促性腺激素性性腺功能减退症的诊治
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摘要
促性腺激素性性腺功能减退症是临床中影响男性性腺发育,造成男性不育和男性青春期发育延迟最常见的器质性疾病。高促性腺激素性性腺功能减退症是由于睾丸组织自身病变所致,以染色体数目异常的Klinefelter综合征为代表。低促性腺激素性性腺功能减退症是由于下丘脑或垂体功能障碍引起,以特发性低促性腺性性腺功能减退为代表。本文主要分析了2005年10月至2009年5月期间吉林大学第一医院男科门诊就诊的120例促性腺激素性性腺功能减退症的男性患者的诊疗效果。最终检查发现Klinefelter综合征的42例,后天性高促性腺激素性性腺功能减退症8例,Kallmann综合征21例,特发性低促性腺激素性性腺功能减退症49例。低促性腺激素性性腺功能减退症患者,给予HCG联合HMG治疗,并辅以十一酸睾酮,促进其睾丸的发育,自身合成并分泌雄激素,以及生成精子,疗效显著,有效率达95%。而对高促性腺激素性性腺功能减退症患者,只能用雄激素终身替代治疗,对改善男性勃起功能及维持第二性征有显著疗效,对生育无改善。
Background:
Male gonadotropic hypogonadism is a pathological condition that males still have no significant signs of secondary sexual characteristics development and testicular enlargement at the age of 14 years or above two standard deviations of the average age due to malfunction of the hypothalamus or pituitary,or caused by testicular failure. The former is callled hypogonadotropic hypogonadism, or secondary hypogonadism. the latter is named hypergonadotropic hypogonadism, or primary hypogonadism
Clinically, this disease is often misdiagnosed or wrongly treated or delayed treated due to lack of sufficient knowledge of the etiology and pathogenesis, which caused irreparable damage to patients.
The main objective of this research was to analyze the clinical features and to assess treatment effect of 120 male patients with gonadotropic hypogonadism from department of andrology in the First Hospital of Jilin University during October 2005 to May 2009.
Objective:To study the clinical characteristics, diagnosis and treatment of Male gonadotropic hypogonadism
Methods:120 data of male outpatients with gonadotropic hypogonadism from department of andrology in the First Hospital of Jilin University during October 2005 to May 2009.was retrospectively analyzed,its clinical features and treatment effect was evaluated.
Results:According to serum sexual hormone level,50 cases with hypergonadotropic hypogonadism including 42 cases with Klinefelter syndrome, and 8 cases with acquired hypergonadotropic hypogonadism (3 caused by serious testicular injury,3 with a clear history of mumps,2 cases with bilateral cryptorchidism) were diagnosed.Besides,70 cases of hypogonadotropic hypogonadism including 49 patients with idiopathic hypogonadotropic hypogonadism and 21 patients with Kallmann syndrome were diagnosed. Different treatment was given according to different hormone levels,and patients with hypergonado- tropic hypogonadism was given Testosterone undecanoate 40mg,3 times daily po, or 500mg, daily per 6-8 weeks, im. And patients with hypogonadotropic hypogonadism was administered human Chorionic Gonadotropin (HCG),1500IU, and human menopausal gonadotropin (HMG),75-150IU,2 times/week, Testosterone undecanoate 40 mg twice, po, was given simultaneously. After 1-2 years'follow-up, patients with hypogonadotropic hypogonadism received a significant treatment effect, and the effective rate was over 95%. Patients with hypergonadotropic hypogonadism also improved significantly in testicular volume and penile erection.
Conclusion:Male gonadotropic hypogonadism represented by Klinefelter syndrome and idiopathic hypogonadotropic hypogonadism is the most common organic disease that impacts male gonad development, causing male infertility. Patients with hypergonadotropic hypogonadism can receive significantly improvement in sexual desire and penile erection by androgen replacement therapy for whole life. Earlier initial treatment can also improve development of sex organs and secondary sexual characteristics. It is of little significance in improvement of reproductive function.However, to patients with hypogonadotropic hypogonadism, HCG combined with HMG therapy can improve development of adolescent secondary sexual characteristics, and can restore testosterone production and spermatogenesis, with no obvious side effects. At the same time, It can increase semen density in patients with asthenozoospermia and solve fertility problems. In addition, patients with larger testicular volume can obtain better treatment effect. It has no effec to olfactory dysfunction for patients with Kallmann syndrome.
Background:
Male gonadotropic hypogonadism is a pathological condition that males still have no significant signs of secondary sexual characteristics development and testicular enlargement at the age of 14 years or above two standard deviations of the average age due to malfunction of the hypothalamus or pituitary,or caused by testicular failure. The former is callled hypogonadotropic hypogonadism, or secondary hypogonadism. the latter is named hypergonadotropic hypogonadism, or primary hypogonadism
Clinically, this disease is often misdiagnosed or wrongly treated or delayed treated due to lack of sufficient knowledge of the etiology and pathogenesis, which caused irreparable damage to patients.
