TY - CHAP M1 - Book, Section TI - Chapter 516. Alpha-1 Antitrypsin Deficiency Lung Disease A1 - Flotte, Terence R. A1 - MüEller, Christian A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. PY - 2011 T2 - Rudolph's Pediatrics, 22e AB - Alpha1-antitrypsin (AAT) circulates in the plasma as a 52-kD glycoprotein. It is synthesized primarily in hepatocytes and to a lesser extent in macrophages and monocytes. AAT is produced at a basal level, which results in plasma concentrations of 11 micromole or greater. AAT is also an acute phase reactant, with levels increasing dramatically during periods of stress, fever, or infection.1-6 Some individuals with AAT deficiency manifest with neonatal liver disease, as discussed in Chapter 421. This chapter discusses the pulmonary manifestations of AAT deficiency. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=7052010 ER -