The burden of inhibitors in haemophilia patients

2016 ◽  
Vol 116 (S 01) ◽  
pp. S10-S17 ◽  
Author(s):  
Christopher Walsh ◽  
Guenter Auerswald ◽  
Salvador Grancha ◽  
Victor Jiménez-Yuste

SummaryThe burden of disease in haemophilia patients has wide ranging implications for the family and to society. There is evidence that having a current inhibitor increases the risk of morbidity and mortality. Morbidity is increased by the inability to treat adequately and its consequent disabilities, which then equates to a poor quality of life compared with non-inhibitor patients. The societal cost of care, or `burden of inhibitors’, increases with the ongoing presence of an inhibitor. Therefore, it is clear that successful eradication of inhibitors by immune tolerance induction (ITI) is the single most important milestone one can achieve in an inhibitor patient. The type of factor VIII (FVIII) product used in ITI regimens varies worldwide. Despite ongoing debate, there is in vitro and retrospective clinical evidence to support the use of plasma-derived VWF-containing FVIII concentrates in ITI regimens in order to achieve early and high inhibitor eradication success rates.

2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Anjali Bansal ◽  
Laxmi Kant Dwivedi

Abstract Background According to United Nations, 19% of females in the world relied only on the permanent method of family planning, with 37% in India according to NFHS-4. Limited studies tried to measure the sterilization regret, and its correlated factors. The study tried to explore the trend of sterilization regret in India from 1992 to 2015 and to elicit the determining effects of various factors on sterilization regret, especially in context to perceived quality of care in the sterilization operations and type of providers. Data and methods The pooled data from NFHS-1, NFHS-3 and NFHS-4 was used to explore the regret by creating interaction between time and all the predictors. Predicted probabilities were calculated to show the trend of sterilization regret amounting to quality of care, type of health provider at the three time periods. Results The sterilization regret was increased from 5 % in NFHS-1 to 7 % in NFHS-4. According to NFHS-4, for those whose sterilization was performed in private health facility the regret was found to be less (OR-0.937; 95% CI- (0.882–0.996)) compared to public health facility. Also, the results show a two-fold increase in regret when women reported bad quality of care. The results from predicted probabilities provide enough evidence that the regret due to bad quality of care in sterilization operation had increased with each subsequent round of NFHS. Conclusion Many socio-economic and demographic factors have influenced the regret, but the poor quality of care contributed maximum to the regret from 1992 to 2015. The health facilities have seriously strayed from improving the health and well-being of women in providing the family planning methods. In addition, to public facilities, the regret amounting to private facilities have also increased from NFHS-1 to 4. The quality of care provided in the family planning operation should be standardized in every hospital to strengthen the health systems in the country. The couple should be motivated to adopt more of spacing methods.


Author(s):  
A. S. Chaudhry ◽  
E. L. Miller

That alkali treatments can improve the nutritional quality of poor quality roughages has long been established (Sundstol and Owen, 1984). However, their effectiveness is limited by their potential hazards to the animals and mankind. Alkaline hydrogen peroxide (AHP) has recently emerged as a possible substitute (Gould, 1985) but its farm scale application is limited by the need for high amounts of chemicals and water. Lack of any information regarding its effectiveness over NaOH alone is another factor which requires further investigation. The present study was, therefore, planned to assess the effectiveness of pH-regulated (11.5±0.2) H2O2 (AHP) in improving the in vitro dry matter digestibility (IVDMD) of wheat straw (WS, Avalon) under different laboratory conditions. The possibility of using CaO on its own or to regulate pH for AHP was also tested.


