L’évolution à long terme, le fonctionnement de l’intestin et la qualité de vie des patients affectés par la maladie de Hirschsprung : étude prospective cas-témoins

Introduction L’évolution à long terme de la maladie de Hirschsprung (HSCR) est souvent associée à des complications et à un dysfonctionnement de l’intestin, source de conséquences importantes sur la qualité de vie (QdV). L’objectif principal de cette étude est, dans une cohorte québécoise de HSCR, d’étudier la QdV en utilisant un outil spécifique et d’en déterminer les facteurs prédictifs. Méthodes Étude prospective de cohorte et cas-témoins. Les questionnaires 'HAQL' (QdV spécifique de HSCR), 'PedsQL’ (QdV générale), sur le stress et sur la situation sociale ont été administrés, ainsi qu’un journal des selles. Le contenu en cortisol des cheveux (CCC) (mesure du stress chronique) a été quantifié par dosage immuno-enzymatique (ELISA). Résultats 72 patients (72% garçons) et 117 contrôles (65% garçons) ont été analysés. L'âge médian [IQR1-IQR3] à l'inclusion était de 12,1 ans [8-17,5] et 12,6 ans [10,2-15,1] respectivement. La QdV liée à la santé globale était comparable entre les patients et les contrôles. Parmi les patients, la QdV spécifique à la HSCR mesurée par le HAQL était inférieure chez les enfants de 8 à11 ans par rapport aux adolescents de 12 à 16 ans (valeurs moyenne 539,3±66,5/700 vs. 622,6± 54,6/700; p=0,002). La dimension ‘Continence fécale pendant la journée’ était la plus affectée chez les patients âgés de 8 à 11 ans (valeur moyenne 52,6±25,3 sur 100). Chez les patients 12 à 16 ans, la dimension la plus affectée était le ‘Fonctionnement physique’. La prévalence de l'incontinence fécale mesurée chez les patients était de 85% chez les enfants, de 40% chez les adolescents et de 12% chez les adultes. Une association significative entre incontinence fécale et jeune âge était vu (p=<0,0001). Aucune association n'a été démontrée entre incontinence fécale et stress chronique (HCC, événements stressants) ou situation sociale chez les patients. Conclusion L’étude de la QdV par un outil spécifique de la HSCR est nécessaire pour une évaluation adéquate de l’état psychosocial dans cette population qui est à risque d’un dysfonctionnement de l’intestin à long terme.Introduction Multimorbidity and bowel dysfunction are affecting patients with Hirschsprung disease (HSCR) on long-term follow-up, having an important impact on quality of life (QoL). The primary aim of this study is to evaluate the disease-specific QoL with determination of its predictive factors in a French-Canadian cohort of HSCR patients. Methods Prospective cohort and case-control study. The questionnaires ‘HAQL’ (disease-specific QoL questionnaire), 'PedsQL’ (global health related QoL), ‘Stressful life events’, a questionnaire about the socio-economic state and a stool diary were requested to fill in. Hair cortisol concentration (HCC) (measure of chronic stress) was measured using an enzyme-linked immunosorbent assay kit (ELISA). Results 72 patients (72% males) and 117 controls (65% males) were analyzed. Median [IQR1, IQR3] age at study inclusion was 12.1 years [8,17.5] in patients and 12.6 years [10.2,15.1] in controls. General health related QoL was comparable between patients and controls. In the patient’s group, children 8 to 11 years reported lower disease-specific QoL than adolescents (12 to 16 years) (mean scores 539.3±66.5/700 vs. 622.6±54.6/700; p=0.002). The dimension ‘Fecal continence during daytime’ was the most affected one in children (mean score 52.6± 25.3/100) and the dimension ‘Physical functioning’ was the most affected one in adolescents. Prevalence of fecal incontinence/soiling in the patient’s group was 85% in children, 40% in adolescents and 12% in adults. Younger age was associated with a higher prevalence of fecal incontinence (p=<0,0001). No association was seen between presence of fecal soiling/incontinence and chronic stress (HCC, Stressful live events) or social situation. Conclusion Disease-specific QoL investigation is mandatory in HSCR patients, to encounter and evaluate adequately psychosocial problems related to long term bowel dysfunction

Drug-related adverse events necessitating treatment discontinuation in pediatric inflammatory bowel disease patients.

