Multimodality Cardiac Imaging in a Patient with Kawasaki Disease and Giant Aneurysms

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Ranjini Srinivasan

2016-01-01

Full Text Available Kawasaki disease is a well-known cause of acquired cardiac disease in the pediatric and adult population, most prevalent in Japan but also seen commonly in the United States. In the era of intravenous immunoglobulin (IVIG treatment, the morbidity associated with this disease has decreased, but it remains a serious illness. Here we present the case of an adolescent, initially diagnosed with Kawasaki disease as an infant, that progressed to giant aneurysm formation and calcification of the coronary arteries. We review his case and the literature, focusing on the integral role of multimodality imaging in managing Kawasaki disease.

Kawasaki disease: review of 21 cases

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Cem Arat

2016-03-01

Conclusion: Kawasaki disease has difficulty in diagnosis because of having broad spectrum of presenting symptoms. Early diagnosis and treatment is very important in preventing coronary artery abnormalities. [Cukurova Med J 2016; 41(1.000: 97-104

Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

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Lin Ming-Chih

2012-01-01

Full Text Available Abstract Introduction Kawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited. Case presentation We report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease. Conclusions In this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries.

Doença de Kawasaki: experiência clínica em hospital universitário Kawasaki disease: clinical experience in a university hospital

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Angela Esposito Ferronato

2010-06-01

Full Text Available OBJETIVO: A doença de Kawasaki é uma vasculite sistêmica aguda de etiologia desconhecida. Seu diagnóstico baseia-se em critérios clínicos. O objetivo deste estudo foi descrever os casos de pacientes com doença de Kawasaki internados no Hospital Universitário da Universidade de São Paulo entre janeiro/2000 e junho/2008. MÉTODOS: Dentre todos os pacientes internados na Enfermaria de Pediatria no período acima, foram selecionados aqueles cujo CID de alta foi doença de Kawasaki. Realizou-se estudo descritivo por meio da análise dos prontuários dessas crianças. RESULTADOS: Foram encontrados 18 casos. A média de internações foi de 2,1 casos/ano. A idade variou de três meses a nove anos. A proporção meninos:meninas foi 1:1,25. Receberam outros diagnósticos prévios 17 pacientes, sendo escarlatina em 2/3 dos casos. O tempo de febre antes do diagnóstico variou de cinco a 11 dias. Nove crianças apresentaram quatro sinais sugestivos de doença de Kawasaki; oito apresentaram cinco sinais e uma apresentou dois sinais, o que foi considerado doença de Kawasaki incompleta. Receberam gamaglobulina 15 crianças (entre o sexto e o décimo dias de evolução e 11 (73% ficaram afebris após infusão da medicação. Os demais tiveram febre até 24 horas após a administração. Todos os pacientes realizaram ecocardiograma e três apresentaram aneurisma leve da coronária. CONCLUSÕES: A doença de Kawasaki é habitualmente confundida com outras doenças, o que causa retardo no tratamento e aumento no risco de complicações cardíacas.OBJECTIVE: Kawasaki disease is an acute systemic vasculitis of unknown etiology. Its diagnosis is based on clinical criteria. This study aimed to describe Kawasaki disease cases treated at the University Hospital of Universidade de São Paulo, from January/2000 to June/2008. METHODS: Among all patients admitted to the pediatric ward during this period, patients whose discharge ICD was Kawasaki disease were

Enfermedad de Kawasaki

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Carlos Coronel Carvajal

2003-09-01

Full Text Available Se realizó una revisión actualizada de la enfermedad de Kawasaki, con el objetivo de familiarizar a pediatras y médicos de la familia con los elementos clínicos de la enfermedad, para que de esta manera pueda ser diagnosticada y manejada oportunamente. La enfermedad de Kawasaki fue descrita por Tomisaki Kawasaki en 1967, y se manifiesta como un síndrome febril en la niñez temprana. La enfermedad se diagnostica con mayor frecuencia en los menores de 5 años, a través de una serie de criterios clínicos establecidos. La forma típica de la enfermedad tiene 3 fases de duración variable. La afectación cardíaca es la manifestación más importante de la enfermedad, del 10 al 40 % de los niños muestran signos de vasculitis coronaria, manifestada por aneurismas de las arterias coronarias.An updated review of Kawasaki disease was carried out to make pediatricians and family physicians familiar with the clinical elements of the disease so that it can be properly diagnosed and managed. Kawasaki disease was described by Tomisaki Kawasaki in 1967 and manifests as a febrile syndrome at early childhood. The disease is very often diagnosed in under five years-old children on the basis of a series of set clinical criteria. The typical form of the disease had 3 phases of variable duration. The cardiac effect is the most important manifestation of the disease since 10 to 40 % of children show signs of coronary vasculitis in the form of coronary arterial aneurysms.

Use of Corticosteroid in Children with Unresponsiveness to Intravenous Immunoglobulin in Kawasaki Disease

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Abdolkarim Hamedi

2017-08-01

Full Text Available Background Kawasaki Disease (KD is a vasculitis with multi-organ involvementof unknown etiology; it is the most common cause of pediatric-heart diseases in developed countries. Treatment with Intravenous Immunoglobulin (IVIG prevents coronary artery lesions; although there are some IVIG-resistant cases, combination therapy with corticosteroids and IVIG is one of the recommendations for treatment of these cases. The aim of this study was to compare these three options for treatment of Kawasaki Disease and to evaluate their ability to deal with coronary artery complication of Kawasaki Disease. Materials and Methods A prospective cross- sectional study of hospitalized cases of Kawasaki Disease, conducted in pediatric department of Imam Reza hospital, Mashhad-Iran, during 2013 to 2015 (18 months. Based on demographic and clinical data of these patients, children with high risk of unresponsiveness to IVIG therapy (based on Harada score, were determined and treated with IVIG and corticosteroids- combination initially. Follow-up patients for heart complications were 6 weeks. Results Twenty five patients (89.2% out of total 28 hospitalized patients in this period of time who fulfilled diagnostic criteria were considered as complete Kawasaki Disease. Coronary Artery Lesions (CALs were shown in 4 patients during the follow-up period, with high risk in patients with incomplete presentation (33.3% versus 12%, P

Natriuretic Peptides in Kawasaki Disease: the Myocardial Perspective

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Nagib Dahdah

2013-01-01

Full Text Available Making a diagnosis of Kawasaki disease with certainty may be challenging, especially since the recognition of cases with incomplete diagnostic criteria and its consequences. In order to build the diagnostic case in daily practice, clinicians rely on clinical criteria established over four decades ago, aided by non specific laboratory tests, and above all inspired by experience. We have recently studied the diagnostic value of N-terminal pro B-type natriuretic peptide to improve the diagnostic certainty of cases with complete or incomplete clinical criteria. Our working hypothesis was based on the fact that myocarditis is present in nearly all Kawasaki disease patients supported by histology data. In this paper, we review these facts and the myocardial perspective from the diagnostic and the mechanistic standpoints.

Unexpected myocardial uptake on bone scintigraphy in an infant with Kawasaki disease

International Nuclear Information System (INIS)

Macdonald, W.B.G.; Troedson, R.G.

2003-01-01

Full text: A two-month-old female infant was admitted to hospital because of irritability and poor feeding over the preceding two weeks. There was no history of fever but serum inflammatory markers were elevated and a throat swab yielded a pure growth of Strep, pyogenes. There was no response to antibiotics. The nursing staff noted the infant disliked handling and skeletal pathology was suspected. A bone scan using Tc-99m hydroxymethylene diphosphonate (HDP) showed myocardial activity, with no evidence of abnormal skeletal activity. Subsequent echocardiography showed coronary artery ectasia, typical of Kawasaki disease, with papillary muscle dysfunction, indicating likely myocarditis. A diagnosis of myocarditis secondary to Kawasaki disease was made and the patient promptly improved following intravenous immunoglobulin therapy. Cardiac manifestations of Kawasaki disease included coronary artery aneurysms, myocardial infarction, regional perfusion abnormalities and myocarditis. Myocardial uptake of phosphate tracers is well known following myocardial infarction but there was no wall motion disturbance or ECG abnormality to suggest infarction in this patient and she was felt to have myocarditis. Myocardial uptake of phosphate tracers has not previously been reported in Kawasaki disease. Copyright (2003) The Australian and New Zealand Society of Nuclear Medicine Inc

Genome-wide association study identifies FCGR2A as a susceptibility locus for Kawasaki disease

NARCIS (Netherlands)

Khor, Chiea Chuen; Davila, Sonia; Breunis, Willemijn B.; Lee, Yi-Ching; Shimizu, Chisato; Wright, Victoria J.; Yeung, Rae S. M.; Tan, Dennis E. K.; Sim, Kar Seng; Wang, Jie Jin; Wong, Tien Yin; Pang, Junxiong; Mitchell, Paul; Cimaz, Rolando; Dahdah, Nagib; Cheung, Yiu-Fai; Huang, Guo-Ying; Yang, Wanling; Park, In-Sook; Lee, Jong-Keuk; Wu, Jer-Yuarn; Levin, Michael; Burns, Jane C.; Burgner, David; Kuijpers, Taco W.; Hibberd, Martin L.; Lau, Yu-Lung; Zhang, Jing; Ma, Xiao-Jing; Liu, Fang; Wu, Lin; Yoo, Jeong-Jin; Hong, Soo-Jong; Kim, Kwi-Joo; Kim, Jae-Jung; Park, Young-Mi; Mi Hong, Young; Sohn, Sejung; Young Jang, Gi; Ha, Kee-Soo; Nam, Hyo-Kyoung; Byeon, Jung-Hye; Weon Yun, Sin; Ki Han, Myung; Lee, Kyung-Yil; Hwang, Ja-Young; Kuipers, Irene M.; Ottenkamp, Jaap J.; Biezeveld, Maarten; Tacke, Carline

2011-01-01

Kawasaki disease is a systemic vasculitis of unknown etiology, with clinical observations suggesting a substantial genetic contribution to disease susceptibility. We conducted a genome-wide association study and replication analysis in 2,173 individuals with Kawasaki disease and 9,383 controls from

Incidence, epidemiology and clinical features of Kawasaki disease in Catalonia, Spain.

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Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estíbaliz; Calzada-Hernandez, Joan

2016-01-01

To assess the incidence, epidemiology and clinical features of Kawasaki disease (KD) in Catalonia (northeast region of Spain). This was an observational population-based study including all Paediatric Units in Catalonia, under both public and private management. Retrospective data retrieval was performed for 10 years (2004-2013). A 12-month (March 2013 to March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. Data from 399 patients over the 10-year study period was analysed, revealing that 233 (58.4%) had complete KD, 159 (39.8) incomplete KD and 7 (1.7%) were considered atypical KD. Mean annual incidence was 3.5/105 children 10(th) day of illness, ages 8 yo and the presence of sterile piuria, aseptic meningitis, abdominal pain and uveitis at diagnosis were found to have higher risk of coronary aneurisms (CAA) (pIncidence, clinical features and treatment plans in our cohort are similar to those described in other European studies.

Vaccines and Kawasaki disease.

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Esposito, Susanna; Bianchini, Sonia; Dellepiane, Rosa Maria; Principi, Nicola

2016-01-01

The distinctive immune system characteristics of children with Kawasaki disease (KD) could suggest that they respond in a particular way to all antigenic stimulations, including those due to vaccines. Moreover, treatment of KD is mainly based on immunomodulatory therapy. These factors suggest that vaccines and KD may interact in several ways. These interactions could be of clinical relevance because KD is a disease of younger children who receive most of the vaccines recommended for infectious disease prevention. This paper shows that available evidence does not support an association between KD development and vaccine administration. Moreover, it highlights that administration of routine vaccines is mandatory even in children with KD and all efforts must be made to ensure the highest degree of protection against vaccine-preventable diseases for these patients. However, studies are needed to clarify currently unsolved issues, especially issues related to immunologic interference induced by intravenous immunoglobulin and biological drugs.

Incomplete Kawasaki disease with recurrent skin peeling: a case report with the review of literature.

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Parmar R

2003-01-01

Full Text Available Kawasaki disease (KD is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD.

Coronary artery calcification in Kawasaki disease

International Nuclear Information System (INIS)

Ino, T.; Shimazaki, S.; Akimoto, K.; Park, I.; Nishimoto, K.; Yabuta, K.; Tanaka, A.

1990-01-01

To evaluate the angiographic features of coronary lesions in Kawasaki disease with coronary artery calcification, cinefluoroscopy and cineangiography were retrospectively reviewed in 116 patients who had undergone coronary angiography between 1982 and 1989. Angiographic abnormalities of coronary arteries were demonstrated in 55 of 116 patients. In 5 (9.1%) of the 55 patients, 9 with calcification were identified by cinefluoroscopy and chest X-ray. Eight of the 9 calcified lesions showed a circular or ring-shape configuration. Coronary angiography revealed a total occlusion of the right coronary artery with collateral circulation from the distal left coronary artery in 2 patients and a severe stenosis of the right coronary artery in 2 patients, in whom anticoagulant therapy had not been continued during the follow-up periods. The remaining patient in whom anticoagulant therapy had been continued had bilateral aneurysms but no significant stenosis. These results indicate that a ring-shape calcification on chest X-ray in 2 patients with a history of Kawasaki disease may suggest an involvement by coronary artery stenosis even when anticoagulant drugs had been given. Therefore, coronary angiography should be performed to evaluate the stenotic lesions if this type of calcification is found by routine radiographic examination. (orig.)

CLINICAL MANIFESTATION, DIAGNOSTICS AND TREATMENT OF KAWASAKI DISEASE: KNOWN DATA AND UNSOLVED QUESTIONS

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G. А. Lyskina

2013-01-01

Full Text Available The lecture deals with the most common systemic vasculitis in pediatric practice — Kawasaki disease. This disorder is associated with risk of myocardial infarction and sudden cardiac death in children and young adults and at present is considered to be the main cause of the acquired heard diseases in children. The authors give historical aspects and modern opinions on etiology, pathogenesis, clinical manifestation, diagnostics and treatment of Kawasaki disease. The data were summarized from Russian and foreign literature as well as from the own authors’ experience.

Kawasaki disease in Ghana: Case reports from Korle Bu Teaching ...

African Journals Online (AJOL)

Kawasaki disease, an acute febrile vasculitis, predominantly affects children under the age of 5 years and is thought to be a rare disease in the developing world. . It has previously never been reported in Ghana. We report 3 cases from February, 2007 to February, 2008. This potentially serious disease has no definitive ...

Atypical desquamation in a 2.5-year-old boy with Kawasaki disease: A case report.

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Adib, Ali; Fazel, Ali; Nabavizadeh, Seyed Hesamedin; Alyasin, Sohaila; Kashef, Sara

2017-02-01

Kawasaki disease (KD) is a vasculitis that mostly affects children under 5 years of age. This article presents a 2.5-year-old boy who presented with 6 days of fever, generalized maculopapular rash, bilateral non-exudative conjunctivitis, cracked lips, right cervical lymphadenopathy, erythematous extremities, and perianal desquamation. Laboratory studies showed leukocytosis and sterile pyuria. Because diagnosis of KD was proved, oral acetylsalicylic acid with the anti-inflammatory dose and intravenous immunoglobulin were started for him. On the seventh day of admission time, he developed desquamation and erythema on the site of his right cervical lymphadenopathy as well as periungual scaling. About three weeks after starting the treatment, scaling of the cervical lymphadenopathy and periungual area stopped. Echocardiography was performed for him three times: at the time of diagnosis, four weeks, and 6 months later and revealed normal coronary arteries. We report this sign, desquamation on the site of cervical lymphadenopathy, as a new finding.

Kawasaki syndrome: a case report.

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Blakeley, S L; Cohen, P R

1993-08-01

A four-year-old black boy with Kawasaki syndrome is reported. The child was treated with intravenous gamma globulin and aspirin. He had no disease-associated adverse sequelae. The clinical findings, diagnostic criteria, and treatment of Kawasaki syndrome are reviewed.

Enfermedad de Kawasaki en un infante Kawasaki disease in a child

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José Luis Lobaina Lafita

2012-11-01

Full Text Available Se presenta el caso clínico de un infante de 4 años de edad, con antecedente de amigdalitis a repetición, ingresado en el Hospital Infantil Norte Docente "Dr. Juan de la Cruz Martínez Maceira" de Santiago de Cuba por presentar, desde hacía 9 días, fiebre de 38 y 39 °C, odinofagia, aumento de volumen del cuello y erupción cutánea generalizada. Teniendo en cuenta el examen físico y los resultados de los estudios complementarios efectuados se diagnosticó la enfermedad de Kawasaki, por lo cual se indicó tratamiento con Intacglobin®, Aspirina® y vitaminoterapia. El paciente evolucionó favorablemente y egresó de la institución hospitalaria sin complicaciones.The case of a 4 year-old child with history of recurrent tonsillitis, who was admitted to "Dr. Juan de la Cruz Martínez Maceira" Northern Teaching Children Hospital of Santiago de Cuba is reported for presenting with temperature of 38 and 39°C, odynophagia, enlargement of the neck and generalized rash in the past nine days. Based on physical examination and the results of complementary tests the Kawasaki disease was diagnosed, for which a treatment with Intacglobin®, Aspirin®, and vitamin therapy was indicated. The patient made good progress and was discharged from the hospital without complications.

Acute kidney injury and cholestasis associated with Kawasaki disease in a 9-year-old: Case report.

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Martínez Vázquez, José Allan; Sánchez García, Carlos; Rodríguez Muñoz, Lorena; Martínez Ramírez, Rogelio Osvaldo

2017-12-15

Kawasaki disease (KD) is a systemic vasculitis frequent in children younger than 5 years of age. It involves coronary arteries and other medium-sized vessels. There also exists evidence of inflammatory and proliferative changes affecting the biliary tract and lymphocyte infiltration of the renal interstitial. We describe the case of a 9-year-old girl who developed high-grade fever, bilateral non-purulent conjunctivitis, «strawberry» tongue, desquamation of the fingers and toes, cholestatic syndrome, edema and elevated serum creatinine. KD is a diagnostic challenge for the pediatrician. In every patient with high-grade fever, cholestasis and acute kidney injury, KD should be included in the differential diagnosis, even though more research is necessary to evaluate this atypical association. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Facial nerve palsy in a thirteen-year-old male youth with Kawasaki disease

NARCIS (Netherlands)

Biezeveld, Maarten H.; Voorbrood, Bas S.; Clur, Sally-Ann B.; Kuijpers, Taco W.

2002-01-01

A 13-year-old male youth was hospitalized with Kawasaki disease. In the course of the disease he developed a facial nerve palsy and an aneurysm of the right coronary artery. After treatment with immunoglobulins both complications disappeared within 10 days and 1 month, respectively

Kawasaki disease in Sicily: clinical description and markers of disease severity

OpenAIRE

Maggio, M.; Corsello, G.; Prinzi, E.; Cimaz, R.

2016-01-01

Background Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. Methods We studied the clinical data ...

Biphasic thallium 201 SPECT-imaging for the noninvasive diagnosis of myocardial perfusion abnormalities in a child with Kawasaki disease--a case report

International Nuclear Information System (INIS)

Hausdorf, G.; Nienaber, C.A.; Spielman, R.P.

1988-01-01

The mucocutaneous lymph node syndrome (Kawasaki disease) is of increasing importance for the pediatric cardiologist, for coronary aneurysms with the potential of thrombosis and subsequent stenosis can develop in the course of the disease. The authors report a 2 1/2-year-old female child in whom, fourteen months after the acute phase of Kawasaki disease, myocardial infarction occurred. Biphasic thallium 201 SPECT-imaging using dipyridamole depicted anterior wall ischemia and inferolateral infarction. This case demonstrates that noninvasive vasodilation-redistribution thallium 201 SPECT-imaging has the potential to predict reversible myocardial perfusion defects and myocardial necrosis, even in small infants with Kawasaki disease

Pulse methylprednisolone therapy for impending cardiac tamponade in immunoglobulin-resistant Kawasaki disease

NARCIS (Netherlands)

Dahlem, P. G.; von Rosenstiel, I. A.; Lam, J.; Kuijpers, T. W.

1999-01-01

We describe a boy with Kawasaki disease (KD) whose clinical course was marked by a rapid improvement upon treatment with intravenous immunoglobulin (IVIG) and oral aspirin, which - within 14 days - was followed by the development of a large pericardial effusion with symptoms of impending cardiac

Dipyridamole-thallium-201 tomography documenting improved myocardial perfusion with therapy in Kawasaki disease

International Nuclear Information System (INIS)

Nienaber, C.A.; Spielmann, R.P.; Hausdorf, G.

1988-01-01

Thallium-201 tomographic perfusion studies after pharmacologic vasodilation were performed in seven children (aged 2 years 8 months to 8 years 7 months), 3 to 20 months after the acute stage of the disease. In all patients coronary aneurysms were seen on cross-sectional echocardiograms. The scintigrams of six children showed no significant regional reduction of myocardial thallium-201 uptake. These children had remained asymptomatic in the follow-up period after the acute inflammatory stage of Kawasaki disease. Persistent and transient thallium defects were present in one child with acute posterolateral myocardial infarction; obstruction of two coronary vessels supplying the defect zones was confirmed by contrast angiography. After 8 months of treatment a follow-up nuclear scan showed marked reduction in the size of the defect and almost complete abolishment of the ischemic reaction. Thus tomographic thallium-201 perfusion scintigraphy in conjunction with vasodilation stress is useful to assess myocardial perfusion in children with Kawasaki disease and demonstrates marked improvement in regional perfusion after adequate medical therapy

Myocardial performance and perfusion during exercise in patients with coronary artery disease caused by Kawasaki disease

International Nuclear Information System (INIS)

Paridon, S.M.; Ross, R.D.; Kuhns, L.R.; Pinsky, W.W.

1990-01-01

For a study of the natural history of coronary artery lesions after Kawasaki disease and their effect on myocardial blood flow reserve with exercise, five such patients underwent exercise testing on a bicycle. Oxygen consumption, carbon dioxide production, minute ventilation, and electrocardiograms were monitored continuously. Thallium-201 scintigraphy was performed for all patients. One patient stopped exercise before exhaustion of cardiovascular reserve but had no evidence of myocardial perfusion abnormalities. Four patients terminated exercise because of exhaustion of cardiovascular reserve; one had normal cardiovascular reserve and thallium scintiscans, but the remaining patients had diminished cardiovascular reserve. Thallium scintigrams showed myocardial ischemia in two and infarction in one. No patient had exercise-induced electrocardiographic changes. These results indicate that patients with residual coronary artery lesions after Kawasaki disease frequently have reduced cardiovascular reserve during exercise. The addition of thallium scintigraphy and metabolic measurements to exercise testing improved the detection of exercise-induced abnormalities of myocardial perfusion

Heart and coronary artery damage related to Kawasaki syndrome

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A. A. Leontyeva

2014-01-01

Full Text Available Kawasaki syndrome is an acute systemic vasculitis of unknown etiology, which mainly affects children within the first 5 years of fife. At the present time, Kawasaki syndrome is recognized to be a leading cause of acquired organic heart diseases in children, which may result in coronary heart disease, myocardial infarction, and sudden death in children and young people. Most complications are associated with the cardiovascular system, with coronary artery changes in particular. Transthoracic echocardiography, which, besides coronary artery assessment, makes it possible to evaluate right and left ventricular systolic and diastolic functions and to study the cardiac valves and changes in pericardial effusion, is a major technique in Kawasaki syndrome. The paper outlines an update on the epidemiology, etiology, pathogenetic mechanisms, and pathomorphology of Kawasaki syndrome and considers possible coronary and noncoronary changes, outcomes, and clinical manifestations.

Kawasaki disease: An unusual presentation in a 14-year old boy in ...

African Journals Online (AJOL)

Kawasaki disease (KD) is an acute systemic vasculitis that mostly affects children less than 5years. Occasionally, it may presents with renal involvement of varying severity. In Nigeria and most of Africa, only a few cases of KD have been reported and these were among children within the typical age group. We report an ...

Procalcitonin Levels in Patients with Complete and Incomplete Kawasaki Disease

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Hwa Jin Cho

2013-01-01

Full Text Available Incomplete Kawasaki disease (iKD is considered to be a less complete form of Kawasaki disease (cKD, and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group. Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study. We obtained four measurements of serum procalcitonin levels and those of other inflammatory markers from each patient. Samples were taken for analysis on the day of diagnosis (thus before treatment commenced; D0 and 2 (D2, 14 (D14, and 56 days (D56 after intravenous immunoglobulin infusion. We obtained control group data at D0. The mean D0 serum procalcitonin levels of cKD patients (0.71±1.36 ng/mL and controls (0.67±1.06 ng/mL were significantly higher than those of iKD patients (0.26±0.26 ng/mL (P=0.014 and P=0.041, resp.. No significant difference in mean procalcitonin level was evident among groups at any subsequent time. In conclusion, the serum procalcitonin level of patients with acute-stage cKD was significantly higher than that of iKD patients.

Kawasaki disease in Sicily: clinical description and markers of disease severity.

Science.gov (United States)

Maggio, Maria Cristina; Corsello, Giovanni; Prinzi, Eugenia; Cimaz, Rolando

2016-11-02

Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness. Forty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 ± 2 days after the fever started. Defervescence occurred 39 ± 26 hours after the first IVIG infusion. Fifty-six patients (80 %) received 1 dose of IVIG (responders); 14 patients (20 %) had a resistant KD, with persistent fever after the first IVIG dose (non responders). Ten (14 %) non responders responded to the second dose, 4 (5 %) responded to three doses; one needed treatment with high doses of steroids and Infliximab. Cardiac involvement was documented in twenty-two cases (eighteen with transient dilatation/ectasia, fifteen with aneurysms). Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven. Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses. Non responders

A positron emission tomography study of cardiac sequelae in children with Kawasaki disease, 1

International Nuclear Information System (INIS)

Ohmochi, Yutaka

1994-01-01

This study quantitatively measured regional myocardial blood flow (MBF) and perfusable tissue fraction (pTF) in 25 children (mean age: 17.2±2.7) with Kawasaki disease using positron emission tomography and H 2 15 O. Patients were divided into three groups based on coronary angiographic findings. Group 1 consisted of 11 patients with normal coronary angiograms; Group 2, 7 patients with stenotic coronary lesions. There were no significant differences in MBF and pTF among 5 divided regions on the left ventricular wall. Average MBF at rest in Group 1 was 0.91±0.19 ml/min/g. There was a poor correlation between MBF estimated positron emission tomography and patient's age in Group 1. (r=-0.374, Y=-0.0234X + 1.254: p 2 15 O, to determine the functional capacity of coronary artery lesions and myocardial damage in children with Kawasaki disease. (author)

Computerized tomography in atypical Pott's disease

International Nuclear Information System (INIS)

Cabrera, M.N.B.; Wang, E.H.M.

1993-01-01

Classical Pott's disease is described as a two-vertebrae disease with the destruction of the intervening invertebral disc. Computerized tomography has been used in the differential diagnosis of spine infections and neoplasms. We reviewed CT scans of patients seen at the Philippine General Hospital over a two-year period with atypical presentations of atypical tuberculous spondylitis. We used the computerized tomography findings described as characteristic of classical Pott's disease as criteria in evaluating the CT scans of patients diagnosed to have Atypical Pott's Disease. Although the number of patients prevented sensitivity and specificity studies to be done, our results strongly suggest that the same CT criteria used to diagnose Classical Pott's Disease may also be used to diagnose Pott's disease in its atypical form. (Author.). 13 refs

Atypical disease phenotypes in pediatric ulcerative colitis

DEFF Research Database (Denmark)

Levine, Arie; de Bie, Charlotte I; Turner, Dan

2013-01-01

Definitive diagnosis of pediatric ulcerative colitis (UC) may be particularly challenging since isolated colitis with overlapping features is common in pediatric Crohn's disease (CD), while atypical phenotypes of UC are not uncommon. The Paris classification allows more accurate phenotyping...... of atypical inflammatory bowel disease (IBD) patients. Our aim was to identify the prevalence of atypical disease patterns in new-onset pediatric UC using the Paris classification....

Quality of Life and Behavioral Functioning in Dutch Children with a History of Kawasaki Disease

NARCIS (Netherlands)

Tacke, Carline E.; Haverman, Lotte; Berk, Birgit M.; van Rossum, Marion A.; Kuipers, Irene M.; Grootenhuis, Martha A.; Kuijpers, Taco W.

2012-01-01

Objective The authors evaluated health-related quality of life (HRQOL) and behavioral functioning in patients with a history of Kawasaki disease (KD). Study design A cross-sectional study was conducted at a tertiary referral center for KD follow-up in 280 patients (mean age 8.6 years, 60.0% male).

Japanese scoring systems to predict resistance to intravenous immunoglobulin in Kawasaki disease were unreliable for Caucasian Israeli children.

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Arane, Karen; Mendelsohn, Kerry; Mimouni, Michael; Mimouni, Francis; Koren, Yael; Simon, Dafna Brik; Bahat, Hilla; Helou, Mona Hanna; Mendelson, Amir; Hezkelo, Nofar; Glatstein, Miguel; Berkun, Yackov; Eisenstein, Eli; Aviel, Yonatan Butbul; Brik, Riva; Hashkes, Philip J; Uziel, Yosef; Harel, Liora; Amarilyo, Gil

2018-05-24

This study assessed the validity of using established Japanese risk scoring methods to predict intravenous immunoglobulin (IVIG) resistance to Kawasaki disease in Israeli children. We reviewed the medical records of 282 patients (70% male) with Kawasaki disease from six Israeli medical centres between 2004-2013. Their mean age was 2.5 years. The risk scores were calculated using the Kobayashi, Sano and Egami scoring methods and analysed to determine if a higher risk score predicted IVIG resistance in this population. Factors that predicted a lack of response to the initial IVIG dose were identified. We found that 18% did not respond to the first IVIG dose. The three scoring methods were unable to reliably predict IVIG resistance, with sensitivities of 23-32% and specificities of 67-87%. Calculating a predictive score that was specific for this population was also unsuccessful. The factors that predicted a lacked of response to the first IVIG dose included low albumin, elevated total bilirubin and ethnicity. The established risk scoring methods created for Japanese populations with Kawasaki disease were not suitable for predicting IVIG resistance in Caucasian Israeli children and we were unable to create a specific scoring method that was able to do this. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Crystalline-Like Keratopathy after Intravenous Immunoglobulin Therapy with Incomplete Kawasaki Disease: Case Report and Literature Review

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Elif Erdem

2013-01-01

Full Text Available A 7-year-old girl had presented with high body temperature and joint pain which continued for 3 days. Because of the prolonged history of unexplained fever, rash, bilateral nonpurulent conjunctival injection, oropharyngeal erythema, strawberry tongue, and extreme of age, incomplete Kawasaki disease was considered and started on an intravenous immunoglobulin infusion. Six days after this treatment, patient was referred to eye clinic with decreased vision and photophobia. Visual acuity was reduced to 20/40 in both eyes. Slit-lamp examination revealed bilateral diffuse corneal punctate epitheliopathy and anterior stromal haze. Corneal epitheliopathy seemed like crystal deposits. One day after presentation, mild anterior uveitis was added to clinical picture. All ocular findings disappeared in one week with topical steroid and unpreserved artificial tear drops. We present a case who was diagnosed as incomplete Kawasaki disease along with bilateral diffuse crystalline-like keratopathy. We supposed that unusual ocular presentation may be associated with intravenous immunoglobulin treatment.

Reduced myocardial perfusion reserve in myocardium having coronary artery aneurysm of Kawasaki disease

International Nuclear Information System (INIS)

Yoon, S. N.; Lee, D. S.; Choi, J. Y.; Kil, H. R.; Jeong, Z. K.; Lee, M. C.; Ko, C. S.

1997-01-01

Kawasaki disease is a systemic vasculitis involving the coronary arteries at early childhood and cause coronary artery aneurysms and thrombotic occlusions. These coronary artery aneurysms were usually transformed later into stenotic or obstructive lesions, however, the majority of these aneurysms, even the giant ones, are known to be associated with normal epicardial coronary flow. Flow reserve is difficult to assess in aneurysmal arteries with echo or angiography. We performed this study to question if there are abnormalities in flow reserve in myocardial tissue with normal epicardial arterial flow on angiography in patients with Kawasaki disease, dipyridamole stress and rest Tc-99m-sestamibi SPECT were performed in 37 patients (28 boys, 9 girls, mean age 6.6 years). We compared SPECT findings with coronary angiography (CAG) findings in 21 patients who did both studies after finding abnormality on echocardiaography. On CAG, aneurysms were found in 26 arteries of 16 patients, i.e., 10 left main arteries, 6 left anterior descending arteries (LAD), 2 left circumflex arteries (LCX), and 8 right coronary arteries (RCA). Localized and segmental stenotic lesions were found in 11 arteries in 9 patients (LAD: 4, LCX: 1, RCA: 6). Eight of the 10 patients with aneurysms had no obvious stenosis. On stress-rest SPECT, 16(43%) out of 37 patients showed normal perfusion and the other 21(57%) showed reversible or persistent decrease. Among 11 stenotic artery territories, 3(27%) showed persistent and/or reversible perfusion defects. The other 8 were normal. Among 26 aneurysmal artery territories, 12 artery territories showe perfusion decrease. Three of the 5 patients with normal CAG showed persistent and/or transient perfusion defects. Among 14 artery territories with perfusion decrease in the 16 patients, 3(21%) could be localized to vascular territory having stenosis of supplying coronary arteries, 12(86%) were related to the coronary artery aneurysms. Two were not related to

Septated pericarditis associated with Kawasaki disease: a brief case report.

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Sonçaği, Arzu; Devrim, Ilker; Karagöz, Tevfik; Dilber, Embiya; Celiker, Alpay; Ozen, Seza; Seçmeer, Gülten

2007-01-01

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.

The prevention of coronary arterial abnormalities in Kawasaki disease: A meta-analysis of the corticosteroid effectiveness.

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Yang, Tsung-Ju; Lin, Ming-Tai; Lu, Chun-Yi; Chen, Jong-Min; Lee, Ping-Ing; Huang, Li-Min; Wu, Mei-Hwan; Chang, Luan-Yin

2017-09-06

The use of corticosteroid in Kawasaki disease (KD) remains controversial among current guidelines. The objective of this study is to summarize the effectiveness and safety of corticosteroid to prevent coronary arterial lesions in Kawasaki disease, both as initial and rescue therapy. The Medline, EMBASE, Google scholar, Cochrane Central Register of Controlled Trials databases, ClinicalTrials.gov, and Japanese Institutional Repositories Online were searched for studies up to 31 March 2017. Studies that compared incidence of coronary artery lesions between regimens with corticosteroid and regimen without it in a well-defined controlled group were included. The incidence of coronary artery lesion was analyzed by meta-analysis. Nineteen studies published between 1999 and 2016 fulfilled eligibility criteria. There were 3591 patients included for analysis. There was a significant reduction in incidence of coronary artery lesions with usage of corticosteroid with a pooled odds ratio of 0.72 (95% CI 0.57-0.92; p = 0.01) than that without usage of corticosteroid. In general, a greater effect was seen in the patient received corticosteroid as initial and adjuvant therapy with intravenous immune globulin (pooled odds ratio 0.39, 95% CI 0.21-0.73, p = 0.007) than those who received corticosteroid as rescue therapy. The risk reduction was statistically significant in Japanese groups (OR 0.56, 95% CI 0.42-0.75 in fixed effects model) but not significant in non-Japanese groups (OR 1.45, 95% CI 0.91-2.30 in fixed effects model). We demonstrated an overall reduction in incidence of coronary artery lesions with the use of corticosteroid as initial and adjuvant treatment for Kawasaki disease. Copyright © 2017. Published by Elsevier B.V.

Evidence of correlation between TGFBR2 gene expression mediated by NF-kB signaling pathways and Kawasaki disease in children.

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Gao, Qinling; Yuan, Shuhua; Yuan, Dawei

2017-09-15

We explored the correlation between the TGFBR2 gene that is mediated by NF-kb signaling pathways and the pathogenesis of Kawasaki disease in children. In this study, 43 children with Kawasaki disease from April 2014 to January 2016 at our hospital were selected as the observation group, and 42 healthy children were selected as the control group. The mRNA expression levels of NF-kb gene and TGFBR2 gene in different groups were detected using fluorescence quantitative PCR. The protein expression levels of the NF-kb and TGFBR2 were detected using enzyme-linked immunosorbent assay (ELISA) in different groups. The expression levels of NF-kb and TGFBR2 in the observation group and the control group were detected using immunohistochemistry. Compared to the control group, the mRNA expression levels of NF-kb and TGFBR2 were 12.3 times and 27.5 times as high as those in the control group respectively and there were significant differences between the two groups (pkb and TGFBR2 in the control group (0.87±0.12, 1.25±0.18) ug/l and those in the observation group (3.27±0.17, 8.16±0.22) ug/l (pkB and TGFBR2 in children with Kawasaki disease were significantly higher than those in healthy subjects (pkB signaling pathways.

Predicting Coronary Artery Aneurysms in Kawasaki Disease at a North American Center: An Assessment of Baseline z Scores.

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Son, Mary Beth F; Gauvreau, Kimberlee; Kim, Susan; Tang, Alexander; Dedeoglu, Fatma; Fulton, David R; Lo, Mindy S; Baker, Annette L; Sundel, Robert P; Newburger, Jane W

2017-05-31

Accurate risk prediction of coronary artery aneurysms (CAAs) in North American children with Kawasaki disease remains a clinical challenge. We sought to determine the predictive utility of baseline coronary dimensions adjusted for body surface area ( z scores) for future CAAs in Kawasaki disease and explored the extent to which addition of established Japanese risk scores to baseline coronary artery z scores improved discrimination for CAA development. We explored the relationships of CAA with baseline z scores; with Kobayashi, Sano, Egami, and Harada risk scores; and with the combination of baseline z scores and risk scores. We defined CAA as a maximum z score (zMax) ≥2.5 of the left anterior descending or right coronary artery at 4 to 8 weeks of illness. Of 261 patients, 77 patients (29%) had a baseline zMax ≥2.0. CAAs occurred in 15 patients (6%). CAAs were strongly associated with baseline zMax ≥2.0 versus Baseline zMax ≥2.0 had a C statistic of 0.77, good sensitivity (80%), and excellent negative predictive value (98%). None of the risk scores alone had adequate discrimination. When high-risk status per the Japanese risk scores was added to models containing baseline zMax ≥2.0, none were significantly better than baseline zMax ≥2.0 alone. In a North American center, baseline zMax ≥2.0 in children with Kawasaki disease demonstrated high predictive utility for later development of CAA. Future studies should validate the utility of our findings. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Assessment of coronary artery aneurysms in paediatric patients with Kawasaki disease by multidetector row CT angiography: feasibility and comparison with 2D echocardiography

International Nuclear Information System (INIS)

Chu, W.C.W.; Lam, W.W.M.; Mok, G.C.F.; Yam, M.; Sung, R.Y.T.

2006-01-01

Transthoracic ECHO is the locally accepted method for coronary surveillance of patients with Kawasaki disease but it may have limited visualization in the older child. To assess the feasibility of multidetector CT (MDCT) angiography in the follow-up of coronary artery aneurysms in children with previous Kawasaki disease. Six children (5 boys, 1 girl; mean age 11.5 years) with known Kawasaki disease and coronary artery involvement underwent CT coronary angiography using 16-detector MDCT. The visualized lengths and diameter of all coronary segments were measured. The number, size and location of coronary artery aneurysms were recorded and compared with recent ECHO. Twelve coronary artery aneurysms (seven saccular, five fusiform) were identified by MDCT angiography. One saccular aneurysm at the junction of the distal right coronary artery and posterior descending artery was not detected by ECHO while the remaining six in proximal segments were detected by both modalities. Two of five fusiform aneurysms were not detected by ECHO due to their small sizes. Excellent agreement was found between CT and ECHO for maximal diameter and length of the visualized aneurysms. MDCT angiography accurately defines coronary artery aneurysms. It is more sensitive for detecting aneurysms at distal coronary segments and fusiform aneurysms of small size

Replication and meta-analysis of GWAS identified susceptibility loci in Kawasaki disease confirm the importance of B lymphoid tyrosine kinase (BLK) in disease susceptibility

NARCIS (Netherlands)

Chang, Chia-Jung; Kuo, Ho-Chang; Chang, Jeng-Sheng; Lee, Jong-Keuk; Tsai, Fuu-Jen; Khor, Chiea Chuen; Chang, Li-Ching; Chen, Shih-Ping; Ko, Tai-Ming; Liu, Yi-Min; Chen, Ying-Ju; Hong, Young Mi; Jang, Gi Young; Hibberd, Martin L.; Kuijpers, Taco; Burgner, David; Levin, Michael; Burns, Jane C.; Davila, Sonia; Chen, Yuan-Tsong; Chen, Chien-Hsiun; Wu, Jer-Yuarn; Lee, Yi-Ching; Liang, Chi-Di; Hwang, Kao-Pin; Chang, Luan-Yin; Huang, Li-Min; Chen, Ming-Ren; Chi, Hsin; Huang, Fu-Yuan; Chiu, Nan-Chang; Lee, Meng-Luen; Huang, Yhu-Chering; Hwang, Betau; Lee, Pi-Chang; Yoo, Jeong-Jin; Park, In-Sook; Hong, Soo-Jong; Kim, Kwi-Joo; Kim, Jae-Jung; Sohn, Saejung; Young Jang, Gi; Ha, Kee-Soo; Nam, Hyo-Kyoung; Byeon, Jung-Hye; Yun, Sin Weon; Han, Myung Ki; Kuipers, Irene M.; Ottenkamp, Jaap J.; Biezeveld, Maarten

2013-01-01

The BLK and CD40 loci have been associated with Kawasaki disease (KD) in two genome-wide association studies (GWAS) conducted in a Taiwanese population of Han Chinese ancestry (Taiwanese) and in Japanese cohorts. Here we build on these findings with replication studies of the BLK and CD40 loci in

Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease.

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Philip, Saji; Lee, Wen-Chuan; Cherian, Kotturathu Mammen; Wu, Mei-Hwan; Lue, Hung-Chi

2017-08-01

Horse serum-induced immune complex coronary vasculitis in swine is the first experimental model to mimic most of the pictures of Kawasaki disease. Immune complex mechanism has been implicated as one of the possible mechanisms in the pathogenesis of vasculitis in Kawasaki disease. Antioxidants have a significant role in the reduction of cardiovascular diseases in both human and animal studies. We tried giving vitamins A, E, and C to treat immune complex vasculitis, in the hope of mitigating coronary vasculitis in Kawasaki disease. Our study group consisted of 30 pure bred male piglets of 2-3 months of age, and they were divided into test and control groups. The test (AEC) group (n = 20) received two doses of horse serum, 10 mL (0.65 g protein)/kg body weight at 5-day intervals, and oral vitamins A, E, and C once daily for 14 days. The control group (n = 10) was further divided into the saline group (n = 3) receiving two doses of normal saline and the horse serum group (n = 7) receiving two doses of horse serum at 5-day intervals. Piglets were observed for the rashes and coronary artery dimensions. Both the AEC and the control horse serum group developed rashes after horse serum infusions, but the AEC group developed significantly fewer rashes, and no rashes were seen in the saline group. The control horse serum group (mean ± standard deviation = 2.13 ± 0.72) showed significant coronary artery dilatation, whereas there was no significant dilatation in the AEC group (mean ± standard deviation = 0.81 ± 0.58) or the control saline group (p = 0.002). Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin. Copyright © 2017. Published by Elsevier B.V.

Augmented TLR2 Expression on Monocytes in both Human Kawasaki Disease and a Mouse Model of Coronary Arteritis

OpenAIRE

Lin, I-Chun; Kuo, Ho-Chang; Lin, Ying-Jui; Wang, Feng-Shen; Wang, Lin; Huang, Shun-Chen; Chien, Shao-Ju; Huang, Chien-Fu; Wang, Chih-Lu; Yu, Hong-Ren; Chen, Rong-Fu; Yang, Kuender D.

2012-01-01

BACKGROUND: Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. METHODS: Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globul...

Health related quality of life and perceptions of child vulnerability among parents of children with a history of Kawasaki disease

NARCIS (Netherlands)

van Oers, Hedy A.; Tacke, Carline E.; Haverman, Lotte; Kuipers, Irene M.; Maurice-Stam, Heleen; Kuijpers, Taco W.; Grootenhuis, Martha A.

2014-01-01

Kawasaki disease (KD) is an acute paediatric vasculitis. The psychosocial consequences of this sudden illness for parents are unknown. This study aimed to evaluate health related quality of life (HRQOL) and parental perceptions of child vulnerability (PPCV) in parents of children with KD, and to

Kawasaki Disease with Retropharyngeal Edema following a Blackfly Bite

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Toru Watanabe

2014-01-01

Full Text Available We describe a patient with Kawasaki disease (KD and retropharyngeal edema following a blackfly bite. An 8-year-old boy was referred to our hospital because of a 3-day-history of fever and left neck swelling and redness after a blackfly bite. Computed tomography of the neck revealed left cervical lymph nodes swelling with edema, increased density of the adjacent subcutaneous tissue layer, and low density of the retropharyngeum. The patient was initially presumed to have cervical cellulitis, lymphadenitis, and retropharyngeal abscess. He was administered antibiotics intravenously, which did not improve his condition. The patient subsequently exhibited other signs of KD and was diagnosed with KD and retropharyngeal edema. Intravenous immunoglobulin therapy and oral flurbiprofen completely resolved the symptoms and signs. A blackfly bite sometimes incites a systemic reaction in humans due to a hypersensitive reaction to salivary secretions, which may have contributed to the development of KD in our patient.

Cardiac Complications, Earlier Treatment, and Initial Disease Severity in Kawasaki Disease.

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Abrams, Joseph Y; Belay, Ermias D; Uehara, Ritei; Maddox, Ryan A; Schonberger, Lawrence B; Nakamura, Yosikazu

2017-09-01

To assess if observed higher observed risks of cardiac complications for patients with Kawasaki disease (KD) treated earlier may reflect bias due to confounding from initial disease severity, as opposed to any negative effect of earlier treatment. We used data from Japanese nationwide KD surveys from 1997 to 2004. Receipt of additional intravenous immunoglobulin (IVIG) (data available all years) or any additional treatment (available for 2003-2004) were assessed as proxies for initial disease severity. We determined associations between earlier or later IVIG treatment (defined as receipt of IVIG on days 1-4 vs days 5-10 of illness) and cardiac complications by stratifying by receipt of additional treatment or by using logistic modeling to control for the effect of receiving additional treatment. A total of 48 310 patients with KD were included in the analysis. In unadjusted analysis, earlier IVIG treatment was associated with a higher risk for 4 categories of cardiac complications, including all major cardiac complications (risk ratio, 1.10; 95% CI, 1.06-1.15). Stratifying by receipt of additional treatment removed this association, and earlier IVIG treatment became protective against all major cardiac complications when controlling for any additional treatment in logistic regressions (OR, 0.90; 95% CI, 0.80-1.00). Observed higher risks of cardiac complications among patients with KD receiving IVIG treatment on days 1-4 of the illness are most likely due to underlying higher initial disease severity, and patients with KD should continue to be treated with IVIG as early as possible. Published by Elsevier Inc.

[Giant coronary aneurysms in infants with Kawasaki disease].

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Sánchez Andrés, Antonio; Salvador Mercader, Inmaculada; Seller Moya, Julia; Carrasco Moreno, José Ignacio

2017-08-01

Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Lack of association between infection with a novel human coronavirus (HCoV), HCoV-NH, and Kawasaki disease in Taiwan

NARCIS (Netherlands)

Chang, Luan-Yin; Chiang, Bor-Luen; Kao, Chuan-Liang; Wu, Mei-Hwan; Chen, Pei-Jer; Berkhout, Ben; Yang, Hui-Ching; Huang, Li-Min

2006-01-01

We investigated whether infection with a novel human coronavirus (HCoV), called "New Haven coronavirus" (HCoV-NH)--which is similar to and likely represents the same species as another novel HCoV, HCoV-NL63--is associated with Kawasaki disease (KD) in Taiwan. Fifty-three patients with KD were

FCGR2A Promoter Methylation and Risks for Intravenous Immunoglobulin Treatment Responses in Kawasaki Disease

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Ho-Chang Kuo

2015-01-01

Full Text Available Kawasaki disease (KD is characterized by pediatric systemic vasculitis of an unknown cause. The low affinity immunoglobulin gamma Fc region receptor II-a (FCGR2A gene was reported to be involved in the susceptibility of KD. DNA methylation is one of the epigenetic mechanisms that control gene expression; thus, we hypothesized that methylation status of CpG islands in FCGR2A promoter associates with the susceptibility and therapeutic outcomes of Kawasaki disease. In this study, 36 KD patients and 24 healthy subjects from out-patient clinic were recruited. Eleven potential methylation sites within the targeted promoter region of FCGR2A were selected for investigation. We marked the eleven methylation sites from A to K. Our results indicated that methylation at the CpG sites G, H, and J associated with the risk of KD. CpG sites B, C, E, F, H, J, and K were found to associate with the outcomes of IVIG treatment. In addition, CpG sites G, J, and K were predicted as transcription factors binding sites for NF-kB, Myc-Max, and SP2, respectively. Our study reported a significant association among the promoter methylation of FCGR2A, susceptibility of KD, and the therapeutic outcomes of IVIG treatment. The methylation levels of CpG sites of FCGR2A gene promoter should be an important marker for optimizing IVIG therapy.

Lipoma arborescens: Comparison of typical and atypical disease presentations

International Nuclear Information System (INIS)

Howe, B.M.; Wenger, D.E.

2013-01-01

Aim: To determine whether the aetiology differed between typical cases of lipoma arborescens with unilateral knee involvement and atypical cases involving joints other than the knee, polyarticular disease, and disease outside of the knee joint. Materials and methods: Cases of lipoma arborescens involving the knee joint were evaluated for the distribution of the disease and severity of degenerative arthritis. Joints other than the knee were evaluated for the presence and severity of degenerative arthritis, and the distribution was classified as either intra-articular, extra-articular, or both. Clinical history was reviewed for patient age at presentation, a history of inflammatory arthritis, diabetes mellitus, and known steroid use. Fisher's exact test was used to determine whether there was a statistically significant difference between typical and atypical presentations of the disease. Results: Lipoma arborescens was identified in 45 joints in 39 patients. Twenty-eight patients were classified as “typical” and 11 patients had “atypical” disease. There was no significant difference in age at presentation, presence of degenerative arthritis, or known inflammatory arthritis when comparing typical and atypical presentations of the disease. Conclusion: Twenty-eight percent of patients in the present study had atypical presentation of lipoma arborescens with multifocal lipoma arborescens or disease in joints other than the knee. There was no significant difference in age at presentation, presence of degenerative arthritis, or known inflammatory arthritis when comparing typical and atypical presentations of the disease. Of the 39 patients, only three had no evidence of degenerative arthritis, which suggests that many cases of lipoma arborescens are secondary to chronic reactive change in association with degenerative arthritis

Seasonality of Kawasaki Disease: A Global Perspective

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Burns, Jane C.; Herzog, Lauren; Fabri, Olivia; Tremoulet, Adriana H.; Rodó, Xavier; Uehara, Ritei; Burgner, David; Bainto, Emelia; Pierce, David; Tyree, Mary; Cayan, Daniel

2013-01-01

Background Understanding global seasonal patterns of Kawasaki disease (KD) may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide. Methods Data from 1970-2012 from 25 countries distributed over the globe were analyzed for seasonality. The number of KD cases from each location was normalized to minimize the influence of greater numbers from certain locations. The presence of seasonal variation of KD at the individual locations was evaluated using three different tests: time series modeling, spectral analysis, and a Monte Carlo technique. Results A defined seasonal structure emerged demonstrating broad coherence in fluctuations in KD cases across the Northern Hemisphere extra-tropical latitudes. In the extra-tropical latitudes of the Northern Hemisphere, KD case numbers were highest in January through March and approximately 40% higher than in the months of lowest case numbers from August through October. Datasets were much sparser in the tropics and the Southern Hemisphere extra-tropics and statistical significance of the seasonality tests was weak, but suggested a maximum in May through June, with approximately 30% higher number of cases than in the least active months of February, March and October. The seasonal pattern in the Northern Hemisphere extra-tropics was consistent across the first and second halves of the sample period. Conclusion Using the first global KD time series, analysis of sites located in the Northern Hemisphere extra-tropics revealed statistically significant and consistent seasonal fluctuations in KD case numbers with high numbers in winter and low numbers in late summer and fall. Neither the tropics nor the Southern Hemisphere extra-tropics registered a statistically significant aggregate seasonal cycle. These data suggest a seasonal exposure to a KD agent that operates over large geographic regions and is concentrated during winter

Parental anxiety associated with Kawasaki disease in previously healthy children.

Science.gov (United States)

Chahal, Nita; Clarizia, Nadia A; McCrindle, Brian W; Boydell, Katherine M; Obadia, Maya; Manlhiot, Cedric; Dillenburg, Rejane; Yeung, Rae S M

2010-01-01

The objective of this study was to explore the lived experience of parents of children diagnosed with Kawasaki disease (KD) and to identify factors associated with increased levels of parental anxiety. Three focus groups were conducted including 25 parents of 17 patients with KD, seven (41%) of whom had coronary artery complications. A conceptual model was developed to depict parental experiences and illustrate the key issues related to heightened anxiety. Themes identified included anxiety related to the child's sudden illness and delay in obtaining a correct diagnosis because of the lack of health care providers' awareness and knowledge regarding KD. Parents were frustrated by the lack of information available in lay language and the limited scientific knowledge regarding the long-term consequences of the disease. Parents also reported positive transformations and different perspective toward challenges in life. However, the parents of children with coronary artery complications expressed persistent anxiety even years after the acute phase of the illness due to the uncertainty of the long-term prognosis. There remains a critical need for richly textured research data on the perspective and experience of families of children with KD. Copyright 2010 National Association of Pediatric Nurse Practitioners. Published by Mosby, Inc. All rights reserved.

Safety and Efficacy of Warfarin Therapy in Kawasaki Disease.

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Baker, Annette L; Vanderpluym, Christina; Gauvreau, Kimberly A; Fulton, David R; de Ferranti, Sarah D; Friedman, Kevin G; Murray, Jenna M; Brown, Loren D; Almond, Christopher S; Evans-Langhorst, Margaret; Newburger, Jane W

2017-10-01

To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications. We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR >6 occurred in 3 patients (4 events); INRs ≥5 warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin. Copyright © 2017 Elsevier Inc. All rights reserved.

Kawasaki disease with G6PD deficiency--report of one case and literature review.

Science.gov (United States)

Chen, Chia-Hao; Lin, Li-Yan; Yang, Kuender D; Hsieh, Kai-Sheng; Kuo, Ho-Chang

2014-06-01

Kawasaki disease (KD) is a systemic vasculitis primarily affecting children who are younger than 5 years. The most serious complications are coronary artery aneurysms and sequelae of vasculitis with the subsequent development of coronary artery aneurysm. According to the literature, intravenous immunoglobulin (IVIG) plus high-dose aspirin (acetylsalicylic acid) were standard treatment for KD, whereas low-dose aspirin (3-5 mg/kg/day) was used for thrombocytosis in KD via antiplatelet effect. However, aspirin has been reported to have hemolytic potential in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency. We report a child with G6PD-deficiency who has KD, and review the literature. Copyright © 2012. Published by Elsevier B.V.

Predicting the characteristics of the aetiological agent for Kawasaki disease from other paediatric infectious diseases in Japan.

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Nagao, Y; Urabe, C; Nakamura, H; Hatano, N

2016-02-01

Although Kawasaki disease (KD), which was first reported in the 1960s, is assumed to be infectious, its aetiological agent(s) remains unknown. We compared the geographical distribution of the force of infection and the super-annual periodicity of KD and seven other paediatric infectious diseases in Japan. The geographical distribution of the force of infection, which was estimated as the inverse of the mean patient age, was similar in KD and other paediatric viral infections. This similarity was due to the fact that the force of infection was determined largely by the total fertility rate. This finding suggests that KD shares a transmission route, i.e. sibling-to-sibling infection, with other paediatric infections. The super-annual periodicity, which is positively associated with the sum of an infectious disease's incubation period and infectious period, was much longer for KD and exanthema subitum than other paediatric infectious diseases. The virus for exanthema subitum is known to persist across the host's lifespan, which suggests that the aetiological agent for KD may also be capable of persistent infection. Taken together, these findings suggest that the aetiological agent for KD is transmitted through close contact and persists asymptomatically in most hosts.

Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease

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Saji Philip

2017-08-01

Conclusion: Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin.

Atrioventricular depolarization differences identify coronary artery anomalies in Kawasaki disease.

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Cortez, Daniel; Sharma, Nandita; Jone, Pei-Ni

2017-03-01

Kawasaki disease (KD) is the leading cause of acquired heart disease in children. Signal average electrocardiogram changes in patients during the acute phase of KD with coronary artery anomalies (CAA) include depolarization changes. We set out to determine if 12-lead-derived atrioventricular depolarization differences can identify CAA in patients with KD. A blinded, retrospective case-control study of patients with KD was performed. Deep Q waves, corrected QT-intervals (QTc), spatial QRS-T angles, T-wave vector magnitudes (RMS-T), and a novel parameter for assessment of atrioventricular depolarization difference (the spatial PR angle) and a two dimensional PR angle were assessed. Comparisons between groups were performed to test for significant differences. One hundred one patients with KD were evaluated, with 68 having CAA (67.3%, mean age 3.6 ± 3.0 years, 82.6% male), and 32 without CAA (31.7%, mean age 2.7 ± 3.2 years, 70.4% male). The spatial PR angle significantly discriminated KD patients with CAA from those without, 59.7° ± 31.1° versus 41.6° ± 11.5° (p differences, measured by the spatial or two dimensional PR angle differentiate KD patients with CAA versus those without. © 2016 Wiley Periodicals, Inc.

Atypical Celiac Disease: From Recognizing to Managing

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B. Admou

2012-01-01

Full Text Available The nonclassic clinical presentation of celiac disease (CD becomes increasingly common in physician’s daily practice, which requires an awareness of its many clinical faces with atypical, silent, and latent forms. Besides the common genetic background (HLA DQ2/DQ8 of the disease, other non-HLA genes are now notably reported with a probable association to atypical forms. The availability of high-sensitive and specific serologic tests such as antitissue transglutuminase, antiendomysium, and more recent antideamidated, gliadin peptide antibodies permits to efficiently uncover a large portion of the submerged CD iceberg, including individuals having conditions associated with a high risk of developing CD (type 1 diabetes, autoimmune diseases, Down syndrome, family history of CD, etc., biologic abnormalities (iron deficiency anemia, abnormal transaminase levels, etc., and extraintestinal symptoms (short stature, neuropsychiatric disorders, alopecia, dental enamel hypoplasia, recurrent aphtous stomatitis, etc.. Despite the therapeutic alternatives currently in developing, the strict adherence to a GFD remains the only effective and safe therapy for CD.

Evaluation of echogenicity of the heart in Kawasaki disease.

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Nagata, Hazumu; Yamamura, Kenichiro; Uike, Kiyoshi; Nakashima, Yasutaka; Hirata, Yuichiro; Morihana, Eiji; Mizuno, Yumi; Ishikawa, Shiro; Hara, Toshiro

2014-08-01

Pathologic studies of the heart in patients with Kawasaki disease (KD) revealed vasculitis, valvulitis, myocarditis, and pericarditis. However, there have been no studies on the quantitative determination of multi-site echogenicity of the heart in KD patients. It is also undetermined whether the degree of echogenicity of each site of the heart in patients with KD might be related to the response to intravenous immunoglobulin (IVIG) treatment. In 81 KD patients and 30 control subjects, we prospectively analyzed echogenicity of the heart. Echogenicity was measured in four sites: coronary artery wall (CAW), mitral valve (MV), papillary muscle (PM), and ascending aortic wall (AAo wall) by the calibrated integrated backscatters (cIBs). The cIB values of all measurement sites at acute phase in KD patients were significantly higher than those in control subjects (KD patients vs control subjects; CAW, 19.8 ± 6.2 dB vs 14.5 ± 2.0 dB, p IVIG nonresponders were significantly higher than those in responders. Conclusion: Echogenicity of the heart in KD patients at the acute phase increased not only in the coronary artery wall but also in other parts of the heart. Echogenicity of CAW might be helpful in determining the unresponsiveness of IVIG treatment.

Usefulness of 64-slice MDCT for follow-up of young children with coronary artery aneurysm due to Kawasaki disease: initial experience.

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Peng, Yun; Zeng, Jinjin; Du, Zhongdong; Sun, Guoqiang; Guo, Huling

2009-03-01

To evaluate the initial application and value of 64-slice multidetector computed tomography as an alternative diagnostic modality in the follow-up of young children with coronary artery aneurysm due to Kawasaki disease. Twelve boys (mean age 5.1 years, range 1.8-7.8 years) for follow-up (time range from 1.1 to 5.1 years) of known Kawasaki disease and coronary artery aneurysm underwent 64-slice MDCT ECG-gated coronary angiography. All data were acquired without breath holding. Two pediatric radiologists independently assessed image quality and the diameter of all coronary segments were measured for each patient. The number, position, shape and size of each coronary artery aneurysm were observed and compared with those of ECHO performed previously. A total of 118/156 segments permitted visualization with diagnostic image quality, the CT measurements showed good inter-observer and intra-observer reliability, coefficients were 0.93 and 0.88, respectively. A total of 30 coronary artery aneurysms were identified with measured mean of 7.5+/-3.8 mm in diameter, and of 12.4+/-9.1 mm in longitudinal lengths.10 tumors were small, 8 tumors were medium and 12 tumors were giant aneurysm. The affected segments included LM7/12(58.3%), 9/12(75%) of LAD1, 4/12(33.3%) of LAD2, 2/12(16.7%) of LCX1; 6/12(50%) of RCA1, 9/12(75%) of RCA2 and 4/12(33.3%) of RCA3, including affected two segments in 9 tumors and three segments in 1 tumor. Calcifications were found in 5 aneurysms and 3/5 with thrombosis; six stenotic segments were found. ECHO failed to detect 8 tumors with 2/8 in LAD, 1/8 in LCX and 5/8 in RCA, and those included 4 small aneurysms. The use of 64-slice MDCT angiography proved valuable for monitoring young children with Kawasaki disease. However, further study is necessary to specify the sensitivity and specificity of MDCT in the follow-up.

Usefulness of 64-slice MDCT for follow-up of young children with coronary artery aneurysm due to Kawasaki disease: Initial experience

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Peng Yun; Zeng Jinjin; Du Zhongdong; Sun Guoqiang; Guo Huling

2009-01-01

To evaluate the initial application and value of 64-slice multidetector computed tomography as an alternative diagnostic modality in the follow-up of young children with coronary artery aneurysm due to Kawasaki disease. Twelve boys (mean age 5.1 years, range 1.8-7.8 years) for follow-up (time range from 1.1 to 5.1 years) of known Kawasaki disease and coronary artery aneurysm underwent 64-slice MDCT ECG-gated coronary angiography. All data were acquired without breath holding. Two pediatric radiologists independently assessed image quality and the diameter of all coronary segments were measured for each patient. The number, position, shape and size of each coronary artery aneurysm were observed and compared with those of ECHO performed previously. A total of 118/156 segments permitted visualization with diagnostic image quality, the CT measurements showed good inter-observer and intra-observer reliability, coefficients were 0.93 and 0.88, respectively. A total of 30 coronary artery aneurysms were identified with measured mean of 7.5 ± 3.8 mm in diameter, and of 12.4 ± 9.1 mm in longitudinal lengths.10 tumors were small, 8 tumors were medium and 12 tumors were giant aneurysm. The affected segments included LM7/12(58.3%), 9/12(75%) of LAD1, 4/12(33.3%) of LAD2, 2/12(16.7%) of LCX1; 6/12(50%) of RCA1, 9/12(75%) of RCA2 and 4/12(33.3%) of RCA3, including affected two segments in 9 tumors and three segments in 1 tumor. Calcifications were found in 5 aneurysms and 3/5 with thrombosis; six stenotic segments were found. ECHO failed to detect 8 tumors with 2/8 in LAD, 1/8 in LCX and 5/8 in RCA, and those included 4 small aneurysms. The use of 64-slice MDCT angiography proved valuable for monitoring young children with Kawasaki disease. However, further study is necessary to specify the sensitivity and specificity of MDCT in the follow-up.

Usefulness of 64-slice MDCT for follow-up of young children with coronary artery aneurysm due to Kawasaki disease: Initial experience

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Peng Yun [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)], E-mail: ppengyun@yahoo.com; Zeng Jinjin [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Du Zhongdong [Pediatric Cardiovascular Department, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Sun Guoqiang [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Guo Huling [Pediatric Cardiovascular Department, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)

2009-03-15

To evaluate the initial application and value of 64-slice multidetector computed tomography as an alternative diagnostic modality in the follow-up of young children with coronary artery aneurysm due to Kawasaki disease. Twelve boys (mean age 5.1 years, range 1.8-7.8 years) for follow-up (time range from 1.1 to 5.1 years) of known Kawasaki disease and coronary artery aneurysm underwent 64-slice MDCT ECG-gated coronary angiography. All data were acquired without breath holding. Two pediatric radiologists independently assessed image quality and the diameter of all coronary segments were measured for each patient. The number, position, shape and size of each coronary artery aneurysm were observed and compared with those of ECHO performed previously. A total of 118/156 segments permitted visualization with diagnostic image quality, the CT measurements showed good inter-observer and intra-observer reliability, coefficients were 0.93 and 0.88, respectively. A total of 30 coronary artery aneurysms were identified with measured mean of 7.5 {+-} 3.8 mm in diameter, and of 12.4 {+-} 9.1 mm in longitudinal lengths.10 tumors were small, 8 tumors were medium and 12 tumors were giant aneurysm. The affected segments included LM7/12(58.3%), 9/12(75%) of LAD1, 4/12(33.3%) of LAD2, 2/12(16.7%) of LCX1; 6/12(50%) of RCA1, 9/12(75%) of RCA2 and 4/12(33.3%) of RCA3, including affected two segments in 9 tumors and three segments in 1 tumor. Calcifications were found in 5 aneurysms and 3/5 with thrombosis; six stenotic segments were found. ECHO failed to detect 8 tumors with 2/8 in LAD, 1/8 in LCX and 5/8 in RCA, and those included 4 small aneurysms. The use of 64-slice MDCT angiography proved valuable for monitoring young children with Kawasaki disease. However, further study is necessary to specify the sensitivity and specificity of MDCT in the follow-up.

Atypical form of cat scratch disease in immunocompetent patient

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Kojić Miroslav

2013-01-01

Full Text Available Introduction. Cat scratch disease (CSD is an acute infectious disease with benign course caused by the bacteria Bartonella henselae. Clinically, it is usually manifested as regional lymphadenopathy and mild infective syndrome. Rare forms of the disease which usually occur in immunocompromised presons are: encephalitis, transverse myelitis, neuroretinitis, granulomatosus conjunctivitis, arthritis, hepatitis etc. Case report. We presented an atypical form of cat scratch disease in a young immunocompetent female person. The disease was manifested with prolonged fever, rash, purulent lymphadenitis and hepatitis. The diagnosis was based on characteristic patohystological finding and exclusion of the other causes of lymphadenopathy. The patient was treated by antibiotics for a few weeks, with surgical incision and drainage of the purulent lymphadenitis. Conclusion. Atypical forms of CSD could be an important differential-diagnostic problem, especially if there is no opportunity for serological confirmation of the disease.

Wilson’s disease: Atypical imaging features

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Venugopalan Y Vishnu

2016-10-01

Full Text Available Wilson’s disease is a genetic movement disorder with characteristic clinical and imaging features. We report a 17- year-old boy who presented with sialorrhea, hypophonic speech, paraparesis with repeated falls and recurrent seizures along with cognitive decline. He had bilateral Kayser Flescher rings. Other than the typical features of Wilson’s disease in cranial MRI, there were extensive white matter signal abnormalities (T2 and FLAIR hyperintensities and gyriform contrast enhancement which are rare imaging features in Wilson's disease. A high index of suspicion is required to diagnose Wilson’s disease when atypical imaging features are present.

Comparison of Endoscopic and Histological Findings between Typical and Atypical Celiac Disease in Children.

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Semwal, Pooja; Gupta, Raj Kumar; Sharma, Rahul; Garg, Kapil

2018-04-01

Celiac disease is a common non-communicable disease with varied presentations. Purpose of this study was to find the duodeno-endoscopic features in celiac disease and to compare duodeno-endoscopic and histological findings between typical and atypical celiac disease in children. Hospital based observational study was conducted at Sir Padampat Mother and Child Health Institute, Jaipur from June 2015 to May 2016. Patients were selected and divided in two groups- typical and atypical celiac disease based upon the presenting symptoms. Upper gastrointestinal endoscopy and duodenal biopsy was performed for serology positive patients. Results were analysed using appropriate statistical test of significance. Out of 101 enrolled patients, 47.5% were male. Age ranged from 1 to 18 years. Study showed that 54.5% were typical and 45.5% were atypical. Patients presenting with atypical symptoms were predominantly of older age group. On endoscopy, scalloping, mosaic pattern, reduced fold height and absent fold height; and in histology, advanced Marsh stage were significantly higher in the typical group. Awareness of atypical presentations as well as duodeno-endoscopic features may have considerable practical importance for the diagnosis of celiac disease in children. Scalloping, mosaic pattern, reduced fold height and nodularity are main endoscopic markers of celiac disease in children. Endoscopic markers of duodenal mucosa may be important in early diagnosis of celiac disease, in children subjected to endoscopy for atypical presentations or indication other than suspected celiac disease.

Kawasaki disease-associated coronary artery lesions with navigator echo-based. Respiratory-gated three dimensional coronary magnetic resonance angiography compared with echocardiography in young children

International Nuclear Information System (INIS)

Amino, Masayuki; Teraoka, Kunihiko; Hirano, Masaharu; Kawashima, Naoshi; Kakizaki, Dai; Ookubo, Yasuo; Sasaki, Kazuyoshi; Katuyama, Hiroaki

2004-01-01

Navigator echo-based respiratory-gated three dimensional coronary magnetic resonance angiography (3D-CMRA) was compared with echocardiography, to determine whether 3D-CMRA was useful for the evaluation of Kawasaki disease-associated coronary artery lesions. Sixteen consecutive patients (imaging was performed 17 times in total) who were given a diagnosis of Kawasaki's disease at the pediatric department of our hospital and examined for the precise examination of complicating coronary artery lesions on MRI using a navigator-echo technique because of their incapability of holding their breath during imaging were entered into the present study. A 1.5T MRI system was used. Gd-DOTA was given at a total volume of 0.1 mmol/kg. During imaging, CMRA visualized the left coronary arteries in all 17 cases and the right coronary arteries in 16 cases, but not in one case. The left main coronary trunk segment no.5 was demonstrated in all cases with CMRA, but not in 4 cases with echocardiography. The left anterior descending branch no.6 was visualized in 11 of the 17 cases with CMRA, but only in 5 cases with echocardiography. The left circumflex branch no.11 was observed in 6 cases with CMRA, but only in 2 cases with echocardiography. As for the right coronary arteries, branches no.1 and no.2 were observed in 16 and 9 cases with CMRA, respectively, and in 13 and 3 cases with echocardiography, respectively. Vascular diameters measured on CMRA were almost identical to those on echocardiography, within the range of arteries visualized. 3D-CMRA combined with a navigator echo technique appears to be a useful tool for the observation of coronary artery lesions associated with Kawasaki's disease because it is superior in lesion visualization to echocardiography. (author)

Atypical presentations of celiac disease

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Balasa Adriana Luminita

2016-08-01

Full Text Available In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016 in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

Classification of coronary artery tissues using optical coherence tomography imaging in Kawasaki disease

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Abdolmanafi, Atefeh; Prasad, Arpan Suravi; Duong, Luc; Dahdah, Nagib

2016-03-01

Intravascular imaging modalities, such as Optical Coherence Tomography (OCT) allow nowadays improving diagnosis, treatment, follow-up, and even prevention of coronary artery disease in the adult. OCT has been recently used in children following Kawasaki disease (KD), the most prevalent acquired coronary artery disease during childhood with devastating complications. The assessment of coronary artery layers with OCT and early detection of coronary sequelae secondary to KD is a promising tool for preventing myocardial infarction in this population. More importantly, OCT is promising for tissue quantification of the inner vessel wall, including neo intima luminal myofibroblast proliferation, calcification, and fibrous scar deposits. The goal of this study is to classify the coronary artery layers of OCT imaging obtained from a series of KD patients. Our approach is focused on developing a robust Random Forest classifier built on the idea of randomly selecting a subset of features at each node and based on second- and higher-order statistical texture analysis which estimates the gray-level spatial distribution of images by specifying the local features of each pixel and extracting the statistics from their distribution. The average classification accuracy for intima and media are 76.36% and 73.72% respectively. Random forest classifier with texture analysis promises for classification of coronary artery tissue.

Identification of candidate diagnostic serum biomarkers for Kawasaki disease using proteomic analysis

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Kimura, Yayoi; Yanagimachi, Masakatsu; Ino, Yoko; Aketagawa, Mao; Matsuo, Michie; Okayama, Akiko; Shimizu, Hiroyuki; Oba, Kunihiro; Morioka, Ichiro; Imagawa, Tomoyuki; Kaneko, Tetsuji; Yokota, Shumpei; Hirano, Hisashi; Mori, Masaaki

2017-01-01

Kawasaki disease (KD) is a systemic vasculitis and childhood febrile disease that can lead to cardiovascular complications. The diagnosis of KD depends on its clinical features, and thus it is sometimes difficult to make a definitive diagnosis. In order to identify diagnostic serum biomarkers for KD, we explored serum KD-related proteins, which differentially expressed during the acute and recovery phases of two patients by mass spectrometry (MS). We identified a total of 1,879 proteins by MS-based proteomic analysis. The levels of three of these proteins, namely lipopolysaccharide-binding protein (LBP), leucine-rich alpha-2-glycoprotein (LRG1), and angiotensinogen (AGT), were higher in acute phase patients. In contrast, the level of retinol-binding protein 4 (RBP4) was decreased. To confirm the usefulness of these proteins as biomarkers, we analyzed a total of 270 samples, including those collected from 55 patients with acute phase KD, by using western blot analysis and microarray enzyme-linked immunosorbent assays (ELISAs). Over the course of this experiment, we determined that the expression level of these proteins changes specifically in the acute phase of KD, rather than the recovery phase of KD or other febrile illness. Thus, LRG1 could be used as biomarkers to facilitate KD diagnosis based on clinical features. PMID:28262744

Enfermedad de kawasaki: A propósito de un caso

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Raquel Fernández Ferrán

2000-09-01

Full Text Available Se presenta una paciente de 2 meses de edad con los síntomas y signos de la enfermedad de Kawasaki. Se descartan mediante los exámenes complementarios otras enfermedades más comunes en la infancia. Se hace una revisión de la enfermedad y se señala la importancia del diagnóstico y tratamiento precoces. A pesar de la corta edad de la niña ésta no presentó afectación de las arterias coronarias. Su evolución fue favorable, y se observó mejoría (caída de la temperatura a menos de 38 C a partir del 4to. día después de comenzar el tratamiento. Se utilizó el esquema de dosis única de gammaglobulina asociada con los salicilatos.A two-months old female patient with symptoms and signs of Kawasaki disease is presented in this paper. Supplementary tests disregarded the presence of other common diseases in early childhood. The disease is reviewed, pointing out the importance of early diagnosis and treatment. Despite her small age, the girls did not have any lesion in coronary arteries. A recovery was observed (temperature fell below 38 C on the 4th day after beginning of the treatment. The single dosage of gammaglobulin scheme associated to salicylates was used.

Kawasaki Disease With Coronary Artery Aneurysms: Psychosocial Impact on Parents and Children.

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Chahal, Nita; Jelen, Ahlexxi; Rush, Janet; Manlhiot, Cedric; Boydell, Katherine M; Sananes, Renee; McCrindle, Brian W

For those living with Kawasaki disease and coronary artery aneurysms, little is known about the psychosocial burden faced by parents and their children. Exploratory, descriptive, mixed-methods design examining survey and interview data about health-related uncertainty, intrusiveness, and self-efficacy. Parents' uncertainty was associated with missed diagnosis, higher income, and maternal education. Higher uncertainty scores among children were associated with absence of chest pain and lower number of echocardiograms. High intrusiveness scores among parents were associated with previous cardiac catheterization, use of anticoagulants, lower parent education and income, and missed diagnosis. High intrusiveness scores among children were associated with high paternal education. Children's total self-efficacy scores increased with chest pain and larger aneurysm size. Qualitative analysis showed two central themes: Psychosocial Struggle and Cautious Optimism. Negative illness impact is associated with a more intense medical experience and psychosocial limitations. Timely assessment and support are warranted to meet parents' and children's needs. Copyright © 2016 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.

Olfactory and imaging features in atypical Alzheimer’s disease

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Huihong Zhang

2018-02-01

Full Text Available Cognition and speech disorders are the most common symptoms of dementia in neurodegenerative disease. Here, we present a detailed clinical evaluation of a case of logopenic variant of primary progressive aphasia (lv-PPA, an atypical form of Alzheimer disease (AD, including cognitive testing over time, brain imaging, electrophysiology, and tests of olfactory function.

Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey.

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Dinler, Gönül; Atalay, Erdal; Kalayci, Ayhan Gazi

2009-11-01

Celiac disease presents with a spectrum of clinical disorders. The variety of clinical presentations largely depends on age and extraintestinal findings. This study aimed to determine typical and atypical cases according to presenting symptoms and to evaluate their biochemical and pathological parameters. Eighty-seven patients with celiac disease in our unit between 2000 and 2007 were reviewed. Their diagnosis was made by serological and histological examination. The patients were divided into two groups according to their typical or atypical symptoms. The mean age of the patients at diagnosis was 8.2 years (range, 1-18 years), but patients presenting with typical symptoms were younger than those presenting with atypical symptoms. The patients in the two groups did not differ significantly in sex, weight and height Z scores except age. Diarrhea (96.3%), abdominal distention (65.4%) and failure to thrive (60%) were the most common clinical presentations in the typical group, and short stature (62.5%) and anemia (31.2%) were the most common in the atypical group. Total/subtotal villous atrophy was significantly higher in the typical group than in the atypical group. Many children with celiac disease show an atypical form. The understanding of presentations of celiac disease may prevent delayed diagnosis. Celiac disease should be specially investigated in patients with recurrent iron deficiency anemia, short stature and autoimmune disorders.

Genetic variants of CD209 associated with Kawasaki disease susceptibility.

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Ho-Chang Kuo

Full Text Available BACKGROUND: Kawasaki disease (KD is a systemic vasculitis with unknown etiology mainly affecting children in Asian countries. Dendritic cell-specific intercellular adhesion molecule-3 grabbing non-integrin (DC-SIGN, CD209 in humans was showed to trigger an anti-inflammatory cascade and associated with KD susceptibility. This study was conducted to investigate the association between genetic polymorphisms of CD209 and the risk KD. METHODS: A total of 948 subjects (381 KD and 567 controls were recruited. Nine tagging SNPs (rs8112310, rs4804800, rs11465421, rs1544766, rs4804801, rs2287886, rs735239, rs735240, rs4804804 were selected for TaqMan allelic discrimination assay. Clinical phenotypes, coronary artery lesions (CAL and intravenous immunoglobulin (IVIG treatment outcomes were collected for analysis. RESULTS: Significant associations were found between CD209 polymorphisms (rs4804800, rs2287886, rs735240 and the risk of KD. Haplotype analysis for CD209 polymorphisms showed that A/A/G haplotype (P = 0.0002, OR = 1.61 and G/A/G haplotype (P = 0.0365, OR = 1.52 had higher risk of KD as compared with G/G/A haplotype in rs2287886/rs735239/rs735240 pairwise allele analysis. There were no significant association in KD with regards to CAL formation and IVIG treatment responses. CONCLUSION: CD209 polymorphisms were responsible for the susceptibility of KD, but not CAL formation and IVIG treatment responsiveness.

Genetic Variants of CD209 Associated with Kawasaki Disease Susceptibility

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Kuo, Ho-Chang; Huang, Ying-Hsien; Chien, Shu-Chen; Yu, Hong-Ren; Hsieh, Kai-Sheng; Hsu, Yu-Wen; Chang, Wei-Chiao

2014-01-01

Background Kawasaki disease (KD) is a systemic vasculitis with unknown etiology mainly affecting children in Asian countries. Dendritic cell-specific intercellular adhesion molecule-3 grabbing non-integrin (DC-SIGN, CD209) in humans was showed to trigger an anti-inflammatory cascade and associated with KD susceptibility. This study was conducted to investigate the association between genetic polymorphisms of CD209 and the risk KD. Methods A total of 948 subjects (381 KD and 567 controls) were recruited. Nine tagging SNPs (rs8112310, rs4804800, rs11465421, rs1544766, rs4804801, rs2287886, rs735239, rs735240, rs4804804) were selected for TaqMan allelic discrimination assay. Clinical phenotypes, coronary artery lesions (CAL) and intravenous immunoglobulin (IVIG) treatment outcomes were collected for analysis. Results Significant associations were found between CD209 polymorphisms (rs4804800, rs2287886, rs735240) and the risk of KD. Haplotype analysis for CD209 polymorphisms showed that A/A/G haplotype (P = 0.0002, OR = 1.61) and G/A/G haplotype (P = 0.0365, OR = 1.52) had higher risk of KD as compared with G/G/A haplotype in rs2287886/rs735239/rs735240 pairwise allele analysis. There were no significant association in KD with regards to CAL formation and IVIG treatment responses. Conclusion CD209 polymorphisms were responsible for the susceptibility of KD, but not CAL formation and IVIG treatment responsiveness. PMID:25148534

Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease

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Hyo Min Park

2013-02-01

Full Text Available <b>Purpose:</b> It has been reported that 10% to 20% of children with Kawasaki disease (KD will not respond to intravenous immunoglobulin (IVIG treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD. <b>Methods:</b> We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005 and June 2011. We retrospectively reviewed their medical records and analyzed multiple parameters in responders and nonresponders to IVIG. <b>Results:</b> Among the 309 children, 30 (9.7% did not respond to IVIG. They had significantly higher proportion of neutrophils, and higher levels of aspartate aminotransferase, alanine aminotransferase (ALT, total bilirubin, and N-terminal fragment of B-type natriuretic peptide than did responders. IVIGnonresponders had a significantly longer duration of hospitalization, and more frequently experienced coronary artery lesion, and sterile pyuria. No differences in the duration of fever at initial treatment or, clinical features were noted. <b>Conclusion:</b> Two independent predictors (ALT?#248;4 IU/L, total bilirubin?#240;.9 mg/dL for nonresponse were confirmed through multivariate logistic regression analysis. Thus elevated ALT and total bilirubin levels might be useful in predicting nonresponse to IVIG therapy in children with KD.

Atypical early-onset Alzheimer's disease caused by the Iranian APP mutation

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Lindquist, S.G.; Nielsen, J.E.; Stokholm, J.

2008-01-01

BACKGROUND: Approximately 1% of all cases of Alzheimer's disease are inherited autosomal dominantly, and to date, three causative genes have been found, the Presenilin 1 (PSEN1) gene, the Presenilin 2 (PSEN2) gene and the Amyloid precursor protein (APP) gene. We describe atypical phenotypic...... features in a family with a pathogenic APP gene mutation and discuss possible explanations for these atypical features. METHODS AND RESULTS: We report a family with a history of dementia compatible with autosomal dominant transmission. The disease course in the proband was not typical for Alzheimer......'s disease as the diagnosis was preceded by 8 years of an isolated amnesia. Further, the proband had epilepsy with complex partial seizures and central degenerative autonomic failure as determined by clinical physiology. Sequencing the three known causative Alzheimer genes revealed a pathogenic missense...

Association of the Resistin Gene Promoter Region Polymorphism with Kawasaki Disease in Chinese Children

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Ruixi Liu

2012-01-01

Full Text Available Objectives. The −420C>G polymorphism located in the resistin gene (RETN promoter has recently been suggested to play a potential role in proinflammatory conditions and cardiovascular disease. This study investigated the association of the RETN promoter polymorphism with Kawasaki disease (KD and its clinical parameters in Chinese children. Methods. We compared patients with complete KD to incomplete KD children. Genotyping of the RETN promoter polymorphism was performed using MassARRAY system, and serum resistin levels were estimated using the sandwich enzyme immunoassay method. Results. There was no significant difference in RETN (−420C>G genotypes between KD and control groups. However, the frequency of the G allele was higher in iKD patients than in cKD children due to a significantly increased frequency of the GG genotypes. Serum levels of resistin were significantly higher in KD patients than in controls regardless of the presence of coronary artery lesions (CALs. Conclusion. The present findings suggest that while resistin may play a role in the pathogenesis of KD, there is no apparent association between CAL and the RETN (−420C>G gene polymorphism in KD children. However, the diagnosis of iKD is challenging but can be supported by the presence of the G allele and the GG genotypes.

Ethnic Kawasaki Disease Risk Associated with Blood Mercury and Cadmium in U.S. Children

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Yeter, Deniz; Portman, Michael A.; Aschner, Michael; Farina, Marcelo; Chan, Wen-Ching; Hsieh, Kai-Sheng; Kuo, Ho-Chang

2016-01-01

Kawasaki disease (KD) primarily affects children <5 years of age (75%–80%) and is currently the leading cause of acquired heart disease in developed nations. Even when residing in the West, East Asian children are 10 to 20 times more likely to develop KD. We hypothesized cultural variations influencing pediatric mercury (Hg) exposure from seafood consumption may mediate ethnic KD risk among children in the United States. Hospitalization rates of KD in US children aged 0–4 years (n = 10,880) and blood Hg levels in US children aged 1–5 years (n = 713) were determined using separate US federal datasets. Our cohort primarily presented with blood Hg levels <0.1 micrograms (µg) per kg bodyweight (96.5%) that are considered normal and subtoxic. Increased ethnic KD risk was significantly associated with both increasing levels and detection rates of blood Hg or cadmium (Cd) in a linear dose-responsive manner between ethnic African, Asian, Caucasian, and Hispanic children in the US (p ≤ 0.05). Increasing low-dose exposure to Hg or Cd may induce KD or contribute to its later development in susceptible children. However, our preliminary results require further replication in other ethnic populations, in addition to more in-depth examination of metal exposure and toxicokinetics. PMID:26742052

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

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Hegazi, Mohamed Osama; Ahmed, Sherif

2012-01-01

Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual) manifestations of Graves' disease (GD), that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD. PMID:22132347

The biophysical properties of the aorta are altered following Kawasaki disease.

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Vaujois, Laurence; Dallaire, Frédéric; Maurice, Roch L; Fournier, Anne; Houde, Christine; Thérien, Johanne; Cartwright, Daniel; Dahdah, Nagib

2013-12-01

The long-term sequelae of Kawasaki disease (KD) are based on the coronary complications. Because KD causes generalized vasculitis, with documented aneurysms in the femoral, iliac, renal, axillary, and brachial arteries, the aim of this study was to assess the biophysical properties of the aorta (BPA) after KD. The BPA are biometric measurements representing vascular structural and dynamic changes in response to cardiac work. Anthropometric and echocardiographic measurements of the aorta in a series of patients with KD were compared with those of healthy subjects. The BPA were calculated noninvasively by extrapolating previously validated equations that were conceived for invasive measurements. Because BPA vary with body habitus, control subjects were used to normalize BPA parameters for height to compute BPA Z-score equations. Between June 2007 and February 2010, BPA were recorded in 57 patients with KD >1 year after the onset of the disease, 45 without and 12 with coronary artery sequelae. The mean intervals between the acute onset of KD and enrollment were 10.0 ± 5.0 and 5.8 ± 4.5 years for patients with and without coronary artery sequelae, respectively (P = .008). Patients with KD with coronary artery sequelae had significantly altered Z scores of aortic diameter modulation, Peterson's elastic modulus, and β stiffness index (P = .001-.016). Patients with KD without coronary artery sequelae also exhibited altered elasticity, stiffness, and pulse-wave velocity (P = .001-.026). Altered BPA after KD are detectible despite apparent resolution of acute vasculitis. Future directions toward determining multilevel and multilayer vascular impact, including vascular autonomous homeostasis, require thorough investigation. Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.

LABORATORY BIOMARKERS TO FACILITATE DIFFERENTIAL DIAGNOSIS BETWEEN MEASLES AND KAWASAKI DISEASE IN A PEDIATRIC EMERGENCY ROOM: A RETROSPECTIVE STUDY

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Danilo Buonsenso

2018-05-01

Full Text Available This retrospective study was conducted to analyze clinical and laboratoristic parameters to individuate specific differences and facilitate differential diagnosis between Measles and Kawasaki Disease (KD at first evaluation in a emergency room. We found similar clinical features as duration of fever and number of KD criteria (p >0.5 but significant differences in white blood cell count, neutrophils, CRP and LDH levels (p < 0.001. LDH value ≥ 800 mg/dl had sensibility of 89% and specificity of 90% for Measles while CRP ≥ 3 mg/dl had sensibility 89% and specificity of 85% for KD. The combined use of CRP, LDH and AST showed accuracy of 86.67%.

Hemodynamic Based Coronary Artery Aneurysm Thrombosis Risk Stratification in Kawasaki Disease Patients

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Grande Gutierrez, Noelia; Mathew, M.; McCrindle, B.; Kahn, A.; Burns, J.; Marsden, A.

2017-11-01

Coronary artery aneurysms (CAA) as a result of Kawasaki Disease (KD) put patients at risk for thrombosis and myocardial infarction. Current AHA guidelines recommend CAA diameter >8 mm or Z-score >10 as the criterion for initiating systemic anticoagulation. Our hypothesis is that hemodynamic data derived from computational blood flow simulations is a better predictor of thrombosis than aneurysm diameter alone. Patient-specific coronary models were constructed from CMRI for a cohort of 10 KD patients (5 confirmed thrombosis cases) and simulations with fluid structure interaction were performed using the stabilized finite element Navier-Stokes solver available in SimVascular. We used a closed-loop lumped parameter network (LPN) to model the heart and vascular boundary conditions coupled numerically to the flow solver. An automated parameter estimation method was used to match LPN values to clinical data for each patient. Hemodynamic data analysis resulted in low correlation between Wall Shear Stress (WSS)/ Particle Residence Time (PRT) and CAA diameter but demonstrates the positive correlation between hemodynamics and adverse patient outcomes. Our results suggest that quantifying WSS and PRT should enable identification of regions at higher risk of thrombosis. We propose a quantitative method to non-invasively assess the abnormal flow in CAA following KD that could potentially improve clinical decision-making regarding anticoagulation therapy.

IL-10 Promoter Genetic Polymorphisms and Risk of Kawasaki Disease in Taiwan

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Kai-Sheng Hsieh

2011-01-01

Full Text Available Kawasaki disease (KD is the most common cause of pediatric acquired heart disease. KD patients have spontaneously high plasma/serum levels of IL-10 during the acute phase. Therefore, two independent studies were carried out to investigate the association between genetic variants in IL-10 promoter (−1082, −819, and −592 and risk of KD. A total of 134 trios were included for the family-based association study. A significantly preferential transmission of the C allele at loci −819 T > C and −592 A > C for KD cases was observed (Ppermutation = 0.029 and Ppermutation = 0.034, respectively. There was a significant increase in the transmission of haplotype CC (p = 0.016 at the above two loci (OR, 1.632; 95% CI, 1.090–2.443; Ppermutation = 0.019. We also carried out a follow-up case-control study that included 146 KD cases and 315 unrelated healthy children. {The haplotype CC (−819, −592 showed an increased risk of KD (but statistically non-significant; OR, 1.332; 95% CI, 0.987–1.797; p = 0.061. In diplotype analysis, a trend was found between number of CC haplotype and risk of KD (but non-significant, p = 0.061. In conclusion, CC genotype and CC/CC diplotype at IL-10-819T > C and −592A > C were significantly associated with risk of KD in case-parent trio study, which were replicated partially in our follow-up case-control study.

Characteristics and Fate of Systemic Artery Aneurysm after Kawasaki Disease.

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Hoshino, Shinsuke; Tsuda, Etsuko; Yamada, Osamu

2015-07-01

To determine the long-term outcome of systemic artery aneurysms (SAAs) after Kawasaki disease (KD). We investigated the characteristics and the fate of SAAs in 20 patients using medical records and angiograms. The age of onset of KD ranged from 1 month to 20 months. The interval from the onset of KD to the latest angiogram ranged from 16 months to 24 years. The regression rate of peripheral artery aneurysm and the frequency of stenotic lesions were analyzed by the Kaplan-Meier method in 11 patients who had undergone initial angiography within 4 months. The mean duration of fever was 24 ± 12 days. All 20 patients had at least 1 symmetric pair of aneurysms in bilateral peripheral arteries, and 16 patients had multiple SAAs. The distributions of SAAs was as follows: brachial artery, 30; common iliac artery, 20; internal iliac artery, 21; abdominal aortic aneurysm, 7; and others, 29. The frequencies of regression of SAA and of the occurrence of stenotic lesions at 20 years after the onset of KD were 51% and 25%, respectively (n = 42). The diameter of all SAAs in the acute phase leading to stenotic lesions in the late period was >10 mm. SAAs occurred symmetrically and were multiple in younger infants and those with severe acute vasculitis. The fate of SAAs resembles that of coronary artery aneurysms, and depends on the diameter during the acute phase. Larger SAAs can lead to stenotic lesions in the late period. Copyright © 2015 Elsevier Inc. All rights reserved.

Cardiac Complications in 38 Cases of Kawasaki Disease with Coronary Artery Aneurysm Diagnosed by Echocardiography.

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Wei, Ya Juan; Zhao, Xiao Lan; Liu, Bao Min; Niu, Hua; Li, Qian

2016-05-01

The long-term prognosis of patients with Kawasaki disease (KD) complicated by coronary artery aneurysm (CAA) is unclear. The aim of this study was to evaluate the complications of KD with CAAs. We retrospectively analyzed the clinical data and complications of 38 KD patients with CAAs who were treated and underwent regular follow-up with echocardiography between January 1989 and May 2013. During a period of 29 days to 19 years after disease onset, complications seen included coronary stenosis and occlusion (six patients), thrombosis (17 patients), myocardial infarction (six patients), and calcification of CAAs (seven patients). Rupture of giant CAAs occurred in two patients and caused sudden death in one of these patients at 29 days and in the other patient at 5 months after disease onset. A total of seven deaths occurred, with five deaths caused by myocardial infarction. Three of these had undiagnosed incomplete KD or had not received regular treatment, while two experienced sudden death after several asymptomatic myocardial infarctions. Cardiac complications of KD with CAAs include thrombosis, coronary stenosis, myocardial infarction, sudden death, and calcification. Although rare, rupture of giant CAAs is fatal and might occur earlier after the onset of disease. Mortality occurred primarily in the earlier cases when anticoagulant therapy was insufficient and in patients who did not receive regular treatment. Echocardiography can provide reliable information for assessing the progression and prognosis of this condition. © 2015, Wiley Periodicals, Inc.

Lack of association between miR-218 rs11134527 A>G and Kawasaki disease susceptibility.

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Pi, Lei; Fu, Lanyan; Xu, Yufen; Che, Di; Deng, Qiulian; Huang, Xijing; Li, Meiai; Zhang, Li; Huang, Ping; Gu, Xiaoqiong

2018-05-01

Abstract Kawasaki disease (KD) is a type of disease that includes the development of a fever that lasts at least five days and involves the clinical manifestation of multicellular vasculitis. KD has become one of the most common pediatric cardiovascular diseases. Previous studies have reported that miR-218 rs11134527 A>G is associated with susceptibility to various cancer risks. However, there is a lack of evidence regarding the relationship between this polymorphism and KD risk. This study explored the correlation between the miR-218 rs11134527 A>G polymorphism and the risk of KD. We recruited 532 patients with KD and 623 controls to genotype the miR-218 rs11134527 A>G polymorphism with a TaqMan allelic discrimination assay. Our results illustrated that the miR-218 rs11134527 A>G polymorphism was not associated with KD risk. In an analysis stratified by age, sex, and coronary artery lesions, we found only that the risk of KD was significantly decreased for children older than 5 years (GG vs. AA/AG: adjusted OR=0.26, 95% CI=0.07-0.94, P =0.041). This study demonstrated that the miR-218 rs1113452 A>G polymorphism may have an age-related relationship with KD susceptibility that has not previously been revealed. ©2018 The Author(s).

Caregiver burden in atypical dementias: comparing frontotemporal dementia, Creutzfeldt-Jakob disease, and Alzheimer's disease.

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Uflacker, Alice; Edmondson, Mary C; Onyike, Chiadi U; Appleby, Brian S

2016-02-01

Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt-Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies. 76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores. Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD. This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

Case Reports of Cat Scratch Disease with Typical and Atypical Clinical Manifestations: A Literature Review

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Gulshan Umbreen

2017-04-01

Full Text Available Cat scratch disease (CSD is the most well-known zoonotic disease spread by domestic animals like cats. Cats are the source of Bartonella henselae. Most patients more than ninety percent 3-12 days after a scratch from a cat, undoubtedly a little cat with insects present with one or more erythematous injuries at the site of inoculation, the sore is typically a crusted papule or, once in a while, a pustule. More than half of cases in one study show that the systemic indications went with the lymphadenopathy. These may incorporate fever, discomfort, migraine and anorexia and frequently happen in immunocompromised patients. Atypically clinical manifestations happen are altered mental status, perplexity, prolonged fever, respiratory protestations (atypical pneumonitis, Joint pain, synovitis, Back agony is uncommon. The hypothesis of the study to find out that cat scratch disease cause typical and atypical clinical manifestation. Study was conducted July 2015 to September 2015. The methodology sections of a review article are listed all of the databases and citation indexes that were searched such as Web of Science and PubMed and any individual journals that were searched. Various case reports were mentioned in the study. Case reports of cat scratch diseases with typical and atypical clinical manifestation included in the study. The objective of review of these reporting cases is to make physicians aware about cat scratch diseases and also need to create awareness about cat scratch disease in pet owner. Although it is self-limiting needs to report to health authorities. There are few cases reported in which mostly cases reported in twain, japan, Brazil, Texas, United States, Dhaka, Spain with typical and atypical clinical manifestation

Use of Lagrangian transport models and Sterilized High Volume Sampling to pinpoint the source region of Kawasaki disease and determine the etiologic agent

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Curcoll Masanes, Roger; Rodó, Xavier; Anton, Jordi; Ballester, Joan; Jornet, Albert; Nofuentes, Manel; Sanchez-Manubens, Judith; Morguí, Josep-Anton

2015-04-01

Kawasaki disease (KD) is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 years. A former study [Rodó et al. 2011] demonstrated that certain patterns of winds in the troposphere above the earth's surface flowing from Asia were associated with the times of the annual peak in KD cases and with days having anomalously high numbers of KD patients. In a later study [Rodó et al. 2014], we used residence times from an Air Transport Model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. In order to cope with stationarity of synoptic situations, only trajectories for the winter months, when there is the maximum in KD cases, were considered. Trajectories traced back in time 10 days for each dataset and location were generated using the flexible particle Lagrangian dispersion model (FLEXPART Version 8.23 [Stohl et al. 2005]) run in backward mode. The particles modeled were air tracers, with 10,000 particles used on each model run. The model output used was residence time, with an output grid of 0.5° latitude × longitude and a time resolution of 3 h. The data input used for the FLEXPART model was gridded atmospheric wind velocity from the European Center for Medium-Range Weather Forecasts Re-Analysis (ERA-Interim at 1°). Aggregates of winter period back-trajectories were calculated for three different regions of Japan. A common source of wind air masses was located for periods with High Kawasaki disease. Knowing the trajectories of winds from the air transport models, a sampling methodology was developed in order to capture the possible etiological agent or other tracers that could have been released together. This methodology is based on the sterilized filtering of high volumes of the transported air at medium tropospheric levels by aircraft sampling and a later analyze these filters with adequate techniques. High purity

Adolescent Kawasaki disease: usefulness of 64-slice CT coronary angiography for follow-up investigation

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Carbone, Iacopo; Cannata, David; Algeri, Emanuela; Galea, Nicola; Napoli, Alessandro; Catalano, Carlo; Passariello, Roberto; Francone, Marco [Sapienza University of Rome, Department of Radiological, Onchological and Anatomopathological Sciences, Policlinico Umberto I, Rome (Italy); De Zorzi, Andrea [Bambino Gesu Hospital, Cardiology Division, Rome (Italy); Bosco, Giovanna; D' Agostino, Rita [Sapienza University of Rome, Unit of Paediatric Cardiology, Policlinico Umberto I, Rome (Italy); Menezes, Leon [University College of London, Institute of Nuclear Medicine, London (United Kingdom)

2011-09-15

Kawasaki disease (KD) is a systemic vasculitis that mainly affects coronary arteries in children, and requires regular follow-up from the time of diagnosis. To evaluate the feasibility of 64-slice CT angiography (CTA) for follow-up of patients with KD using previously performed invasive catheter coronary angiography (CCA) as reference standard. The study group comprised 12 patients (age 17.6 {+-} 2.9 years, mean{+-}SD) with a diagnosis of KD and a previously performed CCA (interval, 32.6 {+-} 13.5 months) who underwent 64-slice cardiac CTA. The quality of the images for establishing the presence of coronary abnormalities was determined by two observers. The CTA findings were compared with those from the prior CCA. Adequate image quality was obtained in all patients. Mean effective dose for CTA was 6.56 {+-} 0.95 mSv. CTA allowed accurate identification, characterization and measurement of all coronary aneurysms (n = 32), stenoses (n = 3) and occlusions (n = 9) previously demonstrated by CCA. One patient with disease progression went on to have percutaneous coronary intervention. Coronary lesions were reliably evaluated by 64-slice CTA in the follow-up of compliant patients with KD, reducing the need for repeated diagnostic invasive CCA. Hence, in an adequately selected patient population, the role of CCA could be limited almost only to therapeutic procedures. (orig.)

Classification of Rhinoentomophthoromycosis into Atypical, Early, Intermediate, and Late Disease: A Proposal.

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Christian G Blumentrath

Full Text Available Rhinoentomophthoromycosis, or rhino-facial conidiobolomycosis, is a rare, grossly disfiguring disease due to an infection with entomophthoralean fungi. We report a case of rhinoentomophthoromycosis from Gabon and suggest a staging system, which provides information on the prognosis and duration of antifungal therapy.We present a case of rhinoentomophthoromycosis including the histopathology, mycology, and course of disease. For the suggested staging system, all cases on confirmed rhinoentomophthoromycosis published in the literature without language restriction were eligible. Exclusion criteria were missing data on (i duration of disease before correct diagnosis, (ii outcome, and (iii confirmation of entomophthoralean fungus infection by histopathology and/or mycology. We classified cases into atypical (orbital cellulitis, severe pain, fever, dissemination, early, intermediate, and late disease based on the duration of symptoms before diagnosis. The outcome was evaluated for each stage of disease.The literature search of the Medpilot database was conducted on January 13, 2014, (updated on January 18, 2015. The search yielded 8,333 results including 198 cases from 117 papers; of these, 145 met our inclusion criteria and were included in the final analysis. Median duration of treatment was 4, 3, 4, and 5 months in atypical, early, intermediate, and late disease, respectively. Cure rates were clearly associated with stage of disease and were 57%, 100%, 82%, and 43% in atypical, early, intermediate, and late disease, respectively.We suggest a clinical staging system that underlines the benefit of early case detection and may guide the duration of antifungal treatment. The scientific value of this classification is its capacity to structure and harmonize the clinical and research approach towards rhinoentomophthoromycosis.

Diseases of the gastrointestinal tract in individuals diagnosed as children with atypical autism

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Mouridsen, Svend Erik Birkebæk; Isager, Torben; Rich, Bente

2013-01-01

The purpose of this study is to compare the prevalence and types of diseases (International Classification of Mental and Behavioural Disorders, 10th Edition codes K20-K93) relating to the gastrointestinal tract in a clinical sample of 89 individuals diagnosed as children with atypical autism...... with atypical autism, a total of 22 (24.7%) were registered with at least one diagnosis of any disease of the gastrointestinal tract, against 47 of 258 (18.2%) in the comparison group (p = 0.22; odds ratio = 1.5; 95% confidence interval = 0.8-2.6). Without reaching statistical significance, the rate of diseases...... of the gastrointestinal tract was particularly high (odds ratio = 1.2) in those with intelligence quotient autism had about the same frequency of gastric, intestinal and hepatic diseases as had controls....

Kawasaki disease: a rare pediatric pathology in Mexico. Twenty cases report from the Hospital Infantil del Estado de Sonora.

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Sotelo, Norberto; González, Luis Antonio

2007-01-01

Kawasaki disease (KD) is an etiological illness that is relatively unknown and scarcely identified in Mexico; it affects children mainly aged 1-4 years, evolves with fever, vasculitis in diverse organs, and in the heart the disease mainly affects the coronary arteries. Our aim was to inform the clinical findings and evolution of 20 patients diagnosed with KD. We reviewed the patient clinical files retrospectively and descriptively to obtain information with regard to age, sex, clinical signs, laboratory and consultory results, echocardiography findings, complications, evolution during hospitalization, followup, and out-patient ambulatory consultations. Eighteen patients were male, two were female, six developed coronary damage, two aortic mitral-valve insufficiency, one pericardial shedding, and one, myocarditis. All patients received gamma globulin treatment with aspirin, and 16 were controlled during 6-8 months after the acute medical profile. The opportune clinical diagnostic it is fundamental to establish an early treatment with gammmaglobuline to avoid injuries in the arterial coronary level. This injury may cause eventualy ischemia or myocardial infarct

Transforming growth factor beta receptor II polymorphisms are associated with Kawasaki disease

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Yu Mi Choi

2012-01-01

Full Text Available Purpose : Transforming growth factor beta receptor 2 (TGFBR2 is a tumor suppressor gene that plays a role in the differentiation of striated cells and remodeling of coronary arteries. Single nucleotide polymorphisms (SNPs of this gene are associated with Marfan syndrome and sudden death in patients with coronary artery disease. Cardiovascular remodeling and T cell activation of TGFBR2 gene suggest that the TGFBR2 gene SNPs are related to the pathogenesis of Kawasaki disease (KD and coronary artery lesion (CAL. Methods : The subjects were 105 patients with KD and 500 healthy adults as controls. Mean age of KD group was 32 months age and 26.6% of those had CAL. We selected TGFBR2 gene SNPs from serum and performed direct sequencing. Results : The sequences of the eleven SNPs in the TGFBR2 gene were compared between the KD group and controls. Three SNPs (rs1495592, rs6550004, rs795430 were associated with development of KD (P=0.019, P=0.026, P=0.016, respectively. One SNP (rs1495592 was associated with CAL in KD group (P=0.022. Conclusion : Eleven SNPs in TGFBR2 gene were identified at that time the genome wide association. But, with the change of the data base, only six SNPs remained associated with the TGFBR2 gene. One of the six SNPs (rs6550004 was associated with development of KD. One SNP associated with CAL (rs1495592 was disassociated from the TGFBR2 gene. The other five SNPs were not functionally identified, but these SNPs are notable because the data base is changing. Further studies involving larger group of patients with KD are needed.

Disease: H01718 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. If there is no r... H01718 Kawasaki disease; Mucocutaneous lymph node syndrome Kawasaki disease (KD) is an acute systemic vascu...litis of childhood that does not have a known cause or aetiology. KD is a self-limi

Detection Rate and Clinical Impact of Respiratory Viruses in Children with Kawasaki Disease

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Ja Hye Kim

2012-12-01

Full Text Available &lt;B&gt;Purpose:&lt;/B&gt; The purpose of this prospective case-control study was to survey the detection rate of respiratory viruses in children with Kawasaki disease (KD by using multiplex reverse transcriptasepolymerase chain reaction (RT-PCR, and to investigate the clinical implications of the prevalence of respiratory viruses during the acute phase of KD. &lt;B&gt;Methods:&lt;/B&gt; RT-PCR assays were carried out to screen for the presence of respiratory syncytial virus A and B, adenovirus, rhinovirus, parainfluenza viruses 1 to 4, influenza virus A and B, metapneumovirus, bocavirus, coronavirus OC43/229E and NL63, and enterovirus in nasopharyngeal secretions of 55 KD patients and 78 control subjects. &lt;B&gt;Results:&lt;/B&gt; Virus detection rates in KD patients and control subjects were 32.7% and 30.8%, respectively (P=0.811. However, there was no significant association between the presence of any of the 15 viruses and the incidence of KD. Comparisons between the 18 patients with positive RT-PCR results and the other 37 KD patients revealed no significant differences in terms of clinical findings (including the prevalence of incomplete presentation of the disease and coronary artery diameter. &lt;B&gt;Conclusion:&lt;/B&gt; A positive RT-PCR for currently epidemic respiratory viruses should not be used as an evidence against the diagnosis of KD. These viruses were not associated with the incomplete presentation of KD and coronary artery dilatation.

Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease

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Yoichi Kawamura

2017-01-01

Full Text Available Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD. We investigated the potential significance of urinary lactate dehydrogenase (U-LDH activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5 levels were compared among 120 patients with KD, 18 patients with viral infection (VI, and 43 patients with upper urinary tract infection (UTI and additionally compared between intravenous immunoglobulin (IVIG responders (n=89 and nonresponders (n=31 with KD. Total U-LDH activity was higher in KD (35.4±4.8 IU/L, P<0.05 and UTI patients (66.0±8.0 IU/L, P<0.01 than in VI patients (17.0±6.2 IU/L. In the isozyme pattern analysis, KD patients had high levels of U-LDH1 and U-LDH2, while UTI patients had high levels of U-LDH3, U-LDH4, and U-LDH5. Furthermore, IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1±4.7 IU/L, P<0.05, especially U-LDH1 and U-LDH2 (P<0.05, than IVIG responders (32.0±2.8 IU/L. KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients.

Pro-inflammatory cytokine single nucleotide polymorphisms in Kawasaki disease.

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Assari, Raheleh; Aghighi, Yahya; Ziaee, Vahid; Sadr, Maryam; Rahmani, Farzaneh; Rezaei, Arezou; Sadr, Zeinab; Moradinejad, Mohammad Hassan; Raeeskarami, Seyed Reza; Rezaei, Nima

2016-07-25

Kawasaki disease (KD) is a systemic vasculitis of children associated with cardiovascular sequelae. Proinflammatory cytokines play a major role in KD pathogenesis. However, their role is both influenced and modified by regulatory T-cells. IL-1 gene cluster, IL-6 and TNF-α polymorphisms have shown significant associations with some vasculitides. Herein we investigated their role in KD. Fifty-five patients with KD who were randomly selected from referrals to the main pediatric hospital were enrolled in this case-control study. Single nucleotide polymorphisms (SNPs) of the following genes were assessed in patients and 140 healthy subjects as control group: IL-1α at -889 (rs1800587), IL-1β at -511 (rs16944), IL-1β at +3962 (rs1143634), IL-1R at Pst-I 1970 (rs2234650), IL-1RN/A at Mspa-I 11100 (rs315952), TNF-α at -308 (rs1800629), TNF-α at -238, IL-6 at -174 (rs1800795) and IL-6 at +565. Twenty-one percent of the control group had A allele at TNF-α -238 while only 8% of KD patients had A allele at this position (P = 0.003, OR [95%CI] = 0.32 [0.14-0.71]). Consistently, TNF-α genotype GG at -238 had significant association with KD (OR [95% CI] = 4.31 [1.79-10.73]). Most controls carried the CG genotype at IL-6 -174 (n = 93 [66.9%]) while GG genotype was the most common genotype (n = 27 [49%]) among patients. Carriers of the GG haplotype at TNF-α (-308, -238) were significantly more prevalent among the KD group. No association was found between IL-1 gene cluster, allelic or haplotypic variants and KD. TNF-α GG genotype at -238 and GG haplotype at positions -308 and -238 were associated with KD in an Iranian population. © 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

Serum albumin level predicts initial intravenous immunoglobulin treatment failure in Kawasaki disease.

Science.gov (United States)

Kuo, Ho-Chang; Liang, Chi-Di; Wang, Chih-Lu; Yu, Hong-Ren; Hwang, Kao-Pin; Yang, Kuender D

2010-10-01

Kawasaki disease (KD) is a systemic vasculitis primarily affecting children who are initial IVIG treatment. This study was conducted to investigate the risk factors for initial IVIG treatment failure in KD. Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients were divided into IVIG-responsive and IVIG-resistant groups. Initial laboratory data before IVIG treatment were collected for analysis. A total of 131 patients were enrolled during the study period. At 48 h after completion of initial IVIG treatment, 20 patients (15.3%) had an elevated body temperature. Univariate analysis showed that patients who had initial findings of high neutrophil count, abnormal liver function, low serum albumin level (≤2.9 g/dL) and pericardial effusion were at risk for IVIG treatment failure. Multivariate analysis with a logistic regression procedure showed that serum albumin level was considered the independent predicting factor of IVIG resistance in patients with KD (p = 0.006, OR = 40, 95% CI: 52.8-562). There was no significant correlation between age, gender, fever duration before IVIG treatment, haemoglobin level, total leucocyte and platelet counts, C-reactive protein level, or sterile pyuria and initial IVIG treatment failure. The specificity and sensitivity for prediction of IVIG treatment failure in this study were 96% and 34%, respectively. Pre-IVIG treatment serum albumin levels are a useful predictor of IVIG resistance in patients with KD. © 2010 The Author(s)/Journal Compilation © 2010 Foundation Acta Paediatrica.

Pregnancy in women with a history of Kawasaki disease: management and outcomes.

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Gordon, C T; Jimenez-Fernandez, S; Daniels, L B; Kahn, A M; Tarsa, M; Matsubara, T; Shimizu, C; Burns, J C; Gordon, J B

2014-10-01

To characterise the obstetrical management and outcomes in a series of women with a history of Kawasaki disease (KD) in childhood. Retrospective case series. Tertiary healthcare setting in the USA. Women with a history of KD in childhood. Women completed a detailed health questionnaire and participated in research imaging studies as part of the San Diego Adult KD Collaborative Study. Obstetrical management, complications during pregnancy and delivery, and infant outcomes. Ten women with a history of KD in childhood carried a total of 21 pregnancies to term. There were no cardiovascular complications during labour and delivery despite important cardiovascular abnormalities in four of the ten subjects. Pregnancy was complicated by pre-eclampsia and the post-partum course was complicated by haemorrhage in one subject each. Two of the 21 progeny subsequently developed KD. Women with important cardiovascular sequelae from KD in childhood should be managed by a team that includes both a maternal-fetal medicine specialist and a cardiologist. Pre-pregnancy counselling should include delineation of the woman's current functional and structural cardiovascular status and appropriate adjustment of medications, but excellent outcomes are possible with appropriate care. Review of the English and Japanese literature on KD and pregnancy revealed the occurrence of myocardial infarction during pregnancy in women with missed KD and aneurysms that were not diagnosed until their acute event. Our study highlights the need for counselling with regard to the increased genetic risk of KD in offspring born to these mothers. © 2014 Royal College of Obstetricians and Gynaecologists.

Atypical Cutaneous Manifestations in Syphilis.

Science.gov (United States)

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. Copyright © 2015 AEDV. Published by Elsevier España, S.L.U. All rights reserved.

A Novel Truncated Form of Serum Amyloid A in Kawasaki Disease.

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John C Whitin

Full Text Available Kawasaki disease (KD is an acute vasculitis in children that can cause coronary artery abnormalities. Its diagnosis is challenging, and many cytokines, chemokines, acute phase reactants, and growth factors have failed evaluation as specific biomarkers to distinguish KD from other febrile illnesses. We performed protein profiling, comparing plasma from children with KD with febrile control (FC subjects to determine if there were specific proteins or peptides that could distinguish the two clinical states.Plasma from three independent cohorts from the blood of 68 KD and 61 FC subjects was fractionated by anion exchange chromatography, followed by surface-enhanced laser desorption ionization (SELDI mass spectrometry of the fractions. The mass spectra of KD and FC plasma samples were analyzed for peaks that were statistically significantly different.A mass spectrometry peak with a mass of 7,860 Da had high intensity in acute KD subjects compared to subacute KD (p = 0.0003 and FC (p = 7.9 x 10-10 subjects. We identified this peak as a novel truncated form of serum amyloid A with N-terminal at Lys-34 of the circulating form and validated its identity using a hybrid mass spectrum immunoassay technique. The truncated form of serum amyloid A was present in plasma of KD subjects when blood was collected in tubes containing protease inhibitors. This peak disappeared when the patients were examined after their symptoms resolved. Intensities of this peptide did not correlate with KD-associated laboratory values or with other mass spectrum peaks from the plasma of these KD subjects.Using SELDI mass spectrometry, we have discovered a novel truncated form of serum amyloid A that is elevated in the plasma of KD when compared with FC subjects. Future studies will evaluate its relevance as a diagnostic biomarker and its potential role in the pathophysiology of KD.

Genetics Home Reference: atypical hemolytic-uremic syndrome

Science.gov (United States)

... Kidney Diseases: Kidney Failure: Choosing a Treatment That's Right for You Educational Resources (6 links) Disease InfoSearch: Hemolytic uremic syndrome, atypical MalaCards: genetic atypical hemolytic-uremic syndrome Merck Manual Consumer Version: Overview of Anemia Merck Manual Consumer Version: ...

Increased Risk of Atopic Dermatitis in Preschool Children with Kawasaki Disease: A Population-Based Study in Taiwan

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Peng Yeong Woon

2013-01-01

Full Text Available Kawasaki disease (KD is an acute febrile systemic vasculitis and has been reported to be associated with allergic disease. The risk of atopic dermatitis (AD in preschool children with KD has not been investigated. The study was to determine the longitudinal risk of the development of AD in preschool children with KD. A nationwide 5-year population-based study was performed using data from the National Health Insurance Database in Taiwan between 1999 and 2003. The risk factors for AD were compared between the 2 study groups during the follow-up period using the Cox proportional hazards model. In addition, plasma interleukin (IL-5 levels were analyzed in normal subjects and KD patients. Among the 1440 subjects included, 21.6% developed AD during the 5-year follow-up period, of which 30.3% and 18.7% belonged to the study cohort and the comparison group, respectively. Children with KD were 1.25 times more likely to have AD than those in controls (P=0.04. Levels of IL-5 and IgE were significantly higher in KD patients. Children with KD had a higher risk of developing AD during the 5-year follow-up period than the control group. Increased IL-5 and IgE levels may be key factors contributing to the risk of AD.

Value of amino-terminal pro B-natriuretic peptide in diagnosing Kawasaki disease.

Science.gov (United States)

McNeal-Davidson, Ariane; Fournier, Anne; Spigelblatt, Linda; Saint-Cyr, Claire; Mir, Thomas S; Nir, Amiram; Dallaire, Frédéric; Cousineau, Jocelyne; Delvin, Edgard; Dahdah, Nagib

2012-10-01

The aim of the present study was to investigate the diagnostic value of the N-terminal B-type natriuretic peptide (NT-proBNP) in acute Kawasaki disease (KD) given that the clinical criteria and the current basic laboratory tests lack the necessary specificity for accurate diagnosis. Basic biological tests and serum NT-proBNP levels obtained from acute KD patients were compared to that of febrile controls. NT-proBNP was considered abnormal based on the following definitions: above a cut-off determined on receiver operator characteristic (ROC) analysis, above the upper limit for age, or above 2 SD calculated from healthy children. Analyses were also performed for KD cases with complete or incomplete criteria combined and separately. There were 81 patients and 49 controls aged 3.60 ± 2.77 versus 4.25 ± 3.88 years (P= 0.69). ROC analysis yielded significant area under the curve for NT-proBNP. The sensitivity, specificity, positive and negative predictive values were 70.4-88.9%, 69.4-91.8%, 82.8-93.4%, and 65.2-79.1%. The odds ratios based on NT-proBNP definitions varied between 18.13 (95% confidence interval [CI]: 7.21-45.57), 20.82 (95%CI: 8.18-53.0), and 26.71 (95%CI: 8.64-82.57; P < 0.001). Results were reproducible for cases with complete or incomplete criteria separately. NT-proBNP is a reliable marker for the diagnosis of KD. Prospective clinical studies with emphasis on NT-proBNP in a diagnostic algorithm are needed. © 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.

Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein.

Science.gov (United States)

Wadsworth, Jonathan D F; Joiner, Susan; Linehan, Jacqueline M; Balkema-Buschmann, Anne; Spiropoulos, John; Simmons, Marion M; Griffiths, Peter C; Groschup, Martin H; Hope, James; Brandner, Sebastian; Asante, Emmanuel A; Collinge, John

2013-11-01

Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source of infection, we inoculated transgenic mice that overexpressed human prion protein with brain tissue from sheep with natural field cases of classical and atypical scrapie, sheep with experimental BSE, and cattle with BSE. We found that these mice were susceptible to BSE prions, but disease did not develop after prolonged postinoculation periods when mice were inoculated with classical or atypical scrapie prions. These data are consistent with the conclusion that prion disease is less likely to develop in humans after exposure to naturally occurring prions of sheep than after exposure to epizootic BSE prions of ruminants.

Atypical Primary Cutaneous Rosai Dorfman Disease: A Case Report.

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Kinio, Anna E; Sawchuk, Michael A; Pratt, Melanie

Rosai Dorfman disease (RDD) is a rare disorder that typically presents with bilateral cervical lymphadenopathy and follows a benign course. We present a case of late-onset atypical primary cutaneous RDD that is resistant to treatment modalities described in the literature. Case report. An 84-year-old woman presented with a 7-year history of cutaneous lesions histologically consistent with RDD. She later failed initial treatments of acitretin and thalidomide. Physicians must be aware of unusual presentations of RDD. Also, further treatment options must be explored for patients resistant to classical management of RDD.

Lifting of a sector block for YE-2 at Kawasaki.

CERN Multimedia

R. Loveless/U. of Wisconsin

2000-01-01

YE-2 is build from machined sector blocks. Trial assembly is carried out horizontally. This picture represents the lifting of a machined sector block destined to the trial assembly of a half disk YE-2 at Kawasaki (KHI) Kobe, Japan.

Atypicality of Atypical Antipsychotics

OpenAIRE

Farah, Andrew

2005-01-01

Objective: To review the current definition of atypicality, discuss the unique features of each atypical antipsychotic, and determine whether the available drugs in this class really meet the classical definition of atypicality.

Increased Kawasaki Disease Incidence Associated With Higher Precipitation and Lower Temperatures, Japan, 1991-2004.

Science.gov (United States)

Abrams, Joseph Y; Blase, Jennifer L; Belay, Ermias D; Uehara, Ritei; Maddox, Ryan A; Schonberger, Lawrence B; Nakamura, Yosikazu

2018-06-01

Kawasaki disease (KD) is an acute febrile vasculitis, which primarily affects children. The etiology of KD is unknown; while certain characteristics of the disease suggest an infectious origin, genetic or environmental factors may also be important. Seasonal patterns of KD incidence are well documented, but it is unclear whether these patterns are caused by changes in climate or by other unknown seasonal effects. The relationship between KD incidence and deviations from expected temperature and precipitation were analyzed using KD incidence data from Japanese nationwide epidemiologic surveys (1991-2004) and climate data from 136 weather stations of the Japan Meteorological Agency. Seven separate Poisson-distributed generalized linear regression models were run to examine the effects of temperature and precipitation on KD incidence in the same month as KD onset and the previous 1, 2, 3, 4, 5 and 6 months, controlling for geography as well as seasonal and long-term trends in KD incidence. KD incidence was negatively associated with temperature in the previous 2, 3, 4 and 5 months and positively associated with precipitation in the previous 1 and 2 months. The model that best predicted variations in KD incidence used climate data from the previous 2 months. An increase in total monthly precipitation by 100 mm was associated with increased KD incidence (rate ratio [RR] 1.012, 95% confidence interval [CI]: 1.005-1.019), and an increase of monthly mean temperature by 1°C was associated with decreased KD incidence (RR 0.984, 95% CI: 0.978-0.990). KD incidence was significantly affected by temperature and precipitation in previous months independent of other unknown seasonal factors. Climate data from the previous 2 months best predicted the variations in KD incidence. Although fairly minor, the effect of temperature and precipitation independent of season may provide additional clues to the etiology of KD.

Urinary Colorimetric Sensor Array and Algorithm to Distinguish Kawasaki Disease from Other Febrile Illnesses.

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Zhen Li

Full Text Available Kawasaki disease (KD is an acute pediatric vasculitis of infants and young children with unknown etiology and no specific laboratory-based test to identify. A specific molecular diagnostic test is urgently needed to support the clinical decision of proper medical intervention, preventing subsequent complications of coronary artery aneurysms. We used a simple and low-cost colorimetric sensor array to address the lack of a specific diagnostic test to differentiate KD from febrile control (FC patients with similar rash/fever illnesses.Demographic and clinical data were prospectively collected for subjects with KD and FCs under standard protocol. After screening using a genetic algorithm, eleven compounds including metalloporphyrins, pH indicators, redox indicators and solvatochromic dye categories, were selected from our chromatic compound library (n = 190 to construct a colorimetric sensor array for diagnosing KD. Quantitative color difference analysis led to a decision-tree-based KD diagnostic algorithm.This KD sensing array allowed the identification of 94% of KD subjects (receiver operating characteristic [ROC] area under the curve [AUC] 0.981 in the training set (33 KD, 33 FC and 94% of KD subjects (ROC AUC: 0.873 in the testing set (16 KD, 17 FC. Color difference maps reconstructed from the digital images of the sensing compounds demonstrated distinctive patterns differentiating KD from FC patients.The colorimetric sensor array, composed of common used chemical compounds, is an easily accessible, low-cost method to realize the discrimination of subjects with KD from other febrile illness.

Kawasaki dynamics with two types of particles : critical droplets

NARCIS (Netherlands)

Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

2012-01-01

This is the third in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large finite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

Kawasaki dynamics with two types of particles : critical droplets

NARCIS (Netherlands)

Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

2012-01-01

This is the third in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large ¿nite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

Kawasaki Disease – A Review of Treatment and Outcomes in an Irish Paediatric Cohort 2010-14.

LENUS (Irish Health Repository)

Flinn, AM

2018-02-01

Diagnosis of Kawasaki Disease (KD) can be challenging due to lack of a diagnostic test, and some children present with ‘incomplete’ KD when not all diagnostic criteria are met. Treatment with intravenous immunoglobulin (IVIG) and aspirin reduces the risk of coronary artery complications. There is sub-group of patients who are resistant to IVIG\\/aspirin therapy and are at increased risk of complications. Recent evidence suggests that additional treatment of this high-risk group with corticosteroids is beneficial in reducing this risk. We examine the treatment and coronary artery outcomes, by retrospective review of medical records, of a cohort of 32 paediatric patients with KD admitted to a single Irish tertiary centre from January 2010-December 2014. Twenty-eight percent of patients (9\\/32) had an incomplete diagnosis of KD; these patients received IVIG later compared to those with a complete KD diagnosis. 15\\/32 (47%) had abnormal echocardiogram findings in the acute phase, 8\\/32 (25%) had echocardiogram abnormalities at 6-week follow-up, and 4\\/32 (12.5%) had persisting abnormalities. This study highlights the potential for adverse outcome in KD, the difficulty in diagnosis in ‘incomplete’ cases, and the need to identify children at higher risk for adverse outcome where adjunctive therapies would be most beneficial.

Epidemiologic study of Kawasaki disease at a single hospital in Daejeon, Korea (1987 through 2000).

Science.gov (United States)

Lee, Kyung-Yil; Han, Ji-Whan; Lee, Hyung-Shin; Hong, Ja-Hyun; Hahn, Seung-Hoon; Lee, Joon-Sung; Whang, Kyung-Tai

2004-01-01

We evaluated the epidemiology and a range of clinical characteristics in children with Kawasaki disease (KD) in one area of South Korea. We retrospectively analyzed 506 medical records of children with KD, who were admitted at Daejeon St. Mary's Hospital from January 1987 through December 2000. The mean annual frequency was 36.1 +/- 11.1 cases per year. There were 55 cases (10.9%) in 1993, 50 cases (9.9%) in 1994 and 47 cases (9.3%) in 2000. There was a slightly higher occurrence in summer with no significant difference in seasonal frequency. Age distribution ranged from 2 months to 13 years of age (mean, 2.4 +/- 1.7 years) and 485 children (95.8%) were 500 mg/day for 4 to 5 days, 231 cases, 45.7%) and one dose IVIG (2.0 g/kg, 210 cases, 41.5%) were used. Between 1996 and 2000, 143 cases were treated with only one dose IVIG, and 21 cases (14.7%) showed coronary artery lesions (CAL). Among the 143 cases 22 cases (15.4%) were retreated with IVIG and/or steroid pulse therapy. The incidence of CAL in this group was 50.0%. In Daejeon, Korea, KD showed slight annual variations without seasonal differences. The rate of CAL in acute stage with one dose IVIG therapy (2 g/kg) was 8.3% in the IVIG responders.

