scholarly journals Long-term undiagnosed tuberous sclerosis

2021 ◽  
Vol 97 (3) ◽  
pp. 66-72
Author(s):  
Tatyana N. Varenova ◽  
Venera A. Goshadze ◽  
Daria I. Akutina ◽  
Oleg N. Pomerantsev
Keyword(s):  
Tuberous Sclerosis ◽  
Genetic Disease ◽  
Clinical Case ◽  
State Research ◽  
Diagnostic Center ◽  
The Face ◽  
Good Cosmetic ◽  
Cosmetic Effect ◽  
The Russian Federation

The article presents the history, etiology, pathogenesis and variability of the clinical picture of a rare genetic disease tuberous sclerosis. The clinical and laboratory criteria on the basis of which this diagnosis is made are described. Specialists of the Nizhny Novgorod Branch of the State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation carried out treatment and observation of the patient K. Patient was born in 1997, repeatedly turned to dermatologists in different cities of Russia with complaints about formations on the face, hands, legs and spots on the skin of the trunk that appeared in early childhood. The diagnosis was not confirmed. The doctors of the consultative and diagnostic center diagnosed with tuberous sclerosis based of four large and two small diagnostic criteria. Treatment of skin manifestations was carried out by laser destruction, it gave a good cosmetic effect. We recommended further examination by doctors of related specialties for identify lesions of other organs. This clinical case is of interest for dermatologists, because the described dermatosis is rarely found in everyday practice.

scholarly journals Long-Term Exposure and Safety of a Novel Topical Rapamycin Cream for the Treatment of Facial Angiofibromas in Tuberous Sclerosis Complex: Results From a Single-Center, Open-Label Trial

2019 ◽  
Vol 6 ◽  
pp. 2329048X1983504
Author(s):  
Margaret C. Wheless ◽  
Apana A. Takwi ◽  
Hassan Almoazen ◽  
James W. Wheless
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Fibrous Tissue ◽  
Systemic Absorption ◽  
Topical Formulation ◽  
Open Label ◽  
Long Term Follow Up ◽  
The Face ◽  
Systemic Side Effects

Facial angiofibromas, composed of fibrous tissue and blood vessels appearing on the face, are closely associated with tuberous sclerosis complex. Historically, oral rapamycin, a mammalian target of the rapamycin inhibitor of cell proliferation, has been used to treat visceral tuberous sclerosis–related tumors; however, the side effect profile of this medicine generally precludes its use in patients lacking significant internal involvement. The authors developed a novel topical formulation of rapamycin cream to treat the facial angiofibroma without exposing patients to possible systemic side effects. We followed 11 patients in a long-term, open-label, prospective study to evaluate the safety and effectiveness of rapamycin cream when used chronically. All of the patients showed an improvement in the appearance of their facial angiofibroma which was maintained in long-term follow-up without safety concerns or systemic absorption. The novel rapamycin cream was tolerated well by all patients and represents a way to address the cutaneous manifestation of tuberous sclerosis complex.

scholarly journals A clinical case of long-term macitentan therapy in a female patient with idiopathic pulmonary hypertension

2016 ◽  
Vol 88 (12) ◽  
pp. 88-93 ◽  
Author(s):  
O A Arkhipova ◽  
T V Martynyuk ◽  
I E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Female Patient ◽  
Clinical Case ◽  
Endothelin Receptor ◽  
Pathogenetic Therapy ◽  
Pulmonary Arterial ◽  
Idiopathic Pulmonary Hypertension ◽  
The Russian Federation ◽  
The Given

In late 2015, the Russian Federation registered the new non-selective endothelin receptor antagonist macitentan for the pathogenetic therapy of pulmonary arterial hypertension. The given clinical case demonstrates the possibility of using macitentan in a female patient with idiopathic pulmonary hypertension and its ability to affect the clinical, hemodynamic, and functional status of patients and to slow down the progression of the disease.

scholarly journals Retinal astrocytic hamartoma in tuberous sclerosis

2017 ◽  
Vol 10 (1) ◽  
pp. 97-101
Author(s):  
Yury S Astakhov ◽  
Pavel A Nechiporenko ◽  
Liya K Atlasova ◽  
Aleksandra I Titarenko ◽  
Konstantin N Monakhov ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Diagnostic Criteria ◽  
Clinical Case ◽  
Astrocytic Hamartoma

The article describes a clinical case of long-term undiagnosed tuberous sclerosis with retinal astrocytic hamartoma. Etiology, pathogenesis, clinical and pathomorphological diagnostic criteria of tuberous sclerosis are also described.

scholarly journals Political Instability Equals the Collapse of Tourism in Ukraine?

