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1

Cutaneous Manifestations  

Microsoft Academic Search

Cutaneous lesions are well-recognized extraintestinal manifestations of inflammatory bowel disease (IBD), specifically. Crohn’s\\u000a disease (CD) and ulcerative colitis (UC). These extraintestinal manifestations, associated with intestinal symptoms, contribute\\u000a to the impaired quality of life of patients with CD. During the course of the disease, a great variety of cutaneous lesions\\u000a may develop, many of which are secondary to granulomatous cutaneous disease,

Luciano Alessandroni; Andrea Scotti

2

Extrahepatic manifestations of HCV.  

PubMed

The hepatic consequences of an infection with the hepatitis C virus (HCV) are well recognised, but extrahepatic manifestations of HCV may be just as severe. Here we have reviewed various extrahepatic manifestations of HCV such as mixed cryoglobulinemia, lymphoma, metabolic features and neurologic consequences and we discuss pathogenesis and management of these clinical problems. We concluded with important aspects of therapy with novel anti-HCV agents and its effects on extrahepatic manifestations. PMID:25390287

Grignoli, R; Goossens, N; Negro, F

2015-03-01

3

Electrocardiographic manifestations of hyperkalemia  

Microsoft Academic Search

Hyperkalemia is one of the more common acute life-threatening metabolic emergencies seen in the emergency department. Early diagnosis and empiric treatment of hyperkalemia is dependent in many cases on the emergency physician's ability to recognize the electrocardiographic manifestations of hyperkalemia. The electrocardiographic manifestations commonly include peaked T-waves, widening of the QRS-complex, and other abnormalities of altered cardiac conduction. Peaked T-waves

Amal Mattu; William J. Brady; David A. Robinson

2000-01-01

4

Haematological manifestations of lupus  

PubMed Central

Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent TTP. Myelofibrosis is an uncommon yet well-documented manifestation of SLE. We have compiled the cases that were reported in MEDLINE sources. PMID:25861458

Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

2015-01-01

5

Cutaneous Manifestations of ESRD  

PubMed Central

Summary A broad range of skin diseases occurs in patients with ESRD: from the benign and asymptomatic to the physically disabling and life-threatening. Many of them negatively impact on quality of life. Their early recognition and treatment are essential in reducing morbidity and mortality. The cutaneous manifestations can be divided into two main categories: nonspecific and specific. The nonspecific manifestations are commonly seen and include skin color changes, xerosis, half-and-half nails, and pruritus. The specific disorders include acquired perforating dermatosis, bullous dermatoses, metastatic calcification, and nephrogenic systemic fibrosis. This review article describes these conditions and considers the underlying pathophysiology, clinical presentations, diagnosis, and treatment options. PMID:24115194

Cronin, Antonia J.; Leslie, Kieron S.

2014-01-01

6

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

7

Manifest and Latent Variates  

ERIC Educational Resources Information Center

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

Maraun, Michael D.; Halpin, Peter F.

2008-01-01

8

[Urologic manifestations of AIDS].  

PubMed

The frequency of urological manifestations of AIDS appears to be increasing at the present time: between 1985 and 1988, 38 HIV1 seropositive patients were examined in the Urology department. In seven cases, the positive serology was discovered during investigation of a urogenital infection and 21 patients suffered from declared AIDS. The reasons for admission to hospital were: urogenital infection: 14 cases, dominated by epididymitis (7) and prostatitis (5), characterized by their frequent progression towards abscess formation; tumours: 6 cases, including 5 Kaposi sarcomas of the penis; and, lastly, neurological manifestations, chronic renal failure and urological diseases unrelated to AIDS. This study indicates that serology should be performed routinely in cases with a known risk factor and/or with an atypical urogenital condition, particularly infectious. The adoption of aseptic techniques and rigorous protection of the entire surgical team is now absolutely necessary. PMID:2742345

Boccon-Gibod, L; Delmas, V; Baron, J C

1989-01-01

9

Cutaneous Manifestations of Hypothyroidism  

Microsoft Academic Search

\\u000a On any given day, in any given bustling dermatology practice, it is highly likely that at least one patient has clinically\\u000a overt or subclinical hypothyroidism. The cutaneous manifestations of hypothyroidism are protean, affecting the skin and its\\u000a appendages, as outlined in this chapter. Hypothyroidism literally affects all organ systems and has a profound effect on a\\u000a patient's overall health and

Sandra A. Kopp; Pascal G. Ferzli; Chad M. Hivnor; Warren R. Heymann

10

Pulmonary manifestations of bioterrorism  

Microsoft Academic Search

Along with smallpox, inhalation anthrax and pneumonic plague are among the diseases most likely to be spread by biowarfare,\\u000a either from a rogue nation or terrorist group. Neither anthrax nor plague has been seen by many pulmonary (or any other) physicians\\u000a in the United States. This article summarizes these two diseases as pulmonary manifestations of bioterrorism and discusses\\u000a the possibility

Renuka Heddurshetti; Wadchara Pumpradit; Larry I. Lutwick

2001-01-01

11

Cutaneous Manifestations of Hyperthyroidism  

Microsoft Academic Search

\\u000a The key to diagnosing hyperthyroidism from a dermatologic perspective is based on having a high index of suspicion that excess\\u000a thyroid hormone is responsible for the patient's signs and symptoms. As there are no definitive cutaneous manifestations of\\u000a hyperthyroidism, a careful review of systems may yield important clinical clues to the diagnosis: Is the patient intolerant\\u000a of heat? Has there

Clara-Dina Cokonis; Carrie W. Cobb; Warren R. Heymann; Chad M. Hivnor

12

[Respiratory manifestations in aspergillosis].  

PubMed

Aspergillus is a genus of cosmopolitan fungi with a selective pulmonary tropism. Their pathogenic role is due either to spreading in pre-existing pulmonary cavities, or to their allergizing capacity. Cavitary sequellae of tuberculosis and suppuration, particularly frequent and important in tropical environment, are elective localization for Aspergillus colonization. Surgical treatment is nowadays the only efficient one. Allergic manifestations are a more complex problem of therapy, exclusion of allergen being difficult to get in tropical environment. PMID:3773680

Regimbaud, M

1986-01-01

13

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. PMID:22919396

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

2012-01-01

14

Radiological manifestations of melioidosis.  

PubMed

Melioidosis is a serious infection that is associated with high mortality. It is due to a Gram-negative bacterium, Burkholderia pseudomallei which is an environmental saprophyte found in wet soils. Melioidosis is endemic to northern Australia and the Southeast Asia. However, there is now increasing number of reports of imported cases to regions where this infection has not been previously encountered. Almost any organ can be affected. Like many other conditions, radiological imaging is an integral part of the diagnostic workup of melioidosis. Awareness of the various radiological manifestations can help direct appropriate investigations to achieve early diagnosis and the initiation of appropriate treatment. Generally, there are no known characteristic features on imaging that can specifically differentiate melioidosis from other infections. However, the "honeycomb" appearance has been described to be characteristic for large melioidosis liver abscesses. Simultaneous involvement of various organs is also characteristics. To date, there are few data available on the radiological manifestations of melioidosis. The present pictorial essay describes melioidosis affecting the various organs. PMID:20103424

Lim, K S; Chong, V H

2010-01-01

15

Hepatitis C: extrahepatic manifestations.  

PubMed

Chronic hepatitis C virus (HCV) infection is associated with multiple extrahepatic manifestations (EHM) affecting various organs in the body. Approximately 40% to 75% of patients with chronic HCV infection experience at least one clinical EHM during the course of the HCV infection. Mixed cryoglobulinemia (type 2) is the most documented EHM associated with chronic HCV infection. This has been documented in up to 50% of patients. Clinically, it presents as arthralgia, weakness, and cutaneous symptoms. Morphologically, immune complex depositions are identified in small vessels and glomerular capillary walls, leading to leukocytoclastic vasculitis in the skin and membranoproliferative glomerulonephritis in the kidney. In other EHMs of chronic HCV infection not related to cryoglobulinemia, such as Sjögren syndrome, lichen planus, and autoimmune thyroiditis, autoimmune processes resulting in chronic inflammatory infiltrates are thought to be the underlying mechanism. PMID:25478648

Metts, Julius; Carmichael, Lesley; Kokor, Winfred; Scharffenberg, Robert

2014-12-01

16

Manifesting the Quantum World  

NASA Astrophysics Data System (ADS)

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

Mohrhoff, Ulrich

2014-06-01

17

Manifesting the Quantum World  

E-print Network

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

Ulrich Mohrhoff

2014-04-27

18

Ovarian tumors with functioning manifestations  

Microsoft Academic Search

Various categories of ovarian tumors, particularly those of gonadal stromal origin, are capable of producing a variety of\\u000a hormones that occasionally induce interesting clinical manifestations. The endocrine manifestations associated with gonadal\\u000a stromal tumors are often due to hormone production by the tumor cells. Sometimes the tumor cells produce only one hormone,\\u000a while more frequently the hormonal manifestations result from a

Fattaneh A. Tavassoli

1994-01-01

19

Oral Manifestations of Vitiligo  

PubMed Central

Background: Vitiligo is one of the disorder that has social impact. Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment. Aim and objectives: The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site. Materials and methods: The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison's disease were excluded in this study. Results: Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1%. Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also. Conclusion: In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin. PMID:25657420

Nagarajan, Anitha; Masthan, Mahaboob Kader; Sankar, Leena Sankari; Narayanasamy, Aravindha Babu; Elumalai, Rajesh

2015-01-01

20

[Skin manifestations of monoclonal gammopathies].  

PubMed

Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies. PMID:24070793

Hello, M; Barbarot, S; Néel, A; Connault, J; Graveleau, J; Durant, C; Decaux, O; Hamidou, M

2014-01-01

21

Hematological Manifestations of Nephropathic Cystinosis  

Microsoft Academic Search

Pancytopenia is an uncommon manifestation of cystinosis, a congenital lysosomal storage disease. We describe a 34-year-old patient with nephropathic cystinosis with multisystem involvement who developed progressive bone marrow failure after renal transplantation. Bone marrow examination demonstrated widespread deposition of cystine crystals in histiocytes and in the background. We review the literature on the hematologic manifestations of cystinosis and discuss the

Ashkan Emadi; Kathleen H. Burns; Bradley Confer; Michael J. Borowitz; Michael B. Streiff

2008-01-01

22

Cutaneous manifestations of viral hepatitis.  

PubMed

There are several extrahepatic cutaneous manifestations associated with hepatitis B and hepatitis C virus infection. Serum sickness and polyarteritis nodosa are predominantly associated with hepatitis B infection, whereas mixed cryoglobulinemia associated vasculitis and porphyria cutanea tarda are more frequently seen in hepatitis C infection. The clinico-pathogenic associations of these skin conditions are not completely defined but appear to involve activation of the host immune system including the complement system. Management of the aforementioned cutaneous manifestations of viral hepatitis is often similar to that done in cases without viral hepatitis, with control of immune activation being a key strategy. In cases associated with hepatitis B and C, control of viral replication with specific antiviral therapy is also important and associated with improvement in most of the associated clinical manifestations. PMID:25809574

Akhter, Ahmed; Said, Adnan

2015-02-01

23

[Cutaneous leishmaniosis: unusual clinical manifestation].  

PubMed

Clinical manifestations of leishmaniasis are diverse and related to the infecting species, its relationship with the environment and the host immune response. A case of late Andean cutaneous leishmaniasis with extensive manifestation is presented. The case was confirmed through microbiological and immunological studies; identification was performed by cytochrome b gene sequencing and the species was determined as Leishmania (Leishmania) amazonensis. The patient was treated with sodium stibogluconate and at the end of therapy the patient showed clinical improvement of the lesions. It is recommended to consider leishmaniasis in differential diagnosis when treating atypical dermatological chronic ulcers. PMID:25418662

Sandoval-Juárez, Aidé; Minaya-Gómez, Gloria; Rojas-Palomino, Nyshon; Falconi, Eduardo; Cáceres, Omar

2014-01-01

24

Breast manifestations of systemic diseases  

PubMed Central

Although much emphasis has been placed on the primary presentations of breast cancer, little focus has been placed on how systemic illnesses may affect the breast. In this article, we discuss systemic illnesses that can manifest in the breast. We summarize the clinical features, imaging, histopathology, and treatment recommendations for endocrine, vascular, systemic inflammatory, infectious, and hematologic diseases, as well as for the extramammary malignancies that can present in the breast. Despite the rarity of these manifestations of systemic disease, knowledge of these conditions is critical to the appropriate evaluation and treatment of patients presenting with breast symptoms. PMID:22371658

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

2012-01-01

25

14 CFR 125.383 - Load manifest.  

Code of Federal Regulations, 2014 CFR

...Rules § 125.383 Load manifest. (a) Each...preparation and accuracy of a load manifest in duplicate...that flight; (4) The center of gravity limits; ...manifest indicating that the center of gravity is within...an airplane for which a load manifest must be...

2014-01-01

26

14 CFR 125.383 - Load manifest.  

Code of Federal Regulations, 2012 CFR

...Rules § 125.383 Load manifest. (a) Each...preparation and accuracy of a load manifest in duplicate...that flight; (4) The center of gravity limits; ...manifest indicating that the center of gravity is within...an airplane for which a load manifest must be...

2012-01-01

27

14 CFR 125.383 - Load manifest.  

Code of Federal Regulations, 2011 CFR

...Rules § 125.383 Load manifest. (a) Each...preparation and accuracy of a load manifest in duplicate...that flight; (4) The center of gravity limits; ...manifest indicating that the center of gravity is within...an airplane for which a load manifest must be...

2011-01-01

28

14 CFR 125.383 - Load manifest.  

Code of Federal Regulations, 2013 CFR

...Rules § 125.383 Load manifest. (a) Each...preparation and accuracy of a load manifest in duplicate...that flight; (4) The center of gravity limits; ...manifest indicating that the center of gravity is within...an airplane for which a load manifest must be...

2013-01-01

29

14 CFR 125.383 - Load manifest.  

Code of Federal Regulations, 2010 CFR

...Rules § 125.383 Load manifest. (a) Each...preparation and accuracy of a load manifest in duplicate...that flight; (4) The center of gravity limits; ...manifest indicating that the center of gravity is within...an airplane for which a load manifest must be...

2010-01-01

30

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

31

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. PMID:24002088

Fraidakis, M J

2013-01-01

32

[Neurological manifestations of Behçet's disease].  

PubMed

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

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

2014-02-01

33

Porphyria and its neurologic manifestations.  

PubMed

Porphyrias are rare disorders resulting from a defect in the heme biosynthetic pathway. They can produce significant disease of both the peripheral and central nervous systems, in addition to other organ systems, with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria as the subtypes associated with neurologic manifestations. The presence of a motor-predominant peripheral neuropathy (axonal predominant), accompanied by gastrointestinal distress and neuropsychiatric manifestations, should be a strong clue to the diagnosis of porphyria. Clinical confirmation can be made through evaluation of urine porphyrins during an exacerbation of disease. While hematin is helpful for acute treatment, long-term effective management requires avoidance of overstimulation of the cytochrome P450 pathway, as well as other risk factor control. PMID:24365356

Tracy, Jennifer A; Dyck, P James B

2014-01-01

34

Multisystem manifestations of mitochondrial disorders  

Microsoft Academic Search

Mitochondria are cytoplasmic organelles in eukaryotic cells that accomplish several distinct vital functions, including oxidative\\u000a phosphorylation, metabolic anaplerotic and degradative pathways, and integration of signaling for apoptosis. Impaired oxidative\\u000a phosphorylation, the common final pathway of mitochondrial metabolism, results in a variety of clinical manifestations, and\\u000a the term mitochondrial disorders is currently ascribed to (mostly) genetic diseases of the respiratory chain

Stefano Di Donato

2009-01-01

35

Cutaneous Manifestations of Lightning Injury:  

E-print Network

Background: Lightning injuries are relatively uncommon and have been a subject of awe since primitive times. It most significantly affects the cardiorespiratory, nervous, and integumentary systems. Surprisingly, cutaneous burn injuries caused by lightning are usually superficial. Objective: To present the cutaneous manifestations of lightning injuries and the sequelae of improper management. Case report: A 22-year-old woman presented with cutaneous manifestations of lightning-induced burns and bilateral upper limb gangrene after 2 months of improper treatment. She refused amputation after counseling and left the hospital. Conclusion: This is a rare case of burns with cutaneous manifestations peculiar to lightning injury. These features serve as evidence of lightning injury, when in doubt, especially in societies where superstition is rife. Education concerning the nature of lightning and proper management would improve outcome. Injuries from man-made, generated, or technical electricity have been reported for only about 150 years, but injuries from lightning surely predate written records. Over the centuries, superstitions and myths about lightning have grown. 1 Its power has been a subject of awe since primitive times. It is a natural atmospheric electrical discharge that occurs between regions of net positive and negative electric charges. 2 Lightning injuries are commoner in rural or exposed environments than in the city where high buildings have metal frames and lightning protection devices. It has been estimated that lightning strikes have a 30 % mortality rate. 3 Most significantly affected are the cardiorespiratory, nervous, and integumentary systems. 4 Skin injuries vary from cutaneous injury to that typically caused by high-voltage, commercial electricity. Injury is affected by the type of clothing, amount of moisture on the skin, and the presence of metal on the body. Burn injuries are surprisingly superficial. 2 We present this report to highlight the importance of appreciating the various cutaneous manifestations of lightning injury, including the complications of improper management.

I. Abang

2008-01-01

36

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. PMID:23606974

Murakami, Jun

2013-01-01

37

Unusual manifestations of hereditary angioedema.  

PubMed

Hereditary angioedema is a hereditary disorder transmitted as an autosomal dominant trait, characterized by reduced plasma concentration of C1 esterase inhibitor (type 1) or the presence of non functional C1 esterase inhibitor (type 2). We describe and discuss the case of a 35-year-old man who presented two unusual clinical manifestations of type 2 hereditary angioedema causing diverse emergency situations: acute abdomen and parasellar oedema. PMID:11132070

Neri, S; Ierna, D; Sfogliano, L

2000-06-01

38

Neurological manifestations of dengue virus infection  

Microsoft Academic Search

AimPaucity of studies on neurological manifestations in dengue virus infection prompted this study. We aim to correlate clinical, radiological and neurophysiological changes in dengue patients with neurological manifestations.

U. K. Misra; J. Kalita; U. K. Syam; T. N. Dhole

2006-01-01

39

Cardiac manifestations in systemic sclerosis  

PubMed Central

Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis (SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disease. In addition, cardiac complications in SSc may develop as a secondary phenomenon due to pulmonary arterial hypertension and kidney pathology. The prevalence of primary cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations, the presence of subclinical periods, the type of diagnostic tools applied, and the diversity of patient populations. When clinically manifested, cardiac involvement is thought to be an important prognostic factor. Profound microvascular disease is a pathognomonic feature of SSc, as both vasospasm and structural alterations are present. Such alterations are thought to predict macrovascular atherosclerosis over time. There are contradictory reports regarding the prevalence of atherosclerosis in SSc. According to some authors, the prevalence of atherosclerosis of the large epicardial coronary arteries is similar to that of the general population, in contrast with other rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, the level of inflammation in SSc is inferior. Thus, the atherosclerotic process may not be as aggressive and not easily detectable in smaller studies. Echocardiography (especially tissue Doppler imaging), single-photon emission computed tomography, magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies. Screening for subclinical cardiac involvement via modern, sensitive tools provides an opportunity for early diagnosis and treatment, which is of crucial importance for a positive outcome. PMID:25276300

Lambova, Sevdalina

2014-01-01

40

Medical conditions with neuropsychiatric manifestations.  

PubMed

Medical disease sometimes affects patients through neuropsychiatric manifestations. When neuropsychiatric symptoms are predominant, identifying medical disease early in the illness course is imperative because many of these conditions are reversible with appropriate treatment. A high index of suspicion is required on the part of clinicians, particularly when patients also present with physical signs or unexplained symptoms that might suggest a broader, systemic process. The processes that most commonly cause neuropsychiatric symptoms include infectious, autoimmune, endocrinologic, metabolic, and neoplastic diseases. This article focuses on the most common of these conditions, and conditions for which early diagnosis and treatment are particularly important. PMID:25134879

Isaac, Margaret L; Larson, Eric B

2014-09-01

41

Gaucher disease and bone manifestations.  

PubMed

Gaucher disease is a relatively rare metabolic disease caused by the inherited deficiency of the lysosomal enzyme glucocerebrosidase. Gaucher disease affects multiple organs, among which is the skeleton. Bone involvement occurs frequently in Gaucher disease, and is one of its most debilitating features, reducing the quality of life of patients. Bone status is an important consideration for treatment to ameliorate symptoms and reduce the risk of irreversible complications. We have conducted a systematic review of all the various aspects of Gaucher disease, focusing on different skeletal manifestations, pathophysiology of bone alterations, clinical symptoms, and current diagnostic and therapeutic approaches. PMID:25377906

Marcucci, Gemma; Zimran, Ari; Bembi, Bruno; Kanis, John; Reginster, Jean-Yves; Rizzoli, Renè; Cooper, Cyrus; Brandi, Maria Luisa

2014-12-01

42

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. PMID:23049626

Serban, AL; Udrea, GF

2012-01-01

43

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

44

Coronal manifestations of preflare activity  

NASA Technical Reports Server (NTRS)

A variety of coronal manifestations of precursors or preheating for flares are discussed. Researchers found that almost everyone with a telescope sees something before flares. Whether an all-encompassing scenario will ever be developed is not at all clear at present. The clearest example of preflare activity appears to be activated filaments and their manifestations, which presumably are signatures of a changing magnetic field. But researchers have seen two similar eruptions, one without any evidence of emerging flux (Kundu et al., 1985) and the other with colliding poles (Simon et al., 1984). While the reconnection of flux is generally agreed to be required to energize a flare, the emergence of flux from below (at least on short timescales and in compact regions) does not appear to be a necessary condition. In some cases the cancelling of magnetic flux (Martin, 1984) by horizontal motions instead may provide the trigger (Priest, 1985) Researchers found similarities and some differences between these and previous observations. The similarities, besides the frequent involvement of filaments, include compact, multiple precursors which can occur both at and near (not at) the flare site, and the association between coronal sources and activity lower in the atmosphere (i.e., transition zone and chromosphere).

Schmahl, E. J.; Webb, D. F.; Woodgate, B.; Waggett, P.; Bentley, R.; Hurford, G.; Schadee, A.; Schrijver, J.; Harrison, R.; Martens, P.

1986-01-01

45

Neurodevelopmental Manifestations of Mitochondrial Disease  

PubMed Central

Mitochondrial disease is an increasingly recognized but widely heterogeneous group of multi-systemic disorders that commonly involve severe neurodevelopmental manifestations in childhood. This article will review the presentation, genetic basis, and diagnostic evaluation of primary mitochondrial disease. Emphasis will be placed on neurodevelopmental findings that may be encountered by a Developmental Pediatrician that should provoke consideration of a mitochondrial disorder. The inheritance patterns and mechanisms by which mutations in genes located in either the nuclear or mitochondrial genomes can cause mitochondrial diseases will be discussed. A general overview of the current diagnostic evaluation that can be readily initiated by the Developmental Pediatrician will be provided, along with a summary of currently available treatment options. PMID:20814259

Falk, Marni J.

2014-01-01

46

Genital manifestations of tropical diseases  

PubMed Central

Genital symptoms in tropical countries and among returned travellers can arise from a variety of bacterial, protozoal, and helminthic infections which are not usually sexually transmitted. The symptoms may mimic classic sexually transmitted infections (STIs) by producing ulceration (for example, amoebiasis, leishmaniasis), wart-like lesions (schistosomiasis), or lesions of the upper genital tract (epididymo-orchitis caused by tuberculosis, leprosy, and brucellosis; salpingitis as a result of tuberculosis, amoebiasis, and schistosomiasis). A variety of other genital symptoms less suggestive of STI are also seen in tropical countries. These include hydrocele (seen with filariasis), which can be no less stigmatising than STI, haemospermia (seen with schistosomiasis), and hypogonadism (which may occur in lepromatous leprosy). This article deals in turn with genital manifestations of filariasis, schistosomiasis, amoebiasis, leishmaniasis, tuberculosis and leprosy and gives clinical presentation, diagnosis, and treatment. PMID:14755029

Richens, J

2004-01-01

47

[Collagen diseases with gastrointestinal manifestations].  

PubMed

Collagen vascular diseases are known to present with a diverse array of gastrointestinal manifestations. These can be classified as: 1) gastrointestinal damage due to the collagen vascular disease itself; 2) adverse events caused by pharmacotherapies; or 3) gastrointestinal infections following immunosuppression due to corticosteroid (CS) administration. The first group includes lupus enteritis and protein-losing gastroenteropathy in systemic lupus erythematosus (SLE), reflux esophagitis, chronic intestinal pseudo-obstruction, and pneumatosis cystoids intestinalis in systemic sclerosis, amyloidosis in rheumatoid arthritis, bowel ulcer and bleeding in rheumatoid vasculitis and microscopic polyangiitis, and ileocecal ulcer in Behcet disease. In particular, colonic ulcers associated with SLE represent refractory lesions resistant to CS. Analysis of reported cases showing colonic lesions with SLE (22 cases in Japan) revealed that mean duration of SLE was 9.9 years and 77% of colonic lesions were observed in the rectum and sigmoid colon. Half of the patients developed intestinal perforation or penetration, and 6 of the 11 patients with perforation died. The second group includes lesions in the small and large intestine due to nonsteroidal anti-inflammatory drugs (NSAIDs) and CSs, in addition to peptic ulcers. As perforation in CS-treated patients displays relatively high incidence with poor prognosis, careful attention to such complications is needed. The third group includes candidal esophagitis and cytomegalovirus (CMV) enteritis. Prompt diagnosis is required to prevent colonic bleeding and perforation due to CMV. PMID:15291251

Takahashi, Hiroki; Ohara, Mikiko; Imai, Kohzoh

2004-06-01

48

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

Federal Register 2010, 2011, 2012, 2013, 2014

...e-Manifest system by imposing service fees as a means to fund an information technology contract to build...following functional requirements: e-Manifest workflow, including mobile field component and handler submission;...

2013-02-20

49

[Neurologic manifestations of infectious endocarditis].  

PubMed

Thirty out of 287 patients (10.4%) admitted to hospital for infective endocarditis between December 1970 and January 1990 had neurological complications. Twenty-three patients had native valve infectious endocarditis and 7 had prosthetic valve endocarditis. The clinical features were characterized by the frequency of aortic valve involvement (23 out of 30) and other complications, especially cardiac failure (16 cases) and peripheral vascular manifestations (7 cases). The commonest organism was the staphylococcus (53% of identified organisms) but the number of negative blood cultures was high (50% of cases). The neurological complication was often the presenting symptom of the endocarditis (19 cases) but it occurred after bacteriological cure in 4 cases. The complications observed were cerebral ischemia (16 cases), cerebral haemorrhage (11 cases), coma (2 cases), and one peripheral neuropathy causing a Claude Bernard Horner syndrome. These complications presented with hemiplegia in 17 cases, a meningeal syndrome in 8 cases, a convulsion in 1 case, a Von Wallenberg syndrome in 1 case, and a Claude Bernard Horner syndrome in 1 case. Twelve patients had a transient or permanent neurological coma. Cerebral CT scan showed ischemic lesions in 7 cases and haemorrhagic lesions in 10 cases. Carotid angiography demonstrated mycotic aneurysms in 6 patients. Twelve patients died: the cause of death was neurological coma (7 cases), low cardiac output (4 cases) and haemorrhagic shock (1 case). Four patients underwent neurosurgery: 3 for clipping a mycotic aneurysm and 1 for drainage of an intracerebral haematoma. Poor prognostic factors were: coma, cardiac failure, cardiac valve prosthesis and, above all, the extent and multiplicity of the neurological lesions. The authors propose the following measures to improve the prognosis: early surgery in cases of large and/or mobile vegetations especially when the infecting organism is a staphylococcus and when a systemic embolism has occurred; routine CT scanning and/or digitised cerebral angiography in all patients with infective endocarditis to detect surgically accessible mycotic aneurysms. PMID:2012489

Hannachi, N; Béard, T; Ben Ismail, M

1991-01-01

50

Neonatal splenic rupture: an unusual manifestation  

Microsoft Academic Search

Neonatal splenic rupture is relatively rare [4] and is usually associated with a traumatic delivery [3]. The clinical manifestation are those of hemorrhage and hypovolemic shock. We present two unusual cases of spontaneous neonatal splenic rupture whose initial clinical manifestation was a hematocele of the scrotal sac. The clinical presentation, diagnostic approach, and management of such cases is discussed with

David Bader; Jorge G. Mogilner; Anna Berger; Samuel Eldar; Daniel Reich; Leonardo Siplovich

1993-01-01

51

Bone and joint manifestations of hypothyroidism  

Microsoft Academic Search

Hypothyroidism is frequently accompanied by musculoskeletal manifestationsranging from myalgias and arthralgias to true myopathy and arthritis. A case is presented in which an arthropathic process in the hip was the isolated finding in a young man who was severely hypothyroid. Previous literature on bone and joint manifestations of hypothyroidism is reviewed, with emphasis on cases where such manifestations were the

Robert M. McLean; David N. Podell

1995-01-01

52

Lupus erythematosus: systemic and cutaneous manifestations  

Microsoft Academic Search

Skin and joint involvements are the most commonly occurring manifestations of systemic lupus erythematosus. There are 3 forms of cutaneous lupus: chronic cutaneous (discoid) lupus, subacute cutaneous lupus, and acute cutaneous lupus. Joint manifestations are usually not associated with warmth of the joints and may be only associated with pain and swelling. Painful or swollen joints respond rapidly to small

Naomi Rothfield; Richard D. Sontheimer; Megan Bernstein

2006-01-01

53

Musculoskeletal Manifestations of Human Immunodeficiency Virus Infection  

Microsoft Academic Search

Human immunodeficiency virus (HIV) causes an infection characterized by a wide spectrum of clinical manifestations, including musculoskeletal conditions that have been recognized with increasing frequency in recent years. Arthralgia, usually of moderate intensity, intermittent, and oligoarticular, is the most frequent rheumatic manifestation of HIV; it occurs in approximately 35% of the cases. Knees, shoulders, and elbows are the most frequently

Luis H. Silveira; Luis J. Jara; Píndaro Martínez-Osuna; Luis R. Espinoza; Mitchel J. Seleznick

1991-01-01

54

Object manifestations for medical imaging applications.  

PubMed

As healthcare providers become increasingly decentralized and distributed, information systems support must follow suit by providing healthcare professionals with flexible, adaptable and secure views of medical data in a timely fashion. To this end, this paper introduces the notion of manifestations of medical image objects in a heterogeneous client environment. We define the fundamental building blocks of manifestations using object-oriented concepts. Examples are given of how constraints and rules can be used to govern the presentation of data within objects and for the environmental (e.g. hardware and network) and user defined factors (e.g. preferences) that act as inputs to manifestations. Using these techniques, we give several examples of user interactions with a system based on manifestations. This paper demonstrates how objects can be efficiently and effectively presented to users with various information appliances using manifestation techniques. PMID:8954236

Adam, N R; Holowczak, R D; Li, W S

1996-01-01

55

Genetic alterations in syndromes with oral manifestations  

PubMed Central

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

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

2013-01-01

56

Genetic alterations in syndromes with oral manifestations.  

PubMed

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

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

2013-11-01

57

7 CFR 905.70 - Manifest report.  

Code of Federal Regulations, 2012 CFR

...AGRICULTURAL MARKETING SERVICE (Marketing Agreements and Orders; Fruits, Vegetables, Nuts), DEPARTMENT OF AGRICULTURE ORANGES, GRAPEFRUIT, TANGERINES, AND TANGELOS GROWN IN FLORIDA Order Regulating Handling Handlers' Reports § 905.70 Manifest...

2012-01-01

58

7 CFR 905.70 - Manifest report.  

Code of Federal Regulations, 2011 CFR

...AGRICULTURAL MARKETING SERVICE (Marketing Agreements and Orders; Fruits, Vegetables, Nuts), DEPARTMENT OF AGRICULTURE ORANGES, GRAPEFRUIT, TANGERINES, AND TANGELOS GROWN IN FLORIDA Order Regulating Handling Handlers' Reports § 905.70 Manifest...

2011-01-01

59

7 CFR 905.70 - Manifest report.  

Code of Federal Regulations, 2014 CFR

...AGRICULTURAL MARKETING SERVICE (MARKETING AGREEMENTS AND ORDERS; FRUITS, VEGETABLES, NUTS), DEPARTMENT OF AGRICULTURE ORANGES, GRAPEFRUIT, TANGERINES, AND TANGELOS GROWN IN FLORIDA Order Regulating Handling Handlers' Reports § 905.70 Manifest...

2014-01-01

60

Important cutaneous manifestations of inflammatory bowel disease  

PubMed Central

Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition. PMID:16143688

Trost, L; McDonnell, J

2005-01-01

61

19 CFR 122.75 - Complete manifest.  

Code of Federal Regulations, 2010 CFR

...PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew...

2010-04-01

62

Hepatobiliary Manifestations of Inflammatory Bowel Disease  

PubMed Central

Hepatobiliary manifestations occur quite frequently in patients suffering from chronic ulcerative colitis and Crohn's disease and carry with them considerable morbidity and mortality. Although the true incidence is difficult to determine, clinically, significant hepatobiliary disease occurs in 5%–10% of patients. At the present moment, the aetiology and pathogenesis of inflammatory bowel disease and its systemic manifestations remains speculative. For those hepatobiliary manifestations that respond to therapy of the underlying bowel disease, medical and/or surgical therapy must be aggressively pursued. More urgent research is required towards understanding the underlying cause(s) of the primary bowel disease and its systemic manifestations in order to improve the overall management of this condition. PMID:10977114

Memon, Mohammed Iqbal; Memon, Breda

2000-01-01

63

Endocrine disorders and the neurologic manifestations  

PubMed Central

The nervous system and the endocrine system are closely interrelated and both involved intimately in maintaining homeostasis. Endocrine dysfunctions may lead to various neurologic manifestations such as headache, myopathy, and acute encephalopathy including coma. It is important to recognize the neurologic signs and symptoms caused by the endocrine disorders while managing endocrine disorders. This article provides an overview of the neurologic manifestations found in various endocrine disorders that affect pediatric patients. It is valuable to think about 'endocrine disorder' as a cause of the neurologic manifestations. Early diagnosis and treatment of hormonal imbalance can rapidly relieve the neurologic symptoms. Better understanding of the interaction between the endocrine system and the nervous system, combined with the knowledge about the pathophysiology of the neurologic manifestations presented in the endocrine disorders might allow earlier diagnosis and better treatment of the endocrine disorders. PMID:25654063

2014-01-01

64

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

PubMed Central

Huntington's disease (HD) is an inherited neurodegenerative disorder typified by involuntary body movements, and psychiatric and cognitive abnormalities. Many HD patients also exhibit metabolic changes including progressive weight loss and appetite dysfunction. Here we have investigated metabolic function in pre-manifest and manifest HD subjects to establish an HD subject metabolic hormonal plasma signature. Individuals at risk for HD who have had predictive genetic testing showing the cytosine-adenine-guanine (CAG) expansion causative of HD, but who do not yet present signs and symptoms sufficient for the diagnosis of manifest HD are said to be “pre-manifest.” Pre-manifest and manifest HD patients, as well as both familial and non-familial controls, were evaluated for multiple peripheral metabolism signals including circulating levels of hormones, growth factors, lipids, and cytokines. Both pre-manifest and manifest HD subjects exhibited significantly reduced levels of circulating growth factors, including growth hormone and prolactin. HD-related changes in the levels of metabolic hormones such as ghrelin, glucagon, and amylin were also observed. Total cholesterol, HDL-C, and LDL-C were significantly decreased in HD subjects. C-reactive protein was significantly elevated in pre-manifest HD subjects. The observation of metabolic alterations, even in subjects considered to be in the pre-manifest stage of HD, suggests that in addition, and prior, to overt neuronal damage, HD affects metabolic hormone secretion and energy regulation, which may shed light on pathogenesis, and provide opportunities for biomarker development. PMID:25002850

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

2014-01-01

65

Neurologic Manifestations of Childhood Rheumatic Diseases  

PubMed Central

Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of neurological manifestations in children with rheumatologic disorders is limited. This review describes the neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally. PMID:24665273

SHIARI, Reza

2012-01-01

66

Pulmonary manifestations of inflammatory bowel disease  

PubMed Central

Extraintestinal manifestations of inflammatory bowel disease (IBD) are a systemic illness that may affect up to half of all patients. Among the extraintestinal manifestations of IBD, those involving the lungs are relatively rare and often overlooked. However, there is a wide array of such manifestations, spanning from airway disease to lung parenchymal disease, thromboembolic disease, pleural disease, enteric-pulmonary fistulas, pulmonary function test abnormalities, and adverse drug reactions. The spectrum of IBD manifestations in the chest is broad, and the manifestations may mimic other diseases. Although infrequent, physicians dealing with IBD must be aware of these conditions, which are sometimes life-threatening, to avoid further health impairment of the patients and to alleviate their symptoms by prompt recognition and treatment. Knowledge of these manifestations in conjunction with pertinent clinical data is essential for establishing the correct diagnosis and treatment. The treatment of IBD-related respiratory disorders depends on the specific pattern of involvement, and in most patients, steroids are required in the initial management. Corticosteroids, both systemic and aerosolized, are the mainstay therapeutic approach, while antibiotics must also be administered in the case of infectious and suppurative processes, whose sequelae sometimes require surgical intervention. PMID:25309080

Ji, Xiao-Qing; Wang, Li-Xia; Lu, De-Gan

2014-01-01

67

8 CFR 251.1 - Arrival manifests and lists.  

Code of Federal Regulations, 2010 CFR

...2010-01-01 false Arrival manifests and lists. 251.1 Section 251.1 Aliens...REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.1 Arrival manifests and lists. (a) Vessels —(1)...

