ACTA FAC. MED. NAISS. 2003; 20 (2): 109-113

Review article

NUTRITIONAL SUPPORT IN CHRONICALLY ILL CHILDREN

 

Simić Dušica¹, Budić Ivana²

 

¹ University Children’s Hospital Belgrade

² Clinic for Pediatric Surgery and Orthopedics, Clinical Centre, Niš

 

 

 

Nutritive requirements estimation

Basal metabolic requirements (BMR) in childhood could be calculated based to the Schofield equation  (15) (Tab1). In clinical practice REE (resting energy expenditure) value is close to BMR, but in essence REE includes: BEE + thermogenesis without shivering and hypermetabolic stress.  Difference between REE and BMR is estimated to be around 10%.

Overall metabolic requirements could be calculated when basal requirements are multiplied with stress factor that is dependent on the disease difficulty.

Proteins requirements are usually bigger. Estimation is based on nitrogen balance (13). Nonprotein energy  (kcal) and protein intake (gram N)  ratio should be between 150 and 250:1.

 

 

 

Cardiac diseases

Nutritive assessment of the pediatric patients with congenital cardiac diseases  includes determination of the lesion type. Cyanotic congenital cardiac diseases are: transposition of the great vessels, Fallot’s tetralogy, tricuspid atresia, persistent truncus artheriosus, complete anomaly of venous turnover, pulmonal atresia, Ebstein’s anomaly, hypoplastic  syndrome of the left heart. They are connected with growth retardation and impaired body weight gain. Heart minute volume is not enough for the effort of feeding.   The lagging in growth and skeletal maturation is related to the degree of hypoxemia. Tachypnea interfere with normal food intake. For that reason, it is sometimes necessary to start with enteral feeding through nasoesophageal feeding tube.  Cyanosis is connected with impairment of nursing and swallowing, and often with vomiting. This conditions show that minute volume is insufficient and that saturation is decreased. These children need supplementary calorie intake (60 kcal/kg/day) compared to the values in table. 

Acyanotic cardiac diseases are atrial septal defect, ventricular septal defect, patent ductus arteriosus, arteriovenous channel, pulmonary stenosis and aortic coarctation. They lead to grater decrease in body weight gain  than in growth (1).

Pulmonary hypertension is the most important cause of malnutrition and growth retardation (2). Timely done surgical correction of  congenital cardiac disease and resultant hemodynamic  improvement hurries patient growth.

The minute volume is decreased in congestive heart failure. This condition diminishes  renal and mesenteric circulation. As a result, gastric emptying, absorption and motility of bowels is disturbed. All this, together with tachypnea, renal failure and digoxin usage determine that volume of meal should be diminished.

In children with acyanotic cardiac diseases total energy expenditure is increased (basal metabolism is not accelerated) as a result of increased energy usage during physical activity. Body weight doesn’t grow  because caloric needs can’t be supplied (3).

The consequence of  Fontan operation could be enteropathy characterized by protein, transferrin, ceruloplasmin, fibrinogen, lipoprotein, alpha1 antitripsin, trace elements and Ca lost. Decrease of oncotic pressure causes mucous membrane edema and leads to malabsorption. Venous and lymphatic obstruction results in edema, ascites and lymphopenia. Diet should be extra supplied with vitamins and the quantity of LCT (long-chain triglycerides)  must be reduced. MCT (medium-chain triglycerides) are favorable because of their direct absorption from portal vein. Lipid-soluble vitamins should be replaced.

 

Tab.1. Schofield equations for basal metabolic requirements (BMR) estimation in children

 

          Tab 2. Stress factor                                                

                                 

                                                                                    

                             

                                          

Patients with diuretic and cardiotonic therapy should undergo careful potassium concentration assessment. A low serum potassium causes increased cardiotonic toxicity and diminishes  renal concentration ability.

Sufficient nutritional support is achieved by cardiac function improvement, drug side-effect reduction, nutrition deficit assessment and  adequate energy  supplying  provided via an enteral or parenteral route.  Continuous monitoring is very important.

Nutrition support recommendations  for the children with cardiac diseases:

1.      increase energy intake from 75-120 kcal/kg/day (for maintenance) for 20-100% (growth, surgery)

2.      increase caloric density of diet, reduce water percentage, increase protein (8-10%), carbohydrates (35-65%), and lipids (35-50% LCT or MCT) quantity.

