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Vasculitides
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Ivy M. Obonyo, Virginia A. Jones, Kayla A. Clark, Maria M. Tsoukas
Definition: Kawasaki disease (KD) is an acute febrile generalized vasculitis that most commonly affects children younger the age of 5 years. The exact origin is unknown, and in developed countries, KD is the primary cause of pediatric acquired heart disease.
Diagnostic Approach to Rash and Fever in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
Lee S. Engel, Charles V. Sanders, Fred A. Lopez
There are no specific or sensitive tests that can be used to diagnose KD. The diagnosis is made by clinical assessment of the criteria discussed earlier. The cause of KD is unknown; however, an infectious etiology is still being sought. Kawasaki disease has seasonal peaks in the winter and spring months, and focal epidemics occurred in the 1970s and 1980s [187].
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Kawasaki disease (KD) is the second commonest vasculitis of childhood. It has a world-wide distribution, with a male preponderance. UK incidence is 8 in 100,000 children aged under 5 years, while in Japan the incidence is 138 in 100,000. KD is the leading cause of childhood acquired heart disease in developed countries.
Plasma Cyclooxygenase-2 as a Potential Biomarker for Early Diagnosis of Kawasaki Disease
Published in Fetal and Pediatric Pathology, 2023
Kawasaki disease (KD) is a systemic vasculitis of medium and small-sized vessels characterized by febrile symptoms of unknown etiology [1]. KD mainly affects children under 5 years old, and can cause coronary artery lesions (CAL) including coronary artery dilatation, aneurysms, and giant aneurysms. In recent years, KD has become the major cause of acquired heart disease among pediatric diseases in countries with high medical standards [2]. The initial treatment for KD is a single high dose of intravenous immunoglobulin (IVIG) combined with acetylsalicylic acid (ASA), which is based on evidence from clinical trials. Approximately 10% to 20% of KD patients continue to have a fever at least 36 h after the initial treatment with IVIG, which is defined as IVIG resistant [3]. Numerous studies have demonstrated that patients who are resistant to standard IVIG treatment have a higher risk of developing CAL [1]. Biomarkers and better predictive models would help choose the appropriate initial treatment, preventing and reducing CAL.
Cyclophosphamide treatment for refractory atypical Kawasaki disease with giant coronary aneurysms: a case report
Published in Scandinavian Journal of Rheumatology, 2022
BY Yi, J Pick, J Votava-Smith, I Reyhan, S Wagner-Lees, J Szmuszkovicz
Kawasaki disease (KD) is a childhood vasculitis of unknown aetiology. It is the leading cause of acquired paediatric heart disease in developed countries, with coronary artery aneurysms (CAAs) occurring in up to 25% of untreated patients (1, 2). About 1% of these cases advance to giant CAAs (3). Giant aneurysms require lifelong treatment and are associated with significant morbidity. Initial treatment for KD traditionally includes intravenous immunoglobulin (IVIG) and aspirin (2). About 10–20% of KD patients are refractory to this therapy and continue to have fevers and elevated systemic inflammatory markers at least 36 h after IVIG treatment. These patients are identified to have refractory KD. Adjunctive therapies for refractory KD can include glucocorticoids, tumour necrosis factor-α (TNF-α) inhibitors, interleukin-1 (IL-1) blockade, calcineurin inhibitors, and statins (4). We present a refractory atypical KD case with rapid progression of giant CAAs despite aggressive additional anti-inflammatory therapies, ultimately responding to treatment with intravenous (IV) cyclophosphamide.
Fulminant acute liver failure as an unusual presentation of Kawasaki disease
Published in Scandinavian Journal of Rheumatology, 2021
G Anjani, R Deglurkar, RK Pilania, H Chaudhary, K Vaiphei, P Vignesh, S Singh
The classic criteria of KD do not include gastrointestinal manifestations. Liver involvement in KD has a varied spectrum, ranging from asymptomatic transaminitis to a severe cholestatic hepatitis that may sometimes occur as a result of hydrops of the gallbladder. Approximately 30% of children with KD manifest with elevated transaminase levels (3). Chen et al reported the case of a 10-year-old boy with persistent fever and hepatitis with signs of KD, who developed multiple coronary artery aneurysms despite standard therapy for KD (4). In some cases, cholestatic jaundice has also been reported (5, 6, 7). Out of 219 children with KD studied by Zulian et al, 10 children presented with acute abdomen, four of whom had jaundice (9). Of these 10 children, 50% developed coronary artery aneurysms despite appropriate therapy with IVIG. Hepatitis in the context of KD can be secondary to vasculitis, macrophage activation syndrome, drug-related aspects, or infections. Some viral infections can also trigger KD (8). In autopsy studies of KD, literature evidence for liver histopathology is scarce. However, the available literature suggests that lymphocytic or mixed interstitial infiltration of hepatic hilar regions and arteritis can be seen in liver histopathology in patients with KD (9). The liver failure in our patient is likely to be related to KD, as the work-up for hepatotropic infections was negative and the liver biopsy showed features of vasculitis.