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1

Ocular Manifestations  

Microsoft Academic Search

Crohn’s disease (CD), or regional enteritis, is a segmentary granulomatous inflammatory bowel pathology of unknown etiology\\u000a although autoimmunity is probably involved. In addition, there are extraintestinal manifestations, such as arthritis, cutaneous\\u000a lesions, and hepatic, renal, and ocular involvement. The incidence of the latter, manifested as uveitis, scleritis, and episcleritis,\\u000a is about 2–9% of the cases of CD [1–4]. Moreover, 30–40%

Vincenzo Petitti; Stefano Tamburi

2

Occult Manifestations of Cancer  

PubMed Central

Clinical syndromes occasionally associated with or heralding cancer are summarized and classified. Some tumours present with manifestations of an endocrine or endocrine-like action; included in this group are thymomas, non-beta-cell tumours of the pancreas and carcinoids. Cushing's syndrome, hypoglycemia, hypercalcemia, polycythemia and gynecomastia have been produced by a wide variety of tumours. Tumour emboli, non-bacterial thromboendocarditis and thrombophlebitis occasionally occur, but thrombophlebitis is not so frequent as was previously considered. Neurological syndromes are rare and show a great variety of presentations. Other occult manifestations of cancer include hypertrophic pulmonary osteoarthropathy, various skin diseases, obscure pyrexias and, in Hodgkin's disease, pain secondary to alcohol consumption.

Henderson, Robert D.

1963-01-01

3

Thermodynamics in 'Manifest Reality'  

SciTech Connect

D'Espagnat's proof that the universe is not a 'strongly objective reality' demands that all physical processes are reconsidered in that light. D'Espagnat suggests a 'Veiled Reality' as a suitable alternative. The most economical way to achieve that is to demand that 'information production' at a quantum level creates the basis for self-consistent perception of a world of macroscopic, 'manifest' entities, as opposed to self-existent objects. Such a 'manifest reality' fulfils both Wheeler's attempt at an 'IT-from-BIT' programme, and Zeilinger's suggestion that 'information is primary'.

Hankey, Alex [Meru Research Institute, Bucks, 1985 (United Kingdom)

2010-12-22

4

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

5

[Pulmonary manifestations of malaria].  

PubMed

We report on the two different types of pulmonary manifestations in acute plasmodium falciparum malaria. The more severe variant shows long standing interstitial pulmonary infiltrates, whereas in the more benign courses only short-term pulmonary edemas are visible. PMID:3035632

Rauber, K; Enkerlin, H L; Riemann, H; Schoeppe, W

1987-05-01

6

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

7

Sinonasal Manifestations in Cystic Fibrosis  

PubMed Central

Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. Although the major clinical manifestations of the disease are pancreatic and pulmonary disease, the majority of cystic fibrosis patients will develop sinonasal manifestations as well. This paper outlines the etiology, evaluation, and management of the nasal and sinus manifestations in patients with cystic fibrosis.

Oomen, Karin P. Q.; April, Max M.

2012-01-01

8

Laryngotracheal manifestations of rhinoscleroma.  

PubMed

Rhinoscleroma is a rare, chronic granulomatous disease of infective causation. It usually begins in the nose and may progress to involve the larynx and trachea and cause dysphonia, stridor, and airway obstruction. Early rhinoscleroma is usually successfully treated with oral tetracycline, yet laryngotracheal disease may require operative intervention. The disease is rare in the United States, but with an increase in immigration from endemic areas, otolaryngologists should be familiar with the management of this rare disease. Current literature contains only a few reports describing the manifestations of this disease, mostly in the form of case studies. This study is a retrospective review of our institutional experience with the management of 22 patients with rhinoscleroma, 13 of whom had laryngotracheal involvement. The focus of this report is on the clinical manifestations of laryngotracheal scleroma. All of the patients were treated with long-term antibiotics. Nine patients underwent endoscopy with or without dilation and laser excision. Three patients required emergency tracheostomy, all of whom were ultimately decannulated without any sequelae. A rational approach to management of this unusual disease is provided. PMID:8651625

Amoils, C P; Shindo, M L

1996-05-01

9

Pulmonary manifestations of sarcoidosis.  

PubMed

Sarcoidosis affects the lungs in more than 90% of cases. Symptoms include cough, dyspnea, and chest pain. The entire respiratory tract can be involved. The most common areas of involvement are the airways and interstitium. Airway disease can lead to airway obstruction while interstitial lung disease can lead to restrictive disease. Patients may have a mix of these areas of involvement. For the symptomatic patient, the identification of disease involvement can usually be determined by pulmonary function testing and chest imaging. The chest X-ray staging system has been widely used in sarcoidosis, high-resolution computer tomography (HRCT) can provide detailed information regarding lung involvement. Unfortunately the various patterns seen on HRCT have limited the ability to develop a simple scoring system. Special studies such as bronchoscopy can be useful for detecting large airway disease. Other chest manifestations include adenopathy, pulmonary hypertension, and pulmonary muscle weakness. Fibrotic lung disease can lead to bronchiectasis, which can become infected. PMID:22579234

Baughman, Robert P; Lower, Elyse E; Gibson, Kevin

2012-05-10

10

Perioperative allergy: clinical manifestations.  

PubMed

Adverse drug reactions or side effects are usually expected, dose dependent, and occur at therapeutic doses. Anaphylactic and anaphylactoid reactions are unexpected and dose independent and can occur at the first exposure to drugs used during anesthesia. Perioperative anaphylaxis is a severe and rapid clinical condition that can be lethal even in previously healthy patients. The initial diagnosis of anaphylaxis is presumptive. A precise identification of the drug responsible for the adverse reaction is more difficult to establish in the case of anaphylactoid reaction because the adverse reaction could result from additive side effects of different drugs injected simultaneously. The timing of the reaction in relation to events, e.g. induction, start of surgery, administration of other drugs, i.v. fluids, is essential for the diagnosis. Generally, reactions are predominant in the induction and recovery phases, and manifested mainly as cutaneous symptoms. Reactions to drugs coincide with the phases when they are administered. Reactions to antibiotics are more frequent in the induction phase, to neuromuscular agents in the initiation and maintenance phases and to non-steroidal anti-inflammatory agents in the recovery phase. The differential diagnosis of any adverse reaction during or following anesthesia should include the possibility of anaphylaxis. PMID:22014928

Peroni, D G; Sansotta, N; Bernardini, R; Cardinale, F; Paravati, F; Franceschini, F; Boner, A L

11

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

12

Cardiac manifestations of subarachnoid hemorrhage.  

PubMed

Subarachnoid hemorrhage (SAH) is a devastating condition. It carries a high mortality rate, with 12% of patients dying before reaching the hospital. Aside from its neurological morbidities, SAH is associated with significant medical complications. Cardiac manifestations are common and can impact morbidity and mortality in SAH patients. This article will discuss the cardiac manifestations of SAH. PMID:21438809

Behrouz, Réza; Sullebarger, John T; Malek, Ali R

2011-03-01

13

NEUROLOGICAL MANIFESTATIONS IN DENGUE PATIENTS  

Microsoft Academic Search

To determine the frequency and the natural history of neurological manifestations of dengue infection in Thai children, 1,493 children diagnosed with dengue infection by serology and admitted to the Department of Pediatrics, Chulalongkorn Hospital, Bangkok, Thailand from 1987 to 1998 were reviewed from prospectively recorded medical charts. There were 80 chil- dren identified with neurological manifestations, an incidence of 5.4%

Chitsanu Pancharoen

14

Ocular manifestations of Behcet's disease.  

PubMed

Behcets disease is a systemic inflammatory vascular disorder characterized by recurrent oral and genital ulcers, eye lesion, arthritis and skin lesions. We report a case of Behcets disease with ocular manifestation in an 8 year old boy. PMID:16208057

A, Sam Ebenezer; Moses, Prabhakar D; George, Renu

2005-09-01

15

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

16

[Cardiovascular manifestations of human toxocariasis].  

PubMed

Toxocariasis is a parasitic infection produced by helminths that cannot reach their adult stage in humans. For their etiological species (Toxocara canis and Toxocara cati), man is a paratenic host. Infection by such helminths can produce a variety of clinical manifestations, such as: visceral larvae migrans syndrome, ocular larvae migrans syndrome and covert toxocariasis. In the visceral larvae migrans syndrome, the organs that are mainly involved include liver, lungs, skin, nervous system, muscles, kidneys and the heart. Regarding the latter, the importance of cardiovascular manifestations in toxocariasis, as well as its clinical relevance, has increasingly begun to be recognized. The current article is based on a systematic information search, focused mainly on the clinical and pathological aspects of cardiovascular manifestations in toxocariasis, including its pathophysiology, laboratory findings, diagnosis and therapeutical options, with the objective of highlighting its importance as a zoonosis and its relevance to the fields of cardiovascular medicine in adults and children. PMID:23462238

Bolívar-Mejía, Adrián; Rodríguez-Morales, Alfonso J; Paniz-Mondolfi, Alberto E; Delgado, Olinda

17

Rheumatological manifestations of infective endocarditis.  

PubMed Central

A retrospective study showed musculoskeletal manifestations in 32 of 108 patients treated for infective endocarditis in several departments at the Poitiers CHU. Such manifestations included articular pain or aseptic arthritis, typically involving the major joints, as well as vertebral osteomyelitis, low back pain (inflammatory or non-inflammatory), and myalgia. Patients showing such signs were generally younger than those without musculoskeletal involvement, diagnosis was made later, and prognosis was worse; streptococcus D was more often involved, and microscopic haematuria was more common. With the exception of vertebral osteomyelitis, the pathogenesis was not clear.

Thomas, P; Allal, J; Bontoux, D; Rossi, F; Poupet, J Y; Petitalot, J P; Becq-Giraudon, B

1984-01-01

18

Material Manifestations of the Divine  

Microsoft Academic Search

The author, a Catholic who studies South Asian languages and culture, describes Hindu and Christian mythologies based on their respective scriptures. She makes the case that, abstractly, both ?akti and logos symbolize a transcen- dent God's Word in creation and, in relation to their personal forms, the means to liberation and salvation. As a relational mode of the divine manifest

Lauren Bausch

19

Neurologic Manifestations of Infective Endocarditis  

Microsoft Academic Search

Background: Many previous studies have endeavored to find appropriate means to reduce the occurrence of neu- rologic manifestations in patients with infective endocar- ditis (IE). We evaluated patients with IE-associated neu- rologic complications and compared them with patients with IE who did not have neurologic symptoms. Particu- lar attention was focused on assessing the impact of car- diac surgery and

Maija Heiro; Jukka Nikoskelainen; Erik Engblom; Esa Kotilainen; Reijo Marttila; Pirkko Kotilainen

20

Segmental Manifestation of Darier Disease  

Microsoft Academic Search

Darier disease is an autosomal dominant disorder which may occasionally become manifest in a segmental form. Two clinical phenotypes with a different genetic background have been elaborated in recent years. More than 50 patients with isolated linear disease expression have been documented. In this phenotype the skin outside the segmental affection is absolutely normal. Such a phenotype is explained by

Peter H. Itin; Stanislaw A. Büchner; Rudolf Happle

2000-01-01

21

Psychiatric manifestations in cerebrotendinous xanthomatosis  

PubMed Central

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.

Fraidakis, M J

2013-01-01

22

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

2013-09-03

23

Radiologic manifestations of pulmonary tuberculosis.  

PubMed

In summary, the following points are reemphasized: 1. The chest film is the mainstay in the radiologic evaluation of suspected or proven pulmonary TB. CT is occasionally useful for clarifying confusing findings but has not been conclusively shown to have a significant impact on patient management. 2. Primary TB is increasingly a disease of adults. 3. Primary TB usually manifests as a parenchymal consolidation in any pulmonary lobe or segment. Distinguishing features from typical bacterial pneumonia include associated adenopathy, lack of systemic toxicity, failure to respond to conventional antibacterial therapy, and recent PPD conversion. 4. Associated ipsilateral hilar and/or mediastinal adenopathy is almost universal in children with primary TB but is less common in adults. Adenopathy without parenchymal disease is an unusual but well-reported manifestation. 5. Many of the so-called unusual manifestations of adult TB are the usual manifestations of primary disease. The terms adult and childhood TB should be discarded. 6. Postprimary TB typically manifests as a heterogeneous, often cavitary opacity in the apical and posterior segments of the upper lobes and the superior segments of the lower lobes. Lymphadenopathy is rare. 7. Activity of postprimary disease cannot be accurately assessed by chest radiography. Radiographic stability for 6 months and negative sputum cultures is the best indicator of inactive disease. The descriptive terms inactive or old TB should be discarded in favor of radiographically stable TB, as viable bacilli may persist despite adequate therapy. 8. Cavitation is the most important radiologic finding in postprimary disease. Cavitation implies a high bacillary burden, high infectivity, and is associated with numerous complications including endobronchial spread, tuberculous empyema, hematogenous dissemination, pulmonary artery pseudoaneurysm, and so forth. 9. Tuberculous pleurisy is more common in primary than postprimary disease. It is a common presenting manifestation in young adults. The effusions are unilateral, large, and self-limited. The pleural fluid usually is a serous exudate with a marked lymphocytosis. Fluid cultures are frequently negative. Correct diagnosis and therapy is important, as untreated patients are at high risk for subsequent pulmonary reactivation. 10. Miliary disease is also more common in primary than postprimary disease; however, its frequency in elderly patients with postprimary TB is increasing. This form, known as late generalized TB, is apt to be misdiagnosed or not diagnosed in life and has a high mortality. PMID:7610237

McAdams, H P; Erasmus, J; Winter, J A

1995-07-01

24

Clinical Manifestations of Synovial Cysts  

PubMed Central

Although synovial cysts are most commonly associated with rheumatoid arthritis and osteoarthritis, they may occur in many other conditions. The clinical manifestations of these cysts are numerous and may result from pressure, dissection or acute rupture. Vascular phenomena occur when popliteal cysts compress vessels, and result in venous stasis with subsequent lower extremity edema or thrombophlebitis. Rarely, popliteal cysts may cause arterial compromise with intermittent claudication. Neurological sequelae include pain, paresthesia, sensory loss, and muscle weakness or atrophy. When synovial cysts occur as mass lesions they may mimic popliteal aneurysms or hematomas, adenopathy, tumors or even inguinal hernias. Cutaneous joint fistulas, septic arthritis or osteomyelitis, and spinal cord and bladder compression are examples of other infrequent complications. Awareness of the heterogeneous manifestations of synovial cysts may enable clinicians to avoid unnecessary diagnostic studies and delay in appropriate management. Arthrography remains the definitive diagnostic procedure of choice, although ultrasound testing may be useful. ImagesFig. 1.Fig. 2.Fig. 3.

Burt, Todd B.; Gelman, Martin I.; MacCarter, Daryl K.; Samuelson, Cecil O.

1980-01-01

25

Musculoskeletal manifestations of bacterial endocarditis.  

PubMed Central

The records of 180 patients out of 247 with bacterial endocarditis were examined. 50 patients had rheumatic manifestations. In 10 there was arthritis of 2-12 weeks' duration before diagnosis; 19 had myalgia/arthralgia; 17 had back or neck pain; 14 had demonstrable arthritis; and 2 tenosynovitis of the foot. Of the 14 patients with arthritis, 8 had monarticular arthritis and 6 polyarticular. All but one patient had a raised erythrocyte sedimentation rate, and in one patient rheumatoid factor was positive. The rheumatic features responded when the endocarditis was treated. Some of the symptoms undoubtedly resulted from the infection and fever of the endocarditis, and emboli may have caused the transient aches but there was no evidence that they caused the synovitis in the patients with arthritis. The rheumatic manifestations of bacterial endocarditis can mimic other rheumatic diseases and disguise the underlying disease.

Meyers, O L; Commerford, P J

1977-01-01

26

[Neuropsychiatric manifestations in systemic sclerosis].  

PubMed

Until recently, systemic sclerosis (SSc) was thought to spare the central nervous system (CNS). Neurological symptoms secondary to CNS involvement are very rare in SSc patients. Conversely, the prevalence of depression in SSc patients ranges from 17 to 65% and is much higher than that observed in the general population. Cognitive impairment has been reported in SSc patients, but these findings require confirmation in further studies. Brain calcifications and hyperintense white matter signals (leukoaraiosis) have been documented in SSc patients at a higher incidence than in control populations. Severe leukoaraiosis lesions seem to be associated with severe vascular manifestations in SSc. If morphological CNS abnormalities could be linked to neuropsychiatric manifestations, it would be possible to identify neuropsychiatric scleroderma as we can now identify neuropsychiatric systemic lupus erythematosus. PMID:20067855

Launay, David; Baubet, Thierry; Cottencin, Olivier; Bérezné, Alice; Zéphir, Hélène; Morell-Dubois, Sandrine; Pruvo, Jean-Pierre; Guillevin, Loïc; Hatron, Pierre-Yves; Hachulla, Eric; Mouthon, Luc

2010-01-10

27

Cutaneous manifestations of fungal infection.  

PubMed

Fungal infection of the skin and subcutaneous tissue may result from either direct contact or inoculation injury (primary infection) or from hematogenous spread from a primary focus of disease (secondary infection). The parainfectious lesions of erythema nodosum and erythema multiforme are manifestations of the host's immune response to the invading fungus, particularly Histoplasma capsulatum and Coccidiodes immitis. In some patients, skin lesions may be the only sign of a systemic fungal infection, and prompt recognition of these lesions may facilitate early diagnosis and treatment. This article first addresses the pathogenesis, host defenses, and diagnosis of fungal skin infections. The specific cutaneous manifestations of the superficial, cutaneous, subcutaneous, and systemic mycoses are then reviewed. PMID:7890937

Chapman, S W; Daniel, C R

1994-12-01

28

Cutaneous manifestations of filovirus infections.  

PubMed

Ebolavirus and Marburgvirus, two filoviruses belonging to the Filoviridae family, are among the most virulent pathogens for humans and non-human primates, causing outbreaks of fulminant hemorrhagic fever (HF) in Central African countries with case fatality rates of up to 90%. Fruit bats are the likely reservoir, and human infection occurs through contact with bats or infected large-animal carcasses or by person-to-person contact (through body fluids, medical care, and burial practices). Schematically, clinical manifestations occur in three successive phases and include general, gastrointestinal, and mucocutaneous disorders. Death usually results from hemorrhagic complications. Cutaneous manifestations rarely make a major contribution to disease severity but can assist with the diagnosis. Rash, the main cutaneous disorder, is nonspecific and cannot guide the differential diagnosis. Immunohistochemical examination of skin biopsy or necropsy specimens can confirm the diagnosis. PMID:22909355

Nkoghe, Dieudonné; Leroy, Eric Maurice; Toung-Mve, Médard; Gonzalez, Jean Paul

2012-09-01

29

Clinical Manifestations of Portal Hypertension  

PubMed Central

The portal hypertension is responsible for many of the manifestations of liver cirrhosis. Some of these complications are the direct consequences of portal hypertension, such as gastrointestinal bleeding from ruptured gastroesophageal varices and from portal hypertensive gastropathy and colopathy, ascites and hepatorenal syndrome, and hypersplenism. In other complications, portal hypertension plays a key role, although it is not the only pathophysiological factor in their development. These include spontaneous bacterial peritonitis, hepatic encephalopathy, cirrhotic cardiomyopathy, hepatopulmonary syndrome, and portopulmonary hypertension.

Al-Busafi, Said A.; McNabb-Baltar, Julia; Farag, Amanda; Hilzenrat, Nir

2012-01-01

30

Uncommon manifestations of shunt infection  

Microsoft Academic Search

Shunt infection is one of the commonest and most troublesome cause of shunt failure. According to the etiology and clinical\\u000a presentations it has been divided into external and internal types.Method : We hereby present four pediatric cases, who presented with atypical manifestations not coinciding with the above mentioned\\u000a types of shunt infection. Three patients developed round, globular or diffuse fluctuant

Raj Kumar

2003-01-01

31

Systemic diseases with cutaneous manifestations.  

PubMed

The purpose of this article is to briefly discuss the following cutaneous manifestations of selected systemic diseases: poxvirus; feline leukemia virus (FeLV); feline immunodeficiency virus (FIV); herpesvirus; calcivirus; pseudorabies; plague; tularemia; toxoplasmosis; leishmania; hypothyroidism; hyperthyroidism; hyperadrenocorticism; diabetes mellitus; acromegaly; thallium poisoning; pancreatic disease; hypereosinophilic syndrome; mucopolysaccharidosis; and pansteatitis. Recognition of these cutaneous signs may help alert the clinician to the possibility of an internal disorder so that the appropriate diagnostic tests can be considered. PMID:8525575

Merchant, S R; Taboada, J

1995-07-01

32

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

33

Manifestations cutanées des syndromes myélodysplasiques  

Microsoft Academic Search

Purpose. – Our purpose was to describe cutaneous manifestations associated with myelodysplastic syndromes.Methods. – Data from seven patients with cutaneous vasculitis (four cases), neutrophilic dermatosis (one case), relapsing polychondritis (one case), and possible erythema elevatum diutinum (one case) in association with myelodysplastic syndrome (refractory anaemia RA, RA with excess of blasts – RAEB-, RAEB in transformation RAEBt, chronic myelomonocytic leukaemia – CMML-), and analysis of the

J. J Morand; E Lightburn; M. A Richard; S Hesse-Bonerandi; F Carsuzaa; J. J Grob

2001-01-01

34

[Vectorcardiographic manifestations of atrial enlargements].  

PubMed

Rational interpretation of changes of the P loop due to atrial enlargements must to rely on the magnitude and spatial orientation of main resultant vectors of the activation sequence of the atria. Under normal conditions, these vectors give rise to a mean vector oriented to the left downward and discretely forward with respect to their point of origin. In the presence of right atrial enlargement, the manifestation of the first vector of atrial depolarization, oriented downward and forward, is increased. This one moves in the same direction as the mean vector of atrial depolarization, originating an elongated P loop of more than 100 mcv in the three planes. Nevertheless, in the horizontal plane, increase of the P loop voltage predominates when hypertrophy exists, while augmentation of its area predominates when dilatation exists. In left atrial enlargement, the manifestation of the second vector of atrial depolarization, oriented to the left and backward, is augmented, and it moves in the same direction as the mean vector of atrial depolarization. For this, the PF loop acquires a characteristic aspect of a boxing glove, an the PH loop becomes diphasic, with its posterior area more or less prominent, or with a typical figure-eight conformation. If a biatrial enlargement is present, the manifestation of both the main resultant vectors of atrial depolarization is accentuated. Therefore the voltage of the diphasic P loop increases. Moreover the Ps loop has a triangular configuration, with its base of 30 msc or more, located below its point of origin. Generally disturbances of interatrial and intraatrial conduction coexist owing to myocardial damage. PMID:2146934

de Micheli, A; Medrano, G A

35

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

36

MANIFEST ANXIETY IN BRONCHIAL ASTHMA  

PubMed Central

SUMMARY Using a vernacular adaptation of MAS 50 bronchial asthma patients were compared with 102 normals, 60 hospital general out-patients and 50 neurotics to determine the level of anxiety in asthma. The manifest anxiety scores of the bronchial asthma patients were found to be significantly high showing that their level of anxiety was abnormally higher in comparison with that of the normals and the hospital general out-patients. The bronchial asthmatics and the neurotics did not differ in anxiety. The implications of these findings were discussed.

Sreedhar, Krishna Prasad

1989-01-01

37

Cutaneous manifestations of chikungunya fever.  

PubMed

Chikungunya fever, a re-emerging RNA viral infection produces different cutaneous manifestations in children compared to adults. 52 children with chikungunya fever, confirmed by positive IgM antibody test were seen during 2009-2010. Pigmentary lesions were common (27/52) followed by vesiculobullous lesions (16/52) and maculopapular lesions (14/52). Vesiculobullous lesions were most common in infants, although rarely reported in adults. Psoriasis was exacerbated in 4 children resulting in more severe forms. In 2 children, guttate psoriasis was observed for the first time. PMID:21992863

Seetharam, K A; Sridevi, K; Vidyasagar, P

2011-08-15

38

Neuropsychiatric manifestations of thyroid disease.  

PubMed

The interface between thyroid hormone action and neuropsychiatric function is intricate, and several mechanisms of thyroid hormone uptake into brain tissues, hormone activation, and influences on neurotransmitter generation have been identified. Symptoms of hypothyroidism are nonspecific, whereas those attributed to thyrotoxicosis may be more characteristic. Neuropsychiatric manifestations triggered by thyroid dysfunction likely respond well to reestablishment of the euthyroid state, although some patients have persistent complaints. The addition of LT3 to ongoing LT4 replacement has yet to be definitively shown to be advantageous. Treatment of euthyroid depression with LT3 in addition to antidepressant therapy lacks convincing evidence of superior outcomes. PMID:24011880

Feldman, Anna Z; Shrestha, Rupendra T; Hennessey, James V

2013-09-01

39

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

40

Digestive manifestations of parathyroid disorders.  

PubMed

The parathyroid glands are the main regulator of plasma calcium and have a direct influence on the digestive tract. Parathyroid disturbances often result in unknown long-standing symptoms. The main manifestation of hypoparathyroidism is steatorrhea due to a deficit in exocrine pancreas secretion. The association with celiac sprue may contribute to malabsorption. Hyperparathyroidism causes smooth-muscle atony, with upper and lower gastrointestinal symptoms such as nausea, heartburn and constipation. Hyperparathyroidism and peptic ulcer were strongly linked before the advent of proton pump inhibitors. Nowadays, this association remains likely only in the particular context of multiple endocrine neoplasia type 1/Zollinger-Ellison syndrome. In contrast to chronic pancreatitis, acute pancreatitis due to primary hyperparathyroidism is one of the most studied topics. The causative effect of high calcium level is confirmed and the distinction from secondary hyperparathyroidism is mandatory. The digestive manifestations of parathyroid malfunction are often overlooked and serum calcium level must be included in the routine workup for abdominal symptoms. PMID:22039319

Abboud, Bassam; Daher, Ronald; Boujaoude, Joe

2011-09-28

41

Cutaneous Manifestations of Internal Malignancy  

PubMed Central

Background: Many malignancies affecting the internal organs display cutaneous manifestations which may be either specific (tumor metastases) or nonspecific lesions. Aims: The study is aimed at determining the frequency and significance of cutaneous manifestations among patients with internal malignancy. Materials and Methods: 750 cases of proven internal malignancy, who attended a cancer chemotherapy center in South India, were studied. Specific infiltrates were confirmed by histopathology, fine needle aspiration cytology (FNAC) and marker studies. Results: Out of the 750 patients with internal malignancy, skin changes were seen in a total of 52 (6.93%) patients. Conclusion: Cutaneous metastases (specific lesions) were seen in 20 patients (2.66%): contiguous in 6 (0.8%), and non-contiguous in 14 (1.86%). Nonspecific skin changes were seen in 32 patients (4.26%). None of our patients presented with more than one type of skin lesions. Herpes zoster was the most common nonspecific lesion noticed in our patients, followed by generalized pruritus, multiple eruptive seborrheic keratoses, bullous disorder, erythroderma, flushing, purpura, pyoderma gangrenosum, insect bite allergy and lichenoid dermatitis.

Ayyamperumal, A; Tharini, GK; Ravindran, Vidhya; Parveen, B

2012-01-01

42

Hematologic manifestations of liver disease.  

PubMed

The liver plays a key role in both protein biosynthesis and lipid metabolism. As a result, hepatic synthetic dysfunction can have adverse effects on both cellular and soluble components of blood. Anemia may occur due to the hemolysis of acanthocytes (spur cells), which is ultimately due to abnormal lipid composition of the red blood cell membrane. Thrombocytopenia may result from several different mechanisms. Cytopenias also may be a consequence of hypersplenism. The liver is the primary site for synthesis of most procoagulant and anticoagulant proteins. The coagulopathy of liver disease is therefore complex. Early in the course of liver disease, thrombocytopenia and a coagulopathy associated with a prothrombotic state are not uncommon, whereas with more advanced disease pancytopenia and coagulopathy associated with hemorrhage become manifest. Fresh frozen plasma and adjuncts to hemostasis may be used as temporizing measures in bleeding patients. However, definitive management of many of the defects due to fulminant hepatic failure requires liver transplantation. PMID:23953338

Marks, Peter W

2013-07-01

43

Cutaneous manifestations of endemic mycoses.  

PubMed

All of the endemic mycoses have cutaneous and mucocutaneous manifestations that are most commonly seen when patients have disseminated infection. Biopsy of skin lesions is simple and safe and can assist in making a timely diagnosis of disseminated infection. Primary cutaneous inoculation infection has been reported with all of the endemic mycoses, but is rare. In this situation, a nodule or ulcer occurs at the inoculation site, is often accompanied by lymphangitis and regional lymphadenopathy, and systemic symptoms and signs as almost always absent. Mucosal lesions are common with disseminated histoplasmosis, but also have been described in patients who have disseminated blastomycosis and coccidioidomycosis. Biopsy is essential to rule out cancer and allows a rapid diagnosis of the endemic fungal infection. PMID:23917880

Smith, Jeannina A; Riddell, James; Kauffman, Carol A

2013-10-01

44

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

45

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

46

MUCOCUTANEOUS MANIFESTATIONS OF CHIKUNGUNYA FEVER  

PubMed Central

Chikungunya fever (CF) is an arboviral acute febrile illness transmitted by the bite of infected Aedes mosquitoes. After a quiescence of more than three decades, CF has recently re-emerged as a major public health problem of global scale. CF is characterized by an acute onset of high fever associated with a severe disabling arthritis often accompanied by prominent mucocutaneous manifestations. The disease is usually self-limiting, but the joint symptoms and some of the cutaneous features may persist after the defervescence. A wide range of mucocutaneous changes has been described to occur in association with CF during the current epidemic. Besides a morbilliform erythema, hyperpigmentation, xerosis, excoriated papules, aphthous-like ulcers, vesiculobullous and lichenoid eruptions, and exacerbation of pre-existing or quiescent dermatoses had been observed frequently. These unusual features may help in the clinical differential diagnosis of acute viral exanthems mimicking CF.

Bandyopadhyay, Debabrata; Ghosh, Sudip Kumar

2010-01-01

47

Orbital manifestations of Noonan syndrome.  

PubMed

Noonan syndrome describes a rare multisystem condition that manifests with Turner syndrome phenotype combined with numerous systemic and facial characteristics. The most common systemic findings include cardiac defects, short stature, chest deformity, hearing loss, and bleeding diatheses. Patients with Noonan syndrome are also at a greater risk of developing various types of malignancies. Facial characteristics of Noonan syndrome consist of broad forehead, low-set ears, short and webbed neck, and low hairline. External ocular findings include hypertelorism, ptosis, epicanthal folds, antimongoloid slant, downward-sloping palpebral fissures, and malar flattening. In this case series and review of the literature, the authors present 2 cases of Noonan syndrome that embody the diverse spectrum of orbital manifestations associated with this rare disorder. The first case demonstrates more profound orbital pathology, including bilateral orbital giant cell reparative granulomas, proptosis, hypertelorism, shallow orbits, upper eyelid ptosis, and lower eyelid retraction. The second case displays classic Turner-syndrome-like facial characteristics including a broad forehead with low hairline, low-set ears, and short and webbed neck. Orbital findings were subtle and consisted of bilateral lower eyelid retraction and shallow orbits. In conclusion, Noonan syndrome is a rare congenital disorder with a wide spectrum of clinical findings. Various intraorbital and extraorbital tumors may occur in patients with Noonan syndrome, with giant cell reparative granuloma being the most commonly encountered. In patients with orbital tumors and eyelid retraction, the authors describe successful treatment through decompression, tumor extraction, and lower eyelid retractor release. Patients who present with ocular irritation and exposure due to less severe lower eyelid malposition may be successfully treated with lower eyelid retraction repair combined with lateral internal tarsoconjunctival tarsorrhaphy. PMID:21464791

Randolph, John C; Sokol, Jason A; Lee, Hui Bae H; Nunery, William R

48

[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

49

Esophageal manifestations of celiac disease.  

PubMed

Celiac disease (CD) may often be associated with various motor disorders affecting the different segments of the digestive tract, including the esophagus. Although it has not been universally reported, some available evidences indicate that pediatric and adult celiac patients could manifest a higher frequency of esophagitis and gastroesophageal reflux disease-related symptoms compared to nonceliac patients. In addition, several published studies have consistently shown the efficacy of a gluten-free diet in rapidly controlling esophageal symptoms and in preventing their recurrence. Since the participation of gluten in the esophageal symptoms of CD seems clear, its intimate mechanisms have yet to be elucidated, and several hypothesis have been proposed, including the specific immune alterations characterizing CD, the reduction in nutrient absorption determining the arrival of intact gluten to distal gastrointestinal segments, and various dysregulations in the function of gastrointestinal hormones and peptides. Recent studies have suggested the existence of a possible relationship between CD and eosinophilic esophagitis, which should be more deeply investigated. PMID:21438963

Lucendo, A J

2011-03-25

50

Neurologic manifestations of angelman syndrome.  

PubMed

Angelman syndrome is a neurogenetic disorder characterized by the loss or reduction of the ubiquitin-protein ligase E3A enzyme. Angelman syndrome results from a deletion or mutation of the maternally inherited 15q11.2-13.1 region, paternal uniparental disomy of chromosome 15, or an imprinting error. Epilepsy is common and may present with multiple seizure types, including nonconvulsive status epilepticus. Seizures are often intractable and typically require broad-spectrum antiepileptic medications. Dietary therapy has also proved successful in Angelman syndrome. Electroencephalographic patterns include notched ? and rhythmic ? activity and epileptiform discharges. Sleep disorders are also common, often characterized by abnormal sleep-wake cycles. Movement disorders are nearly universal in Angelman syndrome, most frequently presenting with ataxia and tremor. Neurocognitive impairment is always present to varying degrees, and expressive speech is typically severely affected. Individuals with Angelman syndrome often manifest psychiatric comorbidities including hyperactivity, anxiety, and challenging behaviors such as aggression and self-injury. We focus on a comprehensive whole-child approach to the diagnosis and long-term clinical care of individuals with Angelman syndrome. PMID:23498559

Thibert, Ronald L; Larson, Anna M; Hsieh, David T; Raby, Annabel R; Thiele, Elizabeth A

2013-04-01

51

Skeletal manifestations of infantile scurvy.  

PubMed

Recent investigations of human skeletal material from the historic St. Martin's cemetery, England, found a range of abnormal lesions in six infants that are almost certainly related to scurvy. Porous and proliferative bone lesions affecting the cranial bones and scapulae were found, and this paper presents images obtained using both macroscopic and scanning electron microscope examination of the lesions. Previous work on infantile scurvy (Ortner et al., 1997-2001) relied heavily on changes at the sphenoid, which is often missing in archaeological bone, so the identification of changes attributable to scurvy on other cranial bones and the scapulae is encouraging. The ability to recognize changes related to scurvy on a range of bones will ensure an enhanced potential for recognition of this disease in future research involving archaeological bone. Research on historical documents from Birmingham dating to the eighteenth and nineteenth centuries, combined with the probable cases of scurvy identified, supports the view that the paucity of cases of infantile scurvy from the archaeological record reflects a lack of understanding and recognition of bone manifestations, rather than a lack of occurrence in this period. Changes linked to scurvy were only found in infants from the poorer sections of the community from St. Martin's, and this is almost certainly linked to patterns of food consumption and may be related to shortages of potatoes, due to blight, experienced during this period. PMID:16323179

Brickley, Megan; Ives, Rachel

2006-02-01

52

[Retinal manifestations of infectious diseases].  

PubMed

The retina and the choroids are richly vascularised structures and can therefore be colonised by germs via the haematogenous route in the course of a systemic infectious disease. The germs responsible for this type of infection can be fungi, viruses, bacteria and parasites. Ocular candidiasis is outstanding amongst these colonisations because of its frequency; it can manifest itself as an endophthalmitis with a slow and hidden course. The so-called ocular histoplasmosis syndrome, although it is infrequent in our setting, is an important cause of choroidal neovascularisation. The viruses that most frequently affect the retina are of the herpes type and can produce devastating symptoms in immunoincompetent patients, named acute retinal necrosis syndrome. Retinitis due to cytomegalovirus is more frequent in immunodepressed patients, as in the case of AIDS, but it must also be contemplated in patients with lymphoma and immunomodulatory treatment. The most frequent bacterial diseases that affect the retina are syphilis and tuberculosis. Disease due to cat scratches, caused by a borrelia, can produce a neuroretinitis. Toxoplasmosis is the most common of the infectious diseases caused by a parasite and gives rise to chorioretinitis. Toxocariasis, also caused by a parasite, is second in importance, giving rise to choroidal granulomas and retinal tractions. PMID:19169295

Pérez de Arcelus, M; Salinas, A; García Layana, A

2008-01-01

53

Rheumatological manifestations in diabetes mellitus.  

PubMed

Rheumatological manifestations of Diabetes Mellitus may be classified in: non articular, articular and bone conditions. Among non articular conditions, diabetic cheiroarthropathy, frequent in type I diabetes, the most important disorder related to limited joint mobility, results in stiff skin and joint contractures. Adhesive capsulitis of the shoulder, flexor tenosynovitis, and Duputryen's and Peyronie's diseases are also linked to limited joint mobility. Diffuse skeletal hyperostosis, due to calcification at entheses, is frequent and early, particularly in type 2 diabetes. Neuropathies cause some non articular conditions, mainly neuropathic arthritis, a destructive bone and joint condition more common in type I diabetes. Algodistrophy, shoulder-hand and entrapment syndromes are also frequent. Mononeuropathy causes diabetic amyotrophy, characterised by painless muscle weakness. Among muscle conditions, diabetic muscle infarction is a rare, sometimes severe, condition. Among articular conditions, osteoarthritis is frequent and early in diabetes, in which also chondrocalcinosis and gout occur. Rheumatoid arthritis (RA) and diabetes I have a common genetic background and the presence of diabetes gives to RA an unfavourable prognosis. Among bone conditions, osteopenia and osteoporosis may occur early in type 1 diabetes. Contrarily, in type 2 diabetes, bone mineral density is similar or, sometimes, higher than in non diabetic subjects, probably due to hyperinsulinemia. PMID:18220648

Del Rosso, Angela; Cerinic, Marco Matucci; De Giorgio, Francesca; Minari, Chiara; Rotella, Carlo Maria; Seghier, Giuseppe

2006-11-01

54

Skin manifestations of drug allergy.  

PubMed

Cutaneous adverse drug reactions range from mild to severe and from those localized only to skin to those associated with systemic disease. It is important to distinguish features of cutaneous drug reactions which help classify the underlying mechanism and likely prognosis as both of these influence management decisions, some of which necessarily have to be taken rapidly. Severe cutaneous reactions are generally T cell-mediated, yet this immunological process is frequently poorly understood and principles for identification of the culprit drug are different to those of IgE mediated allergic reactions. Furthermore, intervention in severe skin manifestations of drug allergy is frequently necessary. However, a substantial literature reports on success or otherwise of glucocorticoids, cyclophsphamide, ciclosporin, intravenous immunoglobulin and anti-tumour necrosis factor therapy for the treatment of toxic epidermal necrolysis without clear consensus. As well as reviewing the recommended supportive measures and evidence base for interventions, this review aims to provide a mechanistic overview relating to a proposed clinical classification to assist the assessment and management of these complex patients. PMID:21480947

Ardern-Jones, Michael R; Friedmann, Peter S

2011-05-01

55

Skin manifestations of drug allergy  

PubMed Central

Cutaneous adverse drug reactions range from mild to severe and from those localized only to skin to those associated with systemic disease. It is important to distinguish features of cutaneous drug reactions which help classify the underlying mechanism and likely prognosis as both of these influence management decisions, some of which necessarily have to be taken rapidly. Severe cutaneous reactions are generally T cell-mediated, yet this immunological process is frequently poorly understood and principles for identification of the culprit drug are different to those of IgE mediated allergic reactions. Furthermore, intervention in severe skin manifestations of drug allergy is frequently necessary. However, a substantial literature reports on success or otherwise of glucocorticoids, cyclophsphamide, ciclosporin, intravenous immunoglobulin and anti-tumour necrosis factor therapy for the treatment of toxic epidermal necrolysis without clear consensus. As well as reviewing the recommended supportive measures and evidence base for interventions, this review aims to provide a mechanistic overview relating to a proposed clinical classification to assist the assessment and management of these complex patients.

