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Short bowel syndrome in children

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https://www.readbyqxmd.com/read/28521656/oral-feeding-difficulties-in-children-with-short-bowel-syndrome-a-narrative-review
#1
Judy Hopkins, Sharon A Cermak, Russell J Merritt
Children with short bowel syndrome (SBS) with associated intestinal failure may be unable to absorb sufficient nutrients to sustain life. Improvements in the medical management of SBS, including use of parenteral nutrition, has significantly increased life expectancy. Independence from parenteral nutrition further improves quality of life. However, children living with SBS often develop oral aversions and feeding difficulties. There is limited research and information on which to base interventions that will preserve and develop oral motor and feeding skills...
May 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28510260/markers-of-enteral-adaptation-in-pediatric-cases-with-short-bowel-syndrome
#2
Masahiro Chiba, Yutaka Sanada, Akira Toki
BACKGROUND: This study aimed to ascertain if prospective determinations of specific gut hormones and growth factors could predict bowel adaptation in children with short bowel syndrome (SBS). METHODS: We studied parenteral nutrition (PN) independency as the short-term result and discontinuation of enteral nutrition (EN) as the long-term result from a retrospective chart review of 7 patients with SBS, who were managed in the absence of growth retardation. The correlation between increased numbers of enteral feedings or enteral nutrients and fasting levels of serum gastrin, glucagon-like peptide 2 (GLP-2), citrulline, and diamine oxidase (DAO) activity was analyzed...
May 16, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28462379/prolonged-absence-of-mechanoluminal-stimulation-in-human-intestine-alters-the-transcriptome-and-intestinal-stem-cell-niche
#3
Minna M Wieck, Christopher R Schlieve, Matthew E Thornton, Kathryn L Fowler, Mubina Isani, Christa N Grant, Ashley E Hilton, Xiaogang Hou, Brendan H Grubbs, Mark R Frey, Tracy C Grikscheit
BACKGROUND & AIMS: For patients with short-bowel syndrome, intestinal adaptation is required to achieve enteral independence. Although adaptation has been studied extensively in animal models, little is known about this process in human intestine. We hypothesized that analysis of matched specimens with and without luminal flow could identify new potential therapeutic pathways. METHODS: Fifteen paired human ileum samples were collected from children aged 2-20 months during ileostomy-reversal surgery after short-segment intestinal resection and diversion...
May 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28351520/toll-like-receptor-4-is-critical-for-the-development-of-resection-associated-hepatic-steatosis
#4
Lauren K Barron, James W Bao, Bola G Aladegbami, Jason J Colasanti, Jun Guo, Christopher R Erwin, Brad W Warner
BACKGROUND: A significant number of children with short bowel syndrome experience intestinal failure-associated liver disease. We recently demonstrated accelerated hepatic steatosis after 50% small bowel resection (SBR) in mice. Since SBR is associated with alterations in the gut microbiome, the purpose of this study was to determine whether TLR4 signaling is critical to the development of resection-associated hepatic steatosis. METHODS: Male C57BL6 (control) and TLR4-knockout (KO) mice underwent 50% proximal SBR...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28343660/a-pilot-study-of-ultrasound-elastography-as-a-non-invasive-method-to-monitor-liver-disease-in-children-with-short-bowel-syndrome
#5
Daniel Lodwick, Molly Dienhart, Jennifer N Cooper, Bonita Fung, Joseph Lopez, Sally Smith, Patrick Warren, Jane Balint, Peter C Minneci
BACKGROUND: Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS). METHODS: This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30days of each other between 12/2014-9/2015. The mean and median SWS were compared to the stage of fibrosis...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28230078/unravelling-the-metabolic-impact-of-sbs-associated-microbial-dysbiosis-insights-from-the-piglet-short-bowel-syndrome-model
#6
Prue M Pereira-Fantini, Sean G Byars, James Pitt, Susan Lapthorne, Fiona Fouhy, Paul D Cotter, Julie E Bines
Liver disease is a major source of morbidity and mortality in children with short bowel syndrome (SBS). SBS-associated microbial dysbiosis has recently been implicated in the development of SBS-associated liver disease (SBS-ALD), however the pathological implications of this association have not been explored. In this study high-throughput sequencing of colonic content from the well-validated piglet SBS-ALD model was examined to determine alterations in microbial communities, and concurrent metabolic alterations identified in urine samples via targeted mass spectrometry approaches (GC-MS, LC-MS, FIA-MS) further uncovered impacts of microbial disturbance on metabolic outcomes in SBS-ALD...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28194723/teduglutide-a-review-in-short-bowel-syndrome
#7
REVIEW
Esther S Kim, Susan J Keam
Subcutaneous teduglutide (Revestive(®)), a glucagon-like peptide-2 analogue that increases intestinal absorption, is approved in the EU for the treatment of short bowel syndrome (SBS) in patients aged ≥1 year who are stable following a period of postsurgical intestinal adaptation. In a phase III trial in adults with SBS intestinal failure (IF) dependent on parenteral support (PS), a significantly greater proportion of teduglutide 0.05 mg/kg/day than placebo recipients achieved a ≥20% reduction in weekly PS volume from baseline to week 20 and maintained it to week 24...
