Involvement Of Many Organs Research Articles

The ultrasound characteristics of the cardiovascular system in systemic lupus erythematosus in children

Background. Systemic lupus erythematosus (SLE) is one of the most typical diffuse connective tissue diseases characterized by the involvement of many organs and systems in the pathological process. Cardiac involvement in SLE is one of the most common manifestations of visceritis in both adults and children. According to various authors, the frequency of heart involvement ranges widely from 40% to 90–95%, with changes being detected in 100% of patients during autopsy. Establishing an accurate diagnosis of cardiovascular system changes in SLE is challenging yet crucial for assessing disease activity and prognosis. This has become possible with the clinical application of modern objective functional methods of examination such as echocardiography and Doppler, which have significantly increased the detection rate of even subclinical forms of heart involvement. Purpose – of the study is to examine the structural and functional state of the heart in children with systemic lupus erythematosus using echocardiography. Materials and Methods. We analyzed echocardiography and Doppler data of 32 children aged from 11 to 18 years old with systemic lupus erythematosus who were examined and treated at the Institute for the Protection of Children’s and Adolescents’ Health of the National Academy of Medical Sciences of Ukraine. There were 7 boys (21.87%) and 25 girls (78.13%) among the patients. The diagnosis was confirmed through comprehensive clinical, laboratory, and instrumental examinations. Ultrasound scanning of the heart and major vessels was performed using «Siemens SL-1» and «Logic-400» General Electric pulse-wave devices with color mapping. Results. The study revealed that the most frequent signs of systemic connective tissue dysplasia diagnosed by echocardiography were the presence of abnormal chords in the left ventricle (in 24 patients – 75%) and mitral valve prolapse (in 12 patients – 37.50%). A typical sign of the disease was lupus carditis manifested by the reduction in the left ventricular diameter systolic contraction (22 patients – 68.75%) and thickening of the left ventricular myocardium and the thickness of the interventricular membrane in 15 patients (46.88%). Left ventricular contractile function was reduced (EF < 60%) in 15 patients (46.88%). In cases with a disease duration of up to two years and grade I activity of the pathological process, an increase in contractile function was observed in 3 patients (9.38%). Grade I/II mitral insufficiency was present in 2 patients (6.25%). Grade I aortic insufficiency was found in 1 patient (3.13%). Characteristic changes in the right ventricle were also observed in patients with systemic lupus erythematosus. The most common findings included enlargement of the right ventricular cavity and the outlet tract of the right ventricle (in 15 patients – 46.88%), hyperkinesia (in 3 patients – 9,38%) and myocardial thickening. Accumulation of fluid in the pericardial cavity during echocardiography was observed in 9 patients (28.13%) as a sign of polyserositis. The amount of liquid was small. Conclusions. The study established the polymorphism of pathological changes in the cardiovascular system of patients with systemic lupus erythematosus, which dictates the necessity of conducting echocardiography and Doppler both at the initial examination and during monitoring of the pathological process and treatment quality. The high frequency of diagnosed abnormal chords may indicate a congenital predisposition of connective tissue in patients with systemic lupus erythematosus to dysplasia. Echocardiography is especially valuable for identifying valve and pericardial lesions.

Importance of ferritin research in clinical practice

Serum ferritin (SF) is typically present in serum at concentrations directly related to iron (Fe) storage and is therefore traditionally used as an indicator of Fe levels in body tissues. Reducing its level is the “gold standard” for diagnosing widespread iron deficiency conditions. No less significant is hyperferritinemia — a nonspecific syndrome that occurs when Fe reserves are overloaded, a number of immunoinflammatory, infectious, oncological diseases, liver diseases, etc. In many pathological conditions, the level of SF determines the severity and prognosis of the disease. Ferritin concentrations greater than 1000 ng/mL, regardless of cause, have been shown to be associated with higher mortality. The reason for the increase in the level of SF in liver pathologies (cancer, hepatitis, cirrhosis) is associated with the process of its release from hepatocytes during their destruction. On the other hand, excessive synthesis and/or cellular secretion of ferritin occurs under the influence of various stimuli (cytokines, oxidative stress, hypoxia, oncogenes and growth factors). The interpretation of elevated ferritin values goes far beyond the role of an indicator of replenishment of Fe stores in tissues. Only 10% of cases of hyperferritinemia are associated with iron overload; in most patients, it is defined as the result of the acute phase and a reactive increase in ferritin levels against the background of any disease. The variety of symptoms of iron deficiency syndromes and hyperferritinemia is due to the involvement of many organs and systems, which requires a thorough examination (study of complaints, anamnesis, family and concomitant diseases, as well as the necessary laboratory and instrumental studies) to search for possible causes. Systemic Fe homeostasis must be closely monitored on a regular basis. It is required to comply with the conditions for blood sampling for SF. Reference values for SF concentrations vary depending on the analytical methods used and the population studied. Age and gender play an important role. Given the range of reference values, it is important for a particular patient to focus on the initial level of his ferritin, determined against the background of health and well-being during clinical observation.

