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Intestinal failure in children

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https://www.readbyqxmd.com/read/27857647/nutrition-chez-le-br%C3%A3-l%C3%A3
#1
G Perro
Nutrition is a challenge for burn patients. Emphasized points are early enteral amounts by means of a naso-gastric tube, semi-recumbent position > 30°, gastro-intestinal survey improved by kinetics, weight evolution, nutritional amounts assessment to prevent proteino-energetic deficiency. Feeding is difficult with children, as with seniors often associating a preexisting starvation. Nutritional supplements are useful in medium severity patients. For overweight patients, hypo caloric and hyperprotidic diet is the better choice...
March 31, 2016: Annals of Burns and Fire Disasters
https://www.readbyqxmd.com/read/27855998/outcomes-from-a-12-week-open-label-multicenter-clinical-trial-of-teduglutide-in-pediatric-short-bowel-syndrome
#2
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
#3
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/27836825/intestinal-failure-in-children-and-young-people-with-neurodisabling-conditions
#4
REVIEW
Amar M Wahid, Colin V Powell, Ieuan H Davies, Jennifer A Evans, Huw R Jenkins
Gastrointestinal dysmotility is common in children and young people with neurodisabling conditions. In this article we seek to highlight the increasing difficulties faced by paediatricians in managing intestinal failure in this patient group. It is becoming clear that, as the median age for survival increases, intestinal failure is a significant problem, and can in some cases become life-limiting. The ethical issues around starting children with life-limiting conditions on parenteral nutrition (PN) are extremely complicated, not least because we are ignorant of the mechanism of intestinal failure in these children, and indeed, which of these children might be able to return to enteral feeding after a period of PN...
November 11, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27784959/endocrine-manifestations-in-celiac-disease
#5
REVIEW
Hugh James Freeman
Celiac disease (CD) is an autoimmune small intestinal mucosal disorder that often presents with diarrhea, malabsorption and weight loss. Often, one or more associated endocrine disorders may be associated with CD. For this review, methods involved an extensive review of published English-language materials. In children and adolescents, prospective studies have demonstrated a significant relationship to insulin-dependent or type 1 diabetes, whereas in adults, autoimmune forms of thyroid disease, particularly hypothyroidism, may commonly co-exist...
October 14, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27782890/pediatric-small-bowel-transplantation-a-single-center-experience-from-turkey
#6
Cem Tuğmen, Maşallah Baran, İsmail Sert, Ayşe Berna Anıl, Eyüp Kebapçı, Sait Murat Doğan, Selçuk Kılınç, Gaye Aydın, Ayşe Gülden Diniz, Mustafa Ölmez, İbrahim Pirim, Cezmi Karaca
BACKGROUND/AIMS: Small bowel transplantation (SBTx) is a treatment option for patients with serious parenteral nutrition-related problems in intestinal failure. İzmir Tepecik Training Research Hospital Organ Transplantation Center is still the only pediatric intestinal transplant center in Turkey. MATERIAL AND METHODS: This study was approved by the local ethics committee. Patients' data were analyzed from the medical charts and the hospital digital database. Seven isolated SBTxs were performed in six children between 2010 and 2016...
September 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27781020/stem-cells-in-functional-bladder-engineering
#7
REVIEW
Jakub Smolar, Souzan Salemi, Maya Horst, Tullio Sulser, Daniel Eberli
Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need urinary diversion or augmentation cystoplasty as when untreated they may cause severe bladder dysfunction and kidney failure. Currently, the gold standard therapy of end-stage bladder disease refractory to conservative management is enterocystoplasty, a surgical enlargement of the bladder with intestinal tissue. Despite providing functional improvement, enterocystoplasty is associated with significant long-term complications, such as recurrent urinary tract infections, metabolic abnormalities, stone formation, and malignancies...
September 2016: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/27743023/a-technique-for-re-utilizing-catheter-insertion-sites-in-children-with-difficult-central-venous-access
#8
S M Johnson, G M Garnett, R K Woo
Maintenance of central venous access in patients with chronic medical conditions such as short bowel syndrome demands forethought and ingenuity. We describe an innovative technique for re-utilizing central venous access sites in patients who have chronic central venous access needs. Records of patients undergoing this technique were reviewed between August 2012 and December 2015. The technique involves "cutting-down" to the sterile fibrous tunnel that naturally forms around tunneled catheters. The fibrous sheath is then isolated and controlled much as would be done for a venous "cut-down...
