Bodemer C, Blétry O, Guillevin L, Dumas P, Brunet P, Godeau P
Service de Médecine Interne, Hôpital de la Pitié, Paris.
Ann Med Interne (Paris). 1987;138(8):625-30.
Three cases of primary amyloidosis and 2 cases of familial amyloidosis complicated by asympathicotonic orthostatic hypotension are reported. Blood pressure measurements on a tilting table, plasma renin activity and plasma aldosterone or catecholamine concentrations enabled localisation of the lesion of the baroreceptor reflex in some cases. When the pre- and post-synaptic efferent sympathetic pathway was intact, treatment associating Tyramine and Tranylcypromine may provide these bedridden patients some autonomy of movement. When this pathway is affected by the disease the association of Indomethacin, Dihydroergotamine and 9-alpha-fluorohydrocortisone may be tried.
报告了3例原发性淀粉样变性和2例伴有失交感神经张力性直立性低血压的家族性淀粉样变性病例。在倾斜台上测量血压、血浆肾素活性以及血浆醛固酮或儿茶酚胺浓度,在某些情况下可确定压力感受器反射病变的部位。当突触前和突触后传出交感神经通路完整时,联合使用酪胺和反苯环丙胺的治疗可能会让这些卧床患者获得一定的行动自主性。当该通路受疾病影响时,可尝试使用吲哚美辛、双氢麦角胺和9-α-氟氢化可的松联合治疗。