RESUMEN
The fatty acid composition of postmortem brain and liver from an adrenoleukodystrophy patient whose diet was supplemented with Lorenzo's oil (glycerol trioleate and glycerol trierucate) for 9 months was determined. The diet depressed plasma and liver saturated very long chain fatty acids (24:0 and 26:0) and increased plasma and liver erucic (22:1) and nervonic (24:1) acids. The levels of plasma linoleic (18:2 n-6), eicosopentaenoic (20:5 n-3), and docosahexaenoic (22:6 n-3) acids were also reduced, while the biochemical marker for essential fatty acid deficiency (20:3 n-9) was markedly increased in liver. However, we were unable to detect any corresponding changes in brain indicating that little erucic acid crossed the blood-brain barrier. Our findings suggest that dietary supplementation with Lorenzo's oil is of limited value in correcting the accumulation of saturated very long chain fatty acids in the brain of patients with adrenoleukodystrophy.
Asunto(s)
Adrenoleucodistrofia/tratamiento farmacológico , Adrenoleucodistrofia/metabolismo , Química Encefálica/efectos de los fármacos , Ácidos Erucicos/uso terapéutico , Ácidos Grasos/metabolismo , Ligamiento Genético , Trioleína/uso terapéutico , Cromosoma X , Adolescente , Adrenoleucodistrofia/genética , Niño , Combinación de Medicamentos , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Classic and concealed entrainment was demonstrated in a patient with spontaneous typical atrial flutter and pacing-induced atypical atrial flutter. The form of entrainment manifested depended on the site of pacing and the direction of tachycardia as determined by endocardial mapping.