Endothelium-dependent vasodilatation in patients with familial amyloidotic polyneuropathy
β Scribed by Konen Obayashi; Yukio Ando; Masaaki Nakamura; Hisayasu Terazaki; Taro Yamashita; Kazuhiro Tashima; Moritaka Suga; Makoto Uchino; Masayuki Ando
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 826 KB
- Volume
- 23
- Category
- Article
- ISSN
- 0148-639X
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β¦ Synopsis
We examined endothelium-dependent vasodilatation in 15 familial amyloidotic polyneuropathy (FAP) amyloidogenic transthyretin (ATTR) Valine30Methionine (Val30Met) patients and 12 healthy volunteers. Using ultrasonography, we measured the radial artery diameters under both baseline and hyperemic conditions. Endothelium-dependent vasodilatation was expressed as a percent increase in the diameters of the radial artery after induced hyperemia. Endothelium-dependent vasodilatation tended to decrease in the patients, compared with healthy volunteers. Responses were not elicited at all in patients with disease of more than 9 years' duration. Linear negative correlation was observed between endothelium-dependent vasodilatation and disease duration (P < 0.01). Correlation between endothelium-dependent vasodilatation and degree of autonomic dysfunction was significant (P = 0.0524) and for age was close to significance (P = 0.051). These results suggest that the peripheral vasomotor dysfunction in FAP patients may predominantly depend on the amount of amyloid deposition around the vessels through the course of illness.
π SIMILAR VOLUMES
Amyloidosis is characterised by the extracellular deposition of certain different proteins in a distinctively abnormal fibrillar conformation. All types of amyloid fibril share remarkably similar structural and biophysical properties despite substantial chemical heterogeneity among their respective
Mutations in the serum protein transthyretin (TTR) cause amyloidosis involving the peripheral nerves, heart, and other organs. In Ashkenazic Jews, the only TTR variant described to date has been TTR Ile 33. We have studied DNA from another Ashkenazic Jewish kindred with familial amyloidotic polyneur