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Sample records for atypical kawasaki disease

  1. ATYPICAL KAWASAKI DISEASE.

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    Ristovski, Ljiljana; Milankov, Olgica; Vislavski, Melanija; Savić, Radojica; Bjelica, Milena

    2016-01-01

    Kawasaki disease is an acute vasculitis which occurs primarily in children under the age of 5. The etiology of the disease is still unknown. Diagnostic criteria for Kawasaki disease are fever and at least four of the five additional clinical signs. Incomplete Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. The diagnosis of incomplete Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients. CASE REPORT. In this report we present a case of atypical Kawasaki disease in a 3.5-month-old infant. As soon as the diagnosis was made, the patient received high doses of intravenous immunoglobulin, with the initial introduction of ibuprofen, then aspirin with a good clinical response. Due to the presence of aneurysm of coronary arteries, further therapy involved aspirin and clopidogrel over the following 3 months, and then only aspirin for 2 years. There was a gradual regression of the changes in the coronary blood vessels to the normalization of the echocardiographic findings after 2 years. Kawasaki disease is the second most common vasculitis of childhood, so it should be included in the differential diagnosis for any child with a prolonged unexplained fever. Atypical Kawasaki disease should be taken into consideration in cases when not all clinical criteria are present but coronary abnormalities are documented.

  2. [Neurological manifestations in atypical Kawasaki disease].

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    Martínez-Guzmán, Edgar; Gámez-González, Luisa Berenise; Rivas-Larrauri, Francisco; Sorcia-Ramírez, Giovanni; Yamazaki-Nakashimada, Marco

    2017-01-01

    Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease. Treatment consisted of immunoglobulin and corticosteroids with good evolution. KD is a systemic vasculitis that can involve many territories. Atypical manifestations can mislead the clinician and delay diagnosis. Pediatricians and sub-specialists should be aware of these neurological manifestations in order to provide adequate and opportune treatment.

  3. Unusual imaging presentation of infantile atypical Kawasaki disease.

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    Kumar, Nishith; Mittal, Mahesh Kumar; Sinha, Mukul; Gupta, Arpita; Thukral, Brij Bhushan

    2016-01-01

    Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  4. Kawasaki Disease

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    Kawasaki disease is a rare childhood disease. It makes the walls of the blood vessels in the ... veins, and capillaries. No one knows what causes Kawasaki disease. Symptoms include High fever that lasts longer ...

  5. Living with Kawasaki Disease

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    ... Research Home / Kawasaki Disease Kawasaki Disease What Is Kawasaki (KAH-wah-SAH-ke) disease is a rare ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout ...

  6. Multiple giant succular and fusiform right and left coronary artery aneurysms after early and adequate treatment of atypical kawasaki disease with unusual presentation.

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    Mostafa Behjati-Ardakani

    2014-06-01

    Full Text Available The major complication of Kawasaki disease is coronary artery dilatation and aneurysm. It occurs in approximately 15-25% of untreated children with Kawasaki Disease. Early diagnosis and treatment with Intravenous immune globulin (IVIG and aspirin (ASA can reduce the incidence of coronary artery abnormality to 2%-5%. We report one case of Atypical Kawasaki Disease with Multiple giant coronary artery aneurysms despite early adequate treatment with IVIG and ASA.

  7. What Is Kawasaki Disease?

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    ANSWERS by heart Cardiovascular Conditions What is Kawasaki Disease? Kawasaki disease is a children’s illness. It’s also known as Kawasaki syndrome or mucocutaneous lymph node syndrome. About 75 percent of people ...

  8. Kawasaki Disease (For Parents)

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    ... Late for the Flu Vaccine? Eating Disorders Arrhythmias Kawasaki Disease KidsHealth > For Parents > Kawasaki Disease Print A A A What's in this ... Complications Diagnosis Treatment en español La enfermedad de Kawasaki Kawasaki disease is an illness that involves the ...

  9. How Is Kawasaki Disease Diagnosed?

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    ... Research Home / Kawasaki Disease Kawasaki Disease What Is Kawasaki (KAH-wah-SAH-ke) disease is a rare ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout ...

  10. How Is Kawasaki Disease Treated?

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    ... Research Home / Kawasaki Disease Kawasaki Disease What Is Kawasaki (KAH-wah-SAH-ke) disease is a rare ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout ...

  11. A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus.

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    Micallef, Eynaud S; Attard, Montalto S; Grech, V

    2016-01-01

    Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation. Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied.

  12. A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus

    OpenAIRE

    Micallef Eynaud, Stephen; Attard Montalto, Simon; Grech, Victor E.

    2016-01-01

    Introduction Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. Case Presentation A 9-month-old Caucasian boy...

  13. Evidence of Acute Mycoplasma Infection in a Patient with Incomplete and Atypical Kawasaki Disease: A Case Report

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    M. Ebrahim

    2011-01-01

    Full Text Available The etiology of Kawasaki disease remains unknown despite extensive studies. Some researchers suggest that it is caused by an infectious agent. This is a case report where a patient with incomplete Kawasaki disease was found to have evidence compatible with acute Mycoplasma pneumoniae infection. This is one of the several case reports linking Mycoplasma pneumoniae to Kawasaki disease as a possible trigger. This is perhaps due to a superantigen or is mediated by some other mechanism. Accurate and timely testing for Mycoplasma infections is difficult and has its limitations. Despite this, Mycoplasma pneumoniae should be considered in the differential and workup for Kawasaki disease.

  14. Genetics Home Reference: Kawasaki disease

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    ... Email Facebook Twitter Home Health Conditions Kawasaki disease Kawasaki disease Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Kawasaki disease is a sudden and time-limited (acute) ...

  15. History of Kawasaki disease.

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    Kawasaki, Tomisaku; Naoe, Shiro

    2014-04-01

    We describe a short history of Kawasaki disease. In 1967, we published a paper entitled 'Infantile acute febrile mucocutaneous lymph node syndrome with specific desquamation of the fingers and toes. Clinical observation of 50 cases'; this was the first report on what is now called Kawasaki disease. Since then, many reports on cardiology, treatment, epidemiology, pathology and etiology of Kawasaki disease have been published. Furthermore, a recent Chapel Hill Consensus Statement on Kawasaki disease in the classification of vasculitis is given, along with a figure on the relationship and classification of childhood vasculitis by autopsy material.

  16. Extreme thrombocytosis predicts Kawasaki disease in infants.

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    Nigrovic, Lise E; Nigrovic, Peter A; Harper, Marvin B; Chiang, Vincent W

    2006-06-01

    Infants with Kawasaki disease are at high risk of developing life-threatening coronary complications, yet may elude timely diagnosis because they often lack the full complement of classic clinical features. We retrospectively studied 26,540 children 1 year of age or less who were evaluated at a tertiary care pediatric emergency department in whom a platelet count was performed. Among those infants with fever without a source identified, 8.5% with platelet counts of 800,000 cells/mm(3) or greater had Kawasaki disease compared to 0.4% with platelet counts of less than 800,000 cells/mm(3) (likelihood ratio for Kawasaki disease was 17 [95% confidence interval, 8-34]). Because many infants present atypically, Kawasaki disease should be considered in all children of 1 year or less with prolonged fever, extreme elevation of the platelet count, and no compelling alternative diagnosis.

  17. 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.

  18. What Are the Signs and Symptoms of Kawasaki Disease?

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    ... Research Home / Kawasaki Disease Kawasaki Disease What Is Kawasaki (KAH-wah-SAH-ke) disease is a rare ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout ...

  19. Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl.

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    Doğan, Vehbi; Karaaslan, Erhan; Özer, Samet; Gümüşer, Rüveyda; Yılmaz, Resul

    2016-07-01

    Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease. Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.

  20. Kawasaki Disease: Complications, Treatment and Prevention

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    ... Peripheral Artery Disease Venous Thromboembolism Aortic Aneurysm More Kawasaki Disease: Complications, Treatment and Prevention Updated:May 8, ... possibility of heart and coronary artery involvement makes Kawasaki disease unpredictable, but these problems usually are not ...

  1. Kawasaki Disease Presenting as Acute Clinical Hepatitis

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    Seyed Ali Jafari

    2013-12-01

    Full Text Available Kawasaki disease is a systemic vasculitis of children. Among gastrointestinal symptoms of this disease jaundice occurs uncommonly. We present a 23 month boy with icter and clinical hepatitis and final diagnosis of kawasaki disease.

  2. Kawasaki disease for dermatologists

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    Aman Gupta

    2016-01-01

    Full Text Available Kawasaki disease (KD is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term “incomplete KD” refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be “incomplete” at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.

  3. Recognition of a Kawasaki Disease Shock Syndrome

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    Kanegaye, John T.; Wilder, Matthew S.; Molkara, Delaram; Frazer, Jeffrey R.; Pancheri, Joan; Tremoulet, Adriana H.; Watson, Virginia E.; Best, Brookie M.; Burns, Jane C.

    2010-01-01

    objective We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease. methods We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of ≥20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease. results Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients [31%] vs 2 of 86 patients [4%]), mitral regurgitation (5 of 13 patients [39%] vs 2 of 83 patients [2%]), coronary artery abnormalities (8 of 13 patients [62%] vs 20 of 86 patients [23%]), and intravenous immunoglobulin resistance (6 of 13 patients [46%] vs 32 of 174 patients [18%]). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances. conclusions Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery

  4. Kawasaki disease and immunisation: A systematic review.

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    Phuong, Linny Kimly; Bonetto, Caterina; Buttery, Jim; Pernus, Yolanda Brauchli; Chandler, Rebecca; Felicetti, Patrizia; Goldenthal, Karen L; Kucuku, Merita; Monaco, Giuseppe; Pahud, Barbara; Shulman, Stanford T; Top, Karina A; Trotta, Francesco; Ulloa-Gutierrez, Rolando; Varricchio, Frederick; de Ferranti, Sarah; Newburger, Jane W; Dahdah, Nagib; Singh, Surjit; Bonhoeffer, Jan; Burgner, David

    2017-03-27

    Kawasaki disease is a complex and potentially serious condition. It has been observed in temporal relation to immunisation. We conducted a systematic literature review using various reference sources to review the available evidence published in the literature. We identified twenty seven publications reporting a temporal association between immunisation and Kawasaki disease. We present a systematic review of data drawn from randomised controlled trials, observational studies, case series and reports, and reviews. Overall there was a lack of standardised case definitions, making data interpretation and comparability challenging. Although a temporal relationship between immunisation and Kawasaki disease is suggested, evidence for an increased risk or a causal association is lacking. Implementation of a standardised Kawasaki disease case definition would increase confidence in the findings and add value to future studies of pre- or post-licensure vaccine safety studies. Copyright © 2016. Published by Elsevier Ltd.

  5. Kawasaki disease - an Indian perspective.

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    Singh, Surjit; Kawasaki, Tomisaku

    2009-07-01

    Kawasaki disease (KD) was first reported from Japan in 1967 by a young pediatrician, Tomisaku Kawasaki, while working at the Red Cross Hospital in Tokyo. Soon therafter, Marian Melish independently reported children with a similar clinical profile from Hawaii in the United States. KD has now been reported from all parts of the world, including several centers in India. Based on the epidemiology and clinical features, an infectious etiology has been suspected for long but no definitive causative agent has been implicated so far. Like many other vasculitides, the diagnosis of this condition is based on the recognition of a temporal sequence of clinical features, none of which is pathognomonic in isolation. KD is believed to be the commonest vasculitic disorder of children. Incidence rates as high as 60-150 per 100,000 children below 5 years of age have been reported from several countries. In India (as also perhaps in many other developing countries), however, majority of children with KD continue to remain undiagnosed probably because of the lack of awareness amongst pediatricians. The clinical features of KD can be confused with other common conditions like scarlet fever and the Stevens Johnson syndrome, if the clinician is not careful. Development of coronary artery abnormalities (CAA) is the hallmark of KD and accounts for most of the morbidity and mortality associated with the disease. Prompt recognition of the disease and early initiation of treatment with intravenous immunoglobulin (IVIG) results in significant reduction in the occurrence of CAA. It is, therefore, imperative for the pediatrician to diagnose and treat KD expeditiously. KD should be considered in the differential diagnosis of all febrile illnesses in young children where the fever persists for more than 5-7 days.

  6. Management of Kawasaki disease in adults.

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    Denby, Kara J; Clark, Daniel E; Markham, Larry W

    2017-11-01

    Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Sensorineural hearing loss in Kawasaki disease.

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    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  8. kawasaki disease in ghana: case reports from korle bu teaching ...

    African Journals Online (AJOL)

    pyretics and antibiotics, accompanied by a rash and non purulent conjunctivitis should be sus- pected to have Kawasaki disease. Keywords: Kawasaki Disease, Coronary Arterial. Aneurysms, Aspirin, Ghana. INTRODUCTION. Kawasaki disease (KD) is a systemic vasculitis affecting small to medium sized arteries but with a.

  9. Role of the Egami Score in Predicting Intravenous Immunoglobulin Resistance in Kawasaki Disease Among Different Ethnicities.

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    Loomba, Rohit S; Raskin, Alexander; Gudausky, Todd M; Kirkpatrick, Edward

    Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.

  10. Kawasaki Disease and Peripheral Gangrene in Infancy.

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    Malekzadeh, Iran; Ziaee, Vahid; Sadrosadat, Taravat; Moardinejad, Mohammad-Hassan; Sayadpour-Zanjani, Keyhan

    2015-12-01

    Early diagnosis and treatment of Kawasaki disease as the most common cause of acquired heart disease in childhood, may significantly improve the prognosis. Diagnosing infantile Kawasaki (younger than a year) is difficult because of obscure symptoms; at the same time they are at the higher risk of coronary abnormalities. We report three infants with prolonged (more than 5 days) fever and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed due to dilation of coronary artery and other aortic branches, thrombocytosis, and rising of ESR and CRP. All patients were treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. Additionally, cytotoxic drugs or infliximab were used for two of them because of severe aneurysms in the aortic branches. All 3 patients received aspirin with anti-platelet aggregation dose and 2 patients heparin as an anti-coagulant agent for longtime. After adequate treatment, peripheral gangrene, arterial dilations and aneurysms improved, but during 12 months follow-up coronary aneurysms did not improve completely. Peripheral gangrene must be regarded as an important sign of infantile Kawasaki disease early treatment of which can prevent severe permanent coronary involvements and sequels.

  11. Kawasaki disease: etiopathogenesis and novel treatment strategies.

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    Agarwal, Shreya; Agrawal, Devendra K

    2017-03-01

    Kawasaki disease is an acute febrile systemic vasculitis that predominantly occurs in children below five years of age. Its etiopathogenesis is still not clear, but it is thought to be a complex interplay of genetic factors, infections and immunity. Areas covered: This review article discusses in detail Kawasaki disease, with particular emphasis on the recent updates on its pathogenesis and upcoming alternate treatment options. Though self-limiting in many cases, it can lead to severe complications like coronary artery aneurysms and thrombo-embolic occlusions, and hence requires early diagnosis and urgent attention to avoid them. Intravenous immunoglobulin (IVIG) with or without aspirin has remained the sole treatment option for these cases, but 10-15% cases develop resistance to this treatment. Expert commentary: There is a need to develop additional treatment strategies for children with Kawasaki disease. Targeting different steps of pathogenesis could provide us with alternate therapeutic options.

  12. Doença de Kawasaki Kawasaki disease

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    Patrícia Aparecida de Castro

    2009-08-01

    Full Text Available A doença de Kawasaki é vasculite sistêmica e aguda de etiologia desconhecida. Constitui a principal causa de doença cardíaca adquirida em crianças nos EUA. Ocorre mais frequentemente em meninos, 80% dos casos em crianças com menos de cinco anos, sendo rara após os oito anos. Pode atingir crianças de todas as raças, tendo maior incidência entre os descendentes asiáticos. Caracteriza-se por febre, conjuntivite bilateral não exsudativa, eritema e edema de língua, lábios e mucosa oral, alterações de extremidades, linfonodomegalia cervical, exantema polimórfico. Aneurismas e estenoses de artérias coronárias são comuns em percentual que varia de 20 a 25% dos pacientes não tratados, podendo posteriormente levar a infarto agudo do miocárdio e morte súbita. O tratamento com imunoglobulina intravenosa é efetivo e deve ser iniciado precocemente a fim de evitar sequelas cardíacas. O desenvolvimento de testes diagnósticos, terapêuticas mais específicas e a prevenção dessa doença potencialmente fatal em crianças dependem dos contínuos avanços na determinação de sua etiopatogenia.Kawasaki disease is a systemic acute vasculitis of unknown etiology. It is the leading cause of acquired heart disease in children in the USA. It occurs more frequently in boys and eighty percent of the cases occur in children under five years of age. The disease rarely occurs after eight years and it can affect children of all races, with higher incidence among Asian descendants. Kawasaki disease is characterized by fever, bilateral non-exudative conjunctivitis, redness and swelling of the tongue, lips and oral mucosa, abnormalities in the extremities, cervical lymph node, and polymorphic exanthema. Aneurysms and stenoses of coronary arteries occur in pproximately 20 to 25% of untreated patients and subsequently can lead to acute myocardial infarction and sudden death. Treatment with intravenous immunoglobulin is effective and should be initiated

  13. Influenza infection and Kawasaki disease

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    Xijing Huang

    2015-06-01

    Full Text Available INTRODUCTION: The objective of this study was to investigate the possible link between influenza (Flu infection and Kawasaki disease (KD. METHODS: We examined the medical records of 1,053 KD cases and 4,669 influenza infection cases hospitalized at our institute from January 1, 2011 to December 31, 2013. Cases of KD with concomitant influenza infection formed the KD + Flu group. Each KD + Flu case was matched with 2 KD cases and 2 influenza infection cases, and these cases were assigned to the KD group and Flu group, respectively. The differences in the principal clinical manifestations, course of disease, incomplete KD rate, intravenous immunoglobulin (IVIG resistance rate, and echocardiographic detection results between the KD + Flu group and KD group were compared. The fever durations and laboratory test results of these three groups were compared. RESULTS: 1 The seasonal variations of the KD + Flu group, KD group and Flu group were similar. 2 The morbidity rate of incomplete KD was higher in the KD + Flu group compared with the KD group. 3 Patients in the KD + Flu group exhibited a longer time to KD diagnosis compared with patients in the KD group. 4 The KD + Flu group exhibited the longest fever duration among the three groups. 5 The CRP and ESR values in the KD + Flu group were higher those in the Flu or KD groups. CONCLUSIONS: Concomitant influenza infection affects the clinical manifestations of KD and can impact the laboratory test results and the diagnosis and treatment of the disease. However, it remains unclear whether influenza contributes to KD etiology.

  14. Seasonality of Kawasaki disease: a global perspective

    NARCIS (Netherlands)

    Burns, Jane C.; Herzog, Lauren; Fabri, Olivia; Tremoulet, Adriana H.; Rodó, Xavier; Uehara, Ritei; Burgner, David; Bainto, Emelia; Pierce, David; Tyree, Mary; Cayan, Daniel; Dahdah, N.; Gibbon, M.; Scuccimarri, R.; McCrindle, B.; Manlhiot, C.; Jin, C.-J.; Jin, L.-H.; Jin, Z.-Y.; Piao, J.-H.; Zhou, Y.; Jiao, F.; Huang, G.-Y.; Salo, E.; Cimaz, R.; Di-Filippo, S.; Lega, J.-C.; Aulakh, R.; Singh, S.; Suri, D.; Bar-Meir, M.; Nakamura, Y.; Uehara, R.; Lee, K.-Y.; Han, J.-W.; Han, M.-K.; Hong, Y.-M.; Jang, G.-Y.; Kim, D.-S.; Lee, H.-D.; Lee, J.-K.; Park, I.-S.; Song, M.-S.; Yun, S.-W.; Breunis, W. B.; Kuijpers, T. W.; Tacke, C. E.; Lyskina, G.; Shirniskaya, O.; Torbyak, A.; Bregel, L.; Soldatova, T.; Subbotin, V.; Anton, J.; Prada, F.; Ricart, S.; Bou, R.; Atalay, S.; Çiftçi, E.; İnce, E.; Karbuz, A.; Özdemir, H.; Harnden, A.; Levin, M.; Mayon-White, R.; Tulloh, R.; Michie, C.; Wright, V.; Baker, A. L.; Newburger, J. W.; Innocentini, N.; Shulman, S. T.; Anderson, M.; Dominguez, S.; Glode, M.; Arrieta, A.; Dozal, C.; Mason, W.; Beck, J.; Burns, J. C.; Tremoulet, A.; Fernandez, S.; Magalhaes, C. M.; Pratesi, R.; Cheung, Y.-F.; Reyes, M.; Advani, N.; Olugbuyi, O.; Pierre, R.; Castaño, E.; Estripeaut, D.; Sáez-Llorens, X.; Chen, C. K.; Choo, T. L. J.; Tan, T. H.; Wong, K. Y.; Kuo, H.-C.; Lin, M.-T.; Wu, M.-H.; Sittiwangkul, R.; Melish, M.; Burgner, D.; Odam, M.; Salgado, A.; Soza, G.; Doran, J.; Heaton, P.; Wilson, N.; Eley, B.; Moore, D.

    2013-01-01

    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. Data from 1970-2012 from 25 countries distributed over the globe were

  15. Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease.

    Science.gov (United States)

    Çakan, Mustafa; Gemici, Hakan; Aktay-Ayaz, Nuray; Keskindemirci, Gonca; Bornaun, Helen; İkizoğlu, Tarkan; Çeliker, Alpay

    2016-01-01

    Kawasaki disease is an acute systemic vasculitis that occurs most commonly in young children. It affects medium-sized muscular arteries and the coronary arteries are the predominant site of involvement. Morbidity and mortality is generally due to coronary artery aneurysms that develop during the chronic phase. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. Herein we describe two cases of Kawasaki disease shock syndrome that were treated in the pediatric intensive care unit and followed a course without morbidity or mortality.

  16. Management of acute and refractory Kawasaki disease

    NARCIS (Netherlands)

    Tacke, Carline E.; Burgner, David; Kuipers, Irene M.; Kuijpers, Taco W.

    2012-01-01

    Expert Rev. Anti Infect Ther. 10(10), 1203-1215 (2012) Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less

  17. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

    Science.gov (United States)

    Koh, Young Kwon; Lee, Jae Hee; Park, Yeong Bong

    2017-07-01

    Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

  18. Erythema at BCG Inoculation Site in Kawasaki Disease Patients.

    Science.gov (United States)

    Rezai, Mohammad Sadegh; Shahmohammadi, Soheila

    2014-08-01

    Kawasaki disease is an acute, self-limiting childhood systemic vasculitis with unknown etiology. Because there is no diagnostic test for Kawasaki disease, the diagnosis is based on clinical criteria. An important clinical sign that is not included in the classical clinical criteria for Kawasaki disease is a reaction at the Bacille Calmette-Guérin inoculation site that are present in about 30-50% of Kawasaki disease patients. of this review was to highlight the usefulness of the inflammation at the Bacille Calmette-Guérin inoculation site for early diagnosis of Kawasaki disease, we conducted a literature review on Medline in PubMed area, Google scholar, Magiran and Scientific Information Database using the search terms "Kawasaki disease, Erythema, BCG, inoculation site, children, cardiac complications, coronary artery lesion, aneurysm, incomplete Kawasaki in 2013. A total of 15 articles had been found. Erythema at the Bacille Calmette-Guérin inoculation site was found in 49.87% of Kawasaki disease patients. Coronary artery abnormalities were found in 10.3% of cases. According to this review, BCGitis is more prevalent than cervical lymphadenopathy and rash and it can be a useful criterion in the diagnosis of incomplete Kawasaki disease in cases not fulfills the classic criteria of at least four of the five findings.

  19. Risk of coronary artery involvement in Kawasaki disease.

    Science.gov (United States)

    Soriano-Ramos, María; Martínez-Del Val, Elena; Negreira Cepeda, Sagrario; González-Tomé, María I; Cedena Romero, Pilar; Fernández-Cooke, Elisa; Albert de la Torre, Leticia; Blázquez-Gamero, Daniel

    2016-04-01

    Kawasaki disease refers to systemic vasculitis with risk of coronary artery disease. Our objective is to identify risk factors associated with coronary artery disease in patients with complete and incomplete Kawasaki disease. Descriptive, retrospective study conducted in patients diagnosed with Kawasaki disease in a tertiary-care hospital between 2008 and 2014. The American Heart Association diagnostic criteria were used to define complete and incomplete Kawasaki disease. Thirty-one children were diagnosed with Kawasaki disease; 24 met the criteria for the complete form, and 7, for the incomplete form of this condition. Five had coronary artery disease. One of them had incomplete Kawasaki disease (1/7= 14.3%), and the remaining four had the complete form (4/24= 16.7%). No significant differences were found between both groups (p= 1.0). Patients with coronary artery involvement had a higher C-reactive protein level (median: 16.2 mg/dL versus 8.4 mg/dL, p= 0.047) and lower albuminemia (median: 3.2 mg/dL versus 3.99 mg/dL, p= 0.002). The risk of coronary artery involvement in incomplete Kawasaki disease is similar to that in complete Kawasaki disease; therefore, in patients with the incomplete form, immunoglobulin therapy should not be delayed. In our population, C-reactive protein and albumin levels were related to a higher risk of coronary artery involvement. Sociedad Argentina de Pediatría.

  20. Update on etio and immunopathogenesis of Kawasaki disease.

    Science.gov (United States)

    Takahashi, Kei; Oharaseki, Toshiaki; Yokouchi, Yuki

    2014-01-01

    This review first discusses the pathogenesis of Kawasaki disease based on the results of recently performed studies aimed at identifying Kawasaki disease-susceptibility genes and the results of analyses of the immune system. Following that, we discuss the findings generated using a murine Kawasaki disease arteritis model and speculate regarding the mechanism of Kawasaki disease onset based on immune function aberrations seen in that model. Recent advances in gene analysis studies of Kawasaki disease are contributing not only to prediction of disease susceptibility but also to improving our understanding of the pathogenesis of Kawasaki disease and development of new improved therapies. In addition, Th17/Treg imbalance is observed in patients with acute-phase Kawasaki disease. Th17/Treg imbalance may be an important factor causing disturbed immunological function. IL-17 induced by Th17 cells have proinflammatory properties and act on inflammatory cells, thereby inducing expression of cytokines and chemokines and resulting in tissue inflammation. Kawasaki disease vasculitis may be triggered by aberrant activation of inflammatory cytokines mediated by IL-17 that is produced by Th17 cells that have been activated by some infectious agent(s).

  1. [Kawasaki disease: report of 6 cases].

    Science.gov (United States)

    Scarpellini Vera, A; Borque, C; García Sicilia, J; Barrionuevo, J L; Sebastián, M; Hortelano, J G

    1984-06-01

    Six cases of children between 20 months and 6 years old, who suffer Kawasaki disease are presented. All of them had diagnostic parameters required by the Investigation Committee of this disease, with similar clinical symptoms as in other countries but with an upper average age (3 years old) to Japanese patients and nearer to that of the North American patients. It is important to emphasize the appearance of exanthema in one case, as well as a multiforme erythema exudative, a rare fact, and the observation, up to now not shown, of a positive rheumatoid factor in another two of the patients. All have undergone a good evolution without cardiovascular complications.

  2. [Aftermaths of lesions of coronary arteries in Kawasaki disease].

    Science.gov (United States)

    Vostokova, A A; Grunina, E A; Klemenov, A V

    2016-01-01

    Kawasaki disease, also known as cutaneous-mucous-glandular mucocutaneous glandular syndrome, is acute systemic vasculitis of small-to-medium calibre arteries, frequently involving coronary arteries, affect almost exceptionally children, with reports concerning cases of Kawasaki syndrome in 20-to-30-year-old adults being extremely rare. The most serious manifestation of Kawasaki disease is coronaritis and formation of coronary artery aneurysms. The dynamics of the formed coronary aneurysms and, consequently, the fate of patients may be different. Thrombosis of an aneurysm in the early period of the disease and stenosing of the affected coronary artery later on present possible complications of Kawasaki disease and potential causes of myocardial infection in young adults. Increased risk of coronary artery thromboses in Kawasaki disease is conditioned by a decrease in velocity of blood flow and its turbulent pattern in the aneurysms, endothelial dysfunction due to currently existing or endured coronaritis and thrombocytosis typical of this pathology. Predisposing factors of coronary artery stenosing are unfavourable haemodynamic conditions appearing at the sites of the "entry" and "exit" of the aneurysm. Described herein are two case reports of myocardial infarction, one of which being a complication of an acute case of Kawasaki disease in a 29-year-old patient, with the second one being a consequence of coronary artery stenosing in a 25-year-old patient who had endured Kawasaki disease in his childhood.

  3. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence.

    Science.gov (United States)

    Bratis, Konstantinos; Hachmann, Pauline; Child, Nicholas; Krasemann, Thomas; Hussain, Tarique; Mavrogeni, Sophie; Botnar, Rene; Razavi, Reza; Greil, Gerald

    2017-01-01

    The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

  4. Kawasaki Disease in A Nigerian Child- a case report | Sotimehin ...

    African Journals Online (AJOL)

    cutaneous hypersensitivity reaction during treatment for a febrile illness, but eventually manifested all the diagnostic criteria for Kawasaki Disease (KD) while on admission. Although an initial diagnosis of Steven-Johnson's Syndrome was made, ...

  5. Severe jaundice in two children with Kawasaki disease: a possible association with Gilbert syndrome.

    Science.gov (United States)

    Karpathios, Themistocles; Moustaki, Maria; Yiallouros, Panagiotis; Sharifi, Fariba; Attilakos, Achilleas; Papadopoulou, Anna; Fretzayas, Andrew

    2012-01-01

    Kawasaki disease is a systemic vasculitis, mainly encountered in children. It may affect any organ. Acute cholestasis and severe obstructive jaundice is an atypical manifestation of the disease. We herein present two children with Kawasaki disease and severe direct hypebilibirunemia who also were homozygous and heterozygous respectively for the (TA)(7) promoter polymorphism of Gilbert syndrome. Intravenous immunoglobulin was administered to both patients at the acute phase of the disease and the fever remitted within 24 hr following the immunoglobulin administration. Furthermore oral aspirin at a dose of 80-100 mg/kg/24 hr was also given. The first child did not develop any coronary ectasia or aneurysm, whereas dilation of the right coronary artery was identified in the second child, one month after the disease onset. We discuss the possible contribution of Gilbert syndrome to the development of jaundice in our patients.

  6. Role of imaging studies in Kawasaki disease.

    Science.gov (United States)

    Tsuda, Etsuko; Singhal, Manphool

    2017-11-08

    Kawasaki disease is a medium vessel vasculitis which may be associated with coronary artery abnormalities. Recognition of these abnormalities depends upon various imaging modalities. While two-dimensional echocardiography remains the first line modality to identify coronary artery abnormalities, it is subject to several fallacies and is operator dependent. Computed tomography coronary angiography is rapidly emerging as a useful imaging modality for better characterization of dilatations, ectasia and aneurysms in the mid- and distal segments of coronary arteries. It provides precise details in terms of aneurysm size and morphology. In this review we here described the importance of computed tomography coronary angiography and have also given a brief description of magnetic resonance coronary angiography. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  7. Erythema Marginatum as an Unusual Presentation for Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    I. Sedighi

    2008-10-01

    Full Text Available Introduction: Kawasaki disease is an acute, systemic vacuity that predominantly manifests in infants and young children. Severe systemic inflammation and vacuities can cause cardiovascular involvement, particularly coronary artery injury that includes dilatation or aneurysm. The final goal of treatment for acute Kawasaki disease، therefore، is to diminish systemic inflammation and vacuities as soon as possible, thereby, preventing progression to coronary artery injury and thrombosis.Case Report: In this report we present a case of Kawasaki disease in a 7-year-old boy with prolonged fever and cervical adenopathy presented with erythema marginatum as a first skin presentation. We treated the patient with intravenous gamma-globulin (IVIG by six day after admission. He developed cardiac and pulmonary involvement two days later.Conclusion: Any skin rash is described in Kawasaki disease except for bullous and vesicular lesions. The common skin manifestation of Kawasaki disease are erythematous and maculopapular rash, although it may appear urticarial, scarlatiniform, erythema multiforme-like, or as erythroderma. Erythema marginatum had a limited differential diagnosis in pediatric patients. The major cause of erythema marginatum is rheumatic fever but there is no report of erythema marginatum in face and cervical region and Kawasaki disease should be considered in differential diagnosis of erythema marginatum in face and neck.

  8. Laporan Kasus: Penyakit Kawasaki Atipikal

    Directory of Open Access Journals (Sweden)

    Budi Setiabudiawan

    2011-09-01

    Full Text Available Kawasaki disease is the most common cause of acquired heart disease in children. In Indonesia the disease is rare to diagnosed, because of difficulty in diagnosis and not widely known. These were 2 case reports about a girl and a boy age 17 months and 3 years, who came with persistent fever more than 5 days and only fulfilled 3 criteria of Kawasaki disease, which are red eyes, changes in lips, mucose of oral and extremities. They were diagnosed as atypical Kawasaki disease. Laboratory examinations showed an increased of C-reactive protein and erythrocyte sedimentation rate with normal echocardiography. The patients were improved after treated with 2 grams per bodyweight of intravenous immunoglobulin (IVIG and 80 mg per bodyweight of aspirin. The patients were better after one day combination therapy. In conclusion that atypical Kawasaki disease has good response to combination of IVIG and aspirin.

  9. Kawasaki disease: a matter of innate immunity.

    Science.gov (United States)

    Hara, T; Nakashima, Y; Sakai, Y; Nishio, H; Motomura, Y; Yamasaki, S

    2016-11-01

    Kawasaki disease (KD) is an acute systemic vasculitis of childhood that does not have a known cause or aetiology. The epidemiological features (existence of epidemics, community outbreaks and seasonality), unique age distribution and clinical symptoms and signs of KD suggest that the disease is caused by one or more infectious environmental triggers. However, KD is not transmitted person-to-person and does not occur in clusters within households, schools or nurseries. KD is a self-limited illness that is not associated with the production of autoantibodies or the deposition of immune complexes, and it rarely recurs. Regarding the underlying pathophysiology of KD, innate immune activity (the inflammasome) is believed to play a role in the development of KD vasculitis, based on the results of studies with animal models and the clinical and laboratory findings of KD patients. Animal studies have demonstrated that innate immune pathogen-associated molecular patterns (PAMPs) can cause vasculitis independently of acquired immunity and have provided valuable insights regarding the underlying mechanisms of this phenomenon. To validate this concept, we recently searched for KD-specific PAMPs and identified such molecules with high specificity and sensitivity. These molecules have structures similar to those of microbe-associated molecular patterns (MAMPs), as shown by liquid chromatography-tandem mass spectrometry. We propose herein that KD is an innate immune disorder resulting from the exposure of a genetically predisposed individual to microbe-derived innate immune stimulants and that it is not a typical infectious disease. © 2016 British Society for Immunology.

  10. Sensorineural hearing loss in patients with Kawasaki disease.

    Science.gov (United States)

    Park, Sun Young; Kim, Young Hyun; Kim, Yeo Hyang; Hyun, Myung Chul; Lee, Young Hwan

    2015-11-01

    Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

  11. Tomographic myocardial perfusion scintigraphy in children with Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-12-01

    Myocardial infarction and stenotic coronary lesions are serious late complications in children with Kawasaki disease. For the noninvasive assessment of myocardial perfusion, dipyridamole-redistribution /sup 201/Tl emission computed tomography (ECT) was performed in seven children (age 2 8/12-8 7/12 yr) 3-20 mo after the acute stage of the disease. In all patients, coronary aneurysms had been demonstrated by cross-sectional echocardiography. The scintigrams of six children showed no significant regional reduction of myocardial thallium uptake. These children had remained asymptomatic since the acute stage of Kawasaki disease. Persistent and transient thallium defects were present in one child with documented myocardial infarction. For this patient, obstruction of corresponding coronary vessels was confirmed by contrast angiography. It is suggested, that /sup 201/Tl ECT after dipyridamole-induced vasodilation may be used as a safe alternative to invasive coronary angiography for follow-up investigations in patients with Kawasaki disease.

  12. Coronary CT angiography and MR angiography of Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology, Asan Medical Center, 388-1 Poongnap-2dong, Songpa-gu, Seoul (Korea); Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee [University of Ulsan College of Medicine, Department of Paediatric Cardiology, Asan Medical Center, Seoul (Korea)

    2006-07-15

    Although the incidence of coronary artery aneurysms has diminished in patients with Kawasaki disease, coronary artery involvement is still regarded as a major complication of the disease, significantly affecting morbidity and mortality. Recent technical advances in coronary CT angiography (CTA) and MR angiography (MRA) have led to the possibility of using these two imaging methods as minimally invasive alternatives to the more invasive diagnostic catheter angiography in evaluating coronary artery abnormalities, such as aneurysm, stenosis, and occlusion. In this article, we describe imaging techniques and findings of coronary CTA and MRA in Kawasaki disease. (orig.)

  13. 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 ...

  14. Incomplete Kawasaki disease - a diagnostic and therapeutic challenge

    Directory of Open Access Journals (Sweden)

    S Datta

    2014-04-01

    Full Text Available Background The diagnosis of classical Kawasaki Disease was based on clinical criteria. The conventional criteria is particularly useful in preventing over diagnosis, but at the same time it may result in failure to recognize the incomplete form of Kawasaki Disease. Objective To suspect incomplete Kawasaki Disease, because early diagnosis and proper treatment may reduce substantial risk of developing coronary artery abnormality which is one of the leading causes of acquired heart disease in children. Method Nine cases of incomplete Kawasaki Disease were diagnosed over a period of one year. The diagnosis of incomplete Kawasaki Disease was based on fever for five days with less than four classical clinical features and cardiac abnormality detected by 2D- echocardiography. A repeat echocardiography was done after 6 weeks of onset of illness. The patients were treated with Intravenous Immunoglobulin and/or aspirin. Result The mean age of the patients was 3.83 years and the mean duration of symptoms before diagnosis was 12.1 days. Apart from other criteria all of our patients had edema and extreme irritability. All the patients had abnormal echocardiographic finding. Five patients received only aspirin due to nonaffordability of Intravenous Immunoglobulin and four patients received both aspirin and Intravenous Immunoglobulin, but the outcome was excellent in all the cases. Conclusion Incomplete Kawasaki Disease can be diagnosed with more awareness and aspirin alone may be used as a second line therapy in case of non affordability of Intravenous Immunoglobulin. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 30-35 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10234

  15. Four cases with Kawasaki disease and viral infection: aetiology or association.

    Science.gov (United States)

    Giray, Tuba; Biçer, Suat; Küçük, Öznur; Çöl, Defne; Yalvaç, Zerrin; Gürol, Yeşim; Yilmaz, Gülden; Saç, Ahmet; Mogol, Yigit

    2016-12-01

    The aetiology of Kawasaki disease has not yet been precisely determined. It has been associated with a variety of bacterial and viral agents. Some viruses including human adenovirus, coronavirus, and parainfluenza virus type 3 have been isolated from patients with Kawasaki disease. Clinical presentation of patients with human coronavirus and adenovirus infections mimics Kawasaki disease. In addition, these viruses may also be detected in Kawasaki disease as a coinfection. In this report, we present four Kawasaki disease patients infected with adenovirus, coronavirus OC43/HKU1 and parainfluenza virus type 3.

  16. The effect of TNFalpha blockade in complicated, refractory Kawasaki disease

    DEFF Research Database (Denmark)

    Stenbøg, Elisabeth; Nielsen, Bent Windelborg; Hørlyck, Arne

    2006-01-01

    In Kawasaki disease (KD), a systemic vasculitis of childhood, serum levels of proinflammatory cytokines such as tumour necrosis factor alpha (TNFalpha) are elevated during the acute phase of the disease. Although the majority of children recover completely from a single dose of intravenous...

  17. Giant coronary artery aneurysms complicating Kawasaki disease in Mexican children.

    Science.gov (United States)

    Garrido-García, Luis M; Morán-Villaseñor, Edna; Yamazaki-Nakashimada, Marco A; Cravioto, Patricia; Galván, Fernando

    2018-03-01

    One of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear. A retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of pKawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms. One of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.

  18. A case of incomplete and refractory Kawasaki disease: Diagnostic ...

    African Journals Online (AJOL)

    The diagnosis of incomplete Kawasaki disease (KD) – i.e. cases that do not full all the diagnostic criteria – requires a high index of suspicion. We report a case of incomplete KD that was refractory to treatment with intravenous immunoglobulin and subsequently responded to treatment with intravenous methylprednisolone.

  19. Recognising Kawasaki disease in UK primary care: a descriptive study using the Clinical Practice Research Datalink.

    Science.gov (United States)

    Moore, Abigail; Harnden, Anthony; Mayon-White, Richard

    2014-08-01

    Kawasaki disease is a rare childhood illness that can present non-specifically, making it a diagnostic challenge. The clinical presentation of Kawasaki disease has not been previously described in primary care. To describe how children with an eventual diagnosis of Kawasaki disease initially present to primary care in the UK. The Clinical Practice Research Datalink was used to find cases coded as Kawasaki disease. Hospital Episode Statistics, hospital admissions, and hospital outpatient attendances were used to identify the children with a convincing diagnosis of Kawasaki disease. Questionnaires and a request for copies of relevant hospital summaries, discharge letters, and reports were sent to GPs of the 104 children with a diagnosis of Kawasaki disease between 2007 and 2011. Most children presented with few clinical features typical of Kawasaki disease. Of those with just one feature, a fever or a polymorphous rash were the most common. By the time that most children were admitted to hospital they had a more recognisable syndrome, with three or more clinical features diagnostic of Kawasaki disease. Most GPs did not consider Kawasaki disease among their differential diagnoses, but some GPs did suspect that the child's illness was unusual. The study highlighted the difficulty of early diagnosis, with most children having a non-specific presentation to primary care. GPs are encouraged to implement good safety netting, and to keep Kawasaki disease in mind when children present with fever and rashes. © British Journal of General Practice 2014.

  20. Kawasaki Disease in India, Lessons Learnt Over the Last 20 Years.

    Science.gov (United States)

    Singh, Surjit; Kawasaki, Tomisaku

    2016-02-01

    Over the last 20 years, Kawasaki disease is being increasingly recognized in India and it may soon replace acute rheumatic fever to become the commonest cause of acquired heart disease amongst children. However, the vast majority of children with Kawasaki disease in India are still not being diagnosed. Diagnosis of Kawasaki disease is based on a constellation of clinical findings which have a typical temporal sequence. All pediatricians must we familiar with the nuances involved in arriving at a diagnosis of Kawasaki disease. With early diagnosis and prompt treatment, the risk of coronary artery abnormalities can be significantly reduced.

  1. Quantitative evaluation of myocardial fibrosis by cardiac integrated backscatter analysis in Kawasaki disease.

    Science.gov (United States)

    Xie, Lijian; Wang, Renjian; Huang, Min; Zhang, Yongwei; Shen, Jie; Xiao, Tingting

    2016-01-12

    Kawasaki disease is an acute, systemic vasculitis that affects the coronary arteries. However, the relationship between myocardial fibrosis and Kawasaki disease has been completely unknown until now. We aimed to provide quantitative information about myocardial fibrosis using cardiac integrated backscatter in Han race Kawasaki disease patients. Ninety Kawasaki disease patients and 90 healthy control subjects were recruited. Based on Kawasaki disease status, the patients were categorized into 3 groups: acute, subacute, and convalescence phase. Based on coronary artery status, the Kawasaki disease patients were categorized into 3 groups: without coronary artery lesions, with coronary artery dilation, and with coronary artery aneurysms. All subjects underwent two-dimensional and Doppler examinations to measure clinical echocardiographic parameters. Myocardial fibrosis was detected with calibrated integrated backscatter imaging. Left ventricle systolic functions were normal in both the Kawasaki disease and control participants. The myocardial calibrated integrated backscatter values of the left ventricles of the acute (p Kawasaki disease patients were significantly greater than those of the healthy controls. The left ventricle myocardial calibrated integrated backscatter values were significantly smaller in the Kawasaki disease patients without coronary artery lesions than in the Kawasaki disease patients with coronary artery dilations and coronary artery aneurysms in different phases. The left ventricle myocardial calibrated integrated backscatter results were positively correlated with coronary artery status in the acute (r = 0.331, p Kawasaki disease. Our findings may suggest that myocardial fibrosis occurs during early episodes of Kawasaki disease given uncertainties that exist regarding correlations of calibrated integrated backscatter and myocardial fibrosis.

  2. Enfermedad de Kawasaki:: reporte de 3 casos Kawasaki's disease:: report on 3 cases

    Directory of Open Access Journals (Sweden)

    Gladys Fernández Couce

    2010-06-01

    Full Text Available Se presentan 3 pacientes que acudieron al Hospital Pediátrico «Juan M. Márquez» con manifestaciones de exantema y fiebre. Se realizaron estudios para concluir el diagnóstico y en todos los casos los resultados fueron normales. Para arribar al diagnóstico de enfermedad de Kawasaki se aplicaron los criterios de la American Heart Association. Ninguno de los pacientes presentó complicaciones y se excluyeron otras enfermedades según el cuadro clínico y los exámenes complementarios realizados. Los 3 mejoraron con tratamiento de intacglobin en dosis de 400 mg/(kg∙día, durante 5 días.These three patients came to "Juan Manuel Márquez" with manifestations of exanthema and fever. We conducted studies to conclude the diagnosis and in all the cases results were normal. To arrive to diagnosis of Kawasaki's disease criteria from the American Heart Association were applied. No patient had complications excluding other diseases according to clinical picture and complementary examinations performed. The three patients improved with the 400 mg (kg/day Intacglobin treatment for 5 days.

  3. Preventing Long-Term Cardiac Damage in Pediatric Patients With Kawasaki Disease.

    Science.gov (United States)

    Williams, Kelly

    Kawasaki disease is currently the leading cause of long-term cardiac damage in pediatric patients in the United States. Kawasaki disease is diagnosed based on symptomatology and by ruling out other etiology. There is a significant need for an improved, standardized treatment protocol for patients diagnosed with Kawasaki disease and a more rapid initiation of treatment for these patients. Decreasing the cardiac damage caused by Kawasaki disease with timely diagnosis and treatment needs be a principal goal. Copyright © 2016 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.

  4. BCG and Kawasaki disease in Mexico and Japan.

    Science.gov (United States)

    Gamez-Gonzalez, Luisa Berenise; Hamada, Hiromichi; Llamas-Guillen, Beatriz Adriana; Ruiz-Fernandez, Miguel; Yamazaki-Nakashimada, Marco

    2017-05-04

    Dr. Tomisaku Kawasaki was the first to describe BCG reactivation in Kawasaki Disease (K D ), and this sign is present in about 30-50% of K D patients. It is a very specific early sign of the disease and although it has been recognized for decades, its pathophysiology continues to be an enigma. Recently, Yamada et al. reported a severe BCG reaction with tuberculid in 2 Japanese K D patients. We present 2 cases with K D and severe BCG reaction, one from Japan and the other from Mexico and review the policies of administration of BCG in both countries. The BCG vaccine has a worldwide coverage of 88%. Differences in BCG strains and methods of administration may influence BCG reactions in K D . The BCG reaction in the inoculation site may represent the most useful sign in K D .

  5. Natriuretic Peptides in Kawasaki Disease: the Myocardial Perspective

    Directory of Open Access Journals (Sweden)

    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.

  6. Establishment of Kawasaki disease database based on metadata standard.

    Science.gov (United States)

    Park, Yu Rang; Kim, Jae-Jung; Yoon, Young Jo; Yoon, Young-Kwang; Koo, Ha Yeong; Hong, Young Mi; Jang, Gi Young; Shin, Soo-Yong; Lee, Jong-Keuk

    2016-07-01

    Kawasaki disease (KD) is a rare disease that occurs predominantly in infants and young children. To identify KD susceptibility genes and to develop a diagnostic test, a specific therapy, or prevention method, collecting KD patients' clinical and genomic data is one of the major issues. For this purpose, Kawasaki Disease Database (KDD) was developed based on the efforts of Korean Kawasaki Disease Genetics Consortium (KKDGC). KDD is a collection of 1292 clinical data and genomic samples of 1283 patients from 13 KKDGC-participating hospitals. Each sample contains the relevant clinical data, genomic DNA and plasma samples isolated from patients' blood, omics data and KD-associated genotype data. Clinical data was collected and saved using the common data elements based on the ISO/IEC 11179 metadata standard. Two genome-wide association study data of total 482 samples and whole exome sequencing data of 12 samples were also collected. In addition, KDD includes the rare cases of KD (16 cases with family history, 46 cases with recurrence, 119 cases with intravenous immunoglobulin non-responsiveness, and 52 cases with coronary artery aneurysm). As the first public database for KD, KDD can significantly facilitate KD studies. All data in KDD can be searchable and downloadable. KDD was implemented in PHP, MySQL and Apache, with all major browsers supported.Database URL: http://www.kawasakidisease.kr. © The Author(s) 2016. Published by Oxford University Press.

  7. Blood N-terminal Pro-brain Natriuretic Peptide and Interleukin-17 for Distinguishing Incomplete Kawasaki Disease from Infectious Diseases.

    Science.gov (United States)

    Wu, Ling; Chen, Yuanling; Zhong, Shiling; Li, Yunyan; Dai, Xiahua; Di, Yazhen

    2015-06-01

    To explore the diagnostic value of blood N-terminal pro-brain natriuretic peptide (NT-proBNP) and interleukin-17(IL-17) for incomplete Kawasaki disease. Patients with Kawasaki disease, Incomplete Kawasaki disease and unclear infectious fever were included in this retrospective study. Their clinical features, and laboratory test results of blood NT-proBNP and IL-17 were collected and compared. 766 patients with complete clinical information were recruited, consisting of 291 cases of Kawasaki disease, 74 cases of incomplete Kawasaki disease, and 401 cases of unclear infectious diseases. When the consistency with indicator 2 and 3 in Kawasaki disease diagnosis criteria was assessed with blood IL-17 ?11.55 pg/mL and blood NT-proBNP ? 225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases reached 86.5% and 94.8%, respectively. When we chose the consistency with indicator 1 and 2 in Kawasaki disease diagnosis criteria, the appearance of decrustation and/or the BCG erythema, blood IL-17 ?11.55 pg/mL and blood NT-Pro BNP ?225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases was 43.2% and 100%, respectively. Blood NT-proBNP and IL-17 are useful laboratory indicators for distinguishing incomplete Kawasaki disease and infectious diseases at the early stage.

  8. Kawasaki disease in an infant: a case report

    OpenAIRE

    Karine Guimarães Lacerda; Gabriela Moreira de Toledo; Izilda das Eiras Tâmega; Bárbara Werner Griciunas; Luciana Cristina Vieira dos Santos

    2017-01-01

    Infant case report diagnosed with Kawasaki disease, a febrile vasculitis that affects mainly children between 2 and 3 years old. The causative agent is still unknown, but the epidemiology and clinical features suggest infectious character. There may be cardiac involvement and micro aneurysms in the coronary arteries. Differential diagnoses are measles, adenovirus and juvenile rheumatoid arthritis. Treatment is with intravenous immunoglobulin, salicylates and steroids. The patient, who is 13 m...

  9. Oral necrotizing microvasculitis in a patient affected by Kawasaki disease.

    Science.gov (United States)

    Scardina, Giuseppe Alessandro; Fucà, Gerlandina; Carini, Francesco; Valenza, Vincenzo; Spicola, Michele; Procaccianti, Paolo; Messina, Pietro; Maresi, Emiliano

    2007-12-01

    Kawasaki disease (KD) was first described in 1967 by Kawasaki, who defined it as "mucocutaneous lymph node syndrome". KD is an acute systemic vasculitis, which mainly involves medium calibre arteries; its origin is unknown, and it is observed in children under the age of 5, especially in their third year. The principal presentations of KD include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Within KD, oral mucositis - represented by diffuse mucous membrane erythema, lip and tongue reddening and lingual papillae hypertrophy with subsequent development of strawberry tongue - can occur both in the acute stage of the disease (0-9 days), and in the convalescence stage (>25 days) as a consequence of the pharmacological treatment. KD vascular lesions are defined as systemic vasculitis instead of systemic arteritis. This study analyzed the anatomical-pathological substrata of oral mucositis in a baby affected by Kawasaki disease and suddenly deceased for cardiac tamponade caused by coronary aneurysm rupture (sudden cardiac death of a mechanical type).

  10. 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; Rhim, Jung-Woo; Seob Song, Min; Lee, Hyoung-Doo; Kim, Dong Soo; Lee, Jae-Moo; Chang, Jeng-Sheng; Tsai, Fuu-Jen; Liang, Chi-Di; Chen, Ming-Ren; Chi, Hsin; Chiu, Nan-Chang; Huang, Fu-Yuan; Chang, Luan-Yin; Huang, Li-Min; Kuo, Ho-Chang; Huang, Kao-Pin; Lee, Meng-Luen; Hwang, Betau; Huang, Yhu-Chering; Lee, Pi-Chang; Odam, Miranda; Christiansen, Frank T.; Witt, Campbell; Goldwater, Paul; Curtis, Nigel; Palasanthiran, Pamela; Ziegler, John; Nissen, Michael; Nourse, Clare; Kuipers, Irene M.; Ottenkamp, Jaap J.; Geissler, Judy; Biezeveld, Maarten; Tacke, Carline; Filippini, Luc; Brogan, Paul; Klein, Nigel; Shah, Vanita; Dillon, Michael; Booy, Robert; Shingadia, Delane; Bose, Anu; Mukasa, Thomas; Tulloh, Robert; Michie, Colin; Newburger, Jane W.; Baker, Annette L.; Rowley, Anne H.; Shulman, Stanford T.; Mason, Wilbert; Takahashi, Masato; Melish, Marian E.; Tremoulet, Adriana H.; Viswanathan, Ananth; Rochtchina, Elena; Attia, John; Scott, Rodney; Holliday, Elizabeth; Harrap, Stephen

    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

  11. Vascular function long term after Kawasaki disease: another piece of the puzzle?

    Science.gov (United States)

    Pinto, Fátima F; Gomes, Inês; Loureiro, Petra; Laranjo, Sérgio; Timóteo, Ana T; Carmo, Miguel M

    2017-04-01

    Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. Thus far, endothelial dysfunction in patients with no coronary lesions is poorly understood. Our aim was to access the vascular function in adolescents and young adults long term after Kawasaki disease, but without coronary aneurysms or any other cardiac risk factors. We carried out a single-centre prospective study in a Portuguese population. We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed >5 years ago, with no coronary lesions or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically assessed. Endo-PAT and carotid intima-media thickness assessment were performed to determine vascular function. In total, 43 Kawasaki disease patients were assessed and compared with 43 controls. Kawasaki disease patients presented a decreased reactive hyperaemia index compared with controls (1.59±0.45 versus 1.98±0.41; pKawasaki disease group. There were no statistically significant changes with regard to laboratory data. Children with Kawasaki disease may have long-term sequelae, even when there is no discernible coronary artery involvement in the acute stage of the disease. Further research is needed to assess whether known strategies to improve endothelial function would bring potential benefits to Kawasaki disease patients.

  12. Possible involvement of infection with human coronavirus 229E, but not NL63, in Kawasaki disease.

    Science.gov (United States)

    Shirato, Kazuya; Imada, Yoshio; Kawase, Miyuki; Nakagaki, Keiko; Matsuyama, Shutoku; Taguchi, Fumihiro

    2014-12-01

    Although human coronavirus (HCoV)-NL63 was once considered a possible causative agent of Kawasaki disease based on RT-PCR analyses, subsequent studies could not confirm the result. In this study, this possibility was explored using serological tests. To evaluate the role of HCoV infection in patients with Kawasaki disease, immunofluorescence assays and virus neutralizing tests were performed. Paired serum samples were obtained from patients with Kawasaki disease who had not been treated with γ-globulin. HCoV-NL63 and two antigenically different isolates of HCoV-229E (ATCC-VR740 and a new isolate, Sendai-H) were examined as controls. Immunofluorescence assays detected no difference in HCoV-NL63 antibody positivity between the patients with Kawasaki disease and controls, whereas the rate of HCoV-229E antibody positivity was higher in the patients with Kawasaki disease than that in controls. The neutralizing tests revealed no difference in seropositivity between the acute and recovery phases of patients with Kawasaki disease for the two HCoV-229Es. However, the Kawasaki disease specimens obtained from patients in recovery phase displayed significantly higher positivity for Sendai-H, but not for ATCC-VR740, as compared to the controls. The serological test supported no involvement of HCoV-NL63 but suggested the possible involvement of HCoV-229E in the development of Kawasaki disease. © 2014 Wiley Periodicals, Inc.

  13. The Complexities of the Diagnosis and Management of Kawasaki Disease.

    Science.gov (United States)

    Rowley, Anne H

    2015-09-01

    Kawasaki disease (KD) must be considered in the differential diagnosis of any child with fever for 4 to 5 days and compatible clinical and laboratory features, and in any infant with prolonged fever and compatible laboratory features, even in the absence of the classic clinical signs. Prompt therapy is required, because delayed or unrecognized KD can lead to lifelong heart disease or death in previously healthy children. Most children with KD respond to a single 2 g/kg dose of intravenous gammaglobulin with oral aspirin, but a small subset require additional therapies to resolve the clinical illness. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Childhood vaccines and Kawasaki disease, Vaccine Safety Datalink, 1996-2006.

    Science.gov (United States)

    Abrams, Joseph Y; Weintraub, Eric S; Baggs, James M; McCarthy, Natalie L; Schonberger, Lawrence B; Lee, Grace M; Klein, Nicola P; Belongia, Edward A; Jackson, Michael L; Naleway, Allison L; Nordin, James D; Hambidge, Simon J; Belay, Ermias D

    2015-01-03

    Kawasaki disease is a childhood vascular disorder of unknown etiology. Concerns have been raised about vaccinations being a potential risk factor for Kawasaki disease. Data from the Vaccine Safety Datalink were collected on children aged 0-6 years at seven managed care organizations across the United States. Defining exposure as one of several time periods up to 42 days after vaccination, we conducted Poisson regressions controlling for age, sex, season, and managed care organization to determine if rates of physician-diagnosed and verified Kawasaki disease were elevated following vaccination compared to rates during all unexposed periods. We also performed case-crossover analyses to control for unmeasured confounding. A total of 1,721,186 children aged 0-6 years from seven managed care organizations were followed for a combined 4,417,766 person-years. The rate of verified Kawasaki disease was significantly lower during the 1-42 days after vaccination (rate ratio=0.50, 95% CL=0.27-0.92) and 8-42 days after vaccination (rate ratio=0.45, 95% CL=0.22-0.90) compared to rates during unexposed periods. Breaking down the analysis by vaccination category did not identify a subset of vaccines which was solely responsible for this association. The case-crossover analyses revealed that children with Kawasaki disease had lower rates of vaccination in the 42 days prior to symptom onset for both physician-diagnosed Kawasaki disease (rate ratio=0.79, 95% CL=0.64-0.97) and verified Kawasaki disease (rate ratio=0.38, 95% CL=0.20-0.75). Childhood vaccinations' studied did not increase the risk of Kawasaki disease; conversely, vaccination was associated with a transient decrease in Kawasaki disease incidence. Verifying and understanding this potential protective effect could yield clues to the underlying etiology of Kawasaki disease. Copyright © 2014. Published by Elsevier Ltd.

