Haemophilia Registry of the Medical Committee of the Swiss Haemophilia Society

2010 ◽  
Vol 30 (S 01) ◽  
pp. S15-S18 ◽  
Author(s):  
N. von der Weid

SummaryThe Swiss Haemophilia Registry of the Medical Committee of the Swiss Haemophilia Society started in 1996 but was set as an internet-based, double password-protected facility in the year 2000. With the inclusion of patients’ data from two new centres in 2009, we assume a coverage rate of about 90% of all patients with inherited bleeding disorders in our country. Data concerning the phenotype and genotype of the disorder, its severity, its therapy, the prevalence of inhibitors are readily available to the registered users, allowing quality control of haemophilia therapy at a national level, but also rapid care of the patient visiting the emergency room of another treatment centre. Basing on the available data, about two thirds of the WFH global survey can be answered; the mortality statistics shows that bleeding remains a cause of death in haemophiliacs, also in the 21th century. The Registry allows for comparisons with international datasets, especially with respect to treatment (prophylaxis vs. on-demand therapy), factor consumption and costs.

2009 ◽  
Vol 29 (S 01) ◽  
pp. S16-S18 ◽  
Author(s):  
B. Brand ◽  
N. von der Weid

SummaryThe Swiss Haemophilia Registry of the Medical Committee of the Swiss Haemophilia Society was established in 2000. Primarily it bears epidemiological and basic clinical data (incidence, type and severity of the disease, age groups, centres, mortality). Two thirds of the questions of the WFH Global Survey can be answered, especially those concerning use of concentrates (global, per capita) and treatment modalities (on-demand versus prophylactic regimens). Moreover, the registry is an important tool for quality control of the haemophilia treatment centres.There are no informations about infectious diseases like hepatitis or HIV, due to non-anonymisation of the data. We plan to incorporate the results of the mutation analysis in the future.


2012 ◽  
Vol 32 (S 01) ◽  
pp. S20-S24 ◽  
Author(s):  
N. von der Weid

SummaryThe Haemophilia Registry of the Swiss Haemo philia Society was created in the year 2000. The latest records from October 31st 2011 are presented here. Included are all patients with haemophilia A or B and other inherited coagulation disorders (including VWD patients with R-Co activity below 10%) known and followed by the 11 paediatric and 12 adult haemophilia treatment or reference centers. Currently there are 950 patients registered, the majority of which (585) having haemophilia A. Disease severity is graded according to ISTH criteria and its distribution between mild, moderate and severe haemophilia is similar to data from other European and American registries. The majority (about two thirds) of Swiss patients with haemophilia A or B are treated on-demand, with only about 20% of patients being on prophylaxis. The figure is different in paediatrics and young adults (1st and 2nd decades), where 80 to 90% of patients with haemophilia A are under regular prophylaxis. Interestingly enough, use of factor concentrates, although readily available, is rather low in Switzerland, especially when taking the country’s GDP into account: The total amount of factor VIII and IX was 4.94 U pro capita, comparable to other European countries with distinctly lower incomes (Poland, Slovakia, Hungary). This finding is mainly due to the afore mentioned low rate of prophylactic treatment of haemophilia in our country. Our registry remains an important instrument of quality control of haemophilia therapy in Switzerland.


2017 ◽  
Vol 17 (2) ◽  
pp. 112-118
Author(s):  
Taufik Suryadi

Abstrak. Kematian mendadak merupakan kematian yang terjadi pada 24 jam sejak gejala-gejala timbul, namun pada kasus-kasus forensik sebagian besar kematian terjadi   dalam   hitungan   menit   bahkan   detik   sejak   gejala   pertama   timbul. Dilaporkan laki-laki berusia 42 tahun   meninggal secara mendadak setelah mengalami kejang-kejang dan tidak sadarkan diri. Setelah dilakukan pemeriksaan oleh tenaga medis di Instalasi Gawat Darurat (IGD), pasien dinyatakan Death on Arrival (DOA). Dari hasil pemeriksaan luar dijumpai bintik kemerahan pada kelopak mata bagian dalam serta warna kebiruan pada ujung jari tangan dan kaki tanda-tanda terjadinya asfiksia. Sebab kematian adalah akibat kegagalan sistem kardiovaskuler yang terjadi secara mendadak. (JKS 2017; 2: 115-122)Kata kunci : Kematian mendadak, penyakit kardiovaskuler, aspek medikolegalAbstract. Sudden death is a death that occurred in the 24 hour  since symptoms arise, but in cases of forensic most deaths occur within minutes if not seconds since the first symptoms arise. Reported men aged 42 years died suddenly after suffering convulsions and unconsciousness. After examination by medical personnel  in  the  emergency  room,  the  patient  is  declared  Death  on  Arrival (DOA). From the results of external examination found red spots on the inside of the eyelid and a bluish color on the tip of the fingers and toes signs of asphyxia. Cause of death was due to failure of the cardiovascular system that occurs suddenly.(JKS 2017; 2: 115-122)Keywords: Sudden death, cardiovascular disease, medicolegal aspect;