[Development of the community mental health system and activities of the community mental health team in Kawasaki City].

Science.gov (United States)

Ito, Masato

2012-01-01

Since the 1960s, Kawasaki City has been leading the nation in its efforts regarding community mental health practices. Public institutions such as the Psychiatric Rehabilitation Center in the central area of the city and the Mental Health and Welfare Center in the southern area have mainly developed the psychiatric rehabilitation system. However, since 2000, new mental health needs have emerged, as the target of mental health and welfare services has been diversified to include people with developmental disorders, higher brain dysfunction, or social withdrawal, in addition to those with schizophrenia. Therefore, Kawasaki City's plan for community-based rehabilitation was drawn up, which makes professional support available for individuals with physical, intellectual, and mental disabilities. As the plan was being implemented, in 2008, the Northern Community Rehabilitation Center was established by both the public and private sectors in partnership. After the community mental health teams were assigned to both southern and northern areas of the city, the community partnership has been developed not only for individual support but also for other objectives that required the partnership. Takeshima pointed out that the local community should be inclusive of the psychiatric care in the final stage of community mental health care in Japan. Because of the major policies regarding people with disabilities, the final stage has been reached in the northern area of Kawasaki City. This also leads to improvement in measures for major issues in psychiatry, such as suicide prevention and intervention in psychiatric disease at an early stage.

Addison's Disease Caused by Tuberculosis with Atypical Hyperpigmentation and Active Pulmonary Tuberculosis.

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Namikawa, Hiroki; Takemoto, Yasuhiko; Kainuma, Shigeto; Umeda, Sakurako; Makuuchi, Ayako; Fukumoto, Kazuo; Kobayashi, Masanori; Kinuhata, Shigeki; Isaka, Yoshihiro; Toyoda, Hiromitsu; Kamata, Noriko; Tochino, Yoshihiro; Hiura, Yoshikazu; Morimura, Mina; Shuto, Taichi

2017-01-01

We herein report a case of Addison's disease caused by tuberculosis characterized by atypical hyperpigmentation, noted as exacerbation of the pigmentation of freckles and the occurrence of new freckles, that was diagnosed in the presence of active pulmonary tuberculosis. The clinical condition of the patient was markedly ameliorated by the administration of hydrocortisone and anti-tuberculosis agents. When exacerbation of the pigmentation of the freckles and/or the occurrence of new freckles are noted, Addison's disease should be considered as part of the differential diagnosis. In addition, the presence of active tuberculosis needs to be assumed whenever we treat patients with Addison's disease caused by tuberculosis, despite its rarity.

A simple method for assessment of human anti-Neu5Gc antibodies applied to Kawasaki disease.

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Vered Padler-Karavani

Full Text Available N-glycolylneuraminic acid (Neu5Gc is an immunogenic sugar of dietary origin that metabolically incorporates into diverse native glycoconjugates in humans. Anti-Neu5Gc antibodies are detected in all human sera, though with variable levels and epitope-recognition profiles. These antibodies likely play a role in several inflammation-mediated pathologies including cardiovascular diseases and cancer. In cancer, they have dualistic and opposing roles, either stimulating or repressing disease, as a function of their dose, and some of these antibodies serve as carcinoma biomarkers. Thus, anti-Neu5Gc antibodies may signify risk of inflammation-mediated diseases, and changes in their levels could potentially be used to monitor disease progression and/or response to therapy. Currently, it is difficult to determine levels of anti-Neu5Gc antibodies in individual human samples because these antibodies recognize multiple Neu5Gc-epitopes. Here we describe a simple and specific method for detection and overall estimation of human anti-Neu5Gc antibodies. We exploit the difference between two mouse models that differ only by Neu5Gc-presence (wild-type or Neu5Gc-absence (Cmah(-/- knockout. We characterize mouse serum from both strains by HPLC, lectin and mass-spectrometry analysis and show the target Neu5Gc-epitopes. We then use Cmah(-/- knockout sera to inhibit all non-Neu5Gc-reactivity followed by binding to wild-type sera to detect overall anti-Neu5Gc response in a single assay. We applied this methodology to characterize and quantify anti-Neu5Gc IgG and IgA in sera of patients with Kawasaki disease (KD at various stages compared to controls. KD is an acute childhood febrile disease characterized by inflammation of coronary arteries that untreated may lead to coronary artery aneurysms with risk of thrombosis and myocardial infarction. This estimated response is comparable to the average of detailed anti-Neu5Gc IgG profile analyzed by a sialoglycan microarray

A rare disease in an atypical location - Kimura's Disease of the upper extremity

Energy Technology Data Exchange (ETDEWEB)

Lam, Alan Cheuk Si; Lau, Vince Wing Hang [Queen Mary Hospital, Department of Radiology, Hong Kong (China); Au Yeung, Rex Kwok Him [University of Hong Kong, Department of Pathology, Li Ka Shing Faculty of Medicine, Hong Kong (China)

2015-12-15

Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

A rare disease in an atypical location - Kimura's Disease of the upper extremity

International Nuclear Information System (INIS)

Lam, Alan Cheuk Si; Lau, Vince Wing Hang; Au Yeung, Rex Kwok Him

2015-01-01

Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

Frequency of celiac disease in adult patients with typical or atypical malabsorption symptoms in isfahan, iran.

Science.gov (United States)

Emami, Mohammad Hassan; Kouhestani, Soheila; Karimi, Somayeh; Baghaei, Abdolmahdi; Janghorbani, Mohsen; Jamali, Nahid; Gholamrezaei, Ali

2012-01-01

Aim. Atypical presentations of celiac disease (CD) have now been shown to be much more common than classical (typical) form. We evaluated the frequency of CD among adult patients with typical or atypical symptoms of CD. Materials and Methods. Patients referred to two outpatient gastroenterology clinics in Isfahan (IRAN) were categorized into those with typical or atypical symptoms of CD. IgA antitissue transglutaminase antibody was assessed and followed by duodenal biopsy. In patients for whom endoscopy was indicated (independent of the serology), duodenal biopsy was taken. Histopathological changes were assessed according to the Marsh classification. Results. During the study period, 151 and 173 patients with typical and atypical symptoms were evaluated (mean age = 32.8 ± 12.6 and 35.8 ± 14.8 years, 47.0% and 56.0% female, resp.). Frequency of CD in patients with typical and atypical symptoms was calculated, respectively, as 5.9% (9/151) and 1.25% (3/173) based on positive serology and pathology. The overall frequency was estimated as at least 9.2% (14/151) and 4.0% (7/173) when data of seronegative patients were also considered. Conclusions. CD is more frequent among patients with typical symptoms of malabsorption and these patients should undergo duodenal biopsy, irrespective of the serology. In patients with atypical symptoms, serological tests should be performed followed by endoscopic biopsy, and routine duodenal biopsy is recommended when endoscopic evaluation is indicated because of symptoms.

Frequency of Celiac Disease in Adult Patients with Typical or Atypical Malabsorption Symptoms in Isfahan, Iran

Directory of Open Access Journals (Sweden)

Mohammad Hassan Emami

2012-01-01

Full Text Available Aim. Atypical presentations of celiac disease (CD have now been shown to be much more common than classical (typical form. We evaluated the frequency of CD among adult patients with typical or atypical symptoms of CD. Materials and Methods. Patients referred to two outpatient gastroenterology clinics in Isfahan (IRAN were categorized into those with typical or atypical symptoms of CD. IgA antitissue transglutaminase antibody was assessed and followed by duodenal biopsy. In patients for whom endoscopy was indicated (independent of the serology, duodenal biopsy was taken. Histopathological changes were assessed according to the Marsh classification. Results. During the study period, 151 and 173 patients with typical and atypical symptoms were evaluated (mean age = 32.8±12.6 and 35.8±14.8 years, 47.0% and 56.0% female, resp.. Frequency of CD in patients with typical and atypical symptoms was calculated, respectively, as 5.9% (9/151 and 1.25% (3/173 based on positive serology and pathology. The overall frequency was estimated as at least 9.2% (14/151 and 4.0% (7/173 when data of seronegative patients were also considered. Conclusions. CD is more frequent among patients with typical symptoms of malabsorption and these patients should undergo duodenal biopsy, irrespective of the serology. In patients with atypical symptoms, serological tests should be performed followed by endoscopic biopsy, and routine duodenal biopsy is recommended when endoscopic evaluation is indicated because of symptoms.

Epilepsy and other central nervous system diseases in atypical autism: a case control study

DEFF Research Database (Denmark)

Mouridsen, Svend Erik; Rich, Bente; Isager, Torben

2011-01-01

There is an increased but variable risk of epilepsy in autism spectrum disorders. The objective of this study is to compare the prevalence and types of epilepsy and other central nervous system (CNS) diseases in a clinical sample of 89 individuals diagnosed as children with atypical autism (AA...

Atypical presentation of probable Creutzfeldt-Jakob disease associated with anti-Zic4 antibody: Literature review of neuronal antibodies in Creutzfeldt-Jakob disease.

Science.gov (United States)

Salazar, Richard

2018-05-01

Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia. His initial examination demonstrated akinetic mutism, diffuse rigidity, dysautononia, and Cheyne-Stokes respiration. Over the following weeks his condition progressed to profound coma. A comprehensive infectious, metabolic, inflammatory and autoimmune work-up yielded negative results. Empiric immunosuppressive therapy ensued. He expired three months after symptoms onset. Autopsy was not performed. After his demise, prion tests came back abnormal for elevated 14-3-3 protein, total tau and positive RTQuIC. Later on, anti-Zic4 antibodies were found in serum. This case underscores the importance of a high index of suspicion for CJD even in case of atypical features or the concurrence of neuronal antibodies. Further larger prospective studies on the prevalence of these neuronal antibodies in CJD and the contribution of these autoantibodies to disease pathophysiology are necessary. Copyright © 2018 Elsevier B.V. All rights reserved.

Pyrosequencing Analysis of Norovirus Genogroup II Distribution in Sewage and Oysters: First Detection of GII.17 Kawasaki 2014 in Oysters.

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Pu, Jian; Kazama, Shinobu; Miura, Takayuki; Azraini, Nabila Dhyan; Konta, Yoshimitsu; Ito, Hiroaki; Ueki, You; Cahyaningrum, Ermaya Eka; Omura, Tatsuo; Watanabe, Toru

2016-12-01

Norovirus GII.3, GII.4, and GII.17 were detected using pyrosequencing in sewage and oysters in January and February 2015, in Japan. The strains in sewage and oyster samples were genetically identical or similar, predominant strains belonging to GII.17 Kawasaki 2014 lineage. This is the first report of GII.17 Kawasaki 2014 in oysters.

An evaluation of coronary artery lesions of Kawasaki disease and congenital heart disease using rotary three dimensional digital cardiovascular angiography

International Nuclear Information System (INIS)

Watanabe, Masanori; Ogawa, Shunichi; Kumazaki, Tatsuo; Hirayama, Tsuneo

1994-01-01

Congenital heart disease and the coronary artery lesions of children suffering from Kawasaki disease were evaluated by cardiovascular angiography using a newly developed rotary three-dimensional digital angiography method, and the usefulness of the device was examined. This method enable the observation of lesions from 144 directions within a 180 degree range depicting an image from optimal directions. In addition, the radiation exposure during one angiography was about one fifth of that of conventional cineangiography. With regard to the lesions of the coronary artery, identification of the localization of the stenotic lesions were made possible, especially at bifurcations, or the stenotic lesions overlapping with other bifurcations or coronary arteries aneurysms as well as the structure at the ostium of the left and right coronary arteries, which were difficult to identify using conventional coronary artery angiography. For the case of patient ductus arteriosus or major aortopulmonary collateral artery (MAPCA), separation and imaging of the overlap with other blood vessels through the three-dimensional observation became possible. This method is effective for the evaluation of the site, direction and morphology of these arteries. With regard to stenosis of the right ventricular outflow tract, the morphology and the degree of stenosis could be evaluated more accurately than by conventional cineangiography. In addition, the images matched well with the operative findings. This method was also effective for the diagnosis and evaluation of the stenosis at the main pulmonary artery and stenosis of the bifurcation of the right and left pulmonary arteries overlapping with the main trunk of the pulmonary artery. The problem with this method is that it cannot be used for the quantitative evaluation of the cardiac function because it cannot take images from multiple directions at the same time or cannot take temporal images from one direction. (author)

The Role of IL-1 signaling in a mouse model of Kawasaki Disease-associated Abdominal Aortic Aneurysm

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Wakita, Daiko; Kurashima, Yosuke; Crother, Timothy R.; Rivas, Magali Noval; Lee, Youngho; Chen, Shuang; Fury, Wen; Bai, Yu; Wagner, Shawn; Li, Debiao; Lehman, Thomas; Fishbein, Michael C.; Hoffmann, Hal; Shah, Prediman K.; Shimada, Kenichi; Arditi, Moshe

2016-01-01

Objective Kawasaki disease (KD) is the most common cause of acquired cardiac disease in US children. In addition to coronary artery abnormalities and aneurysms, it can be associated with systemic arterial aneurysms. We evaluated the development of systemic arterial dilatation and aneurysms, including abdominal aortic aneurysm (AAA) in the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model. Methods and Results We discovered that in addition to aortitis, coronary arteritis and myocarditis, the LCWE-induced KD mouse model is also associated with abdominal aorta dilatation and AAA, as well as renal and iliac artery aneurysms. AAA induced in KD mice was exclusively infrarenal, both fusiform and saccular, with intimal proliferation, myofibroblastic proliferation, break in the elastin layer, vascular smooth muscle cell loss, and inflammatory cell accumulation in the media and adventitia. Il1r−/−, Il1a−/−, and Il1a−/− mice were protected from KD associated AAA. Infiltrating CD11c+ macrophages produced active caspase-1 and caspase-1 or NLRP3 deficiency inhibited AAA formation. Treatment with IL-1R antagonist (Anakinra), anti-IL-1α, or anti-IL-1β mAb blocked LCWE-induced AAA formation. Conclusions Similar to clinical KD, the LCWE-induced KD vasculitis mouse model can also be accompanied by AAA formation. Both IL-1α and IL-1β play a key role, and that use of an IL-1R blocking agent that inhibits both pathways may be a promising therapeutic target not only for KD coronary arteritis, but also for the other systemic arterial aneurysms including AAA that maybe seen in severe cases of KD. The LCWE-induced vasculitis model may also represent an alternative model for AAA disease. PMID:26941015

Clinical implications in laboratory parameter values in acute Kawasaki disease for early diagnosis and proper treatment.

Science.gov (United States)

Seo, Yu-Mi; Kang, Hyun-Mi; Lee, Sung-Churl; Yu, Jae-Won; Kil, Hong-Ryang; Rhim, Jung-Woo; Han, Ji-Whan; Lee, Kyung-Yil

2018-05-01

This study aimed to analyse laboratory values according to fever duration, and evaluate the relationship across these values during the acute phase of Kawasaki disease (KD) to aid in the early diagnosis for early-presenting KD and incomplete KD patients. Clinical and laboratory data of patients with KD (n=615) were evaluated according to duration of fever at presentation, and were compared between patients with and without coronary artery lesions (CALs). For evaluation of the relationships across laboratory indices, patients with a fever duration of 5 days or 6 days were used (n=204). The mean fever duration was 6.6±2.3 days, and the proportions of patients with CALs was 19.3% (n=114). C-reactive proteins (CRPs) and neutrophil differential values were highest and hemoglobin, albumin, and lymphocyte differential values were lowest in the 6-day group. Patients with CALs had longer total fever duration, higher CRP and neutrophil differential values and lower hemoglobin and albumin values compared to patients without CALs. CRP, albumin, neutrophil differential, and hemoglobin values at the peak inflammation stage of KD showed positive or negative correlations each other. The severity of systemic inflammation in KD was reflected in the laboratory values including CRP, neutrophil differential, albumin, and hemoglobin. Observing changes in these laboratory parameters by repeated examinations prior to the peak of inflammation in acute KD may aid in diagnosis of early-presenting KD patients.

Radiographic differentiation of atypical tuberculosis from mycobacterium tuberculosis

International Nuclear Information System (INIS)

Tarver, R.D.; Pearcy, E.A.; Conces, D.J. Jr.; Mathur, P.N.

1987-01-01

The chest radiographs of 95 patients with the new diagnosis of atypical turberculosis were reviewed to determine if any significant differences between atypical tuberculosis and that caused by Mycobacterium tuberculosis could be discerned. Findings included upper lobe involvement in B4 of the 95 patients and cavities in 76, with nearly equal groups having no, moderate, or extensive surrounding alveolar disease. Nodules were common; in six patients a nodule was the sole manifestation of disease. Adenopathy was seen in 12 of the 95 patients, atlectasis in 45, pleural thickening in 90, and effusions in three. These radiographic findings did not allow the radiographic differentiation of atypical tuberculosis from Mycobacterium tuberculosis infection

The importance of biochemical and genetic findings in the diagnosis of atypical Norrie disease.

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Rodríguez-Muñoz, Ana; García-García, Gema; Menor, Francisco; Millán, José M; Tomás-Vila, Miguel; Jaijo, Teresa

2018-01-26

Norrie disease (ND) is a rare X-linked disorder characterized by bilateral congenital blindness. ND is caused by a mutation in the Norrie disease pseudoglioma (NDP) gene, which encodes a 133-amino acid protein called norrin. Intragenic deletions including NDP and adjacent genes have been identified in ND patients with a more severe neurologic phenotype. We report the biochemical, molecular, clinical and radiological features of two unrelated affected males with a deletion including NDP and MAO genes. Biochemical and genetic analyses were performed to understand the atypical phenotype and radiological findings. Biogenic amines in cerebrospinal fluid (CSF) were measured by high-performance liquid chromatography. The coding exons of NDP gene were amplified by polymerase chain reaction. Multiplex ligation-dependent probe amplification and chromosomal microarray were carried out on both affected males. Computed tomography and magnetic resonance imaging were performed on the two patients. In one patient, the serotonin and catecholamine metabolite levels in CSF were virtually undetectable. In both patients, genetic studies revealed microdeletions in the Xp11.3 region, involving the NDP, MAOA and MAOB genes. Radiological examination demonstrated brain and cerebellar atrophy. We suggest that alterations caused by MAO deficit may remain during the first years of life. Clinical phenotype, biochemical findings and neuroimaging can guide the genetic study in patients with atypical ND and help us to a better understanding of this disease.

Diagnostic Accuracy of a Combined Analysis of Cerebrospinal Fluid t-PrP, t-tau, p-tau, and Aβ42 in the Differential Diagnosis of Creutzfeldt-Jakob Disease from Alzheimer's Disease with Emphasis on Atypical Disease Variants.

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Abu Rumeileh, Samir; Lattanzio, Francesca; Stanzani Maserati, Michelangelo; Rizzi, Romana; Capellari, Sabina; Parchi, Piero

2017-01-01

According to recent studies, the determination of cerebrospinal fluid (CSF) total tau (t-tau)/phosphorylated tau (p-tau) ratio and total prion protein (t-PrP) levels significantly improves the accuracy of the diagnosis of Alzheimer's disease (AD) in atypical cases with clinical or laboratory features mimicking Creutzfeldt-Jakob disease (CJD). However, this has neither been validated nor tested in series including atypical CJD variants. Furthermore, the added diagnostic value of amyloid-β (Aβ)42 remains unclear. To address these issues, we measured t-PrP, 14-3-3, t-tau, p-tau, and Aβ42 CSF levels in 45 typical and 44 atypical/rapidly progressive AD patients, 54 typical and 54 atypical CJD patients, and 33 controls. CJD patients showed significantly lower CSF t-PrP levels than controls and AD patients. Furthermore, atypical CJD was associated with lower t-PrP levels in comparison to typical CJD. T-tau, 14-3-3, or t-PrP alone yielded, respectively, 80.6, 63.0, and 73.0% sensitivity and 75.3, 92.1, and 75% specificity in distinguishing AD from CJD. On receiver operating characteristic (ROC) curve analyses of biomarker combinations, the (t-tau×Aβ42)/(p-tau×t-PrP) ratio achieved the best accuracy, with 98.1% sensitivity and 97.7% specificity overall, and 96.2% sensitivity and 95.5% specificity for the "atypical" disease groups. Our results show that the combined analysis of CSF t-PrP, t-tau, p-tau, and Aβ42 is clinically useful in the differential diagnosis between CJD and AD. Furthermore, the finding of reduced CSF t-PrP levels in CJD patients suggest that, likewise Aβ42 in AD, CSF t-PrP levels reflect the extent of PrPc conversion into abnormal PrP (PrPSc) and the burden of PrPSc deposition in CJD.

Correlation of HAMP gene polymorphisms and expression with the susceptibility and length of hospital stays in Taiwanese children with Kawasaki disease

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Lu, Hsing-Fang; Wong, Henry Sung-Ching; Yu, Hong-Ren; Kuo, Hsing-Chun; Huang, Fu-Chen; Lo, Mao-Hung; Hsieh, Kai-Sheng; Chen, Su-Fen; Chang, Wei-Chiao; Kuo, Ho-Chang

2017-01-01

Kawasaki disease (KD) is a form of systemic vasculitis. Regarding its pathogenesis, HAMP gene encoding hepcidin, which is significant for iron metabolism, has a vital function. In this study, we recruited a total of 381 KD patients for genotyping. Data from 997 subjects (500 subjects from cohort 1; 497 subjects from cohort 2) were used for analysis. Using TaqMan allelic discrimination, we determined five tag SNPs (rs916145, rs10421768, rs3817623, rs7251432, and rs2293689). Treatment outcome data related to such clinical phenotypes as coronary artery lesions (CAL), coronary artery aneurysms (CAA), and intravenous immunoglobulin (IVIG) effects were also collected. Furthermore, we measured plasma hepcidin levels with an enzyme-linked immunosorbent assay. We found that HAMP gene polymorphism (rs7251432, and rs2293689) was significantly correlated with KD risk and that plasma hepcidin levels both before and after IVIG treatment had a significantly positive correlation with length of hospital stays (R = 0.217, p = 0.046 and R = 0.381, p < 0.0001, respectively). In contrast, plasma hepcidin levels has a negative correlation with KD patients’ albumin levels (R = −0.27, p < 0.001) prior to IVIG treatment. This study's findings indicate that HAMP might have a role in the disease susceptibility, as well as its expressions correlated length of hospital stays, and albumin levels in Taiwanese children with KD. PMID:28881695

Ideal gas approximation for a two-dimensional rarefied gas under Kawasaki dynamics

NARCIS (Netherlands)

Gaudillière, A.; Hollander, den W.Th.F.; Nardi, F.R.; Olivieri, E.; Scoppola, E.

2009-01-01

In this paper we consider a two-dimensional lattice gas under Kawasaki dynamics, i.e., particles hop around randomly subject to hard-core repulsion and nearest-neighbor attraction. We show that, at fixed temperature and in the limit as the particle density tends to zero, such a gas evolves in a way

Metastability for Kawasaki dynamics at low temperature with two types of particles

NARCIS (Netherlands)

Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

2011-01-01

This is the fi??rst in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large fi??nite box with an open boundary. Each pair of particles occupying neighboring sites has a negative

Atypical meningococcal meningitis with rashless presentation：A case report

Institute of Scientific and Technical Information of China (English)

Sunita; Singh Manpreet; Kapoor Dheeraj

2012-01-01

Meningococcal disease is the major health problem in developing world. The clinical presentation is varied, ranging from transient fever and bacteraemia to fulminant disease with death ensuing within hours of the onset of clinical symptoms. The classical clinical manifestations of meningococcal disease have been well described, but atypical presentations if unrecognized, may lead to a delay in treatment and fatal outcome. We here report a case presented with atypical presentation of meningococcal meningitis without classical rash, which was diagnosed and managed successfully.

Decreasing fertility rate correlates with the chronological increase and geographical variation in incidence of Kawasaki disease in Japan.

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Yoshiro Nagao

Full Text Available BACKGROUND: Kawasaki disease (KD is a common cause of acquired paediatric heart disease in developed countries. KD was first identified in the 1960s in Japan, and has been steadily increasing since it was first reported. The aetiology of KD has not been defined, but is assumed to be infection-related. The present study sought to identify the factor(s that mediate the geographical variation and chronological increase of KD in Japan. METHODS AND FINDINGS: Based upon data reported between 1979 and 2010 from all 47 prefectures in Japan, the incidence and mean patient age at the onset of KD were estimated. Using spatial and time-series analyses, incidence and mean age were regressed against climatic/socioeconomic variables. Both incidence and mean age of KD were inversely correlated with the total fertility rate (TFR; i.e., the number of children that would be born to one woman. The extrapolation of a time-series regressive model suggested that KD emerged in the 1960s because of a dramatic decrease in TFR in the 1940s through the 1950s. CONCLUSIONS: Mean patient age is an inverse surrogate for the hazard of contracting the aetiologic agent. Therefore, the observed negative correlation between mean patient age and TFR suggests that a higher TFR is associated with KD transmission. This relationship may be because a higher TFR facilitates sibling-to-sibling transmission. Additionally, the observed inverse correlation between incidence and TFR implies a paradoxical "negative" correlation between the incidence and the hazard of contracting the aetiologic agent. It was hypothesized that a decreasing TFR resulted in a reduced hazard of contracting the agent for KD, thereby increasing KD incidence.

Duration of high-dose aspirin therapy does not affect long-term coronary artery outcomes in Kawasaki disease.

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Migally, Karl; Braunlin, Elizabeth A; Zhang, Lei; Binstadt, Bryce A

2018-05-02

BackgroundHigh-dose aspirin (HDA) is used with intravenous immunoglobulin (IVIg) in Kawasaki disease (KD). Practice regarding HDA varies, and it is unclear whether HDA duration affects the long-term course.MethodsWe retrospectively studied KD patients at our hospital for over 10 years. Patients were categorized as having received HDA for 0, 1-7, or >7 days. Primary outcome was the maximum coronary Z-score at diagnosis and follow-up; secondary outcomes included inflammatory markers.ResultsOne hundred and three patients had HDA duration documented, of which 35 patients had coronary artery abnormalities (CAAs) at diagnosis. There was no difference in demographics or inflammatory markers between the HDA groups, and no difference in HDA duration between patients with or without CAAs. Seventeen patients received no HDA; they had longer illness and defervescence duration before diagnosis, and were less likely to receive IVIg. For CAAs, multivariate regression revealed that HDA duration did not predict the coronary Z-score at 9-15 months. Higher Z-score at diagnosis was associated with higher Z-score at 9-15 months.ConclusionThe only factor associated with coronary Z-score at 9-15 months was the Z-score at diagnosis. At our institution, longer illness and defervescence duration and the lack of IVIg administration were associated with not administering HDA. HDA duration did not affect the clinically relevant outcomes, particularly CAA persistence.Pediatric Research advance online publication, 2 May 2018; doi:10.1038/pr.2018.44.

PENGARUH KELOMPOK REFERENSI TERHADAP KEPUTUSAN PEMBELIAN KAWASAKI NINJA 250 CC

OpenAIRE

Kartika WB, Sylvia; Sidig, Rosyid

2018-01-01

The purpose of this study is to examine the affect of reference groups  on purchasing decision of  Kawasaki Ninja 250 cc in the city of Jambi. Size of sample consist of 100 unit that chousen randomly. While data analysis used  multiple regression analysis. The results showed that simultaneouly   normative, value expression, and  informative have significant role. But, based on partiall test  normative has no significant affect. Hence,  reference group h...

Hepcidin-Induced Iron Deficiency Is Related to Transient Anemia and Hypoferremia in Kawasaki Disease Patients

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Huang, Ying-Hsien; Kuo, Ho-Chang; Huang, Fu-Chen; Yu, Hong-Ren; Hsieh, Kai-Sheng; Yang, Ya-Ling; Sheen, Jiunn-Ming; Li, Sung-Chou; Kuo, Hsing-Chun

2016-01-01

Kawasaki disease (KD) is a type of systemic vasculitis that primarily affects children under the age of five years old. For sufferers of KD, intravenous immunoglobulin (IVIG) has been found to successfully diminish the occurrence of coronary artery lesions. Anemia is commonly found in KD patients, and we have shown that in appropriately elevated hepcidin levels are related to decreased hemoglobin levels in these patients. In this study, we investigated the time period of anemia and iron metabolism during different stages of KD. A total of 100 patients with KD and 20 control subjects were enrolled in this study for red blood cell and hemoglobin analysis. Furthermore, plasma, urine hepcidin, and plasma IL-6 levels were evaluated using enzyme-linked immunosorbent assay in 20 KD patients and controls. Changes in hemoglobin, plasma iron levels, and total iron binding capacity (TIBC) were also measured in patients with KD. Hemoglobin, iron levels, and TIBC were lower (p < 0.001, p = 0.009, and p < 0.001, respectively) while plasma IL-6 and hepcidin levels (both p < 0.001) were higher in patients with KD than in the controls prior to IVIG administration. Moreover, plasma hepcidin levels were positively and significantly correlated with urine hepcidin levels (p < 0.001) prior to IVIG administration. After IVIG treatment, plasma hepcidin and hemoglobin levels significantly decreased (both p < 0.001). Of particular note was a subsequent gradual increase in hemoglobin levels during the three weeks after IVIG treatment; nevertheless, the hemoglobin levels stayed lower in KD patients than in the controls (p = 0.045). These findings provide a longitudinal study of hemoglobin changes and among the first evidence that hepcidin induces transient anemia and hypoferremia during KD’s acute inflammatory phase. PMID:27187366

Hepcidin-Induced Iron Deficiency Is Related to Transient Anemia and Hypoferremia in Kawasaki Disease Patients

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Ying-Hsien Huang

2016-05-01

Full Text Available Kawasaki disease (KD is a type of systemic vasculitis that primarily affects children under the age of five years old. For sufferers of KD, intravenous immunoglobulin (IVIG has been found to successfully diminish the occurrence of coronary artery lesions. Anemia is commonly found in KD patients, and we have shown that in appropriately elevated hepcidin levels are related to decreased hemoglobin levels in these patients. In this study, we investigated the time period of anemia and iron metabolism during different stages of KD. A total of 100 patients with KD and 20 control subjects were enrolled in this study for red blood cell and hemoglobin analysis. Furthermore, plasma, urine hepcidin, and plasma IL-6 levels were evaluated using enzyme-linked immunosorbent assay in 20 KD patients and controls. Changes in hemoglobin, plasma iron levels, and total iron binding capacity (TIBC were also measured in patients with KD. Hemoglobin, iron levels, and TIBC were lower (p < 0.001, p = 0.009, and p < 0.001, respectively while plasma IL-6 and hepcidin levels (both p < 0.001 were higher in patients with KD than in the controls prior to IVIG administration. Moreover, plasma hepcidin levels were positively and significantly correlated with urine hepcidin levels (p < 0.001 prior to IVIG administration. After IVIG treatment, plasma hepcidin and hemoglobin levels significantly decreased (both p < 0.001. Of particular note was a subsequent gradual increase in hemoglobin levels during the three weeks after IVIG treatment; nevertheless, the hemoglobin levels stayed lower in KD patients than in the controls (p = 0.045. These findings provide a longitudinal study of hemoglobin changes and among the first evidence that hepcidin induces transient anemia and hypoferremia during KD’s acute inflammatory phase.

[Immunofluorescence assay with Crithidia luciliae for the detection of anti-DNA antibodies. Atypical images and their relationship with Chagas' disease and leishmaniasis].

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Griemberg, Gloria; Ferrarotti, Nidia F; Svibel, Graciela; Ravelli, Maria R; Taranto, Nestor J; Malchiodi, Emilio L; Pizzimenti, Maria C

2006-01-01

Anti-native DNA antibodies can be detected by indirect immunofluorescence assay with Crithidia luciliae, displaying an annular image due to a kinetoplast containing double stranded DNA. Other structures such as membrane, flagellum and basal corpuscle can be stained as well, showing what is called atypical fluorescent images. As C. luciliae belongs to the Trypanosomatidae family, which include the human pathogens Trypanosoma cruzi and Leishmania spp., it was considered that these atypical images could be caused by cross-reactions. Serological studies for Chagas' disease were performed in 105 serum samples displaying atypical images. Sixty four percent of the samples from non endemic and 78.3% from endemic areas for Chagas' disease showed fluorescence in both, membrane and flagellum (joint image). Fifty samples from normal blood donors and 57 samples from patients with conective tissue diseases were tested with C. luciliae. None of them presented the joint image except for two patients with lupus who were also chagasic. In addition, 54 samples from chagasic patients were studied and all of them presented the joint image. We also studied 46 samples from patients with leishmaniasis from whom 28 were coinfected with T. cruzi. The joint image was observed in 88.0% of the samples with leishmaniasis and in 89.3% of the co-infected samples. The results suggest that C. luciliae could be used as an economical, and of low risk, alternative substrate for the serological diagnosis of Chagas' disease, even though it does not discriminate for Leishmania spp. infection. This study also suggests that whenever atypical images are observed in C. luciliae during the search for anti-DNA antibodies, it would be convenient to submit the patient to clinical and serological tests for the diagnosis of leishmaniosis and Chagas' disease.

Kawasaki dynamics with two types of particles : stable/megastable configurations and communication heights

NARCIS (Netherlands)

Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

2011-01-01

This is the second in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large finite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

Kawasaki dynamics with two types of particles: stable/megastable configurations and communication heights

NARCIS (Netherlands)

Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

2011-01-01

This is the second in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large finite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

Oral considerations of Kawasaki syndrome : a case report

OpenAIRE

Figueiredo, Márcia Cançado; Pires, Patrícia Duarte Simões; Silva, Daniel Demétrio Faustino da; Ouriques, Maurício Cernicchiaro; Squef, Rocío

2010-01-01

El objetivo del presente estudio es presentar un caso clínico y revisar la literatura existente sobre el Síndrome de Kawasaki (SK) tan frecuente en pacientes con necesidades especiales. Las características clínicas de esta patología se observan ya en niños muy jóvenes, en la gran mayoría de los casos menores de 5 años de edad. Esta es una patología que puede llevar al óbito, pues revela un compromiso cardíaco importante. Hay señales y síntomas en la cavidad oral en la fase aguda de la enferme...

COMBINED 24-HOURS ESOPHAGEAL PH MONITORING AND MULTICHANNEL INTRALUMINAL IMPEDANCE FOR COMPARISON OF GASTROESOPHAGEAL REFLUX IN CHILDREN WITH TYPICAL VERSUS ATYPICAL SYMPTOMS OF GASTROESOPHAGEAL REFLUX DISEASE.

Science.gov (United States)

Dehghani, Seyed Mohsen; Taghavi, Seyed Alireza; Javaherizadeh, Hazhir; Nasri, Maryam

2016-01-01

- Gastroesophageal reflux disease is the most common esophageal disorder in pediatrics. - The aim of this study was to compare reflux parameters of typical and atypical symptoms of gastroesophageal reflux disease using 24-hour esophageal pH monitoring and multichannel intraluminal impedance in pediatric population. - In this prospective study, 43 patients aged less than 18 year with suspected gastroesophageal reflux disease were enrolled. The patients were divided into two groups based on the main presenting symptoms (typical versus atypical). Twenty four-hour pH monitoring and multichannel intraluminal impedance were performed in all the patients for comparing these two group regarding association of symptoms and reflux. Number of refluxes, pH related reflux, total reflux time, reflux more than 5 minutes, longest time of the reflux, lowest pH at reflux, reflux index were recorded and compared. Data comparison was done using SPSS. - The mean age of the patients was 5.7±3.4 years and 65.1% were male. Out of 43 patients 24 cases had typical symptoms and 19 had atypical symptoms. The mean reflux events detected by multichannel intraluminal impedance was more than mean reflux events detected by pH monitoring (308.4±115.8 vs 69.7±66.6) with P value of 0.037, which is statistically significant. The mean symptom index and symptom association probability were 35.01% ± 20.78% and 86.42% ± 25.79%, respectively in multichannel intraluminal impedance versus 12.73% ± 12.48% and 45.16% ± 42.29% in pH monitoring (P value reflux was 46.26±47.16 and 30.9±22.09 for atypical and typical symptoms respectively. The mean symptom index was 18.12% ± 13.101% and 8.30% ± 10.301% in atypical and typical symptoms respectively (P=0.034). Bolus clearance was longer in atypical symptoms compared typical symptoms(Preflux was found in children with atypical symptoms of reflux. Longer duration of bolus clearance was found in group with atypical symptoms of reflux.

Using atypical symptoms and red flags to identify non-demyelinating disease.

LENUS (Irish Health Repository)

Kelly, Siobhan B

2012-01-01

Red flags and atypical symptoms have been described as being useful in suggesting alternative diagnoses to multiple sclerosis (MS) and clinically isolated syndrome (CIS); however, their diagnostic utility has not been assessed. The aim of this study was to establish the predictive value of red flags and the typicality\\/atypicality of symptoms at presentation in relation to the final diagnosis of patients referred with suspected MS.

Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor.

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Yeo, Yunku; Kim, TaeYeon; Ha, KeeSoo; Jang, GiYoung; Lee, JungHwa; Lee, KwangChul; Son, ChangSung; Lee, JooWon

2009-02-01

Kawasaki disease (KD) patients younger than 1 year of age are at especially high risk of developing coronary artery abnormalities (CAA). To define the clinical characteristics of this group, as well as the risk factors predisposing them to CAA, we reviewed the medical records of 136 KD patients younger than 1 year of age who were treated at the Korea University Medical Center from January 2001 to July 2006. Of these patients, 16 developed CAA (11.8%). The CAA(+) group had a longer duration of total fever than the CAA(-) group (9.1+/-3.7 days vs. 6.3+/-2.0 days, p=0.011), but did not differ in the duration of pre- and post-intravenous gamma-globulin (IVGG) fever. The CAA(+) group had fewer diagnostic symptoms than the CAA(-) group (2.7+/-1.1 vs. 4.3+/-1.2, p<0.001). Of the hematological findings, the CAA(+) group only differed from the CAA(-) group in having significantly higher total white blood cell (19.2+/-6.0 vs. 14.7+/-4.7 K/mm(3), p=0.007) and platelet (462.9+/-101.0 vs. 383.6+/-121.1 K/mm(3), p=0.014) levels. Multivariable logistic regression analysis showed that the only factors which were significantly associated with the development of CAA were the total number of symptoms (OR=0.493, 95% CI=0.293-0.829, p=0.007) and the duration of total fever (OR=1.405, 95% CI=1.092-1.808, p=0.008). Conclusively, incomplete clinical manifestations and a longer duration of total fever are significantly associated with the development of CAA in KD patients younger than 1 year of age. Therefore, these patients should be monitored for incomplete KD, especially if unexplained fever continues, and treatment to shorten the duration of total fever should be initiated.

Identification of novel susceptibility Loci for kawasaki disease in a Han chinese population by a genome-wide association study.

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Fuu-Jen Tsai

Full Text Available Kawasaki disease (KD is an acute systemic vasculitis syndrome that primarily affects infants and young children. Its etiology is unknown; however, epidemiological findings suggest that genetic predisposition underlies disease susceptibility. Taiwan has the third-highest incidence of KD in the world, after Japan and Korea. To investigate novel mechanisms that might predispose individuals to KD, we conducted a genome-wide association study (GWAS in 250 KD patients and 446 controls in a Han Chinese population residing in Taiwan, and further validated our findings in an independent Han Chinese cohort of 208 cases and 366 controls. The most strongly associated single-nucleotide polymorphisms (SNPs detected in the joint analysis corresponded to three novel loci. Among these KD-associated SNPs three were close to the COPB2 (coatomer protein complex beta-2 subunit gene: rs1873668 (p = 9.52×10⁻⁵, rs4243399 (p = 9.93×10⁻⁵, and rs16849083 (p = 9.93×10⁻⁵. We also identified a SNP in the intronic region of the ERAP1 (endoplasmic reticulum amino peptidase 1 gene (rs149481, p(best = 4.61×10⁻⁵. Six SNPs (rs17113284, rs8005468, rs10129255, rs2007467, rs10150241, and rs12590667 clustered in an area containing immunoglobulin heavy chain variable regions genes, with p(best-values between 2.08×10⁻⁵ and 8.93×10⁻⁶, were also identified. This is the first KD GWAS performed in a Han Chinese population. The novel KD candidates we identified have been implicated in T cell receptor signaling, regulation of proinflammatory cytokines, as well as antibody-mediated immune responses. These findings may lead to a better understanding of the underlying molecular pathogenesis of KD.

Expression and function of the atypical cadherin FAT1 in chronic liver disease

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Valletta, Daniela; Czech, Barbara; Thasler, Wolfgang E.; Müller, Martina; Bosserhoff, Anja-Katrin; Hellerbrand, Claus

2012-01-01

Highlights: ► The expression of the atypical cadherin FAT1 is increased in chronic liver disease. ► FAT1 expression goes up during the activation of hepatic stellate cells (HSCs). ► Activated HSCs are the cellular source of enhanced FAT1 expression in diseased livers. ► FAT1 enhanced NFkB activity and resistance to apoptosis in activated HSCs. ► FAT1 is a new therapeutic target for prevention and treatment of hepatic fibrosis. -- Abstract: Hepatic fibrosis can be considered as wound healing process in response to hepatocellular injury. Activation of hepatic stellate cells (HSCs) is a key event of hepatic fibrosis since activated HSCs are the cellular source of enhanced extracellular matrix deposition, and reversion of liver fibrosis is accompanied by clearance of activated HSCs by apoptosis. The atypical cadherin FAT1 has been shown to regulate diverse biological functions as cell proliferation and planar cell polarity, and also to affect wound healing. Here, we found increased FAT1 expression in different murine models of chronic liver injury and in cirrhotic livers of patients with different liver disease. Also in hepatic tissue of patients with non-alcoholic steatohepatitis FAT1 expression was significantly enhanced and correlated with collagen alpha I(1) expression. Immunohistochemistry revealed no significant differences in staining intensity between hepatocytes in normal and cirrhotic liver tissue but myofibroblast like cells in fibrotic septa of cirrhotic livers showed a prominent immunosignal. Furthermore, FAT1 mRNA and protein expression markedly increased during in vitro activation of primary human and murine HSCs. Together, these data indicated activated HSCs as cellular source of enhanced FAT1 expression in diseased livers. To gain insight into the functional role of FAT1 in activated HSCs we suppressed FAT1 in these cells by siRNA. We newly found that FAT1 suppression in activated HSCs caused a downregulation of NFκB activity. This

Expression and function of the atypical cadherin FAT1 in chronic liver disease

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Valletta, Daniela; Czech, Barbara [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany); Thasler, Wolfgang E. [Grosshadern Tissue Bank and Center for Liver Cell Research, Department of Surgery, Ludwig-Maximilians-University Munich (Germany); Mueller, Martina [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany); Bosserhoff, Anja-Katrin [Institute of Pathology, University of Regensburg, Regensburg (Germany); Hellerbrand, Claus, E-mail: claus.hellerbrand@ukr.de [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany)

2012-09-28

Highlights: Black-Right-Pointing-Pointer The expression of the atypical cadherin FAT1 is increased in chronic liver disease. Black-Right-Pointing-Pointer FAT1 expression goes up during the activation of hepatic stellate cells (HSCs). Black-Right-Pointing-Pointer Activated HSCs are the cellular source of enhanced FAT1 expression in diseased livers. Black-Right-Pointing-Pointer FAT1 enhanced NFkB activity and resistance to apoptosis in activated HSCs. Black-Right-Pointing-Pointer FAT1 is a new therapeutic target for prevention and treatment of hepatic fibrosis. -- Abstract: Hepatic fibrosis can be considered as wound healing process in response to hepatocellular injury. Activation of hepatic stellate cells (HSCs) is a key event of hepatic fibrosis since activated HSCs are the cellular source of enhanced extracellular matrix deposition, and reversion of liver fibrosis is accompanied by clearance of activated HSCs by apoptosis. The atypical cadherin FAT1 has been shown to regulate diverse biological functions as cell proliferation and planar cell polarity, and also to affect wound healing. Here, we found increased FAT1 expression in different murine models of chronic liver injury and in cirrhotic livers of patients with different liver disease. Also in hepatic tissue of patients with non-alcoholic steatohepatitis FAT1 expression was significantly enhanced and correlated with collagen alpha I(1) expression. Immunohistochemistry revealed no significant differences in staining intensity between hepatocytes in normal and cirrhotic liver tissue but myofibroblast like cells in fibrotic septa of cirrhotic livers showed a prominent immunosignal. Furthermore, FAT1 mRNA and protein expression markedly increased during in vitro activation of primary human and murine HSCs. Together, these data indicated activated HSCs as cellular source of enhanced FAT1 expression in diseased livers. To gain insight into the functional role of FAT1 in activated HSCs we suppressed FAT1 in these

Atypical antipsychotics and glucose homeostasis.

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Bergman, Richard N; Ader, Marilyn

2005-04-01

Persistent reports have linked atypical antipsychotics with diabetes, yet causative mechanisms responsible for this linkage are unclear. Goals of this review are to outline the pathogenesis of nonimmune diabetes and to survey the available literature related to why antipsychotics may lead to this disease. We accessed the literature regarding atypical antipsychotics and glucose homeostasis using PubMed. The search included English-language publications from 1990 through October 2004. Keywords used included atypical antipsychotics plus one of the following: glucose, insulin, glucose tolerance, obesity, or diabetes. In addition, we culled information from published abstracts from several national and international scientific meetings for the years 2001 through 2004, including the American Diabetes Association, the International Congress on Schizophrenia Research, and the American College of Neuropsychopharmacology. The latter search was necessary because of the paucity of well-controlled prospective studies. We examined publications with significant new data or publications that contributed to the overall comprehension of the impact of atypical antipsychotics on glucose metabolism. We favored original peer-reviewed articles and were less likely to cite single case studies and/or anecdotal information. Approximately 75% of the fewer than 150 identified articles were examined and included in this review. Validity of data was evaluated using the existence of peer-review status as well as our own experience with methodology described in the specific articles. The metabolic profile caused by atypical antipsychotic treatment resembles type 2 diabetes. These agents cause weight gain in treated subjects and may induce obesity in both visceral and subcutaneous depots, as occurs in diabetes. Insulin resistance, usually associated with obesity, occurs to varying degrees with different antipsychotics, although more comparative studies with direct assessment of resistance are

Reflux episodes and esophageal impedance levels in patients with typical and atypical symptoms of gastroesophageal reflux disease

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Ye, Bi Xing; Jiang, Liu Qin; Lin, Lin; Wang, Ying; Wang, Meifeng

2017-01-01

Abstract To determine the relationship between baseline impedance levels and gastroesophageal reflux, we retrospectively enrolled 110 patients (54 men and 56 female; mean age, 51 ± 14 years) with suspected gastroesophageal reflux disease (GERD) who underwent 24-h multichannel intraluminal impedance and pH monitoring. Patients were stratified according to symptom (typical or atypical) and reflux types (acid reflux, nonacid reflux [NAR], or no abnormal reflux). Mean nocturnal baseline impedance (MNBI) were measured 3 cm (distal esophagus) and 17 cm (proximal esophagus) above the lower esophageal sphincter. Median distal esophageal MNBI was lower in the acid reflux group (1244 Ω; 647–1969 Ω) than in the NAR (2586 Ω; 1368–3666 Ω) or no abnormal reflux groups (3082 Ω; 2495–4472 Ω; all P < .05). Distal MNBI were negatively correlated with DeMeester score and acid exposure time. Atypical symptoms were more frequently associated with NAR than typical symptoms (P < .01). Among patients with positive symptom-association probability (SAP) for NAR, median proximal MNBI tended to be lower in patients with typical symptoms (median, 3013 Ω; IQR, 2535–3410 Ω) than in those with atypical symptoms (median, 3386 Ω; IQR, 3044–3730 Ω, P = .05). Thus, atypical GERD symptoms were more likely to be associated with NAR. The mucosal integrity of the proximal esophagus might be relatively impaired in GERD patients with typical symptoms for NAR. PMID:28906377

Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

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I-Chun Lin

Full Text Available BACKGROUND: Kawasaki disease (KD of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE-induced coronary arteritis. METHODS: Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old were intraperitoneally injected with LCWE (1 mg/mL to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. RESULTS: Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL-2, IL-6, IL-10, monocyte chemoattractant protein (MCP-1, and tumor necrosis factor (TNF-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. CONCLUSION: This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by

Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

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Lin, I-Chun; Kuo, Ho-Chang; Lin, Ying-Jui; Wang, Feng-Shen; Wang, Lin; Huang, Shun-Chen; Chien, Shao-Ju; Huang, Chien-Fu; Wang, Chih-Lu; Yu, Hong-Ren; Chen, Rong-Fu; Yang, Kuender D

2012-01-01

Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2-mediated immune activation on CD14

Pattern of subcutaneous fat during follow-up of a cohort of North Indian children with Kawasaki disease: a preliminary study.

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Suthar, Renu; Singh, Surjit; Bhalla, Anil Kumar; Attri, Savita Verma

2014-03-01

Kawasaki disease (KD) has been associated with abnormal lipid profiles. The latter, in turn, have been linked to changes in subcutaneous fat. In this comparative cross-sectional study we have quantified distribution of subcutaneous fat during follow-up of a cohort of North Indian children with KD. We compared 35 KD children (at least 2 years after disease) and 33 healthy controls. Study parameters included weight, height and skinfold thickness (SFT) over biceps, triceps, midaxillary, subscapular, medial calf and suprailiac areas. Waist and hip circumferences were also recorded. All parameters were measured four times at 6-monthly intervals using standardized techniques. Serum lipids were assayed in the study group. Study children were enrolled 3.7 ± 2.5 years after KD and mean age at enrolment was 8.26 ± 3.65 years. Suprailiac SFT measured higher in boys with KD (P ≤ 0.05). Biceps SFT was higher in the study group, but the difference was not significant. Other SFT were not affected. Waist and hip circumference was higher in the study group than controls (P ≤ 0.05). Waist/hip circumference ratio was not affected. Serum low-density lipoprotein cholesterol (LDL-C) and triglycerides were higher in the study group as compared to historical controls (95.60 ± 36.12 and 129.40 ± 64.62 mg/dL vs. 80.10 ± 2.20 and 91.1 ± 29.85; P ≤ 0.05). Total cholesterol and high-density lipoprotein cholesterol levels remained unaffected. Children with KD (especially boys) had increased subcutaneous fat deposition in the suprailiac region and waist, during follow-up. Serum LDL-C and triglycerides were elevated. KD children may have a tendency to develop central obesity. Further studies, with longer follow-up, would be necessary to show whether this has implications for development of coronary artery disease later in life. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

Atypical manifestations of early syphilis

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R V Koranne

1990-01-01

Full Text Available A study of 36 untreated patients with early syphilis revealed atypical variations namely; long incubation period of 101 days in I patient, more than 3 chancres in 1, undermined margin of the chancre along with tenderness in 1 and moderate to severe tenderness of the ulcers in 2 cases. In 3 patients there was no indurations of the ulcers. Three patients with primary syphilis had unilateral lymphadenitis, and in I case the lymph nodes were not only tender but showed tendency towardsmatingawell. Insecondarysyphilis, 11 out of 16 patients having condylomata lata had no other muco-cutaneous lesions. Concomitant presence of other venereal disease to account for the atypical manifestations was discounted- by appropriate laboratory tests, response to therapeutic agents and follow up.

Building a Natural Language Processing Tool to Identify Patients With High Clinical Suspicion for Kawasaki Disease from Emergency Department Notes.

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Doan, Son; Maehara, Cleo K; Chaparro, Juan D; Lu, Sisi; Liu, Ruiling; Graham, Amanda; Berry, Erika; Hsu, Chun-Nan; Kanegaye, John T; Lloyd, David D; Ohno-Machado, Lucila; Burns, Jane C; Tremoulet, Adriana H

2016-05-01

Delayed diagnosis of Kawasaki disease (KD) may lead to serious cardiac complications. We sought to create and test the performance of a natural language processing (NLP) tool, the KD-NLP, in the identification of emergency department (ED) patients for whom the diagnosis of KD should be considered. We developed an NLP tool that recognizes the KD diagnostic criteria based on standard clinical terms and medical word usage using 22 pediatric ED notes augmented by Unified Medical Language System vocabulary. With high suspicion for KD defined as fever and three or more KD clinical signs, KD-NLP was applied to 253 ED notes from children ultimately diagnosed with either KD or another febrile illness. We evaluated KD-NLP performance against ED notes manually reviewed by clinicians and compared the results to a simple keyword search. KD-NLP identified high-suspicion patients with a sensitivity of 93.6% and specificity of 77.5% compared to notes manually reviewed by clinicians. The tool outperformed a simple keyword search (sensitivity = 41.0%; specificity = 76.3%). KD-NLP showed comparable performance to clinician manual chart review for identification of pediatric ED patients with a high suspicion for KD. This tool could be incorporated into the ED electronic health record system to alert providers to consider the diagnosis of KD. KD-NLP could serve as a model for decision support for other conditions in the ED. © 2016 by the Society for Academic Emergency Medicine.

Behaviors of chemical elements in the atmosphere, Kawasaki, Japan

International Nuclear Information System (INIS)

Oda, K.; Kikawada, Y.; Oi, T.

2008-01-01

A total of 19 elements in the samples of atmospheric deposition collected in Kawasaki, Japan, were determined by neutron activation analysis, ICPAES and flame photometry. The amounts of soil dust depositions were larger in springs and those of Sb and Zn depositions were larger in summers than in the other seasons. The values of the enrichment factors were higher for Sb and Zn than for the other elements determined throughout the sampling period. A factor analysis showed that the two elements were characterized as industrial components. Rubber products like tires that contain noncombustibles and rubber accelerators were a possible origin of high concentrations of Sb and Zn in the present samples. (author)

Immunohistochemical differentiation of atypical hyperplasia vs. carcinoma in situ of the breast.

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Masood, S; Sim, S J; Lu, L

1992-01-01

The distinction between atypical hyperplasia and carcinoma in situ in breast lesions can be difficult. The identification of myoepithelial cell layers may be helpful in establishing a diagnosis of proliferative breast disease vs. intraepithelial neoplasia. We reviewed pathologic material on 20 cases of atypical hyperplasia and 29 cases of carcinoma in situ. Immunohistochemical stains were employed against muscle-specific actin, S-100 protein, and cytokeratin to identify myoepithelial cells and to recognize different staining patterns. In atypical hyperplasia, muscle-specific actin staining identified myoepithelial cells in fine branching fibrovascular layers or as scattered cells between other proliferating cells. This pattern was absent in carcinoma in situ. S-100 protein showed more positive staining in atypical hyperplasia than in carcinoma in situ with patterns distinct from muscle-specific actin. Immunostaining for cytokeratin demonstrated distinctly different patterns between the two lesions. This study suggests that muscle-specific actin, S-100 protein, and cytokeratin in combination may assist in distinguishing proliferative breast disease with atypia from carcinoma in situ.

Differential processing of natural scenes in typical and atypical Alzheimer disease measured with a saccade choice task

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Muriel eBoucart

2014-07-01

Full Text Available Though atrophy of the medial temporal lobe, including structures (hippocampus and parahippocampal cortex that support scene perception and the binding of an object to its context, appears early in Alzheimer disease (AD few studies have investigated scene perception in people with AD. We assessed the ability to find a target object within a natural scene in people with typical AD and in people with atypical AD (posterior cortical atrophy. Pairs of colored photographs were displayed left and right of fixation for one second. Participants were asked to categorize the target (an animal either in moving their eyes toward the photograph containing the target (saccadic choice task or in pressing a key corresponding to the location of the target (manual choice task in separate blocks of trials. For both tasks performance was compared in two conditions: with isolated objects and with objects in scenes. Patients with atypical AD were more impaired to detect a target within a scene than people with typical AD who exhibited a pattern of performance more similar to that of age-matched controls in terms of accuracy, saccade latencies and benefit from contextual information. People with atypical AD benefited less from contextual information in both the saccade and the manual choice tasks suggesting a higher sensitivity to crowding and deficits in figure/ground segregation in people with lesions in posterior areas of the brain.

CD8+ T Cells Contribute to the Development of Coronary Arteritis in the Lactobacillus casei Cell Wall Extract-Induced Murine Model of Kawasaki Disease.

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Noval Rivas, Magali; Lee, Youngho; Wakita, Daiko; Chiba, Norika; Dagvadorj, Jargalsaikhan; Shimada, Kenichi; Chen, Shuang; Fishbein, Michael C; Lehman, Thomas J A; Crother, Timothy R; Arditi, Moshe

2017-02-01

Kawasaki disease (KD) is the leading cause of acquired heart disease among children in developed countries. Coronary lesions in KD in humans are characterized by an increased presence of infiltrating CD3+ T cells; however, the specific contributions of the different T cell subpopulations in coronary arteritis development remain unknown. Therefore, we sought to investigate the function of CD4+ and CD8+ T cells, Treg cells, and natural killer (NK) T cells in the pathogenesis of KD. We addressed the function of T cell subsets in KD development by using a well-established murine model of Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis. We determined which T cell subsets were required for development of KD vasculitis by using several knockout murine strains and depleting monoclonal antibodies. LCWE-injected mice developed coronary lesions characterized by the presence of inflammatory cell infiltrates. Frequently, this chronic inflammation resulted in complete occlusion of the coronary arteries due to luminal myofibroblast proliferation (LMP) as well as the development of coronary arteritis and aortitis. We found that CD8+ T cells, but not CD4+ T cells, NK T cells, or Treg cells, were required for development of KD vasculitis. The LCWE-induced murine model of KD vasculitis mimics many histologic features of the disease in humans, such as the presence of CD8+ T cells and LMP in coronary artery lesions as well as epicardial coronary arteritis. Moreover, CD8+ T cells functionally contribute to the development of KD vasculitis in this murine model. Therapeutic strategies targeting infiltrating CD8+ T cells might be useful in the management of KD in humans. © 2016, American College of Rheumatology.

Atypical teratoid rhabdoid tumor in a 65-year-old man presenting with disseminated leptomeningeal disease: A case report and review of the literature.

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Babi, Marc-Alain; Fecci, Peter; Luedke, Matthew; Pineda, Olinda; O'Keefe, Yasmin Ali

2018-01-01

Central nervous system atypical teratoid rhabdoid tumors are very rare aggressive tumor of childhood, primarily occurring at age of less than 3 years old. The prognosis of these tumors is very poor, with a reported median survival of 6-12 months in most cases. Treatment typically consists of aggressive chemotherapy and radiotherapy. We present the case of a 65-year-old man who presented with progressive encephalopathy and change in personality over 3 months period. The patient had further accelerated decline over 3 weeks. The diagnosis of atypical teratoid rhabdoid tumor initially remained elusive despite very extensive workup, but was eventually confirmed via open brain biopsy. To the best of our knowledge, this is the oldest reported case of atypical teratoid rhabdoid tumor in the literature. We further extend the spectrum of this rare disease.

Typical and atypical symptoms of gastro esophageal reflux disease: Does Helicobacter pylori infection matter?

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Grossi, Laurino; Ciccaglione, Antonio Francesco; Marzio, Leonardo

2015-11-06

To analyze whether the presence of Helicobacter pylori (H. pylori) infection could affect the quality of symptoms in gastro-esophageal reflux disease (GERD) patients. one hundred and forty-four consecutive patients referred to our Unit for suspected GERD were recruited for the study. All patients underwent esophageal pH-metric recording. For those with a positive test, C13 urea breath test was then performed to assess the H. pylori status. GERD patients were stratified according to the quality of their symptoms and classified as typical, if affected by heartburn and regurgitation, and atypical if complaining of chest pain, respiratory and ears, nose, and throat features. H. pylori-negative patients were also asked whether they had a previous diagnosis of H. pylori infection. If a positive response was given, on the basis of the time period after successful eradication, patients were considered as "eradicated" (E) if H. pylori eradication occurred more than six months earlier or "recently eradicated" if the therapy had been administered within the last six months. Patients without history of infection were identified as "negative" (N). χ (2) test was performed by combining the clinical aspects with the H. pylori status. one hundred and twenty-nine of the 144 patients, including 44 H. pylori-positive and 85 H. pylori-negative (41 negative, 21 recently eradicated, 23 eradicated more than 6 mo before), were eligible for the analysis. No difference has been found between H. pylori status and either the number of reflux episodes (138 ± 23 vs 146 ± 36, respectively, P = 0.2, not significant) or the percentage of time with pH values < 4 (6.8 ± 1.2 vs 7.4 ± 2.1, respectively, P = 0.3, not significant). The distribution of symptoms was as follows: 13 typical (30%) and 31 atypical (70%) among the 44 H. pylori-positive cases; 44 typical (52%) and 41 atypical (48%) among the 85 H. pylori-negative cases, (P = 0.017 vs H. pylori+; OR = 2.55, 95%CI: 1.17-5.55). Furthermore

Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

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Qiang Zhou

2011-01-01

Full Text Available We describe here a rare case of variant of mycosis fungoides (MF: ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG, IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

HMGB1 in vascular diseases : Its role in vascular inflammation and atherosclerosis

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de Souza, A. W. S.; Westra, J.; Limburg, P. C.; Bijl, M.; Kallenberg, C. G. M.

2012-01-01

The nuclear protein high mobility group box 1 (HMGB1) has been suggested to be involved in the pathogenesis of several vascular diseases such as systemic vasculitis and atherosclerosis. In systemic vasculitides including ANCA-associated vasculitis and Kawasaki disease, serum HMGB1 levels are higher

Technical features of steel structure construction by Kawasaki Steel; Kawasaki Seitetsu no kokozo gijutsu no tokucho

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Kaneko, T.; Urata, I.; Okata, S. [Kawasaki Steel Corp., Tokyo (Japan)

1996-03-01

In the steel structure technology of Kawasaki Steel, the joint technique (e.g., welding) is added to them while developing or improving the products that meet the social needs as a material supplier. Moreover, the execution technique that manufactures materials or constructs them as an integrated structure, the structural analysis that conforms to the function and application of a structure, and the design technique on dynamic properties or durability such as earthquake resistance, fatigue, and corrosion resistance are synthetically expanded for engineering. In this paper, a building steel frame, non-residence building, bridge, and harbor structure as steel structure in the building and construction fields were described for each structure genre. The structural technology of a building steel frame is summarized to the products of pillar materials. An earthquake brace, using a dead soft steel, with high earthquake energy absorption capability and a damping wall were also developed. The design and execution technique of a large roof was systematized. The exchange technique of a road bridge RC floor and the technique of an unstiffened suspension bridge for pipeline were developed. A new technique was also developed for a suspension monorail track and offshore structure. 30 refs., 5 figs.

Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.

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Shohei Ogata

Full Text Available Although intravenous immunoglobulin (IVIG is highly effective in Kawasaki disease (KD, mechanisms are not understood and 10-20% of patients are treatment-resistant, manifesting a higher rate of coronary artery aneurysms. Murine models suggest that α2-6-linked sialic acid (α2-6Sia content of IVIG is critical for suppressing inflammation. However, pro-inflammatory states also up-regulate endogenous levels of β-galactoside:α2-6 sialyltransferase-I (ST6Gal-I, the enzyme that catalyzes addition of α2-6Sias to N-glycans. We asked whether IVIG failures correlated with levels of α2-6Sia on infused IVIG or on the patient's own endogenous IgG.We quantified levels of α2-6Sia in infused IVIG and endogenous IgG from 10 IVIG-responsive and 10 resistant KD subjects using multiple approaches. Transcript levels of ST6GAL1, in patient whole blood and B cell lines were evaluated by RT-PCR. Plasma soluble (sST6Gal-I levels were measured by ELISA.There was no consistent difference in median sialylation levels of infused IVIG between groups. However, α2-6Sia levels in endogenous IgG, ST6GAL1 transcript levels, and ST6Gal-I protein in serum from IVIG-resistant KD subjects were lower than in responsive subjects at both pre-treatment and one-year time points (p <0.001, respectively.Our data indicate sialylation levels of therapeutic IVIG are unrelated to treatment response in KD. Rather, lower sialylation of endogenous IgG and lower blood levels of ST6GALI mRNA and ST6Gal-I enzyme predict therapy resistance. These differences were stable over time, suggesting a genetic basis. Because IVIG-resistance increases risk of coronary artery aneurysms, our findings have important implications for the identification and treatment of such individuals.

Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.

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Ogata, Shohei; Shimizu, Chisato; Franco, Alessandra; Touma, Ranim; Kanegaye, John T; Choudhury, Biswa P; Naidu, Natasha N; Kanda, Yutaka; Hoang, Long T; Hibberd, Martin L; Tremoulet, Adriana H; Varki, Ajit; Burns, Jane C

2013-01-01

Although intravenous immunoglobulin (IVIG) is highly effective in Kawasaki disease (KD), mechanisms are not understood and 10-20% of patients are treatment-resistant, manifesting a higher rate of coronary artery aneurysms. Murine models suggest that α2-6-linked sialic acid (α2-6Sia) content of IVIG is critical for suppressing inflammation. However, pro-inflammatory states also up-regulate endogenous levels of β-galactoside:α2-6 sialyltransferase-I (ST6Gal-I), the enzyme that catalyzes addition of α2-6Sias to N-glycans. We asked whether IVIG failures correlated with levels of α2-6Sia on infused IVIG or on the patient's own endogenous IgG. We quantified levels of α2-6Sia in infused IVIG and endogenous IgG from 10 IVIG-responsive and 10 resistant KD subjects using multiple approaches. Transcript levels of ST6GAL1, in patient whole blood and B cell lines were evaluated by RT-PCR. Plasma soluble (s)ST6Gal-I levels were measured by ELISA. There was no consistent difference in median sialylation levels of infused IVIG between groups. However, α2-6Sia levels in endogenous IgG, ST6GAL1 transcript levels, and ST6Gal-I protein in serum from IVIG-resistant KD subjects were lower than in responsive subjects at both pre-treatment and one-year time points (p treatment response in KD. Rather, lower sialylation of endogenous IgG and lower blood levels of ST6GALI mRNA and ST6Gal-I enzyme predict therapy resistance. These differences were stable over time, suggesting a genetic basis. Because IVIG-resistance increases risk of coronary artery aneurysms, our findings have important implications for the identification and treatment of such individuals.

[Facial diplegia with atypical paresthesia. A variant of Guillain-Barré syndrome].

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Dal Verme, Agustín; Acosta, Paula; Margan, Mercedes; Pagnini, Cecilia; Dellepiane, Eugenia; Peralta, Christian

2015-01-01

Guillain-Barré syndrome is an acute demyelinating disease which presents in a classic form with muscular weakness and the lack of reflexes. There are multiple variations and atypical forms of the disease, being facial diplegia with paresthesia one of them. Also, the absence of reflexes in this syndrome is typical but not constant, since 10% of patients present reflexes. We describe a case of atypical presentation with bilateral facial palsy, paresthesia, brisk reflexes and weakness in the lower limbs in a 33 year old woman.

Cushing disease revealed by bilateral atypical central serous chorioretinopathy: case report.

Science.gov (United States)

Giovansili, Iama; Belange, Georeges; Affortit, Aude

2013-01-01

We report the case of a patient with Cushing disease revealed by bilateral central serous chorioretinopathy (CSCR). We present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year-old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed. A 53-year-old patient initially presented to the Department of Ophthalmology with a 4-week history of decreased vision in the left eye. Standard ophthalmologic examination and fluorescein angiography established the diagnosis of bilateral CSCR. Systemic clinical signs and biochemical analysis indicated hypercortisolism. Magnetic resonance imaging (MRI) of the pituitary gland showed a left-side lesion compatible with a microadenoma. The diagnosis of Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome secondary to a pituitary microadenoma was selected. Endoscopic endonasal transsphenoidal surgery was performed and the pituitary adenoma was successfully removed. The histology confirmed the presence of ACTH-immunopositive pituitary adenoma. Early postoperative morning cortisol levels indicated early remission. At 6 weeks postoperatively, the patient's morning cortisol remains undetectable, and serous retinal detachments had regressed. CSCR is an uncommon manifestation of endogenous Cushing syndrome. It can be the first presentation of hypercortisolism caused by Cushing disease. CSCR should be considered when assessing patients with Cushing syndrome complaining of visual disorders. On the other hand, it is useful in patients with an atypical form of CSCR to exclude Cushing's syndrome.

[Atypical subacute thyroiditis in combination with Grave's disease:Diagnostic difficulties in a case report].

Science.gov (United States)

Koutouridou, Emmanouela; Planck, Tereza; Uddman, Erik; Lantz, Mikael

2018-04-13

Subacute thyroiditis is a common inflammatory disorder of the thyroid gland, possibly of viral etiology, that typically presents with neck pain, fever and tenderness on palpation of the thyroid gland. Graves' disease is an autoimmune thyroid disorder caused by stimulation of the thyroid gland by thyrotropin receptor antibodies (TRAb). The development of Graves´ disease and subacute thyroiditis simultaneously is an uncommon condition and only a few cases have been reported. In this article we present a case of a 46-year old woman diagnosed with Graves´ disease who was started on thiamazole and weeks later developed high fever. Several differential diagnoses were considered such as infection, lymphoma and vasculitis due to thiamazole. Finally, the fine needle aspiration of the thyroid gland displayed histopathological features of subacute thyroiditis. Remarkably, our patient did not have neck pain or tenderness on palpation of the thyroid gland and overall the clinical presentation of subacute thyroiditis was atypical. Thus, subacute thyroiditis may be considered as a potential cause of fever of unknown origin.

An Atypical Case of Pityriasis Rosea Gigantea after Influenza Vaccination

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Dimitrios Papakostas

2014-04-01

Full Text Available Pityriasis rosea is a common erythematosquamous eruption, typically presenting along the cleavage lines of the skin. A wide spectrum of atypical manifestations may challenge even the most experienced physician. Here we report a rare case of a suberythrodermic pityriasis rosea with gigantic plaques after an influenza vaccination, and we discuss the possible triggers of atypical manifestations of such a common dermatological disease in the setting of an altered immunity.

The differences in esophageal motility and its clinical significance between the patients with typical and atypical symptoms of non-erosive reflux disease

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Ling LIN

2016-01-01

Full Text Available Objective 　To study the characteristics of esophageal motility in the patients with different symptoms of nonerosive reflux disease (non-erosive reflux disease, NERD. Methods 　Eighty-six patients with NERD who visited the Department of Gastroenterology in our hospital from August 2012 to December 2014 were selected. They were all evaluated with upper gastrointestinal endoscopy and high resolution esophageal manometry. Those who had neither definite esophageal mucosal erosion nor Barrett's esophagus at endoscopy were diagnosed as NERD. According to the presence or absence of typical acid reflux and/or heartburn symptoms, patients with NERD were divided into two groups: the typical symptom group (47 cases, and the atypical symptom group (39 cases. High resolution measurement of esophageal pressure was used to evaluate the differences in esophageal dynamic characteristics between patients with different symptoms of NERD. The final results were analyzed with t test and chi-square test. Results 　Compared with atypical symptom group, both the resting pressure (20.68±1.64mmHg vs 15.79±1.21mmHg respectively, P0.05. Conclusion 　There is a difference in esophageal motility characteristics between the patients with typical and atypical symptoms of NERD, and the main differences are changes in the resting pressure and residual pressure of LES. DOI: 10.11855/j.issn.0577-7402.2015.12.12

Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein.

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Wilson, Rona; Plinston, Chris; Hunter, Nora; Casalone, Cristina; Corona, Cristiano; Tagliavini, Fabrizio; Suardi, Silvia; Ruggerone, Margherita; Moda, Fabio; Graziano, Silvia; Sbriccoli, Marco; Cardone, Franco; Pocchiari, Maurizio; Ingrosso, Loredana; Baron, Thierry; Richt, Juergen; Andreoletti, Olivier; Simmons, Marion; Lockey, Richard; Manson, Jean C; Barron, Rona M

2012-07-01

The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans.

Ophthalmologic complications of systemic disease.

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Klig, Jean E

2008-02-01

The human eye, as an organ, can offer critical clues to the presence of systemic disease. This article discusses the various ophthalmologic manifestations of systemic disease that can be evident on examination by an emergency department provider, as well as some findings that can be discerned with specialty consultation. The following topics are reviewed with respect to potential ocular signs and complications: syphilis, herpes zoster, Lyme disease, acquired immunodeficiency syndrome, Reiter's syndrome, Kawasaki's disease, temporal arteritis, endocarditis, hypertension, and diabetes mellitus. Indications for emergent ophthalmologic consultation are also emphasized.

Unusual infectious mononucleosis complicated by vasculitis

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Srimanta Kumar Sahu

2016-01-01

Full Text Available Infectious mononucleosis (IM is a clinical syndrome caused by Epstein-Barr virus (EBV. It manifests as fever, pharyngitis, malaise, adenopathy, and atypical lymphocytosis. Cardiovascular complications are thought to be rare in IM. There are very few case reports of EBV-associated vasculitides, like Kawasaki disease and systemic polyarteritis nodosa, however, involvement of the large caliber arteries like the aorta and its branches have been reported only scarcely. Myocarditis also is rare as an early manifestation of EBV infection. We present here a rare case of IM, presented initially with acute myocarditis and later with large-vessels arteritis.

Replication and meta-analysis of GWAS identified susceptibility loci in Kawasaki disease confirm the importance of B lymphoid tyrosine kinase (BLK in disease susceptibility.

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Chia-Jung Chang

Full Text Available The BLK and CD40 loci have been associated with Kawasaki disease (KD in two genome-wide association studies (GWAS conducted in a Taiwanese population of Han Chinese ancestry (Taiwanese and in Japanese cohorts. Here we build on these findings with replication studies of the BLK and CD40 loci in populations of Korean and European descent. The BLK region was significantly associated with KD susceptibility in both populations. Within the BLK gene the rs2736340-located linkage disequilibrium (LD comprising the promoter and first intron was strongly associated with KD, with the combined results of Asian studies including Taiwanese, Japanese, and Korean populations (2,539 KD patients and 7,021 controls providing very compelling evidence of association (rs2736340, OR = 1.498, 1.354-1.657; P = 4.74×10(-31. We determined the percentage of B cells present in the peripheral blood mononuclear cell (PBMC population and the expression of BLK in the peripheral blood leukocytes (leukocytes of KD patients during the acute and convalescent stages. The percentage of B cells in the PBMC population and the expression of BLK in leukocytes were induced in patients in the acute stage of KD. In B cell lines derived from KD patients, and in purified B cells from KD patients obtained during the acute stage, those with the risk allele of rs2736340 expressed significantly lower levels of BLK. These results suggest that peripheral B cells play a pathogenic role during the acute stage of KD. Decreased BLK expression in peripheral blood B cells may alter B cell function and predispose individuals to KD. These associative data suggest a role for B cells during acute KD. Understanding the functional implications may facilitate the development of B cell-mediated therapy for KD.

Primary lateral sclerosis mimicking atypical parkinsonism

DEFF Research Database (Denmark)

Norlinah, Ibrahim M; Bhatia, Kailash P; Østergaard, Karen

2007-01-01

of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa-unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67......Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one...... in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features...

Atypical pyoderma gangrenosum mimicking an infectious process.

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To, Derek; Wong, Aaron; Montessori, Valentina

2014-01-01

We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

Occurrence and significance of atypical Aeromonas salmonicida in non-salmonid and salmonid fish species : A review

DEFF Research Database (Denmark)

Wiklund, T.; Dalsgaard, Inger

1998-01-01

, non-salmonids as well as salmonids, inhabiting fresh water, brackish water and marine environments in northern and central Europe, South Africa, North America, Japan and Australia. In non-salmonid fish species, infections with atypical strains often manifest themselves as superficial skin ulcerations...... information is available about the ecology, spread and survival of atypical strains in water. The commonly used therapeutic methods for the control of diseases in farmed fish caused by atypical A. salmonicida are generally effective against the atypical strains. Resistance to different antibiotics...

Atypical Manifestations of Hyperthyroidism

Science.gov (United States)

Boxall, E. A.; Lauener, R. W.; McIntosh, H. W.

1964-01-01

Patients with hyperthyroidism usually present with symptoms of hypermetabolism with or without goitre and/or eye signs. Occasionally, however, the chief complaints are not immediately suggestive of hyperthyroidism. Patients with hyperthyroidism are described who presented with such atypical manifestations as periodic muscular paralysis, myasthenia, myopathy, encephalopathy, psychosis, angina pectoris, atrial fibrillation, heart failure without underlying heart disease, skeletal demineralization, pretibial myxedema, unilateral eye signs, and pitting edema of the ankles. ImagesFig. 2Fig. 3Fig. 5Fig. 7Fig. 8Fig. 9Fig. 10 PMID:14178405

Atypical myopathy in Denmark confirmed with the aTRAQ assay

DEFF Research Database (Denmark)

Høffer, Sofie Esbjørn; Votion, Dominique-Marie; Anderberg, Marie

2016-01-01

Atypical myopathy is a severe form of rhabdomyolysis that occurs in grazing horses. Over the past decades, the disease has been emerging in Europe. The disease is widespread in Europe and has been suspected in Denmark since 2000, yet no cases have been confirmed. The objective of this study...

Atypical Neuroleptic Malignant Syndrome Associated with Use of Clozapine

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Quevedo-Florez Leonardo

2017-01-01

Full Text Available The Neuroleptic Malignant Syndrome (NMS is a medical emergency of infrequent presentation in the emergency department, which is associated with the use of psychiatric drugs, such as typical and atypical antipsychotics. Our case addresses a 55-year-old patient diagnosed with undifferentiated schizophrenia for 10 years, who had been receiving clozapine and clonazepam as part of their treatment. This patient presents the symptoms of Neuroleptic Malignant Syndrome without fever, which improves with treatment especially with the withdrawal of clozapine. In the absence of fever and clinical improvement, the patient is considered to have an atypical presentation of this disease.

Atypical Pyoderma Gangrenosum Mimicking an Infectious Process

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Derek To

2014-01-01

Full Text Available We present a patient with atypical pyoderma gangrenosum (APG, which involved the patient’s arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

Early Freezing of Gait: Atypical versus Typical Parkinson Disorders

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Abraham Lieberman

2015-01-01

Full Text Available In 18 months, 850 patients were referred to Muhammad Ali Parkinson Center (MAPC. Among them, 810 patients had typical Parkinson disease (PD and 212 had PD for ≤5 years. Among the 212 patients with early PD, 27 (12.7% had freezing of gait (FOG. Forty of the 850 had atypical parkinsonism. Among these 40 patients, all of whom had symptoms for ≤5 years, 12 (30.0% had FOG. FOG improved with levodopa in 21/27 patients with typical PD but did not improve in the 12 patients with atypical parkinsonism. FOG was associated with falls in both groups of patients. We believe that FOG unresponsive to levodopa in typical PD resembles FOG in atypical parkinsonism. We thus compared the 6 typical PD patients with FOG unresponsive to levodopa plus the 12 patients with atypical parkinsonism with the 21 patients with typical PD responsive to levodopa. We compared them by tests of locomotion and postural stability. Among the patients with FOG unresponsive to levodopa, postural stability was more impaired than locomotion. This finding leads us to believe that, in these patients, postural stability, not locomotion, is the principal problem underlying FOG.

Widened coronary arteries in a feverish child

NARCIS (Netherlands)

van Doorn, H. Rogier; Lo-A-Njoe, Shirley M.; Ottenkamp, Jaap; Pajkrt, Dasja

2006-01-01

A 3-year-old girl with fever of unknown origin after a visit to Surinam was seen at our hospital. Signs and symptoms were indicative of either Kawasaki syndrome or an acute viral or (atypical) bacterial illness. No cardiac abnormalities were noted at echocardiography. She was treated with

Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease

OpenAIRE

Kasuya, Shusuke; Suzuki, Masayo; Inaoka, Tsutomu; Odashima, Masayuki; Nakatsuka, Tomoya; Ishikawa, Rumiko; Tokuyama, Wataru; Terada, Hitoshi

2016-01-01

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Approximately 50% of patients with AFD may have cardiac involvement. Gadolinium-enhanced cardiac magnetic resonance (CMR) is useful for the diagnosis of cardiac involvement of AFD by recognizing typical late gadolinium enhancement (LGE) patterns. We report a 48-year-old man with cardiac involvement in classical AFD, showing atypical distribution of the LGE at the mid-lateral...

Skeletal and muscular status in juveniles with GFD treated clinical and newly diagnosed atypical celiac disease--preliminary data.

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Płudowski, Paweł; Karczmarewicz, Elzbieta; Socha, Jerzy; Matusik, Halina; Syczewska, Małgorzata; Lorenc, Roman S

2007-01-01

Undiagnosed and untreated celiac disease (CD) constitutes an increasing skeletal health problem due to its association with low bone density and fractures. Examinations of skeletal status in children using dual-energy X-ray absorptiometry (DXA) are prone to size-related misinterpretation. In contrary, the analysis of muscle-bone relationship seems to limit a possibility of misdiagnosis because skeletal status is evaluated from the functional perspective. The study was aimed to assess skeletal status of children suffering from CD with the use of muscle-bone functional algorithm. The study group comprised 29 celiac patients (13.7yr+/-2.9) on gluten-free diet (GFD), and 24 newly diagnosed atypical celiac patients, including subgroup with normal height (n=14; 12.6yr+/-3.9) and subgroup with short stature (n=10; 12.2yr+/-2.9). Muscular and skeletal status was evaluated by DXA (DPX-L, GE). Anthropometry, total body bone mineral density (TBBMD, g/cm(2)). and total body bone mineral content (TBBMC, g) as well as lean body mass (LBM, g) were evaluated. Muscle-bone interactions were estimated using TBBMC/LBM ratio. Previously established references for healthy controls were used for the calculation of Z-scores (age-matched) and SD-scores (height-matched). GFD treated celiacs and atypical celiacs with normal body height had TBBMD, TBBMC, LBM, and TBBMC/LBM ratio Z-scores and SD-scores within normal range for healthy controls. In contrary, atypical celiacs with short stature had significantly lower Z-scores for TBBMD (-2.3+/-0.4), TBBMC (-2.1+/-0.3), LBM (-1.4+/-0.3). and TBBMC/LBM ratio (-2.3+/-0.6) when compared to respective values observed in GFD treated celiacs (pnormal height (pvalues observed in GFD treated celiacs (+0.04+/-0.2; pnormal height (-0.4+/-0.2; pvalues of DXA assessed indicators of bone and muscle status as well as normal muscle-bone interactions. Untreated atypical celiacs may present a broad spectrum of heterogeneous abnormalities from normal to markedly

Atypical Rocky Mountain spotted fever with polyarticular arthritis.

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Chaudhry, Muhammad A; Scofield, Robert Hal

2013-11-01

Rocky Mountain spotted fever (RMSF) is an acute, serious tick borne illness caused by Rickettsia rickettsi. Frequently, RMSF is manifested by headache, a typical rash and fever but atypical disease is common, making diagnosis difficult. Inflammatory arthritis as a manifestation is rare. The purpose of this study is to describe a patient with serologically proven RMSF who presented in an atypical manner with inflammatory arthritis of the small joints of the hands and to review the previously reported patients with rickettsial infection and inflammatory arthritis. An 18-year-old woman presented with a rash that began on the distal extremities and spread centrally, along with hand pain and swelling. She had tenderness and swelling of the metacarpophlangeal joints on examination in addition to an erythematosus macular rash and occasional fever. Acute and convalescent serology demonstrated R rickettsi infection. She was successfully treated with doxycycline. Inflammatory arthritis is a rare manifestation of RMSF or other rickettsial infection with 8 previously reported patients, only 1 of whom had RMSF. Physician must have a high index of suspicion for RMSF because of atypical presentations.

Discovering disease associations by integrating electronic clinical data and medical literature.

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Antony B Holmes

Full Text Available Electronic health record (EHR systems offer an exceptional opportunity for studying many diseases and their associated medical conditions within a population. The increasing number of clinical record entries that have become available electronically provides access to rich, large sets of patients' longitudinal medical information. By integrating and comparing relations found in the EHRs with those already reported in the literature, we are able to verify existing and to identify rare or novel associations. Of particular interest is the identification of rare disease co-morbidities, where the small numbers of diagnosed patients make robust statistical analysis difficult. Here, we introduce ADAMS, an Application for Discovering Disease Associations using Multiple Sources, which contains various statistical and language processing operations. We apply ADAMS to the New York-Presbyterian Hospital's EHR to combine the information from the relational diagnosis tables and textual discharge summaries with those from PubMed and Wikipedia in order to investigate the co-morbidities of the rare diseases Kaposi sarcoma, toxoplasmosis, and Kawasaki disease. In addition to finding well-known characteristics of diseases, ADAMS can identify rare or previously unreported associations. In particular, we report a statistically significant association between Kawasaki disease and diagnosis of autistic disorder.

Plasma Biomarkers Differentiate Parkinson’s Disease From Atypical Parkinsonism Syndromes

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Chin-Hsien Lin

2018-04-01

Full Text Available Objective: Parkinson’s disease (PD has significant clinical overlaps with atypical parkinsonism syndromes (APS, which have a poorer treatment response and a more aggressive course than PD. We aimed to identify plasma biomarkers to differentiate PD from APS.Methods: Plasma samples (n = 204 were obtained from healthy controls and from patients with PD, dementia with Lewy bodies (DLB, multiple system atrophy, progressive supranuclear palsy (PSP, corticobasal degeneration (CBD, or frontotemporal dementia (FTD with parkinsonism (FTD-P or without parkinsonism. We measured plasma levels of α-synuclein, total tau, p-Tau181, and amyloid beta 42 (Aβ42 by immunomagnetic reduction-based immunoassay.Results: Plasma α-synuclein level was significantly increased in patients with PD and APS when compared with controls and FTD without parkinsonism (p < 0.01. Total tau and p-Tau181 were significantly increased in all disease groups compared to controls, especially in patients with FTD (p < 0.01. A multivariate and receiver operating characteristic curve analysis revealed that a cut-off value for Aβ42 multiplied by p-Tau181 for discriminating patients with FTD from patients with PD and APS was 92.66 (pg/ml2, with an area under the curve (AUC of 0.932. An α-synuclein cut-off of 0.1977 pg/ml could separate FTD-P from FTD without parkinsonism (AUC 0.947. In patients with predominant parkinsonism, an α-synuclein cut-off of 1.388 pg/ml differentiated patients with PD from those with APS (AUC 0.87.Conclusion: Our results suggest that integrated plasma biomarkers improve the differential diagnosis of PD from APS (PSP, CBD, DLB, and FTD-P.

Realizing CO2 emission reduction through industrial symbiosis: A cement production case study for Kawasaki

OpenAIRE

Hashimoto, Shizuka; Fujita, Tsuyoshi; Geng, Yong; Nagasawa, Emiri

2010-01-01

This article is one effort to examine the present and potential performances of CO2 emission reduction though industrial symbiosis by employing a case study approach and life cycle CO2 analysis for alternative industrial symbiosis scenarios. As one of the first and the best-known eco-town projects, Kawasaki Eco-town was chosen as a case study area. First, the current industrial symbiosis practices in this area are introduced. To evaluate the potential of reducing the total CO2 emission throug...

Reassessment of carotid intima-media thickness by standard deviation score in children and adolescents after Kawasaki disease.

Science.gov (United States)

Noto, Nobutaka; Kato, Masataka; Abe, Yuriko; Kamiyama, Hiroshi; Karasawa, Kensuke; Ayusawa, Mamoru; Takahashi, Shori

2015-01-01

Previous studies that used carotid ultrasound have been largely conflicting in regards to whether or not patients after Kawasaki disease (KD) have a greater carotid intima-media thickness (CIMT) than controls. To test the hypothesis that there are significant differences between the values of CIMT expressed as absolute values and standard deviation scores (SDS) in children and adolescents after KD and controls, we reviewed 12 published articles regarding CIMT on KD patients and controls. The mean ± SD of absolute CIMT (mm) in the KD patients and controls obtained from each article was transformed to SDS (CIMT-SDS) using age-specific reference values established by Jourdan et al. (J: n = 247) and our own data (N: n = 175), and the results among these 12 articles were compared between the two groups and the references for comparison of racial disparities. There were no significant differences in mean absolute CIMT and mean CIMT-SDS for J between KD patients and controls (0.46 ± 0.06 mm vs. 0.44 ± 0.04 mm, p = 0.133, and 1.80 ± 0.84 vs. 1.25 ± 0.12, p = 0.159, respectively). However, there were significant differences in mean CIMT-SDS for N between KD patients and controls (0.60 ± 0.71 vs. 0.01 ± 0.65, p = 0.042). When we assessed the nine articles on Asian subjects, the difference of CIMT-SDS between the two groups was invariably significant only for N (p = 0.015). Compared with the reference values, CIMT-SDS of controls was within the normal range at a rate of 41.6 % for J and 91.6 % for N. These results indicate that age- and race-specific reference values for CIMT are mandatory for performing accurate assessment of the vascular status in healthy children and adolescents, particularly in those after KD considered at increased long-term cardiovascular risk.

The extracellular domain of myelin oligodendrocyte glycoprotein elicits atypical experimental autoimmune encephalomyelitis in rat and Macaque species.

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Alan D Curtis

Full Text Available Atypical models of experimental autoimmune encephalomyelitis (EAE are advantageous in that the heterogeneity of clinical signs appears more reflective of those in multiple sclerosis (MS. Conversely, models of classical EAE feature stereotypic progression of an ascending flaccid paralysis that is not a characteristic of MS. The study of atypical EAE however has been limited due to the relative lack of suitable models that feature reliable disease incidence and severity, excepting mice deficient in gamma-interferon signaling pathways. In this study, atypical EAE was induced in Lewis rats, and a related approach was effective for induction of an unusual neurologic syndrome in a cynomolgus macaque. Lewis rats were immunized with the rat immunoglobulin variable (IgV-related extracellular domain of myelin oligodendrocyte glycoprotein (IgV-MOG in complete Freund's adjuvant (CFA followed by one or more injections of rat IgV-MOG in incomplete Freund's adjuvant (IFA. The resulting disease was marked by torticollis, unilateral rigid paralysis, forelimb weakness, and high titers of anti-MOG antibody against conformational epitopes of MOG, as well as other signs of atypical EAE. A similar strategy elicited a distinct atypical form of EAE in a cynomolgus macaque. By day 36 in the monkey, titers of IgG against conformational epitopes of extracellular MOG were evident, and on day 201, the macaque had an abrupt onset of an unusual form of EAE that included a pronounced arousal-dependent, transient myotonia. The disease persisted for 6-7 weeks and was marked by a gradual, consistent improvement and an eventual full recovery without recurrence. These data indicate that one or more boosters of IgV-MOG in IFA represent a key variable for induction of atypical or unusual forms of EAE in rat and Macaca species. These studies also reveal a close correlation between humoral immunity against conformational epitopes of MOG, extended confluent demyelinating plaques in

The extracellular domain of myelin oligodendrocyte glycoprotein elicits atypical experimental autoimmune encephalomyelitis in rat and Macaque species.

Science.gov (United States)

Curtis, Alan D; Taslim, Najla; Reece, Shaun P; Grebenciucova, Elena; Ray, Richard H; Rosenbaum, Matthew D; Wardle, Robert L; Van Scott, Michael R; Mannie, Mark D

2014-01-01

Atypical models of experimental autoimmune encephalomyelitis (EAE) are advantageous in that the heterogeneity of clinical signs appears more reflective of those in multiple sclerosis (MS). Conversely, models of classical EAE feature stereotypic progression of an ascending flaccid paralysis that is not a characteristic of MS. The study of atypical EAE however has been limited due to the relative lack of suitable models that feature reliable disease incidence and severity, excepting mice deficient in gamma-interferon signaling pathways. In this study, atypical EAE was induced in Lewis rats, and a related approach was effective for induction of an unusual neurologic syndrome in a cynomolgus macaque. Lewis rats were immunized with the rat immunoglobulin variable (IgV)-related extracellular domain of myelin oligodendrocyte glycoprotein (IgV-MOG) in complete Freund's adjuvant (CFA) followed by one or more injections of rat IgV-MOG in incomplete Freund's adjuvant (IFA). The resulting disease was marked by torticollis, unilateral rigid paralysis, forelimb weakness, and high titers of anti-MOG antibody against conformational epitopes of MOG, as well as other signs of atypical EAE. A similar strategy elicited a distinct atypical form of EAE in a cynomolgus macaque. By day 36 in the monkey, titers of IgG against conformational epitopes of extracellular MOG were evident, and on day 201, the macaque had an abrupt onset of an unusual form of EAE that included a pronounced arousal-dependent, transient myotonia. The disease persisted for 6-7 weeks and was marked by a gradual, consistent improvement and an eventual full recovery without recurrence. These data indicate that one or more boosters of IgV-MOG in IFA represent a key variable for induction of atypical or unusual forms of EAE in rat and Macaca species. These studies also reveal a close correlation between humoral immunity against conformational epitopes of MOG, extended confluent demyelinating plaques in spinal cord and

Atypical memory B cells in human chronic infectious diseases: An interim report.

Science.gov (United States)

Portugal, Silvia; Obeng-Adjei, Nyamekye; Moir, Susan; Crompton, Peter D; Pierce, Susan K

2017-11-01

Immunological memory is a remarkable phenomenon in which survival of an initial infection by a pathogen leads to life-long protection from disease upon subsequent exposure to that same pathogen. For many infectious diseases, long-lived protective humoral immunity is induced after only a single infection in a process that depends on the generation of memory B cells (MBCs) and long-lived plasma cells. However, over the past decade it has become increasingly evident that many chronic human infectious diseases to which immunity is not readily established, including HIV-AIDS, malaria and TB, are associated with fundamental alterations in the composition and functionality of MBC compartments. A common feature of these diseases appears to be a large expansion of what have been termed exhausted B cells, tissue-like memory B cells or atypical memory B cells (aMBCs) that, for simplicity's sake, we refer to here as aMBCs. It has been suggested that chronic immune activation and inflammation drive the expansion of aMBCs and that in some way aMBCs contribute to deficiencies in the acquisition of immunity in chronic infectious diseases. Although aMBCs are heterogeneous both within individuals and between diseases, they have several features in common including low expression of the cell surface markers that define classical MBCs in humans including CD21 and CD27 and high expression of genes not usually expressed by classical MBCs including T-bet, CD11c and a variety of inhibitory receptors, notably members of the FcRL family. Another distinguishing feature is their greatly diminished ability to be stimulated through their B cell receptors to proliferate, secrete cytokines or produce antibodies. In this review, we describe our current understanding of the phenotypic markers of aMBCs, their specificity in relation to the disease-causing pathogen, their functionality, the drivers of their expansion in chronic infections and their life span. We briefly summarize the features of a

Identification of a New Cotton Disease Caused by an Atypical Cotton Leafroll Dwarf Virus in Argentina.

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Agrofoglio, Yamila C; Delfosse, Verónica C; Casse, María F; Hopp, Horacio E; Kresic, Iván Bonacic; Distéfano, Ana J

2017-03-01

An outbreak of a new disease occurred in cotton (Gossypium hirsutum) fields in northwest Argentina starting in the 2009-10 growing season and is still spreading steadily. The characteristic symptoms of the disease included slight leaf rolling and a bushy phenotype in the upper part of the plant. In this study, we determined the complete nucleotide sequences of two independent virus genomes isolated from cotton blue disease (CBD)-resistant and -susceptible cotton varieties. This virus genome comprised 5,866 nucleotides with an organization similar to that of the genus Polerovirus and was closely related to cotton leafroll dwarf virus, with protein identity ranging from 88 to 98%. The virus was subsequently transmitted to a CBD-resistant cotton variety using Aphis gossypii and symptoms were successfully reproduced. To study the persistence of the virus, we analyzed symptomatic plants from CBD-resistant varieties from different cotton-growing fields between 2013 and 2015 and showed the presence of the same virus strain. In addition, a constructed full-length infectious cDNA clone from the virus caused disease symptoms in systemic leaves of CBD-resistant cotton plants. Altogether, the new leafroll disease in CBD-resistant cotton plants is caused by an atypical cotton leafroll dwarf virus.

Herpes zoster - typical and atypical presentations.

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Dayan, Roy Rafael; Peleg, Roni

2017-08-01

Varicella- zoster virus infection is an intriguing medical entity that involves many medical specialties including infectious diseases, immunology, dermatology, and neurology. It can affect patients from early childhood to old age. Its treatment requires expertise in pain management and psychological support. While varicella is caused by acute viremia, herpes zoster occurs after the dormant viral infection, involving the cranial nerve or sensory root ganglia, is re-activated and spreads orthodromically from the ganglion, via the sensory nerve root, to the innervated target tissue (skin, cornea, auditory canal, etc.). Typically, a single dermatome is involved, although two or three adjacent dermatomes may be affected. The lesions usually do not cross the midline. Herpes zoster can also present with unique or atypical clinical manifestations, such as glioma, zoster sine herpete and bilateral herpes zoster, which can be a challenging diagnosis even for experienced physicians. We discuss the epidemiology, pathophysiology, diagnosis and management of Herpes Zoster, typical and atypical presentations.

Experimental transmission of atypical scrapie to sheep

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Spiropoulos John

2007-08-01

Full Text Available Abstract Background Active surveillance for transmissible spongiform encephalopathies in small ruminants has been an EU regulatory requirement since 2002. A number of European countries have subsequently reported cases of atypical scrapie, similar to previously published cases from Norway, which have pathological and molecular features distinct from classical scrapie. Most cases have occurred singly in flocks, associated with genotypes considered to be more resistant to classical disease. Experimental transmissibility of such isolates has been reported in certain ovinised transgenic mice, but has not previously been reported in the natural host. Information on the transmissibility of this agent is vital to ensuring that disease control measures are effective and proportionate. Results This report presents the successful experimental transmission, in 378 days, of atypical scrapie to a recipient sheep of homologous genotype with preservation of the pathological and molecular characteristics of the donor. This isolate also transmitted to ovinised transgenic mice (Tg338 with a murine phenotype indistinguishable from that of Nor 98. Conclusion This result strengthens the opinion that these cases result from a distinct strain of scrapie agent, which is potentially transmissible in the natural host under field conditions.

Homozygous TREM2 mutation in a family with atypical frontotemporal dementia.

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Le Ber, Isabelle; De Septenville, Anne; Guerreiro, Rita; Bras, José; Camuzat, Agnès; Caroppo, Paola; Lattante, Serena; Couarch, Philippe; Kabashi, Edor; Bouya-Ahmed, Kawtar; Dubois, Bruno; Brice, Alexis

2014-10-01

TREM2 mutations were first identified in Nasu-Hakola disease, a rare autosomal recessive disease characterized by recurrent fractures because of bone cysts and presenile dementia. Recently, homozygous and compound heterozygous TREM2 mutations were identified in rare families with frontotemporal lobar degeneration (FTLD) but without bone involvement. We identified a p.Thr66Met heterozygous mutation in a new consanguineous Italian family. Two sibs had early onset autosomal recessive FTLD without severe bone disorders. Atypical signs were present in this family: early parietal and hippocampus involvement, parkinsonism, epilepsy, and corpus callosum thickness on brain magnetic resonance imaging. This study further demonstrates the implication of TREM2 mutations in FTLD phenotypes. It illustrates the variability of bone phenotype and underlines the frequency of atypical signs in TREM2 carriers. This and previous studies evidence that TREM2 mutation screening should be limited to autosomal recessive FTLD with atypical phenotypes characterized by: (1) a very young age at onset (20-50 years); (2) early parietal and hippocampal deficits; (3) the presence of seizures and parkinsonism; (4) suggestive extensive white matter lesions and corpus callosum thickness on brain magnetic resonance imaging. Copyright © 2014 Elsevier Inc. All rights reserved.

Uremic Encephalopathy with Atypical Magnetic Resonance Features on Diffusion-Weighted Images

International Nuclear Information System (INIS)

Kang, Eu Gene; Jeon, Se Jeong; Choi, See Sung

2012-01-01

Uremic encephalopathy is a well-known disease with typical MR findings including bilateral vasogenic or cytotoxic edema at the cerebral cortex or basal ganglia. Involvement of the basal ganglia has been very rarely reported, typically occurring in uremic-diabetic patients. We recently treated a patient who had non-diabetic uremic encephalopathy with an atypical lesion distribution involving the supratentorial white matter, without cortical or basal ganglia involvement. To the best of our knowledge, this is only the second reported case of non-diabetic uremic encephalopathy with atypical MR findings.

Uremic Encephalopathy with Atypical Magnetic Resonance Features on Diffusion-Weighted Images

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Kang, Eu Gene; Jeon, Se Jeong; Choi, See Sung [Dept. of Radiology, Wonkwang University School of Medicine and Hospital, Iksan (Korea, Republic of)

2012-11-15

Uremic encephalopathy is a well-known disease with typical MR findings including bilateral vasogenic or cytotoxic edema at the cerebral cortex or basal ganglia. Involvement of the basal ganglia has been very rarely reported, typically occurring in uremic-diabetic patients. We recently treated a patient who had non-diabetic uremic encephalopathy with an atypical lesion distribution involving the supratentorial white matter, without cortical or basal ganglia involvement. To the best of our knowledge, this is only the second reported case of non-diabetic uremic encephalopathy with atypical MR findings.

From appearance to essence: 10 years review of atypical amniotic fluid embolism.

Science.gov (United States)

Shen, Fangrong; Wang, Lu; Yang, Weiwen; Chen, Youguo

2016-02-01

Amniotic fluid embolism (AFE) is an unpredictable and unpreventable complication of maternity. The presentation may range from relatively subtle clinical events to sudden maternal cardiac arrest. However, the neglected diagnosis of non-classical form of AFE (atypical AFE) is very common. The aim of this study was to examine population-based regional data from Suzhou, China. Based on the analysis of all available case reports, we put forward an outline of atypical AFE and investigate whether any variation identified could be ascribed to methodology. Retrospective study from January 2004 to December 2013, 53 cases was identified from the database of Center for Disease Control (CDC) in the city of Suzhou. We investigated the presentations of atypical AFE and maternal characteristics with potential factors underlying AFE. Multiple-regression analysis was used to calculate adjusted odds ratios (ORs) and 95 % confidence intervals (CIs). The incidence of AFE was 6.91 per 100,000 deliveries (53/766,895). Seventeen deaths occurred, a mortality rate of 32 %. Atypical AFE may as the earlier stage or mild form of AFE, there was no death case in the study with timely remedy. The atypical AFE appear is obstetric hemorrhage and/or pulmonary and renal dysfunction postpartum. Hyperfibrinolysis and coagulopathy may the early laboratory findings of atypical AFE. Atypical and classical AFE shared the same risks, such as advanced maternal age, placental abnormalities, operative deliveries, eclampsia, cervical lacerations, and induction of labor. Staying alert to premonitory symptoms of AFE is critical to turn it to a remediable disease. Patient complaints such as breathlessness, chest pain, feeling cold, distress, panic, a feeling of nausea, and vomiting should elicit close attention. The management of a suspected episode of amniotic fluid embolism is generally considered to be supportive. Hysterectomy must be performed if there is further progression of symptoms. Due to advances in

Complement Mutations in Diacylglycerol Kinase-ε–Associated Atypical Hemolytic Uremic Syndrome

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Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

2014-01-01

kinase-ε and C3 mutations. Conclusions Data suggest that complement dysregulation influences the onset and disease severity in carriers of diacylglycerol kinase-ε mutations and that treatments on the basis of plasma infusions and complement inhibition are potentially useful in patients with combined diacylglycerol kinase-ε and complement mutations. A comprehensive understanding of the genetic component predisposing to atypical hemolytic uremic syndrome is, therefore, critical to guide an effective treatment. PMID:25135762

Complement mutations in diacylglycerol kinase-ε-associated atypical hemolytic uremic syndrome.

Science.gov (United States)

Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

2014-09-05

the onset and disease severity in carriers of diacylglycerol kinase-ε mutations and that treatments on the basis of plasma infusions and complement inhibition are potentially useful in patients with combined diacylglycerol kinase-ε and complement mutations. A comprehensive understanding of the genetic component predisposing to atypical hemolytic uremic syndrome is, therefore, critical to guide an effective treatment. Copyright © 2014 by the American Society of Nephrology.

Atypical presentation of sporotrichosis: report of three cases

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Melissa Orzechowski Xavier

2013-01-01

Full Text Available Sporotrichosis occurs after fungal implantation of Sporothrix spp. in the skin, and is the main subcutaneous mycosis in Latin America. Here we describe three atypical cases of the disease. The first case report an extra-cutaneous occurrence of the disease with joint infection; the second one describes a patient with bilateral lymphocutaneous form of sporotrichosis; and the third shows a zoonotic cutaneous case with the development of an erythema nodosum as a hypersensitivity reaction. These cases show the disease importance on the region and the necessity of fungal culture to the diagnosis confirmation.

High Efficiency of Human Normal Immunoglobulin for Intravenous Administration in a Patient with Kawasaki Syndrome Diagnosed in the Later Stages

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Tatyana V. Sleptsova

2016-01-01

Full Text Available The article describes a case of late diagnosis of mucocutaneous lymphonodular syndrome (Kawasaki syndrome. At the beginning of the therapy, the child had fever, conjunctivitis, stomatitis, rash, solid swelling of hands and feet, and coronaritis with the development of aneurysms. The article describes the successful use of normal human immunoglobulin for intravenous administration at a dose of 2 g/kg body weight per course in combination with acetylsalicylic acid at the dose of 80 mg/kg per day. After 3 days of treatment, the rash disappeared; limb swelling and symptoms of conjunctivitis significantly reduced; and laboratory parameters of disease activity became normal (erythrocyte sedimentation rate, C-reactive protein concentration. After 3 months, inflammation in the coronary arteries was stopped. After 6 months, a regression of coronary artery aneurysms was recorded. No adverse effects during the immunoglobulin therapy were observed.

Aspects of atypical degenerative lesions of vertebrae

International Nuclear Information System (INIS)

Battikha, J.G.; Garcia, J.F.; Wettstein, P.

1981-01-01

Over the last 20 years, several authors have reported aspects of degenerative disease of the vertebral column with irregularity and sclerosis of the margins of the vertebral bodies [2, 4, 7-9, 13, 15, 17]. Twenty cases of such atypical degenerative vertebral lesions have been studied over a two year period and their radiological characteristics have been compared with vertebral lesions of infective origin and in the rheumatoid disorders. (orig.)

Atypical idiopathic inflammatory demyelinating lesions

DEFF Research Database (Denmark)

Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo

2013-01-01

Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class......Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can...... be classified according to previously suggested radiologic characteristics and how this classification relates to prognosis. Searching the databases of eight tertiary referral centres we identified 90 adult patients (61 women, 29 men; mean age 34 years) with ≥1 AIIDL. We collected their demographic, clinical...

Atypical subtrochanteric and diaphyseal femoral fractures

DEFF Research Database (Denmark)

Shane, Elizabeth; Burr, David; Abrahamsen, Bo

2014-01-01

Bisphosphonates (BPs) and denosumab reduce the risk of spine and nonspine fractures. Atypical femur fractures (AFFs) located in the subtrochanteric region and diaphysis of the femur have been reported in patients taking BPs and in patients on denosumab, but they also occur in patients with no exp......Bisphosphonates (BPs) and denosumab reduce the risk of spine and nonspine fractures. Atypical femur fractures (AFFs) located in the subtrochanteric region and diaphysis of the femur have been reported in patients taking BPs and in patients on denosumab, but they also occur in patients...... with no exposure to these drugs. In this report, we review studies on the epidemiology, pathogenesis, and medical management of AFFs, published since 2010. This newer evidence suggests that AFFs are stress or insufficiency fractures. The original case definition was revised to highlight radiographic features...... a minor to a major feature. The association with specific diseases and drug exposures was removed from the minor features, because it was considered that these associations should be sought rather than be included in the case definition. Studies with radiographic review consistently report significant...

Identification of an atypical etiological head and neck squamous carcinoma subtype featuring the CpG island methylator phenotype

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K. Brennan

2017-03-01

Further distinguishing features of this ‘CIMP-Atypical’ subtype include an antiviral gene expression profile associated with pro-inflammatory M1 macrophages and CD8+ T cell infiltration, CASP8 mutations, and a well-differentiated state corresponding to normal SOX2 copy number and SOX2OT hypermethylation. We developed a gene expression classifier for the CIMP-Atypical subtype that could classify atypical disease features in two independent patient cohorts, demonstrating the reproducibility of this subtype. Taken together, these findings provide unprecedented evidence that atypical HNSCC is molecularly distinct, and postulates the CIMP-Atypical subtype as a distinct clinical entity that may be caused by chronic inflammation.

Atypical Hand, Foot, and Mouth Disease Caused by Coxsackievirus A6 in Denmark:

DEFF Research Database (Denmark)

Horsten, Hans-Henrik; Kemp, Michael; Fischer, Thea K

2018-01-01

, vasculitis, syphilis, dermatophytid, erythema multiforme and Stevens-Johnson syndrome. Three adults and 3 children required hospitalization due to extensive skin involvement and fever. All reported patients had laboratory confirmed enterovirus infection. This study demonstrated an upsurge in atypical HFMD...

Atypical presentation of dopa-responsive dystonia in Taiwan.

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Weng, Yi Ching; Wang, Chun Chieh; Wu, Yih Ru

2018-02-01

The typical clinical presentation of dopa-responsive dystonia, which is also called Segawa disease, is a young age of onset, with predominance in females, diurnal fluctuation of lower limb dystonia, and fair response to low-dose levodopa. This disease has both autosomal dominant and autosomal recessive inheritance. Autosomal dominant Segawa disease is caused by GCH1 mutation on chromosome 14q22.1-q22.2. Here, we report the case of a male patient with genetically confirmed Segawa disease and atypical presentations including no diurnal symptom fluctuation and insufficient response to levodopa. The patient's father who had the same mutation presented parkinsonism in old age. We also review the literature to address the broad clinical heterogeneity of Segawa disease and the influence of onset age on clinical presentation.

An atypical case of Reye's syndrome

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Maehara, Fumiaki; Goto, Katsuya; Okudera, Toshio; Mitsudome, Akihisa; Hara, Kunio; Shiraishi, Masayuki

1982-01-01

An atypical case of Reye's syndrome was reported with emphasis on usefulness of CT for the diagnosis and follow-up study of this disease. The patient was a 13-month-old girl who had been transferred to our hospital because of status epilepticus, a comatous state and a high temperature. She was diagnosed as having Reye's syndrome according to data of liver function tests, findings in CSF and body CT which revealed swelling of the liver with diminished attenuation value suggesting fatty infiltration. However, there were atypical features in this patient: epileptic seizures since age 5 months, no vomiting at the time of onset and no evidence of brain swelling on CT in acute phase. She was discharged 2 months later with impaired neuropsychological functions of marked degree. When she was 2 year-old, she again went into status epilepticus, was comatous and had a high temperature. She was dead when she arrived at emergency room of our hospital. Autopsy findings revealed features of Reye's syndrome as follows: abundant accumulation of small fat droplets without nuclear displacement in the liver, fatty infiltration in the kidney and myocardium, and mild swelling in the cerebral cortex with marked ventricular dilatation. The possibility of recurrence of Reye's syndrome was discussed based on the clinical and autopsy findings. The value of CT in the diagnosis and the follow-up study of this disease was emphasized. (author)

Perianal atypical leiomyoma: A case report.

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Sun, Pingliang; Ou, Hailing; Huang, Shen; Wei, Longxiang; Zhang, Sen; Liu, Jiali; Geng, Shuguang; Yang, Kun

2017-12-01

Reports on perianal atypical leiomyoma, a perianal tumor, are rare. We confirmed a perianal atypical leiomyoma by its clinical presentation, magnetic resonance imaging findings, and immunohistochemistry. A 28-year-old female with a perianal mass found more than 4 years ago. The 5cm_4cm_4cm sized mass was located on the left side of the anus and vagina; The magnetic resonance imaging (MRI) scan revealed: A 4.1cm × 5.2cm × 4.9cm sized round mass was observed on the left side of the circumference. Perianal atypical leiomyoma. anal peripheral mass resection was performed under lumbar anesthesia. The postoperative course was uneventful, healing, the patient was discharged. Perianal atypical leiomyomas are benign tumors, but with the clinically atypical leiomyoma, it is sometimes difficult to distinguish between potential malignant smooth muscle tumors,and there may be malignant changes. Surgery should ensure complete resection, and to avoid postoperative recurrence, there should be a regular follow-up.

Extensive hypertrophic lupus erythematosus: Atypical presentation

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Tarun Narang

2012-01-01

Full Text Available Lupus erythematosus (LE is a disease with a wide spectrum of cutaneous and systemic manifestations. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Our objective is to present a case of hypertrophic LE with atypical morphology and extensive involvement, who responded favorably to isotretinoin. Diagnosis of hypertrophic lupus erythematosus (HLE was confirmed by characteristic histopathological findings. Combination therapy with isotretinoin and hydroxychloroquine resulted in flattening and repression of previously refractory skin lesions. Sometimes, HLE lesions may present a diagnostic and therapeutic dilemma. In long standing lesions, squamous cell carcinoma may arise. Therefore, HLE requires adequate therapy with clinical and histopathological follow up.

Atypical calcific tendinitis with cortical erosions

International Nuclear Information System (INIS)

Kraemer, E.J.; El-Khoury, G.Y.

2000-01-01

Objective. To present and discuss six cases of calcific tendinitis in atypical locations (one at the insertion of the pectoralis major and five at the insertion of the gluteus maximus).Patients and results. All cases were associated with cortical erosions, and five had soft tissue calcifications. The initial presentation was confusing and the patients were suspected of having infection or neoplastic disease.Conclusion. Calcific tendinitis is a self-limiting condition. It is important to recognize the imaging features of this condition to avoid unnecessary investigation and surgery. (orig.)

Effectiveness and cost of atypical versus typical antipsychotic treatment for schizophrenia in routine care.

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Stargardt, Tom; Weinbrenner, Susanne; Busse, Reinhard; Juckel, Georg; Gericke, Christian A

2008-06-01

statistical power for subgroup analyses, the lack of clinical severity scale data and of life-course medical history data which both increase the risk of residual confounding by disease severity. This study provides evidence that the effectiveness of atypical and typical antipsychotics measured in terms of hospital readmissions appears to be similar in routine care. From a clinical perspective, this study provides evidence that the effectiveness of atypical and typical antipsychotics measured in terms of hospital readmissions appears to be similar in routine care. Routine data studies can yield valuable information for policy decision-makers on the costs and the effectiveness of pharmaceuticals in routine care, complementing efficacy data from randomised clinical trials currently used for licensing and reimbursement decisions. The non-significant differences in the effectiveness of atypical compared to typical antipsychotics according to severity of disease should be investigated in a prospective observational study or in a randomised clinical trial.

Tracing the associations between sex, the atypical and the combined atypical-melancholic depression subtypes: A path analysis.

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Rodgers, Stephanie; Vandeleur, Caroline L; Ajdacic-Gross, Vladeta; Aleksandrowicz, Aleksandra A; Strippoli, Marie-Pierre F; Castelao, Enrique; Glaus, Jennifer; Lasserre, Aurélie M; Müller, Mario; Rössler, Wulf; Angst, Jules; Preisig, Martin

2016-01-15

Numerous studies have examined determinants leading to preponderance of women in major depressive disorder (MDD), which is particularly accentuated for the atypical depression subtype. It is thus of interest to explore the specific indirect effects influencing the association between sex and established depression subtypes. The data of 1624 subjects with a lifetime diagnosis of MDD derived from the population-based PsyCoLaus data were used. An atypical (n=256), a melancholic (n=422), a combined atypical and melancholic features subtype (n=198), and an unspecified MDD group (n=748) were constructed according to the DSM-IV specifiers. Path models with direct and indirect effects were applied to the data. Partial mediation of the female-related atypical and combined atypical-melancholic depression subtypes was found. Early anxiety disorders and high emotion-orientated coping acted as mediating variables between sex and the atypical depression subtype. In contrast, high Body Mass Index (BMI) served as a suppression variable, also concerning the association between sex and the combined atypical-melancholic subtype. The latter association was additionally mediated by an early age of MDD onset and early/late anxiety disorders. The use of cross-sectional data does not allow causal conclusions. This is the first study that provides evidence for a differentiation of the general mechanisms explaining sex differences of overall MDD by depression subtypes. Determinants affecting the pathways begin early in life. Since some of them are primarily of behavioral nature, the present findings could be a valuable target in mental health care. Copyright © 2015 Elsevier B.V. All rights reserved.

A family with atypical Hailey Hailey disease--is there more to the underlying genetics than ATP2C1?

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Nina van Beek

Full Text Available The autosomal dominant Hailey Hailey disease (HHD is caused by mutations in the ATP2C1 gene encoding for human secretory pathway Ca2+/Mn2+ ATPase protein (hSPCA1 in the Golgi apparatus. Clinically, HHD presents with erosions and hyperkeratosis predominantly in the intertrigines. Here we report an exome next generation sequencing (NGS based analysis of ATPase genes in a Greek family with 3 HHD patients presenting with clinically atypical lesions mainly localized on the neck and shoulders. By NGS of one HHD-patient and in silico SNP calling and SNP filtering we identified a SNP in the expected ATP2C1 gene and SNPs in further ATPase genes. Verification in all 3 affected family members revealed a heterozygous frameshift deletion at position 2355_2358 in exon 24 of ATP2C1 in all three patients. 7 additional SNPs in 4 ATPase genes (ATP9B, ATP11A, ATP2B3 and ATP13A5 were identified. The SNPs rs138177421 in the ATP9B gene and rs2280268 in the ATP13A5 gene were detected in all 3 affected, but not in 2 non affected family members. The SNPs in the ATP2B3 and ATP11A gene as well as further SNPs in the ATP13A5 gene could not be confirmed in all affected family members. One may speculate that besides the level of functional hSPCA1 protein, levels of other ATPase proteins may influence expressivity of the disease and might also contribute, as in this case, to atypical presentations.

Neurovestibular analysis and falls in Parkinson's disease and atypical parkinsonism.

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Venhovens, J; Meulstee, J; Bloem, B R; Verhagen, W I M

2016-06-01

The primary aim of our study was to determine the extent of vestibular dysfunction in patients with Parkinson's disease (PD). Our secondary aim was to determine if vestibular dysfunction in PD is a risk factor for falling. The tertiary aim was to determine both the extent of vestibular dysfunction and if this dysfunction is a risk factor for falling in patients with atypical parkinsonism (AP). Twenty-five healthy subjects, 30 PD patients and 14 AP patients were matched for age and gender in a case-control study design. All subjects underwent clinical neurological and neurotological assessments, cervical and ocular vestibular evoked myogenic potentials (VEMPs), brainstem auditory evoked potentials (BAEPs), subjective visual vertical measurements, and videonystagmography with caloric and rotatory chair stimulation. Ninety per cent of PD patients (27 of 30) and all 14 AP patients had signs of vestibular dysfunction on laboratory examinations. The evoked potential (VEMPs and BAEPs) test results of PD patients showed significant prolongation of the p13, n1 and interpeak III-V latencies on the symptomatic brainstem side (0.003 ≤  P ≤ 0.019) compared with healthy subjects. Also, vestibular testing abnormalities were correlated with an increased risk for falling when fallers among PD and AP patients were compared with the non-fallers (P ≤ 0.001). To conclude, vestibular dysfunction on vestibular laboratory testing is highly prevalent in both PD and AP patients compared with healthy subjects, and is associated with an increased risk for falling. © 2016 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.

Detection of respiratory bacterial pathogens causing atypical pneumonia by multiplex Lightmix® RT-PCR.

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Wagner, Karoline; Springer, Burkard; Imkamp, Frank; Opota, Onya; Greub, Gilbert; Keller, Peter M

2018-04-01

Pneumonia is a severe infectious disease. In addition to common viruses and bacterial pathogens (e.g. Streptococcus pneumoniae), fastidious respiratory pathogens like Chlamydia pneumoniae, Mycoplasma pneumoniae and Legionella spp. can cause severe atypical pneumonia. They do not respond to penicillin derivatives, which may cause failure of antibiotic empirical therapy. The same applies for infections with B. pertussis and B. parapertussis, the cause of pertussis disease, that may present atypically and need to be treated with macrolides. Moreover, these fastidious bacteria are difficult to identify by culture or serology, and therefore often remain undetected. Thus, rapid and accurate identification of bacterial pathogens causing atypical pneumonia is crucial. We performed a retrospective method evaluation study to evaluate the diagnostic performance of the new, commercially available Lightmix ® multiplex RT-PCR assay that detects these fastidious bacterial pathogens causing atypical pneumonia. In this retrospective study, 368 clinical respiratory specimens, obtained from patients suffering from atypical pneumonia that have been tested negative for the presence of common agents of pneumonia by culture and viral PCR, were investigated. These clinical specimens have been previously characterized by singleplex RT-PCR assays in our diagnostic laboratory and were used to evaluate the diagnostic performance of the respiratory multiplex Lightmix ® RT-PCR. The multiplex RT-PCR displayed a limit of detection between 5 and 10 DNA copies for different in-panel organisms and showed identical performance characteristics with respect to specificity and sensitivity as in-house singleplex RT-PCRs for pathogen detection. The Lightmix ® multiplex RT-PCR assay represents a low-cost, time-saving and accurate diagnostic tool with high throughput potential. The time-to-result using an automated DNA extraction device for respiratory specimens followed by multiplex RT-PCR detection was

Atypical Antidepressants

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... health-medications/index.shtml. Accessed May 16, 2016. Hirsch M, et al. Atypical antidepressants: Pharmacology, admininstration, and ... www.uptodate.com/home. Accessed May 23, 2016. Hirsch M, et al. Discontinuing antidepressant medications in adults. ...

Recovery of atypical Aeromonas salmonicida from ulcerated fish from the Baltic Sea

DEFF Research Database (Denmark)

Wiklund, T.; Tabolina, I; Bezgachina, T.V.

1999-01-01

Ulcerated fish of six different species were collected during the BMB/ICES Sea-going Workshop "Fish Diseases and Parasites in the Baltic Sea", 25 November to 8 December 1994, and examined for bacteriological infections. Atypical Aeromonas salmonicida strains were isolated from the majority...

Typical Versus Atypical Anorexia Nervosa Among Adolescents: Clinical Characteristics and Implications for ICD-11.

Science.gov (United States)

Silén, Yasmina; Raevuori, Anu; Jüriloo, Elisabeth; Tainio, Veli-Matti; Marttunen, Mauri; Keski-Rahkonen, Anna

2015-09-01

There is scant research on the clinical utility of differentiating International Classification of Diseases (ICD) 10 diagnoses F50.0 anorexia nervosa (typical AN) and F50.1 atypical anorexia. We reviewed systematically records of 47 adolescents who fulfilled criteria for ICD-10 F50.0 (n = 34) or F50.1 (n = 13), assessing the impact of diagnostic subtype, comorbidity, background factors and treatment choices on recovery. Atypical AN patients were significantly older (p = 0.03), heavier (minimum body mass index 16.7 vs 15.1 kg/m(2) , p = 0.003) and less prone to comorbidities (38% vs 71%, p = 0.04) and had shorter, less intensive and less costly treatments than typical AN patients. The diagnosis of typical versus atypical AN was the sole significant predictor of treatment success: recovery from atypical AN was 4.3 times (95% confidence interval [1.1, 17.5]) as likely as recovery from typical AN. Overall, our findings indicate that a broader definition of AN may dilute the prognostic value of the diagnosis, and therefore, ICD-11 should retain its distinction between typical and atypical AN. Copyright © 2015 John Wiley & Sons, Ltd and Eating Disorders Association.

Viral pneumonias: Typical and atypical findings

International Nuclear Information System (INIS)

Westhoff-Bleck, M.; Bleck, J.S.; Schirg, E.

1987-01-01

The clinical and radiological features of viral pneumonias are summarized and discussed. Although viral infections of the lung belong to atypical pneumonias they demonstrate not always the radiographic pattern of an interstitial pneumonia. Characteristic radiographic findings are quite rare. In most cases the microbial etiology cannot be predicted from chest radiographs. The appearance varies depending on the virulence of the organism and the resistence of the host. In this regard knowledge of epidemiological data as well as patients condition and underlying disease is of utmost importance. Differentiation between community- and hospital-acquired infection may be very helpful. (orig.) [de

Quantitative assessment of urban and industrial symbiosis in Kawasaki, Japan.

Science.gov (United States)

Van Berkel, Rene; Fujita, Tsuyoshi; Hashimoto, Shizuka; Fujii, Minoru

2009-03-01

Colocated firms can achieve environmental benefit and competitive advantage from exchanging physical resources (known as industrial symbiosis) with each other or with residential areas (referenced here as urban symbiosis). Past research illustrated that economic and environmental benefits appear self-evident, although detailed quantification has only been attempted of symbioses for energy and water utilities. This article provides a complimentary case studyfor Kawasaki, Japan. The 14 documented symbioses connect steel, cement, chemical, and paperfirms and their spin-off recycling businesses. Seven key material exchanges divert annually at least 565 000 tons of waste from incineration or landfill. Four of these collectively present an estimated economic opportunity of 13.3 billion JPY (approximately 130 million USD) annually. Five symbioses involve utilization of byproduct and two sharing of utilities. The others are traditional or new recycling industries that do not specifically benefit from geographic proximity. The synergistic effect of urban and industrial symbiosis is unique. The legislative framework for a recycling-oriented society has contributed to realization of the symbioses, as has the availability of government subsidies through the Eco-Town program.

Late onset of atypical paroxysmal non-kinesigenic dyskinesia with remote history of Graves′ disease

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Abdul Qayyum Rana

2013-01-01

Full Text Available Paroxysmal non-kinesigenic dyskinesia (PNKD is a rare hyperkinetic movement disorder and falls under the category of paroxysmal movement disorders. In this condition, episodes are spontaneous, involuntary, and involve dystonic posturing with choreic and ballistic movements. Attacks last for minutes to hours and rarely occur more than once per day. Attacks are not typically triggered by sudden movement, but may be brought on by alcohol, caffeine, stress, fatigue, or chocolate. We report a patient with multiple atypical features of PNKD. She had a 7-year history of this condition with onset at the age of 59, and a remote history of Graves′ disease requiring total thyroidectomy. The frequency of attacks in our case ranged from five to six times a day to a minimum of twice per week, and the duration of episode was short, lasting not more than 2 min. Typically, PNKDs occur at a much younger age and have longer attack durations with low frequency. Administering clonazepam worked to reduce her symptoms, although majority of previous research suggests that pharmacological interventions have poor outcomes.

Late onset of atypical paroxysmal non-kinesigenic dyskinesia with remote history of Graves' disease.

Science.gov (United States)

Rana, Abdul Qayyum; Nadeem, Ambreen; Yousuf, Muhammad Saad; Kachhvi, Zakerabibi M

2013-10-01

Paroxysmal non-kinesigenic dyskinesia (PNKD) is a rare hyperkinetic movement disorder and falls under the category of paroxysmal movement disorders. In this condition, episodes are spontaneous, involuntary, and involve dystonic posturing with choreic and ballistic movements. Attacks last for minutes to hours and rarely occur more than once per day. Attacks are not typically triggered by sudden movement, but may be brought on by alcohol, caffeine, stress, fatigue, or chocolate. We report a patient with multiple atypical features of PNKD. She had a 7-year history of this condition with onset at the age of 59, and a remote history of Graves' disease requiring total thyroidectomy. The frequency of attacks in our case ranged from five to six times a day to a minimum of twice per week, and the duration of episode was short, lasting not more than 2 min. Typically, PNKDs occur at a much younger age and have longer attack durations with low frequency. Administering clonazepam worked to reduce her symptoms, although majority of previous research suggests that pharmacological interventions have poor outcomes.

Solitary, multiple, benign, atypical, or malignant: the "Granular Cell Tumor" puzzle.

Science.gov (United States)

Machado, Isidro; Cruz, Julia; Lavernia, Javier; Llombart-Bosch, Antonio

2016-05-01

The clinical evolution and biology of granular cell tumors (GCT) are poorly understood and treatment remains an issue of discussion. The majority of GCT are benign, although some display malignant behavior. The distinction between benign, atypical, and malignant GCT is controversial due to morphological and immunohistochemical overlap and lack of consistent histological and phenotypic criteria that predict behavior. Although histological criteria may indicate increased risk of malignant evolution, some GCT with evident benign appearance exceptionally progress towards metastatic disease. In this review, we discuss current knowledge on GCT, including histologic, immunophenotypic, and molecular characteristics and differential diagnosis. We focus on the following problematic items in GCT: (1) evolution of classification, (2) neural versus non-neural GCT, (3) neoplastic versus reactive disease, (4) malignant transformation of benign GCT, and (5) multiple versus metastatic GCT. We conclude that although a Ki-67 index >10 % and the presence of mitoses and/or of necrosis are frequently associated with malignant behavior, metastasis remains the only unequivocal sign of malignancy in GCT. An infiltrative growth pattern and vascular and/or perineural invasion are not indicative of malignancy. GCT with atypical/uncertain features almost never metastasize, and many of these tumors either behave in a benign fashion or only recur locally (similar to incompletely excised benign tumors). We therefore propose that classical and atypical histological variants form a single group of GCT. GCT with various unfavorable histological features might be labeled as "GCT with increased risk of metastasis" rather than malignant GCT.

