Urological anomalies in OHVIRA syndrome (Herlyn-Werner-Wunderlich syndrome)

We provide a systematic review of articles related to OHVIRA syndrome (Herlyn-Werner-Wunderlich syndrome), found in PubMed/MedLine, Web of Science, Scopus, Scirus, The Cochrane Library and eLIBRARy to show how the urological component of this congenital malformation, previously considered as a concomitant anomaly, has become an important diagnostic marker. Several attempts were made to classify this condition, and they got more complex to include these variants of abnormalities of the kidney and ureter that were found due to advances in imaging technology. As a result, the understanding of the medical community about the OHVIRA syndrome has gradually changed over the past hundred years, and the growing interest of pediatric urologists and surgeons, in turn, has contributed to the early diagnosis of the syndrome and the improvement of examination methods

Superior strength of carbon steel with an ultrafine-grained microstructure and its enhanced thermal stability

© 2015, Springer Science+Business Media New York. The paper presents the results of a study on the microstructure and mechanical properties of a medium-carbon steel (0.45 % C) processed by severe plastic deformation (SPD) via high-pressure torsion (HPT). Martensite quenching was first applied to the material, and then HPT processing was conducted at a temperature of 350 °C. As a result, a nanocomposite type microstructure is formed: an ultrafine-grained (UFG) ferrite matrix with fine cementite particles located predominantly at the boundaries of ferrite grains. The processed steel is characterized by a high-strength state, with an ultimate tensile strength over 2500 MPa. Special attention is given to analysis of the thermal stability of the microstructure and properties of the steel after HPT processing in comparison with quenching. It is shown that the thermal stability of the UFG structure produced by HPT is visibly higher than that of quenching-induced martensite. The origin of the enhanced strength and thermal stability of the UFG steel is discussed

Lower pole obstructive megaureter of duplex kidney: an exception to the Weigert-Meyer rule

Introduction. Anatomical features of the urinary tract in patients with duplicate kidneys are described using the Weigert-Meyer rule, since the orifice of the upper ureter has an ectopic location (inferomedial) and the orifice of the lower ureter has an orthotopic location (superolateral). However, there are rare cases of violation of this rule, complicated by obstructive megaureter, ectopic ureteral orifice, the presence of ureterocele.Objective. To report the rare clinical case of a lower pole obstructive megaureter as a violation of the Meyer-Weigert rule in the patient with complete ureteral duplication and to describe the use of ureteroureterostomy as an effective and safe method of surgical correction of the presented anomaly.Clinical case. We present a case of the infant (5 months old) with a lower pole obstructive megaureter. This pathology was identified through intravenous urography and voiding cystourethrography. Laparoscopic proximal end-to-side ureteroureteroanastomosis was chosen as a surgical treatment. Postoperative control intravenous urography showed the effectiveness (a reduction in the lower pole collecting system of the duplex kidney was revealed) and the safety of this method of correction.Conclusion.  There are only several clinical cases about exceptions to the Weigert-Meyer rule reported in literature, and most of them are about adult patients. The main surgical method of treatment in such cases is heminephrectomy. To our knowledge, this is the only reported case of using ureteroureterostomy in the patient with a lower pole obstructive megaureter. This technique has shown its effectiveness and safety for restoring the patency of the urinary tract, confirmed during the control postoperative examination

Attitudes towards vaccination against COVID-19 among athletes of Russian national teams in comparison with non-athletes aged 18 to 40

The purpose of this work was to study the attitude of the Russian population to vaccination against COVID-19. Vaccination attitudes in young adults were studied both in the general population and among those who are professionally involved in sports, since attitudes towards health and the initial level of physical resources are important for forming attitudes towards vaccination. A total of 2579 people aged 18 to 40 were examined. Of these, 2233 are from the general population, 346 are high performance athletes.Methods. This was a cohort cross-sectional study. A specially designed questionnaire was used, located on Internet resources 2 months after the start of mass vaccination in Russia.Results. In the group of elite athletes, there are significantly more people who consider vaccination unnecessary or treat it indifferently, and a smaller proportion of those who consider it useful or doubt its effectiveness as compared to the general population of the same age. Low adherence to vaccination may be associated with fear of potential complications. Among elite athletes, there are significantly more people who are seriously afraid of complications from vaccination — 143 (41.3 %), compared to the first group of respondents — 745 (33.4 %) (p < 0.01, φ = 2.839). Probably, these concerns are related to the crucial importance of health and physical condition for professional athletes.Conclusions. It is advisable to develop recommendations for vaccination against COVID-19 for elite athletes, which will describe in detail the benefits of vaccination for athletes, possible side effects, their frequency and impact on the training process

