porphyria

porphyria

English

Etymology

From porphyrin, from German Porphyrin.

Pronunciation

• IPA^(key): /pɔː(ɹ)ˈfɪɹɪə/

Noun

porphyria (plural porphyrias)

1. (pathology) Any of several usually hereditary abnormalities of porphyrin metabolism characterized by excretion of excess porphyrins in the urine.
   • 2000, Herbert L. Bonkovsky, Richard W. Lambrecht, 44: Hemochromatosis, iron overload, and porphyria cutanea tarda, James C. Barton, Corwin Q. Edwards (editors), Hemochromatosis: Genetics, Pathophysiology, Diagnosis and Treatment, page 453,

     The term porphyria, derived from the Greek word porphyra, meaning purple, is apt because a hallmark of most of the porphyrias is the overexcretion of porphyrins in urine or feces.

   • 2003, M. Lecha, C. Herrero, D. Ozalla, Porphyrias, Andreas D. Katsambas, Torello M. Lotti (editors), European Handbook of Dermatological Treatments, page 419,

     However, the appearance of homozygous forms without consanguinity and coincidence of two types of porphyria in the same patient, dual porphyrias, is possible.

   • 2010, James C. Barton, Corwin Q. Edwards, Pradyumna D. Phatak, Robert S. Britton, Bruce R. Bacon, Handbook of Iron Overload Disorders, page 160,

     The usual presenting symptoms and signs of porphyrias are either skin photosensitivity or neurovisceral symptoms and signs.

Derived terms

• hematoporphyria
• porphyric

Related terms

• protoporphyria

Translations

abnormality of porphyrin metabolism

Catalan: porfíria f Czech: porfyrie f German: Porphyrie f

Italian: porfiria f Spanish: porfiria f Swedish: porfyri c