scholarly journals Reduced fitness and abnormal cardiopulmonary responses to maximal exercise testing in children and young adults with sickle cell anemia

2015 ◽  
Vol 3 (4) ◽  
pp. e12338 ◽  
Author(s):  
Robert I. Liem ◽  
Madhuri Reddy ◽  
Stephanie A. Pelligra ◽  
Adrienne P. Savant ◽  
Bo Fernhall ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Exercise Testing ◽  
Sickle Cell Anemia ◽  
Maximal Exercise ◽  
Children And Young Adults

scholarly journals The acute phase inflammatory response to maximal exercise testing in children and young adults with sickle cell anaemia

2015 ◽  
Vol 171 (5) ◽  
pp. 854-861 ◽  
Author(s):  
Robert I. Liem ◽  
Kasiemobi Onyejekwe ◽  
Marie Olszewski ◽  
Chisalu Nchekwube ◽  
Frank P. Zaldivar ◽  
...  
Keyword(s):  
Young Adults ◽  
Inflammatory Response ◽  
Sickle Cell ◽  
Acute Phase ◽  
Exercise Testing ◽  
Sickle Cell Anaemia ◽  
Maximal Exercise ◽  
Children And Young Adults

Decreased Fitness Is Associated with Abnormal Cardiopulmonary Response to Maximal Exercise in Pediatric Sickle Cell Anemia.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2109-2109
Author(s):  
Madhuri G. Reddy ◽  
Stephanie A. Pelligra ◽  
Alexis A. Thompson ◽  
Robert I. Liem
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Maximal Exercise ◽  
Ventilatory Threshold ◽  
Work Rate ◽  
Heart Rate Reserve ◽  
Ventilatory Efficiency ◽  
Exercise Limitation ◽  
Peak Vo2 ◽  
Children And Young Adults

Abstract Abstract 2109 The clinical burden of sickle cell anemia (SCA) has a tremendous impact on physical functioning, including cardiopulmonary fitness, among affected individuals. However, the physiologic basis of exercise limitation remains poorly understood in this population. The objective of our study was to characterize the cardiopulmonary response to maximal exercise and to delineate the physiologic mechanisms responsible for decreased fitness among children and young adults with SCA. Methods: We prospectively performed maximal cardiopulmonary exercise testing (CPET) on 60 subjects with SCA (hemoglobin SS or S/β0 thalassemia) and 20 controls without SCA or sickle cell trait matched for race and gender. CPET was completed using a graded, symptom-limited cycle ergometry protocol with breath-by-breath, gas exchange analysis and pre/post spirometry. The primary outcome of fitness was defined by weight-adjusted, peak oxygen consumption (peak VO2). Slopes for determining oxygen uptake kinetics and ventilatory efficiency were calculated using 10-second averages of data points. We used the V-slope method to determine ventilatory threshold. Bivariate comparisons of continuous data were performed using Student's t-test for independent samples (IBM, SPSS V20). We used multivariate analysis to derive a model for determining independent contributors to peak VO2 in subjects. Results: There was no difference in gender distribution among subjects and controls, but subjects were older (15.1 ± 3.44 vs. 13.2 ±2.9 years, p = 0.03) and had lower hemoglobin (8.8 ±1.3 vs. 12.8 ±1.5 g/dL, p < 0.0001). All subjects met criteria for a maximal test as defined by a respiratory exchange ratio (RER) ≥ 1.1, and in all, testing was terminated due to excessive fatigue. No major adverse events occurred during CPET in any subject. Only 1/60 (1.7%) subjects developed vaso-occlusive pain requiring hospitalization in the 2-week follow-up period after testing. Nearly all of the major indicators of CPET performance and gas exchange were adversely affected in our subjects. Compared to controls, subjects demonstrated significantly lower mean peak VO2 (26.9 ±6.9 vs. 40.6 ±8.2 mL/kg/min, p < 0.0001), even after adjustment for age and hemoglobin. Average total test time (5.6 ±1.3 vs. 7.8 ±2.2 min, p = 0.012) and peak work rate (108 ±37 vs. 151 ±57 watts, p = 0.011) were similarly reduced as was ventilatory threshold (1.01 ±0.29 vs. 1.34 ±0.34 L/min, p < 0.0001), indicating earlier transition to anaerobic metabolism during exercise. Heart rate reserve, the difference between achieved maximal and baseline heart rates, was significantly lower (99 ±14 vs. 111 ±15, p = 0.002) in subjects. Slopes calculated using minute ventilation (VE), expired CO2 (VCO2), VO2 and work rate also indicated significantly reduced ventilatory efficiency (ΔVE/ΔVCO2), oxygen delivery (ΔVO2/ΔWR) and oxygen uptake (ΔVO2/ΔVE) kinetics in subjects versus controls. To examine the physiologic contributors to peak VO2 in subjects with SCA alone, we developed a multivariate model that included age, baseline hemoglobin, heart rate reserve, maximal VE, pre-exercise forced expiratory volume in 1 second, and ventilatory threshold. This model explained 67% of the variability observed in peak VO2 in subjects, with age, maximal VE and ventilatory threshold retaining independent contributions to peak VO2 and ventilatory threshold making the largest contribution with an non-standardized β coefficient of 11.9 (SE ±3.2), p < 0.0001. Conclusions: Maximal CPET is safe in children and young adults with SCA, suggesting that acute exercise challenge is well tolerated in this population even at high levels of exercise intensity and physical exertion. When compared to their peers, children and young adults with SCA demonstrate significantly reduced fitness levels. Exercise limitation in SCA may be attributed to complex derangements in the cardiopulmonary and metabolic responses to exercise that are independent of anemia. Our findings highlight the need to develop targeted exercise training strategies aimed at improving fitness in this population and to assess its impact on overall disease severity. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Heart Rate Recovery Is Impaired after Maximal Exercise Testing in Children with Sickle Cell Anemia

