RT Book, Section 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. SR Print(0) ID 6726955 T1 Chapter 161. Sphingolipidoses T2 Rudolph's Pediatrics, 22e YR 2011 FD 2011 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-149723-7 LK accesspediatrics.mhmedical.com/content.aspx?aid=6726955 RD 2024/03/29 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).