What question did this study set out to answer?

To determine the role of epithelial sodium channel-mediated active sodium absorption in pulmonary physiology, airway surface liquid regulation, and airway host defense.

July 15, 1999New England Journal of MedicineOpen Access

Pulmonary Epithelial Sodium-Channel Dysfunction and Excess Airway Liquid in Pseudohypoaldosteronism

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Authors

EKEitan KeremHadassah Medical Center TBTzvy BistritzerTel Aviv University AHAaron HanukogluTel Aviv University

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Implication

Observational study reveals increased airway surface liquid in pseudohypoaldosteronism, indicating that epithelial sodium channels regulate pulmonary liquid volume.

Key Points

To determine the role of epithelial sodium channel-mediated active sodium absorption in pulmonary physiology, airway surface liquid regulation, and airway host defense.
Evaluated 9 patients aged 1.5 to 22 years with systemic pseudohypoaldosteronism for epithelial sodium channel mutations, airway sodium transport rates, and airway surface liquid volume and composition.
Assessed clinical pulmonary features and measured mucociliary clearance in 3 adult patients compared with 12 normal subjects.
Patients demonstrated loss-of-function epithelial sodium channel mutations, absent airway sodium absorption, and airway surface liquid volumes exceeding twice the normal value.
The mean (±SE) mucociliary transport rate was significantly elevated in 3 adult patients compared with 12 normal controls (2.0 ± 0.7 vs. 0.5 ± 0.3 percent per minute, P=0.009).
Patients 5 years of age or younger had recurrent chest congestion, coughing, and wheezing without Staphylococcus aureus or Pseudomonas aeruginosa infections, whereas older patients experienced fewer respiratory symptoms.

Cite This Study

Kerem et al. (1999) studied this question.

synapsesocial.com/papers/6a0e576f8561d4cf804c291a https://doi.org/10.1056/nejm199907153410304

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Implication

Observational study reveals increased airway surface liquid in pseudohypoaldosteronism, indicating that epithelial sodium channels regulate pulmonary liquid volume.

Pulmonary Epithelial Sodium-Channel Dysfunction and Excess Airway Liquid in Pseudohypoaldosteronism

Discussion

Member takes

Implication

Key Points

Cite This Study

Pulmonary Epithelial Sodium-Channel Dysfunction and Excess Airway Liquid in Pseudohypoaldosteronism

Discussion

Member takes

Implication

Key Points

Cite This Study

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