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Intestinal failure in adult

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https://www.readbyqxmd.com/read/28701261/five-year-survival-and-causes-of-death-in-patients-on-home-parenteral-nutrition-for-severe-chronic-and-benign-intestinal-failure
#1
Francisca Joly, Janet Baxter, Michael Staun, Darlene G Kelly, Yi Lisa Hwa, Olivier Corcos, Antonella De Francesco, Federica Agostini, Stanislaw Klek, Lidia Santarpia, Franco Contaldo, Cora Jonker, Geert Wanten, Luisa Chicharro, Rosa Burgos, Andre Van Gossum, Cristina Cuerda, Nuria Virgili, Loris Pironi
BACKGROUND & AIM: Home parenteral nutrition (HPN) is the primary treatment for chronic intestinal failure (IF). Intestinal transplantation (ITx) is indicated when there is an increased risk of death due to HPN complications or to the underlying disease. Age, pathophysiologic conditions and underlying disease are known predictors of HPN dependency and overall survival. Although the cause of death on HPN is mostly related to underlying disease in these patients, the relationship between mortality and duration of HPN use remains unclear...
June 19, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28686309/absence-of-calcitriol-causes-increased-lactational-bone-loss-and-lower-milk-calcium-but-does-not-impair-post-lactation-bone-recovery-in-cyp27b1-null-mice
#2
Brittany R Gillies, Brittany A Ryan, Brett A Tonkin, Ingrid J Poulton, Yue Ma, Beth J Kirby, René St-Arnaud, Natalie A Sims, Christopher S Kovacs
We hypothesized that adaptation to calcium supply demands of pregnancy and lactation do not require calcitriol. Adult Cyp27b1 null mice lack calcitriol and have hypocalcemia, hypophosphatemia, and rickets. We studied wild-type (WT) and null sister pairs raised on a calcium, phosphorus and lactose-enriched "rescue" diet that prevents hypocalcemia and rickets. Bone mineral content (BMC) increased >30% in pregnant nulls, declined 30% during lactation, and increased 30% by 4 weeks post-weaning. WT showed less marked changes...
July 7, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28629281/noninvasive-quantitative-assessment-of-hepatic-steatosis-by-proton-magnetic-resonance-spectroscopy-among-adult-patients-receiving-home-parenteral-nutrition
#3
Angelique Huijbers, Geert Wanten, Helena Maria Dekker, Marinette van der Graaf
BACKGROUND: Intestinal failure-associated liver disease is a frequent complication in patients with chronic intestinal failure (CIF), with steatosis as a dominant feature in adults. Proton magnetic resonance spectroscopy (1H-MRS) is a noninvasive method to quantify liver fat content (LFC). In this study, LFC was assessed with 1H-MRS, taking into account the possible accumulation of paramagnetic components of home parenteral nutrition (HPN) that may disturb these measurements. METHODS: LFC was measured in 15 adult CIF patients who had been receiving HPN for >6 months...
June 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28620494/endemic-goitre-and-hypothyroidism-in-an-adult-female-patient-dependent-on-total-parenteral-nutrition
#4
S Pearson, C Donnellan, L Turner, E Noble, K Seejore, R D Murray
We present the case of a thirty-year-old female patient who was referred to the endocrinology team with an enlarging goitre and biochemical hypothyroidism. She had been dependent on total parenteral nutrition for the previous six years as a result of intestinal failure thought to be caused by possible underlying mitochondrial disease. The patient also suffers from a Desmin myopathy, and at present, the exact aetiology behind her intestinal failure is not certain. The goitre was smooth and had been enlarging slowly over the previous few months...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28605168/characteristics-and-management-of-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura-in-adults-data-from-the-260-patients-included-in-the-igavas-survey
#5
Alexandra Audemard-Verger, Benjamin Terrier, Agnès Dechartres, Johan Chanal, Zahir Amoura, Noémie Le Gouellec, Patrice Cacoub, Noémie Jourde-Chiche, Geoffrey Urbanski, Jean-François Augusto, Guillaume Moulis, Loic Raffray, Alban Deroux, Aurélie Hummel, Bertrand Lioger, Mélanie Catroux, Stanislas Faguer, Julie Goutte, Nihal Martis, François Maurier, Etienne Rivière, Sébastien Sanges, Aurélie Baldolli, Nathalie Costedoat-Chalumeau, Mélanie Roriz, Xavier Puéchal, Marc André, Christian Lavigne, Boris Bienvenu, Arsène Mekinian, Elie Zagdoun, Charlotte Girard, Alice Bérezné, Loïc Guillevin, Eric Thervet, Evangéline Pillebout
OBJECTIVES: Data on adult IgA vasculitis (IgAV) are scarce. This survey was designed to better define clinical spectrum and efficacy of treatments in this population. METHODS: We analyzed data from 260 patients with IgAV included in a French multicenter retrospective survey. RESULTS: Mean age at diagnosis was 50.1±18 years, and 63% were male. Baseline manifestations included purpura (100%), arthralgia (62%), glomerulonephritis (70%) or gastro-intestinal involvement (53%)...
