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胆道闭锁:一例病例报告。

Biliary Atresia: A Case Report.

作者信息

Muzaffer Maliha, Masarath Anees, Mohammed Fareedullah

机构信息

Department of Pharmacy, Mesco College of Pharmacy, Hyderabad, IND.

出版信息

Cureus. 2024 Dec 4;16(12):e75087. doi: 10.7759/cureus.75087. eCollection 2024 Dec.

DOI:10.7759/cureus.75087
PMID:39759692
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11697770/
Abstract

Biliary atresia (BA) is a serious hepatobiliary disorder that occurs due to progressive inflammation and scarring obstruction in the bile ducts, posing a threat to life. This condition usually appears in infants, and timely identification is fundamental for a better prognosis. If left untreated, individuals will inevitably experience liver damage and mortality. This case report describes a nine-month-old female infant presenting with jaundice, icteric sclera, yellowish skin, acholic feces, and hepatomegaly. Elevated liver enzymes and a hepatobiliary iminodiacetic acid (HIDA) scan confirmed BA. Histopathological examination revealed fibrosis, cholestatic disease, and an atretic gallbladder. A modified Kasai portoenterostomy (KPE) with Roux-en-Y jejunojejunostomy was performed, and the infant was discharged with supportive care. However, seven months post-Kasai portoenterostomy, the infant presented with persistent jaundice and progressive deterioration of liver function, indicative of a failed Kasai procedure. Consequently, she was scheduled to undergo liver transplantation (LT) as a definitive treatment. BA is a rare disorder that is observed across nearly all ethnic groups, though the incidence rates vary significantly. This case highlights the efficacy of liver transplantation in treating failed Kasai procedures and demonstrates the potential for enhanced outcomes in infants with end-stage liver disease.

摘要

胆道闭锁(BA)是一种严重的肝胆疾病,由于胆管进行性炎症和瘢痕性梗阻而发生,对生命构成威胁。这种情况通常出现在婴儿身上,及时识别对于更好的预后至关重要。如果不进行治疗,患者将不可避免地出现肝损伤和死亡。本病例报告描述了一名9个月大的女婴,出现黄疸、巩膜黄染、皮肤发黄、无胆汁粪便和肝肿大。肝酶升高和肝胆亚氨基二乙酸(HIDA)扫描确诊为BA。组织病理学检查显示纤维化、胆汁淤积性疾病和闭锁胆囊。进行了改良的Kasai肝门空肠吻合术(KPE)并加做Roux-en-Y空肠空肠吻合术,婴儿在支持治疗下出院。然而,Kasai肝门空肠吻合术后7个月,婴儿出现持续黄疸和肝功能进行性恶化,提示Kasai手术失败。因此,她被安排接受肝移植(LT)作为最终治疗。BA是一种罕见疾病,几乎在所有种族中都有观察到,但其发病率差异很大。本病例突出了肝移植在治疗Kasai手术失败中的疗效,并证明了改善终末期肝病婴儿预后的潜力。

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1
Biliary Atresia: A Case Report.胆道闭锁:一例病例报告。
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2
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Am J Surg Pathol. 2021 Nov 1;45(11):1499-1508. doi: 10.1097/PAS.0000000000001803.

本文引用的文献

1
Biliary atresia.先天性胆道闭锁。
Nat Rev Dis Primers. 2024 Jul 11;10(1):47. doi: 10.1038/s41572-024-00533-x.
2
Evaluation of Perioperative Complications in the Management of Biliary Atresia.胆道闭锁治疗中围手术期并发症的评估
Front Pediatr. 2020 Aug 28;8:460. doi: 10.3389/fped.2020.00460. eCollection 2020.
3
Adjuvant treatments for biliary atresia.胆道闭锁的辅助治疗
Transl Pediatr. 2020 Jun;9(3):253-265. doi: 10.21037/tp.2016.10.08.