In inherited porphyrias, lead intoxication is a toxogenetic disorder
1. 1. δ-Aminolevulinic acid dehydratase (ALA-D), blood lead and several enzymes and metabolites of the heme biosynthetic pathway were measured in a number of symptomatic porphyric patients, 22 with acute intermittent porphyria, three with hereditary hepatic coproporphyria, 10 with hereditary porphyr...
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1987
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| Acceso en línea: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v19_n8_p717_DelCBatlle http://hdl.handle.net/20.500.12110/paper_0020711X_v19_n8_p717_DelCBatlle |
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paper:paper_0020711X_v19_n8_p717_DelCBatlle2023-06-08T14:41:09Z In inherited porphyrias, lead intoxication is a toxogenetic disorder lead porphobilinogen synthase acute intermittent porphyria blood blood and hemopoietic system congenital disorder congenital erythropoietic porphyria erythropoietic protoporphyria etiology human intoxication lead poisoning porphyria porphyria cutanea tarda priority journal review Heme Humans Lead Lead Poisoning Porphobilinogen Synthase Porphyrias 1. 1. δ-Aminolevulinic acid dehydratase (ALA-D), blood lead and several enzymes and metabolites of the heme biosynthetic pathway were measured in a number of symptomatic porphyric patients, 22 with acute intermittent porphyria, three with hereditary hepatic coproporphyria, 10 with hereditary porphyria cutanea tarda, two with erythropoietic protoporphyria and two with congenital erythropoietic porphyria and in 84 lead intoxicated persons. 2. 2. In the 39 individuals suffering from the inherited porphyrias and in 32 lead poisoned patients with a 30-50% reduced deaminase, blood lead content was not sufficiently increased (average 28 μg%) to account for the greatly decreased activity of ALA-D (average 36% of controls). 3. 3. After a relatively trifling lead exposure they developed the signs of acute lead intoxication. 4. 4. A second group of lead intoxicated patients showing low ALA-D activity and corresponding high concentration of lead in blood, exhibited no other physiologic deviation in the enzymes and metabolites of porphyrin biosynthesis. 5. 5. Individuals with inherited porphyrias are ultrasensitive to low level lead exposure and that lead would also act as a triggering factor. In these patients, lead intoxication can be considered a toxogenetic disorder. 6. 6. An inversely linear correlation between ALA-D activity and blood lead content was obtained for both groups of lead intoxicated patients, however, a different constant (k) for each was obtained, which we have taken as a measure of lead toxogeneticity: k = 10 ± 1 for lead intoxicated individuals with otherwise normal heme metabolism and k = 5 ± 0.5 for lead intoxicated symptomatic porphyric patients. 7. 7. Determination of erythrocytic ALA-D, besides blood lead, will be a valuable indicator for preventive medical care for these patients, when they are expected to be exposed to lead either environmentally or in their professional life. © 1987. 1987 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v19_n8_p717_DelCBatlle http://hdl.handle.net/20.500.12110/paper_0020711X_v19_n8_p717_DelCBatlle |
| institution |
Universidad de Buenos Aires |
| institution_str |
I-28 |
| repository_str |
R-134 |
| collection |
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) |
| topic |
lead porphobilinogen synthase acute intermittent porphyria blood blood and hemopoietic system congenital disorder congenital erythropoietic porphyria erythropoietic protoporphyria etiology human intoxication lead poisoning porphyria porphyria cutanea tarda priority journal review Heme Humans Lead Lead Poisoning Porphobilinogen Synthase Porphyrias |
| spellingShingle |
lead porphobilinogen synthase acute intermittent porphyria blood blood and hemopoietic system congenital disorder congenital erythropoietic porphyria erythropoietic protoporphyria etiology human intoxication lead poisoning porphyria porphyria cutanea tarda priority journal review Heme Humans Lead Lead Poisoning Porphobilinogen Synthase Porphyrias In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| topic_facet |
lead porphobilinogen synthase acute intermittent porphyria blood blood and hemopoietic system congenital disorder congenital erythropoietic porphyria erythropoietic protoporphyria etiology human intoxication lead poisoning porphyria porphyria cutanea tarda priority journal review Heme Humans Lead Lead Poisoning Porphobilinogen Synthase Porphyrias |
| description |
1. 1. δ-Aminolevulinic acid dehydratase (ALA-D), blood lead and several enzymes and metabolites of the heme biosynthetic pathway were measured in a number of symptomatic porphyric patients, 22 with acute intermittent porphyria, three with hereditary hepatic coproporphyria, 10 with hereditary porphyria cutanea tarda, two with erythropoietic protoporphyria and two with congenital erythropoietic porphyria and in 84 lead intoxicated persons. 2. 2. In the 39 individuals suffering from the inherited porphyrias and in 32 lead poisoned patients with a 30-50% reduced deaminase, blood lead content was not sufficiently increased (average 28 μg%) to account for the greatly decreased activity of ALA-D (average 36% of controls). 3. 3. After a relatively trifling lead exposure they developed the signs of acute lead intoxication. 4. 4. A second group of lead intoxicated patients showing low ALA-D activity and corresponding high concentration of lead in blood, exhibited no other physiologic deviation in the enzymes and metabolites of porphyrin biosynthesis. 5. 5. Individuals with inherited porphyrias are ultrasensitive to low level lead exposure and that lead would also act as a triggering factor. In these patients, lead intoxication can be considered a toxogenetic disorder. 6. 6. An inversely linear correlation between ALA-D activity and blood lead content was obtained for both groups of lead intoxicated patients, however, a different constant (k) for each was obtained, which we have taken as a measure of lead toxogeneticity: k = 10 ± 1 for lead intoxicated individuals with otherwise normal heme metabolism and k = 5 ± 0.5 for lead intoxicated symptomatic porphyric patients. 7. 7. Determination of erythrocytic ALA-D, besides blood lead, will be a valuable indicator for preventive medical care for these patients, when they are expected to be exposed to lead either environmentally or in their professional life. © 1987. |
| title |
In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| title_short |
In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| title_full |
In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| title_fullStr |
In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| title_full_unstemmed |
In inherited porphyrias, lead intoxication is a toxogenetic disorder |
| title_sort |
in inherited porphyrias, lead intoxication is a toxogenetic disorder |
| publishDate |
1987 |
| url |
https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v19_n8_p717_DelCBatlle http://hdl.handle.net/20.500.12110/paper_0020711X_v19_n8_p717_DelCBatlle |
| _version_ |
1768542534382911488 |