Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Institute for Neurological Research Raúl Carrea, FLENI, Buenos Aires, Argentina.
Auton Neurosci. 2019 Dec;222:102590. doi: 10.1016/j.autneu.2019.102590. Epub 2019 Oct 23.
Autonomic dysfunction, an early symptom of transthyretin amyloidosis (ATTR amyloidosis), requires investigations not readily available in many clinics. Although monitoring of orthostatic hypotension (OH) will not be a substitute for more specialized tests, it can add important information about initiation of dysautonomia. The aim of this study was to investigate whether simple blood pressure (BP) monitoring may be a useful tool for evaluation of disease progression and an early sign of autonomic dysfunction.
BP and OH data were from subjects enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS). Characteristics associated with changes in BP and orthostatic difference were identified by regression analyses.
OH tended to be present relatively early in the course of disease and was more common at enrollment (11.7%) than either diarrhea (2.4%) or unintentional weight loss (3.1%). In subjects with OH at enrollment, progressive increase in systolic and diastolic orthostatic difference was observed. OH was also associated with significantly worse quality of life.
BP variability is a useful tool for assessing disease onset and severity in ATTR amyloidosis, particularly in patients with OH. Trial registration ClinicalTrials.gov: NCT00628745.
自主神经功能障碍是转甲状腺素淀粉样变性（ATTR 淀粉样变性）的早期症状，需要进行许多诊所无法进行的检查。虽然直立性低血压（OH）监测不能替代更专业的测试，但它可以提供有关自主神经功能障碍起始的重要信息。本研究旨在探讨简单的血压（BP）监测是否可作为评估疾病进展和自主神经功能障碍早期迹象的有用工具。
BP 和 OH 数据来自参与转甲状腺素淀粉样变性结局调查（THAOS）的受试者。通过回归分析确定与 BP 变化和直立性差异相关的特征。
OH 在疾病过程中相对较早出现，且在登记时（11.7%）比腹泻（2.4%）或非故意体重减轻（3.1%）更为常见。在登记时患有 OH 的受试者中，观察到收缩压和舒张压直立性差异逐渐增加。OH 也与明显较差的生活质量相关。
BP 变异性是评估 ATTR 淀粉样变性发病和严重程度的有用工具，特别是在患有 OH 的患者中。
ClinicalTrials.gov：NCT00628745。