3.      reduce liquids intake

4.      reduce sodium intake to 2.2-3mEq/kg/d

5.      monitor electrolytes concentration, especially potassium (2-3mEq/kg/d)

6.      keep urine osmolarity    under 400mosm/l

Contraindications for enteral feeding are:

1.      decreased minute volume or unstable physical condition

2.      patent ductus arteriosus dependent lesions with decreased mesenteric circulation

3.      recent reanimation

4.      intubation or extubation within last 4 hours

5.      ectopic tachycardia

6.      intestinal obstruction

7.      gastrointestinal bleeding

If enteral route is contraindicated, nutrients are given parenteraly.

 

The goal of nutritive therapy is not only to improve growth in children with cardiac diseases. Nutritional support should prevent cardiac disease development. In that sense, education in adequate, balanced nutrition should be organized, prevention of obesity in childhood should be done, and everyday physical activity should be practiced.

 

Renal diseases

Children with chronic renal failure have many differences  in nutritional needs. Renal failure is a pan-metabolic and  pan-endocrine abnormality affecting more or less every metabolic pathway of the body. When glomerular function falls below 50% of normal values secondary hyperparatireoidism is developing. Synthesis of 1.25(OH)2D3 is decreased and metabolic acidosis occurs. Osteodistrophy, growth retardation, anemia, Na, K and water retention develop when renal function falls below 25%. Growth retardation is a consequence of malnutrition (it points out the importance of adequate nutrition in these patients). If chronic renal failure is caused  by interstitial processes (obstructive uropathy, nephritis et.) there is inability in renal ability to concentrate  urine, there are losses of water and water-soluble vitamins, and by all this dehydratation could happened. In addition to everything else, different disturbances such as malnutrition, hypermagnesemia,  edema, calculosis could happened (1). The aim of nutritional therapy is to achieve normal nutrition despite the fact that there are some limits provoked by impaired renal function.

Energy and protein needs are estimated by the table values based on the normal height for the age. This valued ere increasing gradually.  In these patients, malnutrition is provoked by anorexia and by insufficient calories intake. Introduction of enteral feeds during the night, through the nasogastric feeding tube, leads to the better growth. It is advocated that it should decrease intake of solutions (according to the metabolic status and renal function), use formulas with higher caloric content characterized by appropriate ratio between fats and carbohydrates. Too much carbohydrates shouldn’t be given because they could provoke hypertriglicerydemia  and increase the risk of vascular complications development. Energy needs should be mainly supplied by partially unsaturated fatty acids (PUFA).

Although sometimes is necessary to reduce proteins intake because of renal function disturbances, there is no evidence that reduced proteins intake influence the course of disease in children. By the way, there is very important characteristic of the children’s age – normal growth and sexual function development must be accomplished – these two processes require a lot of proteins. In  chronic renal diseases proteins with large amount of essential amino acids  should be given. If oral intake becomes insufficient, preparations for oral (EAK, Zdravlje, Leskovac) or parenteral nutrition could be applied. Every patient should be treated according to the specific needs, based to the history of illness and nutrition requirements, with continuous monitoring of laboratory parameters and clinical examination (13).

Vitamins and minerals intake is assessed according to the blood values of urea, creatinine, NA, K, glucose, P, Ca, Mg, bicarbonates, hematocrit, triglycerides and cholesterol. Na intake is reduced only if hypertension, edema, circulatory congestion exist  or if the acidosis treatment is based on the large amount of given barbiturates. If the lost of salt is increased (nephritis, diuretics) supplemental intake should be provided. In hyperkalaemia state, potassium intake should be decreased, acidosis should be treated and/or ionic  exchange resins should be used. In the presence of the high serum magnesium, antacid and Mg contain purgative intake should be monitored carefully.

Osteodystrophy        is treated by Ca carbonate or acetate administration, which  is followed by decreased intake of phosphate (P). The goal of this therapy is to keep the level of  P on the bottom line  specific  for the age. Administration of vitamin D analogs in obese children could be associated with their accumulation in fatty tissue by consequent release which could leads to the intoxication (12).

Anemia is  solved by erythropoietin and Fe administration. If calculosis exists, it is wise to omit vitamin C and spinach in diet, to avoid chocolate and other food which contains oxalats in larger extent.

If parenteral nutrition is recomanded, K, Mg, and P should be avoided at the begining until adequate excretion has been established. 

Vitamin A and Se are excreted by the kidneys and their level should be followed. Vitamin A and liver in diet are forbidden.

Lost of proteins during peritoneal dialysis could lead to malnutrition. Usage of intradyalitic parenteral nutrition is investigated at the moment (5).

In children on hemodialysis, frequent dialyses with adequate nutrition could normalize growth (6).

After the transplantation, renal function is reestablished and many metabolic disturbances disappeared. On the other hand, immunosuppressive drugs (prednisone and cyclosporine) lead to hyperkalaemia, , hypertension, glucose intolerance, gastritis and increase appetite.

