Porphyria

  • Photosensitive rash is common in Erythopoietic photoporphyria
  • In Porphyria spastic ileus is seen
  • Patients with axonal neuropathy, unexplained abdominal pain along with history of psychiatric illness may be suffering from Porphyria
  • Acute intermittent porphyria, Porphyria cutanea tarda & Hereditary coproporphyria are autosomal dominant
  • The enzyme deficient in erythropoietic porphyria is Ferrochelatase
  • Hepatomegaly is an essential feature of Hepatic porphyria
  • Steroids, Griseofulvin & Estrogen can precipitate porphyria
  • A girl on sulphonamides developed abdominal pain and presented to emergency with seizure may be suffering from Acute intermittent porphyria
  • Porphyria cutanea tarda can be treated by Phlebotomy & Low dose chlotoquine
  • Treatment of choice in the cutaneous complication of porphyria is Beta carotene
  • A pinkish red Flurorescence of urine with wood's Lamp is seen in Porphyria cutanea tarda
  • Propofol can be used in porphyria
  • Drugs contraindicated in acute intermittent porphyria are Thiopentone & Etomidate
  • A child has Hb-6.5 gm%, MCV-65, MCH-15 and protoporphyria with red cell distribution width much less is most likely to be suffering from Iron deficiency anaemia
  • In intermittent porphyria, Porphobilinogens is present in the urine
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