ADRENAL GLANDS [glandulae suprarenales (PNA); synonym epinephral glands] — the pair bodies of incretion located in retroperitoneal space over upper poles of kidneys. Each N. consists of internal marrow (medulla) and outside cortical substance (cortex); falls to the share of marrow apprx. 20% of mass of gland. Marrow and cortical substance are dvukhmya various by origin, to a structure and function by the glands which are put separately and combined in the course of phylogeny and ontogenesis in morphologically uniform body.
- 1 History
- 2 The comparative anatomy and embryology
- 3 Topography
- 4 The anatomy and histology
- 5 Physiology
- 6 Methods of a research
- 7 Pathological anatomy
- 8 Pathology
- 9 The principles of an operative measure on adrenal glands
- 10 Some features of physiology and pathology of adrenal glands at children
For the first time N. were described by B. Evstakhy in 1563, but value is found only rather recently out them fiziol. Oliver and Schaefer (G. Oliver, E. A. Schafer) in 1894 showed that introduction to an organism of extract of marrow H. raises the ABP. In 1902 Mr. J. J. Abel emitted from marrow H. biologically active compound in a crystal look and called it Epinephrinum. In 1927. Rogov and Stewart (J. Rogoff, G. H. Stewart) by means of salt extract from N. could prevent death of adrenalectomized dogs; the same effect (in their later researches) the fat-soluble fraction of cortical substance of adrenal glands had. During the period from 1936 to 1954 were allocated, received in a crystal look and the main are chemically identified corticosteroids (see). G. Ping-kus et al. (1954) offered the scheme of stages of biosynthesis of corticosteroids, edges was specified and added with N. A. Yudayev's works and sotr. (1963 — 1971).
The comparative anatomy and embryology
Interrenalovy body (a mezhiochechny, or so-called epithelial adrenal gland) corresponding to cortical substance H. [cortex Suprarenalis (PNA), substantia corticalis (BNA, JNA)] in phylogenesis appears later, than the chromaffin (adrenal) bodies similar to marrow H. [medulla Suprarenalis (PNA), substantia medullaris (BNA, JNA)].
The lowest fishes have metamericly located chromaffin bodies and separate chromaffin cells which are in walls of cardinal veins and giving characteristic reactions (coloring of cells in brown color). The Interrenalovy body in the form of an unpaired tyazh of ferruterous cells for the first time appears from a mesoblast (an average germinal leaf) at shchelezhaberny animals in back epinephral area, reaching front poles of kidneys. Close pair adrenal bodies (bodies) form. At cyclostomous animals separate cells of interrenalo-vy bodies are located near walls of an aorta and cardinal veins near chromaffin (adrenal) bodies, to-rye in the form of continuous strips between an aorta and cardinal veins stretch from the second couple a zhaber to a tail.
The first (partial) connection of adrenal bodies with interrenalo-vy is observed at bony fishes. Fuller association of interre-nalovy and adrenal cells in the general body — an adrenal gland, happens at amphibians at which future chromaffin cells (hro-maffinoblasta) get in body therefore interre-nalovy cells appear on its periphery. At reptiles and birds interrenalovy and adrenal parts are even more closely united in one body. However at them the peresloyeniye tyazhy interrena-lovy cells (cortical substance) from tyazha of chromaffin cells still remains (marrow).
At mammals chromaffin cells (hromaffinotsita) concentrate in the central part H., and interre-nalovy cells, i.e. cortical substance outside are located. Nek-ry mammals on border cortical and marrow have an expressed layer from connecting fabric — the so-called capsule of marrow, an origin connect a cut with process of association of chromaffin and interrenalovy cells.
Reptiles, birds, and also for nek-ry mammals (insectivorous, rodents) have numerous additional groups of interrenalovy cells — so-called interrenalovy bodies, or additional adrenal glands [gll. suprarenales accessoriae (PNA)]; they lie gl.obr. on the course of large vessels. Chromaffin educations in the form of the free additional bodies similar on a structure and biochemical reactions to marrow H. — a paraganglion (paraganglia, PNA) are found in the person, a rabbit and other mammals in nodes of a boundary sympathetic trunk, nodes large vegetative (celiac) textures (see. Paragangliya ).
The process of formation of N. in phylogenesis subdivided into stages of separate existence of pnter-renalovy and adrenal bodies, their partial, and then full association is considered as development of interrenalovy system. The reasons of association two various by origin, cortical and marrow up to the end are not clear to a structure and function yet.
At animal N. are located between cranial poles of kidneys and the main vessels (a caudal vena cava and a ventral aorta). By outward of N. of the person and animals differ. At animals, depending on their look and the sizes, N. in a form remind small haricot (rodents, a cat) or have the form of a bean (dog).
At an embryo of the person N. develops from two various rudiments. Cortical substance is derivative a coelomic epithelium (mesoderm). At an embryo 6 — 8 mm long medially from laying of primary kidney appears cellular tyazh, to-ry in the course of growth plunges into the subject connecting fabric where N. (fig. 1) forms further. Marrow H. has an origin, the general with a nervous system, and develops from embryonic sympathetic cells, to-rye at embryos 16 mm long begin to grow into accumulation of interrenalovy cells (an epithelial adrenal gland). At an embryo 16 — 20 mm long together with sympathoblasts and hromaffinoblasta in N. outside, and also from the forming central vein connecting fabric begins to grow. Association of interrenalovy and adrenal cells happens to a differentiation of cellular elements and layers cortical and marrow.
Regulation of development of N. in the embryonal period happens in uniform neuroendocrinal system: mother — a placenta — a fruit. The first signs of rudiments of cortical substance H. appear on 4 — the 5th week of fetation; on 7 — the 8th week two zones clearly come to light: fetalis (germinal) and constant cortical substance (the most part — to 80% — the germinal zone makes). After the 20th week the strengthened development of constant cortical substance begins. The major control factor of development of N. in the antenatal period is adrenocorticotropic hormone (AKTG) of a hypophysis of a fruit, to-ry begins to be emitted with the 20th week of pregnancy. In this period control from outside is established gipotalamo-pituitary system (see) for morfofiziol. development and function of cortical substance. In the first half of pregnancy the regulating role is rendered, apparently, by chorionic gonadotrophin (see), to-ry activity of germinal cortical substance N influences development and funkts.
Enzymatic systems of a steroidogenesis form from 8th week of embryonic development; since 21st week cortical substance has gormonosinteziruyushchy activity. Up to 21st week it contains only trace quantities of a hydrocortisone that is connected with insufficient products of AKTG a hypophysis of a fruit. Since 21st week products of a hydrocortisone and its allocation in blood quickly increase. Funkts, activity of cortical substance in the first weeks of fetation is defined by hl. obr. gradual strengthening of function at the expense of constant cortical substance happens germinal cortical substance, further. Funkts, activity of both germinal, and constant cortical substance is close to activity of cortical substance of the adult.
A metabolic barrier between hormones H. of a fruit and maternal N. is the placenta — a temporary endocrine organ.
The glucocorticoids formed in excess quantity at a patrimonial tension get into blood of the newborn, to-ry it is overloaded with maternal hormones. It leads to sharp reduction of secretion of AKTG and involution of a germinal zone of cortical substance. The mass (weight) of N. after the birth of the child decreases due to involution of a germinal zone of cortical substance that is followed by considerable decrease in glucocorticoid function. In cortical substance it is possible to find in the newborn narrow the glomerular and developed puchkovy zones; the mesh zone forms a little later.
Post-natal changes in cortical substance up to the period of puberty come down to completion of differentiation of zones. By 18 — 20 years N.'s development comes to the end.
At the person N. are located at the level of XI — the XII chest vertebrae zabryushinno, over poles of kidneys. Behind and from above they prilezhat to a lumbar part of a diaphragm. Medially from the left N. the aorta lies, to the right N. in front of and from the medial party the lower vena cava prilezhit. In front the right N. adjoins to a liver and to a duodenum at its upper bend. In front and up from the left N. there is a tail of a pancreas with the splenic vessels lying along it, and also a cardial part of a stomach. A part of a front surface of the left N. is covered with a parietal peritoneum. Together with N.'s kidneys are put into an adipose capsule of a kidney (capsula adiposa) and are covered with a renal fascia (fascia renalis).
The anatomy and histology
In a form H. of the person reminds the cone flattened in the perednezadny direction with smoothed top, at to-rogo distinguish three surfaces: lobby (facies ant.), back (facies post.) and lower, renal (facies renalis). Concavity of the basis of N. corresponds to camber of an upper pole of a kidney. Front and back surfaces have the general upper and medial edges (margines sup. et med). The left N. on l ul the union in front has the form and it is extended in transverse direction, the upper corner is absent; the right N. has the form of a triangle with smoothed things over. On a front surface, especially left N., the well-marked horizontal furrow — gate of H is visible. (hillis); N.'s surface uneven, melkobugristy. N.'s length of the adult from 30 to 70 mm, width is from 20 to 35 mm, thickness is from 3 to 8 mm. Lump of both N makes 13 — 14 g.
Outside N. is covered with the connective tissue capsule, dense at a surface of body and more friable knaruzha. Along with collagenic, elastic and reticular fibers the capsule contains smooth muscle cells, nervous cells and small small knots, fatty segments, and also groups of cells of cortical substance (additional N.). Accumulation of cells of cortical substance in the capsule is considered as an otshnurovka from a surface layer of gland (a so-called nodular hypertrophy of bark). Bunches of connective tissue fibers divide a parenchyma into groups of cells and cellular tyazh.
