PMID: 7337330Jan 1, 1981Paper

Drepanocytosis and diffuse interstitial pulmonary fibrosis (author's transl)

Annales de médecine interne
J RaisonG Delzant

Abstract

Respiratory disorders in homozygous drepanocytosis and double SC heterozygosis are mainly dependent on two factors : repeated infections with, more particularly, pneumococcus or mycoplasma, and epidoses of occlusion of the pulmonary circulation. Mutual reinforcement of these two factors occurs, in so far as the relative hypoxia of an infected lung increases the risk of falciform and thrombosis formation in the pulmonary arterioles. The particular physical and chemical properties of the drepanocyte red cell, and the anaemia, themselves lead to parallel disturbances in pulmonary circulation function and gas exchanges. Possible development of respiratory insufficiency is generally, therefore, the result of chronic pulmonary arterial hypertension. However, the results of respiratory function tests are often fairly analogous to those observed in moderate interstitial fibrosis : reduction in vital capacity, alveolo-capillary block, reduction in CO diffusion space, a shunt effect, and diminished pulmonary compliance. This problem is discussed in relation to findings of diffuse interstitial fibrosis in a 34-year-old man with double SC heterozygosis, who had numerous episodes of bone, abdominal, and pulmonary microinfarcts. The presence...Continue Reading

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