System of galactic planetary nebulae

Нами уточнены расстояния до 554 галактических планетарных туманностей (ПТ) с использованием соотношения между ионизированной массой туманности μ и параметром оптической толщины, полученного [1]. Мы определили типы туманностей согласно [2] в модификации [3]. Полученные нами расстояния до галактических ПТ использовались для получения шкалы высот h различных подсистем Млечного Пути. Были найдены следующие значения: h = 208 ± 10 пк для объектов тонкого диска, h = 600 ± 54 пк для толстого диска и h = 1378 ± 180 пк для ПТ гало.We recalculated the distance to 554 galactic Planetary Nebulae (PNe) using the relation between the ionized mass of the nebula μ and the optical thickness parameter obtained by [1]. We classified these nebulae according to [2] types modified by [3]. The obtained by us distances to galactic PNe were used to obtain the scale heights h for different subsystems of the Milky Way. The following values of h were obtained: h = 208 ± 10 pc for thin disk objects, h = 600 ± 54 pc for thick disk ones, and h = 1378 ± 180 pc for halo PNe

Determining the Vertical Scale of the Planetary Nebulae of the Disk by the Method of Maximum Likelihood

Based on the scale from Akimkin et al. (2012), a catalog of distances to galactic planetary nebulae (PN), classified by Peimbert types, was created. The distribution function for Z-coordinate of the PN is modeled, the values of the scales of the heights of the thin and thick disks, as well as the height of the plane of symmetry of the distribution of the PN relative to the Sun are obtained. The calculated elevations are consistent with the results of other authors, including those obtained from stellar samples.На основании шкалы, описанной в работе Акимкина и др. (2012), был создан каталог расстояний до галактических планетарных туманностей (ПТ), классифицированных по Пеймбертовским типам. Промоделирована функция распределения Z-координаты ПТ, получены значения шкал высот тонкого и толстого дисков, а также высота плоскости симметрии распределения ПТ относительно Солнца. Рассчитанные шкалы высот согласуются с результатами других авторов, в том числе с полученными на основании звездных выборок

Field-Dependent Critical Current in Type-II Superconducting Strips: Combined Effect of Bulk Pinning and Geometrical Edge Barrier

Recent theoretical and experimental research on low-bulk-pinning superconducting strips has revealed striking dome-like magnetic-field distributions due to geometrical edge barriers. The observed magnetic-flux profiles differ strongly from those in strips in which bulk pinning is dominant. In this paper we theoretically describe the current and field distributions of a superconducting strip under the combined influence of both a geometrical edge barrier and bulk pinning at the strip's critical current Ic, where a longitudinal voltage first appears. We calculate Ic and find its dependence upon a perpendicular applied magnetic field Ha. The behavior is governed by a parameter p, defined as the ratio of the bulk-pinning critical current Ip to the geometrical-barrier critical current Is0. We find that when p > 2/pi and Ip is field-independent, Ic vs Ha exhibits a plateau for small Ha, followed by the dependence Ic-Ip ~ 1/Ha in higher magnetic fields.Comment: 4 pages, 2 figures, Fig. 1 revised, submitted to Phys. Rev.

Magnetic-field dependence of the critical currents in a periodic coplanar array of narrow superconducting strip

We calculate the magnetic-field dependence of the critical current due to both geometrical edge barriers and bulk pinning in a periodic coplanar array of narrow superconducting strips. We find that in zero or low applied magnetic fields the critical current can be considerably enhanced by the edge barriers, but in modest applied magnetic fields the critical current reduces to that due to bulk pinning alone.Comment: 23 pages, 7 figure

Compound heterozygous variants in NBAS as a cause of atypical osteogenesis imperfecta

Background Osteogenesis imperfecta (OI), the commonest inherited bone fragility disorder, affects 1 in 15,000 live births resulting in frequent fractures and reduced mobility, with significant impact on quality of life. Early diagnosis is important, as therapeutic advances can lead to improved clinical outcome and patient benefit. Report Whole exome sequencing in patients with OI identified, in two patients with a multi-system phenotype, compound heterozygous variants in NBAS (neuroblastoma amplified sequence). Patient 1: NBAS c.5741G > A p.(Arg1914His); c.3010C > T p.(Arg1004*) in a 10-year old boy with significant short stature, bone fragility requiring treatment with bisphosphonates, developmental delay and immunodeficiency. Patient 2: NBAS c.5741G > A p.(Arg1914His); c.2032C > T p.(Gln678*) in a 5-year old boy with similar presenting features, bone fragility, mild developmental delay, abnormal liver function tests and immunodeficiency. Discussion Homozygous missense NBAS variants cause SOPH syndrome (short stature; optic atrophy; Pelger-Huet anomaly), the same missense variant was found in our patients on one allele and a nonsense variant in the other allele. Recent literature suggests a multi-system phenotype. In this study, patient fibroblasts have shown reduced collagen expression, compared to control cells and RNAseq studies, in bone cells show that NBAS is expressed in osteoblasts and osteocytes of rodents and primates. These findings provide proof-of-concept that NBAS mutations have mechanistic effects in bone, and that NBAS variants are a novel cause of bone fragility, which is distinguishable from ‘Classical’ OI. Conclusions Here we report on variants in NBAS, as a cause of bone fragility in humans, and expand the phenotypic spectrum associated with NBAS. We explore the mechanism underlying NBAS and the striking skeletal phenotype in our patients

