Compliance with Gluten Free Diet Is Associated with Better Quality of Life in Celiac Disease

The quality of life (QOL) of patients with celiac disease (CD) can be altered by both symptoms of the disease and by the restrictions of the gluten-free diet (GFD). The objective was to determine the factors associated with better QOL in a large cohort of CD patients. A link to an online survey was sent to the members of the French Association of Gluten Intolerant People (AFDIAG). The French-Celiac Disease Questionnaire (F-CDQ), scoring from 0 to 100, was used to measure the QOL. Other data collected were sociodemographic characteristics, information on CD, purchasing and consumption habits of gluten-free products, and a self-assessment scale (ranging from 0 to 10) to determine the compliance with the GFD. Among the 907 CD patients who returned the questionnaire, 787 were analyzed (638 women (81%); median age: 49 years; 71% with self-assessed GFD compliance > 8). Their median F-CDQ was 73 (range: 59-82). In multivariate analysis, the main factors associated with a better quality of life were the long duration of the GFD, good compliance with the GFD, and the number of follow-up visits. Compliance with and duration of the GFD are associated with a better quality of life in patients with CD. Taking this into consideration would offset its restrictive aspect and improve its adherence

Front Pediatr

BACKGROUND: Nutritional status is a major prognostic factor for breathing and the survival of patients with cystic fibrosis (CF). Since 2012, the development of CFTR modulators has considerably transformed the outcome of this disease. Indeed, both lung function and body mass index are improved by CFTR modulators, such as Lumacaftor/Ivacaftor. However, few data exist regarding the outcome of nutritional intakes under Lumacaftor/Ivacaftor. METHODS: We conducted a prospective single-center study in children with CF treated with Lumacaftor/Ivacaftor to evaluate their nutritional intake before and after treatment. RESULTS: Thirty-four children were included in this study, with a median age of 12.4 years [11.9; 14.7]. There was no significant improvement in weight, height or BMI. Patients' total energy intake was not significantly changed with Lumacaftor/Ivacaftor, while carbohydrate intakes decreased significantly. We found that blood levels of vitamin E and Selenium were significantly increased under Lumacaftor/Ivacaftor, without a significant increase in supplementation. In patients with a BMI Z-score < 0 at treatment initiation, there was a significant improvement in weight and BMI Z-score, while TEI and carbohydrate intakes were significantly lower. CONCLUSION: We showed that treatment with Lumacaftor/Ivacaftor improved the nutritional status of patients without necessarily being associated with an increase in nutritional intake. Although these data need to be confirmed in larger cohorts, they support the hypothesis that weight gain under modulators is multifactorial, and may be related to a decrease in energy expenditure or an improvement in absorption

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

The respiratory microbiota alpha-diversity in chronic lung diseases: a systematic review and meta-analysis

International audienceImbalance in microbial composition (i.e. dysbiosis) in the gut microbiome is consensually considered an indicator of deteriorated health and has been associated to different chronic health conditions. However, there is no clear evidence how this generalizes to the other human microbiomes. Especially, researches on the relationship between respiratory microbiota imbalance and chronic lung diseases are recent whereas microbial colonization of the airways respiratory tract have characterized chronic lung diseases. Imbalance is mainly measured through the relative abundance of microbial species in space and time within a given community (i.e. alpha-diversity). Identifying a range of values in alpha-diversity when comparing exacerbated, stable patients and healthy subjects may lead to identify new biomarker in chronic respiratory diseases. In the present work, we propose a systematic review of studies investigating the lung microbiota alpha-diversity in patients with chronic respiratory diseases in which a control group based on disease status or healthy subjects is provided for comparison. We focused on the most common measures of alpha-diversity (Chao1, Shannon, and Simpson) indexes and the most common chronic diseases (asthma, chronic obstructive pulmonary disease –COPD-, cystic fibrosis –CF-, bronchiectasis, and pulmonary hypertension). Subsequently, we conducted a meta-analysis based on random-effects models using the R package metafor to characterize the difference in alpha-diversity indexes when comparing cases to controls. We also explored heterogeneity of sources and risk of bias though Factor Analysis of Mixed Data (FAMD) using the FactoMineR R package.After removing duplicate records, we screened 351 articles on title and abstract, of which 27 met our inclusion criteria for the systematic review. Finally, data from 25 studies were used in the meta-analysis. Eight studies deal with CF, 8 with COPD, 10 with asthma and 1 with bronchiectasis. All of the studies dedicated to the respiratory tract microbiota, mainly based on sputum samples analysis and, the majority of the studies used metataxonomy approaches. As highlighted by the meta-analysis, these metataxonomy methods exhibited numerous heterogeneities. Differences in alpha-diversity indexes between healthy and diseased people were observed only in some of the diseases studied. However, prudence is required in its interpretation because of substantial heterogeneity

