Document Detail

Pulmonary hypertension in children and adolescents with sickle cell disease.
MedLine Citation:
PMID:  17680298     Owner:  NLM     Status:  MEDLINE    
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.
O C Onyekwere; A Campbell; M Teshome; S Onyeagoro; C Sylvan; A Akintilo; S Hutchinson; G Ensing; P Gaskin; G Kato; S Rana; J Kwagyan; V Gordeuk; J Williams; O Castro
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Publication Detail:
Type:  Comparative Study; Journal Article; Multicenter Study     Date:  2007-08-07
Journal Detail:
Title:  Pediatric cardiology     Volume:  29     ISSN:  0172-0643     ISO Abbreviation:  Pediatr Cardiol     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-03-14     Completed Date:  2008-07-22     Revised Date:  2011-10-19    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  309-12     Citation Subset:  IM    
Center for Sickle Cell Disease, Howard University, Washington, DC, USA.
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MeSH Terms
Age Factors
Anemia, Sickle Cell / blood,  complications*,  ultrasonography
Bilirubin / blood
Biological Markers / blood
Blood Flow Velocity
Echocardiography, Doppler
Follow-Up Studies
Hemoglobin, Sickle / metabolism
Hemolysis / physiology
Hypertension, Pulmonary / epidemiology,  etiology*,  physiopathology
L-Lactate Dehydrogenase / blood
Odds Ratio
Prospective Studies
Pulmonary Wedge Pressure / physiology
Risk Factors
Tricuspid Valve Insufficiency / etiology,  physiopathology,  ultrasonography
United States / epidemiology
Grant Support
Reg. No./Substance:
0/Biological Markers; 0/Hemoglobin, Sickle; 635-65-4/Bilirubin; EC Dehydrogenase

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