[Pathological profile of S.P. Botkin infectious hospital during the siege of Leningrad (1941-1944)]. / Patologo-anatomicheskii profil' infektsionnoi bol'nitsy im. S.P. Botkina v gody blokady Leningrada (1941­1944 gg.).

The results of autopsies performed in the pathological department of the Infectious Diseases Hospital named after. S.P. Botkin during the siege of Leningrad (from September 8, 1941 to January 27, 1944). The structure of diseases of the deceased varied during different periods of the siege of Leningrad. In the first period (September-December 1941), diphtheria, dysentery, measles, typhoid fever, and scarlet fever prevailed among the diseases. The most common causes of death in the second period (April-December 1942) were typhus, dysentery, tuberculosis, lobar pneumonia, and typhoid fever. Nosological structure in the third period of the blockade (January 1943 - January 1944): tuberculosis, dysentery, cachexia, lobar pneumonia, infectious jaundice. The discrepancy between clinical and morphological diagnoses is most often noted for the following nosology: pulmonary tuberculosis, typhoid fever, pneumonia, stomach and hepatopancreatobiliary cancer, measles, influenza. The first period of the blockade was distinguished by a high specific proportion of examination of children's bodies - 51.2% of all autopsies; in subsequent periods, the specific share of autopsies of deceased adults (20-59 years) increased to 76.2%. The difference in the nosological structure and age groups of those who died during different periods of the siege of Leningrad was determined by the epidemiological situation in the city, social and living conditions and medical and organizational factors. Conducted in the pathological-anatomical department of the hospital named after. S.P. Botkin during the siege of Leningrad, pathological studies made it possible to timely establish the causes of deaths and identify the peculiarities of the course of infectious diseases against the background of cachexia. Regularly held clinical and anatomical conferences contributed to the reduction of defects in the diagnosis and treatment of infectious diseases.

In Vivo DYSF Gene Viral Delivery Provides a Histoprotective Effect in Skeletal Muscle Tissue in Dysferlin-Deficient Mice.

We studied the effects of a dual-vector DYSF gene delivery system based on adeno-associated virus serotype 9 capsids on pathological manifestations of dysferlinopathy in skeletal muscles of Bla/J mice lacking DYSF expression. The mice received intravenous injection of 3×1013 genomic copies of the virus containing the dual-vector system. M. gastrocnemius, m. psoas major, m. vastus lateralis, and m. gluteus superficialis were isolated for histological examination in 3, 6, and 12 weeks after treatment. Healthy wild-type (C57BL/6) mice served as positive control and were sacrificed 3 weeks after injection of 150 µl of 0.9% NaCl into the caudal vein. To detect dysferlin in muscle cryosections, immunohistochemical analysis with diagnostic antibodies was performed; paraffin sections were stained with hematoxylin and eosin for morphometric analysis. After administration of gene-therapeutic constructs, muscle fibers with membrane or cytoplasmic dysferlin location were detected in all examined muscles. The proportion of necrotic muscle fibers decreased, the number of muscle fibers with central location of the nucleus increased, and the mean cross-section area of the muscle fibers decreased.

[Masks hiding mitochondrial neurogastrointestinal encephalomyopathy. Case report].

The first documented case of mitochondrial neurogastrointestinal encephalomyopathy was described in 1962 by R. Luft. The variety and am-biguity of the clinical manifestations of the disease complicate its early diagnosis and treatment. The first clinical manifestations of the disease are associated with the pathology of the gastrointestinal tract. Low alertness and insufficient awareness of doctors delays the timely diagnosis of mitochondrial neurogastrointestinal encephalomyopathy. The aim of the work is to increase the alertness and awareness of narrow specialties about the possibility of differential diagnosis of an extremely rare detected disease on the base of our clinical observation.

Structural and ultrastructural changes in the skeletal muscles of dysferlin-deficient mice during postnatal ontogenesis.

A number of sarcolemma proteins are responsible for muscle fiber repair. Dysferlin encoded by the DYSF gene is one of these proteins. Dysferlin promotes membrane repair in striated muscle fibers (MFs). Mutations in DYSF lead to loss of or decreased dysferlin expression, impaired membrane repair in MF, and its destruction, clinically manifesting as dysferlinopathy. Preclinical studies of cell and gene therapies aimed at restoring impaired muscle regeneration require well-characterized small animal models. Our investigation aimed to distinguish the histopathological features of a mouse strain lacking dysferlin expression (Bla/J strain). Ultrastructural changes in the sarcolemma, mitochondria and contractile apparatus were observed. It was shown that postnatal histogenesis of skeletal muscles in genetically determined dysferlin deficiency is characterized by a higher proportion of necrotic muscle fibers, compensatory hypertrophy of muscle fibers with their subsequent atrophy, and decreases in proliferative activity and the level of myogenic differentiation of myogenic progenitor cells compared to wild-type mice (C57Bl/6).

[Pathohistological characteristics of muscles in patients with chronic lower limb ischemia]. / Patogistologicheskaya kharakteristika myshts u patsientov s khronicheskoi arterial'noi nedostatochnost'yu nizhnikh konechnostei.

