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pediatric short bowel syndrome

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https://www.readbyqxmd.com/read/28267037/intravenous-lipid-emulsions-in-pediatric-patients-with-intestinal-failure
#1
Olivier Goulet, Cécile Lambe
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...
April 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28185632/risk-factors-and-outcomes-of-tapering-surgery-for-small-intestinal-dilatation-in-pediatric-short-bowel-syndrome
#2
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
#3
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/28030482/pediatric-parenteral-nutrition-associated-liver-disease
#4
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
#5
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)...
January 25, 2017: Pediatric Research
https://www.readbyqxmd.com/read/27889929/twenty-eight-years-of-intestinal-transplantation-in-paris-experience-of-the-oldest-european-centre
#6
F Lacaille, S Irtan, L Dupic, C Talbotec, F Lesage, V Colomb, N Salvi, F Moulin, F Sauvat, Y Aigrain, Y Revillon, O Goulet, C Chardot
AIM: Our aim is to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. METHODS: After a period (1987-90) of 9 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, 4 multivisceral (3 with kidneys), 1 modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34) and others (1)...
November 27, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27888039/total-midgut-volvulus-in-adults-with-intestinal-malrotation-report-of-eleven-patients
#7
H Kotobi, V Tan, J Lefèvre, F Duramé, G Audry, Y Parc
OBJECTIVE: Total small-intestinal volvulus with malrotation (TSIVM) classically presents in the neonatal period; it occurs much less frequently in the adult and is often misdiagnosed. Prognosis is directly related to the degree and duration of intestinal ischemia. Our goal is to describe our experience with TSIVM in the adult, to identify any specific findings and to discuss its management. METHOD: Eleven patients who had undergone surgery for TSIVM at three centers between 1992 and 2012 were included...
November 22, 2016: Journal of Visceral Surgery
https://www.readbyqxmd.com/read/27855998/outcomes-from-a-12-week-open-label-multicenter-clinical-trial-of-teduglutide-in-pediatric-short-bowel-syndrome
#8
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
#9
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
https://www.readbyqxmd.com/read/27816208/absent-ileocecal-valve-predicts-the-need-for-repeated-step-in-children
#10
Tomas Wester, Helene Engstrand Lilja, Pernilla Stenström, Mikko Pakarinen
BACKGROUND: Serial transverse enteroplasty facilitates weaning from parenteral support in selected patients with short bowel syndrome, although repeated procedure is frequently required. Our aim was to evaluate the outcome of a series of patients after serial transverse enteroplasty and define predictors of repeated serial transverse enteroplasty and weaning off parenteral support. METHODS: All children who underwent serial transverse enteroplasty at 4 Nordic pediatric surgery centers from 2004-2015 were included in this observational study...
March 2017: Surgery
https://www.readbyqxmd.com/read/27696233/clostridium-difficile-infection-in-children-epidemiology-and-risk-of-recurrence-in-a-low-prevalence-country
#11
MULTICENTER STUDY
A Lo Vecchio, L Lancella, C Tagliabue, C De Giacomo, S Garazzino, M Mainetti, L Cursi, E Borali, M V De Vita, E Boccuzzi, L Castellazzi, S Esposito, A Guarino
Clostridium difficile infection (CDI) is increasingly found in children worldwide, but limited data are available from children living in southern Europe. A 6-year retrospective study was performed to investigate the epidemiology, clinical features, treatment, and risk of recurrence in Italy. Data of children with community- and hospital-acquired CDI (CA-CDI and HA-CDI, respectively) seen at seven pediatric referral centers in Italy were recorded retrospectively. Annual infection rates/10,000 hospital admissions were calculated...
January 2017: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/27503937/glucagon-like-peptide-2-stimulates-postresection-intestinal-adaptation-in-preterm-pigs-by-affecting-proteins-related-to-protein-carbohydrate-and-sulphur-metabolism
#12
Pingping Jiang, Andreas Vegge, Thomas Thymann, Jennifer Man-Fan Wan, Per Torp Sangild
BACKGROUND: Exogenous glucagon-like peptide 2 (GLP-2) stimulates intestinal adaptation after resection in animal models of pediatric short bowel syndrome (SBS). It is unknown whether the molecular mechanisms of such GLP-2 effects are similar to those of postresection spontaneous adaptation. Using preterm pigs as a model, we hypothesized that GLP-2 treatment would change the intestinal proteome within the first week after resection, relative to individuals not resected or resected without GLP-2 treatment...
August 8, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27433892/small-bowel-dilatation-predicts-prolonged-parenteral-nutrition-and-decreased-survival-in-pediatric-short-bowel-syndrome
#13
Maria Hukkinen, Reetta Kivisaari, Laura Merras-Salmio, Antti Koivusalo, Mikko P Pakarinen
OBJECTIVE: To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). BACKGROUND: In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. METHODS: SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0...
