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西那卡塞治疗一名因R185Q钙敏感受体突变导致甲状旁腺功能亢进的婴儿:病例报告及文献综述

Cinacalcet therapy in an infant with an R185Q calcium-sensing receptor mutation causing hyperparathyroidism: a case report and review of the literature.

作者信息

Forman Thomas E, Niemi Anna-Kaisa, Prahalad Priya, Shi Run Zhang, Nally Laura M

机构信息

Department of Anesthesiology, Perioperative, and Pain Medicine, Stanford University School of Medicine, Stanford, CA, USA.

Department of Pediatrics, Division of Neonatal and Developmental Medicine, Stanford University School of Medicine, Stanford, CA, USA.

出版信息

J Pediatr Endocrinol Metab. 2019 Mar 26;32(3):305-310. doi: 10.1515/jpem-2018-0307.

Abstract

Background Neonatal severe hyperparathyroidism (NSHPT) is commonly treated with either parathyroidectomy or pharmacologic agents with varying efficacy and numerous side effects. Reports of using cinacalcet for NSHPT have increased, however, the effective dose for pediatric patients from the onset of symptoms through infancy has not been established. Case presentation We describe the clinical course of a newborn with a de novo R185Q mutation in the calcium-sensing receptor (CASR) gene, causing NSHPT. The infant received cinacalcet from the first days of life until 1 year of age. Conclusions Cinacalcet therapy effectively controlled the patient's serum calcium, phosphorus, and parathyroid hormone (PTH) levels without side effects.

摘要

背景 新生儿重症甲状旁腺功能亢进症(NSHPT)通常采用甲状旁腺切除术或药物治疗,疗效各异且副作用众多。使用西那卡塞治疗NSHPT的报道有所增加,然而,从症状出现到婴儿期的儿科患者有效剂量尚未确定。病例报告 我们描述了一名新生儿的临床过程,该新生儿钙敏感受体(CASR)基因发生了新生的R185Q突变,导致NSHPT。该婴儿从出生第一天起至1岁接受西那卡塞治疗。结论 西那卡塞治疗有效控制了患者的血清钙、磷和甲状旁腺激素(PTH)水平,且无副作用。

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