Pediatric Chronic Intestinal Failure: Something Moving?
Abstract
:1. Introduction: Definitions, Prevalence and Goal of Treatment
2. Underlying Conditions
2.1. Short Bowel Syndrome
2.2. Intestinal Neuromuscular Motility Disorders
2.3. Congenital Enteropathies
3. Current Treatment of Pediatric Intestinal Failure
3.1. Multidisciplinary Care
3.2. Home Parenteral Nutrition
3.3. Medical Management
3.4. Intestinal Transplantation
4. Complications of Long-Term Home Parenteral Nutrition
4.1. Central Venous Catheter-Related Bloodstream Infection
4.2. Catheter-Related Thrombosis
4.3. Intestinal Failure-Associated Liver Disease (IFALD)
4.4. Small Intestinal Bacterial Overgrowth
4.5. Metabolic Bone Disease
4.6. Renal Dysfunction
4.7. Growth
4.8. Health-Related Quality of Life
5. Future Perspectives
5.1. Outcomes in Research
5.2. GLP-2
5.3. Transition
6. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Medication | Type of Intestinal Failure | Reason to Use | Use for Pediatrics/Adults/Both | Evidence and References | Comment and References |
---|---|---|---|---|---|
1.1. Antisecretory agents | |||||
1.1. Histamine H2 receptors | I, II, III | Reduction in hyperacidity after massive resection. | Both | Level I [61] | |
1.2. Proton-pump inhibitors | I, II, III | Reduction in hyperacidity after massive resection. | Both | Level I [61]. | - Altering the fecal gut microbiota. - Causing hypomagnesaemia. [62,63]. |
1.3. Opioid receptor agonist | I, III | Prolonged intestinal transit time. | Both | Level I [64,65] | Increasing duodenal muscle tone and inhibiting propulsive motor activity. Side effects: fatigue, dizziness, nausea and vomiting. Loperamide associated with less side effects compared to codeine. |
1.5. Bile acid sequestrants | I, II, III | Bile salt malabsorption after terminal ileal resection. | Both | Level IIa | Reducing absorption of fat-soluble nutrients. |
1.6. Somatostatin analogue | I | Reduction in gastric acid and secretion of pancreatic enzymes. Reduction in gastric emptying, gall-bladder contractions and ileal and longitudinal muscle contractions. | Both | Level IIb [66,67] | Glucose levels assessment and blood pressure monitoring whilst on an octreotide infusion is advised. |
1.7. Enkephalinase inhibitor | I | Decrease in intestinal secretion and increase in absorption. | Both | Level I [68] | No change in gastrointestinal transit times in rat or mice or healthy human volunteers on racecadotril (enkephalinase inhibitor). [69,70,71] |
1.8. α2-receptor agonist | I, II | Stimulation of α2-adrenergic receptors on enteric neurons that also reduce gastric and colonic motility and intestinal fluid secretion. | Adults | Level I [72]. | No significant benefit of clonidine (α2-receptor agonist) on volume output reported. |
2. Adjunctive absorptive agents | |||||
2.1. Fibers (e.g., psyllium seeds) | I, II, III | Improvement of feces consistency. | Both | Level IIa [73,74] | |
2.2. Pancreatic enzymes | I | Increase in intestinal digestion (especially fat) in pancreatic atrophy and exocrine insufficiency. | Both | Level III expert opinion | Proven fat malabsorption (preferably assessed by BOMB calorimetry). [75]. |
3. Prokinetic agents | |||||
3.1. Serotonin 5-HT4 agonist | II | Delayed gastric emptying. | Both | Level IIb [76,77] | Prucalopride is safe and effective in children. Patients treated with cisapride (serotonin 5-HT4 agonist) require careful cardiac monitoring because of corrected QT prolongation, risk of cardiac dysrhythmias and deaths. |
3.2. Erythromycin, clarithromycin, amoxicillin–clavulanic Acid | II | Pro-motility effect. | Both | Level IIa | |
3.3. Acetylcholinesterase inhibitor | II | Pro-motility effect. | Both | Level IIb [78]. | Pyridostigmine and neostigmine are safe and effective in children in case series. |
4. Antibiotic agents | |||||
Mostly used: Rifaximin, Metrodinazole and Amoxicillin/Clavulanic Acid | I, II, III | Treatment of small-intestinal bacterial overgrowth. | Both | Level IIa [79]. | - Risk of fungal infections, antimicrobial resistance, or Clostridium difficile infection. - Functional ileocecal valve is of influence for maintaining a balanced microbial flora. - Risk of altering fecal gut microbiota. |
5. Probiotic agents | |||||
Many strains and dosages over the counter are available | I, II, III | No evidence of benefit in small studies; risk of bacterial translocation leading to sepsis. | Both | Level IIa [80]. | High risk of adverse effects in immunocompromised and debilitated patients. |
6. Growth factors | |||||
GLP-2 analogues | I | Stimulation of intestinal adaptation and absorption, decreasing fecal losses. | Both | Level I [81]. | Teduglutide is licensed for use in adults and children with short bowel syndrome. |
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Demirok, A.; Nagelkerke, S.C.J.; Benninga, M.A.; Jonkers-Schuitema, C.F.; van Zundert, S.M.C.; Werner, X.W.; Sovran, B.; Tabbers, M.M. Pediatric Chronic Intestinal Failure: Something Moving? Nutrients 2024, 16, 2966. https://doi.org/10.3390/nu16172966
Demirok A, Nagelkerke SCJ, Benninga MA, Jonkers-Schuitema CF, van Zundert SMC, Werner XW, Sovran B, Tabbers MM. Pediatric Chronic Intestinal Failure: Something Moving? Nutrients. 2024; 16(17):2966. https://doi.org/10.3390/nu16172966
Chicago/Turabian StyleDemirok, Aysenur, Sjoerd C. J. Nagelkerke, Marc A. Benninga, Cora F. Jonkers-Schuitema, Suzanne M. C. van Zundert, Xavier W. Werner, Bruno Sovran, and Merit M. Tabbers. 2024. "Pediatric Chronic Intestinal Failure: Something Moving?" Nutrients 16, no. 17: 2966. https://doi.org/10.3390/nu16172966