Self-reported level of and factors influencing the compliance to desferrioxamine therapy in multitransfused thalassaemias.

AIM: 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. METHODS: A cross-sectional study was conducted on all children with thalassaemia major on DFO attending Likas Hospital, Sabah, in September 2008. Patients or carer-providers were interviewed to report on the degree of compliance as either highly (administering >90% of DFO), moderately (51-90%), poorly (1%-50%) or non-compliant (0%) to DFO in the preceding month. The latest serum ferritin levels were noted. RESULTS: The median (range) age at first blood transfusion of these 139 (73 males, median (range) age at interview: 9.0 years (2-16 years)) patients were 1.0 year (2 months to 10 years). The median (range) duration of regular DFO therapy was 2 years (2 months to 10 years). Forty-three (31%) of the patients reported themselves to be highly compliant, 70 (50%) moderately and 26 (19%) poorly or non-compliant. Multivariate analysis showed that a lower family income negatively affected the degree of compliance, while family support positively affected the degree of compliance to DFO. No correlation existed between self-reported degree of compliance and latest serum ferritin level (6444 ± µmol/L; P = 0.186). CONCLUSION: The self-reported compliance to DFO therapy was moderate in this cohort of patients with thalassaemia major in Malaysia. The serum ferritin level was high, possibly because of the relatively short duration of DFO therapy and compliance. Oral iron chelator should be considered to improve the compliance to iron chelation.

Clinical characteristics and mortality risk prediction in critically ill children in Malaysian Borneo.

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.