2021 ◽  
Vol 13 (8) ◽  
pp. 4126
Author(s):  
Natalia Tomczewska-Popowycz ◽  
Łukasz Quirini-Popławski
Keyword(s):  
Political Instability ◽  
Secondary Data ◽  
Unobserved Component ◽  
Tourist Flows ◽  
The Face ◽  
The Russian Federation ◽  
Unobserved Component Model ◽  
Tourism Economy ◽  
The Impact

The purpose of this study was to determine how political instability influences inbound tourist flows in Ukrainian cities, performance of tourism-related businesses, and tourism-based profits in general. This study allows us to present the impact of various events on the tourism economy in Ukraine; however, the available secondary data with the unobserved component model procedure detection give only a general overview of the situation. Thus, interviews were conducted with experts, including managers of accommodation facilities, employees of municipal tourism development departments, and researchers investigating tourism. Interviews with experts revealed opportunities, threats, and future scenarios of tourism in Ukraine in the face of five years of political instability. The results support previous findings that political instability reduces tourist traffic over the short term. On the other hand, the interviews with experts representing major province cities have shown different results for the long-term perspective. Cities with developed tourism sectors in areas away from the place of conflict are beneficiaries of political instability. Disadvantaged are cities that had their tourist flows based on the citizens of the aggressor’s country—the Russian Federation. Cities that are underdeveloped in terms of tourism did not experience a significant impact of the political instability in eastern Ukraine.

Russian low carbon development strategy

2020 ◽  
pp. 51-74
Author(s):  
I. A. Bashmakov
Keyword(s):  
Economic Growth ◽  
Development Strategy ◽  
Low Carbon ◽  
Security Risk ◽  
Ghg Emission ◽  
Emission Mitigation ◽  
Russian Economy ◽  
The Russian Federation ◽  
Scenario Projections

The article presents the key results of scenario projections that underpinned the Strategy for long-term low carbon economic development of the Russian Federation to 2050, including analysis of potential Russia’s GHG emission mitigation commitments to 2050 and assessment of relevant costs, benefits, and implications for Russia’s GDP. Low carbon transformation of the Russian economy is presented as a potential driver for economic growth that offers trillions-of-dollars-worth market niches for low carbon products by mid-21st century. Transition to low carbon economic growth is irreversible. Lagging behind in this technological race entails a security risk and technological backwardness hazards.

Development of thickened semi-synthetic engine oil M-5z / 20 AERO for four-stroke gasoline engines aircraft piston of unmanned aerial vehicles (UAVs)

2020 ◽  
Vol 06 ◽  
pp. 44-47
Author(s):  
A.A. Moykin ◽  
◽  
A.S. Medzhibovsky ◽  
S.A. Kriushin ◽  
M.V. Seleznev ◽  
...  
Keyword(s):  
Unmanned Aerial Vehicles ◽  
Engine Oil ◽  
Motor Oil ◽  
Gasoline Engines ◽  
State Research Institute ◽  
Technical Requirements ◽  
Aerial Vehicles ◽  
State Research ◽  
Industrial Batch ◽  
The Russian Federation

Nowadays, the creation of remotely-piloted aerial vehicles for various purposes is regarded as one of the most relevant and promising trends of aircraft development. FAU "25 State Research Institute of Chemmotology of the Ministry of Defense of the Russian Federation" have studied the operation features of aircraft piston engines and developed technical requirements for motor oil for piston four-stroke UAV engines, as well as a new engine oil M-5z/20 AERO in cooperation with NPP KVALITET, LLC. Based on the complex of qualification tests, the stated operational properties of the experimental-industrial batch of M-5z/20 AERO oil are generally confirmed.

