Patients And Methods: Between March 1, 1995 and February 29, 1996, a multicentric prospective study was conducted in France in order to analyze the cardiovascular complications in Kawasaki syndrome, and to describe the echocardiographic features and the outcome of coronary lesions. Forty-nine cases of Kawasaki syndrome were observed.
Results: There were 32 boys and 17 girls (sex ratio: 1:9). The age at diagnosis was under one year in ten cases (20.4%), between one and five years in 27 cases (55.1%) and more than five years in 12 cases (24.5%). The complete diagnostic criteria were present in 42 cases (85.1%). Forty-five children (91.8%) were given intravenous immunoglobulin treatment but only 20 (40.8%) received this treatment within the seven days following the onset of the illness. Cardiovascular complications consisted of: pericardial effusion in 12 cases (24.5%), coronary dilation in seven cases (14.3%), coronary aneurysms of moderate size in seven cases (14.3%,) with hypokinetic left ventricle in two cases. No death was reported. All patients with coronary dilation and four patients with coronary aneurysms had a normal size of coronary arteries at echocardiography within the nine months of the follow-up. Among the three other patients, after a follow-up of two years, one still has a small coronary aneurysm and two have a normal size of coronary arteries.
Conclusion: Despite a delayed administration of immunoglobulin therapy in the majority of patients in this study, outcome of coronary lesions was favorable and severe cardiac complications were rare in the acute phase of the Kawasaki syndrome.
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http://dx.doi.org/10.1016/s0929-693x(00)00303-1 | DOI Listing |
Ther Adv Rare Dis
January 2025
Department of Pediatrics, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic hyperinflammatory syndrome, rarely associated with bone marrow failure (BMF). Telomere biology disorders (TBD) are caused by inherited defects in telomerase processes and can have heterogeneous presentations including idiopathic pulmonary fibrosis, cirrhosis, and BMF. We report a case of a 10-year-old male from Lima, Peru, who presented with HLH as the initial manifestation of a TBD.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Pediatric Emergency Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40139 Bologna, Italy.
The endothelium plays a key role in regulating vascular homeostasis by responding to a large spectrum of chemical and physical stimuli. Vasculitis is a group of inflammatory conditions affecting the vascular bed, and it is known that they are strongly linked to endothelial dysfunction (ED). Kawasaki disease (KD) is one childhood systemic vasculitis, and it represents the leading cause of acquired cardiac disease in children due to coronary damage and subsequent cardiovascular (CV) morbidity and mortality.
View Article and Find Full Text PDFKawasaki disease (KD) is a leading cause of acquired heart disease in children, often resulting in coronary artery complications such as dilation, aneurysms, and stenosis. While intravenous immunoglobulin (IVIG) is effective in reducing immunologic inflammation, 10-15% of patients do not respond to initial therapy, and some show resistance even after two consecutive treatments. Predicting which patients will not respond to these two IVIG treatments is crucial for guiding treatment strategies and improving outcomes.
View Article and Find Full Text PDFCureus
December 2024
Pediatric Cardiology, Children's Hospital at Montefiore, Bronx, USA.
Brugada syndrome (BrS) is a genetic channelopathy that may predispose to ventricular arrhythmia. It is inherited as an autosomal dominant pattern with incomplete penetrance. Fever can unmask Brugada syndrome in children who have a genetic predisposition.
View Article and Find Full Text PDFPLoS One
December 2024
Department of Computer Science and Engineering, National Sun Yat-sen University, Kaohsiung, Taiwan.
Kawasaki Disease (KD) is a rare febrile illness affecting infants and young children, potentially leading to coronary artery complications and, in severe cases, mortality if untreated. However, KD is frequently misdiagnosed as a common fever in clinical settings, and the inherent data imbalance further complicates accurate prediction when using traditional machine learning and statistical methods. This paper introduces two advanced approaches to address these challenges, enhancing prediction accuracy and generalizability.
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