Damage to the organ of hearing and upper respiratory tract in vasculitis associated with antineutrophil cytoplasmic antibodies

Antineutrophil cytoplasmic antibodies associated vasculitis (ANCA-AV) is a systemic necrotizing granulomatous vasculitis affecting mainly small-caliber vessels. ANCA-AV occupy a special place among systemic vasculitis, which is characterized by a highly active life-threatening course of the disease, requiring rapid differential diagnosis and aggressive immunosuppressive therapy. The ANCA-AV group consists of 3 nosologies: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. The “calling card” of ANCA-AV is the lesion of the upper respiratory tract, especially the ENT organs (70–100 % of patients). The nasal cavity and paranasal sinuses are the most common areas of lesion in the head and neck (85–100 %), whereas ear damage occurs in about 35% (range, 19–61 %) of cases. Lesion of the ENT organs is typical for the debut of ANCA-AV, which makes early diagnosis difficult, since diseases of the upper respiratory tract are extremely common in all age groups. Diagnosis verification occurs mainly at the stage of generalized involvement of many organs and systems, causing severe course with the development of pulmonary-cardiac and renal insufficiency, which lead to the death of the patient. The main ENT manifestations of ANCA-AV can be grouped into several groups: sinonasal, otological, tracheobronchial, oral cavity lesions and others. Pseudotumors are often found in ANCA-AV. They are characterized by the appearance of parapharyngeal, parotid, submandibular, paratracheal volumetric formations. As a rule, the appearance of tumor-like formations is observed at an early stage of the disease and is associated with the presence of antibodies to proteinase 3, systemic manifestations of vasculitis. Pseudotumor in the ENT region may be accompanied by secondary neuropathies of cranial nerves, destruction of bone tissue, which requires histological verification of the disease.

CLINICAL AND ANAMNESTIC FEATURES OF THE DYNAMICS AND TRANSFORMATION OF IRON-DEFICIENCY ANEMIA IN ADOLESCENT GIRLS

Annotation: According to the World Health Organization, in any population, for every person suffering from IDA, there are three with iron deficiency (IAD). The risk group for the development of anemia includes children, women of reproductive age and the elderly. A special group among them are girls of pubertal age, who have a high potential for the formation of various organ dysfunctions. The purpose of this work was to study the clinical and anamnestic features of the dynamics and transformation of iron deficiency anemia in adolescent girls. Object and methods of the study: adolescent girls aged 12-14 years (n=177) of the Uzbek population, respectively, with LAD (45), mild IDA (56) and moderate (25), and the control group (51). For the study, whole blood and serum of adolescent girls with IDA were used. General clinical, instrumental, biochemical and statistical methods were used. Results: From the data of our material it follows that the clinical picture of IDA in schoolgirls was somewhat transformed and among them the syndrome of decreased immunological resistance of the body and gastrointestinal syndrome began to prevail, their frequency increased by more than 2-5 times compared to the above authors. The clinical picture of iron deficiency anemia in adolescent girls is characterized by polymorphism of symptoms and syndromes, which indicates the involvement of many organs and systems in the pathological process. It is concluded that it is necessary to update data on the clinical symptoms of ID in adolescent girls, depending on age, gender and regions of residence.

