Quality of haemophilia treatment in Serbia: National haemophilia registry report

Introduction. The National Registry of patients with inherited bleeding disorders was established in 1963 and ever since it has been in charge of the Haemophilia Centre, Blood Transfusion Institute of Serbia, Belgrade. Objective. Purpose was to assess the quality of haemophilia treatment in Serbia from 2000 to 2008 based on the National Registry data related with the organization of care and quantities, and the choice of products. Methods. Analysis of data collected by the National Registry from January 2000 to December 2008. Results. The National Registry of patients with hereditary coagulopathy encompasses a database of 392 patients with haemophilia A (HA), 64 haemophilia B (HB), 217 von Willebrand?s disease and 19 with rare bleeding disorders. Treatment can be obtained in seven haemophilia treatment centres; haematological and paediatric institutes and hospitals in Belgrade, Nis and Novi Sad, as well as in other twenty local hospitals. From 2000 to 2003 about three million units of FVIII concentrate were administered annually, e.g. 0.25 IU/capita/year. Besides, national cryoprecipitate was available for the treatment. In 2003, National Haemophilia Committee was founded and centralized products supply was introduced. During 2004 and 2005, about five million units of FVIII concentrate were provided: annually, i.e. 0.65 IU/capita/ year. The choice of products was also improved. Namely, until 2004 the availability of DDAVP, antifibrinolytic drugs and rFVIIa concentrate was limited, while from 2004 these products became available for haemophilia treatment in Serbia. In order to improve haemophilia care we established international cooperation; education, training, consulting and participation in clinical and research projects. As the result, FVIII concentrate consumption in 2008 was 10.5 million units, e.g. 1.35 IU/capita/year. Conclusion. The considerable improvement of treatment is the result of efforts made by health care and regulatory institutions in Serbia. Significant support has been provided by cooperation within twinning programmes between Stockholm and Belgrade Haemophilia Centres in 2003-2004 and Hamilton and Belgrade Haemophilia Centres in 2005-2008.

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Gynaecological and obstetric care for women with inherited bleeding disorders

The Journal of Haemophilia Practice ◽

10.17225/jhp00055 ◽

2015 ◽

Vol 2 (2) ◽

pp. 20-21

Author(s):

Rezan Abdul-Kadir

Keyword(s):

Quality Of Life ◽

Quality Care ◽

Obstetric Care ◽

Hard Work ◽

Bleeding Disorders ◽

Von Willebrand

Abstract The issue of women with bleeding disorders was first reported by Professor von Willebrand in 1926, but it is only from around the mid 1990s that the issue has been fully recognised. Much of this is due to the vision and hard work of Professor Christine Lee and colleagues at the Katharine Dormandy Haemophilia Centre. This work has led to better diagnosis, better quality care and improved quality of life for women with bleeding disorders.

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Kidney Graft Survival in Italy and Factors Influencing it

Progress in Transplantation ◽

10.1177/152692480501500411 ◽

2005 ◽

Vol 15 (4) ◽

pp. 385-391 ◽

Cited By ~ 3

Author(s):

Orsola Pugliese ◽

Francesca Quintieri ◽

Dino Alberto Mattucci ◽

Sante Venettoni ◽

Emanuela Taioli ◽

...

Keyword(s):

Graft Survival ◽

Developed Countries ◽

National Registry ◽

Kidney Graft ◽

Allograft Function ◽

Recipient Age ◽

Transplant Registry ◽

Using Data ◽

National Registry Data

Purpose National registry data are often a suitable basis for examination of transplant outcomes. Using data supplied by the Italian National Transplant Registry, established in 1995, we performed the first nationwide analysis of this kind. Methods A retrospective analysis of 4893 recipients of cadaveric kidneys transplanted in all Italian centers from 1995 through 2000 was done to study 5-year graft survival. The association between some donor and recipient variables and outcomes in renal transplantation was analyzed. Graft survival was 93% at 3 months, 89% at 1 year, 82% at 3 years, and 80% at 5 years after transplantation. Results A significant association between graft survival and donor age (old vs young, relative risk [RR] = 1.62, 95% CI 1.27–2.06) and recipient age (old vs young, RR = 1.25, 95% CI 1.02–1.53). Graft survival was also associated with cold ischemia time (24–36 hours, RR=1.39, 95% CI 1.05–1.85 and >36 hours, RR=1.94, 95% CI 1.32–2.86 vs 0–24 hours) and donor/recipient sex mismatch (female/male vs male/male, RR=1.50, 95% CI 1.17–1.93). Conclusion The quality of kidney transplantation in Italy is satisfactory and is comparable to that in other developed countries. Furthermore, our experience confirms that both donor and recipient factors are major determinants of renal allograft function.

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Quality of periprocedural care in patients with von Willebrand disease

Haemophilia ◽

10.1111/hae.14389 ◽

2021 ◽

Author(s):

Craig D. Seaman ◽

Marnie Bertolet ◽

Jun Zhang ◽

Margaret V. Ragni

Keyword(s):

Von Willebrand Disease ◽

Von Willebrand

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Safety profile of clozapine: Analysis using national registry data in Japan

Journal of Psychiatric Research ◽

10.1016/j.jpsychires.2021.06.041 ◽

2021 ◽

Author(s):

Shinichi Imazu ◽

Takeo Hata ◽

Katsunori Toyoda ◽

Yoichiro Kubo ◽

Shigeru Yamauchi ◽

...

Keyword(s):

Safety Profile ◽

National Registry ◽

Registry Data ◽

National Registry Data

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Orchestration of Primary Hemostasis by Platelet and Endothelial Lysosome-Related Organelles

Arteriosclerosis Thrombosis and Vascular Biology ◽

10.1161/atvbaha.120.314245 ◽

2020 ◽

Vol 40 (6) ◽

pp. 1441-1453 ◽

Cited By ~ 4

Author(s):

Ellie Karampini ◽

Ruben Bierings ◽

Jan Voorberg

Keyword(s):

Endothelial Cells ◽

Intracellular Signaling ◽

Protein Complexes ◽

Biological Properties ◽

Bleeding Disorders ◽

Granule Formation ◽

Primary Hemostasis ◽

Granule Maturation ◽

Lysosome Related Organelles ◽

Von Willebrand

Megakaryocyte-derived platelets and endothelial cells store their hemostatic cargo in α- and δ-granules and Weibel-Palade bodies, respectively. These storage granules belong to the lysosome-related organelles (LROs), a heterogeneous group of organelles that are rapidly released following agonist-induced triggering of intracellular signaling pathways. Following vascular injury, endothelial Weibel-Palade bodies release their content into the vascular lumen and promote the formation of long VWF (von Willebrand factor) strings that form an adhesive platform for platelets. Binding to VWF strings as well as exposed subendothelial collagen activates platelets resulting in the release of α- and δ-granules, which are crucial events in formation of a primary hemostatic plug. Biogenesis and secretion of these LROs are pivotal for the maintenance of proper hemostasis. Several bleeding disorders have been linked to abnormal generation of LROs in megakaryocytes and endothelial cells. Recent reviews have emphasized common pathways in the biogenesis and biological properties of LROs, focusing mainly on melanosomes. Despite many similarities, LROs in platelet and endothelial cells clearly possess distinct properties that allow them to provide a highly coordinated and synergistic contribution to primary hemostasis by sequentially releasing hemostatic cargo. In this brief review, we discuss in depth the known regulators of α- and δ-granules in megakaryocytes/platelets and Weibel-Palade bodies in endothelial cells, starting from transcription factors that have been associated with granule formation to protein complexes that promote granule maturation. In addition, we provide a detailed view on the interplay between platelet and endothelial LROs in controlling hemostasis as well as their dysfunction in LRO related bleeding disorders.

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