scholarly journals Evaluation of functional capacity for exercise in children and adolescents with sickle-cell disease through the six-minute walk test

2013 ◽  
Vol 89 (6) ◽  
pp. 588-594 ◽  
Author(s):  
Sandro V. Hostyn ◽  
Werther B. de Carvalho ◽  
Cíntia Johnston ◽  
Josefina A.P. Braga
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Functional Capacity ◽  
Walk Test ◽  
Cell Disease ◽  
Six Minute Walk Test ◽  
Minute Walk

Hemolysis-Associated Elevation in Tricuspid Regurgitation Velocity Predicts Reduction in Six-Minute Walk Distance After Two Years of Follow up in Children and Adolescents with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 574-574
Author(s):  
Victor R. Gordeuk ◽  
Lori Luchtman-Jones ◽  
Andrew D. Campbell ◽  
Sohail R Rana ◽  
Mehdi Nouraie ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Cell Disease ◽  
Walk Distance ◽  
Six Minute Walk Test ◽  
Minute Walk Distance ◽  
Minute Walk

Abstract Abstract 574 Background. Elevated tricuspid regurgitation velocity (TRV) as determined by echocardiography correlates with elevated systolic pulmonary artery pressure and is associated with increased morbidity and mortality in adults with sickle cell disease. The importance of elevated TRV in children and adolescents with sickle cell disease is not known. The Pulmonary Hypertension and the Hypoxic Response in SCD (PUSH) study is an ongoing, longitudinal and observational multicenter study of children with sickle cell disease. Methods. Baseline echocardiography and six-minute walk test were performed prospectively in 361 children and adolescents with sickle cell disease at steady state and then repeat studies were performed in 209 after a median of 22 months of follow up (range 10 months to 36 months), also at steady state. A hemolytic component was derived by principal component analysis of baseline values for reticulocyte count, lactate dehydrogenase, aspartate aminotransferase and total bilirubin. Results. TRV or six-minute walk test were measured at both baseline and follow-up in 193 patients. Twenty-one of these 193 patients had elevated TRV of 2.60 m/sec or higher at baseline. Elevated baseline TRV was associated with high hemolytic rate in 15 patients, defined as hemolytic component above the median for the population studied, and with lower hemolytic rate in six patients. Elevated baseline TRV with high hemolytic rate predicted elevated TRV at follow up (odds ratio 7.7; 95% confidence interval [CI] 2.5 to 24.2; P <0.001) but elevated baseline TRV with lower hemolytic rate did not (odds ratio 1.6; 95% CI 0.2 to 14.1; P = 0.7). Elevated baseline TRV with high hemolytic rate also predicted a decline in the six-minute walk distance by 10% or more at follow-up (hazard ratio 3.9; 95% CI 1.4 to 10.7; P = 0.009). In contrast, higher cardiac output as measured by the left ventricular end diastolic dimension z-score was associated with reduced risk for a decline in the walk distance (hazard ratio 0.7; 95%CI 0.6 to 0.9; P = 0.006). Conclusion. Steady-state TRV elevation in association with a high hemolytic rate occurs on screening in about 8% of children and adolescents with sickle cell disease and is predictive of elevated TRV and reduced six-minute walk distance after approximately two years of follow. Such children may be at risk for adverse clinical consequences of pulmonary hypertension as young adults. Further studies are indicated to identify the molecular mechanisms and to develop appropriate medical management for children and adolescents with this complication. Disclosures: No relevant conflicts of interest to declare.

The Six-Minute Walk Test In Children With Sickle Cell Disease

2010 ◽  
Author(s):  
Jason B. Caboot ◽  
Abbas F. Jawad ◽  
Cheryl Y. Bowdre ◽  
Raanan Arens ◽  
Carole L. Marcus ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Walk Test ◽  
Cell Disease ◽  
Six Minute Walk Test ◽  
Minute Walk

scholarly journals Lung function and six-minute walk test performance in individuals with sickle cell disease

2014 ◽  
Vol 18 (1) ◽  
pp. 79-87 ◽  
Author(s):  
Daniela G. Ohara ◽  
Gualberto Ruas ◽  
Isabel A. P. Walsh ◽  
Shamyr S. Castro ◽  
Mauricio Jamami
Keyword(s):  
Sickle Cell Disease ◽  
Lung Function ◽  
Sickle Cell ◽  
Test Performance ◽  
Walk Test ◽  
Cell Disease ◽  
Six Minute Walk Test ◽  
Minute Walk

scholarly journals Six-minute walk test as a determinant of the functional capacity of children and adolescents with cystic fibrosis: A systematic review