Ardern-Jones, Michael R; Friedmann, Peter S

2011-01-01

56

[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

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

Manifestations dermatologiques du syndrome des antiphospholipides  

Microsoft Academic Search

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may

C. Francès; S. Barete; A. Soria

59

Hematological Manifestations of Primary Mitochondrial Disorders  

Microsoft Academic Search

At onset mitochondrial disorders (MID) frequently manifest as a mono-organic problem but turn into multisystem disease during the disease course in most of the cases. Organs\\/tissues most frequently affected in MID are the cerebrum, peripheral nerves, and the skeletal muscle. Additionally, most of the inner organs may be affected alone or in combination. Hematological manifestations of MID include aplastic, megaloblastic,

Josef Finsterer

2007-01-01

60

Augmenting SCORM manifests with adaptive links  

Microsoft Academic Search

This paper describes an experiment to augment SCORM manifests with adaptive links using AuldLinky in order to promote content reusability, interoperability and personalized e-learning. Our technique involves the automatic deduction of a concept map from a manifest and the transformation of its pertinent elements into FOHM (Fundamental Object Hypermedia Model) objects before augmenting the information with complimentary and adaptive links

Nor Aniza Abdullah; Christopher Bailey; Hugh C. Davis

2004-01-01

61

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

62

21 CFR 11.50 - Signature manifestations.  

Code of Federal Regulations, 2010 CFR

...2010-04-01 false Signature manifestations. 11...DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL...RECORDS; ELECTRONIC SIGNATURES Electronic Records...associated with the signature. (b) The items...included as part of any human readable form of...

2010-04-01

63

21 CFR 11.50 - Signature manifestations.  

Code of Federal Regulations, 2010 CFR

...2009-04-01 false Signature manifestations. 11...DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL...RECORDS; ELECTRONIC SIGNATURES Electronic Records...associated with the signature. (b) The items...included as part of any human readable form of...

2009-04-01

64

Dermatitis Herpetiformis: Skin Manifestation of Celiac Disease  

MedlinePLUS

... of Celiac Disease Dermatitis Herpetiformis: Skin Manifestation of Celiac Disease On this page: Symptoms Causes Diagnosis Treatment References ... University of Utah School of Medicine. [ Top ] The Celiac Disease Awareness Campaign The National Institutes of Health Celiac ...

65

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

66

Dengue hemorrhagic fever: Clinical manifestations and management  

Microsoft Academic Search

Dengue virus infection may remain asymptomatic or manifest as nonspecific viral infection to life threatening dengue hemorrhagic\\u000a fever (DHF)\\/dengue shock syndrome (DSS). Patients with DHF\\/DSS have fever, hemorrhagic manifestations along with thrombocytopenia\\u000a and hemoconcentration. Thrombocytopenia and hemoconcentration are distinguishing features between DHF\\/DSS and dengue fever\\u000a (DF). Some patients with dengue fever may have significant bleed and mild thrombocytopenia but no

S. K. Kabra; Y. Jain; T. Singhal; V. H. Ratageri

1999-01-01

67

40 CFR 263.20 - The manifest system.  

Code of Federal Regulations, 2013 CFR

...HAZARDOUS WASTE Compliance With the Manifest System and Recordkeeping § 263.20 The manifest system. (a)(1) Manifest requirement... (2) The transporter records, on a log or shipping paper, the following...

2013-07-01

68

Extra-articular Manifestations in Rheumatoid Arthritis  

PubMed Central

ABSTRACT Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main characteristic is persistent joint inflammation that results in joint damage and loss of function. Although RA is more common in females, extra-articular manifestations of the disease are more common in males. The extra-articular manifestations of RA can occur at any age after onset. It is characterised by destructive polyarthritis and extra-articular organ involvement, including the skin, eye, heart, lung, renal, nervous and gastrointestinal systems. The frequence of extra-articular manifestations in RA differs from one country to another. Extra-articular organ involvement in RA is more frequently seen in patients with severe, active disease and is associated with increased mortality. Incidence and frequence figures for extra-articular RA vary according to study design. Extra-articular involvement is more likely in those who have RF and/or are HLA-DR4 positive. Occasionally, there are also systemic manifestations such as vasculitis, visceral nodules, Sjögren's syndrome, or pulmonary fibrosis present. Nodules are the most common extra-articular feature, and are present in up to 30%; many of the other classic features occur in 1% or less in normal clinic settings. Sjögren's syndrome, anaemia of chronic disease and pulmonary manifestations are relatively common – in 6-10%, are frequently present in early disease and are all related to worse outcomes measures of rheumatoid disease in particular functional impairment and mortality. The occurrence of these systemic manifestations is a major predictor of mortality in patients with RA. This paper focuses on extra-articular manifestations, defined as diseases and symptoms not directly related to the locomotor system.

Cojocaru, Manole; Cojocaru, Inimioara Mihaela; Silosi, Isabela; Vrabie, Camelia Doina; Tanasescu, R

2010-01-01

69

[Skin manifestations of pediatric inflammatory bowel disease].  

PubMed

Skin manifestations have been described in 10-15% of patients with inflammatory bowel disease (Crohn's disease, ulcerative colitis, indeterminate colitis/inflammatory bowel disease type unclassified). There are limited data on the prevalence of these manifestations in paediatric patients, but recent studies have reported its presence in 8% of them at diagnosis. Our aim is to report the different skin manifestations observed in our paediatric patients with inflammatory bowel disease. Case 1: Erythema nodosum at Crohn's disease diagnosis. Typical presentation with painful erythematous nodules in the pretibial region and with good response to infliximab. Case 2: Coexistence of pyoderma gangrenosum and mucocutaneous Sweet's syndrome in a Crohn's disease patient. A rapidly progressive disease that was controlled with systemic steroids but with significant residual lesions. Case 3 and 4: Metastatic Crohn's disease with good response to infliximab. PMID:19446510

Martín de Carpi, J; Chávez Caraza, K; Vicente Villa, M A; González Enseñat, M A; Vilar Escrigas, P; Vila Miravet, V; Guallarte Alias, P; Varea Calderón, V

2009-05-14

70

[Dermatologic manifestations of the antiphospholipid syndrome].  

PubMed

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies. PMID:22078802

Francès, C; Barete, S; Soria, A

2011-11-10

71

Mitochondrial Disorders with Significant Ophthalmic Manifestations  

PubMed Central

Mitochondrial diseases are a clinically hetyerogenous group of disorders. They can be caused by mutations of nuclear or mitochondrial DNA (mtDNA). Some affect a single organ, but many involve multiple organ systems and often present with prominent neurologic and myopathic features. The eye is frequently affected, along with muscles and brain, but multisystem disease is common. Ophthalmic manifestations include cataract, retinopathy, optic atrophy, cortical visual loss, ptosis and ophthalmoplegia. Kearns-Sayre Syndrome (KSS), Mitochondrial Encephalopathy, Lactic Acidosis Stroke (MELAS), Myoclonic Epilepsy and Ragged Red Fiber myopathy (MERRF) and Lebers Hereditary Optic Neuropathy (LHON) are well known clinical entities that are secondary to mtDNA abnormalities, which has ophthalmic manifestations. Mitochondrial Dysfunction should be considered in the differential diagnosis of progressive multisystem disorder and specifically if there is associated neuro-ophthalmic manifestations, which may be the presenting symptom of these disorders.

Al-Enezi, Mona; Al-Saleh, Hanan; Nasser, Murad

2008-01-01

72

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

73

Neurologic Manifestations of Leishmania spp. Infection  

PubMed Central

When listing common clinical signs of the spectra of Leishmania-derived diseases, neurologic malfunctions are not commonly included. Despite this, there are multiple reported instances both in human and veterinary medicine where neurologic manifestations, whether central or peripheral, are described. In this review, we describe neurologic manifestations seen during infection with Leishmania spp. with some discussion of the implicit effect of inflammation on the blood brain barrier in both medical and veterinary cases. Taken together, the material discussed here suggests that in patients from Leishmania-endemic areas, when observing neurologic symptoms, causation secondary to infection with Leishmania spp. should be highly considered.

Petersen, Christine Anne; Greenlee, M. Heather West

2011-01-01

74

Electrocardiographic manifestations: narrow QRS complex tachycardias  

Microsoft Academic Search

Narrow QRS complex tachycardia is a common dysrhythmia in Emergency Medicine practice. Diagnosis and mechanism often can be made by 12-lead electrocardiographic (EKG) analysis but may subsequently require electrophysiologic testing. The clinical manifestations are varied and dependent upon heart rate, prior cardiac disease, and general physiologic status. Patient management is directed towards the etiology and mechanism of the dysrhythmia and

Marc L Pollack; William J Brady; Theodore C Chan

2003-01-01

75

The extrahepatic manifestations of hepatitis B virus.  

PubMed

Hepatitis B Virus (HBV) leads to a number of hepatic complications, from acute to chronic hepatitis, cirrhosis and hepatocellular carcinoma, is a well-established fact. Upcoming clinical research, over the years, associates numerous extrahepatic manifestations during the acute and chronic episodes of hepatitis B with significant morbidity and mortality. A causal relationship between HBV and serious autoimmune disorders has also been observed among certain susceptible vaccine recipients in a defined temporal period following immunization. The cause of these extrahepatic manifestations is generally believed to be immune mediated. The most commonly described include skin rash, arthritis, arthralgia, glomerulonephritis, polyarteritis nodosa, and papular acrodermatitis etc. The serum-sickness like "arthritis-dermatitis" prodrome has also been observed in approximately one-third of patients acquiring HBV infections. Skin manifestations of HBV infection typically present as palpable purpura reported to be caused by chronic HBV, although this association remains controversial. To consider the relationship between HBV and other clinically significant disorders as well as serious autoimmune disorders among certain vaccine recipients is the topic of this review. Variable factors that influence extrahepatic manifestation are discussed, including possible synergy between hepatitis B virus and the immune system. PMID:18760074

Baig, Saeeda; Alamgir, Mohiuddin

2008-07-01

76

Unusual manifestation of Marden-Walker syndrome.  

PubMed

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. PMID:23162309

Taksande, Amar M; Vilhekar, K Y

2012-05-01

77

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

78

Rheumatic manifestations of inflammatory bowel disease  

PubMed Central

This article reviews the literature concerning rheumatic manifestations of inflammatory bowel disease (IBD), including common immune-mediated pathways, frequency, clinical course and therapy. Musculoskeletal complications are frequent and well-recognized manifestations in IBD, and affect up to 33% of patients with IBD. The strong link between the bowel and the osteo-articular system is suggested by many clinical and experimental observations, notably in HLA-B27 transgenic rats. The autoimmune pathogenic mechanisms shared by IBD and spondyloarthropathies include genetic susceptibility to abnormal antigen presentation, aberrant recognition of self, the presence of autoantibodies against specific antigens shared by the colon and other extra-colonic tissues, and increased intestinal permeability. The response against microorganisms may have an important role through molecular mimicry and other mechanisms. Rheumatic manifestations of IBD have been divided into peripheral arthritis, and axial involvement, including sacroiliitis, with or without spondylitis, similar to idiopathic ankylosing spondylitis. Other periarticular features can occur, including enthesopathy, tendonitis, clubbing, periostitis, and granulomatous lesions of joints and bones. Osteoporosis and osteomalacia secondary to IBD and iatrogenic complications can also occur. The management of the rheumatic manifestations of IBD consists of physical therapy in combination with local injection of corticosteroids and nonsteroidal anti-inflammatory drugs; caution is in order however, because of their possible harmful effects on intestinal integrity, permeability, and even on gut inflammation. Sulfasalazine, methotrexate, azathioprine, cyclosporine and leflunomide should be used for selected indications. In some cases, tumor necrosis factor-? blocking agents should be considered as first-line therapy.

Rodriguez-Reyna, Tatiana Sofia; Martinez-Reyes, Cynthia; Yamamoto-Furusho, Jesus Kazuo

2009-01-01

79

Dyslexia in Regular Orthographies: Manifestation and Causation  

ERIC Educational Resources Information Center

|This article summarizes our research on the manifestation of dyslexia in German and on cognitive deficits, which may account for the severe reading speed deficit and the poor orthographic spelling performance that characterize dyslexia in regular orthographies. An only limited causal role of phonological deficits (phonological awareness,…

Wimmer, Heinz; Schurz, Matthias

2010-01-01

80

Dyslexia in Regular Orthographies: Manifestation and Causation  

ERIC Educational Resources Information Center

This article summarizes our research on the manifestation of dyslexia in German and on cognitive deficits, which may account for the severe reading speed deficit and the poor orthographic spelling performance that characterize dyslexia in regular orthographies. An only limited causal role of phonological deficits (phonological awareness,…

Wimmer, Heinz; Schurz, Matthias

2010-01-01

81

Gestural Manifestation of Knowledge in Conceptual Frames  

Microsoft Academic Search

Frames are cognitive structures of knowledge grounded in people's social interaction in recurrent socio-cultural 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 knowledge in conceptual frames. The present study provides empirical evidence that frame knowledge

Kawai Chui

2012-01-01

82

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

83

Oral manifestation of cleido cranial displasia.  

PubMed

Cleidocranial dysplasia (CCD) is a congenital hereditary condition caused by a dominant autosomal mutation. The orthodontic management of CCD patients is often complicated by the manifestations associated with the condition. The aim of this study was to evaluate the oral manifestations of patients suffering from CCD. The subjects underwent a thorough clinical evaluation that examined both hard and soft tissue. The subjects also underwent an X-ray examination in order to isolate possible anomalies in the stomach region related to the condition. Case 1 presented with a bilateral cross-bite, retention of deciduous teeth, presence of supernumerary teeth and agenesis. Case 2 presented with bilateral cross-bite, retention of deciduous teeth, and the presence of supernumerary teeth. The individuals in this study represent a broad range of typical CCD manifestations and confirm the close correlation between the mutation at CBFA1 level and the presence or oral-dental alterations. An early diagnosis of CCD is essential and based on clinical manifestations, x-ray evidence and genetic tests. Diagnosing the condition can be difficult in cases where there are no evident morphological signs; however, in most cases, an alteration in the rhythm of deciduous tooth loss is immediately apparent, making a closer collaboration between dentists and geneticists fundamental in achieving early diagnosis. As stated above, the timing of the treatment is crucial in establishing a correct course of treatment that involves extractions, orthodontic surgery and the use of prosthetics. PMID:23076024

Sberna, M T; De Angelis, D; Laruffa, F; Tettamanti, L; Storti, E

2012-10-01

84

[Depressive schizoaffective disorder (typology and manifest episodes)].  

PubMed

A group of patients (n=48) with a manifestation of depressive schizoaffective disorder (SAD) (F25.1 ICD-10) was studied. The considerable frequency (79.2%) of pre-manifest affective disorders, which preceded the development of psychosis in overwhelming majority of cases (81.6%), was found. In accordance with the modality of the affect, pre-manifest affective disorders were attributed to depression (60.5%), bipolar affective disorder (31.6%), with dual phases, or, more rarely, with continuous course, and hypomania (7.9%). The considerable frequency (47.9%) of hypomania, bipolar disorder, mixed depression, alternating variants of mixed disorders was found at the active stage of current depressive SAD, in the end of the attack or in the next post-psychotic period without concomitant positive symptoms. Depending on the number of psychopathological syndromes, authors singled out mono- and polymorphic structures of a manifestation of depressive SAD. A number of significant clinical features inherent to the polymorphic clinical structure of depressive SAD, including those with mixed affective disorders, was noted compared to the monomorphic structure of the attack. PMID:23612405

Bobrov, A S; Rozhkova, M Iu; Rozhkova, N Iu

2013-01-01

85

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

86

The neuropsychiatric manifestations of Lyme borreliosis  

Microsoft Academic Search

Lyme borreliosis (Lyme disease), a tick-borne spirochetal illness, has multi-systemic involvement and is rapidly increasing in certain areas of the United States. Although its neurologic manifestations are becoming increasingly well recognized, its psychiatric presentations are not well known. The first section of this paper will provide an overview of Lyme borreliosis and a review of the relevant neuropsychiatric literature. The

Brian A. Fallon; Jenifer A. Nields; Joseph J. Burrascano; Kenneth Liegner; Donato DelBene; Michael R. Liebowitz

1992-01-01

87

Musculoskeletal manifestations in hyperlipidaemia: a controlled study  

Microsoft Academic Search

Eighty eight patients with hyperlipidaemia (81 white patients from South Africa and seven patients of mixed race from the West Cape area) were studied. Forty eight had adult familial hypercholesterolaemia, 16 had juvenile familial hypercholesterolaemia, and 24 had mixed hyperlipidaemia (increased cholesterol and triglycerides). They were interviewed and examined and their musculoskeletal manifestations compared with 88 controls with normal lipid

P Klemp; A M Halland; F L Majoos; K Steyn

1993-01-01

88

Neurological manifestations in systemic sclerosis (scleroderma).  

PubMed

One hundred and twenty-five patients with systemic sclerosis were surveyed for neurological manifestations in a prospective study. Seven (5.6%) were found to have a defined neurologic lesion: 4 with carpal tunnel syndrome and one each with trigeminal neuralgia, mononeuritis multiplex and peripheral neuropathy. Neurological involvement occurs but is uncommon in this connective tissue disorder. PMID:6090661

Lee, P; Bruni, J; Sukenik, S

1984-08-01

89

Real-Life Contextual Manifestations of Wisdom  

ERIC Educational Resources Information Center

Wisdom pertains to managing human affairs, and it arises in highly contextualized situations. The present study aims to investigate manifestations of wisdom in real-life contexts through semi-structured interviews with 66 individuals nominated as wise persons. All nominees were ethnic Chinese from Taiwan, an East Asian country which has…

Yang, Shih-Ying

2008-01-01

90

Systemic lupus erythematosus and myoclonic epileptic manifestations.  

PubMed

Systemic lupus erythematosus (SLE) frequently involves the central nervous system (CNS) and, in fact, epileptic manifestations may be one of the earliest symptoms of SLE. These early occurrences of epilepsy, however, can easily be misdiagnosed as indication of pure epileptic syndrome when the SLE diagnosis is still largely incomplete. We present a young girl who developed myoclonic photosensitive seizures at the onset of the illness, erroneously diagnosed as manifestation of a "pure" epileptic syndrome. Shortly after the onset of an anticonvulsant therapy (lamotrigine), there was a remarkable impairment of the general clinical condition: at that time a diagnosis of SLE was made and a specific treatment began. However, the seizures persisted and evolved toward status epilepticus which needed pentobarbitone therapy in an intensive care unit (ICU). After recovery, the girl gradually got better and during the 23 months of follow-up she received only corticosteroid therapy and did not experience seizures nor SLE relapses. PMID:10933433

Mecarelli, O; de Feo, M R; Accornero, N; Paffetti, A

1999-04-01

91

The multiple hematologic manifestations of neuroblastoma.  

PubMed

The hematologic manifestations of neuroblastoma are numerous and varied. Bone marrow invasion by tumor cells may cause leukoerythroblastic changes or depression of one or more of the cell lines in the peripheral blood; occasionally bone marrow involvement may be so extensive that tumor cells may be released into the peripheral blood and lead to an erroneous diagnosis of leukemia. Anemia in neuroblastoma patients may result not only from bone marrow involvement, but also from bleeding into a tumor mass or from the hemolysis accompanying a consumption coagulopathy. A specific morphologic abnormality, the cogwheel erythrocyte, has been reported in patients with neuroblastoma. Neuroblastoma may also be associated with elevation of the platelet count or a hypercoagulable state. Recognition of these protean hematologic manifestations may facilitate diagnosis in children with atypical presentations of this highly malignant tumor. PMID:543514

Quinn, J J; Altman, A J

1979-01-01

92

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

93

Biliary tract manifestations of the Marfan syndrome.  

PubMed

The diagnosis of the Marfan syndrome rests on the four criteria of characteristic musculoskeletal, cardiovascular, ocular, and familial features. Biliary tract manifestations have not been described. A case of Marfan syndrome with recurrent biliary obstruction is presented. Endoscopic retrograde cholangiograms presented a grossly ectatic biliary system which is postulated to be an unrecognized manifestation of this syndrome. In addition, the patient developed choledocholithiasis, which may have resulted from biliary stasis secondary to bile duct ectasia. Biliary obstruction developed in the presence and absence of choledocholithiasis, the latter apparently through kinking of the ectatic common bile duct. Obstruction was clinically relieved by biliary endoprosthesis. The presence of a Zenker's diverticulum in this patient, also unreported in the Marfan syndrome, is of incidental interest. PMID:3605039

Merza, A P; Raiser, M W

1987-08-01

94

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

95

Oral manifestations of triple A syndrome.  

PubMed

Triple A (four A; Allgrove syndrome) syndrome is a an autosomal recessive disorder characterized by alacrimia, achalasia, adrenocortical insufficiency, and various neurological abnormalities. We report a case of triple A syndrome in a 14-year-old female patient together with oral manifestations. The importance of this case report is to highlight the necessity of referring the young patients with xerostomia from dental to medical specialists. PMID:12911870

Vucicevic-Boras, V; Juras, D; Gruden-Pokupec, J S; Vidovic, A

2003-07-31

96

Manifestation peculiarities of idiopathic chronic eosinophilic pneumonia  

Microsoft Academic Search

Chronic eosinophilic pneumonia is a rare interstitial lung disorder, which causes diagnostic difficulties. Often the disease\\u000a is diagnosed correctly after several weeks or months following initial presentation. The aim of the study was to prospectively\\u000a evaluate peculiarities of manifestation of idiopathic chronic eosinophilic pneumonia (ICEP), which may allow to improving\\u000a early diagnosis. Twenty patients with ICEP were involved in this

Edvardas Danila; Jolita Nork?nien?; Remigijus Narg?la; Edvardas Žurauskas; Bronislovas Šatkauskas; Regina Aleksonien?

2010-01-01

97

Ocular manifestations of the antiphospholipid syndrome  

Microsoft Academic Search

Antiphospholipid syndrome (APS) is an autoimmune disease characterised by a heterogenous group of antibodies directed against negatively charged phospholipids including antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) and ?-2 glycoprotein I (a?-2-GP1). The major features of this disorder include arterial and venous thrombosis and recurrent fetal loss. The vasculature of the eye is frequently involved and may be the presenting manifestation.

Virginia Miraldi Utz; Johnny Tang

2010-01-01

98

Turcot syndrome and its characteristic colonic manifestations  

Microsoft Academic Search

The reported clinical manifestations of Turcot syndrome were studied to determine whether these corresponded to those of Turcot's\\u000a original cases. Among the patients with well-documented colonic lesions, the colonic lesions were classified into three groups.\\u000a First, there was a main group in which colonic lesions had the following characteristics that coincided with those of Turcot's\\u000a original cases: 1) a low

H. I-I-Oil; K. Ohsato

1985-01-01

99

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

100

Atypical manifestations of multiple myeloma: radiological appearance.  

PubMed

Diagnostic procedures performed on patients with multiple myeloma typically reveal lytic bone lesions, osteopenia or osteoporosis, bone marrow infiltration by plasma cells as well as overproduction of immunoglobulin or light chains in the serum or urine. Skeletal manifestations are extremely variable and the unusual forms have been described extensively. Extramedullary plasma-cell tumours (plasmocytoma) are found in about 5% of newly diagnosed patients with multiple myelomas. In this paper we present eight patients with atypical forms of multiple myeloma. PMID:16431064

Hess, Thomas; Egerer, Gerlinde; Kasper, Bernd; Rasul, Kakil Ibrahim; Goldschmidt, Hartmut; Kauffmann, G W

2006-01-23

101

LYMPHOBLASTOMAS IN CHILDHOOD--Cutaneous Manifestations  

PubMed Central

The lymphoblastomas occurring in childhood are divided for purposes of discussion into lymphocytoma cutis, mycosis fungoides, lymphosarcoma, Hodgkin's disease, and leukemia. The cutaneous lesions may be either specific (as a result of the infiltration of the skin with specific cells of the conditions) or toxic (non-specific). With the possible exception of mycosis fungoides, the cutaneous manifestations are not diagnostic. The final diagnosis depends upon microscopic examination of the specific tissue involved and the coordination of the clinical and microscopic findings.

Nelson, Lawrence M.

1953-01-01

102

Manifestly covariant Jüttner distribution and equipartition theorem  

NASA Astrophysics Data System (ADS)

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.

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

2010-02-01

103

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

104

Central nervous system manifestations of mitochondrial disorders.  

PubMed

The central nervous system (CNS) is, after the peripheral nervous system, the second most frequently affected organ in mitochondrial disorders (MCDs). CNS involvement in MCDs is clinically heterogeneous, manifesting as epilepsy, stroke-like episodes, migraine, ataxia, spasticity, extrapyramidal abnormalities, bulbar dysfunction, psychiatric abnormalities, neuropsychological deficits, or hypophysial abnormalities. CNS involvement is found in syndromic and non-syndromic MCDs. Syndromic MCDs with CNS involvement include mitochondrial encephalomyopathy, lactacidosis, stroke-like episodes syndrome, myoclonic epilepsy and ragged red fibers syndrome, mitochondrial neuro-gastrointestinal encephalomyopathy syndrome, neurogenic muscle weakness, ataxia, and retinitis pigmentosa syndrome, mitochondrial depletion syndrome, Kearns-Sayre syndrome, and Leigh syndrome, Leber's hereditary optic neuropathy, Friedreich's ataxia, and multiple systemic lipomatosis. As CNS involvement is often subclinical, the CNS including the spinal cord should be investigated even in the absence of overt clinical CNS manifestations. CNS investigations comprise the history, clinical neurological examination, neuropsychological tests, electroencephalogram, cerebral computed tomography scan, and magnetic resonance imaging. A spinal tap is indicated if there is episodic or permanent impaired consciousness or in case of cognitive decline. More sophisticated methods are required if the CNS is solely affected. Treatment of CNS manifestations in MCDs is symptomatic and focused on epilepsy, headache, lactacidosis, impaired consciousness, confusion, spasticity, extrapyramidal abnormalities, or depression. Valproate, carbamazepine, corticosteroids, acetyl salicylic acid, local and volatile anesthetics should be applied with caution. Avoiding certain drugs is often more beneficial than application of established, apparently indicated drugs. PMID:16942541

Finsterer, J

2006-10-01

105

Determinants of systemic manifestations of food allergy.  

PubMed

The myriad of systemic manifestations induced by food hypersensitivity responses is testament to the ability of localized exposure to foods in the gastrointestinal tract to result in symptoms in distal target organs. Cow's milk protein, for example, may induce hives (urticaria), atopic dermatitis, isolated gastrointestinal symptoms, or severe generalized anaphylaxis in different individuals or in the same person at different times. These diverse manifestations are the result of complex interactions among the causal food protein, gut, immune system, and target organs. The dynamic state of these interactions is demonstrated by the development of food tolerance in most subjects and by the ability to experience the development of new allergies in some subjects. This review explores the variety of clinical manifestations of food hypersensitivity disorders in the context of the question: What determines the local or systemic expression of food allergy in a given individual at a particular time? Evidence is provided for both systemic and local immune activation. The role of food-protein chemistry, absorption and processing of ingested allergen, immune responses (type, degree, and specificity), and target organ hyperreactivity are considered as determinants in the expression of food allergic disorders. PMID:11080740

Sicherer, S H

2000-11-01

106

Upper airway manifestations of granulomatosis with polyangiitis.  

PubMed

Upper airway manifestations, particularly sinonasal manifestations, are encountered frequently in granulomatosis with polyangiitis (GPA). Nasal endoscopy often reveals crusting, friable erythematous mucosa, and granulation. Up to 25% of patients may have a "saddle-nose" deformity as cartilage destruction worsens. Treatment is often complicated by loss of mucociliary function and necrosis, leading to refractory symptoms. Culture-directed antibiotics, topical antibiotic and saline irrigations, and occasional debridement of adherent crusts can reduce the frequency of sinonasal exacerbations and improve obstructive symptoms. Surgery should be reserved for patients unresponsive to maximal medical therapy. Saddle-nose reconstruction is possible in highly selected patients and can improve nasal breathing and resolve anosmia. Up to 20% of patients with GPA have subglottic stenosis; patients with respiratory symptoms should undergo laryngoscopy to assess the presence of subglottic narrowing. Although systemic manifestations of GPA are managed by immunosuppressive therapy, most patients with subglottic stenosis of GPA require surgical management (ie, endoscopic dilation, endoscopic or laser excision, surgical resection followed by reconstruction). PMID:23203639

Alam, Daniel S; Seth, Rahul; Sindwani, Raj; Woodson, Erika A; Rajasekaran, Karthik

2012-11-01

107

Hypoperfusive and hypertensive ocular manifestations in Takayasu arteritis  

PubMed Central

Takayasu arteritis is a relatively rare inflammatory arteritis that can be associated with ocular manifestations. We report four patients with proven Takayasu arteritis; two patients manifested hypoperfusive ocular manifestations of ocular ischemic syndrome and anterior ischemic optic neuropathy whilst two others had exudative retinal detachment and papilledema as a result of severe hypertension. The ischemic ocular manifestations were a result of hypoperfusion of the ocular structures due to occlusive arteritis of the aortic arch and its branches. The exudative retinal detachment and papilledema were manifestations of severe hypertension due to renal arterial involvement. Patients with Takayasu arteritis should be referred for ophthalmic assessment and screening for hypoperfusive and hypertensive manifestations.

Peter, Jayanthi; David, Sarada; Joseph, George; Horo, Saban; Danda, Debashish

2010-01-01

108

40 CFR 761.214 - Retention of manifest records.  

Code of Federal Regulations, 2013 CFR

...POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION...retain a copy of the shipping paper containing all the information...of the manifest and shipping paper with all the information required...manifest (or the shipping paper if signed by the...

2013-07-01

109

40 CFR 761.211 - Manifest system-Transporter requirements.  

Code of Federal Regulations, 2013 CFR

...POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION...facility; and (2) A shipping paper containing all the information...the manifest or the shipping paper; and (4) The person...5) A copy of the shipping paper or manifest is retained by...

2013-07-01

110

[Hereditary hemochromatosis: Presenting manifestations and diagnostic delay.  

PubMed

PURPOSE: Hereditary hemochromatosis is characterized by an excessive absorption and progressive accumulation of iron in the liver, the pancreas, the heart, and the joints. Tiredness and joint manifestations occur usually before hepatopathy, diabetes or cardiopathy. Such common and unspecific symptoms seem to be largely unknown and important diagnostic delays have been reported. The aim of this study was to investigate the discovery circumstances and the diagnostic delay. METHODS: A survey was carried out amongst French patients with C282Y homozygous hemochromatosis who were contacted through patients associations or blood centers. RESULTS: The questionnaire was answered by 374 patients. Mean age at diagnosis was 48.6±11.9years. In 53% of the cases, the serum level of ferritin was greater than 1000 ?g/L. Diagnosis was based on family genetic survey (29%), or fortuitous analyses showing an abnormal serum ferritin (26%), or clinical manifestations (45%). Main complaints were joint pain, tiredness or liver disease. Only 2.1% consulted for diabetes, cardiopathy or changed complexion. Time to diagnosis was lower than 1 year for 98% of patients who presented with fatigue but from 1 to 15 years for 23.4% and 29% of patients who presented with arthropathy and hepatopathy, respectively. CONCLUSION: For 55% of patients, diagnosis was based on familial genetic survey or fortuitous abnormal results of blood samples. An initial serum level of ferritin greater than 1000 ?g/L was a factor of severity for 50% of patient. These two elements must be taken into account to consider a population mass screening. Long time to diagnosis required a sensitization of the population to be aware of the clinical manifestations of hemochromatosis. PMID:23566434

Gasser, B; Courtois, F; Hojjat-Assari, S; Sauleau, E A; Buffet, C; Brissot, P

2013-04-01

111

Cutaneous manifestations of category A bioweapons.  

PubMed

The 2001 anthrax attacks on the United States brought bioterrorism to the forefront of the medical community. Because many bioterrorist agents produce cutaneous disease, dermatologists will likely be first responders during a future attack. Despite this, many dermatologists are not adequately prepared to deal with a bioterrorism attack. It is critical that all first responders be able to recognize symptoms of bioterrorism-related disease and prepared to respond to a bioterrorist attack to minimize threats to public health. This article reviews the diagnosis and treatment of diseases caused by potential biowarfare agents that produce cutaneous manifestations, and also provides information regarding reporting and containment of possible bioterrorism-related diseases. PMID:21777993

Aquino, Lisa L; Wu, Jashin J

2011-07-20

112

[Sjögren's syndrome: diagnosis and systemic manifestations].  

PubMed

Sjögren's syndrome is an autoimmune exocrinopathy characterized by keratoconjunctivis sicca, xerostomia and immune-inflammatory systemic manifestations. The diagnosis is easy to establish when the patient presents with sicca complex as a main symptom, or recurring attacks of parotitis. However, it is way more complex when the disease begins with extraglandular features, such as non erosive polyarticular arthritis, Raynaud's phenomenon, peripheral or central nervous system involvement, kidney disease or interstitial pneumonary disease, or even vasculitis. In such circumstances, diagnosis is often delayed several years. PMID:22408869

Hatron, Pierre-Yves

2012-02-01

113

Manifestations of systemic diseases on thoracic imaging.  

PubMed

A familiarity with the numerous findings in commonly encountered systemic diseases is necessary for the radiologist interpreting any chest study. These systemic diseases include collagen vascular diseases, vasculitides, granulomatous diseases, neoplasms, hematologic and metabolic abnormalities, neurocutaneous syndromes, deposition disease, and miscellaneous conditions such as cystic fibrosis and Goodpasture's syndrome. Although the imaging findings of these and other recognized systemic diseases are often nonspecific and varied, an understanding of their typical manifestations allows the radiologist to play a significant role in suggesting a particular diagnosis and impacting patient care. PMID:20875612

Kang, James; Litmanovich, Diana; Bankier, Alexander A; Boiselle, Phillip M; Eisenberg, Ronald L

114

[Cardiac manifestations during viral acute hepatitis].  

PubMed

The authors describe a retrospective study conducted on 46 patients with acute viral hepatitis, searching for cardiac disorders. These disorders appeared in about 43% of cases, only with benign evolution. The most frequent alterations are electrocardiographic disorders, followed by conduction blocks, axis deviations and arrhythmias. Acute pericarditis was also described, associated with HCV infection. The viral agents most frequently involved are HBV and HCV, followed by cytomegalovirus, and Epstein-Barr virus. In conclusion, the incidence of cardiac manifestations during viral acute hepatitis is rather high, but with benign evolution. PMID:16794376

Rombola, F; Spinoso, A; Bertuccio, S N

2006-03-01

115

Clinical and genetic characterization of manifesting carriers of DMD mutations  

Microsoft Academic Search

Manifesting carriers of DMD gene mutations may present diagnostic challenges, particularly in the absence of a family history of dystrophinopathy. We review the clinical and genetic features in 15 manifesting carriers identified among 860 subjects within the United Dystrophinopathy Project, a large clinical dystrophinopathy cohort whose members undergo comprehensive DMD mutation analysis. We defined manifesting carriers as females with significant

Payam Soltanzadeh; Michael J. Friez; Diane Dunn; Andrew von Niederhausern; Olga L. Gurvich; Kathryn J. Swoboda; Jacinda B. Sampson; Alan Pestronk; Anne M. Connolly; Julaine M. Florence; Richard S. Finkel; Carsten G. Bönnemann; Livija Medne; Jerry R. Mendell; Katherine D. Mathews; Brenda L. Wong; Michael D. Sussman; Jonathan Zonana; Karen Kovak; Sidney M. Gospe; Eduard Gappmaier; Laura E. Taylor; Michael T. Howard; Robert B. Weiss; Kevin M. Flanigan

2010-01-01

116

Detection of Disease Outbreaks by the Use of Oral Manifestations  

Microsoft Academic Search

Oral manifestations of diseases caused by bioterrorist agents could be a potential data source for biosurveillance. This study had the objectives of determining the oral manifestations of diseases caused by bioterrorist agents, measuring the prevalence of these manifestations in emergency department reports, and constructing and evaluating a detection algorithm based on them. We developed a software application to detect oral

M. H. Torres-Urquidy; G. Wallstrom; T. K. L. Schleyer

2009-01-01

117

Unusual retinal manifestations of cat scratch disease.  

PubMed

We report on 2 patients with unusual retinal manifestations of cat scratch disease (CSD), caused by Bartonella henselae. Case 1. A 42-year-old farmer presented with a 5-day history of blurred vision in his right eye. Right visual acuity was 20/25. Fundus examination of the right eye revealed mild vitreous hemorrhage and diffuse retinal hemorrhages in the mid-peripheral retina. Fluorescein angiography showed multiple vasculitic occlusions in the same area. A blood sample taken on the day of examination revealed the presence of immunoglobulin (Ig)M and IgG to B. henselae. Oral azithromycin was given for 8 days. One month later, right visual acuity was 20/20, the vitreous and retinal hemorrhages resolved, and arteriolar attenuation and sclerosis was observed in the peripheral temporal retina. Case 2. A 66-year-old craftsman with systemic hypertension and hypercholesterolemia complained of sudden visual loss (light perception) in his left eye. Fundus evaluation and fluorescein angiography revealed central retinal artery occlusion (CRAO) in the affected eye. About 2 weeks earlier, he had been bitten and scratched on his right hand by a stray cat. Serologic testing detected the presence of IgM to B. henselae. Oral azithromycin was given for 6 days. One month later, left visual acuity was hand motion. Ophthalmologists should be aware that unusual ocular complications associated with CSD include vitreous hemorrhage with retinal vasculitis and isolated CRAO. Vitreous hemorrhage and retinal vasculitis may be the only clinical manifestation of CSD. PMID:21267628

Pinna, Antonio; Puglia, Ester; Dore, Stefano

2011-01-26

118

Nocturia as a manifestation of systemic disease.  

PubMed

Nocturia is commonly referred to urologists, but the mechanisms underlying the problem, together with the appropriate clinical assessment and management, may lie outside the ordinary scope of the specialty. Some serious conditions may manifest nocturia as an early feature, often as a consequence of nocturnal polyuria (NP). Voiding frequency is influenced by rate of urine output, reservoir capacity of the bladder, lower urinary tract (LUT) sensation and psychological response. Polyuria can result from polydipsia or endocrine dysfunction. NP can result from endogenous fluid and solute shifts, cardiovascular and autonomic disease, obstructive sleep apnoea, and chronic kidney disease. Nocturia without polyuria occurs in the presence of LUT pathology, pelvic masses and sleep disturbance. Drug intake can contribute to, or counteract, each of these problems. In assessing nocturia, clinicians need to consider an undiagnosed serious condition that may manifest nocturia as an early feature, or suboptimal management of a known condition. The frequency-volume chart is a key tool in categorizing the basis of nocturia, identifying those patients with global polyuria or NP, for whom involvement of other specialties is often necessary for assessment and management. Treatment should be directed at the cause of the problem, with a view to improving long-term health and health-related quality of life. Simple steps should be undertaken by all patients, including improvement of the sleep environment and behaviour modification. Evaluation of treatment response requires objective data to corroborate subjective impressions. Some mechanisms of nocturia do not reliably improve with treatment, leading to refractory symptoms. PMID:21355977

Gulur, Dev Mohan; Mevcha, Amit M; Drake, Marcus J

2011-03-01

119

Cutaneous manifestations in patients with POEMS syndrome.  