  15. A diagnostic algorithm combining clinical and molecular data distinguishes Kawasaki disease from other febrile illnesses

    Directory of Open Access Journals (Sweden)

    Ling Xuefeng B

    2011-12-01

    Full Text Available Abstract Background Kawasaki disease is an acute vasculitis of infants and young children that is recognized through a constellation of clinical signs that can mimic other benign conditions of childhood. The etiology remains unknown and there is no specific laboratory-based test to identify patients with Kawasaki disease. Treatment to prevent the complication of coronary artery aneurysms is most effective if administered early in the course of the illness. We sought to develop a diagnostic algorithm to help clinicians distinguish Kawasaki disease patients from febrile controls to allow timely initiation of treatment. Methods Urine peptidome profiling and whole blood cell type-specific gene expression analyses were integrated with clinical multivariate analysis to improve differentiation of Kawasaki disease subjects from febrile controls. Results Comparative analyses of multidimensional protein identification using 23 pooled Kawasaki disease and 23 pooled febrile control urine peptide samples revealed 139 candidate markers, of which 13 were confirmed (area under the receiver operating characteristic curve (ROC AUC 0.919 in an independent cohort of 30 Kawasaki disease and 30 febrile control urine peptidomes. Cell type-specific analysis of microarrays (csSAM on 26 Kawasaki disease and 13 febrile control whole blood samples revealed a 32-lymphocyte-specific-gene panel (ROC AUC 0.969. The integration of the urine/blood based biomarker panels and a multivariate analysis of 7 clinical parameters (ROC AUC 0.803 effectively stratified 441 Kawasaki disease and 342 febrile control subjects to diagnose Kawasaki disease. Conclusions A hybrid approach using a multi-step diagnostic algorithm integrating both clinical and molecular findings was successful in differentiating children with acute Kawasaki disease from febrile controls.

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

    Science.gov (United States)

    Srinivasan, Ranjini; Weller, Rachel; Einstein, Andrew J.

    2016-01-01

    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. PMID:27872783

  17. Prevalence and predictors of coronary artery disease in adults with Kawasaki disease.

    Science.gov (United States)

    Garg, Jalaj; Krishnamoorthy, Parasuram; Palaniswamy, Chandrasekar; Paudel, Rajiv; Chatterjee, Saurav; Ahmad, Hasan; Snyder, Christopher

    2015-08-01

    Accelerated coronary atherosclerosis in patients with Kawasaki disease, in conjunction with coronary artery aneurysm and stenosis that characterise this disease, are potential risk factors for developing coronary artery disease in young adults. We aimed to determine the prevalence and predictors of coronary artery disease in adult patients with Kawasaki disease. All patients aged 18-55 years of age diagnosed with Kawasaki disease were sampled from Nationwide Inpatient Sample database using International Classification of Diseases 9th revision (ICD 9 code 446.1) from 2009 to 2010. Demographics, prevalence of coronary artery disease, and other traditional risk factors in adult patients with Kawasaki disease were analysed using ICD 9 codes. The prevalence of Kawasaki disease among adults was 0.0005% (n=215) of all in-hospital admissions in United States. The mean age was 27.3 years with women (27.6 years) older than men (27.1 years). Traditional risk factors were hypertension (21%), hyperlipidaemia (15.6%), diabetes (11.5%), tobacco use (8.8%), and obesity (8.8%), with no significant difference between men and women. Coronary artery disease (32.4%), however, was more prevalent in men (44.7%) than in women (12.1%; p=0.03). In multivariate regression analysis, after adjusting for demographics and traditional risk factors, hypertension (OR=13.2, p=0.03) was an independent risk factor of coronary artery disease. There was increased preponderance of coronary artery disease in men with Kawasaki disease. On multivariate analysis, hypertension was found to be the only independent predictor of coronary artery disease in this population after adjusting for other risk factors.

  18. Evidence-based management of Kawasaki disease in the emergency department.

    Science.gov (United States)

    Seaton, Kara K; Kharbanda, Anupam

    2015-01-01

    Kawasaki disease, also known as mucocutaneous lymph node syndrome, was first described in Japan in 1967. It is currently the leading cause of acquired heart disease in children in the United States. Untreated Kawasaki disease may lead to the formation of coronary artery aneurysms and sudden cardiac death in children. This vasculitis presents with fever for ≥ 5 days, plus a combination of key criteria. Because each of the symptoms commonly occurs in other childhood illnesses, the disease can be difficult to diagnose, especially in children who present with an incomplete form of the disease. At this time, the etiology of the disease remains unknown, and there is no single diagnostic test to confirm the diagnosis. This issue reviews the presentation, diagnostic criteria, and management of Kawasaki disease in the emergency department. Emergency clinicians should consider Kawasaki disease as a diagnosis in pediatric patients presenting with prolonged fever, as prompt evaluation and management can significantly decrease the risk of serious cardiac sequelae.

  19. Kawasaki disease in an infant: a case report

    Directory of Open Access Journals (Sweden)

    Karine Guimarães Lacerda

    2017-06-01

    Full Text Available Infant case report diagnosed with Kawasaki disease, a febrile vasculitis that affects mainly children between 2 and 3 years old. The causative agent is still unknown, but the epidemiology and clinical features suggest infectious character. There may be cardiac involvement and micro aneurysms in the coronary arteries. Differential diagnoses are measles, adenovirus and juvenile rheumatoid arthritis. Treatment is with intravenous immunoglobulin, salicylates and steroids. The patient, who is 13 months old, previously healthy, first presented high fever and runny nose eye congestion, treated with symptomatic medication; three days later she evolved with hyperemia frame in the face, trunk and limbs, again treated with symptomatic and antihistamine. After two days, there was swelling in the face, hands and feet, diffuse hyperemia, oral erythema and fever, and she was referred to a hospital for investigation. She presented all the criteria for Kawasaki disease: high fever, eye congestion, alterations in the mouth, polymorph rash, edema in the extremities and acute cervical lymphadenopathy. She was treated with intravenous immuglobulin and salicylates. After leaving hospital, she coursed with fever, swelling and palmoplantar desquamation. She was hospitalized and intravenous immunoglobulin, salicylates and corticosteroids were reintroduced; she progressed well and was discharged in two days. During her hospitalization, the echocardiogram ruled out heart problems.

  20. Comorbidity of Kawasaki disease and group A streptococcal pleural effusion in a healthy child: a case report

    Directory of Open Access Journals (Sweden)

    Alhammadi AH

    2013-07-01

    Full Text Available Ahmed H Alhammadi, Mohamed A HendausGeneral Pediatrics Section, Department of Pediatrics, Hamad Medical Corporation, Doha, QatarBackground: Kawasaki disease is an acute self-limiting vasculitis that affects children. The most dreaded complication of Kawasaki disease reported in the literature over the years is coronary artery disease, which is considered as the main cause of acquired heart disease. However, pulmonary associations with Kawasaki disease have been overlooked. We present a rare, if not unique, case of Kawasaki disease associated with group A streptococcus pleural effusion in the English language literature. A search of the PubMed database was carried out, using a combination of the terms “Kawasaki disease”, “pneumonia”, and “group A streptococcus”. The majority of studies conducted in children with Kawasaki disease have concentrated on the coronary artery implications. Kawasaki disease is considered a self-limiting illness, but can have detrimental consequences if not diagnosed early. When there is a prolonged inflammatory reaction, with no infectious agent identified or remittent fever unresponsive to antibiotics, Kawasaki disease should be taken into consideration. Elevated Vβ2+ T cells compared with healthy controls suggest possible involvement of a superantigen in the etiology of Kawasaki disease, so it is wise that the health care provider concentrates not only on the cardiac consequences, but also on pulmonary associations.Keywords: Kawasaki disease, pneumonia, group A streptococcus

  1. [Clinical features and course of Kawasaki disease in central Tunisia: a study about 14 cases collected over a period of three years (2000-2002)].

    Science.gov (United States)

    Chemli, Jalel; Kchaou, Habib; Amri, Fethi; Belkadhi, Adel; Essoussi, Ahmed Sahloul; Gueddiche, Neji; Harbi, Abdelaziz

    2005-08-01

    To analyze the clinical features and course of Kawasaki disease in central Tunisia. We studied retrospectively 14 cases of children with Kawasaki disease collected in tunisian center during three years (2000-2002). The study is about 11 boys and 3 girls (sex - ratio: 3.6/1) aged from 6 months to 8 years (mean age : 4 years). Twelve patients had at least 5 diagnostic criteria of the illness, the two others had an incomplete form. We noted cardiac complications in seven patients treated belatedly, beyond 10 days of progression, because of atypical clinical presentations. All patients had all a middle caliber coronary aneurysm that was complicated by a thrombus in three cases, associated with pericarditis and minimal mitral insufficiency in a case and with a cardiac rhythm disturbance (block of branch) in another case. Besides the cardiac complications, several other visceral manifestation could be noted: joint symptoms in five cases, GI tract symptomes in three cases, neuro-meningeal in two cases and urinary trad symptomes in two other cases. Specific treatment (aspirin with antiinflammatory dose and intravenous immune globulin (IVIG)) has been instituted in all patients. The course was favorable for 12 patients with fast regression of clinical manifestation and progressive normalisation of biologic values. Two patients did not respond to the initial IVIG treatment, and had to recense received an additional course of IGIV but without clinical nor biological improvement. These two patients were treated with corticosteroids. Cardiac lesions disappeared completely in all patients even for those with thrombosis and in patients with IVIG-resistant Kawasaki disease. Only one patient had kept neurologic sequellae: aphasia, bevavioral problemes and partial epilepsy. Kawasaki disease is not rare in our region. Incomplete or atypical presentations are frequent and are a source of diagnostic delay. Coronary aneurysm due to the delay of treatment often regresses even in patients

  2. An Adult Case of Kawasaki Disease in a Pregnant Japanese Woman: A Case Report

    Directory of Open Access Journals (Sweden)

    Kyoko Kanno

    2011-04-01

    Full Text Available Kawasaki disease is an acute febrile disease predominantly seen in young children. We report a case of Kawasaki disease in a 32-year-old pregnant woman. She developed a generalized erythematous skin rash accompanied by high fever. Bilateral conjunctival congestion, tender cervical lymphadenopathy, an edematous lower lip and peripheral edema followed by desquamation were observed. She was successfully treated with aspirin and intravenous gammaglobulin (1 g/kg/day. Her course was not complicated by coronary artery aneurysm and she delivered a healthy baby. To the best of our knowledge, this is the first case of Kawasaki disease in a pregnant woman. We suggest that Kawasaki disease should be included in the differential diagnosis of a generalized, erythematous skin rash accompanied by high fever in adults.

  3. Common variants in CASP3 confer susceptibility to Kawasaki disease.

    Science.gov (United States)

    Onouchi, Yoshihiro; Ozaki, Kouichi; Buns, Jane C; Shimizu, Chisato; Hamada, Hiromichi; Honda, Takafumi; Terai, Masaru; Honda, Akihito; Takeuchi, Takashi; Shibuta, Shoichi; Suenaga, Tomohiro; Suzuki, Hiroyuki; Higashi, Kouji; Yasukawa, Kumi; Suzuki, Yoichi; Sasago, Kumiko; Kemmotsu, Yasushi; Takatsuki, Shinichi; Saji, Tsutomu; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Kishi, Fumio; Ouchi, Kazunobu; Sato, Yoshitake; Newburger, Jane W; Baker, Annette L; Shulman, Stanford T; Rowley, Anne H; Yashiro, Mayumi; Nakamura, Yoshikazu; Wakui, Keiko; Fukushima, Yoshimitsu; Fujino, Akihiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Hata, Akira; Nakamura, Yusuke; Tanaka, Toshihiro

    2010-07-15

    Kawasaki disease (KD; OMIM 611775) is an acute vasculitis syndrome which predominantly affects small- and medium-sized arteries of infants and children. Epidemiological data suggest that host genetics underlie the disease pathogenesis. Here we report that multiple variants in the caspase-3 gene (CASP3) that are in linkage disequilibrium confer susceptibility to KD in both Japanese and US subjects of European ancestry. We found that a G to A substitution of one commonly associated SNP located in the 5' untranslated region of CASP3 (rs72689236; P = 4.2 x 10(-8) in the Japanese and P = 3.7 x 10(-3) in the European Americans) abolished binding of nuclear factor of activated T cells to the DNA sequence surrounding the SNP. Our findings suggest that altered CASP3 expression in immune effecter cells influences susceptibility to KD.

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

    Science.gov (United States)

    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.

  5. The serum concentration of soluble interleukin-2 receptor in patients with Kawasaki disease.

    Science.gov (United States)

    Teraura, Hiroyuki; Kotani, Kazuhiko; Minami, Takaomi; Takeshima, Taro; Shimooki, Osamu; Kajii, Eiji

    2017-03-01

    Kawasaki disease is a febrile disease of childhood that is associated with increased inflammatory cytokines and immunoregulatory abnormalities. While the serum concentrations of soluble IL-2 receptor can change under such pathologies, the relevance of the soluble IL-2 receptor concentration in patients with Kawasaki disease has not been specified. We aimed to summarize the existing studies that reported the soluble IL-2 receptor concentrations in patients with Kawasaki disease. Original articles that were published up to July 2016 were collected using a PubMed/Medline-based search engine. A total of nine articles that reported the serum soluble IL-2 receptor concentrations in acute-phase Kawasaki disease were eligible. All of the articles described a high soluble IL-2 receptor concentration in patients with Kawasaki disease relative to the level of controls or the reference range. Two of five articles on patients with coronary artery aneurysms described a significantly higher soluble IL-2 receptor concentration in patients with coronary artery aneurysms than patients without. Two articles on patients with intravenous immunoglobulin therapy described a significant decrease of the soluble IL-2 receptor concentration after the therapy. Accordingly, the serum soluble IL-2 receptor can be a potent marker of disease activity and therapeutic effects in patients with Kawasaki disease; further studies are thus warranted for its use in the clinical setting.

  6. An update on Kawasaki disease II: clinical features, diagnosis, treatment and outcomes.

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    Yim, Deane; Curtis, Nigel; Cheung, Michael; Burgner, David

    2013-08-01

    This is the second of two updates on Kawasaki disease. The first review focused on epidemiology and aetio-pathogenesis. Here, we review the clinical features and diagnosis of Kawasaki disease, as well as recent evidence on treatment, follow-up and cardiovascular outcomes. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  7. Dissecting Kawasaki disease: a state-of-the-art review.

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    Dietz, S M; van Stijn, D; Burgner, D; Levin, M; Kuipers, I M; Hutten, B A; Kuijpers, T W

    2017-08-01

    Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. The diagnosis is based on the presence of persistent fever and clinical features including exanthema, lymphadenopathy, conjunctival injection, and changes to the mucosae and extremities. Although the etiology remains unknown, the current consensus is that it is likely caused by an (infectious) trigger initiating an abnormal immune response in genetically predisposed children. Treatment consists of high dose intravenous immunoglobulin (IVIG) and is directed at preventing the development of CAA. Unfortunately, 10-20% of all patients fail to respond to IVIG and these children need additional anti-inflammatory treatment. Coronary artery lesions are diagnosed by echocardiography in the acute and subacute phases. Both absolute arterial diameters and z-scores, adjusted for height and weight, are used as criteria for CAA. Close monitoring of CAA is important as ischemic symptoms or myocardial infarction due to thrombosis or stenosis can occur. These complications are most likely to arise in the largest, so-called giant CAA. Apart from the presence of CAA, it is unclear whether KD causes an increased cardiovascular risk due to the vasculitis itself. Many aspects of KD remain unknown, although there is growing knowledge on the etiology, treatment, and development and classification of CAA. Since children with previous KD are entering adulthood, long-term follow-up is increasingly important. What is known: • Kawasaki disease (KD) is a pediatric vasculitis with coronary artery damage as its main complication. • Although KD approaches its 50th birthday since its first description, many aspects of the disease remain poorly understood. What is new: • In recent years, multiple genetic candidate pathways involved in KD have been identified, with recently promising information about the ITPKC pathway. • As increasing numbers of KD patients are reaching adulthood

  8. 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.

  9. Kawasaki disease incidence in children and adolescents: an observational study in primary care.

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    Hall, Gillian C; Tulloh, Louise E; Tulloh, Robert M R

    2016-04-01

    Kawasaki disease is reported to be increasing in incidence and is the commonest childhood cause of acquired heart disease in the Western world. To determine the current UK incidence of Kawasaki disease across childhood and adolescence; and investigate trends over time and season. An observational, descriptive study in the UK. The Health Improvement Network (THIN) database of primary healthcare records was searched for codes or text indicating Kawasaki disease. Identified records were compared with a study case definition and a date of onset was assigned to cases. The incidence, age/sex distribution, and trend in seasonal and temporal distribution were estimated (2008-2012). A total of 110 episodes of Kawasaki disease in 109 children were identified from 3.9 million person-years observation. The incidence of Kawasaki disease was 2.8 per 100 000 person-years (95% confidence interval [CI] = 2.3 to 3.4) when aged Kawasaki disease remains low and has stabilised in the UK, GPs should recognise that the condition occurs throughout childhood and across the seasons. © British Journal of General Practice 2016.

  10. Markers of a recent bocavirus infection in children with Kawasaki disease: "a year prospective study".

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    Bajolle, F; Meritet, J-F; Rozenberg, F; Chalumeau, M; Bonnet, D; Gendrel, D; Lebon, P

    2014-12-01

    Retrospective studies and case-reports have suggested the possible role of various viruses in the pathogenesis of the Kawasaki disease. To determine prospectively the incidence of Kawasaki diseases associated with a recent bocavirus infection in the course of a year. Thirty-two children with Kawasaki disease were enrolled in a 13 months prospective study to assess the frequency of human bocavirus type 1 infections. Seasonal shedding of virus, markers of recent infection such as viraemia, viral load, and serum interferon alpha were analyzed. Three of 32 (9%) children had HBoV-DNA in the serum suggesting a recent infection. HBoV-DNA was detected in naso-pharyngeal aspiration of 7/32 (21.8%) children with Kawasaki Disease and six of them (18%) had an increased viral load. No common respiratory viruses were isolated from the 32 patients with the exception of one adenovirus. The seven bocaviruses were identified during the winter-spring season. In addition, 4 of 7 of Kawasaki disease patients shedding bocavirus had detectable interferon alpha in the blood, indicating a possible active or recent viral infection. This study shows that a recent bocavirus infection is concomitant with the onset of some cases of Kawasaki disease. Bocavirus may be a cofactor in the pathogenesis of this disease as previously reported for other infectious agents. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  11. [Brain hemorrhage in a patient with Kawasaki disease].

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    Yamazaki-Nakashimada, Marco Antonio; Rivas-Larrauri, Francisco; Alcántara-Salinas, Adriana; Hernández-Bautista, Victor; Rodríguez-Lozano, Ana Luisa

    2013-01-01

    Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.

  12. The immunomodulatory effects of intravenous immunoglobulin therapy in Kawasaki disease

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    Burns, Jane C.; Franco, Alessandra

    2016-01-01

    Summary The introduction of intravenous immunoglobulin (IVIG) for modulation of inflammation in acute Kawasaki disease (KD) was a great therapeutic triumph. However, three decades later, the mechanisms underlying immune regulation by IVIG are only beginning to be revealed. Stimulation of an immature myeloid population of dendritic cells (DC) that secretes IL-10 and the elucidation of Fc-specific, HLA-restricted natural regulatory T cells (Treg) provide insights into mechanisms of IVIG. Other potential mechanisms include provision of agent-specific neutralizing antibody, anti-idiotype and anti-cytokine antibodies, blockade of activating Fcγ receptors, and stimulation of the inhibitory FcγRIIb receptor. New initiatives must seek to understand the mechanisms of IVIG in order to one day replace it with more affordable and more targeted therapies. PMID:26099344

  13. Revisiting the role of steroids and aspirin in the management of acute Kawasaki disease.

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    Dhanrajani, Anita; Yeung, Rae S M

    2017-09-01

    Kawasaki disease is an acute multisystem childhood vasculitis with a predilection for the coronary arteries. The role of corticosteroids and acetyl salicylic acid (ASA) in the treatment of acute Kawasaki disease are matters of ongoing debate and changing attitudes from one extreme to the other. Recent work has provided new evidence to guide our thinking about these two therapeutic agents, which will be the focus of this review. Corticosteroids are effective and well tolerated in Kawasaki disease, both as initial adjunctive treatment in those at high-risk for poor outcome, and as rescue therapy after failed intravenous immunoglobulin (IVIG).Higher doses of ASA (> 30 mg/kg/day) in the acute phase of Kawasaki disease, have no clear benefit over antiplatelet doses in improving coronary outcome. Corticosteroids should be used in patients at high-risk for poor coronary outcome, and in patients who fail IVIG. The absence of widely applicable and validated risk-scoring systems in Kawasaki disease outside of Japan remains a limiting factor to identify high-risk children. Current evidence does not demonstrate any advantage of high-dose over low-dose ASA in the acute phase of Kawasaki disease, in preventing coronary artery aneurysms.

  14. Aspirin dose in Kawasaki disease - the ongoing battle.

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    Dhanrajani, Anita; Chan, Mercedes; Pau, Stephanie; Ellsworth, Janet; Petty, Ross; Guzman, Jaime

    2017-12-29

    Kawasaki disease (KD) is an acute childhood vasculitis that may result in coronary aneurysms. Treatment of Kawasaki disease (KD) with a single infusion of 2g/kg intravenous immunoglobulin (IVIG) is well established, but acetyl-salicylic acid (ASA) dose remains controversial. We reviewed charts of patients with KD from two Canadian centres to assess the impact of ASA dose on IVIG resistance (operationally defined as administration of a second dose of IVIG). Both centres used standard IVIG dosing, but centre 1 used low-dose ASA from diagnosis (3-5 mg/kg/day) while centre 2 used initial high-dose ASA (80-100 mg/kg/day). Primary: To determine the difference in the incidence of IVIG resistance between the two centres. Secondary: To compare the duration of hospital stay and the incidence of CAA. There were no significant differences in baseline characteristics between the two centres. Re-treatment with a second dose of IVIG was required in 28/122 (23%) patients treated with low-dose ASA, and in 11/127 (8.7%) patients treated with high-dose ASA (p=0.003). After adjusting for confounders, low-dose ASA was associated with higher odds of IVIG resistance (OR=3.2, 95% CI 1.1, 9.1). The mean duration of hospital stay was 4.1 and 4.7 days, respectively (p= 0.37). Coronary artery aneurysms were seen in 2/117 and 6/125 patients from centre 1 and 2, respectively (p= 0.28). Low-dose ASA was associated with 3 times higher odds of IVIG re-treatment compared to high-dose ASA, with no significant difference in duration of hospital stay or incidence of coronary artery aneurysms. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  15. Comorbidity of Kawasaki disease and group a streptococcal pleural effusion in a healthy child: a case report.

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    Alhammadi, Ahmed H; Hendaus, Mohamed A

    2013-01-01

    Kawasaki disease is an acute self-limiting vasculitis that affects children. The most dreaded complication of Kawasaki disease reported in the literature over the years is coronary artery disease, which is considered as the main cause of acquired heart disease. However, pulmonary associations with Kawasaki disease have been overlooked. We present a rare, if not unique, case of Kawasaki disease associated with group A streptococcus pleural effusion in the English language literature. A search of the PubMed database was carried out, using a combination of the terms "Kawasaki disease", "pneumonia", and "group A streptococcus". The majority of studies conducted in children with Kawasaki disease have concentrated on the coronary artery implications. Kawasaki disease is considered a self-limiting illness, but can have detrimental consequences if not diagnosed early. When there is a prolonged inflammatory reaction, with no infectious agent identified or remittent fever unresponsive to antibiotics, Kawasaki disease should be taken into consideration. Elevated Vβ2+ T cells compared with healthy controls suggest possible involvement of a superantigen in the etiology of Kawasaki disease, so it is wise that the health care provider concentrates not only on the cardiac consequences, but also on pulmonary associations.

  16. Lymph-node-first presentation of Kawasaki disease compared with bacterial cervical adenitis and typical Kawasaki disease.

    Science.gov (United States)

    Kanegaye, John T; Van Cott, Elizabeth; Tremoulet, Adriana H; Salgado, Andrea; Shimizu, Chisato; Kruk, Peter; Hauschildt, John; Sun, Xiaoying; Jain, Sonia; Burns, Jane C

    2013-06-01

    To identify characteristics differentiating the node-first presentation of Kawasaki disease (NFKD) from bacterial cervical lymphadenitis (BCL) and typical Kawasaki disease (KD). From our prospectively collected database, we compared clinical, laboratory, and imaging characteristics of NFKD and BCL cohorts and performed multivariable logistic regression to identify variables that distinguish NFKD from BCL. We then compared outcomes of patients with NFKD and patients with typical KD treated during the same period. Over 7 years, 57 patients were hospitalized for NFKD, 78 for BCL, and 287 for typical KD. Patients with NFKD were older and had more medical encounters and longer duration of illness before the correct diagnosis was made than did patients with BCL. Of patients with NFKD, 33% had an admission diagnosis of bacterial adenitis or abscess. Compared with patients with BCL, patients with NFKD had lower leukocyte (white blood cell), hemoglobin, and platelet counts and higher absolute band counts (ABCs), C-reactive protein (CRP), alanine transaminase and γ-glutamyl transpeptidase levels, and erythrocyte sedimentation rates. In the multivariable analysis, smaller nodes, lower white blood cell count, and higher ABC and CRP were independently associated with NFKD. Patients with NFKD had multiple enlarged solid nodes and comparable rates of retropharyngeal edema. Compared with patients with typical KD, patients with NFKD were older, had more severe inflammation, and had similar rates of coronary artery abnormalities and resistance to intravenous immune globulin. High ABC and CRP values and multiple enlarged solid nodes in febrile patients with cervical adenopathy should prompt consideration of NFKD to prevent delayed diagnosis of KD. Retropharyngeal edema on radiography should not dissuade from the diagnosis of NFKD. Copyright © 2013 Mosby, Inc. All rights reserved.

  17. 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.

  18. Cardiac complications in children with Kawasaki disease in our own experience.

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    Gowin, Ewelina; Małecka, Ilona; Stryczyńska-Kazubska, Joanna; Michalak, Michał; Wysocki, Jacek; Górzna-Kamińska, Hanna

    2016-01-01

    Kawasaki disease is the most common cause of acquired heart disease in children in developed countries. The incidence of Kawasaki disease varies from 180 in Japan through 20 in United States to 5-8 in the European countries per 100,000 children younger than 5 years of age. To evaluate cardiac complications in children hospitalised with Kawasaki disease. Retrospective analysis of the medical records of patients hospitalised with Kawasaki disease in the Specialist Mother and Child Healthcare Facility in Poznan (Poland) in 2008-2014. The diagnosis was based on the American Heart Association criteria. Study group included 30 patients (25 boys and 5 girls). The mean age was 49 months; 21 (70%) children were younger than 5 years of age. All patients had oral mucosal lesions, while an elevated leukocyte count was observed in a minority of patients. Cardiac involvement was detected in 18 (60%) patients, aneurysms in 4 (13.3%) patients, coronary artery dilatation in 6 patients, pericarditis in 6 patients, mitral regurgitation in 3 patients, and aortic regurgitation in 2 patients. In 5 children, more than 1 cardiac abnormality was detected. During 12 months of follow-up, coronary artery dilatation resolved in 5 children, and 1 patient developed aneurysm. Our findings suggest that Kawasaki disease should be considered in the differential diagnosis of children with prolonged fever. During the acute stage of the disease, children with Kawasaki disease require regular cardiac evaluation, and long-term care is needed when cardiovascular complications occur. A central case reporting system to monitor all cases of Kawasaki disease in the Polish paediatric population should be introduced.

  19. Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study.

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    Wu, Mei-Hwan; Lin, Min-Tai; Chen, Hui-Chi; Kao, Feng-Yu; Huang, San-Kuei

    2017-01-01

    To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third-highest incidence of Kawasaki disease worldwide. We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database. Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1-4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%. The postnatal risk of Kawasaki disease was 3‰-4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Comportamiento clínico-epidemiológico de la enfermedad de Kawasaki Clinical-epidemiological behavior of Kawasaky' disease

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    Milagros Morales Leiva

    2011-06-01

    Full Text Available INTRODUCCIÓN. La enfermedad de Kawasaki es una vasculitis sistémica, aguda, febril, de evolución autolimitada, con alto riesgo de secuelas en niños menores de 5 años, si no se diagnostica y trata en su estadio inicial. Fue objetivo de este estudio describir el comportamiento clínico-epidemiológico de la enfermedad de Kawasaki en la infancia. MÉTODOS. Se realizó un estudio descriptivo, de aspectos clínicos y epidemiológicos, de los pacientes con diagnóstico de enfermedad de Kawasaki atendidos en el Hospital Pediátrico «William Soler» entre enero del 2002 y diciembre del 2009. Las variables estudiadas fueron edad, sexo, manifestaciones clínicas, fecha de inicio de los síntomas y de diagnóstico, exámenes complementarios y tratamiento, y respuesta al tratamiento. RESULTADOS. El 100 % de los pacientes eran menores de 8 años. El 57,1 % fueron del sexo masculino. La enfermedad se observó en mayor frecuencia en la época de sequía. En el 100 % de los pacientes se documentó fiebre de más de 5 días de evolución, inyección conjuntival, lesiones orofaríngeas y eritema palmar y plantar; en el 85,7 % de los pacientes se halló exantema polimorfo y en el 57,1 %, adenopatía única cervical. La eritrosedimentación se observó elevada en el 100 % de los casos y en el 85,7 % se determinó anemia. Tres pacientes (42,8 % presentaron alteraciones coronarias. Todos los pacientes fueron tratados con inmunoglobulina endovenosa (Intacglobin y aspirina; en 3 de los casos fue necesario administrar una segunda dosis de inmunoglobulina endovenosa.INTRODUCTION. The Kawasaki's disease is a systemic, acute, febrile of auto-course vasculitis with a high risk of sequelae in children under 5 years old if it is not diagnosed and treated in its early stage. The aim of present study was to describe the clinical-epidemiological behavior of the Kawasaki's disease in childhood. METHODS. A descriptive study was conducted of the clinical and epidemiological

  1. 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.

  2. 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 at...... 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.......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...

  3. Absence of association of FCGR2A gene polymorphism rs1801274 with Kawasaki disease in Greek patients.

    Science.gov (United States)

    Chatzikyriakidou, Anthoula; Aidinidou, Louiza; Giannopoulos, Andreas; Papadopoulou-Legbelou, Kyriaki; Kalinderi, Kallirhoe; Fidani, Liana

    2015-04-01

    Kawasaki disease is an acute, febrile syndrome in infancy, characterised by vasculitis of medium-sized arteries, and affects predominantly young children. Family-based studies on Kawasaki disease supports the contribution of genetic factors in disorder manifestation. In a recent genome-wide association study, the polymorphism rs1801274 of FCGR2A [Fc fragment of immunoglobulin G, low-affinity IIa, receptor] gene has been implicated in disease pathogenesis. The aim of the present study was to explore the association of this variant, for the first time, in a group of Kawasaki-diseased patients of Greek origin. A total of 47 Kawasaki-diseased children and 50 control subjects were enrolled in the study. Polymerase chain reaction-restriction fragment length polymorphism assay was performed in rs1801274 genotyping. No association was observed between this polymorphism genotypes' or alleles' distribution between Kawasaki-diseased patients and controls. Furthermore, no association was revealed between this polymorphism and cardiovascular complications in Kawasaki-diseased patients. In the literature, the reported data over this polymorphism association with Kawasaki disease in Caucasian patients are contradictory. In addition, the disease shows low prevalence in the Caucasian populations. Therefore, the independent genetic association studies on rs1801274 with Kawasaki disease in various Caucasian groups increase the amount of genetic data, which could be used in a future meta-analysis, increasing the statistical power of the resultant conclusions.

  4. [Predicting value of thrombospondin-2 for coronary artery dilatation in patients with Kawasaki disease].

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    Song, R X; Li, X H; Zhang, T; Cui, X D; Fu, J

    2016-04-01

    To investigate the predictive value of thrombospondin-2 in children with coronary artery dilatation secondary to Kawasaki disease. This study was a retrospective controlled study, the patients were divided into Kawasaki disease group and control group.Patients first time hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics during the acute phase of Kawasaki disease were enrolled in Kawasaki disease group from May 2013 to August 2014.Sixty-four Kawasaki disease patients were enrolled into the Kawasaki disease group based on inclusion and exclusion criteria, including 39 males and 25 females, with an average age of 2.3 years.According to the results of echocardiography of children with Kawasaki disease, patients were divided into coronary artery dilatation group and non-coronary artery dilatation group, there were 33 cases in the coronary dilatation (CAD) group, 26 males and 7 females, the average age was 2.0 years, and 31 cases in the non-coronary dilatation (NCAD) group, 13 males and 18 females, the average age was 2.6 years.The control group consisted of children with fever but excluding vascular inflammatory diseases and healthy children, 32 cases in fever group, 19 males and 13 females, mean age was 4.5 years; in which healthy group had 32 cases, 18 males and 14 females and the average age was 3.3 years. Enzyme linked immunosorbent assay (ELISA) method was used to measure the levels of thrombospondin-1 (TSP-1) and TSP-2 in the plasma of each group, and the related laboratory parameters, and general information was analyzed.Multiple sets of normal distribution of data were compared by analysis of unifactorial analysis of variance and the two groups were compared by two independent samples t-test.Analysis of covariance was used to remove the effect of age.The χ(2) test was used to analyze categeorical data and receiver operating characteristic (ROC) curve for evaluating the predictive value of TSP-2. Compared with the febrile group

  5. Differential expression of miR-145 in children with Kawasaki disease.

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    Chisato Shimizu

    Full Text Available Kawasaki disease is an acute, self-limited vasculitis of childhood that can result in structural damage to the coronary arteries. Previous studies have implicated the TGF-β pathway in disease pathogenesis and generation of myofibroblasts in the arterial wall. microRNAs are small non-coding RNAs that modulate gene expression at the post-transcriptional level and can be transported between cells in extracellular vesicles. To understand the role that microRNAs play in modifying gene expression in Kawasaki disease, we studied microRNAs from whole blood during the acute and convalescent stages of the illness.RNA isolated from the matched whole blood of 12 patients with acute and convalescent Kawasaki disease were analyzed by sequencing of small RNA. This analysis revealed six microRNAs (miRs-143, -199b-5p, -618, -223, -145 and -145* (complementary strand whose levels were significantly elevated during the acute phase of Kawasaki disease. The result was validated using targeted qRT-PCR using an independent cohort (n = 16. miR-145, which plays a critical role in the differentiation of neutrophils and vascular smooth muscle cells, was expressed at high levels in blood samples from acute Kawasaki disease but not adenovirus-infected control patients (p = 0.005. miR-145 was also detected in small extracellular vesicles isolated from acute Kawasaki disease plasma samples. Pathway analysis of the predicted targets of the 6 differentially expressed microRNAs identified the TGF-β pathway as the top pathway regulated by microRNAs in Kawasaki disease.Sequencing of small RNA species allowed discovery of microRNAs that may participate in Kawasaki disease pathogenesis. miR-145 may participate, along with other differentially expressed microRNAs, in regulating expression of genes in the TGF-β pathway during the acute illness. If the predicted target genes are confirmed, our findings suggest a model of Kawasaki disease pathogenesis whereby miR-145 modulates TGF

  6. 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

  7. Relapse of nephrotic syndrome triggered by Kawasaki disease.

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    Maeda, Ryo; Kawasaki, Yukihiko; Suzuki, Shigeo; Ohara, Shinichiro; Kazuhide, Suyama; Hosoya, Mitsuaki

    2017-11-15

    Minor infections, allergies, insect bites, and bee stings are commonly reported causes of nephrotic syndrome (NS). Herein, we report, to the best of our knowledge, the first case of NS relapse due to Kawasaki disease (KD). An 8-year-old boy presented with high fever of 4-day duration. He had developed steroid-dependent NS at the age of 4 years and remained in remission after steroid and mizonbin therapy. Renal biopsy, performed at the age of four, showed minimal change (MC) disease. Upon examination, the patient fulfilled 5 of 6 criteria for KD under the Japanese diagnostic guidelines, with positive proteinuria. He was diagnosed with NS relapse caused by KD. Proteinuria resolved after treatment with intravenous immunoglobulin and cyclosporine A. We present the case of an 8-year-old boy, whose NS relapsed due to KD. To the best of our knowledge, this is the first case report. It is necessary to recognize that KD can trigger relapse of MCNS.

  8. Clinical and laboratory characteristics of children with Kawasaki disease

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    Fatih Akın

    2015-03-01

    Full Text Available Objective: In this study, we aimed to evaluate clinical and laboratory characteristics of patients with Kawasaki disease (KD in Konya region of Turkey. Methods: The hospital records of patients who were hospitalized with the diagnosis of KD in the Pediatrics Clinics of Konya Training and Research Hospital between May 2010 and June 2012 were reviewed retrospectively. Results: Seven cases were found to have the diagnosis of KD, two of whom were incomplete KD. Oropharynx changes were the most common (100% feature in our patients. Five (71% patients had bulbar conjunctivitis. Three (43% patients had erythema at the site of BCG inoculation. Adenopathy was present in all of our patients with the classical form. A desquamation was observed in one case at the seventh day of fever. No cardiac manifestation was seen. Elevated erythrocyte sedimentation rate and thrombocytosis were present in all patients. All of the patients were received intravenous immunoglobulin in the first ten days of the fever. Conclusion: KD should be considered as a possible diagnosis in any child presenting with prolonged fever. BCG reaction can be attributed as a diagnostic criterion for incomplete form of the disease especially in countries where BCG vaccination is routinely performed. Early treatment is essential to prevent cardiovascular complications.

  9. [Diagnosis and treatment of Kawasaki disease in burn children].

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    Feng, Jie; Li, Wei-ren; Wang, Wei

    2011-08-01

    We try to discuss the relationship between burn and Kawasaki disease (KD), and to study the diagnosis and treatment of KD in burn children. The medical records of one burn child with KD from our pediatric ward together with those of 5 burn children with KD retrieved from foreign literature were analyzed. The clinical features of KD, including bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, skin rash, cervical lymphadenopathy, changes in the distal part of extremities, were enrolled in the study. Six patients were male and younger than 5 years old, with 2 suffering from mild burn, 2 with moderate burn, and 2 with severe burn. Two days after second degree burn, all burn children had fever and skin rash with 4 or 5 clinical symptoms and signs of KD. Among them, coronary artery dilatation was found in 1 case as detected by echocardiography, positive wound culture was found in 2 cases, negative blood culture was found in 6 cases. All patients were given high-dose gamma globulin or (and) aspirin within 10 days after the first fever, followed by control and amelioration of the disease. We conclude that the pathogenesis of KD may be related with burn wound and reabsorption of edema. KD may be suspected in burn children younger than 5 years when they had fever and skin rash at the same time.

  10. National survey of pediatric hospitalizations due to Kawasaki disease and coronary artery aneurysms in the USA.

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    Okubo, Yusuke; Nochioka, Kotaro; Sakakibara, Hiroshi; Testa, Marcia; Sundel, Robert P

    2017-02-01

    Several studies revealed the epidemiology of Kawasaki disease-related hospitalizations among children in the USA and other countries. However, disparities of developing coronary artery aneurysms by race/ethnicity, patient socioeconomic status, and geographic locations remain unknown in the USA. Hospital discharge record data of patients with Kawasaki disease aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kid's Inpatient Database. The data were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Multivariable logistic regression was conducted to ascertain the factors associated with the development of coronary artery aneurysms. Total annual hospitalization rates of Kawasaki disease showed a decreasing trend, ranging from 6.54 per 100,000 children in 2003 to 6.11 per 100,000 children in 2012 (p Kawasaki disease ranged from 2.25 to 3.20%. Factor associated with the development of coronary artery aneurysms was hospitals in West (OR 2.15, 95% CI 1.42-3.26). Race/ethnicity, health insurance status, and household income were not associated with the development of coronary artery aneurysms. Total hospitalization rates of Kawasaki disease showed a decreasing trend. Children admitted to hospitals in West region were more likely to develop coronary artery aneurysms.

  11. [Three Cases of Moyamoya Disease with a History of Kawasaki Disease].

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    Kawasaki, Toshinari; Arakawa, Yoshiki; Sugino, Toshiya; Mitsuhara, Takafumi; Funaki, Takeshi; Kikuchi, Takayuki; Koyanagi, Masaomi; Yoshida, Kazumichi; Kunieda, Takeharu; Takahashi, Jun C; Takagi, Yasushi; Miyamoto, Susumu

    2015-11-01

    Here, we report three cases of moyamoya disease with a history of Kawasaki disease. A 33-year-old man was found to have stenotic lesions of the internal carotid arteries(ICAs)on both sides at a nearby hospital where he visited complaining of headache and lisping. He had received immunoglobulin therapy for Kawasaki disease at the ages of 1, 2, and 6 years. MRI showed only a chronic ischemic lesion in the white matter. Angiography showed occlusion at the terminal portion of the ICAs on both sides. He was diagnosed with moyamoya disease, but as he had no symptoms and preserved cerebral blood flow (CBF), he was kept under observation. An 8-year-old boy was diagnosed with moyamoya disease and underwent right encephaloduroarteriosynangiosis at a nearby hospital. He had received immunoglobulin therapy for Kawasaki disease at the age of 1 year. His ischemic symptoms worsened. Although MRI detected no apparent ischemic lesion, angiography revealed severe stenosis at the terminal portions of the ICAs on both sides, and 123I-IMP SPECT showed CBF impairment. Bilateral direct bypass was performed. His father was subsequently also diagnosed with moyamoya disease. A 4-year-old girl with epilepsy was diagnosed with moyamoya disease at a nearby hospital. She had been treated with aspirin for Kawasaki disease at the age of 1 year. MRI detected no remarkable ischemic lesions, but angiography revealed mild stenosis at the terminal portions of the ICAs on both sides. Five months later, her ischemic symptoms were worsening with progressing stenotic lesions, and she underwent bilateral direct bypass.

  12. Lipoprotein Particle Concentrations in Children and Adults following Kawasaki Disease

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    Lin, Jonathan; Jain, Sonia; Sun, Xiaoying; Liu, Victoria; Sato, Yuichiro Z.; Jimenez-Fernandez, Susan; Newfield, Ron S.; Pourfarzib, Ray; Tremoulet, Adriana H.; Gordon, John B.; Daniels, Lori B.; Burns, Jane C.

    2014-01-01

    Objective To test the hypothesis that children and adults with history of Kawasaki disease (KD) are more likely to have abnormal lipoprotein particle profiles that could place them at increased risk of atherosclerosis later in life. Study design Fasting serum samples were obtained from 192 children and 63 adults with history of KD and 90 age-similar healthy controls. Lipoprotein particle (P) concentrations and sizes were measured by Nuclear Magnetic Resonance (NMR) spectroscopy (Liposcience Inc., Raleigh, NC) and serum was assayed for total cholesterol (TC), triglycerides (TG), and high-density lipoprotein cholesterol (HDL)-C. Low-density lipoprotein cholesterol (LDL)-C was estimated using the Friedewald formula. Data were analyzed in a least-square means model adjusting for age and sex and using Holm correction for multiple comparisons. Results Compared with respective control groups, both adult and pediatric subjects with KD had significantly lower mean very-low-density lipoprotein-chylomicron particle concentrations (VLDLC-P), intermediate-density lipoproteins (IDL), TG, and TC concentrations. Pediatric subjects with KD had significantly lower LDL-P and LDL-C concentrations and lower mean TC/HDL-C ratio (ppediatric and adult subjects with KD regardless of their aneurysm status are no more likely than age-similar, healthy controls to have lipid patterns associated with increased risk of atherosclerosis. PMID:25039043

  13. INCIDENCE OF KAWASAKI DISEASE IN NORTHERN EUROPEAN COUNTRIES

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    Salo, Eeva; Griffiths, Elizabeth P.; Farstad, Teresa; Schiller, Bodil; Nakamura, Yosikazu; Yashiro, Mayumi; Uehara, Ritei; Best, Brookie M.; Burns, Jane C.

    2012-01-01

    Background We sought to compare the epidemiologic features of Kawasaki disease (KD) in three Northern European countries and Japan. Methods Data were obtained from discharge databases for hospitals in Finland, Norway, and Sweden from 1999–2009 and from nationwide epidemiologic surveys in Japan from 1998–2008. Annual incidence for each country was calculated using regional census data. Results During the 11-year period, 1,390 KD patients were recorded in the registries of the three Northern European countries. Average annual incidence rates per 100,000 children less than 5 years were: Finland 11.4, Norway 5.4, and Sweden 7.4. Overall, 86.4% of Japanese KD patients were less than 5 years compared to only 67.8% in the four Northern European countries (p<0.001). Conclusions The incidence of KD in Northern Europe was constant over the study period and much lower than in Japan. There was a significant age difference between Northern European and Japanese KD patients that remains unexplained. PMID:22726311

  14. Variations in ORAI1 Gene Associated with Kawasaki Disease.

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    Onouchi, Yoshihiro; Fukazawa, Ryuji; Yamamura, Kenichiro; Suzuki, Hiroyuki; Kakimoto, Nobuyuki; Suenaga, Tomohiro; Takeuchi, Takashi; Hamada, Hiromichi; Honda, Takafumi; Yasukawa, Kumi; Terai, Masaru; Ebata, Ryota; Higashi, Kouji; Saji, Tsutomu; Kemmotsu, Yasushi; Takatsuki, Shinichi; Ouchi, Kazunobu; Kishi, Fumio; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Sato, Yoshitake; Honda, Akihito; Kobayashi, Hironobu; Sato, Junichi; Shibuta, Shoichi; Miyawaki, Masakazu; Oishi, Ko; Yamaga, Hironobu; Aoyagi, Noriyuki; Yoshiyama, Megumi; Miyashita, Ritsuko; Murata, Yuji; Fujino, Akihiro; Ozaki, Kouichi; Kawasaki, Tomisaku; Abe, Jun; Seki, Mitsuru; Kobayashi, Tohru; Arakawa, Hirokazu; Ogawa, Shunichi; Hara, Toshiro; Hata, Akira; Tanaka, Toshihiro

    2016-01-01

    Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+)/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+) release activated Ca(2+) (CRAC) channel mediating store-operated Ca(2+) entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+)/NFAT pathway in the pathogenesis of this disorder.

  15. Qing Re Liang Xue Decoction Alleviates Hypercoagulability in Kawasaki Disease

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    Jiao-yang Chen

    2015-01-01

    Full Text Available Objective. Kawasaki disease (KD is a multisystemic autoimmune vasculitis. Intravenous immunoglobulin (IVIG is the first-line treatment for KD. It is unclear whether traditional Chinese medicine (TCM has an effect on KD. We aimed to observe the clinical efficacy of TCM on acute KD via serum interleukin-33 (IL-33 and tumor necrosis factor alpha (TNF-α measurements. Methods. Thirty-one KD patients were treated with Qing Re Liang Xue decoction and Western medicine (integrative medicine treatment group, while 28 KD patients were treated with Western medicine only (Western medicine treatment group. Thirty patients were included in a febrile group and 28 healthy children were included in the control group. Clinical characteristics and laboratory findings were gathered and compared. Serum IL-33 and TNF-α levels were measured by multiplex Luminex assay. Results. The platelet count in the integrative medicine treatment group was significantly lower than that in the Western medicine treatment group. The integrative medicine group had a shorter fever duration and lower IL-33 and TNF-α levels than those in the Western medicine group, but there were no significant differences between the two KD groups after treatment. Conclusion. Qing Re Liang Xue decoction improved the hypercoagulable state of KD patients. Potential myocardial protective effects require further research.

  16. Kawasaki disease and acute haemolytic anaemia after two IVIG infusions

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    Albena Telcharova-Mihaylovska

    2016-05-01

    Full Text Available Kawasaki disease (KD is one of the most common vasculitis disorders of childhood, affecting predominantly medium-sized arteries, particularly the coronary arteries. For treatment, high-dose intravenous immunoglobulin (IVIG is indicated. IVIG infusions are usually safe and well tolerated even though serious complications can be observed. We present a brief overview of KD and report a two-year-old girl with KD and two IVIG infusions (Gammagard® because of persistent fever after the completion of the first IVIG. Haemolytic anaemia developed after IVIG retreatment. The direct antiglobulin test after haemolysis was positive. The etiology of the haemolysis was related to the presence of transient, passively acquired antibodies that cause a direct antibody-mediated attack. There are few reports of haemolytic anaemia after IVIG infusions. The haemolysis in KD is dose-dependent and occurs more frequently after the second IVIG dose. Non-0 blood group patients are at greater risk. Another factor increasing the risk of haemolysis is also the presence of anaemia due to inflammation in KD.

  17. 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.

  18. Recent advances in the treatment of Kawasaki disease

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    Ken-Pen Weng

    2011-11-01

    Full Text Available Kawasaki disease (KD is acute systemic vasculitis that occurs mainly in infants and children under 5 years of age. The etiology of KD remains unknown. KD is liable to be complicated by coronary artery lesions (CALs, which develop in approximately 15–25% of untreated KD children and in approximately 5% of KD children after intravenous immunoglobulin (IVIG therapy. A single high dose of IVIG (2 g/kg is the gold standard therapy in the acute stage of KD. However, approximately 8–38% of children are unresponsive to initial IVIG treatment and at increased risk for CAL development. Anti-inflammatory high doses of aspirin are recommended in conjunction with IVIG, but our study demonstrated that there is no evidence of efficacy in preventing CAL development. The usefulness of steroids in initial therapy for KD or treatment of IVIG-resistant patients is not well established. Other immunosuppressive therapies, including infliximab, have been used in the treatment of refractory KD, but merit additional investigation. Subclinical atherosclerosis may develop early in KD patients, which makes early initiation of therapy to improve chronic inflammation an important issue. Future multicenter studies may help to define the optimal management of KD patients.

  19. 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.

  20. Hemodynamic simulations in coronary aneurysms of children with Kawasaki disease

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    Sengupta, Dibyendu; Burns, Jane; Marsden, Alison

    2009-11-01

    Kawasaki disease (KD) is a serious pediatric illness affecting the cardiovascular system. One of the most serious complications of KD, occurring in about 25% of untreated cases, is the formation of large aneurysms in the coronary arteries, which put patients at risk for myocardial infarction. In this project we performed patient specific computational simulations of blood flow in aneurysmal left and right coronary arteries of a KD patient to gain an understanding about their hemodynamics. Models were constructed from CT data using custom software. Typical pulsatile flow waveforms were applied at the model inlets, while resistance and RCR lumped models were applied and compared at the outlets. Simulated pressure waveforms compared well with typical physiologic data. High wall shear stress values are found in the narrow region at the base of the aneurysm and low shear values occur in regions of recirculation. A Lagrangian approach has been adopted to perform particle tracking and compute particle residence time in the recirculation. Our long-term goal will be to develop links between hemodynamics and the risk for thrombus formation in order to assist in clinical decision-making.

  1. Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease

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    Burns, Jane C.; Best, Brookie M.; Mejias, Asuncion; Mahony, Lynn; Fixler, David E.; Jafri, Hasan S.; Melish, Marian E.; Jackson, Mary Anne; Asmar, Basim I.; Lang, David J.; Connor, James D.; Capparelli, Edmund V.; Keen, Monica L.; Mamun, Khalid; Keenan, Gregory F.; Ramilo, Octavio

    2010-01-01

    Objective To test the safety, tolerability, and pharmacokinetics of the anti- TNF-α monoclonal antibody, infliximab, in subjects with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD). Study design We conducted a multicenter, randomized, prospective trial of second IVIG infusion (2 g/kg) versus infliximab (5 mg/kg) in 24 children with acute KD and fever following initial treatment with IVIG. Primary outcome measures were infliximab safety, tolerability, and pharmacokinetics. Secondary outcome measures were duration of fever and changes in markers of inflammation. Results Study drug infusions were associated with cessation of fever within 24 hours in 11 of 12 subjects treated with infliximab and 8 of 12 subjects retreated with IVIG. No infusion reactions or serious adverse events were attributed to either study drug. No significant differences were observed between treatment groups in the change from baseline for laboratory variables, fever, or echocardiographic assessment of coronary arteries. Conclusion Both infliximab and a second IVIG infusion were safe and well-tolerated in subjects with KD who were resistant to standard IVIG treatment. The optimal management of patients resistant to IVIG remains to be determined. PMID:18672254

  2. Variations in ORAI1 Gene Associated with Kawasaki Disease.

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    Yoshihiro Onouchi

    Full Text Available Kawasaki disease (KD; MIM#61175 is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+ release activated Ca(2+ (CRAC channel mediating store-operated Ca(2+ entry (SOCE on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596 was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls, P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method. Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012. These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+/NFAT pathway in the pathogenesis of this disorder.

  3. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002.

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    Huang, Guo-Ying; Ma, Xiao-Jing; Huang, Min; Chen, Shu-Bao; Huang, Mei-Rong; Gui, Yong-Hao; Ning, Shou-Bao; Zhang, Tuo-Hong; Du, Zhong-Dong; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2006-01-01

    Epidemiologic features of Kawasaki disease in China is still not clear. A questionnaire form and diagnostic guidelines for Kawasaki disease were sent to hospitals in Shanghai, which provided with pediatric medical care. All patients with Kawasaki disease diagnosed during January 1998 through December 2002 were recruited in this study. A total of 768 patients with Kawasaki disease were reported. The incidence rates of Kawasaki disease for each year were 16.79 (1998), 25.65 (1999), 28.16 (2000), 28.05 (2001), and 36.76 (2002) per 100,000 children under 5 years of age. The male/female ratio was 1.83:1. The age at onset ranged from 1 month to 18.8 years (median: 1.8 years). The disease occurred more frequently in spring and summer. Fever was the most common clinical symptom, followed by oral changes, extremities desquamate, rash, conjunctive congestion, lymphadenopathy, extremities swelling, and crissum desquamate. Cardiac abnormalities were found in 24.3% of patients. The most common cardiac abnormality was coronary artery lesions including dilatation (68%) and aneurysm (10%). The case-fatality rate at acute stage of the disease was 0.26%. A second onset of the disease occurred in 1.82% of patients. The incidence rate of Kawasaki disease in Shanghai is lower than that reported in Japan, but higher than those in western countries. The increasing trend in incidence, sex distribution and cardiac abnormalities are similar to those in previous reports. The seasonal distribution is similar to the report from Beijing and different from other reports.

  4. [Kawasaki disease: clinical behaviour and cardiovascular complications in children in a tertiary-care level hospital].

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    Gil Veloz, Mariana; Flores Ruiz, Eric M; Beirana Palencia, Luisa G; Miranda Novales, María Guadalupe; Huerta García, Gloria C; Solórzano Santos, Fortino

    2009-01-01

    To describe the clinical characteristics, outcome, and treatment response in a series of patients with Kawasaki disease (KD). Case-series, review of clinical records of children with KD diagnosis cared for from november 1999 to september 2006. 22 patients were included, male:female ratio, 1.4:1, 82% younger than 5 years, 14% with atypical presentation. Of the 22 patients, 10 (45%) received intravenous gammaglobulin (IVGG) in the first 10 days of symptoms onset at the recommended dose; four of them had coronary artery damage but none developed residual aneurysmatic lesions. Eight received IVGG in a different dose or after 10 days, six of them had coronary lesions and in two the damage was permanent. Four patients did not receive IVGG, and two had residual aneurysmatic lesions. None of those who received adequate treatment developed coronary residual damage, in comparison with 33% who received inadequate or no treatment, but difference was not statistically significant (P =.06). KD is a rare clinical entity in our country; even though most of the cases occurred with classic clinical criteria, late referral and delay of treatment worsen the prognosis.

  5. Difference in Risk Factors for Subtypes of Acute Cardiac Lesions Resulting from Kawasaki Disease.

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    Yamashita, Maho; Ae, Ryusuke; Yashiro, Mayumi; Aoyama, Yasuko; Sano, Takashi; Makino, Nobuko; Nakamura, Yosikazu

    2017-02-01

    Few studies discuss the risk factors for acute cardiac lesions (within 30 days) resulting from Kawasaki disease (KD). We aimed to clarify the characteristics of patients with cardiac lesions within 30 days and determine the risk factors for acute cardiac lesion subtypes. Using the 23rd nationwide survey of KD in Japan, we analyzed data from patients with or without acute cardiac lesions resulting from KD (n = 31,380). We subdivided patients with acute cardiac lesions into three types: acute valvular lesions, coronary aneurysms, and giant coronary aneurysms (GCA), and calculated the odds ratios of potential risk factors for acute cardiac lesion subtypes. The prevalence of acute cardiac lesions was 8.6%, and these lesions were more prevalent among males than females (1.98:1). Male sex, age valvular lesions differed from the risk factors for CALs, but GCA risk factors were similar to CAL risk factors: age acute GCA. We found differences in cardiac lesion risk factors within 30 days of diagnosing KD between acute CAL and valvular lesions resulting from KD. In particular, pediatricians should consider atypical definite cases and resistance to initial IVIG when assessing the risk of acute-phase GCA.

  6. 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) (p<0.05). This is the first population-based study on the epidemiology of KD in the western Mediterranean area. Incidence, clinical features and treatment plans in our cohort are similar to those described in other European studies.

  7. Found in Translation: International initiatives pursuing interleukin-1 blockade for treatment of acute Kawasaki Disease

    Science.gov (United States)

    Burns, Jane C.; Koné-Paut, Isabelle; Kuijpers, Taco; Shimizu, Chisato; Tremoulet, Adriana; Arditi, Moshe

    2016-01-01

    The decision to move forward with three clinical trials of IL-1 blockade for treatment of acute Kawasaki disease is a case study in translational science. These trials were born on the one hand from transcriptome studies of host response during the acute disease coupled with animal model investigations of key immune signaling pathways and, on the other hand, out of clinical desperation to intervene in patients with severe inflammation in the setting of acute Kawasaki disease. The convergence of laboratory science and clinical observations led to the clinical trials described here and serves as a model for how such observations can be translated into new therapies. PMID:27792871

  8. [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.

  9. A systematic review of validated methods for identifying Kawasaki disease using administrative or claims data.

    Science.gov (United States)

    Williams, Candice L; Sathe, Nila A; Krishnaswami, Shanthi; McPheeters, Melissa L

    2013-12-30

    To identify and assess algorithms used to identify Kawasaki syndrome/Kawasaki disease in administrative and claims databases. We searched the MEDLINE database from 1991 to September 2012 using controlled vocabulary and key terms related to Kawasaki disease. We also searched the reference lists of included studies. Two investigators independently assessed the full text of studies against pre-determined inclusion criteria. Two reviewers independently extracted data regarding participant and algorithm characteristics. Our searches identified 177 citations of which 22 met our inclusion criteria. All studies used algorithms including International Classification of Diseases, Ninth Revision (ICD-9) code 446.1 either alone, or with evidence of intravenous immunoglobulin (IVIG) administration, or with ICD-10 code M30.3. Six studies confirmed diagnoses by medical chart review. Three of these six studies reported validation statistics, with positive predictive values of 74%, 84%, and 86%, respectively. All studies that reported algorithms used either the ICD-9 code 446.1 either alone, with evidence of IVIG administration or with ICD-10 code M30.3. The ICD-9 code 446.1 alone produced positive predictive values of 74%, 84%, and 86% in separate studies in Georgia and California. The sensitivity of these codes to detect Kawasaki disease is unknown, as no sampling of medical records for missed true cases of Kawasaki disease was done. Further research would be helpful to determine whether the relatively high positive predictive values found in southern California are seen elsewhere and also to evaluate the performance of other codes to identify cases of Kawasaki disease and the sensitivity of the narrow algorithms that have been used to date. Copyright © 2013 Elsevier Ltd. All rights reserved.

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

    OpenAIRE

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

    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 ...

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

    Science.gov (United States)

    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.

