Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-6-12
S. Kutsev, S. Moiseev
Family genetic testing of probands with newly diagnosed rare hereditary diseases including Fabry disease improves early diagnosis and allows to initiate specific treatment, if available, at earlier stage in affected family members. Diagnosis of Fabry disease, an X-linked lysosomal storage disorder affecting kidneys, heart, brain and other organs, is usually late due to low awareness of physicians about rare diseases. Moreover, early symptoms can be non-specific (e.g. gastrointestinal disorders and autonomic neuropathy) or misleading (e.g. recurrent unexplained fever) whereas characteristic skin rash and keratopathy (cornea verticillata) are frequently overlooked. Undiagnosed patients with Fabry disease can be detected by screening in at-risk populations, such as patients with end-stage renal disease undergoing dialysis or renal transplantation, patients with unexplained left ventricular hypertrophy, and young adults with a history of stroke or transient ischemic attack who have a higher prevalence of the disease compared to general population. High-risk screening paves the way to family screening to identify affected relatives, including children, who can benefit from earlier treatment and genetic counselling. The major barriers to family screening include costs of testing, cultural and societal issues, stigma associated with a diagnosis of genetic disease, low contacts in the family, weak infrastructure, national regulations.
{"title":"Family genetic screening in rare hereditary diseases","authors":"S. Kutsev, S. Moiseev","doi":"10.32756/0869-5490-2021-4-6-12","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-6-12","url":null,"abstract":"Family genetic testing of probands with newly diagnosed rare hereditary diseases including Fabry disease improves early diagnosis and allows to initiate specific treatment, if available, at earlier stage in affected family members. Diagnosis of Fabry disease, an X-linked lysosomal storage disorder affecting kidneys, heart, brain and other organs, is usually late due to low awareness of physicians about rare diseases. Moreover, early symptoms can be non-specific (e.g. gastrointestinal disorders and autonomic neuropathy) or misleading (e.g. recurrent unexplained fever) whereas characteristic skin rash and keratopathy (cornea verticillata) are frequently overlooked. Undiagnosed patients with Fabry disease can be detected by screening in at-risk populations, such as patients with end-stage renal disease undergoing dialysis or renal transplantation, patients with unexplained left ventricular hypertrophy, and young adults with a history of stroke or transient ischemic attack who have a higher prevalence of the disease compared to general population. High-risk screening paves the way to family screening to identify affected relatives, including children, who can benefit from earlier treatment and genetic counselling. The major barriers to family screening include costs of testing, cultural and societal issues, stigma associated with a diagnosis of genetic disease, low contacts in the family, weak infrastructure, national regulations.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"43 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88307668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-52-61
V. Rameev, S. Moiseev, L. Lysenko (Kozlovskaya)
AA amyloidosis complicates various chronic inflammatory disorders and is characterized by the accumulation of amyloid fibrils composed of serum amyloid A protein, an acute phase reactant. In recent decades, the role of chronic infections and rheumatoid arthritis in the ethiology of AA amyloidosis have decreased significantly as a result of their treatment improvement, whereas both monogenic (familial Meditarranean fever, cryopirin-associated periodic syndrome, etc.) or polygenic (ankylosing spondilitis, psoriatic arthritis, adult onset Still’s disease, etc) autoinflammatory diseases more frequently account for AA-amyloidosis today. Autoinflammatory diseases are a consequence of innate immunity disorders although the latter can contribute to the pathogenesis of autoimmune diseases as well. In patients with autoinflammatory diseases, the suppression of inflammation, even subclinical, is essential to prevent development or progression of AA amyloidosis. The choice of inflammatory agents that can be used to achieve this aim depends on the pathogenesis of autoinflammation, e.g. key mediators that are involved in the activation of inflammatory cascade.
