alpha-1-antitrypsin deficiency

MeSH: D019896ORPHA: 60

Overview

genetic disorder that may result in lung disease or liver disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with alpha-1-antitrypsin deficiency, sourced from HPO and Orphanet clinical annotations.

EmphysemaBronchiectasisReduced circulating alpha-1-antitrypsin concentrationJaundiceHepatic fibrosisElevated circulating hepatic transaminase concentrationProlonged neonatal jaundiceNeonatal unconjugated hyperbilirubinemiaHepatitisBronchitisDecreased DLCOIntrahepatic inclusion bodiesCirrhosisCholestasisPortal hypertensionFailure to thrive in infancyAsthmaChronic pulmonary obstructionCytoplasmic antineutrophil antibody positivityHepatocellular carcinomaPanniculitis

Classification & Codes

MeSH Code

D019896

Orphanet Code

ORPHA:60
alpha-1-antitrypsin deficiency
MeSHD019896
OrphanetORPHA:60
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO