ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00031.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.tb00032.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.tb00033.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryBetween 1980 and 1994, 169in vivorecovery studies were performed in one haemophilia centre on casually‐recruited outpatients with severe haemophilia A, and 149 early‐phase half‐life studies were performed on patients scheduled for elective surgery. Average recoveries improved from 80% to 97% of the expected values over the study period. No relationship of recovery to level of product purification was seen. In the disappearance studies, plasma factor VIII levels fell to half the peak levels by 3.25h after infusion, on av

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00034.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryThe haemostatic efficacy of a new highly purified factor IX concentrate, prepared by metal chelate affinity chromatography, was assessed in 13 patients with haemophilia B undergoing a variety of surgical operations. Four of the patients had developed post‐operative thromboembolic complications following previous operations, when treated with a prothrombin complex concentrate. None of the patients in the present series developed any evidence of post‐operative thrombotic complications. Effective haemostasis was achieved in all patients, with the exception of a surgical bleed in one case, and late post‐operative bleeding in a second patient when the factor IX activity fell below 20iu/dl. The product is treated with a solvent‐detergent process that destroys lipid‐enveloped viruses, while the affinity chromatography process during manufacture removes in excess of 4 log10of a non‐lipid‐enveloped virus. In follow‐up studies, none of the patients has shown evidence of fresh infection from the concentrate, when assessed by virological markers. It is concluded that this high‐purity concentrate (tradenane ‘Replenine’) is effective for the treatment of patients with haemophilia B who underg

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00035.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryWe report our clinical experience in the immune tolerance (IT) therapy of 21 paediatric haemophiliacs with FVIII inhibitor: high responders (16HR) received initially FVIII twice daily at a dosage of 50–300 U/kg/day, 11/16 received a concomitant treatment with activated prothrombin complex concentrate (100–200 U/kg/day). Low responders (five LR) received 20–100 FVIII U/kg every second or third day. Inhibitor elimination was achieved in 19/21 patients in a median time of 4 months in HR and 1.5 months in LR. The outcome and length of time needed to induce IT was significantly correlated with FVIII exposure between the first inhibitor detection and onset of IT therapy and to interruption of IT therapy. For a rapid elimination of FVIII inhibitors it is important to start continuous administration of high‐dose FVIII (≥ 100 FVIII U/kg/day) before repeated exposure to FVIII, in order to prevent rebooster effects, prolongation of elimination time, and to reduc

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00036.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryIn France, patients with haemophilia were infected by HIV up until October 1985, with a maximum of seroconversion between 1983 and 1985. There was a progressive development of AIDS in the 1158 infected patients as reported by Health Authorities. By the end of 1992, 32% of the haemophilia population had developed AIDS and 38 had developed clinical or biological symptoms of immunodeficiency. However, 27% had no clinical symptoms and no severe disorder of the immune system. The present study was established to determine factors common to patients with prolonged survival.

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00037.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryIt has been suggested that the range of AIDS‐defining conditions witnessed in patients with HIV infection has changed since the early years of the HIV epidemic. In this paper we consider the range of AIDS‐defining conditions in a cohort of 111 HIV‐positive men with haemophilia registered at the Royal Free Hospital Haemophilia Centre. In particular we assess whether the incidence ofPneumocystis cariniipneumonia (PCP) has changed over time. The men were all infected between 1979 and 1985 after treatment with infected blood products and have now been followed prospectively for up to 13 years from HIV seroconversion. By the end of 1992, 44/111 patients had developed AIDS. Of the 44 men, 18 (41%) presented with PCP as their first AIDS‐defining condition (ADC), mainly before the initiation of primary prophylaxis in 1989. The remaining 26 patients presented with a range of conditions as their first ADC, but there were no more than four cases in any one disease category. It is estimated that patients suffer from 0.7 further ADCs per year after being diagnosed with AIDS. After taking account of the increased levels of immuno‐suppression in the cohort with time, it appears that the incidence of PCP, both as the first ADC or as any ADC, has declined since the introduction of primary prophylaxis for the disease in 1989. However, non‐compliance with prophylaxis for PCP appears to have played a major role in the continuing occurrence of PCP since 1988. Improvements in compliance with therapy should result in a further reduction in the incidence of PCP both as a first ADC and

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00038.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryTo evaluate the effect and side‐effects of alpha interferon 2B (IFN) treatment in haemophilia patients with chronic hepatitis C (positive HCV antibody test, positive HCV‐RNA test and ALT levels>2.5 × upper limit of normal) eight HIV and HBs‐ag negative haemophilia patients were treated with IFN for 26 weeks in a dosage of 5 mega units (MU) daily for 2 weeks, 2.5 MU daily for 4 weeks and 1.5 MU 3 times a week for 20 weeks. Patients who were transient or non‐responders after 26 weeks of IFN therapy were treated for 2.5 years with 5 MU IFN three times a week. At the end of 3 years follow‐up, four patients were considered complete responders (HCV‐RNA negative) with complete and sustained ALT normalization and disappearance from HCV‐RNA from blood, two patients only showed a transient response and two patients were non‐responders.All patients experienced the common side‐effects of IFN treatment. Two patients complained about feelings of depression and impotence. For both this was the reason to stop IFN treatment. In one patient, IFN treatment was stopped 90 weeks after start of therapy because of persistent fatigue. In another patient the dosage was adjusted because of a decrease in platelet count.No effect of IFN on bleeding frequency and chronic arthopathy was seen.We conclude that the clinical effects and side‐effects of IFN therapy in patients with haemophilia and chronic hepatitis C are comparable with those of non‐haemophilia patients with chronic hepatitis C. Haemophilia patients can be treated for chronic hepatitis C infection in the same way as patien

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00039.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

SummaryWe have conducted a controlled trial in 20 haemophilic patients in which intravenous recombinant interferon alpha‐2a, 3 mega units thrice weekly, was used to treat chronic hepatitis C infection. The study endpoints included complete and paritial normalization of serum ALT, and loss of serum HCV RNA as determined by polymerase chain reaction (PCR). Interferon treatment was effective, and resulted in improvement in ALT in nine (45%) and a loss of HCV RNA in five patients (26%), but a sustained normalization of ALT has been seen in only one case. Responses were poor in those with HIV coinfection or with HCV genotype 1 (Simmonds classification). Troublesome side‐effects were reported in 80%. The occurrence of a factor VIIIc inhibitor during the study was possibly an autoimmune complication of interferon. In conclusion, we have shown lower response rates than those seen in post‐transfusion hepatitis C infection and suspect that current interferon regimes are unlikely to influence the natural history of HCV infection in haemophilia. Consideration should be given to trials of higher dosage interferon, and of long‐term maintenance therapy for those who

ISSN:1351-8216    

DOI:10.1111/j.1365-2516.1995.tb00040.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY