Beta-thalassaemia major is one of the commonest genetic disorders in South East Asia. The strategy for the community control of beta-thalassaemia major requires the characterisation of the spectrum of beta-globin gene mutations in any multi-ethnic population. There is only a single report of mutation analyses of the beta-globin gene in an isolated Kadazandusun community in Kota Belud, Sabah, Malaysia, which showed the presence of a common 45 kb deletion.
To analyse the self-reported degree of and factors influencing the compliance to desferrioxamine (DFO) therapy in children with transfusion-dependent thalassaemia major in Malaysia.
We aimed to determine the epidemiology and burden of rotavirus (RV) gastroenteritis (GE) in children requiring hospital care in an urban setting in Sabah, Malaysia. A prospective study of all patients younger than 12 years of age admitted with acute GE to Queen Elizabeth Hospital, Sabah, over a six-month period (October 2005 to March 2006) was conducted. During the study period, a total of 167 children with acute GE who had stool samples examined for RV were studied. RV accounted for 16% of all diarrhoeal cases, and 1.7% of all admissions to the children's wards during the study period. There was no difference in severity of GE between RV and non-RV groups. RV infection is a common cause of childhood GE requiring hospital care in Sabah.
This prospective, descriptive study was carried out to determine the pattern and the type of congenital heart disease seen in the Paediatric Departments in 4 government hospitals. The accessibility of surgical or transcatheter interventional treatment was also assessed. Consecutive new patients seen for suspected congenital heart disease from 1/1/95 till 31/12/95 (Group I) were registered. Records of patients seen from 1/1/95 to 31/8/95 (Group Ia) were reviewed 6 months after presentation to determine the nature of treatment received. Group II were cardiac patients who were first seen prior to the year 1995 and had undergone cardiac surgery or transcatheter interventional procedures in 1995. Of the 375 patients registered in the 4 hospitals, 250 were new patients and 125 were patients first seen prior to 1995 and had surgery or transcatheter interventional procedures in 1995. Of the 250 new patients, 83.2% had acyanotic cardiac lesions and 16.8% had cyanotic lesions. Ventricular septal defect was the commonest lesion, constituting 37.2%, followed by patent arterial duct (18.8%) and atrial septal defects (9.6%). At 6 month review, access to close-heart surgery or transcatheter interventional treatment were readily available. However, for patients with ventricular septal defects, 42.1% who required closure were still waiting for surgery 6 months after presentation. Of the 4 patients with Fallot's Tetralogy who required surgery, 2 had modified Blalock-Taussig shunt and 2 were awaiting surgery. In 1995, a total of 204 cardiac procedures were performed, there were 114 (55.9%) open heart procedures, 50 (24.5%) surgical ligation of the arterial duct, 28 (13.7%) modified Blalock-Taussig shunts, 11 (5.4%) transcatheter duct occlusion and 1 (0.5%) balloon valvuloplasty. The mean age of Fallot's Tetralogy repair was 6.4 years. No corrective surgery was performed for patients with complex cardiac lesions from the 4 hospitals in 1995. In conclusion, the pattern of congenital heart disease was similar to that seen world wide. Except for patent arterial duct, access to surgical treatment was inadequate.
INTRODUCTION: Mortality risk prediction scores are important for benchmarking quality of care in paediatric intensive care units (PICUs). We aimed to benchmark PICU outcomes at our hospital against the Pediatric Index of Mortality 2 (PIM2) mortality risk prediction score, and evaluate differences in diagnosis on admission and outcomes between Malaysian and immigrant children.
METHODS: We prospectively collected demographic and clinical data on paediatric medical patients admitted to the PICU of Sabah Women's and Children's Hospital in Kota Kinabalu, Sabah, Malaysia. The PIM2 risk score for mortality was tabulated.
RESULTS: Of the 131 patients who met the inclusion criteria, data was available for 115 patients. The mean age of the patients was 2.6 ± 3.8 years, with 79% of the cohort aged less than five years. Patients were mainly of Kadazan (38%) and Bajau (30%) descent, and 26% of patients were non-citizens. Leading diagnoses on admission were respiratory (37%), neurological (18%) and infectious (17%) disorders. Out of the 29 patients who died, 23 (79%) were Malaysians and the main mortality diagnostic categories were respiratory disorder (22%), septicaemia (22%), haemato-oncological disease (17%) and neurological disorder (13%). Calculated standardised mortality ratios (SMRs) were not significantly > 1 for any patient category for variables such as age and admission diagnosis. However, infants less than two years old with comorbidities were significantly worse (SMR 2.61, 95% confidence interval 1.02-6.66).
CONCLUSION: The patient profile at our centre was similar to that reported from other PICUs in Asia. The PIM2 score is a useful mortality risk prediction model for our population.