid

2007-06-01

273

Unusual manifestations of Yersinia enterocolitica infections diagnosed using novel methods.  

PubMed

We report the cases of two patients who had infections due to Yersinia enterocolitica. The first patient exhibited chronic recurrent fever, hepatic and splenic granulomas, and bone marrow abnormalities, and the second patient presented with enterocolitis with leukocytoclastic vasculitis of the skin. Cultures and agglutination titers were negative. Indirect immunofluorescence techniques with use of serotype-specific antisera and antisera to Yersinia outer-membrane proteins (Yops) were applied to biopsy specimens, and immunoblotting techniques for determining class-specific circulating antibodies to Yops were used for demonstrating these unusual manifestations of Y. enterocolitica infections. PMID:1420678

Tak, P P; Visser, L G; Hoogkamp-Korstanje, J A; Kluin-Nelemans, J C; Hogendoorn, P C; Kluin, P M; Barza, M; de Koning, J; van Furth, R

1992-10-01

274

Coherence manifestation in a continuous-wave laser  

NASA Astrophysics Data System (ADS)

We extend the existing theory of continuous-wave lasers by systematically considering spontaneous emission. In a simple rate-equation approach, the laser eigenvalue, defined as the ratio of coherent photons coupled out of the resonator divided by the number of photons coupled in via spontaneous emission, emerges as the fundamental parameter describing a continuous-wave laser. We derive a general equation for the laser eigenvalue and confirm the point at which coherence manifests itself in the resonator. The theory describes all types of lasers of three-level, four-level, or any intermediate nature.

Eichhorn, M.; Pollnau, M.

2014-05-01

275

Various Manifestations of Hyperthyroidism in an Ambulatory Clinic: Case Studies  

PubMed Central

This study reviews five cases of women with hyperthyroidism, three black women and two Hispanic women. Initially, two patients presented with voice changes, weight loss, and increased appetite. Only two patients presented with classical symptoms of hyperthyroidism. Examination showed all patients had diffusely enlarged thyroids and exaggerated reflexes. Two patients showed Graves' opthalmopathy. These cases document the variety of presentations of hyperthyroidism. Hence, a high index of suspicion must exist for this disease, even in the absence of a number of the classical manifestations of hyperthyroidism. When patients present to primary care centers with a constellation of symptoms, an examination of the thyroid gland is essential.

Tripp, Warren; Rao, Vijaya; Creary, Ludlow B.

1987-01-01

276

Hemophagocytic lymphohistiocytosis and Kawasaki disease: combined manifestation and differential diagnosis.  

PubMed

Both hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD) are diagnosed in patients with prolonged resistant fever by using a scoring system. Concurrent manifestation of both conditions has been reported previously. We describe an infant of 7 weeks whose condition fulfilled the criteria of HLH, but who, after clinical response to treatment, suddenly died from a myocardial infarction at 11 weeks. Post-mortem examination revealed a previously unknown coronary arteritis typical for KD. Since it is difficult to distinguish between KD and HLH, both diseases should be considered in young children with overlapping symptoms. Repeated echocardiograms may be helpful in these cases. PMID:19434728

Titze, Ulf; Janka, Gritta; Schneider, E Marion; Prall, Friedrich; Haffner, Dieter; Classen, Carl Friedrich

2009-09-01

277

Adverse drug reactions: oral and dental manifestations and complications.  

PubMed

Adverse, that is unintended untoward effects of medications, are increasing in incidence and their severity, given the aging of the Australian and New Zealand population and associated drug use. Not only are the number of agents that our patients are using increasing, including the increased use of "alternate" or "complimentary" medications, but also their complexity, with the advent of potent, targeted, biological agents. The result is an increasing number of our patients will be at greater risk of adverse effects from their medications. These adverse effects include the impeding of the safe delivery of dental treatment and the adverse oral and dental manifestations and complications related to the use of medications. PMID:24783838

Schifter, M

2012-04-01

278

Automated Detection of Anomalous Shipping Manifests to Identify Illicit Trade  

SciTech Connect

We describe an approach to analyzing trade data which uses clustering to detect similarities across shipping manifest records, classification to evaluate clustering results and categorize new unseen shipping data records, and visual analytics to provide to support situation awareness in dynamic decision making to monitor and warn against the movement of radiological threat materials through search, analysis and forecasting capabilities. The evaluation of clustering results through classification and systematic inspection of the clusters show the clusters have strong semantic cohesion and offer novel ways to detect transactions related to nuclear smuggling.

Sanfilippo, Antonio P.; Chikkagoudar, Satish

2013-11-12

279

Hydrops fetalis: manifestation in lysosomal storage diseases including Farber disease  

Microsoft Academic Search

The authors describe a case of disseminated lipogranulomatosis (Farber disease) presenting as nonimmune hydrops fetalis. This\\u000a is the tenth lysosomal storage disease which can show this clinical manifestation. The literature is reviewed for all hydrops\\u000a cases associated with lysosomal storage diseases.\\u000a \\u000a \\u000a Conclusion Although rare, the lysosomal storage diseases collectively are significant causes of non-immune hydrops and appropriate investigations\\u000a are required

Evelyn Kattner; A. Schäfer; K. Harzer

1997-01-01

280

Manifest causality in quantum field theory with sources and detectors  

NASA Astrophysics Data System (ADS)

We introduce a way to compute scattering amplitudes in quantum field theory including the effects of particle production and detection. Our amplitudes are manifestly causal, by which we mean that the source and detector are always linked by a connected chain of retarded propagators. We show how these amplitudes can be derived from a path integral, using the Schwinger-Keldysh "in-in" formalism. Focussing on ? 3 theory, we confirm that our approach agrees with the standard S-matrix approach in the case of positive energy plane-wave scattering.

Dickinson, Robert; Forshaw, Jeff; Millington, Peter; Cox, Brian

2014-06-01

281

Brain abscess as a manifestation of spinal dermal sinus  

PubMed Central

Dermal sinuses have been associated with a wide spectrum of clinical manifestations ranging from asymptomatic to drainage of purulent material from the sinus tract, inclusion tumors, meningitis, and spinal abscess. To date, there has been no documented report of brain abscess as a complication of spinal dermal sinus. Here, we report an 8-month-old girl who was presented initially with a brain abscess at early infancy but lumbar dermal sinus and associated spinal abscess were discovered afterwards. The probable mechanisms of this rare association have been discussed.

Emami-Naeini, Parisa; Mahdavi, Ali; Ahmadi, Hamed; Baradaran, Nima; Nejat, Farideh

2008-01-01

282

A Case of Duodenal Duplication Cyst Manifested by Duodenal Polyp  

PubMed Central

Duodenal duplication cyst is a rare anomaly, totaling only 4% to 12% of gastrointestinal duplications, and is usually encountered during infancy or in early childhood. Most are commonly located posterior to the first or second portion of the duodenum. Presenting signs and symptoms include vomiting, decreased oral intake, periumbilical tenderness, abdominal distention, obstructive jaundice, acute pancreatitis, and gastrointestinal bleeding. The traditional treatment of a duodenal duplication cyst has been complete surgical resection, but very few cases of endoscopic treatment have been reported in the literature. Here, we report a case of duodenal duplication cyst that was manifested by a duodenal polyp.

Park, Su Bin; Kim, Jin Hee; Lee, Hyun Jeong; Jang, Seong Pil; Kim, Gwang Ha; Song, Geun Am

2012-01-01

283

Dermatologic manifestations and neuropathic symptoms in women with Fabry disease.  

PubMed

Fabry disease (angiokeratoma corporis diffusum universale) is a rare, progressive, X-linked lysosomal storage disease. Deficiency of the ?-galactosidase A (?-gal A) enzyme leads to accumulation of neutral glycosphingolipids within vascular endothelial lysosomes of various organs, including skin, kidneys, heart, and brain.1. We herein describe the case of a 30-year-old female presenting two classic signs of Fabry disease, angiokeratomas and episodic acroparesthesias, in the absence of other clinical manifestations. An haplotype corresponding to the combination of three different nucleotide polymorphic variants (g. 7192-7198del5+ g. 10115A>G + g. 10956 C>T) at the heterozygous state, was identified.2. PMID:24897976

Melpignano, Andrea; Mandurino-Mirizzi, Alessandro; Besagni, Francesca; Leri, Annarosa

2014-01-01

284

Orthopaedic manifestations and management of spondyloepimetaphyseal dysplasia Strudwick type.  

PubMed

Spondyloepimetaphyseal dysplasia (SEMD) Strudwick type is a rare autosomal dominant condition arising from defects in COL2A1 the genes responsible for the biosynthesis of procollagen type II. The orthopaedic manifestations of patients can be hypoplastic odontoid peg with atlantoaxial instability, severe kyphosis or lordosis of dorsal and lumbar spines, hip subluxation, coxa vara and early severe hip osteoarthritis, and malalignment of lower limbs like genu valgum or club foot. We report a mother and daughter with SEMD Strudwick Type and describe their orthopaedic problems, surgical management and clinical outcome after 30 years and 7 years of follow-up respectively. PMID:16280719

Amirfeyz, Rouin; Taylor, A; Smithson, S F; Gargan, M F

2006-01-01

285

An autopsy case of Fabry's disease with cardiac manifestations.  

PubMed

We report an autopsy case of Fabry's disease with cardiac manifestations. Electron microscopic examination of rectal biopsy specimens revealed lamellar bodies and osmiophilic irregular bodies. Biochemical analysis showed low enzymatic activity of alpha-galactosidase A in plasma fluid. Microscopic examination on autopsy showed marked hypertrophy and vacuolation of cardiac muscle cells. Intracytoplasmic vacuolation was also found in glomerular epithelial or endothelial cells and smooth muscle cells of renal arteries, Meissner's plexus in the submucosa of small and large intestines, and smooth muscle cells of arterioles in the cerebrum. The diagnosis of Fabry's disease was made. The patient died suddenly of cardiomyopathy. PMID:19907117

Suzuki, Osamu; Abe, Masafumi

2009-11-01

286

Diffuse systemic sclerosis with bullous lesions without systemic manifestations*  

PubMed Central

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Macedo, Paula Renaux Wanderley Caratta; Mota, Amanda Nascimento Cavalleiro de Macedo; Gripp, Alexandre Carlos; Alves, Maria de Fatima Guimaraes Scotelaro; Klumb, Evandro Mendes

2013-01-01

287

Primary antiphospholipid syndrome manifesting as partial status epilepticus.  

PubMed

Primary antiphospholipid syndrome (APS) is an autoimmune disease defined by vascular thrombosis, pregnancy complications, and persistent antiphospholipid antibodies. Neurological manifestations include stroke, seizures, and chorea among others. Seizures are often precipitated by an acute ischemic event, but occasionally, structural abnormalities are absent. We present a 61-year-old man who developed partial seizures that progressed into partial status epilepticus. His seizures were intractable and required aggressive treatment with multiple anti-epileptic medications. He was diagnosed with primary APS and treated with anticoagulation. Head imaging did not reveal any acute ischemic events. This case demonstrates that primary APS may present as a refractory status epilepticus unrelated to acute cerebral ischemia. PMID:23545615

Maalouf, Nancy N; Hinduja, Archana; Shihabuddin, Bashir S

2013-04-01

288

Organising pneumonia as the first manifestation of rheumatoid arthritis  

PubMed Central

Organising pneumonia (OP) is an inflammatory lung disease with distinctive clinicopathological features. OP can be evident during the course of rheumatoid arthritis (RA) with increased disease activity. The authors report an OP associated with RA case in which pulmonary symptoms preceded the onset of joint symptoms. An OP patient with elevated serum anticyclic citrullinated peptide antibody is likely to manifest RA in the near future, reflecting its high disease activity. Thus, an early rheumatologic consultation should be taken into consideration to make an early decision to initiate disease-modifying antirheumatic drugs therapy.

Hoshino, Chisho; Satoh, Noriyuki; Narita, Masashi; Kikuchi, Akio; Inoue, Minoru

2011-01-01

289

Possible experimental manifestations of the many-body localization  

NASA Astrophysics Data System (ADS)

Recently, it was predicted that if all one-electron states in a noninteracting disordered system are localized, the interaction between electrons in the absence of coupling to phonons leads to a finite-temperature metal-insulator transition. Here, we show that even in the presence of a weak coupling to phonons the transition manifests itself (i) in the nonlinear conduction, leading to a bistable I-V curve, and (ii) by a dramatic enhancement of the nonequilibrium current noise near the transition.

Basko, D. M.; Aleiner, I. L.; Altshuler, B. L.

2007-08-01

290

Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus.  

PubMed

In the course of Systemic Lupus Erythematosus (SLE), a variety of neuropsychiatric disturbances is reported with a prevalence ranging from 17% to 75%. The diagnosis of these syndromes is difficult and requires a careful psychiatric evaluation. Distinct autoantibodies detectable in serum or cerebrospinal fluid of patients with SLE are associated with the presence of neuropsychiatric disorders. These autoantibodies may have a pathogenic relevance in neuropsychiatric SLE or they may be merely an epiphenomenon. This review describes the various autoantibodies reported to be associated with neuropsychiatric manifestations in SLE and discusses their possible role. PMID:19500858

Colasanti, Tania; Delunardo, Federica; Margutti, Paola; Vacirca, Davide; Piro, Ettore; Siracusano, Alessandra; Ortona, Elena

2009-07-25

291

Neurological manifestations in Baggio-Yoshinari Syndrome (Brazilian Lyme disease-like syndrome)  

Microsoft Academic Search

Introduction: Lyme disease (LD) is a tick-borne disease, caused by Borrelia burgdorferi sensu lato spirochetes, trans- mitted by Ixodes ricinus complex ticks, which leads to multiple systemic clinical manifestations. In Brazil, a different syndrome is described that mimics LD symptoms, but that also manifests high frequencies of recurrent episodes and immune-allergic manifestations. It is transmitted by the Amblyomma cajennense tick

Samuel Katsuyuki Shinjo; Giancarla Gauditano; Paulo Euripedes Marchiori; Virgínia Lúcia Nazário Bonoldi; Izaias Pereira da Costa; Elenice Mantovani; Natalino Hajime Yoshinari

292

75 FR 43997 - Agency Information Collection Activities: Transportation Entry and Manifest of Goods Subject to...  

Federal Register 2010, 2011, 2012, 2013

...paperwork and respondent burden, CBP invites the general...and Manifest of Goods Subject to CBP Inspection and...and Manifest of Goods Subject to CBP Inspection and...with no change to the burden hours. Type of Review...and Manifest of Goods Subject to CBP Inspection...

2010-07-27

293

78 FR 57405 - Agency Information Collection Activities: Transportation Entry and Manifest of Goods Subject to...  

Federal Register 2010, 2011, 2012, 2013

...and Manifest of Goods Subject to CBP Inspection and...paperwork and respondent burden, CBP invites the general...and Manifest of Goods Subject to CBP Inspection and...and Manifest of Goods Subject to CBP Inspection and...with no change to the burden hours or to the...

2013-09-18

294

19 CFR 4.7b - Electronic passenger and crew arrival manifests.  

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false Electronic passenger and crew arrival manifests...Arrival and Entry of Vessels § 4.7b Electronic passenger and crew arrival manifests...beginning November 28, 2009). (b) Electronic arrival manifest â(1) General...

2013-04-01

295

Neurological manifestations in children with Sanjad-Sakati syndrome  

PubMed Central

Background: Sanjad–Sakati syndrome (SSS), also known as hypoparathyroidism–mental retardation–dysmorphism syndrome, or HRD, is a rare disorder characterized by growth and developmental delay, and by mental retardation and dysmorphic features. Objective: The objective of this study was to clarify the clinical and neurological features of SSS. Patients: Twenty-four patients were included in the study. They were seen at two hospitals in Kuwait. Methods: This was a retrospective study of patients with SSS who attended the pediatric endocrinology, genetic, and neurology clinics in the Aladan and Alfarawanya hospitals in Kuwait from September 2007 to September 2012. Clinical and radiological data were obtained from each patient’s medical records. Results: All 24 patients had the characteristic dysmorphic features and laboratory findings of SSS. Consanguinity was reported in 75% of parents. Neurological manifestations in the form of microcephaly, developmental delay, mental retardation, and seizures were reported in all patients. Computerized tomography scans and/or magnetic resonance imaging showed evidence of intracranial calcifications in 29.2% of patients. Two patients showed a thin corpus callosum, and one patient showed intraventricular hemorrhaging. Conclusion: Patients with SSS display a variety of dysmorphic features and neurological manifestations, including microcephaly, mental retardation, intracranial calcification, and epilepsy.

Elhassanien, Ahmed Farag; Alghaiaty, Hesham Abdel-Aziz

2013-01-01

296

Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome.  

PubMed

Hutchinson-Gilford progeria syndrome (HGPS) is a rare, uniformly fatal, premature aging disease with distinct dermatologic features. We sought to identify and describe the initial skin and hair findings as potential diagnostic signs of the disease. We performed a chart review of the structured initial intake histories of 39 individuals with HGPS enrolled in clinical trials from 2007 to 2010 at Boston Children's Hospital, limited to cutaneous history from birth to 24 months. Medical photographs were provided through the clinical trials and the Progeria Research Foundation Medical and Research Database at Brown University Center for Gerontology and Healthcare Research. All 39 patients reported skin and hair abnormalities within the first 24 months of life. Pathologies included sclerodermoid change, prominent superficial veins, dyspigmentation, and alopecia. The mean age of presentation for each finding was <12 months. The most frequently reported skin feature was sclerodermoid change, which commonly involved the abdomen and bilateral lower extremities. Prominent superficial vasculature manifested as circumoral cyanosis and pronounced veins on the scalp and body. Hypo- and hyperpigmentation were observed over areas of sclerodermoid change. Scalp alopecia progressed in a distinct pattern, with preservation of the hair over the midscalp and vertex areas for the longest period of time. HGPS has distinct cutaneous manifestations during the first 2 years of life that may be the first signs of disease. Awareness of these findings could expedite diagnosis. PMID:24456199

Rork, Jillian F; Huang, Jennifer T; Gordon, Leslie B; Kleinman, Monica; Kieran, Mark W; Liang, Marilyn G

2014-01-01

297

Neural correlates of impaired emotion processing in manifest Huntington's disease.  

PubMed

The complex phenotype of Huntington's disease (HD) encompasses motor, psychiatric and cognitive dysfunctions, including early impairments in emotion recognition. In this first functional magnetic resonance imaging study, we investigated emotion-processing deficits in 14 manifest HD patients and matched controls. An emotion recognition task comprised short video clips displaying one of six basic facial expressions (sadness, happiness, disgust, fear, anger and neutral). Structural changes between patients and controls were assessed by means of voxel-based morphometry. Along with deficient recognition of negative emotions, patients exhibited predominantly lower neural response to stimuli of negative valences in the amygdala, hippocampus, striatum, insula, cingulate and prefrontal cortices, as well as in sensorimotor, temporal and visual areas. Most of the observed reduced activity patterns could not be explained merely by regional volume loss. Reduced activity in the thalamus during fear correlated with lower thalamic volumes. During the processing of sadness, patients exhibited enhanced amygdala and hippocampal activity along with reduced recruitment of the medial prefrontal cortex. Higher amygdala activity was related to more pronounced amygdala atrophy and disease burden. Overall, the observed emotion-related dysfunctions in the context of structural neurodegeneration suggest both disruptions of striatal-thalamo-cortical loops and potential compensation mechanism with greater disease severity in manifest HD. PMID:23482620

Dogan, Imis; Saß, Christian; Mirzazade, Shahram; Kleiman, Alexandra; Werner, Cornelius J; Pohl, Anna; Schiefer, Johannes; Binkofski, Ferdinand; Schulz, Jörg B; Shah, N Jon; Reetz, Kathrin

2014-05-01

298

Seizure as Initial Manifestation of Aortic Dissection Type A  

PubMed Central

Background Seizure as the initial manifestation of aortic dissection is rare. Case report An 88-year-old female experienced a first generalized tonic clonic seizure, which was terminated with midazolam. Acute cerebral magnetic resonance imaging and angiography were non-informative. After awaking she complained about cramping pain in the right upper extremity, which was accompanied by involuntary flexion movements of the right upper extremity. Blood pressure was initially normal. Blood gases revealed metabolic acidosis and blood chemical investigations a markedly increased D-dimer. Consecutively blood pressure declined and transthoracic echocardiography showed pericardial effusion. A computed tomography scan of the thorax revealed an aortic dissection type A. The patient died 16 hours after admission after cardiothoracic surgeons had refused surgical treatment. Conclusion This case shows that a generalized tonic-clonic seizure may be the initial manifestation of an aortic dissection type A in the absence of thoracic chest pain and that brachyalgia may not develop earlier than with progression of the dissection.

Finsterer, Josef; Frank, Marlies; Keller, Hans; Bastowanski, Adam

2010-01-01

299

Treatment of extraintestinal manifestations in inflammatory bowel disease.  

PubMed

Inflammatory bowel disease (IBD) is a systemic disease associated with a large number of extraintestinal manifestations (EIM). EIM are present in 15-20% of patients with ulcerative colitis and in 20-40% of patients with Crohn's disease. The management of EIM is best provided by a multidisciplinary team, which includes specialists in the affected organ systems with training in the treatment of IBD. Therapeutic strategy is often empirical. This is explained by the paucity of randomized-controlled studies for the specific treatment of EIM in IBD and by the fact that treatment models are based on extrapolation from patients with similar conditions but without IBD. For most EIM, the mainstay of therapy is the treatment of the underlying active IBD. However, some EIM such as axial arthritis, pyoderma gangrenosum, uveitis and primary sclerosing cholangitis run a clinical course independent of IBD activity and need specific therapy (e.g. TNF antagonists in ankylosing spondylitis and skin manifestations). This review summarizes the conventional and novel (e.g. anti-TNF) treatment modalities, and the therapeutic implications for the management of extraintestinal symptoms in IBD, in order to assist clinicians in optimizing treatment strategies for IBD patients with EIM. PMID:23051724

Lakatos, Peter L; Lakatos, Laszlo; Kiss, Lajos S; Peyrin-Biroulet, Laurent; Schoepfer, Alain; Vavricka, Stephan

2012-01-01

300

Cistoid macular edema as first manifestation of sarcoidosis.  

