Infantile and very early onset-inflammatory bowel disease: a multicenter study

Objective: In this study, we described disease characteristics and assessed long-term outcomes, in patients diagnosed with very-early-onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age), and infantile-IBD (before 2 years). Methods: Cases from 21 centers worldwide diagnosed with VEOIBD (2008-2018), with minimum two years of follow-up, were retrospectively reviewed. Results: The total cohort included 243 patients (52% males, median follow-up of 5.8[IQR 3.2- 8.4] years, including 69[28%] with infantile-IBD. IBD subtypes included Crohn’s disease (CD), ulcerative colitis (UC) or IBD-unclassified (IBDU) in 30%, 59% and 11%, respectively. Among patients with CD - 94% had colonic involvement, and among patients with UC/IBDU – 75% had pancolitis. Compared to non-infantile VEOIBD, patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels and higher C-reactive protein, and had lower response rates to first induction therapy and to corticosteroids therapy (p<0.05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention and higher rates of major infections (p<0.05 for all). Conclusion: Patients with VEOIBD, including infantile-IBD, exhibit low rate of disease complications and surgical interventions at the long-term. Patients with monogenic-IBD are at risk of more severe disease course

Chronic Intestinal Failure in Children : An International Multicenter Cross-Sectional Survey

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1-4 and 14-18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.Peer reviewe

Children with Intestinal Failure Maintain Their Renal Function on Long-Term Parenteral Nutrition

Background: Long-term parenteral nutrition (PN) has been associated with renal complications, including hypercalciuria, nephrocalcinosis, proteinuria and reduced glomerular filtration rate (GFR). Pediatric data are scarce and mostly short-term. Our study aimed to evaluate renal complications in children with intestinal failure (IF) receiving long-term PN. Methods: A cross-sectional study was performed in a tertiary pediatric IF clinic of patients receiving home-PN treatment for more than 1 year. Data regarding medical background, anthropometrics, laboratory investigations and abdominal sonography were retrieved. Results: Complete data were available for 15 children (67% males), with a median age of 6 (range 1.5–15) years and a median (IQR) PN duration of 4 (1.5–6) years. Low-grade proteinuria was identified in 61% and microalbuminuria in 30% of the cohort. Hypercalciuria and hyperoxaluria were present in 50% and 46%, respectively. One patient had nephrocalcinosis. The estimated GFR was normal in all but one patient who had pre-existing kidney disease. Conclusions: Pediatric IF patients can present with preserved kidney function after years of PN treatment. Despite the high prevalence of hypercalciuria, nephrocalcinosis was not common. Base line and long-term monitoring of various aspects of renal function would be essential to characterize the effects of prolonged PN on kidney functions in pediatric patients

Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1–4 and 14–18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children

COVID-19 infection in patients on long-term home parenteral nutrition for chronic intestinal failure

International audienceRationale: To investigate the incidence and the severity of COVID-19 infection in patients on long-term home parenteral nutrition (HPN) for chronic intestinal failure (CIF).Methods: Period of observation, from March 1st 2020 to March 1st 2021. Inclusion criteria: patients included in the database since 2015 and still on HPN on March 1st 2020; patients included in the database during the period of observation. Data recorded on March 1st 2021: 1) occurrence of infection since the beginning of pandemic (yes, no, unknown); 2) infection syndrome (asymptomatic, mild-no hospitalization, moderate-hospitalization no-ICU, severe-hospitalization in ICU); 3) vaccination (yes, no, unknown); 4) patient outcome at March 1st 2021: still on HPN, weaned form HPN, deceased, lost to follow up. Statistics by Pearson Chi-Square.Results: 68 centres from 23 countries included 4680 patients; COVID-19 data were available for 55.1% of patients. The cumulative incidence of infection was 9.7% in the total group and ranged from 0% to 21.9% among countries. Infection syndrome was asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.2%. Vaccination status was unknown 62.0%, non-vaccinated 25.2%, vaccinated 12.8%. Infection rate was lower in pediatric patients (p=0.03) and in those with cancer (p=0.03). In the group of the deceased patients, a higher incidence of infection (p=0.04), a more severe degree of infection syndrome (p<0.001) and a lower percentage of vaccination (p=0.01) were observed.Conclusion: In patients with CIF, the incidence of COVID-19 infection differed greatly among countries and was asymptomatic or with mild symptoms in most cases. COVID infection, severity of infection and vaccination status were associated with a higher risk of death