Table 5.

Recommendations for management of children and adolescents with HeFH.

HeFH must be diagnosed as early as possible, so as to “gain decades of life” A late diagnosis of FH leads to a considerable reduction in the duration and in the quality of life Genetic diagnosis of FH is important for awareness of the early start of the atherosclerotic process, in order to obtain a greater adherence to the therapy and as an important knowledge for future offsprings. Positive family history for premature CHD is a very important risk factor, but it fades out if a prompt and adequate treatment is started. Analysing family history for CHD including second degree relatives may be a good suggestion. Clinical signs and symptoms of HeFH are not common in paediatric ages, except for Achilles tendon pain. Nutritional and lifestyle treatment must be started in the earliest stages of life and must be well established before puberty Smoking habit must be strictly discouraged In case of HeFH, statin therapy is available from 8 years of age. For patients with HoFH, statin therapy must be started as early as possible. Statin therapy lasts lifelong, therefore it is important to stress its safety, both for clinical health and for therapy adherence. Therapy should be started as early in girl as in boys, considering that statin therapy must be discontinued in case of pregnancy and/or lactation. If therapeutical target is not reached, adding a second pharmacological treatment might be necessary.