RESUMEN
Primary pulmonary hypertension (PPH) and Castleman's disease (CD) are rare conditions infrequently encountered in clinical practice. In this paper, two patients diagnosed with both of these diseases are reported. The authors speculate that rather than being a chance occurrence, these conditions are linked by a common angio-proliferative mechanism. Therefore, an association between infection with the human herpesvirus-8 and the diseases of PPH and CD was sought. Evidence of human herpesvirus-8 infection was found in the lung tissue and, specifically, in the plexiform lesions from one of the patients.
Asunto(s)
Enfermedad de Castleman/virología , Infecciones por Herpesviridae/complicaciones , Herpesvirus Humano 8/aislamiento & purificación , Hipertensión Pulmonar/virología , Adulto , Cateterismo Cardíaco , Enfermedad de Castleman/patología , Femenino , Infecciones por Herpesviridae/patología , Humanos , Hipertensión Pulmonar/patología , Inmunohistoquímica , Pulmón/patología , Ganglios Linfáticos/patología , Masculino , Reacción en Cadena de la PolimerasaRESUMEN
A key factor in the genetically programmed development of the nervous system is the death of massive numbers of neurons. Therefore, genetic mechanisms governing cell survival are of fundamental importance to developmental neuroscience. We report that inner ear sensory neurons are dependent on a basic helix-loop-helix transcription factor called NeuroD for survival during differentiation. Mice lacking NeuroD protein exhibit no auditory evoked potentials, reflecting a profound deafness. DiI fiber staining, immunostaining and cell death assays reveal that the deafness is due to the failure of inner ear sensory neuron survival during development. The affected inner ear sensory neurons fail to express neurotrophin receptors, TrkB and TrkC, suggesting that the ability of NeuroD to support neuronal survival may be directly mediated through regulation of responsiveness to the neurotrophins.
