Pediatric Nutrition in Practice

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-f luid 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

Ta b l e 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 K 1 (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