Chronic disorders of nutrition

1. M.Gorky Donetsk National Medical UniversityDepartment No. 2 of PediatricsHead of the Department Dr. Churilina A.V., Ph.D. Chronic disorders of nutrition Assistant professor Masyuta D.I.

2. Chronic disorders of nutrition are seen predominantly in infants and may be of 2 types: adiposity and malnutrition (hypotrophy).Hypotrophia is the chronic disturbance of nutrition and digestion, characterized by lag of growth and weight and accompanied by disorders of metabolic and trophic processes, decrease of immunity and development of polyhypovitaminosis.Nutritional marasmus and kwashiorkorare two extreme forms of malnutrition. Patients showing features of both syndromes are said to have marasmic kwashiorkor. Such extreme forms account for a small proportion of cases of malnutrition.Patients with mild to severe manifestations of nutritional deficiencies are described as suffering from protein energy malnutrition (or protein calorie malnutrition).

3. Protein energy malnutrition (PEM) is the most often variant of hypotrophy. • Worldwide, malnutrition is one of the leading causes of morbidity and mortality in childhood. By data of WHO there are 20-30% of infants in developing countries who have PEM and other variants of undernutrition.

4. Etiology • Malnutrition is • primary when there is deficiency of food availability • or secondary when food is available but body can not assimilate it for one or another reason.

5. Primary Malnutrition • Alimentary factors. • Quantitative undernutrition. Breast milk may become insufficient for the requirement of the child (hypohalactia). The pediatrician may administrate insufficient quantity of weaning mixtures. • Qualitative undernutrition. Mothers are ignorant about weaning foods, for not only the time at which to wean but also the type of weaning food to be introduced. • Infectious factors. Repeated infections like diarrhea, respiratory infections are the major factors leading to malnutrition.

6. Secondary Malnutrition • Congenital diseases. It may be heart disease (Fallout's tetralogy); congenital lung disease; urinary tract anomalies; malformations of mouth, gastrointestinal tract (ileus, pylorostenosis); anorexia because of perinatal or other brain disease. • Syndromes of malabsorpsion. It may be intolerance of lactose, protein of cow milk; celiac disease; cystic fibrosis; exudative enteropathy, enteropathic acrodermatitis. • Hereditary anomalies of metabolism. It may be galactosemia; leucinosis; fructosemia; diseases of Niman-Pick, Tey-Saks. • Psychosocial deprivation. It means anorexia because of unfavorable factors of surroundings and neuroses.

7. Pathogenesis • Despite of different causes leading to malnutrition, there is disorder of utilization of nutritional substances (protein first of all) both in intestine and in tissues. Activity of gastric, intestine, pancreatic enzymes is decreased. Therefore nutritional load adequate to healthy infant may cause acute disorders of digestion (dyspepsia) in sick one. • Functions of liver, heart, kidneys, lungs are disturbed. • Anemia isn't rare, not only because of protein deficiency, but because of deficiency of zinc, copper, iron, folic acid, pyridoxine.

8. Pathogenesis • Immunologic insufficiency is common in malnutrition and is demonstrated by lymphopenia and anergy to skin test antigens. It leads to frequent infectious processes, which may have latent course. • In mild degrees of malnutrition there are signs of activation of simpatho-adrenal system that is considered as chronic stress; on the contrary, in severe malnutrition functional insufficiency of internal glands activity may occur. • Hypovolemia is typical for all patients. • They are susceptible to hypothermia.

9. Classification • PEM may be classified according to the severity. Classifications are based on anthropometric measurements, mainly weight and height. In our country we use such classification the severity of malnutrition. • 1st degree. If infant has 10-20 % loss of body weight. • 2nd degree. If infant has 20-30 % loss of body weight. • 3rd degree. If infant has more than 30 % loss of body weight. • Besides as to the time of appearance of malnutrition there are a prenatal and postnatal malnutrition.

10. Clinical manifestations • Diagnostics of malnutrition is based on the clinical signs. Expression of the clinical signs depends on degree of malnutrition. • The skin is pale, flabby, in 3rd degree wrinkled because of loss of elasticity. • Subcutaneous fatty layer is thinned • on the abdomen (1st degree), • extremities (2nd degree), • n the 3rd degree of malnutrition is absolutely absent. Buccal pad of fat (clots of Bichaut) may be preserved only. • Decrease of tissue's turgor may be seen. • Muscles are flabby, thinned.

11. Clinical manifestations • Lag of development of motor functions up to temporary loss of static skills is observed. • Lag of growth in 2nd degree malnutrition is 1-3 cm, in 3rd degree malnutrition - more than 3 cm. • Flat weight curve is observed in 1st degree malnutrition. It’s irregular type in more deep degrees of disease. • Decrease of tolerance to food develops. Lowering of appetite and development of dyspeptic disorders may be seen in these children. • Disappearance of monothermia, exceeding of twenty-four hours temperature swing more than 0.5-0.6° C are observed in 2nd and 3rd degree of disease.

12. Comparison of a normal, wasted and stunted child at 1 year. Low weight for height reveals a child of normal height, but who is thin and wasted, whereas low height for age reveals a short, non-wasted child.

