Castañeyra-Perdomo Agustín, Castañeyra-Ruiz Leandro, González-Marrero Ibrahim, Castañeyra-Ruiz Agustin, Gonzalez-Toledo Juan M, Castañeyra-Ruiz Maria, Carmona-Calero Emilia M
Departamento de Anatomia, Facultad de Medicina, Universidad de La Laguna, La Laguna, Tenerife, Islas Canarias, Spain; Instituto dey Ciencias de Puerto del Rosario, Puerto del Rosario, Fuerteventura, Islas Canarias, Spain.
Departamento de Anatomia, Facultad de Medicina, Universidad de La Laguna, La Laguna, Tenerife, Islas Canarias, Spain; Departamento de Farmacologiıa, Facultad de Medicina, Universidad de La Laguna, La Laguna, Tenerife, Islas Canarias, Spain.
Med Hypotheses. 2014 Jan;82(1):74-6. doi: 10.1016/j.mehy.2013.11.011. Epub 2013 Nov 16.
Kallmann syndrome (KS) is a genetic disorder which combines hypogonadotropic hypogonadism and anosmia. Hypogonadism is characterized by the absence or reduced levels of gonadotropin-releasing hormone and anosmia due to olfactory bulb aplasia. KS treatment usually begins just before puberty, but brain sexual maturation occurs long before puberty normally at perinatal age. As brain cells implicated in the development of the olfactory and reproductive system have a rostral and a caudal origin, and the rostral origin is affected by aplasia in KS and the caudal origin does not seem to be affected, the early treatment of KS, as proposed in this paper, is to attain brain sexual maturation at the most appropriate age possible to prevent the eunuchoid behavior and appearance observed in KS.
卡尔曼综合征(KS)是一种遗传性疾病,其特征为低促性腺激素性性腺功能减退和嗅觉缺失。性腺功能减退的特点是促性腺激素释放激素缺乏或水平降低,而嗅觉缺失则是由于嗅球发育不全所致。KS的治疗通常在青春期前开始,但大脑性成熟通常在围产期就已发生,远远早于青春期。由于参与嗅觉和生殖系统发育的脑细胞分别起源于头侧和尾侧,且在KS中头侧起源受到发育不全的影响,而尾侧起源似乎未受影响,因此本文提出,KS的早期治疗是在尽可能合适的年龄实现大脑性成熟,以防止出现KS中所见的类宦官行为和外貌。