2021 ◽  
Vol 21 (4) ◽  
pp. 371-383
Author(s):  
Václav Šmejkal

Abstract Distribution cartels in the automotive sector used to be frequently dismantled and sanctioned by the European Commission and the EU Courts still some 15 years ago. In recent years, however, only a few cases have been reported at the national level of EU Member States. Is it because the distribution of new cars really ceased to be a competition problem as the European Commission declared when it removed this part of the automotive business from the specific Block Exemption Regulation for the automotive sector in 2010? The purpose of the present analysis is first to inspect the car distribution cases that emerged in the EU after the year 2000 and, second, to speculate somewhat whether new forms of distribution, brought by the digitalization of marketing and sales, cannot bring about also new risks to cartel agreements and other types of distortions of competition in car sales.


1975 ◽  
Vol 21 (1) ◽  
pp. 87-92 ◽  
Author(s):  
Philip Whitehurst ◽  
Thomas V Di Silvio ◽  
Gaydzag Boyadjian

Abstract A computer program has been devised to select those clinical chemistry results that have a high probability of error for inclusion on a discrepancy report, which is printed on demand throughout the day. Each report entry is evaluated by a supervisor, who decides whether to accept the result or to re-assay. With this program, 8.4% of all results were included on the report, 1.9% were re-assayed, and 0.83% were judged to be in error and corrected. Checking results at the time of their release to the computer has led to earlier report delivery and more convenient timing of re-assays without compromise of patient safety.


2010 ◽  
Vol 30 (S 01) ◽  
pp. S35-S36
Author(s):  
H. Richter ◽  
H. Pollmann ◽  
B. Siegmund

SummaryBased on the documentation from patients with severe haemophilia B (FIX : C <1%) in home treatment the positive effect of continuous prophylaxis compared to on demand treatment was investigated over one year in a retrospective study from a single treatment centre (Centre for Haemostaseology Muenster).The advantage of the reduction in the number of bleeding episodes by 90% was opposed by a threefold higher consumption of FIX concentrates and a fourfold higher exposure of the patients to intravenous injections.


1996 ◽  
Vol 20 (8) ◽  
pp. 461-462 ◽  
Author(s):  
Riadh T. Abed

Since the Health of the Nation government document (Department of Health, 1992) set the target for the reduction of suicide in the severely mentally ill by 33% by the year 2000 it has become necessary to collect the suicide data for this population of patients at district and national level. There are a range of problems concerning the definition and identification of patients with severe mental illness as well as problems concerning the compilation of suicide data for this population at district level.


2013 ◽  
Vol 28 (2) ◽  
pp. 291
Author(s):  
Elmyra Ybáñez Zepeda ◽  
Maritel Yanes Pérez

En el año 2000, cinco millones de personas perdieron la vida por algún tipo de muerte violenta en el mundo. Las muertes por accidentes de tránsito llegaron a 1.2 millones; 815 mil personas cometieron suicidio y hubo 512 mil asesinatos. En México las muertes violentas han disminuido en general, pero se han incrementado las muertes por homicidio: los datos del Sistema Nacional de Información en Salud muestran que en el año 2000 éste ocupaba el noveno lugar entre las veinte principales causas de mortalidad y en 2010 llegó al quinto puesto. El objetivo central de este artículo es analizar si la condición de marginación  de un municipio urbano de los estados más violentos de México tiene relación con una alta tasa de homicidios. Los resultados de este estudio muestran que no existe una relación entre la tasa de homicidio y el grado de marginación. Otro de los hallazgos es que se observan tasas más altas e inestables en ciudades con fuerte crecimiento de la población o ubicadas en puertos y zonas de tránsito poblacional intenso. AbstractIn the year 2000, five million persons lost their life as a result of some form of violent death worldwide. Deaths due to traffic accidents totaled 1.2 million; 815,000 people committed suicide and there were 512,000 murders. In Mexico, although the overall number of violent deaths has decreased, the number of homicides has risen: Data from the National System of Health Information shows that in 2000 homicides were the ninth highest cause of death among the twenty main causes of mortality, and that by 2010 they were the fifth highest.  The main purpose of this article is to analyze whether the degree of marginalization of an urban municipality in the most violent states in Mexico is linked to a high degree of homicides. The results of this study show that there is no link between homicide rates and the degree of marginalization. Another of the findings is that higher, more unstable rates are observed in cities with rapid population growth or those located in ports and zones with an intense flow of people.


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