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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Detalles Bibliográficos
Autor principal: Guolo, M.
Otros Autores: Stella, A.M, Ferraris, J., Ramirez, J., De Enriquez Salamanca, R., Batlle, A.
Formato: Capítulo de libro
Lenguaje:Inglés
Español
Publicado: 1996
Acceso en línea:Registro en Scopus
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Registro en la Biblioteca Digital
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024 7 |2 cas  |a carbocisteine, 638-23-3; coproporphyrin, 27121-71-7; heme, 14875-96-8; porphyrin, 24869-67-8; uroporphyrin, 18273-06-8, 26316-36-9, 553-18-4, 607-14-7; uroporphyrinogen decarboxylase, 9024-70-8 
040 |a Scopus  |b spa  |c AR-BaUEN  |d AR-BaUEN 
041 0 |a eng  |a spa 
030 |a RADEB 
100 1 |a Guolo, M. 
245 1 0 |a Urodecarboxilasa en sangre de pacientes hemodializados 
260 |c 1996 
270 1 0 |m Centra de Investigaciones Sobre Porfirinas y Porfirias (CIPYP), Facultad de Ciencias Exactas y Naturales, UBA -Consejo Nacional de Investigaciones Cientificas y Técnicas(CONICET)Argentina 
506 |2 openaire  |e Política editorial 
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504 |a Mascarö, J., M.; Lecha, M.; Herrera, C.; Muniesa S. y Smith, S.: Common and Uncommon Dermatological Manifestations of Porphyrias. A Study Based on Our Personal Experience. XVI International Congress of Dermatology, Symposium of Porphyrias, Tokyo, Ed. Por Garsi S.A., Madrid, 1982, , pp. 13-28 
504 |a Rocchi, E., Cassanelli, M.; Borghi A.; Paolillo, F.; Pradelli, M.; Pellizardi, S.; Vezzosi, A.; Gallo, E.; Baccarani Contri, M. y Ventura, E.: Liver Iron Overload and Desferrioxamine Treatment of Porphyria Cutaneatarda., , Dermatologica, 18227-31,1991 
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504 |a Batlle, A., M. Del C.; Stella, A.M.; Kaminisky, A. R.; Kaminisky, C. y Mariano, H. G.: Two Cases of Infantil Porphyria Cutanea Tarda: Successful Treatment with Oral S-adenosyl-L-methionine and Low-dose of Oral Chloroquine. Br. J., , Dermatol. 116:407-415,1987 
504 |a Jeronimo, A., E.; Magnin, P.; Enriquez de Salamanca, R.; Batlle, A. M. Del C.; Stella. A. M.; Melito, V. y Parera, V: Porfiria Cutânea Tarda y Hepatitis. Rev. Arg. Derm. 76, , 139 -147,1995 
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504 |a Korting, G., W.: Über Porphyria-cutanea-tardaartige Langzeithamodialyse Patienten., , Dermatologica 150,58-61,1975 
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504 |a Trick, P.-F., M.B.; Masullo,A. S. y Grossman, M. E.: Porphyria Cutanea Tarda Associated with Chronic Renal Disease and Hemodialysis. Arch., , Dermatol. 116,191-195,1980 
504 |a Topi, G., C.; D'Alessandro Gandolfo, L.; Costanza, F. y de Cancarini, G. C.: Porphyria and Pseudoporphyria in Hemodialyzed Patients. Int. J., , Biochem. 12,963-967,1980 
504 |a Seubert, S., Seubert, A.; Wolfgang Rumpf, K. y Kiffe, H.: A Porphyria Cutanea Tarda - Like Distribution Pattern of Porphyrins in Plasma, Hemodialysate, Hemofil Träte, and Urine of Patients on Chronic Hemodialysis. J. Invest. Dermatol., , 85, 107-109,1985 
504 |a Guolo, M., Stella, A. M.; Melito, V; Parera, V. y Batlle, A. M. Del C.: Altered D-aminolevulinic Acid Metabolism Leading to Pseudoporphyria in Hemodialysed Patients. Int J. Biochem. & Cell Biol., , 28;311-317, 1996 
504 |a Stellaa, M., Melito, V; Parera, V. Cohan, R.; Hoyo, G.; Garcia Dîaz, R.; Pierini, A.; Ferraris, J. y Batlle, A. M. Del C.: Acerca de Dos Nuevos Casos de Porfiria Cutânea Tardia Infantil en Una Nina Con Leucemia y en un Nino Hemodializado en Tratamiento Con Sadenosil-L-metionina, Aspectos Bioquîmicos. Rev. Arg. Dermatol., , 69; 118-124,1988 
504 |a Schoua, A., E. M. y Batlle, A. M. Del C.: Indice de Porfirinas Plasmâticas: IPP. un Método Râpido Para la Caracterizacion de Las Porfirinas. Rev. Arg. Derm., , 68, 79-85,1987 
504 |a Rimington, C., Spectral-absorption Coefficients of Some Porphyrins in the Soret-band Region. Biochem J 75,620, , - 624,1960 
504 |a Seubert, A., Y Seubert,S. High-performance Liquid Chromatographie Analysis of Porphyrins and Their Isomers with Radical Compression Columns. Anal Biochem 124,303, , - 307,1982 
