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YMER Digital ◽  
2022 ◽  
Vol 21 (01) ◽  
pp. 56-62
Author(s):  
Dr. Nandita Bhalla ◽  
◽  
Dr. Shrikiran Aroor ◽  

Posterior reversible encephalopathy syndrome is an acute neurological illness presenting with clinical symptoms and distinctive MRI findings. Symptoms include headaches, seizures, altered consciousness as well as visual impairment. PRES is always accompanied by peculiar radiological findings of edematous change affecting the rear cerebral area. It commonly occurs in settings where patients are undergoing hypertensive crisis, or there is the use of steroids, calcineurin inhibitors, in the nephritic state or end-stage renal disease. The management includes treating the underlying cause and symptomatic therapy. However, due to relatively fewer pediatric reports, its management isn’t specific and rather based on experience. Our patient is a 3-year-old male, who presented with hypertensive crisis and MRI findings confirmed it to be a case of PRES. He was managed with a combined regime of antihypertensive and steroids which lead to complete neurological recovery and resolution of PRES. There are a scarce number of case reports on the use of steroids for the treatment of vasogenic oedema in children.


PLoS ONE ◽  
2021 ◽  
Vol 16 (12) ◽  
pp. e0261410
Author(s):  
Elena L. Amelina ◽  
Stanislav A. Krasovsky ◽  
Nina E. Akhtyamova-Givirovskaya ◽  
Nataliya Yu. Kashirskaya ◽  
Diana I. Abdulganieva ◽  
...  

Background Patients with cystic fibrosis (CF) need costly medical care and adequate therapy with expensive medicinal products. Tigerase® is the first biosimilar of dornase alfa, developed by the lead Russian biotechnology company GENERIUM. The aim of the manuscript to present post hoc sub-analysis of patients’ data with cystic fibrosis and severe pulmonary impairment of a larger comparative study (phase III open label, prospective, multi-centre, randomized study (NCT04468100)) of a generic version of recombinant human DNase Tigerase® to the only comparable drug, Pulmozyme® Methods In the analyses included subgroup of 46 severe pulmonary impairment patients with baseline FEV1 level 40–60% of predicted (23 patients in each treatment group) out of 100 patients registered in the study phase III open label, prospective, multi-center, randomized study (NCT04468100), and compared efficacy endpoints (FEV1, FVC, number and time of exacerbations, body weight, St.George’s Respiratory Questionnaire) as well as safety parameters (AEs, SAEs, anti-drug antibody) within 24 treatment weeks. Results All outcomes were comparable among the studied groups. In the efficacy dataset, the similar mean FEV1 and mean FVC changes for 24 weeks of both treatment groups were observed. The groups were also comparable in safety, all the secondary efficacy parameters and immunogenicity. Conclusions The findings from this study support the clinical Tigerase® biosimilarity to Pulmozyme® administered in CF patients with severe impairment of pulmonary function.


Author(s):  
Valeriya A Lemeshko ◽  
Svetlana S Ratushnyak ◽  
Filipp V Gorkavenko ◽  
Evgeniya V Nazarova ◽  
Natalia I Ilina ◽  
...  

AIMS: to evaluate the cost-effectiveness of sASIT in children and adult patients with allergic rhinitis and / or allergic rhinoconjunctivitis. MATERIALS AND METHODS: the hypothesis of the study was based on results of the study by Devillier P. et al., 2019, according to which the incidence of asthma was 13.7% and 17.0% in the sASIT + symptomatic therapy groups and the symptomatic therapy group (odds ratio - 0.776, 95% confidence interval (0.622; 0.968)). Pharmacoeconomic study based on decision tree model. Costs taken into account: cost of sASIT, symptomatic therapy, diagnostics and routine follow-up visits due to BA, outpatient BA drug therapy, the cost of hospitalization due to BA. The modeling horizon was 5 years, including 2 years of sASIT therapy and 3 years of follow-up. RESULTS: the cost per patient when using sASIT in combination with symptomatic therapy was 166,711.93 rubles, with symptomatic therapy - 101,700.35 rubles. The CER for sASIT in combination with symptomatic therapy was 193,177.20 rubles. per 1 prevented case of asthma, for symptomatic therapy - 122,530.55 rubles. for 1 prevented case of BA. Thus, the cost of 1 averted AB case when using sASIT in combination with symptomatic therapy is 57.7% higher than with symptomatic therapy. According to the results of the cost-benefit analysis, ICUR for an additional year of life adjusted for its quality (QALY) when performing sASIT in combination with symptomatic therapy compared with symptomatic therapy alone in children and adults was 567,365.48 rubles, which is less than calculated willingness to pay threshold (WTPT) (RUB 2,248,898.50). CONCLUSIONS: based on the results of comparing the cost of 1 added QALY and WTPT, it can be concluded that sASIT in combination with symptomatic therapy compared to symptomatic therapy alone is potentially cost-effective in children and adults with AR.


Vestnik ◽  
2021 ◽  
pp. 367-371
Author(s):  
Б.У. Шалекенов ◽  
Е.А. Куандыков

Мочекаменная болезнь является одной из главных проблем современной урологии как наиболее часто встречающаяся патология органов мочевой системы и составляющая 30 - 50% всех больных урологических стационаров. Целью настоящего исследования явилось изучение эффективности применения препарата «Фитолизин» у больных мочекаменной болезнью. В основу исследования положены результаты лечения 160 (85 женщин и 75 мужчин) пациентов, находившихся на стационарном лечение, средний возраст больных составил 42,2±12,4 года, длительность заболевания от 1-3 до 72 часов, размер конкрементов, в том числе и дезинтегрированных фрагментов после дистанционной ударно-волновой литотрипсии и контактно-лазерная литотрипсия варьировал от 0,4 до 0,8 см. Все больные были разделены на две группы. В основную группу вошли 70 больных, получавших помимо традиционной симптоматической терапии (спазмолитики и водная нагрузка) «Фитолизин» по 43 мл 3 раза в день. В контрольной группе (90 больных) проводилась только традиционная терапия. При включение в терапию препарата «Фитолизин» самостоятельное отхождение отмечено у 64(91,4%) больных, частота возникновения почечной колики имело место у 7(10%), лейкоцитурия - у 14(20%), бактериурия - у 10(15%). Применение фитопрепарата «Фитолизин» в составе комплексной терапии, является эффективным методом консервативного лечения больных мочекаменной болезнью и после после дистанционной ударно-волновой литотрипсии и контактно-лазерная литотрипсия в 90,9% случаев в отношении самостоятельного отхождения конкрементов. Urolithiasis is one of the main problems of modern urology as the most common pathology of the urinary system and makes up 30 - 50% of all patients in urological hospitals. The purpose of this study was to study the effectiveness of the use of the drug "Phytolysin" in patients with urolithiasis. The study is based on the treatment results of 160 (85 women and 75 men) patients who were hospitalized, the average age of the patients was 42.2 ± 12.4 years, the duration of the disease from 1-3 to 72 hours, the size of the stones and disintegrated fragments after remote shock wave lithotripsy and contact laser lithotripsy ranged from 0.4 to 0.8 cm. All patients were divided into two groups. The main group included 70 patients who received in addition to the traditional symptomatic therapy (antispasmodics and water load) "Phytolysin" 43 ml 3 times a day. In the control group (90 patients), only traditional therapy was performed. When "Phytolysin" was included in the therapy, independent discharge was observed in 64 (91.4%) patients, the incidence of renal colic occurred in 7 (10%), leukocyturia in 14 (20%), bacteriuria in 10 (15%). The use of the phytopreparation "Phytolysin" as a part of complex therapy is an effective method of conservative treatment of patients with urolithiasis and after remote shock-wave lithotripsy and contact laser lithotripsy in 90.9% of cases with regard to self-discharge of calculi.


Vestnik ◽  
2021 ◽  
pp. 143-146
Author(s):  
Б.Г. Султанова ◽  
С.Б. Бодесова ◽  
А.Т. Ибрашева ◽  
Б.С. Мусабаев ◽  
Д.Ш. Бетирова ◽  
...  

В статье описан «неклассический» случай, редко встречающаяся форма заболевания системной красной волчанкой без типичного поражения кожи с проявлением быстропрогрессирующего гломерулонефрита, с поражением тазобедренного сустава, выраженным болевым синдромом у юноши. С применением новых инновационных методов диагностики (непрямая иммунофлюоресценция на анализаторе AKLIDES), что позволило провести своевременно комплексную терапию включая в себя патогенетическую, эфферентную (гемодиализ, плазмаферез), тем самым получен хороший клинический эффект. This article describes a non- racial case of systemic lupus erythematosus with the manifestation of a rapidly progressive glomerulonephritis, hip joint lesion, a pronounced painful syndrom, without dermal manifestations. By the use of new diagnostic methods (immunofluorescence), timely complex therapy: pathogenetic, efferent therapy, symptomatic therapy, it is possible to obtain a fairly good clinical - laboratory- instrumental result.


