Development of exercise guidelines for individuals with sickle cell trait (SCT) and sickle cell anemia (SCA) is hampered by the need to weigh the benefits against risks of exercise in these ...populations. In SCT, concern for exercise collapse associated with sickle cell trait has resulted in controversial screening of student athletes for SCT. In SCA, there exists unsubstantiated concerns that high-intensity exercise may result in pain and other complications. In both, finding the "right dose" of exercise remains a challenge for patients and their providers. Despite assumptions that factors predisposing to adverse events from high-intensity exercise overlap in SCT and SCA, the issues that frame our understanding of exercise-related harms in both are distinct. This review will compare issues that affect the risk-benefit balance of exercise in SCT and SCA through these key questions: (1) What is the evidence that high-intensity exercise is associated with harm? (2) What are the pathophysiologic mechanisms that could predispose to harm? (3) What are the preventive strategies that may reduce risk? and (4) Why do we need to consider the benefits of exercise in this debate? Addressing these knowledge gaps is essential for developing an evidence-based exercise prescription for these patient populations.
Summary
We examined heart rate variability (HRV) during exercise testing in 20 children with sickle cell anaemia (SCA) and 12 controls. Subjects achieved lower median HRV at peak exercise standard ...deviation of R‐wave to R‐wave intervals (SDNN), 2·3 vs 2·9 ms, P = 0·027; logarithmic transformation of high frequency power (lnHF), 0·9 vs 1·3 ln(ms2), P = 0·047 and had lower post‐exercise HRV across minute‐by‐minute analysis of recovery. After adjustment for haemoglobin, fitness and SCA status, subjects had lower HRV at the end of recovery with differences increasing as baseline HRV increased. Further investigation of HRV and exercise safety in SCA is warranted.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Cardiopulmonary exercise testing (CPET) provides clinicians with information vital to the management of pediatric cardiology patients. CPET can also be used to measure cardiorespiratory fitness (CRF) ...in these patients. CRF is a robust marker of overall health in children. However, a complete understanding of CRF in pediatric cardiology patients is limited by lack of large, standardized CPET databases. Our purpose was to develop a standardized CPET database, describe available data at our institution, and discuss challenges and opportunities associated with this project. CPETs performed from 1993 to present in an urban pediatric hospital were collected and compiled into a research database. Historical data included demographic and clinical variables and CPET outcomes, and additional variables were calculated and coded to facilitate analyses in these cohorts. Patient diagnoses were coded to facilitate sub-analyses of specific cohorts. Quality assurance protocols were established to ensure future database contributions and promote inter-institutional collaborations. This database includes 10,319 CPETs (56.1% male), predominantly using the Bruce Protocol. Patients ranging from ages 6 to 18 years comprise 86.8% of available CPETs. Diagnosis classification scheme includes patients with structurally normal hearts (n = 3,454), congenital heart disease (n = 3,614), electrophysiological abnormalities (n = 2,082), heart transplant or cardiomyopathy (n = 833), and other diagnoses (n = 336). Historically, clinicians were provided with suboptimal interpretive resources for CPET, often generalizing inferences from these resources to non-representative clinical populations. This database supports representative CRF comparisons and establishes a framework for future CRF-based registries in pediatric patients referred for CPET, ultimately improving clinical decision-making regarding fitness in these populations.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Background
Hydroxyurea is the only Food and Drug Administration approved medication for sickle cell disease (SCD) with short‐ and long‐term benefits for both morbidity and mortality. However, ...hydroxyurea underutilization and adherence remain challenges for patients with SCD. The objectives of this study were to determine access to technology among adolescents and young adults (AYA) with SCD and to identify their preferred technology‐based strategies for improving medication adherence.
Procedure
A cross‐sectional survey was administered in a variety of clinical settings from October 2014 through May 2015 to AYA (12–22 years) with SCD (all genotypes) followed in a Comprehensive Sickle Cell Program.
