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ENDOCRINE SYSTEM

Endocrine system combines the endocrine glands (endocrine) cells that produce and
excrete into the internal environment of the body specific biologically active substances -
hormones that bind to receptors on target cells and regulate their functional activity.

Organs of the endocrine system is divided into the following groups.

Hypothalamic-pituitary system (neurosecretory neurons of the hypothalamus and


adenohypophysis).

Appendages of the brain (neurohypophysis and the pineal gland).

Branchial group originating from the epithelium of the pharyngeal pocket (thyroid,
parathyroid and thymus).

Adrenal-adrenal system (cortex and medulla of adrenal glands, paraganglia).

Islands Langerhansa pancreas.

Endocrine cells in the gonads (testes and ovaries).

Functions of the endocrine system are in the regulation of various body systems, metabolic
processes, growth, development, reproduction, adaptation, behavior. The activity of the
endocrine system is based on principles of hierarchy (subordination of peripheral to the
central level), the vertical line of feedback (enhanced formulation stimulating hormone
deficiency in the synthesis of the hormone in the periphery), horizontal networks of
interaction between a peripheral glands, synergy and antagonism of some hormones,
reciprocal autoregulation.

Hypothalamic-pituitary system
Hypothalamic-pituitary system includes:

front portion of pituitary - adenohypophysis (synthesis of tropic hormones, ex-! Press


Proopiomelanocortin gene); *

Table 2 -16. Hypothalamic hormones

Name hormone Action

Thyrotropin - Increases the secretion of thyroid stimulating hormone (TSH) and prolactin

releasing-hormone
Gonadotropin Increases the secretion of gonadotropins
Releasing Hormone
Kortikoliberin
Increases the secretion of ACTH, and P-melanotropinov lipotropina
Growth hormone Increases the secretion of growth hormone
releasing hormone
Somatostatin Reduces the secretion of growth hormone, TSH, ACTH
ADH Enhances the reabsorption of water in the distal renal tubules, the regulator of water
balance of the organism, has a vasoconstrictor effect, stimulates glycogenolysis and
increases platelet aggregation

Oxytocin It is the reduction of smooth muscle cells of the uterus, especially during childbirth,
and myoepithelial cells of mammary glands, contributing to the separation of milk

Dopamine Physiological inhibition of production of prolactin

The activity of the hypothalamus is under the control of the overlying parts of the brain, as
well as a number of hormones.

HYPOPHYSIS

The pituitary is located in the Turkish saddle - deepening the base of the skull. From the
cranial cavity pituitary delimited fold of dura mater (diaphragm sellae). Thin leg,
penetrating through the diaphragm, the pituitary gland is connected with the
hypothalamus.

neurosecretory nuclei of the hypothalamus (synthesis-releasing hormone, ADH, oxytocin,


neyrofizinov);

hypothalamic-pituitary tract (transport of hormones in the axons of neuro-secretory


neurons);

aksovazalnye synapses (secretion of ADH and oxytocin in the capillaries of the posterior
lobe of the pituitary, releasing-hormone secretion in the capillaries of the median
eminence);

portal system of blood flow between the median elevation and front lobe of the pituitary.

Hypothalamus

The hypothalamus forms the lower parts of the diencephalon and participates in the
formation of the bottom of the III ventricle. Clusters of nerve cells make up 32 pairs of
nuclei of the hypothalamus. The hormone-producing nerve cells are part of many nuclei of
the hypothalamus. Perikarionah In these neurons synthesized-releasing hormone
[stimulating factors (liberiny) and inhibiting (statins)] coming into the capillaries of the
anterior pituitary, and also ADH, oxytocin and their neyrofiziny (Table 2-16).
Histologically the pituitary gland is divided into the adeno-and neurohypophysis.
Adenohypophysis consists of anterior and intermediate lobes, and posterior superior
alveolar nerve of the pituitary stalk. Six tropic adenohypophyseal peptide hormone
secreted nature of 5 different types of cells (Table 2-17).

Table 2-17. Adenohypophyseal hormones

Name hormone Action

Somatotropin In children and adolescents stimulates bone growth (mainly tubular, less porous),
(somatotropin, growth activating the chondrite-and osteogenesis, activates the exchange of bone tissue,
hormone, growth causing increased osteoobrazovaniya and to a lesser extent - osteo-resorption;
hormone) initiates the differentiation of myoblasts, has anabolic effects, stimulating protein
synthesis, has a lipolytic effect, leads to a decrease in total adipose tissue, has a
biphasic effect on carbohydrate metabolism (insulin-like effect at first, then
kontrinsulyarny, with prolonged exposure to high doses of growth hormone
develop insulin resistant)

Corticotropin (ACTH) Promotes proliferation of cells of the adrenal cortex, stimulates the synthesis and
secretion of hormones of the adrenal cortex (mainly corticosteroids).

It stimulates the synthesis of melanin in melanocytes, causing increased skin


pigmentation
Thyrotropin (thyroid- It stimulates the differentiation of cells of the thyroid gland and synthesis of
stimulating hormone, iodine-containing hormones, their connection with the release of thyroglobulin
TSH) and secretion

Lyutropin (Officer- Facilitates the completion of egg maturation, ovulation and corpus luteum
luteinizing hormone, formation, stimulates the secretion of progesterone, stimulates the synthesis of
LH) androgens in the cells oftheca, in males stimulates androgen production in
testicular cells Lyaydiga
Follitropin In the women's body stimulates the growth and maturation ovarial-tion of the
(follikulostimu follicles, increases the secretion of estrogen, in men - the growth and proliferation
liruyuschy-stimulating semyaobrazuyuschih testicular tubules and spermatogenesis, activates the
hormone, FSH) synthesis and secretion in Sertoli cells of androgen-binding protein, ingibina,
estrogen, etc.
Prolactin Stimulates the growth of mammary glands during pregnancy and lactation after
delivery, the male is a factor in the growth of prostate

The effects of growth hormone sold by insulin growth factor (IRF). In kortikotropah
anterior pituitary of the molecule proopio-melanokortina posletranskriptsionnyh changes
in the form of ACTH, melanotropiny (A, B and y) and B-endorphin. Melanotropiny control
pigmentation of the skin and mucous membranes, such as OS-melanokortin stimulates the
synthesis of eumelanin in the skin. Established that the y-melanokortin stimulates the
synthesis of aldosterone. Secretory activity of the adenohypophysis is under the control of
the hypothalamus, a number of hormones and other factors.
Rear share - neurohypophysis - a protuberance of the brain and is composed of cells of the
neuroglia (pituitsitov). In the neurohypophysis hormones are not synthesized. The axons
of the hypothalamic-pituitary tract it from the hypothalamus receives ADH, oxytocin and
neyrofiziny.

Anatomic and physiologic features

Average weight of pituitary gland in neonates is 0,1-0,15 g. By 10 years of its mass is


doubled, and by 15 years - tripling. In the adult human pituitary gland weighs 0,53-0,56 g.
In functional terms, the hypothalamic-pituitary region at the time of birth immature and
develops as they grow.

Growth hormone in the highest concentration detected in newborns with which


associated increased lipolysis and decrease blood glucose levels in the postnatal
period. Then its level is somewhat reduced. There is a clear link to the stage of growth
hormone concentration of puberty. The concentration of IRF-1 lowest at birth, then it
gradually increases in teacher-bertatnom period and significantly increased during
puberty. The level of IRF-N changes little with age and does not depend on the stage of
sexual development.

The highest concentration of ACTH also noted in the newborn, which provides the
processes of adaptation, then its concentration decreases.

The concentration of TSH in newborns are several times higher than in subsequent age
periods.

Concentration of gonadotropic hormones (LH and FSH) increases the period of puberty
(boys and girls).

METHODS AND SEMIOTICS SHOCK

In the clinical examination can identify these or other signs of pituitary function, which
evaluate the length and weight, the dynamics of their increase, the state trophics tissues,
development and distribution of subcutaneous adipose tissue, the timeliness of the
appearance of secondary sexual characteristics, and neurological status. Furthermore, it
should measure urine output, to assess the proportion of urine, to determine the
concentration of potassium ions, sodium and osmolarity of blood and urine.

