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Sucrose intolerance

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183:, 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. 82: 290:
person has CSID and there are little or no working sucrase-isomaltase enzymes in the intestines, greater than normal levels of hydrogen and/or methane are generated and exhaled in the breath. However, there may be other reasons why the person is exhaling excess hydrogen and/or methane gas, such as an overgrowth of bacteria in the small intestine, called small intestinal bacterial overgrowth (SIBO).
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this breath test, the exhaled breath is collected in sealed test tubes at 30-minute intervals over a 90-minute period after drinking the sugar-water solution. If the cumulative amount of 13CO2 exhaled is below the normal level that occurs when sucrose is digested by sucrase, the person may have CSID and the person should undergo further examination by their doctor.
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been investigated for their ability to cause CSID. Therefore, a negative genetic test only means that the person does not carry one of the 37 SI gene variations that are known to be associated with CSID, but they may have an SI gene variation that has not yet been identified as one that can cause CSID.
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A positive genetic test for one or more of the 37 SI gene variations known to cause CSID can help confirm a diagnosis of CSID. However, a negative genetic test cannot rule out a diagnosis of CSID. There are more than 2,000 different variations of the SI gene, and many of these variations have not yet
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Infants may not show symptoms of CSID until they begin to eat sucrose- and starch-containing foods such as juices, solid foods, and medications sweetened with sucrose. Chronic, watery diarrhea and failure to thrive are the most common symptoms in infants and toddlers. Other symptoms include abdominal
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The findings from a 13C-breath test are believed to be more definitive for CSID than the hydrogen/methane breath test although neither test is validated to provide, by themselves, a diagnosis for CSID. In both breath tests, the consumption of sugar may cause severe gastrointestinal symptoms in those
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The timing of gastrointestinal symptoms associated with CSID is distinctive. CSID symptoms are frequent, daily events; they are lifelong, and they are postprandial (occurring after eating food). These symptoms can range from mild to severe and include chronic, watery, acidic diarrhea; intestinal gas
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It is also advised that the patient work with a registered dietitian or nutritionist who can help identify the foods that can be tolerated and those that cannot be tolerated. A registered dietitian or nutritionist can also help the patient plan a diet that will meet the nutritional needs for normal
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A relatively new, noninvasive test that shows promise is a genetic test for the gene SI, which codes for the enzyme sucrase-isomaltase. This test requires a swab of tissue from the inside of the cheek. So far, 37 aberrant variations of the SI gene have been found in patients who have been diagnosed
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The second breath test is called the carbon-13 (13C) breath test. Carbon-13 is a stable isotope of carbon that occurs naturally in sucrose, making it possible to track a person's ability to digest and absorb sucrose by measuring the amount of 13CO2 exhaled after drinking a sugar-water solution. In
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While the breath tests and genetic test have not been validated to be diagnostic for CSID, they may become important tests that help in identifying patients with CSID. As with all diagnoses, a diagnosis of CSID depends on combining a doctor's clinical findings with results from objective tests. A
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The first of these is the hydrogen/methane breath test, which measures the amount of hydrogen and methane gases a person exhales after consuming sugary water. The exhaled breath is collected in sealed test tubes at 30-minute intervals over a three-hour period after drinking the sugary water. If a
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A deficiency or absence of sucrase-isomaltase function is likely to cause chronic gastrointestinal symptoms whenever a person eats food containing sucrose or starch sugars, which are very common in carbohydrates. In fact, the sucrase-isomaltase enzyme is responsible for the digestion of all foods
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In addition to CSID, a primary gastrointestinal disorder such as a gastrointestinal infection, celiac disease or Crohn's disease, can transiently suppress the digestive function of sucrase-isomaltase, causing an acquired form of sucrase-isomaltase deficiency (SID). Once the underlying disorder is
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This diagnostic method, called a disaccharidase assay, is conducted on tissue samples taken from the small intestine during an endoscopic procedure, also called an upper GI (gastrointestinal) examination. If the level of sucrase activity is below the level considered necessary for normal sucrase
118:. 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. 278:
Determining the cause of chronic gastrointestinal symptoms may take a long time because the symptoms can be common to many gastrointestinal conditions. One diagnostic method, considered the gold standard for diagnosing CSID, measures the level of activity of four intestinal enzymes that digest
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of sugar, which can lead to potentially serious symptoms. Since sucrase-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.
222:, which breaks the bond between 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. 355:
For these reasons, a restriction diet needs to be specific for each patient who has CSID. Before making any changes to the diet, it is important for a patient to speak with a healthcare provider, especially if the patient is underweight or not gaining weight as would be expected.
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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.
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If it is clinically inappropriate or difficult to perform a biopsy or if a CSID diagnosis is in doubt, a physician may suggest a two-week therapeutic trial with an enzyme replacement. If the patient shows a reduction of symptoms it is considered diagnostic for CSID.
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Adults with CSID are usually lean, with a low body-mass index and an aversion to eating carbohydrates and "sweets". Because CSID is an inherited condition, patients with CSID often have close relatives who also experience chronic diarrhea.
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containing sucrose and approximately 60% to 80% of all foods containing starch sugars. When sucrose or starch sugars are not absorbed from the gastrointestinal tract, they travel to the large intestine (colon) where two things happen:
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Chronic gastrointestinal symptoms that are fairly common but difficult to diagnose may be caused by congenital sucrase-isomaltase deficiency (CSID). CSID is an inherited condition characterized by a dysfunctional digestive enzyme.
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The purpose of the digestive enzyme, sucrase-isomaltase, is to break down the compound sugars sucrose (table sugar) and starch sugars so they will be small enough to be absorbed from the gastrointestinal tract.
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The food is broken down by the normal bacteria that reside in the colon by a process called fermentation; byproducts of all fermentation include the production of excess gas and an acidic environment.
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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
1250:"Relationships among dietary intakes and persistent gastrointestinal symptoms in patients receiving enzyme treatment for genetic sucrase-isomaltase deficiency" 338:
The level of ability to digest sucrose or starch sugars is unique to each person who is living with CSID and depends on many factors, including the following:
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Noninvasive diagnostic methods include two breath tests, which can be useful screening tools but are not specific enough for a confirmed diagnosis of CSID.
