| Popis: |
Porphyrias are known as disorders of the heme biosynthesis pathway. There are about eight metabolic disorders that make up the porphyrias. These eight metabolic disorders are porphyria cutanea tarda, acute intermittent porphyria, ALA-dehydratase deficiency porphyria, hepatoerythropoietic porphyria, variegate porphyria, hereditary coproporphyria, congenital erythropoietic porphyria, erythropoietic protoporphyria, and X-linked protoporphyria. These metabolic disorders can be classified as either erythropoietic or hepatic porphyria. Additionally, these metabolic disorders can be classified as either cutaneous or acute based on the signs and symptoms. This article looks at the pathophysiology, clinical manifestations, diagnosis, treatments, and novel drug therapy options of porphyrias in hopes of increasing awareness of these diseases and dosage forms among healthcare professionals. |
