Pathological anatomy of the endocrine system

There are three most common forms of pathological changes in the endocrine portion of the hypothalamus: (1) impaired production of liberins ( releasing factors ) and statins , (2) pathology of antidiuretic hormone ( ADH ) and (3) oxytocin deficiency in women.

1. Violations of the production of liberins and statins are diverse. They lead to a violation of various functions of the adenohypophysis, and therefore a number of peripheral endocrine glands.

2. The pathology of the antidiuretic hormone is manifested by two types of disorders: (1) its insufficiency ( diabetes insipidus ) and hyperproduction ( Parkhon's syndrome , or hydropexic syndrome ).

Diabetes insipidus - ADH deficiency. The kidneys lose their ability to concentrate urine, which leads to polyuria and polydipsia. Complications of diabetes insipidus are associated with the loss of water by the body and impaired mineral metabolism.

Parkhon's syndrome ( hydropexic syndrome ) - hyperproduction of ADH. The main manifestation of the disease is fluid retention in the body (edema).

3. Oxytocin deficiency in women is accompanied by weak labor.

Diverse pathological changes in the adenohypophysis are divided into three groups: hypo- , hyper- and dispituitarism (the terms come from the old name of the pituitary gland - the pituitary gland ). Hypopituitarism - pituitary insufficiency. Hyperpituitarism is a pituitary hyperfunction that develops as a result of hyperplasia of its glandular cells or tumor (more often than adenomas, less often cancer). Dispituitarism is the failure of some and the overproduction of other hormones of the adenohypophysis. Hypopituitarism can be total ( panhypopituitarism ) or partial (partial). In the case of total hypopituitarism , a deficiency of all hormones of the adenohypophysis develops, partial hypopituitarism is characterized by a deficiency of any one hormone.

I. Hypopituitarism

1. Panhypopituitarism
2. Partial hypopituitarism .

II. Hyperpituitarism.

III. Dispositarism.

Forms of Panhypopituitarism

Panhypopituitarism manifests Symmonds syndrome and its variant Shihan syndrome.

Simmonds Syndrome

Symmonds syndrome ( hypothalamic-pituitary cachexia ) is caused by necrosis of the pituitary gland or atrophy of its parenchymal elements, as a result of which the production of pituitary hormones decreases or completely stops. The atrophic processes in various organs and tissues and premature aging, most characteristic of Simmonds syndrome, are largely associated with insufficiency of somatotropic homon (STH). In typical cases, patients develop exhaustion ( cachexia ).

Postpartum hypopituitarism

Sheehan's Syndrome ( Sheean's syndrome , postpartum hypopituitarism ) is a variant of Simmonds syndrome that develops after childbirth. Pituitary tissue necrosis occurs due to ischemia due to bleeding during childbirth.

Forms of Partial Hypopituitarism

Partial hypopituitarism is characteristic of pituitary microsomy and adiposogenital dystrophy.

Pituitary microsomy

Pituitary microsomy ( pituitary dwarfism ) is characterized by a lack of effects of STH. All organs in this disease are underdeveloped ( congenital hypoplasia ), patients are stunted. The term "nanism" comes from the Greek. nanos is a dwarf.

Adiposogenital Dystrophy

Adiposogenital dystrophy ( Fröhlich syndrome ). The disease is based on pituitary gonadotropin deficiency. Their deficiency leads to insufficient synthesis and secretion of sex hormones ( secondary hypogonadism ). The disease manifests itself at prepubertal or pubertal age and is characterized by two main syndromes due to hypogonadism: hypogenitalism (underdevelopment of the genitals and secondary sexual characteristics) and obesity . Lawrence-Moon-Bidel disease is a congenital variant of adiposogenital dystrophy.

Forms of Hyperpituitarism

Hyperpituitarism is the basis of the syndromes of hyperproduction of GH, Cushing's disease, Chiari-Frommel syndrome, juvenile basophilism and hypermuscular lipodystrophy.

GH hyperproduction syndromes

Syndromes of hyperproduction of GH. Excessive formation of GH is caused by hyperplasia or pituitary adenoma. Occasionally, increased synthesis of STH occurs in tumors of other localizations ( "ectopic STG syndrome" ). Moreover, all organs undergo hypertrophy , patients of high growth (usually above 200 cm in men and 190 cm in women).

