Hayashi Shigeki, Oba Takaaki, Ichikawa Kanami, Nakamura Chizuko, Hara Yosuke, Kanai Toshiharu, Sato Yoshinori, Uehara Takeshi, Ito Ken-Ichi
Division of Breast and Endocrine Surgery, Department of Surgery, Shinshu University School of Medicine, Matsumoto, Japan.
Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
Gland Surg. 2022 Jul;11(7):1279-1286. doi: 10.21037/gs-22-92.
Hypercalcemic crisis caused by primary hyperparathyroidism (PHPT) in pediatric patients is very rare, and appropriate treatment approach for this condition has not been well demonstrated. Here, we report a case of PHPT-induced hypercalcemic crisis in a boy.
An 11-year-old boy visited the clinic with abdominal pain and nausea that lasted for 3 months, but the cause of his symptoms could not be identified. As these symptoms worsened after 1 month, he was referred to a nearby hospital. The boy's albumin-corrected serum calcium level was very high (14.3 mg/dL). Treatment was immediately started with the administration of normal saline, furosemide, and calcitonin to lower his serum calcium levels. Based on elevated intact-parathyroid hormone (i-PTH) (405 pg/mL) level and enlargement of the right superior parathyroid on diagnostic imaging, he was diagnosed with hypercalcemic crisis due to PHPT As his albumin-corrected serum calcium level increased to 16.5 mg/dL and he could not take almost any foods due to severe nausea, he was transferred to our hospital and treated with pamidronate. Although his albumin-corrected serum calcium level decreased to 14.0 mg/dL, his symptoms did not improve completely. Therefore, 2 days after transfer to our hospital, he underwent emergency surgery to resect the enlarged right superior parathyroid gland. Fifteen minutes after removal of the enlarged parathyroid gland, the serum intact-PTH level decreased to 41.7 pg/mL. The histopathological diagnosis of the enlarged parathyroid gland was adenoma. The boy became asymptomatic, and his albumin-corrected serum calcium level was maintained within the normal limits for 6 months post operatively. Genetic testing performed after the surgery did not detect any pathogenic mutations in the and genes, and no genetic predisposition has been identified to date.
Emergency focused parathyroidectomy prior to genetic testing might be an appropriate strategy when the pediatric patient presents with a PHPT-induced hypercalcemic crisis.
小儿原发性甲状旁腺功能亢进症（PHPT）引起的高钙血症危象非常罕见，针对这种情况的适当治疗方法尚未得到充分证实。在此，我们报告一例男孩因PHPT引发高钙血症危象的病例。
一名11岁男孩因腹痛和恶心症状持续3个月前来就诊，但症状原因不明。1个月后症状加重，遂转诊至附近医院。该男孩白蛋白校正后的血清钙水平非常高（14.3mg/dL）。立即开始给予生理盐水、呋塞米和降钙素以降低其血清钙水平。根据诊断性影像学检查中升高的完整甲状旁腺激素（i-PTH）水平（405pg/mL）以及右上甲状旁腺肿大，他被诊断为因PHPT导致的高钙血症危象。由于他白蛋白校正后的血清钙水平升至16.5mg/dL，且因严重恶心几乎无法进食，他被转至我院并接受帕米膦酸治疗。尽管他白蛋白校正后的血清钙水平降至14.0mg/dL，但症状并未完全改善。因此，转至我院2天后，他接受了紧急手术，切除肿大的右上甲状旁腺。切除肿大的甲状旁腺15分钟后，血清完整PTH水平降至41.7pg/mL。肿大甲状旁腺的组织病理学诊断为腺瘤。术后6个月，该男孩无症状，其白蛋白校正后的血清钙水平维持在正常范围内。术后进行的基因检测未在相关基因中检测到任何致病突变，迄今为止未发现遗传易感性。
当小儿患者出现PHPT引起的高钙血症危象时，在进行基因检测之前进行紧急聚焦甲状旁腺切除术可能是一种合适的策略。
