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(Investigative Ophthalmology and Visual Science. 1971;10:784-793.)
© 1971 by The Association for Research in Vision and Ophthalmology, Inc.

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Retinitis Pigmentosa in Abetalipoproteinemia: Effects of Vitamin A

P. GOURAS 1, R. E. CARR 1, and R. D. GUNKEL 1

1 Laboratory of Vision Research, National Eye Institute, National Institutes of Health, United States Department of Health, Education, and Welfare, Bethesda, Md. 20014

Two patients with abetalipoproteinemia are shown to develop a serum vitamin A deficiency when maintained on a normal diet. This deficiency leads to a severe impairment of vision, including abnormalities of dark adaptation and the electroretinogram which involve both rod and cone receptor systems. Massive oral doses of vitamin A can reverse these abnormalities. Cone function recovers more rapidly than rod function after vitamin A therapy. The relationship between vitamin A and the retinitis pigmentosa which these patients develop is discussed.

Key Words: abetalipoproteinemia • vitamin A • retinitis pigmentosa • rods • cones • dark adaptation • electroretinogram

Submitted on July 6, 1971
Accepted on August 19, 1971




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