scholarly journals Familial Mediterranean fever: clinical state of the art

QJM ◽  
2020 ◽  
Author(s):  
B H Egeli ◽  
S Ugurlu
Keyword(s):  
Familial Mediterranean Fever ◽  
Clinical Relevance ◽  
Scientific Literature ◽  
State Of The Art ◽  
Treatment Modalities ◽  
Clinical State ◽  
Inflammation Research ◽  
The Past ◽  
Mediterranean Fever

Summary As the inflammation research improves year-by-year, so does our understanding of the autoinflammatory conditions. Over the past years, the number of monogenic autoinflammatory conditions snowballed thanks to our understanding of basic immunology and genetics. Familial Mediterranean fever (FMF), being the entrance to this fascinating world, still has clinical relevance as it enables us to understand our approach to these patients, treatment modalities and pathological mechanisms. This review can be used as a tool for clinicians already working with FMF patients to update themselves on recent scientific literature.

Familial Mediterranean Fever (FMF), Female and Male Fertility: Literature Review

2021 ◽  
pp. 3-11
Author(s):  
П.О. Соцкий
Keyword(s):  
Familial Mediterranean Fever ◽  
Male Fertility ◽  
Eastern Mediterranean ◽  
Reproductive Function ◽  
Peritoneal Adhesion ◽  
The Past ◽  
The World ◽  
Mediterranean Fever ◽  
Testicular Insufficiency ◽  
The Relationship

Семейная средиземноморская лихорадка (CCЛ) - моногенное наследственное аутовоспалительное заболевание, характеризующееся периодически повторяющимися приступами лихорадки, серозитами разной локализации, артритами, эризипелоидоподобным высыпанием. Хотя ССЛ болеют преимущественно представители популяций восточно-средиземноморского происхождения (турки, армяне, арабы, евреи-сефарды), в наши дни болезнь регистрируется по всему миру, что связано с непрерывными миграционными процессами. Потенциальные осложнения ССЛ (фиброз и амилоидоз) и их лечение колхицином могут повлиять на репродуктивную систему больных либо путем обструкции маточных труб, либо через избыточное образование дефектных сперматозоидов и ооцитов, либо вследствие овариальной/тестикулярной недостаточности. Амилоидоз с преимущественным отложением амилоида в яичниках и яичках может приводить к женскому и мужскому бесплодию. У мужчин колхицин в редких случаях индуцирует олиго/азооспермию. У женщин основными причинами бесплодия в прошлом считались дисовуляция и перитонеальная адгезия. В последние годы ситуация с фертильностью значительно улучшилась благодаря применению колхицина. В обзоре обобщается текущая информация о взаимосвязи женской и мужской фертильности с ССЛ и приемом колхицина; излагаются современные подходы к восстановлению репродуктивной функции при бесплодии. Акцент сделан на аспектах безопасности терапии, основные положения которой представлены в клинических рекомендациях по лечению ССЛ Европейской антиревматической лиги (EULAR,2016). Familial Mediterranean fever (FMF) is a monogenic inherited autoinflammatory disease characterized by recurrent episodes (attacks) of fever, serositis of different localization, arthritis, erysipelaslike rashes. Eastern Mediterranean persons (especially Turks, Armenians, Arabs, Sephardic Jews) are most frequently affected. Nowadays the disease is registered throughout the world because of migration processes. Potential complications of FMF (fibrosis and amyloidosis) and their treatment with colchicine may affect the reproductive system either by salpinx obstruction causing mechanical infertility, or by excessive formation of defective sperm and oocytes, either due to ovarian /testicular insufficiency. Amyloidosis can lead to female and male infertility with amyloid preponderance in the ovaries and testicles. Colchicine rarely induces oligo/ azoospermia in men. In women, disovulation and peritoneal adhesion were the main causes of infertility in the past. In recent years, the fertility situation has improved considerably due to the use of colchicine. The review summarizes current information on the relationship between female and male fertility with FMF and colchicine; modern approaches to restoring reproductive function in infertility are outlined. The focus is on the aspects of the safety of therapy, the main provisions of which are presented in the clinical recommendations for treatment of the FMF of the European Anti-Rheumatic League (EULAR, 2016).

