Table 1. Summary of the characteristics of HGPS and WS.
HGPS | WS | |
---|---|---|
Disease prevalence | 1 in 20 million 5) | 9.0 in 1 million in Japan 87) , 1.0 to 2.7 in 1 million globally 47) |
Ethnicity | Ubiquitous 7) | Relatively prevalent in Japanese and Sardinian 47) |
Sex | Equally affected 7) | Equally affected 48) |
Lifespan | Average 14.5 years 5) | Average 55.0 years 65) , median 54.3 years 88) |
Responsible gene and frequent mutation | LMNA : c.1824C>T (90% of patients) 5) | WRN : c.3139-1G>C (70.7% of allele in Japanese patients), WRN : c.1105C>T (18.6% of allele in non-Japanese patients) 47) |
Diabetes or IGT | 15.4% of patients 6) | 67.5% of patients 67) |
Dyslipidemia | 71.4% of patients 6) | 65.0 to 85.0% of patients 52 , 67) |
Hypertension | 46.7% of patients 6) | 42.5% of patients 67) |
CVD | 100% had adventitial thickening in the carotid artery, 18.2% had the low ankle- brachial index 6) | 15% had ASO, 2.5% had AP or MI, none had cerebral artery disease 67) |
Causes of death (percentage of total) |
Heart failure (80%), head injury (9%), complications of surgery (4%), stroke (3%) 7) |
Malignancy (56%), AMI (28%), infection (14%), cerebral bleeding (2%) 65) |
Abbreviations: IGT, impaired glucose tolerance; ASO, atherosclerosis obliterans; AP, angina pectoris; MI, myocardial infarction; AMI, acute myocardial infarction.