Pediatric Nutrition in Practice

Nutritional Management in Cholestatic Liver Disease 179

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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-

Ta b l e 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

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