Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study.

The optimal duration of eculizumab treatment in patients with atypical hemolytic uremic syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter open-label study to assess eculizumab discontinuation in children and adults with aHUS. Fifty-five patients (including 19 children) discontinued eculizumab (mean treatment duration, 16.5 months). Twenty-eight patients (51%) had rare variants in complement genes, mostly in MCP (n = 12; 22%), CFH (n = 6; 11%), and CFI (n = 6; 10%). At eculizumab discontinuation, 17 (30%) and 4 patients (7%) had stage 3 and 4 chronic kidney disease, respectively. During follow-up, 13 patients (23%; 6 children and 7 adults) experienced aHUS relapse. In multivariable analysis, female sex and presence of a rare variant in a complement gene were associated with an increased risk of aHUS relapse, whereas requirement for dialysis during a previous episode of acute aHUS was not. In addition, increased sC5b-9 plasma level at eculizumab discontinuation was associated with a higher risk of aHUS relapse in all patients and in the subset of carriers with a complement gene rare variant, both by log-rank test and in multivariable analysis. Of the 13 relapsing patients, all of whom restarted eculizumab, 11 regained their baseline renal function and 2 had a worsening of their preexisting chronic kidney disease, including 1 patient who progressed to end-stage renal disease. A strategy of eculizumab discontinuation in aHUS patients based on complement genetics is reasonable and safe. It improves the management and quality of life of a sizeable proportion of aHUS patients while reducing the cost of treatment. This trial was registered at www.clinicaltrials.gov as #NCT02574403.

Effects of upper airway obstruction or hypoxia on gastroesophageal reflux in newborn lambs.

BACKGROUND: Although it is commonly accepted that upper airway obstruction (UAO) increases gastroesophageal reflux (GER), the link is poorly understood and insufficiently documented. In addition, while hypoxia is often encountered in infants with UAO, its consequences on GER are virtually unknown. The two aims of the present study were to characterize the effects of (1) UAO and (2) hypoxia on GER. METHODS: Seventeen lambs underwent polysomnographic and esophageal impedance/pH-metry monitoring during UAO vs. a control condition (6 h, ten lambs) or 10% hypoxia vs. normoxic condition (3 h, seven other lambs). RESULTS: Moderate-to-severe UAO was maintained throughout monitoring (inspiratory tracheal pressure of -13 (-15, -12) cm H2O vs. -1 (-1, -1) cm H2O in control condition, p = 0.005). While the number of GERs increased with UAO (2 (1, 4) vs. 0 (0, 3) in the control condition, p = 0.03), the increase was less than anticipated and inconsistent among the lambs. Also, sustained 10% hypoxia did not alter the number of GERs (2 (1, 3) vs. 0 (0, 5) in the control condition, p = 0.9). CONCLUSIONS: The presence of an UAO for 6 h mildly increased the number of GERs, whereas hypoxia for 3 h had no significant effect. IMPACT: The effect of upper airway obstruction and hypoxia on gastroesophageal reflux is poorly documented in the neonatal period. A moderate-to-severe upper airway obstruction for 6 h results in a mild, inconsistent increase in the number of gastroesophageal refluxes. Overall, a hypoxia of 10% for 3 h had no significant impact on gastroesophageal reflux. The prescription of an antireflux medication in infants with upper airway obstruction must not be systematic but rely on objective signs of a pathologic gastroesophageal reflux.

Effects of Smoking Exposure in Infants on Gastroesophageal Reflux as a Function of the Sleep-Wakefulness State.

