Studies on lipids from liver and spleen of a child (O.L.) with Niemann-Pick's disease type C.

Large accumulations of sphingomyelin and bis(monoacylglycero)phosphate were found in the spleen of a child, who died from a lipidosis (Morbus Niemann-Pick). The enrichment of both phospholipids in this organ was more extensive than in liver. Moreover: bis(monoacylglycero)phosphate, the lipid characteristic for lysosomes, had a remarkably high content of oleic acid: 76% in spleen and 63% in liver.