porphyrias ppt biochemistry vkunder637@gmail.com
Post on 24-May-2015
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PORPHYRIAS
Dr. Vijay Marakala, MBBS, MD.
Assistant professor BIOCHEMISTRY SIMS & RC
PORPHYRIA - Definition
Porphyrias are diseases of heme biosynthesis characterized by increased excretion of porphyrins
or porphyrin precursors in urine and feces.
PORPHYRIA - Classification
Genetic
Acquired
PORPHYRIA - Classification
Erythropoietic porphyrias
• Enzyme deficiency occurs in bone marrow
Hepatic porphyrias
• Enzyme deficiency occurs in liver
PORPHYRIAClinical Features
Acute abdominal pain
Acute psychiatric disturbances
Peripheral neuropathy
Photosensitivity and skin lesions
ACUTE INTERMITTENT PORPHYRIA
Most commonLatent prior to pubertyAbdominal pain, neuropsychiatric symptoms
Photosensitivity Pink coloured urine
CONGENITAL ERYTHROPOIETIC PORPHYRIA
PORPHYRIA CUTANEA TARDA
THANK YOU
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