METHODS: The inclusion criteria were normal term neonates (gestation ≥ 37 weeks). Parents/care-givers were interviewed to obtain data on demography, clinical problems, feeding practice and age when first TSB was measured. Polymerase chain reaction-restriction fragment length polymorphism method was used to detect common G6PD, UGT1A1 and SLCO1B1 variants on each neonate's dry blood specimens.
RESULTS: Of 1121 jaundiced neonates recruited, 232 had SNH. Logistic regression analysis showed that age (in days) when first TSB was measured [adjusted odds ratio (aOR) = 1.395; 95% confidence interval (CI) 1.094-1.779], age (in days) of admission (aOR = 1.127; 95% CI 1.007-1.260) and genetic mutant UGT1A1 promoter A(TA)7TAA (aOR = 4.900; 95% CI 3.103-7.739), UGT1A1 c.686C>A (aOR = 6.095; 95% CI 1.549-23.985), SLCO1B1 c.388G>A (aOR = 1.807; 95% CI 1.242-2.629) and G6PD variants and/or abnormal G6PD screening test (aOR = 2.077; 95% CI 1.025-4.209) were significantly associated with SNH.
CONCLUSION: Genetic predisposition, and delayed measuring first TSB and commencing phototherapy increased risk of SNH.
METHODS: This population-based and observational cohort study reviewed pediatric RHD patients (0-18 years) from the Sabah Pediatric Rheumatic Heart Registry from 2015 till 2018.
RESULTS: A total of 188 RHD were reviewed with 120 new cases. The incidence of RHD is 2.19 [95% confidence interval (CI): 1.83-2.61] per 100 000 population, with a rising trend over time. Meanwhile, the prevalence of RHD was 13.78 (95% CI: 11.92-15.86) per 100 000 pediatric population. The majority of patients were from indigenous groups (59.0%), male (56.4%) with a mean age of 14.3 (3.31) years. About 77.9% had the lowest household income, and a significant proportion lived in overcrowded conditions. At diagnosis, 59% were diagnosed with severe RHD. There is heightened risk but no statistical significance between modifiable factors (low weight and height percentile at diagnosis, lowest income group, renting a house, overcrowding and healthcare access of more than 5 km) with severe RHD. Severe RHD is significantly associated with risk for intervention (p = 0.016). Sixteen (13.8%) patients required surgical intervention. About 97.6% of patients were on intramuscular benzathine penicillin G with 84.5% compliance.
CONCLUSION: The rising prevalence and incidence of pediatric RHD in Sabah, with the most being severe RHD at diagnosis, necessitates the development of an echocardiographic screening and a comprehensive national disease program.