scholarly journals Current and Future Therapies for β-Thalassaemia: A Review Article

2021 ◽  
pp. 94-104
Author(s):  
Livia Giannoni ◽  
Emanuele Angelucci

This article will review recent and forthcoming advances in the treatment of thalassaemia. Prognosis of thalassaemia has dramatically improved in the last 50 years with the development of regular and safe blood transfusions and iron chelation. Almost 20 years ago, development of oral chelators, and more recently the improvement in the knowledge and understanding of iron pathophysiology, have led to optimal iron toxicity prevention and treatment. These considerable advancements in medical therapy have transformed transfusion-dependent thalassaemia from a lethal childhood disease to a chronic disease with an open prognosis, even in those individuals over 50 years of age, and with the disease being, in some instances, curable. In the 1980s, the introduction of allogeneic haematopoietic cell transplantation provided the possibility of curing the congenital disease for the first time. More recent developments include an improved understanding of erythropoiesis, which led to the development of new erythroid-stimulating factors effective in thalassaemia, an expansion of donor pull for transplantation, and the approach of the long-term promised gene therapy in clinical practice. Moreover, ongoing trials of gene editing and agents modulating iron metabolism promise new improvements. Today, patients with thalassaemia have several weapons in their therapeutic arsenal and, hopefully, will have much more to come. As usual in medical practice, new advancements provide new challenges for the medical community, and it is the duty of this community to clearly understand the benefits and challenges of any new approach in order to provide the highest clinical benefit to patients.

Author(s):  
Brian Haller ◽  
James Anderson

This paper details some of the recent developments that ALSTOM has made, in collaboration with QinetiQ, to develop and test highly loaded high pressure turbine stage designs. A new approach was used for the preliminary design of the stage (adapted from the Denton U3 method) and this is described. For the first time the standard empirical loss correlations were abandoned. The targets for the stage are very aggressive. The stage incorporates many innovative features to address aerodynamic and other parasitic losses, including “Controlled Flow” technology to address secondary flow losses. This new approach is then validated against detailed steady performance measurements. Additional unsteady traverse measurements taken have been used to validate the predicted rotor flow field. These unsteady traverse measurements have also been used to resolve the unsteady flow field. Although detailed interpretation of these results has not yet been completed, some understanding is drawn from these results.


Author(s):  
Muhas C. ◽  
Naseef P. P.

Diabetes mellitus is a chronic illness that requires continuing medical care and ongoing patient self-management education and support to prevent acute complications and to reduce the long-term complications. Moderate to severe maternal hyperglycemia in pregnancy has unique diabetes-related risks to mother and her unborn baby. So Gestational Diabetes mellitus (GDM) is a carbohydrate intolerance that is not diabetes that has developed or been discovered for the first time during pregnancy. Approximately 7% of pregnancies are affected by GDM. Patient with GDM are at higher risk for excessive weight gain, preeclampsia, and cesarean sections. Infants born to mothers with GDM are at higher risk for macrosomia, birth trauma, and shoulder dystocia. After delivery, these infants have a higher risk of developing hypoglycemia, hypocalcemia, hyperbilirubinemia, respiratory distress syndrome, polycythemia and subsequent obesity and type 2 diabetes. So the management of GDM is very important, and its management remains a challenge for the obstetricians and endocrinologists. MNT is the most common therapy which suffices for GDM, but when required. The pharmacological treatment becomes necessary, and the treatment of choice is human insulin. OHAs have also reached the high tables in the management of GDM. Glyburide and metformin have been found to be safe, effective and economical for the treatment of gestational diabetes. Let us join hands to manage the GDM effectively, not only for the present generation but also for the generations to come.


