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Pathology of Familial Amyloidotic Polyneuropathy Occurring in Kumamoto

  • Kiyoshi Takahashi
  • Shukuro Araki
  • Yoshihiro Kimura
  • Shigehiro Yi
Chapter

Abstract

Familial amyloidotic polyneuropathy (FAP) is a heredofamilial amyloidosis transmitted by autosomal dominant trait, occurring in middle-aged adults, and characterized clinically by sensory dominant polyneuropathy starting from the lower limbs, autonomic dysfunction, cardiac arrhythmias, cardiac conduction disturbances and emaciation. Since its description by Andrade (1952) in Portugal, this disorder was reported in Sweden, United States of America, Finland, Japan or some other countries. In Japan, it is known that there are two major groups of families of FAP in Kumamoto and Nagano prefectures. Recent biochemical studies have elucidated that amyloid precursor protein of FAP is abnormal prealbumin and that in its primary structure valine located at the 30th portion of 127 amino acid sequence of normal prealbumin is replaced by methionine (Tawara et al. 1983). Furthermore, a couple of new methods for the diagnosis of FAP, such as radioimmunoassay method or DNA diagnostic method, have been developed and analysis of the disorder in the gene level is also in progress more recently. As for the families of FAP in the Kumamoto prefecture; however, there is no extensive pathologic study except for two autopsy cases reported by Shirabe et al. (1973).

Keywords

Amyloid Deposition Sural Nerve Autopsy Case Small Blood Vessel Myelinated Nerve Fiber 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Kiyoshi Takahashi
    • 1
  • Shukuro Araki
    • 1
    • 2
  • Yoshihiro Kimura
    • 1
    • 2
  • Shigehiro Yi
    • 2
  1. 1.Second Department of PathologyKumamoto University Medical SchoolKumamotoJapan
  2. 2.First Department of Internal MedicineKumamoto University Medical SchoolKumamotoJapan

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