A Macintosh Interface for the Cameca Camebax-Micro Electron Microprobe

1990 ◽  
Vol 48 (1) ◽  
pp. 438-439
Author(s):  
Carl E. Henderson
Keyword(s):  
Electron Microprobe ◽  
Processing Speed ◽  
Word Processing ◽  
State Of The Art ◽  
Computer Control ◽  
Third Party ◽  
Disk Storage ◽  
The Past ◽  
User Facility ◽  
Instrument Control

Over the past few years it has become apparent in our multi-user facility that the computer system and software supplied in 1985 with our CAMECA CAMEBAX-MICRO electron microprobe analyzer has the greatest potential for improvement and updating of any component of the instrument. While the standard CAMECA software running on a DEC PDP-11/23+ computer under the RSX-11M operating system can perform almost any task required of the instrument, the commands are not always intuitive and can be difficult to remember for the casual user (of which our laboratory has many). Given the widespread and growing use of other microcomputers (such as PC’s and Macintoshes) by users of the microprobe, the PDP has become the “oddball” and has also fallen behind the state-of-the-art in terms of processing speed and disk storage capabilities. Upgrade paths within products available from DEC are considered to be too expensive for the benefits received. After using a Macintosh for other tasks in the laboratory, such as instrument use and billing records, word processing, and graphics display, its unique and “friendly” user interface suggested an easier-to-use system for computer control of the electron microprobe automation. Specifically a Macintosh IIx was chosen for its capacity for third-party add-on cards used in instrument control.

A 200-Year History of the Study of Childhood Language Disorders of Unknown Origin: Changes in Terminology

2020 ◽  
Vol 5 (1) ◽  
pp. 6-11 ◽  
Author(s):  
Laurence B. Leonard
Keyword(s):  
Language Impairment ◽  
Specific Language Impairment ◽  
Scientific Literature ◽  
Language Disorder ◽  
Language Disorders ◽  
Unknown Origin ◽  
Developmental Language Disorder ◽  
The Past ◽  
History Of ◽  
The Many

Purpose The current “specific language impairment” and “developmental language disorder” discussion might lead to important changes in how we refer to children with language disorders of unknown origin. The field has seen other changes in terminology. This article reviews many of these changes. Method A literature review of previous clinical labels was conducted, and possible reasons for the changes in labels were identified. Results References to children with significant yet unexplained deficits in language ability have been part of the scientific literature since, at least, the early 1800s. Terms have changed from those with a neurological emphasis to those that do not imply a cause for the language disorder. Diagnostic criteria have become more explicit but have become, at certain points, too narrow to represent the wider range of children with language disorders of unknown origin. Conclusions The field was not well served by the many changes in terminology that have transpired in the past. A new label at this point must be accompanied by strong efforts to recruit its adoption by clinical speech-language pathologists and the general public.

PLANEJAMENTO FAMILIAR COMO ASSUNTO DE MULHER!? PERFIL DE GÊNERO NA PRODUÇÃO CIENTÍFICA NO BRASIL

2019 ◽  
Vol 8 (1) ◽  
pp. 221-235 ◽  
Author(s):  
Daniella De Paula Chiesa ◽  
Mário Antônio Sanches ◽  
Daiane Priscila Simão-Silva
Keyword(s):  
Family Planning ◽  
Scientific Literature ◽  
State Of The Art ◽  
Health Sciences ◽  
Scientific Production ◽  
The State ◽  
Review Of The Literature ◽  
The Subject

O estudo do Planejamento familiar, no contexto da bioética, abre-se para diversas perspectivas, entre elas a valorização dos seus diferentes atores. Situado neste contexto o artigo tem como objetivo identificar o perfil de gênero na produção científica sobre Planejamento Familiar no Brasil, entre 2000 e 2014, assim como a área de formação e especialização dos autores. Foram utilizadas metodologias que permitiram mapear o estado da arte do tema estudado, a partir de uma revisão da literatura. O resultado da pesquisa identifica que a produção científica sobre Planejamento Familiar no Brasil se compõe de perfil destacadamente feminino (71,76%). Dos 73 artigos analisados, 42 (57,53%) o foco do tema está direcionado à mulher assim como evidencia-se a área de ciências da saúde com maior concentração das publicações do tema.  Este aspecto da pesquisa abre para uma realidade complexa onde se buscam criticamente as razões para a pesquisa em Planejamento Familiar ter ênfase na mulher e ser um tema de relevância nas ciências da saúde.Palavras-chave: Produção científica, Planejamento Familiar, Gênero.  ABSTRACT: The study of Family Planning, in the context of bioethics, opens to diverse perspectives, among them the appreciation of their different agents. Situated in this context the article aims to identify the profile of gender in scientific literature on Family Planning in Brazil, between 2000 and 2014, as well as the area of training and specialization of the authors. Methodologies were used which allowed to map the State of the art of the subject studied, from a review of the literature. The results found identify that the scientific production on Family Planning in Brazil is formed with a outstandingly female profile (71,76%). Of the 73 articles examined, 42 (57.53%) the focus of the topic is directed to women as well as showing the health sciences area with highest concentration of publications. This aspect of the research opens to a complex reality where we seek critically the reasons for Research in Family Planning have emphasis on woman and be a topic of relevance in health sciences.Keywords: Scientific Production, Family Planning, Gender.

Sign in / Sign up

Export Citation Format

Share Document