Bjerle P, Ek B, Linderholm H, Steen L
Department of Clinical Physiology, University of Umeå, Sweden.
Clin Physiol. 1993 Jan;13(1):57-69. doi: 10.1111/j.1475-097x.1993.tb00317.x.
Sixteen patients with familial amyloidosis and polyneuropathy (FAP) and 14 health subjects underwent oesophageal manometry. Six of the patients had a severe oesophageal dysmotility with almost completely abolished propulsive pressure waves on swallowing in the lower 2/3 of the oesophagus. Ten patients had moderate dysfunction with reduced propulsive pressure wave amplitudes. Neostigmine increased the pressure wave amplitudes in healthy subjects but less so in the FAP patients. Scopolamine (Scopyl)-terbutaline (Bricanyl) almost abolished the propulsive pressure waves in healthy subjects and all patients in the lower 2/3 of the esophagus. Oesophageal distensibility, tested by inflating a rubber balloon in the oesophagus, was similar in FAP patients and healthy subjects. Thus, it is unlikely that amyloid deposits in the mucosal wall increased the oesophageal stiffness. An autonomic, predominantly vagal, denervation probably explains the disturbed function.
16例家族性淀粉样变性和多神经病（FAP）患者及14名健康受试者接受了食管测压。其中6例患者存在严重的食管运动功能障碍，食管下2/3在吞咽时推进性压力波几乎完全消失。10例患者有中度功能障碍，推进性压力波振幅降低。新斯的明可增加健康受试者的压力波振幅，但对FAP患者的作用较小。东莨菪碱（Scopyl）-特布他林（Bricanyl）几乎可消除健康受试者及所有患者食管下2/3的推进性压力波。通过在食管内充入橡皮气球测试的食管扩张性，在FAP患者和健康受试者中相似。因此，黏膜壁中的淀粉样沉积物不太可能增加食管硬度。自主神经（主要是迷走神经）去神经支配可能解释了功能紊乱。
