LITERATURE REVIEWS
The literature review presents the current status of the method of high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation in the treatment of Crohn’s disease.In recent decades, there has been an increase in Crohn’s disease. The frequency of CD the response rate to biopreparations ranges from 20 to 50%, up to 80% of patients need surgical treatment. Due to the insufficient effectiveness of therapy, an active research is underway for new therapeutic opportunities, one of which is the method of HDIT-AHSCT. In the Russian Federation, there are data on the use of mesenchymal stem cells for the treatment of CD, there are no studies on the use of HDIT-AHSCT in IBD. According to the EBMT register in the period from 1994 to 2023. more than 4000 HDIT-AHSCT were performed in patients with autoimmune diseases, the proportion of CD was 6.13% of all protocols. The HDIT-AHSCT procedure consists of the following stages: mobilization of autologous HSCs, apheresis and cryopreservation of HSCs, immunoablative conditioning and infusion of HSCs. Most of the publications on the use of HDIT-AHSCT in CD were not universal in the selection and management of patients. Currently, there is the only one completed RCT on the use of HDIT-AHSCT in CD, the results of which have not received a definite outcome. However, for a cohort of patients with refractory CD, it is necessary to consider the possibility of using this method. The review presents the indications and selection criteria for the HDIT-AHSCT protocol. Further studies of the HDIT-AHSCT in CD will help expand the therapeutic opportunities of treating patients with refractory CD to standard and biological therapy.
In recent years, increasing attention of researchers has been paid to the role of melatonin in the regulation of body weight. When analyzing literature data, the problem of the mechanism of action of melatonin on the body and its use in the correction of metabolic disorders is considered. Disruption of circadian rhythms caused by the use of artificial light at night leads to decreased melatonin secretion. This helps increase appetite and reduce energy costs. In addition, melatonin plays a major role in modulating the secretion of adipokines by influencing lipid metabolism. Decreased melatonin production causes insulin resistance and metabolic circadian disorganization, leading to obesity. Assessing the role of melatonin in the development of obesity and its accompanying pathologies is a promising area of scientific research in the field of diagnosis, prevention and treatment of endocrine and cardiovascular pathologies.
Bronchiolitis obliterans syndrome (BOS) is a rare non-infectious pulmonary complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) or lung transplantation. Early diagnosis of BOS requires new approaches including the search for biological markers of pulmonary damage after allo-HSCT. The aim of this work is to review literary data on biological markers of BOS. Conclusion. Literary data on biomarkers of BOS in allo-HSCT recipients are limited. Further research is needed.
Chronic fatigue is one of the most common syndromes in the clinical practice of medical doctors of various specialties. However, issues of criteria for distinguishing chronic fatigue from physiological fatigue, differential diagnosis of reactive asthenia, secondary fatigue syndrome and myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) as a distinct nosological form can be challenging for physicians. The provision of care to patients with ME/CFS is negatively impacted by the lack of accurate and up-to-date knowledge about the disease among healthcare professionals. Even if doctors are compassionate, they often do not know how to make a diagnosis or what can be done to improve the well-being of patients. Currently, this problem is especially relevant due to the increased risk of developing ME/CFS after suffering from a new coronavirus infection, COVID-19. This article presents a modern approach to the diagnosis of ME/CFS, set out in consensus recommendations that were developed in 2021 independently by groups of experts on the diagnosis and treatment of this disease in European countries and the USA, and taking into account the recommendations of domestic specialists.
Connective tissue dysplasia is a disturbance of its development during embryogenesis and the postnatal period due to genetically altered fibrillogenesis of the extracellular matrix. Undifferentiated connective tissue dysplasia (UCTD) includes a set of phenotypic characteristics that do not fit into any already known dysplastic syndrome or phenotype. It is this pathology of connective tissue that is widespread and is constantly encountered in the practice of doctors of various profiles, serving as the basis for the formation of various chronic diseases. As for bronchial asthma (BA), it is quite natural that studies have appeared to identify the characteristics of the course of BA with connective tissue dysplasia, primarily in children. It has been shown that asthma in children associated with connective tissue dysplasia has a number of clinical features: an earlier onset of the disease, a higher incidence of concomitant diseases, and a more severe course. The features of the course of bronchial asthma associated with connective tissue dysplasia in adults have been practically not studied. Our very first results from a study of adult patients with BA showed that in patients with a mild course of the disease, up to 3 stigmas of disembryogenesis were recorded, in patients with a moderate course - from 2 to 4, and in patients with a severe course - from 1 to 8 stigmas. Correlation analysis (the data was obtained on a small material, therefore we do not consider it statistically significant) provides a distinct basis for continuing the study. Thus, negative correlations have been identified between the presence of stigmas (myopia, dolichostenomelia, joint hypermobility, wide atrophic scars, varicose veins) and such an important characteristic of the disease as its controllability.
