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Delayed diagnosis of alpha-1-antitrypsin deficiency following post-hepatectomy liver failure: A case report

World Journal of Gastroenterology 2016年第11期22卷 3289-3295页

作者： Benjamin Norton Jemimah Denson Christopher Briggs Matthew Bowles David Stell Somaiah Aroori Peninsula College of Medicine and Dentistry Tamar Science Park Department of Histopathology Plymouth Hospitals NHS Trust Peninsula HPB Unit Derriford Hospital

Post-hepatectomy liver failure(PHLF) is a leading cause of morbidity and mortality following major liver resection. The development of PHLF is dependent on the volume of the remaining liver tissue and hepatocyte function. Without effective pre-operative assessment, patients with undiagnosed liver disease could be at increased risk of PHLF. We report a case of a 60-year-old male patient with PHLF secondary to undiagnosed alpha-1-antitrypsin deficiency(AATD) following major liver resection. He initially presented with acute large bowel obstruction secondary to a colorectal adenocarcinoma, which had metastasized to the liver. There was no significant past medical history apart from mild chronic obstructive pulmonary disease. After colonic surgery and liver directed neo-adjuvant chemotherapy, he underwent a laparoscopic partially extended right hepatectomy and radio-frequency ablation. Post-operatively he developed PHLF. The cause of PHLF remained unknown, prompting reanalysis of the histology, which showed evidence of AATD. He subsequently developed progressive liver dysfunction, portal hypertension, and eventually an extensive parastomal bleed, which led to his death; this was ultimately due to a combination of AATD and chemotherapy. This case highlights that formal testing for AATD in all patients with a known history of chronic obstructive pulmonary disease, heavy smoking, or strong family history could help prevent the development of PHLF in patients undergoing major liver resection.

关键词： Post-hepatectomy liver failure Alpha-1-antitrypsin deficiency Hepatectomy functional liver remnant liver resection

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