PubMed

Background? Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia. Methods? From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. We assessed prevalence of skin findings and associations between dermatologic and other characteristic disease findings. Results? Of the 107 patients, 96 (90%) had a recognized cutaneous manifestation. Hyperpigmentation and hemangioma were most common (47%), followed by hypertrichosis (38%). Vascular skin changes - acrocyanosis (34%), Raynaud phenomenon (20%), hyperemia/erythema (20%), flushing (16%), or rubor (11%) - occurred in 62%; white nails, sclerodermoid changes, and clubbing occurred in 30%, 26%, and 6%, respectively. Mean number of skin findings per patient was 2.9 (median, 3.0; range, 0-7). Presence of cutaneous manifestation was associated with abnormal pulmonary function tests (P?

Miest, Rachel Y N; Comfere, Nneka I; Dispenzieri, Angela; Lohse, Christine M; El-Azhary, Rokea A

2013-04-04

120

POLYCYTHEMIA VERA--Peripheral Vascular Manifestations  

PubMed Central

The presenting manifestations of polycythemia vera are often complications involving the vascular system. These include myocardial infarction, cerebro-vascular accidents and ischemic changes in the extremities. The concept of increased atherogenesis in cases of polycythemia vera has been questioned. A possible mechanism by which small, otherwise subclinical atheromatous plaques produce ischemic symptoms in patients with polycythemia vera is discussed. The blood in polycythemic patients has been shown to have an increased viscosity resulting in a prolonged circulation time. If a small atheromatous plaque is present in association with increased blood viscosity, this combination may well produce ischemic symptoms. This explains why treatment of polycythemia vera, with restoration of blood to normal viscosity, often reverses the patient's ischemic symptoms. Two cases of polycythemia vera here reported, in which the presenting manifestations were gangrenous extremities, emphasize the need for prompt diagnosis and treatment of polycythemia vera. In the first case, early recognition and treatment of polycythemia vera successfully reversed the ischemic changes in the extremities, while failure of early recognition and treatment in the second case resulted in two major amputations. ImagesFigure 1.Figure 2.Figure 3.

Moore, Wesley S.; Blaisdell, F. William; Hall, Albert D.

1964-01-01

121

Pulmonary manifestations of gastroesophageal reflux disease  

PubMed Central

Gastroesophageal reflux disease (GERD) may cause, trigger or exacerbate many pulmonary diseases. The physiological link between GERD and pulmonary disease has been extensively studied in chronic cough and asthma. A primary care physician often encounters patients with extra esophageal manifestations of GERD in the absence of heartburn. Patients may present with symptoms involving the pulmonary system; noncardiac chest pain; and ear, nose and throat disorders. Local irritation in the esophagus can cause symptoms that vary from indigestion, like chest discomfort and abdominal pain, to coughing and wheezing. If the gastric acid reaches the back of the throat, it may cause a bitter taste in the mouth and/or aspiration of the gastric acid into the lungs. The acid can cause throat irritation, postnasal drip and hoarseness, as well as recurrent cough, chest congestion and lung inflammation leading to asthma and/or bronchitis/ pneumonia. This clinical review examines the potential pathophysiological mechanisms of pulmonary manifestations of GERD. It also reviews relevant clinical information concerning GERD-related chronic cough and asthma. Finally, a potential management strategy for GERD in pulmonary patients is discussed.

Gaude, Gajanan S.

2009-01-01

122

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

123

Mechanisms and clinical manifestations of supraventricular tachycardias.  

PubMed

Supraventricular tachycardia (SVT) is used as a general label for a variety of fast heart rhythms originating in any part of the conduction system above the ventricles. Supraventricular tachycardia encompasses subclasses of tachycardias such as: atrioventricular nodal reentrant tachycardia (AVNRT), atrioventricular reciprocating tachycardia (AVRT), atrial tachycardia, and atrial flutter. Nurses may encounter patients with these common types of dysrhythmias in inpatient or outpatient settings. Supraventricular tachycardia causes numerous symptoms which may make life difficult for these patients. This article reviews the mechanisms, prevalence, clinical manifestations, and treatment options for each of the major types of supraventricular tachycardia. Radiofrequency ablation has currently made the treatment options more optimistic for these patients than in past years. Further research needs to be done on if, or how, these patients' symptoms and quality of life change after ablation. PMID:7651948

Wood, K

1995-01-01

124

Respiratory manifestations of gastroesophageal reflux disease.  

PubMed

Gastroesophageal reflux disease (GERD) is highly associated with a range of respiratory symptoms, arising from a variety of etiologies. The following commentaries on respiratory manifestations of GERD address evidence for a role of a vagally mediated bronchoconstriction reflex in the development of asthma; the direct effects of airway obstruction on lower esophageal sphincter (LES) pressure and reflux episodes; the mechanisms by which reflux may play roles in chronic cough and airway stenosis; the limited efficacy of laparoscopic antireflux surgery (LARS) in improving GERD-related respiratory symptoms; the search for a marker for microaspiration and reflux-induced airway disease; and the potential of proton pump inhibitor (PPI) treatment for patients presenting with asthma and GERD. PMID:24117633

Harding, Susan M; Allen, Jacqui E; Blumin, Joel H; Warner, Elizabeth A; Pellegrini, Carlos A; Chan, Walter W

2013-10-01

125

Skin manifestation of mantle cell lymphoma.  

PubMed

Mantle cell lymphoma (MCL) is a distinct type of non-Hodgkin's lymphoma that commonly affects extranodal sites. The most commonly affected sites are bone marrow, gastrointestinal tract and Waldeyer's ring, however, skin is rarely involved. We reported a 62-year-old Japanese patient with MCL, exhibiting multiple small dome-shaped red nodules and skin ulcers. Histopathological examination demonstrated numerous atypical lymphoid cells in the dermis and subcutis. Immunohistochemically, tumor cells were positive for CD20 (L26), CD5, CD43 and cyclin D1, but negative for CD45RO (UCHL-1), CD3, CD10 and CD23. Our patient showed a significant improvement of skin lesions and lymphadenopathy with a combination chemotherapy. Awareness of skin manifestations of MCL is essential for dermatologists to establish an early diagnosis and perform appropriate treatment. PMID:16935806

Motegi, Sei-Ichiro; Okada, Etsuko; Nagai, Yayoi; Tamura, Atsushi; Ishikawa, Osamu

126

Pancreatic trauma: acute and late manifestations.  

PubMed

A retrospective analysis of 47 patients with pancreatic trauma is presented. A total of 43 patients presented with acute pancreatic injury, 32 after blunt abdominal trauma. Isolated blunt pancreatic injuries were a considerable diagnostic problem with a mean delay from trauma to operation of 9.4 days. At operation peripancreatic drainage in mild injuries and distal resection in cases of ductal injury were the commonest procedures. The overall mortality was 19 per cent, but only three of the eight deaths were attributable to the pancreatic injury. The overall complication rate was 63 per cent and the pancreatic complication rate was 33 per cent. Four patients presented with chronic pancreatitis resulting from previously untreated blunt abdominal trauma 0.5-21 years earlier. Clinically, they did not differ from the manifestations of chronic pancreatitis of other aetiological origins. PMID:3349308

Leppäniemi, A; Haapiainen, R; Kiviluoto, T; Lempinen, M

1988-02-01

127

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

128

Epidemiology and clinical manifestations of mucormycosis.  

PubMed

Mucormycosis is an emerging angioinvasive infection caused by the ubiquitous filamentous fungi of the Mucorales order of the class of Zygomycetes. Mucormycosis has emerged as the third most common invasive mycosis in order of importance after candidiasis and aspergillosis in patients with hematological and allogeneic stem cell transplantation. Mucormycosis also remains a threat in patients with diabetes mellitus in the Western world. Furthermore, this disease is increasingly recognized in recently developed countries, such as India, mainly in patients with uncontrolled diabetes or trauma. Epidemiological data on this type of mycosis are scant. Therefore, our ability to determine the burden of disease is limited. Based on anatomic localization, mucormycosis can be classified as one of 6 forms: (1) rhinocerebral, (2) pulmonary, (3) cutaneous, (4) gastrointestinal, (5) disseminated, and (6) uncommon presentations. The underlying conditions can influence clinical presentation and outcome. This review describes the emerging epidemiology and the clinical manifestations of mucormycosis. PMID:22247442

Petrikkos, George; Skiada, Anna; Lortholary, Olivier; Roilides, Emmanuel; Walsh, Thomas J; Kontoyiannis, Dimitrios P

2012-02-01

129

[Acute abdomen as initial manifestation of meningococcemia].  

PubMed

Abdominal pain as an initial symptom of meningococcemia is an infrequent entity, rarely described in literature. We present a case of a 4 year-old, male, previously healthy child with a 24 hour history of fever and abdominal pain. He is admitted in a surgical unit with a diagnosis of acute abdomen for surgical resolution. The clinical course turns unfavorably, and patient presents signs of severe sepsis. Urgent laparotomy is performed, observing little brownish fluid and mesenteric adenitis. He then exhibits palpable purpuric rapidly progressive lesions in lower extremities, progressing to septic shock. Later, Neisseria meningitidis serogroup B is isolated from blood cultures. The aim of this article is drawing attention to a nontypical form of manifestation of meningococcemia, as a delayed diagnosis and treatment has an impact on morbidity and mortality among the pediatric population. PMID:18695840

Tomezzoli, Silvana; Juárez, María del V; Rossi, Santiago I; Lema, Diego A; Barbaro, Cristian R; Fiorini, Sandra

2008-06-01

130

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

131

Manifestations of dynamical facilitation in glassy materials.  

PubMed

By characterizing the dynamics of idealized lattice models with a tunable kinetic constraint, we explore the different ways in which dynamical facilitation manifests itself within the local dynamics of glassy materials. Dynamical facilitation is characterized both by a mobility transfer function, the propensity for highly mobile regions to arise near regions that were previously mobile, and by a facilitation volume, the effect of an initial dynamical event on subsequent dynamics within a region surrounding it. Sustained bursts of dynamical activity-avalanches-are shown to occur in kinetically constrained models, but, contrary to recent claims, we find that the decreasing spatiotemporal extent of avalanches with increased supercooling previously observed in granular experiments does not imply diminishing facilitation. Viewed within the context of existing simulation and experimental evidence, our findings show that dynamical facilitation plays a significant role in the dynamics of systems investigated over the range of state points accessible to molecular simulations and granular experiments. PMID:23005099

Elmatad, Yael S; Keys, Aaron S

2012-06-20

132

[Neurologic and psychiatric manifestations of Lyme disease].  

PubMed

The neurological and psychiatric manifestations of Borrelia burgdorferi sensu lato are so numerous that Borrelia is also called the "new great imitator". Thus knowing about the multiple clinical aspects of neuroborreliosis is necessary for the clinician. We reviewed literature for "classical" neuroborreliosis such as acute meningoradiculitis or chronicle encephalomyelitis, but also for encephalitis, myelitis, polyneuritis, radiculitis and more controversial disorders such as chronic neurological disorders, ischemic and hemorrhagic stroke, and motor neuron disease. We specified every time on which basis each disorder was attributed to Lyme disease, particularly if European or American criteria were met. Every part of the nervous system can be involved: from central to peripheral nervous system, and even muscles. In endemic areas, Lyme serology must be assessed in case of unexplained neurological or psychiatric disorder. In case of positive serology, CSF assessment with intrathecal anti-Borrelia antibody index will be more efficient to prove the diagnosis. PMID:17350199

Blanc, F

2007-03-09

133

[Hepatic manifestations in typhoid fever (author's transl)].  

PubMed

Typhoid fever is always endemic in Ivory Coast. Among the various visceral injuries able to arrive during the course of the illness, a study of hepatic manifestations realized in 279 patients show, by the realization of systematic LBP, that, beside clinically and/or biologically certain forms, an hepatic injury is histologically constant. Aetiological, clinical, biological, diagnostic and therapeutic particularities connected with the hepatic localizations are considered and compared with findings of other authors. To remark, in Ivory Coast, on one hand the great predominance of Eberth bacillus aetiology, on the other hand the lack of statistically significative relation with drepanocytary waste. The pathogenic signification of the constancy of the hepatic injury and its peculiar histological pattern of non specific reactive hepatitis is discussed. PMID:6246606

Soubeyrand, J; Rain, B; Condat, J M; Ticolat, R; Beugre, K; Diallo, A D; Fadiga, D; Beda, B Y

134

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

135

Manifestations of top compositeness at colliders.  

SciTech Connect

We explore the possibility that the right-handed top quark is composite, identifying possible signatures of compositeness and how they might manifest themselves at the LHC and Tevatron. We perform a complete analysis of the dimension six modifications of the top coupling to gluons and find that cancellations among operators in the t{bar t} rate allow for very low compositeness scales, but this can be drastically improved by looking at kinematic distributions. Turning to the LHC, we examine four top production from a dimension six four-top operator and estimate the LHC with 100 fb{sup -1} collected luminosity to be sensitive to compositeness scales as high as 5 TeV.

Kumar, K.; Tait, T. M. P.; Vega-Morales, R.; High Energy Physics; Northwestern Univ.

2009-01-01

136

[Radiological manifestations of Baastrup's disease in children].  

PubMed

Baastrup's disease is not usually considered among the possible causes of low back pain in children. Classically, Baastrup's disease is characterized by degenerative phenomena secondary to friction between adjacent spinous processes, with sclerosis of the margins and decrease in the interspinous space. Baastrup's disease becomes more prevalent with age and is usually accompanied by degenerative changes in the vertebral column, such as lumbar facet hypertrophy or disc disease. Certain activities like dance or gymnastics can make Baastrup's disease more likely to appear at an earlier age. In children, Baastrup's disease can manifest in a different way, with increased interspinous spaces and bone remodeling. In this article, we present the cases of two patients with low back pain who were diagnosed with Baastrup's disease. PMID:21958726

Arias Fernández, J; Broncano Cabrero, J; Bondía Gracia, J M; Aquerreta Beola, J D

2011-09-28

137

GI manifestations of Ehlers-Danlos syndrome.  

PubMed

Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue that is distinguished not only by the triad of skin hyperextensibility, articular hypermobility, and tissue fragility but also by its heterogeneity on clinical, genetic, and biochemical grounds. The phenotypical variance that characterizes this syndrome often makes its recognition difficult, and failure to recognize the disease despite a classic course is not uncommon. Diagnosis is paramount, however, so life-threatening associations can be searched for and unique principles of management can be instituted. Patients are prone to GI catastrophes such as perforation and massive bleeding, which can be compounded by grave surgical and vascular complications. A thorough knowledge of the GI manifestations of EDS and their management is mandatory to prevent unnecessary morbidity and mortality. PMID:8931403

Solomon, J A; Abrams, L; Lichtenstein, G R

1996-11-01

138

Oral manifestations of HIV patients in South Indian population  

PubMed Central

Objectives: To study the prevalence of oral manifestations in HIV-infected patients and to correlate oral manifestations with age, gender, severity, and clinical staging. Materials and Methods: Fifty patients of either sex diagnosed as HIV positive were included in the study. The data obtained were analyzed statistically using Fisher's exact test and Chi-square test. Results: Among the 50 HIV-infected patients, oral manifestations were found in 40 (80.0%) patients. Thirty (60%) patients were seen in the age range between 31 and 65 years, and 29 (58%) patients were females. Majority of the patients [26 (52%)] were in the clinical staging C, of whom 23 (88.5%) were with manifestations with significant statistical value (P < 0.05). Patients with CD4 count less than 200 had manifestations in 22 (88%) patients. Correlation between reduction in CD4 count and presence of manifestations was significant (P < 0.05). Twenty-eight (80%) patients without antiretroviral therapy (ART) reported with manifestations. Correlation between ART and presence of manifestations was not significant (P = 1.00). Interpretation and Conclusion: Oral manifestations are the indicators for the disease progression. Clinical stage C and lower CD4 count may be useful predictors for HIV, with greater prevalence of oral manifestations.

Annapurna, Chandrakala Shekarappa; Prince, Christo Naveen; Sivaraj, S.; Ali, I. M.

2012-01-01

139

Electro Convulsive Therapy in Psychiatric Manifestations in Wilson's Disease  

PubMed Central

Wilson's disease occurs due to an inborn error of metabolism. Psychiatric symptoms are often the first manifestation of the disease and can obscure the diagnosis. There are five neuropsychiatric symptoms clusters established for Wilson's disease patients: Behavior and/or personality disorders, mood disorders, cognitive deficits, psychotic manifestations, and others. The frequency with which psychiatric manifestations appears in Wilson's disease remains vague. However, whenever they occur, they need to be correctly identified and treated. Though encouraging results have been obtained in controlling psychiatric manifestations of Wilson's with psychotropic medications, some sub-group of patients fail to respond to any therapy. We aim at finding options for controlling psychosis in these patients with electro convulsive therapy (ECT). A patient exhibiting rare neuropsychiatric manifestations of Wilson's and who is not responding to psychotropic medication was considered for ECT. Considerable control over psychiatric manifestations with ECT was observed and later treated with maintenance ECTs for relapse control.

Vaishnav, Parth; Gandhi, H. A.

2013-01-01

140

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

141

[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

142

Dermatologic manifestations of parathyroid-related disorders.  

PubMed

Dermatologic manifestations of parathyroid-related disorders, although rare in sporadic cases, are not uncommon in familial syndromes. Patients with familial hyperparathyroidism have several types of skin lesions. In multiple endocrine neoplasia 1, patients commonly have angiofibromas (85%) and collagenomas (70%), lesions that show loss of one 11q13 allele, the molecular abnormality in multiple endocrine neoplasia 1. They can also present with lipomas or café-au-lait spots. Cutaneous amyloidosis, an entity that can occur sporadically, has been described in multiple endocrine neoplasia 2a and is usually localized to the interscapular area. Metastatic calcification is an entity commonly encountered in patients with hyperparathyroidism and renal failure. It can be complicated by infections and necrosis. It is best treated by controlling hypercalcemia, hyperphosphatemia, hyperparathyroidism, antibiotics, and analgesia. Parathyroidectomy is reserved for refractory cases. Hypoparathyroidism presenting in the context of polyglandular failure type 1 is characterized by mucocutaneous candidiasis. Pseudohypoparathyroidism, an inherited disorder with end-organ unresponsiveness to parathyroid hormone, is characterized by Albright hereditary osteodystrophy. Patients present with short stature, round facies, brachydactyly, and short fourth or fifth metacarpals. PMID:16828410

Fuleihan, Ghada El-Hajj; Rubeiz, Nelly

143

Gastrointestinal manifestations in primary immune disorders.  

PubMed

The gastrointestinal tract is the largest lymphoid organ in the body containing T and B lymphocytes, macrophages, and dendritic cells. Despite the fact that these cells are constantly confronted with antigen primarily in the form of food and bacteria, immune responses in the gut are tightly regulated to maintain homeostasis. Without this balance of active immunity and tolerance, mucosal inflammation may ensue, and manifest as Crohn's disease, ulcerative colitis, pernicious anemia, or celiac sprue. Therefore, it is not unreasonable that inflammatory diseases of the gut are commonly encountered in patients with primary immune deficiencies. The exact pathogenesis of gastrointestinal diseases in the setting of primary immunodeficiency remains unknown, however, both humoral and cell-mediated immunity appear to play a role in preventing intestinal inflammation. Patients presenting with atypical gastrointestinal disease and/or failure to respond to conventional therapy should be evaluated for an underlying primary immune disorder in order to initiate appropriate treatment, such as immunoglobulin or in more severe cases bone marrow transplantation, to prevent long term complications. PMID:19637385

Agarwal, Shradha; Mayer, Lloyd

2010-04-01

144

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

145

Radiation nephritis. Clinical manifestations and pathophysiologic mechanisms  

SciTech Connect

Radiation nephritis is both volume and dose related. Clinical experience would indicate that a minimum of one third of the renal volume needs to be excluded from nephrotoxic doses which appears to have a threshold of 2,000 cGy. The site of damage leading to renal failure appears to be the microvasculature ultimately expressed as glomerulosclerosis. How much direct damage to the tubular system contributes to this process is unclear, but undoubtedly the resultant systemic physiologic effects potentiate the expression of damage in the irradiated kidney. The acute syndrome, with all the potential manifestations of renal failure, rarely presents sooner than six months and appears to have no clear prodrome, although it would seem reasonable that a subclinical syndrome consisting of abnormalities detectable by urinalysis may occur. Treatment of radiation-induced nephritis or hypertension is no different from treatment for nephritis from any other cause and should be aggressive with lifelong follow-up. Carcinogenesis is a rare late expression of radiation-induced kidney damage. 25 references.

Krochak, R.J.; Baker, D.G.

1986-05-01

146

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

2013-05-13

147

Neuromuscular Manifestations of West Nile Virus Infection  

PubMed Central

The most common neuromuscular manifestation of West Nile virus (WNV) infection is a poliomyelitis syndrome with asymmetric paralysis variably involving one (monoparesis) to four limbs (quadriparesis), with or without brainstem involvement and respiratory failure. This syndrome of acute flaccid paralysis may occur without overt fever or meningoencephalitis. Although involvement of anterior horn cells in the spinal cord and motor neurons in the brainstem are the major sites of pathology responsible for neuromuscular signs, inflammation also may involve skeletal or cardiac muscle (myositis, myocarditis), motor axons (polyradiculitis), and peripheral nerves [Guillain–Barré syndrome (GBS), brachial plexopathy]. In addition, involvement of spinal sympathetic neurons and ganglia provides an explanation for autonomic instability seen in some patients. Many patients also experience prolonged subjective generalized weakness and disabling fatigue. Despite recent evidence that WNV may persist long-term in the central nervous system or periphery in animals, the evidence in humans is controversial. WNV persistence would be of great concern in immunosuppressed patients or in those with prolonged or recurrent symptoms. Support for the contention that WNV can lead to autoimmune disease arises from reports of patients presenting with various neuromuscular diseases that presumably involve autoimmune mechanisms (GBS, other demyelinating neuropathies, myasthenia gravis, brachial plexopathies, stiff-person syndrome, and delayed or recurrent symptoms). Although there is no specific treatment or vaccine currently approved in humans, and the standard remains supportive care, drugs that can alter the cascade of immunobiochemical events leading to neuronal death may be potentially useful (high-dose corticosteroids, interferon preparations, and intravenous immune globulin containing WNV-specific antibodies). Human experience with these agents seems promising based on anecdotal reports.

Leis, A. Arturo; Stokic, Dobrivoje S.

2012-01-01

148

Neuromuscular manifestations of west nile virus infection.  

PubMed

The most common neuromuscular manifestation of West Nile virus (WNV) infection is a poliomyelitis syndrome with asymmetric paralysis variably involving one (monoparesis) to four limbs (quadriparesis), with or without brainstem involvement and respiratory failure. This syndrome of acute flaccid paralysis may occur without overt fever or meningoencephalitis. Although involvement of anterior horn cells in the spinal cord and motor neurons in the brainstem are the major sites of pathology responsible for neuromuscular signs, inflammation also may involve skeletal or cardiac muscle (myositis, myocarditis), motor axons (polyradiculitis), and peripheral nerves [Guillain-Barré syndrome (GBS), brachial plexopathy]. In addition, involvement of spinal sympathetic neurons and ganglia provides an explanation for autonomic instability seen in some patients. Many patients also experience prolonged subjective generalized weakness and disabling fatigue. Despite recent evidence that WNV may persist long-term in the central nervous system or periphery in animals, the evidence in humans is controversial. WNV persistence would be of great concern in immunosuppressed patients or in those with prolonged or recurrent symptoms. Support for the contention that WNV can lead to autoimmune disease arises from reports of patients presenting with various neuromuscular diseases that presumably involve autoimmune mechanisms (GBS, other demyelinating neuropathies, myasthenia gravis, brachial plexopathies, stiff-person syndrome, and delayed or recurrent symptoms). Although there is no specific treatment or vaccine currently approved in humans, and the standard remains supportive care, drugs that can alter the cascade of immunobiochemical events leading to neuronal death may be potentially useful (high-dose corticosteroids, interferon preparations, and intravenous immune globulin containing WNV-specific antibodies). Human experience with these agents seems promising based on anecdotal reports. PMID:22461779

Leis, A Arturo; Stokic, Dobrivoje S

2012-03-21

149

Sex Differences in the Clinical Manifestations of Alzheimer Disease Pathology  

Microsoft Academic Search

Context: Sex differences in risk of clinically diagnosed Alzheimer disease (AD) have been studied extensively, but little is known about the relation of the pathologic indices of AD to the clinical manifestations of the dis- ease in men compared with women. Objective: To test whether the relation of AD pathol- ogy to the clinical manifestations of the disease differs in

Lisa L. Barnes; Robert S. Wilson; Julia L. Bienias; Julie A. Schneider; Denis A. Evans; David A. Bennett

2005-01-01

150

Silent Brain Infarcts in Patients With Manifest Vascular Disease  

Microsoft Academic Search

Background and Purpose—Silent infarcts are frequently found on MRIs of brains of healthy elderly persons (aged 60 years). The purpose of this study was to investigate the prevalence and determinants of silent infarcts in a population of patients with clinically manifest vascular disease. Methods—To detect silent infarcts, MR images were made in 308 participants of the Second Manifestations of ARTerial

Janneke L. P. Giele; Theo D. Witkamp; Willem P. T. M. Mali

2010-01-01

151

A Review of the Latent and Manifest Benefits (LAMB) Scale  

ERIC Educational Resources Information Center

|The latent and manifest benefits (LAMB) scale (Muller, Creed, Waters & Machin, 2005) was designed to measure the latent and manifest benefits of employment and provide a single scale to test Jahoda's (1981) and Fryer's (1986) theories of unemployment. Since its publication in 2005 there have been 13 studies that have used the scale with 5692…

Muller, Juanita; Waters, Lea

2012-01-01

152

Eye manifestations in medullary carcinoma of the thyroid.  

PubMed Central

Multiple endocrine adenomatosis type III (MEA--III) in a 32-year-old white woman is reported. The initial manifestation, a thyroid nodule, was detected at the age of 4 years. The characteristic ocular manifestation of MEA--III are discussed. Images

Khalil, M K; Lorenzetti, D W

1980-01-01

153

The Magic of the Manifest: Freud's Egyptian Dream Book  

Microsoft Academic Search

Textual evidence shows that dream interpretation preceded Freud by several millennia, but his method was unprecedented. In asserting that the dream's remembered, or manifest, content was merely a disguise for censored, latent meaning, he associated manifest content with notions of animism and magical thinking that characterized colonialist attitudes toward non-European cultures. This paper examines Freud's investment in devaluing dream imagery

Diane ODonoghue

2009-01-01

154

Manifestation of thrombocytopenia in dengue-2-virus-infected mice  

Microsoft Academic Search

Dengue virus infection causes dengue fever, dengue haemorrhagic fever and dengue shock syndrome. No animal model is available that mimics these clinical manifestations. In this study, the establishment is reported of a murine model for dengue virus infection that resembles the thrombocytopenia manifestation. Dengue-2 virus (dengue virus type 2) can infect murine cells either in vitro (primary cell culture) or

Kao-Jean Huang; Shu-Yi J. Li; Shiour-Ching Chen; Hsiao-Sheng Liu; Yee-Shin Lin; Trai-Ming Yeh; Ching-Chuan Liu; Huan-Yao Lei

2000-01-01

155

Holism and life manifestations: molecular and space-time biology.  

PubMed

Appeals of philosophers to look for new concepts in sciences are being met with a weak response. Limited attention is paid to the relation between synthetic and analytic approach in solving problems of biology. An attempt is presented to open a discussion on a possible role of holism. The term "life manifestations" is used in accordance with phenomenology. Multicellular creatures maintain milieu intérieur to keep an aqueous milieu intracellulair in order to transform the energy of nutrients into the form utilizable for driving cellular life manifestations. Milieu intérieur enables to integrate this kind of manifestations into life manifestations of the whole multicellular creatures. The integration depends on a uniqueness and uniformity of the genome of cells, on their mutual recognition and adherence. The processes of ontogenetic development represent the natural mode of integration of cellular life manifestations. Functional systems of multicellular creatures are being established by organization of integrable cells using a wide range of developmental processes. Starting from the zygote division the new being displays all properties of a whole creature, although its life manifestations vary. Therefore, the whole organism is not only more than its parts, as supposed by holism, but also more than developmental stages of its life manifestations. Implicitly, the units of whole multicellular creature are rather molecular and developmental events than the cells per se. Holism, taking in mind the existence of molecular and space-time biology, could become a guide in looking for a new mode of the combination of analytical and synthetic reasoning in biology. PMID:20482219

Krecek, J

2010-01-01

156

Clinical and genetic characterization of manifesting carriers of DMD mutations.  

PubMed

Manifesting carriers of DMD gene mutations may present diagnostic challenges, particularly in the absence of a family history of dystrophinopathy. We review the clinical and genetic features in 15 manifesting carriers identified among 860 subjects within the United Dystrophinopathy Project, a large clinical dystrophinopathy cohort whose members undergo comprehensive DMD mutation analysis. We defined manifesting carriers as females with significant weakness, excluding those with only myalgias/cramps. DNA extracted from peripheral blood was used to study X-chromosome inactivation patterns. Among these manifesting carriers, age at symptom onset ranged from 2 to 47 years. Seven had no family history and eight had male relatives with Duchenne muscular dystrophy (DMD). Clinical severity among the manifesting carriers varied from a DMD-like progression to a very mild Becker muscular dystrophy-like phenotype. Eight had exonic deletions or duplications and six had point mutations. One patient had two mutations (an exonic deletion and a splice site mutation), consistent with a heterozygous compound state. The X-chromosome inactivation pattern was skewed toward non-random in four out of seven informative deletions or duplications but was random in all cases with nonsense mutations. We present the results of DMD mutation analysis in this manifesting carrier cohort, including the first example of a presumably compound heterozygous DMD mutation. Our results demonstrate that improved molecular diagnostic methods facilitate the identification of DMD mutations in manifesting carriers, and confirm the heterogeneity of mutational mechanisms as well as the wide spectrum of phenotypes. PMID:20630757

Soltanzadeh, Payam; Friez, Michael J; Dunn, Diane; von Niederhausern, Andrew; Gurvich, Olga L; Swoboda, Kathryn J; Sampson, Jacinda B; Pestronk, Alan; Connolly, Anne M; Florence, Julaine M; Finkel, Richard S; Bönnemann, Carsten G; Medne, Livija; Mendell, Jerry R; Mathews, Katherine D; Wong, Brenda L; Sussman, Michael D; Zonana, Jonathan; Kovak, Karen; Gospe, Sidney M; Gappmaier, Eduard; Taylor, Laura E; Howard, Michael T; Weiss, Robert B; Flanigan, Kevin M

2010-07-13

157

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2012 CFR

...this section. (b) The shipper (generator) shall prepare the manifest in accordance...representing: (1) The shipper (generator) of the waste at the time it is offered...waste, (4) Returned to the shipper (generator) by the carrier that transported...

2012-10-01

158

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2011 CFR

...this section. (b) The shipper (generator) shall prepare the manifest in accordance...representing: (1) The shipper (generator) of the waste at the time it is offered...waste, (4) Returned to the shipper (generator) by the carrier that transported...

2011-10-01

159

Orofacial manifestations of bacterial and viral infections in children.  

PubMed

Orofacial manifestations of bacterial and viral infections in children may cause significant discomfort and suffering. Recognition of the clinical presentation of these disorders is paramount to their clinical management and appropriate referral. PMID:23705243

Pinto, Andres; Hong, Catherine H

2013-04-01

160

8 CFR 251.1 - Arrival manifests and lists.  

Code of Federal Regulations, 2013 CFR

... (3) Exception for certain Great Lakes vessels. (i) A manifest shall...engaged solely in traffic on the Great Lakes or the St. Lawrence River and...waterways, herein designated as a Great Lakes vessel, unless: (A) The...

2013-01-01

161

Periorbital edema as initial manifestation of chronic cutaneous lupus erythematosus  

PubMed Central

Periorbital edema occurs frequently in dermatomyositis, but it has rarely been noted in systemic systemic lupus erythematosus. We describe a patient who developed bilateral periorbital edema and erythema as the sole manifestation of systemic lupus erythematosus.

Erras, Samar; Benjilali, Laila; Essaadouni, Lamiaa

2012-01-01

162

[Unusual muscular manifestations in a case of Horton's disease].  

PubMed

The authors report the case of a 66-year-old woman with temporal arteritis. The unusual muscular involvement consists in proximal muscle wasting, electromyographic changes and increased muscle enzyme levels. The improvement of these objective manifestations under steroid therapy allows to include them in the systemic spectrum of temporal anteritis. Attention is drawn to the infrequency of such objective manifestations published in the literature on temporal arteritis or polymyalgia rheumatica. PMID:6316527

Wendling, D; Hory, B; Guidet, M; Saint-Hillier, Y; Cassou, M; Perol, C

1983-10-27

163

Conformal compensators and manifest type IIB S-duality  

NASA Astrophysics Data System (ADS)

Using the conformal compensator superfields of N=2 D=4 supergravity, the Type IIB S-duality transformations are expressed as a linear rotation which mixes the compensator and matter superfields. The classical superspace action for D=4 compactifications of Type IIB supergravity is manifestly invariant under this transformation. Furthermore, the introduction of conformal compensators allows a Fradkin-Tseytlin term to be added to the manifestly SL(2,Z)-covariant sigma model action of Townsend and Cederwall. © 1998

Berkovits, Nathan

1998-03-01

164

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

165

Mucocutaneous manifestations of acquired hypoparathyroidism: An observational study  

PubMed Central

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.

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

2012-01-01

166

Hematological Manifestations of SLE at Initial Presentation: Is It Underestimated?  

PubMed Central

SLE can present with hematological manifestations alone or along with features of other system involvement. With a low index of clinical suspicion or inadequate follow up the diagnosis may be delayed or missed at the time of presentation, in those with hematological abnormalities as the initial manifestation. An observational study was conducted among patients of SLE, in a tertiary referral centre of North Kerala, with the purpose of estimating the proportion of patients with hematological manifestations as the initial presentation of the disease and to study their nature. It was observed that 82% of the patients had hematological manifestations at presentation. It is the most common presenting manifestation of SLE in people of North Kerala. Autoimmune hypothyroidism was one of the common coexisting abnormalities in these patients, which is not included in the American College of Rheumatology (ACR) criteria for diagnosis. Arthritis was uncommon among those who presented with hematological manifestations. A significant number of patients do not satisfy the ACR criteria at the time of diagnosis but do so on follow up. The ACR criteria are weak to diagnose such patients and therefore need revision. We therefore propose an alternative to ACR criteria as “Kozhikode criteria for SLE”.

Sasidharan, P. K.; Bindya, M.; Sajeeth Kumar, K. G.

2012-01-01

167

40 CFR 761.213 - Use of manifest-Commercial storage and disposal facility requirements.  

Code of Federal Regulations, 2013 CFR

...POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION...is accompanied by a shipping paper containing all the information...of the manifest or shipping paper (if the manifest has not...by the manifest or shipping paper was received; (2)...

2013-07-01

168

Ocular Manifestations of Acquired Immunodeficiency Syndrome in Korea  

PubMed Central

The clinical features of HIV/AIDS-related ocular manifestations in Korean patients were investigated in this study. Data on 200 consecutive Korean patients diagnosed with AIDS who visited the Seoul National University Hospital from January 2003 to June 2008 were reviewed. Fifty-seven patients (28.5%) had ocular manifestations, and they showed significantly lower CD4+ T cell count than patients without ocular manifestations. Among them, 23 (40.3%) patients showed retinal microvasculopathy, and 22 (38.5%) patients showed cytomegalovirus (CMV) retinitis. Other manifestations included retinal vein occlusion (n = 4), herpes zoster ophthalmicus (n = 4), syphilitic uveitis (n = 2), acute retinal necrosis (n = 1), and progressive outer retinal necrosis (n = 1). The mean CD4+ lymphocyte counts of the patients with retinal microvasculopathy and cytomegalovirus retinitis were 108.5 cells/µL and 69.4 cells/µL, respectively. In conclusion, ocular manifestations including CMV retinitis are common complications in Korean patients with AIDS even in the era of highly active anti-retroviral therapy. Compared to previous reports in western countries, prevalence of CMV retinitis is relatively low and CD4+ lymphocytes count at the time of diagnosis is relatively high.

Kim, Sang Jin; Park, Sang Jun; Kim, Nam Joong; Jang, Hee-Chang; Oh, Myoung-don

2012-01-01

169

Neurological manifestations of human parvovirus B19 infection.  

PubMed

Since its discovery, human parvovirus B19 has been linked with a broad spectrum of clinical syndromes. An aetiological role for the virus has been confirmed in erythema infectiosum, transient aplastic crisis, persistent infection manifesting as pure red cell aplasia in immunocompromised persons, non-immune hydrops fetalis and arthritis. Less commonly recognised, but receiving increasing attention recently, are the neurological manifestations, a variety of which have been described in patients with either clinically diagnosed or laboratory confirmed B19 infection. The purpose of this review is to summarise present knowledge of B19, its known and potential pathogenic mechanisms and its association with human diseases, particularly those with neurological manifestations. The outcome of the review supports an aetiological role of the virus in neurological disease. However, the pathogenesis remains unknown and elucidating this is a priority. PMID:12740833

Barah, Faraj; Vallely, Pamela J; Cleator, Graham M; Kerr, Jonathan R

170

Classification of epileptic motor manifestations using inertial and magnetic sensors.  

PubMed

In order to characterize objectively the succession of movements observed during motor seizures, inertial and magnetic sensors were placed on epileptic patients. Video recordings synchronized with motion recordings were analyzed visually during seizures and divided, for each limb, into events corresponding to different classes of motor manifestations. For each classified event, features were extracted and a subset selection was automated using artificial neural networks. The best artificial neural network was simulated on whole recordings to generate a stereotypic evolution of motor manifestations that we called motorograms. It is shown that motorograms can point out seizure movements and emphasize epileptic patterns. PMID:21112583

Becq, Guillaume; Bonnet, Stéphane; Minotti, Lorella; Antonakios, Michel; Guillemaud, Régis; Kahane, Philippe

2010-11-26

171

Oral manifestations and treatment of a child with Sjögren's syndrome.  

PubMed

Sjögren's syndrome (SS) is a chronic inflammatory systemic autoimmune disease affecting the exocrine, salivary and lacrimal glands. The condition occurs more often in adults and is rare in childhood. SS should be considered in the differential diagnosis of recurrent parotitis and keratoconjunctivitis sicca. Oral manifestations include early tooth decay and xerostomia. Treatment consists of operative dentistry and saliva management. This paper reports a case of a 10-year-old Brazilian boy with SS, stressing the oral manifestations, treatment conduct, clinical importance and need for an early diagnosis in order to improve the patient's quality of life. PMID:20819406

Sardenberg, Fernanda; Goursand, Daniela; Polletto, Luiz Thadeu; Vale, Miriam Pimenta; Zarzar, Patrícia Maria; Paiva, Saul Martins

172

Progressive kidney failure as the sole manifestation of extrapulmonary sarcoidosis.  

