Pediatric refractory chronic immune thrombocytopenia: Identification, patients' characteristics, and outcome.

Refractory chronic immune thrombocytopenia (r-cITP) is one of the most challenging situations in chronic immune thrombocytopenia (cITP). Pediatric r-cITP is inconsistently defined in literature, contributing to the scarcity of data. Moreover, no evidence is available to guide the choice of treatment. We compared seven definitions of r-cITP including five pediatric definitions in 886 patients with cITP (median [min-max] follow-up 5.3 [1.0-29.3] years). The pediatric definitions identified overlapping groups of various sizes (4%-20%) but with similar characteristics (higher proportion of immunopathological manifestations [IM] and systemic lupus erythematosus [SLE]), suggesting that they adequately captured the population of interest. Based on the 79 patients with r-cITP (median follow-up 3.1 [0-18.2] years) according to the CEREVANCE definition (≥3 second-line treatments), we showed that r-cITP occurred at a rate of 1.15% new patients per year and did not plateau over time. In multivariate analysis, older age was associated with r-cITP. One patient (1%) experienced two grade five bleeding events after meeting r-cITP criteria and while not receiving second-line treatment. The cumulative incidence of continuous complete remission (CCR) at 2 years after r-cITP diagnosis was 9%. In this analysis, splenectomy was associated with a higher cumulative incidence of CCR (hazard ratio: 5.43, 95% confidence interval: 1.48-19.84, p = 7.8 × 10-4). In sum, children with cITP may be diagnosed with r-cITP at any time point of the follow-up and are at increased risk of IM and SLE. Second-line treatments seem to be effective for preventing grade 5 bleeding. Splenectomy may be considered to achieve CCR.

Antinuclear antibody-associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus.

ABSTRACT: Autoimmune cytopenia (AIC) in children may be associated with positive antinuclear antibodies (ANA) and may progress to systemic lupus erythematosus (SLE). We evaluated the risk of progression to SLE of childhood-onset ANA-associated AIC. In the French national prospective OBS'CEREVANCE cohort, the long-term outcome of children with ANA-associated AIC (ANA titer ≥1/160) and a subgroup of children who developed SLE were described. ANA were positive in 355 of 1803 (20%) children with AIC. With a median follow-up of 5.8 (range, 0.1-29.6) years, 79 of 355 (22%) patients developed SLE at a median age of 14.5 (1.1-21.4) years; 20% of chronic immune thrombocytopenic purpura, 19% of autoimmune hemolytic anemia, and 45% of Evans syndrome. None of the patients with ANA-negative test developed SLE. Severe manifestations of SLE were observed in 21 patients, and 2 patients died. In multivariate analysis including patients with positive ANA within the first 3 months after AIC diagnosis, age >10 years at AIC diagnosis (relative risk [RR], 3.67; 95% confidence interval [CI], 1.18-11.4; P = .024) and ANA titer >1/160 (RR, 5.28; 95% CI, 1.20-23.17; P = .027) were associated with the occurrence of SLE after AIC diagnosis. ANA-associated AIC is a risk factor for progression to SLE, especially in children with an initial ANA titer >1/160 and an age >10 years at AIC diagnosis. ANA screening should be recommended in children with AIC, and patients with ANA should be monitored long-term for SLE, with special attention to the transition period. This trial was registered at www.ClinicalTrials.gov as #NCT05937828.

Effects of a Cycling versus Running HIIT Program on Fat Mass Loss and Gut Microbiota Composition in Men with Overweight/Obesity.

