Home About us Contact
|
Home About us Contact | ||
|
|
||
Kawasaki Disease (kawasaki + disease)
Selected AbstractsRefractory Progression of Coronary Aneurysms, a Case of Delayed Onset Kawasaki Disease as Depicted by Cardiac Computed Tomography AngiographyCONGENITAL HEART DISEASE, Issue 3 2010FACP, Shah Azmoon MD ABSTRACT Background., Kawasaki disease (KD) is an immune-mediated vasculitis of unknown etiology with self-limited clinical course that was first described in 1967 by Dr. Tomisaku Kawasaki. It is a disease of early childhood and rare past late adulthood but one that can have detrimental consequences when there is a delay in diagnosis and treatment. Cardiovascular complications causing increased morbidity and mortality may include coronary artery aneurysms, myocardial infarction, heart failure, arrhythmias, and peripheral artery occlusion. Case Presentation., Here, we present an atypical case of delayed onset KD in a young teenager. DS had visited three different emergency departments during the course of 2 weeks for unrelenting fevers. Despite multiple treatment protocols including immunoglobulin, steroids, and tumor necrosis factor-alpha antagonists, he continued to have progression of cardiovascular complications. While echocardiographic findings were suspicious for cardiac complications, a cardiac computed tomography (CT) angiography was able to clearly distinguish giant coronary aneurysms. Conclusion., Without prompt therapy, fever and manifestations of acute inflammation can last for several weeks to months with increased risk toward complications. The incidence of coronary artery aneurysms has been noted to be 25% in untreated patients with a mortality rate of up to 2%. Using low-dose protocols along with high spatial and temporal resolution of cardiac CT angiography may provide a useful and complimentary imaging modality in accurate diagnosis and follow-up of patients with KD. [source] Migraine and Raynaud Phenomenon: Possible Late Complications of Kawasaki DiseaseHEADACHE, Issue 3 2002Cris S. Constantinescu MD Migraine and Raynaud phenomenon often coexist and may reflect similar vascular reactions. Both have been associated with vascular endothelial cell dysfunction. Kawasaki disease is a systemic vasculitis of unknown etiology that affects children and may lead to the formation of coronary artery aneurysms. Endothelial cell dysfunction has been demonstrated late in Kawasaki disease and is not restricted to coronary vessels. We report the case of a patient who developed typical migraine with aura and Raynaud phenomenon at the age of 14, 12 years after onset of Kawasaki disease. His migraine responded well to pizotifen, and both migraine and Raynaud phenomenon improved after initiation of treatment with valproic acid. We postulate that both migraine and Raynaud phenomenon in this case represent late consequences of Kawasaki disease and result from extracoronary endothelial dysfunction. [source] Plaque Type Psoriasiform Eruption Following Kawasaki DiseasePEDIATRIC DERMATOLOGY, Issue 3 2007YOON SUN YOUNG M.D. No abstract is available for this article. [source] Kawasaki Disease with Facial Nerve ParalysisPEDIATRIC DERMATOLOGY, Issue 6 2003Margarita Larralde M.D., Ph.D. We describe an instance of facial nerve paralysis in a patient with KD. A 5-month-old boy developed fever, irritability, and diarrhea, treated 8 days later with cefaclor and ibuprofen. Three days later a confluent, erythematous and papular rash appeared, his lips were reddened and swollen, and his white blood count and platelet count were 20,900/mm3 and 558,000/mm3, respectively. He was admitted to the hospital with a diagnosis of KD, and an echocardiogram showed a right coronary aneurysm. The patient then developed an acute, right-sided, facial nerve peripheral paralysis that resolved over the next 6 weeks. He was treated with intravenous immune globulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with improvement of signs and symptoms. This report documents facial nerve paralysis as an uncommon complication of KD and points out that it may be a marker of increased risk of cardiovascular disease in this disorder. [source] Refractory Progression of Coronary Aneurysms, a Case of Delayed Onset Kawasaki Disease as Depicted by Cardiac Computed Tomography AngiographyCONGENITAL HEART DISEASE, Issue 3 2010FACP, Shah Azmoon MD ABSTRACT Background., Kawasaki disease (KD) is an immune-mediated vasculitis of unknown etiology with self-limited clinical course that was first described in 1967 by Dr. Tomisaku Kawasaki. It is a disease of early childhood and rare past late adulthood but one that can have detrimental consequences when there is a delay in diagnosis and treatment. Cardiovascular complications causing increased morbidity and mortality may include coronary artery aneurysms, myocardial infarction, heart failure, arrhythmias, and peripheral artery occlusion. Case Presentation., Here, we present an atypical case of delayed onset KD in a young teenager. DS had visited three different emergency departments during the course of 2 weeks for unrelenting fevers. Despite multiple treatment protocols including