Morbidity in Turner Syndrome
Morbidity in Turner Syndrome
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DOI：
10.1016/s0895-4356(97)00237-0
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发表时间：
1998-02-01
影响因子：
7.2
通讯作者：
Hansen, J
中科院分区：
文献类型：
--
作者：
Gravholt, CH;Juul, S;Hansen, J
特纳综合征大约每100,000名女性中有50人患病，其特征是生长迟缓、性腺发育不全和不孕。许多注意力集中在生长和促进生长的疗法上，而对该综合征的自然病程，特别是成年后的自然病程知之甚少。我们进行了这项研究，以评估特纳综合征研究中相关疾病的发病率。研究时间为1984年1月1日至1993年12月31日，研究基数为研究期间居住在丹麦的所有女性。我们使用了来自丹麦细胞遗传学中央登记处和丹麦国家患者登记处的数据来评估发病率。这项研究支持了几项早期报告发病率增加的研究，并证实了最近一项关于特纳综合征癌症的研究结果。患有特纳综合征的女性在成年后骨折、骨质疏松性骨折和儿童时期的非骨质疏松性骨折的发病率似乎有所增加。此外，糖尿病，包括NIDDM和IDDM，在Turner综合征以及缺血性心脏病、高血压和中风中的发病率显著增加。在特纳综合征中，除大肠癌外，患癌症的风险似乎并未增加。我们的数据表明，特纳综合征患者特别容易出现构成代谢综合征的异常(例如高血压、血脂异常、NIDDM、肥胖、高胰岛素血症和高尿酸血症)。目前的数据可能有助于解释特纳综合征患者寿命缩短的原因。(C)1998年爱思唯尔科学公司。
Turner syndrome afflicts approximately 50 per 100,000 females and is characterized by retarded growth, gonadal dysgenesis, and infertility. Much attention has been focused on growth and growth promoting therapies, while less is known about the natural course of the syndrome, especially in adulthood. We undertook this study to assess the incidence of diseases relevant in the study of Turner syndrome. The study period was from January 1, 1984 to December 31, 1993, and the study base was all women living in Denmark during the study period. We used data from the Danish Cytogenetic Central Register and the Danish National Registry of Patients to assess morbidity. This study supports several earlier studies reporting increased morbidity and confirms results of a recent study on cancer in Turner syndrome. Women with Turner syndrome seem to have an increased incidence of fractures, osteoporotic fractures in adulthood, and non-osteoporotic fractures in childhood. Furthermore, diabetes mellitus, both NIDDM and IDDM, was found with a markedly increased incidence in Turner syndrome, as well as ischemic heart disease, hypertension and stroke. The risk of cancer, except cancer of the large bowel, does not seem to be elevated in Turner syndrome. Our data suggest that patients with Turner syndrome are extraordinarily prone to abnormalities constituting the metabolic syndrome (e.g., hypertension, dyslipidaemia, NIDDM, obesity, hyperinsulinemia and hyperuricemia). The present data may help to explain the decreased life span found in patients with Turner syndrome. (C) 1998 Elsevier Science Inc.