AUTHOR AND EDITOR INFORMATION

Section 1 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

BACKGROUND AND PATHOPHYSIOLOGY

Section 2 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

Background

Advancements in donor-organ selection, in the availability of immunosuppressive therapy, and in the expertise of specialized pediatric transplant teams have contributed to the success of pediatric solid-organ transplantation. Pediatricians and pediatric transplant surgeons must continue to improve outcomes in this specialized group of patients. According to Shepherd (1996), despite vast improvements made thus far in the field of pediatric transplantation, "the single area in which the largest improvement can be made is in the area of nutritional support."¹

Data regarding nutrition and pediatric transplantation are limited. However, findings suggest that adult transplant recipients who are well nourished before transplantation have reduced morbidity and mortality rates, shortened stays in the intensive care unit and in the hospital, improved long-term survival rates, and enhanced quality of life (see Nutritional Requirements of Adults Before Transplantation).

Children clearly differ from adults in nutritional risk based on age and growth patterns. Because of more rapid growth, younger patients are more likely to experience long-term consequences of nutritional deficiencies than are older children or adults. The pediatric response to illness and operation predictably affects macronutrient and micronutrient metabolism and is frequently influenced by severe dietary restrictions. Because of shortages of appropriate donor organs, patients are often subjected to extensive waiting periods prior to operation. Aggressive nutritional management during this interval is crucial to achieve optimal outcomes.

For further details regarding particular solid organ transplants, see Heart Transplantation, History of Pediatric Liver Transplantation, Immunosuppression, Intestinal Transplantation, Intestinal and Multivisceral Transplantation, Kidney Transplantation, Liver Transplantation, Lung Transplantation, and Xenotransplantation.

The topics Bone Marrow Transplantation, Growth and Development After Transplantation, Immunology of Transplant Rejection, Infections After Bone Marrow Transplantation, and Posttransplant Lymphoproliferative Disease are beyond the scope of this review.

Pathophysiology

As a normal response to stress, catecholamines are released from the adrenal medulla and initiate hypermetabolism. Epinephrine release increases hepatic gluconeogenesis, pancreatic suppression of insulin production, and glucagon release. Low insulin levels stimulate fat mobilization for fuel and catabolism of skeletal muscle, increasing plasma concentrations of amino acids.

Increased glucagon production fosters carbohydrate metabolism and resultant ureagenesis. The effect of glucagon on urea production results from hepatic gluconeogenesis and the use of alanine for the formation of more pyruvate for new glucose production. This process increases urea synthesis. Therefore, ureagenesis and gluconeogenesis usually proceed at similar rates. Finally, growth hormone is also stimulated during stress and enhances nitrogen retention in the fed state but not during fasting.

Increases in energy expenditure and nitrogen excretion are typical manifestations of inflammation, infection, and injury. Operative procedures can increase resting energy expenditure by 24-79%. In such patients, maintaining body protein during catabolic illness is difficult (see Media file 1).

NUTRITIONAL SUPPORT

Section 3 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

Goals of nutritional support

• Prevent or treat malnutrition and establish a positive nitrogen balance.
• Optimize nutritional status by achieving desirable body weight, muscle mass, and visceral protein stores.
• Monitor and supplement vitamin and mineral levels to prevent deficiency.
• Avoid potentially toxic substances.
• Improve nutritional status to counteract the catabolic effects of operative intervention and high-dose immunosuppression.
• Limit the risk of complications, such as hypoglycemia and infection.
• Promote growth and development.
• Manage nutritional adverse effects of immunosuppressive therapy.
• Support wound healing.
• Optimize the patient's quality of life.

Assessment of nutritional needs

Nutritional assessment should be started early and regularly monitored. It is based on complete medical history taking, physical examination for signs of nutrient deficiencies or toxicities, and biochemical measurements of nutritional status. Reassessment should be completed at least every 3-4 months. Special attention should be given to the completion of a 72-hour food diary or a food frequency questionnaire. Furthermore, a thorough evaluation for drug-nutrient interactions should occur at routine intervals.