immunoglobulin, steroids, and tumor necrosis factor-alpha antagonists, he continued to have progression of cardiovascular complications. While echocardiographic findings were suspicious for cardiac complications, a cardiac computed tomography (CT) angiography was able to clearly distinguish giant coronary aneurysms. Conclusion., Without prompt therapy, fever and manifestations of acute inflammation can last for several weeks to months with increased risk toward complications. The incidence of coronary artery aneurysms has been noted to be 25% in untreated patients with a mortality rate of up to 2%. Using low-dose protocols along with high spatial and temporal resolution of cardiac CT angiography may provide a useful and complimentary imaging modality in accurate diagnosis and follow-up of patients with KD. [source] Migraine and Raynaud Phenomenon: Possible Late Complications of Kawasaki DiseaseHEADACHE, Issue 3 2002Cris S. Constantinescu MD Migraine and Raynaud phenomenon often coexist and may reflect similar vascular reactions. Both have been associated with vascular endothelial cell dysfunction. Kawasaki disease is a systemic vasculitis of unknown etiology that affects children and may lead to the formation of coronary artery aneurysms. Endothelial cell dysfunction has been demonstrated late in Kawasaki disease and is not restricted to coronary vessels. We report the case of a patient who developed typical migraine with aura and Raynaud phenomenon at the age of 14, 12 years after onset of Kawasaki disease. His migraine responded well to pizotifen, and both migraine and Raynaud phenomenon improved after initiation of treatment with valproic acid. We postulate that both migraine and Raynaud phenomenon in this case represent late consequences of Kawasaki disease and result from extracoronary endothelial dysfunction. [source] The liver as a crucial organ in the first line of host defense: the roles of Kupffer cells, natural killer (NK) cells and NK1.1 Ag+ T cells in T helper 1 immune responsesIMMUNOLOGICAL REVIEWS, Issue 1 2000Shuhji Seki Summary: The liver remains a hematopoietic organ after birth and can produce all leukocyte lineages from resident hematopoietic stem cells. Hepatocytes produce acute phase proteins and complement in bacterial infections. Liver Kupffer cells are activated by various bacterial stimuli, including bacterial lipopolysaccharide (LPS) and bacterial superantigens, and produce interleukin (IL)-12. IL-12 and other monokines (IL-18 etc.) produced by Kupffer cells activate liver natural killer (NK) cells and NK1.1 Ag+ T cells to produce interferon-g and thereby acquire cytotoxicity against tumors and microbe-infected cells. These liver leukocytes and the T helper 1 immune responses induced by them thus play a crucial role in the first line of defense against bacterial infections and hematogenous tumor metastases. However, if this defense system is inadequately activated, shock associated with multiple organ failure takes place. Activated liver NK1.1 Ag+ T cells and NK cells also cause hepatocyte injury. NK1.1 Ag+ T cells and another T-cell subset with an intermediate T-cell receptor, CD122+CD8+ T cells, can develop independently of thymic epithelial cells. Liver NK cells and NK1.1 Ag+ T cells physiologically develop in situ from their precursors, presumably due to bacterial antigens brought from the intestine via the portal vein. NK cells activated by bacterial superantigens or LPS are also probably involved in the vascular endothelial injury in Kawasaki disease. [source] Viral exanthems in childhood , infectious (direct) exanthems.JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 4 2009Part 1: Classic exanthems Summary Exanthems during childhood occur quite often and are mostly harmless in nature. Among different trigger factors, viruses are of prime importance. Viral exanthems may manifest as a macular, maculopapular, papular, urticarial or vesicular rash. Exanthems with other causes (bacterial toxins, drugs, autoimmune diseases) as well as those with unclear etiology such as unilateral lat-erothoracic exanthem or Kawasaki disease must be differentiated from viral exanthems. This review focuses on the classic viral exanthems. [source] Failure to distinguish systemic-onset juvenile idiopathic arthritis from incomplete Kawasaki disease in an infantJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 10 2007Haruki Komatsu Abstract: In an infant, an initial diagnosis of incomplete Kawasaki disease was made according to the American Heart Association guidelines. However, the diagnosis of systemic-onset juvenile idiopathic arthritis was established later. Physicians need to recognize that systemic-onset juvenile idiopathic arthritis can be mistaken for incomplete Kawasaki disease, even when the guidelines are used. [source] The diagnosis and management of Kawasaki diseaseJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2005J Royle Abstract:, Kawasaki disease (KD) is a systemic vasculitis of childhood with a predilection for the coronary arteries. It is the predominant cause of paediatric acquired heart disease in developed countries. The aetiology