Для вивчення особливостей клінічного перебігу й порушення нервово-м’язової передачі в пацієнтів з міастенією залежно від структурних змін тимуса були проаналізовані результати клініко-нейрофізіологічного обстеження 78 хворих. Матеріали та методи. Залежно від структурних змін тимуса всі хворі були поділені на 3 групи. Перша група — 29 пацієнтів з міастенією без змін структури тимуса. Друга група — 30 пацієнтів, у яких міастенія супроводжувалася гіперплазією тимуса. Третя група — 19 хворих з міастенією на фоні тимоми. Ступінь тяжкості захворювання оцінювався за допомогою кількісної шкали тяжкості клінічних проявів міастенії (QMGS) та клінічної класифікації Myathenia Gravis Foundation of America (MGFA), 2001. Оцінка нервово-м’язової передачі проводилася на підставі аналізу результатів декремент-тесту та амплітуди негативної фази М-відповіді при низькочастотній непрямій супрамаксимальній стимуляції m. abductor digiti minimi та m. orbicularis oculi. Результати та висновки. Зміни структури тимуса негативно впливають на перебіг міастенії, про що свідчать показники за шкалою QMGS та класифікацією MGFA. Найбільша тяжкість захворювання (з переважанням ураження бульбарної групи м’язів) поєднувалася із наявністю у хворих тимоми, а найменша відзначалась у хворих з нормальною структурою тимуса. Переважання пацієнтів жіночої статі в групі хворих з міастенією на тлі гіперплазії тимуса може свідчити про вплив гормонального статусу як на структуру вилочкової залози з розвитком її гіперплазії, так і на перебіг міастенії, що потребує подальшого вивчення. Простежена пряма залежність між структурними змінами в тимусі та зниженням амплітуди негативної фази М-відповіді, що свідчить про негативний вплив морфологічних порушень тимуса на нервово-м’язову передачу імпульсів. Значне зниження амплітуди негативної фази М-відповіді на фоні високих показників декремент-тесту можна вважати маркером тяжкого перебігу захворювання.
Aim: The purpose of this article is to determine the role of rehabilitation in the structure of the treatment algorithm for patients with myasthenia gravis. materials and methods: All patients admitted to the SI «Zaycev V.T. Institute of General and Urgent surgery of National Academy of Medical Sciences of Ukraine», Kharkiv, Ukraine for surgical treatment for thymoma or carcinoma of the thymus gland. 102 people aged 18 to 69 with myasthenia have been comprehensively surveyed and their data have been analyzed and studied. Diagnosis of myasthenia was established according to the tenth edition of the International Classification of Diseases ICD-10 (WHO, 1992). Results: The severity of the patients’ condition corresponded to grade IIIA in 5 patients (16.7%), IIIB grade – 14 patients (46.7%), IVA grade – 7 patients (23.3%), IVB grade – in 4 patients (13.3%). According to the clinical classification of MGFA, the severity of the condition in most patients in this group corresponded to Class IIB (32.4%) and IIIA class (35.2%). Conclusions: The results of our study suggest that clinical and anamnestic features of myasthenia in absence of structural thymus disorders are debut before the age of 40 years, predominant affection of skeletal muscles, mild course (severity of the disease corresponds to 12.7 ± 1.76 points on the QMGS scale) and characterized by a debut after 40 years regardless of sex, manifestation of generalized muscle weakness and severe course (in 68.4% of cases severity of the disease was 31.68 ± 3.76 points on the QMGS scale).
