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Nutritional selection pediatric liver disease

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
Brenna S Fullerton, Eric A Sparks, Amber M Hall, Christopher Duggan, Tom Jaksic, Biren P Modi
PURPOSE: Patient selection for transplant evaluation in pediatric intestinal failure is predicated on the ability to assess long-term transplant-free survival. In light of trends toward improved survival of intestinal failure patients in recent decades, we sought to determine if the presence of biopsy-proven hepatic cirrhosis or the eventual achievement of enteral autonomy were associated with transplant-free survival. METHODS: After IRB approval, records of all pediatric intestinal failure patients (parenteral nutrition (PN) >90 days) treated at a single intestinal failure center from February 2002 to September 2014 were reviewed...
January 2016: Journal of Pediatric Surgery
Mikko P Pakarinen
Pediatric intestinal failure (IF) remains to be associated with significant morbidity and mortality, the most frequent underlying etiologies being short bowel syndrome (SBS), and primary motility disorders. Management aims to assure growth and development, while preventing complications and facilitating weaning off parenteral support (PS) by fully utilizing adaptation potential of the remaining gut. Probability of survival and weaning off PS is improved by coordinated multidisciplinary intestinal rehabilitation combining individualized physiological enteral and parenteral nutrition (PN), meticulous central line care and medical management with carefully planned surgical care...
May 2015: Pediatric Surgery International
S S Kaufman, C S Matsumoto
Intestinal failure (IF) is defined as the state of the intestinal tract where the function is below the minimum required for the absorption of macronutrients, water, and electrolytes. The etiology may be a multitude of causes, but short bowel syndrome (SBS) remains the most common. The successful management and prognosis of SBS in infants and children depends a multitude of variables such as length, quality, location, and anatomy of the remaining intestine. Prognosis, likewise, depends on these factors, but also is dependent on the clinical management of these patients...
August 2015: Minerva Pediatrica
Faraz A Khan, Jeremy G Fisher, Eric A Sparks, Alexis Potemkin, Christopher Duggan, Bram P Raphael, Biren P Modi, Tom Jaksic
OBJECTIVES: Intestinal failure-associated liver disease (IFALD) contributes to significant morbidity in pediatric patients with intestinal failure (IF); however, the use of parenteral nutrition (PN) with a fish oil-based intravenous (IV) emulsion (FO) has been associated with biochemical reversal of cholestasis and improved outcomes. Unfortunately, FO increases the complexity of care: because it can be administered only under Food and Drug Administration compassionate use protocols requiring special monitoring, it is not available as a 3-in-1 solution and is more expensive than comparable soy-based IV lipid emulsion (SO)...
March 2015: Journal of Pediatric Gastroenterology and Nutrition
Edith A Nutescu, Janet P Engle, Sacheeta Bathija, Shellee A Grim, Juliana Chan, Jeffrey J Mucksavage, Kirsten H Ohler, Eljim P Tesoro, James J Thielke, Nancy L Shapiro, Andrew J Donnelly, John Garofalo, Robert J DiDomenico
STUDY OBJECTIVES: To characterize the balance of clinical and academic responsibilities of clinical track pharmacy faculty in the United States and evaluate organizational structures that promote satisfactory balance between these responsibilities. DESIGN: Prospective cross-sectional survey. SETTING: A 22-item online survey was developed and distributed via Qualtrics software. PARTICIPANTS: Clinical faculty members of the American College of Clinical Pharmacy Adult Medicine, Ambulatory Care, Cardiology, Critical Care, Gastrointestinal/Liver/Nutrition, Immunology/Transplantation, Infectious Disease, and Pediatrics Practice and Research Networks (PRNs) were invited to participate via the PRN electronic mailing list...
December 2014: Pharmacotherapy
Juan J Díaz, Kathleen M Gura, Juliamna Roda, Antonio R Perez-Atayde, Christopher Duggan, Tom Jaksic, Clifford W Lo
BACKGROUND AND OBJECTIVES: Patients with intestinal failure (IF) require parenteral nutrition (PN) support to obtain enough nutrients to sustain growth; long-term PN use is associated with significant liver damage. The aim of this study was to analyze the use of a noninvasive test, the aspartate aminotransferase to platelet ratio index (APRI), in the diagnosis of liver disease in pediatric patients with IF. METHODS: Medical records of all Boston Children's Hospital patients who received PN and underwent a liver biopsy from January 2006 until November 2010 were reviewed...
September 2013: Journal of Pediatric Gastroenterology and Nutrition
Michael B Krawinkel, Dietmar Scholz, Andreas Busch, Martina Kohl, Lukas M Wessel, Klaus-Peter Zimmer
BACKGROUND: Chronic intestinal failure (CIF) in childhood is caused by congenital malformations and inflammatory diseases of the gut. Its reported prevalence is 13.7 per million population. Long-term home parenteral nutrition has dramatically improved the life expectancy and quality of life of children with CIF. The affected children are now treated with parenteral nutrition at home as soon as their medical state and family circumstances allow. METHODS: The authors present data from a patient registry and review publications retrieved by a selective literature search...
June 2012: Deutsches Ärzteblatt International
J K Dowman, D Watson, S Loganathan, B K Gunson, J Hodson, D F Mirza, J Clarke, C Lloyd, D Honeybourne, J L Whitehouse, E F Nash, D Kelly, I van Mourik, P N Newsome
Early liver transplant (LT) has been advocated for patients with cystic fibrosis liver disease (CFLD) and evidence of deterioration in nutritional state and respiratory function to prevent further decline. However, the impact of single LT on long-term respiratory function and nutritional status has not been adequately addressed. We performed a retrospective analysis of the outcomes of 40 (21 adult/19 pediatric) patients with CFLD transplanted between 1987 and 2009 with median follow-up of 47.8 months (range 4-180)...
April 2012: American Journal of Transplantation
Fabio Panetta, Valerio Nobili, Maria Rita Sartorelli, Raffaele Edo Papa, Francesca Ferretti, Arianna Alterio, Antonella Diamanti
Celiac disease (CD) is defined as a permanent intolerance to ingested wheat gliadins and other cereal prolamins, occurring in genetically susceptible people. Persistent elevation of serum aminotransferase activity is expression of liver damage related to CD, which occurs in two distinctive forms. The most frequent is a mild asymptomatic liver injury, with a moderate increase of serum aminotransferase activities and a mild inflammatory portal and lobular infiltrate on liver biopsy (celiac hepatitis), reversible on a gluten-free diet (GFD)...
February 1, 2012: Paediatric Drugs
Mortada H F El-Shabrawi, Naglaa M Kamal
Treatment of the causes of many chronic liver diseases (CLDs) may not be possible. In this case, complications must be anticipated, prevented or at least controlled by the best available therapeutic modalities. There are three main goals for the management of portal hypertension: (i) prevention of the first episode of variceal bleeding largely by non-selective β-adrenoceptor antagonists, which is not generally recommended in children; (ii) control of bleeding by using a stepwise approach from the least to most invasive strategies; (iii) and prevention of re-bleeding using bypass operations, with particular enthusiasm for the use of meso-Rex bypass in the pediatric population...
December 1, 2011: Paediatric Drugs
Ivo G Tzvetanov, Jose Oberholzer, Enrico Benedetti
PURPOSE OF REVIEW: To analyze the current status of living donor intestinal transplantation (LDIT) as a treatment option for intestinal failure. RECENT FINDINGS: Long-term outcomes from LDIT and combined living donor intestinal/liver transplantation (CLDILT) are comparable with those from transplantation using deceased donors. In certain life-threatening situations, especially in pediatric patients, this strategy may offer potential advantages. SUMMARY: According United Network for Organ Sharing (UNOS) data children with intestinal failure affected by liver disease secondary to parenteral nutrition have the highest mortality on a waiting list compared with all candidates for solid organ transplantation...
June 2010: Current Opinion in Organ Transplantation
George V Mazariegos, Kyle Soltys, Geoffrey Bond, Alin Girnita, Zurab Machaidze, Ronald Jaffe, Michael Green, Dolly Martin, Adriana Zeevi, Robert Squires, Graciela Perez, Kareem Abu-Elmagd, Rakesh Sindhi
BACKGROUND: Intestinal retransplantation (Re-ITx) has historically been associated with high morbidity and mortality. METHODS: The outcomes of all children receiving Re-ITx between 1990 and 2007 at our center were reviewed. RESULTS: One hundred seventy-two children received primary intestinal grafts. Fourteen children (8.1%) were retransplanted with 15 grafts. Causes of graft failure were acute cellular rejection (ACR, n=4), liver failure (n=2), chronic rejection (n=3), posttransplant lymphoproliferative disorder (n=1), graft dysmotility or dysfunction (n=3), ACR with severe infection (n=1), and arterial graft aneurysm (n=1)...