Indirectly, the status of the pituitary can be judged by the size, shape and structure of the
Turkish saddle on radiographs. More accurate data are obtained by CT and MRI. To
determine the functional state of the pituitary and hypothalamus used immunological
methods of investigation of hormone levels in the blood of the child. The maximum
allocation of growth hormone occurs during sleep at night. To assess the concentration of
growth hormone secretion determine its primary, and then repeat the study after
stimulation tests (with insulin, klofelinom, etc.).

Hypopituitarism

Hypopituitarism - deficiency of one or more pituitary hormones, may occur in diseases of


the pituitary or hypothalamus. The reasons for hypopituitarism in children may be
anomalies of the CNS, the hypothalamus or pituitary tumors, brain trauma syndrome
"empty" sella turcica, hydrocephalus, infections, autoimmune lymphocytic gipofi-sum,
thrombosis, hemorrhage, granuloma, iatrogenic factors (a complication of radiation
therapy or overdose of glucocorticoids).

The cause of family forms of multiple hormones adenohypophyseal insufficiency may be a


mutation of the gene PIT1 (pituitary-specific transcription factor-th 1). This raises a
combined deficit of growth hormone, TSH, and prolactin. In children with a defect in the
gene PROP1 (predecessor PIT1) developed growth hormone deficiency, TSH, prolactin,
gonadotropins, and in some cases of ACTH.

Lack of hormones acting on peripheral endocrine glands, causing their dysfunction: TSH
deficiency - a cause of secondary hypothyroidism, deficiency of gonadotropic hormones -
secondary hypogonadism, ACTH deficiency - secondary adrenal insufficiency and
hypopigmentation. Growth hormone deficiency in infants can cause hypoglycemia in pre-
pubertal age of Mr. - growth retardation, and adults emerge with wrinkles around the eyes
and mouth, excessive deposition of fat on the abdomen and lower muscle mass. Lack of
prolactin appears violations of lactation.

Panhypopituitarism may occur panhypopituitarism (Simmonds' disease), followed by


general exhaustion, degenerative changes in the skin and its appendages, the destruction
and loss of teeth and hair, atrophy of muscles and internal organs, hypothermia,
hypotension, hypoglycemia.

Partial hypopituitarism

Partial hypopituitarism is the result of failure of any one of the tropic hormones.

The most characteristic disease associated with partial hypopituitarism th - ateleiosis


(pituitary dwarfism), due to lack of growth hormone and gonadotropin. In addition to
these causes of growth hormone deficiency, distinguish some hereditary forms | isolated its
deficiency: gene defect Gffl, defective gene receptor \ torus-releasing hormone growth
hormone, etc.

Isolated gonadotropic hormone deficiency leads to delayed sexual development j (it does
not begin or end) and manifests \ symptoms of secondary hypogonadism. In most cases,
the state] is due to a congenital defect of GnRH, occasionally - innate-\ Noah or acquired
deficiency of gonadotropic cells gipofi-\ for . The most common congenital isolated
gonadotropic deficiency [hormone occurs Kallmana syndrome (anosmia, the absence of CEE-
f tovogo of view, the facial deformities of the median line) and Laurence Moon Bar: de Bill
(mental retardation, Polydactyly, obesity, pig - \ mentny retinitis, deafness, low-growing
beard, diabetes insipidus).

Isolated ACTH deficiency rarely develops and leads to gipokor-titsizmu, most often are
relatively non-severe form of sign-chechnikovoy deficiency, symptoms which usually occur
only during stress. Hyperpigmentation and increased demand for salt is not typical.

TSH deficiency clinically evident hypothyroidism: hypothyroidism with pituitary TSH


concentration after the introduction of Thyrotropin-releasing hormone is low, and at the
hypothalamic - increases.

Giperpituitariam

Giperpituitarizm often has a partial nature. The reason for increasing production of
pituitary hormones may be the process of tumor (adenoma, a tumor of the diencephalon,
suprasellyarnaya tumor craniopharyngioms-ma), encephalitis, or degenerative process in
the brain.

The excess production of growth hormone in children (before the closure of epiphyseal
cartilage growth zones) - a cause of pituitary gigantism or you-sokoroslosti, in adults (after
epiphyseal closure growth zones of cartilage) - acromegaly, characterized by thickening
and deformation of bones and increase the mass of soft tissue and internal bodies,
metabolic disorders.

Increased gonadotropin secretion may lead to early puberty. However, the gonadotropic
pituitary adenomas, despite the increased formation of LH and FSH, is developing
hypogonadism, which is related to an inadequate secretion of LH biological activity or
violations of the nature of the secretion of LH (normal pulse).

With an excess of TSH increased the concentration of thyroid hormones, there are clinical
signs of hyperthyroidism and goiter.

Excessive secretion of ACTH leads to the development of pituitary Cushing's.

When the excess production of prolactin in girls is delayed or disrupted sexual


development, the women developed galactorrhoea, oligo-or amenorrhea, and
gynecomastia in men occurs.

Diabetes insipidus
Diabetes insipidus is manifested violation of concentrating urine in diuriey, polydipsia,
frequent urination. Central diabetes insipidus due to ADH deficiency, primary (as a result
of gene mutations of enzymes ADH and its synthesis) or secondary (in tumors, histiocytosis
X, granulomatosis, infections, abnormalities of blood vessels, brain injury). In addition,
diabetes insipidus may be due to congenital anomalies of the brain and the autoimmune
destruction supraopti-profit and paraventricular nuclei of the hypothalamus. Nephrogenic
diabetes insipidus develops in renal insensitivity to ADH.

ADH hypersecretion syndrome

For ADH hypersecretion syndrome characterized by hyponatremia, gipoosmo-polarity


plasma and excretion of concentrated urine. Children hypersecretion

ADH occasionally occurs in diseases of the lungs (pneumonia, tuberculosis) and brain
(infection, hemorrhage, traumatic brain injury, intracranial hypertension, tumors). In
addition, neonatal causes hypersecretion ADH may be ventilated with positive pressure,
the older children -- use of vincristine, cyclophosphamide, chlor-propamida, some
analgesics and barbiturates, as well as heart disease, severe hypothyroidism, primary
adrenal insufficiency.

Pineal gland
Pineal gland (pineal gland) refers to epithalamus diencephalon and connected "leashes" on
the thalamus. Pineal gland function poorly studied. It is believed that he was involved in the
implementation of biological rhythms in humans, affects sexual maturation and function of
sex glands, inhibits activity of the thyroid gland.

Epiphysis in the birth weight is about 7 mg and during the first year of life increased to 100
mg. By 10 years nearly doubled the mass of epiphysis and subsequently does not
change. Dimensions epiphysis in neonates are 2,6 x2, 3x1, 7 mm, at the age of 10 years - 6,0
x5, 5x4, 0 mm. Differentiation epiphysis ends to 7 years. Pineal gland dysfunction observed
in his aplasia, the expansion of the neuroglia with the formation of cysts, calcification in the
outcome of destructive changes after the inflammatory process (epiphyte-Zita),
hemorrhage, necrosis, etc. Perhaps the development of tumors, and pineal-pineotsitom
pineoblastom.

Thyroid
The thyroid gland - an unpaired organ, consisting of two parts (left and right) connected by
an isthmus. Often there is extra (pyramidal), the proportion originating from the isthmus
or the left lobe and directed upward. The thyroid gland is located in front of the neck
between the thyroid cartilage and V-VI annular cartilage of the trachea.
The thyroid gland is covered by a fibrous capsule, inside of which depart from the
connective tissue partitions - trabeculae separating its tissue into slices, consisting of
follicles, filled with a homogeneous mass (colloid). The walls of the follicles (rounded
closed entities) are composed of epithelial cells (thyrocyte) that produce iodine-containing
hormones (thyroxine - T 4 and triiodothyronine - T 3). The function of TSH stimulates the
follicular cells under the control of hypothalamic-Tyro liberina.

In addition, between the follicles are rare clusters of larger light cells (P-cells,
parafollikulyarnyh cells), in which there is a synthesis of the hormone calcitonin, does not
contain iodine.

Iodinated thyroid hormones have a broad spectrum of action, includes metabolic,


physiological and morphogenetic effects (Table 2-18). From the thyroid gland depends on
the basic vital functions of man, as her hormones affect the activity of all its organs and
systems.

