Department of Epidemiology, Emory University Rollins School of Public Health, Atlanta, Georgia.
Malformation Monitoring Centre Saxony-Anhalt, Medical Faculty, Otto-von-Guericke-University, Magdeburg, Germany.
Am J Perinatol. 2024 Jul;41(9):1143-1154. doi: 10.1055/s-0042-1748318. Epub 2022 May 29.
Bladder exstrophy (BE) is a rare but severe birth defect affecting the lower abdominal wall and genitourinary system. The objective of the study is to examine the total prevalence, trends in prevalence, and age-specific mortality among individuals with BE.
We conducted a retrospective cohort study. Data were analyzed from 20 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research in 16 countries. Live births, stillbirths, and elective terminations of pregnancy for fetal anomaly (ETOPFA) diagnosed with BE from 1974 to 2014. Pooled and program-specific prevalence of BE per 100,000 total births was calculated. The 95% confidence intervals (CI) for prevalence were estimated using Poisson approximation of binomial distribution. Time trends in prevalence of BE from 2000 to 2014 were examined using Poisson regression. Proportion of deaths among BE cases was calculated on the day of birth, day 2 to 6, day 7 to 27, day 28 to 364, 1 to 4 years, and ≥5 years. Mortality analysis was stratified by isolated, multiple, and syndromic case status.
The pooled total prevalence of BE was 2.58 per 100,000 total births (95% CI = 2.40, 2.78) for study years 1974 to 2014. Prevalence varied over time with a decreasing trend from 2000 to 2014. The first-week mortality proportion was 3.5, 17.3, and 14.6% among isolated, multiple, and syndromic BE cases, respectively. The majority of first-week mortality occurred on the first day of life among isolated, multiple, and syndromic BE cases. The proportion of first-week deaths was higher among cases reported from programs in Latin America where ETOPFA services were not available.
Prevalence of BE varied by program and showed a decreasing trend from 2000 to -2014. Mortality is a concern among multiple and syndromic cases, and a high proportion of deaths among cases occurred during the first week of life.
· Total prevalence of BE was 2.58 per 100,000 births.. · Prevalence decreased from 2000 to 2014.. · The first-week mortality was 9.3%..
膀胱外翻（BE）是一种罕见但严重的出生缺陷，影响下腹壁和泌尿生殖系统。本研究的目的是检查 BE 患者的总患病率、患病率趋势和特定年龄死亡率。
我们进行了一项回顾性队列研究。数据来自 16 个国家的 20 个出生缺陷监测计划，这些计划是国际出生缺陷监测和研究信息交换所的成员。1974 年至 2014 年间，通过 BE 诊断的活产、死产和因胎儿异常（ETOPFA）而选择性终止妊娠。每 10 万例总出生人数中 BE 的总患病率和各计划的患病率均以泊松近似二项分布计算。使用泊松回归检查 2000 年至 2014 年 BE 患病率的时间趋势。BE 病例的死亡比例按出生当天、第 2 至 6 天、第 7 至 27 天、第 28 至 364 天、1 至 4 岁和≥5 岁计算。分层分析了孤立性、多发性和综合征性病例的死亡率。
1974 年至 2014 年，BE 的总患病率为每 10 万例总出生人数 2.58 例（95%置信区间[CI]：2.40，2.78）。患病率随时间变化，从 2000 年到 2014 年呈下降趋势。孤立性、多发性和综合征性 BE 病例的第一周死亡率分别为 3.5%、17.3%和 14.6%。孤立性、多发性和综合征性 BE 病例的大多数第一周死亡发生在生命的第一天。在没有提供 ETOPFA 服务的拉丁美洲计划中报告的病例中，第一周死亡的比例较高。
BE 的患病率因计划而异，从 2000 年到 2014 年呈下降趋势。多发性和综合征性病例的死亡率是一个问题，并且大多数死亡发生在生命的第一周。
·BE 的总患病率为每 10 万例出生人数 2.58 例。·从 2000 年到 2014 年，患病率呈下降趋势。·第一周死亡率为 9.3%。
