Investigation of the metabolism of porphyrins

From WikiLectures

  • 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

|book =

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

|collection =

  Incomplete citation of contribution in proceedings. SCHNEIDERKA, Peter, et al. Chapters in Clinical Biochemistry. Prague : Karolinum, 2004. {{
  #if: 80-246-0678-X |978-80-7262-438-6} }
  |article = 
  Incomplete article citation.  SCHNEIDERKA, Peter, et al. 2004, year 2004, 

|web =

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

|cd =

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

|db =

  Incomplete database citation. Karolinum, ©2004. 

|corporate_literature =

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