Onset of manifestations of hepatic porphyria in relation to the influence of female sex hormones

Abstract

Of 54 female patients with hepatic porphyria, 11 or 19.6% had onset or exacerbation of symptoms in relation to menstruation or pregnancy. Ten of the 11 were of the acute intermittent, one of cutanea tarda type. Serial observations of urinary porphyrin and precursor excretion over relatively long periods failed to reveal significant changes in relation to clinical manifestations and phase of menstrual cycle or the effect of ovariectomy. Administration of female sex hormones was associated with the 1st appearance or with an exacerbation of hepatic porphyria in 12 patients. Ten of these had cutaneous manifestations, 1 had only neuropsychiatric acute symptoms, and 1 had both. In 5 in which estrogens had been discontinued there was disappearance of the cutaneous manifestations. A majority of thepatients evidencing some form of endocrine relationship with symptoms of porphyria exhibited mild evidence of abnormality of liver function.