Сhair of Children’s Surgery and Propaedeutic of Pediatrics

Theme of the lecture:

“Objective laws of age changes in energetic exchange in children. Peculiarities of protein

metabolism and semiotics of its impairment in

children.”

Plan Of The Lecture:

Actuality.Introduction.Characteristics of the main exchange in children.Energetic need in different age periods.Peculiarities of energetic exchange in children.General notion about diseases of exchange of substances.Peculiarities of protein exchange in children.Semiotics of protein exchange in children.Conclusion.

The actuality The actuality Energy spending by a child organism is achieved by high level of oxidation and phosphorylating processes.In children in all age periods, especially in the first years of life, the main exchange is much more intensive than in adults. Considerable amount of energy objectively is spent on the processes of growth and assimilation.So, energetic and oxidation processes in the child’s organism are going on more intensively, which are witnessed by the indications of the main metabolism.

Peculiarities of energetic exchange of substances in children

Energetic exchange in children has peculiarities, dependent on:intensive growth;high need in the main ingredients;high level of biosynthesis;immaturity of some regulatory systems;age peculiarities of fats, proteins, carbohydrates exchange.

Peculiarities of energetic exchange of substances in children

Hippocratus marked long time ago that …” a growing organism has the largest amount of natural warmness and so requires more food”.Actually, the child’s organism in the conditions of intensive growth for normal life ability needs relatively more plastic substances and energy, which are produced in the result of exchange of organic complexes entering with food.

Energy spending by a child organism is achieved by high level of oxidation and phosphorylating processes.In children in all age periods, especially in the first years of life, the main exchange is much more intensive than in adults. Considerable amount of energy objectively is spent on the processes of growth and assimilation.So, energetic and oxidation processes in the child’s organism are going on more intensively, which are witnessed by the indications of the main metabolism.

Peculiarities of exchange of substances in children

It is necessary to stress the imperfectness of exchange processes regulation, connected with age on the side of central nervous system (CNS) and glands of internal secretion as well as on the side of neurohumoral mechanisms.Everything this determines instability of exchange of substances in children and easiness of its disturbances.

Peculiarities of exchange of substances in children

Together with the above said, in childhood age every kind of exchange processes is peculiar – protein, fat and carbohydrate exchange.The knowledge of them enables correct orientation in the problems of feeding babies of the first months and years of life, and also in peculiarities of pathology, caused by violation of exchange processes, based very often on genetically determined diseases.

newborn child 38-42 1 month 44-46 1 year 55-60 2 years 55-56 5 years 42-44 7 years 38-42 10 years 36-37 14 years 31-33 adults 23-24

The main exchange in children in kcal. on 1 kg of body mass

Formation of energy from the food products

Disintegration of large molecules on little ones (hydrolysis). From carbohydrates are formed 3 gectoses (glucose, galactose, fructose); from proteins- 20 amino acids; from fat triglycerids - glycerin and fatty acids.Disintegration by incomplete burning - formation of CO2, H2O, oxalic-acetic and acetic acids (30% of energy is saved).Disintegration in a cycle of tricarbonic acids (Krebas cycle) to the end products – CO2, H2O, 60-70 % of energy is saved.

Saving energySaving energy is achieved on the account of transformation of food products disintegration energy into a specific form of chemical compounds - macroergs, phosphorous compounds.Central place is occupied by ATP - 60-70 % of energy in the organism.For 24 hours in the organism is disintegrated and formed ATP in the amount of body weight.

The formed energy is spent

on: The main exchange (OO) - this is minimal amount of energy, necessary for supporting life activity of the organism in the conditions of full muscular and emotional rest.Plastic exchange (for the growth of the child).Digestion and absorption of food products.Activity of muscular system.

Protein exchangeProtein is plastic material for tissues, its violation causes many disturbances.Protein is one of the most important life needed products. The daily need in proteins is considerably high. Fats and carbohydrates cannot substitute proteins. Even a small disturbance in protein exchange may cause serious problems for the health.

The functions of protein in the organism

Plastic - in disintegration of protein amino acids are formed, so necessary for the organism life ability. Amino acids are the basis for the synthesis of new protein molecules.Immunologic - immunoglobulins and immuno comopetent cells are protein structures. Energetic - (1g-4 kcal) comprise 10-15% of a day caloricity.Proteins are ingredients of enzymes, hormones, vitamins, hemoglobin, etc.Proteins are buffers keeping oncotic pressure in the blood and cerebrospinal fluid.

Peculiarities of protein disintegration

Proteins of the food eaten, are disintegrated in gastro-intestinal through enzymes into amino acids. In small children this process is rather slow because of low activity of pepsin.In breast milk protein is partially disintegrated and for 50% is composed of ready plastic material.

The need in proteinThe need of breast fed babies in protein:2-2,5 g/kg/day - in natural feeding;3-3,5 g/kg/day - in mixed and artificial feeding.It is important not only to supply protein amount, but also the quality of it. For children 10 amino acids are indispensable. Up to 5 years the most important amino acid is histidine.

