University College Hospital, Ibadan, Nigeria.
Eleta Eye Institute, Ibadan, Nigeria.
Pan Afr Med J. 2023 Aug 4;45:150. doi: 10.11604/pamj.2023.45.150.40668. eCollection 2023.
ophthalmic genetics is rapidly evolving globally but is still nascent in much of sub-Saharan Africa, with gaps in knowledge about the burden in the region. This study evaluated the burden and manifestations of genetic eye diseases in children in Ibadan, Nigeria.
this was a hospital-based cross-sectional study in which new and follow-up paediatric eye clinic patients were recruited consecutively at the University College Hospital, Ibadan. Children with genetic eye diseases had comprehensive ocular and systemic examinations, and their pedigrees were charted to determine the probable modes of inheritance. The main outcome variables were the proportion of study participants with genetic eye diseases, the probable modes of inheritance, and the clinical diagnoses. Summary statistics were performed using means and standard deviations for numerical variables and proportions for categorical variables.
fifty-two (12%) of 444 children had genetic eye diseases, and their mean (SD) age was 88.8 ± 50.4 months. Thirteen different phenotypic diagnoses were made following the evaluation of the 52 children, including primary congenital glaucoma (13, 25%) and familial non-syndromic cataracts (8, 15%). The probable modes of inheritance were derived from the pedigree charts, and 30 (58%) conditions were presumed to be sporadic.
this study demonstrated a significant burden and a wide range of paediatric genetic eye diseases in this tertiary referral centre in Nigeria. This information provides invaluable evidence for planning ophthalmic genetic services.
眼科遗传学在全球范围内迅速发展,但在撒哈拉以南非洲的许多地区仍处于起步阶段,该地区对疾病负担的了解存在空白。本研究评估了尼日利亚伊巴丹儿童遗传眼病的负担和表现。
这是一项基于医院的横断面研究,连续招募了伊巴丹大学教学医院新的和随访的儿科眼科诊所患者。患有遗传眼病的儿童接受了全面的眼部和全身检查,并绘制了他们的家系图,以确定可能的遗传模式。主要观察变量是遗传眼病患者的比例、可能的遗传模式和临床诊断。使用均值和标准差表示数值变量,使用比例表示分类变量进行汇总统计。
444 名儿童中有 52 名(12%)患有遗传眼病,他们的平均(SD)年龄为 88.8±50.4 个月。对 52 名儿童进行评估后,共做出 13 种不同的表型诊断,包括原发性先天性青光眼(13 例,25%)和家族性非综合征性白内障(8 例,15%)。遗传模式是从系谱图中推断出来的,其中 30 种(58%)疾病被认为是散发性的。
本研究表明,在尼日利亚的这家三级转诊中心,遗传眼病的儿童负担显著且种类繁多。这些信息为规划眼科遗传服务提供了宝贵的证据。