July 18, 2016: Annals of Surgery
https://www.readbyqxmd.com/read/27412739/-clinical-analysis-of-enteral-nutrition-in-47-children
#14
R D Zhuang, L J Tang, Y H Fang, K R Peng, J Chen
OBJECTIVE: To explore the efficacy and safety of the application of enteral nutrition (EN) in gastrointestinal disease in children, and to explore the possibility of the implementation of family EN. METHOD: Retrospective analysis of disease spectrum, EN approach, preparation, speed and time as well as adverse reactions and outcomes in 47 pediatric patients with gastrointestinal disease underwent EN therapy during July 2014 to March 2015. The nutrition indicators before and after EN therapy were compared by paired t-test...
July 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27352967/genetic-screening-of-congenital-short-bowel-syndrome-patients-confirms-clmp-as-the-major-gene-involved-in-the-recessive-form-of-this-disorder
#15
Maria M Alves, Danny Halim, Reza Maroofian, Bianca M de Graaf, Raoul Rooman, Christine S van der Werf, Els Van de Vijver, Mohammad Yv Mehrjardi, Majid Aflatoonian, Barry A Chioza, Emma L Baple, Mohammadreza Dehghani, Andrew H Crosby, Robert Mw Hofstra
Congenital short bowel syndrome (CSBS) is an intestinal pediatric disorder, where patients are born with a dramatic shortened small intestine. Pathogenic variants in CLMP were recently identified to cause an autosomal recessive form of the disease. However, due to the rare nature of CSBS, only a small number of patients have been reported to date with variants in this gene. In this report, we describe novel inherited variants in CLMP in three CSBS patients derived from two unrelated families, confirming CLMP as the major gene involved in the development of the recessive form of CSBS...
November 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27109979/intestinal-failure-and-transplant-the-australian-experience-2009-to-2014
#16
B Chapman, P De Cruz, R Jones, W Hardikar, A Testro
BACKGROUND: A joint adult and pediatric intestinal transplant (ITx) program for Australia was developed in 2009 to provide life-saving ITx to patients with irreversible intestinal failure (IF). We aimed to analyze the outcomes of patients treated by our service over the past 5 years. METHODS: A retrospective medical record review was conducted on all IF patients referred to our service. Patient demographics, underlying disease, nutrition support, TPN complications, and current transplant program status were evaluated...
March 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27066448/poor-prognostic-factors-in-patients-with-parenteral-nutrition-dependent-pediatric-intestinal-failure
#17
Shin Jie Choi, Kyung Jae Lee, Jong Sub Choi, Hye Ran Yang, Jin Soo Moon, Ju Young Chang, Jae Sung Ko
PURPOSE: Parenteral nutrition (PN) not only provides nutritional support but also plays a crucial role in the treatment of children with intestinal failure. The aim of this study was to evaluate the clinical significance and clinical outcomes of long-term PN. METHODS: Retrospective cohort study was conducted using the medical records of patients treated at Seoul National University Children's Hospital. This study included 19 patients who received PN for over six months...
March 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/26995513/accuracy-of-radiographic-estimation-of-small-bowel-dimensions-in-pediatric-patients-with-short-bowel-syndrome
#18
Daniel Lodwick, Molly Dienhart, Erica Ambeba, Desalegn Yacob, Jane Balint, D Gregory Bates, Peter C Minneci
BACKGROUND: The decision to perform autologous intestinal lengthening in patients with short bowel syndrome (SBS) depends on total bowel length and the diameter and length of dilated segments. This study evaluated the accuracy of radiologic measurements of intestinal length and diameter. METHODS: Patients who underwent an intestinal lengthening procedure with preoperative upper gastrointestinal study (UGI) were identified from 10/2012 through 1/2015. Measurements of total length and diameters and lengths of dilated segments on UGI were compared to intraoperative measurements using Spearman's rank correlation coefficients and Bland-Altman plots...
June 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/26897781/-the-world-experience-of-the-pediatric-intestinal-failure-program-successful-outcomes-from-intestinal-rehabilitation
#19
REVIEW
Benyamine Abbou, Igor Sukhotnik, Amnon Rofe
Management of children with short bowel syndrome is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Pediatric Intestinal Failure Centers were established in 14 pediatric hospitals throughout the United States and Canada and the Pediatric Intestinal Failure Consortium has been developed and is implementing prospective, multi-institutional studies to better define the specific aspects of intestinal failure management that optimize long-term outcomes...
December 2015: Harefuah
https://www.readbyqxmd.com/read/26769282/adding-innovative-practices-and-technology-to-central-line-bundle-reduces-bloodstream-infection-rate-in-challenging-pediatric-population
#20
Marianne Pavia, Marianne Mazza
A specialized pediatric hospital serves many patients with short bowel syndrome. The patients' fecal residue plus frequent access of intravenous lines increases bloodstream infection (BSI) risk. To reduce BSIs, the hospital first implemented an alcohol-dispensing disinfection cap and then added 3 more interventions, with both the cap-only phase and the multipronged phase successfully lowering the hospital's BSI rate.
January 1, 2016: American Journal of Infection Control
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