Pulmonary hypertension in children and adolescents with sickle cell disease.

Full Abstract

The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV > or =2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV > or =3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD.

Author information

Author/s: Onyekwere, O C (OC); Campbell, A (A); Teshome, M (M); Onyeagoro, S (S); Sylvan, C (C); Akintilo, A (A); Hutchinson, S (S); Ensing, G (G); Gaskin, P (P); Kato, G (G); Rana, S (S); Kwagyan, J (J); Gordeuk, V (V); Williams, J (J); Castro, O (O);

Affiliation: Center for Sickle Cell Disease, Howard University, Washington, DC, USA. oonyekwere(-atsign-)howard.edu

Journal and publication information

Publication Type: Comparative Study; Journal Article; Multicenter Study

Journal: Pediatric cardiology (Pediatr Cardiol), published in United States. (Language: eng)

Reference: 2008-Mar; vol 29 (issue 2) : pp 309-12

Dates: Created 2008/03/14; Completed 2008/07/22;

PMID: 17680298, status: MEDLINE (last retrieval date: 2/18/2009, IMS Date: )

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

External Links for this article
(including full text providers, if available):

Click Electronic Full-text Provider Links to see options for finding the electronic full text links to this article. Note there may be a subscription or fee required for access to the full text. See our FAQ for information on finding FREE full text articles.

This article may also be located in paper journal collections available in many libraries. Use the Journal and Publication Information above to find the full article.