Burden of Mycobacterium tuberculosis and Associated Factors among Presumptive Extra pulmonary Tuberculosis Patients from Selected Health Facilities, Addis Ababa, Ethiopia

BackgroundExtra pulmonary tuberculosis (EPTB) accounts for a significant proportion of tuberculosis (TB), a devastating disease of public health concern. The complexity of the cases, the involvement of many organs, resource constraints, and concerns regarding drug resistance make disease diagnosis and treatment difficult. This study aimed to determine the burden of tuberculosis and associated factors among presumptive EPTB patients in selected hospitals in Addis Ababa. Material and methodsA cross-sectional study was conducted from February to August 2022 in selected public hospitals in Addis Ababa. Those who attended the hospitals and were presumptively diagnosed as EPTB patient were included in the study. Sociodemographic and clinical data were collected using a semistructured questionnaire. The GeneXpert MTB/RIF assay, Mycobacterium Growth Indicator Tube (MGIT) culture, and solid culture using Löwenstein–Jensen (LJ) medium were used. The data were entered and analyzed using SPSS version 23, and a p-value ≤ 0.05 was considered as statistically significant. ResultsFrom a total of 308 participants enrolled in this study, the measured burdens of extrapulmonary tuberculosis using the Xpert MTB/RIF assay, liquid culture, and solid culture were 54 (17.5%), 45 (14.6%), and 39 (12.7%), respectively. In this study, sex, contact history with known TB cases, having a purulent type of aspirate, and being HIV positive had statistically significant associations with EPTB. ConclusionsThe burden of extrapulmonary tuberculosis among presumptive extrapulmonary tuberculosis cases was found to be significant. Sex, contact history with a known TB case, having apurulent type of aspirate, and being HIV positive were found to be associated with extrapulmonary tuberculosis infection. Strict adherence to the national tuberculosis diagnosis and treatment guidelines is important, while the true burden of the disease should be ascertained using standard diagnostic tests for better prevention and control interventions.

Clinical and anamnestic features of the dynamics and transformation of iron-deficiency anemia in adolescent girls

According to the World Health Organization, in any population, for every person suffering from IDA, there are three with iron deficiency (ID). The risk group for the anemia development includes children, women of reproductive age and the elderly. A special group among them are girls of pubertal age, who have a high potential for the formation of various organ dysfunctions. The purpose of this work was to study the clinical and anamnestic features of the dynamics and transformation of iron deficiency anemia in adolescent girls. Object and methods of the study: adolescent girls aged 12-14 years (n=177) of the Uzbek population, respectively, with LID (45), mild IDA (56) and moderate IDA (25), and the control group (51). For the study, whole blood and serum of adolescent girls with IDA were used. General clinical, instrumental, biochemical and statistical methods were used. From the data of our material, it follows that the clinical picture of IDA in schoolgirls was somewhat transformed and among them the syndrome of decreased immunological resistance of the body and gastrointestinal syndrome began to prevail, their frequency increased by more than 2-5 times compared to the above authors. The clinical picture of iron deficiency anemia in adolescent girls is characterized by polymorphism of symptoms and syndromes, which indicates the involvement of many organs and systems in the pathological process. We’ve concluded that it is necessary to update data on the ID clinical symptoms in adolescent girls, depending on age, gender and regions of residence.