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The use of enzyme replacement therapy to replace the action of the sucrase enzyme that is deficient. This medication requires a prescription from a doctor.
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which are bonded together. A more familiar name is table, beet, or cane sugar. It was believed that most cases of sucrose intolerance were due to an
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compound sugars, also called disaccharides. The four disaccharides digested in the small intestine are lactose, sucrose, maltose, and isomaltose.
1051:"Altered folding, turnover, and polarized sorting act in concert to define a novel pathomechanism of congenital sucrase-isomaltase deficiency" 1828: 923: 310:
physical examination, medical history, and other secondary tests, such as stool pH test for acidic stool, can aid in the diagnosis of CSID.
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distention, gassiness, colic, irritability, excoriated buttocks, severe diaper rash due to acidic diarrhea, indigestion, and vomiting.
3199: 2041: 1448: 3043: 2713: 2560: 1098:"Congenital sucrase-isomaltase deficiency: heterogeneity of inheritance, trafficking, and function of an intestinal enzyme complex" 114:(sugar) and starch (e.g., grains), is not produced or the enzyme produced is either partially functional or non-functional in the 3093: 3061: 3049: 2998: 2458: 3204: 3194: 3055: 3004: 2992: 2919: 2839: 2599: 2211: 2194: 1699: 1694: 1571: 961:"Compound heterozygous mutations affect protein folding and function in patients with congenital sucrase-isomaltase deficiency" 1724: 3148: 2953: 2453: 2409: 2303: 2125: 2664: 234:
or medications may be taken as a substitute for the missing enzyme or to introduce healthy bacteria into the immune system.
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who have CSID. For this reason, these breath tests should be conducted under the supervision of a healthcare professional.
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Diet modification that eliminates or restricts the consumption of foods containing sucrose (table sugar) or starch sugars
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growth and development. Vitamins, minerals, and additional supplements may be needed to meet all nutritional needs.
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Pelton N, Tran C, Leo A (2009). "The reproducibility of the 13C-sucrose breath test in children and adults".
2865: 2543: 2320: 2061: 1853: 1512: 451:"Novel mutations in the human sucrase-isomaltase (SI) gene that cause congenital carbohydrate malabsorption" 148: 3308: 2854: 2623: 2347: 2330: 1740: 1714: 1590: 1541: 1502: 1480: 1472: 598: 596: 594: 592: 378: 780:"Reference intervals for intestinal disaccharidase activities determined from a non-reference population" 3264: 3164: 2914: 2555: 2463: 2424: 2352: 212: 2474: 1655: 1146: 960: 2594: 2538: 2359: 2255: 1838: 1833: 1667: 1585: 1551: 881: 2815: 2688: 2498: 2391: 2374: 2335: 2308: 2260: 2103: 2080: 2073: 2022: 1994: 1916: 1911: 1906: 1823: 1390: 824:"The role of disaccharidase deficiencies in functional abdominal pain disorders—a narrative review" 388: 204: 81: 3067: 3015: 2700: 2565: 2548: 2533: 2520: 2503: 2369: 1896: 1868: 1628: 1524: 1489: 1320: 1127: 753: 528: 231: 219: 99: 2004: 1802: 660: 658: 656: 654: 652: 650: 3122: 2678: 2659: 2032: 1423: 1312: 1271: 1212: 1168: 1119: 1075: 1031: 982: 901: 855: 804: 745: 698: 636: 578: 520: 472: 431: 70: 875: 873: 871: 869: 499:"Four Mutations in the SI Gene Are Responsible for the Majority of Clinical Symptoms of CSID" 2859: 2848: 2725: 2645: 2640: 2630: 2492: 2364: 2292: 2110: 2017: 2012: 1942: 1901: 1709: 1674: 1302: 1261: 1202: 1158: 1109: 1065: 1021: 1013: 972: 893: 845: 835: 794: 737: 688: 678: 626: 616: 568: 510: 462: 421: 176: 3186: 2683: 2613: 2479: 2414: 2379: 2250: 2115: 2051: 1953: 1872: 1863: 1813: 1753: 1638: 1615: 115: 1440: 1230: 211:
and compound heterozygous genotypes can have symptom presentation as well. GSID involves
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Baudon JJ, Veinberg F, Thioulouse E, Morgant G, Aymard P, Charritat JL (April 1996).
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The food draws in excess water by a process called osmosis, creating watery diarrhea.
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The two ways to manage the gastrointestinal symptoms associated with CSID are:
55: 3271: 3131: 2484: 2157: 2135: 1780: 1719: 1380: 897: 683: 621: 107: 799: 550: 548: 546: 544: 542: 2930: 2434: 2404: 2267: 2245: 2098: 1949: 1923: 1785: 1775: 1662: 1623: 1556: 1291:"Clinical Aspects and Treatment of Congenital Sucrase-Isomaltase Deficiency" 723:"Clinical aspects and treatment of congenital sucrase-isomaltase deficiency" 1316: 1275: 1216: 1207: 1172: 1123: 1079: 1070: 1035: 986: 905: 859: 808: 749: 702: 640: 524: 476: 342:
The individual level of function of the digestive enzyme sucrase-isomaltase
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Uhrich, Stefanie; Wu, Zaining; Huang, Jie-Yu; Scott, C. Ronald (2012).
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How well the other functions of the gastrointestinal tract are working
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Ritz V, Alfalah M, Zimmer KP, Schmitz J, Jacob R, Naim HY (2003).
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Keiser M, Alfalah M, Pröpsting MJ, Castelletti D, Naim HY (2006).
410:"Sucrase-isomaltase deficiency: changing pattern over two decades" 351:
If a person has any other health issues that may affect digestion
3175: 1406: 2744: 1444: 207:, genetic, metabolic disease. Based on new data patients with 1186:
Spodsberg N, Jacob R, Alfalah M, Zimmer KP, Naim HY (2001).
