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丹麦非手术性甲状旁腺功能减退症的流行病学:一项全国性病例发现研究。

The Epidemiology of Nonsurgical Hypoparathyroidism in Denmark: A Nationwide Case Finding Study.

作者信息

Underbjerg Line, Sikjaer Tanja, Mosekilde Leif, Rejnmark Lars

机构信息

Department of Endocrinology and Internal Medicine, Tage-Hansens Gade (THG), Aarhus University Hospital, Aarhus, Denmark.

出版信息

J Bone Miner Res. 2015 Sep;30(9):1738-44. doi: 10.1002/jbmr.2501. Epub 2015 May 31.

DOI:10.1002/jbmr.2501
PMID:25753591
Abstract

Nonsurgical hypoparathyroidism (HypoPT) is a rare disorder most often caused by mutations in different genes. It is characterized by hypocalcaemia with inappropriately low PTH levels. Knowledge about this group of patients, including their mortality and morbidity, is very sparse. The aim was to identify all patients diagnosed with nonsurgical HypoPT in Denmark and assess their mortality and risk of complications. Through registers and review of individual patient hospital charts we identified all patients diagnosed with nonsurgical HypoPT in Denmark between 1977 and 2012. We assessed their mortality and morbidity by comparing them with a group of age- and gender-matched population-based controls. We identified a total of 180 patients with nonsurgical HypoPT among whom 123 (68%) were alive at the date of follow-up (prevalence of 2.3/100,000 inhabitants). Compared with controls, mortality was not increased, but patients had a significantly increased risk of renal insufficiency (hazard ratio [HR] 6.01), cardiovascular diseases (HR 1.91), neuropsychiatric complications (HR 2.45), infections (HR 1.94), seizures (HR 10.05), cataract (HR 4.21), and fractures at the upper extremities (HR 1.93). In contrast patients had significantly reduced risk of malignant diseases (HR 0.44). In conclusion, nonsurgical HypoPT is a rare disease associated with a number of complications that should be considered when taking care of these patients.

摘要

非手术性甲状旁腺功能减退症(HypoPT)是一种罕见疾病,最常见的病因是不同基因的突变。其特征为低钙血症伴甲状旁腺激素(PTH)水平异常降低。关于这类患者的情况,包括其死亡率和发病率,了解非常有限。本研究目的是识别丹麦所有诊断为非手术性HypoPT的患者,并评估其死亡率和并发症风险。通过登记册及查阅个体患者的医院病历,我们识别出了1977年至2012年间丹麦所有诊断为非手术性HypoPT的患者。我们通过将他们与一组年龄和性别匹配的基于人群的对照进行比较,评估了他们的死亡率和发病率。我们共识别出180例非手术性HypoPT患者,其中123例(68%)在随访时仍存活(患病率为2.3/100,000居民)。与对照组相比,死亡率没有增加,但患者出现肾功能不全(风险比[HR] 6.01)、心血管疾病(HR 1.91)、神经精神并发症(HR 2.45)、感染(HR 1.94)、癫痫发作(HR 10.05)、白内障(HR 4.21)和上肢骨折(HR 1.93)的风险显著增加。相比之下,患者患恶性疾病的风险显著降低(HR 0.44)。总之,非手术性HypoPT是一种罕见疾病,与多种并发症相关,在照料这些患者时应予以考虑。

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