OBJECTIVE: To determine whether perinatal smoking exposure is associated with gastroesophageal reflux (GER)-related changes in sleep-wakefulness states in neonates. STUDY DESIGN: Thirty-one neonates, referred for the investigation of suspected GER, were recruited and underwent multichannel impedance-pH monitoring and synchronized 8- to 12-hour polysomnography. The infants' exposure to tobacco smoke was estimated by means of a urine cotinine assay. The total number, frequency (h-1), and mean duration (minutes) of GER-pH (reflux events detected by the pH electrode only) and GER-imp (reflux events with bolus movement detected by impedance) events were determined. Intergroup differences (smoking-exposed group vs nonexposed group) were probed with nonparametric, unpaired Mann-Whitney U tests. A χ2 test was used to assess a possible intergroup difference in bolus retrograde migration during GER-imp events. RESULTS: According to the urine cotinine assay, 21 of the 31 neonates had been exposed to cigarette smoke during the perinatal period. The number (and frequency) of GER-imp was significantly greater (P = .016) in the exposed group (29 [0-90]) than in the nonexposed group (12 [2-35]). Migration of the esophageal bolus from the distal segment to the most proximal segment was significantly more frequent (P = .016) in the exposed group (83% of GER) than in the nonexposed group (41%). The GER pattern associated with smoking exposure was particularly obvious during Rapid eye movement sleep. CONCLUSIONS: The more frequent occurrence and greater proximal migration of GER-imp in the smoking-exposed group (especially during rapid eye movement sleep) may have clinical relevance. Smoking exposure is a preventable risk factor for limiting the occurrence of GER in neonates.

Clinical and Genetic Spectrum of Bartter Syndrome Type 3.

Bartter syndrome type 3 is a clinically heterogeneous hereditary salt-losing tubulopathy caused by mutations of the chloride voltage-gated channel Kb gene (CLCNKB), which encodes the ClC-Kb chloride channel involved in NaCl reabsorption in the renal tubule. To study phenotype/genotype correlations, we performed genetic analyses by direct sequencing and multiplex ligation-dependent probe amplification and retrospectively analyzed medical charts for 115 patients with CLCNKB mutations. Functional analyses were performed in Xenopus laevis oocytes for eight missense and two nonsense mutations. We detected 60 mutations, including 27 previously unreported mutations. Among patients, 29.5% had a phenotype of ante/neonatal Bartter syndrome (polyhydramnios or diagnosis in the first month of life), 44.5% had classic Bartter syndrome (diagnosis during childhood, hypercalciuria, and/or polyuria), and 26.0% had Gitelman-like syndrome (fortuitous discovery of hypokalemia with hypomagnesemia and/or hypocalciuria in childhood or adulthood). Nine of the ten mutations expressed in vitro decreased or abolished chloride conductance. Severe (large deletions, frameshift, nonsense, and essential splicing) and missense mutations resulting in poor residual conductance were associated with younger age at diagnosis. Electrolyte supplements and indomethacin were used frequently to induce catch-up growth, with few adverse effects. After a median follow-up of 8 (range, 1-41) years in 77 patients, chronic renal failure was detected in 19 patients (25%): one required hemodialysis and four underwent renal transplant. In summary, we report a genotype/phenotype correlation for Bartter syndrome type 3: complete loss-of-function mutations associated with younger age at diagnosis, and CKD was observed in all phenotypes.

Presentation and endoscopic management of sigmoid volvulus in children.

UNLABELLED: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients. Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy. CONCLUSION: Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence. WHAT IS KNOWN: • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.

A patient with Simpson-Golabi-Behmel syndrome, biliary cirrhosis and successful liver transplantation.

Simpson-Golabi-Behmel syndrome type 1 (SGBS1) -OMIM 312870- is a rare X-linked inherited overgrowth syndrome caused by a loss of function mutation in the GPC3 gene. Affected patients present a variable phenotype with pre- and post-natal macrosomia, distinctive facial dysmorphism, organomegaly, and multiple congenital anomalies. Intellectual disability is not constant. About 10% of patients have an increased risk of developing embryonic tumors in early childhood. Only one case of biliary disease has been described so far. GPC3 is localized on Xq26. It encodes for glypican 3, a heparan sulfate proteoglycan, which among its different known roles, negatively regulates liver regeneration and hepatocyte proliferation. This report concerns a male with a SGBS1, carrier of a GPC3 pathogenic mutation, and neonatal liver disease, who developed an early biliary cirrhosis. Together with the associated risk of cancer and developmental delay, liver transplantation was discussed and then successfully performed at the age of 19 months. A hypothesis on the role of GPC3 in the patient's liver disease is also proposed.