Organisational and methodological challenges of CAR-T manufacturing in the Russian Federation

Despite their widespread clinical implementation, chimeric antigen receptor T-cell (CAR-T) therapy products, including those manufactured by industrial processes, are still not legally available or used in the Russian Federation.The aim of the study was to describe the current challenges associated with specific aspects of CAR-T manufacturing in the Russian Federation and the potential ways to overcome them.This article discusses the regulatory, legal, organisational, and methodological challenges of CAR-T manufacturing. It analyses differences in the interpretation of CAR-T therapy products under national and supranational law. According to Russian Federal Law No. 180-FZ “On Biomedical Cell Products” of 23 June 2016, CAR-T therapy products are considered biomedical cell products. However, according to Decision No. 78 of the Council of the Eurasian Economic Commission “On the Rules of Marketing Authorisation and Assessment of Medicinal Products for Human Use” of 3 November 2016, CAR-T therapy products are considered advanced therapy medicinal products (ATMPs). This article provides a detailed overview of the difficulties in obtaining starting biological materials (i.e. the inability to consider the patient as a donor) and transferring the materials for CAR-T manufacturing (i.e. the inapplicability of national law). In addition, this article describes export aspects specific to biological materials. The authors reckon that CAR-T therapy products should be categorised as ATMPs and that the corresponding active pharmaceutical ingredients, genetically modified autologous lymphocytes, should be defined as starting materials. Therefore, genetically modified autologous lymphocytes should be regulated under the requirements for starting materials for the manufacturing of active pharmaceutical ingredients that are set forth in Decision No. 77 of the Council of the Eurasian Economic Commission “On the Adoption of the Rules of Good Manufacturing Practice of the Eurasian Economic Union” of 3 November 2016. In conclusion, the authors recognise the need for national and supranational law harmonisation. For this task, it is necessary to establish expert groups that will include clinicians, legal experts, and representatives from the relevant authorities and the pharmaceutical industry

АТРЕЗИЯ ТОЛСТОЙ КИШКИ В СОЧЕТАНИИ С БОЛЕЗНЬЮ ГИРШПРУНГА: РЕДКОЕ КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

Introduction. Hirschsprung’s disease is a congenital anomaly characterized by the absence of ganglion cells in submucosal and intramuscular layers of intestinal wall that leads to the intestinal obstruction. 70–80 % of cases are isolated malformation but it can be combined with chromosomal abnormalities and other malformations. Coexistence of Hirschsprung’s disease with intestinal atresia is extremely rare. It can cause significant difficulties in diagnostics and treatment.Material and methods. Patient A, a boy, was transferred to the surgical department at the age of two days with a history of intestinal obstruction. He had abdominal X-ray studies: intestinal obstruction. The contrast enema showed microcolon that was coiled in the pelvis. Patient was prepared to the surgical treatment. Intraoperatively colonic atresia was identified at 10 sm from the ileocecal valve. A double colostomy was performed to the child and biopsy on the level of mucous fistula was taken. Histological study showed the aganglionosis of the distal colon. At the age of 4 months, the patient underwent Soave-Swenson endorectal pullthrough procedure with intraoperative extended express-biopsy that confirmed the absence of ganglion cells in whole distal bowel. The aganglionic part was resected, the ileocecal valve with the part of the colon of 10 cm long was mobilized and the endorectal bringing the colon down to the perineum was performed by Soave – Swenson. Postoperative recovery was uneventful.Results. A high index of suspicion is required to promptly diagnose Hirschsprung’s disease in a child with colonic atresia despite the rare combination of these two anomalies. In this case the histological study allowed to recognize association of colonic atresia with Hirschsprung’s disease and helped to avoid complications after further surgery.Conclusions. Early detection of coexisting of these two anomalies helps to prevent the development of serious postoperative complications. Введение. Болезнь Гиршпрунга – врожденное заболевание, которое характеризуется отсутствием нервных ганглиев в подслизистом и межмышечном слоях кишечной стенки, приводящее к нарушению пассажа содержимого по желудочно-кишечному тракту. В 70–80 % случаев является изолированным пороком развития, однако может сочетаться с хромосомными патологиями и другими аномалиями. Сочетание болезни Гиршпрунга с атрезией тонкой или толстой кишки встречается довольно редко и может вызвать значительные трудности в диагностике и лечении.Материал и методы. Мальчик А. был переведен в стационар в возрасте 2 суток жизни с клиникой низкой кишечной непроходимости. Обследован рентгенологически: картина кишечной непроходимости. При ирригографии, помимо картины микроколона, обращало на себя внимание необычное расположение толстой кишки в малом тазу. Подготовлен к оперативному лечению. Интраоперационно выявлена атрезия толстой кишки на расстоянии 10 см от илеоцекального угла. Ребенку наложена двойная колостома, взята биопсия отводящей и приводящей кишки. По гистологическому заключению – аганглиоз отключенной кишки. В 4 месяца выполнена радикальная операция. Интраоперационно произведена расширенная экспресс-биопсия толстой кишки, ганглии выявлены только в приводящем отделе. Аганглионарный участок резецирован, мобилизован илеоцекальный угол с участком толстой кишки длиной 10 см и произведено эндоректальное низведение толстой кишки на промежность по Soave – Swenson. Послеоперационный период – без хирургических осложнений.Результаты. Несмотря на довольно редкое сочетание этих двух патологий, при оперативном лечении по поводу атрезии толстой кишки необходимо помнить о вероятности аганглиоза отводящего отдела. В данном случае гистологическое исследование участка отводящей кишки позволило рано выявить сочетание порока с болезнью Гиршпрунга и избежать возможных осложнений при последующем оперативном вмешательстве. Выводы. Раннее выявление сочетания двух аномалий позволит предупредить развитие тяжелых послеоперационных осложнений.

