Asunto(s)
Adrenoleucodistrofia/dietoterapia , Ácidos Erucicos/uso terapéutico , Ligamiento Genético , Cromosoma X , Adolescente , Adrenoleucodistrofia/sangre , Adrenoleucodistrofia/genética , Niño , Grasas de la Dieta/administración & dosificación , Ácidos Grasos/sangre , Estudios de Seguimiento , Humanos , MasculinoRESUMEN
We report the biochemical and clinical results obtained during a dietary erucic acid (C22:1) therapy in 20 patients affected by X-linked adrenoleukodystrophy (ALD). Six patients were very severely affected, 9 had milder neurological symptoms and 5 were presymptomatic. Mean basal levels of plasma C26:0 were 1.41 +/- 0.48 micrograms/ml in ALD patients (control values: 0.33 +/- 0.12). In all patients C26:0 decreased to virtually normal values. In spite of good biochemical response and absence of consistent side effects of therapy, no encouraging data were observed during the clinical follow-up. The presymptomatic subjects were still free of symptoms after more than 1 year of therapy. The symptomatic patients, however worsened or did not show any improvement.
Asunto(s)
Adrenoleucodistrofia/dietoterapia , Grasas de la Dieta/uso terapéutico , Ácidos Erucicos/uso terapéutico , Ácidos Grasos/metabolismo , Adolescente , Adrenoleucodistrofia/metabolismo , Adulto , Niño , Coenzima A Ligasas/deficiencia , Coenzima A Ligasas/metabolismo , Grasas de la Dieta/administración & dosificación , Enfermedades en Gemelos , Estudios de Seguimiento , Humanos , Masculino , Microcuerpos/enzimología , Gemelos DicigóticosAsunto(s)
Complejo Relacionado con el SIDA/terapia , Síndrome de Inmunodeficiencia Adquirida/terapia , Interferón Tipo I/uso terapéutico , Suramina/uso terapéutico , Complejo Relacionado con el SIDA/tratamiento farmacológico , Síndrome de Inmunodeficiencia Adquirida/tratamiento farmacológico , Adulto , Femenino , Humanos , Interferón Tipo I/efectos adversos , Masculino , Proteínas Recombinantes/efectos adversos , Proteínas Recombinantes/uso terapéutico , Sarcoma de Kaposi/terapiaRESUMEN
The effect of molybdate on the kinetic and thermodynamic properties of the dexamethasone-receptor interaction was studied in calf thymus cytosol. In the presence of molybdate both the equilibrium binding studies and the association and dissociation experiments reveal a significantly lower affinity of the receptor for [3]dexamethasone. At 0 degrees C the equilibrium dissociation constant increases from 0.8 nM to 1.8 nM, the association rate constant shifts from 1.5 X 10(8) M-1 h-1 to 0.2 X 10(8) M-1 h-1, whereas the rate of dissociation of the untransformed receptor increases from 0.04 h-1 to 1.1 h-1 in the molybdate-containing buffer. All these effects appear dependent on the concentration of molybdate but the dissociation of the transformed receptor (0.01 h-1) is unaffected. The enthalpy for the association, delta H not equal to, increases at least twofold whereas the entropy, both for the association (delta S not equal to = -25 to +104 J K-1 mol-1) and for the equilibrium (delta S degrees = -100 to +38 J K-1 mol-1), is markedly influenced by the presence of molybdate. Taken all together these data suggest that molybdate interacts with the receptor molecule turning it into a form that displays low affinity for steroid, in addition to the well-documented incapacity to transform itself. This fact leads us to think that both the binding and the transformation are the expression of conformational modifications involving molybdate-sensitive groups.
Asunto(s)
Molibdeno/farmacología , Receptores de Glucocorticoides/efectos de los fármacos , Receptores de Esteroides/efectos de los fármacos , Timo/metabolismo , Animales , Bovinos , Citosol/metabolismo , Dexametasona/metabolismo , Técnicas In Vitro , Cinética , Modelos Biológicos , Receptores de Glucocorticoides/metabolismo , TermodinámicaRESUMEN
The authors estimated E, E.A.C., and E-active rosette in 13 chronic active hepatitis (C.A.H.), 5 chronic persistent hepatitis and 4 cirrhosis. The results showed a significant decrease of the concentration of peripheral blood T-lymphocytes in patients with C.A.H., C.P.H., cirrhosis. E.A.C. rosette forming cells were not significantly different from the control population in all groups studied. E-active rosette were decrease in patients with C.A.H. and C.P.H. and were increased after stimulation by P.P.D. (5 U.V.I.) in C.A.H. and C.P.H. and in control group studied.
Asunto(s)
Hepatitis/inmunología , Inmunidad Celular , Linfocitos/inmunología , Humanos , Cirrosis Hepática/inmunología , Formación de Roseta , Tuberculina/farmacologíaRESUMEN
The AA., present a case of "agnogenic myeloid metaplasia" (MMA). They describe the principal findings that have led to this diagnosis: Fe59-erythrocynesis, radiologic study of skeleton and cytogenetic study. Fe59-erythrocynesis have shown an hypergetting of spleen and liver and an hypogetting of skeleton. The radiography of skeleton and particularly of humerus, femur and pelvis have shown a diffuse osteosclerosis with rare osteolytic areas. The cytogenetic study have shown an alteration with C-group trisomy.