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本文引用的文献

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Growth and the Growth Hormone-Insulin Like Growth Factor 1 Axis in Children With Chronic Inflammation: Current Evidence, Gaps in Knowledge, and Future Directions.慢性炎症患儿的生长和生长激素-胰岛素样生长因子 1 轴:现有证据、知识空白和未来方向。
Endocr Rev. 2016 Feb;37(1):62-110. doi: 10.1210/er.2015-1026. Epub 2015 Dec 31.
2
CFTR potentiator therapy ameliorates impaired insulin secretion in CF patients with a gating mutation.CFTR增强剂疗法可改善患有门控突变的CF患者受损的胰岛素分泌。
J Cyst Fibros. 2016 May;15(3):e25-7. doi: 10.1016/j.jcf.2015.10.012. Epub 2015 Nov 4.
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Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.囊性纤维化中的骨病:新的致病见解开启新疗法
Osteoporos Int. 2016 Apr;27(4):1401-1412. doi: 10.1007/s00198-015-3343-3. Epub 2015 Oct 2.
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Treatment of vitamin D deficiency in cystic fibrosis.囊性纤维化中维生素D缺乏的治疗。
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Recombinant growth hormone therapy for cystic fibrosis in children and young adults.重组生长激素治疗儿童和青年成人囊性纤维化
Cochrane Database Syst Rev. 2015 May 20(5):CD008901. doi: 10.1002/14651858.CD008901.pub3.
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Management of endocrine disease: Cystic fibrosis-related diabetes: novel pathogenic insights opening new therapeutic avenues.内分泌疾病的治疗:囊性纤维化相关性糖尿病:新的发病机制研究为治疗提供新途径。
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ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis-related diabetes in children and adolescents.《2014年国际儿童青少年糖尿病学会临床实践共识指南:儿童及青少年囊性纤维化相关糖尿病的管理》
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Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass.尽管胰岛细胞数量未减少,但囊性纤维化猪的血糖调节和胰岛素分泌仍存在异常。
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囊性纤维化中的内分泌紊乱

Endocrine Disorders in Cystic Fibrosis.

作者信息

Blackman Scott M, Tangpricha Vin

机构信息

Division of Pediatric Endocrinology, Department of Pediatrics, Johns Hopkins Hospital, Johns Hopkins University, 200 North Wolfe Street, Baltimore, MD 21287, USA.

Division of Endocrinology, Metabolism and Lipids, Department of Medicine, Atlanta VA Medical Center, Emory University School of Medicine, 101 Woodruff Circle NE, WMRB1301, Atlanta, GA 30322, USA.

出版信息

Pediatr Clin North Am. 2016 Aug;63(4):699-708. doi: 10.1016/j.pcl.2016.04.009.

DOI:10.1016/j.pcl.2016.04.009
PMID:27469183
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5354098/
Abstract

Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages.

摘要

囊性纤维化常并发内分泌紊乱。预计大多数患有囊性纤维化且伴有胰腺功能不全的患者会出现糖尿病,其发病年龄差异很大,但早期识别和治疗可改善发病率和死亡率。青春期相对延迟以及使用吸入性糖皮质激素会加剧身材矮小。囊性纤维化患者的骨病会导致脆性骨折,应通过监测骨密度和优化维生素D状态进行评估。检测和管理囊性纤维化患者的内分泌并发症可降低其发病率和死亡率。随着囊性纤维化患者群体的老龄化,这些并发症预计会变得更加常见。