Rhys C, Snyers B, Pirson Y
Department of Nephrology, University of Louvain Medical School, Cliniques Universitaires St. Luc, Brussels, Belgium.
Kidney Int. 1997 Jul;52(1):208-11. doi: 10.1038/ki.1997.321.
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.
与奥尔波特综合征(AS)相关的眼部缺陷包括前圆锥形晶状体和视网膜斑点。我们报告复发性角膜糜烂(RCE)是该疾病的另一种眼部表现。三名患有AS的兄弟报告有RCE自发发作史(其中两人在1至3年内发作2次,一名兄弟在过去10年中发作约60次),其特征为急性眼痛、流泪和畏光,持续两到五天。同一亲属关系中另外两名未受影响的兄弟没有RCE,这表明AS与RCE之间存在关联,并促使我们评估其患病率。对41例患有AS和肾衰竭的患者以及67例对照移植患者(患有另一种原发性肾病)进行了评估。7例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例(P = 0.003)。总之,约20%患有AS和肾衰竭的患者有RCE病史。RCE可能是由于角膜上皮基底膜(含IV型胶原)固有的结构薄弱所致。在评估AS患者时应询问是否有RCE病史。眼科医生在面对非创伤性RCE患者时也应意识到这种关联。