PubMed

Sarcoidosis is a chronic multisystem disorder characterized by an accumulation of T lymphocytes and mononuclear phagocytes, non-caseating epitheliod granulomas and derangement of normal tissue architecture in affected organs. Sarcoidosis can affect any organ system, however approximately 90% of patients with sarcoidosis have pulmonary, lymph node, cutaneous or ocular manifestations. Renal involvement in sarcoidosis is rare and clinically significant renal dysfunction even less common. We present a case of isolated renal sarcoidosis which manifested with progressively worsening renal function and hypercalcemia. A systematic diagnostic approach with pertinent laboratory studies, imaging and renal biopsy elucidated the diagnosis of renal sarcoidosis without any evidence of systemic involvement. PMID:24079056

Sethi, Supreet; Relia, Nitin; Syal, Gaurav; Kaushik, Chhavi; Gokden, Neriman; Malik, Ahmad B

2013-09-01

173

Insights into novel biological mediators of clinical manifestations in cancer.  

PubMed

A myriad of novel mediators in neoplastic development and progression are currently being explored. Of significance are those that directly explain clinical manifestations of cancer, because understanding these may lead to new diagnostic, preventive, and therapeutic strategies. This review focuses on novel mediators that address how cancer, before it is treated, can induce cachexia, pain, hematological, and immune alterations. It highlights two concepts: first, that a synergy between tumor and stromal cells may be partly responsible for these manifestations, and second, that soluble factors, and in particular cytokines are being identified as major players in tumor-induced local and systemic effects. PMID:14767369

Sanchez, Otto H

174

Manifest sadomasochism of males: Results of an empirical study  

Microsoft Academic Search

Two hundred forty-five manifestly sadomasochistic West German men completed an anonymous questionnaire concerning their sexual behavior and psychosocial problems. They were reached as placers of sadomasochistic contact advertisements or as members of sadomasochistic clubs. Thirty percent were exclusively heterosexually oriented, 31% bisexually oriented, and 38% homosexually oriented. Results are described with respect to the invisibility of deviant behavior, seeking of

Andreas Spengler

1977-01-01

175

Bone Marrow Necrosis as the Only Manifestation of Disseminated Carcinomatosis  

Microsoft Academic Search

An unusual case with thrombocytopenia and bone marrow necrosis as the only manifestation of disseminated carcinomatosis is reported. The diagnosis was first established by repeated bone marrow biopsy which revealed marked necrosis without evidence of malignancy. The patient’s general condition deteriorated rapidly despite therapy with steroids. Autopsy revealed disseminated metastatic adenocarcinoma, but the primary site of the tumor was not

Haim Granot; Aaron Polliack; Yaacov Matzner

1980-01-01

176

Isolated Bone Marrow Manifestation of HIV-Associated Hodgkin Lymphoma  

Microsoft Academic Search

Human immunodeficiency virus-associated Hodgkin lymphoma frequently involves the bone marrow and is usually recognized at staging after Hodgkin lymphoma diagnosis on a lymph node or other tissue biopsies, but occasionally the marrow involvement is the only apparent manifestation of disease. In the latter setting, diagnosis can be problematic. From a total of 42 patients with newly diagnosed human immunodeficiency virus–associated

Maurilio Ponzoni; Luca Fumagalli; Giuseppe Rossi; Massimo Freschi; Alessandro Re; Maria Grazia Viganò; Massimo Guidoboni; Riccardo Dolcetti; Robert W. McKenna; Fabio Facchetti

2002-01-01

177

Portrait of a narcissist: Manifestations of narcissism in physical appearance  

Microsoft Academic Search

Narcissism is characterized in part by an acute concern for one’s appearance. Despite this fundamental aspect of narcissism, little is known about whether narcissism is manifested in features of physical appearance. Can you tell if someone is narcissistic just by looking at them? Our results indicate that snap judgments of narcissism based on full-body photographs are at least as accurate

Simine Vazire; Laura P. Naumann; Peter J. Rentfrow; Samuel D. Gosling

2008-01-01

178

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

179

Exploring the endocrine manifestations of DICER1 mutations.  

PubMed

The discovery of each new cancer susceptibility gene answers one set of questions but poses many more. In this article, we outline a recent example: a new cancer syndrome caused by germline mutations in DICER1, responsible for microRNA processing. In particular, we discuss the endocrine manifestations of mutations in this crucial gene. PMID:22867905

Choong, Catherine S; Priest, John R; Foulkes, William D

2012-08-03

180

Perfectionism: Its Manifestations and Classroom-based Interventions.  

ERIC Educational Resources Information Center

|This article discusses perfectionism in gifted students, aspects of perfectionism, manifestations of perfectionism in the classroom, and classroom based interventions. Recommended interventions include creating a classroom that encourages sharing of self, active listening, and self-evaluation and metacognition, using bibliotherapy, and providing…

Nugent, Stephanie A.

2000-01-01

181

Effects of Firing Atmosphere on the Color Manifestation of Celadon  

Microsoft Academic Search

Effects of firing atmosphere on the color manifestation of celadon were studied. The firing atmosphere was obtained by the combustion of LPG, which generates CO gas during incomplete combustion, which acted as the reducing agent. The amount of CO gas increased as the amount of injected LPG gas increased. As the amount of generated CO gas was few little, the

J. H. Pee; H. S. Choi; K. J. Kim; J. H. Ryu; G. I. Gang; H. Katsuki

2011-01-01

182

Obsessive Compulsive Disorder as Early Manifestation of B12 Deficiency  

PubMed Central

B12 acts as a cofactor in synthesis of neurotransmitters such as serotonin and dopamine, thus B12 deficiency affects mood, emotions and sleeping and can lead to psychiatric disorders. Psychiatric manifestations of B12 deficiency are varied. They seldom precede anemia. We want to present a case of B12 deficiency which was presented with obsessive compulsive disorder.

Valizadeh, Maryam; Valizadeh, Nasim

2011-01-01

183

Stiff-Person Syndrome First Manifesting in Pregnancy  

Microsoft Academic Search

Stiff-person syndrome (SPS) is a rare neurological disorder characterised by progressive stiffness and painful muscle spasms. We present a case of the autoimmune form of glutamate decarboxylase-positive SPS that initially manifested in pregnancy. The diagnosis was made based on clinical, laboratory and electromyoneurographic criteria. The patient was administered low doses of diazepam and baclofen. Considering the clinical picture of SPS

Denis Cerimagic; Ervina Bilic

2009-01-01

184

The articular manifestations of progressive systemic sclerosis (scleroderma)  

Microsoft Academic Search

The articular manifestations of progressive systemic sclerosis (PSS) were studied in 38 patients. Of these, 66% experienced joint pain and 61% had signs of joint inflammation. Limitation of joint movement was seen in 45%. Radiological abnormalities included periarticular osteoporosis (42%), joint space narrowing (34%), and erosions (40%). Erosive disease did not correlate with disease duration, presence of rheumatoid factor, antinuclear

M Baron; P Lee; E C Keystone

1982-01-01

185

Ambiguity surface manifestation of downslope converted noise sources  

Microsoft Academic Search

Deals with the manifestation of downslope converted continental shelf noise sources (primarily ships) in the matched-field processor (MFP) ambiguity surface. Of interest is how noise sources outside the range of interest leak into the ambiguity surface through the sidelobe structure of the effective MFP beam (or cell) pattern. This study is carried out with simulations using the parabolic equation model.

Jean-Marie Q. D. Tran; W. S. Hodgkiss

1993-01-01

186

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

187

76 FR 36480 - Hazardous Waste Manifest Printing Specifications Correction Rule  

Federal Register 2010, 2011, 2012, 2013

...that red ink, as well as other distinct colors, or other methods to distinguish the...in the Direct Final rule will not take effect, and the reasons for such a withdrawal...manifest printing specifications, and the effect of this proposed change would make...

2011-06-22

188

Perfectionism: Its Manifestations and Classroom-based Interventions.  

ERIC Educational Resources Information Center

This article discusses perfectionism in gifted students, aspects of perfectionism, manifestations of perfectionism in the classroom, and classroom based interventions. Recommended interventions include creating a classroom that encourages sharing of self, active listening, and self-evaluation and metacognition, using bibliotherapy, and providing…

Nugent, Stephanie A.

2000-01-01

189

Social Support in Elderly Nursing Home Populations: Manifestations and Influences  

ERIC Educational Resources Information Center

|The purpose of this study was to describe the characteristics of social support and the influencing factors on social support in nursing home environments. Observations and staff questionnaires from two central Florida nursing homes were used in this grounded theory study to answer the following questions: (1) How is social support manifested?…

Rash, Elizabeth M.

2007-01-01

190

Apparent Mineralocorticoid Excess Manifested in An Elderly Patient with Hypothyroidism  

Microsoft Academic Search

The syndrome of apparent mineralocorticoid excess (AME) is characterized by persistent hypertension and hypokalemia, which is caused by impaired inactivation of cortisol (F) to cortisone (E). The thyroid hormone has been known to influence the F to E conversion leading to efficacious inactivation of F into E. However, there have been no reports regarding the clinical manifestation of secondary AME

Kenichi Inagaki; Fumio Otsuka; Hiroyuki Otani; Chikage Sato; Tomoko Miyoshi; Toshio Ogura; Hirofumi Makino

2007-01-01

191

Testing Manifest Monotonicity Using Order-Constrained Statistical Inference  

ERIC Educational Resources Information Center

|Most dichotomous item response models share the assumption of latent monotonicity, which states that the probability of a positive response to an item is a nondecreasing function of a latent variable intended to be measured. Latent monotonicity cannot be evaluated directly, but it implies manifest monotonicity across a variety of observed scores,…

Tijmstra, Jesper; Hessen, David J.; van der Heijden, Peter G. M.; Sijtsma, Klaas

2013-01-01

192

Spermatic cord metastasis as early manifestation of small bowel adenocarcinoma  

PubMed Central

Malignant tumors of the spermatic cord are rare. There are a few case reports on spermatic cord metastasis from colonic, gastric, pancreas, and prostatic cancer. Here, we report a 36-year-old man with brucellosis presenting with spermatic cord metastasis as early manifestation of small bowel adenocarcinoma.

Valizadeh, Nasim; Oskuie, Ali Eishi; Tehranchi, Ali

2013-01-01

193

Neurological manifestations of Hansen's disease and their management  

Microsoft Academic Search

Hansen's disease is almost eliminated from developed countries but in developing countries of Africa, Asia and Latin America leprosy is still considered to be a public health problem. Mycobacterium leprae have the affinity for peripheral nerves and neuropathy is a cardinal manifestation of the disease. The nerve damage affects sensory, motor, and autonomic fibers resulting in the physical impairments and

Amit Agrawal; Lekha Pandit; Monica Dalal; J. P. Shetty

2005-01-01

194

[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

2009-05-18

195

Sickle cell disease: Its signs, symptoms and varied manifestations  

Microsoft Academic Search

Seventy-five sickle cell patients, age 3-36 years from Houston, Texas, participated in the research study to investigate sickle cell manifestations, conducted between November 1989 and August 1990. All the participants were blacks. There were 35 females and 39 males among the participants in this research study. One of the participants did not document the gender.^ The sickle cell history questionnaire

Simon Onwe Ogamdi

1990-01-01

196

Age and Preference for Complexity among Manifestly Creative Women.  

ERIC Educational Resources Information Center

Investigates the relationship between age and creativity among a sample of 271 manifestly creative women aged 23-87 years. Creativity showed no significant negative correlation with age. However, among control subjects not selected for creativity, the negative correlation between age and creativity scores was statistically significant. (Author/RH)

Crosson, C. W.; Robertson-Tchabo, E. A.

1983-01-01

197

Prognosis and possible presymptomatic manifestations of congestive cardiomyopathy (COCM)  

Microsoft Academic Search

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

H. Kuhn; G. Breithardt; H. J. Knieriem; E. Köhler; B. Lösse; L. Seipel; F. Loogen

1978-01-01

198

Market Power in China: Manifestations, Effects and Legislation  

Microsoft Academic Search

This paper presents a picture of market power in China – its current situation, manifestations, causes, damaging effects and related economic laws and regulations. China is in a transition from a planned economy to a socialist market economy. Market power in China is reflected largely in administrative monopolies inherited from the old system, as well as in economic monopolies created

Jijian Yang

2002-01-01

199

Self-Organization in the Manifestations of Youth Extremism  

ERIC Educational Resources Information Center

|The analysis of the nature of youth extremism has shown that there is a connection between the extremist tendency ["ekstremal'nost'"] that is an essential property of young people, on the one hand, and extremist manifestations that come about in that community under certain conditions. These conditions include external ones (the economic,…

Zubok, Iu. A.; Chuprov, V. I.

2011-01-01

200

Carcinomatous lymphangitis as the initial manifestation of ovarian adenocarcinoma.  

PubMed

Carcinomatous lymphangitis accounts for approximately 8% of all cases of metastatic pulmonary tumors. The most common primary sites are breast, lung, stomach, prostate and pancreas. We describe herein the case of a 42-year-old woman in whom the first manifestation of an ovarian adenocarcinoma was carcinomatous lymphangitis of the lung, an unusual presentation of the disease. PMID:18026661

Martynychen, Mariane Gonçalves; Rabelo, Lêda Maria; Silva, Rodney Luiz Frare E; Escuissato, Dante Luiz

201

Dyspnea as a Clinical Manifestation in Primary Retroperitoneal Teratoma  

Microsoft Academic Search

The retroperitoneum is an unusual location for a teratoma in adults. We report a case of retroperitoneal teratoma with clinical manifestation of dyspnea and chest discomfort. Chest radiograph showed elevated left hemidiaphragm. Ultrasonography (US) showed a hypoechoic tumor in the left subphrenic area. Computed tomography (CT) revealed a hypodense tumor in the left subphrenic area. Endoscopic ultrasound-guided fine-needle aspiration of

MICHAEL CHE-HUNG LIN; KOON-KWAN NG; CHIEN-FU HUNG; JENG-HWEI TSENG; YUN-CHUNG CHEUNG; YUNG-LIANG WAN

202

The cutaneous manifestations of child abuse and neglect.  

PubMed

The most easily recognizable physical manifestations of child abuse and neglect are seen in the skin. The findings may be the result of intentional trauma, neglect to provide adequate child care, alterations in family dynamics, or the emotional problems secondary to the maltreatment. PMID:474541

Ellerstein, N S

1979-09-01

203

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

204

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

205

Pathological yawning as an ictal seizure manifestation in the elderly.  

PubMed

Excessive yawning has been reported in the peri-ictal period preceding or following seizures. We describe an exceptional case of an elderly man with impairment of consciousness and paroxysmal excessive yawning. We hypothesise that this can be regarded as an autonomic seizure originating from diencephalic/brainstem structures, manifesting with yawning as an ictal phenomenon. PMID:23076687

Nicotra, Alessia; Khalil, Nofal M; Owbridge, P; Hakda, Mina; Beitverda, Younatan

2012-10-12

206

Fabry Disease: Focus on Cardiac Manifestations and Molecular Mechanisms  

Microsoft Academic Search

Pathogenesis: Fabry disease is an inherited lysosomal storage disorder caused by deficiency of the enzyme !-galactosidase A. The enzyme deficiency results in accumulation of glycosphingolipids in the lysosomes n nearly all cell types and tissues leading to a multisystem disease. Manifestations include painful crisis, angiokeratomas, corneal dystrophy, and hypohydrosis. The severe renal, cerebrovascular, and cardiac involvement is predominantly responsible for

Andreas Perrot; Karl Josef Osterziel; Michael Beck; Rainer Dietz; Christoph Kampmann

2002-01-01

207

Manifest Content in the Dreams of Clinical Populations.  

ERIC Educational Resources Information Center

|Reviews relevant literature to examine relationships between dream content and diagnosis of clinical populations. Although many clinical populations remain unexamined in terms of meaningful characterizations of their dream life, relationship between manifest content and differential diagnosis is noted and generalizations are offered for different…

Mellen, Ronald R.; And Others

1993-01-01

208

A study of cutaneous manifestations in HIV infected persons.  

PubMed

Skin is one of the major target organs during all stages of HIV. The present study is conducted to know the dermatological manifestations among 181 HIV positive patients over a period of 17 months ie, from 01-03-2006 to 31-08-2007 at Mandya Institute of Medical Sciences, Mandya. Of these 181 patients, skin diseases were seen in 82 patients (45.3%), 41 patients had only one dermatological disease, 25 had two, 10 had three and 6 patients had more than four diseases. It was observed that a total of 45.3% patients among the study group developed mucocutaneous manifestations. Therefore early recognition of mucocutaneous lesions will help in early diagnosis and better management of HIV/AIDS patients. PMID:23738406

Harish, M R; Shanmukhappa; Kumar, Shashi; Kumar, Mahesh; Gowda, M S Sidde

2012-10-01

209

Oral manifestations and their treatment in Sjögren's syndrome.  

PubMed

Sjögren's syndrome (SS) is a complex, chronic, systemic, autoimmune disease that mainly affects the exocrine glands, especially the salivary and lacrimal glands, leading to dryness of the oral and ocular mucosae. Several factors have been studied that could explain the glandular hypofunction primarily related to water transport. Recent reports have shown alterations in secretory route and trafficking in labial salivary glands, explaining alterations in the saliva quality. The decrease in salivary flow and qualitative alterations in saliva could explain many of the oral manifestations. The exocrine manifestations and systemic involvement significantly impact the patient's perception of health-related quality of life. For this reason and given its systemic nature, the treatment of these patients should be multidisciplinary. This review addresses some particular oral health aspects of SS patients and focuses on relevant topics concerning the treatment and prevention of common oral disorders associated with this disease. PMID:23557026

González, S; Sung, H; Sepúlveda, D; González, Mj; Molina, C

2013-03-18

210

Ocular manifestations in the Hutchinson-Gilford progeria syndrome  

PubMed Central

The Hutchinson-Gilford progeria (HGP) syndrome is an extremely rare genetic condition characterized by an appearance of accelerated aging in children. The word progeria is derived from the Greek word progeros meaning ‘prematurely old’. It is caused by de novo dominant mutation in the LMNA gene (gene map locus 1q21.2) and characterized by growth retardation and accelerated degenerative changes of the skin, musculoskeletal and cardiovascular systems. The most common ocular manifestations are prominent eyes, loss of eyebrows and eyelashes, and lagophthalmos. In the present case some additional ocular features such as horizontal narrowing of palpebral fissure, superior sulcus deformity, upper lid retraction, upper lid lag in down gaze, poor pupillary dilatation, were noted. In this case report, a 15-year-old Indian boy with some additional ocular manifestations of the HGP syndrome is described.

Chandravanshi, Shivcharan L; Rawat, Ashok Kumar; Dwivedi, Prem Chand; Choudhary, Pankaj

2011-01-01

211

Type 2 segmental manifestation of congenital multiple glomangiomas.  

PubMed

Congenital multiple glomus tumors are extremely rare, and less than 20 cases have been well documented. We report an uncommon case of congenital multiple glomangiomas with a segmental manifestation in a 9-year-old girl. Since birth, the child had presented asymptomatic angiomatous macules arranged in a segmental pattern on the neck and trunk. During a follow-up period of 9 years, disseminated smaller papulonodular lesions developed on both arms and the left leg with a segmental distribution. Histopathology of congenital and acquired lesions confirmed the diagnosis of glomangiomas. The family history was negative for glomus tumors. This type of presentation supports the recently described type 2 segmental manifestation of multiple glomus tumors, with a segmental involvement of congenital early developing lesions. PMID:12771473

Allombert-Blaise, Carole Jouffrey; Batard, Marie-Laure; Ségard, Magali; Martin de Lassalle, Elisabeth; Brevière, Georges-Marie; Piette, Frédéric

2003-01-01

212

Long-range manifestation of surface-enhanced Raman scattering  

NASA Astrophysics Data System (ADS)

The spatial scale at which the effect of surface-enhanced Raman scattering by planar Ag nanostructures manifests itself is investigated experimentally by direct measurements of the dependence of the enhancement factor on the distance between the surface of the Ag nanostructure and a layer of test organic molecules. It is found that the enhancement factor remains almost constant up to distances as large as 30 nm and drops abruptly at larger distances. The obtained dependence is universal for all kinds of organic molecules investigated. The fact that the surface enhancement of Raman scattering manifests itself on such a long spatial scale sharply contradicts the broadly accepted model assuming that the surface-enhanced Raman scattering decreases rapidly at distances as short as 2-3 nm.

Kukushkin, V. I.; Van'kov, A. B.; Kukushkin, I. V.

2013-09-01

213

Toxocara infection and its association with allergic manifestations.  

PubMed

Toxocara canis and Toxocara cati are roundworms of dogs and cats that can also infect humans worldwide. Although these parasites do not reach the adult stage in the human host the larvae migrate to different organs and can persist for many years. Migration of larvae through the lungs may result in respiratory distress such as wheezing, coughs, mucous production and hyper-reactivity of the airways. Epidemiological and experimental studies suggest that infection with this helminth contributes to the development of allergic manifestations, including asthma. These findings are however conflicting since in others studies no association between these two immunopathologies has been found. This article reviews information on Toxocara spp. and findings from epidemiological and experimental studies on the association between Toxocara infection and allergic manifestations. In addition, the immunological mechanisms and the factors involved in the helminth allergy-association are discussed. PMID:22214330

Pinelli, Elena; Aranzamendi, Carmen

2012-03-01

214

Disabling pansclerotic morphea of childhood with extracutaneous manifestations.  

PubMed

Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Mortality is due to complications of the disease such as bronchopneumonia, sepsis, or gangrene. There is no specific laboratory finding. Treatment protocols are still evolving for this severe recalcitrant disorder. Extracutaneous manifestations are rarely reported in DPM. We present the case of a 7-year-old girl with DPM with severe extracutaneous manifestations in the form of gastrointestinal and vascular disease, whose disease progressed rapidly. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis. PMID:23716826

Kura, Mahendra M; Jindal, Saurabh R

2013-03-01

215

Cutaneous changes: an initial manifestation of pulmonary Wegener's granulomatosis.  

PubMed

Cutaneous vasculitis can occur as an isolated dermatologic disorder or as manifestation of a potentially life-threatening systemic vasculitis such as Wegener's granulomatosis (WG). The aim of the study was to characterize cutaneous lesions in 66 WG patients (30 female, 36 male) and to assess the viability of skin biopsy the diagnosis of WG. Skin involvement was observed in 21 (32%) WG patients; in 14 (21%) patients as an initial manifestation and in other seven during the diagnosis establishment. Cutaneous lesions included palpable purpura(n=10), subcutaneous nodules(=4), hemorrhagic bullae (n=3), ulcers (n=2), pustules (n=1), pyoderma gangrenosum (n =1). The patient with pulmonary WG can present initially with cutaneous symptoms and positive PR3-ANCA serologic test results. Leukocytoclastic vasculitis (LCV) was the predominant histopathologic pattern. PMID:22826081

Zycinska, Katarzyna; Wardyn, Kazimierz; Zielonka, Tadeusz M; Nitsch-Osuch, Aneta; Smolarczyk, Roman

2013-01-01

216

Disabling Pansclerotic Morphea of Childhood with Extracutaneous Manifestations  

PubMed Central

Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Mortality is due to complications of the disease such as bronchopneumonia, sepsis, or gangrene. There is no specific laboratory finding. Treatment protocols are still evolving for this severe recalcitrant disorder. Extracutaneous manifestations are rarely reported in DPM. We present the case of a 7-year-old girl with DPM with severe extracutaneous manifestations in the form of gastrointestinal and vascular disease, whose disease progressed rapidly. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis.

Kura, Mahendra M; Jindal, Saurabh R

2013-01-01

217

Familial pellagra-like skin rash with neurological manifestations  

Microsoft Academic Search

A 14-year-old boy of Arabic origin presented with a pellagra-like rash and neurological manifestations including ataxia, dysarthria, nystagmus, and coma. There was a striking response to oral nicotinamide. The laboratory findings were not typical of Hartnup disease: aminoaciduris and indicanuria were absent and there was no evidence of tryptophan malabsorption. Tryptophan loading did not induce tryptophanuria nor did it increase

E Freundlich; M Statter; S Yatziv

1981-01-01

218

Dracunculiasis of the Breast: Radiological Manifestations of a Rare Disease  

PubMed Central

Dracunculiasis of the breast is a rare manifestation of prior infection with the parasite dracunculus medinensis (guinea worm). We describe a case of a 60- year-old woman, who had recently emigrated from Cameroon. She presented for an annual screening mammogram and was found to have coarse linear or coiled serpiginous calcifications. These findings were recognized as calcified guinea worms within the breast tissue. This case report aims to highlight the mammographic findings of this rare infectious disease.

Barry, Shannon K.; Schucany, William Gregory

2012-01-01

219

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

220

Possible experimental manifestations of the many-body localization  

Microsoft Academic Search

Recently, it was predicted that if all one-electron states in a\\u000anon-interacting disordered system are localized, the interaction between\\u000aelectrons in the absence of coupling to phonons leads to a finite-temperature\\u000ametal-insulator trnasition. Here we show that even in the presence of a weak\\u000acoupling to phonons the transition manifests itself (i) in the nonlinear\\u000aconduction, leading to a bistable

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

2007-01-01

221

Psychological picture of manifest and latent carbon disulphide poisoning  

Microsoft Academic Search

Hänninen, H. (1971).Brit. J. industr. Med.,28, 374-381. Psychological picture of manifest and latent carbon disulphide poisoning. A battery of psychological tests was administered to 50 viscose workers with carbon disulphide (CS2) poisoning, 50 viscose workers exposed to CS2 without known poisoning, and 50 workers not exposed to CS2. There were large and statistically significant differences between the group means of

Helena Hänninen

1971-01-01

222

Silent Intravascular Lymphoma Initially Manifesting as a Unilateral Adrenal Incidentaloma  

PubMed Central

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

Takahashi, Yoshiko; Iida, Keiji; Hino, Yasuhisa; Ohara, Takeshi; Kurahashi, Toshifumi; Tashiro, Takashi; Chihara, Kazuo

2012-01-01

223

Oral manifestations in Rett syndrome: a study of 17 cases  

Microsoft Academic Search

Seventeen patients with a mean age of 7.33 (range 2.7- 12.7) years with Rett syndrome (a progressive neurologi- cal disorder that occurs mainly in females) were evaluated for oral manifestations and habits. The most frequent habits were digit~hand sucking and~or biting (17\\/17), bruxism (14\\/17), mouth breathing (7\\/17), drooling (5\\/17), tongue thrusting (5\\/17). Gingivitis (13\\/17) was the common alteration of soft

Rosangela Almeida Ribeiro; Regina Romano

1997-01-01

224

Multiple hymenoptera stings in children: clinical and laboratory manifestations  

Microsoft Academic Search

Multiple hymenoptera stings are a cause of rhabdomyolysis, elevated liver enzymes, clotting abnormalities, kidney injury,\\u000a and even death. However, the progression of the clinical and laboratory findings has been described mainly in sporadic case\\u000a reports. We report the clinical and laboratory manifestations of multiple hymenoptera stings in six children who were hospitalized\\u000a and referred for a nephrology evaluation and follow-up

Arnon Broides; Michal S. Maimon; Daniel Landau; Matitiahu Lifshitz

2010-01-01

225

CNS manifestations in non-Hodgkin lymphomas (NHL)  

Microsoft Academic Search

Summary In a group of 241 patients with non-Hodgkin lymphoma investigated retrospectively, CNS manifestations occurred in 8%, mainly as meningeosis lymphoblastomatosa. Lymphoblastic and immunoblastic NHL showed the highest risk of CNS infiltration (40.7% and 12.5% respectively). Further risk factors were disseminated stage of the disease, prior involvement of the bone marrow and juvenile age. Characteristic symptoms were eye muscle paresis,

M. Freund; P. Ostendorf; V. H. Gärtner; H. D. Waller

1983-01-01

226

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

227

Manifest electromagnetic duality in closed superstring field theory  

Microsoft Academic Search

The free action for massless Ramond-Ramond fields is derived from closed superstring field theory using the techniques of Siegel and Zwiebach. For the uncompactified Type IIB superstring, this gives a manifestly Lorentz-covariant action for a self-dual five-form field strength. Upon compactification to four dimensions, the action depends on a U(1) field strength from 4D N = 2 supergravity. However, unlike

Nathan Berkovits

1996-01-01

228

Empirical manifestations of power among adult learners in online discussions  

Microsoft Academic Search

This study examined the nature of power manifestations in a specific online learning setting. The two online classes selected\\u000a for this study were Master’s level courses in a professional school at a large state university in the United States. A total\\u000a of 1340 postings were made in the two classes over the span of the semester. To test the research

JuSung Jun

2007-01-01

229

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

230

Citrullination in extra-articular manifestations of rheumatoid arthritis  

Microsoft Academic Search

Background. Anti-citrullinated protein antibodies have been detected with high specificity in serum of patients with rheumatoid arthritis (RA), and citrullination of proteins may play a key role in the pathogenesis of RA. We therefore investigated the presence of citrullination in two extra-articular manifestations of RA, interstitial pneumonia (IP) and rheumatoid nodules. Methods. Open-lung biopsy specimens from patients with RA-associated IP

T. Bongartz; T. Cantaert; S. R. Atkins; P. Harle; J. L. Myers; C. Turesson; J. H. Ryu; D. Baeten; E. L. Matteson

2007-01-01

231

MRI manifestations and differentiated diagnosis of postoperative spinal complications  

Microsoft Academic Search

Summary  To analyze MR manifestations of postoperative spinal complications and investigate the value of MRI in the diagnosis and differentiated\\u000a diagnosis, 114 cases of spinal postoperative complications were analyzed retrospectively and compared with the clinical data.\\u000a The results showed that the main postoperative spinal complications included spinal stenosis (n=33, consisting of 21 cases of epidural fibrosis and 12 cases of epidural

Haitao Yang; Renfa Wang; Tianyou Luo; Yu Ouyang; Fajin Lv; Liming Xia; Chengyuan Wang

2009-01-01

232

Republished review: Ocular manifestations of the antiphospholipid syndrome  

Microsoft Academic Search

Antiphospholipid syndrome (APS) is an autoimmune disease characterised by a heterogenous group of antibodies directed against negatively charged phospholipids including antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) and ?-2 glycoprotein I (a?-2-GP1). The major features of this disorder include arterial and venous thrombosis and recurrent fetal loss. The vasculature of the eye is frequently involved and may be the presenting manifestation.

Virginia Miraldi Utz; Johnny Tang

2010-01-01

233

Head and neck cancers manifested as deep neck infection  

Microsoft Academic Search

The incidence of head and neck cancers in patients with an initial presentation of deep neck infection is unclear and may\\u000a be underestimated. Thus, the aim of this study was to assess the incidence of head and neck cancers initially manifested as\\u000a deep neck infection. Also, the possible risk factors and pathophysiology are discussed. This study was a retrospective medical

Yuan-Yung Lin; Chiang-Hung Hsu; Jih-Chin Lee; Hsing-Won Wang; Yaoh-Shiang Lin; Chih-Hung Wang; Chuan-Hsiang Kao; Wan-Fu Su; Yueng-Hsiang Chu

234

Stiff-person syndrome first manifesting in pregnancy.  

PubMed

Stiff-person syndrome (SPS) is a rare neurological disorder characterised by progressive stiffness and painful muscle spasms. We present a case of the autoimmune form of glutamate decarboxylase-positive SPS that initially manifested in pregnancy. The diagnosis was made based on clinical, laboratory and electromyoneurographic criteria. The patient was administered low doses of diazepam and baclofen. Considering the clinical picture of SPS patients, caesarean section is the method of choice for pregnancy termination. PMID:19005261

Cerimagic, Denis; Bilic, Ervina

2008-11-13

235

Pulmonary Embolism as the First Manifestation of Multiple Myeloma  

PubMed Central

Multiple myeloma is considered a hypercoagulable state due to several mechanisms such as the increased IL-6 and immunoglobulins production, the defective fibrinolytic mechanism, and the acquired resistance to activated protein C that are involved in the pathogenesis and clinical futures of the disease. We describe a case of a female patient who presented to the hospital with pulmonary embolism as the first manifestation of the hypercoagulability of multiple myeloma.

Vallianou, N.; Lazarou, V.; Tzangarakis, J.; Barounis, R.; Sioula, E.

2013-01-01

236

Plasmacytoma of the pancreas: an unusual manifestation of multiple myeloma.  

PubMed

Extramedullary plasmacytoma of the pancreas is a rare entity. Although this condition is uncommon, it should be considered in the differential diagnosis of solid mass in the pancreas, especially in patients with underlying multiple myeloma. We report a case of pancreatic plasmacytoma in a 56-year-old woman with newly diagnosed multiple myeloma. We highlight this rare manifestation of multiple myeloma among other better recognised presentations. PMID:23716161

Hue, Susan Swee Shan; Azhar, Rafay

2013-05-01

237

Unusual thoracic CT manifestations of osteosarcoma: review of 16 cases  

Microsoft Academic Search

Pulmonary metastases are common in osteosarcoma and the most common appearance is of multiple well-defined nodules in the\\u000a lung parenchyma. However, a variety of atypical locations and presentations of osteosarcoma metastasis can occur in the thorax.\\u000a We present a review of the thoracic CT findings in 16 patients with histopathologically confirmed osteosarcoma with unusual\\u000a thoracic manifestations. The 16 patients were

Ruchi Rastogi; Rachana Garg; Sanjay Thulkar; Sameer Bakhshi; Ajay Gupta

2008-01-01

238

Small vessel vasculitis as first manifestation of paracoccidioidomycosis - case report.  

PubMed

Paracoccidioidomycosis is a systemic disease caused by inhalation of conidia of Paracoccidioides brasiliensis, a dimorphic fungus that initially affects the airway, spreading by lymphatic and hematogenous routes to various organs and systems, and that can be fatal if the diagnosis and treatment are not correct. The authors describe a patient who presents small vessel vasculitis as an initial manifestation of the disease, which is an atypical clinical form with a single description in the literature. PMID:22281916

Caldeira, Alessandra Cristiana de Barros Figueiredo; Cas, Kelly da; Pinto, Taili Pedroso Lemes; Zômpero, Camila Machado; Pés, Cacinele; Guolo, Cristian Eduardo

239

Microthrombotic\\/Microangiopathic Manifestations of the Antiphospholipid Syndrome  

Microsoft Academic Search

The paper presents an overview of clinical manifestations and histopathologic findings in different organs in microvascular\\u000a thrombotic and microangiopathic antiphospholipid syndrome (MAPS). Subsets of antiphospholipid syndrome (APS) are presented\\u000a and defined. Clinico-pathologic correlations seem insufficient so far, because of a lack of detailed systematic studies of\\u000a the histopathology in different organs. Based on their own autopsy and biopsy studies, the

Sonja Praprotnik; Dušan Ferluga; Alenka Vizjak; Anastazija Hvala; Tadej Av?in; Blaž Rozman

2009-01-01

240

[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

241

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

2013-06-27

242

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

243

Ocular manifestations as key features for diagnosing mucopolysaccharidoses.  

PubMed

Diagnosis of mucopolysaccharidosis (MPS) requires awareness of the multisystem disease manifestations and their diverse presentation in terms of time of onset and severity. Many patients with MPS remain undiagnosed for years and progressively develop irreversible pathologies, which ultimately lead to premature death. To foster timely treatment and ensure a better outcome, it is of utmost importance to recognize and evaluate the typical ocular features that present fairly early in the course of the disease in many children with MPS. These include corneal clouding, ocular hypertension/glaucoma, retinal degeneration, optic disc swelling and optic nerve atrophy. Other associations include pseudo-exophthalmos, amblyopia, strabismus and large refractive errors requiring spectacle correction. While some ocular manifestations require specialized equipment for detecting abnormalities, light sensitivity, pseudo-exophthalmos and strabismus are often apparent on a routine physical examination. In addition, patients may be symptomatic from vision impairment, photosensitivity, night blindness and visual field constriction. Combined with the skeletal/joint complications and other manifestations, these ocular features are key in the differential diagnosis of children with joint abnormalities. Rheumatologists should have a high index of suspicion for MPS to facilitate early diagnosis. Referral to a geneticist, a metabolic specialist or physician who specializes in MPS can confirm the diagnosis and provide disease management. Consultation with an ophthalmologist who has expertise in MPS is also needed for thorough examination of the eyes and regular follow-up care. PMID:22210668

Summers, C Gail; Ashworth, Jane L

2011-12-01

244

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

Code of Federal Regulations, 2013 CFR

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

2013-04-01

245

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

246

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

247

Isolated cilioretinal artery occlusion as an initial manifestation of polycythemia vera.  

PubMed

Isolated cilioretinal artery occlusion is a rarely reported initial manifestation of polycythemia vera. In this study, we reported a case of a 65-year-old man with polycythemia vera with cilioretinal artery occlusion as an initial manifestation. PMID:20844687

Elasri, Fouad; Souhail, H; Reda, K; Iferkhass, S; Idrissi, A; Naoumi, A; Oubaaz, A

2010-07-01

248

Isolated Cilioretinal Artery Occlusion as an Initial Manifestation of Polycythemia Vera  

PubMed Central

Isolated cilioretinal artery occlusion is a rarely reported initial manifestation of polycythemia vera. In this study, we reported a case of a 65-year-old man with polycythemia vera with cilioretinal artery occlusion as an initial manifestation.

Elasri, Fouad; Souhail, H.; Reda, K.; Iferkhass, S.; Idrissi, A.; Naoumi, A.; Oubaaz, A.

2010-01-01

249

A study of neuropsychiatric manifestations in patients of neurocysticercosis  

PubMed Central

Background: Neurocysticercosis (NCC) is an endemic parasitic infection of Asia, Africa, Latin America, and central Europe. Neuropsychiatric manifestations of the illness include epilepsy and behavioral disturbances. There is a dearth of systematic studies on psychiatric manifestations of NCC from various Asian counties. The present study assessed the prevalence of various psychiatric disorders in a cohort of patients with NCC attending a neurological service. Materials and Methods: Detailed psychiatric assessment was carried out on 50 patients of NCC with epilepsy and 50 patients of epilepsy without any evidence of NCC. Comprehensive Psychopathological Rating Scale was used to elicit the symptoms. Cognitive functions were assessed using Mini Mental Status Examination. Psychiatric diagnoses were made as per International Classification of Diseases, 10th edition (ICD-10). Results: Sixty eight percent of the patients with NCC suffered from a psychiatric disorder, as compared to 44% of those without NCC (P=0.02). Major depression and mixed anxiety depression were the two most common diagnoses. None of the patients was to found to suffer from a psychotic disorder. The most frequent site of brain lesion of NCC was the parietal lobe, followed by frontal lobes and disseminated lesions. Left sided lesions were associated with greater psychiatric morbidity. Focal seizures with or without secondary generalizations were present more frequently in patients with NCC whereas primary generalized seizures were more common in patients with idiopathic epilepsy (P=0.05). Conclusion: Psychiatric manifestations are more common in patients of epilepsy with NCC than those without NCC. The treating clinician need to be vigilant about the phenomenon.

Srivastava, Smita; Chadda, Rakesh Kumar; Bala, Kiran; Majumdar, Pradipta

2013-01-01

250

[Electrovectocardiographic manifestations of left ventricular and biventricular growth].  

