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[儿童囊性纤维化的内分泌并发症]

[Endocrine complications of cystic fibrosis in childhood].

作者信息

Castanet M, Wieliczko M-C

机构信息

Service de Pédiatrie Médicale, CHU Charles Nicolle, Université de Rouen, 1 rue de Germont 76031 Rouen cedex, France. mireille.castanet@chu- rouen.fr

出版信息

Arch Pediatr. 2012 May;19 Suppl 1:S27-9. doi: 10.1016/S0929-693X(12)71105-3.

DOI:10.1016/S0929-693X(12)71105-3
PMID:22682486
Abstract

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.

摘要

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。

相似文献

1
[Endocrine complications of cystic fibrosis in childhood].[儿童囊性纤维化的内分泌并发症]
Arch Pediatr. 2012 May;19 Suppl 1:S27-9. doi: 10.1016/S0929-693X(12)71105-3.
2
Endocrine complications of cystic fibrosis.囊性纤维化的内分泌并发症
Adolesc Med. 2002 Feb;13(1):145-59, vii-viii.
3
Advances in the recognition and treatment of endocrine complications in children with chronic illness.慢性病患儿内分泌并发症的识别与治疗进展。
Adv Pediatr. 1999;46:101-49.
4
Endocrine and bone metabolic complications in chronic liver disease and after liver transplantation in children.儿童慢性肝病及肝移植后内分泌和骨骼代谢并发症。
J Pediatr Gastroenterol Nutr. 2012 Mar;54(3):313-21. doi: 10.1097/MPG.0b013e31823e9412.
5
Nutrition in cystic fibrosis.囊性纤维化中的营养
Semin Respir Crit Care Med. 2009 Oct;30(5):579-86. doi: 10.1055/s-0029-1238916. Epub 2009 Sep 16.
6
Endocrine Disorders in Cystic Fibrosis.囊性纤维化中的内分泌紊乱
Pediatr Clin North Am. 2016 Aug;63(4):699-708. doi: 10.1016/j.pcl.2016.04.009.
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Growth and nutritional status in children and adolescents with cystic fibrosis.患有囊性纤维化的儿童和青少年的生长发育及营养状况
Ann Hum Biol. 2008 Mar-Apr;35(2):145-53. doi: 10.1080/03014460701824132.
8
[Glucose intolerance and diabetes during cystic fibrosis].[囊性纤维化期间的葡萄糖不耐受和糖尿病]
Ann Pediatr (Paris). 1991 Apr;38(4):244-8.
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[Genotype and phenotype of gastrointestinal symptoms analysis in children with cystic fibrosis].[囊性纤维化患儿胃肠道症状的基因型与表型分析]
Pol Merkur Lekarski. 2005 Feb;18(104):205-9.
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[Value of continuous glucose monitoring in screening for diabetes in cystic fibrosis].[持续葡萄糖监测在囊性纤维化患者糖尿病筛查中的价值]
Arch Pediatr. 2009 Dec;16(12):1540-6. doi: 10.1016/j.arcped.2009.09.007. Epub 2009 Oct 24.