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囊性纤维化中的免疫反应性胰蛋白酶

Immunoreactive trypsin in cystic fibrosis.

作者信息

Davidson A G, Wong L T, Kirby L T, Applegarth D A

出版信息

J Pediatr Gastroenterol Nutr. 1984;3 Suppl 1:S79-88. doi: 10.1097/00005176-198400031-00014.

Abstract

Since the first observation in 1979 that CF infants have elevated blood IRT, studies in various centres have enabled us to more fully understand the importance of this phenomenon. There is increasing evidence to show that mass newborn screening for CF using the IRT assay is practical and is capable of detecting essentially all CF newborns at a cost comparable to existing screening programs for other disorders such as Hypothyroidism. Although the elevated IRT levels seen in infancy in CF soon decrease, IRT levels in older CF patients appear to quite closely reflect the capability of that patient to secrete pancreatic enzymes and can be helpful in separating CF patients whose ability to secrete enzymes is preserved, from those with diminished exocrine pancreatic function. In all CF patients there appears to be an altered relationship between pancreatic exocrine secretion and circulating IRT levels as compared to control patients. This is probably one further manifestation of a secretory obstructive defect which although not uniformly severe, is common to all CF patients.

摘要

自1979年首次观察到囊性纤维化(CF)婴儿血液中免疫反应性胰蛋白酶(IRT)升高以来,各中心的研究使我们能够更全面地理解这一现象的重要性。越来越多的证据表明,使用IRT检测法对CF进行大规模新生儿筛查是可行的,并且能够以与现有其他疾病(如甲状腺功能减退症)筛查项目相当的成本检测出几乎所有CF新生儿。尽管CF婴儿期出现的IRT水平升高很快会下降,但年长CF患者的IRT水平似乎相当密切地反映了该患者分泌胰酶的能力,并且有助于区分胰酶分泌能力保留的CF患者和外分泌胰腺功能减退的患者。与对照患者相比,所有CF患者的胰腺外分泌与循环IRT水平之间的关系似乎都有所改变。这可能是分泌性梗阻缺陷的又一表现,这种缺陷虽然并非一律严重,但在所有CF患者中都很常见。

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