Keratomalacia in a child with familial hypo-retinol-binding proteinemia

T. Matsuo, N. Matsuo, Fumio Shiraga, N. Koide

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3 Citations (Scopus)


Retinol-binding protein (RBP) is a plasma protein with a molecular weight of 21,000 synthesized in the hepatocytes, binding with retinol (vitamin A), and transporting retinol to peripheral tissues. Keratomalacia is caused by a deficiency of vitamin A itself and/or protein-calorie malnutrition. In the latter condition, production of RBP is inhibited. We report herein familial hypo-retinol-binding proteinemia in a child aged 19 months who developed keratomalacia during measles infection in spite of good nourishment. To the best of our knowledge this is the first description of such a case. 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-rich diet. They had normal liver function tests, normal serum levels of other proteins and fat-soluble vitamins and lipids. This hypo-retinol-binding proteinemia was thought to predispose the child to develop keratomalacia during measles. Family members would be heterozygous with one normal RBP gene and one defective RBP gene.

Original languageEnglish
Pages (from-to)249-254
Number of pages6
JournalJapanese Journal of Ophthalmology
Issue number3
Publication statusPublished - Jan 1 1988

ASJC Scopus subject areas

  • Ophthalmology


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