2007 ◽  
Vol 04 (04) ◽  
pp. 393-413 ◽  
Author(s):  
FRANCES T. J. M. FORTUIN ◽  
S. W. F. (ONNO) OMTA

In large technology-based firms, especially in long life cycle industries, often a tension exists between corporate R&D and the business unit (BU) customers. The long term R&D orientation needed to come to the more radical (even disruptive) innovations for the long term survival of the prospector firm being at odds with the need of the BUs for more incremental "sustaining" innovations for their day-to-day activities. This paper takes a new approach to this problem by analyzing the corporate R&D to business relationship from a customer value perspective by identifying R&D flexibility, R&D communication, strategic alignment and R&D performance as the main attributes of the value map of the BU customers of corporate R&D. We then present the Cusvalin instrument (Customer Value Learning in INnovation) that was constructed to overcome the R&D to business incongruence by providing feedback on the gaps between the value maps of R&D and their BU customers. This instrument has been tested in a longitudinal survey from 1997 through 2002 (696 respondents) in a large technology-based supplier company (±30 000 employees world wide). It is concluded that the Cusvalin model is an effective instrument to monitor the strategic alignment of R&D and the BUs, and ultimately leads to better R&D performance, as perceived by the BU customers. From the longitudinal analysis it is concluded that a system that balances radical innovation (via Technology Board-funding, in which R&D management, headquarters, and BU directors jointly decide on long-term radical R&D projects) and incremental innovation (via BU unit-funding) is effective in providing strategic alignment between R&D and business.


1978 ◽  
Vol 3 (3) ◽  
pp. 415-444 ◽  
Author(s):  
Jane M.O. Sharp

This paper argues that the 1978 United Nations Special Session on Disarmament (UNSSOD) provides an ideal opportunity for governments to consider the feasibility of national initiatives in disarmament as distinct from relying primarily on negotiated agreements. Traditional arms control diplomacy, inadequate at best, may be completely misdirected. Instead of building confidence and reducing force levels, the bargaining process inherent in the pursuit of formal international treaties tends not only to exacerbate tensions but also to generate more armaments. Extra forces have been rationalized to support particular negotiating positions, and defense planners have been encouraged to assess military requirements in relative terms, as a function of worst-case estimates of the capabilities and intentions of potential adversaries, rather than in absolute terms, as a function of basic national security needs. An attempt is made here to establish criteria on which effective disarmament initiatives might be based and judged in the long term. While these criteria are intended to be generally applicable, the leadership for any new approach to the armaments problem will probably have to come from Washington; specific proposals thus focus on measures which appear feasible for the US in the near future, and which the Carter Administration could conceivably initiate at the UNSSOD.


Author(s):  
I. A. Dolmatov ◽  
I. YU. Zolotova ◽  
I. V. Maskaev

For the last several years, the Russian Federation has been artificially restraining the growth of tariffs for the services of natural monopolies. A simple decision, which is taken hastily a few years ago as a short-term anti-crisis measure, has every chance of becoming a "solution" for many years to come. The authors attempt to analyze the short-term and long-term effects of the tariff regulation and present the results obtained in the framework of the basic research program of the Higher School of Economics on the impact of tariffs in the energy sector on the main macroeconomic indicators, most often used as arguments in favor of tariff restraint. It is shown that tariffs, including the ones for electricity networks, should cover all efficient costs of regulated companies in full which enables reliable and safe power supply for sustainable and efficient development of industries and wellbeing of the population. The authors present for the first time the results of the estimations of the efficiency of the operational costs of electric networks in Russia obtained on the models developed by the Institute of Pricing and Regulation of Natural Monopolies. The models are based on the data envelopment analysis (DEA) technique, one of the most advanced and most common methods in contemporary tariff setting.


2007 ◽  
Vol 68 (3) ◽  
pp. 314-324 ◽  
Author(s):  
Alison J. Blyth ◽  
Asfawossen Asrat ◽  
Andy Baker ◽  
Pauline Gulliver ◽  
Melanie J. Leng ◽  
...  

AbstractA hundred-year stalagmite lipid biomarker record from Mechara, southeastern Ethiopia, is presented. The record has been recovered at a 10-yr temporal resolution, marking the first time this has been achieved in stalagmite biomarker work and providing the first opportunity to investigate the relationship between stalagmite lipid records and hydrological transport lags, a vital issue in interpreting palaeoenvironmental signals. Preserved plant-derived n-alkanes and n-alkanols show clear changes in composition over time, relating to known land-use changes in the area, particularly the expansion of agriculture in the early twentieth century. The level of environmental detail provided by this technique, combined with the long-term chronological framework offered by stalagmites, holds significant promise for the investigation of early human environments and their associated climatic and anthropogenic controls.


