Enteropatía congénita y trasplante intestinal

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Abstract

La insuficiencia intestinal (II) exige el uso de nutrición parenteral (NP). Entre las causas de II prolongada grave destacan el síndrome del intestino corto, trastornos de la motilidad grave (aganglionosis total o subtotal o síndrome de seudoobstrucción intestinal crónica) y enfermedades congénitas del desarrollo de los enterocitos. Puede aparecer insuficiencia hepática grave en pacientes con II como consecuencia de una enfermedad digestiva subyacente y una NP no adaptada. La sepsis relacionada con el catéter y/o la trombosis vascular extensa pueden impedir el mantenimiento de una NP segura y eficiente. Por tanto, el tratamiento de pacientes con II exige la identificación precoz del proceso y el análisis de su riego de irreversibilidad. La cronología de la derivación para trasplante intestinal continúa siendo una cuestión crucial. En consecuencia, la asistencia debe incluir tratamientos adaptados a cada una de las etapas de la II, basándose en la puesta en práctica de un enfoque multidisciplinario en centros que cuenten con gastroenterología pediátrica, experiencia en NP, programa de NP domiciliaria, cirugía pediátrica y programa de trasplantes de hígado e intestino.

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Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans.

C Coudray, L Cynober, P Crenn … (2000)

No blood marker assessing the functional absorptive bowel length has been identified. Plasma citrulline, a nonprotein amino acid produced by intestinal mucosa, is one candidate. We tested this hypothesis in adult patients with the short-bowel syndrome, whose condition can lead to intestinal failure. In 57 patients, after a minimal follow-up of 2 years subsequent to final digestive circuit modification, postabsorptive citrulline concentration was measured and parenteral nutrition dependence was used to define permanent (n = 37) and transient (n = 20) intestinal failure. Absorptive function, studied over a 3-day period, was evaluated by net digestive absorption for protein and fat (n = 51). Relations between quantitative values were assessed by linear regression analysis and cutoff citrulline threshold, for a diagnosis of intestinal failure by linear discriminant analysis. Cox model was used to compare citrulline threshold and anatomic variables of the short bowel as indicators of transient as opposed to permanent intestinal failure. In patients with short-bowel syndrome, citrulline levels were lower than in controls (n = 51): 20 +/- 13 vs. 40 +/- 10 micromol/L (mean +/- SD), respectively (P < 0.001). After multivariate analysis, citrullinemia was correlated to small bowel length (P < 0.0001, r = 0.86) and to net digestive absorption of fat, but to neither body mass index nor creatinine clearance. A 20-micromol/L threshold citrullinemia, (1) classified short bowel patients with permanent intestinal failure with high sensitivity (92%), specificity (90%), positive predictive value (95%), and negative value (86%); and (2) was a more reliable indicator (odds ratio, 20.0; 95% confidence interval, 1.9-206.1) than anatomic variables (odds ratio, 2.9; 95% confidence interval, 0. 5-15.8) to separate transient as opposed to permanent intestinal failure. In patients with short-bowel syndrome, postabsorptive plasma citrulline concentration is a marker of functional absorptive bowel length and, past the 2-year adaptive period, a powerful independent indicator allowing distinction of transient from permanent intestinal failure.

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Irreversible intestinal failure.

Olivier Goulet, Florence Lacaille, Virginie Colomb-Jung … (2004)

Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.

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Plasma citrulline: A marker of enterocyte mass in villous atrophy-associated small bowel disease.

Pascal Crenn, Kouroche Vahedi, Anne Lavergne-Slove … (2003)

Plasma citrulline, a nonprotein amino acid produced by enterocytes, was suggested as a marker of remnant enterocyte mass in patients with short bowel. Our objective was to evaluate citrulline as a marker of severity and extent of villous atrophy in patients without intestinal resection. Forty-two patients with celiac disease and 10 patients with non-celiac villous atrophy disease were studied by plasma postabsorptive citrulline and biological dosages, biopsies of proximal (duodenojejunal) small bowel and distal ileum (n = 25), or measurement of vitamin B(12) absorption (n = 4). Nine patients were reevaluated after following a gluten-free diet for 1 year. Controls were 51 healthy subjects and 10 severely malnourished patients with anorexia nervosa with no intestinal mucosal abnormalities. Plasma citrulline concentration was lower (P < 0.001) in patients with villous atrophy (24 +/- 13 micromol/L) than in healthy subjects (40 +/- 10 micromol/L) and patients with anorexia nervosa (39 +/- 9 micromol/L). Three thresholds were individualized: <10 micromol/L for patients with diffuse total villous atrophy (n = 10), 10-20 micromol/L for patients with proximal-only total villous atrophy (n = 12), and 20-30 micromol/L for patients with partial villous atrophy (n = 10). Plasma citrulline concentration was correlated to the severity and extent of villous atrophy (r = 0.81; P < 0.001) and to albuminemia (r = 0.47; P < 0.01). Receiver operating characteristic curves indicated that plasma citrulline concentration was the best biological variable to predict villous atrophy. Following a 1-year gluten-free diet, plasma citrulline concentration increased in histologically responsive (n = 6) but not in unresponsive (n = 3) patients. In patient villous atrophy diseases, plasma citrulline concentration may prove to be a simple and reliable marker of reduced enterocyte mass.

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Journal

Journal ID (publisher-id): ANS

Journal ID (doi): 10.1159/issn.0252-8185

Title: Annales Nestlé (Ed. española)

Publisher: S. Karger AG

ISBN: 978-3-8055-8099-1

ISBN: 978-3-318-01333-7

ISSN (Print): 0252-8185

ISSN (Electronic): 1661-4003

Publication date Subscription-year: 2006

Publication date (Print): April 2006

Publication date (Electronic): 26 May 2006

Volume: 64

Issue: 1

Pages: 25-37

Affiliations

Centro de Referencia de Enfermedades Digestivas Raras; Departamento de Gastroenterología-Hepatología Pediátrica y Nutrición, Hôpital Necker-Enfants Malades, París, Francia

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Publisher ID: 91006 Other ID: Ann Nestlé [Spa] 2006;64:25–37

DOI: 10.1159/000091006

SO-VID: 936a6449-ff5a-4180-96e6-9a2af7525ee1

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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Enteropatía congénita y trasplante intestinal

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Karger: Nutrition and Dietetics

Most cited references 60

Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans.

Irreversible intestinal failure.

Plasma citrulline: A marker of enterocyte mass in villous atrophy-associated small bowel disease.

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