PMID- 27004008
OWN - NLM
STAT- MEDLINE
DCOM- 20170117
LR  - 20181113
IS  - 2219-2840 (Electronic)
IS  - 1007-9327 (Print)
IS  - 1007-9327 (Linking)
VI  - 22
IP  - 11
DP  - 2016 Mar 21
TI  - Delayed diagnosis of alpha-1-antitrypsin deficiency following post-hepatectomy 
      liver failure: A case report.
PG  - 3289-95
LID - 10.3748/wjg.v22.i11.3289 [doi]
AB  - 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 re-analysis 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.
FAU - Norton, Benjamin
AU  - Norton B
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
FAU - Denson, Jemimah
AU  - Denson J
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
FAU - Briggs, Christopher
AU  - Briggs C
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
FAU - Bowles, Matthew
AU  - Bowles M
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
FAU - Stell, David
AU  - Stell D
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
FAU - Aroori, Somaiah
AU  - Aroori S
AD  - Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, 
      Plymouth PL6 8BU, United Kingdom.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - World J Gastroenterol
JT  - World journal of gastroenterology
JID - 100883448
RN  - alpha-1-Antitrypsin Deficiency, Autosomal Recessive
SB  - IM
MH  - Adenocarcinoma/diagnostic imaging/drug therapy/secondary/*surgery
MH  - Biopsy
MH  - Chemotherapy, Adjuvant/adverse effects
MH  - Colorectal Neoplasms/*pathology
MH  - *Delayed Diagnosis
MH  - Fatal Outcome
MH  - Hepatectomy/*adverse effects
MH  - Humans
MH  - Liver Failure, Acute/diagnosis/*etiology
MH  - Liver Neoplasms/diagnostic imaging/drug therapy/secondary/*surgery
MH  - Male
MH  - Middle Aged
MH  - Risk Factors
MH  - Tomography, X-Ray Computed
MH  - Treatment Outcome
MH  - alpha 1-Antitrypsin Deficiency/complications/*diagnosis
PMC - PMC4790006
OTO - NOTNLM
OT  - Alpha-1-antitrypsin deficiency
OT  - Functional liver remnant
OT  - Hepatectomy
OT  - Liver resection
OT  - Post-hepatectomy liver failure
EDAT- 2016/03/24 06:00
MHDA- 2017/01/18 06:00
PMCR- 2016/03/21
CRDT- 2016/03/23 06:00
PHST- 2015/05/24 00:00 [received]
PHST- 2015/09/05 00:00 [revised]
PHST- 2015/11/30 00:00 [accepted]
PHST- 2016/03/23 06:00 [entrez]
PHST- 2016/03/24 06:00 [pubmed]
PHST- 2017/01/18 06:00 [medline]
PHST- 2016/03/21 00:00 [pmc-release]
AID - 10.3748/wjg.v22.i11.3289 [doi]
PST - ppublish
SO  - World J Gastroenterol. 2016 Mar 21;22(11):3289-95. doi: 10.3748/wjg.v22.i11.3289.