{"title":"AA-amyloidosis in autoinflammatory diseases","authors":"V. Rameev, S. Moiseev, L. Lysenko (Kozlovskaya)","doi":"10.32756/0869-5490-2021-4-52-61","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-52-61","url":null,"abstract":"AA amyloidosis complicates various chronic inflammatory disorders and is characterized by the accumulation of amyloid fibrils composed of serum amyloid A protein, an acute phase reactant. In recent decades, the role of chronic infections and rheumatoid arthritis in the ethiology of AA amyloidosis have decreased significantly as a result of their treatment improvement, whereas both monogenic (familial Meditarranean fever, cryopirin-associated periodic syndrome, etc.) or polygenic (ankylosing spondilitis, psoriatic arthritis, adult onset Still’s disease, etc) autoinflammatory diseases more frequently account for AA-amyloidosis today. Autoinflammatory diseases are a consequence of innate immunity disorders although the latter can contribute to the pathogenesis of autoimmune diseases as well. In patients with autoinflammatory diseases, the suppression of inflammation, even subclinical, is essential to prevent development or progression of AA amyloidosis. The choice of inflammatory agents that can be used to achieve this aim depends on the pathogenesis of autoinflammation, e.g. key mediators that are involved in the activation of inflammatory cascade.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"287 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82835881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-29-35
E. Nabatchikova, T. Rozina, E. Nikulkina, E. Tanaschuk, S. A. Parfenova, N.Y. Nyaikina, L. V. Dubrovskaya, E. Starostina, D. Abdurakhmanov
To study the changes in liver function and portal hypertension, and clinical outcomes after elimination of hepatitis C virus (HCV) by direct-acting antiviral agents in patients awaiting an orthotopic liver transplantation (OLT).
{"title":"Elimination of hepatitis C virus in patients on the waiting list for liver transplantation","authors":"E. Nabatchikova, T. Rozina, E. Nikulkina, E. Tanaschuk, S. A. Parfenova, N.Y. Nyaikina, L. V. Dubrovskaya, E. Starostina, D. Abdurakhmanov","doi":"10.32756/0869-5490-2021-4-29-35","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-29-35","url":null,"abstract":"To study the changes in liver function and portal hypertension, and clinical outcomes after elimination of hepatitis C virus (HCV) by direct-acting antiviral agents in patients awaiting an orthotopic liver transplantation (OLT).","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"4 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90511533","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-23-28
N. Bulanov, P. Novikov, S. Gulyaev, I. Smitienko, A. Meshkov, O.O. Borodin, E. Makarov, T. Shevtsova, E. Shchegoleva, V. Nadtocheeva, A. V. Naumov, M. Brovko, S. Moiseev
To evaluate the tolerability and safety profile of GamCOVID-Vak (Sputnik V) vaccine in adult patients with autoimmune inflammatory rheumatic diseases.
{"title":"Tolerability and safety of Gam-COVID-Vac (Sputnik V) vaccine in adult patients with autoimmune rheumatic diseases","authors":"N. Bulanov, P. Novikov, S. Gulyaev, I. Smitienko, A. Meshkov, O.O. Borodin, E. Makarov, T. Shevtsova, E. Shchegoleva, V. Nadtocheeva, A. V. Naumov, M. Brovko, S. Moiseev","doi":"10.32756/0869-5490-2021-4-23-28","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-23-28","url":null,"abstract":"To evaluate the tolerability and safety profile of GamCOVID-Vak (Sputnik V) vaccine in adult patients with autoimmune inflammatory rheumatic diseases.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"8 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83469570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-36-43
P. Novikov, E. Shchegoleva, S. Moiseev
Interleukin (IL)-6 is a proinlammatory cytokine contributing significantly to the pathogenesis of joint disease and systemic manifestations of rheumatoid arthritis (RA). Levilimab is a new original monoclonal antibody that blocks both soluble and membrane-bound IL-6 receptors. Efficacy and favorable safety profile of levilimab in combination with methotrexate were shown in two randomized double-blind placebo-controlled trials (AURORA and SOLAR) that included patients with active RA despite treatment with methotrexate alone. Both primary and multiple secondary efficacy endpoints including ACR response, low disease activity or remission rates, changes in RA activity scores, etc, confirmed a higher efficacy of levilimab compared to placebo. Profile of adverse events was typical for IL-inhibitors. Several observational studies suggested that unlike rituximab or medium or high dose glucocorticoids IL-6 receptors inhibitors do not worsen outcomes of COVID19 and do not impair immunogenicity of vaccines against COVID-19. Therefore, patients treated with levilimab should not delay vaccination or modify the dosing regimen prior to vaccination.
