Abstract
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 language | English |
|---|---|
| Pages (from-to) | 249-254 |
| Number of pages | 6 |
| Journal | Japanese Journal of Ophthalmology |
| Volume | 32 |
| Issue number | 3 |
| Publication status | Published - 1988 |
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ASJC Scopus subject areas
- Ophthalmology
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Keratomalacia in a child with familial hypo-retinol-binding proteinemia. / Matsuo, Toshihiko; Matsuo, N.; Shiraga, Fumio; Koide, N.
In: Japanese Journal of Ophthalmology, Vol. 32, No. 3, 1988, p. 249-254.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Keratomalacia in a child with familial hypo-retinol-binding proteinemia
AU - Matsuo, Toshihiko
AU - Matsuo, N.
AU - Shiraga, Fumio
AU - Koide, N.
PY - 1988
Y1 - 1988
N2 - 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.
AB - 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.
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M3 - Article
C2 - 3148050
AN - SCOPUS:0023711981
VL - 32
SP - 249
EP - 254
JO - Japanese Journal of Ophthalmology
JF - Japanese Journal of Ophthalmology
SN - 0021-5155
IS - 3
ER -