Combined Diffusion Tensor Imaging and Apparent Transverse Relaxation Rate Differentiate Parkinson Disease and Atypical Parkinsonism.

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Du, G; Lewis, M M; Kanekar, S; Sterling, N W; He, L; Kong, L; Li, R; Huang, X

2017-05-01

Both diffusion tensor imaging and the apparent transverse relaxation rate have shown promise in differentiating Parkinson disease from atypical parkinsonism (particularly multiple system atrophy and progressive supranuclear palsy). The objective of the study was to assess the ability of DTI, the apparent transverse relaxation rate, and their combination for differentiating Parkinson disease, multiple system atrophy, progressive supranuclear palsy, and controls. A total of 106 subjects (36 controls, 35 patients with Parkinson disease, 16 with multiple system atrophy, and 19 with progressive supranuclear palsy) were included. DTI and the apparent transverse relaxation rate measures from the striatal, midbrain, limbic, and cerebellar regions were obtained and compared among groups. The discrimination performance of DTI and the apparent transverse relaxation rate among groups was assessed by using Elastic-Net machine learning and receiver operating characteristic curve analysis. Compared with controls, patients with Parkinson disease showed significant apparent transverse relaxation rate differences in the red nucleus. Compared to those with Parkinson disease, patients with both multiple system atrophy and progressive supranuclear palsy showed more widespread changes, extending from the midbrain to striatal and cerebellar structures. The pattern of changes, however, was different between the 2 groups. For instance, patients with multiple system atrophy showed decreased fractional anisotropy and an increased apparent transverse relaxation rate in the subthalamic nucleus, whereas patients with progressive supranuclear palsy showed an increased mean diffusivity in the hippocampus. Combined, DTI and the apparent transverse relaxation rate were significantly better than DTI or the apparent transverse relaxation rate alone in separating controls from those with Parkinson disease/multiple system atrophy/progressive supranuclear palsy; controls from those with Parkinson

First case report of atypical disseminated cutaneous leishmaniasis in an opium abuser in Iran

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Seyed Ahmad Hashemi

2018-02-01

Full Text Available ABSTRACT Leishmaniasis is a worldwide tropical infectious disease caused by different species of intracellular protozoa parasites of the genus Leishmania . Herein, we report a 78-year-old man with unusual diffuse cutaneous leishmaniasis (DCL who had a history of opium abuse and chronic obstructive pulmonary disease (COPD. He had multiple papular, crusted and severely ulcerated lesions extended to his arm and chest. Direct smears and skin punch biopsy of the lesions were suggestive of leishmaniasis. Parasite DNA was amplified from ulcers, and identified as Leishmania major by PCR-RFLP, confirmed by sequencing analyses. The aim of the current study was to bring to attention this atypical form of disease in CL endemic countries. Thus, this is the first case of DCL in an opium abuser with COPD due to L. major in Northeastern Iran indicating that atypical and extensive forms of CL (DCL owing to L. major are increasing in Iran.

Determining the relative susceptibility of four prion protein genotypes to atypical scrapie

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Atypical scrapie is a sheep prion (PrPSc) disease whose epidemiology is consistent with a sporadic origin and is associated with specific polymorphisms of the normal cellular prion protein (PrPC). We describe a mass spectrometry-based method of detecting and quantifying the polymorphisms of sheep P...

A naturally occurring cowpox virus with an ectromelia virus A-type inclusion protein gene displays atypical A-type inclusions.

Science.gov (United States)

Okeke, Malachy Ifeanyi; Hansen, Hilde; Traavik, Terje

2012-01-01

Human orthopoxvirus (OPV) infections in Europe are usually caused by cowpox virus (CPXV). The genetic heterogeneity of CPXVs may in part be due to recombination with other OPV species. We describe the characterization of an atypical CPXV (CPXV-No-H2) isolated from a human patient in Norway. CPXV-No-H2 was characterized on the basis of A-type inclusion (ATI) phenotype as well as the DNA region containing the p4c and atip open reading frames. CPXV-No-H2 produced atypical V(+/) ATI, in which virions are on the surface of ATI but not within the ATI matrix. Phylogenetic analysis showed that the atip gene of CPXV-No-H2 clustered closely with that of ectromelia virus (ECTV) with a bootstrap support of 100% whereas its p4c gene is diverged compared to homologues in other OPV species. By recombination analysis we identified a putative crossover event at nucleotide 147, downstream the start of the atip gene. Our results suggest that CPXV-No-H2 originated from a recombination between CPXV and ECTV. Our findings are relevant to the evolution of OPVs. Copyright © 2011 Elsevier B.V. All rights reserved.

A bone fide atypical fibroxanthoma of penis

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Roberto Cuomo

2014-01-01

Full Text Available Malignant mesenchymal tumors of the penis are very rare and they have vascular origin. We present a case of a 71-year-old man with a painless nodule of 2.0 cm in diameter located in the penile foreskin. There was no history of urinary or sexually transmitted disease. An excisional biopsy revealed a markedly pleomorphic sarcoma resembling atypical fibroxanthoma (AFX associated with a squamous cell carcinoma in situ. The patient refused a wide re-excision and was free of disease after 36 months. Because the different therapeutic management and prognosis, differential diagnosis should be made with sarcomatoid squamous cell carcinoma and melanoma: A diagnosis of AFX or malignant fibrous histiocytoma may be considered only after the complete exclusion of these two entities.

Detection and partial discrimination of atypical and classical bovine spongiform encephalopathies in cattle and primates using real-time quaking-induced conversion assay.

Science.gov (United States)

Levavasseur, Etienne; Biacabe, Anne-Gaëlle; Comoy, Emmanuel; Culeux, Audrey; Grznarova, Katarina; Privat, Nicolas; Simoneau, Steve; Flan, Benoit; Sazdovitch, Véronique; Seilhean, Danielle; Baron, Thierry; Haïk, Stéphane

2017-01-01

The transmission of classical bovine spongiform encephalopathy (C-BSE) through contaminated meat product consumption is responsible for variant Creutzfeldt-Jakob disease (vCJD) in humans. More recent and atypical forms of BSE (L-BSE and H-BSE) have been identified in cattle since the C-BSE epidemic. Their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of Creutzfeldt-Jakob disease (CJD) in humans. Transmissions studies in primates and transgenic mice expressing a human prion protein (PrP) indicated that atypical forms of BSE may be associated with a higher zoonotic potential than classical BSE, and require particular attention for public health. Recently, methods designed to amplify misfolded forms of PrP have emerged as promising tools to detect prion strains and to study their diversity. Here, we validated real-time quaking-induced conversion assay for the discrimination of atypical and classical BSE strains using a large series of bovine samples encompassing all the atypical BSE cases detected by the French Centre of Reference during 10 years of exhaustive active surveillance. We obtained a 100% sensitivity and specificity for atypical BSE detection. In addition, the assay was able to discriminate atypical and classical BSE in non-human primates, and also sporadic CJD and vCJD in humans. The RT-QuIC assay appears as a practical means for a reliable detection of atypical BSE strains in a homologous or heterologous PrP context.

Clinical characteristics and serum N-terminal pro-brain natriuretic peptide as a diagnostic marker of Kawasaki disease in infants younger than 3 months of age.

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Bae, Hyun Kyung; Lee, Do Kyung; Kwon, Jung Hyun; Kim, Hae Soon; Sohn, Sejung; Hong, Young Mi

2014-08-01

The incidence of Kawasaki disease (KD) is rare in young infants (less than 3 months of age), who present with only a few symptoms that fulfill the clinical diagnostic criteria. The diagnosis for KD can therefore be delayed, leading to a high risk of cardiac complications. We examined the clinical characteristics and measured the serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) levels of these patients for assessing its value in the early detection of KD. We retrospectively reviewed the data of young infants diagnosed with KD from 2004 to 2012. The control group included 20 hospitalized febrile patients. Laboratory data, including NT-proBNP were obtained for each patient in both groups. Incomplete KD was observed in 21/24 patients (87.5%). The mean fever duration on admission was 1.36±1.0 days in the KD group. Common symptoms included erythema at the site of Bacille Calmette-Guerin inoculation (70.8%), skin rash (50.0%), changes of oropharyngeal mucosa (29.1%), and cervical lymphadenopathy (20.8%). The mean number of major diagnostic criteria fulfilled was 2.8±1.4. Five KD patients (20.8%) had only one symptom matching these criteria. The incidence of coronary artery complications was 12.5%. The mean serum NT-proBNP level in the acute phase, in the KD and control groups, were 4,159±3,714 pg/mL and 957±902 pg/mL, respectively, which decreased significantly in the convalescent phase. Incomplete KD was observed in 87.5% patients. Serum NT-proBNP might be a valuable biomarker for the early detection of KD in febrile infants aged <3 months.

Diagnostic and therapeutic dilemma associated with atypical glandular cells on liquid-based cervical cytology.

LENUS (Irish Health Repository)

Chummun, K

2012-12-01

In 2008, the management of women in Ireland with atypical glandular cells changed to immediate referral to colposcopy. The optimal management of these women is unclear. A balance between the detection of occult disease and overtreatment is required.

A systematic review of lessons learned from PET molecular imaging research in atypical parkinsonism

International Nuclear Information System (INIS)

Niccolini, Flavia; Politis, Marios

2016-01-01

To systematically review the previous studies and current status of positron emission tomography (PET) molecular imaging research in atypical parkinsonism. MEDLINE, ISI Web of Science, Cochrane Library, and Scopus electronic databases were searched for articles published until 29th March 2016 and included brain PET studies in progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal syndrome (CBS). Only articles published in English and in peer-reviewed journals were included in this review. Case-reports, reviews, and non-human studies were excluded. Seventy-seven PET studies investigating the dopaminergic system, glucose metabolism, microglial activation, hyperphosphorilated tau, opioid receptors, the cholinergic system, and GABA A receptors in PSP, MSA, and CBS patients were included in this review. Disease-specific patterns of reduced glucose metabolism have shown higher accuracy than dopaminergic imaging techniques to distinguish between parkinsonian syndromes. Microglial activation has been found in all forms of atypical parkinsonism and reflects the known distribution of neuropathologic changes in these disorders. Opioid receptors are decreased in the striatum of PSP and MSA patients. Subcortical cholinergic dysfunction was more severe in MSA and PSP than Parkinson's disease patients although no significant changes in cortical cholinergic receptors were seen in PSP with cognitive impairment. GABA A receptors were decreased in metabolically affected cortical and subcortical regions in PSP patients. PET molecular imaging has provided valuable insight for understanding the mechanisms underlying atypical parkinsonism. Changes at a molecular level occur early in the course of these neurodegenerative diseases and PET imaging provides the means to aid differential diagnosis, monitor disease progression, identify of novel targets for pharmacotherapy, and monitor response to new treatments. (orig.)

A systematic review of lessons learned from PET molecular imaging research in atypical parkinsonism

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Niccolini, Flavia; Politis, Marios [Neurodegeneration Imaging Group, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King' s College London, London (United Kingdom)

2016-11-15

To systematically review the previous studies and current status of positron emission tomography (PET) molecular imaging research in atypical parkinsonism. MEDLINE, ISI Web of Science, Cochrane Library, and Scopus electronic databases were searched for articles published until 29th March 2016 and included brain PET studies in progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal syndrome (CBS). Only articles published in English and in peer-reviewed journals were included in this review. Case-reports, reviews, and non-human studies were excluded. Seventy-seven PET studies investigating the dopaminergic system, glucose metabolism, microglial activation, hyperphosphorilated tau, opioid receptors, the cholinergic system, and GABA{sub A} receptors in PSP, MSA, and CBS patients were included in this review. Disease-specific patterns of reduced glucose metabolism have shown higher accuracy than dopaminergic imaging techniques to distinguish between parkinsonian syndromes. Microglial activation has been found in all forms of atypical parkinsonism and reflects the known distribution of neuropathologic changes in these disorders. Opioid receptors are decreased in the striatum of PSP and MSA patients. Subcortical cholinergic dysfunction was more severe in MSA and PSP than Parkinson's disease patients although no significant changes in cortical cholinergic receptors were seen in PSP with cognitive impairment. GABA{sub A} receptors were decreased in metabolically affected cortical and subcortical regions in PSP patients. PET molecular imaging has provided valuable insight for understanding the mechanisms underlying atypical parkinsonism. Changes at a molecular level occur early in the course of these neurodegenerative diseases and PET imaging provides the means to aid differential diagnosis, monitor disease progression, identify of novel targets for pharmacotherapy, and monitor response to new treatments. (orig.)

A Case of Early Disseminated Neurological Lyme Disease Followed by Atypical Cutaneous Manifestations

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Vamsi Kantamaneni

2017-01-01

Full Text Available Lyme disease (LD is a tick-borne illness caused by Borrelia burgdorferi sensu stricto. An 80-year-old female from Pennsylvania, USA, presented to an outside hospital with fever, confusion, lower extremity weakness, and stool incontinence. CT head and MRI spine were unremarkable. An infectious work-up including lumbar puncture was negative. She was transferred to our tertiary care hospital. Patient was noted to have mild unilateral right-sided facial droop and a diffuse macular rash throughout the body. She denied any outdoor activities, tick bites, or previous rash. Intravenous ceftriaxone was started for suspected LD. The patient’s symptoms including facial droop resolved within 24 hours of antibiotic therapy. Polymerase chain reaction of the blood, IgM ELISA, and IgM Western blot testing for LD came back positive a few days after initiation of therapy. She was treated for a total of 21 days for neurological LD with complete symptom resolution. Not all patients have the classic “targetoid” EM rash on initial presentation, rash could develop after neurological manifestations, and prompt initiation of antibiotics without awaiting serology is paramount to making a quick and a full recovery. There should be a high index of suspicion for early disseminated LD, as presentations can be atypical.

Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

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Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

2014-05-01

Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Flat epithelial atypia and atypical ductal hyperplasia: carcinoma underestimation rate.

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Ingegnoli, Anna; d'Aloia, Cecilia; Frattaruolo, Antonia; Pallavera, Lara; Martella, Eugenia; Crisi, Girolamo; Zompatori, Maurizio

2010-01-01

This study was carried out to determine the underestimation rate of carcinoma upon surgical biopsy after a diagnosis of flat epithelial atypia and atypical ductal hyperplasia and 11-gauge vacuum-assisted breast biopsy. A retrospective review was conducted of 476 vacuum-assisted breast biopsy performed from May 2005 to January 2007 and a total of 70 cases of atypia were identified. Fifty cases (71%) were categorized as pure atypical ductal hyperplasia, 18 (26%) as pure flat epithelial atypia and two (3%) as concomitant flat epithelial atypia and atypical ductal hyperplasia. Each group were compared with the subsequent open surgical specimens. Surgical biopsy was performed in 44 patients with atypical ductal hyperplasia, 15 patients with flat epithelial atypia, and two patients with flat epithelial atypia and atypical ductal hyperplasia. Five cases of atypical ductal hyperplasia were upgraded to ductal carcinoma in situ, three cases of flat epithelial atypia yielded one ductal carcinoma in situ and two cases of invasive ductal carcinoma, and one case of flat epithelial atypia/atypical ductal hyperplasia had invasive ductal carcinoma. The overall rate of malignancy was 16% for atypical ductal hyperplasia (including flat epithelial atypia/atypical ductal hyperplasia patients) and 20% for flat epithelial atypia. The presence of flat epithelial atypia and atypical ductal hyperplasia at biopsy requires careful consideration, and surgical excision should be suggested.

Atypical features of hyperthyroidism in Blacks

International Nuclear Information System (INIS)

Kalk, W.J.

1980-01-01

Hyperthyroidism is reportedly uncommon in the indigenous populations of Africa. The presenting symptoms volunteered, the symptoms elicited by direct questioning, and the results of physical examination were therefore prospectively compared in 60 Black and 56 White patients with thyrotoxicosis attending a single thyroid clinic. Fewer Blacks than Whites volunteered information about weight loss, while more Blacks complained only of the presence of a goitre. A 'chance' diagnosis of hyperthyroidism was made more frequently in Blacks. Symptomatology elicited by direct questioning and findings on physical examination were generally similar in each group, except that Blacks presented more frequently with complicated disease (cardiac failure and overt myopathy) and infiltrative ophthalmopathy. The frequency with which hyperthyroidism presents 'atypically' in Black compared with White patients may reflect educational, socio-economic and cultural differences in the Black and White populations, and may partly explain the infrequency with which this disease is diagnosed in Blacks

Atypical evening cortisol profile induces visual recognition memory deficit in healthy human subjects

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Gilpin Heather

2008-08-01

Full Text Available Abstract Background Diurnal rhythm-mediated endogenous cortisol levels in humans are characterised by a peak in secretion after awakening that declines throughout the day to an evening trough. However, a significant proportion of the population exhibits an atypical cycle of diurnal cortisol due to shift work, jet-lag, aging, and mental illness. Results The present study has demonstrated a correlation between elevation of cortisol in the evening and deterioration of visual object recognition memory. However, high evening cortisol levels have no effect on spatial memory. Conclusion This study suggests that atypical evening salivary cortisol levels have an important role in the early deterioration of recognition memory. The loss of recognition memory, which is vital for everyday life, is a major symptom of the amnesic syndrome and early stages of Alzheimer's disease. Therefore, this study will promote a potential physiologic marker of early deterioration of recognition memory and a possible diagnostic strategy for Alzheimer's disease.

Atypically presenting kaposiform hemangioendothelioma of the knee: ultrasound findings.

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Erdem Toslak, Iclal; Stegman, Matthew; Reiter, Michael P; Barkan, Güliz A; Borys, Dariusz; Lim-Dunham, Jennifer E

2018-04-10

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of early childhood and infancy. Kasabach-Merritt phenomenon, a common complication of KHE, is characterized by life-threatening thrombocytopenia, hemolytic anemia, and consumption coagulopathy. There may be atypical cases that do not present with Kasabach-Merritt phenomenon and do have atypical imaging findings. Knowledge of atypical imaging features may assist radiologists in identifying KHE. In this report, we present a 4-year-old case of KHE with atypical ultrasound findings.

Imaging the neurobiological substrate of atypical depression by SPECT

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Pagani, Marco [Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy); Karolinska University Hospital, Department of Nuclear Medicine, Stockholm (Sweden); Salmaso, Dario [Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy); Nardo, Davide [University of Rome La Sapienza, Department of Psychology, Rome (Italy); Jonsson, Cathrine; Larsson, Stig A. [Karolinska University Hospital, Department of Nuclear Medicine, Stockholm (Sweden); Jacobsson, Hans [Karolinska University Hospital, Department of Radiology, Stockholm (Sweden); Gardner, Ann [Karolinska University Hospital Huddinge, Karolinska Institutet, Department of Clinical Neuroscience, Section of Psychiatry, Stockholm (Sweden)

2007-01-15

Neurobiological abnormalities underlying atypical depression have previously been suggested. The purpose of this study was to explore differences at functional brain imaging between depressed patients with and without atypical features and healthy controls. Twenty-three out-patients with chronic depressive disorder recruited from a service for patients with audiological symptoms were investigated. Eleven fulfilled the DSM-IV criteria for atypical depression (mood reactivity and at least two of the following: weight gain, hypersomnia, leaden paralysis and interpersonal rejection sensitivity). Twenty-three healthy subjects served as controls. Voxel-based analysis was applied to explore differences in {sup 99m}Tc-HMPAO uptake between groups. Patients in the atypical group had a higher prevalence of bilateral hearing impairment and higher depression and somatic distress ratings at the time of SPECT. Significantly higher tracer uptake was found bilaterally in the atypical group as compared with the non-atypicals in the sensorimotor (Brodmann areas, BA1-3) and premotor cortex in the superior frontal gyri (BA6), in the middle frontal cortex (BA8), in the parietal associative cortex (BA5, BA7) and in the inferior parietal lobule (BA40). Significantly lower tracer distribution was found in the right hemisphere in the non-atypicals compared with the controls in BA6, BA8, BA44, BA45 and BA46 in the frontal cortex, in the orbito-frontal cortex (BA11, BA47), in the postcentral parietal cortex (BA2) and in the multimodal association parietal cortex (BA40). The differences found between atypical and non-atypical depressed patients suggest different neurobiological substrates in these patient groups. The putative links with the clinical features of atypical depression are discussed. These findings encourage the use of functional neuroimaging in psychiatric disorders. (orig.)

Imaging the neurobiological substrate of atypical depression by SPECT

International Nuclear Information System (INIS)

Pagani, Marco; Salmaso, Dario; Nardo, Davide; Jonsson, Cathrine; Larsson, Stig A.; Jacobsson, Hans; Gardner, Ann

2007-01-01

Neurobiological abnormalities underlying atypical depression have previously been suggested. The purpose of this study was to explore differences at functional brain imaging between depressed patients with and without atypical features and healthy controls. Twenty-three out-patients with chronic depressive disorder recruited from a service for patients with audiological symptoms were investigated. Eleven fulfilled the DSM-IV criteria for atypical depression (mood reactivity and at least two of the following: weight gain, hypersomnia, leaden paralysis and interpersonal rejection sensitivity). Twenty-three healthy subjects served as controls. Voxel-based analysis was applied to explore differences in 99m Tc-HMPAO uptake between groups. Patients in the atypical group had a higher prevalence of bilateral hearing impairment and higher depression and somatic distress ratings at the time of SPECT. Significantly higher tracer uptake was found bilaterally in the atypical group as compared with the non-atypicals in the sensorimotor (Brodmann areas, BA1-3) and premotor cortex in the superior frontal gyri (BA6), in the middle frontal cortex (BA8), in the parietal associative cortex (BA5, BA7) and in the inferior parietal lobule (BA40). Significantly lower tracer distribution was found in the right hemisphere in the non-atypicals compared with the controls in BA6, BA8, BA44, BA45 and BA46 in the frontal cortex, in the orbito-frontal cortex (BA11, BA47), in the postcentral parietal cortex (BA2) and in the multimodal association parietal cortex (BA40). The differences found between atypical and non-atypical depressed patients suggest different neurobiological substrates in these patient groups. The putative links with the clinical features of atypical depression are discussed. These findings encourage the use of functional neuroimaging in psychiatric disorders. (orig.)

Effect of Long-Term Use of Bisphosphonates on Forearm Bone: Atypical Ulna Fractures in Elderly Woman with Osteoporosis

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Yusuf Erdem

2016-01-01

Full Text Available Osteoporosis is a common musculoskeletal disease of the elderly population characterized by decreased bone mineral density and subsequent fractures. Bisphosphonates are a widely accepted drug therapy which act through inhibition of bone resorption and prevent fractures. However, in long-term use, atypical bisphosphonate induced fractures may occur, particularly involving the lower weight bearing extremity. Atypical ulna fracture associated with long-term bisphosphonate use is rarely reported in current literature. We present a 62-year-old woman with atypical ulna due to long-term alendronate therapy without a history of trauma or fall. Clinicians should be aware of stress fracture in a patient who has complaints of upper extremity pain and history of long-term bisphosphonate therapy.

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

NARCIS (Netherlands)

Loirat, C.; Fakhouri, F.; Ariceta, G.; Besbas, N.; Bitzan, M.; Bjerre, A.; Coppo, R.; Emma, F.; Johnson, S.; Karpman, D.; Landau, D.; Langman, C.B.; Lapeyraque, A.L.; Licht, C.; Nester, C.; Pecoraro, C.; Riedl, M.; Kar, N.C.A.J. van de; Walle, J. Vande; Vivarelli, M.; Fremeaux-Bacchi, V.

2016-01-01

Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review

ATYPICAL ANTIPSYCHOTICS USE IN CHILDREN AND ADOLESCENTS

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Nataša Potočnik-Dajčman

2002-06-01

Full Text Available Background. Classical antipsychotics – neuroleptics are one of the most frequently prescribed psychotropic drugs in child psychiatry. Atypical antipsychotics are used for the same indications – psychotic (schizophrenia as well as unpsychotic disorders (pervasive developmental disorders, mood disorders, conduct disorders and tics disorders. It is surprising that the studies on their use with regard to this age group are rather rare. They are carried out on a small number of samples and only exceptionally double blind. This article summarizes published clinical experience with atypical antipsychotics in children and adolescents. A short overview of pharmacodynamics, pharmacokinetics and side effects is given. Schizophrenia and pervasive developmental disorders are major indications for use of atypical antipsychotics in children and adolescents, but they have also been successfully used for other disorders such as aggressive behaviour, tics and anorexia nervosa.Conclusions. With better side-effect profile, some of the atypical antipsychotics are expected to be doctrinally recognised as the first-line treatment for childhood schizophrenia and pervasive developmental disorders. However, more long-term studies carried out on a larger sample are needed. Atypical antipsychotics are already used in everyday practice as first-line treatment of childhood and adolescents schizophrenia.

[Development of an Atypical Response Scale.

Science.gov (United States)

Mendelsohn, Mark; Linden, James

The development of an objective diagnostic scale to measure atypical behavior is discussed. The Atypical Response Scale (ARS) is a structured projective test consisting of 17 items, each weighted 1, 2, or 3, that were tested for convergence and reliability. ARS may be individually or group administered in 10-15 minutes; hand scoring requires 90…

Diagnostic accuracy of atypical p-ANCA in autoimmune hepatitis using ROC- and multivariate regression analysis.

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Terjung, B; Bogsch, F; Klein, R; Söhne, J; Reichel, C; Wasmuth, J-C; Beuers, U; Sauerbruch, T; Spengler, U

2004-09-29

Antineutrophil cytoplasmic antibodies (atypical p-ANCA) are detected at high prevalence in sera from patients with autoimmune hepatitis (AIH), but their diagnostic relevance for AIH has not been systematically evaluated so far. Here, we studied sera from 357 patients with autoimmune (autoimmune hepatitis n=175, primary sclerosing cholangitis (PSC) n=35, primary biliary cirrhosis n=45), non-autoimmune chronic liver disease (alcoholic liver cirrhosis n=62; chronic hepatitis C virus infection (HCV) n=21) or healthy controls (n=19) for the presence of various non-organ specific autoantibodies. Atypical p-ANCA, antinuclear antibodies (ANA), antibodies against smooth muscles (SMA), antibodies against liver/kidney microsomes (anti-Lkm1) and antimitochondrial antibodies (AMA) were detected by indirect immunofluorescence microscopy, antibodies against the M2 antigen (anti-M2), antibodies against soluble liver antigen (anti-SLA/LP) and anti-Lkm1 by using enzyme linked immunosorbent assays. To define the diagnostic precision of the autoantibodies, results of autoantibody testing were analyzed by receiver operating characteristics (ROC) and forward conditional logistic regression analysis. Atypical p-ANCA were detected at high prevalence in sera from patients with AIH (81%) and PSC (94%). ROC- and logistic regression analysis revealed atypical p-ANCA and SMA, but not ANA as significant diagnostic seromarkers for AIH (atypical p-ANCA: AUC 0.754+/-0.026, odds ratio [OR] 3.4; SMA: 0.652+/-0.028, OR 4.1). Atypical p-ANCA also emerged as the only diagnostically relevant seromarker for PSC (AUC 0.690+/-0.04, OR 3.4). None of the tested antibodies yielded a significant diagnostic accuracy for patients with alcoholic liver cirrhosis, HCV or healthy controls. Atypical p-ANCA along with SMA represent a seromarker with high diagnostic accuracy for AIH and should be explicitly considered in a revised version of the diagnostic score for AIH.

Atypical chemokine receptors in cancer: friends or foes?

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Massara, Matteo; Bonavita, Ornella; Mantovani, Alberto; Locati, Massimo; Bonecchi, Raffaella

2016-06-01

The chemokine system is a fundamental component of cancer-related inflammation involved in all stages of cancer development. It controls not only leukocyte infiltration in primary tumors but also angiogenesis, cancer cell proliferation, and migration to metastatic sites. Atypical chemokine receptors are a new, emerging class of regulators of the chemokine system. They control chemokine bioavailability by scavenging, transporting, or storing chemokines. They can also regulate the activity of canonical chemokine receptors with which they share the ligands by forming heterodimers or by modulating their expression levels or signaling activity. Here, we summarize recent results about the role of these receptors (atypical chemokine receptor 1/Duffy antigen receptor for chemokine, atypical chemokine receptor 2/D6, atypical chemokine receptor 3/CXC-chemokine receptor 7, and atypical chemokine receptor 4/CC-chemokine receptor-like 1) on the tumorigenesis process, indicating that their effects are strictly dependent on the cell type on which they are expressed and on their coexpression with other chemokine receptors. Indeed, atypical chemokine receptors inhibit tumor growth and progression through their activity as negative regulators of chemokine bioavailability, whereas, on the contrary, they can promote tumorigenesis when they regulate the signaling of other chemokine receptors, such as CXC-chemokine receptor 4. Thus, atypical chemokine receptors are key components of the regulatory network of inflammation and immunity in cancer and may have a major effect on anti-inflammatory and immunotherapeutic strategies. © Society for Leukocyte Biology.

The effect of FcγRIIA and FcγRIIB on coronary artery lesion formation and intravenous immunoglobulin treatment responses in children with Kawasaki disease

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Chang, Ling-Sai; Lo, Mao-Hung; Li, Sung-Chou; Yang, Ming-Yu; Hsieh, Kai-Sheng; Kuo, Ho-Chang

2017-01-01

Previous research has found patients with the FcγRIIIB NA1 variant having increased risk of intravenous immunoglobulin (IVIG) resistance in Kawasaki disease (KD). Our previous studies revealed that elevated FcγRIIA expression correlated with the susceptibility of KD patients. We conducted this research to determine whether and how Fcγ receptors affect the susceptibility, IVIG treatment response, and coronary artery lesions (CAL) of KD patients. The activating FcγRIIA and inhibitory FcγRIIB methylation levels of seven patients with KD and four control subjects were examined using HumanMethylation27 BeadChip. We enrolled a total of 44 KD patients and 10 control subjects with fevers. We performed real-time RT-PCR to determine the FcγRIIA and FcγRIIB expression levels, as well as a luciferase assay of FcγRIIA. We found a considerable increase in methylation of both FcγRIIA and FcγRIIB in KD patients undergoing IVIG treatment. Promoter methylation of FcγRIIA inhibited reporter activity in K562 cells using luciferase assay. The FcγRIIB mRNA expression levels were not found to increase susceptibility, CAL formation, or IVIG resistance. FcγRIIA mRNA expression levels were significantly higher in IVIG-resistant patients than in those that responded to IVIG during the pre-treatment period. Furthermore, the FcγRIIA/IIB mRNA expression ratio was considerably higher in KD patients with CAL than in those without CAL. FcγRIIA and FcγRIIB both demonstrated increased methylation levels in KD patients that underwent IVIG treatment. FcγRIIA expression influenced the IVIG treatment response of KD patients. The FcγRIIA/IIB mRNA expression ratio was greater in KD patients with CAL formation. PMID:27893416

Cryptogenic organizing pneumonia: typical and atypical imaging features on computed tomography

International Nuclear Information System (INIS)

Hamer, O.W.; Silva, C.I.; Mueller, N.L.

2008-01-01

Organizing pneumonia (OP) occurs without any identifiable cause (''cryptogenic organizing pneumonia'') as well as secondary to a multitude of disorders of various origins (''secondary organizing pneumonia''). Possible triggers are infections, drugs, collagen vascular disease, inflammatory bowel disease, transplantations, and radiation directed to the chest. The present manuscript provides an overview of the histopathological, clinical and CT imaging features of OP. Classic CT morphologies (peripheral and peribronchovascular consolidations and ground glass opacities) and atypical imaging features (nodules, crazy paving, lines and bands, perilobular consolidations and the reversed halo sign) are discussed. (orig.)

Kawasaki steam power plant of Tokyo Electric Power Co. and an example of geothermal power generation

Energy Technology Data Exchange (ETDEWEB)

1961-01-01

The first part of this discussion is devoted to a description of the Kawasaki steam power plant, installed by Tokyo Electric Co. to supply electricity to the Keihin industrial area. The output is 700 MW and it possesses a thermal efficiency of 36.9%. The plant is operated automatically by remote control. The latter section describes the status of a geothermal power station in Hakone. It outlines the steam distribution piping, the steam itself, the turbine and vapor/water separation equipment. With regard to technical problems, it is suggested that old wells having weak pressure can be restored by self-cleaning and that further improvement can be brought about by dynamiting the base of the borehole.

Atypical sexual behavior during sleep.

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Guilleminault, Christian; Moscovitch, Adam; Yuen, Kin; Poyares, Dalva

2002-01-01

This article reports a case series of atypical sexual behavior during sleep, which is often harmful to patients or bed partners. Eleven subjects underwent clinical evaluation of complaints of sleep-related atypical sexual behavior. Complaints included violent masturbation, sexual assaults, and continuous (and loud) sexual vocalizations during sleep. One case was a medical-legal case. Sleep logs, clinical evaluations, sleep questionnaires, structured psychiatric interviews, polysomnography, actigraphy, home electroencephalographic monitoring during sleep, and clinical electroencephalographic monitoring while awake and asleep were used to determine clinical diagnoses. Atypical sexual behaviors during sleep were associated with feelings of guilt, shame, and depression. Because of these feelings, patients and bed partners often tolerated the abnormal behavior for long periods of time without seeking medical attention. The following pathologic sleep disorders were demonstrated on polysomnography: partial complex seizures, sleep-disordered breathing, stage 3 to 4 non-rapid eye movement (REM) sleep parasomnias, and REM sleep behavior disorder. These findings were concurrent with morning amnesia. The atypical behaviors were related to different syndromes despite the similarity of complaints from bed partners. In most cases the disturbing and often harmful symptoms were controlled when counseling was instituted and sleep disorders were treated. In some cases treatment of seizures or psychiatric disorders was also needed. Clonazepam with simultaneous psychotherapy was the most common successful treatment combination. The addition of antidepressant or antiepileptic medications was required in specific cases.

Google-driven search for big data in autoimmune geoepidemiology: analysis of 394,827 patients with systemic autoimmune diseases.

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Ramos-Casals, Manuel; Brito-Zerón, Pilar; Kostov, Belchin; Sisó-Almirall, Antoni; Bosch, Xavier; Buss, David; Trilla, Antoni; Stone, John H; Khamashta, Munther A; Shoenfeld, Yehuda

2015-08-01

Systemic autoimmune diseases (SADs) are a significant cause of morbidity and mortality worldwide, although their epidemiological profile varies significantly country by country. We explored the potential of the Google search engine to collect and merge large series (>1000 patients) of SADs reported in the Pubmed library, with the aim of obtaining a high-definition geoepidemiological picture of each disease. We collected data from 394,827 patients with SADs. Analysis showed a predominance of medical vs. administrative databases (74% vs. 26%), public health system vs. health insurance resources (88% vs. 12%) and patient-based vs. population-based designs (82% vs. 18%). The most unbalanced gender ratio was found in primary Sjögren syndrome (pSS), with nearly 10 females affected per 1 male, followed by systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and antiphospholipid syndrome (APS) (ratio of nearly 5:1). Each disease predominantly affects a specific age group: children (Kawasaki disease, primary immunodeficiencies and Schonlein-Henoch disease), young people (SLE Behçet disease and sarcoidosis), middle-aged people (SSc, vasculitis and pSS) and the elderly (amyloidosis, polymyalgia rheumatica, and giant cell arteritis). We found significant differences in the geographical distribution of studies for each disease, and a higher frequency of the three SADs with available data (SLE, inflammatory myopathies and Kawasaki disease) in African-American patients. Using a "big data" approach enabled hitherto unseen connections in SADs to emerge. Copyright © 2015 Elsevier B.V. All rights reserved.

123I-IMP SPECT studies in schizophrenia and atypical psychosis

International Nuclear Information System (INIS)

Hayashi, Takuji; Suga, Hidemichi

1993-01-01

According to the classification of Mitsuda, 23 patients with endogenous psychosis aged 40 years or younger, presenting with hallucination and delusion, were classified as having schizophrenia (n=12) or atypical psychosis (n=11). These patients were studied by single photon emission computed tomography (SPECT) with N-isopropyl-p-I-123-iodoamphetamine (I-123 IMP). Sixteen healthy persons served as controls. Early and delayed SPECT images were obtained 30 min and 4 hr, respectively, after intravenous injection of I-123 IMP. The group of schizophrenic patients had markedly decreased uptake of I-123 in the basal ganglia, as well as the right temporal and left occipital areas on both early and delayed images. In the group of atypical psychosis patients, however, decreased uptake of I-123 was noted in both the right basal ganglia and left occipital area on early images, but none of such findings were seen on delayed images. Regarding the uptake ratio in the frontal area on both early and delayed images, there were significant differences between the two groups. These findings have important implications for the different etiology of both disease types: not only functional disturbance in the frontal area but also irreversible changes may be involved in the occurrence of schizophrenia, and functional disturbance particularly in the right basal ganglia may be involved in the occurrence of atypical psychosis. (N.K.)

Tuberous sclerosis: Analysis of 24 cases with emphasis on atypical findings

International Nuclear Information System (INIS)

Lee, Eun Ju; Suh, Jung Ho; Joo, Suk Hyun; Chung, Tae Sub

1990-01-01

We retrospectively analysed the clinical and CT findings of 24 cases with tuberous sclerosis with special emphasis upon the atypical presentation. The cases with classic clinical triad were accounted for in 21%, while 33% presented with atypical clinical features. The most common and characteristic CT finding is that of the subependymal calcified nodules of the lateral ventricle, which was seen in 85% of our cases. Cortical tuber and white matter lesions were demonstrated in 65% and 55%, respectively. Three cases showed cortical tuber and white matter lesion without subependymal tuber. The cortical tuber usually exhibited low density or calcified lesion, but showed high density in 2 cases. Other findings of tuberous sclerosis included intraventricular tumor, ventriculomegaly, and cortical atrophy. One case clearly demonstrated parenchymal tuber on MR imaging. In conclusion, diagnosis of tuberous sclerosis is usually made on the clinical bases initially. However, CT and MR are also diagnostic even in unusual clinical presentation such as unexplained retardation or epilepsy or when the classic clinical triad is incomplete. If only cortical tuber or white matter lesion is present, it is difficult to diagnose tuberous sclerosis and to differentiate from other cortical mass lesion. Therefore, careful evaluation is required with familiarity with the atypical appearance of the disease

Atypical choroid plexus papilloma: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases.

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Scala, Marcello; Morana, Giovanni; Milanaccio, Claudia; Pavanello, Marco; Nozza, Paolo; Garrè, Maria Luisa

2017-09-01

Atypical choroid plexus papillomas can metastasize in the form of leptomeningeal seeding. Postoperative chemotherapy is the recommended first-line treatment when gross-total removal is not achieved or in cases of disseminated disease. Here the authors report on 2 children with atypical choroid plexus papillomas and MRI findings of diffuse leptomeningeal enhancement at diagnosis, later presenting with spontaneous resolution of the leptomeningeal involvement after removal of the primary lesions. Observations in this report expand our knowledge about the natural history and biological behavior of these tumors and highlight the role of close neuroimaging surveillance in the management of atypical choroid plexus papillomas in cases with MRI evidence of diffuse leptomeningeal enhancement at presentation.

An atypical presentation of cystic fibrosis: a case report

Directory of Open Access Journals (Sweden)

Joshi Deepak

2008-06-01

Full Text Available Abstract Introduction The presentation of cystic fibrosis is dependant upon which organs are affected. Common presentations include chronic respiratory infections and malabsorption. Patients with atypical disease tend to present late in childhood or as adults. Eye manifestations of cystic fibrosis are less well known. Case presentation A 14-year-old Caucasian boy presented with tiredness and difficulty seeing at night, over a period of 6 months. Good vision was only described in bright conditions. There was no history of jaundice, steatorrhea or diarrhoea. Conclusion This is the first reported case of newly diagnosed cystic fibrosis-related liver disease in a teenage boy, whose presenting symptom was night blindness secondary to vitamin A deficiency.

The Incidence of Atypical Femoral Fractures in Patients with Rheumatic Disease: Yamagata Prefectural Committee of Atypical Femoral Fractures (YamaCAFe) Study.

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Takakubo, Yuya; Ohta, Daichi; Ishi, Masaji; Ito, Juji; Oki, Hiroharu; Naganuma, Yasushi; Uno, Tomohiro; Sasaki, Akiko; Akabane, Takeru; Dairaku, Katsuyuki; Goto, Shinichi; Goto, Yasuo; Kanauchi, Yumiko; Kobayashi, Shinji; Nakajima, Taku; Masuda, Keiji; Matsuda, Michiharu; Mura, Nariyuki; Takenouchi, Kenji; Tsuchida, Hiroyuki; Onuma, Yasushi; Shibuya, Junichirou; Seino, Mitsuyoshi; Yamaguchi, Osamu; Hiragami, Ken; Urayama, Yasuhiro; Furukawa, Takashi; Okuda, Shouta; Ogura, Ken; Nakamura, Takeshi; Sasaki, Kan; Konta, Tsuneo; Takagi, Michiaki

2017-08-01

Atypical femoral fractures (AFFs) have been reported to occur with minimal or spontaneous subtrochanteric and femoral shaft fractures with a characteristic transverse pattern, compared with typical femoral fractures in young patients with high-energy trauma. AFFs are related to long-term use of bisphosphonates (BPs), glucocorticoids and rheumatic diseases. We have estimated a blind analysis of AFFs in rheumatic patients receiving BPs and glucocorticoids ordinary over a long time in all Yamagata prefectural area through radiographic examination. The 123 AFFs including suspected cases over six years were collected and reviewed by two independent orthopedic surgeons. We found 86 patients with a total of 99 AFFs between 2009 and 2014 (1.43 cases/100,000 person/year). Of these 99 AFFs, 11 were in 8 rheumatic patients including three patients with bilateral AFFs. The incidence of AFFs in rheumatic patients had trend to increase from 2012. The mean age of all 8 patients was 54.9 years. All 8 patients received BPs and 7/8 received prednisolone (PSL). The mean dose of PSL was 14 mg/day. Compared to patients with unilateral AFFs, those with bilateral AFFs in rheumatic patients were on a higher dose of PSL (20 mg/day vs. 7 mg/day) and had less femoral neck-shaft angle (129° vs. 136°, p rheumatic patients showed a trend to increase from 2012 to 2014 in Yamagata prefecture. Careful management of AFFs is of particular importance in rheumatic patients who have taken high doses of PSL and have small femoral neck-shaft angle.

Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease.

Science.gov (United States)

Fujita, Kengo; Ito, Hidefumi; Nakano, Satoshi; Kinoshita, Yoshimi; Wate, Reika; Kusaka, Hirofumi

2008-10-01

This report concerns an immunohistochemical investigation on RNA-related proteins in the basophilic inclusions (BIs) from patients with adult-onset atypical motor neuron disease. Formalin-fixed, paraffin-embedded sections of the motor cortex and the lumbar spinal cord were examined. The BIs appeared blue in color with H&E and Nissl stain, and pink with methylgreen-pyronin stain. Ribonuclease pretreatment abolished the methylgreen-pyronin staining, suggesting that the BIs contained RNA. Immunohistochemically, the BIs were distinctly labeled with the antibodies against poly(A)-binding protein 1, T cell intracellular antigen 1, and ribosomal protein S6. These proteins are essential constituents of stress granules. In contrast, the BIs were not immunoreactive for ribosomal protein L28 and decapping enzyme 1, which are core components of transport ribonucleoprotein particles and processing bodies, respectively. Moreover, the BIs were not immunopositive for TDP-43. Our results imply that translation attenuation could be involved in the processes of BI formation in this disorder.

Malignant atypical cell in urine cytology: a diagnostic dilemma

Directory of Open Access Journals (Sweden)

Kakkar Nandita

2006-01-01

Full Text Available Abstract Aims The aim of this study was to find out the characteristic morphology of malignant atypical cells which were missed on routine cytology of urine. Materials and methods In this retrospective study, we examined detailed cytomorphology of 18 cases of atypical urinary cytology which were missed on routine examination and were further proved on histopathology as transitional cell carcinoma (TCC of bladder. The cytological features of these cases were compared with 10 cases of benign urine samples. Results There were 11 cases of high grade TCC and 7 cases of low grade TCC on histopathology of the atypical urine samples. Necrosis in the background and necrosed papillae were mostly seen in malignant atypical cells. The comet cells and cells with India ink nuclei (single cells with deep black structure-less nuclei were only observed in malignant atypical cells. The most consistent features in malignant atypical cells were: i high nuclear and cytoplasmic (N/C ratio ii nuclear pleomorphism iii nuclear margin irregularity iv hyperchromasia and v chromatin abnormalities Conclusion The present study emphasizes that nuclear features such as high N/C ratio, hyperchromasia and chromatin abnormalities are particularly useful for assessing the malignant atypical cells. Other cytological features such as comet cells and cells with India ink nuclei are also helpful for diagnosis but have limited value because they are less frequently seen.

Atypical Food Packaging Affects The Persuasive Impact of Product Claims

NARCIS (Netherlands)

van Ooijen, M.L.; Fransen, P.W.J.; Verlegh, P.W.J.; Smit, E.G.

2016-01-01

Atypical food packaging draws attention in the retail environment, and therefore increases product salience. However, until now, no research has focused on how atypical packaging affects the persuasive impact of other food information. In the present study, we propose that atypical packaging

Atypical food packaging affects the persuasive impact of product claims

NARCIS (Netherlands)

van Ooijen, I.; Fransen, M.L.; Verlegh, P.W.J.; Smit, E.G.

Atypical food packaging draws attention in the retail environment, and therefore increases product sal- ience. However, until now, no research has focused on how atypical packaging affects the persuasive impact of other food information. In the present study, we propose that atypical packaging

Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.

Science.gov (United States)

Fink, Adam Z; Gittler, Julia K; Nakrani, Radhika N; Alis, Jonathan; Blumfield, Einat; Levin, Terry L

Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. We review the imaging findings in childhood diseases associated with dermatologic manifestations. Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura). Familiarity with pertinent findings in childhood diseases presenting with dermatologic manifestations in childhood diseases aids the radiologist in confirming the diagnosis and guiding imaging workup. Copyright © 2017 Elsevier Inc. All rights reserved.

Isolation and characterization of an atypical Listeria monocytogenes associated with a canine urinary tract infection

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Listeria monocytogenes, a well-described cause of encephalitis and abortion in ruminants and of food-borne illness in humans, is rarely associated with disease in companion animals. A case of urinary tract infection associated with an atypical, weakly hemolytic L. monocytogenes strain is described i...

Neuromyelitis optica, atypical hemophagocytic lymphohistiocytosis and heterozygous perforin A91V mutation.

Science.gov (United States)

Palterer, Boaz; Brugnolo, Francesca; Sieni, Elena; Barilaro, Alessandro; Parronchi, Paola

2017-10-15

Neuromyelitis optica is an autoimmune demyelinating inflammatory disease characterized by optic neuritis and myelitis with anti-aquaporin 4 antibodies. Hemophagocytic lymphohistiocytosis is a severe systemic inflammatory syndrome that can present in a genetic primary form or secondarily to infective, neoplastic or autoimmune diseases. Our case discusses the first reported case of atypical late-onset hemophagocytic lymphohistiocytosis in a patient with neuromyelitis optica, with multiple triggering factors and carrying the common A91V hypomorphic perforin mutation, that blurs the distinction between primary and secondary forms. Copyright © 2017 Elsevier B.V. All rights reserved.

The usefulness of SPECT and MRI in the diagnosis of atypical parkinsonian syndromes

International Nuclear Information System (INIS)

Skogseid, I.M.; Gerdts, R.; Nyberg-Hansen, R.; Rootwelt, K.; Bakke, S.J.

2001-01-01

Clinico-pathological studies have shown that only three out of four patients with parkinsonism have idiopathic Parkinson's disease. In patients with so-called Parkinson plus syndrome, the degeneration in the brain is more widespread and the variety of neurological signs greater than in Parkinson's disease. The differentiation of these syndromes from Parkinson's disease can be difficult. Single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRl) can be of value in the differential diagnosis of parkinsonism. We present three patients with atypical parkinsonism in whom MRI and SPECT with β-CIT and epidepride was performed in addition to the clinical evaluation. The three patients all had a rapidly developing symmetric akinetic-rigid syndrome that responded poorly to levodopa. MRI showed findings regarded as typical for multiple system atrophy in two patients, but only nonspecific findings in the third patient. SPECT with β-CIT showed a pronounced bilateral and relatively symmetric reduction in the striatal dopaminergic activity in all patients. SPECT with epidepride showed a clearly reduced striatal D 2 -receptor binding bilaterally in only one of the patients. In patients with atypical parkinsonism, MRI and SPECT with β-CIT and epidepride can give valuable support to the clinical diagnosis of a Parkinson plus syndrome

Atypical Presentations of Tularemia.

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Odegaard, Karah; Boersma, Beth; Keegan, James

2017-05-01

Francisella tularensis is a gram-negative coccobacillus that causes a condition commonly referred to as tularemia. There has been a dramatic increase in tularemia cases reported in South Dakota, many of which were challenging to diagnose due to atypical clinical manifestations. We describe an interesting case of pneumonic tularemia and summarize six similar cases, several of which presented with lung nodules suggestive of malignancy. According to the literature, this is only the third outbreak of pneumonic tularemia reported in the U.S. We believe it is important for clinicians to be aware of the increased incidence of tularemia in the area and to be vigilant in the diagnosis and management of these atypically presenting cases. Copyright© South Dakota State Medical Association.

[Atypical cerebellar neurocytoma resembling a hemangioblastoma. A case report].

Science.gov (United States)

Lista Martínez, Olalla; Rivas López, Luis Alfredo; Pombo Otero, Jorge Francisco; Amaro Cendón, Santiago; Bravo García, Christian; Villa Fernández, Juan Manuel

2014-01-01

Through August 2013, 105 cases of intracranial extraventricular neurocytoma (EVN) had been described; 6% were located in cerebellum and 22% were atypical EVN. A rare morphologic form of neurocytoma, atypical EVN has had only 24 cases reported to date. Its prognosis is poorer than the typical central neurocytoma. This case report describes an atypical cerebellar EVN, a form that has not been reported yet, hence the interest of this article. We emphasise its cystic nature and mural nodule, in an infrequent presentation. EVN are low-incidence tumours that we need to take into consideration when making the differential diagnosis of cystic cerebellar lesions with mural nodule. Given that the prognosis of atypical EVNs depends on the atypical nature and on the grade of resection, medical follow up has to be more constant, due to the greater degree of recurrence. Copyright © 2013 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

Amide proton transfer imaging for differentiation of benign and atypical meningiomas

Energy Technology Data Exchange (ETDEWEB)

Joo, Bio [The Armed Forces Capital Hospital, Department of Radiology, Seongnam, Gyeonggi-do (Korea, Republic of); Han, Kyunghwa; Choi, Yoon Seong; Lee, Seung-Koo [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, College of Medicine, Seoul (Korea, Republic of); Ahn, Sung Soo [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, College of Medicine, Seoul (Korea, Republic of); Yonsei University, Department of Radiology, College of Medicine, Seoul (Korea, Republic of); Chang, Jong Hee; Kang, Seok-Gu [Yonsei University College of Medicine, Department of Neurosurgery, Seoul (Korea, Republic of); Kim, Se Hoon [Yonsei University College of Medicine, Department of Pathology, Seoul (Korea, Republic of); Zhou, Jinyuan [Johns Hopkins University School of Medicine, Division of MRI Research, Department of Radiology, Baltimore, MD (United States)

2018-01-15

To investigate the difference in amide proton transfer (APT)-weighted signals between benign and atypical meningiomas and determine the value of APT imaging for differentiating the two. Fifty-seven patients with pathologically diagnosed meningiomas (benign, 44; atypical, 13), who underwent preoperative MRI with APT imaging between December 2014 and August 2016 were included. We compared normalised magnetisation transfer ratio asymmetry (nMTR{sub asym}) values between benign and atypical meningiomas on APT-weighted images. Conventional MRI features were qualitatively assessed. Both imaging features were evaluated by multivariable logistic regression analysis. The discriminative value of MRI with and without nMTR{sub asym} was evaluated. The nMTR{sub asym} of atypical meningiomas was significantly greater than that of benign meningiomas (2.46% vs. 1.67%; P < 0.001). In conventional MR images, benign and atypical meningiomas exhibited significant differences in maximum tumour diameter, non-skull base location, and heterogeneous enhancement. On multivariable logistic regression analysis, high nMTR{sub asym} was an independent predictor of atypical meningiomas (adjusted OR, 11.227; P = 0.014). The diagnostic performance of MRI improved with nMTR{sub asym} for predicting atypical meningiomas. Atypical meningiomas exhibited significantly higher APT-weighted signal intensities than benign meningiomas. The discriminative value of conventional MRI improved significantly when combined with APT imaging for diagnosis of atypical meningioma. (orig.)

Atypical Pneumonia: Updates on Legionella, Chlamydophila, and Mycoplasma Pneumonia.

Science.gov (United States)

Sharma, Lokesh; Losier, Ashley; Tolbert, Thomas; Dela Cruz, Charles S; Marion, Chad R

2017-03-01

Community-acquired pneumonia (CAP) has multiple causes and is associated with illness that requires admission to the hospital and mortality. The causes of atypical CAP include Legionella species, Chlamydophila, and Mycoplasma. Atypical CAP remains a diagnostic challenge and, therefore, likely is undertreated. This article reviews the advancements in the evaluation and treatment of patients and discusses current conflicts and controversies of atypical CAP. Copyright © 2016 Elsevier Inc. All rights reserved.

Rapid diagnosis of hypoglycin A intoxication in atypical myopathy of horses.

Science.gov (United States)

Sander, Johannes; Cavalleri, Jessika-M V; Terhardt, Michael; Bochnia, Mandy; Zeyner, Annette; Zuraw, Aleksandra; Sander, Stefanie; Peter, Michael; Janzen, Nils

2016-03-01

Hypoglycin A (2-amino-3-(2-methylidenecyclopropyl)propanoic acid) is the plant toxin shown to cause atypical myopathy in horses. It is converted in vivo to methylenecyclopropyl acetic acid, which is transformed to a coenzyme A ester that subsequently blocks beta oxidation of fatty acids. Methylenecyclopropyl acetic acid is also conjugated with carnitine and glycine. Acute atypical myopathy may be diagnosed by quantifying the conjugates of methylenecyclopropyl acetic acid plus a selection of acyl conjugates in urine and serum. We describe a new mass spectrometric method for sample volumes of acid in urine, the coefficients of variation for intraday quantification were 2.9% and 3.0%, respectively. The respective values for interday were 9.3% and 8.0%. Methylenecyclopropyl acetyl carnitine was detected as high as 1.18 µmol/L in serum (median: 0.46 µmol/L) and 1.98 mmol/mol creatinine in urine (median: 0.79 mmol/mol creatinine) of diseased horses, while the glycine derivative accumulated up to 1.97 mmol/mol creatinine in urine but was undetectable in most serum samples. In serum samples from horses with atypical myopathy, the intraday coefficients of variation for C4-C8 carnitines and glycines were ≤4.5%. Measured concentrations exceeded those in healthy horses by ~10 to 1,400 times. © 2015 The Author(s).

[sup 123]I-IMP SPECT studies in schizophrenia and atypical psychosis

Energy Technology Data Exchange (ETDEWEB)

Hayashi, Takuji; Suga, Hidemichi (Aichi Medical University, Nagakute (Japan))

1993-05-01

According to the classification of Mitsuda, 23 patients with endogenous psychosis aged 40 years or younger, presenting with hallucination and delusion, were classified as having schizophrenia (n=12) or atypical psychosis (n=11). These patients were studied by single photon emission computed tomography (SPECT) with N-isopropyl-p-I-123-iodoamphetamine (I-123 IMP). Sixteen healthy persons served as controls. Early and delayed SPECT images were obtained 30 min and 4 hr, respectively, after intravenous injection of I-123 IMP. The group of schizophrenic patients had markedly decreased uptake of I-123 in the basal ganglia, as well as the right temporal and left occipital areas on both early and delayed images. In the group of atypical psychosis patients, however, decreased uptake of I-123 was noted in both the right basal ganglia and left occipital area on early images, but none of such findings were seen on delayed images. Regarding the uptake ratio in the frontal area on both early and delayed images, there were significant differences between the two groups. These findings have important implications for the different etiology of both disease types: not only functional disturbance in the frontal area but also irreversible changes may be involved in the occurrence of schizophrenia, and functional disturbance particularly in the right basal ganglia may be involved in the occurrence of atypical psychosis. (N.K.).

78 FR 15368 - Agency Forms Undergoing Paperwork Reduction Act Review

Science.gov (United States)

2013-03-11

... include: Creutzfeldt-Jakob Disease (CJD), Cyclosporiasis, Dengue, Hantavirus, Kawasaki Syndrome... treatment. The data collected will also be used to recommend target areas most in need of vaccinations for... Epidemiologist Dengue 55 182 15/60 Epidemiologist Hantavirus 46 3 20/60 Epidemiologist Kawasaki Syndrome...

Atypical pathogens and challenges in community-acquired pneumonia

African Journals Online (AJOL)

Atypical organisms such as Mycoplasma pneumoniae, Chlamydia pneumoniae, and Legionella pneumophila are implicated in up to 40 percent of cases of community-acquired pneumonia. Antibiotic treatment is empiric and includes coverage for both typical and atypical organisms. Doxycycline, a fluoroquinolone with ...

Computerized tomography findings on schizophrenia and atypical psychosis

International Nuclear Information System (INIS)

Hayashi, Takuji; Watanabe, Toyonobu; Kito, Hiroshi; Sekine, Takeo

1988-01-01

The brain CTs of 54 endogenous psychotics (27 males, 27 females) who were less than 40 years of age and were first adimitted in Aichi Medical University from 1982 to 1986, and 20 controls (10 males, 10 females) were examined. Using Mitsuda's classification, we devided all the cases into 29 schizophrenics (18 males, 11 females) and 25 atypical psychotics (9 males, 16 females). In order to investigate the differences of CT findings between the two patient groups, the 3rd ventricle index (the ratio of 3rd ventricle width to the internal diameter of the skull), Evans'ratio, lateral ventricle brain ratio (VBR), Sylvian fissure to brain ratio, 4th ventricle to cerebellum ratio were determined. Schizophrenics had larger 3rd and lateral ventricles as well as Sylvian fissures when compared to controls, but atypical psychotics had not. Moreover, schizophrenics had larger 3rd and lateral ventricle than atypical psychotics. But in widths of Sylvian fissures there was no statistical significant difference between the two groups. Ventricle enlargements of schizophrenics did not correlate with duration of illness as well as age, and were not results of prior psychiatric treatment such as medication and EST. Therefore the following is suggested that, this abnormal CT findings predate the onset of schizophrenic psychoses. In atypical psychotics the changes of Sylvian fissures correlated with duration of illness, but not with age. Such observations may possibly suggest that recurrence of the illness might finally attain irreversible changes even in atypical psychotics. Finally, the heterogeneity of schizophrenia and the independence of atypical psychosis were also discussed. (author) 53 refs

Atypical Porcine Pestivirus: A Possible Cause of Congenital Tremor Type A-II in Newborn Piglets

NARCIS (Netherlands)

de Groof, Ad; Deijs, Martin; Guelen, Lars; van Grinsven, Lotte; van Os-Galdos, Laura; Vogels, Wannes; Derks, Carmen; Cruijsen, Toine; Geurts, Victor; Vrijenhoek, Mieke; Suijskens, Janneke; van Doorn, Peter; van Leengoed, Leo; Schrier, Carla; van der Hoek, Lia

2016-01-01

Congenital tremor type A-II in piglets has been regarded as a transmissible disease since the 1970s, possibly caused by a very recently-described virus: atypical porcine pestivirus (APPV). Here, we describe several strains of APPV in piglets with clinical signs of congenital tremor (10 of 10 farms

Atypical antipsychotics in the treatment of pathological aggression in children and adolescents: literature review and clinical recommendations

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Eduardo Henrique Teixeira

2013-01-01

Full Text Available Objective: To review the literature about the use of atypical antipsychotics in the treatment of pathological aggression in children and adolescents. Method: The databases MEDLINE, SciELO, and LILACS were searched for publications in Portuguese or English from 1992 to August 2011 using the following keywords: mental disease, child, adolescent, treatment, atypical antipsychotic, aggressive behavior, aggression, and violent behavior. Results: Sixty-seven studies of good methodological quality and clinical interest and relevance were identified. Studies including children and adolescents were relatively limited, because few atypical antipsychotics have been approved by the Food and Drug Administration (FDA. All the medications included in this review (risperidone, olanzapine, quetiapine, ziprasidone, aripiprazole and clozapine have some effectiveness in treating aggression in children and adolescents, and choices should be based on clinical indications and side effects. Conclusions: There are few studies about the effectiveness and safety of atypical antipsychotics for the pediatric population, and further randomized controlled studies with larger groups of patients and more diagnostic categories, such as severe conduct disorder and oppositional defiant disorder, should be conducted to confirm the results reported up to date and to evaluate the impact of long-term use.

The Use of Electroconvulsive Therapy in Atypical Psychotic Presentations

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Vasu, Devi

2007-01-01

Convulsive therapy and its progeny, electroconvulsive therapy (ECT), were originally used for the treatment of catatonic schizophrenia, and there is little doubt that ECT remains an effective intervention for the treatment of schizophrenia. However, current practice tends to favor the use of ECT in severe or treatment refractory affective disorders, and its use in schizophrenia and other nonaffective (atypical) psychotic disorders has become controversial. Case reports have suggested a role for ECT in two specific atypical psychotic disorders: Cotard's syndrome and cycloid psychosis. In this article, we review the atypical psychotic disorders and report a series of five case examples that signify the role of ECT in atypical psychotic presentations, particularly when the symptoms resemble those found in Cotard's syndrome and cycloid psychosis. PMID:20428309

Atypical relapse of hemolytic uremic syndrome after transplantation

NARCIS (Netherlands)

Olie, Karolien H.; Florquin, Sandrine; Groothoff, Jaap W.; Verlaak, René; Strain, Lisa; Goodship, Timothy H. J.; Weening, Jan J.; Davin, Jean-Claude

2004-01-01

Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of

Typical and atypical presentations of aspergilloma

International Nuclear Information System (INIS)

Villajos, M.; Darnell, A.; Gallardo, X.; Castaner, E.; Mata, J. M.; Paedavila, E.

1999-01-01

To show the different forms of radiological presentations of aspergilloma, emphasizing the importance of recognizing the atypical forms. The explorations of 11 patients with aspergilloma were examined retrospectively between 1993 and 1997. These patients were studied using conventional X-rays and computed tomography (CT): Typical and atypical radiological findings were observed. In two patients, who presented recurrent hemoptysis, a percutaneous installation of amphotericin B was carried out with tomographic control. Out of the 11 patients, two were female and nine male. In eight of the cases the radiological findings showed an intercavity injury with different evolutionary forms, while in three of the cases there was a progressive pleural swelling. In the two patients treated pertinaciously, no significant radiological changes were observed, however, neither of them showed hemoptysis again. The pleural swelling adjacent to the cavity and/or the swelling of the cavity wall are atypical radiological presentations of the aspergilloma, that can accompany or precede the appearance of this illness. (Author) 7 refs

Nonmotor Features in Atypical Parkinsonism.

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Bhatia, Kailash P; Stamelou, Maria

2017-01-01

Atypical parkinsonism (AP) comprises mainly multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), which are distinct pathological entities, presenting with a wide phenotypic spectrum. The classic syndromes are now called MSA-parkinsonism (MSA-P), MSA-cerebellar type (MSA-C), Richardson's syndrome, and corticobasal syndrome. Nonmotor features in AP have been recognized almost since the initial description of these disorders; however, research has been limited. Autonomic dysfunction is the most prominent nonmotor feature of MSA, but also gastrointestinal symptoms, sleep dysfunction, and pain, can be a feature. In PSP and CBD, the most prominent nonmotor symptoms comprise those deriving from the cognitive/neuropsychiatric domain. Apart from assisting the clinician in the differential diagnosis with Parkinson's disease, nonmotor features in AP have a big impact on quality of life and prognosis of AP and their treatment poses a major challenge for clinicians. © 2017 Elsevier Inc. All rights reserved.