{"title":"Efficacy and safety of levilimab, a monoclonal antibody to interleukin-6 receptors, in patients with rheumatoid arthritis","authors":"P. Novikov, E. Shchegoleva, S. Moiseev","doi":"10.32756/0869-5490-2021-4-36-43","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-36-43","url":null,"abstract":"Interleukin (IL)-6 is a proinlammatory cytokine contributing significantly to the pathogenesis of joint disease and systemic manifestations of rheumatoid arthritis (RA). Levilimab is a new original monoclonal antibody that blocks both soluble and membrane-bound IL-6 receptors. Efficacy and favorable safety profile of levilimab in combination with methotrexate were shown in two randomized double-blind placebo-controlled trials (AURORA and SOLAR) that included patients with active RA despite treatment with methotrexate alone. Both primary and multiple secondary efficacy endpoints including ACR response, low disease activity or remission rates, changes in RA activity scores, etc, confirmed a higher efficacy of levilimab compared to placebo. Profile of adverse events was typical for IL-inhibitors. Several observational studies suggested that unlike rituximab or medium or high dose glucocorticoids IL-6 receptors inhibitors do not worsen outcomes of COVID19 and do not impair immunogenicity of vaccines against COVID-19. Therefore, patients treated with levilimab should not delay vaccination or modify the dosing regimen prior to vaccination.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"25 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76454809","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-13DOI: 10.32756/0869-5490-2021-4-13-22
S. Moiseev, P. Novikov, N. Bulanov
The estimates of incidence and prevalence of systemic lupus erythematosus (SLE) in Europe are 1.5-4.9 per 100 000 persons-years and 30-70 per 100 000 people, respectively. Over the last 50 years, survival in SLE patients has improved significantly. Moreover, immunosuppressive treatment resulted in a decreased risk of death from active disease, whereas infections and cardiovascular disease have become the main causes of death in SLE populations. Almost 70% of SLE patients have recurrent course of disease, although long-term remissions or persistent disease activity also occur in a proportion of patients. Annually, every third SLE patient develops moderately severe or severe flares. Recurrent flares, complications of immunosuppressive treatment and comorbidity are associated with accrual of organ damage that increases the risk of death. SLE patients have impaired health-related quality of life correlating with both disease activity and organ damage. Being on remission of SLE or on low disease activity is associated with better outcomes, including lower mortality and risk of damage or flares, improved quality of life, lower hospitalisation rates and costs. Glucocorticoids remain the mainstay of SLE treatment, although their use should be limited, e.g. by proper administration of immunosuppressive or antiinflammatory agents that have steroid-sparing activity. Treatment and prevention of infections and cardiovascular outcomes are also essential for further improvement of survival of SLE patients.
{"title":"Systemic lupus erythematosus: epidemiology, outcomes and burden","authors":"S. Moiseev, P. Novikov, N. Bulanov","doi":"10.32756/0869-5490-2021-4-13-22","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-4-13-22","url":null,"abstract":"The estimates of incidence and prevalence of systemic lupus erythematosus (SLE) in Europe are 1.5-4.9 per 100 000 persons-years and 30-70 per 100 000 people, respectively. Over the last 50 years, survival in SLE patients has improved significantly. Moreover, immunosuppressive treatment resulted in a decreased risk of death from active disease, whereas infections and cardiovascular disease have become the main causes of death in SLE populations. Almost 70% of SLE patients have recurrent course of disease, although long-term remissions or persistent disease activity also occur in a proportion of patients. Annually, every third SLE patient develops moderately severe or severe flares. Recurrent flares, complications of immunosuppressive treatment and comorbidity are associated with accrual of organ damage that increases the risk of death. SLE patients have impaired health-related quality of life correlating with both disease activity and organ damage. Being on remission of SLE or on low disease activity is associated with better outcomes, including lower mortality and risk of damage or flares, improved quality of life, lower hospitalisation rates and costs. Glucocorticoids remain the mainstay of SLE treatment, although their use should be limited, e.g. by proper administration of immunosuppressive or antiinflammatory agents that have steroid-sparing activity. Treatment and prevention of infections and cardiovascular outcomes are also essential for further improvement of survival of SLE patients.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"106 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78189810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-09-05DOI: 10.32756/0869-5490-2021-3-67-75
P. Novikov, M. Brovko, V. Sholomova, L. Akulkina, V. Nadtocheeva, S. Moiseev
Levilimab, a monoclonal antibody interacting with soluble and mebrane bound IL-6 receptors, was recently approved in Russia for the treatment of acute respiratory distress syndrome in COVID-19 patients. Efficacy and favorable safety profile of levilimab were shown in the randomised doubleblind placebo controlled CORONA trial in 206 patients with COVID-19 associated pneumonia, who had at least one criteria of the disease severity, that is, increased respiration rate, reduced SpO2 (≤93%) or PaO2 /FiO2 (≤300 mm Hg), progressive lung disease, etc. The primary end-point of this study was the percentage of patients with stable improvement by ordinal scale (without a need in rescue levilimab injection) at day 14. Stable improvement was achieved in 63.1% and 42.7% of patients in the levilimab and placebo groups, respectively (р=0.0017), and this difference was consistent at the end of 30-day follow-up. Analysis of various secondary end-points, such as a need in rescue open-label levilimab or admission to ICU, confirmed efficacy of IL-6 inhibitor in patients with COVID -19. IL-6 inhibition was not associated with a higher risk of opportunistic infections. The authors present the case of successful levilimab administration in hospitalized patient with hypoxia and systemic inflammation and discuss the practical issues of IL-6 inhibitors use in COVID-19 patients.