Musculoskeletal pain in patients with COVID-19

Introduction: The end of 2019 marked the beginning of a new disease for the mankind, which changed the lives of people all over the world. Almost 3 years have passed from the declaration of a global pandemic till today, but despite this, our knowledge of the disease COVID-19 caused by SARS CoV-2 is still the object of many researches. This is because the heterogeneous manifestation of the disease and the involvement of many organs and systems in the clinical presentation. The spectrum of rheumatological manifestations that directly concern the rheumatologist is also very miscellaneus. Most common are musculoskeletal pain phenomena as a rheumatic manifestation of the disease. Objectives: The purpose of the study is to assess the frequency and intensity of pain of musculoskeletal origin - joint (arthralgia) and muscle (myalgia) and its relationship with anxiety and depressive attitudes in hospitalized patients with COVID-19 in the Rheumatology Clinic - Varna. Material and methods: In a single-center, observational study, were included patients with diagnosed COVID-19, hospitalized in the Rheumatology Clinic, UMBAL "St. Marina" - Varna. The etiological diagnosis was accepted with a positive result of polymerase chain reaction (PCR) test or rapid antigen test for SARS-CoV-2. An inclusion criterion was new-onset musculoskeletal pain during the acute phase of COVID-19. Visual analogue scales (VAS) were used to assess pain intensity (muscular and joint) and Tsung self-report scales for depression (SDS) and anxiety (SAS). Laboratory acute inflammatory indicators and thrombotic biomarkers were investigated, chest imaging was performed in all patients. Descriptive statistics, Spearman Rho correlation analysis and SPSS linear regression analysis were used. A significance level of p<0.05 was accepted. Results: 226 patients (aged 26-91 years) with musculoskeletal pain were included. 46.5% (n=105) were women and 53.5% (n=121) were men. The mean age of the study population was 65.5 years (65.57 ± SD). A significant correlation (p=0.001) and predictive value was found between the score for depression and anxiety and the intensity of pain (for the two studied pain phenomena - arthralgias and myalgias). No correlation was found between pain intensity and inflammatory markers, nor with thrombogenic markers in patients with SARS-CoV-2. Of all examined patients with musculoskeletal pain, 46.5% (n=105) were women and 53.5% (n=121) were men. The average age of the study population was 65.5 years (65.57). After the correlation and regression analysis, a significant correlation (0.001) and predictive value was found between the score for depression and anxiety and the strength of pain (for the two studied pain phenomena - arthralgias and myalgias). On the other hand, in the studied patients, we found no correlation with either inflammation indicators or thrombogenic markers in the SARS-CoV-2 patients.mong all 226 COVID-19 patients with musculoskeletal pain, 46.5% (n =105) were women and 53.5% (n =121), were men. Mean age was 65 and a half years (65.57). After correlation and regression (ANOVA) analysis made we found strongly significant correlation (correlation is significant et the 0.001 level) and predictive value between depression and anxiety scales score from one side and pain intensity from other (both arthralgia and myalgia). On the other hand, musculoskeletal pain does not correlate with any of inflammation and thrombotic biomarkers assessed in SARS-CoV-2 patients. mong all 226 COVID-19 patients with musculoskeletal pain, 46.5% (n =105) were women and 53.5% (n =121), were men. Mean age was 65 and a half years (65.57). After correlation and regression (ANOVA) analysis made we found strongly significant correlation (correlation is significant et the 0.001 level) and predictive value between depression and anxiety scales score from one side and pain intensity from other (both arthralgia and myalgia). On the other hand, musculoskeletal pain does not correlate with any of inflammation and thrombotic biomarkers assessed in SARS-CoV-2 patients. Conclusion: Musculoskeletal pain is one of the most common clinical presentations of COVID-19. The intensity of the pain correlates with anxiety and depressive symptoms in these patients and does not correlate with the levels of inflammation and thrombotic biomarkers.

Neurological Manifestations of Fabry Disease: Literature Review

BACKGROUND: Fabry disease (FD) or Anderson FD is a hereditary disease belonging to the group of lysosomal storage diseases caused by decreased or absent activity of the enzyme α-galactosidase A. Enzyme deficiency leads to accumulation of glycospholipids in the lysosomes of cells of various organs, including the heart, kidneys, nervous system, and vascular endothelium. The complexity of the diagnosis of FD is due to the variety of its symptoms, the simultaneous involvement of many organs and systems. At present, possible pathogenetic treatment of the disease is enzyme replacement therapy, but its effectiveness is reduced in the later stages of the disease, when there are irreversible abnormal changes in vital organs and systems. In this regard, an urgent task is the early diagnosis of FD. AIM: Determination of neurological manifestations of FD as well as clinical criteria for screening for FD. MATERIALS AND METHODS: We analyzed cohort studies, randomized controlled trials, systematic reviews and meta-analyses, case-control studies, and case series from scientific medical databases: PubMed, Web of Science, Google Scholar in Russian, and English languages. CONCLUSION: The authors found that lesions of the nervous system in FD are detected in more than 80% of patients and can manifest as isolated or combined lesions of both the central and peripheral and autonomic nervous systems.

Disability in systemic lupus erythematosus

Systemic lupus erythematosus is one of the most severe systemic diseases with autoimmune damage to the connective tissue, microvasculature vessels and a number of internal organs. In the clinical picture of the disease, lesions of the skin (pathognomonic feature is the appearance of an erythematous rash in the form of a butterfly on the bridge of the nose and cheeks), the occurrence of Raynaud's syndrome, involvement of the joints in the pathological process, proceeding as arthritis, damage to the lungs and kidneys are in the first place. Most often, the onset of disease is noted at the age of 15-25 years; mainly women are affected. The disease onset is not very specific: a temperature reaction and asthenic syndrome are noted; skin rashes or a microcirculatory reaction may appear in response to exposure to cold or stressful situations. The danger of systemic lupus erythematosus is the polysyndromic nature of the disease, which consists in the involvement of many organs and systems in the pathological process. The kidneys are most often and severely affected with the development of lupus nephritis, which can cause the rapid development of chronic renal failure. The manifestation of a convulsive syndrome with the subsequent development of mental disorders is not uncommon. Disability in systemic lupus erythematosus is given in case of the development of chronic renal failure of stage 2A and above, with complete atrioventricular block with the need to install a pacemaker, with damage to the vision organ and the nervous system with impaired functional activity. Systemic lupus erythematosus requires lifelong use of glucocorticoid drugs and, in some cases, the use of extracorporeal detoxification methods to remove metabolic products and circulating immune complexes.

