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Sample records for manifestations rhumatologiques revelatrices

  1. Rheumatic manifestations of scurvy.

    PubMed

    Ferrari, Claudia; Possemato, Niccolò; Pipitone, Nicolò; Manger, Bernhard; Salvarani, Carlo

    2015-04-01

    This paper reviews the rheumatological manifestations of scurvy, based on articles published in English from 1965 until October 2014, with a particular focus on rheumatological manifestations. Scurvy is a rare, uncommon disease in developed countries. Due to its clinical heterogeneity, the disease can easily mimic rheumatologic conditions leading to a delay in diagnosis and treatment.

  2. [Cutaneous manifestations of sarcoidosis].

    PubMed

    Amschler, K; Seitz, C S

    2017-03-17

    Skin manifestations of sarcoidosis occur in up to 30% of cases. This review summarizes and illustrates in detail the differences between specific and unspecific skin manifestations of sarcoidosis. Important differential diagnoses, such as tuberculosis, cutaneous lymphoma and syphilis have to be excluded. The indications for systemic treatment are primarily determined by the extent of organ involvement and also by the cosmetic impairment.

  3. Ophthalmic manifestations of neurofibromatosis.

    PubMed Central

    Huson, S; Jones, D; Beck, L

    1987-01-01

    The eyes of 64 patients known to have neurofibromatosis were examined. Lisch nodules were the commonest manifestation of the disease and were present in 95% of all patients (100% of those aged 16 years or older). Images PMID:3103673

  4. [Unusual Migraine Manifestations].

    PubMed

    Schipper, Sivan; Gantenbein, Andreas R; Sandor, Peter S

    2016-06-08

    Migraine is a complex neurologic disorder by which several systems of the central nervous system (autonomous system, affective, cognitive, sensoric and motoric system) may be affected on different levels. Around a fourth of the patients have migraine aura. The most common aura is the visual aura, followed by sensoric aura. But motoric deficits as well as deficits of higher cortical centers (disorders of thinking, orientation, coherence or concentration) may occur as well. In analogy with a headache calendar, an aura calendar can deliver important help in the diagnostic process of rare migraine manifestations and prevent underdiagnosis of unusual migraine manifestations. Complex migraine manifestations are diagnoses of exlusion, and a broad diagnostic work-up is warranted in order to exclude dangerous neurologic pathologies. There are no specific therapeutic recommendations, as there is a lack of randomized controlled studies.

  5. Manifestations of Lyme carditis.

    PubMed

    Kostić, Tomislav; Momčilović, Stefan; Perišić, Zoran D; Apostolović, Svetlana R; Cvetković, Jovana; Jovanović, Andriana; Barać, Aleksandra; Šalinger-Martinović, Sonja; Tasić-Otašević, Suzana

    2017-04-01

    The first data of Lyme carditis, a relatively rare manifestation of Lyme disease, were published in eighties of the last century. Clinical manifestations include syncope, light-headedness, fainting, shortness of breath, palpitations, and/or chest pain. Atrioventricular (AV) electrical block of varying severity presents the most common conduction disorder in Lyme carditis. Although is usually mild, AV block can fluctuates rapidly and progress from a prolonged P-R interval to a His-Purkinje block within minutes to hours and days. Rarely, Lyme disease may be the cause of endocarditis, while some studies and reports, based on serological and/or molecular investigations, have suggested possible influence of Borrelia burgdorferi on degenerative cardiac valvular disease. Myocarditis, pericarditis, pancarditis, dilated cardiomyopathy, and heart failure have also been described as possible manifestations of Lyme carditis. The clinical course of Lyme carditis is generally mild, short term, and in most cases, completely reversible after adequate antibiotic treatment.

  6. Cutaneous Manifestations of ESRD

    PubMed Central

    Cronin, Antonia J.; Leslie, Kieron S.

    2014-01-01

    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

  7. Destiny - Not So Manifest.

    ERIC Educational Resources Information Center

    Kerr, Clark

    Higher education in the United States is facing a period of uncertainty, confusion, conflict, and potential change, and it has little to guide it in its past experiences. For most of its three and one-third century history, it has had a manifest destiny and through the period from 1820-1870 was marked by rapid change and some student unrest. Two…

  8. Hazardous Waste Manifest System

    EPA Pesticide Factsheets

    EPA’s hazardous waste manifest system is designed to track hazardous waste from the time it leaves the generator facility where it was produced, until it reaches the off-site waste management facility that will store, treat, or dispose of the waste.

  9. Oral manifestations of hyperoxaluria.

    PubMed

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

    2011-11-01

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

  10. Inflammatory Manifestations of Lymphedema

    PubMed Central

    Ly, Catherine L.; Kataru, Raghu P.; Mehrara, Babak J.

    2017-01-01

    Lymphedema results from lymphatic insufficiency leading to a progressive inflammatory process that ultimately manifests as discomfort, recurrent infections, and, at times, secondary malignancy. Collectively, these morbidities contribute to an overall poor quality of life. Although there have been recent advances in microsurgical interventions, a conservative palliative approach remains the mainstay of treatment for this disabling disease. The absence of a cure is due to an incomplete understanding of the pathophysiological changes that result in lymphedema. A histological hallmark of lymphedema is inflammatory cell infiltration and recent studies with animal models and clinical biopsy specimens have suggested that this response plays a key role in the pathology of the disease. The purpose of this report is to provide an overview of the ongoing research in and the current understanding of the inflammatory manifestations of lymphedema. PMID:28106728

  11. Diverse manifestations of trichloroethylene.

    PubMed Central

    McCunney, R J

    1988-01-01

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

  12. [Respiratory manifestations in aspergillosis].

    PubMed

    Regimbaud, M

    1986-01-01

    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.

  13. Hazardous Waste Electronic Manifest System (E-Manifest)

    EPA Pesticide Factsheets

    This webpage provides information on EPA's work toward developing a hazardous waste electronic manifest system. Information on the Hazardous Waste Electronic Manifest Establishment Act, progress on the project and frequent questions are available.

  14. Ophthalmologic manifestations of celiac disease

    PubMed Central

    Martins, Thiago Gonçalves dos Santos; Costa, Ana Luiza Fontes de Azevedo; Oyamada, Maria Kiyoko; Schor, Paulo; Sipahi, Aytan Miranda

    2016-01-01

    Celiac disease is an autoimmune disorder that affects the small intestine of genetically predisposed individuals. Ophthalmic manifestations are within the extra-intestinal manifestations, and can be divided into those of autoimmune disorders or those due to absorptive disabilities. This article reviewed the ophthalmologic manifestation of celiac disease. Ophthalmic symptoms are rare, but should be investigated in patients with celiac disease and taken into consideration as the first systemic manifestation. PMID:26949627

  15. [Ocular Manifestations in Sarcoidosis].

    PubMed

    Walscheid, K; Tappeiner, C; Heiligenhaus, A

    2016-05-01

    Sarcoidosis is an inflammatory multi-organ disease of unknown pathogenesis, characterised by non-necrotising granulomata. Sarcoidosis predominantly manifests in the lung, but any other organ may be affected. Ocular involvement is present in about 25 to 50 % of patients. The most common ocular manifestation is uveitis, especially of the anterior eye segment. If ocular sarcoidosis is suspected, interdisciplinary assessment of the patient is mandatory, including laboratory tests, chest X-ray, assessment by a specialist in internal medicine and, ideally, histological evidence of granuloma formation in a tissue specimen. Other (infectious) causes of granulomatous inflammation need to be excluded, especially tuberculosis or syphilis. For the ophthalmological assessment, detection of granulomatous lesions is of particular importance, especially by visualising chorioretinal granuloma by fluorescein and indocyanin green angiography. Cystoid macular oedema and glaucoma are the most frequent complications limiting visual acuity. Corticosteroids, which can be administered either locally or systemically, are the mainstay of therapy. Depending on the clinical course and the development of ocular complications, systemic steroid-sparing immunosuppressive medication may be indicated.

  16. Genitourinary manifestations of tuberculosis.

    PubMed

    Wise, Gilbert J; Marella, Venkata K

    2003-02-01

    By the 1980s, the availability of antituberculosis chemotherapy reduced the incidence and prevalence of tuberculosis. Changing patterns of population emigration and the development of large pools of immune-compromised individuals reversed the downward trend of tuberculosis. The incidence of genitourinary tuberculosis has remained constant. The manifestations of GU TB can be variable and cause a variety of clinical patterns that mimic other diseases. Adrenal insufficiency, renal disease, obstructive uropathy, and chronic cystitis are not uncommon with TB. The patient with TB may have genital disease that simulates STD or scrotal tumors. Infertility can be caused by GU tuberculosis. Awareness of environmental factors and patient history should alert the urologist to the wide array of clinical findings in the genitourinary system that can be caused by tuberculosis.

  17. Ophthalmic manifestations postlightning strike

    PubMed Central

    Dhillon, Permesh Singh; Gupta, Mohit

    2015-01-01

    Various ophthalmic complications affecting the anterior and posterior segments have been identified due to lightning strike. We report the first case of an indirect lightning-induced full thickness macular hole formation in the UK as evidenced by slit lamp examination and optical coherence tomography (OCT) scan in a 77-year-old woman presenting with sudden visual loss in her right eye and thermal skin injury affecting her scalp. Her best corrected visual acuities were LogMAR 0.46 and 0.12 in the right and left eyes, respectively. There were no other ocular manifestations observed in either eye. She was initially managed conservatively with non-steroidal anti-inflammatory drug eye drops but surgery was later advised due to minimal changes in the visual acuity and macular hole on follow-up. OCT scanning is important in diagnosing macular holes, which usually warrant surgical intervention. PMID:25827914

  18. Manifold manifestations of ergotism.

    PubMed

    Payne, B; Sasse, B; Franzen, D; Hailemariam, S; Gemsenjäger, E

    2000-08-19

    Vasospastic side effects leading to organic manifestations are rare in ergotamine therapy. To our knowledge, combinations of more than two signs of ergotism have rarely been described in the literature so far. We present a 65-year-old male patient who as a consequence of severe migraine had developed ergotamine abuse. He was admitted to our hospital after one week of increasing abdominal pain. During laparotomy, necrotic areas of the small intestine and the sigmoid colon were resected, which on histopathologic examination revealed severe hypertrophy of the smooth musculature of mesenteric arteries, resulting from chronic vasospasms. Postoperatively, the patient developed ischaemia of the limbs which was confirmed by angiography. Before death, the patient also showed ischaemic signs in the acrae and necrosis of the tongue.

  19. Cardiovascular manifestations of autonomic epilepsy.

    PubMed

    Freeman, Roy

    2006-02-01

    Cardiovascular autonomic manifestations of seizures occur frequently in the epileptic population. Common manifestations include alterations in heart rate and rhythm, blood pressure, ECG changes and chest pain. The neuroanatomical and neurophysiological underpinnings of these autonomic manifestations are not been fully elucidated. Diagnostic confusion may arise when ictal symptoms are confined to the autonomic nervous system; conversely, such symptoms in association with convulsions or altered consciousness are more readily recognized as concomitant ictal features. Awareness of the diverse autonomic manifestations of epilepsy will enhance diagnosis and lead to more effective therapy of these patients.

  20. Oral Manifestations of Vitiligo

    PubMed Central

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

    2015-01-01

    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

  1. Hyaline fibromatosis syndrome: cutaneous manifestations*

    PubMed Central

    Marques, Silvio Alencar; Stolf, Hamilton Ometto; Polizel, Juliana Ocanha; Munhoz, Tânia; Brandão, Marcela Calixto; Marques, Mariangela Esther Alencar

    2016-01-01

    Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”. The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary approach. PMID:27192526

  2. Cutaneous Manifestations of Crohn Disease.

    PubMed

    Hagen, Joshua W; Swoger, Jason M; Grandinetti, Lisa M

    2015-07-01

    Awareness of the extraintestinal manifestations of Crohn disease is increasing in dermatology and gastroenterology, with enhanced identification of entities that range from granulomatous diseases recapitulating the underlying inflammatory bowel disease to reactive conditions and associated dermatoses. In this review, the underlying etiopathology of Crohn disease is discussed, and how this mirrors certain skin manifestations that present in a subset of patients is explored. The array of extraintestinal manifestations that do not share a similar pathology, but which are often seen in association with inflammatory bowel disease, is also discussed. Treatment and pathogenetic mechanisms, where available, are discussed.

  3. Ocular manifestations of feline herpesvirus.

    PubMed

    Andrew, S E

    2001-03-01

    Feline herpesvirus-1 (FHV-1) infection is ubiquitous in the domestic cat population worldwide. The most common clinical ocular manifestations of infection with FHV-1 are conjunctivitis and keratitis. This paper reviews the pathogenesis of feline herpesvirus-1 and discusses the various clinical ocular manifestations, diagnostic techniques and treatment of FHV-1-induced diseases. Ocular manifestations include: conjunctivitis, keratitis, stromal keratitis, keratoconjunctivitis sicca, ophthalmia neonatorium, symblepharon, corneal sequestrum, eosinophilic keratitis and anterior uveitis. Diagnostic techniques discussed include: virus isolation, fluorescent antibody testing, serum neutralising titers, ELISA and polymerase chain reaction. Various therapies are also discussed.

  4. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  5. [Oral manifestations in systemic diseases].

    PubMed

    Pedersen, Anne Marie Lynge; Jensen, Siri Beier

    2010-11-01

    Systemic diseases may affect the oral tissues, i.e. oral mucosa, salivary glands, teeth or bone, and oral manifestations will frequently present early, i.e. in association with (non-fulminant) systemic disease. Thus, recognition and proper diagnosis is essential to initiate appropriate treatment schedules. Key examples of systemic disease groups with oral manifestations include dermatological, inflammatory connective tissue diseases, haematological and inflammatory gastrointestinal diseases, as well as neurological and endocrine diseases.

  6. Cutaneous manifestations of child abuse.

    PubMed

    Kos, Liborka; Shwayder, Tor

    2006-01-01

    Dermatologists and child abuse are not frequently associated in the minds of most physicians. Yet the most common manifestations of child abuse are cutaneous. This article reviews cutaneous manifestations of physical abuse, including bruises, lacerations, abrasions, human bites, and burns. It also discusses ways that dermatologists can differentiate abusive injuries from accidental ones as well as from the many dermatologic conditions that can mimic child abuse. Finally, we review what actions the dermatologist should take when suspecting abuse in a patient.

  7. Cutaneous manifestations of human toxocariasis.

    PubMed

    Gavignet, Béatrice; Piarroux, Renaud; Aubin, François; Millon, Laurence; Humbert, Philippe

    2008-12-01

    Human toxocariasis is a parasitic disease characterized by the presence of larvae of the genus Toxocara in human tissues. T canis and T cati, the adult roundworms of which are found in dog and cat intestines, respectively, are the most common causative agents of the disease. Toxocaral larvae usually cause two severe syndromes: visceral larva migrans and ocular larva migrans, depending on the location of the larvae. Two other syndromes, covert toxocariasis and common toxocariasis, which are less typical and not as severe, have also been described. During the last two decades, cutaneous manifestations such as chronic urticaria, chronic pruritus, and miscellaneous eczema, in patients with Toxocara antibodies, have been studied by different authors. In some cases, these cutaneous manifestations are the only signs indicating the presence of the disease, and they are cured after antihelmintic treatment when there is good patient compliance. In this review, we focus on these particular skin manifestations regarding their clinical description, diagnosis, and treatment.

  8. Neurocutaneous Manifestations of Genetic Mosaicism

    PubMed Central

    van Steensel, Maurice A. M.

    2015-01-01

    Genetic mosaicism is defined as the presence of two or more genetically distinct cell populations in a single individual. Ever more disorders are found to be manifestations of mosaicism and together constitute a significant proportion of the morbidity confronting pediatric specialists. An emerging category is that of overgrowth syndromes with skin manifestations and neurological or developmental abnormalities, such as the well-known Proteus syndrome. In recent years, we have seen dramatic advances in our understanding of these disorders and we now know the genetic basis of many of them. This has profound consequences for diagnosis, counselling, and even treatment, with therapies targeted to specific pathways becoming available for clinical use. Recognizing such overgrowth syndromes, therefore, is more important than ever. Fortunately, their skin manifestations can provide important diagnostic clues when evaluated in the entire phenotypic context. In this review, I provide an overview of the most frequently seen mosaic neurocutaneous phenotypes and discuss their molecular basis. PMID:27617125

  9. [Cardiovascular manifestations of human toxocariasis].

    PubMed

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

    2013-01-01

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

  10. Atypical manifestations of tinea corporis.

    PubMed

    Ziemer, Mirjana; Seyfarth, Florian; Elsner, Peter; Hipler, Uta-Christina

    2007-01-01

    Tinea corporis classically presents as an erythematous annular plaque with a scaly, centrifugally advancing border. However, sometimes vesicles and pustules are observed. Occasionally, even frank bullae appear secondary to severe inflammation. Diagnostic difficulties arise when atypical manifestations mimic other inflammatory skin diseases, including atopic or seborrheic dermatitis, subacute cutaneous lupus erythematosus, or vesicular diseases. We report five cases of atypical tinea corporis, where the initial clinical diagnosis was different from dermatophytosis. The differential diagnoses and the diagnostic difficulties related to atypical manifestations of fungal infections are discussed. Moreover, our cases emphasise the importance of conventional histological examination, which enables a fast, correct diagnosis.

  11. Cutaneous manifestations in celiac disease

    PubMed Central

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

    2006-01-01

    Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of intestinal villi that improves after gluten-free diet (GFD). CD is often associated with extra-intestinal manifestations; among them, several skin diseases are described in CD patients. The present review reports all CD-associated skin manifestations described in the literature and tries to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed. PMID:16521210

  12. Skin manifestations of child abuse.

    PubMed

    Ermertcan, Aylin Turel; Ertan, Pelin

    2010-01-01

    Child abuse is a major public health problem all over the world. There are four major types of abuse: physical abuse, sexual abuse, emotional abuse and neglect. The most common manifestations of child abuse are cutaneous and their recognition; and differential diagnosis is of great importance. Clinicians, especially dermatologists, should be alert about the skin lesions of child abuse. In the diagnosis and management of child abuse, a multidisciplinary approach with ethical and legal procedures is necessary. In this manuscript, cutaneous manifestations of physical, sexual, emotional abuse and neglect are reviewed and discussed.

  13. Hematological manifestations of nephropathic cystinosis.

    PubMed

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

    2008-01-01

    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 available treatment options for patients with bone marrow failure secondary to cystine accumulation. The availability of effective oral therapy and the limited activity of hematopoietic growth factors in these patients highlight the importance of bone marrow examination early in the evaluation of cystinosis patients with abnormal blood counts.

  14. Parasitic Diseases With Cutaneous Manifestations.

    PubMed

    Ash, Mark M; Phillips, Charles M

    2016-01-01

    Parasitic diseases result in a significant global health burden. While often thought to be isolated to returning travelers, parasitic diseases can also be acquired locally in the United States. Therefore, clinicians must be aware of the cutaneous manifestations of parasitic diseases to allow for prompt recognition, effective management, and subsequent mitigation of complications. This commentary also reviews pharmacologic treatment options for several common diseases.

  15. Mucocutaneous manifestations of Cowden's syndrome

    PubMed Central

    Reddy, Kundoor Vinay Kumar; Anusha, Amarthuluri; Maloth, Kotya Naik; Sunitha, Kesidi; Thakur, Moni

    2016-01-01

    Cowden's syndrome is an autosomal dominant genodermatosis with variable orofacial and systemic manifestations. Here we present one such classical case of Cowden's syndrome in a 45-year-old female patient with features such as multiple cutaneous papillomatosis, oral fibromas, and fibromas involving multiple organs such as gastrointestinal tract (multiple polyps), thyroid disorders, and breast cancer. PMID:27990388

  16. ENT manifestations of Wegeners granulomatosis.

    PubMed

    Sharma, Arpit; Deshmukh, Shraddha; Dabholkar, Jyoti

    2013-01-01

    Wegeners granulomatosis is a necrotizing granulomatous vasculitis with multisystemic involvement. We present two cases of Wegener's presenting with otological manifestations as the first symptom. These symptoms are subtle and diagnosis may be easily overlooked. Hence a high index of suspicion is required. Early diagnosis and treatment goes a long way in improving the outcomes and in preventing further complications.

  17. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... delivery of the waste to the first transporter designated on the manifest prepared under paragraph (e) or... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.215 Manifest discrepancies. (a) Manifest... quantity or type of PCB waste designated on the manifest or shipping paper, and the quantity and type...

  18. Cardiopulmonary Manifestations of Ankylosing Spondylitis

    PubMed Central

    Momeni, Mahnaz; Taylor, Nora; Tehrani, Mahsa

    2011-01-01

    Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications. PMID:21547038

  19. Assessment of Parkinson Disease Manifestations

    PubMed Central

    Perlmutter, Joel S.

    2010-01-01

    Parkinson disease (PD) is a progressive neurologic condition that causes motor and non-motor manifestations. Treatment provides symptomatic benefit but no current treatment has been proven to slow disease progression. Research studies of PD require a means of rating the severity of disease by measurement of motor manifestations, assessment of ability to perform daily functional activities, and symptomatic response to medication. The most common rating scales are the Unified Parkinson Disease Rating Scale (UPDRS), Hoehn and Yahr staging, and the Schwab and England rating of activities of daily living. Each of these rating scales are described, including detailed instructions on how to implement these ratings. Although these are the most widely applied rating scales of PD, there are still substantial limitations to these scales that must be considered when using them for research. Finally, some common applications of these scales are described. PMID:19802812

  20. Porphyria and its neurologic manifestations.

    PubMed

    Tracy, Jennifer A; Dyck, P James B

    2014-01-01

    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.

  1. Orofacial manifestations of systemic sclerosis.

    PubMed

    Veale, B J; Jablonski, R Y; Frech, T M; Pauling, J D

    2016-09-23

    Systemic sclerosis (SSc) is a multisystem disease of unknown aetiology characterised by microangiopathy, dysregulated immune function and tissue remodelling, which commonly involves the oral cavity. Orofacial manifestations of SSc contribute greatly to overall disease burden and yet are regularly overlooked and under-treated. This may reflect a pre-occupation amongst rheumatology clinicians on potentially life-threatening internal organ involvement, but is also a consequence of insufficient engagement between rheumatologists and dental professionals. A high proportion of SSc patients report difficulty accessing a dentist with knowledge of the disease and there is recognition amongst dentists that this could impact negatively on patient care. This review shall describe the clinical features and burden of orofacial manifestations of SSc and the management of such problems. The case is made for greater collaborative working between rheumatologists and dental professionals with an interest in SSc in both the research and clinical setting.

  2. Clinical manifestations of synovial cysts.

    PubMed

    Burt, T B; MacCarter, D K; Gelman, M I; Samuelson, C O

    1980-08-01

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

  3. Rheumatic manifestations of diabetes mellitus.

    PubMed

    Lebiedz-Odrobina, Dorota; Kay, Jonathan

    2010-11-01

    DM is associated with various musculoskeletal manifestations. The strength of this relationship varies among the various musculoskeletal disorders; the associations are based mostly on epidemiologic data. For most of these conditions, definitive pathophysiologic correlates are lacking.Hand and shoulder disorders occur more frequently than other musculoskeletal manifestations of DM. Recognition of the association between DM and shoulder adhesive capsulitis, DD, and stenosing flexor tenosynovitis facilitates their correct diagnosis in the setting of DM and prompt initiation of appropriate treatment, which may include optimizing glycemic control. Conversely, awareness and identification of the characteristic musculoskeletal manifestations of DM may facilitate earlier diagnosis of DM and initiation of glucose-lowering therapy to retard the development of diabetic complications.Much less has been published about the musculoskeletal complications of DM than about its micro- and macrovascular complications. Prospective case-control cohort studies are needed to establish the true prevalence of musculoskeletal complications of DM and the metabolic syndrome, especially in this era of tighter glycemic control.The potential relationship between DM and the development of OA needs to be clarified in large, prospective, case-control cohort studies. The effect on musculoskeletal manifestations of various therapeutic regimens to manage DM should be studied prospectively. Treatment regimens for some musculoskeletal conditions associated with DM, such as DISH, should be studied in larger prospective, randomized,controlled clinical trials.At the molecular level, further studies are warranted to clarify the potential contribution of AGEs and adipokines to the development of OA and diabetic musculoskeletal syndromes, such as shoulder adhesive capsulitis, DD, stenosing flexor tenosynovitis, and LJM. Identification of such molecular targets for therapy would promote the development of

  4. Articular Manifestations of Systemic Diseases

    PubMed Central

    Bensen, W. G.

    1983-01-01

    Many systemic diseases present with articular manifestations. An understanding of the clinical, laboratory and radiological features of these diseases can lead to early diagnosis and appropriate therapy. This article describes the articular presentation and management of four generalized disorders: idiopathic hemachromatosis; sarcoidosis; hepatitis-B virus-induced arthritis, and polymyositis-dermatomyositis induced arthritis. ImagesFig. 2Fig. 3Fig. 4 PMID:21283470

  5. Systemic diseases with cutaneous manifestations.

    PubMed

    Merchant, S R; Taboada, J

    1995-07-01

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

  6. [Pulmonary manifestations of antisynthetase syndrome].

    PubMed

    Jouneau, S; Hervier, B; Jutant, E-M; Decaux, O; Kambouchner, M; Humbert, M; Delaval, P; Montani, D

    2015-06-01

    Antisynthetase syndrome is an inflammatory myopathy frequently associated with pulmonary manifestations, especially interstitial lung diseases, and uncommonly pulmonary hypertension. In the context of a suggestive clinical and radiological picture, positive anti-RNA synthetase antibodies confirm the diagnosis. Anti-Jo1, anti-PL7, and anti-PL12 antibodies are the more commonly encountered. The presence of a number of extra-thoracic manifestations in association with pulmonary disease may suggest the diagnosis. These include: myalgia or muscular deficit, Raynaud's phenomenon, polyarthritis, fever, mechanics hands. Serum creatine kinase levels are usually increased. Electromyogram, muscular magnetic resonance imaging or muscle pathology are not mandatory to make the diagnosis. There is a high variability in symptoms and severity, between patients but also during the course of the disease in the same patient. The presence of an interstitial lung disease is a major prognostic factor and an indication for more intensive treatment, principally with systemic corticosteroids with or without immunosuppressive drugs. Improving respiratory physicians' knowledge of this disease, which is often revealed by its pulmonary manifestations, should help diagnosis, therapeutic management, and possibly prognosis.

  7. Clinical manifestations of sleep apnea

    PubMed Central

    2015-01-01

    Obstructive sleep apnea (OSA) may manifest in a number of ways from subtle intrusion into daily life to profound sleepiness, snoring, witnessed apneas and other classic symptoms. Although there is increasing evidence suggesting OSA can adversely affect health in a variety of ways, this disorder remains underdiagnosed. The most well-escribed health consequences of OSA relate to the cardiovascular system. Hypertension and arrhythmias have a strong association with OSA, and evidence suggests that treatment of OSA in patients with refractory hypertension and in patients planning cardioversion for atrial fibrillation may be of particularly importance. Significant associations between heart failure and OSA as well as complex sleep apnea have also been well-described. Cerebrovascular insult, impaired neurocognition, and poorly controlled mood disorder are also associated with in OSA. Therapy for OSA may ameliorate atherosclerotic progression and improve outcomes post-cerebrovascular accident (CVA). OSA should be considered in patients complaining of poor concentration at work, actual or near-miss motor vehicle accidents, and patients with severe sleepiness as a component of their co-morbid mood disorders. The metabolic impact of OSA has also been studied, particularly in relation to glucose homeostasis. Also of interest is the potential impact OSA has on lipid metabolism. The adverse effect untreated OSA has on glucose tolerance and lipid levels has led to the suggestion that OSA is yet another constituent of the metabolic syndrome. Some of these metabolic derangements may be related to the adverse effects untreated OSA has on hepatic health. The cardiovascular, neurocognitive, and metabolic manifestations of OSA can have a significant impact on patient health and quality of life. In many instances, evidence exists that therapy not only improves outcomes in general, but also modifies the severity of co-morbid disease. To mitigate the long-term sequela of this disease

  8. Clinical manifestations of sleep apnea.

    PubMed

    Stansbury, Robert C; Strollo, Patrick J

    2015-09-01

    Obstructive sleep apnea (OSA) may manifest in a number of ways from subtle intrusion into daily life to profound sleepiness, snoring, witnessed apneas and other classic symptoms. Although there is increasing evidence suggesting OSA can adversely affect health in a variety of ways, this disorder remains underdiagnosed. The most well-escribed health consequences of OSA relate to the cardiovascular system. Hypertension and arrhythmias have a strong association with OSA, and evidence suggests that treatment of OSA in patients with refractory hypertension and in patients planning cardioversion for atrial fibrillation may be of particularly importance. Significant associations between heart failure and OSA as well as complex sleep apnea have also been well-described. Cerebrovascular insult, impaired neurocognition, and poorly controlled mood disorder are also associated with in OSA. Therapy for OSA may ameliorate atherosclerotic progression and improve outcomes post-cerebrovascular accident (CVA). OSA should be considered in patients complaining of poor concentration at work, actual or near-miss motor vehicle accidents, and patients with severe sleepiness as a component of their co-morbid mood disorders. The metabolic impact of OSA has also been studied, particularly in relation to glucose homeostasis. Also of interest is the potential impact OSA has on lipid metabolism. The adverse effect untreated OSA has on glucose tolerance and lipid levels has led to the suggestion that OSA is yet another constituent of the metabolic syndrome. Some of these metabolic derangements may be related to the adverse effects untreated OSA has on hepatic health. The cardiovascular, neurocognitive, and metabolic manifestations of OSA can have a significant impact on patient health and quality of life. In many instances, evidence exists that therapy not only improves outcomes in general, but also modifies the severity of co-morbid disease. To mitigate the long-term sequela of this disease

  9. Clinical manifestation of mitochondrial diseases.

    PubMed

    Magner, Martin; Kolářová, Hana; Honzik, Tomáš; Švandová, Ivana; Zeman, Jiří

    2015-01-01

    Mitochondrial disorders (MD) represent a clinically, biochemically and genetically heterogeneous group of diseases associated with dysfunction of the oxidative phosphorylation system and pyruvate dehydrogenase complex. Our aim was to illustrate the most common clinical presentation of MD on the example of selected diseases and syndromes. The minimal prevalence of MD is estimated as 1 to 5,000. MD may manifest at any age since birth until late-adulthood with acute manifestation or as a chronic progressive disease. Virtually any organ may be impaired, but the organs with the highest energetic demands are most frequently involved, including brain, muscle, heart and liver. Some MD may manifest as a characteristic cluster of clinical features (e.g. MELAS syndrome, Kearns-Sayre syndrome). Diagnostics includes detailed history, the comprehensive clinical examination, results of specialized examinations (especially cardiology, visual fundus examination, brain imaging, EMG), laboratory testing of body fluids (lactate, aminoacids, organic acids), and analysis of bioptic samples of muscle, skin, and liver, eventually. Normal lactate level in blood does not exclude the possibility of MD. Although the aimed molecular genetic analyses may be indicated in some of mitochondrial diseases, the methods of next generation sequencing come into focus. Examples of treatment are arginine supplementation in MELAS syndrome, ketogenic diet in pyruvate oxidation disorders or quinone analogs in patients with LHON. Conclusion: The clinical suspicion of a mitochondrial disorder is often delayed, or the disease remains undiagnosed. The correct diagnosis and adequate treatment can improve prognosis of the patient. Access to genetic counseling is also of great importance.

  10. Rheumatologic manifestations of diabetes mellitus.

    PubMed

    Crispin, José C; Alcocer-Varela, Jorge

    2003-06-15

    Diabetes mellitus has been linked to disorders of bones and joints, including neuroarthropathy, limited joint mobility, and hyperostosis. Some of the relations have known pathogenic mechanisms, but most are based on epidemiologic findings. This article reviews the associations between diabetes mellitus and its putative rheumatologic manifestations, and proposes a classification composed of four categories: consequences of diabetic complications, consequences of metabolic derangements inherent to diabetes, syndromes that may share etiologic mechanisms with microvascular disease, and probable associations. This approach may facilitate a clearer understanding of the musculoskeletal conditions that are prevalent in patients with diabetes mellitus.

  11. Cutaneous manifestations of chikungunya fever.

    PubMed

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

    2012-01-01

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

  12. Atypical Cutaneous Manifestations in Syphilis.

    PubMed

    Ivars Lleó, M; Clavo Escribano, P; Menéndez Prieto, B

    2016-05-01

    Although the diversity of the clinical manifestations of syphilis is well-known, atypical presentations can also occur. Such atypical presentations are associated with a high risk of transmission as a result of diagnostic confusion and treatment delays owing to the disease's ability to mimic other common skin diseases, deviate from classic clinical presentations, and adopt unique forms. Cases of atypical syphilis have been described most frequently in patients with concomitant human immunodeficiency virus (HIV) infection. Because the incidence of syphilis has been growing over recent years -particularly in patients with HIV co-infection- dermatologists need to be familiar with the less well-known clinical presentations of this venereal disease.

  13. Cutaneous manifestation of gastrointestinal disease

    PubMed Central

    Kerstetter, Justin

    2016-01-01

    The gastrointestinal (GI) and cutaneous systems are closely linked in origin. Skin manifestations are frequently seen as a part of different GI syndromes. Gastroenterologists play an important role in recognizing the symptoms, patient workup and arriving at appropriate diagnoses, often in consultation with dermatologists. This review discusses the diseases with both cutaneous and intestinal involvement. Hereditary polyposis GI cancers, hereditary nonpolyposis colorectal cancers (CRCs), hamartomatous disorders, and inflammatory bowel disease (IBD) are reviewed with emphasis on the genetic basis, diagnostic, histologic findings, screening modalities, and therapeutic options. PMID:27034812

  14. Cutaneous manifestations of breast cancer.

    PubMed

    Tan, Antoinette R

    2016-06-01

    Breast cancer may present with cutaneous symptoms. The skin manifestations of breast cancer are varied. Some of the more common clinical presentations of metastatic cutaneous lesions from breast cancer will be described. Paraneoplastic cutaneous dermatoses have been reported as markers of breast malignancy and include erythema gyratum repens, acquired ichthyosis, dermatomyositis, multicentric reticulohistiocytosis, and hypertrichosis lanuginosa acquisita. Mammary Paget's disease, often associated with an underlying breast cancer, and Cowden syndrome, which has an increased risk of breast malignancy, each have specific dermatologic findings. Recognition of these distinct cutaneous signs is important in the investigation of either newly diagnosed or recurrent breast cancer.

  15. [Respiratory manifestations of Marfan's syndrome].

    PubMed

    Neuville, M; Jondeau, G; Crestani, B; Taillé, C

    2015-02-01

    Marfan's syndrome is a rare genetic disorder caused by a mutation of the gene FBN1, coding for the protein fibrillin-1. Cardiovascular, musculoskeletal and ophthalmic manifestations are the most commonly observed, but minor diagnostic criteria also include pulmonary manifestations. Pneumothorax, frequently relapsing, affects 5 to 11% of patients. Rib cage abnormalities (pectus excavatum or pectus carinatum) and apical blebs may contribute to their occurrence. Treatment does not require any specific procedure but there is an increased risk of recurrence. Pectus excavatum affects up to 60% of the patients, without any functional impairment in most cases. Surgery may be required (using the Nuss procedure) in case of cardiovascular or psychological symptoms. Marfan's syndrome is frequently associated with obstructive sleep apnoea, which may itself contribute to aortic dilatation. Some studies suggest a potential role of craniofacial abnormalities in the pathogenesis of sleep apnea in these patients. Pulmonologists should consider Marfan's syndrome when treating patients for recurrent spontaneous pneumothorax or rib cage abnormalities, since early detection of cardiac abnormalities improves the prognosis significantly.

  16. Coronal manifestations of preflare activity

    NASA Technical Reports Server (NTRS)

    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

    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).

  17. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  18. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Section 761.215 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.215 Manifest discrepancies. (a) Manifest... quantity or type of PCB waste designated on the manifest or shipping paper, and the quantity and type...

  19. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  20. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  1. Extraintestinal Manifestations of Inflammatory Bowel Disease

    PubMed Central

    Burakoff, Robert

    2011-01-01

    Extraintestinal manifestations of inflammatory bowel disease are prevalent in both ulcerative colitis and Crohn's disease. The most common manifestations involve the musculoskeletal and dermatologic systems. Other manifestations involve the hepatopan-creatobiliary system (eg, primary sclerosing cholangitis) as well as the ocular, renal, and pulmonary systems. A multidisciplinary team approach is often needed for effective management, and emergency situations require prompt evaluation. PMID:21857821

  2. 14 CFR 121.665 - Load manifest.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 3 2011-01-01 2011-01-01 false Load manifest. 121.665 Section 121.665..., FLAG, AND SUPPLEMENTAL OPERATIONS Dispatching and Flight Release Rules § 121.665 Load manifest. Each certificate holder is responsible for the preparation and accuracy of a load manifest form before each...

  3. 14 CFR 121.665 - Load manifest.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 3 2013-01-01 2013-01-01 false Load manifest. 121.665 Section 121.665..., FLAG, AND SUPPLEMENTAL OPERATIONS Dispatching and Flight Release Rules § 121.665 Load manifest. Each certificate holder is responsible for the preparation and accuracy of a load manifest form before each...

  4. 14 CFR 121.665 - Load manifest.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 3 2012-01-01 2012-01-01 false Load manifest. 121.665 Section 121.665..., FLAG, AND SUPPLEMENTAL OPERATIONS Dispatching and Flight Release Rules § 121.665 Load manifest. Each certificate holder is responsible for the preparation and accuracy of a load manifest form before each...

  5. 14 CFR 121.665 - Load manifest.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 3 2014-01-01 2014-01-01 false Load manifest. 121.665 Section 121.665..., FLAG, AND SUPPLEMENTAL OPERATIONS Dispatching and Flight Release Rules § 121.665 Load manifest. Each certificate holder is responsible for the preparation and accuracy of a load manifest form before each...

  6. 14 CFR 121.665 - Load manifest.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Load manifest. 121.665 Section 121.665..., FLAG, AND SUPPLEMENTAL OPERATIONS Dispatching and Flight Release Rules § 121.665 Load manifest. Each certificate holder is responsible for the preparation and accuracy of a load manifest form before each...

  7. MUCOCUTANEOUS MANIFESTATIONS OF CHIKUNGUNYA FEVER

    PubMed Central

    Bandyopadhyay, Debabrata; Ghosh, Sudip Kumar

    2010-01-01

    Chikungunya fever (CF) is an arboviral acute febrile illness transmitted by the bite of infected Aedes mosquitoes. After a quiescence of more than three decades, CF has recently re-emerged as a major public health problem of global scale. CF is characterized by an acute onset of high fever associated with a severe disabling arthritis often accompanied by prominent mucocutaneous manifestations. The disease is usually self-limiting, but the joint symptoms and some of the cutaneous features may persist after the defervescence. A wide range of mucocutaneous changes has been described to occur in association with CF during the current epidemic. Besides a morbilliform erythema, hyperpigmentation, xerosis, excoriated papules, aphthous-like ulcers, vesiculobullous and lichenoid eruptions, and exacerbation of pre-existing or quiescent dermatoses had been observed frequently. These unusual features may help in the clinical differential diagnosis of acute viral exanthems mimicking CF. PMID:20418982

  8. Manifestations of Systemic Lupus Erythematosus

    PubMed Central

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

    2011-01-01

    ABSTRACT Systemic lupus erythematosus (SLE) is a chronic, multifaceted autoimmune inflammatory disease that can affect any part of the body. SLE is a disease of unknown aetiology with a variety of presenting features and manifestations. Interest in the disease has been stimulated in recent years, and improved methods of diagnosis have resulted in a significant increase in the number of cases recognized. It is apparent that it can no longer be regarded as a rare disease. The majority of the pathology in SLE is related to deposits of immune complexes in various organs, which triggers complement and other mediators of inflammation. Symptoms vary from person to person, and may come and go, depend on what part of the body is affected, can be mild, moderate, or severe. Diagnosis can be difficult because lupus mimics many other diseases; it requires clinical and serologic criteria. PMID:22879850

  9. Cardiac manifestations in Behcet's disease

    PubMed Central

    Demirelli, Selami; Degirmenci, Husnu; Inci, Sinan; Arisoy, Arif

    2015-01-01

    Summary Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD. PMID:25984424

  10. [Protracted rheumatologic manifestations in travelers].

