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Hepatoerythropoietic Porphyria (HEP)

Specialty:

Endocrinology, Nutrition & Dermatology
Clinical Laboratory
Gastroenterology

Objective:

ICD-10:

Description:

Hepatoertyropoietic porphyria (HEP) is a rare form of porphyria due to a marked defect in uroporphyrinogen III decarboxylase, the same enzyme affected in porphyria cutanea tarda (PCT). An excess production of porphyrins occurs in both the liver and red blood cells.

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