• 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

一种用于治疗糖原贮积病的新型淀粉。

A novel starch for the treatment of glycogen storage diseases.

作者信息

Bhattacharya K, Orton R C, Qi X, Mundy H, Morley D W, Champion M P, Eaton S, Tester R F, Lee P J

机构信息

Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK.

出版信息

J Inherit Metab Dis. 2007 Jun;30(3):350-7. doi: 10.1007/s10545-007-0479-0. Epub 2007 May 19.

DOI:10.1007/s10545-007-0479-0
PMID:17514432
Abstract

OBJECTIVE

To determine whether a new starch offers better short-term metabolic control than uncooked cornstarch in patients with glycogen storage diseases (GSDs).

STUDY DESIGN

A short-term double-blind cross-over pilot study comparing uncooked physically modified cornstarch (WMHM20) with uncooked cornstarch in patients with GSD types Ia, Ib and III. Twenty-one patients (ages 3-47, 9 female) were given 2 g/kg cornstarch or WMHM20 mixed in water. Blood glucose, lactate and insulin, and breath hydrogen and (13)CO2 enrichment were measured, at baseline and after each load. The hourly biochemical evaluations terminated when blood glucose was < or = 3.0 mmol/L, when the study period had lasted 10 h or when the patient wished to end the test. The alternative starch was administered under similar trial conditions a median of 10 days later.

RESULTS

The median starch load duration was 9 h for WMHM20 versus 7 h for cornstarch. Glucose decreased more slowly (p = 0.05) and lactate was suppressed faster (p = 0.17) for WMHM20 compared with cornstarch. Peak hydrogen excretion was increased (p = 0.05) when cornstarch was taken.

CONCLUSION

These data indicate longer duration of euglycaemia and better short-term metabolic control in the majority of GSD patients with WMHM20 compared to cornstarch.

摘要

目的

确定一种新型淀粉在糖原贮积病(GSD)患者中是否比生玉米淀粉能提供更好的短期代谢控制。

研究设计

一项短期双盲交叉试验性研究,比较生的物理改性玉米淀粉(WMHM20)与生玉米淀粉在Ia型、Ib型和III型GSD患者中的效果。21名患者(年龄3 - 47岁,9名女性)服用2 g/kg混入水中的玉米淀粉或WMHM20。在基线期及每次负荷后测量血糖、乳酸和胰岛素,以及呼气氢气和(13)CO2富集情况。当血糖≤3.0 mmol/L、研究期持续10小时或患者希望结束试验时,终止每小时的生化评估。在类似试验条件下,10天后中位数时间给予另一种淀粉。

结果

WMHM20的淀粉负荷持续时间中位数为9小时,而玉米淀粉为7小时。与玉米淀粉相比,WMHM20的葡萄糖下降更慢(p = 0.05),乳酸抑制更快(p = 0.17)。服用玉米淀粉时,氢气排泄峰值增加(p = 0.05)。