of KD remains unknown and consequently there is no diagnostic test. The diagnosis is made using a constellation of clinical criteria that in isolation have poor sensitivity and specificity. Early treatment prevents overt coronary artery damage in the majority of children. The long-term effects of childhood KD on later cardiovascular health remain unknown. A recent study showed that treatment of KD in Australia is suboptimal, with late diagnosis occurring in approximately half of the cases and an unacceptably high incidence of acute cardiac involvement. These guidelines highlight the difficulties in the diagnosis of KD and offer some clues that may assist early recognition of this important paediatric disease. They also detail current treatment recommendations and the evidence on which they are based. Increased awareness of the epidemiology and spectrum of the clinical presentation of KD is essential for early recognition and optimal management. [source] Acute Juvenile Pityriasis Rubra Pilaris: A Superantigen Mediated Disease?PEDIATRIC DERMATOLOGY, Issue 5 2001Isabel Betlloch M.D. Juvenile acute pityriasis rubra pilaris (JAPRP) is a form of pityriasis rubra pilaris (PRP) with particular clinical characteristics and course. It is usually preceded by an infectious condition. We report a boy, initially thought to have Kawasaki disease, and subsequently diagnosed as having JAPRP, who made a satisfactory recovery. In this case, prior antibiotic treatment made it impossible to show the presence of any microorganism. However, the clinical characteristics of these patients and the similarities with other disorders involving the skin that are mediated by superantigens led us to think that these antigens may be involved in the development of this disease. Also, in view of the obvious differences between JAPRP and the other PRP, we suggest that, in the future this disorder may be considered a separate entity as a reactive exanthem. [source] Inflammatory pulmonary nodules in Kawasaki diseasePEDIATRIC PULMONOLOGY, Issue 2 2003Alexandra F. Freeman MD Abstract Symptomatic pulmonary manifestations of Kawasaki disease (KD) are uncommon. However, epidemiologic, radiologic, and histologic studies have indicated that respiratory symptoms and findings occur in KD and suggest that the KD agent may have a respiratory portal of entry. We report on three young infants with KD who developed pulmonary nodules, in addition to coronary artery aneurysms. Two patients had pathologic specimens available, one from biopsy and the other from autopsy. The nodules had predominantly mononuclear cell infiltrates, which were within the lung parenchyma and infiltrating vessel walls. Immunohistochemical studies of the nodules, using antibodies to common leukocyte antigen (LCA) and factor VIII-related antigen, confirmed the inflammatory nature of the lesions and showed capillary proliferation. IgA plasma-cell infiltration was observed in the nodule, consistent with previous KD findings of IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract. The two patients with nonfatal KD were treated with intravenous immunoglobulin and aspirin, with resolution of the nodules. We propose that when pulmonary involvement occurs in KD, it ranges from subclinical interstitial micronodular infiltrates to larger inflammatory pulmonary nodules. These pulmonary infiltrates and nodules likely reflect the host response to the etiologic agent of KD, and may resolve with the disease process. Recognition of this pulmonary complication of KD may enable cautious observation of such lesions for spontaneous resolution. Pediatr Pulmonol. 2003; 36:102,106. © 2003 Wiley-Liss, Inc. [source] Repeated systematic surveillance of Kawasaki disease in Ontario from 1995 to 2006PEDIATRICS INTERNATIONAL, Issue 5 2010Yahui T. Lin Abstract Background:, Rising incidences of Kawasaki disease (KD) have been reported worldwide. Reported herein are the results of 4 triennial KD surveillances conducted in Ontario. Methods:, Between 1995 and 2006 all hospitals in Ontario were asked on 4 occasions to identify all patients with discharge diagnoses of KD and report incident cases. Results:, The latest surveillance identified 697 new KD patients (100% response rate) for a total of 2378 KD patients through all 4 surveillances. Yearly incidence was 26.2/100 000 for <5 years old, 6.7/100 000 for 5,9 years old and 0.9/100 000 for 10,14 years old. KD incidence significantly increased from 1995 to 2006, although the increase seemed to plateau between the 3rd and 4th surveillance. There was an increase in the proportion of patients diagnosed with incomplete KD and a significant reduction in the rate of coronary artery abnormalities, possibly due to better disease recognition and treatment. Hospitals reporting <20 cases per surveillance were found to be more likely to report cases with incomplete KD. These patients were also less likely to be treated with i.v. immunoglobulin and aspirin but were more likely to be treated with antibiotics, suggesting uncertainties regarding diagnosis and management of KD patients in those centers. Conclusions:, The incidence of KD in Ontario