[Ultrastructure of epidermocytes in true pemphigus acantholysis]. / Ul'trastruktura épidermotsitov pri istinnoi akantoliticheskoi puzyrchatke.

OBJECTIVE: To investigate the ultrastructure of epidermocytes in skin biopsy specimens, by taking into account an update on the pathomorphogenesis of true pemphigus acantholysis. MATERIAL AND METHODS: Affected skin biopsy specimens from 4 patients with Pemphigus vulgaris (a mixed mucosal-epidermal variant) and from 1 patient with P. foliaceus at the onset of the disease in the absence of therapy were examined by light microscopy of semifine sections and by transmission electron microscopy. RESULTS: Skin biopsy specimens from pemphigus patients from the foci with a positive Nikolsky's sign were characterized by acantholysis and dilated intercellular spaces in the basal and spinous layers of the epidermis in combination with desmosomal hypoplasia and destruction. The reduction in the organelles of mitochondrial protein synthesis, which causes a decrease in the cytoplasm of the perikaryon and especially in the processes of a number of tonofilaments involved in the formation of desmosomes engaged our attention when studying the ultrastructure of epidermocytes. CONCLUSION: A marked reduction in the number and size of desmosomes in P. vulgaris and P. foliaceus starts in the basal layer of the epidermis; however, acanthosis occurs in the suprabasal and spinous layers, respectively. The universal manifestations of pathology of cytoskeletal elements involved in the formation of desmosomes, as well as their underproduction must be considered in the concept of the pathogenesis of true pemphigus acantholysis.

[Inherited epidermolysis bullosa]. / Vrozhdennyi bulleznyi épidermoliz.

OBJECTIVE: To summarize an update on epidermolysis bullosa as a polymorphic group of inherited diseases with a failure of epidermal-dermal integrity. Emphasis is placed on the role of transmission electron microscopy in diagnosis and search directions for new types of the abnormality and its molecular markers. Despite numerous mutations in the genes encoding the components of desmosomes and epithelial basement membrane, the stereotyped manifestations of pathological processes in the group of epidermolysis bullosa have been identified. The paper gives a positive result of cell and gene therapies used by European scientists in the treatment of a 7-year-old child with borderline epidermolysis bullosa, which opens up new prospects for patients with butterfly disease that has long been considered fatal.

PANCREATITIS IN PREGNANCY: CASE MANAGMENT.

The purpose of the review is to summarise the current data on chronic and acute pancreatitis with the goal to improve the diagnostics and treatment of women during pregnancy, which complicates differential diagnosis of hepatopancreatobiliary system pathology. The features of the incidence, etiology, parhogenesis and evaluation of the severity of the clinical manifestations of acute pancreatitis in pregnant women are given, It is emphasis that the most frequent its reason is the gallstones following by the biliary pancreatitis. The experience of use in pregnant patients imaging methods - endoscopic ultrasound, magnetic resonance cholangiopancrearography and endoscopic retrograde cholangiopancreatography, as well as treatments that include endoscopic sphincterotomy, stone extraction from the common bile duct and laparoscopic cholecystectomy. The recommendations are based on expert opinions and are not supported by randomized controlled trials. Nevertheless more active clinical management allows to diagnose and treat effectively pancreatitis, including biliary etiology, which contributes to a sharp decline in maternal and perinatal mortality.

Cytochrome P450 2D6 polymorphism is a molecular genetic marker of liver cirrhosis progression.

Patients with infectious viral or toxic cirrhosis of the liver participated in complex clinical pathomorphological and molecular-genetic study aimed at the search for markers of predisposition to accelerated liver fibrosis, in which the xenobiotic biotransformation system is involved. The results demonstrate association between CYP2D6 (1846G/A) genotype and rapid cirrhosis development and indicate the necessity of studying the mechanisms underlying this association.

Ultrastructural and stereological study of the liver in chronic mixed HCV+HBV infection.

The stereological values of the structural density of hepatocyte cytoplasmic organelles were similar in chronic mixed HCV+HBV infection irrespective of the form of HBV infection (seropositive or latent). High incidence of HBsAg- and HBeAg-negative forms of HBV infection determines the leading role of PCR diagnosis and studies of liver biopsy specimens with detection of HBcAg structural markers and HBV DNA in native liver tissue in the diagnosis of mixed HCV+HBV infection.