Table 2-18. Effects of iodine in thyroid hormone

Type effect Support provided to effect

On metabolism Contribute to oxidative phosphorylation Raise heat production

Control the synthesis of proteins: a physiological quantities have anabolic


effects, but in high concentrations - catabolic

Increase the mobilization of fat from the depot, activate lipolysis

and the oxidation of fats, suppress lipogenesis from carbohydrates,

help reduce blood cholesterol

Increase the breakdown of glycogen, inhibit its synthesis from glucose;

promote gluconeogenesis from proteins, stimulate

absorption of carbohydrates in the intestine, providing a general hyper -

glycemic effect

Influence water-electrolyte balance

Affect the exchange of vitamins, enzymes, neurotransmitters


On the function of organs Activate sympathoadrenal and cardiovascular systems, causing hyperdynamic
and systems state of the last

Influence the function of the higher divisions of the CNS, in particular the
mental processes

They stimulate hematopoiesis

Increase the appetite and increase the secretion of gastric juice in the digestive
tract

Affect skeletal muscles improves metabolic processes in the liver

Influence on other endocrine glands (sex, adrenals, etc.)

Are potent immunomodulators


At the level of tissues Regulate the process of differentiation of tissues

Thyroid hormones (T 3 and T 4) are necessary for the organism's development, especially in
the prenatal and early postnatal periods, when there is a formation of organs and systems,
thyroid hormones stimulate the proliferation and migration of neuroblasts, the growth of
axons and dendrites, the differentiation of oligodendrocytes, they determine the normal
differentiation of the brain brain and cognitive development. T 4 and T 3 regulate the
processes of human growth and maturation of its skeleton (bone age), the development of
skin and its appendages.

Calcitonin regulates phospho-calcium metabolism, as an antagonist of parathyroid


hormone (PTH). It protects the body from excess revenues of calcium ions, reducing its
reabsorption in the tubules of the kidneys and absorption from the intestine, while
increasing fixation of calcium in bone tissue. Production of calcitonin is dependent on the
content of calcium ions in the blood.

Anatomic and physiologic features

Dimensions of the thyroid gland in the newborn is much higher than that of the fetus, but
the follicles are smaller in children than in adults, and contain small amounts of colloid.
During the first six months of life weight of prostate decreased slightly, decreasing the
concentration of thyroid hormones in the follow-up to 5-6 age is the rapid growth of the
thyroid gland, then it slows down to a few prepubertal period, when its size is once again
growing rapidly, and reaches a weight of 10-15 g. As we age, the thyroid gland increases
the size and number of follicles, as well as the content of the colloid. The final histological
structure of the iron gets after 15 years.

Maximum concentrations of TSH, T 3 and T 4 to reveal the baby's blood during the first
hours and days of life, which indicates the important role of these hormones in the period
of postnatal adaptation. During the first week of the concentration of TSH gradually
decreases somewhat more slowly declining levels of T 3 and T 4.

Development of calcitonin in the newborn compared with the result of lower and remained
stable throughout the period of childhood.

Survey methodology and semiotics SHOCK

Looking around the front surface of the neck, you can make an approximate idea of the size
of the thyroid gland, which normally is not visualized. On palpation of the thyroid gland is
necessary to pay attention to the following parameters.

Dimensions (in normal thyroid gland can be palpated, and the size of its share should not
exceed the size of the nail phalanx of thumb patient).

The nature of the increase (diffuse or nodular).

Consistency (normal myagkoelasticheskaya).

Surface (normally smooth).

The degree of mobility in swallowing (normal mobile).

The presence of pain (normal pain-free).

To better define the shape, size, location and density of the thyroid gland conduct
ultrasound, scintigraphy, if necessary, and morphological study punctate. To evaluate the
thyroid determine the concentration of thyroid hormones (T 3, T 4) and TSH in serum of the
child, with Thyrotropin-releasing hormone test to estimate the iron absorption of
radioactive iodine, the degree of iodine deficiency on the level ioduria reveal a Ag AT gland.

Goiter

Goiter (enlarged thyroid gland) occurs in its various diseases and may involve a breach of
duties.

Diffuse non-toxic goiter - is the most common thyroid cancer in children and teenagers -
accompanied by a diffuse increase in its size and clinically euthyroid state, typical of the
endemic (iodine deficiency), goiter, autoimmune thyroiditis and goitre dishormonal. The
latter is formed by genetic defects of synthesis, secretion or metabolism of thyroid
hormones.

Graves' Disease is characterized by a diffuse increase in thyroid cancer and


hyperthyroidism phenomena.
Nodes in the thyroid gland may have a nontumorous nature and represent a colloid cysts,
abscesses. Benign tumors of the thyroid gland in children include adenoma and teratoma,
malignant - various forms of cancer, lymphosarcoma, metastases of other tumors.

Hypothyroidism

Hypothyroidism - a syndrome caused by thyroid hormone deficiency or insensitivity to his


target-tissues. Hypothyroid state is divided into peripheral (primary) and central
[secondary (hyper-pofizarnye), a lack of TSH, and tertiary (hypothalamic) associated with a
deficit of Thyrotropin-releasing hormone]. Clinical manifestations of hypothyroidism are
varied and depend on time of onset.

Congenital hypothyroidism

Congenital hypothyroidism can be caused by thyroid dysgenesis (more distopicheskim


location of cancer, at least - hypo-and aplasia), genetic defects in the synthesis, secretion or
peripheral metabolism of thyroid hormones, iodine deficiency in pregnant women,
treatment of pregnant with radioactive iodine, etc. Transient primary hypothyroidism in
the newborn may be due to treatment of pregnant antithyroid drugs or transplacental
transfer of maternal tireoblokiruyuschih AT. Secondary congenital hypothyroidism in
children with malformations of the brain and skull, pituitary aplasia, rupture or separation
of the pituitary stalk during delivery. Transient its form is in preterm and in term infants
born with low birth weight, intrauterine growth retardation.

Children with congenital hypothyroidism are often born postmaturity with a mass of
more than 4000 g. They may later discharge of meconium, constipation, sluggish sucking, a
rare weeping and low tone of voice in a scream. Characteristic somnolence, lethargy,
hypothermia, the latest defection from umbilical cord remnant, prolonged jaundice. For
severe congenital hypothyroidism is characterized by the appearance of newborns:
puffiness face, flat or sunken nasal bridge, hypertelorism, gapping of the skull and an
increase in the size of fontanelles, macroglossia, an increase in the abdomen, umbilical
hernia, dry and marbling of the skin, muscle hypotonia, a weakening of tendon reflexes,
bradycardia.Frequent difficulty in nasal breathing, seizures, cyanosis, susceptibility to
respiratory diseases. In this note the lag in psychomotor and physical development and
bone age of the passport. Goiter palpable rare.

Acquired hypothyroidism

Hypothyroidism in older children and adolescents is most often the result of Hashimoto's
thyroiditis. Furthermore, it may be due to iodine deficiency, iodine intake of drugs,
radioactive iodine treatment, removal of the thyroid gland. Hypothyroidism can occur
when infiltrative lesions of the thyroid gland (histiocytosis X), cystic fibrosis. Inborn errors
of thyroid structure and defects in the enzymes of synthesis and metabolism of thyroid
hormones may be asymptomatic at an early age and clinically manifested later as child
growth and development, sometimes at the beginning of puberty. Causes of secondary
hypothyroidism can be hypopituitarism, pituitary adenomas, tumors, head trauma,
infection, radiation and chemotherapy, etc.

For children with hypothyroidism is characterized by general lethargy, sluggishness,


drowsiness, weakness, fatigue, slow speech, low hoarse voice, poor tolerance of cold,
decreased appetite, constipation. The examination reveal bradycardia, often transudate in
the pericardial cavity, reduced tendon reflexes, low growth, overweight, delayed eruption
and replacement of teeth, bone age delay of the passport, dysgenesis epiphyses, osteohon-
dropatiyu the femoral head. In addition, patients are mental retardation, memory loss,
poor school performance, may be sensorineural hearing loss, ataxia, tunnel
neuropathy. Usually there is a delay (sometimes premature) sexual development,
amenorrhea or oligomenorrhea. During lab tests reveal anemia.

