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pediatric short bowel syndrome

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
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
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
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
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...
June 29, 2016: European Journal of Human Genetics: EJHG
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
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
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
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
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
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
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
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
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
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
Jane K Naberhuis, Andrew S Deutsch, Kelly A Tappenden
BACKGROUND: Teduglutide, a glucagon-like peptide-2 (GLP-2) analogue, is available for long-term use by parenteral nutrition (PN)-dependent adults to promote intestinal adaptation but is not approved for use in pediatric patients. The objective of this study was to assess teduglutide-stimulated induced intestinal adaptation, potential synergies with partial enteral nutrition (PEN), and distinct temporal markers of adaptation in a neonatal piglet model of short bowel syndrome (SBS). MATERIALS AND METHODS: Neonatal piglets (48 hours old; n = 72) underwent an 80% jejunoileal resection and were randomized to 1 of 4 treatment groups, in a 2 × 2 factorial design, with PN or PEN (80% standard PN/20% standard enteral nutrition) and teduglutide (0...
August 24, 2015: JPEN. Journal of Parenteral and Enteral Nutrition
Mansi R Shah, Terry L Levin, Steven L Blumer, Walter E Berdon, Dominique M Jan, David K Yousefzadeh
BACKGROUND: Midgut volvulus is a complication of malrotation of bowel and mesenteric malfixation. In contrast, primary volvulus of the small bowel is a distinctly different and rare entity characterized by torsion of the entire small bowel with normal mesenteric fixation. OBJECTIVE: To present the clinical and imaging findings in four infants with primary small bowel volvulus and normal bowel fixation in order to improve awareness of this entity among clinicians and radiologists and to discuss the potential etiologies of this entity to distinguish it from other causes of small bowel volvulus...
December 2015: Pediatric Radiology
Silvana Federici, Maria Domenica Sabatino, Vincenzo Domenichelli, Simona Straziuso
Objective This report documents the authors' experiences in the management of "complex" jejunoileal atresia (JIA) and provides a review of the recent literature on "simple" and "complex" JIA. Materials and Methods This is a retrospective study of eight cases of "complex" JIA managed at the Pediatric Surgical Unit of Infermi Hospital in Rimini from 2002 to 2012. The inclusion criteria are all cases of JIA associated with distal bowel deformities and Types IIIb or IV. One patient had gastroschisis. Results The authors of this study performed primary anastomosis on three patients and enterostomies on five patients...
June 2015: European Journal of Pediatric Surgery Reports
Maryam Khazdouz, Majid Sezavar, Bahareh Imani, Hossein Akhavan, Alireza Babapour, Gholamreza Khademi
BACKGROUND: Bowel function has been reported to be adversely affected following surgery in cases of Hirschsprung. We retrospectively studied both the clinical outcome and bowel function status following surgery in patients diagnosed with Hirschprung's disease (HD). 161 cases, who underwent pull-through operations for HD in Sheikh Pediatric Tertiary Centre, Mashhad, Iran. The specified time bracket spanned between 2006 and 2011. MATERIALS AND METHODS: Data was extracted from Health Information System with the aim of investigating patients for both short and long-term gastrointestinal (GI) complications after surgery bases in addition to the concurrence of any associated anomalies...
April 2015: African Journal of Paediatric Surgery: AJPS
Zev H Davidovics, Beth A Carter, Ruth Ann Luna, Emily B Hollister, Robert J Shulman, James Versalovic
BACKGROUND: Changes in the intestinal microbiome of patients with short bowel syndrome (SBS) are thought to significantly affect clinical outcome. These changes may not only delay enteral diet advancement but may also predispose patients to bacterial translocation, bacteremia, and liver disease. Patients with SBS are thought to be more susceptible to changes in gut microbial communities due to intestinal dysmotility and/or lack of anatomic safeguards such as the ileocecal valve. MATERIALS AND METHODS: We analyzed the bacterial composition of 21 fecal specimens from 9 children with SBS and 8 healthy children ages 4 months to 8 years by 16S ribosomal RNA gene sequencing...
June 9, 2015: JPEN. Journal of Parenteral and Enteral Nutrition
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