2018 ◽  
Vol 137 ◽  
pp. 83-88 ◽  
Author(s):  
Cibelle Andrade Lima ◽  
Armèle Dornelas de Andrade ◽  
Shirley Lima Campos ◽  
Daniella Cunha Brandão ◽  
Ianny Pereira Mourato ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cystic Fibrosis ◽  
Children And Adolescents ◽  
Functional Capacity ◽  
Walk Test ◽  
Six Minute Walk Test ◽  
Minute Walk

scholarly journals Improvement of the Six-Minute Walk Test in Children with Sickle Cell Disease over Time

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1085-1085
Author(s):  
Geoffrey Bourg ◽  
Laurence Dedeken ◽  
Phu-Quoc Le ◽  
Laurence Rozen ◽  
Safiatou Diallo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Hemoglobin Level ◽  
Walk Test ◽  
Cell Disease ◽  
Walk Distance ◽  
Six Minute Walk Test ◽  
Clinical Events ◽  
Minute Walk Distance ◽  
Minute Walk ◽  
Over Time

Abstract The six-minute walk test (6MWT) was introduced in adults and children suffering from pulmonary or cardio-vascular conditions to assess their sub-maximal functional exercise capacity. In sickle cell disease (SCD), a reduced 6-minute walk distance was observed in adults with chronic pain, hip avascular necrosis and osteopenia ; and in children with low hemoglobin level, low fetal hemoglobin, a baseline elevated TRV. In a previous study (Dedeken et al., PLoS One 2014), we also showed that abnormal 6MWT was significantly associated with the presence of silent infarct. The aim of our study is to explore the evaluation of the 6MWT over time and to confirm the correlation with the cerebral vasculopathy in a larger cohort. This study was conducted at Hôpital Universitaire des Enfants Reine Fabiola (Brussels, Belgium) and included SCD children older than 6 years, regularly followed between 2011 and 2017 and who had at least two 6MWT. The age-standardized predicted value of the 6-minute walk distance (6MWD) was established as reported by Geiger. The 6MWT was considered as normal if the 6MWD was more than 80% of the age-standardized predicted value. Baseline hematological values, clinical events, cerebro-vascular disease, cardio-pulmonary parameters and disease-modifying treatment (DMT) were compared between those with normal and abnormal 6MWT and according to the 6MWD and between the 1st and the 2nd 6MWT overtime. 118 patients have been assessed twice and had at first evaluation a 6MWD of 90.6% (Range 49-119%), with an abnormal test found in 5.1%. The characteristics of the patients are detailed in the Table 1. The changes of the 6MWD and the biological data over time are detailed in Table 2. After 4 years of follow-up, 77.1% of patients were treated with Hydroxyurea (HU) and 16.6% patients were chronically transfused. In parallel with the increased HU prescribing rate, we have observed a significant increase of the Hb and the MCV and a decrease of reticulocytes and hemolysis parameters. The first 6WMT was performed at the median age of 10.3 years and the last one at the median age of 14.1 years. The median 6MWD increased over time including for non-chronically transfused patients. Girls performed less well in the 6MWT (93% for girls vs. 95.7% for boys; P = 0.03). Acute chest syndrome was significantly more frequent in boys (62%) compare to girls (38.7%). Nevertheless, no other differences were founded between boys and girls regarding biological values, clinical events or DMT. 26.5% of our patients have silent infarcts at a median age of 14.6 years. The 6MWD was the same in patients with and without silent infarcts (92.5% vs. 95% ; P=0.17) even when chronically transfused patients were excluded (94% vs. 95% ; P= 0.20). Patients with silent infarcts have a significant lower hemoglobin level and higher reticulocytes count, neutrophils count, LDH and MCV. In conclusion, the 6MWD observed in our cohort characterized by a very high rate of HU treatment is much higher than published in others series and improved over time. With only 5% of SCD patients having a 6MWD < 80% of the normal predicted value at last evaluation, we were not able anymore to confirm a correlation between the presence of silent infracts and abnormal 6MWT. Disclosures No relevant conflicts of interest to declare.