667:"The clinical consequences of sucrase-isomaltase deficiency" 3096:(Pompe's disease, glucosidase deficiency, formerly GSD-IIa) 449:
Sander P, Alfalah M, Keiser M, et al. (January 2006).
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treated and resolved, the symptoms of SID usually go away.
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Robayo-Torres CC, Quezada-Calvillo R, Nichols BL (2006).
3207:(von Gierke's disease, glucose 6-phosphatase deficiency) 3052:(Hers' disease, liver glycogen phosphorylase deficiency) 959:
Alfalah M, Keiser M, Leeb T, Zimmer KP, Naim HY (2009).
882:"Disaccharide digestion: clinical and molecular aspects" 3276:
Fatal congenital nonlysosomal cardiac glycogenosis (
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Journal of Pediatric Gastroenterology and Nutrition
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Journal of Pediatric Gastroenterology and Nutrition
283:function, the patient will be diagnosed with CSID. 69: 26: 21: 3151:(Tarui's disease, phosphofructokinase deficiency) 3001:(Andersen's disease, branching enzyme deficiency) 3058:(McArdle's disease, myophosphorylase deficiency) 3046:(Cori's disease, debranching enzyme deficiency) 1000:Jacob R, Zimmer KP, Schmitz J, Naim HY (2000). 348:How much sucrose and starch sugars are consumed 2756: 1456: 931:QuinTron Instrument Company Inc. Data on File 8: 3228:Glucose-6-phosphate dehydrogenase deficiency 1562:Esophagogastric junction outflow obstruction 3244:6-phosphogluconate dehydrogenase deficiency 3127: 3031: 2980: 2899: 2889: 2802: 2763: 2749: 2741: 2582: 2233: 1749: 1601:Esophageal intramural pseudodiverticulosis 1485: 1463: 1449: 1441: 1354: 1343:"Congenital sucrase-isomaltase deficiency" 787:The Journal of Applied Laboratory Medicine 557:"Congenital sucrase-isomaltase deficiency" 267:and bloating; nausea; and abdominal pain. 80: 54: 18: 3176:Mitochondrial pyruvate carrier deficiency 1306: 1265: 1206: 1162: 1113: 1069: 1025: 976: 849: 839: 798: 692: 682: 630: 620: 572: 514: 466: 425: 3304:Inborn errors of carbohydrate metabolism 2845:Inborn errors of renal tubular transport 492: 490: 488: 486: 35:Congenital sucrase-isomaltase deficiency 400: 778:Hackenmueller SA, Grenache DG (2016). 225:A deficiency of sucrase may result in 1829:Small intestinal bacterial overgrowth 1248:Boney A, Elser HE, Silver HJ (2018). 924:"Breath Tests & Gastroenterology" 195:and is a two-sugar chain composed of 175:Sucrose intolerance can be caused by 96:genetic sucrase-isomaltase deficiency 43:Genetic sucrase-isomaltase deficiency 7: 3155:Triosephosphate isomerase deficiency 3102:(LAMP2 deficiency, formerly GSD-IIb) 1096:Naim HY, Heine M, Zimmer KP (2012). 3169:Phosphoglucose isomerase deficiency 3070:(PGM1-CDG, CDG1T, formerly GSD-XIV) 603:Gericke B, Amir M, Naim HY (2016). 3200:Fructose bisphosphatase deficiency 3172:Phosphoglycerate kinase deficiency 1349:. US National Library of Medicine. 1308:10.1097/01.mpg.0000421401.57633.90 1115:10.1097/01.mpg.0000421402.57633.4b 742:10.1097/01.mpg.0000421401.57633.90 516:10.1097/01.mpg.0000421408.65257.b5 14: 3064:(phosphorylase kinase deficiency) 2714:Spontaneous bacterial peritonitis 2561:Exocrine pancreatic insufficiency 561:J Pediatr Gastroenterol Nutrition 98:(GSID) is the condition in which 3094:Glycogen storage disease type II 2459:Secondary sclerosing cholangitis 574:10.1097/00005176-199507000-00001 427:10.1097/00005176-199604000-00010 3195:Pyruvate carboxylase deficiency 3005:Adult polyglucosan body disease 2840:Glucose-galactose malabsorption 1700:Gastric antral vascular ectasia 1695:Portal hypertensive gastropathy 1572:Gastroesophageal reflux disease 414:J. Pediatr. Gastroenterol. Nutr 2995:(glycogen synthase deficiency) 2454:Primary sclerosing cholangitis 2304:Hepatic veno-occlusive disease 2126:Solitary rectal ulcer syndrome 822:Puertolas MV, Fifi AC (2018). 1: 3068:Phosphoglucomutase deficiency 2511:Sphincter of Oddi dysfunction 948:. Data on File (white paper). 