Sunday, 24 May 2015

Porphyrias

#HYI PORPHYRIA
Most Common Type: Porphyria Cutanea Tarda with basic enzyme deficiency: uroporphyrinogen decarboxylase and Maximum Phototoxicity.

Psychiatric manifestation and Phototoxicity NOT seen: Acute Intermittent Porphyria

Erythropoietic protoporphyria: Ferrochelatase deficiency

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

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

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

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

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