PEDIATRICS Vol. 10 No. 1 July 1952, pp. 40-47
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GALACTOSEMIA

Report of Case with Autopsy

ALBERT M. EDMONDS M.D.1, GORDON R. HENNIGAR M.D.1, and ROBERT CROOKS M.D.1

1 The Medical College of Virginia, Richmond, Va.

A case of proved galactosemia in a two month old female infant with necropsy findings has been reported. The liver lesion in this case does not clearly fit into the category of Laennec's cirrhosis or biliary cirrhosis. The presence of the adenomata, rich in glycogen, and the peculiar acinar structures separates it into a distinctive type of lesion. It is proposed that these cases represent congenital and familial cirrhosis.

The administration of large amounts of galactose to rabbits failed to produce liver cell degeneration or necrosis. As suggested from the cases reported in the literature and in view of the lack of agreement on the pathogenesis among observers, the same mechanism may not be responsible for all of the cases of galactose diabetes recorded.

Submitted on July 26, 1951




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K. Fishier, R. Koch, G. N. Donnell, and E. Wenz
Developmental Aspects of Galactosemia from Infancy to Childhood
Clinical Pediatrics, January 1, 1980; 19(1): 38 - 44.
[Abstract] [PDF]