RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164080305 T1 Niemann-Pick Disease T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164080305 RD 2024/10/04 AB Group of rare inherited disorders of fat metabolism with varying degrees of lipid storage and foam cell infiltration in tissues. Clinical features include jaundice, progressive loss of motor skills, feeding difficulties, learning disabilities, and hepatosplenomegaly. Lysosomal storage disorder caused by deficient activity of the enzyme acid sphingomyelinase (Types A and B) or due to defective function in cholesterol transport (Type C).