PubMed

The purpose of this study is to report a case of cystoid macular edema (CME) as a rare first manifestation of ocular sarcoidosis after cataract surgery. A 60-year-old male developed a CME following uneventful phacoemulsification cataract extraction on his left eye. It resolved with conventional medical therapy. One year later the patient was diagnosed with bilateral CME. Oral corticosteroid therapy produced a significant regression. His medical and ocular histories were unremarkable and all tests for etiological diagnosis were negative. There were inflammation recurrences in his left eye, which were also treated with steroids. Optical coherence tomography showed complete resolution of foveal thickening without improvement in vision. Four years later, the patient presented with CME in both eyes. The laboratory tests included high angiotensin-converting enzyme levels and a gallium scan which were also consistent with sarcoidosis. Azathioprine was needed for management of ocular involvement, but it was withheld due to side-effects. At the present time, the CME is controlled with low-dose corticoids. Ocular involvement in sarcoidosis occurs in 20-50 % of patients. CME is not often the initial manifestation of the disease, but ocular sarcoidosis may present with a wide variety of ocular symptoms in all parts of the eye. Therefore, sarcoidosis should be kept in mind when evaluating a patient with ocular inflammation. PMID:24322273

Cabrillo-Estevez, Lucia; Juan-Marcos, Lourdes de; Kyriakou, Danai; Hernández-Galilea, Emiliano

2014-08-01

301

Neural Manifestations of Implicit Self-Esteem: An ERP Study  

PubMed Central

Behavioral research has established that humans implicitly tend to hold a positive view toward themselves. In this study, we employed the event-related potential (ERP) technique to explore neural manifestations of positive implicit self-esteem using the Go/Nogo association task (GNAT). Participants generated a response (Go) or withheld a response (Nogo) to self or others words and good or bad attributes. Behavioral data showed that participants responded faster to the self paired with good than the self paired with bad, whereas the opposite proved true for others, reflecting the positive nature of implicit self-esteem. ERP results showed an augmented N200 over the frontal areas in Nogo responses relative to Go responses. Moreover, the positive implicit self-positivity bias delayed the onset time of the N200 wave difference between Nogo and Go trials, suggesting that positive implicit self-esteem is manifested on neural activity about 270 ms after the presentation of self-relevant stimuli. These findings provide neural evidence for the positivity and automaticity of implicit self-esteem.

Wu, Lili; Cai, Huajian; Gu, Ruolei; Luo, Yu L. L.; Zhang, Jianxin; Yang, Jing; Shi, Yuanyuan; Ding, Lei

2014-01-01

302

Cardiac Manifestations of Rheumatological Conditions: A Narrative Review  

PubMed Central

Cardiovascular diseases are common in systemic rheumatologic diseases. They can be presented at the time of diagnosis or after diagnosis. The cardiac involvements can be the first presentation of rheumatologic conditions. It means that a patient with rheumatologic disease may go to a cardiologist when attacked by this disease at first. These manifestations are very different and involve different structures of the heart, and they can cause mortality and morbidity of patients with rheumatologic diseases. Cardiac involvements in these patients vary from subclinical to severe manifestations. They may need aggressive immunosuppressive therapy. The diagnosis of these conditions is very important for choosing the best treatment. Premature atherosclerosis and ischemic heart disease are increased in rheumatoid arthritis and systemic lupus erythematosus, and may be causes of mortality among them. The aggressive control of systemic inflammation in these diseases can reduce the risk of cardiovascular disease especially ischemic heart disease. Although aggressive treatment of primary rheumatologic diseases can decrease mortality rate and improve them, at this time, there are no specific guidelines and recommendations, to include aggressive control and prevention of traditional risk factors, for them.

Owlia, Mohammad Bagher; Mostafavi Pour Manshadi, Seyed Mohammad Yousof; Naderi, Nafiseh

2012-01-01

303

Amount of manifested test anxiety in the heterogeneous classroom.  

PubMed

The influence of the achievement motives, the future time orientation (FTO), the level of ability, sex of S, and perceived intrinsic instrumentality of school activity (PiI) were considered in relation to manifested test anxiety (i.e., state anxiety) at school. The analyses of results, based on data from 507 pupils in the sixth grade in elementary school showed that the achievement motives and the individual's FTO played the major roles, with success-oriented pupils having lower test anxiety than failure-oriented pupils, and high FTO pupils lower test anxiety than low FTO pupils. Ability was negatively related to test anxiety, and girls had higher test anxiety than boys. The PiI revealed no main effect on test anxiety. However, its significant interaction with the other variables indicated that the influence of the personality factors on test anxiety was most pronounced under high PiI condition. A number of interactions between the variables indicated that they had a complex relation to manifested test anxiety at school. PMID:7069636

Gjesme, T

1982-03-01

304

Epstein-Barr virus and skin manifestations in childhood.  

PubMed

Epstein-Barr virus (EBV) is a human B-lymphotropic herpes virus and one of the most common viruses in humans. Specific skin signs related to EBV infection are the exanthem of mononucleosis, which is observed more frequently after ingestion of amoxicillin, and oral hairy leukoplakia, a disease occurring mostly in immunocompromised subjects with HIV infection. Other more uncommon cutaneous disorders that have been associated with EBV infection include virus-related exanthems or diseases such as Gianotti-Crosti syndrome, erythema multiforme, and acute genital ulcers. Other skin manifestations, not correlated to virus infection, such as hydroa vacciniforme and drug-induced hypersensitivity syndrome have also been linked to EBV. The putative involvement of EBV in skin diseases is growing similarly to other areas of medicine, where the role of EBV infection is being investigated in potentially debilitating inflammatory diseases. The prognosis of EBV infection in healthy, immunocompetent individuals is excellent. However, lifelong infection, which is kept in check by the host immune system, determines an unpredictable risk of pathologic unpredictable scenarios. In this review, we describe the spectrum of non-tumoral dermatological manifestations that can follow EBV primary infection or reactivation of EBV in childhood. PMID:24073903

Di Lernia, Vito; Mansouri, Yasaman

2013-10-01

305

Hepatic manifestations of familial patent ductus venosus in adults  

PubMed Central

BACKGROUND—The ductus venosus connects the umbilical vein to the inferior vena cava during fetal life and subsequently closes rapidly after birth. It is known as patent ductus venosus when it remains patent in adulthood.?PATIENTS—A 43 year old man with a history of panhypopituitarism presented with recurrent bouts of pedal oedema associated with fatigue, hypoalbuminaemia, and elevated prothrombin time. An ultrasound examination of his abdomen with Doppler revealed notable attenuation of the main portal vein with diminished intrahepatic branches; a computed tomography scan with angiography revealed a large collateral vein within the liver consistent with a patent ductus venosus. Sequential liver biopsies showed a considerable reduction in the calibre and number of the portal veins. His younger brother, who was diagnosed with alcohol related cirrhosis, suffered from intermittent bouts of encephalopathy and was found to have the same vascular lesion. A third brother was found to have a patent ductus venosus as well as two large hepatic masses consistent with focal nodular hyperplasia.?CONCLUSION—The syndrome of familial patent ductus venosus has only previously been described in three infant brothers who presented with hepatic encephalopathy and fatty degeneration of the liver. This report documents three brothers with a patent ductus venosus presenting in adulthood with different manifestations of liver disease. The presence of the same vascular anomaly in three brothers is highly suggestive of a recessive genetic trait with an anatomical manifestation of patent ductus venosus.???Keywords: patent ductus venosus; portal vein sclerosis; familial; hepatic synthetic dysfunction; hepatic encephalopathy

Jacob, S; Farr, G; De Vun, D; Takiff, H; Mason, A

1999-01-01

306

Case report: bipolar disorder as the first manifestation of CADASIL  

PubMed Central

Background Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebrovascular disease, clinically characterized by variable manifestations of migraine, recurrent transient ischemic attack or lacunar strokes, cognitive decline, and mood disturbances. However, manic episodes have rarely been documented as an initial symptom of CADASIL and bipolar disorder presenting as the first manifestation in CADASIL has not been reported previously from evaluations by psychiatrists or psychological testing by psychologists. Case presentation A 53 year old woman developed symptoms of mania in her 50s leading to a personality change involving a continuously labile mood and irritability over a number of years. Neuropsychological testing revealed an intact memory, but impairment in attention and executive function. In the Rorschach test, she showed a high level of cognitive rigidity. Magnetic resonance imaging findings were very consistent with a diagnosis of CADASIL, which was confirmed by genetic testing for NOTCH3 mutations. Atypical antipsychotics proved to be helpful in treating her manic symptoms and for behavior control. Conclusion We present a novel case of CADASIL that first presented as bipolar disorder. We contend that when patients show a late onset personality change or chronically irritable mood that deteriorates over many years, an organic cause such as CADASIL must be considered. Further studies are needed to better understand the exact impacts of cerebral tissue lesions and psychiatric symptoms in CADASIL patients.

2014-01-01

307

Autoimmune bullous skin diseases. Part 1: Clinical manifestations.  

PubMed

Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders. PMID:21955378

Kneisel, Andrea; Hertl, Michael

2011-10-01

308

Clinical manifestations of food protein-induced enterocolitis syndrome  

PubMed Central

Purpose of review To raise awareness among healthcare providers about the clinical and laboratory findings in acute and chronic food protein-induced enterocolitis syndrome (FPIES). Recent findings FPIES can be caused by trivial exposure or rare foods. Summary FPIES is a non-IgE-mediated reaction that usually presents with acute severe repetitive vomiting and diarrhea associated with lethargy, pallor, dehydration, and even hypovolemic shock. Manifestations resolve usually within 24–48?h of elimination of the causative food. In chronic cases, symptoms may include persistent diarrhea, poor weight gain, failure to thrive, and improvement may take several days after the food elimination. In the acute cases, laboratory evaluation may reveal thrombocytosis and neutrophilia, peaking about 6?h postingestion. Depending on the severity, metabolic acidosis and methemoglobinemia may occur. In chronic cases, anemia, hypoalbuminemia and eosinophilia may be seen. Radiologic evaluation or other procedures, such as endoscopy and gastric juice analysis may show nonspecific abnormal findings. The diagnosis is based on clinical manifestations. Further studies looking at the phenotypes of FPIES are needed to identify clinical subtypes, and to understand the predisposing factors for developing FPIES compared with immediate-type, IgE-mediated gastroenteropathies.

Mane, Shikha K.; Bahna, Sami L.

2014-01-01

309

Esophageal Rupture as a Primary Manifestation in Eosinophilic Esophagitis  

PubMed Central

Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and, histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, and chest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause food impaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to the friability of the esophageal mucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave's syndrome, as a primary manifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initial manifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 of the patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majority of previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history of dysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE.

Vernon, Natalia; Mohananey, Divyanshu; Ghetmiri, Ehsan; Ghaffari, Gisoo

2014-01-01

310

19 CFR 122.49a - Electronic manifest requirement for passengers onboard commercial aircraft arriving in the United...  

Code of Federal Regulations, 2010 CFR

...2010-04-01 2010-04-01 false Electronic manifest requirement for passengers...Aircraft Entry and Entry Documents; Electronic Manifest Requirements for Passengers...Overflying the United States § 122.49a Electronic manifest requirement for...

2010-04-01

311

MUCOCUTANEOUS MANIFESTATIONS OF CHIKUNGUNYA FEVER: A STUDY FROM AN EPIDEMIC IN COASTAL KARNATAKA  

PubMed Central

Background: Chikungunya fever (CF) epidemic has recently re-emerged in India affecting large population. Mucocutaneous manifestations are an important clinical feature of the disease. We have reviewed mucocutaneous manifestations of the disease during a recent epidemic in coastal Karnataka. Patients and Methods Seventy-five “suspect cases” of CF with dermatological manifestations were examined. Results: We categorized the mucocutaneous manifestation into six groups: 1. skin rash, 2. apthae like ulcers, 3. pigmentary changes, 4. desquamation, 5. exacerbation of the existing dermatoses, 6. miscellaneous. Skin rashes were the most commonly observed skin changes followed by apthae like ulcers and pigmentary changes. Desquamation of the skin over the face is a new manifestation observed by us. Conclusion: wide spectrum of mucocutaneous manifestations is observed in CF. We have provided a classification for these manifestations, which may help in better recognition and uniform recording in future.

Bhat, Ramesh M; Rai, Yashaswi; Ramesh, Amitha; Nandakishore, B; Sukumar, D; Martis, Jacintha; Kamath, Ganesh H

2011-01-01

312

An atlas of the morphological manifestations of hidradenitis suppurativa.  

PubMed

This article is dermatological atlas of the morphologic presentations of Hidradenitis Suppurativa (HS). It includes: superficial abscesses (boils, furnucles, carbuncles), abscesses that are subcutaneous and suprafascial, pyogenic granulomas, cysts, painful erythematous papules and plaques, folliculitis, open ulcerations, chronic sinuses, fistulas, sinus tracts, scrotal and genital lyphedema, dermal contractures, keloids (some that are still pitted with follicular ostia), scarring, skin tags, fibrosis, anal fissures, fistulas (i.e. circinate, linear, arcuate), scarring folliculitis of the buttocks (from mild to cigarette-like scarring), condyloma like lesions in intertrigous areas, fishmouth scars, acne inversa, honey-comb scarring, cribiform scarring, tombstone comedones, and morphia-like plaques. HS can co-exist with other follicular diseases such as pilonidal cysts, dissecting cellulitis, acne conglobata, pyoderma gangrenosum, and acanthosis nigricans. In sum, the variety of presentations of HS as shown by these images supports the supposition that HS is a reaction pattern.HS is a follicular based diseased and its manifestations involve a multitude of follicular pathologies [1,2]. It is also known as acne inversa (AI) because of one manifestation that involves the formation of open comedones on areas besides the face. It is as yet unclear why HS is so protean in its manifestations. HS severity is assessed using the Hurley Staging System (Table 1). It also remains unclear why hidradentitis may remain limited to Hurley Stage 1, evolve to the more confluent (Hurley Stage 2), or progress even further to the fully confluent (Hurley Stage 3).In addition, HS can be associated with other follicular based diseases such as pilonidal cysts (PCs) of the sacrum and buttocks, dissecting cellulitis (DC), and acne conglobata (AC), which usually involves the face, chest, When HS occurs with PCs, DC, and/or AC it is referred to as the follicular occlusion triad or tetrad [2]. HS can more rarely be associated with pyoderma gagrenosum (PG) or Crohn disease (CD), other inflammatory diseases of the skin that are not follicular. The reason for this is unclear [2]. What AC, DC, HS, CD, and PG share is that they occur in bacterially rich environments. HS probably occurs with acanthosis nigricans because many HS patients are obese [2]. This concurrence seems under reported. PMID:24746309

Scheinfeld, Noah

2014-01-01

313

LUPUS PANNICULITIS AS AN INITIAL MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS  

PubMed Central

In May, 2003, a 28-year-old female presented with large non-healing ulcers on face, trunk and limbs covered with black hemorrhagic crust. There were no other systemic manifestations. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The lesions healed completely, with scarring, with systemic corticosteroid, hydroxychloroquine and topical 2% mupirocin. She came again in November, 2005, with malar rash, joint pain, scarring alopecia of the scalp and albuminuria. Her ANA, AntidsDNA came positive and diagnosed as having systemic lupus erythematosus (SLE). She responded well to systemic corticosteroid, antimalarial and topical antibacterial. The evolution of lupus panniculitis is slow and characterized by regression of the inflammatory lesions when treated with antimalarial drugs. The lupus panniculitis generally has a favorable course.

Patel, Raksha M; Marfatia, Y S

2010-01-01

314

Photosensitivity: epidemiology, genetics, clinical manifestations, assessment, and management.  

PubMed

Photosensitivity is a genetically determined trait that may be asymptomatic throughout life or manifest with epileptic seizures. Photosensitivity usually begins before the age of 20 years with a peak age at onset at around 12. Both natural and artificial light may trigger seizures. Precise investigation must be carried out by intermittent photic stimulation that can elicit a clearly defined EEG response; video-EEG samples are reported to illustrate the various determinants of response and the main factors altering the effectiveness of intermittent photic stimulation. Management of photosensitive epilepsy includes non-pharmacological (e.g. avoidance of the provocative stimuli and wearing appropriate tinted glass) and pharmacological treatment. This review focuses on the emerging aspects of photosensitivity, in particular, the new guidelines for intermittent photic stimulation and briefly addresses epidemiological (in non-epileptic and epileptic subjects), genetic, diagnostic, and therapeutic issues. [Published with video sequences]. PMID:23274161

Verrotti, Alberto; Beccaria, Francesca; Fiori, Federica; Montagnini, Alessandra; Capovilla, Giuseppe

2012-12-01

315

Neurobehavioral manifestations of developmental impairment of the brain  

PubMed Central

Individual characteristics of human nature (e.g. introversion, extroversion, mood, activity, adaptability, aggressiveness, social ability, anxiety) do not need to be primarily innate. They can be determined by the action of various influences and their interactions on functional development of the brain. There is ample epidemiological and experimental evidence that chemical and/or physical factors acting during sensitive time windows of the brain development can cause mental, behavioral, emotional and/or cognitive disorders. Environmental pollutants, addictive substances, drugs, malnutrition, excessive stress and/or hypoxia-ischemia were reported to induce functional maldevelopment of the brain with consequent neurobehavioral disorders. The article provides review on most significant neurobehavioral manifestations of developmental impairment of the brain during prenatal, perinatal and early postnatal period. The most known adverse factors causing developmental neurobehavioral dysfunctions in humans as well as in experimental animals are discussed.

Dubovicky, Michal

2010-01-01

316

Acute scrotal pain: an uncommon manifestation of renal vein thrombosis.  

PubMed

The clinical manifestation of renal vein thrombosis varies with the speed and degree of venous occlusion. Such patients may be asymptomatic, have minor nonspecific symptoms such as nausea or weakness, or have more specific symptoms such as upper abdominal pain, flank pain, or hematuria. Acute scrotal pain is a very uncommon clinical expression of renal vein thrombosis. Here, we report a case of membranous glomerulonephritis-induced renal vein thrombosis presented with the symptom of acute scrotal pain caused by thrombosis-induced varicocele. This case report suggests that renal vein thrombosis should be considered in the diagnosis of acute scrotal pain; it also emphasizes that an investigation of retroperitoneum should be performed for adult patients with the sudden onset of varicocele. PMID:24495531

Jou, Yeong-Chin; Jong, Ing-Chin; Hsieh, Ying-Chen; Kang, Chun-Hsiung

2014-03-01

317

The orthopaedic manifestations of the Langer-Giedion syndrome.  

PubMed

Less than 50 cases of Langer-Giedion syndrome (also known as trichorhinophalangeal syndrome with exostoses) have been reported in the English literature since its first description in 1974. Affected individuals have been described as having a bulbous nose, micrognathia, short stature, multiple cartilaginous exostoses, and large, protruding ears. We recently treated a 5-year-old, mentally retarded boy with Langer-Giedion syndrome for symptomatic multiple exostoses involving his proximal tibia and distal femur. This paper will highlight the musculoskeletal abnormalities found in this child and compare them to those of 43 patients reported in the world literature. The comparison reveals a very distinctive pattern of exostosis, demonstrating a primary altered growth pattern in the lower extremities and deformity secondary to marked ligamentous laxity. Orthopaedic surgeons are frequently the first consultants to see these children for their obvious osteochondromata. They must consider the diagnosis of Langer-Giedion syndrome to facilitate the treatment of its other manifestations. PMID:1565510

Bauermeister, S; Letts, M

1992-01-01

318

[Colonic histoplasmosis as a diagnostic manifestation of AIDS].  

PubMed

We report the case of 48 year old female patient without a history of significance importance.(refuses blood transfusion). She complaint of diarrhea of four months of duration and weight loss of 8 kg. she added episodes of hematochezia and severe anemia requiring transfusion. An intrahemorrhagic colonoscopy was performed detecting three ulcerated lesions. First at 10 cm from the anus, one in transverse colon distal, another similar ulcer in the proximal transverse The biopsies showed ulcer granulation tissue, abundant macrophages with intracytoplasmic structures consistent with histoplasmosis. Tests showed no tuberculosis or intestinal parasitosis. HIV testing (ELISA and Western Blot) were positive. The CD4 (78 cells) and extrapulmonary histoplasmosis were the criteria defined stage AIDS. The extrapulmonary histoplasmosis defines stage IV in immunosuppressed patients with HIV. Lower gastrointestinal bleeding colonic ulcer secondary to Histoplasma is a rare form presentation as a diagnostic manifestation of AIDS. PMID:20644610

Alva, Edgar; Vásquez, Jorge; Frisancho, Oscar; Yoza, Max; Yábar, Alejandro

2010-01-01

319

Dengue encephalitis-A rare manifestation of dengue fever  

PubMed Central

The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue. The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with fever, altered sensorium and seizures. Dengue NS-1 antigen test was reactive. Dengue IgM was also positive. CSF PCR was negative for herpes simplex 1 & 2. Dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant.

Madi, Deepak; Achappa, Basavaprabhu; Ramapuram, John T; Chowta, Nityananda; Laxman, Mridula; Mahalingam, Soundarya

2014-01-01

320

The multiple electrocardiographic manifestations of ventricular repolarization memory.  

PubMed

T wave "memory" is a peculiar variety of cardiac remodeling caused by a transient change in the course of ventricular depolarization (due to ventricular pacing, rate-dependent intraventricular block, ventricular preexcitation or tachyarrhythmias with wide QRS complexes). It is usually manifested by inverted T waves that appears when normal ventricular activation is restored. This phenomenon is cumulative and occurs earlier if the ventricular myocardium has previously been exposed to the same conditioning stimuli. In this article the different conditions giving rise to "classical" T wave memory development are reviewed and also "another" type of T wave memory is described. It is also shown that cardiac memory may induce not only negative (pseudo-primary) T waves but also a reversal of primary and pseudoprimary T waves leading to "normalization" of ventricular repolarization. The knowledge of these dissimilar consequences of T wave memory is essential to assess the characteristics of ventricular repolarization. PMID:24827802

Chiale, Pablo A; Etcheverry, Daniel; Pastori, Julio D; Fernandez, Pablo A; Garro, Hugo A; González, Mario D; Elizari, Marcelo V

2014-08-01

321

Central nervous system leukemia and lymphoma: computed tomographic manifestations  

SciTech Connect

Computed tomographic (CT) abnormalities in the brain were identified in 31 of 405 patients with leukemia or lymphoma. Abnormalities included neoplastic masses (15), hemorrhage (nine), abscess (two), other brain tumors (four), and methotrexate leukoencephalopathy (one). CT was normal in 374 patients including 148 with meningeal disease diagnosed by cerebrospinal fluid cytologic examination. Prior to treatment, malignant masses were isodense or of greater density with varying amounts of edema. Increase in size or number of the masses indicated worsening. Response to radiation and chemotherapy was manifested by development of a central low density region with an enhancing rim. CT findings correlated with clinical and cerebrospinal fluid findings. The differential diagnosis of the various abnormalities is considered.

Pagani, J.J. (Univ. of Texas System Cancer Center, Houston); Libshitz, H.I.; Wallace, S.; Hayman, L.A.

1981-12-01

322

Fetal haemoglobin and early manifestations of homozygous sickle cell disease.  

PubMed Central

The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age.

Bailey, K; Morris, J S; Thomas, P; Serjeant, G R

1992-01-01

323

Oral manifestations of celiac disease: a clinical guide for dentists.  

PubMed

Celiac disease (gluten sensitive enteropathy) is a common disorder affecting both children and adults. As many people with celiac disease do not present with the classic malabsorptive syndrome, delays in diagnosis are common. Dental enamel defects and recurrent aphthous ulcers, which may occur in patients with celiac disease, may be the only manifestation of this disorder. When dentists encounter these features, they should enquire about other clinical symptoms, associated disorders and family history of celiac disease. In suspected cases, the patient or family physician should be advised to obtain serologic screening for celiac disease and, if positive, confirmation of the diagnosis by intestinal biopsy. Dentists can play on important role in identifying people who may have unrecognized celiac disease. Appropriate referral and a timely diagnosis can help prevent serious complications of this disorder. PMID:22073722

Rashid, Mohsin; Zarkadas, Marion; Anca, Alexandra; Limeback, Hardy

2011-10-01

324

Neurological Manifestations of an Old Disease: A Case Report  

PubMed Central

Objective Neurological manifestations of neonatal disorders have various causes, among them neonatal tetanus, albeit rare, is a potentially fatal and preventable disease, which is seen in underdeveloped and developing countries. Although the disease has been eradicated from I.R. Iran, pregnant women immigrating to Iran from neighboring countries, especially from eastern border, may carry a risk of neonatal tetanus to the child due to inadequate tetanus immunization and inappropriate post-delivery care. It is then important to maintain a high index of suspicion for early diagnosis and prompt treatment, when infants present with poor feeding and abnormal behavior. Case presentation Here, we report the clinical course of a newborn with neonatal tetanus, who was admitted with complaints of poor feeding and muscle rigidity, more than a decade after eradication of the disorder.

ISLAMI, Zia; ATAEE NAKHAEI, Mohammad Hosein

2013-01-01

325

Clinical Manifestations and Diagnostic Challenges in Acute Porphyrias  

PubMed Central

The porphyrias are a group of disorders characterized by an enzyme deficiency in the heme biosynthetic pathway. These can be classified into either erythropoietic or hepatic forms depending on the site of the major enzyme deficiency. The diagnosis of acute porphyrias, however, can be very challenging due to overlapping features amongst the various types. Initial suspicion is based on a myriad of clinical manifestations, which then are confirmed by laboratory testing where available. Genetic testing is now also available for the different types of porphyrias, aiding in the definitive diagnosis. Here, we present a challenging case of porphyria in a patient with end-stage renal disease and present the diagnostic challenges associated with the case and the ways forward.

Trier, Henry; Krishnasamy, Vikram P.; Kasi, Pashtoon Murtaza

2013-01-01

326

Manifestation of Work Function Difference in High Order Gundlach Oscillation  

NASA Astrophysics Data System (ADS)

Gundlach oscillation (or standing-wave state) is a general phenomenon manifesting in the tunneling spectrum acquired from a metal surface using scanning tunneling spectroscopy. Previous studies relate the energy shift between peaks of the lowest-order Gundlach oscillation observed on the thin film and the metal substrate to the difference in their work functions. By observing Gundlach oscillations on Ag/Au(111), Ag/Cu(111) and Co/Cu(111) systems, we demonstrate that the work function difference is not the energy shift of the lowest order but the ones of higher order where a constant energy shift exhibits. Higher order Gundlach oscillations can thus be applied to determine the work function of thin metal films precisely.

Lin, Chun-Liang; Lu, Shin-Ming; Su, Wei-Bin; Shih, Hwa-Te; Wu, Bi-Fen; Yao, Yeong-Der; Chang, Chia-Seng; Tsong, Tien-Tzou

2008-03-01

327

Monoenomycin: A Simplified Trienomycin A Analogue that Manifests Anticancer Activity  

PubMed Central

Macrocyclic natural products are a powerful class of lead-like chemical entities. Despite commonly violating Lipinski’s “rule of 5”, these compounds often demonstrate superior drug-like physicochemical and pharmacokinetic attributes when compared to their acyclic counterparts. However, the elaborate structural architectures of such molecules require rigorous synthetic investigation that complicates analogue development and their application to drug discovery programs. To circumvent these limitations, a conformation-based approach using limited SAR and molecular modeling was implemented to design simplified analogues of trienomycin A, in which the corresponding analogues could be prepared in a succinct manner to rapidly identify essential structural components necessary for biological activity. Trienomycin A is a member of the ansamycin family of natural products that possesses potent anticancer activity. These studies revealed a novel trienomycin A analogue, monoenomycin, which manifests potent anticancer activity.

Brandt, Gary E. L.; Blagg, Brian S. J.

2011-01-01

328

Pulmonary cystic disease associated with integumentary and renal manifestations  

PubMed Central

A 69-year-old man with multiple skin lesions on his face, neck and upper torso, which first appeared in the 3rd decade of his life, was admitted to our hospital. He had cystic changes in his lungs noted on chest computed tomography (CT) scanning, as well as a left kidney mass. This patient exhibited a rare complex of renal, cutaneous and pulmonary manifestations, eponymously named Birt-Hogg-Dube syndrome, with characteristic skin features (fibrofolliculomas, trichodiscomas and acrochordons). This syndrome is due to an autosomal dominant germ-line mutation of the folliculin (FLCN) gene located at chromosome 17p11.2. Diagnosis and differentiation from other disease complexes including the skin, kidneys and lungs are important in prognostication and management of potentially life-threatening complications such as renal cell carcinoma and pneumothoraces.

Cayetano, Katherine S.; Albertson, Timothy E.; Chan, Andrew L.

2013-01-01

329

Pulmonary cystic disease associated with integumentary and renal manifestations.  

PubMed

A 69-year-old man with multiple skin lesions on his face, neck and upper torso, which first appeared in the 3rd decade of his life, was admitted to our hospital. He had cystic changes in his lungs noted on chest computed tomography (CT) scanning, as well as a left kidney mass. This patient exhibited a rare complex of renal, cutaneous and pulmonary manifestations, eponymously named Birt-Hogg-Dube syndrome, with characteristic skin features (fibrofolliculomas, trichodiscomas and acrochordons). This syndrome is due to an autosomal dominant germ-line mutation of the folliculin (FLCN) gene located at chromosome 17p11.2. Diagnosis and differentiation from other disease complexes including the skin, kidneys and lungs are important in prognostication and management of potentially life-threatening complications such as renal cell carcinoma and pneumothoraces. PMID:24285950

Cayetano, Katherine S; Albertson, Timothy E; Chan, Andrew L

2013-11-01

330

Cerebral Scedosporium apiospermum infection presenting with intestinal manifestations.  

PubMed

We present a case of cerebral Scedosporium apiospermum infection presenting with intestinal manifestations in a 64-year-old male patient on immunosuppression for orthotopic liver transplantation. At admission, the patient's chief complaint was chronic watery diarrhea and he was found to have colonic ulcers on endoscopy. His hospital course was complicated by a tonic-clonic seizure caused by a left frontal brain abscess, with the causative agent being identified by culture. He was treated with lobectomy, high-dose intravenous voriconazole, and liposomal amphotericin with clinical, endoscopic, and histologic improvement. To our knowledge, S. apiospermum has not been previously described as a cause of colitis. The septate branching appearance of the Scedosporium species is similar to the more common Aspergillus species. This case of gastrointestinal Scedosporium brings into question previously reported cases of isolated gastrointestinal aspergillosis diagnosed by histopathology. Clinical suspicion for S. apiospermum must be maintained in immunosuppressed patients presenting with neurologic and gastrointestinal symptoms. PMID:23440749

Lin, D; Kamili, Q; Qurat-Ul-Ain, K; Lai, S; Musher, D M; Hamill, R

2013-06-01

331

[Serrapeptase-induced lung injury manifesting as acute eosiniphilic pneumonia].  

PubMed

An 84-year-old man was referred to our hospital because of fever, cough, and hemoptysis. The patient had acute respiratory failure (PaO2 < 40 mmHg) on admission, with diffuse interstitial infiltration and bilateral pleural effusion. The bronchoalveolar lavage fluid was bloody, and contained a high percentage of eosinophils (32%). A diagnosis of acute eosinophilic pneumonia was established, and the patient made a rapid recovery after corticosteroids were administered. When the DLST (drug lymphocyte stimulation test) was performed after the corticosteroid therapy was stopped, it was positive for serrapeptase, which had been prescribed for chronic cystitis for 3 months before the onset of the pneumonia. This was a case of drug (serrapeptase)-induced pneumonitis manifesting as acute eosinophilic pneumonia. PMID:11019569

Sasaki, S; Kawanami, R; Motizuki, Y; Nakahara, Y; Kawamura, T; Tanaka, A; Watanabe, S

2000-07-01

332

Lupus panniculitis as an initial manifestation of systemic lupus erythematosus.  

PubMed

In May, 2003, a 28-year-old female presented with large non-healing ulcers on face, trunk and limbs covered with black hemorrhagic crust. There were no other systemic manifestations. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The lesions healed completely, with scarring, with systemic corticosteroid, hydroxychloroquine and topical 2% mupirocin. She came again in November, 2005, with malar rash, joint pain, scarring alopecia of the scalp and albuminuria. Her ANA, AntidsDNA came positive and diagnosed as having systemic lupus erythematosus (SLE). She responded well to systemic corticosteroid, antimalarial and topical antibacterial. The evolution of lupus panniculitis is slow and characterized by regression of the inflammatory lesions when treated with antimalarial drugs. The lupus panniculitis generally has a favorable course. PMID:20418989

Patel, Raksha M; Marfatia, Y S

2010-01-01

333

Abdominal Manifestations of Lymphoma: Spectrum of Imaging Features  

PubMed Central

Non-Hodgkin and Hodgkin lymphomas frequently involve many structures in the abdomen and pelvis. Extranodal disease is more common with Non-Hodgkin's lymphoma than with Hodgkin's lymphoma. Though it may be part of a systemic lymphoma, single onset of nodal lymphoma is not rare. Extranodal lymphoma has been described in virtually every organ and tissue. In decreasing order of frequency, the spleen, liver, gastrointestinal tract, pancreas, abdominal wall, genitourinary tract, adrenal, peritoneal cavity, and biliary tract are involved. The purpose of this review is to discuss and illustrate the spectrum of appearances of nodal and extranodal lymphomas, including AIDS-related lymphomas, in the abdominopelvic region using a multimodality approach, especially cross-sectional imaging techniques. The most common radiologic patterns of involvement are illustrated. Familiarity with the imaging manifestations that are diagnostically specific for lymphoma is important because imaging plays an important role in the noninvasive management of disease.

Borba-Filho, Paulo; D'Ippolito, Giuseppe; Farias, Marcella

2013-01-01

334

Cutaneous manifestation of ??-antitrypsin deficiency: panniculitis absent on biopsy.  

PubMed

Patients with ??-antitrypsin (AAT) deficiency may develop cutaneous manifestations of the disorder that histologically appear as panniculitis. Algorithms consistently emphasize measuring AAT levels when both clinical and histological features of deficiency are present; however, the patient's medical history and a physical examination alone can be extremely helpful in guiding the physician to the diagnosis of AAT deficiency. We describe a patient who presented with the classic clinical findings of AAT deficiency-associated panniculitis with surprising absence of panniculitis on repeated deep incisional biopsies. We propose a triad of classic findings that should alert the clinician to check the patient's serum AAT levels, even in the absence of panniculitis on histologic evaluation. Consideration of this clinical triad may prevent delays in the diagnosis of AAT deficiency, as early lesions may not yet demonstrate subcutaneous fat involvement. PMID:24999642

Streicher, Jenna L; Sheehan, Michael P; Armstrong, Andrew B; Mousdicas, Nico

2014-06-01

335

Oral manifestations of celiac disease: a clinical guide for dentists.  

PubMed

Celiac disease (gluten sensitive enteropathy) is a common disorder affecting both children and adults. As many people with celiac disease do not present with the classic malabsorptive syndrome, delays in diagnosis are common. Dental enamel defects and recurrent aphthous ulcers, which may occur in patients with celiac disease, may be the only manifestation of this disorder. When dentists encounter these features, they should enquire about other clinical symptoms, associated disorders and family history of celiac disease. In suspected cases, the patient or family physician should be advised to obtain serologic screening for celiac disease and, if positive, confirmation of the diagnosis by intestinal biopsy. Dentists can play an important role in identifying people who may have unrecognized celiac disease. Appropriate referral and a timely diagnosis can help prevent serious complications of this disorder. PMID:21507289

Rashid, Mohsin; Zarkadas, Marion; Anca, Alexandra; Limeback, Hardy

2011-01-01

336

Cutaneous manifestations of injectable drug use: hidden secrets.  

PubMed

Abscesses related to drug use are the most common cutaneous manifestations among injection drug users, often occurring when the veins become less accessible. In these cases, other techniques may be used to administer drugs, such as skin popping (subcutaneous injection) or muscle popping (intramuscular injection). The main risk factors for abscess formation include skin popping, use of unsterilized needles, and injection of speedball (a mixture of cocaine and heroin). We present a case of recurrent abscesses accompanied by fever, hypersomnia alternating with insomnia, diaphoresis, fatigue, recent weight loss, and agitation following subcutaneous injection of a tramadol, opipramol, and clonazepam mixture. Differential diagnoses included pyoderma gangrenosum on the basis of hepatitis C virus, skin lesions connected with human immunodeficiency virus infection, vasculitis, endocarditis, and serotonin syndrome. The patient was treated with oral antibiotics, surgical incision, and drainage of the abscesses, with consequent improvement. PMID:24818177

Bara?ska-Rybak, Wioletta; B?a?ewicz, Izabela; K?kol, Monika; Roter, Miros?aw; Nowicki, Roman

2014-04-01

337

Overflow proteinuria as a manifestation of unrecognized polymyositis  

PubMed Central

Polymyositis is a rare and gradually progressive autoimmune disease of skeletal muscle. Two main types of renal involvement have been described: acute tubular necrosis related to rhabdomyolysis and glomerulonephritis. However, cases of overflow proteinuria related to polymyositis have rarely been reported. Herein, we report a case of a 41-year-old male who presented with edema of both lower extremities. Laboratory studies revealed elevated creatine phosphokinase level, hypoalbuminemia, and a moderate amount of proteinuria, although albuminuria was not dominant. Urine electrophoresis showed an abnormally restricted zone in the ?-fraction, which suggested overflow proteinuria of non-glomerular origin. Despite intravenous hydration, his serum creatine phosphokinase level did not decrease and his symptoms did not improve. Electromyography showed myopathy, and muscle biopsy revealed findings consistent with polymyositis. After corticosteroid therapy, his creatine phosphokinase level and proteinuria decreased and his clinical symptoms improved. This case demonstrates an atypical presentation of polymyositis manifested by overflow proteinuria.

Kim, Hyun Ho; Kim, Jae Young; Kim, Sung Jun; Park, Eun Su; Shin, Seok Joon; Kang, Kwi Young; Hong, Yeon Sik; Yoon, Hye Eun

2014-01-01

338

An overview on bone manifestations in Gaucher disease.  

PubMed

Gaucher disease (GD), the most prevalent lysosomal storage disorder, affects multiple organ systems. Patients with non-neuronopathic (type 1) GD, the most common form of GD, present with hepatomegaly, splenomegaly, anemia, bleeding tendencies, thrombocytopenia, skeletal pathologies, growth retardation, and, in severe cases, pulmonary disease. The bone manifestations include bone infarcts, avascular bone necrosis, lytic lesions, osteosclerosis, fractures due to osteopenia or osteoporosis, and rarely acute osteomyelitis. Bone pain of varying intensity, fractures, and progressive joint collapses may cause impaired mobility and performances status, and increased morbidity. Enzyme replacement therapy and substrate reduction therapy have demonstrated to have beneficial effects on bone pain, bone crises, and the extent of osteoporosis. This review article gives an overview of the clinical appearance of bone pathology in GD, the possible pathophysiological mechanisms, diagnostic approaches, and the therapeutic effects of enzyme replacement therapy, substrate reduction therapy, and bone specific therapies as evaluated in current literature. PMID:21221913

Mikosch, Peter; Hughes, Derralynn

2010-12-01

339

Cutaneous Manifestations in HTLV-I Positive Blood Donors.  

PubMed

Objective(s): Infection with the human T-cell lymphotrophic virus type-I (HTLV-I) is endemic in Mashhad, Iran. In our research we evaluated the relation between exposure to this infection and the occurrence of dermatologic manifestations. Materials and Methods: 100 blood donors, who were seropositive but asymptomatic for infection with HTLV-I, were selected as case group. They were identified by the Blood Transfusion Organization Mashhad via the ELISA test and documented by PCR. Another 100 blood donors, that were seronegative for HTLV-I via the ELISA test and who were matched to the case group for age, gender, and existence of systemic diseases, were considered as the controls. Dermatologic evaluations and skin biopsies were performed if deemed necessary, and the results were statistically analyzed. Results: 73% of the case and control groups were male, while 27% in each of these groups were female. The mean age in both groups was 40.96±11.94 years. The examination indicated that 58% of the case group and 37% of the control group had cutaneous manifestations (P<0.01). The most common diseases found in the case group were aphthous stomatitis, herpes labialis, and non-genital warts, while common diseases found in the control group were herpes labialis, aphthous stomatitis, and skin tag. The frequency of aphthous stomatitis, eczema, and non-genital warts in the case group were significantly more than the control group (P<0.05). Conclusion : Cutaneous diseases can be found more frequent in asymptomatic carriers of HTLV-I than those who are HTLV-I seronegative. The aphthous stomatitis, eczema, and non-genital warts are more prevalent in those infected by HTLV-I. PMID:24470876

Yazdanpanah, Mohammad Javad; Maleki, Masoud; Joneidi, Nasaibe; Khalighi, Amir Reza; Azarpazhooh, Mahmoud Reza; Khajedaluee, Mohammad; Tehranian, Farahnaz; Shahabi, Majid; Esmaeil Khayami, Mohammad; Livani, Fatemeh

2013-03-01

340

Cutaneous Manifestations in HTLV-I Positive Blood Donors  

PubMed Central

Objective(s): Infection with the human T-cell lymphotrophic virus type-I (HTLV-I) is endemic in Mashhad, Iran. In our research we evaluated the relation between exposure to this infection and the occurrence of dermatologic manifestations. Materials and Methods: 100 blood donors, who were seropositive but asymptomatic for infection with HTLV-I, were selected as case group. They were identified by the Blood Transfusion Organization Mashhad via the ELISA test and documented by PCR. Another 100 blood donors, that were seronegative for HTLV-I via the ELISA test and who were matched to the case group for age, gender, and existence of systemic diseases, were considered as the controls. Dermatologic evaluations and skin biopsies were performed if deemed necessary, and the results were statistically analyzed. Results: 73% of the case and control groups were male, while 27% in each of these groups were female. The mean age in both groups was 40.96±11.94 years. The examination indicated that 58% of the case group and 37% of the control group had cutaneous manifestations (P<0.01). The most common diseases found in the case group were aphthous stomatitis, herpes labialis, and non-genital warts, while common diseases found in the control group were herpes labialis, aphthous stomatitis, and skin tag. The frequency of aphthous stomatitis, eczema, and non-genital warts in the case group were significantly more than the control group (P<0.05). Conclusion : Cutaneous diseases can be found more frequent in asymptomatic carriers of HTLV-I than those who are HTLV-I seronegative. The aphthous stomatitis, eczema, and non-genital warts are more prevalent in those infected by HTLV-I.

Yazdanpanah, Mohammad Javad; Maleki, Masoud; Joneidi, Nasaibe; Khalighi, Amir Reza; Azarpazhooh, Mahmoud Reza; Khajedaluee, Mohammad; Tehranian, Farahnaz; Shahabi, Majid; Esmaeil Khayami, Mohammad; Livani, Fatemeh

2013-01-01

341

Fabry disease: a review of ophthalmic and systemic manifestations.  

