• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

先天性蔗糖酶-异麦芽糖酶缺乏症采用酿酒酵母进行酶替代治疗。

Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency.

作者信息

Harms H K, Bertele-Harms R M, Bruer-Kleis D

出版信息

N Engl J Med. 1987 May 21;316(21):1306-9. doi: 10.1056/NEJM198705213162104.

DOI:10.1056/NEJM198705213162104
PMID:3553946
Abstract

Sucrase-isomaltase deficiency is an inherited disaccharidase deficiency that leads to malabsorption of sucrose, with resulting diarrhea and abdominal distention and cramps. We investigated the sucrose-splitting effect of viable yeast cells in eight children with congenital sucrase-isomaltase deficiency, by means of the sucrose hydrogen breath test. This test is based on the fact that hydrogen is released from the malabsorbed sucrose by the colonic microflora. We found that 0.3 g of lyophilized Saccharomyces cerevisiae, given after loading with 2 g of sucrose per kilogram of body weight, reduced hydrogen excretion in all patients, on average by 70 percent, in parallel with a complete loss or evident reduction of clinical symptoms. In vitro, lyophilized and fresh S. cerevisiae (fresh baker's yeast) had appreciable sucrase activity, a low isomaltase and maltase activity, and virtually no lactase activity. The sucrase activity was more inhibited by undiluted than by diluted gastric juice. We conclude that patients with congenital sucrase-isomaltase deficiency who intentionally or unintentionally consume sucrose can ameliorate the malabsorption by subsequently ingesting a small amount of viable yeast cells, preferably on a full stomach.

摘要

蔗糖酶-异麦芽糖酶缺乏症是一种遗传性双糖酶缺乏症,可导致蔗糖吸收不良,进而引起腹泻、腹胀和痉挛。我们通过蔗糖氢呼气试验,研究了活酵母细胞对8名先天性蔗糖酶-异麦芽糖酶缺乏症患儿的蔗糖分解作用。该试验基于结肠微生物群从吸收不良的蔗糖中释放出氢气这一事实。我们发现,每千克体重摄入2克蔗糖后给予0.3克冻干酿酒酵母,所有患者的氢气排泄量平均降低了70%,同时临床症状完全消失或明显减轻。在体外,冻干和新鲜的酿酒酵母(新鲜面包酵母)具有相当的蔗糖酶活性、较低的异麦芽糖酶和麦芽糖酶活性,且几乎没有乳糖酶活性。与稀释后的胃液相比,未稀释的胃液对蔗糖酶活性的抑制作用更强。我们得出结论,先天性蔗糖酶-异麦芽糖酶缺乏症患者在有意或无意中摄入蔗糖后,随后摄入少量活酵母细胞(最好在饱腹时)可改善吸收不良的情况。

相似文献

1
Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency.先天性蔗糖酶-异麦芽糖酶缺乏症采用酿酒酵母进行酶替代治疗。
N Engl J Med. 1987 May 21;316(21):1306-9. doi: 10.1056/NEJM198705213162104.
2
Breath hydrogen test and sucrase isomaltase deficiency.呼气氢试验与蔗糖酶异麦芽糖酶缺乏症
Arch Dis Child. 1983 Aug;58(8):595-7. doi: 10.1136/adc.58.8.595.
3
Evaluation of liquid yeast-derived sucrase enzyme replacement in patients with sucrase-isomaltase deficiency.蔗糖酶-异麦芽糖酶缺乏症患者中液体酵母衍生蔗糖酶的酶替代治疗评估。
Gastroenterology. 1993 Oct;105(4):1061-8. doi: 10.1016/0016-5085(93)90950-h.
4
[Primary saccharose-isomaltose deficit: a 20-year case load].原发性蔗糖-异麦芽糖缺乏症:20年病例数
An Esp Pediatr. 1990 Apr;32(4):317-20.
5
Sacrosidase therapy for congenital sucrase-isomaltase deficiency.用于先天性蔗糖酶-异麦芽糖酶缺乏症的蔗糖酶治疗
J Pediatr Gastroenterol Nutr. 1999 Feb;28(2):137-42. doi: 10.1097/00005176-199902000-00008.
6
[Congenital sucrase-isomaltase deficiency: a rare cause of chronic or recurrent diarrhea in children].先天性蔗糖酶-异麦芽糖酶缺乏症:儿童慢性或复发性腹泻的罕见病因
Rev Med Suisse Romande. 1980 May;100(5):441-2.
7
[Peripheral gangrene in a congenital sucrase isomaltase deficiency (author's transl)].先天性蔗糖酶异麦芽糖酶缺乏症中的周围性坏疽(作者译)
An Esp Pediatr. 1980 Aug;13(8):720-5.
8
Maltase-glucoamylase and residual isomaltase in sucrose intolerant patients.蔗糖不耐受患者中的麦芽糖酶-葡萄糖淀粉酶和残余异麦芽糖酶
J Pediatr Gastroenterol Nutr. 1986 May-Jun;5(3):365-71. doi: 10.1097/00005176-198605000-00005.
9
[Congenital saccharase-isomaltase defect--diagnostic difficulties].[先天性蔗糖酶-异麦芽糖酶缺陷——诊断困难]
Orv Hetil. 1989 Nov 26;130(48):2577-82.
10
Sucrase-isomaltase deficiency in humans. Different mutations disrupt intracellular transport, processing, and function of an intestinal brush border enzyme.人类蔗糖酶-异麦芽糖酶缺乏症。不同的突变会破坏一种肠刷状缘酶的细胞内运输、加工和功能。
J Clin Invest. 1988 Aug;82(2):667-79. doi: 10.1172/JCI113646.

