Department of Pediatric and Adolescent Endocrinology, University Children's Hospital of Krakow, Poland.
Department of Endocrinological Surgery, Third Chair of General Surgery, Medical Faculty, Jagiellonian University Medical College, Krakow, Poland.
Pediatr Endocrinol Diabetes Metab. 2020;26(4):220-224. doi: 10.5114/pedm.2020.100805.
Primary hyperparathyroidism (PHP) in children is a rare condition and has a very dynamic course with nonspecific symptoms, what complicates the diagnosis and delays PHP treatment.
A 15-year-old boy was admitted to the Orthopedic Ward with the diagnosis of juvenile bilateral slipped capital femoral epiphysis and valgus deformities. Gait disturbances, limb pains and valgus knee deformities, polyuria, polydipsia and weight loss, have been increasing for 8 months. Despite the hypercalcemia found in laboratory tests and bone destruction demonstrated in computed tomography of the hips, orthopedic correction was performed. In histopathological examination -brown bone tumors. The PTH concentration was determined (PTH - 589.1 pg/ml; (N: 10-60) and the child was referred to the Department of Pediatric Endocrinology, where severe hypercalcemia (Ca - 4.07 mmol/l, N: 2.2-2.84) and hypophosphatemia (P - 0.68 mmol/l; N: 0.95-1.75) and adenoma of the left lower parathyroid gland was diagnosed. Forced diuresis, loop diuretics and pamidronic acid were used to obtain normocalcemia. The complications of hypercalcemia were excluded; diagnostic management excluding multiple endocrine neoplasia type 1 and 2a (MEN 1 and MEN 2A) syndrome were performed. The child was referred to the Department of Endocrinological Surgery, where the adenoma of the left inferior parathyroid gland was resected.
儿童原发性甲状旁腺功能亢进症（PHP）是一种罕见疾病，其病程非常活跃，症状不典型，这使得诊断变得复杂，并导致 PHP 治疗的延误。
一名 15 岁男孩因双侧青少年股骨头骨骺滑脱伴内翻畸形被收入矫形病房。8 个月来，步态障碍、肢体疼痛和膝内翻畸形、多尿、多饮和体重减轻的情况不断加重。尽管实验室检查发现血钙升高，髋关节计算机断层扫描显示骨破坏，但仍进行了矫形矫正。组织病理学检查显示“棕色骨肿瘤”。测定甲状旁腺激素浓度（PTH-589.1 pg/ml；（N：10-60），并将患儿转至儿科内分泌科，在那里发现严重高钙血症（Ca-4.07 mmol/l，N：2.2-2.84）和低磷血症（P-0.68 mmol/l；N：0.95-1.75），并诊断为左侧下甲状旁腺腺瘤。使用强力利尿剂、袢利尿剂和帕米膦酸以获得正常血钙。排除了高钙血症的并发症；进行了排除多发性内分泌肿瘤 1 型和 2a 型（MEN 1 和 MEN 2A）综合征的诊断性治疗。患儿被转至内分泌外科，在那里切除了左侧下甲状旁腺腺瘤。
1）PHP 患者应根据临床症状诊断疾病。对于有特定症状的患者，有必要确定血清 Ca 水平，特别是在手术前。2）在每个 PHP 病例中，应测定血 PTH、Ca 和 P，并检测 MEN 1 和 MEN 2A 综合征。3）甲状旁腺功能亢进症患者需要治疗高钙血症的多器官并发症。4）在甲状旁腺腺瘤手术后，需要进行长期的内分泌随访。
