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[严重低钠血症作为囊性纤维化的诊断症状]

[Severe hyponatremia as diagnostic symptom of cystic fibrosis].

作者信息

Sauter R, Will M, Helwig H

机构信息

St. Josefskrankenhaus, Abt. Kinderheilkunde und Jugendmedizin St. Hedwig, Freiburg.

出版信息

Klin Padiatr. 1997 Nov-Dec;209(6):361-3. doi: 10.1055/s-2008-1043976.

Abstract

Report on two babies, 5 and 6 months old with severe salt depletion were admitted to our hospital with fever and gastro-enteritis. One of them failed to thrive despite normal nutrition, the other one had a protracted gastro-enteritis. Both of them had a great loss of weight, a strong dehydration and were in reduced general condition but without any signs of coma or cerebral seizures. In the differential diagnosis cystic fibrosis with chronic loss of salt exacerbated during an infection, described as "Pseudo Bartter-Syndrome" was suspected. Both babies showed increased salt concentration in the sweat and decreased chymotrypsin in the stool.

摘要

报告了两名分别为5个月和6个月大的患有严重盐耗竭的婴儿,他们因发热和肠胃炎入院。其中一名尽管营养正常但发育不良,另一名患有迁延性肠胃炎。两人均体重大幅减轻、严重脱水,全身状况较差,但无昏迷或癫痫发作迹象。在鉴别诊断中,怀疑为感染期间慢性盐丢失加剧的囊性纤维化,即所谓的“假性巴特综合征”。两名婴儿均表现为汗液中盐浓度升高,粪便中糜蛋白酶降低。

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