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Is oestrogen therapy justified in the treatment of hereditary haemorrhagic telangiectasia: a biochemical evaluation

INTRODUCTION: Systemic and topical oestrogen can provoke squamous metaplasia of epithelium. In Hereditary Haemorrhagic Telangiectasia (HTT) the underlying telangiectasia may be protected from trauma and epistaxis reduced. Oestrogens have been advocated but their efficacy is unclear.1 Recent advances have now identified two oestrogen and one progesterone receptors. The aim of this study is to analyse the sex receptor status of HHT nasal mucosa to determine if oestrogen therapy is biochemically justified. METHOD: Five HHT patients (three men, two women) and eight controls (four men, four women) underwent nasal mucosa biopsy. Samples were fixed in formalin and paraffin embedded. Alpha oestrogen (ERalpha ), beta oestrogen (Erbeta) and progesterone receptors (PgR) receptors wre identified using mouse monoclonal antibodies by the Streptavidin-biotin peroxidase method. RESULTS: Erbeta was detected in two HHT patients (one man, one woman) and two control patients. ERalpha and PgR was absent in HHT patients. CONCLUSION: This pilot study demonstrated that a subgroup of HHT patients is Erbeta positive. Oestrogen theraphy therefore has a potential therapeutic role on a biochemical basis in these patients. Erbeta status should be determined before considering oestrogen therapy.