Familial hypo-retinol-binding proteinemia found in a child with keratomalacia

Toshihiko Matsuo, N. Matsuo, Fumio Shiraga, N. Koide

Research output: Contribution to journalArticle

Abstract

Retinol-binding protein (RBP) is a plasma protein with a molecular weight of 21,000 synthesized in the hepatocytes, binds with retinol (vitamin A), and transports it to peripheral tissues. Keratomalacia is caused by deficiency of vitamin A itself and/or protein-calorie malnutrition in the latter of which production of RBP is inhibited. We found for the first time familial hypo-retinol-binding proteinemia in a child at an age of 19 months who developed keratomalacia during measles infection in spite of good nourishment. The patient, her sister and mother showed persistent low levels, about half the normal levels of retinol and RBP which were unresponsive to oral vitamin A and protein diet. But they had normal liver function tests, normal levels of the other serum proteins, lipids, and fat-soluble vitamins. This hypo-retinol binding proteinemia was thought to predispose the child to develop keratomalacia during measles. The family members involved would be heterozygous for the normal gene and the defective gene of RBP.

Original languageEnglish
Pages (from-to)694-698
Number of pages5
JournalJournal of Japanese Ophthalmological Society
Volume92
Issue number4
Publication statusPublished - 1988

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Vitamin A
Retinol-Binding Proteins
Measles
Blood Proteins
Vitamin A Deficiency
Protein-Energy Malnutrition
Liver Function Tests
Vitamins
Genes
Keratomalacia
Siblings
Hepatocytes
Molecular Weight
Fats
Mothers
Diet
Lipids
Infection
Proteins

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Familial hypo-retinol-binding proteinemia found in a child with keratomalacia. / Matsuo, Toshihiko; Matsuo, N.; Shiraga, Fumio; Koide, N.

In: Journal of Japanese Ophthalmological Society, Vol. 92, No. 4, 1988, p. 694-698.

Research output: Contribution to journalArticle

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