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autosomal dominant condition 
affect 1 in 500 people. 
results in high levels of LDL-cholesterol  - may cause early CVD 
FH caused by mutations in gene encodes the LDL-receptor protein.

''Clinical diagnosis'' is based on //Simon Broome// criteria:
in adults total cholesterol (TC) > 7.5 mmol/l and LDL-C > 4.9 mmol/l or children TC > 6.7 mmol/l and LDL-C > 4.0 mmol/l, plus:
for definite FH: tendon xanthoma in patients or 1st or 2nd degree relatives or DNA-based evidence of FH
for possible FH: family history of myocardial infarction below age 50 years in 2nd degree relative, below age 60 in 1st degree relative, or a family history of raised cholesterol levels

''Management''
don't use  CVD risk estimation standard tables - not accurately reflect risk 
referral to a specialist lipid clinic usually required
maximum dose of potent statins usually required
first-degree relatives have 50% chance of having disorder - offer screening. ( includes children  at age 10)
statins should be discontinued in women 3 months before conception due to the risk of congenital defects