1948 ◽  
Vol 38 (3) ◽  
pp. 323-331 ◽  
Author(s):  
T. Mann

SUMMARY1. A method is described whereby fructose content and fructolysis can be assayed accurately in small samples of semen. The advantages of this method lie in its simplicity, accuracy and practical convenience as a tool for the assessment of semen quality, applicable also under field conditions.2. The content of fructose in fresh semen depends upon the secretory function of accessory glands which is influenced directly by the activity of the male sex hormone. A low level of seminal fructose may coincide with other symptoms of hormonal malfunction and poor quality of spermatozoa. A high level of seminal fructose indicates satisfactory functional ability of the accessory glands, but it does not necessarily coincide with high quality of spermatozoa as expressed in terms of density and motility.3. The normal level of fructose in fresh semen undergoes frequent fluctuations which can be observed if semen collections are made from the same individual at different times. Considerable variations in the sperm/fructose ratio may also occur in the semen of the same individual as illustrated by the results of an ‘exhaustion test’.4. Fructose disappears from semen incubated in vitro. The rate of fructose disappearance forms a convenient measure of sperm fructolysis. The normal rate of fructolysis in bull semen is 1·4–2 mg. fructose per 109 sperm cells in 1 hr. at 37° C. At this high level it can be maintained until almost the whole reserve of fructose has been exhausted. Azoospermic and necrospennic semen, as well as that from vasectomized animals, are unable to utilize fructose. A reduced rate of fructolysis is found in low quality semen of subfertile and infertile animals.5. The conditions of sperm survival in semen incubated in narrow tubes as used for the fructolysis test as well as for storage of semen in the practice of artificial insemination, are almost purely anaerobic. Under such conditions the survival of spermatozoa must largely depend upon fructolysis and not upon respiration.


2016 ◽  
Vol 116 (07) ◽  
pp. 32-41 ◽  
Author(s):  
Anja Schmidt ◽  
Kerstin Brettschneider ◽  
Jörg Kahle ◽  
Aleksander Orlowski ◽  
Karin Becker-Peters ◽  
...  

SummaryFollowing replacement therapy with coagulation factor VIII (FVIII), up to 30 % of haemophilia A patients develop FVIII-specific inhibitory antibodies (FVIII inhibitors). Immune tolerance induction (ITI) is not always successful, resulting in a need for alternative treatments for FVIII inhibitor-positive patients. As tolerance induction in the course of ITI appears to involve the formation of anti-idiotypes specific for anti-FVIII antibodies, such anti-idiotypes might be used to restore haemostasis in haemophilia A patients with FVIII inhibitors. We isolated antiidiotypic antibody fragments (scFvs) binding to murine FVIII inhibitors 2-76 and 2-77 from phage-displayed libraries. FVIII inhibitor/anti-idiotype interactions were very specific as no cross-reactivity with other FVIII inhibitors or isotype controls was observed. ScFvs blocked binding of FVIII inhibitors to FVIII and neutralised their cognate inhibitors in vitro and a monoclonal mouse model. In addition, scFv JkH5 specific for FVIII inhibitor 2-76 stained 2-76-producing hybridoma cells. JkH5 residues R52 and Y226, located in complementary determining regions, were identified as crucial for the JkH5/2-76 interaction using JkH5 alanine mutants. SPR spectroscopy revealed that JkH5 interacts with FVIII inhibitor 2-76 with nanomolar affinity. Thus, FVIII inhibitorspecific, high-affinity anti-idiotypes can be isolated from phagedisplayed libraries and neutralise their respective inhibitors. Furthermore, we show that anti-idiotypic scFvs might be utilised to specifically target inhibitor-specific B cells. Hence, a pool of anti-idiotypes could enable the reestablishment of haemostasis in the presence of FVIII inhibitors in patients or even allow the depletion of inhibitors by targeting inhibitor-specific B cell populations.