Digestion of proteins

protein peptides

pepsinogen pepsin рН 1-3

Stomach

Proteins in blood plasmaThe contents of protein in blood plasma - 57-81 g/l.In newborns - 47-65g/l.In premature children - 50 g/l.Proteins in blood plasma are renovated every day for 25%.Albumins are well soluble. They contain 580 amino acids.

Functions of albumins:regulation of osmotic pressure (1g of albumin connects 18 ml of water);transport of substances, which are badly soluble in water (fatty acids, bilirubin, steroid hormones, thyroxin, adrenalin, calcium, copper, vitamins, toxins);albumin -depot of amino acids.

Globulins – – αα1 1 -, α-, α22 -, β - ,γ – -, β - ,γ – fractions Functions of αα11 globulin:

transport of lipids; transport of thyroxin; transport of measles hormones of adrenal glands.

Functions of αα22 globulin:

transport of lipids ; transport of copper (ceruloplasmin connects Cu);B fraction of protein includes lipoproteins, transferring, C-reactive protein;Y-globulin fraction of protein contains the most number of antibodies, these are immunoglobulins A, M, G, D, E.

Laboratory findings of protein exchange disturbances

In clinics the following changes in proteinogram are differentiated:

hyperproteinemia (under blood clotting);hypoproteinemia - starving, diseases of the kidneys;dysproteinemia - acute and chronic inflammatory processes.

The indication of protein exchange are the end products of protein disintegration:

urine (N – 3,33-8,33 mmol/l);creatinin (N – 0,04-0,1 mmol/l, 1-2 mg%);resting nitrogen (N- 14,3-28,6 mmol/l).

The reasons of protein exchange violation are:

alimentary factor (in cases of underfeeding of a child); infectious;anorexia as a result of perinatal pathology:failure of oral cavity and gastro-intestinal tract development;syndrome of “short bowel” after massive resection of intestines;malabsorption syndrome;hereditary anomalies of metabolism.

Manifestation of protein exchange violation

hypotrophies and immune deficiency states;children are more sensitive to protein starvation than adults. Long protein starvation in children younger than 3 years can cause unreparable changes, even lead to death. Adults who suffered protein starvation in childhood react to stresses more painfully.

Disturbed protein absorptionIn N – excretion of protein into the intestinal lumen is absent.

Malabsorption

Violation of CO barrier of intestine entering of protein into

intestinal lumen

violation of amino acids absorption violation of albumin synthesis

hypoproteinemiaedemasascitis

Malassimilation syndrome, there are 70 diseases

Types:pancreatogenic;enterogenic;gastrogenic ;hepatogenic;immunogenic

(neurogenic, endocrine, iatrogenic).

Important to remember that

practically in all diseases processes of absorption and digestion are disturbed

Clinical signs of protein deficiency

Excitement, weakness, apathy.Subcutaneous fatty layer is decreased.Body mass grows very slowly.Edema syndrome (edema of the feet).Depigmentation of the hair.Dermatitis.Hepatomegalia.Muscular tiredness.

Disturbed protein exchangeMost frequently the following disturbances are present:hypotrophy;immune deficiency states;these diseases are life thretening for a child;less frequently are present congenital and hereditary disturbances in protein exchange.

Hypotrophy – chronic violation of nutrition

hypotropy is caused by coming into the organism of insufficient amount of food or disturbance in its assimilation;clinical picture is characterized by deficit of body mass of a child. Hypotrophy is met more frequently in children up to 2 years;according to time of arising hypotrophies are: congenital (intrauterine, prenatal) and acquired.

Hypotrophy

a - normotrophy; b - hypotrophy 1 degreec - hypotrophy II degree d - hypotrophy III degree

Severe, combined immune deficiency (SCID) - Switzerland type of immune

deficiencyCharacteristic features:

- the disease is manifested in the first months of life;- is inherited as autosome-recessive or taken in vaccination

(75% of patients are boys);- low body mass, anorexia, uneasiness;- measles type of rash on the skin;- diarrhea, not reacting on dietotherapy;- susceptibility to upper respiratory diseases, pneumonias;- infections of DNK viruses can lead to lethal outcome.

Agammaglobulinemia of BrutonThe disease was first described by Bruton. There is no gammaglobulin fraction in the blood;general amount of gammaglobulin in the blood is less than 2g/l. The disease is met only in boys. The disease is manifested in the first months of life.

Agammaglobulinemia of Bruton patients are not resistant to staphylococcus, streptococcus,

pneumococcus, often are met parasitary pneumonias;the children frequently suffer from otitis, pyodermia, recurrent pneumonias, often leading to sepsis. High susceptibility to poliomyelitis virus.

The problem of hereditary disturbancies in exchange of substances

The great ancient poet Dante A. in 13th century wrote: “When God created a man devil added a spoon of ointment.” It is a genetic “spoon of ointment.” Every doctor must know about hereditary diseases.

Kwashiorkor - quantitative and qualitative protein deficiency

Kwashiorkor - described by Wiliams in 1933, synonims - “ a red child”, “changing skin”. Arises after stopping to feed with the breast milk, when in the diet there are no milk products, which contain amino acids. Clinical signs - weakness, apathy, lagging behind in neuro-psychic and physical development, edemas, dermatitis, hepatomegalia, immune deficiency, infectious diseases of centralized type, poli hypovitaminosis.

