Pulmonary hypertension in patients with sickle cell disease: a longitudinal study.

摘要: Summary Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development PHT and factors that affect progression are unknown. We observed 93 over median follow-up period 2·6 years (range 0·2–5·1 years). Data were censored at time death or loss to follow-up. Pulmonary was associated an increased risk (relative risk, 9·24; 95% confidence interval: 1·2–73·3; P = 0·01). There no difference when different degrees compared. Lactate dehydrogenase blood urea nitrogen significantly logistic regression model. Higher levels fetal haemoglobin treatment hydroxycarbamide more frequently without PHT. Thirteen per cent previous evidence developed following 3 years observation. In conclusion: (1) PHT, regardless severity, SCD patients; (2) haemolysis strongly SCD; (3) high therapy may decrease occurrence PHT; (4) screening for indicated their non-crisis, steady states.