NUTRITION OF LIVER DISEASE

IN PEDIATRIC

HVCK1 : LÊ TÂN
Central role in energy metabolism, nutritional homeostasis , and absorption of
nutrients

Severe liver disease : leads to multiorgan failure, effects on growth and

development in the long term
PATHOPHYSIOLOGY OF MALNUTRITION IN
LIVER DISEASE
 Severe malnutrition (weight and/or height less than 2
SD ) with loss of fat stores and muscle wasting , affect
60% of infants with liver disease

 Morbidity and mortality post liver transplantation are

related to the degree of pretransplant malnutrition
REDUCED ENERGY INTAKE
 Anorexia : lead to considerable loss of body stores or body mass

The complications of portal hypertension : ascites and hepatosplenomegaly

> malabsorption of nutrients
 The treatment of ascites: fluid restriction, prescription of unpalatable feeds, or
both > anorexia , reduce energy intake further
 CARBOHYDRATE METABOLISM
 Normal circumstances, the liver receives portal vein blood rich in absorbed glucose,
stored in the liver as glycogen or circulated to extrahepatic tissues, especially muscle

The loss of glycogen stores in chronic liver disease leads to fasting hypoglycemia and
an inability to meet energy demands

Any significant hepatocyte loss, especially in acute liver failure, has an immediate
effect on glucose metabolism, leading to hypoglycemia.
PROTEIN METABOLISM
Amino acids are absorbed by the intestine directly into the portal
vein and transferred to the liver, where they are synthesized into
protein or used for energy. The liver is responsible for
approximately 10% of plasma protein synthesis; thus, amino acids
are constantly recycled

Nonessential amino acids are oxidized in both liver and muscle.

The seven essential aromatic amino acids (AAAs) (arginine,
histidine, lysine, methionine, phenylalanine, tryptophan, and
threonine) are metabolized in the liver
The three essential branched-chain amino acids (BCAAs) (leucine, isoleucine, and valine)
are metabolized predominantly in muscle and pass unaltered through the liver to the
periphery, where their uptake is regulated by insulin.

The liver detoxifies nitrogenous wastes via the urea cycle, leading to the production of
ammonia—hence the rise of plasma ammonia in both acute and chronic liver failure.
 The liver is the site of synthesis of all of the coagulation factors except factor VIII,
von Willebrand’s factor .Thus, coagulopathy is an early sign of significant acute or
chronic liver failure.

 In chronic liver disease, these metabolic changes result in muscle wasting,

hyperammonemia, hypoalbuminemia, hypoglycemia, hypolipidemia .

Hypoalbuminemia : reduction in plasma oncotic pressure, exacerbating the

development of ascites and peripheral edema. In acute liver failure, the main effects
of deranged protein metabolism are hyperammonemia and abnormal coagulation
FAT METABOLISM

Most dietary fat is in the form of long-chain triglycerides (LCTs), which are a high-energy source.

Fat emulsification in the stomach > micellar solubilization of di- and monoglycerides by bile acids
>transported into enterocytes.

 Once they are in the enterocytes, fatty acids are reesterified and chylomicrons are formed and removed
via the lymphatics through the portal system to the liver and other tissues.
 Medium-chain triglycerides (MCTs) do not depend on micellar solubilization for absorption
and can be transferred directly from the enterocytes to the portal circulation without
reesterification.

 The very-low-density lipoproteins and high-density lipoproteins are synthesized in the liver,
as is cholesterol, which is the precursor for many hormones, vitamins, and bile acids
LONG-CHAIN POLYUNSATURATED FATTY ACIDS
 Long-chain polyunsaturated fatty acids (LCPs) such as arachidonic acid and
docosahexaenoic acid (DHA) are essential nutrients in neonates and infants.
LCPs, in particular DHA, play a major role in the development of visual
acuity and mental development in the first year of life, particularly in preterm
infants

 Children with cholestatic liver disease have normal LCP and DHA levels at
birth but can become deficient within 8–12 weeks from malabsorption of
LCTs
FAT-SOLUBLE VITAMIN DEFICIENCY

 Chronic liver disease affects vitamin absorption, metabolism, and storage.

 Reduction in bile salt secretion leads to malabsorption of the fat-soluble

vitamins A, D, E, and K. Fat-soluble vitamin deficiency can develop within 6–
12 weeks of birth, depending on body stores and availability of vitamin
supplementation
ENDOCRINE DYSFUNCTION
 Growth failure in chronic liver disease can be exacerbated by an impaired
growth hormone (GH)/insulin-like growth factor (IGF-I) axis because IGF-I
and its major circulating binding protein

 Children with chronic liver disease have GH resistance due to

downregulation of GH receptor expression resulting in low IGF-I levels ,
despite elevated levels of GH, which could be a result of diminished hepatic
synthesis
INCREASED ENERGY EXPENDITURE
Energy requirements of up to 140% in children with both acute and chronic
liver disease.
Mechanisms : portosystemic shunting and ascites; abnormal intermediate
metabolism; and energy demands of specific complications, such as sepsis and
variceal hemorrhage

 Children with acute liver failure also have excess energy expenditure and
requirements because of multiorgan failure
TRACE ELEMENTS AND METALS

Biochemical deficiencies of water-soluble vitamins : thiamin and pyridoxine

can occur and lead to nutritional cardiomyopathy and peripheral neuropathy.

Trace metal deficiencies include iron deficiency secondary to gastrointestinal

bleeding or diminished intake and zinc and
selenium deficiencies caused by reduced enteral intake, malabsorption, or
increased losses
CONSEQUENCES OF MALNUTRITION
NUTRITIONAL ASSESSMENT

 The assessment of malnutrition should be performed using a number

of parameters, such as triceps or subscapular skinfolds, midarm
circumference, and arm muscle measurements (midarm muscle area).
Triceps, skinfolds, and midarm circumference are useful indicators
of body fat and protein and serial recordings demonstrate early loss of
fat stores before weight and height changes become obvious
 Children who are at particular risk for developing malnutrition include
the following:
+ Children under 2 years of age who have severe cholestasis (serum
bilirubin > 70 mmol/L [4 mg/dL])

+ Those with progressive liver disease such as biliary atresia or

severe familial intrahepatic cholestasis
+ Those with end-stage liver failure awaiting liver transplantation
+ Children with recurrent hepatic complications such as ascites and
bleeding varices
STRATEGIES FOR NUTRITIONAL SUPPORT
VITAMIN AND MINERAL SUPPLEMENTATION

 Both water- and fat-soluble vitamins should be supplemented in children with

chronic liver disease. Supplementation should be based on plasma levels of
selenium, zinc, calcium, and magnesium. Iron supplementation might be required
in children with chronic blood loss.

 Fat-soluble vitamins are required in all children with prolonged or cholestatic

liver disease . Most children will be maintained adequately on oral fat-soluble
vitamin administration
ACUTE LIVER DISEASE

 Fulminant hepatic failure requires careful management of protein to prevent

hepatic encephalopathy and reduce excess protein catabolism.
 Protein should be restricted only for short periods to treat severe encephalopathy

 Fluid volume can be reduced to prevent or treat cerebral edema;

 It is critical to maintain adequate glucose levels by infusing 6–8 mg/kg/min of
glucose to prevent hypoglycemia
CHRONIC LIVER DISEASE

 The majority of children with chronic liver disease will have cholestasis, from
biliary atresia, Alagille syndrome, or familial intrahepatic cholestasis, and should
be managed as indicated earlier
 The modular feeding system is extremely flexible in its composition and allows
individual prescription of protein, sodium, and water to which complex
carbohydrate polymers, fats, vitamins, and minerals are added to produce a highly
energydense/nutrient-dense feed to suit the child’s specific nutritional requirements
CONCLUSION

 Role of liver in energy and growth has particular relevance

for infants and children with end-stage liver failure
Nutritional rehabilitation has been achieved by intensive nutritional
support, and mortality following liver transplantation has been reduced

Its success depends on a multidisciplinary approach based on early

intervention and prevention of malnutrition