Proton pump inhibitors are still overprescribed for hospitalized children

OBJECTIVE: The use of proton pump inhibitors has increased exponentially over the past 20 years. Several side effects have been reported and concerns exist about the consequences of long-term proton pump inhibitors on health, leading to limitation of their use. The present study analyzed prescriptions of proton pump inhibitors at inpatient units and assessed their compliance with current recommendations. METHODS: This single-center, observational, retrospective study reviewed medical file of patients hospitalized at the pediatric medical departments of the Bordeaux University Hospital between April 1 and September 30, 2019. Patients younger than 18 years, hospitalized in the pediatric hospital units and treated with proton pump inhibitors were included. Prescriptions of proton pump inhibitors were compared with French and international guidelines. RESULTS: Proton pump inhibitors were prescribed for 251 of 2237 children (11%), mainly for gastroesophageal reflux disease (47%) and prevention of peptic ulcer disease (32.7%). Proton pump inhibitor prescription complied to recommendations in 34.5% of cases, less often in children aged younger than 1 year (13.5%) than in older children. Compliance to recommendations was lower when proton pump inhibitors were indicated for the prevention of peptic disease (5%) than for gastroesophageal reflux disease (48%). CONCLUSIONS: Proton pump inhibitors are frequently prescribed for hospitalized children, and indications comply with recommendations in only 35% of the cases. Efforts in spreading awareness of the recommendations on the use of proton pump inhibitors in children are mandatory among hospital pediatricians

The respiratory microbiome alpha-diversity and chronic respiratory diseases in children: A systematic review

Background and Aims: While there seems to be a consensus that a decrease in gut microbiome diversity is related to poorer health status, the associations between respiratory microbiome diversity and chronic respiratory disease remain a matter of debate, as highlighted by a recent review of the literature in adults. In industrialized countries, respiratory diseases and allergies are the most common chronic diseases in children. These diseases vary considerably between childhood and adulthood. We performed a systematic review of the alpha diversity of the respiratory microbiome in children with chronic respiratory diseases. We focused on studies in which a control allowed comparison.Methods: We searched PubMed and Scopus. Four items were required: respiratory microbiome, diversity, chronic respiratory disease, and children younger than 18 years. Any articles that did not address these items, were not research articles, or did not have a case-control design were excluded.Results: We reviewed 486 articles on the basis of title and abstract, of which 25 met our inclusion criteria. The diseases studied were mainly asthma, cystic fibrosis, wheezing, and respiratory allergies. The main sampling technique was the nasopharyngeal swab. All studies focused solely on bacteriome and measured the Shannon index. In more than half of the studies, the control group consisted of healthy subjects.Conclusions: The majority of diseases were significantly associated with a decrease in diversity, with the exception of asthma, which, on the contrary, led to an increase. Only the studies on bronchiectasis, pulmonary hypertension and bronchitis showed no change or not concluding difference in the respiratory microbiome diversity

Pre-hospital triage of children at risk of oesophageal button battery impaction: the button battery impaction score

Button battery ingestion in children can be fatal if oesophageal perforation occurs. Such children require chest radiography in the emergency department to determine the button battery position and number. Current guidelines recommend that a button battery impacted in the oesophagus should be removed within two hours. We developed a clinical tool (the button battery impaction score) to estimate the risk of oesophageal impaction and help determine the most appropriate healthcare facility for initial assessment, either a local medical centre or a medical centre with the infrastructure for endoscopic retrieval. A multi-centre retrospective study was conducted over seven years in eight French poison centres. We included patients aged less than 12 years with radiography showing the button battery position and a symptom description before radiography. Button battery impaction scores were calculated using backward stepwise selection. A total of 1,430 patients were included, of whom 86, 461, and 375 had a button battery in their oesophagus, stomach, and post-pyloric position, respectively. No button batteries were identified by radiography in 508 patients. Sixteen of thirty-five factors independently predicted oesophageal impaction before chest radiography (P  A button battery impaction score used readily available data to predict the risk of oesophageal impaction after button battery ingestion and before chest radiography. When further validated, this rapid tool may be widely applicable in determining an appropriate facility for patient transfer to either a local medical centre or a medical centre with the infrastructure for endoscopic retrieval.</p

