The Clinical Practice Guidelines on Management of Neonatal Jaundice 2003 was updated by a multidisciplinary development group and approved by the Ministry of Health Malaysia in 2014. A systematic review of 13 clinical questions was conducted using evidence retrieved mainly from Medline and Cochrane databases. Critical appraisal was done using the Critical Appraisal Skills Programme checklist. Recommendations were formulated based on the accepted 103 evidences and tailored to local setting as stated below.

We report the biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency. Organic aciduria typical of this rare inborn error metabolism was found when the elder sibling presented with an episode of severe ketoacidosis at 20 months of age, which consisted of excessive excretion of ketones, tiglylglycine, 2-methyl-3-hydroxybutyrate, and 2-methylacetoacetate. Blood acylcarnitiness profile showed elevation of C5OH-carnitine, which represents 2-methyl-3-hydroxybutyrylcarnitine. A similar biochemical profile was identified in the younger sibling during screening although he had only mild clinical symptoms. Both patients reported a favourable outcome on follow-up.
2-methylacetoacetyl-coenzyme A ; deficiency ; Acetyl-CoA C-Acetyltransferase ; Biochemical ; Siblings

Introduction: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services. Methods: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out. Results: There were 315 patients analysed, 90 (28.6%) and 46 (14.6%) were neonates and adolescents respectively. The main ICD-10 diagnostic categories for all patients were identified to be ‘Congenital malformations, deformations and chromosomal abnormalities’ 117 (37.1%), ‘Diseases of nervous system’ 76 (24.1%) and ‘Neoplasms’ 60 (19.0%). At referral 156 (50%) patients had holistic needs assessments. Patients with ‘Diseases of nervous system’ were assessed to have significantly more physical needs than the other two diagnostic categories. Majority of patients who knew of their diagnosis and prognosis were those with malignancy. Over a fifth of referrals were at their terminal admission. Of 144 who died, 111 (77.1%) had advanced care plans. There was bereavement follow-up in 98 (68.1%) patients. Conclusion: Patients referred for palliative care have varied diagnoses and needs. To ensure all paediatricians are competent to deliver quality care to all children, further education and training initiatives is imperative.
Palliative Care