March 2017: Drugs
https://www.readbyqxmd.com/read/28190552/use-of-balloon-dilatation-for-management-of-postoperative-intestinal-strictures-in-children-with-short-bowel-syndrome
#8
Christina Belza, Kevin Fitzgerald, Joao Amaral, Karen Steinberg, Yaron Avitzur, Paul W Wales
PURPOSE: Children with short bowel syndrome (SBS) often require numerous operations to optimize intestinal function. Postoperative intestinal strictures are a complication that inhibits enteral feeding advancement and prolongs parenteral nutrition dependency, often requiring reoperation. Our objective was to review our experience with fluoroscopic balloon dilatation to treat intestinal strictures. METHODS: A retrospective cohort study of intestinal failure patients with SBS was completed...
May 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185632/risk-factors-and-outcomes-of-tapering-surgery-for-small-intestinal-dilatation-in-pediatric-short-bowel-syndrome
#9
Maria Hukkinen, Reetta Kivisaari, Antti Koivusalo, Mikko P Pakarinen
BACKGROUND: In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. METHODS: Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n=16) to those managed conservatively (n=44) with Cox proportional hazards regression. RESULTS: SBS was caused by necrotizing enterocolitis (NEC) (n=31), SB atresia (SBA) (n=13), midgut volvulus (n=12), or gastroschisis (n=4)...
January 31, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28052307/ultrashort-bowel-syndrome-outcome-in-children-treated-in-a-multidisciplinary-intestinal-rehabilitation-unit
#10
Mariela Dore, Paloma Triana Junco, Ane Andres Moreno, Vanesa Nuñez Cerezo, Martha Romo Muñoz, Alba Sánchez Galán, Alejandra Vilanova Sánchez, Gerardo Prieto, Esther Ramos, Francisco Hernandez, Leopoldo Martínez Martínez, Manuel Lopez Santamaria
Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed...
February 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28045772/nutritional-practices-and-growth-in-premature-infants-after-surgical-necrotizing-enterocolitis
#11
Grace C Lin, Daniel T Robinson, Steven Olsen, Kristina M Reber, Mohannad Moallem, Robert DiGeronimo, Cecilia Mulroy, Ankur Datta, Karna Murthy
OBJECTIVE: To describe the nutritional provisions received by infants with surgical NEC and the associated effects on short-term growth. STUDY DESIGN: Through the Children's Hospitals Neonatal Database we identified infants born ≤32 weeks' gestation with surgical NEC from 5 regional neonatal intensive care units over 4 years. Excluded infants had isolated intestinal perforation and died <14 days post-operatively. Infants were stratified by their median parenteral protein dose (low (LP) or high (HP) protein) over the first post-operative week...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28039510/surgical-strategies-in-short-bowel-syndrome
#12
REVIEW
Michael E Höllwarth
Extensive loss of small bowel in all age groups has significant morbidity and mortality consequences. Despite the astonishing ability of the small bowel to compensate for an extensive loss, long-term parenteral nutrition and enteral nutrition, tailored to the need of the patients in relation to the missing intestinal regions is needed. Organ-preserving surgical intervention becomes necessary in patients with a very short intestinal transit time and in an other group of patients with impaired propulsive peristalsis...
April 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28030482/pediatric-parenteral-nutrition-associated-liver-disease
#13
REVIEW
Jill C Israelite
Pediatric parenteral nutrition-associated liver disease (PNALD) is typically defined as a decrease in bile flow that is independent of a mechanical obstruction and of any other underlying liver disease. It is most often seen in pediatric patients receiving parenteral nutrition support. Up to 50% to 66% of children receiving long-term parenteral nutrition are reported to be diagnosed with PNALD. The goal of treatment for PNALD is advancement to full enteral nutrition and elimination of dependence on parenteral nutrition support...
