Delayed diagnosis of Kawasaki disease in Malaysia: Who is at risk and what is the outcome.

BACKGROUND: Data on the delayed diagnosis of (KD) is limited. This study aimed to determine the prevalence and trend of delayed diagnosis over time and identify the associated risk factors in Malaysia. METHODS: This retrospective, observational, population-based study involved all children with KD registered in Johor Kawasaki Clinical Registry over 10 years (January 2010 and December 2019). The prevalence of delayed diagnosis and its trend over time was calculated. Multivariable binary logistic regression was used to identify the independent risk factors for delayed diagnosis. RESULTS: There were 556 cases of KD, with 28% having incomplete criteria, 11% atypical presentation, while 10% developed a coronary aneurysm. The overall prevalence of delayed diagnosis was 9.9% (95% confidence interval (CI): 7.6-12.7%). There was a statistically significant decrease in delayed diagnosis over time (P = 0.008), with the latest rate of 4.6%. The majority of delayed diagnoses were due to failure to diagnose the disease during the initial consultation. Independent risk factors for delayed diagnosis were children older than 1 year, diagnosis before 2015, atypical presentation, and incomplete KD, with adjusted odds ratios (ORs) of 2.7, 2.3, 4.3, and 3.6, respectively. Compared to early diagnosis of KD, delayed diagnosis was significantly associated with coronary aneurysms (27.3% vs. 8.2%, P < 0.001, OR 4.2, [95% CI: 2.1-8.3]). CONCLUSIONS: One-tenth of cases of KD were diagnosed late, but it has improved over time. Children > 1 year, presenting with atypical presentation, and incomplete criteria are associated with late diagnosis.

Epidemiology, clinical characteristics, and immediate outcome of Kawasaki disease: a population-based study from a tropical country.

Data on Kawasaki disease from tropical countries are scarce. Hence, this population-based study aims to determine the epidemiology, clinical characteristics, and outcome of Kawasaki disease in children enrolled in the Kawasaki disease registry between 2006 and 2019 in Southern Malaysia. Diagnosis of Kawasaki disease was made using standard criteria. Primary outcome measure was a coronary artery aneurysm. Multivariable logistic regression was used to analyze the associated risk factors for coronary artery aneurysm. There were 661 Kawasaki disease, with 27% incomplete and 11% atypical presentations. Male-to-female ratio was 2:1, and median age of diagnosis was 1.4 years (interquartile range 9 to 32 months). Incidence in children of less than 5 years was 14.8 (95% confidence interval [CI]: 13.6 to 16.0) per 100,000 population, higher in males (19/100,000) and Chinese (22/100,000), with a gradual increase from 5.7/100,000 in 2006 to 19.6/100,000 in 2019, p < 0.001. Incidence in children between 5 and 9 years old was 1.3 (95% CI: 0.9 to 1.6) per 100,000 population and remained stable over time. There was a seasonal pattern with peak incidence during the rainy season. Out of 625 intravenous immunoglobulins (IVIG)-treated Kawasaki disease, 7.4% were resistant, and 9% had coronary artery aneurysms. Atypical presentation, male sex, late diagnosis, and IVIG resistance were independent risk factors for coronary artery aneurysms.Conclusions: Despite the tropical climate, Kawasaki disease epidemiology is similar to non-tropic regions with seasonal patterns and a rising incidence. Atypical presentation, male sex, late diagnosis, and IVIG resistance were significantly associated with coronary artery aneurysms. What is Known: • Kawasaki disease predominantly occurs in males, children less than 5 years old, and the Asian population. • Male sex, late diagnosis, incomplete Kawasaki disease, and IVIG resistance were associated with coronary artery aneurysms. What is New: • In multi-ethnic Asian countries such as Malaysia, ethnic Chinese have a higher prevalence of Kawasaki disease compared to other ethnicities. • Kawasaki disease with atypical presentation can occur in both complete and incomplete Kawasaki disease and is significantly associated with a coronary artery aneurysm.

Survival and associated risk factors for mortality among infants with persistent pulmonary hypertension of the newborn in Malaysia.