The main objective of this research was to analyze the clinical features and to assess treatment effect of 120 male patients with gonadotropic hypogonadism from department of andrology in the First Hospital of Jilin University during October 2005 to May 2009.
Objective:To study the clinical characteristics, diagnosis and treatment of Male gonadotropic hypogonadism
Methods:120 data of male outpatients with gonadotropic hypogonadism from department of andrology in the First Hospital of Jilin University during October 2005 to May 2009.was retrospectively analyzed,its clinical features and treatment effect was evaluated.
Results:According to serum sexual hormone level,50 cases with hypergonadotropic hypogonadism including 42 cases with Klinefelter syndrome, and 8 cases with acquired hypergonadotropic hypogonadism (3 caused by serious testicular injury,3 with a clear history of mumps,2 cases with bilateral cryptorchidism) were diagnosed.Besides,70 cases of hypogonadotropic hypogonadism including 49 patients with idiopathic hypogonadotropic hypogonadism and 21 patients with Kallmann syndrome were diagnosed. Different treatment was given according to different hormone levels,and patients with hypergonado- tropic hypogonadism was given Testosterone undecanoate 40mg,3 times daily po, or 500mg, daily per 6-8 weeks, im. And patients with hypogonadotropic hypogonadism was administered human Chorionic Gonadotropin (HCG),1500IU, and human menopausal gonadotropin (HMG),75-150IU,2 times/week, Testosterone undecanoate 40 mg twice, po, was given simultaneously. After 1-2 years'follow-up, patients with hypogonadotropic hypogonadism received a significant treatment effect, and the effective rate was over 95%. Patients with hypergonadotropic hypogonadism also improved significantly in testicular volume and penile erection.
Conclusion:Male gonadotropic hypogonadism represented by Klinefelter syndrome and idiopathic hypogonadotropic hypogonadism is the most common organic disease that impacts male gonad development, causing male infertility. Patients with hypergonadotropic hypogonadism can receive significantly improvement in sexual desire and penile erection by androgen replacement therapy for whole life. Earlier initial treatment can also improve development of sex organs and secondary sexual characteristics. It is of little significance in improvement of reproductive function.However, to patients with hypogonadotropic hypogonadism, HCG combined with HMG therapy can improve development of adolescent secondary sexual characteristics, and can restore testosterone production and spermatogenesis, with no obvious side effects. At the same time, It can increase semen density in patients with asthenozoospermia and solve fertility problems. In addition, patients with larger testicular volume can obtain better treatment effect. It has no effec to olfactory dysfunction for patients with Kallmann syndrome.
引文
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[14]Silveira LF, MacColl GS, Bouloux PM. Hypogonadotropic hypogonadism[J]. Semin Reprod Med,2002,20:327-328
[15]Sato N, Katsumata N, Kagami M.et al.Clinical assessment and mutation analysis of Kallmann syndrome 1 (KAL1) and fibroblast growth factor receptor 1 (FGFR1, or KAL2) in five families and 18 sporadic patients[J].J Clin Endocrinol Metab.2004.89(3): 1079-88.
[16]Bhagavath B, Xu N, Ozata M. et al. KAL1 mutations are not a common cause of idiopathic hypogonadotrophic hypogonadism in humans[J]. Mol Hum Reprod.2007, 13(3):165-170.
[17]Groth C, Lardelli M,The structure and function of vertebrate fibroblast growth factor receptor 1[J]. Int J Dev Biol.2002,46(4):393-400.
[18]Dode C, Levilliers J, Dupont JM,et al. Loss-of-function mutations in FGFR1 cause autosomal dominant Kallmann syndrome [J]. Nat Genet.2003,33(4):463-465
[19]Pitteloud N, Acierno JS Jr, Meysing AU, et al. Reversible kallmann syndrome, delayed puberty, and isolated anosmia occurring in a single family with a mutation in the fibroblast growth factor receptor 1 gene[J]. J Clin Endocrinol Metab.2005,90(3):1317-22.
[20]Dode C, Teixeira L, Levilliers J,et al. Kallmann syndrome:mutations in the genes encoding prokineticin-2 and prokineticin receptor-2[J]. PLoS Genet.2006,2(10):e175.
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[22]Matsumoto S, Yamazaki C, Masumoto KH,et al. Abnormal development of the olfactory bulb and reproductive system in mice lacking prokineticin receptor PKR2[J].Proc Natl Acad Sci USA.2006,103(11):4140-4045.
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