Types of dystrophies according to biochemical principles. Parenchymal dystrophies

The classification is based on 4 principles: 1) morphological 2)biochemical 3)genetic 4)quantitative

According to the morphological principle, three types of dystrophies are distinguished depending on what is primarily affected - parenchyma (cells) or mesenchyme (intercellular structures - stroma, vessels).

1) Parenchymatous - cells are primarily affected 2) Mesenchymal - intercellular structures are primarily affected 3) Mixed - simultaneous damage to both parenchyma and mesenchyme.

According to the biochemical principle, dystrophies with disturbances of protein, fat, carbohydrate, mineral, pigment and nucleoprotein metabolism are distinguished. According to genetic principles, dystrophies are classified as acquired and hereditary. According to the quantitative principle, local and widespread dystrophies are distinguished. The basic principle is morphological. Other classifications also work within the framework of morphological classification.

Parenchymal dystrophies

According to the biochemical principle, they are divided into: 1) protein(dysproteinoses) 2) fatty(lipidoses) 3) carbohydrate

Dysproteinoses

The basis of these dystrophies is a violation of protein metabolism. There are 4 types of protein dystrophies 1) Grainy 2) Hydropic 3) Hyaline droplet 4) Horny

1) Granular dystrophy Synonyms: dull, cloudy swelling. The term granular reflects the histological picture of the pathology. With this type of dystrophy, the cytoplasm becomes granular instead of homogeneous. The essence of the pathology is that under the influence of a damaging factor, an increase in mitochondria occurs, which gives the cytoplasm a granular appearance. There are two stages in the development of dystrophy: a) compensation b) decompensation

At the compensation stage, mitochondria are enlarged but not damaged. At the stage of decompensation, mitochondria are enlarged and somewhat damaged. However, mitochondrial damage is mild. When the damaging factor ceases, they completely restore their structure.

Microscopically cytoplasmic granularity is observed in the cytoplasm of cells of different organs - hepatocytes, renal tubular epithelium, myocardiocytes. The state of mitochondria is revealed only by electron microscopic studies. Macroscopic type of organs:

Bud somewhat enlarged in size, dull and cloudy on section. Liver flabby, the edges of the liver are rounded. Heart flabby, myocardium is dull, cloudy, the color of boiled meat.

Causes of granular dystrophy: a) impaired blood supply to organs b) infections c) intoxication d) physical, chemical factors e) impaired nervous trophism

Meaning and outcome- the process is reversible, but with continued action of the damaging factor, granular dystrophy turns into a more severe type of dystrophy. The clinical significance is determined by the scale of dystrophy and localization. With total damage to the myocardium, heart failure may occur.

2) Hydrotic dystrophy or dropsy.

Characterized by the appearance of liquid vacuoles in the cytoplasm. Localization- skin epithelium, hepatocytes, renal tubular epithelium, myocardiocytes, nerve cells, adrenal cortex cells and cells of other organs.

Macroscopy- the picture is non-specific.

Microscopy- vacuoles filled with tissue fluid are detected.

Electron microscopy- indicates that tissue fluid accumulates primarily in mitochondria, the structure of which is completely destroyed and leaves behind bubbles filled with tissue fluid. In cases of severe hydropic dystrophy, one large vacuole filled with cytoplasmic fluid remains in place of the cell. In this variant of dystrophy, all organelles of the cell’s cytoplasm are destroyed, and the nucleus is pushed to the periphery. This variant of hydropic dystrophy is called - balloon dystrophy.

Outcome of hydropic dystrophy, especially balloon, is unfavorable. The cell may subsequently die. And the function of the damaged organ is significantly reduced. Causes of hydropic dystrophy– infections, intoxication, hypoproteinemia during fasting, and other etiological factors of damage.

Topic: “Metabolic disorders. Dystrophy".

Meaning of the theme: Metabolic disorders in the body and its tissues underlie all pathological processes and manifest themselves in the form of symptoms and syndromes of human diseases. Knowledge of structural changes during alteration will help future nurses better understand clinical pathology, understand the significance of their future profession, and show sustained interest in it.

