WordNet
- impatience with annoyances; "his intolerance of interruptions"
- unwillingness to recognize and respect differences in opinions or beliefs
- a complex carbohydrate found in many plants and used as a sweetening agent (同)saccharose
PrepTutorEJDIC
- 〈U〉(宗教・意見・習慣の相違について)偏狭で認めないこと / 〈U〉(寒暑・痛みなどに)耐えられないこと;〈C〉過敏症
- 蔗糖(しょとう)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/04/10 17:13:25」(JST)
[Wiki en表示]
| Sucrose intolerance |
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| Sucrose |
| Classification and external resources |
| Specialty |
endocrinology |
| ICD-10 |
E74.3 |
| ICD-9-CM |
271.3 |
| OMIM |
222900 |
| DiseasesDB |
29844 |
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[edit on Wikidata]
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Sucrose intolerance, also called congenital sucrase-isomaltase deficiency (CSID),[1] sucrase-isomaltase deficiency,[2] genetic sucrase-isomaltase deficiency (GSID) or sucrase-isomaltase deficiency, is the condition in which sucrase-isomaltase, an enzyme needed for proper metabolism of sucrose (sugar) and starch (i.e., grains and rice), is not produced or the enzyme produced is either partially functional or non-functional in the small intestine. All GSID patients lack fully functional sucrase, while the isomaltase activity can vary from minimal functionality to almost normal activity. The presence of residual isomaltase activity may explain why some GSID patients are better able to tolerate starch in their diet than others with GSID. The highest prevalence rates are seen in the Inuit populations of Greenland (5–10%), Alaska (3–7%) and Canada (about 3%). European descent prevalence ranges from 0.2% to 0.05%. There is a lower prevalence reported in African Americans and Hispanics compared to Caucasians.[3] [4]
Contents
- 1 Overview
- 2 Signs and symptoms
- 3 See also
- 4 References
- 5 External links
Overview
Sucrose (also termed saccharose) is a disaccharide and is a two-sugar chain composed of glucose and fructose which are bonded together. A more familiar name is table, beet, or cane sugar. It was believed that most cases of sucrose intolerance were to do an autosomal recessive, genetic, metabolic disease. Based on new data patients with heterozygous and compound heterozygous genotypes can have symptom presentation as well. GSID involves deficiency in the enzyme sucrase-isomaltase, which breaks apart the glucose and fructose molecules. When disaccharides are consumed, they must be broken down into monosaccharides by enzymes in the intestines before they can be absorbed. Monosaccharides, or single sugar units, are absorbed directly into the blood.[5]
A deficiency of sucrase may result in malabsorption of sugar, which can lead to potentially serious symptoms. Since sucrose-isomaltase is involved in the digestion of starches, some GSID patients may not be able to absorb starches as well. It is important for those with sucrose intolerance to minimize sucrose consumption as much as possible. Dietary supplements or medications may be taken as a substitute for the enzyme missing or to introduce healthy bacteria into the immune system.
Sucrose intolerance can be caused by genetic mutations in which both parents must contain this gene for the child to carry the disease (so-called primary sucrose intolerance). Sucrose intolerance can also be caused by irritable bowel syndrome, aging, or small intestine disease (secondary sucrose intolerance). There are specific tests used to help determine if a person has sucrose intolerance. The most accurate test is the enzyme activity determination, which is done by biopsying the small intestine. This test is a diagnostic for GSID. Other tests which can aid in the diagnosis of GSID but which are not truly diagnostic for the disease are the sucrose breath test, and a genetic test which tests for the absence of certain genes which are thought to be responsible for GSID.[6]
Signs and symptoms
- Abdominal cramps and bloating
- Diarrhea and constipation
- Vomiting
- Hypoglycemia and headaches
- Poor weight gain and growth
- Upper respiratory tract and viral diseases
- Anxiety and heart palpitations
- Excess gas production
See also
- Fructose malabsorption
- Gastroenterology
- Lactose intolerance
References
- ^ Sander P, Alfalah M, Keiser M, et al. (January 2006). "Novel mutations in the human sucrase-isomaltase (SI) gene that cause congenital carbohydrate malabsorption". Hum. Mutat. 27 (1): 119. doi:10.1002/humu.9392. PMID 16329100.
