Publications by authors named "Olivier Goulet"

165 Publications

Quality of life in long term survivors of pediatric intestinal transplantation compared with liver transplantation and home parenteral nutrition: A prospective single-center pilot study.

Pediatr Transplant 2021 May 16;25(3):e13982. Epub 2021 Feb 16.

Gastroenterology-Hepatology-Nutrition Unit, Hôpital Necker-Enfants Malades, Paris, France.

Health-related quality of life (HRQOL) after intestinal transplantation (IT) is important, as many psychological troubles have been reported in these patients on the long term. Our aim was to assess and compare HRQOL of patients after IT to patients after liver transplantation (LT) or on home parenteral nutrition (HPN) for intestinal failure. A cross-sectional study included patients and their parents between 10 and 18 years of age, on HPN for more than 2 years, or who underwent IT or LT, with a graft survival longer than 2 years. Quality of life was explored by Child Health Questionnaire. Thirteen children-parents dyads after IT, 10 after LT, and eight children on HPN completed the survey. Patients were a median age of 14 years old, a median of 10 years post-transplantation or on HPN. Patients after IT scored lower than patients after LT or on HPN in "social limitations due to behavioral difficulties" and in "behavior." They scored higher than those on HPN in "global health." Parents of children after IT scored lower than those after LT in many domains. No relevant correlation with clinical data was found. Our study showed the multi-level impact of IT on quality of life of patients and their parents. It highlights the importance of a regular psychological follow-up for patients, but also of a psychological support for families. Helping the patients to overcome the difficulties at adolescence may improve their mental health in adulthood.
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http://dx.doi.org/10.1111/petr.13982DOI Listing
May 2021

Dysregulation of the NRG1/ERBB pathway causes a developmental disorder with gastrointestinal dysmotility in humans.

J Clin Invest 2021 Mar;131(6)

Department of Genetics, La Pitié-Salpêtrière Hospital, AP-HP, Paris, France.

Hirschsprung disease (HSCR) is the most frequent developmental anomaly of the enteric nervous system, with an incidence of 1 in 5000 live births. Chronic intestinal pseudo-obstruction (CIPO) is less frequent and classified as neurogenic or myogenic. Isolated HSCR has an oligogenic inheritance with RET as the major disease-causing gene, while CIPO is genetically heterogeneous, caused by mutations in smooth muscle-specific genes. Here, we describe a series of patients with developmental disorders including gastrointestinal dysmotility, and investigate the underlying molecular bases. Trio-exome sequencing led to the identification of biallelic variants in ERBB3 and ERBB2 in 8 individuals variably associating HSCR, CIPO, peripheral neuropathy, and arthrogryposis. Thorough gut histology revealed aganglionosis, hypoganglionosis, and intestinal smooth muscle abnormalities. The cell type-specific ErbB3 and ErbB2 function was further analyzed in mouse single-cell RNA sequencing data and in a conditional ErbB3-deficient mouse model, revealing a primary role for ERBB3 in enteric progenitors. The consequences of the identified variants were evaluated using quantitative real-time PCR (RT-qPCR) on patient-derived fibroblasts or immunoblot assays on Neuro-2a cells overexpressing WT or mutant proteins, revealing either decreased expression or altered phosphorylation of the mutant receptors. Our results demonstrate that dysregulation of ERBB3 or ERBB2 leads to a broad spectrum of developmental anomalies, including intestinal dysmotility.
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http://dx.doi.org/10.1172/JCI145837DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7954599PMC
March 2021

The use of biofeedback for children with fecal incontinence secondary to retentive constipation: Experience of a French Pediatric Center.

Clin Res Hepatol Gastroenterol 2020 Oct 22:101550. Epub 2020 Oct 22.

Department of Pediatric Gastroenterology, Hepatology, and Nutrition, APHP, Hôpital Necker Enfants Malades, 149 Rue de Sèvres, 75015, Paris, France; Université de Paris, Faculté de Médecine, 2 rue de l'Ecole de Médecine, 75006, Paris, France.