Mortality and cancer in pediatric-onset inflammatory bowel disease: a population-based study.

OBJECTIVES: Although the incidence of pediatric inflammatory bowel disease (IBD) continues to rise in Northern France, the risks of death and cancer in this population have not been characterized. METHODS: All patients <17 years, recorded in EPIMAD registry, and diagnosed between 1988 and 2004 with Crohn's disease (CD) or ulcerative colitis (UC) were included. The observed incidences of death and cancer were compared with those expected in the regional general population obtained by French Statistical Institute (INSEE) and the cancer Registry from Lille. Comparisons were performed using Fisher's exact test and were expressed using the standardized mortality ratios (SMRs) and standardized incidence ratios. RESULTS: A total of 698 patients (538 with CD and 160 with UC) were identified; 360 (52%) were men, the median age at IBD diagnosis was 14 years (12-16) and the median follow-up time was 11.5 years (7-15). During follow-up, the mortality rate was 0.84% (6/698) and did not differ from that in the reference population (SMR=1.4 (0.5-3.0); P=0.27). After a median follow-up of 15 years (10-17), 1.3% of patients (9/698) had a cancer: colon (n=2), biliary tract (cholangiocarcinoma; n=1), uterine cervix (n=1), prepuce (n=1), skin (basal cell carcinoma (n=2), hematological (acute leukemia; n=1), and small bowel carcinoid (n=1). There was a significantly increased risk of cancer regardless of gender and age (standardized incidence ratio=3.0 (1.3-5.9); P<0.02). Four out of nine patients who developed a cancer had received immunosuppressants or anti-tumor necrosis factor-α therapy (including combination therapy in three patients). CONCLUSIONS: In this large pediatric population-based IBD cohort, mortality did not differ from that of the general population but there was a significant threefold increased risk of neoplasia.

Milk temperature influences esophageal motility in the newborn lamb.

Esophageal dysmotility is common in infants. We aimed to evaluate the influence of milk temperature on esophageal motility using multichannel intraluminal impedance-pH monitoring (MII-pH). Five healthy lambs, ages 2 to 3 days, underwent a MII-pH whereas bottle-fed randomly with 50 mL of ewe milk at 26 °C, 38.5 °C, and 41.5 °C. Impedance motility parameters were studied on 5 swallows at each temperature. At 38.5 °C we noted a higher total propagation velocity and a shorter total bolus transit time (TBTT) (P < 0.05). These unique results suggest a potential role of milk temperature alterations in improving oral feeding in infants with esophageal dysmotility.

Absence of effect of nasal continuous positive airway pressure on the esophageal phase of nutritive swallowing in newborn lambs.

OBJECTIVES: It is presently recommended that oral feeding be started in premature infants as soon as possible, often at an age at which nasal continuous positive airway pressure (nCPAP) is still required for ventilatory support. Our previous data showed that application of nCPAP up to 10 cmH2O in full-term lambs had no deleterious effect on cardiorespiratory safety, feeding efficiency, or on nutritive swallowing-breathing coordination. Besides fear of swallowing-breathing coordination disturbances, esophageal motility disruption by nCPAP could be a reason to delay oral feeding. To our knowledge, no study has focused on the effects of nCPAP on esophageal motility in the neonatal period. The aim of the present study was therefore to further assess the effects of nCPAP on oral feeding by assessing its effects on the esophageal phase of nutritive swallowing (nutritive esophagodeglutition). METHODS: Six full-term lambs, ages 2 to 3 days, underwent esophageal multichannel intraluminal impedance-pH monitoring. Lambs were bottle-fed under 2 randomized conditions, namely spontaneous breathing and nCPAP 6 cmH(2)O. RESULTS: Beyond confirmation of unaltered feeding efficiency, analysis of multiple variables measured by impedance monitoring revealed that nCPAP 6 does not alter nutritive esophagodeglutition in any way (nCPAP vs spontaneous breathing, P > 0.1 for all variables). CONCLUSIONS: offering further support to neonatologists pleading for initiation of oral feeding in infants still on nCPAP, the present results set the foundations for similar clinical studies in preterm human infants to confirm the absence of effects of nCPAP on nutritive swallowing.

