Document Detail

Impaired surfactant protein b synthesis in infants with congenital diaphragmatic hernia.
MedLine Citation:
PMID:  22700843     Owner:  NLM     Status:  Publisher    
Pulmonary hypoplasia and hypertension account for significant morbidity and mortality in neonates with congenital diaphragmatic hernia (CDH). Whether CDH is associated with surfactant dysfunction remains controversial. Therefore, we measure disaturated phosphatidylcholine and surfactant protein B concentration in tracheal aspirates and their synthesis rate in infants with CDH compared to infants without lung disease.(2)H2O as precursor of disaturated-phosphatidylcholine and 1(13)C-leucine as precursor of surfactant protein B were administered to 13 infants with CDH and 8 GA-matched controls. Disaturated-phosphatidylcholine and surfactant protein B were isolated from tracheal aspirates, and their fractional synthesis rate was derived from (2)H and (13)C enrichment curves obtained by mass spectrometry. Disaturated-phosphatidylcholine and surfactant protein B amounts in tracheal aspirates were also measured.In infants with CDH, surfactant protein B fractional synthesis rate and amount were 62±27 % and 57±22 % lower than the value found in infants without lung disease (p<0.01 and p<0.05). There were no significant group differences in disaturated phosphatidylcholine fractional synthesis rate and amount.Infants with CDH have a lower rate of synthesis of SP-B and less SP-B in tracheal aspirates. In these infants, partial SP-B deficiency could contribute to the severity of respiratory failure and its correction might represent a therapeutic goal.
P E Cogo; M Simonato; O Danhaive; G Verlato; G Cobellis; F Savignoni; D Peca; A Baritussio; V P Carnielli
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-14
Journal Detail:
Title:  The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology     Volume:  -     ISSN:  1399-3003     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Bambino Gesù Children's Hospital, Rome, Italy.
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