TY  - CHAP
M1  - Book, Section
TI  - Chapter 518. Pulmonary Alveolar Proteinosis
A1  - Trapnell, Bruce C.
A1  - Young, Lisa R.
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  - Pulmonary  alveolar  proteinosis  (PAP)  is  a  syndrome  characterized  by  the  accumulation  of  surfactant  lipids  and  proteins  within  the  pulmonary  alveoli,  resulting  in  impaired  gas  exchange  and  respiratory  insufficiency.1  Our  understanding  of  PAP  has  advanced  significantly  over  the  past  several  decades  due  to  a  series  of  contributions  from  basic,  clinical,  and  translational  research.2,3  These  studies  have  revealed  a  critical  role  for  pulmonary  granulocyte/macrophage-colony  stimulating  factor  (GM-CSF)  in  the  terminal  differentiation  of  alveolar  macrophages  and  in  alveolar  macrophage-mediated  surfactant  catabolism,  pulmonary  surfactant  homeostasis,  and  lung  host  defense.4-6  PAP  comprises  part  of  a  larger  group  of  disorders  associated  with  disruption  of  surfactant  homeostasis  and  includes  disorders  of  surfactant  production  (hereafter  referred  to  as  pulmonary  surfactant  metabolic  dysfunction  disorders)  and  disorders  of  surfactant  clearance  (hereafter  referred  to  as  PAP;  Table  518-1).  The  distinct  epidemiological,  pathogenic,  clinical,  and  prognostic  features  of  these  two  disease  categories  indicate  they  are  usually  considered  separately  rather  than  as  a  continuum  of  a  single  disease  process.  PAP  can  be  further  divided  into  primary  and  secondary  PAP,  which,  respectively,  are  associated  with  either  loss  of  GM-CSF  signaling  or  the  presence  of  an  underlying  disorder  that  reduces  alveolar  macrophage  numbers  or  functions.  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/20
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=7052114
ER  -