Hypogonadism is one of the most common endocrine complications in patients with β-thalassemia major(β-TM). It can be clinically characterized by pubertal developmental delay, primary or secondary amenorrhea infertility that would significantly compromises the quality of life of patients with β-TM.Its pathogenesis is complex and may be related to long-term anemia, iron overload, decreased leptin levels, chronic liver disease, transplant pretreatment drugs and so on.Regular monitoring and active intervention are crucial for promoting adolescent development, sexual function maturation and retention of fertility in β-TM patients.However, there is no relevant guidelines and consensus in our country to guide clinicians on the follow-up of gonadal function in β-TM patients.This review aims to summarize the research progress in hypogonadism of patients with β-TM in order to improve the level of prevention and treatment.
Key words:
β-thalassemia major; Hypogonadism; Iron overload