    PubMed

    Levy, Tali; Kivity, Shaye; Schwartz, Eli

    2010-09-01

    Protracted rheumatological manifestations especially arthralgia and or polyarthritis may occur as a consequence of a wide range of pathogens including viral, bacterial and parasites. Few pathogenetic mechanisms leading to these clinical presentations have been suggested including a direct invasion of the synovial cells by the pathogens, immune complex formation, and others. The natural history of infectious arthritis/arthralgia is altogether benign, with full recovery and without sequelae, albeit sometimes very long. Diagnosis of infections-related arthralgia/arthritis is important since these diseases have a better prognosis, and can relieve anxiety among patients who are afraid of developing a chronic rheumatic disease. Since many patients will seek medical advice with these chronic complaints a long time after travel, physicians should be aware of the possible association between these complaints and remote travel. Thus, travel history should be mandatory, even in a rheumatologic setting.

  11. Genital manifestations of tropical diseases

    PubMed Central

    Richens, J

    2004-01-01

    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

  12. Oral manifestations in transplant patients

    PubMed Central

    Nappalli, Deepika; Lingappa, Ashok

    2015-01-01

    Organ transplantation is a widely undertaken procedure and has become an important alternative for the treatment of different end-stage organ diseases that previously had a poor prognosis. The field of organ transplant and hematopoietic stem cell transplant is developing rapidly. The increase in the number of transplant recipients also has an impact on oral and dental services. Most of the oral problems develop as a direct consequence of drug-induced immunosuppression or the procedure itself. These patients may present with oral complaints due to infections or mucosal lesions. Such lesions should be identified, diagnosed, and treated. New treatment strategies permit continuous adaptation of oral care regimens to the changing scope of oral complications. The aim of this review is to analyze those oral manifestations and to discuss the related literature. PMID:26005458

  13. Psoriasis: pathophysiology and oral manifestations.

    PubMed

    Zhu, J F; Kaminski, M J; Pulitzer, D R; Hu, J; Thomas, H F

    1996-06-01

    Psoriasis is a chronic, remitting and relapsing inflammatory skin disorder with a strong genetic predisposition. Psoriasis affects 1-3% of the world's population in their early lives representing a disabling condition with significant social and economic impact. Despite a great deal of research on the etiology and tissue destruction mechanisms, the disease is not well understood. The purpose of this paper is to provide current information from the literature with a special focus on oral manifestations. The major signs and symptoms presented in the oral environment of a psoriasis patient may include geographic tongue, fissure tongue, gingival and/or mucosal lesions. Inflammatory temporomandibular joint lesions have been reported in less than 5% of psoriasis patients. Multiple treatment strategies, be they topical or systemic, have been applied to these patients for symptom relief but not for cure.

  14. Cardiac manifestations of parasitic diseases.

    PubMed

    Nunes, Maria Carmo P; Guimarães Júnior, Milton Henriques; Diamantino, Adriana Costa; Gelape, Claudio Leo; Ferrari, Teresa Cristina Abreu

    2017-03-11

    The heart may be affected directly or indirectly by a variety of protozoa and helminths. This involvement may manifest in different ways, but the syndromes resulting from impairment of the myocardium and pericardium are the most frequent. The myocardium may be invaded by parasites that trigger local inflammatory response with subsequent myocarditis or cardiomyopathy, as occurs in Chagas disease, African trypanosomiasis, toxoplasmosis, trichinellosis and infection with free-living amoebae. In amoebiasis and echinococcosis, the pericardium is the structure most frequently involved with consequent pericardial effusion, acute pericarditis, cardiac tamponade or constrictive pericarditis. Chronic hypereosinophilia due to helminth infections, especially filarial infections, has been associated with the development of tropical endomyocardial fibrosis, a severe form of restrictive cardiomyopathy. Schistosomiasis-associated lung vasculature involvement may cause pulmonary hypertension (PH) and cor pulmonale Tropical pulmonary eosinophilia, which is characterised by progressive interstitial fibrosis and restrictive lung disease, may lead to PH and its consequences may occur in the course of filarial infections. Intracardiac rupture of an Echinococcus cyst can cause membrane or secondary cysts embolisation to the lungs or organs supplied by the systemic circulation. Although unusual causes of cardiac disease outside the endemic areas, heart involvement by parasites should be considered in the differential diagnosis especially of myocardial and/or pericardial diseases of unknown aetiology in both immunocompetent and immunocompromised individuals. In this review, we updated and summarised the current knowledge on the major heart diseases caused by protozoan and metazoan parasites, which either involve the heart directly or otherwise influence the heart adversely.

  15. Neurologic manifestations of Angelman syndrome.

    PubMed

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

    2013-04-01

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

  16. Respiratory manifestations in endocrine diseases

    PubMed Central

    LENCU, CODRUŢA; ALEXESCU, TEODORA; PETRULEA, MIRELA; LENCU, MONICA

    2016-01-01

    The control mechanisms of respiration as a vital function are complex: voluntary – cortical, and involuntary – metabolic, neural, emotional and endocrine. Hormones and hypothalamic neuropeptides (that act as neurotrasmitters and neuromodulators in the central nervous system) play a role in the regulation of respiration and in bronchopulmonary morphology. This article presents respiratory manifestations in adult endocrine diseases that evolve with hormone deficit or hypersecretion. In hyperthyroidism, patients develop ventilation disorders, obstructive and central sleep apnea, and pleural collection. The respiratory abnormalities in hyperthyroidism as a result of the hypermetabolic action of thyroid hormones are hyperventilation, myopathy and cardiovascular involvement; recent studies have reported pulmonary arterial hypertension in Graves’ disease, as a result of the association of several mechanisms. Thyroid hypertrophy can induce through compression of the upper airways dyspnea, stridor, wheezing and cough. The respiratory disorders in acromegaly are ventilatory dysfunction and sleep apnea, which contribute to an unfavorable evolution of the disease. Respiratory changes in parathyroid, adrenal and reproductive system diseases have been described. Respiratory disorders should be recognized, investigated and monitored by medical practitioners of various specialties (family physicians, internists, endocrinologists, pneumologists, cardiologists). They are frequently severe, causing an unfavorable evolution of the associated endocrine and respiratory disease. PMID:27857512

  17. [Collagen diseases with gastrointestinal manifestations].

    PubMed

    Takahashi, Hiroki; Ohara, Mikiko; Imai, Kohzoh

    2004-06-01

    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.

  18. Rheumatological manifestations in diabetes mellitus.

    PubMed

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

    2006-11-01

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

  19. Skin manifestations of drug allergy

    PubMed Central

    Ardern-Jones, Michael R; Friedmann, Peter S

    2011-01-01

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

  20. Skin manifestations in autoinflammatory syndromes.

    PubMed

    Braun-Falco, Markus; Ruzicka, Thomas

    2011-03-01

    Autoinflammatory diseases encompass a group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune and non-immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most common and best known. Genetic and molecular analyses demonstrated for the majority of these diseases an impairment of inflammasomes to cause an increased activity of an interleukin-1-dependent inflammatory response. Over the last years an increasing number of either rare hereditary syndromes or acquired common diseases could be summarized under the designation of autoinflammatory disease, thus creating an emerging new rubric of inflammatory diseases. Many of them display cutaneous manifestations as both concomitant or more rarely main symptoms. To name some of them like erysipelas-like erythema in FMF; urticaria-like rashes in tumor necrosis factor receptor 1- or cryopyrin-associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. Based on the new insights into pathogenesis one increasingly realizes the good response of these diseases to IL-1 antagonist therapies.

  1. Ocular manifestations of HIV infection.

    PubMed Central

    Jabs, D A

    1995-01-01

    all stages of HIV infection. Fifty-six percent of the cases of ocular toxoplasmosis had simultaneous toxoplasmic cerebritis. Ocular toxoplasmosis responded to standard anti-microbial therapy. Varicella zoster virus retinitis, when manifested by the acute retinal necrosis (ARN) syndrome, responded to intravenous acyclovir therapy. Conversely, in a limited number of patients with the progressive outer retinal necrosis syndrome, the disease responded poorly to intravenous acyclovir therapy, but appeared to respond to combination foscarnet and acyclovir therapy. Neuro-ophthalmic lesions were present in 6% of the patients with AIDS. The most common cause of a neuro-ophthalmic lesion was cryptococcal meningitis, and 25% of the patients with cryptococcal meningitis developed a neuro-ophthalmic complication. CONCLUSIONS: Ocular manifestations are common in patients with AIDS. CMV retinitis represented a major vision-threatening problem in these patients. While available therapy was successful in initially controlling the retinitis, the phenomenon of relapse resulted in some degree of long-term visual loss. Preservation of the patient's visual acuity in at least one eye was generally successful. Other opportunistic ocular infections were substantially less common than CMV retinitis but require aggressive therapy. Images FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6 PMID:8719695

  2. Gastrointestinal manifestations in cystic fibrosis.

    PubMed

    Eggermont, E

    1996-08-01

    CFTR, or cystic fibrosis transmembrane conductance regulator, the gene product that is defective in cystic fibrosis, is present in the apical membrane of the epithelial cells from the stomach to the colon. In the foregut, the clinical manifestations are not directly related to the primary defect of the CFTR chloride channel. The most troublesome complaints and symptoms originate from the oesophagus as peptic oesophagitis or oesophageal varices. In the small intestinal wall, the clinical expression of CF depends largely on the decreased secretion of fluid and chloride ions, the increased permeability of the paracellular space between adjacent enterocytes and the sticky mucous cover over the enterocytes. As a rule, the brush border enzyme activities are normal and there is some enhanced active transport as shown for glucose and alanine. The results of continuous enteral feeding of CF patients clearly show that the small intestinal mucosa, in the daily situation, is not functioning at maximal capacity. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, meconium ileus equivalent, intussusception, volvulus and silent appendicitis. In recent years colonic strictures, after the use of high-dose pancreatic enzymes, are being increasingly reported; the condition has recently been called CF fibrosing colonopathy. The CF gastrointestinal content itself differs mainly from the normal condition by the lower acidity in the foregut and the accretion of mucins and proteins, eventually resulting in intestinal obstruction, in the ileum and colon. Better understanding of the CF gastrointestinal phenotype may contribute to improvement of the overall wellbeing of these patients.

  3. [Neurologic manifestations of infectious endocarditis].

    PubMed

    Hannachi, N; Béard, T; Ben Ismail, M

    1991-01-01

    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

  4. 40 CFR 761.208 - Obtaining manifests.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Obtaining manifests. (a)(1) A generator may use... print the manifest under 40 CFR 262.21 (c) and (e). A registered source may be a: (i) State agency; (ii) Commercial printer; (iii) PCB waste generator, transporter or, designated facility; or (iv) PCB waste...

  5. Teaching about Manifest Destiny: Clarifying the Concept.

    ERIC Educational Resources Information Center

    Chiodo, John J.

    2000-01-01

    Provides information on Manifest Destiny and the expansionist ideals of the United States, especially during the 19th century. Offers a lesson plan that will help students understand the concept of Manifest Destiny, including an assessment activity, how to expand the lesson, an appendix, and references. (CMK)

  6. Systemic manifestations of traumatic brain injury.

    PubMed

    Gaddam, Samson Sujit Kumar; Buell, Thomas; Robertson, Claudia S

    2015-01-01

    Traumatic brain injury (TBI) affects functioning of various organ systems in the absence of concomitant non-neurologic organ injury or systemic infection. The systemic manifestations of TBI can be mild or severe and can present in the acute phase or during the recovery phase. Non-neurologic organ dysfunction can manifest following mild TBI or severe TBI. The pathophysiology of systemic manifestations following TBI is multifactorial and involves an effect on the autonomic nervous system, involvement of the hypothalamic-pituitary axis, release of inflammatory mediators, and treatment modalities used for TBI. Endocrine dysfunction, electrolyte imbalance, and respiratory manifestations are common following TBI. The influence of TBI on systemic immune response, coagulation cascade, cardiovascular system, gastrointestinal system, and other systems is becoming more evident through animal studies and clinical trials. Systemic manifestations can independently act as risk factors for mortality and morbidity following TBI. Some conditions like neurogenic pulmonary edema and disseminated intravascular coagulation can adversely affect the outcome. Early recognition and treatment of systemic manifestations may improve the clinical outcome following TBI. Further studies are required especially in the field of neuroimmunology to establish the role of various biochemical cascades, not only in the pathophysiology of TBI but also in its systemic manifestations and outcome.

  7. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.

  8. Genetic alterations in syndromes with oral manifestations.

    PubMed

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

    2013-11-01

    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.

  9. Wegener Granulomatosis: Otologic Manifestation as First Symptom.

    PubMed

    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2015-07-01

    Introduction Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective To describe a case of Wegener disease with atypical manifestation. Resumed Report We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.

  10. Genetic alterations in syndromes with oral manifestations

    PubMed Central

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

    2013-01-01

    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

  11. Important cutaneous manifestations of inflammatory bowel disease

    PubMed Central

    Trost, L; McDonnell, J

    2005-01-01

    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

  12. Musculoskeletal manifestations of the antiphospholipid syndrome.

    PubMed

    Noureldine, M H A; Khamashta, M A; Merashli, M; Sabbouh, T; Hughes, G R V; Uthman, I

    2016-04-01

    The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports.

  13. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... represent a full and complete enumeration and description of the cargo carried in this flight except that... manifests: Attached Shipper's Export Declarations represent a full and complete enumeration and...

  14. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... represent a full and complete enumeration and description of the cargo carried in this flight except that... manifests: Attached Shipper's Export Declarations represent a full and complete enumeration and...

  15. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... represent a full and complete enumeration and description of the cargo carried in this flight except that... manifests: Attached Shipper's Export Declarations represent a full and complete enumeration and...

  16. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... represent a full and complete enumeration and description of the cargo carried in this flight except that... manifests: Attached Shipper's Export Declarations represent a full and complete enumeration and...

  17. Cutaneous manifestations of dermatomyositis and their management.

    PubMed

    Callen, Jeffrey P

    2010-06-01

    Dermatomyositis is a condition with pathognomonic and characteristic cutaneous lesions. This article describes the skin manifestations observed in patients with dermatomyositis, their differential diagnosis, their relationship to internal disease (particularly malignancy), and their management.

  18. Mucocutaneous manifestations of helminth infections: Nematodes.

    PubMed

    Lupi, Omar; Downing, Christopher; Lee, Michael; Pino, Livia; Bravo, Francisco; Giglio, Patricia; Sethi, Aisha; Klaus, Sidney; Sangueza, Omar P; Fuller, Claire; Mendoza, Natalia; Ladizinski, Barry; Woc-Colburn, Laila; Tyring, Stephen K

    2015-12-01

    In the 21st century, despite increased globalization through international travel for business, medical volunteerism, pleasure, and immigration/refugees into the United States, there is little published in the dermatology literature regarding the cutaneous manifestations of helminth infections. Approximately 17% of travelers seek medical care because of cutaneous disorders, many related to infectious etiologies. This review will focus on the cutaneous manifestations of helminth infections and is divided into 2 parts: part I focuses on nematode infections, and part II focuses on trematode and cestode infections. This review highlights the clinical manifestations, transmission, diagnosis, and treatment of helminth infections. Nematodes are roundworms that cause diseases with cutaneous manifestations, such as cutaneous larval migrans, onchocerciasis, filariasis, gnathostomiasis, loiasis, dracunculiasis, strongyloidiasis, ascariasis, streptocerciasis, dirofilariasis, and trichinosis. Tremadotes, also known as flukes, cause schistosomiasis, paragonimiasis, and fascioliasis. Cestodes (tapeworms) are flat, hermaphroditic parasites that cause diseases such as sparganosis, cysticercosis, and echinococcus.

  19. Endocrine disorders and the neurologic manifestations

    PubMed Central

    2014-01-01

    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

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

    PubMed

    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

    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.

  1. Extra-articular Manifestations in Rheumatoid Arthritis

    PubMed Central

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

    2010-01-01

    ABSTRACT Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main characteristic is persistent joint inflammation that results in joint damage and loss of function. Although RA is more common in females, extra-articular manifestations of the disease are more common in males. The extra-articular manifestations of RA can occur at any age after onset. It is characterised by destructive polyarthritis and extra-articular organ involvement, including the skin, eye, heart, lung, renal, nervous and gastrointestinal systems. The frequence of extra-articular manifestations in RA differs from one country to another. Extra-articular organ involvement in RA is more frequently seen in patients with severe, active disease and is associated with increased mortality. Incidence and frequence figures for extra-articular RA vary according to study design. Extra-articular involvement is more likely in those who have RF and/or are HLA-DR4 positive. Occasionally, there are also systemic manifestations such as vasculitis, visceral nodules, Sjögren's syndrome, or pulmonary fibrosis present. Nodules are the most common extra-articular feature, and are present in up to 30%; many of the other classic features occur in 1% or less in normal clinic settings. Sjögren's syndrome, anaemia of chronic disease and pulmonary manifestations are relatively common – in 6-10%, are frequently present in early disease and are all related to worse outcomes measures of rheumatoid disease in particular functional impairment and mortality. The occurrence of these systemic manifestations is a major predictor of mortality in patients with RA. This paper focuses on extra-articular manifestations, defined as diseases and symptoms not directly related to the locomotor system. PMID:21977172

  2. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... transported under Customs red in-bond seals and is accompanied by a transportation in-bond manifest, a.... (c) Manifest forms to be used. The in-transit manifest forms to be used are: (1) For trucks,...

  3. Nocturnal manifestations of atypical parkinsonian disorders.

    PubMed

    Bhidayasiri, Roongroj; Jitkritsadakul, Onanong; Colosimo, Carlo

    2014-01-01

    Although nocturnal disturbances are increasingly recognized as an integral part of the continuum of daytime manifestations of Parkinson's disease (PD), there is still little evidence in the medical literature to support the occurrence of these complex phenomena in patients with atypical parkinsonian disorders (APDs). Based on the anatomical substrates in APDs, which are considered to be more extensive outside the basal ganglia than in PD, we might expect that patients with APDs encounter the whole range of nocturnal disturbances, including motor, sleep disorders, autonomic dysfunctions, and neuropsychiatric manifestations at a similar, or even greater, frequency than in PD. This article is a review of the current literature on the problems at nighttime of patients with progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. MEDLINE, life science journals and online books were searched by querying appropriate key words. Reports were included if the studies were related to nocturnal manifestations in APDs. Forty articles fulfilled the selection criteria. Differences between these symptoms in APDs and PD are highlighted, given the evidence available about each manifestation. This analysis of nocturnal manifestations of APDs suggests the need for future studies to address these issues to improve the quality of life not only of patients with APDs but the caregivers who encounter the challenges of supporting these patients on a daily basis.

  4. Pulmonary drug toxicity: radiologic and pathologic manifestations.

    PubMed

    Rossi, S E; Erasmus, J J; McAdams, H P; Sporn, T A; Goodman, P C

    2000-01-01

    Pulmonary drug toxicity is increasingly being diagnosed as a cause of acute and chronic lung disease. Numerous agents including cytotoxic and noncytotoxic drugs have the potential to cause pulmonary toxicity. The clinical and radiologic manifestations of these drugs generally reflect the underlying histopathologic processes and include diffuse alveolar damage (DAD), nonspecific interstitial pneumonia (NSIP), bronchiolitis obliterans organizing pneumonia (BOOP), eosinophilic pneumonia, obliterative bronchiolitis, pulmonary hemorrhage, edema, hypertension, or veno-occlusive disease. DAD is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. It manifests radiographically as bilateral hetero- or homogeneous opacities usually in the mid and lower lungs and on high-resolution computed tomographic (CT) scans as scattered or diffuse areas of ground-glass opacity. NSIP occurs most commonly as a manifestation of carmustine toxicity or of toxicity from noncytotoxic drugs such as amidarone. At radiography, it appears as diffuse areas of heterogeneous opacity, whereas early CT scans show diffuse ground-glass opacity and late CT scans show fibrosis in a basal distribution. BOOP, which is commonly caused by bleomycin and cyclophosphamide (as well as gold salts and methotrexate), appears on radiographs as hetero- and homogeneous peripheral opacities in both upper and lower lobes and on CT scans as poorly defined nodular consolidation, centrilobular nodules, and bronchial dilatation. Knowledge of these manifestations and of the drugs most frequently involved can facilitate diagnosis and institution of appropriate treatment.

  5. Cutaneous Manifestations of Systemic Lupus Erythematosus

    PubMed Central

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient. PMID:22888407

  6. Ocular manifestations in systemic lupus erythematosus.

    PubMed

    Silpa-archa, Sukhum; Lee, Joan J; Foster, C Stephen

    2016-01-01

    Systemic lupus erythematosus (SLE) can involve many parts of the eye, including the eyelid, ocular adnexa, sclera, cornea, uvea, retina and optic nerve. Ocular manifestations of SLE are common and may lead to permanent blindness from the underlying disease or therapeutic side effects. Keratoconjunctivitis sicca is the most common manifestation. However, vision loss may result from involvement of the retina, choroid and optic nerve. Ocular symptoms are correlated to systemic disease activity and can present as an initial manifestation of SLE. The established treatment includes prompt systemic corticosteroids, steroid-sparing immunosuppressive drugs and biological agents. Local ocular therapies are options with promising efficacy. The early recognition of disease and treatment provides reduction of visual morbidity and mortality.

  7. Caffeine-induced psychiatric manifestations: a review.

    PubMed

    Wang, Hee Ryung; Woo, Young Sup; Bahk, Won-Myong

    2015-07-01

    The association between caffeine consumption and various psychiatric manifestations has long been observed. We present two cases that show the ability of caffeine to induce psychotic and manic symptoms, and we also review the extant literature on caffeine-induced psychiatric manifestations. On the basis of our own and others' findings, we suggest that caffeine may be related to not only de-novo psychotic or mood symptoms but also to aggravation of pre-existing psychotic or mood disorders. We therefore suggest that caffeine consumption among patients with mood or psychotic symptoms should be assessed carefully in clinical practice as part of routine psychiatric evaluations.

  8. Ocular manifestations of Mycoplasma pneumoniae infection.

    PubMed

    Salzman, M B; Sood, S K; Slavin, M L; Rubin, L G

    1992-05-01

    Ocular manifestations of Mycoplasma pneumoniae infection, other than conjunctivitis, are uncommon. Optic disk swelling, optic nerve atrophy, retinal exudates and hemorrhages, and cranial nerve palsies have been infrequently reported. We describe a 15-year-old patient who developed bilateral optic disk edema and iritis during an acute infection with M. pneumoniae and review the world literature on findings associated with ocular manifestations of infection with this pathogen. Although our patient experienced complete resolution of iritis and optic disk edema after 6 weeks, several patients described in the literature have experienced permanent sequelae as a result of optic neuropathy.

  9. Superimposed segmental manifestation of cherry angiomas.

    PubMed

    Jaeger, Teresa; Andres, Christian; Hein, Rüdiger; Ring, Johannes; Chen, Wenchieh

    2011-01-01

    Cherry angioma is a common, acquired, vascular proliferation, probably of a polygenic mode of inheritance. Segmental manifestation of multiple cherry angiomas associated with the development of non-segmental lesions has not yet been reported. We describe a 62-year-old Caucasian woman with early formation of segmental cherry angiomas after pregnancy, which are superimposed on non-segmental lesions of later onset after menopause. In this pattern, segmental cherry angiomas could be taken as a further example of superimposed segmental manifestation of a polygenic skin disorder. Molecular research would be needed to confirm this hypothesis.

  10. Bodily manifestations in the psychoanalytic process.

    PubMed

    Vartzopoulos, Ioannis; Beratis, Stavroula

    2012-07-01

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

  11. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

  12. 21 CFR 11.50 - Signature manifestations.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ELECTRONIC RECORDS; ELECTRONIC SIGNATURES Electronic Records § 11.50 Signature manifestations. (a) Signed electronic... the same controls as for electronic records and shall be included as part of any human readable...

  13. Imaging the Abdominal Manifestations of Cystic Fibrosis

    PubMed Central

    McDermott, S.; Chan, V. O.; Ridge, C. A.

    2017-01-01

    Cystic fibrosis (CF) is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease. The capacity to predict those patients at risk of developing cirrhosis remains a significant challenge. We review representative abdominal imaging findings in patients with CF selected from the records of two academic health centres, with a view to increasing familiarity with the abdominal manifestations of the disease. We review their presentation and expected imaging findings, with a focus on the challenges facing diagnosis of the hepatic manifestations of the disease. An increased familiarity with these abdominal manifestations will facilitate timely diagnosis and management, which is paramount to further improving outcomes for patients with cystic fibrosis. PMID:28250993

  14. 40 CFR 761.208 - Obtaining manifests.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Obtaining manifests. (a)(1) A generator may use...) Commercial printer; (iii) PCB waste generator, transporter or, designated facility; or (iv) PCB waste...

  15. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping... 267.72 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE FACILITIES OPERATING UNDER A STANDARDIZED...

  16. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Complete manifest. 122.75 Section 122.75 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart;...

  17. Real-Life Contextual Manifestations of Wisdom

    ERIC Educational Resources Information Center

    Yang, Shih-Ying

    2008-01-01

    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…

  18. Hepatic angiosarcoma manifested as recurrent hemoperitoneum.

    PubMed

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

    2008-05-14

    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.

  19. Neoliberalism, Curriculum Development and Manifestations of "Creativity"

    ERIC Educational Resources Information Center

    Hakala, Juha T.; Uusikylä, Kari; Järvinen, Esa-Matti

    2015-01-01

    There is a manifest tendency for national education policy to follow global economic trends. In many Western industrialized countries, this relationship has intensified or strengthened within the last decades. The strengthening of this relationship has been seen, among other things, as evidence of the growing power of neoliberal ideology. The…

  20. Candidal endophthalmitis: a manifestation of systemic candidiasis.

    PubMed Central

    Patel, B. C.; Kaye, S. B.; Morgan, L. H.

    1987-01-01

    Two patients on total parenteral nutrition who developed endophthalmitis secondary to Candida albicans are described. Candidal endophthalmitis as a manifestation of systemic candidiasis is discussed and its early diagnosis by bedside fundal examination in patients at risk is stressed. Images Figure 1 Figure 2 PMID:3116523

  1. Retinal pigment epithelial hamartoma--unusual manifestations.

    PubMed Central

    Rosenberg, P. R.; Walsh, J. B.

    1984-01-01

    Hamartoma of the retinal pigment epithelium is an uncommon tumour of young adults. We have seen 2 patients with this clinical diagnosis, both with unusual manifestations. In one patient growth of the tumour was observed over a 5-year period. In the second patient arterial-arterial anastomoses were detected at a site distal to the tumour. Images PMID:6722077

  2. Rheumatic manifestations of inflammatory bowel disease.

    PubMed

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

    2009-11-28

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

  3. [Extra-articular manifestations of seronegative spondylarthritis].

    PubMed

    Cammelli, Daniele

    2006-05-01

    Seronegative spondylarthritis are frequently characterised by extra-articular manifestations. They are frequently in recurrent uveitis. Between the cutaneous manifestations should be mentioned erythema nodosum, typical of inflammatory bowel diseases, and keratoderma blenorrhagicum, in the Reiter's syndrome. Cardiac complications in ankylosing spondylitis (AS) include aortic valvular regurgitation and arrhythmia and, more rarely, mitral valvulopathy, cardiomyopathy and pericarditis. Pulmonary involvement in AS includes ventilatory restrictive syndrome and fibro-bullous disease of the apex. Vertebral osteoporosis is a very important extra-articular manifestation because of the possibility of spontaneous fractures of the vertebrae. Central neurological manifestations include medullary compression from cervical sub-luxation while the most important peripheral involvements are lumbar stenosis and the cauda equina syndrome. Type AA amyloidosis is a rare late complication of the AS, possible cause of death especially in patients with aggressive disease. Kidney complications can be observed as consequences of prolonged anti-inflammatory therapy, but the most frequent renal complications are amyloidosis and mesangial IgA segmental and focal glomerulonephritis.

  4. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 19 Customs Duties 1 2012-04-01 2012-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required. A manifest in duplicate covering the in-transit merchandise which is...

  5. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 1 2013-04-01 2013-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required. A manifest in duplicate covering the in-transit merchandise which is...

  6. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required. A manifest in duplicate covering the in-transit merchandise which is...

  7. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 19 Customs Duties 1 2014-04-01 2014-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required. A manifest in duplicate covering the in-transit merchandise which is...

  8. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of the... § 262.40(a). (b) The generator must give the transporter the remaining copies of the manifest. (c)...

  9. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of the... § 262.40(a). (b) The generator must give the transporter the remaining copies of the manifest. (c)...

  10. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of the... § 262.40(a). (b) The generator must give the transporter the remaining copies of the manifest. (c)...

  11. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of the... § 262.40(a). (b) The generator must give the transporter the remaining copies of the manifest. (c)...

  12. 40 CFR 262.24 - Use of the electronic manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.24 Use of the electronic... requirement in these regulations for a generator to keep or retain a copy of each manifest is satisfied by retention of a signed electronic manifest in the generator's account on the national e-Manifest...

  13. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of the... § 262.40(a). (b) The generator must give the transporter the remaining copies of the manifest. (c)...

  14. How to Participate: Testing the e-Manifest System (e-Manifest)

    EPA Pesticide Factsheets

    This page explains options for stakeholders like TSDFs and generators who wish to contribute to the discussion about the development of e-manifest. Directions are provided here to help the stakeholders test and provide feedback on the system.

  15. PATTERN OF CUTANEOUS MANIFESTATIONS IN DIABETES MELLITUS

    PubMed Central

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

    2010-01-01

    Background: Diabetes mellitus affects individuals of all ages and socioeconomic status. Skin is affected by the acute metabolic derangements as well as by chronic degenerative complications of diabetes. Aims: To evaluate the prevalence of skin manifestations in patients with diabetes mellitus. To analyze the prevalence and pattern of skin disorders among diabetic patients from this region of Western Himalayas. Materials and Methods: One hundred consecutive patients with the diagnosis of diabetes mellitus and having skin lesions, either attending the diabetic clinic or admitted in medical wards were included in this study. Results: The common skin disorders were: Xerosis (44%), diabetic dermopathy (36%), skin tags (32%), cutaneous infections (31%), and seborrheic keratosis (30%). Conclusion: Skin is involved in diabetes quite often and the manifestations are numerous. High prevalence of xerosis in our diabetic population is perhaps due to cold and dry climatic conditions in the region for most of the time in the year. PMID:20418975

  16. [Cerebrotendinous xanthomatosis: physiopathology, clinical manifestations and genetics].

    PubMed

    Preiss, Yudith; Santos, José L; Smalley, Susan V; Maiz, Alberto

    2014-05-01

    Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disease, caused by genetic deficiency of the 27-hydroxylase enzyme (encoded by CYP27A1). It plays a key role in cholesterol metabolism, especially in bile acid synthesis and in the 25-hydroxylation of vitamin D3 in the liver. Its deficiency causes reduced bile acid synthesis and tissue accumulation of cholestanol. Clinical manifestations are related to the presence of cholestanol deposits and include tendon xanthomas, premature cataracts, chronic diarrhea, progressive neurologic impairment and less frequently coronary heart disease, early onset osteoporosis and abnormalities in the optic disk and retina. An early diagnosis and treatment with quenodeoxycholic acid may prevent further complications, mainly neurological manifestations. This review summarizes cholesterol metabolism related to bile acid synthesis, physiopathology, biochemistry and treatment of cerebrotendinous xanthomatosis.

  17. Acute dacryocystitis: another clinical manifestation of sporotrichosis.

    PubMed

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

    2014-04-01

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

  18. Cutaneous manifestations of familial amyloidotic polyneuropathy.

    PubMed

    Rocha, N; Velho, G; Horta, M; Martins, A; Massa, A

    2005-09-01

    Familial amyloidotic polyneuropathy is an autosomal dominant amyloidosis, characterized by the systemic deposition of amyloid with a particular involvement of the peripheral nerves. The disease generally manifests as a severe sensory, motor and autonomic neuropathy. Cardiomyopathy, nephropathy, vitreous opacities and carpal tunnel syndrome may occur in a variable association with the neuropathy. Trophic dermatological lesions are frequent in the more advanced stages of the disease. We examined the skin of 142 patients. The cutaneous manifestations more frequently observed were: xerosis (81.6%), seborrheic dermatitis (21.8%), traumatic and burn lesions (19.7%), acne (18.3%), neurotrophic ulcers (14%) and onychomycosis (10.5%). Among the hepatic transplanted patients (31%), seborrheic dermatitis and acne were the most frequent diagnoses.

  19. Pulmonary manifestations of sickle cell disease

    PubMed Central

    Siddiqui, A; Ahmed, S

    2003-01-01

    Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed. PMID:12897216

  20. Oral manifestations of HIV positive children.

    PubMed

    Magalhães, M G; Bueno, D F; Serra, E; Gonçalves, R

    2001-01-01

    Oral manifestations in HIV positive children were observed in thirty-eight HIV infected children that have received care at the Special Care Dentistry Center (SCDC) of the School of Dentistry, University of Sao Paulo. Results have shown that 52.63% of the children presented at least one oral manifestation related with HIV/AIDS. Angular cheilitis occurred in 28.94%, parotid gland bilateral enlargement, pseudomembranous candidiasis and erythematous candidiasis in 18.42%, conventional gingivitis in 13.15%, herpes simplex in 5.26%, hairy leukoplakia, recurrent aphthous ulcer and condyloma acuminatum in 2.63%. Although enamel hypoplasia occurred in 23.68%, this could not be attributed specifically to HIV infection.

  1. The Chronic Gastrointestinal Manifestations of Chagas Disease

    PubMed Central

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

    2009-01-01

    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

  2. Gastrointestinal Manifestations in Systemic Autoimmune Diseases

    PubMed Central

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

    2011-01-01

    ABSTRACT In an autoimmune disease, the immune system attacks and harms the body's own tissues. The systemic autoimmune diseases include collagen vascular diseases, the systemic vasculitides, Wegener granulomatosis, and Churg-Strauss syndrome. These disorders can involve any part of the gastrointestinal tract, hepatobiliary system and pancreas. They can cause a variety of gastrointestinal manifestations that are influenced by the pathophysiologic characteristics of the underlying disease process. There is a wide variation of gastrointestinal manifestations from these autoimmune disorders including, but not limited to: oral ulcers, dysphagia, gastroesophageal reflux disease, abdominal pain, constipation, diarrhea, fecal incontinence, pseudo-obstruction, perforation and gastrointestinal bleeding. Clinical workup should be initiated by the patient's subjective complaints. In this review, we analyze the effects of autoimmune diseases on the gastrointestinal tract. PMID:21977190

  3. Acute dacryocystitis: another clinical manifestation of sporotrichosis

    PubMed Central

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

    2013-01-01

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

  4. Nasal manifestations in chromium industry workers.

    PubMed

    Aiyer, R G; Kumar, Gaurav

    2003-04-01

    People working in mines, plating factories, cement industries are mainly exposed to chrome substances, IIexavalent chromium has been implicated for its toxic effect on the nasal mucosa. Hereby we present a rare study of 28 patients who attended out patient department of Otorhinolaryngology at SSG Hospital, Baroda from a nearby chromium industry. This study aims to present various nasal manifestations of toxic effects of prolonged chromium exposure.

  5. Oral manifestations of Ehlers-Danlos syndrome.

    PubMed

    Létourneau, Y; Pérusse, R; Buithieu, H

    2001-06-01

    Ehlers-Danlos syndrome is a rare hereditary disease of the connective tissue which can present oral manifestations. A brief history of the disease is presented along with the epidemiology and characteristics of the 8 main phenotypes of the syndrome. The article also describes the case of a 12-year-old patient presenting with hypermobility of the temporo-mandibular joint and capillary fragility, and highlights the precautions to take when treating patients with this syndrome.

  6. Peripheral Nervous System Manifestations of Infectious Diseases

    PubMed Central

    Brizzi, Kate T.

    2014-01-01

    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

  7. Endocrine manifestations of systemic mastocytosis in bone.

    PubMed

    Greene, Loren Wissner; Asadipooya, Kamyar; Corradi, Patricia Freitas; Akin, Cem

    2016-09-01

    Systemic Mastocytosis (SM) is characterized by accumulation of clonal, neoplastic proliferations of abnormal mast cells (MC) in one or more organ system other than skin. Presence of these multifocal clusters of abnormal mast cells is an essential feature of SM. Frequently associated with D816V (KIT) mutation, the presence of this mutation and elevated serum tryptase are minor criteria for diagnosis. SM manifestations depend on the degree of mast cell proliferation, activation and degranulation. SM has a variable prognosis and presentation, from indolent to "smoldering" to life-threatening disease. Bone manifestations of SM include: osteopenia with or without lytic lesions, osteoporosis with or without atraumatic fracture, osteosclerosis with increased bone density, and isolated lytic lesions. Male sex, older age, higher bone resorption markers, lower DKK1 level, lower BMD, absence of urticaria pigmentosa, and alcohol intake are all associated with increased risk of fracture. Treatment of SM is generally palliative. Most therapy is symptom-directed; and, infrequently, chemotherapy for refractory symptoms is indicated. Anti-histamines may alleviate direct bone effects of histamine. Bisphosphonates, including alendronate, clodronate, pamidronate and zoledronic acid are recommended as a first line treatment of SM and osteoporosis. Interferon α may act synergistically with bisphosphonates. As elevation of RANKL and OPG is reported in SM, denosumab could be an effective therapy for bone manifestations of SM.

  8. Orthopaedic manifestations of neurofibromatosis type 1.

    PubMed

    Feldman, David S; Jordan, Charles; Fonseca, Lauren

    2010-06-01

    Neurofibromatosis type 1 (NF-1) is an autosomal dominant disease that affects 1 in 3,000 persons worldwide. Café-au-lait macules and peripheral nerve sheath tumors (ie, neurofibromas) are the most commonly recognized manifestations of NF-1. However, NF-1 affects multiple organ systems, and a multidisciplinary approach to treatment is required. Management of the orthopaedic manifestations of NF-1 is often difficult. The most complex manifestations are scoliosis (dystrophic and nondystrophic), congenital pseudarthrosis of the tibia, and problems related to soft-tissue tumors. Metabolic bone disease is common; many patients are frankly osteopenic, which further complicates treatment. Dystrophic scoliosis, which may be caused by either bony dysplasia or intraspinal pathology, is characterized by early presentation and rapid progression. Pseudarthrosis is common even after instrumented fusion. Nondystrophic scoliosis tends to behave like adolescent idiopathic scoliosis, although it may present earlier and is associated with a higher rate of pseudarthrosis. Congenital pseudarthrosis of the tibia is a long-bone dysplasia that afflicts patients with NF-1. Management of this osseous deformity is challenging. Failure to achieve union and refracture are common.

  9. Skin Manifestations of Inflammatory Bowel Disease

    PubMed Central

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

    2012-01-01

    Inflammatory bowel disease (IBD) is a disease that affects the intestinal tract via an inflammatory process. Patients who suffer from IBD often have diseases that affect multiple other organ systems as well. These are called extraintestinal manifestations and can be just as, if not more debilitating than the intestinal inflammation itself. The skin is one of the most commonly affected organ systems in patients who suffer from IBD. The scientific literature suggests that a disturbance of the equilibrium between host defense and tolerance, and the subsequent over-activity of certain immune pathways are responsible for the cutaneous disorders seen so frequently in IBD patients. The purpose of this review article is to give an overview of the types of skin diseases that are typically seen with IBD and their respective pathogenesis, proposed mechanisms, and treatments. These cutaneous disorders can manifest as metastatic lesions, reactive processes to the intestinal inflammation, complications of IBD itself, or side effects from IBD treatments; these can be associated with IBD via genetic linkage, common autoimmune processes, or other mechanisms that will be discussed in this article. Ultimately, it is important for healthcare providers to understand that skin manifestations should always be checked and evaluated for in patients with IBD. Furthermore, skin disorders can predate gastrointestinal symptoms and thus may serve as important clinical indicators leading physicians to earlier diagnosis of IBD. PMID:22347192

  10. Cutaneous Manifestations of Common Liver Diseases

    PubMed Central

    Dogra, Sunil; Jindal, Rashmi

    2012-01-01

    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. PMID:25755383

  11. Hematological manifestations of primary mitochondrial disorders.

    PubMed

    Finsterer, Josef

    2007-01-01

    At onset mitochondrial disorders (MID) frequently manifest as a mono-organic problem but turn into multisystem disease during the disease course in most of the cases. Organs/tissues most frequently affected in MID are the cerebrum, peripheral nerves, and the skeletal muscle. Additionally, most of the inner organs may be affected alone or in combination. Hematological manifestations of MID include aplastic, megaloblastic, or sideroblastic anemia, leukopenia, neutropenia, thrombocytopenia, or pancytopenia. In single cases either permanent or recurrent eosinophilia has been observed. Hematological abnormalities may occur together with syndromic or nonsyndromic MIDs. Syndromic MIDs, in which hematological manifestations predominate, are the Pearson syndrome (pancytopenia), Kearns-Sayre syndrome (anemia), Barth syndrome (neutropenia), and the autosomal recessive mitochondrial myopathy, lactic acidosis and sideroblastic anemia syndrome. In single cases with Leigh's syndrome, MERRF (myoclonic epilepsy and ragged-red fiber) syndrome, Leber's hereditary optic neuropathy, and Friedreich's ataxia anemia has been described. Anemia, leukopenia, thrombocytopenia, eosinophilia, or pancytopenia can frequently also be found in nonsyndromic MIDs with or without involvement of other tissues. Therapy of blood cell involvement in MID comprises application of antioxidants, vitamins, iron, bone marrow-stimulating factors, or substitution of cells.

