Estrada-Arce Emma Valeria, Aguila-Cano Renata, Lona-Reyes Juan Carlos, Flores-Fong Laura Esther, Rivera-Chávez Elva
From the Pediatric Gastroenterology Service, Hospital Civil de Guadalajara "Dr. Juan I. Menchaca" Salvador Quevedo y Zubieta, Guadalajara, Jalisco, México.
Universidad de Guadalajara, University Center for Health Sciences.
JPGN Rep. 2023 Jun 9;4(3):e318. doi: 10.1097/PG9.0000000000000318. eCollection 2023 Aug.
We describe the survival of children with acute liver failure (ALF), chronic liver disease (CLD), or acute-on-chronic liver failure (ACLF) with poor access to liver transplantation (LT). A retrospective cohort study of 42 patients <18 years of age was conducted in the Hospital Civil de Guadalajara "Dr. Juan I. Menchaca". The median age was 76 months; 57.1% were female, 40.5% presented with ALF, 35.7% with CLD, and 23.8% with ACLF. Also, 38.1% (16/42) presented liver disease of unknown etiology. Death occurred in 45.2%; 14.3% were transferred to another hospital, and none received LT. Mortality in ALF, CLD, and ACLF was 76%, 0%, and 60%, respectively. In the survival analysis, within the first 20 months after diagnosis, the mortality rate was greater than 50% with ALF. The importance of having referral programs that perform liver transplantation is highlighted by the poor prognosis of the patients, despite conservative treatment.
我们描述了那些难以获得肝移植(LT)的急性肝衰竭(ALF)、慢性肝病(CLD)或慢加急性肝衰竭(ACLF)患儿的生存情况。在瓜达拉哈拉“胡安·I·门查卡博士”市民医院对42例18岁以下患者进行了一项回顾性队列研究。中位年龄为76个月;57.1%为女性,40.5%为急性肝衰竭,35.7%为慢性肝病,23.8%为慢加急性肝衰竭。此外,38.1%(16/42)病因不明。45.2%的患者死亡;14.3%被转至其他医院,无一例接受肝移植。急性肝衰竭、慢性肝病和慢加急性肝衰竭的死亡率分别为76%、0%和60%。在生存分析中,诊断后的前20个月内,急性肝衰竭患者的死亡率超过50%。尽管进行了保守治疗,但患者预后不佳,这凸显了开展肝移植转诊项目的重要性。