Pulmonary hypertension in children and adolescents with sickle cell disease.
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.
Onyekwere
O C
OC
Center for Sickle Cell Disease, Howard University, Washington, DC, USA. oonyekwere@howard.edu
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
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001783:Blood Flow Velocity; D002648:Child; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006400:Hematocrit; D006451:Hemoglobin, Sickle / Q000378:metabolism; D006461:Hemolysis / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D016017:Odds Ratio; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D012307:Risk Factors; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D014481:United States / Q000453:epidemiology
D015415:Biomarkers; D006451:Hemoglobin, Sickle; D007770:L-Lactate Dehydrogenase; D001663:Bilirubin