  12. Long-term cardiovascular outcomes in survivors of Kawasaki disease.

    Science.gov (United States)

    Holve, Taylor J; Patel, Ajay; Chau, Quyen; Marks, Amy R; Meadows, Alison; Zaroff, Jonathan G

    2014-02-01

    Kawasaki disease (KD) may result in coronary aneurysm formation, but there is incomplete knowledge regarding its long-term effects. Our objective was to quantify the longer-term rates of adverse cardiac events in a modern North American KD cohort. Using the Kaiser Permanente Northern California population, we performed a retrospective cohort study in patients with a history of KD versus matched patients without KD. Chart review was used to confirm the diagnosis of KD and all outcomes of interest, including acute coronary syndrome, coronary revascularization, heart failure, ventricular arrhythmia, valve disease, aortic aneurysm, and all-cause mortality. All outcomes occurring at age ≥15 years were included in the primary analysis. Outcome rates were compared between the 2 groups by using Cox proportional hazards analysis. The study included 546 KD patients and 2218 matched patients without KD. Seventy-nine percent of the KD patients received intravenous immunoglobulin and 5% had persistent coronary aneurysm. The average follow-up time was 14.9 years. Only 2 KD patients experienced outcomes after age 15 (0.246 events per 1000 person-years) compared with 7 events in the non-KD group (0.217 events per 1000 person-years), a nonsignificant difference (hazard ratio: 0.81; 95% confidence interval: 0.16-4.0). Within the KD subgroup, persistent coronary aneurysm predicted the occurrence of adverse events (P = .007). This is the largest US study of longer-term cardiac outcomes after KD and reveals a low rate of adverse cardiovascular events through age 21. Additional validation studies, including studies with longer-term follow-up, should be performed.

  13. 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

  14. International Initiatives Pursuing Interleukin-1 Blockade for Treatment of Acute Kawasaki Disease

    NARCIS (Netherlands)

    Burns, Jane C.; Koné-Paut, Isabelle; Kuijpers, Taco; Shimizu, Chisato; Tremoulet, Adriana; Arditi, Moshe

    2017-01-01

    The decision to move forward with three clinical trials of IL-1 blockade for treatment of acute Kawasaki disease is a case study in translational science. These trials were born on the one hand from transcriptome studies of host response during the acute disease coupled with animal model

  15. 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

  16. Five years of Kawasaki disease in the Netherlands: a national surveillance study

    NARCIS (Netherlands)

    Tacke, Carline E.; Breunis, Willemijn B.; Pereira, Rob Rodrigues; Breur, Johannes M.; Kuipers, Irene M.; Kuijpers, Taco W.

    2014-01-01

    The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands. The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012. Questionnaires were sent to

  17. Five years of Kawasaki disease in the Netherlands a national surveillance study

    NARCIS (Netherlands)

    Tacke, C.E.; Breunis, W.B.; Pereira, R.R.; Breur, J.M.; Kuipers, I.M.; Kuijpers, T.W.

    2014-01-01

    Background: The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands. Methods: The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012.

  18. Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease

    Directory of Open Access Journals (Sweden)

    Hyo Min Park

    2013-02-01

    Full Text Available &lt;b&gt;Purpose:&lt;/b&gt; 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. &lt;b&gt;Methods:&lt;/b&gt; 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. &lt;b&gt;Results:&lt;/b&gt; 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. &lt;b&gt;Conclusion:&lt;/b&gt; 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.

  19. Progressive Coronary Dilatation Predicts Worse Outcome in Kawasaki Disease.

    Science.gov (United States)

    Chih, Wan-Ling; Wu, Pei-Yuan; Sun, Li-Chuang; Lin, Ming-Tai; Wang, Jou-Kou; Wu, Mei-Hwan

    2016-04-01

    To explore the implication of serial coronary changes on the late coronary outcomes in patients with Kawasaki disease (KD) with coronary aneurysms ≧ 4 mm. We performed a retrospective review of 78 patients with KD with large coronary aneurysms (1980-2013, male: 76.9%; 792 patient-years). Progressive coronary dilatation was defined for those with progressive enlargement of coronary arteries in 3 consecutive echocardiograms. We studied 27 patients with KD with giant aneurysms (≧ 8 mm) and 51 patients with KD with medium aneurysms (4-8 mm). All the giant and 43.1% of medium aneurysms persisted during the study period. For the patients with giant aneurysms, their 10-year freedom from acute myocardial infarction/cardiovascular death and all ischemia was 66% and 52%, respectively. The median intervals for the aneurysm diameters reaching their peak were 3.3 months (giant) and 0.25 months (medium), respectively. In patients with giant aneurysms, the 10-year freedom from ischemia was much lower in those with progressive coronary dilatation (28% vs 59%, P = .021). In patients with medium aneurysms, the probability of 5-year persistence of aneurysm was much greater (67.2% vs 14.8%, P outcomes in the patients with KD who had aneurysms larger than 4 mm. In addition to coronary diameters 1 month after the onset of KD, progressive coronary dilatation at 2 or more months after diagnosis may be an indicator of duration, and the severity of vasculitis and adverse dilative remodeling were associated with worse late coronary outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Clinical characteristics of Kawasaki disease with sterile pyuria

    Directory of Open Access Journals (Sweden)

    Ja Yun Choi

    2013-01-01

    Full Text Available &lt;B&gt;Purpose:&lt;/B&gt; Kawasaki disease (KD is a systemic vasculitis and affects many organ systems. It often presents sterile pyuria, microscopic hematuria, and proteinuria due to renal involvement. The aims of this study were to define clinical characteristics of acute KD patients with pyuria and to analyze meaning of pyuria in KD. &lt;B&gt;Methods:&lt;/B&gt; The medical records and laboratory findings including serum and urine test of 133 patients with KD admitted to Yeungnam University Hospital from March 2006 to December 2010 were reviewed retrospectively. &lt;B&gt;Results:&lt;/B&gt; Forty patients had sterile pyuria and their clinical characteristics including age, gender and body weight were not significantly different with those who did not have pyuria. Fever duration after treatment was significantly longer in KD patients with pyuria. Erythrocyte sedimentation rate, C-reactive protein and serum concentration of alanine aminotransferase were significantly higher in patients with pyuria. Hyponatremia and coronary artery lesion were seen more often in patients with pyuria but there was no significant difference. Also serum blood urea nitrogen was significantly higher in KD patients with pyuria. Urine �ⲭmicroglobulin was elevated in both patients groups and showed no difference between two groups. &lt;B&gt;Conclusion:&lt;/B&gt; We found more severe inflammatory reaction in KD patients with pyuria. We also found elevation of some useful parameters like �ⲭmicroglobulin that indicate renal involvement of KD through the urine test. Careful management and follow up will need for KD patients with pyuria and it is necessary in the future to study the specific parameters for renal involvement of KD.

  1. Kawasaki disease in adults: Observations in France and literature review.

    Science.gov (United States)

    Fraison, Jean-Baptiste; Sève, Pascal; Dauphin, Claire; Mahr, Alfred; Gomard-Mennesson, Emeline; Varron, Loig; Pugnet, Gregory; Landron, Cédric; Roblot, Pascal; Oziol, Eric; Chalhoub, Gihane; Galempoix, Jean-Marc; Humbert, Sébastien; Humbert, Philippe; Sbidian, Emilie; Grange, Florent; Bayrou, Olivier; Cathebras, Pascal; Morlat, Philippe; Epaulard, Olivier; Pavese, Patricia; Huong, Du Le Thi; Zoulim, Abdelkader; Stankovic, Katia; Bachelez, Hervé; Smail, Amar; Bachmeyer, C; Granel, Brigitte; Serratrice, Jacques; Brinchault, Graziella; Mekinian, Arsène; Costedoat-Chalumeau, Nathalie; Bourgarit-Durand, Anne; Puéchal, Xavier; Guillevin, Loïc; Piram, Maryam; Koné-Paut, Isabelle; Fain, Olivier

    2016-03-01

    Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France. We collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature. We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18-68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8-21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1-117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p=0.01). Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome. Copyright © 2015 Elsevier B.V. All rights reserved.

  2. Aspirin Dose and Prevention of Coronary Abnormalities in Kawasaki Disease.

    Science.gov (United States)

    Dallaire, Frederic; Fortier-Morissette, Zoe; Blais, Samuel; Dhanrajani, Anita; Basodan, Dania; Renaud, Claudia; Mathew, Mathew; De Souza, Astrid M; Dionne, Audrey; Blanchard, Joel; Saulnier, Harrison; Kaspy, Kimberley; Rached-d'Astous, Soha; Dahdah, Nagib; McCrindle, Brian W; Human, Derek G; Scuccimarri, Rosie

    2017-06-01

    Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA to be used. We aimed to evaluate the noninferiority of ASA at an antiplatelet dose in acute KD in preventing coronary artery (CA) abnormalities. This is a multicenter, retrospective, nonrandomized cohort study including children 0 to 10 years of age with acute KD between 2004 and 2015 from 5 institutions, of which 2 routinely use low-dose ASA (3-5 mg/kg per day) and 3 use high-dose ASA (80 mg/kg per day). Outcomes were CA abnormalities defined as a CA diameter with a z score ≥2.5. We assessed the risk difference of CA abnormalities according to ASA dose. All subjects received ASA and intravenous immunoglobulin within 10 days of fever onset. There were 1213 subjects included, 848 in the high-dose and 365 in the low-dose ASA group. There was no difference in the risk of CA abnormalities in the low-dose compared with the high-dose ASA group (22.2% vs 20.5%). The risk difference adjusted for potential confounders was 0.3% (95% confidence interval [CI]: -4.5% to 5.0%). The adjusted risk difference for CA abnormalities persisting at the 6-week follow-up was -1.9% (95% CI: -5.3% to 1.5%). The 95% CI of the risk difference of CA abnormalities adjusted for confounders was within the prespecified 5% margin considered to be noninferior. In conjunction with intravenous immunoglobulin, low-dose ASA in acute KD is not inferior to high-dose ASA for reducing the risk of CA abnormalities. Copyright © 2017 by the American Academy of Pediatrics.

  3. The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update.

    Science.gov (United States)

    Zhu, Frank H; Ang, Jocelyn Y

    2016-09-01

    Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.

  4. INTESTINAL PSEUDO-OBSTRUCTION AND TRANSIENT CARDIOVASCULAR ABNORMALITIES IN KAWASAKI DISEASE

    Directory of Open Access Journals (Sweden)

    Maria L Avila-Aguero

    2005-08-01

    Full Text Available SUMMARYWe describe a 2 year-old boy with severe vasculitis who presented with a typical Kawasaki disease complicated with an intestinal pseudo-obstruction, gallbladder hydrops, myocarditis and transient coronary abnormalities despite early administration of intravenous immunoglobulin treatment.RESUMENDescribimos el caso de un niño de 2 años con vasculitis grave que presentó un cuadro típico de enfermedad de Kawasaki complicada con una pseudo-obtrucción intestinal, hidrops vesicular, miocarditis y anormalidades coronarias transitorias, a pesar de la administración temprana de tratamiento con inmunoglulina intravenosa

  5. Usefulness of real-time 3-dimensional echocardiography to identify and quantify left ventricular dyssynchrony in patients with Kawasaki disease.

    Science.gov (United States)

    Yu, Yi; Sun, Kun; Xue, Haihong; Chen, Sun; Yang, Jianping

    2013-06-01

    The role of left ventricular (LV) dyssynchrony in Kawasaki disease is unknown. This study sought to establish values for real-time 3-dimensional (3D) echocardiographically derived LV dyssynchrony parameters and identify and quantify LV dyssynchrony in patients with Kawasaki disease. Forty patients hospitalized for Kawasaki disease were analyzed retrospectively, and 40 sex- and age-matched healthy control volunteers were also enrolled. The systolic dyssynchrony index (percentage of the cardiac cycle) from 16 and 12 LV segments on real-time 3D echocardiography was analyzed to calculate LV dyssynchrony (defined as the standard deviation of the time to reach the minimum systolic volume for 16 LV segments) according to a 17-segment model. We analyzed the 3D LV ejection fraction (LVEF), end-diastolic volume, and end-systolic volume in the patients with Kawasaki disease compared to the controls. The 16-segment systolic dyssynchrony index ± SD was significantly higher in the patients with Kawasaki disease: 2.73% ± 0.96% compared to 2.01% ± 0.85% in the controls (P Kawasaki disease was 2.65% ± 0.93% compared to 1.98% ± 0.81% in the controls (PKawasaki disease and an LVEF of less than 50% had a significantly higher systolic dyssynchrony index compared to patients with an LVEF of 50% or greater (2.89% ± 0.79% versus 2.26% ± 0.73%; P Kawasaki disease, and global systolic function was impaired. The LVEF measured by a biplane method was sufficiently related to the LVEF measured by echocardiography. Real-time 3D echocardiography is a noninvasive and feasible method for identifying and evaluating LV dyssynchrony in children with Kawasaki disease. Left ventricular dyssynchrony is significantly impaired and related to LV systolic function in patients with Kawasaki disease.

  6. Extensive cervical lymphadenitis mimicking bacterial adenitis as the first presentation of Kawasaki disease.

    Science.gov (United States)

    Rossi, Felipe de Souza; Silva, Marco Felipe Castro da; Kozu, Kátia Tomie; Camargo, Luís Fernando Aranha; Rossi, Flávia Feijó Panico; Silva, Clovis Artur; Campos, Lúcia Maria de Arruda

    2015-01-01

    Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient's symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.

  7. Pediatric Kawasaki Disease and Adult Human Immunodeficiency Virus Kawasaki-Like Syndrome Are Likely the Same Malady.

    Science.gov (United States)

    Johnson, Raymond M; Bergmann, Kelly R; Manaloor, John J; Yu, Xiaoqing; Slaven, James E; Kharbanda, Anupam B

    2016-09-01

    Background.  Pediatric Kawasaki disease (KD) and human immunodeficiency virus (HIV) + adult Kawasaki-like syndrome (KLS) are dramatic vasculitides with similar physical findings. Both syndromes include unusual arterial histopathology with immunoglobulin (Ig)A + plasma cells, and both impressively respond to pooled Ig therapy. Their distinctive presentations, histopathology, and therapeutic response suggest a common etiology. Because blood is in immediate contact with inflamed arteries, we investigated whether KD and KLS share an inflammatory signature in serum. Methods.  A custom multiplex enzyme-linked immunosorbent assay (ELISA) defined the serum cytokine milieu in 2 adults with KLS during acute and convalescent phases, with asymptomatic HIV + subjects not taking antiretroviral therapy serving as controls. We then prospectively collected serum and plasma samples from children hospitalized with KD, unrelated febrile illnesses, and noninfectious conditions, analyzing them with a custom multiplex ELISA based on the KLS data. Results.  Patients with KLS and KD subjects shared an inflammatory signature including acute-phase reactants reflecting tumor necrosis factor (TNF)-α biologic activity (soluble TNF receptor I/II) and endothelial/smooth muscle chemokines Ccl1 (Th2), Ccl2 (vascular inflammation), and Cxcl11 (plasma cell recruitment). Ccl1 was specifically elevated in KD versus febrile controls, suggesting a unique relationship between Ccl1 and KD/KLS pathogenesis. Conclusions.  This study defines a KD/KLS inflammatory signature mirroring a dysfunctional response likely to a common etiologic agent. The KD/KLS inflammatory signature based on elevated acute-phase reactants and specific endothelial/smooth muscle chemokines was able to identify KD subjects versus febrile controls, and it may serve as a practicable diagnostic test for KD.

  8. Incomplete Kawasaki disease in an adolescent. A case report Enfermedad de Kawasaki incompleta en un adolescente. Informe de un caso

    Directory of Open Access Journals (Sweden)

    Ruth María Eraso Garnica

    2010-05-01

    Full Text Available

    We report the case of a 12 year old boy with incomplete Kawasaki disease (KD. During the course of his illness he presented with symptoms of acute hepatitis, which delayed the final diagnosis. Although patients with KD may present with elevation of hepatic enzymes and bilirubin, associated acute hepatitis is of rare occurrence in this entity.

    Se presenta el caso de un joven de doce años, con diagnóstico de enfermedad de Kawasaki (EK incompleta, quien presentó, en el trascurso de la misma, un cuadro clínico compatible con hepatitis aguda. El diagnóstico definitivo se retrasó por la necesidad de descartar otros diagnósticos diferenciales relacionados con la hepatitis aguda. Aunque en la EK puede ocurrir una disfunción hepática, caracterizada por aumento de las transaminasas y bilirrubinas, el cuadro clínico de una hepatitis aguda es muy raro en el curso de esta enfermedad.

  9. Usefulness of Neutrophil to Lymphocyte Ratio in Prediction of Coronary Artery Lesions in Patients with Kawasaki Disease.

    Science.gov (United States)

    Demir, Fikri; Karadeniz, Cem; Özdemir, Rahmi; Yozgat, Yılmaz; Çelegen, Kübra; Karaaslan, Utku; Demirol, Mustafa; Meşe, Timur; Ünal, Nurettin

    2015-10-01

    Kawasaki disease is an inflammatory condition. Neutrophil to lymphocyte ratio is a marker reflecting inflammation. The aim of the study is to evaluate usefulness of neutrophil to lymphocyte ratio in diagnosis of Kawasaki disease and in prediction of coronary artery lesions. Retrospective cross-sectional study. Seventy-five children with Kawasaki disease and 66 controls were retrospectively enrolled. Their leukocyte, neutrophil, and lymphocyte counts were recorded. Abnormally distributed data were shown as median (interquartile range). Cases having coronary artery diameter two standard deviation above mean were diagnosed to have coronary artery lesions. Median age of Kawasaki disease patients was 34 months. Twentyfive of those (33.33%) had incomplete Kawasaki disease and twenty-six (34.66%) had coronary artery lesions. Leukocyte [12.61 (6.09)×10(3)/µL vs. 8.48 (5.58)×10(3)/µL], neutrophil [6.73 (4.10) ×10(3)/µL vs. 4.62 (5.47)×10(3)/µL], and lymphocyte [4.04 (2.91)×10(3)/µL vs. 3.02 (2.57) ×10(3)/µL] counts were significantly higher in Kawasaki disease patients compared to controls (all p values Kawasaki disease and incomplete Kawasaki disease cases did not differ, while comparison of patients with and without coronary artery lesions revealed significantly higher neutrophil to lymphocyte ratio values in former group [2.02 (1.63) vs. 1.50 (1.28), p=0.01]. The cut-off neutrophil to lymphocyte ratio value for prediction of coronary artery lesions was determined as 1.32. Neutrophil to lymphocyte ratio values in Kawasaki patients with coronary lesions were significantly higher than the ones without and values greater than 1.32 were useful in prediction of coronary lesions.

  10. Association of mannose-binding lectin genotype with cardiovascular abnormalities in Kawasaki disease

    NARCIS (Netherlands)

    Biezeveld, Maarten H.; Kuipers, Irene M.; Geissler, Judy; Lam, Jan; Ottenkamp, Jaap J.; Hack, C. Erik; Kuijpers, Taco W.

    2003-01-01

    Kawasaki disease is an acute vasculitis of possible infectious cause, which in particular affects the coronary arteries. Young children rely mostly on their innate immune system for protection against invading microorganisms, of which mannose-binding lectin is an important component. We aimed to

  11. 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 ...

  12. 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

  13. Transforming Growth Factor-beta Signaling Pathway in Patients With Kawasaki Disease

    NARCIS (Netherlands)

    Shimizu, Chisato; Jain, Sonia; Davila, Sonia; Hibberd, Martin L.; Lin, Kevin O.; Molkara, Delaram; Frazer, Jeffrey R.; Sun, Shelly; Baker, Annette L.; Newburger, Jane W.; Rowley, Anne H.; Shulman, Stanford T.; Burgner, David; Breunis, Willemijn B.; Kuijpers, Taco W.; Wright, Victoria J.; Levin, Michael; Eleftherohorinou, Hariklia; Coin, Lachlan; Popper, Stephen J.; Relman, David A.; Fury, Wen; Lin, Calvin; Mellis, Scott; Tremoulet, Adriana H.; Burns, Jane C.

    2011-01-01

    Background-Transforming growth factor (TGF)-beta is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-beta signaling might be important in KD

  14. Extracardial Vasculopathy After Kawasaki Disease: A Long-Term Follow-up Study

    NARCIS (Netherlands)

    Dietz, Sanne M.; Tacke, Carline E.; de Groot, Eric; Kuipers, Irene M.; Hutten, Barbara A.; Kuijpers, Taco W.; ten Berge, Maartje; Biezeveld, Maarten H.; Bruijn, Martijn; Delemarre, Luçan C.; Dolman, Koert M.; Filippini, Luc H. P. M.; Hendriks, Tom; Maingay-Visser, Dianne A. P. G. F.; Noordzij, Jeroen G.; Nuboer, Roos; Plötz, Frans B.; Rozendaal, Lieke; ten Tusscher, Gavin W.; Starreveld, Sander; Verhoeven, Jennifer J.; Weggelaar, Nielske M.; Weijer, Olivier; de Winter, Peter

    2016-01-01

    Background-Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysm (CAA) as a major complication. Controversy exists about cardiovascular risk later in life. The aim of our study was to evaluate whether KD patients are at increased risk, as assessed by carotid intima-media

  15. A genome-wide association study identifies novel and functionally related susceptibility loci for Kawasaki Disease

    NARCIS (Netherlands)

    Burgner, D.; Davila, S.; Breunis, W.B.; Ng, S.B.; Li, Y.; Bonnard, C.; Ling, L.; Wright, V.J.; Thalamuthu, A.; Odam, M.; Shimizu, C.; Burns, J.C.; Levin, M.; Kuijpers, T.W.; Hibberd, M.L.

    2009-01-01

    Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD

  16. Matrix metalloproteinase haplotypes associated with coronary artery aneurysm formation in patients with Kawasaki disease

    Science.gov (United States)

    Shimizu, Chisato; Matsubara, Tomoyo; Onouchi, Yoshihiro; Jain, Sonia; Sun, Shelly; Nievergelt, Caroline M.; Shike, Hiroko; Brophy, Victoria H.; Takegawa, Tsuyoshi; Furukawa, Susumu; Akagi, Teiji; Newburger, Jane W.; Baker, Annette L.; Burgner, David; Hibberd, Martin L.; Davila, Sonia; Levin, Michael; Mamtani, Manju; He, Weijing; Ahuja, Sunil K.; Burns, Jane C.

    2010-01-01

    Aneurysms of the vascular wall represent a final common pathway for a number of inflammatory processes including atherosclerosis and idiopathic vasculitis syndromes. Kawasaki disease is an acute, self-limited vasculitis in children and the leading cause of acquired coronary artery aneurysms. We sought to identify shared molecular mechanisms of aneurysm formation by genotyping 8 polymorphisms in MMP-1, 3, 7, 12 and 13 in the gene cluster on Chr.11q22 whose gene products have been implicated in aneurysm formation or are known to have elastase activity. We genotyped 482 US-UK Kawasaki disease patients (aneurysm+: n=111, aneurysm−: n=371) and tested our findings in an independent cohort of 200 Japanese Kawasaki disease patients (aneurysm+: n=58, aneurysm−: n=142). Analysis of the five MMP genes identified modest trends in allele and genotype frequencies for MMP-3 rs3025058 (−/T) and haplotypes containing MMP-3 rs3025058 (−/T) and MMP-12 rs2276109 (A/G) (nominal p= 2-4 × 10−5) that conferred increased risk of aneurysm formation in US-UK subjects. This finding was validated in Japanese subjects and suggests the importance of this locus in aneurysm formation in children with Kawasaki disease. The region encompassing these risk haplotypes is a prime candidate for re-sequencing to look for rare genetic variation that may influence aneurysm formation. PMID:20827277

  17. A genome-wide association study identifies novel and functionally related susceptibility Loci for Kawasaki disease

    NARCIS (Netherlands)

    Burgner, David; Davila, Sonia; Breunis, Willemijn B.; Ng, Sarah B.; Li, Yi; Bonnard, Carine; Ling, Ling; Wright, Victoria J.; Thalamuthu, Anbupalam; Odam, Miranda; Shimizu, Chisato; Burns, Jane C.; Levin, Michael; Kuijpers, Taco W.; Hibberd, Martin L.; Christiansen, Frank; Goldwater, Paul; Curtis, Nigel; Palasanthiran, Pamela; Ziegler, John; Nissan, Michael; Kuipers, Irene M.; Ottenkamp, Jaap J.; Geissler, Judy; Biezeveld, Maarten; Filippini, Luc; Brogan, Paul; Klein, Nigel; Shah, Vanita; Dillon, Michael; Booy, Robert; Shingadia, Delane; Bose, Anu; Mukasa, Thomas; Tulloh, Robert; Michie, Colin; Shike, Hiroko; Nievergelt, Caroline M.; Schork, Nicholas J.; Newburger, Jane W.; Baker, Annette L.; Sundel, Robert P.; Rowley, Anne H.; Shulman, Stanford T.

    2009-01-01

    Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD

  18. Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease

    NARCIS (Netherlands)

    Khor, Chiea Chuen; Davila, Sonia; Shimizu, Chisato; Sheng, Stephanie; Matsubara, Tomoyo; Suzuki, Yasuo; Newburger, Jane W.; Baker, Annette; Burgner, David; Breunis, Willemijn; Kuijpers, Taco; Wright, Victoria J.; Levin, Michael; Hibberd, Martin L.; Burns, Jane C.; Baker, Annette L.; Rowley, Anne H.; Mason, Wilbert; Melish, Marian E.; Tremoulet, Adriana H.; Odam, Miranda; Christiansen, Frank; Goldwater, Paul; Curtis, Nigel; Palasanthiran, Pamela; Ziegler, John; Nissan, Michael; Nourse, Clare; Kuipers, Irene M.; Ottenkamp, Jaap J.; Geissler, Judy; Biezeveld, Maarten; Filippini, Luc; Ling, Ling; Brogan, Paul; Klein, Nigel; Shah, Vanita; Dillon, Michael; Booy, Robert; Shingadia, Delane; Bose, Anu; Mukasa, Thomas; Tulloh, Robert; Michie, Colin

    2011-01-01

    Kawasaki disease (KD) is a self limited vasculitis in which host genetics plays a prominent role. To further the understanding of the role of host genetics in KD, a three-stage genetic study was conducted that began with a family linkage study and ultimately involved more than 3000 individuals to

  19. 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).

  20. Cardiac lesions and initial laboratory data in Kawasaki disease: a nationwide survey in Japan.

    Science.gov (United States)

    Kuwabara, Masanari; Yashiro, Mayumi; Kotani, Kazuhiko; Tsuboi, Satoshi; Ae, Ryusuke; Nakamura, Yosikazu; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2015-01-01

    Cardiac lesions, such as coronary dilatation, aneurysms, narrowing, myocardial infarction, and valvular lesions, sometimes occur in Kawasaki disease, but most studies have only evaluated cardiac lesions in the later phase of the disease. This study was undertaken to clarify the related factors between cardiac lesions and laboratory data in the initial phase of Kawasaki disease. We conducted a cross-sectional study using data for 26 691 patients from the 22nd nationwide survey of Kawasaki disease in Japan, the observation period of which was from January 2011 through December 2012. We excluded patients with recurrent Kawasaki disease and who were more than seven days from the start of symptoms at admission. We analyzed 23 155 cases (13 353 boys; mean age: 923 ± 734 days) with available laboratory data for white blood cell count, platelet count, serum albumin, and C-reactive protein (CRP). Cardiac lesions were detected in 984 cases (656 boys and 328 girls); lesions were classified as coronary dilatation (764 cases), coronary aneurysm (40), giant coronary aneurysm (6), coronary narrowing (3), and valvular lesions (204). The significant related factors of initial coronary dilatation were male sex (odds ratio [OR] 1.73), older age (OR per 100 days increase 1.03), higher platelet count (OR per 10 000 cells/µL increase 1.006), lower albumin (OR per 1 g/dL increase 0.66), and higher CRP (OR per 1 mg/dL increase 1.02). The factors related to coronary aneurysm were higher platelet count (OR 1.01) and lower albumin (OR 0.34). No factors were significantly related to giant coronary aneurysm. The related factors of valvular lesions were age (OR 0.98), and higher CRP (OR 1.05). Clinicians should consider male sex, older age, higher platelet count, lower albumin levels, and higher CRP levels when assessing risk of cardiac lesions in the initial phase of Kawasaki disease.

  1. Comportamiento clínico-epidemiológico de la enfermedad de Kawasaki Clinical-epidemiological behavior of Kawasaky' disease

    OpenAIRE

    Milagros Morales Leiva; María del Carmen Luis Álvarez; Frances Seiglie Díaz; Odette Pantoja Pereda

    2011-01-01

    INTRODUCCIÓN. La enfermedad de Kawasaki es una vasculitis sistémica, aguda, febril, de evolución autolimitada, con alto riesgo de secuelas en niños menores de 5 años, si no se diagnostica y trata en su estadio inicial. Fue objetivo de este estudio describir el comportamiento clínico-epidemiológico de la enfermedad de Kawasaki en la infancia. MÉTODOS. Se realizó un estudio descriptivo, de aspectos clínicos y epidemiológicos, de los pacientes con diagnóstico de enfermedad de Kawasaki atendidos ...

  2. Differential protein analysis of serum exosomes post-intravenous immunoglobulin therapy in patients with Kawasaki disease.

    Science.gov (United States)

    Zhang, Li; Song, Qi-Fang; Jin, Jing-Jie; Huang, Ping; Wang, Zhou-Ping; Xie, Xiao-Fei; Gu, Xiao-Qiong; Gao, Xue-Juan; Jia, Hong-Ling

    2017-11-01

    Kawasaki disease, which is characterised by systemic vasculitides accompanied by acute fever, is regularly treated by intravenous immunoglobulin to avoid lesion formation in the coronary artery; however, the mechanism of intravenous immunoglobulin therapy is unclear. Hence, we aimed to analyse the global expression profile of serum exosomal proteins before and after administering intravenous immunoglobulin. Two-dimensional electrophoresis coupled with mass spectrometry analysis was used to identify the differentially expressed proteome of serum exosomes in patients with Kawasaki disease before and after intravenous immunoglobulin therapy. Our analysis revealed 69 differential protein spots in the Kawasaki disease group with changes larger than 1.5-fold and 59 differential ones in patients after intravenous immunoglobulin therapy compared with the control group. Gene ontology analysis revealed that the acute-phase response disappeared, the functions of the complement system and innate immune response were enhanced, and the antibacterial humoral response pathway of corticosteroids and cardioprotection emerged after administration of intravenous immunoglobulin. Further, we showed that complement C3 and apolipoprotein A-IV levels increased before and decreased after intravenous immunoglobulin therapy and that the insulin-like growth factor-binding protein complex acid labile subunit displayed reverse alteration before and after intravenous immunoglobulin therapy. These observations might be potential indicators of intravenous immunoglobulin function. Our results show the differential proteomic profile of serum exosomes of patients with Kawasaki disease before and after intravenous immunoglobulin therapy, such as complement C3, apolipoprotein A-IV, and insulin-like growth factor-binding protein complex acid labile subunit. These results may be useful in the identification of markers for monitoring intravenous immunoglobulin therapy in patients with Kawasaki disease.

  3. Cardiovascular status after Kawasaki disease in the UK

    Science.gov (United States)

    Shah, V; Christov, G; Mukasa, T; Brogan, K S; Wade, A; Eleftheriou, D; Levin, M; Tulloh, RM; Almeida, B; Dillon, MJ; Marek, J; Klein, N; Brogan, PA

    2015-01-01

    Objective Kawasaki disease (KD) is an acute vasculitis that causes coronary artery aneurysms (CAA) in young children. Previous studies have emphasised poor long-term outcomes for those with severe CAA. Little is known about the fate of those without CAA or patients with regressed CAA. We aimed to study long-term cardiovascular status after KD by examining the relationship between coronary artery (CA) status, endothelial injury, systemic inflammatory markers, cardiovascular risk factors (CRF), pulse-wave velocity (PWV) and carotid intima media thickness (cIMT) after KD. Methods Circulating endothelial cells (CECs), endothelial microparticles (EMPs), soluble cell-adhesion molecules cytokines, CRF, PWV and cIMT were compared between patients with KD and healthy controls (HC). CA status of the patients with KD was classified as CAA present (CAA+) or absent (CAA−) according to their worst-ever CA status. Data are median (range). Results Ninety-two KD subjects were studied, aged 11.9 years (4.3–32.2), 8.3 years (1.0–30.7) from KD diagnosis. 54 (59%) were CAA−, and 38 (41%) were CAA+. There were 51 demographically similar HC. Patients with KD had higher CECs than HC (p=0.00003), most evident in the CAA+ group (p=0.00009), but also higher in the CAA− group than HC (p=0.0010). Patients with persistent CAA had the highest CECs, but even those with regressed CAA had higher CECs than HC (p=0.011). CD105 EMPs were also higher in the KD group versus HC (p=0.04), particularly in the CAA+ group (p=0.02), with similar findings for soluble vascular cell adhesion molecule 1 and soluble intercellular adhesion molecule 1. There was no difference in PWV, cIMT, CRF or in markers of systemic inflammation in the patients with KD (CAA+ or CAA−) compared with HC. Conclusions Markers of endothelial injury persist for years after KD, including in a subset of patients without CAA. PMID:26316045

  4. Th17- and Treg-related cytokine and mRNA expression are associated with acute and resolving Kawasaki disease.

    Science.gov (United States)

    Guo, M M-H; Tseng, W-N; Ko, C-H; Pan, H-M; Hsieh, K-S; Kuo, H-C

    2015-03-01

    Kawasaki disease is a vasculitis most commonly afflicting children Kawasaki disease. Blood samples were obtained from 186 children with Kawasaki disease at 24 h before IVIG therapy, followed by 3 days and 21 days after IVIG therapy. Thirty children with an acute febrile infectious disease and 30 healthy children were obtained as control. Plasma levels of Th17- and Treg-related cytokines including IL-6, IL-17A, IL-10, TGF-β, and mRNA expression levels of RORγt and Foxp3 were tested. Patients with Kawasaki disease had higher levels of plasma IL-17A (25.35 ± 3.21 vs 7.78 ± 1.78 pg/ml, P Kawasaki disease was associated with higher IL-17A and IL-6, a cytokine profile similar to other autoimmune diseases. IVIG therapy resulted in increased expression of Treg-related FoxP3. IVIG resistance was associated with higher levels of IL-10 and IL-17A. Our findings provide further evidence that Kawasaki disease is an autoimmune-like disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  5. Overview of Pharmacological Treatment Options for Pediatric Patients with Refractory Kawasaki Disease

    Science.gov (United States)

    Saneeymehri, Seyyedeh; Baker, Katherine

    2015-01-01

    Kawasaki disease is an autoimmune disease found predominantly in children under the age of 5 years. Its incidence is higher in those who live in Asian countries or are of Asian descent. Kawasaki disease is characterized as an acute inflammation of the vasculature bed affecting mainly the skin, eyes, lymph nodes, and mucosal layers. Although the disease is usually self-limiting, patients may develop cardiac abnormalities that can lead to death. The exact cause of the disease is unknown; however, researchers hypothesize that an infectious agent is responsible for causing Kawasaki disease. Initial treatment options with intravenous immune globulin and aspirin are sufficient to cure most patients who acquire this disease. Unfortunately, in up to one-quarter of patients, the disease will be refractory to initial therapy and will require further management with corticosteroid, immunomodulatory, or cytotoxic agents. The lack of randomized, controlled trials makes treatment of refractory disease difficult to manage. Until larger randomized, controlled trials are published to give more guidance on therapy for this stage of disease, clinicians should use the data available from observational studies and case reports in conjunction with their clinical expertise to make treatment decisions. PMID:26170768

  6. Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14

    Science.gov (United States)

    Borjali, Davood

    Title: Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14 Kawasaki disease(KD) is a kind of vasculitis diagnosed by clinical manifestation and it caused acquired heart disease in children because of coronary arteries involvement. Method: patient divided to three group of American Japanese and incomplete and also study in two group according to fever days and then clinical features and laboratory data were checked. Result: A total of 150 patients were enrolled during the study period. number of patients with incomplete Kawasaki disease was 128 american group was 28 and Japanese was 4 patients, the most prevalent symptom was scaling of extremities(61 bladder most seen in group with fever more than five days. Keyword: Kawasaki , epidemiology , criteria

  7. An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient.

    Science.gov (United States)

    Friedland-Little, Joshua; Aiyagari, Ranjit; Gajarski, Robert J; Schumacher, Kurt R

    2013-02-01

    We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three-yr-old girl two-yr status post-orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies. © 2012 John Wiley & Sons A/S.

  8. Epidemiology of Kawasaki disease in Italy: surveillance from national hospitalization records.

    Science.gov (United States)

    Cimaz, Rolando; Fanti, Eleonora; Mauro, Angela; Voller, Fabio; Rusconi, Franca

    2017-08-01

    Kawasaki disease is a systemic vasculitis with an acute and self-limited course. The incidence of the disease differs widely among ethnic groups and is higher in the Asian populations. In Italy, no recent data are available. We studied the epidemiology of Kawasaki disease in the years 2008-2013 in children 0-14 years old in Italy using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates in order to select the first hospital admission for the disease. The disease peaked in the first 2 years of life, with 85.5% of cases under 5 years. Male/female ratio was 1.4:1. The incidence rate was 5.7 per 100,000 children 0-14 years old and 14.7 for children younger than 5 years. The incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring. A coronary artery aneurysm was recorded in 2.2% of the patients younger than 5 years of age. This is the first epidemiologic study on Kawasaki disease incidence in the country of Italy. Figures are in line but slightly higher than those reported for other European countries. What is known: • Kawasaki disease is more common in Asian populations. • European incidence data are scarce. What is new: • Epidemiological data in Italy show similar incidence, albeit slightly higher, than in other European countries. • Incidence data slightly rose in the recent past.

  9. Primary percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with giant coronary aneurysm due to Kawasaki disease.

    Science.gov (United States)

    Mongiovì, Maurizio; Alaimo, Annalisa; Vernuccio, Federica; Pieri, Daniele

    2014-01-01

    We report a case of acute myocardial infarction in an 8-year-old boy with a history of Kawasaki disease and giant coronary aneurysms in the right and left coronary arteries. We performed coronary angiography and percutaneous coronary intervention 4 hours after the onset of symptoms. This case suggests that primary percutaneous coronary intervention might be safe and effective in the long-term treatment of acute myocardial infarction due to coronary sequelae of Kawasaki. © 2013 Wiley Periodicals, Inc.

  10. Clinical Presentation and Cardiovascular Outcome in Complete versus Incomplete Kawasaki Disease.

    Science.gov (United States)

    Shivalingam, G; Prashanth, G P; Hebbal, Kallesh; Aguiar, Rodney

    2017-10-15

    To compare the demographic, clinical, and laboratory features of incomplete and complete presentations of Kawasaki disease. A retrospective review of the electronic case records between January 2000 and December 2015 in a tertiary care referral center of Sohar, Oman. 31 out of 64 children (48.4%) had incomplete presentation. Children with incomplete presentation had higher incidence of skin rash, lymphadenopathy and conjunctivitis. They took a longer time to show clinical response to intravenous immunoglobulin [mean (SD) 52.6 (17.4) h vs 40.1 (16.4) h, P=0.005], and had prolonged hospitalization [mean (SD) 6.2 (2.5) d vs 4.6 (1.7) d, P=0.009]. Children with incomplete presentation of Kawasaki disease tend to have prolonged hospitalization but short- and long-term coronary outcomes appear to be similar.

  11. Pregnancy in Women with a History of Kawasaki Disease: Management and Outcomes

    OpenAIRE

    Gordon, Catherine T.; Jimenez-Fernandez, Susan; Daniels, Lori B.; Kahn, Andrew M.; Tarsa, Maryam; Matsubara, Tomoyo; Shimizu, Chisato; Burns, Jane C.; Gordon, John B.

    2014-01-01

    © 2014 Royal College of Obstetricians and Gynaecologists. Objective To characterise the obstetrical management and outcomes in a series of women with a history of Kawasaki disease (KD) in childhood. Design Retrospective case series. Setting Tertiary healthcare setting in the USA. Population Women with a history of KD in childhood. Methods Women completed a detailed health questionnaire and participated in research imaging studies as part of the San Diego Adult KD Collaborative Study. Main out...

  12. QT Dispersion and T Wave Peak–to–end Interval Dispersion in Children with Kawasaki Disease

    OpenAIRE

    Hamid Amoozgar; Maryam Ahmadipour; Anis Amirhakimi

    2013-01-01

    Background: The main complication of Kawasaki disease is the Coronary Artery (CA) involvement and long term follow up of patients depends on the severity of coronary arterial aneurysms, ischemia, and thrombosis. Early diagnosis of these complications can lead to a more desirable outcome for patients. Myocardial ischemia can prolong QT dispersion and increase the risk of cardiac arrhythmias as well as sudden cardiac arrests. Also, T wave peak–to–end (Tp-Te) interval dispersion, which provides ...

  13. Kawasaki disease and the emerging coronary artery disease epidemic in India: is there a correlation?

    Science.gov (United States)

    Singh, Surjit; Aulakh, Roosy; Kawasaki, Tomisaku

    2014-04-01

    Although Kawasaki disease (KD) is now being increasingly reported from India, the vast majority of children with KD are still not being diagnosed and treated. A recent study from Chandigarh has shown that the incidence of KD is at least 4.54/100,000 children below 15 y of age. Extrapolations of this figure suggest that a minimum of 17,417 new cases of KD would be occurring every year in our country. A significant proportion of these children may develop coronary artery abnormalities. These children would then be at risk of developing myocardial ischemia as young adults. It is authors' contention that (undiagnosed) KD in childhood may be contributing to the growing pool of coronary artery disease (CAD) in India. Similarly, a missed diagnosis of KD in childhood should be considered as a possibility while evaluating adults with CAD, especially when there are no overt risk factors and no family history of the disease.

  14. Retrospective observational study indicates that the Pediatric Assessment Triangle may suggest the severity of Kawasaki disease.

    Science.gov (United States)

    Kawai, Reiko; Nomura, Osamu; Tomobe, Yutaro; Morikawa, Yoshihiko; Miyata, Koichi; Sakakibara, Hiroshi; Miura, Masaru

    2018-01-31

    We examined whether the Pediatric Assessment Triangle (PAT) could predict the severity of Kawasaki disease. We enrolled patients diagnosed with Kawasaki disease between July 2012 and June 2016 at the emergency department of Tokyo Metropolitan Children's Medical Center in Tokyo, Japan. Triage nurses assigned participants to unstable or stable PAT groups. We compared the incidence of coronary artery aneurysms (CAA), the Kobayashi score, which measures resistance to intravenous immunoglobulin treatment, and the incidence of initial treatment resistance. Of the 420 participants, who were aged 0-145 months with a mean age of 31.2 ±23.9 months, 66 (16%) were assigned to the unstable PAT group. The incidence of CAA was similar between the two groups. The percentage of unstable PAT group participants with a Kobayashi score of at least five points (39% versus 18%, p<0.001) and initial treatment resistance (25% versus 15%, p=0.047) were significantly higher than in the stable PAT group. Unstable PAT was an independent risk factor for initial treatment resistance (odds ratio 2.02, 95% confidence interval 1.05-3.90, p=0.035). An unstable PAT was able to predict the severity of Kawasaki disease when measured by a higher rate of initial treatment resistance. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  15. Atypical radiological findings in cerebral hydatid disease.

    Science.gov (United States)

    Benzagmout, Mohammed; Maaroufi, Mustapha; Chakour, Khalid; Chaoui, Mohammed E

    2011-07-01

    Cerebral hydatid disease is very rare, representing only 2% of all cerebral space occupying lesions. The diagnosis is usually based on a pathognomonic CT pattern. Exceptionally, the image is atypical raising suspicion of many differential diagnoses such as intracerebral infectious, vascular lesions, or tumors. We report 2 atypical cases of cerebral hydatid cysts diagnosed in a 21, and a 24-year-old woman. The CT scan results suggest oligodendroglioma in the first case and brain abscess in the second. An MRI was helpful in the diagnosis of the 2 cases. Both patients underwent successful surgery with a good outcome. The hydatid nature of the cyst was confirmed by histology in both cases.

  16. Increased risk of Kawasaki disease in children with common allergic diseases.

    Science.gov (United States)

    Wei, Chang-Ching; Lin, Cheng-Li; Kao, Chia-Hung; Liao, Yen-Hsiu; Shen, Te-Chun; Tsai, Jeng-Dau; Chang, Yen-Jung; Li, Tsai-Chung

    2014-05-01

    Growing evidence reveals a link between Kawasaki disease (KD) and allergic diseases. This population-based case-control study is to investigate the onset of allergic diseases in children and the associated risks of KD. From 1996 to 2008, 2748 children with KD and 10,656 randomly selected controls were enrolled. Odds ratios of KD were calculated for the association with pre-existing allergic diseases. The children with a single allergic disease had an increased risk of KD, with adjusted odds ratios of having KD of 1.82 for urticaria (95% confidence interval [CI], 1.54-2.14), 1.44 for allergic rhinitis (95% CI, 1.23-1.70), and 1.22 for atopic dermatitis (95% CI, 1.06-1.39). The adjusted odds ratios increased with the number of concurrent allergic diseases, from 1.61 (95% CI, 1.43-1.82) for those with only one allergic disease to 1.71 (95% CI, 1.48-1.98) for those with at least two allergic diseases. The children who made two or more medical visits for associated allergic diseases per year had an increased risk of KD. Children with onset of allergic diseases were at increased risk for KD, and the increased risk was associated with the cumulative effect of concurrent allergic diseases and frequency of seeking medical care. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. A male infant had subdural effusion and paroxysmal supraventricular tachycardia during the febrile episode of Kawasaki disease: a case report and literature review.

    Science.gov (United States)

    Chou, Chia-Pei; Lin, I-Chun; Kuo, Kuang-Che

    2016-05-28

    Kawasaki disease is an acute, febrile, self-limiting, inflammatory systemic vasculitis seen in early childhood, most commonly in those below 5 years of age. In Kawasaki disease, the coronary arteries are most commonly affected, which may lead to asymptomatic coronary artery ectasia or formation of an aneurysm. Paroxysmal supraventricular tachycardia(PSVT) is a severe and rare cardiovascular complication of Kawasaki disease. A case of Kawasaki disease presenting with unusual findings, including subdural effusion and PSVT is reported. This is a 4-month-10-day-old boy presents with anterior fontanelle bulging and moderate bilateral subdural effusion at the acute stage of Kawasaki disease and PSVT at the subacute stage of Kawasaki disease. The subdural effusion was resolution after intravenous immunoglobulin(IVIG) administration. And the PSVT was subsided after administered 3 doses of adenosine, 1 dose of amiodarone loading and Propranolol twice per day use. At 1-year follow-up has made a complete recovery with no arrhythmia episodes, developmental effects or abnormal neurologic findings. Subdural effusion in the acute stage of Kawasaki disease may be an inflammatory response. It may resolves spontaneously after anti-inflammatory treatment such as IVIG infusion. PSVT is a severe cardiovascular complication of Kawasaki disease. In those who taking aspirin, we need to carefully observe the heart rhythm and PSVT side effects, especially in the first month.

  18. Wilson’s disease: Atypical imaging features

    Directory of Open Access Journals (Sweden)

    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.

  19. N-terminal pro-brain natriuretic peptide can be an adjunctive diagnostic marker of hyper-acute phase of Kawasaki disease.

    Science.gov (United States)

    Kwon, Hyuksool; Lee, Jin Hee; Jung, Jae Yun; Kwak, Young Ho; Kim, Do Kyun; Jung, Jin Hee; Chang, Ikwan; Kim, Kyuseok

    2016-12-01

    The purpose of this study was to determine whether the serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level could be a useful marker for Kawasaki disease in the pediatric emergency department (PED) and in the presence of fever duration of 4 days or less (hyper-acute phase of Kawasaki disease). Medical records of patients who were 1 month to 15 years old of age and presented at the PED with suspected Kawasaki disease from January 1, 2010, to December 31, 2014, were collected retrospectively. Two hundred thirty-nine patients with a history of fever for 4 days or less were diagnosed with Kawasaki disease, as well as 111 patients with other febrile diseases, and were enrolled. The NT-proBNP level was significantly higher in patients with Kawasaki disease (Kawasaki disease vs. other febrile disease group, 444.8 (189.7-951.5) vs. 153.4 (68.9-287.6) pg/mL; p marker for hyper-acute phase of Kawasaki disease in the PED. What is Known: • N-terminal pro-brain natriuretic peptide level has been reported as a useful marker for diagnosis in patients with the acute phase of Kawasaki disease. • But, in the cases of less than 5 days of fever, the appropriate level of NT-proBNP for differentiating Kawasaki disease in PED has not been yet evaluated. What is New: • NT-proBNP might be an adjunctive laboratory marker for hyper-acute phase of Kawasaki disease.

  20. Ruptured pediatric posterior cerebral artery aneurysm 9 years after the onset of Kawasaki disease: a case report.

    Science.gov (United States)

    Tanaka, Satoshi; Sagiuchi, Takao; Kobayashi, Ikuo

    2007-06-01

    A 12-year-old boy who had a history of Kawasaki disease 9 years ago experienced a subarachnoid hemorrhage by ruptured right posterior cerebral artery aneurysm. On day 1 operation, as the aneurysm was very fragile and bled easily, two intraoperative ruptures, including a very premature rupture, were encountered. As a result, a left hemiparesis especially severe in the left hand was caused by the right anterior thalamic infarction due to the occlusion of a thalamo-perforating artery arising near the neck of the aneurysm. The histopathological examination of the intraoperative excised aneurysmal dome disclosed the thickening of the endothelial inner due to the endothelial hypertrophy and the invasion of inflammatory cells. This finding of the aneurysm was partially mimicking the finding of the coronary artery of the patients with Kawasaki disease. The combination of cerebral aneurysm and Kawasaki disease has never been reported until now, and the etiology of the aneurysm of this patient is unclear.

  1. Atypical Celiac Disease Resistant to Thyroxine Replacement

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    Oguzhan Aksu

    2014-08-01

    Full Text Available Celiac disease, an immune-mediated enteropathy that develops in susceptible individuals upon ingestion of gluten containing diet, is closely associated with other autoimmune endocrine disorders, particularly autoimmune thyroid disease. Celiac disease and hypothyroidism ( especially due to Hashimoto disease cooccurence is frequently mentioned in the literature. The relationship between celiac disease and autoimmune thyroid disease was first described three decades ago. Patients usually have the classical presentation of diarrhoea and steatorrhoea but hypothyroidism with weight loss and increased dose requirement of L Thyroxine are two well recognised presentations of celiac disease in hypothyroidism. It is known that these cases are resistant to thyroxine replacement. Herein we presented a 35 year old female patient with atypical celiac disease and needed an extremely high dose of thyroxine such as 1600 mcg/day for treatment.

  2. Kawasaki Disease in Latin American Children: Past, Current, and Future Challenges.

    Science.gov (United States)

    Ulloa-Gutierrez, Rolando; Salgado, Andrea P; Tremoulet, Adriana H

    2014-12-01

    Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children in developed countries and Asia. However, there is a paucity of data available from Latin America. In response to the gap in knowledge about KD in Latin America, a group of pediatric infectious disease researchers from the Kawasaki Disease Research Center at the University of California San Diego and the Sociedad Latinoamericana de Infectología Pediátrica joined efforts during the last decade to address this problem. The Red de Enfermedad de Kawasaki en América Latina (Latin American Kawasaki Disease Network) was launched in 2013 to study the epidemiology of KD among children from the major pediatric tertiary referral hospitals in Latin America. This multinational multicenter network is primarily composed of pediatric infectious diseases, cardiology, rheumatology, and immunology subspecialists and pediatricians from 20 countries, and it is one of the world's largest networks to study the general epidemiology of KD. The first 2 prospective and retrospective multinational multicenter studies looking at the epidemiology of KD in the region were initiated in 2014. Future plans for the network include establishing collaborative research alliances and projects with other centers around the world. To date [ 1], there have been no published studies describing the overall incidence and prevalence of KD in Latin American children. The most important and recent epidemiological study addressing this issue, related to Chile, was published in 2012 [ 2]. Of these, the most recent relevant study addressed the seasonality of KD in different parts of the globe, including some Latin American and Caribbean countries [ 4]. In this document, we briefly summarize relevant available information from Latin America. Although there have been other publications from individual countries that are outside the scope of this communication, the majority of these reports are single case reports, or case series

  3. Kawasaki disease and subsequent risk of allergic diseases: a population-based matched cohort study.

    Science.gov (United States)

    Kuo, Ho-Chang; Chang, Wei-Chiao; Yang, Kuender D; Yu, Hong-Ren; Wang, Chih-Lu; Ho, Shu-Chen; Yang, Chun-Yuh

    2013-03-23

    The risk of allergic diseases among Kawasaki disease (KD) patients relative to the general population is not known. The aim of this study was to perform a population-based cohort study to investigate the risk of allergic diseases among children after KD in Taiwan- a country with the third highest incidence of KD in the world. Data were obtained from the Taiwan National Health Insurance Research Database. In total, 253 patients who were 5 years of age or younger and had a first-time hospitalization with a diagnosis of KD between 1997 and 2005 were included as the study cohort and 1,012 non-KD patients matched for age and sex were included as comparison cohort. Multivariate Cox proportional hazard regression model was used to adjust for confounding and to compare the 6-year allergic-free survival rate between these two cohorts. The incidence rate of allergic diseases (184.66 per 1000 person-year) was significantly higher in the KD cohort than in the control cohort (124.99 per 1000 person-years). After adjusting for potential confounders, the adjusted hazard ratios of asthma and allergic rhinitis were 1.51 (95% confidence interval = 1.17-1.95) and 1.30 (95% confidence interval = 1.04-1.62), respectively. We conclude that KD patients were at an increased risk for allergic diseases compared with the comparison cohort.

  4. [Identification of mutations associated with coronary artery lesion susceptibility in Kawasaki disease by targeted enrichment of genomic region sequencing technique].

    Science.gov (United States)

    Zhu, D Y; Song, S R; Xie, L J; Qiu, F; Yang, J; Xiao, T T; Huang, M

    2017-07-02

    Objective: To screen and identify the mutations in Kawasaki disease by targeted enrichment of genomic region sequencing technique and investigate susceptibility genes associated with coronary artery lesion. Method: This was a case-control study.A total of 114 patients diagnosed as Kawasaki disease treated in Shanghai Children's Hospital between December 2015 and November 2016 were studied and another 45 healthy children who were physically examined in outpatient department were enrolled as control group. Patients were divided into two groups based on the results of echocardiogram. Peripheral venous blood was obtained from patients and controls. Genomic DNA was extracted. SeqCap EZ Choice libraries were prepared by targeted enrichment of genomic region technology. Then the libraries were sequenced to identify susceptibility genes associated with coronary artery lesion in patients diagnosed as Kawasaki disease.Susceptible genes were identified by Burden test, Pearson chi-square test or Fisher's exact probability test. Result: There was statistically significant difference in TNFRSF11B(rs2073618)G>C(p.N3K)mutation and GG/GC/CC genotype between Kawasaki disease group and control group(χ(2)=15.52, P =0.00). There was statistically significant difference in TNFRSF13B(rs34562254)C>T(p.P251L)mutation(χ(2)=10.40, P =0.01)and LEFTY1(rs360057)T>G(p.D322A)mutation(χ(2)=8.505, P =0.01)between patients with coronary artery lesions and those without. Conclusion: Targeted enrichment of genomic region sequencing technology can be used to do primary screening for the susceptible genes associated with coronary artery lesions in Chinese Kawasaki patients and may provide theoretical basis for larger sample investigation of risk prediction score standard in Kawasaki disease.

  5. Comparison of early and late intravenous gamma globulin treatment of Kawasaki disease on fever and cardiovascular complications.

    Science.gov (United States)

    Mohammadzadeh, Iraj; Noei, Somayyeh; Babazadeh, Kazem; Zamani, Hassan; Barari-Savadkoohi, Rahim; Alizadeh-Navaei, Reza

    2016-01-01

    Cardiac involvement was the major leading cause of death in patients with Kawasaki and IVIG administration reduces cardiac complications. The objective of this study was to determine the frequency of cardiovascular complications and duration of fever with regard to the time of intravenous immunoglobulin (IVIG) administration of patients with Kawasaki disease. This follow-up study was done on all patients with Kawasaki disease who were hospitalized at Amirkola Children's Hospital between 2006 and 2011. Diagnosis of Kawasaki was clinical and included fever more than 5 days with 4 of 5 signs containing mucosal changes, scaling and skin rash, bilateral nonexudative conjunctivitis, cervical lymph adenopathy and edema in lower extremities. After diagnosis of Kawasaki, all patients received standard treatment (intravenous immunoglobulins and aspirin) and undergoing cardiac echocardiography in 2 weeks, 2 months and 6 months. Information including age, sex, sign of diseases, laboratory findings, and cardiac complications in echocardiography were recorded. This study was performed on 100 patients (61 boys and 39 girls) with Kawasaki disease. The mean age of children was 2.8±2.6 years. Cardiac complication rate was 47% at the onset of the disease and had reached to 7% at the end of the sixth month (P=0.000). Distribution of cardiovascular complications in the second week, the second month and the sixth month after treatment was not significantly different according to the start of time of treatment (p>0.05). Duration of fever in patients who received treatment before 10 th day (1.5±1.3) did not have significant difference (P=0.78) with patients who received after 10 th day (1.6±0.9). Result shows that most of patients (99%) responded to the treatment with IVIG and ASA and cardiovascular complication ratio decreased. There was not significant relationship between duration of fever and time of IVIG treatment initiation.

  6. Tricuspid and mitral regurgitation detected by color flow Doppler in the acute phase of Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Suzuki, A.; Kamiya, T.; Tsuchiya, K.; Sato, I.; Arakaki, Y.; Kohata, T.; Ono, Y.

    1988-02-01

    Valvular lesions in the acute phase of Kawasaki disease were studied in 19 children. The patients were intensively observed by color flow Doppler every day from the day of hospitalization up to 12 days after the onset of the disease and 2 or more times a week thereafter, for up to 28 days. Mitral regurgitation (MR) was found in 9 patients (47%) and tricuspid regurgitation (TR) in 10 (53%). MRs were of transient type and confirmed from 7.5 +/- 1.6 (mean +/- standard deviation) to 13.1 +/- 6.5 days after the onset of the disease. Both types of valvular regurgitation were mild. The direction of regurgitation was from the center of valvular coaptation toward the posterior wall of the atrium. Neither valvular prolapse nor valvular deformity was noted. In patients with MR, left ventricular ejection fraction on M-mode echocardiography was significantly lower in the acute phase than in the convalescent phase of the disease (p less than 0.05). Using gallium-67 scintigram, the positive uptake of the isotope was noted in 7 (88%) of 8 patients with MR, but not found at all in 8 patients free of MR. These results suggest that MR and TR are often transient in the acute phase of Kawasaki disease and could be attributed to myocarditis.

  7. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease

    OpenAIRE

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Abstract Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise. Sixty-three KD patients were r...

  8. [Evaluation of treatment and management of Kawasaki coronary artery disease based on clinical severity classification].

    Science.gov (United States)

    Liu, Fang; Zhao, Lu; Wu, Lin; Liang, Xuecun; Chu, Chen; He, Lan; Huang, Guoying

    2015-09-01

    To evaluate the efficacy and safety of evaluation,treatment and follow-up of Kawasaki coronary artery disease based on the clinical severity classification. This study evaluated 52 patients admitted to the Children's Hospital of Fudan University between July 2005 and December 2013 who were diagnosed with Kawasaki Disease with coronary artery disease.Inclusion criteria were a disease course of more than two months, initial echocardiography showing severity of grade IV and above, and confirmation of disease severity by angiography. Of those studies, 44 were male and eight were female, aged 6 to 142 (average 41) months. Treatment was planned according to protocols in "Suggestions for Management of Kawasaki Coronary Artery Disease" with follow-up. Those patients with grade IV and above confirmed by angiogram were given oral low-dose asprin and warfarin, and those with grade Vb were given coronary artery bypass grafting (CABG) after comprehensive evaluation. Analysis was carried out for diagnosis, treatment, complications, and results of follow-up. (1) Satisfied images were shown by the angiography of all 52 cases. Forty five patients (86%) had giant aneurysm or multiple aneurysms, with thrombosis found in 10 of 45 patients (22%). Coronary artery lesions occurred in 138 coronary branches, and more common in left anterior descending branch (47 branches, with incidence 34%) and right coronary artery (48 branches, with incidence 35%). There were no complications during or after angiography. (2) After angiography, 49 patients remained at grade IV or above, and three improved to grade III. Ultimately, clinical severities of coronary artery disease included three patients at grade III, 31 patients at grade IV, nine patients at grade Va, and nine patients at grade Vb. (3) Thirty-eight patients were properly using aspirin and warfarin, and two patients with severely elevated international normalized ratio (INR) levels presented with knee joint and gastric hemorrhage, both of

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1988-12-01

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1988-02-01

    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.

