PORPHYRIAS Flashcards
URINE COLOR
Red / purple / burgundy-red / red-purplish
PORT WINE
Urine color in LEAD POISONING
COLORLESS
ALA hydratase deficiency porphyria
Aminolevulinic acid (ALA) synthetase
Acute intermittent porphyria
UROPORPHYRINOGEN SYNTHETHASE
Congenital erythropoietic porphyria
UROPORPHYRINOGEN COSYNTHASE
Porphyria cutanea tarda
UROPORPHYRINOGEN DECARBOXYLASE
Hereditary coproporphyria
COPROPORPHYRINOGEN OXIDASE
Variegate porphyria
Protoporphyrinogen oxidase
SCREENING TESTS
Ehrlich’s RXN
Fluorescence @ 550-600 nm
FEP
SPECIMEN
Urine, blood, stool, bile
Ehrlich’s rxn
Detects D-ALA and porphobilinogen
Fluorescence @ 550-600 nm
- Test for
- Result
- Uroporphyrin, coproporphyrin, protoporphyrin
2. Violet / pink / red fluorescence
CDC- recommended test for lead poisoning
FREE ERYTHROCYTE PROTOPORPHYRIN