2015 ◽  
Vol 166 (2) ◽  
pp. 389-393.e1 ◽  
Author(s):  
Anthony M. Alvarado ◽  
Kendra M. Ward ◽  
Devin S. Muntz ◽  
Alexis A. Thompson ◽  
Mark Rodeghier ◽  
...  
Keyword(s):  
Heart Rate ◽  
Sickle Cell ◽  
Exercise Testing ◽  
Sickle Cell Anemia ◽  
Maximal Exercise ◽  
Heart Rate Recovery

scholarly journals Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy

2021 ◽  
Author(s):  
Arushi Dhar ◽  
Tung Ming Leung ◽  
Abena Appiah-Kubi ◽  
Dorota Gruber ◽  
Banu Aygun ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cardiac Remodeling ◽  
Left Ventricular ◽  
Ventricular Dilation ◽  
Left Ventricular Dilation ◽  
Children And Young Adults ◽  
Hydroxyurea Therapy ◽  
Reverse Cardiac Remodeling

Cardiac abnormalities such as left ventricular hypertrophy, dilation and pulmonary hypertension in sickle cell anemia, have been previously described. Hydroxyurea, a disease modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echocardiographic abnormalities in children and young adults with sickle cell anemia on hydroxyurea therapy is lacking. In this retrospective study, we aim to determine the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and to examine the effects of hydroxyurea on reverse cardiac remodeling. We reviewed the records of patients with sickle cell anemia who underwent routine cardiac screening at Cohen Children's Medical Center between 2010 and 2017, followed by retrospective longitudinal analysis of echocardiograms performed on patients receiving treatment with hydroxyurea. Data on a total of 100 patients with sickle cell anemia were analyzed; 60 (60%) were on hydroxyurea. Twenty-five (41.6%) of the patients on hydroxyurea had been treated for less than 1 year; these patients had a significantly greater prevalence of left ventricular dilation compared to those who had been on treatment for more than 1 year. Serial echocardiograms were then analyzed on patients receiving hydroxyurea. Left ventricular dilation and hypertrophy improved significantly with hydroxyurea treatment. Additionally, the left ventricular volume and mass correlated negatively with duration of treatment with hydroxyurea. Our study provides evidence that prolonged hydroxyurea therapy may lead to reverse cardiac remodeling. Future studies should attempt to follow this patient cohort for a longer duration.

scholarly journals Children and Young Adults Admitted to a NYC Children's Hospital Had a Similar Rate of Severe COVID-19 Coagulopathy As That Reported in Older Adults

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 27-28
Author(s):  
William Beau Mitchell ◽  
Jennifer G. Davila ◽  
Janine Keenan ◽  
Jenai Jackson ◽  
Adit Tal ◽  
...  
Keyword(s):  
Older Adults ◽  
Young Adults ◽  
Venous Thromboembolism ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Ventilatory Support ◽  
Children's Hospital ◽  
Children’S Hospital ◽  
Children And Young Adults ◽  
D Dimer