Results
Eighty of 107 eligible participants completed the survey for a 75% response rate. Participants (51% female, 94% Black) had a mean age of 15.3 ± 2.8 years. Most participants (75%) were on a daily medication with about half on hydroxyurea. Forgetfulness (67%) was the most common barrier to medication adherence. The majority of participants (85%) owned smartphones and either owned or had access to electronic tablets (83%), laptops (72%), or desktops (70%). Of the proposed smartphone app features, daily medication reminders were ranked first most frequently, followed by education about SCD, adherence text prompts, education about SCD medications, and medication log.
Conclusions
The majority of our AYA with SCD owned smartphones and had access to other electronic devices. Our survey results provided valuable insight into the preferred app features and optimal strategies for developing technology‐based interventions, such as a multicomponent app, to increase medication adherence for AYA with SCD or other chronic conditions.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Sickle cell anaemia (SCA) patients display elevated levels of circulating pro-inflammatory cytokines and endothelial activation markers compared to healthy peers. The impact of exercise on the ...pro-inflammatory state in SCA remains unclear. This study aimed to characterize the whole-blood transcriptome profile in response to an acute bout of exercise in paediatric SCA patients. Twenty-three SCA participants (13 ± 3 years, 52% girls) and 17 healthy controls (14 ± 3 years, 29% girls) performed eight 2-min bouts of cycle ergometry interspersed with 1-min rest intervals. Whole-blood transcriptome profile (RNA-seq) was performed before and after exercise. At baseline, gene pathways associated with gas transport in erythrocytes were up-regulated in SCA patients compared to controls. Following exercise, gene pathways associated with innate immunity were altered in both groups. Interaction analyses revealed 160 annotated genes (101 up- and 59 down-regulated) that differentially altered by exercise in SCA patients. Moreover, genes that exhibited a blunted response to exercise in SCA patients were enriched in the IL-17 signalling pathway, suggesting an impaired innate immune response to exercise. This data will contribute to the development of evidence-based exercise prescription guidelines for this patient population.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Summary
We used near‐infrared spectroscopy to examine tissue oxygenation (StO2) during exercise in 17 children and young adults with sickle cell anaemia (SCA) and 13 controls. Patients had lower ...cerebral StO2 at all exercise stages and demonstrated significantly greater decreases in cerebral StO2 later during exercise. Quadriceps StO2 increased similarly in patients and controls during early exercise, but decreases from baseline were greater in patients during later exercise. At similar workloads, patients demonstrated lower cerebral StO2 (69·2 ± 6·6 vs. 79·5 ± 5·3%, P < 0·001) and trended towards lower quadriceps StO2 (67·7 ± 9·0 vs. 73·2 ± 7·9%, P = 0·09). Further studies of tissue oxygenation during exercise in SCA are warranted.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Critically ill infants are susceptible to thrombosis due to several risk factors. The aim of this study was to identify risk factors associated with venous and arterial thrombosis in neonates ...admitted to the neonatal intensive care unit (NICU) and to identify differences in risk factors for venous versus arterial thrombosis.
We conducted a case-control study at 31 level IV NICUs using the Children's Hospital Neonatal Database between Jan 1, 2010, and Dec 13, 2016, in the USA. Cases were identified on the basis of having an outcome of venous or arterial thrombosis. Controls were matched by gestational age, presence of a central access device (CAD), hospital, and admission year. Four controls per case (1:4) were randomly selected. Bivariable and multivariable regression analyses were performed to examine the associations between potential risk factors and venous or arterial thrombosis. CAD-related risk factors were analysed in the subset of neonates with a CAD.
