title: Characteristic features of long-living patients with familial hypercholesterolemia in Japan.
abstract: OBJECTIVE
  To assess characteristics of long-living familial hypercholesterolemics (FHs) in comparison with younger patients.
  
  
  METHODS
  Cross-sectional study.
  
  
  METHODS
  Lipid clinics of a university hospital and 14 related medical institutions.
  
  
  METHODS
  A total of 335 heterozygous FHs including 17 patients over 70 years old. The average ages of the aged (> or = 70 years old) and the younger groups were 73.5 +/- 3.7 and 46.5 +/- 15.0 years, respectively.
  
  
  METHODS
  Medical history, serum lipids and apolipoproteins, and radiographic measurement of Achilles tendon xanthomas.
  
  
  RESULTS
  Age distribution of FHs suggests shorter life of FHs compared with the general population. The age distribution of FH females was shifted to older age compared with that of FH males (P < 0.01). No significant differences were found between the levels of serum lipids and apolipoproteins in the aged and the younger groups. The thickness of the Achilles tendon was positively correlated with the product of excess total serum cholesterol and age in the patients (P < 0.01). Progression of Achilles tendon thickening was less in females than in males. A few cases of longevity could not be explained by any of the anti-atherogenic factors including female gender, a relatively low concentration of serum total cholesterol, a high concentration of HLD-cholesterol, a non-smoking habit, and a familial predisposition for longevity.
  
  
  CONCLUSIONS
  The female gender was found to be one of the most important factors for long survival of FHs. The different progression of Achilles tendon thickening in females and males may be related to the slower development of atherosclerosis and higher survival rate of the female patients.
authors.affiliation: Second Department of Internal Medicine, School of Medicine, Chiba University, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Murano
authors.forename: M
authors.initials: M
authors.lastname: Shinomiya
authors.forename: K
authors.initials: K
authors.lastname: Shirai
authors.forename: Y
authors.initials: Y
authors.lastname: Saito
authors.forename: S
authors.initials: S
authors.lastname: Yoshida
mesh_terms: D000125:Achilles Tendon / Q000473:pathology; D000328:Adult; D000367:Age Factors; D000368:Aged; D002423:Cause of Death; D005260:Female; D006579:Heterozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000401:mortality* / Q000473:pathology; D007564:Japan / Q000453:epidemiology; D008055:Lipids / Q000097:blood; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012737:Sex Factors
chemical_list: D008055:Lipids