December 27, 2008: Transplantation
Valerio Nobili, Melania Manco, Paolo Ciampalini, Anna Alisi, Rita Devito, Elisabetta Bugianesi, Matilde Marcellini, Giulio Marchesini
BACKGROUND: There is no consensus on the treatment of pediatric nonalcoholic fatty liver disease (NAFLD). However, in a small pilot study conducted in 10 children, metformin has been proposed to be effective. OBJECTIVE: We aimed to determine the effect of metformin in addition to lifestyle intervention/modification in children with NAFLD. METHODS: Overweight or obese children aged 9 to 18 years with biopsy-proven NAFLD or nonalcoholic steatohepatitis were enrolled in an observational pilot study, initially planned for 12 months, which aimed to estimate the effect of metformin on liver enzymes...
June 2008: Clinical Therapeutics
Loris Pironi, Alastair Forbes, Francisca Joly, Virginie Colomb, Malgorzata Lyszkowska, André Van Gossum, Janet Baxter, Paul Thul, Xavier Hébuterne, Manuela Gambarara, Frederic Gottrand, José M Moreno Villares, Bernard Messing, Olivier Goulet, Michael Staun
BACKGROUND & AIMS: The US Medicare indications for intestinal transplantation are based on failure of home parenteral nutrition. The American Society of Transplantation also includes patients at high risk of death from their primary disease or with high morbidity intestinal failure. A 3-year prospective study evaluated the appropriateness of these indications. METHODS: Survival on home parenteral nutrition or after transplantation was analyzed in 153 (97 adult, 56 pediatric) candidates for transplantation and 320 (262 adult, 58 pediatric) noncandidates, enrolled through a European multicenter cross-sectional survey performed in 2004...
July 2008: Gastroenterology
Neal R Barshes, Beth A Carter, Saul J Karpen, Christine A O'Mahony, John A Goss
BACKGROUND: Mild liver dysfunction is common after prolonged use of parenteral nutrition (PN), but end-stage liver failure occurs only rarely. Few treatment options other than combined liver-intestine transplantation exist for patients with liver failure associated with PN use, however. Herein, we report the results of a cohort of patients undergoing isolated orthotopic liver transplantation (OLT) for PN-associated liver injury. METHODS: A retrospective cohort study of 80 patients (73 pediatric patients and 7 adults) who have undergone isolated OLT for PN-associated liver injury as the primary indication for transplantation was performed...
November 2006: JPEN. Journal of Parenteral and Enteral Nutrition
Loris Pironi, Xavier Hébuterne, Andre Van Gossum, Bernard Messing, Malgorzata Lyszkowska, Virginie Colomb, Alastair Forbes, Ann Micklewright, Jose Manuel Moreno Villares, Paul Thul, Federico Bozzetti, Olivier Goulet, Michael Staun
OBJECTIVES: Epidemiology of candidacy for intestinal transplantation (ITx) and timing for referral for ITx are unknown. Patient candidacy and physician attitudes toward ITx were investigated among centers that participated in previous European surveys on home parenteral nutrition (HPN). METHODS: Patients on HPN for benign intestinal failure (IF) were evaluated by a structured questionnaire. Candidacy was assessed by USA Medicare and American Transplantation Society criteria, categorized as: (1) life-threatening HPN complications; (2) high risk of death because of the gastrointestinal disease; (3) IF with high morbidity or patient HPN refusal...
July 2006: American Journal of Gastroenterology
Olivier Goulet, Frédérique Sauvat
PURPOSE OF REVIEW: This review summarizes recent knowledge and clinical practice for pediatric patients suffering extensive intestinal resection causing short bowel syndrome. This condition requires the use of parenteral nutrition, as long as intestinal failure persists, and may be, in some selected cases, an indication for intestinal transplantation. RECENT FINDINGS: Biological evaluation of intestinal failure is becoming possible with the use of plasma citrulline as a marker of intestinal mass...
May 2006: Current Opinion in Clinical Nutrition and Metabolic Care
Olivier Goulet, Frank Ruemmele
Intestinal failure is a condition requiring the use of parenteral nutrition as long as it persists. Causes of severe protracted intestinal failure include short bowel syndrome, congenital diseases of enterocyte development, and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). Intestinal failure may be irreversible in some patients, thus requiring permanent parenteral nutrition. Liver disease may develop with subsequent end-stage liver cirrhosis in patients with intestinal failure as a consequence of both underlying digestive disease and unadapted parenteral nutrition...
February 2006: Gastroenterology
Thomas M Fishbein, Cal S Matsumoto
Current treatment options for patients suffering from intestinal insufficiency include all forms of intestinal replacement therapy (IRT). Parenteral nutrition has achieved extended success for the majority of patients requiring interval treatment, however, complications leading to failure of this treatment increases with the duration of therapy. There is currently no consensus as to the appropriate timing for transplantation of the intestine or the timing of referral for evaluation at a center experienced with this therapy...
February 2006: Gastroenterology
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