Table 1 Epidemiology and recurrence risk figures in HSCR
L-HSCRS-HSCR
% probands1981
Sex ratio (male:female)1.755.5
Genetic modelDominantMultifactorial or recessive
Penetrance (%) (male:female)52:4017:4
Recurrence risk to sibs* (%)
Male proband17/135/1
Female proband33/95/3

  • Relative risk  =  200.

  • *Recurrence risk is given for male/female siblings, respectively.