Objective: To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major. Materials and methods: A prospective study of the function of the hypothalamic-...Objective: To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major. Materials and methods: A prospective study of the function of the hypothalamic-pituitary-gonadal axis function of 31 beta-thalassemia major females aged between 13 and 22 years and in 12 control females aged between 12 and 22 years. All were treated at Princess Rahma Teaching Hospital, Irbid, Northern Jordan between April 2001 and April 2003. Results: Of the 31 beta-thalassemia major females, 13 (41.9% ) had delayed puberty. Hypothalamic- pituitary-ovarian axis dysfunction was found in 15 patients (48.4% ). Twelve patients (38.7% ) had hypogonadotropic hypogonadism and 5 (16.1% ) had ovarian failure. High levels of serum ferritin were significantly higher in patients with delayed puberty. Conclusion: Pituitary and ovarian dysfunction are common problems in beta- thalassemia major patients. The main possible cause is iron overload. This stresses the need for intensive and regular use of chelation therapy to prevent damage to the hypothalamic-pituitary- ovarian axis.展开更多
体质性青春期发育延迟(constitutional delay of growth and puberty,CDGP)与先天性低促性腺激素性性腺功能减退(congenital hypogonadotropic hypogonadism,CHH)是两类导致青春期发育延迟的常见病因。两者在临床表现上具有较高的相似性...体质性青春期发育延迟(constitutional delay of growth and puberty,CDGP)与先天性低促性腺激素性性腺功能减退(congenital hypogonadotropic hypogonadism,CHH)是两类导致青春期发育延迟的常见病因。两者在临床表现上具有较高的相似性,均表现为缺乏正常青春期启动、血液循环中促性腺激素及性激素水平低下。尽管CDGP为一过性、自限性的青春期发育延迟,而CHH是持久性的性腺功能减退,但两者在成年期前较难准确鉴别。近年来的研究显示,微小青春期对于早期诊断CHH是一个非常重要的时间窗口。此外,抑制素B水平以及Kisspeptin兴奋试验可能在两者的鉴别诊断上具有较大的应用价值。展开更多
文摘Objective: To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major. Materials and methods: A prospective study of the function of the hypothalamic-pituitary-gonadal axis function of 31 beta-thalassemia major females aged between 13 and 22 years and in 12 control females aged between 12 and 22 years. All were treated at Princess Rahma Teaching Hospital, Irbid, Northern Jordan between April 2001 and April 2003. Results: Of the 31 beta-thalassemia major females, 13 (41.9% ) had delayed puberty. Hypothalamic- pituitary-ovarian axis dysfunction was found in 15 patients (48.4% ). Twelve patients (38.7% ) had hypogonadotropic hypogonadism and 5 (16.1% ) had ovarian failure. High levels of serum ferritin were significantly higher in patients with delayed puberty. Conclusion: Pituitary and ovarian dysfunction are common problems in beta- thalassemia major patients. The main possible cause is iron overload. This stresses the need for intensive and regular use of chelation therapy to prevent damage to the hypothalamic-pituitary- ovarian axis.
文摘体质性青春期发育延迟(constitutional delay of growth and puberty,CDGP)与先天性低促性腺激素性性腺功能减退(congenital hypogonadotropic hypogonadism,CHH)是两类导致青春期发育延迟的常见病因。两者在临床表现上具有较高的相似性,均表现为缺乏正常青春期启动、血液循环中促性腺激素及性激素水平低下。尽管CDGP为一过性、自限性的青春期发育延迟,而CHH是持久性的性腺功能减退,但两者在成年期前较难准确鉴别。近年来的研究显示,微小青春期对于早期诊断CHH是一个非常重要的时间窗口。此外,抑制素B水平以及Kisspeptin兴奋试验可能在两者的鉴别诊断上具有较大的应用价值。