Pulmonary Hypertension in Sickle Cell Disease: Cardiopulmonary Evaluation and Response to Chronic Phosphodiesterase 5 Inhibitor Therapy.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 235-235 ◽  
Author(s):  
Roberto F. Machado ◽  
Sabrina E. Martyr ◽  
Anastasia Anthi ◽  
Gregory J. Kato ◽  
Lori A. Hunter ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Functional Capacity ◽  
Cell Disease ◽  
Walk Distance ◽  
Jet Velocity ◽  
Minute Walk Distance ◽  
Minute Walk ◽  
Tricuspid Regurgitant Jet Velocity

Abstract Pulmonary hypertension (PH) is a risk factor for mortality in Sickle Cell Disease, but it is unclear whether pulmonary hypertension is a marker or a direct cause of mortality. To better understand the pathophysiology of pulmonary hypertension in patients with sickle cell disease we performed evaluations of cardiopulmonary function in sickle cell disease patients with pulmonary hypertension (n= 15, mean age = 41 ± 2.4 years, males = 7, HbSS = 15, mean Hb = 8.3 ± 0.2 g/dl, mean tricuspid regurgitant jet velocity = 3.2 ± 0.11 m/s) compared to matched controls with sickle cell disease without pulmonary hypertension (n=11, mean age=40.2 ± 2.5 years, males=4, HbSS=11, mean Hb=8.5 ± 0.3 g/dl, mean tricuspid regurgitant jet velocity = 2.28 ± 0.07 m/s). To evaluate if specific therapy for pulmonary hypertension has any impact on systolic pulmonary artery pressure (PAP), estimated by tricuspid regurgitant jet velocity (TRJ), and functional capacity, measured by six-minute walk test (a well validated surrogate of functional capacity and response to therapy in patients with other causes of pulmonary hypertension), we treated 14 patients with sickle cell disease and pulmonary hypertension (mean age = 40 ± 2.5 years, males = 3, HbSS = 14, mean Hb = 8.8 ± 0.6 g/dl, mean TRJ = 3.4 ± 0.1 m/s) with sildenafil for at least three months. When compared to controls pulmonary hypertension patients had lower maximal oxygen consumption (VO2 max (% predicted), +PH: 44 ± 4, −PH: 55 ± 4; P=0.41), walked shorter six-minute walk distance (meters, +PH: 308.5 ± 53.8, −PH: 427.1 ± 44.6; P=0.03), demonstrated greater degree of interstitial lung disease by chest CT (P &lt; 0.05), and more perfusion impairments measured by ventilation perfusion scan (P &lt; 0.05). Six-minute walk distance correlated directly with maximal oxygen consumption (R=0.6; P=0.01), and inversely with mean pulmonary arterial pressure (R= −0.5; P=0.03) and tricuspid regurgitant jet velocity (R= −0.6;P=0.002), suggesting that the test is an adequate surrogate of functional capacity and response to therapy in pulmonary hypertension patients with sickle cell disease. Chronic treatment with sildenafil (up to 100 mg TID) decreased pulmonary arterial pressure (PAP mmHg, baseline: 50 ± 4.4, sildenafil: 41 ± 2.5; P=0.04) and increased six-minute walk distance (meters, baseline: 394 ± 31, sildenafil: 476 ± 26: P= 0.02). Sildenafil was well tolerated with only 2 patients stopping the drug due to headaches. We also observed 3 episodes of transient eyelid edema not requiring discontinuation of drug. Priapism was not observed in the 3 males treated (2 on exchange transfusion therapy, 1 with erectile dysfunction). In conclusion, we find that in patients with sickle cell disease, 1) pulmonary hypertension, though relatively mild, is associated with severe impairments in cardiopulmonary function, 2) traditional markers of functional capacity such as six-minute walk test can be utilized in this population as a therapeutic endpoint for clinical trials, 3) and therapy with sildenafil seems to have a favorable impact on pulmonary pressures and functional capacity.

scholarly journals Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

2016 ◽  
Vol 42 (6) ◽  
pp. 409-415 ◽  
Author(s):  
Ana Karine Vieira ◽  
◽  
Cristina Gonçalves Alvim ◽  
Maria Cristina Marquez Carneiro ◽  
Cássio da Cunha Ibiapina ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Pulmonary Function Tests ◽  
Cell Disease ◽  
Restrictive Lung Disease ◽  
Minute Walk ◽  
Abnormal Pulmonary Function

