M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Anomalies of constitution icon

M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Anomalies of constitution




НазваM. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Anomalies of constitution
Дата19.09.2012
Розмір446 b.
ТипДокументи


M.Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A.V., Ph.D. Anomalies of constitution

  • Associate professor Masyuta D.I.




The term "diathesis" means "predisposition", that is not wide as the mean of term "constitution's anomaly". "Diathesis" means polygenically heritable susceptibility to diseases, deviations from normal phenotype.

  • The term "diathesis" means "predisposition", that is not wide as the mean of term "constitution's anomaly". "Diathesis" means polygenically heritable susceptibility to diseases, deviations from normal phenotype.

  • Since ealy the 20th century four types of diatheses are described in pediatrics. These are follows:

    • exudative-catarrhal,
    • lymphatic-hypoplastic,
    • neuroarthritic and
    • allergic.


First three of them are anomalies of constitution and we use the term "diathesis" conditionally.

  • First three of them are anomalies of constitution and we use the term "diathesis" conditionally.

  • We undestend that these diathesis are specific dysfunctions of maturation, overstrain in proper systems. Getting each of the four constitution's anomalies is reasonable. They are concrete conditions in infants, which demand special approach to organisation of diet, regimen, vaccinations, treatment of diseases appeared on the ground of diathesis.



^ EXUDATIVE - CATARRHAL DIATHESIS

  • The term "exudative diathesis" (we use letters E and D for this term) was used for the first time by A. Cherny in 1905, and means state of reactivity in infants, which is characterized with susceptibility to recurrent infiltrative, desquamate skin and mucous membranes lesions, to development of allergic reactions and prolonged course of inflammative processes, to lymphoid hyperplasia, lability of water-salt metabolism.

  • Transitory signs of ED and/or infantile eczema are marked in 40-60% of infants during first two years of life.



Etiology

  • Allergy plays the main role in etiology and pathogenesis of ED. At the same time ED may be only an episode in infant's life; allergic diseases will develop later only in 25% of children.

  • Infants have decreased barrier intestine function because of lack of enzymes' activity for complete proteolysis, hyperpermeability of intestine's wall, decreased activity of secretory IgA.



Etiology

  • The majority of children have non-immune (we also called it in other words allergoid) genesis of ED, that is pathochemical and pathophysiological phases of allergic reaction of immediate type developed without first immunologic phase. It may be caused by

    • excessive secretion and liberation of histamine from mast cells (it means liberatory type), or
    • it may be caused by its insufficient inactivation (it means hystaminaze type).


Etiology

  • Factors which provoke clinical manifestations of ED are the following:

    • proteins of cow milk (especially in overloading - more then 3 g/kg/day),
    • eggs,
    • citric plants,
    • wild and garden strawberries.
  • Eggs, wild and garden strawberries, lemons, bananas, chocolate, and fish also contain liberators of endogenic histamine (without participation of reagins).

  • In infants who use breast-feeding diathesis may appear because their mothers could eat these food products.



Pathogenesis

  • Principal difference of infants with ED from infants with atopic diathesis is food dose-dependence of ED manifestations. Only great quantity of food eaten by mother or by infant may provoke allergic reaction. In atopic diathesis even minimal quantities of allergen lead to severe generalized allergic reaction.

  • Infants with ED have hydrolability. It means susceptibility to retention of water, sodium, and, due to it, - excessive weight gain. In another cases it means quick dehydration with great weight loss in a course of intercurrent diseases.

  • Infants with ED have unstable stool because of decreased activity of some digestive glands.

  • They have increased lymph nodes, intestine dysbacteriosis due to peculiarities of immunologic reactivity.





Clinical Manifestations of ED

  • persistent intertrigo in skin folds from the first month of life

  • xerodermia and skin pallor

  • adipose seborrhea crust on the scalp ("potato chips")

  • milk crust (temperature depending cheek's reddening and peeling)

  • wrong weight gain (more often excessive weight gain)

  • skin eruptions (erythematovesicular, erythematopapular)

  • strophulus (itching nodes with serous contents),

  • "geographical tongue“

  • prolonged conjunctivitis

  • rhinites

  • catarrh of respiratory tract with obstructive syndrome

  • anemia

  • unstable stool

















Infantile eczema



























Neurodermatitis



Neurodermatitis









  • Eczema

















Urticaria















Laboratory findings of ED

  • blood test: eosinophilia, hypocholesterinemia, hypo- and dysproteinemia

  • urine test: elevation of content of epithelium cells, eosinophiles, and mucus

  • stool: elevated content of epithelium cells, eosinophiles, and mucus



The course of ED

  • The course of ED is undulated; at the end of the second year its manifestations alleviate and disappear, but in 25-30% of children eczema, neurodermitis, bronchial asthma and other allergic diseases may develop.



