RESUMO
A 13 year-old girl with clinical features of Addison's disease developed acute cardiac failure after initiation of treatment and after initial clinical improvement. Large doses of i.v. hydrocortisone and oral fludrocortisone, in addition to inotropic and ventilatory support, were required to achieve cardiovascular stability. The cardiomyopathy improved over one week and her condition then remained stable on oral glucocorticoid and mineralocorticoid replacement therapy. Reversible cardiomyopathy is a rare and potentially life-threatening complication of Addison's disease. The second reported paediatric patient is presented, the only one reported to require ventilatory support.
Assuntos
Doença de Addison/complicações , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/tratamento farmacológico , Doença de Addison/diagnóstico , Doença de Addison/tratamento farmacológico , Administração Oral , Adolescente , Austrália , Cardiomiopatia Dilatada/diagnóstico , Cardiotônicos/uso terapêutico , Dobutamina/farmacologia , Dobutamina/uso terapêutico , Dopamina/farmacologia , Dopamina/uso terapêutico , Esquema de Medicação , Ecocardiografia , Feminino , Fludrocortisona/administração & dosagem , Fludrocortisona/uso terapêutico , Hidratação , Humanos , Hidrocortisona/administração & dosagem , Hidrocortisona/uso terapêutico , Injeções Intravenosas , Fatores de Tempo , Resultado do TratamentoRESUMO
Earlier attempts to purify and characterize nonrecombinant pyruvate kinase from Schizosaccharomyces pombe proved difficult due to problems associated with the instability of the protein. The enzyme has been overexpressed in Saccharomyces cerevisiae strain AH22, permitting studies to determine the conditions required to stabilize the enzyme during purification. Recombinant S. pombe pyruvate kinase was purified by a combination of ion-exchange chromatography and gel filtration. The purified enzyme showed sigmoidal kinetics with respect to PEP; in the presence of FBP, the kinetics were restored to Michaelis-Menten behavior. With respect to ADP, the Hill coefficient was not affected by FBP. Determination of the molecular mass of the purified enzyme by ultracentrifugation showed that it behaved as a dimer-tetramer system with a Kd of approximately 1 microM.
Assuntos
Conformação Proteica , Piruvato Quinase/química , Proteínas Recombinantes/química , Schizosaccharomyces/enzimologia , Difosfato de Adenosina/metabolismo , Frutosedifosfatos/farmacologia , Proteínas Fúngicas/química , Expressão Gênica/genética , Cinética , Peso Molecular , Fosfoenolpiruvato/metabolismo , Plasmídeos/genética , Saccharomyces cerevisiae/genética , UltracentrifugaçãoRESUMO
Fifty brands of margarine were analysed for cis-polyunsaturated acids by lipoxidase, for trans fatty acid by infared spectroscopy, and for fatty acid composition by gas-liquid chromatography. High concentrations of trans fatty acids tended to be associated with low concentrations of linoleic acid. Later analyses on eight of the brands, respresenting various proportions of linoleic to trans fatty acids, indicated that two of them contained still higher levels of trans fatty acids (greater than 60%) and negligible amounts of linoleic acid. It is proposed that margarine could be a vehicle for the distribution of some dietary linoleic acid and that the level of linoleic acid and the summation of the saturated plus trans fatty acids be known to ascertain nutritional characteristics.