The coagulopathy associated with COVID-19 has not been previously described in children and young adults. We reviewed the clinical and laboratory characteristics of children and young adults admitted for COVID-19 to an urban Children's Hospital in New York City, focusing on coagulation and venous thromboembolism. Clinical and laboratory data were analyzed from 54 patients aged 2 months to 30 years treated by the Pediatric Hematology service at a single Children's Hospital between January 1 and May 31, 2020. Information was obtained from hospital records with IRB approval. There was a moderate male predominance, with 32 (59%) males and 22 (41%) females. There were 28 (52%) patients younger than 18 years and 26 (48%) patients 18 - 30 years old. 26% of patients identified as Black, and 57% as Hispanic/Latino, similar to the community demographics in the Bronx in the 2019 census. Obesity was the most prevalent comorbid condition, with 19 (35%) patients having BMI of 30 or higher. There were also 12 (22%) patients with sickle cell anemia. There were 28 (52%) patients in this cohort with severe and critical illness, as based on established criteria, and 25 (46%) patients required increased ventilatory support. This was defined by the need for &gt; 5L nasal cannula, high-flow nasal cannula, non-rebreather, or intubation. 11 patients (20%) had documented venous thromboembolism (VTE). Four patients died of COVID-19 complications at ages 2 months, 11, 14 and 18 years old. The VTE rate was similar in those patients under 18 years of age (5 of 28, 18%) and those 18 - 30 years of age (6 of 26, 23%). Most (94%) patients had a D-Dimer &gt; 0.5 (upper limit of normal) at admission and 57% developed peak D-Dimer &gt; 5 ug/mL during their admission. Elevated D-dimer &gt; 5 was a risk factor for VTE with 3 of 23 (13%) and 7 of 17 (41%) patients developing VTE with D-dimer &lt; 5 and &gt; 5, respectively (OR 4.7, p=0.042). Patients requiring increased ventilatory support had a 36% rate of VTE as compared to 1 of 28 (4%) of those without (OR 15.2, p=0.003). Six of 24 patients on prophylactic anticoagulation developed VTE. One patient developed a pulmonary embolism 10 days post discharge from the hospital. No patients on anti-Xa-based low molecular weight heparin prophylaxis developed VTE. None of 12 patients with sickle cell anemia developed VTE, had peak D-Dimer &gt; 5 ug/mL or required increased ventilatory support. Hospitalized children and young adults with COVID-19 in our cohort developed a coagulopathy similar to that of older adults, characterized by elevated D-Dimer and high rate of VTE. This is in contrast to the published pediatric series out of China and Singapore that described mild illness and did not comment on VTE rates. Presence of elevated D-dimer or need for increased ventilatory support were significant risk factors for thrombosis. Patients with sickle cell anemia had a lower risk of VTE and less severe illness. Anti-Xa monitored thromboprophylaxis may aid in preventing or ameliorating the COVID-19 coagulopathy in children and young adults. Institutional anticoagulation guidelines were developed based on these observations. Disclosures Davila: Spire Learning: Speakers Bureau; ATHN: Other: Grant Funding. O'Brien:Bristol Myers Squibb: Consultancy, Membership on an entity's Board of Directors or advisory committees.

scholarly journals Enuresis Is a Common and Persistent Problem Among Children and Young Adults with Sickle Cell Anemia

Urology ◽  
2008 ◽  
Vol 72 (1) ◽  
pp. 81-84 ◽  
Author(s):  
Joshua J. Field ◽  
Paul F. Austin ◽  
Ping An ◽  
Yan Yan ◽  
Michael R. DeBaun
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Persistent Problem ◽  
Children And Young Adults

Heart Rate Recovery Is Impaired After Maximal Exercise Challenge in Pediatric Sickle Cell Anemia

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3220-3220
Author(s):  
Anthony M. Alvarado ◽  
Stephanie A. Pelligra ◽  
Kendra M. Ward ◽  
Alexis A. Thompson ◽  
Robert I. Liem
Keyword(s):  
Sickle Cell ◽  
Exercise Testing ◽  
Maximal Exercise ◽  
Heart Rate Recovery ◽  
Cardiopulmonary Exercise Testing ◽  
Recovery Phase ◽  
Qtc Interval ◽  
Post Exercise ◽  
Children And Young Adults ◽  
The Difference