Effect of the COVID-19 pandemic on surgery for indeterminate thyroid nodules (THYCOVID): a retrospective, international, multicentre, cross-sectional study

Background Since its outbreak in early 2020, the COVID-19 pandemic has diverted resources from non-urgent and elective procedures, leading to diagnosis and treatment delays, with an increased number of neoplasms at advanced stages worldwide. The aims of this study were to quantify the reduction in surgical activity for indeterminate thyroid nodules during the COVID-19 pandemic; and to evaluate whether delays in surgery led to an increased occurrence of aggressive tumours.Methods In this retrospective, international, cross-sectional study, centres were invited to participate in June 22, 2022; each centre joining the study was asked to provide data from medical records on all surgical thyroidectomies consecutively performed from Jan 1, 2019, to Dec 31, 2021. Patients with indeterminate thyroid nodules were divided into three groups according to when they underwent surgery: from Jan 1, 2019, to Feb 29, 2020 (global prepandemic phase), from March 1, 2020, to May 31, 2021 (pandemic escalation phase), and from June 1 to Dec 31, 2021 (pandemic decrease phase). The main outcomes were, for each phase, the number of surgeries for indeterminate thyroid nodules, and in patients with a postoperative diagnosis of thyroid cancers, the occurrence of tumours larger than 10 mm, extrathyroidal extension, lymph node metastases, vascular invasion, distant metastases, and tumours at high risk of structural disease recurrence. Univariate analysis was used to compare the probability of aggressive thyroid features between the first and third study phases. The study was registered on ClinicalTrials.gov, NCT05178186.Findings Data from 157 centres (n=49 countries) on 87 467 patients who underwent surgery for benign and malignant thyroid disease were collected, of whom 22 974 patients (18 052 [78 center dot 6%] female patients and 4922 [21 center dot 4%] male patients) received surgery for indeterminate thyroid nodules. We observed a significant reduction in surgery for indeterminate thyroid nodules during the pandemic escalation phase (median monthly surgeries per centre, 1 center dot 4 [IQR 0 center dot 6-3 center dot 4]) compared with the prepandemic phase (2 center dot 0 [0 center dot 9-3 center dot 7]; p<0 center dot 0001) and pandemic decrease phase (2 center dot 3 [1 center dot 0-5 center dot 0]; p<0 center dot 0001). Compared with the prepandemic phase, in the pandemic decrease phase we observed an increased occurrence of thyroid tumours larger than 10 mm (2554 [69 center dot 0%] of 3704 vs 1515 [71 center dot 5%] of 2119; OR 1 center dot 1 [95% CI 1 center dot 0-1 center dot 3]; p=0 center dot 042), lymph node metastases (343 [9 center dot 3%] vs 264 [12 center dot 5%]; OR 1 center dot 4 [1 center dot 2-1 center dot 7]; p=0 center dot 0001), and tumours at high risk of structural disease recurrence (203 [5 center dot 7%] of 3584 vs 155 [7 center dot 7%] of 2006; OR 1 center dot 4 [1 center dot 1-1 center dot 7]; p=0 center dot 0039).Interpretation Our study suggests that the reduction in surgical activity for indeterminate thyroid nodules during the COVID-19 pandemic period could have led to an increased occurrence of aggressive thyroid tumours. However, other compelling hypotheses, including increased selection of patients with aggressive malignancies during this period, should be considered. We suggest that surgery for indeterminate thyroid nodules should no longer be postponed even in future instances of pandemic escalation.Funding None.Copyright (c) 2023 Published by Elsevier Ltd. All rights reserved

Первичные результаты применения стромально-васкулярной фракции аутологичной жировой ткани при стрессовом недержании мочи у мужчин

