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1.
Pediatriia ; (7): 66-9, 1991.
Artigo em Russo | MEDLINE | ID: mdl-1721696

RESUMO

To define the clinico-pathogenetic importance of alpha 1-inhibitor of proteinases and alpha 2-macroglobulin of the blood in children with glomerulonephritis, a study was made of the phenotype of alpha 1-inhibitor of proteinases and its concentration in the blood serum of 156 patients with different clinical forms of glomerulonephritis. Overall 1290 practically healthy children were examined as control. The patients suffering from glomerulonephritis did not demonstrate phenotypes responsible for acute deficiency of alpha 1-inhibitor of proteinases (PISS, PISZ). A relationship was established between the amount of alpha 1-inhibitor of proteinases in the blood serum in children with different clinical forms of glomerulonephritis: the patients with the nephrotic form manifested a significant decrease of the inhibitor concentration in the blood serum, whereas in the hematuric form, a significant rise of it was recorded. All the patients suffering from glomerulonephritis showed a significant increase of the content of alpha 2-macroglobulin, particularly in the nephrotic form, which is likely to be determined by the enhanced output of the given protein and its negligible loss with urine in connection with a high molecular weight.


Assuntos
Glomerulonefrite Membranoproliferativa/sangue , Nefrose Lipoide/sangue , alfa 1-Antitripsina/análise , alfa-Macroglobulinas/análise , Adolescente , Criança , Pré-Escolar , Glomerulonefrite Membranoproliferativa/enzimologia , Glomerulonefrite Membranoproliferativa/genética , Humanos , Nefrose Lipoide/enzimologia , Nefrose Lipoide/genética , Fenótipo , alfa 1-Antitripsina/genética , Deficiência de alfa 1-Antitripsina , alfa-Macroglobulinas/genética
2.
Lab Delo ; (4): 17-9, 1989.
Artigo em Russo | MEDLINE | ID: mdl-2470956

RESUMO

Employment of isoelectrofocussing techniques for the determination of proteinase alpha 1-inhibitor (p alpha 1i) phenotypes helped detect original rare phenotypes. Their accurate identification should be performed with a set of standard sera. The findings of studies on the number of p alpha 1i by Mancini's immunoprecipitation test are analyzed, as are the data of phenotype studies by p alpha 1i isoelectrofocussing in children suffering from acute leukemia and sepsis. The findings evidence a drastic increase of p alpha 1i number, parallelled by the emergence of a manifest anode fraction of a common Pi MM phenotype; this may be regarded as a manifestation of a rare Pi MM anode phenotype in phenotyping. The possible reasons of this phenomenon are discussed; the authors emphasize the significance of analyzing rare p alpha 1i phenotypical variants with standard sera.


Assuntos
Infecções Bacterianas/genética , Proteínas Sanguíneas/genética , Leucemia/genética , Inibidores de Proteases/genética , Criança , Humanos , Fenótipo , alfa 1-Antitripsina
4.
Ter Arkh ; 60(12): 60-2, 1988.
Artigo em Russo | MEDLINE | ID: mdl-3266811

RESUMO

Some data on the determination of proteinase alpha 1-inhibitor phenotypes in children and adults with chronic non-specific pulmonary diseases were described. In pediatric patients the frequency of detection of heterozygotes PI MZ, PI MS did not exceed that in a healthy population whereas in adult patients heterozygotes were detected twice as more frequently. Among 149 adult patients one case of PI ZZ and one case of PI SS were detected. A patient with bronchial asthma had the MI ZZ phenotype. A possible frequency of the prevalence of PI ZZ phenotypes in a population and appropriateness of determination of blood levels and phenotypes of proteinase alpha 1-inhibitor in patients with bronchial asthma and other chronic nonspecific pulmonary diseases were considered.


Assuntos
Asma/sangue , Proteínas Sanguíneas/deficiência , Inibidores de Proteases/deficiência , Adulto , Asma/genética , Asma/patologia , Proteínas Sanguíneas/análise , Criança , Doença Crônica , Feminino , Humanos , Pneumopatias/sangue , Pneumopatias/genética , Linhagem , Fenótipo , Inibidores de Proteases/sangue , alfa 1-Antitripsina
7.
Vopr Med Khim ; 33(1): 54-9, 1987.
Artigo em Russo | MEDLINE | ID: mdl-3495068

RESUMO

Human homogeneous alpha-I-inhibitor of proteases was isolated and purified by means of chromatography on DEAE cellulose, hydroxyapatite and concanavalin Sepharose 4B. After immunization of rabbits specific monovalent antiserum towards the inhibitor was produced. The antiserum was used in diagnosis of hereditary deficiency of alpha-I-inhibitor of proteases and for estimation of its content in newborns.


Assuntos
Soros Imunes/isolamento & purificação , alfa 1-Antitripsina/análise , Humanos , Imunoquímica , Imunoeletroforese , alfa 1-Antitripsina/imunologia , alfa 1-Antitripsina/isolamento & purificação
8.
Ter Arkh ; 58(4): 77-80, 1986.
Artigo em Russo | MEDLINE | ID: mdl-3086999

RESUMO

The authors review biological and biochemical properties and the clinical importance of the serum proteases alpha 1-inhibitor with broad-range antiproteolytic activity. Congenital deficiency of this protein is a frequent enough condition linked with predisposition to some diseases of the lungs and liver. Early determination of the deficiency of alpha 1-Pi is fairly urgent, since it permits the early administration of the preventive measures and substitution therapy. The immunochemical technique makes it possible to determine all the varieties of alpha 1-Pi, as they are antigenically similar. Sera from 267 patients with different diseases were examined. The content of alpha 1-Pi was found to be elevated in Waldenström's macroglobulinemia, chronic active hepatitis and liver cirrhosis and to be lowered in bronchial asthma. In multiple myeloma and pollinoses, no alterations in the alpha 1-Pi content were recorded.


Assuntos
Asma/enzimologia , Hepatite Crônica/enzimologia , Cirrose Hepática/enzimologia , Paraproteinemias/enzimologia , Rinite Alérgica Sazonal/enzimologia , alfa 1-Antitripsina/análise , Adulto , Criança , Ativação Enzimática , Humanos , Recém-Nascido , Mieloma Múltiplo/enzimologia , Macroglobulinemia de Waldenstrom/enzimologia , Deficiência de alfa 1-Antitripsina
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