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Intestinal failure in children

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https://www.readbyqxmd.com/read/28987713/radiographic-measurement-of-intestinal-length-among-children-with-short-bowel-syndrome-retrospective-determination-remains-problematic
#1
Cristine S Velazco, Brenna S Fullerton, Joseph A Brazzo, Charles R Hong, Tom Jaksic, Carol E Barnewolt
PURPOSE: Small bowel length is the most reliable predictor of enteral independence in pediatric short bowel syndrome. Retrospectively measured bowel lengths on upper GI with small bowel follow-through (UGI/SBFT) were compared to operative measurements. METHODS: A pediatric radiologist and surgical trainees blinded to operative measurements retrospectively analyzed UGI/SBFT studies using the digital radiography curved measurement tool. Children with SBS and severe intestinal failure (parenteral nutrition >90days) at a multidisciplinary intestinal failure program 2002-2015 were included...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28964480/pediatric-intestinal-transplantation
#2
Kyle A Soltys, Geoff Bond, Rakesh Sindhi, Sara K Rassmussen, Armando Ganoza, Ajai Khanna, George Mazariegos
The field of intestinal transplantation has experienced dramatic growth since the first reported cases 3 decades ago. Improvements in operative technique, donor assessment and immunosuppressive protocols have afforded children who suffer from life-threatening complications of intestinal failure a chance at long-term survival. As experience has grown, newer diseases, with more systemic manifestations have arisen as potential indications for transplant. After discussing the historical developments of intestinal transplant as a backdrop, this review focuses on the specific pre-operative indications for transplant as well as the great success that intestinal rehabilitation has witnessed over the past decade...
August 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28943136/the-extent-of-intestinal-failure-associated-liver-disease-in-patients-referred-for-intestinal-rehabilitation-is-associated-with-increased-mortality-an-analysis-of-the-pediatric-intestinal-failure-consortium-database
#3
Patrick J Javid, Assaf P Oron, Christopher Duggan, Robert H Squires, Simon P Horslen
BACKGROUND: The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome. METHODS: The multicenter, retrospective Pediatric Intestinal Failure Consortium (PIFCon) database was used to identify all subjects with baseline bilirubin data...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28934206/estimating-the-prevalence-and-intensity-of-schistosoma-mansoni-infection-among-rural-communities-in-western-tanzania-the-influence-of-sampling-strategy-and-statistical-approach
#4
Jared S Bakuza, Matthew J Denwood, Gamba Nkwengulila, Barbara K Mable
BACKGROUND: Schistosoma mansoni is a parasite of major public health importance in developing countries, where it causes a neglected tropical disease known as intestinal schistosomiasis. However, the distribution of the parasite within many endemic regions is currently unknown, which hinders effective control. The purpose of this study was to characterize the prevalence and intensity of infection of S. mansoni in a remote area of western Tanzania. METHODOLOGY/PRINCIPAL FINDINGS: Stool samples were collected from 192 children and 147 adults residing in Gombe National Park and four nearby villages...
September 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28899741/small-bowel-stenosis-a-manifestation-of-chronic-graft-versus-host-disease-in-children
#5
Mickael Tordjman, Marie Ouachee, Arnaud Bonnard, Bogdana Tilea, Karima Yakouben, Jerome Viala, Michel Peuchmaur, Dominique Berrebi
Digestive graft versus host disease (GVHD) is a frequent complication after bone marrow transplantation (BMT), but small bowel obstruction is an extremely rare event. We present herein the first pediatric series of 4 cases of small bowel obstruction after BMT with detailed gross, histological data and their genetic status of the NOD2 gene. All patients had a history of severe acute GVHD treated by immunosuppressive agents and/or infliximab (in 3 cases). Acute or progressively worsening abdominal pain accompanied by small bowel occlusion occurred 5-16months after graft and CT-scan revealed multiple small intestinal stenosis...
September 9, 2017: Human Pathology
https://www.readbyqxmd.com/read/28889146/celiac-disease-in-saudi-children-evaluation-of-clinical-features-and-diagnosis
#6
Anjum Saeed, Asaad Assiri, Hebah Assiri, Anhar Ullah, Mohsin Rashid
 Objectives: To characterize the clinical presentations and diagnosis including serological tests and histopathological findings in children with celiac disease. Methods: All children (less than 18 years) with confirmed celiac disease diagnosed over a 6 year period at a private tertiary care health care center in Riyadh,  Saudi Arabia were studied retrospectively. Information collected included demographics, clinical presentation and diagnostic modalities with serology and small intestinal histology reported by Marsh grading...
