Wednesday, 7 January 2009

Porphyria

Porphyria

Most Common Type: Porphyria Cutanea Tarda
Basic enzyme deficiency: uroporphyrinogen decarboxylase
Maximum Phototoxicity.


Psychiatric manifestation: Acute Intermittent Porphyria
Phototoxicity NOT seen: Acute Intermittent Porphyria




Erythropoietic protoporphyria: Ferrochelatase deficiency
Congenital Erythropoietic porphyria: (Only porphyria with A.R)Uroporphyrinogen III synthase

Pseudoporphyria: A bullous photosensitivity that clinically and histologically mimics PCT.chronic renal failure treated with hemodialysis,exposure to ultraviolet A (UV-A), Drugs.


Variegate porphyria
: protoporphyrinogen oxidase.
cutaneous photosensitivity, systemic symptoms arising from neurologic dysfunction, or both.


Acute Intermittent Porphyria, Varigate Porphyria and Congenital erythropoietic porphyria produce increase in urine prophobilinogen.

No comments:

Post a Comment