| Original language | English |
|---|---|
| Pages (from-to) | 297-302 |
| Number of pages | 6 |
| Journal | Journal of Pediatrics |
| Volume | 120 |
| Issue number | 2 PART 1 |
| DOIs | |
| State | Published - Feb 1992 |
ASJC Scopus Subject Areas
- Pediatrics, Perinatology, and Child Health
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In: Journal of Pediatrics, Vol. 120, No. 2 PART 1, 02.1992, p. 297-302.
Research output: Contribution to journal › Article › peer-review
}
TY - JOUR
T1 - Congenital pulmonary alveolar proteinosis
T2 - Failure of treatment with extracorporeal life support
AU - Moulton, Steven L.
AU - Krous, Henry F.
AU - Merritt, T. Allen
AU - Odell, Richard M.
AU - Gangitano, Ernesto
AU - Cornish, J. Devn
N1 - Funding Information: Pulmonary alveolar proteinosis is a rare pulmonary disease characterized by an overproduction of surface active material composed of phospholipids and lipoprotein complexes. These components coalesce into insoluble microparticles within the alveoli and smaller airways of affected individuals, causing impaired gas exchange and progressive hypoxemia. The onset of the disease in older children and adults is usually insidious, and the disease is thought to represent a nonspecific response to alveolar injury.1 At presentation, infants usually have failed to thrive and have had vomiting, diarrhea, or pyogenie infections. Respiratory symptoms, although initially not prominent, tend to progress and ultimately account for the majority of deaths. 2 Supported by the Foundation for Surgical Education, University of California, San Diego. Submitted for publication March 11, 1991; accepted Sept. 6, 1991. Reprint requests: J. Devn Cornish, MD, Divisiono f Neonatai-Perinatal Medicine, Pediatric Administration Building, 2040 Ridge-wood Dr., NE, Atlanta, GA 30322. 9/23/33581 In newborn infants the pulmonary symptoms are severe and persistent. Shortly after birth, these neonates have progressive respiratory distress requiring early tracheal intubation and mechanical ventilation. Three cases of PAP in which respiratory distress was present at birth have been reported. TM All three neonates subsequently died, one after a 7-day course of extracorporeal life support (unpublished data: Extracorporeal Life Support Organization Registry,
PY - 1992/2
Y1 - 1992/2
UR - http://www.scopus.com/inward/record.url?scp=0026542636&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0026542636&partnerID=8YFLogxK
U2 - 10.1016/S0022-3476(05)80448-2
DO - 10.1016/S0022-3476(05)80448-2
M3 - Article
C2 - 1341413
AN - SCOPUS:0026542636
SN - 0022-3476
VL - 120
SP - 297
EP - 302
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 2 PART 1
ER -