127:Abdominal cramps and bloating 31:Sucrase-isomaltase deficiency 3278:AMP-activated protein kinase 2420:Postcholecystectomy syndrome 1939:Intestinal pseudoobstruction 1547:Esophageal motility disorder 1164:10.1053/j.gastro.2003.09.022 1102:J Pediatr Gastroenterol Nutr 978:10.1053/j.gastro.2008.11.038 145:Poor weight gain and growth 3325: 3240:(Transketolase deficiency) 3160:Pyruvate kinase deficiency 2405:Gallstone / Cholelithiasis 1929:Functional colonic disease 1725:Zollinger–Ellison syndrome 1289:Treem, William R. (2012). 1267:10.1016/j.jand.2017.11.005 886:Clin Gastroenterol Hepatol 730:Pediatr Gastroenterol Nutr 555:Treem, William R. (1995). 3220:Pentose phosphate pathway 2786:glycogen storage diseases 1606:Acute esophageal necrosis 898:10.1016/j.cgh.2005.12.023 684:10.1186/s40348-015-0028-0 622:10.1186/s40348-016-0033-y 384:Digestive system diseases 62: 53: 3233:Transaldolase deficiency 1705:Gastric dumping syndrome 1580:Laryngopharyngeal reflux 1567:Diffuse esophageal spasm 800:10.1373/jalm.2016.020388 181:irritable bowel syndrome 3018:(glycogenin deficiency) 2866:Fanconi-Bickel syndrome 2776:carbohydrate metabolism 2321:Alcoholic liver disease 1854:Bile acid malabsorption 1798:Peptic (duodenal) ulcer 1231:"SI sucrase-isomaltase" 149:Upper respiratory tract 2855:Fructose malabsorption 2331:Hepatic encephalopathy 1715:Buried bumper syndrome 1646:Peptic (gastric) ulcer 1530:Mallory–Weiss syndrome 1473:human digestive system 1347:Genetic Home Reference 1208:10.1074/jbc.c100219200 1071:10.1074/jbc.M513631200 379:Fructose malabsorption 3265:Primary hyperoxaluria 3165:Aldolase A deficiency 2915:Essential fructosuria 2782:metabolism disorders 2556:Pancreatic pseudocyst 2425:Porcelain gallbladder 1537:Zenker's diverticulum 922:Hamilton, LH (1998). 166:Excess gas production 2920:Fructose intolerance 2816:Congenital alactasia 2360:Hepatorenal syndrome 2299:Budd–Chiari syndrome 2256:Autoimmune hepatitis 2081:Intestinal adhesions 1839:Short bowel syndrome 1668:Functional dyspepsia 1586:Esophageal stricture 1552:Nutcracker esophagus 2821:Sucrose intolerance 2499:Choledocholithiasis 2370:Metabolic disorders 2336:Acute liver failure 2309:Portal hypertension 2261:Alcoholic hepatitis 2104:Radiation proctitis 2023:Mesenteric ischemia 1995:Gastroenterocolitis 1824:Blind loop syndrome 1634:MĂ©nĂ©trier's disease 1542:Barrett's esophagus 1108:(suppl 2): S13-20. 389:Lactose intolerance 232:Dietary supplements 205:autosomal recessive 92:Sucrose intolerance 22:Sucrose intolerance 2868:(GLUT2 deficiency) 2862:(GLUT1 deficiency) 2674:Undefined location 2665:Grynfeltt–Lesshaft 2566:Pancreatic fistula 2549:Pancreatic abscess 2504:Biliary dyskinesia 2475:Mirizzi's syndrome 1976:Large and/or small 1656:Dieulafoy's lesion 1525:Boerhaave syndrome 1237:. Broad Institute. 841:10.3390/nu10121835 736:(suppl 2): S7-13. 721:Treem, WR (2012). 665:Cohen, SA (2016). 220:sucrase-isomaltase 122:Signs and symptoms 106:needed for proper 100:sucrase-isomaltase 3291: 3290: 3214: 3213: 3178:(MPC1 deficiency) 3113: 3112: 3109: 3108: 2966: 2965: 2962: 2961: 2875: 2874: 2738: 2737: 2734: 2733: 2574: 2573: 2176: 2175: 2033:Bowel obstruction 1735: 1734: 1438: 1437: 468:10.1002/humu.9392 334:Diet modification 177:genetic mutations 89: 88: 16:Medical condition 3316: 3128: 3087: 3037: 3032: 2981: 2938: 2908: 2900: 2890: 2849:Renal glycosuria 2803: 2765: 2758: 2751: 2742: 2726:Pneumoperitoneum 2583: 2493:Common bile duct 2375:Wilson's disease 2365:Peliosis hepatis 2234: 2111:Proctalgia fugax 2018:Abdominal angina 1943:Ogilvie syndrome 1892:Pseudomembranous 1750: 1710:Gastric volvulus 1675:Pyloric stenosis 1486: 1471:Diseases of the 1465: 1458: 1451: 1442: 1355: 1350: 1329: 1328: 1310: 1286: 1280: 1279: 1269: 1254:J Acad Nutr Diet 1245: 1239: 1238: 1227: 1221: 1220: 1210: 1201:(26): 23506–10. 