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低促性腺激素性性腺功能减退合并嗅觉缺失(卡尔曼综合征)

[Hypogonadotropic hypogonadism combined with anosmia (Kallmann's syndrome)].

作者信息

Kozlov G I

出版信息

Probl Endokrinol (Mosk). 1987 May-Jun;33(3):17-20.

PMID:2889206
Abstract

Diagnosis in Kallmann's syndrome is often erroneous and results in the administration of inadequate therapy. The aim of the study was to define characteristic features of a course of disease and to administer adequate therapy. It has been established that cases of Kallmann's syndrome are rather frequent. Patients with hypogonadism should be examined with respect to anosmia because such a combination suggests the diagnosis of Kallmann's syndrome. The selection of a therapeutic method should be strictly individual bearing in mind a degree of hypoplasia and the presence of cryptorchism. In the combination of Kallmann's syndrome with cryptorchism CG therapy results in the elimination of cryptorchism without surgery in most patients. Chorionic gonadotropin should be administered in combination with androgens in marked testicular hypoplasia in any period at the reproductive age; later on it can be only chorionic gonadotropin. Correct diagnosis and adequate therapy lead to sex and social rehabilitation of patients with Kallmann's syndrome decreasing the number of sterile men.

摘要

卡尔曼综合征的诊断常常有误,导致治疗方法不当。本研究的目的是明确疾病进程的特征并实施恰当治疗。现已确定卡尔曼综合征病例相当常见。对于性腺功能减退患者,应检查是否存在嗅觉缺失,因为这种组合提示卡尔曼综合征的诊断。治疗方法的选择应严格个体化,要考虑发育不全的程度和隐睾的存在情况。在卡尔曼综合征合并隐睾的情况下,绒毛膜促性腺激素(CG)治疗可使大多数患者无需手术即可消除隐睾。在生殖年龄的任何时期,对于明显睾丸发育不全的患者,绒毛膜促性腺激素应与雄激素联合使用;之后则可仅使用绒毛膜促性腺激素。正确的诊断和恰当的治疗可使卡尔曼综合征患者实现性和社会康复,减少不育男性的数量。

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