- ^ Baudon JJ, Veinberg F, Thioulouse E, Morgant G, Aymard P, Charritat JL (April 1996). "Sucrase-isomaltase deficiency: changing pattern over two decades". J. Pediatr. Gastroenterol. Nutr. 22 (3): 284–8. doi:10.1097/00005176-199604000-00010. PMID 8708882.
- ^ Treem, William R. (2012). "Clinical Aspects and Treatment of Congenital Sucrase-Isomaltase Deficiency". Journal of Pediatric Gastroenterology and Nutrition. 55: S7–S13. doi:10.1097/01.mpg.0000421401.57633.90.
- ^ Uhrich, Stefanie; Wu, Zaining; Huang, Jie-Yu; Scott, C. Ronald (2012). "Four Mutations in the SI Gene Are Responsible for the Majority of Clinical Symptoms of CSID". Journal of Pediatric Gastroenterology and Nutrition. 55: S34–S35. doi:10.1097/01.mpg.0000421408.65257.b5.
- ^ Treem, William R. (2012). "Clinical Aspects and Treatment of Congenital Sucrase-Isomaltase Deficiency". Journal of Pediatric Gastroenterology and Nutrition. 55: S7–S13. doi:10.1097/01.mpg.0000421401.57633.90.
- ^ Uhrich, Stefanie; Wu, Zaining; Huang, Jie-Yu; Scott, C. Ronald (2012). "Four Mutations in the SI Gene Are Responsible for the Majority of Clinical Symptoms of CSID". Journal of Pediatric Gastroenterology and Nutrition. 55: S34–S35. doi:10.1097/01.mpg.0000421408.65257.b5.
External links
http://CSIDINFO.com
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Inborn error of carbohydrate metabolism: monosaccharide metabolism disorders (including glycogen storage diseases) (E73–E74, 271)
|
|
Sucrose, transport
(extracellular) |
| Disaccharide catabolism |
- Congenital alactasia
- Sucrose intolerance
|
|
| Monosaccharide transport |
- Glucose-galactose malabsorption
- Inborn errors of renal tubular transport (Renal glycosuria)
- Fructose malabsorption
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|
|
| Hexose → glucose |
| Monosaccharide catabolism |
| fructose: |
- Essential fructosuria
- Fructose intolerance
|
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| galactose/galactosemia: |
- GALK deficiency
- GALT deficiency/GALE deficiency
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|
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| Glucose ⇄ glycogen |
| Glycogenesis |
- GSD type 0, glycogen synthase
- GSD type IV, Andersen's, branching
- Adult polyglucosan body disease
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| Glycogenolysis |
| extralysosomal: |
- GSD type V, McArdle, muscle glycogen phosphorylase/GSD type VI, Hers', liver glycogen phosphorylase
- GSD type III, Cori's, debranching
|
|
- lysosomal/LSD: GSD type II, Pompe's, glucosidase
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|
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| Glucose ⇄ CAC |
| Glycolysis |
- MODY 2/HHF3
- GSD type VII, Tarui's, phosphofructokinase
- Triosephosphate isomerase deficiency
- Pyruvate kinase deficiency
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| Gluconeogenesis |
- PCD
- Fructose bisphosphatase deficiency
- GSD type I, von Gierke, glucose 6-phosphatase
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|
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| Pentose phosphate pathway |
- Glucose-6-phosphate dehydrogenase deficiency
- Transaldolase deficiency
- 6-phosphogluconate dehydrogenase deficiency
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| Other |
- Hyperoxaluria
- Pentosuria
- Aldolase A deficiency
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Indomethacin Treatment Prevents High Fat Diet-induced Obesity and Insulin Resistance but Not Glucose Intolerance in C57BL/6J Mice.
- Fjære E1, Aune UL1, Røen K2, Keenan AH3, Ma T2, Borkowski K2, Kristensen DM4, Novotny GW5, Mandrup-Poulsen T6, Hudson BD7, Milligan G7, Xi Y8, Newman JW9, Haj FG10, Liaset B11, Kristiansen K12, Madsen L13.
- The Journal of biological chemistry.J Biol Chem.2014 Jun 6;289(23):16032-45. doi: 10.1074/jbc.M113.525220. Epub 2014 Apr 17.
- Chronic low grade inflammation is closely linked to obesity-associated insulin resistance. To examine how administration of the anti-inflammatory compound indomethacin, a general cyclooxygenase inhibitor, affected obesity development and insulin sensitivity, we fed obesity-prone male C57BL/6J mice a
- PMID 24742673
- Plasminogen activator inhibitor-1 deficiency ameliorates insulin resistance and hyperlipidemia but not bone loss in obese female mice.
- Tamura Y1, Kawao N, Yano M, Okada K, Matsuo O, Kaji H.
- Endocrinology.Endocrinology.2014 May;155(5):1708-17. doi: 10.1210/en.2013-1888. Epub 2014 Feb 28.
- We previously demonstrated that plasminogen activator inhibitor-1 (PAI-1), an inhibitor of fibrinolysis, is involved in type 1 diabetic bone loss in female mice. PAI-1 is well known as an adipogenic factor induced by obesity. We therefore examined the effects of PAI-1 deficiency on bone and glucose
- PMID 24605827
- Congenital diseases of the gastrointestinal tract.
- Lentze M.
- Georgian medical news.Georgian Med News.2014 May;(230):46-53.
- With the rapid increase in knowledge on the genetic origin of diseases within the gastrointestinal tract the number of congenital diseases, which already manifest during childhood have drastically increased. Due to the large application of molecular genetics the number is steadily increasing. To mak
- PMID 24940857
Japanese Journal
- Long-term hypertriglyceridemia and glucose intolerance in rats fed chronically an isocaloric sucrose-rich diet
- 抗高血圧薬Budralazineの実験的糖尿病合併高血圧ラットにおける糖および脂質代謝に及ぼす影響
- 白崎 康文,増村 秀三,明石 章
- 日本薬理学雑誌 87(3), 301-311, 1986
- … 1) SHR treated with 10% sucrose solution as drinking water for 3 months exhibited an impaired glucose tolerance with higher serum insulin levels and a reduction of ΣΔIRI/ΣΔBS. … Streptozotocin (30 mg/kg, i.v.)-treated SHR had a glucose intolerance with lower serum insulin levels and a reduction of ΣΔIRI/ΣΔBS, suggesting a similarity to hypertensive states with insulin-dependent diabetes (IDD) in humans. …
- NAID 130000760492
- 菅原 利夫,幸 雅樹,加納 康行,大川 剛生,別所 正明,西川 典良,中村 浩,黒沢 治彦,森 悦秀,黒井 正人,北 松子,作田 正義
- 日本口腔外科学会雑誌 30(10), 1634-1643, 1984
- … Ensure Liquid, an enteral nutrient, does not contain lactose in order to prevent diarrhea clue to lactose intolerance. … Further, by the use of solid corn syrup and sucrose, the product is synthesized to have a low osmotic pressure so that it can prevent the appearance of hypertonic diarrhea. …
- NAID 130001358459
Related Links
- Definition of Sucrose intolerance in the Medical Dictionary. Sucrose intolerance explanation. Information about Sucrose intolerance in Free online English dictionary. What is Sucrose intolerance? Meaning of Sucrose intolerance ...
- The condition may also be referred as: Congenital Sucrase-Isomaltase Deficiency (CSID) Disaccharide Intolerance Congenital Sucrose Intolerance... ... We created Allergy Mate because we know what it's like to share life with food ...
- Definition of sucrose intolerance – Our online dictionary has sucrose intolerance information from A Dictionary of Food and Nutrition dictionary. Encyclopedia.com: English, psychology and medical dictionaries Search over 100 ...
Related Pictures
★リンクテーブル★
[★]
- 英
- sucrose intolerance
- 関
- ショ糖不耐症、蔗糖不耐症
[★]
- 英
- sucrose intolerance
- 関
- 糖不耐性、ショ糖不耐症
[★]
- 英
- sucrose intolerance
- 関
- 糖不耐性、蔗糖不耐症
[★]
ショ糖、スクロース、シュクロース、(製剤)精製白糖
- 関
- cane sugar、purified sucrose、saccharose