Structural modification of the gastrointestinal epithelium during immune-dependent granulomatosis.

We present results of pathomorphological study of the mucosa epithelium in various parts of the gastrointestinal tract in Crohn's disease. Particular attention was paid to structural transformation of epithelial cells. Barrier dysfunction of the gastrointestinal mucosa was shown to be one of the major pathogenetic factors for Crohn's disease.

Morphometric study of trephine biopsy specimens in aggressive and indolent non-Hodgkin's lymphomas.

Morphometric study of the erythroid stem was performed in aggressive and indolent non-Hodgkin's lymphomas vs. other cells and tissues of the bone marrow (including the tumor tissue) before chemotherapy. Hypoplasia and abnormal maturation of the erythroid stem were particularly pronounced in diffuse infiltration of the bone marrow, which did not depend on lymphoma aggressiveness. Hypoplasia of the erythroid stem was often observed during focal infiltration of the bone marrow with lymphoma cells (despite a smaller area of tumor tissue in aggressive lymphomas than in indolent lymphomas). A decrease in the relative area of adipose tissue, smooth resorption of bone tissue, and myelofibrosis are the major changes in the bone marrow microenvironment.

Ultrastructural modification of the endothelium in placental insufficiency and microangiopathies.

Structural reorganization of endotheliocytes was studied on models of various pathological processes: placental dysfunction, glomerular pathology, vibration syndrome, antiphospholipid syndrome, and diffuse angiokeratoma, all of these characterized by endothelial insufficiency. Universal modification of endothelial associations was revealed. It included a chain of stereotypical reactions: from degeneration alternating with compensatory hypertrophy to subsequent atrophy and death of endotheliocytes. The time course of the process was confirmed by the results of light microscopy in combination with ultrastructural examination and by evaluation of the biosynthetic reactions by in vitro radioautography.

Ultrastructural and histochemical markers of endometrial secretion induction in habitual miscarriage.

Biphasic hormone therapy at the stage of pre-gestation treatment of patients with habitual miscarriages stimulates the expression of progesterone receptors in the endometrium during the secretory phase of the menstrual cycle with full-value ultrastructural rearrangement of the endometrial glandular components in comparison with the patients receiving metabolic therapy alone.

Ultrastructure of connective tissue of eye drainage system in ophthalmic hypertension associated with primary juvenile glaucoma.

In primary juvenile glaucoma, the connective tissue of the juxtacanalicular zone and sclera undergo constant reorganization because of incessant synthesis and degradation of extracellular matrix components with pronounced fluctuations of electron density of collagen fibrils. Destructive changes in the perivascular nerve trunks were detected at all stages of the glaucoma process, including the initial stages: numerous large foci of cytoplasmic organelle destruction with the formation of autophagosomes and residual bodies predominated in the cytoplasm of myelinated axons of nerve cells.

67 stereotypes of structural modification of the urothelium in various diseases of the urinary bladder and prostate.

Structural modification of the urothelium was studied in various diseases of the urinary bladder and prostate, including urinary bladder cancer, vibration cystopathy, chronic prostatitis, benign prostate hyperplasia, and chronic cystitis. The general phenomena of changes in urinary bladder epithelium were atrophy, squamous metaplasia, and instability of the urothelium (focal atrophy, dysplasia, hyperplasia, and metaplasia). This phenomenon can be interpreted as a morphological marker for cancer risk.

Ultrastructure of the endothelium of the drainage system of the eye.

The endothelium of the ocular drainage system (Schlemm's canal, collector tubules, and aqueous veins) in primary juvenile glaucoma undergoes degenerative dystrophic changes with compensatory hypertrophy and proliferation at the initial stages of the glaucomatous process and atrophy and desquamation at advanced and terminal stages. Progressive decrease in the pinocytous function of endotheliocytes, reduction of the protein-synthesizing and mitochondrial compartments of the cytoplasm, and formation of autophagosomes reflect the process of endotheliocyte degeneration in general.

Structural modifications of the bronchial epithelium in asthma.