The syndrome can develop in childhood ( gigantism ) or in an adult ( acromegaly ). Gigantism is characterized by proportional hypertrophy of parts of the skeleton and internal organs, with acromegaly, organs are enlarged to varying degrees (disproportionate hypertrophy). Change in appearance with acromegaly is associated with coarsening of facial features, an increase in the superciliary arches, cheekbones, and lower jaw. Gigantism when a patient grows up is transformed into acromegaly. Hypertension syndrome of STH in 20% of patients is manifested by the development of secondary diabetes mellitus , since STH is a contra-hormonal hormone. With somatotropocyte hyperplasia, the disease usually proceeds benignly, patients live for several decades even without treatment, and in the presence of a pituitary tumor, the life expectancy of patients without adequate therapy is 3-4 years.

Acromegaly, which occurred against the background of a pituitary tumor, ends with cachexia . Gigantism and acromegaly in 5-10% of cases are formed without hyperproduction of STH: with excessive sensitivity of peripheral tissues to normal concentration of STH or with increased synthesis of non-hormonal growth factors ( somatomedins ).

Cushing's Disease

Cushing's disease ( Itsenko-Cushing's disease ) is the result of increased synthesis of ACTH, resulting in the development of hyperfunction of the bundle zone of the adrenal cortex ( secondary hypercorticism ). Hyperproduction of the hormone is associated with adenoma or hyperplasia of corticotropocytes.

The disease is manifested by the following symptoms:

1. Secondary immunodeficiency (glucocorticoids have an immunosuppressive effect)
2. Secondary diabetes mellitus with high glucocorticoids
3. Secondary arterial hypertension , also caused by high glucocorticoids
4. Steroid osteoporosis - a decrease in the degree of mineralization of bone tissue, as a result of which pathological fractures often occur
5. Cushing - type obesity is the deposition of fat primarily on the face (“moon-shaped face”) and trunk, while limbs remain thin.

The death of patients occurs most often from infectious complications (pneumonia, sepsis) or diabetic nephropathy.

Chiari-Frommel Syndrome

Chiarí-Fromelma persistent lactorrhea syndrome - hyperproduction of prolactin. Both women and men are ill. A characteristic sign of the syndrome is the release of milk from the mammary glands, which in women is not associated with pregnancy and feeding ( lactorrhea ). In women, in addition, the production of gonadotropins and, consequently, sex hormones is reduced, as a result of which atrophy of the endometrium and ovaries develops; amenorrhea forms, therefore, in women, the disease is called persistent lactorrhea-amenorrhea syndrome .

Youthful Basophilism

Juvenile basophilism ( hypothalamic syndrome of puberty ) - hyperproduction of ACTH, STH and gonadotropic hormones due to increased production of hypothalamic releasing factors. In the adenohypophysis, the number of basophilic cells increases (hence the term "basophilism"). The disease is formed in adolescents during puberty and is manifested by obesity, as well as accelerated physical and sexual development ( biological acceleration ).

Hypermuscular Lipodystrophy

Hypermuscular lipodystrophy is a hyperproduction of lipotropic hormones (primarily β-lipotropin ). The syndrome is manifested by atrophy of adipose tissue. Due to chronic hyperinsulinemia, patients develop skeletal muscle hypertrophy and moderate visceromegaly.

Dispositarism

Manifestations of dispituitarism are diverse, but they are much less common hypo- and hyperpituitary syndromes. One of the most common forms of dispititarism is Neurath syndrome - a combination of gigantism (increased production of STH) and adiposogenital dystrophy (pituitary gonadotropin deficiency).

There are two main forms of pathological changes in the pineal gland: hyperfunction ( Marburg-Milku syndrome ) and epiphyseal melatonin deficiency ( Pellizzi syndrome ).

Marburg Syndrome — Milku

Marburg-Milk syndrome - overproduction of epiphyseal melatonin due to hyperplasia of pineocytes or a tumor ( pinealoma ). In addition to the pineal gland, melatonin is produced in other organs, for example, by intestinal apudocytes. Epiphyseal melatonin is an antagonist of a number of tropic pituitary hormones (lipotropins, thyroid-stimulating hormone and gonadotropins), therefore, with its excess, (1) obesity ( epiphyseal obesity ), (2) hypothyroidism and (3) hypogenitalism develop.

