Familial hypercholesterolemia (FH) is characterized by elevated low-density lipoprotein cholesterol (LDL-C) levels and increased cardiovascular disease (CVD) risk. FH patients often have increased lipoprotein(a) [Lp(a)] levels, which further increase CVD risk. Novel methods for accurately calculating LDL-C have been proposed. Patients with FH were recruited by a network of Greek sites participating in the HELLAS-FH registry. LDL-C levels were calculated using the Friedewald (LDL-CF) and the Martin/Hopkins (LDL-CM/H) equations as well as after correcting LDL-CM/H for Lp(a) levels [LDL-CLp(a)corM/H]. The objective was to compare LDL-C levels and target achievement as estimated by different methods in FH patients. This analysis included 1620 patients (1423 adults and 197 children). In adults at diagnosis, LDL-CF and LDL-CM/H levels were similar [235 ± 70 mg/dL (6.1 ± 1.8 mmol/L) vs 235 ± 69 mg/dL (6.1 ± 1.8 mmol/L), respectively; P = NS], while LDL-CLp(a)corM/H levels were non-significantly lower than LDL-CF [211 ± 61 mg/dL (5.5 ± 1.6 mmol/L); P = 0.432]. In treated adults (n = 966) both LDL-CF [150 ± 71 mg/dL (3.9 ± 1.8 mmol/L)] and LDL-CM/H levels [151 ± 70 mg/dL (6.1 ± 1.8 mmol/L); P = 0.746] were similar, whereas LDL-CLp(a)corM/H levels were significantly lower than LDL-CF [121 ± 62 mg/dL (3.1 ± 1.6 mmol/L); P < 0.001]. Target achievement as per latest guidelines in treated patients using the LDL-CM/H (2.5%) and especially LDL-CLp(a)corM/H methods (10.7%) were significantly different than LDL-CF (2.9%; P < 0.001). In children, all 3 formulas resulted in similar LDL-C levels, both at diagnosis and in treated patients. However, target achievement by LDL-CF was lower compared with LDL-CM/H and LDL-CLp(a)corM/H methods (22.1 vs 24.8 vs 33.3%; P < 0.001 for both comparisons). LDL-CLp(a)corM/H results in significantly lower values and higher target achievement rate in both treated adults and children. If validated in clinical trials, LDL-CLp(a)corM/H may become the method of choice to more accurately estimate ‘true’ LDL-C levels in FH patients.

中文翻译：

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通过不同方法评估家族性高胆固醇血症患者的低密度脂蛋白胆固醇目标：来自 HELLAS-FH 登记的分析。

家族性高胆固醇血症 (FH) 的特征是低密度脂蛋白胆固醇 (LDL-C) 水平升高和心血管疾病 (CVD) 风险增加。FH 患者的脂蛋白(a) [Lp(a)] 水平通常升高，这进一步增加了 CVD 风险。已经提出了用于准确计算 LDL-C 的新方法。FH 患者由参与 HELLAS-FH 登记的希腊站点网络招募。使用 Friedewald (LDL-CF) 和 Martin/Hopkins (LDL-CM/H) 方程以及在 Lp(a) 水平校正 LDL-CM/H 后计算 LDL-C 水平 [LDL-CLp(a)科尔M/H]。目的是比较 FH 患者通过不同方法估计的 LDL-C 水平和目标实现情况。该分析包括 1620 名患者（1423 名成人和 197 名儿童）。在诊断时的成年人中，LDL-CF 和 LDL-CM/H 水平相似 [分别为 235 ± 70 mg/dL (6.1 ± 1.8 mmol/L) 和 235 ± 69 mg/dL (6.1 ± 1.8 mmol/L)；P = NS]，而 LDL-CLp(a)corM/H 水平不显着低于 LDL-CF [211 ± 61 mg/dL (5.5 ± 1.6 mmol/L)；P = 0.432]。在接受治疗的成人 (n = 966) 中，LDL-CF [150 ± 71 mg/dL (3.9 ± 1.8 mmol/L)] 和 LDL-CM/H 水平 [151 ± 70 mg/dL (6.1 ± 1.8 mmol/L) ; P = 0.746] 相似，而 LDL-CLp(a)corM/H 水平显着低于 LDL-CF [121 ± 62 mg/dL (3.1 ± 1.6 mmol/L)；P < 0.001]。根据最新指南，使用 LDL-CM/H（2.5%），尤其是 LDL-CLp(a)corM/H 方法（10.7%）的治疗患者的目标实现与 LDL-CF（2.9%；P < 0.001）显着不同）。在儿童中，所有 3 种配方奶粉在诊断时和接受治疗的患者中都产生了相似的 LDL-C 水平。然而，与 LDL-CM/H 和 LDL-CLp(a)corM/H 方法相比，LDL-CF 的目标实现率较低（22.1% 对 24.8% 对 33.3%；两项比较 P < 0.001）。在接受治疗的成人和儿童中，LDL-CLp(a)corM/H 导致显着降低的值和更高的目标实现率。如果在临床试验中得到验证，LDL-CLp(a)corM/H 可能成为更准确估计 FH 患者“真实”LDL-C 水平的首选方法。