Background: Fecal incontinence (FI) secondary to chronic retentive constipation is a frequent demand in pediatric gastroenterology clinics. The management of constipation in children includes laxatives (polyethylene glycol, PEG), enhanced toilet training, and dietary advice. Biofeedback is a possible treatment for children above the age of 7 years with resistant FI.

Aim: To analyze any changes in volume to trigger defecation (VTD) and envy score over the course of biofeedback sessions according to clinical response.

Methods: In this retrospective study, we reviewed the medical records of 23 children diagnosed with FI according to the Rome IV criteria and treated with biofeedback. For each biofeedback session, a mean VTD by subject was measured. At the end, therapy was considered a success if soiling disappeared and a failure if any persisted. The need to defecate expressed by the child was described as an envy score. A 0-10 visual analog scale was used to express the intensity of this sensation. Follow-up involved calling the parents 12 months after the biofeedback sessions had ended to assess symptoms remotely.

Results: The study included 19 boys and 4 girls with a median age of 10 years. Patients' ages ranged between 7 and 17 years. None of them had any associated neurological disorders. All children had FI for >1 year. The median number of soiling episodes per week was 7. The average number of biofeedback sessions was 3 (range 1-5). At the end of the rehabilitation sessions, 12 children (52%) were in the "success" group. In the latter, median VTD decreased from 97 ml to 70 ml between the first and last session. In the "failure" group, VTD decreased from 120 ml to 100 ml. The between-group difference in the median VTD at the first session was not statistically significant. The last observation carried forward (LOCF) VTD was significantly lower in the "success" group compared to the "failure" group (70 ml versus 100 ml, p = 0.03). Median envy scores decreased during the biofeedback sessions with no statistical difference between the groups at the last session. Follow-up of children in the "success" group one year after the last biofeedback session revealed that 10 patients had no relapse (83%) and 2 were lost to follow-up.

Conclusions: Biofeedback might be an effective tool for the management of FI resistant to medical treatment in children.
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http://dx.doi.org/10.1016/j.clinre.2020.09.011DOI Listing
October 2020

Metabolic bone disease in children with intestinal failure is not associated with the level of parenteral nutrition dependency.

Clin Nutr 2021 Apr 15;40(4):1974-1982. Epub 2020 Sep 15.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, National Reference Center for Rare Digestive Diseases, Necker Enfants Malades University Hospital, 75015, Paris, France; Université de Paris, Faculty of Medicine, 75006, Paris, France.

Background & Aims: Children on long-term home parenteral nutrition (HPN) are at increased risk of suboptimal growth and metabolic bone disease (MBD) i.e. decreased bone mineral density (BMD). The aims of this cross-sectional study were to assess growth and bone health in children on long term HPN and to identify risk factors for MBD.

Methods: Children above the age of 5 years, stable on HPN for more than 2 years were included. Medical files were reviewed retrospectively and included demographics, gestational age, birth weight and height, indication for PN, age at PN start, duration of PN, number of weekly PN infusions, weight-for-age and height-for-age (SD), body mass index (BMI, kg/m) as well as blood and urine analyses at the time of Dual X-ray absorptiometry (DXA) measurements. All BMD values were adjusted to statural age which corresponds to the 50th percentile of height. Growth failure (height-for-age ≤ -2SD) and MBD (at least one BMD measurement ≤ -2SD) were analyzed according to the indication of PN, duration of PN and PN dependency index (PNDI) by comparing means and performing logistic regression analysis. PNDI is the ratio of non-protein energy intake in HPN to resting energy expenditure using Schofield equations.

Results: Forty children were assessed at 12.4 ± 4.5 years of age. Mean age at PN start was 1.1 ± 3.6 y (median 0.5). The indications for PN were short bowel syndrome (SBS, n = 21), chronic intestinal pseudo-obstruction syndrome (CIPOS, n = 10) and congenital enteropathies (CE, n = 9). The mean number of PN perfusions was 6 ± 1/week. PNDI was 110 ± 30%. The mean serum level of 25-OHD3 was suboptimal at 26.5 ± 9.1 ng/mL (66.2 ± 22.8 nmol/L). The mean concentrations of calcium, phosphorus, and parathyroid hormone (PTH) were in the normal ranges. Eight children (20%) had PTH levels above normal with low 25-OHD3 levels. The mean weight-for-age and height-for-age Z-scores SDS were 0.4 ± 0.9 and -0.5 ± 1.1 respectively. The actual height was lower than genetic target height (p < 0.001). The BMD Z-scores, adjusted to the 50th percentile of height, of the spine, the left femur and the whole body were: -1.1 ± 1.7, -1.2 ± 1.5 and -1.5 ± 1.8 SDS respectively. Children with CE had significantly lower BMD values than those with SBS and CIPOS (p = 0.01). Only two children had bone fractures after a mild trauma (5%).

