Sterilisations at delivery or after childbirth: addressing continuing abuses in the consent process

Non-consensual sterilisation is not only a historic abuse. Cases of unethical treatment of women around the time of a pregnancy continue in the Twenty-First Century in five continents. Sterilisation is being carried out by some healthcare professionals at the time of delivery, or soon afterwards, without valid consent. A range of contemporary examples of such practices is given. Respecting women's autonomy should be the touchstone of the consent process. Avoidance of force, duress, deception and manipulation should go without saying. Ethnic minority communities and women living with HIV, in particular, are being targeted for this kind of abuse. Attempts have been made in various countries and by international professional organisations to introduce clinical guidelines to steer health professionals away from this malpractice. Survivors have sought justice in domestic and international courts. This paper critically assesses the evidence on the practical, ethical and legal issues around the handling of consent for these procedures. Suggestions are made about possible regulatory responses that address abuse, whilst maintaining access for those individuals who freely elect to undergo these procedures

Респираторные нарушения при полимиозите/дерматомиозите

Idiopathic inflammatory myopathies (IIM) are rare disorders characterized by inflammatory lesions in skeletal muscles. These diseases include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis, which exhibit clinicoimmunological heterogeneity and give different response to therapy. The most frequent manifestation in PM/DM patients is respiratory system dysfunction. The developing respiratory disorders are varied and may outpace the presentation of muscle pathology.Идиопатические воспалительные миопатии (ИВМ) – редкие аутоиммунные заболевания, характеризующиеся воспалительным поражением скелетной мускулатуры. К наиболее распространенным ИВМ относят: полимиозит (ПМ), дерматомиозит (ДM), которые характеризуются клинико-иммунологической неоднородностью и различным ответом на лечение. Наиболее часто встречающимся системным проявлением при ПМ/ДМ является поражение дыхательной системы. Развивающиеся при этом респираторные нарушения весьма разнообразны и могут опережать симптомы мышечной патологии

ASSESSMENT OF THE IMMUNOGENICITY AND SAFETY OF 23-VALENT POLYSACCHARIDE PNEUMOCOCCAL VACCINE IN PATIENTS WITH RHEUMATIC DISEASES

Objective: to investigate the immunogenicity and safety of 23-valent polysaccharide pneumococcal vaccine in patients with rheumatic diseases (RD).Subjects and methods. The prospective open-label comparative study enrolled 133 people (102 (76.7%) women and 31 (23.3%) men) aged 23 to 76 years, including 79 patients with rheumatoid arthritis (RA), 16 with systemic sclerosis, and 7 with dermatomyositis/polymyositis, as well as 31 subjects without systemic inflammatory RD (a control group), who had a recent history of at least two cases of lower respiratory tract infections (bronchitis, pneumonia). At their inclusion, all the patients with RD were receiving ant-inflammatory therapy, including 52 taking methotrexate (MT), 14 – leflunomide (LEF), and 13 – MT + tumor necrosis factor-α (TNF-α) inhibitors. The 23-valent polysaccharide pneumococcal vaccine Pneumo-23 (Sanofi Pasteur, France) was administered in a single dose of 0.5 ml subcutaneously during continuous MT or LEF therapy for the underlying disease or 3–4 weeks before the use of TNF-α inhibitors. Clinical examinations of the patients and conventional laboratory studies were performed during control visits (1, 3, and 12 months after vaccination). The serum levels of anti-pneumococcal capsular polysaccharide antibodies were measured in 102 patients by enzyme immunoassay using commercial VaccZymeTM Anti-PCP IgG Enzyme Immunoassay kits (The Binding Site Group Ltd, United Kingdom).Results and discussion. No clinical and radiological symptoms of pneumonia were recorded in any case during the follow-up period of 12 months. The patients with RD and the control group showed a significant, more than double increase in anti-pneumococcal antibodies 12 months following vaccination. Vaccination was well tolerated: 90 (68%) patients displayed no adverse events; 37 (28%) had pain, cutaneous swelling and hyperemia up to 2 cm in diameter at the site of injection for vaccination;6 (4%) had low-grade fever. There were no episodes of a RD exacerbation or any new autoimmune disorders during the follow-up period.Conclusion. The findings were suggestive of the sufficient immunogenicity and good tolerability of 23-valent pneumococcal vaccine in patients with RD

