Pathogenesis of neural manifestations in acute porphyria
| dc.contributor.author | Shanley, B. C. | en_ZA |
| dc.contributor.author | Percy, V. A. | en_ZA |
| dc.contributor.author | Neethling, A. C. | en_ZA |
| dc.date.accessioned | 2011-03-18T14:57:53Z | |
| dc.date.available | 2011-03-18T14:57:53Z | |
| dc.date.issued | 1977 | |
| dc.description | CITATION: Shanley, B. C., Percy, V. A. & Neethling, A. C. 1977. Pathogenesis of neural manifestations in acute porphyria. South African Medical Journal, 51:458-460. | |
| dc.description | The original publication is available at http://www.samj.org.za | |
| dc.description.abstract | At least 4 possible mechanisms may be postulated to explain the neural manifestations of acute porphyria in the hereditary hepatic porphyrias. These are: (i) excessive amounts of porphyrins or porphyrin precursors produced in the liver during acute attacks are transported to the central and peripheral nervous system, where they exert neurotoxic effect; (ii) unidentified metabolites of the aforementioned compounds may be responsible; (iii) in patients with these diseases there may be a metabolic defect in neural haem biosynthesis which is aggravated by precipitating factors, thereby leading to acute neural manifestations; and (iv) the hepatic and nervous system lesions may be metabolically quite unrelated. Each of these possibilities is considered, and evidence is adduced that a genetic defect in haem biosynthesis in the nervous system is the most plausible hypothesis. | |
| dc.description.version | Publisher’s version | |
| dc.format.extent | 3 pages | |
| dc.identifier.issn | 2078-5135 (online) | |
| dc.identifier.issn | 0256-9574 (print) | |
| dc.identifier.uri | http://hdl.handle.net/10019.1/7713 | |
| dc.language.iso | en | |
| dc.publisher | Health & Medical Publishing Group | |
| dc.rights.holder | South African Medical Journal | |
| dc.subject | Porphyria | en_ZA |
| dc.title | Pathogenesis of neural manifestations in acute porphyria | en_ZA |
| dc.type | Article |
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