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肾淀粉样变性病患者的严重高钠血症。

Excessive hypernatremia in a patient with renal amyloid disease.

作者信息

Schorn T, Manschwetus H, Kühn K W

机构信息

Abteilung Nephrologie, Medizinische Hochschule Hannover.

出版信息

Klin Wochenschr. 1991 Jul 22;69(10):436-9. doi: 10.1007/BF01666829.

DOI:10.1007/BF01666829
PMID:1942955
Abstract

A 24-year-old Italian male presented with a nephrotic syndrome in September 1984. In February 1985 renal biopsy showed amyloid disease with tubular atrophy and interstitial fibrosis. Edema was treated with furosemide, and cholchicine was started. Because he feared side effects of drug therapy, the patient stopped all medication by July 1985. Instead, he decided to restrict severely fluids in order to fight edema. In early November 1985 his family noted he was becoming increasingly lethargic. Two weeks later, on his admission to the hospital, he had a serum sodium concentration of 193 mmol/l and serum osmolality of 397 mosm/kg. Apart from mild mental status changes neurological examination was normal. The fluid deficit was slowly corrected. He was discharged three weeks later with normal serum electrolytes. This case demonstrates that (1) severe hypernatremia can present with mild neurological symptoms and (2) it can be survived provided that it develops slowly and is corrected cautiously.

摘要

一名24岁的意大利男性于1984年9月出现肾病综合征。1985年2月肾活检显示为淀粉样变性病,伴有肾小管萎缩和间质纤维化。使用呋塞米治疗水肿,并开始使用秋水仙碱。由于患者担心药物治疗的副作用,到1985年7月他停用了所有药物。相反,他决定严格限制液体摄入以对抗水肿。1985年11月初,他的家人注意到他变得越来越嗜睡。两周后,他入院时血清钠浓度为193 mmol/L,血清渗透压为397 mosm/kg。除了轻度精神状态改变外,神经系统检查正常。液体缺乏得到缓慢纠正。三周后他出院,血清电解质正常。该病例表明:(1)严重高钠血症可表现为轻度神经症状;(2)如果高钠血症发展缓慢且谨慎纠正,患者可以存活。

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本文引用的文献

1
Nephrogenic diabetes insipidus caused by amyloid disease. Evidence in man of the role of the collecting ducts in concentrating urine.淀粉样疾病所致肾性尿崩症。人体中集合管在尿液浓缩中作用的证据。
Am J Med. 1960 Sep;29:539-44. doi: 10.1016/0002-9343(60)90050-4.
2
A case of 'essential' hypernatraemia due to resetting of the osmostat.一例因渗透压感受器重调而导致的“原发性”高钠血症病例。
Clin Endocrinol (Oxf). 1985 Apr;22(4):545-51. doi: 10.1111/j.1365-2265.1985.tb00155.x.
3
Hypodipsic hypernatremia with normal osmoregulation of vasopressin.
使用转基因NFAT-荧光素酶报告基因小鼠分析原代细胞中内源性NFAT5的转录活性。
BMC Mol Biol. 2008 Jan 25;9:13. doi: 10.1186/1471-2199-9-13.
伴有正常抗利尿激素渗透压调节的低渗性高钠血症
N Engl J Med. 1986 Aug 14;315(7):433-6. doi: 10.1056/NEJM198608143150706.
4
[Severe hypernatremia in acquired disorder of thirst and vasopressin regulation].[后天性口渴及抗利尿激素调节障碍所致的严重高钠血症]
Klin Wochenschr. 1988 Jun 1;66(11):498-501. doi: 10.1007/BF01876172.
5
Severe hypernatremia with impaired thirst.伴有口渴障碍的严重高钠血症
Am J Nephrol. 1989;9(5):421-34. doi: 10.1159/000168005.
6
Survival with severe hypernatremia.重度高钠血症患者的生存情况
Arch Intern Med. 1979 Aug;139(8):936-7.
7
Effects on the central nervous system of hypernatremic and hyponatremic states.高钠血症和低钠血症状态对中枢神经系统的影响。
Kidney Int. 1976 Jul;10(1):104-16. doi: 10.1038/ki.1976.82.