is possibly one of the highest outside of Asia and has been rising since 1995. Although the most recent surveillance demonstrated improved cardiac outcomes, treatment delays or absence thereof continue to be a problem. Effective diagnosis and prompt treatment remain critical aspects of KD management. [source] Original Article: A systematic review and meta-analysis of tumor necrosis factor ,-308 polymorphism and Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 4 2010Sakda Arj-Ong Abstract Background:, There have been genetic studies assessing the association between tumor necrosis factor (TNF)-,-308 and Kawasaki disease (KD) but the results have been conflicting due to lack of power. Therefore, a systematic review and meta-analysis was conducted to increase the power for identifying the association between the TNF-,-308 polymorphism and KD. Method:, Studies were identified from MEDLINE and EMBASE databases and were included if the subjects were children and the frequencies between TNF-,-308 and KD were reported. Data were pooled using a random effect model if heterogeneity between studies was present. Results:, Thirteen studies were identified however only six studies were included. The pooled prevalence of minor A allele was 5.2% (95% confidence interval [CI]: 0.1%,9.5%). Gene effect was assessed using per-allele and per-genotype approaches. The pooled odds ratio of G versus A with the random effect model was 1.13 (95%CI: 0.34,3.27). The genotype effects for GG versus GA+AA was estimated and the pooled odds ratio was 1.08 (95%CI: 0.42,2.92). Conclusion:, This review suggests a trend of association between the TNF-,-308 G-allele and KD. However, the gene effects are heterogeneous and assessing sources of heterogeneity are limited. An updated meta-analysis is needed if more studies are published. [source] Isolated abducens palsy in adolescent girl with Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 2 2010Baris Guven No abstract is available for this article. [source] Neutropenia in the acute phase of Kawasaki disease and prevention of coronary artery aneurysmPEDIATRICS INTERNATIONAL, Issue 4 2009Zenshiro Onouchi Abstract Background:, The significance of neutropenia in Kawasaki disease (KD) has not been fully elucidated as yet. Methods:, Subjects were retrospectively sampled from two clinical trials. These patients treated with aspirin alone (ASA) and PolyglobinN-Bayel (PolyN) given as i.v. immunoglobulin were categorized as ASA-early (n = 0), ASA-late (n = 8), PolyN-early (n = 18), or PolyN-late (n = 27) based on the therapy administered and the incidence of neutropenia before the 10th day of illness (DI) and after 11 DI. Data regarding the time of onset of neutropenia, and incidence of coronary artery lesion (CAL) formation were obtained. P < 0.05 was considered statistically significant. Results:, No patients in the ASA group exhibited neutropenia within 10 DI. The time of onset of neutropenia in the PolyN-early group was 8 ± 1.3 DI. That in the PolyN-late group (19.8 ± 8 DI) was earlier than in the ASA-late group (26.6 ± 14 DI; P < 0.025). PolyN-early patients had a lower incidence of CAL formation than ASA-non patients (patients without neutropenia in the ASA group; P = 0.00019) and ASA-late patients (P = 0.04). That in the PolyN-early group tended to be lower than in the PolyN-late group (P < 0.1). Conclusion:, Early neutropenia indicated that circulating neutrophils within 10 DI may play an indispensable role in the following sequence to CAL formation in KD. [source] Acute myocardial infarction after Kawasaki disease in an infant: Treatment with coronary artery bypass graftingPEDIATRICS INTERNATIONAL, Issue 3 2009Etsuko Tsuda First page of article [source] Re-treatment regimens for acute stage of Kawasaki disease patients who failed to respond to initial intravenous immunoglobulin therapy: Analysis from the 17th nationwide surveyPEDIATRICS INTERNATIONAL, Issue 4 2007RITEI UEHARA Abstract Background: Current regimens for re-treatment of patients with Kawasaki disease who failed to respond to the initial intravenous immunoglobulin (IVIG) therapy are still uncertain. The purpose of this study is to reveal what regimens were used as the initial therapy and re-treatment for acute stage of Kawasaki disease in the current Japanese medical setting. Methods: The 17th nationwide survey on Kawasaki disease covered patients whose onset was in 2001 and 2002. In questionnaires sent to all hospitals with a bed capacity of 100 or more and a pediatric department, several questions related to therapeutic regimens for Kawasaki disease were posed. The authors observed the proportions of hospitals that had regimens for patients who failed to respond to the initial therapy. Results: Among those hospitals that responded to the survey, 1052 (64.1%) reported that at least one patient with Kawasaki disease visited the hospital. Among these 1052 hospitals, 73.3% had a regimen to administer 30,39 mg/kg per day of oral aspirin with initial IVIG. The proportion of hospitals that used 1 g/kg per day of IVIG for 2 days was the largest among the options for the initial treatment. For those