Boffa C, Franchi E, Colleoni M C, Mariani D, Rivolta S, Siccardo L, Terzoli S, Carnelli V
Pediatr Med Chir. 1984 Nov-Dec;6(6):759-64.
Endocrine disfunction are well known in young patients with thalassaemia major and are mainly due to iron overload. Gonadotrophin releasing hormone (GnRH) was administered to 13 boys aging more than 14 years and to 13 girls aging more than 13 years to assess pituitary function. Human Chorionic gonadotrophin (HCG) was also administered to 13 boys to evaluate their gonadal function. Most of the patients had evidence of pituitary hypofunction and in 4 boys there was evidence of gonadic failure. This study is mandatory for a correct therapeutic approach.
重型地中海贫血的年轻患者中内分泌功能障碍很常见,主要是由于铁过载。对13名年龄超过14岁的男孩和13名年龄超过13岁的女孩给予促性腺激素释放激素(GnRH)以评估垂体功能。还对13名男孩给予人绒毛膜促性腺激素(HCG)以评估他们的性腺功能。大多数患者有垂体功能减退的证据,4名男孩有性腺功能衰竭的证据。这项研究对于正确的治疗方法是必不可少的。