Low pulmonary expression of epithelial Na(+) channel and Na(+), K(+)-ATPase in newborn infants with congenital diaphragmatic hernia

Neonatology. 2011;99(1):14-22. doi: 10.1159/000292503. Epub 2010 Jun 30.


Background: It has been suggested from several animal studies and clinical observations that congenital diaphragmatic hernia (CDH) with pulmonary hypoplasia is accompanied by a disturbed perinatal ion transport. This could lead to respiratory distress due to slower clearance of fetal lung fluid at birth.

Objectives: The purpose of this study was to determine whether CDH is related to changes in the expression of three rate-limiting transporter proteins in lung epithelium at birth.

Methods: Tracheal aspirate was collected from 12 newborn infants with CDH and from 8 newborn control patients. Sampling was performed at postnatal age 18 and at 43 h in the CDH group and at 18 h in the control group. The protein abundance of α-, β- and γ-epithelial Na(+) channel (ENaC), aquaporin 5 and Na(+), K(+)-ATPase α(1) was analyzed using semiquantitative immunoblotting.

Results: The levels of β-ENaC, γ-ENaC and Na(+), K(+)-ATPase α(1) collected at 18 h postnatally were significantly lower in CDH infants compared to control infants. In the CDH group, no significant difference in the expression of the ENaC subunits, Na(+), K(+)-ATPase α(1) or aquaporin 5 could be detected between the two sampling time points.

Conclusions: This downregulation may result in an abnormal lung fluid absorption which could be an important mechanism behind the respiratory distress seen in newborn CDH patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple
  • Aquaporin 5 / analysis
  • Aquaporin 5 / metabolism*
  • Bronchoalveolar Lavage Fluid / chemistry
  • Down-Regulation
  • Epithelial Sodium Channels / analysis
  • Epithelial Sodium Channels / metabolism*
  • Female
  • Gestational Age
  • Hernia, Diaphragmatic / enzymology*
  • Hernia, Diaphragmatic / pathology
  • Hernias, Diaphragmatic, Congenital
  • Humans
  • Infant, Newborn
  • Lung / enzymology*
  • Lung / pathology
  • Male
  • Respiration, Artificial
  • Respiratory Mucosa / enzymology*
  • Respiratory Mucosa / pathology
  • Sodium-Potassium-Exchanging ATPase / analysis
  • Sodium-Potassium-Exchanging ATPase / metabolism*


  • AQP5 protein, human
  • Aquaporin 5
  • Epithelial Sodium Channels
  • Sodium-Potassium-Exchanging ATPase