ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00078.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00079.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Summary.Factor XI deficiency is a rare autosomally transmitted coagulopathy that is associated with a variable bleeding tendency. Recently there have been reports of thrombotic events following the administration of a virally inactivated factor XI concentrate (BPL) to factor XI deficient patients. We have therefore reviewed a single centre's experience of the use of factor XI concentrate over a 6‐year period and compared this to our previous experience of either no treatment or treatment with fresh frozen plasma (FFP) in 103 patients.There were 156 procedures performed without haemo‐ static cover. The incidence of bleeding was greatest following tonsillectomy (71%) and dental extraction (Sl'h). There was a trend for bleeding complications to be associated with lower levels of factor XI but patients with all levels of factor XI suffered bleeding complica‐ tions. There were 38 procedures carried out under FFP cover, with only one patient suffering excessive bleeding and no serious complications.Factor XI concentrate was given to 25 patients to cover 45 episodes. There were no bleeding complications. Three patients suffered serious complications. One patient, with a previous history of cardiovascular disease, died of a myocardial infarction and a second had an ischaemic episode resulting in a %day hospital admission. These episodes both occurred on the same day as the factor XI infusion. A third patient suffered bilateral pulmonary emboli 7 weeks after a prolonged course of factor XI concentrate.These finding suggest that factor XI concentrate should be contraindicated in patients with a history of cardiovascular disease, when FFP should be used. Guide‐ lines for the use of factor XI concentrates should be revised, and work performed to establish the mechanism of these thrombotic

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00080.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Summary.Objectiues. To evaluate haemophilia patients' knowledge and understanding of factor concentrate products and to assess their feelings regarding purity and viral safety of these products.Methods. Sixty questionnaires were distributed and completed by patients or parents of patients with severe/ moderate haemophilia A at two large comprehensive haemophilia centres in the USA and Italy.Results. Most patients knew what brand of factor concentrate they used, but less than half were aware of how their concentrate was virally inactivated. The majority of responders perceived recombinant factor VlII as being the concentrate with the highest degree of purity. The majority felt the decision of which concentrate to use should be the care‐givers not the patients.Conclusions. It appears that patients need more education concerning the concentrate they are using, and its method of viral inactivation and purit

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00081.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Summary. To evaluate the frequency and potency of inhibitor formation based on the product used, we retrospectively reviewed the records of 99 children with various types and severity of haemophilia (haemophilia A 82, severe 46; haemophilia B 10, severe 6; vWD 7) treated for the last 20 years. After a mean observation period of 8 years an overall of 23 patients (23.2′/0) developed an inhibitor (haernophilia A 26.8%; severe 4O%, moderate 20%, mild 3.8%). None of the haemophilia B patients presented with an inhibitor, and only one child with bevere vWD (1/7, 14.3%) showed a transient inhibitor under cryoprecipitate therapy. Inhibitor titre was low (<5 BU) in most cases (91.3%) and in only two patients (8.75%) was 6 and 8 BU respectively. Antibodies to FVIII were transient (detected only once) in four (17.4%) and intermittent in 19 patients (82.6%). By the age of 12 years, 17/23 patients (73.9%) had demonstrated an inhibitor. The inhibitor detection seemed to be higher in the groups of patients exposed to monoclonal (3115, 20%), SID‐treated (10159, 16.9%) or H/T FVIII concentrates (6/41, 14.6%), compared to groups of patients who received cryo/plasma (9.5%) or unmodified concentrates (5.1%); nevertheless the differences were not statistically significant.Surprisingly, none of the 52 patients who received a S/D + chromatography‐treated factor VIIl concentrate developed an inhibitor after a mean observation period of 1.7 years (range 0.2–2 years). The overall prevalence of inhibitor formation in previously untreated haemophiliacs was 14.3% (4/28), irrespective of the product used.Our data indicate that a high proportion of our haemophilic children exposed to several products of various purities have developed a low‐titre inhibitor which in most cases was transient or intermittent. However, despite the presence of the antibody, none of the patients needed a change in the mode of