The nutrition-related adverse effects and other adverse effects of immunosuppressive therapy are as follows:

• Cyclosporine - Hyperglycemia, hypercholesterolemia, hyperkalemia, hypomagnesemia, gum hyperplasia, hirsutism
• Prednisone - Fluid and/or sodium retention, hyperglycemia, increased appetite, poor statural growth, GI ulcerations, osteoporosis, pancreatitis, mood swings
• OKT3, murine monoclonal antibodies - Nausea, vomiting, diarrhea, anorexia, fluid retention
• Azathioprine - Hyperkalemia, hyperglycemia, hypomagnesemia, alopecia, diarrhea, insomnia, tremor, paresthesias of the extremities
• Mycophenolate mofetil - Diarrhea, vomiting, gastritis, neutropenia, thrombocytopenia

Anthropometry

Upper-body anthropometry is the most accurate measure of lean body mass in patients with alterations in fluid status. Acute malnutrition is best estimated by calculating midarm muscle circumference from triceps skinfold measurements, as follows:

midarm muscle circumference = midarm circumference (in centimeters)-[0.314 X triceps fat fold (in millimeters)]

Daily weights and estimated dry weights should be obtained in patients with altered fluid status. In addition, the weight-height index can be determined, as can the standard deviation score or Z score (distance in standard deviations of the sample from the mean) for height and the head circumference (for patients £3 y).

Analysis of growth charts

Growth charts are analyzed to determine the type and degree of malnutrition. Chronic malnutrition is manifested as stunting. This is best determined by performing serial determinations of the height-age index.

Assessment of energy requirements

With measurements of oxygen consumption and carbon dioxide production, indirect calorimetry allows for precise measurement of the patient's daily caloric needs. For each liter of carbon dioxide produced, the body must generate 1.1 kcal of energy. When indirect calorimetry is not practical, basal energy expenditure (BEE) can be determined on the basis of the Harris-Benedict equation.

For male patients: BEE = 66.5 + (13.7 X weight in kilograms) + (5 X height in centimeters) - (6.78 X age in years).

For female patients: BEE = 655 + (9.56 X weight in kilograms) + (1.85 x height in centimeters) - (4.68 X age in years).

In patients with clinically significant edema or ascites, calculate energy needs on the basis of adjusted body weight or estimated dry weight, as follows:

Adjusted body weight = (ideal body weight - actual body weight) X 20% + ideal body weight.

To calculate estimated daily caloric requirements, multiply BEE by the stress factor and by the activity factor.

Adjust BEE for the added stress of operation, disease, infections, and wounds as follows:

• For elective operation, multiply BEE by 1.2.
• For wound or infection, multiply BEE by 1.5.

Adjust the BEE for activity, as follows:

• For patients confined to a bed, multiply by 1.2.
• For patients allowed very light activity, multiply by 1.3.
• For patients allowed light activity, multiply by 1.5.
• For patients allowed moderate activity, multiply by 1.6.

Kilocalorie requirements can also be estimated by using standard nomograms, such as the recommended daily allowance (RDA). Although these values apply to most children, the data may need to be adjusted if patients have severe malnutrition.

The estimated energy requirements in infants and children are as follows:

• Age 0-1 years - 90-120 kcal/kg body weight
• Age 1-7 years - 75-90 kcal/kg body weight
• Age 7-12 years - 60-75 kcal/kg body weight
• Age 12-18 years - 30-60 kcal/kg body weight

In malnourished patients who lose or fail to gain weight, energy requirements may need to be increased by up to 50% more than the calculated maintenance requirements.

Assessment of protein requirements

Nitrogen balance studies, such as the 24-hour urinary urea nitrogen test, are the standard methods for assessing protein needs. When these studies are not feasible, estimation based on the RDA for age can be used. These values serve as baselines and may need to be adjusted for the patient's state of malnutrition and/or physiologic stress.