14. Boys

15. Girls

16. Malnutrition

17. Marasmus in a 3-month-old baby who was unable to establish breast-feeding because of a cleft palate.

18. Malnutrition

19. Malabsorption postinfectious

20. Clinical manifestations • Laboratory tests show hypoproteinemia, hypoalbuminemia, hypoholesterinemia, aminoaciduria, plane sugar curves and susceptibility to hypoglycemia, acidosis, hypokalemia. • Severely malnourished children may suddenly die due to following causes: hypothermia, hypoglycemia, dyselectrolytemia, diarrhea and dehydration, congestive cardiac failure, infections.

21. Kwashiorkor • Kwashiorkoris a clinical syndrome that results from a severe deficiency of protein and an inadequate caloric intake. Itis the most serious and prevalent form of malnutrition in the world today, especially in industrially underdeveloped areas. • First it was described by Cicely William in 1930. It is derived from Ghanian dialect meaning first second i.e., after birth of the second baby, the first baby is deposed from the breast, which is the only source of protein. • Kwashiorkor means "deposed child," that is, the child no longer suckled. It may become evident from early infancy to about 5 yr. of age, usually after weaning from the breast.

22. Kwashiorkor • There may also be impaired absorption of protein, such as in chronic diarrheal states, abnormal losses of protein in proteinuria (nephrosis), infection, hemorrhage or burns, and failure of protein synthesis, such as in chronic liver disease. • Either from lack of intake or from excessive losses or increases in metabolic rate caused by chronic infections, secondary vitamin and mineral deficiencymay contributeto the signs and symptoms.

23. Kwashiorkor • Kwashiorkor is another manifestation of severe protein malnutrition, in which body weight is 60-80% of expected and generalised oedema is present. In addition, there may be: • a 'flaky-paint' skin rash with hyperkeratosis (thickened skin) and desquamation • a distended abdomen and enlarged liver (usually due to fatty infiltration) • angular stomatitis • hair which is sparse and depigmented • diarrhoea, hypothermia, bradycardia and hypotension • low plasma albumin, potassium, glucose and magnesium.

24. Clinical manifestations • Early clinical evidence of protein malnutrition is vague but does include lethargy, apathy, or irritability. • When well advanced, it results in inadequate growth, lack of stamina, loss of muscular tissue, increased susceptibility to infections, and edema. • The child may develop anorexia, flabbiness of subcutaneous tissues, and loss of muscle tone. • The liver may enlarge early or late. Fatty infiltration of the liver is common. • Edema usually develops early; failure to gain weight may be masked by edema, which is often present in internal organs before it can be recognized in the face and limbs. • Renal plasma flow, glomerular filtration rate, and renal tubular function are decreased. • The heart may be small in the early stages of the disease but is usually enlarged later.

25. Clinical manifestations • Dermatitis is common. Darkening of the skin appears in irritated areas but not in those exposed to sunlight, a contrast to the situation in pellagra. Dyspigmentation may occur in these areas after desquamation or may be generalized. • The hair is often sparse and thin and loses its elasticity. In dark-haired children, dyspigmentation may result in streaky red or gray hair color (hypochromotrichia). Hair texture becomes coarse in chronic disease. • Mental changes, especially irritability and apathy, are common. • Stupor, coma, and death may follow.

26. Kwashiorkor, a particular manifestation of severe protein-energy malnutrition in some developing countries where infants are weaned late from the breast and the young child's diet is high in starch. There is oedema, hyperkeratosis and depigmentation of the skin and redness of the hair.

27. Marasmic Kwashiorkor

28. Kwashiorkor. The infant shows generalized edema, seen in the form of puffiness of the face, arms, and legs.

31. Assessment of nutritional status. This cannot be determined by a single measurement but is a composite of a number of variables.

32. Laboratory Data of Kwashiorkor • Decrease in the concentration of serum albumin is the most characteristic change. • Ketonuria is common in the early stage of inanition but frequently disappears in the later stages. • Blood glucose values are low,but glucose tolerance curve may be diabetic in type. • Plasma values of essential amino acids may be decreased relative to nonessential ones, and there may be increased aminoaciduria. • Potassium and magnesium deficiencies are frequent. • Theserum cholesterol level is low, but it returns to normal after a few days of treatment.

33. Laboratory Data of Kwashiorkor • The serum values ofamylase, esterase, cholinesterase,transaminase, lipase, and alkaline phosphatase are decreased. • There is diminished activity of the pancreatic enzymes and of xanthine oxidase, but these values return to normal shortly after the onset of treatment. • Anemia may be normocytic, microcytic, or macrocytic. • Signs of vitamin and mineral deficiencies are usually evident. • Growth hormone secretion may be increased.