Diagnosis of ED

  • Diagnosis usually is not difficult; it's necessary to differentiate it from infantile eczema and other skin diseases.

  • We can speak about true eczema when allergic essence of disease is clear. Hereditary aggravation as for allergy exists, Not only overloading with cow milk leads to appearance of skin lesion but even its minimal quantity has the same result.



Rational feeding

  • Infant with ED has to receive proteins, fats and carbohydrates with accordance to age norm and type of feeding.

  • It is useful to restrict caloric value owing to easily assimilated carbohydrates (sugar, porridge).

  • It is expedient to introduce approximately 30 % of fat owing to vegetable fats, rich with polyunsaturated fat acid.

  • For all of them restriction of salt, additive prescription of potassium salts is recommended.

  • Mother has to exclude obligatory allergens from her diet.



Rational feeding

  • For infants received formula feeding and mixed feeding it is necessary to reduse quantity of cow milk. It's better prepare porridge not with milk but with vegetable water, to give yoghurt instead of milk because during souring lactoalbumen (the main allergic protein of cow milk) is destroyed.

  • In some cases of persistent diathesis, especially in demonstrated allergy to cow milk, it's necessary to feed an infant with milk of soybean.

  • It is expedient to give additional food for infants with formula feeding at 4-4,5 months, mashed vegetable is preferable.

  • For infants with breast-feeding - vice versa - it is recommended to give additional food later than for healthy infants. It's possible to give them porridge from 6-6,5 months.



Rational vitaminisaton of food

  • It is proposed to give vitamin B6 in a dose of 50-75 mg/day.

  • In dry eczema it is useful to prescribe vitamin A 1000 ME/kg during 3 weeks, but not more than 10000 ME/day.

  • In exacerbation of process co-enzymes are evident: cocarboxylase, riboflavine mononucleotide, calcium pantothenate (vitamin B5) 100-150 mg/day, calcium pangamate (vitamin B15) 50-100 mg/day, tocoferol (vitamin E) 25-30 mg/day.



Treatment

  • Since in majority of cases allergens enter with food, courses of lactobacterin, bifidumbacterin, magnesium sulphate have positive influence.

  • We use 7-10 days courses of antihistaminic drugs. Local therapy of skin lesions is necessary.







^ LYMPHOHYPOPLASTIC DIATHESIS (LHD)

  • Lymphatic diathesis is a constitutional anomaly which is characterized by the generalised constant increase of lymph nodes even in the absence of infections, dysfunction of endocrine system (dysfunction of adrenal gland and simpatico-adrenal system, dysplasia of thymus) with decreased adaptation to influences of surroundings, susceptibility to allergic reactions.



^ LYMPHOHYPOPLASTIC DIATHESIS

  • Such children have hypoplasia of

    • chromaffine tissue,
    • reticuloepithelial apparatus of thymus - Hassal's corpuscles (with simultaneous hyperplasia of reticular stroma of lymplh nodes),
    • sexual glands,
    • cardio-vascular apparatus,
    • non-striated muscles.
  • Therefore it is lymphohypoplastic diathesis.

  • It occurs in 3.2-6.8% of children under school age.



Etiology of LHD

  • Factors of surroundings, acting both in intrauterine

    • (such as gestoses of pregnant women,
    • mother's diseases leading to increased placental permeability and passive fetal sensibilisation,
    • mother's infectious diseases of the second half of pregnancy)
  • and in extrauterine period

    • (such as prolonged infectious-toxic diseases,
    • unrational feeding with excess of proteins or carbohydrates etc.)
  • play the main role in forming of LHD.



Etiology of LHD

  • LHD occurs more often in children from families with allergic predisposition.

  • Probably, prolonged toxico-infectious diseases play the main role in formation of LHD while allergic factors of surroundings - in formation of ED.

  • Under influence of prolonged infectious-toxic factors on the ground of allergic predisposition function both of peripheral and central (thymus) lymph apparatus gets broken that leads to renal gland and chromaffine system dysfunction.



Pathogenesis of LHD

  • There is interaction between thymus and renal glands like inverse link. Probably, substances which are synthesised by reticuloepithelial apparatus of thymus, suppress glucocorticoid secretion, while hormones which are synthesised by lymphoid apparatus, stimulate it.

  • It has been proved that intensity of synthesis of catecholamines and glucocorticoids in children with LHD is decreased, and on this ground ratio of synthesis of glucocorticoids-mineralocorticoids is moved to the side of mineralocorticoids. It is conducive to the secondary hyperplasia of lymphoid tissue, retention of sodium, water and chlorides in organism.

