Experience of vaccination of a patient with systemic juvenile idiopathic arthritis (sJIA) with a 13-valent pneumococcal conjugate vaccine, prior to the appointment of therapy with tocilizumab, an anti-IL-6-receptor monoclonal antibody
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01.01.2018 |
Vankova D.
Alekseeva E.
Soloshenko M.
Dvoriakovskaia T.
Isaeva K.
Denisova R.
Mamutova A.
Rudnitskaya M.
Mayansky N.
Tkachenko N.
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Voprosy Sovremennoi Pediatrii - Current Pediatrics |
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© 2018 Voprosy Sovremennoi Pediatrii - Current Pediatrics. All rights reserved. Background. Infections are the main cause of death for patients with autoimmune rheumatic diseases. In adult patients with rheumatoid arthritis (RA), mortality caused by respiratory infections is 2-5 times higher than in the population. One of the frequent infectious complications in the course of treatment with tocilizumab, the first-choice drug for treating systemic juvenile idiopathic arthritis (sJIA), is pneumonia characterized by a poor clinical picture, normal values of laboratory indices of the disease activity (ESR, C-reactive protein) with pronounced changes in the lungs revealed by computed tomography. In case of acute respiratory infection in children with systemic JIA, immunosuppressants and genetically engineered biological preparations (GEBP) are discontinued. This often leads to an exacerbation of the underlying disease and the progression of a pathological process. At present, vaccination against pneumococcal infection in Russia is not included in the standard for managing patients with rheumatic diseases. Studies of the safety and efficacy of vaccination with 13-valent pneumococcal conjugate vaccine (PCV) in patients with sJIA receiving genetically engineered biological preparations were not conducted. Clinical Case Description. The article shares the experience of vaccination of a girl aged 9 years with a 13-valent PCV that was conducted in the course of a scientific investigation, which studied the efficacy and safety of vaccination of children with systemic JIA prior to prescription of GEBP tocilizumab. Vaccination did not cause a deterioration in the course of the main disease (1 month), led to a reduction in the incidence of acute respiratory infections (from 4 to 1 time within 6 months before and after vaccination), and discontinuation of antibacterial drugs within 6 months after vaccination. Conclusion. The safety of a 13-valent PCV in a child with sJIA and a decrease of the incidence of respiratory diseases after vaccination, their complications, and the use of antibacterial drugs have been shown.
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A familial case of insulin-dependent diabetes mellitus with a mutation in the PTF1A gene
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01.01.2018 |
Svetlova G.
Kuraeva T.
Sechko E.
Peterkova V.
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Problemy Endokrinologii |
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Copyright © 2018 by the MediaSphere. Diabetes mellitus (DM) is a genetically heterogeneous disease, and some cases of type 1 diabetes mellitus (T1DM) are caused by a mutation of one gene. The child has suffered from brittle diabetes since the age of 1 year and received insulin. The HbA1c level is 9.9-11.4%. The patient was examined at the age of 14 years; the disease duration is 13 years. The daily dose of insulin is 1.15-1.35 U/kg. DM complications include initial manifestations of distal diabetic polyneuropathy as well as cheiropathy. The mother (39 years) and grandmother (74 years) have T1DM with multiple complications. A genetic study revealed that all three family members had a heterozygous substitution p.P274 in the PTF1A gene with an unknown pathological significance. At present, it can not be asserted that the identified mutation is the etiologic factor of diabetes in the described case. We can not exclude T1DM in this child because specific antibodies have not been tested. However, the mutation is detected in all three members of the same family with diabetes, which does not exclude the discovery of a new form of MODY, not described earlier.
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Clinical masks of bone sarcomas in children: Six clinical cases
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01.01.2018 |
Rykov M.
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Voprosy Sovremennoi Pediatrii - Current Pediatrics |
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© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. Solid tumors in children are one of the most common childhood malignancy, second only to hemoblastosis. Among solid tumours, about 5% are bone sarcomas: osteosarcoma (3%) and Ewing's sarcoma (2%). Atypicality of the these diseases course makes an early diagnosis a real challenge. Case Reports. The article presents six clinical observations of patients with bone sarcomas. We demonstrate the difficulties in diagnosing of this disease group which is associated with the absence of both specific symptoms and distinct clinical picture. Conclusion. Pediatricians and pediatric surgeons should take into account the possibility of atypical course of bone sarcomas in children. Low cancer alertness is the reason for a significant delay in establishing the correct diagnosis which contributes to the tumour process generalization and reduces the chances of achieving remission while increasing the cost of treating such patients.
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Toxic epidermal necrolysis as a variant of severe skin lesions in systemic lupus erythematosus
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01.01.2018 |
Vorobyeva L.
Aseeva E.
Solovyev S.
Belousova T.
Lopatina N.
Sazhina E.
Serikova G.
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Nauchno-Prakticheskaya Revmatologiya |
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© 2018 Ima-Press Publishing House. All rights reserved. Toxic epidermal necrolysis (TEN) has been long believed to be the most severe manifestation of drug allergy. However, cutaneous changes as TEN in systemic lupus erythematosus (SLE) were first described in the late 1970s. As of now, the English-language literature published reports of 30 cases of such lesions in SLE. This paper describes a clinical case of TEN as a direct manifestation of SLE; the positive experience has been first depicted in using not only intravenous immunoglobulin, but also rituximab with a good therapeutic effect in Russian clinical practice.
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Bone sarcomas in children: Clinical features
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01.01.2018 |
Rykov M.
Sevryukov D.
Senzhapova E.
Hajrullova V.
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Rossiyskiy Vestnik Perinatologii i Pediatrii |
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© 2018 National Academy of Pediatric Science and Innovation. All rights reserved. Solid tumors in children occupy the second place in the structure of morbidity, yielding to hemoblastosis. Among solid tumors, approximately 5% are bone sarcomas: osteosarcoma (3%) and Ewing's sarcoma (2%). Atypicality of the course of these diseases makes it difficult to diagnose them early. The article describes a series of clinical observations of patients with bone sarcomas, which illustrate the complexity of diagnosing diseases of this group.
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The problem of diagnosis of generalized and focal forms of salmonellosis
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01.01.2018 |
Malov V.
Maleyev V.
Parkhomenko Y.
Tsvetkova N.
Smetanina S.
Gorobchenko A.
Belugin V.
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Terapevticheskii Arkhiv |
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© 2018 Media Sphera Publishing Group.All Rights Reserved. The article describes the clinical observation with an unfavorable outcome of the generalized form of salmonella infection caused by Salmonella typhimurium group B in a woman of 60 years without immunodeficiency, complicated by the development of multiple abscesses of the lower parts of abdominal cavity, probably of appendicular origin. In a short literary review, the pathogenetic mechanisms that contribute to the formation of generalized and extraintestinal forms of salmonella infection are discussed.
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