Suppr超能文献

9日龄新生儿肝外胆道闭锁的早期Kasai肝门空肠吻合术:一例强调及时干预可改善预后的病例报告。

Early Kasai portoenterostomy in a 9-day-old newborn with extrahepatic biliary atresia: a case report highlighting improved prognosis with prompt intervention.

作者信息

El-Shabrawi Mortada H F, Eltagy Gamal, Qinawy Mohamed, Oshi Mohammed Am, Algethami Amjad, Alhujayri Nawaf A, Alharthi Shaker S, Alelyani Abdullah M, Kamal Naglaa M

机构信息

Professor of Pediatrics, Pediatric Hepatology Unit, Kasr Al Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.

Professor of Pediatric Surgery, Kasr Al Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.

出版信息

J Int Med Res. 2025 Feb;53(2):3000605241311115. doi: 10.1177/03000605241311115.

Abstract

Extrahepatic biliary atresia (EHBA) is a leading cause of neonatal cholestasis, often resulting in end-stage cirrhosis and portal hypertension without early diagnosis and treatment. This report highlights the importance of timely intervention, describing a 6-day-old male newborn diagnosed with EHBA who underwent successful Kasai portoenterostomy at 9 days of age. While the procedure is typically performed within the first 60 days of life, this exceptionally early intervention led to significantly improved outcomes. Postoperative recovery was marked by bilirubin normalization within 2 months and steady improvement in liver function tests, demonstrating the advantages of early surgery. The procedure involved creating a Roux-en-Y hepatic portojejunostomy to restore bile flow, preventing progression to biliary cirrhosis. Early intervention achieved effective bile drainage and substantial clinical improvement. At the 1-year follow-up, the infant displayed normal growth and liver function. This case supports the hypothesis that performing Kasai portoenterostomy earlier than current guidelines recommend may lead to better outcomes. It underscores the need for vigilant neonatal care to recognize early signs of cholestasis and enable prompt surgical intervention. Early diagnosis and intervention can preserve liver function, potentially delaying or preventing the need for liver transplantation.

摘要

肝外胆管闭锁(EHBA)是新生儿胆汁淤积的主要原因,若不及早诊断和治疗,常导致终末期肝硬化和门静脉高压。本报告强调了及时干预的重要性,描述了一名6日龄男性新生儿被诊断为EHBA,并在9日龄时成功接受了Kasai肝门空肠吻合术。虽然该手术通常在出生后的前60天内进行,但这种异常早期的干预导致了显著改善的结果。术后恢复的标志是2个月内胆红素正常化,肝功能检查稳步改善,证明了早期手术的优势。该手术包括创建一个Roux-en-Y肝门空肠吻合术以恢复胆汁流动,防止发展为胆汁性肝硬化。早期干预实现了有效的胆汁引流和显著的临床改善。在1年的随访中,该婴儿生长和肝功能正常。本病例支持这样的假设,即比当前指南建议的时间更早进行Kasai肝门空肠吻合术可能会带来更好的结果。它强调了需要警惕的新生儿护理,以识别胆汁淤积的早期迹象并及时进行手术干预。早期诊断和干预可以保留肝功能,有可能延迟或避免肝移植的需要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e03c/11807277/57b064082ca1/10.1177_03000605241311115-fig1.jpg

相似文献

2
Surgical experience in children with biliary atresia treated with portoenterostomy.
Curr Surg. 2005 Jul-Aug;62(4):439-43. doi: 10.1016/j.cursur.2004.11.022.
3
The Kasai procedure in the treatment of biliary atresia.
J Pediatr Surg. 1995 Jul;30(7):1077-80; discussion 1080-1. doi: 10.1016/0022-3468(95)90345-3.
4
Effect of repeat Kasai hepatic portoenterostomy on pediatric live-donor liver graft for biliary atresia.
Exp Clin Transplant. 2013 Jun;11(3):259-63. doi: 10.6002/ect.2012.0188. Epub 2013 Mar 26.
5
Biliary atresia: pathogenesis and treatment.
Semin Liver Dis. 1998;18(3):281-93. doi: 10.1055/s-2007-1007164.
7
Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?
Pediatr Surg Int. 2008 Aug;24(8):931-3. doi: 10.1007/s00383-008-2190-0. Epub 2008 Jun 28.
9
The Kasai portoenterostomy: when is it too late?
J Pediatr Surg. 2001 Jan;36(1):97-9. doi: 10.1053/jpsu.2001.20020.

本文引用的文献

1
Awareness, referral and age at Kasai surgery for biliary atresia in Europe: A survey of the Quality-of-Care Task Force of ESPGHAN.
J Pediatr Gastroenterol Nutr. 2024 Jun;78(6):1374-1382. doi: 10.1002/jpn3.12186. Epub 2024 Mar 18.
3
Impacts of Early Kasai Portoenterostomy on Short-Term and Long-Term Outcomes of Biliary Atresia.
Hepatol Commun. 2020 Nov 8;5(2):234-243. doi: 10.1002/hep4.1615. eCollection 2021 Feb.
4
Diagnostic Value and Effectiveness of an Artificial Neural Network in Biliary Atresia.
Front Pediatr. 2020 Aug 6;8:409. doi: 10.3389/fped.2020.00409. eCollection 2020.
5
Management of Biliary Atresia in France 1986 to 2015: Long-term Results.
J Pediatr Gastroenterol Nutr. 2019 Oct;69(4):416-424. doi: 10.1097/MPG.0000000000002446.
6
Kasai portoenterostomy for biliary atresia - Surgical precautions for better outcomes.
J Pediatr Surg. 2019 Apr;54(4):868-869. doi: 10.1016/j.jpedsurg.2018.09.028. Epub 2018 Nov 27.
7
Liver fibrosis in biliary atresia.
World J Pediatr. 2019 Apr;15(2):117-123. doi: 10.1007/s12519-018-0203-1. Epub 2018 Nov 21.
9
Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature.
Surg Today. 2015 Jul;45(7):813-8. doi: 10.1007/s00595-014-1024-z. Epub 2014 Sep 12.
10
The CARE guidelines: consensus-based clinical case reporting guideline development.
Headache. 2013 Nov-Dec;53(10):1541-7. doi: 10.1111/head.12246.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验