Department of Neuroscience, Reproductive Science and Odontostomatology, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Department of Neuroscience, Reproductive Science and Odontostomatology, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Reprod Biomed Online. 2021 Apr;42(4):835-841. doi: 10.1016/j.rbmo.2020.12.012. Epub 2020 Dec 27.
Wilson's disease (WD) is a disorder of copper metabolism that can cause hormonal alterations. The impact of WD and its therapies on fertility is not well defined. The aim of this study was to evaluate ovarian reserve and sperm parameters in long-term treated WD patients with hepatic onset.
WD patients with hepatic onset treated for at least 5 years were compared with healthy controls. Men underwent spermiogram and sperm DNA fragmentation (SDF) analysis. Women were tested for serum FSH, anti-Müllerian hormone (AMH) and sonographic antral follicle count (AFC) in the early follicular phase. Ovulation was monitored with ultrasound and progesterone serum concentrations in the luteal phase.
The WD group included 26 patients (12 males), the control group 19 subjects (9 males). All patients apart from four (one male) were responders to WD treatment. Sperm count and morphology were comparable between cases and controls. Sperm motility (total and after 1 h) was significantly lower in cases (44.78 ± 21.65%; 47.85 ± 21.52%) than controls (61.88 ± 11.03; 69.44 ± 11.02%, P = 0.03 and 0.01, respectively). The only non-responder had severe oligo-astheno-teratozoospermia. SDF values were normal in cases and controls. AMH, AFC and FSH did not differ between cases and controls. LH was significantly lower in cases (3.36 ± 1.65 mIU/ml) than controls (6.25 ± 1.03 mIU/ml, P < 0.0001). A non-responder woman who developed neurological signs had a 7-year history of infertility.
WD patients with hepatic onset, diagnosed early and treated, have no impairment in fertility potential even if males show reduced sperm motility and females lower LH values. Only patients with poor disease control have some evidence of impaired fertility.
威尔逊病（WD）是一种铜代谢紊乱疾病，可导致激素改变。WD 及其治疗对生育力的影响尚未明确。本研究旨在评估肝起病的长期 WD 患者的卵巢储备和精子参数。
将肝起病且至少接受 5 年治疗的 WD 患者与健康对照组进行比较。男性接受精子常规检查和精子 DNA 碎片化（SDF）分析。女性在卵泡早期检测血清卵泡刺激素（FSH）、抗苗勒氏管激素（AMH）和超声窦卵泡计数（AFC）。通过超声和黄体期血清孕激素浓度监测排卵。
WD 组包括 26 例患者（12 例男性），对照组 19 例（9 例男性）。除 4 例（1 例男性）外，所有患者均对 WD 治疗有反应。病例组和对照组的精子计数和形态无差异。病例组的精子活力（总活力和 1 小时后活力）明显低于对照组（44.78±21.65%和 47.85±21.52%；61.88±11.03%和 69.44±11.02%，P=0.03 和 0.01）。唯一的无反应者患有严重的少精-弱精-畸形精子症。病例组和对照组的 SDF 值均正常。病例组和对照组的 AMH、AFC 和 FSH 无差异。病例组的 LH 明显低于对照组（3.36±1.65mIU/ml 和 6.25±1.03mIU/ml，P<0.0001）。1 例出现神经系统症状的无反应女性有 7 年不孕史。
早期诊断和治疗的肝起病 WD 患者的生育能力没有受损，即使男性的精子活力降低，女性的 LH 值降低。只有疾病控制不佳的患者才有一些生育能力受损的证据。
