A nationwide Danish study of 1027 cases of congenital/infantile cataracts - Etiological and clinical classifications
A nationwide Danish study of 1027 cases of congenital/infantile cataracts - Etiological and clinical classifications
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DOI：
10.1016/j.ophtha.2004.06.024
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发表时间：
2004-12-01
期刊：
影响因子：
13.7
通讯作者：
Melbye, M
中科院分区：
文献类型：
--
作者：
Haargaard, B;Wohlfahrt, J;Melbye, M
目的：根据病因和临床分类，研究先天性/婴幼儿白内障在丹麦总人口中的分布。设计：以人群为基础的队列研究，采用回顾性图表回顾。参与者：所有出生于1959-2001年间，在丹麦登记患有先天性/婴幼儿白内障的儿童(0-17岁)，在1977-2001年间。方法：根据病历资料进行白内障的病因和临床分类。主要观察指标：先天性/婴幼儿白内障按假定病因分类；性别；临床表现，包括偏侧性和形态学；以及根据病因和偏侧的时间趋势。结果：共纳入先天性/婴幼儿白内障1 027例，其中男孩529例，女孩498例，其中双眼占%。男性以双眼白内障为主，女性以单眼为主。单纯性白内障是最常见的临床表现(占所有病例的71%)，其次是伴有额外眼部畸形的白内障和伴有全身异常的白内障。几乎三分之二的病例病因不明(特发性)。与已知病因的病例相比，特发性病例表现为单眼白内障和额外的眼部畸形的比例更高。在87%的单侧病例和50%的双侧病例中，病因不明。除因母体感染引起的白内障外，其余病例分布稳定，主要是由于先天性风疹的消除而下降。结论：自20世纪70年代末以来，先天性/婴幼儿不明原因、遗传性和感染性白内障病例所占比例除先天性风疹外均呈下降趋势。87%的单侧白内障和50%的双眼先天性/婴儿性白内障的病因尚不清楚，这使得该病的预防成为一个持续的挑战。眼科2004；美国眼科学会111：2292-2298(C)2004。
Objective: To study the distribution of congenital/infantile cataract in the entire population of Denmark according to etiological and clinical classifications.Design: Population-based cohort study with retrospective chart review.Participants: All children (0 to 17 years old) who were born between 1959 and 2001 and registered with congenital/infantile cataract in Denmark during the period 1977 to 2001. Cases were ascertained from the mandatory Danish National Register of Patients, and all medical records were reviewed.Methods: Etiological and clinical classifications of the cataract cases were based on information from the medical records.Main Outcome Measures: Classification of congenital/infantile cataract according to presumed etiology; gender; clinical appearance, including laterality and morphology; and the time trends according to etiology and laterality.Results: A total of 1027 children with congenital/infantile cataract, 529 boys and 498 girls, were included, of whom 64% were bilateral. Males predominated with bilateral cataract, whereas females predominated with unilateral cases. Isolated cataract was the most frequent clinical presentation (71 % of all cases), followed by an even proportion of cataract associated with additional ocular dysmorphology and cataract associated with systemic anomalies. Almost two thirds of all cases had an unknown etiology (idiopathic). Idiopathic cases showed a higher proportion of unilateral cataract and of additional ocular dysmorphology compared with cases of known etiology. The etiology was unknown in 87% of unilateral cases and in 50% of bilateral cases. The distribution by presumed etiology was stable during the study period, except for cataract caused by maternal infections, which decreased mainly due to the elimination of congenital rubella.Conclusions: With the exception of the decline of congenital rubella, the proportion of congenital/infantile cataract cases of unknown, genetic, and infectious origins has been stable since the late 1970s. The causes of 87% of unilateral cataracts and 50% of bilateral congenital/infantile cataracts remain unknown, making the prevention of the disease a continuing challenge. Ophthalmology 2004;111:2292-2298 (C) 2004 by the American Academy of Ophthalmology.