Publications by authors named "Cecile Lambe"

20 Publications

  • Page 1 of 1

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 Feb 16: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
February 2021

Antenatal finding of 16q24.1 duplication including FOXF1, revealing an autosomal dominant familial pathology with congenital short bowel, malrotation and renal abnormalities.

Clin Res Hepatol Gastroenterol 2020 Nov 15:101562. Epub 2020 Nov 15.

CHU Rennes, Service de Cytogénétique et Biologie Cellulaire, F-35033 Rennes, France; Univ Rennes, CHU Rennes, INSERM, EHESP, IRSET (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, F-35000 Rennes, France.

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http://dx.doi.org/10.1016/j.clinre.2020.10.007DOI Listing
November 2020

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

Clin Nutr 2020 Sep 15. 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
September 2020

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 Feb 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

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

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

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

Experience of Using a Semielemental Formula for Home Enteral Nutrition in Children: A Multicenter Cross-sectional Study.

J Pediatr Gastroenterol Nutr 2019 04;68(4):585-590

Department of Pediatric Gastroenterology, Hepatology and Nutrition, CHU Lille, University Lille, Lille, France.

Objectives: The use of semielemental diets concerns a small proportion of children on enteral nutrition whose characteristics have never been reported. Our aim was to describe a cohort of patients on home enteral nutrition with Peptamen Junior, including the tolerance and nutritional efficacy of this product.

Methods: We performed a retrospective multicenter survey on a cohort of patients receiving this semielemental diet at home between 2010 and 2015 in 14 tertiary pediatric French centers. We recorded at baseline, 3, 6, and 12 months, and then every year the anthropometric characteristics of the patients, indications and modalities of administration of the diet, and the tolerance and adverse events.

Results: We recruited 136 patients ages 9.8 ± 4.4 years at baseline. Mean body mass index z score was -1.0 ± 1.8; mean height z score was -1.1 ± 1.9. The main underlying diseases were digestive (35.3%), neurological (33.1%), and hematological (19.9%). The indications for a semielemental diet were failure of another diet in 70 patients (51.9%), severe malnutrition in 19 (14.1%), cystic fibrosis in 11 (8.1%), and switch from parenteral nutrition in 11 (8.1%). Side effects were observed in 39.2% of the patients, and required medical attention in 8.2%. Body mass index improved or remained normal in 88.3% of children.

Conclusions: This semielemental diet seems to be well tolerated and efficient in the setting of home enteral nutrition in children with complex diseases featuring malabsorption and/or after failure of polymeric diet.
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http://dx.doi.org/10.1097/MPG.0000000000002236DOI Listing
April 2019

Salvage Strategy for Long-Term Central Venous Catheter-Associated Infections in Children.

Front Pediatr 2018 25;6:427. Epub 2019 Jan 25.

Department of General Pediatrics and Infectious Diseases, Necker-Enfants Malades University Hospital, AP-HP, Paris Descartes University, Sorbonne Paris Cité, Paris, France.

Current international guidelines strongly recommend catheter removal in case of central line-associated bloodstream infection (CLASBI), but a catheter salvage strategy may be considered in children given age-related specificities. No data is available regarding the outcome of this strategy in children. This study aims to evaluate catheter salvage strategy in children with CLABSI, and to determine treatment failure rates and associated risk factors. We retrospectively analyzed data for all children <18 years having CLABSI on a long-term central venous catheter in a tertiary hospital from 2010 to 2014. We defined catheter salvage strategy as a central venous catheter left in place ≥3 days after initiation of empiric treatment for suspected bacteremia, and catheter salvage strategy failure as the persistence or relapse of bacteremia with a strain harboring the same antibiotic susceptibility pattern, or the occurrence or the worsening of local or systemic infectious complication between 72 h and 28 days after the first positive blood culture. During the study period, 49 cases of CLABSI on long-term central venous catheters were observed in 41 children (including 59% with long-term parenteral nutrition) and 6 (15%) isolates were resistant to methicillin. A catheter salvage strategy was chosen in 37/49 (76%) cases and failed in 12/37 (32%) cases. Initial presence of bloodstream co-infection, serum concentration of vancomycin under the targeted value and inadequate empiric treatment were significantly associated with catheter salvage therapy failure. The catheter salvage strategy of CLABSI on a long-term central venous catheter was frequent in the studied hospital and failed only in one third of cases.
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http://dx.doi.org/10.3389/fped.2018.00427DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355702PMC
January 2019

Long term outcomes of intestinal rehabilitation in children with neonatal very short bowel syndrome: Parenteral nutrition or intestinal transplantation.

Clin Nutr 2019 04 15;38(2):926-933. Epub 2018 Feb 15.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France; Faculté de Médecine, Universitè of Sorbonne-Paris-Cité, Paris Descartes, 15 Rue de l'École de Médecine, 75006 Paris, France.

Background & Aims: Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments.

Patients And Methods: This retrospective cross-sectional study enrolled 36 children with very SBS (<40 cm) who entered our intestinal rehabilitation program from 1987 to 2007.

Results: All the children on long-term PN (n = 16) survived with a follow-up of 17 years (9-20). Six of them were eventually weaned off PN. Twenty children underwent ITx: eight children died (40%) 29 months (0-127) after Tx. The others 12 patients were weaned off PN 73 days (13-330) after Tx. Follow-up after transplantation was 14 years (6-28). Seven out of 8 (88%) patients with a history of gastroschisis required ITx. Patients who required ITx had longer stoma duration.

Conclusion: Survival rate of children with very short bowel was excellent if no life-threatening complications requiring transplantation developed. Gastroschisis and delayed ostomy closure are confirmed as risk factor for nutritional failure. Intestinal rehabilitation may allow a total weaning of PN before adulthood. A follow-up by a multidisciplinary team is necessary to avoid PN complications in order to minimize indications for ITx.
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http://dx.doi.org/10.1016/j.clnu.2018.02.004DOI Listing
April 2019

Strategies to Reduce Catheter-Related Bloodstream Infections in Pediatric Patients Receiving Home Parenteral Nutrition: The Efficacy of Taurolidine-Citrate Prophylactic-Locking.

JPEN J Parenter Enteral Nutr 2018 Aug 31;42(6):1017-1025. Epub 2018 Jan 31.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Rehabilitation Center for Intestinal Failure and Home Parenteral Nutrition, Necker-Enfants Malades Hospital, Paris, France.

Background: Catheter-related bloodstream infections (CRBSIs) remain a major issue in patients who are receiving home parenteral nutrition (HPN). The aim of this interventional study was to assess the impact of a new strategy using taurolidine-citrate (T-C) prophylactic locks on the CRBSI rate in children with intestinal failure who are receiving HPN.

Methods: The rate of CRBSIs was monitored every calendar year in a prospective cohort of 195 children with intestinal failure. T-C locks were initiated from October 2011 in children with recurring CRBSIs (≥2 episodes per year).

Results: In the whole cohort, the median annual CRBSI rate per 1000 catheter days decreased significantly from 2.07 in 2008 to 2010 to 1.23 in 2012 to 2014 (P < .05). T-C locks were used in 40 patients. No adverse events were reported. In taurolidine-treated patients, the CRBSI rate per 1000 catheter days decreased from 4.16 to 0.25 (P < .0001). The cumulative percentage of patients free of CRBSI at 18 months was 92% (95% confidence interval [CI]: 71-98) on T-C lock vs 61% (95% CI: 49-72) in controls (P = .01). In multivariate analysis, factors associated with CRBSI were immune deficiency (adjusted hazard ratio 3.49; 95% CI: 1.01-12.17) and the young age of the parents (adjusted hazard ratio 4.79, 95% CI: 2.16-10.62), whereas T-C locks were protective (adjusted hazard ratio 0.22, 95% CI: 0.06-0.74).

