porphyria - Diagnosis to Treatment

1. Porphyria
Dr. Subhash Thakur
Sr. Lecturer, CMC, Bharatpur

2. Contents
• Introduction
• Etiology
• Pathogenesis
• Morphology
• Clinical Features
• Investigations
• Treatment

3. Introduction
• Derived from greek work “Porphuros” meaning red/purple
• Patient pass urine,which on standing acquires port wine color

4. Aetiology
• Deficiencies in enzyme involved in heme biosynthesis, which leads to
blockade of porphyrin pathway and subsequent accumulation of
porphyrin and their precursors