34. Treatment of Malnutrition • In the first place all factors responsible for malnutrition are considered. It's necessary to attempt to eliminate them. • Immediate management of any acute problems such as those of severe diarrhea, renal failure, and shock and, ultimately, the replacement of missing nutrients is essential. • For mild to moderate dehydration, fluids are administered orally or by nasogastric tube (5% glucose solution, glucose-salt solutions). • For severe dehydration, intravenous fluids are necessary (Ringer lactate solution, 5% albumin). • Restoration of normal volume of circulating blood, correction of disordered metabolism of electrolytes, stimulation of protein synthesis are the main tasks of the first two days of therapy in severe malnutrition.

35. Nutritional Treatment • When dehydrationis corrected, oral feeding starts. • Nutritional treatment is based on estimating of the degree of malnutrition. But degree of body weight deficiency and deprivation of appetite not always correlates with seriousness of disease because of lesions of gastrointestinal tract and central nervous system. Therefore, two-phase feeding is the basic principle of nutritional therapy: • 1) period of finding out (ascertaining) food tolerance; • 2) period of high-caloric diet. • Food overload may lead to dyspepsia.

36. The important principles of nutritional therapy • Use of easily assimilated food at the initial stages of treatment (breast milk, or formula) as dysbacteriosis, intestinal lactase insufficiency are often seen in patient with malnutrition • More often food intake (7 - in mild malnutrition, 8 - in moderate, 10 - in severe) • Adequate systemic control of nutrition, stool, diuresis, quantity of fluids taken in and injected, calculation of food load once a week, coprogram - twice a week

37. Period of finding out (ascertaining) food tolerance • Period of finding out of food tolerance lasts in mild cases 1-3 days, in moderate - 3-5 days, in severe - 7-10 days. • Daily food volume in mild cases at first year of life must correspond to infant's age and weight, caloric value - 110-130 Kcal/kg/day • In moderate and severe cases - initial food volume 2/3-1/2 of due volume, caloric value - 100-95 Kcal/kg/day. • In very severe cases its necessary to begin with daily quantity of breast milk of 60 ml/kg and increase it by 20 ml/kg/day. • Protein load in severe malnutrition is 0.6 g/kg. • It is important to secure increase of weight from 3-4 day by 20 g/day and more.

38. Period of high-caloric diet • During this period in mild malnutrition an infant receives approximately 140-160-180 Kcal/day, in moderate and severe cases - 180-200 Kcal/day. • Proteins consist 10-15% of caloric value (in healthy infants - 7-9%), or 3.5-4 g/kg. • Augmentation of food load is made after its calculation and under control of coprogram (once per 3-4 days). When high-calorie and high-protein diets are given too early and rapidly, the liver may become enlarged, the abdomen becomes markedly distended, and the child improves more slowly.

39. Treatment of Malnutrition • Vitamins and minerals, especially vitamin A, potassium, and magnesium, are necessary from the outset of treatment. Iron and folic acid usually correct the anemia. • Bacterial infections must be treated concomitantly with the dietary therapy. Effective antibiotics should be given parenterally for 5-10 days. Whereas treatment of parasitic infestations, if they are not severe, may be postponed until recovery is under way. • All mentioned above - schema of nutrition therapy of patients with malnutrition - exists, but individual approach to diet is necessary for every sick child.

40. Organization of nursing • It's possible to treat patients with mild malnutrition at home. Moderately and severe malnourished children should be treated in the hospital. • The patient has to stay in light, spacious, regularly ventilated room. • Air temperature must be not lower than 24-25°C, but not higher than 26-27°C, as infants may be overheated or supercooled easily.

41. Organization of nursing • In absence of contraindications to walks it's useful to go for a walk several times a day when temperature isn't lower than -5°C. • In autumn and winter hot water bottle on the legs is recommended. • It's very important to create positive emotions, to pay attention to prevention of cross infections, to bath a child every day (t° 38°C). • Massage and gymnastics are necessary as well.

42. Prevention • The prevention of PEM in young children is based on • improvement in the diet in pregnancy and early childhood (based on breast feeding and locally available weaning mixture), • prevention and early management of microbial and parasitic infections, • minimization of emotional trauma following cessation of breast feeding.

43. Pyloric stenosis • In pyloric stenosis, there is hypertrophy of the pylorus causing gastric outlet obstruction. It presents at between 2 and 7 weeks of age, irrespective of gestational age. It is more common in boys (4 : 1), particularly first-borns, and there may be a family history, especially on the maternal side. Clinical features are: • projectile vomiting (not bile-stained), which increases in frequency and severity with time • constant hunger even after vomiting; only when markedly dehydrated do they refuse to feed • a hypochloraemic alkalosis with a low plasma potassium from vomiting acid stomach contents • weight loss or poor weight gain if presentation is delayed.

45. Visible gastric peristalsis in an infant with pyloric stenosis.

46. Pyloric stenosis

47. Ultrasound examination showing pyloric stenosis.

48. Diagram showing a test feed being performed to diagnose pyloric stenosis. The pyloric mass feels like an 'olive' on gentle, deep palpation halfway between the midpoint of the anterior margin of the right ribcage and the umbilicus.

49. Pyloric stenosis. Thepylorusis enlarged and the duodenalbulb is indented, consistent withhypertrophy

50. Pyloric stenosis at operation showing pale, thick pyloric muscle and pyloromyotomy incision.