Мукополісахаридоз II типу в практиці терапевта

Стаття присвячена одній із спадкових хвороб — мукополісахаридозу II типу, що розвивається в результаті дефіциту лізосомальних ферментів і призводить до порушення катаболізму основної речовини сполучної тканини — вуглеводної частини молекули мукополісахаридів (глікозаміногліканів). При цьому в тканинах (переважно у фібробластах і мезенхімальних клітинах) накопичуються хондроїтинсульфат В і/або гепаранмоносульфат, що веде до неповноцінної будови сполучної тканини, викликає грубі клітинні зміни і виникнення характерної клінічної картини. До особливостей їх клінічної картини належать різноманіття проявів, залучення в патологічний процес багатьох органів і систем, різний вік початку хвороби, з чим і пов’язані труднощі в діагностиці цих захворювань. За наявності вираженого клінічного поліморфізму (інфантильні, ювенільні, дорослі форми) всі лізосомальні хвороби характеризуються прогредієнтним перебігом і в більшості своїй призводять до ранньої інвалідизації і передчасної смерті. У світі найбільш часто зустрічається мукополісахаридоз II типу — синдром Хантера. Автори наводять власне клінічне спостереження і вказують на особливості перебігу респіраторної патології у даної категорії дорослих пацієнтів.

Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis

Objectives In IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), involvement of two or more sets of lacrimal glands (LGs) and/or major salivary glands (MSGs) is regarded as a specific finding with diagnostic significance. This study aimed to clarify the influence of this factor on the overall clinical picture of IgG4-DS. Methods We retrospectively reviewed the medical records of 130 patients with IgG4-related disease, 97 of whom were diagnosed with IgG4-DS. We determined their clinical features according to the presence/absence of involvement of ≥2 sets of LGs and/or MSGs and compared the results with those obtained in 33 DS-limited patients. Results The IgG4-DS patients comprised 60 men and 37 women (median age 65 years). The median serum IgG4 level at diagnosis was 548 mg/dL. The patients with involvement of ≥2 sets (n = 44) had significantly more affected organs, lower serum C3 and C4 levels, and a tendency to have higher serum IgG levels and IgG4-RD responder index than did those without it (n = 53). In the 33 DS-limited patients, these two groups had no significant differences in clinical features. Conclusions Involvement of ≥2 sets of LGs and/or MSGs suggests greater systemic disease activity mainly reflected by involvement of more organs.

Lysosomal storage diseases: mucopolysaccharidosis type I and II

Mucopolysaccharidosis (MPS) are a genetically heterogeneous group of rare monogenic metabolic diseases associated with hereditary insufficiency of lysosomal enzymes involved in the catabolism of glycosaminoglycans, or mucopolysaccharides. The pathogenesis of MPS is due to the accumulation of non-cleaved glycosaminoglycans in lysosomes, which can destroy cells. All MPS are characterized by a polysystemic manifestation, the simultaneous involvement of many organs and tissues in the pathological process, first of all, connective tissues, bones and cartilaginous. This review presents the epidemiology, clinical, biochemical, and molecular genetic characteristics of MPS types I and II, caused by the recessive mutations in the alpha-L-iduronidase and iduronate-2-sulfatase genes, respectively, and by the accumulation of dermatan and heparan sulfate. Each of these diseases is characterized by clinical polymorphism, especially observed in MPS I, which often manifests in a severe form of Hurler syndrome, but can also occur in a milder form of Scheie syndrome. Currently, there is an increased interest in MPS in the world due to the identification of the spectrum and frequencies of mutations in theIDUAandIDSgenes in various populations, including in Russia, and the practical availability of methods for individual molecular diagnostics. The description of the existing experimental models, their role in the study of the biochemical basis of the pathogenesis of these severe hereditary diseases and the development of various therapeutic approaches are given. Discusses the possibility of early diagnosis of MPS I and II types based on neonatal screening in order to increase the effectiveness of their prevention and treatment, as well as the advantages and disadvantages of the main approaches to the treatment of these serious diseases, such as hematopoietic stem cell transplantation, enzyme replacement and substrate-reducing therapy. A clinical example of a combination therapy for a severe form of mucopolysaccharidosis type I Hurler syndrome is presented