PubMed

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by accumulation of Gb-3 (globotriaosylceramide) in cellular lysosomes of tissues throughout the body. With advancing age, lysosomal Gb-3 accumulates in blood vessel walls, nerve cells, smooth muscle, and vital organs. Premature death commonly results from renal failure, heart attack, and stroke when the diagnosis is delayed or overlooked. One of the earliest and most distinctive physical features of FD is a whorl-like keratopathy. This finding is easily identifiable during a routine eye examination with a slit lamp, making eye care practitioners uniquely postured to identify patients and families with this incurable genetic disorder. Much of the pain, suffering, and adverse impact of FD can be avoided if an alert eye care expert sees the patient at an early age, identifies the condition, and makes the appropriate referral. The importance of obtaining a thorough medical history, ancestral health history, and review of systems to correlate ocular and systemic manifestations is emphasized. This report reviews the multisystem involvement of FD and describes the clinical characteristics and expected chronological appearance of ophthalmic and systemic manifestations. The discoveries of late-onset variants, increased prevalence, and modified inheritance pattern of FD are discussed. The profound therapeutic effects of recombinant enzyme replacement therapy (ERT) on multiple organ systems are detailed and demonstrated in a Fabry proband. Improved quality and quantity of life after initiation of ERT underscore the importance of early recognition and correlation of FD symptoms and clinical signs. Treatment strategies and the effectiveness of new adjunctive chaperone therapy are addressed. PMID:23334311

Sivley, Melanie D

2013-02-01

342

Lateral asymmetry of early seizure manifestations in experimental generalized epilepsy.  

PubMed

Reorganization of seizure networks during epileptogenesis involves cortico-subcortical and interhemispheric interactions. In the audiogenic kindling (AK) model of generalized tonic-clonic seizures, upstream seizure propagation along ascending brainstem-to-forebrain pathways determines progressive intensification of repeated sound-induced convulsions. Full-blown audiogenic seizures are bilaterally symmetric and their repetition results in bisynchronous recruiting the cortex in secondary epileptogenesis. The present study describes lateral asymmetry of initial behavioral and EEG manifestations of audiogenic seizures and AK in Wistar and WAG/Rij rats with acoustic hypersensitivity. These rats exhibit consistent individual lateralization of running seizures (run directionality) induced by repeated binaural stimulation. Since this initial preconvulsive running reflects seizure onset in the auditory brainstem, the running asymmetry suggests non-symmetric early epileptic activation of brainstem substrates by sound in these rats. Repetition of the asymmetric brainstem seizures led to asynchronous recruiting the cortex into seizure network and lateralization of running seizures was predictive for asymmetry of early cortical seizure manifestations in Wistar and WAG/Rij rats. Both electrographic markers of AK, spreading depression (SD) and post-running afterdischarge, first appeared in the cortex ipsilateral to run direction, suggesting lateralized brainstem-to-forebrain seizure generalization during AK. At the population level, no bias in lateralization of running and SD was found in Wistar and WAG/Rij rats but incidence of secondary cortical seizures varied, depending on strain and run laterality. Among Wistar rats, cortical seizures developed more rarely in right-runners than in left-runners, suggesting enhanced resistance of the right hemisphere to epileptogenesis in rats of this strain. WAG/Rij rats with mixed (absence and audiogenic) epilepsy showed weak lateralization of early cortical seizures and no left-right difference in their incidence during AK. Present findings suggest (1) lateralized brainstem-to-forebrain seizure propagation and hemispheric difference in its facility in Wistar rats, (2) alterations of intra- and interhemispheric seizure propagation in WAG/Rij rats with genetic absence epilepsy. PMID:22525136

Vinogradova, L V; Shatskova, A B

2012-06-28

343

Rapidly Progressive Cardiac Manifestation of Beh?et's Disease Involving Conduction System and Aortic Valve  

PubMed Central

Cardiac conduction system impairment is a rare clinical manifestation of Behçet's disease. We report a patient who showed 1st degree atrioventricular block at first presentation, and showed aggravated finding of 3rd degree atrioventricular block on five months later. His cardiac manifestation finally developed to acute severe aortic regurgitation on six months later from his first cardiac manifestation. We observed this rapid progression during 6 months and successfully improved symptom and disease severity of the patient with treatment targeting Behçet's disease.

Yu, Jin Sok; Ji, Eun Hye; Kwon, Hee Sun; Kim, Jin Seok; Choi, Kyu Young; Kwon, Beom June; Kim, Dong Bin; Jang, Seong Won; Kim, Jae Hyung

2011-01-01

344

Clinical profile of cutaneous manifestations with and without hematologic disease: A comparative study  

PubMed Central

Aim: The aim was to study the clinical profile of cutaneous manifestations of hematologic disorders and to compare it with that of non-hematologic disorders. Materials and Methods: Cutaneous manifestations of hematologic diseases fall in seven well-defined categories. A total of 153 outpatients with skin manifestations fitting in these categories were enrolled in a comparative study of 1-year duration. Clinical profile of these cutaneous manifestations was studied and any underlying hematologic disorder was ruled out with the help of a hematologist. Difference in the clinical profile of cutaneous manifestations with and without hematologic diseases was studied. Result: Of the 26,174 outpatients during the study period, 153 had cutaneous manifestations fitting in the categories of hematologic disorders. Of these 153 patients, 33 had hematologic disease as the cause of their cutaneous manifestation (21.57%), whereas 78.42% had no hematologic disorder. Disorders of hemostasis formed the largest group (36%) followed by cutaneous deposits/infiltrations (15%), vesiculobullous disorders (6%), and cutaneous vasculitis (9%) were least commonly associated with hematologic disorders. Conclusion: Hematologic diseases are associated with complex array of cutaneous manifestations. The incidence of hematologic disease–associated cutaneous manifestations was 0.13%. Findings of this study will help dermatologists and physicians with the early recognition of cutaneous signs of hematologic disorders.

Pande, Sushil Yashwant; Kharkar, Vidya

2014-01-01

345

Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis.  

PubMed

Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs' positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease. PMID:22607576

Korkmaz, Serdal; Elaldi, Nazif; Kayatas, Mansur; Sencan, Mehmet; Yildiz, Esin

2012-01-01

346

Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis  

PubMed Central

Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs’ positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease.

2012-01-01

347

Obsessive-Compulsive Disorder is not a Clinical Manifestation of the DYT1 Dystonia Gene  

PubMed Central

Prior studies suggest that obsessive-compulsive symptoms (OCS) and disorder (OCD) are comorbid with dystonia. We tested if OCS/OCD is a clinical manifestation of the DYT1 dystonia mutation by interviewing members of families with an identified DYT1 mutation, and classifying by manifesting carriers (MC), non-manifesting carriers (NMC), and non-carriers (NC). We found that OCD/OCS are not increased in DYT1 mutation carriers compared with NC, nor is OCD associated with manifesting DYT1 dystonia.

Heiman, Gary A.; Ottman, Ruth; Saunders-Pullman, Rachel J.; Ozelius, Laurie J.; Risch, Neil J.; Bressman, Susan B.

2013-01-01

348

Chest radiographic and computed tomographic manifestations in allergic bronchopulmonary aspergillosis  

PubMed Central

AIM: To investigate the chest radiographic and high resolution computed tomography (HRCT) chest manifestations in glucocorticoid-naïve allergic bronchopulmonary aspergillosis (ABPA) patients. METHODS: This is a prospective observational study and includes 60 consecutive glucocorticoid-naïve patients with ABPA who underwent chest radiography and HRCT of the chest (1.25 mm every 10 mm) in the routine diagnostic workup for ABPA. RESULTS: Chest radiographs were normal in 50% of cases. Of the remainder, most patients demonstrated permanent findings in the form of parallel line and ring shadows suggesting bronchiectasis. Consolidation was detected in 17 cases but in the majority, the corresponding HRCT chest scan showed mucus-filled bronchiectatic cavities. Chest HRCT was normal in 22 patients, while central bronchiectasis (CB) was demonstrated in the remaining 38 patients. Bronchiectasis extended to the periphery in 33%-43% depending on the criteria used for defining CB. The other findings observed on HRCT were mucoid impaction, centrilobular nodules and high-attenuation mucus in decreasing order of frequency. CONCLUSION: Patients with ABPA can present with normal HRCT chest scans. Central bronchiectasis cannot be considered a characteristic feature of ABPA as peripheral bronchiectasis is commonly observed. Consolidation is an uncommon finding in ABPA.

Agarwal, Ritesh; Khan, Ajmal; Garg, Mandeep; Aggarwal, Ashutosh N; Gupta, Dheeraj

2012-01-01

349

[A case of toxic epidermal necrolysis with severe intestinal manifestation].  

PubMed

TEN is a severe dermatological disorder characterized by extended epidermal necrosis. Disseminated mucosal erosions have been occasionally reported to occur in the gastrointestinal tract. We report a case of toxic epidermal necrolysis (TEN) with severe intestinal manifestation. A 52-year-old woman was admitted with high fever, skin eruption and severe diarrhea. She was diagnosed as toxic epidermal necrolysis (TEN), which was most likely due to nonsteroidal anti-inflammatory drugs (NSAIDs). After skin lesion recovered, fever and bloody diarrhea went on. Colonoscopy and X-ray revealed lead-pipe like stenotic long loops which was caused by erosion and sublation of whole mucosa of large intestinum, and severe stenosis of ileum end. The ileo-cecal region was resected on the 216(th) hospital day. Pathological examination showed sublation of mucoepithelium and inflammatory change in the muco-submucosal layer, but muscular layers of mucosa remained intact. Pathologically, the intestinal lesion resemble the changes in the early skin lesions and seems to be part of the systemic lesion of TEN. PMID:18772576

Otomi, Megumi; Yano, Mitsuyasu; Aoki, Hidetoshi; Takahashi, Kouji; Omoya, Toshihiro; Suzuki, Yasuhiro; Nakamoto, Jiro; Kataoka, Koichi; Yagi, Yoshiyuki; Yamamoto, Yosuke

2008-09-01

350

Cardiac manifestations in the mouse model of mucopolysaccharidosis I  

PubMed Central

Mucopolysaccharidosis I (MPS I, ?-l-iduronidase deficiency disease) is a heritable lysosomal storage disorder involving multiple organs, including the heart. Malfunction of the heart is also a major manifestation in the mouse model of MPS I, progressing in severity from 6 to 10 months (of a one-year life span). In comparisons of MPS I with wild type mice, the heart was found enlarged, with thickened septal and posterior walls, primarily because of infiltration of the muscle by storage-laden cells. Heart valves were enlarged and misshapen, and contained large numbers of highly vacuolated interstitial cells. The thickened aortic wall contained vacuolated smooth muscle cells and interrupted elastic fibers. Hemodynamic measurements and echocardiography revealed reduced left ventricular function as well as mitral and aortic regurgitation. But despite these abnormalities, free-roaming MPS I mice implanted with radio telemetry devices showed surprisingly normal heart rate and blood pressure, though their electrocardiograms were abnormal. An incidental finding of the telemetry studies was a disturbed circadian rhythm in the MPS I mice. Restoration of enzyme activity in the heart of one mouse, by transplantation of retrovirally modified bone marrow, resulted in normalization of left ventricular function as well as loss of storage vacuoles in myocytes and endothelial cells, though not in valvular interstitial cells. This study demonstrates the usefulness of the mouse model for in-depth studies of the cardiovascular component of MPS I.

Jordan, Maria C.; Zheng, Yi; Ryazantsev, Sergey; Rozengurt, Nora; Roos, Kenneth P.; Neufeld, Elizabeth F.

2005-01-01

351

[Cardiovascular manifestations of thyrotoxicosis and thyroid dysfunction caused by amiodarone].  

PubMed

Cardiovascular manifestations of thyrotoxicosis and thyroid dysfunction caused by amiodarone. The cardiovascular symptoms of thyrotoxicosis were described more than two hundred years ago and remained the basis of diagnosis in modern medicine. Myocardium, peripheral circulation and sympathetic nerve system, all affecting cardiovascular hemodynamics, are influenced by thyroid hormones in many ways. Sub-clinical hyperthyroidism is characterized by suppressed thyroid stimulating hormone and normal free triiodothyronine and free thyroxine levels. Cardiovascular symptoms: elevation of heart rate, myocardial contractility, stroke volume, myocardial oxygen consumption, systolic blood pressure and reduction in systemic vascular resistance and diastolic blood pressure can be often seen even in case of subclinical hyperthyroidism. Thyrotoxicosis exacerbates the symptoms of a preexisting heart disease, but it can also cause complaints in case of a structurally normal heart. The most common cardiac complications are arrhythmias (mainly atrial fibrillation), heart failure and hypertension. Amiodarone is used for the treatment and prevention of several arrhythmias. It is safely applicable even in case of left ventricular dysfunction. The more common application is limited by its side effects that can develop even at low doses and may involve several organs (thyroid gland, lungs, liver, heart, nerve system among others). The complex effect of amiodarone on thyroid function ranges from mild abnormalities of thyroid function tests to overt thyrotoxicosis or hypothyroidism. PMID:15638034

Simkó, József; Barta, Kitti; Szabó, Zoltán; Varga, Emma; Nagy, Endre; Lorincz, István

2004-11-28

352

STING manifests self DNA-dependent inflammatory disease  

PubMed Central

Inflammatory autoimmune diseases such as systemic lupus erythematosus (SLE) and polyarthritis are characterized by chronic cytokine overproduction, suggesting that the stimulation of host innate immune responses, speculatively by persistent infection or self nucleic acids, plays a role in the manifestation of these disorders. Mice lacking DNase II die during embryonic development through comparable inflammatory disease because phagocytosed DNA from apoptotic cells cannot be adequately digested and intracellular host DNA sensor pathways are engaged, resulting in the production of a variety of cytokines including type I IFN. The cellular sensor pathway(s) responsible for triggering DNA-mediated inflammation aggravated autoimmune disease remains to be determined. However, we report here that Stimulator of IFN Genes (STING) is responsible for inflammation-related embryonic death in DNase II defective mice initiated by self DNA. DNase II-dependent embryonic lethality was rescued by loss of STING function, and polyarthritis completely prevented because cytosolic DNA failed to robustly trigger cytokine production through STING-controlled signaling pathways. Our data provides significant molecular insight into the causes of DNA-mediated inflammatory disorders and affords a target that could plausibly be therapeutically controlled to help prevent such diseases.

Ahn, Jeonghyun; Gutman, Delia; Saijo, Shinobu; Barber, Glen N.

2012-01-01

353

Cutaneous manifestations of primary immunodeficiency diseases in children.  

PubMed

Primary immunodeficiency diseases (PIDs) are rare but include severe conditions found predominantly in children, Most PIDs have cutaneous manifestations that may be important as early diagnostic features. The purpose of this study was to determine the frequency and nature of cutaneous alterations associated with PIDs. This article is a cross-sectional study at the department of allergy and clinical immunology of children's medical center conducted between December 5, 2001 and April 20, 2002. The subjects included pediatric patients with a diagnosis of PID and dermatological diagnoses were made by a dermatologist. Two hundred and ten patients were studied They consisted of 68 cases of humoral deficiency, 22 cases of cellular and combined deficiencies, 57 cases of phagocytic defects and 63 cases of other PIDs. In 67 cases (31.8%) the cutaneous alterations preceded and were the basis for clinical immunological diagnosis. Overall cutaneous alterations were infections in 99 cases and eczematous dermatitis in 27 cases. Our findings support the results of other studies that most PIDs have cutaneous features which being their typical aspects are highly suggestive for the diagnosis of PIDs. PMID:17237563

Moin, Athar; Farhoudi, Abolhassan; Moin, Mostafa; Pourpak, Zahra; Bazargan, Nasrin

2006-09-01

354

Season of birth as predictor of atopic manifestations  

PubMed Central

Accepted 8 November 1996? The relation between month of birth, sensitisation, and manifestations of atopy was assessed in 209 children who were followed from birth to 12-15 years. Children born during the tree pollen season were less likely to develop allergic rhinoconjunctivitis, IgE antibodies to pollen, or a positive screening test for IgE antibodies (odds ratio 0.28, 0.41, 0.35, respectively) than children born during the rest of the year. The prevalence of IgE antibodies to food and animal dander at 9 months and to atopic disease was higher in children born in the autumn and winter, that is, September to February, compared to the spring and summer (egg 20% v 6%; milk 10% v 2%). Thus sensitisation to pollen and allergic rhinoconjunctivitis is least common in children born in the spring, while birth in September to February is associated with an increased incidence of sensitisation to food and of atopic disease.??

Nilsson, L; Bjorksten, B; Hattevig, G; Kjellman, B; Sigurs, N; Kjellman, N

1997-01-01

355

Hematologic manifestations in a child with HIV; a Case Report  

PubMed Central

Background Immune deficiency in human might be primary or secondary and could be seen with a wide variety of manifestations. In the following, we presented a Child with various complains that diagnosed to have HIV infection. Case Report A 2/5 y/o child was admitted to the hospital for FUO with prolonged cough, FTT, cervical lymphadenopathy, hepatosplenomegaly and bilateral optic neuritis. . He was hospitalized for fever, cytopenia and hepatosplenomegaly one year ago, and three months later in an outpatient visit, these signs improved, except thrombocytopenia. In evaluation, bicytopenia, elevated ESR, hyperlipidemia, hyperproteinemia, thrombosis of the transverse sinus of brain, antiphospholipid antibodies , decreased levels of protein S and factor V Leiden and increased level of anti thrombin III were detected. Consequently, the result of HIV antibody showed positive. In addition to warfarin and cotrimoxazole therapy, he was referred to special center for possible HARRT therapy. Conclusion In approach to patients with various clinical presentations such as cytopenia, recurrent or persistent lymphadenopathy, unexplained hyperproteinemia or hyperlipidemia, evaluation of HIV infection is highly recommended for consideration and further therapy.

Osiya, Sh; Binesh, F; Ferdosian, F; Shakiba, M

2012-01-01

356

Clinical manifestation and prognosis of active ocular toxoplasmosis in Iran.  

PubMed

Toxoplasmosis is the most common cause of posterior uveitis in the world. This study described the clinical characteristics and visual outcome of 193 patients with ocular toxoplasmosis at Feiz Hospital (Isfahan, Iran) during the last six years. The setting and design used was a retrospective non-comparative observational case series. In this study, 193 patients with ocular toxoplasmosis (111 female, 82 male) were enrolled. The distribution of symptoms and fundoscopic findings were studied. The most-reported chief complaint was blurred vision in 96 % (184 patients) and floaters in 13.47 % (25 patients) of cases and most frequent clinical manifestations were chorioretinitis 98.48 % (190 patients), macular scars 50.7 % (98 patients), and atrophic optic papilla two (1.03 %) patients. Primary retinal lesions were observed in 16 (8.2 %) and combination of active lesions and old retinochoroidal scars in 177 (91.7 %) of the patients. Retinal detachment occurred in 11 (5.69 %) patients. Bilateral involvement was found in 27 % of patients. Blindness was 0.05 % after treatment. Recurrence rate was 14.5 %. In conclusion, ocular toxoplasmosis substantially varies among patients with different age, gender, site of lesion and other factors. Suddenly blurred vision, floater, and pain could be caused by Toxoplasma gondii. Flashing, may necessitate a more precise peripheral fundus examination. PMID:22733253

Kianersi, Farzan; Naderi Beni, Afsaneh; Naderi Beni, Zahra

2012-12-01

357

MHC associations with clinical and autoantibody manifestations in European SLE.  

PubMed

Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease affecting multiple organ systems and characterized by autoantibody formation to nuclear components. Although genetic variation within the major histocompatibility complex (MHC) is associated with SLE, its role in the development of clinical manifestations and autoantibody production is not well defined. We conducted a meta-analysis of four independent European SLE case collections for associations between SLE sub-phenotypes and MHC single-nucleotide polymorphism genotypes, human leukocyte antigen (HLA) alleles and variant HLA amino acids. Of the 11 American College of Rheumatology criteria and 7 autoantibody sub-phenotypes examined, anti-Ro/SSA and anti-La/SSB antibody subsets exhibited the highest number and most statistically significant associations. HLA-DRB1*03:01 was significantly associated with both sub-phenotypes. We found evidence of associations independent of MHC class II variants in the anti-Ro subset alone. Conditional analyses showed that anti-Ro and anti-La subsets are independently associated with HLA-DRB1*0301, and that the HLA-DRB1*03:01 association with SLE is largely but not completely driven by the association of this allele with these sub-phenotypes. Our results provide strong evidence for a multilevel risk model for HLA-DRB1*03:01 in SLE, where the association with anti-Ro and anti-La antibody-positive SLE is much stronger than SLE without these autoantibodies. PMID:24598797

Morris, D L; Fernando, M M A; Taylor, K E; Chung, S A; Nititham, J; Alarcón-Riquelme, M E; Barcellos, L F; Behrens, T W; Cotsapas, C; Gaffney, P M; Graham, R R; Pons-Estel, B A; Gregersen, P K; Harley, J B; Hauser, S L; Hom, G; Langefeld, C D; Noble, J A; Rioux, J D; Seldin, M F; Vyse, T J; Criswell, L A

2014-04-01

358

Adult Mitochondrial DNA Depletion Syndrome with Mild Manifestations  

PubMed Central

Mitochondrial DNA depletion syndrome (MDS) is usually a severe disorder of infancy or childhood, due to a reduced copy number of mtDNA molecules. MDS with only mild, nonspecific clinical manifestations and onset in adulthood has not been reported. A 47-year-old Caucasian female with short stature and a history of migraine, endometriosis, Crohn’s disease, C-cell carcinoma of the thyroid gland, and a family history positive for mitochondrial disorder (2 sisters, aunt, niece), developed day-time sleepiness, exercise intolerance, and myalgias in the lower-limb muscles since age 46y. She slept 9-10 hours during the night and 2 hours after lunch daily. Clinical exam revealed sore neck muscles, bilateral ptosis, and reduced Achilles tendon reflexes exclusively. Blood tests revealed hyperlipidemia exclusively. Nerve conduction studies, needle electromyography, and cerebral and spinal magnetic resonance imaging were noninformative. Muscle biopsy revealed detached lobulated fibers with subsarcolemmal accentuation of the NADH and SDH staining. Realtime polymerase chain reaction revealed depletion of the mtDNA down to 9% of normal. MDS may be associated with a mild phenotype in adults and may not significantly progress during the first year after onset. In an adult with hypersomnia, severe tiredness, exercise intolerance, and a family history positive for mitochondrial disorder, a MDS should be considered.