patients who fail to respond to the initial therapy, 464 hospitals (44.1%) reported that their pediatricians would use additional IVIG only. The number of hospitals that planned to administer high-dose IVIG and ulinastatin was 185 (17.6%). The number of hospitals having regimens of additional IVIG and steroids was 54 (5.1%). Conclusions: The current status of the treatment for patients with Kawasaki disease not responding to the initial IVIG therapy in Japan was revealed. A randomized trial of a large sample is needed to ascertain the effectiveness of several options for re-treating Kawasaki disease. [source] Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999,2002PEDIATRICS INTERNATIONAL, Issue 4 2006HIROSHI YANAGAWA Abstract Objective: The purpose of the present study was to describe the results of nationwide epidemiologic surveys of Kawasaki disease for the 4 year period 1999,2002. Methods: The design is a retrospective incidence survey. The patients reported in these two surveys are all new patients who were reported during the two study periods (1999,2000 and 2001,2002), although the data were collected retrospectively. A questionnaire was sent to all pediatric departments of hospitals with 100 beds or more throughout Japan, requesting data on patients with Kawasaki disease, such as sex, age, date of first hospital visit, recurrence, and cardiac lesions. Results: The total number of patients reported in the 4 year period 1999,2002 was 32 266 (18 604 male, 13 662 female), with an average annual incidence of 137.7 per 100 000 children younger than 5 years old. The male/female ratio was 1.30. The incidence peaked at 9,11 months of age, and the proportion of patients under 1 year of age was 26%. The monthly distribution had a high peak in January and a gradual increase in summer. Geographically, the high-incidence areas were limited to certain prefectures and moved from year to year. The cardiac lesions at acute stage and cardiac sequelae occurred more in children under 1 year and older than 4 years. Among the principal symptoms, fever persisting ,5 days occurred most commonly, followed by conjunctival congestion, changes in lips and oral cavity, polymorphous exanthema, and changes of extremities. Cervical lymphadenopathy occurred less. Conclusion: More than 32 000 patients with Kawasaki disease during the 4 year period 1999,2002 were reported to the nationwide incidence surveys. The number of patients is steadily increasing despite the decrease of children. The seasonal variation, geographical distribution, and age-specific distribution support the infection theory for the etiology of Kawasaki disease. [source] Exercise- or dipyridamole-loaded QGS is useful to evaluate myocardial ischemia and viability in the patients with a history of Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 5 2005Yuichi Ishikawa AbstractBackground:,Evaluation of myocardial ischemia and viability is very important for the management of patients with a history of Kawasaki disease (KD). 99mTc-tetrofosmin myocardial perfusion scintigraphy combined with quantitative gated single photon computed emission tomography (QGS) gives us information, not only about perfusion, but also the percentage change in left ventricular wall thickness (%WT) and relative changes in left ventricular wall motion (LVM). Methods:,The subjects were 27 patients with a history of KD followed as outpatients at the National Cardiovascular Center, Osaka, Japan. Exercise-loaded QGS was performed on 21 patients, and dipyridamole- loaded QGS was performed in six patients younger than 7 years old. Results:,Perfusion defects (PD) were observed in 12 patients. Of the 12 patients, four with old myocardial infarction (OMI) had decreased %WT. All patients with OMI showed a decrease in %WT in the areas where PD was seen on the image. The other eight patients without OMI showed no decrease in %WT. In non-infarcted cases, the %WT was normal in the PD-positive area. Conclusions:,It is possible to evaluate myocardial ischemia and viability in KD patients by comparing PD on the image with %WT determined by QGS using exercise or drug-loaded myocardial scintigraphy alone. [source] Absence of hypercoagulability in acute Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 2 2005Ming-Tsan Lin AbstractBackground:,Kawasaki disease (KD) is a systemic vasculitis syndrome with the striking feature of cardiovascular involvement. Endothelial cell (EC) damage has been suggested to predispose individuals to the development of coronary vascular disorders. When EC are perturbed, prethrombotic complications ensue. The purpose of this study was to examine the clinical relevance of EC activation and hypercoagulability in the pathogenesis of KD and to determine if plasma levels of these markers are correlated with the development of coronary aneurysms. Methods:,EC function and coagulation status were assessed in 52 patients with acute KD, 20 febrile control subjects, and 20 healthy control subjects. Biological markers of EC and hypercoagulability were measured and included thrombomodulin, tissue factor, tissue factor pathway inhibitor, von Willebrand factor (vWF), coagulation factor VII (FVII), activated factor VII, prothrombin fragment 1 + 2 (F1 + 2), and D-dimer. Results:,Transient dilatation of coronary arteries was the most common complication (55.8%), and coronary aneurysm was noted in five patients (9.6%). Levels of vWF, FVII, F1 + 2 and D-dimer were higher in acute KD patients compared with healthy controls but not febrile controls. Markers of EC and hypercoagulability were not different between patients with cardiac complications and those without cardiac complications. Biological and immunological assays did not demonstrate the prethrombotic state in acute KD. Conclusions:,Our results suggest that hypercoagulability does not occur during the acute stage of KD. Markers of EC damage and hypercoagulability are not predictive of coronary aneurysms in KD. [source] Spontaneous platelet aggregation in Kawasaki disease using the particle counting methodPEDIATRICS INTERNATIONAL, Issue 6 2003Masashi Taki AbstractBackground:,Platelet aggregation is generally measured by the optical density method. This method is not very sensitive in detecting platelet activation because of the poor correlation between the formation of platelet aggregates and light transmission, and inability to detect small platelet aggregates. Recently, a new method was developed that detects small platelet aggregates formed in the early phase of platelet aggregation by means of a particle counting technique using light scattering. Methods:,The present study used the particle counting method to measure spontaneous platelet aggregation (SPA) produced by agitation with a stirrer bar without agonists in patients with Kawasaki disease (KD) and children's thrombotic disease, and compared the results with the conventional optical density method. Results:,Using the particle counting method, significantly higher SPA rates were found before treatment than in healthy adults and at the other stages of treatment. A similar tendency was noted with the optical density method; however, the positive rate of SPA was significantly higher with the particle counting method in each stage except the second stage, which was the period from the start of gamma-globulin therapy to 20 days. Conclusion:,These results indicate that enhanced platelet aggregation is noted at a high frequency before treatment of KD and that the particle counting method is superior to the optical density method in detecting enhanced platelet aggregation. [source] Evaluation of 80 children with prolonged feverPEDIATRICS INTERNATIONAL, Issue 5 2003Ozgur Cogulu Abstract Background:,Several studies have been published regarding the etiology and evaluation of a child with prolonged fever, however, the reasons for the prolonged fever have changed during the years. The present study aims to determine the causes of prolonged fever, to investigate the relationship of fever using some basic laboratory tests, and to establish guidelines for the approach in those children. Methods:,The charts of 80 out of 17 490 hospitalized children who were seen between 1996 and 2001 with prolonged fever of longer than 2 weeks and unknown origin were reviewed in the university hospital of Izmir, Turkey. Their charts were evaluated in respect of age, sex, growth curves, educational level of their families, the duration and the magnitude of fever, causes of fever, and basic laboratory investigations such as white blood cell, blood smear, hemoglobin, erythrocyte sedimentation rate, and C-reactive protein. Results:,Forty-four (55.00%) were boys and 36 (45.00%) were girls. Forty-four children (55.00%) were aged between 1 month and 2 years, 21 (26.25%) were aged 3,6 years, seven (8.75%) were aged 7,10 years, and eight (10.00%) were older than 10 years. The mean age was 3.87 ± 4.17 years (range 3 months,17 years). Forty-six children (57.50%) had a prolonged fever that had lasted from 15,30 days, 18 (22.50%) from 31,60 days, and 16 (20.00%) had fever lasting more than 60 days. Final diagnosis had been reached in 70 of the 80 children (87.50%). The most common causes were infection (47/80), followed by immune deficiency (6/80), collagen tissue disorder (5/80), neoplasia (2/80), and miscellaneous (10/80) such as central fever in three, diabetes insipidus in two, familial Mediterranean fever in two, Kawasaki disease, foreign body in the respiratory system, and Crohn disease in one patient each. Among the laboratory tests white blood cell count, hemoglobin level and blood smear distribution of infection group were statistically significant. Conclusions:,The most common cause of fever of unknown origin remains infection. The proportion of collagen tissue disorders and neoplasia have been found to be decreased. Unusual reasons such as diabetes insipidus and foreign body in the respiratory system in the miscellaneous group have been detected. Age plays important role in the diagnosis of prolonged fever, while some basic laboratory tests might give clues in the evaluation and may suggest a diagnosis. [source] Inducible and endothelial constitutive nitric oxide synthase gene polymorphisms in Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 2 2003Vahid Khajoee