TY - CHAP M1 - Book, Section TI - Chapter 161. Sphingolipidoses A1 - Clarke, Joe T. R. A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. Y1 - 2011 N1 - T2 - Rudolph's Pediatrics, 22e AB - The sphingolipidoses are a clinically heterogeneous group of disorders characterized by inherited point defects in the breakdown of complex lipids; this results in the accumulation of compounds containing a large lipophilic core called ceramide and either a hydrophilic oligosaccharide (glycosphingolipids) or a phosphorylcholine (sphingomyelin).1 Ceramide is composed of a long-chain fatty alcohol containing an amine group in amide linkage with a long-chain fatty acid (Fig. 161-1). SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/19 UR - accesspediatrics.mhmedical.com/content.aspx?aid=6726955 ER -