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生长激素替代治疗伴中隔-视神经发育不良患者的严重高钠血症。

Severe hypernatraemia associated with growth hormone replacement therapy in a patient with septo-optic dysplasia.

机构信息

Department of Endocrinology, St Bartholomew's Hospital, London, UK.

出版信息

Pituitary. 2010 Jun;13(2):186-8. doi: 10.1007/s11102-008-0144-0.

DOI:10.1007/s11102-008-0144-0
PMID:18814036
Abstract

BACKGROUND

The anti-natriuretic properties of growth hormone (GH) are well established. Growth hormone deficiency (GHD) results in salt and water depletion and studies confirm that replacement leads to sodium and vasopressin-mediated water retention in patients with intact posterior pituitary function.

METHODS

We report the case of a 20-year-old male patient with septo-optic dysplasia, fixed cranial diabetes insipidus (DI) and an abnormal thirst threshold. With careful parental support, his sodium levels remained stable for many years on a fixed dose of DDAVP and a supervised fluid intake of 2.5 l/day. Several years after the original diagnosis, he was found to be ACTH deficient and following commencement of hydrocortisone replacement therapy became hypernatraemic. A new sodium homoeostasis was established with a higher dose of DDAVP. Subsequently, he developed symptoms typical of GHD and, after biochemical confirmation, GH replacement was commenced.

RESULTS

There was an immediate clinical improvement (increased alertness, improved concentration) but severe hypernatraemia developed (peak 169 mmol/l) necessitating revision of his desmopressin and fluid intake regimen.

CONCLUSION

Most GHD patients have intact posterior pituitary function. This case report highlights the powerful anti-natriuretic properties of GH. Endocrine physicians should be alert to this in patients with fixed DI and an abnormal thirst threshold.

摘要

背景

生长激素(GH)具有抗利尿作用,这一特性已得到充分证实。生长激素缺乏(GHD)可导致盐和水耗竭,研究证实,对于具有完整后叶垂体功能的患者,替代治疗会导致钠和血管加压素介导的水潴留。

方法

我们报告了一例 20 岁男性患者的病例,该患者患有隔-视神经发育不良、固定性颅糖尿病性尿崩症(DI)和异常口渴阈值。在仔细的家长支持下,他的钠水平在固定剂量的 DDAVP 和 2.5 升/天的监督液体摄入下多年来保持稳定。在最初诊断后的几年中,他被发现存在促肾上腺皮质激素缺乏症,并且在开始皮质醇替代治疗后出现高钠血症。通过增加 DDAVP 的剂量来建立新的钠稳态。随后,他出现了 GHD 的典型症状,经过生化确认后,开始进行生长激素替代治疗。

结果

立即出现了临床改善(警觉性提高,注意力集中),但随后出现严重的高钠血症(峰值 169mmol/l),需要调整去氨加压素和液体摄入方案。

结论

大多数 GHD 患者的后叶垂体功能完整。本病例报告强调了 GH 强大的抗利尿作用。内分泌科医生应该对固定性 DI 和异常口渴阈值的患者保持警惕。

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