Pomarède R, Girot R, Constant S, Rappaport R
Arch Fr Pediatr. 1984 Apr;41(4):255-9.
This study reports on the endocrine abnormalities associated with delayed growth and puberty, observed in 10 children presenting with thalassemia major. Hormonal changes were followed up during treatment with regular transfusions and efficient chelating agents. Recovery of growth and onset of puberty were observed in most cases. When puberty was delayed, an associated substitutive treatment with sexual hormones was useful. In one case only, an isolated gonadotropic deficiency could be proven. In all cases STH, TSH and PRL secretions were normal. Without efficient and early treatment, the main consequence of the disease with respects to growth seems to be the risk of delayed puberty, mainly functional, or rarely hypogonadotropic due to definite LH and FSH deficiency.
本研究报告了10例重型地中海贫血患儿中观察到的与生长发育迟缓和青春期延迟相关的内分泌异常情况。在用定期输血和有效的螯合剂治疗期间对激素变化进行了随访。大多数病例观察到生长恢复和青春期启动。青春期延迟时,给予性激素替代治疗是有效的。仅在1例中证实存在孤立性促性腺激素缺乏。所有病例中生长激素、促甲状腺激素和催乳素分泌均正常。若没有有效且早期的治疗,该疾病在生长方面的主要后果似乎是青春期延迟的风险,主要是功能性的,或因明确的促黄体生成素和促卵泡生成素缺乏而罕见地出现低促性腺激素性青春期延迟。