Clinical manifestations and risk factors of shock in children with multisystem inflammatory syndrome.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a novel disease associated with COVID-19. The COVID-19 epidemic peaked in May 2022 in Taiwan, and we encountered our first case of MIS-C in late May 2022. We aimed to present patients' clinical manifestations and identify risk factors for shock. METHODS: We included patients diagnosed with MIS-C at two medical centers from May 2022 to August 2022. We separated those patients into two groups according to whether they experienced shock. We collected demographic, clinical manifestation, and laboratory data of the patients and performed statistical analysis between the two groups. RESULTS: We enrolled 28 patients, including 13 (46 %) with shock and 15 (54 %) without shock. The median age was 6.4 years (IQR: 1.9-7.5). In single variable analysis, patients with shock tended to be older, had more neurological symptoms, more conjunctivitis and strawberry tongue, lower lymphocyte count, lower platelet counts, and higher C-reactive protein, higher procalcitonin, higher ferritin, and higher D-dimer levels than those without shock. The area under the ROC curve that used procalcitonin to be the risk factor of shock with MIS-C was 0.815 (95 % CI 0.644 to 0.987). The cutoff value obtained by ROC analysis of procalcitonin was 1.68 ng/mL. With this cutoff, the test characteristics of procalcitonin were as follows: sensitivity 77 %, specificity 93 %, positive predictive value 91 %, negative predictive value 82 %. Multivariable analysis revealed that procalcitonin was the only independent risk factor of shock with MIS-C on admission (OR, 26.00, 95 % CI, 1.01-668.89). CONCLUSIONS: MIS-C patients with high initial procalcitonin levels have higher risks of experiencing shock and may need ICU admission.

Study on ECG in the Adolescent.

Normal ECG values in newborns, infants, and children have been collected and published. ECG in the adolescent, however, remains, to be collected and studied. The present study was designed and carried out to establish the normal ECG standards in male and female adolescents. A total of 898 school children and adolescents screened and examined as healthy were divided by age and sex into 6-9, 9-13, and 13-18 years age-groups. A 12 lead conventional ECG was recorded in 10 mm/mV and 25 mm/s, utilizing an automated Fukuda Denshi FCP-4301, MS-DOS/IBM-AT ECG machine. Lead V3R was not taken. Analog-to-digital conversion was performed by Fukuda signal acquisition module at a sampling rate of 500 Hz. The data on 69 ECG parameters were analyzed for the mean, standard deviation, 2nd to 98th percentiles, 95% confidence intervals, and sex difference. Normal values on 69 ECG parameters, sex-specific heart rate, P-QRS-T interval, duration, axis, wave amplitude, and calculated R/S amplitude ratio and ventricular activation time by age-group and sex were established. Male and female difference was noted in 49 (71.0%) parameters, of which 3 (6.1%) began in 6-9 years age-group, 30 (61.2%) began in 9-13 years age-group, and 16 (32.7%) in 13-18 years age-group. No sex difference occurred in 20 (29.0%) parameters. Normal male and female ECG standards on 69 ECG parameters in the adolescent were established. ECG sex difference began to appear the earliest at ages 6-9 years, and it occurred mostly at ages 9-13 years and 13-18 years, reflecting the anatomical and physiological consequences of puberty.

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

OBJECTIVE: 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. STUDY DESIGN: We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database. RESULTS: 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 < .001) and exhibited an increasing trend with birth year (from 2.28‰ for 2000 to 3.67‰ for 2009). The incidence ratio was 1.535 in boys and 1.055 in each increasing year. 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%. CONCLUSIONS: 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.

Preconception Counseling for Women with Congenital Heart Disease.

UNLABELLED: With advances that have been made over the recent decades in transcatheter and surgical interventions, most patients with congenital heart disease (CHD) can survive into adulthood. Overall, probably half of these surviving patients are female. When these female CHD patients reach childbearing age, however, pregnancy management will be a major issue. In order to meet the demands of fetal growth, the maternal cardiovascular system starts a series of adaptations beginning in early pregnancy. These adaptations include: decreased systemic and pulmonary vascular resistances, decreased blood pressure, expansion of the blood volume, increased heart rate and increased cardiac output. For women with CHD, this hemodynamic alteration may increase the risks of adverse cardiovascular events as well as the fetal and neonatal complications. Therefore, proper risk stratification and effective counseling for women with CHD who are planning their pregnancies is an important undertaking. KEY WORDS: Congenital heart disease; Pregnancy.

