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Case Reports
July 1928

LIPOID HISTIOCYTOSIS (NIEMANN-PICK'S DISEASE): WITH A REPORT OF A CASE DIAGNOSED DURING LIFE

Author Affiliations

Associate in Pediatrics, University of Illinois College of Medicine CHICAGO
From the Departments of Pediatrics and Pathology of the Research and Educational Hospitals and the University of Illinois College of Medicine.

Am J Dis Child. 1928;36(1):102-114. doi:10.1001/archpedi.1928.01920250109010
Abstract

At no period during life is an enlarged spleen so common an observation as in infancy. The majority of these splenic enlargements are secondary to or associated with an easily determined primary condition. There remains, however, a considerable group of obscure cases of splenomegaly in infancy, which are difficult of differentiation and which are frequently diagnosed only postmortem. There is, nevertheless, a splenomegaly occurring in lipoid histiocytosis (Niemann-Pick's disease) in which the diagnosis can definitely be made by means of splenic puncture and the determination of the blood lipoids. This is clearly shown in the following case.

REPORT OF CASE  History.—L. S., a white Jewish girl, aged 15 months, entered the hospital on April 4, 1927, with a complaint of vomiting and distended abdomen since the second month of life, and evidence of mental impairment, such as inability to sit up or to recognize parents. The condition was of

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