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首页> 外文期刊>Journal of pediatric ophthalmology and strabismus >Evolution of ocular manifestations in nephropathic cystinosis: a long-term study of a population treated with cysteamine.
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Evolution of ocular manifestations in nephropathic cystinosis: a long-term study of a population treated with cysteamine.

机译：肾病性胱氨酸病中眼部表现的演变：对半胱胺治疗人群的长期研究。

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BACKGROUND: Nephropathic cystinosis is characterized by an accumulation of cystine crystals within most body tissues. Renal transplantation and oral cysteamine have improved the general prognosis of the disease, and ocular manifestations are now the most common complication. This long-term follow-up study describes the sequence of ocular manifestations in patients with nephropathic cystinosis treated with oral and topical cysteamine. METHODS: Data were recorded for all patients with cystinosis examined between 1980 and 2000. For each patient, photophobia and visual acuity were evaluated and slit-lamp and fundus examinations were performed. For some patients, an electroretinogram was also performed. RESULTS: Twenty-nine patients were observed during this period. They received oral and topical cysteamine. Photophobia and loss of visual acuity generally began by 10 years of age but were severe only after 15 years of age. Peripheral corneal epithelial infiltration appeared in the first few years of life. Infiltration evolved toward the depth and center of the cornea during the second decade of life. Retinopathy was present in 51.7% of the patients, with 3 cases of maculopathy and 3 cases of flattening on electroretinogram. CONCLUSIONS: Photophobia and corneal infiltration, although generally severe after 15 years of age, could be treated with topical cysteamine and corneal transplantation. Retinal infiltration, previously described as frequent and potentially blinding, is currently observed in only half of these patients, with mild visual impairment. This could be related to the treatment with oral cysteamine reaching the retinal vascularization.
机译：背景：肾病性胱氨酸病的特征是大多数人体组织中胱氨酸晶体的积累。肾移植和口服半胱胺改善了该病的一般预后，眼部表现是目前最常见的并发症。这项长期的随访研究描述了口服和局部半胱胺治疗的肾病性胱氨酸病患者的眼部表现顺序。方法：记录所有1980年至2000年间检查的囊性变患者的数据。评估每位患者的畏光和视力，并进行裂隙灯和眼底检查。对于某些患者，还进行了视网膜电图检查。结果：在此期间观察到29名患者。他们接受了口服和局部半胱胺。畏光和视力丧失通常从10岁开始，但仅在15岁以后才开始严重。生命的最初几年出现了周围角膜上皮浸润。在生命的第二个十年中，浸润向角膜的深度和中心发展。 51.7％的患者存在视网膜病变，其中3例为黄斑病变，3例在视网膜电图上扁平化。结论：畏光和角膜浸润虽然在15岁以后通常很严重，但可以通过局部半胱胺和角膜移植治疗。目前仅在这些患者中，有一半患有轻度视力障碍，观察到视网膜浸润，以前被描述为频繁且可能致盲。这可能与口服半胱胺达到视网膜血管化的治疗有关。

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