PURPOSE: High-intensity interval training (HIIT) can efficiently decrease total and (intra-)abdominal fat mass (FM); however, the effects of running versus cycling HIIT programs on FM reduction have not been compared yet. In addition, the link between HIIT-induced FM reduction and gut microbiota must be better investigated. The aim of this study was to compare the effects of two 12-wk HIIT isoenergetic programs (cycling vs running) on body composition and fecal microbiota composition in nondieting men with overweight or obesity. METHODS: Sixteen men (age, 54.2 ± 9.6 yr; body mass index, 29.9 ± 2.3 kg·m -2 ) were randomly assigned to the HIIT-BIKE (10 × 45 s at 80%-85% of maximal heart rate, 90-s active recovery) or HIIT-RUN (9 × 45 s at 80%-85% of maximal heart rate, 90-s active recovery) group (3 times per week). Dual-energy x-ray absorptiometry was used to determine body composition. Preintervention and postintervention fecal microbiota composition was analyzed by 16S rRNA gene sequencing, and diet was controlled. RESULTS: Overall, body weight, and abdominal and visceral FM decreased over time ( P < 0.05). No difference was observed for weight, total body FM, and visceral FM between groups (% change). Conversely, abdominal FM loss was greater in the HIIT-RUN group (-16.1% vs -8.3%; P = 0.050). The α-diversity of gut microbiota did not vary between baseline and intervention end and between groups, but was associated with abdominal FM change ( r = -0.6; P = 0.02). The baseline microbiota profile and composition changes were correlated with total and abdominal/visceral FM losses. CONCLUSIONS: Both cycling and running isoenergetic HIIT programs improved body composition in men with overweight/obesity. Baseline intestinal microbiota composition and its postintervention variations were correlated with FM reduction, strengthening the possible link between these parameters. The mechanisms underlying the greater abdominal FM loss in the HIIT-RUN group require additional investigations.

Fitness and metabolic response to exercise in young adult survivors of childhood lymphoma.

PURPOSE: Childhood lymphoma survivors (CLSs) are at high risk of reduced daily activity. This work studied metabolic substrate use and cardiorespiratory function in response to exercise in CLSs. METHODS: Twenty CLSs and 20 healthy adult controls matched for sex, age, and BMI took an incremental submaximal exercise test to determine fat/carbohydrate oxidation rates. Resting echocardiography and pulmonary functional tests were performed. Physical activity level, and blood metabolic and hormonal levels were measured. RESULTS: CLSs reported more physical activity than controls (6317 ± 3815 vs. 4268 ± 4354 MET-minutes/week, p = 0.013), had higher resting heart rate (83 ± 14 vs. 71 ± 13 bpm, p = 0.006), and showed altered global longitudinal strain (- 17.5 ± 2.1 vs. - 19.8 ± 1.6%, p = 0.003). We observed no difference in maximal fat oxidation between the groups, but it was reached at lower relative exercise intensities in CLSs (Fatmax 17.4 ± 6.0 vs. 20.1 ± 4.1 mL/kg, p = 0.021). At V̇O2 peak, CLSs developed lower relative exercise power (3.2 ± 0.9 vs. 4.0 ± 0.7 W/kg, p = 0.012). CONCLUSION: CLSs reported higher levels of physical activity but they attained maximal fat oxidation at lower relative oxygen uptake and applied lower relative power at V̇O2 peak. CLSs may thus have lower muscular efficiency, causing greater fatigability in response to exercise, possibly related to chemotherapy exposure during adolescence and childhood. Long-term follow-up is essential and regular physical activity needs to be sustained.

Is it wiser to train in the afternoon or the early evening to sleep better? The role of chronotype in young adolescent athletes.

STUDY OBJECTIVES: To examine the effect of the timing of high-intensity exercise (afternoon vs. evening) on adolescent athletes' bedtime psychological state, sleep quality, sleep staging, and next-day wellness/sleepiness according to chronotype. METHODS: Forty-two young athletes (morning type: n = 12, intermediate type: n = 14; evening type: n = 16) completed a randomized crossover study under free-living conditions. The counterbalanced sessions include: (AEX) afternoon (1:00-3:00 p.m.) and (EEX) evening (5:30-7:30 p.m.) high-intensity exercise. Sessions were conducted over three days each and were separated by a 1-week washout period. The time in bed was fixed (10:30 p.m.-7:30 a.m.). Sleep was assessed through ambulatory polysomnography. RESULTS: The effect of high-intensity exercise on sleep differs significantly depending on the time of exercise with lower sleep efficiency: SE (-1.50%, p < .01), and higher SOL (+4.60 min, p ≤=< .01), during EEX vs. AEX. Contrary to the previous view, we discovered differences in the mediated response based on the chronotype of young athletes. These differences were observable in the psychological state at bedtime, objective sleep, and the next day's self-reported wellness. Whereas the sleep of participants with a late chronotype remains stable regardless of the time of exercise, those with an early chronotype experience higher mood disturbances and clinically significant sleep disruptions following evening high-intensity exercise. CONCLUSIONS: Exercise timing and chronotype affect the psychological state at bedtime and objective sleep in adolescent athletes. This also alters next morning signs of pre-fatigue and wellness which suggest that the consideration of both features is important to adolescent athletes' recovery.

