scholarly journals The changing face of a rare disease: lymphangioleiomyomatosis

2015 ◽  
Vol 46 (5) ◽  
pp. 1471-1485 ◽  
Author(s):  
Sergio Harari ◽  
Olga Torre ◽  
Roberto Cassandro ◽  
Joel Moss
Keyword(s):  
Rare Disease ◽  
Tuberous Sclerosis Complex ◽  
Therapeutic Approach ◽  
Clinical Manifestations ◽  
Therapeutic Strategies ◽  
The Past ◽  
Lymphatic Abnormalities ◽  
Abdominal Tumours ◽  
Remarkable Progress ◽  
Made In

Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex.In the past decade remarkable progress has been made in understanding of the pathogenesis of this disease leading to a new therapeutic approach. This review summarises recent advances regarding pathogenic mechanisms and clinical manifestations, and highlights the current and the most promising future therapeutic strategies.

scholarly journals Molecular Biology of Rotavirus Entry and Replication

2009 ◽  
Vol 9 ◽  
pp. 1476-1497 ◽  
Author(s):  
Marie Christine Ruiz ◽  
Theresa Leon ◽  
Yuleima Diaz ◽  
Fabian Michelangeli
Keyword(s):  
Molecular Mechanisms ◽  
Rna Virus ◽  
Viral Proteins ◽  
Therapeutic Strategies ◽  
Viral Gastroenteritis ◽  
Structural Studies ◽  
Double Stranded Rna ◽  
The Individual ◽  
Remarkable Progress ◽  
Made In

Rotavirus is a nonenveloped, double-stranded, RNA virus belonging to the Reoviridae family and is the major etiological agent of viral gastroenteritis in young children and young animals. Remarkable progress in the understanding of the rotavirus cycle has been made in the last 10 years. The knowledge of viral replication thus far acquired is based on structural studies, the expression and coexpression of individual viral proteins, silencing of individual genes by siRNAs, and the effects that these manipulations have on the physiology of the infected cell. The functions of the individual rotavirus proteins have been largely dissected; however, the interactions between them and with cell proteins, and the molecular mechanisms of virus replication, are just beginning to be understood. These advancements represent the basis for the development of effective vaccination and rational therapeutic strategies to combat rotavirus infection and diarrhea syndromes. In this paper, we review and try to integrate the new knowledge about rotavirus entry, replication, and assembly, and pose some of the questions that remain to be solved.

scholarly journals Anthocyanins as Antidiabetic Agents—In Vitro and In Silico Approaches of Preventive and Therapeutic Effects

Molecules ◽  
2020 ◽  
Vol 25 (17) ◽  
pp. 3813 ◽  
Author(s):  
Hélder Oliveira ◽  
Ana Fernandes ◽  
Natércia F. Brás ◽  
Nuno Mateus ◽  
Victor de Freitas ◽  
...  
Keyword(s):  
In Silico ◽  
Functional Foods ◽  
Therapeutic Strategies ◽  
Therapeutic Effects ◽  
Metabolizing Enzymes ◽  
The Past ◽  
The Matrix ◽  
Made In

Many efforts have been made in the past two decades into the search for novel natural and less-toxic anti-diabetic agents. Some clinical trials have assigned this ability to anthocyanins, although different factors like the food source, the amount ingested, the matrix effect and the time of consumption (before or after a meal) seem to result in contradictory conclusions. The possible mechanisms involved in these preventive or therapeutic effects will be discussed—giving emphasis to the latest in vitro and in silico approaches. Therapeutic strategies to counteract metabolic alterations related to hyperglycemia and Type 2 Diabetes Mellitus (T2DM) may include: (a) Inhibition of carbohydrate-metabolizing enzymes; (b) reduction of glucose transporters expression or activity; (c) inhibition of glycogenolysis and (d) modulation of gut microbiota by anthocyanin breakdown products. These strategies may be achieved through administration of individual anthocyanins or by functional foods containing complexes of anthocyanin:carbohydrate:protein.

The State of Democracy in Africa and the Middle East 2021: Resilient Democratic Aspirations and Opportunities for Consolidation

2021 ◽  
Author(s):  
Keyword(s):  
Middle East ◽  
North Africa ◽  
Democracy Assistance ◽  
The Past ◽  
Bright Spots ◽  
The World ◽  
Democratic Principles ◽  
Political Turmoil ◽  
Remarkable Progress ◽  
Made In

Recent declines in democracy have undermined some of the remarkable progress made in Africa over the past three decades, although bright spots remain. The Covid-19 pandemic, though seemingly less damaging to public health than elsewhere in the world, has added pressure on governance, rights, and social inequality. The report also covers the Middle East and North Africa which is one of the least democratic regions in the world. The Covid-19 pandemic has reinforced the erosion in democratic principles and the deepening authoritarianism that has accompanied a decade of economic, social and political turmoil in the region. This Report provides lessons and recommendations that governments, political and civic actors, and international democracy assistance providers should consider in order to counter the concerning trends in the erosion of democracy, and to foster its resilience and deepening.

