Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder affecting blood vessels of the skin, mucous membrane and viscera. The otorhinolaryngologist is the commonest clinician involved in management as epistaxis occurs in 93% of the patients. As marked advances have recently been made regarding the pathogenesis and management of the condition, the otorhinolaryngological perspective is reviewed.
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.
Systemic and topical oestrogen can provoke squamous metaplasia of epithelium. In Hereditary Haemorrhagic Telangiectasia (HHT) the underlying telangiectasia may be protected from trauma and epistaxis reduced. Oestrogens have been advocated but their efficacy is unclear. 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. 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) and beta oestrogen (ERss) and progesterone (PgR) receptors were identified using mouse monoclonal antibodies by the Streptavidin-biotin peroxidase method. ERss was detected in two HHT subjects (1 M: 1F) and two control subjects. ERalpha and PgR was absent in HHT subjects. This pilot study demonstrated that a subgroup of HHT patients were ERss positive. Oestrogen therapy therefore has a potential therapeutic role on a biochemical basis in these patients. ERss status should be determined before considering oestrogen therapy.
Using the PAP method IgG-antibodies were found in a Mooren's ulcer on the surface of corneal epithelia, plasma cells and stromal lamellae in the ulceral pit. The histological picture is indicative of an autoimmune disease.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.