Relationship between sleep and acid gastro-oesophageal reflux in neonates.

The aim of the present study was to investigate the impact of gastro-oesophageal acid reflux on sleep in neonates and, reciprocally, the influence of wakefulness (W) and sleep stages on the characteristics of the reflux (including the retrograde bolus migration of oesophageal acid contents). The pH and multichannel intraluminal impedance were measured during nocturnal polysomnography in 25 infants hospitalised for suspicion of gastro-oesophageal reflux. Two groups were constituted according to whether or not the infants displayed gastro-oesophageal reflux (i.e. a reflux group and a control group). There were no differences between the reflux and control groups in terms of sleep duration, sleep structure and sleep state change frequency. Vigilance states significantly influenced the gastro-oesophageal reflux pattern: the occurrence of gastro-oesophageal reflux episodes was greater during W (59 ± 32%) and active sleep (AS; 35 ± 30%) than during quiet sleep (QS; 6 ± 11%), whereas the mean duration of gastro-oesophageal reflux episodes was higher in QS than in W and AS. The percentage of retrograde bolus migrations of distal oesophageal acid content was significantly higher in AS (62 ± 26%) than in W (42 ± 26%) and QS (4.5 ± 9%). In neonates, gastro-oesophageal reflux occurred more frequently during W, whereas the physiological changes associated with sleep state increase the physiopathological impact of the gastro-oesophageal reflux. The duration of oesophagus-acid contact was greater during sleep; AS facilitated the retrograde migration of oesophageal acid content, and QS was characterised by the risk of prolonged acid mucosal contact.

Newborn lamb as a new model for studying gastroesophageal reflux.

We aimed to determine whether the newborn lamb at term is a valid model for studying gastroesophageal reflux. Seven bottle-fed lambs, ages 2 to 3 days, underwent esophageal multichannel intraluminal impedance-pH monitoring (MII-pH). A total of 196 reflux episodes were recorded, including 73% alkaline and 27% weakly acidic. No acid refluxes were observed. Median bolus clearance time was 4  seconds (3; 5.5), and proximal reflux extent was 35% (26). This first report of MII-pH in the newborn mammal sets the foundations for future studies with physiological and clinical relevance to human neonates.

Physical fitness in children and adolescents with inflammatory bowel disease: protocol for a case-control study.

INTRODUCTION: Inflammatory bowel disease (IBD) is a chronic disorder of the gastrointestinal tract, associated with adverse health consequences that may adversely influence physical activity and body composition in youth. These effects may lead to changes in physical fitness, which is positively associated with health-related outcomes. The aim is to assess health-related physical fitness levels in paediatric patients with IBD and to compare these levels with those in healthy matched controls. METHODS AND ANALYSIS: This trial is a bicentric case-control study. Fifty paediatric patients with IBD and 50 matched healthy controls will be recruited (1:1), and physical fitness levels (cardiorespiratory fitness, muscular strength, speed/agility and flexibility) will be assessed. The primary outcome is cardiorespiratory fitness, which will be compared between children and adolescents with IBD and healthy controls matched for age, sex and body mass index class. We will assess whether the two groups differ with respect to other physical fitness components and cardiovascular risk in adulthood according to sex-specific cut-offs for a healthy cardiorespiratory fitness level in adolescents. We will identify relationships between physical fitness and characteristics of IBD, quality of life and daily physical activity. ETHICS AND DISSEMINATION: This study was approved by the Research Ethics Committee (Comité de Protection des Personnes, Centre-Ouest I, Tours, France; No 2019-A02651-56) and was declared to the Commission Nationale de l'Informatique et des Libertés. All procedures will be performed according to the ethical standards of the 1975 Declaration of Helsinki, as revised in 2008, and the European Union's Guidelines for Good Clinical Practice. Written informed consent will be obtained from the youths and their parents. Research findings will be disseminated in peer-reviewed journals and scientific meetings, as well as in social media and IBD family support groups. TRIAL REGISTRATION NUMBER: NCT04647578.

