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儿童暂时性外分泌胰腺功能不全:一个已存在的实体?

Transient Exocrine Pancreatic Insufficiency in Children: An Existing Entity?

机构信息

Pediatric Gastroenterology and Nutrition Institute, Rambam Health Care Campus.

Rappaport Family Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.

出版信息

J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):574-577. doi: 10.1097/MPG.0000000000002267.

DOI:10.1097/MPG.0000000000002267
PMID:30633105
Abstract

OBJECTIVES

Pancreatic insufficiency in children is usually associated with diseases such as cystic fibrosis, Shwachman-Diamond syndrome, or chronic pancreatitis. Fecal elastase-1 is a reliable laboratory test for the diagnosis of exocrine pancreatic insufficiency (EPI). Transient pancreatic insufficiency has been rarely described and data on this entity are lacking in the medical literature. In this retrospective study we report 17 cases of transient pancreatic insufficiency presented mainly with failure to thrive and/or diarrhea.

METHODS

We followed 43 children (age range 1 month-18 years) with low fecal elastase-1 in our institution between the years 2009 and 2017. We followed growth and laboratory results (particularly, complete blood count, albumin, transaminases, celiac serology, sweat test, and fat-soluble vitamins). Elastase levels <200 mg/g were considered as pancreatic insufficiency.

RESULTS

Twenty-six were excluded due to missing data, a comorbidity or being syndromatic. Enrolled children (17) were all otherwise healthy.The median age at diagnosis was 3 years (range 0.2-15 years), 11 girls and 6 boys. Their main presenting symptoms were failure to thrive and/or diarrhea. Median fecal elastase-1 levels were 71 mg/g (range 18-160). Median time for normalization was 6 months (range 1-48 months). Abdominal sonography, celiac serology, and sweat test were normal for all patients. Most patients were treated with pancreatic enzymes until resolution.

CONCLUSIONS

Transient EPI without clear etiology should be in the differential diagnosis of EPI after ruling out known etiologies. The resolving course pattern may be attributed to an unidentified infectious agent. Further studies to assess the etiology are mandated.

摘要

目的

儿童胰腺功能不全通常与囊性纤维化、Shwachman-Diamond 综合征或慢性胰腺炎等疾病有关。粪便弹性蛋白酶-1 是诊断外分泌胰腺功能不全(EPI)的可靠实验室检测。短暂性胰腺功能不全很少被描述,且医学文献中缺乏关于该病症的数据。在这项回顾性研究中,我们报告了 17 例以生长不良和/或腹泻为主要表现的短暂性胰腺功能不全病例。

方法

我们对 2009 年至 2017 年在我院就诊的 43 例粪便弹性蛋白酶-1 水平较低的儿童(年龄范围为 1 个月至 18 岁)进行了随访。我们观察了他们的生长情况和实验室结果(尤其是全血细胞计数、白蛋白、转氨酶、乳糜泻血清学、汗液试验和脂溶性维生素)。将弹性蛋白酶水平<200mg/g 视为胰腺功能不全。

结果

由于数据缺失、合并症或综合征,26 例患儿被排除在外。纳入的患儿(17 例)均无其他健康问题。中位诊断年龄为 3 岁(范围为 0.2-15 岁),其中 11 例为女孩,6 例为男孩。他们的主要表现为生长不良和/或腹泻。中位粪便弹性蛋白酶-1 水平为 71mg/g(范围为 18-160)。中位恢复时间为 6 个月(范围为 1-48 个月)。所有患者的腹部超声、乳糜泻血清学和汗液试验均正常。大多数患者在缓解后接受胰酶治疗。

结论

在排除已知病因后,对于 EPI 病因不明的短暂性 EPI 应纳入鉴别诊断。这种缓解过程可能归因于未识别的感染因子。需要进一步研究以评估病因。

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Transient Exocrine Pancreatic Insufficiency in Children: An Existing Entity?儿童暂时性外分泌胰腺功能不全:一个已存在的实体?
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2
Diagnostic Performance of Measurement of Fecal Elastase-1 in Detection of Exocrine Pancreatic Insufficiency: Systematic Review and Meta-analysis.粪便弹性蛋白酶-1 检测在外分泌胰腺功能不全诊断中的性能:系统评价和荟萃分析。
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[Fecal elastase-1: a useful test in pediatric practice].
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