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Article

Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, 'PiEE'.

BMJ case reports - 28 Mar 2018

Reilkoff Ronald, Stephenson Laurel

Abstract excerpt

Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to...

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Share to alpha 1-Antitrypsin
Share to Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 29592975
DOI: 10.1136/bcr-2017-222036
PMCID: PMC5878378

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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