I am having confusing about treatment of phorpyria . I read it somewhere that for treating phopyria hemin and glucose is given IV . Please give me an easy explanation of it


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    $\begingroup$ Some more background and a link to the source of the "somewhere" is recommended. Until edited, I vote for closure $\endgroup$ – AliceD May 2 '15 at 16:30
  • $\begingroup$ From book Lippincot review $\endgroup$ – user60180 May 2 '15 at 17:11
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    $\begingroup$ Hi and welcome! As @AliceD said, this question needs a lot more detail in order for us to help you. This page in our Help Center will show you what she means. We look forward to seeing more of you! $\endgroup$ – Sue Saddest Farewell TGO GL May 2 '15 at 17:18
  • $\begingroup$ @Sue acute phorpyria is a disease in which there is accumulation of intermediate compounds in response to defective heme synthesis pathway .what else I can tell u . $\endgroup$ – user60180 May 2 '15 at 17:22
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    $\begingroup$ @user60180, details like what exactly you are after, what effort you made in finding the answer (if any). You can refer this for a guide: biology.stackexchange.com/help/how-to-ask $\endgroup$ – Rover Eye May 2 '15 at 17:25

Since porphyria is not one disease but many, I suppose that with "acute porphyria" you mean acute intermittent porphyria. The reason of the disease is an autosomal dominant mutation on the enzyme porphobilinogen deaminase, an enzyme that converts porphobilinogen to hydroxymethylbilane by a deamination reaction.

Now the heme is produced in many steps, and the enzyme above is important in the early steps of heme synthesis. In persons with porphyria, these heme precursors accumulate, causing toxicity. In acute porphyria the symptoms are mostly from the central nervous system. The figure under shows the steps of the biosynthesis of heme, and as you see at the upper right corner, defect in the enzyme porphobilinogen deaminase (PBG-deaminase) results in accumulation of porphobilinogen. enter image description here

Glucose and other carbohydrates can help suppress disease activity, are given by vein or by mouth, and are part of initial treatment. Intravenous heme, however, is both more specific and effective than glucose and should be started if the patient’s symptoms fail to improve within 36 hours.http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/AIP [2015-05-02]

More about carbohydrates and the suppression of symptoms in porphyria http://www.porphyriafoundation.com/about-porphyria/diet-and-nutrition/the-glucose-effect-in-acute-porphyria [2015-05-02].

  • $\begingroup$ I like the explanation . But how glucose do this job . Does it help in removing those excessive intermediates? Or something else $\endgroup$ – user60180 May 2 '15 at 17:33
  • $\begingroup$ If you see at the figure above you can see at the second step that glucose and hemin (which you mentioned in your question) inhibits the Aminolevulinic acid synthase. Glucose therefore inhibits the formation of the heme precursor 5-aminolevulinic acid and thus downstream formation of porphobilinogen. flipper.diff.org/app/pathways/info/6362 [2015-05-02]. $\endgroup$ – Sarin May 2 '15 at 17:38

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