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DOI 10.1007/s12098-016-2219-7
REVIEW ARTICLE
Received: 21 October 2015 / Accepted: 11 August 2016 / Published online: 5 October 2016
# Dr. K C Chaudhuri Foundation 2016
Introduction
The intestine is an organ that has the potential to recover its
function from major insults in the majority of cases. The discovery of this potential has come about due to the advent of
parenteral nutrition (PN). In addition, development in surgical
* Girish Gupte
girish.gupte@bch.nhs.uk
1
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Malrotation
Volvulus
Necrotising enterocolitis (NEC)
Congenital SBS
Small bowel atresia
Gastroschisis
Long segment Hirschsprungs disease
Enteropathies
&
&
&
&
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Evidence exists in the literature that supports a multidisciplinary team in managing children with intestinal failure (IF)
whilst in the hospital and supporting families discharged on
home PN [6, 7]. Improved outcomes such as reduced rates of
catheter-related sepsis and lower mortality might be attributed
to more and more units with NST, practicing evidence based
medicine in the area of clinical nutrition. Catheter management and preventing loss of venous access are especially
Common symptoms in children with intestinal failure, their investigations and management
Symptom
Possible causes
Investigations
Possible findings
Possible management
Vomiting
Obstruction
Dilated bowel
Surgery
Vomiting
Bacterial overgrowth
Vomiting
Foregut dysmotility
Plain X-ray
Contrast studies
Serum D-lactate +
blood gas
Contrast studies
Manometry
Diarrhea
Malabsorption
Diarrhea
Ulceration
Endoscopy
Diarrhea
Stricture
Growth failure
Plain X-ray
Contrast studies
Urinary sodium
Growth failure
Micronutrient deficiencies
Jaundice
IFALD
Hematochezia
Ulcerations
Allergic colitis
Cobalamin/
Iron deficiency
Tiredness
Antibiotics
Surgery
Surgery
Erosions
Eosinophils
Low vitamin B12, iron
and ferritin
important in cases with prolonged or permanent intestinal failure. This is important as there are limited sites of venous
access for the children on long term parenteral nutrition.
Although the composition of the NST varies but they generally are composed of a medical practitioner (e.g., gastroenterologist with interest in clinical nutrition), nurse practitioner,
dietitian, pharmacist and a social worker/psychologist.
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not appear for sometime and so careful review of PN prescriptions is required. Iron deficiency is seen commonly in children
with IF. Many factors contribute to this: frequent blood letting
for blood tests, poor enteral absorption and incompatibility
preventing sufficient provision in PN. Separate parenteral iron
might be required (e.g., iron sucrose) but care must be taken as
frequent use might cause central venous catheters blockage.
Parenteral Nutrition
Intestinal Adaptation
PN is the mainstay therapy for children with intestinal failure.
PN provides fluid and nutrition whilst allowing the intestine
time to recover from surgery and for the adaptive response
(see below). This therapy has become widely available and
increasingly used, but remains a complex and specialised area.
Long-term PN can be associated with life-threatening
complications:
a) Catheter-related sepsis
b) Venous thromboembolism resulting in venous occlusion
c) Intestinal failure associated liver disease (IFALD)
PN should provide balanced combination of macronutrients
(carbohydrate, amino acids and lipids) and micronutrients (vitamins and trace elements). As requirements vary throughout
childhood, regular and detailed review of PN prescriptions to
tailor the PN to individual requirements with the NST is mandatory. This involves close monitoring of biochemical and nutritional parameters. Fluid and electrolyte imbalance is common
in SBS, particularly GI water and salt losses in patients with a
high jejunostomy for example. Assessment of urinary and stool
losses of sodium and potassium can help to guide managing
electrolytes in the prescription. If these are not accounted for in
the PN, then growth is unlikely to be supported, even if sufficient calories are provided e.g., renal conservation of sodium
and/or hydrogen ions may lead to low serum potassium, unresponsive to potassium supplementation.
Intestinal failure associated renal disease is an another complication although very rarely mentioned. Altered calcium,
phosphate and vitamin D metabolism due to changes in renal
tubular function can lead to nephrocalcinosis. Acid and base
metabolism can also be affected and so changes to chloride
and acetate content of the PN may be required.
Specific diagnoses as well as sepsis and post-operative
stress may result in reduced metabolic capacity for lipid oxidation and may be seen as hypertriglyceridemia. In these circumstances, reduction or change in lipid emulsion have been
advocated but care must be taken to avoid essential fatty acid
deficiency when lipid is completely ceased.
Micronutrient deficiency might arise when there are increased losses (e.g., zinc in high ileostomy output) or absence
of anatomical absorption site (e.g., vitamin B12 with terminal
ileal resection). Clinical signs of micronutrient deficiency might
Adaptation is the process in which there is intestinal architectural and functional changes post bowel resection to restore
normal function. Although there are some adult histological
evidences [8], little pediatric histological evidence is available
and most of the evidence has been from animal studies [9].
