Alkatheeri Manal Salem, Alawadhi Haifa, Almheiri Mira, Amin Saista, Qazi Abid, Tzivinikos Christos, Muneer Doha, Bitar Rana
Pediatrics Gastroenterology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates.
Department of Pediatrics, Tawam Hospital, Al Ain, Abu Dhabi, United Arab Emirates.
Saudi J Gastroenterol. 2025 Jul 1;31(4):227-232. doi: 10.4103/sjg.sjg_38_25. Epub 2025 Jul 14.
While extensive research provides valuable insights into biliary atresia (BA) globally, there remain gaps in region-specific data, such as West Asia. We aim to evaluate the incidence and survival of patients with BA in the United Arab Emirates.
We retrospectively reviewed the records of all patients diagnosed with BA in all paediatric tertiary hospitals from 2011 to 2021.
50 patients were identified, 11 were excluded due to insufficient information to confirm a diagnosis of BA. The final cohort was 39 patients. The overall incidence of biliary atresia was 3.75 per 100,000 live births. The incidence rates varied over the years, with the lowest recorded incidence in 2011 at 1.19 per 100,000 live births and the highest in 2017 at 7.16 per 100,000 live births. The median age at Kasai portoenterostomy procedure was 52 days (CI: 42-60, range: 6-142) The survival with native liver was 50% at 12 months, and 42.1% at 5 and 10 years post-operatively. The overall survival with native or transplant liver was 88.2% at 5 and 10 years.
While the incidence of BA is variable worldwide, the incidence in the United Arab Emirates in amongst the lowest reported. The role of early diagnosis and specialized surgical intervention is of importance for patient survival with native liver. The overall patient survival is further enhanced by availability of well-structured liver transplantation programs.
尽管广泛的研究为全球范围内的胆道闭锁（BA）提供了有价值的见解，但在特定区域的数据方面仍存在差距，如西亚地区。我们旨在评估阿拉伯联合酋长国BA患者的发病率和生存率。
我们回顾性分析了2011年至2021年期间所有儿科三级医院中所有诊断为BA的患者的记录。
共识别出50例患者，其中11例因信息不足无法确诊BA而被排除。最终队列有39例患者。胆道闭锁的总体发病率为每10万活产3.75例。发病率多年来有所变化，2011年记录的最低发病率为每10万活产1.19例，2017年最高，为每10万活产7.16例。Kasai肝门空肠吻合术的中位年龄为52天（可信区间：42 - 60天，范围：6 - 142天）。术后12个月时，保留自身肝脏的生存率为50%，术后5年和10年时为42.1%。保留自身肝脏或接受肝移植后的总体5年和10年生存率为88.2%。
虽然BA在全球的发病率各不相同，但阿拉伯联合酋长国的发病率在报告中处于最低水平。早期诊断和专业手术干预对患者保留自身肝脏的生存至关重要。完善的肝移植项目的可及性进一步提高了患者的总体生存率。