  11. Atypical Celiac Disease: From Recognizing to Managing

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

  13. Sonographic Gallbladder Abnormality Is Associated with Intravenous Immunoglobulin Resistance in Kawasaki Disease

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    Chih-Jen Chen

    2012-01-01

    Full Text Available Objective. Kawasaki disease (KD is an acute systematic vasculitis in children which causes coronary arterial lesions and hydrops of gallbladder. Our objective is to correlate the clinical significance and influence on disease outcome of patients with gallbladder abnormalities in Kawasaki dissease. Methods. Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients with abdominal sonography were divided into 2 groups based on the absence (Group A, N=61 or presence (Group B, N=16 of gallbladder abnormalities (GBA, defined as hydrops or acalculous cholecystitis. Between the two groups, clinical features, demographic data (including admission days, coronary artery lesions, IVIG resistance, and laboratory data before/after IVIG treatment were collected for analysis. Results. The presence of sonographic gallbladder abnormalities is correlated with higher levels of serum CRP, GPT, and neutrophils. It also points to an increased number of IVIG resistance rates in group B. There was no significant statistical difference among clinical features, age, gender, admission days, or coronary artery lesions between the two groups. Conclusion. Sonographic gallbladder abnormalities are associated with higher CRP, GPT, neutrophil and IVIG resistance in KD. It can be used as a predictor of IVIG resistance in patients with KD.

  14. Intravenous immunoglobulin treatment responsiveness depends on the degree of CD8+ T cell activation in Kawasaki disease.

    Science.gov (United States)

    Ye, Qing; Gong, Fang-Qi; Shang, Shi-Qiang; Hu, Jian

    2016-10-01

    Kawasaki disease (KD) has become the most common cause of acquired heart disease in children and is also a risk factor for ischemic heart disease in adults. However, Kawasaki disease lacks specific laboratory diagnostic indices. Thus, this study analyzed the T cell activation profiles of Kawasaki disease and assessed their value in the diagnosis of Kawasaki disease and the prediction of intravenous immunoglobulin (IVIG) sensitivity. We analyzed human leukocyte antigen-DR (HLA-DR), CD69 and CD25 expression on peripheral blood CD4+ and CD8+ T cells during the acute phase of KD. We compared the percentages of HLA-DR+/CD69+/CD25+ T cells in the CD4+ and CD8+ T cell populations of IVIG-effective and IVIG-resistant groups. Receiver operating characteristic curves were used to assess the diagnostic value of the above parameters. The median percentage of CD8+HLA-DR+ T cells and the median ratio of CD8+HLA-DR+ T cells/CD8+CD25+ T cells were significantly elevated in the patient group compared with those in the control group during the acute phase of KD. Regarding the diagnosis of Kawasaki disease, the area under the ROC curve was 0.939 for the percentage of CD8+HLA-DR+ T cells. There was a significant difference in the ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells between IVIG-resistant patients and IVIG-sensitive patients. Regarding IVIG sensitivity, the area under the ROC curve was 0.795 for it. Excessive CD8+ T cell activation, as well as an imbalance between CD8+ T cell activation and inhibition, underlies the pathogenesis of Kawasaki disease. The percentage of CD8+ HLA-DR+ T cells may be used as an index to diagnose Kawasaki disease. IVIG inhibits CD8+ T cell activation, but excessive CD8+ T cell activation may cause IVIG resistance. The ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells may be used as a predictor of IVIG sensitivity. Copyright © 2016. Published by Elsevier Inc.

  15. Percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with coronary aneurysm and stenosis due to Kawasaki disease.

    Science.gov (United States)

    Drossner, David M; Chappell, Clay; Rab, Tanveer; Kim, Dennis

    2012-06-01

    We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.

  16. Disruption of vascular homeostasis in patients with Kawasaki disease: Involvement of vascular endothelial growth factor and angiopoietins

    NARCIS (Netherlands)

    Breunis, Willemijn B.; Davila, Sonia; Shimizu, Chisato; Oharaseki, Toshiaki; Takahashi, Kei; van Houdt, Michel; Khor, Chiea Chuen; Wright, Victoria J.; Levin, Michael; Burns, Jane C.; Burgner, David; Hibberd, Martin L.; Kuijpers, Taco W.; Odam, Miranda; Christiansen, Frank; Witt, Campbell; Goldwater, Paul; Curtis, Nigel; Palasanthiran, Pamela; Ziegler, John; Nissan, Michael; Nourse, Clare; Kuipers, Irene M.; Ottenkamp, Jaap J.; Geissler, Judy; Tacke, Carline C. E.; Ng, Sarah B.; Li, Yi; Bonnard, Carine; Ling, Ling; Brogan, Paul; Klein, Nigel; Shah, Vanita; Dillon, Michael; Booy, Robert; Shingadia, Delane; Bose, Anu; Mukasa, Thomas; Tulloh, Robert; Michie, Colin; Shike, Hiroko; Nievergelt, Caroline M.; Schork, Nicholas J.; Newburger, Jane W.; Baker, Annette L.; Sundel, Robert P.; Rowley, Anne H.; Shulman, Stanford T.

    2012-01-01

    Objective In Kawasaki disease (KD), a pediatric vasculitis of medium-sized arteries, the coronary arteries are most commonly affected. Angiopoietins and vascular endothelial growth factor (VEGF) play an important role in maintaining vascular homeostasis. Recently, we identified ANGPT1 and VEGFA as

  17. The effect of Kawasaki disease on childhood allergies - a sibling control study.

    Science.gov (United States)

    Liew, Woei Kang; Lim, Chee Wen Terence; Tan, Teng Hong; Wong, Keng Yean; Tai, Bee Choo; Quek, Swee Chye; Bever, Hugo van

    2011-08-01

    Kawasaki disease (KD) is a multisystem inflammatory vasculitis of childhood, with widespread T-helper cell type 1 immune activation. We hypothesize that children who suffered from KD will have a lower risk of developing allergic diseases. This was a cross-sectional study, recruiting children with a history of KD, together with well sibling controls. All children underwent the standardized core ISAAC questionnaire for allergy, physical examination and skin prick test evaluation. McNemar's test was employed to evaluate the effect of Kawasaki disease on allergy. Multivariable analysis based on mixed-effects logistic regression model was used to adjust for potential confounding effect of age and gender. One hundred and eighty-six children (93 KD sibling pairs) completed the above evaluation. Allergic rhinitis was more common in patients with KD (crude OR 2.40; 95% CI 1.11-5.62, p=0.024) when compared with controls. The effect was further intensified after accounting for the potential confounding effect of age and gender (adjusted OR=2.90; 95% CI 1.27-6.60). Children in whom KD occurred beyond the age of 12 months had more allergic rhinitis (crude OR 4.00, 95% CI 1.29-16.44, p=0.012), 'any' allergies (crude OR 3.75, 95% CI 1.19-15.52, p=0.019) and Blomia tropicalis sensitization (crude OR 2.57, 95% CI 1.02-7.28, p=0.043) when compared with their sibling controls. Interestingly, children in whom KD course resulted in no coronary artery abnormalities have more allergic rhinitis (crude OR 8.50, 95% CI 2.02-75.85, p=0.003) and 'any' allergies (crude OR 5.00, 95% CI 1.41-26.94, p=0.011), when compared with their sibling controls. Kawasaki disease may be a risk factor for subsequent allergic diseases. We postulate that KD occurs more frequently in children at risk of immune disequilibrium, with an abnormal inflammatory response initially, and subsequently more allergic manifestations. © 2011 John Wiley & Sons A/S.

  18. A case of incomplete and refractory Kawasaki disease : diagnostic ...

    African Journals Online (AJOL)

    disease.[4,5] Not all cases ful l all the diagnostic criteria, and these are termed incomplete KD.[6,7] We report a case of incomplete KD that was refractory to the recommended standard treatment with intravenous immunoglobulin (IVIG), but responded to treatment with intravenous methylprednisolone. e case highlights the.

  19. Hemodynamic simulations in coronary aneurysms of a patient with Kawasaki Disease

    Science.gov (United States)

    Sengupta, Dibyendu; Marsden, Alison; Burns, Jane

    2010-11-01

    Kawasaki Disease is the leading cause of acquired pediatric heart disease, and can cause large coronary artery aneurysms in untreated cases. A simulation case study has been performed for a 10-year-old male patient with coronary aneurysms. Specialized coronary boundary conditions along with a lumped parameter heart model mimic the interactions between the ventricles and the coronary arteries, achieving physiologic pressure and flow waveforms. Results show persistent low shear stress in the aneurismal regions, and abnormally high shear at the aneurysm neck. Correlation functions have been derived to compare wall shear stress and wall shear stress gradients with recirculation time with the idea of localizing zones of calcification and thrombosis. Results are compared with those of an artificially created normal coronary geometry for the same patient. The long-term goal of this work is to develop links between hemodynamics and thrombotic risk to assist in clinical decision-making.

  20. CLINICAL AND EPIDEMIOLOGICAL CHARACTERISTICS OF KAWASAKI DISEASE WITH SPECIAL REFERENCE TO CARDIAC INVOLVEMENT

    Directory of Open Access Journals (Sweden)

    Jayanta Kumar

    2016-01-01

    Full Text Available BACKGROUND Kawasaki disease (KD is an acute medium vessel vasculitis with multisystem involvement especially in young children of unknown etiology and present as acute febrile illness. It is the leading cause of acquired heart disease in children across the globe. OBJECTIVES We aimed to evaluate the epidemiological characteristics, clinical manifestations and cardiac involvement of KD in children residing in Tripura, a North Eastern State of India. MATERIALS AND METHODS In this retrospective study, we reviewed the medical records of all children with KD who had been admitted in Pediatric ward of Tripura Medical College & DR BRAM Teaching Hospital, Agartala from March 2007 to February 2015. Data regarding clinical, epidemiological characteristics, Cardiac involvement, laboratory parameter, management, and the outcome of disease for each patient were obtained. The patients were divided into cardiac and non-cardiac groups based on echocardiographic results. RESULTS In total, 30 patients with KD (18boys and 12 girls were enrolled in this study. The male to female ratio was 1.5:1. The median age at diagnosis was 15 months, and the diagnosis was made after a mean of 8.1 days of fever. A seasonal peak during the winter-spring months was observed. 23 (76.6% had classical presentation of KD. Fever, polymorphs, skin rash, conjunctivitis, changes in the oropharynx were the most common manifestations. Cardiac involvement was detected in 7(23.3%%, with coronary artery abnormalities (CAA and 26(86.5% patient showed ECG abnormality. Patients were treated with immunoglobulin and aspirin. The CAA regressed in all patients but one persisted even after 12 months. CONCLUSION Kawasaki disease is not rare in Tripura. The age, gender distribution and clinical findings are similar to that of other reports. Patients with cardiac abnormalities had delayed treatment and prolonged hospital stays

  1. Bull's eye dermatoscopy pattern at bacillus Calmette-Guérin inoculation site correlates with systemic involvements in patients with Kawasaki disease.

    Science.gov (United States)

    Tseng, Han-Chi; Ho, Ji-Chen; Guo, Mindy Ming-Huey; Lo, Mao-Hung; Hsieh, Kai-Sheng; Tsai, Wen-Chien; Kuo, Ho-Chang; Lee, Chih-Hung

    2016-09-01

    For the past decades, although the rash at the bacillus Calmette-Guérin (BCG) inoculation site has been recognized as a diagnostic clue in Kawasaki disease, the present study is the first known one attempting to characterize BCG inoculation by dermatoscopy in Kawasaki disease and correlate the grade of BCG reaction with systemic involvement. Thirty-four patients diagnosed with Kawasaki disease by pediatric specialists were enrolled. We performed detailed history taking, laboratory examination, physical examination and dermatoscopy examinations. Based on the BCG reaction pattern by dermatoscopy, we were able to characterize three patterns: (A) Bull's eye pattern in 18 patients; (B) faint homogenous erythema in nine; and (C) central white patch in seven. Patients from group A exhibited the highest elevation of blood aspartate aminotransferase levels (P Kawasaki disease. © 2016 Japanese Dermatological Association.

  2. Persistent high fever for more than 10 days during acute phase is a risk factor for endothelial dysfunction in children with a history of Kawasaki disease.

    Science.gov (United States)

    Mori, Yasuhiko; Katayama, Hiroshi; Kishi, Kanta; Ozaki, Noriyasu; Shimizu, Tatsuo; Tamai, Hiroshi

    2016-07-01

    Endothelial dysfunction has previously been reported in children with a history of Kawasaki disease, but the determinants of endothelial function in Kawasaki disease patients are still unknown. In this study, we investigated endothelial function in Kawasaki disease patients and attempted to identify risk factors for persistent endothelial dysfunction. Using high-resolution ultrasound, we measured the percent flow-mediated dilatation, an arterial response to reactive hyperemia, to evaluate endothelial function in 67 patients with a history of Kawasaki disease and 28 age- and sex-matched control subjects. We divided the Kawasaki disease patients into a group with impaired endothelial function (the percent flow-mediated dilatation below -2 standard deviations of the control group) and a group with normal endothelial function (the percent flow-mediated dilatation more than -2 standard deviations of control). Logistic multiple regression analysis was performed to identify independent predictors of impaired endothelial function. In Kawasaki disease patients, the percent flow-mediated dilatation was significantly lower than in the control subjects (9.8±3.6%, compared with 13.1±3.4%, pKawasaki disease patients (3 patients with coronary artery lesions and 10 patients without coronary artery lesions), the percent flow-mediated dilatation was below -2 standard deviations of control. Logistic multiple regression analysis showed that a febrile period of longer than 10 days during the acute phase was the significant risk factor for endothelial dysfunction (odds ratio: 8.562; 95% confidence interval: 1.366-53.68). Presence of coronary artery lesions was not a determinant of endothelial dysfunction. Systemic endothelial dysfunction exists in children with a history of Kawasaki disease, and a febrile period of longer than 10 days during the acute phase is an independent predictor of endothelial dysfunction irrespective of coronary artery involvement. Copyright © 2015 Japanese

  3. Strict management of a pregnant patient with giant coronary artery aneurysm due to Kawasaki disease.

    Science.gov (United States)

    Taniguchi, Kosuke; Ono, Hiroshi; Sato, Anna; Kinomoto, Satoko; Tagawa, Naomi; Umehara, Nagayoshi; Kato, Hitoshi; Sago, Haruhiko

    2015-10-01

    Coronary artery aneurysms (CAA) may occur in Kawasaki disease (KD). Patients with giant CAA (diameter >8 mm), in particular, have higher risk of myocardial infarction. Previous reports have demonstrated the necessity of anticoagulation therapy in such cases. The management of patients with KD complicated by giant CAA later in life, however, remains controversial. Here, we describe the strict management in the case of a 28-year-old pregnant Japanese woman with KD with giant CAA (diameter, 11 mm). Instead of warfarin, the patient was given low-dose aspirin and i.v. unfractionated heparin during pregnancy to prevent thrombosis in the giant CAA. At 38 weeks of gestation, she had spontaneous delivery of a healthy baby. No thrombotic or bleeding complications were observed. The strict anticoagulation therapy resulted in successful pregnancy and delivery without any adverse events. © 2015 Japan Pediatric Society.

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

    Science.gov (United States)

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

    2016-01-05

    Kawasaki disease (KD) primarily affects children 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 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.

  5. The incidence of Kawasaki disease after vaccination within the UK pre-school National Immunisation Programme: an observational THIN database study.

    Science.gov (United States)

    Hall, Gillian C; Tulloh, Robert Mr; Tulloh, Louise E

    2016-11-01

    To provide expected incidence rates of Kawasaki disease after vaccination in routine clinical practice and as recommended within a pre-school National Immunisation Programme (NIP). A post-immunisation risk period when Kawasaki disease onset might be associated with vaccination was defined as 28 days. Immunisation records for children under 6 years were identified from The Health Improvement Network (THIN) database of electronic UK primary health care records (2008-2012) and linked to previously validated cases of Kawasaki disease with an assigned date of onset. Kawasaki disease incidence in the risk period after a complete NIP recommended set of vaccinations was estimated for five vaccination stages individually and in total. A total of 642 170 complete pre-school immunisation stages from 275 986 children were included. Six cases of Kawasaki disease had onset in the risk period after any NIP stage providing an incidence of 12.8 per 100 000 person years (95%CI 5.7, 28.4). The incidence after any single immunisation stage ranged from 0 to 27.4 (95%CI 8.8, 84.8) per 100 000 person years. There were few cases of Kawasaki disease in the risk period after any NIP vaccination combination. The incidence rates will aid in the interpretation of clinical trials and post-marketing surveillance of new vaccines. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  6. Comparative effectiveness of intravenous immunoglobulin for children with Kawasaki disease: a nationwide cohort study.

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    Ming-Chih Lin

    Full Text Available INTRODUCTION: Different immunoglobulin manufacturing processes may influence its effectiveness for Kawasaki disease. However, nationwide studies with longitudinal follow-up are still lacking. The aim of this study was to evaluate the comparative effectiveness of immunoglobulin preparations from a nationwide perspective. MATERIALS AND METHODS: This is a nationwide retrospective cohort study with a new user design. Data came from the National Health Insurance Research Database of Taiwan. From 1997 to 2008, children under 2 years old who received immunoglobulin therapy for the first time under the main diagnosis of Kawasaki disease were enrolled. The manufacturing processes were divided into β-propiolactonation, acidification and those containing IgA. The endpoints were immunoglobulin non-responsiveness, acute aneurysm, prolonged use of anti-platelets or anti-coagulants, and recurrence. RESULTS: In total, 3830 children were enrolled. β-propiolactonation had a relative risk of 1.45 (95% CI 1.08~1.94 of immunoglobulin non-responsiveness, however, the relative risks for acidification and containing IgA were non-significant. For acute aneurysms, acidification had a relative risk of 1.49 (95% CI 1.17~1.90, however the relative risks for β-propiolactonation and containing IgA were non-significant. For prolonged use of anti-platelets or anti-coagulants, β-propiolactonation had a relative risk of 1.44 (95% CI 1.18~1.76, and acidification protected against them both with a relative risk of 0.82 (95% CI 0.69~0.97, whereas the relative risk for containing IgA was non-significant. For recurrence, all three factors were non-significant. CONCLUSIONS: The effectiveness of immunoglobulin may differ among different manufacturing processes. β-propiolactonation had a higher risk of treatment failure and prolonged use of anti-platelets or anti-coagulants. Acidification may increase the risk of acute coronary aneurysms.

  7. 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...

  8. Enfermedad de Kawasaki

    Directory of Open Access Journals (Sweden)

    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.

  9. Kawasaki Disease

    Science.gov (United States)

    ... of heart problems. Do not give your child aspirin without your doctor’s orders. It can cause side effects, depending on your child’s health history.Your doctor might give your child immunoglobulin. This ...

  10. Predictive value of red blood cell distribution width for coronary artery lesions in patients with Kawasaki disease.

    Science.gov (United States)

    Xu, Haiyan; Fu, Songling; Wang, Wei; Zhang, Qing; Hu, Jian; Gao, Lichao; Zhu, Weihua; Gong, Fangqi

    2016-08-01

    Recent studies have shown that elevated red blood cell distribution width is associated with poor outcome in cardiovascular diseases. In order to assess the predictive value of red blood cell distribution width, before treatment with intravenous immunoglobulins, for coronary artery lesions in patient with Kawasaki disease, we compared 83 patients with coronary artery lesions and 339 patients without coronary artery lesions before treatment with intravenous immunoglobulin. Clinical, echocardiographic, and biochemical values were evaluated along with red blood cell distribution width. A total of 422 consecutive patients with Kawasaki disease were enrolled into our study. According to receiver operating characteristic curve analysis, the optimal red blood cell distribution width cut-off value for predicting coronary artery lesions was 14.55% (area under the curve was 0.721; p=0.000); eighty-three patients (19.7%) had coronary artery lesions, and 70% of the patients with coronary artery lesions had red blood cell distribution width level >14.55%. Logistic regression analysis revealed that fever duration >14 days (odds ratio was 3.42, 95% confidence interval was 1.27-9.22; p=0.015), intravenous immunoglobulin resistance (odds ratio was 2.33, 95% confidence interval was 1.02-5.29; p=0.04), and red blood cell distribution width >14.55% (odds ratio was 3.49, 95% confidence interval was 2.01-6.05; p=0.000) were independent predictors of coronary artery lesions in patients with Kawasaki disease. In Conclusion, red blood cell distribution width may be helpful for predicting coronary artery lesions in patients with Kawasaki disease.

  11. 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.

  12. The outcome of short-term low-dose aspirin treatment in Kawasaki disease based on inflammatory markers

    OpenAIRE

    Yoo, Jae Won; Kim, Ji Mok; Kil, Hong Ryang

    2017-01-01

    Purpose Previously, Kawasaki disease (KD) treatment with low-dose aspirin was administered for 6?8 weeks after the acute phase. However, inflammatory marker levels normalize before 6?8 weeks. In this study, we aimed to investigate the clinical outcome of short-term low-dose aspirin treatment based on inflammatory and thrombotic marker levels. Methods We performed a retrospective review of the medical records of patients with KD who were hospitalized at Chungnam National University Hospital be...

  13. Coronary artery thrombus resulting in sudden cardiac death in an infant with Kawasaki disease and giant coronary artery aneurysms

    Directory of Open Access Journals (Sweden)

    Umakumaran Ponniah

    2013-01-01

    Full Text Available We report a case of a six-month-old Hispanic male infant who had Kawasaki disease and coronary artery aneurysms on echocardiography. He died suddenly five months later in spite of aggressive medical therapy. Autopsy showed extensive coronary artery thrombosis. Giant coronary artery aneurysms need diligent follow up as they pose significant risks including risk of thrombus, myocardial infarction and sudden death.

  14. Tumor necrosis factor antagonist-induced psoriasis in a 3-year-old boy with Kawasaki disease.

    Science.gov (United States)

    Geller, Lauren; Kellen, Roselyn

    2017-02-15

    Tumor necrosis factor (TNF) antagonists have beenknown to trigger new-onset psoriasis in adult andpediatric patients. Here we report a case of TNFantagonist-induced psoriasis in a 3-year-old boytreated with infliximab for Kawasaki disease. Ourpatient is a 3-year-old boy with Kawasaki diseaseunresponsive to intravenous immunoglobulinwho was then treated with one dose of infliximab.A few days later he developed psoriatic plaqueson the face and extremities. The psoriatic plaqueswere treated with topical calcineurin inhibitors andtopical corticosteroids, with marked improvement.Prior reports of TNF antagonist-induced psoriasis inthe pediatric population have been in children withinflammatory bowel disease or juvenile idiopathicarthritis. To the best of our knowledge, this is thefirst case of TNF antagonist-induced psoriasis ina pediatric patient with Kawasaki disease, andthe youngest patient to date. Although we donot fully understand the mechanism behind thisphenomenon, in vitro studies have implicated theimportance of interferon-α, a pro-inflammatorycytokine, and plasmacytoid dendritic cells. Furtherresearch is necessary to understand who is at riskfor this condition and the molecular basis for thisparadoxical reaction.

  15. Oxidative stress and Kawasaki disease: how is oxidative stress involved from the acute stage to the chronic stage?

    Science.gov (United States)

    Yahata, Tomoyo; Hamaoka, Kenji

    2017-01-01

    Inflammation and oxidative stress are closely related. Further, oxidative stress plays an important role in the pathology of inflammation-based Kawasaki disease. An excessive in vivo production of reactive oxygen species increases oxidative stress in the body, which triggers an endless vicious spiral of inflammation reactions and reactive oxygen metabolites. This presumably forms diffuse vasculitis in the acute phase. Acute inflammation and oxidative stress can be rapidly controlled by treatments; however, they may remain for a long time. This has recently been identified as a problem in the chronic phase of Kawasaki disease. Generally, the presence of vascular inflammation and oxidative stress impairs blood vessels, leading to the onset of atherosclerosis, which is a widely recognized risk. The current discussion focuses on whether the same is valid for blood vessels in the chronic phase of Kawasaki disease. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  16. Platelet Endothelial Cell Adhesion Molecule-1 Gene Polymorphisms are Associated with Coronary Artery Lesions in the Chronic Stage of Kawasaki Disease.

    Science.gov (United States)

    Lu, Wen-Hsien; Huang, Sin-Jhih; Yuh, Yeong-Seng; Hsieh, Kai-Sheng; Tang, Chia-Wan; Liou, Huei-Han; Ger, Luo-Ping

    2017-05-01

    Kawasaki disease is the most common cause of pediatric acquired heart disease. The role of platelet endothelial cell adhesion molecule-1 in the inflammatory process has been documented. To date, no report has investigated the relationship between coronary artery lesions of Kawasaki disease and platelet endothelial cell adhesion molecule-1 polymorphisms. A total of 114 Kawasaki disease children with coronary artery lesions and 185 Kawasaki disease children without coronary artery lesions were recruited in this study. The TaqMan assay was conducted to identify the genotype in this case-control study. In three single nucleotide polymorphisms (Leu125Val, Ser563Asn, and Arg670Gly) of platelet endothelial cell adhesion molecule-1, we found that the Leu-Ser-Arg haplotype was associated with a significantly increased risk for coronary artery lesions in the chronic stage (odds ratio 3.05, 95% confidence interval 1.06-8.80, p = 0.039), but not for coronary artery lesions in the acute stage. Analysis based on the diplotypes of platelet endothelial cell adhesion molecule-1 also showed that Kawasaki disease with one or two alleles of Leu-Ser-Arg had a significantly increased risk of chronic coronary artery lesions (odds ratio 3.38, 95% confidence interval 1.11-10.28, p = 0.032) and had increased platelet counts after Kawasaki disease was diagnosed, as compared to those with other diplotypes. The haplotype of platelet endothelial cell adhesion molecule-1 Leu-Ser-Arg might be associated with the increased platelet counts and the following risk of chronic coronary artery lesions in a dominant manner in Kawasaki disease.

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

    Science.gov (United States)

    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.

  18. Family-based association analysis implicates IL-4 in susceptibility to Kawasaki disease.

    Science.gov (United States)

    Burns, J C; Shimizu, C; Shike, H; Newburger, J W; Sundel, R P; Baker, A L; Matsubara, T; Ishikawa, Y; Brophy, V A; Cheng, S; Grow, M A; Steiner, L L; Kono, N; Cantor, R M

    2005-08-01

    Several compelling lines of evidence suggest an important influence of genetic variation in susceptibility to Kawasaki disease (KD), an acute vasculitis that causes coronary artery aneurysms in children. We performed a family-based genotyping study to test for association between KD and 58 genes involved in cardiovascular disease and inflammation. By analysis of a cohort of 209 KD trios using the transmission disequilibrium test, we documented the asymmetric transmission of five alleles including the interleukin-4 (IL-4) C(-589)T allele (P=0.03). Asymmetric transmission of the IL-4 C(-589)T was replicated in a second, independent cohort of 60 trios (P=0.05, combined P=0.002). Haplotypes of alleles in IL-4, colony-stimulating factor 2 (CSF2), IL-13, and transcription factor 7 (TCF7), all located in the interleukin gene cluster on 5q31, were also asymmetrically transmitted. The reported associations of KD with atopic dermatitis and allergy, elevated serum IgE levels, eosinophilia, and increased circulating numbers of monocyte/macrophages expressing the low-affinity IgE receptor (FCepsilonR2) may be related to effects of IL-4. Thus, the largest family-based genotyping study of KD patients to date suggests that genetic variation in the IL-4 gene, or regions linked to IL-4, plays an important role in KD pathogenesis and disease susceptibility.

  19. GM-CSF primes cardiac inflammation in a mouse model of Kawasaki disease

    Science.gov (United States)

    McKenzie, Brent S.

    2016-01-01

    Kawasaki disease (KD) is the leading cause of pediatric heart disease in developed countries. KD patients develop cardiac inflammation, characterized by an early infiltrate of neutrophils and monocytes that precipitates coronary arteritis. Although the early inflammatory processes are linked to cardiac pathology, the factors that regulate cardiac inflammation and immune cell recruitment to the heart remain obscure. In this study, using a mouse model of KD (induced by a cell wall Candida albicans water-soluble fraction [CAWS]), we identify an essential role for granulocyte/macrophage colony-stimulating factor (GM-CSF) in orchestrating these events. GM-CSF is rapidly produced by cardiac fibroblasts after CAWS challenge, precipitating cardiac inflammation. Mechanistically, GM-CSF acts upon the local macrophage compartment, driving the expression of inflammatory cytokines and chemokines, whereas therapeutically, GM-CSF blockade markedly reduces cardiac disease. Our findings describe a novel role for GM-CSF as an essential initiating cytokine in cardiac inflammation and implicate GM-CSF as a potential target for therapeutic intervention in KD. PMID:27595596

  20. 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; Lee, Kyung-Yil; Hwang, Ja-Young; Rhim, Jung-Woo; Song, Min Seob; Lee, Hyoung Doo; Kim, Dong Soo; Kil, Hong-Ryang; Kim, Gi-Beom; Yoon, Kyung Lim; Kim, Jong-Duk; Lee, Jae-Moo; Odam, Miranda; Christiansen, Frank; Goldwater, Paul; Curtis, Nigel; Palasanthiran, Pamela; Ziegler, John; Nissan, Michael; Kuipers, Irene M.; Ottenkamp, Jaap J.; Geissler, Judy; Biezeveld, Maarten; Filippini, Luc; Ling, Ling; Wright, Victoria J.; Brogan, Paul; Klein, Nigel; Shah, Vanita; Dillon, Michael; Booy, Robert; Shingadia, Delane; Bose, Anu; Mukasa, Thomas; Tulloh, Robert; Michie, Colin

    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

  1. Kawasakis syndrom hos voksne

    DEFF Research Database (Denmark)

    Roed, Casper; Skinhøj, Peter

    2009-01-01

    Kawasaki syndrome (KS), which is febrile systemic vasculitis complicated by coronary aneurysms, was initially described in children, but an increasing number of case-reports address adult-KS. The disease seems to be infectious of origin, but the causative agent has not been established. The debut...

  2. QT Dispersion and T Wave Peak-to-end Interval Dispersion in Children with Kawasaki Disease.

    Science.gov (United States)

    Amoozgar, Hamid; Ahmadipour, Maryam; Amirhakimi, Anis

    2013-09-01

    The main complication of Kawasaki disease is the Coronary Artery (CA) involvement and long term follow up of patients depends on the severity of coronary arterial aneurysms, ischemia, and thrombosis. Early diagnosis of these complications can lead to a more desirable outcome for patients. Myocardial ischemia can prolong QT dispersion and increase the risk of cardiac arrhythmias as well as sudden cardiac arrests. Also, T wave peak-to-end (Tp-Te) interval dispersion, which provides a valuable index of transmural dispersion of repolarization, can trigger the arrhythmia. We evaluated the non-corrected QT interval dispersion (QTD) and the corrected QT (QTc) dispersion and measured Tp-Te interval dispersion in 49 Iranian children (28 males and 21 females) with the diagnosis of Kawasaki disease (KD) in the acute phase and 49 age-matched controls in a prospective study from 2009 to 2012. Student's t-test and Pearson correlation were used to analyze the data. All the statistical analyses were performed through the SPSS 16. Besides, P<0.05 was considered as statistically significant. Patients with KD had significantly longer QTc dispersion (0.099±0.055 s versus. 0.040±0.018 s; P<0.001), non-corrected QT dispersion (0.075±0.046 versus 0.042±0.019; P<0.001), and Tp-Te dispersion (0.047±0.054 versus 0.022±0.011; P=0.015). The patients with elevation in white blood cell count (above 15000) had a statistically significant increased in QTD (P=0.011). No significant correlation was found between coronary involvement and repolarization indexes. In conclusion, the QT interval (corrected or non-corrected) and the Tp-Te dispersion significantly increased in the patients with KD which shows repolarization changes during the acute phase of KD. However, there is no correlation between the QT interval and the coronary involvement.

  3. QT Dispersion and T Wave Peak–to–end Interval Dispersion in Children with Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Hamid Amoozgar

    2013-09-01

    Full Text Available Background: The main complication of Kawasaki disease is the Coronary Artery (CA involvement and long term follow up of patients depends on the severity of coronary arterial aneurysms, ischemia, and thrombosis. Early diagnosis of these complications can lead to a more desirable outcome for patients. Myocardial ischemia can prolong QT dispersion and increase the risk of cardiac arrhythmias as well as sudden cardiac arrests. Also, T wave peak–to–end (Tp-Te interval dispersion, which provides a valuable index of transmural dispersion of repolarization, can trigger the arrhythmia. Materials and Methods: We evaluated the non-corrected QT interval dispersion (QTD and the corrected QT (QTc dispersion and measured Tp–Te interval dispersion in 49 Iranian children (28 males and 21 females with the diagnosis of Kawasaki disease (KD in the acute phase and 49 age-matched controls in a prospective study from 2009 to 2012. Student’s t-test and Pearson correlation were used to analyze the data. All the statistical analyses were performed through the SPSS 16. Besides, P<0.05 was considered as statistically significant. Results: Patients with KD had significantly longer QTc dispersion (0.099±0.055 s versus. 0.040±0.018 s; P<0.001, non-corrected QT dispersion (0.075±0.046 versus 0.042±0.019; P<0.001, and Tp-Te dispersion (0.047±0.054 versus 0.022±0.011; P=0.015. The patients with elevation in white blood cell count (above 15000 had a statistically significant increased in QTD (P=0.011. No significant correlation was found between coronary involvement and repolarization indexes. Conclusions: In conclusion, the QT interval (corrected or non-corrected and the Tp-Te dispersion significantly increased in the patients with KD which shows repolarization changes during the acute phase of KD. However, there is no correlation between the QT interval and the coronary involvement.

  4. Sudden death as a late sequel of Kawasaki disease: postmortem CT demonstration of coronary artery aneurysm.

    Science.gov (United States)

    Okura, Naoki; Okuda, Takahisa; Shiotani, Seiji; Kohno, Mototsugu; Hayakawa, Hideyuki; Suzuki, Atsuko; Kawasaki, Tomisaku

    2013-02-10

    Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that primarily affects the coronary artery (CA) and presents during childhood. The characteristic coronary arterial lesion of KD is an aneurysm. Ischemic heart disease derived from a CA aneurysm is experienced approximately two decades after the onset of acute KD. In recent years, the primary issue of concern has been asymptomatic adults with a CA aneurysm caused by undiagnosed KD. We present a case of sudden death as a late KD sequel in a young adult. A postmortem CT scan revealed a coarse calcification of a left anterior descending CA aneurysm, which was confirmed at the time of autopsy. A postmortem CT scan is useful in cases of sudden death where the detection of a calcified CA aneurysm would suggest to the forensic pathologist that the deceased suffered from a late sequel of KD. The use of screening postmortem CT scans for young people may detect cases of unsuspected CA aneurysms, raising the possibility of untreated KD. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  5. Platelet count and erythrocyte sedimentation rate are good predictors of Kawasaki disease: ROC analysis.

    Science.gov (United States)

    Xiu-Yu, Song; Jia-Yu, Huang; Qiang, Hong; Shu-Hui, Dai

    2010-01-01

    Kawasaki disease (KD) is the leading cause of acquired pediatric cardiac disease and requires a timely diagnosis. Available effective therapy is ideally administered within 10 days of illness diagnosis. Recent reports of several laboratory tests in KD have been published. In this study, we aimed to evaluate the sensitivity and specificity of several laboratory tests. We performed a retrospective study of consecutive patients diagnosed with KD from January to December 2008. We studied the sensitivity and specificity of several different tests [T-cell subgroups, platelet count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP)] to predict KD using receiver operator characteristic curve analysis. No significant difference was demonstrated in T-cell subgroups between patients with KD and referent patients (P>0.05). However, platelet count, ESR, and CRP were significantly higher in patients with KD than in referent patients (P<0.05). ESR showed a sensitivity of 93.9% and specificity of 83.3% with a cut-off of 15  mm/hr (area under the curve [AUC], 89.1%; P=0.03). Platelet count showed a sensitivity of 70.6% and specificity of 75% with a cut-off of 336.5×10(9)/l (AUC, 71.2%; P=0.03). These results indicate that platelet count and ESR are good predictors of KD. © 2010 Wiley-Liss, Inc.

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

    Directory of Open Access Journals (Sweden)

    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.

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

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    Deniz Yeter

    2016-01-01

    Full Text Available 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.

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

    Science.gov (United States)

    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 (pKawasaki disease were significantly higher than those in healthy subjects (pKawasaki disease than that in healthy children (4.5%); there was significant difference between the two groups (pKawasaki disease in children through NF-kB signaling pathways.

  9. [Kawasaki disease is more prevalent in rural areas of Catalonia (Spain)].

    Science.gov (United States)

    Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estibaliz; Calzada-Hernandez, Joan; Rodó, Xavier; Morguí, Josep-Antón

    2017-10-01

    Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region. Observational population-based study including all Pediatric Units in Catalan hospitals, between 2004 and 2014. A 12-month (March 2013-March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. The rest of the data was retrieved retrospectively. Data from 399 patients over the 10-year study period was analyzed. Among the total KD patients, 353 (88.5%) lived in non-rural areas and 46 (11.5%) in rural areas. It was found that there is a significant difference (P<.001) between the percentage of rural population observed in patients with KD (11.5%), and the expected 5% of the Catalan population. This is the first population-based study showing significant differences on KD incidence rates between rural and non-rural areas. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

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

    Science.gov (United States)

    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.

  11. Transluminal Attenuation Gradient for Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Grande Gutierrez, Noelia; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2014-11-01

    Kawasaki Disease (KD) can result in coronary aneurysms in up to 25% of patients if not treated early putting patients at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines for administering anti-coagulation therapy currently rely on anatomy alone. Previous studies including patient specific modeling and computer simulations in KD patients have suggested that hemodynamic data can predict regions susceptible to thrombus formation. In particular, high Particle Residence Time gradient (PRTg) regions have shown to correlate with regions of thrombus formation. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length. TAG has been used for characterizing coronary artery stenoses, however this approach has not yet been used in aneurysmal vessels. The aim of this study is to analyze the correlation between TAG and PRTg in KD patients with aneurysms and evaluate the use of TAG as an index to quantify thrombotic risk. Patient specific anatomic models for fluids simulations were constructed from CT angiographic image data from 3 KD aneurysm patients and one normal control. TAG values for the aneurysm patients were markedly lower than for the non-aneurysmal patient (mean -18.38 vs. -2). In addition, TAG values were compared to PRTg obtained for each patient. Thrombotic risk stratification for KD aneurysms may be improved by incorporating TAG and should be evaluated in future prospective studies.

  12. Assessment Of Coronary Artery Aneurysms Using Transluminal Attenuation Gradient And Computational Modeling In Kawasaki Disease Patients

    Science.gov (United States)

    Grande Gutierrez, Noelia; Kahn, Andrew; Shirinsky, Olga; Gagarina, Nina; Lyskina, Galina; Fukazawa, Ryuji; Owaga, Shunichi; Burns, Jane; Marsden, Alison

    2015-11-01

    Kawasaki Disease (KD) can result in coronary artery aneurysms (CAA) in up to 25% of patients, putting them at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines recommend CAA diameter >8 mm as the arbitrary criterion for initiating systemic anticoagulation. KD patient specific modeling and flow simulations suggest that hemodynamic data can predict regions at increased risk of thrombosis. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length and has been proposed as a non-invasive method for characterizing coronary stenosis from CT Angiography. We hypothesized that CAA abnormal flow could be quantified using TAG. We computed hemodynamics for patient specific coronary models using a stabilized finite element method, coupled numerically to a lumped parameter network to model the heart and vascular boundary conditions. TAG was quantified in the major coronary arteries. We compared TAG for aneurysmal and normal arteries and we analyzed TAG correlation with hemodynamic and geometrical parameters. Our results suggest that TAG may provide hemodynamic data not available from anatomy alone. TAG represents a possible extension to standard CTA that could help to better evaluate the risk of thrombus formation in KD.

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

    Science.gov (United States)

    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.

  14. Reaction of the BCG Scar in the Acute Phase of Kawasaki Disease in Mexican Children.

    Science.gov (United States)

    Garrido-García, Luis Martín; Castillo-Moguel, Ariel; Vázquez-Rivera, Mirella; Cravioto, Patricia; Fernando, Galván

    2017-10-01

    Kawasaki disease (KD) is an acute self-limited systemic vasculitis that primarily affects children BCG) inoculation site has been reported as a common finding in patients with KD where BCG vaccination is mandatory. The purpose of this study was to evaluate the frequency of reactivation of the BCG in Mexican children diagnosed with KD. A retrospective study of all patients diagnosed with KD from August 1, 1995, to August 31, 2015, at our Institution was performed. The clinical profile, laboratory results, treatment used and coronary artery abnormalities in the BCG reactive and the BCG nonreactive groups were analyzed and compared. We included 399 patients with KD. Ninety-seven (24.3%) had BCG reaction at the inoculation site. The BCG(+) group was younger than the BCG(-) group (P BCG(+) group compared with 65 (21.52%) in the BCG(-) group without statistical significance. The BCG+ group developed coronary artery aneurysms in 37 cases and the BCG(-) group developed coronary artery aneurysms in 111 cases without statistical significance. Multivariate analysis showed that younger age at diagnosis was the only variable associated with a reaction at the BCG inoculation site in patients with KD. In Mexico, a country with a National BCG Vaccination Program and a low incidence of KD, reaction at the BCG inoculation site could be a useful diagnostic sign of KD.

  15. Orbital Cellulitis Presenting as a First Sign of Incomplete Kawasaki Disease

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    Eren Çerman

    2013-12-01

    Full Text Available A 6-year-old boy was referred to our hospital with orbital cellulitis. He had a history of 7 days of fever despite antibiotherapy. At first, he only had pharyngitis and conjunctivitis, but then an orbital mass evolved which restricted the movement of his right eye and there was also periorbital inflammation resembling orbital cellulitis. Examination at presentation revealed conjunctivitis with secretion, periocular inflammation and edema, right-preauricular lymphadenopathy and restriction of upgaze in the right eye. Laboratory findings included a white blood cell count of 19,000 cells per mm3, with 81.5% neutrophils, 15.0% lymphocytes, 1.2% monocytes and 0.4% basophils. The erythrocyte sedimentation rate was 52 mm/h and the C-reactive protein level was 46.3 mg/dl. Magnetic resonance imaging confirmed orbital cellulitis and pansinusitis. Vancomycin (60 mg/kg/day and meropenem (100 mg/kg/day were administered, but desquamation on his fingertips and a rash appeared on the tenth day. A pediatric consultation resulted in a diagnosis of incomplete Kawasaki disease (KD. After administration of aspirin, the orbital inflammation regressed in 3 days. No coronary artery lesions were detected on the first echocardiography, but these did appear 6 weeks later. This confirmed the KD diagnosis.

  16. Coronary magnetic resonance angiography and vessel wall imaging in children with Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Greil, Gerald F.; Hofbeck, Michael; Sieverding, Ludger [University of Tuebingen, Department of Pediatric Cardiology, Children' s Hospital, Tuebingen (Germany); Seeger, Achim; Miller, Stephan; Claussen, Claus D. [University of Tuebingen, Department of Diagnostic Radiology, Tuebingen (Germany); Botnar, Rene M. [Technical University Munich, Department of Nuclear Medicine, Cardiovascular Division, Munich (Germany)

    2007-07-15

    In patients with Kawasaki disease (KD) serial evaluation of the distribution and size of coronary artery aneurysms (CAA) is necessary for risk stratification and therapeutic management. To apply whole-heart coronary MR angiography (CMRA) and black-blood coronary vessel wall imaging in children with KD. Six children (mean age 4.6 years, range 2.5-7.8 years) with KD underwent CMRA using a free-breathing, T2-prepared, three-dimensional steady-state free-precession (3D-SSFP), whole-heart approach with navigator gating and tracking. Vessel walls were imaged with an ECG-triggered and navigator-gated double inversion recovery (DIR) black-blood segmented turbo spin-echo sequence. There was complete agreement between CMRA and conventional angiography (n=6) in the detection of CAA (n=15). Excellent agreement was found between the two techniques in determining the maximal diameter (mean difference 0.2{+-}0.7 mm), length (mean difference 0.1{+-}0.8 mm) and distance from the ostium (mean difference -0.8{+-}2.1 mm) of the CAAs. In all subjects with a CAA, abnormally thickened vessel walls were found (2.5{+-}0.5 mm). CMRA accurately defines CAA in free-breathing sedated children with KD using the whole-heart approach and detects abnormally thickened vessel walls. This technique may reduce the need for serial X-ray coronary angiography, and improve risk stratification and monitoring of therapy. (orig.)

  17. Dobutamine stress radionuclide ventriculography reveals silent myocardial dysfunction in Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Hamamichi, Yuji; Ichida, Fukiko; Tsubata, Shinichi [Toyama Medical and Pharmaceutical Univ., Sugitani (Japan). Faculty of Medicine] (and others)

    2002-01-01

    Dobutamine (DOB) stress radionuclide ventriculography (RVG) is proposed for evaluating left ventricular performance in patients with Kawasaki disease (KD). Dobutamine stress RVG, up to 15{mu}g{center_dot}kg{sup -1}{center_dot}min{sup -1}, was performed in 40 patients with a history of KD, some of whom had a perfusion defect (PD group) on dipyridamole stress thallium-201 myocardial imaging, some of whom had no perfusion defects (NPD group), and some of whom had no coronary artery lesions (C group). No significant differences in either systolic or diastolic indices of the left ventricle at rest were observed between the 3 groups. Although hemodynamic responses were similar in all patients after DOB stress, early diastolic index of the first third filling fraction decreased only in the PD group and was significantly lower in this group compared with the C group (p<0.01). The asynchrony index increased significantly in those patients with coronary stenosis after DOB stress (p<0.05). No serious side-effects were observed during the study. Even late after onset, patients with myocardial ischemia as a result of KD still had impaired early diastolic filling and asynchronous relaxation of the left ventricle. As an alternative to exercise testing, DOB stress RVG is a safe and promising means for serially evaluating left ventricular performance in patients with KD. (author)

  18. CFD-based Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Sengupta, Dibyendu; Kung, Ethan; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2012-11-01

    Coronary aneurysms occur in 25% of untreated Kawasaki Disease (KD) patients and put patients at increased risk for myocardial infarction and sudden death. Clinical guidelines recommend using aneurysm diameter >8 mm as the arbitrary criterion for treating with anti-coagulation therapy. This study uses patient-specific modeling to non-invasively determine hemodynamic parameters and quantify thrombotic risk. Anatomic models were constructed from CT angiographic image data from 5 KD aneurysm patients and one normal control. CFD simulations were performed to obtain hemodynamic data including WSS and particle residence times (PRT). Thrombosis was clinically observed in 4/9 aneurysmal coronaries. Thrombosed vessels required twice as many cardiac cycles (mean 8.2 vs. 4.2) for particles to exit, and had lower mean WSS (1.3 compared to 2.8 dynes/cm2) compared to vessels with non-thrombosed aneurysms of similar max diameter. 1 KD patient in the cohort with acute thrombosis had diameter < 8 mm. Regions of low WSS and high PRT predicted by simulations correlated with regions of subsequent thrombus formation. Thrombotic risk stratification for KD aneurysms may be improved by incorporating both hemodynamic and geometric quantities. Current clinical guidelines to assess patient risk based only on aneurysm diameter may be misleading. Further prospective study is warranted to evaluate the utility of patient-specific modeling in risk stratifying KD patients with coronary aneurysms. NIH R21.

  19. Evaluation of Coronary Involvement in Patients With Kawasaki Disease After Treatment

    Directory of Open Access Journals (Sweden)

    Noor Mohammad Noori

    2016-03-01

    Full Text Available Background Kawasaki disease (KD is a systemic syndrome of inflammatory with unknown cause and usually occurs in children. It is the most common cause of acquired heart disease in industrialized countries. Objectives The aim of this study was to examine the course of cardiovascular involvement in patients with KD after treatment with intravenous immunoglobulin and aspirin. Patients and Methods This retrospective study was performed on all children who were admitted with diagnosis of KD and had received intravenous immunoglobulin therapies and aspirin in Ali Ibne Abi Talib hospital during 2004 to 2013 years. Analysis was done by SPSS-20 with 0.05 as significant level. Results Out of 53 patients, 60% were male. Early involvement of the right and left coronary heart disease were 20.75% and 26% respectively. The frequency of aneurysm for left and right coronary artery was 3 (5% cases. The mean of left coronary artery and right coronary artery diameters in before and after treatment were 2.39 ± 0.84, 1.95 ± 0.72 and 2.12 ± 0.54, 1.81 ± 0.31 mm respectively and they had significant differences (P = 0.001. The means of duration of fever for with and without coronary aneurysm (13 ± 4.2 vs. 8.8 ± 2.8 days, sedimentation rate (121.5 ± 21 vs. 73.9 ± 29.8 mm and alkaline phosphatase (808 ± 101 vs. 476 ± 148 Iu/L in patients with KD had significant difference of P = 0.04, P = 0.03 and P = 0.003 respectively. Conclusions We found coronary artery disease and aortic aneurysm is almost similar to other studies and showed age, duration of fever onset, intravenous immunoglobulin, sedimentation and alkaline phosphatase have a role in coronary involvement and aneurysm.

  20. Neurological manifestations of atypical celiac disease in childhood.

    Science.gov (United States)

    Sel, Çiğdem Genç; Aksoy, Erhan; Aksoy, Ayşe; Yüksel, Deniz; Özbay, Ferda

    2017-09-01

    Various typical and atypical neurological manifestations can be seen as the initial symptoms of celiac disease (CD). We suggest that gluten toxicity is the most suspicious triggering risk factor for probable pathophysiological pathways of neurological involvement in atypical CD. The medical charts of 117 patients diagnosed with atypical CD were retrieved from a tertiary center in Ankara, Turkey. Eight patients reported as having neurologic manifestations as initiating symptoms were evaluated in detail. The initial neurological manifestations of CD in our study included atypical absence, which was reported first in this study, generalized tonic-clonic seizures, complex partial seizures, severe axial hypotonia and down phenotype, multifocal leukoencephalopathy, mild optic neuritis, attention deficit hyperactivity disorder, and short duration headaches. Seizures mostly emphasizing atypical absence could be the initial presentation manifestation of CD, first described in this literature. Gluten toxicity could be one of the most powerful triggering factors for developing epilepsy in CD. Learning disorders such as attention deficit hyperactivity disorder, short duration headaches, mild optic neuritis, encephalopathy, and DS could also be the initial neurological manifestations of atypical CD. A gluten-restricted diet may improve neurological complaints, epileptic discharges, and neuropsychiatric symptoms. All we found may be a small part of the full range of neurological disorders of unknown origin related to CD. Clinical suspicion should be the rule for accurate diagnosis of the disease.

  1. Transforming growth factor-beta signaling pathway in patients with Kawasaki disease.

    Science.gov (United States)

    Shimizu, Chisato; Jain, Sonia; Davila, Sonia; Hibberd, Martin L; Lin, Kevin O; Molkara, Delaram; Frazer, Jeffrey R; Sun, Shelly; Baker, Annette L; Newburger, Jane W; Rowley, Anne H; Shulman, Stanford T; Davila, Sonia; Burgner, David; Breunis, Willemijn B; Kuijpers, Taco W; Wright, Victoria J; Levin, Michael; Eleftherohorinou, Hariklia; Coin, Lachlan; Popper, Stephen J; Relman, David A; Fury, Wen; Lin, Calvin; Mellis, Scott; Tremoulet, Adriana H; Burns, Jane C

    2011-02-01

    Transforming growth factor (TGF)-β is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-β signaling might be important in KD susceptibility and disease outcome. We investigated genetic variation in 15 genes belonging to the TGF-β pathway in a total of 771 KD subjects of mainly European descent from the United States, the United Kingdom, Australia, and the Netherlands. We analyzed transcript abundance patterns using microarray and reverse transcriptase-polymerase chain reaction for these same genes, and measured TGF-β2 protein levels in plasma. Genetic variants in TGFB2, TGFBR2, and SMAD3 and their haplotypes were consistently and reproducibly associated with KD susceptibility, coronary artery aneurysm formation, aortic root dilatation, and intravenous immunoglobulin treatment response in different cohorts. A SMAD3 haplotype associated with KD susceptibility replicated in 2 independent cohorts and an intronic single nucleotide polymorphism in a separate haplotype block was also strongly associated (A/G, rs4776338) (P=0.000022; odds ratio, 1.50; 95% confidence interval, 1.25 to 1.81). Pathway analysis using all 15 genes further confirmed the importance of the TGF-β pathway in KD pathogenesis. Whole-blood transcript abundance for these genes and TGF-β2 plasma protein levels changed dynamically over the course of the illness. These studies suggest that genetic variation in the TGF-β pathway influences KD susceptibility, disease outcome, and response to therapy, and that aortic root and coronary artery Z scores can be used for phenotype/genotype analyses. Analysis of transcript abundance and protein levels further support the importance of this pathway in KD pathogenesis.

  2. Transforming Growth Factor-β Signaling Pathway in Patients with Kawasaki Disease

    Science.gov (United States)

    Shimizu, Chisato; Jain, Sonia; Lin, Kevin O.; Molkara, Delaram; Frazer, Jeffrey R.; Sun, Shelly; Baker, Annette L.; Newburger, Jane W.; Rowley, Anne H.; Shulman, Stanford T.; Davila, Sonia; Hibberd, Martin L.; Burgner, David; Breunis, Willemijn B.; Kuijpers, Taco W.; Wright, Victoria J.; Levin, Michael; Eleftherohorinou, Hariklia; Coin, Lachlan; Popper, Stephen J.; Relman, David A.; Fury, Wen; Lin, Calvin; Mellis, Scott; Tremoulet, Adriana H.; Burns, Jane C.

    2011-01-01

    Background Transforming growth factor (TGF)-β is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-β signaling might be important in KD susceptibility and disease outcome. Methods and Results We investigated genetic variation in 15 genes belonging to the TGF-β pathway in a total 771 KD subjects of mainly European descendent from the US, UK, Australia and the Netherlands. We analyzed transcript abundance patterns using microarray and RT-PCR for these same genes and measured TGF-β2 protein levels in plasma. Genetic variants in TGFB2, TGFBR2 and SMAD3 and their haplotypes were consistently and reproducibly associated with KD susceptibility, coronary artery aneurysm formation, aortic root dilatation, and intravenous immunoglobulin treatment response in different cohorts. A SMAD3 haplotype associated with KD susceptibility replicated in two independent cohorts and an intronic SNP in a separate haplotype block was also strongly associated (A/G, rs4776338) (p=0.000022, OR 1.50, 95% CI 1.25-1.81). Pathway analysis using all 15 genes further confirmed the importance of the TGF-β pathway in KD pathogenesis. Whole blood transcript abundance for these genes and TGF-β2 plasma protein levels changed dynamically over the course of the illness. Conclusions These studies suggest that genetic variation in the TGF-β pathway influences KD susceptibility, disease outcome, and response to therapy and that aortic root and coronary artery Z scores can be used for phenotype/genotype analyses. Analysis of transcript abundance and protein levels further support the importance of this pathway in KD pathogenesis. PMID:21127203

  3. Update on diffusion MRI in Parkinson's disease and atypical parkinsonism

    NARCIS (Netherlands)

    Meijer, F.J.A.; Bloem, B.R.; Mahlknecht, P.; Seppi, K.; Goraj, B.

    2013-01-01

    Differentiating Parkinson's disease (PD) from other types of neurodegenerative atypical parkinsonism (AP) can be challenging, especially in early disease stages. Routine brain magnetic resonance imaging (MRI) can show atrophy or signal changes in several parts of the brain with fairly high

  4. Significance of serum 25-hydroxyvitamin D3and interleukin-6 levels in immunoglobulin treatment of Kawasaki disease in children.

    Science.gov (United States)

    An, Xinjiang; Fu, Mingyu; Tian, Jing; Xue, Ying; Xu, Hui

    2016-09-01

    The aim of the study was to investigate the significance of the level of serum 25-hydroxyvitamin D 3 [25-(OH)D 3 ] and interleukin (IL)-6 in serum prior to and after immunoglobulin treatment in children suffering from Kawasaki disease in order to provide a reference for the successful treatment of Kawasaki disease in children. From February, 2013 to February, 2015, 45 patients with Kawasaki disease were enrolled in the observation group. The normal control group comprised 43 healthy volunteers and the feverish control group 46 patients with respiratory infection and fever. Venous blood was collected from each case before and after immunoglobulin treatment and the level of 25-(OH)D 3 and IL-6 in the serum were measured using fluorescent quantitative PCR, enzyme-linked immunosorbent assay and western blotting. Before treatment, the level of 25-(OH)D 3 in the feverish control group was significantly lower than that of the normal control group, while the level of 25-(OH)D 3 in the observation group was significantly higher than that of the normal control group. The level of 25-(OH)D 3 in the feverish control group was lower than the IL-6 level in the normal children, but the difference was not statistically significant (P>0.05). The level 25-(OH)D 3 in the observation group was significantly higher than the IL-6 level in the normal control group. The serum content of 25-(OH)D 3 was significantly higher after the treatment compared to before treatment levels and after treatment IL-6 level was only slightly lower. It was observed that the 25-(OH)D 3 level in the observation group was significantly increased after immunoglobulin treatment and this was positively correlated with the effects of the treatment. The IL-6 level had no significant changes after treatment and had little correlation with the treatment effect. The results suggested that 25-(OH)D 3 may be involved in the occurrence of Kawasaki disease in children and in the aggravation of the disease to some extent.

  5. 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.

  6. 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.

  7. The relationship between history of ischemic heart disease in parents of children with Kawasaki Disease with severity of heart complications and disease recurrence in these children

    Directory of Open Access Journals (Sweden)

    saeid Mojtahedzadeh

    2011-03-01

    Full Text Available Kawasaki disease (KD, also called mucocutaneous lymph node syndrome is one of the most common childhood vasculitis. Although the etiology of the disease is unknown infectious, genetic and immunologic factors have been supposed to be responsible for KD occurrence. Complications such as coronary artery aneurysm, cardiomyopathy and heart failure, may occur, which can cause significant morbidity and mortality in children. KD is the common cause of acquired heart disease in children in the United States and Japan. Materials and Methods: 61 patients with approved KD admitted in the Mofid Children’s Hospital (from December 2004 to January 2008 and their parents were studied in this cross-sectional survey. 11 patients were omitted according to exclusion criteria, 50 patients were divided into two groups: Mild (n=26 and severe (n=24. Data was analyzed via Fisher’s exact test and student’s t-test using SPSS software, V. 11,5. p<0.05was taken into account significant. Results: 50 patients (32 males, 18 females with mean age of 43 months were concluded in this survey. All of the patients had fever (100%, coronary artery aneurism was seen in 2% of the patients. No recurrence of KD was seen among these children and there was no history of KD in their parents and siblings. Mitral valve prolaps in mothers and ischemic heart disease in fathers among sever group were significantly higher than mild group (p=0.03 and p=0.001, respectively. Mitral regurgitation and 2-times receiving IVIG was significantly higher in severe group (p=0.02 and p=0.008, respectively. Conclusion: This study showed that mitral valve prolaps in mothers and ischemic heart disease in fathers had significant relation with Kawasaki disease in their children. Also, CRP, ESR, Na, LFT and echocardiography are useful to detect and manage severe Kawasaki disease. To provide more accurate conclusions, prospective and multicentric studies with larger sample sizes are necessary.

