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Home > West Medicine > Surgery > Hepatobiliary Surgery

Classroom classification

百科

Alpha-1 antitrypsin deficiency liver disease

肝病

Common symptoms

出血倾向、肝脾肿大、上消化道出血、肝功能衰竭、静脉曲张

The pathology of disease

Alpha-1 antitrypsin deficiency liver disease is misfolded A1AT (PiZZ) accumulating in liver; neonatal cholestasis; adult cirrhosis; hepatocellular carcinoma; also emphysema; liver transplant.

Symptoms

Neonatal jaundice; adult cirrhosis; hepatomegaly; emphysema; family history; PiZZ genotype; elevated liver enzymes

Causes

SERPINA1 mutation; PiZZ; misfolded A1AT accumulation; liver injury.

Treatment

Supportive; avoid alcohol/smoking; liver transplant for decompensation; A1AT augmentation for lung; hepatology/genetics

Dept

Hepatology / pulmonology / genetics.

Warm reminder: Health education reference only; seek care at regular hospital.

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肝病Overview of the Western Medical Room