In most cases diet without salt and supplemented by phosphate is recomended.

 

Liver disease

Since liver has a major role in metabolism, every disturbance of its functioning decrease absorption and utilization of nutrients. Parenchymal liver damage seen in biliary  atresia, autoimmune or chronic infectious hepatitis decrease synthesis of biliary salts, proteins and carbohydrates (7). Stenosis or obstruction of biliary ductule’s decreases presence of bile in bowels and diminishes fats absorption. Steatorrhea leads to weigh lost. TPN (total parenteral nutrition) in neonate and infant could provoke cirrhosis and liver inefficiency (8). In children with biliary ductule’s atresia, nutritive deficiency develop early so, continuous monitoring and early intervention is advocated.

These children are often anorexic and must be fed trough the feeding tube with preparations reach in MCT (Portagen) or parenteral nutrition must be introduced.

Condition of the child with chronic liver disease must be followed  on a daily bases. Energy needs are adequate with values given in the table but could be 50% higher. Because of the lack of the biliary salts and acids, MCT should represent 75% of triglycerides quantity (12). If portal hypertension exists and if digestion products shunt to the systemic circulation, MCT should use carefully because ketosis could be provoked (12).   If there is a need of enteral nutrition in patients with portal hypertension, silicon feeding tube must be used because there is risk of bleeding from varices esophagi. There is no limit for carbohydrates intake. Proteins intake  decreases only in case of threatening hepatic encephalopaty  and then is  useful usage of  branchy chain  amino acids although their usage in clinical practice is under the question mark.

Malabsorption of proteins could happened as a result of protease inactivation caused by low  pH in the bowels. For that reason, protein hydrolysates  are used in nutrition (12). Infant should be given Portagen (Mead Johnson) or Pregestimil (Mead Johnson) with 85 or 55% MCT. In children over 1 year of age Peptamen junior (Nestle) and Vivonex Pediatric (Sandoz) are the best choice.

If there is an ascites, the content of Na should be decreased. But, there is a problem because children don’t like to eat unsalted food. Meals should be smaller several times per day because gastric distention could provoke pain.

Dosage of vitamin A and D should be doubled with  supplemental vitamin K (12). In Wilson disease intake of Cu should be decreased (milk contains Cu in a great extent).

Many drags used in liver diseases could influence nutrition status. Holestiramin (which is given in a case of pruritus in cholsestasis) bonds biliary acids and increase fat absorption and absorption of lipid-solved vitamins. In children who are given this drug lipid-soluble vitamins should be compensated and MCT should be provided.

Phenobarbital, which is given to hurry bile excretion , changes vitamin D metabolism and after long usage could lead to rachitis.

Neomycin, given in a case of hepatic encephalopathy could damage small bowel mucous membrane and cause steatorrhea, decreased carbohydrates absorption and vitamin B12 deficit. Because of that, there is a need for MCT and B12 substitution. Lactulosis could be given in the case of complication like this. It has, as the unwanted effects, osmotic diarrhea and increased Na and K lost by the stool.

Liver transplantation is an adequate therapy for liver cirrhosis in children. In that case, perioperative nutritive support has direct influence on this patients survival (14).

 

Cystic fibrosis  (CF)

 

Patients with cystic fibrosis have thick, viscous mucus , especially in lung and pancreas,  that blocks airways and pancreatic channels. As a result, chronic obstructive pulmonary disease and pancreas insufficiency occur. In these children, nutritive support is the most important factor because it not only improves nutritional status but also respiratory and pancreatic function.

Growth is limited because increased energy consumption caused by chronic cough, recurrent pulmonary infections and decreased oxygen saturation. Basal metabolism is increased to 104-130% of normal values.

Pancreas insufficiency, biliary salt and acid disturbances, vomiting caused by coughing are major factors in the etiology of nutritional depletion. Pancreatic enzymes deficit  is complete in 80% of patients, partial is in 10%, and almost normal is in 10% (11). Proteolytic enzymes deficit and hypercatabolism induce hypoalbuminemia. 

Calort intake is decreased as a result of muscle fatigue, anorexia and depression. In 25% of patients malabsorption of carbohydrates occurs because of amylase deficit (11). But, when are not in acute phase of illness, these children intake more calories than healthy children of the same age (9). Steatorrhea happens because of lipase deficiency and intestinal mucose inability. Together with lipids lipid-soluble vitamins are lost, and that deficiency should be supplemented in all patients with CF.