Conclusions: All children on long-term PN, are at risk of low BMD. High dependency on PN (PNDI>120%) and very long-term PN (>10 years) do not appear to increase the risk of growth failure nor MBD. PN-related bone fractures were rare. Close follow-up remains mandatory.
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http://dx.doi.org/10.1016/j.clnu.2020.09.014DOI Listing
April 2021

Beyond 10 years, with or without an intestinal graft: Present and future?

Am J Transplant 2020 10 3;20(10):2802-2812. Epub 2020 May 3.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Necker-Enfants Malades Hospital, University of Paris, Paris, France.

Long-term outcomes in children undergoing intestinal transplantation remain unclear. Seventy-one children underwent intestinal transplantation in our center from 1989 to 2007. We report on 10-year posttransplant outcomes with (group 1, n = 26) and without (group 2, n = 9) a functional graft. Ten-year patient and graft survival rates were 53% and 36%, respectively. Most patients were studying or working, one third having psychiatric disorders. All patients in group 1 were weaned off parenteral nutrition with mostly normal physical growth and subnormal energy absorption. Graft histology from 15 late biopsies showed minimal abnormality. However, micronutrient deficiencies and fat malabsorption were frequent; biliary complications occurred in 4 patients among the 17 who underwent liver transplantation; median renal clearance was 87 mL/min/1.73 m . Four patients in group 1 experienced late acute rejection. Among the 9 patients in group 2, 4 died after 10 years and 2 developed significant liver fibrosis. Liver transplantation and the use of a 3-drug regimen including sirolimus or mycophenolate mofetil were associated with improved graft survival. Therefore, intestinal transplantation may enable a satisfactory digestive function in the long term. The prognosis of graft removal without retransplantation is better than expected. Regular monitoring of micronutrients, early psychological assessment, and use of sirolimus are recommended.
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http://dx.doi.org/10.1111/ajt.15899DOI Listing
October 2020

Cannabinoids Improve Gastrointestinal Symptoms in a Parenteral Nutrition-Dependent Patient With Chronic Intestinal Pseudo-Obstruction.

JPEN J Parenter Enteral Nutr 2021 02 17;45(2):427-429. Epub 2020 Mar 17.

Pediatric Gastroenterology-Hepatology-Nutrition Unit, Necker-Enfants-Malades Hospital, Paris, France.

Chronic intestinal pseudo-obstruction (CIPO) is a rare and challenging cause of pediatric intestinal failure, requiring long-term parenteral nutrition in most cases. Despite optimal management, some patients experience chronic abdominal pain and recurrent obstructive episodes with a major impact on their quality of life. Cannabinoids have been successfully used in some conditions. However, their use in CIPO has never been reported in the literature. We report a case of successful use of medicinal cannabinoids in a patient with CIPO, resulting in a significant reduction of abdominal pain, vomiting, and subocclusive episodes and increased appetite and weight, without major adverse events. Although further observations are required to consolidate these findings, this case may be helpful for other patients suffering from the same condition.
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http://dx.doi.org/10.1002/jpen.1821DOI Listing
February 2021

Lipid Emulsion Use in Pediatric Patients Requiring Long-Term Parenteral Nutrition.