  8. Adult-onset Kawasaki disease (mucocutaneous lymph node syndrome and concurrent Coxsackievirus A4 infection: a case report

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    Ueda Y

    2015-09-01

    Full Text Available Yuki Ueda,1 Tsuneaki Kenzaka,1,2 Ayako Noda,1 Yu Yamamoto,1 Masami Matsumura1 1Division of General Internal Medicine, Jichi Medical University Hospital, Shimotsuke, 2Division of Community Medicine and Career Development, Kobe University Graduate School of Medicine, Japan Introduction: Kawasaki disease (KD most commonly develops in infants, although its specific cause is still unclear. We report here a rare case of adult-onset KD which revealed to be concurrently infected by Coxsackievirus A4. Case presentation: The patient was a 37-year-old Japanese man who presented with fever, exanthema, changes in the peripheral extremities, bilateral non-exudative conjunctival injection, and changes in the oropharynx, signs that meet the diagnostic criteria for KD defined by the Centers for Disease Control and Prevention. In this case, the patient had a significantly high antibody titer for Coxsackievirus A4, which led us to presume that the occurrence of KD was concurrent Coxsackievirus A4 infection. Conclusion: We reported a very rare case of KD which suggests that the disease can be concurrent Coxsackievirus A4 infection. Although KD is an acute childhood disease, with fever as one of the principal features, KD should also be considered in the differential diagnosis when adult patients present with a fever of unknown cause associated with a rash. Keywords: adult-onset, Coxsackievirus A4, Kawasaki disease, mucocutaneous lymph node syndrome, skin rash

  9. A child with Epstein-Barr Virus-associated hemophagocytic lymphohistiocytosis complicated by coronary artery lesion mimicking Kawasaki disease.

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    Kato, Shogo; Yoshimura, Ken; Tanabe, Yuko; Kimata, Takahisa; Noda, Yukihiro; Kawasaki, Hirohide; Kaneko, Kazunari

    2013-10-01

    There is considerable overlap between hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD) in terms of aberrant immune response though the etiology of KD remains unknown. We present a case fulfilling the criteria of both HLH and KD complicated by coronary artery dilatation: HLH was confirmed to be triggered by Epstein-Barr virus. This case alarms us the possibility that even patients with HLH may be complicated by coronary artery lesion, which is one of the hallmarks of KD. We would like to draw attention that if features of KD become apparent in patients with HLH, echocardiographic examinations should be performed not to miss coronary artery lesion.

  10. Kawasaki disease: Studies on etiology, treatment and long-term follow-up

    NARCIS (Netherlands)

    Tacke, C.E.A.

    2014-01-01

    De ziekte van Kawasaki is een pediatrische vasculitis waarbij als complicatie coronaire arteriële aneurysmata kunnen ontstaan. Carline Tacke beschrijft in de 5 delen van haar proefschrift verschillende studies met betrekking tot de etiologie, behandeling en lange termijn uitkomsten van dit zeldzame

  11. Inflammation @ the heart of Kawasaki disease: Immunogenetic studies on etiology, pathophysiology and treatment effect

    NARCIS (Netherlands)

    Breunis, W.B.

    2010-01-01

    Bij de ziekte van Kawasaki raken de bloedvaten in het lichaam ontstoken. Om onbekende redenen is er een voorkeur voor de kransslagaderen. In Nederland is dit een zeldzame aandoening, maar in andere landen (bijvoorbeeld Japan) komt de ziekte veel vaker voor. Willemijn Breunis onderzocht welke genen

  12. Cardiac Events and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease.

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    Tsuda, Etsuko; Tsujii, Nobuyuki; Hayama, Yosuke

    2017-09-01

    To clarify the occurrence of cardiac events based on the maximal diameter of the maximal coronary artery aneurysm (CAA) in Kawasaki disease (KD). Two hundred fourteen patients (160 male and 54 female) who had had at least 1 CAA in the selective coronary angiogram less than 100 days after the onset of KD were studied. We measured the maximal CAA diameters in the major branches of the initial coronary angiograms. Death, myocardial infarction and coronary artery revascularization were included as cardiac events in this study. We divided the patients into three groups based on the maximal CAA diameter (large ≥8.0 mm; medium ≥6.0 mm and events based on laterality of maximal CAA (bilateral, unilateral) and body surface area (BSA). Cardiac events occurred in 44 patients (21%). For BSA event-free survival in the large and medium groups was 66% (n = 38, 95% CI, 49-80) and 62% (n = 27, 95% CI, 38-81), respectively. For BSA ≥ 0.50 m2, that in large group was 54% (n = 58, 95% CI, 40-67). There were no cardiac events in the medium group for BSA ≥0.50 m2 (n = 36) and the small group (n = 56). In the large analyzed group, the 30-year cardiac event-free survival in the bilateral and unilateral groups was 40% (n = 48, 95% CI, 27-55) and 78% (n = 48, 95% CI, 63-89), respectively (P events was the patient group with the maximal CAA diameter ≥6.0 mm with BSA event-free survival was about 60%. Given the high rate of cardiac events in this patient population, life-long cardiovascular surveillance is advised. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. 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.

  14. Kawasaki Disease in Mongolia: Results From 2 Nationwide Retrospective Surveys, 1996–2008

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    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Background Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Methods Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. Results For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. Conclusions The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia. PMID:21691035

  15. Kawasaki disease in Mongolia: results from 2 nationwide retrospective surveys, 1996-2008.

    Science.gov (United States)

    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia.

  16. The Spectrum of Cardiovascular Lesions Requiring Intervention in Adults After Kawasaki Disease.

    Science.gov (United States)

    Gordon, John B; Daniels, Lori B; Kahn, Andrew M; Jimenez-Fernandez, Susan; Vejar, Matthew; Numano, Fujito; Burns, Jane C

    2016-04-11

    The aim of this study was to characterize the range of management issues raised by adults with cardiovascular sequelae from Kawasaki disease (KD) in childhood. Aneurysms resulting from vascular inflammation associated with KD in childhood may remain clinically silent until adulthood. Adults with large aneurysms, unstable angina, or myocardial infarction following KD in childhood present unique challenges to interventional cardiologists and cardiothoracic surgeons. In an observational study of adults with histories of KD in childhood, data were collected regarding the medical histories and outcomes of 154 adult KD patients, of whom 21 underwent either percutaneous interventions or surgery. Of the 21 subjects with interventions, 11 had been diagnosed with KD in childhood, and 10 had histories of KD-compatible illnesses. Seventeen subjects were asymptomatic until experiencing acute cardiovascular symptoms: acute myocardial infarction (n = 12), angina (n = 2), end-stage congestive heart failure requiring cardiac transplantation (n = 1), and claudication (n = 2). Cardiovascular complications in these subjects illustrate the following points: 1) even small to moderate-sized aneurysms that "normalize" on echocardiography in childhood can lead to stenosis and thrombosis decades after the acute illness; 2) coronary interventions without intravascular ultrasound may result in clinically significant underestimation of vessel luminal diameter; 3) failure to assess the extent of calcification may lead to suboptimal procedural outcomes; and 4) patients with symptomatic peripheral aneurysms may benefit from endarterectomy or resection. Interventional cardiologists should be aware of the potential challenges in treating this growing population of adults. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  17. Kawasaki disease: an unexpected etiology of shock and multiple organ dysfunction syndrome.

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    Gatterre, Pauline; Oualha, Mehdi; Dupic, Laurent; Iserin, Franck; Bodemer, Christine; Lesage, Fabrice; Hubert, Philippe

    2012-05-01

    Severe forms of Kawasaki disease (KD) associated with shock have recently been reported in which a greater number of coronary artery abnormalities (CAA) were observed. In this study, we analyzed organ involvement not restricted to cardiovascular aspects in severe KD and assessed whether their outcome is different than in common forms. Retrospective study. A 12-bed pediatric intensive care unit (PICU) in a university hospital setting. All patients managed in the PICU with a diagnosis of KD from 1 January 2001 to 30 April 2009. Eleven patients were admitted because of moderate febrile shock without initial KD diagnosis. Median age was 75 months (6-175) with a male:female ratio of 1.4. KD was diagnosed and treated after a delay of 1 day (0-2), for a total of 7 days (5-9) after fever onset. Seven patients (63%) developed CAA after 21 days (6-30) with complete regression within a delay of 120 days (18-240). Nonspecific encephalopathy (n = 6) as well as acute kidney injury (n = 10) were also observed. Multiple organ dysfunction syndrome (MODS) occurred in eight patients. Although predicted mortality according to the PELOD score [21 (10-43)] ranged from 20% to up to 50%, all 11 children survived with no sequelae. Moderate shock is the main reason for PICU admission in children suffering from KD. These forms can be associated with surprising MODS. Despite the severity of symptoms, all patients survived without any sequelae, hence the need for proper diagnosis and rapid treatment of these unusual severe forms.

  18. Performance of 2004 American Heart Association Recommendations for Treatment of Kawasaki Disease

    Science.gov (United States)

    Yellen, Elizabeth S.; Gauvreau, Kimberlee; Takahashi, Masato; Burns, Jane C.; Shulman, Stanford; Baker, Annette L.; Innocentini, Nancy; Zambetti, Chiara; Pancheri, Joan M.; Ostrow, Adam; Frazer, Jeffrey R.; Sundel, Robert P.; Fulton, David R.; Newburger, Jane W.

    2010-01-01

    OBJECTIVE The 2004 American Heart Association (AHA) statement included a clinical case definition and an algorithm for diagnosing and treating suspected incomplete Kawasaki disease (KD). We explored the performance of these recommendations in a multicenter series of US patients with KD with coronary artery aneurysms (CAAs). METHODS We reviewed retrospectively records of patients with KD with CAAs at 4 US centers from 1981 to 2006. CAAs were defined on the basis of z scores of >3 or Japanese Ministry of Health and Welfare criteria. Our primary outcome was the proportion of patients presenting at illness day ≤21 who would have received intravenous immunoglobulin (IVIG) treatment by following the AHA guidelines at the time of their initial presentation to the clinical center. RESULTS Of 195 patients who met entry criteria, 137 (70%) met the case definition and would have received IVIG treatment at presentation. Fifty-three patients (27%) had suspected incomplete KD and were eligible for algorithm application; all would have received IVIG treatment at presentation. Of the remaining 5 patients, 3 were excluded from the algorithm because of fever for 6 months of age. Two of these 5 patients would have entered the algorithm and received IVIG treatment after follow-up monitoring. Overall, application of the AHA algorithm would have referred ≥190 patients (97%) for IVIG treatment. CONCLUSIONS Application of the 2004 AHA recommendations, compared with the classic criteria alone, improves the rate of IVIG treatment for patients with KD who develop CAAs. Future multicenter prospective studies are needed to assess the performance characteristics of the AHA algorithm in febrile children with incomplete criterion findings and to refine the algorithm further. PMID:20100771

  19. 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.

  20. Clinical features and related factors for Kawasaki disease with liver damage

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    ZHANG Haijun

    2014-07-01

    Full Text Available ObjectiveTo study the features of Kawasaki disease (KD with liver damage and investigate the possible related factors, and to improve the understanding of liver damage in KD patients. MethodsThe clinical data of 75 KD patients admitted from 2008 to 2013 were collected to retrospectively analyze the clinical features of liver damage, as well as routine blood test, C-reactive protein (CRP, erythrocyte sedimentation rate (ESR, liver function, and abdominal ultrasound. Categorical data were analyzed by chi-square test; continuous data were expressed as mean±standard deviation; comparison between groups was made by t test and multivariate stepwise regression analysis. ResultsThe incidence of abnormal alanine aminotransferase (ALT level in 75 cases was 32%, and 79.2% of these patients had a serum ALT level of less than 200 IU/L, including 3 patients with hepatomegaly, 1 patient with jaundice, and 1 patient with splenomegaly, which accounted for 12.5%, 4.2%, and 4.2%, respectively, of patients with liver damage. CRP and ESR were not found to be related to liver damage (t=1.560, P=0.123; t=1.291, P=0.201. There was significant difference in platelet count between patients with liver damage and controls (t=-3.228, P=0.003. No significant difference in white blood cell (WBC count increase was found between the liver damage group and control group, but simultaneous increase in WBC count and platelet count was significantly associated with an increased risk of liver damage (P<0.05. Conclusion CRP and ESR are not found to be significantly related to the development of liver damage, but there is a close relationship between liver damage and abnormal platelet count. WBC count shows a certain relationship with liver damage. Clinicians should pay attention to these factors and take timely measures for intervention.

  1. Atypical Celiac Disease Resistant to Thyroxine Replacement

    OpenAIRE

    Oguzhan Aksu

    2014-01-01

    Celiac disease, an immune-mediated enteropathy that develops in susceptible individuals upon ingestion of gluten containing diet, is closely associated with other autoimmune endocrine disorders, particularly autoimmune thyroid disease. Celiac disease and hypothyroidism ( especially due to Hashimoto disease) cooccurence is frequently mentioned in the literature. The relationship between celiac disease and autoimmune thyroid disease was first described three decades ago. Patients usually have...

  2. Effect of revision of Japanese diagnostic criterion for fever in Kawasaki disease on treatment and cardiovascular outcome.

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    Muta, Hiromi; Ishii, Masahiro; Iemura, Motofumi; Suda, Kenji; Nakamura, Yosikazu; Matsuishi, Toyojiro

    2007-11-01

    The aim of this study was to investigate the effect of a revision of the fever criterion for initial intravenous immunoglobulin (IVIG) treatment, and cardiovascular sequelae, in the new Japanese diagnostic criteria for Kawasaki disease. Patients who were reported in the 16th and 18th nationwide surveys in Japan were analyzed. New criteria group comprised patients who received the diagnosis of Kawasaki disease in the 18th nationwide survey (n=18,789). Old criteria group was comprised patients who received their diagnosis in the 16(th) nationwide survey (n=15,017). The difference between the new and old criteria for complete cases was only 1%. The proportion of patients who were treated with IVIG within 4 days of illness onset in the new criteria group was significantly lower than in the old criteria group (27.7% vs 30.7%). Multivariate logistic regression analysis identified criteria sex, age, recurrence, diagnosis, last day of initial IVIG and additional IVIG treatment as significant independent factors for cardiovascular sequelae. The guideline revision improves diagnostic sensitivity somewhat, but reflects the clinical reality of the disease that approximately 30% of patients are treated with IVIG within 4 days of illness onset.

  3. Kawasaki disease in children and adolescents: clinical data of Kawasaki patients in a western region (Tyrol) of Austria from 2003-2012.

    Science.gov (United States)

    Binder, Elisabeth; Griesmaier, Elke; Giner, Thomas; Sailer-Höck, Michaela; Brunner, Juergen

    2014-09-02

    Kawasaki disease (KD) is a rare vasculitis seen predominantly in children. In developing countries, it is the leading cause of childhood-acquired heart disease. Besides a case report from 1981 there have been no data published dealing with the epidemiology and clinical aspects of KD in Austria. The purpose of the present study was to investigate the clinical spectrum of KD in a geographically determined cohort of infants, children, and adolescents that were diagnosed and treated at the University Hospital of Innsbruck from 2003-2012. Thirty-two patients were included in the study with a median age of 32.96 months (2-192). 59.4% of the patients were aged between six months and four years. The male-to-female ratio was 1:1.13. Clinical examination revealed non-purulent conjunctivitis and exanthema as the most common symptoms (84.4%). 75% showed oropharyngeal changes, 21.9% had gastrointestinal complaints such as diarrhoe, stomachache or vomiting prior to diagnosis. One third of the patients were admitted with a preliminary diagnosis, whereas 78.1% were pre-treated with antibiotics. The median fever duration at the time of presentation was estimated with 4.96 days (1-14), at time of diagnosis 6.76 days (3-15).75% were diagnosed with complete KD, and 25% with an incomplete form of the disease. There was no significant difference in the duration of fever neither between complete and incomplete KD, nor between the different age groups. Typical laboratory findings included increased C-reactive protein (CRP) (80.6%) and erythrocyte sedimentation rate (ESR) (96%),leukocytosis (48.4%) and thrombocytosis (40.6%) without any significant quantitative difference between complete and incomplete KD. Coronary complications could be observed in six patients: one with a coronary aneurysm and five with tubular dilatation of the coronary arteries. Our patient cohort represents the age distribution as described in literature and emphasizes that KD could affect persons of any age. The

  4. A genome-wide association study identifies novel and functionally related susceptibility Loci for Kawasaki disease.

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    David Burgner

    2009-01-01

    Full Text Available Kawasaki disease (KD is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s in genetically susceptible children. To investigate genetic determinants of KD susceptibility, we performed a genome-wide association study (GWAS in 119 Caucasian KD cases and 135 matched controls with stringent correction for possible admixture, followed by replication in an independent cohort and subsequent fine-mapping, for a total of 893 KD cases plus population and family controls. Significant associations of 40 SNPs and six haplotypes, identifying 31 genes, were replicated in an independent cohort of 583 predominantly Caucasian KD families, with NAALADL2 (rs17531088, p(combined = 1.13 x 10(-6 and ZFHX3 (rs7199343, p(combined = 2.37 x 10(-6 most significantly associated. Sixteen associated variants with a minor allele frequency of >0.05 that lay within or close to known genes were fine-mapped with HapMap tagging SNPs in 781 KD cases, including 590 from the discovery and replication stages. Original or tagging SNPs in eight of these genes replicated the original findings, with seven genes having further significant markers in adjacent regions. In four genes (ZFHX3, NAALADL2, PPP1R14C, and TCP1, the neighboring markers were more significantly associated than the originally associated variants. Investigation of functional relationships between the eight fine-mapped genes using Ingenuity Pathway Analysis identified a single functional network (p = 10(-13 containing five fine-mapped genes-LNX1, CAMK2D, ZFHX3, CSMD1, and TCP1-with functional relationships potentially related to inflammation, apoptosis, and cardiovascular pathology. Pair-wise blood transcript levels were measured during acute and convalescent KD for all fine-mapped genes, revealing a consistent trend of significantly reduced transcript levels prior to treatment

  5. A Genome-Wide Association Study Identifies Novel and Functionally Related Susceptibility Loci for Kawasaki Disease

    Science.gov (United States)

    Breunis, Willemijn B.; Ng, Sarah B.; Li, Yi; Bonnard, Carine; Ling, Ling; Wright, Victoria J.; Thalamuthu, Anbupalam; Odam, Miranda; Shimizu, Chisato; Burns, Jane C.; Levin, Michael; Kuijpers, Taco W.; Hibberd, Martin L.

    2009-01-01

    Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD susceptibility, we performed a genome-wide association study (GWAS) in 119 Caucasian KD cases and 135 matched controls with stringent correction for possible admixture, followed by replication in an independent cohort and subsequent fine-mapping, for a total of 893 KD cases plus population and family controls. Significant associations of 40 SNPs and six haplotypes, identifying 31 genes, were replicated in an independent cohort of 583 predominantly Caucasian KD families, with NAALADL2 (rs17531088, p combined = 1.13×10−6) and ZFHX3 (rs7199343, p combined = 2.37×10−6) most significantly associated. Sixteen associated variants with a minor allele frequency of >0.05 that lay within or close to known genes were fine-mapped with HapMap tagging SNPs in 781 KD cases, including 590 from the discovery and replication stages. Original or tagging SNPs in eight of these genes replicated the original findings, with seven genes having further significant markers in adjacent regions. In four genes (ZFHX3, NAALADL2, PPP1R14C, and TCP1), the neighboring markers were more significantly associated than the originally associated variants. Investigation of functional relationships between the eight fine-mapped genes using Ingenuity Pathway Analysis identified a single functional network (p = 10−13) containing five fine-mapped genes—LNX1, CAMK2D, ZFHX3, CSMD1, and TCP1—with functional relationships potentially related to inflammation, apoptosis, and cardiovascular pathology. Pair-wise blood transcript levels were measured during acute and convalescent KD for all fine-mapped genes, revealing a consistent trend of significantly reduced transcript levels prior to treatment

  6. Polymorphisms in the mannose-binding lectin gene as determinants of age-defined risk of coronary artery lesions in Kawasaki disease

    NARCIS (Netherlands)

    Biezeveld, Maarten H.; Geissler, Judy; Weverling, Gerrit Jan; Kuipers, Irene M.; Lam, Jan; Ottenkamp, Jaap; Kuijpers, Taco W.

    2006-01-01

    OBJECTIVE: To evaluate the relationship between polymorphisms in the gene coding for mannose-binding lectin (MBL) and the occurrence of coronary artery lesions (CALs) among different age groups of patients with Kawasaki disease. METHODS: The frequencies of the genotypes, defined as mutations in

  7. Evaluation of myocardial deformation in patients with Kawasaki disease using speckle-tracking echocardiography during mid-term follow-up.

    Science.gov (United States)

    Dedeoglu, Reyhan; Barut, Kenan; Oztunc, Funda; Atik, Sezen; Adrovic, Amra; Sahin, Sezgin; Cengiz, Dicle; Kasapcopur, Ozgur

    2017-09-01

    Speckle-tracking echocardiography is a recently developed technique for the evaluation of myocardial deformation or strain. Our objective was to examine strain through a mid-term follow-up of patients with Kawasaki disease. We explored left ventricular mechanics using speckle-tracking echocardiography in 35 patients with a history of Kawasaki disease at least 6 months after the acute phase. We also included 30 healthy children as controls. Strain data sets were acquired for the quantification of left ventricular global strain, segmental strain, and left ventricular ejection fraction. The mean age of our patients was 25.6±15.4 months. At a median follow-up of 57.5 months (16.5-98.2), although both values were in the normal range, the mean left ventricular ejection proportion of patients (57.3%) was a little lower than that of controls (p⩽0.05). Patient strain values at the basal inferoseptal (20.0), basal anterolateral (19.5), apical septal (23.3), and apical inferior (24.0) segments were lower compared with controls. In all, seven patients had coronary aneurysms during follow-up. Kawasaki disease patients with pyuria had lower left ventricular strain at the mid anterior, mid anteroseptal, apical anterior, and apical inferior segments and global longitudinal strain compared with patients with no pyuria (p⩽0.05). In children with a history of Kawasaki disease, impairment of left ventricular mechanics occurs especially within the left anterior descending artery territories.

  8. 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.

  9. 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

  10. [Phosphatase and tensin homolog deleted on chromosome ten/phosphatidyl Inositol 3-kinase/vascular endothelial growth factor signaling pathway changes in the rabbit Kawasaki disease model].

    Science.gov (United States)

    Niu, L; Fu, M Y; Tian, J; He, X H; Zhang, H N; Wang, Q W; Wang, Y; Li, C L; Wang, Z Z; An, X J

    2016-03-01

    To observe the changes of phosphatase and tensin homolog deleted on chromosome ten(PTEN)/ phosphatidyl Inositol 3-kinase(PI3K)/ vascular endothelial growth factor(VEGF)signaling pathway in a rabbit Kawasaki disease model. Model of Kawasaki disease was established in weanling Japanese big-eared rabbits with 10% bovine serum venous injection (2.5 ml/kg, 2 times, and 2 week's interval) through the ear. Twenty four rabbits were divided into 4 groups: control group (without injection of 10% bovine serum albumin, six rabbits); 1 day group (sacrificed a the second day after the establishment of Kawasaki disease models, six rabbits); 7 day group (sacrificed at the seventh day after establishment of Kawasaki disease model, six rabbits); 30 day group (sacrificed at the thirtieth day after establishment of Kawasaki disease model, six rabbits). Pathological analysis was performed on coronary artery tissue samples. The express of PTEN and PI3K were detected by immunohistochemistry. The levels of VEGF and CK were also examined with ELISA and white blood cells were counted. (1) Coronary artery of model groups was thinner, distorted and had enlarged lumen. (2) PTEN expression in 1 d group, 7 d group and 30 d group were 58.5 ± 12.9, 73.2±9.9 and 109.6 ± 24.4, respectively, significantly higher than in the control group (25.5 ± 6.9, P0.05) and significantly lower than 1 d and 7 d group (both P0.05). (6)White blood cell count were significantly higher in 1 d group, 7 d group and 30 d group than in control group (all PKawasaki disease model and the signaling pathway might be involved in this model.

  11. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease: An Observational Study.

    Science.gov (United States)

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise.Sixty-three KD patients were recruited as KD group 1 from children (aged 5-18 y) who received transthoracic echocardiographic examinations and symptom-limited treadmill exercise test for regular follow-up of KD from January 2010 to October 2014 in 1 medical center. We then divided KD group 1 into KD group 2 (LCA or RCA by Dalliarre equation or Fuse calculator.All routine parameters measured during standard exercise test were similar between KD and control groups, except that peak rate pressure products (PRPPs) in KD group 1 to 3 were all lower than corresponding control groups significantly (P = 0.010, 0.020, and 0.049, respectively). PRPPs correlated with Max-Z of CA by both equations modest inversely (by Dallaire, P = 0.017, Spearman rho = -0.301; by Fuse, P = 0.014, Spearman rho = -0.309).Our study recruited larger number of KD patients and provided a newer data of EC of KD patients. Our finding suggests that after acute stage of KD, patients could maintain normal cardiorespiratory fitness. Therefore, we believe that it is important to promote cardiovascular health to KD patients and KD patients should exercise as normal peers. However, since KD patients might still have compromised coronary perfusion during exercise, it remains crucial to assess and monitor cardiovascular risk of KD patients. Max-Z of CA correlates with PRPP modest inversely and might be used as a follow-up indicator of CA reserve during exercise after acute stage of KD.

  12. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease

    Science.gov (United States)

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Abstract Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise. Sixty-three KD patients were recruited as KD group 1 from children (aged 5–18 y) who received transthoracic echocardiographic examinations and symptom-limited treadmill exercise test for regular follow-up of KD from January 2010 to October 2014 in 1 medical center. We then divided KD group 1 into KD group 2 (LCA or RCA by Dalliarre equation or Fuse calculator. All routine parameters measured during standard exercise test were similar between KD and control groups, except that peak rate pressure products (PRPPs) in KD group 1 to 3 were all lower than corresponding control groups significantly (P = 0.010, 0.020, and 0.049, respectively). PRPPs correlated with Max-Z of CA by both equations modest inversely (by Dallaire, P = 0.017, Spearman rho = −0.301; by Fuse, P = 0.014, Spearman rho = −0.309). Our study recruited larger number of KD patients and provided a newer data of EC of KD patients. Our finding suggests that after acute stage of KD, patients could maintain normal cardiorespiratory fitness. Therefore, we believe that it is important to promote cardiovascular health to KD patients and KD patients should exercise as normal peers. However, since KD patients might still have compromised coronary perfusion during exercise, it remains crucial to assess and monitor cardiovascular risk of KD patients. Max-Z of CA correlates with PRPP modest inversely and might be used as a follow-up indicator of CA reserve during exercise after acute stage of KD. PMID:26765431

  13. Early Differentiation of Kawasaki Disease Shock Syndrome and Toxic Shock Syndrome in a Pediatric Intensive Care Unit.

    Science.gov (United States)

    Lin, Ying-Jui; Cheng, Ming-Chou; Lo, Mao-Hung; Chien, Shao-Ju

    2015-11-01

    Kawasaki disease shock syndrome (KDSS) and toxic shock syndrome (TSS) can present as shock and fever with skin rash, but the management of these 2 groups of patients is different. This report proposes to help clinicians earlier distinguish these 2 diseases and expedite institution of appropriate therapy. We retrospectively reviewed the medical records of patients admitted to the pediatric intensive care unit with the diagnosis of KDSS or TSS from January 2000 through December 2010. Clinical, laboratory and echocardiographic data were collected for analysis of differences between them. Seventeen patients met the inclusion criteria of KDSS and 16 had a confirmed diagnosis of TSS. The mean age of the KDSS group was significantly younger than that of the TSS group (36.8 ± 41.1 vs. 113.3 ± 55.6 months, P thrombocytosis are useful early differentiating features between KDSS and TSS patients.

  14. Coronary artery aneurysms and congestive heart failure--possible long-term course of Kawasaki disease in an adult--a case report.

    Science.gov (United States)

    Sakai, Y; Takayanagi, K; Inoue, T; Yamaguchi, H; Hayashi, T; Morooka, S; Takabatake, Y; Sato, Y

    1988-07-01

    Multiple coronary artery aneurysms, rarely seen in patients with atherosclerotic heart disease, can be frequently observed in children with Kawasaki disease. However, their long-term clinical courses still remain obscure. A thirty-nine-year-old male came to our clinic because of congestive heart failure. A left ventriculogram revealed highly reduced wall motion. A coronary angiogram showed left main trunk aneurysm with complete occlusion of the left anterior descending artery and ramification of the right coronary artery close to the ostium. Six months after discharge, he died suddenly. On autopsy, aneurysms were observed in the left main trunk and right coronary artery, together with an old anteroseptal myocardial infarction. Although he did not have a clear history of febrile disease in childhood, he was highly suspected to be a long-term survivor of Kawasaki disease because of the unique form and distribution of the coronary artery aneurysms.

  15. Factors associated with low moderate-to-vigorous physical activity levels in pediatric patients with Kawasaki disease.

    Science.gov (United States)

    Banks, Laura; Lin, Yahui Tammy; Chahal, Nita; Manlhiot, Cedric; Yeung, Rae S M; McCrindle, Brian W

    2012-09-01

    We sought to determine functional health status and physical activity determinants in 27 patients with Kawasaki disease (KD; 20 males, 11 ± 3 years old). Patient physical activity data were compared with a population-based study of healthy children (Canadian Health Measures Survey). KD patients performed less moderate-to-vigorous physical activity (MVPA) than healthy children (males, 27 vs 61 min/d, P self-efficacy score; lower Child Health Questionnaire (CHQ-PF50) scores for role functioning behavioral issues, physical functioning, and family cohesion; and higher CHQ-PF50 scores for self-esteem and family activity limitations. Physical activity counseling should be a focus of management for children with a history of KD.

  16. Intrauterine and Early Postnatal Exposure to Particulate Air Pollution and Kawasaki Disease: A Nationwide Longitudinal Survey in Japan.

    Science.gov (United States)

    Yorifuji, Takashi; Tsukahara, Hirokazu; Kashima, Saori; Doi, Hiroyuki

    2017-12-04

    To examine the effects of prenatal and postnatal exposure to particulate matter on Kawasaki disease (KD) occurrence, using data from a nationwide population-based longitudinal survey in Japan that began in 2010. Prenatal and postnatal suspended particulate matter concentrations were obtained at municipality level and assigned to participants based on their municipality of birth. We analyzed data from 30 367 participants with data on either exposure period. We used hospital admission for KD from 6 to 30 months of age as the main outcome of interest. We conducted a multilevel logistic regression analysis, adjusting for individual and municipality-level variables. Children who were exposed to higher levels of suspended particulate matter, in particular during pregnancy, were more likely to be hospitalized for KD. The ORs for ≥25 µg/m3 exposure compared with Japan. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Evaluation of clinical characteristics of Kawasaki syndrome and risk factors for coronary artery abnormalities among children in Denmark.

    Science.gov (United States)

    Patel, Amy; Holman, Robert C; Callinan, Laura S; Sreenivasan, Nandini; Schonberger, Lawrence B; Fischer, Thea K; Belay, Ermias D

    2013-04-01

    To examine clinical characteristics, treatment and outcome of Kawasaki syndrome patients in Denmark. A retrospective chart review of hospitalization records for children Kawasaki syndrome discharge diagnosis identified through the Danish National Patient Registry during 1994 through June 2008 was conducted. A total of 284 cases Kawasaki syndrome (n = 279) and atypical Kawasaki syndrome (n = 5); 70.4% were Kawasaki syndrome patients were diagnosed with coronary artery abnormalities. Not receiving intravenous immunoglobulin treatment before the 10th day of illness, young age and male sex were significantly associated with the development of coronary artery abnormalities. In Denmark, more than one in 10 children with Kawasaki syndrome develop coronary artery abnormalities. Physicians should increase their index of suspicion for early diagnosis and treatment of Kawasaki syndrome among patients susceptible to increased risk of coronary artery abnormalities, particularly in infants who may have a more atypical presentation of the illness. ©2012 The Author(s)/Acta Paediatrica ©2013 Foundation Acta Paediatrica.

  18. Atypical presentation is dominant and typical for coeliac disease.

    Science.gov (United States)

    Rostami Nejad, Mohammad; Rostami, Kamran; Pourhoseingholi, Mohamad Amin; Nazemalhosseini Mojarad, Ehsan; Habibi, Manijeh; Dabiri, Hossein; Zali, Mohammad Reza

    2009-09-01

    Atypical presentation is the most prevalent form of coeliac disease (CD) and mostly clinically indistinguishable from other gastrointestinal (GI) disorders. The first objective of this study was to determine the prevalence of CD in patients with GI symptoms and the second objective was to characterize the typical manifestations of the atypical forms of CD. This was a cross sectional study comprising 5,176 individuals by random sampling of self-referred people from the Tehran province, during the years 2006-2007 in a primary care setting. From 5,176 individuals, 670 with GI symptoms were selected for coeliac serology including total immunoglobulin A (IgA) and anti-tissue transglutaminase (tTG) antibodies. Those with IgA deficiency were tested with IgG tTG. This study shows that 13% (670/5176) of self-referred patients to a general practice suffer from GI symptoms. Dyspepsia was the most common symptom in 25 seropositive cases similar to the rest of the study group. A positive anti-tTG test was found in 22 from 670 investigated subjects (17 women, 5 men) (95% CI: 1.70-4.30) and 8/670 were IgA deficient. A positive IgG tTG was detected in 3/8 IgA deficient individuals. The prevalence of CD antibodies in serologically screened samples excluding IgA-deficient was 3.3% and 3.7% when including those IgA-deficient with positive tTG-IgG. Non-specific GI symptoms seem to be the typical presentation of atypical CD. This study indicated that there is a high prevalence of CD antibodies among patients with GI symptoms (3.7%). More awareness regarding the atypical presentation of CD could be the key step in identifying asymptomatic patients.

  19. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

    Science.gov (United States)

    Kobayashi, Tohru; Saji, Tsutomu; Otani, Tetsuya; Takeuchi, Kazuo; Nakamura, Tetsuya; Arakawa, Hirokazu; Kato, Taichi; Hara, Toshiro; Hamaoka, Kenji; Ogawa, Shunichi; Miura, Masaru; Nomura, Yuichi; Fuse, Shigeto; Ichida, Fukiko; Seki, Mitsuru; Fukazawa, Ryuji; Ogawa, Chitose; Furuno, Kenji; Tokunaga, Hirohide; Takatsuki, Shinichi; Hara, Shinya; Morikawa, Akihiro

    2012-04-28

    Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, pKawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Japanese Ministry of Health, Labour and Welfare. Copyright © 2012 Elsevier Ltd. All rights reserved.

  20. [Anti TNF-α (infliximab) treatment for intravenous immunoglobulin (IVIG) resistance patients with acute Kawasaki disease the effects of anticytokine therapy].

    Science.gov (United States)

    Saji, Tsutomu; Takatsuki, Shinichi; Kobayashi, Toru

    2014-09-01

    Among the patients with acute Kawasaki disease treated with intravenous immunoglobulin (IVIG), 10-20 % demonstrate resistance or incomplete effects. Cardiac complication such as the coronary arterial aneurysm is frequent in these patients. For patients with IVIG-resistance, we have surveyed the efficacy and safety of anti-cytokine therapy with use of infliximab (Remicade), chimera type anti TNF-α agent, for children. After May, 2005, Remicade has been used in >500 pediatric patients in whom IVIG and intravenous methylprednisolone pulse therapy did not show significant effects. The efficacy and safety of Remicade on patients with IVIG-resistant Kawasaki disease has been observed but 10~20 % of patients was Remicade-resistant. Re-treatment with IVIG or steroids was also effective. The efficacy of Remicade for reducing the fever duration, CRP, WBC counts was promising, but reduction of the incidence of coronary aneurysm was not confirmed. Randomized clinical trial will be needed.

  1. Losartan attenuates the coronary perivasculitis through its local and systemic anti-inflammatory properties in a murine model of Kawasaki disease.

    Science.gov (United States)

    Suganuma, Eisuke; Niimura, Fumio; Matsuda, Shinichi; Ukawa, Toshiko; Nakamura, Hideaki; Sekine, Kaori; Kato, Masahiko; Aiba, Yuji; Koga, Yasuhiro; Hayashi, Kuniyoshi; Takahashi, Osamu; Mochizuki, Hiroyuki

    2017-04-01

    Kawasaki disease is a common systemic vasculitis that leads to coronary artery lesions. Besides its antihypertensive effects, losartan can modulate inflammation in cardiovascular disease. We examined whether losartan can attenuate coronary inflammation in a murine model of Kawasaki disease. Five-wk-old C57/BL6J male mice were intraperitoneally injected with Lactobacillus casei cell wall extract to induce coronary inflammation and divided into four groups: placebo, intravenous immunoglobulin (IVIG), losartan, and IVIG+losartan. After 2 wk, mice were harvested. The coronary perivasculitis was significantly attenuated by losartan but not by IVIG alone, and further dramatic attenuation by IVIG+losartan was observed. The frequency of Lactobacillus casei cell wall extract-induced myocarditis (80%) was markedly lowered by losartan (22%) and IVIG+losartan (0%). Furthermore, interleukin (IL)-6 mRNA was markedly attenuated by IVIG+losartan. Serum levels of IL-6, TNF-α, MCP-1, and IL-10 after Lactobacillus casei cell wall extract injection were slightly decreased by IVIG or losartan. Moreover, IL-1β, IL-10, and MCP-1 levels were significantly decreased by IVIG+losartan. The addition of losartan to IVIG strongly attenuated the severity of coronary perivasculitis and the incidence of myocarditis, along with suppressing systemic/local cytokines as well as the activated macrophage infiltration. Therefore, losartan may be a potentially useful additive drug for the acute phase of Kawasaki disease to minimize coronary artery lesions.

  2. Corticosteroid therapy for primary treatment of Kawasaki disease ? weight of evidence: a meta-analysis and systematic review of the literature

    OpenAIRE

    Athappan, Ganesh; Gale, Seth; Ponniah, Thirumalaikolundusubramanian

    2009-01-01

    Objective Corticosteroids are the treatment of choice in most forms of vasculitis. However, their role in the primary treatment of Kawasaki disease (KD) is controversial. Our aim was to conduct a meta-analysis to assess the clinical course and coronary artery outcome of adding corticosteroids to standard therapy [intravenous immunoglobulin (IVIG) + aspirin] in patients with acute KD. Methods We included randomised trials comparing the addition of corticosteroids to conventional primary therap...

  3. High-Dose Aspirin is Associated with Anemia and Does Not Confer Benefit to Disease Outcomes in Kawasaki Disease.

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    Ho-Chang Kuo

    Full Text Available Kawasaki disease (KD is also known as multiple mucocutaneous lymph node syndrome of systemic vasculitis and is a leading cause of coronary artery lesions (CAL in childhood. Intravenous immunoglobulin (IVIG has been proven to effectively reduce the incidence of CAL, but the role and effect dose of aspirin in KD is still unclear. Moreover, overt bleeding and anemia are associated with the use of aspirin, and anemia is common in patients with KD. Thus, the aim of this study was conducted to compare the treatment efficacy, degree of anemia and inflammation, and changes in serum hepcidin in children who received a combination of high-dose aspirin and IVIG in the acute stage of KD, and those who received IVIG alone.KD patients from two medical centers were retrospectively analyzed from 1999-2009. All patients were initially treated with a single dose of IVIG (2 g/kg as the standard care of treatment. In group 1, high-dose aspirin was prescribed (> 30 mg/kg/day until the fever subsided, and then low-dose aspirin (3-5 mg/kg/day was prescribed until all the inflammation signs had resolved. In group 2, low-dose aspirin was prescribed without high-dose aspirin. Laboratory data were collected for analysis in both groups.A total of 851 KD patients (group 1, N = 305, group 2, N = 546 were enrolled in this study. There were no significant differences between group 1 and group 2 in terms of gender (p = 0.51, IVIG resistance rate (31/305 vs. 38/546, p = 0.07, CAL formation (52/305 vs. 84/546, p = 0.67, and duration of hospitalization (6.3 ± 0.2 vs. 6.7 ± 0.2 days, p = 0.13. There were also initially no significant differences in total white blood cell count, hemoglobin level, platelet count, and CRP before IVIG treatment between groups (all p>0.1. After IVIG treatment, group 1 had significantly lower levels of hemoglobin (p = 0.006 and higher CRP (p<0.001 as well as a smaller decrease in CRP level (p = 0.012. Furthermore, there was also a higher serum level of

  4. High-Dose Aspirin is Associated with Anemia and Does Not Confer Benefit to Disease Outcomes in Kawasaki Disease.

    Science.gov (United States)

    Kuo, Ho-Chang; Lo, Mao-Hung; Hsieh, Kai-Sheng; Guo, Mindy Ming-Huey; Huang, Ying-Hsien

    2015-01-01

    Kawasaki disease (KD) is also known as multiple mucocutaneous lymph node syndrome of systemic vasculitis and is a leading cause of coronary artery lesions (CAL) in childhood. Intravenous immunoglobulin (IVIG) has been proven to effectively reduce the incidence of CAL, but the role and effect dose of aspirin in KD is still unclear. Moreover, overt bleeding and anemia are associated with the use of aspirin, and anemia is common in patients with KD. Thus, the aim of this study was conducted to compare the treatment efficacy, degree of anemia and inflammation, and changes in serum hepcidin in children who received a combination of high-dose aspirin and IVIG in the acute stage of KD, and those who received IVIG alone. KD patients from two medical centers were retrospectively analyzed from 1999-2009. All patients were initially treated with a single dose of IVIG (2 g/kg) as the standard care of treatment. In group 1, high-dose aspirin was prescribed (> 30 mg/kg/day) until the fever subsided, and then low-dose aspirin (3-5 mg/kg/day) was prescribed until all the inflammation signs had resolved. In group 2, low-dose aspirin was prescribed without high-dose aspirin. Laboratory data were collected for analysis in both groups. A total of 851 KD patients (group 1, N = 305, group 2, N = 546) were enrolled in this study. There were no significant differences between group 1 and group 2 in terms of gender (p = 0.51), IVIG resistance rate (31/305 vs. 38/546, p = 0.07), CAL formation (52/305 vs. 84/546, p = 0.67), and duration of hospitalization (6.3 ± 0.2 vs. 6.7 ± 0.2 days, p = 0.13). There were also initially no significant differences in total white blood cell count, hemoglobin level, platelet count, and CRP before IVIG treatment between groups (all p>0.1). After IVIG treatment, group 1 had significantly lower levels of hemoglobin (p = 0.006) and higher CRP (paspirin in the acute phase of KD does not confer any benefit with regards to inflammation and it does not appear to

  5. Effects of HMG-CoA reductase inhibitors on continuous post-inflammatory vascular remodeling late after Kawasaki disease.

    Science.gov (United States)

    Hamaoka, Akiko; Hamaoka, Kenji; Yahata, Tomoyo; Fujii, Maiko; Ozawa, Seiichiro; Toiyama, Kentaro; Nishida, Masashi; Itoi, Toshiyuki

    2010-09-01

    In Kawasaki disease (KD), it has been clinically and experimentally reported that post-inflammatory vascular remodeling would induce the development of arteriosclerosis or early onset of atherosclerosis in the future. The effects of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors on continuous vascular remodeling late after Kawasaki disease were clinically evaluated. We enrolled and treated a total of 11 KD patients (age range, 7-25 years) with fluvastatin (0.5-0.7 mg/kg/day) for 12 months. All of them had significant coronary aneurysmal or stenotic lesions and more than 3 of the following 5 abnormal findings: reduced %flow-mediated dilatation (%FMD), reduced urinary NOx, elevated high-sensitivity C-reactive protein (hs-CRP), reduced urinary 8-isoprostane, and elevated brachial-ankle pulse wave velocity (baPWV; control, ≤1400 cm/s). A statistically significant improvement was observed in each biomarker after fluvastatin treatment: %FMD, from 9.29% (3.41)% to 10.55% (3.27)% (p=0.003) after 3 months; NOx/creatinine (cre), from 1.16 (0.54) µmol/mg cre to 1.30 (0.50) µmol/mg cre (p=0.038) after 12 months; baPWV, from 1175.4 (277.3) cm/s to 1031.8 (155.6) cm/s (p=0.009) after 3 months; hs-CRP, from 0.073 (0.035) mg/dl to 0.028 (0.014) mg/dl (p=0.0002) after 3 months; and 8-iso/cre, from 751.8 (241.8) pg/mg cre to 660.0 (198.5) pg/mg cre (p=0.018) after 3 months. No adverse events were clinically observed in the patients. The results of this study suggested that HMG-CoA reductase inhibitors are useful as an alternative therapeutic strategy for stabilizing continuous post-inflammatory vascular remodeling that results in the development of arteriosclerosis late after KD or early onset of atherosclerosis in the future. Copyright © 2010 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  6. 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

  7. 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.

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

    Science.gov (United States)

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

    2014-06-01

    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 identify variables associated with PPCV. This cross-sectional study included 288 parents (83% mothers) of KD patients (mean age 8.7 years). HRQOL was assessed using the TNO-AZL Questionnaire for Adult's HRQOL (TAAQOL) and PPCV using the Child Vulnerability Scale (CVS). Scores of KD parents were compared with reference groups of Dutch parents. Logistic regression analyses were performed to examine associated variables. The HRQOL of KD parents was comparable to the HRQOL of parents of healthy children. However, KD parents showed significantly higher PPCV, regarding both the median CVS total score and the percentage in the clinical range. No differences were found in CVS outcomes between KD parents and parents of a chronically ill child. None of the studied parental, child and disease characteristics were significantly associated with PPCV. Parents perceived their KD child more vulnerable to illness than healthy children, while in reality the majority had fully recovered from KD. ©2014 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  9. Whole genome sequencing of an African American family highlights toll like receptor 6 variants in Kawasaki disease susceptibility.

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    Jihoon Kim

    Full Text Available Kawasaki disease (KD is the most common acquired pediatric heart disease. We analyzed Whole Genome Sequences (WGS from a 6-member African American family in which KD affected two of four children. We sought rare, potentially causative genotypes by sequentially applying the following WGS filters: sequence quality scores, inheritance model (recessive homozygous and compound heterozygous, predicted deleteriousness, allele frequency, genes in KD-associated pathways or with significant associations in published KD genome-wide association studies (GWAS, and with differential expression in KD blood transcriptomes. Biologically plausible genotypes were identified in twelve variants in six genes in the two affected children. The affected siblings were compound heterozygous for the rare variants p.Leu194Pro and p.Arg247Lys in Toll-like receptor 6 (TLR6, which affect TLR6 signaling. The affected children were also homozygous for three common, linked (r2 = 1 intronic single nucleotide variants (SNVs in TLR6 (rs56245262, rs56083757 and rs7669329, that have previously shown association with KD in cohorts of European descent. Using transcriptome data from pre-treatment whole blood of KD subjects (n = 146, expression quantitative trait loci (eQTL analyses were performed. Subjects homozygous for the intronic risk allele (A allele of TLR6 rs56245262 had differential expression of Interleukin-6 (IL-6 as a function of genotype (p = 0.0007 and a higher erythrocyte sedimentation rate at diagnosis. TLR6 plays an important role in pathogen-associated molecular pattern recognition, and sequence variations may affect binding affinities that in turn influence KD susceptibility. This integrative genomic approach illustrates how the analysis of WGS in multiplex families with a complex genetic disease allows examination of both the common disease-common variant and common disease-rare variant hypotheses.

  10. Single-nucleotide polymorphism rs7251246 in ITPKC is associated with susceptibility and coronary artery lesions in Kawasaki disease.

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    Ho-Chang Kuo

    Full Text Available Kawasaki disease (KD is a multi-systemic vasculitis that preferentially affects children. A single nucleotide polymorphism (SNP in inositol 1,4,5-trisphosphate 3-kinase C (ITPKC has been identified to be an important polymorphism in the risk of KD. This study was conducted to comprehensively investigate the associations between all tagging SNPs of ITPKC in the risk of KD in a Taiwanese population. A total of 950 subjects (381 KD patients and 569 controls were recruited. Seven tagging SNPs (rs11673492, rs7257602, rs7251246, rs890934, rs10420685, rs2607420, rs2290692 were selected for TaqMan allelic discrimination assay. Clinical data of coronary artery lesions (CAL and aneurysms were collected for analysis. A significant association was found between rs7251246 in ITPKC and CAL formation. Haplotype analysis for ITPKC polymorphisms also confirmed this association in the patients with CAL and aneurysm formation. This is the first study to identify that SNP rs7251246 in ITPKC is associated with the severity of KD.

  11. DC-SIGN (CD209) Promoter −336 A/G (rs4804803) Polymorphism Associated with Susceptibility of Kawasaki Disease

    Science.gov (United States)

    Yu, Hong-Ren; Chang, Wei-Pin; Wang, Lin; Lin, Ying-Jui; Liang, Chi-Di; Yang, Kuender D.; Kuo, Chiu-Ming; Huang, Yi-Chuan; Chang, Wei-Chiao; Kuo, Ho-Chang

    2012-01-01

    Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is the most effective therapy for KD to reduce the prevalence of coronary artery lesion (CAL) formation. Recently, the α2, 6 sialylated IgG was reported to interact with a lectin receptor, specific intracellular adhesion molecule-3 grabbing nonintegrin homolog-related 1 (SIGN-R1) in mice and dendritic cell-specific intercellular adhesion molecule-3 grabbing nonintegrin (DC-SIGN) in human, and to trigger an anti-inflammatory cascade. This study was conducted to investigate whether the polymorphism of DC-SIGN (CD209) promoter −336 A/G (rs4804803) is responsible for susceptibility and CAL formation in KD patients using Custom TaqMan SNP Genotyping Assays. A total of 521 subjects (278 KD patients and 243 controls) were investigated to identify an SNP of rs4804803, and they were studied and showed a significant association between the genotypes and allele frequency of rs4804803 in control subjects and KD patients (P = 0.004 under the dominant model). However, the promoter variant of DC-SIGN gene was not associated with the occurrence of IVIG resistance, CAL formation in KD. The G allele of DC-SIGN promoter −336 (rs4804803) is a risk allele in the development of KD. PMID:22629172

  12. DC-SIGN (CD209) promoter -336 A/G (rs4804803) polymorphism associated with susceptibility of Kawasaki disease.

    Science.gov (United States)

    Yu, Hong-Ren; Chang, Wei-Pin; Wang, Lin; Lin, Ying-Jui; Liang, Chi-Di; Yang, Kuender D; Kuo, Chiu-Ming; Huang, Yi-Chuan; Chang, Wei-Chiao; Kuo, Ho-Chang

    2012-01-01

    Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is the most effective therapy for KD to reduce the prevalence of coronary artery lesion (CAL) formation. Recently, the α2, 6 sialylated IgG was reported to interact with a lectin receptor, specific intracellular adhesion molecule-3 grabbing nonintegrin homolog-related 1 (SIGN-R1) in mice and dendritic cell-specific intercellular adhesion molecule-3 grabbing nonintegrin (DC-SIGN) in human, and to trigger an anti-inflammatory cascade. This study was conducted to investigate whether the polymorphism of DC-SIGN (CD209) promoter -336 A/G (rs4804803) is responsible for susceptibility and CAL formation in KD patients using Custom TaqMan SNP Genotyping Assays. A total of 521 subjects (278 KD patients and 243 controls) were investigated to identify an SNP of rs4804803, and they were studied and showed a significant association between the genotypes and allele frequency of rs4804803 in control subjects and KD patients (P = 0.004 under the dominant model). However, the promoter variant of DC-SIGN gene was not associated with the occurrence of IVIG resistance, CAL formation in KD. The G allele of DC-SIGN promoter -336 (rs4804803) is a risk allele in the development of KD.

  13. 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.

  14. Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan.

    Science.gov (United States)

    Rodó, Xavier; Curcoll, Roger; Robinson, Marguerite; Ballester, Joan; Burns, Jane C; Cayan, Daniel R; Lipkin, W Ian; Williams, Brent L; Couto-Rodriguez, Mara; Nakamura, Yosikazu; Uehara, Ritei; Tanimoto, Hiroshi; Morguí, Josep-Anton

    2014-06-03

    Evidence indicates that the densely cultivated region of northeastern China acts as a source for the wind-borne agent of Kawasaki disease (KD). KD is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 y. We used residence times from simulations with the flexible particle dispersion model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. The postepidemic interval (1987-2010) and the extreme epidemics (1979, 1982, and 1986) pointed to the same source region. Results suggest a very short incubation period (Japan during the KD season detected major differences in the microbiota of the tropospheric aerosols compared with ground aerosols, with the unexpected finding of the Candida species as the dominant fungus from aloft samples (54% of all fungal strains). These results, consistent with the Candida animal model for KD, provide support for the concept and feasibility of a windborne pathogen. A fungal toxin could be pursued as a possible etiologic agent of KD, consistent with an agricultural source, a short incubation time and synchronized outbreaks. Our study suggests that the causative agent of KD is a preformed toxin or environmental agent rather than an organism requiring replication. We propose a new paradigm whereby an idiosyncratic immune response, influenced by host genetics triggered by an environmental exposure carried on winds, results in the clinical syndrome known as acute KD.

  15. On What Day of Illness Does the Dilatation of Coronary Arteries in Patients With Kawasaki Disease Begin?

    Science.gov (United States)

    Fuse, Shigeto; Mori, Toshihiko; Kuroiwa, Yuki; Hirakawa, Satoshi

    2017-12-25

    In the present study we used echocardiography to investigate coronary artery diameter at the time of diagnosis of Kawasaki disease (KD), before the start of treatment.Methods and Results:Diameters of the right, left main, left anterior descending, and left circumflex coronary arteries were determined in 410 patients before KD treatment commenced. The maximum Z-score was considered to be the pretreatment, maximum coronary artery Z-score (preZmax). The cumulative probability of coronary arterial dilatation was analyzed using the Kaplan-Meier method. In the present study, 31 patients (7.6%) had a preZmax ≥3.0, 56 (13.7%) had a preZmax ≥2.5, and 96 (23.4%) had a preZmax ≥2.0. The cumulative probability of a preZmax ≥2.0 was >20% on Day 5 of illness, 40% on Day 7, and 70% on Day 10. The positive predictive value (PPV) of a preZmax of 2.0 was approximately 0.9 on Day 5 of illness. The present study demonstrates that the coronary arteries may dilate before Day 5 of illness, and that the rate of dilatation increases gradually until Day 10. Because preZmax 2.0 has high PPV after Day 5 of illness, it is a useful marker of coronary artery dilatation in the early phase of KD.

  16. The Roles of Genetic Factors in Kawasaki Disease: A Systematic Review and Meta-analysis of Genetic Association Studies.

    Science.gov (United States)

    Xie, Xiaochuan; Shi, Xiaohan; Liu, Meilin

    2017-11-02

    This systematic review and meta-analysis aimed to better elucidate the roles of genetic factors in Kawasaki disease (KD), and determine the potential genetic biomarkers of KD. The systematic literature search of PubMed, Medline, Embase, Web of Science and CNKI identified 164 eligible studies. The qualitative synthesis revealed that 62 genes may be correlated with the susceptibility to KD, and 47 genes may be associated with the incidence of coronary artery lesions (CALs) in KD. A total of 53 polymorphisms in 34 genes were investigated in further quantitative synthesis. Of these, 23 gene polymorphisms were found to be significantly correlated with KD susceptibility, and 10 gene polymorphisms were found to be significantly associated with the incidence of CALs in KD. In conclusion, our findings indicate that gene polymorphisms of ACE, BLK, CASP3, CD40, FCGR2A, FGβ, HLA-E, IL1A, IL6, ITPKC, LTA, MPO, PD1, SMAD3, CCL17 and TNF may affect KD susceptibility. Besides, genetic variations in BTNL2, CASP3, FCGR2A, FGF23, FGβ, GRIN3A, HLA-E, IL10, ITPKC and TGFBR2 may serve as biomarkers of CALs in KD.

  17. [Effects of intravenous immunoglobulin and aspirin treatment on the functions of circulating endothelial progenitor cells in children with Kawasaki disease].

    Science.gov (United States)

    Xu, Ming-Guo; Men, Li-Na; Zu, Ying; Zhao, Chun-Yu; Meng, Xiang-Chun

    2011-12-01

    To study the effects of intravenous immunoglobulin (IVIG) and aspirin treatment on the functions of circulating endothelial progenitor cells (EPCs) in children with Kawasaki disease (KD) and possible mechanisms. Blood samples were obtained in 10 children with KD before and 7 days after the treatment by IVIG and aspirin. MTT method, modified Boyden chamber method and cell culture plate adhesion method were used to assess the functions of EPCs, including proliferation, adhension and migration activities. The plasma levels of tumor necrosis factor-α (TNF-α) and high-sensitivity C reactive protein (hs-CRP) were also measured. The functions of circulating EPCs 7 days after IVIG and aspirin treatment were significantly improved. IVIG and aspirin treatment significantly reduced plasma TNF-α and hs-CRP concentrations. There was a significant linear regression relationship between the reduced plasma TNF-α and hs-CRP levels and the increased functions of circulating EPCs. IVIG and aspirin treatment can improve the functions of circulating EPCs, possibly through reducing plasma concentrations of TNF-α and hs-CRP.

  18. Relationship between T-cell HLA-DR expression and intravenous immunoglobulin treatment response in Kawasaki disease.

    Science.gov (United States)

    Wakiguchi, Hiroyuki; Hasegawa, Shunji; Suzuki, Yasuo; Kudo, Keiko; Ichiyama, Takashi

    2015-04-01

    Kawasaki disease (KD) is an acute febrile illness associated with the development of vasculitis. Administration of intravenous immunoglobulin (IVIG) is the standard treatment for KD. However, IVIG treatment is not effective in approximately 15% of children with KD. Some reports have presented evidence of immunological responses in IVIG-resistant KD patients. We assessed the possibility that T-cell activation is a contributing mechanism underlying this phenomenon. We analyzed human leukocyte antigen-DR (HLA-DR) expression on peripheral blood CD4+ and CD8+ T cells in 82 children with KD who were admitted to the hospital between October 2007 and February 2012. We compared the percentages of HLA-DR+ T cells among the CD4+ T-cell and CD8+ T-cell populations for the IVIG-effective and IVIG-resistant groups. Among the 82 subjects, 51 had IVIG-effective KD and 31 children had IVIG-resistant KD. The percentages of HLA-DR+ T cells among the CD4+ T-cell and CD8+ T-cell populations in the IVIG-effective group were significantly lower than those in the IVIG-resistant group. Our results suggest that increased T-cell HLA-DR expression is associated with IVIG resistance in KD patients, indicating that HLA-DR expression would be a useful tool for predicting IVIG responsiveness during KD pathogenesis.