From the facts noted above, these children are prone to malnutrition and growth retardation, and nutritive support is based on malabsorption compensation and enzyme application.  In infants the most optimal nutrient is mother milk supplemented by enzymes. Satisfactory results could be achieved by soybean and cow milk or hydrolysates, also given together with enzymes. Proteins intake shouldn’t be increased above normal values because renal function could be affected. Renal function is already compromised by aminoglycoside usage.

There are different opinions according lipid intake, but the conclusion is that it should be normal (1). 35-40% of calorie intake is in LCT form (long-chain triglycerides). MCT could be used also, because lipase or biliary acids are not needed  for their absorption. This kind of solution is expensive and doesn’t taste good.   If child’s height  falls below third percentile, or if there is a growth retardation enteral feeding is necessary (polymeric formula supplied with pancreatic enzymes or semi elemental diet) (14).

During theirs lives, patients with CF become intolerant to glucose. Diabetes could be asymptomatic. They continue to take hypercaloric  food with the same quantity of carbohydrates, but insulin or oral hypoglycemic are applied.

Water-soluble   vitamins should be restitute via oral route. Salts are compensated during the hot days when sweating is increased. They are susceptible to osteoporosis, and need vitamin D and Ca in their diet.

Pancreatic preparations generally contain lipase, amylase and tripsin.  In commercial use they are in powder or enteral capsule form, and should be given with every meal.

Bicarbonates production is decreased. They should be given with H2 blockers in order to decrease gastric and small bowel acidity.

Nutritive supplementation improves nutritive status and respiratory function. Nutritive support and better infection control contribute to longer survival of this children.

 

AIDS

In children with HIV infection, malnutrition cause immunity deficiency, intestinal infections and  malabsorption. These conditions aggravate  immune functions, opportune infection occur  more frequently,   malignancies affecting earlier and wasting is more obvious.   Complications include growth retardation, body weight decreasing and numbers of nutritive deficits. When body weight start to decrease and when growth retardation is noticed, nutritive assessment  should be done. This assessment should be repeated every 6 month. In this children “lean body mass” is mostly decreasing, even when there is no evidence of body weight lost. For that reason anthropometric measurements are very important (sceletal muscle mass, mid-upper arm circumference).

The most important for optimal immune function is to maintain nutritive status. The goal of nutritive support in these children is to provide adequate calories intake, adequate content of nutrients and to increase malabsorption. By all this, “lean body mass” should remain unchanged and normal growth and development provided. The point is that high caloric diet should be applied as soon as possible.  

The major causes of malnutrition are intake decreases coupled by increase of loses in the same time when needs are increased too (1). The reasons for this condition are illness itself together with side effects of medicaments given as a therapy. Decreased intake is a consequence of  oesophageal candidiasis, oesophageal herpes simplex ulcers, gastro-intestinal infections with m. avium, gastritis, duodenitis, anorexia, depresion, pain, neurological disturbances,  changes in taste (side effect of medications or Zn deficiency), aversion to the food or poor economy situation.  Increased losses are due to vomiting, diarrhea (medications), pancreatitis and malabsorption. Increased requirements are caused by fever, infection, catch-up-growth state, cytokines production and endocrine and metabolic disturbances. HIV virus itself increases the level of basal metabolism. Fever and recurrent infections should be treated.

If appetite is decreased, meals should be caloric and small quantity of food should be given more frequently. Drugs that stimulate appetite could be prescribed, and vitamins and minerals added. If the early satiety occurs,   meals must be really small and frequent, and medications that improve intestinal motility should  be applied.

Children with AIDS early develop lactase deficiency, and in a case of diarrhea and meteorism lastase intake must be stoped. Protein hydrolisates with MCT could be useful too. Steatorrhea could be resolved with pancreatic enzymes supplementation.  Vitamins and minerals should be given in dosages that are twice than usual.

If is impossible to correct malnutrition oraly, it should start with enteral nutrition via nasogastric feeding tube or through the gastrostomy. If intestinal mucosa is swollen PN is indicated (10). Growth and development maintenance is one of the greatest goal of nutrition support in children affected by AIDS.

 

Conclusion

Nutrition support provided during the chronic illness increase the ability of the child to achieve inherited genetic potential.  Sometimes, there is no clinical evidence of improvement, mostly because nutritional assessment was inadequate or the nutritional therapy started too late. It shouldn’t be neglected that nutrition therapy is a dynamic process and it should estimate the nutrition requirements continuously during the child growth and development. In some cases reasons for failure could be find in the child organism that sometimes doesn’t respond to therapy procedures. Nnutritive support is necessary in chronically ill children because chronic disease influence on digestion and absorption. Improvement of child condition decreases the need for nutritional support. 

 

References

 

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