  12. Chronic polyarthritis as isolated manifestation of toxocariasis.

    PubMed

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

    2016-01-01

    Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or Toxocara cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of the 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 the 5809 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 30min. 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 (1:1280). She was treated with paracetamol (40mg/kg/day) and thiabendazole (25mg/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.

  13. Phenotypic and dermatological manifestations in Down Syndrome.

    PubMed

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

    2011-02-15

    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.

  14. [Psychiatric manifestations in dementia: phenomenologic perspectives].

    PubMed

    Paquette, I

    1993-12-01

    The study of psychiatric manifestations in dementia has long been overshadowed by the more classical manifestations of the disease, such as memory loss and other cognitive deficits. In recent years, however, psychiatric symptoms as part of the demential process have attracted interest and research has become more specific. Clinicians are faced with diagnostic, treatment and management difficulties related to affective or psychotic symptoms, which account for much distress and morbidity. Several studies indicate that the prevalence of psychiatric manifestations in clinical populations of patients suffering from dementia is high: 15% to 30% for hallucinations, 15% to 30% for delusions, ten percent to 20% for major depression and 40% to 50% for depressed mood. These figures tend to confirm the hypothesis that psychiatric features in dementia are neither infrequent nor atypical. Thus, researchers have sought to link psychotic or depressive symptomatology with several clinical characteristics of dementia, namely stage, severity, prognosis or cognitive dysfunction. Some recent studies involving extensive neuropsychological evaluations indicate that subgroups of patients can be defined according to psychiatric criteria, as well as cognitive or neurological criteria. Unfortunately, results are inconsistent. Some of the contradictions in the literature are related to poorly defined terms and symptoms, a lack of reliable operational criteria, absence of validation of instruments and scales and heterogeneity of the populations studied. Ambiguous syndromes, such as pseudodementia, while illustrative of certain clinical situations, have not been helpful in categorizing demented patients. The author suggests that research focused on specific and clearly defined psychiatric symptoms in dementia will better serve our comprehension of mixed syndromes.

  15. Morphologic manifestations of testicular and epididymal toxicity

    PubMed Central

    Vidal, Justin D; Whitney, Katharine M

    2014-01-01

    Histopathologic examination of the testis is the most sensitive means to detect effects on spermatogenesis; however, the complexity of testicular histology, interrelatedness of cell types within the testis, and long duration of spermatogenesis can make assessment of a testicular toxicant challenging. A thorough understanding of the histology and morphologic manifestations of response to injury is critical to successfully identify a testicular effect and to begin to understand the underlying mechanism of action. The basic patterns of response to xenobiotic-induced injury to the testis and epididymis are detailed and discussed. PMID:26413388

  16. Hemiplegia: an initial manifestation of Japanese encephalitis.

    PubMed

    Nalini, A; Arunodaya, G R; Taly, A B; Swamy, H S; Vasudev, M K

    2003-09-01

    A 7-year-old boy from an area endemic to Japanese encephalitis (JE) manifested with acute febrile illness, left hemiplegia and preserved consciousness during the prodromal phase of illness. The child developed features of encephalitis 48 hours after the onset of hemiplegia. IgM MAC ELISA for JE virus revealed high titers in the serum and cerebrospinal fluid suggestive of JE. MRI of the brain showed asymmetrical bilateral thalamic hyperintense lesions on T2 weighted image, considered diagnostic of JE. Hemiplegia during the prodromal phase or as an initial symptom of JE is rather unusual.

  17. Norwegian scabies - rare case of atypical manifestation*

    PubMed Central

    Ebrahim, Karina Corrêa; Alves, Júlia Barazetti; Tomé, Lísias de Araújo; de Moraes, Carlos Floriano; Gaspar, Arianne Ditzel; Franck, Karin Fernanda; Hussein, Mohamad Ali; da Cruz, Lucas Raiser; Ebrahim, Leonardo Duque; Sidney, Luis Felipe de Oliveira

    2016-01-01

    Human scabies affects all social classes and different races around the world. It is highly contagious, but the exact figures on its prevalence are unknown. A 19-year-old male patient was admitted to the emergency room reporting fever (38°C) and multiple lesions throughout the body, except face, soles, and palms. Lesions were non-pruritic, which hampered the initial diagnostic suspicion. Skin biopsy was performed, and the final diagnosis was crusted scabies (Norwegian). It was concluded that human scabies is a significant epidemic disease, due to its different clinical manifestations, and because it is extremely contagious. PMID:28099611

  18. Delayed cutaneous manifestations of drug hypersensitivity.

    PubMed

    Bircher, Andreas J; Scherer, Kathrin

    2010-07-01

    Drugs may elicit a considerable variety of clinical signs, often affecting the skin and the mucous membranes. The most common are maculopapular exanthemas and urticaria, more rarely pustules, bullae vasculitic lesions, and lichenoid lesions may also be observed. Apart from the morphology, the chronology of the occurrence and the evolution of single skin lesions and exanthema are also paramount in the clinical diagnosis of cutaneous drug hypersensitivity. Often, the skin represents the only organ manifestation; however, it may be the herald for a systemic involvement of internal organs, such as in severe drug-induced hypersensitivity syndromes or anaphylaxis.

  19. Extraesophageal manifestations of gastroesophageal reflux disease.

    PubMed

    Hom, Christopher; Vaezi, Michael F

    2013-03-01

    This article discusses the current state of knowledge regarding the relationship between gastroesophageal reflux disease (GERD) and pulmonary and ear/nose/throat manifestations of reflux and outlines the recent developments in the diagnostic and treatment strategies for this difficult group of patients. Upper gastrointestinal endoscopy and pH monitoring are poorly sensitive for diagnosing reflux in this group of patients. Instead it is recommended that in those without warning symptoms, an empiric trial of proton-pump inhibitors be the initial approach to diagnosing and treating the potential underlying cause of these extraesophageal symptoms.

  20. Dark matter: Observational manifestation and experimental searches

    NASA Astrophysics Data System (ADS)

    Vavilova, I. B.; Bolotin, Yu. L.; Boyarsky, A. M.; Danevich, F. A.; Kobychev, V. V.; Tretyak, V. I.; Babyk, Iu. V.; Iakubovskyi, D. A.; Hnatyk, B. I.; Sergeev, S. G

    2015-08-01

    This monograph is the third issue of a three volume edition under the general title "Dark Energy and Dark Matter in the Universe". The authors discuss the astrophysical direct and indirect manifestation and properties of dark matter in galaxies, galaxy clusters and groups; the different mechanisms of energy exchange between dark energy and dark matter that expand the capabilities of the Standard Cosmological Model; the experimental search for dark matter particle candidates (including the sterile neutrinos, solar axions,weakly-interacting massive particles, and superheavy dark matter particles) using space, ground-based, and underground observatories.

  1. [Ebola: characterization, history and cutaneous manifestations].

    PubMed

    Kolbach, Marianne; Carrasco-Zuber, Juan Eduardo; Vial-Letelier, Verónica

    2015-11-01

    Ebola virus (EV) is one of the most virulent human pathogens. Fruits bats are its natural reservoir, the transmission to humans is across wild animals (especially primates) and the propagation in human populations is through bodily fluid contact. The actual outbreak started in December 2013 and crossed continental borders. Up to now, there are 17,145 suspected and confirmed cases with 6,070 deaths, resulting a total case fatality rate of 35%. Clinical manifestations can be divided in 3 phases. In phase I, symptoms are similar to flu, which may appear in a range of 2 to 21 days. In phase II which occurs in over 50% of cases, visceral symptoms and mucocutaneous manifestations appear within 4 and 5 days of the onset of symptoms. The main symptoms are a macular or maculopapular non-pruritic rash, desquamation and mucosal involvement of eyes, mouth and pharynx. In phase III, recovery or death occurs. The diagnosis is made on clinical grounds, epidemiological suspicion and a positive polymerase chain reaction (PCR) test. The treatment is supportive. If there is a suspected case, it should be notified immediately and all relevant safety measures should be instituted.

  2. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis

    PubMed Central

    Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-01-01

    Hashimoto’s encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient’s paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto’s encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids. PMID:27672526

  3. Hematologic manifestations of Helicobacter pylori infection

    PubMed Central

    Campuzano-Maya, Germán

    2014-01-01

    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

  4. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis.

    PubMed

    Haider, Ali S; Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-07-05

    Hashimoto's encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient's paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto's encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids.

  5. [Psychiatric manifestations by prions. A narrative review].

    PubMed

    Carrillo Robles, Daniel; García Maldonado, Gerardo

    2016-01-01

    Prion diseases are a group of rare and rapidly progressive neurodegenerative conditions that may cause neuropsychiatric symptoms. This group of diseases has been described since the 18(th) century, but they were recognized decades later, when it became clear that the humans were affected by infected animals. There was controversy when the problem was attributed to a single protein with infective capacity. The common pathological process is characterized by the conversion of the normal cellular prion protein into an abnormal form. In humans, the illness has been classified as idiopathic, inherited and acquired through exposure to exogenous material containing abnormal prions. The most prominent neurological manifestation of prion diseases is the emergence of a rapidly progressive dementia, mioclonus associated with cerebellar ataxia and also extra pyramidal symptoms. Psychiatric symptoms occur in early stages of the illness and can contribute to timely diagnosis of this syndrome. Psychiatric symptoms have traditionally been grouped in three categories: affective symptoms, impaired motor function and psychotic symptoms. Such events usually occur during the prodromal period prior to the neurological manifestations and consists in the presence of social isolation, onset of delusions, irritability/aggression, visual hallucinations, anxiety and depression, and less frequent first-rank symptoms among others. Definite diagnosis requires post mortem examination. The possibility that a large number of cases may occur in the next years or that many cases have not been considered with this diagnosis is a fact. In our opinion, psychiatrists should be aware of symptoms of this disease. The main objective of this research consisted of assessing the correlation between this disturbance and neuro-psychiatric symptoms and particularly if this psychiatric manifestations integrate a clinical picture suggestive for the diagnosis of these diseases, but firstly reviewed taxonomic

  6. 14 CFR 121.693 - Load manifest: All certificate holders.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Load manifest: All certificate holders. 121... OPERATING REQUIREMENTS: DOMESTIC, FLAG, AND SUPPLEMENTAL OPERATIONS Records and Reports § 121.693 Load manifest: All certificate holders. The load manifest must contain the following information concerning...

  7. Oral osteomyelitis: pre-AIDS manifestation or strange coincidence?

    PubMed

    Harel-Raviv, M; Gorsky, M; Lust, I; Raviv, E

    1996-01-01

    A bizarre and unexplained localized osteomyelitis was discovered in the mandible of an otherwise apparently healthy 36-year-old man. This strange oral manifestation was followed 2 years later by a diagnosis of Pneumocystis carinii pneumonia, which indicated full-blown AIDS. Could osteomyelitis of the mandible be an alarming oral manifestation of AIDS before the disease is manifested in other ways?

  8. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for... paragraphs (d) and (e) of this section, an air cargo manifest need not be filed or retained aboard the... air express or freight shall be manifested as other air express or freight. (e) Accompanied baggage...

  9. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs... AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for..., and Overflying the United States § 122.48 Air cargo manifest. (a) When required. Except as provided...

  10. 76 FR 36480 - Hazardous Waste Manifest Printing Specifications Correction Rule

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-22

    ... AGENCY 40 CFR Part 262 Hazardous Waste Manifest Printing Specifications Correction Rule AGENCY... proposing a minor change to the Resource Conservation and Recovery Act (RCRA) hazardous waste manifest regulations that affects those entities that print the hazardous waste manifest form in accordance with...

  11. Extraintestinal manifestations in inflammatory bowel disease

    PubMed Central

    Danese, Silvio; Semeraro, Stefano; Papa, Alfredo; Roberto, Italia; Scaldaferri, Franco; Fedeli, Giuseppe; Gasbarrini, Giovanni; Gasbarrini, Antonio

    2005-01-01

    Inflammatory bowel diseases (IBD) can be really considered to be systemic diseases since they are often associated with extraintestinal manifestations, complications, and other autoimmune disorders. Indeed, physicians who care for patients with ulcerative colitis and Crohn’s disease, the two major forms of IBD, face a new clinical challenge every day, worsened by the very frequent rate of extraintestinal complications. The goal of this review is to provide an overview and an update on the extraintestinal complications occurring in IBD. Indeed, this paper highlights how virtually almost every organ system can be involved, principally eyes, skin, joints, kidneys, liver and biliary tracts, and vasculature (or vascular system) are the most common sites of systemic IBD and their involvement is dependent on different mechanisms. PMID:16437620

  12. Cerebellar Stroke-manifesting as Mania

    PubMed Central

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

    2014-01-01

    Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed. PMID:25035567

  13. Uncommon manifestations of neuroborreliosis in children.

    PubMed

    Baumann, Matthias; Birnbacher, Robert; Koch, Johannes; Strobl, Robert; Rostásy, Kevin

    2010-05-01

    Lyme borreliosis is a tick-borne spirochetal infection which affects the skin, joints, heart and nervous system. Children with a neuroborreliosis usually present with a facial nerve palsy or aseptic meningitis, but the spectrum also includes other rare manifestations. We report four unusual cases of childhood neuroborreliosis and show that seizures with regional leptomeningeal enhancement, acute transverse myelitis, meningoradiculitis with pain and paraesthesia and cranial nerve palsies other than facial nerve palsy can be the leading symptoms of children with neuroborreliosis. All children had serological evidence of an acute infection with Borrelia burgdorferi, a pleocytosis in the cerebrospinal fluid and a complete response to antibiotic treatment. An intrathecal synthesis of IgG antibodies was detected in three children. Thus, diagnostic work up in children with unusual neurological symptoms should include cerebrospinal fluid studies with determination of the white blood cell count and calculation of the antibody index against B. burgdorferi.

  14. [Orthostatic hypotension: an unusual manifestation of pheochromocytoma].

    PubMed

    Tagle, Rodrigo; Acosta, Pamela; Valdés, Gloria

    2003-12-01

    Pheochromocytoma, though an uncommon cause of hypertension, can be a lethal condition. Because of this it is mandatory to diagnose it or rule it out in presence of suggestive symptoms. Typical symptoms are palpitations, sweating, severe headaches and hypertension. However, there are other suggestive symptoms of this dangerous endocrine entity, one of which is the orthostatic hypotension. We report the case of a 65 years old female patient with long standing hypertension in whom the pheochromocytoma was suspected after episodes of orthostatic hypotension. Although this manifestation was described almost fifty years ago, its frequency and pathophysiology has not yet been well established and fully elucidated. Moreover, it has meaningful implications in relation to preoperatory management and the timing of surgery.

  15. Gingival hypertrophy: a solitary manifestation of scurvy.

    PubMed

    Li, Ryan; Byers, Karin; Walvekar, Rohan R

    2008-01-01

    A 48-year-old woman presented with rapid onset hypertrophy of both the upper and lower gingiva. A detailed history and a diet nearly void of fruits and vegetables raised the suspicion of a possible deficiency of vitamin C. An estimation of the serum ascorbic acid level confirmed our suspicions (<0.12 mg/dL; normal range, 0.4-1.0 mg/dL) and a diagnosis of scurvy was confirmed. A course of 1000 mg/d of ascorbic acid was initiated that caused a dramatic resolution of the gingival lesions. Gingival hypertrophy has a large differential diagnosis; however, it is also known to be an extremely rare manifestation of scurvy. A high index of suspicion for scurvy is relevant given its low prevalence in developed nations and how easily remediable the disease can be, even when it presents in a severe form.

  16. Patent foramen ovale: clinical manifestations and treatment.

    PubMed

    Kedia, Gautam; Tobis, Jonathan; Lee, Michael S

    2008-01-01

    A persistent patent foramen ovale produces an intermittent intra-atrial right-to-left shunt and occurs in approximately 25% of the general population. Although the vast majority of people with patent foramen ovale are asymptomatic, a patent foramen ovale is believed to act as a pathway for chemicals or thrombus that can result in a variety of clinical manifestations, including stroke, migraine headache, decompression sickness, high-altitude pulmonary edema, and platypnea-orthodeoxia syndrome. The optimal management of patients with patent foramen ovale who experience cryptogenic stroke is unclear. Percutaneous closure appears to have a low risk profile and has been considered in high-risk patients who are not candidates for randomized clinical trials. Randomized clinical trials that are underway should help define the best management of patent foramen ovale, as well as the true safety and efficacy of percutaneous closure devices.

  17. [Chronic obstructive lung disease. Systemic manifestations].

    PubMed

    Grassi, Vittorio; Carminati, Luisa; Cossi, Stefania; Marengoni, Alessandra; Tantucci, Claudio

    2003-05-01

    Chronic obstructive lung diseases (COPD) are a complex disease state which not rarely can be associated with significant systemic manifestations. These alterations, though recognized since long time, are currently under extensive research, due to the increasing appreciation of their relevant negative role in the prognosis and health-related quality of life (Hr-QoL) of the COPD patients. The most clinically important are the decrease in body weight with loss of skeletal muscle mass (cachexia), osteoporosis, hypercapnia-induced peripheral edema, neuro-psychiatric disorders, such as oxygen-related cognitive impairment and depression, excessive polycytaemia and sleep disorders. Chronic systemic inflammation, oxidative stress and chronic hypoxia are believed as the main factors involved in the pathogenesis of systemic effects seen in COPD. Their adequate control with nutritional support, change of life-style and targeted pharmacological treatment is able to improve the prognosis and Hr-QoL among these COPD patients.

  18. Vascular Manifestations of von Recklinghausen's Disease

    PubMed Central

    Fye, Kenneth H.; Jacobs, Richard P.; Roe, Robert L.

    1975-01-01

    A casual relationship between von Recklinghausen's disease, or neurofibromatosis, and arteriolar abnormalities has been reported in the European literature. A patient was seen who had biopsy-proved neurofibromatosis and renovascular hypertension and retroperitoneal bleeding. An arteriographic study showed multiple small aneurysms throughout the coeliac axis, the superior mesenteric artery and in several small intrarenal vessels. Renal vein renin levels were elevated particularly in the right renal vein, supporting the diagnosis of renovascular hypertension. Both the aneurysms seen in angiographic studies and the retroperitoneal hemorrhage are probably vascular manifestations of von Recklinghausen's disease. Support for this conclusion is enhanced by the absence of clinical, laboratory or histologic data supporting the only tenable differential diagnosis, periarteritis nodosa. ImagesFigure 1.Figure 2.Figure 3.Figure 4. PMID:803743

  19. [Uncommon clinical manifestations of cutaneous leishmaniasis].

    PubMed

    Hayani, K; Dandashli, A; Weisshaar, E

    2014-10-01

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

  20. Hereditary angioedema: imaging manifestations and clinical management.

    PubMed

    Gakhal, Mandip S; Marcotte, Gregory V

    2015-02-01

    Hereditary angioedema is a genetic disorder typically related to insufficient or dysfunctional C1-esterase inhibitor. Patients present with episodic swelling of various body parts, such as the face, neck, bowel, genitals, and extremities. Acute or severe symptoms can lead to patients presenting to the emergency room, particularly when the neck and abdominopelvic regions are affected, which is often accompanied by radiologic imaging evaluation. Patients with hereditary angioedema can pose a diagnostic challenge for emergency department physicians and radiologists at initial presentation, and the correct diagnosis may be missed or delayed, due to lack of clinical awareness of the disease or lack of its consideration in the radiologic differential diagnosis. Timely diagnosis of hereditary angioedema and rapid initiation of appropriate therapy can avoid potentially life-threatening complications. This article focuses on the spectrum of common and characteristic acute imaging manifestations of hereditary angioedema and provides an update on important recent developments in its clinical management and treatment.

  1. Coronary Artery Manifestations of Fibromuscular Dysplasia

    PubMed Central

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

    2015-01-01

    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

  2. Coronary artery manifestations of fibromuscular dysplasia.

    PubMed

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

    2014-09-09

    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.

  3. Imaging Manifestations of Mediastinal Fat Necrosis

    PubMed Central

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

    2013-01-01

    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

  4. Clinical manifestations of choanal atresia in infancy.

    PubMed

    Cozzi, F; Steiner, M; Rosati, D; Madonna, L; Colarossi, G

    1988-03-01

    The clinical features of 41 infants with various types of nasal obstruction were reviewed to study the correlation between the degree of nasal obstruction and clinical manifestations. Twenty-one subjects had bilateral choanal atresia/stenosis; 12 had unilateral choanal atresia/stenosis, and eight who were referred with suspected choanal atresia had a simple inflammatory nasal obstruction. Patients with bilateral choanal obstruction and patients with unilateral choanal obstruction or rhinitis showed no differences in clinical picture or in referral age. Many infants with either unilateral or bilateral choanal obstruction had one or more symptoms not fully relieved after surgical repair, although the choanae were widely patent. At long-term follow-up the surviving patients showed spontaneous recovery and good nasal respiration. Overall, five patients died. Since the common syndrome appeared to be related to a dysfunction of the autonomic nervous system, we conclude that any type of nasal obstruction may exacerbate or precipitate an underlying maturational autonomic disturbance.

  5. Pulmonary manifestations of inflammatory bowel disease

    PubMed Central

    Majewski, Sebastian

    2015-01-01

    Bronchopulmonary signs and symptoms are examples of variable extraintestinal manifestations of the inflammatory bowel diseases (IBD). These complications of Crohn's disease (CD) and ulcerative colitis (UC) seem to be underrecognized by both pulmonary physicians and gastroenterologists. The objective of the present review was to gather and summarize information on this particular matter, on the basis of available up-to-date literature. Tracheobronchial involvement is the most prevalent respiratory presentation, whereas IBD-related interstitial lung disease is less frequent. Latent and asymptomatic pulmonary involvement is not unusual. Differential diagnosis should always consider infections (mainly tuberculosis) and drug-induced lung pathology. The common link between intestinal disease and lung pathology is unknown, but many hypotheses have been proposed. It is speculated that environmental pollution, common immunological mechanisms and predisposing genetic factors may play a role. PMID:26788078

  6. [Clinical manifestations of epilepsy in hereditary schizophrenia].

    PubMed

    Dvirskiĭ, A E; Shevtsov, A G

    1991-01-01

    A study was made of the effect of hereditary loading with schizophrenia on the clinical characteristics of epilepsy. In the population sample of epileptic patients (n = 3489), hereditary loading with schizophrenia was revealed in 4.7% of the patients, among whom loading with both schizophrenia and epilepsy was recorded in 0.6% of the cases. The clinical manifestations of epilepsy were compared in 143 patients loaded with schizophrenia and in 158 patients without hereditary loading with mental diseases. It has been established that in epileptic patients, the factor of hereditary loading with schizophrenia exerts a modifying effect on the premorbid characteristics, increasing the rate of schizoepileptoid, schizoid, and astheno-neurotic++ personality traits, and contributes to a favorable course of the disease. The data obtained standpoint of the previously advanced ++clinico-genetic concept of combined psychoses.

  7. Neonatal muscular manifestations in mitochondrial disorders.

    PubMed

    Tulinius, Már; Oldfors, Anders

    2011-08-01

    During the last decade rapid development has occurred in defining nuclear gene mutations causing mitochondrial disease. Some of these newly defined gene mutations cause neonatal or early infantile onset of disease, often associated with severe progressive encephalomyopathy combined with other multi-organ involvement such as cardiomyopathy or hepatopathy and with early death. Findings suggesting myopathy in neonates are hypotonia, muscle weakness and wasting, and arthrogryposis. We aim to describe the clinical findings of patients with mitochondrial disease presenting with muscular manifestations in the neonatal period or in early infancy and in whom the genetic defect has been characterized. The majority of patients with neonatal onset of mitochondrial disease have mutations in nuclear genes causing dysfunction of the mitochondrial respiratory chain, leading to defective oxidative phosphorylation.

  8. Cutaneous manifestations in disorders of hepatobiliary system

    PubMed Central

    Godara, Shashi K.; Thappa, Devinder M.; Pottakkatt, Biju; Hamide, Abdoul; Barath, Jagadisan; Munisamy, Malathi; Chiramel, Minu J.

    2017-01-01

    Background: Hepatobiliary diseases are associated with various mucocutaneous changes that are directly and indirectly associated with these diseases. There is a dearth of studies from India dealing with cutaneous manifestations in liver disorders. Aim: This study was conducted to know the spectrum of cutaneous changes and their correlation with liver function tests in patients with disorders of hepatobiliary system. Methods: This was a descriptive study conducted among in patients and outpatients with primary hepatobiliary disorders at a tertiary care center in South India. Detailed history taking and examination was done. Visual analog score (VAS) and 5D itch score was employed to quantify pruritus. Correlation of liver function tests with pruritus and comparison of skin changes in the major disease groups was attempted. Results: A total of 303 patients were recruited. Hepatic mass/malignancy followed by nonalcoholic liver disease were the most common diagnoses. Icterus followed by pruritus were the most common cutaneous complaints. The mean VAS and 5D itch scores among the patients with pruritus were 4.7 ± 1.9 and 13.6 ± 4.9. The most common mucocutaneous findings observed were icterus (241 patients; 79.5%), ichthyosis/xerosis (172 patients; 56.7%), pallor (131 patients; 43.2%), excoriations (118 patients; 38.9%), hyperpigmented palmar creases (118 patients; 38.9%), clubbing (113 patients; 37.2%), and pedal edema (85 patients; 28%). There was statistically significant correlation between serum bilirubin levels and pruritus. Conclusion: The common mucocutaneous manifestations associated with primary hepatobiliary disorders were icterus, ichthyosis/xerosis, pallor, excoriations, hyperpigmented palmar creases, clubbing, and pedal edema. PMID:28217465

  9. Vaccines for viral diseases with dermatologic manifestations.

    PubMed

    Brentjens, Mathijs H; Yeung-Yue, Kimberly A; Lee, Patricia C; Tyring, Stephen K

    2003-04-01

    Vaccines against infectious diseases have been available since the 1800s, when an immunization strategy against smallpox developed by Jenner gained wide acceptance. Until recently, the only vaccination strategies available involved the use of protein-based, whole killed, and attenuated live virus vaccines. These strategies have led to the development of effective vaccines against a variety of diseases with primary or prominent cutaneous manifestations. Effective and safe vaccines now used worldwide include those directed against measles and rubella (now commonly used together with a mumps vaccine as the trivalent MMR), chickenpox, and hepatitis B. The eradication of naturally occurring smallpox remains one of the greatest successes in the history of modern medicine, but stockpiles of live smallpox exist in the United States and Russia. Renewed interest in the smallpox vaccine reflects concerns about a possible bioterrorist threat using this virus. Yellow fever is a hemorrhagic virus endemic to tropical areas of South America and Africa. An effective vaccine for this virus has existed since 1937, and it is used widely in endemic areas of South America, and to a lesser extent in Africa. This vaccine is recommended once every 10 years for people who are traveling to endemic areas. Advances in immunology have led to a greater understanding of immune system function in viral diseases. Progress in genetics and molecular biology has allowed researchers to design vaccines with novel mechanisms of action (eg, DNA, vector, and VLP vaccines). Vaccines have also been designed to specifically target particular viral components, allowing for stimulation of various arms of the immune system as desired. Ongoing research shows promise in prophylactic and therapeutic vaccination for viral infections with cutaneous manifestations. Further studies are necessary before vaccines for HSV, HPV, and HIV become commercially available.

  10. Thoracic manifestations of Behçet disease at CT.

    PubMed

    Hiller, Nurith; Lieberman, Sivan; Chajek-Shaul, Tova; Bar-Ziv, Jacob; Shaham, Dorith

    2004-01-01

    Behçet disease is a multisystemic and chronic inflammatory disorder of unknown cause that is characterized by recurrent oral and genital ulcerations, ocular manifestations, and additional clinical manifestations in multiple organ systems. Behçet disease involving the chest can manifest as a wide spectrum of abnormalities. Although conventional chest radiography is commonly used for initial assessment, spiral computed tomography can demonstrate the entire spectrum of thoracic manifestations of Behçet disease, including abnormalities of the vessel lumen and wall, perivascular tissues, lung parenchyma, pleura, and mediastinal structures. Aneurysms of the pulmonary arteries, with or without thrombosis, are a typical manifestation of Behçet disease. Other manifestations include thrombosis, vasculitis, hemorrhage, infarction, and inflammation. Familiarity with these manifestations can be useful in the diagnosis of Behçet disease, helping to determine the cause of symptoms in patients who present with hemoptysis and guide the choice of appropriate therapy.

  11. Giant-cell arteritis without cranial manifestations

    PubMed Central

    de Boysson, Hubert; Lambert, Marc; Liozon, Eric; Boutemy, Jonathan; Maigné, Gwénola; Ollivier, Yann; Ly, Kim; Manrique, Alain; Bienvenu, Boris; Aouba, Achille

    2016-01-01

    Abstract Diagnosis of giant-cell arteritis (GCA) is challenging in the absence of cardinal cranial symptoms/signs. We aimed to describe the clinical presentation, diagnostic process, and disease course of GCA patients without cranial symptoms, and to compare them to those of patients with typical cranial presentation. In this retrospective multicenter study, we enrolled patients with GCA who satisfied at least 3 of the 5 American College of Rheumatology criteria for GCA, or 2 criteria associated with contributory vascular biopsy other than temporal artery biopsy or with demonstration of large-vessel involvement; underwent iconographic evaluation of large arterial vessels (aortic CT scan or a positron emission tomography with 18F-fluorodeoxyglucose combined with computed tomography (FDG-PET/CT) scan or cardiac echography combined with a large-vessel Doppler) at diagnosis. We divided the cohort into 2 groups, distinguishing between patients without cranial symptoms/signs (i.e., headaches, clinical temporal artery anomaly, jaw claudication, ophthalmologic symptoms) and those with cranial symptoms/signs. In the entire cohort of 143 patients, all of whom underwent vascular biopsy and vascular imaging, we detected 31 (22%) patients with no cranial symptoms/signs. In the latter, diagnosis was biopsy proven in an arterial sample in 23 cases (74% of patients, on a temporal site in 20 cases and on an extratemporal site in 3). One-third of these 31 patients displayed extracranial symptoms/signs whereas the remaining two-thirds presented only with constitutional symptoms and/or inflammatory laboratory test results. Compared to the 112 patients with cardinal cranial clinical symptoms/signs, patients without cranial manifestations displayed lower levels of inflammatory laboratory parameters (C-reactive level: 68 [9–250] mg/L vs 120 [3–120] mg/L; P < 0.01), highest rate of aorta and aortic branch involvement identified (19/31 (61%) vs 42/112 (38%); P = 0.02) and also

  12. [Thoracic manifestations of AIDS (acquired immunodeficiency syndrome)].

    PubMed

    Bernasconi, A; Zompatori, M; Chiodo, F; Costigliola, P; Ricchi, E; Colangeli, V; Canini, R; Gavelli, G

    1989-11-01

    AIDS (acquired immunodeficiency syndrome) seems to be related to human immunodeficiency virus (HIV) and is characterized by severe T-helpers lymphocyte dysfunction. Many of the AIDS patients (47-70%) develop pulmonary manifestations, both infectious and neoplastic, in the course of their disease. In the Department of Infectious Diseases of our Hospital are studied many patients HIV+. Every year 246 seropositive new patients have been discovered. Among them we have studied 25 subjects with respiratory disease, by chest radiographs; successively, according to clinical picture, we have performed thoracic computed tomography, Gallium scintigraphy, fiberoptic bronchoscopy with transbronchial biopsy (TBB), bronchoalveolar lavage (BAL); the majority of these patients (68%) had AIDS, only 28% had ARC and 4% had PGL. In our experience, the diagnosed diseases were mainly infections (92%), and most frequently (52%) due to Pneumocystis carinii, alone or in association with other etiologic agents. We have not found pathognomonic radiographic abnormalities, but chest X-ray evaluated with clinical and laboratory data, may often be useful to obtain diagnostic indications and in order to determine a more specific and aggressive diagnostic approach.

  13. Hypertonicity: Clinical entities, manifestations and treatment

    PubMed Central

    Rondon-Berrios, Helbert; Argyropoulos, Christos; Ing, Todd S; Raj, Dominic S; Malhotra, Deepak; Agaba, Emmanuel I; Rohrscheib, Mark; Khitan, Zeid J; Murata, Glen H; Shapiro, Joseph I; Tzamaloukas, Antonios H

    2017-01-01

    Hypertonicity causes severe clinical manifestations and is associated with mortality and severe short-term and long-term neurological sequelae. The main clinical syndromes of hypertonicity are hypernatremia and hyperglycemia. Hypernatremia results from relative excess of body sodium over body water. Loss of water in excess of intake, gain of sodium salts in excess of losses or a combination of the two are the main mechanisms of hypernatremia. Hypernatremia can be hypervolemic, euvolemic or hypovolemic. The management of hypernatremia addresses both a quantitative replacement of water and, if present, sodium deficit, and correction of the underlying pathophysiologic process that led to hypernatremia. Hypertonicity in hyperglycemia has two components, solute gain secondary to glucose accumulation in the extracellular compartment and water loss through hyperglycemic osmotic diuresis in excess of the losses of sodium and potassium. Differentiating between these two components of hypertonicity has major therapeutic implications because the first component will be reversed simply by normalization of serum glucose concentration while the second component will require hypotonic fluid replacement. An estimate of the magnitude of the relative water deficit secondary to osmotic diuresis is obtained by the corrected sodium concentration, which represents a calculated value of the serum sodium concentration that would result from reduction of the serum glucose concentration to a normal level. PMID:28101446

  14. Observational Manifestation of Chaos in Astrophysical Objects

    NASA Astrophysics Data System (ADS)

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

    2002-12-01

    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

  15. Oral manifestations of hepatitis C virus infection

    PubMed Central

    Carrozzo, Marco; Scally, Kara

    2014-01-01

    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

  16. Radiation nephritis. Clinical manifestations and pathophysiologic mechanisms

    SciTech Connect

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

    1986-05-01

    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.

  17. Oral manifestations of lymphoma: a systematic review

    PubMed Central

    Silva, Taísa Domingues Bernardes; Ferreira, Camila Belo Tavares; Leite, Gustavo Boehmer; de Menezes Pontes, José Roberto; Antunes, Héliton S

    2016-01-01

    Lymphoma is a malignant disease with two forms: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). Non-Hodgkin’s lymphoma is diagnosed in extranodal sites in 40% of cases, and the head and neck region is the second most affected, with an incidence of 11–33%, while HL has a very low incidence in extranodal sites (1–4%). The aim of this study was to identify the oral manifestations of lymphoma through a systematic literature review, which we conducted using the PubMed, Lilacs, Embase, and Cochrane Library databases. We found 1456 articles, from which we selected 73. Among the intraoral findings, the most frequent were ulcerations, pain, swelling, and tooth mobility, while the extraoral findings included facial asymmetry and cervical, submandibular, and submental lymphadenopathy. Among the few studies reporting imaging findings, the most cited lesions included hypodense lesions with diffuse boundaries, bone resorptions, and tooth displacements. The publications reviewed highlight gaps in the areas of early detection, diagnosis, and proper treatment. PMID:27594910

  18. Cutaneous Manifestations of Diabetes Mellitus: A Review.

    PubMed

    Lima, Ana Luiza; Illing, Tanja; Schliemann, Sibylle; Elsner, Peter

    2017-04-03

    Diabetes mellitus is a widespread endocrine disease with severe impact on health systems worldwide. Increased serum glucose causes damage to a wide range of cell types, including endothelial cells, neurons, and renal cells, but also keratinocytes and fibroblasts. Skin disorders can be found in about one third of all people with diabetes and frequently occur before the diagnosis, thus playing an important role in the initial recognition of underlying disease. Noninfectious as well as infectious diseases have been described as dermatologic manifestations of diabetes mellitus. Moreover, diabetic neuropathy and angiopathy may also affect the skin. Pruritus, necrobiosis lipoidica, scleredema adultorum of Buschke, and granuloma annulare are examples of frequent noninfectious skin diseases. Bacterial and fungal skin infections are more frequent in people with diabetes. Diabetic neuropathy and angiopathy are responsible for diabetic foot syndrome and diabetic dermopathy. Furthermore, antidiabetic therapies may provoke dermatologic adverse events. Treatment with insulin may evoke local reactions like lipohypertrophy, lipoatrophy and both instant and delayed type allergy. Erythema multiforme, leukocytoclastic vasculitis, drug eruptions, and photosensitivity have been described as adverse reactions to oral antidiabetics. The identification of lesions may be crucial for the first diagnosis and for proper therapy of diabetes.

  19. Oral mucosal manifestations of autoimmune skin diseases.

    PubMed

    Mustafa, Mayson B; Porter, Stephen R; Smoller, Bruce R; Sitaru, Cassian

    2015-10-01

    A group of autoimmune diseases is characterised by autoantibodies against epithelial adhesion structures and/or tissue-tropic lymphocytes driving inflammatory processes resulting in specific pathology at the mucosal surfaces and the skin. The most frequent site of mucosal involvement in autoimmune diseases is the oral cavity. Broadly, these diseases include conditions affecting the cell-cell adhesion causing intra-epithelial blistering and those where autoantibodies or infiltration lymphocytes cause a loss of cell-matrix adhesion or interface inflammation. Clinically, patients present with blistering, erosions and ulcers that may affect the skin as well as further mucosal surfaces of the eyes, nose and genitalia. While the autoimmune disease may be suspected based on clinical manifestations, demonstration of tissue-bound and circulating autoantibodies, or lymphocytic infiltrates, by various methods including histological examination, direct and indirect immunofluorescence microscopy, immunoblotting and quantitative immunoassay is a prerequisite for definitive diagnosis. Given the frequency of oral involvement and the fact that oral mucosa is the initially affected site in many cases, the informed practitioner should be well acquainted with diagnostic and therapeutic aspects of autoimmune dermatosis with oral involvement. This paper reviews the pathogenesis and clinical presentation of these conditions in the oral cavity with a specific emphasis on their differential diagnosis and current management approaches.

  20. Clinical manifestations and management of Gaucher disease

    PubMed Central

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Summary Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage. PMID:26604942

  1. Oral Manifestation in Mentally Challenged Children

    PubMed Central

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

    2015-01-01

    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. PMID:25859105

  2. Talc: understanding its manifestations in the chest.

    PubMed

    Feigin, D S

    1986-02-01

    Four distinct forms of pulmonary disease caused by talc have been defined. The first form, talcosilicosis, is caused by talc mined with high-silica-content mineral. Findings in this form are identical with those of silicosis. Talcoasbestosis closely resembles asbestosis and is produced by crystalline talc, generally inhaled with asbestos fibers. Pathologic and radiographic abnormalities are virtually identical with those of asbestosis, including calcifications and malignant tumor formation. The third form, talcosis, caused by inhalation of pure talc, may include acute or chronic bronchitis as well as interstitial inflammation; radiographically, it appears as interstitial reticulations or small, irregular nodules, typical of small-airway obstruction. The fourth form, due to intravenous administration of talc, is usually associated with abuse of oral medications and production of vascular granulomas manifested by consolidations, large nodules, and masses. Radiographic abnormalities associated with talc can be predicted when there is sufficient history of the nature of exposure, including the region of origin of the talc in cases of inhalation. Radiographic changes, such as diaphragmatic plaques, often attributed to both talc and asbestos have not been documented to be caused by talc alone. The author provides review of 18 well-documented cases.

  3. Neurological manifestation of methyl bromide intoxication.

    PubMed

    Suwanlaong, Kanokrat; Phanthumchinda, Kammant

    2008-03-01

    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.

  4. Opsoclonus as a manifestation of Hashimoto's encephalopathy.

    PubMed

    Salazar, R; Mehta, C; Zaher, N; Miller, D

    2012-10-01

    We present a 59-year-old male with early manifestation of opsoclonus associated with gait ataxia as a rare clinical presentation of Hashimoto's encephalopathy. Empiric use of intravenous immunoglobulin followed by intravenous high dose methylprednisolone was initiated with subsequent remittance of opsoclonus, encephalopathy, ataxia, and tremor. Extensive workup for infectious, autoimmune, and paraneoplastic etiologies were undertaken and all studies were negative. Thyroglobulin antibodies (312 U/mL) and thyroid peroxidase antibodies (457 U/mL) were elevated (normal <60 U/mL) with a euthyroid state (thyroid stimulating hormone 3.13 μIU/mL). Three months after intravenous steroid therapy, the concentrations of thyroglobulin and thyroid peroxidase antibodies were retested and found to have decreased considerably. Thus, with steroid therapy, the patient's opsoclonus and encephalopathy improved. We have presented a patient with a rare case of opsoclonus as the principal presenting feature of Hashimoto's encephalopathy that was incompletely responsive to intravenous immunoglobulin and resolved with corticosteroids. This report underscores the importance for clinical practitioners to maintain a high index of suspicion for Hashimoto's encephalopathy in cases of opsoclonus, especially when accompanied by an atypical presentation.

