Recurrent corneal erosion associated with Alport's syndrome. Rapid communication.
Recurrent corneal erosion associated with Alport's syndrome. Rapid communication.
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与奥尔波特综合征相关的复发性角膜糜烂。
DOI：
10.1038/ki.1997.321
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发表时间：
1997
影响因子：
19.6
通讯作者：
Y. Pirson
中科院分区：
文献类型：
--
作者：
Ceril Rhys;B. Snyers;Y. Pirson
与阿尔波特综合征 (AS) 相关的眼部缺陷包括前圆锥形和视网膜斑点。我们报告复发性角膜糜烂（RCE）是该疾病的另一种眼部表现。患有 AS 的三名兄弟报告有自发性 RCE 发作史（其中 2 名兄弟在 1 至 3 年内发作了 2 次，其中一名兄弟在过去 10 年中发作了约 60 次），其特征是急性眼部疼痛、流泪和持续 2 至 5 天的畏光。来自同一家族的另外两个未受影响的兄弟没有出现 RCE，这表明 AS 和 RCE 之间存在关联，并促使我们评估其患病率。对 41 名患有 AS 和肾功能衰竭的患者和 67 名对照移植患者（患有另一种原发性肾病）进行了评估。 7 名 AS 患者有 RCE 病史（首次出现在 12 至 21 岁之间），而对照患者中只有 1 名 (P = 0.003)。总之，约 20% 的 AS 合并肾功能衰竭患者有 RCE 病史。 RCE 可能是由于角膜上皮基底膜（含有 IV 型胶原）固有的结构弱点所致。在评估患者是否患有 AS 时，应询问 RCE 病史。当眼科医生面对患有非创伤性 RCE 的患者时，也应该意识到这种关联。
Ocular defects associated with Alport syndrome (AS) include anterior lenticonus and retinal flecks. We report on recurrent corneal erosion (RCE) as another ocular manifestation of the disease. Three brothers with AS reported a history of spontaneous attacks of RCE (2 episodes over 1 to 3 years in 2 of them and about 60 episodes in one brother over the last 10 years) characterized by acute ocular pain, lacrimation and photophobia lasting two to five days. The absence of RCE in the two other non-affected brothers from the same kindred suggested an association between AS and RCE, and prompted us to assess its prevalence. Forty-one patients with AS and renal failure and 67 control transplanted patients (with another original nephropathy) were evaluated. Seven AS patients had a history of RCE (first manifested between the ages of 12 and 21) versus only one control patient (P = 0.003). In conclusion, a history of RCE is found in about 20% of patients with AS and renal failure. RCE is likely to result from an inherent structural weakness of the corneal epithelial basement membrane (containing type IV collagen). A history of RCE should be sought when evaluating a patient for AS. Ophthalmologists should also be aware of this association, when confronted with a patient suffering from non-traumatic RCE.
纯合子和杂合子 Alport 家族性肾炎患者正常组织和肾基底膜中家族性肾炎抗原的分布。
DOI：
--
发表时间：
1989
期刊：
Laboratory investigation; a journal of technical methods and pathology
影响因子：
--
作者：
Kleppel,MM;Kashtan,C;Santi,PA;Wieslander,J;Michael,AF
通讯作者：
Michael,AF