Abstract Abstract 3220 Slow heart rate recovery (HRR) after exercise, particularly at 1 and 2 minutes during recovery, is a strong indicator of autonomic nervous system (ANS) imbalance and represents an important risk factor for increased cardiovascular morbidity and mortality in the general population. Recent evidence suggests cardiovascular ANS dysfunction and vagal tone impairment also exist in individuals with sickle cell anemia (SCA). Despite the known impact of disease burden on exercise capacity and overall fitness in SCA, post-exercise HRR has not previously been studied in this population. The objective of this study was to examine HRR in the post-exercise recovery phase following maximal exercise testing in a cohort of children and young adults with SCA. Methods We prospectively performed maximal cardiopulmonary exercise testing using a ramp, cycle ergometry protocol in 60 subjects with SCA (hemoglobin SS and S-β0 thalassemia) and 20 controls without SCA or sickle cell trait matched for race and gender. Data from standard 12-lead electrocardiography (ECG) was assessed during a 10-minute recovery phase, and HR and corrected QT (QTc) interval measured from tracings obtained at 1-minute intervals. A single investigator performed all measurements manually using leads II, V5, and V6. Final values were averaged from 3 independent measurements. The difference between maximal HR and both HR at 1-minute (ΔHR1min) and 2-minute (ΔHR2min) recovery was our primary outcome. Between-group differences were compared using Mann-Whitney U testing (IBM, SPSS V20). The relationship between ΔHR1min and exercise parameters was examined using Spearman's rank correlation coefficient. Results Post-exercise ECG data from 58 subjects with SCA (median age 15.5 years) and 20 controls without SCA (median age 12 years) were interpretable for this analysis. A total of 30/58 (52%) and 22/58 (38%) subjects were male and on hydroxyurea, respectively. There was no significant difference in median baseline HR or QTc interval in subjects versus controls. Median peak HR was also not significantly different in the 2 groups. When compared to controls, subjects with SCA demonstrated significantly lower HR reserve (100 vs. 111 bpm, p = 0.005), representing the difference between peak and baseline HR. Impaired HRR, defined by slower declines in HR during recovery, was also evident among subjects with SCA following maximal exercise challenge. Although the absolute HR measured at each minute of recovery did not differ significantly in the 2 groups, subjects demonstrated significantly smaller median ΔHR1min (21 vs. 33 bpm, p < 0.0001) and ΔHR2min (39 vs. 51 bpm, p = 0.003), even after adjustment for age between groups. Significantly smaller ΔHR values were also noted in subjects at each minute up to 8 minutes throughout recovery. When compared to subjects not on hydroxyurea, subjects on treatment had significantly greater median ΔHR1min (25 vs. 19 bpm, p = 0.037) but similar ΔHR2min. In subjects with SCA, ΔHR1min correlated inversely with age (−0.41, p = 0.001) and directly with peak oxygen consumption (peak VO2) (0.30, p = 0.03) and oxygen delivery (ΔVO2/Δwork rate) (0.46, p < 0.0001), but not with baseline QTc interval or hemoglobin. Through mono-exponential curve fitting, we found that the time constant associated with HR decline over the first 5 minutes of recovery was greater in subjects with SCA versus controls (T = 128 sec, 95% CI [123, 134] versus 104 sec, 95% [97, 110]). Conclusions Compared to controls without SCA, children and young adults with SCA demonstrate impaired HRR following maximal cardiopulmonary exercise testing, as evidenced by smaller decrements in HR at 1 and 2 minutes during recovery and exponential time constants that indicate a longer period of time over which HR declines. Slower HRR is also associated with decreased fitness and measures of oxygen delivery during exercise testing in subjects with SCA. Although the mechanisms are not well understood, prolonged HRR in this population may in part be explained by ANS imbalance, suggesting either inadequate sympathetic withdrawal or suboptimal parasympathetic reactivation in the post-exercise period. Future studies should focus on delineating the role of the ANS in post-exercise HRR and assessing the prognostic potential of this marker as it relates to disease severity and outcomes in SCA. Disclosures: No relevant conflicts of interest to declare.

Sign in / Sign up

Export Citation Format

Share Document