Investigation of the metabolism of porphyrins


 * porphyria is classically divided into "hepatic", "erythropoietic" and "erythrohepatic"
 * according to the tissue that produces porphyrins.
 * liver diseases are acute and chronic



Porphyria cutanea tarda

 * 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

 * 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"

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 * Porphyria