Management of Central Venous Catheters in Children and Adults on Home Parenteral Nutrition: A French Survey of Current Practice.

Although central venous catheter (CVC)-related thrombosis (CRT) is a severe complication of home parenteral nutrition (HPN), the amount and quality of data in the diagnosis and management of CRT remain low. We aimed to describe current practices regarding CVC management in French adult and pediatric HPN centers, with a focus on CVC obstruction and CRT. Current practices regarding CVC management in patients on HPN were collected by an online-based cross-sectional survey sent to expert physicians of French HPN centers. We compared these practices to published guidelines and searched for differences between pediatric and adult HPN centers' practices. Finally, we examined the heterogeneity of practices in both pediatric and adult HPN centers. The survey was completed by 34 centers, including 21 pediatric and 13 adult centers. We found a considerable heterogeneity, especially in the responses of pediatric centers. On some points, the centers' responses differed from the current guidelines. We also found significant differences between practices in adult and pediatric centers. We conclude that the management of CVC and CRT in patients on HPN is a serious and complex situation for which there is significant heterogeneity between HPN centers. These findings highlight the need for more well-designed clinical trials in this field.

Feeding disorders in children with oesophageal atresia: a cross-sectional study.

INTRODUCTION: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD. METHODS: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale. RESULTS: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication. CONCLUSION: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth.

Renal Prognosis in Children With Tubulointerstitial Nephritis and Uveitis Syndrome.

INTRODUCTION: Tubulointerstitial nephritis (TIN) and uveitis (TINU) syndrome is a rare disease. The renal prognosis is generally thought to be better in children with TINU syndrome than in adults. However, data are scarce. We aimed to investigate the long-term renal prognosis in a French cohort of children with TINU syndrome. METHODS: We performed a national retrospective study including 23 French pediatric nephrology centers enrolling patients with TINU syndrome diagnosed between January 2000 and December 2018. RESULTS: A total of 46 patients were included (52% female, median age 13.8 years). At diagnosis of TIN, the median estimated glomerular filtration rate (eGFR) was 30.6 ml/min per 1.73 m2 (4.9-62.8). The median time between diagnosis of uveitis and TIN was 0.4 months (-4.1; +17.1). All patients had anterior uveitis, but 12 (29%) were asymptomatic. Nearly all patients (44 of 46) received steroid treatment, and 12 patients (26%) received a second-line therapy. At last follow-up (median 2.8 years), the median eGFR was 87.5 ml/min per 1.73 m2 (60.3-152.7) and <90 ml/min per 1.73 m2 in 20 patients. CONCLUSION: In our study, nearly half of the patients had renal sequelae at last follow-up. Given the possible progression to chronic kidney disease, long-term monitoring of children with TINU syndrome is mandatory. Approximately a quarter of the children had asymptomatic uveitis suggesting all children presenting with TIN should undergo systematic ophthalmologic screening even in the absence of ocular signs.

Functional abdominal pain disorders and patient- and parent-reported outcomes in children with inflammatory bowel disease in remission.

BACKGROUND: Chronic abdominal pain occurs frequently in pediatric patients with inflammatory bowel disease (IBD) in remission. AIMS: To assess the prevalence and factors associated with Functional Abdominal Pain Disorders among IBD children in remission (IBD-FAPD). METHODS: Patients with IBD for > 1 year, in clinical remission for ≥ 3 months were recruited from a National IBD network. IBD-FAPDs were assessed using the Rome III questionnaire criteria. Patient- or parent- reported outcomes were assessed. RESULTS: Among 102 included patients, 57 (56%) were boys, mean age (DS) was 15.0 (± 2.0) years and 75 (74%) had Crohn's disease. Twenty-two patients (22%) had at least one Functional Gastrointestinal Disorder among which 17 had at least one IBD-FAPD. Past severity of disease or treatments received and level of remission were not significantly associated with IBD-FAPD. Patients with IBD-FAPD reported more fatigue (peds-FACIT-F: 35.9 ± 9.8 vs. 43.0 ± 6.9, p = 0.01) and a lower HR-QoL (IMPACT III: 76.5 ± 9.6 vs. 81.6 ± 9.2, p = 0.04) than patients without FAPD, and their parents had higher levels of State and Trait anxiety than the other parents. CONCLUSIONS: Prevalence of IBD-FAPD was 17%. IBD-FAPD was not associated with past severity of disease, but with fatigue and lower HR-QoL.