1192: 1183: 1177: 1176: 1166: 1151:Gastroenterology 1142: 1136: 1135: 1117: 1093: 1084: 1083: 1073: 1055: 1046: 1040: 1039: 1029: 997: 991: 990: 980: 965:Gastroenterology 956: 950: 949: 941: 935: 934: 928: 919: 910: 909: 877: 864: 863: 853: 843: 819: 813: 812: 802: 784: 775: 769: 768: 766: 760:. Archived from 727: 718: 707: 706: 696: 686: 671:Mol Cell Pediatr 662: 645: 644: 634: 624: 609:Mol Cell Pediatr 600: 587: 586: 576: 552: 537: 536: 518: 494: 481: 480: 470: 446: 440: 439: 429: 405: 133:and constipation 85: 84: 58: 19: 3324: 3323: 3319: 3318: 3317: 3315: 3314: 3313: 3294: 3293: 3292: 3287: 3248: 3210: 3187:Gluconeogenesis 3181: 3105: 3078: 3073: 3036:Extralysosomal: 3035: 3021: 2958: 2954:GALE deficiency 2950:GALT deficiency 2945:GALK deficiency 2929: 2924: 2903: 2871: 2860:De Vivo Disease 2825: 2799:(extracellular) 2798: 2789: 2783: 2769: 2739: 2730: 2695: 2684:Internal hernia 2570: 2515: 2480:Biliary fistula 2438: 2429: 2415:Adenomyomatosis 2410:Cholesterolosis 2386: 2380:Hemochromatosis 2251:Viral hepatitis 2223: 2172: 2130: 2116:Rectal prolapse 2085: 2052:Fecal impaction 2042:Intussusception 2005:Crohn's disease 1971: 1954:Toxic megacolon 1866: 1864:Large intestine 1858: 1803:Curling's ulcer 1756: 1754:Small intestine 1743: 1731: 1639:Gastroenteritis 1610: 1475: 1469: 1439: 1434: 1433: 1366: 1341: 1338: 1333: 1332: 1288: 1287: 1283: 1247: 1246: 1242: 1229: 1228: 1224: 1190: 1185: 1184: 1180: 1144: 1143: 1139: 1095: 1094: 1087: 1064:(20): 14393–9. 1053: 1048: 1047: 1043: 1018:10.1172/JCI9677 999: 998: 994: 958: 957: 953: 943: 942: 938: 926: 921: 920: 913: 879: 878: 867: 821: 820: 816: 782: 777: 776: 772: 764: 725: 720: 719: 710: 664: 663: 648: 602: 601: 590: 554: 553: 540: 496: 495: 484: 448: 447: 443: 407: 406: 402: 397: 375: 366: 336: 320: 240: 173: 124: 116:small intestine 79: 17: 12: 11: 5: 3322: 3320: 3312: 3311: 3306: 3296: 3295: 3289: 3288: 3286: 3285: 3274: 3269: 3268: 3267: 3256: 3254: 3250: 3249: 3247: 3246: 3241: 3235: 3230: 3224: 3222: 3216: 3215: 3212: 3211: 3209: 3208: 3202: 3197: 3191: 3189: 3183: 3182: 3180: 3179: 3173: 3170: 3167: 3162: 3157: 3152: 3146: 3136: 3134: 3125: 3115: 3114: 3111: 3110: 3107: 3106: 3104: 3103: 3097: 3090: 3088: 3075: 3074: 3072: 3071: 3065: 3059: 3053: 3047: 3040: 3038: 3029: 3027:Glycogenolysis 3023: 3022: 3020: 3019: 3013: 3011:Lafora disease 3008: 3002: 2996: 2989: 2987: 2978: 2968: 2967: 2964: 2963: 2960: 2959: 2957: 2956: 2947: 2941: 2939: 2926: 2925: 2923: 2922: 2917: 2911: 2909: 2897: 2894:Monosaccharide 2887: 2877: 2876: 2873: 2872: 2870: 2869: 2863: 2857: 2852: 2842: 2836: 2834: 2831:Monosaccharide 2827: 2826: 2824: 2823: 2818: 2812: 2810: 2800: 2791: 2790: 2780:monosaccharide 2770: 2768: 2767: 2760: 2753: 2745: 2736: 2735: 2732: 2731: 2729: 2728: 2723: 2721:Hemoperitoneum 2718: 2717: 2716: 2705: 2703: 2697: 2696: 2694: 2693: 2692: 2691: 2686: 2681: 2670: 2669: 2668: 2667: 2662: 2649: 2648: 2643: 2638: 2633: 2628: 2627: 2626: 2621: 2610: 2609: 2604: 2603: 2602: 2591: 2589: 2580: 2576: 2575: 2572: 2571: 2569: 2568: 2563: 2558: 2553: 2552: 2551: 2546: 2541: 2536: 2525: 2523: 2517: 2516: 2514: 2513: 2508: 2507: 2506: 2501: 2488: 2487: 2482: 2477: 2468: 2467: 2466: 2461: 2456: 2445: 2443: 2431: 2430: 2428: 2427: 2422: 2417: 2412: 2407: 2402: 2396: 2394: 2388: 2387: 2385: 2384: 2383: 2382: 2377: 2367: 2362: 2357: 2356: 2355: 2350: 2340: 2339: 2338: 2333: 2323: 2318: 2317: 2316: 2311: 2306: 2301: 2289: 2288: 2287: 2277: 2276: 2275: 2265: 2264: 2263: 2258: 2253: 2242: 2240: 2231: 2225: 2224: 2222: 2221: 2220: 2219: 2209: 2208: 2207: 2202: 2192: 2190:Blood in stool 2186: 2184: 2178: 2177: 2174: 2173: 2171: 2170: 2165: 2163:Anal dysplasia 2160: 2155: 2150: 2140: 2138: 2132: 2131: 2129: 2128: 2123: 2118: 2113: 2108: 2107: 2106: 2095: 2093: 2087: 2086: 2084: 2083: 2078: 2077: 2076: 2066: 2065: 2064: 2054: 2049: 2044: 2039: 2030: 2028:Angiodysplasia 2025: 2020: 2009: 2008: 2007: 1997: 1992: 1991: 1990: 1979: 1977: 1973: 1972: 1970: 1969: 1963:Diverticulosis 1959:Diverticulitis 1956: 1947: 1946: 1945: 1936: 1926: 1921: 1920: 1919: 1914: 1909: 1904: 1899: 1894: 1884: 1878: 1876: 1860: 1859: 1857: 1856: 1851: 1849:Milroy disease 1846: 1841: 1836: 1831: 1826: 1821: 1819:Tropical sprue 1816: 1807: 1806: 1805: 1795: 1794: 1793: 1788: 1783: 1772: 1770: 1747: 1741:Lower GI tract 1737: 1736: 1733: 1732: 1730: 1729: 1728: 1727: 1717: 1712: 1707: 1702: 1697: 1692: 1687: 1682: 1677: 1672: 1671: 1670: 1660: 1659: 1658: 1653: 1643: 1642: 1641: 1636: 1631: 1620: 1618: 1612: 1611: 1609: 1608: 1603: 1598: 1593: 1588: 1583: 1577: 1576: 1575: 1569: 1564: 1559: 1554: 1544: 1539: 1534: 1533: 1532: 1527: 1517: 1516: 1515: 1510: 1505: 1494: 1492: 1483: 1481:Upper GI tract 1477: 1476: 1470: 1468: 1467: 1460: 1453: 1445: 1436: 1435: 1432: 1431: 1420: 1409: 1398: 1383: 1367: 1362: 1361: 1359: 1358:Classification 1352: 1351: 1337: 1336:External links 1334: 1331: 1330: 1281: 1240: 1222: 1178: 1157:(6): 1678–85. 