Inactivation of α-ketoglutarate dehydrogenase during oxidative decarboxylation of α-ketoadipic acid

Abstractα-Ketoglutarate dehydrogenase was inactivated irreversibly and completely during oxidation of α-ketoadipic acid. The inactivation was revealed both in the model system with ferricyanide and in the overall reaction catalyzed by the α-ketoglutarate dehydrogenase complex. Neither substrate depletion nor product accumulation induced the inactivation. The results obtained were compared with recent data on the enzyme inactivation during oxidation of α-ketoglutaric acid. The differences in the inactivation kinetics observed with the two substrates of the enzyme were analyzed. They seem not to reflect the different mechanisms of the inactivation, but, rather, depend on the changes in the rates of the individual stages of the process

ПРИМЕНЕНИЕ КОМПЛЕКСА «ИНТЕРФЕЙС “МОЗГ – КОМПЬЮТЕР” И ЭКЗОСКЕЛЕТ» И ТЕХНИКИ ВООБРАЖЕНИЯ ДВИЖЕНИЯ ДЛЯ РЕАБИЛИТАЦИИ ПОСЛЕ ИНСУЛЬТА

Background: Efficacy of physical exercise and movement imagination for restoration of motor dysfunction after a stroke is seen as proven. However, the use of movement imagination is complicated by impossibility of objective and subjective control over  the exercise, as well as by the absence of their motor support. The brain-computer interface based on electroencephalography is a technique that enables a feedback during movement imagination.Materials and methods: We assessed 10 patients (6 men and 4 women) aged from 30 to 66 years (mean age, 47 ± 7.7 years) with an ischemic (n = 9) and hemorrhagic (n = 1) stroke during the last 2 months to 4 years. Online recognition of movement imagination was done by a classifier with a brain computer interface. An exo-skeleton supported passive movements in a paretic hand managed by the brain-computer interface. During 2 weeks the patients had 10 sessions of 45–90 minute duration each. For control, we used data from 5 stroke patients who, in addition to their standard treatment, underwent an imitation of rehabilitation procedures without movement imagination and feedback. To assess efficacy of treatment, we used a modified Ashworth scale, Fugl-Meyer scale, test for evaluation of hand functions ARAT, British scale for assessment of muscle force MRC-SS. Level of everyday activity and working ability was measured with a modified Rankin scale and Bartel index. Cognitive functions were assessed with Schulte tables.Results: Online recognition of movement imagination according to desynchronization of μ rhythm was registered in 50–75% of patients. All patients reported a subjective improvement of motor functions and working ability. Positive results for at least one parameter were observed in all patients; however, there were no significant difference between the parameters before and after rehabilitation procedures, excluding cognitive functions (degree of warming-up, p 0.02).Conclusion: In post stroke patients, the use of movement imagination, brain-computer interface and exo-skeleton does not seem to affect the rehabilitation process negatively. In all cases, some positive results were achieved in motor recovery, as well as in working ability and daily activity. The results of the rehabilitation procedure are promising; however, the study should be continued.Актуальность. Эффективность физических упражнений и воображения движений для восстановления двигательных нарушений после инсульта считается доказанной. Однако применение воображения движений осложняется невозможностью объективного и субъективного контроля за выполнением упражнений, а также отсутствием их двигательного подкрепления. Интерфейс «мозг – компьютер» на основе электроэнцефалограммы – метод, позволяющий осуществлять обратную связь при выполнении воображения движений.Материал и методы. Обследованы 10 пациентов (6 мужчин и 4 женщины) в возрасте от 30 до 66 лет (средний возраст 47 ± 7,7 года), перенесших ишемический (n = 9) и геморрагический (n = 1) инсульт в срок от 2 месяцев до 4 лет. Онлайн-распознавание воображения движений осуществлялось классификатором с помощью интерфейса «мозг – компьютер». Экзоскелет осуществлял пассивное движение в паретичной кисти под управлением интерфейса «мозг – компьютер». Пациенты получали по 10 занятий длительностью 45–90 минут в течение 2 недель. В качестве контроля использовали данные 5 пациентов, перенесших инсульт, которым в дополнение к стандартной терапии проводилась имитация реабилитационной процедуры без воображения движения и обратной связи. Для оценки эффективности проводимых мероприятий использовали модифицированную шкалу Ашворта, шкалу Fugl-Meyer, тест исследования функций руки ARAT, Британскую шкалу оценки мышечной силы MRC-SS; уровень дееспособности и повседневной активности определяли при помощи модифицированной шкалы Рэнкина и индекса Бартел; когнитивные функции исследовали с использованием таблиц Шульте.Результаты. Онлайн-распознавание воображения движений по реакции десинхронизации μ-ритма зарегистрировано у пациентов в 50–75%. Субъективно все пациенты отметили улучшение двигательных функций и дееспособности. Положительный результат по данным одного и более показателей был отмечен у всех пациентов, однако статистически значимого различияпоказателей до и после проведения реабилитационных мероприятий не получено за исключением когнитивной сферы (степень врабатываемости, p 0,02).Заключение. У пациентов, перенесших инсульт, процедура с использованием воображения движений, интерфейса «мозг – компьютер» и экзоскелета не оказывала отрицательного влияния на процесс реабилитации. Во всех наблюдениях был достигнут положительный результат как в отношении восстановления движений, так и дееспособности и повседневной активности. Результат применения реабилитационной процедуры перспективен, однако следует продолжить исследование.

Issues of diagnosis and management of patients with neuroendocrine cell hyperplasia of infancy

Neuroendocrine hyperplasia of infancy (NEHI) is an interstitial lung disease (ILD) of unknown etiology, which develops in infancy and manifests as persistent tachypnea. Objective of the research: to summarize results of observations of patients with NEHI, including during the pandemic of a new coronavirus infectious disease (COVID-19). Materials and methods: clinical and follow-up observation, laboratory and instrumental examination, including lung biopsy, 52 children with NEHI aged from 21 days to 10 years. Results: NEHI more often affects boys in the first 6 months of life; can be diagnosed on the basis of pediatric ILD-syndrome detection; specific CT signs, lung biopsy. It is characterized by undulating course with increased respiratory failurec aused by acute respiratory viral infections, COVID-19; half of the patients need oxygen therapy. © 2020, Pediatria Ltd.. All rights reserved

The structure of interstitial lung diseases in children of the first two years of life

For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. © 2015, Pediatria Ltd. All rights reserved

The structure of interstitial lung diseases in children of the first two years of life

For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. © 2015, Pediatria Ltd. All rights reserved