June 12, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28514243/celiac-disease-symptom-resolution-effectiveness-of-the-gluten-free-diet
#6
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/28483328/clinical-classification-of-adult-patients-with-chronic-intestinal-failure-due-to-benign-disease-an-international-multicenter-cross-sectional-survey
#7
Loris Pironi, Denise Konrad, Chrisoffer Brandt, Francisca Joly, Geert Wanten, Federica Agostini, Cecile Chambrier, Umberto Aimasso, Sarah Zeraschi, Darlene Kelly, Kinga Szczepanek, Amelia Jukes, Simona Di Caro, Miriam Theilla, Marek Kunecki, Joanne Daniels, Mireille Serlie, Florian Poullenot, Jian Wu, Sheldon C Cooper, Henrik H Rasmussen, Charlene Compher, David Seguy, Adriana Crivelli, Maria C Pagano, Sarah-Jane Hughes, Francesco W Guglielmi, Nada Rotovnik Kozjek, Stéphane M Schneider, Lyn Gillanders, Lars Ellegard, Ronan Thibault, Przemysław Matras, Anna Zmarzly, Konrad Matysiak, Andrè Van Gossum, Alastair Forbes, Nicola Wyer, Marina Taus, Nuria M Virgili, Margie O'Callaghan, Brooke Chapman, Emma Osland, Cristina Cuerda, Peter Sahin, Lynn Jones, Andre D W Lee, Valentino Bertasi, Paolo Orlandoni, Ferenc Izbéki, Corrado Spaggiari, Marta Bueno Díez, Maryana Doitchinova-Simeonova, Carmen Garde, Aurora E Serralde-Zúñiga, Gabriel Olveira, Zeljko Krznaric, Laszlo Czako, Gintautas Kekstas, Alejandro Sanz-Paris, Estrella Petrina Jáuregui, Ana Zugasti Murillo, Eszter Schafer, Jann Arends, José P Suárez-Llanos, Jon Shaffer, Simon Lal
BACKGROUND & AIMS: The aim of the study was to evaluate the applicability of the ESPEN 16-category clinical classification of chronic intestinal failure, based on patients' intravenous supplementation (IVS) requirements for energy and fluids, and to evaluate factors associated with those requirements. METHODS: ESPEN members were invited to participate through ESPEN Council representatives. Participating centers enrolled adult patients requiring home parenteral nutrition for chronic intestinal failure on March 1st 2015...
April 19, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28425133/fecal-microbiota-signatures-of-adult-patients-with-different-types-of-short-bowel-syndrome
#8
Yuhua Huang, Feilong Guo, Yousheng Li, Jian Wang, Jieshou Li
BACKGROUND AND AIM: Short bowel syndrome (SBS) is a common cause of intestinal failure and can be divided into three types depending on intestinal anatomy. Gut dysbiosis has been observed in pediatric SBS patients and is associated with impaired outcome. Little is known about the changes in gut microbiota of adult SBS patients. Therefore, we aim to characterize the fecal microbiota of adult patients with different types of SBS. METHODS: Fifteen fecal samples from healthy controls and adult patients with type II or type III SBS were collected (five in each group)...
April 19, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28391291/rejuvenation-of-mucosal-immunosenescence-by-adipose-tissue-derived-mesenchymal-stem-cells
#9
Akitoshi Tsuruhara, Kazuyoshi Aso, Daisuke Tokuhara, Junichiro Ohori, Masaki Kawabata, Yuichi Kurono, Jerry R McGhee, Kohtaro Fujihashi
Age-associated alterations in the mucosal immune system are generally termed mucosal immunosenescence. The major change seen in the aged mucosa is a failure to elicit an antigen-specific secretory IgA (SIgA) antibody response, which is a central player for host defense from various pathogens at mucosal surfaces. In this regard, it would be a first priority to compensate for mucosal dysregulation in the elderly in order to maintain their health in aging. We have successfully established antigen-specific SIgA antibody responses in aged (2 years old) mice, which provide protective immunity from Streptococcus pneumoniae and influenza virus infections, by using a new adjuvant system consisting of a plasmid encoding Flt3 ligand (pFL) and CpG ODN...