结论

这些数据表明,与玉米淀粉相比,WMHM20在大多数GSD患者中能使血糖正常的持续时间更长,短期代谢控制更好。

相似文献

1
A novel starch for the treatment of glycogen storage diseases.一种用于治疗糖原贮积病的新型淀粉。
J Inherit Metab Dis. 2007 Jun;30(3):350-7. doi: 10.1007/s10545-007-0479-0. Epub 2007 May 19.
2
Use of modified cornstarch therapy to extend fasting in glycogen storage disease types Ia and Ib.使用改良玉米淀粉疗法延长 Ia 型和 Ib 型糖原贮积病的禁食时间。
Am J Clin Nutr. 2008 Nov;88(5):1272-6. doi: 10.3945/ajcn.2008.26352.
3
Impact of hematopoietic stem cell transplantation in glycogen storage disease type Ib: A single-subject research design using C-glucose breath test.造血干细胞移植对Ⅰb型糖原贮积病的影响:一项采用¹³C-葡萄糖呼气试验的单病例研究设计
Mol Genet Metab Rep. 2023 Jan 3;34:100955. doi: 10.1016/j.ymgmr.2023.100955. eCollection 2023 Mar.
4
Sleep and quality of life of patients with glycogen storage disease on standard and modified uncooked cornstarch.糖原贮积病患者在标准和改良生玉米淀粉治疗下的睡眠和生活质量。
Mol Genet Metab. 2018 Mar;123(3):326-330. doi: 10.1016/j.ymgme.2017.09.003. Epub 2017 Sep 11.
5
A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study.一项关于糯玉米热改性淀粉用于治疗成人Ⅰ型糖原贮积病的前瞻性纵向研究:一项随机双盲交叉研究。
Orphanet J Rare Dis. 2015 Feb 15;10:18. doi: 10.1186/s13023-015-0229-6.
6
Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study.延长释放型玉米淀粉在肝糖原贮积病中的短期和长期可接受性和疗效:Glyde 研究结果。
Orphanet J Rare Dis. 2024 Jul 9;19(1):258. doi: 10.1186/s13023-024-03274-y.
7
Potential use of other starch sources in the treatment of glycogen storage disease type Ia - an in vitro study.其他淀粉源在治疗糖原贮积病 Ia 型中的潜在应用——一项体外研究。
Orphanet J Rare Dis. 2024 Jul 30;19(1):283. doi: 10.1186/s13023-024-03201-1.
8
Development of minimally invasive C-glucose breath test to examine different exogenous carbohydrate sources in patients with glycogen storage disease type Ia.开发微创C-葡萄糖呼气试验以检测Ia型糖原贮积病患者的不同外源性碳水化合物来源。
Mol Genet Metab Rep. 2022 May 11;31:100880. doi: 10.1016/j.ymgmr.2022.100880. eCollection 2022 Jun.
9
Effect of dietary interventions in the maintenance of normoglycaemia in glycogen storage disease type 1a: a systematic review and meta-analysis.饮食干预对 1a 型糖原贮积病患者血糖维持正常的效果:系统评价和荟萃分析。
J Hum Nutr Diet. 2013 Aug;26(4):329-39. doi: 10.1111/jhn.12030. Epub 2013 Jan 7.
10
[Type I glycogenosis: extending therapy with uncooked cornstarch].
Monatsschr Kinderheilkd. 1986 Mar;134(3):142-5.

引用本文的文献

1
Oral Cornstarch and Glycyrrhizin Improve Severe Liver Injury Caused by Glycogen Storage Disease Type IXa.口服玉米淀粉和甘草甜素可改善IXa型糖原贮积病所致的严重肝损伤。
Cureus. 2025 Feb 2;17(2):e78396. doi: 10.7759/cureus.78396. eCollection 2025 Feb.
2
Potential use of other starch sources in the treatment of glycogen storage disease type Ia - an in vitro study.其他淀粉源在治疗糖原贮积病 Ia 型中的潜在应用——一项体外研究。
Orphanet J Rare Dis. 2024 Jul 30;19(1):283. doi: 10.1186/s13023-024-03201-1.
3
Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study.

