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首页> 外文期刊>BMC Endocrine Disorders >Wasting syndrome with deep bradycardia as presenting manifestation of long-standing severe male hypogonadotropic hypogonadism: a case series
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Wasting syndrome with deep bradycardia as presenting manifestation of long-standing severe male hypogonadotropic hypogonadism: a case series

机译:患有深层心动过缓的消瘦综合征表现为长期严重的男性性腺功能减退性性腺机能减退的病例系列

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Background Physiological functioning of the testes is important for cardiac health besides for virilisation, physical strength, behavior and reproduction; moreover, hypogonadism has been demonstrated as a significant risk marker of increased all-cause and cardiovascular mortality. Cases presentation We reported two cases of long-standing hypogonadotropic hypogonadism presenting with wasting, bradycardia and heart failure. The two patients were admitted to emergency department for deep weakness, unresponsive anemia and severe bradycardia, requiring in one case the implanting of a monocameral pace-maker for treatment of heart failure. No previous cardiologic disorders were known and cardiac ischemia was ruled out in both patients. The first patient presented congenital hypogonadotropic hypogonadism combined with mild central hypothyroidism and growth hormone deficiency occurred in the peripubertal age, while the second one was diagnosed with isolated adult-onset severe central hypogonadism. Testosterone deficiency was the main feature in both patients as physical examination revealed clinical stigmata of hypogonadism and testosterone replacement induced a dramatic improvement of general condition. Genetic analysis of genes involved in hypogonadotropic hypogonadism failed to identify alterations. Conclusion Long-standing hypogonadism in males can be associated with life threatening body alterations including severe bradycardia and heart failure.
机译:背景睾丸的生理功能除了对男性化,体力,行为和繁殖外,对心脏健康也很重要。此外,性腺功能低下已被证明是增加全因和心血管疾病死亡率的重要危险标志。病例报告我们报告了两例长期性低促性腺功能减退症,伴有消瘦,心动过缓和心力衰竭。两名患者因严重虚弱,无反应性贫血和严重心动过缓入急诊室,其中一例需要植入单前房起搏器以治疗心力衰竭。既往无心脏病史,并且均排除了心脏缺血。第一例患者出现先天性性腺功能低下性腺功能减退症,并伴有轻度中枢性甲状腺功能减退症,并在青春期左右出现生长激素缺乏症,而第二例患者被诊断出患有成人发作的严重中枢性性腺功能低下。睾丸激素缺乏症是这两名患者的主要特征,因为体格检查发现性腺机能减退的临床污名和睾丸激素替代引起了总体状况的显着改善。对性腺功能减退性腺功能减退症相关基因的遗传分析未能发现改变。结论男性长期性腺功能减退可能与危及生命的身体改变有关,包括严重的心动过缓和心力衰竭。

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