INTRODUCTION: Takayasu arteritis is the most common large-vessel vasculitis in childhood, but there is a lack of literature regarding childhood-onset Takayasu arteritis (c-TAK) in Southeast Asia. We aim to describe a c-TAK cohort in Singapore and highlight a unique subset that first presents with Kawasaki-like disease (KD). METHOD: A single-centre cohort study in Singapore of consecutive children diagnosed with c-TAK between 2002 and 2023 was performed. Demographic and clinical features, laboratory and angiographic findings, treatment, and outcomes were summarised. Disease activity was evaluated using the Paediatric Vasculitis Disease Activity Score and inflammatory markers. RESULTS: Twenty-three patients, fulfilling both the EULAR/ PRINTO/PReS and ACR/EULAR 2022 criteria, were recruited. The most common clinical features at diagnosis were fever (15, 65%) and neurological symptoms (11, 48%, half of which presented with stroke), while the most prevalent angiographic pattern by Hata's classification was Type V (21, 91%). Eight children (35%) initially presented with refractory KD, and these patients were significantly younger, more male-predominant, and had higher inflammatory markers at diagnosis; all of them had coronary artery involvement, but none had intracranial vascular findings. Of the entire cohort, 16 (70%) achieved inactive disease on medications with a median duration of 6 months (interquartile range [IQR]: 4-11), and 8 (35%) achieved remission off medications with a median duration of 43 months (IQR 35-60). CONCLUSION Our c-TAK cohort has high proportions of neurological involvement and stroke. This is also the first cohort study to describe a distinct group of patients who first presented with refractory KD.
Humans ; Takayasu Arteritis/complications* ; Singapore/epidemiology* ; Male ; Female ; Child ; Adolescent ; Age of Onset ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Cohort Studies ; Child, Preschool ; Fever/etiology* ; Stroke/epidemiology* ; Retrospective Studies

INTRODUCTION: Multisystem inflammatory syndrome in children (MIS-C) is a rare inflammatory syndrome with multisystem involvement affecting children exposed to COVID-19. This condition is rarely reported in East Asia and was not detected in Singapore until 2021. We present 12 cases of MIS-C diagnosed in KK Women's and Children's Hospital (KKH) from October 2021 to December 2021. METHOD: We conducted an observational study on cases fulfilling the Singapore Ministry of Health criteria for MIS-C from January 2020 to December 2021 in KKH. Medical records were reviewed to obtain information on clinical presentation, disease course, treatment received and outcomes. RESULTS: In the 12 cases detected, the median age was 7.50 years (interquartile range 4.00-9.25); 8 were male. All patients had mucocutaneous symptoms similar to Kawasaki disease. Other commonly involved systems were: haematological (coagulopathy 100%, lymphopaenia 91.70% and thrombocytopaenia 75.00%), gastrointestinal (75.00%) and cardiovascular (83.30%). Six patients (50.00%) had shock and were admitted to the intensive care unit. The majority of patients received treatment within 2 days of hospitalisation with intravenous immunoglobulin (IVIg) and steroids. All survived; the majority had normal echocardiograms and no long-term organ sequelae at 6 months post-discharge. CONCLUSION MIS-C emerged in Singapore as the incidence of COVID-19 in the community increased in 2021. The clinical presentation of our patients is similar to earlier reports, with some significant differences from Kawasaki disease. Multidisciplinary management, timely diagnosis, and early initiation of treatment with IVIg and steroids likely contributed to comparatively good outcomes. Our cases highlight the need for continued awareness of MIS-C among physicians, and surveillance of its incidence, short- and long-term outcomes.
Child ; Humans ; Female ; Male ; COVID-19/epidemiology* ; Mucocutaneous Lymph Node Syndrome ; Immunoglobulins, Intravenous/therapeutic use* ; Aftercare ; Singapore/epidemiology* ; Patient Discharge