2010-01-01

68

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2010 CFR

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

2010-07-01

69

40 CFR 262.24 - Use of the electronic manifest.  

Code of Federal Regulations, 2014 CFR

...CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest ...requirement in these regulations for a generator to keep or retain a copy of each manifest...of a signed electronic manifest in the generator's account on the national...

2014-07-01

70

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2011 CFR

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

2011-07-01

71

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2012 CFR

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

2012-07-01

72

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2013 CFR

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

2013-07-01

73

40 CFR 262.23 - Use of the manifest.  

Code of Federal Regulations, 2014 CFR

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

2014-07-01

74

14 CFR 121.693 - Load manifest: All certificate holders.  

Code of Federal Regulations, 2011 CFR

... 2011-01-01 false Load manifest: All certificate...and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the...schedule that insures that the center of gravity is within...

2011-01-01

75

14 CFR 121.693 - Load manifest: All certificate holders.  

Code of Federal Regulations, 2014 CFR

... 2014-01-01 false Load manifest: All certificate...and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the...schedule that insures that the center of gravity is within...

2014-01-01

76

14 CFR 121.693 - Load manifest: All certificate holders.  

Code of Federal Regulations, 2010 CFR

... 2010-01-01 false Load manifest: All certificate...and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the...schedule that insures that the center of gravity is within...

2010-01-01

77

14 CFR 121.693 - Load manifest: All certificate holders.  

Code of Federal Regulations, 2013 CFR

... 2013-01-01 false Load manifest: All certificate...and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the...schedule that insures that the center of gravity is within...

2013-01-01

78

14 CFR 121.693 - Load manifest: All certificate holders.  

Code of Federal Regulations, 2012 CFR

... 2012-01-01 false Load manifest: All certificate...and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the...schedule that insures that the center of gravity is within...

2012-01-01

79

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. PMID:21666756

Petersen, Christine Anne; Greenlee, M. Heather West

2011-01-01

80

Unfamiliar Manifestations of Anti-tubercular Therapy.  

PubMed

Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved. PMID:24791242

Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi

2014-01-01

81

Pituitary tumors: pathophysiology, clinical manifestations and management  

Microsoft Academic Search

Pituitary tumors are frequently encountered intracranial neoplasms. They present with a variety of clinical manifestations that include symptoms and signs of excessive hormone secretion by the tumor, signs of hormone deficits by the normal pituitary gland and others related to expansion of the tumor mass and the resulting compression of surrounding structures such as the optic chiasm and cranial nerves.

B M Arafah; M P Nasrallah

2001-01-01

82

Oropharyngeal Dysphagia: neurogenic etiology and manifestation.  

PubMed

To determine the type, severity and manifestation of dysphagia in patients with neurogenic etiology. Clinical documentation was done on the different etiologies, its manifestation, assessment findings and management strategies taken for patients with neurogenic oropharyngeal dysphagia who were referred for assessment and management of dysphagia over a period of three months in a tertiary care teaching hospital. Flexible endoscopic examination was done in all the patients. The severity of dysphagia in these patients were graded based on Gugging Swallowing Screen (GUSS). A total of 53 patients with neurogenic oropharyngeal dysphagia were evaluated by an otolaryngologist and a speech language pathologist over a period of three months. The grading of severity based on GUSS for these patients were done. There were 30 patients with recurrent laryngeal nerve injury due to various etiologies, one patient with Neurofibroma-vestibular schwanoma who underwent surgical excision, 16 patients with stroke, two patients with traumatic brain injury, two patients with Parkinsonism and two patients with myasthenia gravis. The manifestation of dysphagia was mainly in the form of prolonged masticatory time, oral transit time, and increased number of swallows required for each bolus, cricopharyngeal spasms and aspiration. Among the dysphagia patients with neurogenic etiology, dysphagia is manifested with a gradual onset and is found to have a progressive course in degenerative disorders. Morbidity and mortality may be reduced with early identification and management of neurogenic dysphagia. PMID:25621266

Sebastian, Swapna; Nair, Prem G; Thomas, Philip; Tyagi, Amit Kumar

2015-03-01

83

Cutaneous Manifestations in Italian Kidney Transplant Recipients  

Microsoft Academic Search

Several cutaneous disorders may occur in organ transplant recipients. We examined the incidence and the clinical spectrum of cutaneous manifestations among kidney transplant recipients. One hundred nine patients (70 males and 39 females), aged 19 to 69 years (mean: 42.5 years), were consecutively examined as outpatients between June 2000 and August 2004. The mean interval after kidney transplantation was 61

F. Formicone; M. C. Fargnoli; F. Pisani; M. Rascente; A. Famulari; K. Peris

2005-01-01

84

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. PMID:23162309

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

2012-01-01

85

Multifocal musculoskeletal cystic tuberculosis without systemic manifestations.  

PubMed

A case of multifocal cystic tuberculosis is presented, affecting the clavicle and the iliac bones with considerable soft tissue involvement. It was found in a 34-year-old male, an immigrant from Yemen. The extensive cystic nature of both lesions and the lack of systemic manifestations despite multifocal disease are unusual. PMID:7747104

Eid, A; Chaudry, N; el-Ghoroury, M; Hawasli, A; Salot, W L; Khatib, R

1994-01-01

86

Rheumatologic manifestations of pediatric HIV infection.  

PubMed

In order to assess the frequency of rheumatologic manifestations at different stages of pediatric human immunodeficiency virus (HIV) infection, 26 HIV-infected children at any stage of infection, seen at the Children's AIDS Clinic of "La Raza" National Medical Center from January 1997 to December 1998, were studied. Rheumatologic manifestations were assessed following the criteria established by the American College of Rheumatology. Blood samples were taken for measuring CD4+ and CD8+ T cells, antinuclear antibodies (ANA), anticardiolipin (ACL) antibodies, and rheumatoid factor (RF). The results were compared to those of 25 HIV-negative children of similar ages. Rheumatologic manifestations were identified in 5 (19.2%) of 26 children. Two of whom were twin sisters with biphasic Raynaud's syndrome, and one had necrosing vasculitis of a finger, as well as lip necrosis and livedo reticularis. These patients were positive for ANA and ACL. One case each of knee arthalgias, vasculitis, and septic arthritis of the ankle were also seen. All of the rheumatologic manifestations were in advanced stages of HIV disease. These rheumatologic changes are similar to those reported for HIV-positive adults, and should be considered as part of the HIV acquired immune deficiency syndrome (AIDS) clinical spectrum in the pediatric population. PMID:11689139

Martínez-Rojano, H; Juárez Hernández, E; Ladrón De Guevara, G; del Carmen Gorbea-Robles, M

2001-10-01

87

Clinical Manifestations of Superior Semicircular Canal Dehiscence  

Microsoft Academic Search

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

Lloyd B. Minor

2005-01-01

88

Orthopeedic Manifestations of Sickle-Cell Disease  

E-print Network

Sickle-cell disease is a well-recognized clinical entity. The pathophysiology of this hemoglobinopathy has been described in detail by numerous investigators since the first case report appeared in 1910. Orthopedic manifestations of sickle-cell disease account for much of the morbidity associated

Michael H. Huo; A Gary E. Friedlaender; B; James S. Marsh

1990-01-01

89

Persistent chorea as a manifestation of thyrotoxicosis.  

PubMed Central

We report a case of persistent chorea as a manifestation of thyrotoxicosis. The chorea was severe and persisted after the patient was rendered euthyroid. Dopamine antagonists only partially suppressed the involuntary movements during the first few months. It was eventually controlled with haloperidol, but whenever she discontinues the treatment the chorea has returned during the 16 months since she first presented. PMID:3255920

Javaid, A.; Hilton, D. D.

1988-01-01

90

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

91

Hepatic angiosarcoma manifested as recurrent hemoperitoneum  

PubMed Central

Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum. PMID:18473427

Lee, Seung-Woo; Song, Chun-Young; Gi, Young-Hwa; Kang, Sang-Beom; Kim, Yon-Soo; Nam, Soon-Woo; Lee, Dong-Soo; Kim, Jong-Ok

2008-01-01

92

Head and neck manifestations of Kawasaki disease  

Microsoft Academic Search

Kawasaki disease, also known as acute infantile febrile mucocutaneous lymph node syndrome, is a self-limited vasculitic disease of infants and young children. The cause of the disease remains uncertain. Within the constellation of signs and symptoms, there are numerous otolaryngologic manifestations. The following represents the largest series of patients in the otolaryngology literature, involving 155 confirmed cases of Kawasaki disease

Adi Yoskovitch; Ted L. Tewfik; Ciarán M. Duffy; Brenda Moroz

2000-01-01

93

A gingival manifestation of histoplasmosis leading diagnosis  

PubMed Central

Histoplasmosis is a world-wide distributed deep mycosis caused by Histoplasma capsulatum which has been endemic in many countries. We present a case involving an immunocompetent man evidencing the necessity of a multidisciplinary approach and rational requisition of exams. The disease has started as a pulmonary disease mimicking tuberculosis, although the exams have been negative. Immunodiffusion test indicate histoplasmosis, not confirmed by culture of sputum. After days the patient was forwarded by a private doctor for evaluation of oral lesions at our Department of Stomatology. An incisional biopsy revealed a nonspecific granulomatous inflammation and the Grocott-Gomori methenamine silver stain identified scarce oval structures that could represent fungal yeast. Sampling oral lesions with swab, it was observed the typical growth of H. capsulatum on culture. This case highlights the importance of doctor's integration diagnosing histoplasmosis, while a wide spectrum of clinical manifestations should be expected. Oral lesions may be the critical manifestation leading diagnosis. PMID:23853464

Brazão-Silva, Marco Tullio; Mancusi, Grabriela Wagner; Bazzoun, Francine Vilar; Ishisaki, Gleyce Yakushijin; Marcucci, Marcelo

2013-01-01

94

Ocular manifestations of the autoinflammatory syndromes.  

PubMed

The autoinflammatory syndromes are rare inherited disorders characterized by recurrent attacks of multi-system inflammation caused by genetic mutations that result in abnormal upregulation of key innate immune mediators. The term autoinflammatory syndromes includes a broad variety of disorders, including cryopyrin-associated periodic syndromes (CAPS) such as neonatal onset multisystem inflammatory disease (NOMID), familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), periodic fever syndromes, including familial Mediterranean fever (FMF), TNF receptor-1 associated periodic syndrome (TRAPS), and Blau syndrome. Ocular manifestations are frequent and diverse in affected patients, and visual impairment and blindness are not uncommon sequelae of chronic active disease. Novel therapeutic interventions targeting specific pathophysiologic mechanisms have been extremely promising in the treatment of these disorders. The purpose of this article is to provide a review of these disorders with a focus on pathogenesis, clinical manifestations, ophthalmologic involvement, and available treatment options. PMID:22924780

Tarabishy, Ahmad Bakir; Hise, Amy G; Traboulsi, Elias I

2012-12-01

95

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. PMID:22518126

Lugo, Gloria; Pena, Lara; Cordido, Fernando

2012-01-01

96

The Chronic Gastrointestinal Manifestations of Chagas Disease  

PubMed Central

Chagas disease is an infectious disease caused by the protozoan Trypanosoma cruzi. The disease mainly affects the nervous system, digestive system and heart. The objective of this review is to revise the literature and summarize the main chronic gastrointestinal manifestations of Chagas disease. The chronic gastrointestinal manifestations of Chagas disease are mainly a result of enteric nervous system impairment caused by T. cruzi infection. The anatomical locations most commonly described to be affected by Chagas disease are salivary glands, esophagus, lower esophageal sphincter, stomach, small intestine, colon, gallbladder and biliary tree. Chagas disease has also been studied in association with Helicobacter pylori infection, interstitial cells of Cajal and the incidence of gastrointestinal cancer. PMID:20037711

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

2009-01-01

97

Real-life contextual manifestations of wisdom.  

PubMed

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 Confucianism, Taoism, and Buddhism as its predominant philosophies. Analysis of these interview transcripts yielded 220 wisdom incidents that fall into five categories of wisdom. Results of the present study suggests that in real life, wisdom most likely is manifested through: 1) striving for common good by helping others and contributing to society; 2) achieving and maintaining a satisfactory state of life; 3) deciding and developing life paths; 4) resolving difficult problems at work; and 5) insisting on doing the right thing when facing adversity. PMID:19266867

Yang, Shih-Ying

2008-01-01

98

A gingival manifestation of histoplasmosis leading diagnosis.  

PubMed

Histoplasmosis is a world-wide distributed deep mycosis caused by Histoplasma capsulatum which has been endemic in many countries. We present a case involving an immunocompetent man evidencing the necessity of a multidisciplinary approach and rational requisition of exams. The disease has started as a pulmonary disease mimicking tuberculosis, although the exams have been negative. Immunodiffusion test indicate histoplasmosis, not confirmed by culture of sputum. After days the patient was forwarded by a private doctor for evaluation of oral lesions at our Department of Stomatology. An incisional biopsy revealed a nonspecific granulomatous inflammation and the Grocott-Gomori methenamine silver stain identified scarce oval structures that could represent fungal yeast. Sampling oral lesions with swab, it was observed the typical growth of H. capsulatum on culture. This case highlights the importance of doctor's integration diagnosing histoplasmosis, while a wide spectrum of clinical manifestations should be expected. Oral lesions may be the critical manifestation leading diagnosis. PMID:23853464

Brazão-Silva, Marco Tullio; Mancusi, Grabriela Wagner; Bazzoun, Francine Vilar; Ishisaki, Gleyce Yakushijin; Marcucci, Marcelo

2013-01-01

99

The Melologue and Its Latin American Manifestations  

E-print Network

FALL 1981 31 The Melologue and its Latin American Manifestations Duane Rhoades Toward the end of the eighteenth century and following the precedent set in Jean Jacques Rousseau's Pygmalion,1 Tomás de Iriarte, with his Guzman el bueno (1789... of the popularity of Rousseau's scene lyrique lay in the novel use of music and gesture as vehicles for intensifying the emotional effect of declamation.2 A practical musician, Iriarte's attention was immediately captivated by this hybrid French piece which...

Rhoades, Duane

1981-10-01

100

Musculoskeletal manifestations in patients with malignant disease  

Microsoft Academic Search

To detect and describe the incidence of musculoskeletal manifestations in different malignant diseases as well as their relation\\u000a to the treatment received whether by chemotherapy or radiation therapy. Sixty patients with different malignant diseases were\\u000a included in this study, 45 with solid tumors and 15 patients with hematological malignancy. The mean age was 46.55?±?11.04 years\\u000a and the mean disease duration was

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

2010-01-01

101

Pediatric manifestations of Hashimoto’s encephalopathy  

Microsoft Academic Search

Hashimoto’s encephalopathy is a steroid-responsive encephalopathy associated with elevated blood concentrations of antithyroid antibodies. The patients are usually euthyroid or mildly hypothyroid. The authors report two pediatric patients with Hashimoto’s encephalopathy and review the literature. The clinical picture in adolescents, as with adults, is pleomorphic but frequently associated with seizures, confusion, and hallucinations. Alternatively, progressive cognitive decline manifested by a

Elza Vasconcellos; Jesús Eric Piña-Garza; Toufic Fakhoury; Gerald M Fenichel

1999-01-01

102

Cutaneous manifestations associated with rheumatoid arthritis  

Microsoft Academic Search

Rheumatoid arthritis presents various cutaneous manifestations, either specific or nonspecific skin features, which are induced\\u000a by the activation of inflammatory cells (neutrophils, lymphocytes, macrophages), vasculopathy, vasculitis, acral deformity,\\u000a drugs, and so on. These include (1) specific findings, (2) findings due to vascular impairment, (3) findings due to immune\\u000a dysfunction, (4) characteristic neutrophilic conditions, and (5) miscellaneous conditions. On the other

T. Yamamoto

2009-01-01

103

Hematological manifestations of congenital cytomegalovirus infection  

Microsoft Academic Search

Nine infants suffering from congenital cytomegalovirus infection were studied for clinical features with special reference\\u000a to hematological manifestations. The mean age of appearance of first clinical symptom of disease was 14 days and mean age\\u000a at presentation was 58 days. Significant pallor, thrombocytopenia and evidence of hemolysis were present in 8 (89%), 4 (44%)\\u000a and 4 (44%) patients resepctively out

Harish Kumar Pemde; S. K. Kabra; Rajiv Agarwal; Yogesh Jain; V. Seth

1995-01-01

104

Peripheral Nervous System Manifestations of Infectious Diseases  

PubMed Central

Infectious causes of peripheral nervous system (PNS) disease are underrecognized but potentially treatable. Heightened awareness educed by advanced understanding of the presentations and management of these infections can aid diagnosis and facilitate treatment. In this review, we discuss the clinical manifestations, diagnosis, and treatment of common bacterial, viral, and parasitic infections that affect the PNS. We additionally detail PNS side effects of some frequently used antimicrobial agents. PMID:25360209

Brizzi, Kate T.

2014-01-01

105

Cutaneous Manifestations of Common Liver Diseases  

PubMed Central

Skin functions as a window to our overall health and a number of systemic diseases result in various cutaneous changes. Knowledge of these manifestations helps in suspecting an underlying systemic illness. Cutaneous abnormalities are quite common in patients with liver diseases and this article aims to focus on these dermatoses. Cutaneous manifestations seen in patients with liver disease though common are nonspecific. They can also be seen in patients without liver diseases and generally do not indicate about a specific underlying hepatic disorder. The presence of a constellation of signs and symptoms is more useful in pointing toward an underlying hepatobiliary condition. The commonest symptom in patients with liver disease is pruritus which is often protracted and disabling. Other common features include spider angiomas, palmar erythema, paper money skin, xanthelasmas, pigmentary changes, and nutritional deficiencies. In this article, first the common cutaneous manifestations that may be associated with liver disorders are discussed and then common liver diseases with their specific cutaneous findings are discussed. Cutaneous abnormalities may be the first clue to the underlying liver disease. Identifying them is crucial for early diagnosis and better management.

Dogra, Sunil; Jindal, Rashmi

2012-01-01

106

Superimposed segmental manifestation of polygenic skin disorders.  

PubMed

In common acquired skin disorders with a polygenic background such as psoriasis, a linear or otherwise segmental arrangement may sometimes be noted. The segmental involvement tends to be rather severe and may be associated with milder, nonsegmental lesions of the same disorder. Such cases may be best explained by an early postzygotic event in the form of loss of heterozygosity involving one of the genes that predispose to the disorder. The following pertinent examples are reviewed in this article: psoriasis vulgaris, pustular psoriasis, atopic dermatitis, lichen planus, systemic lupus erythematosus, pemphigus vulgaris, vitiligo, graft-versus-host disease, granuloma annulare, erythema multiforme, and drug eruption to ibuprofen. Such cases should not be categorized as a "type 2 segmental manifestation" because this term exclusively applies to monogenic traits, whereas in polygenic disorders the more descriptive term "superimposed segmental manifestation" seems appropriate. The concept of early loss of heterozygosity offers a plausible explanation as to why: the segmental involvement tends to appear at a rather young age and often precedes the development of milder, nonsegmental lesions of the same disorder; the segmental lesions are notoriously difficult to treat; and family members may show the disorder in its nonsegmental form. On the other hand, the theory of isolated versus superimposed segmental manifestation may help to elucidate the origin of polygenic skin disorders at the molecular level. PMID:17870433

Happle, Rudolf

2007-10-01

107

Phenotypic and dermatological manifestations in Down Syndrome.  

PubMed

Down syndrome (DS) is associated with various uncommon dermatological disorders and increased frequency of some common dermatoses. This study was conducted over a 2-year period to evaluate the frequency of phenotypic and dermatologic manifestations in patients with Down syndrome in south India. The most common phenotypic manifestations that characterize DS include the epicanthic fold (93.7%), brachicephaly (90.6%), flat nasal bridge (84.2%), upward angle of eyes (83.2%), wide gap between first and second toe (81.1%), clinodactyly (77.9%), small nose (74.7%), short broad neck (72.6%), single palmar crease (61.1%), increased nuchal skin fold (61.1%), and fissured tongue (52.6%). The most common dermatological manifestation seen in patients with DS were lichenification, xerosis, dental anomaly, fine, sparse hair, and delayed dentition. Alopecia areata was seen in 9.4 percent of patients and tended to be severe. Infections were relatively less common in our study. Our study has highlighted many phenotypic features and dermatoses, which may help provide better care for patients and counseling to the families. PMID:21382286

Sureshbabu, Rengasamy; Kumari, Rashmi; Ranugha, Subramaniam; Sathyamoorthy, Ramanathan; Udayashankar, Carounanidy; Oudeacoumar, Paquirissamy

2011-01-01

108

[Chronic polyarthritis as isolated manifestation of toxocariasis.  

PubMed

Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or T. cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of cases, generally with acute duration (less than 6 weeks). However, to our knowledge, chronic polyarthritis, as the isolated presentation of Toxocara infection, was not reported. One of 5,809 patients that was followed up at our service (0.017%) had chronic polyarthritis as the single manifestation of toxocariasis and was described herein. A 3-year-old girl was referred to our service with severe painful chronic polyarthritis for a period longer than 10 weeks and morning stiffness of 30minutes. Dog contact exposure history in the recreational areas of neighborhood was reported. Her exams showed high levels of eosinophils in peripheral blood (29%), bone marrow aspirate revealed marked eosinophilia (32%) and Toxocara enzyme-linked immunosorbent assay (Elisa) was positive (1:1,280). She was treated with paracetamol (40 mg/kg/day) and thiabendazole (25 mg/kg/day) for 10 days, and all manifestations reduced. After eight months of follow-up, she was on clinical and laboratorial remission. In conclusion, we described a case of chronic polyarthritis, as isolated manifestation of toxocariasis, mimicking juvenile idiopathic arthritis and leukemia. Importantly, this zoonosis should be considered in patients with arthritis and eosinophilia. PMID:25435169

Viola, Gabriela R; Giacomin, Maria Fernanda A; França, Camila M P; Sallum, Adriana M E; Jacob, Cristina M A; Silva, Clovis A

2014-10-16

109

19 CFR 122.116 - Identification of manifest sheets.  

Code of Federal Regulations, 2010 CFR

... AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM...given shall be used by the airline to identify all copies of the transit air cargo manifest. All...from the port of arrival as transit air...

2010-04-01

110

Gene Therapy for Neurologic Manifestations of Mucopolysaccharidoses  

PubMed Central

Introduction Mucopolysaccharidoses are a family of lysosomal disorders caused by mutations in genes that encode enzymes involved in the catabolism of glycoaminoglycans. These mutations affect multiple organ systems and can be particularly deleterious to the nervous system. At the present time, enzyme replacement therapy and hematopoietic stem-cell therapy are used to treat patients with different forms of these disorders. However, to a great extent the nervous system is not adequately responsive to current therapeutic approaches. Areas Covered Recent advances in gene therapy show great promise for treating mucopolysaccharidoses. This article reviews the current state of the art for routes of delivery in developing genetic therapies for treating the neurologic manifestations of mucopolysaccharidoses. Expert Opinion Gene therapy for treating neurological manifestations of mucopolysaccharidoses can be achieved by intraventricular, intrathecal, intranasal, and systemic administration. The intraventricular route of administration appears to provide the most wide-spread distribution of gene therapy vectors to the brain. The intrathecal route of delivery results in predominant distribution to the caudal areas of the brain while the intranasal route of delivery results in good distribution to the rostral areas of brain. The systemic route of delivery via intravenous delivery can also achieve wide spread delivery to the CNS, however, the distribution to the brain is greatly dependent on the vector system. Intravenous delivery using lentiviral vectors appear to be less effective than adeno-associated viral (AAV) vectors. Moreover, some subtypes of AAV vectors are more effective than others in crossing the blood-brain-barrier. In summary, the recent advances in gene vector technology and routes of delivery to the CNS will facilitate the clinical translation of gene therapy for the treatment of the neurological manifestations of mucopolysaccharidoses. PMID:25510418

Wolf, Daniel A.; Banerjee, Sharbani; Hackett, Perry B.; Whitley, Chester B.; McIvor, R. Scott; Low, Walter C.

2015-01-01

111

[Lipoprotein(a): influence on cardiovascular manifestation].  

PubMed

The clinical relevance of lipoprotein(a) (Lp(a)) as a cardiovascular risk factor is currently underestimated. The aim of our study was to assess the influence of increased Lp(a) values on the development and severity of coronary artery disease (CAD).In our retrospective analysis of 31,274 patients, who were hospitalized for the first time, we compared patients with isolated increased Lp(a) (> 110 mg/dl) and normal Lp(a) (< 30 mg/dl), with increased Lp(a) concentrations (30-60 mg/dl, 61-90 mg/dl, 91-110 mg/dl), and in a third analysis with additionally increased LDL cholesterol and HbA1c values.Patients with high Lp(a) levels showed a significantly higher incidence of advanced CAD with a three-vessel disease being present in 50.2 vs. 25.1 %. Patients with high Lp(a) levels had a significantly more frequent history of myocardial infarction (34.6 vs. 16.6 %, p < 0.001), surgical myocardial revascularization (40.8 vs. 20.8 %, p < 0.001) and percutaneous coronary intervention (55.3 vs. 33.6 %, p < 0.001). In addition, there was a marked difference in gender to the disadvantage of male patients regarding development and severity of CAD. CAD risk (Odds ratio) was increased 5.5-fold in patients with Lp(a) ? 110 mg/dl. Additionally elevated LDL and HbA1c levels were not associated with increased manifestation and severity of CAD.High Lp(a) concentration leads to an increased manifestation and severity of coronary artery disease. Additional risk factors do not aggravate manifestation of CAD. PMID:25666917

Mellwig, K-P; Schatton, C; Biermann, B; Kottmann, T; Horstkotte, D; van Buuren, F

2015-04-01

112

Cutaneous manifestation of giardiasis - case report.  

PubMed

Giardia intestinalis is a protozoan parasiting the gastrointestinal tract of vertebrate hosts widely distributed throughout the world. Patients with giardiasis are usually asymptomatic but the presence of the parasite may lead to a variety of clinical manifestations, including skin lesions. In this report we present a case of a 31-year-old female patient with skin lesions of granuloma annulare type confirmed by a skin biopsy, who was diagnosed with giardiasis. The clearance of the skin lesions after antiparasite treatment seems to support correlation between dermatological symptoms and infection with Giardia intestinalis . PMID:16457489

Pietrzak, Aldona; Chodorowska, Grazyna; Urban, Janusz; Bogucka, Violetta; Dybiec, Ewa

2005-01-01

113

Catatonia: An Unusual Manifestation of Wilson's Disease.  

PubMed

Wilson's disease, characterized by abnormal copper accumulation in the human body, may present with psychiatric manifestations in about one-fifth of patients. The authors report a patient with Wilson's disease who initially presented with acute psychosis and later developed catatonic symptoms. The atypical presentation led to a delay in diagnosis and institution of appropriate treatment. Wilson's disease can be ruled out in all young patients presenting with psychiatric symptoms for the first time by screening for a Kayser-Fleischer ring. PMID:25716489

Basu, Aniruddha; Thanapal, Sivakumar; Sood, Mamta; Khandelwal, Sudhir K

2015-01-01

114

Dermatologic manifestations of diabetes mellitus: a review.  

PubMed

Diabetes mellitus affects every organ of the body including the skin. Certain skin manifestations of diabetes are considered cutaneous markers of the disease, whereas others are nonspecific conditions that occur more frequently among individuals with diabetes compared with the general population. Diabetic patients have an increased susceptibility to some bacterial and fungal skin infections, which account, in part, for poor healing. Skin complications of diabetes provide clues to current and past metabolic status. Recognition of cutaneous markers may slow disease progression and ultimately improve the overall prognosis by enabling earlier diagnosis and treatment. PMID:24286954

Murphy-Chutorian, Blair; Han, George; Cohen, Steven R

2013-12-01

115

Manifestation of optical activity in different materials  

NASA Astrophysics Data System (ADS)

Various manifestations of optical activity (OA) in crystals and organic materials are considered. Examples of optically active enantiomorphic and nonenantiomorphic crystals of 18 symmetry classes are presented. The OA of enantiomorphic organic materials as components of living nature (amino acids, sugars, and proteins) is analyzed. Questions related to the origin of life on earth are considered. Examples of differences in the enantiomers of drugs are shown. The consequences of replacing conventional left-handed amino acids with additionally right-handed amino acids for living organisms are indicated.

Konstantinova, A. F.; Golovina, T. G.; Konstantinov, K. K.

2014-07-01

116

19 CFR 122.116 - Identification of manifest sheets.  

Code of Federal Regulations, 2012 CFR

...2012-04-01 2012-04-01 false Identification of manifest sheets. 122.116 Section 122.116 Customs Duties U...TACM) Procedures § 122.116 Identification of manifest sheets. When the original cargo manifest for the aircraft on...

2012-04-01

117

19 CFR 122.116 - Identification of manifest sheets.  

Code of Federal Regulations, 2011 CFR

...2011-04-01 2011-04-01 false Identification of manifest sheets. 122.116 Section 122.116 Customs Duties U...TACM) Procedures § 122.116 Identification of manifest sheets. When the original cargo manifest for the aircraft on...

2011-04-01

118

19 CFR 122.116 - Identification of manifest sheets.  

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false Identification of manifest sheets. 122.116 Section 122.116 Customs Duties U...TACM) Procedures § 122.116 Identification of manifest sheets. When the original cargo manifest for the aircraft on...

2013-04-01

119

19 CFR 122.116 - Identification of manifest sheets.  

Code of Federal Regulations, 2014 CFR

...2014-04-01 2014-04-01 false Identification of manifest sheets. 122.116 Section 122.116 Customs Duties U...TACM) Procedures § 122.116 Identification of manifest sheets. When the original cargo manifest for the aircraft on...

2014-04-01

120

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2010 CFR

...2010-04-01 2010-04-01 false In-transit manifest. 123.22 Section 123.22...RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required....

2010-04-01

121

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2014 CFR

...2014-04-01 2014-04-01 false In-transit manifest. 123.22 Section 123.22...RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required....

2014-04-01

122

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false In-transit manifest. 123.22 Section 123.22...RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required....

2013-04-01

123

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2012 CFR

...2012-04-01 2012-04-01 false In-transit manifest. 123.22 Section 123.22...RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required....

2012-04-01

124

19 CFR 123.22 - In-transit manifest.  

Code of Federal Regulations, 2011 CFR

...2011-04-01 2011-04-01 false In-transit manifest. 123.22 Section 123.22...RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required....

2011-04-01

125

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

126

Hematologic manifestations of Helicobacter pylori infection  

PubMed Central

Helicobacter pylori (H. pylori) is the most common infection in humans, with a marked disparity between developed and developing countries. Although H. pylori infections are asymptomatic in most infected individuals, they are intimately related to malignant gastric conditions such as gastric cancer and gastric mucosa-associated lymphoid tissue (MALT) lymphoma and to benign diseases such as gastritis and duodenal and gastric peptic ulcers. Since it was learned that bacteria could colonize the gastric mucosa, there have been reports in the medical literature of over 50 extragastric manifestations involving a variety medical areas of specialization. These areas include cardiology, dermatology, endocrinology, gynecology and obstetrics, hematology, pneumology, odontology, ophthalmology, otorhinolaryngology and pediatrics, and they encompass conditions with a range of clear evidence between the H. pylori infection and development of the disease. This literature review covers extragastric manifestations of H. pylori infection in the hematology field. It focuses on conditions that are included in international consensus and management guides for H. pylori infection, specifically iron deficiency, vitamin B12 (cobalamin) deficiency, immune thrombocytopenia, and MALT lymphoma. In addition, there is discussion of other conditions that are not included in international consensus and management guides on H. pylori, including auto-immune neutropenia, antiphospholipid syndrome, plasma cell dyscrasias, and other hematologic diseases. PMID:25278680

Campuzano-Maya, Germán

2014-01-01

127

Classifying functional manifestations of ectodermal dysplasias.  

PubMed

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

Simeonsson, Rune J

2009-09-01

128

Neurologic manifestations of inflammatory bowel diseases.  

PubMed

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

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

2014-01-01

129

Neurological manifestations in patients with antiphospholipid syndrome  

PubMed Central

Background Anti-phospholipids syndrome (APS) is considered a non inflammatory auto-immune disease with a significant thrombophilic risk with varied clinical manifestations. The purpose of the current study was to investigate the frequency of thrombotic and non-thrombotic events in patients with APS. Methods In this retrospective study, 102 definite APS subjects were recruited (2007-2011) at Alzahra Hospital, Isfahan, Iran. The patients were referred to Multiple Sclerosis Clinic with the diagnosis of definite APS according to 2006 Sydney's criteria. Disorders associated with APS such as pregnancy complication, vascular thrombosis and livedo reticularis (LR) were assessed. Neurological signs and symptoms such as cognitive dysfunction were recorded. Data analyses were performed using SPSS software and P < 0.05 were considered to be statistically significant. Results Our findings showed that majority of female gender, higher rate of ischemic thrombotic stroke and high miscarriage lied in a large number of APS patients. Conclusion Overall recurrent miscarriage is a common complication among (antiphospholidpid antibody) aPL patients. Furthermore, ischemic stroke is the second common neurological manifestations of APS patients. PMID:24250929

Etemadifar, Masoud; Tahani, Soheil; Toghianifar, Nafiseh; Rahaimi, Marzieh; Eskandari, Nahid

2013-01-01

130

Neurologic manifestations of pediatric chikungunya infection.  

PubMed

Chikungunya virus, a mosquito-borne arbovirus, was responsible for a massive epidemic in La Réunion Island during 2005 to 2006. The disease is usually benign, but neurologic involvement, with sometimes fatal outcome, has been described. We report a retrospective hospital-based pediatric series of 30 children (23 boys and 7 girls) who presented neurologic manifestations of chikungunya such as encephalitis (n = 12), febrile seizures (n = 10), meningeal syndrome (n = 4), and acute encephalopathy (n = 4). Cerebrospinal fluid biological and cytological analyses (n = 23) were unremarkable except for 1 case of acute disseminated encephalomyelitis. The presence of viral genome in cerebrospinal fluid was inconstantly positive. Brain magnetic resonance imaging (MRI) scans (n = 14) were abnormal in 5 cases. Electroencephalography was nonspecific. Two patients died. At discharge and 6 months later, 5 children had neurologic sequelae. Patients with initial severe neurologic presentation and having pathological brain MRI had more sequelae or fatal disease. PMID:18287573

Robin, Stéphanie; Ramful, Duksha; Le Seach', Florence; Jaffar-Bandjee, Marie-Christine; Rigou, Gérald; Alessandri, Jean-Luc

2008-09-01

131

Coronary artery manifestations of fibromuscular dysplasia.  

PubMed

Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic "string of beads" that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies. PMID:25190240

Michelis, Katherine C; Olin, Jeffrey W; Kadian-Dodov, Daniella; d'Escamard, Valentina; Kovacic, Jason C

2014-09-01

132

Ocular manifestations in porphyria cutanea tarda.  

PubMed

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

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

2014-01-01

133

Cutaneous manifestations of disease in athletes.  

PubMed

Various means of skin injury in athletes are examined, supplementing those discussed in Cutaneous Manifestations of Disease Part 1 (November/ December). Skin injury due to mechanical means such as corns, calluses, talon noir (calcaneal petechiae), tennis toe, joggers nipples, and piezogenic pedal papules will be discussed followed by a thorough discussion of environmental means of skin injury. There are a variety of ways that the environment may affect athletes both during competition and years after the competition is finished. The discussion will conclude with a look at performance-enhancing drugs and their effect on an athletes skin, and how the practitioner can better appreciate and perhaps prevent the long-term sequelae of drug abuse. PMID:14673322

Bender, Thomas W

2003-01-01

134

Imaging Manifestations of Mediastinal Fat Necrosis  

PubMed Central

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

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

2013-01-01

135

[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

2013-09-01

136

Diabetic neuropathy: Clinical manifestations and current treatments  

PubMed Central

Diabetic peripheral neuropathy is a prevalent, disabling condition. The most common manifestation is a distal symmetric polyneuropathy (DSP), but many patterns of nerve injury can occur. Currently, the only effective treatments are glucose control and pain management. While glucose control dramatically decreases the development of neuropathy in those with type 1 diabetes, the effect is likely much smaller in those with type 2 diabetes. High levels of evidence support the use of certain anticonvulsants and antidepressants for pain management in diabetic peripheral neuropathy. However, the lack of disease modifying therapies for diabetic DSP makes the identification of new modifiable risk factors essential. Intriguingly, growing evidence supports an association between metabolic syndrome components, including pre-diabetes, and neuropathy. Future studies are needed to further explore this relationship with implications for new treatments for this common disease. PMID:22608666

Callaghan, Brian C.; Cheng, Hsinlin; Stables, Catherine L.; Smith, Andrea L.; Feldman, Eva L.

