Hereditary porphyria manifesting itself during renal failure in a 49-year-old woman

Clinical findings in a female patient aged 49 years suggested Conn's syndrome (hypokalemia, alkalosis, hypertension) developing in the course of renal failure. During treatment biochemically confirmed porphyria manifested itself, and the patient died several days later. On autopsy and in histological examinations malignant nephrosclerosis of vascular origin, nodular hyperplasia of the adrenal cortex and lesions of other organs were found. The largest content of porphyrins was shown in the adrenals, and then in the liver, pituitary gland, thyroid and pancreas. A study of other family members confirmed the genetic background of porphyria. Probably, this hereditary latent hepatic porphyria manifested itself after a breakdown of the feedback mechanisms between the kidneys and the endocrine glands, the adrenals in particular.