["Hypo-alpha-lipoproteinemia" (Tangier-disease) (author's transl)]

Report on a case of Tangier disease in a girl aged 4.11 m. Clinically the tonsils were hypertrophic and of a yellow color. Their stroma contained many cholesterinesters which produced the histologic picture of foam-cell xanthomatosis. The serum contained only traces of alpha-lipoprotein. The level of Cholesterin of the HDL fraction was reduced to one twelvth of the norm. The serum of her father also showed a clearly reduced content of HDL cholesterin. In the patient herself lipolytic enzymes like lecithin-cholesterin-acyltransferase, post-heparin plasma-lipoprotein lipase and hepatic triglycerid-lipase showed normal activity.