THERE ARE ONLY a few distinct clinical conditions in childhood which are classified under the heading of "lipidoses." These diseases have in common an increase in tissue lipid content and are most frequently identified by the presence of a morphologically distinct lipidladen histiocyte within the reticuloendothelial system. Gaucher's and Niemann-Pick are the two most common forms of lipidoses and in both, the pathology, histochemistry, and biochemical abnormalities are well documented.1-5 They therefore serve as a guide to which other lipid-storage syndromes may be compared. The purpose of this report is to describe an unusual lipid-storage syndrome occurring in childhood which differs in clinical pattern, cell morphology, and histochemical evaluation from either Gaucher's or Niemann-Pick disease.
Report of a Case
First Admission.—The patient was first seen in the Louisville Children's Hospital at 2½ months of age with a history of jaundice since the first week of life. On physical examination