JPEN J Parenter Enteral Nutr 2020 02;44 Suppl 1:S55-S67

Division of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

The ability to deliver nutrients via parenteral nutrition (PN) has markedly improved the prognosis of infants and children with intestinal failure. Technical refinements and advances in knowledge have led to the development of highly sophisticated PN solutions that are tailored to meet the needs of pediatric patients. However, children who require long-term PN have an increased risk of complications such as catheter-related sepsis, liver disease, and bone disease. Although the pathogenesis of intestinal failure associated liver disease (IFALD) is multifactorial, studies have identified a possible link between the dose of lipid emulsions based on soybean oil and cholestasis, shown to occur with a significantly higher frequency in patients receiving >1 g lipids/kg/d. Potential contributing factors include oxidative stress, high ω-6 polyunsaturated fatty acid (PUFA) and phytosterol content, and relatively low α-tocopherol levels. Lipid emulsions containing fish oil offer potential advantages compared with traditional emulsions with a high soybean oil content, such as decreased ω-6 and increased ω-3 PUFA concentrations, high concentrations of α-tocopherol, and reduced phytosterol content. Studies in PN-dependent children at risk for IFALD have shown that lipid emulsions containing fish oil reduce the risk of cholestasis and improve biochemical measures of hepatobiliary function compared with pure soybean oil emulsions. This review summarizes evidence regarding the role of lipid emulsions in the management of pediatric patients with intestinal failure requiring long-term PN, with a particular focus on the prevention and treatment of IFALD.
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http://dx.doi.org/10.1002/jpen.1762DOI Listing
February 2020

Summary of Proceedings and Expert Consensus Statements From the International Summit "Lipids in Parenteral Nutrition".

JPEN J Parenter Enteral Nutr 2020 02;44 Suppl 1:S7-S20

Department of General and Oncology Surgery, Intestinal Failure Unit, Stanley Dudrick's Memorial Hospital, Skawina, Poland.

Background: The 2018 Lipids in Parenteral Nutrition summit involved a panel of experts in clinical nutrition, lipid metabolism, and pharmacology, to assess the current state of knowledge and develop expert consensus statements regarding the use of intravenous lipid emulsions in various patient populations and clinical settings. The main purpose of the consensus statements is to assist healthcare professionals by providing practical guidance on common clinical questions related to the provision of lipid emulsions as part of parenteral nutrition (PN).

Methods: The summit was designed to allow interactive discussion and consensus development. The resulting consensus statements represent the collective opinion of the members of the expert panel, which was informed and supported by scientific evidence and clinical experience.

Results: The current article summarizes the key discussion topics from the summit and provides a set of consensus statements designed to complement existing evidence-based guidelines. Lipid emulsions are a major component of PN, serving as a condensed source of energy and essential fatty acids. In addition, lipids modulate a variety of biologic functions, including inflammatory and immune responses, coagulation, and cell signaling. A growing body of evidence suggests that lipid emulsions containing ω-3 fatty acids from fish oil confer important clinical benefits via suppression of inflammatory mediators and activation of pathways involved in the resolution of inflammation.

Conclusions: This article provides a set of expert consensus statements to complement formal PN guideline recommendations.
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http://dx.doi.org/10.1002/jpen.1746DOI Listing
February 2020

Colon importance in short bowel syndrome.

Aging (Albany NY) 2019 11 15;11(22):9961-9962. Epub 2019 Nov 15.

Pediatric Hepatology Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, Bergamo, Italy.

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http://dx.doi.org/10.18632/aging.102447DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6914415PMC
November 2019

Introduction to the Sixth Global Summit on the Health Effects of Yogurt: Yogurt, More than the Sum of Its Parts.

Adv Nutr 2019 09;10(5):913S-916S

Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Disease, Hôpital Necker-Enfants Malades, University of Paris Descartes, Paris, France.

Foods are not only a collection of individual components but are complex matrices. The food matrix is defined by the USDA as "the nutrient and nonnutrient components of foods and their molecular relations." The matrix of a food is an important factor in evaluating its nutritional and health contributions to the consumer. Dairy foods are a complex mix of various nutrients and other components, which together form the food matrix. There are three main types of dairy food matrices: liquid (milk, some fermented milks), semi-solid (yogurt, some fresh cheeses), and solid (most cheeses). The nutritional value of dairy foods is determined by their nutrient composition and matrix structure, which can affect digestibility and the bioavailability of nutrients. Additionally, a number of studies have shown that the health effects of dairy products, of similar nutrient content, vary by their matrix.
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http://dx.doi.org/10.1093/advances/nmz017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6743843PMC
September 2019

Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management.