Based theoretical knowledge and practical skills the student must

know: general patterns development of dystrophy, necrosis, atrophy; structural and functional patterns of development and course of dystrophy, necrosis, atrophy

be able to: determine the signs of typical pathological processes: dystrophy, necrosis, atrophy.

Learning Objectives:

common goal

The student must master general competencies

OK 1. Understand the essence and social significance of your future profession, show sustained interest in it.

OK 2. Organize your own activities, determine methods and means of performing professional tasks, evaluate their effectiveness and quality.

OK 4. Search, analyze and evaluate information necessary for setting and solving professional problems, professional and personal development.

OK 5. Use information and communication technologies to improve professional activities.

The student must master professional competencies

PC 2.1. Present information in a form understandable to the patient, explain to him the essence of the interventions.

Control of the initial level of knowledge

1. Concept of damage

2. Main types of damage

3 Mechanisms of development of dystrophies

4 Reasons for the development of dystrophies

5 Classification of dystrophies

6 Types of parenchymal dystrophies

7 Types of mesenchymal dystrophies

8 Types of mixed dystrophies

9 Necrosis, causes, types.

10Atrophy, causes, types

Brief summary of the topic

Damage, or alteration, is called a change in cells, intercellular substance, and depending on the volume of damaged cells - tissues and organs. In damaged cells, tissues and organs, metabolism changes, which leads to disruption of their vital functions and usually to dysfunction. Damage accompanies any disease or pathological process.

Among the damage highest value have dystrophy, necrosis and atrophy.

Dystrophy– a pathological process reflecting metabolic disorders in the body. Dystrophy is characterized by damage to cells and intercellular substance, as a result of which the function of the organ changes.

Mechanisms of development of dystrophies:

1. Infiltration, in which substances characteristic of it enter the cell with the blood, but in more than normal. For example, infiltration of cholesterol and its derivatives into the intima of large arteries in atherosclerosis.

2. Perverted synthesis, in which abnormal, i.e., are formed in cells or intercellular substance. substances not characteristic of these cells and tissues. For example, under certain conditions, cells synthesize amyloid protein, which is not normally present in humans.

3. Transformation, in which, for certain reasons, instead of products of one type of metabolism, substances characteristic of another type of metabolism are formed, for example, proteins are transformed into fats or carbohydrates .

4. Decomposition, or phanerosis. With this mechanism, dystrophy develops as a result of the breakdown of complex chemical compounds that make up cellular or intercellular structures. For example, the disintegration of membranes of intracellular structures consisting of fat-protein complexes during hypoxia leads to the appearance in the cell of an excess amount of either proteins or fats. There is a protein or fatty degeneration.

The classification of dystrophies is based on several principles.

Depending on the disturbed type of metabolism, dystrophies are divided into protein, fat, carbohydrate, and mineral.

Depending on the localization of dystrophy in the parenchyma or stroma, which is of mesenchymal origin, they are parenchymal, mesenchymal and mixed.

Based on their prevalence, dystrophies are divided into general and local.

Depending on the causes, acquired and hereditary types of dystrophies are distinguished.

Parenchymal Lystrophies

Parenchymal dystrophies occur in cells. Depending on the type of metabolic disorder, parenchymal dystrophies are divided into protein (dysproteinoses), fatty (lipidoses) and carbohydrate.

Protein dystrophies: granular, hyaline-droplet, hydropic

Granular dystopia. Occurs in the cells of the heart, liver and kidneys.

Macroscopically, the organs look dull, swollen, and when cut, they resemble boiled meat. Therefore, this type of dystrophy is also called cloudy swelling.

Microscopically: free protein forms multiple small nuclei in the cytoplasm. At the same time, the cells swell, their cytoplasm looks granular. With granular dystrophy, organ function changes slightly. Dystrophy is reversible.