Selective statistical estimates of multi-year monthly concentrations of biogenous pollution of small rivers of the Northwest zone of the Russian Federation

2020 ◽  
pp. 9-13
Author(s):  
Sergey Kovalenko
Keyword(s):  
Statistical Processing ◽  
Point Of View ◽  
Small Rivers ◽  
Statistical Parameters ◽  
Monthly Data ◽  
Statistical Estimates ◽  
Scientific Task ◽  
The Russian Federation ◽  
Agricultural Areas

The management of surface watercourses is an urgent scientific task. The article presents the results of statistical processing of long-term monthly data of field observations of hydrological and hydrochemical parameters along the Upper Yerga small river in the Vologda region. Sampling estimates of statistical parameters are obtained, autocorrelation and correlation analyzes are performed. The limiting periods from the point of view of pollution for water receivers receiving wastewater from drained agricultural areas are identified.

The Relevance of Energy Service Contracts in the Budget Sphere

2018 ◽  
pp. 64-68
Author(s):  
George V. Boos ◽  
Elena Yu. Matveeva
Keyword(s):  
Public Sector ◽  
Institutional Development ◽  
The Public ◽  
Debt Burden ◽  
Comprehensive Plan ◽  
Effective Implementation ◽  
Service Contracts ◽  
The Face ◽  
Energy Service ◽  
The Russian Federation

The problematic aspects related to the implementation of energy saving policy in the budget sphere are examined in the article. The factors hindering the mass and effective implementation of energysaving measures are highlighted in the article. Among these factors, there is the technical complexity of energysaving projects, the presence of innovative and investment risks, problems with the financial provision of costs in the face of increasing debt burden in most public budgets. The article concludes that in these circumstances only the energy service contract is a tool that allows implementing energy­saving measures without the first participation of budgetary funds in financing and allows transferring the risks of making technically inefficient decisions directly to the investor. In the article, the authors substantiate the importance of the institutional development of energy services directly in the public sector and analyze the measures of the comprehensive plan to improve the energy efficiency of the economy of the Russian Federation aimed at expanding the scope of energy service contracts in the public sector.

Challenges, Concerns, and Perspectives for Poland's Energy Transition during Global COVID-19 Pandemic

2020 ◽  
Vol 1 (2) ◽  
pp. 169-173
Author(s):  
Andrzej Lorkowski ◽  
Robert Jeszke
Keyword(s):  
Internal Energy ◽  
Energy Transition ◽  
Energy Transformation ◽  
Modern Times ◽  
Current Discussion ◽  
The Face ◽  
National Governments ◽  
The Eu ◽  
At Home

The whole world is currently struggling with one of the most disastrous pandemics to hit in modern times – Covid-19. Individual national governments, the WHO and worldwide media organisations are appealing for humanity to universally stay at home, to limit contact and to stay safe in the ongoing fight against this unseen threat. Economists are concerned about the devastating effect this will have on the markets and possible outcomes. One of the countries suffering from potential destruction of this situation is Poland. In this article we will explain how difficult internal energy transformation is, considering the long-term crisis associated with the extraction and usage of coal, the European Green Deal and current discussion on increasing the EU 2030 climate ambitions. In the face of an ongoing pandemic, the situation becomes even more challenging with each passing day.

Reabilitação estética de incisivo central superior severamente escurecido

2020 ◽  
Vol 12 (45) ◽  
pp. 91-96
Author(s):  
Washington José Batista das Neves ◽  
João Paulo Alves da Silva Aguiar ◽  
Jamile Menezes de Souza ◽  
Evandro Augusto Feitosa de Lima Amorim ◽  
Marlus da Silva Pedrosa ◽  
...  
Keyword(s):  
Clinical Case ◽  
Tooth Whitening ◽  
Human Eye ◽  
Self Confidence ◽  
The Face ◽  
Natural Dentition ◽  
Dental Whitening

Dental unit darkening is the cause of aesthetic complaint by the patient, mainly because it is easily noticed by the human eye. Tooth whitening and ceramic veneers allow aesthetic rehabilitation in a pattern of visual equality to the natural dentition, by restoring teeth color and shape symmetry, bringing to the face a harmonious impact that is reflected in the entire set that composes it. By recovering teeth healthy appearance, this association of procedures restores people’s self-confidence. This study reports a clinical case of an esthetic management for darkened tooth 21, describing the sequence of techniques association of dental whitening and ceramic veneer to achieve aesthetics balance, showing the advantages and limitations of these procedures.

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