Thyrotoxicosis

Thyrotoxicosis - syndrome caused by the increased formation of thyroid hormones.

Thyrotoxicosis in newborns, usually transient, are not common, due to transplacental


transfer of maternal tireostimu-liruyuschih AT and ends after 3-12 months. Children with
congenital thyrotoxicosis are usually born prematurely, and are in full-term fetal
retardation and low body weight. Usually observed tachycardia, sometimes arrhythmia,
increased excitability and motor activity, sweating, increased appetite with poor weight
gain. Often noted exophthalmos, palpable goiter, in some cases are microcephaly and
expansion of the ventricles of the brain, there may be n-patosplenomegaliya and
jaundice. Also identify accelerated skeletal maturation and premature closure of sutures of
the skull.

Thyrotoxicosis in older children, most often the case with diffuse toxic goiter (organ-
autoimmune disease), less often - with a toxic adenoma and thyroid cancer, an overdose of
thyroid hormones, excess TSH, subacute and Hashimoto's thyroiditis, excess iodine. Clinical
symptoms of hyperthyroidism include weight loss with increased appetite, thirst,
excitability, irritability, emotional lability, violation of concentration, increased tendon
reflexes, tremors, excessive sweating, heat intolerance, tachycardia, increased
peristalsis.Characteristic ocular symptoms: bright eyes, increased eye slits, a rare flash
(normally within a minute is 3.5 blinks), the lag of the upper eyelid from the iris during
movement of the eyeball downwards (symptom Graefe), weak convergence of diverting
sight while trying to commit on close objects (a symptom of Moebius), hyperpigmentation
of the skin age, exophthalmos. When thyrotoxicosis in children may speed up growth in
girls is delayed menarche, boys may develop gynecomastia.

Thyroiditis
Thyroiditis - inflammatory diseases of the thyroid gland, usually characterized by its
increase, in some cases - pain and disability.

Acute thyroiditis - a rare disease caused as a rule, gram-positive bacteria. In this disease
may form an abscess.

Subacute granulomatous thyroiditis in children occur rarely. The reason for its
development believe viral infection. In the initial period, patients may develop
thyrotoxicosis.

Autoimmune thyroiditis - organospecific disease characterized by the elaboration of


antithyroid AT (for microsomal Ar ti-reoglobulinu, etc.) and cytotoxic reactions. In children
with dominant hypertrophic form of infiltration of the thyroid gland by lymphocytes and
plasma cells, the presence of cell-Ashkenazi Hyurtlya, moderate fibrosis. Autoimmune
thyroiditis can lead to the development of hypothyroidism.

Parathyroid gland
Parathyroid glands are oval cells, located on the rear surface of the shares of the thyroid
gland. Parathyroid glands secrete into the blood parathyroid hormone (parathormone,
PTH), whose primary function is to maintain a constant concentration of calcium ions and
phosphate in the blood. PTH increases the concentration of calcium ions in serum by
activation of osteoclasts and increased bone resorption, as well as increasing reabsorption
of calcium ions in the distal tubules of the kidneys. PTH stimulates the formation of
calcitriol in the proximal tubules, which increases calcium absorption in the
intestine. Interactions of the kidney, PTH reduces the reabsorption of phosphate in the
tubules, increasing phosphaturia, and also promotes leaching of phosphorus from the
bones.

Anatomic and physiologic features

The total mass of the parathyroid glands in newborn infants is 6-9 mg. Already during the
first year of life, it increases by 3-4 times, to 5 years are doubled, and by 10 - tripled.

For newborns is characterized by physiologic hypocalcemia caused by the abrupt cessation


of receipt of calcium ions from the mother and the failure of parathyroid glands to mobilize
calcium ions from the bone tissue. Giperkaltsiemicheskomu Effects of PTH, in addition,
prevent calcitonin and cortisol release that occurs after childbirth. The system provides a
constant concentration of calcium ions in serum, finally formed by the end of neonatal
period. Maximum functional activity of the parathyroid glands occur during the first 2
years of life, especially when intense osteogenesis.

Survey methodology and semiotics SHOCK


Parathyroid glands unavailable fizikalnom survey methods. However, questions and
general examination may reveal clinical signs of dysfunction parathyroid glands. Also,
using ultrasound, radioisotope scanning, CT, thermography. Direct and objective method of
assessing parathyroid gland function - definition of blood concentrations of PTH. Moreover,
investigating the concentration of ionized and total calcium and phosphorus in the blood
serum, as well as providing them with urine. The concentration of ions of calcium and
phosphorus in the urine when changing the content of PTH is inversely proportional to
their content in the blood.

Gipoparatireoe

Gipoparatireoz - a syndrome caused by deficiency of PTH. For all forms of gipoparatireoza


characterized by hypocalcaemia and hyperphosphataemia in the background of low
concentrations of PTH.

Clinical manifestations of hypocalcemia are mainly caused by a violation of neuromuscular


conduction. In the newborn a condition manifested by increased excitability, a piercing cry,
muscle twitching, seizures, apnea, arrhythmias, and in older children may develop tetany
and paresthesias. Severe hypocalcemia may lead to a decrease in cardiac output and a drop
in blood pressure. When gipoparatire Oz having seizures tonic seizures, which capture the
predominantly symmetrical groups of muscles, flexors and develop in response to tactile
stimulation during the inspection, swaddling, overheating, after trauma, etc. Cramps often
occur in the upper extremities (hand midwife "), but can be at the bottom (the legs
straightened, pressed against one another, feet flexed), as well as the muscles of the face ("
fish mouth ").

When tetany can cause specific symptoms: chvostek (high - spasm of muscles
corresponding half of the face when tapped on the zygomatic arch at the place of exit of the
facial nerve in the external auditory canal, the bottom - a spasm of muscles corresponding
half of the face when tapped on the corner of the lower jaw), Trousseau (appearance "
hands midwife "in pulling the shoulder braid to the disappearance of the pulse for 2-3 min),
Lust (abduction of the foot when tapped below fibular head). In addition, hypocalcemia
occur paresthesias, hyperreflexia, laryngospasm (usually occurs with seizures, most often
at night), bron-hospazm, spastic bowel reduction.

The ECG reveal the elongation of the interval Q-T and the segment ST, the low triangular
teeth of T. Perhaps the development of arterial hypotension.

Transient neonatal gipoparatireoz usually appears on the 2-10-day life and is due to
suppression of secretion of PTH due giperkal-tsiemii mother, including those caused by
hyperparathyroidism. Gipoparatireoz in infants and young children is usually caused by
congenital, but at a later age - acquired lesion of parathyroid glands. Isolated idiopathic
gipoparatireoz caused by a defect of synthesis or secretion of PTH, - a rare sporadic or
family illness - can occur at any age. Gipoparatireoz may be a component of diseases such
as autoimmune poliglandulyarny syndrome type I or syndrome Di Giorgio with agenesis or
dysgenesis parathyroid and thyroid glands, heart defects. By gipoparatireozu may also
cause removal of parathyroid glands, the violation of their blood supply and innervation,
infection and radiation damage, haemochromatosis, hypomagnesaemia.

Gipvrparatireoe

Hyperparathyroidism - a syndrome that occurs when an excess of PTH. His clinical picture
is primarily associated with hypercalcemia. Characteristic weakness, malaise, polydipsia,
polyuria, izostenuriya, vomiting, constipation, abdominal pain, early childhood - delayed
physical development. In addition, patients may experience defeat muscles (myalgia, and
marked muscle weakness), ossalgii, gout, pseudogout, hondrokal-tsinoz, erosive
arthritis. Radiological findings reveal fibrocystic Osse, with densitometry - reduced bone
density, which increases the risk of fractures. Often the bones discovered cysts and
tumors. Increased excretion of calcium ions leads to urolithiasis may develop
nephrocalcinosis. Possible arrhythmia, shortening of the Q-T interval on the
electrocardiogram, arterial hypertension.