October 14, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27721967/multi-visceral-transplantation-in-a-21-year-old-man-with-prior-pancreatoblastoma
#9
R Girlanda, A Pozzi, C S Matsumoto, T M Fishbein
Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver transplantation have so far been reported in children with hepatic metastases from pancreatoblastoma, a rare malignant neoplasm originating from the epithelial exocrine cells of the pancreas. Herein, we describe a case of a successful multi-visceral transplant in a man with intestinal failure after surgical resection of pancreatoblastoma.
2016: International Journal of Organ Transplantation Medicine
https://www.readbyqxmd.com/read/27704325/intestinal-failure-in-children
#10
REVIEW
Theodoric Wong, Girish Gupte
Children with intestinal failure have had improved survival, particularly those with extreme short bowel syndrome, over the last 10-15 y. This has been attributed to better understanding of the pathophysiology of intestinal failure, improvement in line care, recognition of the importance of a team approach as well as the progress of intestinal transplant as a viable option. Parenteral nutrition remains the cornerstone for the continual survival of these patients. This review will cover contemporary approaches to intestinal failure including post surgical approaches, non-transplant surgery, dietetic and medication approaches during the adaptation process, considerations for home parenteral nutrition and latest in intestinal transplantation...
November 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27695493/congenital-duodenal-obstruction-with-delayed-presentation-seven-years-of-experience
#11
Seyed Abdollah Mousavi, Hasan Karami, Hossein Saneian
INTRODUCTION: The duodenum is the most common site for congenital intestinal obstruction. The duodenal web with a central hole can present without any overt signs of obstruction at a later age. MATERIAL AND METHODS: Over a 7-year period, children with congenital intestinal obstruction were identified in this study. The complications and operative findings of patients with duodenal web with conditions such as wind sock deformity and delayed diagnosis were evaluated in this study...
October 1, 2016: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/27683392/pediatric-intestinal-failure-associated-liver-disease-challenges-in-identifying-clinically-relevant-biomarkers
#12
Kathleen M Gura, Andrew E Mulberg, Paul D Mitchell, John Yap, Clara Y Kim, Minjun Chen, Alexis Potemkin, Mark Puder
BACKGROUND: Intestinal failure-associated liver disease (IFALD) is complex and diagnosed by concurrent use of parenteral nutrition, clinical presentation, and alterations in hepatic biomarkers exclusive of other causes of liver disease. In comparison with individual measures, composite biomarkers may provide a more effective means for assessing disease progression and response to treatment than single parameters. Since IFALD is considered by some to be a type of drug-induced liver injury (DILI), those diagnostic criteria could potentially be used in this population...
September 28, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27663542/serum-fasting-glp-1-and-glp-2-associate-with-intestinal-adaptation-in-pediatric-onset-intestinal-failure
#13
Annika Mutanen, Mikko P Pakarinen
AIM: Glukagon-like-peptide-1 (GLP-1) and -2 (GLP-2), produced by intestinal L-cells, are key hormones regulating intestinal transit, secretion, absorption, and mucosal growth. We evaluated naïve fasting serum GLP-1 and GLP-2 levels in pediatric intestinal failure (IF). METHODS: Fifty-five IF patients with median age 4.2 (IQR 1.3-12) years and 47 matched healthy controls underwent measurement of fasting serum GLP-1 and GLP-2. RESULTS: Serum GLP-2 [19...
September 14, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27659551/-somatotropic-axis-and-molecular-markers-of-mineral-metabolism-in-children-undergoing-chronic-peritoneal-dialysis
#14
María Luisa Ceballos Osorio, Francisco Cano Schuffeneger
: Growth failure is one of the most relevant complications in children with chronic kidney disease (CKD). Among others, growth hormone (GH) resistance and bone mineral disorders have been identified as the most important causes of growth retardation. OBJECTIVES: 1. To characterize bone mineral metabolism and growth hormone bio-markers in CKD children treated with chronic peritoneal dialysis (PD). 2. To evaluate height change with rhGH treatment. PATIENTS AND METHOD: A longitudinal 12-month follow-up in prepuberal PD children...