Pediatr Gastroenterol Hepatol Nutr 2019 Jul 27;22(4):303-329. Epub 2019 Jun 27.

Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Sorbonne-Paris-Cité Paris Descartes Medical School, Paris, France.

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.
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http://dx.doi.org/10.5223/pghn.2019.22.4.303DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629594PMC
July 2019

Clostridium difficile: A Frequent Infection in Children After Intestinal Transplantation.

Transplantation 2020 01;104(1):197-200

Hôpital Necker-Enfants Malades Assistance Publique-Hôpitaux de Paris, Department of Pediatric Gastroenterology, Hepatology and Nutrition, Université Paris Descartes - Sorbonne Paris Cité, Paris, France.

Background: Organ transplantation (Tx) is a risk factor for Clostridium difficile infection (CDI). After intestinal transplantation (ITx), few data are available on the impact of this graft infection and the possible induction of rejection.

Methods: We included retrospectively all children after ITx in our unit, with at least 1 year of graft survival. All samples positive for Clostridium difficile (CD) and its toxin were considered.

Results: Among the 57 ITx recipients (60 Txs), 22 children (39%) developed culture-proven CDI, 12 after isolated small bowel Tx, 9 after liver-small bowel Tx, and 1 after multivisceral Tx. Twenty patients had diarrhea, 8 bloody stools, 4 fever, and 1 hypothermia. Nine were hospitalized for an average of 6.5 days (2-20) and 4 with severe dehydration. Nine (40%) had received antibiotics for an average of 19 days (7-60) before CDI. Two patients were asymptomatic. CDI was treated with metronidazole in 12 children, vancomycin in 6, and both in 3. Three children presented mild-to-severe rejections. Two patients presented concomitantly CDI and rejection. The third patient presented a rejection with severe complications 4 years after CDI. Recurrence of toxinogenic CD was observed in 9 children, in 7 associated with clinical symptoms. During the last follow-up, the stool number was the same as before CDI except for 1 patient with ongoing infection.

Conclusions: CDI is more prevalent in children after ITx compared with other organ Tx; it is most often symptomatic but mildly or moderately severe. Standard antibiotics efficiently control the symptoms. Induction of rejection is a rare event.
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http://dx.doi.org/10.1097/TP.0000000000002795DOI Listing
January 2020

Paediatricians play a key role in preventing early harmful events that could permanently influence the development of the gut microbiota in childhood.

Acta Paediatr 2019 11 8;108(11):1942-1954. Epub 2019 Jul 8.

Pediatric Department, Vittore Buzzi Children's Hospital, Università degli Studi di Milano, Milan, Italy.

Aim: The development of the gut microbiota occurs primarily during infancy, and growing evidence has emphasised its positive role and implications for human health. The aim of this review was to provide essential knowledge about the gut microbiota and to describe and highlight the importance of the factors that influence the gut microbiota in early life and their potential harmful effects later in life.

Methods: The European Paediatric Association, the Union of the National European Paediatric Societies and Associations, convened a panel of independent European experts to summarise the research on microbiota for general paediatricians. They used PubMed and the Cochrane Library to identify studies published in English up to June 2018.

Results: A number of clinical conditions can disrupt the development of a stable gut microbiota. Changes in the microbiome have been documented in many chronic diseases, mainly immune-mediated gastrointestinal and liver diseases, and distinct patterns have been associated with each specific disease. The gut microbiota can be positively modulated with probiotics, prebiotics, synbiotics, paraprobiotics and postbiotics.

Conclusion: Paediatricians can play a key role in preventing harmful events that could permanently influence the composition and/or function of the gut microbiota. Various treatment strategies can be used.
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http://dx.doi.org/10.1111/apa.14900DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6852013PMC
November 2019

The colon as an energy salvage organ for children with short bowel syndrome.

Am J Clin Nutr 2019 04;109(4):1112-1118

Department of Pediatric Gastroenterology-Hepatology-Nutrition, Hôpital Necker-Enfants Malades, Université Paris Descartes, Paris, France.