  19. Marked Acceleration of Atherosclerosis following Lactobacillus casei induced Coronary Arteritis in a Mouse Model of Kawasaki Disease

    Science.gov (United States)

    Chen, Shuang; Lee, Young Ho; Crother, Timothy R.; Fishbein, Michael; Zhang, Wenxuan; Yilmaz, Atilla; Shimada, Kenichi; Schulte, Danica J; Lehman, Thomas J.A.; Shah, Prediman K.; Arditi, Moshe

    2012-01-01

    Objective To investigate if Lactobacillus casei cell wall extract (LCWE)-induced Kawasaki Disease (KD) accelerates atherosclerosis in hypercholesterolemic mice. Method and Resuslts Apoe−/− or Ldlr−/− mice were injected with LCWE (KD mice) or PBS, fed high fat diet for 8 weeks, and atherosclerotic lesions in aortic sinuses (AS), arch (AC) and whole aorta were assessed. KD mice had larger, more complex aortic lesions with abundant collagen, and both extracellular and intracellular lipid and foam cells, compared to lesions in control mice despite similar cholesterol levels. Both Apoe−/− KD and Ldlr−/− KD mice showed dramatic acceleration in atherosclerosis vs. controls, with increases in en face aortic atherosclerosis and plaque size in both the AS and AC plaques. Accelerated atherosclerosis was associated with increased circulating IL-12p40, IFN-γ, TNF-α, and increased macrophage, DC, and T cell recruitment in lesions. Furthermore, daily injections of the IL-1Ra, which inhibits LCWE induced KD vasculitis, prevented the acceleration of atherosclerosis. Conclusions Our results suggest an important pathophysiologic link between coronary arteritis/vasculitis in the KD mouse model and subsequent atherosclerotic acceleration, supporting the concept that a similar relation may also be present in KD patients. These results also suggest that KD in childhood may predispose to accelerated and early atherosclerosis as adults. PMID:22628430

  20. Atypical localizations of hydatid disease: Experience from a single institute

    Directory of Open Access Journals (Sweden)

    Majid Mushtaque

    2012-01-01

    Full Text Available Introduction: The hydatid disease most often involves the liver and the lungs. The disease can involve any part of the body except the hair, teeth and nails. Primary extrahepatico-pulmonary hydatid cysts are rare and only a few sporadic cases have been reported. Materials and Methods: Two hundred and forty-four patients with hydatid cysts managed surgically from January 2005 to December 2009 were evaluated retrospectively. Fourteen (5.7% patients had isolated involvement of the atypical sites, while six (2.4% also had a primary involvement of liver. Results: The cysts were present in gall bladder (0.4%, peritoneum (1.6%, spleen (1.6%, ovary (0.4%, subcutaneous (0.8%, seminal vesicle (0.4%, spinal (0.4%, pancreas (0.4%, kidney (0.4%, mediastinal (0.4%, muscle (0.4%, and brain (0.8%. Discussion and Conclusions: Involvement of sites other than liver and lungs by hydatid disease is rare. Symptoms are related to size, location or possible complication of the cyst. It should be strongly suspected in differential diagnosis of all abdominal cysts especially in an endemic area. Proper surgical and medical management to avoid any recurrences, and a regular follow-up, are of utmost importance to detect any late complications such as local recurrence of the disease and development of hydatidosis at the primary sites.

  1. Update on diffusion MRI in Parkinson's disease and atypical parkinsonism.

    Science.gov (United States)

    Meijer, Frederick J A; Bloem, Bastiaan R; Mahlknecht, Philipp; Seppi, Klaus; Goraj, Bozena

    2013-09-15

    Differentiating Parkinson's disease (PD) from other types of neurodegenerative atypical parkinsonism (AP) can be challenging, especially in early disease stages. Routine brain magnetic resonance imaging (MRI) can show atrophy or signal changes in several parts of the brain with fairly high specificity for particular forms of AP, but the overall diagnostic value of routine brain MRI is limited. In recent years, various advanced MRI sequences have become available, including diffusion weighted imaging (DWI) and diffusion tensor imaging (DTI). Here, we review available literature on the value of diffusion MRI for identifying and quantifying different patterns of neurodegeneration in PD and AP, in relation to what is known of underlying histopathologic changes and clinical presentation of these diseases. Next, we evaluate the value of diffusion MRI to differentiate between PD and AP and the potential value of serial diffusion MRI to monitor disease progression. We conclude that diffusion MRI may quantify patterns of neurodegeneration which could be of additional value in clinical use. Future prospective clinical cohort studies are warranted to assess the added diagnostic value of diffusion MRI. © 2013 Elsevier B.V. All rights reserved

  2. Noninvasive quantification of coronary endothelial function by SPECT imaging in children with a history of Kawasaki disease

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    Cicala, Silvana; Paladini, Rodolfo; Leva, Francesco de [Santobono-Pausilipon Children Medical Hospital, Division of Cardiology, Department of Paediatrics, Naples (Italy); Pellegrino, Teresa; Caprio, Maria Grazia [Institute of Diagnostic and Nuclear Development, SDN Foundation, Naples (Italy); Storto, Giovanni [IRCCS, CROB, Rionero in Vulture (Italy); Mainolfi, Ciro; Cuocolo, Alberto [Federico II University, Department of Biomorphological and Functional Sciences, Naples (Italy); National Council of Research, Institute of Biostructures and Bioimages, Naples (Italy)

    2010-12-15

    The feasibility of coronary function estimation by single photon emission computed tomography (SPECT) has been recently demonstrated. The aim of this study was to apply SPECT imaging in patients with previous Kawasaki disease (KD) to assess the coronary functional status at long-term follow-up of the acute phase of the disease. Sixteen children with a history of KD underwent {sup 99m}Tc-sestamibi imaging at rest and during the cold pressor test (CPT). Myocardial blood flow (MBF) was estimated by measuring first transit counts in the pulmonary artery and myocardial counts from SPECT images. Coronary endothelial function was expressed as the ratio of the CPT to rest MBF. Six KD patients without coronary artery lesions served as controls and ten with coronary artery aneurysms during the acute phase of the disease were separated into two groups: group 1 (n = 4) with regressed and group 2 (n = 6) with persistent aneurysm at follow-up. The estimated coronary endothelial function was higher in controls compared to patients with coronary artery aneurysms (2.5 {+-} 0.3 vs 1.7 {+-} 0.7, p < 0.05). A significant difference in coronary endothelial function among groups was found (F = 5.21, p < 0.02). Coronary endothelial function was higher in patients of group 1 than in those of group 2 (1.9 {+-} 0.6 vs 1.4 {+-} 0.7, p < 0.02). SPECT may be applied as a noninvasive method for assessing coronary vascular function in children with a history of KD, demonstrating an impaired response to the CPT, an endothelial-dependent vasodilator stimulus. These findings reinforce the concept that coronary endothelial dysfunction may represent a long-term sequela of KD. (orig.)

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  6. Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

    Science.gov (United States)

    Bang, Ji Seok; Kwon, Bo Sang; Song, Mi Kyung; An, Hyo Soon; Song, Young Whan; Bae, Eun Jung; Noh, Chung Il

    2017-01-01

    Background and Objectives Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. Subjects and Methods The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. Results The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. Conclusions Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms. PMID:28765744

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

    Directory of Open Access Journals (Sweden)

    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.

  8. Allograft Inflammatory Factor-1 Links T-Cell Activation, Interferon Response, and Macrophage Activation in Chronic Kawasaki Disease Arteritis.

    Science.gov (United States)

    Rowley, Anne H; Baker, Susan C; Kim, Kwang-Youn A; Shulman, Stanford T; Yang, Amy; Arrollo, David; DeBerge, Matthew; Han, Shuling; Sibinga, Nicholas E S; Pink, Adam J; Thorp, Edward B

    2017-09-01

    Kawasaki disease (KD) is widely viewed as an acute arteritis. However, our pathologic studies show that chronic coronary arteritis can persist long after disease onset and is closely linked with arterial stenosis. Transcriptome profiling of acute KD arteritis tissues revealed upregulation of T lymphocyte, type I interferon, and allograft inflammatory factor-1 (AIF1) genes. We determined whether these immune responses persist in chronic KD arteritis, and we investigated the role of AIF1 in these responses. Gene expression in chronic KD and childhood control arteries was determined by real-time reverse-transcriptase polymerase chain reaction, and arterial protein expression was determined by immunohistochemistry and immunofluorescence. Allograft inflammatory factor-1 small-interfering ribonucleic acid macrophage treatment was performed to investigate the role of AIF1 in macrophage and T lymphocyte activation. Allograft inflammatory factor-1 protein was highly expressed in stenotic KD arteries and colocalized with the macrophage marker CD68. T lymphocyte and interferon pathway genes were significantly upregulated in chronic KD coronary artery tissues. Alpha interferon-induced macrophage expression of CD80 and major histocompatibility complex class II was dependent on AIF1, and macrophage expression of AIF1 was required for antigen-specific T lymphocyte activation. Allograft inflammatory factor-1, originally identified in posttransplant arterial stenosis, is markedly upregulated in KD stenotic arterial tissues. T lymphocyte and type I interferon responses persist in chronic KD arteritis. Allograft inflammatory factor-1 may play multiple roles linking type I interferon response, macrophage activation, and antigen-specific T lymphocyte activation. These results suggest the likely importance of lymphocyte-myeloid cell cross-talk in the pathogenesis of KD arteritis and can inform selection of new immunotherapies for clinical trials in high-risk KD children.

  9. Clarithromycin Plus Intravenous Immunoglobulin Therapy Can Reduce the Relapse Rate of Kawasaki Disease: A Phase 2, Open-Label, Randomized Control Study.

    Science.gov (United States)

    Nanishi, Etsuro; Nishio, Hisanori; Takada, Hidetoshi; Yamamura, Kenichiro; Fukazawa, Mitsuharu; Furuno, Kenji; Mizuno, Yumi; Saigo, Kenjiro; Kadoya, Ryo; Ohbuchi, Noriko; Onoe, Yasuhiro; Yamashita, Hironori; Nakayama, Hideki; Hara, Takuya; Ohno, Takuro; Takahashi, Yasuhiko; Hatae, Ken; Harada, Tatsuo; Shimose, Takayuki; Kishimoto, Junji; Ohga, Shouichi; Hara, Toshiro

    2017-07-06

    We previously reported that biofilms and innate immunity contribute to the pathogenesis of Kawasaki disease. Therefore, we aimed to assess the efficacy of clarithromycin, an antibiofilm agent, in patients with Kawasaki disease. We conducted an open-label, multicenter, randomized, phase 2 trial at 8 hospitals in Japan. Eligible patients included children aged between 4 months and 5 years who were enrolled between days 4 and 8 of illness. Participants were randomly allocated to receive either intravenous immunoglobulin (IVIG) or IVIG plus clarithromycin. The primary end point was the duration of fever after the initiation of IVIG treatment. Eighty-one eligible patients were randomized. The duration of the fever did not differ between the 2 groups (mean±SD, 34.3±32.4 and 31.1±31.1 hours in the IVIG plus clarithromycin group and the IVIG group, respectively [ P =0.66]). The relapse rate of patients in the IVIG plus clarithromycin group was significantly lower than that in the IVIG group (12.5% versus 30.8%, P =0.046). No serious adverse events occurred during the study period. In a post hoc analysis, the patients in the IVIG plus clarithromycin group required significantly shorter mean lengths of hospital stays than those in the IVIG group (8.9 days versus 10.3 days, P =0.049). Although IVIG plus clarithromycin therapy failed to shorten the duration of fever, it reduced the relapse rate and shortened the duration of hospitalization in patients with Kawasaki disease. URL: http://www.umin.ac.jp/ctr/index.htm. Unique identifier: UMIN000015437. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

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

    Directory of Open Access Journals (Sweden)

    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

  11. The outcome of short-term low-dose aspirin treatment in Kawasaki disease based on inflammatory markers.

    Science.gov (United States)

    Yoo, Jae Won; Kim, Ji Mok; Kil, Hong Ryang

    2017-01-01

    Previously, Kawasaki disease (KD) treatment with low-dose aspirin was administered for 6-8 weeks after the acute phase. However, inflammatory marker levels normalize before 6-8 weeks. In this study, we aimed to investigate the clinical outcome of short-term low-dose aspirin treatment based on inflammatory and thrombotic marker levels. We performed a retrospective review of the medical records of patients with KD who were hospitalized at Chungnam National University Hospital between September 2012 and May 2014. When fever subsided, low-dose aspirin treatment was started. Inflammatory (white blood cell count, erythrocyte sedimentation rate, and C-reactive protein) and thrombotic markers (D-dimer) were monitored at follow-ups conducted in 1- to 2-week intervals. The low-dose aspirin administration was terminated when both markers were normalized and no cardiovascular complications were observed. Eighty-four patients with KD (complete KD, n=49; incomplete KD, n=35) were enrolled. The inflammatory and thrombotic marker levels were normalized within 3-4 weeks on average. At the beginning the low-dose aspirin treatment, 9 patients had coronary artery lesions but 75 did not. When the low-dose aspirin administration was terminated at the time the inflammatory marker levels were normalized, no new CALs developed during the follow-up at 6-8 weeks. Most of the inflammatory marker levels were normalized within 3-4 weeks after the acute phase of KD. New cardiovascular complications did not develop during the course of the short-term aspirin treatment based on the inflammatory marker levels, clinical findings, and echocardiography.

  12. Unique Molecular Patterns Uncovered in Kawasaki Disease Patients with Elevated Serum Gamma Glutamyl Transferase Levels: Implications for Intravenous Immunoglobulin Responsiveness.

    Directory of Open Access Journals (Sweden)

    Yue Wang

    Full Text Available Resistance to intravenous immunoglobulin (IVIG occurs in 10-20% of patients with Kawasaki disease (KD. The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels.We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I. Gene expression data from 130 children with acute KD (Cohort II were analyzed using the R square statistic and false discovery analysis to identify genes that were differentially represented in patients with elevated GGT levels with regard to IVIG responsiveness. Two additional KD cohorts (Cohort III and IV were used to test the hypothesis that sialylation and GGT may be involved in IVIG resistance through neutrophil apoptosis.Thirty-six genes were identified that significantly explained the variations of both GGT levels and IVIG responsiveness in KD patients. After Bonferroni correction, significant associations with IVIG resistance persisted for 12 out of 36 genes among patients with elevated GGT levels and none among patients with normal GGT levels. With the discovery of ST6GALNAC3, a sialyltransferase, as the most differentially expressed gene, we hypothesized that sialylation and GGT are involved in IVIG resistance through neutrophil apoptosis. We then confirmed that in Cohort III and IV there was significantly less reduction in neutrophil count in IVIG non-responders.Gene expression analyses combining molecular and clinical datasets support the hypotheses that: (1 neutrophil apoptosis induced by IVIG may be a mechanism of action of IVIG in KD; (2 changes in sialylation and GGT level in KD patients may contribute synergistically to IVIG resistance through blocking IVIG-induced neutrophil apoptosis. These findings have implications for understanding the mechanism of action in IVIG resistance, and

  13. A genome-wide association analysis identifies NMNAT2 and HCP5 as susceptibility loci for Kawasaki disease.

    Science.gov (United States)

    Kim, Jae-Jung; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Ryang; Kim, Gi Beom; Han, Myung-Ki; Song, Min Seob; Lee, Hyoung Doo; Ha, Kee Soo; Sohn, Sejung; Johnson, Todd A; Takahashi, Atsushi; Kubo, Michiaki; Tsunoda, Tatsuhiko; Ito, Kaoru; Onouchi, Yoshihiro; Hong, Young Mi; Jang, Gi Young; Lee, Jong-Keuk

    2017-08-31

    Kawasaki disease (KD), a systemic vasculitis of infants and children, manifests as fever and mucocutaneous inflammation. Although its etiology is largely unknown, the epidemiological data suggest that genetic factors are important in KD susceptibility. To identify genetic variants influencing KD susceptibility, we performed a genome-wide association study (GWAS) and replication study using a total of 915 children with KD and 4553 controls in the Korean population. Six single-nucleotide polymorphisms (SNPs) in three loci were associated significantly with KD susceptibility (P<1.0 × 10(-5)), including the previously reported BLK locus (rs6993775, odds ratio (OR)=1.52, P=2.52 × 10(-11)). The other two loci were newly identified: NMNAT2 on chromosome 1q25.3 (rs2078087, OR=1.33, P=1.15 × 10(-6)) and the human leukocyte antigen (HLA) region on chromosome 6p21.3 (HLA-C, HLA-B, MICA and HCP5) (rs9380242, rs9378199, rs9266669 and rs6938467; OR=1.33-1.51, P=8.93 × 10(-6) to 5.24 × 10(-8)). Additionally, SNP rs17280682 in NLRP14 was associated significantly with KD with a family history (18 cases vs 4553 controls, OR=6.76, P=5.46 × 10(-6)). These results provide new insights into the pathogenesis and pathophysiology of KD.Journal of Human Genetics advance online publication, 31 August 2017; doi:10.1038/jhg.2017.87.

  14. Common variants in the CRP promoter are associated with a high C-reactive protein level in Kawasaki disease.

    Science.gov (United States)

    Kim, Jae-Jung; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Ryang; Kim, Gi Beom; Han, Myung Ki; Song, Min Seob; Lee, Hyoung Doo; Byeon, Jung Hye; Sohn, Saejung; Hong, Young Mi; Jang, Gi Young; Lee, Jong-Keuk

    2015-02-01

    Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10(-6) according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10(-8) according to the replication study; beta = 3.97 and p = 1.11 × 10(-13) according to combined analysis) and explained 8.1% of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1% of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.

  15. Erdheim-Chester disease: atypical presentation of a rare disease.

    Science.gov (United States)

    Calandra, Cristian Ricardo; Bustos, Ariel; Falcon, Florencia; Arakaki, Naomi

    2017-10-11

    We report the clinical case of an adult patient referred to our hospital because of trismus due to a tumour in the right infratemporal and pterygomaxillary fossa. He referred hyporexia, weight loss and right trigeminal neuralgia. On physical examination, he had trismus and diplopia. On neuroimaging, the tumour invaded the central nervous system affecting the right temporal lobe and orbit, and the sellar region. Tumour biopsy revealed foamy histiocytes and isolated giant multinuclear cells immunoreactive to CD68 and negative to CD1a and S100. A diagnosis of Erdheim-Chester disease was made. Non-evidence of large bone involvement was found in neither plain radiographs nor Technetium 99 m bone scintigraphy. BRAFV600E mutation analysis was negative. Because of raised intracranial pressure, a debulking surgery of the intracranial histiocytic process was performed. The patient improved his symptoms and remains clinically stable after 12 months of treatment with pegylated interferon-α-2a 180 µg/weekly. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Mid-term Risk for Subclinical Atherosclerosis and Chronic Myocarditis in Children with Kawasaki Disease and Transient Coronary Abnormalities.

    Science.gov (United States)

    Parihar, Mansingh; Singh, Surjit; Vignesh, Pandiarajan; Gupta, Anju; Rohit, Manojkumar

    2017-08-01

    There is evidence for premature atherosclerosis and systemic arterial stiffening during follow-up of children with Kawasaki disease (KD) and coronary artery abnormalities (CAA). Moreover, patients with KD may also have subclinical myocardial involvement and inhomogeneous ventricular repolarization. The inhomogeneous ventricular repolarization manifests as increased QT dispersion on electrocardiography. There is a paucity of studies in endothelial dysfunction and QT dispersion in children with KD and transient CAA. Twenty children with KD and transient CAA were studied at least 1 year after resolution of CAA. Mean follow-up period between KD onset and enrolment in the study was 53.7 months. Twenty age and sex-matched controls were enrolled. High-resolution B-mode ultrasonography was used to analyze brachial artery dilatation in response to reactive hyperemia (cases and controls) and sublingual nitroglycerine (cases only). Carotid artery intima-media thickness (cIMT) and stiffness index were calculated. The difference between maximum and minimum QTc intervals on 12 lead electrocardiogram was calculated as QTc dispersion (QTcd). No statistically significant difference was noted in percent flow-mediated dilatation of brachial arteries in response to reactive hyperemia between cases (13.31 ± 10.41%) and controls (12.86 ± 7.09%). Sublingual nitroglycerine-mediated dilatation in children with KD was 14.88 ± 12.03%. Mean cIMT was similar in cases (0.036 ± 0.015 cm) and controls (0.035 ± 0.076 cm; p = 0.791). No statistically significant difference between groups was observed in mean QTcd values (0.057 ± 0.018 s vs. 0.059 ± 0.015 s in controls, p = 0.785). No evidence of significant endothelial dysfunction or increased QT dispersion in patients with KD and transient coronary artery abnormalities was found in our cohort when studied at a mean follow-up of 53.7 months. This is reassuring, and indicates that risk of subclinical atherosclerosis

  19. Microbleeds in atypical presentations of Alzheimer's disease: a comparison to dementia of the Alzheimer's type.

    Science.gov (United States)

    Whitwell, Jennifer L; Kantarci, Kejal; Weigand, Stephen D; Lundt, Emily S; Gunter, Jeffrey L; Duffy, Joseph R; Strand, Edythe A; Machulda, Mary M; Spychalla, Anthony J; Drubach, Daniel A; Petersen, Ronald C; Lowe, Val J; Jack, Clifford R; Josephs, Keith A

    2015-01-01

    Microbleeds in the brain have been shown to occur in Alzheimer's disease (AD), affecting approximately a third of subjects that present with typical dementia of the Alzheimer's type (DAT). However, little is known about the frequency or distribution of microbleeds in subjects with AD that present with atypical clinical presentations. To determine whether the frequency and regional distribution of microbleeds in atypical AD differs from that observed in subjects with DAT, and to determine whether microbleeds in atypical AD are associated with age, demographics, or cognitive impairment. Fifty-five subjects with amyloid-β deposition on Pittsburgh compound B (PiB) PET who presented with predominant language (n = 37) or visuospatial/perceptual (n = 18) deficits underwent T2*weighted MRI. These subjects were compared to 41 PiB-positive subjects with DAT. Microbleeds were identified and assigned a lobar location. The proportion of subjects with microbleeds did not differ between atypical AD (42%) and DAT (32%). However, atypical AD had larger numbers of microbleeds than DAT. In addition, the topographic distribution of microbleeds differed between atypical AD and DAT, with atypical AD showing the highest density of microbleeds in the frontal lobes. Among atypical AD, number of microbleeds was associated with age, but not gender or cognition. Microbleeds were more common in subjects with language (51%) versus visuospatial/perceptual deficits (22%). Microbleeds are relatively common in both DAT and atypical AD, although atypical AD subjects appear to be at particular risk for developing large numbers of microbleeds and for developing microbleeds in the frontal lobe.

  20. Atypical Localizations of Hydatid Disease: Experience from a Single ...

    African Journals Online (AJOL)

    Departments of General Surgery, 1Radiodiagnosis and Imaging and 2Neurosurgery, Sheri Kashmir Institute of Medical. Sciences, Soura, Srinagar, Jammu and ... development of hydatidosis at the primary sites. KEYWORDS: Atypical locations ..... Conflict of Interest: None declared. 24. Senyuz OF, Yesildag E, Celayir S.

  1. Typical and atypical appearance of early-onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study.

    Science.gov (United States)

    Kawakatsu, Shinobu; Kobayashi, Ryota; Hayashi, Hiroshi

    2017-04-01

    The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia. We reported MRI, single-photon emission CT and neuropathological findings in six representative cases. We also described a "left temporal variant" of AD presenting with transcortical cortical sensory aphasia, which has not been reported previously and is another subtype of the posterior variant of AD. We found a significant correlation between regional cerebral blood flow and counts of NFTs in the cerebral cortices. An atypical presentation with focal neuropsychological symptoms roughly correlated with the density of NFTs in the cerebral cortex and more directly related to spongiform changes in the superficial layers of these areas. In contrast, the distribution of amyloid depositions was diffuse and did not necessarily correlate with focal neuropsychological symptoms. Braak staging or ABC score is not necessarily appropriate to evaluate atypical AD, and instead, spongiform changes in addition to tau pathology in the association cortices better explain the diversity of atypical AD. Interestingly, another patient with a posterior variant of AD had a novel type of atypical plaque, which we referred to as "lucent plaque". They were recognizable with HE staining in the circumference and dystrophic neurites were abundant with Gallyas-Braak staining. These plaques demonstrated intense immunoreactivity to both tau AT-8 and amyloid β (Aβ), suggesting a peculiar coexistence pattern of amyloid and tau in these plaques. Clinicopathological studies

  2. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.

    Science.gov (United States)

    McCrindle, Brian W; Rowley, Anne H; Newburger, Jane W; Burns, Jane C; Bolger, Anne F; Gewitz, Michael; Baker, Annette L; Jackson, Mary Anne; Takahashi, Masato; Shah, Pinak B; Kobayashi, Tohru; Wu, Mei-Hwan; Saji, Tsutomu T; Pahl, Elfriede

    2017-04-25

    Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. © 2017 American Heart Association, Inc.

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

    Science.gov (United States)

    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

  4. Catheter-based anatomic and functional assessment of coronary arteries in anomalous aortic origin of a coronary artery, myocardial bridges and Kawasaki disease.

    Science.gov (United States)

    Qureshi, Athar M; Agrawal, Hitesh

    2017-09-01

    Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar with these techniques. In this article, the role of angiography, intravascular ultrasound, fractional flow reserve, and optical coherence tomography in these patients is reviewed. © 2017 Wiley Periodicals, Inc.

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

    DEFF Research Database (Denmark)

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

    2017-01-01

    Since 2008, outbreaks of atypical hand, foot, and mouth disease (HFMD) in children and adults have been reported worldwide. The majority of these outbreaks are caused by a new lineage of Coxsackie virus A6 (CV-A6) presenting a more severe clinical phenotype than the classical childhood HFMD caused...... by CV-A16. Between June 2014 and January 2016, 23 cases of atypical HFMD disease presented at a Dermatology Department at a regional University Hospital in Denmark. Patients were referred by general practitioners and dermatologists with a variety of clinical diagnoses, including eczema herpeticum...... caused by CV-A6 in the Region of Southern Denmark and that atypical HFMD can be difficult to diagnose clinically as it may mimic other severe skin diseases....

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

    DEFF Research Database (Denmark)

    Lindquist, Suzanne Granhøj; Nielsen, Jørgen Erik; Stokholm, Jette

    2008-01-01

    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 fam......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...

  7. Mortality among persons with a history of Kawasaki disease in Japan: can paediatricians safely discontinue follow-up of children with a history of the disease but without cardiac sequelae?

    Science.gov (United States)

    Nakamura, Yosikazu; Aso, Eiko; Yashiro, Mayumi; Uehara, Ritei; Watanabe, Makoto; Tajimi, Morihiro; Oki, Izumi; Ojima, Toshiyuki; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2005-04-01

    To clarify the question of whether patients with Kawasaki disease suffer a higher mortality rate after the incidence of the disease in comparison with age-matched healthy individuals. Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new, definite diagnosis of Kawasaki disease. Patients were followed up until 31 December 2001 or their death. The expected number of deaths was calculated from Japanese vital statistics data and compared with the observed number. Of 6576 patients enrolled, 29 (20 males and 9 females) died. The standardized mortality ratio (SMR: the observed number of deaths divided by the expected number of deaths based on the vital statistics in Japan) was 1.15 (95% CI: 0.77-1.66). In spite of the high SMRs during the acute phase, the mortality rate was not high after the acute phase for the entire group of patients. Although the SMR after the acute phase was 0.75 for those without cardiac sequelae, six males (but none of the females) with cardiac sequelae died during this period; and the SMR for the male group with cardiac sequelae was 1.95 (95% CI: 0.71-4.25). The mortality from congenital anomalies of the circulatory system was elevated, but no increase in cancer deaths was observed. Although it was not statistically significant, the mortality rate among males with cardiac sequelae due to Kawasaki disease appeared to be higher than in the general population. On the other hand, the mortality rates for females with the sequelae and both males and females without sequelae were not elevated.

  8. 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.

  9. Inkomplet Kawasakis syndrom med koronaraneurisme hos et spædbarn

    DEFF Research Database (Denmark)

    Vinding, Rebecca Kofod; Debes, Nanette Mol; Stantchev, Hristo

    2014-01-01

    We report a four-month-old boy with incomplete Kawasaki syndrome. He developed a coronary artery aneurysm. Incomplete Kawasaki syndrome is rare in Denmark, and only reported in five patient cases in the past 25 years. It is difficult to diagnose Kawasaki disease; early diagnosis and treatment...... reduces the risk for cardiac sequel. In lack of diagnostic test, we recommend that the algorithm from the American Heart Association to diagnose incomplete Kawasaki is implemented in Denmark. The algorithm is based on classic clinical features, echocardiography and laboratory findings....

  10. Estudo prospectivo das complicações da Doença de Kawasaki: análise de 115 casos Prospective study of Kawasaki Disease complications: review of 115 cases

    Directory of Open Access Journals (Sweden)

    Natália Ribeiro de M. Alves

    2011-06-01

    após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações.OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7% had coronary aneurisms. Thirty eight patients (33% had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3% maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9%, ataxia in acute and subacute phases in 11 (9.5%; 15 patients had ophthalmologic complications (13.2%, with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9%, with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9% and another patient had a necrotizing vasculitis

  11. Examination of Huntington's disease with atypical clinical features in a Bangladeshi family tree.

    Science.gov (United States)

    Al-Mamun, Md Mahfuz; Sarker, Suprovath Kumar; Qadri, Syeda Kashfi; Shirin, Tahmina; Mohammad, Quazi Deen; LaRocque, Regina; Karlsson, Elinor K; Saha, Narayan; Asaduzzaman, Muhammad; Qadri, Firdausi; Mannoor, Md Kaiissar

    2016-12-01

    Atypical manifestation of Huntington's disease (HD) could inform ongoing research into HD genetic modifiers not present in the primarily European populations studied to date. This work demonstrates that expanding HD genetic testing into under-resourced healthcare settings can benefit both local communities and ongoing research into HD etiology and new therapies.

  12. 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...... epilepsy diagnosis against 11 (4.3%) in the comparison group (P ...

  13. Atypical extensive genital ulcer in full blown aids with slim disease ...

    African Journals Online (AJOL)

    Background: Atypical and exceptional clinical presentation of full blown AIDS may be observed in sub-Saharan Africa. We report herein the case of a Central African 37-year-old male patient presented with full blown AIDS, a typical picture of slim disease with marked cachexia and wasting faces. In addition, the patient was ...

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

    Directory of Open Access Journals (Sweden)

    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

  15. Atypical hand-foot-and-mouth disease associated with coxsackievirus A6 infection.

    Science.gov (United States)

    Lott, Jason P; Liu, Kristina; Landry, Marie-Louise; Nix, W Allan; Oberste, M Steven; Bolognia, Jean; King, Brett

    2013-11-01

    Hand-foot-and-mouth disease (HFMD) is an acute viral illness commonly caused by coxsackievirus (CV)-A16 and enterovirus 71 infections. Recently, atypical HFMD has been reported in association with CV-A6, an uncommon enterovirus strain. We sought to describe the clinical features of atypical HFMD associated with CV-A6 infection and its diagnostic laboratory evaluation. Patients presenting to our institution with history and examination suggestive of atypical HFMD from January 2012 to July 2012 were identified. Morphology and distribution of mucocutaneous lesions were recorded. Enterovirus infection was assessed by reverse transcriptase polymerase chain reaction of biologic specimens. Enterovirus type was determined by viral capsid protein 1 gene sequencing. Two adults and 3 children with atypical HFMD were identified. Four of 5 patients exhibited widespread cutaneous lesions. In 2 patients with a history of atopic dermatitis, accentuation in areas of dermatitis was noted. Associated systemic symptoms prompted 4 of 5 patients to seek emergency care, and both adults were hospitalized for diagnostic evaluation. Infection with CV-A6 was confirmed in all patients. This study is a case series from a single institution. Consideration of the expanded range of cutaneous findings in atypical HFMD caused by CV-A6 infection may assist clinicians in diagnosis and management. Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  16. Enfermedad de Kawasaki

    Directory of Open Access Journals (Sweden)

    Sandra Matiz Mejía

    2017-05-01

    Full Text Available La enfermedad de Kawasaki es un estado febril agudo, cuya incidencia es de 175 por cada 100.000 niños menores de 5 años. Es una vasculitis con predilección por los vasos de pequeño y mediano calibre, especialmente por las arterias coronarias; sin tratamiento pueden desarrollarse aneurismas coronarios que conllevan riesgo de infarto agudo de miocardio y muerte súbita. Se describe el caso de un niño de 4 años a quien se diagnosticó enfermedad de Kawasaki y aneurismas fusiformes moderados en las arterias coronarias derecha e izquierda. En control a los dos meses desarrolló nuevo cuadro febril con hipercolesterolemia, trombocitosis y alteración de las pruebas hepáticas. En ecocardiograma de control se evidenció aneurisma gigante en la coronaria izquierda y mediante angiografía coronaria se detectaron dos aneurismas en la coronaria derecha. En vista de que se consideró un caso de evolución atípica, se realizó revisión de la literatura mundial en enfermedad de Kawasaki y se recomendó diagnóstico y tratamiento precoz para disminuir la morbilidad con compromiso cardiovascular severo y/o la mortalidad.

  17. Genetic Variations in the Receptor-Ligand Pair CCR5 and CCL3L1 Are Important Determinants of Susceptibility to Kawasaki Disease

    Science.gov (United States)

    Burns, Jane C.; Shimizu, Chisato; Gonzalez, Enrique; Kulkarni, Hemant; Patel, Sukeshi; Shike, Hiroko; Sundel, Robert S.; Newburger, Jane W.; Ahuja, Sunil K.

    2010-01-01

    Kawasaki disease (KD) is an enigmatic, self-limited vasculitis of childhood that is complicated by development of coronary-artery aneurysms. The high incidence of KD in Asian versus European populations prompted a search for genetic polymorphisms that are differentially distributed among these populations and that influence KD susceptibility. Here, we demonstrate a striking, inverse relationship between the worldwide distribution of CCR5-Δ32 allele and the incidence of KD. In 164 KD patient-parent trios, 4 CCR5 haplotypes including the CCR5-Δ32 allele were differentially transmitted from heterozygous parents to affected children. However, the magnitude of the reduced risk of KD associated with the CCR5-Δ32 allele and certain CCR5 haplotypes was significantly greater in individuals who also possessed a high copy number of the gene encoding CCL3L1, the most potent CCR5 ligand. These findings, derived from the largest genetic study of any systemic vasculitis, suggest a central role of CCR5-CCL3L1 gene-gene interactions in KD susceptibility and the importance of gene modifiers in infectious diseases. PMID:15962231

  18. The functions of endothelial progenitor cells were significantly improved after treatment with intravenous immunoglobulin and aspirin in children with Kawasaki disease.

    Science.gov (United States)

    Xu, Ming Guo; Men, Li Na; Zu, Ying; Zhao, Chun Yu; Meng, Xiang Chun; Wang, Tao

    2011-04-01

    We sought to determine the effects of treatment with intravenous immunoglobulin (IVIG) and aspirin on the functions of endothelial progenitor cells (EPCs) in patients with Kawasaki disease (KD) as well as its relationship with concentrations of tumor necrosis factor-α (TNF-α) and high-sensitivity C-reactive protein (hs-CRP). Ten KD patients in the acute phase of their disease were recruited. We investigated EPC functions in children with KD before and after treatment with IVIG and aspirin. In vitro assays were used to measure the functions, including proliferation, adhesion, and migration activities, of EPCs. Plasma levels of TNF-α and hs-CRP were also assessed. All of the data were assessed before and at 7 days after treatment initiation. EPC functions after 7 days of treatment with IVIG and aspirin were significantly improved than they were before treatment with IVIG and aspirin. Treatment with IVIG and aspirin significantly decreased TNF-α and hs-CRP concentrations. There was a significant linear regression relationship between decreased plasma TNF-α levels, hs-CRP levels, and increased functions of circulating EPCs. The results of our study indicate that the functions of circulating EPCs improved after treatment with IVIG and aspirin, which may be related to decreased concentrations of TNF-α and hs-CRP.

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

    DEFF Research Database (Denmark)

    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...

  20. Diffuse Caroli's disease with atypical presentation: a case report.

    Science.gov (United States)

    Acioli, Maíra Lima; Costa, Lawrence Raizama Gonçalves; de Miranda Henriques, Mônica Souza

    2014-01-01

    This paper describes a case of Caroli's disease in a female patient aged 32, who complained of nonspecific abdominal pain without cholesthasis or cholangitis. Liver resonance shows segment saccular dilations closely connected to intrahepatic biliary ducts, that differ from the Caroli's syndrome, which is more common and consists of multiple intrahepatic cystic dilatations, associated to congenital hepatic fibrosis. This patient has a congenital anomaly with an uncommon oligosymptomatic form of Caroli's disease that should be included in the differential diagnosis of patients with recurrent abdominal pain.

  1. Clinicopathologic analysis of atypical hand, foot, and mouth disease in adult patients.

    Science.gov (United States)

    Second, Julie; Velter, Charles; Calès, Sophie; Truchetet, François; Lipsker, Dan; Cribier, Bernard

    2017-04-01

    Hand, foot, and mouth disease is a contagious viral infection usually affecting children. A resurgence of cases in adults, mainly caused by coxsackievirus A6 and with an atypical and more severe presentation, has taken place. The goal was to examine the clinical, histologic, and immunohistochemical features of this disease in adults. This is a retrospective study on documented cases of adult hand, foot, and mouth disease from France's Dermatology Department of Strasbourg University Hospital and Bel-Air Hospital in Thionville. Six patients with severe and atypical presentation were included, 4 caused by coxsackievirus A6. The histologic features were: spongiosis, neutrophilic exocytosis, massive keratinocyte necrosis, shadow cells in the upper epidermis, vacuolization of basal cells, necrotic cells in follicles and sweat glands, dense superficial dermal infiltrate of CD3+ lymphocytes, and strong granulysin expression. This is a retrospective case series. In adult patients presenting with atypical hand, foot, and mouth disease caused by coxsackievirus A6, biopsy specimens show distinctive changes in the epidermis but also in adnexal structures. The inflammatory infiltrate is made of T cells with a cytotoxic profile, with numerous granulysin-positive cells, as observed in severe drug-induced eruption with necrosis of keratinocytes. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  2. Atypical Localizations of Hydatid Disease: Experience from a Single ...

    African Journals Online (AJOL)

    Echinococcal disease in humans is a parasitic tapeworm infection caused by a larval stage (the metacestode) of ... definitive host and sheep, swine, cattle, and zebra as the intermediate host. Man is an accidental, intermediate .... in countries like Australia, New Zealand, Middle East, Africa,. India, South America, Turkey, and ...

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

    NARCIS (Netherlands)

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

    2016-01-01

    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

  4. Association of CCR2-CCR5 haplotypes and CCL3L1 copy number with Kawasaki Disease, coronary artery lesions, and IVIG responses in Japanese children.

    Directory of Open Access Journals (Sweden)

    Manju Mamtani

    2010-07-01

    Full Text Available The etiology of Kawasaki Disease (KD is enigmatic, although an infectious cause is suspected. Polymorphisms in CC chemokine receptor 5 (CCR5 and/or its potent ligand CCL3L1 influence KD susceptibility in US, European and Korean populations. However, the influence of these variations on KD susceptibility, coronary artery lesions (CAL and response to intravenous immunoglobulin (IVIG in Japanese children, who have the highest incidence of KD, is unknown.We used unconditional logistic regression analyses to determine the associations of the copy number of the CCL3L1 gene-containing duplication and CCR2-CCR5 haplotypes in 133 Japanese KD cases [33 with CAL and 25 with resistance to IVIG] and 312 Japanese controls without a history of KD. We observed that the deviation from the population average of four CCL3L1 copies (i.e., four copies was associated with an increased risk of KD and IVIG resistance (adjusted odds ratio (OR=2.25, p=0.004 and OR=6.26, p=0.089, respectively. Heterozygosity for the CCR5 HHF*2 haplotype was associated with a reduced risk of both IVIG resistance (OR=0.21, p=0.026 and CAL development (OR=0.44, p=0.071.The CCL3L1-CCR5 axis may play an important role in KD pathogenesis. In addition to clinical and laboratory parameters, genetic markers may also predict risk of CAL and resistance to IVIG.

  5. Infliximab versus intravenous immunoglobulin for refractory Kawasaki disease: a phase 3, randomized, open-label, active-controlled, parallel-group, multicenter trial.

    Science.gov (United States)

    Mori, Masaaki; Hara, Takuma; Kikuchi, Masako; Shimizu, Hiroyuki; Miyamoto, Tomoyuki; Iwashima, Satoru; Oonishi, Tatsuya; Hashimoto, Kunio; Kobayashi, Norimoto; Waki, Kenji; Suzuki, Yasuo; Otsubo, Yoshikazu; Yamada, Hiroshi; Ishikawa, Chikao; Kato, Taichi; Fuse, Shigeto

    2018-01-31

    We compared the efficacy and safety of infliximab with intravenous immunoglobulin (IVIG), a standard therapy, in a phase 3 trial (NCT01596335) for Japanese patients with Kawasaki disease (KD) showing persistent fever after initial IVIG. Patients with initial IVIG-refractory KD, aged 1-10 years, received a single dose of IV infliximab 5 mg/kg or IV polyethylene glycol-treated human immunoglobulin (VGIH) 2 g/kg on day 0. Primary outcome was defervescence rate within 48 h after the start of treatment. Safety was evaluated through day 56. Overall, 31 patients were randomized (infliximab, n = 16; VGIH, n = 15); 31.3% and 60.0% patients discontinued due to worsening KD. Defervescence rate within 48 h was greater with infliximab (76.7%) than VGIH (37.0%) (p = 0.023), and defervescence was achieved earlier with infliximab (p = 0.0072). Coronary artery lesions occurred in 1 (6.3%) and 3 (20.0%) patients receiving infliximab and VGIH, respectively, up to day 21. Adverse events occurred in 15 (93.8%) and 15 (100.0%) patients in the infliximab and VGIH groups, respectively. No serious adverse events in the infliximab group and one in the VGIH group were observed. Infliximab improved the defervescence rate within 48 h and time to defervescence versus standard therapy, and was well tolerated in patients with IVIG-refractory KD.

  6. Z-score of Mitral Annular Plane Systolic Excursion is a Useful Indicator of Evaluation of Left Ventricular Function in Patients with Acute-Phase Kawasaki Disease.

    Science.gov (United States)

    Hashimoto, Ikuo; Watanabe, Kazuhiro

    2017-06-01

    We previously reported the clinical usefulness of the mitral annular plane systolic excursion (MAPSE) to evaluate the left ventricular (LV) function in patients with Kawasaki disease (KD) in the acute-phase. However, the feasibility of the MAPSE z-score has not been evaluated in patients with acute KD. We prospectively studied 60 KD patients without coronary aneurysms. The MAPSE z-scores were calculated using our standard MAPSE data. Brain natriuretic peptide (BNP) was measured as a parameter of LV function. In total, 281 healthy age- and body size-matched subjects were chosen as the control group. The MAPSE z-score decreased in the acute-phase (median value, -1.4) and increased in the convalescent phase (median value, 0.18; P characteristic (ROC) analysis, the optimal cutoff value for the MAPSE z-score to judge LV dysfunction was -0.9. The MAPSE z-score is a useful index to evaluate LV function, and the cutoff value of -0.9 can be an indicator to judge LV dysfunction in the patients with acute-phase KD.

  7. Safety and efficacy of warfarin plus aspirin combination therapy for giant coronary artery aneurysm secondary to Kawasaki disease: a meta-analysis.

    Science.gov (United States)

    Su, Danyan; Wang, Kai; Qin, Suyuan; Pang, Yusheng

    2014-01-01

    To compare the safety and efficacy of warfarin plus aspirin versus aspirin alone for the treatment of children with giant coronary artery aneurysm (CAA) secondary to Kawasaki disease (KD). We searched the PubMed, EMBASE, Cochrane Library, CNKI, WANFAN and VIP databases. We selected case-controlled trials of warfarin plus aspirin versus aspirin alone for the treatment of children with giant CAA secondary to KD. Six retrospective studies met our inclusion criteria. There was no significant difference between the warfarin plus aspirin and aspirin alone groups in the rate of CAA regression (OR 1.38, 95% CI 0.52-3.68, p = 0.52) or the incidence of persistent CAA (OR 2.34, 95% CI 0.16-33.50, p = 0.53), coronary artery stenosis (OR 0.55, 95% CI 0.18-1.72, p = 0.30) or thrombus formation (OR 0.50, 95% CI 0.15-1.69, p = 0.26). There was evidence that warfarin plus aspirin reduced the incidence of coronary artery occlusion (OR 0.08, 95% CI 0.02-0.29, p aspirin therapy reduced the incidence of occlusion, cardiac infarction and death in children with giant CAA secondary to KD. © 2014 S. Karger AG, Basel.

  8. Review of Kawasaki Patients in Yazd

    Directory of Open Access Journals (Sweden)

    P Roozbeh

    2009-01-01

    Full Text Available Introduction: Kawasaki syndrome is an acute febrile vasculitis with multiorgan involvment in children. The objective of this study was assessment of demographic, clinical and laboratory data of Kawasaki patients in Yazd. Methods: In a descriptive-analytic study, the hospital records of patients treated for Kawasaki disease in all hospitals with pediatric wards between March 1996 and March 2006 at Yazd were reviewed and demographic, clinical and laboratory data recorded in a questionnaire and analyzed by SPSS, version 13 . Chi-square test was used to assess statistical differences. Results: 60.4% of patients were male and male /female ratio was 1.52. Seven percent of them were less than 5 years old. Duration of fever before treatment was in range of 1-20 days with mean of 9.1±5.07 days. Fever less than 5 days, 6-10 days, 11-15 days and more than 15 days was seen in 45.8%, 25 %, 22.9% and 6.2%, respectively. The most common symptoms were non purulent conjunctivitis (83.3%, strawberry tongue (79% ,dry-cracked lips(77%, skin rash(66.6% and cervical lymphadenopathy(47.9%. Leukocytosis, anemia and thrombocytosis were seen in 64%, 80% and 45%, respectively. Cardiac involvement was seen in 39.5% of the patients. Conclusion: The Kawasaki disease is predominantly seen in children less than 5 years old and most common symptoms include non purulent conjunctivitis ,strawberry tongue, dry-cracked lips, skin rash and cervical lymphadenopathy .

  9. Atypical presentation of late-onset Tay-Sachs disease.

    Science.gov (United States)

    Deik, Andres; Saunders-Pullman, Rachel

    2014-05-01

    Late-onset Tay-Sachs disease (LOTS) is a lysosomal storage disease caused by deficient Beta-hexosaminidase A activity. We describe a 53-year-old woman who presented with adult-onset leg weakness, and whose initial diagnosis was progressive muscular atrophy without identifiable etiology. Development of cerebellar ataxia in mid-life prompted reassessment. Beta-hexosaminidase A quantification assay demonstrated absence of the isozyme. Genetic testing identified compound heterozygous mutations in the HEXA gene, confirming the diagnosis of LOTS. The phenotypic spectrum of LOTS includes motor neuronopathy, ataxia, choreoathetosis, neuropathy, and psychiatric symptoms in various combinations. This patient highlights the emergence of different clinical features over many years and emphasizes the need to consider LOTS in the differential diagnosis of progressive muscular atrophy. Copyright © 2013 Wiley Periodicals, Inc.

  10. [An atypical presentation of Infantile Alexander disease lacking macrocephaly].

    Science.gov (United States)

    Esmer, Carmen; Villegas-Aguilera, Miguel; Morales-Ibarra, Juan José; Bravo-Oro, Antonio

    Alexander disease is a rare form of leukodystrophy that involves mainly astrocytes; it is inherited in an autosomal recessive manner and occurs by mutations in the GFAP gene, located on chromosome 17q21. It can occur at any age and its infantile form is characterized by macrocephaly, seizures, severe motor and cognitive delay, and progressive spasticity or ataxia. An 8-month-old female was evaluated with a history of neurodevelopmental delay and unprovoked focal motor seizures. Physical examination showed normal head circumference, increased motor responses to tactile and noise stimuli, pyramidal signs and no visceromegalies. Widespread hypodense white matter was found on magnetic resonance and lumbar puncture showed hyperproteinorrachia. Krabbe disease was ruled out by enzymatic assay and gene sequencing of GALC. In the reassessment of the case, abnormalities in neuroimaging lead to suspicion of Alexander disease, and GFAP gene sequencing reported a pathogenic mutation in exon 4 c.716G>A, which caused a change of arginine to histidine at position 239 of the protein (p.Arg239His). The radiographic signs observed in the resonance were decisive for the diagnosis, later confirmed by molecular study. It is important to consider that certain mutations are not associated with macrocephaly, which may cause delay in diagnosis. Copyright © 2016 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  11. Spontaneous Remission of Bronchial Asthma as Onset of Atypical Cushing’s Disease

    Directory of Open Access Journals (Sweden)

    Ye.M. Маrtsynik

    2016-06-01

    Full Text Available The article presents a case of delayed diagnosis of Сushing’s disease in a female patient as a result of the incomplete and atypical clinical picture of hypercorticoidism. In everyday practice, professionals should be concerned about the timely diagnosis of Сushing’s disease in patients with syntropic pathology (dysplastic obesity, hypertension, carbohydrate metabolism disorder. It is proved that an unrecognized hypercortisolism significantly increases cardiovascular risks. Timely and correct diagnosis of the disease, before development of severe complications, will greatly improve the prognosis of patients.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. An atypical presentation and diagnostic dilemma of sickle cell disease

    Directory of Open Access Journals (Sweden)

    Mohammad Mizanur Rahman

    2017-06-01

    Full Text Available A 5½ year old male child of a consanguineous couple presented with moderately high fever, hepatosplenomegaly and severe pain in the left upper quadrant of the abdomen for four days. On examination, the child was found severely anemic, ill-looking and mildly icteric but the abdomen was soft and non-tender. Radiological investigations and ultrasonography of the whole abdomen revealed the diagnosis of space occupying lesions which may be either due to splenic abscess/cyst or leukemia/lymphoma. However, blood film revealed the features of hereditary hemolytic anemia and later on hemoglobin electrophoresis initially commented as HbS–beta thalassemia but subsequent family screening of the patient turned out to a case of homozygous sickle cell anemia or sickle cell disease. To resolve such diagnostic dilemma, it is very much essential to analyze critically the history including family history, clinical and physical findings as well as investigational findings.

  14. Experience in the diagnosis and treatment of Kawasaki disease in the Saint-Petersburg State Pediatric Medical University Clinic and Saint Petersburg City Children’s Hospital One

    Directory of Open Access Journals (Sweden)

    A. V. Santimov

    2017-01-01

    Full Text Available Kawasaki disease (KD is acute systemic vasculitis of unknown etiology. Approximately 20–25% of untreated patients develop coronary artery changes with a range of severity from asymptomatic coronary artery dilatation to giant coronary artery aneurysms with thrombosis, myocardial infarction, and sudden death. To date there is no official data on the incidence of KD in Russia. In Russia, the disease is not enough known now to a wide circle of physicians and often masks other more common diseases. Since 2010, the detection rate of KD has dramatically increased in Saint-Petersburg.Objective: to analyze the experience in diagnosing and treating KD in two largest hospitals of Saint Petersburg.Patients and methods. The retrospective study included data on 30 children (18 boys, 12 girls who were hospitalized with a diagnosis of KD in the Saint-Petersburg State Pediatric Medical University Clinic and Children’s Hospital One (Saint Petersburg between January 2011 and September 2016. Data are represented by median and extreme values. The age of the children was 2.8 (0.2; 4.6 years; of them 5 (16.7% patients were under the age of 1 year. The children were hospitalized on 5 (1; 14 days of disease onset; KD was diagnosed on 9 (3; 52 day of the disease.Results. Immediately after diagnosis, 27 (90% children received aspirin. In early stages (before 10 days of the disease, intravenous immunoglobulin (IVIG therapy was performed in 15 (50% children, one of them received IVIG before disease day 5 (on day 3, but without effect. On disease days 11-20 (immediately after diagnosis, 10 (33.3% children were prescribed with IVIG; thereafter fever was abolished in all the patients. Their body temperature became normal on day 11 (6; 23. Ultrasonography revealed coronary artery lesions in 13 (43.3% patients. Out of the 30 children followed up, one baby who fell ill at the age of 3 months and received IVIG died on day 30 of the disease.Conclusion. Currently

  15. Arterial stiffness in patients after Kawasaki disease without coronary artery involvement: Assessment by performing brachial ankle pulse wave velocity and cardio-ankle vascular index.

    Science.gov (United States)

    Nakagawa, Ryo; Kuwata, Seiko; Kurishima, Clara; Saiki, Hirofumi; Iwamoto, Yoichi; Sugimoto, Masaya; Ishido, Hirotaka; Masutani, Satoshi; Senzaki, Hideaki

    2015-08-01

    It remains unclear whether systemic arterial beds other than the coronary arteries are truly healthy in patients without coronary artery lesions (CAL) after Kawasaki disease (KD). We tested the hypothesis that patients with KD without echocardiographic evidence of CAL during the acute phase of the disease have abnormal mechanical properties in systemic arteries later. We studied 201 consecutive patients with KD (age 2-23 years, mean 10±4 years; 109 male, 92 female) without CAL during the acute phase. Data were compared with those in 129 control subjects (age 2-25 years, mean 10±4 years; 73 male, 56 female; control group). We examined arterial stiffness by using the brachial-ankle pulse wave velocity (baPWV) and the cardio-ankle vascular index (CAVI). The baPWV in the KD group was significantly higher than that in the control group (913±121cm/s vs. 886±135cm/s, p=0.04). In contrast, there was no significant difference in CAVI (4.0±1.0 vs. 4.2±1.0, p=0.9) between the two groups. Multivariate analysis indicated a highly significant difference in baPWV (higher baPWV in patients with KD than in controls, p=0.004), after controlling for age, gender, body height and weight, and systolic and diastolic blood pressure, but no difference in CAVI between the groups. Years after KD occurs in patients without apparent CAL during the acute phase, there is a small but significant change in systemic arterial properties, characterized by increased wall stiffness. The clinical importance of these findings must be clarified by performing long-term follow-up studies. Copyright © 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  16. Epidemiology of typical coronay heart disease versus heart disease of uncertain etiology (atypical) fatalities and their relationships with classic coronary risk factors

    NARCIS (Netherlands)

    Menotti, A.; Puddu, P.E.; Lanti, M.; Kromhout, D.; Tolonen, H.; Parapid, B.

    2013-01-01

    Objectives: The relationships were explored of some cardiovascular risk factors to typical (TYP) and atypical (ATYP) fatal coronary events (CHD). Material and methods: Thirteen cohorts of 40-59 year-old men of the Seven Countries Study were followed-up for 40 years (N = 9704 heart disease free

  17. Atypical clinical and radiological course of a patient with Canavan disease.

    Science.gov (United States)

    Sarret, Catherine; Boespflug-Tanguy, Odile; Rodriguez, Diana

    2016-04-01

    Canavan disease (CD) is a rare metabolic disorder caused by aspartoacylase (ASPA) deficiency. It leads to severe neurological degeneration with spongiform brain degeneration. Accumulation of N-acetylaspartate (NAA) in brain and urine is specific to the disease and guides diagnosis. Magnetic resonance imaging (MRI) usually shows diffuse white matter abnormalities with involvement of the basal ganglia. Mild forms of the disease with a more favorable clinical course and radiological involvement of the basal ganglia without white matter abnormalities have also been reported. Here we report an atypical case of a girl aged nine years with CD. The disease started at the classical age of five months. Classical elevation of NAA in brain and urine was present and genetic analysis identified mutations in the ASPA gene. However, clinical evolution was milder than typical CD, with partial motor impairment and relatively well-preserved cognitive skills. MRI was also atypical with low white matter involvement and unusual topography and evolution of abnormalities in the basal ganglia.

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

    DEFF Research Database (Denmark)

    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/pervasive...... developmental disorder not otherwise specified with 258 controls from the general population. All participants were screened through the nationwide Danish National Hospital Register. The average observation time was 32.9 years, and mean age at the end of the observation period was 48.5 years. Among the 89 cases...

  19. De ziekte van Kawasaki, nog steeds een mysterieuze ziekte

    NARCIS (Netherlands)

    Winterberg, D. h

    2007-01-01

    Kawasaki disease is an acute vasculitis that occurs especially in young children. Because there is no specific laboratory test available, diagnosis has to be made on the basis of clinical characteristics: prolonged fever, oropharyngeal changes, conjunctival injection, erythema and edema of hands and

  20. Clinical and Etiological Characteristics of Atypical Hand-Foot-and-Mouth Disease in Children from Chongqing, China: A Retrospective Study

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    Xiang Yan

    2015-01-01

    Full Text Available Background. Hand-foot-and-mouth disease (HFMD is a disease that had similar manifestations to chickenpox, impetigo, and measles, which is easy to misdiagnose and subsequently causes delayed therapy and subsequent epidemic. To date, no study has been conducted to report the clinical and epidemiological characteristics of atypical HFMD. Methods. 64 children with atypical HFMD out of 887 HFMD children were recruited, stool was collected, and viral VP1 was detected. Results. The atypical HFMD accounted for 7.2% of total HFMD in the same period (64/887 and there were two peaks in its prevalence in nonepidemic seasons. Ten children (15.6% had manifestations of neurologic involvement, of whom 4 (6.3% were diagnosed with severe HFMD and 1 with critically severe HFMD, but all recovered smoothly. Onychomadesis and desquamation were found in 14 patients (21.9% and 15 patients (23.4%, respectively. The most common pathogen was coxsackievirus A6 (CV-A6 which accounted for 67.2%, followed by nontypable enterovirus (26.6%, enterovirus 71 (EV-A71 (4.7%, and coxsackievirus A16 (A16 (1.5%. Conclusions. Atypical HFMD has seasonal prevalence. The manifestations of neurologic involvement in atypical HFMD are mild and usually have a good prognosis. CV-A6 is a major pathogen causing atypical HFMD, but not a major pathogen in Chongqing, China.

  1. Clinical and Etiological Characteristics of Atypical Hand-Foot-and-Mouth Disease in Children from Chongqing, China: A Retrospective Study.

    Science.gov (United States)

    Yan, Xiang; Zhang, Zhen-Zhen; Yang, Zhen-Hua; Zhu, Chao-Min; Hu, Yun-Ge; Liu, Quan-Bo

    2015-01-01

    Hand-foot-and-mouth disease (HFMD) is a disease that had similar manifestations to chickenpox, impetigo, and measles, which is easy to misdiagnose and subsequently causes delayed therapy and subsequent epidemic. To date, no study has been conducted to report the clinical and epidemiological characteristics of atypical HFMD. 64 children with atypical HFMD out of 887 HFMD children were recruited, stool was collected, and viral VP1 was detected. The atypical HFMD accounted for 7.2% of total HFMD in the same period (64/887) and there were two peaks in its prevalence in nonepidemic seasons. Ten children (15.6%) had manifestations of neurologic involvement, of whom 4 (6.3%) were diagnosed with severe HFMD and 1 with critically severe HFMD, but all recovered smoothly. Onychomadesis and desquamation were found in 14 patients (21.9%) and 15 patients (23.4%), respectively. The most common pathogen was coxsackievirus A6 (CV-A6) which accounted for 67.2%, followed by nontypable enterovirus (26.6%), enterovirus 71 (EV-A71) (4.7%), and coxsackievirus A16 (A16) (1.5%). Atypical HFMD has seasonal prevalence. The manifestations of neurologic involvement in atypical HFMD are mild and usually have a good prognosis. CV-A6 is a major pathogen causing atypical HFMD, but not a major pathogen in Chongqing, China.

  2. Atypical nature of coronary artery disease in women: a proposal for measurement and classification

    Directory of Open Access Journals (Sweden)

    Viviana Marycel Céspedes Cuevas

    2015-01-01

    Full Text Available Objective: To identify subgroups of women with Acute Coronary Syndrome according to symptom experience: perception, evaluation and response, based on the Symptom Management Conceptual Model. Methodology: Quantitative, descriptive, exploratory, cross-sectional study. The sample was made of 380 women positively diagnosed with Acute Coronary Syndrome, hospitalized in two institutions in Bogotá, Colombia. A measurement instrument was designed and validated. Results: Final instrument was made of 37 items that reported content validity, scale validity, discriminant validity, and construct validity. A Cronbach's Alpha coefficient of 0,76 was obtained, which guarantees homogeneity in the measurement according to the Maximum Validity-Maximum Reliability Model. A total of 11 subgroups of women with Acute Coronary Syndrome were identified, those were characterized by atypical coronary heart disease symptomatology, evaluation processes related to extra-cardiac causes and inadequate symptom management strategies. It was possible to demonstrate that psychosocial factors, previous coronary heart disease and delays were variables making a significant influence on the components of symptom experience. Conclusions: Women with Acute Coronary Syndrome, belonging to the 11 subgroups that were identified and studied, showed atypical symptoms. The instrument designed features a proven psychometric quality; it was valid, reliable, and useful for clinical research and practice.