Hyaline-drip dystrophy. a more severe type of protein dystrophy, develops in the kidneys, less often in the liver and extremely rarely in the myocardium. Microscopically: coagulation of the protein occurs, it becomes denser, compressed into homogeneous drops, reminiscent of the ground substance of hyaline cartilage. This type of protein dystrophy is irreversible. Organ function in hyaline droplet dystrophy is significantly impaired.

Hydropic. This dystrophy is associated with a violation of protein-water metabolism and occurs in the epithelium of the skin and intestines, cells of the liver, kidneys, heart, and adrenal cortex. For various reasons, the permeability of cell membranes increases, and vacuoles are formed in the cytoplasm due to the entry of water. Under these conditions, lysosomal enzymes, hydrolases, are activated, which destroy the cells’ own organelles, their protein disintegrates, and fluid enters the cell. The cell dies. Appearance organs with hydropic dystrophy are little changed. Organ function is significantly reduced.

Fatty degenerations. A disturbance in the metabolism of cytoplasmic fat consists either in the accumulation of fat of an unusual composition for these cells, or in the formation of lipids in those cells that normally do not have them. Fatty degenerations are also called lipidoses. Develops in the heart, liver and kidneys.

The main cause of this type of dystrophy is hypoxia. For all diseases accompanied oxygen starvation, fatty degeneration develops in the heart, liver and kidneys. Such diseases include ischemic heart disease, hypertension, atherosclerosis, congenital and acquired heart defects, chronic lung diseases and many others leading to pulmonary heart failure, various infections, especially chronic ones, as well as intoxication. If the cause that caused fatty degeneration is eliminated relatively quickly at the stage of moderate morphological changes in the cells, it is possible to restore their structure and function. Otherwise, fatty degeneration leads to the death of a significant number of cells, and therefore sclerotic changes in organs and disruption of their function subsequently develop.

The mechanisms of development of fatty and protein dystrophies are identical. At the same time, different organs have specific features of the formation of intracellular fat.

Carbohydrate dystrophies. Disorders of carbohydrate metabolism are associated either with the accumulation of protein-polysaccharide complexes (glycogen, glycoproteins) in tissues and cells, or with the formation of these substances in those cells where they are not normally present, or with changes in their chemical composition. Disturbance of glycogen metabolism is of greatest importance, as it is associated with the development of diabetes mellitus, a severe and common disease.

Mesenchymal dystrophies. Mesenchymal dystrophies occur due to metabolic disorders in the interstitial connective tissue, which makes up the stroma of organs and is part of the walls of blood vessels..

Depending on the type of metabolic disorder, mesenchymal dystrophies are divided into protein (dysproteinoses), fat (lipidoses) and carbohydrate.

Protein dystrophies: mucoid swelling, fibrinoid, hyalinosis, amyloidosis.

Mucoid swelling. The cause of mucoid swelling, as well as fibrinoid swelling, can be infectious and allergic diseases, including rheumatic diseases; atherosclerosis, hypertension.

The essence of mucoid swelling is a change in the basic substance of connective tissue. Glycosaminoglycans are redistributed into the main substance, which attract water and it swells and changes its physicochemical properties. With mucoid swelling, the connective tissue becomes unfibered, but the structure of the collagen fibers themselves does not change, so this type of dystrophy is reversible if the cause that caused it is eliminated.

Fibrinoid. This is the next stage after mucoid swelling. Due to increased vascular permeability, plasma containing proteins, including fibrinogen, enters the interstitial tissue, the ground substance of the connective tissue swells (fibrinoid swelling) and then collapses. Fibrinoid ends either with sclerosis, i.e., replacement of dead interstitial tissue with coarse fibrous connective tissue, or with hyalinosis.

Hyalinosis. Hyalinosis, completing fibrinoid changes in the mesenchyme, may be their outcome, but may represent an independent type of mesenchymal protein dystrophy. This type of dystrophy is irreversible. Hyalin is a protein formed from decomposed proteins of interstitial connective tissue and blood plasma proteins, in particular fibrin, released from blood vessels due to increased permeability.