Finsterer, Josef; G. Kovacs, Gabor; Ahting, Uwe

2013-01-01

359

Morphologic features of extrahepatic manifestations of hepatitis C virus infection.  

PubMed

Cirrhosis and hepatocellular carcinoma are the prototypic complications of chronic hepatitis C virus infection in the liver. However, hepatitis C virus also affects a variety of other organs that may lead to significant morbidity and mortality. Extrahepatic manifestations of hepatitis C infection include a multitude of disease processes affecting the small vessels, skin, kidneys, salivary gland, eyes, thyroid, and immunologic system. The majority of these conditions are thought to be immune mediated. The most documented of these entities is mixed cryoglobulinemia. Morphologically, immune complex depositions can be identified in small vessels and glomerular capillary walls, leading to leukoclastic vasculitis in the skin and membranoproliferative glomerulonephritis in the kidney. Other HCV-associated entities include porphyria cutanea tarda, lichen planus, necrolytic acral erythema, membranous glomerulonephritis, diabetic nephropathy, B-cell non-Hodgkin lymphomas, insulin resistance, sialadenitis, sicca syndrome, and autoimmune thyroiditis. This paper highlights the histomorphologic features of these processes, which are typically characterized by chronic inflammation, immune complex deposition, and immunoproliferative disease in the affected organ. PMID:22919404

Ko, Huaibin M; Hernandez-Prera, Juan C; Zhu, Hongfa; Dikman, Steven H; Sidhu, Harleen K; Ward, Stephen C; Thung, Swan N

2012-01-01

360

Prognosis and possible presymptomatic manifestations of congestive cardiomyopathy (COCM).  

PubMed Central

In order to find evidence of prognosis and of presymptomatic manifestation of congestive cardiomyopathy (COCM) in fifty-eight patients, the extent of morphological changes of endomyocardial catheter biopsy (EMCB), clinical and haemodynamic data were correlated to the clinical course. In addition, clinical, haemodynamic, angiographic, morphological and His-bundle electrographic studies were performed in patients with left bundle branch block (LBBB), normal left ventricular end-diastolic volume, and normal coronary arteries (n = 43). Related to a 10-year mortality rate of 70% from the onset of symptoms, COCM is one of the most severe heart diseases. Endomyocardial catheter biopsy (EMCB) allowed clear prognostic separation in patients with COCM and seems to be of diagnostic value in patients with only slightly enlarged hearts and in patients with a short history of symptoms. The studies also revealed much evidence that at least some patients with LBBB, normal left ventricular end-diastolic volume (LVEDV) and normal coronary arteries exhibit an early stage of COCM. In these patients especially EMCB with severe changes of heart muscle cells and/or impaired left ventricular function may indicate subsequent COCM. So that there is now a new indication for performing EMCB.

Kuhn, H.; Breithardt, G.; Knieriem, H. J.; Kohler, E.; Losse, B.; Seipel, L.; Loogen, F.

1978-01-01

361

The urological manifestations of the tethered spinal cord.  

PubMed

The tethered cord is the fixation of the cord resulting in stretching as growth occurs. In this paper, three cases of tethered cord with symptoms related to the urinary tract were presented. In the first case, a 12-year-old girl presenting with abdominal pain and urinary incontinence had bilateral hydronephrosis and neurogenic bladder due to a tethered cord without having any other neuropathological manifestation. In the second case, an eight-year-old girl presented with enuresis and a mass in her back was found to have a lipomyomeningocele, hyperactive tendon reflexes in the lower limbs and pes cavus. Tethered cord associated with lipomyomeningocele caused a neurogenic bladder and bilateral hydronephrosis. In the third case, a seven-month-old girl presented with hydrocephalus as well as bilateral dilation of the renal pelvis, unilateral ureteral duplication and vesicoureteral reflux. A tethered cord was revealed in this patient, who had a meningomyelocele operation in the neonatal period. Renal function test in the first two cases were abnormal. PMID:8160284

Kavukçu, S; Ozaksoy, D; Türkmen, M; Kovanlikaya, I; Köse, G; Tavli, V

1993-01-01

362

Thrombosed aneurysm as the initial manifestation of Takayasu arteritis.  

PubMed

Takayasu arteritis (TA) is a large-vessel vasculitis characterised by stenosis, dilation and/or aneurysm formation. We present a case of a 43-year-old man with an initial manifestation of an acute thrombosed aneurysm. He was found to have a thrombosed right common femoral artery aneurysm on Doppler ultrasound. Physical examinations revealed a substantial difference in blood pressure level between bilateral upper extremities, and absent pulses at right upper and lower extremities. The diagnosis was confirmed by angiography, which revealed 100% occlusion of the right common femoral artery. Biopsy of the thrombosed aneurysm was consistent with a diffuse lymphocytic subendothelial infiltrate. The patient was treated with high-dose corticosteroids and had a good response to treatment, as demonstrated by a decrease in sedimentation rate, and recovery of the right side pulses. This case helps to create awareness among physicians that TA may present with an acute occlusion of an aneurysm without the typical warning symptoms of TA. PMID:24913078

Ojeda, Joel; Rodríguez, Yerania; Ríos, Grissel

2014-01-01

363

Ergot Intoxication: Historical Review and Description of Unusual Clinical Manifestations  

PubMed Central

Clinical ergotism as seen today results almost exclusively from the excessive intake of ergotamine tartrate in the treatment of migraine headache. Although both gangrenous and convulsive symptoms are seen in naturally occurring ergotism resulting from the ingestion of fungus infected rye, only gangrenous ergotism has been reported following the excessive ingestion of ergotamine tartrate. The symptoms of both iatrogenic and naturally occurring ergotism appear to result from regional ischemia caused by ergot induced vasospasm. This report discribes experiences in the diagnosis and management of two patients with unusual manifestations of iatrogenic ergotism. One patient presented with ischemia of all extremities and bilateral foot drop probably due to ischemic damage to the common peroneal nerves, a finding not previously described in ergot intoxication. The foot drop totally resolved in several months following the discontinuation of ergot. A second patient presented with unilateral leg ischemia and transient monocular blindness, both of which resolved after discontinuation of ergot. Both patients displayed typical angiographic findings of ergotism. There is no convincing evidence that any treatment other than discontinuation of ergotamine is of benefit in the treatment of iatrogenic ergotism. ImagesFig. 1A.Fig. 1B.

Merhoff, G. Craig; Porter, John M.

1974-01-01

364

Para-dichlorobenzene toxicity - a review of potential neurotoxic manifestations  

PubMed Central

Background: Para-dichlorobenzene (PDCB) is an active ingredient of mothballs, deodorizers and fumigants. Due to the easy availability of this chemical, there is a considerable risk for accidental or intentional toxic exposure. Recently, multiple cases of PDCB toxicity due to mothball ingestion were reported. PDCB toxicity can affect multiple organ systems including liver, kidneys, skin, lung and the central nervous system (CNS). CNS toxicity often results in leukoencephalopathy and heterogeneous neurological manifestations. Objectives: The objective of this study was to illustrate the clinical presentation, imaging findings, diagnosis and management of PDCB toxicity. Methods: We carried out a literature review of the pharmacological and toxicological properties of PDCB. Conclusions: PDCB and other aromatic hydrocarbons are capable of CNS tissue damage and in promoting functional neurological decline. While very little is currently known about prevalence of PDCB addiction, it cannot be ruled out that its illicit use among young people is under-recognized. The number of cases of PDCB toxicity might also rise due to the increasing industrial and domestic use of this chemical.

Dubey, Divyanshu; Sharma, Vibhash D.; Pass, Steven E.; Sawhney, Anshudha

2014-01-01

365

Computer assisted learning (CAL) of oral manifestations of HIV disease.  

PubMed

General dental practitioners (GDPs) in the UK may wish additional education on relevant aspects of human immunodeficiency virus (HIV) disease. The aim of the present study was to develop and assess a computer assisted learning package on the oral manifestations of HIV disease of relevance to GDPs. A package was developed using a commercially-available software development tool and assessed by a group of 75 GDPs interested in education and computers. Fifty-four (72%) of the GDPs completed a self-administered questionnaire of their opinions of the package. The majority reported the package to be easy to load and run, that it provided clear instructions and displays, and that it was a more effective educational tool than videotapes, audiotapes, professional journals and textbooks, and of similar benefit as post-graduate courses. The GDPs often commented favourably on the effectiveness of the clinical images and use of questions and answers, although some had criticisms of these and other aspects of the package. As a consequence of this investigation the package has been modified and distributed to GDPs in England and Wales. PMID:8854425

Porter, S R; Telford, A; Chandler, K; Furber, S; Williams, J; Price, S; Scully, C; Triantos, D; Bain, L

1996-09-01

366

Age Dependence of Clinical and Pathological Manifestations of Autoimmune Demyelination  

PubMed Central

A prominent feature of the clinical spectrum of multiple sclerosis (MS) is its high incidence of onset in the third decade of life and the relative rarity of clinical manifestations during childhood and adolescence, features suggestive of age-related restriction of clinical expression. Experimental allergic encephalomyelitis (EAE), a model of central nervous system (CNS) autoimmune demyelination with many similarities to MS, has a uniform rapid onset and a high incidence of clinical and pathological disease in adult (mature) animals. Like MS, EAE is most commonly seen and studied in female adults. In this study, age-related resistance to clinical EAE has been examined with the adoptive transfer model of EAE in SJL mice that received myelin basic protein-sensitized cells from animals 10 days (sucklings) to 12 weeks (young adults) of age. A variable delay before expression of clinical EAE was observed between the different age groups. The preclinical period was longest in the younger (<14 days of age) animals, and shortest in animals 6 to 8 weeks old at time of transfer. Young animals initially resistant to EAE eventually expressed well-developed clinical signs by 6 to 7 weeks of age. This was followed by a remitting, relapsing clinical course. For each age at time of sensitization, increased susceptibility of females compared to males was observed. Examination of the CNS of younger animal groups during the preclinical period showed lesions of acute EAE. Older age groups developed onset of signs coincident with acute CNS lesions. This age-related resistance to clinical EAE in developing mice is reminiscent of an age-related characteristic of MS previously difficult to study in vivo. The associated subclinical CNS pathology and age-related immune functions found in young animals may be relevant to the increasing clinical expression of MS with maturation, and may allow study of factors associated with the known occasional poor correlation of CNS inflammation and demyelination and clinical changes in this disease.

Smith, Mary E.; Eller, Nancy L.; McFarland, Henry F.; Racke, Michael K.; Raine, Cedric S.

1999-01-01

367

Ocular Manifestations in Infants Resulted from Assisted Reproductive Technology (ART)  

PubMed Central

Objective Nowadays, many infertile couples can have child by assistant reproductive technology (ART). Always the undesirable effects of these methods on newborn are considered and are evaluated. The aim of this study is to describe the impact of ART on ocular and visual performances of infants born by these methods. Materials and methods In a cross-sectional descriptive study, 479 infants aged three-nine months presented to an optometry clinic of Child Health and Development Research Department (CHDRD), Tehran, Iran. Static retinoscopy, qualitative fixation evaluation, Hirschberg test, red reflex assessment and external eye examination were carried out. Other information such as birth weight and maturity of the infants was recorded. Results It was possible to assess only 320 out of 479 infants due to general condition of some participants. Comparison of mean refractive error in infants’ right and left eyes did not show any significant difference. Our findings confirmed that 20.3% had poor fixation, while 2.9% revealed manifest strabismus. The results also revealed the prevalences of myopia, hyperopia and emmetropia are 2.9%, 87%, and 10.1%, respectively. Red reflex abnormalities were significantly found in boys and in preterm infants (p < 0.05). Failure of fixation control was seen more frequently with increasing refractive error, which significantly developed in preterm infants (p < 0.001). Conclusion These results reflect the necessity of more comprehensive assessments and further follow-up of infants born by ART, especially for premature male ART infants. These results also suggest the probability of fixation condition and visual deficiencies in these infants. It is recommended to pay close attention to this preliminary report about the refractive and fixation condition of the infants born after ART.

Jafarzadehpur, Ebrahim; Mohhamadi, Ali Reza; Nateghi, Mohammad Reza; Fazeli, Abolhasan Shahzade; Kashi, Khashayar Mehdizadeh

2013-01-01

368

Diverse Manifestations of Convective Upwelling Beneath the North Atlantic Ocean  

NASA Astrophysics Data System (ADS)

The Icelandic Plume dominates the North Atlantic Ocean. Residual depth anomalies of oceanic lithosphere, long wavelength gravity anomalies, and seismic tomographic models show that this large upwelling reaches from Baffin Bay to Western Norway, and from offshore Newfoundland to Spitzbergen. At continental margins, there is excellent evidence for present-day dynamic support of crust beneath Scotland and Western Norway. It is generally agreed that the Icelandic Plume started at 62 Ma. In recent years, a quantitative understanding of the temporal evolution of this upwelling has begun to emerge. The best evidence occurs in the oceanic basins north and south of Iceland. Since the mid-oceanic ridge straddles the plume, it acts as a linear sampler of transient activity over the last 40-50 Ma. A pair of seismic reflection flowlines acquired in 2010 have enabled us to determine the detailed history of transient activity. The implications of this history are profound. Waxing and waning of convective upwelling beneath this important oceanic gateway appears to have modulated the overflow of the ancient precursor to North Atlantic Deep Water (NADW). The growth of contourite drifts which plaster deep-water margins can also be directly linked to changing vertical motions at this gateway. Finally, there is increasing evidence that the otherwise uniform thermal subsidence of sedimentary basins, which fringe both sides of the North Atlantic Ocean, has been periodically interrupted by transient uplift events which generated ephemeral landscapes. These geologic manifestations of convective activity should lead to improved insights into the fluid dynamics of the mantle.

White, Nicky; Parnell-Turner, Ross

2013-04-01

369

Peripancreatic lymphoadenopathy and extrahepatic immunological manifestations in chronic hepatitis C.  

PubMed

The aim of the study was to determine the role of peripancreatic lymph node swelling in systemic immunological alterations during chronic hepatitis C (HC). The prospective study was carried out as a clinical study in a university hospital. Clinical, haematochemical and ultrasonographic findings in 182 patients were studied. Ultrasonography was performed by the same operator and the findings were evaluated blind without the operator knowing the clinical and haematochemical parameters. Hepatitis B virus (HBV) markers, anti-HCV antibodies, LKM1, cryoglobulinaemia, rheumatoid factor and anti-tissue antibodies were determined. Liver biopsy was carried out in 43 of the 182 patients. One or two pathological peripancreatic lymph nodes (PLNs) were present in 30 of the 182 patients and, of the 30, 28 were anti-HC positive. Only one patient in the non-PLN group was positive for anti-HCV, there being statistical significance (P < 0.0001) between the PLN and non-PLN groups. In HCV-positive patients, extrahepatic immunological manifestations were observed (cryoglobulinaemia; positivity to anti-smooth muscle, antinuclear and antimitochondrial antibodies; positivity to rheumatoid factor and LKM1). In five patients the presence of focal lymphocytic aggregates was detected by biopsy, whereas one patient presented typical ocular lesion of Mikulicz's syndrome. Our results may confirm the marked lymphotropism shown by the HC virus and indicate more complex immune system involvement, especially in view of the coexisting signs of immune system involvement related to the presence of intrahepatic cellular aggregates detected in our study. We believe that the peripancreatic adenopathy in chronic HCV hepatitis is an important diagnostic sign and may indicate an involvement of the C virus in the still unexplained extrahepatic immunological disorders. PMID:8872061

Neri, S; Bruno, C M; D'Angelo, G; Russo, S

1996-08-01

370

[Osteoarticular pains as early manifestation of malignancies in children].  

PubMed

Based on the rewiew of the literature and own clinical observations, we presented examples of the most common pediatric onco-hematologic malignancies, that are manifested early by osteoarticular complaints. When these complaints predominate in the clinical presentation, they lead the diagnosis towards nonmalignant conditions, that are most common cause of such symptoms in children, like injuries, nonspecific reactive arthritis or inflammatory connective tissue diseases. However, in acute lymphoblastic leukaemia, the most prevalent childhood malignancy, bone and joint pains are present early in 40-60% of cases and they frequently anticipate any abnormalities in complete blood counts. Findings reported in the literature and own observations indicate that these complaints correlate with: lower white cell counts, lower percentage of blast cells in the peripheral blood and lower incidence of organomegaly - that may delay the decision of bone marrow aspiration. In our study we have also presented 4 cases of other malignancies in children, who were complaining of the osteoarticular pains, that limited they activity, long time before the beginning of treatment in Department of Oncology and Pediatric Hematology of University's Pediatric Hospital in Cracow. Long-lasting and intensive osteoarticular complains, that restraint normal activity and do not resolve during rest, spinal compression symptoms, coexisting adenopathy, hepatosplenomegaly, weight loss, change of behaviour, unexplicained fever must be recognized as specific "red flags". Oncologic vigilance must be inspired by discreet hematological abnormalites (like increased anemia, lower white cell counts with lymphocytosis, mild thrombocytopenia) -that indicate bone marrow infiltration, as well as high erythrocyte sedimentation rate, accompanied by moderately elevated C-reactive protein - characteristic for malignancy. Basic and commonly accessible radiological imaging may provide valuable information, because it can reveal tumors, osteolytic lesions or destruction of bone architecture. Laboratory tests of lactate dehydrogenase (LDH) and uric acid level, often raised in malignancies are also helpful. The aim of this study was to focus the attention of pediatricians to the necessity of including malignancy in the differential diagnosis of intensive or unexplained osteoarticular complaints. PMID:19485254

Cwikli?ska, Magdalena; Balwierz, Walentyna

2009-01-01

371

Clinical manifestations of CNS infections caused by enterovirus type 71  

PubMed Central

Purpose Enterovirus 71, one of the enteroviruses that are responsible for both hand-foot-and-mouth disease and herpangina, can cause neural injury. During periods of endemic spread of hand-foot-andmouth disease caused by enterovirus 71, CNS infections are also frequently diagnosed and may lead to increased complications from neural injury, as well as death. We present the results of our epidemiologic research on the clinical manifestations of children with CNS infections caused by enterovirus 71. Methods The study group consisted of 42 patients admitted for CNS infection by enterovirus 71 between April 2009 and October 2009 at the Department of Pediatrics of 5 major hospitals affiliated with the Catholic University of Korea. We retrospectively reviewed initial symptoms and laboratory findings on admission, the specimen from which enterovirus 71 was isolated, fever duration, admission period, treatment and progress, and complications. We compared aseptic meningitis patients with encephalitis patients. Results Of the 42 patients (23 men, 19 women), hand-foot-and-mouth disease was most prevalent (n=39), followed by herpangina (n=3), upon initial clinical diagnosis. Among the 42 patients, 15 (35.7%) were classified as severe, while 27 (64.3%) were classified as mild. Factors such as age, fever duration, presence of seizure, and use of intravenous immunoglobulin (IVIG) were statistically different between the 2 groups. Conclusion Our results indicate that patients with severe infection caused by enterovirus 71 tended to be less than 3 years old, presented with at least 3 days of fever as well as seizure activity, and received IVIG treatment.

Choi, Cheol Soon; Choi, Yun Jung; Choi, Ui Yoon; Han, Ji Whan; Jeong, Dae Chul; Kim, Hyun Hee; Kim, Jong Hyun

2011-01-01

372

Oral Manifestations in HIV/AIDS-Infected Children  

PubMed Central

Objectives: To assess factors influencing the distribution of oral manifestations in HIV/AIDS-infected children attending the Paediatric Infectious Disease Clinic in Mulago Hospital, Kampala. Methods: This was a cross-sectional study comprising 237 children (males/females: 113/124) aged 1 to 12 years. The parents/guardians were interviewed to obtain demographic information, oral hygiene practices, dietary habits and health seeking behaviours as well as any medications taken. The children were clinically examined for oral lesions based on World Health Organization criteria with modifications. Results: About 71.7% of the children cleaned their teeth. About 16.9% of the children had visited a dentist since birth, mainly for emergency care. One or more oral lesions were recorded in 73% of the children of whom 19.0% experienced discomfort during oral functions. Cervical lymphadenopathy, oral candidiasis and gingivitis were the most common soft tissue oral lesions: 60.8%, 28.3% and 19.0%, respectively. Except for dental caries, the overall frequency distribution of soft tissue oral lesions was significantly lower in children on highly active antiretroviral therapy (HAART) as compared to their counterparts not on HAART. The prevalence of dental caries in deciduous and permanent dentitions was 42.2% and 11.0%, respectively. Tooth brushing and previous visits to the dentist were indirectly and significantly associated with dental caries. About 5.9% (n=14) of the children had <200 CD3 + CD4 T-lymphocyte cells per ?l of blood. Conclusions: The majority of the children had one or more oral lesions, particularly in the group not on HAART. Some of the lesions were associated with discomfort during oral functions.

Rwenyonyi, Charles Mugisha; Kutesa, Annet; Muwazi, Louis; Okullo, Isaac; Kasangaki, Arabat; Kekitinwa, Addy

2011-01-01

373

First report of HIV-related oral manifestations in Mali  

PubMed Central

Introduction In 2004, the sudden availability of free antiretroviral therapy (ART in Mali, within the context of an already overburdened health care system created gaps in individual patient quality of care. The objective of this study was to determine the prevalence of HIV-related oral manifestations (OM) during the first month of ART therapy in a Malian health facility. Methods Medical records of adult patients who initiated ART regimens at the Gabriel Touré Hospital, Mali (2001 to 2008) were randomly identified. Multiple logistic regression models were used to evaluate the relationship between the presence of OM during the first month of ART and selected variables, including CD4 counts and WHO clinical staging at ART initiation. Results Out of 205 patients on ART (mean age 39 ± 10 years), 71.0% were females and 36.1% had no formal education. 40.6% were in WHO clinical stage III. OM prevalence during the first month of HIV care was 31.4%, being oral candidiasis the commonest lesion. 73.2% and 82.5% of the patients with OM had CD4 count < 200 cells/mm3 and were classified as WHO clinical stage III or IV. WHO clinical stage III and VI patients had 5.4-fold increased odds of having any OM (both p< 0.01) when controlling for age, ethnicity, gender, marital status, and CD4 counts. Conclusion OM detected in people with low CD4 count and WHO clinical stage III and IV at ART initiation suggested that they were very immune-compromised when initiating HIV care. Early identification of OM could improve the quality of care and guarantee the benefits of ART.

Tami-Maury, Irene; Coulibaly, Yaya Ibrahim; Cissoko, Souare Salimata; Dao, Sounkalo; Kristensen, Sibylle

2012-01-01

374

Constrictive pleuropericarditis: a dominant clinical manifestation in Whipple's disease  

PubMed Central

Background Whipple’s disease is a rare, multisystemic, chronic infectious disease which classically presents as a wasting illness characterized by polyarthralgia, diarrhea, fever, and lymphadenopathy. Pleuropericardial involvement is a common pathologic finding in patients with Whipple’s disease, but rarely causes clinical symptoms. We report the first case of severe fibrosing pleuropericarditis necessitating pleural decortication in a patient with Whipple’s disease. Case presentation Our patient, an elderly gentleman, had a chronic inflammatory illness dominated by constrictive pericarditis and later severe fibrosing pleuritis associated with a mildly elevated serum IgG4 level. A pericardial biopsy showed dense fibrosis without IgG4 plasmacytic infiltration. The patient received immunosuppressive therapy for possible IgG4-related disease. His poor response to this therapy prompted a re-examination of the diagnosis, including a request for the pericardial biopsy tissue to be stained for Tropheryma whipplei. Conclusions Despite a high prevalence of pleuropericardial involvement in Whipple’s disease, constrictive pleuropericarditis is rare, particularly as the dominant disease manifestation. The diagnosis of Whipple’s disease is often delayed in such atypical presentations since the etiologic agent, Tropheryma whipplei, is not routinely sought in histopathology specimens of pleura or pericardium. A diagnosis of Whipple’s disease should be considered in middle-aged or elderly men with polyarthralgia and constrictive pericarditis, even in the absence of gastrointestinal symptoms. Although Tropheryma whipplei PCR has limited sensitivity and specificity, especially in the analysis of peripheral blood samples, it may have diagnostic value in inflammatory disorders of uncertain etiology, including cases of polyserositis. The optimal approach to managing constrictive pericarditis in patients with Whipple’s disease is uncertain, but limited clinical experience suggests that a combination of pericardiectomy and antibiotic therapy is of benefit.

2013-01-01

375

Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt  

Microsoft Academic Search

Ocular manifestations resulting from Rift Valley fever (RVF) virus infection were studied during an extensive RVF epidemic in Egypt during 1977. Colour photography and fluorescein angiography of 7 serologically diagnosed patients showed the commonest manifestations to be macular, paramacular, and\\/or extramacular retinal lesions, often occurring bilaterally. Haemorrhage and oedema were frequently associated with the lesions, and vasculitis, retinitis, and vascular

A L Siam; J M Meegan; K F Gharbawi

1980-01-01

376

75 FR 60772 - Agency Information Collection Activities: Transportation Entry and Manifest of Goods Subject to...  

Federal Register 2010, 2011, 2012, 2013

...and Manifest of Goods Subject to CBP Inspection and...with a change to the burden hours. This document...components estimate of the burden of The proposed collection...and (4) Minimize the burden of the collections of...and Manifest of Goods Subject to CBP Inspection...

2010-10-01

377

78 FR 76152 - Agency Information Collection Activities: Transportation Entry and Manifest of Goods Subject to...  

Federal Register 2010, 2011, 2012, 2013

...and Manifest of Goods Subject to CBP Inspection and...with no change to the burden hours. This document...components estimate of the burden of the proposed collection...and (4) Minimize the burden of the collections of...and Manifest of Goods Subject to CBP Inspection...

2013-12-16

378

Prevalence of rheumatic manifestations and antineutrophil cytoplasmic antibodies in haematological malignancies. A prospective study  

Microsoft Academic Search

Objective. To evaluate the prevalence of antineutrophil cytoplasmic antibodies (ANCA) and rheumatic manifestations associated with chronic haematological malignancies. Methods. Two groups of patients were prospectively studied (group I: 60 patients with myelodysplastic syndromes and group II: 140 patients with lymphoid malignancies) for clinical 'immune' manifestations and ANCA. Results. In the myelodysplastic group, six patients had ANCA-negative systemic medium- size vasculitis,

M. A. Hamidou; S. Derenne; M. A. P. Audrain; J. M. Berthelot; A. Boumalassa; J. Y. Grolleau

2000-01-01

379

Framing Space: A Popular Geopolitics of American Manifest Destiny in Outer Space  

Microsoft Academic Search

This paper examines how ‘ways of seeing’ landscape, as practised within the little-known American astronomical art community, can be used to examine the popular geopolitical scripting of an American manifest destiny in outer space. A significant body of work in critical geopolitics has sought to recognise the way in which culturally manifest representations of space and place, together with embedded

Daniel Sage

2008-01-01

380

Right Bundle Branch Block: An Uncommon Cardiotoxic Manifestation of Hair Dye Poisoning-A Case Report  

PubMed Central

Hair dye poisoning has been rising in incidence in the recent years. Apart from the commoner manifestations of upper airway edema, rhabdomyolysis and acute renal failure, cardiac toxicity, convulsions and sudden cardiac death are relatively rare complications. We discuss a case of hair dye poisoning manifesting as oropharyngeal edema along with cardiac complication. The patient survived.

Balasubramanian, Deepak; Subramanian, Saravanan; Thangaraju, Pugazhenthan; Shanmugam, Kani

2014-01-01

381

Guillain-Barr? syndrome as an extraintestinal manifestation of Crohn's disease.  

PubMed Central

A variety of extraintestinal manifestations, probably immune-mediated, may appear during relapses of Crohn's disease. We report the clinical observation of a 34-year-old woman who developed a Guillain-Barré syndrome, aphthous stomatitis and oligoarthritis during a relapse of Crohn's ileocolitis. This case suggests that the Guillain-Barré syndrome may be another extraintestinal manifestation of Crohn's disease.

de la Fuente-Fernandez, R.; Rubio-Nazabal, E.; de la Iglesia-Martinez, F.

1995-01-01

382

Right bundle branch block: an uncommon cardiotoxic manifestation of hair dye poisoning-a case report.  

PubMed

Hair dye poisoning has been rising in incidence in the recent years. Apart from the commoner manifestations of upper airway edema, rhabdomyolysis and acute renal failure, cardiac toxicity, convulsions and sudden cardiac death are relatively rare complications. We discuss a case of hair dye poisoning manifesting as oropharyngeal edema along with cardiac complication. The patient survived. PMID:24596762

Balasubramanian, Deepak; Subramanian, Saravanan; Thangaraju, Pugazhenthan; Shanmugam, Kani

2014-01-01

383

On the Misuse of Manifest Variables in the Detection of Measurement Bias.  

ERIC Educational Resources Information Center

A unified treatment is presented for conditions that should allow detection of measurement bias using statistical procedures involving only observed or manifest variables. Computational results demonstrate that methods for studying bias that rely exclusively on manifest variables are not generally diagnostic of the presence or absence of…

Meredith, William; Millsap, Roger E.

1992-01-01

384

19 CFR 122.73 - General declaration and air cargo manifest.  

Code of Federal Regulations, 2011 CFR

...2014-04-01 false General declaration and air cargo manifest. 122.73 Section 122...HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required...States § 122.73 General declaration and air cargo manifest. (a) General...

2014-04-01

385

19 CFR 4.7b - Electronic passenger and crew arrival manifests.  

Code of Federal Regulations, 2010 CFR

...false Electronic passenger and crew arrival manifests. 4.7b Section 4.7b...VESSELS IN FOREIGN AND DOMESTIC TRADES Arrival and Entry of Vessels § 4.7b Electronic passenger and crew arrival manifests. (a) Definitions....

2009-04-01

386

19 CFR 4.7b - Electronic passenger and crew arrival manifests.  

Code of Federal Regulations, 2010 CFR

...false Electronic passenger and crew arrival manifests. 4.7b Section 4.7b...VESSELS IN FOREIGN AND DOMESTIC TRADES Arrival and Entry of Vessels § 4.7b Electronic passenger and crew arrival manifests. (a) Definitions....

2010-04-01

387

Generalized granuloma annulare as an initial manifestation of chronic myelomonocytic leukemia: a report of 2 cases.  

PubMed

Granuloma annulare is a dermatologic condition of unknown etiology that has been associated with systemic diseases and reported to be a paraneoplastic manifestation. Two patients with generalized granuloma annulare as an initial manifestation of chronic myelomonocytic leukemia are herein described. We suggest that chronic myelomonocytic leukemia should be added to the list of systemic diseases associated with generalized granuloma annulare. PMID:18496432

Hinckley, Michael R; Walsh, Sarah N; Molnár, István; Sheehan, Daniel J; Sangueza, Omar P; Yosipovitch, Gil

2008-06-01

388

Gastrointestinal Manifestations of Multiple Endocrine Neoplasia Type 2  

PubMed Central

Objective To determine the clinical features, natural history, and role of surgery for gastrointestinal manifestations of the multiple endocrine neoplasia type 2 (MEN 2) syndromes. Summary Background Data The MEN 2 syndromes are characterized by medullary thyroid carcinoma and other endocrinopathies. In addition, some patients with MEN 2A develop Hirschsprung’s disease (HD), and all patients with MEN 2B have intestinal neuromas and megacolon that can cause significant gastrointestinal problems. Methods From 83 families with MEN 2A, eight patients with HD were identified (MEN 2A-HD). These and all patients with MEN 2B followed at the authors’ institution (n = 53) were sent questionnaires to describe the onset and type of gastrointestinal symptoms and treatment they had before the diagnosis of MEN 2. Records of all patients responding were reviewed, including radiographic imaging, histology, surgical records, and genetic testing. Results Thirty-six of the 61 patients (59%) responded (MEN 2A = 8, MEN 2B = 28) to the questionnaires. All patients with MEN 2A-HD were operated on for HD 2 to 63 years before being diagnosed with MEN 2. All patients responding were underweight as infants and had symptoms of abdominal pain, distention, and constipation. Eighty-eight percent had hematochezia, 63% had emesis, and 33% had intermittent diarrhea before surgery. All patients with MEN 2A-HD had rectal biopsies with a diverting colostomy as the initial surgical procedure. This was followed by a colostomy takedown and pull-through procedure at a later interval. Ninety-three percent of patients with MEN 2B had gastrointestinal symptoms 1 to 24 years before the diagnosis of MEN 2. Symptoms included flatulence (86%), abdominal distention or being underweight as a child (64%), abdominal pain (54%), constipation or diarrhea (43%), difficulty swallowing (39%), and vomiting (14%). Seventy-one percent of patients with MEN-2B with gastrointestinal symptoms had radiographic imaging, 32% were admitted to the hospital, and 29% underwent surgery. Conclusions Patients with MEN 2A-HD had a typical HD presentation and always required surgery. Patients with MEN 2B have significant gastrointestinal symptoms, but less than a third had surgical intervention. Understanding the clinical course and differences in these patients will improve clinical management.

Cohen, Mark S.; Phay, John E.; Albinson, Charlotte; DeBenedetti, Mary K.; Skinner, Michael A.; Lairmore, Terry C.; Doherty, Gerard M.; Balfe, Dennis M.; Wells, Samuel A.; Moley, Jeffrey F.

2002-01-01

389

The cutaneous manifestations of human parvovirus B19 infection.  

PubMed

The prototypical cutaneous manifestations of human parvovirus B19 (B19) infection include a petechial eruption in a glove and stocking distribution, reticular truncal erythema, and the "slapped cheek" sign. An association with connective tissue disease (CTD) stigmata has recently been made. The clinical and dermatopathologic findings in 14 patients whose skin lesions were accompanied by serological evidence of B19 infection or documentation of B19 genome in lesional skin are presented. The authors encountered skin biopsy specimens from 14 patients who presented with skin eruptions accompanied by clinical signs or serology suggestive of antecedent B19 infection. Clinical findings were correlated to the light microscopic appearance of the lesions and the presence of B19 genome in lesional skin. The study group comprised 9 women, 3 men, and 2 boys. Eruptions characteristic of fifth disease, including the slapped cheek sign, reticulated truncal erythema, and acral petechiae, were present in 3 patients, 1 of whom later developed granuloma annulare. The other patients had atypical clinical presentations comprising an asymptomatic papular eruption (2), an eruption clinically resembling Sweet's syndrome (3), myopathic dermatomyositis (DM) (2), lupus erythematosus (LE)-like syndromes (2), and lower-extremity palpable purpura (2). Skin biopsy specimens in 12 cases showed interstitial histiocytic infiltrates with piecemeal fragmentation of collagen and a mononuclear cell-predominant vascular injury pattern. Other features included an interface dermatitis, eczematous alterations, and papillary dermal edema. Lesions with features of DM or LE also showed mesenchymal mucinosis, whereas a biopsied lesion of palpable purpura showed leukocytoclastic vasculitis (LCV). Immunofluorescent testing showed a positive lupus band test (LBT) with epidermal IgG and C5b-9 decoration in 1 patient with a systemic LE-like illness, whereas the DM patients had negative LBTs and vascular C5b-9 deposition typical for DM. Skin biopsy specimens from 11 patients, including those whose presentations resembled LE and DM, were positive for B19 genome. The dermatopathology of B19 infection suggests tissue injury mediated by delayed-type hypersensitivity, by antibody-dependent cellular immunity directed at microbial antigenic targets in the epidermis and endothelium, and by circulating immune complexes in the setting of LCV. These mechanisms appear to generate a clinical and histopathological picture that recapitulates that of CTD. PMID:10821497

Magro, C M; Dawood, M R; Crowson, A N

2000-04-01

390

CT manifestations of adrenal trauma: experience with 73 cases.  

PubMed

Adrenal injuries, although an uncommon consequence of abdominal trauma, are important to recognize. If bilateral, adrenal trauma could result in life-threatening adrenal insufficiency. Furthermore, in the setting of trauma, adrenal injury can point to other concomitant injuries and has been associated with overall increased morbidity and mortality. In the past, before the advent of computed tomography (CT), detection was difficult, and the diagnosis was often made only at surgery or postmortem. Today, the diagnosis of adrenal injuries can be quickly and accurately made with CT. This retrospective review was carried out to identify, describe, and analyze different CT appearances of adrenal injuries and correlated with associated injuries and observed clinical context and outcomes. A patient cohort of CT-detected adrenal injuries was identified through a radiology software research tool by searching for keywords in radiology reports. The identified CT scans were reviewed and correlated with the patients' available clinical chart data and follow-up. Between April 1995 and October 2004, 73 cases of CT-detected adrenal injuries were identified, including 48 men and 25 women, with an age range 6 to 90 years and a mean age of 42.7 years. Of the cases, 77% were right-sided, 15% were left-sided, and 8% were bilateral. The causes of injuries were motor vehicle collisions (75%), falls (14%), sports related (4%), and miscellaneous causes (7%). Associated trauma included injuries of the liver (43%), spleen (23%), lung (19%), and kidney (18%), as well as pneumothoraces/hemothoraces (22%). Skeletal injuries included fractures of the ribs, clavicles, and/or scapulae (39%), pelvis and hips (30%), and the spine (23%). Isolated adrenal trauma was seen in only 4% of the cases. The CT findings of adrenal trauma were focal hematoma (30%), indistinct (27%) or enlarged (18%) adrenal gland, gross (15%) or focal (7%) adrenal hemorrhage, and adrenal mass (11%). Associated CT findings included periadrenal fat stranding (93%), retroperitoneal hemorrhage (22%), and thickened diaphragmatic crura (10%). Active adrenal bleeding was seen in one case (1.4%). The incidence of adrenal trauma was estimated to be 0.86%. Surgical management was required only for the associated injuries. The most common CT manifestations of adrenal trauma include focal hematoma, indistinct or ill-defined adrenal gland, adrenal enlargement or mass, and gross or focal adrenal hemorrhage in a normal-sized gland. Periadrenal stranding is very common. Retroperitoneal hemorrhage and crural thickening are also important associated findings. Operative intervention is typically required only for the associated injuries, which commonly accompany adrenal trauma. PMID:17252249

Sinelnikov, Alex O; Abujudeh, Hani H; Chan, David; Novelline, Robert A

2007-03-01

391

Sex-Specific Manifestations of L?fgren's Syndrome  

PubMed Central

Motivation: It has been debated whether patients need to have erythema nodosum to be classified as having Löfgren's syndrome. In this study, we have therefore in detail evaluated and compared a large number of patients with an acute onset of sarcoidosis and bilateral hilar lymphadenopathy (BHL), with or without erythema nodosum (EN). This study is important because it may lead to a more accurate definition of Löfgren's syndrome, and an exact phenotype of patients is crucial in modern medical research. Background: Löfgren's syndrome is commonly regarded as a distinct clinical entity. Methods: We have in detail evaluated a large group of patients (n = 150) with an acute onset of sarcoidosis with BHL, in most cases with fever, EN, and/or bilateral ankle arthritis or periarticular inflammation. Within this group, 87 patients had EN (EN positive), whereas 63 were without EN (EN negative), though with distinct symmetric ankle inflammation. Results: EN-positive and EN-negative patients were identical in every aspect except that there were significantly more women in the EN-positive group: 58 women (67%) in the EN-positive group compared with only 17 (27%) women in the EN-negative group (p < 0.0001). In all other aspects, such as age, smoking habits, seasonal clustering of disease onset, rate of positive biopsies, chest radiography, pulmonary function, bronchoalveolar lavage cell distributions including the typically increased CD4/CD8 ratio, and clinical development of the disease, the EN-positive and EN-negative groups were close to identical. The two groups were also identically strongly associated with HLA-DRB1*0301/DQB1*0201, with 60 (69.0%) and 44 (69.8%) patients having this particular HLA type in the EN-positive and EN-negative groups, respectively. Such patients recovered to the same degree—that is, at almost 100%. Conclusions: We conclude that manifestations of Löfgren's syndrome differ between men and women, with EN found predominantly in women, whereas a marked periarticular inflammation of the ankles or ankle arthritis without EN is seen preferentially in men.

Grunewald, Johan; Eklund, Anders

2007-01-01

392

Severe Acute Respiratory Syndrome: Clinical and Laboratory Manifestations  

PubMed Central

Severe acute respiratory syndrome (SARS) is a recently emerged infectious disease with significant morbidity and mortality. An epidemic in 2003 affected 8,098 patients in 29 countries with 774 deaths. The aetiological agent is a new coronavirus spread by droplet transmission. Clinical and general laboratory manifestations included fever, chills, rigor, myalgia, malaise, diarrhoea, cough, dyspnoea, pneumonia, lymphopenia, neutrophilia, thrombocytopenia, and elevated serum lactate dehydrogenase (LD), alanine aminotransferase (ALT) and creatine kinase (CK) activities. Treatment has been empirical; initial potent antibiotic cover, followed by simultaneous ribavirin and corticosteroids, with or without pulse high-dose methylprednisolone, have been used. The postulated disease progression comprises (1) active viral infection, (2) hyperactive immune response, and (3) recovery or pulmonary destruction and death. We investigated serum LD isoenzymes and blood lymphocyte subsets of SARS patients, and found LD1 activity as the best biochemical prognostic indicator for death, while CD3+, CD4+, CD8+ and natural killer cell counts were promising predictors for intensive care unit (ICU) admission. Plasma cytokine and chemokine profiles showed markedly elevated Th1 cytokine interferon (IFN)-?, inflammatory cytokines interleukin (IL)-1?, IL-6 and IL-12, neutrophil chemokine IL-8, monocyte chemoattractant protein-1 (MCP-1), and Th1 chemokine IFN-?-inducible protein-10 (IP-10) for at least two weeks after disease onset, but there was no significant elevation of inflammatory cytokine tumor necrosis factor (TNF)-? and anti-inflammatory cytokine IL-10. Corticosteroid reduced IL-8, MCP-1 and IP-10 concentrations from 5–8 days after treatment. Measurement of biochemical markers of bone metabolism demonstrated significant but transient increase in bone resorption from Day 28–44 after onset of fever, when pulse steroid was most frequently given. With tapering down of steroid therapy, there was a decrease in bone resorption marker together with an increase in bone formation markers round Day 50, suggesting that some of the bone loss might be reversed. Our research studies on the chemical pathology and clinical immunology of SARS should have implications for the pathophysiology and therapy of this potentially lethal infection.

Lam, Christopher W K; Chan, Michael H M; Wong, Chun K

2004-01-01

393

Rapid onset asthma: a severe but uncommon manifestation  

PubMed Central

BACKGROUND—Studies of asthma death and severe life threatening asthma (SLTA) include reports of patients who had rapid onset asthma. A study was undertaken to determine the relative frequency of rapid (<6 hours duration) and slow (?6 hours) onset attacks in patients admitted to hospital with acute severe asthma, and to establish whether those with rapid onset asthma differ in terms of risk factors for asthma morbidity and mortality such as indices of asthma severity/control, socioeconomic factors, health care, and psychological factors.?METHODS—A cross sectional study was performed on 316 patients aged 15-49 years admitted with acute severe asthma and interviewed within 24-48 hours of admission.?RESULTS—Patients underestimated the duration of the index attack. Only 27 (8.5%) were classified as rapid onset. There were more men in the rapid onset group than in the slow onset group (52% versus 26%), and there was evidence of socioeconomic advantage in the patients with rapid onset attacks. The rapid onset group had more previous episodes of SLTA and were more likely to present with SLTA, but there was no difference in length of stay in hospital. The rapid onset group were less likely to have presented to a GP during the index attack and were more likely to have used ambulance services. There was no difference between the groups in any psychological or health care measure.?CONCLUSIONS—Rapid onset attacks are an important but uncommon manifestation of asthma that are more likely to present with SLTA in patients who are more likely to have had previous SLTA. Male subjects are at increased risk of rapid onset attacks, and socioeconomic disadvantage, deficiencies in health care (ongoing and acute), and psychological factors are no more common in these patients than in those with attacks of slow onset. These data are consistent with the hypothesis that there is a small proportion of patients with rapid onset severe asthma who do not have the usual risk factors associated with asthma morbidity or mortality, and thus require different management strategies.??