Introduction. Male stress urinary incontinence (SUI), which affects 10 % of the population, is most commonly caused by the effects of operations on the prostate gland. Despite the presence of a large number of surgical and conservative methods to treat this disease, the problem of choosing treatment tactics is still relevant. There are limited literature data on the effectiveness of using the stromal-vascular fraction (SVF) of autologous fat in the treatment of stress urinary incontinence. The aim of this study is to analyse the usage of SVF in the treatment of SUI patients.Materials and methods. A randomised study was carried out on a group of 8 patients with stress urinary incontinence of mild and moderate severity more than one year after radical prostatectomy or transurethral resection of the prostate. The patients received stromal-vascular fractions of autologous fat into the bladder sphincter area during the period November 2017 — June 2018.Results. After 4 weeks, the Pad Test and ICIQ-SF QoL assessments showed the first positive clinical results and improving quality of life. Checkpoints of 3, 6 and 12 months were selected for measuring results. Cytological analysis of the stromalvascular fraction of autologous fat revealed the expression of mesenchymal stem cell markers CD44, CD73, CD90, which may explain the method’s high efficiency.Conclusion. This work supports the hypothesis that transplantation of SVF from autologous adipose tissue is an effective and safe method for treating patients with incontinence.Введение. Стрессовое недержание мочи (СНМ) встречается у 10 % мужчин. Наиболее частой причиной возникновения СНМ у мужчин являются последствия операций на предстательной железе. Несмотря на наличие большого количества хирургических и консервативных методов лечения данного заболевания, проблема выбора тактики лечения до сих пор остается актуальной. Имеются ограниченные данные литературы об эффективности использования стромально-васкулярной фракции (СВФ) аутологичного жира в лечении стрессового недержания мочи. Целью данного исследования является анализ эффективности применения СВФ в лечении пациентов СНМ.Материалы и методы. Проводится открытое проспективное рандомизированное исследование. Исследуемая группа включала 8 пациентов со стрессовым недержанием мочи легкой и средней степени тяжести более одного года после радикальной простатэктомии или трансуретральной резекции простаты. Данным пациентам в период с ноября 2017 по июнь 2018 г. было выполнено введение стромально-васкулярной фракции аутологичного жира в зону сфинктера мочевого пузыря.Результаты. На основании Pad test и опросников ICIQ-SF, QoL через 4 недели получены первые положительные клинические результаты и улучшение качества жизни. Контрольными точками для оценки результатов выбраны 3, 6 и 12 месяцев. Цитологический анализ стромально-васкулярной фракции аутологичного жира выявил экспрессию маркеров мезенхимальных стволовых клеток CD44, CD73, CD90, что, вероятнее всего, и обуславливает высокую эффективность данного метода.Заключение. В данной работе отмечено, что трансплантация СВФ из аутологичной жировой ткани представляется эффективным и безопасным методом лечения пациентов с недержанием мочи.Введение. Стрессовое недержание мочи (СНМ) встречается у 10 % мужчин. Наиболее частой причиной возникновения СНМ у мужчин являются последствия операций на предстательной железе. Несмотря на наличие большого количества хирургических и консервативных методов лечения данного заболевания, проблема выбора тактики лечения до сих пор остается актуальной. Имеются ограниченные данные литературы об эффективности использования стромально-васкулярной фракции (СВФ) аутологичного жира в лечении стрессового недержания мочи. Целью данного исследования является анализ эффективности применения СВФ в лечении пациентов СНМ.Материалы и методы. Проводится открытое проспективное рандомизированное исследование. Исследуемая группа включала 8 пациентов со стрессовым недержанием мочи легкой и средней степени тяжести более одного года после радикальной простатэктомии или трансуретральной резекции простаты. Данным пациентам в период с ноября 2017 по июнь 2018 г. было выполнено введение стромально-васкулярной фракции аутологичного жира в зону сфинктера мочевого пузыря.Результаты. На основании Pad test и опросников ICIQ-SF, QoL через 4 недели получены первые положительные клинические результаты и улучшение качества жизни. Контрольными точками для оценки результатов выбраны 3, 6 и 12 месяцев. Цитологический анализ стромально-васкулярной фракции аутологичного жира выявил экспрессию маркеров мезенхимальных стволовых клеток CD44, CD73, CD90, что, вероятнее всего, и обуславливает высокую эффективность данного метода.Заключение. В данной работе отмечено, что трансплантация СВФ из аутологичной жировой ткани представляется эффективным и безопасным методом лечения пациентов с недержанием мочи.