Impact of age at diagnosis, sex, and immunopathological manifestations in 886 patients with pediatric chronic immune thrombocytopenia.

Pediatric chronic immune thrombocytopenia (cITP) is a heterogeneous condition in terms of bleeding severity, second-line treatment use, association with clinical and/or biological immunopathological manifestations (IMs), and progression to systemic lupus erythematosus (SLE). No risk factors for these outcomes are known. Specifically, whether age at ITP diagnosis, sex, or IMs impact cITP outcomes is unknown. We report the outcomes of patients with pediatric cITP from the French nationwide prospective cohort OBS'CEREVANCE. We used multivariate analyses to investigate the effect of age at ITP diagnosis, sex, and IMs on cITP outcomes. We included 886 patients with a median (min-max) follow-up duration of 5.3 (1.0-29.3) years. We identified an age cutoff that dichotomized the risk of the outcomes and defined two risk groups: patients with ITP diagnosed <10 years (children) and ≥ 10 years (adolescents). Adolescents had a two to four-fold higher risk of grade ≥3 bleeding, second-line treatment use, clinical and biological IMs, and SLE diagnosis. Moreover, female sex and biological IMs were independently associated with higher risks of biological IMs and SLE diagnosis, second-line treatment use, and SLE diagnosis, respectively. The combination of these three risk factors defined outcome-specific risk groups. Finally, we showed that patients clustered in mild and severe phenotypes, more frequent in children and adolescents, respectively. In conclusion, we identified that age at ITP diagnosis, sex, and biological IMs impacted the long-term outcomes of pediatric cITP. We defined risk groups for each outcome, which will help clinical management and further studies.

Isocaloric Diets with Different Protein-Carbohydrate Ratios: The Effect on Sleep, Melatonin Secretion and Subsequent Nutritional Response in Healthy Young Men.

This study aimed to determine the short-term effect of two isocaloric diets differing in the ratio of protein−carbohydrate on melatonin levels, sleep, and subsequent dietary intake and physical activity in healthy young men. Twenty-four healthy men took part in a crossover design including two sessions of three days on isocaloric diets whether high-protein, low-carbohydrate (HPLC) or low-protein, high-carbohydrate (LPHC) followed by 24-h free living assessments. Sleep was measured by ambulatory polysomnography pre-post-intervention. Melatonin levels were assessed on the third night of each session on eight-point salivary sampling. Physical activity was monitored by accelerometry. On day 4, participants reported their 24-h ad-libitum dietary intake. LPHC resulted in better sleep quality and increased secretion of melatonin compared to HPLC. A significant difference was noted in sleep efficiency (p < 0.05) between the two sessions. This was mainly explained by a difference in sleep onset latency (p < 0.01) which was decreased during LPHC (PRE: 15.8 ± 7.8 min, POST: 11.4 ± 4.5 min, p < 0.001). Differences were also noted in sleep staging including time spent on REM (p < 0.05) and N1 (p < 0.05). More importantly, REM latency (PRE: 97.2 ± 19.9 min, POST 112.0 ± 20.7 min, p < 0.001) and cortical arousals (PRE: 7.2 ± 3.9 event/h, POST 8.5 ± 3.3 event/h) increased in response to HPLC diet but not LPHC. On day 4, 24-h ad-libitum energy intake was higher following HPLC compared to LPHC (+64 kcal, p < 0.05) and explained by increased snacking behavior (p < 0.01) especially from carbohydrates (p < 0.05). Increased carbohydrates intake was associated with increased cortical arousals.

Determinants of long-term outcomes of splenectomy in pediatric autoimmune cytopenias.

