ISSN:1424-8832    

DOI:10.1159/000216325

出版商:S. Karger AG    

年代:1992

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000216326

出版商:S. Karger AG    

年代:1992

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000216327

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

In type III von WiUebrand disease (vWD) patients, the bleeding time was only partially corrected or not modified after cryoprecipitate infusion, although the levels and the multimeric structure of plasma von WiUebrand factor (vWF) were normal. However, the adhesion of normal platelets on the vessel wall subendothelium in the presence of postinfusion patient plasma improved more significantly than the bleeding time. These results suggest a role of the vWF released from normal platelets which is absent in type III vWD platelets. In 5 patients transfusion of normal platelet concentrates performed 1 h after cryoprecipitate infusion without modification of the bleeding time &(> 30 min) normalized this parameter, and platelet adhesion to the subendothelium elicited a marked improvement. These last results confirm the suggestion that platelet vWF plays an important ‘in vivo’ role in the hemostatic process, particularly in patients suffering from severe

ISSN:1424-8832    

DOI:10.1159/000216328

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

The research on von Willebrand’s disease performed in Sweden and Finland since the late 1920s is reviewed. The difficulties in diagnosing mild von Willebrand’s disease, due to intra-individual variability, is discussed in relation to making correct pedigrees for genetic studies. As shown by some examples, new techniques for genetic studies will eliminate such difficulties and lead to better understanding of the pathogenesis.

ISSN:1424-8832    

DOI:10.1159/000216329

出版商:S. Karger AG    

年代:1992

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000216330

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Within the past 20 years, home therapy has become an accepted treatment for the majority of people with severe haemophilia in the developed world. The intravenous administration of blood products by suitably trained patients or their relatives has proved to be safe and effective. It allows for the early treatment of bleeding episodes before the appearance of physical signs. It results in reduced morbidity, especially in terms of reduction in long-term arthropathy, and in reduced socio-economic handicap. It is cost effective both in terms of savings in time previously lost from school or work and reduction of reliance on expensive hospital facilities. Home therapy forms a vital part of the overall comprehensive care of people with haemophilia and their families.

ISSN:1424-8832    

DOI:10.1159/000216331

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Between 1978 and 1989, a controlled study was performed on children and adolescents suffering from severe haemophilia A. Special attention was given to long-term therapy adjusted to the patients’ requirements. The clinical and the X-ray scores of the Orthopaedic Advisory Committee of the World Haemophilia Society were used to assess the orthopaedic findings. The present investigation is a 12-year follow-up study of 90 patients affected with severe forms of haemophilia A. All knee and ankle joints of the patients were evaluated. After 12 years, the clinical scores remained unchanged or had been improved (knees 94%, X-ray score 97, ankles 86, X-ray score 88).

ISSN:1424-8832    

DOI:10.1159/000216332

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Between 1960 and 1991, 156 episodes of central nervous system (CNS) bleeding were documented in 106 patients from a total population of 1,410 hemophiliacs (7.5%). Ninety-one hemophilia A patients presented 131 bleeding episodes; 15 hemophilia B patients had 25 episodes. 32% of these episodes took place in patients less than 5 years of age. 46 % were age 10 or less, and 72% were age 20 or less. The mean age was 14.8 years in hemophilia A and 9 years in hemophilia B patients. A significant increase in the mean age of hemophilia A patients has been observed over the last 10 years; this may be related to HIV infection. A history of recent trauma was documented in 39.7% of the episodes. Spontaneous CNS bleeding was predominant in severe hemophilia (85.2%). One hundred and fifty-four CNS bleeding episodes were intracranial and 2 intraspinal. Of the intracranial episodes, 37.7% were subarach-noid, 29.8 subdural, and 22.7% intracerebral. Factor VIII or IX inhibitors were present in 11.3% of the patients; this figure is slightly lower than that observed in our total hemophilic population. Over 50% of the patients had psychoneurological sequelae; the most frequent were seizure disorders and motor impairment. The overall mortality rate was 29.2%. The mortality was more closely related to the CNS bleeding site than to the severity of hemophilia. Treatment should be based on prompt and prolonged replacement therapy to ensure hemostatic levels of antihemophilia factors.

ISSN:1424-8832    

DOI:10.1159/000216333

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Management of patients with factor VIII (and IX) inhibitors includes management of acute bleeds and methods to induce immune suppression and tolerance and to detect patients at risk of developing inhibitors. The methods used over the years to treat acute bleeding have been more or less successful. The best method is to raise factor VIII levels by human or porcine factor VIII concentrate, but this is not usually possible. Prothrombin complex concentrates, activated or non-activated, have enjoyed some success as factor VIII by passing agents, but the development of recombinant activated factor VII represents a new and promising method of inducing haemoslasis at the site of bleeding whilst minimizing the risk of disseminated intravascular coagulation. Alternatively, the use of tissue factor is under consideration to exploit the extrinsic system. Methods to induce immunological tolerance by use of the ‘Bonn’ regime or by the introduction of immunomodulation with the ‘Malmö’ regime of extracorporeal immunodepletion, cyclophosphamide, and intravenous immunoglobulin continue to be attempted with significant but variable success. Gradually the inhibitor problem is being contained, but it is still an important complication of haemophilia therapy.

ISSN:1424-8832    

DOI:10.1159/000216334

出版商:S. Karger AG    

年代:1992

数据来源: Karger