Pellizzi Syndrome

Pellizzi syndrome - hypofunction of the pineal gland . The absence of inhibitory effect of melatonin on the formation of the reproductive system manifests itself in its premature maturation (by the end of the first decade of life, and in some cases earlier). Mostly boys are affected ( androtropism ).

Dysfunctions of the parathyroid glands are manifested in the form of increased production of parathyroid hormone ( hyperparathyroidism ) or its insufficiency ( hypoparathyroidism ).

Hyperparathyroidism

Hyperparathyroidism ( Engel-Recklinghausen disease ) is a hyperproduction of parathyroid hormone followed by the development of hypercalcemia . Hyperproduction of the hormone is caused by hyperplasia or a tumor of the parathyroid glands and is accompanied by the following main changes:

• Hyperparathyroid visceropathy (primarily nephropathy ) as a result of metastatic calcification . In addition, kidney stones form.
• Hyperparathyroid osteopathy , manifested by a combination of osteoporosis and proliferation of malignant bone tissue. The bones are thickened, pathological fractures are characteristic.

Hypoparathyroidism

Hypoparathyroidism is a parathyroid hormone deficiency leading to hypocalcemia , which causes the development of a convulsive syndrome .

Thyroid diseases are widespread. The main ones are struma (goiter), thyroiditis and tumors.

Depending on the level of thyroid hormone production, three forms of the functional state of the thyroid gland are distinguished:

1. Euthyroidism - with a normal level of thyroid hormones (triiodothyronine and thyroxine)
2. Hyperthyroidism is the result of hyperproduction of thyroid hormones (the extreme severity of hyperthyroidism is called thyrotoxicosis )
3. Hypothyroidism is a consequence of a lack of triiodothyronine and thyroxine in the body (an extreme degree of severity of hypothyroidism occurs with myxedema ).

Struma (goiter)

Struma (goiter) - macroscopically expressed hyperplasia of thyroid tissue, not associated with inflammation.

The evolution of the concept of "goiter"

The term “goiter” until the mid- 19th century was used to refer to all three-dimensional processes in the front and side of the neck. R. Virkhov was the first to apply this term only in relation to an enlargement of the thyroid gland. By the beginning of the 20th century , tumors were excluded from the group of goiter, and by the middle of the century - thyroiditis, therefore the terms “Ridel’s goiter” and “Hashimoto's goiter” are outdated.

Classification of goiter forms

Goiter forms are classified as follows:

I. Clinical and morphological principle

1. Endemic goiter
2. Sporadic goiter
3. Dyshormonogenetic goiter
4. Diffuse toxic goiter
5. Ectopic goiter .

II. Macromorphological principle

1. Diffuse goiter - a uniform increase in the entire organ without the formation of nodular structures (develops with Graves disease ).
2. Nodular goiter - focal or total enlargement of the gland due to the formation of multiple, closely located nodules. The concept of “nodular goiter” is an inaccurate translation of the term adopted in the international classifications of thyroid gland diseases “ nodular goiter ” ( nodular goiter , that is, characterized by the formation of multiple nodules). Endemic, sporadic and dishormonogenetic goiter appears in the form of nodular.

III. Morphogenetic principle

1. Parenchymal goiter is formed by small follicles, in which colloid is not detected by ordinary optical microscopy (initial phase of the process).
2. Colloidal goiter ( macro- , normo- , microfollicular ) is formed by follicles of various sizes with the presence of a colloid, which is determined by the usual micromorphological study of the material.

IV. The functional state of the gland

1. Euthyroid goiter is a goiter with normal production of thyroid hormones.
2. Hypothyroid goiter - goiter with deficiency of thyroid hormones.
3. Goiter with signs of cretinism is a hypothyroid goiter that develops in childhood or in utero and leads to physical and mental underdevelopment ( cretinism ).
4. Hyperthyroid goiter is a form of goiter, accompanied by increased production of thyroid hormones (mainly diffuse toxic goiter ).

Nodular forms of goiter

1. Endemic goiter - goiter among residents of iodine-deficient areas. The resulting deficiency of thyroid hormones leads to the activation of thyroid pituitary thyroid cells. The increased formation of thyroid-stimulating hormone leads to hyperplasia of thyroid tissue.

2. Sporadic goiter is an acquired iodine-independent form of goiter that is found everywhere. The basis of the disease is the lack of enzymes for the synthesis of thyroid hormones, as well as the primary hyperproduction of the thyroid-stimulating hormone of the pituitary gland. Foods rich in calcium and fluorine compounds interfere with the absorption of iodine, which contributes to the development of sporadic goiter. A certain role in the genesis of this form of goiter is played by alimentary strumogens (for example, thiocyanates contained in some fresh vegetables).

3. Dyshormonogenetic goiter is an iodine-independent form of goiter associated with hereditary insufficiency of thyroid hormone synthesis enzymes.

4. Ectopic goiter - hyperplasia of an atypically located thyroid tissue, that is, outside the thyroid gland. The most common types of ectopic goiter include (1) tongue root goiter , (2) retrosternal goiter (can be located in any part of the anterior mediastinum) and (3) ovarian struma (ovarian teratoma with a predominance of mature thyroid tissue).

Diffuse Toxic Goiter

Diffuse toxic goiter ( Graves disease ) is a hyperthyroid goiter of an autoimmune nature. The disease is based on an allergic reaction of type V (the appearance of stimulating autoantibodies to the follicular cells of the thyroid gland).

Diffuse toxic goiter is manifested by the following syndromes:

1. Thyrotoxic myopathy , including thyrotoxic cardiopathy ( thyrotoxic myocarditis ), - changes in muscle tissue. In severe cases, with symptoms of heart failure on the background of diffuse cardiosclerosis or due to weakness of the respiratory muscles, death can occur.
2. Thyrotoxic hepatitis can lead to liver cirrhosis.
3. Secondary arterial hypertension (moderate).
4. An enlargement of the thyroid gland (up to a pronounced, called thyromegaly ).
5. The characteristic appearance of patients: general atrophy (weight loss) due to the predominance of biopolymer catabolism over synthetic processes (thyroid hormones - hormones with a catabolic effect), which leads to atrophy of adipose tissue and skeletal muscles.
6. Thyrogenic ( autoimmune ) ophthalmopathy (a combination of exophthalmos and ophthalmoplegia). Exophthalmos develops due to the growth of fibrous tissue in retrobulbar cellulose ( retrobulbar fibrosis ). Ophthalmoplegia - the immobility of the eyeballs as a result of compression of the oculomotor muscles and their atrophy.

Thyroiditis

Thyroiditis is an inflammatory lesion of the thyroid gland. Acute , subacute and chronic forms of thyroiditis are distinguished. A peculiar clinical and morphological form is traumatic thyroiditis. Among the chronic variants of thyroiditis, Riedel and Hashimoto's diseases are of primary importance.

Traumatic thyroiditis

Traumatic thyroiditis ( palpable thyroiditis ) develops as a result of trauma. Macromorphologically in the gland at the site of the injury, compaction is determined without clear boundaries. A micromorphological study reveals a lot of foam cells (macrophages phagocytic colloid) in the lumen of damaged follicles, and lymphohistiocytic infiltrate between the follicles.

Acute thyroiditis

Acute thyroiditis occurs in the form of serous and purulent variants. Purulent thyroiditis is characterized by the formation of abscesses (abscesses) or phlegmon of the thyroid gland. It develops as a result of the ingestion of pyogenic flora into the thyroid tissue by hematogenous or lymphogenous pathways, as well as by the spread of a purulent process from parathyroid tissues with neck phlegmons and ascending purulent mediastinitis. Acute serous thyroiditis occurs as a complication of certain viral infections, such as mumps.

Thyroiditis de Curvena

The cause of subacute granulomatous thyroiditis de Kerwin are considered viruses, especially paramyxoviruses (measles and mumps viruses). Subacute thyroiditis lasts from several weeks to 2 years (an average of 5-6 months) and usually ends in recovery. In some cases, the disease lasts for years, periodically recurring. Macromorphological examination shows an enlargement of the thyroid gland, which develops rather quickly, which resembles the growth of an extremely malignant tumor. Micromorphologically revealed foci of follicular necrosis, the presence of a free colloid in the tissue around which giant cell granulomas form.

Riedel's thyroiditis

Chronic fibrous thyroiditis of Ridel - the growth of coarse-fibrous tissue in the thyroid gland in the form of a dense knot without clear boundaries. Macromorphologically and clinically, such a node resembles a malignant tumor.