The neurogenetics of atypical parkinsonian disorders.

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Fogel, Brent L; Clark, Mary C; Geschwind, Daniel H

2014-04-01

Although classic Parkinson disease is the disorder most commonly associated with the clinical feature of parkinsonism, there is in fact a broader spectrum of disease represented by a collection of phenotypically similar neurodegenerative conditions that mimic many of its core features. These atypical parkinsonian disorders most commonly include progressive supranuclear palsy and corticobasal degeneration, disorders both associated with frontotemporal dementia, as well as multiple system atrophy and dementia with Lewy bodies. Although the clinical distinction of these disorders still remains a challenge to physicians, recent advances in genetics are poised to tease apart the differences. Insights into the molecular etiologies underlying these conditions will improve diagnosis, yield a better understanding of the underlying disease pathology, and ultimately lend stimulation to the development of potential treatments. At the same time, the wide range of phenotypes observed from mutations in a single gene warrants broad testing facilitated by advances in DNA sequencing. These expanding genomic approaches, ranging from the use of next-generation sequencing to identify causative or risk-associated gene variations to the study of epigenetic modification linking human genetics to environmental factors, are poised to lead the field into a new age of discovery. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Efficacy of iodine-123-15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid single photon emission computed tomography imaging in detecting myocardial ischemia in children with Kawasaki disease

International Nuclear Information System (INIS)

Hoshina, Masaru; Shiraishi, Hirohiko; Igarashi, Hiroshi; Kikuchi, Yutaka; Ichihashi, Kou; Momoi, Mariko Y.

2003-01-01

To evaluate its efficacy in detecting myocardial ischemia in children, iodine-123-labeled 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) myocardial single photon emission computed tomography (SPECT) imaging was performed in 16 pediatric patients with Kawasaki disease (KD, 11 male, 5 female; mean age and range: 13 years 8 months and 8 years 11 months to 17 years 7 months). Five children with chest pain and no cardiac disease were studied as controls (2 male, 3 female; mean age and range: 13 years 4 months and 9 years 4 months to 17 years 11 months). Selective coronary angiography was also performed in the 16 patients to evaluate the location of coronary stenosis and coronary aneurysms. The SPECT images were expressed as polar maps (Bull's eye maps) and the 'defect' area was defined as where the uptake of BMIPP was less than the standardized BMIPP images of the 5 control children. In the 16 patients, 33 segments had coronary aneurysms and 10 (10/33: 30.3%) had significant coronary stenosis on selective coronary angiography. Nine of the 10 (90%) segments with significant coronary stenosis showed a defect on the BMIPP image whereas only 6 of the 23 (26.1%) segments without coronary stenosis showed a defect on BMIPP imaging. The sensitivity of BMIPP SPECT imaging for detection of coronary stenosis was 90% (9/10) and its specificity was 73.9% (17/23), whereas the sensitivity of 201 Tl SPECT imaging was 80% (8/10) and its specificity was 60% (14/23). There was no significant difference between the BMIPP and 201 Tl SPECT images in either the sensitivity or specificity for the detection of coronary stenosis. In the present series, only one case had discordant BMIPP uptake (BMIPP uptake 201 Tl uptake) in which there was a large coronary aneurysm and re-canalization after complete obstruction at segment 1 of the right coronary artery. This discordant BMIPP uptake reflects the possibility of ischemic but viable myocardium after re-canalization of a large aneurysm in

[Anxiety driven atypical eating disorder in the course of phenylketonuria].

Science.gov (United States)

Rapps, Nora; Mack, Isabelle; Herrmann-Werner, Anne; Zipfel, Stephan; Teufel, Martin

2015-07-01

Phenylketonuria is the most common genetic disease in amino acid metabolism. We report the case of a 22-year old patient with phenylketonuria and psychological symptoms. After early treatment, phenylalanine levels had been controlled and were within target area. Clinical interview and psychometrics showed atypical eating disorder and anxiety disorder. Possible toxic effects and psychological factors may play a role in pathogenesis. Most likely the frequency of eating disorders and anxiety disorders in phenylketonuria is underestimated. © Georg Thieme Verlag KG Stuttgart · New York.

Atypical metatarsal fracture in a patient on long term bisphosphonate therapy

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Pavan Pradhan

2012-01-01

Full Text Available A 24 years old female of cushing disease had undergone adrenelectomy. She was put on alendronate and steroid. After six and a half years she developed pathological fracture subtrochanteric femur. The patient was treated with proximal femoral nailing and the fracture united. 2 years later she developed pain right foot. She was diagnosed as transverse fracture of fifth metatarsal. We report this rare case of atypical metatarsal fracture in a patient on long term bisphosphonate therapy.

Atypical antipsychotics in bipolar disorder: systematic review of randomised trials

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Moore R Andrew

2007-08-01

Full Text Available Abstract Background Atypical antipsychotics are increasingly used for treatment of mental illnesses like schizophrenia and bipolar disorder, and considered to have fewer extrapyramidal effects than older antipsychotics. Methods We examined efficacy in randomised trials of bipolar disorder where the presenting episode was either depression, or manic/mixed, comparing atypical antipsychotic with placebo or active comparator, examined withdrawals for any cause, or due to lack of efficacy or adverse events, and combined all phases for adverse event analysis. Studies were found through systematic search (PubMed, EMBASE, Cochrane Library, and data combined for analysis where there was clinical homogeneity, with especial reference to trial duration. Results In five trials (2,206 patients participants presented with a depressive episode, and in 25 trials (6,174 patients the presenting episode was manic or mixed. In 8-week studies presenting with depression, quetiapine and olanzapine produced significantly better rates of response and symptomatic remission than placebo, with NNTs of 5–6, but more adverse event withdrawals (NNH 12. With mania or mixed presentation atypical antipsychotics produced significantly better rates of response and symptomatic remission than placebo, with NNTs of about 5 up to six weeks, and 4 at 6–12 weeks, but more adverse event withdrawals (NNH of about 22 in studies of 6–12 weeks. In comparisons with established treatments, atypical antipsychotics had similar efficacy, but significantly fewer adverse event withdrawals (NNT to prevent one withdrawal about 10. In maintenance trials atypical antipsychotics had significantly fewer relapses to depression or mania than placebo or active comparator. In placebo-controlled trials, atypical antipsychotics were associated with higher rates of weight gain of ≥7% (mainly olanzapine trials, somnolence, and extrapyramidal symptoms. In active controlled trials, atypical antipsychotics

Real frequency of ordinary and atypical sub-trochanteric and diaphyseal fractures in France based on X-rays and medical file analysis.

Science.gov (United States)

Beaudouin-Bazire, Constance; Dalmas, Noémie; Bourgeois, Julie; Babinet, Antoine; Anract, Philippe; Chantelot, Christophe; Farizon, Frédéric; Chopin, Florence; Briot, Karine; Roux, Christian; Cortet, Bernard; Thomas, Thierry

2013-03-01

Atypical sub-trochanteric and femoral shaft fractures have been reported in patients treated with bisphosphonates. Their incidence has been determined from registered data analysis using international codes. Therefore, the aim of our study was to estimate the real frequency of typical and atypical sub-trochanteric or diaphyseal fractures, based on radiological and clinical data compared to registered data. In the registers of three large French University Hospitals, patients identified with International Classification of Diseases, 10th Revision diagnosis codes for sub-trochanteric or diaphyseal fracture were selected. Frequencies of ordinary and atypical fractures were calculated after both registered data, radiological and clinical files analysis. Among the 4592 patients hospitalized for a femoral fracture over 5 years, 574 were identified to have had a sub-trochanteric or femoral shaft fracture. 47.7% of the sub-trochanteric and femoral shaft fractures were misclassified, predominantly in the sub-trochanteric fractures subset. 12 patients had an atypical fracture (4% of the sub-trochanteric and femoral shaft fractures) and 11 fractures presented radiological features of atypical fractures, whereas clinical files analysis revealed they were pathological or traumatic fractures. Atypical fractures frequency is very low. Because of their low frequency and the unreliability of registered databases, the risk of atypical fractures is very difficult to estimate retrospectively. A prospective study is needed to clarify the risk factors associated with these fractures. Copyright © 2012 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Estudo de validação das equações de Tanaka e de Kawasaki para estimar a excreção diária de sódio através da coleta da urina casual

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José Geraldo Mill

2015-12-01

Full Text Available RESUMO: Objetivo: Validar as fórmulas de Tanaka e Kawasaki para cálculo do consumo de sal pela relação sódio/creatinina na urina casual. Métodos: Foram estudados 272 adultos (20 - 69 anos, 52,6% de mulheres com coleta urinária de 24 h e duas coletas casuais no mesmo dia (em jejum - casual 1 - e fora do jejum - casual 2. Antropometria, pressão arterial e coleta de sangue foram obtidos no mesmo dia. A concordância entre o consumo de sal estimado pela urina de 24 h e pela urina casual foi feita por Pearson (r e Bland & Altman. Resultados: O consumo médio de sal medido pela urina de 24 h foi de 10,4 ± 5,3 g/dia. A correlação entre a excreção de sódio na urina de 24 h e a estimada pela urina casual 1 ou 2, respectivamente, foi apenas moderada, tanto por Tanaka (r = 0,51 e r = 0,55; p < 0,001 como por Kawasaki (r = 0,52 e r = 0,54; p < 0,001. Observa-se subestimação crescente dos valores estimados em relação ao medido com o aumento do consumo de sal pela fórmula de Tanaka e, ao contrário, superestimação ao usar a fórmula de Kawasaki. As fórmulas estimam adequadamente o consumo diário de sal (diferença entre sal medido e estimado de, no máximo, 1 g/dia somente com consumo entre 9 - 12 g/dia (Tanaka e 12 - 18 g/dia (Kawasaki. Conclusão: A coleta de urina casual estima adequadamente o consumo de sal apenas nos indivíduos próximos à média populacional.

Clinical diagnostic criteria for typical and atypical variants of Rett syndrome in children

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S. Ya. Volgina

2016-01-01

Full Text Available Rett Syndrome is one of the most socially significant neuropsychiatric hereditary diseases in children. This syndrome is mainly found in girls: its frequency is 1:10000–15000. Currently mutations in X-linked gene MESR2 considered as the main cause of the syndrome. Diagnosis of typical and atypical variants of the syndrome is based on the use of clinical criteria, determining gene mutations МЕСР2, CDKL5 and FOXG1. In 2010, the Expert Consortium for Rett syndrome have revised the existing diagnostic criteria for the syndrome and come to a new consensus. If there is a regression of mental development for the diagnosis of Rett syndrome exemplary embodiment only four basic criteria for the diagnosis of atypical variant — two of the four main criteria, and five of the eleven additional criteria. 

Atypical bovine interstitial pneumonia in a semi-intensive beef cattle system

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Ana Carolina Barreto Coelho

2017-11-01

Full Text Available ABSTRACT: The aim of the present study was to describe an outbreak of atypical interstitial pneumonia affecting 15 Angus cattle aged 7-30 months in the south of Rio Grande do Sul. The clinical course of the disease was approximately 1-3 days. The cattle were kept in the native field and had received feed containing the remains of wafers from a food factory before the outbreak. Clinical signs included dyspnea and abdominal breathing, with a clinical evolution period of up to 3 days. Nine animals presented apathy and respiratory difficulty and died within 24h, while three animals exhibited a clinical evolution period of 36-48h. Three other animals died without any clinical signs. The main macroscopic lesions were observed in the lungs, which did not collapse at the opening of the thoracic cavity and showed a rubbery consistency. On the cut surface, the organs were firm and dark red, with marked distension of the interlobular septa due to edema. Histopathological analysis revealed thickening of the interlobular pulmonary septa; interstitial edema and emphysema; mild mononuclear cell, eosinophil, and neutrophil infiltration; areas of type II pneumocyte proliferation, and thickening of the alveolar walls. Fibrin was observed in the alveolar lumen with the formation of hyaline membranes. On the basis of macroscopic and histological examinations and epidemiological data, a final diagnosis of atypical interstitial pneumonia was made. Although, the cause of the disease has not been identified, it is believed that the bran wafer meal contained ingredients that were not suitable for animals, consequently triggering the disease.

Identification of methylenecyclopropyl acetic acid in serum of European horses with atypical myopathy.

Science.gov (United States)

Votion, D-M; van Galen, G; Sweetman, L; Boemer, F; de Tullio, P; Dopagne, C; Lefère, L; Mouithys-Mickalad, A; Patarin, F; Rouxhet, S; van Loon, G; Serteyn, D; Sponseller, B T; Valberg, S J

2014-03-01

It is hypothesised that European atypical myopathy (AM) has a similar basis as seasonal pasture myopathy in North America, which is now known to be caused by ingestion of hypoglycin A contained in seeds from the tree Acer negundo. Serum from horses with seasonal pasture myopathy contained the conjugated toxic metabolite of hypoglycin A, methylenecyclopropyl acetic acid (MCPA). Retrospective study on archived samples. 1) To determine whether MCPA-carnitine was present in serum of European horses confirmed to have AM; 2) to determine whether Acer negundo or related Acer species were present on AM pastures in Europe. Concentrations of MCPA-carnitine were analysed in banked serum samples of 17 AM horses from Europe and 3 diseased controls (tetanus, neoplasia and exertional rhabdomyolysis) using tandem mass spectrometry. Atypical myopathy was diagnosed by characteristic serum acylcarnitine profiles. Pastures of 12 AM farms were visited by experienced botanists and plant species were documented. Methylenecyclopropyl acetic acid-carnitine at high concentrations (20.39 ± 17.24 nmol/l; range 0.95-57.63 nmol/l; reference: <0.01 nmol/l) was identified in serum of AM but not disease controls (0.00 ± 0.00 nmol/l). Acer pseudoplatanus but not Acer negundo was present on all AM farms. Atypical myopathy in Europe, like seasonal pasture myopathy in North America, is highly associated with the toxic metabolite of hypoglycin A, MCPA-carnitine. This finding coupled with the presence of a tree of which seeds are known to also contain hypoglycin A indicates that ingestion of Acer pseudoplatanus is the probable cause of AM. This finding has major implications for the prevention of AM. © 2013 EVJ Ltd.

A case of atypical adult-onset tic disorder.

Science.gov (United States)

Colosimo, Carlo

2015-04-01

The differential diagnosis of adult tic disorder is complex, and several common and uncommon causes have to be taken into consideration. A 30-year-old man came to our movement disorders clinic with multiple tics which had begun insidiously about 10 years earlier. No family history was reported, but his 65-year-old otherwise healthy father also had very subtle involuntary movements. A diagnosis of atypical Gilles de la Tourette syndrome was made. However, the neurological and psychiatric symptoms of the patient rapidly progressed over the following 2 years, resulting in increasingly severe involuntary movements and profound mood disorder. Further diagnostic tests were performed, and a genetic screening for Huntington disease revealed 45 repeats of the CAG nucleotide in the IT-15 gene. This case underlines the marked phenotypic variability of Huntington disease at presentation, including the presence of involuntary movements different from chorea and possibility of an apparently sporadic disorder.

Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment

Science.gov (United States)

... teratoid/rhabdoid tumor. There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor. The extent or spread ... different types of treatment for patients with central nervous system atypical teratoid/rhabdoid tumor. Different types of treatment ...

Atypical centrioles during sexual reproduction.

Science.gov (United States)

Avidor-Reiss, Tomer; Khire, Atul; Fishman, Emily L; Jo, Kyoung H

2015-01-01

Centrioles are conserved, self-replicating, microtubule-based, 9-fold symmetric subcellular organelles that are essential for proper cell division and function. Most cells have two centrioles and maintaining this number of centrioles is important for animal development and physiology. However, how animals gain their first two centrioles during reproduction is only partially understood. It is well established that in most animals, the centrioles are contributed to the zygote by the sperm. However, in humans and many animals, the sperm centrioles are modified in their structure and protein composition, or they appear to be missing altogether. In these animals, the origin of the first centrioles is not clear. Here, we review various hypotheses on how centrioles are gained during reproduction and describe specialized functions of the zygotic centrioles. In particular, we discuss a new and atypical centriole found in sperm and zygote, called the proximal centriole-like structure (PCL). We also discuss another type of atypical centriole, the "zombie" centriole, which is degenerated but functional. Together, the presence of centrioles, PCL, and zombie centrioles suggests a universal mechanism of centriole inheritance among animals and new causes of infertility. Since the atypical centrioles of sperm and zygote share similar functions with typical centrioles in somatic cells, they can provide unmatched insight into centriole biology.

Atypical Centrioles During Sexual Reproduction

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Tomer eAvidor-Reiss

2015-04-01

Full Text Available Centrioles are conserved, self-replicating, microtubule-based 9-fold symmetric subcellular organelles that are essential for proper cell division and function. Most cells have two centrioles and maintaining this number of centrioles is important for animal development and physiology. However, how animals gain their first two centrioles during reproduction is only partially understood. It is well established that in most animals, the centrioles are contributed to the zygote by the sperm. However, in humans and many animals, the sperm centrioles are modified in their structure and protein composition, or they appear to be missing altogether. In these animals, the origin of the first centrioles is not clear. Here, we review various hypotheses on how centrioles are gained during reproduction and describe specialized functions of the zygotic centrioles. In particular, we discuss a new and atypical centriole found in sperm and zygote, the proximal centriole-like structure (PCL. We also discuss another type of atypical centriole, the zombie centriole, which is degenerated but functional. Together, the presence of centrioles, PCL, and zombie centrioles suggests a universal mechanism of centriole inheritance among animals and new causes of infertility. Since the atypical centrioles of sperm and zygote share similar functions with typical centrioles in somatic cells, they can provide unmatched insight into centriole biology.

Atypical Papular Purpuric Eruption Induced by Parvovirus B19 Infection

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Şeyma Kayalı

2016-03-01

Full Text Available Parvovirus B19 infection’s most common dermatological manifestation is erythema infectiosum as also known the fifth disease. Rare clinical presentations of parvovirus B 19 like papulopurpuric gloves and socks syndrome and acropetechial syndrome has also been described re­cently. This study presents report of a case with atypical feature and distribution of rash due to parvovirus B19 in­fection. We want to emphasize that pediatricians should consider parvovirus B19 infection of any patient who has leukopenia presenting with petechial/purpuric eruption of an unclear origin.

Physical and Psychological Morbidity in Adolescents With Atypical Anorexia Nervosa.

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Sawyer, Susan M; Whitelaw, Melissa; Le Grange, Daniel; Yeo, Michele; Hughes, Elizabeth K

2016-04-01

Adolescents with atypical anorexia nervosa (AN) have lost significant weight but are not underweight. This study aimed to describe the physical and psychological morbidity of adolescents diagnosed with atypical AN, and to compare them with underweight adolescents with AN. All first presentations of atypical AN (n = 42) and full-threshold AN (n = 118) to a specialist pediatric eating disorder program between July 2010 and June 2014 were examined. Diagnosis was assessed by using the Eating Disorder Examination and anthropometric measurement. Psychological morbidity measures included eating and weight concerns, bingeing, purging, compulsive exercise, and psychiatric comorbidity. Compared with AN, more adolescents with atypical AN were premorbidly overweight or obese (71% vs 12%). They had lost more weight (17.6 kg vs 11.0 kg) over a longer period (13.3 vs 10.2 months). There was no significant difference in the frequency of bradycardia (24% vs 33%;) or orthostatic instability (43% vs 38%). We found no evidence of a difference in frequency of psychiatric comorbidities (38% vs 45%) or suicidal ideation (43% vs 39%). Distress related to eating and body image was more severe in atypical AN. Atypical AN considerably affects physical and psychological functioning, despite adolescents presenting within or above the normal weight range. There was little evidence that the morbidity of adolescents with atypical AN was any less severe than that of adolescents with full-threshold AN. The findings support the need for vigilance around weight loss in adolescents, regardless of body size. Copyright © 2016 by the American Academy of Pediatrics.

'Atypical' bacteria are a common cause of community-acquired ...

African Journals Online (AJOL)

Objectives. To assess the proportion of cases of community· acquired pneumonia caused by 'atypical' bacteria, inclUding the recently discovered Chlamydia pneumoniae, and to compare the clinical, radiographic and laboratory features of patients with and without 'atypical' bacteria. Methods. A prospective serological ...

[Atypical manifestations in familial type 1 Waardenburg syndrome].

Science.gov (United States)

Sans, B; Calvas, P; Bazex, J

1998-01-01

Waardenburg syndrome is an uncommon genetic disorder. Four clinical types are recognized. Three responsible genes have been identified (PAX 3: for type I syndrome, MITF and EDN3 for types II and IV respectively). We report the case of a patient with Waardenburg type I morphotype who had atypical neurological manifestations. Decisive elements for diagnosis were the presence of Waardenburg syndrome in the family and, in affected kin, a mutation causing a shift in PAX 3 gene reading. This case confirms the variability of Waardenburg signs within one family. The association of unusual neurological manifestations in the proband suggested that Vogt Koyanagi Harada disease may have been associated and may show some relationship with familial Waardenburg syndrome.

Congenital infection with atypical porcine pestivirus (APPV) is associated with disease and viral persistence.

Science.gov (United States)

Schwarz, Lukas; Riedel, Christiane; Högler, Sandra; Sinn, Leonie J; Voglmayr, Thomas; Wöchtl, Bettina; Dinhopl, Nora; Rebel-Bauder, Barbara; Weissenböck, Herbert; Ladinig, Andrea; Rümenapf, Till; Lamp, Benjamin

2017-01-06

In 2013, several Austrian piglet-producing farms recorded outbreaks of action-related repetitive myoclonia in newborn piglets ("shaking piglets"). Malnutrition was seen in numerous piglets as a complication of this tremor syndrome. Overall piglet mortality was increased and the number of weaned piglets per sow decreased by more than 10% due to this outbreak. Histological examination of the CNS of affected piglets revealed moderate hypomyelination of the white substance in cerebellum and spinal cord. We detected a recently discovered pestivirus, termed atypical porcine pestivirus (APPV) in all these cases by RT-PCR. A genomic sequence and seven partial sequences were determined and revealed a 90% identity to the US APPV sequences and 92% identity to German sequences. In confirmation with previous reports, APPV genomes were identified in different body fluids and tissues including the CNS of diseased piglets. APPV could be isolated from a "shaking piglet", which was incapable of consuming colostrum, and passaged on different porcine cells at very low titers. To assess the antibody response a blocking ELISA was developed targeting NS3. APPV specific antibodies were identified in sows and in PCR positive piglets affected by congenital tremor (CT). APPV genomes were detected continuously in piglets that gradually recovered from CT, while the antibody titers decreased over a 12-week interval, pointing towards maternally transmitted antibodies. High viral loads were detectable by qRT-PCR in saliva and semen of infected young adults indicating a persistent infection.

Molecular characterization of atypical antigenic variants of canine rabies virus reveals its reintroduction by wildlife vectors in southeastern Mexico.

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Garcés-Ayala, Fabiola; Aréchiga-Ceballos, Nidia; Ortiz-Alcántara, Joanna M; González-Durán, Elizabeth; Pérez-Agüeros, Sandra I; Méndez-Tenorio, Alfonso; Torres-Longoria, Belem; López-Martínez, Irma; Hernández-Rivas, Lucía; Díaz-Quiñonez, José Alberto; Ramírez-González, José Ernesto

2017-12-01

Rabies is an infectious viral disease that is practically always fatal following the onset of clinical signs. In Mexico, the last case of human rabies transmitted by dogs was reported in 2006 and canine rabies has declined significantly due to vaccination campaigns implemented in the country. Here we report on the molecular characterization of six rabies virus strains found in Yucatan and Chiapas, remarkably, four of them showed an atypical reaction pattern when antigenic characterization with a reduced panel of eight monoclonal antibodies was performed. Phylogenetic analyses on the RNA sequences unveiled that the three atypical strains from Yucatan are associated with skunks. Analysis using the virus entire genome showed that they belong to a different lineage distinct from the variants described for this animal species in Mexico. The Chiapas atypical strain was grouped in a lineage that was considered extinct, while the others are clustered within classic dog variants.

Evidence for Broadening Criteria for Atypical Depression Which May Define a Reactive Depressive Disorder.

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Silverstein, Brett; Angst, Jules

2015-01-01

Objective. Arguing that additional symptoms should be added to the criteria for atypical depression. Method. Published research articles on atypical depression are reviewed. Results. (1) The original studies upon which the criteria for atypical depression were based cited fatigue, insomnia, pain, and loss of weight as characteristic symptoms. (2) Several studies of DSM depressive criteria found patients with atypical depression to exhibit high levels of insomnia, fatigue, and loss of appetite/weight. (3) Several studies have found atypical depression to be comorbid with headaches, bulimia, and body image issues. (4) Most probands who report atypical depression meet criteria for "somatic depression," defined as depression associated with several of disordered eating, poor body image, headaches, fatigue, and insomnia. The gender difference in prevalence of atypical depression results from its overlap with somatic depression. Somatic depression is associated with psychosocial measures related to gender, linking it with the descriptions of atypical depression as "reactive" appearing in the studies upon which the original criteria for atypical depression were based. Conclusion. Insomnia, disordered eating, poor body image, and aches/pains should be added as criteria for atypical depression matching criteria for somatic depression defining a reactive depressive disorder possibly distinct from endogenous melancholic depression.

Evidence for Broadening Criteria for Atypical Depression Which May Define a Reactive Depressive Disorder

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Brett Silverstein

2015-01-01

Full Text Available Objective. Arguing that additional symptoms should be added to the criteria for atypical depression. Method. Published research articles on atypical depression are reviewed. Results. (1 The original studies upon which the criteria for atypical depression were based cited fatigue, insomnia, pain, and loss of weight as characteristic symptoms. (2 Several studies of DSM depressive criteria found patients with atypical depression to exhibit high levels of insomnia, fatigue, and loss of appetite/weight. (3 Several studies have found atypical depression to be comorbid with headaches, bulimia, and body image issues. (4 Most probands who report atypical depression meet criteria for “somatic depression,” defined as depression associated with several of disordered eating, poor body image, headaches, fatigue, and insomnia. The gender difference in prevalence of atypical depression results from its overlap with somatic depression. Somatic depression is associated with psychosocial measures related to gender, linking it with the descriptions of atypical depression as “reactive” appearing in the studies upon which the original criteria for atypical depression were based. Conclusion. Insomnia, disordered eating, poor body image, and aches/pains should be added as criteria for atypical depression matching criteria for somatic depression defining a reactive depressive disorder possibly distinct from endogenous melancholic depression.

Delayed Recurrence of Atypical Pulmonary Carcinoid Cluster: A Rare Occurrence

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Salim Surani

2014-01-01

Full Text Available Carcinoid is one of the most common tumors of the gastrointestinal tract followed by the tracheobronchial tree. Bronchial carcinoid compromises 20% of total carcinoid and accounts for 1–5% of pulmonary malignancies. Carcinoid can be typical or atypical, with atypical carcinoid compromises 10% of the carcinoid tumors. Carcinoid usually presents as peripheral lung lesion or solitary endobronchial abnormality. Rarely it can present as multiple endobronchial lesion. We hereby present a rare case of an elderly gentleman who had undergone resection of right middle and lower lobe of lung for atypical carcinoid. Seven years later he presented with cough. CT scan of chest revealed right hilar mass. Flexible bronchoscopy revealed numerous endobronchial polypoid lesions in the tracheobronchial tree. Recurrent atypical carcinoid was then confirmed on biopsy.

Extracutaneous atypical syphilis in HIV-infected patients.

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Prieto, Paula; Imaz, Arkaitz; Calatayud, Laura; García, Olga; Saumoy, María; Podzamczer, Daniel

2017-12-07

We describe a series of cases of syphilis with atypical extracutaneous clinical presentation diagnosed in HIV-infected patients. Retrospective observational study. All cases of syphilis diagnosed in HIV-infected patients during the period between June 2013 and June 2016 in a tertiary hospital of the Barcelona metropolitan area were analysed. A total of 71 cases of syphilis were diagnosed, 32 of them presenting with clinical signs or symptoms. Seven of these cases (9.8% of the total and 21.8% of the symptomatic cases) had atypical presentations with extracutaneous involvement: ocular (4), gastric (1), multiple hepatic abscesses (1) and generalised adenopathies (1). Patients were treated with intramuscular or intravenous penicillin and the clinical and serological evolution was good in all of them. Extracutaneous atypical clinical presentations were observed in 21.8% of symptomatic cases of syphilis in HIV+ patients with ocular involvement being the most freqent. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

Globalization and deregulation: does flexicuritiy protect atypically employed?

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Seifert, Hartmut; Tangian, Andranik S.

2006-01-01

"Hitherto, discussion of flexicurity has focused on normal employment (permanent full-time), with atypical work receiving only cursory attention. Nevertheless, the most affected are just atypically employed (= other than normally employed). To monitor effects of flexicurity policies in Europe, flexicurity indices are constructed from: (a) scores of the strictness of employment protection legislation provided by the OECD, (b) qualitative juridical data on social security benefits (unemployment...

The Efficacy of Acute Electroconvulsive Therapy in Atypical Depression

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Husain, Mustafa M.; McClintock, Shawn M.; Rush, A. John; Knapp, Rebecca G.; Fink, Max; Rummans, Teresa A.; Rasmussen, Keith; Claassen, Cynthia; Petrides, Georgios; Biggs, Melanie M.; Mueller, Martina; Sampson, Shirlene; Bailine, Samuel H.; Lisanby, Sarah H.; Kellner, Charles H.

2013-01-01

Objective This study examined the characteristics and outcomes of patients with major depressive disorder (MDD), with or without atypical features, who were treated with acute bilateral electroconvulsive therapy (ECT). Method Analyses were conducted with 489 patients who met DSM-IV criteria for MDD. Subjects were identified as typical or atypical on the basis of the Structured Clinical Interview for DSM-IV obtained at baseline prior to ECT. Depression symptom severity was measured by the 24-item Hamilton Rating Scale for Depression (HAM-D24) and the 30-item Inventory of Depressive Symptomatology–Self-Report (IDS-SR30). Remission was defined as at least a 60% decrease from baseline in HAM-D24 score and a total score of 10 or below on the last 2 consecutive HAM-D24 ratings. The randomized controlled trial was performed from 1997 to 2004. Results The typical (N = 453) and atypical (N = 36) groups differed in several sociodemographic and clinical variables including gender (p = .0071), age (p = .0005), treatment resistance (p = .0014), and age at first illness onset (p < .0001) and onset of current episode (p = .0008). Following an acute course of bilateral ECT, a considerable portion of both the typical (67.1%) and the atypical (80.6%) groups reached remission. The atypical group was 2.6 (95% CI = 1.1 to 6.2) times more likely to remit than the typical group after adjustment for age, psychosis, gender, clinical site, and depression severity based on the HAM-D24. Conclusion Acute ECT is an efficacious treatment for depressed patients with typical or atypical symptom features. PMID:18278988

Atypical real estate objects: legal regime and control system

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Voskresenskaya Elena

2017-01-01

Full Text Available The legal concept of immovable things raises controversy in legal practice. Determining and understanding the definition of real estate, the complexity and diversity of these objects, a growing appearance of so-called atypical properties (such as sport stadiums, roads, boreholes, analyzing legislation and judicial practice of this field – all these issues call for a deep study of this topic. There is a conflicting arbitration practice, the subject of which is the learning of the legal nature of atypical real estate (for instance, asphalt playgrounds, car parks, fences, wells. The object of the research is the learning of the legal status of atypical real estate.

Development of a low grade lymphoma in the mastoid bone in a patient with atypical Cogan’s syndrome: A case report

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Chris Kalogeropoulos

2015-05-01

Full Text Available Cogan’s syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. It is considered as an autoimmune disease. We present the first case in the literature of a 67 year old woman with the development of low grade non-Hodgkin lymphoma (NHL in the mastoid bone in a pre-existing history of atypical Cogan’s syndrome. The anatomical development of NHL was to a “target” organ of Cogan’s syndrome, which is the inner ear.

Atypical visual loss in giant cell arteritis

DEFF Research Database (Denmark)

Thystrup, Jan Deichmann; Knudsen, G M; Mogensen, A M

1994-01-01

Three patients with atypical ocular involvement due to histologically verified giant cell arteritis are reported. Prior to diagnosis, the first patient had periods of amaurosis fugax. He presented with normal vision. In spite of high-dose systemic corticosteroid therapy, he became blind in the te......Three patients with atypical ocular involvement due to histologically verified giant cell arteritis are reported. Prior to diagnosis, the first patient had periods of amaurosis fugax. He presented with normal vision. In spite of high-dose systemic corticosteroid therapy, he became blind...

Aneurysmal coronary artery disease: An overview

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ElGuindy, Mohamed S.

Aneurysmal coronary artery disease (ACAD) comprises both coronary artery aneurysms (CAA) and coronary artery ectasia (CAE). The reported prevalence of ACAD varies widely from 0.2 to 10%, with male predominance and a predilection for the right coronary artery (RCA). Atherosclerosis is the commonest cause of ACAD in adults, while Kawasaki disease is the commonest cause in children and adolescents, as well as in the Far East. Most patients are asymptomatic, but when symptoms do exist, they are usually related to myocardial ischemia. Coronary angiography is the mainstay of diagnosis, but follow up is best achieved using noninvasive imaging that does not involve exposure to radiation. The optimal management strategy in patients with ACAD remains controversial. Medical therapy is indicated for the vast majority of patients and includes antiplatelets and/or anticoagulants. Covered stents effectively limit further expansion of the affected coronary segments. Surgical ligation, resection, and coronary artery bypass grafting are appropriate for large lesions and for associated obstructive coronary artery disease. PMID:29564347

Atypical PKC-iota Controls Stem Cell Expansion via Regulation of the Notch Pathway

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In Kyoung Mah

2015-11-01

Full Text Available The number of stem/progenitor cells available can profoundly impact tissue homeostasis and the response to injury or disease. Here, we propose that an atypical PKC, Prkci, is a key player in regulating the switch from an expansion to a differentiation/maintenance phase via regulation of Notch, thus linking the polarity pathway with the control of stem cell self-renewal. Prkci is known to influence symmetric cell division in invertebrates; however a definitive role in mammals has not yet emerged. Using a genetic approach, we find that loss of Prkci results in a marked increase in the number of various stem/progenitor cells. The mechanism used likely involves inactivation and symmetric localization of NUMB, leading to the activation of NOTCH1 and its downstream effectors. Inhibition of atypical PKCs may be useful for boosting the production of pluripotent stem cells, multipotent stem cells, or possibly even primordial germ cells by promoting the stem cell/progenitor fate.

Zoonotic atypical pneumonia due to Chlamydophila psittaci: First reported psittacosis case in Taiwan

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Yu-Jen Cheng

2013-07-01

Full Text Available Human psittacosis caused by Chlamydophila psittaci is one of the most common zoonotic atypical pneumonias featuring pulmonary as well as extrapulmonary infections. Most of the cases involve avian contact history especially with psittacine birds. Herein we report a 44-year-old male patient displaying atypical pneumonia symptoms of intermittent fever, dry cough, chest pain, dyspnea, headache, hepatitis, and hyponatremia. He had two sick cockatiels, one of which had died a month previously. A microimmunofluorescence test was performed to check the serum antibody levels against Chlamydophila psittaci. The serum IgM titer showed positive titer of 1:256, 1:256, and 1:128 on Days 11, 23, and 43 after disease onset, respectively. His fever subsided soon and clinical symptoms improved after minocycline was administrated on Day 12. The psittacosis case was confirmed by history of psittacine bird contact, clinical symptoms, treatment response, and positive IgM titer. To our knowledge, this is the first report of a psittacosis case in Taiwan.

Atypical sonographic patterns of fibroadenoma of the breast : pathologic correlation

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Kook, Shin Ho; Kim, Myung Sook; Pae, Won Kil [Kangbuk Samsung Hospital, Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

1999-03-01

To correlate the atypical sonographic patterns of fibroadenoma of the breast with the pathologic findings. Among 203 surgically proven 43 which were sonographically atypical fibroadenomas, were retrospectively reviewed. The diagnostic criteria for atypical variety, as seen on sonography, were an ill-defined margin, microlobulated or irregular shape, heterogeneous internal echo-pattern, posterior shadowing, microcalcification, and clefts. The atypical sonographic patterns of these 43 fibroadenomas were analysed and compared with the pathologic findings. Among 43 lesions, ill-defined margins or irregular shapes were seen in 15 cases, heterogeneous internal echo-patterns in 27, posterior attenuation in nine, and clefts in seven. Thirty-seven (86%) of the 43 were predominantly ductal or had a mixed ductal and stromal component. Eleven (73.3%) of fifteen ill-defined margin or irregular shaped lesions were caused by interdigitation of surrounding normal breast parenchyma and mass. Twenty two (81.5%) of 27 heterogeneous internal echo-pat-terns were related to dilated ducts, phyllodes features, collagen bundles, adenosis, microcalcification, or fat vacuoles. Eight (88.9%) of nine posterior attenuations were caused by collagen bundles, microcalcification, ductal proliferation or dilatation. All seven cases showing clefts revealed phyllodes features and dilated ducts. Most atypical fibroadenomas had a predominantly ductal or mixed component. Ill-defined margin or irregular shape was mainly due to interdigitation of normal surrounding parenchyma. Variable histologic features were related to the heterogeneous internal echo-pattern, posterior shadowing, and the clefts revealed by atypical sonographic findings.

Atypical sonographic patterns of fibroadenoma of the breast : pathologic correlation

International Nuclear Information System (INIS)

Kook, Shin Ho; Kim, Myung Sook; Pae, Won Kil

1999-01-01

To correlate the atypical sonographic patterns of fibroadenoma of the breast with the pathologic findings. Among 203 surgically proven 43 which were sonographically atypical fibroadenomas, were retrospectively reviewed. The diagnostic criteria for atypical variety, as seen on sonography, were an ill-defined margin, microlobulated or irregular shape, heterogeneous internal echo-pattern, posterior shadowing, microcalcification, and clefts. The atypical sonographic patterns of these 43 fibroadenomas were analysed and compared with the pathologic findings. Among 43 lesions, ill-defined margins or irregular shapes were seen in 15 cases, heterogeneous internal echo-patterns in 27, posterior attenuation in nine, and clefts in seven. Thirty-seven (86%) of the 43 were predominantly ductal or had a mixed ductal and stromal component. Eleven (73.3%) of fifteen ill-defined margin or irregular shaped lesions were caused by interdigitation of surrounding normal breast parenchyma and mass. Twenty two (81.5%) of 27 heterogeneous internal echo-pat-terns were related to dilated ducts, phyllodes features, collagen bundles, adenosis, microcalcification, or fat vacuoles. Eight (88.9%) of nine posterior attenuations were caused by collagen bundles, microcalcification, ductal proliferation or dilatation. All seven cases showing clefts revealed phyllodes features and dilated ducts. Most atypical fibroadenomas had a predominantly ductal or mixed component. Ill-defined margin or irregular shape was mainly due to interdigitation of normal surrounding parenchyma. Variable histologic features were related to the heterogeneous internal echo-pattern, posterior shadowing, and the clefts revealed by atypical sonographic findings

Prognostic significance of atypical papillary urothelial hyperplasia.

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Swierczynski, Sharon L; Epstein, Jonathan I

2002-05-01

Typical papillary hyperplasia, a recently recognized precursor lesion to low-grade papillary urothelial neoplasms, consists of undulating folds of cytologically benign urothelium. Well-developed, branching fibrovascular cores of a papillary neoplasm are not evident. We have noted lesions with the architectural pattern of papillary hyperplasia; however, the overlying urothelium demonstrated varying degrees of cytologic atypia. We identified 15 cases of atypical papillary hyperplasia (13 males, 2 females, age 55 to 92) with overlying urothelium showing cytologic atypia. Of these cases, 8 (53%) were received in consultation. Of the 15 cases, 8 exhibited overlying flat carcinoma in situ (CIS), 4 had overlying dysplasia, and 3 were transitional between papillary hyperplasia with atypia and the earliest lesions of papillary neoplasia. Of these cases, 5 patients had multiple specimens with atypical papillary hyperplasia (range, 2 to 8) over time. Concurrent to the diagnosis of atypical papillary hyperplasia, there were 25 different urothelial lesions: CIS (n = 11), papilloma (n = 1), papillary neoplasm of low malignant potential with CIS (n = 1), high-grade papillary urothelial carcinoma (n = 10; 3 with CIS), small-cell carcinoma (n = 1), and infiltrating urothelial carcinoma (n = 1). Of 11 patients with known prior history, 2 had 12 prior urothelial neoplasms (9 low-grade papillary neoplasms, 2 papillary urothelial neoplasms of low malignant potential, and 1 high-grade papillary cancer). Of 10 patients with atypical papillary hyperplasia and a minimum of 1 year of follow-up, 9 had 19 recurrences: CIS (n = 4), papilloma (n = 1), papillary neoplasm of low malignant potential (n = 1), infiltrating urothelial carcinoma (n = 3; 1 with CIS), and high-grade papillary urothelial carcinoma (n = 10; 5 with invasion and 2 with CIS). Whether the papillary hyperplasia had overlying CIS or dysplasia did not affect the correlation with urothelial neoplasms. Immunohistochemical analysis

Generic penetration in the retail atypical antipsychotic market.

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Lenderts, Susan; Kalali, Amir H; Buckley, Peter

2010-03-01

In this article, we explore the penetration of generic atypical antipsychotics in the United States market before and after the availability of generic risperidone in July 2008. Analysis suggests that, overall, generic penetration into the atypical antipsychotic market has grown from approximately three percent in January 2008 to more than 25 percent in December 2009. Similar trends are uncovered when branded and generic prescriptions are analyzed by specialty.

Atypical Odontalgia (Phantom Tooth Pain)

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... atypical facial pain, phantom tooth pain, or neuropathic orofacial pain, is characterized by chronic pain in a tooth ... such as a specialist in oral medicine or orofacial pain. The information contained in this monograph is for ...

Cohort study of atypical pressure ulcers development.

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Jaul, Efraim

2014-12-01

Atypical pressure ulcers (APU) are distinguished from common pressure ulcers (PU) with both unusual location and different aetiology. The occurrence and attempts to characterise APU remain unrecognised. The purpose of this cohort study was to analyse the occurrence of atypical location and the circumstances of the causation, and draw attention to the prevention and treatment by a multidisciplinary team. The cohort study spanned three and a half years totalling 174 patients. The unit incorporates two weekly combined staff meetings. One concentrates on wound assessment with treatment decisions made by the physician and nurse, and the other, a multidisciplinary team reviewing all patients and coordinating treatment. The main finding of this study identified APU occurrence rate of 21% within acquired PU over a three and a half year period. Severe spasticity constituted the largest group in this study and the most difficult to cure wounds, located in medial aspects of knees, elbows and palms. Medical devices caused the second largest occurrence of atypical wounds, located in the nape of the neck, penis and nostrils. Bony deformities were the third recognisable atypical wound group located in shoulder blades and upper spine. These three categories are definable and time observable. APU are important to be recognisable, and can be healed as well as being prevented. The prominent role of the multidisciplinary team is primary in identification, prevention and treatment. © 2013 The Authors. International Wound Journal © 2013 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide.

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Castellino, Alessia; Guidi, Stefano; Dellacasa, Chiara Maria; Gozzini, Antonella; Donnini, Irene; Nozzoli, Chiara; Manetta, Sara; Aydin, Semra; Giaccone, Luisa; Festuccia, Moreno; Brunello, Lucia; Maffini, Enrico; Bruno, Benedetto; David, Ezio; Busca, Alessandro

2018-01-01

Hepatic Veno-Occlusive Disease (VOD) is a potentially severe complication of hematopoietic stem cell transplantation (HSCT). Here we report two patients receiving an allogeneic HSCT who developed late onset VOD with atypical clinical features. The two patients presented with only few risk factors, namely, advanced acute leukemia, a myeloablative busulphan-containing regimen and received grafts from an unrelated donor. The first patient did not experience painful hepatomegaly and weight gain and both patients showed only a mild elevation in total serum bilirubin level. Most importantly, the two patients developed clinical signs beyond day 21 post-HSCT. Hepatic transjugular biopsy confirmed the diagnosis of VOD. Intravenous defibrotide was promptly started leading to a marked clinical improvement. Based on our experience, liver biopsy may represent a useful diagnostic tool when the clinical features of VOD are ambiguous. Early therapeutic intervention with defibrotide represents a crucial issue for the successful outcome of patients with VOD.

Late-onset hepatic veno-occlusive disease after allografting: report of two cases with atypical clinical features successfully treated with defibrotide.

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Alessia Castellino

2018-01-01

Full Text Available Hepatic Veno-occlusive disease (VOD is a potentially severe complication of hematopoietic stem cell transplantation (HSCT. Here we report two patients receiving an allogeneic HSCT  who developed late onset VOD with atypical clinical features. The two  patients presented with only few risk factors, namely, advanced acute leukemia, a myeloablative busulphan-containing regimen and received grafts from an unrelated donor. The first patient did not experience painful hepatomegaly and weight gain and both  patients showed only a mild elevation in total serum bilirubin level. Most importantly, the two patients developed clinical signs beyond day 21 post-HSCT. Hepatic transjugular biopsy confirmed the diagnosis of VOD. Intravenous defibrotide was promptly started leading to a marked clinical improvement. Based on our experience, liver biopsy may represent a useful diagnostic tool when the clinical features of VOD are ambiguous. Early therapeutic intervention with defibrotide  represents a crucial issue for the successful outcome of patients with VOD.

Atypical Focal Osteomyelitis as Initial Manifestation of AIDS

OpenAIRE

A. Akiki; Y. Bilde

2011-01-01

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

[Symptoms of Celiac disease in childhood].

Science.gov (United States)

Mouterde, O; Dumant, C; Mallet, E

2013-06-01

The knowledge regarding celiac disease has increased dramatically in recent years, due to the availability of accurate serologic markers. Mass screening studies have shown that the prevalence of sensitization can be as high as 1/80. The range of symptoms is wide, from the classic growth failure, denutrition and diarrhea in infancy to clinically and histologically asymptomatic sensitized subjects. The interest of a routine mass screening is debated. The classical celiac disease in infancy is well known. Atypical symptoms and potentially associated disease are more frequent and potentially confounding. Physicians should be aware of any clue for celiac disease in atypical cases in order to improve the diagnostic yield, and therefore avoiding short or long term consequences. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Clinical utility of FDG PET in Parkinson's disease and atypical parkinsonism associated with dementia.

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Walker, Zuzana; Gandolfo, Federica; Orini, Stefania; Garibotto, Valentina; Agosta, Federica; Arbizu, Javier; Bouwman, Femke; Drzezga, Alexander; Nestor, Peter; Boccardi, Marina; Altomare, Daniele; Festari, Cristina; Nobili, Flavio

2018-05-19

There are no comprehensive guidelines for the use of FDG PET in the following three clinical scenarios: (1) diagnostic work-up of patients with idiopathic Parkinson's disease (PD) at risk of future cognitive decline, (2) discriminating idiopathic PD from progressive supranuclear palsy, and (3) identifying the underlying neuropathology in corticobasal syndrome. We therefore performed three literature searches and evaluated the selected studies for quality of design, risk of bias, inconsistency, imprecision, indirectness and effect size. Critical outcomes were the sensitivity, specificity, accuracy, positive/negative predictive value, area under the receiving operating characteristic curve, and positive/negative likelihood ratio of FDG PET in detecting the target condition. Using the Delphi method, a panel of seven experts voted for or against the use of FDG PET based on published evidence and expert opinion. Of 91 studies selected from the three literature searches, only four included an adequate quantitative assessment of the performance of FDG PET. The majority of studies lacked robust methodology due to lack of critical outcomes, inadequate gold standard and no head-to-head comparison with an appropriate reference standard. The panel recommended the use of FDG PET for all three clinical scenarios based on nonquantitative evidence of clinical utility. Despite widespread use of FDG PET in clinical practice and extensive research, there is still very limited good quality evidence for the use of FDG PET. However, in the opinion of the majority of the panellists, FDG PET is a clinically useful imaging biomarker for idiopathic PD and atypical parkinsonism associated with dementia.

An atypical presentation of amoebic hepatic abscess

International Nuclear Information System (INIS)

Crespo Ramírez, Eduardo; Ruz Hernández, Mario; Guanche Garcell, Humberto; Castañeda Hernández, Mirtha

2015-01-01

Introduction: amoebic hepatic abscess is the extraintestinal manifestation of an infection by entamoeba histolytica. Estimations are made that 10% of the world population has been infected by the parasite, being more frequent in tropical regions, where overcrowding and bad sanitary conditions occur. Africa, Latin America, Southeast Asian and India have considerable health problems, resulting from this disease. In Cuba amoebiasis has been proved not to be one of the most frequent parasitism cases. Case presentation: male patient of age 48 years and of Indian nationality, presenting clinical findings, which proves a unique lesion in the right liver lobule, diagnosed by ultrasound scan and nuclear magnetic resonance. The presence is confirmed of serum antibodies against entamoeba histolytica. Treatment with metronidazole produced clinical and radiological improvement. Conclusions: atypical clinical presentation constitutes a remarkable element worthy considered in daily clinical practice. (author)

Atypical Fibroxanthoma in a 13-Year-Old Guatemalan Girl with Xeroderma Pigmentosum.

Science.gov (United States)

Chappell, Ava G; Chase, Elizabeth P; Chang, Beverly; Cunningham, Eric; Mihm, Fred; Calame, Antoanella; Fudem, Gary; Cunningham, Bari

2016-05-01

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disease involving a defect in DNA repair leading to the premature development of numerous aggressive cutaneous malignancies. Although atypical fibroxanthoma (AFX) is a neoplasm typically found in the setting of extensive sun exposure or therapeutic radiation, AFXs are rarely associated with children with XP. We report the case of a 13-year-old Guatemalan girl with the XP type C variant who developed one of the largest AFXs reported on a child's finger. © 2016 Wiley Periodicals, Inc.

Noninvasive detection of coronary abnormalities in pediatric patients with Kawassaki disease using multi-slice spiral CT

International Nuclear Information System (INIS)

Hou Yang; Guo Wenli; Yue Yong; Chen Liying; Guo Qiyong; Yu Xianyi; Wang Hong

2006-01-01

Objective: To evaluate the feasibility and value of detecting coronary artery lesions in Kawasaki disease using multi-slice computed tomography (MSCT). Methods: Thirty-four pediatric patients underwent 16-slice or 64-slice CT coronary, angiography. 18 patients were also examined with 2 dimension echocardiography (2DE). In all cases, visibility of coronary artery segment was recorded. The diameter of the LCA, RCA were measured in MSCTA and compared with 2DE. Correlation coefficient of dimension and coincidence rate of two methods were calculated. Results: Coronary artery lesions were found in 14 patients (22 branches) of the 34 cases with KD on MSCT. Six cases were dialated, 3 cases were dialated with aneurysms, 2 cases had aneurysms without dialation. Coronary artery stenosis in 1 eases, calcification in 2 cases. Three cases had multiple aneurysms with the presence of alternate stenosis that made the artery a bead-like appearance. CC of LM and RCA were 0.85, 0.91, respectively (P>0.05). Three coronary artery aneurysm in the distal RCA was missed by 2DE. MSCT could not detect slight or moderate mitral regurgitation in 2 patients and artery wall thickening in 5 patients. Conclusion: MSCT would be an effective complementary or alternative method for CDEC to evaluate coronary artery lesions non-invasively in pediatric patients with Kawasaki disease. (authors)

Refractory Rheumatic Disorder: Atypical Postpregnancy Osteoporosis

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Cindy Mourgues

2015-01-01

Full Text Available This is a case report on a young patient with severe osteoporosis that was initially revealed when she presented with polyarthralgia during her second pregnancy. Postpartum, the pain increased and her X-ray did not show any abnormalities. A bone scintigraphy was performed. It indicated an inflammatory rheumatic disorder. Six months after partum, an investigation of right coxalgia revealed a spontaneous basicervical fracture. Given the persistent polyarthralgia, the patient underwent a new scintigraphy, which revealed areas of what looked to be old rib and L1 fractures. A subsequent full body magnetic resonance imaging (MRI scan revealed signal abnormalities that could indicate multiple lower limb bone fractures. Despite exhaustive biological, radiological, and histological testing, no secondary cause for the osteoporosis was found. The patient was started on teriparatide. We finally concluded that, despite the atypical presentation, the patient was suffering from postpregnancy osteoporosis. It is possible that the frequency of occurrence of this still poorly understood disease is underestimated.

Síndrome de Cogan: achados oculares em um caso da forma atípica Cogan's syndrome: ocular findings in an atypical case

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Ana Karina Santiago de Medeiros Lima

2006-12-01

Full Text Available A síndrome de Cogan é entidade multissistêmica rara caracterizada por ceratite intersticial associada à disfunção áudio-vestibular e possível surdez irreversível classificada em duas formas clínicas: típica e atípica. Há discordância na literatura quanto à presença de acometimento corneano na forma atípica. Uma paciente de 32 anos queixando-se de hiperemia e dor ocular, fotofobia e baixa da acuidade visual no olho direito, associada à perda súbita de audição à esquerda, vômitos, diarréia, oligúria, dor na orofaringe e febre. História prévia de semelhante acometimento do olho esquerdo e audição direita. Havia intensa hiperemia conjuntival, esclerite nodular, episclerite e infiltrados circulares no estroma corneano. A paciente recebeu pulsoterapia com metilprednisolona e ciclofosfamida. Evoluiu com grande melhora ocular, porém com resposta auditiva pobre. O caso reportado pode constituir forma típica da síndrome de Cogan (de acordo com autores que defendem o não-acometimento corneano na forma atípica com alguns achados característicos da forma atípica ou um caso da forma atípica da síndrome de Cogan (para aqueles que defendem o acometimento corneano na forma atípica. O diagnóstico diferencial também é discutido.Cogan's syndrome is an unusual multisystemic disease characterized by intersticial keratitis in association with vestibuloauditory dysfunction and possible irreversible deafness, classified into 2 clinical types: typical and atypical. There is disagreement in the literature about corneal disease in the atypical variety. A 32-year-old woman complaining of ocular hyperemia and ocular pain, photophobia and visual acuity loss in the right eye associated with sudden left hearing loss, vomiting, diarrhea, oliguria, oropharynx pain and fever. Previous history of similar disease in left eye and right hearing. There was intense conjunctival hyperemia, nodular scleritis, episcleritis, and circular infiltrates

A novel examination of atypical major depressive disorder based on attachment theory.

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Levitan, Robert D; Atkinson, Leslie; Pedersen, Rebecca; Buis, Tom; Kennedy, Sidney H; Chopra, Kevin; Leung, Eman M; Segal, Zindel V

2009-06-01

While a large body of descriptive work has thoroughly investigated the clinical correlates of atypical depression, little is known about its fundamental origins. This study examined atypical depression from an attachment theory framework. Our hypothesis was that, compared to adults with melancholic depression, those with atypical depression would report more anxious-ambivalent attachment and less secure attachment. As gender has been an important consideration in prior work on atypical depression, this same hypothesis was further tested in female subjects only. One hundred ninety-nine consecutive adults presenting to a tertiary mood disorders clinic with major depressive disorder with either atypical or melancholic features according to the Structured Clinical Interview for DSM-IV Axis-I Disorders were administered a self-report adult attachment questionnaire to assess the core dimensions of secure, anxious-ambivalent, and avoidant attachment. Attachment scores were compared across the 2 depressed groups defined by atypical and melancholic features using multivariate analysis of variance. The study was conducted between 1999 and 2004. When men and women were considered together, the multivariate test comparing attachment scores by depressive group was statistically significant at p depression was associated with significantly lower secure attachment scores, with a trend toward higher anxious-ambivalent attachment scores, than was melancholia. When women were analyzed separately, the multivariate test was statistically significant at p depressive groups. These preliminary findings suggest that attachment theory, and insecure and anxious-ambivalent attachment in particular, may be a useful framework from which to study the origins, clinical correlates, and treatment of atypical depression. Gender may be an important consideration when considering atypical depression from an attachment perspective. Copyright 2009 Physicians Postgraduate Press, Inc.

Atypical pyoderma gangrenosum in a patient with osteomyelofibrosis

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Živanović Dubravka

2007-01-01

Full Text Available Background. Atypical forms of pyoderma gangrenosum generally appear on the upper extremities; most frequently they are associated with myeloproliferative disorders, including osteomyelofibrosis. A response to systemic steroids is more pronounced than in classical form. Sometimes it may be the first sign of an underlying malignancy. Case report. We reported a patient with atypical pyoderma gangrenosum developed during the course of a myeloid malignancy - osteomyelofibrosis. The lesions occurred after a minor trauma. Painful blistering plaques, with an elevated, bluish-gray border were located on the dorsal aspect of hands. No skin malignancy was found. The lesions resolved rapidly to systemic steroids. Conclusion. Considering the unusual clinical presentation which makes the diagnosis difficult, as well as the fact that atypical forms of pyoderma gangrenosum can be the first sign of malignancies, especially myeloproliferative ones, recognizing this entity enables timely guiding future investigations toward their prompt detection.

Celiac disease

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Holtmeier Wolfgang

2006-03-01

Full Text Available Abstract Celiac disease is a chronic intestinal disease caused by intolerance to gluten. It is characterized by immune-mediated enteropathy, associated with maldigestion and malabsorption of most nutrients and vitamins. In predisposed individuals, the ingestion of gluten-containing food such as wheat and rye induces a flat jejunal mucosa with infiltration of lymphocytes. The main symptoms are: stomach pain, gas, and bloating, diarrhea, weight loss, anemia, edema, bone or joint pain. Prevalence for clinically overt celiac disease varies from 1:270 in Finland to 1:5000 in North America. Since celiac disease can be asymptomatic, most subjects are not diagnosed or they can present with atypical symptoms. Furthermore, severe inflammation of the small bowel can be present without any gastrointestinal symptoms. The diagnosis should be made early since celiac disease causes growth retardation in untreated children and atypical symptoms like infertility or neurological symptoms. Diagnosis requires endoscopy with jejunal biopsy. In addition, tissue-transglutaminase antibodies are important to confirm the diagnosis since there are other diseases which can mimic celiac disease. The exact cause of celiac disease is unknown but is thought to be primarily immune mediated (tissue-transglutaminase autoantigen; often the disease is inherited. Management consists in life long withdrawal of dietary gluten, which leads to significant clinical and histological improvement. However, complete normalization of histology can take years.

Atypical antipsychotics: trends in analysis and sample preparation of various biological samples.

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Fragou, Domniki; Dotsika, Spyridoula; Sarafidou, Parthena; Samanidou, Victoria; Njau, Samuel; Kovatsi, Leda

2012-05-01

Atypical antipsychotics are increasingly popular and increasingly prescribed. In some countries, they can even be obtained over-the-counter, without a prescription, making their abuse quite easy. Although atypical antipsychotics are thought to be safer than typical antipsychotics, they still have severe side effects. Intoxications are not rare and some of them have a fatal outcome. Drug interactions involving atypical antipsychotics complicate patient management in clinical settings and the determination of the cause of death in fatalities. In view of the above, analytical strategies that can efficiently isolate atypical antipsychotics from a variety of biological samples and quantify them accurately, sensitively and reliably, are of utmost importance both for the clinical, as well as for the forensic toxicologist. In this review, we will present and discuss novel analytical strategies that have been developed from 2004 to the present day for the determination of atypical antipsychotics in various biological samples.

[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis].

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Pradat, P-F; Bruneteau, G

2006-06-01

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of upper and lower motor neurons. In the absence of any validated biological marker, the diagnosis of ALS depends upon recognition of characteristic symptoms and signs together with supportive electrophysiological findings. The diagnosis of ALS is easy to recognize in its fully developed form but during the early stages both false positive and false negative diagnoses are common. In clinical practice, diagnostic difficulties mostly arise with patients who present either with only upper motor neuron, or with only lower motor neuron signs. It may be difficult to distinguish ALS with clinically predominant lower motor neuron involvement from alternative diagnoses including spinal atrophies of adult onset, Kennedy's disease, inclusion body myositis and motor neuropathies with conduction blocks. The diagnosis of ALS related syndromes (progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy) requires the elimination of alternate diagnoses. This paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS.

Fluctuation theorems and atypical trajectories

International Nuclear Information System (INIS)

Sahoo, M; Lahiri, S; Jayannavar, A M

2011-01-01

In this work, we have studied simple models that can be solved analytically to illustrate various fluctuation theorems. These fluctuation theorems provide symmetries individually to the distributions of physical quantities such as the classical work (W c ), thermodynamic work (W), total entropy (Δs tot ) and dissipated heat (Q), when the system is driven arbitrarily out of equilibrium. All these quantities can be defined for individual trajectories. We have studied the number of trajectories which exhibit behaviour unexpected at the macroscopic level. As the time of observation increases, the fraction of such atypical trajectories decreases, as expected at the macroscale. The distributions for the thermodynamic work and entropy production in nonlinear models may exhibit a peak (most probable value) in the atypical regime without violating the expected average behaviour. However, dissipated heat and classical work exhibit a peak in the regime of typical behaviour only.

Childhood Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®)—Patient Version

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Atypical teratoid/rhabdoid tumor (AT/RT) is a fast-growing tumor of the brain or spinal cord. Treatment may include surgery, radiation therapy, and chemotherapy. Get information about the symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood atypical teratoid/rhabdoid tumors in this expert-reviewed summary.

Typical and Atypical Dementia Family Caregivers: Systematic and Objective Comparisons

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Nichols, Linda O.; Martindale-Adams, Jennifer; Burns, Robert; Graney, Marshall J.; Zuber, Jeffrey

2011-01-01

This systematic, objective comparison of typical (spouse, children) and atypical (in-law, sibling, nephew/niece, grandchild) dementia family caregivers examined demographic, caregiving and clinical variables. Analysis was of 1,476 caregivers, of whom 125 were atypical, from the Resources for Enhancing Alzheimer's Caregivers Health (REACH I and II)…

The contribution of diffusion-weighted MR imaging to distinguishing typical from atypical meningiomas

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Hakyemez, Bahattin [Uludag University School of Medicine, Department of Radiology, Gorukle, Bursa (Turkey); Bursa State Hospital, Department of Radiology, Bursa (Turkey); Yildirim, Nalan; Gokalp, Gokhan; Erdogan, Cuneyt; Parlak, Mufit [Uludag University School of Medicine, Department of Radiology, Gorukle, Bursa (Turkey)

2006-08-15

Atypical/malignant meningiomas recur more frequently then typical meningiomas. In this study, the contribution of diffusion-weighted MR imaging to the differentiation of atypical/malignant and typical meningiomas and to the determination of histological subtypes of typical meningiomas was investigated. The study was performed prospectively on 39 patients. The signal intensity of the lesions was evaluated on trace and apparent diffusion coefficient (ADC) images. ADC values were measured in the lesions and peritumoral edema. Student's t-test was used for statistical analysis. P<0.05 was considered statistically significant. Mean ADC values in atypical/malignant and typical meningiomas were 0.75{+-}0.21 and 1.17{+-}0.21, respectively. Mean ADC values for subtypes of typical meningiomas were as follows: meningothelial, 1.09{+-}0.20; transitional, 1.19{+-}0.07; fibroblastic, 1.29{+-}0.28; and angiomatous, 1.48{+-}0.10. Normal white matter was 0.91{+-}0.10. ADC values of typical meningiomas and atypical/malignant meningiomas significantly differed (P<0.001). However, the difference between peritumoral edema ADC values was not significant (P>0.05). Furthermore, the difference between the subtypes of typical meningiomas and atypical/malignant meningiomas was significant (P<0.001). Diffusion-weighted MR imaging findings of atypical/malignant meningiomas and typical meningiomas differ. Atypical/malignant meningiomas have lower intratumoral ADC values than typical meningiomas. Mean ADC values for peritumoral edema do not differ between typical and atypical meningiomas. (orig.)