本文引用的文献

1
Hydrogen breath test with low-dose rice flour for assessment of exocrine pancreatic insufficiency.低剂量米粉氢呼气试验用于评估外分泌性胰腺功能不全
Pancreas. 2004 Nov;29(4):306-10. doi: 10.1097/00006676-200411000-00010.
2
THE EFFECT OF PORTACAVAL TRANSPOSITION ON CARBOHYDRATE METABOLISM: EXPERIMENTAL AND CLINICAL OBSERVATIONS.门腔静脉转位对碳水化合物代谢的影响:实验与临床观察
Surgery. 1965 May;57:687-97.
3
Glucose-6-phosphatase of the liver in glycogen storage disease.糖原贮积病中肝脏的葡萄糖-6-磷酸酶
延长释放型玉米淀粉在肝糖原贮积病中的短期和长期可接受性和疗效:Glyde 研究结果。
Orphanet J Rare Dis. 2024 Jul 9;19(1):258. doi: 10.1186/s13023-024-03274-y.
4
Intestinal microbiota composition of children with glycogen storage Type I patients.糖原贮积症 I 型患儿的肠道微生物组成。
Eur J Clin Nutr. 2024 May;78(5):407-412. doi: 10.1038/s41430-024-01412-0. Epub 2024 Feb 24.
5
French recommendations for the management of glycogen storage disease type III.法国关于 III 型糖原贮积症管理的建议。
Eur J Med Res. 2023 Jul 24;28(1):253. doi: 10.1186/s40001-023-01212-5.
6
Impact of hematopoietic stem cell transplantation in glycogen storage disease type Ib: A single-subject research design using C-glucose breath test.造血干细胞移植对Ⅰb型糖原贮积病的影响:一项采用¹³C-葡萄糖呼气试验的单病例研究设计
Mol Genet Metab Rep. 2023 Jan 3;34:100955. doi: 10.1016/j.ymgmr.2023.100955. eCollection 2023 Mar.
7
Cellular Therapies in Pediatric Liver Diseases.儿童肝脏疾病的细胞治疗。
Cells. 2022 Aug 10;11(16):2483. doi: 10.3390/cells11162483.
8
Dynamic Methods for Childhood Hypoglycemia Phenotyping: A Narrative Review.动态方法用于儿童低血糖表型分析:叙述性综述。
Front Endocrinol (Lausanne). 2022 Jun 17;13:858832. doi: 10.3389/fendo.2022.858832. eCollection 2022.
9
Facial Dysmorphic Features in a Patient With Nonketotic Hypoglycemia and a Pathogenic Variant in the Gene.一名患有非酮症低血糖且该基因存在致病变异的患者的面部畸形特征
AACE Clin Case Rep. 2021 Nov 27;8(3):109-112. doi: 10.1016/j.aace.2021.11.006. eCollection 2022 May-Jun.
10
Development of minimally invasive C-glucose breath test to examine different exogenous carbohydrate sources in patients with glycogen storage disease type Ia.开发微创C-葡萄糖呼气试验以检测Ia型糖原贮积病患者的不同外源性碳水化合物来源。
Mol Genet Metab Rep. 2022 May 11;31:100880. doi: 10.1016/j.ymgmr.2022.100880. eCollection 2022 Jun.
J Biol Chem. 1952 Dec;199(2):661-7.
4
Utilization of cornstarch in glycogen storage disease type Ia.玉米淀粉在Ia型糖原贮积病中的应用。
Eur J Gastroenterol Hepatol. 2002 Nov;14(11):1251-6. doi: 10.1097/00042737-200211000-00014.
5
Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I).I型糖原贮积病管理指南——欧洲I型糖原贮积病研究(ESGSD I)
Eur J Pediatr. 2002 Oct;161 Suppl 1:S112-9. doi: 10.1007/s00431-002-1016-7. Epub 2002 Aug 24.
6
Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type 1a glycogen storage disease.生玉米淀粉持续葡萄糖治疗对1a型糖原贮积病长期临床病程的影响。
Eur J Pediatr. 2002 Oct;161 Suppl 1:S35-9. doi: 10.1007/s00431-002-1000-2. Epub 2002 Jul 31.
7
Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European Study on Glycogen Storage Disease Type I (ESGSD I).I型糖原贮积病:诊断、管理、临床病程及预后。欧洲I型糖原贮积病研究(ESGSD I)结果
Eur J Pediatr. 2002 Oct;161 Suppl 1:S20-34. doi: 10.1007/s00431-002-0999-4. Epub 2002 Aug 22.
8
Historical highlights and unsolved problems in glycogen storage disease type 1.1型糖原贮积病的历史亮点与未解问题
Eur J Pediatr. 2002 Oct;161 Suppl 1:S2-9. doi: 10.1007/s00431-002-0997-6. Epub 2002 Aug 23.
9
Intestinal function in glycogen storage disease type I.Ⅰ型糖原贮积病中的肠道功能
J Inherit Metab Dis. 2002 Aug;25(4):261-7. doi: 10.1023/a:1016572706488.
10
Effect of continuous glucose therapy begun in infancy on the long-term clinical course of patients with type I glycogen storage disease.婴儿期开始的持续葡萄糖治疗对I型糖原贮积病患者长期临床病程的影响。
J Pediatr Gastroenterol Nutr. 1999 Aug;29(2):136-43. doi: 10.1097/00005176-199908000-00008.