2015 ◽  
Vol 3 (1) ◽  
Author(s):  
Pratima

Family caregivers of persons with bipolar disorder and schizophrenia experience high level of burden and compromised quality of life. A considerable amount of burden on the caregivers often leads to display of certain attitudes towards persons with severe mental illness called expressed emotion, which then leads to poor quality of patients as well. Although numerous studies dealing with these issues separately are present, but studies dealing with relationship, using mixed methodology, among these issues are scarce. The aim of the present study was to understand how actually the construct of quality of life in different demographic conditions affect life conditions of schizophrenic and bipolar patients and determining relapse. The present study was designed mainly to assess the quality of life on patients and the families of a particular group of patients namely those with schizophrenia and bipolar disorder. The objectives if the present research were to study: (i) the quality of life of patients with Schizophrenia and Bipolar Affective disorder. (ii) the quality of life of caregivers of patients with Schizophrenia and Bipolar Affective disorder. Patients with disorders such as schizophrenia and bipolar affective disorder are more likely to relapse when there is high expressed emotion present in their living environment. The stress from the remarks and attitudes of the family is overwhelming because they feel like the cause of the problems. The patient then falls into the cycle of relapse. The only way to escape this vortex for the family is to go through therapy together to prevent the relapse. But before that it becomes necessary to understand that what is the reason behind such attitude towards a family member who is mentally ill, what is the cause of burden and what all changes the caregivers’ and the patients’ quality of life come across.


2018 ◽  
Vol 13 (3) ◽  
pp. 374-381
Author(s):  
Yudhiakuari Sincihu ◽  
W F Maramis ◽  
Muhammad Nur Rezki

Every human is getting old and we must attempt to be happy in the old time. The problem is 17.5% of elderly people have poor quality of life and 62.5% are sufficient. Elderly at this level needs immediate intervention because it will be a burden for families. communities and governments. This study aims to analyze the family participation on improving the quality of life. An observational study with cross sectional approach with population study at Posyandu Lansia Mekar Sari Surabaya. We used purposive sampling method. We took 54 samples elderly aged ≥ 60 years out of 96 elderly who enrolled in Posyandu year 2016. Data were obtained from WHOQoL-BREF and family role questionnaire. We analyzed the data with Rank Spearman’s Correlation. There was a positive correlation between the family role and the quality of life (p=0.000.. r=0.568). mainly by environmental domain (p=0.000. r=0.561). psychological domain (p=0.008. r=0.358). and social relations domain (p=0.011. r=0.345). but not for physical domain (p=0.154. r=0.917). The family participation such as adaptation. partnership. growth. affection. and resolve could be one method to improve the quality of life of elderly. Every increasing family value would add up to 26.3-43.2% assessment the quality of life.


2013 ◽  
Vol 1 (2) ◽  
pp. 30-34 ◽  
Author(s):  
Cesar Alberto Montaño ◽  
Maria Sol Cruz ◽  
Felipe Lemos ◽  
Alfredo Quiroz ◽  
Gerardo Rojas

Abstract The Latin American net of Prophylaxis and Immune Tolerance (RED LAPI) was established in 2010 and comprises a group of physicians dedicated to improving the diagnosis, treatment and quality of life of haemophilia patients in Latin America. The countries represented at RED LAPI are: Argentina, Chile, Uruguay, Venezuela, Colombia, Peru, Honduras, Guatemala, Paraguay, Dominican Republic, Bolivia, Ecuador and Panama. Analysis of the provision of care for haemophilia patients suggests a lack of consistent care both across and within Latin America countries. While some patients receive prophylaxis and immune tolerance induction (ITI), others are not even properly diagnosed, due to variation in patient’s health insurance. Few countries in Latin America have a national program that registers all patients’ information. Therefore, in many countries it is difficult to identify local, regional and national data regarding the number of diagnosed patients, type of hemophilia, severity, and the kind of treatment. With respect to patients with inhibitors, some countries rely on bypass agents for the treatment of bleeding episodes while a few are able to do ITI. This paper summarises available data obtained by a survey of RED LAPI members regarding the diagnosis and treatment of haemophilia in their countries, as well as the incidence of inhibitors and the treatments available to patients. Based on this analysis, the aim is to propose plans to improve the current situation of haemophilia patients in Latin America.