  5. Extraintestinal Manifestations of Inflammatory Bowel Disease

    PubMed Central

    Schoepfer, Alain; Scharl, Michael; Lakatos, Peter L.; Navarini, Alexander; Rogler, Gerhard

    2015-01-01

    Abstract: Extraintestinal manifestations (EIM) in inflammatory bowel disease (IBD) are frequent and may occur before or after IBD diagnosis. EIM may impact the quality of life for patients with IBD significantly requiring specific treatment depending on the affected organ(s). They most frequently affect joints, skin, or eyes, but can also less frequently involve other organs such as liver, lungs, or pancreas. Certain EIM, such as peripheral arthritis, oral aphthous ulcers, episcleritis, or erythema nodosum, are frequently associated with active intestinal inflammation and usually improve by treatment of the intestinal activity. Other EIM, such as uveitis or ankylosing spondylitis, usually occur independent of intestinal inflammatory activity. For other not so rare EIM, such as pyoderma gangrenosum and primary sclerosing cholangitis, the association with the activity of the underlying IBD is unclear. Successful therapy of EIM is essential for improving quality of life of patients with IBD. Besides other options, tumor necrosis factor antibody therapy is an important therapy for EIM in patients with IBD. PMID:26154136

  6. Monogenic autoinflammatory diseases: concept and clinical manifestations.

    PubMed

    Almeida de Jesus, Adriana; Goldbach-Mansky, Raphaela

    2013-06-01

    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.

  7. Dermatologic manifestations of polycystic ovary syndrome.

    PubMed

    Lee, Amy T; Zane, Lee T

    2007-01-01

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

  8. Clinical manifestations of parainfluenza infection in children.

    PubMed

    Yang, Tsung-Yen; Lu, Chun-Yi; Kao, Chuan-Liang; Chen, Rong-Tsung; Ho, Yu-Huai; Yang, Shun-Cheng; Lee, Ping-Ing; Chen, Jong-Min; Lee, Chin-Yun; Huang, Li-Min

    2003-12-01

    Parainfluenza viruses are major pathogens causing respiratory illness, manifesting from mild upper respiratory tract infection to bronchiolitis and pneumonia. This retrospective study aimed at providing clinical and epidemiologic data addressing the parainfluenza virus infection in Taiwan. A total of 39 patients were enrolled in this study from March 1999 to December 2000. Infants and young children were the major susceptible population, with 87.2% of them younger than 3 years. No seasonal trend was noted for parainfluenza type 1 and type 2 infections. One clustering of parainfluenza virus type 3 infections occurred in late spring of 2000 based on collected results. Parainfluenza type 1 viral isolates accounted for all of the cases of croup. Most isolates of parainfluenza virus type 3 were associated with upper and/or lower respiratory tract infections. A substantial proportion of the patients had skin involvement; the identification of one case of possible parainfluenza virus-related erythema multiforme is particularly interesting, especially because the chances of a causal relation between viral infection and skin symptoms are formerly thought to be slight. The identification of parainfluenza virus in illnesses classically considered to be due to other viruses is intriguing and may have important implications in the management of childhood illness clinically.

  9. [Anaphylactic manifestations during protected sexual intercourse disclosing allergy to latex].

    PubMed

    Espin, M; Didier, A; Perez, T; Carre, P; Leophonte, P

    1991-01-01

    Reports of allergy to latex have been increasingly frequent during the last few years. The culprit is the natural Hevea Brasiliensis latex which is present in numerous latex-containing materials for daily use. Clinical manifestations are usually benign. Systemic manifestations have been reported mainly in general anaesthesia. We report a case of severe anaphylactic manifestations that occurred during a condom-protected sexual intercourse. The responsibility of latex for this accident was demonstrated by skin and biological tests.

  10. Neuromuscular Manifestations of West Nile Virus Infection

    PubMed Central

    Leis, A. Arturo; Stokic, Dobrivoje S.

    2012-01-01

    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

  11. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations.

    PubMed

    Cervera, R; Tektonidou, M G; Espinosa, G; Cabral, A R; González, E B; Erkan, D; Vadya, S; Adrogué, H E; Solomon, M; Zandman-Goddard, G; Shoenfeld, Y

    2011-02-01

    The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyze the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations, and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analyzed on thrombocytopenia and skin manifestations, and presents the recommendations elaborated by the Task Force after this analysis.

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

    ERIC Educational Resources Information Center

    Muller, Juanita; Waters, Lea

    2012-01-01

    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…

  13. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  14. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  15. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  16. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  17. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  18. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  19. Cardiac tamponade as an initial manifestation of systemic lupus erythematosus.

    PubMed

    Carrion, Diego M; Carrion, Andres F

    2012-06-12

    Clinical manifestations of pericardial disease may precede other signs and symptoms associated with systemic lupus erythematosus. Although pericardial effusion is one of the most common cardiac problems in patients with systemic lupus erythematosus, haemodynamically significant effusions manifesting as cardiac tamponade are rare and require prompt diagnosis and treatment.

  20. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... manifest (or shipping paper for exports by water (bulk shipment)). For exports of hazardous waste subject...) The transporter must ensure that the manifest accompanies the hazardous waste. In the case of exports... signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the hazardous waste; and (3)...

  1. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... manifest (or shipping paper for exports by water (bulk shipment)). For exports of hazardous waste subject...) The transporter must ensure that the manifest accompanies the hazardous waste. In the case of exports... signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the hazardous waste; and (3)...

  2. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... manifest (or shipping paper for exports by water (bulk shipment)). For exports of hazardous waste subject...) The transporter must ensure that the manifest accompanies the hazardous waste. In the case of exports... signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the hazardous waste; and (3)...

  3. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... manifest (or shipping paper for exports by water (bulk shipment)). For exports of hazardous waste subject...) The transporter must ensure that the manifest accompanies the hazardous waste. In the case of exports... signatures) and, for exports, an EPA Acknowledgment of Consent accompanies the hazardous waste; and (3)...

  4. 19 CFR 122.74 - Incomplete (pro forma) manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Incomplete (pro forma) manifest. 122.74 Section 122.74 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT...; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew Members Onboard...

  5. [Dysphagia as the sole manifestation of myasthenia gravis].

    PubMed

    Romo González, Ramiro Javier; Chaves, Emiliano; Copello, Hercilia

    2010-06-01

    Dysphagia as the sole manifestation of myasthenia gravis is very rare. Here we describe a case of an adult patient who developed an insidious onset of oropharyngeal dysphagia as the first and sole manifestation of myasthenia gravis. After multiple evaluations the underlying disease was recognized by electromyographics studies. English and Spanish literature on the matter was reviewed.

  6. Photoletter to the editor: Disseminated histoplasmosis with initial oral manifestations.

    PubMed

    Sinha, Surabhi; Sardana, Kabir; Garg, Vijay K

    2013-03-30

    Histoplasmosis is a systemic fungal disease that may present in a variety of clinical manifestations. Involvment of the oral mucosa is very rare and may occur as part of disseminated histoplasmosis or as isolated involvement. We present a patient with disseminated histoplasmosis, in whom oral lesions were the initial manifestation of the disease.

  7. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 19 Customs Duties 1 2014-04-01 2014-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements...

  8. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 19 Customs Duties 1 2012-04-01 2012-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements...

  9. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 1 2013-04-01 2013-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements...

  10. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 2 2012-10-01 2012-10-01 false Hazardous waste manifest. 172.205 Section 172.205... SECURITY PLANS Shipping Papers § 172.205 Hazardous waste manifest. (a) No person may offer, transport, transfer, or deliver a hazardous waste (waste) unless an EPA Form 8700-22 and 8700-22A (when...

  11. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    PubMed

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-02-16

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  12. Wolf-Hirschhorn syndrome; oro-dental manifestations and management.

    PubMed

    Roberts, T; Stephen, L X G; Fieggen, K; Beighton, P

    2009-01-01

    The major manifestations of the Wolf-Hirschhorn syndrome are developmental delay, short stature, mental impairment and epilepsy. Clefts of the lip and palate are sometimes present. Dental problems which are overshadowed by the major syndromic manifestations warrant appropriate management. We have documented an affected South African boy, discussed his dental management and reviewed the oro-dental implications of the disorder.

  13. 40 CFR 262.25 - Electronic manifest signatures.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 26 2014-07-01 2014-07-01 false Electronic manifest signatures. 262.25 Section 262.25 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.25 Electronic...

  14. 77 FR 54818 - Polychlorinated Biphenyls (PCBs): Revisions to Manifesting Regulations

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-06

    ...The Environmental Protection Agency (``EPA'' or ``the Agency'') is issuing this direct final rule to update and clarify several sections of the Polychlorinated Biphenyl (PCB) regulations associated with the manifesting requirements, which uses the Resource Conservation and Recovery Act (RCRA) Uniform Hazardous Waste Manifest, under the Toxic Substances Control Act (TSCA). Today's changes are......

  15. Clinic manifestations in granulomatosis with polyangiitis.

    PubMed

    Greco, A; Marinelli, C; Fusconi, M; Macri, G F; Gallo, A; De Virgilio, A; Zambetti, G; de Vincentiis, M

    2016-06-01

    Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility.In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8-99 years).Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70-100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85-100%), whereas otological disease is found in approximately 35% (range, 19-61%) of cases.Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60-70% when there is no kidney involvement.The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase.A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial

  16. Dermatologic manifestation of hyperandrogenism: a retrospective chart review.

    PubMed

    Clark, Charlotte M; Rudolph, Jennifer; Gerber, Donald A; Glick, Sharon; Shalita, Alan R; Lowenstein, Eve J

    2014-01-01

    Several studies have described a wide spectrum of hyperandrogenism diseases, many of which are difficult to distinguish from each other. In order to better understand diseases of hyperandrogenism, the authors performed a retrospective study of the cutaneous features and metabolic findings in women with hyperandrogenism. A retrospective chart analysis compiled by three dermatologists in both academic and private settings was performed, including patients presenting with > or = 2 manifestations of hyperandrogenism. Relevant dermatologic and associated manifestations and laboratory and imaging study findings were reviewed. Moderate to severe acne was the most common manifestation. Other common manifestations that patients first presented with include hirsutism, acanthosis nigricans, androgenic alopecia, and skin tags. Oligomenorrhea was the most common systemic presenting sign. Statistical analysis of various clinical markers revealed correlations with hyperandrogenemia. Acanthosis nigricans and hirsutism were found to be useful clinical markers for hyperandrogenism, whereas androgenic alopecia was not. This study provides some insights into the presentation and diverse manifestations seen in hyperandrogenism.

  17. Diagnosis and treatment of HIV-associated manifestations in otolaryngology

    PubMed Central

    lacovou, Emily; Vlastarakos, Petros V.; Papacharalampous, George; Kampessis, George; Nikolopoulos, Thomas P.

    2012-01-01

    Almost 30 years after its first description, HIV still remains a global pandemic. The present paper aims to review the current knowledge on the ear, nose and throat (ENT) manifestations of HIV infection, and present the available diagnostic and treatment options. A literature review was conducted in Medline and other available database sources. Information from related books was also included in the data analysis. It is well acknowledged that up to 80% of HIV-infected patients eventually develop ENT manifestations; among which, oral disease appears to be the most common. Oro-pharyngeal manifestations include candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy leucoplakia, Kaposi's sarcoma, and non-Hodgkin's lymphoma. ENT manifestations in the neck can present as cervical lymphadenopathy or parotid gland enlargement. Respective nasal manifestations include sinusitis (often due to atypical bacteria), and allergic rhinitis. Finally, otological manifestations include otitis (externa, or media), inner ear involvement (sensorineural hearing loss, disequilibrium), and facial nerve palsy (up to 100 times more frequently compared to the general population). Although ENT symptoms are not diagnostic of the disease, they might be suggestive of HIV infection, or related to its progression and the respective treatment failure. ENT doctors should be aware of the ENT manifestations associated with HIV disease, and the respective diagnosis and treatment. A multi-disciplinary approach may be required to provide the appropriate level of care to HIV patients. PMID:24470939

  18. Clinical Manifestations Associated with Neurocysticercosis: A Systematic Review

    PubMed Central

    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

    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

  19. Hepatic manifestations of non-steroidal inflammatory bowel disease therapy

    PubMed Central

    Hirten, Robert; Sultan, Keith; Thomas, Ashby; Bernstein, David E

    2015-01-01

    Inflammatory bowel disease (IBD) is composed of Crohn’s disease and ulcerative colitis and is manifested by both bowel-related and extraintestinal manifestations. Recently the number of therapeutic options available to treat IBD has dramatically increased, with each new medication having its own mechanism of action and side effect profile. A complete understanding of the hepatotoxicity of these medications is important in order to distinguish these complications from the hepatic manifestations of IBD. This review seeks to evaluate the hepatobiliary complications of non-steroid based IBD medications and aide providers in the recognition and management of these side-effects. PMID:26644815

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

    PubMed

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

    2014-05-01

    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.

  1. Dimensions of Manifest Anxiety and Their Relationship to College Achievement

    ERIC Educational Resources Information Center

    Khan, S. B.

    1970-01-01

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

  2. Dust Acoustic Mode Manifestations in Earth's Dusty Ionosphere

    SciTech Connect

    Kopnin, S.I.; Popel, S.I.

    2005-10-31

    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.

  3. Management of Musculoskeletal Manifestations in Inflammatory Bowel Disease

    PubMed Central

    Sheth, Tejas; Pitchumoni, C. S.; Das, Kiron M.

    2015-01-01

    Musculoskeletal manifestations are the most common extraintestinal manifestations in inflammatory bowel diseases. Some appendicular manifestations are independent of gut inflammation and are treated with standard anti-inflammatory strategies. On the other hand, axial involvement is linked to gut inflammatory activity; hence, there is a considerable amount of treatment overlap. Biological therapies have revolutionized management of inflammatory bowel diseases as well as of associated articular manifestations. Newer mechanisms driving gut associated arthropathy have surfaced in the past decade and have enhanced our interests in novel treatment targets. Introduction of biosimilar molecules is expected in the US market in the near future and will provide an opportunity for considerable cost savings on healthcare. A multidisciplinary approach involving a gastroenterologist, rheumatologist, and physical therapist is ideal for these patients. PMID:26170832

  4. [Ventricular Tachycardia as a First Manifestation of Myotonic Dystrophy].

    PubMed

    Mironov, N Yu; Mironova, N A; Sokolov, S F; Mareev, Yu V; Shlevkov, N B; Saidova, M A; Stukalova, O V; Golitsyn, S P

    2015-01-01

    We report a case of bundle-branch reentrant ventricular tachycardia as a first and severe manifestation of myotonic dystrophy. Progressive cardiac conduction disturbances and cardiac arrhythmias are well-known features of myotonic dystrophy, although they are commonly found in late stage of disease in patients with established diagnosis. We review clinical manifestations, diagnostics, management, and prognostic value of cardiac involvement in myotonic dystrophy.

  5. [Intersticial lung disease as the sole manifestation of antisynthetase syndrome].

    PubMed

    Monteiro, Paulo; Coutinho, Margarida; Machado, Pedro; Garcia, Jorge; Salvador, Maria João; Inês, Luís; Silva, Jorge; Malcata, Armando

    2009-01-01

    The authors report a clinical case of a woman who had a 3 years diagnosis of hipersensitivity pneumonitis based on intersticial lung disease without other manifestations. The diagnosis of antisynthetase syndrome was made three years after the initial symptoms upon the onset of systemic manifestations with articular involvement, myositis and determination of anti-PL 7 antibodies. In this syndrome, the isolated pulmonary involvement is rare.

  6. [Partial trisomy of chromosome 15 with new phenotypic manifestations].

    PubMed

    Mar González, J; Llaurado Robles, R A; Cabrera Rivas, T; Lantigua Cruz, A; Rodríguez Verdecia, B

    1994-01-01

    A patient with a 15 partial trisomy and a 4 target chromosome in 100% of metaphases is presented. Phenotypic manifestations not previously described were observed such as macrocephally, long face, low implantation of ears, narrow forehead, epicanthal fold, copious eyebrows and synophrys, short nasolabial distance, convergent strabismus, delayed bucal eruption, long neck, hypertrophy of thenar and hypothenar bulging and articular hypermobility. The eyeground was degeneratively myopic. This case makes more extensive the variety of clinical manifestations of this disease.

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

    PubMed

    Krecek, J

    2010-01-01

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

  8. Rheumatologic Manifestations in Iranian Patients with Autoimmune Thyroid Diseases

    PubMed Central

    Hezarkhani, Sharabeh; Aghaei, Mehrdad; Shamekhi, Maryam; Nomali, Mahin

    2014-01-01

    Background: Autoimmune thyroid diseases (ATDs) are the most common endocrine diseases which result in rheumatologic manifestations. Some studies have shown association between rheumatologic disorders and ATDs. Thus, the aim of this study was to assess the frequency of rheumatologic manifestations in patients with ATDs. Materials and Methods: In this cross-sectional descriptive study during 2010 to 2011, 65 patients with ATDs referred to the Rheumatology clinic of 5 Azar Hospital in Gorgan (North of Iran) were studied via systematic random sampling and patients with positive antithyroid peroxides (anti-TPO) were included in the study. These patients were examined by a rheumatologist for diagnosis of rheumatologic manifestations and tested for serum levels of TSH, Free T3 and T4, Anti-Nuclear Antibodies (ANAs) and Rheumatoid Factor (RF). SPSS software (version 16) and descriptive statistics were used for data analysis. Results: Nine males (14.8%) and 56 females (86.2%) with mean age of 38.81±1.44 years were studied. Overall, Rheumatologic manifestations were seen in 86.2 % (n=56). In this study, the most frequent rheumatologic manifestations were Carpal Tunnel Syndrome (36.1%) and Osteoarthritis (23%). Reynaud’s phenomenon (RP) (10.7%), Discopathy (8.9%), Fibromyalgia (5.3%), Myopathy (3.6%), Rheumatoid arthritis (3.6%) and trigger finger (3.6%) were other manifestations, respectively. Conclusion: In this region, there is a high frequency of rheumatologic manifestations in patients with ATDs. Thus, initial evaluation and regular checkings are recommended. PMID:25478383

  9. [Myositis and the skin: cutaneous manifestations of dermatomyositis].

    PubMed

    Jinnin, Masatoshi

    2013-11-01

    Idiopathic inflammatory myopathies include dermatomyositis, polymyositis, and inclusion body myositis. Among them, cutaneous manifestations are observed most frequently in dermatomyositis. While dermatomyositis commonly affects the skin and muscles, it can also affect the lungs and other organs. Dermatomyositis presenting clinically and histopathologically with typical cutaneous lesions, but without myositis, is called amyopathic dermatomyositis. Given that the Bohan and Peter criteria cannot distinguish amyopathic dermatomyositis, understanding the characteristic skin manifestations may be essential for diagnosing this condition. The cutaneous manifestations of dermatomyositis are thought to be the result of the Koebner phenomenon, vasculopathy, or photosensitivity; manifestations include various eruptions, such as heliotrope rush, Gottron's sign, Gottron's papules, mechanic's hand, nail-fold bleeding, skin ulcer, vasculitis, flagellate erythema, V-sign, and Shawl sign. The presence of multiple types of eruptions can help diagnose the disease. Several skin diseases, including adult Still's disease, contact dermatitis, and sarcoidosis, can mimic the cutaneous manifestations of dermatomyositis. Skin biopsy is useful for differential diagnoses. Histopathologically, dermatomyositis of the skin is characterized by liquefaction degeneration, vacuolar degeneration, edema, and mucin deposition. Dermatologists, neurologists, and rheumatologists are responsible for the diagnosis and management of dermatomyositis, in cooperation with pulmonologists, pediatricians, and pathologists. This review aims to provide clinicians with recent findings regarding skin involvement in dermatomyositis.

  10. Oral manifestation and salivary changes in renal patients undergoing hemodialysis

    PubMed Central

    Honarmand, Marieh; Nakhaee, Alireza; Sargolzaie, Fahimeh

    2017-01-01

    Background Salivary changes in hemodialysis patients may result in various oral manifestations. This research intended to determine oral manifestations and some salivary markers in hemodialysis patients. Material and Methods This cross-sectional study was conducted on 30 hemodialysis patients (the patient group) and 30 healthy individuals (the control group). Saliva urea and calcium levels and pH values of the participants were measured, and oral manifestations such as pale mucosa, xerostomia, halitosis, changes in the sense of taste, increased calculus formation, gingival bleeding, etc. were recorded in the information collection form. The data was analyzed using T-test and chi-square, and p<0.05 was considered to be significant. Results The mean salivary urea level and pH value in the patient group were significantly higher compared to those of the control group (P<0.05), but there were no significant differences between the two groups with respect to salivary calcium. Halitosis, xerostomia, and increased calculus were the most prevalent manifestations, and gum bleeding was the least prevalent among the patients. Conclusions Advanced chronic renal insufficiency can increase salivary urea level, pH value, halitosis, xerostomia, and calculus formation, and may cause pale mucosa. Key words:Renal dialysis, biomarkers, oral manifestation, saliva. PMID:28210437

  11. Testing manifest monotonicity using order-constrained statistical inference.

    PubMed

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

    2013-01-01

    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, such as the restscore, a single item score, and in some cases the total score. In this study, we show that manifest monotonicity can be tested by means of the order-constrained statistical inference framework. We propose a procedure that uses this framework to determine whether manifest monotonicity should be rejected for specific items. This approach provides a likelihood ratio test for which the p-value can be approximated through simulation. A simulation study is presented that evaluates the Type I error rate and power of the test, and the procedure is applied to empirical data.

  12. Case report of Graves' disease manifesting with odynophagia and heartburn.

    PubMed

    Evsyutina, Yulia; Trukhmanov, Alexander; Ivashkin, Vladimir; Storonova, Olga; Godjello, Elina

    2015-12-28

    Graves' disease is an autoimmune disease, which can manifest with a variety of extrathyroidal clinical syndromes like ophthalmopathy, pretibial myxedema (dermopathy), acropathy, cardiomyopathy, and encephalopathy. Though quite rare, this disease can also manifest with gastrointestinal symptoms such as dysphagia, heartburn, nausea, vomiting and diarrhea. We report a clinical case of Graves' disease manifesting with dysfunction of the esophagus and heartburn in a 61-year-old man. In the muscular layer of the esophagus we found dystrophic changes led to its atony, which was documented by endoscopy and high-resolution manometry. The pathology features of esophageal symptoms were: focal proliferation of the basal cells, vascular distension, and dystrophy of the epithelial cells. Antithyroid treatment led to decrease of all clinical symptoms after 5 d of Thiamazole administration. Complete restoration of peristalsis in the esophagus, according to manometry, was observed in 1 mo after initiation of treatment.

  13. Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease*

    PubMed Central

    Komatsu, Yumi Cristina; Capareli, Gabriela Cunha; Boin, Maria Fernanda Feitosa de Camargo; Lellis, Rute; de Freitas, Thaís Helena Proença; Simone, Karine

    2014-01-01

    Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet's syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease's activity. PMID:25387503

  14. Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease.

    PubMed

    Komatsu, Yumi Cristina; Capareli, Gabriela Cunha; Boin, Maria Fernanda Feitosa de Camargo; Lellis, Rute; Freitas, Thaís Helena Proença de; Simone, Karine

    2014-01-01

    Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet's syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease's activity.

  15. Management of the behavioural manifestations of Hunter syndrome.

    PubMed

    Roberts, Jane; Stewart, Catherine; Kearney, Shauna

    This article reviews the behavioural manifestations of, and the strategies for managing, Hunter syndrome (mucopolysaccharidosis (MPS) type II), a rare X-linked lysosomal storage disorder caused by a deficiency of the enzyme iduronate-2-sulphatase. Hunter syndrome is generally considered to have two manifestations: an attenuated form and a severe form; in the latter, the person has pronounced cognitive decline. Infants with either phenotype usually appear normal at birth, but may show some somatic signs. Children with the severe phenotype show developmental delay and changes in behaviour patterns at about 18 months to 4 years of age. To varying degrees, patients with the severe form manifest behavioural disorders such as hyperactivity, aggression, impulsivity, anxiety and sleep disturbances. Medications, such as antipsychotics, benzodiazepines and anticonvulsants, have been tried with varying degrees of success. Behavioural management strategies may be a worthwhile approach, although published data are lacking. For sleep disturbances, behavioural modification plus melatonin or benzodiazepine may be effective treatments.

  16. Extraintestinal manifestations of Helicobacter pylori: A concise review

    PubMed Central

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

    2014-01-01

    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

  17. Epidemiology of extra-articular manifestations in rheumatoid arthritis.

    PubMed

    Turesson, C; Jacobsson, L T H

    2004-01-01

    Extra-articular RA (ExRA) includes a wide variety of disease manifestations. Although rheumatologists in general are aware that such events are clinically important, the heterogeneity of available data, including discrepancies in case definitions, has complicated constructive discussions on this aspect of the RA disease phenotype. In recent years, there has been a growing recognition of the importance of co-morbidity in patients with RA. ExRA manifestations are not uncommon, explain excess mortality in RA and are predicted by smoking and autoantibodies. Further studies of the mechanisms underlying these associations are likely to be important in improving our understanding of the systemic nature of RA. This article discusses the methodological issues involved in the study of ExRA manifestations, presents suggested criteria that have been used in clinical studies, and reviews important surveys of the epidemiology of extra-articular RA.

  18. Neuralgic Amyotrophy Manifesting as Mimicking Posterior Interosseous Nerve Palsy

    PubMed Central

    Yang, Jin Seo; Kang, Suk Hyung; Choi, Eun Hi

    2015-01-01

    The upper trunk of the brachial plexus is the most common area affected by neuralgic amyotrophy (NA), and paresis of the shoulder girdle muscle is the most prevalent manifestation. Posterior interosseous nerve palsy is a rare presentation in patients with NA. It results in dropped finger on the affected side and may be misdiagnosed as entrapment syndrome or compressive neuropathy. We report an unusual case of NA manifested as PIN palsy and suggest that knowledge of clinical NA phenotypes is crucial for early diagnosis of peripheral nerve palsies. PMID:26713154

  19. Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome

    PubMed Central

    Uehara, Takanori; Ikusaka, Masatomi; Ohira, Yoshiyuki; Noda, Kazutaka; Suzuki, Shingo; Shikino, Kiyoshi; Kondo, Takeshi; Kajiwara, Hideki; Ikegami, Akiko; Hirota, Yusuke

    2016-01-01

    Cases of sprue-like enteropathy associated with olmesartan have sporadically been encountered since it was first reported in 2012, and their most characteristic manifestation is severe diarrhea. We herein report the first case of sprue-like enteropathy manifesting as Wernicke-Korsakoff syndrome due to vitamin B1 malabsorption with only minimally increased bowel movements. When patients are receiving olmesartan and they complain of nonspecific chronic gastrointestinal symptoms, it is important to consider changing the drugs before any serious malabsorption syndrome develops. PMID:27980272

  20. Use of Biologic Agents in Ocular Manifestations of Rheumatic Disease

    PubMed Central

    Kraus, Courtney L.; Culican, Susan M.

    2012-01-01

    Biologic agents have dramatically shifted the treatment paradigm for rheumatic disease. Use of these agents can decrease disease burden, allow the patient to be weaned from corticosteroids, and reduce the likelihood of relapse. Eye disease associated with rheumatic conditions may present with a wide range of signs and symptoms. This coexisting pathology should not be overlooked and should be considered a reason for initiation or continuation of biologic therapy. Additionally, many of the ocular manifestations of rheumatic disease respond preferentially to specific targeting molecules. This paper summarizes the available studies on the use, efficacy, and safety of biologic agents in the treatment of ocular manifestations of rheumatic disease. PMID:22229035

  1. Emerging infectious diseases with cutaneous manifestations: Viral and bacterial infections.

    PubMed

    Nawas, Zeena Y; Tong, Yun; Kollipara, Ramya; Peranteau, Andrew J; Woc-Colburn, Laila; Yan, Albert C; Lupi, Omar; Tyring, Stephen K

    2016-07-01

    Given increased international travel, immigration, and climate change, bacterial and viral infections that were once unrecognized or uncommon are being seen more frequently in the Western Hemisphere. A delay in diagnosis and treatment of these diseases can lead to significant patient morbidity and mortality. However, the diagnosis and management of these infections is fraught with a lack of consistency because there is a dearth of dermatology literature on the cutaneous manifestations of these infections. We review the epidemiology, cutaneous manifestations, diagnosis, and management of these emerging bacterial and viral diseases.

  2. Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome.

    PubMed

    Uehara, Takanori; Ikusaka, Masatomi; Ohira, Yoshiyuki; Noda, Kazutaka; Suzuki, Shingo; Shikino, Kiyoshi; Kondo, Takeshi; Kajiwara, Hideki; Ikegami, Akiko; Hirota, Yusuke

    Cases of sprue-like enteropathy associated with olmesartan have sporadically been encountered since it was first reported in 2012, and their most characteristic manifestation is severe diarrhea. We herein report the first case of sprue-like enteropathy manifesting as Wernicke-Korsakoff syndrome due to vitamin B1 malabsorption with only minimally increased bowel movements. When patients are receiving olmesartan and they complain of nonspecific chronic gastrointestinal symptoms, it is important to consider changing the drugs before any serious malabsorption syndrome develops.

  3. Oral Manifestations and Molecular Basis of Oral Genodermatoses: A Review

    PubMed Central

    Shilpasree, A.S.; Chaudhary, Meenakshi

    2016-01-01

    Genodermatoses refers to group of inherited monogenic disorders with skin manifestations. Many of these disorders are rare and also have oral manifestations, called oral genodermatoses. This article provides a focused review of molecular basis of important genodermatoses that affects the oral cavity and also have prominent associated dermatologic features. In several conditions discussed here, the oral findings are distinct and may provide the first clue of an underlying genetic diagnosis. The article also emphasises on the prenatal diagnosis, genetic counselling and the treatment oral genodermatoses. PMID:27437377

  4. Coronary Artery Manifestation of Ormond Disease: The "Mistletoe Sign".

    PubMed

    Maurovich-Horvat, Pál; Suhai, Ferenc Imre; Czimbalmos, Csilla; Tóth, Attila; Becker, David; Kiss, Emese; Ferencik, Maros; Hoffmann, Udo; Vagó, Hajnalka; Merkely, Béla

    2017-02-01

    A 69-year-old woman presented with symptoms of presumed cardiac involvement of idiopathic retroperitoneal fibrosis, otherwise known as Ormond disease. Distinct pericoronary tissue proliferations were depicted at cardiac magnetic resonance (MR) imaging and coronary computed tomographic (CT) angiography. On images, the coronary manifestation was termed the "mistletoe sign." The presence of the mistletoe sign on cardiac MR and coronary CT angiographic images is probably rare, but it might be a characteristic manifestation of retroperitoneal fibrosis. With the increasing number of noninvasive cardiac imaging tests performed worldwide, the recognition of the mistletoe sign could be helpful in diagnosing retroperitoneal fibrosis. (©) RSNA, 2016 Online supplemental material is available for this article.

  5. Space Launch System Co-Manifested Payload Options for Habitation

    NASA Technical Reports Server (NTRS)

    Smitherman, David

    2015-01-01

    The Space Launch System (SLS) has a co-manifested payload capability that will grow over time as the rocket matures and planned upgrades are implemented. The final configuration is planned to be capable of inserting a payload greater than 10 metric tons (mt) into a trans-lunar injection trajectory along with the crew in the Orion capsule and the service module. The co-manifested payload is located below the Orion and its service module in a 10-meter high fairing similar to the way the Saturn launch vehicle carried the lunar lander below the Apollo command and service modules. A variety of approaches have been explored that utilizes this co-manifested payload capability to build up infrastructure in deep space in support of future asteroid, lunar, and Mars mission scenarios. This paper is a report on the findings from the Advanced Concepts Office study team at the NASA Marshall Space Flight Center, working with the Advanced Exploration Systems Program on the Exploration Augmentation Module Project. It includes some of the possible options for habitation in the co-manifested payload volume on SLS. Findings include module designs that can be developed in 10mt increments to support these missions, including overall conceptual layouts, mass properties, and approaches for integration into various scenarios for near-term support of deep space habitat research and technology development, support to asteroid exploration, and long range support for Mars transfer flights.

  6. Space Launch System Co-Manifested Payload Options for Habitation

    NASA Technical Reports Server (NTRS)

    Smitherman, David

    2015-01-01

    The Space Launch System (SLS) has a co-manifested payload capability that will grow over time as the launch vehicle matures and planned upgrades are implemented. The final configuration is planned to be capable of inserting a payload greater than 10 metric tons (mt) into a trans-lunar injection trajectory along with the crew in the Orion capsule and its service module. The co-manifested payload is located below the Orion and its service module in a 10 m high fairing similar to the way the Saturn launch vehicle carried the lunar lander below the Apollo command and service modules. Various approaches that utilize this comanifested payload capability to build up infrastructure in deep space have been explored in support of future asteroid, lunar, and Mars mission scenarios. This paper reports on the findings of the Advanced Concepts Office study team at NASA Marshall Space Flight Center (MSFC) working with the Advanced Exploration Systems Program on the Exploration Augmentation Module Project. It includes some of the possible options for habitation in the co-manifested payload volume of the SLS. Findings include a set of module designs that can be developed in 10 mt increments to support these co-manifested payload missions along with a comparison of this approach to a large-module payload flight configuration for the SLS.

  7. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...), (d) and (e) do not apply and the following requirements do apply: (1) When accepting hazardous waste... 263.20 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO TRANSPORTERS OF HAZARDOUS WASTE Compliance With the Manifest System...

  8. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 2 2011-10-01 2011-10-01 false Hazardous waste manifest. 172.205 Section 172.205 Transportation Other Regulations Relating to Transportation PIPELINE AND HAZARDOUS MATERIALS SAFETY ADMINISTRATION, DEPARTMENT OF TRANSPORTATION HAZARDOUS MATERIALS REGULATIONS HAZARDOUS MATERIALS TABLE,...

  9. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 2 2010-10-01 2010-10-01 false Hazardous waste manifest. 172.205 Section 172.205 Transportation Other Regulations Relating to Transportation PIPELINE AND HAZARDOUS MATERIALS SAFETY ADMINISTRATION, DEPARTMENT OF TRANSPORTATION HAZARDOUS MATERIALS REGULATIONS HAZARDOUS MATERIALS TABLE,...

  10. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 2 2014-10-01 2014-10-01 false Hazardous waste manifest. 172.205 Section 172.205 Transportation Other Regulations Relating to Transportation PIPELINE AND HAZARDOUS MATERIALS SAFETY ADMINISTRATION, DEPARTMENT OF TRANSPORTATION HAZARDOUS MATERIALS REGULATIONS HAZARDOUS MATERIALS TABLE,...

  11. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 2 2013-10-01 2013-10-01 false Hazardous waste manifest. 172.205 Section 172.205 Transportation Other Regulations Relating to Transportation PIPELINE AND HAZARDOUS MATERIALS SAFETY ADMINISTRATION, DEPARTMENT OF TRANSPORTATION HAZARDOUS MATERIALS REGULATIONS HAZARDOUS MATERIALS TABLE,...

  12. Links between Early Attachment Experiences and Manifestations of Spirituality

    ERIC Educational Resources Information Center

    Surr, John

    2011-01-01

    This essay reviews recent research about infant attachment, then discusses the implications of this research as they relate to the following specific manifestations of children's spirituality: faith, wonder, relational consciousness, flow (as in a sense of wholeness or unity), and compassion, in the light of other research on children's…

  13. 76 FR 36363 - Hazardous Waste Manifest Printing Specifications Correction Rule

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-22

    ... wished to print its own manifest forms using laser printers mounted on its transport vehicles. While the... resulted because the laser printers proposed for use in this mobile application could not produce red print... government and the states, or on the distribution of power and responsibilities among the various levels...

  14. 40 CFR 761.214 - Retention of manifest records.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Section 761.214 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.214 Retention of manifest records. (a)(1) A generator... receives a signed copy from the designated facility which received the PCB waste. This signed copy must...

  15. 40 CFR 761.214 - Retention of manifest records.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... Section 761.214 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.214 Retention of manifest records. (a)(1) A generator... receives a signed copy from the designated facility which received the PCB waste. This signed copy must...

  16. 40 CFR 761.211 - Manifest system-Transporter requirements.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.211 Manifest system—Transporter requirements. (a)(1) A transporter shall not accept PCB waste from a generator unless it is accompanied by...

  17. 40 CFR 761.212 - Transporter compliance with the manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.212 Transporter compliance with the manifest. (a) The transporter must deliver the entire quantity of PCB waste which he has...

  18. 40 CFR 761.212 - Transporter compliance with the manifest.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.212 Transporter compliance with the manifest. (a) The transporter must deliver the entire quantity of PCB waste which he has...

  19. 40 CFR 761.211 - Manifest system-Transporter requirements.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.211 Manifest system—Transporter requirements. (a)(1) A transporter shall not accept PCB waste from a generator unless it is accompanied by...

  20. [Lupus vulgaris manifestation as a destructive nose and facial tumor].

    PubMed

    Haller, D; Reisser, C

    2009-04-01

    Lupus vulgaris is the most frequent manifestation of cutaneous tuberculosis, but in Europe it is limited to isolated cases. Mainly immunocompetent individuals are affected by this result of an endogenous reinfection on a lymphogenous-less frequently hematogenous-pathway. Lupus vulgaris has been observed to develop in more than 50% of all patients who already suffer from other manifestations of tuberculosis. The development of a squamous cell carcinoma in the lupus vulgaris is a rare complication; therefore, lupus vulgaris is deemed a facultative precancerosis.A 68-year-old female Serbo-Croatian patient presented with an extensive ulcerative nose and facial tumor. Her anamnesis included a squamous cell carcinoma of the nose that had been excised alio loco 3 years before. Further examinations revealed enlarged cervical lymphoma on both sides, and pulmonary metastases were also suspected. The tumor biopsy revealed a necrotic, granulomatous inflammation. No acid-fast rods were seen on Ziehl-Neelsen stain. The tuberculous origin of this ulcerative skin tumor-the lupus vulgaris-as an endogenous reinfection of pulmonary tuberculosis manifestation was confirmed by the detection of Mycobacterium tuberculosis DNA in polymerase chain reaction and the growth of Mycobacterium tuberculosis colonies in the bacterial culture (skin biopsy and bronchial secretion). The skin tumor as well as the pulmonary manifestation were successfully treated with combined tuberculostatic therapy and showed a dramatic response within 3 months.

  1. 19 CFR 122.74 - Incomplete (pro forma) manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart; Electronic Manifest Requirements for Passengers, Crew Members, and Non-Crew Members Onboard Commercial... Customs officer within one business day after arrival in Puerto Rico, a proper bond shall be filed at...

  2. Biological therapy for dermatological manifestations of inflammatory bowel disease.

    PubMed

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

    2013-05-16

    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)-α 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-α molecules, infliximab (a fusion protein dimer of the human TNF-α receptor) and adalimumab (a fully human monoclonal antibody to TNF-α), for the treatment of the major cutaneous manifestations associated with IBD (EN, PG and psoriasis).

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

    PubMed

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

    2012-01-01

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

  4. 19 CFR 123.25 - Certification and disposition of manifests.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...; DEPARTMENT OF THE TREASURY CUSTOMS RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.25 Certification and disposition of manifests. (a) Certification. Conveyances proceeding... required when the merchandise is to be transshipped in Canada or Mexico under Customs supervision shall...

  5. Palmoplantar hyperkeratosis: a rare cutaneous manifestation of juvenile dermatomyositis.

    PubMed

    Smith, Vanessa M; Leone, Valentina; Clark, Sheila

    2015-01-01

    We report the case of a 12-year-old girl with juvenile dermatomyositis (JDM). She had a 12-month history of palmoplantar hyperkeratosis that was initially treated as eczema. We wish to alert clinicians to this rare cutaneous manifestation of JDM.

  6. Pathological yawning as an ictal seizure manifestation in the elderly.

    PubMed

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

    2012-10-12

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

  7. Self-Organization in the Manifestations of Youth Extremism

    ERIC Educational Resources Information Center

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

    2011-01-01

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

  8. Pulmonary necrobiotic nodules: a rare extraintestinal manifestation of Crohn's disease.

    PubMed

    Warwick, G; Leecy, T; Silverstone, E; Rainer, S; Feller, R; Yates, D H

    2009-03-01

    The present article reports the case of a 22-yr-old female with new onset Crohn's colitis, anterior uveitis and multiple pulmonary nodules which, on histological examination, were necrobiotic nodules. This is a rare but recognised pulmonary extraintestinal manifestation of Crohn's disease and only the fourth reported case. The present case report is followed by a brief review of the relevant literature.

