Al-Rimawi Hala Saleh, Jallad Mohammad Fayez, Amarin Zouhair Odeh, Al Sakaan Rula
Department of Pediatrics, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Fertil Steril. 2006 Oct;86(4):886-90. doi: 10.1016/j.fertnstert.2006.02.118.
To examine the hormonal status of the hypothalamic-pituitary-gonadal axis in adolescent males with beta-thalassemia major.
Controlled clinical study.
Tertiary referral teaching hospital.
PATIENT(S): Thirty-three adolescent males with beta-thalassemia major.
INTERVENTION(S): Basal LH, FSH, and T were examined. All individuals received 100 microg GnRH analogue. Four hours later the hormone levels were retested. Patients with beta-thalassemia and low T levels received hCG.
MAIN OUTCOME MEASURE(S): The preintervention and postintervention levels of FSH, LH, and T were examined.
RESULT(S): Of the 33 beta-thalassemia major adolescents, 17 had delayed puberty. The difference in basal LH, FSH, and T levels between delayed and normal puberty beta-thalassemia groups were statistically significant. These levels were significantly lower compared with the constitutional delayed puberty group and become even more significant after GnRH analogue administration. The T levels in the beta-thalassemia group were significantly lower than in the control group. After hCG administration, the T levels remained significantly lower in the delayed-puberty beta-thalassemia compared to the normal-puberty beta-thalassemia group.
CONCLUSION(S): Despite recent therapeutic advances in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is one of the most frequent endocrine complications.
研究重型β地中海贫血青少年男性下丘脑-垂体-性腺轴的激素状态。
对照临床研究。
三级转诊教学医院。
33例重型β地中海贫血青少年男性。
检测基础促黄体生成素（LH）、促卵泡生成素（FSH）和睾酮（T）水平。所有个体接受100微克促性腺激素释放激素（GnRH）类似物。4小时后重新检测激素水平。重型β地中海贫血且T水平低的患者接受人绒毛膜促性腺激素（hCG）治疗。
检测干预前后FSH、LH和T的水平。
33例重型β地中海贫血青少年中，17例青春期延迟。青春期延迟和正常的重型β地中海贫血组之间基础LH、FSH和T水平的差异具有统计学意义。与体质性青春期延迟组相比，这些水平显著降低，给予GnRH类似物后差异更加显著。重型β地中海贫血组的T水平显著低于对照组。给予hCG后，青春期延迟的重型β地中海贫血组的T水平仍显著低于青春期正常的重型β地中海贫血组。
尽管近期重型β地中海贫血的治疗取得了进展，但继发性内分泌功能障碍的风险仍然很高。性腺功能减退是最常见的内分泌并发症之一。