Kolbe, J; Fergusson, W; Garrett, J

1998-01-01

394

Experience with Extrarenal Manifestations of Hemorrhagic Fever with Renal Syndrome in a Tertiary Care Hospital in South Korea  

PubMed Central

Reports on the clinical entity of hemorrhagic fever with renal syndrome (HFRS) have focused on acute renal failure. Data on the extrarenal manifestations are limited primarily to case reports. In this study, protean extrarenal manifestations involving the major organs occurred in one-third of patients with HFRS during various stages (i.e., febrile phase through diuretic phase). Pancreatobiliary manifestations and major bleeding occurred in 11% and 10% of patients, respectively. Cardiovascular and central nervous system manifestations developed during the febrile or oliguric phase, whereas pancreatobiliary manifestations and major bleeding were detected even in the diuretic phase. Thus, close monitoring of and additional knowledge about various extrarenal manifestations are needed.

Park, Kyung Hwa; Kang, Yong Un; Kang, Seung-Ji; Jung, Young-Sun; Jang, Hee-Chang; Jung, Sook-In

2011-01-01

395

Clinical and Pathological Manifestations with Differential Diagnosis in Beh?et's Disease  

PubMed Central

Behçet's disease is a multisystemic inflammatory disease of unknown etiology which usually occurs as a trait of symptoms: aphthous stomatitis, genital ulcerations, and ocular disease. At the beginning of the disease the diagnosis is uncertain because of various clinical manifestations and a long period up to the full clinical picture manifestation. Since neither the laboratory data nor the histopathological signs are truly pathognomonic in Behçet's disease, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Sometimes, some laboratory test may help establish the diagnosis. Subspecialty referral to ophthalmology, rheumatology, neurology, and gastroenterology should be considered when indicated.

Kokturk, Aysin

2012-01-01

396

Oral Manifestations of Tropical Infectious Diseases of Central and South America. Part I. Protozoan and Helminthic Infections.  

National Technical Information Service (NTIS)

Oral manifestations of tropical infectious diseases are seldom discussed extensively in most current textbooks of oral diagnosis or oral pathology. This paper is the result of a survey of literature in which the oral manifestations of infectious diseases ...

T. Zislis J. C. Adrian D. E. Cutright

1979-01-01

397

20 CFR 702.604 - Determining the amount of compensation for occupational disease claims which become manifest...  

Code of Federal Regulations, 2013 CFR

...amount of compensation for occupational disease claims which become manifest after retirement...ADMINISTRATION AND PROCEDURE Occupational Disease Which Does Not Immediately Result in Death...amount of compensation for occupational disease claims which become manifest after...

2013-04-01

398

20 CFR 702.603 - Determining the payrate for compensating occupational disease claims which become manifest after...  

Code of Federal Regulations, 2013 CFR

...the payrate for compensating occupational disease claims which become manifest after retirement...ADMINISTRATION AND PROCEDURE Occupational Disease Which Does Not Immediately Result in Death...the payrate for compensating occupational disease claims which become manifest after...

2013-04-01

399

Oral Manifestations of Tropical Infectious Diseases of Central and South America. Part II. Bacterial and Mycotic Infections.  

National Technical Information Service (NTIS)

Oral manifestations of tropical infectious diseases are seldom discussed extensively in most current textbooks of oral diagnosis or oral pathology. This paper is the result of a survey of literature in which the oral manifestations of infectious diseases ...

T. Zislis J. C. Adrian D. E. Cutright

1979-01-01

400

Endemic Diseases with Associated Skin Manifestations - 20 Years Ago and Now. (Reannouncement with New Availability Information).  

National Technical Information Service (NTIS)

Twenty years ago, some various endemic diseases in Egypt produced distinctive skin manifestations. Pallor was due to blood loss, from hookworm infection or from schistosomal colonic polyposis was prevalent. A severe rash on sun exposed skin was seen with ...

N. A. El Masry S. Bassily Z. Farid

1991-01-01

401

Spectrum of neurological manifestations in dengue virus infection in Northwest India.  

PubMed

The objective of this study was to study the spectrum of neurological manifestations in patients with dengue infection. This was a prospective study undertaken at the Departments of Medicine and Neurology, Christian Medical College, Ludhiana, India. All patients diagnosed with dengue fever during an epidemic (1 September 2010-31 December 2010) were screened for neurological manifestations. There were a total of 799 patients with dengue infection. Neurological manifestations were present in 21 (2.63%), 19 of whom were men with a mean age of 33.7 ± 13.9 years. The neurological diagnoses were hypokalaemia with: quadriparesis (7); myositis (4); encephalopathy (4); Guillain-Barre syndrome (2); acute disseminated encephalomyelitis (2); lumbosacral plexopathy (1); and intracranial haemorrhage (1). Three of these patients died. Clinicians should be aware that neurological manifestations in dengue fever are not uncommon. PMID:23405004

Koshy, Jency Maria; Joseph, Deepa Mary; John, Mary; Mani, Anna; Malhotra, Nitin; Abraham, George M; Pandian, Jeyaraj

2012-10-01

402

Investigating Factorial Invariance of Latent Variables Across Populations When Manifest Variables Are Missing Completely  

PubMed Central

Difficulties arise in multiple-group evaluations of factorial invariance if particular manifest variables are missing completely in certain groups. Ad hoc analytic alternatives can be used in such situations (e.g., deleting manifest variables), but some common approaches, such as multiple imputation, are not viable. At least 3 solutions to this problem are viable: analyzing differing sets of variables across groups, using pattern mixture approaches, and a new method using random number generation. The latter solution, proposed in this article, is to generate pseudo-random normal deviates for all observations for manifest variables that are missing completely in a given sample and then to specify multiple-group models in a way that respects the random nature of these values. An empirical example is presented in detail comparing the 3 approaches. The proposed solution can enable quantitative comparisons at the latent variable level between groups using programs that require the same number of manifest variables in each group.

Widaman, Keith F.; Grimm, Kevin J.; Early, Dawnte R.; Robins, Richard W.; Conger, Rand D.

2013-01-01

403

Clinical overview and treatment options for non-skeletal manifestations of mucopolysaccharidosis type IVA.  

PubMed

Mucopolysaccharidosis type IVA (MPS IVA) or Morquio syndrome is a multisystem disorder caused by galactosamine-6-sulfatase deficiency. Skeletal manifestations, including short stature, skeletal dysplasia, cervical instability, and joint destruction, are known to be associated with this condition. Due to the severity of these skeletal manifestations, the non-skeletal manifestations are frequently overlooked despite their significant contribution to disease progression and impact on quality of life. This review provides detailed information regarding the non-skeletal manifestations and suggests long-term assessment guidelines. The visual, auditory, digestive, cardiovascular, and respiratory systems are addressed and overall quality of life as measured by endurance and other functional abilities is discussed. Impairments such as corneal clouding, astigmatism, glaucoma, hearing loss, hernias, hepatomegaly, dental abnormalities, cardiac valve thickening and regurgitation, obstructive sleep apnea, tracheomalacia, restrictive and obstructive respiratory compromise, and muscular weakness are discussed. Increased awareness of these non-skeletal features is needed to improve patient care. PMID:22358740

Hendriksz, Christian J; Al-Jawad, Maisoon; Berger, Kenneth I; Hawley, Sara M; Lawrence, Rebecca; Mc Ardle, Ciarán; Summers, C Gail; Wright, Elizabeth; Braunlin, Elizabeth

2013-03-01

404

19 CFR 4.7a - Inward manifest; information required; alternative forms.  

...comprising the inward manifest shall be completed as follows: (a) Ship's Stores Declaration. Articles to be retained aboard as sea or ship's stores shall be listed on the Ship's Stores Declaration, CBP Form 1303. Less than whole...

2014-04-01

405

19 CFR 4.75 - Incomplete manifest; incomplete export declarations; bond.  

Code of Federal Regulations, 2013 CFR

...complete outward foreign manifest and all required shipper's export declarations have been filed with the port director: Albania Bulgaria Cambodia China, People's Republic of Cuba Czechoslovakia Estonia German Democratic Republic...

2013-04-01

406

19 CFR 123.9 - Explanation of a discrepancy in a manifest.  

Code of Federal Regulations, 2013 CFR

... U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY CBP RELATIONS WITH CANADA AND MEXICO General Provisions § 123.9 Explanation of a discrepancy in a manifest. (a) Provisions applicable...

2013-04-01

407

Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma.  

PubMed

Various fetal or placental disorders cause Ballantyne's (mirror) syndrome. For the first time, we report a maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma (CMN). In a pregnant woman with a CMN fetus, lung edema, hypertension, hyperthyroidism, and high serum human chorionic gonadotrophin level occurred, all of which characterize maternal manifestation of Ballantyne's syndrome. The fetus and placenta were devoid of 'edema', lacking 'triple edema', and thus this condition was not diagnosed as Ballantyne's syndrome; however, we considered this condition as the maternal manifestation of Ballantyne's syndrome. We performed emergent cesarean section at 28 weeks. Delivery acutely ameliorated maternal symptoms. Tumor was resected and was confirmed as CMN. Maternal manifestations of Ballantyne's syndrome, such as lung edema and hypertension, can occur in a mother with fetal CMN even without fetal and/or placental edema. The clinical course of this patient may suggest an etiology of Ballantyne's syndrome. PMID:24428571

Takahashi, Hironori; Matsubara, Shigeki; Kuwata, Tomoyuki; Ohkuchi, Akihide; Mukoda, Yukiko; Saito, Koyomi; Usui, Rie; Suzuki, Mitsuaki

2014-04-01

408

Oral manifestations in HIV-infected individuals from Thailand and Cambodia  

Microsoft Academic Search

Oral manifestations were studied in 87 HIV-positive Thai adults (study 1), 45 HIV-positive children (study 2) and 101 HIV-positive (study 3). In study 1, 48% of patients had oral lesions; 23% had one and 13.8% two oral manifestations. Oral pseudomembranous candidiasis was found in 10.3%, erythematous candidiasis in 6.9%, and hairy leukoplakia (HL) in 11.5% of the patients. In study

P. A. Reichart; P. Khongkhunthian; C. Bendick

2003-01-01

409

Sudden Sensorineural Hearing Loss as a First Manifestation of Systemic Lupus Erythematosus: Association with Anticardiolipin Antibodies  

Microsoft Academic Search

:   Sudden sensorineural hearing loss is a rarely reported manifestation of systemic lupus erythematosus (SLE). This condition\\u000a has been most frequently seen in individuals with concomitant anticardiolipin antibody (ACL) syndrome, although a direct causal\\u000a relationship remains unconfirmed. We report an unusual case of a young male with sudden unilateral hearing loss as the first\\u000a manifestation of SLE. This individual was

L. Green; E. B. Miller

2001-01-01

410

Genetic and Environmental Influences on Ratings of Manifest Anxiety by Parents and Children  

Microsoft Academic Search

Parental reports and children's self-reports of manifest anxiety were obtained from a community-based sample of twin pairs on two occasions approximately 19 months apart, using the Revised Child Manifest Anxiety Scale (Reynolds & Richmond, 1978). In prior cross-sectional studies, a low degree of agreement between parent and child assessments of anxiety was found. Furthermore, parental reports were found to reflect

Tari D. Topolski; John K. Hewitt; Lindon Eaves; Joanne M. Meyer; Judy L. Silberg; Emily Simonoff; Michael Rutter

1999-01-01

411

Atypical Manifestations of Dengue Fever (DF) - Where Do We Stand Today?  

PubMed

Background and Objectives: Dengue fever (DF) is transmitted by Aedes aegypti mosquitoes. With growing population, rapid urbanization and lack of appropriate sanitary measures, proliferation of mosquitoes and subsequent dengue infections have increased rampantly with an estimated 30-fold increase in incidence over last five decades. With rising disease burden, atypical manifestations have increased as well, which are missed most often due to lack of awareness. Our aim was to look for the atypical manifestations of dengue fever. Materials and Methods: A prospective hospital based observational study was conducted at hospitals of Kasturba Medical College in Mangalore over a period of two years (June-2010 to May-2012). One-hundred fifty ELISA confirmed IgM-dengue sero-positive cases satisfying WHO criteria were examined clinically and laboratory data assessed till they got discharged from hospital after ruling out other causes of fever. Atypical manifestations in dengue fever were noted and analyzed. Results: Most common symptoms noticed were myalgia, headache, rash, arthralgia, pain in abdomen and nausea. More than half of the study group had one or the other atypical manifestation. Liver function test derangement was most often seen. Most common atypical manifestation was hepatitis found in 40.6% patients. Febrile diarrhea, renal failure, Acalculous cholecystitis and conduction abnormalities of heart were among other common manifestations. Three patients died of multi-organ dysfunction, disseminated intravascular coagulation and shock. Platelet count did not correlate well with severity of bleeding. Overall recovery rate was good. Conclusion: Some of the atypical manifestations of dengue fever are no more a rare entity. Clinical vigilance for these manifestations is important for timely detection and management as some of them could be fatal. PMID:24596727

Nimmagadda, Satya Sudhish; Mahabala, Chakrapani; Boloor, Archith; Raghuram, Pavan Manibettu; Nayak U, Akshatha

2014-01-01

412

Extrahepatic Manifestations of Hepatitis C Virus Infection: Mixed Cryoglobulinemia and Beyond  

Microsoft Academic Search

Hepatitis C virus (HCV) is the most common blood-borne infection in the United States and is a leading cause of chronic liver\\u000a disease worldwide. Extrahepatic manifestations of HCV infection occur frequently and are important to recognize as well as\\u000a understand if they improve with antiviral therapy. This review summarizes the extrahepatic manifestations of HCV with particular\\u000a emphasis on their degree

Angelo H. Paredes; Dawn M. Torres

2011-01-01

413

Neonatal lupus syndrome: a case with chondrodysplasia punctata and other unusual manifestations.  

PubMed Central

We report a case of a newborn infant whose mother had systemic lupus erythematosus (SLE) diagnosed before pregnancy. The child had clinical manifestations of neonatal lupus as well as chondrodysplasia punctata and other findings that resemble the congenital anomalies associated with the use of oral anticoagulants, with no history of exposure. We speculate that the combined action of the different maternal autoantibodies may produce the whole spectrum of manifestations. Images

Austin-Ward, E; Castillo, S; Cuchacovich, M; Espinoza, A; Cofre-Beca, J; Gonzalez, S; Solivelles, X; Bloomfield, J

1998-01-01

414

Manifestations of juvenile polyposis syndrome in SMAD4 mutation carriers of a kindred.  

PubMed

The autosomal dominantly inherited juvenile polyposis syndrome (JPS) leads to the development of multiple hamartomatous polyps in the gastrointestinal tract and is a precancerous condition. In a large family with a newly identified SMAD4 mutation (c.543delC), we describe the clinical manifestations of JPS. Nine affected SMAD4 mutation-positive family members were screened and treated for manifestations of JPS. Two family members were symptomatic at the time of diagnosis; seven were asymptomatic - independent of the severity of the manifestation. Each mutation carrier presented with colonic juvenile polyps, seven out of nine with additional gastric manifestations. One asymptomatic patient had early gastric cancer; another patient had a villous adenoma with high-grade intraepithelial neoplasia in the colon. Three patients had biliary lesions including a bile duct hamartoma in one and gallbladder polyps in two. Three patients had gastrointestinal vascular malformations. All mutation carriers were affected by JPS. Interestingly, the manifestations and their severity differed considerably between the patients, suggesting secondary factors influencing JPS manifestations such as Helicobacter pylori infection. PMID:22617360

Schwetz, Verena; Uhrig, Sabine; Spuller, Ekkehard; Deutschmann, Andrea; Högenauer, Christoph

2012-08-01

415

Correlation of Serotype-Specific Dengue Virus Infection with Clinical Manifestations  

PubMed Central

Background Disease caused by the dengue virus (DENV) is a significant cause of morbidity throughout the world. Although prior research has focused on the association of specific DENV serotypes (DENV-1, DENV-2, DENV-3, and DENV-4) with the development of severe outcomes such as dengue hemorrhagic fever and dengue shock syndrome, relatively little work has correlated other clinical manifestations with a particular DENV serotype. The goal of this study was to estimate and compare the prevalence of non-hemorrhagic clinical manifestations of DENV infection by serotype. Methodology and Principal Findings Between the years 2005–2010, individuals with febrile disease from Peru, Bolivia, Ecuador, and Paraguay were enrolled in an outpatient passive surveillance study. Detailed information regarding clinical signs and symptoms, as well as demographic information, was collected. DENV infection was confirmed in patient sera with polyclonal antibodies in a culture-based immunofluorescence assay, and the infecting serotype was determined by serotype-specific monoclonal antibodies. Differences in the prevalence of individual and organ-system manifestations were compared across DENV serotypes. One thousand seven hundred and sixteen individuals were identified as being infected with DENV-1 (39.8%), DENV-2 (4.3%), DENV-3 (41.5%), or DENV-4 (14.4%). When all four DENV serotypes were compared with each other, individuals infected with DENV-3 had a higher prevalence of musculoskeletal and gastrointestinal manifestations, and individuals infected with DENV-4 had a higher prevalence of respiratory and cutaneous manifestations. Conclusions/Significance Specific clinical manifestations, as well as groups of clinical manifestations, are often overrepresented by an individual DENV serotype.

Halsey, Eric S.; Marks, Morgan A.; Gotuzzo, Eduardo; Fiestas, Victor; Suarez, Luis; Vargas, Jorge; Aguayo, Nicolas; Madrid, Cesar; Vimos, Carlos; Kochel, Tadeusz J.; Laguna-Torres, V. Alberto

2012-01-01

416

Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy.  

PubMed

The skin is one of the most common extraintestinal organ system affected in patients with inflammatory bowel disease (IBD), including both Crohn's disease and ulcerative colitis. The skin manifestations associated with IBD are polymorphic and can be classified into 4 categories according to their pathophysiology: (1) specific, (2) reactive, (3) associated, and (4) induced by IBD treatment. Cutaneous manifestations are regarded as specific if they share with IBD the same granulomatous histopathological pattern: perianal or metastatic Crohn's disease (commonly presenting with abscesses, fistulas or hidradenitis suppurativa-like features) is the prototype of this setting. Reactive cutaneous manifestations are different from IBD in the histopathology but have close physiopathological links: pyoderma gangrenosum, a neutrophil-mediated autoinflammatory skin disease typically manifesting as painful ulcers, is the paradigm of this group. Among the cutaneous diseases associated with IBD, the most commonly seen are erythema nodosum, a form of panniculitis most commonly involving bilateral pretibial areas, and psoriasis, a T helper 1/T helper 17-mediated erythematous squamous inflammatory disease. Finally, the number of cutaneous adverse reactions because of IBD therapies is progressively increasing. The most frequent drug-induced cutaneous manifestations are psoriasis-like, eczema-like, and lichenoid eruptions, as well as cutaneous lupus erythematosus for biologics, and nonmelanoma skin cancer, mainly basal cell and squamous cell carcinomas for thiopurines. PMID:24105394

Marzano, Angelo V; Borghi, Alessandro; Stadnicki, Antoni; Crosti, Carlo; Cugno, Massimo

2014-01-01

417

HIV-positive patient with herpes zoster: a manifestation of the immune reconstitution inflammatory syndrome.  

PubMed

Herpes zoster is a common illness that can lead to serious morbidity. There is now evidence that HIV-infected patients who have been treated with antiretroviral therapy are at greater risk of developing herpes zoster not when they are severely immunocompromised but, paradoxically, when their immune system is recovering. This is a manifestation of the immune reconstitution inflammatory syndrome. The objectives of this report are to (1) inform health care providers that HIV-infected patients may develop multiple infectious, autoimmune, and oncological manifestations after treatment with antiretroviral medication, as they have immune system reconstitution, and (2) discuss herpes zoster, one of the possible manifestations. The patient is a 68-year-old HIV-positive man who presented with herpes zoster after being treated with highly active antiretroviral therapy (HAART) when his immune system was recovering, not when he was most immunosuppressed. Emergency department physicians should be aware that HIV-infected patients treated with HAART may have clinical deterioration despite immune system strengthening. This immune reconstitution inflammatory syndrome can present with infectious, autoimmune, or oncological manifestations. Our case patient, an HIV-positive man with immune system recovery after treatment with HAART, presented with an infectious manifestation, herpes zoster. PMID:21129888

Lutwak, Nancy; Dill, Curt

2012-01-01

418

Adult-onset Still's disease and chronic recurrent multifocal osteomyelitis: a hitherto undescribed manifestation of autoinflammation.  

PubMed

Still's disease and chronic recurrent multifocal osteomyelitis (CRMO) are febrile rheumatic diseases of unknown etiology, which predominantly affect children but can also have their initial manifestation in adults. Both can present as intermittent, relapsing episodes and are considered potential candidates within the expanding spectrum of autoinflammatory disorders, although no genetic abnormalities have been described for either of them. Here, we describe a man with an initial manifestation of abacterial multifocal osteitis at the age of 41. During a relapsing-remitting course of his illness, he increasingly developed symptoms of adult-onset Still's disease (AOSD), and the diagnosis was established according to the Yamaguchi criteria. When treated with anakinra, not only the acute symptoms disappeared promptly, but also the osteitis went into complete remission. This is to our knowledge the first description of a simultaneous occurrence of these two manifestations of autoinflammation in adulthood. PMID:21769488

Rech, J; Manger, B; Lang, B; Schett, G; Wilhelm, M; Birkmann, J

2012-06-01

419

Superimposed segmental manifestation of both rare and common cutaneous disorders: a new paradigm.  