2014-01-01

137

Neurologic manifestations of chronic methamphetamine abuse  

PubMed Central

Summary Chronic methamphetamine abuse has devastating effects on the central nervous system. The degree to which addicts will tolerate the dysfunction in the way they think, feel, move, and even look, is a powerful testimony to the addictive properties of this drug. While the mechanisms behind these disorders are complex, at their heart they involve the recurring increase in the concentrations of central monoamines with subsequent dysfunction in dopaminergic neurotransmission. The mainstay of treatment for the problems associated with chronic methamphetamine abuse is abstinence. However, by recognizing the manifestations of chronic abuse, clinicians will be better able to help their patients get treatment for their addiction and to deal with the neurologic complications related to chronic abuse. PMID:21803215

Rusyniak, Daniel E.

2011-01-01

138

Rheumatological manifestations in inflammatory bowel disease  

PubMed Central

Rheumatological manifestations in inflammatory bowel disease (IBD) are frequent and include peripheral arthritis, axial involvement and peripheral enthesitis. Secondary osteoporosis and hypertrophic osteoarthropathy may also occur. Complications of IBD (e.g. septic arthritis) must be distinguished from sterile inflammation. Adverse effects of corticosteroid treatment, such as osteonecrosis, may also affect joints. Axial involvement ranges from low back pain to true ankylosing spondylitis. Human leukocyte antigen B27 is associated with axial involvement of IBD. Peripheral arthritis has been classified into two types. Type I is a pauciarticular, asymmetric usually non destructive arthritis affecting large joints and is usually associated with active bowel disease. Type II is a polyarthritis affecting small joints and tends to run a course independent of the bowel disease. Treatment of joint symptoms in IBD include sulphasalazine, azathioprine, methotrexate and glucocorticoids. Anti-tumor necrosis factor antibodies are effective in treating resistant or complicated Crohn’s disease as well as peripheral arthritis and axial involvement. PMID:24713717

Voulgari, Paraskevi V.

2011-01-01

139

Secondary Syphilis: Uncommon Manifestations a Common Disease  

PubMed Central

Reports of cases of primary and secondary syphilis are increasing in the United States, particularly in urban areas and among homosexual men. While primary syphilis poses little diagnostic difficulty, many physicians are unfamiliar with the multisystem nature of secondary lues. Patients who have secondary syphilis commonly present with systemic signs, skin rash, mucous membrane lesions and generalized adenopathy. Less commonly, secondary syphilis may occur as acute meningitis, sensorineural hearing loss, iritis, anterior uveitis, optic neuritis, Bell's palsy, gastropathy, proctitis, hepatitis, pulmonary infiltration, nephrotic syndrome, glomerulonephritis, periostitis, tenosynovitis and polyarthritis. The diagnosis of secondary syphilis is easily confirmed. Its various manifestations are readily treated with penicillin and, if treated early, are entirely reversible. Two recent cases of secondary syphilis, one presenting as nephrotic syndrome and one as chorioretinitis and ptosis, illustrate the usual and unusual features of this common infection. Images PMID:6702190

McPhee, Stephen J.

1984-01-01

140

Brucella arteritis: clinical manifestations, treatment, and prognosis.  

PubMed

Brucellosis is the most common bacterial zoonosis, and causes a considerable burden of disease in endemic countries. Cardiovascular involvement is the main cause of mortality due to infection with Brucella spp, and most commonly manifests as endocarditis, peripheral and cerebrovascular aneurysms, or arterial and venous thromboses. We report a case of brucellosis presenting as bacteraemia and aortic endarteritis 18 years after the last known exposure to risk factors for brucella infection. The patient was treated with doxycycline, rifampicin, and gentamicin, and underwent surgical repair of a penetrating aortic ulcer, with a good clinical recovery. We review the signs and symptoms, diagnostic approach, prognosis, and treatment of brucella arteritis. We draw attention to the absence of consensus about the optimum therapy for vascular brucellosis, and the urgent need for additional studies and renewed scientific interest in this major pathogen. PMID:24480149

Herrick, Jesica A; Lederman, Robert J; Sullivan, Brigit; Powers, John H; Palmore, Tara N

2014-06-01

141

Ophthalmic manifestations of inherited neurodegenerative disorders.  

PubMed

Ophthalmic findings are common features of neurodegenerative disorders and, in addition to being clinically important, have emerged as potentially useful biomarkers of disease progression in several conditions. Clinically, these visual system abnormalities can be a clue to diagnosis, as well as being a prominent cause of disability in affected patients. In this Review, we describe the various afferent visual system and other ophthalmic features of inherited neurodegenerative disorders, including the muscular dystrophies, Friedreich ataxia, the spinocerebellar ataxias, hereditary spastic paraplegia, Charcot-Marie-Tooth disease, and other conditions. We focus on the expanding role of optical coherence tomography in diagnostic imaging of the retina and optic nerve head, and the possible use of ophthalmic findings as biomarkers of disease severity in hereditary neurodegenerative disorders. In addition, we discuss the ophthalmic manifestations and treatment implications of mitochondrial dysfunction, which is a feature of many inherited neurodegenerative diseases. PMID:24840976

Kersten, Hannah M; Roxburgh, Richard H; Danesh-Meyer, Helen V

2014-06-01

142

Unusual manifestation of bronchiolitis obliterans organizing pneumonia.  

PubMed

Bronchiolitis obliterans organizing pneumonia (BOOP) is an uncommon fibrotic lung disease characterized by involvement of the small conducting airways. BOOP has a wide spectrum of radiologic and clinical features. Usually, it appears radiologically as multiple alveolar patchy areas of consolidation. However, different presentations have also been described. We report two cases of solitary masses of the lung that preoperatively presented as malignant lesions but were subsequently diagnosed as bronchiolitis obliterans organizing pneumonia. One of the cases of BOOP was probably secondary to suppuration of a hydatid cyst. Complicated hydatid cyst may give rise to various clinical manifestations and may present radiologically as a solid lung mass. The radiologic findings of pulmonary ruptured or complicated hydatid cyst may resemble primary lung tumor. Open surgery can be required for not only for diagnosis but also for treatment of solitary BOOP. A review of the literature is also presented. PMID:18807607

Kilic, D; Findikcioglu, A; Kocer, E; Hatipoglu, A

2008-01-01

143

Reading Like a Historian: Manifest Destiny  

NSDL National Science Digital Library

In this lesson, students analyze maps, art, and primary source documents in an effort to answer the central historical question: How did Americans justify westward expansion? To begin the lesson, students will examine a painting entitled “American Progress.” Students will compare 2 maps of the U.S.: a political map from 1872 and an electoral map from 1816. Next, students examine another 1816 map; the map is unusual in that it depicts the U.S. stretching to the Pacific—decades before this actually happened! Students will read 2 passages by John O’Sullivan, coiner of the phrase “Manifest Destiny,” and answer guiding questions. A final class discussion reviews students’ answers and touches on the subject of American Exceptionalism.

Stanford History Education Group

2012-10-01

144

Multiple Endocrine Neoplasia Type 1: Clinical Manifestations and Management  

Microsoft Academic Search

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

Anathea C. Powell; Steven K. Libutti

145

Unusual manifestations of a common gastrointestinal disorder.  

PubMed

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

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

2014-02-01

146

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

147

Simulation of the regional manifestation of asthma.  

PubMed

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

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

2004-05-01

148

Monogenic autoinflammatory diseases: concept and clinical manifestations.  

PubMed

The objective of this review is to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary "periodic fever syndromes", familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) and 6. very rare conditions presenting with autoinflammation and immunodeficiency. PMID:23711932

Almeida de Jesus, Adriana; Goldbach-Mansky, Raphaela

2013-06-01

149

Monogenic Autoinflammatory Diseases: Concept And Clinical Manifestations  

PubMed Central

The objectives of this review are to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary “periodic fever syndromes”, familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) 6. very rare conditions presenting with autoinflammation and immunodeficiency. PMID:23711932

De Jesus, Adriana Almeida; Goldbach-Mansky, Raphaela

2013-01-01

150

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

151

Otorhinolaryngological manifestations in granulomatosis with polyangiitis (Wegener's).  

PubMed

Granulomatosis with polyangiitis (Wegener's, GPA) is an uncommon disease of unknown etiology classically involves the ELK triad of the ear, nose, throat (E), lungs (L) and kidneys (K) with necrotizing granulomatous inflammation and vasculitis. Most of the initial symptoms begin in the head and neck region with a wide spectrum of involvement of any site ranging from the nasal septum, paranasal sinuses, oral mucosa, larynx and even the external, middle and internal ear. Diagnosis may be delayed because the onset is heterogeneous and sometimes limited to one organ. The pathologic findings of a characteristic inflammatory reaction pattern, and the serum findings of elevated antineutrophil cytoplasmic antibodies can help to establish the diagnosis. The differentiation from other conditions that mimic GPA such as lymphoma and infections is of critical importance to initiate appropriate treatment. Treatment of the underlying disease is medical with the use of immunosuppressive agents and will not be reviewed here. This review focuses on the otorhinolaryngologic manifestation and complication of GPA as well as their surgical management and specifies the role of the otorhinolaryngologist as an integral member of the multidisciplinary care team for patients with GPA. PMID:22940553

Trimarchi, Matteo; Sinico, Renato Alberto; Teggi, Roberto; Bussi, Mario; Specks, Ulrich; Meroni, Pier Luigi

2013-02-01

152

Neurologic Manifestations of Chronic Methamphetamine Abuse  

PubMed Central

COMMENTARY ON METHAMPHETAMINE ABUSE FOR PSYCHIATRIC PRACTICE Every decade seems to have its own unique drug problem. The 1970s had hallucinogens, the 1980s had crack cocaine, the 1990s had designer drugs, the 2000s had methamphetamine (Meth), and in the 2010s we are dealing with the scourge of prescription drug abuse. While each of these drug epidemics has distinctive problems and history, the one with perhaps the greatest impact on the practice of Psychiatry is Meth. By increasing the extracellular concentrations of dopamine while slowly damaging the dopaminergic neurotransmission, Meth is a powerfully addictive drug whose chronic use preferentially causes psychiatric complications. Chronic Meth users have deficits in memory and executive functioning as well as higher rates of anxiety, depression, and most notably psychosis. It is because of addiction and chronic psychosis from Meth abuse that the Meth user is most likely to come to the attention of the practicing Psychiatrist/Psychologist. Understanding the chronic neurologic manifestations of Meth abuse will better arm practitioners with the diagnostic and therapeutic tools needed to make the Meth epidemic one of historical interest only. PMID:23688691

Rusyniak, Daniel E.

2013-01-01

153

Observational Manifestation of Chaos in Astrophysical Objects  

NASA Astrophysics Data System (ADS)

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

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

2002-12-01

154

Neuro-ophthalmic manifestations of prematurity.  

PubMed

Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children. PMID:25449932

Chhablani, Preeti Patil; Kekunnaya, Ramesh

2014-10-01

155

Behavioural manifestations of anabolic steroid use.  

PubMed

The use of anabolic androgenic steroids (AAS) for gains in strength and muscle mass is relatively common among certain subpopulations, including athletes, bodybuilders, adolescents and young adults. Adverse physical effects associated with steroid abuse are well documented, but more recently, increased attention has been given to the adverse psychiatric effects of these compounds. Steroids may be used in oral, 17alpha-alkylated, or intramuscular, 17beta-esterified, preparations. Commonly, steroid users employ these agents at levels 10- to 100-fold in excess of therapeutic doses and use multiple steroids simultaneously, a practice known as 'stacking'. Significant psychiatric symptoms including aggression and violence, mania, and less frequently psychosis and suicide have been associated with steroid abuse. Long-term steroid abusers may develop symptoms of dependence and withdrawal on discontinuation of AAS. Treatment of AAS abusers should address both acute physical and behavioural symptoms as well as long-term abstinence and recovery. To date, limited information is available regarding specific pharmacological treatments for individuals recovering from steroid abuse. This paper reviews the published literature concerning the recognition and treatment of behavioural manifestations of AAS abuse. PMID:15984895

Trenton, Adam J; Currier, Glenn W

2005-01-01

156

Oral Manifestation in Mentally Challenged Children  

PubMed Central

Background: In general, mentally challenged children have higher rates poor oral hygiene, gingivitis and periodontitis than the general population. An investigation was undertaken to assess the oral manifestations of mentally challenged children in Chennai, India. Materials and Methods: The study group consisted of 150 children (70 Down syndrome patients and 80 cerebral palsy patients). Of which, 93 patients were males and 57 were females. Results: Speech difficulty hindered the communication between the patient and the dentist. Mastication and swallowing difficulties were also present in few children. Profuse salivation was a cause for drooling of saliva down the cheeks, which was a constant finding in cerebral palsy children. The oral hygiene statuses of the patient were significantly poor. The prevalence of periodontitis was 35.7% in Down syndrome and 55.0% in cerebral palsy patients. Whereas, the prevalence of gingivitis was found to be 92.9% and 61.3% respectively. The prevalence of fractured maxillary anterior teeth was found to be more evident in cerebral palsy patients (62.9%) when compared to Down syndrome patients (0.0%). An increase in age shows an increase in the decayed-missing-filled teeth which is statistically significant. Conclusion: The prominent findings like flat nasal bridge (94.3%), hypertelorism (92.9%), high arched palate (78.6%) and fissured tongue (78.6%) in our study, suggest that they could be used as a reliable clinical markers to diagnose Down syndrome condition.

Rahul, V K; Mathew, Cinil; Jose, Sunil; Thomas, George; Noushad, M C; Feroz, T P Mohammed

2015-01-01

157

Oral manifestations of hepatitis C virus infection  

PubMed Central

Extrahepatic manifestations (EHMs) of hepatitis C virus (HCV) infection can affect a variety of organ systems with significant morbidity and mortality. Some of the most frequently reported EHM of HCV infection, involve the oral region predominantly or exclusively. Oral lichen planus (OLP) is a chronic inflammatory condition that is potentially malignant and represents cell-mediated reaction to a variety of extrinsic antigens, altered self-antigens, or super antigens. Robust epidemiological evidence support the link between OLP and HCV. As the virus may replicate in the oral mucosa and attract HCV-specific T lymphocytes, HCV may be implicated in OLP pathogenesis. Sjögren syndrome (SjS) is an autoimmune exocrinopathy, characterized by dryness of the mouth and eyes and a multitude of other systemic signs and symptoms. SjS patients have also an increased risk of non-Hodgkin lymphoma. Patients with chronic hepatitis C do frequently have histological signs of Sjögren-like sialadenitis with mild or even absent clinical symptoms. However, it is still unclear if HCV may cause a disease mimicking SjS or it is directly responsible for the development of SjS in a specific subset of patients. Oral squamous cell carcinoma is the most common oral malignant tumour and at least in some part of the world could be linked to HCV. PMID:24976694

Carrozzo, Marco; Scally, Kara

2014-01-01

158

Metabolic manifestations of multiple systems organ failure.  

PubMed

It is apparent that the metabolic response to injury as manifest in hypermetabolism and organ failure is a markedly different process from standard starvation. As such, it seems to have a different set of support principles that are probably better called metabolic support than nutritional support. The best treatment remains prevention by controlling the presence of dead tissue, injured tissue, and infection, and by appropriately restoring and maintaining oxygen transport. With control of the source and the restoration of oxygen transport, the primary mode of support becomes one of metabolic support. This modality is a support tool that appears to "buy time" and help control malnutrition as a comorbidity or comortality. The principles that have evolved in large part have been those of learning to do no harm. The prevention of substrate limited metabolism and effective support of visceral functions is now available until the late stage of the organ failure process. We still cannot control the catabolic stimulus and the autocannibalism of the skeletal muscle mass with the redistribution of nitrogen into the visceral protein mass and the use of amino acids as prime energy sources. The ability to control this stimulus will probably reside in our ability to understand and manipulate the mediator systems. We are now evolving effective support regimens for preserving organ structure and function and for promoting tissue repair. The route of administration as well as the time of administration of the regimen may have some impact on the disease course itself. When metabolic support has been applied in the setting of intelligent surgery and critical care, a progressive reduction in morbidity and mortality has been realized over the last several years such that the current mortality risk for the organ failure syndrome is in the 35 to 40 per cent range overall. PMID:2493973

Cerra, F B

1989-01-01

159

[Phenylketonuria with adult-onset neurological manifestation].  

PubMed

We report a male patient with phenylketonuria (PKU) who developed multisystem neurological manifestation in his fourth decade. He was born in 1957 when a neonatal mass screening had not been available. His neuropsychological development was entirely normal and he was a good athlete during his high school days. He was in good health until the age of 32, when his vision was blurred. In four months his gait progressively deteriorated to bind him to a wheel chair. On physical examination he had red hair and gray eyes. IQ was 68. Visual field showed concentric narrowing and his visual acuity was 0.2/0.3 (2.0/2.0). The limbs were spastic and weakened. He complained of pain in the extremities. He suffered from pollakisuria. Routine blood tests and CSF findings were normal. He was also found to be normal in peripheral nerve conduction studies and central conduction studies of SEP and VEP. EEG showed diffuse slowing in background activities. T2-weighted MRI of the head revealed widespread high-intensity areas in the deep white matter especially in bilateral occipital lobes. Serum aminogram disclosed the remarkably elevated phenylalanine (Phe) level to 1663 nmol/ml (normal range 50-90) and reduced tyrosine. Urinary secretion of endogenous tetrahydroxy-biopterin (BH4; coenzyme of Phe hydroxylase) remained in a normal range, and oral administration of 100 mg/kg of BH4 failed to normalize the serum Phe level. Despite a strict dietary control (oral intake of Phe less than 0.5 g/day), the serum Phe level remained high around 500 nmol/ml and his neurological deficits still deteriorated.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8299276

Ishimaru, K; Tamasawa, N; Baba, M; Matsunaga, M; Takebe, K

1993-09-01

160

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. PMID:22461779

Leis, A. Arturo; Stokic, Dobrivoje S.

2012-01-01

161

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

162

Manifest Anxiety in Mexican, Japanese, Israeli and American Children.  

ERIC Educational Resources Information Center

Gotts' hypothesis that manifest anxiety in children is related to societal restrictions was examined by comparing the Children's Manifest Anxiety Scale results of Mexican boys and girls with previously published results of American, Israeli, and Japanese children. The Mexican sample was selected to represent a high sex-role differentiation society…

Mebane, Donata F.; And Others

163

Orofacial manifestations of hematological disorders: anemia and hemostatic disorders.  

PubMed

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

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

2011-01-01

164

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

2010-08-01

165

Clinical Manifestations Associated with Neurocysticercosis: A Systematic Review  

PubMed Central

Background The clinical manifestations of neurocysticercosis (NCC) are poorly understood. This systematic review aims to estimate the frequencies of different manifestations, complications and disabilities associated with NCC. Methods A systematic search of the literature published from January 1, 1990, to June 1, 2008, in 24 different electronic databases and 8 languages was conducted. Meta-analyses were conducted when appropriate. Results A total of 1569 documents were identified, and 21 included in the analysis. Among patients seen in neurology clinics, seizures/epilepsy were the most common manifestations (78.8%, 95%CI: 65.1%–89.7%) followed by headaches (37.9%, 95%CI: 23.3%–53.7%), focal deficits (16.0%, 95%CI: 9.7%–23.6%) and signs of increased intracranial pressure (11.7%, 95%CI: 6.0%–18.9%). All other manifestations occurred in less than 10% of symptomatic NCC patients. Only four studies reported on the mortality rate of NCC. Conclusions NCC is a pleomorphic disease linked to a range of manifestations. Although definitions of manifestations were very rarely provided, and varied from study to study, the proportion of NCC cases with seizures/epilepsy and the proportion of headaches were consistent across studies. These estimates are only applicable to patients who are ill enough to seek care in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases. PMID:21629722

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

2011-01-01

166

The tricho-rhino-phalangeal syndrome: oral manifestations and management.  

PubMed

Tricho-rhino-phalangeal Syndrome (TRPS) is a rare inherited dysplasia affecting hair, nasal structure and fingers. A literature review indicated that since first described, three types of manifestations have been identified. A Table summarising the oral manifestations demonstrates the variety of presentations. A South African male child presented with the syndrome and was found to show premature eruption of permanent teeth, a finding that has not been previously reported. His oro-facial manifestations also included malaligned and unerupted crowded teeth, bulbosity of the nasal tip and an elongated philtrum and evidence of mild intellectual impairment. A protocol has been developed to guide the future management of these cases. PMID:24984390

Roberts, T; Chetty, M; Stephen, L; Fieggen, K; Beighton, P

2014-05-01

167

Neuroimaging and renal ultrasound manifestations of Oculocerebrorenal syndrome of Lowe  

PubMed Central

Oculocerebrorenal syndrome of Lowe (OCRL) is a multisystem disorder characterized by congenital cataracts, hypotonia, and cognitive developmental delay with renal complications developing in the first few months of life. Clinical and laboratory findings of Lowe syndrome are well documented. Though a small number of case reports describe the neuroimaging features and the renal ultrasound manifestations of this disease, a comprehensive review of all the imaging manifestations has not been reported. The authors present a case of OCRL and review the neuroimaging and renal ultrasound manifestations of this multisystem disease. PMID:25426219

Allmendinger, Andrew Mark; Desai, Naman S.; Burke, Alanna Teatom; Viswanadhan, Narayan; Prabhu, Sanjay

2014-01-01

168

[Gangrene as an initial manifestation of a catastrophic antiphospholipid syndrome].  

PubMed

Catastrophic antiphospholipid syndrome (CAPS) is an unusual form of presentation of antiphospholipid syndrome with a poor prognosis, so early diagnosis and treatment are necessary. We report a patient who had gangrene as the initial manifestation of CAPS. PMID:21794513

Vázquez Fuentes, Noelia; Tejero García, Sergio; Carnicero González, Fernando; Martínez Bagur, Irene; de Torres, Rafael Merino; Querol Gutiérrez, José Javier; Laza Laza, Enrique Manuel; Querol Gutiérrez, Juan Carlos

2008-05-01

169

Manilyzer: Automated Android Malware Detection through Manifest Analysis  

E-print Network

Manilyzer: Automated Android Malware Detection through Manifest Analysis Stephen Feldman malware threat. To counter this emerging menace, many malware detection techniques have been proposed additional information that can be valuable in identifying malware, which, however, has not been fully

Wang, Bing

170

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2012 CFR

...EMERGENCY RESPONSE INFORMATION, TRAINING REQUIREMENTS, AND SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest...numbers, generator certification and signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the...

2012-10-01

171

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2013 CFR

...EMERGENCY RESPONSE INFORMATION, TRAINING REQUIREMENTS, AND SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest...numbers, generator certification and signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the...

2013-10-01

172

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2011 CFR

...EMERGENCY RESPONSE INFORMATION, TRAINING REQUIREMENTS, AND SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest...numbers, generator certification and signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the...

2011-10-01

173

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2014 CFR

...EMERGENCY RESPONSE INFORMATION, TRAINING REQUIREMENTS, AND SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest...numbers, generator certification and signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the...

2014-10-01

174

49 CFR 172.205 - Hazardous waste manifest.  

Code of Federal Regulations, 2010 CFR

...EMERGENCY RESPONSE INFORMATION, TRAINING REQUIREMENTS, AND SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest...numbers, generator certification and signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the...

2010-10-01

175

19 CFR 122.48 - Air cargo manifest.  

Code of Federal Regulations, 2010 CFR

...AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew Members...

2010-04-01

176

19 CFR 122.74 - Incomplete (pro forma) manifest.  

Code of Federal Regulations, 2010 CFR

...PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew...

2010-04-01

177

Stationary regimes and their manifestations in birth-death processes  

SciTech Connect

The necessary conditions for the existence of stationary regimes in birth-death processes - with polynomial dependence of coefficients on the number of particles - are found. Possible manifestations of stationary regimes in hadron physics are discussed.

Batunin, A.V. (Institute for High Energy Physics, Serpukhov, 142284 Protvino (SU))

1989-05-01

178

Matrix Methods for Optimal Manifesting of Multinode Space Exploration Systems  

E-print Network

This paper presents matrix-based methods for determining optimal cargo manifests for space exploration. An exploration system is defined as a sequence of in-space and on-surface transports between multiple nodes coupled ...

Grogan, Paul Thomas

179

Dust Acoustic Mode Manifestations in Earth's Dusty Ionosphere  

SciTech Connect

Dust acoustic mode manifestations in the dusty ionosphere are studied. The reason for an appearance of the low-frequency radio noises associated with such meteor fluxes as Perseids, Orionids, Leonids, and Gemenids is determined.

Kopnin, S.I.; Popel, S.I. [Institute for Dynamics of Geospheres RAS, Leninsky pr. 38, bld. 1, 119334 Moscow (Russian Federation)

2005-10-31

180

Uncommon neurological manifestations of a common tropical vector borne disease  

PubMed Central

Malaria poses a major public health problem in India, where it is endemic, especially severe malaria caused by Plasmodium falciparum infestation. There have been great changes in the clinical manifestation of severe falciparum malaria over the past couple of decades, with a shift from cerebral malaria to fever with jaundice, renal failure, bleeding diathesis, and multi-organ dysfunction syndrome. Here, we discuss two cases of severe falciparum malaria which presented with extremely uncommon neurological manifestations. PMID:25709956

Ghosh, Saikat; Das, Somak Kumar; Sharma, Anand

2015-01-01

181

Long-standing prion dementia manifesting as posterior cortical atrophy.  

PubMed

Prion diseases commonly manifest with the phenotype of subacute myoclonic encephalopathy. However, genetic forms of prion disease may have prolonged evolution mimicking neurodegenerative disease. We present the clinical and neuropathological features of a family with an early and long-standing dementia manifesting with posterior cortical atrophy and related to a 120 bp insertional mutation of the prion protein gene. Two cases exhibited mixed prion and A? pathology. The differential diagnosis with Alzheimer disease is discussed. PMID:21959360

Depaz, Raphaël; Haik, Stéphane; Peoc'h, Katell; Seilhean, Danielle; Grabli, David; Vicart, Savine; Sarazin, Marie; DeToffol, Bertrand; Remy, Catherine; Fallet-Bianco, Catherine; Laplanche, J L; Fontaine, Bertrand; Brandel, Jean Philippe

2012-01-01

182

Sexist humor: Local and systemic manifestations of privilege and disadvantage  

E-print Network

SEXIST HUMOR: LOCAL AND SYSTEMIC MANIFESTATIONS OF PRIVILEGE AND DISADVANTAGE BY Kate M. Pickett Submitted to the graduate degree program in Psychology and the Faculty of the Graduate School at the University of Kansas in partial fulfillment... committee for Kate Pickett certifies that this is the approved version of the following thesis: SEXIST HUMOR: LOCAL AND SYSTEMIC MANIFESTATIONS OF PRIVILEGE AND DISADVANTAGE Committee: ______________________________ Glenn Adams, Ph.D., Chair...

Pickett, Kate M.

2008-07-29

183

Dermatologic manifestations of HPV in HIV-infected individuals  

Microsoft Academic Search

Dermatologic human papillomavirus (HPV) infection in HIV patients manifests as both anogenital and nongenital skin disease.\\u000a Anogenital HPV-related disease includes benign condyloma acuminata, the most common cutaneous manifestation of genital HPV\\u000a infection; intermediate malignancy or premalignant conditions including giant condyloma acuminata (also called Buschke-Loewenstein\\u000a tumor), anal intraepithelial neoplasia, penile intraepithelial neoplasia, and vaginal or vulvar intraepithelial neoplasia;\\u000a and frankly malignant

Rachel H. Gormley; Carrie L. Kovarik

2009-01-01

184

8 CFR 251.3 - Departure manifests and lists for vessels.  

Code of Federal Regulations, 2010 CFR

...2010-01-01 false Departure manifests and lists for vessels. 251.3 Section 251...REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.3 Departure manifests and lists for vessels. (a) Form I-418,...

2010-01-01

185

8 CFR 251.4 - Departure manifests and lists for aircraft.  

Code of Federal Regulations, 2010 CFR

...2010-01-01 false Departure manifests and lists for aircraft. 251.4 Section 251...REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.4 Departure manifests and lists for aircraft. (a) United...

2010-01-01

186

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

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false Form for transit air cargo manifest procedures. 122...TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures....

2013-04-01

187

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

Code of Federal Regulations, 2012 CFR

...2012-04-01 2012-04-01 false Form for transit air cargo manifest procedures. 122...TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures....

2012-04-01

188

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

Code of Federal Regulations, 2010 CFR

...2010-04-01 2010-04-01 false Form for transit air cargo manifest procedures. 122...TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures....

2010-04-01

189

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

Code of Federal Regulations, 2014 CFR

...2014-04-01 2014-04-01 false Form for transit air cargo manifest procedures. 122...TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures....

2014-04-01

190

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

Code of Federal Regulations, 2011 CFR

...2011-04-01 2011-04-01 false Form for transit air cargo manifest procedures. 122...TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures....

2011-04-01

191

Musculoskeletal manifestations in diabetic patients at a tertiary center  

PubMed Central

Objectives Diabetes mellitus is a major public health problem worldwide. Most diabetic patients will develop functional disabilities due to multiple factors, including musculoskeletal (MSK) manifestations. The purpose of this study was to determine the frequency of MSK in diabetic patients and to examine the possible predictors for its development. Methods We performed a cross-sectional study from June 1, 2010, to June 30, 2011, to evaluate MSK manifestations in adult diabetic patients at an outpatient clinic of King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Baseline variables were examined to determine predictors for the development of MSK complications. Analyses were carried out using the Statistical Package for Social sciences. Results We included 252 diabetic patients; 45 (17.9%) had MSK manifestations. Of these 45 patients, 41 (91.1%) had type 2 diabetes. The most common manifestations were carpal tunnel syndrome (n=17, 6.7%), shoulder adhesive capsulitis (n=17, 6.7%), and diabetic amyotrophy (n=12, 4.8%). A significant association was found between the development of MSK manifestations and manual labor, overweight, and vascular complications. On logistic regression analysis, the presence of vascular complications in general (B-coefficient=1.27, odds ratio=3.57, P<0.05, 95% confidence interval=1.31–9.78), and retinopathy in particular (B-coefficient=1.17, odds ratio=3.21, P<0.05, 95% confidence interval=1.47–7.02) can predict the development of MSK manifestations in about 82% of the cases. Conclusion Musculoskeletal manifestations are under recognized in adult diabetic patients, occurring in 18% of the cases. Physicians should consider examining the periarticular region of the joints in the hands and shoulders whenever a diabetic patient presents with MSK symptoms. PMID:23115579

Attar, Suzan M.

2012-01-01

192

Extrahepatic manifestations in chronic hepatitis C virus carriers.  

PubMed

Patients with chronic hepatitis C virus (HCV) infection frequently present with extrahepatic manifestations covering a large spectrum, involving different organ systems leading to the concept of systemic HCV infection. These manifestations include autoimmune phenomena and frank autoimmune and/or rheumatic diseases and may dominate the course of chronic HCV infection. Chronic HCV infection causes liver inflammation affecting the development of hepatic diseases. HCV is also a lymphotropic virus that triggers B cells and promotes favorable conditions for B lymphocyte proliferation, including mixed cryoglobulinemia (MC) and MC vasculitis, which is the most prominent extrahepatic manifestation of chronic HCV infection. HCV may also promote a low-grade chronic systemic inflammation that may affect the development of some extrahepatic manifestations, particularly cardiovascular and cerebral vascular diseases. Recognition of extrahepatic symptoms of HCV infection could facilitate early diagnosis and treatment. The development of direct-acting antiviral agents (DDAs) has revolutionized HCV treatment. DDAs, as well as new B-cell-depleting or B-cell-modulating monoclonal antibodies, will expand the panorama of treatment options for HCV-related extrahepatic manifestations including cryoglobulinemic vasculitis. In this context, a proactive, integrated approach to HCV therapy should maximize the benefits of HCV therapy, even when liver disease is mild. PMID:25801890

Rosenthal, E; Cacoub, P

2015-04-01

193

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. PMID:22563221

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

2012-01-01

194

Extraintestinal manifestations of Helicobacter pylori: a concise review.  

PubMed

Helicobacter pylori (H. pylori) infection has been clearly linked to peptic ulcer disease and some gastrointestinal malignancies. Increasing evidence demonstrates possible associations to disease states in other organ systems, known as the extraintestinal manifestations of H. pylori. Different conditions associated with H. pylori infection include those from hematologic, cardiopulmonary, metabolic, neurologic, and dermatologic systems. The aim of this article is to provide a concise review of the evidence that supports or refutes the associations of H. pylori and its proposed extraintestinal manifestations. Based on data from the literature, PUD, mucosal associated lymphoid tumors lymphoma, and gastric adenocarcinoma has well-established links. Current evidence most supports extraintestinal manifestations with H. pylori in immune thrombocytopenic purpura, iron deficiency anemia, urticaria, Parkinson's, migraines and rosacea; however, there is still plausible link with other diseases that requires further research. PMID:25232230

Wong, Frank; Rayner-Hartley, Erin; Byrne, Michael F

2014-09-14

195

Large cerebellar mass lesion: A rare intracranial manifestation of blastomycosis  

PubMed Central

Background: Blastomyces dermatitidis is a dimorphic fungus found endemically in the Mississippi and Ohio River basins and in the Midwestern and Canadian provinces that border the Great Lakes. Unlike other fungal infections, it most commonly affects immunocompetent hosts. Blastomycosis typically manifests as pulmonary infection, but may affect nearly any organ, including the skin, bone, and genitourinary system. Central nervous system (CNS) blastomycosis is rare, but potentially fatal manifestation of this disease. When it does occur, it most commonly presents as acute or chronic meningitis. Case Description: We present a case of a patient who suffered intractable nausea and vomiting for several months before discovery of a large cerebellar blastomycoma causing mass effect and obstructive hydrocephalus. The enhancing lesion with unusual peripheral cystic structures is a unique radiographic appearance of CNS blastomycosis. Conclusion: We review this patient's purely intraparenchymal manifestation of CNS blastomycosis and describe the unique imaging characteristics encountered. PMID:24231945

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

2013-01-01

196

Extraintestinal manifestations of Helicobacter pylori: A concise review  

PubMed Central

Helicobacter pylori (H. pylori) infection has been clearly linked to peptic ulcer disease and some gastrointestinal malignancies. Increasing evidence demonstrates possible associations to disease states in other organ systems, known as the extraintestinal manifestations of H. pylori. Different conditions associated with H. pylori infection include those from hematologic, cardiopulmonary, metabolic, neurologic, and dermatologic systems. The aim of this article is to provide a concise review of the evidence that supports or refutes the associations of H. pylori and its proposed extraintestinal manifestations. Based on data from the literature, PUD, mucosal associated lymphoid tumors lymphoma, and gastric adenocarcinoma has well-established links. Current evidence most supports extraintestinal manifestations with H. pylori in immune thrombocytopenic purpura, iron deficiency anemia, urticaria, Parkinson’s, migraines and rosacea; however, there is still plausible link with other diseases that requires further research. PMID:25232230

Wong, Frank; Rayner-Hartley, Erin; Byrne, Michael F

2014-01-01

197

Comprehensive treatment of psoriatic arthritis: managing comorbidities and extraarticular manifestations.  

PubMed

Psoriatic arthritis (PsA) is an inflammatory arthritis associated with psoriasis that can lead to decreased health-related quality of life and permanent joint damage leading to functional decline. In addition to joint and skin manifestations, both psoriasis and PsA are associated with numerous comorbidities and extraarticular/cutaneous manifestations, which may influence the physician's choice of therapy. The objectives of this review are (1) to identify comorbidities in patients with PsA based on the available evidence; (2) to examine the effects of these comorbidities or extraarticular/cutaneous manifestation on the management of patients with PsA as well as the selection of therapy; and (3) to highlight research needs around comorbidities and treatment paradigms. This review is part of a treatment recommendations update initiated by the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA). PMID:25362717

Ogdie, Alexis; Schwartzman, Sergio; Eder, Lihi; Maharaj, Ajesh B; Zisman, Devy; Raychaudhuri, Siba P; Reddy, Soumya M; Husni, Elaine

2014-11-01

198

Dermatological manifestations of hepatitis C virus infection in Saudi Arabia.  

PubMed

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

Halawani, Mona R

2014-06-01

199

Gastrointestinal and Hepatic Manifestations of Primary Immune Deficiency Diseases  

PubMed Central

Primary immune deficiency diseases (PIDs) are a heterogeneous group of inherited diseases characterized by variable genetic immune defects, conferring susceptibility to recurrent infections. They have a vast array of manifestations some of which involve the gastrointestinal and hepatobiliary systems. These complications can be the consequence of five different factors, namely, infection, autoimmune process, unregulated inflammation, malignancies and complications of therapeutic intervention. They may precede the PID diagnosis and, once developed, they pose high risk of morbidity. Untrained clinicians may treat these manifestations only at the level of their presentation, leaving the PIDs dangerously undiagnosed. In fact, early diagnosis of PIDs and accompanied gastrointestinal and hepatic complications clearly require appropriate treatment, and in-turn lead to an improved quality of life for the patient. To improve the awareness of gastroenterologists and related health care providers about these diseases, we have reviewed herein the complications of different PIDs focusing on gastrointestinal and hepatic manifestation. PMID:20339173

Al-Muhsen, Saleh Z.

2010-01-01

200

[Brown tumor as the initial manifestation of primary hyperparathyroidism].  