The aim of the study is to identify the peculiarities of local immune reactions in the skin with underlying soft tissues in patients with different variants of the multiple sclerosis’ course. Material and methods: The study included 35 patients, hospitalized in the neurological department of the Communal Nonprofit Enterprise of Kharkiv Regional Council «Regional Clinical Hospital» with the established diagnosis of multiple sclerosis. The patients were divided into three study groups, based on different variants of this pathology’s course. Group 1 included 16 patients with relapsing-remitting type of multiple sclerosis. Group 2 included 11 patients with a secondary-progressive type of multiple sclerosis course. Group 3 included 8 patients with a primary progressive type of multiple sclerosis. Patients of all groups underwent a biopsy of the skin with underlying soft tissues in the lower third of the inner surface of the right lower leg. The comparison group (group 4) was represented by 10 autopsy cases (7 women and 3 men) conducted on the basis of the pathological anatomy department of the Communal Nonprofit Enterprise of Kharkiv Regional Council «Regional Clinical Hospital». There were no signs of the nervous system’s pathology during life in all cases of this group. The cause of death was a dislocation of the brain stem or hematocephaly and the main disease was arteriovenous malformation or congenital aneurysm of the cerebral vessels. The material for the morphological study was skin with underlying soft tissues. Microspecimens stained with histological and immunohistochemical methods were studied, using an Olympus BX-41 microscope. The obtained data were statistically processed, using Statistica 6.0 and Microsoft Excel 2003 programs. Results: Survey microscopy showed that in groups 1-3 in comparison with group 4 immune cell infiltrations were more pronounced in the skin with underlying hypodermis. Significantly larger mean values of the absolute number of CD 3-, CD 20- and CD 68-positive cells were revealed immunohistochemically in groups 1-3 compared with group 4. Thus, it was found in patients with multiple sclerosis the activation of T-cell immunity, B-cell immunity and macrophage system with the development of an immune imbalance between them. Our results allow us to think about the participation of all the above immune cells in the pathogenesis of multiple sclerosis development. The revealed disorders of local immune reactions in the skin with underlying hypodermis in patients with multiple sclerosis are less pronounced in the remitting-recurrent variant of the course of the disease, more pronounced in the secondary-progressing and, especially, primary-progressing variants. Conclusions: In patients with multiple sclerosis in the skin with underlying hypodermis activation of T-cell immunity, B-cell immunity and the macrophage system is observed with the development of an immune imbalance between them, characterized by the prevalence of the absolute number of macrophages among all immune cells. Less pronounced violations of local immune reactions in the skin with underlying hypodermis are noted in remitting-relapsing variant of multiple sclerosis course, more pronounced in a secondary-progressing and, especially, primary-progressing variants.
Thymoma is characterized by various clinical manifestations. About half of them are manifested as an asymptomatic course, others occur in a combination with different syndromes. 25% of patients develop compression of tumor blood vessels, nerves and organs of the mediastinum or clinical signs of germination of thymic tumor in other organs. 40% of patients are diagnosed with autoimmune diseases due to dysfunction of the thymus. Generalized myasthenia gravis (75%) is most frequently observed.The objective of the research was to study clinical and immunological peculiarities of myasthenia gravis depending on the size and histological type of thymoma.Materials and methods. The results of clinical and immunological, instrumental and histological examination of 30 patients with myasthenia on the background of thymoma were analyzed. The severity of the disease was assessed using clinical classification of the Myasthenia Gravis Foundation of America (MGFA, 2001).To determine the barrier function of phagocytic cells, phagocytosis activity of neutrophils was evaluated using the light microscope. The phagocytic index, the phagocytic number and the index of phagocytosis completion were determined. The suspension culture of Saccharomyces cerevisiae was used as a microbial agent. Preparations were stained using the Romanovsky-Himze methods. Neutrophilic leukocytes were separated from leukocyte suspension of peripheral blood. The expression of differentiation clusters CD3+, CD4+ and CD8+ on subpopulation of T- and B-cells were evaluated by indirect ELISA using monoclonal antibodies labeled with FITC-dye. To diagnose thymoma, we used spiral CT “Marconi” SeleCT/SP. For histologic study thymoma samples were fixed in 10% neutral formalin for 24 hours. The material was embedded in paraffin after posting through the chloroform in the usual way; then, sections with the thickness of 5-7 μm were prepared. Preparations were stained with hematoxylin and eosin.Results and conclusions. The severity of the clinical course of myasthenic syndrome on the background of thymoma does not depend on tumor size; it depends on the histologic type and immunological imbalance. The severest clinical picture was observed in patients with type AB thymoma and the least severe course of myasthenic symptoms was found in patients with lymphoid thymoma (type B1). Lymphoepithelial and epithelial thymomas were accompanied by similar immunological disorders. The reduction in the levels of CD3+ and CD4+ lymphocyte subpopulations can be used as a reliable diagnostic criterion. Lymphoid thymomas are characterized by a significant reduction in the indicators of the phagocytic index, the phagocytic number, and the level of CD8+ lymphocyte subpopulations as well as an increase in the level of CD4+ subpopulations. Surgical treatment as a method of choice in case of radical immunosuppression is indicated for all patients with confirmed thymoma regardless of its size and histological characteristics.
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