We identified 118 952 new admissions to 31 NICUs. The overall thrombosis incidence was 15·5 per 1000 NICU admissions (95% CI 14·8-16·2). After exclusion of patients with a length of hospitalisation longer than 3 days or heart disease, the study included 1326 thrombosis cases (1022 with venous thrombosis and 362 with arterial thrombosis; 58 patients had both types of thrombosis and are included within both of these numbers) and 5304 randomly selected controls. Venous thrombosis was independently associated with bloodstream infection (odds ratio 2·07, 95% CI 1·72-2·49; p<0·0001), maternal diabetes (1·62, 1·30-2·03; p<0·0001), abdominal or gastrointestinal surgery (1·36, 1·17-1·58; p<0·0001), thrombocytopenia (2·44, 2·02-2·94; p<0·0001), prolonged mechanical ventilation (1·27, 1·10-1·46; p=0·0014), and age 7 days or older at admission (1·49, 1·28-1·74; p<0·0001). Arterial thrombosis was independently associated with maternal hypertension (1·42, 1·05-1·91; p=0·030), thrombocytopenia (2·20, 1·59-3·06; p<0·0001), prolonged mechanical ventilation (1·58, 1·24-2·01; p=0·0002), age 7 days or older at admission (1·35, 1·05-1·74; p=0·0018), and small for gestational age (1·56, 1·13-2·16; p=0·0003). In the CAD subset analysis, CAD duration of 21 days or longer (venous thrombosis: 1·52, 1·15-2·01, p=0·0034; arterial thrombosis: 1·98, 1·25-3·14, p=0·035) and CAD in both the upper and lower body (venous thrombosis: 2·43, 1·92-3·08, p<0·0001; arterial thrombosis: 1·58, 1·02-2·45, p=0·040) were associated with higher odds of thrombosis.
Identification of thrombosis-associated risk factors will be useful in developing a risk prediction model to prevent thrombosis and in improving outcomes. The study results add to the knowledge of the differences in risk factors for venous versus arterial thrombosis in neonates and to the understanding of the associations of CAD characteristics with neonatal thrombosis.
Bristol-Myers Squibb-Pfizer Alliance.
Summary
Red blood cell (RBC) transfusions can be life‐sustaining in chronic inherited anaemias, such as thalassaemia, and the indications for blood transfusions in patients with sickle cell disease ...continue to expand. Complications of transfusions, such as allosensitization, can create significant medical challenges in the management of patients with haemoglobinopathies. This review summarizes key findings from the medical literature related to alloimmunization in haemoglobinopathies and examines potential measures to mitigate these risks. Areas where future studies are needed are also addressed.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
To identify risk factors associated with venous and arterial thrombosis in sick neonates admitted to the neonatal intensive care unit.
A case-control study was conducted at 2 centers between January ...2010 and March 2014 using the Children's Hospital Neonatal Database dataset. Cases were neonates diagnosed with either arterial or venous thrombosis during their neonatal intensive care unit stay; controls were matched in a 1:4 ratio by gestational age and presence or absence of central access devices. Bivariable and conditional logistic regression analyses for venous and arterial thrombosis were performed separately.
The overall incidence of neonatal thrombosis was 15.0 per 1000 admissions. A higher proportion of neonates with thrombosis had presence of central vascular access devices (75% vs 49%; P < .01) were of extremely preterm gestational age (22-27 weeks; 26% vs 15.0%; P < .05) and stayed ≥31 days in the neonatal intensive care unit (53% vs 32.9%; P < .01), when compared with neonates without thrombosis. A final group of 64 eligible patients with thrombosis and 4623 controls were analyzed. In a conditional multivariable logistic regression model, venous thrombosis was significantly associated with male sex (AOR, 2.12; 95% CI, 1.03-4.35; P = .04) and blood stream infection (AOR, 3.47; 95% CI, 1.30-9.24; P = .01).
The incidence of thrombosis was higher in our neonatal population than in previous reports. After matching for central vascular access device and gestational age, male sex and blood stream infection represent independent risk factors of neonatal venous thrombosis. A larger cohort gleaned from multicenter data should be used to confirm the study results and to develop thrombosis prevention strategies.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
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