ORIGINAL RESEARCH
In the clinic of coronavirus infection (SARS-CoV-2), inflammatory symptoms of the upper respiratory tract and pulmonary parenchyma prevail, and intestinal damage and the appearance of digestive symptoms are more characteristic of long-term pathology. The aim of the study was to retrospective analyze fatal cases of intestinal lesions in a new Covid-19 infection. In total, 9 patients with fatal intestinal lesions were identified out of a total of 2,781 deaths during the three years of operation of the Nikolaevskaya Hospital hospital in St. Petersburg, which was repurposed to provide medical care to patients with the new coronavirus infection Covid-19 (SARS-Cov-2). The described pathoanatomical manifestations in the form of mesenteric vascular thrombosis and wall necrosis, corresponding to the clinical definition of «fatal intestinal lesion», are recognized as conditions incompatible with life and unavoidable at the stage of qualified and specialized therapeutic care.
According to the clinical recommendations of the Ministry of Health of the Russian Federation in 2021, at least 348 million patients worldwide suffer from bronchial asthma (BA). The treatment of patients with BA has objective difficulties due to the progression of obstruction and insufficient diagnosis of pathogenetic forms of BA. It is known that in women, hormonal status, namely female sex hormones such as estradiol and progesterone, contributes to the course and development of BA. This article presents an original clinical study of 48 patients suffering from mild to moderate asthma with a regular menstrual cycle, and the relationship between the increase in symptoms of asthma and the phase of the menstrual cycle is estimated. According to the results of the study, all patients were divided into groups with regular asthma (RA) and premenstrual asthma (PMA). A significant increase in the level of estradiol was obtained (934.50 (405.00; 1010.25) pmol/l) in patients with PMA in the second phase of the menstrual cycle in relation to the control group (494.00 (328.00; 487.00) pmol/l) and the group of patients with RA (694.50 (495.25; 993.00) pmol/l). No significant and significant changes in progesterone, prolactin, LH, or FSH were detected. The revealed decrease in respiratory function in patients with PMA in the second phase of the menstrual cycle emphasizes and probably explains the worsening of symptoms of asthma in this group of patients, which, with possible hormonal correction, can lead to stabilization of the course of asthma.
CLINICAL CASES
Hereditary angioedema (HAE) is an orphan disease that causes potentially life-threatening edema in various locations and remains serious problem in both pediatrics and adult clinical practice often disguised as various diseases. The HAE diagnosis with normal C1 inhibitor activity is particularly difficult. The purpose of this publication is to demonstrate unique clinical case of HAE with normal C1 inhibitor activity in young patient with a pathogenic mutation in the MEFV gene, which is associated with familial Mediterranean fever. Severe abdominal attacks in this patient have been regarded for a long time as a part of therapeutic pathology (gastroenteritis, gastritis, Familial Mediterranean fever), acute surgical and gynecological diseases, which led to late diagnosis verification. The HAE requires timely diagnosis, elimination of factors that provoke exacerbations and an immediate therapy administration, that significantly improves life quality of patients and helps prevent death.
In rheumatological practice, overlap syndrome (“overlap-syndrome”) is often encountered, which is manifested by the presence of diagnostic signs of more than one of the six classic systemic autoimmune diseases. The presence of chronic viral infections significantly complicates drug therapy. The article presents the history of the disease of a 46-year-old patient who was diagnosed with an overlap syndrome in the form of a combination of idiopathic polymyositis with antisynthetase syndrome and rheumatoid arthritis. The complexity of treating the patient was determined by the identification of active chronic viral hepatitis C. A multidisciplinary approach to the management of the patient in compliance with the principles of rational pharmacotherapy is described.
ANNIVERSARIES AND MEMORABLE DATES
The article is dedicated to the 140th anniversary of the outstanding native clinician, member of the therapeutic school of S. P. Botkin, Academician M. V. Chernorutsky. It traces the life way of Mikhail Vladimirovich from his first steps to formation as an outstanding scientist, teacher and clinician, which years of life fell on a period of difficult trials for the country, covering revolutionary events, the First and Second World Wars. The social and professional activities of M. V. Chernorutsky during the siege of Leningrad, the deprivations of which he shared with the residents of the city, are outlined in detail. The scientific achievements of Mikhail Vladimirovich are examined in detail: the doctrine of constitutional types, the study of nutritional dystrophy and ways of its correction in besieged Leningrad, the creation of the Leningrad rheumatological school. The enormous contribution of M.V. Chernorutsky contributed to the development of the Leningrad society of therapists, and the department of hospital therapy, which he headed for more than 30 years, trained outstanding and bright native clinicians, such as Professor P.K. Bulatov and corresponding member RAS G.B. Fedoseev.