17-ketosteroids and Porphyrias

1. Urinary 17-oxosteroid conjugates were measured by gas-liquid chromatography in five patients with hereditary coproporphyria. 2. Three patients were in an acute attack and showed significantly increased excretion of sulphate or glucuronide conjugates of aetiocholanolone. There was increased excretion of several other related steroids but no consistent pattern was apparent. 3. In the two patients in remission, excretion of urinary 17-oxosteroids was not increased. 4. The ratio of total urinary aetiocholanolone to androsterone (5beta:5alpha) was found to be significantly elevated for the three patients in an acute attack. Serial measurements were made in two of these patients and showed a highly significant linear correaltion between this ratio and the urinary content of delta-aminolaevulic acid and porphobilinogen. 5. These observations suggest the involvement of the 17-oxosteroids, espically aetiocholanolone, in the pathogenesis of hereditary coproporphyria.

Topics: 17-Ketosteroids; Adult; Aminolevulinic Acid; Androsterone; Coproporphyrins; Etiocholanolone; Female; Glucuronates; Humans; Male; Middle Aged; Porphobilinogen; Porphyrias; Porphyrins; Sulfates

Topics: 17-Ketosteroids; 5-Aminolevulinate Synthetase; Adult; Androgens; Androsterone; Animals; Chromatography, Gas; Dexamethasone; Estrogens; Etiocholanolone; Female; Humans; Levulinic Acids; Liver; Male; Middle Aged; Porphobilinogen; Porphyrias; Progestins; Rats

Topics: 17-Ketosteroids; 5-Aminolevulinate Synthetase; Acute Disease; Adult; Animals; Chromatography, Gas; Cycloheximide; Dactinomycin; Dexamethasone; Female; Glucuronates; Humans; Liver; Male; Middle Aged; Porphobilinogen; Porphyrias; Porphyrins; Rats; Remission, Spontaneous; Sulfuric Acids

Topics: 17-Ketosteroids; 5-Aminolevulinate Synthetase; Androstanes; Animals; Animals, Newborn; Chick Embryo; Chickens; Dicarboxylic Acids; Enzyme Induction; Esters; Female; Glutethimide; Hydantoins; Hydroxysteroids; Hypnotics and Sedatives; Ketosteroids; Liver; Mice; Piperidones; Porphyrias; Porphyrins; Pregnanes; Pyridines; Pyrrolidinones; Secobarbital; Sex Factors; Species Specificity; Succinimides

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adult; Blood Glucose; Feces; Female; Growth Hormone; Humans; Insulin; Lipids; Liver Diseases; Porphyrias; Porphyrins

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Alcohol Oxidoreductases; Alcoholism; Aldehyde-Lyases; Chronic Disease; Enzymes; Estrogens; Gonadotropins; Hepatitis; Histocytochemistry; Humans; Italy; Liver; Liver Cirrhosis; Liver Diseases; Liver Function Tests; Ornithine Carbamoyltransferase; Porphyrias; Porphyrins; Pregnanediol; Transaminases

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Acute Disease; Adrenocorticotropic Hormone; Adult; Blood Glucose; Diet; Diet Therapy; Dietary Carbohydrates; Glucose Tolerance Test; Growth Hormone; Humans; Insulin; Lactates; Male; Metyrapone; Pituitary-Adrenal Function Tests; Porphyrias; Porphyrins; Pyrroles; Pyruvates