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Thalassemia intermedia is a clinical entity where anemia is mild or moderate, requiring no or occasional transfusion. Non-transfusion-dependent thalassemia encompasses 3 main clinical forms: beta-thalassemia intermedia, hemoglobin E/beta-thalassemia and alpha-thalassemia intermedia (HbH disease). Clinical severity of thalassemia intermedia increases with age, with more severe anemia and more frequent complications such as extramedullary hematopoiesis and iron overload mainly related to increased intestinal absorption. Numerous adverse events including pulmonary hypertension and hypercoagulability have been associated with splenectomy, often performed in thalassemia intermedia patients. The potential preventive benefit of transfusion and chelation therapies on the occurrence of numerous complications supports the strategy of an earlier therapeutic intervention. Increasing knowledge about pathophysiological mechanisms involved in thalassemia erythropoiesis and related iron overload is currently translating in novel therapeutic approaches.  相似文献   

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The purpose of this study, based on the Theory of planned behavior, is to identify and ultimately better understand what determines the fidelity and the regularity of donors committed in plasmapheresis donation. This qualitative study is a primary stage to establish a classification of donors in a French socio-cultural context. For this reason, we have carried out a survey among 16 regular plasmapheresis donors, by way of semi-structured individual interviews at the Établissement français du sang - Brittany. The level of commitment of these regular donors is considered as a level of appropriation. If subjective norms have initially influenced their decision to donate blood, no specific motivation has been highlighted for them to donate plasma except a generally favourable attitude towards the voluntary donation process. The perception of control over their environment is a variable which played a decisive role in the donor's intention to give, with little cost associated to plasmapheresis donation and recourse to internal causal explanations. A better comprehension of the plasmapheresis donors’ determinants should lead us to a more efficient awareness of new potential donors, thereby enhancing recruitment and retention. Due to the increasing need for plasma-derived products, this study's ultimate issue is closely correlated both with ethical and socio-economic aspects. Following which, after analysing the results, a certain number of recommendations will be made.  相似文献   

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Red blood cell immunization can lead to delays or even an impasse in a transfusion.

Objectives

Determine the specificities of the most common of alloantibodies and their associations to correct management of red blood cell transfused.

Methods and materials

A retrospective study between 2013 and 2015 in immunohematology laboratories at the Blood Transfusion Center of Rabat in Morocco. The following data were studied: frequency, specificities of alloantibodies, blood group involved in alloimmunization and difficult of management of transfusion in case with association of alloantibodies.

Results

Five hundred of alloantibodies were identified in 425 people (372 patients/pregnant women and 53 blood donors). The alloantibodies were directed against the following antigen: RH1 (50.8 %), RH3 (11.4 %), KEL 1 (8.2 %), RH2 (7.6 %), RH4 (4.6 %), MNS1 (4 %), MNS3 (2.6 %), Jka (2.4 %) and Fya (2.2 %). Only one alloantibody was identified in 85 % of cases. In 15 %, at least, two alloantibodies were found. The most common associations were directed against: anti-(D + C) (25), anti-(E + K) (4), anti-(E + c) (3) and anti-(D + C + E) (3). The rhesus system is the most involved in alloimmunization. Frequency of specific associations of alloantibodies was identified: Fya?/Jkb? (18.23 %), Fyb?/Jkb? (11.7 %), Jka?/S? (8.70 %), Jka?/Fyb? (5.20 %), Fyb?/s? (3.40 %) and Fyb?/Jkb?/s? (0.85 %).

Conclusions

Red blood cell immunization is a serious problem in transfused patients. This study proves the data of literature, the interest of using RH-Kel1 red cell units compatibles among women in age to procreate and for the transfused patients to reduce the rate of immunization. Associations of antibodies with low frequency suggest a promotion of donation.  相似文献   

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Aim

Our objectives were to assess the management of patients with major thalassemia and identify the various complications and monitoring means.

Patients and methods

A retrospective study was conducted on 26 β-thalassemic patients in the department of paediatrics, Hédi Chaker hospital, Sfax, Tunisia during a period of 25 years (from 1 January 1990 to 31 December 2014).

Results

The mean age of the beginning of transfusion was 11.5 months. That was with phenotyped red blood cells but not leukodepleted blood. Twenty-three patients received chelation. Before 2001, all patients received deferoxamine, poor adherence to this treatment was observed in 66% of cases. It was replaced by deferiprone since 2006 and deferasirox since 2009. A combination of 2 or 3 chelators was indicated for four patients. A total splenectomy was performed in 10 cases patients; it was due to hypersplenism. The bone marrow transplant was performed for one patient at the age of 9 year but it was rejected. Many complications were detected: endocrine complications (19 cases), immune complications (9 cases), gallbladder stones (5 cases), cardiac complications (4 cases), osteoporosis (3 cases), infectious complications (3 cases) and thromboembolic complications (2 cases). We noted some side effects related to chelation therapy in twelve cases. Four patients were dead.

Conclusion

Improving the medical care of homozygous β-thalassemic children requires adherence to transfusion regimen and chelation therapy. Bone marrow transplantation remains the only possible curative therapy, which must be promoted in our country.  相似文献   

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Complementary Universal Health Insurance (CMUC) which provides free access to health care has been available in France since 2000 for people with an annual income less than 60% of the poverty threshold. Hospitalization rates in 2009 for common diseases among immigrants were compared between beneficiaries of the general scheme under the age of 60 years with (4.5 millions) or without CMUC (34.1 millions) in 2008 and still alive at the end of the year. Data were derived from the French national health insurance reimbursements and short-stay hospital discharge databases. Age - and sex-adjusted hospitalization rates and relative risk significantly greater overall hospitalization rates (17.5% vs 13.2%) (males RR= 2.0, female RR 2.3) and each parasitic diseases (RR = 2.1), which include viral diseases and fevers of unknown origin (1.1/1000, RR =1.6), septicaemia (0.4/1000, RR = 2.2), HIV infection (0.7/1000, RR = 3.5), other infectious and parasitic diseases (0.7/1000, RR= 2.5) and, more precisely, measles (2.7/1000, RR = 5.0). Hospitalization for sickle cell disease (3%, RR = 4.5) were also more frequent as also for lead poisoning (0.12/1000, RR = 5.2). In this low-income population with free access to health care, hospitalizations were higher for many diseases that are targets for prevention and screening actions. This is tha case for immigrant with CMUC coverage arriving in France and when they travel to their country of origin.  相似文献   

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Bulletin de la Société de pathologie exotique - L’épidémie d’Ebola qui a sévit en Afrique de l’Ouest entre 2013 et 2016 a considérablement...  相似文献   

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《ITBM》2002,23(3):149-158
A numerical solution of pulsatile flow of two-phases non Newtonian fluid — Newtonian fluid through three different tubes (rigid, elastic, viscoelastic) is presented. The type of this flow exists in blood microcirculation. The iterative process with an implicit difference method is used to solve the both local and integral systems, and to determine the axial velocity profiles, the pressure, the flow rate and the radius distributions at all sections and at every moment.  相似文献   

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