Acute intermittent porphyria in XXXX : clinical expression, use and experience of exogenous sex hormones. A population-based study in northern Sweden.

@entity1 @entity2208 @entity6052
OBJECTIVE: To describe the clinical expression of @entity6052 ( @entity6052 ) in @entity1 , their use of exogenous sex hormones, and the effects on @entity6052 . DESIGN: A retrospective population-based study. SUBJECTS: All @entity1 aged > or =18 years (n = 190) with DNA-diagnosed @entity6052 in northern Sweden. RESULTS: A total of 166 @entity1 (87%) participated; 91 (55%) had @entity6052 . Severe attacks were reported by 82%; 39% reported recurrent @entity6052 attacks and 22% reported chronic @entity6052 . Oral hormonal contraceptives had been used by 58% of all these @entity1 and by 50 with @entity6052 (57%). Twelve @entity1 (24%) associated oral contraceptives as precipitating @entity6052 attacks; in nine cases their first attack. One @entity1 experienced relief from @entity6052 . On commencing their treatment, 72% of the @entity1 with @entity6052 had not yet suffered their first attack. Twenty-two @entity1 (25%) aged > or =45 years had used hormonal replacement therapy (HRT) at menopause to remedy climacteric symptoms (the percutaneous route was most frequently used); no @entity6052 attack was precipitated. HRT to remedy vaginal dryness was used by 26 @entity1 (28%) aged > or =45 years without triggering an @entity6052 attack. Miscarriages were more frequent in @entity1 with @entity6052 (50%) than in the latent group (30%, P = 0.014). CONCLUSIONS: About half of the @entity1 with @entity6052 had used oral hormonal contraceptives. As 25% of @entity1 with @entity6052 reported attacks associated with such drugs, caution must still be recommended. Menopausal HRT only rarely affected the disorder. @entity2208 was more common amongst @entity1 with @entity6052 .