September 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28866981/carpal-spasm-in-a-girl-as-initial-presentation-of-celiac-disease-a-case-report
#7
Atifete Ramosaj-Morina, A Keka-Sylaj, V Hasbahta, A Baloku-Zejnullahu, M Azemi, R Zunec
BACKGROUND: Celiac disease is an immune-mediated disorder elicited by ingestion of gluten in genetically susceptible persons. This disorder is characterized by specific histological changes of the small intestine mucosa resulting in malabsorption. This case was written up as it was an unusual and dramatic presentation of celiac disease. CASE PRESENTATION: We report the case of a 3-year-old Albanian girl who presented at our clinic with carpal spasms and hand paresthesia...
September 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28866623/epidemiology-management-and-outcome-of-ultrashort-bowel-syndrome-in-infancy
#8
REVIEW
Akshay Batra, Simon Charlie Keys, Mark John Johnson, Robert A Wheeler, Robert Mark Beattie
Ultrashort bowel syndrome (USBS) is a group of heterogeneous disorders where the length of small bowel is less than 10 cm or 10% of expected for the age. It is caused by massive loss of the gut which in the neonatal period can be a result of vanishing gastroschisis or surgical resection following mid-gut volvulus, jejunoileal atresia and/or extensive necrotising enterocolitis. The exact prevalence of USBS is not known although there is a clear trend towards increasing numbers because of increased incidence and improved survival...
September 2, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/28862189/typical-atypical-enteropathogenic-escherichia-coli-in-diarrhoea-their-role-as-carrier-in-children-under-five
#9
Taru Singh, Shukla Das, V G Ramachandran, Dheeraj Shah, Rumpa Saha, Sajad Ahmad Dar, Arvind Rai
BACKGROUND & OBJECTIVES: Multidrug-resistant enteropathogenic Escherichia coli (EPEC) is responsible for a large number of cases of infantile diarrhoea in developing countries, causing failure in treatment with consequent health burden and resulting in a large number of deaths every year. This study was undertaken to determine the proportion of typical and atypical EPEC in under five children with diarrhoea and controls, their function as a carriage and to identify virulent genes associated with them...
April 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28801782/common-pediatric-medical-emergencies-in-office-practice
#10
Bharat Mehra, Suresh Gupta
General Practitioners frequently see children with medical conditions that may evolve into an emergency if not promptly attended to. The most common emergencies encountered in pediatric office practice are respiratory distress, dehydration, anaphylaxis, seizures and trauma. Assessment of children is sometimes difficult as the signs and symptoms might be subtle and not markedly expressed. Also, normal value of vital signs vary with age, thus their interpretation requires discrete knowledge of age appropriate values...
August 12, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28750860/impaired-barrier-function-and-autoantibody-generation-in-malnutrition-enteropathy-in-zambia
#11
Beatrice Amadi, Ellen Besa, Kanekwa Zyambo, Patrick Kaonga, John Louis-Auguste, Kanta Chandwe, Phillip I Tarr, Donna M Denno, James P Nataro, William Faubion, Anne Sailer, Sunil Yeruva, Tricia Brantner, Joseph Murray, Andrew J Prendergast, Jerrold R Turner, Paul Kelly
Intestinal damage in malnutrition constitutes a threat to the survival of many thousands of children globally. We studied children in Lusaka, Zambia, with severe acute malnutrition (SAM) and persistent diarrhea using endoscopy, biopsy and analysis of markers and protective proteins in blood and intestinal secretions. We carried out parallel investigations in apparently healthy adults, and analyzed biomarkers only in apparently healthy children. Villus height and crypt depth did not differ in children with SAM and adult controls, but epithelial surface was reduced in children with SAM (median 445, interquartile range (IQR) 388, 562μm per 100μm muscularis mucosae) compared to adults (578, IQR 465,709; P=0...
August 2017: EBioMedicine
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#12
Anita M Nucci, Kipp Ellsworth, Austin Michalski, Emily Nagel, Jackie Wessel
BACKGROUND: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized. METHODS: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section...
July 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28687683/substantial-harm-associated-with-failure-of-chronic-paediatric-central-venous-access-devices
#13
Amanda J Ullman, Tricia Kleidon, Marie Cooke, Claire M Rickard
Central venous access devices (CVADs) form an important component of modern paediatric healthcare, especially for children with chronic health conditions such as cancer or gastrointestinal disorders. However device failure and complications rates are high.Over 2½ years, a child requiring parenteral nutrition and associated vascular access dependency due to 'short gut syndrome' (intestinal failure secondary to gastroschisis and resultant significant bowel resection) had ten CVADs inserted, with ninesubsequently failing...