Hashimoto Thyroiditis

Chronic autoimmune thyroiditis ( chronic lymphocytic thyroiditis , Hashimoto's disease ) is a chronic autoimmune lesion of the thyroid gland (it is necessary to differentiate with subacute postpartum autoimmune thyroiditis ). The disease is mainly found in women. During Hashimoto's disease, two main stages are distinguished: (1) hyperplastic ( hypertrophic ) and (2) atrophic . The hypertrophic (initial) stage is characterized by an enlargement of the gland due to lymphoid hyperplasia (the formation of facultative lymphoid structures ) and nodular hyperplasia of thyroid tissue in areas of autoimmune destruction of the organ parenchyma. In this case, the gland is slightly enlarged, tuberous from the surface and lobed in the section. The atrophic (final) stage is accompanied by severe autoimmune destruction of thyroid tissue. At this stage, chronic lymphocytic thyroiditis is manifested by signs of hypothyroidism up to myxedema .

Thyroid Tumors

The most common thyroid gland is follicular adenoma (a benign tumor) and papillary carcinoma , which is usually a low-grade tumor.

The most famous forms of pathology of islets of Langerhans include insulin-dependent diabetes mellitus, hyperinsulinism, Mallinson's syndrome, inhibitory syndrome and Werner-Morrison syndrome.

Hyperinsulinism

Hyperinsulinism is the overproduction of insulin due to hyperplasia of β-cells or a tumor of them ( insulinoma ). The main manifestation of the disease is hypoglycemic encephalopathy - dystrophic changes in brain tissue. Hypoglycemia primarily affects the vital activity of the brain (20% of all blood glucose is consumed by the brain), as a result of which the sympathoadrenal system is activated again and the production of catecholamines increases. Catecholamines dilate the vessels of the brain, but prolonged hypercatecholaminemia causes paralytic microvascular dilatation, blood stasis, thrombosis and edema of perivascular tissue, which significantly worsens trophism, causing necrosis , hemorrhages in the brain tissue and its atrophic changes.

Mullinson's Syndrome

Mullinson's syndrome - overproduction of pancreatic glucagon due to hyperplasia of α-cells or a tumor from them ( pancreoglucagonoma ). The main manifestation of the disease is secondary diabetes mellitus (glucagon - a contra-hormonal hormone).

In addition to pancreatic glucagon, there may be an increase in the synthesis of enteroglucagon - duodenal glucagon (the tumor is called enteroglucagon in this case). It is manifested by other signs: the intensive growth of the small intestine mucosa (enteroglucagon is a growth factor for this tissue) and the development of small bowel obstruction.

Inhibitor Syndrome

Inhibitory syndrome is caused by overproduction of pancreatic somatostatin in case of δ-cell hyperplasia or tumor of them ( somatostatinoma ). Somatostatin is a universal inhibitor, it is an insulin antagonist, inhibits gastric secretion and secretion of bile.

The disease is manifested by the following symptoms:

1. Secondary diabetes
2. Chronic Hypersecretory Gastritis
3. Gallstone disease.

Werner-Morrison Syndrome

Werner-Morrison syndrome occurs during the overproduction of a vasoactive intestinal peptide ( VIP ) by tumors from δ1 cells of Langerhans islets ( vipomas ). The disease is manifested by the so-called pancreatic cholera (watery diarrhea). VIP causes the expansion of the capillaries of the intestinal mucosa and its edema. Edematous fluid is absorbed by integumentary epithelial cells and secreted by them into the intestinal lumen. An increase in intestinal contents leads to diarrhea.

Diabetes mellitus is a pathological process, which is based on the insufficiency of the effects of insulin. Diabetes mellitus is not always a disease of the endocrine pancreatic tissue.

According to nosological independence, two forms of diabetes are distinguished:

1. Primary diabetes mellitus (diabetes as an independent disease)
2. Secondary diabetes mellitus (diabetes mellitus as a complication or manifestation of other diseases or conditions).

Primary Diabetes

Primary diabetes mellitus includes type I and type II diabetes, as well as gravidar diabetes mellitus.

1. Insulin-dependent diabetes mellitus ( type I diabetes mellitus ) is a primary diabetes mellitus characterized by absolute insulin deficiency (a decrease in hormone production). The main causes of type I diabetes are viral and autoimmune insulitis (inflammation of the islets of Langerhans). Among viruses, paramyxoviruses (measles and mumps viruses), picornaviruses (Coxsackie viruses), measles rubella and hepatitis B viruses have the greatest diabetic potential. Type I diabetes develops mainly in childhood ( juvenile diabetes ) and in young adults and is more severe than type II diabetes.

