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pediatric short bowel syndrome

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https://www.readbyqxmd.com/read/27889929/twenty-eight-years-of-intestinal-transplantation-in-paris-experience-of-the-oldest-european-centre
#1
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
#2
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
#3
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...
November 15, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27849236/treatment-of-short-bowel-syndrome-in-children-value-of-the-intestinal-rehabilitation-program
#4
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
#5
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...
November 3, 2016: Surgery
https://www.readbyqxmd.com/read/27696233/clostridium-difficile-infection-in-children-epidemiology-and-risk-of-recurrence-in-a-low-prevalence-country
#6
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...
September 30, 2016: 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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/26768639/prevalence-of-lactose-malabsorption-and-lactose-intolerance-in-pediatric-patients-with-selected-gastrointestinal-diseases
#16
Katarzyna Pawłowska, Wioleta Umławska, Barbara Iwańczak
BACKGROUND: Lactase is an enzyme involved in the hydrolysis of lactose. Deficiency of the enzyme (hypolactasia) may be determined genetically or arise secondarily to disease of small intestine. Under this condition, lactose enters the colon where it is fermented by intestinal microflora and turns to gases and short-chain fatty acids, causing gastrointestinal symptoms known as lactose intolerance (LI). OBJECTIVES: To investigate the incidence of lactose malabsorption (LM), LI and the coexistence of these two conditions in children with upper gastrointestinal tract diseases (UGTD), malabsorption syndrome, inflammatory bowel disease (IBD) and functional gastrointestinal disorders (FGID)...
September 2015: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/26722617/challenges-of-banding-jejunal-varices-in-an-8-year-old-child
#17
Dalia Belsha, Mike Thomson
Endoscoic variceal ligation (EVL) by the application of bands on small bowel varices is a relatively rare procedure in gastroenterology and hepatology. There are no previously reported paediatric cases of EVL for jejunal varices. We report a case of an eight-year-old male patient with a complex surgical background leading to jejunal varices and short bowel syndrome, presenting with obscure but profound acute gastrointestinal bleeding. Wireless capsule endoscopy and double balloon enteroscopy (DBE) confirmed jejunal varices as the source of bleeding...
December 25, 2015: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/26528281/disruptions-of-the-intestinal-microbiome-in-necrotizing-enterocolitis-short-bowel-syndrome-and-hirschsprung-s-associated-enterocolitis
#18
REVIEW
Holger Till, Christoph Castellani, Christine Moissl-Eichinger, Gregor Gorkiewicz, Georg Singer
Next generation sequencing techniques are currently revealing novel insight into the microbiome of the human gut. This new area of research seems especially relevant for neonatal diseases, because the development of the intestinal microbiome already starts in the perinatal period and preterm infants with a still immature gut associated immune system may be harmed by a dysproportional microbial colonization. For most gastrointestinal diseases requiring pediatric surgery there is very limited information about the role of the intestinal microbiome...
2015: Frontiers in Microbiology
https://www.readbyqxmd.com/read/26495014/-ninjinto-ginseng-decoction-a-traditional-japanese-herbal-medicine-improves-gastrointestinal-symptoms-and-immune-competence-in-patients-with-chronic-intestinal-failure
#19
Shuichiro Uehara, Keiko Ogawa, Junsuke Arimitsu, Hiroomi Okuyama
Background. Treating functional gastrointestinal disorders is extremely difficult. We herein report the effect of the oral administration of Ninjinto (NJT, ginseng decoction), a traditional Japanese Kampo medicine, on chronic intestinal failure. Patients and Methods. Seven patients with chronic intestinal failure treated with NJT were evaluated in this study. The primary diseases included chronic intestinal pseudoobstruction (CIPO: n = 4), short bowel syndrome (SBS: n = 2), and intestinal atresia (n = 1). All patients orally received NJT extract granules at a dose of 0...
2015: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/26336089/epidemiologic-associations-between-short-bowel-syndrome-and-bloodstream-infection-among-hospitalized-children
#20
Benjamin A Miko, Suma S Kamath, Bevin A Cohen, Christie Jeon, Haomiao Jia, Elaine L Larson
BACKGROUND: Children with short bowel syndrome (SBS) suffer from strikingly high rates of morbidity and mortality, due in part to their susceptibility to life-threatening infectious diseases. Few large, multisite studies have evaluated patient-specific factors associated with bacteremia in hospitalized children with and without SBS. METHODS: We conducted a case-control study to examine the epidemiological associations between SBS and bloodstream infections (BSI) in hospitalized children...
September 2015: Journal of the Pediatric Infectious Diseases Society
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