January 1, 2017: International Immunology
https://www.readbyqxmd.com/read/28295657/severe-acute-cellular-rejection-after-intestinal-transplantation-is-associated-with-poor-patient-and-graft-survival
#10
Genevieve Huard, Thomas D Schiano, Jang Moon, Kishore Iyer
BACKGROUND: Severe acute cellular rejection (ACR) occurs frequently after intestinal transplantation (ITx). AIM: To evaluate the outcomes and the risk factors for graft failure and mortality in patients with severe ACR after ITx. METHODS: Retrospective study evaluating all ITx recipients who developed severe ACR between 01/2000 and 07/2014. Demographic and histologic data were reviewed. RESULTS: 20/126 (15.9%) ITx recipients developed severe ACR...
March 10, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28293969/teduglutide-for-treatment-of-adult-patients-with-short-bowel-syndrome
#11
Lore Billiauws, Julie Bataille, Vanessa Boehm, Olivier Corcos, Francisca Joly
The European Society for Clinical Nutrition has published recommendations on the 'definition and classification of intestinal failure (IF)'. Two criteria must be present: a 'decreased absorption of macronutrients and/or water and electrolytes due to a loss of gut function' and the 'need for parenteral support'. Home parenteral support (HPS) is the primary treatment for chronic IF but is associated with complications. Areas covered: The principal cause of chronic IF is short bowel syndrome (SBS). The aim of treatment is to maximize intestinal absorption and reduce or eliminate the need for HPS to achieve the best possible quality of life...
May 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28270450/ssb1-and-ssb2-cooperate-to-regulate-mouse-hematopoietic-stem-and-progenitor-cells-by-resolving-replicative-stress
#12
Wei Shi, Therese Vu, Didier Boucher, Anna Biernacka, Jules Nde, Raj K Pandita, Jasmin Straube, Glen M Boyle, Fares Al-Ejeh, Purba Nag, Jessie Jeffery, Janelle L Harris, Amanda L Bain, Marta Grzelak, Magdalena Skrzypczak, Abhishek Mitra, Norbert Dojer, Nicola Crosetto, Nicole Cloonan, Olivier J Becherel, John Finnie, Jeffrey R Skaar, Carl R Walkley, Tej K Pandita, Maga Rowicka, Krzysztof Ginalski, Steven W Lane, Kum Kum Khanna
Hematopoietic stem and progenitor cells (HSPCs) are vulnerable to endogenous damage and defects in DNA repair can limit their function. The 2 single-stranded DNA (ssDNA) binding proteins SSB1 and SSB2 are crucial regulators of the DNA damage response; however, their overlapping roles during normal physiology are incompletely understood. We generated mice in which both Ssb1 and Ssb2 were constitutively or conditionally deleted. Constitutive Ssb1/Ssb2 double knockout (DKO) caused early embryonic lethality, whereas conditional Ssb1/Ssb2 double knockout (cDKO) in adult mice resulted in acute lethality due to bone marrow failure and intestinal atrophy featuring stem and progenitor cell depletion, a phenotype unexpected from the previously reported single knockout models of Ssb1 or Ssb2 Mechanistically, cDKO HSPCs showed altered replication fork dynamics, massive accumulation of DNA damage, genome-wide double-strand breaks enriched at Ssb-binding regions and CpG islands, together with the accumulation of R-loops and cytosolic ssDNA...
May 4, 2017: Blood
https://www.readbyqxmd.com/read/28241392/the-high-incidence-of-severe-chronic-kidney-disease-after-intestinal-transplantation-and-its-impact-on-patient-and-graft-survival
#13
Geneviève Huard, Kishore Iyer, Jang Moon, John T Doucette, Vinay Nair, Thomas D Schiano
INTRODUCTION: Using data from the Scientific Registry of Transplant Recipients (SRTR), cumulative incidence, risk factors for, and impact on survival of severe chronic kidney disease (CKD) in intestinal transplantation (ITx) recipients were assessed. METHODS: First-time adult ITx recipients transplanted in the United States between January 1, 1990 and December 31, 2012 were included. Severe CKD after ITx was defined as: glomerular filtration rate (GFR) <30 mL/min/1...
May 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28209419/a-safety-and-pharmacokinetic-dosing-study-of-glucagon-like-peptide-2-in-infants-with-intestinal-failure
#14
David L Sigalet, Mary E Brindle, Dana Boctor, Bryan Dicken, Viona Lam, Lily Sia Lu, Elaine de Heuvel, Bolette Hartmann, Jens J Holst
BACKGROUND & AIMS: Glucagon-like peptide 2 (GLP-2) analogues are approved for adults with intestinal failure (IF), but no studies have included infants. This study examined the pharmacokinetics (PK), safety, and nutritional effects of GLP-2 in infants with IF. METHODS: With parental consent (Health Canada Protocol:150,979), parenteral nutrition (PN)-dependent infants were treated with 5-20-μg/kg/day GLP-2 for 3days (phase 1), and if tolerated continued for 42days (phase 2)...
