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Intestinal failure in children

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https://www.readbyqxmd.com/read/29916949/central-line-in-long-term-parenteral-nutrition-in-children-a-european-survey
#1
Iva Hojsak, Florence Lacaille, Girish L Gupte, Jutta Köglmeier
BACKGROUND AND AIMS: The guidelines for the insertion and maintenance of the central venous catheter (CVC) in children on long-term parenteral nutrition (PN) were published 12 years ago and studies evaluating the outcomes are limited. Therefore, the aim of this study was to perform a survey about criteria for CVC insertion and maintenance in intestinal failure/rehabilitation centres treating children on home PN. METHODS: An on-line cross-sectional survey based on previous European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) PN guidelines was distributed electronically to the members of the ESPGHAN networking group, Network for Intestinal Failure and Transplantation in Europe (NITE) and tertiary paediatric gastroenterology centres in Europe...
June 18, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29892152/neonatal-presentation-of-unremitting-inflammatory-bowel-disease
#2
Sara Ebrahimi, Gholamreza Khademi, Seyed Ali Jafari, Nona Zaboli Nejad, Abdolreza Norouzy, Bahareh Imani
Very-early-onset inflammatory bowel disease (VEO-IBD) has a distinct phenotype and should be considered a specific entity. VEO-IBD presents with very severe clinical pictures and is frequently known by an indeterminate colitis whose clinical remission is unmanageable. This study examines the case of a neonate with VEO-IBD, not responding to medical and surgical treatment. A 7-day-old Iranian female neonate presented with severe bloody diarrhea, poor feeding, abdominal distention, and dehydration suggesting severe proctocolitis due to an allergy to the protein in cow's milk...
May 2018: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29882922/prevention-and-treatment-of-intestinal-failure-associated-liver-disease-in-children
#3
REVIEW
Lorenzo Norsa, Emanuele Nicastro, Angelo Di Giorgio, Florence Lacaille, Lorenzo D'Antiga
Intestinal failure-associated liver disease (IFALD) is a threatening complication for children on long-term parenteral nutrition because of intestinal failure. When progressive and intractable, it may jeopardize intestinal rehabilitation and lead to combined liver and intestinal transplantation. The institution of dedicated intestinal failure centers has dramatically decreased the incidence of such complication. IFALD may rapidly fade away if very early management aimed at preventing progression to end-stage liver disease is provided...
May 24, 2018: Nutrients
https://www.readbyqxmd.com/read/29880053/indications-and-complications-of-inpatient-parenteral-nutrition-prescribed-to-children-in-a-large-tertiary-referral-hospital
#4
C Mantegazza, N Landy, G V Zuccotti, J Köglmeier
BACKGROUND: Parenteral Nutrition (PN) is prescribed to children with intestinal failure. Although life saving, complications are common. Recommendations for indications and constituents of PN are made in the 2005 guidelines by the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). The aim of this study was to establish if the indications for prescribing PN in a tertiary children's hospital were appropriate, and to identify complications encountered. Data were compared to those published by the National Confidential Enquiry into patient outcome and death (NCEPOD) carried out in the United Kingdom in 2010...
June 8, 2018: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29874104/does-malnutrition-have-a-genetic-component
#5
Priya Duggal, William A Petri
Malnutrition is a complex disorder, defined by an imbalance, excess, or deficiency of nutrient intake. The visible signs of malnutrition are stunted growth and wasting, but malnourished children are also more likely to have delays in neurocognitive development, vaccine failure, and susceptibility to infection. Despite malnutrition being a major global health problem, we do not yet understand the pathogenesis of this complex disorder. Although lack of food is a major contributor to childhood malnutrition, it is not the sole cause...
June 6, 2018: Annual Review of Genomics and Human Genetics
https://www.readbyqxmd.com/read/29778436/enhanced-central-venous-catheter-bundle-for-pediatric-parenteral-dependent-intestinal-failure
#6
Jennifer A Ormsby, Bola Bukoye, Debra Lajoie, Herminia Shermont, Lisa Martin, Kierrah Leger, Judy Mahoney, Gail Potter-Bynoe, Jane Carpenter, Al Ozonoff, Grace M Lee
BACKGROUND: Central line-associated bloodstream infections (CLABSIs) cause substantial morbidity and increase antimicrobial use and length of stay among hospitalized children in the United States. CLABSI occurs more frequently among high-risk pediatric patients, such as those with intestinal failure (IF) who are parenteral nutrition (PN) dependent. Following an increase in CLABSI rates, a quality improvement (QI) initiative was implemented. METHODS: Using QI methodology, an enhanced central venous catheter (CVC) maintenance bundle was developed and implemented on 2 units for pediatric PN-dependent patients with IF...