Burden of Illness and Quality of Life in Tuberous Sclerosis Complex: Findings From the TOSCA Study

Research on tuberous sclerosis complex (TSC) to date has focused mainly on the physical manifestations of the disease. In contrast, the psychosocial impact of TSC has received far less attention. The aim of this study was therefore to examine the impact of TSC on health, quality of life (QoL), and psychosocial well-being of individuals with TSC and their families. Questionnaires with disease-specific questions on burden of illness (BOI) and validated QoL questionnaires were used. After completion of additional informed consent, we included 143 individuals who participated in the TOSCA (TuberOus SClerosis registry to increase disease Awareness) study. Our results highlighted the substantial burden of TSC on the personal lives of individuals with TSC and their families. Nearly half of the patients experienced negative progress in their education or career due to TSC (42.1%), as well as many of their caregivers (17.6% employed; 58.8% unemployed). Most caregivers (76.5%) indicated that TSC affected family life, and social and working relationships. Further, well-coordinated care was lacking: a smooth transition from pediatric to adult care was mentioned by only 36.8% of adult patients, and financial, social, and psychological support in 21.1, 0, and 7.9%, respectively. In addition, the moderate rates of pain/discomfort (35%) and anxiety/depression (43.4%) reported across all ages and levels of disease demonstrate the high BOI and low QoL in this vulnerable population

An OBSL1-Cul7Fbxw8 Ubiquitin Ligase Signaling Mechanism Regulates Golgi Morphology and Dendrite Patterning

The elaboration of dendrites in neurons requires secretory trafficking through the Golgi apparatus, but the mechanisms that govern Golgi function in neuronal morphogenesis in the brain have remained largely unexplored. Here, we report that the E3 ubiquitin ligase Cul7Fbxw8 localizes to the Golgi complex in mammalian brain neurons. Inhibition of Cul7Fbxw8 by independent approaches including Fbxw8 knockdown reveals that Cul7Fbxw8 is selectively required for the growth and elaboration of dendrites but not axons in primary neurons and in the developing rat cerebellum in vivo. Inhibition of Cul7Fbxw8 also dramatically impairs the morphology of the Golgi complex, leading to deficient secretory trafficking in neurons. Using an immunoprecipitation/mass spectrometry screening approach, we also uncover the cytoskeletal adaptor protein OBSL1 as a critical regulator of Cul7Fbxw8 in Golgi morphogenesis and dendrite elaboration. OBSL1 forms a physical complex with the scaffold protein Cul7 and thereby localizes Cul7 at the Golgi apparatus. Accordingly, OBSL1 is required for the morphogenesis of the Golgi apparatus and the elaboration of dendrites. Finally, we identify the Golgi protein Grasp65 as a novel and physiologically relevant substrate of Cul7Fbxw8 in the control of Golgi and dendrite morphogenesis in neurons. Collectively, these findings define a novel OBSL1-regulated Cul7Fbxw8 ubiquitin signaling mechanism that orchestrates the morphogenesis of the Golgi apparatus and patterning of dendrites, with fundamental implications for our understanding of brain development

Use of stromal-vascular fraction from autological fat tissue under stressed urinary disease in men

The aim of the work is to develop a minimally invasive method for restoring the function of the sphincter of the urinary bladder using autologous fat in the stromal-vascular fraction (SVF). Materials and methods. Patients with a diagnosis of stress urinary incontinence of moderate severity were given a transurethral injection of an autologous SVF into the zone of the external sphincter of the bladder. Isolation of SVF was performed by a nonenzymatic method using tumescent lipoaspiration. The resulting material was sent to the laboratory for the study of cellular composition. Results. Based on the Pad test and ICIQ-SF questionnaires, Qol received the first positive clinical results and improved quality of life after 4 weeks. Conclusions. In this work, it is noted that transplantation of SVF from autologous adipose tissue appears to be an effective and safe method of treatment of patients with incontinence.Цель работы - разработка малоинвазивного метода восстановления функции сфинктера мочевого пузыря с применением стромально-васкулярной фракции(СВФ) аутологичного жира. Материалы и методы. Пациентам с диагнозом стрессовое недержание мочи легкой и средней степеней тяжести выполнено трансуретральное введение аутологичной СВФ в зону наружного сфинктера мочевого пузыря. Выделение СВФ производилось неферментным методом при помощи тумесцентной липоаспирации. Полученный материал был направлен в лабораторию для исследования клеточного состава. результаты. На основании Pad test и опросников ICIQ-SF, Qol через 4 недели получены первые положительные клинические результаты и улучшение качества жизни. Выводы. В данной работе отмечено, что трансплантация СВФ из аутологичной жировой ткани представляется эффективным и безопасным методом лечения пациентов с недержанием мочи