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.

OBJECTIVES: To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). METHODS: Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis

Protocol of a randomised controlled trial assessing the impact of physical activity on bone health in children with inflammatory bowel disease.

INTRODUCTION: Low bone mineral density (BMD) is a frequent issue in children and adolescents with inflammatory bowel disease (IBD). Several studies in healthy populations have reported a positive impact of physical activity (PA) on bone health. Recently, an observational study in paediatric patients with IBD showed a significant positive relationship between daily PA and BMD. However, intervention studies investigating a causal relationship between PA and BMD are warranted to confirm these results. The aim of this randomised controlled trial will be to investigate the effect of a PA programme on BMD in paediatric patients with IBD. METHODS AND ANALYSIS: This trial is a multicentre (four centres), randomised, controlled, blinded end-point study. Eighty children with IBD will be randomly assigned in a 1:1 ratio to receive a programme with adapted physical exercises (intervention group) or usual PA (control group) during a 9-month period. The primary outcome is the change from baseline at 9 months (the end of the study) in whole-body BMD assessed by dual-energy X-ray absorptiometry. Secondary efficacy outcomes include the changes from baseline at 9 months in: BMD assessed in the lumbar spine and trochanter; daily PA (time spent in moderate-to-vigorous PA); body composition (fat mass and fat-free mass); fatigue resistance; quality of life and activity of IBD. ETHICS AND DISSEMINATION: The study was approved by the Research Ethics Committee in France (Comité de Protection des Personnes, Sud-Ouest and Outre-Mer III, Bordeaux, France, No 2018/27). All procedures will be performed according to the ethical standards of the Helsinki Declaration of 1975, as revised in 2008, and the European Union's Guidelines for Good Clinical Practice. Written informed consent will be obtained from the parents or legal guardian and from the children. Research findings will be disseminated in peer-reviewed journals and scientific meetings. TRIAL REGISTRATION NUMBER: NCT03774329.

Physical activity is associated with improved bone health in children with inflammatory bowel disease.

BACKGROUND & AIMS: Bone health is an important concern in patients with inflammatory bowel disease (IBD). Low bone mineral density (BMD) is a powerful predictor of fracture risk in IBD patients. Physical activity (PA) plays an important role in bone health. However, PA data for children and adolescents with IBD are scarce. The primary aim is to evaluate the relationship between PA and BMD in children with IBD. The secondary aim was to assess the relationship between PA and quality of life. METHODS: Eighty-four IBD paediatric patients (45 boys) aged 14.3 ± 2.7 years were included (disease activity: (i) remission, n = 62; (ii) mild, n = 18; (iii) severe disease, n = 1). BMD was measured using dual-energy X-ray absorptiometry and expressed as age- and sex-based Z-scores. Each patient wore a triaxial accelerometer for seven consecutive days for objective PA quantification. Quality of life was assessed using the PedsQL™ and energy intake was assessed prospectively for three days using a dietary diary. RESULTS: BMD Z-score was -0.96 ± 1.11. Only five patients (6%) fulfilled the recommendation of 60 min of daily moderate-to-vigorous PA (MVPA). The proportion of children with osteopenia and osteoporosis was 51% and 4%, respectively. After adjustment for confounders (pubertal status and body mass index), total PA and time in MVPA were positively associated with BMD (regression coefficient per one standard deviation increase in PA parameters = 0.26; P < 0.05). There was no association between time spent in MVPA and total PA, and total quality of life score. CONCLUSIONS: PA likely is associated with improved bone health in IBD children. Intervention studies investigating a causal relationship between PA and BMD in paediatric patients with IBD are warranted.