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Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism

机译:西那卡塞在MEN1甲状旁腺功能亢进症中的临床应用

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摘要

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.
机译:背景。多发性内分泌肿瘤1型(MEN1-)相关性甲状旁腺功能亢进的治疗与高发率和高发率有关,这是由于多次颈部探查所致。 Cinacalcet是许可用于继发性甲状旁腺功能亢进和甲状旁腺癌治疗的拟钙剂,可能为这些复杂患者的治疗提供医学替代方法。方法。对八名患者进行了前瞻性审核。确诊时年龄为20-38岁的三名男性和五名女性。 2例患者开始接受cinacalcet作为主要治疗,其中6例曾接受过手术。六名患者患有甲状旁腺功能亢进症的并发症:肾结石,肾功能不全和骨矿物质密度降低。对于MEN1相关性甲状旁腺功能亢进,所有患者均于西那卡塞每日30mg mg开始治疗;随后将四名患者的剂量减至30 mg od。结果。血清钙和PTH的测定值明显降低。血清钙中位数降低了0.35 / mmol / L(P = 0.012 Wilcoxon签名等级)。血清PTH水平下降了中位数,为5.05 pmol / L(P = .012)。尿钙没有变化。持续控制时间为10-35个月。西那卡塞(Cinacalcet)对六例患者耐受良好;一位经历恶心,一位经历腹泻。结论。 Cinacalcet是治疗复杂的原发性甲状旁腺功能亢进症的有效且耐受良好的药物。

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