Investigation of the metabolism of porphyrins

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  • porphyria is classically divided into "hepatic", "erythropoietic" and "erythrohepatic"
  • according to the tissue that produces porphyrins.
  • liver diseases are acute and chronic
Searchtool right.svg For more information see Porphyria.
File:Porphyria.png
Heme biosynthesis with marked enzyme deficiencies

Porphyria cutanea tarda[edit | edit source]

  • chronic hepatic porphyria, the most common
  • uroporphyrinogen decarboxylase disorder
  • patients usually come with skin problems (solar localization)
  • without treatment, however, they are most at risk of cirrhosis
  • acute attacks do not occur
  • the precise distinction of cutaneous porphyrias can only be made in the laboratory
  • in untreated patients, there is an increased level of porphyrins in the urine, ALA and PBG are normal
  • uroporphyrin predominates in urine
  • chromatography is done - typical peaks
  • fluorescence is also used for determination - Soret band

Acute hepatic porphyria and lead poisoning[edit | edit source]

  • most often ``acute intermittent porphyria (AIM), then p. variegata, hereditary coproporphyria
  • AD, common - in attacks - form abdominal, neurological, psychiatric
  • they are often operated on for an acute abdominal event
  • it is enough to examine the values ​​of ALA, PBG'' and total porphyrins' in urine
  • Pb blocks ALAdehydratase - ALA accumulates, PBG is normal
  • main examination – "waste of total porphyrins in 24 h", "ALA" and "PBG"

Links[edit | edit source]

Related Articles[edit | edit source]

References[edit | edit source]

  • {{#switch: book

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  Incomplete publication citation. SCHNEIDERKA, Peter, et al. Chapters in Clinical Biochemistry. Prague : Karolinum, 2004. 978-80-7262-438-6.

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  Incomplete article citation.  SCHNEIDERKA, Peter, et al. 2004, year 2004, 

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  Incomplete site citation. SCHNEIDERKA, Peter, et al. Karolinum, ©2004. 

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  Incomplete carrier citation. SCHNEIDERKA, Peter, et al. Karolinum, ©2004. 

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  Incomplete database citation. Karolinum, ©2004. 

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  SCHNEIDERKA, Peter, et al. Chapters in Clinical Biochemistry. Prague : Karolinum, 2004. 978-80-7262-438-6} }
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