title: Doppler-defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years.
abstract: Pulmonary hypertension (PH) is associated with increased mortality in adults with sickle cell disease (SCD), but its prognostic significance in children is unknown. Eighty-eight children with SCD were followed after echocardiographic screening for PH. After a mean follow-up of 3 years, all 18 subjects with PH were alive. In our children, as in adults with SCD, PH was associated with increased haemolysis. In contrast, our subjects with PH did not have overt systemic disease observed in adults. PH may be a manifestation of progressive organ damage from chronic haemolysis and systemic vasculopathy that ultimately leads to early death in adulthood.
authors: Division of Pediatric Hematology, Columbia University College of Physicians and Surgeons, New York, NY, USA. ml653@columbia.edu
authors: Margaret T
authors: MT
authors: Lee
authors: Tania
authors: T
authors: Small
authors: Muhammad A
authors: MA
authors: Khan
authors: Erika B
authors: EB
authors: Rosenzweig
authors: Robyn J
authors: RJ
authors: Barst
authors: Gary M
authors: GM
authors: Brittenham
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality; D001803:Blood Transfusion; D002561:Cerebrovascular Disorders / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality; D007770:L-Lactate Dehydrogenase / Q000097:blood; D016015:Logistic Models; D008297:Male; D016017:Odds Ratio; D012306:Risk; D015996:Survival Rate; D017585:Ultrasonography, Doppler, Transcranial
chemical_list: D007770:L-Lactate Dehydrogenase