引用本文的文献

1
Diet management in congenital diarrheas and enteropathies - general concepts and disease-specific approach, a narrative review.先天性腹泻和肠病的饮食管理 - 一般概念和疾病特异性方法,叙述性综述。
Am J Clin Nutr. 2024 Jul;120(1):17-33. doi: 10.1016/j.ajcnut.2024.05.004. Epub 2024 May 9.
2
From Birth and Throughout Life: Fungal Microbiota in Nutrition and Metabolic Health.从出生到生命全程:营养和代谢健康中的真菌微生物组。
Annu Rev Nutr. 2020 Sep 23;40:323-343. doi: 10.1146/annurev-nutr-013120-043659. Epub 2020 Jul 17.
3
: What Makes It Tick as Successful Probiotic?
是什么使其成为成功的益生菌?
J Fungi (Basel). 2020 Jun 4;6(2):78. doi: 10.3390/jof6020078.
4
Food Intolerances.食物不耐受。
Nutrients. 2019 Jul 22;11(7):1684. doi: 10.3390/nu11071684.
5
CNCM I-745 Improves Intestinal Enzyme Function: A Trophic Effects Review.法国国家微生物资源中心保藏编号I-745改善肠道酶功能:一项营养效应综述。
Clin Med Insights Gastroenterol. 2018 Feb 9;11:1179552217752679. doi: 10.1177/1179552217752679. eCollection 2018.
6
A double-blind, randomized, placebo-controlled trial studying the effects of Saccharomyces boulardii on the gastrointestinal tolerability, safety, and pharmacokinetics of miglustat.一项双盲、随机、安慰剂对照试验,研究布拉酵母菌对米格列醇胃肠道耐受性、安全性及药代动力学的影响。
Orphanet J Rare Dis. 2015 Jun 19;10:81. doi: 10.1186/s13023-015-0297-7.
7
Improving the stability and activity of oral therapeutic enzymes-recent advances and perspectives.提高口服治疗酶的稳定性和活性:最新进展和展望。
Pharm Res. 2014 May;31(5):1099-105. doi: 10.1007/s11095-013-1233-y. Epub 2013 Nov 2.
8
13C-breath tests for sucrose digestion in congenital sucrase isomaltase-deficient and sacrosidase-supplemented patients.用于先天性蔗糖酶异麦芽糖酶缺乏症及补充了蔗糖酶患者蔗糖消化的13C呼气试验
J Pediatr Gastroenterol Nutr. 2009 Apr;48(4):412-8. doi: 10.1097/mpg.0b013e318180cd09.
9
The effect of Saccharomyces boulardii on reducing irinotecan-induced intestinal mucositis and diarrhea.布拉酵母菌对减轻伊立替康所致肠道黏膜炎和腹泻的作用。
Med Oncol. 2009;26(3):350-7. doi: 10.1007/s12032-008-9128-1. Epub 2008 Dec 9.
10
Effects of Saccharomyces boulardii on intestinal mucosa.布拉氏酵母菌对肠黏膜的影响。
Dig Dis Sci. 2006 Aug;51(8):1485-92. doi: 10.1007/s10620-005-9016-x. Epub 2006 Jul 13.