在杜申肌力发育不良症的青春期诱导后,是否需要持续的丸激素?
Claire L Wood1, Kieren G Hollingsworth2, Edrina Bokaie3
1C Wood, Department of Paediatric Endocrinology, Royal Victoria Infirmary, Newcastle upon Tyne, United Kingdom of Great Britain and Northern Ireland.
Endocrine connections
|September 28, 2023
概括
在杜氏肌肉发育不良症 (DMD) 的男孩中,丸激素治疗有助于激活生殖轴,并增加丸激素水平. 这种青春期诱导显示出有希望的生育结果,尽管肌肉质量可能会减少.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 神经肌肉疾病 神经肌肉疾病
背景情况:
- 葡萄糖皮质类药物 (GC) 用于控制杜氏肌肉发育不良 (DMD) 的炎症和肌肉功能,但它们可以延迟青春期.
- 建议在这些患者中进行丸激素治疗以诱导青春期,但长期结果需要进一步调查.
研究的目的:
- 评估丸激素治疗对下丘脑-垂体-淋巴结核 (HPG) 轴,肌肉体积和DMD男孩的功能长期影响,在青春期诱导后5年.
主要方法:
- 一项前性观察后续研究涉及15名接受GC治疗的患有DMD的男性,他们接受了2年的增量丸激素治疗.
- 评估包括丸体积 (TV),淋巴腺素,丸激素,B抑制素,肌肉功能和肢体肌肉MRI在治疗后2.5年和5年.
主要成果:
- 在治疗后5年,水平保持稳定. 丸体积和阻抗B水平显著增加,表明HPG轴激活,尽管阻抗B仍低于参考值.
- 虽然丸体和丸体积有所改善,但在随访期间,肌肉收缩量减少.
结论:
- 在DMD患者中,丸激素治疗与HPG轴激活和改善生殖激素配置相关,为生育提供了潜在的益处.
- 监测和补充丸激素水平至关重要,因为它对骨健康,肌肉和整体福祉的积极影响.
相关概念视频
Signs of Puberty
403
Puberty is a critical phase, typically beginning between the ages of 8 and 13 in girls and 9 and 14 in boys, though timing can vary based on genetics, environmental factors, and overall health. This period is characterized by the development of secondary sexual characteristics and the attainment of reproductive potential. Endocrine changes underpin puberty, with hormonal surges of Luteinizing Hormone (LH) and Follicle-Stimulating Hormone (FSH) instigated by Gonadotropin-Releasing Hormone (GnRH)...
403
Insulin: Dosing Regimen and Adverse Effects
198
Insulin-replacement therapy usually includes both long-acting insulin (basal) and short-acting insulin (to cater to postprandial needs). In a diverse group of type 1 diabetes patients, the average daily insulin dose is typically 0.5-0.7 units/kg body weight. However, obese patients and pubertal adolescents may need more due to insulin resistance.
The basal dose constitutes about 40%-50% of the total daily dose, with the rest as premeal insulin. The mealtime insulin dose should mirror...
The basal dose constitutes about 40%-50% of the total daily dose, with the rest as premeal insulin. The mealtime insulin dose should mirror...
198
Testosterone: Functions and Regulation
725
The intricate hormonal interplay essential for male reproductive health begins with the release of gonadotropin-releasing hormone (GnRH) by the hypothalamus. This hormone prompts the pituitary gland to secrete follicle-stimulating hormone (FSH) and luteinizing hormone (LH). LH targets the Leydig cells in the testes, stimulating them to produce and release testosterone. In concert with testosterone, FSH acts on the Sertoli cells within the seminiferous tubules to facilitate the release of...
725
Satellite Stem Cells and Muscular Dystrophy
2.0K
Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
2.0K