The estimated protein requirements in infants, children, and adolescents are as follows:

• Age 0-6 months - 2.2 g/kg body weight
• Age 6-12 months - 2 g kg/body weight
• Age 1-3 years - 0.18 g/cm height
• Age 4-6 years - 0.21 g/cm height
• Age 7-10 years - 0.21 g/cm height
• Age 11-14 years - 0.29 g/cm height
• Age 15-18 years - 0.34 g/cm height

Assessment of fluid requirements

The daily fluid requirements of nonstressed pediatric patients are as follows:

• Premature neonates who weigh less than 2 kg - 150 mL/kg
• Neonates and infants who weigh 2-10 kg - 100 mL/kg for the first 10 kg
• Infants and children who weigh 10-20 kg - 1000 mL plus 50 mL/kg over 10 kg
• Children who weigh more than 20 kg - 1500 mL plus 20 mL/kg over 20 kg

Micronutrients

Table 1. RDAs and Adequate Intakes for Fat-Soluble Vitamins*^{2, 3}

^*The allowances, expressed as average daily intakes over time, are intended to provide for individual variations among most healthy persons living in the United States under usual environmental stresses. Diets should be based on a variety of common foods to provide other nutrients for which human requirements have been less well defined than these. Asterisks indicate adequate intakes.

† RDAs are set to meet the needs of 97-98% of the individuals in the group.

‡Retinol equivalents, where 1 retinol equivalent = 1 mcg retinol or 12 mcg beta-carotene

^§As cholecalciferol, 1 mcg cholecalciferol = 40 IU of vitamin D

||Alpha-tocopherol equivalents (ie, 1 mg D-alpha-tocopherol)

Table 2. RDAs for Water-Soluble Vitamins*^{4, 5}

^*The allowances, expressed as average daily intakes over time, are intended to provide for individual variations among most healthy persons living in the United States under usual environmental stresses. Diets should be based on various common foods to provide other nutrients for which human requirements have been less well defined than these. Asterisks indicate adequate intakes.

† RDAs are set to meet the needs of 97-98% of the individuals in the group.

‡Niacin equivalents, where 1 niacin equivalent = 1 mg niacin or 60 mg dietary tryptophan

Table 3. RDAs and Adequate Intakes for Minerals*²

^*The allowances, expressed as average daily intakes over time, are intended to provide for individual variations among most healthy persons living in the United States under usual environmental stresses. Diets should be based on a variety of common foods to provide other nutrients for which human requirements have been less well defined than these. Asterisks indicate adequate intakes.

† RDAs are set to meet the needs of 97-98% of the individuals in the group.

Vitamin and trace mineral metabolism in pediatric patients has not been well studied. For infants and children, fat-soluble vitamins (A, D, E, K) and water-soluble vitamins (ascorbic acid, thiamine, riboflavin, pyridoxine, niacin, pantothenate, biotin, folate, vitamin B-12) are required and routinely administered. Trace minerals required for normal development are zinc, iron, copper, selenium, manganese, iodide, molybdenum, and chromium.

Because vitamins and trace minerals act as enzymes, they are not consumed in biochemical reactions. Therefore, unless these substances are lost (eg, in diarrhea), supplementation of these nutrients in great excess of the RDA is not routinely indicated. In children with end-stage cardiac, liver, or renal disease (in whom deficiencies are most likely present), monitoring for signs of deficiencies, conservatively replacing losses, and observing for toxicities is important.

Clinical manifestations of nutrient toxicities are as follows:

• Vitamin A - Headache, vomiting, diplopia, alopecia, dryness of mucous membranes, dermatitis, anemia, insomnia, bone abnormalities, bone and joint pain, hepatomegaly, liver damage, hypercalcemia, hyperlipidemia, menstrual irregularities, spontaneous abortions, birth defects
• Vitamin D - Nausea, vomiting, excessive thirst and urination, muscular weakness, joint pain, hypercalcemia, disorientation, irreversible calcification of heart, lungs, kidneys, and other soft tissues
• Vitamin E - Exacerbation of the coagulation defect due to vitamin K deficiency, dizziness, headache, fatigue, weakness
• Vitamin K - Hemolytic anemia, liver damage, and, in newborns, kernicterus caused by menadione (vitamin K3) but not phylloquinone (vitamin K1)
• Vitamin C (ascorbic acid) - Nausea, diarrhea, kidney stones, mobilization of bone minerals, systematic conditioning to high intakes
• Vitamin B-1 (thiamine) - Gastric upset (Prolonged, large parenteral injections can lead to sensitized anaphylactoid reactions.)
• Vitamin B-2 (riboflavin) - Yellow-orange discoloration of urine
• Niacin
• • Nicotinic acid - Vascular dilatation, GI irritation, increased muscle glycogen use, decreased serum lipids, decreased mobilization of fatty acids from adipose tissues, hepatomegaly
  • Nicotinamide - Nausea, heartburn, fatigue, dry hair, sore throat, inability to focus eyes
• Vitamin B-6 - Dizziness, nausea, ataxia, peripheral neuropathy, systemic conditioning to high intakes
• Folic acid (folate and folacin) - Obscuration of pernicious anemia that leads to nerve damage; possible reduced zinc absorption
• Vitamin B-1 - Occasional mild diarrhea
• Biotin - GI upset
• Pantothenic acid - Occasional diarrhea and edema
• Calcium - Nausea, constipation, hypertension, kidney stones, myopathy; may inhibit absorption of iron and zinc
• Phosphorous - Calcium antagonism, which can result in tetany and convulsions
• Magnesium - Nausea, diarrhea, hypotension, bradycardia, vasodilatation, ECG changes, coma, cardiac arrest
• Iron - Bloody diarrhea, vomiting, hemosiderosis, hemochromatosis, cirrhosis, diabetes mellitus, cardiac failure, increased incidence of hepatoma; may compromise zinc and copper absorption
• Zinc - GI irritation, vomiting, impairment of copper status, microcytic anemia, impairment of immune responses, decline in serum high-density lipoproteins
• Copper - Nausea, gastric pain, diarrhea, vascular collapse; interacts with zinc, cadmium, and molybdenum
• Fluoride - With 4 mg, mottling (chalkiness) of teeth; with 10 mg or more, adverse affects on bone health, kidney function, and possibly muscle and nerve function
• Iodide - Blocks formation of thyroid hormones; may cause goiter
• Selenium - Fingernail changes, hair loss, nausea, abdominal pain, diarrhea, fatigue, irritability, peripheral neuropathy
• Manganese - Severe psychiatric disorder, reproductive and immune system dysfunction, kidney and liver disorders
• Chromium - Observed in individuals exposed to chromate dust or absorption through the skin, increased incidence of lung cancer, dermatitis, allergies
• Molybdenum - Antagonistic to copper, increased incidence of gout

Methods of feeding

The enteral route is the preferred route of feeding because it is the most physiologic, it is associated with trophic effects on the gut and liver, and it has a lower risk of infections than does total parenteral nutrition (TPN). Small, frequent feedings are useful to address anorexia and early satiety associated with end-stage renal disease or end-stage liver disease. In addition, small, frequent feedings may help prevent hypoglycemia and consequently limit the resultant catabolism of muscle associated with hormonal derangements, diminished glycogen storage, and restricted mobilization capacity, all seen in malnutrition.

Nutritional supplementation should be considered when a patient does not have normal height velocity or is unable to meet nutrient needs via regular oral intake. Whether by way of cyclical tube feeding or oral formulas, nutritional supplements aid in the provision of energy and high-quality protein requirements.

Supplementation is especially beneficial in patients who require moderate calories and high protein intake. When prescribing nutrition supplements, one should consider palatability and monitor for intolerance (eg, hyperosmolarity, hyperglycemia, fat intolerance). In infants fed solely by means of tube feedings or TPN, special attention must be paid to regular nonnutritive sucking and repetitive oral stimulation to decrease development of oral aversive behaviors.