BACKGROUND AND AIMS Inflammatory bowel disease (IBD) requires long-term drug therapy in most patients, posing a risk for adverse drug events with the need for discontinuation. In this study, we investigated adverse events (AE) necessitating drug discontinuation in pediatric and adolescent IBD patients. METHODS We used data prospectively collected from IBD patients below the age of 18 enrolled in the Swiss Inflammatory Bowel Disease Cohort Study (SIBDCS), namely demographic variables, medical characteristics, drug treatments and related AE. We analysed the frequency, type, and risk factors for AE necessitating drug discontinuation. RESULTS A total of 509 pediatric IBD patients fulfilled the inclusion criteria of which 262 (51.5%) were diagnosed with Crohn's disease (CD), 206 (40.5%) with ulcerative colitis (UC), and 41 (8%) with IBD-unclassified (IBD-U). In total, 132 (25.9%) presented with at least one drug-related AE that required drug cessation. Immunomodulators (methotrexate 29/120 (24.2%), azathioprine 57/372 (15.3%)) followed by tumor necrosis factor (TNF)-alpha antagonists (adalimumab 8/72 (11.1%), infliximab 22/227 (9.7%)) accounted for the highest proportions of AE necessitating treatment discontinuation. Treatment schemes with at least 3 concomitant drugs significantly amplified the risk for development of drug-related AE (OR = 2.50, 95%CI [1.50-4.17]) in all pediatric IBD patients. CONCLUSIONS Drug-related AE necessitating discontinuation are common in pediatric and adolescent inflammatory bowel disease patients. Caution needs to be taken in the case of concomitant drug use

Dysmotility in Esophageal Atresia: Pathophysiology, Characterization, and Treatment

Esophageal dysmotility is almost universal after esophageal atresia (EA) repair and is mainly related to the developmental anomaly of the esophagus. Esophageal dysmotility is involved in the pathophysiology of numerous symptoms and comorbidities associated with EA such as gastroesophageal reflux disease, aspiration and respiratory complications, and symptoms of dysphagia and feeding disorders. High-resolution esophageal manometry (HREM) has facilitated the characterization of the dysmotility, but there is an incomplete correlation between symptoms and manometrical patterns. Impedance coupled to HREM should help to predict the clinical outcome and therefore personalize patient management. Nowadays, the management of esophageal dysmotility in patients with EA is essentially based on treatment of associated inflammation related to peptic or eosinophilic esophagitis

The Assessment of Steroid Injections as a Potential Risk Factor for Osteochondral Lesions in Children with Juvenile Idiopathic Arthritis

Objective: Intra-articular corticosteroid injections (IACIs) are frequently used to suppress local inflammation, that is, in children with juvenile idiopathic arthritis (JIA). While systemic high-dosage corticosteroids are known to trigger osteonecrosis and result in osteochondral (OC) lesions, the effect of IACIs on joint cartilage and subchondral bone remains unclear. This study was conceived to analyze the coincidence of IACI and the subsequent manifestation of osteochondral lesions in a large cohort of pediatric JIA patients. Design: Retrospective data assessment and comparative analysis of skeletally immature JIA patients treated with IACIs between 1993 and 2017. Results: A total of 280 JIA patients were included in the analysis, the majority were girls (64%). Osteochondral lesions were present in 16 patients (5.7%) at a mean age of 10.7 years (range 4-14 years) and appeared on average after 63-month duration of disease. The majority was present at atypical locations such as the lateral femoral condyle. Multivariable analysis using cox regression showed that steroid injections were a risk factor to develop an OC lesion (hazard ratio [95%CI] for number of steroid injections per year, 8.20 [3.18, 21.16]). Conclusions: Pediatric patients with JIA show a relatively high incidence of osteochondritic lesions, which present at an early age and in rather atypical locations and repetitive steroid injection need to be considered an associated risk factor

New Parents Experienced Lower Parenting Self-Efficacy during the COVID-19 Pandemic Lockdown