Splenectomy is effective in ∼70% to 80% of pediatric chronic immune thrombocytopenia (cITP) cases, and few data exist about it in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). Because of the irreversibility of the procedure and the lack of predictions regarding long-term outcomes, the decision to undertake splenectomy is difficult in children. We report here factors associated with splenectomy outcomes from the OBS'CEREVANCE cohort, which prospectively includes French children with autoimmune cytopenia (AIC) since 2004. The primary outcome was failure-free survival (FFS), defined as the time from splenectomy to the initiation of a second-line treatment (other than steroids and intravenous immunoglobulins) or death. We included 161 patients (cITP, n = 120; AIHA, n = 19; ES, n = 22) with a median (minimum-maximum) follow-up of 6.8 years (1.0-33.3) after splenectomy. AIC subtype was not associated with FFS. We found that immunopathological manifestations (IMs) were strongly associated with unfavorable outcomes. Diagnosis of an IM before splenectomy was associated with a lower FFS (hazard ratio [HR], 0.39; 95% confidence interval [CI], 0.21-0.72, P = .003, adjusted for AIC subtype). Diagnosis of an IM at any timepoint during follow-up was associated with an even lower FFS (HR, 0.22; 95% CI, 0.12-0.39; P = 2.8 × 10-7, adjusted for AIC subtype) as well as with higher risk of recurrent or severe bacterial infections and thrombosis. In conclusion, our results support the search for associated IMs when considering a splenectomy to refine the risk-benefit ratio. After the procedure, monitoring IMs helps to identify patients with higher risk of unfavorable outcomes.

Sleep pattern and staging in elite adolescent rugby players during the in-season competitive phase compared to an age matched non-athlete population.

Young athletes must contend with the constraints of elite sports on top of school commitments and the physiological processes associated with adolescence. This study assessed week and weekend sleep and schedule of activities in elite adolescent rugby players during the in-season competitive phase compared with age-matched non-athlete controls. 32 adolescents (GR: 16 elite rugby players, GC: 16 controls) from the same boarding school filled out a daily schedule of activities and a sleep diary, and wore a multichannel electroencephalogram for 14 days. They later filled out questionnaires on their sleep quality, sleepiness, and perceived stress. Both groups showed insufficient sleep duration during the week (<7 h). Only GC caught up on their sleep debt during the weekend (increased TIB, TST and time spent in REM sleep, all p < 0.001). Weekend TIB, TST and, N3 sleep remained similar to that for weekdays in GR. However, GR experienced lower sleep quality (decreased SE, increased WASO, all p < 0.01) and a decrease in REM sleep (p < 0.01). Schedules of activities showed an increase in time spent on overall activities during the weekend, mainly due to competition and sport-specific travel, which resulted in a decrease in sleep opportunity time compared with GC (p < 0.001). Δ sleep opportunity time (weekend-week) was associated with Δ TST (weekend-week), and Δ TST (weekend-week) was associated with sleepiness and perceived stress. Busy schedules during the competitive season decreased sleep opportunity time and prevented elite adolescent rugby players from catching up on their sleep at weekends.HIGHLIGHTSAdolescent rugby players have insufficient sleep during both week and weekend of the in-season competitive phaseCongested schedule prevented elite adolescent rugby players from catching up on their sleep at weekends.Sleep considerations are necessary when planning the weekend sporting schedules.

Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden.

Pediatric-onset Evans syndrome (pES) is defined by both immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) before the age of 18 years. There have been no comprehensive long-term studies of this rare disease, which can be associated to various immunopathological manifestations (IM). We report outcomes of the 151 patients with pES and more than 5 years of follow-up from the nationwide French prospective OBS'CEREVANCE cohort. Median age at final follow-up was 18.5 years (range, 6.8-50.0 years) and the median follow-up period was 11.3 years (range, 5.1-38.0 years). At 10 years, ITP and AIHA were in sustained complete remission in 54.5% and 78.4% of patients, respectively. The frequency and number of clinical and biological IM increased with age: at the age of 20 years, 74% had at least one clinical IM (cIM). A wide range of cIM occurred, mainly lymphoproliferation, dermatological, gastrointestinal/hepatic and pneumological IM. The number of cIM was associated with a subsequent increase in the number of second-line treatments received (other than steroids and immunoglobulins; hazard ratio 1.4, 95% Confidence Interval: 1.15-1.60, P=0.0002, Cox proportional hazards method). Survival at 15 years after diagnosis was 84%. Death occurred at a median age of 18 years (range, 1.7-31.5 years), and the most frequent cause was infection. The number of second-line treatments and severe/recurrent infections were independently associated with mortality. In conclusion, long-term outcomes of pES showed remission of cytopenias but frequent IM linked to high second-line treatment burden. Mortality was associated to drugs and/or underlying immunodeficiencies, and adolescents-young adults are a high-risk subgroup.