The Creation of the International Thymic Malignancies Interest Group as a Model for Rare Diseases

2012 ◽  
pp. 471-474
Author(s):  
Frank C. Detterbeck
Keyword(s):  
Rare Disease ◽  
Interest Group ◽  
Scientific Collaboration ◽  
Rare Condition ◽  
Orphan Diseases ◽  
Rapid Progress ◽  
International Database ◽  
The Past ◽  
Novel Approaches ◽  
Made In

Overview: Similar to other orphan diseases, little progress has been made in the past decades in thymic malignancies. A determination to make a difference, despite the challenges facing a rare disease, led to the formation of the International Thymic Malignancies Interest Group (ITMIG) in 2010. This organization has brought together the majority of those focused on the management of thymic malignancies and has built a foundation for scientific collaboration, including consistent use of terms, an international database, and multidisciplinary engagement of clinicians and researchers from around the world. ITMIG has embarked on the development of novel approaches to research particularly suited to a rare condition. ITMIG has gained substantial recognition for the rapid progress that has been made and serves as a model for the advancement of knowledge in a rare disease.

scholarly journals Trends, patterns, and differentials in Canadian mortality over nearly a century, 1921-2011

2016 ◽  
Vol 43 (1-2) ◽  
pp. 48 ◽  
Author(s):  
Robert Bourbeau ◽  
Nadine Ouellette
Keyword(s):  
Life Span ◽  
Socioeconomic Factors ◽  
Health Trends ◽  
The Past ◽  
Demographic Indicators ◽  
Specific Mortality ◽  
Remarkable Progress ◽  
Made In

In the past 90 years, remarkable progress was made in substantially improving survival and longevity in Canada, establishing it as a member of today’s group of very low mortality countries. We use several demographic indicators of all-cause and cause-specific mortality to illustrate the nature of these major advances throughout the life span. Reviewing the literature on trends in Canadian mortality, we also uncover various challenges for the next decades. Reducing or even eliminating important disparities in mortality that persist with regards to geography and several socioeconomic factors is one of the greatest challenges ahead. Keywords: Mortality, longevity, health, trends, Canada 

An exon-centric perspective1Canadian Society of Molecular Biosciences (CSMB) Senior Investigator Award

2012 ◽  
Vol 90 (5) ◽  
pp. 603-612 ◽  
Author(s):  
Benjamin J. Blencowe
Keyword(s):  
Alternative Splicing ◽  
Computational Methods ◽  
Large Datasets ◽  
Regulatory Mechanisms ◽  
The Past ◽  
Human Genes ◽  
Insight Into ◽  
Remarkable Progress ◽  
Discrete Networks ◽  
Made In

During the past ten years, remarkable progress has been made in our understanding of the complexity and regulation of alternative splicing. The generation of large datasets of quantitative alternative splicing profiling information has revealed that transcripts from at least 95% of multi-exon human genes undergo alternative splicing, and that thousands of exons in mammalian transcriptomes are subject to striking regulatory patterns. Together with advanced computational methods, these datasets have enabled the inference of a predictive code for tissue-dependent alternative splicing. This code has further provided new insight into splicing regulatory mechanisms. Collectively, these approaches are revealing the existence of discrete networks of exons that are coordinately regulated in diverse biologically normal and disease contexts. A major challenge ahead is to systematically determine the functions of exons comprising these exon networks as well as the factors and mechanisms responsible for their regulation. This perspective provides an account of progress in these areas and also discusses future avenues of exon-centric exploration.

scholarly journals Therapeutic approach to actinomycosis: Experience gained at the Department of infectious and tropical diseases

2011 ◽  
Vol 64 (3-4) ◽  
pp. 207-210
Author(s):  
Natasa Katanic ◽  
Milorad Pavlovic ◽  
Ksenija Bojovic ◽  
Olga Dulovic ◽  
Eleonora Gvozdenovic ◽  
...  
Keyword(s):  
Infectious Disease ◽  
Rare Disease ◽  
Therapeutic Approach ◽  
Clinical Manifestations ◽  
Penicillin G ◽  
Tropical Diseases ◽  
Therapeutic Approaches ◽  
Gram Positive ◽  
Thoracic And Abdominal

Introduction. Actinomycosis is a chronic infectious disease caused by anaerobic, gram-positive microorganisms from the order of Actinomyce or Propionobacterium. The disease manifests itself mostly in cervicofacial form and less frequently in thoracic and abdominal form. The study was aimed at reviewing clinical manifestations and therapeutic approaches in treatment of patients with Actinomycosis. Material and methods. The study sample consisted of four patients with different clinical manifestations of Actinomycosis, who had been treated at the Institute for Infectious and Tropical Diseases in Belgrade in 2002, 2003, 2006 and 2008. Results. Four patients with Actinomycosis were treated during the above mentioned periods of time. One patient had kidney Actinomycosis, which developed into generalized Actinomycosis. Two patients had the most common cervical form, while the forth one had abdominal form; all patients received Penicillin G followed by Ampicillin, during the period of four weeks to up to a year. Conclusion. Actinomycosis is a rare disease, which imposes great diagnostic dilemmas. The treatment of this disease requires long term use of antibiotics.