Conclusion: This study confirms the efficacy of T-C catheter locks in decreasing the incidence of CRBSIs in children with intestinal failure who are receiving HPN.
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http://dx.doi.org/10.1002/jpen.1043DOI Listing
August 2018

A New Concept to Achieve Optimal Weight Gain in Malnourished Infants on Total Parenteral Nutrition.

JPEN J Parenter Enteral Nutr 2018 01 15;42(1):78-86. Epub 2017 Dec 15.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, National Reference Center for Rare Digestive Diseases in Children, APHP, Necker Enfants Malades University Hospital, Paris, France.

Background: Infants with intestinal failure (IF) are at increased risk of malnutrition and require adapted nutrition support. Optimal weight gain during nutrition rehabilitation should occur at the velocity of statural age (adjusted to the 50 percentile of height) and not chronological age. The aim of this study was to assess the relationship between weight gain during catch-up growth, nonprotein energy intake (NPEI) provided by total parenteral nutrition (TPN), and resting energy expenditure (REE) in children with severe malnutrition due to IF.

Materials And Methods: This retrospective study included all infants with severe malnutrition treated with TPN for IF between January 1, 2010, and December 31, 2013. They all had no or minimal oral intake (<10% REE). The REE was calculated using the Schofield equations.

Results: Seventeen children were included (11 boys) with a mean age at TPN onset of 5 mo. They were followed for a mean duration of 39 days. On admission, body weight and height were -3.1 ± 0.9 and -3.3 ± 1.3 SD, respectively. The indications for TPN were short bowel syndrome (n = 10), congenital enteropathy (microvillous inclusion disease, n = 6) and chronic intestinal pseudo-obstruction syndrome (n = 1). After 28 days of nutrition rehabilitation with full NPEI from TPN, the observed weight gain was 110 ± 5% of optimal weight gain for statural age. The mean NPEI from TPN was 104.3 ± 8.0 kcal/kg/d. The mean ratio of NPEI over REE was 2.1 ± 0.2.

Conclusion: Optimal weight gain was achieved with NPEI from TPN twice the REE in severely malnourished infants with IF. NPEI values were adequate and not excessive for age.
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http://dx.doi.org/10.1002/jpen.1014DOI Listing
January 2018

Intravenous lipid emulsions in pediatric patients with intestinal failure.

Curr Opin Organ Transplant 2017 Apr;22(2):142-148

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, National Reference Centre for Rare Digestive Diseases, APHP Necker-Enfants Malades Hospital, Paris-Descartes University, Paris, France.

The incidence of cholestatic liver disease (CLD) in pediatric patients suffering intestinal failure (IF) is not well established. Due to persistent portal inflammation, about 20% of these patients will progress to end-stage intestinal failure associated liver disease (IFALD) leading to liver transplant or death.

Purpose Of Review: Premature babies as well as infants with short bowel syndrome (SBS) and repeated sepsis (catheter or small intestinal bacterial overgrowth related) are at risk of developing CLD. Clinical data in SBS infants focused on intravenous lipid emulsion (ILE) as an important factor of CLD.

Recent Findings: Compared to the last generation of composite ILE containing fish oil (FO), soybean oil (SO) based ILE, have marked differences in term of oil source, omega-3 fatty acids (FAs) composition, vitamin E (α-tocopherols) and plant sterols contents, that may explain CLD and CLD reversal. Randomized controlled trials and meta-analysis allow the following recommendations.

Summary: In pediatric patients with developing or established CLD or IFALD, potential causes should be explored and pure SO ILE should be avoided. A reduction of the ILE dosage and/or the use of the new composite FO based ILE, may be recommended along with the treatment and management of other risk factors. The 10% pure FO ILE should not be used as a sole provision of IV lipids in paediatric patients on total PN but can only serve as a short-term rescue treatment.
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http://dx.doi.org/10.1097/MOT.0000000000000396DOI Listing
April 2017

Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center.

