[Hepatic porphyria]. 1979

G Tornaghi, and L Vitali

Porphyria is making increasing demands on the attention of clinicians and research worker. An account is given of hepatic forms, since these have recently come into prominence on account of recent advances in the understanding of their metabolic, diagnostic and therapeutic aspects. A description of the physiopathology of porphyrin metabolism is followed by an examination of the incidence, genetic features, aetiology, pathogenesis, pathological anatomy, symptomatology, diagnosis, prognosis, and treatment of each form. Particular attention is devoted to intermittent acute and cutanea tarda porphyria, since these are more commonly encountered in practice. Personal experience gathered in a large series of cases of cutanea tarda porphyria is presented.

UI MeSH Term Description Entries
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008107 Liver Diseases Pathological processes of the LIVER. Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D011164 Porphyrias A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues. Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D011166 Porphyrins A group of compounds containing the porphin structure, four pyrrole rings connected by methine bridges in a cyclic configuration to which a variety of side chains are attached. The nature of the side chain is indicated by a prefix, as uroporphyrin, hematoporphyrin, etc. The porphyrins, in combination with iron, form the heme component in biologically significant compounds such as hemoglobin and myoglobin. Porphyrin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000622 Aminolevulinic Acid A compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS. 5-Amino Levulinic Acid,5-Aminolaevulinate,5-Aminolevulinate,Aminolevulinic Acid Hydrochloride,Delta-Aminolevulinic Acid,Levulan,5 Amino Levulinic Acid,5 Aminolaevulinate,5 Aminolevulinate,Acid Hydrochloride, Aminolevulinic,Acid, 5-Amino Levulinic,Acid, Aminolevulinic,Acid, Delta-Aminolevulinic,Delta Aminolevulinic Acid,Hydrochloride, Aminolevulinic Acid,Levulinic Acid, 5-Amino

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