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儿童孤立性淀粉酶缺乏症及其临床意义。

Isolated Amylase Deficiency in Children and Its Clinical Implication.

作者信息

Hopson Puanani, Patel Samit, Bornstein Jeffrey, Mehta Devendra, Horvath Karoly

机构信息

Center for Digestive Health and Nutrition, Arnold Palmer Hospital for Children, University of Florida.

The Florida State University, Center for Digestive Health and Nutrition, Arnold Palmer Hospital for Children, Orlando, FL.

出版信息

J Pediatr Gastroenterol Nutr. 2019 Jun;68(6):854-860. doi: 10.1097/MPG.0000000000002317.

Abstract

OBJECTIVES

Among the 3 lines of pancreatic enzymes, amylase secretion develops last and it is not detected in duodenal aspirates of infants in the first month after birth. The aim of this study was to assess the prevalence and symptoms of isolated amylase deficiency in children.

METHODS

During a 6-year period, we performed endoscopic pancreatic function tests (ePFT) in 712 children. Isolated amylase deficiency was defined as activity that was below the third percentile of our referenced population with normal lipase and protease activities.

RESULTS

Seventy-two children between age 0.21 and 15.7 years (boys, n = 35) had isolated amylase deficiency. The highest prevalence of isolated amylase deficiency was found in patients less than 6 months of age (52.9%). From 6 months to 1 year of age, the prevalence was 40%. The prevalence gradually decreased until 18 months. Failure to thrive, poor weight gain, diarrhea, and abdominal bloating were the most frequent indications for ePFT. Eleven children had repeat ePFT after initial diagnosis and 6 had normal enzyme activity, whereas 5 had remained amylase-deficient an average of 1.65 years later.

CONCLUSIONS

The prevalence of selective amylase deficiency was 10.1% in the 712 children who underwent ePFT with the suspicion of malabsorption. Low amylase activity is "physiologic" in infants <6 months of age, however, this study supports that it should be considered in the differential diagnosis in children older than 6 months of age.

摘要

目的

在胰腺的三种酶系中,淀粉酶分泌发育最晚,出生后第一个月婴儿的十二指肠抽吸物中检测不到淀粉酶。本研究的目的是评估儿童孤立性淀粉酶缺乏症的患病率和症状。

方法

在6年期间,我们对712名儿童进行了内镜胰腺功能测试(ePFT)。孤立性淀粉酶缺乏症定义为活性低于我们参考人群中脂肪酶和蛋白酶活性正常者的第三百分位数。

结果

72名年龄在0.21至15.7岁之间的儿童(男孩35名)存在孤立性淀粉酶缺乏症。孤立性淀粉酶缺乏症患病率最高的是年龄小于6个月的患者(52.9%)。6个月至1岁时,患病率为40%。患病率逐渐下降直至18个月。生长发育不良、体重增加缓慢、腹泻和腹胀是进行ePFT最常见的指征。11名儿童在初次诊断后进行了重复ePFT,6名儿童酶活性正常,而5名儿童在平均1.65年后仍存在淀粉酶缺乏。

结论

在712名因怀疑吸收不良而接受ePFT的儿童中,选择性淀粉酶缺乏症的患病率为10.1%。淀粉酶活性低在6个月以下婴儿中是“生理性的”,然而,本研究支持在6个月以上儿童的鉴别诊断中应考虑这一情况。

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