HCV-RELATED VASCULITIS

Up to 3% of people in the world are infected with HCV, and 75-80% of them have chronic currency of infection. In the clinical picture of HCV infection, a significant place is given to extrahepatic manifestations - more than half of patients suffer from lesions of the skin, kidneys, heart, blood vessels, autoimmune and lymphoproliferative syndromes associated with HCV. Usually, mortality from chronic hepatitis C is associated with liver cirrhosis and hepatocellular carcinoma, but in fact, more patients die from complications associated with the development of cryoglobulinemic vasculitis - chronic renal failure and cardiovascular catastrophes. 40-60% of patients with chronic HCV infection suffer from cryoglobulinemic vasculitis in a wide range of clinical manifestations: from minimal to life-threatening. The pathogenesis of mixed cryoglobulinemia syndrome is based on polyclonal activation of lymphocytes.The systemic nature of the lesion, which is observed in HCV infection, reflects its generalized nature with the involvement of many organs and tissues in the pathological process, which complicates timely diagnosis and treatment. A variety of systemic extrahepatic pathology, which often outstrips the clinical picture of hepatitis itself, masquerading as another disease means that a specialist of any profile can meet with chronic HCV infection and its consequences.

Systemic sclerosis - multidisciplinary disease: clinical features and treatment.

Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.

Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study.

The aim of this study was to investigate the predictive factors for relapse of IgG4-related disease (IgG4-RD) and observe the long-term clinical outcomes in patients with IgG4-RD. We included in the present analysis 122 patients who were newly diagnosed with IgG4-RD, treated with glucocorticoid (GC) monotherapy or GC and immunosuppressant combination therapy, and followed for at least 3years. Clinical relapse, response and side effects were recorded. The cumulative relapse rates of patients in this study were 10.66%, 22.95% and 27.87% at 12, 24 and 36months, respectively. Complete drug withdrawal was an independent risk factor for disease relapse. Higher serum IgG4 concentrations, involvement of more organs, higher IgG4 RI scores and elevation of eosinophils at baseline were closely associated with disease relapse. Re-elevation of serum IgG4 concentrations and low GC maintenance dosage during the follow-up period were significantly associated with clinical relapse. The GC dosage should be more than 6.25mgday-1 as monotherapy during the maintenance stage; moreover, combining with immunosuppressants can reduce the GC dosage. Adding GC or immunosuppressants for patients with re-elevation of serum IgG4 levels could prevent later disease relapse. No serious complications were noted during long-term follow-up. The combination of GC with immunosuppressants was more effective than GC monotherapy during the steroid tapering and maintenance stages. Higher serum IgG4 levels, involvement of more organs, higher IgG4 RI scores, history of allergy, eosinophil elevation at baseline, re-elevation of serum IgG4 levels and lower GC maintenance dosage at follow-up might be predictive of relapse.

IMPORTANCE OF INSULIN-LIKE AND VASCULAR AND ENDOTHELIAL GROWTH FACTORS IN THE PATHOGENESIS OF POLYCYSTIC OVARY SYNDROME (REVIEW OF LITERATURE)

The study of various mechanisms of the pathogenesis of polycystic ovary syndrome (PCOS) shows the multifactorial nature of its development with the involvement of many organs and systems, as well as growth factors that are actively involved in the growth and proliferation processes of both normal and pathologically altered biological tissues, including the ovaries, not only with PCOS, but also with endometriosis and tumor formations. The article discusses new data on the involvement of insulin-like and vascular endothelial growth factors in the pathogenesis of PCOS. The study of their role is relevant and promising task.