Eucaloric Balanced Diet Improved Objective Sleep in Adolescents with Obesity.

BACKGROUND: A better understanding of the influence of energy balance on sleep in adolescents, particularly those with obesity, could help develop strategies to optimize sleep in these populations. The purpose of this study was to investigate sleep under ad libitum-vs-controlled diets adjusted to energy requirement (eucaloric) among adolescents with obesity and their normal weight controls. METHODS: Twenty-eight male adolescents aged between 12 and 15 years, n = 14 adolescents with obesity (OB: BMI ≥ 90th centile) and n = 14 normal weight age matched controls (NW), completed an experimental protocol comprising ad libitum or eucaloric meals for three days, in random order. During the third night of each condition, they underwent in home polysomnography (PSG). RESULTS: An interaction effect of energy intake (EI) was detected (p < 0.001). EI was higher during ad libitum compared to the eucaloric condition (p < 0.001) and in OB compared to NW (p < 0.001) in the absence of any substantial modification to macronutrient proportions. Analyses of energy intake distribution throughout the day showed a significant interaction with both a condition and group effect during lunch and dinner. Sleep improvements were noted in OB group during the eucaloric condition compared to ad libitum with reduced sleep onset latency and N1 stage. Sleep improvements were correlated to reduced EI, especially during the evening meal. CONCLUSION: Simply adjusting dietary intake to energy requirement and reducing the energy proportion of the evening meal could have therapeutic effects on sleep in adolescents with obesity. However, positive energy balance alone cannot justify worsened sleep among adolescents with obesity compared to normal weight counterparts.

Effect of morning versus evening exercise training on sleep, physical activity, fitness, fatigue and quality of life in overweight and obese adults.

This study compares the effectiveness of a 12-week moderate exercise training program (METP), performed in the morning versus the evening, on sleep, physical activity, physical fitness, sleepiness, fatigue and health-related quality of life (HRQoL) in overweight and obese patients. Sedentary and inactive overweight/obese adults (n = 36) were included in METP and randomized into two groups: morning group (GM) and evening group (GE). Twenty-eight participants successfully completed METP (3 × 90 min exercise session per week for 12 weeks, completion rates >80%). Sleep, physical activity, and bedtime temperature were measured using accelerometry and infrared tympanic temperature during 3 separate weeks of the study (Week1, Week6, and Week12). Participants also took part in baseline and endpoint assessments including physical fitness as well as subjective physical activity, chronotype, sleep quality, sleepiness, fatigue and HRQoL. METP did not impact objective sleep quality differently between the two groups (morning vs evening). Bedtime and mid-sleep were advanced when METP was done in the morning whereas they were delayed when METP was practiced in the early evening (p = .003). Beside this finding, no valuable differences between the two groups were noted in all the remaining measures. METP resulted in improvements of body composition, cardiorespiratory and muscular endurance, as well as a favorable impact on subjective sleep quality, diurnal sleepiness, fatigue and HRQoL in both groups (all p < .05). Evening METP could be an effective alternative for overweight/obese adults when morning METP is not possible.

Muscle function and architecture in children with juvenile idiopathic arthritis.

AIM: To assess muscle function and functional abilities in children with juvenile idiopathic arthritis (JIA). METHODS: Fourteen children with JIA and 14 healthy controls matched for age and sex were included. Muscle characteristics, both structural (thickness, cross-sectional area (CSA) and fascicle angle) and qualitative (intermuscular adipose tissue; IMAT), were assessed in thigh muscles using ultrasound and peripheral quantitative computed tomography (pQCT). Muscle function and functional abilities were determined from the assessment of maximal voluntary isometric contraction (MVIC) knee extensors force and vertical jump performance. RESULTS: No significant difference in MVIC force was observed between the two groups. However, squat jump height was significantly reduced in children with JIA (18.3 ± 5.4 vs 24.3 ± 7.9 cm, P < .05). No differences in structural parameters were observed, but IMAT/CSA (0.22 ± 0.02 vs 0.25 ± 0.03; P = .01) was significantly lower in children with JIA than in healthy children. CONCLUSION: Knee extensor muscle architecture and force were comparable between children with and without JIA, but functional abilities (vertical jump performance) were poorer in JIA. The lower IMAT area in JIA could result from a lower physical activity level compared with healthy children.