2010 ◽  
Vol 52 (3) ◽  
pp. 387-401 ◽  
Author(s):  
Hiromasa Suzuki

This article reviews the recent developments of employment relations in Japan. The period 2008—9 has been rich in dramatic changes. First, there was the financial and economic crisis of 2008 which dealt a serious blow to the fragile Japanese economy. Second, in September 2009, a new government led by the Democratic Party of Japan was elected for the first time since 1955. The traditional Japanese employment relations system with long-term employment still applies to core workers in large firms, but their share has been diminishing in favour of the continual growth of atypical employment or non-regular employees.


Author(s):  
S.J. Krause ◽  
W.W. Adams

Over the past decade low voltage scanning electron microscopy (LVSEM) of polymers has evolved from an interesting curiosity to a powerful analytical technique. This development has been driven by improved instrumentation and in particular, reliable field emission gun (FEG) SEMs. The usefulness of LVSEM has also grown because of an improved theoretical and experimental understanding of sample-beam interactions and by advances in sample preparation and operating techniques. This paper will review progress in polymer LVSEM and present recent results and developments in the field.In the early 1980s a new generation of SEMs produced beam currents that were sufficient to allow imaging at low voltages from 5keV to 0.5 keV. Thus, for the first time, it became possible to routinely image uncoated polymers at voltages below their negative charging threshold, the "second crossover", E2 (Fig. 1). LVSEM also improved contrast and reduced beam damage in sputter metal coated polymers. Unfortunately, resolution was limited to a few tenths of a micron due to the low brightness and chromatic aberration of thermal electron emission sources.


VASA ◽  
2001 ◽  
Vol 30 (Supplement 58) ◽  
pp. 6-14 ◽  
Author(s):  
Edmonds ◽  
Foster

The diabetic ischaemic foot has become an increasingly frequent problem over the last decade. However, we report a new approach consisting of a basic classification, a simple staging system of the natural history and a treatment plan for each stage, within a multi-disciplinary framework. This approach of "taking control" consists of two parts: 1. long-term conservative care including debridement of ulcers (to obtain wound control), eradication of sepsis (micribiological control), and provision of therapeutic footwear (mechanical control), and 2. revascularisation by angioplasty and arterial bypass (vascular control). This approach has led to a 50% reduction in the rate of major amputations in patients attending with ischaemic ulceration and absent foot pulses from 1989 to 1999 (from 4.6% to 2.3% per year). Patients who underwent angioplasty increased from 6% to 13%. Arterial bypass similarly increased from 3% to 7% of cases. However, even with an increased rate of revascularisation, 80% of patients responded to conservative care alone. This,we conclude, is an essential part of the management of all patients with ischaemic feet.


2017 ◽  
Vol 6 (1) ◽  
pp. 153-168
Author(s):  
Celal Hayir ◽  
Ayman Kole

When the Turkish army seized power on May 27th, 1960, a new democratic constitution was carried into effect. The positive atmosphere created by the 1961 constitution quickly showed its effects on political balances in the parliament and it became difficult for one single party to come into power, which strengthened the multi-party-system. The freedom initiative created by 1961’s constitution had a direct effect on the rise of public opposition. Filmmakers, who generally steered clear from the discussion of social problems and conflicts until 1960, started to produce movies questioning conflicts in political, social and cultural life for the first time and discussions about the “Social Realism” movement in the ensuing films arose in cinematic circles in Turkey. At the same time, the “regional managers” emerged, and movies in line with demands of this system started to be produced. The Hope (Umut), produced by Yılmaz Güney in 1970, rang in a new era in Turkish cinema, because it differed from other movies previously made in its cinematic language, expression, and use of actors and settings. The aim of this study is to mention the reality discussions in Turkish cinema and outline the political facts which initiated this expression leading up to the film Umut (The Hope, directed by Yılmaz Güney), which has been accepted as the most distinctive social realist movie in Turkey. 


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