Amyloidosis. Mesenchymal dysproteinosis, which is characterized by the formation on the basement membranes of the mucous membranes, blood vessels, as well as in the interstitial connective tissue of a special, very durable substance consisting of 96% protein, and 4% carbohydrates and “hematogenous additives” - various chemical substances protein nature from blood plasma and tissues. The resulting substance is called “amyloid” and is not normally found in humans. Amyloid accumulates unhindered in tissues, compresses and destroys their structures. Organs affected by amyloidosis increase in size, become dense, brittle, and have greasy look. Amyloidosis is irreversible.

Mixed dystrophies, when changes occur both in the cell and in the parenchyma of organs and tissues, they occur when the metabolism of complex proteins (chromoproteins, nucleoproteins, lipoproteins) and minerals is disrupted.

Chromoproteins (endogenous pigments) are of three types: hemoglobin derivatives (hemoglobinogenic), proteinogenic, lipidogenic.

Hemoglobinogenic pigments formed as a result of physiological aging and breakdown of red blood cells: ferritin, hemosiderin, bilirubin.

Bilirubin is captured by liver cells, combines with glucuronic acid in them and is released into bile ducts; in the intestine it is partially absorbed, part of it is excreted in the feces in the form of stercobilin, and part in the urine in the form of urobilin. A violation of bilirubin metabolism manifests itself in the form of its accumulation in the blood - jaundice.

There are three types of jaundice:

Suprahepatic (hemolytic) – the cause of the breakdown of red blood cells;

Hepatic (parenchymal) – the cause is damage to liver cells and impaired uptake of btltrubin;

Subhepatic (obstructive) – the cause is obstruction of the outflow of bile.

Proteinogenic pigments. Basic melanin determines the color of human skin, hair, and eyes. Its increased content in the skin is observed in the endocrine disease Addison's disease, in birthmarks(nevi).

Lipodogenic pigments. The main lipofuscin is intensively deposited in the cells of the liver and heart in old age, during exhaustion, with defects, etc.

Necrosis

This is the death of individual cells, tissue sections, part of an organ or an entire organ in a living organism. At the same time, metabolism in dead cells and tissues completely and irreversibly stops, and they lose all their functions.

Causes of necrosis mechanical (trauma), temperature (burns, frostbite) factors; ionizing radiation, chemicals, disturbance of nervous and vascular trophism of tissues, influence of toxins in infectious diseases (diphtheria, tuberculosis.)

Signs of necrosis. Irreversible changes in the nuclei and cytoplasm of cells. In the process of necrobiosis and necrosis, cells lose water, the nuclei shrink and become denser - karyopyknosis develops. Nucleic acids in the form of separate clumps leave the nucleus into the cytoplasm, and its disintegration occurs - karyorrhexis. Then the nuclear substance dissolves and karyolysis occurs. The same is observed in the cytoplasm - plasmorhexis and plasmolysis develop in it. Then the entire cell dissolves—cytolysis occurs.

Forms of necrosis : dry(coagulation) or wet(colliquation).

Gangrene– special shape necrosis - distinguished by the black or brown color of necrotic tissue. Gangrene can be dry or wet.

Bedsores(a type of gangrene) - areas of necrosis of the skin, subcutaneous tissue or mucous membranes, subject to pressure in conditions of exhaustion of the body or impaired neurovascular trophism.

Sequestration- an area of ​​dead tissue freely located among living tissue. Typically, a necrotic bone fragment in osteomyelitis serves as a sequester. The presence of sequestration supports purulent inflammation.

Heart attack– tissue necrosis resulting from an acute circulatory disorder due to thrombosis, embolism, or compression of the vessel.

Atrophy- This is a decrease in the volume of an organ and a decrease in its functions that occurs during normal life person or as a result of diseases.