2019 ◽  
Vol 6 (1) ◽  
pp. 11-15
Author(s):  
Hari Krishna Saiju ◽  
Abhishesh Bajracharya ◽  
Brishav Rajbahak ◽  
Stuti Ghimire

Paulownia is a fast-growing woody tree, native to the forests of China. It belongs to the family Scrophulariaceae and is mainly used as a source of wood for furniture and musical instruments. Due to its fast-growing nature and high-quality of wood, there has been growing interest in cultivation and research of Paulownia in Nepal. Growth comparison was performed by measuring shoot length in in vitro condition. Among two species of Paulownia - Paulownia tomentosa (Thunb.) Steud and Paulownia fortuneii (Seem.) Hemsl., the growth rate of P. tomentosa was found to be 0.355 cm/week while that of P. fortuneii was found to be 0.637 cm/week in in-vitro conditions in MS medium supplemented with 0.1 mg/l NAA and 1mg/l BAP. Optimization of rooting methods was also performed, in which, sand rooting was found to be easier and more effective than in-vitro rooting. Dipping the plantlets in 1 mg/l of NAA was found to produce longer and denser roots than lower or higher concentrations during sand rooting.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Vasco Silva-Neves ◽  
Vitor Hugo ◽  
Paulo Alves ◽  
João Costa Amado ◽  
Carla Pais-Vieira ◽  
...  

AbstractOnychomycosis or tinea unguium (EE12.1) and Onychomycosis due to non-dermatophyte moulds (1F2D.5) (OM) is a fungal infection of the nail plates with a high prevalence that often affects vulnerable people with co-existing health problems. Gold standard pharmacological treatments for onychomycosis have been associated with low success rates and increasing antifungal resistance, suggesting that treatment outcome is dependent on multiple variables. Here, the prevalence of OM and quality of life were characterized in two vulnerable populations—Hospital patients and Homeless people. Comparing both groups, the most prevalent fungal species were identified in Hospital patients. Then, the in vitro fungicidal properties of the antiseptics povidone-iodine, polyhexamethylene biguanide-betaine, octenidine dihydrochloride, and a super-oxidized solution against two ATCC strains (Candida albicans and Aspergillus niger) and three clinical fungal isolates from Hospital patients (Candida parapsilosis, Trichophyton interdigitale, and Trichophyton rubrum) were tested. OM prevalence was high in both patient groups studied, who also reported a reduction in quality of life and concerns about the state of their feet. In addition, Hospital patients had a non-negligent therapeutic regimen management style. Antiseptics tested in vitro revealed antifungal properties. As antiseptics are low-cost and easy to apply and have few iatrogenic effects, the demonstration of fungicidal properties of these solutions suggests that they may constitute potential supportive therapeutics for OM.


Plants ◽  
2020 ◽  
Vol 9 (3) ◽  
pp. 317
Author(s):  
Yong-Goo Kim ◽  
Richard Komakech ◽  
Dae Hui Jeong ◽  
Yun mi Park ◽  
Tae Kyoung Lee ◽  
...  

Rehmannia glutinosa (Gaertn.) DC is a perennial plant belonging to the family Scropulariidae. The root of R. glutinosa is used in oriental medicine and mainly grown using rootstock rather than seed cultivation, which gives rise to several problems including root rot, and results in a low productivity and poor quality. To solve the challenges involved in R. glutinosa seed cultivation, our team previously used the formative features and genetic analysis of R. glutinosa to determine the optimal in vitro tissue culture conditions for producing sterile culture seedlings and rootstocks of R. glutinosa. The aim of the present study was to identify differences between R. glutinosa standard rootstock seedlings (SR), R. glutinosa culture rootstock seedlings (CR), and culture seedlings (CS) under field conditions. The reproductive characteristics of the aerial part were more robust while the area and length of leaves were smaller for SR than those for CR and CS. The characteristic that differed the most in SR was flowering, which did not occur in CR and CS. In addition, the fresh and dry weights of the subterranean parts of CR and CS were two-fold greater than those of SR. Fourier transform near-infrared (FT-NIR) analysis showed only slight differences between the chemical constituents of SR and its culture products, which was confirmed by measuring the content of catalpol, an indexing substance. Catalpol had a reduced content in the culture products compared to SR. However, this difference was not significant. Our findings will be useful for the identification of the best seedling type of R. glutinosa to enable its mass production.


Sign in / Sign up

Export Citation Format

Share Document