INTRODUCTION: Kawasaki disease (KD) is a challenging diagnosis. Erythema and induration of the Bacillus Calmette-Guérin (BCG) site is increasingly recognised as a significant clinical clue. However, there is little data to support its specificity for KD as compared to other febrile illnesses. We aimed to evaluate BCG reaction or induration as a diagnostic tool for KD. METHODS: A retrospective case-controlled study of patients discharged with a diagnosis of KD from 2007 to 2010 was conducted. Another group of patients admitted over the same period for possible KD, but later found not to have KD, served as control. RESULTS: Significantly more infants with KD (69.7%) had BCG site changes than older children (27.8%; p < 0.001). It also presented earlier in the course of KD; < 5 days (53.3%) compared to ≥ 5 days of fever (30.0%; p < 0.001). Positive predictive value of BCG site reaction or induration for KD was 90.8% (95% confidence interval [CI] 0.819-0.962) for infants and 96.2% (95% CI 0.868-0.995) for older children. The prevalence rate of changes at the BCG site was 9.9% among patients with non-KD febrile illnesses and 42.6% among patients with KD. CONCLUSION BCG site reaction or induration is a useful clinical clue for the diagnosis of KD in both infants and older children, with a higher prevalence in infants. Physicians should consider KD in children with febrile illness and redness or crust formation at the BCG site, especially in view of low rates of BCG reaction or induration in non-KD febrile illnesses.
BCG Vaccine ; administration & dosage ; adverse effects ; Case-Control Studies ; Child, Preschool ; Erythema ; complications ; epidemiology ; Female ; Fever ; complications ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; epidemiology ; Risk Factors ; Singapore ; epidemiology

Epidemiologic studies of Kawasaki disease (KD) have shown a new pattern or change of its occurrence suggestive of its pathophysiology or risk factors from the first patient with KD reported in 1961. The incidence of KD in Northeast Asian countries including Japan, South Korea, China, and Taiwan is 10–30 times higher than that in the United States and Europe. Knowing the true epidemiology of KD in each country and the availability of publications of KD epidemiology also could benefit general health care providers and general population. This would enable the early detection and treatment of KD, ultimately reducing the incidence of coronary artery complications and mortality. Therefore, efforts to investigate the true epidemiology of KD should be continued in every country using a questionnaire survey, National Health Insurance system data, or combined methods depending on each country’s medical environment to ensure high-quality care of patients with KD.
Asian Continental Ancestry Group ; China ; Coronary Vessels ; Epidemiologic Studies ; Epidemiology ; Europe ; Health Personnel ; Humans ; Incidence ; Japan ; Korea ; Mortality ; Mucocutaneous Lymph Node Syndrome ; National Health Programs ; Risk Factors ; Seasons ; Surveys and Questionnaires ; Taiwan ; United States

The aim of this study is to assess the actual epidemiologic features of Kawasaki disease (KD) in Korea using the data from Health Insurance Review & Assessment Service (HIRA) claims from 2007 to 2014. We investigated HIRA claims of patients who had KD (International Classification of Diseases-10, M30.3) as a major diagnosis and were given intravenous immunoglobulin (IVIG) from 2007 to 2014. A total of 39,082 patients were reported during the period. The male-to-female ratio was 1.42 and the median age was 28 months. The incidence rates were 168.3 per 100,000 population aged 0 to 4 years in 2007, 159.1 in 2008, 167.3 in 2009, 190.4 in 2010, 188.2 in 2011, 190.2 in 2012, 210.4 in 2013 and 217.2 in 2014. These rates were much higher than those in the previous studies in Korea. KD occurred more often in early summer (May, June and July) and winter (December and January). The annual incidence rate of KD had been increasing every year, reaching 217.2 per 100,000 population aged 0 to 4 years in 2014. It is the second highest incidence rate of KD in the world after Japan.
Classification ; Diagnosis ; Epidemiologic Studies* ; Epidemiology ; Humans ; Immunoglobulins ; Incidence ; Insurance, Health* ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome*

Child ; Congresses as Topic ; Coronary Disease ; diagnosis ; etiology ; therapy ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Incidence ; Injections, Intravenous ; International Cooperation ; Mucocutaneous Lymph Node Syndrome ; epidemiology ; etiology ; therapy

BACKGROUNDThe epidemiologic pictures of Kawasaki disease (KD) in Shanghai from 1998 through 2002 were reported, while the current status of KD in the following five years remains unknown.

