[Definition and classification of porphyrias].
According to the newest recognitions a disease entity is to be ascribed to every deficient enzyme of the chain of haemobiosynthesis, so that the subdivisions of the porphyrin diseases, having often changed since several decades, may be substituted by classifications on ascertained enzymatic basis. An establishment of the well-known porphyrias is given, taking into consideration the first describers, the hereditary factors and first individual of homozygous diseases.
Hautklinik, Bezirkskrankenhauses Dresden-Friedrichstadt.
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Köstler
D006418:Heme / Q000096:biosynthesis; D006801:Humans; D008107:Liver Diseases / Q000145:classification; D011164:Porphyrias / Q000145:classification*; D011165:Porphyrinogens / Q000096:biosynthesis; D012871:Skin Diseases / Q000145:classification
D011165:Porphyrinogens; D006418:Heme
Definition und Klassifikation der Porphyrien.