Pediatric Nutrition in Practice

3 Nutritional Challenges in Special Conditions and Diseases

Key Words

Cholestasis · Cirrhosis · Biliary atresia · Vitamins

Key Messages

• Nutritional issues are common in children with cho-
  lestatic liver disease


• Patients need special attention to prevent fat-solu-
  ble vitamin deficiencies


• Cirrhosis and ascites require fluid and electrolyte
  management


• Liver transplantation is associated with improve-
  ment in growth and development
  © 2015 S. Karger AG, Basel

The most common types of cholestatic liver dis-
ease in children include extrahepatic biliary atre-
sia, Alagille’s syndrome, α 1 -antitrypsin deficien-
cy, cystic fibrosis and intestinal failure-associated
liver disease. Other causes include primary scle-
rosing cholangitis and drug-induced liver injury.
Nutritional issues are common in children with
all types of cholestatic liver disease.
Causes of malnutrition are inadequate energy
intake, intestinal malabsorption and increased
energy needs ( table  1 ). The underlying mecha-

nisms are multifactorial, including gastric dys-

motility, ascites, bacterial overgrowth, hepato-

megaly and secondary effects from medications.

There may be problems with malabsorption. De-

creased hepatic bile salt excretion leads to inade-

quate micelle formation and impaired lipid and

fat-soluble vitamin uptake. Triglyceride clear-

ance may also be impaired. Moreover, infants

and children with chronic liver disease may have

increased energy needs. For example, resting en-

ergy expenditure is increased by 30% in extrahe-

patic biliary atresia [1]. Factors contributing to

hypermetabolism include ascites, infection and

portal hypertension.

Nutritional assessment begins with a careful

dietary history and review of growth records.

Special attention should be devoted to sodium

and f luid intake. Over time, ascites and edema

may lead to changes in weight deceptively similar

to weight gain. In addition, hypoalbuminemia

may ref lect impaired hepatic synthetic function

as opposed to protein energy malnutrition. Inter-

pretation of certain micronutrient levels requires

simultaneous measurement of carrier proteins.

Because calcium and magnesium are bound to

albumin, serum levels may appear falsely low in

the setting of hypoalbuminemia. Ionized calci-

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

3.8 Nutritional Management in Cholestatic Liver

Disease

Bram P. Raphael