PubMed

The basic criteria for the electrical diagnosis of left ventricular and biventricular enlargements are discussed on the basis of the myocardial depolarization and repolarization sequence. Left ventricular dilatation secondary to isolated diastolic overloading increases the manifestation of the main vectors resulting from the activation of this ventricle. These changes reflect the proximity of the left ventricular walls to the exploring electrodes. The above mentioned vectors appear as tall R waves and wide ventricular curves with counterclockwise rotation on the three planes. If the diastolic overload is a isolated phenomenon, T waves are positive and asymmetric on the left leads while the T loop, of secondary type, is concordant in its orientation with the R loop. This fact is due to a prolonged duration of the repolarization phase of the left ventricle. Global left ventricular hypertrophy produced by a sustained systolic overloading increases the magnitude and manifestation of all the vectors resulting from the depolarization of this ventricle (I, II l, III l) owing to the prolonged duration of the corresponding activation fronts. When LBBB is also present, the first septal vector is not evident. In extreme degrees of the systolic overload, the T wave is inverted and shows morphologic secondary characteristics in left leads, and the T loop opposes the R loop on frontal and horizontal planes. The directional changes of the repolarization fronts of free left ventricular walls can satisfactorily explain these features. Left ventricular hypertrophy of a segmentary type, such as that observed in idiopathic myocardiopathy, generally increases the magnitude and manifestation of septal vector I and II left. When both ventricles are hypertrophied, the electromotive forces originating in the more severely affected heart chamber predominate in electrical records. PMID:2967067

de Micheli, A; Medrano, G A

251

Skin Manifestations Associated With Mantle Cell Lymphoma: a Case Report  

PubMed Central

Mantle cell lymphoma (MCL) is a distinct non-Hodgkin’s lymphoma type that commonly affects extra nodal sites. The most often affected sites are bone marrow, gastrointestinal tract and Waldeyer’s ring, being the skin rarely involved. We report a case of 56 year-old man with MCL, exhibiting multiple large maculopapular skin rashes and skin ulcers. Histopathological examination had not shown direct infiltration by any atypical cells. He had significant improvement of skin lesions with combination chemotherapy and debridement. Awareness of skin manifestations of MCL is crucial for dermatologists and haematologists to establish the early diagnosis and timely administration of appropriate treatment.

Singh, Avinash Kumar; Dixit, Gaurav; Sharma, Sanjeev; Kumar, Suman; Yadav, Rajni; Agrawal, Narendra; Mishra, Pravas; Seth, Tulika; Sarkar, C.; Mahapatra, M.; Saxena, Renu

2013-01-01

252

Skin manifestations associated with mantle cell lymphoma: a case report.  

PubMed

Mantle cell lymphoma (MCL) is a distinct non-Hodgkin's lymphoma type that commonly affects extra nodal sites. The most often affected sites are bone marrow, gastrointestinal tract and Waldeyer's ring, being the skin rarely involved. We report a case of 56 year-old man with MCL, exhibiting multiple large maculopapular skin rashes and skin ulcers. Histopathological examination had not shown direct infiltration by any atypical cells. He had significant improvement of skin lesions with combination chemotherapy and debridement. Awareness of skin manifestations of MCL is crucial for dermatologists and haematologists to establish the early diagnosis and timely administration of appropriate treatment. PMID:23505608

Singh, Avinash Kumar; Dixit, Gaurav; Sharma, Sanjeev; Kumar, Suman; Yadav, Rajni; Agrawal, Narendra; Mishra, Pravas; Seth, Tulika; Sarkar, C; Mahapatra, M; Saxena, Renu

2013-02-25

253

Silent intravascular lymphoma initially manifesting as a unilateral adrenal incidentaloma.  

PubMed

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed. PMID:22927863

Takahashi, Yoshiko; Iida, Keiji; Hino, Yasuhisa; Ohara, Takeshi; Kurahashi, Toshifumi; Tashiro, Takashi; Chihara, Kazuo

2012-08-13

254

PANMYELOPHTHISIS WITH HEMORRHAGIC MANIFESTATIONS IN RATS ON A NUTRITIONAL BASIS.  

PubMed

During the 12 months ending May, 1937, 72 rats were observed that manifested typical symptoms of panmyelophthisis. The disease may start as agranulocytosis, thrombocytopenia or pure red cell anemia, leading progressively, often rapidly, to aleukia hemorrhagica with its typical manifestations (epistaxis, melena, hematuria, purpura). Blood examinations revealed correspondingly low white cell, red cell and platelet counts with very pronounced granulocytopenia (0 to 4 per cent). Bone marrow smears and histological findings were consistent with the diagnosis of panmyelophthisis. Suprarenal hemorrhage was a frequent postmortem finding. The pathogenesis of this experimental panmyelophthisis and this hemorrhagic diathesis is confined to special nutritional conditions. These diseases have been observed by us in rats kept on a diet deficient in vitamin B(6), containing cane sugar and supplemented with vitamin B(1) and crystalline natural or synthetic lactoflavin. Lack of vitamin B(6), however, is not a necessary condition, since the disease was encountered in the majority of the animals after the specific deficiency disease which became apparent in rats kept on the B(6) free diet was successfully treated with purified B(6) preparations. Even in the untreated animals kept on the B(6) deficient diet acrodynia was, as a rule, in distinct remission before symptoms of panmyelophthisis and hemorrhagic diathesis became manifest. By means of the addition of Peters' eluate to the basal diet, panmyelophthisis could be prevented and, in animals where it was recognized in an early stage, cured. In view of these facts it is suggested that Peters' eluate contains a specific maturation factor for the primary blood-producing tissue, the reticulo-endothelium, a factor which, being different from lactoflavin, vitamin B(6) and probably also from the so called filtrate factor, constitutes another distinct component of the vitamin B(2) group. Bacteriological studies brought forward no conclusive positive evidence for the infectious etiology of the experimental panmyelophthisis in our rats. The possible relation of this new disease in rats to aleukia hemorrhagica and its partial manifestations in man, as well as to the so called Waterhouse-Friderichsen syndrome, is discussed. Administration of amidopyrine, at least under the conditions chosen, failed to provoke panmyelophthisis in rats kept on the same diet as that given to rats in which the disease spontaneously developed. PMID:19870685

György, P; Goldblatt, H; Miller, F R; Fulton, R P

1937-10-31

255

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

256

Cutaneous manifestations of anabolic-androgenic steroid use in athletes.  

PubMed

Increased public awareness of the performance-enhancing benefits of anabolic-androgenic steroids (AASs) in athletes has resulted from the media coverage of the BALCO scandal and Mitchell report. The impact of this increased notoriety on the consumption of these drugs remains unclear, however. In addition, the negative connotation associated with the use of AASs may make users less forthcoming when interacting with their physicians. Cutaneous manifestations develop early in the use of AASs, placing dermatologists in a unique position to make an early diagnosis of AAS abuse in patients who engage in competitive sports. This review of the literature focuses on dermatologic presentations of AAS use. PMID:19785085

Walker, Jennifer; Adams, Brian

2009-10-01

257

Enophthalmos as a rare manifestation of metastatic orbital involvement.  

PubMed Central

Two cases in which orbital metastasis produced enophthalmos, instead of the usual exophthalmos, are reported. Only fourteen other cases have been described previously and they are reviewed. At time of diagnosis of the enophthalmos, meningeal carcinomatosis coexisted in the first case and probably also in the second case. This association can lead to diagnostic errors with either the orbital metastasis or the meningeal carcinomatosis being missed. In addition, as in our second case, the enophthalmos can be the initial manifestation of cancer. Images Figure 1 Figure 2

de Keyser, J.; Bruyland, M.; de Greve, J.; Bernheim, J.; Ebinger, G.

1985-01-01

258

Ethnic and migrational impact on the clinical manifestation of depression  

Microsoft Academic Search

Purpose  Depressive disorders are still underdiagnosed. Ethnic and cultural factors may influence the way depression is presented and\\u000a therefore contribute to problems in assessing these disorders in different ethnic populations appropriately. In this investigation,\\u000a the impact of both ethnicity and migration on the manifestation of depression was studied.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  Three groups of depressed female patients (n = 136) were included in this investigation on

Eberhard A. Deisenhammer; Müberra Çoban-Ba?aran; At?l Mantar; Regina Prunnlechner; Georg Kemmler; Tunç Alk?n; Hartmann Hinterhuber

259

An alternative NMSSM phenomenology with manifest perturbative unification  

SciTech Connect

Can supersymmetric models with a moderate stop mass be made consistent with the negative Higgs boson searches at LEP, while keeping perturbative unification manifest? The NMSSM achieves this rather easily, but only if extra matter multiplets filling complete SU(5) representations are present at intermediate energies. As a concrete example which makes use of this feature, we give an analytic description of the phenomenology of a constrained NMSSM close to a Peccei-Quinn symmetry point. The related pseudo-Goldstone boson appears in decays of the Higgs bosons and possibly of the lightest neutralino, and itself decays into (b anti-b) and (tau anti-tau).

Hall, Lawrence; Barbieri, Riccardo; Pappadopulo, Duccio; Rychkov, Vyacheslav S.; Hall, Lawrence J.; Papaioannou, Anastasios Y.

2007-12-18

260

The blue man: a novel cutaneous manifestation of systemic amyloidosis.  

PubMed

Multiple cutaneous manifestations of amyloidosis have been described, and include periorbital purpura, plaques, nodules, and papules. Blue skin tint is not a previously described feature of cutaneous amyloidosis. We report the clinical case of a patient with known multiple myeloma and amyloidosis of the gastrointestinal tract who presented with progressive "blueness" of the skin. Dermatopathological examination and mass spectrometry of a skin biopsy specimen revealed the presence of subcutaneous amyloid light chain (AL) deposition. We believe this to be the first reported case of blue skin resulting from systemic amyloidosis. PMID:21506660

Campbell, Mark; Rosenthal, Allison; Kundranda, Madappa; Pickert, Amanda; Dicaudo, David; Dogan, Ahmet; Mikhael, Joseph

2011-04-20

261

[Clinical aspects, course and extrahepatic manifestations of hepatitis C].  

PubMed

Hepatitis C virus infection occasionally causes acute icteric hepatitis. However, primary infection is usually asymptomatic. Viral clearance occurs only in 20%; most patients develop a chronic hepatitis, which tends to be hardly symptomatic for many years. The risk of progression to cirrhosis, hepatocellular carcinoma and end-stage liver disease is associated with the severity of histopathological changes, and is increased by additional factors, such as excessive alcohol intake or coinfection with hepatitis B virus and HIV. Immunologically mediated extrahepatic manifestations of hepatitis C, in particular essential mixed cryoglobulinemia, can often be observed. Occasionally, they lead to fatal complications. PMID:11205176

Hartmann, G; Ben Ramadar, E B; Hübl, G; Sebesta, C

2000-01-01

262

Manifestation of resonance-related chaos in coupled Josephson junctions  

NASA Astrophysics Data System (ADS)

Manifestation of chaos in the temporal dependence of the electric charge is demonstrated through the calculation of the maximal Lyapunov exponent, phase-charge and charge-charge Lissajous diagrams and correlation functions. It is found that the number of junctions in the stack strongly influences the fine structure in the current-voltage characteristics and a strong proximity effect results from the nonperiodic boundary conditions. The observed resonance-related chaos exhibits intermittency. The criteria for a breakpoint region with no chaos are obtained. Such criteria could clarify recent experimental observations of variations in the power output from intrinsic Josephson junctions in high temperature superconductors.

Shukrinov, Yu. M.; Hamdipour, M.; Kolahchi, M. R.; Botha, A. E.; Suzuki, M.

2012-11-01

263

Circumscribed alopecia: an unusual manifestation of pediculosis capitis.  

PubMed

A healthy 10-year-old girl was referred for evaluation of patchy hair loss on the scalp of longer than 6 months duration. She had been diagnosed and treated for head lice approximately 1 month before onset of the hair loss. Examination of the scalp showed discrete ovoid patches of hair loss at the vertex scalp. A scrape of the area of hair loss was performed, and a nit was visible on microscopic examination. Focal hair loss may represent an atypical manifestation of ongoing pediculosis capitis. PMID:22011127

Hall, Rebecca R; McMichael, Amy J

2011-10-20

264

Subacute pulmonary manifestation in a survivor of severe paraquat intoxication.  

PubMed

Paraquat has been widely used as a nonselective contact herbicide since 1962. It is highly toxic for humans, and many cases of acute poisoning, especially intentional self-poisoning, have been reported over the past few decades in developing countries. Ingestion of a threshold volume results in multiple organ failure and death after a longer period of time, but aggressive clinical studies are rarely done when the diagnosis is clear. We report the case of a patient who survived severe paraquat intoxication; he presented with subacute pulmonary manifestations including physiologic dysfunction and abnormalities on radiographs. PMID:16284488

Huang, Chung-Jen; Yang, Mei-Chen; Ueng, Shir-Hwa

2005-11-01

265

C3b inactivator deficiency with immune complex manifestations.  

PubMed Central

We report a complete C3b inactivator deficiency in a 28-year-old patient referred for bronchiectasis and recurrent pneumonia. In addition to these recurrent infections, previously described with this deficiency, he also had several immune complex manifestations (rheumatoid factor, circulating immune complexes and one episode of serum sickness). The consequences of C3b inactivator deficiency on complement activation, chemotaxis and opsonization, clearance of immune complexes, and on red blood cell sensitization are discussed. The study of the parents showed an autosomal dominant transmission. Images Fig. 1

Solal-Celigny, P; Laviolette, M; Hebert, J; Atkins, P C; Sirois, M; Brun, G; Lehner-Netsch, G; Delage, J M

1982-01-01

266

PANMYELOPHTHISIS WITH HEMORRHAGIC MANIFESTATIONS IN RATS ON A NUTRITIONAL BASIS  

PubMed Central

During the 12 months ending May, 1937, 72 rats were observed that manifested typical symptoms of panmyelophthisis. The disease may start as agranulocytosis, thrombocytopenia or pure red cell anemia, leading progressively, often rapidly, to aleukia hemorrhagica with its typical manifestations (epistaxis, melena, hematuria, purpura). Blood examinations revealed correspondingly low white cell, red cell and platelet counts with very pronounced granulocytopenia (0 to 4 per cent). Bone marrow smears and histological findings were consistent with the diagnosis of panmyelophthisis. Suprarenal hemorrhage was a frequent postmortem finding. The pathogenesis of this experimental panmyelophthisis and this hemorrhagic diathesis is confined to special nutritional conditions. These diseases have been observed by us in rats kept on a diet deficient in vitamin B6, containing cane sugar and supplemented with vitamin B1 and crystalline natural or synthetic lactoflavin. Lack of vitamin B6, however, is not a necessary condition, since the disease was encountered in the majority of the animals after the specific deficiency disease which became apparent in rats kept on the B6 free diet was successfully treated with purified B6 preparations. Even in the untreated animals kept on the B6 deficient diet acrodynia was, as a rule, in distinct remission before symptoms of panmyelophthisis and hemorrhagic diathesis became manifest. By means of the addition of Peters' eluate to the basal diet, panmyelophthisis could be prevented and, in animals where it was recognized in an early stage, cured. In view of these facts it is suggested that Peters' eluate contains a specific maturation factor for the primary blood-producing tissue, the reticulo-endothelium, a factor which, being different from lactoflavin, vitamin B6 and probably also from the so called filtrate factor, constitutes another distinct component of the vitamin B2 group. Bacteriological studies brought forward no conclusive positive evidence for the infectious etiology of the experimental panmyelophthisis in our rats. The possible relation of this new disease in rats to aleukia hemorrhagica and its partial manifestations in man, as well as to the so called Waterhouse-Friderichsen syndrome, is discussed. Administration of amidopyrine, at least under the conditions chosen, failed to provoke panmyelophthisis in rats kept on the same diet as that given to rats in which the disease spontaneously developed.

Gyorgy, Paul; Goldblatt, Harry; Miller, Franklin R.; Fulton, Robert P.

1937-01-01

267

Neonatal erythroderma as a first manifestation of Menkes disease.  

PubMed

Menkes disease is an X-linked recessive lethal multisystemic disorder of copper metabolism. Progressive neurodegeneration, connective tissue disturbances, and peculiar kinky hair are the main manifestations. The low serum copper and ceruloplasmin suggests the diagnosis, which is confirmed by mutation analysis of the ATP7A gene. We report an exceptional presentation of classic Menkes disease with neonatal erythroderma. Genetic study revealed a deletion in exons 8 to 12 in the ATP7A gene. This study could allow pediatricians and pediatric dermatologists to diagnose the disorder as early as possible to establish prompt treatment with parenteral copper-histidine supplementation to improve prognosis. PMID:22711717

Galve, Javier; Vicente, Asunción; González-Enseñat, María Antonia; Pérez-Dueñas, Belén; Cusí, Victoria; Møller, Lisbeth Birk; Julià, Marc; Domínguez, Anna; Ferrando, Juan

2012-06-18

268

Risk prediction and risk reduction in patients with manifest arterial disease  

Microsoft Academic Search

Risicovoorspelling en risicoverlaging bij patienten met manifest vaatlijden Engelstalig abstract The number of patients with clinical manifest arterial disease is increasing because of the aging of the population. Patients with manifest arterial disease have an increased risk of a new vascular event in the same or different arterial bed. Medical treatment of vascular risk factors (hypertension, hyperlipidemia, diabetes mellitus) and

B. M. B. Goessens

2006-01-01

269

Oral manifestations in hypertensive patients: A clinical study  

PubMed Central

Aim: The aim of this work was to determine the frequency and nature of oral manifestations in a hypertensive patient. Materials and Methods: The study sample consisted of 465 known hypertensive patients, between the age group of 20-80 years of which 250 patients were males and 215 were females. 105 were between the age group of 1-40 years, 136 in 41-50 years, 110 in 51-60 years, and 114 in 60 and above year of age. Data were statistically analyzed Student's t-test. Results: 79.14% of the patients presented with Russell's periodontal index score ranging from 2- 4.9 which suggested an established periodontal lesion. The risk factors for old men and women to develop hypertension were 90%, among those who survived to ages 65 to 89 years. Conclusion: The frequency of potential oral manifestations in patients with hypertension was significantly high, thus showing an association of gingival and periodontal pathology in hypertensive patients

Kumar, Prashant; Mastan, KMK; Chowdhary, Ramesh; Shanmugam, K

2012-01-01

270

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

2012-10-05

271

Symptomatic brain involvement as the initial manifestation of neuromyelitis optica.  

PubMed

Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder that predominantly affects the optic nerve and spinal cord; however, symptomatic brain involvement is not rare and is sometimes an initial manifestation in NMO. In this study, we investigated the characteristic features of patients with NMO with symptomatic brain involvement as the initial manifestation of disease (NMO(brain)) compared with patients with NMO who presented initially with optic neuritis or myelitis (NMO(ON/myelitis)). We retrospectively reviewed 27 consecutive Korean patients with NMO with aquaporin-4 antibodies. Patients with NMO(brain) (n=9) initially presented with intractable hiccup/nausea/vomiting and/or encephalopathy at a younger age than the patients with NMO(ON/myelitis) (n=18) (p<0.01). During the disease course, the patients with NMO(brain) continued to show more frequent symptomatic involvement of the brain than the 18 patients with NMO(ON/myelitis) (p<0.05). At the final visit, the mean age was also significantly lower in patients with NMO(brain) than in patients with NMO(ON/myelitis) (p<0.01); however, the Expanded Disability Status Scale scores, used to evaluate disease progression, were not different between the two groups. Our study suggests that patients with NMO who present initially with symptomatic brain involvement may have earlier disease onset and become disabled at a younger age compared to patients with typical NMO. Additional large scale prospective studies are warranted. PMID:23673142

Min, Ju-Hong; Waters, Patrick; Vincent, Angela; Kang, Eun-Suk; Lee, Sujin; Lee, Dong Kyoo; Lee, Kwang Ho; Kim, Byoung Joon

2013-05-11

272

Analysis of Recent Manifests for Goods Imported through US Ports  

SciTech Connect

Several active interrogation techniques are being developed to detect shielded special nuclear materials (SNM) hidden in cargo containers loaded on container ships arriving at US ports. It raises the questions of the types of cargos in which SNM could be hidden, and their impact on detected signatures. Since the definition of a set of ''typical'' or standard cargos has proven to be difficult, we analyzed shipping manifests for US imports shipped through North American ports collected on 14 days distributed over 12 months. From these data, we generated several distribution functions such as commodity categories, average densities, and packaging types that could be of interest to the cargo scanning community. One of the cargo scanning techniques currently under development at LLNL is based on neutron active interrogation, and relies on the unique signature of beta-delayed gammas emitted by fission products in the 3 to 7 MeV energy range. {sup 19}F(n,a){sup 16}N, has been identified as the main potential interference for 7 and 9 MeV neutron beams. Estimates of cargo compositions based on manifests identified as containing fluorine are presented.

Descalle, M; Manatt, D; Slaughter, D

2006-09-27

273

Monoclonal gammopathies and the related autoimmune manifestations in Taiwan.  

PubMed

A total of 50,000 patients were surveyed for the presence of monoclonal immunoglobulins during the past two decades. There were 411 cases of monoclonal gammopathies including 243 cases of plasma cell neoplasms and 168 cases of secondary plasma-cell dyscrasia. Among the 227 cases of multiple myeloma and Waldenström's macroglobulinemia, there were 49.3% IgG class, 22.9% IgA class, 9.7% IgM class and 13.2% light chain type. In addition, there were 1.3% of nonexcretory myeloma including an IgM type. A relatively high frequency (4.8%) of IgD M-proteins was detected but heavy chain disease was not encountered in the present series. Purified M-components from patients with possible autoimmune manifestations were subjected to immunofluorescence studies. Autoimmune activity of M-proteins was found in a patient of Waldenström's macroglobulinemia with peripheral neuropathy, and another patient of cryofibrinogenemia with recurrent purpura and gangrene. In conclusion, a high frequency of IgD myeloma is found in Chinese patients of this area. M-components may have autoimmune activity resulting in unusual clinical manifestations. PMID:1305814

Wang, C R; Chuang, C Y; Lin, K T; Chen, M Y; Lee, G L; Hsieh, R P; Chen, C Y

1992-12-01

274

Oral manifestations in HIV-positive adults from Northern Thailand.  

PubMed

Eighty-seven HIV-infected patients in a provincial hospital in Northern Thailand were examined for oral manifestations of HIV disease and AIDS. The median age was 31.3 years. Seventy-four of the patients were women, 13 were men. 96.6% had a history of heterosexual transmission. Sixty-one patients were CDC-category A, 20 were category B and 6 were category C (AIDS). Thirty-eight percent of the patients revealed oral lesions; 23% had one oral lesion and 13.8% had two oral lesions. Common lesions were oral candidiasis (10.3% pseudomembranous candidiasis, 6.9% erythematous candidiasis and 3.4% both forms), oral hairy leukoplakia (11.5%) and exfoliative cheilitis (6.9%). Gingival linear erythema was seen in 8% of the patients; periodontal lesions and necrotising ulcerative gingivitis were not observed. Men were more commonly affected by oral manifestations than women (P < 0.004). The spectrum of oral lesions is comparable to other studies from the region, although most of these reported more men than women. Also, the degree of immunosuppression was more marked (AIDS). PMID:11302241

Khongkunthian, P; Grote, M; Isaratanan, W; Plyaworawong, S; Reichart, P A

2001-04-01

275

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

Lernia, Vito Di; Mansouri, Yasaman

2013-10-01

276

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

277

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

278

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

279

Oral manifestations of Ellis-van Creveld syndrome  

PubMed Central

Ellis-van Creveld syndrome is a rare autosomal-recessive disorder characterized by short limbs, post-axial polydactyly, ectodermal dysplasia, edentulous mandibular incisor region, absence of mucobuccal fold, congenitally missing teeth, slight serrations of the alveolar ridge and multiple small alveolar notches. The clinical report not only describes the classical oral and dental manifestations of Ellis-van Creveld syndrome but also presents unusual findings such as single-rooted and funnel-shaped primary first molars, single conical roots of primary second molars and taurodontisum, which must be considered in the differential diagnostic criteria to avoid misdiagnosis of syndromes. The article also discusses the differential diagnosis and preventive and therapeutic oral health care for these patients. The management of Ellis-van Creveld syndrome is multidisciplinary and, therefore, the oral health care provider should get updated with latest knowledge for timely referral to prevent the patient from further complications of heart defect and bony deformity.

Kalaskar, Ritesh; Kalaskar, Ashita R.

2012-01-01

280

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

2013-02-26

281

Neurological manifestations of avian influenza viruses in mammals.  

PubMed

The H5N1 viruses isolated from humans in Hong Kong directly infected both mice and ferrets without prior adaptation to either host. Two representative viruses, A/Hong Kong/483/97 (HK/483) and A/Hong Kong/486/97 (HK/486) were equally virulent in outbred ferrets but differed in their virulence in inbred mice. Both HK/483 and HK/486 replicated systemically in ferrets and showed neurologic manifestations. In contrast, intranasal infection of mice with HK/483, but not HK/486, resulted in viral spread to the brain, neurologic signs, and death. However, HK/486 was able to replicate in the brain and induce lethal disease following direct intracerebral inoculation. PMID:14575126

Rowe, T; Cho, D S; Bright, R A; Zitzow, L A; Katz, J M

2003-01-01

282

Acute respiratory failure as a first manifestation of syringomyelia  

PubMed Central

A 40 year old woman presented with a short history of acute onset of breathlessness to the ER of our hospital and after initial evaluation for acute pulmonary embolism which was ruled out after carrying out the appropriate investigations, she was diagnosed to be afflicted with syringomyelia based on her neurological symptoms and clinical findings, which was confirmed by doing an MRI scan, which was her basic diagnosis that was complicated by acute hypercapnic respiratory failure. This case is being reported to highlight syringomyelia as an unusual cause of acute respiratory failure, which manifested clinically in this patient as its first presentation and the underlying neurological diagnosis has been found to be present in very few reported cases (less than 0.01% of case reports) in the available literature as the basic disease in the absence of its classical presenting features. Problems associated with acute respiratory failure in the setting of syringomyelia are discussed.

Al Bashapshe, Ali; Bhatia, Harsha; Aziz, Shahid

2010-01-01

283

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

284

Transient cortical blindness as a manifestation of solitary cysticercus granuloma.  

PubMed

Neurocysticercosis is recognised as a significant cause of neurological morbidity in endemic regions. The wide range of pleomorphic and non-specific neurological manifestations of neurocysticercosis must be kept in mind by physicians, as the disease has shown resurgence in developed countries. When an atypical presentation of an unusual tropical disease occurs in non-endemic regions, the diagnosis is often missed. We describe a case of a 4-year-old girl who presented with a history of transient bilateral loss of vision with headache and vomiting. Brain MRI revealed the presence of a single cysticercus granuloma in the occipital lobe. A diagnosis of symptomatic occipital lobe seizure secondary to neurocysticercosis was made. She was given a course of albendazole. There was no recurrence of symptoms at 3 years follow-up. Occipital seizures that are associated with ictal amaurosis closely mimic basilar migraine. Such cases benefit from neuroimaging in order to rule out the underlying structural causes. PMID:23230251

Hussain, Shabbir; Hussain, Kosar; Hussain, Sahar

2012-12-10

285

Reversible dementia as a presenting manifestation of racemose neurocysticercosis  

PubMed Central

Racemose cysticercosis is a less frequent presentation of neurocysticercosis (NCC). It's presentation and management is quite different from cerebral parenchymal NCC. Diagnosis of racemose cysticercosis is based on the combination of clinical, epidemiologic, radiographic, and immunologic information. Compared with cysticercus cellulose, which most commonly presents as seizures, racemose NCC due to its extraaxial location presents with raised intracranial pressure and meningitis, and frequently requires neurosurgical intervention. Dementia as a sole presenting feature of NCC is rare. We report a case of racemose NCC with dementia as the presenting manifestation. The outcome of dementia patients with NCC seems favorable in most cases therefore a high index of suspicion for NCC should be kept especially in endemic areas.

Sharma, Sudhir; Modi, Manish; Lal, Vivek; Prabhakar, Sudesh; Bhardwaj, Amit; Sehgal, Rakesh

2013-01-01

286

Secondary syphilis: uncommon manifestations of a common disease.  

PubMed

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. PMID:6702190

McPhee, S J

1984-01-01

287

Star copolymers in porous environments: scaling and its manifestations.  

PubMed

We consider star polymers, consisting of two different polymer species, in a solvent subject to quenched correlated structural obstacles. We assume that the disorder is correlated with a power-law decay of the pair-correlation function g(x)~x(-a). Applying the field-theoretical renormalization group approach in d dimensions, we analyze different scenarios of scaling behavior working to first order of a double ?=4-d, ?=4-a expansion. We discuss the influence of the correlated disorder on the resulting scaling laws and possible manifestations such as diffusion-controlled reactions in the vicinity of absorbing traps placed on polymers as well as the effective short-distance interaction between star copolymers. PMID:21405706

Blavatska, V; von Ferber, C; Holovatch, Yu

2011-01-13

288

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

289

Right putamen hemorrhage manifesting as apraxia of eyelid opening  

PubMed Central

Purpose The purpose of this report is to demonstrate a rare clinical manifestation of apraxia eyelid opening related to a basal ganglia lesion. Case report In this study, we report a 91-year-old woman suffering from difficulty in eyelid opening after being treated for myocardial ischemia with dual antiplatelet medications. She could open her eyelid with fingers touching her forehead. Brain computed tomography revealed a right putamen hemorrhage. Surface electromyography revealed persistent frontalis muscle contraction during relaxation of orbicularis oculi muscles. Apraxia of eyelid opening was diagnosed. Her eyelid symptom resolved 2 months later. Conclusion Apraxia of eyelid opening may be caused by subcortical hemorrhage of the basal ganglia. In addition to the primary motor cortex and supplemental motor area, the basal ganglia may also play a role in eyelid opening.

Lin, Yi-Hui; Liou, Li-Min; Lai, Chiou-Lian; Chang, Yang-Pei

2013-01-01

290

Triple A syndrome with ophthalmic manifestations in two siblings.  

PubMed

Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by adrenocorticotropic hormone resistant adrenal insufficiency, alacrima, achalasia of the esophageal cardia, progressive neurological degeneration and occasionally autonomic instability. We report the ophthalmic manifestations in 2 siblings from a consanguineous family with this syndrome. A routine ophthalmic examination showed absence of palpebral portion of lacrimal gland, dry eyes and sluggish pupillary reflexes in both eyes of these patients. Both of these patients had achalasia cardia while the boy additionally had increased serum cortisol levels. Topical ocular lubricants were prescribed for both of them and Heller's cardiomyotomy with fundus plication was done for achalasia. These cases would create an awareness of this rare condition in ophthalmology in addition to suggesting its management. PMID:17595484

Babu, Kalpana; Murthy, Krishna R; Babu, Narendra; Ramesh, S

291

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma  

PubMed Central

Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas.??

Lee, E; Maselli, R; Ellis, W; Agius, M

1998-01-01

292

Hypertensive emergency: A unique manifestation of a pituitary disorder  

PubMed Central

Acromegaly a common pituitary disorder has significant adverse effects on well-being and survival. The slight increase in the prevalence of hypertension in acromegaly is well known and is proposed to be the direct effects of growth hormone. The hypertension for calculating the prevalence in most series was defined as diastolic more than 100 mmHg, but hypertensive emergency is rarely ever described in the literature. Growth hormone excess has been associated with renal manifestations such as hypercalciuria and nephrolithiasis, but never with renal failure. We present a case referred to a tertiary care nephrology center with malignant hypertension. This is the first case of a patient of acromegaly presenting with hypertensive emergency progressing to malignant nephrosclerosis and renal failure.

Yanamandra, Uday; Jairam, Anantharam; Kotwal, Narendra; Venkata, Baliga Krishna; Nair, Velu

2013-01-01

293

Clinical and pathological manifestations of human henipavirus infection.  

PubMed

The clinicopathological features of human Nipah virus and Hendra virus infections appear to be similar. The clinical manifestations may be mild, but if severe, includes acute encephalitic and pulmonary syndromes with a high mortality. The pathological features in human acute henipavirus infections comprise vasculopathy (vasculitis, endothelial multinucleated syncytia, thrombosis), microinfarcts and parenchymal cell infection in the central nervous system, lung, kidney and other major organs. Viral inclusions, antigens, nucleocapsids and RNA are readily demonstrated in blood vessel wall and numerous types of parenchymal cells. Relapsing henipavirus encephalitis is a rare complication reported in less than 10% of survivors of the acute infection and appears to be distinct from the acute encephalitic syndrome. Pathological evidence suggests viral recrudescence confined to the central nervous system as the cause. PMID:22427144

Wong, K T; Tan, C T

2012-01-01

294

Livedoid vasculitis: a manifestation of the antiphospholipid syndrome?  

PubMed

Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition. PMID:10215783

Acland, K M; Darvay, A; Wakelin, S H; Russell-Jones, R

1999-01-01

295

[Multiple phenotypic manifestations of X-linked spinobulbar muscular atrophy].  

PubMed

Recessive X-linked amyotrophic spinobulbar muscular atrophy (SBMA) or Kennedy disease is a neuroendocrine disorder with a slowly progressive phenotype, caused by an expansion of a polymorphic tandem CAG repeat of the androgen receptor gene. Classical clinical hallmarks include onset in the third decade of life, weakness and wasting predominantly in proximal extremity muscles, variable weakness of bulbar muscles, abundant muscle fasciculations, sensory nerve action potential abnormalities and signs of androgen insensitivity such as gynecomastia and testicular atrophy. The diagnosis has been recently made easier by the availability of genetic testing but Kennedy disease is probably still underdiagnosed because of phenotypic variability. We report 11 new cases, of which seven had atypical initial manifestations presenting respectively with myasthenia, cramps and fasciculation syndrome, polyneuropathy, post-trauma monomelic neuronopathy, effort-dependent muscle intolerance and/or muscular dystrophy, with the aim to enlarge the phenotypic spectrum of the published series. PMID:18667219

Vandenberghe, N; Bouhour, F; Petiot, P; Gonnaud, P-M; Latour, P; Broussolle, E; Vial, C

2008-07-29

296

Ictal Priapism as an Autonomic Manifestation of Panayiotopoulos Syndrome.  

PubMed

Panayiotopoulos syndrome is an idiopathic epilepsy syndrome presenting with a large variety of autonomic symptoms. The mechanism of autonomic symptoms is still not well understood. A neurologically normal 13-month-old boy presented on 2 occasions with complex partial status epilepticus that included significant ictal priapism. Inpatient brain magnetic resonance imaging (MRI) scan showed restricted diffusion-weighted imaging within his left temporal lobe and electroencephalograms (EEGs) left temporal slowing only. An outpatient EEG 6 months later showed abundant multifocal, predominantly posterior, as well as irregular generalized spike-and-slow-wave discharges on normal background consistent with the diagnosis of Panayiotopoulos syndrome. Ictal priapism is a previously undescribed phenomenon that is consistent with parasympathetic manifestations noted in this young boy. Acute postictal MRI and EEG findings suggest that this undeniably fascinating seizure semiological sign may be localized to the left temporal region. PMID:23112246

Brabec, Jiri; Chaudhary, Shuchi; Ng, Yu-Tze

2012-10-30

297

The neuropsychiatric manifestations of Huntington's disease-like 2.  

PubMed

Huntington's disease-like 2 (HDL2) is a rare neuropsychiatric disorder that resembles HD but results from a distinct mutation. The authors present a patient with HDL2, hospitalized for psychiatric management, and they review the neuropsychiatric manifestations of this disorder. Depression, irritability/aggression, and frontal lobe personality changes are common presentations of HDL2 and are comparable to classic HD. Patients with HDL2 may differ from those with HD in having a lower incidence of obsessive-compulsive acts, known suicides, antisocial acts, and changes in sexuality. Clinicians should be aware of the psychiatric presentations of this disorder, when to obtain genetic testing, and how to manage problematic behaviors. PMID:23224457

Fischer, Christopher A; Licht, Eliot A; Mendez, Mario F

2012-01-01

298

Rheumatic manifestations of autoimmune thyroid disease: the other autoimmune disease.  

PubMed

Autoimmune thyroid disease (AITD) is an inflammatory thyroiditis that in some cases is characterized by lymphocytic infiltration of the thyroid gland, also referred to as chronic lymphocytic thyroiditis or Hashimoto thyroiditis. Hashimoto thyroiditis is one of the commonest causes of hypothyroidism. Hypothyroidism has been associated with osteoarthritis (OA) and inflammatory forms of arthritis and with several well defined connective tissue diseases, which in turn can cause arthritis. The presence of arthritis in patients with AITD with normal thyroid function is now being increasingly recognized. There is also considerable evidence to suggest that AITD is highly associated with fibromyalgia syndrome. We review the current literature on the rheumatologic manifestations of AITD and describe the features in its presentation that set it apart from other forms of autoimmune arthritis. PMID:22505695

Tagoe, Clement E; Zezon, Anna; Khattri, Saakshi

2012-04-15

299

Oral manifestations of lupus erythematosus - report of two cases.  

PubMed

BACKGROUND: Lupus erythematosus is a serious autoimmune disease, which can cause oral mucosal lesions, manifesting as white striae and erythematous, atrophic and hyperkeratotic areas, as well as erosions and ulcerations. Sometimes, these signs can be misdiagnosed as other oral diseases such as lichen planus. Also, on histopathological examination with haematoxylin-eosin staining (HE), the features of these diseases can overlap. MATERIALS AND METHODS: The authors report here two cases of oral lesions of lupus erythematosus, with a discussion of their clinical features and the process of differential diagnosis. CONCLUSIONS: It is crucial to consider in the diagnostic process for lupus erythematosus the whole clinical information as well as haematological tests, histopathological features in HE and direct immunofluorescence, not only at first evaluation but also during patient follow-up. PMID:23489373

Simões, Débora M; Fava, Márcia; Figueiredo, Maria A; Salum, Fernanda G; Cherubini, Karen

2013-03-14

300

Supraclavicular Lymphadenopathy: Initial Manifestation of Metastasis in Carcinoma of Cervix  

PubMed Central

Introduction. Carcinoma of cervix rarely metastasizes to cervical lymph nodes and is associated with poor prognosis. To date, only few case reports have been reported in the medical literature. Here, we report a case of this unusual manifestation of carcinoma of cervix. Case Presentation. A 39-year-old Saudi woman who was treated three years ago for bulky IB stage carcinoma of cervix with total abdominal hysterectomy and bilateral salpingo-oophorectomy and adjuvant chemoradiation presented to us during her routine follow-up visit with left supraclavicular lymphadenopathy. Staging workup revealed additional para-aortic nodal and osseous metastases. The biopsy of left supraclavicular mass confirmed the diagnosis of carcinoma of the cervix. Patient was started on chemotherapy and bisphosphonates. Conclusion. Supraclavicular lymph nodes are a rare site of metastasis in carcinoma of cervix, and this can be explained by outlining the drainage of the lymphatic system from the cervix. Supraclavicular lymphadenopathy is associated with variable prognosis.

Tunio, Mutahir A.; Al Asiri, Mushabbab; Mohamed, Reham; Al-Dandan, Sadeq

2013-01-01

301

Personal concerns and manifest anxiety in black students.  

