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现报导同型胱氨酸尿症三例,病者为同一家系的兄妹三人。先证者,女,11岁,住院号348。母孕及分娩正常。患者因双眼晶体脱位,左、右眼先后继发性青光限,于1984年元月及8月两次住院。首次查:左眼视力眼前手动,混合性充血,角膜水肿、混浊,前房浅,晶体脱位于前房,瞳孔7mm,无对光反应,眼底不能窥见。右眼视力2尺指数,角膜透明,前房浅,晶体向鼻下方半脱位,可见部分悬韧带,瞳孔3mm,光反射灵敏,眼底(一)。限压:左69.27mmHg,右15 6lmmHg.行左眼晶体摘出术,术中顺利。二次住院查:左眼晶体摘出术后,视力同前。右眼视力不明(因不合作),混合性充血,角膜
Is reported homocystinuria in three cases, the same family of siblings three. Probation, female, 11 years old, hospital number 348. Mother’s pregnancy and childbirth normal. Patients with dislocation of both eyes because of the lens, the left and right eyes have secondary to the glaucoma limit, in January 1984 and August hospitalization twice. For the first time check: left eye vision in front of manual, mixed congestion, corneal edema, opacity, shallow anterior chamber, crystal off in the anterior chamber, pupil 7mm, no light reaction, fundus can not glimpse. 2-foot index of right eye vision, corneal transparency, shallow anterior chamber, subluxation of the lens below the nose, visible part of the hanging ligament, the pupil 3mm, light reflection sensitivity, fundus (a). Limit pressure: left 69.27mmHg, right 15 6lmmHg. Left eye crystal removal surgery, intraoperative smooth. Second hospitalization: left eye crystal removal surgery, visual acuity with the former. Right eyesight unknown (due to non-cooperation), mixed congestion, the cornea