  3. Atypical celiac disease as cause of increased need for thyroxine: a systematic study.

    Science.gov (United States)

    Virili, Camilla; Bassotti, Giulia; Santaguida, Maria Giulia; Iuorio, Raffaella; Del Duca, Susanna Carlotta; Mercuri, Valeria; Picarelli, Antonio; Gargiulo, Patrizia; Gargano, Lucilla; Centanni, Marco

    2012-03-01

    Replacement T4 dose in hypothyroid patients bearing both chronic autoimmune thyroiditis and atypical celiac disease (CD) has been analyzed. Replacement T4 dose has been analyzed in 35 hypothyroid patients with Hashimoto's thyroiditis (HT) and atypical CD, as defined by the American Gastroenterological Association. We have evaluated the ability of the same dose of T4 to reach target TSH in 21 patients before and during gluten-free diet (GFD). In the remaining 14 patients, noncompliant with GFD, we analyzed replacement T4 dose and compared it with that in a similar group consisting of 68 patients with hypothyroid HT but no evidence of celiac sprue or other conditions interfering with T4 absorption. In patients with isolated HT, the desired serum TSH (median=1.02 mU/liter) was reached in all patients after 5±2 months of treatment at a median T4 dose of 1.31 μg/kg·d. After a similar period and dose of T4, higher levels of TSH (median=4.20 mU/liter) were observed in patients with HT and CD. In 21 CD patients, target TSH (median TSH=1.25 mU/liter) has been attained after 11±3 months of GFD without increasing T4 dose (1.32 μg/kg·d). In the remaining 14 patients, who were noncompliant with GFD, target TSH has also been achieved but at a higher T4 dose (median=1.96 μg/kg·d; +49%; P=0.0002) than in hypothyroid patients without CD. Atypical CD increases the need for T4. The effect was reversed by GFD or by increasing T4 dose. Malabsorption of T4 may provide the opportunity to detect CD that was overlooked until the patients were put under T4 therapy.

  4. Atypical Huntington's disease with the clinical presentation of behavioural variant of frontotemporal dementia.

    Science.gov (United States)

    Sutovsky, Stanislav; Smolek, Tomas; Alafuzoff, Irina; Blaho, Andrej; Parrak, Vojtech; Turcani, Peter; Palkovic, Michal; Petrovic, Robert; Novak, Michal; Zilka, Norbert

    2016-12-01

    Huntington's disease is an incurable, adult-onset, autosomal dominant inherited disorder caused by an expanded trinucleotide repeat (CAG). In this study, we describe a Huntington's disease patient displaying clinical symptoms of the behavioural variant of frontotemporal dementia in the absence of tremor and ataxia. The clinical onset was at the age of 36 years and the disease progressed slowly (18 years). Genetic testing revealed expanded trinucleotide CAG repeats in the Huntingtin gene, together with a Glu318Gly polymorphism in presenilin 1. Neuropathological assessment revealed extensive amyloid β (Aβ) aggregates in all cortical regions. No inclusions displaying hyperphosphorylated tau or phosphorylated transactive response DNA-binding protein 43 (TDP43) were found. A high number of p62 (sequestosome 1) immunopositive intranuclear inclusions were seen mainly in the cortex, while subcortical areas were affected to a lesser extent. Confocal microscopy revealed that the majority of p62 intranuclear lesions co-localised with the fused-in-sarcoma protein (FUS) immunostaining. The morphology of the inclusions resembled intranuclear aggregates in Huntington's disease. The presented proband suffered from Huntington's disease showed atypical distribution of FUS positive intranuclear aggregates in the cortical areas with concomitant Alzheimer's disease pathology.

  5. Atypical Depression

    Science.gov (United States)

    Atypical depression Overview Any type of depression can make you feel sad and keep you from enjoying life. However, atypical depression — also called depression with atypical features — means that ...

  6. Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury

    Directory of Open Access Journals (Sweden)

    Banan AlSowailmi

    2017-01-01

    Full Text Available Antiglomerular basement membrane (anti-GBM disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. All serology tests were negative, except for anti-GBM which was weakly positive. Renal biopsy showed proliferative glomerulonephritis (GN with nodular transformation of the glomerular tufts, mesangial hypercellularity (mesangial cell proliferation, segmental endocapillary hypercellularity and three incomplete cellular crescents, and recapitulating membranoproliferative GN pattern of glomerular injury. Direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG and Kappa and Lambda light chains, and compatible with anti-GBM disease. The patient was treated with cyclophosphamide and corticosteroids in addition to therapeutic plasma exchange which resulted in mild improvement in renal function over a period of six weeks. We emphasize the importance of recognition of atypical pathological and serological patterns of anti-GBM disease, which is crucial for proper and early diagnosis and possibly improved clinical outcome and we highlight the importance of clinicopathological correlation in cases with atypical clinical and pathological presentations.

  7. Atypical Vascular Involvement in a Case of Behçet's Disease

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    Alejandro Rodríguez Morata

    2012-01-01

    Full Text Available Introduction. Behçet's disease (BD is a form of vasculitis of unknown etiology which is rare in our environment. It is characterized by a variety of clinical manifestations and usually affects young adults. Recurrent oral and genital ulcers are a characteristic and extremely frequent symptom, but mortality is linked with more significant symptoms such as aortic pseudoaneurysm, pulmonary pseudoaneurysm, and cerebral venous thrombosis. Patient and Method. We present a case of a young male with atypical BD and severe polyvascular involvement (previous cerebral venous thrombosis and current peripheral venous thrombosis, acute ischemia, and peripheral arterial pseudoaneurysm who required urgent surgical intervention due to a symptomatic external iliac pseudoaneurysm. Result. The pseudoaneurysm was successfully treated, we performed an iliofemoral bypass, and we treated it with steroids and immunosuppressive therapy. Conclusions. These rare clinical manifestations highlight the importance of considering BD in young patients, even in usual cases of vascular intervention, whether arterial or venous in nature.

  8. An atypical course of coxsackievirus A6 associated hand, foot and mouth disease in extremely low birth weight preterm twins

    NARCIS (Netherlands)

    Bruning, Andrea H. L.; van der Sanden, Sabine M. G.; ten Hoedt, Amber E.; Wolthers, Katja C.; van Kaam, Anton H.; Pajkrt, Dasja

    2015-01-01

    The incidence of coxsackievirus A6 (CV-A6) associated hand, foot and mouth disease (HFMD) has reportedly increased since 2008 with sometimes severe complications. We here describe an atypical course of CV-A6-associated HFMD in extremely low birth weight twins. The CV-A6-strains are genetically

  9. An atypical case of Canavan disease with stroke-like presentation.

    Science.gov (United States)

    Delaney, Katherine E; Kralik, Stephen F; Hainline, Bryan E; Golomb, Meredith R

    2015-02-01

    Canavan disease is an autosomal recessive leukodystrophy caused by a deficiency of aspartoacylase. The disease has a severe course, with death occurring in the first few years of life. Atypical patients with mild courses have been reported, but acute presentations similar to stroke have not been well described. We present a boy who presented at 4 months of age with seizures after an episode of cardiopulmonary arrest is discussed. He was initially thought to have an ischemic watershed stroke based on his initial clinical presentation and magnetic resonance imaging. However, biochemical and follow-up radiologic evaluation were consistent with mild Canavan disease. DNA sequencing of the ASPA gene indicated one known mutation (A305E) and a novel mutation, L30V. Follow-up magnetic resonance imaging did not reveal the atrophy which would have been expected with watershed ischemia. Magnetic resonance spectroscopy demonstrated elevated N-acetyl aspartate to creatinine and N-acetyl aspartate to choline ratios. At 4 years of age, he was normocephalic, with mild clumsiness, speech delay, and seizures. This child's unusual acute presentation, along with his prolonged mild course, raises questions about the relationship between biochemical signs of abnormal aspartoacylase function and clinical findings. This patient highlights the need for long-term clinical follow-up of children with mild Canavan disease to clarify the significance of these biochemical abnormalities. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. 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.

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

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  13. [Headache as atypical presentation of celiac disease: report of a clinical case].

    Science.gov (United States)

    Spina, M; Incorpora, G; Trigilia, T; Branciforte, F; Franco, G; Di Gregorio, F

    2001-01-01

    Coeliac disease (CD) is a gluten dependent enteropathy with genetic predisposition. The introduction of the gluten with the diet leads to a damage of the intestinal mucosa losing the ability of absorption. Together with the "classic forms", in wich the intestinal symptomatology is prevalent, there are atypical forms, with unusual clinical presentation and silent forms with no clinical symptoms. The neurologic symptoms are not frequent and regard seizures, headache, ataxia and psychiatric problems. We report on a patient with headache since 3 years of age in which the headache the only manifestation of CD. The diagnosis of CD was made at 11 years, when he came at our observation for episodes of headache. Also the older sister is found affected by CD. After three months of gluten free diet, it was obtained the complete resolution of the headache. Also if the pathogenesis of the headache in patient with CD is unknown we think that a autoimmune, vascular or blood flow mechanism could be ipotizeable.

  14. 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...

  15. 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.

  16. Atypical pneumonia

    Science.gov (United States)

    Walking pneumonia; Community-acquired pneumonia - atypical ... Bacteria that cause atypical pneumonia include: Mycoplasma pneumonia is caused by the bacteria Mycoplasma pneumoniae . It often affects people younger than age 40. Pneumonia due ...

  17. Atypical Depression

    Directory of Open Access Journals (Sweden)

    Erhan Ertekin

    2013-09-01

    Full Text Available Atypical depression is defined as a specifier of major depressive disorder. Columbia criteria for atypical depression are commonly used to make a diagnosis. Female sex, onset at early age, chronic course, and higher rate of comorbidity (especially anxiety disorder and bipolar disorder is noteworthy in atypical depression. Although, the atypical depression seems to support the familial genetic transition, there is not any specific study supporting these data. In the treatment of atypical depression, monoamine oxidase inhibitors are reported to be more effective than tricyclic antidepressants. In recent studies, selective serotonin reuptake inhibitors have also proven to be efficient.

  18. [A case of atypical moyamoya disease with a ruptured aneursym on moyamoya vessel].

    Science.gov (United States)

    Okamoto, J; Mukai, K; Kashihara, M; Ueda, D; Matumoto, K

    1982-08-01

    A 45-year-old female had sudden onset of severe headache, nausea and vomiting without any inducing moments and was transferred to our hospital by an ambulance car. On admission the patient was alert and showed only signs of meningeal irritation. There were no other neurological deficits at all. Immediate CT examination revealed a small high density spot in the area of the right basal ganglia, and a large high density in the frontal horns of both lateral ventricles. Right carotid angiogram showed completely patent carotid siphon with Moyamoya vessels. A small round aneurysmal shadow of 3 mm in diameter was seen in the area of these Moyamoya vessels of the right basal ganglia, and it was thought to be corresponding to the high density spot in the CT examination. Left carotid angiogram showed stenosis of C portion with typical Moyamoya vessels. Therefore, diagnosis of atypical Moyamoya disease with a ruptured aneurysm and ventricular penetration of hematoma was made. The patient was placed on conservative treatment. On 25th day from onset, repeated angiogram showed enlargement of the aneurysmal shadow from 3 mm to 5 mm in diameter. So, on 33rd day, right STA-MCA anastomosis and encephalomyosynangiosis were carried out. Angiogram on 12th day after surgery revealed no aneurysmal shadow. The patient was discharged without any neurological deficit at all. Previous paper, which reported cases of Moyamoya disease with aneurysm, were reviewed. According to the location of the aneurysm, these cases were classified into 3 types angiographically; type I: aneurysm in the area of Moyamoya vessels, type II: aneurysm of cerebral-peripheral artery, and type III: aneurysm of the circle of Willis. It has been generally believed that the Moyamoya disease may be associated with either a true or a pseudoaneurysm in the case of type I. However, pseudoaneurysm in the area of Moyamoya vessels has not been found in postmortem examination but true aneurysms so far. Therefore it may not be

  19. Atypical hand, foot and mouth disease in adults associated with coxsackievirus A6: a clinico-pathologic study.

    Science.gov (United States)

    Laga, Alvaro C; Shroba, Suzanne M; Hanna, John

    2016-11-01

    Hand, foot, and mouth disease (HFMD) is a contagious illness most commonly occurring in children 5 years old or younger. The most common cause of HFMD in the United States is Coxsackievirus A16. HFMD is uncommon in adults, and may show other atypical features including a broader spectrum of cutaneous involvement and a greater degree of severity. We evaluated the clinical, histopathologic and molecular features of three cases of atypical HFMD occurring in adults. All three cases showed clinical features that were worrisome for erythema multiforme or a disseminated herpesvirus infection. The histopathologic findings were quite uniform, and showed intraepidermal vesiculation with a predominantly neutrophil-rich infiltrate. A characteristic feature was the specific involvement of the upper stratum spinosum and stratum granulosum, with relative sparing of the stratum corneum. In none of the cases was there evidence of herpesvirus. Molecular analysis performed on two of the cases showed involvement by Coxsackievirus A6, an uncommon serotype in HFMD. All three cases resolved spontaneously. Atypical HFMD associated with Coxsackievirus A6 represents an uncommon and potentially diagnostically challenging cutaneous eruption. Its recognition is critical to avoid unneeded therapy and to establish accurate prognostic expectations. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Seasonality and temporal clustering of Kawasaki syndrome.

    Science.gov (United States)

    Burns, Jane C; Cayan, Daniel R; Tong, Garrick; Bainto, Emelia V; Turner, Christena L; Shike, Hiroko; Kawasaki, Tomisaku; Nakamura, Yosikazu; Yashiro, Mayumi; Yanagawa, Hiroshi

    2005-03-01

    The distribution of a syndrome in space and time may suggest clues to its etiology. The cause of Kawasaki syndrome, a systemic vasculitis of infants and children, is unknown, but an infectious etiology is suspected. Seasonality and clustering of Kawasaki syndrome cases were studied in Japanese children with Kawasaki syndrome reported in nationwide surveys in Japan. Excluding the years that contained the 3 major nationwide epidemics, 84,829 cases during a 14-year period (1987-2000) were analyzed. To assess seasonality, we calculated mean monthly incidence during the study period for eastern and western Japan and for each of the 47 prefectures. To assess clustering, we compared the number of cases per day (daily incidence) with a simulated distribution (Monte Carlo analysis). Marked spatial and temporal patterns were noted in both the seasonality and deviations from the average number of Kawasaki syndrome cases in Japan. Seasonality was bimodal with peaks in January and June/July and a nadir in October. This pattern was consistent throughout Japan and during the entire 14-year period. Some years produced very high or low numbers of cases, but the overall variability was consistent throughout the entire country. Temporal clustering of Kawasaki syndrome cases was detected with nationwide outbreaks. Kawasaki syndrome has a pronounced seasonality in Japan that is consistent throughout the length of the Japanese archipelago. Temporal clustering of cases combined with marked seasonality suggests an environmental trigger for this clinical syndrome.

  2. Seasonality and Temporal Clustering of Kawasaki Syndrome

    Science.gov (United States)

    Burns, Jane C.; Cayan, Daniel R.; Tong, Garrick; Bainto, Emelia V.; Turner, Christena L.; Shike, Hiroko; Kawasaki, Tomisaku; Nakamura, Yosikazu; Yashiro, Mayumi; Yanagawa, Hiroshi

    2010-01-01

    Background The distribution of a syndrome in space and time may suggest clues to its etiology. The cause of Kawasaki syndrome, a systemic vasculitis of infants and children, is unknown, but an infectious etiology is suspected. Methods Seasonality and clustering of Kawasaki syndrome cases were studied in Japanese children with Kawasaki syndrome reported in nationwide surveys in Japan. Excluding the years that contained the 3 major nationwide epidemics, 84,829 cases during a 14-year period (1987–2000) were analyzed. To assess seasonality, we calculated mean monthly incidence during the study period for eastern and western Japan and for each of the 47 prefectures. To assess clustering, we compared the number of cases per day (daily incidence) with a simulated distribution (Monte Carlo analysis). Results Marked spatial and temporal patterns were noted in both the seasonality and deviations from the average number of Kawasaki syndrome cases in Japan. Seasonality was bimodal with peaks in January and June/July and a nadir in October. This pattern was consistent throughout Japan and during the entire 14-year period. Some years produced very high or low numbers of cases, but the overall variability was consistent throughout the entire country. Temporal clustering of Kawasaki syndrome cases was detected with nationwide outbreaks. Conclusions Kawasaki syndrome has a pronounced seasonality in Japan that is consistent throughout the length of the Japanese archipelago. Temporal clustering of cases combined with marked seasonality suggests an environmental trigger for this clinical syndrome. PMID:15703537

  3. Phenomenology and epidemiology of impulsive-compulsive behaviours in Parkinson's disease, atypical Parkinsonian disorders and non-Parkinsonian populations.

    Science.gov (United States)

    Maloney, Eimer M; Djamshidian, Atbin; O'Sullivan, Sean S

    2017-03-15

    Impulsive-compulsive behaviours are common, quality of life affecting consequences of dopamine replacement therapy which are well recognized in patients with idiopathic Parkinson's disease. Details of the occurrence and nature of these disorders in the atypical parkinsonian neurodegenerative disorders, and in non-Parkinson's patients prescribed dopaminergic stimulation for other disease processes, are slowly emerging. Here we review what is known about the phenomenology, epidemiology and risk factors for impulsive-compulsive behaviours in Parkinson's disease and in other, less well studied, patient groups. By analyzing the available published data, this review identifies potential clues as to the underlying neurobiological mechanism of these disorders, and further identifies critical gaps yet to be addressed. Copyright © 2016 Elsevier B.V. All rights reserved.

  4. Atypical placental site nodule (APSN) and association with malignant gestational trophoblastic disease; a clinicopathologic study of 21 cases.

    Science.gov (United States)

    Kaur, Baljeet; Short, Dee; Fisher, Rosemary A; Savage, Philip M; Seckl, Michael J; Sebire, Neil J

    2015-03-01

    The WHO Classification of Gestational Trophoblastic Tumors classifies placental site nodule (PSN) as a benign tumor-like trophoblastic neoplasm. Cases of PSN with atypical features were described [atypical placental site nodule (APSN)] and we started registering APSN in our unit in 2005. The aim of this study is to present our initial experience with these lesions. The Trophoblastic Disease Unit database was searched to identify all patients who were either referred with, or on review were diagnosed with, APSN from September 2005 to May 2013. Case notes and the pathology findings for these patients were retrieved and reviewed. A total of 21 cases of APSN were included, 3 of which were associated with gestational trophoblastic neoplasm on follow-up or review. Malignant gestational trophoblastic disease was associated with 3/21 (14%) cases of APSN, either concurrently or developing/manifesting within 16 mo of APSN diagnosis. None of these patients had raised serum hCG levels either at presentation or follow-up. Presence of APSN should indicate a thorough clinical and radiologic investigation and follow-up if diagnosed on curettage specimens. With increased recognition of this entity and corresponding larger series with longer follow-up, more accurate patient counseling will be possible.

  5. The Role of Calprotectin in Pediatric Disease

    National Research Council Canada - National Science Library

    Vaos, George; Kostakis, Ioannis D; Zavras, Nick; Chatzemichael, Athanasios

    2013-01-01

    ... disease, necrotizing enterocolitis, celiac disease, intestinal cystic fibrosis, acute appendicitis, juvenile idiopathic arthritis, Kawasaki disease, polymyositis-dermatomyositis, glomerulonephritis, IgA...

  6. Atypical Antidepressants

    Science.gov (United States)

    ... which is also used to treat insomnia Vortioxetine (Trintellix) Side effects may occur with antidepressants, including atypical ... traz_imtb_ins.pdf. Accessed May 23, 2016. Trintellix (prescribing information). Deerfield, Ill.: Takeda Pharmaceuticals; 2016. http:// ...

  7. Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor.

    Science.gov (United States)

    Yang, Xiang; Liu, Xue-Song; Fang, Yuan; Zhang, Xiu-Hui; Zhang, Yue-Kang

    2014-01-01

    The authors described a case of a patient with co-existing endolymphatic sac tumor (ELST) and hemangioblastoma in the posterior cranial fossa, which belonged to a subtype of Von Hippel-Lindau (VHL) disease confirmed by the test of VHL-gene. The signs in this 42-year-old female included intermittent headache and dizziness. Imaging revealed a giant mass in the right cerebellopontine angle (CPA) region and another lesion in the left cerebellar hemisphere. The results of biopsy after two operations confirmed the diagnosis respectively. Both of the tumors were resected totally. Nevertheless, we had to confess the misdiagnosis as vascular tumor instead of ELST at the initial diagnosis because of the rarity of ELST associated with atypical histological characteristics. The purposes we reported this case were to describe the atypical pathological feature of ELST and the mutation of germline VHL not mentioned in previously literature, furthermore, to foster understanding of ELSTs with the avoidance of the similar misdiagnosis as far as possible in future.

  8. Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease.

    Science.gov (United States)

    Berghoff, Anna S; Trummert, Anita; Unterberger, Ursula; Ströbel, Thomas; Hortobágyi, Tibor; Kovacs, Gabor G

    2015-08-01

    We describe an atypical neuropathological phenotype of sporadic Creutzfeldt-Jakob disease in a 76-year-old man. The clinical symptoms were characterized by progressive dementia, gait ataxia, rigidity and urinary incontinence. The disease duration was 6 weeks. MRI did not show prominent atrophy or hyperintensities in cortical areas, striatum or thalamus. Biomarker examination of the cerebrospinal fluid deviated from that seen in pure Alzheimer's disease. Triphasic waves in the EEG were detected only later in the disease course, while 14-3-3 assay was positive. PRNP genotyping revealed methionine homozygosity (MM) at codon 129. Neuropathology showed classical CJD changes corresponding to the MM type 1 cases. However, a striking feature was the presence of abundant kuru-type plaques in the white matter. This rare morphology was associated with neuropathological signs of intranuclear inclusion body disease and advanced stage of argyrophilic grain disease. These alterations did not show correlation with each other, thus seemed to develop independently. This case further highlights the complexity of neuropathological alterations in the ageing brain. © 2015 Japanese Society of Neuropathology.

  9. Evaluation of the association between atypical bacteria infections and respiratory tract diseases with emphasis on bronchial asthma exacerbations in children.

    Science.gov (United States)

    Szczepanik, Agnieszka; Kozioł-Montewka, Maria; Tuszkiewicz-Misztal, Ewa; Niedzielska, Grazyna; Górnicka, Grazyna; Niedźwiadek, Justyna; Niedzielski, Artur

    2004-01-01

    Mycoplasma pneumoniae and Chlamydia pneumonie are important etiological agents responsible for human respiratory tract diseases. Recently, these atypical microorganisms received much attention regarding their role in bronchial asthma pathogenesis, which is one of the most frequent chronic diseases in children. The aim of the study was to investigate the association between infections caused by these pathogens and respiratory tract diseases in children. Levels of M. pneumoniae and C. pneumoniae-specific antibodies were determined in serum samples obtained from 30 patients suffering from bronchial asthma exacerbations, 10 patients with pneumonia, 28 patients with chronic otitis media with effusion (COME) and 22 sinusitis patients. Specific anti-M. pneumoniae antibodies were detected more frequently in the patients enrolled in the study than in control subjects. The highest percentage of the serum samples, which demonstrated the presence of M. pneumoniae-specific antibodies was demonstrated in patients with asthma (60%) and it was twofold higher than in control subjects. Serologic profile of 26.6% patients with asthma, 50% of patients with pneumonia, 39.2% of patients with COME, 45.4% of patients with sinusitis and 10% of control subjects was consistent with a possible acute infection caused by M. pneumoniae. The presence of specific anti-C. pneumoniae antibodies was demonstrated in a smaller percentage of patients--in 13.3% of children with asthma, 10% of children with pneumonia and in 7.1% of patients with COME; the level of specific antiobodies was suggestive of acute chlamydial infection only in COME patients. Analysis of serologic markers for atypical bacteria infections indicates a possible association between infections caused by M. pneumoniae and bronchial asthma exacerbations and other respiratory tract disorders including pneumonia, sinusitis and COME.

  10. [Primary sclerosing cholangitis: case report of a young patient with an atypical diagnostic process in complications of the disease].

    Science.gov (United States)

    Hrstić, Irena; Ostojić, Rajko; Rustemović, Nadan; Opacić, Milorad; Cuković-Cavka, Silvija; Barbarić-Babić, Vinka

    2003-01-01

    The primary sclerotic colangitis is the chronic disease of the liver which in its finish very often requires the liver transplantation. The disease gets diagnosed mainly in males of middle age and is often joined with the inflammation disease of the intestines. The course of the disease not rarely is complicated by the stenosis of the liver ducts or by the occurrence of the concrements. In the presented case it is the word about the young patient in which the is the diagnosis made with the atypical way primarily from the reason that the biopsy of the liver is performed too early and the patohystologic changes were non specific. Immediately after the graft is being developed the biliary peritonitis because of the injury of the gallbladder so is done the cholecystectomy. Only in the later course is performed the endoscopic retrograde colangiography, during which are getting the typical changes of the biliary trunk for the primary sclerotic colangitis. The underlying disease in the demonstrated patient is complicated by the recidivens occurrence of the concrements of the choledokus and stenoses of the bladder ducts primarily for the left slice what, up to new, always successfully was treated by the extraction of the concrements and the dilatation of the liver ducts by endoscopic way.

  11. Atypical teratoid/rhabdoid tumor of the sellar region in adult women: Is it a sex-related disease?

    Science.gov (United States)

    Nishikawa, Akihiro; Ogiwara, Toshihiro; Nagm, Alhusain; Sano, Kenji; Okada, Maki; Chiba, Akihiro; Agata, Masahiro; Kaneko, Tomoki; Tamada, Hisashi; Uehara, Takeshi; Hongo, Kazuhiro

    2018-03-01

    Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal tumor in children and a rare entity. Although adult AT/RT is extremely rare, some cases of adult-onset AT/RT in the sellar region have been described. Here, we report an adult patient with AT/RT of the sellar region in whom it was difficult to make a definitive diagnosis and the clinical course was aggressive. This is the first report of autopsy findings that could confirm the clinical characteristics of this rare unresolved pathology, and will contribute to the improvement of prognosis. In addition, a literature review was performed to clarify this exceptionally rare condition. Interestingly, all reported adult patients with sellar AT/RT, along with the present case, were female. This raises the possibility of its being a sex-related disease. However, further studies are required to come to a definitive conclusion. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. Atypical Cities

    Science.gov (United States)

    DiJulio, Betsy

    2011-01-01

    In this creative challenge, Surrealism and one-point perspective combine to produce images that not only go "beyond the real" but also beyond the ubiquitous "imaginary city" assignment often used to teach one-point perspective. Perhaps the difference is that in the "atypical cities challenge," an understanding of one-point perspective is a means…

  13. Atypical Depression

    Science.gov (United States)

    ... coping Other mental health disorders such as anxiety Suicide from feelings of depression Prevention There's no sure way to prevent depression. ... the association between oversleeping and overeating in atypical depression. Journal of Psychosomatic Research. 2015;78:52. Koyuncu A, et al. Relationship ...

  14. [Atypical Lewy body disease revealed by burning mouth syndrome and a pseudo-psychiatric syndrome].

    Science.gov (United States)

    Varvat, J; Thomas-Anterion, C; Decousus, M; Perret-Liaudet, A; Laurent, B

    2010-05-01

    Among the degenerative diseases of the nervous system, Lewy body disease has the most psychiatric symptoms especially hallucinations, delusion and identification disorders. We report a case of Lewy body disease that started with a burning mouth syndrome for three year as the only symptom before the development of a pseudo-psychiatric syndrome (melancholy and Capgras). None of the usual cardinal criteria were present. MRI, cerebrospinal fluid, and DAT scan findings enabled the diagnosis. The dopaminergic hypothesis put forward in some cases of burning mouth syndrome might explain this symptom in Lewy body disease. Copyright 2009 Elsevier Masson SAS. All rights reserved.

  15. Adult onset coats disease in a 56-year-old male: An atypical presentation

    Directory of Open Access Journals (Sweden)

    Bodhraj Dhawan

    2015-01-01

    Full Text Available Coats disease is a congenital retinal vascular disease, which is seen commonly in childhood and remits and exacerbates throughout life. However, it is not rare for coats disease to present in adulthood for the first time. This has been referred to as adult onset coats disease. We do hereby present a rare case of adult onset coats disease in a 56-year-old male patient who presented with visual acuity of 20/200 in the left eye. Fundus findings typical of coats disease included telangiectasis, macular exudation and edema. Fluorescein angiography revealed peripheral capillary nonperfusion areas and telangiectasis. Optical coherence tomography showed cystoid macular edema. After the treatment with intravitreal injection of bevacizumab (1.25 mg in 0.05 ml and sectoral panretinal photocoagulation to the area of capillary nonperfusion the macular edema was reduced by 100 microns, which translated to a visual acuity of 20/80, proving the point that the prognosis in these cases is not as grave as in classical coats disease.

  16. 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.

    Science.gov (United States)

    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.

  17. “Sickle Cell Disease in the Emergency Department: Atypical Complications and Management”

    OpenAIRE

    Brandow, Amanda M.; Liem, Robert

    2011-01-01

    Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hall...

  18. Mass mortality of eastern box turtles with upper respiratory disease following atypical cold weather.

    Science.gov (United States)

    Agha, Mickey; Price, Steven J; Nowakowski, A Justin; Augustine, Ben; Todd, Brian D

    2017-04-20

    Emerging infectious diseases cause population declines in many ectotherms, with outbreaks frequently punctuated by periods of mass mortality. It remains unclear, however, whether thermoregulation by ectotherms and variation in environmental temperature is associated with mortality risk and disease progression, especially in wild populations. Here, we examined environmental and body temperatures of free-ranging eastern box turtles Terrapene carolina during a mass die-off coincident with upper respiratory disease. We recorded deaths of 17 turtles that showed clinical signs of upper respiratory disease among 76 adult turtles encountered in Berea, Kentucky (USA), in 2014. Of the 17 mortalities, 11 occurred approximately 14 d after mean environmental temperature dropped 2.5 SD below the 3 mo mean. Partial genomic sequencing of the major capsid protein from 1 sick turtle identified a ranavirus isolate similar to frog virus 3. Turtles that lacked clinical signs of disease had significantly higher body temperatures (23°C) than sick turtles (21°C) during the mass mortality, but sick turtles that survived and recovered eventually warmed (measured by temperature loggers). Finally, there was a significant negative effect of daily environmental temperature deviation from the 3 mo mean on survival, suggesting that rapid decreases in environmental temperature were correlated with mortality. Our results point to a potential role for environmental temperature variation and body temperature in disease progression and mortality risk of eastern box turtles affected by upper respiratory disease. Given our findings, it is possible that colder or more variable environmental temperatures and an inability to effectively thermoregulate are associated with poorer disease outcomes in eastern box turtles.

  19. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease

    Energy Technology Data Exchange (ETDEWEB)

    Skelton, Brandon W.; Phillips, C.D. [University of Virginia Health System, Department of Neuroradiology, Charlottesville, VA (United States); Hollingshead, Michael C.; Castillo, Mauricio [University of North Carolina School of Medicine, Neuroradiology Section, Chapel Hill, NC (United States); Sledd, Andrew T. [University of Virginia Health System, Department of Pediatrics, Charlottesville, VA (United States)

    2008-07-15

    Acute necrotizing encephalopathy of childhood (ANEC) is a disease entity seen nearly exclusively in East Asian children that is characterized by multifocal, symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. We present a child who developed dramatic neurologic symptoms following a viral prodrome. Serial MRI examinations demonstrated characteristic lesions of ANEC, while laboratory analyses revealed evidence of acute infection with human herpesvirus-6 (HHV-6). We highlight the MRI findings in both the acute and convalescent phases of ANEC, discuss the implications of neuroimaging on the child's clinical course, and emphasize the integral role of the radiologist in correctly diagnosing this rare disease. (orig.)

  20. Diffuse Caroli’s disease with atypical presentation: a case report

    Science.gov (United States)

    Acioli, Maíra Lima; Costa, Lawrence Raizama Gonçalves; de Miranda Henriques, Mônica Souza

    2014-01-01

    This paper describes a case of Caroli’s disease in a female patient aged 32, who complained of nonspecific abdominal pain without cholesthasis or cholangitis. Liver resonance shows segment saccular dilations closely connected to intrahepatic biliary ducts, that differ from the Caroli’s syndrome, which is more common and consists of multiple intrahepatic cystic dilatations, associated to congenital hepatic fibrosis. This patient has a congenital anomaly with an uncommon oligosymptomatic form of Caroli’s disease that should be included in the differential diagnosis of patients with recurrent abdominal pain. PMID:24714420

  1. Characterisation of an atypical manifestation of black band disease on Porites lutea in the Western Indian Ocean.

    Science.gov (United States)

    Séré, Mathieu; Wilkinson, David A; Schleyer, Michael H; Chabanet, Pascale; Quod, Jean-Pascal; Tortosa, Pablo

    2016-01-01

    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.

  2. Diagnosis by whole exome sequencing of atypical infantile onset Alexander disease masquerading as a mitochondrial disorder.

    Science.gov (United States)

    Nishri, Daniella; Edvardson, Simon; Lev, Dorit; Leshinsky-Silver, Esther; Ben-Sira, Liat; Henneke, Marco; Lerman-Sagie, Tally; Blumkin, Lubov

    2014-07-01

    There are many similarities, both clinical and radiological, between mitochondrial leukoencephalopathies and Alexander disease, an astrogliopathy. Clinically, both can manifest with a myriad of symptoms and signs, arising from the neonatal period to adulthood. Radiologically, both can demonstrate white matter changes, signal abnormalities of basal ganglia or thalami, brainstem abnormalities and contrast enhancement of white matter structures. Magnetic resonance spectroscopy may reveal elevation of lactate in the abnormal white matter in Alexander disease making the distinction even more challenging. We present a child who was considered to have an infantile onset mitochondrial disorder due to a combination of neurological symptoms and signs (developmental regression, failure to thrive, episodic deterioration, abnormal eye movements, pyramidal and cerebellar signs), urinary excretion of 3-methyl-glutaconic acid and imaging findings (extensive white matter changes and cerebellar atrophy) with a normal head circumference. Whole exome sequence analysis was performed. The child was found to harbor the R416W mutation, one of the most prevalent mutations in the glial fibrillary acidic protein (GFAP) gene that causes Alexander disease. Alexander disease should be considered in the differential diagnosis of infantile leukoencephalopathy, even when no macrocephaly is present. Next generation sequencing is a useful aid in unraveling the molecular etiology of leukoencephalopathies. Copyright © 2014 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

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

    NARCIS (Netherlands)

    Blumentrath, Christian G.; Grobusch, Martin P.; Matsiégui, Pierre-Blaise; Pahlke, Friedrich; Zoleko-Manego, Rella; Nzenze-Aféne, Solange; Mabicka, Barthélemy; Sanguinetti, Maurizio; Kremsner, Peter G.; Schaumburg, Frieder

    2015-01-01

    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

  4. Atypical Creutzfeldt-Jakob Disease Evolution after Electroconvulsive Therapy for Catatonic Depression

    Directory of Open Access Journals (Sweden)

    Iria Grande

    2011-01-01

    Full Text Available We describe a case report of an 80-year-old woman who presented with symptomatology compatible with an episode of major depression with catatonia. After psychiatric admission, electroconvulsive therapy (ECT was applied, but symptoms progressed with cognitive impairment, bradykinesia, widespread stiffness, postural tremor, and gait disturbance. After compatible magnetic resonance imaging (MRI, diffusion changes, and electroencephalogram (EEG findings the case was reoriented to Creutzfeldt-Jakob disease (CJD. The genetic study found a methionine/valine heterozygosity at codon 129 of the prion protein gene PrPSc. On followup, a significant clinical recovery turned out. For this reason, EEG and MRI were repeated and confirmed the findings. The patient subsequently demonstrated progressive clinical deterioration and died 21 months later. The diagnosis was verified postmortem by neuropathology. The vCJD subtype MV2 is indeed characterized by early and prominent psychiatric symptoms and a prolonged disease duration however no frank clinical recovery has before been reported.

  5. Cavitary pulmonary nodules in atypical collagen disease and lupoid drug reaction. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Muren, C.; Strandberg, O.

    The case histories of two patients with cavitary pulmonary nodules and the findings at chest radiography are reviewed. The first patient had a connective tissue disease with features common to systematic lupus erythematosus and Wegener's granulomatosis. In the second patient the lung changes developed as part of a drug reaction to carbamezapine and/or phenytoin. The common denominator of the cavitating nodules is probably the presence of granulomas, developing as a sequela of pulmonary vasculitis. (orig.).

  6. [Atypical odontalgia].

    Science.gov (United States)

    Türp, Jens Christoph

    2005-01-01

    In spite of its first description by the English surgeon JOHN HUNTER more than 200 years ago, atypical odontalgia (AO), or phantom tooth pain, is not universally known among dentists. AO is a persistent neuropathic pain which may be initiated after deafferentiation of trigeminal nerve fibers following root canal treatment, apicectomy, or tooth extraction. In the absence of pathological clinical or radiological findings, the diagnosis is made by exclusion. After a thorough patient education about the condition, pharmacological and psychological pain management is required. Invasive and irreversible treatment attempts are contraindicated.

  7. Atypical MRI features in familial adult onset Alexander disease: case report.

    Science.gov (United States)

    Liu, Yonghong; Zhou, Heng; Wang, Huabing; Gong, Xiaoqing; Zhou, Anna; Zhao, Lin; Li, Xindi; Zhang, Xinghu

    2016-11-04

    Alexander disease (AxD) is a rare neurological disease, especially in adults. It shows variable clinical and radiological features. We diagnosed a female with AxD presenting with paroxysmal numbness of the limbs at the onset age of 28-year-old, progressing gradually to spastic paraparesis at age 30. One year later, she had ataxia, bulbar paralysis, bowel and bladder urgency. Her mother had a similar neurological symptoms and died within 2 years after onset (at the age of 47), and her maternal aunt also had similar but mild symptoms at the onset age of 54-year-old. Her brain magnetic resonance imaging (MRI) showed abnormal signals in periventricular white matter with severe atrophy in the medulla oblongata and thoracic spinal cord, and mild atrophy in cervical spinal cord, which is unusual in the adult form of AxD. She and her daughter's glial fibrillary acidic protein (GFAP) gene analysis revealed the same heterozygous missense mutation, c.1246C > T, p.R416W, despite of no neurological symptoms in her daughter. Our case report enriches the understanding of the familial adult AxD. Genetic analysis is necessary when patients have the above mentioned symptoms and signs, MRI findings, especially with family history.

  8. Effects of miglustat treatment in a patient affected by an atypical form of Tangier disease.

    Science.gov (United States)

    Sechi, Annalisa; Dardis, Andrea; Zampieri, Stefania; Rabacchi, Claudio; Zanoni, Paolo; Calandra, Sebastiano; De Maglio, Giovanna; Pizzolitto, Stefano; Maruotti, Valerio; Di Muzio, Antonio; Platt, Frances; Bembi, Bruno

    2014-09-18

    Tangier disease (TD) is a rare autosomal recessive disorder, resulting from mutations in the ATP binding cassette transporter (ABCA1) gene. The deficiency of ABCA1 protein impairs high density lipoprotein (HDL) synthesis and cholesterol esters trafficking. A 58 year-old female, presenting with complex clinical signs (splenomegaly, dysarthria, dysphagia, ataxia, tongue enlargement, prurigo nodularis, legs lymphedema, pancytopenia and bone marrow foam cells), was misdiagnosed as Niemann-Pick C (NPC) and treated with miglustat (300 mg/day), normalizing neurological symptoms and improving skin lesions and legs lymphedema. Subsequently filipin-staining and molecular analysis for NPC genes were negative. Lipid profiling showed severe deficiency of HDL, 2 mg/dl (n.v. 45-65) and apoAI, 5.19 mg/dl (n.v. 110-170), suggesting TD as a probable diagnosis. Molecular analysis of ABCA1 gene showed the presence of a novel homozygous deletion (c.4464-486_4698 + 382 Del). Miglustat treatment was then interrupted with worsening of some neurological signs (memory defects, slowing of thought processes) and skin lesions. Treatment was restarted after 7 months with neurological normalization and improvement of skin involvement. These results suggest miglustat as a possible therapeutic approach in this untreatable disease. The mechanisms by which miglustat ameliorates at least some clinical manifestations of TD needs to be further investigated.

  9. Pain processing in atypical Parkinsonisms and Parkinson disease: A comparative neurophysiological study.

    Science.gov (United States)

    Avenali, Micol; Tassorelli, Cristina; De Icco, Roberto; Perrotta, Armando; Serrao, Mariano; Fresia, Mauro; Pacchetti, Claudio; Sandrini, Giorgio

    2017-10-01

    Pain is a frequent non-motor feature in Parkinsonism but mechanistic data on the alteration of pain processing are insufficient to understand the possible causes and to define specifically-targeted treatments. we investigated spinal nociception through the neurophysiological measure of the threshold (TR) of nociceptive withdrawal reflex (NWR) and its temporal summation threshold (TST) comparatively in 12 Progressive Supranuclear Palsy (PSP) subjects, 11 Multiple System Atrophy (MSA) patients, 15 Parkinson's disease (PD) subjects and 24 healthy controls (HC). We also investigated the modulatory effect of L-Dopa in these three parkinsonian groups. We found a significant reduction in the TR of NWR and in the TST of NWR in PSP, MSA and PD patients compared with HC. L-Dopa induced an increase in the TR of NWR in the PSP group while TST of NWR increased in both PSP and PD. Our neurophysiological findings identify a facilitation of nociceptive processing in PSP that is broadly similar to that observed in MSA and PD. Specific peculiarities have emerged for PSP. Our data advance the knowledge of the neurophysiology of nociception in the advanced phases of parkinsonian syndromes and on the role of dopaminergic pathways in the control on pain processing. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

  10. Osteoporosis, weight gain and atypical fat accumulations - a typical feature not only for Cushing's, but also Madelung's disease: A case report.

    Science.gov (United States)

    Friedl, Claudia; Sampl, Eva; Prandl, Eva-Christina; Wagner, Doris; Piswanger-Soelkner, Jutta C; Rosenkranz, Alexander R; Fahrleitner-Pammer, Astrid

    2012-03-01

    Madelung's disease, or multiple symmetric lipomatosis, is an extremely rare disease and very likely to be under-diagnosed. It is characterized by multiple symmetrical non-encapsulated fat accumulations mainly located around the neck, shoulders, upper extremities and upper parts of the trunk. The disorder predominantly affects middle-aged men of Mediterranean origin with a history of ethanol intake. Reports of this uncommon disorder in women are very rare. The aim of this article is to report the endocrine and metabolic workup leading to the diagnosis of this uncommon disorder. A 55-year-old woman with osteoporosis was referred to our outpatient clinic with a suspected diagnosis of Cushing's disease. The patient complained of undesired weight gain with atypical fat accumulations predominantly in the upper trunk region during the previous 10 weeks. She presented with the characteristic physical features of Madelung's disease and underwent a thorough examination with endocrine and metabolic evaluation of this rare condition and was finally diagnosed with Madelung's disease. This report demonstrates how a diagnosis of this rare disorder can be reached efficiently. A history of osteoporosis in combination with weight gain and atypical fat accumulations ultimately led to the diagnosis of Madelung's diseases, a rather unknown disorder likely to be under-diagnosed. Although treatment options are limited, a diagnosis is still important for the affected individual.

  11. Subcellular localization of SREBP1 depends on its interaction with the C-terminal region of wild-type and disease related A-type lamins

    Energy Technology Data Exchange (ETDEWEB)

    Duband-Goulet, Isabelle; Woerner, Stephanie [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Gasparini, Sylvaine [Laboratoire de Biologie Structurale et Radiobiologie, URA CNRS 2096, Commissariat a l' Energie Atomique Saclay, 91190 Gif-sur-Yvette (France); Attanda, Wikayatou [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Konde, Emilie; Tellier-Lebegue, Carine [Laboratoire de Biologie Structurale et Radiobiologie, URA CNRS 2096, Commissariat a l' Energie Atomique Saclay, 91190 Gif-sur-Yvette (France); Craescu, Constantin T. [INSERM U759, Institut Curie/Universite de Paris-Sud, 91405 Orsay Cedex (France); Gombault, Aurelie [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Roussel, Pascal [Institut Jacques Monod, UMR 7592, Universite Paris Diderot-Paris 7, CNRS, 15 rue Helene Brion, 75205 Paris (France); Vadrot, Nathalie; Vicart, Patrick [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Oestlund, Cecilia; Worman, Howard J. [Department of Medicine and Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, NY (United States); and others

    2011-12-10

    Lamins A and C are nuclear intermediate filament proteins expressed in most differentiated somatic cells. Previous data suggested that prelamin A, the lamin A precursor, accumulates in some lipodystrophy syndromes caused by mutations in the lamin A/C gene, and binds and inactivates the sterol regulatory element binding protein 1 (SREBP1). Here we show that, in vitro, the tail regions of prelamin A, lamin A and lamin C bind a polypeptide of SREBP1. Such interactions also occur in HeLa cells, since expression of lamin tail regions impedes nucleolar accumulation of the SREBP1 polypeptide fused to a nucleolar localization signal sequence. In addition, the tail regions of A-type lamin variants that occur in Dunnigan-type familial partial lipodystrophy of (R482W) and Hutchison Gilford progeria syndrome ( Increment 607-656) bind to the SREBP1 polypeptide in vitro, and the corresponding FLAG-tagged full-length lamin variants co-immunoprecipitate the SREBP1 polypeptide in cells. Overexpression of wild-type A-type lamins and variants favors SREBP1 polypeptide localization at the intranuclear periphery, suggesting its sequestration. Our data support the hypothesis that variation of A-type lamin protein level and spatial organization, in particular due to disease-linked mutations, influences the sequestration of SREBP1 at the nuclear envelope and thus contributes to the regulation of SREBP1 function.

  12. HENOCH-SCHÖNLEIN PURPURA WITH AN ATYPICAL PRESENTATION OF THE DISEASE – presentation of three case reports and review of the current literature

    Directory of Open Access Journals (Sweden)

    Daša Kumprej

    2016-04-01

    Full Text Available Background: Henoch-Schönlein purpura is the most common vasculitis of small blood vessels in children. The diagnosis of the disease is confirmed in a patient with a specific rash, joint inflamation, abdominal pain or renal disease. The specific rash is necesary for the confirmation of the diagnosis. Henoch-Schönlein purpura can rarely present with a complication withot a prior presentation of the rash. In these cases diagnosis is difficult until the presentation of the specific skin manifestation. In the majority of patients the disease course is not complicated and has a good prognosis.Conclusion: In this article we present three patients with an atypical presentation of the disease and a review of current literature on the topic.

  13. Treatment options for atypical optic neuritis

    Directory of Open Access Journals (Sweden)

    Amina Malik

    2014-01-01

    Full Text Available Context: Optic neuritis (ON is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or "typical" ON, usually associated with multiple sclerosis. This is in contrast to "atypical" causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision. Aims: The aim was to describe the clinical presentations of atypical ON and their treatments. Settings and Design: Review article. Materials and Methods: Literature review. Results: Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression. Conclusions: Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

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

    CERN Document Server

    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.

  15. National atypical mycobacteria survey, 2000.

    Science.gov (United States)

    Haverkort, Frank

    2003-01-01

    Infections with atypical mycobacteria in Australia during 2000 occurred at a rate of 1.8 cases per 100,000 population. The main sites of disease were the respiratory tract, soft tissue, and the lymphatics. The Mycobacterium avium complex was the most common group of mycobacteria isolated from respiratory, lymphatic sites, and blood. The rapidly growing mycobacteria, predominantly the M. fortuitum-M. abscessus-M. chelonae group were the most common soft tissue infections. Atypical mycobacteria were isolated from significant numbers of sputum 'smear positive' patients, requiring further tests to exclude M. tuberculosis. Geographical differences were observed for some Mycobacterium species, notably the isolation of M. haemophilum from Western Australia, and M. ulcerans from Victoria and Queensland. Newer molecular techniques, while improving precision and accuracy of identification, raise additional questions about the ecology of the atypical mycobacteria and their role in disease.

  16. Atypical hodgkin and reed-sternberg cells in peripheral blood of a patient with advanced stage of Hodgkin's disease - a case report

    Directory of Open Access Journals (Sweden)

    Milošević Rajko

    2003-01-01

    Full Text Available The occurrence of abnormal Hodgkin's and Reed-Sternberg cells in the peripheral blood in a patient suffering from Hodgkin's disease has been noticed exceptionally rare in a previous period, and especially rare in last ten years primarily due to successfull treatment of this disease. The presence of atypical mononuclear cells in peripheral blood which cytomorphologically resembled Reed-Sternberg cells was registered in 8 patients till 1966. During the last decade, the presence of atypical mononuclear cells in the peripheral blood was used for their isolation cultivation, and detailed immunophenotypic and genetic analysis. The analysis of mononuclear cells in rare patients with Hodgkin's disease was established that they belong to the B-lymphoid cells with expression of CD30 and CD15 antigens. The examination of presence of Hodgkin's cells in the peripheral blood of patients with Hodgkin's disease is important for patients with advanced stage of the disease in which autologous stem cell transplantation and high dose chmeotherapy is planned. The authors present a 33-year-old patient, who noticed enlarged neck lymph nodes in September 2000, high temperature and loss in weight. On physical examination enlarged neck lymph nodes 5x8 cm and hepatosplenomegaly were found. There was anemia and thrombo-cytopenia, and normal WBC count with 24% of lymphoid elements in differential formula. On histologic examination of lymph nodes Hodgkin¢s disease, type nodular sclerosis with mixed cellularity was found. Histology of bone marrow showed nodal lymphomatous infiltration. Immunohistochemistry with monoclonal antibodies of concentrate of peripheral blood cells showed expression of CD30+ and CD15+, immunophenotypically and morphologically matching Reed-Sternberg cells. Cytogentic analysis of mononuclear cells of the bone marrow showed normal karyotype. The patient was in clinical stage IV/V of the disease and chemotherapy with 9 cycles of ABVD+Mp protocol was

  17. Comparative Utility of Atypical Antipsychotics for the Treatment of Psychosis in Parkinson's Disease: A Systematic Review and Bayesian Network Meta-analysis.

    Science.gov (United States)

    Iketani, Ryo; Kawasaki, Yohei; Yamada, Hiroshi

    2017-01-01

    We performed a systematic review and Bayesian network meta-analysis to determine atypical antipsychotics that are effective and safe for the treatment of psychosis in Parkinson's disease (PD). We conducted a comprehensive literature search using PubMed/MEDLINE, Cochrane Library, and Japana Centra Revuo Medicina (Ichu-shi Web). We used randomized controlled trials evaluating the utility of atypical antipsychotics for the treatment of psychosis in PD using the Brief Psychiatric Rating Scale (BPRS) and the Unified PD rating Scale parts III (UPDRS-III) as the endpoints. Posterior distributions of mean differences between each treatment and placebo were estimated using Bayesian network meta-analysis. The distributions describing each treatment effect were expressed as means (95% credible intervals). Ten trials involving any two treatment arms using clozapine (64 subjects in four trials), olanzapine (99 subjects in three trials), quetiapine (79 subjects in five trials), risperidone (five subjects in one trial), or placebo (156 subjects in seven trials) were finally included in the present study. Pooled estimates of each posterior distribution based on the BPRS were as follows: clozapine, -2.0 (-6.7 to 2.7); olanzapine, 0.5 (-2.3 to 3.4); quetiapine, 0.3 (-3.9 to 4.5); and risperidone, -4.7 (-57.4 to 53.3). Based on the UPDRS-III, the pooled estimates were clozapine, 0.7 (-3.8 to 4.3); olanzapine, 2.8 (0.8 to 5.1); quetiapine, 3.3 (-0.7 to 5.8); and risperidone, 4.5 (-57.7 to 63.4). Although clozapine had an effective and relatively safe profile, all atypical antipsychotics included in the present study may be unsafe, as they may worsen motor function when compared to placebo.

  18. Transpupillary thermotherapy for atypical central serous chorioretinopathy

    Directory of Open Access Journals (Sweden)

    Kawamura R

    2012-01-01

    Full Text Available Ryosuke Kawamura1,2, Hidenao Ideta1, Hideyuki Hori1, Kenya Yuki2, Tsuyoshi Uno1, Tatsurou Tanabe1, Kazuo Tsubota2, Tsutomu Kawasaki11Ideta Eye Hospital, Kumamoto, Japan; 2Keio University, School of Medicine, Department of Ophthalmology, Tokyo, JapanBackground: Central serous chorioretinopathy (CSC has been traditionally treated with laser photocoagulation. We thought that transpupillary thermotherapy (TTT utilizing a lower temperature than that of conventional laser photocoagulation might minimize permanent retinal and choroidal damage. Studies suggest that undesirable effects on vision due to TTT are minimal even if it is applied to foveal and/or parafoveal lesions when TTT requires a larger irradiation spot. The aim of this study was to evaluate the efficacy of TTT in the management of atypical CSC.Methods: We defined atypical CSC as bullous retinal detachment with diffuse or several leakages, severe leakage with fibrin formation under serous retinal detachment, or leakage within a pigment epithelium detachment. Eight consecutive patients with atypical CSC underwent visual acuity testing, ophthalmic examination, color photography, fluorescein angiography, and optical coherence tomography to evaluate the results of transpupillary thermotherapy. Retreatment of atypical CSC was based on ophthalmic examination, optical coherence tomography, and fluorescein angiography. TTT was performed on the leaking spots shown in fluorescein angiography, with a power of 50–250 mW, spot size of 500–1200 µm, and exposure time of 13–60 seconds to minimize retinal damage.Results: In five of eight affected eyes, serous detachments completely resolved within 1 month after the initial TTT. One eye had persistent subretinal fluid and required a second TTT treatment. Two eyes showed no resolution of CSC and were treated by conventional photocoagulation. Initial best-corrected visual acuity (BCVA ranged from 20/600 to 20/20 (mean, 20/40; median, 20/30. Final BCVA

  19. Atypical case of Wilson's disease with psychotic onset, low 24 hour urine copper and the absence of Kayser-Fleischer rings

    Directory of Open Access Journals (Sweden)

    Krstić Dragan

    2014-01-01

    Full Text Available Introduction. Wilson's disease is typically manifested in two clinical forms, neurological and hepatic and in rare cases it starts with psychiatric symptoms exclusively. We presented a rare atypical case of Wilson's disease with psychotic onset. Case report. A 22-year-old male patient was initially presented with predominant signs and symptoms of psychiatric disorder and then later with the development of neurological signs and symptoms. Neuroimaging, detected metal deposits in central nervous system (CNS but not in peripheral organs, while serum analysis excluded pantothenate-kinase associated neurodegeneration (PKAN and aceruloplasminemia. In favor of the diagnosis of Wilson's disease there were reduced concentrations of copper and ceruloplasmin concentrations and metal deposits in CNS, but other pathognomonic signs and symptoms were absent: in-creased copper in urine, Kayser-Fleischer rings in Descemet’s corneal membrane and deposits of copper in liver. Introduction of penicillamine treatment resulted in improvement in mental and general health of the patient. Molecular genetic analysis definitely confirmed the diagnosis of Wilson's disease. Conclusion. Wilson's disease can remain undetected for a long period of time if masked with dominant or exclusive psychiatric symptoms. If clear clinical symptoms and signs, and unambiguous laboratory findings are not present, it is necessary to perform molecular genetic analysis to confirm the definitive diagnosis.

  20. Recurrent atypical haemolytic uraemic syndrome post kidney transplant due to a CD46 mutation in the setting of SMARCAL1-mediated inherited kidney disease.

    Science.gov (United States)

    Chan, Samuel; Mallett, Andrew J; Patel, Chirag; Francis, Ross S; Johnson, David W; Mudge, David W; Isbel, Nicole M

    2017-02-01

    Disorders in the regulation of the alternate complement pathway often result in complement-mediated damage to the microvascular endothelium and can be associated with both glomerulonephritis and atypical haemolytic uraemic syndrome. Inherited defects in complement regulatory genes or autoantibodies against complement regulatory proteins are predictive of the severity of the disease and the risk of recurrence post kidney transplantation. Heterozygous mutations in CD46, which codes for a transmembrane cofactor glycoprotein membrane cofactor protein, usually have a lower incidence of end-stage kidney disease and decreased risk of recurrent disease post transplant, as wild-type membrane cofactor protein is present in the transplanted kidney. However, some patients with CD46 mutations have a second variant in other complement regulatory genes increasing the severity of disease. The following case report illustrates the course of a young adult patient with end-stage kidney disease initially ascribed to seronegative systemic lupus erythematosus, who presented with biopsy-proven thrombotic microangiopathy following kidney transplantation. It highlights the complexity associated with disorders of complement regulation and the need for a high index of suspicion and genetic testing in patients who present with thrombotic microangiopathy post-transplant. © 2017 Asian Pacific Society of Nephrology.

  1. Distinguishing Parkinson’s disease from atypical parkinsonian syndromes using PET data and a computer system based on support vector machines and Bayesian networks

    Directory of Open Access Journals (Sweden)

    Fermín eSegovia

    2015-11-01

    Full Text Available Differentiating between Parkinson's disease (PD and atypical parkinsonian syndromes (APS is still a challenge, specially at early stages when the patients show similar symptoms. During last years, several computer systems have been proposed in order to improve the diagnosis of PD, but their accuracy is still limited. In this work we demonstrate a full automatic computer system to assist the diagnosis of PD using 18F-DMFP PET data. First, a few regions of interest are selected by means of a two-sample t-test. The accuracy of the selected regions to separate PD from APS patients is then computed using a support vector machine classifier. The accuracy values are finally used to train a Bayesian network that can be used to predict the class of new unseen data. This methodology was evaluated using a database with 87 neuroimages, achieving accuracy rates over 78%. A fair comparison with other similar approaches is also provided.

  2. Distinguishing Parkinson's disease from atypical parkinsonian syndromes using PET data and a computer system based on support vector machines and Bayesian networks.

    Science.gov (United States)

    Segovia, Fermín; Illán, Ignacio A; Górriz, Juan M; Ramírez, Javier; Rominger, Axel; Levin, Johannes

    2015-01-01

    Differentiating between Parkinson's disease (PD) and atypical parkinsonian syndromes (APS) is still a challenge, specially at early stages when the patients show similar symptoms. During last years, several computer systems have been proposed in order to improve the diagnosis of PD, but their accuracy is still limited. In this work we demonstrate a full automatic computer system to assist the diagnosis of PD using (18)F-DMFP PET data. First, a few regions of interest are selected by means of a two-sample t-test. The accuracy of the selected regions to separate PD from APS patients is then computed using a support vector machine classifier. The accuracy values are finally used to train a Bayesian network that can be used to predict the class of new unseen data. This methodology was evaluated using a database with 87 neuroimages, achieving accuracy rates over 78%. A fair comparison with other similar approaches is also provided.

  3. α-Synuclein and anti-α-synuclein antibodies in Parkinson's disease, atypical Parkinson syndromes, REM sleep behavior disorder, and healthy controls.

