Bram P. Raphael
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um may be helpful in this setting. Similarly, the retinol-to-retinol-binding protein ratio (retinol divided by retinol-binding protein, both in mi- cromoles per liter) is a preferred method of inter- preting vitamin A status. Possible toxicity is sug- gested by a ratio of >1, and deficiency is suggested by a ratio of <0.8. Similarly, vitamins E status should be interpreted by the ratio of vitamin E to the total lipid concentration. Vitamin E deficien- cy is suggested by a ratio of <0.6 mg/g in children under the age of 1 year, and <0.8 mg/g in older children.
To compensate for increased energy needs and anorexia, supplemental nasogastric tube feeds
may be helpful. Hepatosplenomegaly, ascites or varices may limit the feasibility of placing a per- manent feeding tube. Parenteral nutrition should be reserved for those incapable of receiving en- teral feeds by mouth or feeding tube, or for those unwilling to do so. Although there is unease sur- rounding parenteral nutrition due to possible hepatotoxicity, it may be used safely for short in- tervals without problems. Standard parenteral amino acid solutions are adequate in nearly all situations. In studies on adults, the use of branched-chain amino acids may have shown beneficial effects on uncontrolled encephalopa- thy. At this time, the use of branched-chain ami-
Table 1. Clinical manifestations and etiologies of common nutritional problems in liver disease
Nutrient affected Manifestation Etiology
Protein Stunting, muscle wasting, motor developmental delay
Protein energy malnutrition, decreased insulin-like growth factor 1 synthesis Ascites or peripheral edema Decreased albumin synthesis leading to
decreased oncotic pressure
Coagulopathy Alteration of synthesis in clotting factors Hepatic encephalopathy Decreased aromatic amino acid metabolism Fat Steatorrhea, essential fatty acid deficiency
(rash), fat-soluble vitamin deficiencies (see below)
Impaired intestinal absorption, decreased intake of essential fatty acids
Hypercholesterolemia,
hypertriglyceridemia (xanthomas)
Impaired hepatic lipid clearance
Carbohydrate Hyperglycemia Insulin resistance leading to impaired muscle and liver glycogen synthesis
Fasting hypoglycemia Decreased glycogen stores with hepatocellular dysfunction
Vitamin A Night blindness, degeneration of retina, xerophthalmia, poor growth,
hyperkeratosis
Impaired intestinal absorption
Vitamin D Rickets, osteoporosis, cranial bossing, epiphyseal enlargement, persistently open fontanelle in infants
Impaired intestinal absorption, and decreased hepatic 25-hydroxylation
Vitamin E Peripheral neuropathy, ataxia, hemolytic anemia
Impaired intestinal absorption Vitamin K Coagulopathy, hemorrhagic manifestations
such as bruising, bone disease
Impaired intestinal absorption Minerals Low iron, copper, zinc, selenium, calcium Impaired intestinal absorption
180 Raphael
no acid formulae in children with chronic liver disease is only investigational.
With coexisting cirrhosis there are added nutri- tional challenges such as anorexia, impaired glu- cose tolerance and salt-fluid balance. Patients with cirrhosis can have impaired glucose tolerance with hyperinsulinemia and insulin resistance. Protein energy malnutrition is present in 20% of patients with well-compensated cirrhosis and in 60% of pa- tients with severe liver dysfunction [2] . On the oth- er hand, decreased glycogen stores and decreased glucose production in all types of end-stage liver disease may result in fasting hypoglycemia. Pro- tein turnover is usually normal or increased.
Where the cholestasis is attributed primarily to chronic parenteral nutritional exposure, re- stricting the dose of intravenous lipid emulsion may be considered, typically 1–2 g/kg/day. The use of alternative lipid emulsions should be con- sidered because components of soybean-based lipids have been suspected for their possible role in intestinal failure-associated liver disease, in- cluding plant sterols, high intakes of precursor polyunsaturated fatty acids, low-bioactivity vita- min E content, and others. Restricting enteral fat intake is not indicated. Alternative intravenous lipid emulsions containing fish oil show promise, and further studies are anticipated.
In choosing enteral formulae, standard prod- ucts may be adequate. With intestinal malab- sorption, patients may benefit from formulae rich
in medium-chain triglycerides or formulae sup- plemented with medium-chain triglyceride oil.
When using specialized formulae chronically, long-chain triglycerides are also needed to pre- vent essential fatty acid deficiency. Restrictive di- ets are not necessary, and may be dangerous. Pro- tein should be provided according to the recom- mended daily allowance in order to prevent protein catabolism. It may be appropriate to tem- per sodium intake for ascites or edema. Table 2 lists recommendations on vitamin and mineral supplementation for chronic liver disease.
