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DISTURBANCE IN GLYCOGEN METABOLISM WITH HEPATOMEGALY

DISTURBANCE IN GLYCOGEN METABOLISM WITH HEPATOMEGALY In 1929, von Gierke1 first described a syndrome which he called glycogenic hepatonephromegaly. He reported the cases of two patients, aged 8 and 4½ years, both of which came to autopsy undiagnosed. The postmortem examination revealed, in both cases, a tremendously enlarged liver, moderately enlarged kidneys and, in one case, hypoplastic suprarenal glands. Microscopically, the cells of the liver and of the collecting tubules of the kidneys were packed with glycogen. Since this report was published, four cases of a similar nature have been described. At present we have under observation, at the Mount Sinai Hospital, a child whose history, physical examination, clinical course and laboratory data in many ways resemble those of the cases previously reported. We feel that the case is of sufficient interest to be reported and compared with the seven other cases found in the literature. REPORT OF CASE History.—The patient, an Italian boy, http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American journal of diseases of children American Medical Association

DISTURBANCE IN GLYCOGEN METABOLISM WITH HEPATOMEGALY

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Publisher
American Medical Association
Copyright
Copyright © 1934 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0096-8994
eISSN
1538-3628
DOI
10.1001/archpedi.1934.01960110091009
Publisher site
See Article on Publisher Site

Abstract

In 1929, von Gierke1 first described a syndrome which he called glycogenic hepatonephromegaly. He reported the cases of two patients, aged 8 and 4½ years, both of which came to autopsy undiagnosed. The postmortem examination revealed, in both cases, a tremendously enlarged liver, moderately enlarged kidneys and, in one case, hypoplastic suprarenal glands. Microscopically, the cells of the liver and of the collecting tubules of the kidneys were packed with glycogen. Since this report was published, four cases of a similar nature have been described. At present we have under observation, at the Mount Sinai Hospital, a child whose history, physical examination, clinical course and laboratory data in many ways resemble those of the cases previously reported. We feel that the case is of sufficient interest to be reported and compared with the seven other cases found in the literature. REPORT OF CASE History.—The patient, an Italian boy,

Journal

American journal of diseases of childrenAmerican Medical Association

Published: Apr 1, 1934

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