Background: The main cause of intestinal failure is short bowel syndrome (SBS). The management goal for children with SBS is to promote intestinal adaptation while preserving growth and development with the use of parenteral nutrition (PN).

Objectives: This study evaluated the intestinal absorption rate in children with SBS, focusing on the role of the remnant colon. In addition, the relation between intestinal absorption rate, citrulline concentration, and small bowel length was studied.

Methods: Thirty-two children with SBS on PN were included. They were divided into 3 groups according to the European Society for Clinical Nutrition and Metabolism (ESPEN) anatomical classification system: type 1 SBS (n = 9), type 2 (n = 13), and type 3 (n = 10). Intestinal absorption rate was assessed by a stool balance analysis of a 3-d collection of stools. Plasma citrulline concentrations were measured and the level of PN dependency was calculated.

Results: The total energy absorption rate did not differ significantly between the 3 groups: 68% (61-79% ) for type 1, 60% (40-77%) for type 2, and 60% (40-77%) for type 3 ( P = 0.45). Children with type 2 or 3 SBS had significantly shorter small bowel length than children with type 1: 28 cm (19-36 cm) and 16 cm (2-29 cm), respectively, compared with 60 cm (45-78 cm) ( P = 0.04). Plasma citrulline concentrations were lower in type 3 SBS but not significantly different: 15 µmol/L (11-25 µmol/L) in type 1, 14 µmol/L (7-21 µmol/L) in type 2 , and 9 µmol/L (6-14 µmol/L) in type 3 ( P = 0.141). A multivariate analysis confirmed the role of the remnant colon in providing additional energy absorption.

Conclusion: This study demonstrated the importance of the colon as a salvage organ in children with SBS. Plasma citrulline concentrations should be interpreted according to the type of SBS. Efforts should focus on conservative surgery, early re-establishment of a colon in continuity, and preserving the intestinal microbiota.
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http://dx.doi.org/10.1093/ajcn/nqy367DOI Listing
April 2019

Nurturing Children's Healthy Eating: Position statement.

Appetite 2019 06 21;137:124-133. Epub 2019 Feb 21.

Developmental Psychologist, US Department of Agriculture/Agricultural Research Service Children's Nutrition Research Center, Baylor College of Medicine, Houston, TX, USA. Electronic address:

The relationship between eating a healthy diet and positive health outcomes is well known; nurturing healthy eating among children therefore has the potential to improve public health. A healthy diet occurs when one's usual eating patterns include adequate nutrient intake and sufficient, but not excessive, energy intake to meet the energy needs of the individual. However, many parents struggle to establish healthy eating patterns in their children due to the pressures of modern life. Moreover, healthcare providers often do not have the time or the guidance they need to empower parents to establish healthy eating practices in their children. Based on existing evidence from epidemiologic and intervention research, the Nurturing Children's Healthy Eating collaboration, established by Danone Institute International, has identified four key themes that encourage and support healthy eating practices among children in the modern Western world. The first - positive parental feeding - explores how parenting practices and styles, such as avoiding food restriction, allowing children to make their own food choices, and encouraging children to self-limit their portion sizes, can influence children's dietary intake. The second - eating together - highlights the link between eating socialization through regular family meals and healthful diet among children. The third - a healthy home food environment - explores the impact on eating practices of family resources, food availability/accessibility, parental modeling, and cues for eating. The fourth - the pleasure of eating - associates children's healthy eating with pleasure through repeated exposure to healthful foods, enjoyable social meals, and enhancement of the cognitive qualities (e.g. thoughts or ideas) of healthful foods. This paper reviews the evidence leading to the characterization of these nurturing themes, and ways in which recommendations might be implemented in the home.
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http://dx.doi.org/10.1016/j.appet.2019.02.007DOI Listing
June 2019

Efficacy of Ruxolitinib Therapy in a Patient With Severe Enterocolitis Associated With a STAT3 Gain-of-Function Mutation.

Gastroenterology 2019 03 15;156(4):1206-1210.e1. Epub 2018 Dec 15.