PubMed

The areas and magnitude of personal problems and their relationships with manifest anxiety were investigated in 154 male and 202 female black undergraduate students in a predominantly black state university in the South. Both males and females were highly concerned over problems in college work, finance, living conditions, and employment, social and recreational activities, and curriculum and teaching procedure. A significant sex difference at the .05 level was evidenced by the Mann-Whitney U test. Significant positive correlations were found between the number of problems and anxiety scores in 9 areas for males and in all 11 areas for females. Two problem areas that did not correlate significantly with anxiety scores for males were social and recreational activities and home and family. PMID:1249237

Wen, S S; Mccoy, R E

1976-01-01

302

JNK REGULATION OF HEPATIC MANIFESTATIONS OF THE METABOLIC SYNDROME  

PubMed Central

Nonalcoholic fatty liver disease (NAFLD) is now recognized as both an important component of the metabolic syndrome and the most prevalent liver disease in the United States. Although the mechanisms for development of steatosis and chronic liver injury in NAFLD remain unclear, recent investigations have indicated that overactivation of c-Jun N-terminal kinase (JNK) is critical to this process. These findings, together with evidence for the involvement of JNK signaling in other manifestations of the metabolic syndrome such as obesity and insulin resistance, have suggested that JNK may be a novel therapeutic target in this disorder. This review details findings that JNK mediates lipid accumulation and cell injury in fatty liver disease and discusses the possible cellular mechanisms of JNK actions.

Czaja, Mark J.

2010-01-01

303

Central nervous system manifestations of Q fever responsive to steroids.  

PubMed

We report the clinical and radiological central nervous system manifestations of a 27-year-old man with Q fever who subsequently developed acute disseminated encephalomyelitis and showed a significant response to steroids. The patient presented with headache and fever and quickly progressed to develop acute respiratory failure and hepatitis. A prompt evaluation revealed positive serology for Q fever and doxycycline was initiated. Approximately 1 week into his illness he was noted to be profoundly weak. Neuroimaging with magnetic resonance imaging (MRI) revealed diffuse white matter T2/FLAIR hyperintensities, with evidence of restricted diffusion. He was given high-dose steroids for a presumed diagnosis of acute disseminated encephalomyelitis (ADEM) and within days he had both clinical and MRI improvement. In addition to well-described meningitis and encephalitis, Q fever may also be associated with diffuse CNS lesions that may be demyelinating inflammatory in pathophysiology, and therefore responsive to high-dose steroids. PMID:19743743

Skiba, Virginia; Barner, Kristen C

2009-08-01

304

Unusual clinical manifestation of virus-associated hemophagocytic syndrome.  

PubMed

A 22-year-old man presented with bilateral painless cervical lymphadenomegaly, difficulties in nasal breathing and bilateral conductive hearing loss. Rhinoscopy and computer tomography disclosed mucosal polyps in the nasal cavity and a polypoid soft mass almost completely filling the whole nasal cavity and the paranasal sinuses. Thoracic and abdominal computer tomography showed mild hepatosplenomegaly and a solitary round lesion in the right lung. No fever or abnormal laboratory parameters were detected. The biopsy from the nasal polypoid lesions and from the cervical lymph nodes showed extensive proliferation of histologically benign erythrophagocytic histiocytes. The diagnosis of virus (Epstein-Barr virus)-associated hemophagocytic histiocytosis was confirmed by immunohistochemical reactions, by polymerase chain reaction, and by Epstein-Barr-Encodes (Early)-RNA in situ hybridization. This case illustrates an unusual clinical manifestation of virus-associated hemophagocytic histiocytosis presenting as mucosal polyps of the upper respiratory tract. PMID:14708643

Buza, Natália; Bálint, Ildikó; Schneider, Tamás; Koltai, László; Orosz, Zsolt

2003-01-01

305

[Renal aspergilloma: first manifestation of an occult sarcomatoid carcinoma].  

PubMed

A patient with b-symtoms and a sonographically detected kidney tumor entered hospital for a nephrectomy. An aspergillosis had been histologically accounted for. Up to that point, there was no indication of an immunodeficiency or invasive aspergillosis. Three months later, the patient was readmitted to hospital due to an indefinite retroperitoneal and hepatic mass on the right side. A punch biopsy ascertained a remanifestation of an aspergillosis at both localizations. The mass increased during systemic and antimycotic therapy, indicating the surgical resection of hepatic and retroperitoneal findings. The histological report yielded the diagnosis of a poorly differentiated sarcomatous carcinoma. Thus, an aspergillosis was the initial manifestation of what had up to that point been an occult sarcomatous carcinoma. PMID:22836941

Sobel, C; Franzaring, I; Pullwitt, J; Melchior, S

2012-10-01

306

Ophthalmic manifestations of tuberous sclerosis: a population based study  

PubMed Central

BACKGROUND/AIMS—Tuberous sclerosis complex (TSC) has retinal and non-retinal ophthalmic manifestations. This study was designed to determine the prevalence of the ophthalmic manifestations and of refractive errors in a population of patients with TSC.?METHODS—179 patients identified were in a prevalence study of TSC in the south of England and 107 of these agreed to full ophthalmic examination which was successful in 100. Ophthalmic examination included examination of the eyelids, cover test, examination of the irides, dilation funduscopy using both direct and indirect ophthalmoscopy, and refraction using retinoscopy. Myopia was defined as a spherical equivalent +0.5D.?RESULTS—Retinal hamartomas were seen in 44 of the 100 patients. The commonest morphological type of hamartoma seen was the flat, translucent lesion in 31 of the 44 patients (70%). The multinodular "mulberry" lesion was seen in 24 of the 44 patients (55%) and the transitional type lesion was seen in four of the 44 patients (9%). Punched out areas of retinal depigmentation were seen in 39 of the 100 patients but only six of 100 controls. 27% of eyes were myopic, 22% were hyperopic, and 27% had astigmatism >0.75D. Of the non-retinal findings, 39 patients had angiofibromas of the eyelids, five had non-paralytic strabismus, and three had colobomas.?CONCLUSION—Apart from the higher prevalence of flat retinal hamartomas, the findings of this study compare closely with previous large clinic based series of TSC patients. Refractive findings were similar to previous studies of a similarly aged non-TSC population. This is the first series to document the statistically significant association of punched out chorioretinal depigmentation with TSC and the authors believe that it should be looked for as an aid to diagnosis.??

Rowley, S; O'Callaghan, F; Osborne, J

2001-01-01

307

Gastrointestinal manifestations of food allergies in pediatric patients.  

PubMed

Foods that account for 90% of allergic reactions in children are cow's milk protein, eggs, peanut, soy, tree nuts, fish, and wheat. Food allergy can manifest as urticaria/angioedema, anaphylaxis, atopic dermatitis, respiratory symptoms, or a gastrointestinal (GI) disorder. GI allergic manifestations can be classified as immunoglobulin E (IgE) mediated (immediate GI hypersensitivity and oral allergy syndrome); "mixed" GI allergy syndromes (involving some IgE components and some non-IgE or T-cell-mediated components) include eosinophilic esophagitis and eosinophilic gastroenteritis. Non-IgE-mediated or T-cell-mediated allergic GI disorders include dietary protein enteropathy, protein-induced enterocolitis, and proctitis. All these conditions share a common denominator: the response of the immune system to a specific protein leading to pathologic inflammatory changes in the GI tract. This immunological response can elicit symptoms such as diarrhea, vomiting, dysphagia, constipation, or GI blood loss, symptoms consistent with a GI disorder. The detection of food allergies can be accomplished by the use of radioallergosorbent (RAST) testing and skin prick tests in helping to assess the IgE-mediated disorders. Patch tests may help evaluate delayed hypersensitivity reactions. Treatment of GI allergic disorders ranges from strict dietary elimination of offending food(s), use of protein hydrolysates, and use of L-amino acid-based formula when protein hydrolysates fail. Treatment with topical (for eosinophilic esophagitis) or systemic steroids is used if all dietary measures are unsuccessful. Maternal breast feeding or the use from birth of hydrolysate formulas (extensive or partial hydrolysates) may be efficacious in the prevention of atopic disease in "high-risk" families (with at least 1 parent or sibling with a history of atopic disease). PMID:16207693

Garcia-Careaga, Manuel; Kerner, John A

2005-10-01

308

Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations  

PubMed Central

Pseudoexfoliation syndrome (PXS) is a systemic condition with eye manifestations. In the eye, pseudoexfoliation material deposits on various structures of the anterior segment. The nature of this material is mostly fibrillar with fibers made up of microfibrils and coated with amorphous material. The composition of these fibrils is diverse and includes basement membrane components as well as enzymes involved in extracellular matrix maintenance. Pseudoexfoliation is the most common cause of secondary open-angle glaucoma (pseudoexfoliation glaucoma, PXG) worldwide. The goal of this review is to summarize our knowledge on the genetics of this systemic disorder and its resultant ocular manifestations. PXS familial aggregation suggests genetic inheritance. PXS has been strongly associated with single nucleotide polymorphisms (SNPs) of the lysyl oxidase-like 1 (LOXL1) gene on chromosome 15q24.1. Two of these SNPs confer a higher than 99% population attributable risk for PXS and PXG in the Nordic population; however, they carry different risks in different populations. The high risk haplotypes also vary among different populations. LOXL1 is one of group of the enzymes involved in the cross-linking of collagen and elastin in the extracellular matrix. Its function in connective tissue maintenance has been confirmed in mice; however, its actual role in PXS remains unclear. Contactin-associated protein-like 2 also has a strong genetic association with PXS in a German cohort and is an attractive candidate molecule. It encodes for a protein involved in potassium channel trafficking. Other candidate genes linked to PXS include lysosomal trafficking regulator, clusterin, adenosine receptors, matrix metalloproteinase-1 (MMP1), and glutathione transferase. These genes may be modifying genes for development of PXS and PXG.

2012-01-01

309

Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations.  

PubMed

Pseudoexfoliation syndrome (PXS) is a systemic condition with eye manifestations. In the eye, pseudoexfoliation material deposits on various structures of the anterior segment. The nature of this material is mostly fibrillar with fibers made up of microfibrils and coated with amorphous material. The composition of these fibrils is diverse and includes basement membrane components as well as enzymes involved in extracellular matrix maintenance. Pseudoexfoliation is the most common cause of secondary open-angle glaucoma (pseudoexfoliation glaucoma, PXG) worldwide. The goal of this review is to summarize our knowledge on the genetics of this systemic disorder and its resultant ocular manifestations. PXS familial aggregation suggests genetic inheritance. PXS has been strongly associated with single nucleotide polymorphisms (SNPs) of the lysyl oxidase-like 1 (LOXL1) gene on chromosome 15q24.1. Two of these SNPs confer a higher than 99% population attributable risk for PXS and PXG in the Nordic population; however, they carry different risks in different populations. The high risk haplotypes also vary among different populations. LOXL1 is one of group of the enzymes involved in the cross-linking of collagen and elastin in the extracellular matrix. Its function in connective tissue maintenance has been confirmed in mice; however, its actual role in PXS remains unclear. Contactin-associated protein-like 2 also has a strong genetic association with PXS in a German cohort and is an attractive candidate molecule. It encodes for a protein involved in potassium channel trafficking. Other candidate genes linked to PXS include lysosomal trafficking regulator, clusterin, adenosine receptors, matrix metalloproteinase-1 (MMP1), and glutathione transferase. These genes may be modifying genes for development of PXS and PXG. PMID:23157966

Elhawy, Eman; Kamthan, Gautam; Dong, Cecilia Q; Danias, John

2012-10-10

310

Trends in the Prevalence of Tuberous Sclerosis Complex Manifestations: An Epidemiological Study of 166 Japanese Patients  

PubMed Central

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with multi-system involvement and variable manifestations. There has been significant progress in TSC research and the development of technologies used to diagnose this disorder. As a result, individuals with mild TSC are now being diagnosed, including many older adults who have not developed seizures or cognitive abnormalities. We conducted a statistical analysis of the frequency of TSC manifestations in a population of Japanese adults and children, comparing our findings with historical data. The chi-square test was used to examine the frequency of each manifestation by age. A total of 166 outpatients at the Department of Dermatology of Osaka University Hospital during the period from January 2001 to March 2011 were included in the study. Compared to previous reports, the frequency of neurologic manifestations (excepting autism) was lower in this cohort, and the frequency of skin manifestations (excepting hypomelanotic macules) was higher in this cohort. The frequencies of pulmonary lymphangioleiomyomatosis and renal manifestations were not significantly different from those previously reported. Regarding the association of each manifestation with age, the frequency of neurologic manifestations (excepting subependymal giant cell astrocytoma) was significantly higher in younger patients than in older patients. The frequency of skin manifestations and renal angiomyolipoma were significantly higher in older patients than in younger patients. Because of their high frequency and visibility, skin manifestations are useful in the diagnosis of TSC. Moreover, uterine perivascular epithelioid cell tumor was also characterized as a new findings associated with TSC.

Wataya-Kaneda, Mari; Tanaka, Mari; Hamasaki, Toshimitsu; Katayama, Ichiro

2013-01-01

311

Pathogenesis of extrapulmonary manifestations of Mycoplasma pneumoniae infection with special reference to pneumonia  

Microsoft Academic Search

Although pneumonia has been a hallmark of Mycoplasma pneumoniae infection, it has been revealed that this infection can cause a number of extrapulmonary manifestations in the absence of\\u000a pneumonia. While the host immune response has been implicated in the pathomechanism of pneumonia, the pathomechanisms of extrapulmonary\\u000a manifestations remain largely unknown. It is proposed in this review that extrapulmonary manifestations due

Mitsuo Narita

2010-01-01

312

Osteoarticular Manifestations: Specific Treatments and/or Treating Intestinal Disease?  

PubMed

Osteoarticular manifestations in inflammatory bowel diseases (IBD) belong to the concept of spondyloarthritis (SpA) including an axial and peripheral SpA according to predominant symptoms (inflammatory back pain vs. peripheral arthritis and enthesopathy). Careful examination of sacroiliac joints on MRI plays a crucial role in the recognition of an early axial SpA in young patients with inflammatory back pain and spinal inflammation on MRI but without structural changes on radiography (non-rx SpA). In this early form of SpA, chronic gut inflammation was already found in about 30% of patients. Moreover, more pronounced bone marrow edema was found in patients with axial SpA and chronic gut inflammation. Identification of a therapeutic window in patients with early gut and spine inflammation is important since anti-TNF suppresses inflammation and seems to prevent evolution to structural changes. Shared genetic factors probably predispose to both diseases. Careful analysis of the effect of medication on gut and spine inflammation in SpA and IBD patients is recommended in order to find new therapeutic agents. © 2013 S. Karger AG, Basel. PMID:24030233

De Vos, M; Van Praet, L; Elewaut, D

2013-09-06

313

Thermogeodynamic Manifestations in the Caucasus and their Genesis  

NASA Astrophysics Data System (ADS)

In the work two aspects of thermal character are considered: first of all this is the connection of subduction phenomena with thermal life of the Caucasus on the basis of over interpreted data of magnetotelluric sounding, and secondly, origin of thermostressed condition of the Caucasus and its geological aspects which is manifested in the following: 1. in the zones of anomalous thermodisplacements thermofaults should occur (Le Pishon et al., 1977) These thermofaults are in good correlation with deep faults which are distinguished by geological and seismic methods, these thermofaults may be earthquake sources (Spitak, Racha, etc. earthquakes), also may be channels through which magma derivates (giving mineral deposits) may penetrate on surface (Gugunava and Gijeishvili, 1989); 2. in the body of sedimentary complex thermostressed seals and seal failures occur, which are apparently traps for oil-gas fluids. Good correlation of thermodense anomalies with oil deposits of the Caucasus is shown (Alexidze et al., 1985; Gugunava, 1980) Everything above mentioned was carried out within frames of stationary thermal model which did not allow us to reveal time characteristics of interconnection of geological medium and thermal field. Now investigations are being carried out within the frames of stationary thermal model and its interconnection with geological environment.

Botchorishvili, T. B.; Gugunava, G. E.; Kiria, J. K.

2009-04-01

314

Thermogeodynamic manifestations in the Caucasus and their genesis  

NASA Astrophysics Data System (ADS)

In the work two aspects of thermal character are considered: first of all this is the connection of subduction phenomena with thermal life of the Caucasus on the basis of over interpreted data of magnetotelluric sounding, and secondly, origin of thermostressed condition of the Caucasus and its geological aspects which is manifested in the following: 1. in the zones of anomalous thermodisplacements thermofaults should occur (Le Pishon et al., 1977). These thermofaults are in good correlation with deep faults which are distinguished by geological and seismic methods, these thermofaults may be earthquake sources (Spitak, Racha, etc. earthquakes), also may be channels through which magma derivates (giving mineral deposits) may penetrate on surface (Gugunava and Gijeishvili, 1989); 2. in the body of sedimentary complex thermostressed seals and seal failures occur, which are apparently traps for oil-gas fluids. Good correlation of thermodense anomalies with oil deposits of the Caucasus is shown (Alexidze et al., 1985; Gugunava, 1980). Everything above mentioned was carried out within frames of stationary thermal model which did not allow us to reveal time characteristics of interconnection of geological medium and thermal field. Now investigations are being carried out within the frames of stationary thermal model and its interconnection with geological environment.

Gugunava, G. E.; Kiria, J. K.; Bochorishvili, T. B.

2009-09-01

315

Manifestation of finite temperature size effects in nanogranular magnetic graphite  

NASA Astrophysics Data System (ADS)

In addition to the double phase transition (with the Curie temperatures TC=300 K and TCt=144 K), a low-temperature anomaly in the dependence of the magnetization is observed in the bulk magnetic graphite (MG) (with an average granular size of L~=10 nm), which is attributed to the manifestation of the size effects below the quantum temperature TL~?2/L2 and is well fitted by the periodic function ML(T)~sin[M(T)?(T)/L] with M(T) being the bulk magnetization and ?(T)~?/T the thermal de Broglie wavelength. The best fits of the high-temperature data (using the mean-field Curie-Weiss and Bloch expressions) produced reasonable estimates for the model parameters, such as defects mediated effective spin exchange energy J~=12 meV (which defines the intragranular Curie temperature TC) and proximity mediated interactions between neighboring grains (through potential barriers U created by thin layers of nonMG) with energy Jt=exp(-d/?)J~=5.8 meV (which defines the intergranular Curie temperature TCt) with d~=1.5 nm and ?~?/U~=2 nm being the intergranular distance and characteristic length, respectively.

Sergeenkov, S.; Souza, N. S.; Speglich, C.; Rivera, V. A. G.; Cardoso, C. A.; Pardo, H.; Mombrú, A. W.; Araújo-Moreira, F. M.

2009-12-01

316

A Unique Manifestation of Pupillary Fatigue in Autoimmune Autonomic Ganglionopathy  

PubMed Central

Objective To demonstrate a unique abnormality of the pupillary light reflex in patients with Autoimmune Autonomic Ganglionopathy (AAG). Design Case series Setting Autonomic clinics at two university hospitals (University of Texas Southwestern Medical Center and Beth Israel Deaconess Medical Center) Participants Seven patients with antibody positive AAG. Intervention All patients with AAG underwent either monocular or binocular infrared pupillometry using a standard 2 second light stimulus at a defined intensity. Findings were compared to healthy controls and patients with other autonomic disorders. The light stimulus used in this study was selected to eliminate the normal phenomenon of pupil escape. Main Outcome measure The time to onset of redilation was the main outcome measure. Other indices of pupillary constriction to light stimulus were also measured. Results Patients with AAG exhibited premature pupillary redilation (1.02 ±0.20 seconds) compared to healthy control subjects (2.24±0.10 seconds) and other patients with autonomic disorders (2.3±0.12 seconds; P<0.0001). In healthy control subjects and patients with other autonomic disorders pupillary redilation always followed the termination of the light stimulus while in AAG patients redilation consistently occurred during the light stimulus. In one patient, serial repetitive light stimulation further decreased the time to onset of redilation. Conclusion Premature redilation of the pupil is a unique physiological feature seen only in patients with AAG. This phenomenon appears to be a manifestation of pupillary fatigue, a clinical correlate of defective synaptic transmission at the level of autonomic ganglia in antibody positive AAG.

Muppidi, Srikanth; Scribner, Maggie; Gibbons, Christopher H.; Adams-Huet, Beverley; Spaeth, Elaine B.; Vernino, Steven

2012-01-01

317

Distinct manifestations of executive dysfunction in aged rats.  

PubMed

Different components of executive function such as working memory, attention, and cognitive flexibility can be dissociated behaviorally and mechanistically; however, the within-subject influences of normal aging on different aspects of executive function remain ill-defined. To better define these relationships, young adult and aged male F344 rats were cross-characterized on an attentional set-shifting task that assesses cognitive flexibility and a delayed response task that assesses working memory. Across tasks, aged rats were impaired relative to young; however, there was significant variability in individual performance within the aged cohort. Notably, performance on the set-shifting task and performance at long delays on the delayed response task were inversely related among aged rats. Additional experiments showed no relationship between aged rats' performance on the set-shifting task and performance on a hippocampal-dependent spatial reference memory task. These data indicate that normal aging can produce distinct manifestations of executive dysfunction, and support the need to better understand the unique mechanisms contributing to different forms of prefrontal cortical-supported executive decline across the lifespan. PMID:23601673

Beas, B Sofia; Setlow, Barry; Bizon, Jennifer L

2013-04-17

318

Ocular manifestations in children and adolescents with Lyme arthritis  

PubMed Central

BACKGROUND—Lyme arthritis is the most frequent late manifestation of Lyme borreliosis and has been associated with ocular inflammation.?METHODS—A group of 153 children and adolescents with arthritis, 84 of whom had Lyme arthritis and 69 other causes of arthritis, were followed prospectively for 22-73 (median 44) months in the course of a national study.?RESULTS—Three of 84 patients with Lyme arthritis had ocular inflammation (4%), including keratitis, anterior uveitis, and uveitis intermedia. All three had symptoms of decreased visual acuity. Whereas anterior uveitis disappeared without sequelae, a corneal scar and a permanent loss of visual acuity in the patients with keratitis and intermediate uveitis remained. Systematic examination of all patients revealed no further ocular involvement. Of 69 patients with other causes of arthritis who were followed in parallel as a control group, four of 15 patients with early onset pauciarticular juvenile rheumatoid arthritis had chronic anterior uveitis and two of 12 patients with juvenile spondyloarthropathy had acute anterior uveitis.?CONCLUSIONS—Ocular involvement with keratitis, anterior uveitis, and intermediate uveitis may occur in children and adolescents with Lyme arthritis. Visual loss appears to be symptomatic, making regular ocular screening of such patients unnecessary.??

Huppertz, H.; Munchmeier, D.; Lieb, W.

1999-01-01

319

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

320

Chronic candidal meningitis: an uncommon manifestation of candidiasis.  

PubMed

Chronic meningitis is an uncommon manifestation of candidiasis. We present the case of an elderly woman who had symptoms such as headache, malaise, and fever for 8 months and was found to have Candida albicans meningitis, and we review 17 similar cases. An underlying illness or risk factor for candidiasis was present in only 13 (72%) of the 18 patients. Headache, fever, and nuchal rigidity were the predominant clinical findings. Analysis of CSF showed either mononuclear or neutrophilic pleocytosis, an elevated protein level, and a decreased level of glucose. Only 17% of CSF smears were positive, and only 44% of initial CSF cultures yielded Candida species. In four cases, Candida species grew only after special techniques were used; in three cases, CSF cultures remained negative. The overall mortality associated with candidal meningitis was 53%, but among 12 patients who were treated and followed, the rate was 33%. In addition to acute meningitis seen with disseminated infection, Candida species can cause chronic meningitis that mimics tuberculosis and the more common fungal meningitides, such as cryptococcosis. PMID:7948559

Voice, R A; Bradley, S F; Sangeorzan, J A; Kauffman, C A

1994-07-01

321

[Necrotizing vasculitis as a manifestation of hypersensitivity to propylthiouracil].  

PubMed

Propylthiouracil (PTU), a thyonamide class drug commonly used to treat hyperthyroidism has been reported to cause adverse reactions in 3% to 12% of patients. The side effects have been described more frequently as mild, but ocasionally severe fatal reactions may occur. We report the case of a fourteen years old patient in use of PTU for the last three years who presented with fever, hemorrhagic blisters, necrotic ulcers, and that developed purpuric lesions and nodules in lower extremities. Laboratory and histopathologic findings were compatible with skin leukocytoclastic vasculitis, a pattern found in hypersensitivity reaction vasculitis. Suspension of PTU and introduction of prednisone, induced complete remission of symptoms and healing of the skin lesions. The importance of this study is to call attention to the occurrence of serious cutaneous manifestation with a mortality rate that might reach 10%, associated with a systemic drug frequently used in internal medicine. Early diagnosis and withdrawal of the suspected medication is mandatory. Administration of corticosteroids and/or immunosuppressives agents must be considered. PMID:19578601

Dinato, Sandra Lopes Mattos e; Dinato, Mauro Cesar; Dinato, Marcelo Mattos e; Ribeiro, Marcela Castro M; Yamashiro, Cintia Yukimi; Romiti, Ney

2009-04-01

322

Head and neck manifestations of myeloma in Nigerians.  

PubMed

A study on the clinicopathologic features of myeloma as it manifests in the head and neck region was conducted over a 15-year period. A total of six patients were seen and they constituted 20% of all myeloma cases. The mean age was 41.5 years, three were males and three were females. Clinical presentation was varied and included swelling, epistaxis and gingival bleeding. The duration of symptom ranged from 3 months to 6 years. Definitive diagnosis was extramedullary myelomatosis in three patients, multiple myeloma in two patients while one patient had solitary plasmacytoma of bone. Chemotherapy alone was the treatment modality in two patients, one had surgery combined with chemotherapy and one patient had only supportive therapy. Two patients did not receive treatment, as they requested for discharge against medical advice. One patient followed up for a period of 1 year recovered with residual neurological deficits. This study confirms the rarity of myeloma in the head and neck region and where it occurs; it is most likely multiple myeloma or extramedullary myelomatosis. In this part of the world, mortality rate is aggravated by late presentation and inability to afford chemotherapy. PMID:15979285

Owotade, F; Ugboko, V; Ajike, S; Salawu, L; Amusa, Y; Omole, M

2005-10-01

323

Aseptic spondylitis as the initial manifestation of the SAPHO syndrome.  

PubMed

We describe the case of a 61-year-old female patient who presented with spondylitis of the lumbar spine. Although the microbiological cultures of the bone biopsy specimens obtained during laminotomy remained negative, the patient was treated with broad-spectrum antimicrobials for 2 months. Eight months later she started to suffer from pain and tenderness in her sternum and the medial portion of her left clavicle. The findings of computed tomography and gallium-labelled isotope scan were indicative of sternoclavicular arthritis. Again, all surgically obtained biopsy specimens yielded negative results in microbiological studies. The diagnosis of the SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteomyelitis) syndrome was then made based on the clinical presentation with recurrent sterile osteitis in two characteristic locations, and the patient was started on immunosuppressive therapy. This case is a reminder that SAPHO may sometimes occur without any skin manifestations. Since this type of patient may be admitted to an orthopedic ward, it is important that orthopedic surgeons are familiar with the syndrome. PMID:9391803

Kotilainen, P; Gullichsen, R E; Saario, R; Manner, I; Kotilainen, E

1997-01-01

324

The manifestation of oxygen contamination in ErD2.  

SciTech Connect

Erbium dihydride Er(H,D,T){sub 2} is a fluorite structure rare-earth dihydride useful for the storage of hydrogen isotopes in the solid state. However, thermodynamic predictions indicate that erbium oxide formation will proceed readily during processing, which may detrimentally contaminate Er(H,D,T){sub 2} films. In this work, transmission electron microscopy (TEM) techniques including energy-dispersive x-ray spectroscopy, energy-filtered TEM, selected area electron diffraction, and high-resolution TEM are used to examine the manifestation of oxygen contamination in ErD{sub 2} thin films. An oxide layer {approx}30-130 nm thick was found on top of the underlying ErD{sub 2} film, and showed a cube-on-cube epitaxial orientation to the underlying ErD{sub 2}. Electron diffraction confirmed the oxide layer to be Er{sub 2}O{sub 3}. While the majority of the film was observed to have the expected fluorite structure for ErD{sub 2}, secondary diffraction spots suggested the possibility of either nanoscale oxide inclusions or hydrogen ordering. In situ heating experiments combined with electron diffraction ruled out the possibility of hydrogen ordering, so epitaxial oxide nanoinclusions within the ErD{sub 2} matrix are hypothesized. TEM techniques were applied to examine this oxide nanoinclusion hypothesis.

Snow, Clark Sheldon; Parish, Chad Michael; Brewer, Luke N.

2008-10-01

325

Ophthalmic manifestation of skull base metastasis from breast cancer  

PubMed Central

Summary Background There is a vast discrepancy between the incidence of skull base metastases reported in vivo and at autopsy. Asymptomatic character or unspecific symptoms make the diagnosis difficult, particularly in patients with no history of cancer. Our case illustrates a skull base metastasis from breast cancer, detected in a diagnostic process initiated by ophthalmologic examination. Case Report We report the case of a 53-year-old woman complaining of ptosis and diplopia, with concomitant loss of skin sensation within the right half of the forehead, and without any other worrisome symptoms or signs. Ophthalmic examination revealed impairment in eye movements, slight proptosis and corneal hypoesthesia on the right side, with normal pupillary light reflexes. The anterior and posterior segments of the eye were normal. Based on CT and MRI, an extensive tumor was detected, infiltrating the right orbit and the frontotemporal region of the skull base, and producing edema of the adjacent aspects of the brain. Aside from partial palsy of the oculomotor nerve and the ophthalmic division of the trigeminal nerve, no abnormalities were found on neurological examination. Explorative craniotomy and histopathological findings revealed a skull base metastasis from breast cancer. Conclusions Diplopia, ptosis, proptosis, and ophthalmic nerve sensory loss may be the only manifestation of a skull base metastasis. Careful ophthalmologic examination is crucial in early detection of this life-threatening condition.

Dmuchowska, Diana Anna; Krasnicki, Pawel; Obuchowska, Iwona; Kochanowicz, Jan; Syta-Krzyzanowska, Anna; Mariak, Zofia

2012-01-01

326

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

327

Ear nose throat manifestations in hypoidrotic ectodermal dysplasia.  

PubMed

The ectodermal dysplasias (EDs) are a large and complex group of inherited disorders. In various combinations, they all share anomalies in ectodermal derived structures: hair, teeth, nails and sweat gland function. Clinical overlap is present among EDs. Few causative genes have been identified, to date. Altered gene expression is not limited to the ectoderm but a concomitant effect on developing mesenchymal structures, with modification of ectodermal-mesenchymal signaling, takes place. The two major categories of ED include the hidrotic and hypohidrotic form, the latter more frequent; they differentiate each other for the presence or absence of sweat glands. We report Ear Nose Throat manifestations of ED, linked to the reduction of mucous glands in the nasal fossae with reduced ciliar function, and decrease salivary glands function. Often patients report an increased rate of infections of the upper respiratory tract and of the ear. Nasal obstruction due to the presence of nasal crusting, hearing loss and throat hoarseness are the most represented symptoms. Environmental measures, including a correct air temperature and humidification, is mandatory above all in subjects affected by hypohidrotic form. PMID:24080322

Callea, Michele; Teggi, Roberto; Yavuz, Izzet; Tadini, Gianluca; Priolo, Manuela; Crovella, Sergio; Clarich, Gabriella; Grasso, Domenico Leonardo

2013-09-13

328

Clinical manifestations and treatment of Menkes disease and its variants.  

PubMed

The clinical manifestations of classical Menkes disease, mild Menkes disease and occipital horn syndrome are reviewed. Menkes disease is a neurodegenerative disease with X-linked recessive inheritance. Orally administered copper accumulates in the intestine, resulting in the failure of copper absorption. The primary metabolic defect that causes copper accumulation in the intestine is present in almost all extrahepatic tissues. The blood, liver and brain are in a state of copper deficiency, which is due to defective copper absorption. The characteristic features, including neurological disturbances, arterial degeneration and hair abnormalities, can be explained by the decrease in cuproenzyme activities. DNA-based diagnosis is now possible. Mild Menkes disease and occipital horn syndrome, which show milder forms than Menkes disease, have been identified as genetic disorders resulting from mutations in the Menkes disease gene. Because the clinical spectrum of Menkes disease is wide, males with mental retardation and connective tissue abnormalities should be evaluated for biochemical evidence of defective copper transport. The treatment accepted currently is parenteral administration of copper. When treatment is started in patients with classical Menkes disease above the age of 2 months, it does not improve the neurological degeneration. When the treatment is initiated in newborn babies affected with this disease, the neurological degeneration can be prevented in some, but not all, cases. Moreover, early treatment cannot improve non-neurological problems, such as connective tissue laxity. Therefore, alternative therapies for Menkes disease and occipital horn syndrome should be studied. PMID:10453199

Kodama, H; Murata, Y; Kobayashi, M

1999-08-01

329

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

2012-05-18

330

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

331

Rash decisions-dermatological manifestations preceding a progressive neurological syndrome.  

PubMed

We describe the case of a 72 year old woman who presented with sequential cranial nerve palsies preceded by irritation, pain and a vesicular rash in the sensory distributions of the affected nerves. She had signs and symptoms suggestive of aphagia and Ramsey-Hunt syndrome. Further vesicular eruptions roughly obeying dermatomal boundaries on the limbs and trunk were observed. She was initially thought to have disseminated VZV/HZV infection and was commenced on IV aciclovir. Investigations seeking evidence for viral particles in skin lesions and CSF, and for evidence of immunosuppression or malignancy, were all negative. Between days 35 and 40 she developed hypertension, seizures, a left facial nerve palsy and encephalopathy. This was complicated by a pneumonia requiring invasive ventilation on intensive care. She developed an itchy maculopapular rash with vesiculation of all four limbs. On day 66 she developed truncal and limb rigidity, which became extreme over two weeks, and was associated with pyramidal weakness. The rash was biopsied demonstrating a leucocytoclastic vasculitis. At the nadir of her illness she was encephalopathic, tracheostomy-and NG feeding-dependent, quadriparetic and bedbound due to severe rigidity (leading rapidly to Achilles tendon contractures). She was found to be glycine receptor (GlyR) antibody positive, without antibodies against amphiphysin or GAD65. She was initially treated with a combination of corticosteroids and intravenous immunoglobulin (IVIG), resulting in resolution of her cranial nerve palsies, and a reduction in her rigidity and rash. Imaging using CT, PET, USS and mammography was undertaken to exclude malignancy-associated antibody production. MRI imaging of the brain and spinal cord was essentially normal. CSF analysis demonstrated only the presence of intrathecal IgG synthesis. Her treatment and rehabilitation continues. This case adds to the small number of reports in the literature of a Stiff Person Syndrome (SPS) plus syndrome considered to be caused by antibodies directed against the extracellular GlyR target on the synaptic neuronal surface.(1 2) Our case demonstrates further clinical variability within this unusual disorder. The GlyR is expressed in skin, and the presence of zoster-like dermatological lesions may provide a clinical clue as to the underlying aetiology in similar cases. Many of the features seen in this case have been previously described, however in this case the features emerged sequentially with rigidity, the cardinal feature of this group of diseases, emerging last. The clinical manifestations of the cases of GlyR antibody associated SPS plus disease described, closely resemble the clinical manifestations of genetic mutations of GlyR subunits and associated proteins, and of poisoning with the GlyR antagonist strychnine. This adds to the evidence that these antibodies play a direct role in the pathogenesis of disease, and that the use of immunosuppressive treatment is justified. Indeed our patient has made a substantial recovery with aggressive immunotherapy. To date no associated malignancy has been associated with GlyR antibody-mediated SPS plus disease, and our case supports these findings. PMID:24109022

Doherty, Gary; Vincent, Angela; Cox, Amanda

2013-11-01

332

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

333

Manifestly gauge-invariant general relativistic perturbation theory: I. Foundations  

NASA Astrophysics Data System (ADS)

Linear cosmological perturbation theory is pivotal to a theoretical understanding of current cosmological experimental data provided e.g. by cosmic microwave anisotropy probes. A key issue in that theory is to extract the gauge-invariant degrees of freedom which allow unambiguous comparison between theory and experiment. When one goes beyond first (linear) order, the task of writing the Einstein equations expanded to nth order in terms of quantities that are gauge-invariant up to terms of higher orders becomes highly non-trivial and cumbersome. This fact has prevented progress for instance on the issue of the stability of linear perturbation theory and is a subject of current debate in the literature. In this series of papers we circumvent these difficulties by passing to a manifestly gauge-invariant framework. In other words, we only perturb gauge-invariant, i.e. measurable quantities, rather than gauge variant ones. Thus, gauge invariance is preserved non-perturbatively while we construct the perturbation theory for the equations of motion for the gauge-invariant observables to all orders. In this first paper we develop the general framework which is based on a seminal paper due to Brown and Kucha? as well as the relational formalism due to Rovelli. In the second, companion, paper we apply our general theory to FRW cosmologies and derive the deviations from the standard treatment in linear order. As it turns out, these deviations are negligible in the late universe, thus our theory is in agreement with the standard treatment. However, the real strength of our formalism is that it admits a straightforward and unambiguous, gauge-invariant generalization to higher orders. This will also allow us to settle the stability issue in a future publication.

Giesel, K.; Hofmann, S.; Thiemann, T.; Winkler, O.

2010-03-01

334

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

335

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

336

Relationship between clinical features of Crohn's disease and the risk of developing extraintestinal manifestations  

Microsoft Academic Search

Objectives Crohn's disease is frequently associated with extraintestinal manifestations. The aim of this study was to evaluate the degree of association between the development of extraintestinal manifestations, the clinical forms of Crohn's disease according to the Vienna Classification and to the presence of several potential risk factors of the disease. Methods One hundred and seventy-three consecutive Crohn's disease patients were

Manuel Barreiro-de Acosta; J. Enrique Dominguez-Munoz; M. Concepcion; Antonio Lozano-Leon

337

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

338

Nominal and Real Group Performance in Relation to Manifest Anxiety and Induced Stress  

ERIC Educational Resources Information Center

Female college students were selected on the basis of their scores on the Manifest Anxiety Scale. The subjects worked either alone or in pairs. The experiment had a two x two x two design, with group type (nominal versus real), manifest anxiety (low versus high), and induces stress (low versus high) as the three variables. (Author)

Kanekar, Suresh; And Others

1975-01-01

339

Convergent and Divergent Validity of the Revised Children's Manifest Anxiety Scale  

Microsoft Academic Search

A modified multitrait multimethod validation matrix was generated to evaluate the construct validity of the newly revised Children's Manifest Anxiety Scale (RCMAS). For both males (N = 49) and females (N = 37) a pattern of results occurred that was generally consistent with the use of the RCMAS as a measure of chronic, manifest anxiety levels in children. Large correlations

Cecil R. Reynolds

1982-01-01

340

Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet's disease  

Microsoft Academic Search

The most serious central nervous system (CNS) manifestation in Behcet's disease is a slowly progressive dementia (progressive NB), which may ultimately lead to the deterioration of the personality of patients. An open trial was designed to investigate the efficacy of low dose weekly methotrexate (MTX) therapy for progressive NB. Six patients with Behcet's disease, whose neuropsychiatric manifestations were judged to

Shunsei Hirohata; Hiroko Suda; Takashi Hashimoto

1998-01-01

341

Sensorimotor Stroke due to Gangliocapsular Hematoma as First Manifestation of Chronic Myelocytic Leukemia  

Microsoft Academic Search

Hemorrhagic complications are occasionally seen in patients with chronic myelocytic leukemia (CML). Cerebral hemorrhage is a severe and rare complication, which is exceptionally observed as its presenting manifestation [1]. We describe the case of a patient with cerebral hemorrhage as the initial presentation of CML manifested as a sensorimotor syndrome, which is described as one of the classical ‘lacunar’ syndromes

Adrià Arboix; Montse Oliveres; Carles Besses

2000-01-01

342

Clinical manifestations and molecular epidemiology of Vibrio vulnificus infections in Denmark  

Microsoft Academic Search

The clinical manifestations of and epidemiological data from 11 patients infected withVibrio vulnificus admitted to Danish hospitals during the unusually warm summer of 1994 are reported. All patients contracted the disease after exposure to seawater; however, none had consumed seafood. Four patients developed bacteremia, one of whom subsequently died; nine patients, including the four with bacteremia, exhibited skin manifestations. Four

A. Dalsgaard; N. Frimodt-Møller; B. Bruun; L. Høi; J. L. Larsen

1996-01-01

343

[Surgical treatment of cardiovascular manifestations of Marfan syndrome].  

PubMed

The present study determines the effect of surgical treatment of cardiovascular manifestations of Marfan syndrome in 72 patients by 114 operations, during 34-year period. This therapy resulted in aortic root repair, aortic arch replacement, or both in 78, mitral valve repair in 9, descending thoracic aortic replacement in 14, thoracoabdominal aortic replacement in 10, and abdominal aortic replacement in 6, including total aortic replacement in 4 and nearly total aortic replacement in 4 patients. Fusiform aneurysms were present in the the ascending aorta in 37, the aortic arch in 2, the thoracoabdominal aorta in 2, and the abdominal aorta in 6 patients. Aortic dissection occurred in 40 (55.6%), including type A aortic dissection in 29 patients. Aortic root repair included separate valve-graft in 8, Bentall composite valve-graft in 25, composite valve-graft with button technique in 26, composite valve-graft with interposition graft technique in 10, and valve sparing procedure in 5 patients. The overall early (30-day) mortality was 7.9%. The early survival was 75% in separate valve-graft procedure and 99.2% in composite valve-graft procedure. Late coronary dehiscence did not occur in the patients with Bentall technique in which the reattachments of coronary ostia were performed in 2 layers, but occurred in 50% of patients with the coronary anastomoses in 1 layer. Aortic valve regurgitation relapsed in 2 of the 5 patients with valve sparing procedure. Event free rate for the patients with composite valve-graft using button technique was 81.1% at 10 years. There were 14 late deaths; dissection or rupture of the residual aorta, composite graft endocarditis and cardiac failure were the principle causes of late deaths. In conclusion, Marfan patients with cardiovascular diseases can undergo surgical treatment with a low operative risk and low morbidity. Although late endocarditis remains a serious problem, we believe that Marfan syndrome is a contraindication for valve sparing procedure. Because of the potential for late dissection or aneurysm in other areas of the aorta, patients with Marfan syndrome should have serial computed tomographic scans indefinitely. PMID:12174652

Fukada, J; Morishita, K; Kawaharada, N; Yamada, A; Baba, T; Harada, N; Abe, T

2002-07-01

344

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

2007-01-25

345

Sleep apnea is a manifestation of the metabolic syndrome.  

PubMed

Obstructive sleep apnea (OSA) is a prevalent disorder particularly among middle-aged, obese men, although its existence in women as well as in lean individuals is increasingly recognized. Despite the early recognition of the strong association between OSA and obesity, and OSA and cardiovascular problems, sleep apnea has been treated as a 'local abnormality' of the respiratory track rather than as a 'systemic illness.' In 1997, we first reported that the pro-inflammatory cytokines interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNFalpha) were elevated in patients with disorders of excessive daytime sleepiness (EDS) and proposed that these cytokines were mediators of daytime sleepiness. Also, we reported a positive correlation between IL-6 or TNFalpha plasma levels and the body-mass-index (BMI). In subsequent studies, we showed that IL-6, TNFalpha, and insulin levels were elevated in sleep apnea independently of obesity and that visceral fat, was the primary parameter linked with sleep apnea. Furthermore, our findings that women with the polycystic ovary syndrome (PCOS) (a condition associated with hyperandrogenism and insulin resistance) were much more likely than controls to have sleep disordered breathing (SDB) and daytime sleepiness, suggests a pathogenetic role of insulin resistance in OSA. Other findings that support the view that sleep apnea and sleepiness in obese patients may be manifestations of the Metabolic Syndrome, include: obesity without sleep apnea is associated with daytime sleepiness; PCOS and diabetes type 2 are independently associated with EDS after controlling for SDB, obesity, and age; increased prevalence of sleep apnea in post-menopausal women, with hormonal replacement therapy associated with a significantly reduced risk for OSA; lack of effect of continuous positive airway pressure (CPAP) in obese patients with apnea on hypercytokinemia and insulin resistance indices; and that the prevalence of the metabolic syndrome in the US population from the Third National Health and Nutrition Examination Survey (1988-1994) parallels the prevalence of symptomatic sleep apnea in general random samples. Finally, the beneficial effect of a cytokine antagonist on EDS in obese, male apneics and that of exercise on SDB in a general random sample, supports the hypothesis that cytokines and insulin resistance are mediators of EDS and sleep apnea in humans. In conclusion, accumulating evidence provides support to our model of the bi-directional, feed forward, pernicious association between sleep apnea, sleepiness, inflammation, and insulin resistance, all promoting atherosclerosis and cardiovascular disease. PMID:15893251

Vgontzas, Alexandros N; Bixler, Edward O; Chrousos, George P

2005-06-01

346

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

347

Oral manifestations of coeliac disease.: A clinical-statistic study.  

PubMed

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. PMID:23285376

Costacurta, M; Maturo, P; Bartolino, M; Docimo, R

2010-11-19

348

Association of anti-cardiolipin antibodies with vascular thrombosis and neurological manifestation of Behçets disease.  

PubMed

We have studied 44 patients with Behçet's Disease (BD) to look for any correlation of arterial and venous thrombosis or central nervous system (CNS) manifestations with anti-cardiolipin antibodies (ACLA). Twenty patients were positive for ACLA by MELISA method. Ten patients had IgG antibody, four had IgM and six had both IgG and IgM. Of these patients, 11 had a history of vascular thrombosis and thrombophlebitis and nine had CNS manifestations. The association of ACLA with vascular thrombosis or CNS manifestation of Behçet's disease was statistically not significant. PMID:8467608

al-Dalaan, A N; al-Ballaa, S R; al-Janadi, M A; Bohlega, S; Bahabri, S

1993-03-01

349

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

350

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

351

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

352

19 CFR 122.73 - General declaration and air cargo manifest.  

Code of Federal Regulations, 2013 CFR

...BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE...declaration with Customs at the departure airport. (3) Exception. A general declaration...manifest with the Customs at the departure airport. Three copies of the air cargo...

2013-04-01

353

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

2012-02-23

354

[Spinal cord compression as initial manifestation of Hodgkin's disease. Report of a case].  

PubMed

A case of Hodgkin's disease in which the first clinical manifestation was a radiculo-spinal compression is reported. The authors comment about the possible mechanisms to explain this radiculo-spinal compression in this granulomatosis. PMID:7436801

De Freitas, M R; Nascimento, O J; Cincinatus, D; Praxedes, H; Hahn, M D

1980-06-01

355

Pulmonary Hemorrhage Syndrome as a Manifestation of Disseminated Intravascular Coagulation: Analysis of 10 Cases.  

National Technical Information Service (NTIS)

Pulmonary hemorrhage occurred in seven patients with disseminated intravascular coagulation (DIC) and was produced in three monkeys when an experimental model for DIC was used. The principal manifestation of the syndrome was either the chief complaint for...

H. Lopas J. W. Minna N. I. Birndorf R. W. Colman S. J. Robboy

1972-01-01

356

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

Code of Federal Regulations, 2013 CFR

...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 (Soviet Zone of Germany and...

2013-04-01

357

Hypokalemic periodic paralysis; two different genes responsible for similar clinical manifestations  

PubMed Central

Primary hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder manifesting as recurrent periodic flaccid paralysis and concomitant hypokalemia. HOKPP is divided into type 1 and type 2 based on the causative gene. Although 2 different ion channels have been identified as the molecular genetic cause of HOKPP, the clinical manifestations between the 2 groups are similar. We report the cases of 2 patients with HOKPP who both presented with typical clinical manifestations, but with mutations in 2 different genes (CACNA1Sp.Arg528His and SCN4A p.Arg672His). Despite the similar clinical manifestations, there were differences in the response to acetazolamide treatment between certain genotypes of SCN4A mutations and CACNA1S mutations. We identified p.Arg672His in the SCN4A gene of patient 2 immediately after the first attack through a molecular genetic testing strategy. Molecular genetic diagnosis is important for genetic counseling and selecting preventive treatment.

Kim, Hunmin; Hwang, Hee; Cheong, Hae Il

2011-01-01

358

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

Code of Federal Regulations, 2013 CFR

...manifest; information required; alternative forms. 4.7a Section...information required; alternative forms. The forms designated...L Nr.â (ii) As an alternative to the procedure described...a particular container, identifying the port of lading,...

2013-04-01

359

15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.  

Code of Federal Regulations, 2012 CFR

...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

2012-01-01

360

15 CFR 30.47 - Clearance or departure of carriers under bond on incomplete manifest.  

Code of Federal Regulations, 2013 CFR

...Customs and Border Protection regulations citations, exclusion, and/or exemption legends...in this part and all required filing citations, exclusion, and/or exemption legends...the manifest and all required filing citations, exclusion, and/or exemption...

2013-01-01

361

Massive bloody pericardial effusion as an initial manifestation of chronic kidney disease  

PubMed Central

We describe a 35-year-old man with a massive bloody pericardial effusion, which was his initial manifestation of chronic kidney disease. Pericardiocentesis and hemodialysis restored cardiac function and relieved the associated massive anasarca.

Fazel, Poorya; Vallabhan, Ravi C.; Roberts, William C.

2013-01-01

362

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

363

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

364

Clinical manifestations and progression of IgM mesangial nephropathy: a single center prospective  

Microsoft Academic Search

Out of 732 renal biopsies performed from 1988 to 1995 in Queen Elizabeth Hospital, 65 patients (8.9%, 43 male, 22 female) were diagnosed to have IgM mesangial nephropathy (IgMN). The mean age of the patients was 35 ±2 years. The clinical manifestations and progression of IgMN were studied in 39 of these 65 patients. The initial manifestations of the disease

Yiu-Han CHAN; Kim-Ming WONG; Koon-Shing CHOI; Wai-Leung CHAK; Chi-Yuen CHEUNG; Ka-Foon CHAU; Chun-Sang LI

2000-01-01

365

Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti  

Microsoft Academic Search

The shrinking lung syndrome (SLS) is a rare manifestation in patients with established systemic lupus erythematosus (SLE).\\u000a Only two cases have been reported in which this syndrome was the presenting manifestation of SLE. We describe a 21-year-old\\u000a female Kuwaiti who presented with SLS. In addition to clinical and serological features of lupus, she had dyspnea, respiratory\\u000a muscle dysfunction, characteristic chest

Haneen Adel Al-Raqum; Sukhbir Singh Uppal; Moudi Al-Mutairy; Rakesh Kumari

2006-01-01

366

Gravity as the Second-Order Relativistic-Manifestation of Electrostatic-Force  

Microsoft Academic Search

It is well known that magnetic force between two current carrying conductors is a relativistic manifestation of net electrostatic force between relatively moving electrons & protons of the two wires. On similar grounds but with more accurate considerations i.e., of second order relativistic considerations, it is shown here that gravitational-force between two bodies is in-fact due to relativistic manifestation of

R. C. Gupta

2005-01-01

367

Gravity as the Second-Order Relativistic-Manifestation of Electrostatic-Force  

Microsoft Academic Search

It is well known that magnetic force between two current carrying conductors\\u000ais a relativistic manifestation of net electrostatic force between relatively\\u000amoving electrons & protons of the two wires. On similar grounds but with more\\u000aaccurate considerations i.e., of second order relativistic considerations, it\\u000ais shown here that gravitational-force between two bodies is in-fact due to\\u000arelativistic manifestation of

R. C. Gupta

2005-01-01

368

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

369

The head, neck, and systemic manifestations of levamisole-adulterated cocaine use.  

PubMed

Systemic complications of levamisole-adulterated cocaine (LAC) use have recently been described. The objective of this review is to increase awareness of these manifestations among oral and maxillofacial surgeons. LAC exposure through inhalation, nasal insufflation, or injection can induce cutaneous vasculopathy and hematologic abnormalities such as neutropenia or agranulocytosis. Unlike other vasculopathies involving the skin, LAC-induced vascular injury frequently manifests with purpuric and necrotic lesions that involve the face and ears. Oral manifestations have also been reported but are not yet well characterized. The aforementioned hematologic manifestations are not uncommon, and patients exposed to LAC are potentially at higher risk for infectious complications. When manifestations of LAC affect the head, neck, and oral cavity, oral and maxillofacial surgeons may be the first providers to encounter the patient. Early recognition of the clinical signs and laboratory abnormalities will better allow for distinguishing LAC-related effects from various clinical mimics, will facilitate appropriate patient management, and may further contribute to the understanding of the biological effects of LAC. PMID:23298805

Magliocca, Kelly R; Coker, Neysa Alice; Parker, Sareeta R

2013-01-05

370

Allelic Imbalance in TOR1A mRNA Expression in Manifesting and Non-Manifesting Carriers of the GAG-Deletion  

PubMed Central

Early onset dystonia (EOD) is associated with a 3bp-(?GAG) in-frame deletion in the TOR1A gene, which encodes for torsinA. Carriers of the mutant (?GAG) allele can either develop or escape a dystonic phenotype (~30% penetrance). The expression ratio of the two alleles could be important for the manifestation or prevention of the disease since wild-type (WT) torsinA is thought to have protective function. Absence of an antibody discriminating WT from ?E torsinA has precluded the determination ?E and WT torsinA levels in manifesting and nonmanifesting carriers. We performed quantitative analysis of TOR1A allele expression in manifesting (MC) and nonmanifesting (NMC) carriers using quantitative allele-specific PCR (qASPCR) to determine the levels of mutant versus WT torsinA mRNA. The technique described showed high degree of specificity in detecting the two alleles. The present study represents the first comprehensive analysis of biallelic expression of the TOR1A gene in lymphoblast and brain samples from patients and NMC relatives. We demonstrate that mRNA is transcribed from both the WT and ?GAG allele in peripheral and neural tissues with a trend for increased expression of the ?GAG allele compared to the WT in carriers regardless of their phenotype and thus cannot account for the reduced penetrance.

Armata, Ioanna A.; Diplas, Andreas I.; Ozelius, Laurie J.; Shashidharan, Pullanipally

2012-01-01

371

Oral and periodontal manifestations associated with systemic sclerosis: A case series and review  

PubMed Central

Systemic sclerosis is a rare connective tissue disorder with a wide range of oral manifestations. This case series reports significant oral and periodontal changes and also makes an attempt to correlate oral and systemic findings in these patients which enable the clinician for a better diagnosis and evolve a comprehensive treatment plan. Six patients with a known diagnosis of systemic sclerosis were included. After obtaining the patient's informed consent, relevant medical history, oral manifestations including periodontal findings and oral hygiene index simplified index were recorded. In these patients, oral changes included restricted mouth opening and, resorption of the mandible. The periodontal changes observed were gingival recession, absence or minimal gingival bleeding on probing, and widened periodontal ligament space, radiographically. Patients with systemic sclerosis often show wide range of oral manifestations, which is of major concern for the dentist.

Jagadish, Rekha; Mehta, Dhoom Singh; Jagadish, P.

2012-01-01

372

Oral and periodontal manifestations associated with systemic sclerosis: A case series and review.  

PubMed

Systemic sclerosis is a rare connective tissue disorder with a wide range of oral manifestations. This case series reports significant oral and periodontal changes and also makes an attempt to correlate oral and systemic findings in these patients which enable the clinician for a better diagnosis and evolve a comprehensive treatment plan. Six patients with a known diagnosis of systemic sclerosis were included. After obtaining the patient's informed consent, relevant medical history, oral manifestations including periodontal findings and oral hygiene index simplified index were recorded. In these patients, oral changes included restricted mouth opening and, resorption of the mandible. The periodontal changes observed were gingival recession, absence or minimal gingival bleeding on probing, and widened periodontal ligament space, radiographically. Patients with systemic sclerosis often show wide range of oral manifestations, which is of major concern for the dentist. PMID:23055598

Jagadish, Rekha; Mehta, Dhoom Singh; Jagadish, P

2012-04-01

373

Oral and systemic manifestations of mucopolysaccharidosis type VI: a report of seven cases.  

PubMed

Mucopolysaccharidosis (MPS) is a group of rare metabolic diseases characterized by intralysosomal accumulation of glycosaminoglycans. MPS type VI or Maroteaux-Lamy syndrome is an autosomal-recessive syndrome caused by mutations in the lysosomal enzyme arylsulfatase B. A defect in the gene leads to accumulation of nondegraded mucopolysaccharides, resulting in severe cellular dysfunction with multisystem expression. The oral manifestations of MPS VI are not well described in the literature. This paper presents a series of seven patients with MPS VI, with the description of the general clinical manifestations and focus on the still rarely studied oral manifestations of the syndrome. Among them were high palate, open bite, impacted and/or included teeth, thickening of the pericoronal follicle, and changes in the temporomandibular joint. PMID:22299127

de Almeida-Barros, Renata Quirino; Oka, Salomão Cury-Rad; Pordeus, Ana Carolina Barbosa; de Medeiros, Paula Frassinetti Vasconcelos; Bento, Patricia Meira; Godoy, Gustavo Pina

2012-03-01

374

Posterior reversible encephalopathy syndrome: an acute manifestation of systemic lupus erythematous.  

PubMed

Stroke mimickers are common, and they represent a diagnostic dilemma for clinicians. Many, like posterior reversible encephalopathy syndrome (PRES), are easily reversible. The manifestation of PRES is characterised by headaches, convulsions, altered mental functioning and blindness. In most cases, computed tomography of the brain will show hypodense lesions in the parieto-occpitial lobe, which only further confounds the physician. Although this syndrome is uncommon, prompt and accurate recognition allows early treatment, which has been shown to produce favourable outcomes. Herein, we report the case of a 54-year-old woman, who presented with PRES, as an acute manifestation of systemic lupus erythematous (SLE) and lupus nephritis. The patient was initially thought to be experiencing an ischaemic stroke, but the diagnosis was later changed. On management of her underlying condition, her symptoms resolved. PRES should be recognised as an acute emergency manifestation of SLE. It should not be mistaken for an ischaemic stroke as inappropriate treatment could have adverse outcomes. PMID:24068069

Chan, M J; Ong, Y S

2013-09-01

375

Skin manifestations of nutritional deficiency disease in children: modern day contexts.  

PubMed

Nutritional deficiency syndromes, such as scurvy, pellagra, and beriberi are of historical significance but have largely disappeared from modern society. However, certain populations of children in modern society are at risk of severe nutritional complications. The rarity of these syndromes and lack of understanding about modern-day risk factors for nutritional deficiency often delays diagnosis. Dermatologists must maintain an appropriate index of suspicion for these characteristic syndromes as many of the deficiency states present with cutaneous manifestations. Here we review the cutaneous manifestations of macronutrient and micronutrient deficiency syndromes as well as those populations of children that remain at risk for developing severe disease. PMID:23171006

Lee, Lara Wine; Yan, Albert C

2012-12-01

376

[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

377

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

378

Adult Onset Still's Disease: A Case Report with a Rare Clinical Manifestation and Pathophysiological Correlations  

PubMed Central

Adult-onset Still's disease is an inflammatory multisystemic disease of unknown etiology. Pleuritis is the most common pulmonary manifestation and pleural effusions are usually exudates with a predominance of neutrophils. We report a case of an eosinophilic pleural effusion as a novel and hitherto unrecognized manifestation of active adult-onset Still's disease. We also observed a marked NLRP3 inflammasome activation with increased production of IL-1? which coincided with the development and resolved upon remission of the pleural effusion suggesting a possible novel pathogenetic pathway for the development of pleural effusions in the context of the auto-inflammatory disorders.

Antoniou, Katerina M.; Margaritopoulos, George A.; Giannarakis, Ioannis; Choulaki, Christianna; Fountoulakis, Nikos; Siafakas, Nikos M.; Sidiropoulos, Prodromos

2013-01-01

379

Kocher debre semelaigne syndrome: a rare case report with orofacial manifestations.  

PubMed

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. PMID:23599883

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

2013-03-01

380

Depression is an Early Disease Manifestation in Lupus-Prone MRL/lpr Mice  

PubMed Central

Many lupus patients develop neuropsychiatric manifestations, including cognitive dysfunction, depression, and anxiety. However, it is not clear if neuropsychiatric lupus is a primary disease manifestation, or is secondary to non-CNS disease. We found that MRL/lpr lupus-prone mice exhibited significant depression-like behavior already at 8 weeks of age, despite normal visual working memory, locomotor coordination and social preference. Moreover, depression was significantly correlated with titers of autoantibodies against DNA, NMDA receptors and cardiolipin. Our results indicate that lupus mice develop depression and CNS dysfunction very early in the course of disease, in the absence of substantial pathology involving other target organs.

Gao, Hua-Xin; Campbell, Sean R.; Cui, Min-Hui; Zong, Pu; Hwang, Jong hee; Gulinello, Maria; Putterman, Chaim

2009-01-01

381

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

382

Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery.  

PubMed

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis. PMID:22281907

Mann, Danielle; Presotto, Carolina; Queen, Stella Maria Facó; Oliveira, Elisa Fontenelle de; Gripp, Alexandre Carlos

383

Mononeuritis multiplex and painful ulcers as the initial manifestation of hepatitis B infection.  

PubMed

Hepatitis B virus infection leads to multisystem manifestations owing to involvement of kidney, skin, vasculature, haematopoietic and nervous system. The hepatitis B infection can cause neuropathy either to vasculitis associated with polyarteritis nodosa or immune-mediated neural damage. In this submission, we report a young woman, who presented with mononeuritis multiplex and painful ulcerations as the first manifestation of chronic hepatitis B virus infection. The antiviral therapy along with steroids led to remarkable recovery. The clinical settings of hepatitis B virus infection should not be ignored in the presentation of mononeuritis multiplex with ulcers, although the commonest cause is leprosy in the Indian sub-continent. PMID:23645658

Verma, Rajesh; Lalla, Rakesh; Babu, Suresh

2013-05-02

384

Recurrent vasodilator-refractory acute coronary syndrome as the exclusive manifestation of Graves disease.  

PubMed

Whether recurrent acute coronary syndrome could be the exclusive manifestation of Graves disease remains unreported. We describe a premenopausal woman who had angiographically normal coronary arteries yet had 3 episodes of acute coronary events in forms of unstable angina, ST elevation, and non-ST elevation myocardial infarction despite the active therapy of calcium-channel blockade. She was finally diagnosed as with Graves disease, treated with antithyroid medication, and free from any angina relapse for up to 18 months. Thus, recurrent coronary events might be the only manifestation of subclinical hyperthyroidism in patients with angiographically normal coronary arteries and could only be prevented by antithyroid agents instead of conventional vasodilators. PMID:22030192

Lee, Chi-Pin; Lee, Wen-Lieng; Lai, Hui-Chin; Ting, Chih-Tai; Wang, Kuo-Yang; Liu, Tsun-Jui

2011-10-24

385

Wolff-Parkinson-White Syndrome as Initial Manifestation of Becker Muscular Dystrophy  

Microsoft Academic Search

Background:  Cardiac involvement may precede the onset of muscular manifestations in Becker muscular dystrophy (BMD), but Wolff-Parkinson-White\\u000a (WPW) syndrome has not been reported as initial cardiac manifestation of BMD.\\u000a \\u000a \\u000a \\u000a Case Study:  In a 43-year-old, HIV-negative male, WPW syndrome was diagnosed at age 26 years upon a routine surface ECG, carried out for\\u000a recurrent palpitations since childhood. Since then, WPW syndrome was occasionally

Josef Finsterer; Claudia Stöllberger; Stefan Quasthoff

2008-01-01

386

Hypodontia and Delayed Dentition as the Primary Manifestation of Cleidocranial Dysplasia Presenting with a Diagnostic Dilemma  

PubMed Central

Cleidocranial dysplasia is a rare autosomal disorder which manifests as partial or complete absence of clavicles, multiple supernumerary teeth, and delayed closure of fontanelle. Classical cases of cleidocranial dysplasia are easily diagnosed very early in the life. However, cases with partial manifestation of the syndrome and noncontributory family history are difficult to diagnose. Here, we report a case of 8.5-year-old girl child who presented with delayed tooth development (without any supernumerary teeth), anterior open fontanelle, and normal clavicles, thus resulting in a diagnostic dilemma.

Chopra, Radhika; Marwaha, Mohita; Chaudhuri, Payal; Bansal, Kalpana; Chopra, Saurabh

2012-01-01

387

[A manifestation suggesting juvenile spondylarthropathy: anterior tibial enthesitis. Apropos of 3 cases].  

PubMed

The tibial tuberosity may be affected by enthesitis in seronegative spondylarthropathies, especially in childhood. Three personal cases illustrate this possibility. Data from the literature seem to confirm that enthesitis is more common in juvenile forms (where it may occur as the first manifestation) than in adult forms. Enthesitis is manifested mainly by clinical symptoms. Roentgenographic changes are inconsistent and variable; ossification may develop. Osgood Schlatter disease is the main differential diagnosis. Persistent enthesitis of the tibial tuberosity in a young boy should suggest spondylarthropathy. PMID:2029120

Wendling, D; Bertrand, A M

1991-02-01

388

Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature  

PubMed Central

About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents.

Palejwala, Neal V.; Walia, Harpreet S.; Yeh, Steven

2012-01-01

389

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

390

Neurological manifestations of gastrointestinal disorders, with particular reference to the differential diagnosis of multiple sclerosis  

Microsoft Academic Search

. Neurological manifestations of gastrointestinal disorders are described, with particular reference to those resembling multiple sclerosis (MS) on clinical or MRI grounds. Patients with celiac disease can present cerebellar ataxia, progressive myoclonic ataxia, myelopathy, or cerebral, brainstem and peripheral nerve involvement. Antigliadin antibodies can be found in subjects with neurological dysfunction of unknown cause, particularly in sporadic cerebellar ataxia (\\

A. Ghezzi; M. Zaffaroni

2001-01-01

391

Classification of epileptic motor manifestations and detection of tonic-clonic seizures with acceleration norm entropy.  

PubMed

In this paper, three triaxis accelerometers positioned on the wrists and the head of epileptic patients submitted to long-term video electroencephalographic monitoring as part of presurgical investigation are evaluated to characterize the different classes of motor manifestations observed during seizures. Quadratic discriminant classifiers are trained on features extracted from 1 or 4 s windows. It is shown that a simple rule applied to the acceleration norm entropy HnA produces the best performances compared to other classifiers trained on other feature sets. The simple rule is as follows with values given in bits: (0 HnA 1.34), no movement; (1.34 HnA 3.87), tonic manifestations; (3.87 HnA), tonic-clonic manifestations. For this classifier, features are extracted from 1 s windows and the misclassification rate is 11% evaluated on 5 607 s of epileptic motor manifestations obtained from 58 seizures in 30 patients. A quantile normalization can improve the results with features based on absolute power spectral density but performances are not as good as the ones obtained with HnA. Based on the classifier using only HnA, a simple tonic-clonic seizure detector is proposed and produces a 80% sensitivity with a 95% specificity. PMID:23392333

Becq, Guillaume; Kahane, Philippe; Minotti, Lorella; Bonnet, Stephane; Guillemaud, Regis

2013-02-04

392

Psychiatric Manifestations in Young Females with Congenital Adrenal Hyperplasia in Taiwan  

Microsoft Academic Search

Background: Congenital adrenal hyperplasia (CAH) is a syndrome of prenatal and\\/or post- natal androgen excess secondary to genetic deficits in the cytochrome p450 enzymes of the cortisol synthesis pathway. Women with CAH may suffer from different degrees of androgenization. This study documented psychi- atric manifestations in young women born with CAH in Taiwan. Methods: From July 1, 2005 to February

Hsin-Yi Liang; Hsueh-Ling Chang; Ching-Yen Chen; Pei-Yeh Chang; Fu-Sung Lo; Li-Wei Lee

393

Change in the Manifestation of Overt Aggression during Early Adolescence: Gender and Ethnicity  

ERIC Educational Resources Information Center

This study examined how the manifestation of overt aggression changes during early adolescence using Hierarchical Linear Modeling. The distinct courses of physical and verbal aggression identified in this study provide support for developmental transformations in overt aggression, which would have been obscured unless aggression had been defined…

Kim, Sangwon; Kamphaus, Randy W.; Orpinas, Pamela; Kelder, Steve H.

2010-01-01

394

Marketing Concept Manifestations in Fiji Enterprises: Confirming the Link to Organizational Competitiveness  

Microsoft Academic Search

This paper proposes an integrative view of the marketing concept (i.e., the ability to understand and satisfy customers) and examines its prevalence and effect on competitiveness in organizations operating in an isolated and less economically developed country. The marketing concept manifests as marketing expertise, market orientation, and externally directed organizational values. Based on a sample of 86 firms operating in

Stern Neill; Raghuvar Dutt Pathak; Narendra Reddy

2009-01-01

395

Attitudes and InactionA Case Study of the Manifest Demographics of Urban Water Conservation  

Microsoft Academic Search

The public’s disposition with respect to conservation is predominantly ascertained by surveys. Rarely are these attitudes contrasted directly against manifested behavior from an independent source. This article analyzes the response to a residential urban water conservation program by using municipal water consumption data at the census tract level in San Antonio, Texas. A selection of demographic variables (i.e., income, education,

Miguel De Oliver

1999-01-01

396

Restless legs syndrome and nocturnal myoclonus: initial clinical manifestation of familial amyloid polyneuropathy  

Microsoft Academic Search

Restless legs syndrome was the first isolated clinical manifestation in four siblings of a family with familial amyloid polyneuropathy. Clinical and electrophysiological evidence of peripheral neuropathy appeared after a variable time interval. Polysomnography showed abnormal sleep patterns and nocturnal myoclonus in all patients. The restless legs syndrome responded favourably to clonazepam.

F Salvi; P Montagna; R Plasmati; G Rubboli; F Cirignotta; M Veilleux; E Lugaresi; C A Tassinari

1990-01-01

397

Violence and Compassion: A Bioethical Insight into Their Cognitive Bases and Social Manifestations  

ERIC Educational Resources Information Center

This article considers the social problem of violence and the alternative of resolution through cooperation and compassion from the perspective of cognitive neuroscience. Violence is a social problem, the manifestations of which have a biological basis reflected in the development of aggression and the neural mechanisms that regulate it.…

Mercadillo, Roberto E.; Arias, Nallely A.

2010-01-01

398

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

399

Metabolic syndrome and incidence of type 2 diabetes in patients with manifest vascular disease  

Microsoft Academic Search

R isk reduction in patients with clinically manifest vas- cular 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 vas- cular disease have an elevated risk of type 2 diabetes. The

ANNEMARIE MJ WASSINK; YOLANDA VAN DER GRAAF; SABITA S SOEDAMAH-MUTHU; WILKO SPIERING; LJ VISSEREN

2010-01-01

400

Change in the Manifestation of Overt Aggression during Early Adolescence: Gender and Ethnicity  

ERIC Educational Resources Information Center

|This study examined how the manifestation of overt aggression changes during early adolescence using Hierarchical Linear Modeling. The distinct courses of physical and verbal aggression identified in this study provide support for developmental transformations in overt aggression, which would have been obscured unless aggression had been defined…

Kim, Sangwon; Kamphaus, Randy W.; Orpinas, Pamela; Kelder, Steve H.

2010-01-01

401

Disturbed post-movement beta synchronization in Wilson's disease with neurological manifestation  

Microsoft Academic Search

We analyzed the changes of post-movement beta synchronization (PMBS) of the electroencephalogram (EEG) in Wilson's disease with neurological manifestation. Our aim was to determine if PMBS in Wilson's disease is altered in a different way than in Parkinson's disease or in essential tremor. Our purpose was to find out whether the analysis of PMBS could help the diagnosis in ambiguous

Gertrúd Tamás; Jan Raethjen; Muthuraman Muthuraman; Anikó Folhoffer; Günther Deuschl; Ferenc Szalay; Annamária Takáts; Anita Kamondi

2011-01-01

402

White Matter Changes: Neurobehavioral Manifestations of Binswanger's Disease and Clinical Correlates in Alzheimer's Disease  

Microsoft Academic Search

Although white matter lesions (WMLs) are among the most common structural neuroimaging changes found on computed tomography and magnetic resonance imaging of older persons with dementia, their presence should not be misconstrued as proof that vascular disease is causing or contributing to the dementia. We report the results of several studies examining the neurobehavioral manifestations of persons meeting explicit operational

David A. Bennett; David W. Gilley; Sarha Lee; Elizabeth J. Cochran

1994-01-01

403

Does heredity determine the allergy manifestation or the sensitisation to a specific allergen?  

Microsoft Academic Search

BackgroundThe role of genetics in allergy development is well accepted. However, studies could not delineate the mode of inheritance or what is specifically being inherited. The purpose of this study was to determine the effect of genetics on the development of allergy manifestation, serum IgE level, and sensitization to specific allergens.

Y. Yilmaz-Demirdag; B. Prather; S. L. Bahna

2010-01-01

404

[Non-ischemic neurological manifestations in patients with primary antiphospholipid syndrome].  

PubMed

Ischemic disorders of brain blood circulation caused by brain artery thrombosis due to antiphospholipids-induced anticoagulopathy are main neurological appearances of primary antiphospholipid syndrome (PAPS). A number of neurological disorders in patients with PAPS are the result of primary involvement of the brain and peripheral nervous system. We analyzed the spectrum of neurological non-ischemic PAPS manifestations in 125 patients (102 female, 23 male, mean age--37.5 +/- 11.3 years) with definite PAPS. These manifestations included headache (67%), epileptic seizures (23%), chorea (15%), optic neuropathy (9%), peripheral neuropathy (6%), multiple sclerosis like syndrome (MSLS) (8%), acute psychosis (2%), myasthenic syndrome (1%), non-vascular parkinsonism (1%). In the development of non-ischemic PAPS manifestations, antiphospholipids as well as other antibodies produced as a result of immune disregulation (antibodies to acetylcholine receptors in myasthenic syndrome, antineuronal antibodies in MSLS) may have pathogenic significance. In some cases a role of infection involved in PAPS manifestation cannot be ruled out. PMID:15792137

Kalashnikova, L A

2005-01-01

405

Identifying On-the-Job Behavioral Manifestations of Drug Abuse: A Guide for Work Supervisors.  

ERIC Educational Resources Information Center

|While extensive materials exist regarding types of drugs and motivations of users, the job supervisor, trainer and teacher still lacks information on how to recognize the behavioral manifestations of drug abuse in an educational and/or work setting. The manual, through detailed vignettes and questions addressed to the reader, deals with various…

Reinish, Harold

406

Manifest Destiny and Competing Voices on the Eve of the Cherokee Removal  

ERIC Educational Resources Information Center

|Manifest Destiny, the idea that Providence guided the conquest and settlement of North America, is one of the most contested ideas in American culture and history. One's opinion about this central aspect of American mythology depends heavily on one's point of view. Exploring westward expansion and the Cherokee Trail of Tears with primary sources…

Chandler, Prentice T.

2011-01-01

407

Extrapulmonary manifestations of severe respiratory syncytial virus infection - a systematic review  

PubMed Central

Introduction Respiratory syncytial virus (RSV) bronchiolitis is the most important cause for admission to the paediatric intensive care unit in infants with lower respiratory tract infection. In recent years the importance of extrapulmonary manifestations of RSV infection has become evident. This systematic review aimed at summarizing the available evidence on manifestations of RSV infection outside the respiratory tract, their causes and the changes in clinical management required. Methods Databases searched were Medline (1950 to present), EMBASE (1974 to present), PubMed and reference lists of relevant articles. Summarized were the findings of articles reporting on manifestations of RSV infection outside the respiratory tract in patients of all age groups. Results Extrapulmonary manifestations reported in previous observational studies included cardiovascular failure with hypotension and inotrope requirements associated with myocardial damage as evident from elevated cardiac troponin levels (35–54% of ventilated infants), cardiac arrhythmias like supraventricular tachycardias and ventricular tachycardias, central apnoeas (16–21% of admissions), focal and generalized seizures, focal neurological abnormalities, hyponatraemia (33%) associated with increased antidiuretic hormone secretion, and hepatitis (46–49% of ventilated infants). RSV or its genetic material have been isolated from cerebrospinal fluid, myocardium, liver and peripheral blood. Conclusion The data summarized indicate a systemic dissemination of RSV during severe disease. Cerebral and myocardial involvement may explain the association of RSV with some cases of sudden infant death. In infants with severe RSV infection cardiac rhythm, blood pressure and serum sodium need to be monitored and supportive treatment including fluid management adjusted accordingly.

Eisenhut, Michael

2006-01-01

408

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

409

Halitosis Manifestation and Prevention Means for Patients with Fixed Teeth Dentures  

Microsoft Academic Search

SUMMARY The objective of this research is to analyse the causal relationship between construction of fixed bridge dentures and the intensity of halitosis manifestations, as well as to establish basic hygiene requirements for construction of fixed dentures which would completely exclude retention of food parti- cles and avoid bad breath. 48 patients (36 men and 12 women), who use fixed

Guntis Zigurs; Aldis Vidzis; Anda Brinkmane

410

Manifestations of Greek-Cypriot Teachers' Discomfort toward a Peace Education Initiative: Engaging with Discomfort Pedagogically  

ERIC Educational Resources Information Center

This study sought to understand how teachers' discomforting emotions were manifest in a teacher education setting and how teacher educators might engage with discomfort pedagogically. A qualitative perspective was used with a group of teachers who participated in a series of peace education workshops in Cyprus. All of the workshops were audio- and…

Zembylas, Michalinos; Charalambous, Panayiota; Charalambous, Constadina

2012-01-01

411

THE EARS OF THE HIPPOPOTAMUS: MANIFESTATIONS, DETERMINANTS, AND ESTIMATES OF THE MALARIA BURDEN  

Microsoft Academic Search

Malarious patients experience asymptomatic parasitemia; acute febrile illness (with cerebral damage, anemia, respiratory distress, hypoglycemia); chronic debilitation (anemia, malnutrition, nervous system-related se- quelae); and complications of pregnancy (anemia, low birth weight, increased infant mortality). These manifestations in patients, communities, and countries reflect intrinsic (human, parasite, mosquito) and extrinsic (environmental, social, behavioral, political, and economic conditions as well as disease-control efforts)

JOEL G. BREMAN

2001-01-01

412

The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations  

Microsoft Academic Search

4,4'-Diaminodiphenylsulphone (Dapsone) is widely used for a variety of infectious, immune and hypersensitivity disorders, with indications ranging from Hansen's disease, inflammatory disease and insect bites, all of which may be seen as manifestations in certain occupational diseases. However, the use of dapsone may be associated with a plethora of adverse effects, some of which may involve the pulmonary parenchyma. Methemoglobinemia

Semaan G Kosseifi; Bhuvana Guha; Dima N Nassour; David S Chi; Guha Krishnaswamy

2006-01-01

413

Intrapersonal and Interpersonal Manifestations of Antilesbian and Gay Prejudice: An Application of Personal Construct Theory  

ERIC Educational Resources Information Center

|This study extended research on prejudice against lesbian and gay (LG) persons by examining theoretically grounded links between intrapersonal and interpersonal manifestations of such prejudice. On the basis of G. A. Kelly's (1955/1991a, 1955/1991b) conceptualization of threat, the authors operationalized intrapersonal homophobia, or LG threat,…

Moradi, Bonnie; van den Berg, Jacob J.; Epting, Franz R.