In cortical substance H. on the basis of distribution of connecting fabric and vessels, and also morfofunktsionalny features of ferruterous cells allocate three zones: glomerular, puchkovy and mesh (fig. 2 and tsvetn. fig. 3). The glomerular zone (zona glomerulosa) consists from various in a form and size of the cellular groups reminding balls, in each of to-rykh is to 5 — 6 large a cubic or polygonal form of cells with fine-grained acidophilic cytoplasm and a big kernel. In cells of a glomerular zone small lipidic inclusions are found, the sharing cells sometimes meet. Under a glomerular zone there is a layer of cells poor in lipids — a sudanofobny layer (transitional, or intermediate, a zone, zona intermedia).
Average, the widest, a zone — puchkovy (zona fasciculata) is formed by radially oriented columns of large secretory cells of a prismatic or cubic form with a big large kernel. In cells of this zone there are a lot of lipids, to-rye give to cortical substance on a section light yellow color and well come to light during the coloring of cuts of N. by Sudan III or IV, red sharlakh, osmium. Cells of a puchkovy zone after processing by fat-dissolving substances take a light spongy form in this connection they are called spongiocytes.
The internal zone, mesh (zona reticularis), consists of the cellular groups of the small size, a rounded or polygonal shape containing from one to several cells with acidophilic fine-grained cytoplasm, in a cut there are droplets of fat and grain of a pigment.
Synthesis of corticosteroid hormones is connected with mitochondrions of adrenokortikotsit (secretory cells of cortical substance). Cells of a glomerular zone contain a large number of mitochondrions of the extended form with the long, located bunches tubules. The quantity of mitochondrions increases in cells of an intermediate zone, they get an oval form, tubules in them are branched out, in mitochondrions puzyrkovidny educations appear. In cells of a puchkovy zone of a mitochondrion have the oval form and contain a large number of bubbles. In cells of a mesh zone of a mitochondrion remind short sticks and are evenly distributed on cytoplasm. The lamellar complex (Golgi's complex) in cells of cortical substance H. is presented in the form of network, thread a cut in cells of a puchkovy zone get between lipidic inclusions; in cells of a mesh zone the network close prilezhit to a kernel. In process of accumulation of a secret the complex is loosened, in its zone there are granules, cells take more dark look. In a stage of allocation of a secret when granules are enlarged, turn into vacuoles, cells become lighter.
Borders between zones are not always accurately expressed, especially in N. at nek-ry animals. Width and a structure of zones can change, especially at raised or lowered funkts, loadings (in an experiment) and at nek-ry patol, states.
Marrow is in the central department of N., the relation of its weight to the mass of cortical substance H. is equal to about 1: 3 (fig. 3). On a cut in the sagittal plane marrow in N. of the person in a form reminds a plate up to 5 mm thick with the thinned edges. Cells of marrow are located in the cells formed by bunches of connective tissue fibers, to-rye, on the one hand, are interwoven into a cover of the central vein of N., with another — pass into a connective tissue skeleton of cortical substance. In each cell there are from 2 to 6 cells to dia. 25 — 30 microns, a cylindrical, cubic and polygonal form with fine-grained cytoplasm and a big light kernel. Ferruterous cells of marrow are painted by salts of chrome in yellow-brown color; because of these Shtil-ling properties (V. of Stilling, 1889) Kohn (A. Kohn, 1889) — chromaffin, Poll (N. of Poll, 1906) — - pheochrome suggested to call them chromophilic. Allocation by methods of light microscopy of two types of secretory cells (an epinefrotsita and norepinefrotsi-you) was confirmed at their further researches. One cells contain larger chromaffin granules (to 0,6 microns), others — smaller (to 0,1 microns).
N.'s blood supply is carried out by the numerous arteries creating a plentiful vput-riorganny bed (tsvetn. fig. 1). Each N. receives blood from three groups of adrenal arteries: upper (aa. suprarenales sup., from 1 to 24), departing from the lower phrenic artery; averages (aa. suprarenales mediae, from 1 to 4), beginning from a ventral aorta; lower (aa. suprarenales inf., from 1 to 6), departing from a renal artery and its large branches. Besides, the non-constant (additional) adrenal arteries (to 20) which are branches of a celiac trunk, lower phrenic, upper mesenteric, ovarian and other arteries participate in N.'s blood supply.
In the capsule H. of an artery are divided into the small branches passing in the capsule and in a parenchyma of body. In cortical substance of an artery branch on capillaries to dia. 5 — 25 microns forming a uniform three-dimensional capillary network of cortical substance, a very techtonic dance a cut correspond to a design of a stroma and a parenchyma of this part of body. Loops of capillaries surround groups of cells of cortical substance from all directions so each cell prilezhit to one or several capillaries. Marrow receives blood from so-called own arteries (aa. perforantes, dd. medullares), getting into this part of gland from the capsule. Own arteries of marrow in the central department form a capillary network, in to-ruyu pass capillaries of a mesh zone of cortical substance. The wide capillaries (with a diameter up to 30 microns) called by sinusoidny circulatory capillaries (vas he-mocapillare sinusoideum), lying between groups of cells of marrow create conditions of the slowed-down blood flow that we promote more global exchange between secretory cells and blood.
Outflow of blood from N. is carried out through the central vein (v. centralis) forming in a mesh zone and in marrow; at the left it falls into the left renal vein, on the right — into the lower vena cava. Other outflow tract is carried out on the numerous superficial veins forming in a surface layer of cortical substance and in the capsule H. and falling in lower phrenic, renal veins, veins of an adipose capsule of an iochka (inflows of the lower vena cava), and also in veins of a stomach, a pancreas and at the left in a splenic vein (inflows of a portal vein of a liver). Between system of the central vein Ii. and an intraorganic venous anastomosis, on the Crimea blood is available superficial veins, and together with it and hormones from brain and cortical substance H. can flow in superficial epinephral veins, and through them in a portal vein of a liver.
Receipt of catecholamines and corticosteroids with blood from intraorganic veins of N. in a portal vein is proved. Regulation of a blood flow in intraorganic veins of N. is carried out by means of well developed muscular coat of veins having muscular thickenings — rollers (fig. 4); thanking etomu'postupleny blood and the hormones which are contained in it in an adrenal vein (v. Suprarenalis) it is limited, the blood flow goes on an intraorganic anastomosis to superficial veins of N., in walls to-rykh there are also muscular devices for regulation of outflow of blood.
The lymph drainage is presented limf, the capillaries lying together with circulatory capillaries in connective tissue partitions between groups of ferruterous cells in cortical and marrow H. and forming three-dimensional network. From limf, capillaries of cortical substance the vessels creating in the capsule I are formed limf. a texture, originative povepkh-nostny taking away limf, to vessels. Deep taking away limf, N.'s vessels leaving body through its gate together with the central vein are formed from limf, capillaries of marrow and a mesh zone of cortical substance.
The innervation is carried out by the nerve fibrils which are a part celiac, wandering, and also phrenic nerves. Shelter nosny vessels of N., including a wall of the central vein, are innervated by sympathetic and parasympathetic fibers of celiac and vagus nerves. Cross innervation of II is proved. the fibers which are a part of celiac nerves. The nerve fibrils entering N. on the course of blood vessels or independently form the texture containing small nervous small knots in the capsule. From this texture the nerve fibrils which are carrying out an afferent and efferent innervation get into N.'s parenchyma, being located in bunches of connecting fabric between groups of cells.
In all layers of cortical substance and in marrow the large number of nerve fibrils and the receptor terminations of various form is revealed. On border cortical and marrow, and also in mesh and even in a puchkovy zone on the course of nerve fibrils are found various size a microganglion, belonging postganglionic to neurons. The innervation of marrow is carried out as postganglionic!!, and preganglionic fibers of celiac nerves. A part of axons of the first neurons of an efferent way from the side horns of a spinal cord going as a part of celiac nerves is interrupted in nodes of a celiac texture, and also in the small small knots lying in the capsule in cortical and marrow N. Other part of preganglionic fibers of celiac nerves reaches chromaffin cells of marrow, to-rye are modified cells of a sympathetic part of a nervous system and correspond to postganglionic neurons.
Transfer of an impulse to cells of marrow is carried out directly, without participation of postganglionic neuron. The secretory role of celiac nerves was proved in 1910 by M. N. Cheboksaro-ey who tracked increase in blood pressure after irritation of celiac nerves. Afterwards it was shown that during irritation of celiac nerves there is not only an increase in content of adrenaline in blood, but preferential receipt it in a portal vein at decrease in concentration of this hormone in blood of the lower vena cava.
Celiac nerves are also secretory nerves for cortical substance of adrenal glands. By M. R. Sanin (1974) it was established that impact on a vagus nerve reduces intake of adrenaline in blood.
N.'s Structure changes with age. The average mass (weight) of both N. at the newborn makes apprx. 6 g. Cells of cortical substance of an adrenal gland at the newborn are poor in lipids. The degrowth of N. in the first days after the birth to 3.5 g occurs at the expense of a resorption of inner layers of cortical substance (a germinal zone). At the expense of an outside part of cortical substance glomerular and puchkovy zones form, and the mesh zone is formed of the remains of a germinal zone. Edges the newborn had N.'s mass, is recovered only by 5 years; further N.'s mass gradually increases and at the adult reaches 13 — 14 g (depending on a yole and funkts, states). The differentiation of cells of cortical substance proceeds up to 11 — 14 years when the differentiation of zones inherent to the adult is traced. Formation of marrow H. comes to an end by the period of puberty. By 20 years the ratio of width of zones of cortical substance is equal to 1:1:1; on the third — the fifth decades puchkovy and mesh zones extend a little, especially puchkovy zone, the relation of width of zones makes 1:2:2, and to 50 godakhm 1:3:2. Age changes of a connective tissue skeleton of N. are insignificant and connected with reorganization and a differentiation of cellular groups and layers.