AbstractBackground: Nitric oxide (NO) is secreted by immune and vascular endothelial cells, and appears to play important roles in the pathophysiology of Kawasaki disease (KD). Thus, genetic variations in NO synthase (NOS) genes may be involved in the development of coronary artery lesions (CAL) in KD. Methods: The present study investigated the association of endothelial constitutive NOS (ecNOS) and inducible NOS (iNOS) gene polymorphisms with the development of CAL in KD in a Japanese population. Results: The genotype distributions of 27-bp tandem repeat polymorphism within intron 4 of ecNOS gene did not show any significant difference between controls and KD patients with or without CAL. In addition, there was no significant association between whole-allele distribution of iNOS gene promoter (penta-repeat CCTTT) polymorphism and KD with or without CAL. Conclusion: These results did not support any association of ecNOS and iNOS gene polymorphisms to the development of CAL in KD patients in a Japanese population. [source] Severely damaged aortic valve and cardiogenic shock in an infant with Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 1 2003SHIGETO FUSE First page of article [source] Patients diagnosed with Kawasaki disease before the fifth day of illness have a higher risk of coronary artery aneurysmPEDIATRICS INTERNATIONAL, Issue 4 2002Yuichi Nomura Abstract Background: A fever lasting for at least 5 days is an essential characteristic of the original diagnostic criteria ofKawasaki disease (KD). However, it is not difficult for an experienced physician to confirm the diagnosis of KD before the fifth day offever. The aim of this study is to investigate the effect of intravenous gamma globulin therapy (IVGG) in KD initiated before the fifth day of illness. Methods: A total of 125 patients treated with IVGG were divided into group A (IVGG was initiated before the fifth dayof illness, n= 46) and group B (IVGG was initiated at the fifth day or after, n= 79). Patients' characteristics,laboratory findings, treatments and outcomes were compared between the groups. Results: White blood cell count value, C-reactive protein and Harada's score showed no difference between thegroups. A significantly higher average value of alanine aminotransferase (ALT) was observed in group A. Although the treatments were identicalin both groups, the average duration of fever from the initial day of IVGG in group A was significantly longer than in group B. Theincidence of aneurysm in group A was significantly higher than that in group B. Stepwise regression analysis using aneurysm as a dependentvariable revealed that group A and ALT were significant. Conclusions: Patients diagnosed with KD before the fifth day of illness showed a poor response to IVGG. This observationmight be related to high ALT values. Further examination concerning the modification of treatment in such patients is necessary. [source] Increased production of serum IgA-class antibody to lipid A in Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 1 2002Seiichiro Takeshita Abstract Background:,The etiology of Kawasaki disease (KD) remains unknown. To investigate whether a conventional bacterial antigen is involved in the pathogenesis of KD, we studied the serum response to lipopolysaccharide (LPS). Methods:,We measured the serum levels of IgG-, IgM- and IgA-class antibodies (Ab) to lipid A, a toxic site of LPS, using enzyme-linked immunosorbent assay in 20 patients with KD, 11 patients with Gram-negative bacterial infection (GNBI), 27 healthy children and 12 healthy adults. Results:,The serum levels of anti-lipid A IgG, IgM and IgA tended to increase with advancing age in healthy children older than 6 months of age. The mean level of anti-lipid A IgM in the acute phase of GNBI and the mean levels of anti-lipid A IgM and IgA in the acute phase of KD were found to increase significantly, in comparison to the age-matched controls. Furthermore, the mean level of anti-lipid A IgA also showed a significant increase from the acute to the subacute phases of KD. Regarding the IgA-subclass response, higher titers of anti-lipid A specific Ab were seen in the IgA2 subclass than in the IgA1 subclass. Conclusion:,These findings indicate that KD patients demonstrate an intense response to lipid A in the IgA, especially IgA2-subclass, thus suggesting that an unusual activation of the mucosal immune response to a ubiquitous antigen derived from Gram-negative bacteria may be involved in the pathogenesis of KD. [source] Longitudinal estimation of signal-averaged electrocardiograms in patients with Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 1 2002Yukio Kuramochi Abstract Background:,Myocarditis associated with Kawasaki (KD) disease is prominent, but rarely detected by conventional methods. The hypothesis of this study is to see if signal-averaged electrocardiogram can detect myocarditis with KD. Methods:,We obtained signal-averaged electrocardiograms from 71 patients with KD (mean age 2.8 ± 2.9 years) in the acute (1st,4th week), subacute (5,7th week), and chronic (8th week or later) phases (mean study period 3.5 ± 1.7 years). Sixteen