  9. [Acute encephalitis. Neuropsychiatric manifestations as expression of influenza virus infection].

    PubMed

    Moreno-Flagge, Noris; Bayard, Vicente; Quirós, Evelia; Alonso, Tomás

    2009-01-01

    The aim is to review the encephalitis in infants and adolescents as well as its etiology, clinical manifestation, epidemiology, physiopathology, diagnostic methods and treatment, and the neuropsyquiatric signs appearing an influenza epidemy. Encephalitis is an inflammation of the central nervous system (CNS) which involves the brain. The clinical manifestations usually are: headache, fever and confusional stage. It could also be manifested as seizures, personality changes, or psiqyiatric symptoms. The clinical manifestations are related to the virus and the cell type affected in the brain. A meningitis or encephalopathy need to be ruled out. It could be present as an epidemic or isolated form, beeing this the most frequent form. It could be produced by a great variety of infections agents including virus, bacterias, fungal and parasitic. Viral causes are herpesvirus, arbovirus, rabies and enterovirus. Bacterias such as Borrelia burgdorferi, Rickettsia and Mycoplasma neumoniae. Some fungal causes are: Coccidioides immitis and Histoplasma capsulatum. More than 100 agents are related to encephalitis. The diagnosis of encephalitis is a challenge for the clinician and its infectious etiology is clear in only 40 to 70% of all cases. The diagnosis of encephalitis can be established with absolute certainty only by the microscopic examination of brain tissue. Epidemiology is related to age of the patients, geographic area, season, weather or the host immune system. Early intervention can reduce the mortality rate and sequels. We describe four patients with encephalitis and neuropsychiatric symptoms during an influenza epidemic.

  10. [Obstructive uropathy as initial manifestation of Crohn disease].

    PubMed

    Hernández Jaras, J; García-Samaniego Rey, J; Paraiso Cuevas, V; Castaños-Mollor Penalva, R; Pajares García, J M; Traver Aguilar, J A

    1990-10-01

    A patient with renal colicky pain caused by urinary tract obstruction, as a result of psoas abscess, is presented. It was the first manifestation of Crohn's disease. A Gram negative bacteria was isolated from the abscess. The CT images performed to evaluate the abscess suggested this etiology, even though there were no previous symptoms.

  11. Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis

    PubMed Central

    Gopal, Mangala; Patel, Harsh

    2017-01-01

    Central pontine myelinolysis (CPM) is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Common clinical manifestations of CPM include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees; however, coma, “locked-in” syndrome, or death can occur in most severe cases. Rarely, CPM presents with neuropsychiatric manifestations, such as personality changes, acute psychosis, paranoia, hallucinations, or catatonia, typically associated with additional injury to the brain, described as extrapontine myelinolysis (EPM). We present a patient with primarily neuropsychiatric manifestations of CPM, in the absence of focal neurologic deficits or radiographic extrapontine involvement. A 51-year-old female without significant medical history presented with dizziness, frequent falls, diarrhea, generalized weakness, and weight loss. Physical examination showed no focal neurological deficits. Laboratory data showed severe hyponatremia, which was corrected rather rapidly. Subsequently, the patient developed symptoms of an acute psychotic illness. Initial brain magnetic resonance imaging (MRI) was unremarkable, although a repeat MRI two weeks later revealed changes compatible with CPM. This case demonstrates that acute psychosis might represent the main manifestation of CPM, especially in early stages of the disease, which should be taken into consideration when assessing patients with acute abnormalities of sodium metabolism. PMID:28392953

  12. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...) The prevailing practice exception for automated vessels; and (E) The reciprocity exception. (iii) If... under the reciprocity exception, the master or agent shall note on the manifest that the work will be done under the reciprocity exception, and will note the nationality of the vessel's registry and...

  13. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...) The prevailing practice exception for automated vessels; and (E) The reciprocity exception. (iii) If... under the reciprocity exception, the master or agent shall note on the manifest that the work will be done under the reciprocity exception, and will note the nationality of the vessel's registry and...

  14. Testing Manifest Monotonicity Using Order-Constrained Statistical Inference

    ERIC Educational Resources Information Center

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

    2013-01-01

    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,…

  15. Stress Manifestation in High School Students: An Australian Sample.

    ERIC Educational Resources Information Center

    Moulds, John D.

    2003-01-01

    Assesses stress symptoms associated with main daily hassles among grades 7, 9 and 11 students in all-female, all-male, and coeducational Catholic high schools in Western Sydney, Australia. Factor analysis identified the stress manifestation domains of anxiety, anger, and physiological arousal. Differences in these are examined and a conceptual…

  16. An Unusual Cutaneous Manifestation in a Patient with Murine Typhus

    PubMed Central

    Blanton, Lucas S.; Lea, Alfred S.; Kelly, Brent C.; Walker, David H.

    2015-01-01

    Murine typhus is a flea-borne febrile illness caused by Rickettsia typhi. Although often accompanied by rash, an inoculation lesion has not been observed as it is with many tick- and mite-transmitted rickettsioses. We describe a patient with murine typhus and an unusual cutaneous manifestation at the site of rickettsial inoculation. PMID:26416115

  17. 77 FR 54863 - Polychlorinated Biphenyls (PCBs): Revisions to Manifesting Regulations

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-06

    ... ``anonymous access'' system, which means EPA will not know your identity or contact information unless you... Toxic Substances Control Act (TSCA). Today's changes are to match, as much as possible, the manifesting... information. Instructions: Direct your comments to Docket ID No. EPA-HQ-RCRA- 2011-0524. EPA's policy is...

  18. Pulmonary vascular manifestations of mixed connective tissue disease.

    PubMed

    Bull, Todd M; Fagan, Karen A; Badesch, David B

    2005-08-01

    Mixed connective tissue disease (MCTD) refers to a disease process with combined clinical features characteristic of systemic lupus erythematous, scleroderma, and polymyositis-dermatomyositis. This article focuses on the pulmonary vasculature manifestations of MCTD. We briefly discuss associations between MCTD and interstitial lung disease, pleural disease, and alveolar hemorrhage.

  19. Lower extremity manifestations of nutritional deficiencies in gastrointestinal disease.

    PubMed

    Schiraldi-Deck, F G; Grovit, M; Desai, S N

    1998-07-01

    Voluminous information can be written on the nutritional deficiencies that are secondary to gastrointestinal disease. This highly complicated system, with its immunologic pathogenesis, can affect every system of the body. This article describes how these manifestations of gastrointestinal disease affect the lower extremities.

  20. Amount of Manifested Test Anxiety in the Heterogeneous Classroom.

    ERIC Educational Resources Information Center

    Gjesme, Torgrim

    1982-01-01

    The influence of achievement motives, future time orientation (FTO), level of ability (IQ), sex of subjects, and perceived intrinsic instrumentality of school activity were considered in relation to manifested test anxiety at school. Subjects were 507 sixth graders. Pupils' achievement motives and their future time orientation were found to be the…

  1. Social Support in Elderly Nursing Home Populations: Manifestations and Influences

    ERIC Educational Resources Information Center

    Rash, Elizabeth M.

    2007-01-01

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

  2. Massive edema of the ovary associated with androgenic manifestations.

    PubMed

    Siller, B S; Gelder, M S; Alvarez, R D; Partridge, E E

    1995-11-01

    Massive ovarian edema is a rare tumor-like condition of the ovary characterized by marked enlargement of one or both ovaries due to marked accumulation of edema fluid in the ovarian stroma. This paper reviews the literature on massive ovarian edema and presents a case associated with androgenic manifestations.

  3. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 26 2014-07-01 2014-07-01 false Special manifest requirements. 262.54 Section 262.54 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54...

  4. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 40 Protection of Environment 26 2011-07-01 2011-07-01 false Special manifest requirements. 262.54 Section 262.54 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54...

  5. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 25 2010-07-01 2010-07-01 false Special manifest requirements. 262.54 Section 262.54 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54...

  6. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 40 Protection of Environment 27 2012-07-01 2012-07-01 false Special manifest requirements. 262.54 Section 262.54 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54...

  7. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 40 Protection of Environment 27 2013-07-01 2013-07-01 false Special manifest requirements. 262.54 Section 262.54 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54...

  8. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 8 Aliens and Nationality 1 2013-01-01 2013-01-01 false Arrival manifests and lists. 251.1 Section 251.1 Aliens and Nationality DEPARTMENT OF HOMELAND SECURITY IMMIGRATION REGULATIONS ARRIVAL AND... or agent of the vessel shall indicate in writing immediately below the name of the last alien...

  9. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 8 Aliens and Nationality 1 2014-01-01 2014-01-01 false Arrival manifests and lists. 251.1 Section 251.1 Aliens and Nationality DEPARTMENT OF HOMELAND SECURITY IMMIGRATION REGULATIONS ARRIVAL AND... or agent of the vessel shall indicate in writing immediately below the name of the last alien...

  10. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 8 Aliens and Nationality 1 2012-01-01 2012-01-01 false Arrival manifests and lists. 251.1 Section 251.1 Aliens and Nationality DEPARTMENT OF HOMELAND SECURITY IMMIGRATION REGULATIONS ARRIVAL AND... or agent of the vessel shall indicate in writing immediately below the name of the last alien...

  11. Skin manifestations and immunological parameters in childhood food allergy.

    PubMed

    Oehling, A; Fernández, M; Córdoba, H; Sanz, M L

    1997-01-01

    According to Hansen's contact rule, the digestive system should be considered as the main shock organ, yet in food allergy, this is not the case. Very often specific food triggers clinical manifestations not involving the digestive system; that is, reactions are manifested either in the respiratory system, as asthma or rhinitis, or in the skin. In these cases the BALT (broncho-alveolar lymphoid tissue) and GALT (gastrointestinal lymphoid tissue) units play a basic role in the sensitizations. The purpose of this study was to determine the most frequent skin manifestations of food allergy among children, and the most frequently involved foods. We also thought it interesting to evaluate the diagnostic reliability of the different standard immunological parameters utilized by the study team in food allergy. All patients underwent intracutaneous tests with 12 groups of the most frequent food allergens, as well as serum IgE, antigen-specific IgE against foods, and antigen-specific histamine release tests. Antigen-specific IgG4 determination was performed in some cases. The results obtained confirmed previous studies, the most common manifestations being: angioedema (48%), followed by urticaria (31%) and atopic dermatitis (21%). Regarding the frequency of sensitization to different food allergens, in mono- or polisensitization, fish and egg stand out in our environment. Certain food allergens are more frequently responsible for specific skin manifestations. Thus, for fish sensitization, the most frequent skin manifestation is atopic dermatitis (50%); for egg sensitization, angioedema is the most frequent skin manifestation (50%); and for milk, urticaria (50%). Finally, and in agreement with previous works regarding the diagnostic reliability of in vitro techniques, we found that the histamine release test offered the highest percentage of diagnostic reliability. Only for sensitization to milk proteins did antigen-specific IgE demonstrate higher reliability. Once again, we

  12. Adult-onset Still's disease with atypical cutaneous manifestations

    PubMed Central

    Narváez Garcia, Francisco Javier; Pascual, María; López de Recalde, Mercè; Juarez, Pablo; Morales-Ivorra, Isabel; Notario, Jaime; Jucglà, Anna; Nolla, Joan M.

    2017-01-01

    Abstract The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis. The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome. The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy

  13. Management and evaluation of extra-articular manifestations in spondyloarthritis

    PubMed Central

    Nurmohamed, Michael T.

    2012-01-01

    Spondyloarthritis (SpA) is a chronic inflammatory disease with either predominantly axial symptoms of the spine and sacroiliac joints (axial SpA, including ankylosing spondylitis) or predominantly arthritis (peripheral SpA). Next to these spinal and articular symptoms, many patients with SpA also have extra-articular manifestations (EAMs). EAMs associated with SpA include anterior uveitis (25–30%), psoriasis (10–25%) or inflammatory bowel disease (IBD) (5–10%) and cardiovascular manifestations. Peripheral arthritis occurs in approximately 30% of patients, especially in large joints, and shows an asymmetrical, oligoarticular pattern. Other common joint complaints are due to enthesitis, which manifest as extra-articular bony tenderness in areas such as the Achilles tendon. Acute anterior uveitis presents with acute pain, loss of vision and redness in one eye that usually subsides spontaneously after several weeks. Rapid treatment by an ophthalmologist is required to prevent synechiae formation which could ultimately result in glaucoma and blindness. Although less common, organ involvement in SpA can also be located in the heart, lungs or kidneys. The risk of cardiovascular events is increased in SpA. Cardiac manifestations can involve the aortic valve (1–10%) or the atrioventricular node and the risk of atherosclerotic events is increased in this group. Treatment of SpA includes physical exercise and nonsteroidal anti-inflammatory drugs (NSAIDs), and in case of peripheral arthritis, sulphasalazine can be added. When there is insufficient response to NSAIDs, tumor necrosis factor blockers, especially infliximab, etanercept, adalimumab and golimumab, are very effective in treating axial manifestations, arthritis, enthesitis and psoriasis. Anterior uveitis in SpA can be treated adequately by the ophthalmologist and in the case of refractory uveitis, treatment with adalimumab and infliximab seems to be more effective compared with etanercept. When IBD occurs with

  14. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 19 Customs Duties 1 2014-04-01 2014-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  15. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  16. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 19 Customs Duties 1 2012-04-01 2012-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  17. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 1 2013-04-01 2013-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  18. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  19. Extrahepatic Manifestations of Hepatitis C Infection: Navigating CHASM

    PubMed Central

    Sherman, Amy C.; Sherman, Kenneth E.

    2015-01-01

    Purpose of review This article describes the importance of extra-hepatic systemic manifestations of chronic hepatitis C virus (HCV) infection. Recent findings While most HCV literature focuses on liver injury and fibrosis progression, a spectrum of systemic disease processes, collectively called CHASM (C Hepatitis Associated Systemic Manifestations) are present in a high proportion of infected persons. These include thyroid disease (Hashimoto’s thyroiditis, Graves disease, thyroid cancer), cardiovascular disease (atherosclerosis, carotid artery disease, coronary artery disease), renal disease (MPGN, glomerulosclerosis), eye disease (Mooren’s ulcers, sicca syndrome), skin disease (PCT, vasculitis, lichen planus), and lymphomas (NHL, splenic T-cell), and diabetes. Summary Mechanistic understanding of how HCV leads to CHASM processes could lead to development of new interventions. The role of early HCV treatment and cure may result in preventive strategies for a variety of complex disease states. PMID:26208812

  20. Advancements in Pharmacotherapy for Noncancerous Manifestations of HPV

    PubMed Central

    Kollipara, Ramya; Ekhlassi, Erfon; Downing, Christopher; Guidry, Jacqueline; Lee, Michael; Tyring, Stephen K.

    2015-01-01

    Human papillomavirus (HPV) is the most common sexually transmitted disease. Via infection of the basal epithelial cells, HPV causes numerous malignancies and noncancerous cutaneous manifestations. Noncancerous cutaneous manifestations of HPV, including common, plantar, plane, and anogenital warts, are among the most common reasons for an office visit. Although there are various therapies available, they are notoriously difficult to treat. HPV treatments can be grouped into destructive (cantharidin, salicylic acid), virucidal (cidofovir, interferon-α), antimitotic (bleomycin, podophyllotoxin, 5-fluorouracil), immunotherapy (Candida antigen, contact allergen immunotherapy, imiquimod) or miscellaneous (trichloroacetic acid, polyphenon E). The mechanism of action, recent efficacy data, safety profile and recommended regimen for each of these treatment modalities is discussed. PMID:26239450

  1. Cardiac tamponade presenting as early manifestation in dengue fever.

    PubMed

    Bendwal, Suresh; Malviya, Kavita; Jatav, O P; Malviya, Kapil

    2014-03-01

    Dengue fever (DF) is an outbreak prone viral disease transmitted by aedes mosquitoes. It is often associated with evidence of plasma leakage due to increased vascular permeability manifested by pleural effusion, ascites, hypoproteinaemia and pericardial effusion. Cases of small pericardial effusion have been reported in association with dengue fever, largely with dengue haemorrhagic fever (DHF) during epidemic outbreaks. Dengue may rarely present with cardiac tamponade as early manifestation and urgent pericardiocentesis is life saving. A 34 year old male presented with low grade fever, headache, myalgia and breathlessness. Echocardiography revealed large pericardial effusion with right ventricular diagnostic collapse requiring urgent drainage. Subsequently patient improved. Dengue serology (both IgM and IgG) was reported as markedly elevated supporting a diagnosis of classic dengue fever.

  2. Acute pancreatitis: Manifestation of acute HIV infection in an adolescent

    PubMed Central

    Bitar, Anas; Altaf, Muhammad; Sferra, Thomas J.

    2012-01-01

    Summary Background: Pancreatitis in the pediatric age group is not as common as in adults. Etiologies are various and differ from those in adults. Although infectious etiology accounts for a significant number of cases of pancreatitis, acute infection with Human Immunodeficiency Virus (HIV) was rarely reported as a possible etiology for acute pancreatitis in adults. Acute pancreatitis has never been reported as a presenting manifestation of acute HIV infection in children. Case Report: We describe a pediatric patient who presented with acute pancreatitis that revealed acute HIV infection. Conclusions: Acute pancreatitis as a primary manifestation of HIV infection is very rare. It may represent an uncommon aspect of primary HIV infection. We suggest that acute HIV infection should be considered in the differential diagnosis of acute pancreatitis at all ages. PMID:23569476

  3. Extraintestinal manifestations and complications in inflammatory bowel diseases

    PubMed Central

    Rothfuss, Katja S; Stange, Eduard F; Herrlinger, Klaus R

    2006-01-01

    Crohn’s disease (CD) and ulcerative colitis (UC) are chronic inflammatory bowel diseases (IBD) that often involve organs other than those of the gastrointestinal tract. These nonintestinal affections are termed extraintestinal symptoms. Differentiating the true extraintestinal manifestations of inflammatory bowel diseases from secondary extraintestinal complications, caused by malnutrition, chronic inflammation or side effects of therapy, may be difficult. This review concentrates on frequency, clinical presentation and therapeutic implications of extraintestinal symptoms in inflammatory bowel diseases. If possible, extraintestinal manifestations are differentiated from extraintestinal complications. Special attention is given to the more recently described sites of involvement; i.e. thromboembolic events, osteoporosis, pulmonary involvement and affection of the central nervous system. PMID:16937463

  4. [Clinical manifestations of hematological non-neoplastic diseases in Dentistry].

    PubMed

    Bascones-Martínez, Antonio; Muñoz-Corcuera, Marta; Bascones-Ilundain, Cristina

    2012-06-02

    Systemic disease can cause clinical manifestations in the oral and maxillofacial area, which is important to recognize because it could be the first symptom of an undiagnosed illness. There are different oral signs that could suggest the clinician a blood disorder, such as pallor, petechiae, ecchymosis, ulcerations, gingival hypertrophy or spontaneous gingival bleeding. In addition, blood disorders will determine the dental management of these patients and the protocol for limiting possible complications that may arise due to the treatment itself. This paper reviews the oral manifestations and dental management of non-neoplastic alterations of red cells, white cells and hemostasis, with emphasis on two-way relationship that must exist between the dentist and the patient's hematologist for making a treatment plan.

  5. Echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus.

    PubMed

    Zapor, M; Murphy, F T; Enzenauer, R

    2001-01-01

    "That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. Echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. Echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.

  6. Neonatal lupus with atypical cardiac and cutaneous manifestation

    PubMed Central

    Morais, Sofia; Santos, Isabel Cristina; Pereira, Dolores Faria; Mimoso, Gabriela

    2013-01-01

    Neonatal lupus erythematosus is a rare, passively acquired autoimmune disease, caused by maternal autoantibodies. The most common manifestations are skin rash and congenital heart block. Cutaneous manifestations may be present at birth, but often develop within a few weeks after delivery. Congenital heart block may present as bradycardia in utero or during physical examination at birth. Approximately 40–60% of mothers are asymptomatic when the infants are diagnosed. We present a case of a child, born with erythematosus lesions in the face, scalp, trunk, limbs and nodules/papules on the palmar and plantar surfaces. He also had hepatosplenomegaly and thrombocytopenia. Echocardiography showed hyperechoic lesions on the anterior papilar muscle of the left ventricle and on the lateral cusp of the tricuspid valve. The mother had unexplained fever and vasculitic lesions in her hands and feet. Antinuclear antibodies, anti-SSa/Ro and anti-SSb/La were positive in the mother and child, making the diagnosis of neonatal lupus. PMID:23839605

  7. Epididymo-orchitis: an unusual manifestation of salmonellosis.

    PubMed

    Bansal, Neha; Kaistha, Neelam; Chander, Jagdish

    2012-08-01

    Salmonellosis continues to be a major public health problem, especially in developing countries. The formation of focal abscesses may occur following either hematogenous or lymphatic spread. There are large number of serious and life-threatening clinical manifestations of Salmonella spp., ranging from osteomyelitis to infective endocarditis and meningitis. However, even though Salmonella epidydimo-orchitis is a relatively rare clinical manifestion, it can present, most often in male babies and adolescent boys, following contact with nontyphoidal Salmonella. Here, we report a case of epididymo-orchitis due to Salmonella Paratyphi A that presented in an otherwise healthy 63-year-old man in order to highlight this organism's unusual clinical presentation. In countries such as India, where Salmonella infections are endemic, a high index of suspicion should be always be maintained and the possibility of a Salmonella infection at an aberrant site where it is hardly expected should not be ruled out.

  8. Endocrine and metabolic manifestations in inflammatory bowel disease.

    PubMed

    Tigas, Stelios; Tsatsoulis, Agathocles

    2012-01-01

    Extraintestinal manifestations from nearly every organ system are common in inflammatory bowel disease (IBD). This review article describes the epidemiology, pathogenesis, diagnosis and management of the main endocrine and metabolic manifestations in IBD, including metabolic bone disease, growth retardation, hypogonadism, pubertal delay, lipid abnormalities and insulin resistance. These clinical problems are commonly interrelated and they share a common basis, influenced by disease-related inflammation and nutritional status. In addition to nutritional support, every effort should be made to achieve and maintain disease remission, thus correcting the underlying chronic inflammation. The criteria for screening and diagnosing osteoporosis are described and treatment options are discussed (lifestyle advice, vitamin D and calcium supplementation, use of bisphosphonates or other specific antiosteoporotic agents, correction of hypogonadism). Chronic glucocorticoid therapy may affect growth as well as predispose to osteoporosis. The diagnosis and management of growth failure, pubertal delay and hypogonadism in IBD are discussed.

  9. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    PubMed

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases.

  10. Ocular manifestations in the Hutchinson-Gilford progeria syndrome.

    PubMed

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

    2011-01-01

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

  11. Staphylococcus aureus Infections: Epidemiology, Pathophysiology, Clinical Manifestations, and Management

    PubMed Central

    Davis, Joshua S.; Eichenberger, Emily; Holland, Thomas L.

    2015-01-01

    SUMMARY Staphylococcus aureus is a major human pathogen that causes a wide range of clinical infections. It is a leading cause of bacteremia and infective endocarditis as well as osteoarticular, skin and soft tissue, pleuropulmonary, and device-related infections. This review comprehensively covers the epidemiology, pathophysiology, clinical manifestations, and management of each of these clinical entities. The past 2 decades have witnessed two clear shifts in the epidemiology of S. aureus infections: first, a growing number of health care-associated infections, particularly seen in infective endocarditis and prosthetic device infections, and second, an epidemic of community-associated skin and soft tissue infections driven by strains with certain virulence factors and resistance to β-lactam antibiotics. In reviewing the literature to support management strategies for these clinical manifestations, we also highlight the paucity of high-quality evidence for many key clinical questions. PMID:26016486

  12. Current Perspectives on Ophthalmic Manifestations of Childhood Rheumatic Diseases

    PubMed Central

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

    2013-01-01

    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

  13. Toxocara infection and its association with allergic manifestations.

    PubMed

    Pinelli, Elena; Aranzamendi, Carmen

    2012-03-01

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

  14. Neurological counterparts of hyponatremia: pathological mechanisms and clinical manifestations.

    PubMed

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

    2015-04-01

    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.

  15. The MANIFEST fibre positioning system for the Giant Magellan Telescope

    NASA Astrophysics Data System (ADS)

    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

    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.

  16. Hyperkalemia Induced Brugada Phenocopy: A Rare ECG Manifestation

    PubMed Central

    Akbar, Ghulam; Mirrani, Ghazi

    2017-01-01

    Brugada syndrome (BrS) is an inherited disorder of cardiac ion channels characterized by peculiar ECG findings predisposing individuals to ventricular arrhythmias, syncope, and sudden cardiac death (SCD). Various electrolyte disturbances and ion channels blocking drugs could also provoke BrS ECG findings without genetic BrS. Clinical differentiation and recognition are essential for guiding the legitimate action. Hyperkalemia is well known to cause a wide variety of ECG manifestations. Severe hyperkalemia can even cause life threatening ventricular arrhythmias and cardiac conduction abnormalities. Most common ECG findings include peaked tall T waves with short PR interval and wide QRS complex. Since it is very commonly encountered disorder, physicians need to be aware of even its rare ECG manifestations, which include ST segment elevation and Brugada pattern ECG (BrP). We are adding a case to the limited literature about hyperkalemia induced reversible Brugada pattern ECG changes. PMID:28326201

  17. Panniculitis: an unusual presenting manifestation of rheumatoid arthritis.

    PubMed

    Fragoulis, George E; Stamopoulos, Paraskevas; Barbatis, Calypso; Tzioufas, Athanasios G

    2016-01-01

    Rheumatoid arthritis (RA) is a systemic inflammatory disease affecting primarily the joints but also other organs including skin. Panniculitis is an extremely rare manifestation of the disease manifesting mainly as reddish, ulcerative painful nodules and papules, usually in the legs. Histopathologically, it is characterised by liponecrobiosis, granulocytic and histiocytic infiltrates and vasculopathy. Herein, we describe a middle-aged woman with past medical history of hypertension and diabetes mellitus, and unremarkable family history, who presented with symmetrical polyarthritis, low grade fever and painful subcutaneous nodules in the abdomen. Her laboratory tests showed high acute phase reactants, positive rheumatoid factor and anti-Ro autoantibodies and negative anti-CCP. Surgical resection and histological examination of the nodules revealed neutrophilic lobular panniculitis associated with RA. She was treated with low doses of glucocorticosteroids and methotrexate. The latter was substituted with leflunomide due to toxicity. The patient had significant clinical and laboratory improvement.

  18. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection.

    PubMed

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C

    2015-05-06

    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started.

  19. Oral manifestation in inflammatory bowel disease: A review

    PubMed Central

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

    2013-01-01

    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

  20. Oral Manifestations and Dental Management of Epidermolysis Bullosa Simplex

    PubMed Central

    Scheidt, Lisa; Sanabe, Mariane Emi

    2015-01-01

    ABSTRACT Epidermolysis bullosa (EB) is a group of hereditary chronic disorders, characterized by fragility of the skin and mucous membranes in response to minor mechanical trauma. The objective of this study was to report the case of a young girl diagnosed with epidermolysis bullosa simplex (EBS), transmitted by an autosomal dominant gene. Cutaneous findings included blisters and dystrophy following minimal friction. Recurrent blisters and vesicle formation on the hard palate were the main oral findings. In conclusion, publications concerning the oral and clinical manifestations of EBS are important for providing knowledge and an early multidisciplinary approach that prevents blister formation and improves these patients’ quality of life, with the dentist playing an important role in oral health management. How to cite this article: Scheidt L, Sanabe ME, Diniz MB. Oral Manifestations and Dental Management of Epidermolysis Bullosa Simplex. Int J Clin Pediatr Dent 2015;8(3):239-241. PMID:26604545

  1. A case of Sheehan's syndrome that manifested as bilateral ptosis.

    PubMed

    Lee, Young Sil; Moon, Seong Su

    2011-04-01

    Hypothyroidism can cause a variety of signs and symptoms of the neuromuscular system. However, ptosis in a patient with hypothyroidism is very rare. We report here on a case of central hypothyroidism that was due to Sheehan's syndrome and it manifested as bilateral ptosis in a 51-yr-old woman. She complained of exertional dyspnea and weakness. About 25-yr ago, she had a history of severe postpartum vaginal bleeding. The laboratory studies demonstrated hypopituitarism with secondary hypothyroidism. The ptosis was improved by replacement of thyroid hormone. Hypothyroidism should be considered in the differential diagnosis of patients who manifest with ptosis and that prompt replacement of hormone can lead to a complete recovery.

  2. Electrocardiographic findings and cardiac manifestations in acute aluminum phosphide poisoning.

    PubMed

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

    2012-07-01

    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.

  3. Oral manifestations of psoriasis. Clinical presentation and management.

    PubMed

    Dreyer, Lois N; Brown, Gwen Cohen

    2012-04-01

    Psoriasis is a chronic immune-mediated disease of unknown etiology that affects the skin and mucous membranes. According to the National Institutes of Health (NIH), approximately five million Americans, 3% of the population, have been diagnosed with psoriasis. Oral manifestations of psoriasis are less well recognized than skin lesions, and treatment for oral lesions is not standardized. This article will review the clinical presentation of skin and mucous membrane psoriasis, along with the therapeutic modalities available to oral health-care providers.

  4. Osteoskeletal manifestations of scurvy: MRI and ultrasound findings.

    PubMed

    Polat, Ahmet Veysel; Bekci, Tumay; Say, Ferhat; Bolukbas, Emrah; Selcuk, Mustafa Bekir

    2015-08-01

    Scurvy has become very rare in the modern world. The incidence of scurvy in the pediatric population is extremely low. In the pediatric population, musculoskeletal manifestations are more common and multiple subperiosteal hematomas are an important indicator for the diagnosis of scurvy. Although magnetic resonance imaging findings of scurvy are well described in the literature, to our knowledge, ultrasound findings have not yet been described. In this article, we report a case of scurvy with associated magnetic resonance imaging and ultrasound findings.

  5. Brachial plexopathy as a rare presenting manifestation of scorpion envenomation.

    PubMed

    Rubin, Devon I; Vavra, Michael

    2011-07-01

    We report a patient who experienced a rare manifestation of an acute, severe brachial plexopathy as the initial complication of scorpion (presumed Hemiscorpius lepturus species) envenomation. Features suggesting conduction block, due to either proximal demyelination or ion channel dysfunction, along with axonal loss were seen on serial electrophysiological studies. Possible mechanisms of the brachial plexopathy include direct compression from tissue edema or a toxic effect on the membrane channels along the nerve.

  6. Interstitial granulomatous dermatitis: rare cutaneous manifestation of rheumatoid arthritis*

    PubMed Central

    Veronez, Isis Suga; Dantas, Fernando Luiz; Valente, Neusa Yuriko; Kakizaki, Priscila; Yasuda, Thaís Helena; Cunha, Thaís do Amaral

    2015-01-01

    Besides being an uncommon clinicopathological entity, interstitial granulomatous dermatitis, also described as interstitial granulomatous dermatitis with arthritis (IGDA), has shown a wide spectrum of clinical manifestations, such as linear and erythematous lesions, papules, plaques and nodules. Histological features include dense dermal histiocytic infiltrate, usually in a palisade configuration, and scattered neutrophils and eosinophils. We describe a middle aged woman with rheumatoid arthritis of difficult management and cutaneous lesions compatible with IGDA. PMID:26131871

  7. Biological therapy and neurological manifestations. What do we know?

    PubMed

    Tejera-Segura, Beatriz; Ferraz-Amaro, Iván

    2016-06-28

    Biological therapy has changed the course of inflammatory rheumatic diseases. The safety is well documented in national and international studies. Neurological manifestations are uncommon and it is difficult to establish a clear causal relationship. The neurological signs and symptoms that may appear are multiple and sometimes mimic demyelinating neurological diseases and/or neurodegenerative diseases. Knowledge and disclosure of these cases is essential for a comprehensive management of biological therapy in our patients.

  8. Clinical manifestations and outcomes of pulmonary aspergillosis: experience from Pakistan

    PubMed Central

    Iqbal, Nousheen; Irfan, Muhammad; Zubairi, Ali Bin Sarwar; Jabeen, Kauser; Awan, Safia; Khan, Javaid A

    2016-01-01

    Introduction Pulmonary aspergillosis has variable course of illness, severity and outcomes depending on underlying conditions. There is limited data available on the clinical manifestations and outcome of pulmonary aspergillosis from Pakistan. Methods To determine the clinical manifestations and outcome of pulmonary aspergillosis in a tertiary care hospital a retrospective study was conducted from 2004 to 2014 in patients admitted with pulmonary aspergillosis at the Aga Khan University Hospital Karachi, Pakistan. Results Of the 280 cases with provisional diagnosis of aspergillosis 69 met the inclusion criteria. The mean age was 45±15.7 years, 48 (69.6%) were men and 21 (30.4%) had diabetes mellitus (DM). The average length of hospital stay (LOS) was 10.61±9.08 days. Aspergillus fumigatus was the most common (42.0%), followed by Aspergillus flavus (28.9%). More than one-third of patients previously had tuberculosis (TB) (39.13%). The commonest pulmonary manifestation was chronic pulmonary aspergillosis (CPA) 47 (68.1%) followed by invasive pulmonary aspergillosis (IPA) 12 (17.4%) and subacute invasive aspergillosis (SAIA) 8 (11.6%). Surgical excision was performed in 28 patients (40.57%). Intensive care unit admission was required for 18 patients (26.08%). Case fatality rate was 14/69 (20.3%). DM, mean LOS and hypoxic respiratory failure were identified as independent risk factors of mortality on multivariate analysis. Conclusion A. fumigatus was the most frequent species found especially in patients with prior TB. CPA was the commonest pulmonary manifestation seen as post TB sequel. Diabetes, hypoxic respiratory failure and increased LOS were independent predictors of poor outcomes. Overall patients had good outcome with CPA compared with SAIA and IPA. PMID:28074136

  9. Chronic disorders with episodic manifestations: focus on epilepsy and migraine

    PubMed Central

    2006-01-01

    Epilepsy and migraine are chronic neurological disorders with episodic manifestations that are commonly treated in neurological practice and frequently occur together. In this review we examine similarities and contrasts between these disorders, with focus on epidemiology and classification, temporal coincidence, triggers, and mechanistically based therapeutic overlap. This investigation draws attention to unique aspects of both epilepsy and migraine, while identifying areas of crossover in which each specialty could benefit from the experience of the other. PMID:16426991

  10. Giant cell arteritis: a systemic disease with rare cutaneous manifestations.

    PubMed

    Baum, E W; Sams, W M; Payne, R R

    1982-06-01

    Giant cell arteritis is a systemic disease usually occurring in patients in the fifth decade or older, more often in women. Dermatologic manifestations are rare but, when found, are usually expressed as scalp ulcerations or blanching associated with gangrene of the tongue. The dermatologist should be familiar with the entity because it is often more severe when associated with scalp necrosis, and prompt intervention with corticosteroids can prevent catastrophic sequelae.

  11. Abdominal manifestations in childhood‐onset systemic lupus erythematosus

    PubMed Central

    Richer, O; Ulinski, T; Lemelle, I; Ranchin, B; Loirat, C; Piette, J C; Pillet, P; Quartier, P; Salomon, R; Bader‐Meunier, B

    2007-01-01

    Background Childhood‐onset lupus erythematosus is a rare disorder of unknown origin. Objectives To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow‐up, and discuss the specific causes of these manifestations. Methods Medical records of 201 patients with childhood‐onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations. Results Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5–16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment‐induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were <40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients. Conclusion Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded. PMID:16818463

  12. Acquired immunodeficiency syndrome: manifestations in the oral cavity.

    PubMed

    Aguirre-Urízar, José Manuel; Echebarría-Goicouría, María Angeles; Eguía-del-Valle, Asier

    2004-01-01

    Human immunodeficiency virus (HIV) infection is a pandemic disease characterised by impairment of the immune system; the main parameter is a progressive decline in the number of CD4 lymphocytes. This circumstance paves the way for opportunistic infections and the development of neoplastic processes that can lead the patient to a state known as acquired immunodeficiency syndrome (AIDS) and ultimately, results in death. The incorporation of treatment based on a cocktail of different active drugs (highly active antiretroviral therapy) has made it possible to drastically change the panorama of the disease in developed nations; improving quality of life for the patient and delaying the progression of the disease. The oral manifestations of HIV infection have been and continue to be an important component of the disease from the very first descriptions and are indicative of progression. At some point in the course of the disease, nine out of every ten patients will present oral manifestations and, on occasion, these symptoms will be the first sign of the syndrome. It is essential that oral healthcare professionals recognize the hallmarks of the illness. In developed countries, the emergence of new therapies has made it possible to significantly reduce immune deficiency-related oral manifestations, both in terms of frequency, as well as severity. This review analyses the most important oral lesions associated with HIV infection and the current state of affairs in this regard.

  13. Oral manifestations of HIV infection: a Panamerican perspective.

    PubMed

    Gillespie, G M; Mariño, R

    1993-01-01

    This paper presents a preliminary approach to the study of the oral manifestations of HIV infections in the region of the Americans. A general description of the lesions encountered is provided together with a review of the prevalence of the different manifestations in some countries of the Americas. Oral candidiasis was the most common oral lesion identified. Among oral candidiasis lesions differences were noted in relation to the frequency of the clinical forms seen. Hairy leukoplakia was the second most frequent lesion in almost all studies, with the exception the Peruvian study, where the most prevalent oral condition was xerostomia. The numbers of cases of HIV-gingivitis and HIV-periodontitis found in the countries of the Americas were lower than the cases in USA. Other oral manifestations of HIV infections seen were: Kaposi's sarcoma, oral erythema, labial herpetic infection. It is concluded that still more studies are needed, oral health professionals need additional training in the detection and treatment of lesions, and information needs to be systematized and standardized such that it is possible to make accurate comparisons among regions and countries. Recommendations are included to improve this situation.

  14. Endocrine manifestations and management of Prader-Willi syndrome.

    PubMed

    Emerick, Jill E; Vogt, Karen S

    2013-08-21

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

  15. [Preeclampsia--two manifestations of the same disease].

    PubMed

    Kornacki, Jakub; Skrzypczak, Jana

    2008-06-01

    Preeclampsia, due to its complicated and unexplained pathophysiology, remains one of the most difficult subjects in modern maternal-fetal medicine. Despite of the fact that diagnostic criteria of preeclampsia are clear and homogenous, the clinical manifestation of the disease, the prognosis and the frequency of maternal and fetal complications differ considerably among the cases. The aim of the review was to emphasize the heterogenous character of preeclampsia which may be manifested in two different clinical forms--a maternal and placental one. The maternal preeclampsia occurs mostly in multiparous patients with known risk factors of preeclampsia such as: 1) insulin resistance, 2) diabetes mellitus, 3) dyslipidemia, 4) obesisty, and 5) chronic hypertension. The placental form of the disease is more common among nulliparous women without prior risk factors of preeclampsia. The main clinical differences between the two forms are: 1) an earlier onset of placental preeclampsia in most cases, and 2) a more frequent presence of placental insufficiency in placental preeclampsia. The following article focuses on both forms of preeclampsia in the aspect of different clinical manifestations, different prognosis, as well as different management of the disease.

