Coexistent variegate porphyria and porphyria cutanea tarda.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to L Eales.
Explore the source record for details and available documents.
Certain drugs are well known to cause acute neurological crises in patients with hereditary coproporphyria, variegate porphyria and acute intermittent porphyria. Guidelines for drug therapy have been provided in this article, particular attention being paid to the manner in which the porphyrogenicity or safety of a drug has been assessed and providing full references to the original reports to allow further research if necessary.
Explore the source record for details and available documents.
The acute neurological crisis is the most significant complication of variegate and acute intermittent porphyria and hereditary coproporphyria. If it is managed correctly, the mortality rate should be negligible. An outline is given of the major symptoms and signs encountered in the acute attack, and the therapy which should be used for their relief is discussed. Mention is made of forms of treatment which may decrease the activity of the haem biosynthetic pathway and thus specifically influence the clinical problems.
A patient with hemodialysis-related porphyria cutanea tarda was treated with plasma exchange. A rapid clinical response occurred coincidentally with a significant fall in the plasma porphyrin level. The level fell further over the following few months without additional therapeutic intervention, whereafter a slow rise occurred without recurrence of skin disease. We suggest that this form of treatment may be ideal for the patient with porphyria cutanea tarda and chronic renal failure in whom no alternative therapy is available for the cutaneous problem.
The porphyrin status of 38 renal transplant patients was investigated using a sensitive thin-layer chromatographic assay. Abnormalities of porphyrin synthesis observed in patients on hemodialysis were generally reversed on receipt of a transplant with sustained good renal function. Urinary coproporphyrin levels and the creatinine clearance appeared directly related in both individual cases and generally, substantiating the theory that coproporphyrin is of renal origin. The pre- and postoperative porphyrin status of a unique patient with variegate porphyria who received a successful renal transplant implies that the excess urinary porphyrins and precursors excreted in the acute porphyrias are also of mainly renal, as opposed to hepatic origin. Furthermore, the posttransplant overproduction of porphyrins by a kidney from a normal donor suggests the existence of an endogenous porphyrogenic agent in the circulation of patients with acute porphyria.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.