Background: The COVID-19 pandemic is a global issue which affects the entire population’s mental health. This study evaluates how restrictions to curtail this pandemic change parenting self-efficacy, depressive symptoms, couple satisfaction and health-related quality of life in parents after delivery of a newborn. Methods: In this prospective single center evaluation of parental self-efficacy and quality of life, four validated questionnaires were used to repeatedly assess parenting self-efficacy (Tool to measure Parental Self-Efficacy, TOPSE), depressive symptoms (Edinburgh Postnatal Depression Scale, EPDS), couple satisfaction (Couple Satisfaction Index, CSI) and health-related quality of life (short form 12, SF12). Fifty-three parents of 50 infants answered a total number of 63 questionnaires during the lockdown period to limit the spread of COVID-19. These questionnaires were matched with 63 questionnaires of 58 other parents that had answered them before or after strong pandemic related measures. Results: Parents experienced lower parenting self-efficacy during the strict pandemic measures as compared to before and after (p = 0.04). In terms of age, socioeconomic, marital status and duration of hospitalization we detected no significant difference between both groups. On univariate linear regression, TOPSE scores were associated with gestational age (p = 0.044, parameter estimate 1.67, 95% CI: 0.048 to 3.301), birth weight (p = 0.035, parameter estimate 0.008, 95% CI: 0.001 to 0.015), number of newborns’ siblings (p = 0.0554, parameter estimate 7.49, 95% CI: −0.174 to 15.145) and distance of home from hospital (p = 0.043, parameter estimate −0.38, 95% CI: −0.745 to −0.011). Interestingly, there was a positive correlation between quality of life and TOPSE scores, suggesting that those who experience a higher self-efficacy also have a higher quality of life. Conclusions: When implementing a lock-down period psychological effects such as lower experience of parental self-efficacy have to be considered

Newborns with Bloody Stools&mdash;At the Crossroad between Efficient Management of Necrotizing Enterocolitis and Antibiotic Stewardship

The onset of bloody stools in neonates often results in antibiotic treatment for suspected necrotizing enterocolitis (NEC). Food protein-induced allergic proctocolitis (FPIAP) is an often-neglected differential diagnosis. We performed a retrospective analysis of antibiotic exposure at our tertiary center from 2011 to 2020 that included three time periods of differing antimicrobial stewardship goals. We compared these data with the conventional treatment guidelines (modified Bell&rsquo;s criteria). In our cohort of 102 neonates with bloody stools, the length of antibiotic exposure was significantly reduced from a median of 4 to 2 days. The proportion of treated neonates decreased from 100% to 55% without an increase in negative outcomes. There were 434 antibiotic days. Following a management strategy according to modified Bell&rsquo;s criteria would have led to at least 780 antibiotic days. The delayed initiation of antibiotic treatment was observed in 7 of 102 cases (6.9%). No proven NEC case was missed. Mortality was 3.9%. In conclusion, with FPIAP as a differential diagnosis of NEC, an observational management strategy in neonates with bloody stools that present in a good clinical condition seems to be justified. This may lead to a significant reduction of antibiotic exposure. Further prospective, randomized trials are needed to prove the safety of this observational approach

Should Proton Pump Inhibitors be Systematically Prescribed in Patients With Esophageal Atresia After Surgical Repair?

To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI

Nutritional Aspects of Pediatric Gastrointestinal Diseases.

In the last decade, the role of nutritional management in pediatric gastrointestinal diseases has gained increasing popularity. Disease-specific diets have been introduced as conventional treatments by international guidelines. Patients tend to more willingly accept food-based therapies than drugs because of their relatively "harmless" nature. Apart from a diet's therapeutic role, nutritional support is crucial in maintaining growth and improving clinical outcomes in pediatric patients. Despite the absence of classical "side effects", however, it should be emphasized that any dietary modification might have negative consequences on children's growth and development. Hence, expert supervision is always advised, in order to support adequate nutritional requirements. Unfortunately, the media provide an inaccurate perception of the role of diet for gastrointestinal diseases, leading to misconceptions by patients or their caregivers that tends to overestimate the beneficial role of diets and underestimate the potential adverse effects. Moreover, not only patients, but also healthcare professionals, have a number of misconceptions about the nutritional benefits of diet modification on gastrointestinal diseases. The aim of this review is to highlight the role of diet in pediatric gastrointestinal diseases, to detect misconceptions and to give a practical guide for physicians on the basis of current scientific evidence

Microbial Colonization From the Fetus to Early Childhood—A Comprehensive Review

The development of the neonatal gastrointestinal tract microbiota remains a poorly understood process. The interplay between neonatal (gestational age, genetic background), maternal (mode of delivery, nutritional status) and environmental factors (antibiotic exposure, available nutrition) are thought to influence microbial colonization, however, the exact mechanisms are unclear. Derangements in this process likely contribute to various gastrointestinal diseases including necrotizing enterocolitis and inflammatory bowel disease. As such, enhanced understanding of microbiota development may hold the key to significantly reduce the burden of gastrointestinal disease in the pediatric population. The most debatable topics during microbial seeding and possible future treatment approaches will be highlighted in this review