1137: 1085: 1041: 992: 951: 936: 911: 865: 814: 770: 767:on 2019-03-03. 708: 646: 588: 538: 482: 441: 399: 398: 396: 393: 392: 391: 386: 381: 374: 371: 365: 362: 353: 352: 349: 346: 343: 335: 332: 331: 330: 327: 319: 316: 264: 263: 260: 239: 236: 172: 169: 168: 167: 164: 155: 153:viral diseases 146: 143: 137: 134: 128: 123: 120: 87: 86: 73: 67: 66: 60: 59: 51: 50: 28: 24: 23: 15: 13: 10: 9: 6: 4: 3: 2: 3321: 3310: 3309:Sensitivities 3307: 3305: 3302: 3301: 3299: 3283: 3279: 3275: 3273: 3270: 3266: 3263: 3262: 3261: 3260:Hyperoxaluria 3258: 3257: 3255: 3251: 3245: 3242: 3239: 3236: 3234: 3231: 3229: 3226: 3225: 3223: 3221: 3217: 3206: 3203: 3201: 3198: 3196: 3193: 3192: 3190: 3188: 3184: 3177: 3174: 3171: 3168: 3166: 3163: 3161: 3158: 3156: 3153: 3150: 3147: 3145: 3141: 3138: 3137: 3135: 3133: 3129: 3126: 3124: 3120: 3116: 3101: 3100:Danon disease 3098: 3095: 3092: 3091: 3089: 3085: 3081: 3076: 3069: 3066: 3063: 3060: 3057: 3054: 3051: 3048: 3045: 3042: 3041: 3039: 3033: 3030: 3028: 3024: 3017: 3014: 3012: 3009: 3006: 3003: 3000: 2997: 2994: 2991: 2990: 2988: 2986: 2982: 2979: 2977: 2973: 2969: 2955: 2951: 2948: 2946: 2943: 2942: 2940: 2936: 2932: 2927: 2921: 2918: 2916: 2913: 2912: 2910: 2906: 2901: 2898: 2895: 2891: 2888: 2886: 2882: 2878: 2867: 2864: 2861: 2858: 2856: 2853: 2850: 2846: 2843: 2841: 2838: 2837: 2835: 2832: 2828: 2822: 2819: 2817: 2814: 2813: 2811: 2808: 2804: 2801: 2796: 2792: 2787: 2781: 2777: 2773: 2766: 2761: 2759: 2754: 2752: 2747: 2746: 2743: 2727: 2724: 2722: 2719: 2715: 2712: 2711: 2710: 2707: 2706: 2704: 2702: 2698: 2690: 2687: 2685: 2682: 2680: 2677: 2676: 2675: 2672: 2671: 2666: 2663: 2661: 2658: 2657: 2656: 2655: 2651: 2650: 2647: 2644: 2642: 2639: 2637: 2634: 2632: 2629: 2625: 2622: 2620: 2617: 2616: 2615: 2612: 2611: 2608: 2605: 2601: 2598: 2597: 2596: 2595:Diaphragmatic 2593: 2592: 2590: 2588: 2584: 2581: 2577: 2567: 2564: 2562: 2559: 2557: 2554: 2550: 2547: 2545: 2542: 2540: 2537: 2535: 2532: 2531: 2530: 2527: 2526: 2524: 2522: 2518: 2512: 2509: 2505: 2502: 2500: 2497: 2496: 2495: 2494: 2490: 2489: 2486: 2483: 2481: 2478: 2476: 2472: 2469: 2465: 2462: 2460: 2457: 2455: 2452: 2451: 2450: 2447: 2446: 2444: 2442: 2436: 2432: 2426: 2423: 2421: 2418: 2416: 2413: 2411: 2408: 2406: 2403: 2401: 2400:Cholecystitis 2398: 2397: 2395: 2393: 2389: 2381: 2378: 2376: 2373: 2372: 2371: 2368: 2366: 2363: 2361: 2358: 2354: 2351: 2349: 2346: 2345: 2344: 2343:Liver abscess 2341: 2337: 2334: 2332: 2329: 2328: 2327: 2326:Liver failure 2324: 2322: 2319: 2315: 2312: 2310: 2307: 2305: 2302: 2300: 2297: 2296: 2295: 2294: 2290: 2286: 2283: 2282: 2281: 2278: 2274: 2271: 2270: 2269: 2266: 2262: 2259: 2257: 2254: 2252: 2249: 2248: 2247: 2244: 2243: 2241: 2239: 2235: 2232: 2230: 2226: 2218: 2215: 2214: 2213: 2210: 2206: 2203: 2201: 2198: 2197: 2196: 2193: 2191: 2188: 2187: 2185: 2183: 2179: 2169: 2166: 2164: 2161: 2159: 2156: 2154: 2151: 2149: 2145: 2142: 2141: 2139: 2137: 2133: 2127: 2124: 2122: 2119: 2117: 2114: 2112: 2109: 2105: 2102: 2101: 2100: 2097: 2096: 2094: 2092: 2088: 2082: 2079: 2075: 2072: 2071: 2070: 2067: 2063: 2060: 2059: 2058: 2055: 2053: 2050: 2048: 2045: 2043: 2040: 2038: 2034: 2031: 2029: 2026: 2024: 2021: 2019: 2015: 2014: 2010: 2006: 2003: 2002: 2001: 1998: 1996: 1993: 1989: 1986: 1985: 1984: 1983:Enterocolitis 1981: 1980: 1978: 1974: 1968: 1964: 1960: 1957: 1955: 1951: 1948: 1944: 1940: 1937: 1935: 1932: 1931: 1930: 1927: 1925: 1922: 1918: 1915: 1913: 1910: 1908: 1905: 1903: 1900: 1898: 1895: 1893: 1890: 1889: 1888: 1885: 1883: 1880: 1879: 1877: 1874: 1870: 1865: 1861: 1855: 1852: 1850: 1847: 1845: 1842: 1840: 1837: 