Epidemiology of Kawasaki disease: prevalence from national database and future trends projection by system dynamics modeling.

OBJECTIVE: To update the epidemiologic trend in Kawasaki disease (KD) and develop models for projection. STUDY DESIGN: From our national databases 2000-2010 and previous studies, we obtained the epidemiologic data to develop and validate system dynamics models. Population model incorporated birth rate and mortality. KD model incorporated the population at risk, incidence, and risk of coronary complications. RESULTS: The average annual incidence in age group <5, 5-10, 10-15, and 15-20 years was 67.3, 5.75, 0.79, and 0.26 per 100,000. The KD population was 23,349 and the model estimated 20,254 patients with KD, and 25% of these patients received medical care or continued surveillance in 2010. Projection up to 2030 suggests an average of 725 new patients with KD annually and a KD population of 35,006 by 2030. In 2030, 1469 patients with KD will need medical care for coronary complications. Simulation on the model modified to US data is also effective and suggests an average of 6200 new patients annually and KD population of 161,776 by 2030, and 5664 patients will need coronary care in 2030. By 2030, there will be 1 per 700 people in Taiwan and 1 per 1600 in the US with a history of KD. CONCLUSION: Simulations on our system dynamics models tailored to any epidemiologic and outcome variables and any changes with medical advance can dynamically project the futures.

Prevalence and the long-term coronary risks of patients with Kawasaki disease in a general population <40 years: a national database study.

BACKGROUND: Patients with Kawasaki disease (kDa) may develop coronary arterial lesions and subsequent coronary events. The first reported case in Taiwan was in 1976, and the annual incidence from 2003 to 2006 was 69/100 000 children < 5 years. A population study from Taiwan, a country with a high incidence of kDa, national health insurance, and easily accessible medical care, would adequately reflect the long-term risk. METHODS AND RESULTS: We retrieved the data of kDa patients from a national health insurance 2000 to 2010 database of Taiwan, a country with a child health index similar to those in the United States. The occurrence of coronary complications and interventions was identified by the respective International Classification of Diseases, Ninth Revision, codes. The prevalence of kDa in the population < 40 years was 34.9/100 000 (male/female ratio, 1.47). Coronary complications occurred in 1254 patients (5.37%; male/female ratio, 2.19), with an average annual risk of 2.4% (2.7% for males and 2.0% for females). An acute myocardial infarction occurred in 19 patients (0.08%; 18 males and 1 female), of whom one third were aged between 10 and 15 years (median, 15.7 years; range, 0.7-36.7 years). A coronary intervention was performed by catheterization in 18 patients (all males) at a median age of 24.5 years and by surgery in 10 patients (male/female ratio, 4.0) at a median age of 21.7 years, with mortality at discharge being 0% and 25%, respectively. CONCLUSIONS: This study estimated the overall prevalence of kDa (≈1/2940) in a population < 40 years. They, particularly the males, carry long-term coronary risks from a young age. Risk stratification for a timely coronary intervention and risk modification are mandatory.

Arterial switch operation for transposition of the great arteries: experience from 2000-2002 in Taiwan.

Arterial switch operation (ASO) is considered the procedure of choice for transposition of great arteries (TGA). The results and long-term prognosis improved with recent advances in perioperative management. We herein analyze the clinical outcome of patients undergoing ASO at our institution during the past 3 years. From 2000 to 2002, 44 patients (30 male and 14 female) of TGA received ASO. Age at operation varied from 4 days to 6.6 years (median 14 days) with body weight ranged from 2.25 kg to 18.1 kg (median 3.3 kg). Palliative procedure prior to ASO was performed in 8 patients (18.8%). Normal coronary artery pattern was found in 28 patients (63.6%). The early mortality was 11% (5/44). Only associated ventricular septal defect (VSD) was a significant predictor for operative mortality (p=0.012). With a follow-up ranged from 11 to 44 months, the gradient of neo-pulmonary artery stenosis was 16.5 +/- 18.2 mmHg. Four patients (10%) received balloon dilatation and the other three (7.7%) underwent reoperation. The gradient of neo-aortic stenosis was 16.5 +/- 18.2 mmHg that needed to be dilated in three patients (7.7%). The probability free from reintervention was 73% at the 3rd postoperative year. One patient had moderate degree of pulmonary valve regurgitation and six had moderate neo-aortic valve regurgitation. In conclusion, the ASO can be performed in infants with satisfactory results, even in those with a body weight less than 2.5 kg. Only associated VSD was shown to be a risk factor.