Despite the widespread occurrence of ischemic diseases of the lower extremities, including atherosclerosis and diseases with an autoimmune component of their pathogenesis, the pathohistological signs of damage and concomitant chronic ischemia, compensatory tissue responses as intracellular and cellular regeneration remain out of the field of vision in researchers. OBJECTIVE: To assess the signs of damage (the extent of necrosis and apoptosis, capillary density (CD)) and regeneration (the cross-sectional muscle fiber area (CSMFA), the proportion of centrinucleated muscle fibers (CNMFs), and that of connective tissue), by using the gastrocnemius medial head biopsy specimens obtained from patients with heterogeneous forms of chronic lower limb obliterating diseases (CLLODs). SUBJECTS AND METHODS: The investigation included the biopsy specimens obtained from 44 men under 65 years of age (their mean age was 54±9.8 years) with Stage IIB-IV chronic limb ischemia (according to the A.V. Pokrovsky-Fontaine classification) with its history of at least six months. The nosological entities were atherosclerotic lesion in 33 patients (distal atherosclerosis n=13), multistage lesion (n=8), and Leriche's syndrome (n=12); autoimmune-mediated vascular injury in 11 patients (Buerger's disease (n=7) and nonspecific aortoarteritis (n=4)). The similar muscle fragments obtained during autopsy from the deceased without obvious signs of cardiovascular system diseases were examined as a control. RESULTS: It was found that there was a statistically significant difference between the nosological entities, as compared to the control in terms of CD and CSMFA (a decrease), the proportion of CNMFs and that of connective tissue (an increase). No substantial differences were found in the studied parameters between the nosological entities. CONCLUSION: The findings may suggest the universal mechanism for damage to striated muscle tissue because of circulatory hypoxia, regardless of its etiology and the common character of tissue compensatory-adaptive responses (regeneration).

[Professor Boris Petrovich Ugryumov is a prominent military pathologist]. / Professor Boris Petrovich Ugryumov ­ vidnyi voennyi patologoanatom.

The biographical article is dedicated to the memory of Boris Petrovich Ugryumov, a graduate of the Imperial Military Medical Academy (1914), a prominent military pathologist, the First Head of the Department of Pathological Anatomy, Ryazan Medical Institute. The paper presents the major milestones in the life of B.P. Ugryumov; his service on the fronts of the First and Second World Wars and his teaching activity at the Military Medical Academy and the Naval Medical Academy occupy an important place. For about 10 years, he was in charge of the Pathology Department, S.P. Botkin Clinical Infectious Diseases Hospital in Leningrad, which largely determined the area of his professional interests, such as the pathomorphology of infectious diseases, tuberculosis in particular. The archiving and personal photographic documents that have been previously unknown to the public are published for the first time.

Reactive Changes in Elements of Stromal-Vascular Differons of Dysferlin-Deficient Skeletal Muscles after Procaine Injection.

The study assessed reactivity of stromal-vascular skeletal muscle differons to acute chemical injury. Dysferlin-deficient Bla/J mice and the wild-type С57BL/6 mice were intramuscularly injected with 100 µl of 0.5% procaine solution. The middle segment of gastrocnemius muscle was taken on postsurgery days 2, 4, 10, and 14 for routine histological examination. To evaluate proliferation and vascularization, the paraffin sections were stained immunohistochemically with antibodies to α-smooth muscle actin and Ki-67. The connective tissue was stained according to Mallory. The study revealed diminished proliferative activity of stromal-vascular differons and decreased vascular density in muscles of Bla/J mice. Thus, mutations in the DYSF gene coding dysferlin down-regulate the reparation processes in all differons of skeletal muscle.

[Oleg Konstantinovich Khmelnitsky (1920-2004). On the occasion of the 100th birth anniversary]. / Oleg Konstantinovich Khmel'nitskii (1920­2004). K 100-letiyu so dnya rozhdeniya.

November 4, 2020 marked the 100th anniversary of the birth of one of the leading pathologists of Russia, Honored Scientist of Russia, Corresponding Member of the Russian Academy of Medical Sciences, President of the Russian Association of Pathologists, Honorary Doctor of St. Petersburg Academy of Postgraduate Education Oleg Khmelnitsky - a man of bright, multifaceted, effective in his work, who had numerous pupils and followers.

[To the 125th birthday of Professor Vyacheslav Konstantinovich Beletsky]. / K 125-letiyu so dnya rozhdeniya professora Vyacheslava Konstantinovicha Beletskogo.

This year marks the 125th anniversary of the famous scientist and pathologist Vyacheslav Konstantinovich Beletsky. The scientific biography of the doctor included the stages of study and diagnosis of infectious lesions of the central nervous system, war pathology and pathomorphology of rheumatism. To a large extent, his professional interests were focused on the problem of the participation of microglia cells in the development of pathological processes in the central nervous system. As an ideological and scientific follower of William Robertson and Pio Rio-Ortega, he created his own system of studying and evidencing of histogenesis of microglia from the mesenchymal embryonic premordium. Applying a complex of histological, pathological, anatomical and embryological research methods, V.K. Beletsky substantiated the doctrine of the so-called mesoglia - the connective tissue (mesenchyme) of the central nervous system.

Early ultra- and microstructural alterations in rat pancreas in alloxan-induced diabetes mellitus.

An adequate experimental model is important to understand pathophysiological processes ongoing in the pancreas with diabetes mellitus. Our study was aimed to describe early ultra- and microstructural changes in the rat pancreas in 12-48 h after alloxan administration in a dose of 180 mg/kg. A histopathological examination of the endocrine pancreas revealed the loss of borders between endocrine cells, granular dystrophy and degranulation, sings of necrosis in central cells of the Langerhans islets and apoptosis of their peripheral ones manifested as DNA fragmentation and an increased expression of apoptosis markers. There was a gradual increase of a Langerhans islet area, a decreased percentage of insulin+ cells and an increased one of glucagon+ cells, as well as the presence of proliferating islet cells were found. Structural changes of the exocrine pancreas included fatty degeneration, signs of exocrine cell mitochondrial damage, increased acini, which are located mainly around the Langerhans islets, as well as perivascular edema and leukocytic infiltration. Described ultra- and microstructural alterations suggest a significant contribution of apoptosis to death of endocrine cells exposed to alloxan. Coexisting damage of the exocrine pancreas with its stroma involvement is for the first time described.