  16. Gastrointestinal manifestations of mitochondrial disorders: a systematic review

    PubMed Central

    Finsterer, Josef; Frank, Marlies

    2016-01-01

    Mitochondrial disorders (MIDs) due to respiratory-chain defects or nonrespiratory chain defects are usually multisystem conditions [mitochondrial multiorgan disorder syndrome (MIMODS)] affecting the central nervous system (CNS), peripheral nervous system, eyes, ears, endocrine organs, heart, kidneys, bone marrow, lungs, arteries, and also the intestinal tract. Frequent gastrointestinal (GI) manifestations of MIDs include poor appetite, gastroesophageal sphincter dysfunction, constipation, dysphagia, vomiting, gastroparesis, GI pseudo-obstruction, diarrhea, or pancreatitis and hepatopathy. Rare GI manifestations of MIDs include dry mouth, paradontosis, tracheoesophageal fistula, stenosis of the duodeno-jejunal junction, atresia or imperforate anus, liver cysts, pancreas lipomatosis, pancreatic cysts, congenital stenosis or obstruction of the GI tract, recurrent bowel perforations with intra-abdominal abscesses, postprandial abdominal pain, diverticulosis, or pneumatosis coli. Diagnosing GI involvement in MIDs is not at variance from diagnosing GI disorders due to other causes. Treatment of mitochondrial GI disease includes noninvasive or invasive measures. Therapy is usually symptomatic. Only for myo-neuro-gastro-intestinal encephalopathy is a causal therapy with autologous stem-cell transplantation available. It is concluded that GI manifestations of MIDs are more widespread than so far anticipated and that they must be recognized as early as possible to initiate appropriate diagnostic work-up and avoid any mitochondrion-toxic treatment. PMID:28286566

  17. Some exact tests for manifest properties of latent trait models

    PubMed Central

    De Gooijer, Jan G.; Yuan, Ao

    2011-01-01

    Item response theory is one of the modern test theories with applications in educational and psychological testing. Recent developments made it possible to characterize some desired properties in terms of a collection of manifest ones, so that hypothesis tests on these traits can, in principle, be performed. But the existing test methodology is based on asymptotic approximation, which is impractical in most applications since the required sample sizes are often unrealistically huge. To overcome this problem, a class of tests is proposed for making exact statistical inference about four manifest properties: covariances given the sum are non-positive (CSN), manifest monotonicity (MM), conditional association (CA), and vanishing conditional dependence (VCD). One major advantage is that these exact tests do not require large sample sizes. As a result, tests for CSN and MM can be routinely performed in empirical studies. For testing CA and VCD, the exact methods are still impractical in most applications, due to the unusually large number of parameters to be tested. However, exact methods are still derived for them as an exploration toward practicality. Some numerical examples with applications of the exact tests for CSN and MM are provided. PMID:21562625

  18. Lysophosphatidylcholine plays critical role in allergic airway disease manifestation

    PubMed Central

    Bansal, Preeti; Gaur, Shailendera Nath; Arora, Naveen

    2016-01-01

    Phospholipase A2 (sPLA2), pivotal for allergic and inflammatory response, hydrolyses phosphatidylcholine (PC) to lysophosphatidylcholine (LPC). In present study, the role of LPC in allergic airway disease manifestation was studied using mouse model. Balb/c mice were immunized using cockroach extract (CE) and LPC release was blocked by sPLA2 inhibitor. Airway hyperresponse (AHR), lung-histology, total and differential leukocyte count (TLC&DLC), Th2 type cytokines, sPLA2 activity and LPC levels in bronchoalveolar lavage fluid (BALF) were measured. Exogenous LPC was given to the mice with or without CE sensitization, to demonstrate its role in allergic airway disease manifestation. Anti-CD1d antibody was given to study the involvement of natural killer T (NKT) cells in LPC induced response. AHR, lung-inflammation, TLC, DLC, Th2 type cytokines, sPLA2 activity and LPC levels were increased on CE challenge. sPLA2 activity and LPC release was blocked by sPLA2-inhibitor, which decreased AHR, and inflammatory parameters. Exogenous LPC with or without CE sensitization increased above parameters. CE challenge or LPC exposure increased LY49C+TCRβ+ NKT cells in BALF and spleen, which was reduced by anti-CD1d antibody, accompanied with reduction in AHR and allergic airway inflammation parameters. Conclusively, LPC induces allergic airway disease manifestation and it does so probably via CD1d-restricted LY49C+TCRβ+ NKT cells. PMID:27282246

  19. The Ocular Manifestations of Drugs Used to Treat Multiple Sclerosis.

    PubMed

    Heath, Gregory; Airody, Archana; Gale, Richard Peter

    2017-03-01

    Recent times have seen an increase in the number of options to treat multiple sclerosis. Ocular manifestations of multiple sclerosis are well known to treating physicians; however, the medications used to treat multiple sclerosis can also have ocular side effects. This review article focuses on the ocular manifestations of corticosteroids and disease-modifying agents such as interferon, fingolomod, natalizumab, alemtuzumab and mitoxantron used to treat the disease. The ocular manifestations of multiple sclerosis treatments can be varied depending on the drug used, and include retinopathy, chronic central serous chorioretinopathy, macular oedema, Graves' ophthalmopathy and cortical blindness. These effects may be specific to the drug or secondary to their immunosuppressive effect. The association of macular oedema with fingolomod is clear and merits ocular screening for toxicity. The immunosuppressive nature of the treatments makes patients prone to acquired infections. Hence, if a patient with multiple sclerosis presents with vision loss, infectious and drug-induced aetiology should be considered alongside relapses of multiple sclerosis itself as a cause.

  20. Uveitis as an initial manifestation of acquired immunodeficiency syndrome.

    PubMed

    Tsen, Chui-Lien; Chen, Shih-Chou; Chen, Yao-Shen; Sheu, Shwu-Jiuan

    2017-01-01

    Human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) is a multisystem disease that can involve the human eyes. Using ophthalmic examination records from January 2006 to November 2015, we retrospectively reviewed all patients who were diagnosed with HIV/AIDS in our hospital. The study was performed at a tertiary referral center in southern Taiwan. Data included age, gender, ophthalmic examinations, systemic conditions, CD4 cell counts, course, and treatment. Eleven patients were identified as having AIDS with uveitis as their presenting manifestation. All were men, with a mean age of 39.5 ± 11.4 years (range 24-56). The mean CD4(+) T-cell counts were 91.7 ± 50.3 cells/μl (range 27-169). Ocular diagnoses included cytomegalovirus (CMV) retinitis in five patients, ocular syphilis in four patients, and ocular toxoplasmosis in two patients. Uveitis resolved in all patients after medical treatment. However, a retinal detachment developed in two eyes in CMV retinitis and one eye in ocular syphilis. Ocular manifestations are among the most common clinical features in patients with HIV/AIDS who have varying clinical presentations that affect almost all ocular structures. This study demonstrated that ocular findings could be an initial manifestation of an underlying disease. Awareness of ocular lesions in HIV/AIDS is important for early recognition and management.

  1. Congenital retrosternal hernias of Morgagni: Manifestation and treatment in children

    PubMed Central

    Slepov, Oleksii; Kurinnyi, Sergii; Ponomarenko, Oleksii; Migur, Mikhailo

    2016-01-01

    Background: Due to scarcity of congenital diaphragmatic hearnias of Morgagni (CDHM), non-specific clinical presentation in the pediatric age group, we aimed to investigate the incidence, clinical manifestations, anatomical characteristics, and develop diagnostic algorithm and treatment of CDHM in children. Materials and Methods: The patients’ records of children with CDHM treated in our hospital during past 20 years were retrospectively reviewed for the age at diagnosis, gender, clinical findings, anatomical features, operative details and outcome. Results: Since 1995 to 2014 we observed 6 (3 boys, 3 girls) patients with CDHM, that comprise 3.2% of all congenital diaphragmatic hernia cases (n = 185). Age at diagnosis varied from 3 mo. to 10y.o. Failure to thrive was main symptom in 4 patients, followed by recurrent respiratory infections (n = 3), dyspnea (n = 3), and gastrointestinal manifestations: constipation (n = 2), abdominal pain (n = 1). Work-up consisted of plain X-ray for all (n = 6), upper GI (n = 3), barium enema (n = 2), sonography (n = 6) and CT (n = 2). Abdominal approach used in 5 patients, and thoracotomy in one. Herniated contents were: liver lobes (n = 4), transverse colon (n = 3) and greater omentum (n = 1). 5 had right-sided lesion, 1- left-sided. Defect repaired using local tissues. Post-operative course was uneventful; all patients appeared well during follow-up. Conclusion: CDHM is very uncommon anomaly, very occasionally diagnosed at the early age. Failure to thrive and recurrent respiratory infections are most frequent clinical manifestations. In suspected CDHM we advocate the following work-up: plain chest and abdominal X-ray, contrast study (upper GI series or barium enema), ultrasonographic screen and CT scan. Surgical repair via abdominal approach, using local tissues and hernia sac removal is preferred. PMID:27251653

  2. Deep Vein Thrombosis as Initial Manifestation of Whipple Disease

    PubMed Central

    Henriques, Mônica Souza de Miranda; da Paz, Alexandre Rolim; Gaertner, Ana Beatriz Person; Melo, Cibelle Ingrid Stephen; Filgueiras, Priscyanne Lins; Jerome, Rafaella Alencar

    2016-01-01

    Introduction Wipple disease (WD) is a rare chronic disease caused by the bacillus Tropheryma whipplei. Constitutive, rheumatologic, gastrointestinal, cardiac, cerebral, lymphatic, cutaneous, and ophthalmological signs are possible systemic symptoms. However, thrombotic manifestations are rarely described as “stroke-like syndrome” or arterial thrombosis. Diagnosis is based on clinical manifestations and pathological examination. Laboratory findings may include anemia, leukocytosis, and thrombocytosis. Objective We report a case of venous thrombosis as initial manifestation of WD. Case Report We describe the case of a 53-year-old male with iliofemoral vein thrombosis followed by intermittent diarrhea, loss of appetite, abdominal distension, and bloating. A mild malnutrition state with a weight loss of 13 kg, pallor (+/4 +), presence of lower-limb edema (+/4 +), and hypertympanic distended abdomen occurred. Laboratory tests on admission revealed anemia, positive inflammatory activity tests, and normal coagulation. Endoscopic examination showed villous edema with white dotted infiltrates in the second duodenal portion and intestinal lymphangiectasia in the terminal ileum. Pathological examination revealed numerous macrophages with positive periodic acid-Schiff inclusions. Venous Doppler ultrasound showed extensive deep thrombosis on the left lower limb and recanalization of the femoral vein in the right lower limb. The patient was treated with ceftriaxone and enoxaparin sodium, which led to an improvement of gastrointestinal and thrombosis symptoms. Comments Hypercoagulability, endothelial damage, vasculitis, and blood stasis are present in T. whipplei infection, which are associated with the activation of inflammatory mechanisms as well as procoagulant and thromboembolic events. WD should be part of the differential diagnosis of diseases that cause venous thrombosis of unknown origin. PMID:27920655

  3. Neurologic Manifestations of Enterovirus 71 Infection in Korea

    PubMed Central

    2016-01-01

    Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients’ mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection. PMID:27051240

  4. Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

    PubMed

    Wu, E Y; Schanberg, L E; Wershba, E C; Rabinovich, C E

    2017-05-01

    Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

  5. Ionospheric manifestations of earthquakes and tsunamis in a dynamic atmosphere

    NASA Astrophysics Data System (ADS)

    Godin, Oleg A.; Zabotin, Nikolay A.; Zabotina, Liudmila

    2015-04-01

    Observations of the ionosphere provide a new, promising modality for characterizing large-scale physical processes that occur on land and in the ocean. There is a large and rapidly growing body of evidence that a number of natural hazards, including large earthquakes, strong tsunamis, and powerful tornadoes, have pronounced ionospheric manifestations, which are reliably detected by ground-based and satellite-borne instruments. As the focus shifts from detecting the ionospheric features associated with the natural hazards to characterizing the hazards for the purposes of improving early warning systems and contributing to disaster recovery, it becomes imperative to relate quantitatively characteristics of the observed ionospheric disturbances and the underlying natural hazard. The relation between perturbations at the ground level and their ionospheric manifestations is strongly affected by parameters of the intervening atmosphere. In this paper, we employ the ray theory to model propagation of acoustic-gravity waves in three-dimensionally inhomogeneous atmosphere. Huygens' wavefront-tracing and Hamiltonian ray-tracing algorithms are used to simulate propagation of body waves from an earthquake hypocenter through the earth's crust and ocean to the upper atmosphere. We quantify the influence of temperature stratification and winds, including their seasonal variability, and air viscosity and thermal conductivity on the geometry and amplitude of ionospheric disturbances that are generated by seismic surface waves and tsunamis. Modeling results are verified by comparing observations of the velocity fluctuations at altitudes of 150-160 km by a coastal Dynasonde HF radar system with theoretical predictions of ionospheric manifestations of background infragravity waves in the ocean. Dynasonde radar systems are shown to be a promising means for monitoring acoustic-gravity wave activity and observing ionospheric perturbations due to earthquakes and tsunamis. We will discuss

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

    PubMed Central

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

    2012-01-01

    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

  7. Central nervous system manifestations of neonatal lupus: a systematic review.

    PubMed

    Chen, C C; Lin, K-L; Chen, C-L; Wong, A May-Kuen; Huang, J-L

    2013-12-01

    Neonatal lupus is a rare and acquired autoimmune disease. Central nervous system abnormalities are potential manifestations in neonatal lupus. Through a systematic literature review, we analyzed the clinical features of previously reported neonatal lupus cases where central nervous system abnormalities had been identified. Most reported neonatal lupus patients with central nervous system involvement were neuroimaging-determined and asymptomatic. Only seven neonatal lupus cases were identified as having a symptomatic central nervous system abnormality which caused physical disability or required neurosurgery. A high percentage of these neurosymptomatic neonatal lupus patients had experienced a transient cutaneous skin rash and had no maternal history of autoimmune disease before pregnancy.

  8. Chromosomal and Multifactorial Genetic Disorders with Oral Manifestations

    PubMed Central

    Patil, Shankargouda; Rao, Roopa S; Majumdar, Barnali

    2014-01-01

    The chromosomal disorders are individually rare, but collectively they are common whereas the multifactorial disorders are the most common form of genetic disorders. The chromosomal anomalies typically arise from alterations in the DNA containing chromosomal regions and can be reliably detected by karyotype analysis, whereas the multifactorial disorders demonstrate multi-gene as well as environmental interactions. Both the chromosomal and multifactorial disorders may manifest signs and symptoms such as a combination of birth defects, physical disabilities, challenging behavior and certain craniofacial defects as well, the knowledge of which can aid in a better patient management in everyday practice of dentistry. PMID:25395808

  9. Myelitis and Lupus: Clinical Manifestations, Diagnosis and Treatment. Review.

    PubMed

    Chiganer, Edson Hernán; Hryb, Javier Pablo; Carnero Contentti, Edgar

    2016-08-23

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple systems. Myelopathy is one of 19 neuropsychiatric syndromes related to SLE defined by the American College of Rheumatology. Although infrequent, it is a severe manifestation, leading to motor and sensory deficits, and sphincter dysfunction. The pathogenesis is not clearly known, but may be related to arterial thrombosis and vasculitis. Diagnosis is based on clinical findings, laboratory tests and the use of gadolinium-enhanced magnetic resonance imaging. The standard therapy is the combination of intravenous cyclophosphamide and glucocorticoids. In refractory disease, other treatments such as plasmapheresis or rituximab have been used.

  10. Chronic dermal sinuses as a manifestation of histiocytosis X.

    PubMed Central

    Sacks, S H; Hall, I; Ragge, N; Pritchard, J

    1986-01-01

    Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus formation secondary to lymph node disease has never before been recorded as a manifestation of histiocytosis X. Histiocytosis X should therefore be considered in the differential diagnosis of "suppurative" lymphadenopathy so that appropriate treatment may be given without delay. Images Case 1 PMID:3084014

  11. [A case of glioblastoma manifesting 49 years after lobotomy].

    PubMed

    Fukushima, H; Yamaguchi, T; Arai, T; Nakagawa, S; Tsuchiya, K; Torii, N; Shirai, M

    1998-08-01

    We report a case of glioblastoma manifesting 49 years after a lobotomy. He was diagnosed as having schizophrenia at age 20 and was operated on with a standard lobotomy when he was 27 years old. He had led a useful life after 40 years old without medication. Because of hallucination and delusion, he was hospitalized at the end of 1996. CT showed a well enhanced tumor adjacent to the cavity made by the lobotomy in the left frontal lobe. This is the second case report of glioblastoma just beside the cavity formed by lobotomy. The relationship between glioblastoma and old lobotomy is discussed, especially in regard to morphology and CT findings.

  12. [Cat-scratch disease with bone compromise: atypical manifestation].

    PubMed

    Rodríguez C, Magdalena; Giachetto L, Gustavo; Cuneo E, Alejandro; Gutiérrez B, María del C; Shimchack R, Mario; Pírez G, M Catalina

    2009-08-01

    Fever, headache, myalgias and lymphadenopathy are characteristic manifestations of cat-scratch disease but other less common findings are described in 2 to 10% of cases. We report two children that presented with hepatosplenic abscesses and bone involvement. One child, had multiple areas of increased uptake in the bone scintigram with a positive serology (IgG > 1/256, IgM slightly positive). The second child had destruction of the L2 vertebral body that compromised the channel and right foramen as visualized by MRI. In both cases, bacilli were observed in the bone biopsy by Warthing-Starry stain.

  13. Extraparenchymal (Racemose) Neurocysticercosis and Its Multitude Manifestations: A Comprehensive Review

    PubMed Central

    Mehta, Anish; Rangasetty, Srinivasa

    2015-01-01

    Neurocysticercosis is an infection of the central nervous system caused by the larval form of the pork tapeworm Taenia solium. In the brain it occurs in two forms: parenchymal and extraparenchymal or racemose cysts. The clinical presentation of racemose cysts is pleomorphic, and is quite different from parenchymal cysticercosis. The clinical diagnosis of racemose cysts is quite challenging, with neuroimaging being the mainstay. However, the advent of newer brain imaging modalities has made a more accurate diagnosis possible. The primary focus of this article is racemose neurocysticercosis and its multitude manifestations, and includes a discussion of the newer diagnostic modalities and treatment options. PMID:26022457

  14. Acute meningitis as an initial manifestation of Erysipelothrix rhusiopathiae endocarditis.

    PubMed

    Joo, Eun-Jeong; Kang, Cheol-In; Kim, Wook Sung; Lee, Nam Yong; Chung, Doo Ryeon; Peck, Kyong Ran; Song, Jae-Hoon

    2011-10-01

    Erysipelothrix rhusiopathiae is a gram-positive bacillus which is found worldwide. Although bloodstream infections caused by E. rhusiopathiae are not common, there is a strong association between bacteremia and the development of infective endocarditis. The risk of human infection with Erysipelothrix is closely related to the opportunity for exposure to the organisms. We report a case of community-acquired meningitis as an initial manifestation of E. rhusiopathiae endocarditis in a 56-year-old woman, who had no history of exposure to animals.

  15. Peripheral nervous system manifestations in systemic autoimmune diseases.

    PubMed

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

    2014-09-01

    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.

  16. Ulcerated Lesion of the Tongue as Manifestation of Systemic Coccidioidomycosis.

    PubMed

    Mendez, Luis A; Flores, Sergio A; Martinez, Ricardo; de Almeida, Oslei Paes

    2017-01-01

    Systemic mycoses and their oral manifestations are very rare. We present a case of a 60-year-old man with an ulcerated lesion on the lateral border of the tongue. Histologic studies revealed a granulomatous fungal infection by Coccidioides immitis. After pharmacological treatment, the lesion resolved. Recently, northern Mexico has been reported to be an endemic zone of C. immitis infections; therefore it should be considered in the differential diagnosis of mouth lesions. A comprehensive clinical history, physical exploration, and complementary studies are essential for an accurate diagnosis.

  17. Complete atrioventricular block as initial manifestation of systemic lupus erythematosus.

    PubMed

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

    2009-01-01

    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.

  18. Ulcerated Lesion of the Tongue as Manifestation of Systemic Coccidioidomycosis

    PubMed Central

    Flores, Sergio A.; Martinez, Ricardo; de Almeida, Oslei Paes

    2017-01-01

    Systemic mycoses and their oral manifestations are very rare. We present a case of a 60-year-old man with an ulcerated lesion on the lateral border of the tongue. Histologic studies revealed a granulomatous fungal infection by Coccidioides immitis. After pharmacological treatment, the lesion resolved. Recently, northern Mexico has been reported to be an endemic zone of C. immitis infections; therefore it should be considered in the differential diagnosis of mouth lesions. A comprehensive clinical history, physical exploration, and complementary studies are essential for an accurate diagnosis. PMID:28386282

  19. [Congenital deficiency of protein C with late clinical manifestation].

    PubMed

    Ortega Calvo, M; Méndez Mora, J L; Macías Pérez, V; Mellado Fuentes, F

    1994-07-01

    The congenital deficit of protein C has a great diversity of clinical manifestations regarding age. In this paper, we describe the case of a man whose initial symptomatology appeared at the age of 68. Protein C is a vitamin-K dependent plasmatic glycoprotein which has anticoagulant activity through the inactivation of factors Va and VIIIa. The hereditary deficit of protein C is usually presented in a dominant autosomic mode with partial penetration and its prevalence is estimated in each of 200-300 healthy blood donors.

  20. Diffuse systemic sclerosis with bullous lesions without systemic manifestations*

    PubMed Central

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

    2013-01-01

    Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement. PMID:24346886

  1. Ophthalmic manifestations in IgG4-related disease

    PubMed Central

    Ebbo, Mikael; Patient, Matthieu; Grados, Aurelie; Groh, Matthieu; Desblaches, Julien; Hachulla, Eric; Saadoun, David; Audia, Sylvain; Rigolet, Aude; Terrier, Benjamin; Perlat, Antoinette; Guillaud, Constance; Renou, Frederic; Bernit, Emmanuelle; Costedoat-Chalumeau, Nathalie; Harlé, Jean-Robert; Schleinitz, Nicolas

    2017-01-01

    Abstract IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series. Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling “modified” comprehensive diagnostic criteria with pathological documentation were retained for the study. Clinical, biological, pathological, radiological findings and data regarding the response to treatment were retrospectively analyzed. According to our data registry, the frequency of IgG4-related ophthalmic disease (IgG4-ROD) was 17%. Mean age at diagnosis was 55.1 ± 7.1 years with a male/female ratio of 2.2. The 19 cases of IgG4-ROD consisted of lacrimal gland (68.4%), soft tissue (57.9%), extra-ocular muscles (36.8%), palpebral (21.1%), optical nerve (10.5%), orbital bone (10.5%), and mononeuritis (V1 and/or V2, 10.5%) involvements. IgG4-ROD was bilateral in 57.9% of cases. Extra-ophthalmic manifestations were reported in 78.9% of cases. All patients responded to prednisone but two-thirds of patients relapsed within a mean (SD) of 9.8 (3.5) months and 72.2% required long-term glucocorticoids and/or immunosuppressive agents. Eight patients were treated by rituximab with a favorable response in 87.5% of cases. Lacrimal involvement is the most frequent ophthalmic manifestation of IgG4-RD and is frequently associated with extra-orbital manifestations. Despite initial favorable response to steroids, the long-term management of relapsing patients needs to be improved. PMID:28272212

  2. Verrucous Eyebrows: A Cutaneous Manifestation of a Systemic Disease.

    PubMed

    Hinojosa, Tiffany; Lewis, Daniel J; Sharghi, Kevin G; Ramos, Elmira; Rapini, Ronald P; Tyring, Stephen K

    2017-03-29

    Sarcoidosis is an inflammatory disease that can involve numerous organ systems, with the skin being the second most commonly affected.(1) The histopathological hallmark of sarcoidosis is the presence of non-caseating epithelioid-cell granulomas, but its numerous clinical manifestations can resemble those of other skin diseases.(2,3) We report the unique presentation of a woman who developed verrucous sarcoidal lesions around the eyebrows bilaterally at the site of cosmetic eyebrow tattoos, ultimately leading to a diagnosis of otherwise asymptomatic systemic sarcoidosis. This article is protected by copyright. All rights reserved.

  3. Esophageal Candidiasis as the Initial Manifestation of Acute Myeloid Leukemia.

    PubMed

    Komeno, Yukiko; Uryu, Hideki; Iwata, Yuko; Hatada, Yasumasa; Sakamoto, Jumpei; Iihara, Kuniko; Ryu, Tomiko

    2015-01-01

    A 47-year-old woman presented with persistent dysphagia. A gastroendoscopy revealed massive esophageal candidiasis, and oral miconazole was prescribed. Three weeks later, she returned to our hospital without symptomatic improvement. She was febrile, and blood tests showed leukocytosis (137,150 /μL, blast 85%), anemia and thrombocytopenia. She was diagnosed with acute myeloid leukemia (AML). She received chemotherapy and antimicrobial agents. During the recovery from the nadir, bilateral ocular candidiasis was detected, suggesting the presence of preceding candidemia. Thus, esophageal candidiasis can be an initial manifestation of AML. Thorough examination to detect systemic candidiasis is strongly recommended when neutropenic patients exhibit local candidiasis prior to chemotherapy.

  4. Imaging of Pulmonary Manifestations of Connective Tissue Diseases.

    PubMed

    Ahuja, Jitesh; Arora, Deepika; Kanne, Jeffrey P; Henry, Travis S; Godwin, J David

    2016-11-01

    Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia. Drug treatment of CTDs can cause complications, including opportunistic infection.

  5. Neonatal erythroderma as a first manifestation of Menkes disease.

    PubMed

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

    2012-07-01

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

  6. Automated Detection of Anomalous Shipping Manifests to Identify Illicit Trade

    SciTech Connect

    Sanfilippo, Antonio P.; Chikkagoudar, Satish

    2013-11-12

    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.

  7. An alternative NMSSM phenomenology with manifest perturbative unification

    SciTech Connect

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

    2007-12-18

    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).

  8. Various Manifestations of Hyperthyroidism in an Ambulatory Clinic: Case Studies

    PubMed Central

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

    1987-01-01

    This study reviews five cases of women with hyperthyroidism, three black women and two Hispanic women. Initially, two patients presented with voice changes, weight loss, and increased appetite. Only two patients presented with classical symptoms of hyperthyroidism. Examination showed all patients had diffusely enlarged thyroids and exaggerated reflexes. Two patients showed Graves' opthalmopathy. These cases document the variety of presentations of hyperthyroidism. Hence, a high index of suspicion must exist for this disease, even in the absence of a number of the classical manifestations of hyperthyroidism. When patients present to primary care centers with a constellation of symptoms, an examination of the thyroid gland is essential. PMID:3694696

  9. Manifestation of severe coronary heart disease after anabolic drug abuse.

    PubMed

    Mewis, C; Spyridopoulos, I; Kühlkamp, V; Seipel, L

    1996-02-01

    Anabolic steroids are frequently abused, thus increasing the risk of cardiovascular disease, despite the known unfavorable influence on lipid profiles. We report on a young bodybuilder who presented with ventricular tachycardia as the first manifestation of severe underlying coronary heart disease. Coronary angiogram revealed severe stenotic lesions in the right coronary artery and the left descending coronary artery, and hypokinetic regions corresponded to posterolateral and anterior myocardial infarctions. This young patient had a history without any coronary risk factors, but with a 2-year abuse of the anabolic steroid stanazolol. No report published so far has shown possible atherogenic consequences of long-term abuse of stanazolol.

  10. Atypical giant haemangioma of liver with systemic inflammatory manifestations.

    PubMed

    Khalid, Mohd; Ahmad, Mehtab; Jain, Amit; Rizvi, Imran

    2013-01-25

    Haemangioma is the most common benign tumour of the liver. Most of them are small in size (less than 4 cm) and are often asymptomatic and discovered incidentally on modern diagnostic imaging. Lesions with a diameter larger than 4 cm are called giant haemangiomas, and these are usually located in the right hepatic lobe. Although haemangioma is the most frequent benign tumour of the liver, 'giant' haemangiomas are rare. Such lesions may give rise to symptoms requiring treatment. In this case report, we describe the case of a 50-year-old Indian man who presented with giant liver haemangioma and systemic inflammatory manifestations.

  11. Oral manifestations caused by the linear IgA disease.

    PubMed

    Eguia del Valle, Asier; Aguirre Urízar, José Manuel; Martínez Sahuquillo, Angel

    2004-01-01

    The Linear IgA deposit related disease or Linear IgA disease (LAD) is a chronic, uncommon and autoimmunological mucocutaneous disease, characterised by linear IgA deposits along the basement membrane zone. In mainly cases, moreover cutaneous lesions, there are oral mucosal and other mucosal lesions. There are also, some cases published of Linear IgA disease limited to oral mucosa. The known of this disease is important for the establishment of a correct differential diagnosis in cases of blistering mucocutaneous diseases. In this paper, we analyze the most important features of this disease, attending specially to the oral manifestations.

  12. Fournier gangrene: first manifestation of occult rectal cancer.

    PubMed

    Ruiz-Tovar, J; Córdoba, L; Devesa, J M

    2011-01-01

    Fournier gangrene is a necrotizing fasciitis of the genital and perineal region. Diverse factors predispose to Fournier gangrene, such as diabetes mellitus, ethylism, liver dysfunction, haematological disorders, obesity or recent regional instrumentation. Rectal tumours can also predispose to Fournier gangrene; most of the reported cases are perforated or unresectable colorectal tumours, but some cases of anorectal cancer diagnosed after recovery from Fournier gangrene have also been reported. In these cases, the presence of a rectal tumour at the time of, or prior to, diagnosis of Fournier gangrene could not be ruled out. We present three cases of rectal cancer whose first manifestation was as Fournier gangrene.

  13. Psychosis as a late manifestation of Sheehan's syndrome.

    PubMed

    Reddy, Mukku Shiva Shanker; Nahar, Abhinav; Thippeswamy, Harish; Kumar, Chaturvedi Santosh

    2017-02-01

    Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum haemorrhage. It is one of the most common causes of hypopituitarism in underdeveloped or developing countries. Characteristic manifestations include failure to lactate or to resume menses, genital and axillary hair loss, asthenia and weakness, fine wrinkles around the eyes and lips, signs of premature aging, dry skin, hypopigmentation and other evidence of hypopituitarism. Uncommonly it can present with psychosis. There are only few case reports of psychoses in patients with Sheehan's syndrome. Our case report illustrates the relationship between psychosis and Sheehan's syndrome. The treatment challenges in managing Sheehan's syndrome and psychosis are discussed.

  14. Rare Bone Diseases and Their Dental, Oral, and Craniofacial Manifestations

    PubMed Central

    Foster, B.L.; Ramnitz, M.S.; Gafni, R.I.; Burke, A.B.; Boyce, A.M.; Lee, J.S.; Wright, J.T.; Akintoye, S.O.; Somerman, M.J.; Collins, M.T.

    2014-01-01

    Hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. Though many of these conditions are individually rare, the total number of people affected is great. These disorders often include dental-oral-craniofacial (DOC) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. In this review, we focus on dental, oral, and craniofacial manifestations of rare bone diseases. Discussed are defects in 4 key physiologic processes in bone/tooth formation that serve as models for the understanding of other diseases in the skeleton and DOC complex: progenitor cell differentiation (fibrous dysplasia), extracellular matrix production (osteogenesis imperfecta), mineralization (familial tumoral calcinosis/hyperostosis hyperphosphatemia syndrome, hypophosphatemic rickets, and hypophosphatasia), and bone resorption (Gorham-Stout disease). For each condition, we highlight causative mutations (when known), etiopathology in the skeleton and DOC complex, and treatments. By understanding how these 4 foci are subverted to cause disease, we aim to improve the identification of genetic, molecular, and/or biologic causes, diagnoses, and treatment of these and other rare bone conditions that may share underlying mechanisms of disease. PMID:24700690

  15. Neurological manifestations of connective tissue diseases mimicking multiple sclerosis.

    PubMed

    Pelidou, Sigliti-Henrietta; Giannopoulos, Sotiris; Tzavidi, Sotiria; Tsifetaki, Niki; Kitsos, Georgios; Stefanou, Dimitrios; Kostadima, Vassiliki; Drosos, Alexandros A; Kyritsis, Athanassios P

    2007-11-01

    The objective of the study was to analyze retrospectively the clinical, laboratory and imaging findings of multiple sclerosis (MS), such as the manifestations in a cohort of 132 patients referred to the neurology in and outpatient clinic. The proposed clinical and laboratory diagnostic criteria for MS and connective tissue disorders were systematically assessed in 132 consecutive patients. Cerebrospinal fluid serology and brain or spinal cord MRI were studied in all cases. In patients suspected for connective tissue disorder, schirmer test, rose bengal staining and biopsy of minor salivary glands were performed. A total of 115 (87%) patients were diagnosed to have definite MS, while 17 (13%) were diagnosed to have connective tissue disorder. Positive neurological and MRI findings were observed in both groups. The majority of patients with connective tissue disorder demonstrated extra-neurological manifestations like Raynaud's phenomenon, arthritis, livedo reticularis, purpura and presence of multiple autoantibodies in their sera. All patients with MS should be screened systematically for connective tissue disorder. In the absence of pathognomonic clinical and laboratory findings, the diagnosis of MS is a diagnosis of exclusion.

  16. Amount of manifested test anxiety in the heterogeneous classroom.

    PubMed

    Gjesme, T

    1982-03-01

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

  17. Cardiac manifestations in HIV-infected Thai children.

    PubMed

    Pongprot, Yupada; Sittiwangkul, Rekwan; Silvilairat, Suchaya; Sirisanthana, Virat

    2004-06-01

    Cardiac complications contribute significantly to morbidity and mortality in HIV-infected children. There have been few reports of cardiac manifestations in HIV-infected children in developing countries. The aims of this study were to evaluate the clinical manifestations and echocardiographic findings in Thai children with HIV infection and determine the clinical predictors of left ventricular dysfunction and pulmonary hypertension. We retrospectively reviewed the medical records of 27 infants infected with HIV perinatally who presented with cardiovascular problems at a tertiary care hospital between 1995 and 2000. The mean age at initial cardiac evaluation was 36 months (range 8-65). Signs and symptoms included dyspnoea in all cases, oedema in 12 (44%), finger clubbing in 11 (41%), cyanosis in 6 (22%) and S(3) gallop in 8 (30%). Echocardiographic abnormalities included pericardial effusion in 12 (44 %), right ventricular dilatation in 12 (44%), pulmonary hypertension in 11 (41%), diminished left ventricular fractional shortening in 10 (37%), left ventricular dilatation in 9 (33%) and combined ventricular dilatation in 2 (7%). Left ventricular dysfunction did not correlate with HIV CDC classification, age, nutritional status or clinical signs and symptoms.

  18. Esophageal Rupture as a Primary Manifestation in Eosinophilic Esophagitis

    PubMed Central

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

    2014-01-01

    Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and, histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, and chest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause food impaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to the friability of the esophageal mucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave's syndrome, as a primary manifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initial manifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 of the patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majority of previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history of dysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE. PMID:24899902

  19. Autoimmune bullous skin diseases. Part 1: Clinical manifestations.

    PubMed

    Kneisel, Andrea; Hertl, Michael

    2011-10-01

    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.

  20. Antibody induction of lupus-like neuropsychiatric manifestations.

    PubMed

    Lawrence, David A; Bolivar, Valerie J; Hudson, Chad A; Mondal, Tapan K; Pabello, Nina G

    2007-01-01

    Although systemic lupus erythematosus (SLE) is usually evaluated with regard to autoimmune reactivity toward the kidney, there are multiple psychiatric abnormalities associated with this autoimmune disease. Lupus-prone male NZM88 mice, derived from NZB/NZW F1 mice, develop early neuropsychiatric manifestations without any signs of nephritis. In addition to the usual repertoire of antibody specificities, including autoantibodies to dsDNA and renal antigens, mice of this inbred strain express autoantibodies to numerous brain antigens. Here, we show that autoantibodies to brain antigens, assessed by Western analysis, are as individually varied as are the diverse neuropsychiatric manifestations observed in SLE patients. Additionally, a monoclonal antibody derived from the spleen of an untreated NZM88 male when injected into healthy BALB/cByJ, but not C57BL/6J, mice induced behaviors similar to those of lupus-prone NZM88 mice. This monoclonal antibody, which is specific to dynamin-1, binds preferentially in BALB/cByJ cortex and induces substantial expression of cytokines mainly in the hypothalamus. Thus, an antibody to just one brain antigen can induce multiple behavioral changes, and multiple autoantibodies to different brain antigens exist in lupus-prone mice; however, susceptibility to the induction of neurobehavioral deficits is dependent on host genetics.

  1. Rare bone diseases and their dental, oral, and craniofacial manifestations.

    PubMed

    Foster, B L; Ramnitz, M S; Gafni, R I; Burke, A B; Boyce, A M; Lee, J S; Wright, J T; Akintoye, S O; Somerman, M J; Collins, M T

    2014-07-01

    Hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. Though many of these conditions are individually rare, the total number of people affected is great. These disorders often include dental-oral-craniofacial (DOC) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. In this review, we focus on dental, oral, and craniofacial manifestations of rare bone diseases. Discussed are defects in 4 key physiologic processes in bone/tooth formation that serve as models for the understanding of other diseases in the skeleton and DOC complex: progenitor cell differentiation (fibrous dysplasia), extracellular matrix production (osteogenesis imperfecta), mineralization (familial tumoral calcinosis/hyperostosis hyperphosphatemia syndrome, hypophosphatemic rickets, and hypophosphatasia), and bone resorption (Gorham-Stout disease). For each condition, we highlight causative mutations (when known), etiopathology in the skeleton and DOC complex, and treatments. By understanding how these 4 foci are subverted to cause disease, we aim to improve the identification of genetic, molecular, and/or biologic causes, diagnoses, and treatment of these and other rare bone conditions that may share underlying mechanisms of disease.

  2. Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome.

    PubMed

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

    2014-01-01

    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.

  3. Musculoskeletal manifestations of mild osteogenesis imperfecta in the adult.

    PubMed

    McKiernan, Fergus E

    2005-12-01

    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.

  4. Manifestations of paediatric Leishmania infantum infections in Malta

    PubMed Central

    Pace, David; Williams, Thomas N.; Grochowska, Alicja; Betts, Alexandra; Attard-Montalto, Simon; Boffa, Michael J.; Vella, Cecil

    2011-01-01

    Summary Leishmania infantum is endemic in the Maltese archipelago, a group of islands in the Mediterranean which are visited frequently by tourists from Northern European countries. The burden of leishmaniasis is highest in children who may present with cutaneous or visceral manifestations. We describe systematically the manifestations, diagnosis and management of leishmaniasis in children <14 years of age, who had a histopathological diagnosis of leishmaniasis in Malta, from 2004 to 2008. Eleven children were diagnosed with leishmaniasis; 8 children (15–44 months of age) had visceral disease and three (aged 9–13 years) suffered cutaneous infections. Prolonged high grade fever, pallor, hepatosplenomegaly, and pancytopenia were common presenting features of visceralisation. Diagnosis was based on the visualisation of amastigotes from bone marrow aspirates. Pentavalent antimonials were associated with treatment failure in two children, whilst liposomal amphotericin B was curative in all. Children with cutaneous leishmaniasis had dry crusted ulcero-nodular lesions on exposed areas which responded to intra-lesional instillation of sodium stibogluconate or to cryotherapy. Leishmaniasis should be included in the differential diagnosis of fever and hepatosplenomegaly or chronic cutaneous lesions in children who travel to Malta. PMID:21212024

  5. Analysis of Recent Manifests for Goods Imported through US Ports

    SciTech Connect

    Descalle, M; Manatt, D; Slaughter, D

    2006-09-27

    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.

  6. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management

    PubMed Central

    Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques. PMID:27034677

  7. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management.

    PubMed

    Srivanitchapoom, Chonticha; Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques.

  8. Achilles tendinitis as a rare extraintestinal manifestation of ulcerative colitis.

    PubMed

    Zenda, Takahiro; Araki, Ichiro; Nakamiya, Otoyuki; Tokuumi, Yuji; Shimada, Yuka; Komai, Keigo; Taniuchi, Yukie

    2016-06-01

    Patients with inflammatory bowel disease often have extraintestinal manifestations (EIMs) involving almost all organ systems, but little has been reported on Achilles tendinitis. Herein, we present a unique case of Achilles tendinitis, which manifested shortly after initiation of mesalazine therapy for ulcerative colitis. A 26-year-old Japanese woman with bloody diarrhea and abdominal cramps lasting for 7 days was referred to our hospital. The Lichtiger clinical activity index (CAI) score was 9 at the first visit. Based on the clinical symptoms and examination results, she was diagnosed with ulcerative pancolitis in the active phase, and treatment with mesalazine (2.4 g/day) and probiotics was initiated. Her symptoms resolved within 7 days of treatment (CAI 3). However, she then developed bilateral Achilles tendinitis without any apparent cause. The Achilles tendinitis subsided with conservative management within 2 weeks, despite continuation of mesalazine therapy. This case instructively suggests that Achilles tendinitis should be noted as an EIM of ulcerative colitis.