Severe neonatal hyperparathyroidism - homozygous gipokal-tsiuricheskaya


hypercalcemia - a rare inherited disorder caused by mutation in a gene receptor calcium
ions in cells of pa-raschitovidnoy gland and kidney tubules. It is secreted excessive
amounts of PTH and increased reabsorption of calcium ions. The clinical picture includes
severe symptoms of hypercalcaemia, the anomalies skeleton, delay physical development
and mental retardation. The same genetic defect, but in the heterozygous form, leads to a
family of benign hypercalcemia (heterozygous gipokaltsiuricheskoy
hypercalcaemia). Usually these children have a hyper-kaltsiemiyu detected by chance.

Primary hyperparathyroidism in children are rare. The reason for its occurrence may be
hyperplasia of all parathyroid glands, which develops in severe neonatal
hyperparathyroidism (see above), as well as the syndrome of multiple endocrine neoplasia
type I (Wermer syndrome) and Na (Sippla syndrome). In addition, hyperparathyroidism
develops in an adenoma or cancer of the parathyroid gland, the presence of cysts.

Secondary hyperparathyroidism occurs when the relative or complete resistance of


target tissues to PTH, which may develop in renal failure, rickets and osteomalacia,
deficiency of vitamin D, hypophosphatemic rickets, psevdogipoparati-reoze types la, lb and
1c (Albright's syndrome).

Adrenal
Adrenal - paired endocrine glands located in the BOAD-tire tissue over the upper pole of
kidneys at the level of T X [1-L ". Weight of one adrenal in the adult is on average 12-15 g,
sizes - 40-60x20-30x2-8 mm. Adrenal consists of the outer cortical substance (up to 80% of
the total mass), which synthesizes steroid hormones (the original product synthesis -
cholesterol), and the internal cerebral, separates catecholamines.

In the adrenal cortex distinguish three zones: the glomerular producing mineralocorticoids
(mainly aldosterone), the beam synthesizing glucocorticoids (cortisol, cortisone, etc.), and
mesh, which are produced by androgens (dehydroepiandrosterone and androstenedione),
trace amounts of estrogen. In the adrenal medulla catecholamines formed: mainly
epinephrine (80-90%), smaller quantities of norepinephrine (10-20%) and dopamine (1-
2%).

Aldosterone stimulates the tubular reabsorption of sodium ions and excretion of


potassium ions, hydrogen, ammonia and magnesium in the kidneys, playing a crucial role in
the regulation of fluid and electrolyte homeostasis of the organism and the volume of
extracellular fluid. After sodium ions aldosterone affects blood vessels, improving their
tone.Secretion of aldosterone is under the regulation of the renin-angiotensin system
(mainly otenzina Angie-I), dopamine, in a small degree of ACTH and depends on the
concentration of potassium ions in blood serum.

Glucocorticoids have an extremely broad spectrum of activity (Table 2-19). The highest
biological activity has glucocorticoid cortisol. The main regulator of the synthesis of
glucocorticoids - ACTH.

Androgens (dehydroepiandrosterone and androstenedione) are involved in the process


of puberty, have anabolic and gipoholesterinemiches-Kim effects.

Catecholamines cause vasoconstriction contribute to increased blood pressure, stimulate


the heart, increase glycogenolysis, lipolysis, glyukoneo-genesis. These and other effects of
catecholamines are sold through a-and | 5-ad-renoretseptory on the surface of target cells.

The anatomic and physiologic features of the organism 213 Table 2 -19. Functions
glucocorticoids

Places application Action

Carbohydrate Increase the concentration of glucose in the blood by increasing the rate of
metabolism gluconeogenesis in the liver, reducing glucose utilization in the periphery,
stimulation of the release of amino acids (substrates of gluconeogenesis) in the
muscles

Proteometabolism Increase protein breakdown and inhibit its synthesis

Lipid metabolism Increase lipolysis in the upper and lower extremities, lipogenesis - in other parts of
the body (torso and face)
Exchange calcium Inhibiting the activity of vitamin D, causing reduced absorption of calcium ions and
increasing its excretion
Immune system In high doses, have immunosuppressive effects
Inflammation They possess anti-inflammatory effect

Collagen synthesis With prolonged use inhibits the synthetic activity of fibroblasts, chondroblasts and
osteoblasts, contributing to thinning of the skin and osteoporosis

Skeletal muscle Cause muscle atrophy and muscle weakness after prolonged use

Secretory function of Secretagogue hydrochloric acid and pepsin


stomach

Anatomic and physiologic features

At birth, the mass of one adrenal gland in a child reaches 7 g, their value is 1 / 3 the size of
the kidneys. Newborn adrenal cortex, as in the fetus, consists of 2 zones - fetal and
definitive (permanent), with the proportion of fetal the bulk of cancer. Definitive zone is
functioning as an adult. Beam area is narrow, poorly formed, reticulated zone yet.

At birth the newborn from the mother receives a large number of glucocorticoids, which
leads to suppression of synthesis of ACTH in the pituitary gland. In this regard, in the
postnatal period the significant reduction in excretion and production of glucocorticoids,
which may lead to the child's clinical signs of adrenal insufficiency. By the 10 th day of life
the synthesis of glucocorticoids re-activated.

During the first 3 months of living adrenal mass is reduced by half, to an average of 3,4 g,
mainly due to thinning and the restructuring of the cortical substance, after a year, she
again begins to increase. By the age of one year fetal zone disappears completely, and in the
definitive cortex is discernible glomerular, beam and mesh band. By 3 years of complete
differentiation of the cortical part of the adrenal. The formation of zones of the cortical
substance continues until 11-14 years, by that time the ratio of the width of the glomerular,
beam and mesh zones is 1:1:1. By 8 years is an increased growth of brain substance. The
final formation of its ends to 10.12 years. Weight of adrenal glands markedly increases pre-
and puberty and to 20 years increased by 1,5 times in comparison with their mass in the
newborn, reaching a performance characteristic of an adult.

Cortisol secretion is subject to diurnal rhythm (the maximum in the morning), which is
installed by the end of the first year of life.

Survey methodology and semiotics INJURIES

In explaining the complaints and conducting a general inspection can identify clinical signs
of adrenal function, characterized by a number of symptoms and syndromes. To determine
the structure and size of adrenal glands using ultrasound and CT. To assess adrenal
function determine the concentration of cortisol, aldosterone in blood and urine, sex
steroid hormones in the blood, catecholamines (epinephrine, norepinephrine and vanillin-
mandelic acid) in urine, conduct diagnostic tests with ACTH or glucocorticoids.

Gipokortitsiem

Gipokortitsizm - Reduced function of the adrenal cortex (adrenal insufficiency-kovaya).

Chronic adrenal insufficiency is manifested in children overall weakness, fatigue,


developmental delay, anorexia, her weight loss, hypoglycemia, occasionally nausea,
vomiting, loose stool, abdominal pain, arterial hypertension. In primary chronic adrenal
insufficiency, in addition, note hyperpigmentation of the skin and mucous membranes
(consequence enhanced secretion of ACTH), increased demand for salt, hyponatremia, and
hyper-perkaliemiyu (due to lack of aldosterone). In secondary chronic adrenal
insufficiency, these symptoms are absent, except for hyponatremia breeding.

- Primary congenital chronic adrenal insufficiency usually develops in children with defects
in enzymes of steroidogenesis, at least with hypoplasia of the adrenal cortex. Congenital
adrenal hyperplasia - a group of hereditary diseases (p), in which disrupted the
biosynthesis of cortisol in the adrenal cortex due to a defect of one of the enzymes or
transport proteins. Reduced synthesis of cortisol causes overproduction of ACTH, which
leads to hyperplasia of the adrenal cortex and the accumulation of metabolites preceding
stage of a defective steroidogenesis. Excrete 5 variants of congenital dysfunction of adrenal
cortex, with different manifestations in children (Table 2-20).