2. Insulin-independent diabetes mellitus ( type II diabetes mellitus ) - primary diabetes mellitus characterized by relative insulin deficiency (decreased sensitivity of insulin receptors, insulin production can even be slightly increased). Type II diabetes mainly develops in older obese people ( "obese diabetes" ).

3. Pregnant diabetes ( gravidar diabetes ) - diabetes beginning and ending during pregnancy or after childbirth. If diabetes begins during pregnancy but does not end after childbirth or abortion, it is classified as type I or type II diabetes.

Latent ( latent ) diabetes mellitus was previously isolated. This process is currently referred to as impaired glucose tolerance . It can be considered as a prediabetic state, but it does not always translate into diabetes.

Secondary Diabetes

Forms of secondary diabetes mellitus:

1. Syndromes of hyperproduction of contra-hormonal hormones (Cushing's disease and syndrome, pheochromocytoma, Mallinson's syndrome, inhibitory syndrome, STH hyperproduction syndromes).
2. Medicinal diabetes (for example, with prolonged use of glucocorticoid hormones in large doses).
3. Pancreatic diabetes - diabetes mellitus in chronic pancreatitis, accompanied by atrophy of pancreatic tissue, including islets of Langerhans. Pancreatic is also called diabetes, which developed against the background of surgical removal of the pancreas.
4. With a number of hereditary diseases (for example, with ataxia-telangiectasia Louis-Bar).

Pathological Anatomy of Type I Diabetes

Insulin-dependent diabetes mellitus is more severe than its other forms and is characterized by the development of immunodeficiency, angiopathy, damage to the kidneys, liver and other organs.

1. Secondary immunodeficiency , against the background of which various infectious complications form up to sepsis. Immunodeficiency develops due to severe disorders of carbohydrate metabolism, which plays an important role in the normal function of immunocompetent cells. Infectious complications of diabetes are one of the main causes of death of patients (sepsis, pneumonia, pyelonephritis, etc.).

2. Diabetic angiopathy (macro- and microangiopathy). Diabetic macroangiopathy ( atherosclerosis ) leads to the development of gangrene of the extremities, myocardial infarction, brain and other complications of atherosclerosis. Diabetic microangiopathy is characterized by hyalinosis of small arteries and arterioles, which causes tissue metabolism disorders and atrophic changes in the parenchyma. At the same time, angiogialin is characterized by a high content of lipids ( lipogialin ).

3. Diabetic nephropathy (a specific form of diabetic nephropathy is called Kimmelstil-Wilson syndrome ). Renal damage affects both the glomeruli ( diabetic glomerulopathy up to glomerulosclerosis ) and the tubules ( glycogen tubule infiltration - the formation of numerous glycogen granules in the tubule epithelial cells).

4. Liver steatosis ( fatty hepatosis ) - fatty parenchymal dystrophy of the liver , is a natural visceropathy in diabetes.

The pathology of the glomerular zone of the adrenal cortex is manifested by an increase ( hyperaldosteronism ) or a decrease ( hypoaldosteronism ) in the production of mineralocorticoids . Hyperaldosteronism is divided into primary ( Conn syndrome ) and secondary .

Classification:

I. Hyperaldosteronism - the overproduction of mineralocorticoids.

1. Conn's primary hyperaldosteronism is an independent disease caused by hyperplasia or a hormone-active tumor of the glomerular zone ( aldosteroma ).
2. Secondary hyperaldosteronism is a manifestation or complication of other diseases and pathological conditions, especially nephrogenic arterial hypertension .

II. Hypoaldosteronism ( salt-losing syndrome ) is a deficiency of mineralocorticoids.

Hyperaldosteronism is manifested by the following main symptoms:

1. Hypokalemic myopathy - skeletal muscle and myocardial dysfunction due to potassium deficiency (muscle weakness, skeletal muscle paralysis and paresis, myocardial contractile insufficiency, metabolic myocardial necrosis ).
2. Secondary arterial hypertension .

Hypoaldosteronism is accompanied by exicosis ( dehydration ) due to loss of sodium and water in the urine.

There are two forms of pathological changes in the bundle zone of the adrenal cortex: increased ( hypercorticism ) and weakened ( hypocorticism ) production of glucocorticoid hormones. Hyper- and hypocorticism are divided into two options - primary and secondary .