Clinical syndromes caused by disturbances in splitting and absorption

Celiac disease (Gluten – susceptible enteropathy)

Chronic genetically determined immune mediated diseases with damage

to small intestineand systemic autoimmune manifestationat a background of gluten

intolerance 

First symptoms appear after introduction of additional food (bread, cereals, cookies).change of mood, loss of appetite, low body mass, polifecalia in unchanged frequency of defecations.In 2-4months (or later) lagging in physical development;meteorism;enlarged abdomen at a background of decrease of body weight;spontaneous fractures of the bones;constipations and diarrheas.

Typical celiac disease (6-24 months) Т

Typical celiac diseaseManifestation of clinical picture - 2-3 years of life: general skeletisation up to cachexy; anorexia, vomiting, pain in the abdomen, meteorism;enlarged abdomen;dryness of skin covering and salivary glands;stool – in much amount, light, with bad smell, loss of fat (60%).

assessment of clinical picture and anamnesis;serological investigation : IgA and IgG, AGA, AEMA, TTG.instruimental stage (biopsy);additional stage: presence of genetically markers HLA DQ2/DG8.

Difficulty in diagnostics !!!

Intolerance of cow milk protein

After introduction of cow milk:bad appetite;lagging in body mass increase;increase of the amount of stools.

In 3-4 months – increase of the symptoms:decrease of body mass, meteorism, decrease of abdomen

size; intestinal impairment. 

In 2-3 years:clinical signs can selfdependently disappear;morphological changes last longer.

Excudative enteropathy(Syndrome of intersticial loss of lymph, protein loosing enteropathy, protein diarrhea, intestinal

lymphoangiectasia)Loss of plasma proteins through gastrointestinal

tract with development of signs of damage togastric absorption: -diarrhea; -hypocalciemia; -hypoproteinemia; -coprogram – steatorea of II and III type.

Disturbed amino acids exchange (amino acidopathy)

are connected with genetically determined enzyme deficiency and as a result toxic metabolites accumulate in the organism;common signs: special smell of urine, lagging in psycho-nervous development, convulsions, low muscular tone, vomiting, ketonuria.

Phenylketonuria Children sharply lagg in psychic development. Syptoms appear in the 1st year of life. The skull is small, in 50% convulsion attacks are observed. Increased muscular tone. Other failures of development can be observed.Urine has a smell of a mouse. Treatment is dietary. Diet is administerd . It contains food rich in vitamins and protein hydrolisates.

Phenylketonuriafrom ration are fully excluded products containing phenylalanine ( meat, fish, liver, eggs, cheese, rice, cacao, chocolade, etc.).The child can eat: sugar, fruit, honey, oil, milk, vegetables, potatoes. Vitamins, nootropic medicines, anticonvulsives are administered. There are special mixtures not containing phenylalanine.

CystinosisCystinosis develops as a result of damage to sulfur containing amino acids exchange. Cystine is distributed in all reticuloendothelial cells of all organs. The children lag in the growth and body mass. There is vomiting, constipations, osteoporosis . Such children live not long and die of intercurrent diseases, kidney insufficiency under toxic syndrome with dehydration of the organism.For treatment anabolic steroids, ATP are used. In cases of toxic syndrome fight against dehydration and acidosis is carried on.

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Homocystinuria – deficit of cystathionin-cyntetasa enzyme

leads to accumulation of homocystin in the organism, which is excreted with urine. In the breast feeding age the disease is not manifested. The main symptoms: skeletal anomalies (long, thin extremities and fingers, scoliosis. Deformed chest cavity), disturbed eye sight (cataracta, glaucoma)Treatment - large doses of vitamin B6 activate deficit of enzyme. In B6 resistent homocystinuria - dietotherapy (excluding from diet products containing metionin) is performed.

Histidinemia - the basis of the disease is deficit of histidasa enzyme.

Accumulation of histidine and its products has negative effect on the CNS.Clinical manifestation is seen at different ages. The main manifestation is psychic underdevelopment. Characteristic disturbances are violation of behavior and attention, convulsions, emotional lability. Violation of speech is seen in 50% of patients. Diagnosis is based on increased level of histidine in blood serum.

Alkaptonuria - deficit of oxiudase homogentysine acid.

Clinically is characterized by 3 symptoms: darkening of urine; pigmentation of cartilages and connective tissue;arthropathy.

Diagnosis is based on presence of homogentisine acid in the urine. Treatment is based on symptoms. Vitamin C is one of the best medicines.  

Violation of exchange of other amino acids

Hypermetionemia – urine with the smell of fish, connected with the p resence of butir acid.Violation of leucin metabolism. Urine with the smell of spoiled oil and fetid sweat as a result of accumulation of isovaleriane acid.Glycenosis - urine has a smell of apples.

Summary So, disturbed exchange of protein can be on the level of hydrolysis, absorption, transport, metabolism, enzyme deficiency (amino acidopathy).Violation of protein exchange is always accompanied by damage to other kinds of exchange of substances.

Thank you for your attention !!!Thank you for your attention !!!