Department of Endocrinology, St. Bartholomew's Hospital, London EC1A 7BE, UK.
Int J Endocrinol. 2010;2010:906163. doi: 10.1155/2010/906163. Epub 2010 May 26.
Background. Management of multiple-endocrine neoplasia type 1- (MEN1-) associated hyperparathyroidism is associated with high recurrence rates and high surgical morbidity due to multiple neck explorations. Cinacalcet, a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism and parathyroid carcinoma, may provide a medical alternative for the management of these complex patients. Methods. A prospective audit was performed of eight patients; three males and five females, aged 20-38 at diagnosis. Two patients commenced cinacalcet as primary treatment and six had previous surgery. Six patients had complications of hyperparathyroidism: renal calculi, renal dysfunction, and reduced bone mineral density. All were commenced on cinacalcet 30 mg bd for MEN1 associated hyperparathyroidism; doses were subsequently reduced to 30 mg od in four patients. Results. Significant reductions were observed in serum calcium and PTH measurements. Serum calcium reduced by a median of 0.35 mmol/L (P = .012 Wilcoxon Signed Rank). Serum PTH levels decreased by a median of 5.05 pmol/L (P = .012). There was no change in urine calcium. Duration ranged from 10-35 months with maintenance of control. Cinacalcet was well tolerated by six patients; one experienced nausea and one experienced diarrhoea. Conclusion. Cinacalcet is an effective and well-tolerated medical treatment for the management of complex primary hyperparathyroidism.
背景。多发性内分泌腺瘤病 1 型(MEN1-)相关甲状旁腺功能亢进症的治疗因多次颈部探查而导致复发率高和手术发病率高。西那卡塞是一种钙敏感受体激动剂,获准用于治疗继发性甲状旁腺功能亢进症和甲状旁腺癌,可能为这些复杂患者的治疗提供一种医学选择。
方法。对 8 例患者(3 名男性和 5 名女性,诊断时年龄为 20-38 岁)进行了前瞻性审计。2 例患者开始使用西那卡塞作为初始治疗,6 例患者有过手术史。6 例患者有甲状旁腺功能亢进症的并发症:肾结石、肾功能不全和骨密度降低。所有患者均开始使用西那卡塞 30 mg,bid,用于治疗 MEN1 相关甲状旁腺功能亢进症;随后有 4 例患者将剂量减少至 30 mg,qd。
结果。观察到血清钙和 PTH 测量值显著降低。血清钙中位数降低 0.35 mmol/L(P =.012 Wilcoxon 符号秩检验)。血清 PTH 水平降低中位数为 5.05 pmol/L(P =.012)。尿钙无变化。治疗时间范围为 10-35 个月,均保持控制。西那卡塞在 6 例患者中耐受性良好;1 例患者出现恶心,1 例患者出现腹泻。
结论。西那卡塞是治疗复杂原发性甲状旁腺功能亢进症的有效且耐受性良好的药物治疗方法。