2006-01-01

414

Seborrheic Area Erythema as a Common Skin Manifestation in Japanese Patients with Dermatomyositis  

Microsoft Academic Search

Background: Although dermatomyositis (DM)-associated facial erythema was noted in the nasolabial folds of Japanese patients, DM-associated facial erythema other than heliotrope rash has drawn little attention in previous studies. Objectives: To characterize phenotypical features and frequencies of erythema, especially those in the seborrheic area of the head, in DM patients. Methods: A retrospective study on skin manifestations in 33 DM

N. Okiyama; H. Kohsaka; N. Ueda; T. Satoh; I. Katayama; K. Nishioka; H. Yokozeki

2008-01-01

415

ERP Manifestations of Processing Printed Words at Different Psycholinguistic Levels: Time Course and Scalp Distribution  

Microsoft Academic Search

The aim of the present study was to examine the time course and scalp distribution of electrophysiological manifestations of the visual word recognition mechanism. Event-related potentials (ERPs) elicited by visually presented lists of words were recorded while subjects were involved in a series of oddball tasks. The distinction between the designated target and nontarget stimuli was manipulated to induce a

S. Bentin; Y. Mouchetant-Rostaing; M. H. Giard; J. F. Echallier; J. Pernier

1999-01-01

416

Skin manifestations of systemic autoimmune connective tissue disease: diagnostics and therapeutics.  

PubMed

Skin disease can significantly affect the quality of life of patients suffering from rheumatic diseases. In addition, important relationships exist between the cutaneous and systemic manifestations of rheumatic disease. It is thus important for practicing rheumatologists to have a solid working understanding of the subject of rheumatic skin disease. Unfortunately, it is not possible within the scope of this chapter to provide a comprehensive overview of the recognition and management of all of the cutaneous manifestations of lupus erythematosus (LE), much less those of dermatomyositis (DM)/polymyositis and scleroderma/systemic sclerosis (SSc) as well. As can be seen in in the text, the cutaneous manifestations of polygenic autoimmune disorders such as LE can be as heterogeneous clinically as are its systemic manifestations. This discussion will therefore focus on recent key developments concerning the diagnosis and management of the more common skin changes that the practicing rheumatologist is likely to encountered in the three major rheumatic diseases: LE, DM and SSc. PMID:15158749

Sontheimer, Richard D

2004-06-01

417

Skin manifestations of systemic autoimmune connective tissue disease: diagnostics and therapeutics  

Microsoft Academic Search

Skin disease can significantly affect the quality of life of patients suffering from rheumatic diseases. In addition, important relationships exist between the cutaneous and systemic manifestations of rheumatic disease. It is thus important for practicing rheumatologists to have a solid working understanding of the subject of rheumatic skin disease. Unfortunately, it is not possible within the scope of this chapter

Richard D Sontheimer

2004-01-01

418

Hypokalemic periodic paralysis; two different genes responsible for similar clinical manifestations.  

PubMed

Primary hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder manifesting as recurrent periodic flaccid paralysis and concomitant hypokalemia. HOKPP is divided into type 1 and type 2 based on the causative gene. Although 2 different ion channels have been identified as the molecular genetic cause of HOKPP, the clinical manifestations between the 2 groups are similar. We report the cases of 2 patients with HOKPP who both presented with typical clinical manifestations, but with mutations in 2 different genes (CACNA1Sp.Arg528His and SCN4A p.Arg672His). Despite the similar clinical manifestations, there were differences in the response to acetazolamide treatment between certain genotypes of SCN4A mutations and CACNA1S mutations. We identified p.Arg672His in the SCN4A gene of patient 2 immediately after the first attack through a molecular genetic testing strategy. Molecular genetic diagnosis is important for genetic counseling and selecting preventive treatment. PMID:22253645

Kim, Hunmin; Hwang, Hee; Cheong, Hae Il; Park, Hye Won

2011-11-30

419

Ocular manifestations of some canine infectious and parasitic diseases commonly encountered in the Mediterranean  

Microsoft Academic Search

Many transmissible diseases, endemic in the Mediterranean area have been spread to countries where they have never been diagnosed before, because of the increasing international trade and travel activities. The purpose of this review is to describe the ocular manifestations of these infectious and parasitic diseases, which are also common in Greece, along with some insights into their etiopathogenesis, differential

A. Komnenou; A. F. Koutinas

420

Clinically isolated left coronary ostial stenosis: a manifestation of cardiovascular syphilis--case history.  

PubMed

The authors report a case of clinically isolated left coronary ostial stenosis in a thirty-seven-year-old man as a manifestation of cardiovascular syphilis. Notably he was free of the usual risk factors for coronary artery disease, and the rest of the coronary tree was angiographically normal. PMID:8239065

Shah, D C; Subramanyan, K

1993-11-01

421

An Investigation of the Manifestation of Sexism in EFL/ESL Textbooks  

ERIC Educational Resources Information Center

The present study, under the sponsorship of Islamic Azad University of Bandar Abbas, Iran, was designed to examine the manifestation of sexism in three EFL/ESL textbook series ("American Headway", "Interchange (3rd Ed.)", and "Person to Person") currently used in Iranian Language Institutes. A critical content analysis was conducted over the text…

Ghorbani, Laya

2009-01-01

422

Violence and Compassion: A Bioethical Insight into Their Cognitive Bases and Social Manifestations  

ERIC Educational Resources Information Center

|This article considers the social problem of violence and the alternative of resolution through cooperation and compassion from the perspective of cognitive neuroscience. Violence is a social problem, the manifestations of which have a biological basis reflected in the development of aggression and the neural mechanisms that regulate it.…

Mercadillo, Roberto E.; Arias, Nallely A.

2010-01-01

423

"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

424

Chronic pain is a manifestation of the Ehlers-Danlos syndrome  

Microsoft Academic Search

The Ehlers-Danlos syndrome (EDS) is a group of heritable systemic disorders of connective tissue manifesting joint hypermobility, skin extensibility, and tissue fragility. Although the presence of pain has been documented in the various types of the EDS, its natural history, distribution, and management have not been defined. We conducted a structured interview in 51 individuals affected with different types of

Anubha Sacheti; Judy Szemere; Bruce Bernstein; Triantafyllos Tafas; Neil Schechter; Petros Tsipouras

1997-01-01

425

Diagnostic possibilities of thermal imaging in neurologic manifestations of lumbar osteochondrosis in different stages of treatment  

NASA Astrophysics Data System (ADS)

On the basis of thermal imaging examinations of 2000 patients suffering lumbar osteochondrosis of the vertebral region, and using their methods, the authors describe the specifics of the thermal images of the lumbar region and the lower extremities in various clinical manifestations of the reflex and radicular syndromes.

Kolesov, S. N.; Fedosenko, T. S.

1993-11-01

426

Clinical Manifestations, Etiology, and Pathogenesis of the Hyper IgE Syndromes  

PubMed Central

Autosomal dominant Hyper IgE syndrome (AD-HIES) is a rare primary immunodeficiency characterized by eczema, recurrent skin and lung infections, elevated serum IgE, and various connective tissue, skeletal, and vascular abnormalities. Mutations in Signal transducer and activator of transcription 3 (STAT3) have recently been found to account for the majority of cases, however the pathogenesis of the varied features remains poorly defined. A distinct syndrome, known as autosomal recessive HIES (AR-HIES) manifests as severe eczema, recurrent bacterial and viral skin infections, and sinopulmonary infections. As opposed to STAT3 deficient HIES, AR-HIES lacks the connective tissue and skeletal manifestations but has an increase in neurologic abnormalities. In this review, we discuss the clinical presentations, genetic etiologies, and immunologic abnormalities of these two syndromes. In addition, we discuss animal models of STAT3 deficiency that provide insight into the pathogenesis of HIES. Further understanding of how STAT3 results in the diverse manifestations of HIES will allow us to develop more specific therapies for HIES as well as for many of the manifestations, such as scoliosis, recurrent staphylococcal infections and eczema, that are common in the general population.

Freeman, Alexandra F.; Holland, Steven M.

2009-01-01

427

Facet effect manifestation during crystallization from small volumes of solution in melt  

NASA Astrophysics Data System (ADS)

Manifestations of the facet effect are possible during the thermomigration of discrete inclusions of the solution of a growing crystal material in small volumes of the melt of a solvent metal. Similar to the case of a bulk crystal growth, the facet effect in small volumes is related to the nonequilibrium trapping of impurity by singular regions of the crystallization front.

Gershanov, V. Yu.; Garmashov, S. I.

2011-07-01

428

Conflict Management Strategies And Manifest Conflict: Do They Matter In Buyer\\/Seller Relationships?  

Microsoft Academic Search

This study addresses the gap in research concerning conflict and conflict management in buyer\\/seller relationships. Two types of manifest conflict, functional and dysfunctional, were tested to determine the distinct effect of each on the buyer\\/seller relationship and to determine if the two types of conflict are determined in some way by the choice of one of five management strategies available

Lisa C. Toms

2006-01-01

429

Vertigo as Manifestation of Vertebral Artery Dissection after Chiropractic Neck Manipulations  

Microsoft Academic Search

We recently observed a case of vertebral artery (VA) dissection following chiropractic neck manipulations. The first manifestation was unusual; in the form of vertigo. Therefore, the patient was referred to the otoneurologist. A VA dissection should be suspected in a case of vertigo following chiropractic neck manipulations, and vestibular tests should be done carefully, avoiding Rose’s positions. In our case,

Dominique Vibert; Josette Rohr-Le Floch; Gèrard Gauthier

1993-01-01

430

Acute renal failure as an initial manifestation of acute lymphoblastic leukemia.  

PubMed

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Acute renal failure is a well-recognized complication of ALL after initiation of chemotherapy. Renal failure as the primary manifestation of ALL is rare. Here, we report three children who presented with acute renal failure and hyperuricemia and were subsequently diagnosed to have ALL. PMID:23960347

Bhatia, N G; Sneha, L M; Selvan, S M; Scott, J J X

2013-07-01

431

Influence of intermediate and uninterrupted FMR1 CGG expansions in premature ovarian failure manifestation  

Microsoft Academic Search

BACKGROUND: Studies attempting to precisely define the range of fragile mental retardation 1 (FMR1) expansions and its influence in premature ovarian failure (POF) manifestation are partially lacking. To this aim, we evaluated a large cohort of POF patients for the size and, in selected cases, for the sequence of the CGG expansion. Furthermore, the correlation between POF and X-inactivation was

B. Bodega; S. Bione; L. Dalprà; D. Toniolo; F. Ornaghi; W. Vegetti; E. Ginelli; Anna Marozzi

2005-01-01

432

Organizing pneumonia by paragonimiasis and coexistent aspergilloma manifested as a pulmonary irregular nodule.  

PubMed

Organizing pneumonia by paragonimiasis and coexistent aspergilloma as a pulmonary nodule is a rare case of lung disease. Its radiographic or CT feature has not been described before in the radiologic literature. We present organizing pneumonia by paragonimiasis and coexistent aspergilloma manifested as a pulmonary irregular nodule on CT. PMID:22606555

Lee, In Jae; Seo, Jinwon; Kim, Dong Gyu

2011-07-02

433

Organizing Pneumonia by Paragonimiasis and Coexistent Aspergilloma Manifested as a Pulmonary Irregular Nodule  

PubMed Central

Organizing pneumonia by paragonimiasis and coexistent aspergilloma as a pulmonary nodule is a rare case of lung disease. Its radiographic or CT feature has not been described before in the radiologic literature. We present organizing pneumonia by paragonimiasis and coexistent aspergilloma manifested as a pulmonary irregular nodule on CT.

Lee, In Jae; Seo, Jinwon; Kim, Dong Gyu

2011-01-01

434

Hemobilia as the initial manifestation of cholangiocarcinoma in a hemophilia B patient  

Microsoft Academic Search

Hemobilia is a rare manifestation of hemophilia and is usually iatrogenic following liver biopsy. There are only few reports of spontaneous hemobilia in hemophilia patients. Cholangiocarcinoma is a well- established cause of hemobilia. We describe a case of a 70-year-old male, with known haemophilia B and a past history of papillotomy, who presented with classical symptoms of hemobilia. The initial

Anastassios C Manolakis; Andreas N Kapsoritakis; Antonis D Tsikouras; Fotis D Tsiopoulos; Athanassios K Psychos

2008-01-01

435

Isolated corneal pseudodendrites as the initial manifestation of tyrosinemia type II in monozygotic twins.  

PubMed

Fifteen-month-old twins presented with photophobia and bilateral corneal pseudodendrites, and tyrosinemia type II was suspected. Plasma tyrosine levels were elevated. After therapy with tyrosine-restricted diet, corneal lesions resolved. Bilateral pseudodendritic keratitis may be the initial or only manifestation of tyrosinemia type II. PMID:22588828

Kymionis, George D; Kankariya, Vardhaman P; Kontadakis, Georgios A; Ziakas, Nikolas G

2012-05-08

436

The NGO-ization of Community Colleges: One (More) Manifestation of Globalization  

ERIC Educational Resources Information Center

|In this essay the author discusses the effects of globalization on Canadian community colleges. She applies contemporary social theories culled from the fields of feminism, geography and political science to understand one hidden manifestation of globalization in community colleges: involvement in global civil society via participation in…

Quint-Rapoport, Mia

2006-01-01

437

Human-computer interaction in information retrieval: nature and manifestations of feedback  

Microsoft Academic Search

This study develops a theoretical framework for expressing the nature of feedback as a critical process in interactive information retrieval (IR). Feedback concepts from cybernetics and social sciences perspectives are used to develop a concept of informational feedback applicable to IR. Models from human-computer interaction and interactive IR are then adapted as a framework for studying the manifestations of feedback

Amanda Spink; Tefko Saracevic

1998-01-01

438

[Peculiarities of clinical manifestations in children with the irritable bowel syndrome].  

PubMed

The article is devoted to the problem of clinical manifestation of irritated bowels syndrome among children. It is stated that the leading symptom of suffering - abdominal pian - is combined with the psychopathologic symptomatology such as various neurosis-like disorders, which should be taken into consideration while developing therapeutic measures among such patients. PMID:23786013

Buriak, V N; Bessarab, P A

2012-12-01

439

Delayed manifestation of aortic stenosis after blunt abdominal trauma: Report of a case  

Microsoft Academic Search

Delayed manifestation of aortic stenosis caused by abdominal blunt trauma is rare. We report herein the case of a 67-year-old man who was taken to a nearby hospital after being crushed between a heavy truck and a wall. An emergency laparotomy was performed, revealing only a mesenteric tear which was repaired. He was discharged after an uneventful postoperative course; however,

Shunya Shindo; Kouji Ogata; Seiichiro Katahira; Keiji Iyori; Tadao Ishimoto; Masahiro Kobayashi; Osamu Suzuki; Kihachiro Kamiya; Yusuke Tada

1997-01-01

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…

Kennedy, Patricia

2008-01-01

441

Subscales to the Taylor Manifest Anxiety Scale in Three Chronically Ill Populations.  

ERIC Educational Resources Information Center

|Examines factors of anxiety in the Taylor Manifest Anxiety Scale in 150 asthma, tuberculosis, and chronic pain patients. Key cluster analysis revealed five clusters: restlessness, embarrassment, sensitivity, physiological anxiety, and self-confidence. Embarrassment is fairly dependent on the other factors. (JAC)|

Moore, Peter N.; And Others

1984-01-01

442

Mechanical and myoelectric manifestations of fatigue in subjects with anorexia nervosa  

Microsoft Academic Search

PurposeAim of this work is to compare mechanical and myoelectric manifestations of fatigue during an isometric contraction at 80% of maximal voluntary contraction (MVC) in a population of eight anorexic female patients (AN: 24.9±6.5 years, mean±SD) with respect to a group of seven healthy female subjects (CO: 30.0±6.6 years, mean±SD).

Giovanni Melchiorri; Alberto Rainoldi

2008-01-01

443

Gastrointestinal manifestations of Fabry disease: Clinical response to enzyme replacement therapy  

Microsoft Academic Search

Gastrointestinal symptoms are often an early and prominent manifestation of Fabry disease, an X-linked inborn error of metabolism caused by the deficient activity of the lysosomal enzyme, ?-galactosidase A. This enzyme deficiency results in the progressive accumulation of globotriaosylceramide and other glycosphingolipids in tissue lysosomes throughout the body. In classically affected patients, glycosphingolipid accumulation in the vascular endothelium eventually culminates

Maryam Banikazemi; Thomas Ullman; Robert. J. Desnick

2005-01-01

444

Mucocutaneous manifestations and nail changes in patients with end-stage renal disease on hemodialysis.  

PubMed

Mucocutaneous manifestations are common among patients on hemodialysis (HD). This study was undertaken to determine the prevalence of mucocutaneous manifestations in patients with end-stage renal disease (ESRD) who are on HD. In this cross-sectional, descriptive and analytic study conducted in 2009, 100 patients on HD at the Five Azar Hospital in Gorgan city were randomly selected. All the patients underwent detailed examination by a dermatologist to look for lesions in the skin, hair, nail and mucous membranes; if felt necessary, biopsy was obtained from the lesions. The findings were statistically analyzed using SPSS-13 software. For evaluation of normality of distribution, Kolmogorov-Smirnov was used, for quantitative variables Mann-Whitney and T-test (abnormal distribution) were used and for qualitative variables, Chi-2 and Fisher were used. In this study, P-value less than 0.05 was considered significant. Fifty-one males and 49 females were enrolled. The mean age was 49 ± 12 years. Diabetes was the most common cause of ESRD. In 95% of the patients, at least one mucocutaneous manifestation was present. Xerosis (78.3%) was the most common lesion, followed by pruritus (39.1%), lentigo (34.8%), skin discoloration (32.6%), leukonychia (32%) and thinning of the nail bed (24%). Xerosis, scaling, lentigo, folliculitis, idiopathic guttate hypopigmentation, leukonychia and half and half nail were associated with age. A significant relationship was seen between duration on dialysis and skin discoloration and leukonychia. Clubbing had a significant association with calcium-phosphorus product (Ca × P). There was a significant association between serum ferritin level and pruritus and tinea versicolor lesions. Our study shows that mucocutaneous manifestations are common among patients with ESRD. Identification of these manifestations and their association with causative factors are useful for preventing the lesions. PMID:23354189

Tajbakhsh, Ramin; Dehghan, Mohammad; Azarhoosh, Ramin; Haghighi, Ali Nobakht; Sadani, Somayeh; Zadeh, Soheila Samad; Kabootari, Maryam; Qorbani, Mostafa

2013-01-01

445

Neurological Manifestation in New Emerging H7N9 Influenza:an Issue in Neurology.  

PubMed

The emerging of new H7N9 influenza virus infection in February 2013 brings attention of world health scientists on its possibility to cause worldwide pandemic (1). This disease was firstly identified as a severe respiratory infection with unknown pathogenic cause. Finally, the new virus can be identified and it is confirmed that the new H7N9 bird flu is the new emerging viral infection( 2-3). The new H7N9 bird flu is a new disease manifesting with acute respiratory features plus additional atypical clinical manifestations. Of those atypical clinical features, neurological presentation is an interesting issue. This work presents the summary on evidence on neurological manifestation in new emerging H7N9 influenza. Neurological disorder in new H7N9 influenza Neurological manifestation can be observed in the patients infected with new H7N9 influenza. According to the report on 111 infected cases, the finding of neurological disorder is not significant(4). However, almost all infected cases usually present the compliant of dizziness as well as decreased level of consciousness(4-5). Also, it is noted that multiple organ involvement is common in the severe cases and the neurological signs as alteration of consciousness can be seen(4-5). According to the case series reported from Huzhou, one-sixth of the cases manifested muscle weakness(6). The question is whether the new H7N9 influenza virus can cause direct pathology to neurological system or not. The recent publication in Science might be the clue for this query(7). According to the animal model, it is proved that the H7N9 influenza can infect into the brain tissue and this might be the possible explanation for the observation on the neurological manifestations in severe H7N9 influenza cases. The abnormality in neurological system can be seen in the patients with H7N9 influenza and this is the point for closed monitoring in patient management. PMID:24030094

Wiwanitkit, Viroj

2013-09-01

446

15 CFR 30.45 - General statement of requirements for the filing of carrier manifests with proof of filing...  

Code of Federal Regulations, 2013 CFR

...carrier manifests with proof of filing citations for the electronic submission of export...carrier manifests with proof of filing citations for the electronic submission of export...contain the appropriate AES proof of filing citations, covering all cargo for which...

2013-01-01

447

15 CFR 30.45 - General statement of requirements for the filing of carrier manifests with proof of filing...  

Code of Federal Regulations, 2012 CFR

...carrier manifests with proof of filing citations for the electronic submission of export...carrier manifests with proof of filing citations for the electronic submission of export...contain the appropriate AES proof of filing citations, covering all cargo for which...

2012-01-01

448

19 CFR 4.76 - Procedures and responsibilities of carriers filing outbound vessel manifest information via the AES.  

Code of Federal Regulations, 2013 CFR

...outbound vessel manifest information via the AES. 4.76 Section 4.76 Customs Duties...outbound vessel manifest information via the AES. (a) The sea carrier's module...component of the Automated Export System (AES) (see, part 192, subpart B,...

2013-04-01

449

Electroencephalographic and seizure manifestations in two patients with folate receptor autoimmune antibody-mediated primary cerebral folate deficiency.  

PubMed

Seizure semiology and electroencephalographic (EEG) manifestations of autoimmune-mediated cerebral folate deficiency (CFD) before and after therapy have yet to be fully characterized. Here, we report these findings in two such patients. Our first patient presented with the novel manifestation of infantile spasms at the age of 3months, while the second developed the previously reported initial onset of tonic seizures with static developmental delay, but subsequently manifested the novel finding of electrical status epilepticus in sleep at the age of 15years. Awareness of these new manifestations, together with the previously reported manifestations of developmental delay, seizure onset during the first 2years of life, occurrence of tonic, myoclonic-astatic, absence, and generalized tonic-clonic seizures, with an EEG of generalized spike-slow waves and multifocal spikes, is important to increase the index of suspicion of this treatable disorder. PMID:22749608

Steele, Sonya U; Cheah, Sue Mei; Veerapandiyan, Aravindhan; Gallentine, William; Smith, Edward C; Mikati, Mohamad A

2012-06-30

450

Pure epileptic headache and related manifestations: a video-EEG report and discussion of terminology.  

PubMed

We present the first video-EEG recording of episodes of "epileptic headache". The case reported is that of a 9-year-old girl with brief episodes (of a few minutes) of severe frontal headache, which corresponded to the presence of concurrent spikes and slow waves, starting in the right temporal area. A dysplastic lesion of the right temporal lobe was observed by MRI and the patient received surgery, with subsequent disappearance of headaches. This case highlights ictal EEG as the main diagnostic tool for epileptic headache. We discuss the terminology regarding this type of manifestation and believe that cases without subsequent epileptic manifestations, as in the present case, should be more appropriately referred to as "pure ictal epileptic headache" or simply "pure epileptic headache". [Published with video sequences]. PMID:23518661

Cianchetti, Carlo; Pruna, Dario; Porcu, Lucia; Peltz, Maria Teresa; Ledda, Maria Giuseppina

2013-03-01

451

Clinical guidelines for diagnosing and managing ocular manifestations in children with mucopolysaccharidosis.  

PubMed

The mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans in several tissues and organs. This accumulation results in an array of clinical manifestations and premature death in severe cases. Ocular problems are very common in children with MPS and may involve the cornea, sclera, trabecular meshwork, retina, optic nerve and also the posterior visual pathways. The aims of this study are to give an overview of ocular problems in MPS and to provide clinical guidelines for paediatric ophthalmologists for early diagnosis and management of ocular manifestations in children with MPS. Diagnostic problems may arise in children with severe corneal clouding, hampering visualization of the fundus. Intraocular pressures may be falsely high, even leading to suspicion and unnecessary pressure-lowering treatment. Simple interventions such as the use of prescription glasses or photochromatic glasses can considerably improve quality of life in children with MPS. PMID:22136369

Fahnehjelm, Kristina T; Ashworth, Jane L; Pitz, Susanne; Olsson, Monica; Törnquist, Alba Lucia; Lindahl, Päivi; Summers, C Gail

2011-12-02

452

Cryptococcuria as a manifestation of disseminated cryptococcosis and isolated urinary tract infection.  

PubMed

Fungal infection of the genitourinary system is a relatively uncommon presentation. Cryptococcuria has rarely been recognized in clinical practice. Patients with positive urine culture for Cryptococcus neoformans from 1992 to 2003 were retrospectively reviewed. Sixteen patients were identified. Nine (56%) patients were male, with a mean age of 44 +/- 21 (range, 16-88) years. Fifteen (94%) patients had underlying conditions such as HIV infection, diabetes mellitus, hypertension, and/or systemic lupus erythematosus. Thirteen (81%) patients had cryptococcuria as a manifestation of disseminated cryptococcosis, and the rest had only isolated cryptococcuria. Urinary analysis revealed proteinuria (75%), pyuria (31%), and budding yeast (13%). Nine (56%) patients received antifungal therapy. Other patients were misdiagnosed or died before treatment. The mortality rate was 64%. In conclusion, cryptococcuria is not extremely rare and can present as a manifestation of disseminated cryptococcosis or isolated urinary tract infection. PMID:15507776

Kiertiburanakul, Sasisopin; Sungkanuparph, Somnuek; Buabut, Benjamas; Pracharktam, Roongnapa

2004-10-01

453

Musician's cramp as manifestation of maladaptive brain plasticity: arguments from instrumental differences.  

PubMed

Musician's cramp is a task-specific movement disorder that presents itself as muscular incoordination or loss of voluntary motor control of extensively trained movements while a musician is playing the instrument. It is characterized by task specificity and gender bias, affecting significantly more males than females. The etiology is multifaceted: a combination of a genetic predisposition, termed endophenotype, and behavioral triggering factors being the leading features for the manifestation of the disorder. We present epidemiological data from 591 musician patients from our outpatient clinic demonstrating an influence of fine-motor requirements on the manifestation of dystonia. Brass, guitar, and woodwind players were at greater risk than other instrumentalists. High temporospatial precision of movement patterns, synchronous demands on tonic and phasic muscular activation, in combination with fine-motor burdens of using the dominant hand in daily life activities, constitute as triggering factors for the disorder and may explain why different body parts are affected. PMID:22524368

Altenmüller, Eckart; Baur, Volker; Hofmann, Aurélie; Lim, Vanessa K; Jabusch, Hans-Christian

2012-04-01

454

Use of pressure manifestations following the water plasma expansion for phytomass disintegration.  

PubMed

A prototype capable of generating underwater high-voltage discharges (3.5 kV) coupled with water plasma expansion was constructed. The level of phytomass disintegration caused by transmission of the pressure shockwaves (50-60 MPa) followed by this expansion was analyzed using gas adsorption techniques. The dynamics of the external surface area and the micropore volume on multiple pretreatment stages of maize silage and sunflower seeds was approximated with robust analytical techniques. The multiple increases on the reaction surface were manifest in up to a 15% increase in cumulative methane production, which was itself manifest in the overall acceleration of the anaerobic fermentation process. Disintegration of the sunflower seeds allowed up to 45% higher oil yields using the same operating pressure. PMID:23579822

Maroušek, Josef; Kwan, Jason Tai Hong

2013-01-01

455

Atypical megadolichoectasia manifesting as brain infarction rapidly followed by fatal subarachnoid hemorrhage.  

PubMed

A 71-year-old female, without medical or family history for cerebrovascular disease, presented with basilar and bilateral carotid dolichoectasia manifesting as dysarthria and hemisensory disturbance, which resolved spontaneously within a day. She suffered brainstem infarction 28 months later, manifesting as drowsiness, dysarthria, and right hemiparesis. Her consciousness level progressively deteriorated to stupor within 4 days. Computed tomography taken on the 5th day confirmed cerebellar infarct in the perfusion area of the superior cerebellar artery but did not show subarachnoid hemorrhage. She died of acute respiratory failure on the 7th day. Autopsy demonstrated a tear in the lateral wall of the broad-based aneurysm on the ectatic basilar artery and diffuse subarachnoid hemorrhage. Vertebrobasilar ectasia is a dynamic vasculopathy that may rapidly progress in the affected basilar artery following an indolent clinical course. The prognosis for patients with vertebrobasilar ectasia may depend mainly on the pathological changes in the basilar artery. PMID:19226335

Tsutsumi, Satoshi; Yasumoto, Yukimasa; Ito, Masanori

2010-10-01

456

Skin manifestations of chronic hepatitis C virus infection in Cairo, Egypt.  

PubMed

We assessed the dermatological manifestations associated with chronic hepatitis C virus (HCV) infection and their association with liver status. Of 155 patients with chronic HCV infection in Cairo, Egypt, 71 (45.8%) had dermatological manifestations: pruritus without evident skin lesions (21.3%), pigmented purpuric eruption (5.2%), aphthous ulcer and lichen planus (3.9% each), leukocytoclastic vasculitis (2.6%), psoriasis (1.9%), tinea versicolor (1.3%) and other conditions (5.8%). Shrunken liver, splenomegaly and ascites were significantly associated with the presence of skin lesions (relative risk 8.0, 2.7 and 1.8 respectively), and shrunken liver was significantly associated with pruritus (relative risk 2.1). Sex was not associated with any of the skin lesions. PMID:19731785

Raslan, H M Z; Ezzat, W M; Abd El Hamid, M F; Emam, H; Amre, K S

457

Long-term follow-up of neurological manifestations in a boy with incontinentia pigmenti.  

PubMed

Incontinentia pigmenti (IP) is an X-linked dominant genodermatosis confined to females. It is usually lethal in males. However, the survival of some males has been reported in literature. We describe a long follow-up case of a 12-year-old male with IP and a normal karyotype but a genomic deletion of the NEMO gene in the Xq28 position in the form of somatic mosaicism. The patient showed severe ophthalmic abnormalities and neurological manifestations characterised by very mild cerebellar ataxia and a history of epilepsy that was severe at the beginning with West syndrome, become moderate overtime and is now resolved. Despite these neurological manifestations, probably related to the presence of at least some mutated cells in his brain, the long-term follow-up in this patient demonstrated good neurological and cognitive outcome. PMID:23652938

Margari, Lucia; Lamanna, Anna Linda; Buttiglione, Maura; Craig, Francesco; Petruzzelli, Maria G; Terenzio, Vanessa

2013-05-08

458

Celiac Disease and Dermatologic Manifestations: Many Skin Clue to Unfold Gluten-Sensitive Enteropathy  

PubMed Central

Cutaneous manifestations of intestinal diseases are increasingly reported both in the adult and in the children, and this association cannot longer be considered a simple random. Besides the well-known association between celiac disease (CD) and dermatitis herpetiformis (DH), considered as the cutaneous manifestation of gluten-dependent enteropathy, is more frequently reported also the association with other mucocutaneous diseases. Among these there are both autoimmune, allergic, and inflammatory diseases, but also a more heterogeneous group called miscellaneous. The knowledge about pathogenic, epidemiological, clinical, and diagnostic aspects of CD is increasing in recent years as well as those about DH, but some aspects still remain to be defined, in particular the possible pathogenetic mechanisms involved in the association between both CD and DH and CD and other immunological skin diseases. The aim of this paper is to describe the skin diseases frequently associated with CD, distinguishing them from those which have a relationship probably just coincidental.

Caproni, Marzia; Bonciolini, Veronica; D'Errico, Antonietta; Antiga, Emiliano; Fabbri, Paolo

2012-01-01

459

Hypoparathyroidism as the major manifestation in two patients with 22q11 deletions  

SciTech Connect

We report on two adolescents with 22q11 deletion. Their main clinical manifestation was chronic symptomatic hypocalcemia secondary to hypoparathyroidism, together with seizures and cerebral calcifications. Neither congenital cardiac abnormality nor T cell deficiency were detected. The phenotypic manifestations of the observed patients were consistent with velo-cardiofacial syndrome (VCFS). A microdeletion of chromosome region 22q11 has been demonstrated in approximately 90% of DiGeorge syndrome (DGS) patients and in 75% of VCFS patients; the association of the deletion with a wide spectrum of clinical findings suggests the existence of a contiguous gene syndrome. The presence of certain traits of DGS/VCFS should lead to investigations of parathtroid function and molecular analysis of the 22q11 region hybridization studies. 10 refs., 5 figs., 2 tabs.

Scire, G.; Bonaiuto, F.; Galasso, C.; Boscherini, B.; Dallapiccola, B.; Mingarelli, R.; Iannetti, P. [Univ. of Rome La Sapienza (Italy)

1994-10-01

460

[Oropharyngeal dysphagia as a first manifestation of dermatomyositis associated with colon cancer].  

PubMed

Dermatomyositis (DM) is an idiopathic inflammatory myopathy associated with characteristic skin manifestations. In 15-20% of patients present with dysphagia, it is associated with nutritional deficiency, predisposition to aspiration pneumonia, decreased quality of life and a poor prognosis. There is a well-recognized association between DM and malignancies, including ovarian, breast, lung, and colon cancer. We report a case of a male patient aged 85 with DM associated with colon adenocarcinoma; progressive dysphagia was the first manifestation, and subsequently proximal muscle weakness and typical skin lesions were present. Given the clinical suspicion of DM as a paraneoplastic syndrome, tumor markers were order and a high carcinoembryonic antigen was found. A colonoscopy study and histopathologic examination revealed the presence of adenocarcinoma of the colon. PMID:21169124

Espinoza-Cobos, J C; Pérez-Figueroa, J; Zúñiga-Ahuet, G; Dorantes, M A; Grube-Pagola, P; Ruíz-Juárez, I; Remes-Troche, J M

461

Perplexing imaging manifestations of multiple metastatic intracranial lesions associated with atrial myxoma.  

PubMed

Neurological manifestations of atrial myxomas may be encountered in different pathological circumstances: cerebral embolism, intracranial aneurysms, and metastatic mass lesions. We report a case of multiple metastatic intracranial lesions associated with atrial myxoma and discuss the potential mechanisms of its perplexing neuroimaging manifestations. Comparing with previous reports of myxoma-associated intracranial lesions, the most notable features of the present case were the multiple high-density "calcified" lesions accompanying the aneurysms on computed tomography scan and the significant contrast-enhanced mass in the left parietal lobe on magnetic resonance scan. This suggested an extremely rare case of coexistence of aneurysms and intracranial metastatic mass lesion associated with cardiac myxoma, although the pathological evidence was not available. PMID:23524767

Zhan, Renya; Ji, Tao; Fan, Zuoxu; Pan, Desheng

2013-03-01

462

Werner syndrome: a changing pattern of clinical manifestations in Japan (1917~2008).  

PubMed

As ~75% of the Werner syndrome (WS) patients recognized between 1904 and 2008 all over the world are of Japanese origin, the most case reports and clinical studies on WS has been published in Japanese journals. Thus, the detailed English-written clinical review on the recent WS case reports has been warranted. Although WS has been characterized by a variety of clinical manifestations mimicking premature aging, the recent longevity and delayed age-associated manifestations observed both from Japanese WS and general population may suggest a common environmental effect on some gene(s) other than WRN and may give us a newer pathophysiological look at WS and also natural aging through the molecular dysfunction of WRN. PMID:23524889

Goto, M; Ishikawa, Y; Sugimoto, M; Furuichi, Y

2013-02-01

463

Protean manifestations of lipoid proteinosis in a 16-year-old boy.  

PubMed

We report a 16-year-old Japanese male with lipoid proteinosis showing various skin manifestations. The patient was born to nonconsanguineous parents and none of his relatives was similarly affected. The patient suffered from a hoarse voice and refractory temporal epilepsy from early childhood. Computed tomography scanning of the brain showed bilateral calcification in the temporal lobes, a characteristic feature of lipoid proteinosis. On physical examination, various skin manifestations, including papules and haemorrhagic blisters, acne-like scars at sites of minor trauma or friction, and beads of small papules along the free margins of the eyelids were noted. A skin biopsy showed deposits of homogeneous hyaline-like material, positive on periodic acid-Schiff staining, throughout the dermis, particularly around small blood vessels. It is noteworthy that a range of characteristic skin lesions can be present in a patient with lipoid proteinosis even with mild systemic involvement. PMID:10671967

Nagasaka, T; Tanaka, M; Ito, D; Tanaka, K; Shimizu, H

2000-01-01

464

Renal manifestations of severe Rabson-Mendenhall syndrome: a case report  

PubMed Central

Introduction Rabson-Mendenhall Syndrome (RMS) is a rare form of severe insulin resistance due to a recessive mutation of the insulin receptor. Associated manifestations include facial dysmorphism, skin abnormalities, and renal anomalies. Case presentation We report a case of a 13 year old African female with RMS, severe insulin resistance, and a cluster of renal pathologies including nephromegaly, nephrolithiasis, hydronephrosis, and medullary sponge kidney. Conclusion This is the first case of severe insulin resistance associated with the collection of renal conditions described. We postulate that renal conditions present in RMS may be under recognised, and recommend screening for the above conditions. This case adds to the scarce body of literature of associated renal manifestations with RMS, including medullary sponge kidney, across the spectrum of insulin resistance.

2013-01-01

465

Nonopportunistic Neurologic Manifestations of the Human Immunodeficiency Virus: An Indian Study  

Microsoft Academic Search

Context  HIV-1 is a neurotropic virus. In a resource-limited country such as India, large populations of affected patients now have\\u000a access to adequate chemoprophylaxis for opportunistic infections (OIs), allowing them to live longer. Unfortunately the poor\\u000a availability of highly active antiretroviral therapy (HAART) has allowed viral replication to proceed unchecked. This has\\u000a resulted in an increase in the debilitating neurologic manifestations

Alaka K Deshpande; Mrinal M Patnaik

2005-01-01

466

Dermatological Manifestations in Primary Biliary Cirrhosis Patients: A Case Control Study  

Microsoft Academic Search

OBJECTIVES:Primary biliary cirrhosis (PBC), a disease of probable autoimmune etiology that affects the small intrahepatic bile ducts of mainly middle-aged women is commonly associated with pruritus, xanthomatous lesions, and melanosis. We conducted a prospective study to systematically describe the skin disorders of a group of PBC patients.METHODS:A prospective evaluation and analysis of dermatological manifestations including oral and genital lesions was

Meri Koulentaki; Despina Ioannidou; Maria Stefanidou; Sofia Maraki; I. Drigiannakis; Philippas Dimoulios; Jean Marie Enele Melono; Androniki Tosca; Elias A. Kouroumalis

2006-01-01

467

Potential Role of Oxidative Damage in Neurological Manifestations of Acute Intermittent Porphyria  

Microsoft Academic Search

\\u000a Accumulation of 5-aminolevulinic acid (ALA) is the main defect in acute porphyria and the most likely potential candidate\\u000a to cause acute neurological manifestations during an acute porphyric attack via multiple direct and indirect mechanisms. ALA\\u000a is a potential endogenous source of reactive oxygen species (ROS). After administration of ALA or inducers of ALA-synthase\\u000a in in vitro conditions or in animal

Elena Pischik; Raili Kauppinen

468

A Case of Metastatic Squamous Cell Carcinoma of the Hypopharynx Manifesting as Acute Abdomen  

Microsoft Academic Search

Introduction: Squamous cell head and neck cancers are usually confined to local and regional sites but occasionally, patients may present with rare manifestations of distant metastases. Clinical Picture: A 23-year-old female was treated with concurrent chemo-radiotherapy for stage IVA post-cricoid squamous cell carcinoma. A month later, she presented with acute abdomen and was found to have extensive peritoneal nodules at

Leong Swan Swan