23 Porphyrias
![](https://photos.thisispiggy.com/file/wikiFiles/IUBM17b.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/o0sKWrq.jpg)
- anything increases activity of P450 can exacerbate condition
![](https://photos.thisispiggy.com/file/wikiFiles/iKEWyuI.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/xELAMnO.jpg)
- anemia can result with severe inhibition of enzymes
![](https://photos.thisispiggy.com/file/wikiFiles/1D0v9I9.jpg)
Porphyria
![](https://photos.thisispiggy.com/file/wikiFiles/kBUTU4e.jpg)
- two common ones are hepatic porphyria: heme synthesis affected in liver
PCT
![](https://photos.thisispiggy.com/file/wikiFiles/IYci69H.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/Tlax3Et.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/tlZOKzR.jpg)
- some carboxyl groups have 3 carbons, others 2
![](https://photos.thisispiggy.com/file/wikiFiles/gdTnjuY.jpg)
- A for acetic, P for propionic
![](https://photos.thisispiggy.com/file/wikiFiles/YXY6Gde.jpg)
- Uroporphyrinogen III must be oxidized, lose H2, to be converted to uroporphyrin III
![](https://photos.thisispiggy.com/file/wikiFiles/jHd7UVH.jpg)
- skin major symptoms (cutanea)
![](https://photos.thisispiggy.com/file/wikiFiles/f9tGJK9.jpg)
- heptatitis from uroporphyrins accumulation in liver
- in kidney: tea colored urine
![](https://photos.thisispiggy.com/file/wikiFiles/fHyp6E0.jpg)
- iron overload like hemochromatosis
![](https://photos.thisispiggy.com/file/wikiFiles/tbOiIU2.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/1mbuvO0.jpg)
- determine high porphyrin, then fractionate to determine high uroporphyrin
![](https://photos.thisispiggy.com/file/wikiFiles/Sp5KatE.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/JkqN33w.jpg)
- vampire hypothesis
- consume heme/blood, inhibit heme synthesis
Acute Intermittent
![](https://photos.thisispiggy.com/file/wikiFiles/hklcFo1.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/dzZDTf8.jpg)
- late in pathway: skin symptoms
- early in pathway: neurologic symptoms
![](https://photos.thisispiggy.com/file/wikiFiles/pXxXW8Y.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/HAwYynF.jpg)
- low glucose, increased ALA synthase, rising heme
![](https://photos.thisispiggy.com/file/wikiFiles/R2ihmZg.jpg)
- confusion, delirium, dementia
![](https://photos.thisispiggy.com/file/wikiFiles/DsIZ3ph.jpg)
- inhibited porphobilinogen deaminase and increased D ala synthase leads to increased D ala and porphobilinogen
![](https://photos.thisispiggy.com/file/wikiFiles/TqeMZ9Y.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/ecYCNHR.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/Os1hLoe.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/Njy0A3T.jpg)
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