Neuropatía autonómica en la drepanocitosis y su relación con la amiloidosis sistémica

we studied autonomic function in 25 patients with sickle cell disease through the analysis of continuous blood pressure during valsalva maneuver, and changes in heart rate and blood pressure in the standing position in order to identify the presence of dysautonomy in them. the existence of systemic amyloidosis had been previously determined by fine needle biopsy of abdominal fat to determine whether its presence is related to such neurological abnormality. amyloidosis was present in 60 % of the patients and the predominant autonomic nervous system involvement was the fall in systolic blood pressure during orthostatism, as well as a vs-iia/iib index greater than 60 %, which is an indicator of sympathetic dysfunction. it was concluded that the presence of amyloidosis was not significantly related to the vegetative neurological dysfunction found.