PubMed

Brown tumor is a localized form of osteitis fibrosa cystica, being part of the hyperparathyroid bone disease. It rarely is the first manifestation of hyperparathyroidism, since nowadays, the diagnosis is made at an asymptomatic or minimally symptomatic stage. We present a case of a left superior maxillar brown tumor as the first manifestation of primary hyperparathyroidism due to a parathyroid adenoma. A parathyroidectomy was performed, and there was a regression of the bone lesion, without the need of performing other local surgical procedures. PMID:18689153

Chavin, Hernán C; Pisarevsky, Ana A; Chavín, Carolina; Koll, Lucrecia; Petrucci, Enrique A

2008-01-01

201

FRAGILE X SYNDROME: PSYCHIATRIC MANIFESTATIONS, ASSESSMENT AND EMERGING THERAPIES  

PubMed Central

Fragile X Syndrome (FXS), the most common inherited cause of intellectual disabilities, is an X-linked dominant disorder caused by the amplification of a CGG repeat in the 5? untranslated region of the fragile X mental retardation gene 1 (FMR1). Prevalence estimates of the disorder are approximately 1/3600. Psychiatric manifestations of the disorder include anxiety, attention deficit hyperactivity disorder, autism, mood instability and aggression. In this article we review the above psychiatric manifestations and challenges to accurate assessment. We also discuss how the neurobiological underpinnings of these symptoms are beginning to be understood and can help guide treatment. PMID:25632275

Wadell, Paula M.; Hagerman, Randi J.; Hessl, David R.

2015-01-01

202

Endocrine symptoms as the initial manifestation of Wilson's disease.  

PubMed

Wilson's disease is a rare genetic disorder of copper metabolism. The difference in copper tissue accumulation is responsible for the various clinical manifestations of this disorder. If left untreated, Wilson's disease progresses to hepatic failure, severe neurological disability, and even death. Due to the complex clinical picture of Wilson's disease, its diagnosis relies on a high index of suspicion. In our paper, we present endocrine symptoms suggesting the presence of insulinoma and hyperprolactinemia as the initial clinical manifestation of Wilson's disease in a young female. Zinc acetate treatment resulted in the disappearance of hypoglycemia, galactorrhea, and menstrual abnormalities. PMID:22737053

Krysiak, Robert; Handzlik-Orlik, Gabriela; Okopien, Boguslaw

2012-06-01

203

Mild Cutaneous Manifestation in Two Young Women with Extraordinary Hyperandrogenemia  

Microsoft Academic Search

Hyperandrogenism with hyperandrogenemia should be considered in those with severe acne of sudden onset or conspicuous male-pattern baldness with hairline recession, although the majority of female patients with acne or androgenetic alopecia possess no endocrine disorder. Herein we describe on the contrary 2 young women with primary amenorrhea displaying prominent hyperandrogenemia but subtle cutaneous manifestation. The first one presenting vertical

WenChieh Chen; Guan-Yu Chen; Shaw-Jenq Tsai; Pei-Wen Wang; Hamm-Ming Sheu; Yen-Sheng Shen; Fen-Fen Chen

2005-01-01

204

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

205

Hiccups, eructation, and other uncommon prodromal manifestations of herpes zoster  

Microsoft Academic Search

Although the most frequent presentation of herpes zoster involves sensory neurons, motor and autonomic symptomatology is also known to occur in this disease. An unusual symptom of hiccups is described here. Other infrequent manifestations of this common illness, including the Ramsay Hunt syndrome, herpes zoster ophthalmicus, urinary and fecal retention, sexual dysfunction, and zoster sine herpete, are reviewed. Greater awareness

Alexander L. Berlin; Channy Y. Muhn; Robin C. Billick

2003-01-01

206

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

207

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

208

REVIEW THE CHRONIC GASTROINTESTINAL MANIFESTATIONS OF CHAGAS DISEASE  

E-print Network

Chagas disease is an infectious disease caused by the protozoan Trypanosoma cruzi. The disease mainly affects the nervous system, digestive system and heart. The objective of this review is to revise the literature and summarize the main chronic gastrointestinal manifestations of Chagas disease

Nilce Mitiko Matsuda; I Steven; M. Miller

209

Prostatic Abscess due to Candida with No Systemic Manifestations  

Microsoft Academic Search

Prostatic abscess due to fungi is a rare condition. It is generally secondary to systemic disease in immunosuppressed patients. It usually occurs with affection of other organs in a septic patient. Only in exceptional cases does it occur isolatedly. We present the case of a prostatic abscess due to Candida albicans with no systemic manifestations. The diagnosis is helped by

A. Collado; J. Ponce de León; D. Salinas; J. Salvador; J. Vicente

2001-01-01

210

Pes anserine bursitis - an extra-articular manifestation of gout.  

PubMed

While hospitalized with polymyositis, a medically complex 56-year-old male experienced an acute exacerbation of gout. Both ultrasound and magnetic resonance imaging cross-sectional modalities were used to detect, localize, and characterize a soft tissue mass. The tumor was ultimately found to be secondary to gouty inflammation of the pes anserine bursa, a previously unrecognized manifestation of acute gout. PMID:20345364

Grover, Raj Pal S; Rakhra, Kawan S

2010-01-01

211

Mucocutaneous Manifestations in Japanese HIV-Positive Hemophiliacs  

Microsoft Academic Search

Background: Although various mucocutaneous manifestations have been reported in patients infected with HIV by sexual transmission or intravenous drug use, the prevalence and characteristics of skin disorders in HIV-positive hemophiliacs coinfected with hepatitits C virus (HCV) have rarely been described. Objective: The purpose of this study was to clarify the characteristics of skin disorders in HIV-positive hemophiliacs and to identify

Satoko Shimizu; Ko-Ron Chen; Hisamichi Tagami; Hideji Hanabusa

2000-01-01

212

Ocular manifestations of Behçet’s disease in Jordanian patients  

PubMed Central

Purpose To study the prevalence, manifestations and severity of ocular involvement of Behçet’s disease in Jordanian patients. Methods The study population consisted of 43 patients diagnosed to have Behçet’s disease through Rheumatologist’s examinations conducted at Jordan University Hospital between January 2002 and July 2009. The sample involved patients who displayed ocular manifestations. This included 18 patients; 12 males and 6 females with a mean age of 35 years (SD = 17.26). Ophthalmological examinations and retrospective analysis of medical files were carried on. Results Ocular manifestations were seen in 41.9% of patients. The most common manifestation for Behçet’s disease was vitritis with a prevalence of 55.6%, followed by anterior uveitis and retinal vasculitis (50% for each). On the other hand, the most frequent complications involved were cataract, cystoid macular edema (CMO), posterior synechiae and glaucoma with a prevalence of (44.4%), (33.3%), (11.1%) and (5.6%), respectively. Conclusion The prevalence and severity of ocular lesions in Behçet’s disease is relatively low in Jordanian patients. This result indicates that early diagnoses and intervention might delay or even prevent vision loss for those patients. PMID:24371419

Abu-Ameerh, Mohammed A.; Mohammed, Sawsan F.; Mohammad, Mona T.; Ababneh, Osama H.; Al-Bdour, Muawyah D.

2013-01-01

213

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

PubMed

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

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

2012-01-01

214

Neurologic manifestations of inherited disorders of connective tissue.  

PubMed

Inherited disorders of connective tissue are single gene disorders affecting structure or function of the connective tissue. Neurological manifestations are classic and potentially severe complications of many such disorders. The most common neurological manifestations are cerebrovascular. Ischemic stroke is a classic complication of vascular Ehlers-Danlos syndrome (type IV), homocystinuria, and arterial tortuosity syndrome, and may occasionally be seen in Marfan syndrome and pseudoxanthoma elasticum with distinct underlying mechanisms for each disease. Vascular Ehlers-Danlos syndrome can also lead to cervical artery dissection (with or without ischemic stroke), carotid-cavernous fistula, intracranial dissections and aneurysms potentially causing subarachnoid or intracerebral hemorrhage, and arterial rupture. Other neurological manifestations include nerve root compression and intracranial hypotension due to dural ectasia in Marfan and Loeys-Dietz syndrome, spinal cord compression in osteogenesis imperfecta, and mucopolysaccharidosis type I and VI, carpal tunnel syndrome in mucopolysaccharidosis type I, II, and VI. Impaired mental development can be observed in homocystinuria, mucopolysaccharidosis type II, and the severe form of mucopolysaccharidosis type I. For the neurologist, being aware of these complications and of the diagnostic criteria for inherited connective tissue disorders is important since neurological complications can be the first manifestation of the disease and because caution may be warranted for the management of these patients. PMID:24365320

Debette, Stéphanie; Germain, Dominique P

2014-01-01

215

The Rolex sign: first manifestation of Parkinson's disease: case report.  

PubMed

We describe a patient whose initial manifestation of Parkinson's disease was a malfunctioning of a self-winding wristwatch secondary to bradykinesia of his left arm. Andrade and Ferraz reported this sign in 1996, suggesting that it be called the Rolex sign. PMID:10973116

Teive, H A; Sá, D S

2000-09-01

216

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

217

Manifesting heterozygosity in sex-linked spastic paraplegia?  

Microsoft Academic Search

An unusual form of hereditary spastic paraplegia is described. Affected females have a late-onset slowly progressive spastic paraparesis. Affected males show oligophrenia with a rapidly progressive spastic quadriplegia. The mode of inheritance is consistent with sex-linkage, with partial manifestation in female carriers.

I D Young; I F Pye; J R Moore

1984-01-01

218

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

219

Biological therapy for dermatological manifestations of inflammatory bowel disease  

PubMed Central

Ulcerative colitis and Crohn’s disease are the two forms of inflammatory bowel disease (IBD). The advent of biological drugs has significantly changed the management of these conditions. Skin manifestations are not uncommon in IBD. Among the reactive lesions (immune-mediated extraintestinal manifestations), erythema nodosum (EN) and pyoderma gangrenosum (PG) are the two major cutaneous ills associated with IBD, while psoriasis is the dermatological comorbidity disease observed more often. In particular, in the last few years, anti-tumor necrosis factor (TNF)-? agents have been successfully used to treat psoriasis, especially these kinds of lesions that may occur during the treatment with biological therapies. The entity of the paradoxical manifestations has been relatively under reported as most lesions are limited and a causal relationship with the treatment is often poorly understood. The reason for this apparent side-effect of the therapy still remains unclear. Although side effects may occur, their clinical benefits are undoubted. This article reviews the therapeutic effects of the two most widely used anti-TNF-? molecules, infliximab (a fusion protein dimer of the human TNF-? receptor) and adalimumab (a fully human monoclonal antibody to TNF-?), for the treatment of the major cutaneous manifestations associated with IBD (EN, PG and psoriasis). PMID:24303470

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

2013-01-01

220

Current Perspectives on Ophthalmic Manifestations of Childhood Rheumatic Diseases  

PubMed Central

Inflammatory eye diseases are an important manifestation of many pediatric rheumatologic conditions. Early screening and diagnosis are imperative as these illnesses can not only result in significant visual morbidity but also be an indicator of systemic inflammation. Time to presentation of ocular inflammation varies significantly and can range from many years prior to the onset of systemic symptoms to well after the diagnosis of the rheumatologic disorder. Due to this variability in presentation, careful monitoring by an ophthalmologist is vital to preventing ocular complications and preserving vision. Both local and systemic immunosuppressive medications have been effective in the management of ocular disease. In this review, we will focus on the known ophthalmologic manifestations of common pediatric rheumatologic diseases and discuss recent advances in therapeutic considerations for these conditions. PMID:23686303

Palejwala, Neal V.; Yeh, Steven; Angeles-Han, Sheila T.

2013-01-01

221

Oral manifestation in inflammatory bowel disease: a review.  

PubMed

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

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

2013-12-14

222

Oral manifestation in inflammatory bowel disease: A review  

PubMed Central

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

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

2013-01-01

223

Secondary glaucoma as initial manifestation of uveal melanoma  

PubMed Central

Purpose Secondary glaucoma can be induced by a variety of local ocular problems. Intraocular tumors may initially present as secondary glaucoma. Methods 8 consecutive patients with secondary glaucoma were found to have uveal melanoma. Thorough examination included detailed history, fundus examination with scleral depression, B scan ultrasonography, and CT/MRI scanning techniques. Results A single case presented with spontaneous hyphema, two patients presented with secondary glaucoma, extraocular melanoma and metastases, a single case was found to have angle block by an iridociliary ring melanoma and 4 cases presented with neovascular glaucoma. Enucleation was necessary in all 8 cases. Conclusions General ophthalmologists should be aware of these rare initial manifestations of intraocular tumors as secondary glaucoma. Enucleation would be recommended in most cases of intraocular malignancy manifesting as secondary glaucoma. One should be extremely cautious in doing a penetrating surgery in such cases. PMID:24227987

Othman, Ihab Saad; Assem, Maher; Zaki, Iman M.A.

2013-01-01

224

The MANIFEST fibre positioning system for the Giant Magellan Telescope  

NASA Astrophysics Data System (ADS)

MANIFEST is a fibre feed system for the Giant Magellan Telescope that, coupled to the seeing-limited instruments GMACS and G-CLEF, offers qualitative and quantitative gains over each instrument's native capabilities in terms of multiplex, field of view, and resolution. The MANIFEST instrument concept is based on a system of semi-autonomous probes called "Starbugs" that hold and position hundreds of optical fibre IFUs under a glass field plate placed at the GMT Cassegrain focal plane. The Starbug probes feature co-axial piezoceramic tubes that, via the application of appropriate AC waveforms, contract or bend, providing a discrete stepping motion. Simultaneous positioning of all Starbugs is achieved via a closed-loop metrology system.

Lawrence, Jon S.; Brown, David M.; Brzeski, Jurek; Case, Scott; Colless, Matthew; Farrell, Tony; Gers, Luke; Gilbert, James; Goodwin, Michael; Jacoby, George; Hopkins, Andrew M.; Ireland, Michael; Kuehn, Kyler; Lorente, Nuria P. F.; Miziarski, Stan; Muller, Rolf; Nichani, Vijay; Rakman, Azizi; Richards, Samuel; Saunders, Will; Staszak, Nick F.; Tims, Julia; Vuong, Minh; Waller, Lew

2014-08-01

225

Clinical Manifestations and Outcomes of West Nile Virus Infection  

PubMed Central

Since the emergence of West Nile virus (WNV) in North America in 1999, understanding of the clinical features, spectrum of illness and eventual functional outcomes of human illness has increased tremendously. Most human infections with WNV remain clinically silent. Among those persons developing symptomatic illness, most develop a self-limited febrile illness. More severe illness with WNV (West Nile neuroinvasive disease, WNND) is manifested as meningitis, encephalitis or an acute anterior (polio) myelitis. These manifestations are generally more prevalent in older persons or those with immunosuppression. In the future, a more thorough understanding of the long-term physical, cognitive and functional outcomes of persons recovering from WNV illness will be important in understanding the overall illness burden. PMID:24509812

Sejvar, James J.

2014-01-01

226

Endocrine manifestations of von Hippel-Lindau disease.  

PubMed

von Hippel-Lindau (VHL) disease is an autosomal dominant disorder caused by heterozygous mutations in the VHL tumor suppressor gene that is characterized by the occurrence of multiple endocrine and nonendocrine lesions. This review focuses on the endocrine manifestations of VHL disease. Pancreatic neuroendocrine proliferations (ductuloinsular complexes, islet dysplasia, endocrine microadenoma, and neuroendocrine tumors), pheochromocytomas, and extra-adrenal paragangliomas are important endocrine manifestations of VHL disease. They frequently display characteristic clinical, biochemical, and histopathologic features that, although not pathognomonic, can be helpful in suggesting VHL disease as the underlying etiology and distinguishing these tumors from sporadic cases. Recent improvements in treatment and outcomes of renal cell carcinomas have allowed pancreatic neuroendocrine tumors to emerge as a significant source of metastatic disease, making the accurate recognition and classification of these neoplasms by the pathologist of utmost importance to determine prognosis, treatment, and follow-up strategies for affected patients. PMID:25611110

Cassol, Clarissa; Mete, Ozgur

2015-02-01

227

Neurological counterparts of hyponatremia: pathological mechanisms and clinical manifestations.  

PubMed

Hyponatremia, defined as a serum sodium concentration <135 mEq/L, represents the most frequent electrolyte disorder in older hospitalized patients. Early recognition of hyponatremia is mandatory, since it represents an independent risk factor that increases hospital mortality by 40 %. Delayed correction of hyponatremia may worsen brain edema, resulting in different degrees of neural damage. However, an overly rapid correction of serum sodium levels can lead to osmotic demyelination syndrome (ODS), a dreadful neurological picture. In recent years, hyponatremia and ODS have received growing attention both in terms of clinical management and pathophysiology, leading to the discovery of new drugs and treatment algorithms. In this review, we recapitulate the pathogenetic background, clinical manifestations, and treatment guidelines of hyponatremia, focusing on the neurological alterations. Neurological symptoms may be neglected when they manifest as early signs of mild hyponatremia, while brain damage can irremediably affect patients' conditions in the context of ODS. PMID:25724319

Podestà, Manuel Alfredo; Faravelli, Irene; Cucchiari, David; Reggiani, Francesco; Oldani, Silvia; Fedeli, Carlo; Graziani, Giorgio

2015-04-01

228

What type of different clinical manifestations can cardiac sarcoidosis present?  

PubMed

Cardiac sarcoidosis is an infiltrative, granulomatous inflammatory disease of the myocardium. Generally, it can be difficult to diagnose cardiac sarcoidosis clinically because of the non-specific nature of its clinical manifestations. This property can be based on the presence of any clinical evidence of sarcoidosis in the other organs. We present two cases of cardiac sarcoidosis so as to demonstrate its different clinical manifestations. The first patient displayed no cardiac symptoms; the electrocardiogram showed an incidental right bundle branch block. Her cardiac magnetic resonance imaging (CMRI) revealed late-phase opaque material enhancement involving the inferior and inferoseptal segment of the left ventricle. The second patient was severely symptomatic in terms of cardiac involvement, and a transthoracic echocardiogram revealed global hypokinesia and septal brightness; his ejection fraction decreased to 45 %. These cases highlighted the challenges encountered in the diagnosis and treatment of cardiac sarcoidosis. CMRI should be considered in all patients who have suspected findings for cardiac involvement. PMID:25429793

Sentürk, Ay?egül; Mara?, Yüksel; Argüder, Emine; Karalezli, Ay?egül; Hasano?lu, H Canan; O?üt, Tuba; Ba?tu?, Serdal; Karabekir, Ercan

2014-11-28

229

Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies  

PubMed Central

Thrombotic microangiopathies (TMA) are rare but severe disorders, characterized by endothelial cell activation and thrombus formation leading to hemolytic anemia, thrombocytopenia, and organ failure. Complement over activation in combination with defects in its regulation is described in an increasing number of TMA and if primary for the disease denominated as atypical hemolytic-uremic syndrome. Although TMA predominantly affects the renal microvasculature, extra-renal manifestations are observed in 20% of patients including involvement of the central nerve system, cardiovascular system, lungs, skin, skeletal muscle, and gastrointestinal tract. Prompt diagnosis and treatment initiation are therefore crucial for the prognosis of disease acute phase and the long-term outcome. This review summarizes the available evidence on extra-renal TMA manifestations and discusses the role of acute and chronic complement activation by highlighting its complex interaction with inflammation, coagulation, and endothelial homeostasis. PMID:25250305

Hofer, Johannes; Rosales, Alejandra; Fischer, Caroline; Giner, Thomas

2014-01-01

230

A Rare Presentation of Hepatitis A Infection with Extrahepatic Manifestations  

PubMed Central

Hepatitis A has a variety of associated extrahepatic manifestations that clinicians should be aware of for early diagnosis and treatment. We report a unique case of hepatitis A presenting with multiple extrahepatic manifestations not previously described in a single patient. A 34-year-old male presented with sudden onset of left sided facial pain, swelling, and skin rash, with diffuse body pains and muscle weakness, and was found to be positive for hepatitis A immunoglobulin M (IgM). He was initially started on antibiotics for concerns of bacterial parotitis but did not show any improvement. A punch biopsy of his mandibular rash and swelling was done which showed lymphohistiocytic infiltration with a few eosinophils. A trial of prednisone resulted in improvement of his symptoms. Clinicians should be aware to look for hepatitis A infection in a patient with atypical clinical picture causing a widespread systemic inflammatory response. Treatment with prednisone may result in resolution. PMID:25295197

Bhatt, Geetika; Sandhu, Varrinder S.; Mitchell, Charlene K.

2014-01-01

231

Extrahepatic immune related manifestations in chronic hepatitis C virus infection.  

PubMed

The association of chronic hepatitis C with immune related syndromes has been frequently reported. There is a great range of clinical manifestations affecting various systems and organs such as the skin, the kidneys, the central and peripheral nervous system, the musculoskeletal system and the endocrine glands. Despite the high prevalence of immune related syndromes in patients with chronic hepatitis C, the exact pathogenesis is not always clear. They have been often associated with mixed cryoglobulinemia, a common finding in chronic hepatitis C, cross reaction with viral antigens, or the direct effect of virus on the affected tissues. The aim of this review is to analyze the reported hepatitis C virus immune mediated syndromes, their prevalence and clinical manifestations and to discuss the most supported theories regarding their pathogenesis. PMID:25253938

Tampaki, Maria; Koskinas, John

2014-09-21

232

Extrahepatic immune related manifestations in chronic hepatitis C virus infection  

PubMed Central

The association of chronic hepatitis C with immune related syndromes has been frequently reported. There is a great range of clinical manifestations affecting various systems and organs such as the skin, the kidneys, the central and peripheral nervous system, the musculoskeletal system and the endocrine glands. Despite the high prevalence of immune related syndromes in patients with chronic hepatitis C, the exact pathogenesis is not always clear. They have been often associated with mixed cryoglobulinemia, a common finding in chronic hepatitis C, cross reaction with viral antigens, or the direct effect of virus on the affected tissues. The aim of this review is to analyze the reported hepatitis C virus immune mediated syndromes, their prevalence and clinical manifestations and to discuss the most supported theories regarding their pathogenesis. PMID:25253938

Tampaki, Maria; Koskinas, John

2014-01-01

233

Rupioid psoriasis and other skin diseases with rupioid manifestations.  

PubMed

The term rupioid has been used to describe well-demarcated, cone-shaped plaques with thick, dark, lamellate, and adherent crusts on the skin that somewhat resemble oyster or limpet shells. We present a case of rupioid psoriasis that was treated with methotrexate, topical steroids, and intralesional steroid injections. Rupioid manifestations have been clinically observed in a variety of disease settings, including rupioid psoriasis, reactive arthritis, disseminated histoplasmosis, keratotic scabies, secondary syphilis, and photosensitive skin lesions in association with aminoaciduria. To diagnose the underlying infectious or inflammatory diseases beneath the thick crusts, skin biopsy and a blood test for syphilis may be necessary. Our aim is to familiarize clinicians with the differential diagnoses for skin diseases with rupioid manifestations. PMID:25279472

Chung, Hye J; Marley-Kemp, Daria; Keller, Matthew

2014-09-01

234

Extrahepatic manifestations of HCV infection: A brief review and update  

Microsoft Academic Search

Hepatitis C affects approximately 3% of the population worldwide and extrahepatic manifestations may occur in nearly 40% of\\u000a all patients. Recent efforts have focused on incidence, prevalence, clinical significance, mechanism of disease, and the role\\u000a of antiviral therapy in treatment. Cryoglobulinemia, cryoglobulinemic vasculitis, membranoproliferative glomerulonephritis,\\u000a and lymphoproliferative disorders have a well-established link to hepatitis C, and many other endocrine, autoimmune,

Nidhi Singh; Nancy Reau

2006-01-01

235

Treatment of extraintestinal manifestations in inflammatory bowel disease  

Microsoft Academic Search

Opinion statement  Extraintestinal manifestations (EIM) of inflammatory bowel disease (IBD) occur rather frequently and may be found in up to\\u000a 30% of patients. However, surprisingly few randomized, controlled studies have been conducted that were specifically aimed\\u000a at the treatment of EIM of IBD patients. Therefore, most therapies of EIM are empiric or deduced from studies in populations\\u000a with other type of

Adrian A. van Bodegraven; A. Salvador Peña

2003-01-01

236

Syphilitic Hepatitis: An Uncommon Manifestation of a Common Disease  

PubMed Central

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

Baveja, Sukriti; Garg, Shilpa; Rajdeo, Amol

2014-01-01

237

Geographic Manifestation of Spanish Moss Physiology Across The Americas  

E-print Network

Geographic Manifestation of Spanish Moss Physiology Across The Americas Narayani Barve Ecology and evolutionary biology University of Kansas GIS Day, November 16, 2011 Problem • Ecological niche models use known occurrences... and background climatic information to predict potential distribution of species on the geography. • Used in conservation, biogeography, invasive species spread etc. • But ENMs do not consider organisms’ physiological limits explicitly. • Explore occurrence representation in the...

Barve, Narayani

2011-11-16

238

Radar manifestations of ship wakes in algae bloom zones  

NASA Astrophysics Data System (ADS)

Radar manifestations of ship wakes in zones of phytoplankton bloom are discussed. It is shown that these signatures can be regarded as indicators of biogenic activity. The main data are satellite radar images. Satellite visible (VIS) and infrared (IR) satellite data are also analyzed. The large amount of the available data allowed us to make some generalizations and obtain statistically reliable results concerning spatial and temporal variability of certain type of ship wake manifestations in synthetic aperture radar (SAR) images of the sea surface. Traditional classification of surface ship wakes manifestations in satellite SAR images specifies distinct features such as a dark trailing centreline region (turbulent wake), narrow V-wakes aligned at some angle to the ship's path (the Kelvin wake), and, sometimes, internal wave wakes generated under conditions of shallow stratification. Their characteristic lengths are reported to be up to tens of kilometers and they can last from tens of minutes up to one hour. Instances of radar signatures of the ship wakes dissimilar to the previously described were detected in radar images obtained in the course of a satellite monitoring campaign of the central and south-eastern Baltic. These ship wakes can be seen in satellite radar images as long bright strips of enhanced backscatter with characteristic length of up to several hundred kilometres lasting more than 5 hours. A hypothesis is put forward of the coherence of this type of ship wakes detected in sea surface radar imagery and areas of intensive biogenic activity under conditions of low near-surface winds. Statistics on their seasonal, spatial and year-to-year distribution are drawn. These results are compared with temporal and spatial variations in chlorophyll a concentration and intensity of phytoplankton bloom in the area of interest. Chlorophyll a concentration maps derived from satellite data are used, as well as those based on in situ measurements. The relation between occurrences of this type of ship wake manifestations and areas of algae blooms is established.

Mityagina, Marina I.; Lavrova, Olga Yu.

2014-10-01

239

An unusual otological manifestation of Usher's syndrome in four siblings.  

PubMed

A family in which four out of ten siblings affected by Usher's syndrome, with progressive hearing loss, is described. Two of the siblings had hearing loss while of school age, but two others first exhibited otological symptoms in their thirties. One of the siblings had retinitis pigmentosa, diagnosed before the hearing impairment became evident. The family seems to exhibit type III Usher's syndrome with unusual manifestations. PMID:6641004

Karjalainen, S; Teräsvirta, M; Kärjä, J; Kääriäinen, H

1983-10-01

240

Electrocardiographic findings and cardiac manifestations in acute aluminum phosphide poisoning.  

PubMed

Aluminium phosphide (AlP) poisoning has a high mortality due to cardiovascular involvement. In this study, we evaluated the frequency of cardiac manifestations and electrocardiographic (ECG) findings in 20 patients with acute AlP poisoning, who were admitted to the intensive care unit (ICU) in Tehran, Iran, over a period of 6 months (between October 2008 and April 2009). The sex, age, cause and manner of ingestion, number of ingested AlP tablets, cardiac and ECG manifestations, creatine phosphokinase (CPK), CPK-myocardial band (CPK-mb) and troponin-T (TnT) were extracted from the patients' files. All data were analysed with Statistical Package for the Social Sciences (SPSS) software. The majority (60%) of patients were male. The mean age was 27 ± 8.7 years. The mortality rate was 40%. In all of the patients, the cause of poisoning was intentional suicide and ingestion was the route of exposure. The mean number of ingested AlP tablets per patient was 2.2 ± 1.1. The average time interval between admission and cardiovascular manifestations or ECG findings was 168.8 ± 116.2 min. The range of systolic (SBP) and diastolic blood pressure was 60-130 mmHg and 40-70 mmHg, respectively. Dysrhythmia was observed in nine (45%) cases. Elevation of the ST segment was seen in nine cases (45%). Seven patients (35%) had prolonged QTc intervals. Bundle branch block (BBB) was observed in four (20%) patients. In nine (45%) patients, the serum cardiac TnT qualitative assay was positive. There were no significant differences between normal and abnormal ECG groups according to sex, age, number and manner of ingested AlP tablets and SBP. There was a significant correlation between cardiac manifestations and ECG findings and TnT-positive results with mortality in acute AlP poisoning. PMID:22687771

Soltaninejad, Kambiz; Beyranvand, Mohammad-Reza; Momenzadeh, Seyed-Akbar; Shadnia, Shahin

2012-07-01

241

Premature centromere division: A possible manifestation of chromosome instability  

SciTech Connect

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

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

1995-03-13

242

Manifest duality invariance for the partially massless graviton  

NASA Astrophysics Data System (ADS)

For a special value of the mass, a massive graviton on de Sitter space acquires an enhanced scalar gauge symmetry, and is called partially massless. The partially massless graviton possesses a duality invariance akin to electromagnetic duality. We display this duality in its manifestly local and covariant form, in which it acts to interchange the first-order field equations and Bianchi identities of a gauge invariant field strength.

Hinterbichler, Kurt

2015-01-01

243

Multi-slice computed tomography manifestations of hepatic epithelioid angiomyolipoma  

PubMed Central

AIM: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate. METHODS: The multi-slice CT manifestations in five patients who were diagnosed with HEA definitely by postoperative pathological examination were analysed retrospectively. Three female patients and two male patients were included. Before operation, four patients received plain CT scanning and dynamic enhancement scanning, and the other patient only received enhancement scanning, with immunohistochemical analysis conducted after postoperative pathological examination. Four patients were misdiagnosed by CT, including three patients misdiagnosed with hepatic cell carcinoma and one patient with focal nodular hyperplasia. RESULTS: Upper abdominal multi-slice spiral CT scanning and three-stage enhancement scanning were conducted in five patients with HEA before operation. HEA had certain characteristic CT manifestations: low density masses, a few relatively high-density masses or fat-density masses diffusely shown in foci, clear boundary, round or oval and large focus, and tumour size ranging from 3.1 cm × 2.5 cm to 7.0 cm × 5.2 cm. During enhancement scanning, the foci were significantly enhanced uniformly or non-uniformly during the arterial phase, while during the venous and equilibrium phases, the foci were enhanced continuously or showed obvious low-density masses. Obviously enhanced and widened vessels could be found adjacent to foci or in the central area of foci during the arterial phase. CONCLUSION: CT manifestations of HEA have certain characteristics. Primary diagnosis can be obtained by combining CT findings with clinical data, but pathological examination is still needed for a definite diagnosis. PMID:24696616

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

2014-01-01

244

Abstract--Pathological manifestations of epilepsy are generally associated with a set of clinical events that  

E-print Network

Abstract--Pathological manifestations of epilepsy are generally associated with a set of clinical It is widely suggested that certain clinical manifestations of epilepsy are directly reflected in the changes

Slatton, Clint

245

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

Code of Federal Regulations, 2012 CFR

... false Electronic passenger and crew arrival manifests...4.7b Electronic passenger and crew arrival manifests...vessel being used to transport persons or property...which is being used to transport only passengers and/or...

2012-04-01

246

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

Code of Federal Regulations, 2013 CFR

... false Electronic passenger and crew arrival manifests...4.7b Electronic passenger and crew arrival manifests...vessel being used to transport persons or property...which is being used to transport only passengers and/or...

2013-04-01

247

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

Code of Federal Regulations, 2011 CFR

... false Electronic passenger and crew arrival manifests...4.7b Electronic passenger and crew arrival manifests...vessel being used to transport persons or property...which is being used to transport only passengers and/or...

2011-04-01

248

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

Code of Federal Regulations, 2014 CFR

... false Electronic passenger and crew arrival manifests...4.7b Electronic passenger and crew arrival manifests...vessel being used to transport persons or property...which is being used to transport only passengers and/or...

2014-04-01

249

14 CFR 121.697 - Disposition of load manifest, flight release, and flight plans: Supplemental operations.  

Code of Federal Regulations, 2010 CFR

...Disposition of load manifest, flight release, and flight plans: Supplemental operations...carry in the airplane to its destination the original or a signed copy of...1) Load manifest; (2) Flight release; (3)...

2010-01-01

250

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

Code of Federal Regulations, 2010 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)...

2010-04-01

251

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

Code of Federal Regulations, 2011 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...

2011-04-01

252

Dentomaxillofacial manifestations of Gaucher's disease: preliminary clinical and radiographic findings  

PubMed Central

Objectives A wide variety of manifestations is presented in patients with Gaucher's disease (GD), including bone, haematology and visceral disturbances. This study was conducted to ascertain the main maxillofacial abnormalities by means of clinical survey, panoramic and cone beam CT (CBCT); to compare the patient's group with an age–sex matched control group; and to correlate clinical and radiological data. Methods Ten patients previously diagnosed with GD were submitted to clinical and radiological surveys (CBCT and panoramic radiographs). The examination consisted of anamnesis, extra- and intraoral examinations and analyses of each patient's records. Imaging data were collected from the point of view of 3 observers, and the results compared with a healthy group (20 individuals) by means of statistical analysis (Fisher's exact test). Results Gaucher patients had significantly more manifestations than otherwise healthy carriers. The most prevalent findings were enlarged marrow spaces, generalized osteopenia and effacement of jaw structures (mandibular canal, lamina dura and mental foramen). Here we describe a case in which thickening of the maxillary sinus mucosa was observed on CBCT rather than opacification of the sinus as seen on panoramic radiographs. Pathological fractures, root resorption and delay on tooth eruption were not observed. Conclusions A poor relationship could be observed between clinical and radiological data. Patients showed important bone manifestations, which require careful diagnostic and surgical planning whenever necessary. Although panoramic radiographs have shown significant differences, CBCT is more effective in pointing out differences between patients and a control group, thus showing it as an important tool for evaluation of Gaucher patients. PMID:22988312

Nobre, RM; Ribeiro, ALR; Alves-Junior, SM; Tuji, FM; Rodrigues Pinheiro, M das G; Pinheiro, LR; Pinheiro, JJV

2012-01-01

253

Hiccups, eructation, and other uncommon prodromal manifestations of herpes zoster.  

PubMed

Although the most frequent presentation of herpes zoster involves sensory neurons, motor and autonomic symptomatology is also known to occur in this disease. An unusual symptom of hiccups is described here. Other infrequent manifestations of this common illness, including the Ramsay Hunt syndrome, herpes zoster ophthalmicus, urinary and fecal retention, sexual dysfunction, and zoster sine herpete, are reviewed. Greater awareness of unusual presentations of herpes zoster is necessary for proper diagnosis and timely management of complications that may otherwise lead to disability and serious long-term sequelae. PMID:14639397

Berlin, Alexander L; Muhn, Channy Y; Billick, Robin C

2003-12-01

254

Spectrum of X-ray manifestations in Cockayne's syndrome  

SciTech Connect

Cockayne's syndrome is a rare familial disorder characterised clinically by premature aging, appearing progressively from the third year on. The radiological manifestations of eight affected children have been studied and summarised. It is concluded that a skeletal survey can provide a roentgenologic pattern suggesting the diagnosis, even when it is inconclusive from the clinical signs during the first years of life. The aetiology of this syndrome is unknown, but the authors postulate the possible role of a defect of thymic hormone which has been found in all their cases.

Bensman, A.; Faure, C.; Kaufmann, H.J.

1981-12-01

255

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

256

A New Assumed Interaction. Experiments and Manifestations in Astrophysics  

E-print Network

Results of experimental investigations of a new assumed interaction in nature with the aid of high-current magnets, torsion and piezoresonance balances, high-precision gravimeter, fluctuations in intensity of betta-decay of radioactive elements, plasma devices and manifestations in astrophysics are presented. A possible explanation of the results obtained based on a hypothesis of global anisotropy of physical space caused by the existence of a cosmological vectorial potential A_g, is given. It is shown that the vector A_g has the following coordinates in the second equatorial coordinate system: right ascension alpha = 293 +- 10, declination delta = 36 +- 10.

Yu A. Baurov

2008-08-20

257

Spectroscopic manifestation of stretching vibrations of glycosidic linkage in polysaccharides  

NASA Astrophysics Data System (ADS)

Manifestation of stretching vibrations of glycosidic linkage in the infrared spectra of polysaccharides (native, microcrystalline, mercerized celluloses, amylose, starches) has been studied using the regularized method of deconvolution. It has been shown that the glycosidic linkage formation in the polysaccharides is characterized by the appearance of new absorption bands in the 1175-1140 cm -1 range as compared to their corresponding monomers. In the 1000-920 cm -1 region differences between the infrared spectra of polysaccharides due to the changes in the glycosidic linkage configuration have been found.

Nikonenko, N. A.; Buslov, D. K.; Sushko, N. I.; Zhbankov, R. G.

2005-10-01

258

Ocular manifestations in a child with systemic brucellosis  

PubMed Central

Brucellosis is a zoonotic disease with widespread prevalence. It presents with in various range and often with the presence of non-specific clinical signs and symptoms. Brucellosis also may cause different manifestations in eyes such as uveitis, keratitis, conjunctivitis and neuro-ophthalmic defects. Ocular brucellosis is rare among children. Herein, we present a 7-year-old girl with systemic and ocular brucellosis. After treatment with systemic steroid and antibiotics, her signs and symptoms disappeared. Since early treatment is important in preventing permanent visual loss and the other complications of ocular brucellosis, examination of the eyes in brucellosis patients must always be noticed by clinicians working in this field. PMID:25364370

Mohammadi, Zahra; Dehghani, Alireza; Ghanbari, Heshmat Ollah; Akhlaghi, Mohammad Reza; Nasrollahi, Kobra; Salam, Hasan

2014-01-01

259

Complete atrioventricular block as initial manifestation of systemic lupus erythematosus.  

PubMed

Only a few cases of complete atrioventricular block (AVB) in adult lupus patients have been previously described, but only one as the initial manifestation. A 19-year-old woman who presented with seizures and loss of consciousness, was diagnosed with complete ABV and underwent pacemaker placement. Over the next weeks she developed serositis, joint, cutaneous, and renal involvement; positive antinuclear antibodies and high anti-SSA/Ro titers. This is the second case with AVB as a feature of SLE at onset. A review of previous complete AVB cases of adult SLE patients is presented. PMID:19473581

Arce-Salinas, C A; Carmona-Escamilla, M A; Rodríguez-García, F

2009-01-01

260

Lacrimal Sac Cysticercosis: A Rare Site for Manifestation  

PubMed Central

Cysticercosis, a parasitic tissue infestation caused by larval cysts (cysticercus cellulosae) of the pork tapeworm, Taenia solium, most commonly affects the central nervous system, subcutaneous tissue, skeletal muscle, heart muscle, and the eye. Ocular adnexal infestation in cysticercosis can involve various sites within the eye like vitreous humor, subretinal tissue, extraocular muscle, and lacrimal gland. In this case report, a lump slightly below and medial to inner corner of eye, surgically removed from a 21-year-old male patient, was diagnosed as case of lacrimal sac cysticercosis on histopathological examination. To our knowledge, this is the one of rare sites for manifestation of cysticercus cellulosae. PMID:25478267

Raoot, Amita

2014-01-01

261

Pulmonary Nodules as an Initial Manifestation of Behçet's Disease  

PubMed Central

Behçet's disease (BD) is a systemic vasculopathy, characterized by recurrent oral aphthae, genital ulcers, uveitis, and skin lesions. Although vascular involvement, including venous and arteries of any size, is a usual manifestation, cases with pulmonary thrombosis as the initial symptom are not common in the absence of pulmonary artery aneurysm (PAA). This report describes a 36-year-old man with recurrent fever, nonmassive hemoptysis, and persistent cough with lung nodules in CT scan who had undergone open lung biopsy. On the basis of morphological findings, BD was suggested and more precise evaluation confirmed the diagnosis. PMID:25436168

Malekmohammad, M.; Emamifar, A.

2014-01-01

262

Ischemic stroke as the first manifestation of hepatic epithelioid hemangioendothelioma.  

PubMed

A 38-year-old obese woman, with a past medical history of cholecystectomy and dyslipidaemia, presented with acute occipital headache, vomiting and rotational vertigo which lasted 8 hours. On admission neurological examination was unremarkable, however general physical examination revealed hepatomegaly. Routine blood tests showed abnormal liver function tests. MRI scan indicated an acute ischemic infarct in the right cerebellum. Extensive investigation was negative. However, liver MRI revealed multiple lesions in both liver lobes. Ultrasound guided liver biopsy and histopathological analysis confirmed the diagnosis of hepatic hemangioendothelioma. In conclusion, hypercoaguable state related to hepatic epithelioid hemangioendothelioma can lead to an ischemic stroke, as a rare first manifestation of the disease. PMID:24231139

Zis, Panagiotis; Assi, Avraam; Kravaritis, Dimitrios; Sevastianos, Vassilios A

2014-03-01

263

HIV infection-related tuberculosis: clinical manifestations and treatment.  

PubMed

Several aspects of human immunodeficiency virus (HIV) infection-related tuberculosis (TB) and its treatment differ from those of TB in HIV-uninfected persons. The risk of TB and the clinical and radiographic manifestations of disease are primary examples. Antiretroviral therapy has a profound effect on lowering the risk of TB in HIV-infected persons, but it can also be associated with immune reconstitution inflammatory disease and unmasking of previously subclinical disease. There are also differences in treatment of HIV infection-related TB because of overlapping drug toxicities and drug-drug interactions between antiretroviral therapy and anti-TB therapy. PMID:20397952

Sterling, Timothy R; Pham, Paul A; Chaisson, Richard E

2010-05-15

264

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

265

Automated Detection of Anomalous Shipping Manifests to Identify Illicit Trade  

SciTech Connect

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

Sanfilippo, Antonio P.; Chikkagoudar, Satish

2013-11-12

266

Peripheral Nervous System Manifestations in Systemic Autoimmune Diseases  

PubMed Central

The peripheral nervous system refers to parts of the nervous system outside the brain and spinal cord. Systemic autoimmune diseases can affect both the central and peripheral nervous systems in a myriad of ways and through a heterogeneous number of mechanisms leading to many different clinical manifestations. As a result, neurological complications of these disorders can result in significant morbidity and mortality. The most common complication of peripheral nervous system (PNS) involvement is peripheral neuropathy, with symptoms of numbness, sensory paresthesias, weakness, or gait imbalance. The neuropathy may be multifocal and asymmetric or, less frequently, distal and symmetric. PMID:25705295

COJOCARU, Inimioara Mihaela; COJOCARU, Manole; SILOSI, Isabela; VRABIE, Camelia Doina

2014-01-01

267

Manifestations bucco-dentaires de la sclérodermie systémique  

PubMed Central

Nous rapportons l'observation d'une jeune femme de 26 ans ayant une sclérodermie systémique diffuse présentant une atteinte bucco-dentaire complexe: microstomie, hyperplasie gingivale, parodontite, dépôts tartriques, caries multiples et chevauchement dentaire antéro-inférieur. Nous discuterons à travers cette observation les manifestations bucco-dentaires de cette connectivite qui sont loin d’être rares mais souvent négligées par les cliniciens malgré leur retentissement fonctionnel majeur. Il convient de surveiller régulièrement l’état bucco-dentaire chez tout patient sclérodermique afin de diagnostiquer précocement ces atteintes. Diagnostiquées à un stade tardif les complications bucco-dentaires de la sclérodermie seront très difficiles à traiter. PMID:24778751

Salem, Bouomrani; Rim, Bel Hadj Ali; Sihem, Ben Khoud; Maher, Béji

2013-01-01

268

Specificity of psychiatric manifestations in relation to neurotoxic chemicals.  

PubMed

Previous impressions of specificity of psychiatric manifestations in relation to particular chemical intoxications have been confirmed by comparisons of the symptoms and signs of two groups of individuals. Nine persons exposed to inorganic mercury had "erethism" and xenophobia in addition to non-specific features of central nervous system poisoning. Twelve men with heavy exposure to organotins, in contrast to ten men with light or no exposure, more frequently presented an unique alternation between outbursts of range and deep depression, the later lasting from a few hours to a few days. The more heavily exposed men also had a greater number of nonspecific symptoms from neurotoxins. PMID:6575580

Ross, W D; Sholiton, M C

1983-01-01

269

Atypical Focal Osteomyelitis as Initial Manifestation of AIDS  

PubMed Central

Persistent pain development after a skeletal contusion rarely poses the diagnosis of osteomyelitis. We report the case of a fibular head contusion as an initial manifestation of a focal abscess development in a healthy young patient. The traditional treatment of surgical drainage revealed the presence of an atypical Mycobacterium haemophilum isolates in the abscess. This lead to further investigations that concluded and established the diagnosis of AIDS. Conclusion. Isolation of an atypical Mycobacterium haemophilum in any abscess should lead the physician to accomplish further investigations and look for AIDS even in young healthy subjects. PMID:23198217

Akiki, A.; Bilde, Y.

2011-01-01

270

Beckwith-Wiedemann syndrome with overlapping Perlman syndrome manifestation.  

PubMed

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome known as exomphalos-macroglossia - gigantism syndrome. Prognosis is good, prenatal diagnosis is important for pregnancy management but might be difficult due to clinical overlap with other syndromes. Perlman syndrome is an overgrowth syndrome with high perinatal mortality, most frequent antenatal findings include polyhydramnios, macrosomia, visceromegaly, nephromegaly and foetal ascites. Authors present a case of prenatally diagnosed BWS with severe ascites as first antenatal finding and lethal course, signs more typical of Perlman syndrome. This combination of clinical signs has not been published yet and may contribute to specification of possible prenatal manifestation of BWS. PMID:24215131

Ferianec, Vladimír; Bartova, Michaela

2014-10-01

271

78 FR 52958 - Announcement of Test Concerning Manifesting and Entry of Residue Found in Instruments of...  

Federal Register 2010, 2011, 2012, 2013, 2014

...any empty container manifested in this fashion and will not be required under the Residue...any empty container manifested in this fashion and will not be required under the Residue...any empty container manifested in this fashion and will not be required under the...

2013-08-27

272

Musculoskeletal manifestations of mild osteogenesis imperfecta in the adult.  

PubMed

The musculoskeletal manifestations of mild forms of osteogenesis imperfecta are not well defined in the adult. The aim of this study was to characterize the musculoskeletal manifestations and resulting impairments reported by adults with mild osteogenesis imperfecta. For this task a survey of musculoskeletal symptoms and impairments was hosted on the Osteogenesis Imperfecta Foundation web site for 6 weeks. Survey responses are reported herein. There were 111 unduplicated, adult respondents (78 female). Mean age was 40.8 years. More than one-quarter of 3,410 lifetime fractures occurred in adulthood. Nearly half of respondents reported an established diagnosis of "arthritis" (usually osteoarthritis), and the majority of these reported some degree of impairment attributable to arthritis. Articular pain, stiffness and instability were dominant in the large, weight-bearing joints of the lower extremities. Back pain and scoliosis were common. Of the respondents, 15% required assistance with light physical tasks and personal care. Two-thirds reported joint hyper-mobility, and one-third reported a previous tendon rupture. Complex regional pain syndrome was rare. Respondents reported frequent use of medications known to have potential adverse skeletal effects. In spite of these concerns the majority rated their overall physical health as good or excellent. Adults with mild osteogenesis imperfecta continue to sustain fractures into adulthood, and the majority reports some functional impairment due to musculoskeletal issues. Significant impairment is not rare. PMID:15902417

McKiernan, Fergus E

2005-12-01

273

Neural Manifestations of Implicit Self-Esteem: An ERP Study  

PubMed Central

Behavioral research has established that humans implicitly tend to hold a positive view toward themselves. In this study, we employed the event-related potential (ERP) technique to explore neural manifestations of positive implicit self-esteem using the Go/Nogo association task (GNAT). Participants generated a response (Go) or withheld a response (Nogo) to self or others words and good or bad attributes. Behavioral data showed that participants responded faster to the self paired with good than the self paired with bad, whereas the opposite proved true for others, reflecting the positive nature of implicit self-esteem. ERP results showed an augmented N200 over the frontal areas in Nogo responses relative to Go responses. Moreover, the positive implicit self-positivity bias delayed the onset time of the N200 wave difference between Nogo and Go trials, suggesting that positive implicit self-esteem is manifested on neural activity about 270 ms after the presentation of self-relevant stimuli. These findings provide neural evidence for the positivity and automaticity of implicit self-esteem. PMID:25006966

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

2014-01-01

274

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

275

[Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.  

PubMed

Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m(2)/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m(2)) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS. PMID:25440709

Couto, Saulo B; Sallum, Adriana M; Henriques, Luciana S; Malheiros, Denise M; Silva, Clovis A; Vaisbich, Maria H

2014-10-22

276

Initial Cutaneous Manifestations of Hutchinson-Gilford Progeria Syndrome  

PubMed Central

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

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

2014-01-01

277

[Molecular pathology and clinical manifestations of Fabry disease].  

PubMed

Fabry disease is a rare, progressive lysosomal storage disorder caused by mutation in the GAL gene and an impaired function of the alpha-galactosidase A enzyme. The enzymatic defect results in the progressive accumulation of glycosphingolipids in endothelial cells, smooth muscle cells, leucocytes and fibroblasts leading to organ damage in the skin, eye, nervous system, kidney and heart. Major clinical manifestations include acroparesthesis, angiokeratoma, corneal opacities, vascular diseases of the heart, kidney, and the central nervous system. Enzyme replacement therapy has recently become available for the treatment of Fabry patients. In this review the authors describe clinical features of Fabry disease in 31 Hungarian patients. At the time of this analysis the database consisted of 31 cases (15 males, 16 females) of whom 5 have died (4 males, 1 female). The most common disease-specific manifestation was angiokeratoma in males, and eye symptoms in females. 25% of female subjects were symptom free. Genotyping was performed in all cases and disease-causing mutations were found in all families. Three new mutations were identified. Twelve patients (8 males and 4 females) are currently receiving enzyme replacement therapy. PMID:17545117

Rákóczi, Eva; Görögh, Sándor; Grubits, János; Erdos, Melinda; Garzuly, Ferenc; Hahn, Katalin; Bencsik, Krisztina; Vécsei, László; Trinn, Csilla; Kristóf, Eva; Mogyorósy, Gábor; Tóth, Beáta; Maródi, László

2007-06-10

278

Neurological manifestations of the Mendelian-inherited autoinflammatory syndromes.  

PubMed

Autoinflammatory syndromes include an expanding list of conditions characterized by unprovoked recurrent attacks of systemic inflammation with lack of auto-antibodies or autoreactive T cells. Many of these syndromes are genetic diseases with a Mendelian inheritance. Neurological manifestations may be one of the major clinical features and, in some cases, the presenting symptom of these syndromes. The purpose of this review is to increase the recognition among neurologists of the Mendelian-inherited autoinflammatory syndromes by highlighting the neurological manifestations in the context of other symptoms that should lead physicians to suspect these syndromes. Most important for neurologists are the cryopyrin-associated periodic syndromes that include familial cold autoinflammatory syndrome, Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease (called chronic infantile neurological cutaneous and articular syndrome in Europe). We also review other syndromes with less common neurological involvement, including familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, and hyperimmunoglobulinemia D syndrome. Because these syndromes are often treatable and irreversible damage is prevented if they are treated early, it is important to recognize the features that may result in these syndromes presenting to a neurologist, especially in early childhood. PMID:19563585

Montealegre Sanchez, Gina A; Hashkes, Philip J

2009-06-01

279

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

PubMed

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

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

2014-05-01

280

Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome.  

PubMed

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

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

2014-01-01

281

Autoimmune bullous skin diseases. Part 1: Clinical manifestations.  

PubMed

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

Kneisel, Andrea; Hertl, Michael

2011-10-01

282

Skin and Systemic Manifestations of Jellyfish Stings in Iraqi Fishermen  

PubMed Central

Background: Jellyfish stings are common worldwide with an estimated 150 million cases annually, and their stings cause a wide range of clinical manifestations from skin inflammation to cardiovascular and respiratory collapse. No studies on jellyfish stings have been carried out in Basra, Iraq. Objectives: To describe the immediate and delayed skin reactions to White Jellyfish (Rhizostoma sp.) stings and the types of local treatment used by fishermen. Methods and Materials: 150 fishermen were enrolled at three Marine stations in Basra, Iraq. Demographic data, types of skin reactions, systemic manifestations and kinds of treatments were collected. Results: Overall, 79% of fishermen in all three Marine stations gave a history of having been stung. The common sites of sings were the hands and arms followed by the legs. Most fishermen claimed that stings led to skin reactions within 5 minutes. The presenting complaints were itching, burning sensation, and erythematic wheals. A few days after the sting, new groups of painless and itchy erythematous monomorphic papular rashes developed at the site of the sting in 62% of cases as a delayed type of skin reaction that resolved spontaneously. The local remedies commonly used by the fishermen were seawater, tap water and ice. A few fishermen considered stings as insignificant and did not think there was a need to seek medical help. Conclusions: We conclude that jellyfish causes many stings among fishermen in the Basra region. Their stings lead to immediate and delayed skin reactions. Self-treatment by topical remedies is common. PMID:21483513

Al-Rubiay, KK; Al-Musaoi, HA; Alrubaiy, L; Al-Freje, MG

2009-01-01

283

What we know about ocular manifestations of Ebola  

PubMed Central

Ebola hemorrhagic fever is a deadly disease caused by several species of ebolavirus. The current outbreak of 2014 is unique in that it has affected a greater number of people than ever before. It also has an unusual geographic distribution. Nonspecific findings such as fever and generalized weakness have traditionally been very common early in the acute phase. Ophthalmic manifestations have also been reported in significant numbers. Conjunctival injection has been identified in both the acute and late phases. Subconjunctival hemorrhage and excessive lacrimation have also been reported. Various forms of uveitis have been associated with the convalescent phase of the disease. When identified in conjunction with other signs such as fever, acute findings such as conjunctivitis may contribute to the diagnosis of Ebola hemorrhagic fever. Ideally, serologic testing should be performed prior to isolation and treatment of these individuals. Considering the prevalence of the current outbreak and the threat of transcontinental spread, ophthalmic health professionals need to be aware of the ocular manifestations of Ebola hemorrhagic fever as well as the associated signs and symptoms in order to prevent further spread. PMID:25473261

Moshirfar, Majid; Fenzl, Carlton R; Li, Zhan

2014-01-01

284

Extrahepatic manifestations of chronic hepatitis C virus infection.  

PubMed

Hepatitis C virus (HCV) infected patients are known to be at risk of developing liver complications i.e. cirrhosis and liver cancer. However, the risks of morbidity and mortality are underestimated because they do not take into account non-liver consequences of chronic hepatitis C virus infection. Numerous extrahepatic manifestations have been reported in up to 74% of patients, from perceived to disabling conditions. The majority of data concern hepatitis C virus-related autoimmune and/or lymphoproliferative disorders, from mixed cryoglobulinaemia vasculitis to frank lymphomas. More recently, other hepatitis C virus-associated disorders have been reported including cardiovascular, renal, metabolic, and central nervous system diseases. This review aims to outline most of the extrahepatic manifestations that are currently being investigated, including some of autoimmune and/or lymphoproliferative nature, and others in which the role of immune mechanisms appears less clear. Beyond the liver, hepatitis C virus chronic infection should be analyzed as a multifaceted systemic disease leading to heavy direct and indirect costs. The accurate consideration of extrahepatic consequences of such a systemic infection significantly increases the weight of its pathological burden. The need for effective viral eradication measures is underlined. PMID:25458776

Cacoub, Patrice; Gragnani, Laura; Comarmond, Cloe; Zignego, Anna Linda

2014-12-15

285

Quantum mechanics and the manifestation of the world  

E-print Network

Quantum theory's irreducible empirical core is a probability calculus. While it presupposes the events to which (and on the basis of which) it serves to assign probabilities, and therefore cannot account for their occurrence, it has to be consistent with it. It must make it possible to identify a system of observables that have measurement-independent values. What makes this possible is the incompleteness of the spatiotemporal differentiation of the physical world. This is shown by applying a novel interpretive principle to interfering alternatives involving distinctions between regions of space. Applying the same interpretive principle to alternatives involving distinctions between things makes it safe to claim that the macroworld comes into being through a progressive differentiation of a single, intrinsically undifferentiated entity. By entering into reflexive spatial relations, this entity gives rise to (i) what looks like a multiplicity of relata if the reflexive quality of the relations is not taken into account, and (ii) what looks like a substantial expanse if the spatial quality of the relations is reified. The necessary distinction between two domains (classical and quantum, or macro and micro) and their mutual dependence is best understood as a distinction between the manifested world and its manifestation.

Ulrich Mohrhoff

2014-08-23

286

An atlas of the morphological manifestations of hidradenitis suppurativa.  

PubMed

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

Scheinfeld, Noah

2014-01-01

287

Isolated facial hemihyperplasia: manifestation of Beckwith-Wiedemann syndrome.  

PubMed

Facial asymmetry is a common finding in infants and can be the result of a number of distinctive conditions such as hemifacial microsomia, overgrowth syndromes, a soft tissue tumor, and a vascular malformation. However, overgrowth syndromes such as Beckwith-Wiedemann syndrome (BWS) typically manifest more extensive involvement; it rarely presents as isolated facial overgrowth.Here, we present a 7-year-old boy who presented with facial asymmetry. He was found to have isolated facial hemihyperplasia, involving his right cheek and teeth. No abnormalities were seen in the rest of his examination. The diagnosis of BWS was considered and was confirmed by detection of a methylation abnormality in H19 (DMR1). This case demonstrates that BWS should be considered, even with isolated facial involvement. This is important, as affected patients are predisposed to certain malignancies, especially in the first 5 to 8 years of life. Therefore, specialized surveillance is recommended as the part of management. PMID:18216702

Sathienkijkanchai, Achara; Prucka, Sandra K; Grant, John H; Robin, Nathaniel H

2008-01-01

288

Misorientation/local plastic strain manifestations in chemical etching color.  

PubMed

Cold plastic deformation produces misorientations inside the crystal grains, and the distribution of the misorientation is quite crucial to understand the deformation behavior of the metals or alloys. The misorientation manifestations in chemical etching contrast are investigated in this study in the case of cold-deformed iron. The chemical etching is performed by using nital, while the crystal orientation is determined by electron backscatter diffraction (EBSD). The correlation between the chemical etching contrast and crystal orientation have been studied in both cold-deformed and undeformed iron. The results clearly show that the chemical etching contrast strongly reflects the crystallographic orientation. The gradual change in chemical etching contrast inside the individual deformed grains gives information of both the misorientation and local plastic strain within the grains. This method can provide an easy and alternative way to qualitatively understand the misorientation and local plastic strain distributions in the microstructures. PMID:24530362

Zhang, Xianguang; Matsuura, Kiyotaka; Ohno, Munekazu

2014-04-01

289

[Cardiac manifestations of Lyme disease. Apropos of 2 cases].  

PubMed

Lyme disease, a well-known entity in the United States, has featured only rarely in the French literature. We report two cases from the Cardiology Department with acute symptomatic atrio-ventricular block. The illness begins with migratory chronic erythema, which is the best marker of the condition. This is followed by neurological, joint and cardiac manifestations. Cardiac involvement consists essentially in A-V block of varying severity, which rapidly regresses. Until the recent discovery of the arthropod vector responsible (Ixodes dammini), the diagnosis was clinical and was confirmed by negative serological results for the other infectious agents. Penicillin and tetracycline are effective at the skin lesion stage. It is important to recognise this condition since there is no case of complete A-V block lasting longer than 2 weeks. PMID:6497303

Cornuau, C; Bernard, M; Daumas, P L; Oblet, B; Poirot, G; Valois, M

1984-10-01

290

Clinical Manifestations and Diagnostic Challenges in Acute Porphyrias  

PubMed Central

The porphyrias are a group of disorders characterized by an enzyme deficiency in the heme biosynthetic pathway. These can be classified into either erythropoietic or hepatic forms depending on the site of the major enzyme deficiency. The diagnosis of acute porphyrias, however, can be very challenging due to overlapping features amongst the various types. Initial suspicion is based on a myriad of clinical manifestations, which then are confirmed by laboratory testing where available. Genetic testing is now also available for the different types of porphyrias, aiding in the definitive diagnosis. Here, we present a challenging case of porphyria in a patient with end-stage renal disease and present the diagnostic challenges associated with the case and the ways forward. PMID:23476835

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

2013-01-01

291

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

2012-01-01

292

Cervical mass as the presenting manifestation of hepatocellular carcinoma  

PubMed Central

Background: Hepatocellular carcinoma is one of the most common cancers in the world due to its association with chronic hepatitis infections. Amongst the most usual metastasis of hepatocellular carcinoma are the lymph nodes, the lungs and the bones. Soft tissue metastases are extremely rare. Case presentation: Herein, we report the case of a 73-years-old male patient who presented with cervical and upper extremities muscle pain along with paresthesias and a palpable mass on the cervical region. Conclusion: This unusual clinical manifestation along with the above-described rare presentations of hepatocellular carcinoma must be taken into account, especially among patients with chronic hepatitis infections. Hippokratia 2014; 18 (3): 285-287. PMID:25694768

Vallianou, NG; Gounari, P; Skourtis, A; Vourlakou, C

2014-01-01

293

Star copolymers in porous environments: scaling and its manifestations  

E-print Network

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)\\sim 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 \\epsilon=4-d, \\delta=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.

Viktoria Blavatska; Christian von Ferber; Yurij Holovatch

2010-10-11

294

Dengue encephalitis-A rare manifestation of dengue fever  

PubMed Central

The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue. The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with fever, altered sensorium and seizures. Dengue NS-1 antigen test was reactive. Dengue IgM was also positive. CSF PCR was negative for herpes simplex 1 & 2. Dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant. PMID:25183150

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

2014-01-01

295

Oral manifestations of thyroid disorders and its management  

PubMed Central

The thyroid is the major regulator of metabolism and affects all of the bodily functions. Thyroid dysfunction is the second most common glandular disorder of the endocrine system which may rear its head in any system in the body including the mouth. The oral cavity is adversely affected by either an excess or deficiency of these hormones. Before treating a patient who has thyroid disorder, the endocrinologist needs to be familiar with the oral manifestations of thyroid dysfunctions. The patient with a thyroid dysfunction, as well as the patient taking medications for it, requires proper risk management before considering dental treatment by the dentist. Thus, communication of dentist with endocrinologist must be bidirectional, to maintain patient's oral and thyroid health. PMID:21966646

Chandna, Shalu; Bathla, Manish

2011-01-01

296

Physicochemical Properties of Nanomaterials: Implication in Associated Toxic Manifestations  

PubMed Central

Nanotechnology has emerged as one of the leading fields of the science having tremendous application in diverse disciplines. As nanomaterials are increasingly becoming part of everyday consumer products, it is imperative to assess their impact on living organisms and on the environment. Physicochemical characteristics of nanoparticles and engineered nanomaterials including size, shape, chemical composition, physiochemical stability, crystal structure, surface area, surface energy, and surface roughness generally influence the toxic manifestations of these nanomaterials. This compels the research fraternity to evaluate the role of these properties in determining associated toxicity issues. Reckoning with this fact, in this paper, issues pertaining to the physicochemical properties of nanomaterials as it relates to the toxicity of the nanomaterials are discussed. PMID:25165707

Gatoo, Manzoor Ahmad; Naseem, Sufia; Arfat, Mir Yasir; Mahmood Dar, Ayaz; Qasim, Khusro

2014-01-01

297

Cutaneous manifestations in children with diabetes mellitus and obesity.  

PubMed

Obesity and diabetes are chronic diseases that affect people all over the world, and their incidence is increasing in both children and adults. Clinically, they affect a number of organs, including the skin. The cutaneous manifestations caused or aggravated by obesity and diabetes are varied and usually bear some relation to the time that has elapsed since the onset of the disease. They include soft fibromas, acanthosis nigricans, striae, xerosis, keratosis pilaris, plantar hyperkeratosis, fungal and bacterial skin infections, granuloma annulare, necrobiosis lipoidica, psoriasis, and atopic dermatitis. In this review article we present the skin changes found in children with diabetes mellitus and obesity and related syndromes and highlight the importance of the skin as a tool for establishing clinical suspicion and early diagnosis of systemic disease. PMID:24698434

Baselga Torres, E; Torres-Pradilla, M

2014-01-01

298

Massive pericardial effusion as the only manifestation of primary hypothyroidism  

PubMed Central

Small pericardial effusion (PE) is not an infrequent manifestation in primary hypothyroidism. But massive PE with or without cardiac tamponade is rare and often associated with severe form of the disease. Here we report an eight-year-old boy who was admitted with massive PE that required repeated pericardiocentesis. Detailed examinations failed to identify the etiology initially. Five months later, child was readmitted with massive PE with impending cardiac tamponade. Primary hypothyroidism was diagnosed based on the clinical and laboratory finding and was thought to be the underlying etiology of previously encountered undiagnosed massive PE. Beside pericardiocentesis, child was treated with thyroid hormone replacement. Condition gradually improved without further recurrence of PE till date. Therefore, irrespective of the presence of clinical signs, primary hypothyroidism should be suspected in every patient presenting with massive PE to prevent recurrence as well as its serious complications like cardiac tamponade. PMID:24653591

Purkait, Radheshyam; Prasad, Anand; Bhadra, Ramchandra; Basu, Arindam

2014-01-01

299

Effect of periodontitis on cardiovascular manifestations in marfan syndrome.  

PubMed

Marfan syndrome (MFS) is a systemic connective tissue disorder that is caused by mutations in the extracellular matrix protein fibrillin-1. While MFS patients are considered to be at high risk of dental disorders and cardiovascular complications, little causal relationship has been provided to date. It is well known that an elevated level of active TGF-? in the plasma is a major manifestation of MFS. TGF-? is known to play a critical role in the development of cardiovascular diseases and its levels were also elevated in the serum and saliva of periodontitis patients. These findings may suggest an association between periodontitis and the cardiovascular complications of MFS. In this article, we review the influence of periodontitis in MFS patients with cardiovascular complications in order to identify critical therapeutic targets of TGF-?. PMID:25740392

Suzuki, Jun-Ichi; Aoyama, Norio; Izumi, Yuichi; Isobe, Mitsuaki; Komuro, Issei; Hirata, Yasunobu

2015-03-20

300

[Severe initial manifestations of psoriasis in staphylococcal infections].  

PubMed

Psoriasis vulgaris is a chronic disease with certain restrictions of life quality, but rarely life threatening. However, psoriatic erythroderma or pustular psoriasis can be a diagnostic or therapeutic challenge, especially when it arises as the primary manifestation of disease. Here we report on two patients with severe clinical course of pustular psoriasis or psoriatic erythroderma, both possibly induced by Staphylococcus aureus. This microorganism was cultivated from skin scrapings in both patients, in the patient with pustular psoriasis from the blood, and in the patient with erythroderma from the nose. Both patients had anti-staphylolysin antibodies. Both patients showed resolution of their disease only by the action of systemic antibiotics. We suggest that staphylococcal superantigens which activate T-cells might play an important role in severe psoriasis by inducing a systemic Köbner phenomenon and thus a progression of disease. PMID:11715754

Tunus, G; Pilger, U; Aberer, E

2001-10-15

301

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

PubMed

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

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

2000-07-01

302

Oral manifestations of epidermolysis bullosa dystrophica: a rare genetic disease.  

PubMed

Epidermolysis bullosa (EB) constitutes a group of phenotypically diverse genodermatoses, which manifests with blistering and erosions of the skin and mucous membranes as the unifying diagnostic feature. It is an acquired disease or inherited as either autosomal-dominant or recessive with an incidence of 1/50 000. The prominent clinical characteristic of the disease is the development of bullae or vesicles in mucosa or skin in response to minor trauma. It is a chronic mechanobullous disease characterised by auto antibodies against Type VII collagen. This paper documents a case of a man diagnosed with dominant dystrophic EB; describing the measures that dentists and healthcare providers should adopt in order to provide a safe and effective dental treatment. Early prevention protocols for these patients have also been discussed. PMID:23349175

Parushetti, Anita Dundappa; Agrawal, Jiwanasha Manish; Nanjannawar, Lalita Girish; Agrawal, Manish Suresh

2013-01-01

303

[Hypoparathyroidism and hypogonadism as a clinical manifestation of hemochromatosis].  

PubMed

Primary hypoparathyroidism is a disease in which the parathyroid glands decrease or cease secretion of parathyroid hormone, leading to clinically significant hypocalcemia. Although considered a rare condition, hypoparathyroidism seems to occur much more frequently than reported. In most cases, hypoparathyroidism remains a complication of neck surgery. However, many other pathologic conditions may be implicated in its pathogenesis. Here we present a case of a patient in whom hypoparathyroidism developed as a complication of hereditary hemochromatosis and was followed by hypogonadism, representing an atypical clinical manifestation of this disorder. In the article, we describe the diagnostic and treatment strategies undertaken in this patient. Our case study suggests that every person with hypoparathyroidism of unknown origin, particularly if this disorder is accompanied by abnormal gonadal function, should be assessed for the possible presence of hereditary hemochromatosis. PMID:23789304

Krysiak, Robert; Okopie?, Bogus?aw

2013-01-01

304

Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation  

PubMed Central

Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown pathogenesis. The frequency of SLE with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism. A 19-year-old female diagnosed with SLE complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report. Other diseases that can lead to such lesions were ruled out in the patient. The patient improved briefly after the initiation of immunosuppressive therapy, but was unresponsive to supportive treatment due to pneumothorax. Pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas – these later caused respiratory distress and death. The patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy. This case report suggests that the differential diagnosis of cavitary lung lesions should include SLE.

Dalili, Amir Reza; Lotfi, Reza; Mousavi, Seyedeh Maryam

2014-01-01

305

Orbital tuberculosis manifesting with enophthalmos and causing a diagnostic dilemma.  

PubMed

A 60-year-old woman with no known systemic disease was referred with a hard mass in the left orbit and enophthalmos of two months duration. Differential diagnoses of metastasis from an undetected scirrhous carcinoma and sclerosing nonspecific orbital inflammatory disease were considered and a biopsy was performed. Histopathology demonstrated granulomatous inflammation with fibrosis. Subsequent polymerase chain reaction was positive for Mycobacterium tuberculosis deoxyribonucleic acid. There was no evidence of systemic tuberculosis. The patient was treated with four-drug combination anti-tubercular therapy for 6 months. The mass regressed and there was no local recurrence at two years follow-up. Orbital tuberculosis generally manifests with proptosis or osteomyelitis. However, enophthalmos may be caused by the fibrosing variant. Biopsy with histopathologic and microbioloic evaluation is essential to distinguish it from other more common causes of an orbital mass with paradoxical enophthalmos such as metastatic scirrhous carcinoma and sclerosing nonspecific orbital inflammatory disease. PMID:16714937

Shome, Debraj; Honavar, Santosh G; Vemuganti, Geeta K; Joseph, Joveeta

2006-01-01

306

Pulmonary cystic disease associated with integumentary and renal manifestations.  

PubMed

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

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

2013-11-01

307

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. PMID:21217874

Dubovický, Michal

2010-01-01

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. PMID:23157966

2012-01-01

309

Marfan's syndrome. Broad spectral surgical treatment cardiovascular manifestations.  

PubMed Central

Most patients with Marfan's syndrome have cardiovascular manifestations and complications of these abnormalities lead to death in 50% of patients by the age of 32. This report is concerned with the performance of 79 operations to control these problems in 41 patients during a 16-year period. There were 3 early deaths and 11 late deaths, with survival at 15 years in 62%. The cardiovascular manifestations assumed eight patterns of involvement, in brief, consisting of aneurysms of ascending aorta, mitral valve insufficiency, aortic dissection and dissecting aortic aneurysm, and degenerative distal fusiform aortic aneurysm. These lesions occurred in isolated form or in association with others, hence the larger number of patterns of involvement. Various methods were employed in treatment of aneurysms of the ascending aorta, which was associated with aortic valvular insufficiency in most. The most reliable method of controlling disease at this level was composite valve graft replacement of fusiform aneurysms and separate valve graft operation for dissections occurring in the previously uninvolved aorta. Isolated mitral valve insufficiency was relieved by standard mitral valve replacement and this operation could be safely combined with composite valve graft replacement of the ascending aorta. More distant aneurysms of the aorta, either degenerative or chronic dissections, were susceptible to reconstruction as employed in the nonMarfan patient. Treatment of multiple lesions was staged, treating the more symptomatic condition first. Regular follow-up examination is important in these patients to detect new lesions and to evaluate known lesions. An aggressive approach is suggested in their treatment because 63% of the 11 late deaths in this series were due to lesions that could be successfully treated by presently available methods. Images Fig. 11. Fig. 11. Fig. 9. Fig. 9. Fig. 10. Fig. 2. Fig. 3. Fig. 4. Fig. 5. Fig. 7. Fig. 12. Fig. 13. Fig. 14. Fig. 15. Fig. 16. PMID:6625720

Crawford, E S

1983-01-01

310

Aspergillus Osteomyelitis: Epidemiology, Clinical Manifestations, Management, and Outcome  

PubMed Central

Background The epidemiology, pathogenesis, diagnosis, and management of Aspergillus osteomyelitis are not well understood. Methods Protocol-defined cases of Aspergillus osteomyelitis published in the English literature were reviewed for comorbidities, microbiology, mechanisms of infection, clinical manifestations, radiological findings, inflammatory biomarkers, antifungal therapy, and outcome. Results Among 180 evaluable patients, 127 (71%) were males. Possible predisposing medical conditions in 103 (57%) included pharmacological immunosuppression, primary immunodeficiency, and neutropenia. Seventy-three others (41%) had prior open fracture, trauma or surgery. Eighty (44%) followed a hematogenous mechanism, 58 (32%) contiguous infections, and 42 (23%) direct inoculation. Aspergillus osteomyelitis was the first manifestation of aspergillosis in 77%. Pain and tenderness were present in 80%. The most frequently infected sites were vertebrae (46%), cranium (23%), ribs (16%), and long bones (13%). Patients with vertebral Aspergillus osteomyelitis had more previous orthopedic surgery (19% vs 0%; P=0.02), while those with cranial osteomyelitis had more diabetes mellitus (32% vs 8%; P=0.002) and prior head/neck surgery (12% vs 0%; P=0.02). Radiologic findings included osteolysis, soft-tissue extension, and uptake on T2-weighted images. Vertebral body Aspergillus osteomyelitis was complicated by spinal-cord compression in 47% and neurological deficits in 41%. Forty-four patients (24%) received only antifungal therapy, while 121(67%) were managed with surgery and antifungal therapy. Overall mortality was 25%. Median duration of therapy was 90 days (range, 10–772 days). There were fewer relapses in patients managed with surgery plus antifungal therapy in comparison to those managed with antifungal therapy alone (8% vs 30%; P=0.006). Conclusions Aspergillus osteomyelitis is a debilitating infection affecting both immunocompromised and immunocompetent patients. The most common sites are vertebrae, ribs, and cranium. Based upon this comprehensive review, management of Aspergillus osteomyelitis optimally includes antifungal therapy and selective surgery to avoid relapse and to achieve a complete response. PMID:24378282

Gamaletsou, Maria N.; Rammaert, Blandine; Bueno, Marimelle A.; Moriyama, Brad; Sipsas, Nikolaos V.; Kontoyiannis, Dimitrios P.; Roilides, Emmanuel; Zeller, Valerie; Prinapori, Roberta; Tajaldeen, Saad Jaber; Brause, Barry; Lortholary, Olivier; Walsh, Thomas J.

