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DISTURBANCE IN GLYCOGEN METABOLISM WITH HEPATOMEGALY(VON GIERKE'S DISEASE)
LOUISE RAUH, M.D.;
CARL ZELSON, M.D.
Am J Dis Child. 1934;47(4):808-820.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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In 1929, von Gierke1 first described a syndrome which he called glycogenic hepatonephromegaly. He reported the cases of two patients, aged 8 and 4 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,
. . . [Full Text PDF of this Article]
Author Affiliations
NEW YORK
From the service of Dr. B. Schick, Mount Sinai Hospital.
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