2021 ◽  
Vol 40 (4) ◽  
pp. 51-58
Author(s):  
Andrey Yu. Emelin

GuillainBarr syndrome is an acute, rapidly progressive immune-mediated disease of the peripheral nervous system, combining several variants and subtypes with various clinical, pathophysiological and electrophysiological signs. GuillainBarr syndrome usually develops 13 weeks after the viral or bacterial infection, which acts as the trigger triggering autoimmune mechanisms, leading to demyelination and axonal damage. The disease is getting more acute due to the emergence of a new coronavirus infection. Behind the diseases dipathophysiology there is the activation of cellular and humoral immunity with the production of autoantibodies to specific gangliosides and glycolipids and the formation of circulating immune complexes that attack peripheral nerves and roots (the phenomenon of molecular mimicry). The examination of patients requires an integrated approach, including, along with clinical and anamnestic data, the results of laboratory and neurophysiological examination. The treatment of patients with GuillainBarr syndrome is carried out in an intensive care unit and includes both pathogenetic therapy and nonspecific measures aimed to correct dysfunctions of vital organs, prevent complications and provide symptomatic therapy. Currently, the main direction of pathogenetic therapy of this disease is the use of high-dose intravenous immunotherapy with human normal immunoglobulin preparations or high-volume therapeutic plasmapheresis. Taking into account the absence of data about differences in the effectiveness of these methods, the choice of direction is determined taking into account the contraindications and possible development of adverse events, as well as the capabilities of the medical institution (2 tables, bibliography: 15 refs)


Author(s):  
Dominik Schüttler ◽  
Konstantinos Mourouzis ◽  
Christoph J Auernhammer ◽  
Konstantinos D Rizas

Abstract Background Neuroendocrine tumors (NETs) can affect the cardiopulmonary system causing carcinoid heart disease and valve destruction. Persistent foramen ovale (PFO) occlusion is indicated in patients with carcinoid heart disease and shunt-related left-heart valve involvement. Case Summary We report the case of a 54-year-old female patient with metastatic NET originating from the small bowel. The patient was on medication with octreotide and telotristat. One year after diagnosis, cardiac involvement of carcinoid developed with regurgitation of right-sided and, due to PFO, left-sided heart valves. Closure of PFO was performed (Occlutech 16/18 mm). One year later she presented with recurrent severe dyspnoea. The PFO-occluder was in situ without residual shunt. Valvular heart disease, including left-sided disease, and metastatic spread of NET were stable. Blood gas analysis revealed arterial hypoxemia (pO2 = 44 mmHg/5.87 kPa), which was related to extensive intrapulmonary shunting (31% shunt fraction) confirmed using contrast-enhanced echocardiography. The patient was prescribed long-term oxygen supplementation as symptomatic therapy and anti-tumoral therapy was intensified with selective internal radiotherapy of the liver metastases in order to improve biochemical control of the carcinoid syndrome. Discussion An echocardiographic assessment of the presence of a PFO is recommended in patients with NET as PFO closure minimizes the risk of left-sided carcinoid valve disease. Deterioration of symptomatic status in metastasized NET might also be due to a hepatopulmonary-like physiology with intrapulmonary shunting and arterial desaturation thought to be caused by vasoactive substances secreted by the tumor. This is a rare case describing the development of this syndrome after PFO closure.


Author(s):  
Л.А. Юсупова ◽  
З.Ш. Гараева ◽  
Е.И. Юнусова ◽  
Г.И. Мавлютова ◽  
А.Р. Галимова

В статье освещены сведения о кератодермиях – гетерогенной группе состояний, характеризующихся аномальным утолщением кожи ладоней и подошв. Традиционно выделяют приобретенные и наследственные формы. В клинической практике наиболее часто встречается гиперкератоз ладоней и подошв как одно из проявлений псориаза, экземы, дерматомикозов и многих других заболеваний. К развитию гиперкератоза ладоней и подошв могут также привести механические и токсические факторы (в том числе прием лекарственных препаратов), поступление с пищей токсических веществ, приводящих к изменениям слизистой кишечника, современные требования моды и красоты могут способствовать развитию множественного дефицита витаминов. Значительно реже встречаются наследственные формы кератодермий, являющиеся самостоятельными заболеваниями. Раннее начало и семейный анамнез предполагают генетическую природу кератодермии. Отличительными особенностями наследственных форм служат характер наследования, степень поражения эпидермиса, наличие/отсутствие распространения очагов за пределы кожи ладоней и подошв, сопутствующая патология. В основе развития наследственных форм лежат мутации различных генов, кодирующих белки (например, кератин, десмосомы, лорикрин, катепсин С, белки щелевых контактов), которые принимают участие в процессе кератинизации. Наследственные ладонно-подошвенные кератодермии имеют большую генетическую и фенотипическую неоднородность, вследствие чего постановка точного диагноза на основе одних лишь клинических проявлений, когда нет возможности выполнить молекулярно-генетическое исследование, является весьма сложной задачей. Благодаря секвенированию нового поколения был достигнут значительный прогресс в расшифровке генетической основы кератодермий. В данном обзоре рассмотрены патогенетические, клинические, диагностические особенности диффузных форм кератодермий, варианты симптоматической терапии, учитывая торпидность и резистентность патологического процесса. The article covers information about keratodermia, a heterogeneous group of conditions characterized by abnormal thickening of the skin of the palms and soles. Traditionally, acquired and hereditary forms are distinguished. In clinical practice, the most common hyperkeratosis of the palms and soles, as one of the manifestations of psoriasis, eczema, dermatomycosis and many other diseases. Mechanical and toxic factors (including taking medications), intake of toxic substances with food that lead to changes in the intestinal mucosa can also lead to the development of hyperkeratosis of the palms and soles, modern fashion and beauty requirements can contribute to the development of multiple vitamin deficiencies. Much less common are hereditary forms of keratoderma, which are independent diseases. Early onset, the presence of a positive family history suggest a genetic nature. The distinctive features of hereditary forms are the nature of inheritance, the degree of damage to the epidermis, the presence/absence of the spread of foci beyond the skin of the palms and soles, and concomitant pathology. The development of hereditary forms is based on mutations of various genes encoding proteins (for example, keratin, desmosomes, loricrin, cathepsin C, gap junction proteins), which are involved in the process of keratinization. Hereditary palmoplantar keratoderma has a large genetic and phenotypic heterogeneity, as a result of which making an accurate diagnosis based on clinical manifestations alone, when it is not possible to perform molecular genetic research, is a very difficult task. Thanks to the next-generation sequencing, significant progress has been made in deciphering the genetic basis of keratoderms. This review examines the pathogenetic, clinical, and diagnostic features of diffuse forms of keratoderma, and options for symptomatic therapy, taking into account the torpidity and resistance of the pathological process.


