&lt;p&gt;The Effects of Hydroxyurea Therapy on the Six-Minute Walk Distance in Patients with Adult Sickle Cell Anemia: An Echocardiographic Study&lt;/p&gt;

Garadah, Taysir S.; Mandeel, Fatema H.; Jaradat, Ahmed; K, Bin Thani

doi:10.2147/jbm.s203828

Cited by 3 publications

(4 citation statements)

References 40 publications

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“…Figure 2 d shows the measurement of Tricuspid regurgitant velocities in SCD patients [ 50 , 61 – 66 ]. The pooled estimates show a significant reduction in TRV post-hydroxyurea treatment with a standardized mean difference of – 1.37(CI − 2·31, − 0.42; p = 0.004).…”

Section: Resultsmentioning

confidence: 99%

“…Post hydroxyurea therapy of 20 mg/kg/day, 2(6.9%) developed new AVN. Five (50%) of SCD patients who were on hydroxyurea for more than 5 years had the worst AVN hip joint [ 66 , 67 ]. In another prospective study by Kris M. Mahadeo et al among 257 SCD patients screened for osteonecrosis of the femoral head, the prevalence of avascular necrosis of the hip joint who were on hydroxyurea therapy was 18(21.68%) which was higher compared to the prevalence of AVN who were not on hydroxyurea therapy 8(8.08%) [ 68 ].…”

Section: Resultsmentioning

confidence: 99%

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Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis

Khargekar,

Banerjee,

Athalye

et al. 2024

Syst Rev

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Background Hydroxyurea is an affordable drug that reduces vaso-occlusive crises and transfusion requirements in sickle cell disease. However, its effectiveness in preventing chronic organ damage is still unclear. This systematic review and meta-analysis aimed to evaluate the role of hydroxyurea in preventing organ morbidity. Method We included original articles published in English from 1st January 1990 to 31st January 2023, reporting hydroxyurea therapy and organ damage from PubMed, Google Scholar, Scopus, and CrossRef databases. A total of 45 studies with 4681 sickle cell disease patients were evaluated for organ damage. Results Our analysis showed that hydroxyurea intervention significantly lowered transcranial Doppler and tricuspid regurgitant velocity, with a standardized mean difference of − 1.03 (− 1.49; − 0.58); I2 = 96% and − 1.37 (CI − 2.31, − 0.42); I2 = 94%, respectively. Moreover, the pooled estimate for albuminuria showed a beneficial effect post-hydroxyurea therapy by reducing the risk of albuminuria by 58% (risk ratio of 0.42 (0.28; 0.63); I2 = 28%). Conclusion Our study found that a hydroxyurea dose above 20 mg/kg/day with a mean rise in HbF by 18.46% post-hydroxyurea therapy had a beneficial role in reducing transcranial doppler velocity, tricuspid regurgitant velocity, albuminuria, and splenic abnormality. Systematic review registration PROSPERO CRD42023401187.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis

Khargekar,

Banerjee,

Athalye

et al. 2024

Syst Rev

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“…There is conflicting data regarding the effect of disease‐modifying therapies (DMT) on diastolic function 13–15,30–32 . While some studies showed hydroxyurea therapy 15,30 or monthly erythrocyte transfusions 14 to be associated with less diastolic dysfunction, other studies did not replicate it 13,14,31,32 .…”

Section: Introductionmentioning

confidence: 99%

“…6,29 There is conflicting data regarding the effect of disease-modifying therapies (DMT) on diastolic function. [13][14][15][30][31][32] While some studies showed hydroxyurea therapy 15,30 or monthly erythrocyte transfusions 14 to be associated with less diastolic dysfunction, other studies did not replicate it. 13,14,31,32 However, most prior studies were limited by their cross-sectional design, small sample size, selection bias, nonuniform duration of treatment with disease-modifying therapies, and concomitant use of other clinical therapies, confounding the outcomes.…”

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confidence: 99%

Longitudinal effect of disease‐modifying therapy on left ventricular diastolic function in children with sickle cell anemia

et al. 2023

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Cardiac abnormalities seen in sickle cell anemia (SCA) include diastolic dysfunction, which has been shown to be associated with high morbidity and early mortality. The effect of disease‐modifying therapies (DMT) on diastolic dysfunction is poorly understood. We prospectively evaluated the effects of hydroxyurea and monthly erythrocyte transfusions on diastolic function parameters over 2 years. A total of 204 subjects with HbSS or HbSβ0‐thalassemia (mean age 11 ± 3.7 years), unselected for disease severity, had diastolic function assessed with surveillance echocardiograms twice, 2 years apart. During this 2‐year observation period, 112 participants received DMTs (hydroxyurea, n = 72, monthly erythrocyte transfusions, n = 40), 34 initiated hydroxyurea, and 58 did not receive any DMT. The entire cohort showed an increase in left atrial volume index (LAVi) of 3.40 ± 10.86 mL/m2, p = .001 over 2 years. This increase in LAVi was independently associated with anemia, high baseline E/e′ or LV dilation. Individuals not exposed to DMT were younger (mean age 8.8 ± 2.9 years), but at baseline their prevalence of abnormal diastolic parameters was similar to that of the DMT‐exposed participants who were older (mean age 12 ± 3.8 years). Participants on DMTs saw no improvement in diastolic function over the study period. In fact, participants on hydroxyurea saw a possible worsening in diastolic parameters (14% increase in LAVi and ~5% decrease in septal e′) but also a ~9% decrease in fetal hemoglobin (HbF) levels. Further studies are needed to evaluate if exposure to DMT for a longer duration or achieving higher HbF might be beneficial in alleviating diastolic dysfunction.

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Exercise Capacity and Biomarkers Among Children and Adolescents With Sickle Cell Disease

Silva

Jesus

Bessa

et al. 2023

Pediatric Exercise Science

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Background: Sickle cell disease is the most common genetic hemoglobinopathy globally and systemically affects body functioning, decreasing exercise capacity. Objective: To assess exercise capacity through the 6-minute walk test (6MWT) and biomarkers in children and adolescents with sickle cell disease. Materials and Methods: Cross-sectional study involving 20 children and adolescents from Brazil. Demographic and socioeconomic data were obtained. Baseline measurements included biomarkers (red blood cells, hemoglobin, hematocrit, white blood cells, platelets, reticulocytes, lactate dehydrogenase, creatine phosphokinase, C-reactive protein, interleukin 6, and fetal hemoglobin). The following data were obtained before, during, and after the 6MWT: heart rate, blood pressure, and peripheral oxygen saturation. Results: Eighteen children and adolescents ages 5–14 years old were analyzed, 61.1% boys, 100% black or brown, and 61.1% in primary education, with low household income. The average distance walked in 6MWT was 463.8 (137.7) m, significantly less than the predicted value (P < .001). The distance of 6MWT was associated positively with age (P = .042) and inversely with reticulocyte count (P = .42) and interleukin 6 (P = .00). Age modified the effect of interleukin 6 in younger children (P = .038). Conclusion: Our findings suggest increased baseline levels of biomarkers of hemolysis and inflammation impact on 6MWT performance.

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

Cited by 3 publications

References 40 publications

Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis

Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis

Longitudinal effect of disease‐modifying therapy on left ventricular diastolic function in children with sickle cell anemia

Exercise Capacity and Biomarkers Among Children and Adolescents With Sickle Cell Disease

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