METHODSA questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited.

RESULTSIn total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 (mean 46.32 ± 6.51) per 100 000 children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of (27.32 ± 7.11) per 100 000, (t = 4.406, P = 0.002). Ages at onset ranged from 12 days to 13.6 years (median 1.8 years). It occurred more frequently in summer and spring. Coronary arterial lesions (CAL), defined as ectasia or aneurysm, accounted for 19.8% (232 cases). Flattened or inverted T wave was the most frequent finding (194 cases, 20.5%) by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases (86.6%). The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice.

CONCLUSIONSThe incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary sequelae.

Adolescent ; Age of Onset ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Mucocutaneous Lymph Node Syndrome ; epidemiology ; Surveys and Questionnaires

Kawasaki disease (KD) is an acute systemic febrile illness, predominantly affecting children less than the age of 5 years. After its first description in 1967 by Dr Tomisaku Kawasaki, KD has become the leading cause of acquired heart diseases in children in developed nations. KD has been reported in all racial and ethnic groups but the incidence of KD is more common among Asians, and the incidence of the disease varies considerably among different Asian countries. There is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. which suggests differences of race-specific susceptibility. In Korea, from 1990s, a nationwide epidemiologic study has been conducted every 3 years by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. So the average annual incidence, 105.0/100,000 children < 5 years in recent study (2003-2005) was the second highest reported rate in the world after Japan.
Asian Continental Ancestry Group ; Child ; Developed Countries ; Epidemiologic Studies ; Epidemiology ; Ethnic Groups ; Heart ; Heart Diseases ; Humans ; Incidence ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Prevalence

Kawasaki disease is an acute febrile, systemic vasculitic syndrome of an unknown etiology that primarily occurs in children younger than five years of age. The principal presentations of Kawasaki disease include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develops in 15% to 25% of untreated children with the disease, which may later lead to myocardial infarction, sudden death, or ischemic heart disease. Treatment with intravenous gamma globulin (IVIG) is effective, but the mode of action is still unclear. The development of a diagnostic test, a more specific therapy, and ultimately the prevention of this potentially fatal illness in children are all dependent upon the continued advances in determining the etiopathogenesis of this fascinating disorder.
Treatment Failure ; Prognosis ; Mucocutaneous Lymph Node Syndrome/*diagnosis/drug therapy/epidemiology/etiology ; Immunoglobulins, Intravenous/therapeutic use ; Humans ; Glucocorticoids/therapeutic use ; Child, Preschool ; Cardiovascular Diseases/diagnosis/drug therapy/etiology ; Aspirin/therapeutic use

PURPOSE: The aim of this study was to investigate the epidemiologic and clinical profiles of Kawasaki disease (KD) in children 8 years of age and older. METHODS: For the epidemiologic study of KD in Korea, data of total 15, 692 KD patients were collected from 1994 to 2002 on a 3 year basis, by the retrospective survey. Among them, data of 211 KD patients 8 years of age and older were analyzed and compared with those of the KD patients less than 8 years of age. RESULTS: 211 patients 8 years of age and older (1.3% of total KD patients) with the mean age of 9.8 years, included 142 cases aged 8-10 years, 42 cases aged 10-12 years, and 27 cases aged 12 years and older. The male-to-female ratio was 1.9: 1, the recurrence rate was 3.8%, and the prevalence of cases in sibling was zero. Coronary arterial (CA) abnormalities were detected by echocardiography in 25.4% of the cases, including 7.6% of CA aneurysm cases. There was 1 case complicated by myocardial infarction. CONCLUSION: Comparing with the data of the KD patients less than 8 years of age, data of 211 patients 8 years of age and older showed significantly higher incidences of CA abnormalities (25.4%: 19.8%) and CA aneurysms (7.6%: 4.0%).
Aneurysm ; Child* ; Coronary Aneurysm ; Echocardiography ; Epidemiologic Studies* ; Epidemiology ; Humans ; Incidence ; Korea ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Prevalence ; Recurrence ; Retrospective Studies ; Siblings