Sphingolipidoses are a group of lipid storage diseases or progressive storing diseases caused by a lack of an enzyme necessary for the catabolism of ceramide-containing lipids, which is also related to sphingolipid biosynthesis. Niemann–Pick illness, Fabry infection, Krabbe illness, Gaucher disease, Tay–Sachs disease, and metachromatic leukodystrophy are the most common members of this category. They are generally regressive in automobile conditions, except Fabry, a recessive X-linked illness.
Sphingolipidoses affect around one out of every 10,000 people, although they affect a far higher percentage of specific groups, like Ashkenazi Jews. Fabry illness and Gaucher disease may both be treated with enzyme replacement treatment, and individuals with these sphingolipidoses can survive well into adulthood. The other kinds are usually deadly by the age of 1 to 5 years for juvenile forms, although juvenile- or adult-onset variants may develop more slowly.
