RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164069782 T1 Farber Uzman Syndrome 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=1164069782 RD 2024/10/03 AB Inborn error of the fatty metabolism caused by a lysosomal enzyme deficiency and resulting in the accumulation of ceramide in body tissues with multiple organ failure and death in infancy (but mildly affected patients in early age can reach adulthood). Severe motor and mental retardation is evident.