Am J Clin Nutr 2016 05 30;103(5):1327-36. Epub 2016 Mar 30.

Departments of Pediatric Gastroenterology, Hepatology and Nutrition, Paris Descartes University, Paris, France; and.

Background: Parenteral nutrition (PN) is the main treatment for intestinal failure.

Objective: We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center.

Design: We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs). We compared our results to a previous study that was performed in our unit from 1980 to 2000 and included 302 patients.

Results: A total of 251 patients were included: 217 (86%) had a PDD. The mean ± SD age at HPN onset was 0.7 ± 0.3 y, with a mean duration of 1.9 ± 0.4 y. The indications for HPN were short bowel syndrome (SBS) (59%), PNDD (14%), congenital enteropathies (10%), chronic intestinal pseudo-obstruction syndromes (9%), inflammatory bowel diseases (5%), and other digestive diseases (3%). By 31 December 2013, 52% of children were weaned off of HPN, 9% of the PDD subgroup had intestinal transplantation, and 10% died mostly because of immune deficiency. The major complications of HPN were catheter-related bloodstream infections (CRBSIs) (1.7/1000 d of PN) and intestinal failure-associated liver disease (IFALD) (51 children; 20% of cohort). An increased rate of CRBSIs was observed compared with our previous study, but we saw a decreasing trend since 2012. No noteworthy deceleration of growth was observed in SBS children 6 mo after weaning off HPN.

Conclusions: SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.
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http://dx.doi.org/10.3945/ajcn.115.121756DOI Listing
May 2016

[NUTRITIONAL STATUS ASSESSMENT IN PATIENTS WITH CYSTIC FIBROSIS].

Rev Prat 2015 Oct;65(8):1100-3

Prognosis of cystic fibrosis has been largely modified over the past 30 years. Optimization of nutrition is one of the most important contributing factors of this improvement. Nutritional defect result from the conjunction of loss of calories, maldigestion, hypercatabolism and insufficient intake. Pancreatic opotherapy and ADEK vitamin administration is mandatory in pancreatic insufficient patients. Nutritional status must be evaluated at each clinics to detect nutritional defect as early as possible. Nutritional intake must be hypercaloric, normalipidic and adapted to the tastes of the patient. The clinician must be aware of at risk nutritional period: first year of life, puberty, infectious exacerbation, respiratory worsening and diabetes, In neonatal screened babies, recovery of birth weight percentile must be targeted at 6 months, and for the height must be in accordance to genetic height at 2 years. In all cases it is mandatory to treat denutrition by oral supplementation and if necessay enteral nutrition.
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October 2015

Late-onset neonatal infections caused by group B Streptococcus associated with viral infection.

Pediatr Infect Dis J 2007 Oct;26(10):963-5

Service de Bacteriologie, Hôpital Cochin-Saint Vincent de Paul, University Paris V, 27 rue du Faubourg Saint Jacques, 75679, Paris Cedex 14, France.

An association between viral infection and late-onset disease caused by group B Streptococcus (GBS), was systematically looked for in neonates hospitalized for fever during a 3 1/2 year period. Five neonates between 5 to 12.5 months of age presented with meningitis (2 cases) or with septicemia (3 cases) caused by GBS. Viral culture, immunofluorescence, and assay of IFNalpha in blood and cerebrospinal fluid were performed. A viral infection was proved in 4 cases and suspected in 1 case (rash and pharyngitis). We speculate that viral infection may provoke late-onset disease in colonized infants with GBS.
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http://dx.doi.org/10.1097/INF.0b013e3181255ed9DOI Listing
October 2007

The burden of respiratory viral disease in hospitalized children in Paris.

Eur J Pediatr 2008 Apr 12;167(4):435-6. Epub 2007 Jun 12.