2014-01-01

311

Clinical Manifestations among Children with Chronic Functional Constipation  

PubMed Central

BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group. PMID:25628851

Dehghani, Seyed Mohsen; Kulouee, Niloofar; Honar, Naser; Imanieh, Mohammad-Hadi; Haghighat, Mahmood; Javaherizadeh, Hazhir

2015-01-01

312

Olfactory dysfunction is associated with neuropsychiatric manifestations in Parkinson's disease.  

PubMed

Hyposmia, psychiatric disorders, and cognitive problems are common nonmotor manifestations in Parkinson's disease, but how they are related remains unclear. We investigated the relationship between olfactory dysfunction and neuropsychiatric manifestations and performed a cross-sectional study of 248 patients at two movement disorders clinics at academic medical centers. Psychiatric measures were the Geriatric Depression Scale-15, Inventory of Depressive Symptomatology, State Anxiety Inventory, Apathy Scale, and Parkinson's Psychosis Rating Scale. Cognitive measures were the Mini-Mental State Examination, Hopkins Verbal Learning Test-Revised, Digit Span, Tower of London-Drexel, and the Stroop Color Word Test. Olfaction was tested with the University of Pennsylvania Smell Identification Test. There was no significant association between olfaction and mood measures, but psychotic symptoms were more common in patients with olfaction scores below the median (30% vs. 12%; P < 0.001). Worse olfaction was associated with poorer memory (Hopkins Verbal Learning Test-Revised delayed recall items: mean [standard deviation], 6.2 [3.2] vs. 8.4 [2.8]; P < 0.001) and executive performance (Tower of London total moves, 52 [38] vs. 34 [21]; P < 0.001). Odor-identification score was a significant predictor of abnormal performance on these cognitive tests after adjustment for age, sex, and disease characteristics in logistic regression models. The relationship between hyposmia, psychosis, and specific cognitive impairments may reflect the anatomic distribution of Lewy pathology and suggests that olfactory dysfunction could be a biomarker of additional extranigral disease. Future prospective studies are warranted to assess whether hyposmia, a very early feature of Parkinson's disease, might be used to predict the appearance of other common nonmotor symptoms. PMID:21611985

Morley, James F; Weintraub, Daniel; Mamikonyan, Eugenia; Moberg, Paul J; Siderowf, Andrew D; Duda, John E

2011-09-01

313

Oral manifestations of Parry–Romberg syndrome: A review of literature  

PubMed Central

Parry–Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS. We searched in PubMed line using specific words such as PRS from 2008 to 2014 (August). We identify 14 papers have described oral manifestations of this syndrome. We excluded all the article papers that did not indicate to oral manifestations of PRS. PMID:25878963

Al-Aizari, Nader A.; Azzeghaiby, Saleh Nasser; Al-Shamiri, Hashem Motahir; Darwish, Shourouk; Tarakji, Bassel

2015-01-01

314

Oro-facial manifestations of 100 leprosy patients  

PubMed Central

Objectives: To verify the frequency of oral and facial involvement in diagnosed leprosy patients. Study design: This study was performed on 100 leprosy patients (62 male, 38 female, mean ages 51.86±6.1). After explaining the study design, we studied descriptive information including: patient’s sex, age, job, place of birth, familial history of leprosy, types of disease (lepromatous, borderline and tuberculoid leprosy), ocular and oral lesions, facial involvement and neuropathy. The statistical signification was measured by chi-square test. Results: A total of 46 (23 lepromatous, 15 borderline, and 8 tuberculoid leproy) out of 100 patients with leprosy had oral lesions. Statistical analysis did not show any significant difference in frequency of oral lesions between different types of disease. Facial lesions were presented in 57 (39 lepromatous, 10 borderline, and 8 tuberculoid leprosy) patients. There was a statistical significant difference in frequency of facial manifestations between different types of leprosy. It has to be mentioned that, atrophy of nasal spine, facial nerve involvement, ocular lesions and facial deformity were seen in 15%, 17%, 22% and 44% of leprosy patients, respectively. Conclusion: Examination of leprosy patients should be extended to the oral mucosa because oral mucosa may be a secondary source of M.Leprae transmission and infection. Key words:Leprosy, lepromatous, tuberculoid, oral lesions, facial lesions. PMID:22322502

Taheri, Jamileh B.; Moshfeghi, Mahkameh; Bakhshi, Mahin; Bakhtiari, Sedigheh; Azari-Marhabi, Saranaz; Alirezaei, Somayeh

2012-01-01

315

Oral Manifestations of Human Immunodeficiency Virus-Infected Patients  

PubMed Central

Introduction: Oral lesions are among the earliest clinical manifestations of human immunodeficiency (HIV) infection and are important in early diagnosis and for monitoring the progression to acquired immunodeficiency syndrome (AIDS). The purpose of this study was to determine the prevalence of oral lesions and their relationship with a number of factors in HIV/AIDS patients attending an HIV center. Materials and Methods: A total of 110 HIV-positive patients were examined to investigate the prevalence of oral lesions according to the criteria established by the European Community Clearing House on Oral Problems Related to HIV Infection. An independent T-test was used for correlation of oral lesions with CD4+ count and a ?2 test was used for analysis of the relationship of co-infection with hepatitis B virus (HBV), sexual contact, route of transmission, history of drug abuse, and history of incarceration. Results: Most of the cases were male patients (82.7%). The mean age across all participants was 36.2±8.1 years. Rampant carries, severe periodontitis and oral candidiasis were the most notable oral lesions. Oral lesions were more prevalent in patients between 26–35 years of age. There was a significant difference between patients with and without pseudomembranous candidiasis and angular cheilitis according to mean level of CD4+. Conclusion: The most common oral presentations were severe periodontitis, pseudomembranous candidiasis and xerostomia.

Pakfetrat, Atessa; Falaki, Farnaz; Delavarian, Zahra; Dalirsani, Zohreh; Sanatkhani, Majid; Zabihi Marani, Mahsa

2015-01-01

316

Adult Mitochondrial DNA Depletion Syndrome with Mild Manifestations  

PubMed Central

Mitochondrial DNA depletion syndrome (MDS) is usually a severe disorder of infancy or childhood, due to a reduced copy number of mtDNA molecules. MDS with only mild, nonspecific clinical manifestations and onset in adulthood has not been reported. A 47-year-old Caucasian female with short stature and a history of migraine, endometriosis, Crohn’s disease, C-cell carcinoma of the thyroid gland, and a family history positive for mitochondrial disorder (2 sisters, aunt, niece), developed day-time sleepiness, exercise intolerance, and myalgias in the lower-limb muscles since age 46y. She slept 9-10 hours during the night and 2 hours after lunch daily. Clinical exam revealed sore neck muscles, bilateral ptosis, and reduced Achilles tendon reflexes exclusively. Blood tests revealed hyperlipidemia exclusively. Nerve conduction studies, needle electromyography, and cerebral and spinal magnetic resonance imaging were noninformative. Muscle biopsy revealed detached lobulated fibers with subsarcolemmal accentuation of the NADH and SDH staining. Realtime polymerase chain reaction revealed depletion of the mtDNA down to 9% of normal. MDS may be associated with a mild phenotype in adults and may not significantly progress during the first year after onset. In an adult with hypersomnia, severe tiredness, exercise intolerance, and a family history positive for mitochondrial disorder, a MDS should be considered. PMID:23888212

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

2013-01-01

317

[Imaging manifestation and medicolegal significance of bipartite patella].  

PubMed

Bipartite patella is uncommon in clinical medicine. It is formed by two or more ossification centers It is repeatedly misdiagnosed as patellar fracture in clinical practice. which do not fused in adolescence. In order to elevate the recognition of imaging signs of bipartite patella and to avoid the fault in medicolegal expertise testimony, three cases of bipartite patella were analyzed in combination of literature review, and the possible causes, histological changes, imaging signs, and clinical manifestation of bipartite patella were also discussed. The three cases concerned were all adults, two male and one female. They all felt pain after knee joint injury and were diagnosed as patellar fracture in medical institutions. Two cases were appraised on the degree of injury and one was on the degree of disability. According to Saupe's grouping, two cases were group III, and one was group II and III. In the practice of medicolegal expertise testimony, recognizing imaging signs of bipartite patella could provide more rigorous and objective conclusion. PMID:20232744

Ying, Chong-liang; Wang, Ya-hui; Wan, Lei

2010-02-01

318

Severe Abdominal Pain as the First Manifestation of Rabies  

PubMed Central

Introduction: Rabies is an acute fatal viral disease that is generally transmitted from animals to humans following wild and domestic animal bites. The rabies virus enters the body from the area where the individual is bitten, and then the virus moves towards the brain and involves the nerves. Case Presentation: During the years 2001-2011, there have been 73 reported rabies cases. About 50,000 reported human deaths are annually due to rabies. The actual number of human deaths due to rabies in Asia especially India, Pakistan and Bangladesh are more than these numbers, since there is no advanced surveillance system for disease control to determine the actual number of infected and fatal human cases. According to the World Health Organization (WHO) reports, more than 10 million people who are bitten by animals are annually treated by prophylactic treatment regimens for rabies, worldwide. Conclusions: This paper reports on a case of human rabies with the first disease manifestation (severe abdominal pain). The patient reported extensive biting on his left leg by a dog. He had a slight fever of 38.1°C. It has been recommended that a careful history should be taken from patients for diagnosis of rabies disease. A complete history should be taken from patients for diagnosis of disease, because rabies could be wrong with various diseases with atypical symptoms. because various diseases with atypical symptoms or long incubation periods can visit. PMID:25485053

Ayatollahi, Jamshid; Sharifi, Mohammad Reza; Shahcheraghi, Seyed Hossein

2014-01-01

319

[Clinical manifestations and hazards of brucellosis in the Netherlands].  

PubMed

Brucellosis, a zoonotic infection characterised by undulant fever, has a low incidence in the Netherlands and is therefore rarely considered. We describe 3 patients aged 26, 47 and 56 years old; each presented with long-standing fever as predominant symptom after having travelled to an endemic area, Iraq or Turkey, 1 week to 4 months prior to manifestation of illness. They had similar symptoms: fever, weight loss, chills, night sweats and dry cough. Blood cultures of all patients became positive for gram-negative rods after 3-4 days of incubation. One patient had imported and consumed sheep-milk cheese from which a genetically indistinguishable Brucella strain was cultured. In another patient, identification of the bacterium proved difficult, resulting in delayed prescription of adequate antibiotic treatment. Serologic testing confirmed the diagnosis in this case. In the laboratory, there is a considerable risk of airborne transmission of the disease necessitating clear notification of the suspicion of brucellosis when material for culturing is submitted. PMID:22436527

Kaan, Jan A; Frakking, Florine N J; Arents, Nicolaas L A; Anten, Sander; Roest, Hendrik I J; Rothbarth, Ph H Flip

2012-01-01

320

Diffuse Lymphadenopathy as the Presenting Manifestation of SLE  

PubMed Central

We report the case of a 27 year old African-American man who presented with 6 months of generalized lymphadenopathy and nothing in his history or examination to suggest systemic lupus erythematosus. He was thought to have lymphoma, syphilis or tuberculosis and an extensive work up was done. Laboratory investigation finally revealed leukopenia (4.0), proteinuria (1.87grams), ANA (640 speckled), anti-dsDNA (640) , anticardiolipin IgG and IgM, anti-Smith, Coombs, anti-Ro, anti-La, CK (531U/L), aldolase (8.5 U/L), high erythrocyte sedimentation rate (130) and low complement (C3 15mg/dl and C4 3mg/dl). A kidney biopsy showed diffuse proliferative glomerulonephritis, ISN class IV. Generalized lymphadenopathy as the first and only manifestation for 6 months made the diagnosis of SLE challenging. Generalized diffuse lymphadenopathy has been associated with SLE, but is much less frequent now than in the past. The differential diagnosis of lymphadenopathy relevant to rheumatologists, includes Kikuchi histiocytic necrotizing lympadenitis, Castleman disease, syphilis, tuberculosis, sarcoidosis and lymphoma. PMID:24048114

Smith, Lanaya Williams; Petri, Michelle

2015-01-01

321

Superwarfarin intoxication: hematuria is a major clinical manifestation.  

PubMed

Since superwarfarin is popular and readily available in stores, it may cause intoxication or overexposure, which can result in coagulopathy or abnormal bleeding in humans and, thus, is an important public health problem. We report our clinical experience with superwarfarin intoxication. Nine patients, including eight patients who had histories of ingesting superwarfarin, were studied. Of the patients, hematuria occurred in eight. Laboratory tests among the nine patients showed extremely prolonged prothrombin times and activated partial thromboplastin times, which could be corrected to normal by mixing 1:1 with normal pooled plasma; they also had very low functional levels of factor II, VII, IX, X, and proteins C and S, but normal functional levels of factors V, VIII, fibrinogen, and anti-thrombin III. Large doses of vitamin K1 were needed for 3 months or more to treat and correct the coagulopathy among the patients. The majority of the patients presented with gross hematuria, suggesting that hematuria is probably a major clinical manifestation of superwarfarin intoxication. Prolonged use of large doses of vitamin K1 is needed for the treatment of superwarfarin intoxication. PMID:19588218

Wu, Yi-Feng; Chang, Cheng-Shyong; Chung, Chih-Yuan; Lin, Hsuan-Yu; Wang, Chuan-Cheng; Shen, Ming-Ching

2009-09-01

322

Fraser syndrome-oral manifestations and a dental care protocol.  

PubMed

Fraser syndrome is a rare genetic malformation with an autosomal recessive pattern of inheritance and an incidence of consanguinity ranging from 15% to 25%. A 5-year-old male patient who is a carrier of Fraser syndrome initiated treatment in the pediatric dentistry sector. The patient was fed parenterally since birth, experienced recurring bouts of chronic lung disease, and was referred to the pediatric dentistry sector by the medical team. Radiographic examination revealed the presence of all permanent teeth. Supragingival dental calculus, halitosis, and gingival inflammation were also observed. Dental calculus was removed by manual scraping, and chemotherapeutic agents were used, chlorhexidine 0,12%, chlorhexidine gel 2%, and PVP-I, to control the bacterial flora. The patient is still being monitored after an 8-year follow-up period, the complications associated with periodontal disease decreased, and since the initiation of treatment, the patient has not needed to be hospitalized because of chronic lung disease. This study reports the case of a patient diagnosed with Fraser syndrome and describes the clinical manifestations (general and oral). PMID:25587460

de Oliveira, Talita Lopes; de Sant'Anna, Giselle Rodrigues

2014-01-01

323

Skeletal Manifestations of Scurvy: A Case Report from Dubai  

PubMed Central

Introduction. Nutritional deficiencies are rarely reported in developed countries. We report a child of Pakistani origin brought up in Dubai who developed skeletal manifestations of scurvy due to peculiar dietary habits. Case Presentation. A 4.5 year old boy presented with pain and swelling of multiple joints for three months and inability to walk for two months. Dietary history was significant for exclusive meat intake for the preceding two years. On examination the child's height and weight were below the 5th percentile for his age. He was pale and tachycardic. There was significant swelling and tenderness over the wrist, knee and ankle joints, along with painful restriction of motion. Basic blood workup was unremarkable except for anemia. However, X-rays showed delayed bone age, severe osteopenia of the long bones, epiphyseal separation, cortical thinning and dense zone of provisional calcification, suggesting a radiological diagnosis of scurvy. The child was started on vitamin C replacement therapy. Over the following two months, the pain and swelling substantially reduced and the child became able to walk. Repeat X-rays showed improvement in the bony abnormalities. Conclusion. Although scurvy is not a very commonly encountered entity in the modern era, inappropriate dietary intake can lead to skeletal abnormalities which may be confused with rickets. A high index of suspicion is thus required for prompt diagnosis of scurvy in patients with bone and joint symptoms. PMID:23259126

Noordin, Shahryar; Baloch, Naveed; Salat, Muhammad Sohail; Rashid Memon, Abdul; Ahmad, Tashfeen

2012-01-01

324

Neurologic and Psychiatric Manifestations of Celiac Disease and Gluten Sensitivity  

PubMed Central

Celiac Disease (CD) is an immune-mediated disease dependent on gluten (a protein present in wheat, rye or barley) that occurs in about 1% of the population and is generally characterized by gastrointestinal complaints. More recently the understanding and knowledge of gluten sensitivity (GS), has emerged as an illness distinct from celiac disease with an estimated prevalence 6 times that of CD. Gluten sensitive people do not have villous atrophy or antibodies that are present in celiac disease, but rather they can test positive for antibodies to gliadin. Both CD and GS may present with a variety of neurologic and psychiatric co-morbidities, however, extraintestinal symptoms may be the prime presentation in those with GS. However, gluten sensitivity remains undertreated and underrecognized as a contributing factor to psychiatric and neurologic manifestiations. This review focuses on neurologic and psychiatric manifestations implicated with gluten sensitivity, reviews the emergence of gluten sensitivity distinct from celiac disease, and summarizes the potential mechanisms related to this immune reaction. PMID:21877216

Jackson, Jessica R.; Eaton, William W.; Cascella, Nicola G.; Fasano, Alessio

2013-01-01

325

Manifestations of pulmonary cryptococcosis in patients with acquired immunodeficiency syndrome.  

PubMed

Cryptococcosis is a common opportunistic infection in patients with AIDS. Meningitis is the most frequent manifestation of infection with Cryptococcus neoformans; pneumonia due to this organism, though less frequently recognized, is also a significant entity. A retrospective review was performed of all patients seen at Duke University Medical Center between January 1981 and July 1989 who were infected with both human immunodeficiency virus type 1 and C. neoformans. Of 31 patients with these concomitant infections, 12 had cryptococcal pneumonia (10 definite and two presumptive cases). Eleven of these 12 patients had evidence of extrapulmonary cryptococcal disease as well. Chest radiography showed interstitial infiltrates in 11 instances. For ten of the 12 patients, pulmonary cultures were positive for C. neoformans. Bronchoalveolar lavage fluid from all five patients who underwent bronchoscopy yielded the organism. Acute-phase mortality from cryptococcosis was 42% among patients with pneumonia. Cryptococcal pneumonia in patients with AIDS is probably more common than has previously been recognized and typically presents as interstitial disease that may mimic other opportunistic infections. PMID:2017634

Cameron, M L; Bartlett, J A; Gallis, H A; Waskin, H A

1991-01-01

326

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

327

Hematologic manifestations in a child with HIV; a Case Report  

PubMed Central

Background Immune deficiency in human might be primary or secondary and could be seen with a wide variety of manifestations. In the following, we presented a Child with various complains that diagnosed to have HIV infection. Case Report A 2/5 y/o child was admitted to the hospital for FUO with prolonged cough, FTT, cervical lymphadenopathy, hepatosplenomegaly and bilateral optic neuritis. . He was hospitalized for fever, cytopenia and hepatosplenomegaly one year ago, and three months later in an outpatient visit, these signs improved, except thrombocytopenia. In evaluation, bicytopenia, elevated ESR, hyperlipidemia, hyperproteinemia, thrombosis of the transverse sinus of brain, antiphospholipid antibodies , decreased levels of protein S and factor V Leiden and increased level of anti thrombin III were detected. Consequently, the result of HIV antibody showed positive. In addition to warfarin and cotrimoxazole therapy, he was referred to special center for possible HARRT therapy. Conclusion In approach to patients with various clinical presentations such as cytopenia, recurrent or persistent lymphadenopathy, unexplained hyperproteinemia or hyperlipidemia, evaluation of HIV infection is highly recommended for consideration and further therapy. PMID:24575260

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

2012-01-01

328

Endocrine manifestations related to inherited metabolic diseases in adults  

PubMed Central

Most inborn errors of metabolism (IEM) are recessive, genetically transmitted diseases and are classified into 3 main groups according to their mechanisms: cellular intoxication, energy deficiency, and defects of complex molecules. They can be associated with endocrine manifestations, which may be complications from a previously diagnosed IEM of childhood onset. More rarely, endocrinopathies can signal an IEM in adulthood, which should be suspected when an endocrine disorder is associated with multisystemic involvement (neurological, muscular, hepatic features, etc.). IEM can affect all glands, but diabetes mellitus, thyroid dysfunction and hypogonadism are the most frequent disorders. A single IEM can present with multiple endocrine dysfunctions, especially those involving energy deficiency (respiratory chain defects), and metal (hemochromatosis) and storage disorders (cystinosis). Non-autoimmune diabetes mellitus, thyroid dysfunction and/or goiter and sometimes hypoparathyroidism should steer the diagnosis towards a respiratory chain defect. Hypogonadotropic hypogonadism is frequent in haemochromatosis (often associated with diabetes), whereas primary hypogonadism is reported in Alström disease and cystinosis (both associated with diabetes, the latter also with thyroid dysfunction) and galactosemia. Hypogonadism is also frequent in X-linked adrenoleukodystrophy (with adrenal failure), congenital disorders of glycosylation, and Fabry and glycogen storage diseases (along with thyroid dysfunction in the first 3 and diabetes in the last). This is a new and growing field and is not yet very well recognized in adulthood despite its consequences on growth, bone metabolism and fertility. For this reason, physicians managing adult patients should be aware of these diagnoses. PMID:22284844

2012-01-01

329

Morphologic Features of Extrahepatic Manifestations of Hepatitis C Virus Infection  

PubMed Central

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

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

2012-01-01

330

Astrophysical manifestations of clumps of cold dark matter  

SciTech Connect

Small-scale structures (clumps) of dark matter may manifest themselves owing to the annihilation of dark-matter particles in them as pointlike gamma-ray sources. In view of this, investigation into respective effects on the basis of data on unidentified pointlike gamma-ray sources is of importance. It is shown that the existing uncertainties in the description of physical properties of dark-matter particles (their annihilation cross section) and in the distribution of their density in the clumps are of crucial importance; therefore, an analysis of data from the observation of pointlike gamma-ray sources makes it possible to impose constraints on the values of respective uncertain parameters (that is, to single out preferable ones). It is considered that the rate of annihilation of dark-matter particles in the clumps may be enhanced both owing to a higher density and owing to the growth of the cross section at low relative velocities of dark-matter particles in the clumps. In particular, dark-matter particles may have self-interaction of the Coulomb type, and this leads to the enhancement of the annihilation rate because of the Sommerfeld-Sakharov effect. It is shown that the heavy-neutrino model featuring an extra interaction can explain partly Fermi and EGRET data on unidentified pointlike gamma-ray sources. It is indicated that the motion of gamma-ray sources over the celestial sphere can be noticed for several clumps within several years of observations.

Belotsky, K. M., E-mail: k-belotsky@yandex.ru; Kirillov, A. A., E-mail: kirillov-aa@yandex.ru; Khlopov, M. Yu., E-mail: khlopov@apc.univ-paris7.fr [National Research Nuclear University MEPhI (Russian Federation)

2013-04-15

331

Para-dichlorobenzene toxicity – a review of potential neurotoxic manifestations  

PubMed Central

Background: Para-dichlorobenzene (PDCB) is an active ingredient of mothballs, deodorizers and fumigants. Due to the easy availability of this chemical, there is a considerable risk for accidental or intentional toxic exposure. Recently, multiple cases of PDCB toxicity due to mothball ingestion were reported. PDCB toxicity can affect multiple organ systems including liver, kidneys, skin, lung and the central nervous system (CNS). CNS toxicity often results in leukoencephalopathy and heterogeneous neurological manifestations. Objectives: The objective of this study was to illustrate the clinical presentation, imaging findings, diagnosis and management of PDCB toxicity. Methods: We carried out a literature review of the pharmacological and toxicological properties of PDCB. Conclusions: PDCB and other aromatic hydrocarbons are capable of CNS tissue damage and in promoting functional neurological decline. While very little is currently known about prevalence of PDCB addiction, it cannot be ruled out that its illicit use among young people is under-recognized. The number of cases of PDCB toxicity might also rise due to the increasing industrial and domestic use of this chemical. PMID:24790648

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

2014-01-01

332

MHC associations with clinical and autoantibody manifestations in European SLE.  

PubMed

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

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

2014-04-01

333

MHC associations with clinical and autoantibody manifestations in European SLE  

PubMed Central

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

Morris, DL; Fernando, MMA; Taylor, KE; Chung, SA; Nititham, J; Alarcón-Riquelme, ME; Barcellos, LF; Behrens, TW; Cotsapas, C; Gaffney, PM; Graham, RR; Pons-Estel, BA; Gregersen, PK; Harley, JB; Hauser, SL; Hom, G; Langefeld, CD; Noble, JA; Rioux, JD; Seldin, MF; Vyse, TJ; Criswell, LA

2014-01-01

334

Ball Lightning: Manifestation of Cosmic Little Black Holes  

E-print Network

A case is made that in encounters with the earth's atmosphere, astrophysical little black holes (LBH) can manifest themselves as the core energy source of balllightning (BL). Relating the LBH incidence rate on earth to BL occurrence has the potential of shedding light on the distribution of LBH in the universe, and their velocities relative to the earth. Most BL features can be explained by a testable LBH model. Analyses are presented to support this model. LBH produce complex and many-faceted interactions in air directly and via their exhaust, resulting in excitation, ionization, and radiation due to processes such as gravitational and electrostatic tidal force, bremsstrahlung, pair production and annihilation, orbital electron near-capture by interaction with a charged LBH. Gravitational interaction of atmospheric atoms with LBH can result in an enhanced cross-section for polarization and ionization. An estimate for the power radiated by BL ~ Watts is in agreement with observation. An upper limit is found for the largest masses that can produce ionization and polarization excitation. It is shown that the LBH high power exhaust radiation is not prominent and its effects are consistent with observations.

Mario Rabinowitz

2001-04-03

335

Enzyme replacement therapy in Fabry disease: influence on cardiac manifestations.  

PubMed

Fabry disease (FD) is an X-linked glycosphingolipid storage disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. This leads to a progressive accumulation of globotriaosylceramide (Gb3) in the lysosomes of different cells and tissues, causing principally ventricular hypertrophy, renal failure and cerebrovascular accidents, reducing lifespan both in hemizygous males and heterozygous females. Residual enzyme activity might lead to slow progression of the disease and result in the so-called cardiac or renal variants with delayed presentation. Two different forms of alpha-galactosidase A enzyme replacement therapies (ERT) are available for the treatment of FD, one genetically engineered in human cell line (agalsidase alfa, Replagal, Shire) and the other produced in a Chinese hamster ovary cell line (agalsidase beta, Fabrazyme, Genzyme). Although both proteins are structurally and functionally very similar, with the same amino acid sequence as the native human enzyme, they differ in the pattern of glycosilation of the protein depending on the originating cell line. Studies with both preparations have described a reduction in plasma, urinary sediment and tissue levels of Gb3, a decrease in the frequency of pain crisis and a reduction in left ventricular mass and improvement or stabilization of renal function. Studies have generally shown the greatest benefit when treatment is started at an early stage of the disease before extensive fibrosis or other irreversible tissue damage takes place. However, more data are needed to document long-term treatment outcomes. The aim of the present review is to provide an update overview of the two different forms of ERT for FD, their clinical effects in cardiac manifestations and their possible differences in terms of efficacy, side effects and safety profiles. PMID:20345350

Caballero, L; Climent, V; Hernández-Romero, D; Quintanilla, M A; de la Morena, G; Marín, F

2010-01-01

336

Fibrotic Myofibroblasts Manifest Genome-Wide Derangements of Translational Control  

PubMed Central

Background As a group, fibroproliferative disorders of the lung, liver, kidney, heart, vasculature and integument are common, progressive and refractory to therapy. They can emerge following toxic insults, but are frequently idiopathic. Their enigmatic propensity to resist therapy and progress to organ failure has focused attention on the myofibroblast–the primary effector of the fibroproliferative response. We have recently shown that aberrant beta 1 integrin signaling in fibrotic fibroblasts results in defective PTEN function, unrestrained Akt signaling and subsequent activation of the translation initiation machinery. How this pathological integrin signaling alters the gene expression pathway has not been elucidated. Results Using a systems approach to study this question in a prototype fibrotic disease, Idiopathic Pulmonary Fibrosis (IPF); here we show organized changes in the gene expression pathway of primary lung myofibroblasts that persist for up to 9 sub-cultivations in vitro. When comparing IPF and control myofibroblasts in a 3-dimensional type I collagen matrix, more genes differed at the level of ribosome recruitment than at the level of transcript abundance, indicating pathological translational control as a major characteristic of IPF myofibroblasts. To determine the effect of matrix state on translational control, myofibroblasts were permitted to contract the matrix. Ribosome recruitment in control myofibroblasts was relatively stable. In contrast, IPF cells manifested large alterations in the ribosome recruitment pattern. Pathological studies suggest an epithelial origin for IPF myofibroblasts through the epithelial to mesenchymal transition (EMT). In accord with this, we found systems-level indications for TGF-? -driven EMT as one source of IPF myofibroblasts. Conclusions These findings establish the power of systems level genome-wide analysis to provide mechanistic insights into fibrotic disorders such as IPF. Our data point to derangements of translational control downstream of aberrant beta 1 integrin signaling as a fundamental component of IPF pathobiology and indicates that TGF-? -driven EMT is one source for IPF myofibroblasts. PMID:18795102

Fan, Danhua; Peterson, Mark; Nho, Richard Seonghun; Bitterman, Peter B.; Henke, Craig A.

2008-01-01

337

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

338

Extra-articular manifestations in psoriatic arthritis patients.  

PubMed

Psoriatic arthritis (PsA) is an inflammatory rheumatic disorder, associated with skin and/or nail psoriasis. It has been included in the spondyloarthropathies (SpA) group, with which it shares clinical, radiologic, and serologic features and familial and genetic relationship. Inclusion of disease among SpA is also based on their striking points of similarity for extra-articular manifestations (EAMs). The aim of study was to describe the EAMs in patients with PsA, evaluating the prevalence and clinical features associated with established and early PsA. The study was a retrospective analysis of case records of 387 PsA patients. Data recorded were demographic data, disease properties, laboratory tests, drug use, and presence of EAMs. Of 387 PsA patients, 190 have shown EAMs: 33.16 % had bowel involvement, 32.63 % ocular, 28.42 % cardiovascular, 25.79 % urogenital, 8.42 % skin (excluding psoriasis), 1.05 % pulmonary, and 0.53 % renal. A higher prevalence of EAMs was found in axial subset (p?

Peluso, Rosario; Iervolino, Salvatore; Vitiello, Maria; Bruner, Vincenzo; Lupoli, Gelsy; Di Minno, Matteo Nicola Dario

2015-04-01

339

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

PubMed Central

Objective Nowadays, many infertile couples can have child by assistant reproductive technology (ART). Always the undesirable effects of these methods on newborn are considered and are evaluated. The aim of this study is to describe the impact of ART on ocular and visual performances of infants born by these methods. Materials and methods In a cross-sectional descriptive study, 479 infants aged three-nine months presented to an optometry clinic of Child Health and Development Research Department (CHDRD), Tehran, Iran. Static retinoscopy, qualitative fixation evaluation, Hirschberg test, red reflex assessment and external eye examination were carried out. Other information such as birth weight and maturity of the infants was recorded. Results It was possible to assess only 320 out of 479 infants due to general condition of some participants. Comparison of mean refractive error in infants’ right and left eyes did not show any significant difference. Our findings confirmed that 20.3% had poor fixation, while 2.9% revealed manifest strabismus. The results also revealed the prevalences of myopia, hyperopia and emmetropia are 2.9%, 87%, and 10.1%, respectively. Red reflex abnormalities were significantly found in boys and in preterm infants (p < 0.05). Failure of fixation control was seen more frequently with increasing refractive error, which significantly developed in preterm infants (p < 0.001). Conclusion These results reflect the necessity of more comprehensive assessments and further follow-up of infants born by ART, especially for premature male ART infants. These results also suggest the probability of fixation condition and visual deficiencies in these infants. It is recommended to pay close attention to this preliminary report about the refractive and fixation condition of the infants born after ART. PMID:24971123

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

2013-01-01

340

Intraocular inflammation as the main manifestation of Rickettsia conorii infection  

PubMed Central

Objective To report the clinical features and management of seven cases of intraocular inflammation caused by Rickettsia infection and review published literature. Methods Rickettsia conorii or Rickettsia spp. infection was diagnosed based on the following criteria: (1) positive serology according to the European Guidelines, (2) titer normalization after specific treatment, and (3) complete resolution of ophthalmic disease and accompanying symptoms after antibiotic therapy. Results Seven patients were referred for uveitis of unknown etiology. All came from regions where Mediterranean spotted fever is prevalent. One patient met the European guidelines criteria for Rickettsia spp. infection, while the other six cases met the criteria for R. conorii infection. The main symptoms were visual loss, floaters, eye redness, photophobia, and ocular pain. Predominant ophthalmic signs included vasculitis, choroiditis, vitritis, and macular edema. All patients required antibiotic treatment that resulted in the remission of the infection. Doxycycline was the first choice and the only antibiotic used to treat four patients. One patient needed ciprofloxacin as a second antibiotic after not responding to doxycycline. Two patients had doxycycline as a second antibiotic after not responding primarily to sulfonamides (which had been given after 2–3 days of doxycycline gastric intolerance); one of these patients needed ciprofloxacin as a third antibiotic. Conclusion Intraocular inflammation can occur as the main manifestation of Rickettsia conorii or Rickettsia spp. infection. It should be considered as a differential diagnosis for uveitis especially for patients living in countries where this infection is endemic in the world. Antibiotic treatment remains effective in the management of Rickettsia infection. PMID:22034560

Agahan, Archimedes LD; Torres, Jenice; Fuentes-Páez, Graciana; Martínez-Osorio, Hernan; Orduña, Antonio; Calonge, Margarita

2011-01-01

341

Oral Manifestations in HIV/AIDS-Infected Children  

PubMed Central

Objectives: To assess factors influencing the distribution of oral manifestations in HIV/AIDS-infected children attending the Paediatric Infectious Disease Clinic in Mulago Hospital, Kampala. Methods: This was a cross-sectional study comprising 237 children (males/females: 113/124) aged 1 to 12 years. The parents/guardians were interviewed to obtain demographic information, oral hygiene practices, dietary habits and health seeking behaviours as well as any medications taken. The children were clinically examined for oral lesions based on World Health Organization criteria with modifications. Results: About 71.7% of the children cleaned their teeth. About 16.9% of the children had visited a dentist since birth, mainly for emergency care. One or more oral lesions were recorded in 73% of the children of whom 19.0% experienced discomfort during oral functions. Cervical lymphadenopathy, oral candidiasis and gingivitis were the most common soft tissue oral lesions: 60.8%, 28.3% and 19.0%, respectively. Except for dental caries, the overall frequency distribution of soft tissue oral lesions was significantly lower in children on highly active antiretroviral therapy (HAART) as compared to their counterparts not on HAART. The prevalence of dental caries in deciduous and permanent dentitions was 42.2% and 11.0%, respectively. Tooth brushing and previous visits to the dentist were indirectly and significantly associated with dental caries. About 5.9% (n=14) of the children had <200 CD3 + CD4 T-lymphocyte cells per ?l of blood. Conclusions: The majority of the children had one or more oral lesions, particularly in the group not on HAART. Some of the lesions were associated with discomfort during oral functions. PMID:21769270

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

2011-01-01

342

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

PubMed Central

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

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

2014-01-01

343

Restoring the equivalence between the light-front and manifestly covariant formalisms  

SciTech Connect

We discuss a treacherous point in light-front dynamics (LFD) which should be taken into account to restore complete equivalence with the manifestly covariant formalism. We present examples that require an inclusion of the arc contribution in the light-front energy contour integration in order to achieve the equivalence between the LFD result and the manifestly covariant result.

