title: Microsomal enzyme induction and clinical aggravation of porphyria: the evaluation of human urinary 6beta-hydroxycortisol/cortisol ratio as the index of hepatic CYP3A4 activity.
abstract: The clinical aspect of porphyria has been investigated, and it is well known that porphyrinogens such as estrogens and alcohol or other inducers of P450 isoenzymes exacerbate the porphyric state. However, there can be a delay in diagnosing porphyria and a difficulty in selecting safe medicine for it even today. A 21-year-old woman developed epilepsy, disturbance of mental state, and spastic tetraparesis during the convalescent period after acute viral encephalitis. She was diagnosed with porphyria after the fifth hospitalization. In the course of modifying her anticonvulsant regimen, the authors examined the 6beta-hydroxycortisol/cortisol ratio (6beta-OHF/F) in her urine, which can be the index of hepatic CYP3A4 activity, with electrospray ionization/mass spectrometry/mass spectrometry (ESI/MS/MS). Generalized and partial complex seizures, other neurological signs and symptoms, and laboratory data were improved after modification of her anticonvulsant regimen. This is the first report of evaluating the urinary 6beta-hydroxycortisol/cortisol ratio in a case of porphyria.
authors.affiliation: Department of Pharmacy, Osaka University Hospital, Suita, Osaka, Japan.
authors.forename: Etsuko
authors.initials: E
authors.lastname: Uejima
authors.forename: Kyoko
authors.initials: K
authors.lastname: Takahashi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Morisaki
authors.forename: Koichi
authors.initials: K
authors.lastname: Takahashi
authors.forename: Masako
authors.initials: M
authors.lastname: Ohno
authors.forename: Yuko
authors.initials: Y
authors.lastname: Nishida
authors.forename: Masayuki
authors.initials: M
authors.lastname: Moriya
authors.forename: Misako
authors.initials: M
authors.lastname: Kaido
authors.forename: Kazuo
authors.initials: K
authors.lastname: Abe
authors.forename: Saburo
authors.initials: S
authors.lastname: Sakoda
authors.forename: Takehiko
authors.initials: T
authors.lastname: Yanagihara
authors.forename: Nobuo
authors.initials: N
authors.lastname: Kurokawa
authors.forename: Junichi
authors.initials: J
authors.lastname: Azuma
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000009:adverse effects*; D051544:Cytochrome P-450 CYP3A; D003577:Cytochrome P-450 Enzyme System / Q000096:biosynthesis* / Q000235:genetics; D020803:Encephalitis, Herpes Simplex / Q000150:complications; D004790:Enzyme Induction; D004827:Epilepsy / Q000188:drug therapy / Q000209:etiology; D005243:Feces / Q000737:chemistry; D005260:Female; D005838:Genotype; D006801:Humans; D006854:Hydrocortisone / Q000031:analogs & derivatives* / Q000652:urine*; D013058:Mass Spectrometry; D008861:Microsomes / Q000201:enzymology*; D011164:Porphyrias / Q000139:chemically induced* / Q000175:diagnosis / Q000201:enzymology; D011166:Porphyrins / Q000032:analysis / Q000652:urine; D011782:Quadriplegia / Q000209:etiology
chemical_list: D000927:Anticonvulsants; D011166:Porphyrins; C001380:6 beta-hydroxycortisol; D003577:Cytochrome P-450 Enzyme System; C104464:CYP3A protein, human; D051544:Cytochrome P-450 CYP3A; C510163:CYP3A4 protein, human; D006854:Hydrocortisone