The ear: on balance, it's very important.
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Biomedical subjects
Publications and source records attributed to J Voke.
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Several studies have recently documented the presence of persistently abnormal liver function tests in asymptomatic haemophiliacs. While the aetiology is unknown it is possible that repeated exposure to agents transmitted in blood products may be important. This study has attempted to determine the prevalence of viral exposure and its relationship to liver function in this multitransfused group of individuals. The prevalence of viral antibodies with the exception of antibody to hepatitis B surface antigen (anti-HBs) and cytomegalovirus (CMV) was normal when compared to that in the general population. Hepatitis B surface antigen (HBsAg) was not detected, but anti-HBs was found in 83% of patients; 50% of patients had abnormal liver function. However, liver function tests were normal in all patients with mild haemophilia and were only rarely abnormal in patients who had no detectable antibody to CMV, Epstein-Barr virus (EBV), and HBsAg. This study demonstrates that multiple transfusions of blood products, that is, cryoprecipitate and factor concentrates, do not increase the risk of exposure to the viruses studied with the exception of hepatitis B virus.
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Ultrasonography was used to demonstrate retroperitioneal haemorrhages and intramuscular haematoma in three patients with antibodies to factor VIII and one with von Willebrand's disease in whom major bleeding episodes occurred. Scans were useful in demonstrating the presence, location, size and regression of haemorrhages.
A patient who had developed a factor VIII antibody three weeks after the birth of her first baby became pregnant for the second time 10 weeks later. The pregnancy was normal apart from extensive spontaneous bruising during the first two months. This bleeding tendency disappeared in the third month of gestation and the antibody became undetectable during the eighth month. It has not reappeared seven months later. An unexplained long bleeding time and low platelet count were noted during the last trimester. The literature on postpartum coagulation inhibitors is reviewed.
Abnormal 125I-Clq-binding activity was found in the sera of 94% of 55 haemophiliacs. Sera from 66% of these patients inhibited macrophage uptake of labelled aggregated human IgG in a competitive radiobioassay. These results suggest that large molecular weight immune complexes are present in these sera. Analysis of the precipitates obtained directly from the sera by addition of 4% polyethylene glycol showed either a mixture of IgG and IgM or IgM alone. There was poor correlation between the radiobioassay results and the Clq-binding activities in the whole group of 55 patients, suggesting a heterogeneous population of complexes. Nevertheless, a significant correlation was found between Clq-binding activities and the radiobioassay results in seven patients with antibodies to factor VIII, suggesting a homogeneous population of complexes. There was poor correlation between the level of immune complexes and the amount of replacement therapy the patient had received in the previous six months. Abnormal liver function tests were found in 55% of the patients studied but there was poor correlation between these abnormal levels and the Clq binding activities and radiobioassay results. Only two patients had clinical evidence of liver disease.
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