Breastfeeding

Human milk has several advantages over commercial formulas. Breast milk contains approximately 87% water and supplies 0.64-0.67 kcal/mL. The fat content of breast milk is high at 3.4 g/dL. Protein and trace elements in human milk are better absorbed than commercial formulas. In addition, breast milk has several immunologic advantages over commercial formulas. When feasible, and with guidance from a lactation consultant, breastfeeding of the infant at nutritional risk should be encouraged.

Physical examination

See Anthropometry.

Clinical manifestations of malnutrition are as follows:

• Protein energy malnutrition - Growth failure
• Protein catabolism - Muscle wasting, motor development delay
• Fat malabsorption - Steatorrhea
• Essential fatty acid deficiency - Conjunctival and corneal drying, abnormal retinal function, night blindness, keratomalacia, xerophthalmia
• Vitamin E deficiency - Peripheral neuropathy, ophthalmoplegia, ataxia, hemolysis, areflexia, poor proprioception
• Vitamin D deficiency - Osteopenia, rickets, fractures
• Vitamin K deficiency - Bruising, epistaxis, coagulopathy, petechiae
• Zinc deficiency - Anorexia, acrodermatitis, poor growth
• Hypercholesterolemia - Xanthomata
• Impaired GI function (hypochlorhydria, reduced mucosal function) - Diarrhea
• Immunosuppression secondary to reduced cell-mediated immunity - Systemic infections

ORGAN TRANSPLANTATIONS

Section 4 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

Heart transplantation

• Frequency: More than 3000 children received heart transplants over the past decade.
• Mortality: The overall 5-year survival rate is 75-85%.
• Age: The prognosis is best for the older child or adolescent.
• Malnutrition
• • Cardiac cachexia secondary to anorexia and hypermetabolism
  • Increased nutrient losses through urine and feces
  • Impaired delivery of nutrients to tissues
• Laboratory values
• • Albumin levels
  • Prealbumin levels
  • Hemoglobin (Hb) and hematocrit (Hct) levels
  • Serum iron levels
  • Transferrin levels
  • Glucose levels
  • BUN levels
  • Creatinine levels
  • Lipid profile
• Recommendations
• • Energy
  • • Follow the RDAs for the patient's chronologic age, as applicable.
    • Provide additional kilocalories for children who have energy malnutrition to facilitate catch-up growth.
    • Maintenance of ideal body weight is recommended. Body weight more than 140% of the reference range is an absolute contraindication in adults. Obesity is unusual in these patients. No outcome data are available for children with regard to obesity.
  • Protein: Maintain a positive nitrogen balance.
  • Fluids
  • • Liberally administer fluids according to the patient's cardiopulmonary tolerance (>160 mL/kg/d is rarely tolerated) unless limiting comorbidities (eg, renal insufficiency) are being managed.
    • Most infants are treated with 100-140 mL/kg/d. Fluids are provided as enteral feeding or breast milk with supplemental fluids given by means of TPN.