Randomized Double-Blind Controlled Trial on the Effect of Proteins with Different Tryptophan/Large Neutral Amino Acid Ratios on Sleep in Adolescents: The PROTMORPHEUS Study.

Disturbed sleep is common in adolescents. Ingested nutrients help regulate the internal clock and influence sleep quality. The purpose of this clinical trial is to assess the effect of protein tryptophan (Trp)/large neutral amino acids (LNAAs) ratio on sleep and circadian rhythm. Ingested Trp is involved in the regulation of the sleep/wake cycle and improvement of sleep quality. Since Trp transport through the blood-brain barrier is competing with LNAAs, protein with higher Trp/LNAAs were expected to increase sleep efficiency. This randomized double-blind controlled trial will enroll two samples of male adolescents predisposed to sleep disturbances: elite rugby players (n = 24) and youths with obesity (n = 24). They will take part randomly in three sessions each held over a week. They will undergo a washout period, when dietary intake will be calibrated (three days), followed by an intervention period (three days), when their diet will be supplemented with three proteins with different Trp/LNAAs ratios. Physical, cognitive, dietary intake, appetite, and sleepiness evaluations will be made on the last day of each session. The primary outcome is sleep efficiency measured through in-home electroencephalogram recordings. Secondary outcomes include sleep staging, circadian phase, and sleep-, food intake-, metabolism-, and inflammation-related biochemical markers. A fuller understanding of the effect of protein Trp/LNAAs ratio on sleep could help in developing nutritional strategies addressing sleep disturbances.

Childhood Leukemia Survivors and Metabolic Response to Exercise: A Pilot Controlled Study.

BACKGROUND: Leukemia is the most common cancer in pediatrics, with many late effects such as higher risk of dyslipidemia, insulin resistance, obesity, and metabolic syndrome. The objective of this work was to investigate substrate oxidation during submaximal exercise in survivors of childhood acute leukemia. METHODS: A total of 20 leukemia survivors and 20 healthy children were matched by sex, age, and Tanner stage. They all took a submaximal incremental exercise test to determine fat and carbohydrate oxidation rates. RESULTS: Cardiorespiratory fitness was significantly lower in leukemia survivors, with lower relative VO2 peaks (p < 0.001), lower heart rate values (p = 0.02), and lower exercise power (p = 0.012), whereas rest metabolism and body mass index did not differ between the two groups. During exercise, upward of heart rate relative to VO2 peak was significantly higher (p < 0.001) in childhood leukemia survivors. We found lower carbohydrate and fat oxidation rates (p = 0.07) in leukemia survivors compared with healthy children, and also a significantly lower relative maximal fat oxidation rate (p = 0.014). CONCLUSION: Despite impaired physical fitness and metabolic response to exercise, childhood leukemia survivors remained sensitive to physical activity interventions, and could readily adapt to submaximal exercise intensity.

HSCT may lower leukemia risk in ELANE neutropenia: a before-after study from the French Severe Congenital Neutropenia Registry.

ELANE neutropenia is associated with myelodysplasia and acute leukemia (MDS-AL), and severe infections. Because the MDS-AL risk has also been shown to be associated with exposure to GCSF, since 2005, in France, patients receiving high daily GCSF doses (>15 µg/kg/day) are eligible for HSCT, in addition to classic indications (MDS-AL or GCSF refractoriness). We analyzed the effect of this policy. Among 144 prospectively followed ELANE-neutropenia patients enrolled in the French Severe Congenital Neutropenia Registry, we defined two groups according to period: "before 2005" for those born before 2005 and followed until 31/12/2004 (1588 person-years); and "after 2005" comprised of those born after 2005 or born before 2005 but followed after 2005 until 31/03/2019 (1327 person-years). Sixteen of our cohort patients underwent HSCT (14 long-term survivors) and six developed MDS-ALs. Six leukemic transformations occurred in the before-2005 group and none after 2005 (respective frequencies 3.8 × 10-3 vs. 0; P < 0.01), while four HSCTs were done before 2005 and 12 since 2005 (respective HSCT rates increased 2.5 × 10-3 vs. 9 × 10-3; P < 0.01). Our results support early HSCT for patients with ELANE mutations who received high GCSF doses, as it might lower the risk of leukemic transformation.

