Internal Medicine

P1: SBT

0521779407-03 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 19:6

Alpha-1-Antitrypsin Alzheimer’s Disease 101

management

What to Do First

■Evaluate severity of liver and lung disease and complications

General Measures

■Adjust or avoid potentially hepatotoxic drugs

■Discourage smoking to slow progression of lung disease

■Assess status of liver and lung disease

■Check serum alpha-1-AT concentration & phenotype

specific therapy

■Liver transplantation: treats progressive hepatic dysfunction; should

perform before pulmonary disease precludes surgery

follow-up

Routine

■Liver biochemistry, INR, CBC every 6–12 months

■Chest roentgenogram & pulmonary function test yearly in patients

with evidence of lung disease

complications and prognosis

■Cirrhosis: treat as per liver failure due to other causes

■HCC: can screen with serum AFP and ultrasound every 6 months

■Emphysema: avoidance of smoking most important; can consider

alpha-1-antitrypsin replacement therapy and/or lung transplanta-

tion for severe lung disease

Prognosis

■90% 1-year and 80% 5-year survival rates among children after liver

transplantation

Alzheimer’s Disease..................................

CHAD CHRISTINE, MD

history & physical

■Acquired, generalized impairment of cognitive function

■Slowly progressive disorder (typically over years)

■Rare hereditary forms

■Not assoc w/ alteration of level of consciousness

■May be personality changes

■Immediate recall intact, but delayed recall & recent memory impaired