Las enfermedades priónicas o encefalopatías espongiformes son una familia de raras patologías neurodegenerativas caracterizadas por periodos de incubación prolongados asociados a una lenta, irreversible e invariablemente mortal evolución. En humanos se las clasifica en esporádica, adquirida y hereditaria o genética. Realizar el diagnóstico de «enfermedad de Creutzfeldt-Jakob» es un verdadero desafío para el médico intensivista dada la variabilidad en la presentación clínica y su baja incidencia. Se presentan 2 pacientes admitidos en la UCI en los que, tras descartar varias patologías, se diagnosticó con un nivel de «probabilidad», de acuerdo a la clasificación de la OMS, enfermedad de Creutzfeldt-Jakob esporádica. Se analizan aspectos diagnósticos clínicos y analíticos de la enfermedad resaltando la utilidad de la identificación de la proteína 14-3-3 en el líquido cefalorraquídeo.

Prion diseases or spongiform encephalopathies are a family of rare neurodegenerative diseases characterized by long incubation periods associated with slow, irreversible and invariably fatal evolution. In humans, they are classified as sporadic, acquired and hereditary or genetic. Diagnosing sporadic "Creutzfeldt-Jakob Disease" (sCJD) is a real challenge for the intensive care physician, given the variability in its clinical presentation and its low incidence. The cases of two patients admitted to the Intensive Care Unit are presented. After ruling out other diseases, they were diagnosed with sCJD with a likelihood level according to the World Health Organization Classification. Clinical and laboratory diagnostic aspects of the disease were analyzed, highlighting the utility of 14-3-3 protein identification in the cerebrospinal fluid.

OBJECTIVE:Ventricular dysfunction (VD) in the context of brain death (BD) is one medical cause that may be reversed to extend the range of donors for cardiac transplant programs. The aim of this study was to identify and quantify the causes for exclusion of potential heart donors and to define risk factors for VD among the BD population.MATERIALS AND METHODS:This study of 100 heart-beating potential donors defined subjects as those younger than 50 years. We defined hemodynamic dysfunction (HD) as failure to achieve hemodynamic objectives despite the use of inotropic agents by protocol or upon diagnosing VD.RESULTS:Among 246 BD subjects were 100 potential heart donors. Of these, 75 were transformed into real donors (RD) including 13 heart RD and 62 noncardiac RD. The conversion rate of BD subjects younger than 50 years to heart RD was 17%. When we analyzed the medical reasons for exclusion of the 62 donors who were not converted to heart RD, we observed that HD was the major cause (34%). When we analyzed the causes for exclusion related to cause of death, cranial trauma predominated (52%; P = .01; relative risk 3.5; 95% confidence interval 1.4-8.5).CONCLUSION:Hemodynamic dysfunction represented the major cause for loss of heart donors; it was associated with younger patients with cranial trauma.

The adrenal of the newborn infant is morphologically and functionally similar to the fetal one, and so the study of steroid excretion at birth can provide some information about fetal adrenal activity. We have studied (GLC Mass spectrometry) the behaviour of some 16 oxidated steroids: 16α-hydroxy-dehydroepiandrostcrone (16α-OH-DHA); 16β-hydroxy-dehydroepiandrosterone (16β-OH-DHA); 16-oxo-androstenediol (16-oxo-A); 16α-estriol (16α-OH-E3) in maternal urine, amniotic fluid, fetal plasma (cord blood) and urine of newborn infants, in pregnancies with premature delivery.