1835: 1832: 1830: 1827: 1825: 1822: 1820: 1817: 1815: 1811: 1810:Malabsorption 1808: 1804: 1801: 1800: 1799: 1796: 1792: 1789: 1787: 1784: 1782: 1779: 1778: 1777: 1774: 1773: 1771: 1768: 1764: 1760: 1755: 1751: 1748: 1746: 1742: 1738: 1726: 1723: 1722: 1721: 1718: 1716: 1713: 1711: 1708: 1706: 1703: 1701: 1698: 1696: 1693: 1691: 1688: 1686: 1685:Gastroparesis 1683: 1681: 1678: 1676: 1673: 1669: 1666: 1665: 1664: 1661: 1657: 1654: 1652: 1651:Cushing ulcer 1649: 1648: 1647: 1644: 1640: 1637: 1635: 1632: 1630: 1627: 1626: 1625: 1622: 1621: 1619: 1617: 1613: 1607: 1604: 1602: 1599: 1597: 1596:Megaesophagus 1594: 1592: 1589: 1587: 1584: 1581: 1578: 1573: 1570: 1568: 1565: 1563: 1560: 1558: 1555: 1553: 1550: 1549: 1548: 1545: 1543: 1540: 1538: 1535: 1531: 1528: 1526: 1523: 1522: 1521: 1518: 1514: 1511: 1509: 1506: 1504: 1501: 1500: 1499: 1496: 1495: 1493: 1491: 1487: 1484: 1482: 1478: 1474: 1466: 1461: 1459: 1454: 1452: 1447: 1446: 1443: 1430: 1426: 1425: 1421: 1419: 1415: 1414: 1410: 1408: 1404: 1403: 1399: 1397: 1393: 1392: 1388: 1384: 1382: 1378: 1377: 1373: 1369: 1368: 1365: 1360: 1356: 1348: 1344: 1340: 1339: 1335: 1326: 1322: 1318: 1314: 1309: 1304: 1300: 1296: 1292: 1285: 1282: 1277: 1273: 1268: 1263: 1259: 1255: 1251: 1244: 1241: 1236: 1232: 1226: 1223: 1218: 1214: 1209: 1204: 1200: 1196: 1189: 1182: 1179: 1174: 1170: 1165: 1160: 1156: 1152: 1148: 1141: 1138: 1133: 1129: 1125: 1121: 1116: 1111: 1107: 1103: 1099: 1092: 1090: 1086: 1081: 1077: 1072: 1067: 1063: 1059: 1052: 1045: 1042: 1037: 1033: 1028: 1023: 1019: 1015: 1011: 1007: 1006:J Clin Invest 1003: 996: 993: 988: 984: 979: 974: 971:(3): 883–92. 970: 966: 962: 955: 952: 947: 940: 937: 932: 925: 918: 916: 912: 907: 903: 899: 895: 892:(3): 276–87. 891: 887: 883: 876: 874: 872: 870: 866: 861: 857: 852: 847: 842: 837: 834:(12): E1835. 833: 829: 825: 818: 815: 810: 806: 801: 796: 793:(2): 172–80. 792: 788: 781: 774: 771: 763: 759: 755: 751: 747: 743: 739: 735: 731: 724: 717: 715: 713: 709: 704: 700: 695: 690: 685: 680: 676: 672: 668: 661: 659: 657: 655: 653: 651: 647: 642: 638: 633: 628: 623: 618: 614: 610: 606: 599: 597: 595: 593: 589: 584: 580: 575: 570: 566: 562: 558: 551: 549: 547: 545: 543: 539: 534: 530: 526: 522: 517: 512: 508: 504: 500: 493: 491: 489: 487: 483: 478: 474: 469: 464: 460: 456: 452: 445: 442: 437: 433: 428: 423: 419: 415: 411: 404: 401: 394: 390: 387: 385: 382: 380: 377: 376: 372: 370: 363: 361: 357: 350: 347: 344: 341: 340: 339: 333: 328: 325: 324: 323: 317: 315: 311: 307: 303: 299: 295: 291: 287: 284: 280: 276: 272: 268: 261: 258: 257: 256: 252: 248: 244: 237: 235: 233: 228: 227:malabsorption 223: 221: 218: 214: 210: 206: 202: 198: 194: 190: 187:(also called 186: 182: 178: 170: 165: 163: 159: 156: 154: 150: 147: 144: 142:and headaches 141: 138: 135: 132: 129: 126: 125: 121: 119: 117: 113: 109: 105: 101: 97: 93: 83: 77: 76:Endocrinology 74: 72: 68: 65: 61: 57: 52: 48: 44: 40: 36: 32: 29: 25: 20: 3280:deficiency, 3149:GSD type VII 3044:GSD type III 2985:Glycogenesis 2935:galactosemia 2820: 2807:Disaccharide 2772:Inborn error 2673: 2652: 2529:Pancreatitis 2491: 2441:biliary tree 2314:Nutmeg liver 2291: 2217:Hematochezia 2168:Pruritus ani 2153:Anal abscess 2148:Anal fistula 2144:Anal fissure 2057:Constipation 2011: 1882:Appendicitis 1690:Gastroptosis 1680:Achlorhydria 1519: 1508:Eosinophilic 1422: 1411: 1400: 1385: 1370: 1346: 1298: 1294: 1284: 1260:(3): 440–7. 1257: 1253: 1243: 1234: 1225: 1198: 1194: 1181: 1154: 1150: 1140: 1105: 1101: 1061: 1057: 1044: 1012:(2): 281–7. 1009: 1005: 995: 968: 964: 954: 945: 939: 930: 889: 885: 831: 827: 817: 790: 786: 773: 762:the original 733: 729: 674: 670: 612: 608: 567:(21): 1–14. 564: 560: 506: 502: 458: 454: 444: 420:(3): 284–8. 