Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Pediatría Dr. Silvestre Frenk Freund, Departamento de Cirugía Pediátrica. Ciudad de México, México.
Universidad Autónoma de Chihuahua, Facultad de Medicina y Ciencias Biomédicas, Laboratorio PABIOM. Chihuahua, Chihuahua, México.
Rev Med Inst Mex Seguro Soc. 2024 Mar 4;62(2):1-6. doi: 10.5281/zenodo.10711842.
Bile atresia is children's leading cause of cirrhosis. Its high morbidity and mortality are explained by its complications, including gastrointestinal bleeding and portal hypertension. Umbilical hernia has been embryologically and clinically associated with portal hypertension and cirrhosis; however, the clinical evolution of patients with bile atresia and umbilical hernia is unknown, so the aim of the study is to describe this link.
To determine the prevalence of the coexistence of AVB and HU in patients in a tertiary hospital, and describe the clinical evolution of these patients with respect to the presence/absence of HU as a strategy in the search for prognostic factors of morbidity and mortality.
This is an observational, descriptive, retrospective, and longitudinal study that included patients with bile atresia in a tertiary pediatric hospital. The variables related to bile atresia were analyzed, and the clinical evolution was described according to the presence or absence of UH.
56 patients with bile atresia were included, of which 69.6% were females. Portal hypertension occurred in 80.4%, with the presence of collateral venous network, gastrointestinal bleeding, ascites, and hepatomegaly being the indirect clinical data most often presented. The frequency of coexistence of umbilical hernia and bile atresia was 75%. Portal hypertension, collateral venous network, gastrointestinal bleeding, ascites, and small esophageal varices were the significantly more prevalent variables in patients with umbilical hernia.
Patients with bile atresia have a greater possibility of developing umbilical hernia and that it is associated with a more advanced evolution of portal hypertension and its associated signs and complications.
先天性胆道闭锁是儿童肝硬化的主要病因。其高发病率和死亡率与其并发症有关，包括胃肠道出血和门静脉高压。脐疝在胚胎学和临床上与门静脉高压和肝硬化有关；然而，先天性胆道闭锁伴脐疝患者的临床演变尚不清楚，因此本研究旨在描述这种联系。
确定三级医院中先天性胆道闭锁伴脐疝患者的共存率，并描述这些患者的临床演变，以探讨 HU 的存在/缺失作为寻找发病率和死亡率预后因素的策略。
这是一项在三级儿科医院进行的观察性、描述性、回顾性和纵向研究，纳入了先天性胆道闭锁患者。分析了与先天性胆道闭锁相关的变量，并根据是否存在 UH 描述了临床演变。
共纳入 56 例先天性胆道闭锁患者，其中 69.6%为女性。80.4%发生门静脉高压，间接临床数据最常表现为侧支静脉网络、胃肠道出血、腹水和肝肿大。脐疝和先天性胆道闭锁共存的发生率为 75%。存在脐疝的患者中，门静脉高压、侧支静脉网络、胃肠道出血、腹水和小食管静脉曲张等变量更为常见。
先天性胆道闭锁患者发生脐疝的可能性更大，且与门静脉高压及其相关体征和并发症的更晚期演变相关。
