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Excretion patterns for porphyrins, porphyrin precursors, and porphyrin metabolites; and deficient erythrocyte enzymes in the porphyrias

Excretion patterns for porphyrins, porphyrin precursors, and porphyrin metabolites; and deficient erythrocyte enzymes in the porphyrias
Type of porphyria Specific porphyria Urine Stool Erythrocytes Plasma
Acute neurovisceral ADP ALA, coproporphyrin III *

Zinc protoporphyrin

Markedly decreased ALAD activity

ALA*
Acute neurovisceral AIP ALA, PBG, uroporphyrin, coproporphyrin * Decreased PBGD activity by approximately 50% (most cases)*

ALA, PBG*

[approximately 620 nm]
Acute neurovisceral, rarely blistering cutaneous HCP ALA, PBG, coproporphyrin III Coproporphyrin III *

Δ

[approximately 620 nm]
Acute neurovisceral, commonly blistering cutaneous VP ALA, PBG, coproporphyrin III Coproporphyrin III, protoporphyrin *

Porphyrin-peptide conjugate

[approximately 626 to 628 nm]
Cutaneous, blistering PCT and HEP Uroporphyrin, heptacarboxyl porphyrin Heptacarboxyl porphyrin, isocoproporphyrins Zinc protoporphyrin (markedly elevated in HEP, normal or mildly elevated in PCT)

Uroporphyrin, heptacarboxyl porphyrin

[approximately 620 nm]
Cutaneous, blistering CEP Uroporphyrin I; coproporphyrin I Coproporphyrin I Uroporphyrin I; coproporphyrin I

Uroporphyrin I, coproporphyrin I

[approximately 620 nm]
Cutaneous, nonblistering EPP and XLP Protoporphyrin* Metal-free protoporphyrin§

Protoporphyrin

[approximately 634 nm]
Refer to UpToDate topics on individual porphyrias for additional information.
ADP: delta-aminolevulinic acid (ALA) dehydratase porphyria; ALAD: ALA dehydratase; AIP: acute intermittent porphyria; PBG: porphobilinogen; PBGD: porphobilinogen deaminase; HCP: hereditary coproporphyria; VP: variegate porphyria; PCT: porphyria cutanea tarda; HEP: hepatoerythropoietic porphyria; CEP: congenital erythropoietic porphyria; EPP: erythropoietic protoporphyria; XLP: X-linked protoporphyria.
* Porphyrin levels normal or slightly increased.
¶ Fluorescence emission peak of diluted plasma at neutral pH.
Δ Plasma porphyrins usually normal, but increased when blistering skin lesions develop.
Urine porphyrins (especially coproporphyrin) increase only with hepatopathy.
§ Zinc protoporphyrin ≤15 percent of total in classic EPP, but 15 to 50% in variant form (XLP).
Provided by Karl Anderson, MD, FACP.
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