In cortical substance H. sexual features are well-marked. At women contain rather trace amount of lipids in puberal age of a cell of a puchkovy zone. During pregnancy in cells of a mesh zone the quantity of lipids increases. After 40 years the mesh zone gradually becomes thinner, in a climacteric almost all cortical substance is occupied by a puchkovy zone. The structure of cortical substance is influenced by various factors of surrounding and internal environment.
On usual roentgenograms N.'s shadow is not visible. The form and the sizes H. can be established by method pneumoretroperitoneum (see), i.e. at a research in the conditions of stratification of the fabrics surrounding with N., gas, in particular if the X-ray analysis at the same time is combined with tomography (see). Against the background of gas kidneys and N. are well visible, to-rye have the form of a triangle with the basis turned to an upper pole of a kidney. Proyektsionno on the left N.'s shadow is imposed a shadow of a stomach, is more rare in the field of right N. the duodenum is projected. All bodies adjoining on N. and fatty accumulations, being imposed on N.'s image on the roentgenogram made in the conditions of a pneumoretroperitoneum can create differentsialno - diagnostic difficulties. On roentgenograms normal N.' sizes in length and width fluctuate from 1 to 3 cm. The left N. quite often happens slightly more right; contours their equal, structure of a shadow homogeneous; heterogeneity, a yacheistost of a shadow is occasionally observed.
N. are closed glands. The hormones produced by them possess big time-noobraziyekhm biol, properties and a broad spectrum of activity on exchange processes, participate in regulation of the vital functions of an organism as in usual fiziol, conditions, and in the course of adaptation of an organism to the changing conditions of the environment, including and at influence of extreme factors.
In marrow H. are synthesized catecholamines (see), to the Crimea belong adrenaline (see), noradrenaline (see) and dopamine. They possess the expressed action on carbohydrate, fatty, electrolytic exchanges, participate in regulation of function of cardiovascular system, influence excitability of a nervous system and sokratitelny function of smooth muscles. Action of catecholamines can change depending on the level of secretion of hormone.
In cortical substance H. are synthesized corticosteroids (see). In a glomerular zone of cortical substance are produced mineralokortikoidny hormones (see), to-rye play the defining role in maintenance of balance of electrolytes and liquid in an organism (see the Water salt metabolism). The average puchkovy zone of cortical substance is the place of education glucocorticoid hormones (see), to-rye participate in regulation of main types of exchange practically in all body tissues and together with other hormones provide constancy of internal environment. During the strengthening of glucocorticoids in blood the brightest effects are strengthening of a gluconeogenesis, braking of synthesis of protein and nucleinic to - t, a lipolysis, decrease in permeability of cellular membranes. Glucocorticoids, especially corticosterone (see), also have effect on mineral metabolism. In internal, mesh, a zone of bark H. sex hormones are synthesized — androgens (see) and is oestrogenic (see), however they make only an insignificant part of sex hormones in an organism, their ground mass is produced by gonads.
Regulation of functions H. is carried out in various ways. Secretion of catecholamines is under the regulating influence of a nervous system which is carried out through a celiac nerve. Secretion of glucocorticoid and sex hormones is regulated kortikoliberiny and adrenocorticotropic hormone (see), to-ry influences also proliferative processes in cortical substance N. Long strengthening of AKTG in blood leads to increase in mass of N.; the hypophysectomy, on the contrary, causes an atrophy of cortical substance. The most essential in regulation of secretion of mineralokortikoid is the ratio in blood of sodium and potassium; the lack of sodium strengthens secretion of Aldosteronum. Believe that influence of deficit of sodium on function of a glomerular zone of cortical substance is mediated through system a renin — angiotensin. Unlike sodium potassium ions influence cortical substance directly, stimulating secretion of mineralokortikoid.
A wide variety biol, effects of hormones H. defines the important place of N. in neuroendocrinal system. Both N.' removal lead to death of an organism in connection with the termination of education Aldosteronum (see) and hydrocortisone (see), having the vital value.
In blood corticosteroids communicate plasma protein — kortikosteroidsvyazyvayushchy globulin (see) and in the form of a proteinaceous and steroid complex reach peripheral fabrics. Getting into cytoplasm of target cells, corticosteroids contact specific proteins receptors. The hormone-receptor-ny a complex provides a translocation of a steroid in a kernel of a cell and access to the genetic device that eventually predetermines implementation of hormonal effect. Fiziol. effects of catecholamines are implemented through alpha and beta adrenoceptors of cells of bodies and fabrics (targets).
Along with other elements of neuroendocrinal system H. take active part in maintenance homeostasis (see). Especially N.'s role at impact on an organism of extreme factors increases. In the conditions of sharply developing stress (see) interaction cortical and marrow N is most brightly shown. So-called gipotalamo - gipofizarno - the adrenal system providing adaptation of an organism to stressorny influences enter into structure and it is specified N.'s participation in reactions of tension by N. Vpervye in U. Kennon (1926) researches, in to-rykh the role of adrenaline at emotional fear reactions was opened, rage, pains. In 1936. Described a Selye adaptation syndrome (see), developing in an organism under the influence of stressorny factors; at the same time increase of secretion is noted by a hypophysis of AKTG and release of glucocorticoids. The catecholamines which are taking part in releasers of an adaptation syndrome influence function of cortical substance H. through stimulation of the corresponding formations of a hypothalamus. The high concentration of glucocorticoids and catecholamines appearing in blood owing to influence of stressorny factors owing to inherent to them biol, actions (stimulation of catabolic processes in nek-ry peripheral fabrics, activation gluconeogenesis and synthetic processes in a liver) provide the organism which is in extreme conditions, power and plastic material. At long action of harmful factors owing to activation kortikoliberi-on and adrenocorticotropic function of a hypophysis the hypertrophy, and then and a hyperplasia of cortical substance H. develops; synthesis of RNA and protein increases, the quantity of cells increases, the steroidogenesis is intensified. All this creates conditions for the maximum secretion of hormones of bark H. in extreme conditions.
Regenerator and compensatory properties of cortical substance H. are so big that, e.g., a wedge, manifestations of acute adrenal insufficiency arise only at destruction apprx. 95% of fabric of gland.
Biochemistry of adrenal glands is defined biochemical, composition of those hormones, to-rye this gland are produced. So, cortical substance produces a large number of steroid connections, to-rye are divided into three groups: C18 steroids — estrogen; C19 steroids — androgens; C21 steroids — actually corticosteroids. All of them are derivatives of a tsiklopentanpergid-rofenantren, hydroxyls are attached to Krom (see. Steroid hormones ). Marrow H. emits the hormones relating to biogenic monoamines — to nitrogen-containing organic compounds (see. Amines ).
Methods of a research
the Most valuable to definition funkts, N.'s conditions are methods of direct definition of hormones in blood. And specific of them are very sensitive radioimmunol. a method, a basis to-rogo reaction makes antigen — an antibody (see. Hormones ), and a method of competitive binding, at Krom with hormones specific proteins, napr react, cytoplasmatic receptors or kortikosteroidsvyazy-vayushchy globulin. Widely use the chemical methods based on extraction of hormones from biol, liquids, their cleaning, processing with special reagents with the subsequent flyuorimetriya (see). The combination of direct methods of definition of hormones to various loadings is applied for the purpose of differential diagnosis. For. examinations funkts, opportunities of cortical substance H. use test with introduction of AKTG and the subsequent registration in blood or urine of level of corticosteroids or eosinophils (see. Thorn test ).
Total or dynamic definition in urine 17 ketosteroids (see) insufficiently informatively as a part them is metabolites of the steroids cosecreted by gonads. At differential diagnosis of a hyperplasia of N. caused by hyperfunction of a hypophysis apply small and big tests with dexamethasone. This test is based on ability of glucocorticoids to suppress secretion of AKTG by the principle of a feed-back (see. Dexamethasonal test ). For differential diagnosis of primary and secondary insufficiency of cortical substance investigate in blood the AKTG level radioimmunol, by method (at primary insufficiency this level is usually increased, at secondary — it is lowered), and also apply test with metopirony.
About a condition of mineralokortikoidny function H. usually judge by the content and a ratio of potassium and sodium in blood: decrease in a ratio of sodium and potassium testifies to insufficiency of this function of cortical substance N. Also methods paper and thin layer are used chromatography (see). The water test has relative value, after a cut investigate a diuresis in vreshn: the delay of water in an organism testifies to mineralokortikoidny insufficiency of H.
In experimental conditions for studying of function of cortical substance widely apply marked corticosteroids (see. Marked connections ). With their help, e.g., determine a so-called half-cycle of life of hormone, speed of secretion of hormone (by the principle of cultivation of a tag) and other indicators of hormonal balance in an organism. Marked hormones use also during the studying of processes of biosynthesis of corticosteroids.
