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Characteristic features of long-living patients with familial hypercholesterolemia in Japan
S Murano1, M Shinomiya, K Shirai
1Second Department of Internal Medicine, School of Medicine, Chiba University, Japan.
Insights
Female gender is key for long survival in familial hypercholesterolemia (FH). Despite high cholesterol, women with FH experience slower atherosclerosis progression and live longer than men.
Area of Science:
- Cardiology
- Genetics
- Longevity Research
Background:
- Familial hypercholesterolemia (FH) is a genetic disorder characterized by high LDL cholesterol levels, increasing atherosclerosis risk.
- Long-term survival in FH patients is often compromised due to premature cardiovascular disease.
Purpose of the Study:
- To compare the characteristics of long-living familial hypercholesterolemic individuals with younger patients.
- To identify factors contributing to longevity in FH patients.
Main Methods:
- A cross-sectional study involving 335 heterozygous FH patients, including 17 individuals aged 70 and above.
- Assessment included medical history, serum lipids, apolipoproteins, and Achilles tendon xanthoma measurements.
Main Results:
- FH females exhibited an older age distribution compared to FH males.
- No significant differences in serum lipids or apolipoproteins were observed between aged and younger FH groups.
- Achilles tendon thickness correlated with cholesterol levels and age, with slower progression in females.
Conclusions:
- Female gender is a significant factor for extended survival in FH patients.
- Slower atherosclerosis development and higher survival rates in females may be linked to reduced Achilles tendon thickening progression.
Objective:
To assess characteristics of long-living familial hypercholesterolemics (FHs) in comparison with younger patients.
Design:
Cross-sectional study.
Setting:
Lipid clinics of a university hospital and 14 related medical institutions.
Patients:
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.
Measurements:
Medical history, serum lipids and apolipoproteins, and radiographic measurement of Achilles tendon xanthomas.
Main 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.