Current status and challenges for LSDs in Bangladesh

Abstract

Inborn errors of metabolism are a common cause of inherited disease1 of which lysosomal storage diseases (LSDs) are a significant subgroup.2,3 Although the first clinical descriptions of patients with lysosomal storage disorders (LSDs) were reported at the end of the nineteenth century by Warren Tay,4 Bernard Sachs (1887; Tay-Sachs disease)5, and by PhillipeGaucher (1882; Gaucher disease)6,the biochemical nature of the accumulated products was only elucidated some 50 years later7. Considerably more time was then required for the demonstration by Hers that there was a link between an enzyme deficiency and a storage disorder (Pompe disease).8