July 6, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28655415/molecular-signature-of-active-fibrogenesis-prevails-in-biliary-atresia-after-successful-portoenterostomy
#14
Anna Kerola, Hanna Lampela, Jouko Lohi, Päivi Heikkilä, Annika Mutanen, Hannu Jalanko, Mikko P Pakarinen
BACKGROUND: In biliary atresia mechanisms of progressive liver injury leading to need of liver transplantation after successful portoenterostomy remain unknown. A better understanding is a prerequisite for development of novel therapies to extend native liver survival, and we aimed to unravel molecular characteristics of liver injury after successful portoenterostomy. METHODS: Liver biopsies obtained from 28 biliary atresia children during successful portoenterostomy and at median age 3...
September 2017: Surgery
https://www.readbyqxmd.com/read/28604513/severe-sarcopenia-and-increased-fat-stores-in-pediatric-patients-with-liver-kidney-or-intestine-failure
#15
Richard S Mangus, Weston J Bush, Chandrashekhar A Kubal, Christina Miller
OBJECTIVES: Malnutrition and wasting predict clinical outcomes in children with severe chronic illness. Objectively calculated malnutrition in children with end-stage organ failure has not been well studied. This analysis compares children with kidney, liver or intestine failure to healthy controls to quantitate the disparity in muscle and fat stores. METHODS: Children younger than age 19 with end stage liver, kidney or intestine failure and with pre-transplant computed tomography (CT) imaging were selected from the transplant database...
June 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28540669/tropical-enteropathies
#16
REVIEW
John Louis-Auguste, Paul Kelly
PURPOSE OF REVIEW: The term 'tropical enteropathy' originated in observations in the 1960s that small intestinal morphology and function differed in the tropics from the norms found in temperate climates. It was subsequently shown that this enteropathy is more closely related to environmental conditions than latitude, and it was re-labelled 'environmental enteropathy'. It is now recognised that environmental enteropathy (also now called environmental enteric dysfunction) has implications for the health and linear growth of children in low- and middle-income countries, and it may underlie poor responses to oral vaccination in these countries...
July 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28521656/oral-feeding-difficulties-in-children-with-short-bowel-syndrome-a-narrative-review
#17
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/28521607/intravenous-fish-oil-and-pediatric-intestinal-failure-associated-liver-disease-changes-in-plasma-phytosterols-cytokines-and-bile-acids-and-erythrocyte-fatty-acids
#18
Kara L Calkins, Andrea DeBarber, Robert D Steiner, Martiniano J Flores, Tristan R Grogan, Susanne M Henning, Laurie Reyen, Robert S Venick
BACKGROUND: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB). DESIGN: This study enrolled IFALD children who received 6 months of FO...
May 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28514243/celiac-disease-symptom-resolution-effectiveness-of-the-gluten-free-diet
#19
Naire Sansotta, Stefano Guandalini, Karine Amirikian, Hilary Jericho
OBJECTIVE: To evaluate the efficacy of the gluten free diet (GFD) on gastrointestinal and extra-intestinal symptom resolution and identify predictors for persistence of symptoms in all celiac patients at the University of Chicago. METHODS: We conducted a retrospective chart review from 2002 to 2015. Gastrointestinal (GI) symptoms included: abdominal pain, bloating, constipation, diarrhea, failure to thrive/weight loss, nausea, reflux and vomiting. Extra-intestinal (EI) symptoms included: abnormal liver enzymes, arthralgia/arthritis, dermatitis herpetiformis, alopecia, fatigue, headache, anemia, stomatitis, myalgia, psychiatric disorders, rashes, seizures, neuropathy, short stature, delayed puberty, osteoporosis, and infertility...
May 16, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28502441/intestinal-transplant-in-children
#20
REVIEW
Nidhi Rawal, Nada Yazigi
The past decade has seen major advances in the field of transplantation; it is the treatment of choice for many with intestinal failure. One-year mortality from pediatric intestinal transplantation has significantly declined, from 30% to 10% to 15% nationally, mainly due to a multidisciplinary approach in transplant centers. Pediatric age carries special considerations along the spectrum of care that continue to cause challenges but also offers growth opportunities. Pediatric intestinal transplantation indications and timing are changing as a result of new developments in diagnostic and treatment tools...
June 2017: Pediatric Clinics of North America
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