RESUMEN
The determinations were carried out in 25 patients with thrombotic encephalomalacia. The changes in 24-hour urine uropepsin activity were studied after administration of metopirone and dexamethasone. The uropepsin activity in a sample of 24-hour urine was determined by the method of West, Ellis and Scott. A statistically significant fall of uropepsin activity was observed in 24-hour urine after metopirone, with a rise in the activity of this enzyme after dexamethasone administration.
Asunto(s)
Encefalomalacia/orina , Endopeptidasas/orina , Sistema Hipotálamo-Hipofisario/fisiopatología , Sistema Hipófiso-Suprarrenal/fisiopatología , Anciano , Encefalomalacia/fisiopatología , Humanos , Persona de Mediana EdadRESUMEN
The investigations were carried out in 30 control cases, in 20 patients with acute transient cerebral circulatory failure, 20 patients with intracerebral haemorrhage, and 22 patients with thrombotic encephalomalacia. The determinations of uropepsin activity were done on the 1st, 3rd, 7th and 14th days of the disease. Uropepsin activity in 24-hour urine was determined by the method of West, Ellis and Scott. In the group of patients with acute transient cerebral circulatory failure abnormalities in urinary uropepsin activity were not significant. The mean activity of uropepsin in 24-hour urine in patients with intracerebral haemorrhage and brain infarction on the 1st day of the disease was significantly higher than in controls. The rise in the activity of this enzyme in patients with encephalomalacia was lower in relation to patients with intracerebral haemorrhages. During improvement of the condition of patients with brain stroke the uropepsin activity in 24-hour urine decreased.
Asunto(s)
Trastornos Cerebrovasculares/orina , Endopeptidasas/orina , Enfermedad Aguda , Adulto , Anciano , Encéfalo/enzimología , Hemorragia Cerebral/orina , Encefalomalacia/orina , Femenino , Humanos , Embolia y Trombosis Intracraneal/orina , Masculino , Persona de Mediana EdadRESUMEN
To determine if ruminal hydrogen sulfide, urine thiosulfate, or blood sulfhemoglobin could be used as diagnostic indicators for sulfur-induced polioencephalomalacia, 16 steers (8 cannulated, 368 ± 12 kg; 8 unmodified, 388 ± 10 kg; mean ± standard error) were fed 1 of 2 dietary treatments. Diets consisted of a low sulfate (0.24% S; control) wheat midd-based pellet or the control pellet with sodium sulfate added to achieve a high-sulfate (0.68% S) pellet. As designed, intake did not differ (P = 0.80) between treatments. At 8 hr postfeeding, ruminal hydrogen sulfide was not affected by cannulation (P = 0.35) but was greater (P < 0.01) in high S (6,005 ± 475 mg/l) than control (1,639 ± 472 mg/l) steers. Time of day of sampling affected (P = 0.01) ruminal hydrogen sulfide, with peak concentrations occurring 4-12 hr after feeding. Urine was collected prefeeding (AM) and 7-9 hr postfeeding (PM). Urine thiosulfate concentrations of high S steers sampled in the PM were greater (P > 0.01) than in the AM. However, there was no difference due to time of sampling for control. In both the AM and PM, urine thiosulfate concentrations of high S were greater (P > 0.01) than control. Although hydrogen sulfide and thiosulfate were elevated by increased dietary S intake, a concentration at which polioencephalomalacia is likely to occur could not be determined. Sampling urine for thiosulfate or rumen gas for hydrogen sulfide of nonsymptomatic pen mates 4-8 hr after feeding may be useful to assess sulfur exposure and differentiate between causes of polioencephalomalacia.
Asunto(s)
Enfermedades de los Bovinos/metabolismo , Encefalomalacia/veterinaria , Sulfuro de Hidrógeno/metabolismo , Rumen/metabolismo , Sulfatos/metabolismo , Sulfatos/toxicidad , Sulfahemoglobina/análisis , Tiosulfatos/orina , Animales , Bovinos , Enfermedades de los Bovinos/inducido químicamente , Enfermedades de los Bovinos/diagnóstico , Enfermedades de los Bovinos/orina , Encefalomalacia/diagnóstico , Encefalomalacia/metabolismo , Encefalomalacia/orina , Concentración de Iones de Hidrógeno , Masculino , Distribución Aleatoria , Sulfatos/administración & dosificaciónAsunto(s)
Tronco Encefálico , Encefalomalacia/orina , Fosfotransferasas/antagonistas & inhibidores , Diagnóstico Diferencial , Errores Diagnósticos , Encefalomalacia/genética , Reacciones Falso Negativas , Reacciones Falso Positivas , Heterocigoto , Homocigoto , Humanos , Discapacidad Intelectual/genética , Discapacidad Intelectual/orina , Fosfatos , Trastornos Psicomotores/genética , Trastornos Psicomotores/orina , Tiamina , Tiamina PirofosfatoAsunto(s)
Tronco Encefálico/enzimología , Encefalomalacia/orina , Tronco Encefálico/metabolismo , Encefalomalacia/enzimología , Encefalomalacia/genética , Encefalomalacia/metabolismo , Heterocigoto , Humanos , Discapacidad Intelectual/genética , Discapacidad Intelectual/metabolismo , Discapacidad Intelectual/orina , Fosfotransferasas/metabolismo , Trastornos Psicomotores/genética , Trastornos Psicomotores/metabolismo , Trastornos Psicomotores/orina , Síndrome/genética , Síndrome/metabolismo , Síndrome/orina , Tiamina Pirofosfato/metabolismoAsunto(s)
Trastornos Cerebrovasculares/complicaciones , Aminoacidurias Renales/diagnóstico , Anciano , Aminoácidos/orina , Aminobutiratos/orina , Hemorragia Cerebral/complicaciones , Trastornos Cerebrovasculares/orina , Encefalomalacia/orina , Humanos , Persona de Mediana Edad , Nitrógeno/orina , Factores de TiempoRESUMEN
The large family of a 21-year-old man who died of Leigh disease was investigated for evidence of neurological abnormalities and presence of the adenosine triphosphate-thiamine diphosphate phosphoryltransferase inhibitor factor. Of 217 persons (seven generations) included in the pedigree, 68 were examined neurologically and biochemically. Fourteen (20%), 5 of whom had abnormal neurological findings, were found to excrete the inhibitor factor. Clinical manifestations varied from severe neurological affliction to subtle deficits. A chronic relapsing course was frequently encountered, with exacerbations occurring in association with apparent metabolic stress. Parental consanguinity was identified in the propositus as well as in other family members with neurological abnormalities. Males and females were affected, and no vertical transmission of the trait was found. These multigenerational data suggest that Leigh disease in adults is inherited in an autosomal recessive manner and has variable degrees of expression with a wide spectrum of neurological manifestations.