Showing papers on "Haemophilia published in 1983"

Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976-80: report on behalf of the directors of haemophilia centres in the United Kingdom.

Abstract: A five year survey of the treatment of patients in the United Kingdom suffering from haemophilia and related disorders was carried out on behalf of the directors of haemophilia centres. The survey showed an increase in the number of patients receiving treatment from the centres, a substantial increase in the total amount of therapeutic materials used, and an increase in the average amount of factor VIII or factor IX used yearly per patient. Home treatment became established for severely affected patients and accounted for roughly half of the total amount of material used. Study of the acquisition of factor VIII or factor IX antibodies (inhibitors) in patients with haemophilia A or haemophilia B showed no increase in antibodies during the survey period, despite the increased use of factor VIII and factor IX concentrates. The occurrence of acute hepatitis in treated patients was also studied and no increased incidence was observed. A near normal median expectation of life in patients with severe haemophilia A was found.

Immunological abnormalities in haemophilia: are they caused by American factor VIII concentrate?

Abstract: Scottish patients with haemophilia, most of whom had received no American factor VIII concentrate for over two years, were found to have immunological abnormalities similar to those in their American counterparts--that is, a reduced proportion of T helper cells, an increased proportion of T suppressor cells, and a reduced response to concanavilin A. Factor VIII from both the United States and Scotland severely inhibited the in vitro lymphocyte response to mitogens in patients and controls. The American and Scottish concentrates could not be distinguished in terms of either patient usage or their effect in vitro. These results argue against a disease vector specific to American blood products.

Coronary artery disease in severe haemophilia.

Abstract: It has been suggested that the coagulation defect in haemophilia A protects these patients from coronary artery disease. We report two patients with severe haemophilia, who each had severe atheroma localised to the coronary arteries. Both patients had received only small amounts of factor VIII concentrates in their lifetime because of various social reasons. In one case death resulted from myocardial infarction secondary to an occlusive thrombus in a coronary artery. The implications of these findings to the pathogenesis of vascular disease are discussed.

The haemophilic patient's self-perception of changes in health and life-style arising from self-treatment.

Abstract: Twenty three adults and twenty children with haemophilia, all on self-treatment, participated in the present study. They were interviewed as to how they perceive the effects of self-treatment upon various aspects of their life. It was found that the ability to treat themselves was perceived by patients as a great improvement over their previous treatment regimes for which they had had to go to hospital. Twelve of the adult patients had participated in our previous studies concerned with the social an psychological aspects of haemophilia. The analysis of the responses of these twelve patients showed that self-treatment is perceived so positively that some of the patients' earlier perceptions were distorted to correspond with their present and more optimistic perceptions of their condition. However, the number and type of psychosomatic symptoms was found to be unchanged by self-treatment and the unemployment rate has not decreased.

Acquired immunodeficiency syndrome, hepatitis, and haemophilia.

Abstract: People with haemophilia, their families, and their doctors feel threatened by the deluge of speculation about the possible side effects of treatment with blood products. Two topics hold their attention: the risk of contracting the acquired immunodeficiency syndrome (AIDS) and the risk of developing hepatitis and subsequent chronic liver disease. Centers for Disease Control in Atlanta define a case of AIDS as \"a reliably diagnosed disease that is at least moderately indicative of an underlying cellular immunodeficiency in a person who has had no known underlying cause of cellular immunodeficiency and no other cause of reduced resistance reported to be associated with that disease.\"' For epidemiological purposes this definition is wide; it does not suggest a single aetiological agent, merely a similar response, or lack of it, to injury. It is important to recognise this in view of the difficulty in classifying the different disorders concerned and the current reaction of the media to announcements of \"new\" cases. Until diagnostic markers for the disorder are found terms like \"confirmed AIDS\" are unhelpful in defining what may well be a multifactorial disorder. To date, patients fulfilling the Centers for Disease Control definition have included male homosexuals (71%) intravenous drug abusers of both sexes (17%O) and people from Haiti (5%). Heterosexual partners of people with AIDS or in a high risk group for the disorder (1%), haemophiliacs (1%), and recipients of blood products (other than haemophiliacs) with immunodeficiency (1%) have also been reported. Some 4% of the 2259 cases notified in the United States and Puerto Rico by September 1983 fitted no particular pattern.' Among the estimated 20 000 people with haemophilia A (factor VIII deficiency) in the United States, 17 cases of AIDS have been reported. Ten patients have died, all with Pneumocystis carinii pneumonia.2 Two of the 17 cases had other risk factors for AIDS. No cases of Kaposi's sarcoma and haemophilia are known. Britain has 4592 people with haemophilia A known to the haemophilia centre directors, and two cases of haemophilia and AIDS have been reported to the Communicable Disease Surveillance Centre at Colindale. One patient with P carinii pneumonia has died (G L Scott, personal communication), and the other has had opportunistic infections (A L Bloom, personal communication). Thus the reported incidence of AIDS in the two countries is about 0-8 per 1000 haemophiliacs. If all disorders of cellular immunity, thrombocytopenia, and lymphopenia are classed

An enzyme-linked immunosorbent assay for an antigen related to non-a, non-b hepatitis and its antibody: Partial characterization of the antigen and chimpanzee transmission