Is of great importance at a research of pathology of N. rentgenol, a research, especially angiography (see). As N. is supplied with blood from several arteries, their vascular system it is possible to contrast in the way aortografiya (see), it is possible to apply also a technique of the selection catheterization of arteries of N. High-speed serial shooting after introduction to an arterial bed of a contrast agent allows to record an arterial phase (the network of arterial vessels of body is visible), a phase of the general increase in intensity of a parenchyma (a so-called parenchymatous phase) and a venous phase (the weak shadow of adrenal veins appears).
The research of venous network H. is more effective at administration of contrast medium by means of a catheter, through a femoral and lower vena cava in adrenal (see. Flebografiya ).
Malformations. The bilateral aplasia of N. is not compatible to life; it is observed seldom, usually in a combination with other heavy malformations.
Hypoplasia of H. can be primary and secondary. Primary hypoplasia (at normal function of gipotalamo-pituitary system) is observed quite often at twins that testifies to a role of a genetic factor; the reason is not found out. The N is small, consist of the compact cells of cortical substance which are irregularly located different size. Clinically proceeds in the form of heavy insufficiency of N. (a so-called inborn disease of Addison). The secondary hypoplasia of N. happens at an anencephalia, an aplasia of a hypophysis, defeat of a hypothalamus or a hypophysis tumoral process, after a hypophysectomy. Marrow H. is created correctly, cortical substance is almost not developed.
Extremely seldom doubling of one is found or both N. Inogd there is an anomaly of formation of separate layers of cortical substance H., partial lack of a glomerular zone with loss of cells of a puchkovy zone under the capsule.
N.'s allotopia is observed often, at the same time their function is normal: all N. (pl a part it) can be located under the renal and hepatic capsule (the so-called encapsulated N.). Sites of fabric H. can be defined in pararenal cellulose, seldom they are implanted into a kidney, a spleen, a pancreas, a liver, into an abdominal wall or generative organs. At the same time they have an appearance of the yellow educations consisting of cells of a puchkovy zone.
The inborn bilateral diffusion or knotty hyperplasia of N. can be the family, hereditary, caused insufficiency of enzyme 11-, 17-or 21 of a hydroxylase, and also 3-beta or 18 dehydrogenases. At this N. are increased to the size of egg, are hilly, have intensive-yellow color. Are microscopically presented by nodular educations from the cells rich with lipids. Clinically — a picture of a pseudohermaphroditism or an adrenogenital syndrome.
Dystrophy of adrenal glands is observed at the general amyloidosis; the mass of amyloid (fig. 5) is postponed in a wall of capillaries of preferential puchkovy, mesh zones and directly in a stroma on border cortical and marrow; cells of an atrofichna. The glomerular zone, as a rule, is not surprised. N are increased, dense, on a section have an opaque grease appearance.
Insufficiency of adrenal glands it is observed rather seldom. At disturbance of intracellular lipid metabolism (a disease to Gosha) N. are increased, soft, bright yellow on a section. Cortical substance is presented by the foamy cells filled with lipids in to-rykh biochemical, kerasin is defined by methods. Among foamy cells the large bulked-up cells of a reticuloendothelium are visible (so-called cells to Gosha). At Nimann's disease — Peak of a cell of cortical substance are overflowed with the cholesterol and phospholipids which are not utilized in process ste-ro ido a gene for. The hemosiderosis of cortical substance is observed at the general hemochromatosis. At the same time N. have brownish color, vessels and cells of cortical substance, especially glomerular zone, are loaded by hemosiderin. N.'s calcification happens in the outcome of necroses or hemorrhages in their bark.
Necroses are small, multiple, are caused by a bacterial embolism or direct effect of toxins at diphtheria, a meningococcal infection, viral diseases (flu, herpes, chicken pox, the Toxoplasmosis), a septicopyemia at newborns; in these cases in a gleam of vessels microbic emboluses are visible.
Disturbances of blood circulation. The reactive hyperemia of N. can arise at increase funkts, activities of N. At this N. are not increased, but their vessels are sharply expanded and filled with blood (fig. 6). At a congestive hyperemia the phenomena of hypostasis of a stroma, sometimes with expansion limf, vessels in cortical substance join.
Hemorrhagic heart attacks of N. are caused by a vein thrombosis, ischemic heart attacks — a stenosis of arteries. At a nodose periarteritis and an arteriolosclerosis the circulatory unefficiency connected with a stenosis of arteries of N. causes a so-called sectoral atrophy of cortical substance with a relacing of N.
Hemorrhages in N. happen diffusion and focal (one - or bilateral). Central hematomas (synonym blood cysts of N.) newborns are formed owing to asphyxia of a fruit or a birth trauma; the hematoma is located around the central vein of N. At a unilateral hematoma in the outcome hemorrhagic cysts are formed, and calcification is observed further or the pigmented hems deforming N. Makroskopicheski the increased N. of dark cherry color is found, to-ry on a section represents as if a bag with blood; microscopically fabric of body is destroyed by the streamed blood. Existence of such hematomas in both N. is not compatible to life. Feature of a structure of N. with the central hematoma at the newborn is absence or poor development of collagenic fibers in a stroma about the central vein and ruptures of walls of sinusoidny collectors in places of their falling into the central vein.
Diffusion bilateral hemorrhages in N. are followed by acute insufficiency of N. and death within a day from the moment of emergence (Waterhouse's syndrome — Frideriksena). At this pathology of N. have an appearance of large roundish formations of dark or blue-red color on a section. Their parenchyma is completely destroyed, impregnated with blood, borders of zones are indiscernible, leukocytic infiltration and vein thromboses is visible. Developing of diffusion hemorrhages is connected more often with meningococcal, and also diphtheritic, pneumo - and a streptococcal infection. Believe that primary and toxic influence leads to a necrosis of cells with the subsequent treatment of a parenchyma blood; the role of an allergic component in development of a necrosis is probable.
Multiple hemorrhages in cortical substance H. have no wedge, symptoms. They can arise at an injury, infections, endogenous (hron, nephrite) and exogenous toksemiya (alcohol, chloroform, adrenaline), at stresses. At this pathology of N. are increased, have red spottiness; microscopically the hyperemia with focuses of disappearance of lipids, hemorrhages in the mesh zone and small necroses capturing groups of cells of cortical substance is found. In the outcome — the pigmented hems and secondary calcification.
Inflammation. The acute inflammation of N. is connected with hematogenous spread of an infection at septic process. In N. the multiple metastatic abscesses which are not giving a wedge, symptoms are found. Granulomas from lymphoid cells are observed in N. of babies at dysentery, a listerellosis, toxoplasmosis, sepsis, a tularemia; at the same time function H. is not broken.
Special forms of an inflammation. At hematogenous spread of tuberculosis to N. miliary hillocks, clinically asymptomatic are defined. In bark H. prosovidny formations of whitish-gray color with a typical gistol, a picture are visible small. Krupnouzlovaty caseous tuberculosis takes almost all thickness of body with death of cells and development of a picture of insufficiency of N. At fibrous a cover - noznom process there can be changes of one or both N. They are reduced, with cicatricial retractions and the dense limy centers. Microcaseous fields with adjournment of calcium in them are limited to cicatricial fabric.
At inborn syphilis in N. the productive intersticial inflammation with the miliary necroses containing the mass of treponemas is observed. Gummas in the form of single or multiple educations are observed seldom, at uncured syphilis at adults. The N is deformed, decreases in sizes; microscopically among hems specific infiltrates are visible.
Primary dystrophy cortical substance H. (synonym: cytotoxic dystrophy of adrenal glands, primary wrinkled adrenal glands, autoimmunoadrenalit) represents an autoimmune disease, a cut can be family and be combined with a Hashimoto's disease, a hyperparathyreosis and a diabetes mellitus (a so-called polien-dokrinopatiya), maybe isolated. In blood find antibodies against a steroid tyrant of tsiruyushchy cells in patients; the antigenic mechanism is not clear. Primary dystrophy is more often observed at children, but maybe at adults. N are sharply reduced, wrinkled; microscopically the reduction of a reticular skeleton of cortical substance comes to light, stromal cells are reduced, kernels of their liknotichna and a polimorfna, cytoplasm is as if dried. Dense lymphocytic infiltrates penetrate the remains of cortical substance which remained in the form of small gnezdny educations, marrow is kept.
Atrophy The N can come in the result of inflammatory and sclerous processes. It also insufficient products of a kortikoliberin and AKTG can be the cause at Simmonds's disease, a hypopituitarism, after a hypophysectomy, and also suppression of secretion of AKTG at long therapy by glucocorticoids. N are reduced, a surface their smooth, cortical substance in the form of a narrow strip. Microscopically the stertost of borders of zones is found, cells are reduced in sizes. Sometimes under the capsule the adenomatous educations having a structure of cortical substance and keeping zonality are defined. These educations are capable to compensate the arising hypocorticoidism (reduced function of cortical substance).
Compensatory and adaptive process, as a rule, is N.'s hypertrophy, to-rye at the same time are increased, have light yellow color, cells large. Dark cells contain RNA, light — a large number of lipids. The hypertrophy can be diffusion iliuzlovaty (adenomatous). The unilateral compensatory hypertrophy is observed at switching off of function of one of N. The bilateral diffusion hypertrophy and N.'s hyperplasia arises at hyperfunction of gipotalamo-pituitary system, at the tumors of a hypophysis producing the raised kolrtestvo of AKTG. The hypertrophy of cortical substance arises also in process a stress reaction of a different origin. N.'s hypertrophy is followed by increase in secretion of hormones of cortical substance — a hypercorticoidism, to-ry can concern both all cortical substance (pan-hypercorticoidism), and each of zones separately. The knotty hypertrophy of cortical substance clinically often does not come to light; it is observed at arterial hypertension of any genesis; adenomatous educations arise as compensation perishing as a result funkts, an overstrain of a parenchyma of N. Death of a parenchyma quite often is followed by the centers of a cytolysis on border of puchkovy and glomerular zones with formation of slit-like spaces, around to-rykh a cell of cortical substance create tubules (so-called psevdotubul).
Postmortem changes. The earliest autolytic changes develop in a mesh zone of cortical substance H., it is possible in connection with high content of hydrolases in it.
Klin, symptomatology patol, changes in N. is connected with disturbance of processes of synthesis and secretion of hormones and caused by change of their concentration in blood. Increase or decrease in concentration of hormones H. leads to disturbance of regulation of exchange processes in many bodies and body tissues.
Not strongly expressed malformations N. (underdevelopment) as well as additional N.' presence, can clinically not be shown if there is no state hypo - or a hypercorticoidism, i.e. falloff or increase in maintenance of corticosteroids in blood of the child.
Damages N practically represent casuistic cases in connection with their very favorable topography. N.'s damage, a cut can arise at severe wounds of lumbar area, can be shown by nothing in connection with big compensatory properties of cortical substance.
Diseases, caused by damage of cortical substance H. of various origin (tuberculosis, hemorrhages, thrombosis of vessels, etc.), are shown by a condition of a hypocorticoidism and the corresponding disturbances of exchange. Bilateral defeat of cortical substance brings to addisonovy disease (see), caused by switching off or reduction of products of hormones.
So-called primary destructive atrophy of N. is described, the etiology a cut remains not clear; it is not excluded that primary atrophy of N. in some cases is caused by autoimmune processes since in blood of patients antibodies to fabrics N are found.
Secondary insufficiency of N. is caused patol, by the process which is localized in gipotalamo-pituitary area and followed by hyposecretion of AKTG; at this form of insufficiency secretion of Aldosteronum changes much less, than secretion of glucocorticoids. As a result of disturbances in system a renin — angiotensin or enzymatic defect in a glomerular zone of cortical substance can develop the isolated insufficiency of mineralokortikoidny function H. (see. Hypoaldosteronism ).
Increase in function of cortical substance, or a hypercorticoidism, can be caused by either primary defeat of gland, or disturbance of gipotalamo-pituitary regulation. Primary defeat of N. usually results from a tumor of cortical substance or N. Simptomatik's hyperplasia giperkor-
a titsizm is defined by that biochemical, and morfol, changes, to-rye arise at long increase in blood of level of these or those hormones produced by N. The phenomena, e.g., are characteristic of an aldo-eteroma hyper aldosteronism (see): hypopotassemia, hypernatremia, hyper-kal iuriya and giponatriuriya. Hypersecretion of glucocorticoids leads to development of a syndrome of Cushing (see. Cushing syndrome ). Hyperfunction of cortical substance with excessive secretion of androgens causes development adrenogenital syndrome (see). The isolated defeat of separate zones of cortical substance meets rather seldom; more often defeat takes different zones H., and the various symptomatology respectively develops.
The secondary hypercorticoidism is connected with hyperproduction of AKTG at giperplaziya or tumors of a hypophysis (see. Itsenko — Cushing a disease ). The symptomatology of primary and secondary hypercorticoidism is very similar.
The hyperfunction of marrow H. which is observed usually at development pheochromocytomas (see), it is characterized by receipt in blood of a large number of catecholamines that leads to hypertensia with sudden increases in the ABP to very high figures, and also to a complex of vegetative and exchange disturbances.
Inflammatory processes develop in N. for the second time, as a result of heavy inf. diseases (sepsis, typhus, etc.); the same diseases can cause hemorrhage in N., and also N.'s heart attack (an embolism or thrombosis of vessels of N.).
Specific hron, inflammatory processes (tuberculosis, syphilis) also strike N. Klien, the picture at hemorrhage in N., acute inflammations, N.'s destruction as a result of tuberculosis, syphilis, as well as at damages, is characterized by acute development of adrenal insufficiency. Its main symptoms are abdominal pains, high temperature of a body, disorders of function went. - kish. path, cyanosis of skin, sharp nervous excitement, development of the phenomena collapse (see), in hard cases — coma (see). The phenomena of adrenal insufficiency arise also at primary destructive atrophy, and also at metastasises of malignant tumors in N. and at an amyloidosis.
Due to the high compensatory properties H. small hemorrhages at nek-ry acute inf. diseases (scarlet fever, measles, smallpox) clinically do not come to light. Adrenal insufficiency can arise sharply at the sudden termination of introduction of high doses of glucocorticoid drugs, especially, if therapy was carried out without a day-night rhythm of activity of N. (see. Corticosteroids, corticosteroid therapy ).
Mental disorders at diseases N are defined by development of a so-called psychoendocrine (psychopatholike) syndrome in the beginning (see. Endocrine mental syndromes ), and in process of deepening of a disease — an organic psychosyndrome (see the Pathopsyhosis) with decrease in intelligence, characteristic of it. Against the background of the specified syndromes there can be various on the structure acute and hron, psychoses. Such shifts arise as at hyper, and hypofunctions.
At the easiest form of insufficiency of cortical substance (addisonizm) and favorable disease it is observed astheno - an adynamic syndrome, the main feature to-rogo is the combination of mental and physical (muscular) weakness to a hyperexcitability and an exhaustion. The increased mental excitability is shown by irritability, irascibility, a hyperesthesia. In process of development of an addisonovy disease the astenoadinamichesky phenomena accrue, reaching such degree when even insignificant tension (including mental) becomes almost impossible. Sharp decrease in mental activity and exhaustion of mental functions can make an impression of decrease in intellectual activity. Frustration of mood are characteristic. More often patients are monotonously depressive, tearful and disturbing. Sometimes apathy and indifference prevails. The increased drowsiness or a combination of drowsiness and sleeplessness can be observed.
At hyperfunction of cortical substance H. psikhopatol, the structure of a psychopatholike syndrome can be changed due to more expressed frustration of mood, to-rye quite often bear in themselves lines of the mixed affect (see. Depressive syndromes ). At an adrenogenital syndrome, Itsenko's disease — Cushing the expressed situational depressions and hypochiondrially-senestopa-ticheskiye states are possible (as reaction to disability and change of appearance).
In process of development of an organic psychosyndrome at diseases of cortical substance decrease in memory, leveling of features of the personality, a primitivization of interests gradually develops, decrease in intelligence accrues (dementia). At an anamnestic and organic syndrome similarity of the mental changes inherent hypo - and hyperfunctions of cortical substance H., becomes still big.
Developing of psychoses is quite often connected with increase of weight of a somatic state. However this dependence is not absolute. Acute psychoses at N.'s diseases have structure exogenous and organic more often (amental, delirious). There can be also epileptiform attacks and twilight conditions of consciousness. Psychoses where leaders are affective disturbances, and also shizofre-nopodobny psychoses are described, to-rye can be chronic in this connection there are difficulties for their otgranicheniye from endogenous psychoses.
Attacks of alarm, melancholy, fear of death are characteristic of mental disturbances at defeat of marrow H. (is more often at a tumor). In nek-ry cases there are changes of consciousness with epileptiform spasms; they are combined with attacks of vascular spasms, paresthesias, extremity pains and in heart, trembling, a fever.
Diagnosis of diseases of N. is based on detection of nature of hormonal disturbances; diagnosis of mental disorders — on studying of course of disease in general, identification of signs of psychoendocrine and organic syndromes.
Lech. actions are also defined by the nature of disturbance of secretion of hormones. At N.'s insufficiency irrespective of genesis of a disease carry out replaceable hormonal therapy (see). If N.'s insufficiency is caused hron, infections (tuberculosis, syphilis), causal treatment a long rate of specific antibacterial drugs is necessary. At sharply arising insufficiency intravenously enter glucocorticoids or their synthetic analogs, injections of drugs from a mineralokortikoidny-ma by properties and drop administration of the shown liquids are at the same time shown.
At patol, the states caused by a hypercorticoidism in connection with a tumor resort to an operative measure — an adrenalectomy with the subsequent replaceable hormonal therapy.
Treatment of mental disturbances at N.'s diseases is defined psikhopatol, structure of a disease, and treatment is carried out in a complex with treatment of a basic disease. At psychoses neuroleptics are shown; in exceptional cases (even at an addisonovy disease) their parenteral administration is possible. Small tranquilizers, antidepressants, psychostimulants and anti-convulsive drugs use at the corresponding frustration in small doses, taking into account individual reaction of the patient.
In the general complex to lay down. actions the psychotherapy shall take the important place, especially at the syndromes of a hypercorticoidism which are followed by change of appearance. The psychotherapy shall be directed to correction depressive, and sometimes and suicide states for creation of confidence of the patient in a curability of a disease.
Tumors are observed rather seldom, more often in N. metastasises of tumors of other bodies are found. Tumors of cortical substance meet more often than tumors of brain.
Complexity of a histogenesis of N. and variety a wedge, the symptoms caused by tumoral diseases complicate creation of uniform classification of new growths N. Naiboley acceptable the classification based on morphological and a wedge, characteristics of tumors is. On the basis of these criteria of a tumor of N. it is possible to divide on primary, coming from the elements forming body, and secondary (or metastatic) tumors.
Primary tumors of N. are subdivided into two groups — hormonal and inactive and gormonalnoaktivny. Treat high-quality hormonal and inactive lipoma (see), fibroma (see. Fibroma, fibromatosis ), myoma (see), fibromyoma (see); to malignant — melanoma (see), teratoma (see). Hormonal and inactive tumors have characteristic no wedge, pictures. Dull aches in a waist where dense education is sometimes palpated are usually noted; the disease is followed by weakness, acceleration of ROE. In far come cases, especially at malignant tumors with metastasises, oznoba, high temperature of a body, a cachexia join.
Treat hormonal and active tumors of cortical substance H. aldosteroma (see), androsteroma (see), kortikosteroma (see), kortikoestroma (see), received the name according to the hormone produced by a tumor, and also enclavomas.
Earlier all hormonal and active tumors, coming from fabric elements H., called a true hypernephroma; further this term lost the value since development of oncology and endocrinology allowed "to differentiate several options of hormonal and active tumors
of N. Kontsentration of hormones in biol, liquids of an organism does not give an idea of high quality or a zlokachestvennost of a new growth. Clinically high-quality and malignant options of hormonal and active tumors are also indiscernible. Metastasises of malignant tumors are located in lumbar (paraortalny) limf in the beginning, nodes, extending further in limf, nodes of a mesentery, a liver, lungs, bones; metastasises produce the hormones characteristic of primary tumor. So, Aldosteronum produces Aldosteronum causing a wedge. syndrome of primary addosteronizm; the androsteroma produces androgens, causing an adrenogenital syndrome. At a kortikosteroma Cushing's syndrome owing to excess quantity in an organism of glucocorticosteroids develops; the kortikoestroma causes feminization in connection with secretion of estrogen in men.
Enclavomas of N meet more often. They cosecrete glucocorticoids, androgens, mineralokortikoida; develop from all zones of bark; zlokachestvenna in 45 — 80% of cases, especially at children. It is necessary to carry to enclavomas also cancer of cortical substance H., to-ry consists of unripe cells and has high hormonal activity. Occurs at children and adults. N.'s cancer sometimes call malignant options of all tumors of N.
Malrelated tumors of cortical substance, high-quality and malignant, more often mixed, meet very seldom, tumors, coming from N. Raspolagayutsya they in a root of a mesentery, a wide ligament of a uterus, an ovary are clinically shown by the same symptoms, as; the case of the aldosteroma localized in the field of gate of a right kidney is described.
Carry a pheochromocytoma to hormonal and active tumors of marrow. Both benign tumor, and malignant pheochromocytoma (tsvetn. fig. 4 and 5) develops from chromaffin cells of marrow and produces catecholamines; in 10% of cases they are located out of N. — in paraortalny gangliya, a bladder, a mediastinum, is extremely rare in a neck, a head cavity and the spinal channel.
Can proceed from elements of nervous tissue of marrow H. ganglioneuroma (see) — a mature benign dofaminprodutsirugoshchy tumor, and also an unripe malignant hormonal and inactive tumor — a sympathoblastoma. In absolute majority of cases these tumors occur at children up to 5 years, is frequent at newborns, early and plentifully metastasize (see. Neuroblastoma ).
As a rule, even huge tumors of N., displacing a kidney, never burgeon in it.
Diagnosis of hormonal and inactive tumors of N. is very difficult because of the absence of characteristic a wedge, signs. The diagnosis and the differential diagnosis of hormonal and active tumors are based on caused? them a wedge, syndromes and determination of the increased content of hormones H. in blood and urine. However in the presence of a hypercorticoidism it is sometimes difficult to differentiate Cushing's syndrome and Itsenko's disease — Cushing that needs to be considered at an operative measure.
In diagnosis of tumors of N. the angiographic research is of great importance. In rentgenol, the image various tumors of N. have a number of common features. The new growth, as a rule, has the form of an oval, an ovoid or a sphere. Existence on roentgenograms of well outlined shadow of a tumor with equal and accurate contours gives the grounds to assume its high-quality nature. Uneven contours and merge of a shadow of a tumor to a kidney, liver or spleen can testify to malignant character. At increase in a tumor in sizes also intensity of its shadow amplifies, edge quite often reaches intensity of a shadow of a kidney.
Irrespective of the place of development of a tumor early rentgenol, a sign is uniform increase and small strengthening of intensity of a shadow of N. extensively without change of its triangular shape; with a further growth of a tumor protrusion of a shadow of the basis or N.'s parties or a curve of corners is found.
Radiodiagnosis by an aldoster, seldom having 3 cm in the diameter is most difficult; at the same time the value of a symptom of increase in intensity of a shadow of a tumor increases. It is easier to diagnose radiological a tumor of marrow (pheochromocytoma) and a tumor of cortical substance of virilny type (androsteroma), it is more difficult — kortiko-stery. At big tumors of N. shift a number of the located bodies can be found.
Major importance in diagnosis of tumors of N. is attached to pneumography; the angiography allows to specify malignant or high-quality character of a new growth. A delay of a contrast agent in N.'s vessels, their shift and deformation and furthermore destruction of vessels shall guard concerning existence of a malignant tumor. Growth of a tumor is followed by death of vessels in the center of a new growth and emergence of new vascular sine on the periphery that leads to formation of an avascular zone in the center of a tumor; these changes are observed in arterial and parenchymatous phases angiografin. On N.'s phlebograms at tumoral process expansion of diameter of veins, their shtoporoobrazny crimpiness is noted. In 65 — 85% of all cases of tumors the atrophy of other N is found.
In all cases of suspicion about existence of a tumor the differential diagnosis with other pathology N. Tak is necessary, at an inborn adrenogenital syndrome, at Itsenko's disease — Cushing bilateral increase in a shadow of N. keeping the form is observed. At an adrenogenital syndrome of N. have homogeneous structure and big intensity of shadows, and at Itsenko's disease — Cushing — heterogeneous, tyazhisty structure (so-called lipoparenkhimatozny N.). At N.'s hyperplasia on angiograms changes both in a parenchyma, and in a vascular bed are found. At an addisonovy disease on survey roentgenograms of an abdominal cavity approximately in 15 — 20% of all cases over upper poles of kidneys the sites of calcification having speckled structure are observed. At very seldom found N.'s echinococcosis the cover of a parasite obyzvestvlyatsya. Calcification in N. at tumors has unsystematic character and, as a rule, occupies the big space. For obtaining the image of N. along with rentgenol, inspection applies a radio isotope research (see. Radio isotope diagnosis ) — so-called radio isotope visualization of N. with use 19-yod-holesterina- 131 I. The method is based by a pas inclusion of marked cholesterol in biosynthesis of hormones of cortical substance H. and is applied to diagnosis of a hyperplasia and tumors of cortical substance N.
All tumors are subject to operational removal. Malignant tumors And. are very steady against radiation therapy. In late stages of malignant tumors of cortical substance H. appoint chemotherapy. Radicalism of an operative measure (in the absence of metastasises) is caused by an oncotomy without damage of its capsule; hit of tumor cells in surrounding cellulose, as a rule, leads to emergence of the remote metastasises, to a recurrent tumor.
In the absence of metastasises and preservation of an integrity of the capsule of a tumor during operation the forecast rather favorable.
The principles of an operative measure on adrenal glands
Indications for operation are N.'s hyperplasia (at Itsenko's disease — Cushing), tumors (cortical and marrow); sometimes an operative measure on N. is undertaken at cancer of an ovary, mammary gland (there are observations that it prolongs life the patient). The absolute indication to operation is the diagnosed N.'s tumor at absence a wedge, and rentgenol, signs of metastasises. Weight of a condition of the patient is not an absolute contraindication to operation since operational treatment of a tumor of a pla of heavy, quickly current disease of Itsenko — Cushing is the only possibility of rescue of the patient. Radical treatment of the arterial hypertension which is a symptom of a basic disease also consists a tumor or gnperfunk-tsioniruyushchy N at a distance.
Preoperative preparation patients with a hyperplasia of cortical substance or tumors (at hypersecretion of glucocorticoids) consists in the greatest possible correction of cardiovascular and exchange disturbances. Therapy of steroid diabetes is reached by a diet and glucose-lowering drugs; as a rule, before operation the most reasonablly fractional administration of simple insulin. The hypopotassemia which is often found at malignant kortikosteroma p kortikoandroste-rums is subject to compensation by drugs of potassium (orally or intravenously) in a combination from a sgshronolaktonama. Correction of a hypopotassemia is feature of preoperative training of patients aldosteromy since allows to lower several the ABP and to increase potassium concentration in serum at least to the lower bound of norm. Not stopped hypopotassemia — the bad predictive sign testimonial of a possibility of heavy and even deadly complications in time and after operation. Injection of 10 or 20% of solutions of albumine in view of loss of protein as a result of the raised gluconeogenesis is useful. The diet shall contain a large number of an animal and phytalbumin. Purpose of polyneuramins and With (some days before operation, intramusculary), the products containing vitamin A is reasonable.
On indications to purpose of corticosteroids in a complex of means of preoperative preparation at any kind of a tumor and even at an adrenal struma there are different opinions. One clinical physicians consider that creation of depot of corticosteroids reduces danger intraoperatsionno of the arising hypocorticoidism. Other clinical physicians consider inexpedient saturation of an organism a hydrocortisone, proceeding from that observation that symptoms of a hypocorticoidism, as a rule, appeared in 5 — 8 hours after operation and in cases of lack of effect of intramuscular introduction of corticosteroids were quickly compensated by intravenous administrations of a hydrocortisone.
