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[患有与囊性纤维化相关的假性巴特综合征患者的食物蛋白诱导的小肠结肠炎综合征。病例报告]

[Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report].

作者信息

Camargo-Vargas Bethy, Varela María, Wilches Laura, Martínez-Quiñones Paula Andrea, Acuña-Cordero Ranniery

机构信息

Hospital Militar Central, Servicio de Neumología Pediátrica, Bogotá, Colombia.

出版信息

Rev Alerg Mex. 2021 Oct-Dec;68(4):300-303. doi: 10.29262/ram.v68i4.974.

Abstract

INTRODUCTION

Food protein-induced enterocolitis is a non-immunoglobulin E-mediated food allergy with acute manifestations like recurrent vomiting, dehydration, and shock. It is a rare pathology that requires a high index of suspicion. Pseudo-Bartter syndrome (metabolic alkalosis, hypokalemia and hypochloremia in the absence of tubulopathy) is an infrequent complication of cystic fibrosis.

CASE REPORT

A 5-month-old boy with recurrent vomiting, dehydration, and shock; who had been breastfed and had consumed baby formula three hours prior to the onset of symptoms. Laboratory tests confirmed hyponatremia, hypochloremic metabolic alkalosis, and hypokalemia in absence of tubulopathy; two iontophoresis showed altered results, stool elastase was decreased, and genetic sequencing confirmed the diagnosis of cystic fibrosis. The provocation test confirmed food protein-induced enterocolitis syndrome.

CONCLUSION

Recurrent vomiting and dehydration after the intake of milk formula must lead to suspicion of food protein-induced enterocolitis syndrome. If pseudo-Bartter syndrome is found, cystic fibrosis must be ruled out.

摘要

引言

食物蛋白诱导的小肠结肠炎是一种非免疫球蛋白E介导的食物过敏,具有反复呕吐、脱水和休克等急性表现。这是一种罕见的病症,需要高度怀疑。假性巴特综合征(在无肾小管病变情况下出现代谢性碱中毒、低钾血症和低氯血症)是囊性纤维化的一种罕见并发症。

病例报告

一名5个月大的男婴,出现反复呕吐、脱水和休克;他一直母乳喂养,在症状发作前三小时食用了婴儿配方奶粉。实验室检查证实存在低钠血症、低氯性代谢性碱中毒和低钾血症,且无肾小管病变;两次离子电渗疗法结果异常,粪便弹性蛋白酶降低,基因测序确诊为囊性纤维化。激发试验证实为食物蛋白诱导的小肠结肠炎综合征。

结论

摄入牛奶配方奶粉后反复出现呕吐和脱水必须怀疑食物蛋白诱导的小肠结肠炎综合征。如果发现假性巴特综合征,必须排除囊性纤维化。

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