Biliary Atresia in Northwest Iran: Epidemiologic Features and Long-Term Outcome
Biliary Atresia in Northwest Iran: Epidemiologic Features and Long-Term Outcome
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伊朗西北部的胆道闭锁：流行病学特征和长期结果
DOI：
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发表时间：
2020
影响因子：
0.5
通讯作者：
M. Shoaran
中科院分区：
文献类型：
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作者：
S. Naghashi;M. Rafeey;M. Ghojazadeh;Zeinab Nikniaz;S. Aslanabadi;M. Shoaran
背景：胆道闭锁是儿童期一种罕见的牵张性胆管病，也是儿科肝移植的最常见原因。本研究是伊朗西北部胆道闭锁及其发病率和结果的第一份报告。方法：在这项回顾性队列研究中，纳入了2006年3月至2016年3月在大不里士儿童医院收治的所有胆道闭锁患者。所有患者的人口统计、临床和临床前数据均从医院记录中提取。有关葛西门肠造口术后结果的信息是通过查看进度记录或通过家长的电话收集的。 Mann-Whitney U 和卡方或 Fisher 精确检验分别用于比较连续变量和分类变量。采用生存曲线和表格、Kaplan-Meier法和Cox回归进行生存分析。结果：胆道闭锁的发生率为1/13280活产儿。 85.1%的病例出现孤立性胆道闭锁。在我们的患者中未观察到脾脏畸形。葛西门肠造口成功率为42.9%。生存分析显示，1年、2年、3年、5年和10年自体肝脏存活率分别为73%、56%、54%、40%和31%。中位总生存率为 36 个月 [95%CI：2.89 - 69.1]。成功的葛西门肠造口术和黄疸清除是影响存活率的唯一因素。结论：伊朗西北部地区胆道闭锁的发病率低于其他亚洲国家。与其他亚洲国家一样，我们的患者很少出现脾脏异常。我们的开西造口术的成功率和存活率低于其他亚洲国家。
Background: Biliaryatresiaisararedistractivecholangiopathyinchildhoodandthemostcommoncauseof livertransplantation in pediatrics. Present study is the first report of biliary atresia and its incidence and outcome from northwest of Iran. Methods: In this retrospective cohort study, all patients with biliary atresia admitted to the Tabriz Children’s Hospital from March 2006 to March 2016were included. Demographical, clinical and preclinical data of all patients are extracted from the hospital records. Information about the outcome after Kasai portoenterostomy was gathered by reviewing the progress notes or by phone call from the parents. The Mann-Whitney U and chi-square or Fisher’s exact tests were used for comparison of continuous and categorical variables respectively. Survival curves and tables, Kaplan-Meier method and Cox regression were used for survival analysis. Results: Theincidencerateof biliaryatresiawas1/13280livebirths. Isolatedformof biliaryatresiawasseenin85.1% of cases. Splenic malformation was not observed in our patients. The rate of successful Kasai Portoenterostomy was 42.9%. Survival analysis showed that 1, 2, 3, 5, and 10-year native liver survival rate was 73%, 56%, 54%, 40% and 31%, respectively. Median overall survival rate was 36 months [95%CI: 2.89 - 69.1]. Successful Kasai Portoenterostomy and clearance of jaundice was the only factor that affected survival rate. Conclusions: Theincidencerateof biliaryatresiainNorthwestof IranwaslowerthanthatinotherAsiancountries. LikeotherAsian countries,spleenanomalywasrareinourpatients. Thesuccessof KasaiPortoenterostomyandsurvivalratesof ourcaseswerelower than that in other Asian countries.