Role of nitric oxide in the peripheral vessels of patients with familial amyloidotic polyneuropathy (FAP) type I.

In order to establish the generation of endothelial derived relaxating factor (EDRF) in patients with familial amyloidotic polyneuropathy (FAP) type I, the muscle temperature of the lower limb was measured with a deep portion thermometer. The temperature of the gastrocunemius muscle in FAP patients was significantly decreased compared with control subjects. In order to investigate the generation of EDRF in FAP patients, we administered NG-monomethyl-L-arginine (L-NMMA), specific inhibitor of nitric oxide, from the brachial artery and measured the changes in blood flow. Although 61.7 +/- 18.2% of the blood flow was transiently decreased after administration of L-NMMA in control subjects, FAP patients showed poor responses. In contrast, in the same way as in control subjects, significant vasodilatation was seen in FAP patients after administration of L-arginine. The urinary secreted NO2-/NO3- levels per day, which reflect the synthesis of nitric oxide in the systemic circulation, was a great deal lower in FAP patients than in control subjects. These results suggest that, although peripheral vessels can be dilated when a large amount of the substrate for NO synthase, L-arginine, is supplied, production of nitric oxide may be suppressed, and, as the result of this phenomenon, blood flow is decreased in the peripheral tissues of FAP patients in a static state.