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肠内肠激酶缺乏症。一种新发现的蛋白质消化紊乱疾病。

Intestinal enterokinase deficiency. A newly-recognized disorder of protein digestion.

作者信息

Tarlow M J, Hadorn B, Arthurton M W, Lloyd J K

出版信息

Arch Dis Child. 1970 Oct;45(243):651-5. doi: 10.1136/adc.45.243.651.

Abstract

Enterokinase is an intestinal enzyme necessary for the activation of pancreatic proteolytic zymogens. Two children with absence of enterokinase activity in the duodenal juice and intestinal mucosa are described. Both had diarrhoea from birth and failed to thrive; one developed hypoproteinaemic oedema. Oral administration of pancreatic extract resulted in prompt control of symptoms and acceleration of growth. A simple screening test for enterokinase deficiency is described.

摘要

肠激酶是激活胰腺蛋白水解酶原所必需的一种肠道酶。本文描述了两名十二指肠液和肠黏膜中缺乏肠激酶活性的儿童。两人自出生起就患有腹泻且发育不良;其中一人出现了低蛋白血症性水肿。口服胰腺提取物可迅速控制症状并促进生长。本文还介绍了一种针对肠激酶缺乏症的简单筛查试验。

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本文引用的文献

1
The diagnostic value of faecal trypsin estimation in chronic pancreatic disease.
J Clin Pathol. 1962 Jan;15(1):62-8. doi: 10.1136/jcp.15.1.62.
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TRYPSINOGEN DEFICIENCY DISEASE.胰蛋白酶原缺乏症
J Pediatr. 1965 Feb;66:275-85. doi: 10.1016/s0022-3476(65)80184-6.
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Trypsinogen deficiency disease.胰蛋白酶原缺乏症
Am J Dis Child. 1967 Aug;114(2):203-8. doi: 10.1001/archpedi.1967.02090230133019.
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Some long-term effects of severe malnutrition in early life.
Lancet. 1969 Sep 6;2(7619):514-7. doi: 10.1016/s0140-6736(69)90216-5.
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Intestinal enterokinase deficiency.
Lancet. 1969 Apr 19;1(7599):812-3. doi: 10.1016/s0140-6736(69)92071-6.

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