Kidney transplantation

• Frequency: Pediatric patients account for 1.4% of all registrants on the waiting list for kidney transplants.
• Morbidity: Five-year graft survival rates are 92% for patients younger than 1 year, 81% for patients aged 1-5 years, and 80% for patients aged 6-10 years.
• Mortality: Annual posttransplantation death rates among pediatric registrants remain low.
• Age: Children aged 1-5 years have posttransplantation mortality rates substantially higher than those of older children.
• Race: African-American and Hispanic patients with end-stage renal disease have growth deficits greater than those of Caucasian patients.
• Malnutrition
• • Anorexia
  • Protein and calorie insufficiency
  • Renal osteodystrophy
  • Aluminum toxicity
  • Uremic acidosis
  • Concurrent infection
  • Impaired somatostatin activity
  • Growth hormone and insulin resistance
  • Interactions with immunosuppressive therapy
• Laboratory values
• • Albumin level
  • BUN level
  • Creatinine level
  • Total lymphocyte count
  • A 24-hour urine urea nitrogen in patients who excrete urine
• Recommendations
• • Energy
  • • Follow the RDAs for the patient's chronologic age, as applicable.
    • Provide additional kilocalories for children who have energy malnutrition to facilitate catch-up growth.
  • Protein
  • • In children treated with maintenance hemodialysis (HD), provide the RDA for the child's age plus 0.4 g/kg/d to achieve a positive nitrogen balance.
    • In children treated with maintenance peritoneal dialysis (PD), provide the RDA for the child's age plus an additional increment based on anticipated peritoneal losses.
  • Sodium
  • • During predialysis, prescribe 23-69 mg/kg/d (1-3 mEq/kg/d).
    • During HD or PD, prescribe 57 mg/kg/d (2.5 mEq/kg/d).
  • Potassium: Patients require 29-87 mg/kg/d (1-3 mEq/kg/d).
  • Phosphorous: Patients require 0.5-1 g/d.
  • Fluids
  • • Administer 35 mL/100 kcal plus urine output plus losses from HD.
    • During PD, give 100-160 mL/kg/d plus urinary output.
  • Vitamins and minerals
  • • Prescribe 100% of the dietary reference intakes for thiamine, riboflavin, pyridoxine, vitamin B-12, and folic acid.
    • Prescribe 100% of the RDA for copper and zinc and for vitamins A, C, E, and K.
• Special considerations: These include management of the patient's acid-base status. When the serum bicarbonate level is less than 22 mmol/L, bicarbonate should be added to the patient's parenteral nutrition formula to avoid the growth-restricting effects of metabolic acidosis.

Liver transplantation

• Frequency: An average of 477 pediatric cadaveric liver transplants are performed annually.
• Morbidity: The 3-month allograft survival rate is 76-86% for all pediatric patients.
• Mortality
• • Approximately 78-88% of children survive 5 years after undergoing cadaveric liver transplantation.
  • Approximately 83-87% of children survive 5 years after receiving living-donor grafts.
• Age
• • During the somatic growth phase, infants are most likely to be malnourished.
  • Children aged 5 years and younger have the highest mortality rate among all patients awaiting liver transplantation.
  • Children aged 6-10 years have the best 5-year posttransplant survival rate (88%) among all patients.
• Race: African-American organ recipients have the worst survival rates at all points of follow-up.
• Malnutrition
• • Reduced energy intake: Ascites, organomegaly, and concurrent infections lead to chronic anorexia and frequent vomiting. These factors, compounded by unpalatable dietary restrictions, almost universally lead to poor intake in patients with chronic liver disease.
  • Fat malabsorption: Interference with intraluminal bile concentration leads to malabsorption of up to one half of the essential polyunsaturated fatty acids and fat-soluble vitamins. By promoting congestion of the gastric and intestinal mucosa, portal hypertension may exacerbate malabsorption.
  • Alterations in hepatic metabolism: Hepatic metabolism of carbohydrates, fat, and protein is disturbed, even in mild liver disease. Reduced hepatic and muscle glycogen stores lead to early recruitment of fat and increased reliance on amino acids as alternative fuels. This alteration in metabolism results in catabolism of muscle, hyperammonemia, hypoproteinemia, diminished glycogen storage and mobilization, hyperlipidemia, reduced circulating triglycerides (because of increased fat oxidation), and hormonal derangements.
  • Increased energy expenditure: Concurrent infections, GI bleeding, operations, and hypercatabolism increase energy requirements by approximately 150% of that predicted by height and weight.
  • Interactions with immunosuppressive therapy: See Nutritional Support.
• Physical examination
• • Muscle mass
  • Jaundice
  • Liver size
  • Ascites
  • Specific signs of nutrient deficiencies (See Clinical manifestations of malnutrition.)
• Laboratory values
• • Serum albumin and/or prealbumin levels
  • Prothrombin time
  • Cholesterol level
  • Fat-soluble vitamins A, D, and E
  • Vitamin K status with coagulation studies (activated partial thromboplastin time)
• Recommendations
• • Energy
  • • In stable patients, use this formula:

      1.1–(1.3 X BEE)

    • In malnourished patients, use this formula:

      1.5-(1.75 X BEE) or 35-40 kcal/kg

  • • To allow for catch-up growth, prescribe 150% of predicted RDA for the patient's height and age.
  • Protein
  • • Avoid restriction. To promote growth and maintain a positive nitrogen balance, 2-3 g of protein per kilogram of body weight per day is recommended.
    • In patients with hepatic encephalopathy that is not attributable to another cause (eg, GI bleeding, infection, dehydration, noncompliance, constipation), restriction to 1 g of protein per kilogram body weight per day may be necessary.
    • Branched-chain amino acid supplementation may improve nitrogen balance of individuals who have severe protein intolerance and whose condition does not respond to aggressive medical treatment of encephalopathy.
  • Sodium (restriction)
  • • Provide 2-3 mEq/kg/d (up to 2 g/d or a no added-salt diet). If ascites is present, decrease sodium intake to 1 mEq/kg/d (up to 0.5-1 g/d).
    • A controlled environment in the hospital may be required for monitoring.
    • Avoid sodium restriction to less than a level that affords palatability.
  • Fluid (restriction): If renal function is normal, restrict fluid to maintenance levels (maximum 1-1.5 L/d), when the serum sodium level decreases to less than 125 mEq/L.
  • Oral vitamin supplementation
  • • Vitamin A, 3000-10000 IU/d
    • Vitamin D, 25-OH 400-4000 IU/d
    • Vitamin E, 25 IU/d
    • Vitamin K, 2.5-5 mg/d
    • Careful monitoring, especially of copper and manganese, to avoid toxicity
• Special considerations
• • In the presence of cholestasis in infants, consider use of semielemental infant formulas that contain medium-chain triglycerides (MCT).
  • This maximizes fat absorption because MCTs do not require micelle formation for absorption.
  • The diet should contain enough linoleic acid to prevent fatty acid deficiencies.

Lung transplantation

• Frequency: Possibly because of improved management of cystic fibrosis, the frequency of pediatric lung transplantation continues to decrease. Children represent approximately 3% of the total population of lung transplant recipients.
• Morbidity: Because of the small number of pediatric recipients, generating statistically significant information regarding graft survival is not possible.
• Mortality: Approximately 134 per 1000 pediatric patients die while on the waiting list.
• Age: Children aged 1-5 years have a 3-fold increased risk of death while on the waiting list. This finding is consistent with the lack of donors for this population and the severity of illnesses for which lung transplantation is considered. The 5-year patient survival rate after transplantation is 54% in infants younger than 1 year compared with 42% in the general population of lung recipients. This suggests that infant recipients of infant lungs may have an advantage over other age groups.
• Malnutrition
• • Poor intake due to dyspnea, early satiety, ascites, or depression (Increased systemic venous pressure and low serum albumin lead to ascites and increased intraabdominal pressure.)
  • Loss of lean body mass due to production of cachexin
  • Increased caloric expenditure related to the excessive work of breathing
  • Chronic infection
  • Malabsorption
  • Nutrient interactions with immunosuppressive therapy
• Laboratory values
• • Albumin and/or prealbumin levels
  • Hgb and Hct levels
  • Serum iron level
  • Transferrin level
  • BUN level
  • Creatinine level
  • Creatinine clearance
  • 24 hour urinary protein
  • Serum glucose level
  • Lipid profile
• Special testing: Measure the patient's bone mineral density at baseline to assess their risk for osteoporosis.
• Recommendations
• • Energy requirements: Patients should receive 120-130% of their BEE.
  • Protein requirements: Patients need to maintain a positive nitrogen balance.