Table 2-20. Clinical variants of congenital dysfunction of adrenal cortex

Shape Clinical manifestations

Deficiency steroidogennogo protein Syndrome of salt loss


STAR (congenital lipoid adrenal
hyperplasia) False male hermaphroditism

Hypogonadism
Lack of 3-p-hydroxy-steroiddegi Classical form: a syndrome of salt loss, a false male and female
drogenazy hermaphroditism

Non-classical form: premature adrenarche, menstrual irregularities,


hirsutism, acne, infertility
Lack of 21-hydro-ksilazy (gene Classical form: a syndrome of salt loss, a false female
CYP2I) hermaphroditism (prenatal virilization), non-classical form:
premature adrenarche, menstrual irregularities, hirsutism, acne,
infertility
Lack of 11-p-hydro-ksilazy (gene Classical form: a false female hermaphroditism (prenatal virilization),
CYP11B1) arterial hypertension

Non-classical form: premature adrenarche, menstrual irregularities,


hirsutism, acne, infertility

Lack of 17-on:-hydro-ksilazy (gene False male hermaphroditism (delay sexual development in girls)
CYP17) Hypertension

-- Secondary congenital chronic adrenal insufficiency is caused by congenital disorders of


the hypothalamus or adenohypophysis.

-- Acquired primary chronic adrenal insufficiency (Addison's disease) is currently most


often develops as a result of autoimmune destruction of the adrenal cortex (autoimmune
Adrena-ta). Condition can be a component poliglandulyarnogo autoimmune syndrome type
I (Blizzard syndrome) or II (Schmidt's syndrome), may be associated with bilateral
tuberculous process in the adrenal glands, as well as histoplasmosis. The rare cause of
chronic adrenal insufficiency include Volmana disease (hereditary lipid storage disease)
Adrenoleukodystrophy (a hereditary disease, caused by the violation of the oxidation of
fatty acids), amyloidosis, tumors and metastases, etc.

-- Secondary acquired forms of chronic adrenal insufficiency (ACTH deficiency or


kortikoliberina) may be a consequence of prolonged treatment with glucocorticoids,
damage adenohypophysis with the hypothalamus or brain injury, after surgery or
radiation, with tumors, infections, etc. Acute adrenal insufficiency is characterized by
severe general condition of the patient , severe weakness, adinamii, anorexia, vomiting,
abdominal pain, loose stool, growing dyspnea, cyanosis, drop in blood pressure, weak
pulse, in some cases, convulsions, loss of consciousness, vascular collapse. Acute adrenal
insufficiency may develop with bilateral damage of the adrenal cortex with hemorrhage
due to birth trauma, venous thrombosis or embolism, a syndrome of DIC. Hemorrhagic
infarction of adrenal glands often occurs against a background of severe infections,
especially Me-ningokokkovoy, pneumococcal go streptococcal etiology. Acute hemorrhage
in the adrenal glands may be under stress, major surgery, sepsis, burns, treatment with
anticoagulants in patients with HIV infection. Acute adrenal insufficiency may occur when
the sudden cessation of treatment with glucocorticoids (withdrawal), as well as in patients
after bilateral hypercorticoidism Adre -nalektomii.

Hypercorticoidism

Hypercorticoidism (hypersecretion of glucocorticoids) can be primary, resulting


melkouzelkovoy or nodular hyperplasia of the cortex adenoma or malignancy of adrenal
glands, and secondary. The latter occurs when excessive secretion of ACTH
adenohypophysis and ectopic ACTH negipofizarnymi tumors (from neural crest cells, or
islet cells in thymoma, Wilms' tumor etc.). The clinical picture is defined in terms of what
glucocorticoids Secretary-tiruyutsya child in abundance.

Excess glucocorticoids may be due to glucocorticoid therapy (medical Itsenko-Cushing's


syndrome). In secondary hypercorticoidism bilateral diffuse hyperplasia of the adrenal
cortex is formed due to overproduction of ACTH associated with pituitary adenoma
(pituitary Cushing's), or ectopic ACTH-secreting-yuschey tumor (ectopic ACTH
syndrome). At the same time children are having a weakness, fatigue, hypotrophy of the
limbs, growth retardation. Characteristic increased appetite, obesity with preferential
deposition of fat on his face ( "moon face" with a bright blush on the cheeks) and in the
neck ( "buffalo neck"), the neck, above the collarbone, in the back and abdomen. On the
skin of the abdomen, chest, hips and shoulders appear stretching bands (striae), purple or
violet color, and hairiness. Osteoporosis, retarded bone age, hypertension, disturbed
glucose tolerance. In the blood reveal leukocytosis, lymphopenia, eosinopenia,
polycythemia, a tendency to increase blood clotting, gipokaliemichesky alkalosis,
giperholesteri-nemiyu.

Gipvrproduktsiya androgvnov estrogen and adrenal

Overproduction of androgens or estrogens observed in hormonally active adrenal tumors,


congenital dysfunction of adrenal cortex. The presence of virilizing tumor in boys leads to a
premature appearance of secondary sexual characteristics without increasing the testicles,
and girls - to hypertrophy of the clitoris, hirsutism and delayed menarche. Feminizing
tumor boys lead to gynecomastia and delayed puberty, and girls - to a false premature
sexual development.

Hypoaldosteronism

For Hypoaldosteronism (lack of development of aldosterone) from a number of symptoms


caused by hyperkalemia and hyponatremia, and their influence on renal function,
cardiovascular system and skeletal muscles. Patients noted fatigue, muscle weakness,
hypotension, occasionally fainting, bradycardia, heart block. Isolated deficiency develop
aldosterone are rare, in the presence of the enzyme defect in the glomerular zone of the
adrenal cortex, as well as after removal aldosteronoma in one adrenal and secondary
atrophy of the glomerular zone to another.

Watching and psevdogipoaldosteronizm due to low sensitivity of the receptors of the


epithelium of renal tubules to aldosterone.

Hyperaldosteronism

Hyperaldosteronism - excessive development of aldosterone, which causes hypertension,


metabolic alkalosis, hypokalemia, and sodium retention. It initially decreased daily urine
output, and then there are polyuria, polydipsia, nocturia, resistance to the antidiuretic
drugs. Hyperaldosteronism in children occur rarely. Hyperaldosteronism may be primary
and secondary.

In primary hyperaldosteronism occurs autonomous hypersecretion of aldosterone,


causing hypokalemia, and mild to moderate hypertension. Primary hyperaldosteronism
(Conn's syndrome) due to aldosteronoma (aldosteronsekretiruyuschey adrenal adenoma)
or melkouzelkovoy bilateral diffuse hyperplasia of the adrenal cortex in children reveal
rare. Hyperaldosteronism, amenable to treatment with glucocorticoids, is a family form of
the disease (9?), At which a duplication of the gene 11-B-hydroxylase.

* Secondary hyperaldosteronism may develop in a number of diseases involving


hypovolemia and ischemia of the kidney (renal parenchymal disease, renovaskulyarnoy
pathology, prolonged hypertension, renin-secreting tumors, Schwartz-Barter syndrome in
violation of chloride reabsorption in the renal tubules, and heart failure, treatment with
diuretics, liver accompanied by ascites, etc.).

Violations of the secretion of catecholamines

When the excess secretion of catecholamine in patients monitored weakness, fatigue,


sweating, decreased appetite, weight loss, headaches, blurred vision, tachycardia,
peripheral vasospasm that defy treatment of hypertension, which can proceed with the
crisis or be constant. Excess catecholamines arise when feohromotsi-volume and other
tumors of chromaffin tissue. Hypersecretion of catecholamines may occur when a large
physical exertion, stress, pain syndrome.

Insufficient secretion of catecholamines as an independent endocrine-patiya occurs only


rarely and may be one of the causes of hypo-glycemic syndrome.

Pancreas
Endocrine part of the pancreas - islets Langerhan-sa - clusters of cells that make up
approximately 1.5% of the gland. Langerhansa islands are composed of several types of
endocrine cells, synthesizing and secreting various hormones (Table 2-21).

Table 2-21. Hormones islands Langerhansa

Type of Synthesized Action


cells hormone
a-cells Glucagon Consider as an antagonist of insulin (causes an increase in the concentration of
glucose in the blood): stimulates the cleavage of glycogen (glycogenolysis) in
liver and muscle, activates gluconeogenesis, stimulating lipolysis
R- Insulin Enhances anabolism and inhibits catabolism. Regulates carbohydrate
metabolism, causing a decrease in blood glucose concentration (increases
cells glucose transport in cells insulin-dependent tissues - liver, skeletal muscle,
adipose tissue, stimulates the synthesis of glycogen, inhibits gluconeogenesis
and glycogenolysis).

