Nutritional Management in Cholestatic Liver Disease 179
3
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