Inserm UMR1163, Intestinal Immunity, Institut Imagine, Paris, France; Université Paris Descartes Sorbonne Paris Cité, Paris, France; AP-HP, Gastroenterology, Hôpital Cochin, Paris, France. Electronic address:

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http://dx.doi.org/10.1053/j.gastro.2018.11.065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433619PMC
March 2019

Mucosal Healing and Bacterial Composition in Response to Enteral Nutrition Vs Steroid-based Induction Therapy-A Randomised Prospective Clinical Trial in Children With Crohn's Disease.

J Crohns Colitis 2019 Jul;13(7):846-855

Hôpital Necker Enfants Malades Université Sorbonne Paris Cité, Paris, APHP.

Aims: Exclusive enteral nutrition [EEN] is as efficacious as corticosteroids [CS] to induce remission in Crohn's disease [CD], without their adverse effects. EEN seems to be more efficient than steroids to induce mucosal healing, but the underlying molecular mechanisms are only sparsely understood. We aimed in the present work to study the anti-inflammatory effects of EEN with Modulen IBD® vs CS in active paediatric CD, and to assess its modulatory effects on the intestinal microbiota as compared with steroids.

Materials And Methods: Nineteen patients with new-onset active CD (Harvey-Bradshaw index [HBI] >5), aged from 6 to 17 years, were included in this prospective randomised induction trial with CS [n = 6] or EEN [n = 13]. Patients were assessed at Weeks 0 and 8 using clinical parameters HBI, endoscopic findings (Crohn's Disease Endoscopic Index of Severity [CDEIS] score) and analysis of faecal microbiota composition.

Results: At 8 weeks, clinical remission [HBI <5] was achieved in 13/13 patients on EEN and 5/6 patients on steroids; the mucosal healing rate was significantly higher in the EEN [89%] compared with steroid group [17%]. There were no significant differences between groups regarding biological markers, but the intestinal microbiota profiles shifted upon EEN-induced remission to a higher proportion of Ruminococcus bacteria compared with steroid-induced remission [p = 0.049], and with higher proportions of bacteria belonging to Clostridium in EEN-treated patients.

Conclusions: Both steroid and EEN induced clinical remission. However, patients with EEN-induced remission showed a higher rate of mucosal healing and this was associated with a different gut microbiota compositional shift in these children.
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http://dx.doi.org/10.1093/ecco-jcc/jjy207DOI Listing
July 2019

Intestinal dysbiosis in inflammatory bowel disease associated with primary immunodeficiency.

J Allergy Clin Immunol 2019 02 10;143(2):775-778.e6. Epub 2018 Oct 10.

French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker-Enfants Malades University Hospital, APHP, Paris, France; Paediatric Haematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, APHP, Paris, France; INSERM UMR 1163, Imagine Institute, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; Collège de France, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2018.09.021DOI Listing
February 2019

ESPGHAN/ESPEN/ESPR/CSPEN guidelines on pediatric parenteral nutrition: Lipids.

Clin Nutr 2018 12 18;37(6 Pt B):2324-2336. Epub 2018 Jun 18.

Dr von Haunersches Kinderspital, Div. Metabolic and Nutritional Medicine, Ludwig Maximilian Universität, University of Munich Medical Center, Munich, Germany.

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http://dx.doi.org/10.1016/j.clnu.2018.06.946DOI Listing
December 2018

Algorithms to Define Abnormal Growth in Children: External Validation and Head-To-Head Comparison.

J Clin Endocrinol Metab 2019 02;104(2):241-249

INSERM, UMR1153 Epidemiology and Biostatistics Sorbonne Paris Cité Center, Early Origins of the Child's Health and Development Team, Paris Descartes University, Villejuif, France.

Background: Growth monitoring of apparently healthy children aims at early detection of serious conditions by use of both clinical expertise and algorithms that define abnormal growth. The seven existing algorithms provide contradictory definitions of growth abnormality and have a low level of validation.

Objective: An external validation study with head-to-head comparison of the seven algorithms combined with study of the impact of use of the World Health Organization (WHO) vs national growth charts on algorithm performance.