Familial Atypical Hemolytic Uremic Syndrome: A Review of Its Genetic and Clinical Aspects

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Fengxiao Bu

2012-01-01

Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare renal disease (two per one million in the USA characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Both sporadic (80% of cases and familial (20% of cases forms are recognized. The study of familial aHUS has implicated genetic variation in multiple genes in the complement system in disease pathogenesis, helping to define the mechanism whereby complement dysregulation at the cell surface level leads to both sporadic and familial disease. This understanding has culminated in the use of Eculizumab as first-line therapy in disease treatment, significantly changing the care and prognosis of affected patients. However, even with this bright outlook, major challenges remain to understand the complexity of aHUS at the genetic level. It is possible that a more detailed picture of aHUS can be translated to an improved understanding of disease penetrance, which is highly variable, and response to therapy, both in the short and long terms.

Gender-Atypical Mental Illness as Male Gender Threat.

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Michniewicz, Kenneth S; Bosson, Jennifer K; Lenes, Joshua G; Chen, Jason I

2016-07-01

The present study examined whether men view gender-atypical (i.e., feminine) psychological disorders as threats to their gender status. Men and women (N = 355) rated their expectations of gender status loss, feelings of distress, and help-seeking intentions in response to 10 different stereotypically masculine and feminine psychological disorders. Men as compared to women expected greater gender status loss for, and reported more distress to, gender-atypical versus gender-typical disorders. Expectations of gender status loss partially mediated the link between participant gender and distress at the thought of gender-atypical disorders. These findings suggest that feminine disorders pose more powerful gender status threats for men than masculine disorders do and that men's expectations of gender status loss for feminine disorders drive their negative reactions to these mental illnesses. The discussion emphasizes the importance of considering the gender-typicality of disorders, and the implications of these findings for clinical interventions. © The Author(s) 2015.

Atypical presentation of HELLP syndrome: clinical case report

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Juan Manuel Tobar Parra

2017-12-01

Full Text Available Objective: To describe a case of HELLP syndrome with atypical presentation form. Background: HELLP syndrome is a complication of preeclampsia, characterized by: haemolysis, elevation of liver enzymes and thrombocytopenia; Can present atypical, without hypertension or proteinuria, 10-20% of the cases. Case report: 38 year old female patient, with a pregnancy of 38.5 weeks of gestation, treated at the Hospital Universitario San José de Popayán (Colombia. Atypical HELLP syndrome is diagnosed in a pregnant woman with thrombocytopenia, impaired liver enzymes, but no evidence of proteinuria or hypertension. Gestation is terminated by cesarean section and magnesium sulfate is given for 24 hours, with adequate post-surgical evolution, clinical improvement of the symptomatology presented, normalization of liver enzymes and platelet elevation. Conclusion: Knowledge of this syndrome, although of rare occurrence, allows a fast action, an effective diagnosis and treatment, to avoid morbidity and greater maternal fetal mortality.

Preparation and cellular response of porous A-type carbonated hydroxyapatite nanoceramics

International Nuclear Information System (INIS)

Li Bo; Liao Xiaoling; Zheng Li; He Huawei; Wang Hong; Fan Hongsong; Zhang Xingdong

2012-01-01

Microwave sintering using the activated carbon as embedding material was applied in preparation of porous A-type carbonated hydroxyapatite ceramics with nano(nCHA) and submicron (mCHA) structure. By examining the linear shrinkages and the compressive strengths of samples at different temperatures, a suitable microwave sintering temperature was achieved. The microwave sintering method was successfully used to prepare A-type CHA with nano or submicron structure, and the mechanism of the formation of A-type carbonate groups was discussed also. Compared with the samples prepared by the conventional sintering method (mHA), the nCHA bioceramics synthesized by the microwave sintering approach had smaller grain size and more uniform microstructure, and showed a compressive strength similar to the conventional samples. In vitro dissolution test proved that nCHA exhibits better degradation property in comparison to pure HA. Rat osteoblasts were cultured with nCHA, mCHA and mHA to evaluate their biocompatibility, and nCHA showed significant enhancement of cells in attachment, proliferation and differentiation. In conclusion, carbonate groups can be easily introduced to HA crystal structure using the activated carbon as embedding material, and microwave sintering is an effective and simple method in preparing A-type CHA with a nanostructure. Results from this in vitro biological study suggest that porous A-type carbonated hydroxyapatite nanoceramics may be a much better candidate for clinical use in terms of bioactivity. - Highlights: ► We prepared porous A-type carbonated hydroxyapatite nanoceramics with microwave sintering. ► We examined physico-chemical characterization and osteoblast response. ► The nanoceramics have a comparable compressive strength to samples with conventional sintering method. ► The nanoceramics enhance degradation property, osteoblast proliferation and differentiation. ► The activated carbon is favorable for preheating samples and providing

[Morpheiform sarcoidosis as atypical manifestation of sarcoidosis. Review of the literature and differential diagnosis].

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Castellanos-González, María; Picazo Talavera, María Remedios

2016-09-16

Sarcoidosis is an idiopathic multisystem granulomatous disease that commonly involves the skin in 25% of affected patients. Because lesions assume a vast array of morphologies, a classification dividing them into specific (with presence of typical granulomas in the biopsy) or nonspecific (not containing granulomas) has been proposed. In the first group the variant morpheaform is considered exceptional. We review the cases reported in the literature and describe the possible differential diagnosis. We highlight the importance of recognizing the very atypical presentation of sarcoidosis and its ability to mimic morpheaform or sclerosis diseases in our patients. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

3D-ultrasound in imaging, diagnosis and follow-up of an atypical hydatid cyst.

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Ockenga, J; Gebel, M; Caselitz, M; Topalidis, T; Boozari, B; Bleck, J; Manns, M P

1998-07-01

Abdominal ultrasonography is the procedure of choice to diagnose hydatid cysts caused by Echinococcus granulosus. Recently three-dimensional ultrasonography has become available for clinical application. We report a case of an atypical seronegative hydatid disease, in which the additional use of 3D-sonography improved the sonographic diagnosis, which was confirmed by a fine needle biopsie complicated by an anaphylactic reaction. In addition the potential advantage of 3D-ultrasonography in diagnosis and follow-up hydatid disease will be discussed, especially in the context of new alternative therapeutic options like chemotherapy with benzimidazoles or the percutaneous drainage by the PAIR procedure (puncture-aspiration-injection-re-aspiration).

Atypical imaging appearances of intracranial meningiomas

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O' Leary, S. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Adams, W.M. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Parrish, R.W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Mukonoweshuro, W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom)]. E-mail: William.mukonoweshuro@phnt.swest.nhs.uk

2007-01-15

Meningiomas are the commonest primary, non-glial intracranial tumours. The diagnosis is often correctly predicted from characteristic imaging appearances. This paper presents some examples of atypical imaging appearances that may cause diagnostic confusion.

[Auricular sporotrichosis. Atypical case report simulating bacterial cellulitis].

Science.gov (United States)

Ochoa-Reyes, Juan; Ramos-Martínez, Ernesto; Treviño-Rangel, Rogelio; González, Gloria M; Bonifaz, Alexandro

Sporotrichosis is the most common subcutaneous or implantation mycosis in Mexico. The case of a preauricular cutaneous-fixed sporotrichosis simulating atypical bacterial cellulitis is reported in an elderly patient with no history of trauma. The biopsy showed a suppurative granuloma with scarce yeast. Sporothrix schenckii was identified in the culture and confirmed by molecular biology. She was treated with itraconazole and a clinical and mycological cure was obtained. The case of atypical presentation is presented, coming from a semi-arid zone with extreme weather.

A Case–Control Study on the Origin of Atypical Scrapie in Sheep, France

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Morignat, Eric; Ducrot, Christian; Calavas, Didier

2009-01-01

A matched case–control study (95 cases and 220 controls) was designed to study risk factors for atypical scrapie in sheep in France. We analyzed contacts with animals from other flocks, lambing and feeding practices, and exposure to toxic substances. Data on the prnp genotype were collected for some case and control animals and included in a complementary analysis. Sheep dairy farms had a higher risk for scrapie (odds ratio [OR] 15.1, 95% confidence interval [CI] 3.3–69.7). Lower risk was associated with organic farms (OR 0.15, 95% CI 0.02–1.26), feeding corn silage (OR 0.16, 95% CI 0.05–0.53), and feeding vitamin and mineral supplements (OR 0.6, 95% CI 0.32–1.14). Genetic effects were quantitatively important but only marginally changed estimates of other variables. We did not find any risk factor associated with an infectious origin of scrapie. Atypical scrapie could be a spontaneous disease influenced by genetic and metabolic factors. PMID:19402956

Occurrence of Autoimmune Diseases Related to the Vaccine against Yellow Fever

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Ana Cristina Vanderley Oliveira

2014-01-01

Full Text Available Yellow fever is an infectious disease, endemic in South America and Africa. This is a potentially serious illness, with lethality between 5 and 40% of cases. The most effective preventive vaccine is constituted by the attenuated virus strain 17D, developed in 1937. It is considered safe and effective, conferring protection in more than 90% in 10 years. Adverse effects are known as mild reactions (allergies, transaminases transient elevation, fever, headache and severe (visceral and neurotropic disease related to vaccine. However, little is known about its potential to induce autoimmune responses. This systematic review aims to identify the occurrence of autoinflammatory diseases related to 17D vaccine administration. Six studies were identified describing 13 possible cases. The diseases were Guillain-Barré syndrome, multiple sclerosis, multiple points evanescent syndrome, acute disseminated encephalomyelitis, autoimmune hepatitis, and Kawasaki disease. The data suggest that 17D vaccination may play a role in the mechanism of loss of self-tolerance.

The role of atypical antipsychotics for treatment of Tourette's syndrome: an overview.

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Budman, Cathy L

2014-07-01

Tourette's syndrome (TS) is a neuropsychiatric disorder of childhood onset characterized by multiple motor and phonic tics that fluctuate over time. Tic symptoms often improve by late adolescence, but some children and adults with TS may experience significant tic-related morbidity, including social and family problems, academic difficulties, and pain. When more conservative interventions are not successful, and when certain psychiatric co-morbidities further complicate the clinical profile, treating TS with an atypical antipsychotic medication may be a reasonable second-tier approach. However, the evidence supporting efficacy and safety of the atypical antipsychotics for treatment of tics is still very limited. The objective of this paper is to provide an updated overview of the role of atypical antipsychotics for treatment of TS, with evidence-based guidance on their use. Evidence for efficacy of different typical and atypical antipsychotics for treatment of tics was examined by conducting a systematic, keyword-related search of 'atypical antipsychotics' and 'Tourette's syndrome' in PubMed (National Library of Medicine, Washington, DC, USA). Four recent treatment consensus publications were also reviewed. This review focused on literature published from 2000 to 2013 and on available randomized controlled trials in TS. Evidence supporting the use of atypical antipsychotics for treatment of TS is limited. There are few randomized medication treatment trials in TS (i.e. risperidone, aripiprazole, ziprasidone), which employed varying methodologies, thereby restricting meaningful comparisons among studies. Future collaborations among clinical sites with TS expertise employing high-quality study design may better elucidate the role of atypical antipsychotics for treatment of TS.

Atypical Rulings of the Indonesian Constitutional Court

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Bisariyadi

2016-08-01

Full Text Available In deciding judicial review cases, the Court may issue rulings that is not in accordance to what is stipulated in the Constitutional Court Law (Law Number 8 Year 2011. Atypical rulings means that the court may reconstruct a provision, delay the legislation/rulings enactment or give instruction to lawmakers. In addition, the court also introduce the “conditionally (unconstitutional” concept. This essay attempts to identify and classify these atypical rulings, including conditionally (un constitutional rulings, by examined the constitutional court judicial review rulings from 2003 to 2015. This study will provide a ground work for advance research on typical rulings by the Indonesian constitutional court.

The use of electroconvulsive therapy in atypical psychotic presentations: a case review.

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Montgomery, John H; Vasu, Devi

2007-10-01

Convulsive therapy and its progeny, electroconvulsive therapy (ECT), were originally used for the treatment of catatonic schizophrenia, and there is little doubt that ECT remains an effective intervention for the treatment of schizophrenia. However, current practice tends to favor the use of ECT in severe or treatment refractory affective disorders, and its use in schizophrenia and other nonaffective (atypical) psychotic disorders has become controversial.CASE REPORTS HAVE SUGGESTED A ROLE FOR ECT IN TWO SPECIFIC ATYPICAL PSYCHOTIC DISORDERS: Cotard's syndrome and cycloid psychosis. In this article, we review the atypical psychotic disorders and report a series of five case examples that signify the role of ECT in atypical psychotic presentations, particularly when the symptoms resemble those found in Cotard's syndrome and cycloid psychosis.

MANIFESTATIONS OF AGGRESSIVE ATYPICAL KAPOSI'S ...

African Journals Online (AJOL)

... weight loss (86.8%), skin nodules (86.4%) and diarrhoea (55.3%). Virtually, all occupational groups were affected, with students, civil servants and businessmen topping the list. Key Words: Atypical Aggressive Kaposi's sarcoma, HIV infection. African Journal Of Clinical And Experimental Microbiology Jan 2004 Vol.5 No.1 ...

Clinical features of Friedreich's ataxia: classical and atypical phenotypes.

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Parkinson, Michael H; Boesch, Sylvia; Nachbauer, Wolfgang; Mariotti, Caterina; Giunti, Paola

2013-08-01

One hundred and fifty years since Nikolaus Friedreich's first description of the degenerative ataxic syndrome which bears his name, his description remains at the core of the classical clinical phenotype of gait and limb ataxia, poor balance and coordination, leg weakness, sensory loss, areflexia, impaired walking, dysarthria, dysphagia, eye movement abnormalities, scoliosis, foot deformities, cardiomyopathy and diabetes. Onset is typically around puberty with slow progression and shortened life-span often related to cardiac complications. Inheritance is autosomal recessive with the vast majority of cases showing an unstable intronic GAA expansion in both alleles of the frataxin gene on chromosome 9q13. A small number of cases are caused by a compound heterozygous expansion with a point mutation or deletion. Understanding of the underlying molecular biology has enabled identification of atypical phenotypes with late onset, or atypical features such as retained reflexes. Late-onset cases tend to have slower progression and are associated with smaller GAA expansions. Early-onset cases tend to have more rapid progression and a higher frequency of non-neurological features such as diabetes, cardiomyopathy, scoliosis and pes cavus. Compound heterozygotes, including those with large deletions, often have atypical features. In this paper, we review the classical and atypical clinical phenotypes of Friedreich's ataxia. © 2013 International Society for Neurochemistry.

Bullying, Physical Aggression, Gender-Atypicality, and Sexual Orientation in Samoan Males.

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Semenyna, Scott W; Vasey, Paul L

2017-07-01

Bullying is characterized by the repeated attempts of a group or individual to gain social advantage by the use of relational, verbal, or physical aggression against a target, especially when there is a perceived or actual power imbalance (Espelage & Swearer, 2003). One consistent finding is that gay (i.e., androphilic) males report higher rates of victimization due to bullying in adolescence than their heterosexual (i.e., gynephilic) counterparts. Western data indicate that gender-atypical behavior, regardless of sexual orientation, is a key predictor of victimization due to bullying. Androphilic males generally display childhood gender-atypicality, including reduced levels of physical aggression, which may cause bullies to perceive them as "easy" targets. In order to test the associations between sexual orientation, childhood gender-atypicality, and recalled victimization due to bullying, a sample of Samoan gynephilic men (n = 100) were compared to a group of Samoan transgender androphilic males (n = 103), known as fa'afafine. Although the fa'afafine reported far more childhood gender-atypicality, the two groups did not differ significantly on measures of physical aggression or their reported rates of victimization due to bullying. Additionally, greater physical aggression, not gender-atypicality, was the only significant predictor of being bullied in both men and fa'afafine. These results suggest that there is nothing inherent in sexual orientation or childhood gender-atypicality that would potentiate victimization from bullying. Instead, the cultural context in which a bully functions influences the extent to which these are "acceptable" reasons to target certain individuals.

Evaluation of Parkinson's disease using magnetic resonance imaging

International Nuclear Information System (INIS)

Vedolin, Leonardo; Marchiori, Edson; Rieder, Carlos

2004-01-01

The objective of this study was to evaluate the magnetic resonance imaging findings in patients with Parkinson's disease. In the period from October 1999 to October 2002, 42 patients with parkinsonism were investigated using a 1.5 T MR equipment. Patients were divided into two groups: patients with Parkinson's disease (n = 26) and patients with atypical Parkinsonian syndrome (n = 16). The results were compared with a control group (n = 18). The following variables were evaluated: thickness of the mesencephalon compact pars, hypointense signal in the putamen, degree of brain atrophy, lesions in the mesencephalon, lesions in the white matter, and the presence of lesions in the posterior-lateral edge of the putamen. Statistical data analysis was carried out using the SPSS program. Results: The mean age was 58.2 years for the Parkinson's disease and control groups, and 60.5 years for the atypical Parkinsonian syndrome group. Patients with Parkinson's disease and atypical Parkinsonian syndromes presented decreased thickness of the compact pars and a higher degree of signal hypointensity in the putamen. Cerebral atrophy was more prominent in the patients with atypical Parkinsonian syndrome. Lesions in mesencephalon and white matter were similar in both groups. The frequency of hyperintense signal in the posterior-lateral edge of the putamen was low within the studied population, although that could suggest multiple-system atrophy. Magnetic resonance imaging allows the detection of brain morphological changes that may help in the diagnosis of Parkinsonian syndromes. (author)

Time course of lung function changes in atypical pneumonia.

Science.gov (United States)

Benusiglio, L N; Stalder, H; Junod, A F

1980-01-01

We measured pulmonary function in each of 21 patients suffering from "atypical", non-bacterial pneumonia during the acute illness and during convalescence (two to 18 months) to study the course and the nature of functional impairment at different stages of the disease. In six patients, no aetiological agent was found. An aetiological agent was identified in 15 of the patients: Mycoplasma pneumoniae (seven patients), influenza A (three patients), parainfluenza 3 (one patient), varicella (two patients), Q fever (one patient), coxsackie B3 (one patient). At the time of admission we observed a restrictive pattern in 52%, an obstructive pattern (decreased FEV1/FVC ratio) in 52% abnormalities in distribution of ventilation (abnormal slope of phase 3) in 63%, and abnormalities in gas exchange (increased AaDO2) in 75% of the patients. The frequency of abnormalities in these pulmonary function tests decreased dramatically after two to four weeks and nearly disappeared in most patients during convalescence. The only major residual abnormality was a decreased FEV1/FVC ratio in five subjects, four of whom were smokers. However, when MMEF and V75 were measured at this stage, their average value for all the groups of patients with the exclusion of the Mycoplasma pneumoniae group, was markedly reduced. These data suggest that small airways involvement can be demonstrated during the convalescence of patients recovering from various types of atypical pneumonia other than those caused by Mycoplasma pneumoniae. PMID:7444825

Asymmetric dimethylarginine in somatically healthy schizophrenia patients treated with atypical antipsychotics

DEFF Research Database (Denmark)

Jørgensen, Anders; Knorr, Ulla Benedichte Søsted; Soendergaard, Mia Greisen

2015-01-01

ratio are positively correlated to measures of oxidative stress. METHODS: We included 40 schizophrenia patients treated with AAP, but without somatic disease or drug abuse, and 40 healthy controls. Plasma concentrations of ADMA and L-arginine were determined by high-performance liquid chromatography...... in a range of cardiovascular disorders. Increased ADMA levels may also lead to increased oxidative stress. We hypothesized that ADMA and the L-arginine:ADMA ratio are increased in somatically healthy schizophrenia patients treated with atypical antipsychotics (AAP), and that the ADMA and the L-arginine: ADMA....... Data were related to markers of systemic oxidative stress on DNA, RNA and lipids, as well as measures of medication load, duration of disease and current symptomatology. RESULTS: Plasma ADMA and the L-arginine:ADMA ratio did not differ between schizophrenia patients and controls. Furthermore, ADMA...

Atypical E2f functions are critical for pancreas polyploidization.

Science.gov (United States)

Matondo, Ramadhan B; Moreno, Eva; Toussaint, Mathilda J M; Tooten, Peter C J; van Essen, Saskia C; van Liere, Elsbeth A; Youssef, Sameh A; Bongiovanni, Laura; de Bruin, Alain

2018-01-01

The presence of polyploid cells in the endocrine and exocrine pancreas has been reported for four decades. In rodents, pancreatic polyploidization is initiated after weaning and the number of polyploid cells increases with age. Surprisingly the molecular regulators and biological functions of polyploidization in the pancreas are still unknown. We discovered that atypical E2f activity is essential for polyploidization in the pancreas, using an inducible Cre/LoxP approach in new-born mice to delete ubiquitously the atypical E2f transcription factors, E2f7 and E2f8. In contrast to its critical role in embryonic survival, conditional deletion of both of both atypical E2fs in newborn mice had no impact on postnatal survival and mice lived until old age. However, deficiency of E2f7 or E2f8 alone was sufficient to suppress polyploidization in the pancreas and associated with only a minor decrease in blood serum levels of glucose, insulin, amylase and lipase under 4 hours starvation condition compared to wildtype littermates. In mice with fewer pancreatic polyploid cells that were fed ad libitum, no major impact on hormones or enzymes levels was observed. In summary, we identified atypical E2fs to be essential for polyploidization in the pancreas and discovered that postnatal induced loss of both atypical E2fs in many organs is compatible with life until old age.

Atypical forms of acute Epstein-Barr virus infection in children

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T.V. Sorokman

2018-03-01

Full Text Available Background. Today, there is a tendency to increase in Epstein-Barr virus infection (EBVI morbidity. The purpose of the study was to identify the incidence and features of atypical forms of acute EBVI in children. Material and methods. We have examined 28 children aged 6 months to 18 years with EBVI who were monitored in pediatric polyclinic. The activity of alanine aminotransferase and aspartate aminotransferase, levels of bilirubin, alkaline phosphatase, gamma-glutamyl transpeptidase, markers of viral hepatitis were evaluated. Enzyme-linked immunosorbent assay was performed with determination of blood markers of EBV (immunoglobulin (Ig M viral capsid antigen (VCA, IgG early antigen, IgG VCA, avidity and cytomegalovirus (CMV (IgM, IgG, avidity, EBV deoxyribonucleic acid (DNA, CMV DNA; polymerase chain reaction was used for serological diagnosis. The data were processed by statistical analysis using Statistica 6 program. Results. In 71.4 % of cases, EBVI had usual course and moderate severity. The atypical forms of acute EBVI were observed in 28.5 % of cases. Clinically atypical forms began mainly from signs of acute respiratory infections followed by lesions of the internal organs (liver and heart, in particular, in children under 1 year of age, and changes in liver functional tests. Conclusions. The incidence of atypical forms of EBVI is 28.5 %. Atypical forms of EBVI are more common in infants and adolescents and associated with the damage to the internal organs (liver and heart.

Atypical E2f functions are critical for pancreas polyploidization.

Directory of Open Access Journals (Sweden)

Ramadhan B Matondo

Full Text Available The presence of polyploid cells in the endocrine and exocrine pancreas has been reported for four decades. In rodents, pancreatic polyploidization is initiated after weaning and the number of polyploid cells increases with age. Surprisingly the molecular regulators and biological functions of polyploidization in the pancreas are still unknown. We discovered that atypical E2f activity is essential for polyploidization in the pancreas, using an inducible Cre/LoxP approach in new-born mice to delete ubiquitously the atypical E2f transcription factors, E2f7 and E2f8. In contrast to its critical role in embryonic survival, conditional deletion of both of both atypical E2fs in newborn mice had no impact on postnatal survival and mice lived until old age. However, deficiency of E2f7 or E2f8 alone was sufficient to suppress polyploidization in the pancreas and associated with only a minor decrease in blood serum levels of glucose, insulin, amylase and lipase under 4 hours starvation condition compared to wildtype littermates. In mice with fewer pancreatic polyploid cells that were fed ad libitum, no major impact on hormones or enzymes levels was observed. In summary, we identified atypical E2fs to be essential for polyploidization in the pancreas and discovered that postnatal induced loss of both atypical E2fs in many organs is compatible with life until old age.

Atypical manifestations of Epstein-Barr virus in children: a diagnostic challenge.

Science.gov (United States)

Bolis, Vasileios; Karadedos, Christos; Chiotis, Ioannis; Chaliasos, Nikolaos; Tsabouri, Sophia

2016-01-01

Clarify the frequency and the pathophysiological mechanisms of the rare manifestations of Epstein-Barr virus infection. Original research studies published in English between 1985 and 2015 were selected through a computer-assisted literature search (PubMed and Scopus). Computer searches used combinations of key words relating to "EBV infections" and "atypical manifestation." Epstein-Barr virus is a herpes virus responsible for a lifelong latent infection in almost every adult. The primary infection concerns mostly children and presents with the clinical syndrome of infectious mononucleosis. However, Epstein-Barr virus infection may exhibit numerous rare, atypical and threatening manifestations. It may cause secondary infections and various complications of the respiratory, cardiovascular, genitourinary, gastrointestinal, and nervous systems. Epstein-Barr virus also plays a significant role in pathogenesis of autoimmune diseases, allergies, and neoplasms, with Burkitt lymphoma as the main representative of the latter. The mechanisms of these manifestations are still unresolved. Therefore, the main suggestions are direct viral invasion and chronic immune response due to the reactivation of the latent state of the virus, or even various DNA mutations. Physicians should be cautious about uncommon presentations of the viral infection and consider EBV as a causative agent when they encounter similar clinical pictures. Copyright © 2016 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Current status of atypical antipsychotics for the treatment of fibromyalgia.

Science.gov (United States)

Rico-Villademoros, F; Calandre, E P; Slim, M

2014-06-01

The treatment of fibromyalgia requires pharmacological and nonpharmacological therapies. The pharmacological treatment of fibromyalgia is limited to a few drugs that have been demonstrated to be moderately effective in some but not all dimensions of the disease. Therefore, the search for new drugs to treat this condition is warranted. Atypical antipsychotics offered an attractive alternative because they had been shown to be active against several key symptoms of fibromyalgia. The results of open-label studies, however, appear to indicate that atypical antipsychotics are poorly tolerated in patients with fibromyalgia, and only quetiapine XR has been studied in randomized controlled trials. Quetiapine XR has demonstrated effectiveness in treating comorbid major depression, anxiety and sleep disturbance. However, in two randomized controlled trials, quetiapine XR was not differentiated from placebo and failed to demonstrate noninferiority to amitriptyline in terms of improving overall symptomatology. The effect of quetiapine XR on pain and its usefulness as part of a combination pharmacological regimen should be further evaluated. Overall, the use of quetiapine (initiated at a low dose and slowly titrated) in fibromyalgia should be limited to patients with comorbid major depression or patients who are currently receiving other treatments and have unresolved and disabling depressive and/or anxiety symptoms. Copyright 2014 Prous Science, S.A.U. or its licensors. All rights reserved.

Case report: long-term survival of an infant syndromic patient affected by atypical teratoid-rhabdoid tumor

International Nuclear Information System (INIS)

Modena, Piergiorgio; Maestro, Roberta; Giangaspero, Felice; Massimino, Maura; Sardi, Iacopo; Brenca, Monica; Giunti, Laura; Buccoliero, Anna Maria; Pollo, Bianca; Biassoni, Veronica; Genitori, Lorenzo; Antonelli, Manila

2013-01-01

Atypical teratoid rhabdoid tumor (ATRT) patients display a dismal median overall survival of less than 1 year. A consistent fraction of cases carries de-novo SMARCB1/INI1 constitutional mutations in the setting of the “rhabdoid tumor predisposition syndrome” and the outcome is worst in infant syndromic ATRT patients. We here describe a patient affected by mosaic Klinefelter syndrome and by rhabdoid tumor predisposition syndrome caused by constitutional SMARCB1/INI1 heterozygous mutation c.118C>T (Arg40X). Patient’s ATRT primary tumor occurred at 2 years of age concurrent with metastatic lesions. The patient was rendered without evidence of disease by combined surgery, high-dose poli-chemotherapy and craniospinal irradiation, followed by autologous hematopoietic stem cell transplantation. At the onset of a spinal lesion 5.5 years later, both tumors were pathologically and molecularly evaluated at the national central pathology review board and defined as ATRT in a syndromic patient, with strong evidence of a clonal origin of the two lesions. The patient was then treated according to SIOP guidelines and is now alive without evidence of disease 24 months after the detection of metastatic disease and 90 months after the original diagnosis. The report underscores the current utility of multiple comprehensive approaches for the correct diagnosis and clinical management of patients affected by rare and atypical brain neoplasms. Successful local control of disease and achievement of long-term survival is possible in ATRT patients even in the setting of rhabdoid tumor predisposition syndrome, infant age at diagnosis and metastatic spread of disease, thus justifying the efforts for the management of this severe condition

Atypical case of Reye's syndrome. Usefulness of CT for diagnosis and follow-up study

Energy Technology Data Exchange (ETDEWEB)

Maehara, Fumiaki; Goto, Katsuya; Okudera, Toshio; Mitsudome, Akihisa; Hara, Kunio; Shiraishi, Masayuki [Fukuoka Univ. (Japan)

1982-12-01

An atypical case of Reye's syndrome was reported with emphasis on usefulness of CT for the diagnosis and follow-up study of this disease. The patient was a 13-month-old girl who had been transferred to our hospital because of status epilepticus, a comatous state and a high temperature. She was diagnosed as having Reye's syndrome according to data of liver function tests, findings in CSF and body CT which revealed swelling of the liver with diminished attenuation value suggesting fatty infiltration. However, there were atypical features in this patient: epileptic seizures since age 5 months, no vomiting at the time of onset and no evidence of brain swelling on CT in acute phase. She was discharged 2 months later with impaired neuropsychological functions of marked degree. When she was 2 year-old, she again went into status epilepticus, was comatous and had a high temperature. She was dead when she arrived at emergency room of our hospital. Autopsy findings revealed features of Reye's syndrome as follows: abundant accumulation of small fat droplets without nuclear displacement in the liver, fatty infiltration in the kidney and myocardium, and mild swelling in the cerebral cortex with marked ventricular dilatation. The possibility of recurrence of Reye's syndrome was discussed based on the clinical and autopsy findings. The value of CT in the diagnosis and the follow-up study of this disease was emphasized.

A case of atypical progressive supranuclear palsy

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Spaccavento S

2013-12-01

Full Text Available Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy Background: Progressive supranuclear palsy (PSP is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS and aphasia. Aim: We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments. Methods: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results: Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20, low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall, and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion: Our case highlights the heterogeneity of the clinical features in

Non-invasive coronary angiography for patients with acute atypical chest pain discharged after negative screening including maximal negative treadmill stress test. A prospective study.

Science.gov (United States)

Bonello, L; Armero, S; Jacquier, A; Com, O; Sarran, A; Sbragia, P; Panuel, M; Arques, S; Paganelli, F

2009-05-01

Among patients admitted in the emergency department for acute atypical chest pain those with an acute coronary syndrome (ACS) who are mistakenly discharged home have high mortality. A recent retrospective study has demonstrated that multislice computed tomography (MSCT) coronary angiography could improve triage of these patients. We aimed to prospectively confirm these data on patients with a negative screening including maximal treadmill stress. 30 patients discharged from the emergency department after negative screening for an ACS were included. All patients underwent MSCT angiography of the coronary artery. Patients with coronary atheroma on MSCT had an invasive coronary angiography to confirm these findings. Seven patients (23%) had obstructive coronary artery disease on MSCT. Invasive coronary angiography (ICA) confirmed the diagnosis in all patients. In patients with no previously known coronary artery disease admitted to the emergency department with atypical acute chest pain and discharged after negative screening, including maximal treadmill stress test, MSCT coronary angiography is useful for the diagnosis of obstructive coronary artery disease.

IPHAS A-TYPE STARS WITH MID-INFRARED EXCESSES IN SPITZER SURVEYS

International Nuclear Information System (INIS)

Hales, Antonio S.; Barlow, Michael J.; Drew, Janet E.; Unruh, Yvonne C.; Greimel, Robert; Irwin, Michael J.; Gonzalez-Solares, Eduardo

2009-01-01

We have identified 17 A-type stars in the Galactic Plane that have mid-infrared (mid-IR) excesses at 8 μm. From observed colors in the (r' - Hα) - (r' - i') plane, we first identified 23,050 early A-type main-sequence (MS) star candidates in the Isaac Newton Photometric H-Alpha Survey (IPHAS) point source database that are located in Spitzer Galactic Legacy Mid-Plane Survey Extraordinaire Galactic plane fields. Imposing the requirement that they be detected in all seven Two Micron All Sky Survey and Infrared Astronomical Satellite bands led to a sample of 2692 candidate A-type stars with fully sampled 0.6 to 8 μm spectral energy distributions (SEDs). Optical classification spectra of 18 of the IPHAS candidate A-type MS stars showed that all but one could be well fitted using MS A-type templates, with the other being an A-type supergiant. Out of the 2692 A-type candidates 17 (0.6%) were found to have 8 μm excesses above the expected photospheric values. Taking into account non-A-Type contamination estimates, the 8 μm excess fraction is adjusted to ∼0.7%. The distances to these sources range from 0.7 to 2.5 kpc. Only 10 out of the 17 excess stars had been covered by Spitzer MIPSGAL survey fields, of which five had detectable excesses at 24 μm. For sources with excesses detected in at least two mid-IR wavelength bands, blackbody fits to the excess SEDs yielded temperatures ranging from 270 to 650 K, and bolometric luminosity ratios L IR /L * from 2.2 x 10 -3 - 1.9 x 10 -2 , with a mean value of 7.9 x 10 -3 (these bolometric luminosities are lower limits as cold dust is not detectable by this survey). Both the presence of mid-IR excesses and the derived bolometric luminosity ratios are consistent with many of these systems being in the planet-building transition phase between the early protoplanetary disk phase and the later debris disk phase.

Preparation and cellular response of porous A-type carbonated hydroxyapatite nanoceramics

Energy Technology Data Exchange (ETDEWEB)

Li Bo, E-mail: Leewave@126.com [Institute of Biomaterials and Living Cell Imaging Technology, School of Metallurgy and Materials Engineering, Chongqing University of Science and Technology, Chongqing 401331 (China) and National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Liao Xiaoling [Institute of Biomaterials and Living Cell Imaging Technology, School of Metallurgy and Materials Engineering, Chongqing University of Science and Technology, Chongqing 401331 (China); Zheng Li [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); He Huawei [Department of Prosthodontics, Beijing Stomatological Hospital, Capital Medical University, Beijing, 100050 (China); Wang Hong [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Fan Hongsong, E-mail: hsfan68@hotmail.com [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Zhang Xingdong [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China)

2012-05-01

Microwave sintering using the activated carbon as embedding material was applied in preparation of porous A-type carbonated hydroxyapatite ceramics with nano(nCHA) and submicron (mCHA) structure. By examining the linear shrinkages and the compressive strengths of samples at different temperatures, a suitable microwave sintering temperature was achieved. The microwave sintering method was successfully used to prepare A-type CHA with nano or submicron structure, and the mechanism of the formation of A-type carbonate groups was discussed also. Compared with the samples prepared by the conventional sintering method (mHA), the nCHA bioceramics synthesized by the microwave sintering approach had smaller grain size and more uniform microstructure, and showed a compressive strength similar to the conventional samples. In vitro dissolution test proved that nCHA exhibits better degradation property in comparison to pure HA. Rat osteoblasts were cultured with nCHA, mCHA and mHA to evaluate their biocompatibility, and nCHA showed significant enhancement of cells in attachment, proliferation and differentiation. In conclusion, carbonate groups can be easily introduced to HA crystal structure using the activated carbon as embedding material, and microwave sintering is an effective and simple method in preparing A-type CHA with a nanostructure. Results from this in vitro biological study suggest that porous A-type carbonated hydroxyapatite nanoceramics may be a much better candidate for clinical use in terms of bioactivity. - Highlights: Black-Right-Pointing-Pointer We prepared porous A-type carbonated hydroxyapatite nanoceramics with microwave sintering. Black-Right-Pointing-Pointer We examined physico-chemical characterization and osteoblast response. Black-Right-Pointing-Pointer The nanoceramics have a comparable compressive strength to samples with conventional sintering method. Black-Right-Pointing-Pointer The nanoceramics enhance degradation property, osteoblast

Conns' syndrome - atypical presentations

International Nuclear Information System (INIS)

Kumar, K V S Hari; Modi, K D; Jha, Sangeeta; Jha, Ratan

2009-01-01

Primary hyperaldosteronism (Conns' syndrome) commonly presents with a combination of clinical features of hypokalemia and hypertension. Atypical presentations like normotension, normokalemia and neurological ailments are described in few cases. We encountered two such cases, the first presenting with acute neurological complaint and second case having insignificant hypertension. Both the patients had a characteristic biochemical and imaging profile consistent with primary hyperaldosteronism and responded to surgical resection of adrenal adenoma. (author)

Acylcarnitines profile best predicts survival in horses with atypical myopathy.

Directory of Open Access Journals (Sweden)

François Boemer

Full Text Available Equine atypical myopathy (AM is caused by hypoglycin A intoxication and is characterized by a high fatality rate. Predictive estimation of survival in AM horses is necessary to prevent unnecessary suffering of animals that are unlikely to survive and to focus supportive therapy on horses with a possible favourable prognosis of survival. We hypothesized that outcome may be predicted early in the course of disease based on the assumption that the acylcarnitine profile reflects the derangement of muscle energetics. We developed a statistical model to prognosticate the risk of death of diseased animals and found that estimation of outcome may be drawn from three acylcarnitines (C2, C10:2 and C18 -carnitines with a high sensitivity and specificity. The calculation of the prognosis of survival makes it possible to distinguish the horses that will survive from those that will die despite severe signs of acute rhabdomyolysis in both groups.

Acylcarnitines profile best predicts survival in horses with atypical myopathy

Science.gov (United States)

Detilleux, Johann; Cello, Christophe; Amory, Hélène; Marcillaud-Pitel, Christel; Richard, Eric; van Galen, Gaby; van Loon, Gunther; Lefère, Laurence; Votion, Dominique-Marie

2017-01-01

Equine atypical myopathy (AM) is caused by hypoglycin A intoxication and is characterized by a high fatality rate. Predictive estimation of survival in AM horses is necessary to prevent unnecessary suffering of animals that are unlikely to survive and to focus supportive therapy on horses with a possible favourable prognosis of survival. We hypothesized that outcome may be predicted early in the course of disease based on the assumption that the acylcarnitine profile reflects the derangement of muscle energetics. We developed a statistical model to prognosticate the risk of death of diseased animals and found that estimation of outcome may be drawn from three acylcarnitines (C2, C10:2 and C18 -carnitines) with a high sensitivity and specificity. The calculation of the prognosis of survival makes it possible to distinguish the horses that will survive from those that will die despite severe signs of acute rhabdomyolysis in both groups. PMID:28846683

Atypical imaging of spinal tuberculosis: a case report and review of ...

African Journals Online (AJOL)

... is a rare imaging manifestation and diagnosis was confirmed by pathology after the surgery. Therefore atypical imaging is often appeared in clinical practice and it is meaningful and necessary for the diagnosis of atypical spinal tuberculosis combined with multiple organ tuberculosis. Pan African Medical Journal 2016; 24 ...

Endoscopic management of pancreatic pseudocysts at atypical locations.

Science.gov (United States)

Bhasin, Deepak Kumar; Rana, Surinder Singh; Nanda, Mohit; Chandail, Vijant Singh; Masoodi, Ibrahim; Kang, Mandeep; Kalra, Navin; Sinha, Saroj Kant; Nagi, Birinder; Singh, Kartar

2010-05-01

There is paucity of data on endoscopic management of pseudocysts at atypical locations. We evaluated the efficacy of endoscopic transpapillary nasopancreatic drain (NPD) placement in the management of pseudocysts of pancreas at atypical locations. Eleven patients with pseudocysts at atypical locations were treated with attempted endoscopic transpapillary nasopancreatic drainage. On endoscopic retrograde pancreatography (ERP), a 5-F NPD was placed across/near the site of duct disruption. Three patients each had mediastinal, intrahepatic, and intra/perisplenic pseudocysts and one patient each had renal and pelvic pseudocyst. Nine patients had chronic pancreatitis whereas two patients had acute pancreatitis. The size of the pseudocysts ranged from 2 to 15 cm. On ERP, the site of ductal disruption was in the body of pancreas in five patients (45.4%), and tail of pancreas in six patients (54.6%). All the patients had partial disruption of pancreatic duct. The NPD was successfully placed across the disruption in 10 of the 11 patients (90.9%) and pseudocysts resolved in 4-8 weeks. One of the patients developed fever, 5 days after the procedure, which was successfully treated by intravenous antibiotics. In another patient, NPD became blocked 12 days after the procedure and was successfully opened by aspiration. The NPD slipped out in one of the patient with splenic pseudocyst and was replaced with a stent. There was no recurrence of symptoms or pseudocysts during follow-up of 3-70 months. Pancreatic pseudocysts at atypical locations with ductal communication and partial ductal disruption that is bridged by NPD can also be effectively treated with endoscopic transpapillary NPD placement.

Interesting coincidence of atypical TSH-secreting pituitary adenoma and chronic lymphocytic leukemia.

Science.gov (United States)

Bolanowski, Marek; Zieliński, Grzegorz; Jawiarczyk-Przybyłowska, Aleksandra; Maksymowicz, Maria; Potoczek, Stanisław; Syrycka, Joanna; Podgórski, Jan K

2014-01-01

Thyrotropin-secreting adenomas (TSH-oma) are very rare pituitary tumours. They are macroadenomas usually presenting with signs and symptoms of hyperthyroidism, and mass effects. They can co-secrete other hormones such as growth hormone or prolactin. Different malignancies, including haematological ones, are reported in patients with pituitary diseases. Chronic lymphocytic leukemia (CLL) occurs mostly in older patients, more often in males. CLL is associated with increased risk of second malignancies such as other blood neoplasms, skin and solid tumours. We present a successful neurosurgical outcome in a patient with an interesting coincidence of atypical TSH-oma and asymptomatic CLL.

Assesment, treatment and prevention of atypical hemolytic uremic syndrome

Directory of Open Access Journals (Sweden)

Azar Nickavar

2013-01-01

Full Text Available Hemolytic uremic syndrome (HUS is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1 D+ HUS: Presentation with a preceding diarrhea; (2 typical HUS: D+ HUS with a single and self-limited episode; (3 atypical HUS (aHUS: Indicated those with complement dysregulation; (4 recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA after improvement of hematologic abnormalities; and (5 familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

Genetic structure of typical and atypical populations of Candida albicans from Africa.

Science.gov (United States)

Forche, A; Schönian, G; Gräser, Y; Vilgalys, R; Mitchell, T G

1999-11-01

Atypical isolates of the pathogenic yeast Candida albicans have been reported with increasing frequency. To investigate the origin of a set of atypical isolates and their relationship to typical isolates, we employed a combination of molecular phylogenetic and population genetic analyses using rDNA sequencing, PCR fingerprinting, and analysis of co-dominant DNA nucleotide polymorphisms to characterize the population structure of one typical and two atypical populations of C. albicans from Angola and Madagascar. The extent of clonality and recombination was assessed in each population. The analyses revealed that the structure of all three populations of C. albicans was predominantly clonal but, as in previous studies, there was also evidence for recombination. Allele frequencies differed significantly between the typical and the atypical populations, suggesting very low levels of gene flow between them. However, allele frequencies were quite similar in the two atypical C. albicans populations, suggesting that they are closely related. Phylogenetic analysis of partial sequences encoding the nuclear 26S rDNA demonstrated that all three populations belong to a single monophyletic group, which includes the type strain of C. albicans. Copyright 1999 Academic Press.

Non-diabetic atypical necrobiosis lipoidica

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Mittal R

1994-01-01

Full Text Available One 8 year female child had asymptomatic, anaesthetic, hypohidrotic, atrophic, yellowish, waxy plaque on the front of left thigh since 2 months. No nerve thickening was observed clinically or histopathologically. Hyperkeratosis, follicular keratosis, epidermal atrophy, degeneration of collagen, mononuclear granulomas and perivascular mononuclear infiltrate confirmed the clinical diagnosis of atypical necrobiosis lipoidica.

Atypical course oferysipelas and coexisting infections. Case study and literature review

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Anna Martyniuk

2015-12-01

Full Text Available Erysipelas is  an acute, erythematous, rapidly spreading skin infection, usually caused by beta-haemolytic group A Streptococcus bacteria. The disease is usually located on the legs and toes, less frequently on the face. One of the predisposing factors for the development of erysipelas are coexisting infections. The aim of the work was to attempt to determine whether there was any connection between the atypical course of erysipelas and Helicobacter pylori infection in a 47-year-old female patient. The patient had a history of treatment for chronic otitis media with effusion and recurring abdominal pain. The current disease started abruptly with fever, erythematous skin lesion located on the right cheek and severe pain in the right ear. After a few days, the facial erythema got worse, oedema appeared on the right side of the face and redness, oedema and pain in the auricle could be observed. After examinations by an ENT specialist and a dermatologist, erysipelas of the face, auricle and external auditory meatus was diagnosed. The diagnosis was confirmed by bacteriological examination. As a result of antibiotic therapy skin lesions subsided and the patient’s general condition improved. As the dyspeptic symptoms exacerbated, a diagnostic test was performed and a coexisting Helicobacter pylori infection was diagnosed. The overall clinical picture and data obtained from medical literature suggest that the coexisting Helicobacter pylori infection could have contributed to both the chronic otitis media with effusion and atypical course of erysipelas. According to medical literature, in the case of patients with dyspeptic symptoms, Helicobacter pylori bacteria can be transferred from the lining of the stomach upwards to the oral cavity, middle ear and paranasal sinuses. Kariya et al. in their review of original work suggested that Helicobacter pylori may contribute to the exacerbation of an existing inflammation in

Characterisation of an atypical manifestation of black band disease on Porites lutea in the Western Indian Ocean

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Mathieu Séré

2016-07-01

Full Text Available Recent surveys conducted on Reunion Island coral reefs revealed an atypical manifestation of black band disease on the main framework building coral, Porites lutea. This BBD manifestation (PorBBD presented a thick lighter-colored band, which preceded the typical BBD lesion. Whilst BBD aetiology has been intensively described worldwide, it remains unclear if corals with apparently similar lesions across coral reefs are affected by the same pathogens. Therefore, a multidisciplinary approach involving field surveys, gross lesion monitoring, histopathology and 454-pyrosequencing was employed to provide the first comprehensive characterization of this particular manifestation. Surveys conducted within two geomorphological zones over two consecutive summers and winters showed spatial and seasonal patterns consistent with those found for typical BBD. Genetic analyses suggested an uncharacteristically high level of Vibrio spp. bacterial infection within PorBBD. However, microscopic analysis revealed high densities of cyanobacteria, penetrating the compromised tissue as well as the presence of basophilic bodies resembling bacterial aggregates in the living tissue, adjacent to the bacterial mat. Additionally, classical BBD-associated cyanobacterial strains, genetically related to Pseudoscillatoria coralii and Roseofilum reptotaenium were identified and isolated and the presence of sulfate-reducers or sulfide-oxidizers such as Desulfovibrio and Arcobacter, previously shown to be associated with anoxic microenvironment within typical BBD was also observed, confirming that PorBBD is a manifestation of classical BBD.

Stereological estimation of nuclear volume in benign and atypical meningiomas

DEFF Research Database (Denmark)

Madsen, C; Schrøder, H D

1993-01-01

A stereological estimation of nuclear volume in benign and atypical meningiomas was made. The aim was to investigate whether this method could discriminate between these two meningeal neoplasms. The difference was significant and it was moreover seen that there was no overlap between the two groups....... The results demonstrate that atypical meningiomas can be distinguished from benign meningiomas by an objective stereological estimation of nuclear volume....

Atypical Manifestation of Vestibular Schwannoma

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Webster, Guilherme

2013-09-01

Full Text Available Introduction: Vestibular schwannoma (also known as acoustic neuroma is a benign tumor whose cells are derived from Schwann sheaths, which commonly occurs from the vestibular portion of the eighth cranial nerve. Furthermore, vestibular schwannomas account for ∼8% of intracranial tumors in adults and 80 to 90% of tumors of the cerebellopontine angle. Its symptoms are varied, but what stands out most is a unilateral sensorineural hearing loss, with a low index of speech recognition. Objective: Describe an atypical manifestation of vestibular schwannoma. Case Report: The 46-year-old woman had vertigo and binaural hearing loss and fullness, with ear, nose, and throat examination suggestive of cochlear injury. After 6 months, the patient developed worsening of symptoms and onset of right unilateral tinnitus. In further exams the signs of cochlear damage remained, except for the vestibular test (hyporeflexia. Magnetic resonance imaging showed an expansive lesion in the right cerebellopontine angle. Discussion: This report warns about the atypical manifestations of vestibular schwannoma, which must always be remembered in investigating and diagnosing hearing loss.

A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I: a case report

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Kvittingen Eli-Anne

2009-12-01

Full Text Available Abstract A male patient, born to unrelated Belgian parents, presented at 4 months with epistaxis, haematemesis and haematochezia. On physical examination he presented petechiae and haematomas, and a slightly enlarged liver. Serum transaminases were elevated to 5-10 times upper limit of normal, alkaline phosphatases were 1685 U/L (180 s ( Fumarylacetoacetase (FAH protein and activity in cultured fibroblasts and liver tissue were decreased but not absent. 4-hydroxyphenylpyruvate dioxygenase activity in liver was normal, which is atypical for tyrosinemia type I. A novel mutation was found in the FAH gene: c.103G>A (Ala35Thr. In vitro expression studies showed this mutation results in a strongly decreased FAH protein expression. Dietary treatment with phenylalanine and tyrosine restriction was initiated at 4 months, leading to complete clinical and biochemical normalisation. The patient, currently aged 12 years, shows a normal physical and psychomotor development. This is the first report of mild tyrosinemia type I disease caused by an Ala35Thr mutation in the FAH gene, presenting atypically without increase of the diagnostically important toxic metabolites succinylacetone and succinylacetoacetate.

Atypical presentation of macrophagic myofasciitis 10 years post vaccination.

LENUS (Irish Health Repository)

Ryan, Aisling M

2012-02-03

Macrophagic myofasciitis (MMF) is an uncommon inflammatory disorder of muscle believed to be due to persistence of vaccine-derived aluminium hydroxide at the site of injection. The condition is characterised by diffuse myalgias, arthralgia and fatigue. We describe a patient with histologically confirmed MMF whose presentation was atypical with left chest and upper limb pain beginning more than 10 years post vaccination. Treatment with steroids led to symptomatic improvement. Although rare, clinicians should consider MMF in cases of atypical myalgia.

Atypical Presentation of Gelsolin Amyloidosis in a Man of African Descent with a Novel Mutation in the Gelsolin Gene.

Science.gov (United States)

Oregel, Karlos Z; Shouse, Geoffrey P; Oster, Cyrus; Martinez, Freddy; Wang, Jun; Rosenzweig, Michael; Deisch, Jeremy K; Chen, Chien-Shing; Nagaraj, Gayathri

2018-03-30

BACKGROUND Gelsolin amyloidosis is a very rare systemic disease presenting with a pathognomonic triad of corneal lattice dystrophy, cutis laxa, and polyneuropathy. The disease is mostly restricted to a Finnish population with known mutations (G654A, G654T) in exon 4 of the gelsolin gene. The mutations lead to errors in protein processing and folding, and ultimately leads to deposition of an amyloidogenic fragment in the extracellular space, causing the symptoms of disease. CASE REPORT We present a case of gelsolin amyloidosis in a male of African descent with an atypical clinical presentation including fevers, skin rash, polyneuropathy, and anemia. Gelsolin amyloidosis was diagnosed based on mass spectrometry of tissue samples. Importantly, a novel mutation in the gelsolin gene (C1375G) in exon 10 was found in this patient. His atypical presentation can possibly be attributed to the presence of a novel mutation in the gelsolin gene as the likely underlying cause of the syndrome. PCR primers were used to amplify the gelsolin gene from genomic DNA. Purified PCR products were then shipped to Eton Biosciences (San Diego, CA) for sequencing. CONCLUSIONS This study carries several important lessons relevant to the practice of medicine. First, the differential diagnosis for multisystem disease presentations should always include amyloidosis. Second, despite what has been uncovered about the molecular biology of disease, there is always more that can be discovered. Finally, further work to verify the link between this mutation and the clinical syndrome is still needed, as are effective treatments for this disease.

Self-limiting atypical antipsychotics-induced edema: Clinical cases and systematic review

OpenAIRE

Musa Usman Umar; Aminu Taura Abdullahi

2016-01-01

A number of atypical antipsychotics have been associated with peripheral edema. The exact cause is not known. We report two cases of olanzapine-induced edema and a brief review of atypical antipsychotic-induced edema, possible risk factors, etiology, and clinical features. The recommendation is given on different methods of managing this side effect.

Self-limiting Atypical Antipsychotics-induced Edema: Clinical Cases and Systematic Review.

Science.gov (United States)

Umar, Musa Usman; Abdullahi, Aminu Taura

2016-01-01

A number of atypical antipsychotics have been associated with peripheral edema. The exact cause is not known. We report two cases of olanzapine-induced edema and a brief review of atypical antipsychotic-induced edema, possible risk factors, etiology, and clinical features. The recommendation is given on different methods of managing this side effect.

Recurrent atypical hemolytic uremic syndrome after renal transplantation: treatment with eculizumab

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Ana B. Latzke

2018-04-01

Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare entity. It is characterized by a thrombotic microangiopathy (nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure, with a typical histopathology of thickening of capillary and arteriolar walls and an obstructive thrombosis of the vascular lumen. The syndrome is produced by a genetic or acquired deregulation of the alternative pathway of the complement system, with high rates of end stage renal disease, post-transplant recurrence, and high mortality. Mutations associated with factor H, factor B and complement C3 show the worst prognosis. Even though plasma therapy is occasionally useful, eculizumab is effective both for treatment and prevention of post-transplant recurrence. We describe here an adult case of congenital aHUS (C3 mutation under preventive treatment with eculizumab after renal transplantation, with neither disease recurrence nor drug-related adverse events after a 36-months follow-up.

ATYPICAL AUTISM IN CHILDREN: THE SPECIFIC FEATURES OF THEIR SOMATIC AND NEUROLOGICAL STATUS AND OUTPATIENT FOLLOW-UP BY A GENERAL PEDIATRICIAN

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M. Yu. Bobylоva

2014-01-01

Full Text Available Autism spectrum disorders in children are attracting the increasing attention of general practitioners, which is due to the specification of diagnostic criteria and to the recognition of the fact that there is atypical autism that is a syndrome of many different diseases. The authors consider in detail the theory of autism and the clinical manifestations of atypical autism, including possible neurological and somatic disorders. Individual attention is placed on the association of autistic disorder with epilepsy or epileptiform activity on the electroencephalogram. An emphasis is made on the need for an interdisciplinary approach and on the interaction of clinicians of different specialties when treating patients with autistic disorder.

[Venous thrombosis of atypical location in patients with cancer].

Science.gov (United States)

Campos Balea, Begoña; Sáenz de Miera Rodríguez, Andrea; Antolín Novoa, Silvia; Quindós Varela, María; Barón Duarte, Francisco; López López, Rafael

2015-01-01

Venous thromboembolism (VTE) is a complication that frequently occurs in patients with neoplastic diseases. Several models have therefore been developed to identify patient subgroups diagnosed with cancer who are at increased risk of developing VTE. The most common forms of thromboembolic episodes are deep vein thrombosis in the lower limbs and pulmonary thromboembolism. However, venous thrombosis is also diagnosed in atypical locations. There are few revisions of unusual cases of venous thrombosis. In most cases, VTE occurs in the upper limbs and in the presence of central venous catheters, pacemakers and defibrillators. We present the case of a patient diagnosed with breast cancer and treated with surgery, chemotherapy and radiation therapy who developed a thrombosis in the upper limbs (brachial and axillary). Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

Summary of the comparative effectiveness review on off-label use of atypical antipsychotics.

Science.gov (United States)

Maher, Alicia R; Theodore, George

2012-06-01

Conventional and atypical antipsychotic medications are approved by the FDA for treatment of schizophrenia and bipolar disorder. Over many decades, the widespread use of conventional antipsychotics produced various side effects requiring additional medications, such as the atypical antipsychotics. Beginning in 2006, 9 atypical antipsychotic drugs have been approved by the FDA for indications that were previously off-label uses: aripiprazole (as augmentation for major depressive disorder [MDD] and for autism spectrum disorders), asenapine, clozapine, iloperidone, olanzapine (in combination with fluoxetine for MDD and bipolar depression), paliperidone, quetiapine (quetiapine and quetiapine XR [extended release] as monotherapy in bipolar depression and quetiapine XR as augmentation for MDD), risperidone (for autism spectrum disorders), and ziprasidone. In 2006, the Agency for Healthcare Research and Quality (AHRQ) published a systematic review on the comparative effectiveness of off-label uses of atypical antipsychotics. Since that time, numerous studies have been published evaluating these therapies in various new off-label uses; new or increased adverse effects have been observed with off-label uses; new atypical antipsychotics have been approved; and previously off-label uses have been approved for some atypical antipsychotics. Hence, AHRQ published an updated review in September 2011 that summarized the benefits and harms of atypical antipsychotics in the treatment of attention-deficit hyperactivity disorder/attention deficit disorder (ADHD), anxiety, behavioral disturbances of dementia and severe geriatric agitation, depression, eating disorders, insomnia, obsessive-compulsive disorder (OCD), personality disorder, post-traumatic stress disorder (PTSD), substance use and dependence disorders, and Tourette's syndrome. The new report also investigated topics for which data in the previous report were found to be insufficient to make conclusions, including

Self-limiting Atypical Antipsychotics-induced Edema: Clinical Cases and Systematic Review

Science.gov (United States)

Umar, Musa Usman; Abdullahi, Aminu Taura

2016-01-01

A number of atypical antipsychotics have been associated with peripheral edema. The exact cause is not known. We report two cases of olanzapine-induced edema and a brief review of atypical antipsychotic-induced edema, possible risk factors, etiology, and clinical features. The recommendation is given on different methods of managing this side effect. PMID:27335511

Loss of Drosophila A-type lamin C initially causes tendon abnormality including disintegration of cytoskeleton and nuclear lamina in muscular defects.

Science.gov (United States)

Uchino, Ryo; Nonaka, Yu-Ki; Horigome, Tuneyoshi; Sugiyama, Shin; Furukawa, Kazuhiro

2013-01-01

Lamins are the major components of nuclear envelope architecture, being required for both the structural and informational roles of the nuclei. Mutations of lamins cause a spectrum of diseases in humans, including muscular dystrophy. We report here that the loss of the A-type lamin gene, lamin C in Drosophila resulted in pupal metamorphic lethality caused by tendon defects, matching the characteristics of human A-type lamin revealed by Emery-Dreifuss muscular dystrophy (EDMD). In tendon cells lacking lamin C activity, overall cell morphology was affected and organization of the spectraplakin family cytoskeletal protein Shortstop which is prominently expressed in tendon cells gradually disintegrated, notably around the nucleus and in a manner correlating well with the degradation of musculature. Furthermore, lamin C null mutants were efficiently rescued by restoring lamin C expression to shortstop-expressing cells, which include tendon cells but exclude skeletal muscle cells. Thus the critical function of A-type lamin C proteins in Drosophila musculature is to maintain proper function and morphology of tendon cells. Copyright © 2012 Elsevier Inc. All rights reserved.

An Atypical Localized Form of Hidradenitis Suppurativa of the Jawline and Neck Mimicking Severe Cystic Acne on Presentation.

Science.gov (United States)

Castrillón Velásquez, María Adriana; Kim, Minhee; Tan, Mei-Heng; Tran, Kim; Murrell, Dedee F

2017-10-01

Hidradenitis suppurativa (HS) is a chronic and debilitating suppurative disease primarily affecting the axillae, perineum, and inframammary regions, where apocrine sweat glands are present. However, HS can occur in atypical locations. We present an interesting case of a 40-year-old man who developed chronic painful subcutaneous nodules, deep sinus tracts, and abscesses involving the jawline and the anterior aspect of the neck as the only parts of the body affected and who responded satisfactorily to adalimumab and laser hair removal treatment. This case is relevant because it helps clinicians to remember that HS may be isolated to atypical locations, such as the anterior aspect of the neck and chin. It also supports another possible HS pathogenesis which consists of the occlusion of terminal hair follicles rather than being essentially a disorder of the apocrine glands.

A critical analysis of the new equal pay provisions relating to atypical ...

African Journals Online (AJOL)

Keywords: Equal pay; Labour Relations Act; equal pay for atypical employees; atypical employment; sections 198A-198D of the Labour Relations Act; Agency Workers Regulations 2010; Fixed-term Employees (Prevention of Less Favourable Treatment) Regulations 2002; Part-time Workers (Prevention of Less Favourable ...

Use of atypical antipsychotics in nursing homes and pharmaceutical marketing.

Science.gov (United States)

Pimentel, Camilla B; Donovan, Jennifer L; Field, Terry S; Gurwitz, Jerry H; Harrold, Leslie R; Kanaan, Abir O; Lemay, Celeste A; Mazor, Kathleen M; Tjia, Jennifer; Briesacher, Becky A

2015-02-01

To describe the current extent and type of pharmaceutical marketing in nursing homes (NHs) in one state and to provide preliminary evidence for the potential influence of pharmaceutical marketing on the use of atypical antipsychotics in NHs. Nested mixed-methods, cross-sectional study of NHs in a cluster randomized trial. Forty-one NHs in Connecticut. NH administrators, directors of nursing, and medical directors (n = 93, response rate 75.6%). Quantitative data, including prescription drug dispensing data (September 2009-August 2010) linked with Nursing Home Compare data (April 2011), were used to determine facility-level prevalence of atypical antipsychotic use, facility-level characteristics, NH staffing, and NH quality. Qualitative data, including semistructured interviews and surveys of NH leaders conducted in the first quarter of 2011, were used to determine encounters with pharmaceutical marketing. Leadership at 46.3% of NHs (n = 19) reported pharmaceutical marketing encounters, consisting of educational training, written and Internet-based materials, and sponsored training. No association was detected between level of atypical antipsychotic prescribing and reports of any pharmaceutical marketing by at least one NH leader. NH leaders frequently encounter pharmaceutical marketing through a variety of ways, although the impact on atypical antipsychotic prescribing is unclear. © 2015, Copyright the Authors Journal compilation © 2015, The American Geriatrics Society.

Reproductive Health in Men and Women With Vasculitis

Science.gov (United States)

2014-06-25

Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

Atypical, unusual, and misleading imaging presentations of spondylolysis.

Science.gov (United States)

Viana, Sergio Lopes; Viana, Maria Angélica de Carvalho Barbosa; de Alencar, Eduardo Lopes Carreiro

2015-09-01

Although lumbar spondylolysis is a widely known and easily recognizable condition in its typical presentation, there are some less well-known forms that may occasionally be challenging and/or demand special attention on imaging. Examples include: acute and/or incomplete lesions; unilateral defects; lesions at unusual levels (cervical, upper lumbar, and multi-level spondylolyses); iatrogenic lesions; non-isthmic spondylolysis; and spondylolysis related to underlying diseases. In addition to their atypical, uncommon or confusing imaging presentations, these forms of spondylolysis are far rarer than the classic type and have been described, to a great extent, in the surgical literature, thus reducing the awareness of radiologists about them and raising the potential for misdiagnosis and inadequate treatment. In this review the authors address these special manifestations of spondylolysis, stressing the more important features to be considered in the differential diagnosis and the impact of a precise diagnosis of spondylolysis on the patient's care.

Atypical femur fractures associated with bisphosphonates: from prodrome to resolution

Directory of Open Access Journals (Sweden)

Braulio Sastre-Jala

2015-10-01

Full Text Available Atypical fractures related to the prolonged use of bisphosphonates are caused by low energy mechanisms and are characterized by oblique and transverse lines and frequent bilateralism. We present a clinical case of a patient who we believe illustrates, both in clinical and radiological aspects, the new definition of atypical femur fracture related to treatment using bisphosphonates treated conservatively and successfully with discharge and teriparatide 20 mcg/80 mcl s.c./24h. The appearance of painful symptoms in the upper thigh, especially if bilateral, in patients treated with bisphosphonates for long periods of time, makes it necessary to dismiss bone lesions that might otherwise suggest atypical fracture. In those cases where the fracture is incomplete, restoring bone metabolism through the administration of teriparatide 20 mcg/80 mcl s.c./24h could prevent displaced fractures.

Early postnatal soluble FGFR3 therapy prevents the atypical development of obesity in achondroplasia.