  • Water metabolism in such children is unstable.



Clinical Manifestations of LHD

  • Children with LHD are pale, flabby, apathetic.

  • They have excessive body weight.

  • Tissue's turgor is decreased.

  • Muscles are badly developed, their tonus is decreased.

  • Height corresponds to the age norm or exceeds it owing to more long extremities.

  • Short neck, wide bones but at the same time not long narrow scapulas, narrowing of upper thorax aperture, horizontal position of ribs, genu valga, pedes plani (it means flat, splay foot) are typical.

  • The children quickly get tired, badly tolerate long and strong irritations.



Clinical Manifestations of LHD

  • Susceptibility to constant increase of peripheral lymph nodes and thymus is the most typical feature as well as increase of mesenteric, mediastinal lymph nodes, lymphatic folliculi of posterior wall of pharynx, tongue, palatine and pharyngeal tonsils.

  • Adenoid vegetations lead to disorders of nasal respiration, rhinitis, peculiar adenoidal type of face, deteriorate brain blood flow.

  • Skin lesions revealed in ED not rare occur in infants with LHD, but they are not considerable, though respiratory allergoses are typical for them.















Clinical Manifestations of LHD

  • These children have susceptibility to slight appearance and severe long course of inflammatory diseases of upper respiratory tract with development of neurotoxicosis, disorders of microcirculation. These patients form the group of so-called "children being frequently ill".

  • Sometimes there is a small, so called "drop heart", sometimes - heart's enlargement, functional systolic murmur on the apex, more rarely - hypoplasia of aortic arch, congenital heart disease.

  • Maximal manifestations of LHD develop usually at 3-6 years, later they disappear, though retardation of sexual maturation may be seen.



Diagnosis of LHD

  • It is possible to diagnose LHD when there is typical outward appearance in combination with constant significant increase of thymus and lymph nodes, preserved even in period free from infections.

  • It's expedient to prove decrease of renal gland function by laboratory methods.

  • Icrease of cardio-thymico-thoracic index (ratio of cardiothymic width in a place of tracheal bifurcation to transversal thoracic diameter at the level of diafragmal cupola expressed in %) is a rentgenologic sign of thymus enlargement.



Thymus gland. Normal appearance.



Treatment of LHD

  • Maintenance of day regimen, planned hardiness, massage and gymnastics play main role in the treatment.

  • It is expedient to restrict cow milk and easily assimilated carbohydrates in infant's feeding. It is better to change fresh milk to sour one, to give vegetable and fruit additional food.

  • Prescription of adaptogens is useful (ginseng, dibasol, metacil, pentoxil, vitamin B5, B6, B12, A, E, potassium orotate).

  • Besides, it is necessary to diagnose and treat rickets, anemia, concomitant infections.



Treatment of LHD

  • It is necessary to resect adenoid vegetation only in complete absence of nasal respiration or in often recurrent inflammations of respiratory tract organs. Not rarely they may appear again after adenoidectomy.

  • In case of thymomegaly the endocrinologist's supervision is desirable and vaccination is provided only after the consultation of an endocrinologist.

  • Thymico-lymphaticus status needs a special caution in all the situations accompanied by stress (surgical operation, vaccination etc).



Prevention of LHD

  • Treatment of infections transmitted by sexual way (>20),

  • rational diet of pregnant women and infant's feeding in accordance to age,

  • maintenance of day regimen,

  • massage and gymnastics

  • have an important value.



^ NEUROARTHRITIC DIATHESIS (NAD)

  • NAD is characterized by nerve hyperexcitability, nutritional disorders, susceptibility to ketoacidosis, and more later - to obesity, interstitial nephritis, nephrocalcinosis, gout and metabolic arthritis, that caused purine metabolism's disorders.



Etiology of NAD

  • On the one hand, inheritance of some pathologic characteristics of metabolism, and on the other hand, diet, regimen, surroundings play a role in formation of NAD.

  • In a family of infant with NAD, such diseases as gout, obesity, hemicrania, neuralgia, nephrocalcinosis, are revealed, as a rule on father's branch.



Pathogenesis of NAD

  • The following disturbances have the main value in NAD pathogenesis:

    • high level of excitability at any level of reception;
    • purine metabolism's disorders with their elevated concentration in blood and urine;
    • low acetylizing liver's ability.


Pathogenesis of NAD

  • Susceptibility to allergic diseases in NAD may be explained with the fact that uric acid inhibits synthesis of cyclic nucleotides, adenilatcyclase.

  • Decrease of glucose blood level, lypolysis in starvation (vomit, long intervals between food intake), acute diseases, stresses, and excess quantities of fat in food caused ketogenesis and development of ketoacidosis up to coma. But to 9-11 years acetonemic attacks cease.



Clinical Manifestations of NAD

  • Nerve hyperexcitability may be seen already in infants. Elder children become even more excitable, for example, may cry because of mosquito bites.

  • Psychical development of these children outstrips the age norm. They are inquisitive, animated. They have excellent memory. They are often said to be child prodigy.

  • They have strong unbalanced hyperexcitable type of nerve system. Not rarely they have night fears, tics, and chorea like attacks, steady resistant anorexia.

  • The great majority of children with NAD are thin, but some of them are stout.

  • The smell of acetone ho mouth may be in the morning.

  • Stool, as a rule, is abudant nevertheless decreased appetite.



Clinical Manifestations of NAD

  • Children with NAD may have causeless rise of temperature, hemicrania attacks, urtic and papulous eruptions after mosquito bites, edema of Quincke, nettle rash, asthmatic bronchitis, and bronchial asthma.

  • Uricosuric nephropathy is characterised with proteinuria, microhematuria, leukocyturia, cilindruria, intestinal nephritis with decrease of concentrative renal ability, susceptibility to hypertension, pyelonephritis, urolithiasis.

  • These children have infectious processes with the same frequency as other children.

  • Typical gout attacks at the infantile age are absent, though many children complain of periodical transitory joint pains.



Acetonemic vomit (crisis)

  • Periodically repeated indomitable vomit lasting 1-2 days, fit like abdomen pains, acetone smell from mouth may appear suddenly or after short indisposition (excitement, headache, anorexia, nausea, constipation, acholic stool).

  • Later exicosis, toxic breathing, hemodynamic disorders, hyperthermia and coma may develop. This condition is named acetonemic vomit (crisis).

  • Acute diseases, stresses, abuse of meat and fatty food with insufficient quantity of carbohydrates, feeding under compulsion may provoke this state.



Treatment of NAD

  • The main method of treatment is rational regimen and diet.

  • It is necessary to protect the children from intensive physical exertion, limit reviews of TV-programs.

  • Systemic hardiness, physical exercises in the morning, walks are of great use.



Diet

  • Milk products, vegetables, fruits must prevail in diet.

  • Meat, fish (especially fried, smoked), broth, fats (except vegetable), are limited.

  • Products rich in purine base and coffein (liver, brain, kidneys, herring, pate, sardine, chocolate, cacao, coffee) are excluded from diet.

  • It is of no use to feed children under compulsion, but it's necessary to avoid long intervals between food intakes. At night it is better to give hardly assimilated carbohydrates (porridge, buckwheat porridge, potato, vegetables, and rye-bread).

  • If bad appetite it is useful to prescribe abomine, gastric juice, and vitamin B,, B6, potassium orotate.



Treatment of NAD

  • Pathogenetically it is necessary to prescribe repeated courses of

    • vitamin B5 (100-150 mg/day),
    • potassium orotate (50-100 mg/day),
    • allopurinol (10 mg/ kg/day) with uricosuric remedies (ethamid, atophan, citric mixture),
    • hepatoprotectors (LIV-52, essentiale, etc.) and
    • sedatives.


Management of acetonemic crisis

  • In initial symptoms of acetonemic attack it is expedient to give glucose solution, sweet tea, fresh fruit juices, melon, watermelon, alkaline mineral water or 0,5-1% solution of sodium bicarbonate every 10-15 minutes.

  • Foods with easily assimilated carbohydrates and minimal quantities of fat (milk, sour milk, potato or vegetable puree) are indicated.

  • Cleansing enema is necessary.



Management of acetonemic crisis

  • Essentiale forte (1-2 capsules/day during 1-2 weeks) or vitamin B12 (100-300 micrograms every second day i.m.) is prescribed.

  • In severe attacks of acetonemic vomit it is necessary to inject i.v. by drops 5-10% solution of glucose in two with 0,9% solution of sodium chloride, cocarboxylase, ascorbinic acid, if pH < 7.2 - 4% solution of sodium hydrocarbonate.

  • Cleansing enema and gastric lavage are necessary.



Prevention of NAD

  • Maintenance of day regimen

  • Rational diet

  • Protection against excessive physical and psychical exertions



Allergic rhinitis







Allergic rhinitis



Allergic rhinitis







Allergic rhinitis



Range of treatment for allergic rhinitis

  • Antihistamines (used singly or in combination)

  • Steroid nasal sprays

  • Cromoglicate eye drops

  • Leukotriene inhibitors

  • Oral steroids

  • Specific immunotherapy







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