Expression of Phenotype in Alpha-1 Antitrypsin Deficiency
Phenotype | Alpha-1 Antitrypsin Serum Level* | Risk of Emphysema |
|---|---|---|
PI*ZZ | 20–45 mg/dL (2.5–7 micromol/L) | High |
PI*MZ | 90–210 mg/dL (17–33 micromol/L) | Minimally increased |
PI*SZ | 75–120 mg/dL (8–16 micromol/L) | Slightly increased |
PI*SS | 100–200 mg/dL (15–33 micromol/L) | Minimally increased |
PI*MM | 150–350 mg/dL (20–48 micromol/L) | Normal |
* Serum levels given are measured using a typical commercial standard (mg/dL) and the purified standard (micromol/L) used in the U.S. Registry. | ||
Data from American Thoracic Society; European Respiratory Society: American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 168(7):818–900, 2003. doi:10.1164/rccm.168.7.818 | ||