Castanet M, Wieliczko M-C
Service de Pédiatrie Médicale, CHU Charles Nicolle, Université de Rouen, 1 rue de Germont 76031 Rouen cedex, France. mireille.castanet@chu- rouen.fr
Arch Pediatr. 2012 May;19 Suppl 1:S27-9. doi: 10.1016/S0929-693X(12)71105-3.
Since the 20 last years, the median age of survival has dramatically improved in children suffering from cystic fibrosis and complications such as growth retardation, pubertal delay and low bone mineral density are now more often than not observed in affected adolescents. The severity of the disease and the poor nutritional status due to pancreatic insufficiency and malabsorption are commonly implicated but recent data suggest that the disease could also play a role though the alteration of the chlore chanel (CFTR). Furthermore an increase prevalence of glucose intolerance and diabetes due to the progressive β cells destruction is observed in these children that make the life sometimes difficult for these adolescents already affected by an heavy chronic disease. The monitoring of the children should thus now become pluridisciplinary and include regular clinical evaluation of height and pubertal status, mineral bone density by DEXA and OGTT every two years since 10 years of age. Therefore, in addition to the standard treatment of cystic fibrosis is now added the vitamin D supplementation, the subcutaneous insulin therapy and may be the growth hormone that could be a new therapeutic demonstrating beneficial effects in these chronic disease. However further studies need to be performed to improve the management of these new endocrine complications more and more frequent in children and adolescents suffering from cystic fibrosis.
在过去20年里,患有囊性纤维化的儿童的中位生存年龄显著提高,现在受影响的青少年中生长发育迟缓、青春期延迟和低骨矿物质密度等并发症屡见不鲜。疾病的严重程度以及胰腺功能不全和吸收不良导致的营养状况不佳通常被认为是相关因素,但最近的数据表明,该疾病也可能通过氯通道(CFTR)的改变发挥作用。此外,由于β细胞逐渐受损,这些儿童中葡萄糖耐量异常和糖尿病的患病率增加,这使得这些已经受到严重慢性病影响的青少年的生活有时变得艰难。因此,现在对这些儿童的监测应成为多学科的,包括自10岁起每两年对身高和青春期状态进行定期临床评估、通过双能X线吸收法(DEXA)测量骨矿物质密度以及进行口服葡萄糖耐量试验(OGTT)。因此,除了囊性纤维化的标准治疗外,现在还增加了维生素D补充、皮下胰岛素治疗,可能还有生长激素,生长激素可能是一种对这些慢性病有有益作用的新疗法。然而,需要进行进一步的研究,以更好地管理囊性纤维化儿童和青少年中越来越常见的这些新的内分泌并发症。