ПРЕДУПРЕЖДЕН — ЗНАЧИТ ВООРУЖЕН: ОСОБЕННОСТИ МРТ-ИССЛЕДОВАНИЯ ПРИ БОЛЕЗНИ ХИРАЯМА

Hirayama disease (HD) is a rare neurological condition typically thought to be endemic for South Asia andJapanregion. Disease is characterized by juvenile muscular atrophy of unilateral distal upper extremity with benign course. MRI of the cervical spine is the way to confirm HD in clinically supposed case. Some typical MR-signs can be visualized in neutral position (abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, localized lower cervical cord atrophy with asymmetric spinal cord flattening and hyperintensity) and in flexion position (anterior shifting of the posterior dura and posterior epidural space enlargement). Here we describe 5 HD cases in Russian population with a detailed MRI description and MRI protocol recommendations in case of HD suspicion. Болезнь Хираяма — заболевание, встречающееся преимущественно у жителей Японии и Юго-Восточной Азии и характеризующееся односторонней (реже — асимметричной двусторонней) прогрессирующей мышечной слабостью и неврогенными атрофиями мышц кисти и предплечья (миотомы С7, С8 и Th1). При МРТ-исследовании выявляется ряд характерных признаков как в стандартной укладке (изменение шейного лордоза, отсутствие прилегания задних отделов дурального мешка к дужкам позвонков, локальная атрофия спинного мозга с его асимметричным уплощением и повышением интенсивности МР-сигнала от его структуры на нижнешейном уровне), так и в положении максимального сгибания (смещение кпереди задней стенки дурального мешка на нижнешейном уровне, расширение заднего эпидурального пространства). В статье представлены пять случаев БХ у жителей Российской Федерации с подробным описанием МРТ-признаков заболевания и рекомендациями по проведению МРТ-исследования у пациентов с подозрением на БХ.

Respiratory disorders in patients with polymyositis/dermatomyositis

Idiopathic inflammatory myopathies (IIM) are rare disorders characterized by inflammatory lesions in skeletal muscles. These diseases include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis, which exhibit clinicoimmunological heterogeneity and give different response to therapy. The most frequent manifestation in PM/DM patients is respiratory system dysfunction. The developing respiratory disorders are varied and may outpace the presentation of muscle pathology

MYOSITIS ASSOCIATED WITH MALIGNANT TUMORS

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired systemic diseases mainly involving skeletal muscles. The main representatives of IIM are polymyositis (PM) and dermatomyositis (DM). Epidemiological surveys demonstrate that there is a relationship between PM/DM and malignant neoplasms (MNs), the detection risk of which is higher than that in the population of respective age groups. The rate of MNs in PM/DM ranges from 9 to 50%. The relationship to MNs is described in each subtype of IIM; however, these are most common in DM. The patients suffering from PM/DM associated with MNs have a worse prognosis than those without MNs. The early detection of MNs could improve the prognosis in these patients. The investigations published identify demographic, clinical, and laboratory factors increasing MN detection risks in patients with PM/DM. Just the same, they all cover small patient groups; the findings are heterogeneous and not well convincing, which calls for a further larger-scale study of this problem.Objective: to reveal and identify the specific features of paraneoplastic myositis (PnM).Subjects and methods. The investigation included 320 patients with a valid diagnosis of IIM, who had been followed up in the period of 1996 to 2016. The patients underwent laboratory tests, manual proximal muscle strength testing using a 10-point scale and electromyographic examination with needle electrodes.Results and discussion. PnM was detected in 32 (10%) of the 320 patients with IIM. Among the patients with PnM, there were 6 (19%) men and 26 (81%) women. The mean age at the onset of PnM was 55.4 years. PnM manifested with characteristic musculocutaneous syndrome in 19 (59%) patients; 18 (41%) of them were found to have MNs within the first year after disease onset. The manifestation of MNs was preliminary to the picture of PM/DM in 13 (41%) patients. The most commonly detected conditions were ovarian cancer (37.5%), MNs of the lung and breast (15%); next were MNs of the intestine (12.5%), blood (6.3%), uterus (6%), and stomach (3.1%). The median survival was 5 years in patients with PnM

PANNICULITIS IN DERMATOMYOSITIS

Dermatomyositis (DM) and polymyositis (PM) belong to idiopathic inflammatory myopathies (IIM) and are characterized by inflammatory injury to the skeletal muscles. In DM, unlike PM, there is skin injury that serves as a pathognomonic sign of this condition. Panniculitis (PN) is one of the rare cutaneous manifestations in this disease.Objective: to investigate the clinical and laboratory characteristics of IIM accompanied by PN and to elaborate guidelines for managing these patients.Subjects and methods. Examinations were made in 318 patients (75 men and 243 women) aged 18 to 80 years who were diagnosed with IIM (mean disease duration of 18.97±7.4 months) and followed up at the V.A. Nasonova Research Institute of Rheumatology in 1996 to 2015.Results and discussion. In 12 (3.8%) of the 318 patients, lobular PN that was associated with the diagnosis of DM verified in all cases. The clinical picture in this patient group was also characterized by skin injury as erythema on the face and trunk and Gottron’s papules (100%), periungual capillaritis (91.7%), ulceronecrotic vasculitis (57.3%), periorbital edema (75%), fever (41.7%), alopecia (50%), and joint involvement (25%).Conclusion. The development of PN is associated with the acute period of DM and the emergence of new foci is related to an exacerbation of this disease, which requires active therapy