May 16, 2018: American Journal of Infection Control
https://www.readbyqxmd.com/read/29754463/intestinal-failure-the-evolving-demographic-and-patient-outcomes-on-home-parenteral-nutrition
#7
Sarah Kate Brown, Natalie Davies, Enda Smyth, Nicky Heather, Caroline Cole, Simon Charles Keys, Robert Mark Beattie, Akshay Batra
AIM: We performed this study to examine and understand the evolving demographics and changing outcomes of intestinal failure, and its implications for healthcare delivery. METHOD: We conducted a retrospective analysis of outcome data of children on home Parenteral Nutrition (HPN), over a 15 year period. RESULTS: 31 patients received HPN, 15 for short bowel syndrome (SBS), 8 neuromuscular disease (NMD) and 8 for other causes. The HPN prevalence increased from 1...
May 13, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29752972/urologic-phenotype-and-patterns-of-care-in-patients-with-megacystis-microcolon-intestinal-hypoperistalsis-syndrome-presenting-to-a-major-pediatric-transplantation-center
#8
Lee A Hugar, Rajeev Chaudhry, Thomas W Fuller, Glenn M Cannon, Francis X Schneck, Michael C Ost, Heidi A Stephany
OBJECTIVES: To describe the phenotype, referral pattern, and trends of urologic management for children with megacystis microcolon intestinal hypoperistalsis syndrome. METHODS: We performed a retrospective review of all patients with megacystis microcolon intestinal hypoperistalsis syndrome treated at a large tertiary children's medical center over a 14-year period. Our primary outcomes included the rate of urology referral, urodynamic characteristics of bladder function, and the proportion of patients managed with clean intermittent catheterization...
May 9, 2018: Urology
https://www.readbyqxmd.com/read/29746341/screening-endoscopy-contributes-to-relevant-modifications-of-therapeutic-regimen-in-children-with-intestinal-failure
#9
Andreas Busch, Ekkehard Sturm
OBJECTIVE: The role of endoscopy in the diagnostic work-up of children with intestinal failure (IF) is not well defined. It is unclear whether endoscopies should be performed as a screening procedure or only upon manifestation of symptoms. The aim of this study is to evaluate, whether performing screening endoscopy contributes to clinical management in children with IF. METHODS: Comparative retrospective case-series study in children with IF (mean age 70,4 months ± 58,4 months) presenting for diagnostic work-up in a single intestinal rehabilitation center...
May 9, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29745058/post-transplant-lymphoproliferative-disorder-in-pediatric-intestinal-transplant-recipients-a-literature-review
#10
REVIEW
Kaitlin Stanley, Erika Friehling, Sarangarajan Ranganathan, George Mazariegos, Linda M McAllister-Lucas, Rakesh Sindhi
Intestinal transplantation is a successful treatment for children with intestinal failure, but has many potential complications. PTLD, a clinically and histologically diverse malignancy, occurs frequently after intestinal transplantation and can be fatal. The management of this disease is particularly challenging. The rejection-prone intestinal allograft requires high levels of immunosuppression, a precondition for PTLD. While EBV infection clearly plays a role in disease pathogenesis, the relatively naïve immune system of children is another likely contributor...
May 10, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29735029/pediatric-intestinal-transplantation
#11
REVIEW
Neslihan Celik, George V Mazariegos, Kyle Soltys, Jeffrey A Rudolph, Yanjun Shi, Geoffrey J Bond, Rakesh Sindhi, Armando Ganoza
Pediatric intestinal transplantation has moved from the theoretic to an actual therapy for children with irreversible intestinal failure who are suffering from complications of total parenteral nutrition. Owing to significant advancement in the management of intestinal failure and prevention of parenteral nutrition-related complications that have led to reduction in incidence of parenteral nutrition-associated liver disease and have improved intestinal adaptation, the indications for intestinal transplantation are evolving...
June 2018: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/29706493/intravenous-fish-oil-lipid-emulsion-prevents-catheter-related-thromboses-in-pediatric-patients-with-intestinal-failure
#12
Mohammad M Jami, Vrinda Bhardwaj, Russell J Merritt
A central venous catheter is a risk factor for deep vein thrombosis. We compared the incidence of deep vein thrombosis in children with intestinal failure patients receiving soy oil lipid emulsion (n = 35) vs fish oil lipid emulsion (n = 35). Ten deep vein thrombosis occurred in the soy oil lipid emulsion group, and none in the fish oil lipid emulsion group (P < .001).
April 26, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29701873/children-with-intestinal-failure-undergoing-intestinal-rehabilitation-are-at-risk-for-essential-fatty-acid-deficiency
#13
Riikka Gunnar, Mirka Lumia, Mikko Pakarinen, Laura Merras-Salmio
BACKGROUND: Essential fatty acid (EFA) status may be compromised during the intestinal failure (IF) rehabilitation. Parenteral lipid restriction is used to treat intestinal failure associated liver disease (IFALD), while the enteral fatty acid (FA) absorption remains limited. We analyzed the FA status among pediatric IF and intestinal insufficiency patients. METHODS: We evaluated 49 patients aged 0-18 years attending our nationwide IF referral center. Their serum FA fractions were determined and examined against previous nutrition, parenteral lipid emulsion, and intestinal anatomy data...