Department of Pediatrics and Emergency, University Paris 5, Saint Vincent de Paul APHP Hospital, 75014, Paris, France.

A virus was identified in 464 out of 1,212 patients, 8 days to 16 years of age, who were admitted to the hospital in Paris during a 3 year period. Respiratory syncytial virus (RSV) was found in 428 patients, 74% were younger than 6 months with bronchiolitis, and 6% were older than 2 years. RSV was the first virus isolated in all patients and in those with pneumonia or asthma. The low number of admissions due to parainfluenzae viruses is characteristic of this area compared to other countries.
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http://dx.doi.org/10.1007/s00431-007-0529-5DOI Listing
April 2008

A nutritional support team in the pediatric intensive care unit: changes and factors impeding appropriate nutrition.

Clin Nutr 2007 Jun 17;26(3):355-63. Epub 2007 Apr 17.

Réanimation Pédiatrique, Hôpital Necker-Enfants Malades, APHP, Université René Descartes, Paris, France.

Background & Aims: The aims of this study were to determine the impact of a nutritional support team (NST) intervention in a pediatric intensive care unit (PICU) and to identify the factors at admission that were associated to a delay to achieve a sustained optimal caloric intake (SOCI).

Methods: Caloric and protein intake and nutritional parameters were compared in 82 children in 2000 and 2003, respectively before and after the introduction of a NST. Predictive factors of a delay to achieve the SOCI were identified using multivariate analysis.

Results: There was no difference in 2000 and 2003, respectively, regarding cumulative caloric deficits (19+/-15.7 vs. 20.7+/-14.8 kcal/kg day), cumulative protein deficits (0.26+/-0.31 vs. 0.22+/-0.20 g/kg day), time to achieve a SOCI (7 vs. 7 days). Factors at admission associated with a delay to achieve a SOCI were a pediatric risk of mortality (PRISM) score > 10 (hazard ratio 0.58; 95% CI 0.44-0.77), a CRP > 50 mg/L (hazard ratio 0.49; 95% CI 0.35-0.70), a fluid restriction (hazard ratio 0.51; 95% CI 0.37-0.71), and a weight for age > 3rd centile (hazard ratio 0.54; 95% CI 0.41-0.72).

Conclusions: The intervention of a NST has not modified significantly the nutritional management. In pediatric intensive care, many factors identified at admission are associated with impairing appropriate nutrition.
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http://dx.doi.org/10.1016/j.clnu.2007.02.004DOI Listing
June 2007

Paediatric phenotype of Kallmann syndrome due to mutations of fibroblast growth factor receptor 1 (FGFR1).

Mol Cell Endocrinol 2006 Jul 6;254-255:78-83. Epub 2006 Jun 6.

Assistance Publique Hôpitaux de Paris, Robert Debre Hospital Paediatric Endocrinology unit, Robert Debré Hospital, Paris, France.

Kallmann syndrome characterised by hypogonadotropic hypogonadism (HH) and anosmia is genetically heterogeneous with X-linked, autosomal dominant and autosomal recessive forms. The autosomal dominant form due to loss of function mutation in the fibroblast growth factor receptor 1 (FGFR1) accounts for about 10% of cases. We report here three paediatric cases of Kallmann syndrome with unusual phenotype in two unrelated patients with severe ear anomalies (hypoplasia or agenesis of external ear) associated with classical features, such as cleft palate, dental agenesis, syndactylia, micropenis and cryptorchidism. We found de novo mutation in these two patients (Cys178Ser and Arg622Gly, respectively), and one inherited Arg622Gln mutation with intrafamilial variable phenotype. These genotype-phenotype correlations indicate that paediatric phenotypic expression of FGFR1 loss of function mutations is highly variable, the severity of the oro-facial malformations at birth does not predict gonadotropic function at the puberty and that de novo mutations of FGFR1 are relatively frequent.
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http://dx.doi.org/10.1016/j.mce.2006.04.006DOI Listing
July 2006