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[用免疫反应性胰蛋白酶进行黏液黏稠症筛查]

[Mucoviscidosis screening with immunoreactive trypsin].

作者信息

Eber E, Ellemunter H, Engele H, Götz M, Grünberger W, Haas J, Janisch H, Leodolter S, Litscher H, Müller G

机构信息

Universitäts-Kinderklinik, Graz.

出版信息

Wien Klin Wochenschr. 1992;104(22):681-5.

PMID:1475973
Abstract

Up to now 49,116 immunoreactive trypsin (IRT) measurements have been carried out in Austrian newborns in the first week of life. Related to provisionally chosen cut-off points, 301 newborns (0.61%) showed an elevated IRT value; 253 of them were successfully recalled. According to a direct strategy, sweat tests were done without a second IRT measurement in 101 infants; eleven of them were identified as cystic fibrosis (CF) patients. In accordance with a 2-step strategy, 152 infants were reinvestigated by a second IRT determination. Twenty-eight of them again showed an elevated IRT value, as based on provisional, age-dependent reference values; seven were subsequently identified as CF patients by sweat testing. So far two false-negative findings were obtained on IRT screening: one child was later identified as having CF on the basis of typical clinical symptoms and a positive sweat test, the other patient presenting with meconium ileus showed a normal IRT value after surgery, but was subjected to a sweat test in view of the underlying condition. These preliminary results suggest a CF incidence of 1 to 2460 newborns in Austria. Hence, IRT screening appears to be a reliable method for identifying CF patients in the newborn period, thereby facilitating early treatment and genetic counselling.

摘要

截至目前,奥地利已对49116名新生儿在出生后第一周进行了免疫反应性胰蛋白酶(IRT)检测。根据临时选定的临界值,301名新生儿(0.61%)IRT值升高;其中253名成功召回。按照直接策略,对101名婴儿未进行第二次IRT检测直接进行了汗液检测;其中11名被确诊为囊性纤维化(CF)患者。按照两步策略,对152名婴儿进行了第二次IRT检测复查。根据临时的、与年龄相关的参考值,其中28名婴儿IRT值再次升高;随后通过汗液检测,7名被确诊为CF患者。到目前为止,IRT筛查出现了两例假阴性结果:一名儿童后来根据典型临床症状和阳性汗液检测结果被确诊为CF,另一名患有胎粪性肠梗阻的患者术后IRT值正常,但鉴于潜在病情仍进行了汗液检测。这些初步结果表明,奥地利CF发病率为每2460名新生儿中有1例。因此,IRT筛查似乎是在新生儿期识别CF患者的可靠方法,从而有助于早期治疗和遗传咨询。

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