2021 ◽  
Vol 42 (06) ◽  
pp. 737-746
Author(s):  
Susanna Esposito ◽  
Luciana Abate ◽  
Serena Rosa Laudisio ◽  
Andrea Ciuni ◽  
Simone Cella ◽  
...  

AbstractIn December 2019, a new infectious disease called coronavirus disease 2019 (COVID-19) attributed to the new virus named severe scute respiratory syndrome coronavirus 2 (SARS-CoV-2) was detected. The gold standard for the diagnosis of SARS-CoV-2 infection is the viral identification in nasopharyngeal swab by real-time polymerase chain reaction. Few data on the role of imaging are available in the pediatric population. Similarly, considering that symptomatic therapy is adequate in most of the pediatric patients with COVID-19, few pediatric pharmacological studies are available. The main aim of this review is to describe and discuss the scientific literature on various imaging approaches and therapeutic management in children and adolescents affected by COVID-19. Clinical manifestations of COVID-19 are less severe in children than in adults and as a consequence the radiologic findings are less marked. If imaging is needed, chest radiography is the first imaging modality of choice in the presence of moderate-to-severe symptoms. Regarding therapy, acetaminophen or ibuprofen are appropriate for the vast majority of pediatric patients. Other drugs should be prescribed following an appropriate individualized approach. Due to the characteristics of COVID-19 in pediatric age, the importance of strengthening the network between hospital and territorial pediatrics for an appropriate diagnosis and therapeutic management represents a priority.


2021 ◽  
Author(s):  
Rot Sergej ◽  
Goelz Leonie ◽  
Arndt Holger ◽  
Gutowski Pawel ◽  
Meier Ullrich ◽  
...  

Abstract Background Mechanical obstruction of ventriculoperitoneal shunt (VPS) during the first year after shunt implantation is a common complication and is widely described in the literature. In this paper, we evaluated the suitability of the shuntography for the diagnosis of mechanical complications of the VPS in patients with idiopathic normal pressure hydrocephalus (iNPH). Methods We retrospectively identified 49 patients with pathologic shuntography over of a period of 20 years in our hospital. The percentage of procedure-associated complications was determined. Results Ninety-eight percent (n = 48) of the patients who underwent shuntography showed clinical and radiographic signs of underdrainage prior to examination. Shuntography revealed mechanical complications of the VP shunt in 37% (n = 18) as a cause of clinical deterioration and following revision operation. During shuntography, mechanical obstruction was discovered in 78% (n = 14) and disconnection of shunt components in 22% (n = 4). In the obstruction group, in 50% (n = 7) the closure was detected in the ventricular catheter, in 29% (n = 4) in the distal catheter of the VPS, and in 21% (n = 3) in both sides of the VPS. In the case of an inconspicuous shuntography (63%, n = 31), the patients received symptomatic therapy (32%, n = 10) or re-adjustment of the valve setting (68%, n = 21). Fifty-seven percent of the patients who underwent surgical treatment improved clinically by at least one point according to the Kiefer score. Conclusion Shuntography can produce valuable clinical information uncovering mechanic complications after implantation VPS in patients with idiopathic normal-pressure hydrocephalus. Patients with mechanical complications of their VPS needed revision surgery and showed clinical benefit after treatment.


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