The main thing in preoperative preparation at a pheochromocytoma or a neuroblastoma — a partial block of adrenoreaktivny systems the troia-hair dryer or phentolamine. Duration of the physician of exchanges that heat under go tovka depends on efficiency of treatment and portability of drugs. Stopping of hypertensive crises is carried out intravenous administration of 10 — 20 mg of Tropaphenum. Premedication can be carried out traditionally — vnutrimp by oven introduction of Promedolum with atropine, thalamonal with addition of diazepam intramusculary.
From positions of the anesthesiologist operation for a pheochromocytoma should be divided into two periods. The first period — approach to a tumor, its mobilization and removal — is characterized, as a rule, by the high ABP. At this time intravenously fractionally enter Tropaphenum (a single dose of 10 — 20 mg, total — 60 — 80 mg), being guided by the ABP level. At tachycardia of St. 120 blows in 1 min. enter inderal (Obsidanum) fractionally on 1 — 2 mg. The combined use alpha and beta adrenoblockers allows to control a hemodynamics well. Use for this purpose of ganglioblokator, including an arfonad is a little justified.
The second period (right after an oncotomy) is characterized by the expressed decrease in the ABP, sometimes to a state collapse (see).
Prevention of acute hypotonia is carried out from the very beginning of an anesthesia by intravenous administration of 800 — 1000 ml of Polyglucinum. After mobilization of a tumor struyno pour on average 1000 — 1200 ml of Polyglucinum.
Blood loss is compensated by equal amount of blood after a final hemostasis; sometimes there is a need of obligatory administration of angiotonic drugs or glucocorticoids after removal of katekholaminprodu-tsiruyushchy tumors. Total quantity of the colloid and crystalloid solutions entered for the first days after operation by 2 — 3 times exceeds blood loss. As at any stage of operational treatment of such patients there can be a cardiac standstill demanding resuscitation actions, the anesthesiologist shall be ready to carrying out them in full, including a cardiac massage, a defibrillation, use of angiotonic and steroid hormones. Indications for use of steroid hormones are single-step removal of tumors from both N., repeated operation for an oncotomy from the second N. At indications to use of steroid hormones the hydrocortisone on 75 mg of 4 — 6 times a day with a gradual dose decline and drug withdrawal is appointed. Since the second - third day a current of the postoperative period usual. Patients with a pheochromocytoma, as a rule, have an initial deficit of volume of the circulating blood, to-ry in the second period of operation eliminate with transfusion of the corresponding amounts of blood or Polyglucinum.
Venous return to time of operation is controlled according to indications of the central venous pressure; it shall not be lower than 80 — 100 mm w.g.
At operations for tumors of cortical substance the intubation of a trachea for carrying out an anesthesia is carried out after introduction of the depolarizing relaxants (Dithylinum) with extra care, considering the possible osteoporosis of vertebrae developing as a result of disorder of exchange of calcium. Apply neyroleptanalgeziya to maintenance of the general anesthesia) in a combination with diazepam or small and medium concentration of Ftorotanum in the conditions of artificial ventilation of the lungs. At a severe form of primary aldosteronism the anesthesia should be carried out in the conditions of gipokaliyemichesky metabolic alkalosis (see), the only way of correction to-rogo consists in sufficient infusion of solutions of potassium chloride from 5 or 10% solution of glucose.
The anesthesia at patients with a pheochromocytoma can be begun only after imposing io to a last resort of two intravenous transfusion systems. More preferable to these purposes to use intravenous catheters, to-rye enter by a puncture of a subclavial vein or an internal jugular vein. For induction use 1% solution of barbiturates, and also neyroleptanalgetik with diazepam. As the main anesthetic apply etran or Ftorotanum. Widely apply a method of a neyroleptanalgeziya in combination with diazepam against the background of insufflation of 60% of nitrous oxide in respiratory mix with oxygen. Much less often a pheochromocytoma delete in the conditions of peridural anesthesia (1% solution of Trimecainum) against the background of a neyroleptanalgeziya and artificial ventilation of the lungs with mix of oxygen and nitrous oxide. It is more reasonable to apply the depolarizing relaxants to muscle relaxation, to-rye unlike should raise the level of a histamine in blood.
Operations on N. can be made from three main types of access: laparotomny, side extra peritoneal (with a resection or without resection of the XII—XI edges) and combined — torakofrenolyum-botomny or torakofrenolaparo-volume. So, e.g., at Itsenko's disease — Cushing side Extra peritoneal access is most reasonable. Two-stage operation is represented to more rational; it is better to begin operation with a right-hand adrenalectomy as more technically difficult (proximity of the lower vena cava).
The isolated bandaging of the central vein of N. is an indispensable condition of a hemostasis. It is necessary to remember that the considerable number of patients with Itsenko's disease — Cushing and Cushing's syndrome has an expressed osteoporosis able to lead to a change of one and even several lumbar vertebrae; the possibility of such heavy complication dictates need of respect for extra care at turn of the patient sideways, vydviganiya of the roller of the operating table etc.
At impossibility to allocate the tumor burgeoning in a leg of a kidney without damage of the capsule or at wound of vessels of a kidney N.'s removal and kidneys is shown (see. Nephrectomy ) uniform block. Danger of wound of the lower vena cava and difficulty of sewing up of its defect from lyumbo languidly go a section during removal of big tumors are the indication for use of the combined access — torakofrenolyumbotomny or torakofrenolaparotomny.
In tactics of the surgeon at an adrenalectomy there is a feature caused by imperfection of methods of differential diagnosis of a disease of Itsenko — Cushing and Cushing's syndrome. In the absence of accurate data on existence of a tumor (Cushing's syndrome) it is necessary to begin operation from right-hand extra peritoneal) access. If at audit of an abdominal cavity giperplazirovanny N. is found, then the adrenalectomy is the first stage in treatment of a disease of Itsenko — Cushing at this patient. If instead of the increased, juicy N. the atrofichny N. representing sometimes a thin petal is found, then existence of a tumor (Cushing's syndrome) in opposite N. is undoubted. In such cases reasonablly urgent oncotomy.
For treatment of a disease of Itsenko — Cushing only the bilateral total adrenalectomy is reasonable. Preservation of sites of cortical substance, adjacent to a tumor, is possible when the integrity of the capsule of a tumor is not broken.
Great difficulties arise by searches of an aldosteroma: this tumor seldom exceeds in dia. 2 — 3 cm (0,5 — 0,7 cm are more often). Therefore in the absence of the expressed symptoms of a nodular hyperplasia the adrenalectomy without the second N.'s audit is not justified.
Operation for a pheochromocytoma has features in connection with the hypertensive crises able to result in acute cardiovascular insufficiency. At unknown localization of a tumor (bilateral, extraadrenal) tactics of two-stage audit of N. is unacceptable; at the established localization side Extra peritoneal access (with a resection of the XII—XI edges is more preferable at indications). At unknown localization and radiological the excluded extraadrenal tumor (e.g., an intrathoracic tumor), especially at children, the wide laparotomy allowing to examine both N. and the place of a possible arrangement of a malrelated tumor is shown.
All kinds of operation on N. are combined by the term «adrenalectomy»; technology of operation is Slee And a drenalektomiya.
In the postoperative period at patients with Cushing's syndrome and Itsenko's disease — Cushing replacement therapy is represented the major condition recovery. Highlights of posleoporatsionny maintaining are also correction of exchange processes (water and electrolytic and carbohydrate), therapy of cardiovascular and pulmonary complications.
Patients with a glucocorticoid hypercorticoidism since even after a removal of sutures for the 12th day almost in 34% of cases discrepancy of edges of a wound and secondary suppuration is observed need especially careful leaving, it is long also inertly current (sometimes 3 — 4 months).
There is a so-called late postoperative adrenal insufficiency, edges develops in 1 — 3 week after cancellation of replacement therapy; in these cases repeated purpose of corticosteroids after an extract from a hospital is required for a long time (under control of the endocrinologist in policlinic). As a rule, at such patients radiation therapy on diencephalic area for treatment of a disease of Itsenko — Cushing took place. After operational removal of an aldosteroma, an androsteroma, the box-tikoestromy of replacement therapy usually is not required, however in some cases at emergence at least of little signs of a hypocorticoidism purpose of corticosteroids is reasonable.
Disturbance of water and electrolytic exchange at patients about my Aldosteronum and nek-ry other tumors of cortical substance is korrigirut intravenous administration of drugs of potassium and veroshpirony. Correction of carbohydrate metabolism is carried out by use of simple insulin before operation and in the first several days after operation. Replacement corticosteroid therapy after a bilateral total adrenalectomy is carried out for life.
Main objective of maintaining patients after operation for a pheochromocytoma is elimination of hemodynamic disturbances.
Some features of physiology and pathology of adrenal glands at children
At children of early, preschool and younger school age excretion 17 oxycorticosteroids (see), reflecting secretion of a hydrocortisone cortical substance H., it is lowered in comparison with adults. In process of development of the child there is a gradual increase in secretion of all hormones of cortical substance. Before pubertal development reliable distinction in excretion of 17 oxycorticosteroids at boys and girls is not noted; distinctions are found only after final formation of gonads.
It is important to emphasize that at boys in the pubertal period along with the high basal level of glucocorticoids decrease in reserve opportunities of cortical substance is noted; girls have these funkts, reserves are much higher. It defines their various reaction to stressful situations, including pathological processes.