Science.gov (United States)

Saint-Laurent, Celine; Garcia, Stephanie; Sarrazy, Vincent; Dumas, Karine; Authier, Florence; Sore, Sophie; Tran, Albert; Gual, Philippe; Gennero, Isabelle; Salles, Jean-Pierre; Gouze, Elvire

2018-01-01

Achondroplasia is a rare genetic disease is characterized by abnormal bone development and early obesity. While the bone aspect of the disease has been thoroughly studied, early obesity affecting approximately 50% of them during childhood has been somewhat neglected. It nevertheless represents a major health problem in these patients, and is associated to life-threatening complications including increasing risk of cardiovascular pathologies. We have thus decided to study obesity in patients and to use the mouse model to evaluate if soluble FGFR3 therapy, an innovative treatment approach for achondroplasia, could also impact the development of this significant complication. To achieve this, we have first fully characterized the metabolic deregulations in these patients by conducting a longitudinal retrospective study, in children with achondroplasia Anthropometric, densitometric measures as well as several blood parameters were recorded and compared between three age groups ranging from [0-3], [4-8] and [9-18] years old. Our results show unexpected results with the development of an atypical obesity with preferential fat deposition in the abdomen that is remarkably not associated with classical complications of obesity such as diabetes or hypercholosterolemia. Because it is not associated with diabetes, the atypical obesity has not been studied in the past even though it is recognized as a real problem in these patients. These results were validated in a murine model of achondroplasia (Fgfr3ach/+) where similar visceral adiposity was observed. Unexpected alterations in glucose metabolism were highlighted during high-fat diet. Glucose, insulin or lipid levels remained low, without the development of diabetes. Very interestingly, in achondroplasia mice treated with soluble FGFR3 during the growth period (from D3 to D22), the development of these metabolic deregulations was prevented in adult animals (between 4 and 14 weeks of age). The lean-over-fat tissues ratio was

Breast Metastases from Extramammary Malignancies: Typical and Atypical Ultrasound Features

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Mun, Sung Hee [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710 (Korea, Republic of); Department of Radiology, Catholic University of Daegu College of Medicine, Daegu 712-702 (Korea, Republic of); Ko, Eun Young; Han, Boo-Kyung; Shin, Jung Hee [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710 (Korea, Republic of); Kim, Suk Jung [Department of Radiology, Inje University College of Medicine, Busan Paik Hospital, Busan 614-735 (Korea, Republic of); Cho, Eun Yoon [Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710 (Korea, Republic of)

2014-07-01

Breast metastases from extramammary malignancies are uncommon. The most common sources are lymphomas/leukemias and melanomas. Some of the less common sources include carcinomas of the lung, ovary, and stomach, and infrequently, carcinoid tumors, hypernephromas, carcinomas of the liver, tonsil, pleura, pancreas, cervix, perineum, endometrium and bladder. Breast metastases from extramammary malignancies have both hematogenous and lymphatic routes. According to their routes, there are common radiological features of metastatic diseases of the breast, but the features are not specific for metastases. Typical ultrasound (US) features of hematogenous metastases include single or multiple, round to oval shaped, well-circumscribed hypoechoic masses without spiculations, calcifications, or architectural distortion; these masses are commonly located superficially in subcutaneous tissue or immediately adjacent to the breast parenchyma that is relatively rich in blood supply. Typical US features of lymphatic breast metastases include diffusely and heterogeneously increased echogenicities in subcutaneous fat and glandular tissue and a thick trabecular pattern with secondary skin thickening, lymphedema, and lymph node enlargement. However, lesions show variable US features in some cases, and differentiation of these lesions from primary breast cancer or from benign lesions is difficult. In this review, we demonstrate various US appearances of breast metastases from extramammary malignancies as typical and atypical features, based on the results of US and other imaging studies performed at our institution. Awareness of the typical and atypical imaging features of these lesions may be helpful to diagnose metastatic lesions of the breast.

Breast Metastases from Extramammary Malignancies: Typical and Atypical Ultrasound Features

International Nuclear Information System (INIS)

Mun, Sung Hee; Ko, Eun Young; Han, Boo-Kyung; Shin, Jung Hee; Kim, Suk Jung; Cho, Eun Yoon

2014-01-01

Breast metastases from extramammary malignancies are uncommon. The most common sources are lymphomas/leukemias and melanomas. Some of the less common sources include carcinomas of the lung, ovary, and stomach, and infrequently, carcinoid tumors, hypernephromas, carcinomas of the liver, tonsil, pleura, pancreas, cervix, perineum, endometrium and bladder. Breast metastases from extramammary malignancies have both hematogenous and lymphatic routes. According to their routes, there are common radiological features of metastatic diseases of the breast, but the features are not specific for metastases. Typical ultrasound (US) features of hematogenous metastases include single or multiple, round to oval shaped, well-circumscribed hypoechoic masses without spiculations, calcifications, or architectural distortion; these masses are commonly located superficially in subcutaneous tissue or immediately adjacent to the breast parenchyma that is relatively rich in blood supply. Typical US features of lymphatic breast metastases include diffusely and heterogeneously increased echogenicities in subcutaneous fat and glandular tissue and a thick trabecular pattern with secondary skin thickening, lymphedema, and lymph node enlargement. However, lesions show variable US features in some cases, and differentiation of these lesions from primary breast cancer or from benign lesions is difficult. In this review, we demonstrate various US appearances of breast metastases from extramammary malignancies as typical and atypical features, based on the results of US and other imaging studies performed at our institution. Awareness of the typical and atypical imaging features of these lesions may be helpful to diagnose metastatic lesions of the breast

Occurrence of atypical myxomatosis in Central Europe: clinical and virological examinations.

Science.gov (United States)

Farsang, A; Makranszki, L; Dobos-Kovács, M; Virág, Györgyi; Fábián, Katalin; Barna, Tímea; Kulcsár, G; Kucsera, L; Vetési, F

2003-01-01

An outbreak of the atypical form of myxomatosis struck a rabbit farm in Hungary. The animals had previously been vaccinated with a vaccine containing Shope rabbit fibroma virus strain. The disease appeared in winter when the presence of mosquitoes and fleas is not common. The virus was isolated from an eyelid specimen of a naturally infected rabbit. The surviving animals were observed for four weeks, blood samples were collected and, after euthanasia, organ specimens were also examined by morphological methods including pathology and electron microscopy. Serum samples were examined by virus neutralisation for antibodies. Genetic analysis of the isolated virus was carried out by polymerase chain reaction (PCR) and direct sequencing. The primers were designed on the basis of the major envelope gene (Env) of the Lausanne reference strain in the GenBank. The viral proteins were examined by SDS-PAGE. The isolated virus (ref. no.: BP04/2001) was able to infect the susceptible animals directly, by contact. The disease was characterised by respiratory symptoms of the upper tracheal tract, conjunctivitis and high mortality by the 11th-14th day. Aerogenic infection with strain BP04/2001 resulted in 100% morbidity among the susceptible animals. Sequencing of the amplified 400-bp-long DNA revealed 97% homology with the Env gene of the Lausanne strain, which proves that strain BP04/2001 is a variant of the Lausanne strain having been enzootic throughout Europe. The live vaccine strain used in Hungary against myxomatosis, which is also a Lausanne-derived strain, protected the animals. According to the protein analysis a protein of 200 kDa in size is not expressed in strain BP04/2001. This is the first report on atypical myxomatosis in Central Europe. The virus spreads by airborne transmission and may cause severe losses in the rabbit population.

Medicolegal aspects of atypical firearm injuries: a case report.

Science.gov (United States)

Gürses, Murat Serdar; Akan, Okan; Eren, Bülent; Durak, Dilek; Türkmen, Nursel; Cetin, Selçuk

2014-01-01

Our case was a twenty year-old man, who was injured during the military duty with G3 infantry rifle in the training area. An atypical firearm entry wound on the left side of sternum which was 4.5 cm in diameter, and was surrounded by six irregular skin burn wounds by a flash-suppressor and a 0.7 cm diameter firearm exit wound at space on the left midscapular line. Our case emphasizes that the interpretation of properties of these atypical firearm entry wounds need to be carefully assessed by physicians.

Association of In Vivo [18F]AV-1451 Tau PET Imaging Results With Cortical Atrophy and Symptoms in Typical and Atypical Alzheimer Disease.

Science.gov (United States)

Xia, Chenjie; Makaretz, Sara J; Caso, Christina; McGinnis, Scott; Gomperts, Stephen N; Sepulcre, Jorge; Gomez-Isla, Teresa; Hyman, Bradley T; Schultz, Aaron; Vasdev, Neil; Johnson, Keith A; Dickerson, Bradford C

2017-04-01

Previous postmortem studies have long demonstrated that neurofibrillary tangles made of hyperphosphorylated tau proteins are closely associated with Alzheimer disease clinical phenotype and neurodegeneration pattern. Validating these associations in vivo will lead to new diagnostic tools for Alzheimer disease and better understanding of its neurobiology. To examine whether topographical distribution and severity of hyperphosphorylated tau pathologic findings measured by fluorine 18-labeled AV-1451 ([18F]AV-1451) positron emission tomographic (PET) imaging are linked with clinical phenotype and cortical atrophy in patients with Alzheimer disease. This observational case series, conducted from July 1, 2012, to July 30, 2015, in an outpatient referral center for patients with neurodegenerative diseases, included 6 patients: 3 with typical amnesic Alzheimer disease and 3 with atypical variants (posterior cortical atrophy, logopenic variant primary progressive aphasia, and corticobasal syndrome). Patients underwent [18F]AV-1451 PET imaging to measure tau burden, carbon 11-labeled Pittsburgh Compound B ([11C]PiB) PET imaging to measure amyloid burden, and structural magnetic resonance imaging to measure cortical thickness. Seventy-seven age-matched controls with normal cognitive function also underwent structural magnetic resonance imaging but not tau or amyloid PET imaging. Tau burden, amyloid burden, and cortical thickness. In all 6 patients (3 women and 3 men; mean age 61.8 years), the underlying clinical phenotype was associated with the regional distribution of the [18F]AV-1451 signal. Furthermore, within 68 cortical regions of interest measured from each patient, the magnitude of cortical atrophy was strongly correlated with the magnitude of [18F]AV-1451 binding (3 patients with amnesic Alzheimer disease, r = -0.82; P localizing and quantifying hyperphosphorylated tau in vivo, results of tau PET imaging will likely serve as a key biomarker that links a

Atypical mycobacterial infection mimicking carbuncle in an elderly patient: A case report

Directory of Open Access Journals (Sweden)

Terlinda Barros

2015-08-01

Full Text Available Background: Atypical mycobacterium infection occurs under certain skin conditions, namely the disruption of skin integrity and mucous membranes accompanied by the reduction of cellular immunity. However, atypical mycobacterial infection in elderly patients is rarely reported. Case: A 64 years old male patient, complained of red lumps on the upper-backfor a month, accompanied by mild fever and minimal pain. Three months before, the patient had accupuncture on the neck and upper back. Physical examination showed multiple miliar to lenticular sized papules and pustules on an erythematous-violaceus base with hard and immobile palpable nodes and infiltrate. After clinical and laboratory workup, the patient was diagnosed withcarbuncle with Candida spp colonization. The treatment consisted of systemicantibiotics and topical antifungals. There was no clinical improvement after 3 weeks. Histopathology and laboratory results suggested atypical mycobacterium infection. Discussion: Atypical mycobacterium infection should be considered in elderly patients with skin and soft tissue infections that show no clinical improvement tostandard therapy.

Atypical femoral fractures related to bisphosphonate therapy

Directory of Open Access Journals (Sweden)

Tarun Pankaj Jain

2012-01-01

Full Text Available Bisphosphonates (BP are a commonly prescribed class of drugs for the prevention of osteoporosis-related fractures. Paradoxically, however, they have recently been linked to atypical fractures in the shaft of the femur. Since many physicians including radiologists, are not aware of this entity, the incidence is likely underreported. These fractures usually occur in the sub-trochanteric region of the femur in the setting of low-energy trauma. It starts as a fracture line involving the lateral cortex and then progresses medially to give rise to a complete fracture. The fracture line is usually transverse, and there is a medial spike associated with a complete fracture. These fractures can be bilateral. Awareness of these atypical fractures and their radiological appearance should enable their early and accurate detection and thus lead to specific treatment.

[Psychotic forms of atypical autism in children].

Science.gov (United States)

Simashkova, N V

2006-01-01

The aim of the study was to determine clinical borders of psychotic forms of atypical autism in children, its psychopathological and age-specific manifestations as well as nosological peculiarities and to specify its pathogenetic features. Eighty patients with childhood endogenous autism, Rett syndrome, fragile X syndrome, Down syndrome have been studied during 14 years. The study showed that psychoses similar by symptoms and course, which are characterized by attacks and regressive-catatonic disorders, may develop in the course of atypical autism. These psychoses develop on the background of dysontogenesis with consequent replacement of the following stages: autistic, regressive, catatonic, with returning to the autistic stage between attacks. Psychopathological similarity of these psychoses in different disorders correlated with EEG changes of the same type (appearance of the marked I-rhythm at the regressive stage of psychosis).

Recurrent conjunctival atypical fibroxanthoma in Pigmentosum Xeroderma.

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Cerdà-Ibáñez, M; Barreiro-González, A; Barranco González, H; Aviñó Martínez, J; Évole-Buselli, M; Harto-Castaño, M Á

2018-02-01

A 7 year-old boy with Xeroderma Pigmentosum (XP) and who presents a recurrent conjunctival atypical fibroxanthoma after two surgeries. This is the third procedure and the patient is treated with a surgical excision of the tumour and cryotherapy at the surgical bed. Due to the risk of recurrence, topical Mitomycin C 0,02% was added at post-operative care achieving a good clinical outcome. Surgical exeresis with cryotherapy and topical Mitomycin C is an effective treatment for a case of an atypical fibroxanthoma with a high potential for recurrence and invasion. An ophthalmologic follow-up is required for these patients, as well as general paediatric care and support aids. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

An atypical case of Noonan syndrome with mutation diagnosed by targeted exome sequencing

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Jinsup Kim

2017-09-01

Full Text Available Noonan syndrome (NS is a genetic disorder caused by autosomal dominant inheritance and is characterized by a distinctive facial appearance, short stature, chest deformity, and congenital heart disease. In individuals with NS, germline mutations have been identified in several genes involved in the RAS/mitogen-activated protein kinase signal transduction pathway. Because of its clinical and genetic heterogeneity, the conventional diagnostic protocol with Sanger sequencing requires a multistep approach. Therefore, molecular genetic diagnosis using targeted exome sequencing (TES is considered a less expensive and faster method, particularly for patients who do not fulfill the clinical diagnostic criteria of NS. In this case, the patient showed short stature, dysmorphic facial features suggestive of NS, feeding intolerance, cryptorchidism, and intellectual disability in early childhood. At the age of 16, the patient still showed extreme short stature with delayed puberty and characteristic facial features suggestive of NS. Although the patient had no cardiac problems or chest wall deformities, which are commonly present in NS and are major concerns for patients and clinicians, the patient showed several other characteristic clinical features of NS. Considering the possibility of a genetic disorder, including NS, a molecular genetic study with TES was performed. With TES analysis, we detected a pathogenic variant of c.458A > T in KRAS in this patient with atypical NS phenotype and provided appropriate clinical management and genetic counseling. The application of TES enables accurate molecular diagnosis of patients with nonspecific or atypical features in genetic diseases with several responsible genes, such as NS.

Glutamatergic neurotransmission modulation and the mechanisms of antipsychotic atypicality.

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Heresco-Levy, Uriel

2003-10-01

The neurotransmission mediated by the excitatory amino acids (EAA) glutamate (GLU) and aspartate is of interest to the pharmacotherapy of psychosis due to its role in neurodevelopment and neurotoxicity, its complex interactions with dopaminergic and other neurotransmitter systems and its pivotal importance in recent models of schizophrenia. Accumulating evidence indicates that modulation of glutamatergic neurotransmission may play an important role in the mechanisms of action of atypical antipsychotic drugs. The principles of the phencyclidine (PCP) model of schizophrenia suggest that conventional neuroleptics cannot counteract all aspects of schizophrenia symptomatology, while a more favorable outcome, including anti-negative and cognitive symptoms effects, would be expected with the use of treatment modalities targeting glutamatergic neurotransmission. Clozapine and other presently used atypical antipsychotics differ from conventional neuroleptics in the way they affect various aspects of glutamatergic receptors function. In this context, a specific hypothesis suggesting an agonistic role of clozapine at the N-methyl-D-aspartate (NMDA) subtype of GLU receptors has been postulated. Furthermore, the results of the first generation of clinical trials with glycine (GLY) site agonists of the NMDA receptor in schizophrenia suggest that this type of compounds (1) have efficacy and side effects profiles different than those of conventional neuroleptics and (2) differ in their synergic effects when used in addition to conventional neuroleptics versus clozapine and possibly additional atypical antipsychotics. These findings (1) bring further support to the hypothesis that glutamatergic effects may play an important role in the mechanism of action of atypical antipsychotics, (2) help explain the unique clinical profile of clozapine, and (3) suggest that GLY site agonists of the NMDA receptor may represent a new class of atypical antipsychotic medication. Future research in

Extracorporeal photochemotherapy and methotrexate in the treatment of atypical oral lichen planus

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A. V. Molochkov

2017-01-01

Full Text Available Background: Some authors have successfully used methotrexate in the treatment of atypical oral lichen planus (LP and noted its good tolerability. High clinical efficacy of the extracorporeal photochemotherapy (ECP has been also reported in the treatment of such patients. However, there is no information on the long-term results of methotrexate and ECP and their combination in the treatment of atypical LP. Aim: To study clinical efficacy and long-term results of the combination of routine therapy with the ECP course and a single injection of methotrexate at a dose of 10 mg in patients with atypical LP of the oral cavity and the skin. Materials and methods: This was a prospective study with an active control. Eighteen (18 patients with various forms of atypical LP of the oral cavity (hypertrophic, erosive/ulcerative, exudative/hyperemic forms and the skin (hypertrophic, pigmented, atrophic, follicular forms were administered the combination of routine therapy (chloroquine, doxycycline, vitamin B6, topical corticosteroids, an ECP course, and a single injection of methotrexate at a dose of 10 mg. Two hours before the ECP session all patients were given 8-methoxypsoralen. Peripheral mononuclear cells were isolated with a cell separator and treated with ultraviolet radiation (λ = 320–400 nm, then the monocyte cell mass was re-infused to the patient. The treatment course included 4 sessions performed every other day. A single injection of methotrexate was given in the middle of the ECP course. Clinical efficacy was assessed with the Thongprasom scale of activity of the disease and by visual analog scale (VAS for pain assessment in patients with oral lesions. Results: The treatment was well tolerated and was not associated with methotrexate-related immune abnormalities. At one month after the 4th ECP session, the mean Thongprasom score was decreased from 5 to 2.2 ± 1.2 (p < 0.001. At Week 24 after the treatment, 15 (83.2% of

Relationships among Sensory Responsiveness, Anxiety, and Ritual Behaviors in Children with and without Atypical Sensory Responsiveness.

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Bart, Orit; Bar-Shalita, Tami; Mansour, Hanin; Dar, Reuven

2017-08-01

To explore relationships between sensory responsiveness, anxiety, and ritual behaviors in boys with typical and atypical sensory responsiveness. Forty-eight boys, ages 5-9 participated in the study (28 boys with atypical sensory responsiveness and 20 controls). Atypical sensory responsiveness was defined as a score of ≤154 on the Short Sensory Profile. Parents completed the Sensory Profile, the Screen for Child Anxiety Related Emotional Disorders, and the Childhood Routines Inventory. Children with atypical sensory responsiveness had significantly higher levels of anxiety and a higher frequency of ritual behaviors than controls. Atypical sensory responsiveness was significantly related to both anxiety and ritual behaviors, with anxiety mediating the relationship between sensory modulation and ritual behaviors. The findings elucidate the potential consequences of atypical sensory responsiveness and could support the notion that ritual behaviors develop as a coping mechanism in response to anxiety stemming from primary difficulty in modulating sensory input.

A phenotype of atypical apraxia of speech in a family carrying SQSTM1 mutation.

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Boutoleau-Bretonnière, Claire; Camuzat, Agnès; Le Ber, Isabelle; Bouya-Ahmed, Kawtar; Guerreiro, Rita; Deruet, Anne-Laure; Evrard, Christelle; Bras, José; Lamy, Estelle; Auffray-Calvier, Elisabeth; Pallardy, Amandine; Hardy, John; Brice, Alexis; Derkinderen, Pascal; Vercelletto, Martine

2015-01-01

SQSTM1 mutations, coding for the p62 protein, were identified as a monogenic cause of Paget disease of bone and of amyotrophic lateral sclerosis. More recently, SQSTM1 mutations were identified in few families with frontotemporal dementia. We report a new family carrying SQSTM1 mutation and presenting with a clinical phenotype of speech apraxia or atypical behavioral disorders, associated with early visuo-contructional deficits. This study further supports the implication of SQSTM1 in frontotemporal dementia, and enlarges the phenotypic spectrum associated with SQSTM1 mutations.

Analysis of the Origin of Atypical Scanning Laser Polarimetry Patterns by Polarization-Sensitive Optical Coherence Tomography

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Götzinger, Erich; Pircher, Michael; Baumann, Bernhard; Hirn, Cornelia; Vass, Clemens; Hitzenberger, Christoph K.

2010-01-01

Purpose To analyze the physical origin of atypical scanning laser polarimetry (SLP) patterns. To compare polarization-sensitive optical coherence tomography (PS-OCT) scans to SLP images. To present a method to obtain pseudo-SLP images by PS-OCT that are free of atypical artifacts. Methods Forty-one eyes of healthy subjects, subjects with suspected glaucoma, and patients with glaucoma were imaged by SLP (GDx VCC) and a prototype spectral domain PS-OCT system. The PS-OCT system acquires three-dimensional (3D) datasets of intensity, retardation, and optic axis orientation simultaneously within 3 seconds. B-scans of intensity and retardation and en face maps of retinal nerve fiber layer (RNFL) retardation were derived from the 3D PS-OCT datasets. Results were compared with those obtained by SLP. Results Twenty-two eyes showed atypical retardation patterns, and 19 eyes showed normal patterns. From the 22 atypical eyes, 15 showed atypical patterns in both imaging modalities, five were atypical only in SLP images, and two were atypical only in PS-OCT images. In most (15 of 22) atypical cases, an increased penetration of the probing beam into the birefringent sclera was identified as the source of atypical patterns. In such cases, the artifacts could be eliminated in PS-OCT images by depth segmentation and exclusion of scleral signals. Conclusions PS-OCT provides deeper insight into the contribution of different fundus layers to SLP images. Increased light penetration into the sclera can distort SLP retardation patterns of the RNFL. PMID:19036999

Imaging biomarkers in Parkinson?s disease and Parkinsonian syndromes: current and emerging concepts

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Saeed, Usman; Compagnone, Jordana; Aviv, Richard I.; Strafella, Antonio P.; Black, Sandra E.; Lang, Anthony E.; Masellis, Mario

2017-01-01

Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson?s disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson?s disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous d...

An Exploration of Eye Movements when Reading Texts with Atypical Spatial Layouts

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F Hermens

2012-07-01

Full Text Available In two experiments we explored eye movements that people make when looking at texts with an atypical spatial layout. In the first experiment, participants repeatedly viewed the same piece of text, containing sections in different languages and printed using different fonts and reading directions, while they answered different questions about what they saw. In agreement with earlier studies showing significant differences in gaze patterns across different tasks, we found that reading was strongly influenced by the questions that participants tried to answer. In the second experiment, we examined gaze patterns for different types of poems while participants performed a single task: To rate their appreciation of the poem. Poems were either in the typical form, with short lines all aligned to the left, or they were in an atypical form (“graphical poem”, with the text dispersed across the page, in different fonts, reading directions, and size. Half of the poems were from an artist known for his graphical poems, the other half were from different authors who posted their poems on a webpage. All these poems were presented in a typical and an atypical form. Participants rated the poems from the web more highly, but took less time to inspect them. Whether the poem was in a typical or an atypical layout did not influence the ratings, even though longer inspection times were found for the atypical forms. Eye movement parameters, such as fixation duration and saccade amplitude, were relatively unaffected by the layout of the poems, although some additional larger amplitude saccades could be observed for the atypical forms.

Protein trans-splicing of multiple atypical split inteins engineered from natural inteins.

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Ying Lin

Full Text Available Protein trans-splicing by split inteins has many uses in protein production and research. Splicing proteins with synthetic peptides, which employs atypical split inteins, is particularly useful for site-specific protein modifications and labeling, because the synthetic peptide can be made to contain a variety of unnatural amino acids and chemical modifications. For this purpose, atypical split inteins need to be engineered to have a small N-intein or C-intein fragment that can be more easily included in a synthetic peptide that also contains a small extein to be trans-spliced onto target proteins. Here we have successfully engineered multiple atypical split inteins capable of protein trans-splicing, by modifying and testing more than a dozen natural inteins. These included both S1 split inteins having a very small (11-12 aa N-intein fragment and S11 split inteins having a very small (6 aa C-intein fragment. Four of the new S1 and S11 split inteins showed high efficiencies (85-100% of protein trans-splicing both in E. coli cells and in vitro. Under in vitro conditions, they exhibited reaction rate constants ranging from ~1.7 × 10(-4 s(-1 to ~3.8 × 10(-4 s(-1, which are comparable to or higher than those of previously reported atypical split inteins. These findings should facilitate a more general use of trans-splicing between proteins and synthetic peptides, by expanding the availability of different atypical split inteins. They also have implications on understanding the structure-function relationship of atypical split inteins, particularly in terms of intein fragment complementation.

Screening frequency and atypical cells and the prediction of cervical cancer risk.

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Chen, Yun-Yuan; You, San-Lin; Koong, Shin-Lan; Liu, Jessica; Chen, Chi-An; Chen, Chien-Jen

2014-05-01

To evaluate the screening efficacy and importance of atypical squamous cells and atypical glandular cells in predicting subsequent cervical cancer risk. This national cohort study in Taiwan analyzed associations between Pap test screening frequency and findings in 1995-2000 and subsequent risk of squamous cell carcinoma and adenocarcinoma after 2002. Women aged 30 years or older in 1995 without a cervical cancer history were included. Multivariate-adjusted hazard ratios and their 95% confidence intervals (CIs) were assessed using Cox regression analysis. During a total follow-up of 31,693,980 person-years in 2002-2008, 9,471 squamous cell carcinoma and 1,455 adenocarcinoma cases were newly diagnosed, resulting in 2,067 deaths. The risk of developing and dying from squamous cell carcinoma decreased significantly with increasing attendance frequency between 1995 and 2000 (all P values for trend1995-2000 had 0.69-fold and 0.35-fold decrease in incidence and mortality of adenocarcinoma, respectively, compared with women who never attended any screenings. Abnormal cytologic findings were significant predictors of the incidence and mortality of cervical cancers. The adjusted hazard ratio (95% CI) of developing squamous cell carcinoma was 29.94 (22.83-39.25) for atypical squamous cells, cannot exclude high-grade squamous intraepithelial lesions, and the adjusted hazard ratio (95% CI) of developing adenocarcinoma was 49.43 (36.49-66.97) for atypical glandular cells. Significant reductions in cervical adenocarcinoma occurred in women who attend three or more annual screenings in 6 years. High-grade atypical squamous cells and atypical glandular cells are important predictors of subsequent adenocarcinoma and squamous cell carcinoma. II.

Familial Interstitial Pulmonary Fibrosis: A Large Family with Atypical Clinical Features

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Ranji Chibbar

2010-01-01

Full Text Available A large kindred of familial pulmonary fibrosis is reported. Six members from the first two generations of this particular kindred were described more than 40 years previously; six more individuals from the third and fourth generations have also been evaluated. The proband, now 23 years of age, has mild disease; the other 11 documented affected family members all died from their disease at an average age of 37 years (range 25 to 50 years. The pathology was that of usual interstitial pneumonia, as is typical in idiopathic pulmonary fibrosis. However, the initial radiographic pattern in many of these individuals was upper lobe and nodular and, along with the young age, was atypical for idiopathic pulmonary fibrosis. Several genetic abnormalities have been associated with familial pulmonary fibrosis. The present study examined the genes coding for surfactant protein-C, ATP-binding cassette protein A3 and telomerase, and found no abnormalities.

Atypical language representation in children with intractable temporal lobe epilepsy.

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Maulisova, Alice; Korman, Brandon; Rey, Gustavo; Bernal, Byron; Duchowny, Michael; Niederlova, Marketa; Krsek, Pavel; Novak, Vilem

2016-05-01

This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity. Copyright © 2016 Elsevier Inc. All rights reserved.

Critical differences between subtrochanteric and diaphyseal atypical femoral fractures: analyses of 51 cases at a single institution in Korean population.

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Cho, Yoon-Je; Kang, Kyung-Chung; Chun, Young-Soo; Rhyu, Kee Hyung; Kim, Sang-Jun; Jang, Tae-Su

2018-05-03

There still remains controversy on the pathomechanism of atypical femoral fracture (AFF). The angle of lateral bowing and bone mineral density showed significant differences between subtrochanteric and diaphyseal atypical fracture groups. In addition to the use of bisphosphonate, mechanical factors might play important roles in the occurrence of AFFs. Although AFF could be divided into subtrochanteric and diaphyseal fracture according to the location of fractures, there is a lack of evidence regarding differences between two fractures and etiology of the occurrence. The aim of study is to determine differences between atypical subtrochanteric and diaphyseal fracture in Korean population. Between February 2010 and March 2015, 51 AFFs in 40 patients were included in this study. Their medical records were retrospectively reviewed. The AFF patients satisfied all the diagnostic criteria of the 2014 revised edition of the ASMBR. To analyze the differences according to the location of fracture, the AFFs were divided into subtrochanteric (n = 16) and diaphyseal (n = 35) fracture groups. The following factors were compared between two groups: patients' demographics, underlying diseases, laboratory findings (serum-25(OH) VitD3, osteocalcin, c-telopeptide, ALP, Ca, and P), bone mineral density (BMD), duration of bisphosphonate (BP) usage, and lateral bowing of the femur at time of the fracture. All AFFs happened in female patients (mean age, 73.8 years) who have received bisphosphonate treatments except three patients. The mean duration of bisphosphonate usage was 95.3 months. Between the two groups, demographic data (age, height, weight, and BMI), underlying diseases, laboratory findings, hip BMD, and duration of BP treatment were comparable to each other (p > 0.05). However, the subtrochanteric fracture group showed higher FNSBA (femoral neck shaft bowing angle, p group. Angle of lateral bowing (FNSBA) and spine BMD showed significant differences between

Unique Neural Characteristics of Atypical Lateralization of Language in Healthy Individuals

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Szymon P. Biduła

2017-09-01

Full Text Available Using functional magnetic resonance imaging (fMRI in 63 healthy participants, including left-handed and ambidextrous individuals, we tested how atypical lateralization of language—i. e., bilateral or right hemispheric language representation—differs from the typical left-hemisphere dominance. Although regardless of their handedness, all 11 participants from the atypical group engaged classical language centers, i.e., Broca's and Wernicke's areas, the right-hemisphere components of the default mode network (DMN, including the angular gyrus and middle temporal gyrus, were also critically involved during the verbal fluency task. Importantly, activity in these regions could not be explained in terms of mirroring the typical language pattern because left-hemisphere dominant individuals did not exhibit similar significant signal modulations. Moreover, when spatial extent of language-related activity across whole brain was considered, the bilateral language organization entailed more diffuse functional processing. Finally, we detected significant differences between the typical and atypical group in the resting-state connectivity at the global and local level. These findings suggest that the atypical lateralization of language has unique features, and is not a simple mirror image of the typical left hemispheric language representation.

An Atypical Presentation on Insulinoma

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2017-06-16

PUBLICATIONS/ PRESENTATIONS 1. TO: CLINICAL RESEARCH 2. FROM: (Author’s Name, Rank, Grade, Office Symbol) 3. GME/GHSE STUDENT: 4. PROTOCOL NUMBER: Kluesner...PROCESSING OF PROFESSIONAL MEDICAL RESEARCH/TECHNICAL PUBLICATIONS/ PRESENTATIONS 1st ENDORSEMENT (59 MDW/SGVU Use Only) TO: Clinical Research Division 24...CAPT JOSEPH KLUESNER FROM: 59 MDW/SGYU SUBJECT: Professional Presentation Approval 1. Your paper, entitled An Atypical Presentation of Insulinoma

Atypical fibroxanthoma-like amelanotic malignant melanoma: A case report and literature review

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Chao-Kai Hsu

2013-09-01

Full Text Available Atypical fibroxanthoma (AFX-like malignant melanoma is very rare. Here, we report a case of amelanotic AFX-like melanoma in a 72-year-old Taiwanese woman presenting with two separate, asymptomatic, enlarging erythematous nodules within a large hypopigmented patch on her left cheek. Histologically, both lesions showed cellular nodules in the reticular dermis separated from the overlying flattened epidermis by a zone of solar elastosis or fibrosis. The tumor consisted of sheets of atypical epithelioid cells arranged in a vague nesting pattern, as well as many atypical large or gigantic cells with one or more, large hyperchromatic, vesicular, or pleomorphic nuclei with prominent nucleoli, and moderate-to-abundant eosinophilic or foamy cytoplasm. Focal intraepidermal proliferation of atypical melanocytes with a pagetoid pattern was found only in the periphery of the main tumor. The tumor cells were moderately to strongly positive for S-100, Melan-A, and HMB-45. The pleomorphic giant cells were focally CD68-positive but CD163-negative. The patient underwent tumor excision followed by radiotherapy due to the narrow surgical margins. A sentinel lymph node biopsy revealed no metastasis of the melanoma. This case illustrates the importance of scrutinizing any subtle proliferation of atypical melanocytes in the epidermis in an AFX-like tumor in order to avoid misdiagnosis.

A child with myoclonus-dystonia (DYT11) misdiagnosed as atypical opsoclonus myoclonus syndrome

DEFF Research Database (Denmark)

Drivenes, Bergitte; Born, Alfred Peter; Ek, Jakob

2015-01-01

INTRODUCTION: DYT11 is an autosomal dominant inherited movement disorder characterized by myoclonus and dystonia. CLINICAL PRESENTATION: We present a case with atypical symptoms and with episodes of ataxia and myoclonus preceded by infections. Atypical presentation of opsoclonus myoclonus syndrom...

Natural infection of cattle with an atypical 'HoBi'-like pestivirus--implications for BVD control and for the safety of biological products.

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Ståhl, Karl; Kampa, Jaruwan; Alenius, Stefan; Persson Wadman, Annie; Baule, Claudia; Aiumlamai, Suneerat; Belák, Sándor

2007-01-01

During a study on Bovine Viral Diarrhoea (BVD) epidemiology in Thailand, a pestivirus was detected in serum from a calf. Comparative nucleotide sequence analysis showed that this virus was closely related to a recently described atypical pestivirus (D32/00_'HoBi') that was first isolated from a batch of foetal calf serum collected in Brazil. The results from virus neutralisation tests performed on sera collected from cattle in the herd of the infected calf, showed that these cattle had markedly higher antibody titres against the atypical pestivirus 'HoBi' than against Bovine Viral Diarrhoea Virus types 1 and 2, or Border Disease Virus. The results also supported, consequently, the results from the molecular analysis, and demonstrated that a 'HoBi'-like pestivirus had been introduced to, and was now circulating in the herd. This study is the first to report a natural infection in cattle with a virus related to this atypical pestivirus, and it suggests that this group of pestiviruses may already be spread in cattle populations. The findings have implications for BVD control and for the biosafety of vaccines and other biological products produced with foetal calf serum. Consequently, these atypical pestiviruses should be included in serological assays, and any diagnostic assay aimed at detection of pestiviruses in biological products or animals should be tested for its ability to detect them.

Primary atypical sacral meningioma- not always benign

International Nuclear Information System (INIS)

Bhadra, A.K.; Casey, A.T.H.; Saifuddin, A.; Briggs, T.W.

2007-01-01

We present a case of an atypical recurrent meningioma of the sacrum with pulmonary metastasis in a 31-year-old man. He presented with deep-seated buttock pain and urinary hesitancy for 3 months. MRI revealed a lesion occupying the central and left side of the sacral canal at the S1-S2 level. Surgical excision of the lesion via a posterior approach was undertaken, and the patient became symptom-free post-operatively. Histology confirmed atypical meningioma. Eight months later he re-presented with similar symptoms, and MRI confirmed local recurrence. The patient underwent left hemisacrectomy. Six months later he again presented with low back pain and MRI confirmed a second local recurrence. A CT scan of the chest showed multiple lung metastases. The patient died of a severe chest infection 18 months later. (orig.)

Primary atypical sacral meningioma- not always benign

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Bhadra, A.K.; Casey, A.T.H.; Saifuddin, A.; Briggs, T.W. [Royal National Orthopaedic Hospital, Stanmore, London (United Kingdom)

2007-06-15

We present a case of an atypical recurrent meningioma of the sacrum with pulmonary metastasis in a 31-year-old man. He presented with deep-seated buttock pain and urinary hesitancy for 3 months. MRI revealed a lesion occupying the central and left side of the sacral canal at the S1-S2 level. Surgical excision of the lesion via a posterior approach was undertaken, and the patient became symptom-free post-operatively. Histology confirmed atypical meningioma. Eight months later he re-presented with similar symptoms, and MRI confirmed local recurrence. The patient underwent left hemisacrectomy. Six months later he again presented with low back pain and MRI confirmed a second local recurrence. A CT scan of the chest showed multiple lung metastases. The patient died of a severe chest infection 18 months later. (orig.)

Atypical manifestations of reversible posterior leukoencephalopathy syndrome: findings on diffusion imaging and ADC mapping

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Ahn, K.J.; You, W.J.; Jeong, S.L.; Lee, J.W.; Kim, B.S.; Lee, J.H.; Hahn, S.T. [Dept. of Radiology, The Catholic Univ. of Korea, St. Mary' s Hospital, Seoul (Korea); Yang, D.W.; Son, Y.M. [Dept. of Neurology, The Catholic Univ. of Korea, St. Mary' s Hospital, Seoul (Korea)

2004-12-01

Typically, reversible posterior leukoencephalopathy syndrome (RPLS) involves the parieto-occipital lobes. When regions of the brain other than the parieto-occipital lobes are predominantly involved, the syndrome can be called atypical RPLS. The purpose of this study is to find radiological and pathophysiological features of atypical RPLS by using diffusion-weighted imaging (D-WI). We retrospectively reviewed seven patients (two with eclampsia, one with cyclosporine neurotoxicity, and four with hypertensive encephalopathy) with atypical MR manifestations of RPLS. Changes in signal intensity on T2-weighted imaging (T2-WI) and D-WI, and ADC ratio, were analyzed. In patients with atypical manifestation of RPLS, high signal intensities on T2-WI were noted in the frontal lobe, basal ganglia, thalamus, brainstem, and subcortical white matter in regions other than the parieto-occipital lobes. These areas of increased signal intensities on T2-WI showed increased ADC values, representing vasogenic edema in all seven patients. This result should be very useful in differentiating atypical RPLS from other metabolic brain disorders that affect the same sites with cytotoxic edema. (orig.)

Atypical manifestations of reversible posterior leukoencephalopathy syndrome: findings on diffusion imaging and ADC mapping

International Nuclear Information System (INIS)

Ahn, K.J.; You, W.J.; Jeong, S.L.; Lee, J.W.; Kim, B.S.; Lee, J.H.; Hahn, S.T.; Yang, D.W.; Son, Y.M.

2004-01-01

Typically, reversible posterior leukoencephalopathy syndrome (RPLS) involves the parieto-occipital lobes. When regions of the brain other than the parieto-occipital lobes are predominantly involved, the syndrome can be called atypical RPLS. The purpose of this study is to find radiological and pathophysiological features of atypical RPLS by using diffusion-weighted imaging (D-WI). We retrospectively reviewed seven patients (two with eclampsia, one with cyclosporine neurotoxicity, and four with hypertensive encephalopathy) with atypical MR manifestations of RPLS. Changes in signal intensity on T2-weighted imaging (T2-WI) and D-WI, and ADC ratio, were analyzed. In patients with atypical manifestation of RPLS, high signal intensities on T2-WI were noted in the frontal lobe, basal ganglia, thalamus, brainstem, and subcortical white matter in regions other than the parieto-occipital lobes. These areas of increased signal intensities on T2-WI showed increased ADC values, representing vasogenic edema in all seven patients. This result should be very useful in differentiating atypical RPLS from other metabolic brain disorders that affect the same sites with cytotoxic edema. (orig.)

Unusual imaging characteristics of complicated hydatid disease

International Nuclear Information System (INIS)

Turgut, Ahmet Tuncay; Altin, Levent; Topcu, Salih; Kilicoglu, Buelent; Altinok, Tamer; Kaptanoglu, Erkan; Karademir, Alp; Kosar, Ugur

2007-01-01

Hydatid disease, a worldwide zoonosis, is caused by the larval stage of the Echinococcus tapeworm. Although the liver and the lungs are the most frequently involved organs in the body, hydatid cysts of other organs are unusual. Radiologically, they usually demonstrate typical imaging findings, but unusual imaging characteristics of complicated cyst of hydatid disease, associated with high morbidity and mortality, are rarely described in the literature. The purpose of this study is to review the general features of hydatidosis and to discuss atypical imaging characteristics of the complicated hydatid disease in the human, with an emphasis on structure and rupture of the cystic lesion as well as ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI) features of the disease. In our study, the available literature and images of the cases with complicated hydatidosis involving liver, lung, brain, spine and orbit were reviewed retrospectively. In hydatid disease, there are many potential local and systemic complications due to secondary involvement in almost any anatomic location in humans. Radiologically, in addition to the presence of atypical findings such as perifocal edema, non-homogenous contrast enhancement, multiplicity or septations and calcification, various unusual manifestations due to rupture or infection of the cyst have been observed in our cases with complicated hydatid disease. To prevent subsequent acute catastrophic results and the development of recurrences in various organs, it should be kept in mind that complicated hydatid cysts can cause unusual USG, CT, and MRI findings, in addition to typical ones, in endemic areas. Therefore, familiarity with atypical radiological appearances of complicated hydatid disease may be valuable in making a correct diagnosis and treatment

Unusual imaging characteristics of complicated hydatid disease

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Turgut, Ahmet Tuncay [Department of Radiology, Ankara Training and Research Hospital, Ankara (Turkey)]. E-mail: ahmettuncayturgut@yahoo.com; Altin, Levent [Department of Radiology, Numune Training and Research Hospital, Ankara (Turkey); Topcu, Salih [Department of Thoracic Surgery, Faculty of Medicine, Kocaeli University, Izmit (Turkey); Kilicoglu, Buelent [Department of 4th General Surgery, Ankara Training and Research Hospital, Ankara (Turkey); Altinok, Tamer [Department of Thoracic Surgery, Meram Faculty of Medicine, Selcuk University, Konya (Turkey); Kaptanoglu, Erkan [Department of Neurosurgery, Numune Training and Research Hospital, Ankara (Turkey); Karademir, Alp [Department of Radiology, Numune Training and Research Hospital, Ankara (Turkey); Kosar, Ugur [Department of Radiology, Ankara Training and Research Hospital, Ankara (Turkey)

2007-07-15

Hydatid disease, a worldwide zoonosis, is caused by the larval stage of the Echinococcus tapeworm. Although the liver and the lungs are the most frequently involved organs in the body, hydatid cysts of other organs are unusual. Radiologically, they usually demonstrate typical imaging findings, but unusual imaging characteristics of complicated cyst of hydatid disease, associated with high morbidity and mortality, are rarely described in the literature. The purpose of this study is to review the general features of hydatidosis and to discuss atypical imaging characteristics of the complicated hydatid disease in the human, with an emphasis on structure and rupture of the cystic lesion as well as ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI) features of the disease. In our study, the available literature and images of the cases with complicated hydatidosis involving liver, lung, brain, spine and orbit were reviewed retrospectively. In hydatid disease, there are many potential local and systemic complications due to secondary involvement in almost any anatomic location in humans. Radiologically, in addition to the presence of atypical findings such as perifocal edema, non-homogenous contrast enhancement, multiplicity or septations and calcification, various unusual manifestations due to rupture or infection of the cyst have been observed in our cases with complicated hydatid disease. To prevent subsequent acute catastrophic results and the development of recurrences in various organs, it should be kept in mind that complicated hydatid cysts can cause unusual USG, CT, and MRI findings, in addition to typical ones, in endemic areas. Therefore, familiarity with atypical radiological appearances of complicated hydatid disease may be valuable in making a correct diagnosis and treatment.

Dysfunctional metacognition and drive for thinness in typical and atypical anorexia nervosa.

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Davenport, Emily; Rushford, Nola; Soon, Siew; McDermott, Cressida

2015-01-01

Anorexia nervosa is complex and difficult to treat. In cognitive therapies the focus has been on cognitive content rather than process. Process-oriented therapies may modify the higher level cognitive processes of metacognition, reported as dysfunctional in adult anorexia nervosa. Their association with clinical features of anorexia nervosa, however, is unclear. With reclassification of anorexia nervosa by DSM-5 into typical and atypical groups, comparability of metacognition and drive for thinness across groups and relationships within groups is also unclear. Main objectives were to determine whether metacognitive factors differ across typical and atypical anorexia nervosa and a non-clinical community sample, and to explore a process model by determining whether drive for thinness is concurrently predicted by metacognitive factors. Women receiving treatment for anorexia nervosa (n = 119) and non-clinical community participants (n = 100), aged between 18 and 46 years, completed the Eating Disorders Inventory (3(rd) Edition) and Metacognitions Questionnaire (Brief Version). Body Mass Index (BMI) of 18.5 kg/m(2) differentiated between typical (n = 75) and atypical (n = 44) anorexia nervosa. Multivariate analyses of variance and regression analyses were conducted. Metacognitive profiles were similar in both typical and atypical anorexia nervosa and confirmed as more dysfunctional than in the non-clinical group. Drive for thinness was concurrently predicted in the typical patients by the metacognitive factors, positive beliefs about worry, and need to control thoughts; in the atypical patients by negative beliefs about worry and, inversely, by cognitive self-consciousness, and in the non-clinical group by cognitive self-consciousness. Despite having a healthier weight, the atypical group was as severely affected by dysfunctional metacognitions and drive for thinness as the typical group. Because metacognition concurrently predicted drive for thinness

Drug information update. Atypical antipsychotics and neuroleptic malignant syndrome: nuances and pragmatics of the association.

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Sarkar, Siddharth; Gupta, Nitin

2017-08-01

Neuroleptic malignant syndrome (NMS) is a rare but potentially fatal adverse event associated with the use of antipsychotics. Although atypical antipsychotics were initially considered to carry no risk of NMS, reports have accumulated over time implicating them in NMS causation. Almost all atypical antipsychotics have been reported to be associated with NMS. The clinical profile of NMS caused by certain atypical antipsychotics such as clozapine has been reported to be considerably different from the NMS produced by typical antipsychotics, with diaphoresis encountered more commonly, and rigidity and tremor encountered less frequently. This article briefly discusses the evidence relating to the occurrence, presentation and management of NMS induced by atypical antipsychotics.

Diabetic muscle infarction: atypical MR appearance

International Nuclear Information System (INIS)

Sharma, P.; Mangwana, S.; Kapoor, R.K.

2000-01-01

We describe a case of diabetic muscle infarction which had atypical features of hyperintensity of the affected muscle on T1-weighted images. Biopsy was performed which revealed diffuse extensive hemorrhage within the infarcted muscle. We believe increased signal intensity on T1-weighted images should suggest hemorrhage within the infarcted muscle. (orig.)

Deleterious sucking habits and atypical swallowing in children with otitis media with effusion.

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Ralli, Giovanni; Ruoppolo, Giovanni; Mora, Renzo; Guastini, Luca

2011-10-01

The aim of this study was to investigate the possible correlation between otitis media with effusion, bad sucking habits and atypical swallowing in children affected by otitis media with effusion. 65 children, aged from 7 to 12 years, observed in the ENT Department of the "La Sapienza" University of Rome, were enrolled in the study group (group A). All children were affected by otitis media with effusion for more than 3 months. As control group, 60 healthy children, aged from 7 to 12 years were identified (group B). All the children underwent medical history, with evaluation of the sucking habits, ENT examination, tympanometry, orthodontic examination and evaluation of swallowing. In the orthodontic examination the variables analyzed were: maximum mouth opening, right and left mandibular lateral movements and mandibular protrusion. Atypical swallowing was considered to occur when lip activity produced strong tension in the perioral musculature, and/or the tip of the tongue was placed or pushed against the anterior teeth during swallowing. In the group A, atypical swallowing was found in 33/65 subjects out of the 65 children (50.7%). In the control group (group B) 16/60 children (26.6%) showed atypical swallowing. Compared with group B, deleterious sucking habits were significantly higher (phabits were present in almost all children with atypical swallowing (28/33 in group A and 12/16 in group B). Our data suggest a correlation between otitis media with effusion, deleterious sucking habits and prevalence of atypical swallowing. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Gain of chromosome arm 1q in atypical meningioma correlates with shorter progression-free survival.

LENUS (Irish Health Repository)

2012-02-01

Aims: Atypical (WHO grade II) meningiomas have moderately high recurrence rates; even for completely resected tumours, approximately one-third will recur. Post-operative radiotherapy (RT) may aid local control and improve survival, but carries the risk of side effects. More accurate prediction of recurrence risk is therefore needed for patients with atypical meningioma. Previously, we used high-resolution array CGH to identify genetic variations in 47 primary atypical meningiomas and found that approximately 60% of tumors show gain of 1q at 1q25.1 and 1q25.3 to 1q32.1 and that 1q gain appeared to correlate with shorter progression-free survival. This study aimed to validate and extend these findings in an independent sample. Methods: 86 completely resected atypical meningiomas (with 25 recurrences) from two neurosurgical centres in Ireland were identified and clinical follow up was obtained. Utilizing a dual-colour interphase FISH assay, 1q gain was assessed using BAC probes directed against 1q25.1 and 1q32.1. Results: The results confirm the high prevalence of 1q gain at these loci in atypical meningiomas. We further show that gain at 1q32.1 and age each correlate with progression-free survival in patients who have undergone complete surgical resection of atypical meningiomas. Conclusions: These independent findings suggest that assessment of 1q copy number status can add clinically useful information for the management of patients with atypical meningiomas.

A diagnostic algorithm for atypical spitzoid tumors: guidelines for immunohistochemical and molecular assessment.

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Cho-Vega, Jeong Hee

2016-07-01

Atypical spitzoid tumors are a morphologically diverse group of rare melanocytic lesions most frequently seen in children and young adults. As atypical spitzoid tumors bear striking resemblance to Spitz nevus and spitzoid melanomas clinically and histopathologically, it is crucial to determine its malignant potential and predict its clinical behavior. To date, many researchers have attempted to differentiate atypical spitzoid tumors from unequivocal melanomas based on morphological, immonohistochemical, and molecular diagnostic differences. A diagnostic algorithm is proposed here to assess the malignant potential of atypical spitzoid tumors by using a combination of immunohistochemical and cytogenetic/molecular tests. Together with classical morphological evaluation, this algorithm includes a set of immunohistochemistry assays (p16(Ink4a), a dual-color Ki67/MART-1, and HMB45), fluorescence in situ hybridization (FISH) with five probes (6p25, 8q24, 11q13, CEN9, and 9p21), and an array-based comparative genomic hybridization. This review discusses details of the algorithm, the rationale of each test used in the algorithm, and utility of this algorithm in routine dermatopathology practice. This algorithmic approach will provide a comprehensive diagnostic tool that complements conventional histological criteria and will significantly contribute to improve the diagnosis and prediction of the clinical behavior of atypical spitzoid tumors.

Atypical presentation of a middle age male with severe hypertriglyceridaemia: a case report

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Albahrani Ali I

2007-07-01

Full Text Available Abstract Background Severe hypertriglyceridaemia (HTG is uncommon but most prevalent in subjects with type 2 diabetes mellitus (T2DM and excess ethanol intake. Case presentation We describe a case of a middle age male (53 y presenting to the emergency room with acute atypical central chest pain and severe HTG in the absence of evidence of overt ischaemic heart disease (IHD. Admission ECG and EET (exercise tolerance test were negative for reversible ischaemic changes. His admission glucose was 12.2 mmol/l, triglycerides (TG were 103 mmol/l, total cholesterol 37 mmol/l. Cardiac Troponin T could not be measured on three occasions but CK MB mass was normal at 3 μg/l. The patient was started on Bezafibrate 400 mg OD, Simvastatin 20 mg nocte, Omacor (Omega-3 fish oil 1 gm bd and Metformin 500 mg tds. Four weeks after admission, lipid and liver profiles showed remarkable improvement, TG 2.9 mmol/l, Tchol 6.3 mmol/l and HDLc 1.5 mmol/l, ALAT and GGT were normal. Conclusion A case report of severe hypertriglyceridaemia with atypical presentation demonstrate the role of combined lipid modifying agents in lowering triglycerides and cholesterol as well as improving liver enzymes.

The Seropositivity Rate of Atypical Agents in Patients with Community-Acquired Pneumonia

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Ruhan Karakoc Gunes

2007-08-01

Full Text Available The aim of this study was to investigate the IgM antibody positivities of atypical pneumonia agents in patients with community-acquired pneumonia (CAP, and to compare the results with controls. The serum samples which were collected from 87 adult patients and 21 healthy controls have been investigated by a commercial ELISA (Pneumobact ELISA IgM, Vircell, Spain in which four different atypical pneumonia agents were fixed onto a slide. In the patients group, IgM positivity rates for the agents were as follows, respectively; 2.3% for Legionella pneumophila, 56.3% Chlamydia pneumoniae, 33.3% for Mycoplasma pneumoniae, 9.2% for Coxiella burnetii. The rates of IgM positivities in the control group varied 7% for all of the agents except M. Pneumoniae and C. Pneumoniae and 2 of these controls were positive for L. Pneumophila IgM, one was positive for C. Burnetii IgM. According to the statistical evaluation, there were significant differences for IgM seropositivities to Mycoplasma pneumoniae and Chlamydia Pneumoniae,between the patient and the control groups (p0.05. We showed that the seropositivity rate of atypical agents in patients with CAP was significantly higher when compared to healthy control group. This result suggests us, atypical agents might be responsible in CAP patients in a great amount. Furthermore, our study also suggests that clinical and radiological findings are not useful for discriminating atypical from typical pneumonia. [TAF Prev Med Bull 2007; 6(4.000: 279-284

Atypical fractures on long term bisphosphonates therapy.

LENUS (Irish Health Repository)

Hussein, W

2011-01-01

Bisphosphonates reduce fractures risk in patients with osteoporosis. A new pattern of fractures is now being noted in patients on prolonged bisphosphonate therapy. We report a case of an atypical femoral fracture with preceding pain and highlight the characteristics of these fractures.

Atypical Eating Attitudes and Behaviors in Thai Medical Students

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Jarurin Pitanupong

2017-01-01

Full Text Available Objective: To determine the prevalence, and associated factors of atypical eating attitudes and behaviors in Thai medical students. Methods: A cross-sectional survey examined the eating abnormalities in Thai medical students, conducted in 2014. Research assistants collected data by using; self-reported questionnaires using The Eating Attitudes Test-26 (EAT-26 Thai Version. The statistical analysis used R-program for qualitative variables and logistic regression was applied to determine the correlation and P-value. Results: 141 Thai, medical students (15.9% were reported to have atypical attitudes towards eating, and displayed abnormal eating behaviors. There was no statistically significant correlation of attitude towards eating, and their current eating behaviors according to the medical students’ gender, year of studying and Grade Point Average. However, their eating attitudes and behaviors were, associated with Body Mass index. Normal weight (BMI 18.5- 23.49 and overweight (BMI 23.5-39.9 groups could increase by 2.2 (95% CI =1.2, 4.3 and 2.3 (95% CI=1.1, 4.8 times risk depending on atypical eating attitudes and abnormal eating behaviors respectively, when compared with the underweight group (BMI<18.5. Conclusion: There was no correlated difference in concerns to the Thai medical student’s abnormal eating habits, with gender, years of their study and Grade Point Average. Only normal to over-weight BMI were associated. Overweight male, medical students significantly represented more atypical attitudes towards eating and behaviors than other groups in this population. These results may reveal the changing trends of eating attitudes and behaviors due to the current ideal body image of being more muscular. However, prospective studies are still needed.

Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans

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Jae-Hyeok Lee

2016-01-01

Full Text Available Objective Neurodegeneration with brain iron accumulation (NBIA represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea. Methods We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN. Results Four subtypes of NBIA including PKAN (n = 30, PLA2G6-related neurodegeneration (n = 2, beta-propeller protein-associated neurodegeneration (n = 1, and aceruloplasminemia (n = 1 have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG. Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN. Conclusions We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.

The diagnosis and management of pre-invasive breast disease: Pathology of atypical lobular hyperplasia and lobular carcinoma in situ

International Nuclear Information System (INIS)

Simpson, Peter T; Gale, Theodora; Fulford, Laura G; Reis-Filho, Jorge S; Lakhani, Sunil R

2003-01-01

The term lobular neoplasia refers to a spectrum of lesions featuring atypical lobular hyperplasia and lobular carcinoma in situ (LCIS). The histopathological characteristics of these lesions are well documented. What is less well understood is the management implications of a patient diagnosed with LCIS; treatment regimes vary and are somewhat controversial. LCIS is now considered a risk factor and a non-obligate precursor for the subsequent development of invasive cancer

Survey report on the environment harmonizing type energy community survey project for the coastal area in the Kawasaki ward in fiscal 1998; 1998 nendo Kawasakiku rinkaibu chiiki kankyo chowagata energy community chosa jigyo chosa hokokusho

Energy Technology Data Exchange (ETDEWEB)

NONE

1999-03-01

In energy utilization in the 'Kawasaki Zero Emission Industrial Complex', optimal regional heat supply systems were discussed. The discussion was made from the viewpoints of introduction of saving-type energies, new energies and renewable energies, and environment preservation performance. The coastal area in the Kawasaki ward is planned of developing an industrial complex with an area of 8.4 ha for operation of about twenty companies. In the assumption of heat demand, four companies only were taken up as the object of the discussion, who utilize heat supply from steam as process heat source for their factories. The total heat demand quantity and heat demand pattern used for the discussion were assumed by hearing and actual record submitted from each company. Optimal systems were discussed on heat supply systems utilizing steam produced by steel mills, with the discussion made on waste heat recovered steam in processes and steam extracted from thermal electric power plants in the steel mills. As a result of the discussion, heat supply utilizing the steel mill steam was found capable of being provided with much lower price level as the estimated unit price than general direct steam supply, leading to a judgement that the system has business feasibility. (NEDO)

Early postnatal soluble FGFR3 therapy prevents the atypical development of obesity in achondroplasia

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Sarrazy, Vincent; Dumas, Karine; Authier, Florence; Sore, Sophie; Tran, Albert; Gual, Philippe; Gennero, Isabelle; Salles, Jean-Pierre; Gouze, Elvire

2018-01-01

Background Achondroplasia is a rare genetic disease is characterized by abnormal bone development and early obesity. While the bone aspect of the disease has been thoroughly studied, early obesity affecting approximately 50% of them during childhood has been somewhat neglected. It nevertheless represents a major health problem in these patients, and is associated to life-threatening complications including increasing risk of cardiovascular pathologies. We have thus decided to study obesity in patients and to use the mouse model to evaluate if soluble FGFR3 therapy, an innovative treatment approach for achondroplasia, could also impact the development of this significant complication. Methods and findings To achieve this, we have first fully characterized the metabolic deregulations in these patients by conducting a longitudinal retrospective study, in children with achondroplasia Anthropometric, densitometric measures as well as several blood parameters were recorded and compared between three age groups ranging from [0–3], [4–8] and [9–18] years old. Our results show unexpected results with the development of an atypical obesity with preferential fat deposition in the abdomen that is remarkably not associated with classical complications of obesity such as diabetes or hypercholosterolemia. Because it is not associated with diabetes, the atypical obesity has not been studied in the past even though it is recognized as a real problem in these patients. These results were validated in a murine model of achondroplasia (Fgfr3ach/+) where similar visceral adiposity was observed. Unexpected alterations in glucose metabolism were highlighted during high-fat diet. Glucose, insulin or lipid levels remained low, without the development of diabetes. Very interestingly, in achondroplasia mice treated with soluble FGFR3 during the growth period (from D3 to D22), the development of these metabolic deregulations was prevented in adult animals (between 4 and 14 weeks of age

Prader-Willi syndrome: a case report with atypical developmental features.

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Sewaybricker, Letícia E; Guaragna-Filho, Guilherme; Paula, Georgette B; Andrade, Juliana G R; Tincani, Bruna J; D'Souza-Li, Lília; Lemos-Marini, Sofia H V; Maciel-Guerra, Andréa T; Guerra-Júnior, Gil

2014-09-01

To describe the case of a male Prader-Willi syndrome (PWS) patient with atypical development features. We report the case of a male adolescent with confirmed diagnosis of PWS which presents atypical phenotype. The patient progressed with spontaneous and complete pubertal development, stature in the normal range, and weight control without any pharmacological treatment, except metformin. PWS is an imprinting paternally inherited disorder of 15q11-13 characterized by hypotonia in infant age, hyperphagia, varied degrees of mental retardation, behavior problems, hypogonadism, short stature, and other less common findings.

Atypical bulimia nervosa in a male patient of rural north-east India

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Manabendra Makhal

2014-01-01

Full Text Available Eating disorders such as bulimia nervosa rarely seem to manifest in males from non-Western cultures like India, but occur infrequently in milder forms with fewer symptoms as atypical bulimia nervosa. This report describes a male case of bulimia nervosa from rural area of India presenting with atypical features. He was treated successfully with fluoxetine and cognitive behavioural therapy.

Atypical work and employment continuity

OpenAIRE

Addison, John T.; Surfield, Christopher J.

2009-01-01

Atypical employment arrangements such as agency temporary work and contracting have long been criticized as offering more precarious and unstable work than regular employment. Using data from two datasets – the CAEAS and the NLSY79 – we determine whether workers who take such jobs rather than regular employment, or the alternative of continued job search, subsequently experience greater or lesser employment continuity. Observed differences between the various working arrangements are starkest...

Detection and identification of the atypical bovine pestiviruses in commercial foetal bovine serum batches.

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Hongyan Xia

Full Text Available The recently emerging atypical bovine pestiviruses have been detected in commercial foetal bovine serum (FBS of mainly South American origin so far. It is unclear how widely the viruses are presented in commercial FBS of different geographic origins. To further investigate the possible pestivirus contamination of commercially available FBS batches, 33 batches of FBS were obtained from ten suppliers and analysed in this study for the presence of both the recognised and the atypical bovine pestiviruses. All 33 batches of FBS were positive by real-time RT-PCR assays for at least one species of bovine pestiviruses. According to the certificate of analysis that the suppliers claimed for each batch of FBS, BVDV-1 was detected in all 11 countries and BVDV-2 was detected exclusively in the America Continent. The atypical pestiviruses were detected in 13 batches claimed to originate from five countries. Analysis of partial 5'UTR sequences showed a high similarity among these atypical bovine pestiviruses. This study has demonstrated, for the first time that commercial FBS batches of different geographic origins are contaminated not only with the recognised species BVDV-1 and BVDV-2, but also with the emerging atypical bovine pestiviruses.

Detection of a human intracisternal A-type retroviral particle antigenically related to HIV

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Garry, R. F.; Fermin, C. D.; Hart, D. J.; Alexander, S. S.; Donehower, L. A.; Luo-Zhang, H.

1990-01-01

Sjogren's syndrome is an autoimmune disease that is characterized by dryness of the mouth and eyes. The loss of salivary and lacrimal gland function is accompanied by lymphocytic infiltration. Because similar symptoms and glandular pathology are observed in certain persons infected with human immunodeficiency virus (HIV), a search was initiated for a possible retroviral etiology in this syndrome. A human intracisternal A-type retroviral particle that is antigenically related to HIV was detected in lymphoblastoid cells exposed to homogenates of salivary tissue from patients with Sjogren's syndrome. Comparison of this retroviral particle to HIV indicates that they are distinguishable by several ultrastructural, physical, and enzymatic criteria.

SAJCH 592.indd

African Journals Online (AJOL)

systemic-onset juvenile rheumatoid arthritis (JRA) may have similar nonspecific ... disease with recurrent skin peeling: A case report with the review of literature. J Postgrad ... infants, children and patients with Kawasaki disease. Pediatr Int ...