Взаимосвязь некоторых параметров мукозального иммунитета полости рта с уровнем витамина D у пациентов с множественным кариесом

Aim. To determine the saliva level of immunoregulatory proteins in patients with rampant caries and 25-hydroxyvitamin D (25(OH)D) deficiency and evaluate the association of their concentration with 25(OH)D plasma level.Materials and methods. The study was performed in two groups. The experimental group included 15 patients aged 20–22 years with rampant caries and the 25(OH)D plasma level of < 20 ng / ml. The control group encompassed 15 healthy age-matched volunteers with the 25(OH)D plasma level of 20–100 ng / ml. The concentrations of B7.2 (CD86), free active TGF-β1, CTLA-4, PD-1, Tim-3, LAG-3, IGFBP-4, and ICAM-1 were assessed using flow cytometry. The levels of LL-37 and secretory immunoglobulin A (sIgA) were measured using ELISA. The Spearman’s rank correlation coefficient was used to reveal a correlation between the indicated proteins and the 25(OH)D plasma level.Results. A decrease in B7.2 (CD86), PD-1, Tim-3, sIgA, and LL-37 and elevation of IGFBP-4 and ICAM-1 saliva levels were detected in patients with rampant caries and 25-hydroxyvitamin D deficiency. A positive Spearman’s rank correlation coefficient was revealed between plasma 25(OH)D and saliva levels of free active TGF-β1, CTLA4, B7.2 (CD86), LL-37, and sIgA. A negative correlation was revealed between 25(OH)Dand ICAM-1.Conclusion. 25(OH)D deficiency in patients with rampant caries is associated with decreased levels of B7.2 (CD86), PD-1, Tim-3, sIgA, and LL-37 and elevated levels of IGFBP-4 and ICAM-1 in the saliva. Цель – оценить содержание иммунорегуляторных молекул в слюне у лиц с множественным кариесом и дефицитом 25(OH)D3 и определить взаимосвязи их величин с концентрацией 25(OH)D3 в крови.Материалы и методы. Обследованы две группы лиц в возрасте 20–22 лет. В одну включены 15 человек с кариесом и уровнем 25(OH)D3 менее 20 нг/мл, в другую (контрольную) – 15 здоровых человек с содержанием 25(OH)D3 30–100 нг/мл. В ротовой жидкости определены концентрации растворимых форм молекул B7.2 (CD86), Free Active TGF-b1, CTLA-4, PD-1, Tim-3, LAG-3, IGFBP-4, ICAM-1 методом проточной цитофлуометрии, количество кателицидина LL-37, секреторного иммуноглобулина A (IgA) методом иммуноферментного анализа. Между определяемыми показателями рассчитан критерий корреляции Спирмена.Результаты. У лиц с кариесом и дефицитом витамина D выявлено снижение значений Free Active TGF-b1, B7.2 (CD86), PD-1, Tim-3, sIgA, кателицидина LL-37 и повышение уровня IGFBP-4 и ICAM-1 в слюне. Обнаружено наличие прямых корреляционных связей между количеством 25(OH)D3 в крови, с одной стороны, и значениями Free Active TGF-b1, CTLA-4, В7.2 (CD86), секреторного IgA, пептида LL-37 – с другой. Зафиксирована отрицательная взаимосвязь между величинами 25(OH)D3 и ICAM-1.Заключение. На фоне дефицита витамина D при множественном кариесе в ротовой жидкости регистрируются низкие концентрации Free Active TGF-b1, B7.2 (CD86), PD-1, Tim-3, секреторного IgA, кателицидина LL-37 по сравнению с контролем, но увеличены значения IGFBP-4 и ICAM-1.

