Urinary Porphyrinogens in Normal Subjects and in Patients with Porphyria Cutanea Tarda and Acute Intermittent Porphyria

Hernández, Antonia; Sepulveda, Paloma; Fernandez-Cuartero, B.; Salamanca, R. E. De

doi:10.1055/s-2007-1002147

Cited by 3 publications

(1 citation statement)

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“…In urine samples of porphyric patients, however, the concentrations of porphyrinogens are significantly lower (22), and therefore the pH-dependent formation of the isomers II and IV is markedly reduced.…”

Section: Formation Of the Atypical Coproporphyrins II And Ivmentioning

confidence: 99%

Composition of Urinary Coproporphyrin Isomers I — IV in Human Porphyrias

Jacob¹,

Doss²

1993

Clinical Chemistry and Laboratory Medicine

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Summary:The urinary distribution and relative proportions of the four Coproporphyrin isomers I-IV were investigated in 50 patients suffering from hepatic and erythropoietic types of hereditary porphyrias. A highly efficient sample preparation method was applied to isolate urinary coproporphyrins, the isomer ratios of which were quantitated by isocratic ion-pair high-performance liquid chromatography. Results showed a significant decrease (p < 0.001) of the proportion of Coproporphyrin I in acute hepatic porphyria (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, porphobilinogen synthase deficiency porphyria) as compared with chronic hepatic porphyria (porphyria cutanea tarda, chronic hepatic porphyria type B and C) (13.2 ± 5.3%, χ ± S.D., vs. 31.4 ± 11.5%). Conversely, the proportion of isomer III was significantly higher (p < 0.001) in acute hepatic porphyria than in chronic hepatic porphyria (80.9 + 5.2% vs. 62.2 + 10.9%). As expected, the highest level of Coproporphyrin I (90.0 ± 1.9%) was found in congenital erythropoietic porphyria.The atypical coproporphyrins II and IV were detected in all types of porphyria analysed and ranged from 0.2 to 9.0%; no significant differences were seen between acute and chronic hepatic porphyrias. The diagnostic importance of the isomer ratios of coproporphyrins I and III has been confirmed in our study, while the significance of the atypical Coproporphyrin isomers II and IV is still unclear at present.

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Section: Formation Of the Atypical Coproporphyrins II And Ivmentioning

confidence: 99%

Composition of Urinary Coproporphyrin Isomers I — IV in Human Porphyrias

Jacob¹,

Doss²

1993

Clinical Chemistry and Laboratory Medicine

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Anesthetic Considerations in Porphyrias

Jensen

Fiddler²,

Striepe³

1995

Anesthesia & Analgesia

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Four hereditary types of porphyria are now classified as acute porphyrias. Enzymatic defects result in accumulation of porphyrin precursors (usually ALA and PGB). The quantity of these precursors may be normal or slightly increased in latent periods but increase to toxic levels during a porphyric crisis. Iatrogenic induction of ALA synthetase by administration of certain triggers (classically barbiturates) is only one of several factors which contribute to porphyric crisis. Signs and symptoms of acute porphyric attack consist primarily of neurologic dysfunction, which occurs secondary to neurotoxicity of ALA or diminished intraneuronal heme levels. Appropriate anesthetic management of porphyria requires knowledge of the type of porphyria (acute vs non-acute), assessment of latent versus active (crisis) phase, awareness of clinical features of porphyric attack, and knowledge of safe pharmacologic intervention.

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