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患有乳糜泻且绒毛完整的儿科患者的双糖酶缺乏症

Disaccharidase deficiency in pediatric patients with celiac disease and intact villi.

作者信息

Mones Richard L, Yankah Abena, Duelfer Diane, Bustami Rami, Mercer Geraldine

机构信息

The Division of Pediatric Gastroenterology and Nutrition of the Goryeb Children's Hospital, Atlantic Health System, Morristown, New Jersey 07960, USA.

出版信息

Scand J Gastroenterol. 2011 Dec;46(12):1429-34. doi: 10.3109/00365521.2011.619276. Epub 2011 Sep 22.

DOI:10.3109/00365521.2011.619276
PMID:21936724
Abstract

BACKGROUND AND AIMS

The "gold standard" for the diagnosis of celiac disease (CD) is the small intestinal biopsy. A significant number of biopsies are inadequate for interpretation. Furthermore, the labeling of a biopsy as a Marsh I or II is somewhat subjective and may vary with the experience of the pathologist. Our hypothesis is that patients with intact villi undergoing biopsies frequently have associated disaccharidase deficiencies (DSD).

METHODS

We reviewed 220 charts of pediatric patients with CD and selected those with a duodenal biopsy Marsh score of I/II. The disaccharidase (DS) levels of these patients were compared with a randomly selected, age-matched control group. DSD is defined as levels below the lower limits of normal.

RESULTS

Lactase (mean lactase = 18.8 in the control group vs. 4.2 in the diseased group, p = 0.004); sucrase (mean sucrase = 46.4 in the control group vs. 21.4 in the diseased group, p = 0.001); maltase (mean maltase = 138 in the control group vs. 52.5 in the diseased group, p = 0.001); palatinase (mean palatinase = 9.6 in the control group vs. 3.3 in the diseased group, p < 0.001).

CONCLUSION

There is a profound deficiency of DS levels in pediatric patients with CD who have intact villi.

摘要

背景与目的

乳糜泻(CD)诊断的“金标准”是小肠活检。大量活检标本难以用于解读。此外,将活检标记为马什I级或II级在一定程度上具有主观性,且可能因病理学家的经验而异。我们的假设是,接受活检且绒毛完整的患者常伴有双糖酶缺乏(DSD)。

方法

我们回顾了220例儿童CD患者的病历,选择十二指肠活检马什评分为I/II级的患者。将这些患者的双糖酶(DS)水平与随机选择的年龄匹配对照组进行比较。DSD定义为低于正常下限的水平。

结果

乳糖酶(对照组平均乳糖酶=18.8,疾病组为4.2,p=0.004);蔗糖酶(对照组平均蔗糖酶=46.4,疾病组为21.4,p=0.001);麦芽糖酶(对照组平均麦芽糖酶=138,疾病组为52.5,p=0.001);帕拉金酶(对照组平均帕拉金酶=9.6,疾病组为3.3,p<0.001)。

结论

绒毛完整的儿童CD患者存在严重的双糖酶水平缺乏。

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