食餌療法により軽快したガラクトース白内障の1例

Diet therapy reversed galactose cataract. Report of a case

　食餌療法により軽快したガラクトース白内障を経験した．ガスリー法陽性，ボイトラー法陰性で，血中ガラクトース値117mg／dlと高値を示した．白内障以外に臨床的に異常所見はなく，赤血球galactokinase活性の低値があり，生後1カ月でgalactokinase欠損型ガラクトース血症と診断された．ただちに，ガラクトースを除去した食餌療法を開始したところ，7日後には血中ガラクトース値はほぼ0mg／dlとなり，尿中還元糖も陰性となり，約30日後には水晶体混濁は著しく減少した．

We confirmed dietary restriction of milk and milk products reversed the cataract in galactosemia in human.To our knowledge, it seems not to have been described that the progress and details concerning dietary management for galacto-kinase defficiency in human photographically.

The patient was a 4-week-old girl,observed nuclear cataract in both eyes.Laboratory examina-tions revealed that Guthrie's method was positive, Beutler's method was negative. Galactose level in the blood was remarkably high.Galactokinase in the red blood cells was almost absent. A diagnosisof galactokinase defficiency was established en-zymatically at 4 weeks of age. The child was placed on a galactose-free diet immediately after the diagnosis. The dense cataract decreased and abnor-mal laboratory values improved after 4 weeks of treatment.

Rinsho Ganka (Jpn J Clin Ophthalmol) 42(12) : 1356-1361, 1988