ABSTRACT Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Prospective Evaluation of the Prevalence of Elevated Tricuspid Regurgitant Jet Velocity and Associated Clinical and Echocardiographic Factors in Children and Adolescents with Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3388-3388
Author(s):  
Andrew D. Campbell ◽  
Caterina Minniti ◽  
Craig Sable ◽  
Greg Ensing ◽  
Niti Dham ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Fold Increase ◽  
Systemic Hypertension ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Jet Velocity ◽  
Minute Walk

Abstract Background: Echocardiography (ECHO)-determined tricuspid regurgitation jet velocity (TRV) ≥2.5 m/s occurs in about 30% of adults with sickle cell disease(SCD)and is associated with increased mortality. The frequency and significance of high TRV in children is not known. We determined the prevalence and risk factors of elevated estimated systolic pulmonary artery pressures in 228 patients with SCD and 39 controls. Methods: Patients with SCD (Hb SS, SC, SB thalassemia or other major sickling phenotypes) aged 3 to 20 years were evaluated clinically and with ECHO and six minute walk tests at their steady state. Healthy, non-SCD controls, matched for ethnicity, sex and age, were also evaluated. Results: A comparison of patients and controls is summarized in Table 1. 184 patients (81%) had hemoglobin SS phenotype and 28 (12%) had hemoglobin SC. TRV was measurable in 214 (94%) of the patients and 34 (87%) of the controls. In a multivariate logistic regression model, each 10 mm Hg increase in systolic blood pressure was associated with an estimated 2.0-fold increase in the odds of TRV≥2.5 m/sec (P = 0.002) and a 10-fold increase in LDH with a 25-fold increase in the odds (P = 0.015). Conclusion: This study of children with SCD who have limited iron overload and limited renal dysfunction compared with the aging SCD population provides a unique window into the early pathogenesis of PH in SCD. TRV and LVMI are significantly higher in children and adolescents with SCD at steady state than age-matched control participants. Among children with SCD, TRV elevation is independently associated with systolic BP and LDH concentration. In this analysis we find a strong association with hemolysis and systemic hypertension and surprisingly no associations with vaso-occlusive events or biomarkers (VOC, ACS, HU use, WBC and platelet counts). Table I. Comparison of SCD and control subjects. Results in mean (SD) unless otherwise stated. SCD(n=228) Control (n=39) p Age in years 12 (5) 12 (5) 0.7 Female (%) 48% 46% 0.8 Systolic BP (mm Hg) 112 (11) 116 (15) 0.1 Diastolic BP (mm Hg) 64 (9) 68 (10) 0.040 Six minute walk in meters 454 (76) 498 (84) 0.006 TRV in m/sec 2.3 (0.3) 2.1 (0.3) 0.003 TRV 2.5 m/sec (%) 21% 3% 0.012 LV intern. diastolic diam Z-score 1.2(1.4) −0.3(1.2) <0.001 LV mass index 88 (25) 59 (13) <0.001 Table II. Comparison of SCD patients according to TRV category. Results in mean (SD) unless otherwise stated. Variables TRV <2.5 (n= 169) TRV ≥2.5 (n=45) p History of acute chest syndrome (%) 20% 40% 0.034 Hydroxyurea therapy 42% 44% 0.8 Hemoglobin in g/dl 9.3 (1.8) 8.9 (1.7) 0.071 LDH in U/L 393 (163) 474 (170) 0.009 Total Bilirubin in mg/dL 2.7 (1.9) 3.8 (2.2) 0.004

scholarly journals Transthoracic Echocardiography and 6-Minute Walk Test in Kuwaiti Sickle Cell Disease Patients

2014 ◽  
Vol 23 (3) ◽  
pp. 212-217 ◽  
Author(s):  
Rajaa Marouf ◽  
Nasser Behbehani ◽  
Mohammed Zubaid ◽  
Hanan Al Wazzan ◽  
Hadeel El Muzaini ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transthoracic Echocardiography ◽  
Walk Test ◽  
Cell Disease ◽  
6 Minute Walk Test ◽  
Minute Walk
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