September 19, 2016: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/27559471/improving-clinical-remission-rates-in-pediatric-inflammatory-bowel-disease-with-previsit-planning
#15
Jeffrey R Savarino, Jess L Kaplan, Harland S Winter, Christopher J Moran, Esther J Israel
Inflammatory Bowel Disease (IBD) is a chronic autoimmune inflammatory disease of the intestine which can lead to malnutrition, poor quality of life, and colon cancer.(1-4) Although there is no cure for the disease, clinical remission is the primary goal.(5) The Center for Inflammatory Bowel Disease at MassGeneral Hospital for Children (MGHfC) adopted a Previsit Planning (PVP) model to identify and discuss symptomatic patients prior to their appointments to identify specific issues that impact disease management...
2016: BMJ Quality Improvement Reports
https://www.readbyqxmd.com/read/27548247/high-prevalence-of-eosinophilic-gastrointestinal-disease-in-children-with-intestinal-failure
#16
Danielle A Stamm, Elizabeth Hait, Heather J Litman, Paul D Mitchell, Christopher Duggan
OBJECTIVES: The aim of the present study was to describe the prevalence and clinical features of gastrointestinal (GI) eosinophilic inflammation among pediatric patients with intestinal failure (IF). METHODS: Medical records of all patients studied in our institution's IF program who underwent GI endoscopy over a 15-year period were reviewed, and clinical, pathologic, nutrition, and laboratory data collected. RESULTS: One hundred five patients underwent 208 GI endoscopic procedures with biopsy...
September 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27540004/potential-hepatotoxicities-of-intravenous-fat-emulsions-in-infants-and-children
#17
REVIEW
T Hang Nghiem-Rao
Infants and children who depend on parenteral nutrition are among the most vulnerable to developing potentially devastating intestinal failure-associated liver disease. While the pathogenesis of intestinal failure-associated liver disease remains unclear, evidence for the contribution of fat emulsions to cholestasis and liver injury has rapidly increased in recent years. Data demonstrating the interaction among phytosterols, fatty acids, and antioxidants in cellular pathways that mediate bile flow and hepatic injury have led to the development of newer alternative fat emulsions...
October 2016: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/27510535/predictors-of-failure-of-fish-oil-therapy-for-intestinal-failure-associated-liver-disease-in-children
#18
Prathima Nandivada, Meredith A Baker, Paul D Mitchell, Alison A O'Loughlin, Alexis K Potemkin, Lorenzo Anez-Bustillos, Sarah J Carlson, Duy T Dao, Gillian L Fell, Kathleen M Gura, Mark Puder
BACKGROUND: Parenteral fish-oil (FO) therapy is a safe and effective treatment for intestinal failure-associated liver disease (IFALD). Patients whose cholestasis does not resolve with FO may progress to end-stage liver disease. OBJECTIVE: We sought to identify factors associated with the failure of FO therapy in treating IFALD to guide prognostication and referral guidelines. DESIGN: Prospectively collected data for patients treated with FO at Boston Children's Hospital from 2004 to 2014 were retrospectively reviewed...
September 2016: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/27499927/increased-mmp-7-expression-in-biliary-epithelium-and-serum-underpins-native-liver-fibrosis-after-successful-portoenterostomy-in-biliary-atresia
#19
Anna Kerola, Hanna Lampela, Jouko Lohi, Päivi Heikkilä, Annika Mutanen, Jaana Hagström, Taina Tervahartiala, Timo Sorsa, Caj Haglund, Hannu Jalanko, Mikko P Pakarinen
The molecular mechanisms underlying progressive liver fibrosis following surgical treatment of biliary atresia (BA) remain unclear. Our aim was to address hepatic gene and protein expression and serum levels of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) after successful portoenterostomy (PE), and relate them to histological signs of liver injury, clinical follow-up data and biochemical markers of hepatic function. LIver biopsies and serum samples were obtained from 25 children after successful PE at median age of 3...
July 2016: Journal of Pathology. Clinical Research
https://www.readbyqxmd.com/read/27491714/transition-to-a-tube-feeding-formula-with-real-food-ingredients-in-pediatric-patients-with-intestinal-failure
#20
Kate Samela, Jasmeet Mokha, Karan Emerick, Zev H Davidovics
Due to concerns related primarily to allergic response and malabsorption, enteral nutrition therapy has traditionally relied on the use of elemental formulas in children with intestinal failure (IF). Blended food diets via a gastrostomy tube have been reported to improve feeding tolerance in pediatric populations receiving long-term enteral nutrition therapy. Complex macronutrients have been shown to stimulate intestinal adaptation in animal models. We report on our experience in children with IF who had an overall improvement in stool output when transitioned from an elemental formula to a tube feeding formula with real food ingredients (TFRF)...
August 4, 2016: Nutrition in Clinical Practice
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