  9. Neural Manifestations of Implicit Self-Esteem: An ERP Study

    PubMed Central

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

    2014-01-01

    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

  10. Cardiac Manifestations of Rheumatological Conditions: A Narrative Review

    PubMed Central

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

    2012-01-01

    Cardiovascular diseases are common in systemic rheumatologic diseases. They can be presented at the time of diagnosis or after diagnosis. The cardiac involvements can be the first presentation of rheumatologic conditions. It means that a patient with rheumatologic disease may go to a cardiologist when attacked by this disease at first. These manifestations are very different and involve different structures of the heart, and they can cause mortality and morbidity of patients with rheumatologic diseases. Cardiac involvements in these patients vary from subclinical to severe manifestations. They may need aggressive immunosuppressive therapy. The diagnosis of these conditions is very important for choosing the best treatment. Premature atherosclerosis and ischemic heart disease are increased in rheumatoid arthritis and systemic lupus erythematosus, and may be causes of mortality among them. The aggressive control of systemic inflammation in these diseases can reduce the risk of cardiovascular disease especially ischemic heart disease. Although aggressive treatment of primary rheumatologic diseases can decrease mortality rate and improve them, at this time, there are no specific guidelines and recommendations, to include aggressive control and prevention of traditional risk factors, for them. PMID:23119182

  11. Stroke as the First Manifestation of Atrial Fibrillation

    PubMed Central

    Jaakkola, Jussi; Mustonen, Pirjo; Kiviniemi, Tuomas; Hartikainen, Juha E. K.; Palomäki, Antti; Hartikainen, Päivi; Nuotio, Ilpo; Ylitalo, Antti; Airaksinen, K. E. Juhani

    2016-01-01

    Atrial fibrillation may remain undiagnosed until an ischemic stroke occurs. In this retrospective cohort study we assessed the prevalence of ischemic stroke or transient ischemic attack as the first manifestation of atrial fibrillation in 3,623 patients treated for their first ever stroke or transient ischemic attack during 2003–2012. Two groups were formed: patients with a history of atrial fibrillation and patients with new atrial fibrillation diagnosed during hospitalization for stroke or transient ischemic attack. A control group of 781 patients with intracranial hemorrhage was compiled similarly to explore causality between new atrial fibrillation and stroke. The median age of the patients was 78.3 [13.0] years and 2,009 (55.5%) were women. New atrial fibrillation was diagnosed in 753 (20.8%) patients with stroke or transient ischemic attack, compared to 15 (1.9%) with intracranial hemorrhage. Younger age and no history of coronary artery disease or other vascular diseases, heart failure, or hypertension were the independent predictors of new atrial fibrillation detected concomitantly with an ischemic event. Thus, ischemic stroke was the first clinical manifestation of atrial fibrillation in 37% of younger (<75 years) patients with no history of cardiovascular diseases. In conclusion, atrial fibrillation is too often diagnosed only after an ischemic stroke has occurred, especially in middle-aged healthy individuals. New atrial fibrillation seems to be predominantly the cause of the ischemic stroke and not triggered by the acute cerebrovascular event. PMID:27936187

  12. Skin and Systemic Manifestations of Jellyfish Stings in Iraqi Fishermen

    PubMed Central

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

    2009-01-01

    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

  13. [The sacroiliac joint dysfunction: clinical manifestations, diagnostics and manual therapy].

    PubMed

    Grgić, Vjekoslav

    2005-01-01

    Sacroiliac joint dysfunction is one of the proved causes of sacroiliac joint syndrome. We are talking about the restricted mobility of sacrum opposite to ilium the type of "reversible blockage of movement". Main characteristics of dysfunction are as follows: restricted "joint play", referred pain, normal radiological finding, normal lab results and disappearance of clinical symptoms after deblocking of articular bodies. Pain from a blocked joint can be referred to lower back, buttocks, hip, groin, thigh, calf and lower part of abdomen. Dispersion of painful regions is a consequence of a complex and variable innervation of articular capsule. Blocked position of articular bodies and protracted tension of articular capsule causes a stimulus of nociceptors by which a capsule is protected. Nociceptive activity is manifested with referred pains in innervational region of stimulated sensitive nerves. In the article, besides the clinical manifestations, there is described a diagnostics and manual therapy of dysfunction. Springing tests by means of which a passive mobility ("joint play") is being tested, are most valuable in dysfunction diagnostics. Manual therapy (mobilization/manipulation) is indicated and efficacious with the patients suffering from dysfunction.

  14. Epstein-Barr virus and skin manifestations in childhood.

    PubMed

    Di Lernia, Vito; Mansouri, Yasaman

    2013-10-01

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

  15. Management of otolaryngological manifestations in mucopolysaccharidoses: our experience.

    PubMed

    Mesolella, M; Cimmino, M; Cantone, E; Marino, A; Cozzolino, M; Della Casa, R; Parenti, G; Iengo, M

    2013-08-01

    Mucopolysaccharidoses (MPSs) are lysosomal storage disorders caused by deficiency of enzymes involved in the degradation of glycosaminoglycans (GAGs). These disorders are associated with the accumulation of GAGs in tissues with organomegaly, mental retardation and short stature. Otologic and upper respiratory tract pathologies are among the earliest clinical manifestations. We analyzed 20 patients (13 male and 7 female, median age at the beginning of the observation 6 years) with MPS (35% type I, 30% type II, 20% type III, 5% type IV, 10% type VI), focusing on their otorhinolaryngologic problems and the impact of surgery on quality of life. We found ear, nose and throat manifestations in all types of MPS; in particular, recurrent otitis media was present in 30% of cases, hearing loss in 75% (mixed in 43.33%, conductive in 43.33%, sensorineural in 13.33%), adenotonsillar hypertrophy in 75%, frequent infections of the upper airway in 75% and obstructive sleep apnoea syndrome in 45% of cases. Fifty percent of patients required surgical therapy (adenotonsillectomy, adenoidectomy with insertion of middle ear ventilation tubes, tonsillectomy, tracheotomy and exeresis of vocal cord polyps). In our experience the ENT surgery reduced the frequency and severity of ear infections and relieved symptoms related to upper airway obstruction, thereby improving the quality of life in affected patients.

  16. Transverse Myelitis as Manifestation of Celiac Disease in a Toddler.

    PubMed

    Krom, Hilde; Sprangers, Fleur; van den Berg, René; Benninga, Marc Alexander; Kindermann, Angelika

    2017-03-01

    We present a 17-month-old girl with rapidly progressive unwillingness to sit, stand, play, and walk. Furthermore, she lacked appetite, vomited, lost weight, and had an iron deficiency. Physical examination revealed a cachectic, irritable girl with a distended abdomen, dystrophic legs with paraparesis, disturbed sensibility, and areflexia. An MRI scan revealed abnormal high signal intensity on T2-weighted images in the cord on the thoracic level, without cerebral abnormalities, indicating transverse myelitis (TM). Laboratory investigations revealed elevated immunoglobulin A antibodies against gliadin (1980.0 kU/L; normal, 0-10.1 kU/L) and tissue transglutaminase (110.0 kU/L; normal, 0-10.1 kU/L). Gastroscopy revealed villous atrophy in the duodenal biopsies and lymphocytic gastritis according to Marsh IIIb, compatible with celiac disease (CD). After the start of a gluten free diet and methylprednisolone, she recovered completely. To our knowledge, this is the first pediatric case of TM as manifestation of CD. We suggest that all children with TM or other neurologic manifestations of unknown origin should be screened for CD.

  17. Neurologic and psychiatric manifestations of celiac disease and gluten sensitivity.

    PubMed

    Jackson, Jessica R; Eaton, William W; Cascella, Nicola G; Fasano, Alessio; Kelly, Deanna L

    2012-03-01

    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 manifestations. 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.

  18. Myopericarditis and severe myocardial dysfunction as the initial manifestation of systemic lupus erythematosus

    PubMed Central

    Peñataro, Joaquín S; De Mingo, Ana; Del Río, Ana; Martínez, José A; Heras, Magda

    2012-01-01

    Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE). However, a large pericardial effusion as the initial manifestation of the disease is infrequent, especially when it is associated with myocardial damage. We describe an unusual case of a young female with pleuropericarditis and severe myocardial dysfunction as the initial manifestation of SLE. PMID:24062915

  19. 8 CFR 251.5 - Paper arrival and departure manifests for crew.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 8 Aliens and Nationality 1 2011-01-01 2011-01-01 false Paper arrival and departure manifests for... REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.5 Paper arrival and... from the United States must submit arrival and departure manifests in a paper format in accordance...

  20. 8 CFR 251.5 - Paper arrival and departure manifests for crew.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 8 Aliens and Nationality 1 2012-01-01 2012-01-01 false Paper arrival and departure manifests for... REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.5 Paper arrival and... from the United States must submit arrival and departure manifests in a paper format in accordance...

  1. Pulmonary manifestations of Sjögren syndrome, systemic lupus erythematosus, and mixed connective tissue disease.

    PubMed

    Mira-Avendano, Isabel C; Abril, Andy

    2015-05-01

    Interstitial lung disease is a common and often life-threatening manifestation of different connective tissue disorders, often affecting its overall prognosis. Systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease, although all unique diseases, can have lung manifestations as an important part of these conditions. This article reviews the different pulmonary manifestations seen in these 3 systemic rheumatologic conditions.

  2. Loss of Coupling Distinguishes GJB1 Mutations Associated with CNS Manifestations of CMT1X from Those Without CNS Manifestations.

    PubMed

    Abrams, Charles K; Goman, Mikhail; Wong, Sarah; Scherer, Steven S; Kleopa, Kleopas A; Peinado, Alejandro; Freidin, Mona M

    2017-01-10

    CMT1X, an X-linked inherited neuropathy, is caused by mutations in GJB1, which codes for Cx32, a gap junction protein expressed by Schwann cells and oligodendrocytes. Many GJB1 mutations cause central nervous system (CNS) abnormality in males, including stable subclinical signs and, less often, short-duration episodes characterized by motor difficulties and altered consciousness. However, some mutations have no apparent CNS effects. What distinguishes mutations with and without CNS manifestations has been unclear. Here we studied a total of 14 Cx32 mutations, 10 of which are associated with florid episodic CNS clinical syndromes in addition to peripheral neuropathy. The other 4 mutations exhibit neuropathy without clinical or subclinical CNS abnormalities. These "PNS-only" mutations (Y151C, V181M, R183C and L239I) form gap junction plaques and produce levels of junctional coupling similar to those for wild-type Cx32. In contrast, mutants with CNS manifestations (F51L, E102del, V139M, R142Q, R142W, R164W T55I, R164Q and C168Y) either form no morphological gap junction plaques or, if they do, produce little or no detectable junctional coupling. Thus, PNS and CNS abnormalities may involve different aspects of connexin function.

  3. Loss of Coupling Distinguishes GJB1 Mutations Associated with CNS Manifestations of CMT1X from Those Without CNS Manifestations

    PubMed Central

    Abrams, Charles K.; Goman, Mikhail; Wong, Sarah; Scherer, Steven S.; Kleopa, Kleopas A.; Peinado, Alejandro; Freidin, Mona M.

    2017-01-01

    CMT1X, an X-linked inherited neuropathy, is caused by mutations in GJB1, which codes for Cx32, a gap junction protein expressed by Schwann cells and oligodendrocytes. Many GJB1 mutations cause central nervous system (CNS) abnormality in males, including stable subclinical signs and, less often, short-duration episodes characterized by motor difficulties and altered consciousness. However, some mutations have no apparent CNS effects. What distinguishes mutations with and without CNS manifestations has been unclear. Here we studied a total of 14 Cx32 mutations, 10 of which are associated with florid episodic CNS clinical syndromes in addition to peripheral neuropathy. The other 4 mutations exhibit neuropathy without clinical or subclinical CNS abnormalities. These “PNS-only” mutations (Y151C, V181M, R183C and L239I) form gap junction plaques and produce levels of junctional coupling similar to those for wild-type Cx32. In contrast, mutants with CNS manifestations (F51L, E102del, V139M, R142Q, R142W, R164W T55I, R164Q and C168Y) either form no morphological gap junction plaques or, if they do, produce little or no detectable junctional coupling. Thus, PNS and CNS abnormalities may involve different aspects of connexin function. PMID:28071741

  4. Connection between classroom abuse and manifest aggressiveness, anxiety and altruism.

    PubMed

    Bilić, Vesna

    2006-12-01

    The objective of this paper is to find out if the students exposed to abuse differ in their level of anxiety, aggressiveness, and altruism from other students, and to test if the pattern of these differences differs depending on whether the abuse they suffer is emotional or physical. The research was carried out on a sample of 127 senior elementary school students. The data was gathered at the end of the 2003/04 school year, and obtained through the respondents' self-statements in questionnaires about childhood abuse and by the scales of manifest aggressiveness, anxiety and altruism. The frequency analysis has shown that various forms of emotional abuse are more common in schools than physical abuse, and that they are reaching disturbing proportions. For example, more than half of the participants in the study reported facing intimidation and threats in school, and over a third of them have been yelled at. Although less commonplace, physical abuse in school can by no means be ignored. Those students who suffer from frequent physical abuse are more dissatisfied with school (r=0.174, p<0.05), display more aggressiveness (r=0.441, p<0.001), and are more often boys (r=0.324, p<0.01). Those students who are frequently emotionally abused are more anxious (r=0.281, p<0.01), dissatisfied with school (r=0.237, p<0.01), and display more manifest aggressiveness (r=398, p<0.01). The discriminant analysis has shown that the bullied students can be differentiated from their non-abused schoolmates as they are manifestly more anxious and aggressive, regardless of whether they suffer physical or emotional abuse. Instances of different forms of emotional and physical classroom abuse have increased alarmingly. Such traumatic experiences affect children's health and functioning in school, as well as in their private lives. The interdisciplinary studies of this phenomenon and the education of all those who work with young people emerge as the top priority in the prevention of this kind

  5. [Rasopathies: developmental disorders that predispose to cancer and skin manifestations].

    PubMed

    Hernández-Martín, A; Torrelo, A

    2011-01-01

    Proteins belonging to the RAS/mitogen activated protein kinase (MAPK) pathway play key roles in cell proliferation, differentiation, survival, and death. For more than 30 years now we have known that 30% of human cancers carry somatic mutations in genes encoding proteins from this pathway. Whereas somatic mutations have a high malignant potential, germline mutations are linked to developmental abnormalities that are often poorly clinically differentiated, although each is dependent upon the specific gene affected. Thus, all patients share varying degrees of mental retardation or learning difficulties, heart disease, facial dysmorphism, skin anomalies, and, in some cases, predisposition to cancer. These syndromes, known as rasopathies, include Noonan syndrome, Costello syndrome, neurofibromatosis-1, LEOPARD syndrome, cardiofaciocutaneous syndrome, and Legius syndrome. Recognizing the skin manifestations of rasopathies can facilitate diagnosis of these syndromes.

  6. Systemic viral infections and their retinal and choroidal manifestations.

    PubMed

    Yoser, S L; Forster, D J; Rao, N A

    1993-01-01

    Viruses are one of the most common causes of infections involving the posterior segment of the eye. Such infections can occur either on a congenital or an acquired basis, and may affect primarily the retina or the choroid. Congenital cytomegalovirus (CMV) and rubella infections may result in retinitis. CMV retinitis is also the most common cause of acquired viral retinitis, primarily because of the acquired immunodeficiency syndrome (AIDS). Other types of viral retinitis, such as those caused by herpes simplex or herpes zoster, can occur in immunocompromised or immunocompetent individuals. Retinitis or choroiditis caused by viruses such as measles, influenza, Epstein-Barr virus, and Rift Valley fever virus, typically occurs subsequent to an acute viral systemic illness. The systemic and ocular manifestations, as well as the histopathology, laboratory tests, differential diagnoses, and treatment regimens for each of the individual viruses are discussed in detail.

  7. The SAPHO syndrome revisited with an emphasis on spinal manifestations.

    PubMed

    Leone, Antonio; Cassar-Pullicino, Victor N; Casale, Roberto; Magarelli, Nicola; Semprini, Alessia; Colosimo, Cesare

    2015-01-01

    The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis.

  8. Physicochemical Properties of Nanomaterials: Implication in Associated Toxic Manifestations

    PubMed Central

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

    2014-01-01

    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

  9. Transient cortical blindness as a manifestation of solitary cysticercus granuloma.

    PubMed

    Hussain, Shabbir; Hussain, Kosar; Hussain, Sahar

    2012-12-10

    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.

  10. Moebius syndrome: clinical manifestations in a pediatric patient.

    PubMed

    Lima, Luciana Monti; Diniz, Michele Baffi; dos Santos-Pinto, Lourdes

    2009-01-01

    Moebius syndrome is a congenital, nonprogressive disorder clinically characterized by loss of facial expression, impaired stomatognathic system functions, incapacity to close the eyelids, and several oral impairments. The purpose of this paper was to present the clinical manifestations and the dental treatment in a 5-year, 2-month-old male Moebius syndrome patient. The child presented with facial asymmetry, difficulty performing facial mimic movements and pronouncing some letters, and compromised suction, mastication, breathing, and deglutition. An intraoral examination revealed hypofunction of the perioral muscles, cheeks and tongue, ankyloglossia, anterior open bite, and absence of carious lesions and dental anomalies. The dental treatment consisted of frenectomy and further placement of a removable orthodontic appliance with a palatal crib for correction of the anterior open bite. After 12 months of follow-up, anterior open bite decreased and speech, deglutition, and mastication improved.

  11. Manifestations of two-dimensional electron gas in molecular crystals

    NASA Astrophysics Data System (ADS)

    Kuklja, Maija M.; Sharia, Onise; Tsyshevsky, Roman

    2017-03-01

    The existence of two-dimensional electron gas in molecular materials has not been reported or discussed. Intriguing properties of two-dimensional electron gas observed on interfaces of polar and nonpolar oxides spurred oxide electronics and advanced nanotechnology. Here we discover how an electrostatic instability occurs on polar surfaces of molecular crystals and explore its manifestations, chemical degradation of surfaces, charge separation, electrical conductivity, optical band-gap closure and surface metallization. A thin layer of polar surface of a dielectric molecular crystal becomes metallic due to interactions of polar molecules. Our findings are illustrated with two polymorphs of cyclotetramethylene-tetranitramine crystals, the polar δ-phase and nonpolar β-phase. Our theory offers an explanation to a relative stability of the β-phase versus the explosive reactivity of δ-phase and to the experimentally observed difference in conductivity of these crystals. We predict that the electrostatic instability takes place on all polar molecular materials.

  12. Visual evoked potential findings in Behcet's disease without neurological manifestations.

    PubMed

    Anlar, Omer; Akdeniz, Necmettin; Tombul, Temel; Calka, Omer; Bilgili, Serap G

    2006-03-01

    Behçet's disease (BD) is a chronic, recurrent multisystem inflammatory disorder firstly described by Turkish dermatologist Dr. Hulusi Behçet in 1937. The classic triad consists of recurrent oral and genital ulcerations and uveitis. The article presents the value of visual evoked potential findings of a series of 44 patients with BD without neurological manifestations seen at the Medical Hospital in Neurology and Dermatology clinics over the past 8 years. The mean latency value of positive peak P100 in BD patients was significantly delayed compared to that of control subjects (patients's mean: 105.6 ms in right eye and 107.7 ms in left eye; control subject's mean: 101.4 ms in right eye and 101.7 ms in left eye).

  13. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation.

    PubMed

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

    2014-01-01

    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.

  14. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation

    PubMed Central

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

    2014-01-01

    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. PMID:25763160

  15. The multiple electrocardiographic manifestations of ventricular repolarization memory.

    PubMed

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

    2014-08-01

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

  16. Clinical and pathological manifestations of human henipavirus infection.

    PubMed

    Wong, K T; Tan, C T

    2012-01-01

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

  17. Rectal prolapse as initial clinical manifestation of colon cancer.

    PubMed

    Chen, C-W; Hsiao, C-W; Wu, C-C; Jao, S-W

    2008-04-01

    Rectal prolapse as the initial clinical manifestation of colorectal cancer is uncommon. We describe the case of a 75-year-old woman who was diagnosed as having adenocarcinoma of the sigmoid colon after presenting with complete rectal prolapse. The tumor caused rectosigmoid intussusception and then it prolapsed out through the anus. She underwent rectosigmoidectomy and rectopexy. The postoperative course was uneventful. The relationship between colorectal cancer and rectal prolapse has not been clearly established. This case report describes an unusual presentation of colorectal cancer. It suggests that rectal prolapse can present as the initial symptom of colorectal cancer and may also be a presenting feature of the occult intra-abdominal pathology. The importance of adequate investigation such as colonoscopy should be emphasized in patients who develop a new onset of rectal prolapse.

  18. Neonatal manifestations of inherited bone marrow failure syndromes.

    PubMed

    Khincha, Payal P; Savage, Sharon A

    2016-02-01

    The inherited bone marrow failure syndromes (IBMFS) are a rare yet clinically important cause of neonatal hematological and non-hematological manifestations. Many of these syndromes, such as Fanconi anemia, dyskeratosis congenita and Diamond-Blackfan anemia, confer risks of multiple medical complications later in life, including an increased risk of cancer. Some IBMFS may present with cytopenias in the neonatal period whereas others may present only with congenital physical abnormalities and progress to pancytopenia later in life. A thorough family history and detailed physical examination are integral to the work-up of any neonate in whom there is a high index of suspicion for an IBMFS. Correct detection and diagnosis of these disorders is important for appropriate long-term medical surveillance and counseling not only for the patient but also for appropriate genetic counselling of their families regarding recurrence risks in future children and generations.

  19. Unilateral lymphocytic pleuritis as a manifestation of familial Mediterranean fever.

    PubMed

    Katsenos, Stamatis; Mermigkis, Charalampos; Psathakis, Kostas; Tsintiris, Kostas; Polychronopoulos, Vlassios; Panagou, Panagiotis; Ritis, Kostas; Light, Richard W

    2008-04-01

    Familial Mediterranean fever (FMF) is an autosomal recessive disease affecting predominantly populations surrounding the Mediterranean basin. It is the most prevalent hereditary periodic fever syndrome characterized mainly by recurrent and short attacks of fever and serositis (pleuritis, arthritis, peritonitis). Unilateral polymorphonuclear exudative pleuritis associated with fever has been reported as the solitary manifestation of the first FMF attack, in < 10% of patients. This case study describes a 30-year-old Greek man with recurrent episodes of lymphocytic exudative pleuritis associated with fever. After a thorough workup (clinical criteria and molecular genetic testing identifying homozygosity polymorphisms of the FMF gene), the diagnosis of FMF was established. Treatment with colchicine, 2 mg/d, eliminated FMF attacks. To our knowledge, this is the first well-documented case report of a patient with FMF presenting with a lymphocytic exudative pleural effusion.

  20. Incidence, pathophysiology, and clinical manifestations of antiphospholipid syndrome.

    PubMed

    Brock, Clifton O'neill; Brohl, Andrew Scott; Običan, Sarah Gloria

    2015-09-01

    Antiphospholipid syndrome (APLS) is a complex systemic disease with a wide variety of clinical manifestations. In the obstetric population, recurrent early pregnancy loss, fetal loss, and thrombosis are hallmarks of the disease. Patients with APLS have developed one or more pathogenic auto-antibodies directed against plasma and cell surface proteins. These antibodies are characterized by their affinity for anionic phospholipids. Interactions between APLS antibodies and their protein targets influence a wide variety of biological systems and signaling pathways, including monocytes, platelets, the complement system, and endothelial cells. While much research is currently directed at understanding the mechanisms involved in this autoimmune disease, the key clinical presentation is the hypercoagulable state resulting in thrombosis occurring in essentially any arterial or venous location, as well as numerous obstetrical complications. Treatment of APLS is generally directed at preventing thrombosis and poor pregnancy outcomes by ameliorating the hypercoagulable state.

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

    PubMed

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

    2012-06-01

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

  2. An oral lesion as the primary clinical manifestation of sarcoidosis.

    PubMed

    Gill, I; Siddiqi, J

    2017-03-16

    An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction. A referral was sent to the respiratory medicine team and a diagnosis was later confirmed of stage II sarcoidosis. This case report highlights the need for clinicians to be aware of all possible causes of oral lesions, including rare manifestations of underlying systemic disease.

  3. Parasomnia with rhythmic movements manifesting as nocturnal tongue biting.

    PubMed

    Tuxhorn, I; Hoppe, M

    1993-06-01

    The case of a healthy 2-year-old girl with repeated nocturnal tongue biting as a result of rhythmic movements of the jaw associated with body rocking in non-REM sleep is described. Parasomnias manifesting with rhythmic, stereotyped movements of the head, trunk and extremities are well described in healthy children. The term rhythmic movement disorders (RMD) was introduced for these repetive movements in sleep which may appear as head banging (jactatio capitis), body rocking or leg rolling. Severe injuries including fractures, subdural effusions and eye injures are reported. Repeated tongue injuries have not been described as a consequence of RMD. The differential diagnosis from nocturnal seizures is crucial to avoid overtreatment of this benign albeit dramatically presenting condition.

  4. MR enterographic manifestations of small bowel Crohn disease.

    PubMed

    Tolan, Damian J M; Greenhalgh, Rebecca; Zealley, Ian A; Halligan, Steve; Taylor, Stuart A

    2010-03-01

    Magnetic resonance (MR) enterography is a clinically useful technique for the evaluation of both intraluminal and extraluminal small bowel disease, particularly in younger patients with Crohn disease. MR enterography offers the advantages of multiplanar capability and lack of ionizing radiation. It allows evaluation of bowel wall contrast enhancement, wall thickening, and edema, findings useful for the assessment of Crohn disease activity. MR enterography can also depict other pathologic findings such as lymphadenopathy, fistula and sinus formation, abscesses, and abnormal fold patterns. Even subtle disease manifestations may be detected when adequate distention of the small bowel is achieved, although endoscopic and double-contrast barium small bowel techniques remain superior in the depiction of changes in early Crohn disease (eg, aphthoid ulceration). Further research will be needed to determine whether MR imaging enhancement patterns may reliably help discriminate between active and inactive disease.

  5. Thrombotic manifestations in SAPHO syndrome. Review of the literature.

    PubMed

    Carranco-Medina, Tatiana Elizabeth; Hidalgo-Calleja, Cristina; Calero-Paniagua, Ismael; Sánchez-González, María Dolores; Quesada-Moreno, Alba; Usategui-Martín, Ricardo; Pérez-Garrido, Laura; Gómez-Castro, Susana; Montilla-Morales, Carlos Alberto; Martínez-González, Olga; Del Pino-Montes, Javier

    2015-01-01

    SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.

  6. [Cutaneous and mucosal manifestations associated with cocaine use].

    PubMed

    Imbernón-Moya, Adrián; Chico, Ricardo; Aguilar-Martínez, Antonio

    2016-06-17

    Complications due to cocaine are a public health problem. The typical cutaneous disease is leukocytoclastic vasculitis and/or thrombotic vasculopathy affecting mainly the ears. No intense systemic involvement is usually present, but there may be several cutaneous, mucosal and systemic manifestations. Other findings associated as arthralgia, neutropaenia or agranulocytosis, low titer positive antinuclear antibodies, antiphospholipid antibody positivity and neutrophil cytoplasmic antibodies against multiple antigens help the diagnosis. This disease requires a clinical suspicion with a clinical history, a complete physical examination and a broad differential diagnosis for an early and correct diagnosis. The course is usually self-limited. In most cases the only treatment is to discontinue the use of cocaine associated with symptomatic treatment, no proven benefit of systemic corticosteroids.

  7. Oral manifestations of thyroid disorders and its management

    PubMed Central

    Chandna, Shalu; Bathla, Manish

    2011-01-01

    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

  8. Neonatal manifestations of inherited bone marrow failure syndromes

    PubMed Central

    Khincha, Payal P.; Savage, Sharon A.

    2015-01-01

    SUMMARY The inherited bone marrow failure syndromes (IBMFS) are a rare yet clinically important cause of neonatal hematological and non-hematological manifestations. Many of these syndromes, such as Fanconi anemia, dyskeratosis congenita and Diamond–Blackfan anemia, confer risks of multiple medical complications later in life, including an increased risk of cancer. Some IBMFS may present with cytopenias in the neonatal period whereas others may present only with congenital physical abnormalities and progress to pancytopenia later in life. A thorough family history and detailed physical examination are integral to the work-up of any neonate in whom there is a high index of suspicion for an IBMFS. Correct detection and diagnosis of these disorders is important for appropriate long-term medical surveillance and counseling not only for the patient but also for appropriate genetic counselling of their families regarding recurrence risks in future children and generations. PMID:26724991

  9. Neurobehavioral manifestations of developmental impairment of the brain

    PubMed Central

    Dubovický, Michal

    2010-01-01

    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

  10. Oral Manifestations of Inflammatory Bowel Diseases: Two Case Reports

    PubMed Central

    Pereira, Manoela Seadi; Munerato, Maria Cristina

    2016-01-01

    Inflammatory bowel diseases (IBD) are known as chronic inflammatory disorders of the digestive tract, represented mainly by Crohn’s disease (CD) and ulcerative colitis (UC). Among the main oral manifestations of IBD are cobblestoning of the oral mucosa, labial swellings with vertical fissures, pyostomatitis vegetans, angular cheilitis, perioral erythema, and glossitis. In this sense, understanding these nosological entities by dentists would help reach early and differential diagnosis. Thus, two case reports are presented and discussed based on theoretical references obtained by a literature review. The first case report refers to an adult patient whose IBD diagnosis was established after stomatological assessment. The second case was a patient with CD diagnosed in childhood with characteristic oral lesions. PMID:26864508

  11. Infertility and miscarriage: common pathways in manifestation and management.

    PubMed

    Agenor, Angena; Bhattacharya, Sohinee

    2015-07-01

    The relationship between miscarriage and fertility is complex. While most healthcare settings treat miscarriage as a problem of subfertility in assisted reproduction units, others believe that miscarriage occurs in super-fertile women. Infertile women undergoing assisted reproduction are at a greater risk of having a miscarriage especially at an advanced age compared with women conceiving naturally. Aberrant expression of immunological factors and chromosomal abnormalities underlie both infertility and miscarriage. Common risk factors include increased maternal age, obesity, smoking, alcohol, pre-existing medical conditions and anatomical abnormalities of the reproductive system. Management pathways of both conditions may be similar with pre-implantation genetic testing and assisted reproductive technology used in both conditions. This paper discusses the synergies and differences between the two conditions in terms of their epidemiology, etiopathogenesis, risk factors and management strategies. The two conditions are related as degrees of severity of reproductive failure with common pathways in manifestation and management.

  12. Splenorenal Manifestations of Bartonella henselae Infection in a Pediatric Patient

    PubMed Central

    Rising, Taylor; Fulton, Nicholas; Vasavada, Pauravi

    2016-01-01

    Bartonella henselae is a bacterium which can cause a wide range of clinical manifestations, ranging from fever of unknown origin to a potentially fatal endocarditis. We report a case of Bartonella henselae infection in a pediatric-aged patient following a scratch from a kitten. The patient initially presented with a prolonged fever of unknown origin which was unresponsive to antibiotic treatment. The patient was hospitalized with worsening fevers and night sweat. Subsequent ultrasound imaging demonstrated multiple hypoechoic foci within the spleen. A contrast-enhanced CT of the abdomen and pelvis was also obtained which showed hypoattenuating lesions in the spleen and bilateral kidneys. Bartonella henselae IgG and IgM titers were positive, consistent with an acute Bartonella henselae infection. The patient was discharged with a course of oral rifampin and trimethoprim-sulfamethoxazole, and all symptoms had resolved following two weeks of therapy. PMID:27127672

  13. Pictorial review of intrathoracic manifestations of progressive systemic sclerosis.

    PubMed

    Al-Jahdali, Hamdan; Rajiah, Prabhakar; Allen, Carolyn; Koteyar, Shyam Sunder; Khan, Ali Nawaz

    2014-10-01

    Intra-thoracic manifestations of progressive systemic sclerosis (PSS) are not well known particularly the imaging features, which forms the basis of accurate and timely diagnosis. The aim of this study is to familiarize the physicians and radiologists with these features. The diagnosis can remain elusive because of the non-specific nature of symptoms which mimic many common conditions. Thus, the diagnosis of PSS can be missed leading to continuous morbidity if the correct imaging is not pursued. The authors examined the records of rheumatology patient referrals of over a 5 year period. A hundred and seventy patients with systemic sclerosis and mixed connective tissue disorders were chosen for detailed study of the imaging available, which form the basis of this review. The images included conventional chest radiographs, digital radiographs computed radiography (CT) and high resolution computed tomography (HRCT). Where applicable computed pulmonary angiography (CTPA) and radionuclide scans were also interrogated.

  14. [Hepatic haemangioma as sistemic manifestation of rheumatoid arthritis].

    PubMed

    Babaeva, A R; Kalinina, E V; Solodenkova, K S

    2015-01-01

    This article presents the clinical data regarding to the incidence of hepatic haemangioma in rheumatoid arthritis (RA) patients. Haemangioma of the liver has been diagnosed by ultrasonography dramatically oftener in RA than in OA: in 8 from 37 RA pts and neither in 120 osteoarthritis pts. Hepatic haemangioma was associated with long standing erosive RA, extra-articular RA manifestations, high levels of rheumatoid factor and ACCP. Moreover in RA pts with hepatic haemangioma we revealed significantly raised levels of pro-inflammatory cytokines, which can impact on the angiogenesis in RA. Obtained clinical data may contribute in the understanding of liver vessels involvement in RA and pose the role of systemic connective tissue lesion and immune inflammation in this process.

  15. Oral and mandibular manifestations in the Ehlers-Danlos syndromes.

    PubMed

    Mitakides, John; Tinkle, Brad T

    2017-02-13

    The Ehlers-Danlos syndromes (EDS) are hereditary disorders that affect the connective tissue and collagen structures in the body. Several types of EDS have been identified. Oral and mandibular structures, which include oral soft tissue, dentition, facial and head pain, and the functioning of the temporomandibular joint (TMJ), are variably affected in the various types of EDS. These various manifestations of EDS have been noted for many years, but newer diagnostic techniques and studies are shedding additional light on the challenges faced by EDS patients in the area of oral and mandibular disorders. Further, the impact of temporomandibular disorder (TMD) on musculoskeletal dysfunction and vice versa, make this an important feature to recognize. Oral and mandibular hypermobility of the TMJ with associated consequences of EDS are noted. These features, diagnostic parameters and treatment procedures are presented. © 2017 Wiley Periodicals, Inc.

  16. [Brown bone tumor as the first manifestation of primary hyperparathyroidism].

    PubMed

    Marcos García, M; Pino Rivero, V; Keituqwa Yáñez, T; Alcaraz Fuentes, M; Trinidad Ruiz, G; Blasco Huelva, A

    2003-01-01

    We report a clinical case of a 26 years old female who had a 2 years evolution chin tumour with hypercalcemia (11.8 mg/dl) and PTH (paratohormone) of 761 pg/ml. She underwent a CT scan and MRI of the mandible, as well as a biopsy followed by excision of the tumour by the maxilofacial surgeons. Our ENT Department asked for a Scintigraphy (Tc99s-mibi) and thoracic-cervical CT, which showed a lesion that turned out to be an adenoma of the lower right parathyroid gland after surgery and pathological examination. The patient suffered a Primary hyperparathyroidism that was the main stimulus for the Brown Tumour made up by macrophagos and multinuclear giant cells, being this the first manifestation of the metabolic disorder. This form of hyperparathyroidism is very rare in the clinic. We do a literature review to establish the differential diagnosis for such pathology.

  17. Pediatric carbamazepine suspension overdose-clinical manifestations and toxicokinetics.

    PubMed

    Perez, Alberto; Wiley, James F

    2005-04-01

    Two toddlers ingested unknown quantities of their older sibling's carbamazepine suspension and rapidly manifested central nervous depression requiring intubation in 1 patient. Coma was the primary clinical finding throughout their care with no anticholinergic syndrome, seizures, or dysrhythmia. Both patients recovered without sequelae within 24 hours. Initial carbamazepine concentrations were 36.6 and 22.7 mg/L. The elimination rates (zero-order kinetic) were approximately 1.4 and 0.75 mg/L per hour. We provide the first toxicokinetic data for carbamazepine suspension overdose in children. We confirm that the oral absorption of suspension carbamazepine is rapid necessitating prompt referral to a health care facility for this exposure.

  18. Effects of manifest ethnic identification on employment discrimination.

    PubMed

    Barron, Laura G; Hebl, Michelle; King, Eden B

    2011-01-01

    Evidence from recent laboratory experiments suggests that ethnic identification can lead to negative evaluations of ethnic minorities (Kaiser & Pratt-Hyatt, 2009). The current research considers the generalizability of these findings to face-to-face interactions in contexts wherein impression management concerns are salient: the workplace hiring process. In a field experiment, Black, Hispanic, and Irish individuals applied for retail jobs with or without visible display of their ethnic identification. Analysis of indicators of formal (e.g., application offering, interview scheduling) and interpersonal discrimination (e.g., interaction length, nonverbal negativity) suggest store personnel interacting with other-race applicants exhibited greater positivity and longer interactions when applicants displayed ethnic identification than when they did not. The findings suggest that psychologists need to understand not only attitudes or intentions expressed in the lab, but also the behavioral consequences of manifest group identity as they unfold in natural environments.

  19. Visual findings as primary manifestations in patients with intracranial tumors

    PubMed Central

    Sefi-Yurdakul, Nazife

    2015-01-01

    AIM To evaluate the visual findings as primary manifestations in patients with intracranial tumors. METHODS The medical charts of the patients with intracranial tumors who initially admitted to the Neuro-ophthalmology and Strabismus Department with ocular complaints between August 1999 and December 2012 were reviewed retrospectively. The detailed clinical history and the findings of neuro-ophthalmologic examination were recorded. Ocular symptoms and signs, the types and locations of intracranial tumors, and the duration of symptoms before the diagnosis were evaluated. RESULTS The mean age of 11 women (61.1%) and 7 men (38.9%) was 42.2±11.0 (range 20-66y) at the time of intracranial tumor diagnosis. Initial symptoms were transient visual obscurations, visual loss or visual field defect in 16 cases (88.9%), and diplopia in 2 cases (11.1%). Neuro-ophthalmologic examination revealed normal optic discs in both eyes of 6 patients (33.3%), paleness, atrophy or edema of optic disc in 12 patients (66.7%), and sixth cranial nerve palsy in 2 patients (11.1%). Visual acuity ranged between normal vision and loss of light perception. Cranial imaging demonstrated craniopharyngioma (n=1), plasmacytoma (n=1), meningioma (n=6; olfactory groove and tuberculum sellae, pontocerebellar angle, anterior cranial fossa, frontal vertex, suprasellar region), and pituitary macroadenoma (n=10). The mean duration between the onset of visual disturbances and the diagnosis of intracranial tumor was 9.8±18mo (range 3d-6y). CONCLUSION The ophthalmologist is frequently the first physician to encounter a patient with clinical manifestations of intracranial tumors that may cause neurological and ocular complications. Neuro-ophthalmologic findings should be carefully evaluated to avoid a delay in the diagnosis of intracranial tumors. PMID:26309882

  20. Cutaneous Manifestations in HTLV-I Positive Blood Donors

    PubMed Central

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

    2013-01-01

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

  1. Skeletal manifestations of tuberculosis in modern human remains.

    PubMed

    Steyn, Maryna; Buskes, Jenifer

    2016-10-01

    Paleopathologists study the presence of diseases in the past and as such have a vast knowledge of skeletal changes associated with different conditions. Tuberculosis is one of the most studied diseases and still remains a major health problem today. Its manifestations in past populations have been extensively described, but less is known about its bony involvement in the post-antibiotic era. The aim of this study was to assess the frequency and manifestations of skeletal lesions in the post-antibiotic era in a South African sample and compare it to that found before the introduction of antibiotics. Skeletons of 205 individuals from modern skeletal collections and who are known to have died from TB were assessed. It was found that 39.2% of all individuals dying in the post-antibiotic era showed skeletal changes that could be associated with TB, while another 27.5% showed nonspecific changes. The highest incidences were found in individuals who died after 1985, when co-infection with HIV and drug resistance became common. While, as expected, vertebral and rib changes were the most common, the number of individuals who showed changes to the skull, and specifically intracranially, was surprising. These could most probably be associated with TB meningitis, although this specific cause of death was noted in only a few individuals. It seems that individuals may be living longer as a result of long-term antibiotic use, leaving more time for lesions to develop. Clin. Anat. 29:854-861, 2016. © 2016 Wiley Periodicals, Inc.

  2. ["Flash" pulmonary edema as a clinical manifestation of renovascular hypertension].