Design: With a case-referent approach, we retrospectively applied all algorithms to growth data for children with Turner syndrome, GH deficiency, or celiac disease (n = 341) as well as apparently healthy children (n = 3406). Sensitivity, specificity, and theoretical reduction in time to diagnosis for each algorithm were calculated for each condition by using the WHO or national growth charts.

Results: Among the two algorithms with high specificity (>98%), the Grote clinical decision rule had higher sensitivity than the Coventry consensus (4.6% to 54% vs 0% to 8.9%, P < 0.05) and offered better theoretical reduction in time to diagnosis (median: 0.0 to 0.9 years vs 0 years, P < 0.05). Sensitivity values were significantly higher with the WHO than national growth charts at the expense of specificity.

Conclusion: The Grote clinical decision rule had the best performance for early detection of the three studied diseases, but its limited potential for reducing time to diagnosis suggests the need for better-performing algorithms based on appropriate growth charts.
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http://dx.doi.org/10.1210/jc.2018-00723DOI Listing
February 2019

More research is needed on the use of probiotics for critically ill patients.

Acta Paediatr 2019 01 8;108(1):181. Epub 2018 Sep 8.

Children's Hospital Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia.

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http://dx.doi.org/10.1111/apa.14547DOI Listing
January 2019

Combined Immunodeficiency in Patients With Trichohepatoenteric Syndrome.

Front Immunol 2018 11;9:1036. Epub 2018 May 11.

APHM, Hôpital de la Timone Enfant, Service de Pédiatrie Multidisciplinaire, Marseille, France.

The syndromic diarrhea/trichohepatoenteric syndrome (SD/THE) is a rare and multi-system genetic disorder caused by mutation in or in , two genes encoding subunits of the putative human SKI complex involved in RNA degradation. The main features are intractable diarrhea of infancy, hair abnormalities, facial dysmorphism, and intrauterine growth restriction. Immunologically this syndrome is associated with a hypogammaglobulinemia leading to an immunoglobulin supplementation. Our immune evaluation of a large French cohort of SD/THE patient revealed several immunological defects. First, switched memory B lymphocytes count is very low. Second, IFN-γ production by T and NK cells is impaired and associated with a reduced degranulation of NK cells. Third, T cell proliferation was abnormal in 3/6 -mutated patients. These three patients present with severe EBV infection and a transient hemophagocytosis which may be related to these immunological defects. Moreover, an immunological screening of patients with clinical features of SD/THE could facilitate both diagnosis and therapeutic management of these patients.
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http://dx.doi.org/10.3389/fimmu.2018.01036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5958188PMC
July 2019

Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study.

J Crohns Colitis 2018 Aug;12(9):1104-1112

GENIUS Group [GENetically ImmUne-mediated enteropathieS] from the European Society for Paediatric Gastroenterology, Hepatology and Nutrition [ESPGHAN].

Background And Aims: An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases [VEO-IBD]. The present study aimed at defining how next-generation sequencing [NGS] methods can be used to improve identification of known molecular diagnosis and to adapt treatment.

Methods: A total of 207 children were recruited in 45 paediatric centres through an international collaborative network [ESPGHAN GENIUS working group] with a clinical presentation of severe VEO-IBD [n = 185] or an anamnesis suggestive of a monogenic disorder [n = 22]. Patients were divided at inclusion into three phenotypic subsets: predominantly small bowel inflammation, colitis with perianal lesions, and colitis only. Methods to obtain molecular diagnosis included functional tests followed by specific Sanger sequencing, custom-made targeted NGS, and in selected cases whole exome sequencing [WES] of parents-child trios. Genetic findings were validated clinically and/or functionally.

Results: Molecular diagnosis was achieved in 66/207 children [32%]: 61% with small bowel inflammation, 39% with colitis and perianal lesions, and 18% with colitis only. Targeted NGS pinpointed gene mutations causative of atypical presentations, and identified large exonic copy number variations previously missed by WES.

Conclusions: Our results lead us to propose an optimised diagnostic strategy to identify known monogenic causes of severe IBD.
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http://dx.doi.org/10.1093/ecco-jcc/jjy068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6113703PMC
August 2018