Successful liver transplantation is usually as- sociated with improved growth and developmen- tal outcomes. Following liver transplantation, en- teral feeds should be started as soon as possible, but short-term parenteral nutrition may be safely used while awaiting return of bowel function.
Tube feeds may be helpful in postoperative an- orexia. Mild sodium restriction may be necessary to minimize edema with steroids.
Conclusions
• Children with cholestatic liver disease have special nutritional needs
• Treatment is aimed at reversing consequences of anorexia, increased energy needs, associat- ed intestinal malabsorption as well as altered carbohydrate and protein metabolism
Table 2. Maintenance recommendations on vitamin and mineral supplementation for patients with cholestasis
Product Dose
Vitamin A [3] Liquid vitamin A 5,000–25,000 IU/day
Vitamin D [4] Vitamin D 200–1,000 IU/day
Vitamin E [5, 6] Liquid vitamin E (preferably water-soluble preparation)
15–25 IU/kg/day
Vitamin K [7] Vitamin K1 (Mephyton) 2.5–5 mg/day, every 2–3 days
Zinc [4] Zinc sulfate 1 mg/kg/day
Calcium [4] Elemental calcium 25–100 mg/kg/day
Phosphorus [4] Elemental phosphorus 25–50 mg/kg/day
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 178–181 DOI: 10.1159/000360333
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5 Sathe MN, Patel AS: Update in pediat- rics: focus on fat-soluble vitamins. Nutr Clin Pract 2010; 25: 340–346.
6 Kelly DA, Davenport M: Current man- agement of biliary atresia. Arch Dis Child 2007; 92: 1132–1135.
7 Duggan C, Watkins JB, Walker WA:
Nutrition in Pediatrics: Basic Science, Clinical Application, ed 4. Hamilton, BC Decker, 2008.
References
1 Pierro A, Koletzko B, Carnielli V, et al:
Resting energy expenditure is increased in infants and children with extrahepat- ic biliary atresia. J Pediatr Surg 1989; 24:
534–538.
2 Nutritional status in cirrhosis. Italian Multicentre Cooperative Project on Nutrition in Liver Cirrhosis. J Hepatol 1994; 21: 317–325.
3 Erlichman J, Hohlweg K, Haber BA: Bili- ary atresia: how medical complications and therapies impact outcome. Expert Rev Gastroenterol Hepatol 2009; 3: 425–
434.
4 Ramirez RO, Sokol KI: Medical man- agement of cholestasis; in Suchy FJ (ed):
Liver Disease in Children. St Louis, Mosby, 1994.
3 Nutritional Challenges in Special Conditions and Diseases
Key Words
Short bowel syndrome ã Intestinal adaptation ã Protracted diarrhea of infancy ã Parenteral nutrition ã Feeding, oral, enteral ã Breast milk ã Long-chain fatty acid-containing formulas ã Medium-chain triglycerides ã Hydrolyzed protein formulas ã Amino acid formulas ã Small intestinal bacterial overgrowth ã Feeding aversion
Key Messages
• Protracted diarrhea of infancy or short bowel syn- drome (SBS) requires parenteral nutrition together with oral feeding (OF) or enteral feeding (EF). The use of the gastrointestinal (GI) tract as early and as much as possible, according to clinical tolerance, should be promoted and feeding aversion pre- vented
• Adaptation – the physical and physiological pro- cesses by which the intestine compensates for loss of intestinal length or function – is optimized with the provision of OF or EF
• Direct mucosal contact with nutrients, pancreatico- biliary secretions and neurohormonal factors ex- plains how the use of the GI tract promotes physio- logical intestinal adaptation. It may also contribute to preventing cholestasis and liver disease • The intestinal microbiota has both positive (short-
chain fatty acid production) and deleterious effects (intraluminal bacterial overgrowth)
• The types of diet regarding breast milk, protein hy- drolysates and formulas containing amino acids or long- or medium-chain fatty acids remain contro- versial. The routes (oral, gastric and transpyloric) and the modes (bolus, continuous or both) of feed- ing are also debated. Very few randomized trials have been performed © 2015 S. Karger AG, Basel
Introduction
The so-called protracted diarrhea of infancy (PDI), which has become a rare condition in de- veloped countries, is caused by severe malabsorp- tion secondary to an acquired intestinal mucosal injury due to infection, inflammation or allergic reaction. ‘Intractable diarrhea of infancy’ refers to congenital enteropathies involving the develop- ment or renewal of intestinal mucosa that lead to very long-lasting or often irreversible intestinal failure (IF) [1] . Short bowel syndrome (SBS) is the leading cause of IF and is a state of malabsorption following extensive small intestinal resection.
The functional consequences as well as the prog- nosis of SBS depend on the age-adjusted small bowel length, site of resection, presence or ab- sence of the ileocecal valve (ICV) and occurrence
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 182–189 DOI: 10.1159/000360339