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January 1959

The Lens in Congenital Galactosemia

Author Affiliations

Rochester, N. Y.
From the Department of Surgery, Division of Ophthalmology, of the University of Rochester School of Medicine and Dentistry.

AMA Arch Ophthalmol. 1959;61(1):88-92. doi:10.1001/archopht.1959.00940090090013

Congenital galactosemia is now well established as a hereditary disease caused by an inborn error of galactose metabolism. The clinical features of this rare condition are manifest very early in life, and their relationship to the ingestion of galactose has been amply documented.1-5 The main features consist of early nutritional failure hepatic and splenic enlargement, jaundice, lethargy, osteoporosis, mental retardation, and cataract formation. The latter two signs usually occur at or about the fourth week of life. The cataracts that have been noted in the literature have varied in appearance from a ring-like lamellar opacity, with or without vacuoles in the anterior cortex, to zonular and nuclear opacities.3,5-11 This varied picture is probably related to the severity and duration of the galactosemia and to the age of the patient.

Mitchell12,13 and Day14 reported on the occurrence of experimental cataracts in rats fed a high-galactose diet. The