Cardiopulmonary Stress Testing in Children With Sickle Cell Disease Who are on Long-term Erythrocytapheresis

Das, Bibhuti B.; Sobczyk, Walter L.; Bertolone, Salvatore; Raj, Ashok

doi:10.1097/mph.0b013e318165b298

Cited by 10 publications

(9 citation statements)

References 23 publications

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“…In the exchange transfusion experiment attempting to keep Hgb the same (but actually Hgb increased by 1.4 g/dL), the effect of Hgb A was directly queried and results suggested improved exercise capacity was associated with Hgb A 21 . In contrast, in a study of children on long‐term exchange transfusion maintaining Hgb S approximately 30%, average peak VO 2 was still less than 80% compared to healthy controls, but the transfused patients also had significantly lower Hgb (9.3 ± 0.5 g/dL) than healthy controls 32 …”

Section: Discussionmentioning

confidence: 99%

“…21 In contrast, in a study of children on long-term exchange transfusion maintaining Hgb S approximately 30%, average peak VO 2 was still less than 80% compared to healthy controls, but the transfused patients also had significantly lower Hgb (9.3 ± 0.5 g/dL) than healthy controls. 32 Several studies documented improved exercise performance in SCA patients after moderate exercise training.…”

Section: Improving Exercise Capacity In Sca Patientsmentioning

confidence: 99%

“…(A) Hemoglobin and (B) the percent predicted peak oxygen consumption (% predicted peak VO 2 ) of each subject at baseline and after voxelotor therapy. (C) Peak VO 2 ranges of male and female subjects before (yellow bar, CPET#1) and after (red bar, CPET#2) voxelotor therapy, compared to healthy adolescents29,30 (green bar), and Olympic runners31,32 (teal bar). p-Values are calculated by two-tailed paired t-tests.…”

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confidence: 99%

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Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

Phan

Hershenson

Caldarera

et al. 2023

Pediatric Blood & Cancer

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In Memoriam: Laura Caldarera passed away in early 2022 after a nearly year-long battle with aggressive cancer. As manager of the stress testing lab and preventive cardiology clinic at Child Cardiology Associates, she spent the majority of her career helping and assessing pediatric and adult congenital patients to improve their exercise capacity with a goal of improving their overall health and life expectancy. She was passionate, smart, and a true advocate for patients and families. She is greatly missed.

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Section: Discussionmentioning

confidence: 99%

Section: Improving Exercise Capacity In Sca Patientsmentioning

confidence: 99%

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confidence: 99%

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Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

Phan

Hershenson

Caldarera

et al. 2023

Pediatric Blood & Cancer

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“…[43] In the literature, only a few pediatric studies have compared the aerobic capacity of children and adolescents with sickle cell disease with healthy controls. [10,29,44] These studies investigated the exercise oxygen extraction physiology of anemic patients on submaximal CPET [44] and the safety of CPET in children with sickle cell disease [29]. In a controlled prospective study, Liem et al reported a VO 2max difference of 10.1 mL/kg/min between 60 children with sickle cell disease (BMI = 21 ± 4 kg/m2) and 30 controls (BMI = 24 ± 7 kg/m 2 ).…”

Section: Discussionmentioning

confidence: 99%

Aerobic physical capacity and health-related quality of life in children with sickle cell disease

Laurent-Lacroix,

Vincenti,

Matecki

et al. 2023

Preprint

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Purpose To evaluate the aerobic capacity by cardiopulmonary exercise test (CPET) in children and adolescents with sickle cell disease in comparison with healthy matched controls and to determine the factors associated with impaired maximum oxygen uptake (VO2max) in this population. Methods and results A total of 72 children (24 with sickle cell disease and 48 healthy controls), aged 6 to 17 years old underwent a complete CPET and were enrolled in a cross-sectional controlled study. Children with sickle cell disease had a poor aerobic capacity, with median VO2max Z-score values significantly lower than matched controls (-3.55[-4.68; -2.02] vs. 0.25[-0.22; 0.66], P < 0.01, respectively), and a high proportion of 92% children affected by an impaired aerobic capacity (VO2max Z-score<-1.64). The ventilatory anaerobic threshold was impaired in 71% of children with sickle cell disease. The VO2max decrease was associated with the level of anemia, the existence of a homozygote HbS/S mutation, restrictive lung disease (low forced vital capacity associated to low total lung capacity) and health-related quality of life. In multivariate analysis, VO2max Z-score was associated with hemoglobin level (ß=0.37; 95%CI=[0.01; 0.72]; P = 0.04). Conclusion Aerobic capacity is poor in children with sickle cell disease. VO2max decrease is associated with the level of anemia, the existence of a homozygote HbS/S mutation, lung function, and health-related quality of life. These results represent a signal in favor of early initiation of cardiac rehabilitation in patients with sickle cell disease. Clinicaltrials: NCT05995743

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“…This will cause a sluggish flow of the blood in the small vessels. Subsequently, will lead to malvascularization of the body tissues and organs that causes tissue hypoxia, vaso-occlusive crisis (VOC), organ failure and severe anemia [2,3].…”

Section: Introductionmentioning

confidence: 99%

The Effect of Physiotherapy Program on Hip Pain and Postural Control in Adolescents with Sickle Cell Disease

A¹,

Heba²

2019

J Med - Clin Res & Rev

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Background: Sickle cell disease (SCD) is one of the most known genetic diseases in Saudi Arabia. With a common complication found to be the avascular necrosis (AVN) of the femoral head by 27% in eastern province. This avascular necrosis causes distraction of the hip joint; pain; and other complications.Objectives: the aim of this study was to measure the effect of physiotherapy program on pain intensity and postural stability in sickle cell disease adolescents with hip avascular necrosis.Methods: 21 participants who are aged between 10 to 18 years old and known to have Sickle cell disease with hip avascular necrosis were recruited. Measurements were taken at baseline and at the end of the treatment program. Wong-Baker FACES pain rating scale was use to evaluate the pain intensity. Biodex Balance System was used to evaluate the Overall stability index. Manual goniometer was use to evaluate the range of motion. Manual muscle testing was used to evaluate the muscle power. The statistical outcome differences within the group analysis was measured using paired t-test. Wilcoxon signed rank test was used to measure the differences within the group analysis for non-parametric variables.Results: All participants showed significant statistical differences in pain intensity; Overall stability index at level 6; range of motion; and muscle power. However; overall stability index at level 12 post-test result was better than the pre-test result; but it did not give a significant statistical difference. Conclusion:Physiotherapy program proved to be an effective method to improve the pain intensity and postural stability in sickle cell disease patients with hip avascular necrosis. Furthermore; improvement of range of motion and muscle power will improve or limit the related complications.

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Cardiopulmonary Stress Testing in Children With Sickle Cell Disease Who are on Long-term Erythrocytapheresis

Cited by 10 publications

References 23 publications

Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

Aerobic physical capacity and health-related quality of life in children with sickle cell disease

The Effect of Physiotherapy Program on Hip Pain and Postural Control in Adolescents with Sickle Cell Disease

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