A complex of structural changes in the parenchymatous and stromal compartments, characteristic of the primary degenerative process (bronchial epithelium degeneration and subsequent diffuse atrophy, reactive sclerosis of the stroma) develops in the large bronchi in asthma. Induction of the regeneratory reactions of the bronchial epithelium with synchronously developing reorganization of the stroma (loosening of the connective tissue and stimulation of its vascularization) is shown during correction of asthma. Association of positive structural changes with lymphoid tissue hyperplasia in the bronchial wall can be interpreted in the context of significant role of lymphocytes in induction of the regeneratory reactions.

Contribution of ovarian follicular tissue abnormalities into the development of ovarian dysfunction.

Ultrastructural, in vitro radioautographic, and stereological study of the morphogenesis of ovarian cystic formations in women of reproductive age distinguished ovariopathy as a common pathological phenomenon, developing as a result of the ovarian follicular cell regenerative and plastic insufficiency syndrome. Degenerative dystrophic changes in the follicular compartment, reduced biosynthetic reactions in the follicular cells, and reactive sclerosis of the stroma remodulate the parenchyma-stromal relationships.

Ultrastructural changes in the epithelium in asthma associated with gastroesophageal reflux.

Asthma concomitant with gastroesophageal reflux is regarded in the context of systemic multifactorial primary degenerative process, based on discoordination of neurohormonal regulation and systemic metabolic and degenerative changes. Degeneration and atrophy of the structural components of the bronchial tree and gastric wall are the leading phenomenons determining the organ dysfunction.

Ultrastructural and immunohistochemical study of hepatic stellate cells over the course of infectious viral fibrosis and cirrhosis of the liver.

The dynamics of structural and functional changes in stellate cells in liver biopsy specimens (from lipid-containing to fibrogenous phenotype) was studied during the development of infectious viral fibrosis and cirrhosis of the liver using ultrastructural, immunohistochemical, and morphometric methods. The priority role of stellate cells in the synthesis of extracellular matrix components is emphasized. Resorption of perihepatocellular collagen fibrils is associated with parenchymatous liver cells.

Structural and functional modification of hollow organs (urinary bladder and stomach) in vibration syndrome.

Exposure to total and local vibration leads to the formation of specific forms of hollow organ diseases: vibration cystopathy and gastropathy. Their characteristic signs are organ dysfunction, thinned wall, atrophy of the mucosa, reduction of the capillary bed, and degenerative atrophic changes in the epithelium, associated with reduction of the protein-producing function and focal metaplasia. Vibration cysto- and gastropathies are systemic manifestations of microangio- and visceropathies of vibration origin.

Structural reactions of the buccal mucosa in diabetic parodontopathy.

Pronounced changes in the capillaries, hemodynamic disorders, epitheliocyte degeneration and atrophy develop in the buccal mucosa of patients with types I and II diabetes mellitus in the absence of inflammatory cellular infiltration or with facultative infiltration. The morphogenesis of pathological changes can be regarded as primary diabetic microangiopathy causing metabolic disorders with the development of degenerative and atrophic changes in all structural components of the buccal mucosa and development of diabetic parodontopathy, a primary degenerative process.

Ultrastructural and biosynthetic changes in epitheliocytes of renal tubules during the development of chronic renal failure.

Structural and functional studies of changes in the tubular compartment during progressive renal dysfunction showed that the major types of damage to tubular cells include dystrophy, degeneration, and atrophy. These changes were associated with inhibition of biosynthetic processes in the tubular epithelium, progressive interstitial fibrosis, and disturbances in the structure and function of peritubular microvessels.

Psoriasis associated with opisthorchiasis under conditions of anthelmintic therapy.

Biopsy specimens from patients with psoriasis concomitant with chronic opisthorchiasis were examined. Severe clinical course of combined disease was associated with degenerative changes in epidermocytes, keratinization disorders, and diffuse inflammatory cell infiltration of the derma. Combined therapy including anthelmintic agents produced a positive effect: decreased the degree of acanthosis, increased intracellular regeneration of epidermocytes, and suppressed inflammatory reaction of the derma and hyperplasia of immunocompetent cells. These data attested to an important role of opisthorchiasis in the pathogenesis of psoriatic disease.