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简称LCAT缺乏症.1967年挪威首次报告是遗传性疾病,1979年又报告1例.本例是第3个家系的LCAT缺乏症.典型病例;47岁男性,从学龄期就有角膜混浊,34岁时出现蛋白尿,45岁时出现下肢浮肿,47岁时浮肿加重,血压220/80mmHg,因角膜混浊来眼科检查.家族史:姐姐和祖母的妹妹患同样的角膜混浊,无血族结婚.视力右1.2,左1.5,两眼角膜混浊以周边部为重,呈环形似老年环,环形混浊和角膜缘之间有一比较窄的透明区,但混浊的周边部界线不清楚.用裂隙灯检查,见Bowman氏层到Descemet膜之间的实质层有散在细点状混浊,角膜内皮细胞的平均面积是315±67μm~2,呈六
Referred to as LCAT deficiency .First reported in Norway in 1967 is a hereditary disease, and in 1979 another case was reported in this case is LCAT deficiency in the third family. Typical cases; 47-year-old male, from school age had corneal opacity 34 Proteinuria at age, lower extremity edema at age 45, increased edema at age 47, blood pressure 220 / 80mmHg, due to corneal opacity to ophthalmic examination. Family history: Sister and grandmother sister with the same corneal opacity, no blood group married. Right 1.2, left 1.5, the two corneal opacity to the periphery as the heavier, ring-like elderly ring, turbidity and limbal ring between a relatively narrow transparent area, but the turbidity of the peripheral boundary is unclear.Slit lamp examination, See Bowman’s layer to the Descemet membrane between the substantive layers of scattered fine turbidity, the average corneal endothelial cell area is 315 ± 67μm ~ 2, was six