RT Book, Section
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.
SR Print(0)
ID 7052010
T1 Chapter 516. Alpha-1 Antitrypsin Deficiency Lung Disease
T2 Rudolph's Pediatrics, 22e
YR 2011
FD 2011
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-149723-7
LK accesspediatrics.mhmedical.com/content.aspx?aid=7052010
RD 2024/04/24
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.