Stimulates the transport of amino acids across the membrane of cells and
protein synthesis in the liver and skeletal muscle, inhibits proteolysis.

Promotes the synthesis of triglycerides in adipose tissue,

inhibits lipolysis.

Stimulates cell proliferation


5 -Cages Somatostatin Inhibits Growth hormone releasing hormone, secretion Gast-rina, glucagon,
insulin, renin, secretin, gastric juice, pancreatic enzymes and electrolytes

The secretion of insulin depends on the concentration of glucose in the blood. Stimulate the
secretion of an increased concentration of potassium ions, acetylcholine, and Gast-rin-
releasing hormone, and inhibit - somatostatin, adrenaline and noradre-Nalin. Glucose
inhibits glucagon secretion.

Anatomic and physiologic features

By the time of birth relative amount of endocrine tissue in the pancreas is reduced
compared with that of the fetus is almost 7 months to 2 times. By the age of 1,5 month, it
increases again and reaches 6% of the entire mass of the pancreas. By the end of the first
year of life mass of endocrine tissue is gradually reduced from 6% to 2,5-3% and
maintained at that level during the whole period of childhood.

The release of insulin significantly increased during the first days of life, then decreases.
Newborn glucose weakly stimulates the secretion of the hormone. In the first hours after
birth for children characterized by a considerable rise in the concentration of glucagon, the
main regulator of its release in the perinatal period is an alanine. The concentration of
somatostatin during the whole period of childhood did not significantly change.

Survey methodology and semiotics SHOCK

To assess the endocrine function of the pancreas are concerned primarily with the state of
carbohydrate metabolism: evaluate the concentration of glucose and its dynamics in the
blood during physiological loading by carbohydrates. If no changes were standard oral
glucose tolerance test. Transient hyperglycemia reveal the concentration of glycated
hemoglobin. In urine determine the concentration of glucose, ketone bodies.
To assess the functional state of L-cells of the pancreas determine the concentration of C-
peptide in blood, free and immunoreaktiv-tion of insulin in the blood serum. Also examine
the blood for the presence of AT to R-Ag cells. Radioimmunological method determines
the concentration in the blood glucagon, gastrin, somatostatin.

Hunger disease

Hunger disease are relatively rare. The most frequent causes of its development in children
- the P-cell hyperplasia of the pancreas, and insulinoma nezidioblastoz. Hunger disease
monitored every 2 nd child Bekuitta-Wiedemann syndrome and children with intolerance
to leucine. Transient hyperinsulinemia occurs in infants born to mothers with diabetes or
diabetes during pregnancy, as well as on the background of HDN.

Hunger disease manifested syndrome of hypoglycemia. In newborns and infants with


clinical symptoms of hypoglycemia include weakness, drowsiness, hypothermia, languid
sucking, cyanosis, apnea, seizures. An important feature of persistent or recurrent
hyperinsulinemia - macrosomia. In older children hypoglycemia manifested anxiety,
general weakness, dizziness, feeling of hunger, trembling fingers, cold sweat, tachycardia,
pain in the heart, dilated pupils (due to increased secretion of adrenaline). In addition, in
relation to carbohydrate starvation of the brain appear sleepy, breach of attention,
headache, impaired consciousness, convulsions, coma may develop.

Diabetes

The cause of diabetes - insulin deficiency or resistance of target cells to its effects. Diabetes
mellitus is divided into 2 main type. Type I, insulin, occurs due to insufficient insulin
secretion due to destruction of (3-cells. In most cases, the cause of Type I diabetes is an
autoimmune process. The disease can be induced by viral infections and certain toxic
substances.

Classic clinical manifestations of diabetes mellitus - polydipsia, polyuria, young children


often arise enuresis, polydipsia, weight loss, dry skin and mucous membranes, polyphagia,
weakness, fatigue, frequent infections. Infants note greed in nursing, decrease or stop
weight gain, "a symptom of starch diapers, oprelosti, stomatitis. Basic biochemical features:
hyperglycemia, glycosuria, ketonuria, lack of or low concentration of insulin and C-peptide
in blood.

Diabetes mellitus type II, insulin, is associated with insulinore resistance of (relative insulin
deficiency). The disease rarely occurs in children (mainly in the pubertal age). Monogenic
forms of insulin-dependent diabetes mellitus caused by defects in single genes, and
typically initially impaired insulin secretion, but the majority has a polygenic nature, in
which the primary violation is insulin resistance. For non-genetic risk factor for insulin
diabetes include obesity, in which there is insulin resistance, and in severe cases and in
violation of insulin secretion, as well as overeating, sedentary lifestyle, stress, early
childhood - intrauterine developmental delay, fasting.

Secondary diabetes may be due to diseases of the pancreas (cystic fibrosis,


hemochromatosis, pankreatektomiey) or an excess of glucocorticoids.

Mutation of glucagon and its receptor

Glucagon gene mutation leads to severe hypoglycemia, and activating its receptor gene
mutation - a form of non-insulin dependent diabetes mellitus.

Gonads
To gland include endocrine cells of male and female gonads.

Testicles

The testicles (male sex glands) - paired glandular organ composed of lobules. Dolka
includes convoluted seminiferous tubules lined spermatogenic epithelium containing
gametes from their predecessors, and supporting Sertoli cells. Between the tubules in the
connective tissue cells are Lyaydiga. Convoluted tubules continue to direct that flow into
the network of the eggs, and then pass into the efferent tubules appendage. Cells Lyaydiga
represent typical endocrine cells, synthesizing the male sex hormones - androgens
(testosterone) and estrogens. Testosterone is associated with the transport proteins in
tissues and eggs - with a specific androgensvyazyvayuschim proteins secreted by Sertoli
cells, thus supported by its high concentration in the spermatogenic epithelium. In
addition, the Sertoli cells is the conversion of testosterone into estrogen, is synthesized
ingibina.

Testosterone controls the development of external genitalia, prostate and seminal vesicles;
determine the growth of body hair in male pattern, the expansion of the larynx and
thickening of the vocal cords; helps to increase muscle mass, erythropoiesis, causes a
change in the chemical composition of sebum, has anabolic effects.

Ovaries

Ovaries (female sex glands) - paired organ located in the pelvis. Endocrine function of
sexual glands regulate the hypothalamic-pituitary system. Ovarian parenchyma consists of
the cortex, which contains follicles at different stages of maturation, yellow and white body
and brain substance, formed by connective tissue. Endocrine ovarian function is the
synthesis of estrogen and progesterone in follicular cells cells of the corpus
luteum. Moreover, in cells of the cortex and medulla is the synthesis of andro-genes.
Estrogens (estradiol, estrone and estriol) stimulate the proliferation of follicular cells and
the expression of new receptors for FSH and steroids, prevent atresia of the follicle, control
the proliferative phase of the menstrual cycle (the restoration of a functional layer of the
endometrium). They are necessary for the maturation of female reproductive organs,
stimulate the development of ducts and stroma of mammary glands, determine the
distribution of fat on the feminine type, causing the closure of epiphyses and cessation of
linear growth, are involved in the metabolism of lipids, calcium ions and the process of
blood clotting.

Progesterone synthesized luteal cells. He controls the secretory phase of the menstrual-
ovarian cycle - preparation of the endometrium for implantation, stimulates the
development of glandular breast tissue.

natomic and physiologic features

In the newborn testis weight is 0.3 grams and dimensions - 10x7 mm. By the year testicular
size increased to 14x9 mm, in 2-5 years - 16x10 mm. To the 10-year length of the testicle M
increases in 2-2,5 times (up to 20-25 mm) and weight - up to 2 years in the adult testis size
is 30-50x20-30 mm and weight - about 20 . newborn seminiferous tubules and tubules
networks do not have a lumen, which appears to the period of puberty.

Sexual development of boys divided into 3 periods: dopubertatny (from 2 to 6-7 years) -
during the hormonal resting, prepubertal (from 6 to 10-11 years), characterized by
increased synthesis of adrenal androgens and the formation of morphological structures of
the testis, and puberty (from 11 - 12 years), when under the influence of testosterone
formed secondary sexual characteristics. First appearing pigmentation and multiple small
folds on the scrotum, the testicles grow and fall on her bottom and starts to grow penis,
body hair is pubic, there's hair in the armpit, upper lip, cheeks, chin. Increasing the larynx,
the mutation is to vote to change the size of the prostate gland, gradually strengthen the
processes of spermatogenesis.