Large-Scale Assessment of Health-Related Physical Fitness in French Older Adults: Feasibility and Validity.

Objectives: This study aims to assess the validity, internal consistency, implementation, and feasibility of a sequence of tests, the Vitality Test Battery, designed to measure physical fitness, at a large scale in French older adults. Methods: A total of 528 volunteers (age ≥60 years) took the battery of 10 tests: 6-min walk, trunk strength, hand grip strength, medicine ball throwing, 30-s chair stand, flexibility, balance, plate tapping, ruler drop, and dual task. Results: Internal consistency was high, with the Cronbach alpha coefficients at around 0.77, explaining 64% of the variance. The test-retest correlations (0.3-0.6) between the items were acceptable and displayed an internal consistency property. Although five components explained 65% of the variance, all the items were kept because their eigenvalues were near to 0.9. External consistency was validated by a significant decrease in fitness scores (p < 0.001) with age and body mass index. Discussion: The Vitality Test Battery is a safe, valid tool for assessing physical fitness in persons aged over 60 years.

Effect of Drop Height on Vertical Jumping Performance in Pre-, Circa-, and Post-Pubertal Boys and Girls.

PURPOSE: To examine the effect of drop height on vertical jumping performance in children with respect to sex and maturity status. METHODS: Thirty-seven pre-pubertal, 71 circa-pubertal, and 69 post-pubertal boys and girls performed, in a randomized order, 2 squat jumps, 2 countermovement jumps, and 2 drop jumps (DJ) from heights of 20, 30, 40, 50, 60, and 70 cm. The trial with the best jump height in each test was used for analysis. RESULTS: No significant sex × maturity status × jump type interaction for jump height was observed. However, on average, the children jumped higher in the countermovement jump than in squat jump and DJs (+1.2 and +1.6 cm, P < .001, respectively), with no significant differences between DJs and squat jumps or between DJs when increasing drop heights. Regarding DJs, 59.3% of the participants jumped higher from drop heights of 20 to 40 cm. CONCLUSIONS: Children, independent of sex and maturity status, performed best in the countermovement jump, and no performance gain was obtained by dropping from heights of 20 to 70 cm. During maturation, the use of drop heights between 20 and 40 cm may be considered in plyometric training, but the optimum height must be obtained individually.

A 1-Year Prospective French Nationwide Study of Emergency Hospital Admissions in Children and Adults with Primary Immunodeficiency.

PURPOSE: Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying "at-risk" patient profiles. METHODS: We performed a prospective observational 12-month multicenter study in France via the CEREDIH network of regional PID reference centers from November 2010 to October 2011. All patients with PIDs requiring emergency hospital admission were included. RESULTS: A total of 200 admissions concerned 137 patients (73 adults and 64 children, 53% of whom had antibody deficiencies). Thirty admissions were reported for 16 hematopoietic stem cell transplantation recipients. When considering the 170 admissions of non-transplant patients, 149 (85%) were related to acute infections (respiratory tract infections and gastrointestinal tract infections in 72 (36%) and 34 (17%) of cases, respectively). Seventy-seven percent of the admissions occurred during winter or spring (December to May). The in-hospital mortality rate was 8.8% (12 patients); death was related to a severe infection in 11 cases (8%) and Epstein-Barr virus-induced lymphoma in 1 case. Patients with a central venous catheter (n = 19, 13.9%) were significantly more hospitalized for an infection (94.7%) than for a non-infectious reason (5.3%) (p = 0.04). CONCLUSION: Our data showed that the annual incidence of emergency hospital admission among patients with PID is 3.4%. The leading cause of emergency hospital admission was an acute infection, and having a central venous catheter was associated with a significantly greater risk of admission for an infectious episode.