    Directory of Open Access Journals (Sweden)

    Lynnae M Smith

    Full Text Available α-synuclein is thought to play a key role in Parkinson's disease (PD because it is the major protein in Lewy bodies, and because its gene mutations, duplication, and triplication are associated with early-onset PD. There are conflicting reports as to whether serum and plasma concentrations of α-synuclein and anti-α-synuclein antibodies differ between PD and control subjects. The objectives of this study were to compare the levels of α-synuclein and its antibodies between individuals with typical PD (n=14, atypical Parkinson syndromes (n=11, idiopathic rapid eye movement sleep behavior disorder (n=10, and healthy controls (n=9, to assess the strength of association between these serum proteins, and to determine group sizes needed for a high probability (80% power of detecting statistical significance for 25% or 50% differences between typical PD and control subjects for these measurements. Analysis of log-transformed data found no statistically significant differences between groups for either α-synuclein or its antibodies. The concentrations of these proteins were weakly correlated (Spearman rho=0.16. In subjects with typical PD and atypical Parkinson syndromes, anti-α-synuclein antibody levels above 1.5 µg/ml were detected only in subjects with no more than four years of clinical disease. Power analysis indicated that 236 and 73 samples per group would be required for an 80% probability that 25% and 50% differences, respectively, in mean α-synuclein levels between typical PD and control subjects would be statistically significant; for anti-α-synuclein antibodies, 283 and 87 samples per group would be required. Our findings are consistent with those previous studies which suggested that serum concentrations of α-synuclein and its antibodies are not significantly altered in PD.

  4. 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 Alzheimer disease, r = -0.51; P Alzheimer disease neuropathologic condition with clinically significant neurodegeneration, which will

  5. The relationship of age, blood pressure, serum cholesterol and smoking habits with the risk of typical and atypical coronary heart disease death in the European cohorts of the Seven Countries Study

    NARCIS (Netherlands)

    Menotti, A.; Lanti, M.; Nedeljkovic, S.; Nissinen, A.; Kafatos, A.; Kromhout, D.

    2006-01-01

    Objective: To explore whether "typical" coronary heart disease (CHD) such as fatal myocardial infarction and sudden death relate to major cardiovascular risk factors in the same way as the "atypical" CHD, such as fatal heart failure and chronic arrhythmias. Design and setting: Ten cohorts (6633

  6. A recently isolated Lassa virus from Mali demonstrates atypical clinical disease manifestations and decreased virulence in cynomolgus macaques.

    Science.gov (United States)

    Safronetz, David; Strong, James E; Feldmann, Friederike; Haddock, Elaine; Sogoba, Nafomon; Brining, Douglas; Geisbert, Thomas W; Scott, Dana P; Feldmann, Heinz

    2013-04-15

    The virulence of Soromba-R, a Lassa virus strain recently isolated from southern Mali, was assessed in 2 animal models of Lassa fever: inbred strain 13 guinea pigs and cynomolgus macaques. In both models, the Malian isolate demonstrated tissue tropism and viral titers similar to those of historical Lassa virus isolates from Sierra Leone (Josiah) and Liberia (Z-132); however, the Soromba-R isolate was found to be less pathogenic, as determined by decreased mortality and prolonged time to euthanasia in macaques. Interestingly, in addition to the classic indicators of Lassa fever, Soromba-R infection presented with moderate to severe pulmonary manifestations in the macaque model. Analysis of host responses demonstrated increased immune activation in Soromba-R-infected macaques, particularly in neutrophil-activating or -potentiating proinflammatory cytokines or growth factors, including tumor necrosis factor α, macrophage inflammatory protein 1α, interleukin 1β, and granulocyte colony-stimulating factor, as well as interleukin 5, which may be responsible for the decreased lethality and uncharacteristic clinical presentation. These results suggest that the strain of Lassa virus circulating in Mali might be less pathogenic than strains circulating in the historical region of endemicity and may result in an atypical presentation for Lassa fever, which could complicate clinical diagnosis.

  7. Lie symmetries of the shigesada-Kawasaki-Teramoto system

    Science.gov (United States)

    Cherniha, Roman; Davydovych, Vasyl'; Muzyka, Liliia

    2017-04-01

    The Shigesada-Kawasaki-Teramoto system, which consists of two reaction-diffusion equations with variable cross-diffusion and quadratic nonlinearities, is considered. The system is the most important case of the biologically motivated model proposed by Shigesada et al. (J. Theor. Biol.79(1979) 83-99). A complete description of Lie symmetries for this system is derived. It is proved that the Shigesada-Kawasaki-Teramoto system admits a wide range of different Lie symmetries depending on coefficient values. In particular, the Lie symmetry operators with highly unusual structure are unveiled and applied for finding exact solutions of the relevant nonlinear system with cross-diffusion.

  8. Kawasaki hastalığı

    OpenAIRE

    ÖZDEMİR, Halil; KENDİRLİ, Tanıl

    2004-01-01

    Kawasaki hastalığı, koroner arter anomalisi gelişme riskitaşıyan önemli bir multisistemik hastalıktır. Sıklıkla 5 yaşaltındaki çocuklarda gelişir. Ateşin ortaya çıktığı birkaçgün içinde hastalığın diğer klinik bulguları da genellikleortaya çıkar. Diğer klinik bulgular: Eksüdatif olmayan bulberkonjunktivit, eritemli ağız ve farinks, çilek dili ve kırmızı,çatlamış görünümde dudaklar, bazen morbiliform,makülopapüler, kızıl benzeri döküntüyü veya eritemamultiformeyi andıran generalize, polimorfik...

  9. 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

    . The best known diseases associated with atypical A. salmonicida are carp Cyprinus carpio erythrodermatitis, goldfish Carassius auratus ulcer disease, and ulcer disease of flounder Platichthys flesus, but atypical strains are apparently involved in more disease outbreaks than previously suspected...

  10. Atypical manifestations of leptospirosis.

    Science.gov (United States)

    Rajapakse, Senaka; Rodrigo, Chaturaka; Balaji, Krishan; Fernando, Sumadhya Deepika

    2015-05-01

    Leptospirosis is an illness with a wide spectrum of clinical manifestations and severe illness affects nearly all organ systems. Serious and potentially life-threatening clinical manifestations of acute leptospirosis are caused by both direct tissue invasion by spirochaetes and by the host immune responses. In its severe form, leptospirosis can cause multi-organ dysfunction and death in a matter of days. Therefore it is critical to suspect and recognize the disease early, in order to initiate timely treatment. While the classical presentation of the disease is easily recognized by experienced clinicians practising in endemic regions, rarer manifestations can be easily missed. In this systematic review, we summarize the atypical manifestations reported in literature in patients with confirmed leptospirosis. Awareness of these unusual manifestations would hopefully guide clinicians towards early diagnosis. © The Author 2015. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  11. Atypical manifestations of early syphilis

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Peripheral myelin protein 22 gene duplication with atypical presentations: a new example of the wide spectrum of Charcot-Marie-Tooth 1A disease.

    Science.gov (United States)

    Mathis, Stéphane; Corcia, Philippe; Tazir, Meriem; Camu, William; Magdelaine, Corinne; Latour, Philippe; Biberon, Julien; Guennoc, Anne-Marie; Richard, Laurence; Magy, Laurent; Funalot, Benoît; Vallat, Jean-Michel

    2014-06-01

    Charcot-Marie-Tooth type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are both autosomal-dominant disorders linked to peripheral myelin anomalies. CMT1A is associated with a Peripheral Myelin Protein 22 (PMP22) duplication, whereas HNPP is due to a PMP22 deletion on chromosome 17. In spite of this crucial difference, we report three observations of patients with the 1.4 megabase CMT1A duplication and atypical presentation (electrophysiological, clinical or pathological): a 10 year-old girl with tomaculous lesions on nerve biopsy; a 26 year-old woman with recurrent paresthesiae and block conduction on the electrophysiological study; a 46 year-old woman with transient recurrent nerve palsies mimicking HNPP. These observations highlight the wide spectrum of CMT1A and the overlap between CMT1A and HNPP (both linked to the PMP22 gene), and finally illustrate the complexity of the genotype-phenotype correlations in Charcot-Marie-Tooth diseases. Copyright © 2014 Elsevier B.V. All rights reserved.

  14. Gendered Uncertainty and Variation in Physicians' Decisions for Coronary Heart Disease: The Double-Edged Sword of "Atypical Symptoms"

    Science.gov (United States)

    Welch, Lisa C.; Lutfey, Karen E.; Gerstenberger, Eric; Grace, Matthew

    2012-01-01

    Nonmedical factors and diagnostic certainty contribute to variation in clinical decision making, but the process by which this occurs remains unclear. We examine how physicians' interpretations of patient sex-gender affect diagnostic certainty and, in turn, decision making for coronary heart disease. Data are from a factorial experiment of 256…

  15. An atypical course of Caroli's disease in a renal transplant patient--case report and review of the literature.

    Science.gov (United States)

    Abouchacra, S; Toumeh, S; Boobes, Y; Bernieh, B; Gorka, W

    2004-04-01

    This is a rare case of Caroli's disease, diagnosed following renal transplantation in a patient with autosomal recessive polycystic kidneys. Despite advanced cystic transformation of the biliary tree with striking architectural changes, there was no evidence of portal hypertension or hepatic fibrosis. Moreover, the patient did not suffer a single episode of cholangitis, a most interesting feature of this case. Her clinical course was punctuated by repeated episodes of gastrointestinal and urinary tract infections with resistant organisms; but fortunately, she had no evidence of septicemia. Recurrent Salmonella gastroenteritis indicated a chronic carrier state with the dilated bile ducts possibly acting as a potential reservoir. This has significant implications considering the immune suppression associated with renal transplantation. In general, Caroli's disease is rare. Therefore, a high index of suspicion for the diagnosis of Caroli's disease is warranted especially in patients with ARPKD or ADPKD. Once confirmed, affected patients with end-stage renal disease such as our patient, should ideally undergo combined liver-kidney transplantation.

  16. Intracranial Tuberculoma Presenting as Atypical Eclampsia: A Case Report

    OpenAIRE

    Arumugam, Sendhil Coumary; Murugesan, Sharmila; Pradeep, Sunitha; John, Lopamudra; Kolluru, Vasavi

    2016-01-01

    Occurrence of eclampsia before 20 weeks of pregnancy and after 48 hours of delivery in the absence of typical signs of hypertension and or proteinuria is termed as atypical eclampsia. Atypical or non-classic eclampsia will have some symptoms of eclampsia but without the usual proteinuria or hypertension. All patients with atypical onset should undergo neurological evaluation to rule out neurologic causes of seizures. Cerebral tuberculosis is a rare and serious form of disease secondary to hae...

  17. The Detrimental Effects of Atypical Nonverbal Behavior on Older Adults’ First Impressions of Individuals with Parkinson’s Disease

    OpenAIRE

    Hemmesch, Amanda R.

    2014-01-01

    After viewing short video clips of individuals with Parkinson’s disease (PD) who varied in the symptoms of facial masking (reduced expressivity) and abnormal bodily movement (ABM: including tremor and related movement disorders), older adult observers provided their first impressions of targets’ social positivity. Impressions of targets with higher masking or ABM were more negative than impressions of targets with lower masking or ABM. Furthermore, masking was more detrimental for impressions...

  18. Atypical charles bonnet syndrome

    OpenAIRE

    Priti Arun; Rajan Jain; Vaibhav Tripathi

    2013-01-01

    Charles Bonnet syndrome (CBS) is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision.

  19. The Detrimental Effects of Atypical Nonverbal Behavior on Older Adults’ First Impressions of Individuals with Parkinson’s Disease

    Science.gov (United States)

    Hemmesch, Amanda R.

    2014-01-01

    After viewing short video clips of individuals with Parkinson’s disease (PD) who varied in the symptoms of facial masking (reduced expressivity) and abnormal bodily movement (ABM: including tremor and related movement disorders), older adult observers provided their first impressions of targets’ social positivity. Impressions of targets with higher masking or ABM were more negative than impressions of targets with lower masking or ABM. Furthermore, masking was more detrimental for impressions of women and when observers considered emotional relationship goals, whereas ABM was more detrimental for instrumental relationship goals. This study demonstrated the stigmatizing effects of both reduced and excessive movement. PMID:25244472

  20. The detrimental effects of atypical nonverbal behavior on older adults' first impressions of individuals with Parkinson's disease.

    Science.gov (United States)

    Hemmesch, Amanda R

    2014-09-01

    After viewing short video clips of individuals with Parkinson's disease (PD) who varied in the symptoms of facial masking (reduced expressivity) and abnormal bodily movement (ABM: including tremor and related movement disorders), older adult observers provided their first impressions of targets' social positivity. Impressions of targets with higher masking or ABM were more negative than impressions of targets with lower masking or ABM. Furthermore, masking was more detrimental for impressions of women and when observers considered emotional relationship goals, whereas ABM was more detrimental for instrumental relationship goals. This study demonstrated the stigmatizing effects of both reduced and excessive movement. PsycINFO Database Record (c) 2014 APA, all rights reserved.

  1. 76 FR 82039 - Receipt of Petition for Decision That Nonconforming 2000-2003 Kawasaki ZR750 Motorcycles Are...

    Science.gov (United States)

    2011-12-29

    ...-2003 Kawasaki ZR750 Motorcycles Are Eligible for Importation AGENCY: National Highway Traffic Safety... Kawasaki ZR750 motorcycles that were not originally manufactured to comply with all applicable Federal... whether non-U.S. certified 2000-2003 Kawasaki ZR750 motorcycles are eligible for importation into the...

  2. Diffusive long-time behavior of Kawasaki dynamics

    CERN Document Server

    Cancrin, C; Roberto, C

    2003-01-01

    If $P_t$ is the semigroup associated with the Kawasaki dynamics on $\\Z^d$ and $f$ is a local function on the configuration space, then the variance with respect to the invariant measure $\\mu$ of $P_t f$ goes to zero as $t\\to\\oo$ faster than $t^{-d/2+\\e}$, with $\\e$ arbitrarily small. The fundamental assumption is a mixing condition on the interaction of Dobrushin and Schlosman type.

  3. [Atypical courses of rheumatoid arthritis].

    Science.gov (United States)

    Keitel, W

    1979-04-01

    For the investigation of the question of atypical forms of course selected findings of a multicentric electronic data processing investigation carried out on 1,000 patients with manifest rheumatoid arthritis were attracted. In these cases differences of the clinical symptomatology in the sexes were the result, at a different moment of the beginning and concerning serological findings. The latter was concerned clearly by the titres of rheumatoid factors, only suggestively cases with antinuclear factors. These differences, however, were not regarded as special forms in the sense of separated disease units. They rather represent only statistically provable deviations, the borderlines of which are by far transgressed by individual characteristics.

  4. Usefulness of Cardiac MIBG Scintigraphy, Olfactory Testing and Substantia Nigra Hyperechogenicity as Additional Diagnostic Markers for Distinguishing between Parkinson's Disease and Atypical Parkinsonian Syndromes.

    Directory of Open Access Journals (Sweden)

    Hiroaki Fujita

    Full Text Available We aimed to evaluate the utility of the combined use of cardiac 123I-metaiodobenzylguanidine (MIBG scintigraphy, olfactory testing, and substantia nigra (SN hyperechogenicity on transcranial sonography (TCS in differentiating Parkinson's disease (PD from atypical parkinsonian syndromes (APSs, such as multiple system atrophy (MSA and progressive supranuclear palsy (PSP.Cardiac MIBG scintigraphy, card-type odor identification testing (Open Essence (OE, Wako, Japan, and TCS were performed with 101 patients with PD and 38 patients with APSs (MSA and PSP. Receiver operating characteristic (ROC curve analysis was used to assess the sensitivity and specificity of these batteries for diagnosing PD from APSs. The diagnostic accuracy of the three tests was also assessed among patients at the early disease stage (drug-naïve patients with a disease duration of 3 years or less.In differentiating PD from APSs, the area under the ROC curve was 0.74 (95% CI, 0.65-0.83, 0.8 (95% CI, 0.73-0.87, and 0.75 (95% CI, 0.67-0.82 for TCS, cardiac MIBG scintigraphy, and olfactory testing, respectively. The diagnostic sensitivity and specificity were 53.1% and 91.7%, respectively, for TCS, 70.3% and 86.8%, respectively, for cardiac MIBG scintigraphy, 58.4% and 76.3%, respectively, for OE. Among early-stage patients, sensitivity and specificity were 50.0% and 93.8%, respectively, for TCS, 57.1% and 87.5%, respectively, for cardiac MIBG scintigraphy, and 54.8% and 79.2%, respectively, for OE. At least one positive result from 3 tests improved sensitivity (86.1% but decreased specificity (63.2%. In contrast, at least 2 positive results from 3 tests had good discrimination for both early-stage patients (50.0% sensitivity and 93.8% specificity and patients overall (57.8% sensitivity and 95.8% specificity. Positive results for all 3 tests yielded 100% specificity but low sensitivity (25%.At least 2 positive results from among TCS, cardiac MIBG scintigraphy, and olfactory

  5. Mammographic breast density and risk of breast cancer in women with atypical hyperplasia: an observational cohort study from the Mayo Clinic Benign Breast Disease (BBD) cohort.

    Science.gov (United States)

    Vierkant, Robert A; Degnim, Amy C; Radisky, Derek C; Visscher, Daniel W; Heinzen, Ethan P; Frank, Ryan D; Winham, Stacey J; Frost, Marlene H; Scott, Christopher G; Jensen, Matthew R; Ghosh, Karthik; Manduca, Armando; Brandt, Kathleen R; Whaley, Dana H; Hartmann, Lynn C; Vachon, Celine M

    2017-01-31

    Atypical hyperplasia (AH) and mammographic breast density (MBD) are established risk factors for breast cancer (BC), but their joint contributions are not well understood. We examine associations of MBD and BC by histologic impression, including AH, in a subcohort of women from the Mayo Clinic Benign Breast Disease Cohort. Women with a diagnosis of BBD and mammogram between 1985 and 2001 were eligible. Histologic impression was assessed via pathology review and coded as non-proliferative disease (NP), proliferative disease without atypia (PDWA) and AH. MBD was assessed clinically using parenchymal pattern (PP) or BI-RADS criteria and categorized as low, moderate or high. Percent density (PD) was also available for a subset of women. BC and clinical information were obtained by questionnaires, medical records and the Mayo Clinic Tumor Registry. Women were followed from date of benign biopsy to BC, death or last contact. Standardized incidence ratios (SIRs) compared the observed number of BCs to expected counts. Cox regression estimated multivariate-adjusted MBD hazard ratios. Of the 6271 women included in the study, 1132 (18.0%) had low MBD, 2921 (46.6%) had moderate MBD, and 2218 (35.4%) had high MBD. A total of 3532 women (56.3%) had NP, 2269 (36.2%) had PDWA and 470 (7.5%) had AH. Over a median follow-up of 14.3 years, 528 BCs were observed. The association of MBD and BC risk differed by histologic impression (p-interaction = 0.03), such that there was a strong MBD and BC association among NP (p women were not significant within subsets defined by type of MBD measure (PP vs. BI-RADS), age at biopsy, number of foci of AH, type of AH (lobular vs. ductal) and body mass index, and after adjustment for potential confounding variables. Women with atypia who also had high PD (>50%) demonstrated marginal evidence of increased BC risk (SIR 4.98), but results were not statistically significant. We found no evidence of an association between MBD and subsequent BC

  6. CASE REPORT CASE Atypical tuberculosis of the knee joint CASE ...

    African Journals Online (AJOL)

    This case report demonstrates the MRI findings of atypical tuberculosis. (TB) of the knee joint, caused by Mycobacterium kansasii. Osteoarticular. TB caused by atypical mycobacteria is rare; instead, it is predomi- nantly a synovial disease affecting the tendon sheaths rather than bone. Predisposing factors are ...

  7. Molecular analysis and associated pathology of beak and feather disease virus isolated in Italy from young Congo African grey parrots (Psittacus erithacus) with an "atypical peracute form" of the disease.

    Science.gov (United States)

    Robino, Patrizia; Grego, Elena; Rossi, Giacomo; Bert, Elena; Tramuta, Clara; Stella, Maria Cristina; Bertoni, Pierfrancesco; Nebbia, Patrizia

    2014-01-01

    This study is the first report on the genetic and pathogenic characterization of beak and feather disease virus (BFDV) occurring in Italy. Twenty BFDV strains isolated in Italy from juvenile Congo African grey parrots (Psittacus erithacus) were investigated. Seventeen strains showed an "atypical peracute form" (aPF) of the disease, and three a chronic form (CF). The birds with aPF had been weaned, were independent as far as food and protection were concerned and apparently were without lesions. The gene coding for the putative coat protein was amplified in all isolates while the BFDV genome was sequenced completely in 10 samples, eight of them belonging to aPF affected birds and two from CF of the disease. All full genomes clustered into the J strain of BFDV, where two new subtypes were identified. Recombination analyses showed evidence of genetic exchanges in two BFDV genomes. In addition, a correlation between viral isolate and origin of the breeding material was shown, while an association between the genetic features of the virus and the clinical form was not observed. Histologically, apoptosis was detected frequently in aPF samples and sporadically in CF samples. Interestingly, BFDV antigens were detected in the nuclei and cytoplasm of such apoptotic cells. The data presented here support the hypothesis that, in the absence of a defined BFDV genetic variant accountable for a specific clinical form of psittacine beak and feather disease, differences in the apoptotic rate between aPF and CF are strictly host related.

  8. Atypical Pyoderma Gangrenosum Mimicking an Infectious Process

    Directory of Open Access Journals (Sweden)

    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.

  9. Atypical pyoderma gangrenosum mimicking an infectious process.

    Science.gov (United States)

    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.

  10. Pediatric Melanoma and Atypical Melanocytic Neoplasms.

    Science.gov (United States)

    Sreeraman Kumar, Radhika; Messina, Jane L; Reed, Damon; Navid, Fariba; Sondak, Vernon K

    2016-01-01

    Melanoma is uncommon in the pediatric age range, but is increasing in frequency and often presents with atypical features compared to the classic ABCDE criteria common to adult melanoma cases. Moreover, many melanocytic neoplasms in childhood pose diagnostic challenges to the pathologist, and sometimes cannot be unequivocally classified as benign nevi or melanoma. This chapter addresses the evaluation and management of pediatric patients with melanoma and atypical melanocytic neoplasms, including the roles of and unresolved questions surrounding sentinel lymph node biopsy, completion lymphadenectomy, adjuvant therapy, and treatment of advanced disease.

  11. Primary lateral sclerosis mimicking atypical parkinsonism

    DEFF Research Database (Denmark)

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

    2007-01-01

    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...... 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...

  12. Describing Kawasaki shock syndrome: results from a retrospective study and literature review.

    Science.gov (United States)

    Taddio, Andrea; Rossi, Eleonora Dei; Monasta, Lorenzo; Pastore, Serena; Tommasini, Alberto; Lepore, Loredana; Bronzetti, Gabriele; Marrani, Edoardo; Mottolese, Biancamaria D'Agata; Simonini, Gabriele; Cimaz, Rolando; Ventura, Alessandro

    2017-01-01

    Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6 %) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60 % vs. 23/79, 30 %, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

  13. Atypical odontalgia: a review of the literature.

    Science.gov (United States)

    Melis, Marcello; Lobo, Silvia Lobo; Ceneviz, Caroline; Zawawi, Khalid; Al-Badawi, Emad; Maloney, George; Mehta, Noshir

    2003-01-01

    To review previous reports of cases of atypical odontalgia to examine its epidemiological and clinical characteristics and to explore the etiology and pathophysiology of the disease. Atypical odontalgia is one of many painful conditions that affect the oral cavity and is often overlooked in the differential diagnosis. A search of the literature was performed for all cases of atypical odontalgia reported from 1966 to the present. The typical clinical presentation of atypical odontalgia that has been reported involves pain in a tooth in the absence of any sign of pathology; the pain may spread to areas of the face, neck, and shoulder. The existing literature suggests that this condition occurs in 3% to 6% of the patients who undergo endodontic treatment, with high female preponderance and a concentration of cases in the fourth decade of life. Deafferentation seems to be the most likely mechanism to initiate the pain, but psychological factors, alteration of neural mechanisms, and even an idiopathic mechanism have been implicated. Not all reported cases were preceded by trauma to the teeth or gums. The treatment of choice is a tricyclic antidepressant, alone or in combination with a phenothiazine. The outcome is usually fair, with many patients obtaining complete relief from pain. Especially in the absence of overt pathology, particular attention should be paid to avoiding any unnecessary and potentially dangerous dental intervention on the teeth. Atypical odontalgia is surprisingly common, of uncertain origin, and potentially treatable.

  14. Atypical swallowing: a review.

    Science.gov (United States)

    Maspero, C; Prevedello, C; Giannini, L; Galbiati, G; Farronato, G

    2014-06-01

    Atypical swallowing is a myofunctional problem consisting of an altered tongue position during the act of swallowing. High incidence in population, multifactorial etiology and the recurring connection with the presence of malocclusions made it a topic of strong interest and discussion in science. The purpose of this review is to illustrate the current orientation on the topic of atypical swallowing, trying in particular to answer two questions: 1) what kind of connection is there between atypical swallowing and malocclusion; 2) what kind of therapy should be used to solve it. This review was conducted on the Medline database [www.ncbi.nim.nih.gov/pubmed] searching for the keywords "atypical swallowing" and "tongue thrust". We examined all the documents from the year 1990 onwards, excluding the ones about syndromic cases of the central motor system. The causal relation between the two problems seems to be biunique: some authors affirm that this oral habit starts as a compensation mechanism for a preexisting malocclusion (especially in case of open-bite); other texts show that it has a tendency to exacerbate cases of malocclusion; it is also proven that a non-physiological tongue thrust can negatively influence the progress of an ongoing orthodontic therapy. Thereby, the best therapeutic approach seems to be a multidisciplinary one: beside orthodontics, which is necessary to correct the malocclusion, it is essential to set up a myofunctional rehabilitation procedure to correct the oral habit, therefore granting long time permanent results. There is also proof of a substantial difference between the results obtained from early (deciduous or primary mixed dentition) or later treatments. The biunique causal relation between atypical swallowing and malocclusion suggests a multidisciplinary therapeutic approach, orthodontic and myofunctional, to temporarily solve both problems. An early diagnosis and a prompt intervention have a significantly positive influence on the

  15. Renormalized dynamics of the Dean-Kawasaki model

    Science.gov (United States)

    Bidhoodi, Neeta; Das, Shankar P.

    2015-07-01

    We study the model of a supercooled liquid for which the equation of motion for the coarse-grained density ρ (x ,t ) is the nonlinear diffusion equation originally proposed by Dean and Kawasaki, respectively, for Brownian and Newtonian dynamics of fluid particles. Using a Martin-Siggia-Rose (MSR) field theory we study the renormalization of the dynamics in a self-consistent form in terms of the so-called self-energy matrix Σ . The appropriate model for the renormalized dynamics involves an extended set of field variables {ρ ,θ } , linked through a nonlinear constraint. The latter incorporates, in a nonperturbative manner, the effects of an infinite number of density nonlinearities in the dynamics. We show that the contributing element of Σ which renormalizes the bare diffusion constant D0 to DR is same as that proposed by Kawasaki and Miyazima [Z. Phys. B Condens. Matter 103, 423 (1997), 10.1007/s002570050396]. DR sharply decreases with increasing density. We consider the likelihood of a ergodic-nonergodic (ENE) transition in the model beyond a critical point. The transition is characterized by the long-time limit of the density correlation freezing at a nonzero value. From our analysis we identify an element of Σ which arises from the above-mentioned nonlinear constraint and is key to the viability of the ENE transition. If this self-energy would be zero, then the model supports a sharp ENE transition with DR=0 as predicted by Kawasaki and Miyazima. With the full model having nonzero value for this self-energy, the density autocorrelation function decays to zero in the long-time limit. Hence the ENE transition is not supported in the model.

  16. Metastable and scaling regimes of one-dimensional Kawasaki dynamics

    Science.gov (United States)

    Albarracín, F. A. Gómez; Rosales, H. D.; Grynberg, M. D.

    2016-04-01

    We investigate the large-time scaling regimes arising from a variety of metastable structures in a chain of Ising spins with both first- and second-neighbor couplings while subject to Kawasaki dynamics. Depending on the ratio and sign of these former, different dynamic exponents are suggested by finite-size scaling analyses of relaxation times. At low but nonzero temperatures these are calculated via exact diagonalizations of the evolution operator in finite chains under several activation barriers. In the absence of metastability the dynamics is always diffusive.

  17. Recurrent Fever Syndromes in Patients After Recovery From Kawasaki Syndrome

    Science.gov (United States)

    Tremoulet, Adriana H.; Burns, Jane C.; Bastian, John F.; Hoffman, Hal M.

    2011-01-01

    The recurrence of fever in a child with a history of Kawasaki syndrome (KS) poses a dilemma for clinicians who must consider the possibility of recurrent KS. In this report we present the cases of 4 patients who presented with classical symptoms of KS, were successfully treated with intravenous immunoglobulin, and later experienced a reappearance of inflammatory symptoms in a pattern consistent with a recurrent fever syndrome. The association of these syndromes within the same patient suggests that some patients may have a genetic propensity toward altered immune responses and autoinflammatory syndromes. We propose that these 2 syndromes exist within a family of febrile disorders related to innate immune dysregulation. PMID:21220401

  18. Comportamiento clínico-epidemiológico de la enfermedad de Kawasaki

    OpenAIRE

    Milagros Morales Leiva; María del Carmen Luis Álvarez; Frances Seiglie Díaz; Odette Pantoja Pereda

    2011-01-01

    INTRODUCCIÓN. La enfermedad de Kawasaki es una vasculitis sistémica, aguda, febril, de evolución autolimitada, con alto riesgo de secuelas en niños menores de 5 años, si no se diagnostica y trata en su estadio inicial. Fue objetivo de este estudio describir el comportamiento clínico-epidemiológico de la enfermedad de Kawasaki en la infancia. MÉTODOS. Se realizó un estudio descriptivo, de aspectos clínicos y epidemiológicos, de los pacientes con diagnóstico de enfermedad de Kawasaki atendidos ...

  19. Pontine Infarct Presenting with Atypical Dental Pain: A Case Report.

    Science.gov (United States)

    Goel, Rajat; Kumar, Sanjeev; Panwar, Ajay; Singh, Abhishek B

    2015-01-01

    Orofacial pain' most commonly occurs due to dental causes like caries, gingivitis or periodontitis. Other common causes of 'orofacial pain' are sinusitis, temporomandibular joint(TMJ) dysfunction, otitis externa, tension headache and migraine. In some patients, the etiology of 'orofacial pain' remains undetected despite optimal evaluation. A few patients in the practice of clinical dentistry presents with dental pain without any identifiable dental etiology. Such patients are classified under the category of 'atypical odontalgia'. 'Atypical odontalgia' is reported to be prevalent in 2.1% of the individuals. 'Atypical orofacial pain' and 'atypical odontalgia' can result from the neurological diseases like multiple sclerosis, trigeminal neuralgia and herpes infection. Trigeminal neuralgia has been frequently documented as a cause of 'atypical orofacial pain' and 'atypical odontalgia'. There are a few isolated case reports of acute pontine stroke resulting in 'atypical orofacial pain' and 'atypical odontalgia'. However, pontine stroke as a cause of atypical odontalgia is limited to only a few cases, hence prevalence is not established. This case is one, where a patient presented with acute onset atypical dental pain with no identifiable dental etiology, further diagnosed as an acute pontine infarct on neuroimaging. A 40 years old male presented with acute onset, diffuse teeth pain on right side. Dental examination was normal. Magnetic resonance imaging(MRI) of the brain had an acute infarct in right pons near the trigeminal root entry zone(REZ). Pontine infarct presenting with dental pain as a manifestation of trigeminal neuropathy, has rarely been reported previously. This stresses on the importance of neuroradiology in evaluation of atypical cases of dental pain.

  20. Weight change after an atypical antipsychotic switch.

    Science.gov (United States)

    Ried, L Douglas; Renner, Bernard T; Bengtson, Michael A; Wilcox, Brian M; Acholonu, Wilfred W

    2003-10-01

    Atypical antipsychotics successfully treat schizophrenia and other conditions, with a lower incidence of extrapyramidal side effects than other agents used in treatment of these disorders. However, some atypical antipsychotics are associated with weight gain. To quantify the impact on weight and identify atypical antipsychotics causing the least amount of weight gain among patients switched from risperidone to olanzapine and olanzapine to risperidone. Patients included in the study (n = 86) were > or =18 years and had received > or =2 prescriptions for risperidone or olanzapine for > or =60 days, switched to the other atypical antipsychotic, and were dispensed > or =2 prescriptions for at least 60 days after the index date. Age, weight, and body mass index (BMI) were retrospectively abstracted from automated databases containing patient-specific prescription and vital sign information. At the time of their switch, the average patient age was 53.2 years (range 25-83). The average weight change in patients switched to olanzapine (n = 47) was +2.3 kg (p = 0.01) and the BMI change was +0.8 kg/m(2) (p = 0.02). The average percent body weight change was +2.8% and the BMI change was +3.0%. The average weight change after patients switched to risperidone (n = 39) was -0.45 kg (p = 0.69) and BMI change was -0.2 kg/m2 (p = 0.64). The average percentage weight change was -0.4% and BMI change was -0.5%. Practitioners' concern regarding weight changes after switching atypical antipsychotics seems warranted and patients should be provided consistent, ongoing weight monitoring. Further investigations should examine whether weight changes associated with atypical antipsychotic treatment further jeopardize this already at-risk population for severe comorbid conditions such as hypertension, coronary artery disease, and type 2 diabetes.

  1. Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

    Directory of Open Access Journals (Sweden)

    Y. T. Udaka

    2013-01-01

    Full Text Available Atypical Teratoid Rhabdoid Tumor (ATRT is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.

  2. Atypical femoral fractures

    OpenAIRE

    Giannini, Sandro; Chiarello, Eugenio; Tedesco, Giuseppe; Cadossi, Matteo; Luciani, Deianira; Mazzotti, Antonio; Donati, Davide Maria

    2013-01-01

    Bisphosphonates (BPs) represent the most widely used therapy for osteoporosis. Recently, a relationship between long-term treatment with BPs and a subset of atypical femoral fractures (AFFs) from below the lesser trochanter to the sovracondilar line has been described. Many etiopathogenetic theories have been invoked to explain AFFs: reduced bone turnover and increased osteoblast bone apposition with accumulation of microdamage and decreased bone toughness with subsequent increased risk of mi...

  3. Kawasaki syndrome: Risk factors for coronary artery aneurysms

    Directory of Open Access Journals (Sweden)

    A. V. Torbyak

    2015-01-01

    Full Text Available Kawasaki syndrome is a childhood systemic vasculitis that may affect coronary arteries, further leading to myocardial ischemia or myocardi-al infarction. This investigation is our country's first experience with regression analysis to find risk factors for coronary artery involvement in Kawasaki syndrome. The outpatient and inpatient medical records of 168 KD patients aged 1 month 13 days to 13 years 6 months were retrospectively analyzed. Results. The investigators revealed risk factors for coronary artery involvement: persistent fever; prolonged fever prior to immunoglobulin infusion; low hemoglobin in acute phase; leukocytosis more than 17* 109/l in acute phase; thrombocytosis more than 790-109/! and obviously increased erythrocyte sedimentation rate; a more than 5-fold rise in normal C-reactive protein; immunoglobulin administration after day 10; and no immunoglobulin therapy. The dose of immunoglobulin of 2 g/kg reduces the risk of coronary artery aneurysms, but its effect depends on the day of administration. Cervical lymphadenopathy and immunoglobulin resistance may be risk factors for coronary artery aneurysms, but further investigations are needed to solve this problem.

  4. Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

    Science.gov (United States)

    Andréoletti, Olivier; Orge, Leonor; Benestad, Sylvie L.; Beringue, Vincent; Litaise, Claire; Simon, Stéphanie; Le Dur, Annick; Laude, Hubert; Simmons, Hugh; Lugan, Séverine; Corbière, Fabien; Costes, Pierrette; Morel, Nathalie; Schelcher, François; Lacroux, Caroline

    2011-01-01

    Atypical/Nor98 scrapie was first identified in 1998 in Norway. It is now considered as a worldwide disease of small ruminants and currently represents a significant part of the detected transmissible spongiform encephalopathies (TSE) cases in Europe. Atypical/Nor98 scrapie cases were reported in ARR/ARR sheep, which are highly resistant to BSE and other small ruminants TSE agents. The biology and pathogenesis of the Atypical/Nor98 scrapie agent in its natural host is still poorly understood. However, based on the absence of detectable abnormal PrP in peripheral tissues of affected individuals, human and animal exposure risk to this specific TSE agent has been considered low. In this study we demonstrate that infectivity can accumulate, even if no abnormal PrP is detectable, in lymphoid tissues, nerves, and muscles from natural and/or experimental Atypical/Nor98 scrapie cases. Evidence is provided that, in comparison to other TSE agents, samples containing Atypical/Nor98 scrapie infectivity could remain PrPSc negative. This feature will impact detection of Atypical/Nor98 scrapie cases in the field, and highlights the need to review current evaluations of the disease prevalence and potential transmissibility. Finally, an estimate is made of the infectivity loads accumulating in peripheral tissues in both Atypical/Nor98 and classical scrapie cases that currently enter the food chain. The results obtained indicate that dietary exposure risk to small ruminants TSE agents may be higher than commonly believed. PMID:21347349

  5. Atypical odontalgia--an update.

    Science.gov (United States)

    Patel, Seena B; Boros, Audrey L; Kumar, Satish K S

    2012-09-01

    Atypical odontalgia is a commonly misdiagnosed condition that frequently leads to unnecessary dental treatments such as extraction and endodontic therapy. These treatments often worsen the pain. Despite greater recognition and understanding of this condition, proper diagnosis and treatment remains a challenge. It is believed that atypical odontalgia is a neuropathic condition. This article updates the current understanding of the etiology and pathophysiology of atypical odontalgia, and provides appropriate diagnostic and management approaches for this condition.

  6. Intracranial Tuberculoma Presenting as Atypical Eclampsia: A Case Report.

    Science.gov (United States)

    Arumugam, Sendhil Coumary; Murugesan, Sharmila; Pradeep, Sunitha; John, Lopamudra; Kolluru, Vasavi

    2016-06-01

    Occurrence of eclampsia before 20 weeks of pregnancy and after 48 hours of delivery in the absence of typical signs of hypertension and or proteinuria is termed as atypical eclampsia. Atypical or non-classic eclampsia will have some symptoms of eclampsia but without the usual proteinuria or hypertension. All patients with atypical onset should undergo neurological evaluation to rule out neurologic causes of seizures. Cerebral tuberculosis is a rare and serious form of disease secondary to haematogenous spread of Mycobacterium tuberculosis. Here we present a case of cerebral tuberculoma with seizures in late pregnancy mimicking eclampsia.

  7. Detection of Norovirus GII.17 Kawasaki 2014 in Shellfish, Marine Water and Underwater Sewage Discharges in Italy.

    Science.gov (United States)

    La Rosa, G; Della Libera, S; Iaconelli, M; Proroga, Y T R; De Medici, D; Martella, V; Suffredini, E

    2017-09-01

    V strains to be widespread in shellfish samples collected in 2015 in Italy, provide indirect evidence that this strain has started circulating in the Italian population. Notably, using a specific assay, we were able to detect many more samples positive for GII.17 NoV, indicating that, in food and water matrices, broad-range assays for NoV may grossly underestimate the prevalence of some, less common, NoVs. The detection of the GII.17 strain Kawasaki 2014 in clinical, water and food samples in Italy highlights the need for more systematic surveillance for future disease control and prevention.

  8. 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.

  9. Association of Sterile Pyuria and Coronary Artery Aneurysm in Kawasaki Syndrome

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    Mohsen Akhavan Sepahi

    2011-09-01

    Full Text Available "nKawasaki disease (KD is an inflammatory multiorgan disease of unknown etiology. The most dramatic organ involved is the heart. There were a few studies about cardiac involvement and sterile pyuria. This study guides to determine if sterile pyuria is associated with coronary artery aneurysm (CAA in KD patients and to consider it as a predicting factor for coronary artery involvement. Forty seven patients with KD were studied by echocardiography in admission and one month later. Urine analysis, complete blood count, erythrocyte sedimentation rate and C-reactive protein were measured in admission. Data were analyzed using SPSS-14 software. Patients' age was ranged from 13 month to 7 years old (mean age of 3.43 ± 1.54 years. Thirty patients (63.8% were male and 17 patients (36.1% were female. Cardiac involvement was detected in 32 patients (68% using echocardiography, of which CAA was reported in 8 cases (17%. Six of CAA (75% were in association with sterile pyuria, although it was statistically insignificant (P>0.05. Although the majority of patients with CAA had sterile pyuria, this association is not statistically significant, thus it couldn't be considered as a predicting factor for CAA.

  10. Development of KB-200. Kawasaki digital burner control system

    Energy Technology Data Exchange (ETDEWEB)

    Miyayama, Toshio; Hoshino, Norihiko; Nakamori, Hideki; Wasada, Norihiko

    1988-04-20

    Control equipments of a thermal generation plant are making progress in getting higher in level, complex and integrated digitalization. Concerning the digital control technology, as micro-electronics technology progresses, programmable controller which excels in flexibility and expandability of software is now widely popularized. Recently, however, higher level of reliability, maintenance ability and safety are required as in the discovery of abnormality and fact-finding or the protection of human error. To respond to these, Kawasaki Heavy Industries, in cooperationwith Fuji Electric Co., has developed a distributed digital burner control unit on the basis of the recent programmable controller. This report outlines the system control functions, construction, measures for high reliability, controllers, and system maintenance, etc.. (8 figs, 2 tabs, 2 refs)

  11. Familial Occurrence of Kawasaki Syndrome in North America

    Science.gov (United States)

    Dergun, Marina; Kao, Annie; Hauger, Sarmistha B.; Newburger, Jane W.; Burns, Jane C.

    2010-01-01

    Objective To describe families with multiple members affected with Kawasaki syndrome (KS) to increase awareness of the familial occurrence of KS among practitioners who care for these patients. Design Retrospective review of medical records at 2 medical centers and data collection from remote KS families who contacted the KS Research Program at the University of California, San Diego. Results Eighteen families with multiple affected members were identified. There were 9 families with 2 affected siblings. In San Diego, 3 (0.7%) of 424 KS families had sibling cases. Nine families were identified with KS in 2 generations or in multiple affected members, yielding a total of 24 KS-affected children. No clear pattern of inheritance could be deduced from these pedigrees, and it is likely that multiple polymorphic alleles influence KS susceptibility. Conclusion Physicians should counsel affected families and make them aware of the potential increased risk of KS among family members. PMID:16143748

  12. Atypical depression in the structure of organic mental disorders (literature review

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    Leonov S.F.

    2014-09-01

    Full Text Available The review of literature presents current data on cli¬nical picture and diagnostics of atypical depression. Rubric “atypical depression” includes a variety of depressive states characterized by reactively caused changes of mood, sensitivity to interpersonal contacts, inverted vegetative and somatic symptoms such as increased appetite and hypersomnia. The article considers the place of atypical depression in the structure of organic mental disorders. Positions of foreign authors that produce atypical depression as a clinical entity in the structure of Bipolar affective disorder II type are represented, the views of other authors on the structure of atypical depression are considered. The analysis of national concept of non-circular depression is carried out. Questions of atypical affective conditions acquire special significance due to preparation of International Classification of Diseases of the 11th revision, because inclusion in it of Bipolar affective disorder II type, a manifestation of which is considered to be atypical depressions, is under discussion.

  13. Nasal potential difference measurements in patients with atypical cystic fibrosis.

    Science.gov (United States)

    Wilschanski, M; Famini, H; Strauss-Liviatan, N; Rivlin, J; Blau, H; Bibi, H; Bentur, L; Yahav, Y; Springer, H; Kramer, M R; Klar, A; Ilani, A; Kerem, B; Kerem, E

    2001-06-01

    The diagnosis of cystic fibrosis (CF) is based on characteristic clinical and laboratory findings. However, a subgroup of patients present with an atypical phenotype that comprises partial CF phenotype, borderline sweat tests and one or even no common cystic fibrosis transmembrane conductance regulator (CFTR) mutations. The aim of this study was to evaluate the role of nasal potential difference (PD) measurements in the diagnosis of CF patients with an atypical presentation and in a population of patients suspected to have CF. Nasal PD was measured in 162 patients from four different groups: patients with classical CF (n = 31), atypical phenotype (n = 11), controls (n = 50), and patients with questionable CF (n = 70). The parameter, or combination of nasal PD parameters was calculated in order to best discriminate all CF patients (including atypical CF) from the non-CF group. The patients with atypical CF disease had intermediate values of PD measurements between the CF and non-CF groups. The best discriminate model that assigned all atypical CF patients as CF used: e(response to chloride-free and isoproterenol/response to amiloride) with a cut-off >0.70 to predict a CF diagnosis. When this model was applied to the group of 70 patients with questionable CF, 24 patients had abnormal PD similar to the atypical CF group. These patients had higher levels of sweat chloride concentration and increased rate of CFTR mutations. Nasal potential difference is useful in diagnosis of patients with atypical cystic fibrosis. Taking into account both the sodium and chloride transport elements of the potential difference allows for better differentiation between atypical cystic fibrosis and noncystic fibrosis patients. This calculation may assist in the diagnostic work-up of patients whose diagnosis is questionable.

  14. Atypical Presentation of Neurosyphilis

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    L C Anand

    1980-01-01

    Full Text Available Five cases of neurospyhilis with atypical manifestation have been reported. Of these four cases presented as acute neurological illness and showed variable recovery after antisyp′iiilitic therapy. One of these cases had parinaud sip which was unaffected by treatment One case presented as dementia and gave poor response to therapy. In only one of these five cases was reagin in CSF demonstrated. Lange′s colloidal gold test was negative in all. As such failure to demonstrate reagin in CSF does not rule out the diagnosis of neurosyphilis. In an antibiotic era patients may inadvertently receive some antibiotics prior to presentation to a clinician and therefore are unlikely to present with typical neurological and laboratory findings.

  15. [Atypical bipolar disorders].

    Science.gov (United States)

    Gay, Christian

    2009-04-20

    Some epidemiologic data reveal how difficult detecting atypic bipolar disorders is: 9 years of progression before the diagnosis is properly established and a specific treatment is initiated, and intervention of 4 to 5 different specialists. Incomplete symptomatology, impulsive actions, periodic alcohol abuse, compulsive buying behaviors, acute delusional episodes, medicolegal actions and comorbidities can hide or modify bipolar symptomatology. Bipolarity should be systematically screened for in case of substance abuse (40 to 60 percent of bipolar disorders), anxiety disorders (panic disorder, generalized anxiety, obsessive-compulsive disorders etc.) and feeding disorders. In these various situations, history taking and clinical examination will help to detect signs of bipolarity: reaction to antidepressants, inefficiency, paradoxical worsening, development of behavior disorders and mood changes. Besides screening for thymic disorders, the examination will be completed by history taking of thymic disorders, suicide, toxic abuse, anxiety disorders, personal history of attention deficit hyperactivity disorder in childhood, depression or postpartum psychosis in women, as well as premenstrual depressive manifestations.

  16. Atypical odontalgia: a review.

    Science.gov (United States)

    Koratkar, Harish; Pedersen, Jerome

    2008-01-01

    Since persistent and chronic pain is more common in the head and neck region than in any other part of the body, dentists are more likely to encounter these rather complex cases in their practices. This article is a review and update on atypical odontalgia (AO). AO is a persistent neuropathic pain which may be initiated after deafferentiation of trigeminal nerve fibers following root canal treatment, apicectomy, or tooth extraction, or it may be of idiopathic origin. Details concerning its characteristics, pathophysiology, diagnostic criteria, differential diagnosis, and treatment are made. The aim of this article is to help the clinician with the diagnosis and management of AO. The prognosis for AO is most often only fair, and the administration of tricyclic antidepressants often resolves symptoms. Invasive and irreversible treatment attempts are not recommended.

  17. Atypical depression: current perspectives

    Directory of Open Access Journals (Sweden)

    Łojko D

    2017-09-01

    Full Text Available Dorota Łojko,1 Janusz K Rybakowski1,2 1Department of Adult Psychiatry, 2Department of Child and Adolescent Psychiatry, Poznan University of Medical Sciences, Poznan, Poland Abstract: The history and present status of the definition, prevalence, neurobiology, and treatment of atypical depression (AD is presented. The concept of AD has evolved through the years, and currently, in Diagnostic and Statistical Manual of Mental Disorders (DSM, Fifth Edition, the specifier of depressive episode with atypical feature is present for both diagnostic groups, that is, depressive disorders and bipolar and related disorders. This specifier includes mood reactivity, hyperphagia, hypersomnia, leaden paralysis, and interpersonal rejection sensitivity. Prevalence rates of AD are variable, depending on the criteria, methodology, and settings. The results of epidemiological studies using DSM criteria suggest that 15%–29% of depressed patients have AD, and the results of clinical studies point to a prevalence of 18%–36%. A relationship of AD with bipolar depression, seasonal depression, and obesity has also been postulated. Pathogenic research has been mostly focused on distinguishing AD from melancholic depression. The differences have been found in biochemical studies in the areas of hypothalamic–pituitary–adrenal axis, inflammatory markers, and the leptin system, although the results obtained are frequently controversial. A number of findings concerning such differences have also been obtained using neuroimaging and neurophysiological and neuropsychological methods. An initial concept of AD as a preferentially monoamine oxidase inhibitor-responsive depression, although confirmed in some further studies, is of limited use nowadays. Currently, despite numerous drug trials, there are no comprehensive treatment guidelines for AD. We finalize the article by describing the future research perspectives for the definition, neurobiology, and treatment. A better

  18. Atypical depression: current perspectives

    Science.gov (United States)

    Łojko, Dorota; Rybakowski, Janusz K

    2017-01-01

    The history and present status of the definition, prevalence, neurobiology, and treatment of atypical depression (AD) is presented. The concept of AD has evolved through the years, and currently, in Diagnostic and Statistical Manual of Mental Disorders (DSM), Fifth Edition, the specifier of depressive episode with atypical feature is present for both diagnostic groups, that is, depressive disorders and bipolar and related disorders. This specifier includes mood reactivity, hyperphagia, hypersomnia, leaden paralysis, and interpersonal rejection sensitivity. Prevalence rates of AD are variable, depending on the criteria, methodology, and settings. The results of epidemiological studies using DSM criteria suggest that 15%–29% of depressed patients have AD, and the results of clinical studies point to a prevalence of 18%–36%. A relationship of AD with bipolar depression, seasonal depression, and obesity has also been postulated. Pathogenic research has been mostly focused on distinguishing AD from melancholic depression. The differences have been found in biochemical studies in the areas of hypothalamic–pituitary–adrenal axis, inflammatory markers, and the leptin system, although the results obtained are frequently controversial. A number of findings concerning such differences have also been obtained using neuroimaging and neurophysiological and neuropsychological methods. An initial concept of AD as a preferentially monoamine oxidase inhibitor-responsive depression, although confirmed in some further studies, is of limited use nowadays. Currently, despite numerous drug trials, there are no comprehensive treatment guidelines for AD. We finalize the article by describing the future research perspectives for the definition, neurobiology, and treatment. A better specification of diagnostic criteria and description of clinical picture, a genome-wide association study of AD, and establishing updated treatment recommendations for this clinical phenomenon should be

  19. Herpes zoster - typical and atypical presentations.

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  2. Profile of hepatitis A infection with atypical manifestations in children.

    Science.gov (United States)

    Samanta, Tryambak; Das, Anjan Kumar; Ganguly, Sutapa

    2010-01-01

    We assessed the clinical course and biochemical profile of symptomatic children with viral hepatitis A who had atypical manifestations. Of 229 children with hepatitis A, atypical manifestations were found in 32 (14%) subjects. Prolonged cholestasis (n = 14), acute liver failure (9), relapse (9), ascites (8), and hematological problems (8) were the common presentations. Liver histology was suggestive of chronic liver disease in six children with protracted jaundice. Patients with atypical presentations were older (7.7 [1.6] years vs. 6.5 [2.6] years; p=0.012) and had higher total serum bilirubin (13.7 [8.1] mg/dL vs. 7.2 [4.0] mg/dL; p=children with acute hepatitis A infection have atypical presentation which is associated with increase in morbidity.

  3. SUCCESSFUL TREATMENT FOR ATYPICAL HEMOLYTIC UREMIC SYNDROME IN A PUERPERA

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    O. N. Ulitkina

    2015-01-01

    Full Text Available Objective: to show the problems of differential diagnosis and treatment of atypical hemolytic-uremic syndrome in a 23-year-old patient.Results. Eculizumab (Soliris, (Alexon Pharmaceuticals Inc., USA that is a glycosylated humanized monoclonal antibody to immunoglobulins (IgG2/4k is shown to be effective in treating this disease.Conclusion. Atypical hemolytical-uremic syndrome in pregnancy is a disease, whose treatment difficulties are largely associated with the problem of differential diagnosis with thrombotic thrombocytopenic purpura and man-ifestations of multiple organ dysfunction. The treatment for this disease gives a key role to Eculizumab.

  4. [Prevalence and determinants of atypical presentation of acute coronary syndrome].

    Science.gov (United States)

    Pinto, David; Lunet, Nuno; Azevedo, Ana

    2011-12-01

    Knowledge of the characteristics of patients with atypical presentation of acute coronary syndromes may contribute to increased sensitivity in diagnosis in a given population. The purpose of this study is to quantify the prevalence of atypical presentation, to identify its determinants, and to describe the presenting symptoms in cases of acute coronary syndrome at the emergency department of Hospital São João, Porto. Systematic sample of 288 emergency admissions with a confirmed diagnosis of acute coronary syndrome in 2007. Atypical presentation was defined as absence of chest pain and/or syncope. The prevalence of atypical presentation was 20.5% [95% confidence interval (CI): 16.0 to 25.5], with no important variation by gender. It increased with age and was more frequent in cases of ST-segment elevation myocardial infarction. In multivariate analysis, atypical presentation was associated with age [>70 versus ≤ 50 years, odds ratio (OR)=3.45; 95%CI: 1.03-11.61] and it was about four times less likely in the presence of history of ischemic heart disease, hypertension, dyslipidemia and smoking. A history of heart failure was independently associated with a higher likelihood of acute coronary syndrome with atypical presentation (OR = 4.15, 95%CI 1.50-11.46). Among the 223 cases who had chest pain or discomfort, a growing, oppressive, prolonged (longer than 30 minutes), recurrent and episodic pain prevailed. Among other symptoms, dyspnea was the most frequently reported, either as the main symptom in cases of atypical presentation or concurrently with typical symptoms. Factors associated with atypical presentation are consistent with those described in other populations. Using routine clinical data allowed access to a large data base on a representative sample of patients admitted to the emergency department of a third-level hospital that serves a large part of the local urban population. In medical records, data are unstandardized and heterogeneous in validity

  5. Atypical presentations of neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    Douglas Sato

    2011-10-01

    Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4 has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.

  6. Atypical human infections by animal trypanosomes.

    Directory of Open Access Journals (Sweden)

    Philippe Truc

    Full Text Available The two classical forms of human trypanosomoses are sleeping sickness due to Trypanosoma brucei gambiense or T. brucei rhodesiense, and Chagas disease due to T. cruzi. However, a number of atypical human infections caused by other T. species (or sub-species have been reported, namely due to T. brucei brucei, T. vivax, T. congolense, T. evansi, T. lewisi, and T. lewisi-like. These cases are reviewed here. Some infections were transient in nature, while others required treatments that were successful in most cases, although two cases were fatal. A recent case of infection due to T. evansi was related to a lack of apolipoprotein L-I, but T. lewisi infections were not related to immunosuppression or specific human genetic profiles. Out of 19 patients, eight were confirmed between 1974 and 2010, thanks to improved molecular techniques. However, the number of cases of atypical human trypanosomoses might be underestimated. Thus, improvement, evaluation of new diagnostic tests, and field investigations are required for detection and confirmation of these atypical cases.

  7. 'INDOTEST' in atypical hemicrania continua.

    Science.gov (United States)

    Baldacci, F; Nuti, A; Cafforio, G; Lucetti, C; Logi, C; Cipriani, G; Orlandi, G; Bonuccelli, U

    2008-03-01

    Hemicrania continua (HC) is an indomethacin-responsive headache characterized by a chronic, strictly unilateral, side-locked without side-shifting, persistent headache. We report three cases of HC with atypical features in which an acute administration of indomethacin 50 mg IM (INDOTEST) was performed. In all three cases INDOTEST predicted chronic responsiveness to indomethacin. Thus, in cases of HC with atypical features, INDOTEST could help for a correct diagnosis and therapy.

  8. Fluctuations of the Concentration of Cs-137 Aerosol in Chernobyl,Fukushima and Kawasaki

    Science.gov (United States)

    Ota, Yohei; Hatano, Yuko; Okada, Yukiko; Hirose, Katsumi

    2017-04-01

    Statistical analysis is applied to a time series of the airborne concentration of Cs-137. In order to extract fractal characteristics of the fluctuations, we employed the Hurst analysis. Interestingly, the Hurst index is around 1/3, which is common to the Chernobyl data, Fukushima data, and Kawasaki data. The Kawasaki data is measured by the Tokyo City University, located at 40km south to Tokyo. We proposed a stochastic differential equation, based on an advection equation with winds fluctuating probabilistically. The averaged solution of the equation is compared with measured data. We found that the index of the power of the time is -4/3, which is common to the three cases, Chernobyl, Fukushima and Kawasaki.

  9. Atypical Chronic Myelogenous Leukemia

    Science.gov (United States)

    ... myeloproliferative neoplasms, leukemia , and other conditions . Chronic Myelomonocytic Leukemia Key Points Chronic myelomonocytic leukemia is a disease ... chance of recovery) and treatment options. Chronic myelomonocytic leukemia is a disease in which too many myelocytes ...

  10. Reye's syndrome developing in an infant on treatment of Kawasaki syndrome.

    Science.gov (United States)

    Wei, Chih-Ming; Chen, Huey-Ling; Lee, Ping-Ing; Chen, Chong-Ming; Ma, Chun-Yi; Hwu, Wuh-Liang

    2005-01-01

    Aspirin is commonly used as an anti-inflammatory therapy for Kawasaki syndrome. Early initiation with high dose aspirin (80 to > 100 mg/kg per day), followed by low-dose therapy at the afebrile stage, has been often used to reduce morbidity and mortality in coronary complications. We report a 10-month-old infant who was diagnosed with Kawasaki syndrome. Sudden onset of poor activity, poor appetite, lethargy, tachycardia, tachypnea, hepatomegaly, increased AST/ALT, coagulopathy and hyperammonemia developed 3 days after the high-dose aspirin therapy. His histopathological and ultrastructural findings from the liver biopsy were compatible with Reye's syndrome. He recovered completely, and there was no recurrence.

  11. Spatial and Temporal Clustering of Kawasaki Syndrome Cases

    Science.gov (United States)

    Kao, Annie S.; Getis, Arthur; Brodine, Stephanie; Burns, Jane C.

    2010-01-01

    Background The etiology of Kawasaki syndrome (KS) remains unknown despite 30 years of intensive search for an agent. Epidemiologic clues to a possible infectious etiology include the seasonal distribution of cases, the previous occurrence of epidemics, the clinical features of the syndrome that mimic other infectious rash/fever illnesses in children, the self-limited nature of the illness, and the peak age incidence in the toddler years. Methods We examined the epidemiology and spatial and temporal distribution of KS cases in San Diego County, California during the 6-year period from 1998 to 2003. Clustering in space and time was analyzed using geo-referenced data with the K-function, the local G-statistic, and Knox statistic. Results A total of 318 patients were identified through active surveillance. The overall annual incidence was 21.7/100,000 in children <5 years, with rates in whites, white Hispanics, and Asian/Pacific Islanders of 15.3, 20.2, and 45.9/100,000, respectively. The Knox test showed significant clustering of cases within the space-time interval of 3 km and 3–5 days. Conclusions This is the first study of KS cases to use geo-referenced point pa