January 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/27997531/redefining-short-bowel-syndrome-in-the-21st-century
#14
REVIEW
Valeria C Cohran, Joshua D Prozialeck, Conrad R Cole
In 1968, Wilmore and Dudrick reported an infant sustained by parenteral nutrition (PN) providing a potential for survival for children with significant intestinal resections. Increasing usage of TPN over time led to some patients developing Intestinal Failure Associated Liver Disease (IFALD), a leading cause of death and indication for liver/intestinal transplant. Over time, multidisciplinary teams called Intestinal Rehabilitation Programs (IRPs) began providing meticulous and innovative management. Usage of alternative lipid emulsions and lipid minimization strategies have resulted in the decline of IFALD and an increase in long-term and transplant-free survival, even in the setting of ultrashort bowel (< 20 cm)...
April 2017: Pediatric Research
https://www.readbyqxmd.com/read/27912975/patient-reported-swedish-nationwide-outcomes-of-children-and-adolescents-with-total-colonic-aganglionosis
#15
Pernilla Stenström, Matilda Brautigam, Helena Borg, Christina Graneli, Helene Engstrand Lilja, Tomas Wester
BACKGROUND: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years. METHODS: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition...
November 17, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27889929/twenty-eight-years-of-intestinal-transplantation-in-paris-experience-of-the-oldest-european-center
#16
Florence Lacaille, Sabine Irtan, Laurent Dupic, Cécile Talbotec, Fabrice Lesage, Virinie Colomb, Nadège Salvi, Florence Moulin, Frédérique Sauvat, Yves Aigrain, Yann Revillon, Olivier Goulet, Christophe Chardot
Our aim was to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. After a period (1987-1990) of nine isolated small bowel transplants (SBTx) where only one patient survived with her graft, 110 ITx were performed on 101 children from 1994 to 2014: 60 SBTx, 45 liver-small bowel, four multivisceral (three with kidneys), and one modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34), and others (1)...
February 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27867690/good-adherence-to-mediterranean-diet-can-prevent-gastrointestinal-symptoms-a-survey-from-southern-italy
#17
Francesco Paolo Zito, Barbara Polese, Letizia Vozzella, Antonietta Gala, Daniela Genovese, Viviana Verlezza, Fabiana Medugno, Antonello Santini, Luigi Barrea, Martina Cargiolli, Paolo Andreozzi, Giovanni Sarnelli, Rosario Cuomo
AIM: To evaluate how different levels of adherence to a mediterranean diet (MD) correlate with the onset of functional gastrointestinal disorders. METHODS: As many as 1134 subjects (598 M and 536 F; age range 17-83 years) were prospectively investigated in relation to their dietary habits and the presence of functional gastrointestinal symptoms. Patients with relevant chronic organic disease were excluded from the study. The Mediterranean Diet Quality index for children and adolescents (KIDMED) and the Short Mediterranean Diet Questionnaire were administered...
November 6, 2016: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/27865472/taking-a-step-back-assessing-the-outcomes-of-multiple-step-procedures
#18
COMPARATIVE STUDY
Meredith Barrett, Farokh R Demehri, Graham C Ives, Kristen Schaedig, Meghan A Arnold, Daniel H Teitelbaum
PURPOSE: Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)-associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. METHODS: This retrospective review of STEPs included 17 children and a total of 24 procedures...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27855998/outcomes-from-a-12-week-open-label-multicenter-clinical-trial-of-teduglutide-in-pediatric-short-bowel-syndrome
#19
Beth A Carter, Valeria C Cohran, Conrad R Cole, Mark R Corkins, Reed A Dimmitt, Christopher Duggan, Susan Hill, Simon Horslen, Joel D Lim, David F Mercer, Russell J Merritt, Peter F Nichol, Luther Sigurdsson, Daniel H Teitelbaum, John Thompson, Charles Vanderpool, Juliana F Vaughan, Benjamin Li, Nader N Youssef, Robert S Venick, Samuel A Kocoshis
OBJECTIVE: To determine safety and pharmacodynamics/efficacy of teduglutide in children with intestinal failure associated with short bowel syndrome (SBS-IF). STUDY DESIGN: This 12-week, open-label study enrolled patients aged 1-17 years with SBS-IF who required parenteral nutrition (PN) and showed minimal or no advance in enteral nutrition (EN) feeds. Patients enrolled sequentially into 3 teduglutide cohorts (0.0125 mg/kg/d [n = 8], 0.025 mg/kg/d [n = 14], 0...
February 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/27849236/treatment-of-short-bowel-syndrome-in-children-value-of-the-intestinal-rehabilitation-program
#20
REVIEW
Uenis Tannuri, Fabio de Barros, Ana Cristina Aoun Tannuri
The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel...
September 2016: Revista da Associação Médica Brasileira
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