Bakker, Bernard L.G. [Department of Physics and Astronomy, Vrije Universiteit, Amsterdam (Netherlands); DeWitt, Martin A.; Ji, C.-R. [Department of Physics, Box 8202, North Carolina State University, Raleigh, North Carolina 27695-8202 (United States); Mishchenko, Yuriy [Cold Spring Harbor Laboratory, 1 Bungtown Road, Cold Spring Harbor, New York 11724 (United States)

2005-10-01

344

Longitudinal Change in Gait and Motor Function in Pre-manifest Huntington's Disease  

E-print Network

Longitudinal Change in Gait and Motor Function in Pre-manifest Huntington's Disease The purpose of this study was to examine longitudinal change in gait and motor function in pre-manifest Huntington's disease with the motor component of the Unified Huntington's Disease Rating Scale. Gait velocity decreased (p=0

345

Longitudinal Change in Gait and Motor Function in Pre-manifest Huntington's Disease  

E-print Network

Longitudinal Change in Gait and Motor Function in Pre-manifest Huntington's Disease October 11 of this study was to examine longitudinal change in gait and motor function in pre-manifest Huntington's disease with the motor component of the Unified Huntington's Disease Rating Scale. Gait velocity decreased (p=0

346

Manifestations of Heterosexism in Icelandic Upper Secondary Schools and the Responses of LGBT Students  

ERIC Educational Resources Information Center

How does institutionalized heterosexism manifest itself in Icelandic upper secondary schools and how do lesbian, gay, bisexual, and transgender (LGBT) students respond to these manifestations? In addressing these questions, interviews were conducted with six current and former LGBT upper secondary school students, using queer theory and thematic…

Kjaran, Jón Ingvar; Jóhannesson, Ingólfur Ásgeir

2013-01-01

347

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

ERIC Educational Resources Information Center

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

Meredith, William; Millsap, Roger E.

1992-01-01

348

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. PMID:18458712

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

2004-01-01

349

[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

350

[Ocular manifestations of Churg-Strauss syndrome: review article and case report].  

PubMed

This paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression. PMID:23765370

Atili, A; Richter, C; Bahn, E; Rustenbeck, H H; Schittkowski, M

2013-11-01

351

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

Code of Federal Regulations, 2011 CFR

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

2011-04-01

352

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

Code of Federal Regulations, 2014 CFR

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

2014-04-01

353

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

Code of Federal Regulations, 2012 CFR

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

2012-04-01

354

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

Code of Federal Regulations, 2010 CFR

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

2010-04-01

355

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

Code of Federal Regulations, 2010 CFR

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

2010-04-01

356

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

Code of Federal Regulations, 2011 CFR

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

2011-04-01

357

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

Code of Federal Regulations, 2014 CFR

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

2014-04-01

358

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

Code of Federal Regulations, 2013 CFR

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

2013-04-01

359

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

Code of Federal Regulations, 2013 CFR

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

2013-04-01

360

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

Code of Federal Regulations, 2012 CFR

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

2012-04-01

361

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

PubMed

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

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

2013-03-01

362

Hand Schuller Christian Disease: A Rare Case Report with Oral Manifestation  

PubMed Central

Langerhan’s Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan’s cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-Letterer- Siwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the result of the accumulation and infilteration of the langerhan cells in organs and tissues. Here is a rare case report of 6-year-old boy with extraoral manifestation of exopthalmic right eye and oral manifestation of mobility of teeth and with typical radiological findings. Basing on the clinical, radiological and histopathological examination the diagnosis of Hand Schuller Christian Disease was given. PMID:25738095

Manjula, M.; Srikant, K.; Goyal, Stuti; Tanveer, Shahela

2015-01-01

363

19 CFR 122.49 - Correction of air cargo manifest or air waybill.  

Code of Federal Regulations, 2010 CFR

...AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew Members...

2010-04-01

364

19 CFR 122.73 - General declaration and air cargo manifest.  

Code of Federal Regulations, 2010 CFR

...PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew...

2010-04-01

365

Hand schuller christian disease: a rare case report with oral manifestation.  

PubMed

Langerhan's Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan's cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-Letterer- Siwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the result of the accumulation and infilteration of the langerhan cells in organs and tissues. Here is a rare case report of 6-year-old boy with extraoral manifestation of exopthalmic right eye and oral manifestation of mobility of teeth and with typical radiological findings. Basing on the clinical, radiological and histopathological examination the diagnosis of Hand Schuller Christian Disease was given. PMID:25738095

Lalitha, Ch; Manjula, M; Srikant, K; Goyal, Stuti; Tanveer, Shahela

2015-01-01

366

14 CFR 125.405 - Disposition of load manifest, flight release, and flight plans.  

Code of Federal Regulations, 2010 CFR

...Disposition of load manifest, flight release, and flight plans. (a) The pilot in...carry in the airplane to its destination the original or a signed copy of...required by § 125.383; (2) Flight release; (3)...

2010-01-01

367

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

Federal Register 2010, 2011, 2012, 2013, 2014

...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection, Department of...

2013-12-16

368

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

Federal Register 2010, 2011, 2012, 2013, 2014

...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection, Department of...

2011-12-09

369

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

Federal Register 2010, 2011, 2012, 2013, 2014

...DEPARTMENT OF HOMELAND SECURITY U.S. Customs and Border Protection Agency Information...Manifest of Merchandise Free of Duty, Carrier's Certificate and Release AGENCY: U.S. Customs and Border Protection (CBP),...

2011-10-06

370

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. PMID:24019738

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

2013-01-01

371

A case of a child with sle presenting with hps as a primary manifestation.  

PubMed

The primary manifestations of systemic lupus erythematosus (SLE) are various. One such manifestation is hemophagocytic syndrome (HPS). We here report a child with SLE presenting with HPS as a primary manifestation. In October 2010, an 11-year-old Japanese boy presented with pancytopenia, elevated liver enzymes, hyperferritinemia and hemophagocytosis due to macrophages in the bone marrow, and was diagnosed with HPS. A year later, he was found to have proteinuria and hematuria. Oral aphtha and Raynaud's phenomenon were observed, and the patient showed low serum complement levels and was positive for anti-nuclear antibodies (ANAs). He was subsequently diagnosed with SLE. Moreover, low serum complement levels and ANA positivity were detected in a serum sample preserved at the onset of HPS. The HPS was considered to be a primary manifestation of SLE on the basis of these findings. Based on this case, the presence of an underlying disease, such as SLE, should be investigated in cases of HPS. PMID:25747608

Ono, Atsushi; Kawasaki, Yukihiko; Kanno, Syuto; Ohara, Shinichiro; Sakai, Nobuko; Suyama, Kazuhide; Hosoya, Mitsuaki

2015-02-26

372

SLEB3 in systemic lupus erythematosus (SLE) is strongly related to SLE families ascertained through neuropsychiatric manifestations  

Microsoft Academic Search

Seizures and psychosis are neuropsychiatric (NP) manifestations of a large number of systemic lupus erythematosus (SLE) patients. Since NP manifestations were part of the SLE phenotype for some, but not all SLE affecteds, we hypothesized that those SLE patient families with NP manifestations might be more genetically homogeneous at loci important to NP-related SLE, and hence have increased power to

Swapan K. Nath; Jennifer A. Kelly; Jeff Reid; Tom Lam; Courtney Gray-McGuire; Bahram Namjou; Christopher E. Aston; John B. Harley

2002-01-01

373

Oral manifestations of Schimmelpenning syndrome: case report and review of literature.  

PubMed

Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities. Here, the SS is briefly reviewed, and the oral and dental manifestations are described in a patient whose medical findings were previously published and included severe hypophosphatemic rickets. Significant oral and dental features included papillomatous lesions of the gingiva, hemihyperplasia (hemihypertrophy) of the tongue, bone cysts, aplasia of teeth, enlarged pulp chambers, hypoplastic or absent enamel, and an odontodysplasia-like permanent tooth. PMID:10597363

Murakami, A; Skovby, F; Andreasen, J O; Cohen, M M; Jensen, B L; Kreiborg, S

1999-09-01

374

[A single metastasis in the carpal bones as the first clinical manifestation of a hepatocellular carcinoma].  

PubMed

Hepatocellular carcinoma is the most common primary tumor of the liver. Spreading outside the liver usually takes place in advanced stages of the disease, and bone is the third most common site of metastases. We present a case of hepatocellular carcinoma in which the first clinical manifestation was a single metastasis to the carpal bones. The interest of this case lies in the way this hepatocellular carcinoma manifested as well as in the unusual site of the metastasis. PMID:23092693

Corrales Pinzón, R; Alonso Sánchez, J M; de la Mano González, S; El Karzazi Tarazona, K

2014-01-01

375

Amyotrophic Lateral Sclerosis Presenting Respiratory Failure as the Sole Initial Manifestation  

PubMed Central

It is rare that amyotrophic lateral sclerosis (ALS) presents with respiratory failure as the sole initial manifestation. A 72-year-old man with mild chronic obstructive pulmonary disease developed exertional dyspnea for 13 months. He then progressed to limb weakness that led to the diagnosis of ALS. Although rare, ALS can present with respiratory failure as the sole initial manifestation more than 1 year prior to limb weakness. PMID:25232334

Tateno, Fuyuki; Sakakibara, Ryuji; Kawashima, Kengo; Kishi, Masahiko; Tsuyusaki, Yohei; Aiba, Yosuke; Ogata, Tsuyoshi

2014-01-01

376

An Unusual Case of Multiple Intraoral Manifestations of Neurofibromatosis Type 1: Case Report with Literature Review  

PubMed Central

The various oral manifestations of neurofibromatosis in dentulous patients have been documented in literature. Although most of the previous documents have discussed on common findings like a prominent lingual papillae, or solitary overgrowth of gingival soft tissue, this article focuses on a relatively rare occurrence of multiple nodular manifestations of Neurofibromatosis–1 on an edentulous alveolar ridge, tongue, palate and lips of an elderly female patient. PMID:25654048

M, Sheejith; Joseph, Benny; Sheejith, Bhavya

2014-01-01

377

Do evolutionary constraints on thermal performance manifest at different organizational scales?  

PubMed

The two foremost hypotheses on the evolutionary constraints on an organism's thermal sensitivity—the hotter-is-better expectation, and the specialist-generalist trade-off—have received mixed support from empirical studies testing for their existence. Could these conflicting results reflect confusion regarding the organizational level (i.e. species > population > individual) at which these constraints should manifest? We propose that these evolutionary constraints should manifest at different organizational levels because of differences in their underlying causes and requirements. The hotter-is-better expectation should only manifest across separate evolutionary units (e.g. species, populations), and not within populations. The specialist-generalist trade-off, by contrast, should manifest within as well as between separate evolutionary units. We measured the thermal sensitivity of sprint performance for 440 rainforest sun skinks (Lampropholis coggeri) representing 10 populations, and used the resulting performance curves to test for evidence for the hypothesized constraints at two organizational levels: (i) across populations and (ii) within populations. As predicted, the hotter-is-better expectation was evident only at the across-population level, whereas the specialist-generalist trade-off was evident within, as well as across, populations. Our results suggest that, depending on the processes that drive them, evolutionary constraints can manifest at different organizational levels. Consideration of these underlying processes, and the organizational level at which a constraint should manifest, may help resolve conflicting empirical results. PMID:25403471

Phillips, B L; Llewelyn, J; Hatcher, A; Macdonald, S; Moritz, C

2014-12-01

378

Paucity of Skeletal Manifestations in Hispanic Families with FBN1 mutations  

PubMed Central

Marfan syndrome (MFS) is an autosomal dominant condition with pleiotropic manifestations involving the skeletal, ocular, and cardiovascular systems. The diagnosis is based primarily on clinical involvement of these and other systems, referred to as the Ghent criteria. We have identified three Hispanic families from Mexico with cardiovascular and ocular manifestations due to novel FBN1 mutations but with paucity of skeletal features. The largest family, hMFS001, had a frameshift mutation in exon 24 (3075delC) identified as the cause of aortic disease in the family. Assessment of eight affected adults revealed no major skeletal manifestation of MFS. Family hMFS002 had a missense mutation (R1530C) in exon 37. Four members fulfilled the criteria for ocular and cardiovascular phenotype but lacked skeletal manifestations. Family hMFS003 had two consecutive missense FBN1 mutations (C515W and R516G) in exon 12. Eight members fulfilled the ocular criteria for MFS and two members had major cardiovascular manifestations, however none of them met criteria for skeletal system. These data suggest that individuals of Hispanic descent with FBN1 mutations may not manifest skeletal features of the MFS to the same extent as Caucasians. We recommend that echocardiogram, ocular examination and FBN1 molecular testing be considered for any patients with possible MFS even in the absence of skeletal features, including Hispanic patients. PMID:19941982

Villamizar, Carlos; Regalado, Ellen S.; Fadulu, Van Tran; Hasham, Sumera N.; Gupta, Prateek; Willing, Marcia C.; Kuang, Shao-Qing; Guo, Dongchuan; Muilenburg, Ann; Yee, Richard W.; Fan, Yuxin; Towbin, Jeffrey; Coselli, Joseph S.; LeMaire, Scott A.; Milewicz, Dianna M.

2015-01-01

379

Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease.  

PubMed

Huntington's disease is an inherited neurodegenerative disease that causes motor, cognitive and psychiatric impairment, including an early decline in ability to recognize emotional states in others. The pathophysiology underlying the earliest manifestations of the disease is not fully understood; the objective of our study was to clarify this. We used functional magnetic resonance imaging to investigate changes in brain mechanisms of emotion recognition in pre-manifest carriers of the abnormal Huntington's disease gene (subjects with pre-manifest Huntington's disease): 16 subjects with pre-manifest Huntington's disease and 14 control subjects underwent 1.5 tesla magnetic resonance scanning while viewing pictures of facial expressions from the Ekman and Friesen series. Disgust, anger and happiness were chosen as emotions of interest. Disgust is the emotion in which recognition deficits have most commonly been detected in Huntington's disease; anger is the emotion in which impaired recognition was detected in the largest behavioural study of emotion recognition in pre-manifest Huntington's disease to date; and happiness is a positive emotion to contrast with disgust and anger. Ekman facial expressions were also used to quantify emotion recognition accuracy outside the scanner and structural magnetic resonance imaging with voxel-based morphometry was used to assess the relationship between emotion recognition accuracy and regional grey matter volume. Emotion processing in pre-manifest Huntington's disease was associated with reduced neural activity for all three emotions in partially separable functional networks. Furthermore, the Huntington's disease-associated modulation of disgust and happiness processing was negatively correlated with genetic markers of pre-manifest disease progression in distributed, largely extrastriatal networks. The modulated disgust network included insulae, cingulate cortices, pre- and postcentral gyri, precunei, cunei, bilateral putamena, right pallidum, right thalamus, cerebellum, middle frontal, middle occipital, right superior and left inferior temporal gyri, and left superior parietal lobule. The modulated happiness network included postcentral gyri, left caudate, right cingulate cortex, right superior and inferior parietal lobules, and right superior frontal, middle temporal, middle occipital and precentral gyri. These effects were not driven merely by striatal dysfunction. We did not find equivalent associations between brain structure and emotion recognition, and the pre-manifest Huntington's disease cohort did not have a behavioural deficit in out-of-scanner emotion recognition relative to controls. In addition, we found increased neural activity in the pre-manifest subjects in response to all three emotions in frontal regions, predominantly in the middle frontal gyri. Overall, these findings suggest that pathophysiological effects of Huntington's disease may precede the development of overt clinical symptoms and detectable cerebral atrophy. PMID:22505631

Novak, Marianne J U; Warren, Jason D; Henley, Susie M D; Draganski, Bogdan; Frackowiak, Richard S; Tabrizi, Sarah J

2012-04-01

380

Revisiting the Molecular Mechanism of Neurological Manifestations in Antiphospholipid Syndrome: Beyond Vascular Damage  

PubMed Central

Antiphospholipid syndrome (APS) is a multiorgan disease often affecting the central nervous system (CNS). Typically, neurological manifestations of APS include thrombosis of cerebral vessels leading to stroke and requiring prompt initiation of treatment with antiplatelet drugs or anticoagulant therapy. In these cases, alterations of the coagulation system at various levels caused by multiple effects of antiphospholipid antibodies (aPL) have been postulated to explain the vascular damage to the CNS in APS. However, several nonvascular neurological manifestations of APS have progressively emerged over the past years. Nonthrombotic, immune-mediated mechanisms altering physiological basal ganglia function have been recently suggested to play a central role in the pathogenesis of these manifestations that include, among others, movement disorders such as chorea and behavioral and cognitive alterations. Similar clinical manifestations have been described in other autoimmune CNS diseases such as anti-NMDAR and anti-VGCK encephalitis, suggesting that the spectrum of immune-mediated basal ganglia disorders is expanding, possibly sharing some pathophysiological mechanisms. In this review, we will focus on thrombotic and nonthrombotic neurological manifestations of APS with particular attention to immune-mediated actions of aPL on the vascular system and the basal ganglia. PMID:24741580

Carecchio, M.; Cantello, R.; Comi, C.

2014-01-01

381

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

PubMed

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

Ea, Hang-Korng; Lioté, Frédéric

2014-05-01

382

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. PMID:19121871

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

2009-01-01

383

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. PMID:23599883

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

2013-01-01

384

Therapy insight: The recognition and treatment of retinal manifestations of systemic vasculitis.  

PubMed

A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and systemic lupus erythematosus. Less frequently occurring vasculitides include Takayasu's arteritis, Goodpasture's disease, microscopic polyangiitis and Henoch-Schönlein purpura, as well as vasculitis secondary to scleroderma and rheumatoid arthritis. This article describes the pathogenesis, clinical features and treatment of retinal manifestations of systemic vasculitides. PMID:16932736

Aristodemou, Petros; Stanford, Miles

2006-08-01

385

Endocrine Symptoms as the Initial Manifestation of Wilson’s Disease  

PubMed Central

Wilson’s disease is a rare genetic disorder of copper metabolism. The difference in copper tissue accumulation is responsible for the various clinical manifestations of this disorder. If left untreated, Wilson’s disease progresses to hepatic failure, severe neurological disability, and even death. Due to the complex clinical picture of Wilson’s disease, its diagnosis relies on a high index of suspicion. In our paper, we present endocrine symptoms suggesting the presence of insulinoma and hyperprolactinemia as the initial clinical manifestation of Wilson’s disease in a young female. Zinc acetate treatment resulted in the disappearance of hypoglycemia, galactorrhea, and menstrual abnormalities. PMID:22737053

Krysiak, Robert; Handzlik-Orlik, Gabriela; Okopien, Boguslaw

2012-01-01

386

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

PubMed Central

There is a spectrum of disorders that clinically manifest as a result of mast cell activation. A non-clonal form has emerged in the literature where many of the clinical features of systemic mastocytosis are shared despite having a distinct mast cell biology. In this review, we summarize key features of the science behind mast cell activation relevant to what is now known as non-clonal mast cell activation syndrome (nc-MCAS). We highlight the clinical manifestations of nc-MCAS with a focus on diagnosis and treatment. PMID:23212667

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

2012-01-01

387

A survey of European and Canadian rheumatologists regarding the treatment of patients with ankylosing spondylitis and extra-articular manifestations  

Microsoft Academic Search

Ankylosing spondylitis (AS) is a disabling inflammatory disease accompanied by a variety of extra-articular manifestations\\u000a in a significant number of patients. These manifestations, including Crohn’s disease, ulcerative colitis, psoriasis, and uveitis,\\u000a share a similar inflammatory mechanism with one another and with AS. Extra-articular manifestations are observed in a larger\\u000a percentage of patients with AS and spondyloarthritides (SpAs) than the normal

Filip Van den Bosch

2010-01-01

388

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

389

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

390

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. PMID:22253645

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

2011-01-01

391

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

2011-11-01

392

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

393

Manifest: A computer program for 2-D flow modeling in Stirling machines  

NASA Technical Reports Server (NTRS)

A computer program named Manifest is discussed. Manifest is a program one might want to use to model the fluid dynamics in the manifolds commonly found between the heat exchangers and regenerators of Stirling machines; but not just in the manifolds - in the regenerators as well. And in all sorts of other places too, such as: in heaters or coolers, or perhaps even in cylinder spaces. There are probably nonStirling uses for Manifest also. In broad strokes, Manifest will: (1) model oscillating internal compressible laminar fluid flow in a wide range of two-dimensional regions, either filled with porous materials or empty; (2) present a graphics-based user-friendly interface, allowing easy selection and modification of region shape and boundary condition specification; (3) run on a personal computer, or optionally (in the case of its number-crunching module) on a supercomputer; and (4) allow interactive examination of the solution output so the user can view vector plots of flow velocity, contour plots of pressure and temperature at various locations and tabulate energy-related integrals of interest.

Gedeon, David

1989-01-01

394

‘Dangerous and severe personality disorder’: A psychiatric manifestation of the risk society  

Microsoft Academic Search

This paper analyses the newly proposed United Kingdom (UK) psychiatric diagnosis of ‘dangerous and severe personality disorder’ (‘DSPD’). The aim is to show how the category of ‘DSPD’ manifests aspects of Beck's analysis of the ‘risk society’ and poststructural ‘governmentality’ approaches to understanding the notion of risk. Concepts such as ‘dangerousness’, ‘violence’ and ‘risk’ are critically reviewed in relation to

Kevin Corbett; Tristen Westwood

2005-01-01

395

Race Moves: Following Global Manifestations of New Racisms in Intimate Space  

ERIC Educational Resources Information Center

This article makes tentative links between abstract global forces and the affective and material reworking of race in intimate spaces of culture and community. Using postcolonial and psychoanalytic resources the article follows enduring manifestations of race as racism surfaces and is mobilized through global shifts of people, ideas and capital.…

Mishra Tarc, Aparna

2013-01-01

396

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

397

Manifestation Determinations under the New Individuals with Disabilities Education Act: An Update  

ERIC Educational Resources Information Center

This article provides an update of a previous analysis of the case law concerning manifestation determinations culminating in the revised pertinent provisions of the Individuals with Disabilities Education Act (IDEA) 2004. Specifically, the update consists of a synthesis of the legislative history, Office of Special Education Programs…

Zirkel, Perry A.

2010-01-01

398

Identifying the Clusters within Nonmotor Manifestations in Early Parkinson's Disease by Using Unsupervised Cluster Analysis  

PubMed Central

Background Classical and data-driven classifications of Parkinson's disease (PD) are based primarily on motor symptoms, with little attention being paid to the clustering of nonmotor manifestations. Methods Clinical data on demographic, motor and nonmotor features, including the Korean version of the sniffin' stick (KVSS) test results, and responses to the screening questionnaire of the nonmotor features were collected from 56 PD patients with disease onset within 3 years. Nonmotor subgroups were classified using unsupervised hierarchical cluster analysis (HCA). In addition to unsupervised HCA, we performed a cross-sectional analysis comparing the performance on the KVSS olfactory test with other nonmotor manifestations of the patients. Results Forty-nine patients (87.5%) had hyposmia based on the KVSS test. HCA suggested three nonmotor clusters for all PD patients and two nonmotor clusters in de novo PD patients, without a priori assumptions about the relatedness. In the cross-sectional analysis, dream-enactment behavior was more prevalent in patients with lower olfactory scores, implying impaired olfactory function (P?=?0.029 for all PD patients; P?=?0.046 for de novo PD patients). Conclusion We propose the existence of different clusters of nonmotor manifestations in early PD by using unsupervised hierarchical clustering. To our knowledge, this study is the first to report the identification of nonmotor subgroups based on unsupervised HCA of multiple nonmotor manifestations in the early stage of the disease. PMID:24643014

Yang, Hui-Jun; Kim, Young Eun; Yun, Ji Young; Kim, Han-Joon; Jeon, Beom Seok

2014-01-01

399

Manifestations of Neoliberal Discourses within a Local Job-Training Program  

ERIC Educational Resources Information Center

The purpose of conducting this study was to understand how neoliberal discourses manifest within the local context of a short-term, job-training program offered at a two-year college in the USA. Ethnographic data were collected at the local site through interviews, observations and document analysis. We then situated these data within a global…

Ayers, David Franklin; Carlone, David

2007-01-01

400

Gut microbiota composition and development of atopic manifestations in infancy: the KOALA Birth Cohort Study  

Microsoft Academic Search

Background and aims: Perturbations in intestinal microbiota composition due to lifestyle changes may be involved in the development of atopic diseases. We examined gut microbiota composition in early infancy and the subsequent development of atopic manifestations and sensitisation. Methods: The faeces of 957 infants aged 1 month and participating in the KOALA Birth Cohort Study were analysed using quantitative real-time

John Penders; Carel Thijs; Piet A van den Brandt; Ischa Kummeling; Bianca Snijders; Foekje Stelma; Hanne Adams; Ronald van Ree; Ellen E Stobberingh

2010-01-01

401

Spiral-type terahertz antennas and the manifestation of the Mushiake principle  

E-print Network

Spiral-type terahertz antennas and the manifestation of the Mushiake principle Ranjan Singh1 on the experimental and theoretical study of the resonant eigenmodes of spiral-type terahertz antennas. The analysis reflection and transmission coefficient. This property makes the spiral-type terahertz antenna not only

Oklahoma State University

402

Exploring the Manifestations of Anxiety in Children with Autism Spectrum Disorders  

ERIC Educational Resources Information Center

This study explores the manifestation and measurement of anxiety symptoms in 415 children with ASDs on a 20-item, parent-rated, DSM-IV referenced anxiety scale. In both high and low-functioning children (IQ above vs. below 70), commonly endorsed items assessed restlessness, tension and sleep difficulties. Items requiring verbal expression of worry…

Hallett, Victoria; Lecavalier, Luc; Sukhodolsky, Denis G.; Cipriano, Noreen; Aman, Michael G.; McCracken, James T.; McDougle, Christopher J.; Tierney, Elaine; King, Bryan H.; Hollander, Eric; Sikich, Linmarie; Bregman, Joel; Anagnostou, Evdokia; Donnelly, Craig; Katsovich, Lily; Dukes, Kimberly; Vitiello, Benedetto; Gadow, Kenneth; Scahill, Lawrence

2013-01-01

403

Hyperfine Interactions 127 (2000) 395398 395 Manifestation of nuclear spin-dependent P-odd  

E-print Network

Hyperfine Interactions 127 (2000) 395­398 395 Manifestation of nuclear spin-dependent P-odd Nuclear Physics Institute, 188350 Gatchina, Leningrad district, Russia E-mail: porsev@thd.pnpi.spb.ru P-odd effects caused by the nuclear spin-dependent electron­nucleon interaction are con- sidered. P-odd

Kozlov, Mikhail G

404

Jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert syndrome  

Microsoft Academic Search

Jaundice associated with hypertrophic pyloric stenosis was recognised in three patients; previous reports have suggested that this is a possible early manifestation of Gilbert syndrome. Most patients with Gilbert syndrome are homozygous for a (TA)7TAA polymorphism in the gene promoter coding for bilirubin glucuronosyltransferase. Two of the reported patients were homozygous for the (TA)7TAA polymorphism whereas the third was heterozygous

Pascale Trioche; Jacqueline Chalas; Jeanne Francoual; Liliane Capel; Albert Lindenbaum; Michel Odièvre; Philippe Labrune

1999-01-01

405

Local peritonitis as the first manifestation of Crohn's disease in a child  

PubMed Central

Crohn's disease (CD) can occur anywhere in the gastrointestinal tract from the mouth to the anus and sometimes it presents in a nonspecific manner. Herein we describe a case of local peritonitis as the first manifestation of CD in an 11-year-old girl, and the subsequent method of treatment. PMID:24741214

Kambouri, Katerina; Gardikis, Stefanos; Agelidou, Maria; Vaos, George

2014-01-01

406

Age and Gender Differences in Motivational Manifestations of the Big Five from Age 16 to 60  

ERIC Educational Resources Information Center

The present cross-sectional study investigated age and gender differences in motivational manifestations of the Big Five in a large German-speaking Internet sample (N = 19,022). Participants ranging in age from 16 to 60 years completed the Five Individual Reaction Norms Inventory (FIRNI; Denissen & Penke, 2008a), and two traditional Big Five…

Lehmann, Regula; Denissen, Jaap J. A.; Allemand, Mathias; Penke, Lars

2013-01-01

407

The spatial manifestation of forest succession in optical imagery The potential of multiresolution imagery  

E-print Network

in multiresolution imagery. This study demonstrates the potential to map tree size automatically from multiresolutionThe spatial manifestation of forest succession in optical imagery The potential of multiresolution changes in forest ecosystems over large areas in a timely and cost efficient manner. This study

Song, Conghe

408

Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE.  

PubMed

Shrinking lung syndrome (SLS) is a rare pulmonary complication of an underlying autoimmune disorder and is reported in association with systemic lupus erythematosus (SLE). We describe the favorable outcome of SLS in an 18-year-old Hispanic male who presented with SLS as the initial pulmonary manifestation of SLE. PMID:24836585

Pillai, S; Mehta, J; Levin, T; Muzumdar, H; Nandalike, K

2014-10-01

409

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

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

Gingival Metastasis from Ovarian Mucinous Cystadenocarcinoma as An Initial Manifestation (A Rare Case Report)  

Microsoft Academic Search

The metastasis of malignant tumors to the oral cavity remains a rare clinical entity. Most metastatic tumors have the propensity for involving the mandible rather than the oral soft tissues. Herein, we describe an unusual case of ovarian mucinous cystadenoca- rcinoma that metastasized to the mandibular gingiva as an initial manifestation. There is little information regarding metastatic ovarian cancer to

HIROKO SASAKI; NORIYUKI OHARA; TSUTOMU MINAMIKAWA; MASAHIRO UMEDA; TAKAHIDE KOMORI; NOBUE KOJIMA; NAOYA TAKEMURA; HIROKI MORITA; RYO SUGIHARA; EISUKE ENOKI; TOMOO ITOH

412

"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

413

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

414

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. PMID:19190525

Freeman, Alexandra F.; Holland, Steven M.

2009-01-01

415

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

416

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

417

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

418

Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers  

PubMed Central

The clinical spectrum of the anti-phospholipid syndrome (APS) is not limited to vascular thrombosis or miscarriages but includes additional manifestations that cannot be explained solely by a thrombophilic state. Anti-cardiolipin, anti-beta2 glycoprotein I (anti-?2GPI) and lupus anticoagulant (LA) assays are not only the formal diagnostic and classification laboratory tools but also parameters to stratify the risk to develop the clinical manifestations of the syndrome. In particular, anti-?2GPI antibodies reacting with an immunodominant epitope on domain I of the molecule were reported as the prevalent specificity in APS patients, correlating with a more aggressive clinical picture. Several laboratory assays to improve the diagnostic and predictive power of the standard tests have been proposed. Plates coated with the phosphatidylserine-prothrombin complex for detecting antibodies represent a promising laboratory tool correlating with LA and with clinical manifestations. Anti-phospholipid antibodies can be found in patients with full-blown APS, in those with thrombotic events or obstetric complications only or in asymptomatic carriers. An inflammatory second hit is required to increase the presence of ?2GPI in vascular tissues, eventually triggering thrombosis. Post-transcriptional modifications of circulating ?2GPI, different epitope specificities or diverse anti-?2GPI antibody-induced cell signaling have all been suggested to affect the clinical manifestations and/or to modulate their occurrence. PMID:25166960

2014-01-01

419

Aging-Related Systemic Manifestations in COPD Patients and Cigarette Smokers  

PubMed Central

Rationale Chronic obstructive pulmonary disease (COPD) is often associated with age-related systemic abnormalities that adversely affect the prognosis. Whether these manifestations are linked to the lung alterations or are independent complications of smoking remains unclear. Objectives To look for aging-related systemic manifestations and telomere shortening in COPD patients and smokers with minor lung destruction responsible for a decline in the diffusing capacity for carbon monoxide (DLCO) corrected for alveolar volume (KCO). Methods Cross-sectional study in 301 individuals (100 with COPD, 100 smokers without COPD, and 101 nonsmokers without COPD). Measurements and Main Results Compared to control smokers, patients with COPD had higher aortic pulse-wave velocity (PWV), lower bone mineral density (BMD) and appendicular skeletal muscle mass index (ASMMI), and shorter telomere length (TL). Insulin resistance (HOMA-IR) and glomerular filtration rate (GFR) were similar between control smokers and COPD patients. Smokers did not differ from nonsmokers for any of these parameters. However, smokers with normal spirometry but low KCO had lower ASMMI values compared to those with normal KCO. Moreover, female smokers with low KCO, had lower BMD and shorter TL compared to those with normal KCO. Conclusions Aging-related abnormalities in patients with COPD are also found in smokers with minor lung dysfunction manifesting as a KCO decrease. Decreased KCO might be useful, particularly among women, for identifying smokers at high risk for aging-related systemic manifestations and telomere shortening. PMID:25785739

Boyer, Laurent; Marcos, Elisabeth; Margarit, Laurent; Le Corvoisier, Philippe; Vervoitte, Laetitia; Hamidou, Leila; Frih, Lamia; Audureau, Etienne; Covali-Noroc, Ala; Andujar, Pascal; Saakashvili, Zakaria; Lino, Anne; Ghaleh, Bijan; Hue, Sophie; Derumeaux, Geneviève; Housset, Bruno; Dubois-Randé, Jean-Luc; Boczkowski, Jorge; Maitre, Bernard; Adnot, Serge

2015-01-01

420

Clinical and immunological manifestations in 134 Puerto Rican patients with systemic lupus erythematosus.  

PubMed

To gain a better understanding of systemic lupus erythematosus (SLE) in Puerto Ricans we studied the clinical and serologic manifestations in a cohort of 134 patients. The female to male ratio was 18:1. Mean age at diagnosis was 32 +/- 12 y. The mean duration of disease and follow-up were 7.4 +/- 6.0 and 5.8 +/- 6.0 years respectively. Mortality was 3%. Photosensitivity (76.9%) and malar rash (71.9%) were the most common clinical manifestations. Arthritis was observed in 67.5% of patients. Anemia was seen in 67.2% of patients, but only 12.7% had autoimmune hemolytic anemia. Leukopenia (41.8%) and lymphopenia (64.9%) were also common. Serositis was observed in only 28%. Severe kidney damage such as nephrotic syndrome (14.2%) or renal failure (4%) was infrequent. Cardiovascular (12.7%) and neurologic (9.0%) manifestations were also uncommon. Antinuclear antibodies (ANA) were detected in 93.3%, anti-dsDNA antibodies in 54.5%, anti-Ro antibodies in 30.1% and anti-La antibodies in 14.2%. Low C3 and low C4 were observed in 38.3% and 35.7% respectively. This study suggests that Puerto Ricans with SLE present a mild form of disease predominantly manifested by cutaneous, musculoskeletal and hematologic involvement, but low prevalence of major organ damage and low mortality. PMID:10413206

Vilá, L M; Mayor, A M; Valentín, A H; García-Soberal, M; Vilá, S

1999-01-01

421

Manifesting Destiny: A Land Education Analysis of Settler Colonialism in Jamestown, Virginia, USA  

ERIC Educational Resources Information Center

Globally, colonization has been and continues to be enacted in the take-over of Indigenous land and the subsequent conversion of agriculture from diverse food and useful crops to large-scale monoculture and cash crops. This article uses a land education analysis to map the rise of the ideology and practices of Manifest Destiny in Virginia.…

McCoy, Kate

2014-01-01

422

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

423

Pulmonary edema following scorpion envenomation: Mechanisms, clinical manifestations, diagnosis and treatment  

Microsoft Academic Search

Scorpion envenomation is common in tropical and subtropical regions. Cardio-respiratory manifestations, mainly cardiogenic shock and pulmonary edema, are the leading causes of death after scorpion envenomation. The mechanism of pulmonary edema remains unclear and contradictory conclusions were published. However, most publications confirm that pulmonary edema has been attributed to acute left ventricular failure. Cardiac failure can result from massive release

Mabrouk Bahloul; Anis Chaari; Hassen Dammak; Mohamed Samet; Kamilia Chtara; Hedi Chelly; Chokri Ben Hamida; Hatem Kallel; Mounir Bouaziz

424

Is Uterine Papillary Serous Adenocarcinoma a Manifestation of the Hereditary Breast–Ovarian Cancer Syndrome?  

Microsoft Academic Search

Background. Uterine papillary serous carcinoma (UPSC) shares common pathologic, genetic, and clinical features with other serous cancers of müllerian origin. The most common histologic type of ovarian tumor associated with BRCA mutations is papillary serous. Because of these histologic similarities, we postulated that, in some cases, UPSC may be a manifestation of a field defect in BRCA1 carriers, which also

Ran Goshen; William Chu; Laurie Elit; Tuya Pal; Jalil Hakimi; Ida Ackerman; Anthony Fyles; Margot Mitchell; Steven A. Narod

2000-01-01

425

Thermal dilepton production from dropping rho based on the vector manifestation  

Microsoft Academic Search

We study the pion electromagnetic form factor and the dilepton production rate in hot matter using the hidden local symmetry theory as an effective field theory for pions and rho mesons. In this framework, the chiral symmetry restoration is realized as the vector manifestation (VM) in which the massless vector meson becomes the chiral partner of the pion, giving a

Masayasu Harada; Chihiro Sasaki

2006-01-01

426

Psychopathological Manifestations of Children with Intellectual Disabilities According to Their Cognitive and Adaptive Behavior Profile  

ERIC Educational Resources Information Center

Children with intellectual disabilities show deficits in cognitive abilities and adaptive behavior which increase the risk of psychopathological disorders. This exploratory study aims at delineating profiles of children based on their cognitive functioning and adaptive behaviors, and to compare them on psychopathological manifestations. A…

Tremblay, Karine N.; Richer, Louis; Lachance, Lise; Cote, Alain

2010-01-01

427

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

428

Epidemiological Features and Clinical Manifestations of Lyme Borreliosis in Korea during the Period 2005-2012.  

