68. Pathologic pigmentation caused by porphyrins Flashcards
1
Q
Porphyria
A
Disorders connected to disturbances in the HEME synthesis pathway
2
Q
Porphyrin as a component of Hemoglobin
A
Protoporphyrin + Fe(iron) + globulin = Hb
3
Q
Where is porphyrin synthesised?
A
Liver & Bone marrow
4
Q
Characteristics of porphyria
A
- Spongy bones are coloured
- Dental cement –> brown
- Dentin–> pink, reddish brown
- Enamel–> White
- Cartilage, ligaments & tendons–»Uncoloured
5
Q
Uro- and coproporphyrins are be excreted with
A
urine and feces
6
Q
Trigger porphyria symptoms
A
Chemicals :Lead, arsenic, anilin
can be heridatary
7
Q
Porphyrins accumulated in
A
tissues –> appear as a brownish-violet colour as well as they exhibit fluorescence under UV light
