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Familial hypercholesterolaemia (FH) is a co-dominant monogenic disorder of lipoprotein metabolism, characterised by severely elevated levels of low-density lipoprotein cholesterol (LDL-C) from birth onwards. Treatment of FH patients with cholesterol-lowering medication is mandatory to prevent premature cardiovascular disease (CVD). As a result of a nationwide screening in the Netherlands, a large group of women with FH in the child-bearing age range has been identified. Physicians are faced with a treatment dilemma if these females present either with a wish for pregnancy or an established pregnancy, since all systemically absorbed lipid-lowering medication is contraindicated during pregnancy. Currently, no evidence-based guidelines exist on the optimal clinical approach in these patients. Animal studies have shown conflicting data on potential teratogenicity of statins. In humans, there is no strong adverse safety signal, but prospective studies are lacking. The consequences of maternal hypercholesterolaemia during pregnancy for both mother and child are not well determined, although it has been suggested that it may increase the risk of CVD in the offspring. This review describes two representative cases from clinical practice, and discusses clinical considerations for treating pregnant FH patients supplemented with what is known from the literature.

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PURPOSE: Although the importance of rational prescribing is generally accepted, the teaching of pharmacotherapy to undergraduate medical students is still unsatisfactory. Because clinical teachers are an important role model for medical students, it is of interest to know whether this extends to therapeutic decision-making. The aim of this study was to find out which factors contribute to the drug choices made by medical students and their teachers (general practitioners and clinical specialists). METHODS: Final-year medical students (n = 32), and general practitioners (n = 29), lung specialists (n = 26), orthopaedic surgeons (n = 24), and internists (n = 24) serving as medical teachers from all eight medical schools in the Netherlands participated in the study. They were asked to prescribe treatment (drug or otherwise) for uncomplicated (A) and complicated (B) written patient cases and to indicate which factors influenced their choice of treatment, using a list of factors reported in the literature to influence drug prescribing. RESULTS: Final-year medical students primarily based their drug choice on the factors 'effectiveness of the drugs' and 'examples from medical teachers'. In contrast, clinical teachers primarily based their drug choice on the factors 'clinical experience', 'effectiveness of the drugs', 'side effects of the drugs', 'standard treatment guidelines', and 'scientific literature'. CONCLUSIONS: Medical teachers would appear to base their drug choice mainly on clinical experience and drug-related factors, whereas final-year medical students base their drug choice mainly on examples provided by their medical teachers. It is essential that medical teachers clearly explain to their students how they arrive at a specific choice of medication since medical students tend to copy the therapeutic drug choices from their teachers, mainly because of a lack of experience. Presenting students with clinical therapeutic problems early during undergraduate training will not only give them a chance to gain experience in solving medical problems but will also give meaning to what they are studying as opposed to merely reproducing what they learn or copying what they are told.

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OBJECTIVE: Functional and morphological changes of the arterial wall already present in young children with heterozygous familial hypercholesterolemia (HeFH) suggest that treatment should be initiated early in life to prevent premature atherosclerotic cardiovascular disease. The purpose of this study was to assess the efficacy and particularly safety of statin therapy in children with HeFH. METHODS AND RESULTS: We performed a meta-analysis of randomized, double-blind, placebo-controlled trials evaluating statin therapy in children aged 8 to 18 years with HeFH. Six studies (n=798 children) with 12 to 104 weeks of treatment were included. Total cholesterol, LDL cholesterol, and apolipoprotein B were significantly reduced, whereas HDL cholesterol and apolipoprotein A1 were significantly increased by statin therapy. No statistically significant differences were found between statin- and placebo-treated children with respect to the occurrence of adverse events (RR 0.99; 95% CI: 0.79 to 1.25), sexual development (RR of advancing > or = 1 stage in Tanner classification 0.96; 95% CI: 0.79 to 1.17), muscle toxicity (RR of CK > or = 10 times the upper limit of normal [ULN] 1.38; 95% CI: 0.18 to 10.82), or liver toxicity (RR of > or = 3 times the ULN for ASAT 0.98; 95% CI: 0.23 to 4.26 and for ALAT 2.03; 95% CI: 0.24 to 16.95). We found a minimal difference in growth in favor of the statin group (0.33 cm; 95% CI: 0.03 cm to 0.63 cm). CONCLUSION: In addition to the fact that statin treatment is efficacious, our results support the notion that statin treatment in children with HeFH is safe. Thus, even though further studies are required to assess lifelong safety, statin treatment should be considered for all children aged 8 to 18 with HeFH.

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