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Intestinal Failure in Children

  • Olivier GouletEmail author
  • Florence Lacaille
  • Cécile Lambe
Chapter

Abstract

Intestinal failure (IF) is a condition in which severe intestinal malabsorption requires parenteral nutrition (PN). Causes of protracted intestinal failure include short bowel syndrome (SBS), congenital diseases of enterocyte development (CDED), and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). IF can result in “nutritional failure,” defined as the incapacity to continue to feed a child by using PN. Today, intestinal failure-associated liver disease (IFALD) is the most common cause of nutritional failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the use of long-term PN. For a child with nutritional failure, intestinal transplantation (ITx), often in the form of a composite visceral graft, offers the only option for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of parenteral nutrition formula rich in fish oil have both resulted in decreased rates of severe complications of IF and its treatment. In addition, improved awareness of the risks and benefits of ITx have resulted in better patient selection, and ultimately in improved patient survival, leading to restrict the indication to ITx only to patients with nutritional failure with no other chance to survive.

Keywords

Intestinal failure Children Parenteral nutrition Short bowel syndrome Congenital enteropathy Intestinal pseudo-obstruction syndrome Intestinal failure-associated liver disease Home parenteral nutrition Intestinal transplantation 

Abbreviations

AABF

Amino acid-based formulas

CRS

Catheter-related sepsis

CLD

Cholestatic liver disease

CIPOS

Chronic intestinal pseudo-obstruction syndrome

(CSD)

Congenital sodium diarrhea

CDED

Congenital diseases of enterocyte development

CTF

Continuous tube feeding

ETF

Enteral tube feeding

EGF

Epidermal growth factor

EFA

Essential fatty acid

GI

Gastrointestinal

GLP-2

Glucagon-like peptide 2

HM

Human milk

ICV

Ileocecal valve

IGF-1

Insulin-like growth factor-1

IF

Intestinal failure

IFALD

Intestinal failure-associated liver disease

ITx

Intestinal transplantation

ILE

Intravenous lipid emulsions

LOT

Ligament of Treitz

LILT

Longitudinal intestinal lengthening and tailoring

MCT

Medium-chain triglyceride

MVID

Microvillus inclusion disease

OF

Oral feeding

PN

Parenteral nutrition

PUFA

Polyunsaturated fatty acid

rhGH

Recombinant human growth hormone

STEP

Serial transverse enteroplasty technique

SBS

Short bowel syndrome

SCFA

Short-chain fatty acid

SIBO

Small intestinal bacterial overgrowth

TIA

Total intestinal aganglionosis

TE

Tufting enteropathy

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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Olivier Goulet
    • 1
    Email author
  • Florence Lacaille
    • 1
  • Cécile Lambe
    • 1
  1. 1.Division of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants MaladesUniversity Paris DescartesParisFrance

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