patients who had pericardial effusion, bundle branch block or myocardial ischemia were excluded from this study. The results were compared with those of Holter and 12-lead electrocardiograms, echocardiography and serum myocardial enzymes. They were also contrasted with the course of each patient. Results:,The incidence of abnormal findings on signal-averaged electrocardiogram was 18.2% in the acute phase versus 10.9% in the subacute and chronic phases. It differed significantly higher than the other conventional tests (P < 0.05). Four patients had abnormalities of signal-averaged electrocardiograms through all three phases. Among these four, two had reduced left ventricular contractility. However, these changes were transient and resolved in the subacute phase. All patients had good courses and no residue. Conclusion:,This study shows the possibility that signal-averaged electrocardiogram is more useful to detect myocarditis associated with KD than the other conventional tests. However, we could not define the prognostic value of abnormal signal-averaged electrocardiograms during this study period. [source] Dobutamine stress surface mapping of myocardial ischemia in Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 3 2001Nobuyuki Takechi Abstract Background: To detect and localize myocardial ischemia, a method that does not require physical exertion is sometimes needed in children with Kawasaki disease. Methods: Dobutamine stress body surface mapping was performed in 115 children with a history of Kawasaki disease (58 without coronary artery lesions, 40 who had coronary lesions without myocardial ischemia and 17 with myocardial ischemia). The maximum infusion rate of dobutamine was 30 ,g/kg per min. Myocardial ischemia was diagnosed by the presence of an area of hypoperfusion on scintigraphy at rest and/or an increase in hypoperfusion during a dobutamine stress test compared with resting scintigraphy. We studied the number of leads that showed significant ST depression on the isopotential map (nST), the number of the row containing the lead with the smallest negative value on the isointegral map (Imin), and the localization of myocardial ischemia on the isointegral map. Based on findings in patients without coronary artery lesions, we defined the criteria for detecting myocardial ischemia as nST , 1 and Imin, 4. Results: The sensitivity of detecting myocardial ischemia was 94.1% using nST and 41.7% using Imin, while the specificity of these methods was 98.9 and 96.9%, respectively. The localization of myocardial ischemia on stress body surface mapping was 100% concordant with that determined by stress myocardial scintigraphy. Conclusions: Dobutamine stress body surface mapping for the detection of myocardial ischemia is a non-invasive, more convenient and repeatable test compared with exercise myocardial scintigraphy and it is a more objective test compared with exercise echocardiography. Dobutamine stress body surface mapping is useful for the identification and localization of silent myocardial ischemia in pediatric patients with Kawasaki disease, especially those who cannot perform tests involving physical exercise. [source] Coronary risks after high-dose ,-globulin in children with Kawasaki diseasePEDIATRICS INTERNATIONAL, Issue 5 2000Yoshiyuki Morikawa Abstract Objectives: The goals of the present study were to develop a predictive coronary risk scoring system after intravenous ,-globulin (IVGG) therapy of any dose for the different preparations currently used in the treatment of children with Kawasaki disease and to determine the predictive value of the system. The previously reported scoring systems were based on treatment with high-dose IVGG therapy at limited doses and were determined using investigative methods. Methods: Four hundred and fifty-one patients were randomized into one of three groups and received either i.v. polyethylene glycol-treated human immunoglobulin at a dose of either 200 (n=147) or 400 mg/kg per day (n=152) or freeze-dried sulfonated human immunoglobulin at 200 mg/kg per day (n=152) for 5 consecutive days. We documented 31 cases of coronary abnormalities (CA). Univariate and multivariate logistic regression was performed using 49 clinical variables and the resulting predictive model was validated. Results: The duration of fever (odds (1 day)/odds (, 5 days)=0.158; 95% confidence interval (CI) 0.0385,0.648), hemoglobin (odds (Q1=10.3)/odds (Q3=11.6) = 3.97; 95% CI 1.92,8.20), IgG (odds (Q1=1900)/odds (Q3=2658)=2.72, 95% CI 1.18,6.25) and IgA (odds (Q1=72)/odds (Q3=160) =0.415; 95% CI 0.253,0.680) levels after completion of ,-globulin infusion were independent predictors. The model is quasi-cross validated and has acceptable sensitivity and selectivity. The estimated risk and observed occurence of CA coincide. Conclusions: Determinants of the risk of CA after IVGG therapy are a longer duration of fever, a lower IgG level, a higher IgA level and a lower hemoglobin level after IVGG infusion. This model is applicable for IVGG doses from 1 to 2 g/kg and for at least two different ,-globulin preparations. [source] | ||||||||||||||||||||||||||||