PubMed

Lyme borreliosis is one of the most common tick-borne infections in the northern hemisphere. However, the epidemiological features and clinical manifestations of this disease in Korea are unknown. The present study is the first to investigate the characteristics of Lyme borreliosis in Korea. We traced suspected cases of Lyme borreliosis during the period 2005-2012. Of the 16 identified patients with the disease, 11 had acquired autochthonous infection within Korea, while 5 patients were infected outside Korea. The history of past exposure was investigated in 8 of the 11 patients with autochthonous infection; 5 of these 8 patients (62.5%) were suspected to have acquired the infection in the northeastern alpine region. Clinically, of 11 patients with autochthonous infection, 6 (54.5%) showed early skin manifestations, 4 (36.4%) showed neurological manifestations, and 1 (9.1%) showed acrodermatitis chronica atrophicans. In conclusion, Lyme borreliosis could be endemic in the northeastern alpine region of Korea, and neurological and early skin manifestations are likely to be the major clinical characteristics of autochthonous Lyme borreliosis in Korea. PMID:25420645

Moon, Shinje; Hong, Yeongseon; Hwang, Kyu-Jam; Kim, Suyeon; Eom, Jihye; Kwon, Donghyok; Park, Ji-Hyuk; Youn, Seung-Ki; Sohn, Aeree

2015-01-26

429

Association between different clinical manifestations of Lyme disease and different species of Borrelia burgdorferi sensu lato  

Microsoft Academic Search

Borrelia burgdorferi sensu lato, the aetiological agent of Lyme disease, has been sub-divided into three species: B. burgdorferi sensu stricto, B. garinii and B. afzelii. We and other authors have hypothesized an association between the three species of B. burgdorferi sensu lato and some of the different clinical manifestations of Lyme disease. In order to demonstrate this hypothesis, we analysed

T Balmelli; J.-C Piffaretti

1995-01-01

430

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

431

Periorbital Oedema and Erythema as a Manifestation of Discoid Lupus erythematosus  

Microsoft Academic Search

Periorbital oedema and erythema are a rare cutaneous manifestation of discoid erythematosus (DLE) which is well known to ophthalmologists but less to dermatologists. We report the case of a 74-year-old female patient with periorbital oedema and erythema. Since swelling of the eyelid is common in the dermatological practice, dermatologists should be aware of the differential diagnosis and its occurrence in

R. P. Braun; L. E. French; I. Massouyé; J.-H. Saurat

2002-01-01

432

The incidence of skin manifestations by dermatophytes in patients with psoriasis  

Microsoft Academic Search

Thirty-four psoriatic patients (23 males, 11 females) were found to have skin manifestations of dermatophyte infection. Tinea pedis was observed in 20 cases, tinea cruris in 6 and tinea mannum in 2. T. rubrum was the causative agent in all of these with the exception of 2 cases caused by E. floccosum. Lesions of tinea corporis were found intermingled with

I. Alteras; A. Ingberg; Rina Segal; Dalia Schvili

1986-01-01

433

Manifest and Latent Components in Methadone Maintenance: The Methadone Maintenance Game  

ERIC Educational Resources Information Center

This paper discusses various difficulties which arise when the staff of a methadone maintenance clinic must come to grips with the manifest and latent issues in service delivery. A solution is suggested which involves severing the tie between methadone and the behaviors which are reinforced by its use. (Author)

King, Charles H.

1975-01-01

434

The Sources and Manifestations of Stress amongst School-Aged Dyslexics, Compared with Sibling Controls  

ERIC Educational Resources Information Center

All school children experience stress at some point in their school careers. This study investigates whether dyslexic children, by way of their educational and social difficulties, experience higher levels of stress at school. The School Situation Survey was used to investigate both the sources and manifestations of stress amongst dyslexic…

Alexander-Passe, Neil

2008-01-01

435

Cobalamin deficiency as an extra intestinal manifestation of Helicobacter pylori infection  

PubMed Central

We read with great interest the excellent review by Wong et al on extra intestinal manifestations of Helicobacter pylori (H. pylori) infection published in the journal. This is a well-documented and structured review. However, I believe that Wong et al failed to report the relationship between H. pylori infection and cobalamin.

Andrès, Emmanuel

2015-01-01

436

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

437

Are Personality Disorders Psychological Manifestations of Executive Function Deficits? Bivariate Heritability Evidence from a Twin Study  

Microsoft Academic Search

This study tested whether personality disorders may be the psychological manifestations of executive function deficits by examining their bivariate heritability in a community sample of 314 twins (ages 5–17 years; M age = 9.7; 96 monozygotic pairs and 61 dizygotic pairs). The parents of the twins completed the Coolidge Personality and Neuropsychological Inventory (Coolidge, 1998; Coolidge et al., 2002). Heritability

Frederick L. Coolidge; Linda L. Thede; Kerry L. Jang

2004-01-01

438

Pseudohemangioma of the Vertebra: An Unusual Radiographic Manifestation of Primary Ewing's Sarcoma  

Microsoft Academic Search

Summary: Primary Ewing's sarcoma (ES) of the spine is uncommon, exhibiting a variety of appearances on plain- film radiographs and cross-sectional images. We report the unusual CT imaging manifestations of a primary ES with a coarse trabecular pattern that mimicked an aggressive hemiangioma of the cervical spine. Ewing's sarcoma (ES) is an aggressive malignant neoplasm accounting for 6% to 8%

Joshua A. Bemporad; Gordon Sze; John C. Chaloupka; Charles Duncan

1999-01-01

439

Influence of reflector edges diffraction on manifestation of backscatter amplification effect  

Microsoft Academic Search

It is shown that in a regime of weak turbulence the edge diffraction on a reflector has a profound effect on intensity distribution of the reflected wave and on the manifestation of backscatter amplification. Therewith the dependence of the amplification factor on the Fresnel number of the reflector radius has an oscillating nature both for the corner reflector and for

Victor A. Banakh

1993-01-01

440

Solar Wind: Manifestations of Solar Activity E N CYC LO PE D IA O F AS T R O N O MY AN D AS T R O PHYS I C S Solar Wind: Manifestations of Solar  

E-print Network

Solar Wind: Manifestations of Solar Activity E N CYC LO PE D IA O F AS T R O N O MY AN D AS T R O PHYS I C S Solar Wind: Manifestations of Solar Activity The Sun's outer atmosphere, the corona, is continually heated and expands to create the solar wind. Solar activity waxes and wanes with the 11 yr cycle

Webb, David F.

441

Mixture Item Response Theory-MIMIC Model: Simultaneous Estimation of Differential Item Functioning for Manifest Groups and Latent Classes  

ERIC Educational Resources Information Center

This study uses a new psychometric model (mixture item response theory-MIMIC model) that simultaneously estimates differential item functioning (DIF) across manifest groups and latent classes. Current DIF detection methods investigate DIF from only one side, either across manifest groups (e.g., gender, ethnicity, etc.), or across latent classes…

Bilir, Mustafa Kuzey

2009-01-01

442

Bringing Social Identity to Work: The Influence of Manifestation and Suppression on Perceived Discrimination, Job Satisfaction, and Turnover Intentions  

Microsoft Academic Search

In the current article, we explored whether manifesting or suppressing an identity (race\\/ethnicity, gender, age, religion, sexual orientation, or disability) at work is related to perceived discrimination, job satisfaction, and turnover intentions. Participants included 211 working adults who completed an online survey. The results showed that efforts to suppress a group identity were positively (and behavioral manifestations of group identity

Juan M. Madera; Eden B. King; Michelle R. Hebl

2012-01-01

443

Manifestly gauge invariant theory of the nonlinear cosmological perturbations in the leading order of the gradient expansion  

Microsoft Academic Search

In the full nonlinear cosmological perturbation theory in the leading order of the gradient expansion, all the types of the gauge invariant perturbation variables are defined. The metric junction conditions across the spacelike transition hypersurface are formulated in a manifestly gauge invariant manner. It is manifestly shown that all the physical laws such as the evolution equations, the constraint equations,

Takashi Hamazaki

2011-01-01

444

14 CFR 121.695 - Disposition of load manifest, dispatch release, and flight plans: Domestic and flag operations.  

Code of Federal Regulations, 2010 CFR

...manifest, dispatch release, and flight plans: Domestic and flag operations...manifest, dispatch release, and flight plans: Domestic and flag operations...carry in the airplane to its destination— (1) A copy of the completed...and (3) A copy of the flight plan. (b) The...

2010-01-01

445

A Learning and Memory Area in the Octopus Brain Manifests a Vertebrate-Like Long-Term Potentiation  

E-print Network

A Learning and Memory Area in the Octopus Brain Manifests a Vertebrate- Like Long-Term Potentiation in the octopus brain manifests a vertebrate-like long-term potentiation. J Neurophysiol 90: 3547­3554, 2003 and memory were investigated in the octopus using a brain slice prepara- tion of the vertical lobe, an area

Hochner, Binyamin

446

High prevalence of vitamin D deficiency and insufficiency in patients with manifest Huntington disease  

PubMed Central

Vitamin D deficiency and insufficiency are common in older institutionalized people and known to be associated with muscle weakness, impaired balance and increased fall risk. Falls and balance problems are common in people with Huntington disease (HD). Despite this, the prevalence of vitamin D deficiency in patients with manifest HD has never been investigated. Serum 25(OH)D levels were measured in routinely drawn blood samples from 28 Dutch institutionalized patients with manifest Huntington disease. Mean serum 25(OH)D level was 33 nmol/l (SD 15). Twenty-five subjects (89%) were vitamin D deficient or insufficient (25(OH)D < 50 nmol/L). A positive association was found between serum 25(OH)D levels and Functional Ambulation Classification (FAC) scores (p = 0.023). PMID:24516688

Chel, Victor GM; Ooms, Marcel E; van der Bent, Jessie; Veldkamp, Fleur; Roos, Raymund AC; Achterberg, Wilco P; Lips, Paul

2013-01-01

447

Manifestation of a nonclassical Berry phase of an electromagnetic field in atomic Ramsey interference  

NASA Astrophysics Data System (ADS)

The Berry phase acquired by an electromagnetic field undergoing an adiabatic and cyclic evolution in phase space is a purely quantum-mechanical effect of the field. However, this phase is usually accompanied by a dynamical contribution and cannot be manifested in any light-beam interference experiment because it is independent of the field state. We here show that such a phase can be produced using an atom coupled to a quantized field and driven by a slowly changing classical field, and it is manifested in atomic Ramsey interference oscillations. We also show how this effect may be applied to one-step implementation of multiqubit geometric phase gates, which is impossible by previous geometric methods. The effects of dissipation and fluctuations in the parameters of the pump field on the Berry phase and visibility of the Ramsey interference fringes are analyzed.

Zheng, Shi-Biao

2012-02-01

448

Personality in its natural habitat: manifestations and implicit folk theories of personality in daily life.  

PubMed

To examine the expression of personality in its natural habitat, the authors tracked 96 participants over 2 days using the Electronically Activated Recorder (EAR), which samples snippets of ambient sounds in participants' immediate environments. Participants' Big Five scores were correlated with EAR-derived information on their daily social interactions, locations, activities, moods, and language use; these quotidian manifestations were generally consistent with the trait definitions and (except for Openness) often gender specific. To identify implicit folk theories about daily manifestations of personality, the authors correlated the EAR-derived information with impressions of participants based on their EAR sounds; judges' implicit folk theories were generally accurate (especially for Extraversion) and also partially gender specific. The findings point to the importance of naturalistic observation studies on how personality is expressed and perceived in the natural stream of everyday behavior. PMID:16737378

Mehl, Matthias R; Gosling, Samuel D; Pennebaker, James W

2006-05-01

449

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

450

IgA deficiency, superior mediastinal tumour with unusual clinical manifestations.  

PubMed

The clinical course of a 17-year-old man with selective IgA deficiency and anaplastic carcinoma in the superior mediastinum is described. High swinging fevers, neutrophil leucocytosis reaching 60 x 10(9)/l, polyarthralgia and skin vasodilatation were unusual manifestations of the tumour. Marked tissue reactions in the neck and polyserositis occurred with a neutrophil granulocyte tissue reaction and progressed to fibrosis. Circulating immune complexes were present and IgE levels were grossly raised. It was not established whether the unusual clinical manifestations were due solely to the tumour or were an abnormal response of an IgA-deficient host. Review of the literature of malignancy in selective IgA deficiency casts doubt upon an increased incidence of neoplasm in this immune deficiency. PMID:288401

Lee, C H; Quin, J W; Wong, C S; Grace, C S; Rozenberg, M C

1979-06-01

451

Pet killing as a manifestation of impulse control disorder secondary to pramipexol.  

PubMed

Impulse control disorders are frequent in Parkinson disease and are commonly associated with dopamine agonists intake. Typical manifestations include punding, hypersexuality, pathological gambling, and other compulsive behaviors. Symptoms often promptly disappear when dopamine agonists are discontinued, but if the origin is misinterpreted, symptoms may become a problem. We here report the case of a patient with juvenile Parkinson disease treated with 4.5 mg/d of pramipexol, who developed the need to adopt cats. He adopted almost 50 of them, but after he adopted them, he felt the need to kill them. The case became well known, and he became depressed and isolated. He was admitted to a psychiatric hospital and thought that he was a psychotic. However, when pramipexol was replaced by levodopa, the symptoms disappeared. This case illustrates the wide range of manifestations of impulse control disorders and warrants the inclusion of violent behaviors among them. PMID:25768852

Micheli, Federico; Pellene, Alejandro; Arcushin, Daniela; Calzinari, Aldo; Farret, Michel Sáenz

2015-01-01

452

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

453

[Exfoliatio areata linguae et mucosae oris: a mucous membrane manifestation of psoriasis pustulosa?].  

PubMed

Lesions of the oral mucosa are frequently described in association with psoriasis, particularly in the pustular type. Controversy surrounds the question whether mucosal lesions can be considered as oral manifestation of psoriasis. Two patients presented with concurrent pustular psoriasis and mucosal lesions with the characteristic picture of geographic tongue. Histopathology of the mucosa showed typical features of psoriasis such as marked acanthosis, clubbing of the rete ridges, focal parakeratosis and neutrophilic infiltrates. There was parallel improvement of the skin and the mucosal lesions with systemic retinoid treatment. On the basis of the histopathological features and the clinical course we favour the hypothesis that geographic tongue is an oral manifestation of pustular psoriasis. PMID:9879485

Casper, U; Seiffert, K; Dippel, E; Zouboulis, C C

1998-11-01

454

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

455

Oral side effects of head and neck irradiation: correlation between clinical manifestations and laboratory data.  

PubMed

Salivary flow rate and composition, oral microflora and clinical manifestations of radiation damage were studied in 32 patients treated with external irradiation to head and neck areas. Several parameters were investigated: field arrangement, amount of salivary glands irradiated, clinical manifestations such as dryness of the mouth, taste impairment, dysphagia, salivary secretion and composition, and oral yeast flora. The salivary glands have a greater sensitivity to radiation damage than the gustatory tissues. The decrease in salivary secretion is accompanied by a rise in salivary sodium concentration, and in oral yeast flora. The clinical symptomatology was correlated with the amount of salivary glands irradiated. We found that most of the parotids have to be outside of the treated volume, when the rest of the major salivary glands are irradiated, to prevent severe dryness phenomena. PMID:3957738

Kuten, A; Ben-Aryeh, H; Berdicevsky, I; Ore, L; Szargel, R; Gutman, D; Robinson, E

1986-03-01

456

Psychiatric manifestations as primary symptom of neurosyphilis among HIV-negative patients.  

PubMed

This study characterizes psychiatric manifestations as a primary symptom of neurosyphilis (NS). Fifty-two of the 169 NS patients presented with psychiatric manifestations, many patients had characteristics of more than one syndrome, including cognitive impairment, personality disorders, delirium, hostility, dysarthria, confusion, disruption of their sleep-wake cycle, fecal and urinary incontinence, dysphoria, paranoia, hallucinations, expansive mood, and mania. Fifty-two patients had positive sera RPR and T. pallidum particle agglutination (TPPA), 75% had positive CSF RPR, 96.2% had positive CSF TPPA, 44.2% had CSF pleocytosis and elevated CSF proteins, and 70.0% had nonspecific, abnormal brain MRIs. These results indicate that NS mimics almost all psychiatric disorders. PMID:24737221

Lin, Li-Rong; Zhang, Hui-Lin; Huang, Song-Jie; Zeng, Yan-Li; Xi, Ya; Guo, Xiao-Jing; Liu, Gui-Li; Tong, Man-Li; Zheng, Wei-Hong; Liu, Li-Li; Yang, Tian-Ci

2014-01-01

457

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. PMID:22693492

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

2012-01-01

458

Radiologic manifestations of bronchoscopic lung volume reduction in severe chronic obstructive pulmonary disease.  

PubMed

OBJECTIVE. Bronchoscopic lung volume reduction promises to become an effective treatment option in severe chronic obstructive pulmonary disease. Several techniques are currently being investigated, including implantation of devices into the lung and instillation of hot water vapor or polymer. This article reviews the spectrum of radiologic manifestations on chest radiography and CT that occur after the intervention. CONCLUSION. Familiarity with the intended effects and adverse events will aid the radiologist in supporting bronchoscopic lung volume reduction. PMID:25714276

Grabenhorst, Matthias; Schmidt, Bernd; Liebers, Uta; Oestmann, Jörg-Wilhelm

2015-03-01

459

Benign nodular tertiary syphilis: a rare presenting manifestation of HIV infection.  

PubMed

Late cutaneous syphilis has become a rarity as a result of the effective treatment of early and latent syphilis with antibiotics. We report a heterosexual male who presented with multiple large annular plaques of two years duration. Skin biopsy revealed endarteritis and non-caseating granulomas in the dermis along with plasma cells. VDRL and TPHA were reactive. ELISA for HIV-1 antibodies was reactive. This is the first report of nodular tertiary syphilis as the presenting manifestation of HIV. PMID:21382288

Revathi, T N; Bhat, Shilpa; Asha, G S

2011-01-01

460

Case study: is selective mutism a manifestation of dissociative identity disorder?  

PubMed

Selective mutism is a rare psychiatric disorder that usually has its onset in early childhood. This case study describes a patient in whom selective mutism developed in adolescence. It was later discovered that he had dissociative identity disorder. The study documents his history, which included violence, abuse, and threats to keep silent. The study suggests that in cases presenting with significant trauma or abuse, selective mutism may be a manifestation of dissociative identity disorder. PMID:7649956

Jacobsen, T

1995-07-01

461

Oral Manifestation of Autosomal Recessive Congenital Ichthyosis in a 2-Year-Old Patient  

PubMed Central

Ichthyosis is a heterogeneous family of hereditary keratinisation disorders mostly characterized by variable erythema of the whole body and different scaling patterns. Although these disorders affect tissues of epidermal origin, there is little evidence regarding the oral and dental manifestations of Lamellar Ichthyosis. A case report of early childhood caries in lamellar ichthyosis is presented and the dental consideration and management is discussed in this paper. PMID:24995134

Ramar, Kavitha; Annamalai, Sankar; Hariharavel, V. P.; Aravindhan, R.; Ganesh, C.; Ieshwaryah, K.

2014-01-01

462

Anasarca as the presenting manifestation of parvovirus B19 associated juvenile dermatomyositis  

Microsoft Academic Search

Anasarca as the presenting manifestation of juvenile dermatomyositis (JDMS) is extremely rare. We report a case of a 4-year-old\\u000a boy who was initially managed for nephrotic syndrome in view of anasarca and mild hypoalbuminemia. Later, at presentation\\u000a to our institute, a diagnosis of severe edematous JDMS was made in view of associated profound muscle weakness and characteristic\\u000a skin changes. The

Shanmuganathan Chandrakasan; Surjit Singh; R. K. Ratho; Sharat Kumar; B. Mishra

2009-01-01

463

Umbilical Metastasis as Primary Manifestation of Cancer: A Small Series and Review of the Literature  

PubMed Central

Umbilical metastasis is a rare manifestation of intra-abdominal cancer. It appears either as the first sign of a primary malignancy or as metastatic site of an already diagnosed cancer, representing an ominous prognostic finding. We report three cases of umbilical metastasis as the first sign of an underlying malignancy. Hypotheses about pathophysiology of umbilical metastasis are based on the embryological origin of the umbilicus and its residual communication with systematic, portal and lymphatic circulation. PMID:25478400

Psarras, Kyriakos; Baltatzis, Minas; Notopoulos, Athanasios; Nikolaidou, Christina

2014-01-01

464

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

Microsoft Academic Search

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, as the extent to which the notion of being LG was perceived to cause undesirable

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

2006-01-01

465

Manifestation of Hamiltonian chaos in an open quantum system with ballistic atoms in an optical lattice  

E-print Network

Manifestation of dynamical instability and Hamiltonian chaos in the fundamental near-resonant matter-radiation interaction has been found analitically and in a Monte Carlo simulation in the behavior of atoms moving in a rigid optical lattice. Character of diffusion of spontaneously emitting atoms changes abruptly in the range of the values of parameters and initial conditions where their Hamiltonian dynamics is shown to be chaotic

V. Yu. Argonov; S. V. Prants

2008-05-12

466

Neurologic manifestations of the antiphospholipid syndrome: Integrating molecular and clinical lessons  

Microsoft Academic Search

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by autoantibody production and thrombosis\\u000a or pregnancy morbidity. The most prevalent neurologic manifestation of APS is cerebrovascular ischemic events due to arterial\\u000a thromboses. Antiphospholipid antibodies can also cause neurologic impairments unrelated to thrombosis, through antibody-cellular\\u000a interactions, possibly because of a disrupted blood-brain barrier. Antiplatelet or anticoagulant therapies are currently indicated

Eyal Muscal; Robin L. Brey

2008-01-01

467

Catalogue of the main gas manifestation of Greece: Geochemical characterisation and preliminary gas hazard assessment  

NASA Astrophysics Data System (ADS)

Like other geodynamically active areas, the Hellenic territory is also affected by a large number of geogenic gas manifestations. These occur either in form of point sources (fumaroles, mofettes, bubbling gases) or as diffuse soil gas emanations. The present work produced a first catalogue of the geogenic gas manifestations of the whole Hellenic territory also considering a few literature data. All collected samples were analysed for their chemical composition (He, Ne, Ar, O2, N2, H2, H2S, CO, CH4 and CO2) and isotopic composition (He, CO2-C, CH4-C, N2-N). Geogenic sources release huge amounts of gases, which, apart from having important influences on the global climate, could have strong impact on human health. Gases have both acute and chronic effects. Carbon Dioxide and Hydrogen Sulphide are the main gases responsible for acute mortality due to their asphyxiating and/or toxic properties. Methane instead represents a risk for its explosive properties. Gas hazard is often disregarded because in fatal episodes connected to geogenic gases the death cause is often not correctly attributed. Due to the fact that geodynamic active areas can release geogenic gases for million years over wide areas, it is important not to underestimate potential risks. A preliminary estimation of the gas hazard has been made for the time period of the last 20 years considering the whole population of Greece. In this period at least two fatal episodes with a total of three victims could be certainly attributed to geogenic gases (specifically CO2). This would give a risk of 1.3×10-8 fatality from geogenic gas manifestations per annum. Such value, although probably underestimated, is much lower than most other natural or anthropogenic risks. Nevertheless this risk, being unevenly distributed along the whole territory, should not be overlooked especially in areas with high density of gas manifestations and high soil gas fluxes.

D'Alessandro, Walter; Kyriakopoulos, Konstantinos; Calabrese, Sergio

2014-05-01

468

Manifestation of fatigue in myoelectric signals of dynamic contractions produced during playing PC games  

Microsoft Academic Search

This paper investigates manifestation of fatigue in myoelectric signals during dynamic contractions produced whilst playing PC games. The hand's myoelectric signals were collected in 26 independent sessions with 10 subjects. Two methods, spectral analysis and time-scale analysis, were applied to compute signal frequency and least-square linear regression was used to model the trend of frequency shift. Non-parametric statistical methods were

Mohammadreza Asghari Oskoei; Huosheng Hu; John Q. Gan

2008-01-01

469

Clinical and Imaging Manifestations of Hemorrhagic Pulmonary Leptospirosis: A State-of-the-Art Review  

Microsoft Academic Search

Leptospirosis, a spirochetal zoonosis, is frequently unrecognized due to its manifestation as an undifferentiated fever. It\\u000a is an emerging infectious disease that has changed from an occupational disease of veterinarians, farmers, butchers, and other\\u000a animal handlers to a cause of epidemics in poor and decayed urban communities in developing countries. Humans are infected\\u000a when mucous membranes or abraded skin come

Edson Marchiori; Sílvia Lourenço; Sérgio Setúbal; Gláucia Zanetti; Taisa Davaus Gasparetto; Bruno Hochhegger

2011-01-01

470

Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease.  

PubMed

A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. Mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein. PMID:215941

Searles, R P; Mladinich, E K; Messner, R P

1978-12-01

471

Abrupt changes in alpha decay systematics as a manifestation of collective nuclear modes  

E-print Network

An abrupt change in $\\alpha$ decay systematics around the N=126 neutron shell closure is discussed. It is explained as a sudden hindrance of the clustering of the nucleons that eventually form the $\\alpha$ particle. This is because the clustering induced by the pairing mode acting upon the four nucleons is inhibited if the configuration space does not allow a proper manifestation of the pairing collectivity.

Chong Qi; A. N. Andreyev; M. Huyse; R. J. Liotta; P. Van Duppen; R. A. Wyss

2010-06-05

472

Type I interferon system activation and association with disease manifestations in systemic sclerosis  

Microsoft Academic Search

ObjectivesTo study the presence of interferogenic autoantibodies in systemic sclerosis (SSc) and their correlation with clinical manifestations, serum levels of interferon ? (IFN?) and chemokines of importance in the disease process.MethodsPeripheral blood mononuclear cells (PBMCs) or purified plasmacytoid dendritic cells (pDCs) from healthy donors were stimulated with sera from patients with SSc (n=70) or healthy individuals (n=30), together with necrotic

Maija-Leena Eloranta; Karin Franck-Larsson; Tanja Lövgren; Sebastian Kalamajski; Anders Rönnblom; Kristofer Rubin; Gunnar V Alm; Lars Rönnblom

2010-01-01

473

Adherence to guideline-recommended therapies among patients with diverse manifestations of vascular disease  

PubMed Central

Background Current guidelines recommend aspirin, statins, angiotensin-converting enzyme inhibitors (ACEIs), and smoking abstinence for all patients with vascular disease. There is little data on the variation in adherence to guideline-recommended therapies among patients with different clinical manifestations of vascular disease. Purpose To analyze the variation in adherence to guideline-recommended therapies among patients with diverse manifestations of vascular disease. Methods We analyzed a comprehensive database of all patients with critical limb ischemia, claudication, acute limb ischemia, carotid artery stenosis, subclavian artery stenosis, renal artery stenosis, or mesenteric ischemia who underwent angiography between 2006 and 2013 at a multidisciplinary vascular center. Results Among 1,114 patients with vascular disease, adherence to guideline-recommended therapy at time of angiography included use of aspirin in 936 (84%), statins in 753 (68%), ACEIs in 673 (60%), and smoking abstinence in 788 (71%). A total of 335 (30%) patients utilized all four guideline-recommended therapies. Adherence to four guideline-recommended therapies was lowest among patients with acute limb ischemia (14%) and highest among patients with renal artery stenosis (37%). Among all patients with vascular disease, the range of adherence to individual guidelines was 64%–91% for aspirin, 43%–83% for statins, 49%–66% for ACEIs, and 47%–78% for smoking abstention. Conclusion The majority of patients with diverse manifestations of vascular disease take aspirin and abstain from smoking while fewer patients are prescribed ACEIs and statins. Among the current recommendations, statins have the widest variation in adherence. Less than one-third of patients with diverse manifestations of vascular disease are prescribed all four guideline-recommended therapies. PMID:25767395

Chen, Debbie C; Armstrong, Ehrin J; Singh, Gagan D; Amsterdam, Ezra A; Laird, John R

2015-01-01

474

Pulmonary Vascular Manifestations of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler Disease)  

Microsoft Academic Search

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1\\/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene

Vincent Cottin; Sophie Dupuis-Girod; Gaetan Lesca; Jean-François Cordier

2007-01-01

475

Infrequent Manifestations of Kabuki Syndrome in a Patient with Novel MLL2 Mutation.  

PubMed

We present a case of a 9-month-old Hispanic female with Kabuki syndrome with some infrequent manifestations including a single umbilical artery, butterfly vertebrae, a small larynx, a preauricular pit, microtia with internal ear abnormalities, abnormal calcium metabolism, premature thelarche, neonatal/persistent hypoglycemia and eventration of the diaphragm. She was found to have a previously unreported nonsense MLL2 mutation. This is the first case that includes all such findings occurring simultaneously that was genotyped. PMID:23239960

Zarate, Y A; Zhan, H; Jones, J R

2012-10-01

476

Infrequent Manifestations of Kabuki Syndrome in a Patient with Novel MLL2 Mutation  

PubMed Central

We present a case of a 9-month-old Hispanic female with Kabuki syndrome with some infrequent manifestations including a single umbilical artery, butterfly vertebrae, a small larynx, a preauricular pit, microtia with internal ear abnormalities, abnormal calcium metabolism, premature thelarche, neonatal/persistent hypoglycemia and eventration of the diaphragm. She was found to have a previously unreported nonsense MLL2 mutation. This is the first case that includes all such findings occurring simultaneously that was genotyped. PMID:23239960

Zarate, Y.A.; Zhan, H.; Jones, J.R.

2012-01-01

477

Musculoskeletal manifestations of brucellosis: A study of 90 cases in Israel  

Microsoft Academic Search

Rheumatological manifestations are frequently reported in patients with brucellosis.In a retrospective study of 90 patients diagnosed with brucellosis over a period of 18 years, 83 (92%) patients were Bedouins, 55 of whom (61%) reported ingestion of unpasteurized goat milk and goat milk products. The male\\/female ratio was 1:1, and the adult to child ratio was 3:2. The mean age of

Nurit Zaks; Shaul Sukenik; Michael Alkan; Daniel Flusser; Lily Neumann; Dan Buskila

1995-01-01

478

Effect of gender on the manifestations of celiac disease: Evidence for greater malabsorption in men  

Microsoft Academic Search

Objective. Because celiac disease is a female-predominant disease we investigated the influence of gender on clinical manifestations of the disease in the United States. Material and methods. Data were obtained on biopsy-proven adult patients with celiac disease from a database of patients seen between 1981 and 2001 in a University-based referral center. Z scores were calculated to adjust for age,

Diane Bai; Pardeep Brar; Steve Holleran; Rajasekhar Ramakrishnan; Peter H. R. Green

2005-01-01

479

Lymphoma of the kidney: Primary or initial manifestation of rapidly progressive systemic disease?  

Microsoft Academic Search

Primary renal lymphoma is a controversial and rare disease. There is no agreement whether or not it is an initial manifestation\\u000a of a rapidly systemic disease. Most reported cases are questionable because of incomplete staging and lack of autopsy studies.\\u000a Herein, we present a 7l-year-old woman, initially diagnosed with primary non-Hodgkin lymphoma of the kidney, but was lost\\u000a 4 months

Ahmet Tefekli; Murat Baykal; Murat Binbay; Mustafa Barut; Ahmet Yaser Muslumanoglu

2006-01-01

480

a Manifestly Gauge Invariant and Universal Calculus for Su(n) Yang-Mills  

NASA Astrophysics Data System (ADS)

Within the framework of the Exact Renormalization Group, a manifestly gauge invariant calculus is constructed for SU(N) Yang-Mills. The methodology is comprehensively illustrated with a proof, to all orders in perturbation theory, that the ? function has no explicit dependence on either the seed action or details of the covariantization of the cutoff. The cancellation of these nonuniversal contributions is done in an entirely diagrammatic fashion.

Rosten, Oliver J.

481

Late Hadronization and Matter Formed at RHIC: Vector Manifestation, Brown-Rho Scaling and Hadronic Freedom  

Microsoft Academic Search

Recent developments in our description of RHIC and related heavy-ion\\u000aphenomena in terms of hidden local symmetry theories are reviewed with a focus\\u000aon the novel nearly massless states in the vicinity of -- both below and above\\u000a-- the chiral restoration temperature T_c. We present complementary and\\u000aintuitive ways to understand both Harada-Yamawaki's vector manifestation\\u000astructure and Brown-Rho scaling

Gerald E. Brown; Jeremy W. Holt; Chang-Hwan Lee; Mannque Rho

2006-01-01

482

Sensory manifestations in experimental and work-related chronic neck-shoulder pain  

Microsoft Academic Search

Little is known about the mechanisms leading to chronic neck-shoulder musculo-skeletal disorders. The aim of the present study was to investigate and compare the sensory manifestations of experimental or chronic neck-shoulder pain under controlled, low load, repetitive work.Experimental and clinical experiments were carried out. In Experiment 1, experimental muscle pain was induced in healthy subjects by intramuscular injection of hypertonic

P. Madeleine; B. Lundager; M. Voigt; L. Arendt-Nielsen

1998-01-01

483

Parallel manifestation of Crohn's disease and acute pericarditis: a report of two cases.  

PubMed

Pericarditis is an uncommon extraintestinal manifestation of inflammatory bowel disease (IBD), which may occur at any time during its natural course. Moreover, it may be associated with the medications used to treat IBD, especially mesalamine. We report on two patients with acute pericarditis who were subsequently diagnosed with Crohn's disease. It is likely that mild, longstanding, virtually asymptomatic intestinal disease preceded the onset of pericarditis in both cases. PMID:17541784

Stasinopoulou, Panagiota; Kaziani, Aikaterini; Mantzaris, Gerassimos; Roussos, Anastasios; Skoutelis, Athanasios

2007-09-01

484

Quality of life and satisfaction with life in SLE patients—the importance of clinical manifestations  

Microsoft Academic Search

To assess the correlation between quality of life (QoL) and satisfaction with life (SL) in SLE patients and correlate both\\u000a with clinical symptoms of the disease. The study was performed in 83 patients. QoL was assessed by Short Form 36, and SL was\\u000a assessed by the Satisfaction with Life Scale. Clinical manifestations presented at the time of examination were taken

Lilianna Kulczycka; Anna Sysa-J?drzejowska; Ewa Robak

2010-01-01

485

Manifestations of the North - South Asymmetry in the Photosphere and in the Green Line Corona  

NASA Astrophysics Data System (ADS)

The north - south asymmetries (NSA) of three solar activity indices are derived and mutually compared over a period of more than five solar cycles (1945 - 2001). A catalogue of the hemispheric sunspot numbers, the data set of the coronal green line brightness developed by us, and the magnetic flux derived from the NSO/KP data (1975 - 2001) are treated separately within the discrete low- and mid-latitude zones (5° - 30°, 35° - 60°). The calculated autocorrelations, cross-correlations, and regressions between the long-term NSA data sets reveal regularities in the solar activity phenomenon. Namely, the appearance of a distinct quasi-biennial oscillation (QBO) is evident in all selected activity indices. Nevertheless, a smooth behavior of QBO is derived only when sufficient temporal averaging is performed over solar cycles. The variation in the significance and periodicity of QBO allows us to conclude that the QBO is not persistent over the whole solar cycle. A similarity in the photospheric and coronal manifestations of the NSA implies that their mutual relation will also show the QBO. A roughly two-year periodicity is actually obtained, but again only after significant averaging over solar cycles. The derived cross-correlations are in fact variable in degree of correlation as well as in changing periodicity. A clear and significant temporal shift of 1 - 2 months in the coronal manifestation of the magnetic flux asymmetry relative to the photospheric manifestation is revealed as a main property of their mutual correlation. This shift can be explained by the delayed large-scale coronal manifestation in responding to the emergence of the magnetic flux in the photosphere. The reliability of the derived results was confirmed by numerical tests performed by selecting different numerical values of the used parameters.

Sýkora, J.; Rybák, J.

2010-02-01

486

Influence of inherited and acquired thrombophilic defects on the clinical manifestations of mixed cryoglobulinaemia  

Microsoft Academic Search

Objective. To investigate the contribution of inherited and acquired thrombophilic defects to the clinical manifestations of mixed cryoglobulinaemia vasculitis. Methods. The following thrombophilic defects were investigated in 64 consecutive patients with HCV-associated mixed cryoglobulinaemia: aPLs, lupus anti-coagulant, homocysteinaemia, protein C and protein S concentrations, activated protein C resistance, plasminogen activator inhibitor-1 4G4G and 5G5G genotypes, and the presence of mutations

M. Casato; M. Carlesimo; A. Francia; C. Timarco; A. Antenucci; M. Bove; H. Martini; M. Visentini; M. Fiorilli; L. Conti

2008-01-01

487

Molecular analysis and association with clinical and laboratory manifestations in children with sickle cell anemia  

PubMed Central

Objectives To analyze the frequency of ?S-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. Method The frequency of ?S-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). Results The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the ?3.7 deletion was 1.71% as homozygous (??3.7/??3.7) and 11.9% as heterozygous (??3.7/??). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of ?S-globin haplotypes and alpha-thalassemia with clinical manifestations. Conclusions In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. ?S-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations. PMID:25305165

Camilo-Araújo, Roberta Faria; Amancio, Olga Maria Silverio; Figueiredo, Maria Stella; Cabanãs-Pedro, Ana Carolina; Braga, Josefina Aparecida Pellegrini

2014-01-01