April 27, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29701871/pediatric-intestinal-failure-the-key-outcomes-for-the-first-100-patients-treated-in-a-national-tertiary-referral-center-during-1984-2017
#14
Laura Merras-Salmio, Annika Mutanen, Elisa Ylinen, Risto Rintala, Antti Koivusalo, Mikko P Pakarinen
BACKGROUND: Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. METHODS: All children with IF who required parenteral nutrition (PN) >2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. RESULTS: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0...
April 27, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29628209/outcomes-in-patients-with-short-bowel-syndrome-after-autologous-intestinal-reconstruction-does-etiology-matter
#15
Federica Pederiva, Alberto Sgrò, Riccardo Coletta, Basem Khalil, Antonino Morabito
BACKGROUND: Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. Many factors have been investigated in an attempt to define which parameters influence most survival and ability to wean off parenteral nutrition (PN). The aim of this study was to investigate if aetiology of SBS affects the outcomes in paediatric patients treated with autologous gastrointestinal reconstructive surgery. METHODS: All children with SBS who underwent autologous gastrointestinal reconstructive surgery between 2002 and 2012 were retrospectively reviewed and outcome measures were recorded...
March 12, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29622358/arterial-reconstructions-for-pediatric-splanchnic-artery-occlusive-disease
#16
Dawn M Coleman, Jonathan L Eliason, James C Stanley
OBJECTIVE: Pediatric splanchnic arterial occlusive disease is uncommon and a rare cause of clinically relevant intestinal ischemia. This study was undertaken to better define the clinical manifestations and appropriate treatment of celiac artery (CA) and superior mesenteric artery (SMA) occlusive disease in children. METHODS: Clinical courses of 30 consecutive children undergoing operations for splanchnic arterial occlusive disease at the University of Michigan from 1992 to 2017 were retrospectively analyzed...
April 2, 2018: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/29610160/the-use-of-pyridostigmine-in-a-child-with-chronic-intestinal-pseudo-obstruction
#17
Ahamodur Choudhury, Abdul Rahyead, Jochen Kammermeier, Mohamed Mutalib
Chronic intestinal pseudo-obstruction is a rare disorder that affects the motility of the gastrointestinal tract. It results in acute or subacute intestinal obstruction symptoms in the absence of mechanical lesion. It can lead to intestinal failure in children with significant strain on nutrition, growth, and development. There is no universally agreed protocol for management of chronic intestinal pseudo-obstruction in children, and there is wide variation in clinical practice.
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29605259/gene-expression-profiling-coupled-with-connectivity-map-database-mining-reveals-potential-therapeutic-drugs-for-hirschsprung-disease
#18
Shang-Jie Xiao, Xiao-Chun Zhu, Hua Deng, Wei-Ping Zhou, Wen-Yi Yang, Li-Ke Yuan, Jiang-Yu Zhang, Song Tian, Lu Xu, Liang Zhang, Hui-Min Xia
BACKGROUND: Hirschsprung disease (HD) is a congenital intestinal anomaly resulting from a failure to form enteric ganglia in the lower bowel. Surgery is the main therapeutic strategy, although neural stem cell transplantation has recently shown promise. However, HD remains a challenging disorder to treat. Our aim was to identify drugs that could counteract the dysregulated pathways in HD and could thus be potential novel therapies. METHODS: We used microarray analysis to identify genes differentially expressed in ganglionic and aganglionic bowel samples from eight children with HD...
March 4, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29602547/ostomy-in-continuity-a-novel-approach-for-the-management-of-children-with-complex-short-bowel-syndrome
#19
Sona Sehgal, Anthony D Sandler, A Alfred Chahine, Parvati Mohan, Clarivet Torres
INTRODUCTION: Despite medical and surgical management, a subset of children with short bowel syndrome (SBS) who have discrepancy between proximal small bowel and distal colon have persistent feeding intolerance. We propose the use of an Ostomy in Continuity (OIC) (Bishop-Koop or Santulli) as a salvage procedure to decompress the proximal bowel while still maintaining maximal intestinal length, in these patients. METHODS: A retrospective chart review of 104 SBS patients identified sixteen patients who underwent an OIC...
March 4, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29550232/central-line-associated-bloodstream-infection-among-children-with-intestinal-failure-presenting-to-the-emergency-department-with-fever
#20
Matthew Eisenberg, Michael C Monuteaux, Gillian Fell, Vera Goldberg, Mark Puder, Joel Hudgins
OBJECTIVES: To determine which factors confer the greatest risk of central line-associated bloodstream infection (CLABSI) in children with intestinal failure and fever presenting to an emergency department (ED), and to assess whether a low-risk group exists that may not require the standard treatment of admission for 48 hours on intravenous antibiotics pending culture results. STUDY DESIGN: This retrospective cohort study included children with intestinal failure and fever presenting to an ED over a 6-year period...
May 2018: Journal of Pediatrics
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