Marrow H. comes from embryonal sympathetic nervous formations of area of a ventral aorta. By the time of growing of marrow into an interrenalo-vy body, i.e. by the beginning of formation of uniform body, there is already a differentiation of cells of marrow. Emergence of the granules containing catecholamines is observed already on 8 — the 9th week of antenatal development. From 13th week in marrow adrenaline and dopamine are found, however the key hormonal product of marrow during all embryonal and post-natal life is noradrenaline. Process of formation of marrow continues till the period of school age. At the age of 7 — 10 years significant increase in amount of marrow and a differentiation of its cellular elements is noted.
Formation of a day-night rhythm of activity of N. happens in the first two weeks of life of the child. To two-week age daily fluctuations of maintenance of corticosteroids in biol, liquids are insignificant; in the subsequent at healthy children they correspond to a day-night rhythm at adults. The rhythm of secretion of catecholamines is established to school age according to formation of fabric of marrow. Activity of secretion of corticosteroids and catecholamines the greatest in the morning that needs to be considered during the performing hormonal therapy.
The stressful reactions which are characterized by the strengthened products of all hormones H. especially clearly are expressed at children 5 — 7 years are more senior.
Dysfunction of cortical substance H., a cut is the cornerstone the lowered products of 17 oxycorticosteroids at the kept or slightly increased synthesis of 17-дезокси-connections (the ratio between them decreases), it is found in children of hl. obr. at the infectious and allergic diseases inclined to a long and wavy current, and also at inf. diseases during development of immunity, at hron, tonsillitis. Dysfunction of cortical substance is found in children at the diseases proceeding with an edematous syndrome (a circulatory unefficiency in an active phase of rheumatism, a nephrotic form of a glomerulonephritis), and also during the performing hormonal therapy without a day-night rhythm of secretion of corticosteroids and their activity in an organism of the child.
The hereditary defects of biosynthesis of corticosteroids connected with insufficiency of separate enzymes cause development of a hereditary adreno-genitaljiy syndrome (see), and also inborn hypoaldosteronism (see). Diagnosis functional and morfol. shifts (a hypercorticoidism, a hypocorticoidism) it is possible only by means of hormonal methods of a research.
Sharply arising adrenal insufficiency at children can be caused by an inborn hypoplasia, hemorrhages are more often hemorrhage in N. Prichina the injury at long childbirth (pelvic presentation, use of nippers) is, a hemolitic disease of the newborn, asphyxia, toxicoses of pregnant women, various heavy inf. diseases. In a wedge, a picture of hemorrhage in N. symptoms of a collapse, the frequent, hardly palpated pulse, spasms, shallow hurried breathing dominate.
At children of early age (is more rare at more seniors) hemorrhage in N. is connected with septic process (a meningococcal infection); in this case set a wedge, manifestations of a basic disease and the accompanying hemorrhage in N. carries the name of a syndrome of Waterhouse — Frideriksena. This syndrome most often begins suddenly. The sick child becomes uneasy, then excitement is replaced by the expressed slackness. Temperature quickly increases to 41,5 °. On skin of a back, extremities, scrotums appear limited sites of cyanosis, mucous membranes of a tsianotichna. Soon there is petekhialny rash, elements a cut merge (so-called star-shaped rash). During the first hours a syndrome there comes the collapse; in some cases there are Meningeal symptoms. Consciousness is oppressed, in an end-stage comes coma (see). In blood the moderate leukocytosis, neutrophylic shift to the left, an eosinophilia, thrombocytopenia, decrease in a sugar content and increase in residual nitrogen is observed.
At early children's age the tranzitorny insufficiency of N. caused by deviations from normal development of these glands can take place. The main a wedge, symptoms are dehydration, vomiting, a collapse. Correction is reached by administration of salt solutions and corticosteroid drugs.
Hron, N.'s insufficiency occurs at children rather seldom and has the same reasons and manifestations, as at adults (see. Addisonova disease ). The hypercorticoidism at children can be caused by a secondary hyperplasia of cortical substance at Itsenko's disease — Cushing (see. Itsenko — Cushing a disease ), the hormonal and active tumors of cortical substance producing corticosteroids (see. Cushing syndrome ).
Pathology of marrow H. occurs at children seldom and is generally caused by tumors (a sympathoblastoma, a pheochromocytoma).
See also Hemadens .
Bibliography: Agarkov G. B. Nervous device of epinephral glands, M., 1964, bibliogr.; Artishevsky A. A. Epinephral glands, Minsk, 1977, bibliogr.; Barstein E. I. and Mar-guliss.d. About mental disturbances at Itsenko's disease — Cushing, Zhurn, a neuropath, and psikhiat., t. 71, No. 2, page 1841, 1971; Biochemistry of hormones and hormonal regulation, under the editorship of N. A. Yudayev, M., 1976; B of an ukhman. I. Radiodiagnosis in endocrinology, M., 1975; Volkova O. V. and Baking M. I. Embriogenez and age histology of internals of the person, M., 1976; P. D. and Protasov T. N Horizons. A role of AKTG and corticosteroids in pathology, M., 1968; Grollman A. Clinical endocrinology and its physiological bases, the lane with English, M., 1969; Dobrzhan-s to and I am A. K. Mental and neurophysiological disturbances at endocrine diseases, M., 1973, bibliogr.; At - to about in with to and y M. A. Hemadens and their diseases at children, M., 1972; Zografskis. Endocrine surgery, the lane with bolg., Sofia, 1977; And c of e of the Tax Code about H. M. K to clinic and a pathogeny of cerebral vegetative syndromes in connection with the doctrine about interstitial and pituitary system, Voronezh, 1946; Clinical oncology, under an edition. TT. N, Blochina and B. E. Peterson, t. 2, M., 1979; Clinical oncology of children's age, under the editorship of M. V. Volkov, page 184, M., 1965; The Clinical onkourologiya, under the editorship of E. B. Marinbakh, page 81, M., 1975; Kravtsov M. P. Adrenal glands of the perinatal period, Minsk, 1978, bibliogr; Krakow V. A. Sindr Itsenko — Cushing, M., 1963, bibliogr.; Masson P. Tumors of the person, the lane with fr., page 296, M., 1965; Nikolaev O. V. and B. I Cockroaches. Hormonal and active tumors of bark of an adrenal gland, M., 1963, bibliogr.; Nikolaev O. V., etc. Pheochromocytoma, M., 1965; Fundamentals of practical anesthesiology, under the editorship of E. A. Damir and G. V. Gulyaeva, page 282, M., 1967; Ratner N. A., Gerasimova E. N and Gerasimenko P. P. Giperaldosteronizm, M., 1968, bibliogr.; The guide to clinical endocrinology, under the editorship of V. G. Baranov, M., 1977; The Guide to endocrinology, under the editorship of B. V. Alyoshin, etc., M., 1973; With and M. R's pin. Vessels of epinephral glands, M., 1974, bibliogr.; Smitten N. A. Sympathoadrenal system in filo-and ontogenesis of vertebrata, M., 1972, bibliogr.; Modern questions of endocrinology, under the editorship of N. A. Yudayev, century 3, M., 1969, century 5, 1975; Modern methods of definition of steroid hormones in biological liquids, under the editorship of N. A. Yudayev, M., 1968; Soffer L., Dorfman R. and Gebrilav of JI. Epinephral glands of the person, the lane with English, M., 1966; Functions of adrenal glands at fruits, newborns and babies, under the editorship of V. A. Tabolin, page 263, M., 1975; Hamidov D. H.idr. Epinephral gland, Tashkent, 1966; Yudayev N. A. Biochemistry of steroid hormones of bark of adrenal glands, M., 1956; Bachman n R. Die Nebenniere, Handb, d. mikro-scop. Anat. desMenschen, hrsg. v. W.Mollen-dorff, Bd 6, T. 5, S. 1, B-, 1954; D i s z f a-1 u s y E. Endocrine functions of the human fetoplacental unit, Fed. Proc., v. 23, p. 791 1964; Functions of the adrenal cortex, ed. by K. W. McKerns, N. Y., 1967; G 1 a z E.a.Yecsei P. Aldosterone, Oxford — N.Y., 1971; Hartman F. A. a. Brownell K. A. The adrenal gland, Philadelphia, 1949; Horton R. Aldosterone, Metabolism, V. 22, p. 1525, 1973, bibliogr.; Lab hartA. Klinik der inneren Sekretion, B. u. a., 1971; Lehrbuch der allgemeinen Pathologie und der pathologischen Anato-mie, hrsg. v. M. Eder u. P. Gedigk, S. 419, B. u. a., 1977; Metabolic endocrine and genetic disorders of children, ed. by Y. C. Kelley, y. 1, p. 263, Hagerstown, 1974; Montgomery D. A. a, Welbourn R. B. Medical and surgical endocrinology, L., 1975; S m i t h C. To. and. lake of Psychiatric disturbances in endocrinologic disease, Psycho-som. Med., v. 34, p. 69, 1972, bibliogr.; Textbook of endocrinology, ed. by R. H. Williams, p. 255, Philadelphia a. o., 1974. V. P. Fedotov; H. K. Bogdanovich (stalemate. An.), A. I. Buchmann (rents.), K. N. Kazeev (PMC., hir.), D. D. Orlovskaya (psikhiat.), G. A. Ryabov (anest.), M. R. Sapin (An.),
V. A. Tabolin, V. P. Lebedev (ped.)