Forecasting the course of occupational chronic fl uorine intoxication by means of univariate analysis

Relevance. In contemporary occupational pathology, diagnosis of occupational fluorosis is based on locomotory apparatus disorder such as fluorine osteopathy. Other significant consequences of negative effects of fluorine compounds are deactivation of enzymatic systems, metabolic disorders — that results in pathologic involvement of many organs and systems (hepato-biliary, cardiovascular, respiratory, nervous, endocrine, gastrointestinal). Topical issue is search of a complex of factors influencing development of the occupational disease, to optimize management of occupational fluorine intoxication risk.Objective. To determine spectrum of factors that promote development of occupational fl uorine intoxication in workers exposed to inorganic fl uorine compounds, for forecasting the disease outcome and selecting main trends of preventive measures.Materials and methods.Single-factor analysis methods helped to carry retrospective cohort study of occupational fl uorine intoxication development in 201 workers of aluminum production in Ural region. Th e study covered infl uence of main occupational factors and somatic health parameter s on occupational fl uorosis development.Results.Findings are reliable infl uence of age (k=0.532, p<0.001), length of service in hazardous work conditions (p<0.001), hydrofl uoride level (p<0.001) and constant magnetic fi eld (p=0.005). Besides that, analysis of immune and metabolic state of the workers revealed signifi cant infl uence of disordered lipid, carbohydrate, purine metabolism, altered immune parameters on occupational fl uorosis development. Other evidence was reliable dependence between concomitant cardiovascular diseases and period of occupational chronic fl uorine intoxication development.Conclusion.Using single-factor analysis helped to determine wide spectrum of factors associated with the workers’ health state and work conditions, that signifi cantly infl uence development of fl uorine intoxication. Th e authors proved that period of occupational chronic fl uorine intoxication is notably infl uenced by occupational factors (length of service, hydrofl uoride level, fl uorine load degree, magnetic fi eld) and somatic state characteristics as age, parameters of carbohydrate, lipid, purine metabolism, status of cardiovascular, excretory systems, kidneys and gastrointestinal tract functions.

Small molecule biomarkers in Alzheimer’s disease

Alzheimer’s disease (AD) is a progressive neurodegenerative disease which affects a growing number of people as the population ages worldwide. Alzheimer’s Disease International estimated that more than 100 million people will be living with dementia by 2050. At present there are no disease-modifying therapies and research has expanded to the −omic sciences with scientists aiming to get a holistic view of the disease using systems medicine. Metabolomics and Lipidomics give a snap-shot of the metabolism. As analyzing the brainin vivois difficult, the metabolic information of the periphery has potential to unravel mechanisms that have not been considered, such as those that link the brain to the liver and the gut or other organs. With that in mind we have produced a mini-review, to record a number of studies in the field and the molecular pathways that have been flagged in animal and human models of AD. Human studies deal with cohorts in the order of the hundreds due to the difficulty of organizing AD studies, however it is possible that these first pilots point towards important mechanisms. The trend in these small studies is the involvement of many organs and pathways. Some findings, that have been reproduced, are ceramides being increased, phospholipids and neurotransmitters depleted and sterols being found depleted too. Initial findings point to an important role to lipid homeostasis in AD, this is not surprising as the brain’s main constituents are water and lipids.

Pathogenesis of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease with an extremely complex pathogenesis. Due to a genetic predisposition the disease can be induced by multiple stress factors involving epigenetic mechanisms and under the influence of the innate immune system. Defective clearance of immune complexes and apoptotic material together with enhanced neutrophil extracellular trap formation (NETosis) as well as up-regulation of type 1 interferon in particular, drive the adaptive immune system to a breakdown of self-tolerance. The result is a B cell hyperactivity, which leads to the generation of a multitude of different autoantibodies that are not only directed against nuclear antigens. Autoantibodies are the initiators for the involvement of many organs, which enhances further inflammatory cells and cytokines by participation of effector T-cells. Finally, an autoreactive immunological (plasma cell) memory is formed, which contributes to chronification and is associated with therapy-refractive courses of the disease. The depletion of the autoreactive immunological memory by immunoablation can lead to induction of self-tolerance and long-term remission.

Therapeutic trial in hereditary angioedema type III: Icatibant

Background Hereditary angioedema (HAE) is a disease transmitted by autosomal dominant inheritance, characterized by quantitative and / or functional deficiency of C1 inhibitor (C1-INH), which causes episodes of swelling, with involvement of many organs. HAE is currently divided into three groups. The HAE type III is a less frequent disorder that mainly affects women and is characterized by normal levels and activity of C1-INH.