利来单抗是一种与可溶性和膜结合IL-6受体相互作用的单克隆抗体，最近在俄罗斯被批准用于治疗COVID-19患者的急性呼吸窘迫综合征。随机双盲安慰剂对照CORONA试验在206例COVID-19相关性肺炎患者中显示了利来单抗的疗效和良好的安全性，这些患者的疾病严重程度至少有一个标准，即呼吸速率增加、SpO2(≤93%)或PaO2 /FiO2(≤300 mm Hg)降低、肺部疾病进展等。本研究的主要终点是在第14天，通过顺序量表(不需要挽救性注射利来单抗)稳定改善的患者百分比。利来单抗组和安慰剂组分别有63.1%和42.7%的患者实现了稳定改善(χ =0.0017)，这一差异在30天随访结束时是一致的。对各种次要终点的分析，如需要使用开放标签的利来单抗或入住ICU，证实了IL-6抑制剂对COVID -19患者的疗效。IL-6抑制与机会性感染的高风险无关。作者介绍了住院缺氧和全身性炎症患者给药成功的案例，并讨论了IL-6抑制剂在COVID-19患者中使用的实际问题。
{"title":"Levilimab, a monoclonal antibody to IL-6 receptors, in COVID-19","authors":"P. Novikov, M. Brovko, V. Sholomova, L. Akulkina, V. Nadtocheeva, S. Moiseev","doi":"10.32756/0869-5490-2021-3-67-75","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-3-67-75","url":null,"abstract":"Levilimab, a monoclonal antibody interacting with soluble and mebrane bound IL-6 receptors, was recently approved in Russia for the treatment of acute respiratory distress syndrome in COVID-19 patients. Efficacy and favorable safety profile of levilimab were shown in the randomised doubleblind placebo controlled CORONA trial in 206 patients with COVID-19 associated pneumonia, who had at least one criteria of the disease severity, that is, increased respiration rate, reduced SpO2 (≤93%) or PaO2 /FiO2 (≤300 mm Hg), progressive lung disease, etc. The primary end-point of this study was the percentage of patients with stable improvement by ordinal scale (without a need in rescue levilimab injection) at day 14. Stable improvement was achieved in 63.1% and 42.7% of patients in the levilimab and placebo groups, respectively (р=0.0017), and this difference was consistent at the end of 30-day follow-up. Analysis of various secondary end-points, such as a need in rescue open-label levilimab or admission to ICU, confirmed efficacy of IL-6 inhibitor in patients with COVID -19. IL-6 inhibition was not associated with a higher risk of opportunistic infections. The authors present the case of successful levilimab administration in hospitalized patient with hypoxia and systemic inflammation and discuss the practical issues of IL-6 inhibitors use in COVID-19 patients.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"27 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78997834","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-09-05DOI: 10.32756/0869-5490-2021-3-7-12
V. Syutkin
Assessment of the severity of liver cirrhosis as a mandatory parameter includes a characteristic of the degree of its compensation. Detailed historical research has shown that this characteristic of liver disease has no authorship, no precise definition, and seems to be a matter of course for the medical community. Moreover, the term “decompensation” (the adjustment of certain functions due to the adaptation of other organs and systems) does not reflect the pathophysiology of changes developing in patients with terminal liver cirrhosis. When determining the degree of compensation for cirrhosis, physicians are forced to focus on the classification of Child and Turcotte in Pugh's modification. This system was developed to assess the outcome of surgical treatment of portal hypertension and validated to predict the survival of patients with liver cirrhosis (expectation of an event in the future) in the short and medium term, but cannot be orrectly applied to assess the severity of liver cirrhosis (at the time of assessment). The inadequacy of the Child-Pugh prognostic system for assessing the severity of liver cirrhosis was shown by the example of determining the possibility of using HCV protease inhibitors in patients with complicated (“decompensated”) liver cirrhosis. It is necessary to develop new principles for assessing the severity of liver cirrhosis, in particular, in relation to the disorders of drug metabolism and the potential toxicity of drugs and their metabolites.