Signs of atrophy. Organs are reduced in volume; inclusions in the form of lipofuscin granules may appear in atrophying cells, giving the organ a brown color. In such cases, they speak of brown atrophy of the organ.

Physiological atrophy accompanies normal human life, pathological atrophy is associated with diseases. Pathological atrophy can affect the entire body (general atrophy) or its individual parts (local atrophy).

General atrophy, or exhaustion, can develop during starvation (nutritional dystrophy), with malignant tumors (cancer cachexia). Cachexia is an extreme degree of exhaustion of the body with atrophy of many organs.

Local atrophy(atrophy of individual organs or parts of the body:

Atrophy from inactivity - it occurs in an organ as a result of a decrease in its function, for example, when a bone of a limb is fractured, atrophy of its muscles occurs.

Pressure atrophy develops when an organ is subjected to pressure by a tumor, cicatricial adhesion, or vascular aneurysm. .

Atrophy from insufficient blood supply occurs in organs to which little blood flows.

Neurogenic atrophy is associated with impaired innervation, mainly of skeletal muscles.

In cases where atrophy of organs and tissues is moderate, when the cause that caused the atrophy is eliminated, the structure and function of the organ is often restored.

Independent work:

Using the reference outline, fill in the graphological structures for dystrophy (Appendix No. 1)

Using an electronic atlas and microcircuits, study micropreparations:

Granular dystrophy, hyaline-drip dystrophy, hydropic, fatty degeneration of the heart, liver, brown induration of the lungs, “mucoid swelling” (Appendix No. 1).

Study macropreparations: “tiger heart”, sebaceous liver, “sago spleen”, “brown lung induration” Appendix No. 1, atlas).

Using the notes, fill in the graphological structures “necrosis” (Appendix No. 1)

Using a computer, study macroscopic specimens of necrosis of the extremities.

Using notes and computer presentations, fill in the graphological structures of “Atrophy.” (Appendix No. 1)

Final knowledge control- tasks in test form

Summarizing.

Homework Subject: General reactions body for damage. Compensatory - adaptive reactions.

Compile a terminological dictionary

Presentations on the topic: “Shock”, “Stress”, “Coma”

Paukov V.S. Litvitsky P.F.page 85-96

Required literature

1. Paukov V. S., Litvitsky P. F. Pathological anatomy and pathological physiology. – Geotar - Media, 2010.

additional literature

1. I.V.Alabin, V.P.Mitrofanenko, “Fundamentals of Pathology”, textbook + CD, GEOTAR-Media, 2011. - 272 p.

2. Paltsev (N) "Atlas of pathological anatomy" Medicine 2007

Details

Dystrophy– a complex pathological process, which is based on a violation of tissue metabolism, leading to structural changes.

Trophic– a set of mechanisms that determine metabolism and structural organization cells (tissues) necessary to perform a specialized function.

Causes of dystrophies:

1) disorders of cell autoregulation, which can be caused by hyperfunction, toxic substances, radiation, enzyme deficiency, etc.

2) dysfunction transport systems, ensuring metabolism and structural preservation of tissues, cause hypoxia.

3) disruption of endocrine, nervous regulation

Morphogenesis of dystrophies:

1) infiltration

Excessive accumulation of a substance (normal, not abnormal) as a result of excess synthesis.

Example: fatty liver hepatosis, kidney hemosiderosis.

2) decomposition (phanerosis)

Disintegration of cell ultrastructures and intercellular substance, leading to disruption of tissue metabolism and accumulation of products of impaired metabolism in the tissue.

3) perverted synthesis

Synthesis of anomalous products. These include: synthesis of abnormal amyloid protein in the cell, synthesis of alcoholic hyaline protein by hepatocytes.

4) transformation

The formation of products of one type of exchange from common initial products that go into the construction of BZHU.

Classifications of dystrophy.

The classification adheres to several principles. Dystrophies are distinguished:

1) by dominance morphological changes V tissue structures ah: parenchymal, mixed, mesenchymal (stromal-vascular)

2) by dominance violations of one or another type of exchange: protein, fat, carbohydrate, mineral.