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00082.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Summary. Twenty‐four of 117 cases of haemophilia A (20.5%) and none of 18 cases of haemophilia B reported in this study had an antibody to the human immuno‐deficiency virus (HIV). Both groups of patients showed similar immunological alterations. HIV‐seropositive haemophilia A patients had an increased CD8 cell count and a similarly decreased CD4/CD8 ratio as compared to HIV‐seronegative haemophilia A patients. Multiple regression analysis for the association of CD4/CD8 ratio with HIV infection status and dosage of plasma products in haemophilia A and B patients, respectively, revealed that there was a significant negative association of ln(CD4/CD8) with dosage of factor VlII concentrates (P = 0.0435) and factor IX concentrates (P = O.O028), respectively. N o association occurred between CD4/CD8 ratio and HIV infection as well as dosage of other plasma products. These data indicate that the immunological abnormalities of our haemophilia A and B patients in their early years were primarily caused by various viral infections and/or a suppressive effect of allogeneic protein through infusion of factor concentrates and not caused simply by HIV in

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00083.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Summary.We have used the polymerase chain reaction technique for the detection of hepatitis C RNA in nine different plasma‐derived factor VllI concentrates and in two factor IX concentrates. Four concentrates were investigated both prior to and after the introduction of donor screening for hepatitis C antibodies. A negative reaction was consistently found in the ultra‐pure factor VIII concentrates Octonativ‐M (Pharmacia) and Hemofil M (Baxter), both prepared by affinity purification with factor VI1I:C monoclonal antibodies and virus inacti‐ vated hy solvent/detergent procedures, as well as in both the low‐purity factor IX concentrates. I f produced from unscreened plasma, the other factor VIII concentrates manifested positive reactions irrespective of preparation procedure and type of virus inactivation or the temperature at which it was performed. We conclude that the preparation procedure of clotting factor concentrates, rather than type of virus inactivation, determines the degree of contamination by hepatitis C virus RNA, and that screening of source plasma seems effective in removing hepatitis C RNA from the final product as determined with a sensitive PCR method. I t is important to stress that the presence of viral RNA does not necessarily imply clinical in

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00084.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryThis study aimed to assess the psychological status of men with haemophilia and HIV infection and to monitor changes in psychlogical status over time, in order to evaluate the need for psychological support. The study included 24 HIV seropositive men and a control group of 21 HIV seronegative men who attended the Haemophilia Centre in Bari (Italy). Subjects underwent psychological tests (STAl‐Y: State and Trait Anxiety Inventory; SDS: Self‐Rating Depression Scale) and completed a questionnaire on the emotional impact of AIDS. Assessment was repeated at 6‐monthly intervals over a 2‐year period. Contrary to expectation, HIV seronegative men with haemophilia had worse anxiety and depression scores, reported more confusion and fear, and had more reluctance towards the use of blood products (despite their present safety) than HIV seropositives. Possible reasons for these findings are considered, and their implications for clinical practice di

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00085.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryResection of the radial head and partial open synovectomy of the elbow have been used in the treatment of repeated haemarthroses of the elbows in 15 patients with haemophilia. The indications for the index operation were: severe pain and bleeding in the elbow that occurred in spite of appropriate, episodic, replacement therapy over a period of more than 6 months, associated with hypertrophy of the radial head and a significant loss of pronation‐supination.The long‐term results were assessed retrospectively according to the classification recommended by the Orthopaedic Advisory Committee of the World Federation of Hemophilia. In the operated group, three patients had a good result, seven were fair, and five poor. It is concluded that this procedure appears to reduce the incidence of haemarthrosis but did not slow the rate of evolution of radiographic chan

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00086.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryWe treated a 20‐month‐old boy with severe haemophilia A who developed a high level factor VIII (FVIII) inhibitor. An implantable intravenous access device (IVAD) was safely placed on the lateral chest under anaesthesia using plasma‐derived factor VIIa (FVIIa). Implantation of an IVAD into a haemophilic child with an inhibitor requires considerable care, because the area of subdermal invasion is broader than in the implantation of a central venous line. However, placement of an IVAD provides very convenient access to the central venous line and is an appropriate tool for frequent injection, such as the induction of immunotolerance to

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00087.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY