Urodecarboxilasa en sangre de pacientes hemodializados
in porphyria cutanea tarda (PCT), there is a fault in the activity of hepatic uroporphyrinogen decarboxylase (URO-D), and as a consequence, the concentration of highly carboxylated porphyrins is increased. However, the activity of blood URO-D is diminished only in hereditary PCT and is normal in the...
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1996
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| Acceso en línea: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252787_v77_n3_p155_Guolo http://hdl.handle.net/20.500.12110/paper_03252787_v77_n3_p155_Guolo |
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paper:paper_03252787_v77_n3_p155_Guolo2023-06-08T15:32:08Z Urodecarboxilasa en sangre de pacientes hemodializados Decarboxilase Hemodialysed patients Porphyria cutanea tarda Pseudoporphyria Uroporphyrinogen carbocisteine coproporphyrin heme porphyrin uroporphyrin uroporphyrinogen decarboxylase adult article clinical article controlled study enzyme activity female hemodialysis human liver function male metabolism porphyria cutanea tarda in porphyria cutanea tarda (PCT), there is a fault in the activity of hepatic uroporphyrinogen decarboxylase (URO-D), and as a consequence, the concentration of highly carboxylated porphyrins is increased. However, the activity of blood URO-D is diminished only in hereditary PCT and is normal in the acquired form, The appearance of cutaneous blistering eruptions in hemodialysed patients has often been reported. Cutaneuous lesions are histoiogically and morphologically similar to those exhibited in PCT. The activity of URO-D was determined in red blood cells (RBC) from 12 hemodialysed patients, without cutaneous symptoms, one of them being carrier of hereditary PCT. Plasma porphyrins were also measured. The levels of plasma porphyrins were increased (0.084 ±1.0 ug/ml; uroporphyrins = 80%, coproporphyrins = 20%) in 30% of the non porphyric patients pior to hemodialysis (p < 0.001) compared with controls (normal value: 0.048 ±ug/ml; coproporphyrins 100%). Plasma porphyrins of the PCT patient at the begining of the treatment with S-adenosyl-L-methionine was: 1.71 ug/ml, uroporphyrins = 48%, heptaporphyrins = 41 %, hexaporphyrins = 7%, pentaporphyrins = 3% and coproporphyrins = 1 %. Six years after the end of treatment, lasma porphyrin vale was: 0.089 ug/ml; uroporphyrins = 80%, heptaporphyrins = 20%. Clinical remission was correlated with biochemical recovery. The activity of RBC URO-D was within the normal value (12.45 ±1.0 U/ml RBC) in all patients, except the true PCT case in whom the activity was reduced 50%. These results show that hemodialysis treatmentperse would not modify blood URO-D activity. Since plasma porphyrins levels were rised in a third of these patients, it is suggested that the observed abnormalities in heme metabolism may be due to altered hepatic function. 1996 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252787_v77_n3_p155_Guolo http://hdl.handle.net/20.500.12110/paper_03252787_v77_n3_p155_Guolo |
| institution |
Universidad de Buenos Aires |
| institution_str |
I-28 |
| repository_str |
R-134 |
| collection |
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) |
| topic |
Decarboxilase Hemodialysed patients Porphyria cutanea tarda Pseudoporphyria Uroporphyrinogen carbocisteine coproporphyrin heme porphyrin uroporphyrin uroporphyrinogen decarboxylase adult article clinical article controlled study enzyme activity female hemodialysis human liver function male metabolism porphyria cutanea tarda |
| spellingShingle |
Decarboxilase Hemodialysed patients Porphyria cutanea tarda Pseudoporphyria Uroporphyrinogen carbocisteine coproporphyrin heme porphyrin uroporphyrin uroporphyrinogen decarboxylase adult article clinical article controlled study enzyme activity female hemodialysis human liver function male metabolism porphyria cutanea tarda Urodecarboxilasa en sangre de pacientes hemodializados |
| topic_facet |
Decarboxilase Hemodialysed patients Porphyria cutanea tarda Pseudoporphyria Uroporphyrinogen carbocisteine coproporphyrin heme porphyrin uroporphyrin uroporphyrinogen decarboxylase adult article clinical article controlled study enzyme activity female hemodialysis human liver function male metabolism porphyria cutanea tarda |
| description |
in porphyria cutanea tarda (PCT), there is a fault in the activity of hepatic uroporphyrinogen decarboxylase (URO-D), and as a consequence, the concentration of highly carboxylated porphyrins is increased. However, the activity of blood URO-D is diminished only in hereditary PCT and is normal in the acquired form, The appearance of cutaneous blistering eruptions in hemodialysed patients has often been reported. Cutaneuous lesions are histoiogically and morphologically similar to those exhibited in PCT. The activity of URO-D was determined in red blood cells (RBC) from 12 hemodialysed patients, without cutaneous symptoms, one of them being carrier of hereditary PCT. Plasma porphyrins were also measured. The levels of plasma porphyrins were increased (0.084 ±1.0 ug/ml; uroporphyrins = 80%, coproporphyrins = 20%) in 30% of the non porphyric patients pior to hemodialysis (p < 0.001) compared with controls (normal value: 0.048 ±ug/ml; coproporphyrins 100%). Plasma porphyrins of the PCT patient at the begining of the treatment with S-adenosyl-L-methionine was: 1.71 ug/ml, uroporphyrins = 48%, heptaporphyrins = 41 %, hexaporphyrins = 7%, pentaporphyrins = 3% and coproporphyrins = 1 %. Six years after the end of treatment, lasma porphyrin vale was: 0.089 ug/ml; uroporphyrins = 80%, heptaporphyrins = 20%. Clinical remission was correlated with biochemical recovery. The activity of RBC URO-D was within the normal value (12.45 ±1.0 U/ml RBC) in all patients, except the true PCT case in whom the activity was reduced 50%. These results show that hemodialysis treatmentperse would not modify blood URO-D activity. Since plasma porphyrins levels were rised in a third of these patients, it is suggested that the observed abnormalities in heme metabolism may be due to altered hepatic function. |
| title |
Urodecarboxilasa en sangre de pacientes hemodializados |
| title_short |
Urodecarboxilasa en sangre de pacientes hemodializados |
| title_full |
Urodecarboxilasa en sangre de pacientes hemodializados |
| title_fullStr |
Urodecarboxilasa en sangre de pacientes hemodializados |
| title_full_unstemmed |
Urodecarboxilasa en sangre de pacientes hemodializados |
| title_sort |
urodecarboxilasa en sangre de pacientes hemodializados |
| publishDate |
1996 |
| url |
https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252787_v77_n3_p155_Guolo http://hdl.handle.net/20.500.12110/paper_03252787_v77_n3_p155_Guolo |
| _version_ |
1768543181394149376 |