Classification:

I. Hypercorticism - hyperproduction of glucocorticoid hormones (endogenous glucocorticoids).

1. Primary hypercorticism ( Cushing's syndrome ) - is caused by hyperplasia or a tumor ( corticosteroma ) of the bundle zone, as well as with prolonged use of glucocorticoid hormone preparations in high doses ( iatrogenic primary hypercorticism ); primary hypercorticism - hypercorticism without hyperproduction of ACTH.
2. Secondary hypercorticism ( Cushing's disease ) - hypercorticism against the background of hyperproduction of ACTH.

II. Hypocorticism is an insufficiency of endogenous glucocorticoids.

1. Primary hypocorticism is caused by hypoplasia, necrosis or atrophy of the bundle zone (its acute form, which develops most often with hemorrhage in both adrenal glands, is called Waterhouse-Friedericksen syndrome ; severe chronic manifestations are called Addison’s disease ).
2. Secondary hypocorticism is hypocorticism that develops against the background of ACTH deficiency, in particular, with the withdrawal syndrome of long-used drugs of glucocorticoid hormones ( iatrogenic secondary hypocorticism ).

Cushing's syndrome manifests itself similarly to Cushing's disease .

Addison's disease ( bronze disease ) is a severe form of chronic adrenal cortex insufficiency. Its main manifestations include the following syndromes:

1. Secondary immunodeficiency - develops as a result of insufficiency of glucocorticoids necessary for maturation of lymphocytes ( dishormonal hyperplasia of lymphoid tissue ), and manifests itself in various infectious processes up to sepsis.
2. Secondary arterial hypotension .
3. Myopathy is an atrophy of skeletal muscles and myocardium (the most characteristic clinical manifestation of the disease is fatigue).
4. Generalized hypermelanosis - hypermelanosis of the skin and mucous membranes as a result of overproduction of pituitary melanotropins.

The most common forms of pathology of the net zone of the adrenal cortex are tumors of androsteroma and corticoestroma , as well as a hereditary disease, adrenogenital syndrome . In general, these diseases are rare.

1. Androsteroma - a tumor of the reticular zone, secreting androgen hormones. In women, androsteroma is manifested by signs of virilization ( masculinization ): the mass of skeletal muscles increases, male-type hair growth is noted, voice coarsening occurs.

2. Corticoestroma - a tumor of the reticular zone, producing estrogen hormones. In men, it is accompanied by the development of signs of feminization : gynecomastia, the redistribution of fat according to the female type, the cessation of facial hair growth, and an increase in the tone of voice.

3. Adrenogenital syndrome - hereditary deficiency of glucocorticoid synthesis enzymes. In this case, an excessive amount of adrenal ( epirenal ) androgens is produced. In boys, this syndrome manifests itself in premature puberty (the adrenal form of premature puberty), in girls - signs of virilization with the development of the so-called female pseudohermaphroditism (due to hypertrophy of the clitoris simulating the presence of the penis).

Catecholamine-producing tissue is located in the form of islands next to the vegetative ganglia. These islets are called paraganglia . With the exception of the adrenal medulla, the paraganglia are spherical in shape, so they are often called glomuses . The largest paraganglia are (1) the adrenal medulla, (2) the carotid glomus, and (3) the lumbar aortic paraganglia ( Zuckerkandl organ ).

Sympathetic and parasympathetic paraganglia are distinguished. Paraganglia are also divided into chromaffin (stained with salts of chromic acid) and nonchromaffin (not stained with these reagents). Chromaffin paraganglia include the adrenal medulla.

Classification of forms of pathology of the paraganglia:

I. Hyperplasia of the paraganglia.

II. Paragangliomas - tumors of the paraganglia

1. Pheochromocytoma ( chromaffin , or sympathetic , paraganglioma )
2. Chemodectoma ( nonchromaffin , or parasympathetic , paraganglioma ).

Hyperplasia of carotid glomuses. Chronic hypoxia in diseases of the lungs, heart, anemia, as well as in residents of high mountains is accompanied by hyperplasia of the carotid ( glomerulus ) carotid .

Pheochromocytoma - a tumor of chromaffin paraganglia (80% of the pheochromocytoma is localized in the adrenal medulla), accompanied by hyperproduction of adrenaline. The main manifestations of the disease are (1) arterial hypertension and (2) secondary diabetes mellitus (adrenaline - a contra-hormonal hormone).

