Kanij Fatema
Associate Professor, Department of Pediatric Neurology , BSMMU, Dhaka, Bangladesh.
Title: Neurometabolic Disease Pattern In Bangladesh: Experience Sharing In Developing Country Perspective
Biography:
Kanij Fatema is a Associate Professor in Bangabandhu Sheikh Mujib Medical University. she has Published her research work in many reputed journals.
Abstract:
Inborn errors of metabolism (IEM) constitute a diverse group of genetic disorders characterized by defects in biochemical pathways. Many have a predominantly neurological phenotype reflecting accumulation of neurotoxic metabolites and/or a deficiency of substrates critical for normal neurological development and function.
In this presentation, the pattern of neurometalbolic disorders found in Bangladesh would be discussed. There is very limited study regarding metabolic disorders affecting nervous system till date particularly in developing country. Thus this presentation would highlight the scenario of neurometabolic diseases in Bangladesh. Here along with the discussion of the neurometabolic diseases, some cases would be discussed with clinical features, investigation profile, neuroimaging , treatment and outcome. In this regard, we would like to share our experience with some interesting cases like methylmalonic academia, nonketotic hyperglycemia, urea cycle disorder, molybodynum cofactor deficiency, isovaleric academia, leigh disease etc. Moreover, a study to observe the incidence of inborn error of metabolism in 50 sick newborn in a tertiary care hospital in Bangladesh would be discussed .
.Conclusion: Although the burden of infectious disease is high in developing country like Bangladesh, neurometabolic disorders are not uncommon which should be recognized and treated in spite of limited resources. This presentation highlights the reorganization and management of those cases.