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December 1964

Idiopathic Hyperlipemia: Case Report of Sudden Death in an Afflicted Infant

Author Affiliations

Nobuhisa Baba, MD, Department of Pathology, Ohio State University, 320 W Tenth Ave, Columbus, Ohio 43210.; From the Department of Pathology, the Ohio State University.

Am J Dis Child. 1964;108(6):633-643. doi:10.1001/archpedi.1964.02090010635009

Idiopathic familial hyperlipemia is not uncommon, particularly in its heterozygous state where only mild changes of blood lipid and/or delayed fat tolerance test is seen. Among 998 college students in Sweden, Hirschhorn et al1 found 36 hyperlipemics. Fat tolerance studies done on 19 of the 36 showed delayed fat tolerance in 16 of the individuals. Numerous clinical observations and biochemical studies on this disorder have appeared since the first clinical description by Bürger and Grütz.2 This disease has been extensively reviewed by Thannhauser3 and Fredrickson.4 Because of its benign course, death due to this disorder is extremely rare. Only one case with autopsy has been reported,5 and in that case, the death could not be attributed to this disorder. This report, the second to include autopsy findings, concerns the sudden unexpected death of a hyperlipemic infant whose death was related to this disease and whose