{"title":"Eligibility of the prognostic systems in assessing the severity of liver cirrhosis","authors":"V. Syutkin","doi":"10.32756/0869-5490-2021-3-7-12","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-3-7-12","url":null,"abstract":"Assessment of the severity of liver cirrhosis as a mandatory parameter includes a characteristic of the degree of its compensation. Detailed historical research has shown that this characteristic of liver disease has no authorship, no precise definition, and seems to be a matter of course for the medical community. Moreover, the term “decompensation” (the adjustment of certain functions due to the adaptation of other organs and systems) does not reflect the pathophysiology of changes developing in patients with terminal liver cirrhosis. When determining the degree of compensation for cirrhosis, physicians are forced to focus on the classification of Child and Turcotte in Pugh's modification. This system was developed to assess the outcome of surgical treatment of portal hypertension and validated to predict the survival of patients with liver cirrhosis (expectation of an event in the future) in the short and medium term, but cannot be orrectly applied to assess the severity of liver cirrhosis (at the time of assessment). The inadequacy of the Child-Pugh prognostic system for assessing the severity of liver cirrhosis was shown by the example of determining the possibility of using HCV protease inhibitors in patients with complicated (“decompensated”) liver cirrhosis. It is necessary to develop new principles for assessing the severity of liver cirrhosis, in particular, in relation to the disorders of drug metabolism and the potential toxicity of drugs and their metabolites.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"38 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87666418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-09-05DOI: 10.32756/0869-5490-2021-3-43-51
A. Moiseev, S. Moiseev, E. Tao, N. Bulanov, E. Mershina, D. Ismailova, N. Nosova, A. Kuchieva, V. Sholomova, P. Novikov, E. Pavlikova, V. Fomin, A. Safarova
To evaluate clinical manifestations and outcomes of Fabry disease (FD) in adult patients in the Russian population.
目的:评价俄罗斯成年患者法布里病(FD)的临床表现和预后。
{"title":"Clinical manifestations and outcomes of Fabry disease in 150 adult patients","authors":"A. Moiseev, S. Moiseev, E. Tao, N. Bulanov, E. Mershina, D. Ismailova, N. Nosova, A. Kuchieva, V. Sholomova, P. Novikov, E. Pavlikova, V. Fomin, A. Safarova","doi":"10.32756/0869-5490-2021-3-43-51","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-3-43-51","url":null,"abstract":"To evaluate clinical manifestations and outcomes of Fabry disease (FD) in adult patients in the Russian population.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89251647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-09-05DOI: 10.32756/0869-5490-2021-3-13-17
Y. Muravyov
The author reviews the current attitudes to vaccination against COVID-19 in patients with autoimmune inflammatory rheumatic diseases. Most experts admit theneed in vaccination, although data on its effectiveness and safety in patients with autoimmune inflammatory rheumatic diseases are limited, since such patients were not enrolled in phase I-III clinical trials. Vaccination is a cornerstone in the fight against the COVID-19 pandemic, althogh it cannot completely prevent SARS-CoV-2 infection. Therefore, vaccinated patients with autoimmune inflammatory rheumatic diseases should adhere to all measures that reduce the risk of infection, including wearing a mask, hand hygiene and social distancing.
{"title":"Current judgments regarding COVID-19 vaccination in patients with autoimmune inflammatory rheumatic diseases","authors":"Y. Muravyov","doi":"10.32756/0869-5490-2021-3-13-17","DOIUrl":"https://doi.org/10.32756/0869-5490-2021-3-13-17","url":null,"abstract":"The author reviews the current attitudes to vaccination against COVID-19 in patients with autoimmune inflammatory rheumatic diseases. Most experts admit theneed in vaccination, although data on its effectiveness and safety in patients with autoimmune inflammatory rheumatic diseases are limited, since such patients were not enrolled in phase I-III clinical trials. Vaccination is a cornerstone in the fight against the COVID-19 pandemic, althogh it cannot completely prevent SARS-CoV-2 infection. Therefore, vaccinated patients with autoimmune inflammatory rheumatic diseases should adhere to all measures that reduce the risk of infection, including wearing a mask, hand hygiene and social distancing.","PeriodicalId":10353,"journal":{"name":"Clinical pharmacology and therapy","volume":"37 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77064845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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