The analysis of detectability of the infectious agent at nephrolithiasis treatment

The purpose of this work is monitoring of a kind and structure of microflora at sick of urolithic patients in the presence of chronic calculous pyelonephritis from 2006 to 2009, and also an estimation of dependence of revealing of the infectious agent from the size of concrement. The retrospective analysis of revealing of the infectious agent at 1132 patients of a nephrolithiasis at a stage of preparation for treatment by a method remote and contact lithotripsies concerning a stone of a kidney in the size to 1.5 sm, a stone in the size K,1 - КЗ is carried out, an ureteral stone in the size from 0.7 to 1.5 sm. Direct dependence of revealing of microflora on the size конкремента and change of a kind and microflora structure for the investigated period speaks about necessity of constant monitoring for a choice of adequate antibacterial therapy.Целью данной работы является мониторинг вида и состава микрофлоры у больных мочекаменной болезнью при наличии хронического калькулезного пиелонефрита за период с 2006 по 2009г.г., а также оценка зависимости выявления инфекционного агента от размера конкремента. Проведен ретроспективный анализ выявления инфекционного агента у 1132 больных нефролитиазом на этапе подготовки к лечению методом дистанционной и контактной литотрипсии, а также перкутанной нефролитолапаксии по поводу камня почки размером до 1.5 см, коралловидного камня размером К1 - КЗ, камня мочеточника размером от 0.7 до 1.5 см. Прямая зависимость выявления микрофлоры от размера конкремента и изменение вида и состава микрофлоры за исследуемый период говорит о необходимости постоянного мониторинга для выбора адекватной антибактериальной терапии

Impact of the Present-Day Climate Changes on the Natural Plague Foci Condition, Situated in the Territory of the Russian Federation and Other CIS Countries

Climate changes determine dynamics of epizootic activity of natural plague foci in the XX and XXI centuries. Obvious shift to continentality in the Northern hemisphere can put an end to inter-epizootic period in lowland steppe and semi-desert natural foci of the Pre-Caucasian region, North and North-West Caspian Sea regions. Presently observed conjoined development of inter-epizootic periods in natural plague foci with different biocoenotic structure in the territory of Pre-Caspian Lowland gives the grounds for assuming the presence of a common cause preconditioning parasitic system depression in the region. One of the key elements of this phenomenon is a change of status of little souslik – formerly the main plague carrier in the region. Subsequent to the climate change the key role of plague carrier is more often played by non-hibernating rodent species nowadays. Thus a significant increase in epizootic potential of steppe natural plague foci in the territory of Kazakhstan and Central Asia is prognosticated. With the further temperature fall during wintertime, snow cover height increase and glacial area extension epizootic activity of the mountain and high mountain foci must drop off significantly. Alongside with this updated data on plague agent ecology, predetermining possibility of its existence in the soil biota, confirm the key role of climactic factors in the plague enzootic outbreak

Lipolysis drives expression of the constitutively active receptor GPR3 to induce adipose thermogenesis

Thermogenic adipocytes possess a therapeutically appealing, energy-expending capacity, which is canonically cold-induced by ligand-dependent activation of β-adrenergic G protein-coupled receptors (GPCRs). Here, we uncover an alternate paradigm of GPCR-mediated adipose thermogenesis through the constitutively active receptor, GPR3. We show that the N terminus of GPR3 confers intrinsic signaling activity, resulting in continuous Gs-coupling and cAMP production without an exogenous ligand. Thus, transcriptional induction of Gpr3 represents the regulatory parallel to ligand-binding of conventional GPCRs. Consequently, increasing Gpr3 expression in thermogenic adipocytes is alone sufficient to drive energy expenditure and counteract metabolic disease in mice. Gpr3 transcription is cold-stimulated by a lipolytic signal, and dietary fat potentiates GPR3-dependent thermogenesis to amplify the response to caloric excess. Moreover, we find GPR3 to be an essential, adrenergic-independent regulator of human brown adipocytes. Taken together, our findings reveal a noncanonical mechanism of GPCR control and thermogenic activation through the lipolysis-induced expression of constitutively active GPR3