RESUMEN
OBJECTIVE: Botulinum toxin A inhibits presynaptic release of acetylcholine at the neuromuscular junction and has reportedly been successful in the treatment of spastic disorders. This prospective study attempted to determine whether botulinum toxin A injection resulted in clinically measurable gains for 4 mo. DESIGN: Measurements were obtained from 32 children (range, 1-18 yr; average age, 6.9 yr) with hemiplegic or quadriplegic cerebral palsy before and at 1, 3, and 4 mo after botulinum toxin A injections. Spasticity was measured using the Modified Ashworth Scale for 12 different joints. RESULTS: Results showed that spasticity as measured by Ashworth scores for elbow and wrist extension clearly declined (P < 0.02) by 1 mo after botulinum toxin A injection, and diminished spasticity continued for 3-4 mo. Caregivers reported improvement in subjectively rated management, appearance, and function. However, patient response to botulinum toxin A injection was not predictable. Age had no significant relationship to gains. CONCLUSIONS: Further research is needed on the use of botulinum toxin A to diminish spasticity and improve function.
Asunto(s)
Antidiscinéticos/uso terapéutico , Toxinas Botulínicas Tipo A/uso terapéutico , Parálisis Cerebral/tratamiento farmacológico , Adolescente , Parálisis Cerebral/fisiopatología , Niño , Preescolar , Articulación del Codo/fisiopatología , Hemiplejía , Humanos , Lactante , Espasticidad Muscular/tratamiento farmacológico , Espasticidad Muscular/fisiopatología , Estudios Prospectivos , Cuadriplejía , Rango del Movimiento Articular , Articulación de la Muñeca/fisiopatologíaRESUMEN
beta-Strands as constituents of beta-pleated sheets in protein tertiary structures often display considerable distortion from a purely extended conformation. The dislocation types are often characterized as "bulging," "twisting," and "bending." The former 2 properties have been extensively studied and classified. In this work an investigation of bent beta-structures is undertaken. The structural characteristics examined included the bending angles within and out of the principal strand plane, their distribution among various strand types such as parallel and antiparallel, the amino acid preferences at bend sites, and the usage of charged and polar residues for stabilization through interactive anchoring with other atoms of the beta-sheet within which the bent strand lies.
Asunto(s)
Estructura Secundaria de Proteína , Estructura Terciaria de Proteína , Aminoácidos/química , Estabilidad de Medicamentos , Enlace de Hidrógeno , Matemática , Estructura MolecularRESUMEN
The purpose of this investigation was to evaluate whether in healthy subjects the GH response following stimulation with releasing hormones is dependent on the spontaneous GH secretion within 24 hr prior to the stimulation test. In 18 male and 9 female healthy subjects (21-59 years) GH was measured every 15 min over 26 hr. Twenty-four hours after the beginning of blood sampling, a GH stimulation test was performed by using a combined releasing hormone test. Sleep was recorded in three consecutive nights. A positive correlation was found between the AUCs of the 24-hr GH secretion and the AUCs of GH stimulation, which could not be explained by an age effect only. This study demonstrates that subjects with comparatively high amounts of GH secreted within 24 hr also show good GH secretory responses when immediately after the 24-hr sampling period a stimulation test is undertaken. Therefore, a low GH response to stimulation cannot be explained by feedback effects of high GH amounts secreted during the 24 hr before the test or by empty pituitary GH storages.
Asunto(s)
Ritmo Circadiano/fisiología , Hormona del Crecimiento/sangre , Fases del Sueño/fisiología , Adulto , Hormona Liberadora de Corticotropina , Femenino , Hormona Liberadora de Gonadotropina , Hormona Liberadora de Hormona del Crecimiento , Humanos , Masculino , Persona de Mediana Edad , Valores de Referencia , Factores Sexuales , Hormona Liberadora de TirotropinaRESUMEN
This is a report of one of the few cases of an adult form of metachromatic leukodystrophy with almost exclusively psychiatric symptoms. This should point out the importance of a careful organic diagnostic assessment in all patients with the first manifestation of a psychiatric disease. In this case an investigation of the patient's family confirmed the heterozygote genetic origin of the disease.
Asunto(s)
Leucodistrofia Metacromática/diagnóstico , Trastornos Neurocognitivos/diagnóstico , Adulto , Cerebrósido Sulfatasa/deficiencia , Cerebrósido Sulfatasa/genética , Tamización de Portadores Genéticos , Humanos , Leucodistrofia Metacromática/genética , Leucodistrofia Metacromática/psicología , Masculino , Trastornos Neurocognitivos/genética , Trastornos Neurocognitivos/psicología , Pruebas Neuropsicológicas , Trastornos Paranoides/diagnóstico , Trastornos Paranoides/genética , Trastornos Paranoides/psicología , Tomografía Computarizada por Rayos XRESUMEN
Pharmacoendocrinological studies have shown that psychotropic drugs with different actions have different effects on anterior pituitary hormone secretion in man. Substances with different effects on the central nervous system are characterized by a different pharmacoendocrinological profile. Studies with various receptor blockers have shown varying influences on the DMI-induced growth hormone, prolactin, and ACTH/cortisol secretion. Growth hormone stimulation was shown to be mediated by alpha 2-receptors and inhibited by beta-receptors. Investigations in male and female endogenous depressive patients demonstrated a significantly blunted growth hormone response to DMI compared with age- and sex-matched healthy subjects. A comparative study in male endogenous depressive patients showed a significantly diminished growth hormone stimulation both after DMI and after growth hormone-releasing hormone compared to healthy male subjects. In further tests a simultaneous application of four releasing hormones (GHRH, CRH, GnRH, TRH) was used. These investigations showed a significantly lower GH stimulation in endogenous depressive patients compared with age- and sex-matched healthy subjects, but not in neurotic depressive or schizophrenic patients. Cortisol stimulation was similar in all groups of patients and healthy subjects. TSH stimulation was significantly lower in endogenous depressive and schizophrenic patients than in healthy subjects. Somatomedin-C concentrations were significantly elevated in endogenous depressed patients compared with healthy subjects. The blunted growth hormone response in endogenous depression could be explained by inhibitory influences such as increased somatomedin-C concentrations or a hyperactivity of central beta-adrenergic-receptors.
Asunto(s)
Trastorno Depresivo/metabolismo , Hormonas Liberadoras de Hormona Hipofisaria/farmacología , Hormonas Adenohipofisarias/sangre , Psicotrópicos/farmacología , Trastorno Depresivo/tratamiento farmacológico , Humanos , Hormonas Liberadoras de Hormona Hipofisaria/uso terapéutico , Psicotrópicos/uso terapéuticoRESUMEN
In an animal preparation of congestive heart failure in the dog, during the development of cardiac failure due to rapid right ventricular pacing we observed significant decreases in cardiac output and arterial pressure and increases in pulmonary arterial and right atrial pressure. We also observed a related increase in right atrial pressure and increases in plasma levels of atrial natriuretic peptide (ANP) and cyclic guanosine monophosphate (c-GMP). Ultrastructure changes in the atrial myoendocrine cells indicated extreme stimulation of the secretory apparatus of ANP. The response of hemodynamic, renal, and hormonal variables was investigated after incremental infusions (0.01, 0.03, 0.1, 0.3, and 0.06 microgram/kg/min) of exogenous ANP. In healthy animals ANP significantly decreased mean arterial pressure, cardiac output, stroke volume, and right atrial pressure without changing heart rate or peripheral vascular resistance. As expected, we found a striking increase in urine flow and urinary excretion of sodium, chloride, magnesium and calcium and a smaller increase in potassium excretion. ANP suppressed renin secretion, and increased renal plasma flow, glomerular filtration rate, and filtration fraction. In dogs with heart failure ANP caused a small reduction in mean arterial pressure. No effect was seen on other hemodynamic variables or plasma renin concentration. The excretory effects on the kidneys were completely absent, and smaller increases in glomerular filtration rate and filtration fraction were observed. We found no difference between healthy dogs and animals with heart failure with respect to the secretion of c-GMP during ANP infusions in relation to the plasma levels of ANP. This suggests an intracellular defect that prevents the mediation of the hormonal signal into biological action in the presence of heart failure.