3) depending on influence genetic factors : acquired, hereditary.

4) by localization: local, general.

Parenchymal dystrophies.

Manifestations of metabolic disorders in functionally highly specialized cells.

1) Parenchymal protein dystrophies (dysproteinoses)

The essence of such dystrophies is a change in the physico-chemical and morphological properties and cell proteins: they undergo denaturation and coagulation or colliquation, which leads to hydration of the cytoplasm. In cases where the bonds between proteins and lipids are disrupted, destruction of cell membrane structures occurs.

Disorders of protein metabolism are often combined with disorders of the Na-K pump: which leads to the accumulation of Na ions and cell swelling. This pathological process is called hydropic dystrophy.

Kinds:

-grainy

Reversible, looks like the accumulation of small protein grains in the cytoplasm. Organs increase in size, become flabby and dull.

- hyaline-drip

Large hyaline-like protein droplets appear in the cytoplasm, merging with each other and filling the cell body. In some cases it ends with focal coagulative necrosis of the cell.

Often found in the kidneys, rarely in the liver and myocardium.

In the kidneys, when examined, the accumulation of droplets is found in nephrocytes. Accumulation is often observed in nephrotic syndrome, since the basis of this dystrophy is the insufficiency of the vacuolar-lysosomal apparatus of the proximal tubule epithelium, in which proteins are normally reabsorbed. This is why protein (proteinuria) and casts (cylindruria) appear in the urine.

The appearance does not have any characteristic features.

In the liver, microscopy reveals Malory bodies, consisting of fibrils and alcoholic hyaline. The appearance of such droplets is a manifestation of the perverted synthetic function of the hepatocyte, which occurs when alcoholic hepatitis, primary biliary cirrhosis. The appearance of the liver varies.

The outcome of hyaline droplet dystrophy is unfavorable; it leads to cell necrosis.

-hydropic dystrophy

Characterized by the appearance in the cell of vacuoles filled with cytoplasmic fluid. It is observed more often in the epithelium of the skin and renal tubules, in hepatocytes and myocytes.

Parenchymal cells are increased in volume, their cytoplasm is filled with vacuoles containing clear liquid. Then the cell turns into a huge balloon (the entire cell has become a large vacuole) - focal liquefaction necrosis. The appearance of the tissues changes little.

A major role in the development mechanism is played by impaired membrane permeability, which leads to acidification of the cytoplasm and activation of hydrolytic enzymes of lysosomes, which break intramolecular bonds with the addition of water.

Causes: in the kidneys – damage to the renal filter, which leads to hyperfiltration, in the liver – hepatitis of various etiologies, in the epidermis - swelling, infection.

The outcome of such dystrophy is usually unfavorable - it ends in focal coagulative necrosis.

-horny dystrophy

It is characterized by excessive formation of horny substance in the keratinizing epithelium (hyperkeratosis, ichthyosis) or the formation of horny substance where it normally does not exist (pathological keratinization on the mucous membranes). The reasons are varied: skin development disorders, chronic inflammation, vitamin deficiencies, etc.

Outcome: sometimes, when the cause is eliminated, tissue restoration occurs, but in advanced cases, cell death occurs.

- hereditary disorders of amino acid metabolism

So-called storage diseases, which are based on a violation of the intracellular metabolism of a number of amino acids as a result of hereditary deficiency of metabolizing enzymes.

A) cystinosis. Science does not yet know which enzyme deficiency leads to this disease. AA accumulates in the liver, kidneys, spleen, eyes, bone marrow, skin.

B) tyrosinosis. Occurs due to tyrosine aminotransferase deficiency. Accumulates in the liver, kidneys, bones.

B) phenylpyruvic oligophrenia. Occurs due to deficiency of phenylalanine-4-hydroxylase and accumulates in nervous system, muscles and blood.

