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The Effects of Hydroxyurea Therapy on the Six-Minute Walk Distance in Patients with Adult Sickle Cell Anemia: An Echocardiographic Study

Authors Garadah T, Mandeel F, Jaradat A, Bin Thani K

Received 1 February 2019

Accepted for publication 16 October 2019

Published 27 December 2019 Volume 2019:10 Pages 443—452


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Dr Martin H Bluth

Taysir Garadah,1,2 Fatema Mandeel,2 Ahmed Jaradat,1 Khalid Bin Thani1,2

1Medical Department, College of Medicine and Medical Sciences, Arabian Gulf University, Manama, Kingdom of Bahrain; 2Medical Department, Cardiac Unit, Salmanyia Medical Complex, Manama, Kingdom of Bahrain

Correspondence: Taysir Garadah
Medical Department, College of Medicine and Medical Sciences, Arabian Gulf University, P.O. Box 26671, Manama, Kingdom of Bahrain
Tel +973-17239681
Fax +973-17230730

Background: The impact of hydroxyurea (HU) medication as treatment of choice has not been evaluated in adult sickle cell anemia (SCA) patients in terms of the 6-min walk distance (6 MWD).
Aim: The aim of the study was evaluating the effects of HU on the 6 MWD, serum brain natriuretic peptide (NT-pro BNP) level, and pulmonary hypertension (PH) measured by tricuspid regurgitation velocity (TRV).
Methods: In this cross-sectional, prospective study, 110 patients with homozygous SCA were studied and compared with age- and gender-matched healthy controls. Every patient was investigated via pulsed and tissue Doppler echo evaluation, 6-min walk test (6 MWT), and blood level for the level of NT-pro-BNP hormone. Data were compared in patients with (n = 59; group 1, G1) and without (n = 51; group 2, G2) HU medication. Pearson correlation analysis was applied and clinical follow-up for the frequency of acute chest syndrome (ACS). Analysis of variance (ANOVA) multivariate statistical analysis was applied between groups.
Results: In the study, 110 patients with SCA were studied and compared with 110 control patients. Patients in G1 compared with G2 had a longer 6 MWD (491 ± 64.4 m vs 428.6 ± 54.3 m, p < 0.005), higher HbF% (21 ± 2.5% vs 8 ± 1.8%, p < 0.005), and lower NT-pro-BNP level (314.1 ± 27.5 pmol/L vs 407 ± 18.9 pmol/L, p = 0.05). The mean TRV values were 2.8 ± 0.5 m/s in G1 versus 3.4 ± 0.4 m/s in G2, p < 0.005, and 1.5 ± 0.7 m/s in the control group. The high probability of PH based on a TRV > 3.4 m/s was 10.1% in G1 versus 17.6% in G2 and 3.6% in the control. There were weak positive correlations between NT-pro-BNP and TRV (r = 0.264; p = 0.005) and HbF% and 6 MWD (r = 0.452; p = 0.001). After 12 months of follow-up, frequency of acute chest syndrome (ACS) was twice as high in G2, at 32 patients, versus 16 in G1.
Conclusion: Patients with SCA on HU medication compared with no HU had significantly longer 6 MWD, lower level of NT-pro-BNP, higher HbF% level. After 1-year follow-up HU patients had less frequency of ACS. There were significant positive correlations between the level of NT-pro BNP level and TRV in m/s on echo.

Keywords: sickle cell anemia, 6-min walk test, hydroxyurea, NT-pro BNP, echocardiography, diastolic dysfunction, pulmonary hypertension

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