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胱氨酸病是一种罕见的常染色体隐性代谢障碍性疾病,其特征是胱氨酸结晶沉积于眼、骨髓、肝、脾、淋巴结和肾脏。它有三种类型:幼儿肾病型,成人良性型和青年中间型。幼儿肾病型最严重,伴有进行性肾功能衰竭,肾性佝偻病,寿命明显缩短。本文报告1例胱氨酸病继发青光眼。患者19岁,女,主诉左眼痛6周,有幼儿型胱氨酸病和两肾异体移植病史。既往检查证实胱氨酸病累及角膜。眼痛开始时,根据浅前房和眼压>35mmHg,诊断为左眼闭角型青光眼,给予0.5％噻吗心安2次/日和2％毛果芸香碱4次/日点眼,眼压无明显下降,疼痛未减轻。就诊时,右眼视力20/70,左眼光感、畏光。双眼角膜弥
Cystine disease is a rare autosomal recessive metabolic disorder characterized by cystine crystal deposition in the eye, bone marrow, liver, spleen, lymph nodes and kidneys. It has three types: child nephropathy, adult benign and youth intermediate. Kidney nephropathy in children with the most serious, accompanied by progressive renal failure, renal rickets, life expectancy was significantly shortened. This article reports 1 case of cystine secondary glaucoma. 19-year-old patient, female, complained of left eye pain for 6 weeks, with a history of childhood cystine disease and renal allograft. Previous examination confirmed cystine disease involving the cornea. Ocular pain at the beginning, according to shallow anterior chamber and intraocular pressure> 35mmHg, diagnosed as left angle closure glaucoma, giving 0.5% timolol 2 times / day and 2% pilocarpine 4 times / day eye, no significant decrease in intraocular pressure, Pain did not relieve. Visits, the right eye 20/70 vision, light perception of the left eye, photophobia. Eyes corneal Mi