2) Parenchymal fatty degenerations (lipidoses)

Disturbances in the metabolism of cytoplasmic lipids can manifest themselves in an increase in their content in cells where they are found normally, in the appearance of lipids where they are not usually found, and in the formation of fats of unusual chemical composition.

-lipid metabolism disorders

In the liver, fatty degeneration is manifested by a sharp increase in the fat content in hepatocytes and a change in their composition. In liver cells, lipid granules first appear (pulverized obesity), then small droplets (small-droplet obesity), which then merge into large droplets (large-droplet obesity) or into one fat vacuole. The liver is enlarged, flabby and ocher-yellow in color. Among the mechanisms of fatty liver degeneration, there is an excessive entry into hepatocytes fatty acids or their increased synthesis by these cells, the impact toxic substances, blocking the oxidation of fatty acids and the synthesis of lipoproteins in hepatocytes, insufficient supply of amino acids necessary for synthesis to the liver cells. So, HDP occurs as a result of: lipoproteinemia (alcoholism, diabetes mellitus, general obesity), hepatotropic intoxications (ethanol, chloroform), and nutritional disorders.

Fatty degeneration of the myocardium occurs due to hypoxia and intoxication. The development mechanism is associated with a decrease in fatty acid oxidation due to the destruction of mitochondria under the influence of hypoxia or toxin. On macroscopic examination, the size of the heart is increased, the cardiac muscle is clay-yellow in color. The myocardium looks like the skin of a tiger - white and yellow striations. Lipids are determined in the form of small drops.

The causes of fatty degeneration are varied. They can be associated with oxygen starvation (which is why it is often found in diseases of the cardiovascular system), infections and intoxications, vitamin deficiencies and one-sided nutrition.

The outcome of fatty degeneration depends on its degree. If it is not accompanied by gross breakdown of cellular structures, then it is reversible.

- hereditary enzymopathies

They arise due to hereditary deficiency of enzymes involved in lipid metabolism.

A) disease Gaucher with glucocerebrosidase deficiency. The lipid accumulates in the liver, spleen, and bone marrow.

B) disease Niemann -Pike with sphingomyelinase deficiency. Accumulation in the liver, spleen, bone marrow.

IN) disease Sachs with acid galactosidase deficiency.

G) disease Norman -Landinga with beta-galactosidase deficiency.

3) Parenchymal carbohydrate dystrophies

-carbohydrate dystrophies associated with impaired glycogen metabolism

At diabetes mellitus there is insufficient use of glucose by tissues, an increase in its content in the blood and excretion in the urine. Tissue glycogen reserves decrease sharply. In the liver, glycogen synthesis is disrupted, which leads to its infiltration with fats and fatty liver degeneration.

The following changes occur in the kidneys with diabetes mellitus: glycogen infiltration of the tubular epithelium.

- hereditary glycogenosis

a) type 1 – Gierke’s disease – glucose-6-phosphatase deficiency

b) type 2 – Pompe disease – acid alpha-1,4-glucosidase deficiency

c) type 3 – Forbes disease – deficiency of amylo-1,6-glucosidase

d) type 4 – Anderson’s disease – deficiency of amylo-(1,4-1,6)-transglucosidase

e) type 5 – McArdle disease – myophosphorylase deficiency

e) type 6 – Hers disease – liver phosphorylase deficiency

In diseases of types 1,2,5,6, the structure of glycogen is not disturbed.

-carbohydrate dystrophies associated with disorders of glycoprotein metabolism

In the cells or intercellular substance, the accumulation of mucins and mucoids, also called mucous or mucus-like substances, occurs.

Many secreting cells die and desquamate, excretory ducts glands become obstructed by mucus, which leads to the development of cysts.

The causes are varied, but most often – inflammation of the mucous membranes as a result of the action of various pathogenic irritants.

Sometimes in clinical practice There is such a thing as parenchymal dystrophy. Pathological anatomy classifies them as metabolic disorders in cells. If we talk in simple language, then the process of nutrition and accumulation in the organ is disrupted useful substances, which leads to morphological (visual) changes. This pathology can be identified during a section or after a series of highly specific tests. Parenchymal and stromal vascular dystrophies underlie many lethal diseases.