OBJECTIVE: This study aims to determine the immediate outcome of persistent pulmonary hypertension of the newborn (PPHN) and risk factors for mortality in the era of inhaled nitric oxide (iNO). STUDY DESIGN: This observational cross-sectional study includes 195 confirmed PPHN with a gestational age of ≥34 weeks without congenital heart disease. Multivariable logistic regression was used to identify risk factors for mortality. RESULTS: The mortality rate was 16.4%, with the highest mortality with pulmonary hypoplasia. Of 195, 65% received iNO; 18% were iNO non-responders with the majority having pulmonary hypoplasia. Independent risk factors for mortality were the presence of reversal of flow at the descending aorta, pulmonary hypoplasia, APGAR scores ≤ 5 at 5 min, and idiopathic PPHN with an adjusted odds ratio of 15.9, 7.5, 6.7, and 6.4, respectively. CONCLUSIONS: Despite the usage of iNO, mortality due to PPHN remains high and is related to etiology and cardiac function.

Unplanned PICU Readmission in a Middle-Income Country: Who Is at Risk and What Is the Outcome?

OBJECTIVES: To study the rate of unplanned PICU readmission, determine the risk factors and its impact on mortality. DESIGN: A single-center retrospective cross-sectional study. SETTING: Tertiary referral PICU in Johor, Malaysia. PATIENTS: All children admitted to the PICU over 8 years were included. Patients readmitted into PICU after the first PICU discharge during the hospitalization period were categorized into "early" (within 48 hr) and "late" (after 48 hr), and factors linked to the readmissions were identified. The mortality rate was determined and compared between no, early, and late readmission. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 2,834 patients in the study with 70 early and 113 late readmissions. Therefore, the rate of early and late PICU readmission was 2.5% (95% CI, 1.9-3.0%) and 3.9% (95% CI, 3.2-4.7%), respectively. The median length of stay of the second PICU admission for early and late readmissions was 2.7 days (interquartile range, 1.1-7.0 d) and 3.2 days (interquartile range, 1.2-7.5 d), respectively. The majority of early and late readmissions had a similar diagnosis with their first PICU admission. Multivariable multinomial logistic regression revealed a Pediatric Index Mortality 2 score of greater than or equal to 15, chronic cardiovascular condition, and oxygen supplement upon discharge as independent risk factors for early PICU readmission. Meanwhile, an infant of less than 1 year old, having cardiovascular, other congenital and genetic chronic conditions and being discharged between 8 AM and 5 PM was an independent risk factor for late readmission. There was no significant difference in the mortality rate of early (12.9%), late (13.3%), and no readmission (10.7%). CONCLUSIONS: Despite the lack of resources and expertise in lower- and middle-income countries, the rate and factors for PICU readmission are similar to those in high-income countries. However, PICU readmission has no statistically significant association with mortality.

The birth prevalence, severity, and temporal trends of congenital heart disease in the middle-income country: A population-based study.

OBJECTIVES: There is limited data on congenital heart disease (CHD) from the lower- and middle-income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time. DESIGN: A population-based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart disease for children. SETTING: State of Johor, Malaysia. PATIENTS: All children (0-12 years of age) born in the state of Johor between January 2006 and December 2015. INTERVENTION: None. OUTCOME MEASURE: The birth prevalence, severity, and temporal trend over time. RESULTS: There were 531,904 live births during the study period with 3557 new cases of CHD detected. Therefore, the birth prevalence of CHD was 6.7 per 1000 live births (LB) (95% confidence interval [CI]: 6.5-6.9). Of these, 38% were severe, 15% moderate, and 47% mild lesions. Hence, the birth prevalence of mild, moderate, and severe CHD was 3.2 (95% CI: 3.0-3.3), 0.9 (95% CI: 0.9- 1.1), and 2.6 (95% CI: 2.4-2.7) per 1000 LB, respectively. There was a significant increase in the birth prevalence of CHD, from 5.1/1000 LB in 2006 to 7.8/1000 LB in 2015 (P < .0001) due to increase in detection of both mild (1.9/1000 LB in 2006 to 3.9/1000 LB in 2015, P < .001) and severe CHD (1.8/1000 LB in 2005 to 2.9/1000 LB in 2015, P < .001). CONCLUSIONS: The birth prevalence of CHD was 6.7 per 1000 live births, and two in five were severe and significantly associated with syndrome and extracardiac defect. There was a significant increase in the detection of severe lesions in recent years leading to more burden to resources that are already limited in the middle-income country. Therefore, strategic and comprehensive pediatric and congenital heart surgery program is required.