Apudopathy is manifested by hypo- or hyperfunction of apudocytes. Hyperfunction of apudocytes is associated with their hyperplasia or tumors ( apudomas ). Apudomas can be benign and malignant. The most common pathological changes in EC-, G- and S-cells.

Pathology of EC cells (serotonin)

The main form of the pathology of EC cells is carcinoid syndrome .

Carcinoid syndrome is a syndrome of hyperproduction of serotonin by tumors from EC cells ( carcinoids ), less commonly by other types of tumors.

The most characteristic manifestations of carcinoid syndrome:

1. Carcinoid cardiopathy is a pathological process pathognomonic for carcinoid syndrome. In this case, valve defects are formed. Valves of the right heart are usually affected in the form of tricuspid valve insufficiency and stenosis of the mouth of the pulmonary trunk . The leaflets of the valves and tendon chords are thickened, milky white, deformed (the leaflets of the tricuspid valve are shortened, the leaflets of the valve of the mouth of the pulmonary trunk are fused together). During micromorphological studies in the valve cusps, proliferation of fibrous connective tissue is noted.

2. Bronchospasm (sometimes bronchial asthma develops). Especially dangerous are asthma states that develop during the operation to remove a carcinoid tumor and often end in death.

3. Diarrhea (the most common symptom of carcinoid syndrome) develops as a result of increased peristalsis under the influence of serotonin.

4. Secondary arterial hypertension .

5. Tides - attacks of redness of the skin of the face, shoulder girdle, upper limbs, accompanied by a feeling of heat.

G-Cell Pathology (Gastrin)

1. Zollinger – Ellison syndrome — gastrin overproduction syndrome in case of G-cell hyperplasia or a tumor ( gastrinoma ) of them. The disease is manifested by chronic hyperacid gastritis with subsequent formation of gastric ulcers and duodenum .

2. Gastrin deficiency syndrome is formed with hypoplasia of G-cells. It manifests itself in the development of chronic hypo- or anacid gastritis .

S-cell pathology (secretin)

1. The syndrome of overproduction of secretin is accompanied by increased formation of pancreatic juice. In this case, pancreatitis develops. In severe forms of the disease, pancreatic necrosis ( destructive pancreatitis ) may develop due to self-digestion of pancreatic tissue.

2. The secretion deficiency syndrome is characterized by a decrease in the formation of pancreatic juice, a digestive disorder ( maldigestia ) developing on this background, and weight loss.

Multiple endocrine neoplasia ( MEN ) - two or more endocrine tumors in one patient.

1. Vermeer syndrome - tumors of the adenohypophysis, parathyroid glands and islets of the pancreas (other tumors may also form).

2. Sipple's syndrome - a combination of medullary (C-cell) thyroid cancer, parathyroid gland tumor and pheochromocytoma.

3. Gorlin's syndrome - a combination of medullary thyroid cancer, pheochromocytoma and neurogenic tumors of the mucous membranes of the digestive tract and eyeball.

• Pathological anatomy
• Tumor growth
• Etiology of malignant tumors
• Childhood tumors
• Pathological anatomy of hemoblastosis
• Pathological anatomy of the female reproductive system
• Alternative processes
• Destructive processes
• Parenchymal dystrophies
• Mesenchymal dystrophies
• Mixed dystrophy
• Circulatory disorders
• Inflammatory response
• Immunopathological processes
• Adaptation and Compensation Processes

• Ivanovskaya T. E., Tsinserling A. V. Pathological anatomy (diseases of childhood) .- M., 1976.
• Kaliteevsky P. F. Macroscopic differential diagnosis of pathological processes. - M., 1987.
• General human pathology: A guide for doctors / Ed. A.I. Strukova, V.V. Serova, D.S. Sarkisova: In 2 vols .-- T. 2.— M., 1990.
• Pathological anatomy of diseases of the fetus and child / Ed. T.E. Ivanovskaya, B.S. Gusman: In 2 vols .-- M., 1981.
• Pathological diagnosis of human tumors / Ed. N. A. Kraevsky, A. V. Smolyannikov, D. S. Sarkisov: In 2 volumes - M., 1993.
• Pathology: A Guide / Ed. M.A. Paltseva, V.S. Paukova, E.G. Ulumbekova.- M., 2002.
• Strukov A.I., Serov V.V. Pathological anatomy.- M., 1995.