Even so, functional adaptation is seen in clinical practice when
children are weaned off parenteral nutrition. Hormonal factors
such as growth hormone, insulin like growth factor and glucagon like peptide 2, to name a few, have been shown to be
important in mediating this process. The preservation of the
terminal ileum, where stimulation of the production of these
factors is most potent, correlates with the clinical observation
of better adaptive responses [10].
The adaptive response must be stimulated by mucosal contact with enteral nutrition. There has been no consensus on the
type of enteral nutrition that would be beneficial for adaptation
[11] but the general strategy, in authors unit, is to use expressed
breast milk where available. When it is not available or when
malabsorption is demonstrated (particularly fat malabsorption),
a hydrolysed formula with a higher medium chain triglyceride
(MCT) content is used. Rarely would an elemental formula be
used due to its high osmotic content which can have an unintended effect to cause osmotic diarrhea.
Bolus feeding has been advocated as the most physiological way to deliver enteral nutrition. Vomiting related to
dysmotility and/or diarrhea caused by large osmotic load in
a shorten bowel may be a barrier to its success. A combination
of bolus feeding and continuous feeding might be an alternative to promote physiological responses (and if able to tolerate
oral feeding, also to support oromotor skills) whilst
maximising mucosal contact with enteral nutrition without a
large osmotic effect.
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Gastric Hypersecretion
H2 receptor antagonist (e.g., ranitidine); Proton pump inhibitors (e.g., omeprazole).
The use of antacid has been particularly pushed in the immediate post operative phase. Its use during this phase has mostly
been based on the understanding of a hypersecretory phase post
intestinal surgery. This phase can last up to a month. Generally
speaking, if there is no effect on gastric output then they should
be stopped as suppression of gastric acid is thought to encourage
bacterial growth in the proximal gastrointestinal tract [13].
Foregut Dysmotility
Prokinetic agents: Erythromycin (1-3 mg/kg, po/iv tds);
Domperidone (100400 microgram/kg dependent on age po
*cautionary note below).
Erythromycin at low doses has been used as a prokinetic
agent. There is variable success with it and it should be used
on a trial basis and discontinued if no response is seen after a
reasonable period of time.
There has been a practice to use domperidone especially in
children with vomiting with variable success. Its use has now
been discouraged as there is an increased risk of prolonged QT
syndrome. The use of domperidone is not the usual practice in
authors unit in intestinal failure patients with vomiting.
Secretory Diarrhea
Octreotide 110 microgram/kg/day iv divided bd initially.
Convert to s/c if successful in symptom control.
Octreotide is a somatostatin analogue and reduces gastrointestinal motility by splanchnic vasoconstriction. It is often
used in its intravenous form with its initial dose being 1 mcg/
kg/h, increasing it to 10 mcg/kg/h. Again its success has been
variable in controlling gastrointestinal output i.e., diarrhea in
intestinal failure patients. It should be discontinued if there is
no response after a 23 wk trial.
Osmotic/Malabsorption Diarrhea
Loperamide (100200 microgram/kg 24 times a day dependent on age po); Cholestyramine (18 g in 24 divided doses
po depending on age); Tedeglutide (not licenced yet in children).
Loperamide Loperamide has antipropulsive action by binding of opiate receptors in the gut wall and thereby inhibiting
release of acetylcholine and prostaglandins. If there is no improvement within 25 d, its used should be discontinued.
Cholestyramine Cholestyramine is a bile acid resin, which
binds unabsorbed bile salts. This is especially useful in patients following terminal ileal resection with interrupted
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Absolute
Malignancy
Psychosocial problems severe and irreconcilable
Relative
Intensive Care Unit (ICU) care
Immunodeficiency
Drug dependency
Loss of conventional venous access
Neoplasia, benign or of uncertain prognosis
Intestinal Transplantation
Intestinal transplantation (ITx) is reserved for children developing life threatening complications secondary to the use of
parenteral nutrition. ITx should not be the first treatment option in children with intestinal failure. All children with IF
should initially be managed on parenteral nutrition and then
referral should be made for intestinal transplantation depending on the criteria laid down by the Intestinal Transplant
Association (Table 2) [25]. Intestinal transplantation is done
in limited centres around the world and at present there are 71
active intestinal transplant centres all over the world. The type
of intestinal transplantation depends on the severity of liver
disease and predominantly there are two types of intestinal
transplant: Liver + intestine transplant and isolated intestinal
transplantation [26, 27]. Further details about the procedure
are beyond the remit of this chapter. The survival of intestinal
transplantation is improving and the current survival rates
from experienced centres are: 1 y survival of 90 %. 5 y survival of 65 % and 10 y survival of 50 %. Re-transplantation is
a possibility and there are more and more number of retransplants being reported according to the reports from the
Intestinal Transplant Registry.
Acknowledgments The authors acknowledge the multidisciplinary
team in the gastroenterology and The Liver Unit at Birmingham
Childerns Hospital. They also thank Mr. Oliver Gee, Consultant
Paediatric Surgeon, BCH for providing diagrams in this article.
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