J Pediatr Gastroenterol Nutr

Objective: This multicentric study aimed to evaluate the quality of life (QOL) in children with Hirschsprung's disease (HD). Methods: HD patients aged from 6 to 18 years and followed-up in 2 French pediatric surgery centers were included in this study. QOL was assessed using the HAQL questionnaires according to age (6–11 and 12–18), filled by patients and their parents (proxy reports) and correlated with initial disease characteristics, nutritional status, and functional score of Krickenbeck. Results: Sixty-three patients were included. The acquisition of satisfactory voluntary bowel movements was found in only 50% of the 6 to 11 years old and 68% of the teenagers. Seventy percentage of the children and 55% of teenagers had soiling issues. The overall HAQLproxy6--11 score was 528/700; best scores were found for “fecal continence” (94/100), “social functioning” (94/100), and “urinary continence” (92/100) whereas the worst scores were for “general well-being” (64/100) and “diurnal fecal continence” (58/100). The overall HAQLproxy12--16 score was 607/700; best scores were for “urinary continence” (96/100) and “social functioning” (93/100). In a multivariate analysis, soiling was the only factor significantly associated with low QOL (P = 0.03). Conclusions: Soiling remains frequent in children operated on for HD and negatively affects their QOL. Assessment and treatment of soiling should be the priority for medical teams in the follow-up of these children

Cystic fibrosis and noninvasive liver fibrosis assessment methods in children

BACKGROUND: Noninvasive assessments of liver fibrosis are currently used to evaluate cystic fibrosis (CF)-related liver disease. However, there is scarce data regarding their repeatability and reproducibility, especially in children with CF. The present study aimed to evaluate the repeatability and reproducibility of transient elastography (TE) (FibroScan®) and point shear-wave elastography using virtual touch quantification (pSWE VTQ) in children with CF. METHODS: TE and pSWE VTQ were performed in 56 children with CF by two different operators. Analysis of repeatability and reproducibility was available in 33 patients for TE and 46 patients for pSWE VTQ. Intra- and interobserver agreement were assessed using the intraclass correlation coefficient (ICC) and their 95% confidence interval (CI), and Bland and Altman graphs. RESULTS: For TE, ICC was 0.91 (0.83-0.95) for intraobserver agreement and 0.92 (95% CI: 0.86-0.96) for interobserver agreement. For pSWE VTQ, ICC was 0.83 (0.72-0.90) for intraobserver agreement and 0.67 (0.48-0.80) for interobserver agreement. CONCLUSIONS: Both technics can be proposed in the follow-up of patients, according to their availability in CF centers. IMPACT: This study shows that TE and pSWE VTQ are reliable methods to evaluate liver fibrosis in children with CF. This study shows for the first time that TE and pSWE VTQ are both repeatable and reproducible in children with CF. These data indicate that both TE and pSWE VTQ can be proposed for the follow-up of patients with CF, according to their availability in each CF center

New Insights in Microbial Species Predicting Lung Function Decline in CF: Lessons from the MucoFong Project

International audienceSeveral predictive models have been proposed to understand the microbial risk factors associated with cystic fibrosis (CF) progression. Very few have integrated fungal airways colonisation, which is increasingly recognized as a key player regarding CF progression. To assess the association between the percent predicted forced expiratory volume in 1 s (ppFEV1) change and the fungi or bacteria identified in the sputum, 299 CF patients from the “MucoFong” project were included and followed-up with over two years. The relationship between the microorganisms identified in the sputum and ppFEV1 course of patients was longitudinally analysed. An adjusted linear mixed model analysis was performed to evaluate the effect of a transient or chronic bacterial and/or fungal colonisation at inclusion on the ppFEV1 change over a two-year period. Pseudomonas aeruginosa, Achromobacter xylosoxidans, Stenotrophomonas maltophilia, and Candida albicans were associated with a significant ppFEV1 decrease. No significant association was found with other fungal colonisations. In addition, the ppFEV1 outcome in our model was 11.26% lower in patients presenting with a transient colonisation with non-pneumoniae Streptococcus species compared to other patients. These results confirm recently published data and provide new insights into bacterial and fungal colonisation as key factors for the assessment of lung function decline in CF patients