    PubMed

    Kalimonovska-Ostrić, Dimitra; Ivanović, Branislava; Ostrić, Vladimir; Knezivić, Vesna; Stojanov, Vesna; Simić, Dragan

    2003-01-01

    One of the clinical manifestations of renovascular hypertension (RVH) may be a recurrent pulmonary oedema both in the absence or in the presence of systolic left ventricular dysfunction. This type of pulmonary oedema characterized as "flash" pulmonary oedema is ascribed to elevated angiotensin II concentrations with consequent hypertension as well as to volume overload resulting from decreased pressor natriuresis when there are significant stenoses of both or one renal arteries. The investigation included 30 patients with RVH treated by percutaneous transluminal angioplasty of the stenosed renal artery (PTRA) and/or stent implantation (PTR-ST) and 30 patients with surgical resection of the abdominal aortic aneurysm (AAA). The first group was divided in two subgroups according to the etiology of renal artery stenosis (RAS). In the subgroup with fibromuscular dysplasia (FMD) the mean age was 37.5 years, in the subgroup with atherosclerotic renal artery stenosis (ARAS) 54.8 years and in the group with operated AAA 68.6 years. There were more females than males only in the FMD subgroup (10:3). Two patients of the first group experienced pulmonary oedema, both in the subgroup with atherosclerotic renal artery stenosis associated with atherosclerosis of other arteries. Normalization of the blood pressure following PTRA in both and an uncomplicated course after a surgical myocardial revascularization in one of them illustrates the importance of renal revascularization. Pulmonary oedema occurred preoperatively in four out of 30 patients with abdominal aortic aneurysm in whom significant renal artery stenoses coexisted. Two patients died despite surgery, one patient is clinically stable and the medicament treatment of heart failure is inevitable in the fourth with a left ventricular aneurysm following myocardial infarction. The occurrence or recurrence of pulmonary oedema in the absence of other explanation should suggest the possibility of bilateral or unilateral renal

  3. Clinical Features and Extraintestinal Manifestations of Crohn Disease in Children

    PubMed Central

    Lee, Young Ah; Chun, Peter; Hwang, Eun Ha; Mun, Sang Wook; Lee, Yeoun Joo

    2016-01-01

    Purpose The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. Methods The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. Results Fifty-two (71.2%) of the patients were males. The mean age of the patients was 12.5 years. The mean follow-up period was 3.4 years. The disease location was ileal in 3 (4.1%) of the patients, colonic in 13 (17.8%), ileocolonic in 56 (76.7%). The clinical behavior was inflammatory in 62 (84.9%) of the patients, stricturing in 8 (11.0%), and penetrating in 3 (4.1%). Perianal abscesses or fistulas were found in 37 (50.7%) of the patients. EIMs observed during the study period were anal skin tag in 25 patients (34.2%), hypertransaminasemia in 20 (27.4%), peripheral arthritis in 2 (2.7%), erythema nodosum in 2 (2.7%), vulvitis in 1 (1.4%), uveitis in 1 (1.4%), and pulmonary thromboembolism in 1 (1.4%). Conclusion Perianal diseases and manifestations were present in more than half of Korean pediatric CD patients at diagnosis. Inspection of the anus should be mandatory in Korean children with suspicious CD, as perianal fistulas, abscesses, and anal skin tags may be the first clue to the diagnosis of CD. PMID:28090468

  4. The Manifestations of Sleep Disturbances 16 Years Post-Trauma

    PubMed Central

    Thordardottir, Edda Bjork; Hansdottir, Ingunn; Valdimarsdottir, Unnur Anna; Shipherd, Jillian C.; Resnick, Heidi; Gudmundsdottir, Berglind

    2016-01-01

    Study Objectives: Limited data exist on the association between trauma and sleep across developmental stages, particularly trauma experienced in childhood and sleep in adulthood. We assessed sleep quality across the developmental spectrum among avalanche survivors 16 years after exposure as compared to a matched comparison cohort. Methods: Participants were survivors of two avalanche-affected towns (n = 286) and inhabitants of non-exposed towns (n = 357). Symptoms were assessed with respect to the survivors' developmental stage at the time of the disaster: childhood (2–12), adolescence (13–19), young adult (20–39), and adult (≥ 40). The Posttraumatic Diagnostic Scale, Pittsburgh Sleep Quality Index and Pittsburgh Sleep Quality Index PTSD Addendum were used. Results: Overall PTSD symptoms were not associated with avalanche exposure in any age groups under study. However, survivors who were children at the time of the disaster were 2.58 times (95% CI 1.33–5.01) more likely to have PTSD-related sleep disturbances (PSQI-A score ≥ 4) in adulthood than their non-exposed peers, especially symptoms of acting out dreams (aRR = 3.54; 95% CI 1.15–10.87). Those who were adults at time of the exposure had increased risk of trauma-related nightmares (aRR = 2.69; 95% CI 1.07–6.79 for young adults aRR = 3.07; 95% CI 1.51–6.24 for adults) compared to their non-exposed peers. Conclusions: Our data indicate a chronicity of PTSD-related sleep disturbances, particularly among childhood trauma survivors. REM sleep disturbances may have different manifestations depending on the developmental stage at the time of trauma exposure. Citation: Thordardottir EB, Hansdottir I, Valdimarsdottir UA, Shipherd JC, Resnick H, Gudmundsdottir B. The manifestations of sleep disturbances 16 years post-trauma. SLEEP 2016;39(8):1551–1554. PMID:27166232

  5. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome

    PubMed Central

    Leal, Inês; Faria, Mun Yueh; Pinto, Luís Abegão

    2016-01-01

    ABSTRACT Aims: To report a case of a patient who developed uveitis-glaucoma-hyphema (UGH) syndrome after an uneventful cataract surgery and to discuss risk factors, diagnostic challenges, management options, and clinical implications. Background: Uveitis-glaucoma-hyphema syndrome is a rare but potentially serious cataract surgery complication. Clinical manifestations include increased intraocular pressure (IOP), anterior chamber inflammation, and recurrent hyphema or microhyphema. Uveitis-glaucoma-hyphema Plus syndrome also includes accompanying vitreous hemorrhage. Although classically associated with rigid anterior chamber intraocular lenses (lOLs), cases of malpositioning and subluxated posterior chamber lOLs have also been described as possible triggers. Case description: We report a case of a 70-year-old Caucasian man who developed UGH Plus syndrome after an uneventful cataract surgery with an lOL implanted in the capsular bag. During postoperative follow-up, persistent intraocular inflammation, increased IOP, hyphema, and vitreous hemorrhage were consistent with this diagnosis. Slit-lamp examination demonstrated progressive localized iris atrophy, compatible with chafing of the posterior iris by the IOL haptic as the trigger for UGH syndrome. A pars plana vitrectomy was performed and a retropupillary intraocular lens was implanted. No further complications occurred during follow-up. Conclusion and clinical significance: Given the increasing prevalence of single-piece lOLs implanted in the capsular bag, it is important to recognize UGH syndrome as a rare but potentially serious complication. How to cite this article: Sousa DC, Leal I, Faria MY, Pinto LA. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome. J Curr Glaucoma Pract 2016;10(2):76-78. PMID:27536051

  6. Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

    PubMed Central

    Alkhotani, Ashjan; Shirah, Bader

    2016-01-01

    Background and Purpose Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. Methods A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. Results In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. Conclusions This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes. PMID:27819416

  7. Ophthalmic manifestations of tuberous sclerosis: a population based study

    PubMed Central

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

    2001-01-01

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

 PMID:11264130

  8. Allergic Interstitial Nephritis Manifesting as a Striated Nephrogram

    PubMed Central

    Moinuddin, Irfan; Bracamonte, Erika; Thajudeen, Bijin; Sussman, Amy; Madhrira, Machaiah; Costello, James

    2015-01-01

    Allergic interstitial nephritis (AIN) is an underdiagnosed cause of acute kidney injury (AKI). Guidelines suggest that AIN should be suspected in a patient who presents with an elevated serum creatinine and a urinalysis that shows white cells, white cell casts, or eosinophiluria. Drug-induced AIN is suspected if AKI is temporally related to the initiation of a new drug. However, patients with bland sediment and normal urinalysis can also have AIN. Currently, a definitive diagnosis of AIN is made by renal biopsy which is invasive and fraught with risks such as bleeding, infection, and hematoma. Additionally, it is frequently unclear when a kidney biopsy should be undertaken. We describe a biopsy proven case of allergic interstitial nephritis which manifested on contrast enhanced Magnetic Resonance Imaging (MRI) as a striated nephrogram. Newer and more stable macrocyclic gadolinium contrast agents have a well-demonstrated safety profile. Additionally, in the presentation of AKI, gadolinium contrast agents are safe to administer in patients who demonstrate good urine output and a downtrending creatinine. We propose that the differential for a striated nephrogram may include AIN. In cases in which the suspicion for AIN is high, this diagnostic consideration may be further characterized by contrast enhanced MRI. PMID:26664405

  9. Progeria: pathogenesis and oral manifestation--a review.

    PubMed

    Saigal, S; Bhargava, A

    2012-01-01

    Our life span is genetically programmed and it is possible that a defect in produced proteins encoded by the longevity gene is a cause of aging. Progeria which is a rare, fatal genetic condition which affects between one in four million and one in eight million children of both sexes equally and characterized by premature and accelerated aging. The appearance and physiology of these children resembles to elderly people but they typically have life span to their mid teens. It is also known as the Hutchinson-Gilford syndrome, which was initially reported by Johnathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. It is an autosomal recessive disorder, which means an individual has inherited a mutated gene from both parents. It is added to the expanding catalogue of laminopathies, diseases caused by mutations affecting nuclear lamina proteins known as lamin A (LMNA). In oral manifestation primary finding is micrognathia with delayed tooth eruption and incomplete formation of root of permanent tooth. Presently there are no known cures for this abnormality.

  10. Multiple cavitating pulmonary nodules: rare manifestation of benign metastatic leiomyoma

    PubMed Central

    Choi, Youn-I; Lee, Seok Jeong; Shim, Sung Shine; Lee, Jin Hwa; Kim, Yoo Kyung; Sung, Soon Hee

    2017-01-01

    Benign metastasizing leiomyoma (BML) is a rare disease of pathologically benign, but the tumor metastasizes to other organs. The most common organ involved in BML is lung. Pulmonary involvement usually manifested as multiple nodules on chest X-ray, however we experienced an interesting case of a 52-year-old premenopausal woman who presented with multiple bilateral lung cavitations and nodules on a chest X-ray without any respiratory symptoms. Chest computed-tomography identified multiple cavitary lesions of 5–12 mm in diameter and well-defined nodules of 5–10 mm in diameter in both lung fields. Transthoracic needle biopsy was performed and the resected lesion consisted of benign spindle cells was positive for estrogen receptor (ER) and progesterone receptor (PR) and was diffuse positive for actin and desmin by immunohistochemical (IHC) staining, suggesting leiomyoma. The final diagnosis was benign pulmonary metastasizing leiomyoma (BPML) and the patient underwent subcutaneous injection of a gonadotrophin releasing hormone (GnRH) agonist for 12 months, follow-up low-dose chest computed tomography (CT) scan at 15 months revealed decreased cavitations and nodular lesions. We should take into consideration the possibility of BPML when we encounter multiple cystic or cavitary lesions on chest X-ray, although the common form of BPML is nodulary lesions on imaging studies. PMID:28203428

  11. Neuropsychiatric manifestations in inflammatory neuropathies: A systematic review

    PubMed Central

    Seri, Stefano; Cavanna, Andrea E.

    2016-01-01

    ABSTRACT We conducted a systematic literature review on psychological and behavioral comorbidities in patients with inflammatory neuropathies. In Guillain–Barré syndrome (GBS), psychotic symptoms are reported during early stages in 30% of patients. Typical associations include mechanical ventilation, autonomic dysfunction, inability to communicate, and severe weakness. Anxiety and depression are frequent comorbidities. Anxiety may increase post‐hospital admissions and be a predictor of mechanical ventilation. Posttraumatic stress disorder may affect up to 20% of ventilated patients. Sleep disturbances are common in early‐stage GBS, affecting up to 50% of patients. In chronic inflammatory demyelinating polyradiculoneuropathy, memory and quality of sleep may be impaired. An independent link between depression and pretreatment upper limb disability and ascites was reported in POEMS (polyneuropathy, organomegaly, endocrinopathy, M‐protein, skin) syndrome, with an association with early death. Hematological treatment of POEMS appears effective on depression. Published literature on psychological/behavioral manifestations in inflammatory neuropathies remains scarce, and further research is needed. Muscle Nerve 54: 1–8, 2016 PMID:26999767

  12. Apparent mineralocorticoid excess: time of manifestation and complications despite treatment.

    PubMed

    Knops, Noël B B; Monnens, Leo A; Lenders, Jacques W; Levtchenko, Elena N

    2011-06-01

    Here we describe the case of a patient followed from birth because of a positive family history for apparent mineralocorticoid excess (AME) in an older brother. The patient, a girl, had normal serum electrolyte and blood pressure measurements in the first months after birth. Not until the age of 11 months did she develop anorexia and failure to thrive in combination with hypertension, hypokalemia, and metabolic alkalosis, which are consistent with the diagnosis of AME. This diagnosis was confirmed by mutation analysis of the HSD11B2 gene (C1228T). Treatment with amiloride and furosemide electrolyte disturbances normalized her blood pressure. At the age of 19 years she unexpectedly suffered a stroke. Additional investigations revealed no accepted risk factor for stroke. We discuss the possible underlying mechanisms for the delayed manifestation of hypertension and electrolyte disturbances in AME, propose an additional explanation for the stroke in this patient, and advise treatment with a mineralocorticoid receptor antagonist to reduce stroke risk in patients with AME.

  13. Severe congenital thrombocytopaenia--first clinical manifestation of Noonan syndrome.

    PubMed

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-03-20

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×10(9)/l).The authors registered a persistent low platelet count (9000-129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan's syndrome.

  14. Cytomegalovirus pneumonia as the first manifestation of severe combined immunodeficiency

    PubMed Central

    Jończyk-Potoczna, Katarzyna; Ossowska, Lidia; Bręborowicz, Anna; Bartkowska-Śniatkowska, Alicja; Wachowiak, Jacek

    2014-01-01

    Severe combined immunodeficiency (SCID) is characterized by the absence of functional T lymphocytes and impairment of adaptive immunity. While heterogeneity of the genetic background in SCID leads to the variability of immune phenotypes, most of affected newborns appear healthy but within the first few months they develop life-threatening opportunistic respiratory or gastrointestinal tract infections. The objective of the study was to define the presenting features and etiology of infections in children with SCID. We retrospectively reviewed five children in whom the diagnosis of SCID had been established in our pediatric immunology clinic over the last 10-year period. A viral respiratory tract infection was the first manifestation of SCID in all the children studied. Cytomegalovirus (CMV) pneumonia was recognized in as many as 4 cases and coronavirus pulmonary infection was diagnosed in one case, whereas Pneumocystis jiroveci was identified as a co-pathogen in one CMV-infected patient. Severe combined immunodeficiency is a pediatric emergency condition and given the significant impact of pulmonary CMV infection in SCID children, establishing an accurate etiological diagnosis is of essential importance in instituting the specific treatment and improving the outcome. PMID:26155153

  15. Severe congenital thrombocytopaenia – first clinical manifestation of Noonan syndrome

    PubMed Central

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-01-01

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×109/l).The authors registered a persistent low platelet count (9000–129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan’s syndrome. PMID:22605701

  16. Early manifestations of replicative aging in the yeast Saccharomyces cerevisiae

    PubMed Central

    Sorokin, Maksim I.; Knorre, Dmitry A.; Severin, Fedor F.

    2014-01-01

    The yeast Saccharomyces cerevisiae is successfully used as a model organism to find genes responsible for lifespan control of higher organisms. As functional decline of higher eukaryotes can start as early as one quarter of the average lifespan, we asked whether S. cerevisiae can be used to model this manifestation of aging. While the average replicative lifespan of S. cerevisiae mother cells ranges between 15 and 30 division cycles, we found that resistances to certain stresses start to decrease much earlier. Looking into the mechanism, we found that knockouts of genes responsible for mitochondria-to-nucleus (retrograde) signaling, RTG1 or RTG3, significantly decrease the resistance of cells that generated more than four daughters, but not of the younger ones. We also found that even young mother cells frequently contain mitochondria with heterogeneous transmembrane potential and that the percentage of such cells correlates with replicative age. Together, these facts suggest that retrograde signaling starts to malfunction in relatively young cells, leading to accumulation of heterogeneous mitochondria within one cell. The latter may further contribute to a decline in stress resistances.

  17. Gendered Manifestations of Depression and Help Seeking Among Men.

    PubMed

    Call, Jarrod B; Shafer, Kevin

    2015-12-31

    Men who do not seek help for mental health problems may experience unnecessary suffering which ultimately affects the well-being of themselves and others. Gendered manifestations of depressive symptoms may play an important role in why some men do not seek help for mental health issues. Using data from 2,382 male respondents in the National Comorbidity Survey Replication, the authors examined the relationship that both traditional and male-typical symptoms of depression had on the help-seeking behaviors of men. Traditional symptoms increased the odds of seeking help for depression for all men. Male-typical symptoms, however, did not increase the odds of seeking help for depression or another mental health concern. Both traditional and male-typical symptoms increased the odds of initially seeking help from a medical provider, and men with male-typical symptoms had an overall higher likelihood of seeking help from a medical provider. Consequently, it is important that medical professionals assess for depression even when it is not a presenting concern.

  18. Cat-scratch disease: ocular manifestations and visual outcome.

    PubMed

    Curi, André L L; Machado, Danuza; Heringer, Gustavo; Campos, Wesley Ribeiro; Lamas, Cristiane; Rozental, Tatiana; Gutierres, Alexandro; Orefice, Fernando; Lemos, Elba

    2010-10-01

    To describe the intra-ocular manifestations of cat-scratch disease (CSD) found at two uveitis reference centers in Brazil. Retrospective case series study. Review of clinical records of patients diagnosed with CSD in the Uveitis Department of São Geraldo Hospital and the Ophthalmology Department of the Instituto de Pesquisa Clínica Evandro Chagas-FIOCRUZ, from 2001 to 2008. In the 8-year period, 24 patients with the diagnosis of CSD were identified. Twelve patients were male and 12 female. The mean age was 27.04 years (range 7-56). Sixteen patients (66.6%) presented with a history of a cat scratch and all patients reported cat exposure. Visual acuity ranged from counting fingers to 1.0 in the affected eye. Thirteen patients presented with bilateral disease. Sixteen (66.6%) patients complained of systemic symptoms, including fever, lymphadenopathy, liver and spleen enlargement and rash. All patients presented with serum antibodies (IgG) to Bartonella henselae. Thirty-seven eyes were affected. The most common findings were small areas of retinal infiltrates which occurred in 11 eyes (29.7%) and angiomatous lesions which occurred in nine eyes (24.3%). Neuroretinitis occurred in only six eyes (16.2%). The most common findings of CSD in our study were retinal infiltrates and angiomatous lesions. CSD patients may present with significant visual loss. Patients may benefit from systemic treatment with antibiotics.

  19. Neuropsychiatric Manifestation of Hashimoto's Encephalopathy in an Adolescent and Treatment

    PubMed Central

    Ransing, Ramdas Sarjerao; Mishra, Kshirod Kumar; Sarkar, Dipayan

    2016-01-01

    Hashimoto's encephalopathy is usually underdiagnosed and untreated because of complex neuropsychiatric manifestation. We report a case of an adolescent female with Hashimoto's encephalopathy who responded well to a combination of aspirin and levothyroxine. A 16-year-old girl presented at psychiatric emergency services with a depressive episode, menstrual irregularities, and a 5-month past history of thyroid swelling. On clinical examination, she was in a euthyroid state with insignificant neurological history. However, her previous investigation revealed a hypothyroid state. Her magnetic resonance imaging findings demonstrated infarcts in the bilateral gangliocapsular region and left frontal periventricular deep white matter lesion. Ultrasonography of the thyroid and fine needle aspiration cytology confirmed lymphocytic thyroiditis. Anti-thyroid peroxidase (289 IU/ml) antibody titer was elevated (289 IU/mL). Her depressive symptoms responded well to antidepressants, mood stabilizers, nonsteroidal anti-inflammatory drugs, and levothyroxine. She remained in the euthyroid state and then in the euthymic state for 3 years. Hashimoto's encephalopathy is steroid-responsive encephalopathy. Most researchers have observed a dramatic response to steroids with or without levothyroxine. A clinician may consider aspirin as an alternative to a steroid in long-term management to avoid steroid-related side effects and contraindications. PMID:27570351

  20. The Multiple Electrocardiographic Manifestations of Ventricular Repolarization Memory

    PubMed Central

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

    2014-01-01

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

  1. Structural manifestations of aging in Se-rich glasses

    NASA Astrophysics Data System (ADS)

    Dash, S.; Ravindren, S.; Chen, P.; Boolchand, P.

    2015-03-01

    We examine weakly cross-linked GexSe100-x (0%manifested. Work supported by NSF Grant DMR 08-53957.

  2. [Widespread gastrointestinal CMV infection as the presenting manifestation of AIDS].

    PubMed

    Dayan, K; Neufeld, D M; Lang, R; Novis, B; Bernheim, J; Freund, U

    1993-02-01

    A 53-year-old man is reported who developed a widespread gastrointestinal infection due to cytomegalic (CMV) virus and was found to be suffering from AIDS. He died of overwhelming pulmonary infection. There is need for awareness of the rapid increase of AIDS in our local population, particularly in groups not regarded as at high risk. It is imperative to keep this diagnosis in mind when dealing with patients with gastroenterocolitis not responding to standard treatments. CMV has a broad spectrum of clinical manifestations, from carrier state to life-threatening infection. In the gastrointestinal system it causes inflammation and ulcers in the mucosa that may bleed or perforate. There are increasing numbers of reports of CMV ileocolitis in homosexuals with AIDS. The rate of sero-positive CMV in healthy homosexual populations is 94-100% and in 14% there is active infection. CMV is the main infective agent in patients suffering from AIDS; 90% will develop an infection with this virus and in most cases it will be fatal.

  3. Orthopaedic manifestations of neurofibromatosis in children: an update.

    PubMed

    Vitale, Michael G; Guha, Abhijit; Skaggs, David L

    2002-08-01

    Neurofibromatosis is one of the most common genetic disorders affecting mankind. Despite extensive basic science research, the diagnosis still is based largely on well-defined clinical criteria, which often present gradually during childhood. Approximately 50% of patients have significant musculoskeletal manifestations, with scoliosis and congenital pseudarthrosis of the tibia most common. Approximately 20% of children with Type I neurofibromatosis present with scoliosis with or without the classic dystrophic features, such as vertebral scalloping and rib penciling. Dystrophic curves portend rapid progression and require early fusion. Surgical treatment often is challenging because of the common presence of neurofibromas adjacent to the spinal cord, significant multiplanar deformity, and poor bone quality. Congenital pseudarthrosis of the tibia also continues to present significant difficulties. The use of a brace is the mainstay of early treatment, whereas intramedullary rodding commonly is used for operative fixation. Grafting of the free fibula and correction using techniques of distraction and compression histiogenesis with Ilizarov fixators have been reported for refractory cases with varying degrees of success. Multiple heroic, operative attempts may have a tremendous toll on the quality of life of affected children through their early childhood. In addition to these and other distinctive musculoskeletal lesions, affected children often suffer from various medical problems.

  4. Multisystem Radiologic Manifestations of Erdheim-Chester Disease

    PubMed Central

    Lodhi, Umairullah; Sarmast, Uzair; Khan, Saadullah; Yaddanapudi, Kavitha

    2016-01-01

    Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans' cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum), and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease. PMID:27340583

  5. Neuropsychiatric manifestations in inflammatory neuropathies: A systematic review.

    PubMed

    Rajabally, Yusuf A; Seri, Stefano; Cavanna, Andrea E

    2016-06-01

    We conducted a systematic literature review on psychological and behavioral comorbidities in patients with inflammatory neuropathies. In Guillain-Barré syndrome (GBS), psychotic symptoms are reported during early stages in 30% of patients. Typical associations include mechanical ventilation, autonomic dysfunction, inability to communicate, and severe weakness. Anxiety and depression are frequent comorbidities. Anxiety may increase post-hospital admissions and be a predictor of mechanical ventilation. Posttraumatic stress disorder may affect up to 20% of ventilated patients. Sleep disturbances are common in early-stage GBS, affecting up to 50% of patients. In chronic inflammatory demyelinating polyradiculoneuropathy, memory and quality of sleep may be impaired. An independent link between depression and pretreatment upper limb disability and ascites was reported in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin) syndrome, with an association with early death. Hematological treatment of POEMS appears effective on depression. Published literature on psychological/behavioral manifestations in inflammatory neuropathies remains scarce, and further research is needed. Muscle Nerve 54: 1-8, 2016.

  6. Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis.

    PubMed

    Haugaa, Kristina H; Haland, Trine F; Leren, Ida S; Saberniak, Jørg; Edvardsen, Thor

    2016-07-01

    This review aims to give an update on the pathogenesis, clinical manifestations, and diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). Arrhythmogenic right ventricular cardiomyopathy is mainly an autosomal dominant inherited disease linked to mutations in genes encoding desmosomes or desmosome-related proteins. Classic symptoms include palpitations, cardiac syncope, and aborted cardiac arrest due to ventricular arrhythmias. Heart failure may develop in later stages. Diagnosis is based on the presence of major and minor criteria from the Task Force Criteria revised in 2010 (TFC 2010), which includes evaluation of findings from six different diagnostic categories. Based on this, patients are classified as having possible, borderline, or definite ARVC. Imaging is important in ARVC diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting structural and functional abnormalities, but importantly these findings may occur after electrical alterations and ventricular arrhythmias. Electrocardiograms (ECGs) and signal-averaged ECGs are analysed for depolarization and repolarization abnormalities, including T-wave inversions as the most common ECG alteration. Ventricular arrhythmias are common in ARVC and are considered a major diagnostic criterion if originating from the RV inferior wall or apex. Family history of ARVC and detection of an ARVC-related mutation are included in the TFC 2010 and emphasize the importance of family screening. Electrophysiological studies are not included in the diagnostic criteria, but may be important for differential diagnosis including RV outflow tract tachycardia. Further differential diagnoses include sarcoidosis, congenital abnormalities, myocarditis, pulmonary hypertension, dilated cardiomyopathy, and athletic cardiac adaptation, which may mimic ARVC.

  7. Stability and its manifestation in the chemical and biological worlds.

    PubMed

    Pascal, Robert; Pross, Addy

    2015-11-21

    Bridging between the phenomenologically distinct biological and physical worlds has been a major scientific challenge since Boltzmann's probabilistic formulation of the second law of thermodynamics. In this review we summarize our recent theoretical attempts to bridge that divide through analysis of the thermodynamic-kinetic interplay in chemical processes and the manner in which that interplay impacts on material stability. Key findings are that the term 'stability' manifests two facets - time and energy - and that stability's time facet, expressed as persistence, is more general than its energy facet. That idea, together with the proposed existence of a logical law of nature, the persistence principle, leads to the mathematically-based insight that stability can come about through either Boltzmann's probabilistic considerations or Malthusian kinetics. Two mathematically-based forms of material persistence then lead directly to the physical likelihood of two material forms, animate and inanimate. Significantly, the incorporation of kinetic considerations into the stability concept appears to bring us closer to enabling two of the central theories in science - the second law of thermodynamics and Darwin's theory of evolution - to be reconciled within a single conceptual framework.

  8. Exertional rhabdomyolysis: physiological response or manifestation of an underlying myopathy?

    PubMed Central

    Scalco, Renata S; Snoeck, Marc; Quinlivan, Ros; Treves, Susan; Laforét, Pascal; Jungbluth, Heinz; Voermans, Nicol C

    2016-01-01

    Exertional rhabdomyolysis is characterised by muscle breakdown associated with strenuous exercise or normal exercise under extreme circumstances. Key features are severe muscle pain and sudden transient elevation of serum creatine kinase (CK) levels with or without associated myoglobinuria. Mild cases may remain unnoticed or undiagnosed. Exertional rhabdomyolysis is well described among athletes and military personnel, but may occur in anybody exposed to unaccustomed exercise. In contrast, exertional rhabdomyolysis may be the first manifestation of a genetic muscle disease that lowers the exercise threshold for developing muscle breakdown. Repeated episodes of exertional rhabdomyolysis should raise the suspicion of such an underlying disorder, in particular in individuals in whom the severity of the rhabdomyolysis episodes exceeds the expected response to the exercise performed. The present review aims to provide a practical guideline for the acute management and postepisode counselling of patients with exertional rhabdomyolysis, with a particular emphasis on when to suspect an underlying genetic disorder. The pathophysiology and its clinical features are reviewed, emphasising four main stepwise approaches: (1) the clinical significance of an acute episode, (2) risks of renal impairment, (3) clinical indicators of an underlying genetic disorders and (4) when and how to recommence sport activity following an acute episode of rhabdomyolysis. Genetic backgrounds that appear to be associated with both enhanced athletic performance and increased rhabdomyolysis risk are briefly reviewed. PMID:27900193

  9. Temporomandibular Joint Hypermobility Manifestation Based on Clinical Observations

    PubMed Central

    Nosouhian, Saeid; Haghighat, Abbas; Mohammadi, Iman; Shadmehr, Elham; Davoudi, Amin; Badrian, Hamid

    2015-01-01

    Background: Joint range of motion might affected by some factors like laxity and increase joint mobility. Generalized joint hypermobility and temporomandibular joint hypermobility (TMJH) are reported as risk factors for temporomandibular disorders. The aim of this study was to survey the etiological factors of TMJH and its relations to habitual status. Materials and Methods: In this cross-sectional descriptive study, 69 patients with TMJH were involved. After profiling personal information and medical history, the patients were divided into three groups based on their maximum mouth opening (MMO) as follow: (Light) MMO of 50-55 mm, (moderate): MMO between 55 and 65 mm, (severe) MMO >65 mm. For subjective observations, patients were asked to fill the prepared questionnaire. The objective evaluations conducted by a specialist. Finally, all the data subjected Chi-Square test by using SPSS software version 22 at a significant level of 0.05. Results: TMJH was more common in women (74.2%). The light group had significant differences with other groups in the discomfort of TMJ and TMJ sound (P < 0.05). Furthermore, sever group manifested highest percentage of masticatory pains, significantly (P < 0.05). Conclusion: It can be concluded that pain in TMJ would have a correlation with MMO. PMID:26464530

  10. Isolated anaemia as a manifestation of Rh isoimmunisation.

    PubMed

    Roda, Juliana; Mimoso, Gabriela; Benedito, Manuela; Pereira, Dolores Faria

    2012-01-18

    Rh isoimmunisation leads to haemolytic anaemia and hyperbilirubinaemia in the first h of life. Isolated early onset neonatal anaemia has rarely been reported. The authors describe the case of a term infant, born to an 'A' negative, second gravida mother. On the second day of life, pallor was noticed. His haemoglobin (Hb) was 6.8 g/dl, he had reticulocytosis and a positive direct antiglobulin test. However, he did not have a high total serum bilirubin (TSB) (87.2 μmol/l). He was transfused with red blood cells and kept under phototherapy for 3 days. Three weeks later, he received another transfusion for severe anaemia (Hb 6 5 g/dl). During this period, he was never jaundiced and the maximum level of TSB was 122 μmol/l. On follow-up, his Hb stabilised and he had no further problems. This report highlights the possibility of early onset anaemia without jaundice as the sole manifestation of Rh isoimmunisation.

  11. Clinical manifestations and oral findings in Fraser syndrome.

    PubMed

    Diniz, Michele Baffi; Lima, Luciana Monti; Sacono, Nancy Tomoko; de Paula, Andréia Bolzan; dos Santos-Pinto, Lourdes

    2007-01-01

    This article is the first known case report of Fraser syndrome in the dental literature. Its purpose was to present the clinical manifestations, oral findings, and dental treatment of a 14-year, 10-month-old female patient. Fraser syndrome is a rare recessive autosomal genetic disorder characterized by multisystemic malformation, usually comprising cryptophthalmos, syndactyly, and renal defects. The child presented with: (1) hydrocephaly; (2) face asymmetry; (3) low-inserted ears; (4) flat nose bridge; (5) cryptophthalmos; (6) bilateral absence of eyeballs; (7) hypertelorism; (8) syndactyly on the left fingers and toes; (9) skeletal defects; and (10) lower limb asymmetry. The intraoral examination revealed: (1) complete primary denture; (2) malocclusion; (3) tooth crowding; (4) ogival palate; (5) normal labial frena; (6) absence of lingual frenum (not compromising the tongue movements); (7) parched lips; (8) supragingival calculus adhered to all tooth surfaces; and (9) moderate gingivitis. The dental treatment consisted of periodic monitoring of the patient's oral health status and supragingival scaling associated with topical applications of 0.12% chlorhexidine digluconate gel at 2-week intervals to reduce gingivitis.

  12. Fraser syndrome-oral manifestations and a dental care protocol.

    PubMed

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

    2014-01-01

    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).

  13. Neurological Manifestations in Parry–Romberg Syndrome: 2 Case Reports

    PubMed Central

    Vix, Justine; Mathis, Stéphane; Lacoste, Mathieu; Guillevin, Rémy; Neau, Jean-Philippe

    2015-01-01

    Abstract Parry–Romberg syndrome (PRS) is a variant of morphea usually characterized by a slowly progressive course. Clinical and radiological involvement of the central nervous system may be observed in PRS. We describe 2 patients with PRS and neurological symptoms (one with trigeminal neuralgia associated with deafness, and the second with hemifacial pain associated with migraine without aura) in conjunction with abnormal cerebral MRI including white matter T2 hyperintensities and enhancement with gadolinium. Despite the absence of specific immunosuppressive treatments, both patients have presented stable imaging during follow-up without any clinical neurologic progression. We have performed a large review of the medical literature on patients with PRS and neurological involvement (total of 129 patients) Central nervous system involvement is frequent among PRS patients and is inconsistently associated with clinical abnormalities. These various neurological manifestations include seizures, headaches, movement disorders, neuropsychological symptoms, and focal symptoms. Cerebral MRI may reveal frequent abnormalities, which can be bilateral or more often homolateral to the skin lesions, localized or so widespread so as to involve the whole hemisphere: T2 hyperintensities, mostly in the subcortical white matter, gadolinium enhancement, brain atrophy, and calcifications. These radiological lesions do not usually progress over time. Steroids or immunosuppressive treatments are controversial since it remains unclear to what extent they are beneficial and there is often no neurological progression. PMID:26181554

  14. Texas Occurrence of Lyme Disease and Its Neurological Manifestations.

    PubMed

    Dandashi, Jad A; Nizamutdinov, Damir; Dayawansa, Samantha; Fonkem, Ekokobe; Huang, Jason H

    2016-06-01

    Today, Lyme disease is the most commonly reported tick-borne disease in the United States and Europe. The culprits behind Lyme disease are the Borrelia species of bacteria. In the USA, Borrelia burgdorferi causes the majority of cases, while in Europe and Asia Borrelia afzelii and Borrelia garinii carry the greatest burden of disease. The clinical manifestations of Lyme disease have been identified as early localized, early disseminated, and late chronic. The neurological effects of Lyme disease include both peripheral and central nervous systems involvement, including focal nerve abnormalities, cranial neuropathies, painful radiculoneuritis, meningitis, and/or toxic metabolic encephalopathy, known as Lyme encephalopathy. Given the geographic predominance of Lyme disease in the Northeast and Midwest of the USA, no major studies have been conducted regarding Southern states. Between 2005 and 2014, the Center for Disease Control has reported 582 confirmed cases of Lyme disease in Texas. Because of the potential for increased incidence and prevalence in Texas, it has become essential for research and clinical efforts to be diverted to the region. The Texas A&M College of Veterinary Medicine and Biomedical Sciences Lyme Lab has been investigating the ecology of Lyme disease in Texas and developing a pan-specific serological test for Lyme diagnosis. This report aimed to exposure materials and raise awareness of Lyme disease to healthcare providers.

  15. Ophthalmic manifestations of atypical IgD multiple myeloma.

    PubMed

    Edmunds, Matthew R; Cikatricis, Peter; Mukherji, Subhanjan; Bowyer, Jeremy D

    2012-07-19

    A previously healthy 32-year-old Caucasian female presented with sudden-onset horizontal diplopia following a paroxysm of coughing. She had recently sustained a pubic ramus fracture during an innocuous fall and had also noted a firm lump developing at the right side of her forehead. On examination, she had a right fronto-temporal mass. Visual acuities were 6/6 bilaterally. There was reduced abduction of the right eye, bilateral white, granular corneal opacities and evidence of bilateral optic disc swelling. Haematological investigations revealed normocytic anaemia, hypercalcaemia and raised erythrocyte sedimentation rate (ESR). CT showed lytic foci throughout the skull, ribs, scapulae, spine, pelvis and upper femora. Serum protein electrophoresis revealed immunoglobulin D (IgD)-kappa paraproteinaemia; urine electrophoresis showed free light chain kappa and bone marrow biopsy demonstrated 87% plasma cells. A diagnosis of IgD multiple myeloma was made, with subsequent chemotherapeutic treatment and eventual autologous stem cell transplant resulting in resolution of neuro-ophthalmic manifestations and prolonged disease remission.

  16. Serotype-specific differences in clinical manifestations of dengue.

    PubMed

    Balmaseda, Angel; Hammond, Samantha N; Pérez, Leonel; Tellez, Yolanda; Saborío, Saira Indira; Mercado, Juan Carlos; Cuadra, Ricardo; Rocha, Julio; Pérez, Maria Angeles; Silva, Sheyla; Rocha, Crisanta; Harris, Eva

    2006-03-01

    Dengue, the most prevalent arthropod-borne viral disease of humans, is caused by four serotypes of dengue virus (DENV 1-4). Although all four DENV serotypes cause a range of illness, defining precisely which clinical characteristics are associated with the distinct serotypes has been elusive. A cross-sectional study was conducted on 984 and 313 hospitalized children with confirmed DENV infections during two time periods, respectively, in the same hospitals in Nicaragua: a 3-year period (1999-2001) when DENV-2 accounted for 96% of the viruses identified, and the 2003 dengue season when DENV-1 predominated (87% of identified serotypes). When the two periods were compared, more shock (OR 1.91, 95% CI 1.35-2.71) and internal hemorrhage (OR 2.05, CI 1.16-3.78) were observed in the period when DENV-2 predominated, whereas increased vascular permeability was associated to a greater degree with the DENV-1 period (OR 2.36, CI 1.80-3.09). Compared with the DENV-2 period, the DENV-1 season was associated with more hospitalized primary dengue cases (OR 3.86, CI 2.72-5.48) and more primary DENV infections with severe manifestations (OR 2.93, CI 2.00-4.28). These findings provide new data to characterize the pathogenic potential of distinct DENV serotypes in human populations.

  17. Epidemiology and Clinical Manifestations of Enteroaggregative Escherichia coli

    PubMed Central

    Hebbelstrup Jensen, Betina; Olsen, Katharina E. P.; Struve, Carsten; Petersen, Andreas Munk

    2014-01-01

    SUMMARY Enteroaggregative Escherichia coli (EAEC) represents a heterogeneous group of E. coli strains. The pathogenicity and clinical relevance of these bacteria are still controversial. In this review, we describe the clinical significance of EAEC regarding patterns of infection in humans, transmission, reservoirs, and symptoms. Manifestations associated with EAEC infection include watery diarrhea, mucoid diarrhea, low-grade fever, nausea, tenesmus, and borborygmi. In early studies, EAEC was considered to be an opportunistic pathogen associated with diarrhea in HIV patients and in malnourished children in developing countries. In recent studies, associations with traveler's diarrhea, the occurrence of diarrhea cases in industrialized countries, and outbreaks of diarrhea in Europe and Asia have been reported. In the spring of 2011, a large outbreak of hemolytic-uremic syndrome (HUS) and hemorrhagic colitis occurred in Germany due to an EAEC O104:H4 strain, causing 54 deaths and 855 cases of HUS. This strain produces the potent Shiga toxin along with the aggregative fimbriae. An outbreak of urinary tract infection associated with EAEC in Copenhagen, Denmark, occurred in 1991; this involved extensive production of biofilm, an important characteristic of the pathogenicity of EAEC. However, the heterogeneity of EAEC continues to complicate diagnostics and also our understanding of pathogenicity. PMID:24982324

  18. Superwarfarin intoxication: hematuria is a major clinical manifestation.

    PubMed

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

    2009-09-01

    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.

  19. Oral Manifestations of Human Immunodeficiency Virus-Infected Patients

    PubMed Central

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

    2015-01-01

    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 χ2 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. PMID:25745611

  20. Trypanosoma cruzi infection: a review with emphasis on cutaneous manifestations

    PubMed Central

    Hemmige, Vagish; Tanowitz, Herbert; Sethi, Aisha

    2013-01-01

    Chagas disease, an infection caused by the protozoan Trypanosoma cruzi and transmitted by the Reduuvid insect vector, remains a major cause of morbidity in Central and South America over a century after its discovery in 1909. Though major advances in preventing the spread of this disease have been made in recent decades, millions of individuals remain chronically infected due to prior exposure to T. cruzi and are at risk for future complications from the disease. Dermatologic manifestations of acute infection may include localized swelling at the site of inoculation (chagoma), conjunctivitis (Romaña’s sign), and a generalized morbilliform eruption (schizotrypanides). Reactivation of quiescent infection in immunocompromised hosts due to the acquired immunodeficiency syndrome or organ transplantation can present with fever and skin lesions including panniculitis. The wide-spread emigration of chronic carriers of T. cruzi to North America, Europe, and Australia makes it imperative that dermatologists worldwide be familiar with this entity to ensure proper diagnosis and treatment. PMID:22515575