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April 1968

Ventilation-Blood Flow Relationships in Cystic Fibrosis: Pulmonary "Claustration"

Author Affiliations

New Orleans
From the departments of pediatrics and physiology, Tulane University School of Medicine, and from the Charity Hospital of Louisiana, New Orleans.

Am J Dis Child. 1968;115(4):420-427. doi:10.1001/archpedi.1968.02100010422003

A FACT supported by auscultatory, roentgenographic, and pulmonary physiologic observations is that the lungs of children with cystic fibrosis (CF) are not affected uniformly. Rales and suppressed breath sounds may persist in a given bronchopulmonary segment or lobe for months or years. Plugging of bronchi with mucus and subsequent cystic bronchiectasis may be confined to certain areas, while the rest of the lung remains apparently unaffected.1 Such localization of bronchopulmonary disease has permitted lobar or segmental surgical resection in some patients.2 Inspired air is usually unevenly distributed, as determined by nitrogen wash-out studies,3 indicating that certain areas of the lung receive less ventilation than others. Moreover, recent work with pulmonary scanning techniques has shown that, as in emphysema in adults,4 striking shifts can occur in the distribution of pulmonary arterial blood flow in CF (personal communication, M. D. Wilhide and A. Spock).

This report concerns observations

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