May 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28194723/teduglutide-a-review-in-short-bowel-syndrome
#15
REVIEW
Esther S Kim, Susan J Keam
Subcutaneous teduglutide (Revestive(®)), a glucagon-like peptide-2 analogue that increases intestinal absorption, is approved in the EU for the treatment of short bowel syndrome (SBS) in patients aged ≥1 year who are stable following a period of postsurgical intestinal adaptation. In a phase III trial in adults with SBS intestinal failure (IF) dependent on parenteral support (PS), a significantly greater proportion of teduglutide 0.05 mg/kg/day than placebo recipients achieved a ≥20% reduction in weekly PS volume from baseline to week 20 and maintained it to week 24...
March 2017: Drugs
https://www.readbyqxmd.com/read/28191783/concise-review-the-potential-use-of-intestinal-stem-cells-to-treat-patients-with-intestinal-failure
#16
Sung Noh Hong, James C Y Dunn, Matthias Stelzner, Martín G Martín
Intestinal failure is a rare life-threatening condition that results in the inability to maintain normal growth and hydration status by enteral nutrition alone. Although parenteral nutrition and whole organ allogeneic transplantation have improved the survival of these patients, current therapies are associated with a high risk for morbidity and mortality. Development of methods to propagate adult human intestinal stem cells (ISCs) and pluripotent stem cells raises the possibility of using stem cell-based therapy for patients with monogenic and polygenic forms of intestinal failure...
February 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28081385/catheter-related-bloodstream-infections-in-adults-receiving-home-parenteral-nutrition
#17
Siri Tribler, Christopher F Brandt, Mark Hvistendahl, Michael Staun, Per Brøbech, Claus E Moser, Palle B Jeppesen
BACKGROUND: A common complication in patients receiving home parenteral nutrition (HPN) is catheter-related bloodstream infections (CRBSIs). The CRBSI incidence has been advocated as an outcome parameter assessing the quality of care. This study aimed to illustrate how the use of different CRBSI definitions affects the reported incidence. MATERIALS AND METHODS: In an observational study based on the Copenhagen intestinal failure database, all clinically reported CRBSIs from 2002-2013 were compared with data from the affiliated microbiological database according to recommended CRBSI criteria...
January 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28052602/optn-srtr-2015-annual-data-report-intestine
#18
J M Smith, M A Skeans, S P Horslen, E B Edwards, A M Harper, J J Snyder, A K Israni, B L Kasiske
Intestine and intestine-liver transplant remains important in the treatment of intestinal failure, despite decreased morbidity associated with parenteral nutrition. In 2015, 196 new patients were added to the intestine transplant waiting list, with equal numbers waiting for intestine and intestine-liver transplant. Among prevalent patients on the list at the end of 2015, 63.3% were waiting for an intestine transplant and 36.7% were waiting for an intestine-liver transplant. The pretransplant mortality rate decreased dramatically over time for all age groups...
January 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28027797/corrigendum-to-espen-guidelines-on-chronic-intestinal-failure-in-adults-clin-nutr-35-2-2016-247-307
#19
Loris Pironi, Jann Arends, Federico Bozzetti, Cristina Cuerda, Lyn Gillanders, Palle Bekker Jeppesen, Francisca Joly, Darlene Kelly, Simon Lal, Michael Staun, Kinga Szczepanek, André Van Gossum, Geert Wanten, Stéphane Michel Schneider
No abstract text is available yet for this article.
April 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27979490/is-om-3-synergistic-with-glp-2-in-intestinal-failure
#20
Avik Karmaker, Caitlyn M Costanzo, Marshall Z Schwartz
INTRODUCTION: Glucagon-like peptide-2 (GLP-2) is a known intestinal growth factor that enhances mucosal mass and function in residual small intestine after massive small bowel resection (MSBR). Luminal omega-3 (OM-3) has been shown to have some growth factor properties. It is possible that their mechanisms of action differ. Thus, we hypothesized that administering these two substances together may have a synergistic effect. METHODS: A total of 60 adult female Sprague-Dawley rats underwent 80% MSBR and divided as follows (n = 15/group): Saline (Control) + regular feeds; GLP-2 + regular feeds; Saline + OM-3 enriched feeds; and GLP-2 + OM-3 enriched feeds...
January 2017: Journal of Surgical Research
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