Abstract: An enzyme-linked immunosorbent assay (ELISA) was developed based on sera from patients convalescent from non-A, non-B hepatitis and haemophilia A patients who had been frequently treated with commercial blood products Using this ELISA, an antigen was detected which appears to be related to non-A, non-B hepatitis The antigen is provisionally designated as DS-antigen (DS-Ag) The serum of another patient with haemophilia A, which was strongly positive for the DS-Ag, caused a typical case of non-A, non-B hepatitis in a chimpanzee DS-Ag could be detected in the serum of the chimpanzee during the acute phase of the infection The ELISA for DS-Ag reacted with neither hepatitis A or B virus antigens, nor Epstein-Barr virus or cytomegalovirus The assay was provisionally evaluated using sera from different groups of patients Out of 17 patients with posttransfusion hepatitis non-A, non-B, 11 were found positive in the ELISA for DS-Ag (65%) As expected, a relatively high prevalence of DS-Ag (9%) was found in patients with haemophilia, who are regularly treated with blood-clotting factor-concentrates Antibodies to DS-Ag were found in 48% of these patients The DS-Ag was found in 8 of 1400 (06%) volunteer blood donors, and antibody to DS-Ag in 3% of the sera Remarkably, a high incidence (41%) of antibodies to DS-Ag was found in prostitutes, suggesting that this antigen may be transmitted by a sexual route The DS-Ag was pelleted by ultracentrifugation for four hours at 100,000g and was found to have a buoyant density of 132 g/cm3 in a CsCl gradient

Prodromal acquired immune deficiency syndrome in Australian homosexual men.

Abstract: The acquired immune deficiency syndrome (AIDS) was first reported in 1981 in homosexual men. Since then, it has shown an alarming increase in frequency. The syndrome has now been described in other population groups as well, in particular in persons with haemophilia who are exposed to frequent parenteral injections of blood or blood products, and in sexual contacts of those in at-risk groups. This report deals with two cases of this syndrome which occurred in homosexual men residing in Sydney. These cases are among the first reported in Australia, but, undoubtedly, many more will be forthcoming. Both patients have a profound defect in cellular immunity. The cause of this condition remains a mystery, although viral infection has been suggested. Therapeutic approaches have not yet been established. Clinicians and laboratory workers should be aware that this condition is now present in Australia, and should take appropriate precautions to avoid infection and to limit its spread.

Chronic arthropathy in von Willebrand's disease.

Abstract: Summary In von Willebrand's disease, chronic arthropathy is thought to be rare. We present four young patients, three girls and a boy, with severe von Willebrand's disease, who developed chronic progressive arthropathy due to joint haemorrhage, similar to that seen in haemophilia. Chronic joint disease is not as uncommon as is generally assumed.

Haemophilia A in the cocker spaniel: a case report

Abstract: A four-year-old entire male cocker spaniel was presented with a history of dyspnoea due to tracheal stenosis. As part of the clinical approach, a coagulation screening profile revealed moderately reduced levels of Factor VIII procoagulant activity (VIII: C), but elevated factor VHI-related antigen levels (VIIIR: Ag). A diagnosis of haemophilia A (classic haemophilia) was made and the tracheal stenosis attributed to a mediastinal haematoma. The dyspnoea was alleviated by the surgical insertion of a tracheostomy tube and the dog was treated with citrated whole blood and fresh-frozen canine plasma. Response to treatment was excellent and follow-up radiography three months later revealed resolution of the tracheal stenosis.

The effects of age, gene source and familial severity on Factor VIII in normals and haemophilia carriers: analysis by multiple regression.

Abstract: There are conflicting views on the effects of age, gene source and familial severity on levels of factor VIII in carriers of haemophilia. Different workers have found that factor VIII increases with age, is higher in paternal carriers, and is higher in carriers from families with more severe haemophilia. Other workers have disagreed with these findings. In this study we explored some of the causes of this conflict. We measured factor VIII related antigen and factor VIII coagulant activity on 40 normal females and 48 carriers, and analysed the results by multiple regression and analysis of covariance. Our results indicated that both factor VIII coagulant activity and factor VIII related antigen increased with age, but were unaffected by the familial severity of haemophilia or whether the defective gene came from the mother or the father. We found that the conflicting reports of previous authors were due to high inter-correlations of the studied variables.

General Medical and Metabolic Diseases

Abstract: Both haemophilia A (factor VIII) and haemophilia B (factor IX) are inherited as X-linked recessive diseases and in both the functional defect is in the intrinsic pathway, the extrinsic tissue-factor-dependent pathway being the only mechanism left intact to subserve haemostasis. The most important consequence of haemophilia is arthritis and it is interesting that synovial tissue is deficient in tissue factor, which may explain this association. Arthritis occurs only in those patients who are severely affected, with concentrations of circulating antihaemophiliac globulin of less than 5%, and onset is usually in childhood. The knee and the elbow joints are most commonly involved and may become swollen acutely either spontaneously or after only the most minor trauma. Range of movement, usually preserved after the first episode, is progressively lost with subsequent attacks and severe joint destruction and deformity may follow. Extra-articular bleeding is also common.