504 |a Mamet, R., Gafter, U.; Korzets, A. y Schoenfeld, N.: Decreased Uroporphyrinogen Decarboxylase Activity in Patients with End-stage Renal Disease Undergoing Hemodialysis. Nephron 70, , 202-206, 1995 
504 |a Senger, E., Bernd, A.; Kachel, H. G.; Schoeppe, W. y Holzmann, H.: Porphyria Cutanea Uraemica: An Obligate Systemic Disease in Chronic Kidney Insufficiency?, , Hautarzt 42,764-769,1991 
504 |a Fried, W., Hématologie Aspects of Uremia, in Clinical Dialysis, Ed. Por Allen, R.; Nissensori, D. E.; Gentile, R. Fine, 2da Ed., Norwalk, Connecticut/San Mateo, California, 1990, Pp, , 391 -408 
504 |a Smith, A., G. y Francis J. E.: Investigation of Rat Liver Uroporphyrinogen Decarboxylase Comparisons of Uroporphyrinogens i and 111 As Substrates and the Inhibition by Porphyrins. Biochem J., , 241,50 -61,1981 
504 |a Mukerji, S., K.; Pimstone, N. R. y Burns, M.: Dual Mechanism of Inhibition of Rat Liver Uroporphyrinogen Decarboxylase Activity by Ferrous Iron: Its Potential Role in the Genesis of Porphyria Cutanea Tarda. Gastroenterology 87, , 1248-1254, 1984 
504 |a Siersema, P., D.; Rademakers, L. H. P.; Cleton, M. 1.; Tenkate, F. J. W.; Debruijn, W. C; Marx, J. J. M. y Wilson, J. H. P. the Difference in Liver Pathology between Sporadic and Familial Forms of Porphyria Cutanea Tarda: the Role of Iron. J. Hepatol., , 23,259 267,1995 
504 |a Stella, A., M.; Rocchi, E.; Varela, L.; Parera, V.; Casalgrandi, G.; Magnin, P. H. y Batlle A. M. Del C.: Vitaminas Liposolubles y Carotenoides Naturales en Pacientes Porfiricos. Rev. Arg. Derm., , 76, 164-170,1995 
504 |a Guolo, M., Biscoglio, M.; Machalinski, C.; Stella, A. M.; Franco, A.; Pataro, L. y Batlle, A. M. Del C.: Isolation of An Inhibitor of D-aminolevulinic Acid Dehydratase Activity from Plasma of Hemodialysed Patients. en Proceedings of the Laboratory Medicine Llth IFCC European Congress of Clinical Chemistry, Helsinki, Finlandia, , pp 149,1995 
504 |a Ramsay, C., A. y Obreshkova, E.: Photosensitivity from Nalidixic Acid. Br J, , Dermatol. 91,523-528,1974 
504 |a Cantarovich, F., Galli, C; Benedetti, L.; Chena, C.; Castro, L.; Correa, C.; Ferez Loredo, J.; Femandez, J. C.; Locatellin, A. y Tizado, J.: High Dose Furesemide in Established Acute Renal Failure. Br Med, , J 24,449-450,1973 
504 |a Fontanellas, A., Herrero, J. A.; Moran, M. J.; Coronel, F.; Sepülveda, P.; Barrientos, A. y Enrîquez de Salamanca, R.: Efficiency of Three Different Hemodiaysis Membranes for Plasma Porphyrin Removal. Am J. Kidney, , Dis 25,30-33,1945 
504 |a Morân, M., J.; Fontanellas, A.; Santos, J. L. y Enrîquez de Salamanca, R. E.: Correlation between Levels of Free and Protein-bound Plasma Porphyrin and Urinary Porphyrins in Porphyria Cutanea Tarda. Int. J. Biochem. Cell Biol, , 27; 585588,1995 
520 3 |a 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.  |l eng 
593 |a Centra de Investigaciones Sobre Porfirinas y Porfirias (CIPYP), Facultad de Ciencias Exactas y Naturales, UBA -Consejo Nacional de Investigaciones Cientificas y Técnicas(CONICET), Argentina 
593 |a Servicio de Hemodialisis Infantil Del Hospital Italiano 
593 |a Hospital 12 de Octubre, Universidad Complutense, Madrid, United States 
593 |a Becario de Perfeccionamiento Del CONICET, Argentina 
690 1 0 |a DECARBOXILASE 
690 1 0 |a HEMODIALYSED PATIENTS 
690 1 0 |a PORPHYRIA CUTANEA TARDA 
690 1 0 |a PSEUDOPORPHYRIA 
690 1 0 |a UROPORPHYRINOGEN 
690 1 0 |a CARBOCISTEINE 
690 1 0 |a COPROPORPHYRIN 
690 1 0 |a HEME 
690 1 0 |a PORPHYRIN 
690 1 0 |a UROPORPHYRIN 
690 1 0 |a UROPORPHYRINOGEN DECARBOXYLASE 
690 1 0 |a ADULT 
690 1 0 |a ARTICLE 
690 1 0 |a CLINICAL ARTICLE 
690 1 0 |a CONTROLLED STUDY 
690 1 0 |a ENZYME ACTIVITY 
690 1 0 |a FEMALE 
690 1 0 |a HEMODIALYSIS 
690 1 0 |a HUMAN 
690 1 0 |a LIVER FUNCTION 
690 1 0 |a MALE 
690 1 0 |a METABOLISM 
690 1 0 |a PORPHYRIA CUTANEA TARDA 
700 1 |a Stella, A.M. 
700 1 |a Ferraris, J. 
700 1 |a Ramirez, J. 
700 1 |a De Enriquez Salamanca, R. 
700 1 |a Batlle, A. 
773 0 |d 1996  |g v. 77  |h pp. 155-162  |k n. 3  |p Rev. Argent. Dermatol.  |x 03252787  |t Revista Argentina de Dermatologia 
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