PubMed

In autosomal dominant skin disorders, a superimposed mosaic involvement arranged in a linear or otherwise segmental pattern is sometimes noted. Molecular proof of such type 2 segmental manifestation has so far been provided in Hailey-Hailey disease and Cowden syndrome. A similar superimposed segmental involvement can be found in numerous common disorders with a polygenic background, such a psoriasis, lichen planus, or vitiligo. In polygenic diseases, however, we can never recognize with certainty a type 1 segmental manifestation, which is why we should use more neutral terms in the form of isolated versus superimposed segmental involvement. In the near future, the new paradigm of superimposed segmental manifestation may hopefully help elucidate the molecular basis of both monogenic and polygenic skin disorders. PMID:20096200

Happle, R

2009-11-01

420

Posterior segment manifestations of human immunodeficiency virus/ acquired immune deficiency syndrome  

PubMed Central

Ocular manifestations can occur in up to 50% of human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) patients and posterior segment involvement is the most common presentation. The posterior segment manifestations of AIDS can be divided into four categories: retinal vasculopathy, opportunistic infections, unusual malignancies and neuro-ophthalmologic abnormalities. Retinal microvasculopathy and cytomegalovirus (CMV) retinitis are the most common manifestations, even in the era of highly active anti-retroviral therapy (HAART). Highly active anti-retroviral therapy has been shown to cause regression of CMV retinitis, reduce the incidence of CMV-related retinal detachments, and prolong patient survival. Immune recovery uveitis is a new cause of vision loss in patients on HAART. Diagnosis and treatment are guided by the particular conditions and immune status of the patient.

2008-01-01

421

Neurofibromatosis type 1: a clinicopathological study of the orofacial manifestations in 6 pediatric patients.  

PubMed

Neurofibromatosis type 1 (NF1) is a relatively frequent mucocutaneous syndrome, which is transmitted as an autosomal dominant trait or which may represent neomutation. It is characterized by a variety of clinical manifestations, including multiple neurofibromas that are associated with a high risk of sarcomatous transformation. The aim of this report was to elucidate the orofacial manifestations observed in 6 pediatric patients (between 4 and 15 years of age) diagnosed with NF1. Physical, clinical, radiological, histological, and immunohistochemical studies were performed. Orofacial lesions were observed in all studied patients, located either in the soft tissues (4 cases) or centrally in the jaws (2 cases). All cases showed facial asymmetry, one of them exhibiting marked facial hemihypertrophy. All cases with soft tissue involvement were plexiform neurofibromas, while the intraosseous cases were diagnosed as solitary neurofibromas. Knowledge of the variability of presentation of orofacial soft tissue and bone manifestations of NF1 in children is necessary for prompt diagnosis. PMID:12528952

Sigillo, R; Rivera, H; Nikitakis, N G; Sauk, J J

2002-01-01

422

Childhood polyarteritis nodosa presenting with central nervous system manifestations and the posterior reversible encephalopathy syndrome.  

PubMed

Polyarteritis nodosa is a systemic necrotizing vasculitis involving medium-sized muscular arteries. Polyneuropathy is the only neurologic manifestation included in the pediatric classification schema. Central nervous system manifestations include infarction, hemorrhage, and encephalitis. We report on a 13-year-old female whose initial presentation of polyarteritis nodosa included hypertension, seizures, and neuroimaging findings of vasogenic edema and posterior reversible encephalopathy syndrome. Posterior reversible encephalopathy syndrome has been reported in association with renal disease, transplantation, autoimmunity, and cytotoxic medications. Posterior reversible encephalopathy syndrome outcomes are usually favorable with supportive care and treatment of the underlying etiology. The patient's neurologic condition improved after treatment of hypertension. Hypertension, posterior reversible encephalopathy syndrome, and abdominal pain led to a diagnostic workup. A systemic vasculitis was confirmed after detection of a perinephric hematoma and intrarenal aneurysms. This is a novel case of posterior reversible encephalopathy syndrome as an initial manifestation of pediatric polyarteritis nodosa. PMID:23155205

Guirola, Ricardo; Hunter, Jill V; Perez, Maria; Muscal, Eyal

2014-01-01

423

Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons.  

PubMed

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by autoantibody production and thrombosis or pregnancy morbidity. The most prevalent neurologic manifestation of APS is cerebrovascular ischemic events due to arterial thromboses. Antiphospholipid antibodies can also cause neurologic impairments unrelated to thrombosis, through antibody-cellular interactions, possibly because of a disrupted blood-brain barrier. Antiplatelet or anticoagulant therapies are currently indicated for APS-related ischemic strokes, but they remain controversial for non-thrombotic neurologic manifestations. Scant literature exists on neurologic manifestations and treatment regimens in childhood APS. Modifiable cardiac risk factors and valvular heart disease may worsen APS cerebrovascular outcomes. Adjunctive therapies (eg, statins, antimalarials, and angiotensin-converting enzyme inhibitors) warrant clinical trials. PMID:18457615

Muscal, Eyal; Brey, Robin L

2008-01-01

424

Restless legs syndrome and akathisia as manifestations of acute pontine infarction.  

PubMed

Although restless legs syndrome (RLS) and akathisia have similar clinical manifestations and seem to share a common pathophysiology, they are regarded as distinct clinical syndromes. We present three patients with acute pontine infarction and RLS or akathisia as clinical manifestations. They presented with abrupt onset of restlessness of various body parts, as well as other neurological signs including dysarthria or weakness of the legs. Brain MRI of all three patients showed acute pontine infarction. The clinical syndrome in two of the patients was compatible with RLS and one with akathisia. Their symptoms improved after a brief period. Our finding of secondary RLS and akathisia as manifestations of acute pontine infarction provides information that assists in understanding the common anatomical and pathophysiological basis of RLS and akathisia. PMID:23953640

Han, Su-Hyun; Park, Kwang-Yeol; Youn, Young Chul; Shin, Hae-Won

2014-02-01

425

Extensive development of flat warts as a cutaneous manifestation of immune reconstitution syndrome.  

PubMed

Cutaneous manifestations of immune recovery in response to highly active antiretroviral therapy may account for up to 54% to 78% of the clinical presentations of the immune reconstitution syndrome (IRS). Genital herpes, varicella-zoster virus infection, genital warts, and molluscum contagiosum represent the majority of these cutaneous manifestations. Inflammation of preexisting cutaneous warts in response to effective antiretroviral therapy has rarely been described. We report the case of sudden extensive development of cutaneous warts, specifically verruca plana confirmed by skin biopsy, observed following antiretroviral therapy-associated immune reconstitution in a patient without a history of warts. The possibility of cutaneous IRS after commencement of antiretroviral therapy should be considered in a patient with unusual skin manifestations. PMID:18975443

Iarikov, Dmitri; Duke, Wayne; Skiest, Daniel

2008-10-01

426

Orthopedic manifestations in patients with mucopolysaccharidosis type II (Hunter syndrome) enrolled in the Hunter Outcome Survey  

PubMed Central

Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare, inherited disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulfatase. As a result of this deficiency, glycosaminoglycans accumulate in lysosomes in many tissues, leading to progressive multisystemic disease. The cardiopulmonary and neurological problems associated with MPS II have received considerable attention. Orthopedic manifestations are common but not as well characterized. This study aimed to characterize the prevalence and severity of orthopedic manifestations of MPS II and to determine the relationship of these signs and symptoms with cardiovascular, pulmonary and central nervous system involvement. Orthopedic manifestations of MPS II were studied using cross-sectional data from the Hunter Outcome Survey (HOS). The HOS is a global, physician-led, multicenter observational database that collects information on the natural history of MPS II and the long-term safety and effectiveness of enzyme replacement therapy. As of January 2009, the HOS contained baseline data on joint range of motion in 124 males with MPS II. In total, 79% of patients had skeletal manifestations (median onset, 3.5 years) and 25% had abnormal gait (median onset, 5.4 years). Joint range of motion was restricted for all joints assessed (elbow, shoulder, hip, knee and ankle). Extension was the most severely affected movement: the exception to this was the shoulder. Surgery for orthopedic problems was rare. The presence of orthopedic manifestations was associated with the presence of central nervous system and pulmonary involvement, but not so clearly with cardiovascular involvement. Orthopedic interventions should be considered on an individual-patient basis. Although some orthopedic manifestations associated with MPS II may be managed routinely, a good knowledge of other concurrent organ system involvement is essential. A multidisciplinary approach is required.

Link, Bianca; de Camargo Pinto, Louise Lapagesse; Giugliani, Roberto; Wraith, James Edmond; Guffon, Nathalie; Eich, Elke; Beck, Michael

2010-01-01

427

Gender disparity between cutaneous and non-cutaneous manifestations of Lyme borreliosis.  

PubMed

Cutaneous manifestations of Lyme borreliosis in Europe include erythema migrans (EM) and acrodermatitis chronica atrophicans (ACA); the most common non-cutaneous manifestations are Lyme neuroborreliosis (LNB) and Lyme arthritis. The purpose of this study was to evaluate the gender distribution of patients with these clinical manifestations of Lyme borreliosis. Data on gender were obtained from the clinical records of patients with Lyme borreliosis aged ?15 years who had been evaluated at the University Medical Center Ljubljana, Ljubljana, Slovenia. Among 10,539 patients diagnosed with EM, 6,245 (59.3%) were female and among 506 ACA patients 347 (68.6%) were female. In contrast, among the 60 patients with Lyme arthritis only 15 (25%) were female (p<0.0001 for the comparison of gender with EM or ACA) and among the 130 patients with LNB only 51 (39.2%) were females (p<0.0001for the comparison of gender with EM or ACA). Although the proportion that was female in the LNB group was greater than that of patients with Lyme arthritis, this difference did not reach statistical significance (p?=?0.10). Although older individuals are more likely to be female in the general Slovenian population, the age of patients with cutaneous versus non-cutaneous manifestations was not the explanation for the observed differences in gender. In conclusion, patients with cutaneous manifestations of Lyme borreliosis were predominantly female, whereas those with non-cutaneous manifestations were predominantly male. This provocative finding is unexplained but may have direct relevance to the pathogenesis of Lyme borreliosis. PMID:23737968

Strle, Franc; Wormser, Gary P; Mead, Paul; Dhaduvai, Kanthi; Longo, Michael V; Adenikinju, Omosalewa; Soman, Sandeep; Tefera, Yodit; Maraspin, Vera; Lotri?-Furlan, Stanka; Ogrinc, Katarina; Cimperman, Jože; Ruži?-Sablji?, Eva; Stupica, Daša

2013-01-01

428

Kocher Debre Semelaigne Syndrome: A Rare Case Report with Orofacial Manifestations  

PubMed Central

Kocher Debre Semelaigne Syndrome is a rare syndrome with clinical manifestation of hypothyroidism associated with pseudomuscular hypertrophy. Very few cases have been reported on kocher-debre semelaigne syndrome in the literature with the incidence reported to be less than 10%. As per our knowledge; until now, only one case of oro-facial manifestations has been reported. Here we report one such case of Kocher debre semelaigne syndrome in an 11 year old boy, showing features of macroglossia, multiple impacted teeth, puffed lips and anterior open bite.

Panat, Sunil R.; Jha, Prakash Chandra; Chinnannavar, Sangamesh N.; Chakarvarty, Ankkita; Aggarwal, Ashish

2013-01-01

429

Quantum manifestations of graphene edge stress and edge instability: a first-principles study.  

PubMed

We have performed first-principles calculations of graphene edge stresses, which display two interesting quantum manifestations absent from the classical interpretation: the armchair edge stress oscillates with a nanoribbon width, and the zigzag edge stress is noticeably reduced by spin polarization. Such quantum stress effects in turn manifest in mechanical edge twisting and warping instability, showing features not captured by empirical potentials or continuum theory. Edge adsorption of H and Stone-Wales reconstruction are shown to provide alternative mechanisms in relieving the edge compression and hence to stabilize the planar edge structure. PMID:19518733

Huang, Bing; Liu, Miao; Su, Ninghai; Wu, Jian; Duan, Wenhui; Gu, Bing-Lin; Liu, Feng

2009-04-24

430

Widespread skin nodules as a manifestation of breakthrough invasive aspergillosis in a bone marrow transplant patient.  

PubMed

Invasive aspergillosis (IA) is a life-threatening complication of bone marrow transplantation manifesting most often as a respiratory tract infection with potential hematogenous dissemination to any organ. The authors report an unusual case of IA manifesting as widespread subcutaneous nodules, with intact overlying skin, complicating pulmonary infection in a transplanted patient who received mold-active antifungal prophylaxis. The present case emphasizes the relevance of cellular immunity, in particular T lymphocytes, in the control of invasive fungal disease and highlights the potential role of positron emission tomography scan in assessing its dissemination. PMID:23381978

Abecasis, Manuel; Ferreira, Isabelina; Teixeira, Gilda; Miranda, Nuno

2013-02-01

431

Diagnosis and clinical manifestations of calcium pyrophosphate and basic calcium phosphate crystal deposition diseases.  

PubMed

Basic calcium phosphate and pyrophosphate calcium crystals are the 2 main calcium-containing crystals that can deposit in all skeletal tissues. These calcium crystals give rise to numerous manifestations, including acute inflammatory attacks that can mimic alarming and threatening differential diagnoses, osteoarthritis-like lesions, destructive arthropathies, and calcific tendinitis. Awareness of uncommon localizations and manifestations such as intraspinal deposition (eg, crowned dens syndrome, tendinitis of longus colli muscle, massive cervical myelopathy compression) prevents inappropriate procedures and cares. Coupling plain radiography, ultrasonography, computed tomography, and synovial fluid analysis allow accurate diagnosis by directly or indirectly identifying the GRAAL of microcrystal-related symptoms. PMID:24703344

Ea, Hang-Korng; Lioté, Frédéric

2014-05-01

432

Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report*  

PubMed Central

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

Leal, Paula Azevedo Borges; Adriano, Adrilena Lopes; Breckenfeld, Marcelle Parente; Costa, Igor Santos; de Sousa, Antonio Rene Diogenes; Goncalves, Heitor de Sa

2013-01-01

433

Immunology and Clinical Manifestations of Non-Clonal Mast Cell Activation Syndrome  

PubMed Central

There is a spectrum of disorders that clinically manifest as a result of mast cell activation. A non-clonal form has emerged in the literature where many of the clinical features of systemic mastocytosis are shared despite having a distinct mast cell biology. In this review, we summarize key features of the science behind mast cell activation relevant to what is now known as non-clonal mast cell activation syndrome (nc-MCAS). We highlight the clinical manifestations of nc-MCAS with a focus on diagnosis and treatment.

Cardet, Juan-Carlos; Castells, Maria C.; Hamilton, Matthew J.

2012-01-01

434

Gyroscopic waves in the base of the solar corona: A model and possible observational manifestations  

NASA Technical Reports Server (NTRS)

A linear model for gyroscopic waves in the base of the solar corona is proposed. The purpose was to point out possible observational manifestations of the phenomenon. According to the model, these waves move slowly around the sun along heliolatitude circles. The fact that the red line corona is fainter on the solar side facing the apex and north-south and east-west asymmetries of the red coronal emission line could be interpreted as a possible observational manifestation of the gyroscopic wave phenomenon in the coronal base.

Pisanko, Y. V.; Tritakis, V. P.; Paliatsos, A. G.

1997-01-01

435

[Psychotic manifestations in childhood. III. Abnormal reactions to adventures (author's transl)].  

PubMed

In childhood psychotic manifestations are in most cases due to somatic diseases, but may be psychogenic. Abnormal reactions to adventures especially to very abnormal occurances--for instance in families with psychotic members--lead to psychotic symptoms. The appearence of those psychotic manifestations is influenced by the age of the child, by his hysterical, anancastic or austistic character and the pathological features (paranoic or depressive) of the inducing person. Usually hospitalization for diagnostic and therapeutical reasons is necessary. In case of induced psychoses separation must be long enough and has to be combined with treatment of the primary patient. PMID:1196651

Asperger, H; Groh, C; Rosenmayr, F W

1975-01-01

436

Psychiatric manifestations of primary Sjögren's syndrome: a case report and literature review.  

PubMed

A 54-year-old woman diagnosed with primary Sjögren's syndrome in 2007 presented with a 1-year history of visual hallucinations requiring admission to a psychiatric unit. The hallucinations resolved while on olanzapine and hydroxychloroquine but recurred when they were stopped. Despite restarting olanzapine, her visual hallucinations persisted. When she started a tapering dose of prednisolone, all the hallucinations resolved. This report adds to the small literature on psychiatric manifestations of Sjögren's syndrome and provides evidence that low-dose corticosteroids may be an effective treatment for this manifestation. PMID:24859541

Wong, John K F; Nortley, Ross; Andrews, Thomasin; D'Cruz, David

2014-01-01

437

Rare manifestations of sarcoidosis in modern era of new diagnostic tools  

PubMed Central

Background & objectives: Growing body of literature on sarcoidosis in India has led to an increased awareness of the disease. With the advent of better imaging tools hitherto under-recognized manifestations of sarcoidosis are likely to be better recognized. We sought to study the rare clinical and radiological manifestations (<5%) in patients with sarcoidosis. Methods: Retrospective review of records of 164 patients with histopathologically proven sarcoidosis seen over six years in a tertiary care centre in north India, was done. Results: Fifty four rare manifestations were observed in 164 patients. Acute presentation in the form of Lofgren syndrome was seen in eight (4.9%) and Heerfordt's syndrome in two (1.2%) patients. Musculoskeletal manifestations included chronic sarcoid arthritis in three (1.8%), deforming arthritis and bone erosion in one (0.6%) each. Rare initial presentation with dilated cardiomyopathy in one (0.6%), complete heart block in two (1.2%), bilateral sequential facial nerve palsy in two (1.2%), and pyrexia of unknown origin was seen in one (0.6%) patient. Other rare manifestations included chronic respiratory failure in one (0.6%), dysphagia in one (0.6%), sicca syndrome in five (3%), massive splenomegaly in one (0.6%), portal hypertension in two (1.2%), hypersplenism, gastric sarcoidosis, ninth and tenth cranial nerve palsies, moderate pericardial effusion and nephrocalcinosis in one (0.6%) each, and pulmonary artery hypertension in two (1.2%) patients. Rare radiological manifestations included moderate pleural effusion in two (1.2%), pleural thickening in five (3%), calcification of intrathoracic lymph nodes in four (2.4%), alveolar (nodular) sarcoidosis in three (1.8%), and myocardial uptake of 18F-fluorodeoxyglucose (F-18 FDG) in two (1.2%) patients. Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids. Interpretation & conclusions: Increased awareness of rare manifestations will facilitate better management of these patients. With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

Sharma, Surendra K; Soneja, Manish; Sharma, Abhishek; Sharma, Mehar C.; Hari, Smriti

2012-01-01

438

Zangfu zheng (patterns) are associated with clinical manifestations of zang shang (target-organ damage) in arterial hypertension  

Microsoft Academic Search

Background: Hypertension is a clinical condition that manifests target-organ damage (TOD) with symptoms. This study investigates the association between Zangfu patterns and symptomatic manifestations of TOD. Methods: Datasets with manifestations of Zangfu patterns (Liver-fire blazing upwards; Kidney-yin deficiency and Liver-yang rising; obstruction of phlegm and dampness of Heart\\/Liver\\/Gallbladder; qi and blood deficiency leading to Liver-yang rising; Kidney-yin\\/yang deficiency) and TODs

Alexandre Bastos Luiz; Ivan Cordovil; José Barbosa Filho; Arthur Sá Ferreira

2011-01-01

439

Long-Term Academic Benefits of Service-Learning: When and Where do They Manifest Themselves?  

ERIC Educational Resources Information Center

During the last several years, researchers have been garnering evidence concerning the benefits of service-learning, and urging its inclusion in the curricular mainstream. This study presents evidence that college students manifest long-lasting, if modest, academic benefits from participating in service-learning. The academic records of 477…

Strage, Amy

2004-01-01

440

Manifestations of Metadata: From Alexandria to the Web--Old is New Again  

ERIC Educational Resources Information Center

This paper is a discussion of the use of metadata, in its various manifestations, to access information. Information management standards are discussed. The connection between the ancient world and the modern world is highlighted. Individual perspectives are paramount in fulfilling information seeking. Metadata is interpreted and reflected upon in…

Kennedy, Patricia

2008-01-01

441

Manifest: A computer program for 2-D flow modeling in Stirling machines  

NASA Technical Reports Server (NTRS)

A computer program named Manifest is discussed. Manifest is a program one might want to use to model the fluid dynamics in the manifolds commonly found between the heat exchangers and regenerators of Stirling machines; but not just in the manifolds - in the regenerators as well. And in all sorts of other places too, such as: in heaters or coolers, or perhaps even in cylinder spaces. There are probably nonStirling uses for Manifest also. In broad strokes, Manifest will: (1) model oscillating internal compressible laminar fluid flow in a wide range of two-dimensional regions, either filled with porous materials or empty; (2) present a graphics-based user-friendly interface, allowing easy selection and modification of region shape and boundary condition specification; (3) run on a personal computer, or optionally (in the case of its number-crunching module) on a supercomputer; and (4) allow interactive examination of the solution output so the user can view vector plots of flow velocity, contour plots of pressure and temperature at various locations and tabulate energy-related integrals of interest.

Gedeon, David

1989-01-01

442

"I Pay Your Salary!" Manifestations of Student Consumerism in the Classroom  

ERIC Educational Resources Information Center

This qualitative case study explored the Student Consumerism phenomenon as displayed by post-secondary students as perceived by their instructors. Because it was not entirely understood how post-secondary instructors react to the manifestations that occur due to Student Consumerism style of behaviors, this study examined the phenomenon from that…

Plunkett, Anthony D.

2011-01-01

443

Metabolic syndrome and incidence of type 2 diabetes in patients with manifest vascular disease  

Microsoft Academic Search

Risk reduction in patients with clinically manifest vascular disease focuses on preventing new vascular events and not on prevention of type 2 diabetes. However, given the common pathophysiological pathways involved in the development of atherosclerosis and type 2 diabetes, it is probable that people with atherosclerotic vascular disease have an elevated risk of type 2 diabetes. The present prospective cohort

Annemarie Mj Wassink; Yolanda Van Der Graaf; Sabita S Soedamah-Muthu; Wilko Spiering; Frank Lj Visseren

2008-01-01