| porphyria, erythropoietic | Autosomal recessive porphyria characterised by splenomegaly, photosensitivity, haemolytic anaemia, and the appearance of red urine in early infancy. This condition results from increased synthesis of uroporphyrinogen I relative to uroporphyrinogen III in bone marrow normoblasts. (12 Dec 1998) |
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| porphyria, hepatic | Porphyria in which the liver is the site where excess formation of porphyrin or its precursors is found. Porphyria, acute intermittent and porphyria cutanea tarda are types of hepatic porphyria. (12 Dec 1998) |
| porphyrin | <protein> Porphyrins are pigments found in both animal and plant life. They are all chelates with metals (Fe, Mg, Co, Zn, Cu, Ni) and constituents of haemoglobin, chlorophyll, cytochromes. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Increased protoporphyrins may be seen in infection, thalassaemia, sideroblastic anaemia, iron deficient anaemia, increased erythropoiesis and lead poisoning. Increased uroporphyrins may indicate congenital erythropoietic porphyria or erythropoietic protoporphyria. (27 Sep 1997) |
| porphyrin cytochrome c peroxidase | <enzyme> From yeast; haem group of cytochrome c peroxidase (EC 1.11.1.5) replaced by protoporphyrin ix Registry number: EC 1.11.1.- Synonym: pcc-peroxidase (26 Jun 1999) |
| porphyrinogen carboxy-lyase | <enzyme> Consider uroporphyrinogen decarboxylase Registry number: EC 4.1.1.- (26 Jun 1999) |
| porphyrinogens | Colourless reduced precursors of porphyrins in which the pyrrole rings are linked by methylene (-ch2-) bridges. (12 Dec 1998) |
| porphyrinopathy | A syndrome which results from abnormal porphyrin metabolism such as acute porphyria. Synonym: porphyrism. Origin: porphyrin + G. Pathos, disease (05 Mar 2000) |
| 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, haematoporphyrin, etc. The porphyrins, in combination with iron, form the haem component in biologically significant compounds such as haemoglobin and myoglobin. (12 Dec 1998) |
| porphyrinuria | Excretion of porphyrins and related compounds in the urine. Synonym: porphyruria, purpurinuria. (05 Mar 2000) |
| porphyrism | A syndrome which results from abnormal porphyrin metabolism such as acute porphyria. Synonym: porphyrism. Origin: porphyrin + G. Pathos, disease (05 Mar 2000) |
| porphyrite | <chemical> A rock with a porphyritic structure; as, augite porphyrite. Source: Websters Dictionary (01 Mar 1998) |
| porphyritic | <chemical> Relating to, or resembling, porphyry, that is, characterised by the presence of distinct crystals, as of feldspar, quartz, or augite, in a relatively fine-grained base, often aphanitic or cryptocrystalline. Origin: Cf. F. Porphyritique. Source: Websters Dictionary (01 Mar 1998) |
| porphyrization | Grinding in a mortar (formerly on a slab of porphyry). (05 Mar 2000) |
| porphyrogenitism | The principle of succession in royal families, especially among the Eastern Roman emperors, by which a younger son, if born after the accession of his father to the throne, was preferred to an elder son who was not so born. Origin: LL. Porphyro genitus, fr. Gr.; purple + root of to be born. Source: Websters Dictionary (01 Mar 1998) |
| porphyromonas | A genus of gram-negative, anaerobic, nonsporeforming, nonmotile rods or coccobacilli. Organisms in this genus had originally been classified as members of the bacteroides genus but overwhelming biochemical and chemical findings indicated the need to separate them from other bacteroides species, and hence, this new genus was created. (12 Dec 1998) |
Synonyms : Congenital Erythropoietic Porphyria, Gunther Disease, Congenital Erythropoietic Porphyrias, Disease, Gunther, Disease, Gunther's, Erythropoietic Porphyria, Congenital, Erythropoietic Porphyrias, Erythropoietic Porphyrias, Congenital, Gunthers Disease
Synonyms : Erythrohepatic Porphyria, Erythrohepatic Porphyrias, Hepatoerythropoietic Porphyrias, Porphyrias, Erythrohepatic, Porphyrias, Hepatoerythropoietic
Synonyms : Protoporphyrinogen Oxidase Deficiency, Variegate Porphyria, Deficiencies, Protoporphyrinogen Oxidase
Synonyms : Porphyria
Synonyms : Porphyria, Hepatic, Hepatic Porphyrias
| porphyria |
A group of a rare inherited blood disorders. Porphyria is associated with a cell's failure to transform biochemicals into other biochemicals which can then be further metabolized and excreted through the feces or urine. Porphyrins can then build up within the body. In fact, urine may be colored blue because of this. Metal disorders are associated with this.
출처: www.gastromd.com/definitionsp.html
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| porphyria |
group of rare, inherited blood disorders in which cells fail to change chemicals (porphyrins) to the substance (heme) that gives blood its color.
출처: ymghealthinfo.org/content.asp
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| porphyria |
Abnormalities of porphyrin metabolism (often hereditary) characterized by excretion of excess porphyrins in the urine.
출처: www.springboard4health.com/notebook/dict_p.html
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| porphyrin |
compounds of a structure that consists of four pyrrole rings joined by four =C- groups; esp: one (as chorophyll or hemoglobin) containing a central metal atom, and usually having biological activity.
출처: www.springboard4health.com/notebook/dict_p.html
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| porphyria erythropoietica |
A mild form of porphyria characterized by cutaneous lesions and excess protoporphyrin in the erythrocytes and feces. SYN: protoporphyria.
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