ADVANCED THERAPY IN PATIENTS WITH PRIMARY AND POSTPOLYCYTHEMIC MYELOFIBROSIS

Ph-negative myeloproliferative neoplasm are the group of hematologic disorders which includes primary myelofibrosis, polycythemia vera, essential trombocytemia and several rare diseases. After the discovery of V617 Fgain-of-function mutation the new period of diagnostics, treatment and evaluating of MPN prognosis began. At the current moment several molecules inhibiting JAK2 function are developed. Advanced therapy in patients with primary and post-polycythemic myelofibrosis included molecules inhibiting JAK2 function resulted in rapid and durable improvements in splenomegaly and disease-related symptoms in the phase 3 trials COMFORT-I and COMFORT-II. The effectiveness of the advanced therapy included molecule inhibiting JAK2 was evaluated in three patients with primary myelofibrosis and post-polycitemic myelofibrosis. All represented clinical cases demonstrated positive dynamics of the disease manifested in spleen size reduction, improvement of the symptoms and in one case in reduction of blood transfusions. None of three patients met serious adverse events leading to dose reduction or discontinuation of the molecule inhibiting JAK2. Target agents therapy demonstrated high treatment rates in patients with primary and post-polycythemic myelofibrosis. Thus, it is clearly necessary to perform molecular diagnosis, screening tests at early stages of the chronic myeloproliferative disease for the selection of patients in need for specific treatment

THE FUNCTIONAL STATE OF VISUAL ANALYZER IN PATIENTS WITH CHRONIC VERTEBROBASILAR INSUFFICIENCY

All patients had various stages of fundus changes typical for the arterial hypertension, a significant reduction in the flicker fusion frequency. When an overall insignificant decrease in ERG amplitude and prolongation of the latency time was determined of a- and. b-waves compared with the control group, decreased the amplitude of oscillatory potentials. Indices of the lability and. sensitivity of the optic nerve were within normal limits. The use new electrophysiological diagnostic methods could be perspective for identification and. assessment of the degree of pathological changes in the visual analyzer at an early sub-clinical level of disease in violation of circulation in the vertebral-basilar basin

Особенности состояния мягких тканей на вершине деформации у больных кифосколиозом на фоне нейрофиброматоза 1-го типа

Objective. Evaluation of skin sensitivity and analysis of morphological changes in paravertebral muscles and back skin in kyphoscoliotic deformity projection in patients with type 1 neurofibromatosis (NF-1).Materials and methods. Ten NF-1 patients who underwent surgery to treat kyphoscoliosis were examined. Using an electrical esthesiometer thermal pain sensitivity before the surgery was studied in dermatomes corresponding to the apex of the deformity. Skin and muscle biopsy samples were collected intraoperatively in the projection of the apex of the deformity curve and were subsequently analyzed by light and scanning electron microscopy.Results. Patients with kyphoscoliosis with underlying NF-1 were characterized by abnormal thermal pain sensitivity, pathological structural changes in skin and muscles accompanied by disrupted innervation and blood supply.Discussion. The observed changes may be responsible for lowered postoperative reparative potential of tissues and they must be considered in prevention and prognosis of treatment and rehabilitation efficacy.Цель работы. Оценка кожной чувствительности и анализ морфологических изменений паравертебральных мышц и кожи спины в проекции вершины кифосколиотической деформации у пациентов с нейрофиброматозом 1-го типа (НФ-1).Материалы и методы. Обследованы 10 больных НФ-1, которым проводилось хирургическое лечение по поводу кифосколиоза. Температурно-болевую чувствительность до операции исследовали с использованием электрического эстезиометра в дерматомах, соответствующих вершине деформации. Биоптаты кожи и мышцы забирали интраоперационно в проекции вершины дуги деформации позвоночника с последующим гистологическим анализом с помощью световой и сканирующей электронной микроскопии.Результаты. У больных кифосколиозом на фоне НФ-1 были выявлены нарушения температурно-болевой чувствительности, патологические структурные изменения кожи и мышц, нарушения их полноценной иннервации и кровоснабжения.Обсуждение. Обнаруженные изменения могут обусловливать снижение репаративного постоперационного потенциала тканей, что необходимо учитывать в профилактике и прогнозе эффективности лечения и реабилитации

Aspects of Surgical Treatment of Spinal Pathology in the Patient with Gurler Syndrome

The clinical case of surgical treatment of combined spinal pathology in the child with Gurler syndrome is described in the article. Indications, major risk factors and aspects of surgical treatment of spinal pathology at this nosology are described in detail; all the approaches are analyzed according to the literature. Background. We would like to draw the attention of our colleagues to this problem with this clinical case. We want to emphasize the peculiarities of orthopedic manifestations in patients with mucopolysaccharidosis type IH (MPS IH). The most crucial is timely detection and surgical treatment. Clinical case description. This article describes the aspects of surgical treatment of spinal pathology in the patient with MPS IH: progressive kyphotic deformation, stenosis and craniovertebral junction instability, significant neurological deficit, internal organs functional disorders. Conclusion. Patients with MPS are characterized by multilevel orthopedic pathology which requires continuous follow-up throughout the life by single multidisciplinary team of specialists

Изменчивость морфологической структуры растений в ценопопуляциях Tulipa biebersteiniana в заповеднике «Черные земли»

The article presents the observation of four Tulipa biebersteiniana (Tulipa biebersteiniana Schult. et Schult.) cenopopulations in the State Biosphere Nature Reserve “Black Soil” conducted in 2013–2015. The obtained data demonstrate the difference between the cenopopulations in the steppe and the desert areas of the reserve in terms of biomorphological plant features. It was revealed that the variability of biomorphological features of plants in the species populations correlates with climatic conditions of each particular year. The data obtained can be used in the organization of Tulipa biebersteiniana preservation.В статье приводятся данные наблюдений четырех ценопопуляций тюльпана Биберштейна (Tulipa biеbersteiniana Schult. et Schult.) в государственном природном биосферном заповеднике «Черные земли» в период с 2013 по 2015 гг. Получены данные о различии ценопопуляций, приуроченных к степному и пустынному участкам заповедника, по биоморфологическим признакам растений. Выявлено, что на изменчивость морфологических признаков растений в ценопопуляциях данного вида особенно влияют климатические условия года вегетации. Полученные данные могут быть использованы при организации охраны тюльпана Биберштейна

Surgical treatment of children with non-traumatic old atlanto-axial rotatory fixation

Atlanto-axial rotatory fixation (AORF) develops on the background of acute torticollis. Widely adopted terms such as C1 subluxation or atlantooccipital rotational subluxation do not reflect the core of this pathology and carry negative weight in the diagnostics and treatment of AORF. Retrospective analysis of the diagnostics and treatment outcome of 5 children with confirmed AORF diagnosis and literature review were performed. Clinical method, radiography and functional computer tomography were used to verify the diagnosis. De-rotational halo-traction and open correction with screw fixation were applied for treatment. Head position was managed to be improved in all patients. In one case the reduction was performed using correction in suboccipital segments and in other 4 cases the correction and fixation by Harms and de-rotational halo-traction allowed to correct torticollis. The pain syndrome had been arrested completely. Disease outcome resulted in formation of C1-C2 fibrous or bone fusion regardless the method of treatment. The patients with neglected AORF represent a great challenge for diagnostics and treatment. When conservative treatment fails it is necessary to involve de-rotational halo-traction with possible application of open reduction and posterior fusion. The purpose of treatment is to eliminate torticollis and pain using creation of proper C1-C2 alignment. The motions in atlantooccipital joint do not restore due to formation of the fibrous or bone fusion