Definition

Parenchymal dystrophies are pathological processes that lead to changes in the structure of organ cells. Among the mechanisms of disease development are cell self-regulation disorders with energy deficiency, fermentopathy, discirculatory disorders (blood, lymph, interstitium, intercellular fluid), endocrine and cerebral dystrophies.

There are several mechanisms of dystrophy:

Infiltration, that is, excessive transport of metabolic products from the blood into the cell or intercellular space, caused by a malfunction in the body’s enzyme systems;

Decomposition, or phanerosis, is the breakdown of intracellular structures, which leads to metabolic disorders and the accumulation of under-oxidized metabolic products;

Perverted synthesis of substances that the cell does not normally reproduce;

Transformation of cells entering the cell nutrients to build one type of final product (protein, fat or carbohydrate).

Classification

Pathomorphologists highlight the following types parenchymal dystrophies:

1. Depending on morphological changes:

Purely parenchymal;

Stromal-vascular;

Mixed.

2. By type of accumulated substances:

Protein or dysproteinoses;

Fatty or lipidoses;

Carbohydrates;

Mineral.

3. According to the prevalence of the process:

System;

Local.

4. By time of appearance:

Acquired;

Congenital.

Pathological anatomy determines certain parenchymal dystrophies not only by the damaging agent, but also by the specificity of the affected cells. The transition of one dystrophy to another is theoretically possible, but in practice only a combined pathology is possible. Parenchymal dystrophies are the essence of the process occurring in the cell, but only part clinical syndrome, which covers morphological and functional impairment a certain organ.

Dysproteinoses

The human body is mostly made up of proteins and water. Protein molecules are components of cell walls, mitochondrial membranes and other organelles; in addition, they are in a free state in the cytoplasm. As a rule, these are enzymes.

Dysproteinosis is another name for a pathology such as parenchymal protein dystrophy. And its essence is that cellular proteins change their properties and also undergo structural changes, such as denaturation or colliquation. Protein parenchymal dystrophies include hyaline-droplet, hydropic, horny and granular dystrophies. The first three will be written in more detail, but the last, granular, is characterized by the fact that grains of protein accumulate in the cells, which is why the cells stretch, and the organ enlarges, becomes loose, dull. That is why granular dystrophy also called dull swelling. But scientists have doubts that this is parenchymal dystrophy. The pathological anatomy of this process is such that compensatory enlarged cellular structures can be mistaken for grains, as a response to functional stress.

Hyaline droplet dystrophy

McArdle's disease;

Hers disease;

Forbes-Cori disease;

Andersen's disease.

Their differential diagnosis possible after a liver biopsy and the use of histoenzyme analysis.

Glycoprotein metabolism disorder

These are parenchymal dystrophies caused by the accumulation of mucins or mucoids in tissues. Otherwise, these dystrophies are also called mucous or mucus-like, due to the characteristic consistency of the inclusions. Sometimes true mucins accumulate, but only substances similar to them that can become denser. In this case we're talking about about colloid dystrophy.

Tissue microscopy allows you to determine not only the presence of mucus, but also its properties. Due to the fact that cell debris, as well as viscous secretion, prevents the normal outflow of fluid from the glands, cysts form, and their contents tend to become inflamed.

The causes of this type of dystrophy can be very different, but most often it is catarrhal inflammation of the mucous membranes. Moreover, if hereditary disease, the pathogenetic picture of which fits well into the definition of mucous dystrophy. This is cystic fibrosis. The pancreas, intestinal tube, urinary tract, bile ducts, sweat and salivary glands are affected.

The resolution of this type of disease depends on the amount of mucus and the duration of its secretion. The less time has passed since the beginning pathological process, the more likely it is that the mucous membrane will recover completely. But in some cases, desquamation of the epithelium, sclerosis and dysfunction of the affected organ are observed.