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对囊性纤维化患者中发现的两个常见临床问题的管理综述:囊性纤维化相关糖尿病和生长发育不良。

A review of the management of two common clinical problems found in patients with cystic fibrosis: cystic fibrosis-related diabetes and poor growth.

作者信息

Hardin Dana S

机构信息

Division of Pediatric Endocrinology, Columbus Children's Hospital, Ohio State University, Columbus, Ohio, USA.

出版信息

Horm Res. 2007;68 Suppl 5:113-6. doi: 10.1159/000110603. Epub 2007 Dec 10.

DOI:10.1159/000110603
PMID:18174725
Abstract

BACKGROUND

Cystic fibrosis-related diabetes (CFRD) and glucose intolerance often occur in teens and adults with CF. The initial deficiency is an impaired first-phase insulin response; as patients age, peak insulin response is delayed and less robust than normal. Decreased insulin sensitivity (insulin resistance) is also present in patients with CF. Insulin is the only currently recommended therapy for all types of CFRD, and many clinicians find that basal/bolus regimens are optimal. Another common finding in children with CF is poor linear growth and inadequate weight gain. Recombinant human growth hormone (rhGH) is being explored in this patient population; studies overall show improvements in height and weight, without development of glucose intolerance.

CONCLUSIONS

rhGH may be a useful growth-promoting therapy in children with CF.

摘要

背景

囊性纤维化相关糖尿病(CFRD)和糖耐量异常常见于患有囊性纤维化(CF)的青少年及成人。最初的缺陷是第一阶段胰岛素反应受损;随着患者年龄增长,胰岛素反应峰值延迟,且不如正常情况强烈。CF患者还存在胰岛素敏感性降低(胰岛素抵抗)。胰岛素是目前针对所有类型CFRD唯一推荐的治疗方法,许多临床医生发现基础/餐时胰岛素方案是最佳的。CF患儿的另一个常见表现是线性生长不良和体重增加不足。重组人生长激素(rhGH)正在该患者群体中进行探索;总体研究表明身高和体重有所改善,且未出现糖耐量异常。

结论

rhGH可能是一种对CF患儿有用的促生长治疗方法。

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