Horton William B, Stumpf Meaghan M, Coppock Joseph D, Lancaster Luke, Dalkin Alan C, Liu Zhenqi, Chisholm Christian A, Smith Philip W, Kirk Susan E
Division of Endocrinology and Metabolism, Department of Medicine, University of Virginia Health System, Charlottesville, Virginia 22903.
Department of Pathology, University of Virginia Health System, Charlottesville, Virginia 22903.
J Endocr Soc. 2017 Jun 30;1(9):1150-1155. doi: 10.1210/js.2017-00172. eCollection 2017 Sep 1.
Gestational primary hyperparathyroidism (GPHPT) is a rare condition with fewer than 200 cases reported. We present the case of a 21-year-old woman who presented at 10 weeks' gestation with severe hypercalcemia. Laboratory investigation was consistent with primary hyperparathyroidism. Neck ultrasound did not reveal any parathyroid enlargement. Due to the persistence of severe hypercalcemia, she was treated with 4 weeks of cinacalcet therapy, which was poorly tolerated due to nausea and vomiting. At 14 weeks' gestation, she underwent neck exploration with right lower, left upper, and partial right upper parathyroid gland excision. Intra- and postoperative parathyroid hormone (PTH) and calcium levels remained elevated. After a thorough discussion of risks/benefits, the patient requested further treatment. A parathyroid sestamibi scan (PSS) revealed an ectopic adenoma in the left mediastinum. The adenoma was removed via video-assisted thorascopic parathyroidectomy with intraoperative PTH declining to nearly undetectable levels. She ultimately delivered a physically and developmentally normal infant at 37 weeks' gestation. Appropriate treatment of severe GPHPT may prevent the maternal and fetal complications of hypercalcemia. This case, in which cinacalcet therapy and PSS were used, adds to the body of literature regarding treatment of severe GPHPT.
妊娠原发性甲状旁腺功能亢进症(GPHPT)是一种罕见疾病,报告病例少于200例。我们报告一例21岁女性病例,该患者在妊娠10周时出现严重高钙血症。实验室检查结果与原发性甲状旁腺功能亢进症相符。颈部超声未发现任何甲状旁腺肿大。由于严重高钙血症持续存在,她接受了4周的西那卡塞治疗,但因恶心和呕吐耐受性差。妊娠14周时,她接受了颈部探查,切除了右下、左上和部分右上甲状旁腺。术中和术后甲状旁腺激素(PTH)及钙水平仍升高。在对风险/益处进行充分讨论后,患者要求进一步治疗。甲状旁腺 sestamibi 扫描(PSS)显示左纵隔有一个异位腺瘤。通过电视辅助胸腔镜甲状旁腺切除术切除了腺瘤,术中PTH降至几乎无法检测的水平。她最终在妊娠37周时分娩了一名身体和发育正常的婴儿。对严重GPHPT进行适当治疗可预防高钙血症的母婴并发症。本病例使用了西那卡塞治疗和PSS,为有关严重GPHPT治疗的文献增添了内容。