Syntactic and rhythmic effects on downstep in Japanese

Phonology ◽  
1989 ◽  
Vol 6 (1) ◽  
pp. 39-67 ◽  
Author(s):  
Haruo Kubozono
Keyword(s):  
Experimental Evidence ◽  
Theoretical Research ◽  
The Past ◽  
Remarkable Progress ◽  
Made In

The past decade or so has seen increasing interest in prosodic research, and remarkable progress has been made in the study of accent and intonation, both empirical and theoretical. Research in Japanese intonation is no exception in this respect, as evidenced by such works as Poser (1984) and Beckman & Pierrehumbert (1986), among others, which have developed theoretical discussions on many interesting phonological issues on the basis of their own experimental evidence.

scholarly journals Az Alzheimer-kór heterogenitása

2021 ◽  
Vol 162 (25) ◽  
pp. 970-977 ◽  
Author(s):  
Nóra Balázs ◽  
Tibor Kovács
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Neurodegenerative Disorder ◽  
Clinical Manifestations ◽  
Symptomatic Therapy ◽  
Significant Progress ◽  
The Past ◽  
Atypical Manifestations ◽  
Recent Classification ◽  
Made In

Összefoglaló. A neurodegeneratív betegségek között az Alzheimer-kór a leggyakoribb kórforma. Morbiditása és mortalitása világszerte egyre gyorsabb ütemben növekszik, ezáltal szociális és gazdasági hatása is folyamatosan fokozódó terhet jelent a társadalomra. Az elmúlt néhány évtizedben jelentős előrelépés történt az Alzheimer-kór megismerésében, számos biomarker támogatja a diagnózis felállítását, tüneti terápiát szolgáló gyógyszerek kerültek bevezetésre. Az Alzheimer-kór klinikai megjelenése, lefolyása, viselkedése rendkívül változatos képet mutat, felismerése a rendelkezésre álló eszközök ellenére is kihívást jelenthet a nagy tapasztalattal bíró klinikusok számára is. Munkánk céljául tűztük ki, hogy összefoglaljuk az Alzheimer-kór genetikai, patológiai és klinikai jellemzőit, segítve ezzel a betegség jobb meg- és felismerését. Bemutatjuk a jelenleg érvényben lévő patológiai és klinikai irányelvek kritériumrendszereit, az újabb klasszifikációs szemléleteket. Részletesen ismertetjük az Alzheimer-kór heterogenitását genotípus és fenotípus szintjén egyaránt. Elemezzük a típusos és atípusos megjelenési formák jellemzőit, a társuló kórállapotoknak a megjelenésre és a progresszióra gyakorolt hatását. Orv Hetil. 2021; 162(25): 970–977. Summary. Alzheimer’s disease is the most prevalent neurodegenerative disorder. Morbidity and mortality of Alzheimer’s disease are increasing worldwide causing important social and economic burden on the society. Over the past few decades, significant progress has been made in the understanding of the pathogenesis of Alzheimer’s disease, several biomarkers support the diagnosis and drugs for symptomatic therapy had been introduced. The clinical manifestations and the course of Alzheimer’s disease have a variable picture, so – despite the diagnostic opportunities – its diagnosis could be a challenge for highly experienced clinicians as well. The aim of our work was to summarize the genetic, pathological and clinical characteristics of Alzheimer’s disease, thus helping to better understand and recognize the disease. We present the criteria systems of the currently valid pathological and clinical guidelines with the most recent classification approaches. The heterogeneity of Alzheimer’s disease at both genotype and phenotype levels is described in detail. The characteristics of typical and atypical manifestations and the effect of co-pathologies on the appearance and progression of Alzheimer’s disease are also discussed. Orv Hetil. 2021; 162(25): 970–977.

South Asian Developmental Crisis (Review Article)

1973 ◽  
Vol 12 (2) ◽  
pp. 181-188
Author(s):  
Rafiq Ahmad
Keyword(s):  
Second World War ◽  
World War ◽  
The Past ◽  
The Great Depression ◽  
Political Independence ◽  
Integrated Theory ◽  
Pass Through ◽  
Developed World ◽  
Second World ◽  
Made In

Like nations and civilizations, sciences also pass through period of crises when established theories are overthrown by the unpredictable behaviour of events. Economics is passing through such a crisis. The challenge thrown by the Great Depression of early 1930s took a decade before Keynes re-established the supremacy of economics. But this supremacy has again been upset by the crisis of poverty in the vast under-developed world which attained political independence after the Second World War. Poverty had always existed but never before had it been of such concern to economists as during the past twenty five years or so. Economic literature dealing with this problem has piled up but so have the agonies of poverty. No plausible and well-integrated theory of economic development or under-development has emerged so far, though brilliant advances have been made in isolated directions.

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