Newborn girl length of the ovaries is 0,5-3 cm, they are cylindrical in shape, smooth surface
and are located high above the entrance to the small pelvis. By 5.7 years ovaries occupy its
normal position, get an egg shape. For 16 years the ovaries is much thicker, and the length
is increased by an average of 0,6 cm

In the sexual development of girls distinguish 3 periods: the neutral (first 5-6 years),
prepubertal (from 6 to 9-10 years) and adolescence (before puberty). In the neutral period,
sex hormones have on growth and development of the child minimal impact. At puberty
under the influence of gonadotropic hormones increases the growth of follicles, increased
synthesis of estrogen. In this period I architectonics of the body, developing breasts,
growing external and internal sex organs, changed the structure of the endometrium. With
increased concentration of estrogen comes first menstruation (menarche), the average
timing of which are 12,5-13 years.
Survey methodology and semiotics SHOCK

On examination, the child pay attention to the growth, the distribution of adipose tissue,
body proportions, muscle development, body hair. Benchmarked expression of secondary
sexual characteristics: the girls - the development of mammary glands, body hair and pubic
hair development in the armpits, the establishment of menstrual function, and the boys -
body hair pubic area, armpits and face, the growth of the thyroid cartilage, changes in
voice. Stages of puberty is determined from Tanner.

Stages of puberty in boys.

I stage. The penis, testicles and scrotum children. Sexual body hair is absent.

II stage. The increase in the testicles and scrotum, the penis is usually not increased.
Scrotal skin sags, becomes a reddish color. Growth of rare, long, slabopigmentirovannyh
hair hair straight, sometimes curled, mainly at the base of the penis.

III stage. Further increase in the testicles and scrotum and the penis increases, mainly in
length. The hair becomes darker, coarser, more curled, slightly extended to pubis.

IV stage. Continuing increase in the testicles and scrotum, the penis increases, mostly in
diameter. Scrotal skin darkens. Sex male pattern body hair, but does not extend to the
inner thighs.

V stage. External genitalia in shape and size correspond to the organs of an adult
male. Sexual body hair extends to inner thighs.

Stages of puberty in girls.

I stage. Mammary glands are not developed, the nipple is elevated. Pubic body hair is
absent.

II stage. The development of breast cancer to the stage of "bud", the beginning of hair
distribution (an increase of rare, long, slabopigmentirovannyh hair hair straight,
sometimes curled, located along the labia).

III stage. Further increase in breast and areola with no separation of their contours. Hair
darken, become more coarse, curly, apply to the pubis, appears underarm body hair.

IV stage. Protrusion of the areola and nipple with the formation of tubercles on the contour
of the secondary cancer. Crab Women's body hair type, but does not extend to the
perineum and inner thighs. The appearance of acne. Menarche.
V stage. Mammary glands correspond to those of adult women areole fits into the general
contour of the breast. Body hair extends to inner thighs. Regular menstruation.

When genital examinations draw attention to the full value of their buildings. The boys can
identify abnormalities such as hypospadias (cleft of the lower wall of the urethra),
epispadias (cleft of the upper wall of the urethra), hypoplasia of the penis
(MICROPENIS). Palpation boys determine the presence of testes in the scrotum, estimate
their size and texture. In girls, watching such anomalies as the hypertrophy of the clitoris,
seam (synechiae), small or large labia, hymen neperforirovannost, aplasia of the labia and
the hymen.

If necessary to ultrasound of the pelvic organs in girls testicles in boys. To assess the
function of sexual glands determine the concentration of gonadotropins, sex hormones in
the blood.

Anomalies of internal genital organs

Monorhizm - absence of one testicle. Cryptorchidism - a condition in which one or both


testicles not descended into the scrotum, with testes located in the wrong place
degenerative changes in germinal epithelium and a decrease in the number of
spermatogonia. As a result of disorders of spermatogenesis patients with bilateral
cryptorchidism are often fruitless. When inguinal cryptorchidism testes are located in the
inguinal canal, during typhoid - in the abdominal cavity.

Anomalies of the ovaries include congenital hypoplasia of one or both ovaries.

Anomalies of sex chromosomes

Anomalies of sex chromosomes usually clinically apparent delayed sexual development, a


violation of the formation of secondary sexual characteristics, infertility, spontaneous
abortion.

Turner's syndrome (karyotype 45, X) is characterized by a low-riser, gonadal


dysgenesis. Characteristic short neck with a wing-skin folds, low line of hair growth on his
neck, barrel chest, X-shaped curvature of hands, X-shaped curvature of the legs, etc.

Trisomy of chromosome X (karyotype 47, XXX) is characterized by a high-riser, mental


retardation, epilepsy, amenorrhea, infertility.

Klyaynfeltera syndrome (karyotype 47, XXY) is characterized by tall-Stu, evnuhoidnym


physique, small size of the testicles, a violation of the formation of secondary sexual
characteristics, infertility.
Karyotype 47, XYY is characterized by high growth, delayed speech development,
aggression, violation of adaptation.

Fragile X syndrome chromosome in males is characterized by high growth, mental


retardation, makroorhizmom.

Disorders of sex differentiation

Disorders of sexual differentiation - the presence of a newborn intermediate between male


and female type external genitalia.

In true hermaphroditism from both sides have ovotestis (testis and ovary as a single
entity), or on one side of the egg, but on the other - the ovary.

False female hermaphroditism (virilization in the female karyotype) occurs when a


congenital dysfunction of adrenal cortex, an excess of Al-drogenov pregnant as a result of
iatrogenic effects.

False male hermaphroditism (incomplete masculinization in the male karyotype)


develops with testicular dysgenesis, violation of synthesis and metabolism of testosterone
to testosterone insensitivity (testicular feminization syndrome), etc.

Hypofunction of sexual glands

Hypofunction of sexual glands of a boy diagnosed in the event that the age of 14 years,
there were no changes in puberty, but the girl - if older than 14 years, not all the secondary
sexual characteristics, or 15.5 years was not menstruating. Excrete functional (delay of
sexual development) and organic (hypogonadism) options. Delayed sexual development
may be constitutional, as well as develop chronic somatic diseases, malnutrition, expressed
deficits in body weight, physical and mental overload, etc.

Hypogonadism

Hypogonadism is divided into primary (hypergonadotropic) due to direct damage of the


sex glands, and secondary (gipogonadotropny), caused by the weakening of the
gonadotrophic function of the pituitary or hypothalamus. Hypogonadism may be
congenital or acquired.

Causes of primary hypogonadism may be congenital anomalies of the sex glands,


infection, autoimmune disease, trauma, surgical damage, radiation therapy, vascular
occlusion, etc.

Secondary hypogonadism is due to antenatal or postnatal CNS pathology,


neuroinfections, brain injuries, tumors, surgical destruction of the pituitary and other
Gynecomastia

Gynecomastia (breast enlargement in boys) may occur in various diseases. The most
frequently observed youthful (physiological) gynecomastia in healthy boys during puberty,
which itself passes through 1-2 years. Pathological gynecomastia may be caused by
disorders of synthesis, secretion or action of androgens, an excess of estrogen synthesis
estrogenprodutsiruyuschey testicular or adrenal tumors, hyperprolactinemia, medication,
in particular the gonadotropins, antiandrogens, estrogens, methyldopa, reserpine,
spironolactone, phenothiazine derivatives, etc.

Premature sexual development

Premature sexual development - the onset of sexual maturation in girls up to 8 years in


boys before 9 years. The true form of premature puberty associated with pathologically
early activation of the hypothalamus or pituitary gland, which leads to excessive secretion
of gonadotropins, stimulating activity of the gonads. This condition can develop in organic
brain disease caused antenatal and intrapartum CNS lesions, increased intracranial
pressure, tumors of the CNS. False premature sexual development due to increased
secretion of sex hormones, independent of the gonad-tropic stimulation. Occurs when
gormonprodutsiruyuschih testicular tumors in boys and ovaries in girls, with
gormonprodutsiruyuschih adrenal tumors, and may also be due to iatrogenic causes, etc.