Heme Derivatives (11.1-11.19)

Porphyrins

class of pigments that have a ring of 4 heterocyclic groups, sometimes with a metal atom

Porphyrias

comes from Greek purple, diseases caused by enzyme deficiencies in heme production

Hemin

black inorganic compound which forms from heme when RBCs are lysed and exposed to air

Porphyria cutanea tarda

most common porphyria, deficiency of decarboxylase, serum porphyrin is best test, defined by blistering

Acute intermittent porphyria

deficiency in PBG deaminase, urine porphobilinogen is best test, defined by blistering and nerve impairment

Erythropoietic Porphyria

deficiency of ferrochelatase, increase in total erythrocyte protoporphyrin, defined by blistering

Watson schwartz

4-dimethylaminobenzaldehyde produces a red color with porphobilinogen at 555nm

Hoesch

quick bedside porphobilinogen test that is not used and out of date for 50 years

porphyrin chromatography

porphyrin fluorometry

porphyrin spectrophotometry

hepatic jaundice

prehaptic jaundice

posthepatic jaundice