Araya A V, Pumarino H, Larenas G, Gac A
Departamento de Medicina, Hospital Clínico, Universidad de Chile, Santiago.
Rev Med Chil. 1992 May;120(5):563-70.
We report the case of a 33-year-old woman who was operated on with the diagnosis of primary hyperparathyroidism (PHP) in 1986. She had bone disease and slight hypercalcemia. Two parathyroid glands were removed with a lack of clinical improvement. Subsequently, the serum calcium levels were normal with occasional slight increases. Depressed phosphorus values and elevated alkaline phosphatases and PTH levels were also present, associated with severe bone involvement and muscular weakness. A second cervical exploration performed in 1989 disclosed only a normal parathyroid gland, which was not removed. In 1990, a thoracic CT scan showed the presence of a 1 cm mediastinal nodule close to the great vessels. A thoracotomy was performed to remove this nodule, which proved to be a parathyroid adenoma. After surgery, the patient presented with a "hungry bone" syndrome, characterized by very low levels of calcium, phosphorus and magnesium, which required enteral and parenteral calcium and magnesium supplements, plus dihydroxyvitamin D. The association of normocalcemia and intermittent hypercalcemia with severe bone disease is very rare, as is the presence of a mediastinal adenoma. This could explain the difficulty in the diagnosis in this case.
我们报告了一例33岁女性的病例,该患者于1986年因原发性甲状旁腺功能亢进症(PHP)接受手术治疗。她患有骨病且有轻度高钙血症。切除了两个甲状旁腺,但临床症状未改善。随后,血清钙水平正常,偶尔有轻微升高。同时存在低磷值、碱性磷酸酶和甲状旁腺激素水平升高的情况,伴有严重的骨受累和肌肉无力。1989年进行的第二次颈部探查仅发现一个正常的甲状旁腺,未予切除。1990年,胸部CT扫描显示在大血管附近有一个1厘米的纵隔结节。进行了开胸手术切除该结节,结果证实是甲状旁腺腺瘤。术后,患者出现了“饥饿骨”综合征,其特征是钙、磷和镁水平极低,需要通过肠内和肠外补充钙和镁,外加二羟维生素D。血钙正常与间歇性高钙血症伴严重骨病的情况非常罕见,纵隔腺瘤的存在也是如此。这可以解释该病例诊断的困难。