[Respiratory function in patients with sickle cell disease (author's transl)]

Respiratory function was systematically studied in 61 patients with sickle cell disease, in and out of crisis. Pulmonary volumes and flows, blood gases and P50 were measured. It was found that : 1. vital capacity was decreased in about half of the patients (obstructive symptoms in five cases), 2. hypoxaemia existed under air, with a fall in hemoglobin saturation, 3. hypoxaemia existed under pure O2 showing the existence of veno-arterial shunts, 4. an increase in P50 revealed a decrease in haemoglobin oxygen affinity (P50 (7.40, 37 degrees C) torr = 49.08 -- 1.57 [Hb] (g/100 ml) +/- 4.81), 5. during sickle cell crisis, there was evidence of obstructive symptoms associated with a Pao2 decrease and a moderate alveolar hypoventilation. Several hypotheses concerning the restrictive syndrome and the hypoxaemia are discussed.