Pancreas transplantation

• Frequency: Pediatric patients are rarely registered for pancreatic transplantation.
• Morbidity and mortality: Meaningful analysis is currently unavailable because pediatric recipients are so few.
• Malnutrition
• • Poor glycemic control
  • Nutrient interactions with immunosuppressive therapy
  • Increased energy expenditure
• Recommendations
• • A moderate, sodium-restricted, low-saturated fat, low-cholesterol diet is recommended.
  • Energy intake should be adequate for weight maintenance.
  • A scheduled plan of steady amounts of carbohydrate intake at regular intervals may be indicated.

Small-bowel transplantation

• Background: Intestinal failure is the inability to maintain nutrition and fluid and electrolyte balance without TPN. When TPN support cannot be maintained because of complications such as advanced liver disease, loss of venous access, or central line sepsis, small-bowel transplantation becomes a therapeutic option.
• Factors contributing to malnutrition include a history of long-term TPN, which puts patients at risk for the following:
• • Metabolic bone disease
  • Essential fatty acid deficiency
  • Ultra–trace mineral deficiencies, including carnitine and selenium
  • Cholestasis
  • Liver dysfunction

PATIENT EDUCATION

Section 5 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

In-depth nutritional education for the child and care providers is indicated. This can be most effectively accomplished via referral to a registered dietitian specializing in pediatric transplantation.

MULTIMEDIA

Section 6 of 7  

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

Media file 1:  Catabolic insult–induced protein-energy malnutrition.
	View Full Size Image
Media type:  Graph

REFERENCES

Section 7 of 7

• Authors and Editors
• Background and Pathophysiology
• Nutritional Support
• Organ Transplantations
• Patient Education
• Multimedia
• References

1. Shepherd RW. Pre- and postoperative nutritional care in liver transplantation in children. J Gastroenterol Hepatol. May 1996;11(5):S7-10. [Medline].
2. National Research Council. Dietary Reference Intakes for Calcium, Phosphorus, Magnesium, Vitamin D, and Fluoride. Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Food and Nutrition Board, Institute of Medicine, National Academies Press: Washington, DC; 1997:[Full Text].
3. National Research Council. Dietary Reference Intakes for Vitamin A, Vitamin K, Arsenic, Boron, Chromium, Copper, Iodine, Iron, Manganese, Molybdenum, Nickel, Silicon, Vanadium, and Zinc. A Report of the Panel on Micronutrients, Subcommittees on Upper Reference Levels of Nutrients. Food and Nutrition Board, Institute of Medicine, National Academies Press;: Washington, DC; 2001:[Full Text].
4. National Research Council. Dietary Reference Intakes for Vitamin C, Vitamin E, Selenium, and Carotenoids. A Report of the Panel on Dietary Antioxidants and Related Compounds, Subcommittees on Upper Reference Levels of Nutrients and Interpretation and Uses of Dietary Reference Inta. Food and Nutrition Board, Institute of Medicine, National Academies Press: Washington, DC; 2000:[Full Text].
5. National Research Council. Dietary Reference Intakes for Thiamin, Riboflavin, Niacin, Vitamin B6, Folate, Vitamin B12, Pantothenic acid, Biotin, and Choline. Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Food and Nutrition Board, Institute of Medicine, National Academies Press;: Washington, DC; 1998:[Full Text].
6. American Dietetic Association. Lung transplant. In: Manual of Clinical Dietetics. 6^th ed. 2000:541-6.
7. American Dietetic Association. Kidney transplant. In: Manual of Clinical Dietetics. 6^th ed. 2000:525-33.
8. American Dietetic Association. Heart transplant. In: Manual of Clinical Dietetics. 6^th ed. 2000:517-23.
9. American Dietetic Association. Small bowel transplant. In: Manual of Clinical Dietetics. 6^th ed. 2000:555-7.
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Article Last Updated: Jun 13, 2008