Soliman Ashraf T, De Sanctis Vincenzo, Elalaily Rania, Yassin Mohamed
Department of Pediatric, Pediatric Endocrinology Division, Alamal Hospital, Hamad Medical Center, Qatar.
Department of Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.
Indian J Endocrinol Metab. 2015 Mar-Apr;19(2):245-51. doi: 10.4103/2230-8210.131750.
Despite improvement of blood transfusion regimens and iron chelation therapy growth and maturational delay, cardiomyopathy, endocrinopathies and osteoporosis still occur in good number of thalassemic patients. Decreased IGF-1 secretion occurs in the majority of the thalassemic patients particularly those with growth and pubertal delay. Many factors contribute to this decreased synthesis of IGF-I including disturbed growth hormone (GH) - insulin-like growth factor - I (IGF-I) axis. The possible factors contributing to low IGF-I synthesis in thalassemia and the possible interaction between low IGF-I secretion and the occurrence of these complications is discussed in this mini-review. Improvement of IGF-I secretion in thalassemic patients should be intended to improve linear growth and bone mineral accretion in thalassemic patients. This can be attained through adequate correction of anemia and proper chelation, nutritional supplementation (increasing caloric intake), correction of vitamin D and zinc deficiencies, induction of puberty and correction of hypogonadism at the proper time and treating GH deficiency. This review paper provides a summary of the current state of knowledge regarding IGF-I and factors affecting it in patients with thalassaemia major (TM). Search on PubMed and reference lists of articles with the term 'IGF-I, GH, growth, thalassemia, thyroxine, anemia, vitamin D, and zinc' was carried out. A hundred and forty-eight articles were found and used in the write up and the data analyzed was included in this report.
尽管输血方案和铁螯合疗法有所改进,但大量地中海贫血患者仍会出现生长和成熟延迟、心肌病、内分泌疾病及骨质疏松。大多数地中海贫血患者,尤其是那些生长和青春期延迟的患者,胰岛素样生长因子-1(IGF-1)分泌减少。多种因素导致IGF-I合成减少,包括生长激素(GH)-胰岛素样生长因子-I(IGF-I)轴紊乱。本综述讨论了地中海贫血中导致IGF-I合成降低的可能因素,以及低IGF-I分泌与这些并发症发生之间的可能相互作用。改善地中海贫血患者的IGF-I分泌旨在改善其线性生长和骨矿物质堆积。这可以通过充分纠正贫血、适当螯合、营养补充(增加热量摄入)、纠正维生素D和锌缺乏、适时诱导青春期和纠正性腺功能减退以及治疗生长激素缺乏来实现。本文综述了重型地中海贫血(TM)患者中IGF-I的现状及其影响因素。通过在PubMed上搜索以及查阅标题中含有“IGF-I、GH、生长、地中海贫血、甲状腺素、贫血、维生素D和锌”的文章参考文献列表进行研究。共找到148篇文章并用于撰写本文,分析的数据包含在本报告中。