Loh K C, Duh Q Y, Shoback D, Gee L, Siperstein A, Clark O H
Department of Medicine, University of California at San Francisco, USA.
Clin Endocrinol (Oxf). 1998 Apr;48(4):435-43. doi: 10.1046/j.1365-2265.1998.00329.x.
Primary hyperparathyroidism (PHPT) is an uncommonly diagnosed condition among adolescents and young adults. We review the clinical characteristics of these patients based on our institutional experience.
Patients aged 12-28 years treated for PHPT at our institution from 1990 to 1996 were evaluated by a review of medical records and current follow-up data. This consisted of 22 patients (8M:14F), constituting approximately 3% of all patients operated for PHPT during this period.
Serum and urinary calcium concentrations, renal function, and serum intact parathyroid hormone (IPTH) levels were measured in all patients. After biochemical confirmation of diagnosis, the patients completed a questionnaire to evaluate the presence of symptoms and/or conditions associated with PHPT. All the patients underwent parathyroidectomy and their tumour characteristics were evaluated. Surgical outcome was determined by measurements of serum calcium and IPTH levels postoperatively and during long-term follow-up.
A third of the patients were diagnosed by routine serum chemistry whereas two-thirds presented with symptoms or conditions associated with hypercalcaemia. Non-specific complaints such as fatigue or exhaustion, and weakness or lethargy constitute the most common findings on questionnaire review. A family history of PHPT was present in only 2 patients. The preoperative peak serum calcium levels ranged from 2.67 to 4.19 mmol/l (norm: 2.10-2.54 mmol/l), with a median of 3.07 mmol/l. Surgical pathologies revealed 59% solitary adenoma, 27% hyperplasia, 9% multiple adenomas and 5% carcinoma. Comparison between the adolescents (aged 12-18 years) and young adults (aged 19-28 years) revealed no differences in the clinical, pathological or laboratory profiles, except for a male predominance in adolescent patients. Fifteen patients had resection of one or more adenomas while 7 underwent subtotal parathyroidectomy. Six patients (27%) were reoperated cases, all received primary treatment elsewhere. All patients with benign PHPT were cured surgically, with a median follow-up of 47 months (range 3-77 months). One reoperated patient developed permanent hypocalcaemia. One patient with carcinoma underwent several operations for recurrence; he is now eucalcaemic despite persistent disease at 80 months from diagnosis.
We found a high incidence of multiglandular disease and relatively non-specific symptomatology in our adolescent and young adult patients with primary hyperparathyroidism. In view of the heterogeneous clinical expression noted in young patients, one should consider primary hyperparathyroidism in the differential diagnosis of unexplained non-specific complaints, and perform serum calcium estimations more readily in these subjects. Our experience suggests that primary hyperparathyroidism can be a serious disease with significant morbidity if left untreated, whereas parathyroidectomy provides successful results.
原发性甲状旁腺功能亢进症（PHPT）在青少年和青年中是一种诊断不常见的疾病。我们根据本机构的经验回顾这些患者的临床特征。
对1990年至1996年在本机构接受PHPT治疗的12至28岁患者进行病历回顾和当前随访数据评估。这包括22例患者（8例男性：14例女性），约占该时期所有接受PHPT手术患者的3%。
对所有患者测量血清和尿钙浓度、肾功能及血清完整甲状旁腺激素（IPTH）水平。在生化确诊后，患者完成一份问卷以评估与PHPT相关的症状和/或情况的存在。所有患者均接受甲状旁腺切除术，并评估其肿瘤特征。手术结果通过术后及长期随访时测量血清钙和IPTH水平来确定。
三分之一的患者通过常规血清化学检查确诊，而三分之二的患者表现出与高钙血症相关的症状或情况。问卷回顾中最常见的发现是非特异性主诉，如疲劳或倦怠、虚弱或无精打采。仅2例患者有PHPT家族史。术前血清钙峰值水平在2.67至4.19 mmol/L之间（正常范围：2.10 - 2.54 mmol/L），中位数为3.07 mmol/L。手术病理显示59%为单发腺瘤，27%为增生，9%为多发腺瘤，5%为癌。青少年（12 - 18岁）和青年（19 - 28岁）之间的比较显示，除青少年患者中男性占优势外，临床、病理或实验室特征无差异。15例患者切除了一个或多个腺瘤，7例接受了甲状旁腺次全切除术。6例患者（27%）为再次手术病例，均在其他地方接受过初次治疗。所有良性PHPT患者手术治愈，中位随访时间为47个月（范围3 - 77个月）。1例再次手术患者发生永久性低钙血症。1例癌患者因复发接受了多次手术；自诊断后80个月，尽管疾病持续存在，但他目前血钙正常。
我们发现青少年和青年原发性甲状旁腺功能亢进症患者中多腺体疾病的发生率较高，且症状相对不具特异性。鉴于年轻患者中观察到的临床表型异质性，在不明原因的非特异性主诉的鉴别诊断中应考虑原发性甲状旁腺功能亢进症，并更易于对这些受试者进行血清钙估计。我们的经验表明，原发性甲状旁腺功能亢进症如果不治疗可能是一种严重疾病，具有显著的发病率，但甲状旁腺切除术可取得成功结果。
