Catalog

Liver diseases due to porphyria

No MMS code

Link to WHO Definition

Liver diseases due to porphyria – http://id.who.int/icd/entity/554074681

The porphyrias are a group of rare inherited or acquired disorders of certain enzymes that normally participate in the production of porphyrins and heme. Pathogenesis is storage of porphyrins, porphyrinogens, and their precursors. They manifest with either neurological complications or skin problems, or occasionally both. Porphyrias are classified as hepatic or erythropoietic based on the sites of accumulation of heme precursors, either in the liver or bone marrow and red blood cells.