417: 413: 403: 367: 364:Epidemiology 358: 354: 337: 321: 312: 308: 304: 300: 296: 292: 288: 285: 281: 277: 273: 269: 265: 253: 249: 245: 241: 224: 209:heterozygous 193:disaccharide 188: 174: 162:palpitations 140:Hypoglycemia 95: 91: 90: 46: 42: 38: 34: 30: 3062:GSD type IX 3050:GSD type VI 3016:GSD type XV 2999:GSD type IV 2797:, transport 2709:Peritonitis 2471:Cholestasis 2449:Cholangitis 2392:Gallbladder 2280:Fatty liver 2200:Hematemesis 2182:GI bleeding 1988:Necrotizing 1917:Lymphocytic 1912:Collagenous 1907:Microscopic 1844:Steatorrhea 1745:Enteropathy 1591:Inlet patch 1513:Herpetiform 1498:Esophagitis 1195:J Biol Chem 1058:J Biol Chem 946:QOL Medical 302:with CSID. 27:Other names 3298:Categories 3272:Pentosuria 3205:GSD type I 3132:Glycolysis 3056:GSD type V 2993:GSD type 0 2896:catabolism 2809:catabolism 2784:Including 2701:Peritoneal 2679:Incisional 2600:Congenital 2544:Hereditary 2521:Pancreatic 2485:Haemobilia 2158:Hemorrhoid 2136:Anal canal 2074:Infectious 2062:Functional 1897:Ulcerative 1781:Duodenitis 1720:Gastrinoma 1424:DiseasesDB 1301:: S7–S13. 615:(1): 2–6. 461:(1): 119. 455:Hum. Mutat 395:References 213:deficiency 189:saccharose 160:and heart 108:metabolism 3080:Lysosomal 2931:Galactose 2833:transport 2689:Richter's 2646:Spigelian 2641:Obturator 2631:Umbilical 2464:Ascending 2435:Bile duct 2268:Cirrhosis 2246:Hepatitis 2229:Accessory 2099:Proctitis 1950:Megacolon 1924:Dysentery 1834:Whipple's 1786:Jejunitis 1776:Enteritis 1663:Dyspepsia 1624:Gastritis 1557:Achalasia 1490:Esophagus 828:Nutrients 509:: S34–5. 318:Treatment 238:Diagnosis 71:Specialty 2976:glycogen 2905:Fructose 2619:Indirect 2614:Inguinal 2348:Pyogenic 2293:Vascular 2069:Diarrhea 2047:Volvulus 2013:Vascular 1952: / 1941: / 1902:Ischemic 1869:Appendix 1759:Duodenum 1629:Atrophic 1503:Candidal 1317:23103658 1276:29311037 1217:11340066 1173:14724820 1132:21520744 1124:23103643 1080:16543230 1036:10903344 987:19121318 906:16527688 860:30501067 809:33626784 750:23103658 703:26857124 677:(1): 5. 641:26812950 525:23103650 477:16329100 373:See also 201:fructose 136:Vomiting 131:Diarrhea 3119:Glucose 2972:Glucose 2885:glucose 2795:Sucrose 2660:Petit's 2636:Femoral 2539:Chronic 2353:Amoebic 2121:Anismus 1887:Colitis 1814:Coeliac 1791:Ileitis 1763:Jejunum 1616:Stomach 1520:Rupture 1418:C538139 1325:7179223 851:6315563 758:7179223 694:4746203 632:4728165 583:8576798 436:8708882 215:in the 197:glucose 191:) is a 185:Sucrose 158:Anxiety 112:sucrose 64:Sucrose 3282:PRKAG2 3140:MODY 2 3007:(APBD) 2881:Hexose 2654:Lumbar 2624:Direct 2607:Hiatus 2587:Hernia 2439:Other 2205:Melena 2091:Rectum 1574:(GERD) 1407:222900 1323:  1315:  1274:  1235:gnomAD 1215:  1171:  1130:  1122:  1078:  1034:  1027:314311 1024:  985:  904:  858:  848:  807:  756:  748:  701:  691:  639:  629:  581:  533:747500 531:  523:  475:  434:  217:enzyme 104:enzyme 78:  41:), or 3253:Other 3238:SDDHD 2788:(GSD) 2579:Other 2534:Acute 2285:MASLD 2238:Liver 2212:Lower 2195:Upper 2037:Ileus 1873:Colon 1767:Ileum 1582:(LPR) 1429:29844 1396:271.3 1381:E74.3 1321:S2CID 1191:(PDF) 1128:S2CID 1054:(PDF) 927:(PDF) 783:(PDF) 765:(PDF) 754:S2CID 726:(PDF) 529:S2CID 171:Cause 102:, an 3144:HHF3 1967:SCAD 1413:MeSH 1402:OMIM 1391:9-CM 1313:PMID 1272:PMID 1213:PMID 1169:PMID 1120:PMID 1076:PMID 1032:PMID 983:PMID 902:PMID 856:PMID 805:PMID 746:PMID 699:PMID 637:PMID 579:PMID 521:PMID 473:PMID 432:PMID 199:and 151:and 47:GSID 39:CSID 3123:CAC 3084:LSD 2774:of 2273:PBC 2000:IBD 1934:IBS 1387:ICD 1372:ICD 1303:doi 1262:doi 1258:118 1203:doi 1199:276 1159:doi 1155:125 1110:doi 1066:doi 1062:281 1022:PMC 1014:doi 1010:106 973:doi 969:136 894:doi 846:PMC 836:doi 795:doi 738:doi 689:PMC 679:doi 627:PMC 617:doi 569:doi 511:doi 463:doi 422:doi 110:of 94:or 3300:: 3121:⇄ 3086:): 2974:⇄ 2933:/ 2883:→ 2778:: 2437:/ 2035:: 2016:: 1812:: 1427:: 1416:: 1405:: 1394:: 1379:: 1376:10 1345:. 1319:. 1311:. 1299:55 1297:. 1293:. 1270:. 1256:. 1252:. 1233:. 1211:. 1197:. 1193:. 1167:. 1153:. 1149:. 1126:. 1118:. 1106:55 1104:. 1100:. 1088:^ 1074:. 1060:. 1056:. 1030:. 1020:. 1008:. 1004:. 981:. 967:. 963:. 929:. 914:^ 900:. 888:. 884:. 868:^ 854:. 844:. 832:10 830:. 826:. 803:. 789:. 785:. 752:. 744:. 734:55 732:. 728:. 711:^ 697:. 687:. 673:. 669:. 649:^ 635:. 625:. 611:. 607:. 591:^ 577:. 565:21 563:. 559:. 541:^ 527:. 519:. 507:55 505:. 501:. 485:^ 471:. 459:27 457:. 453:. 430:. 418:22 416:. 412:. 49:), 33:, 3284:) 3142:/ 3082:( 2952:/ 2937:: 2907:: 2851:) 2847:( 2764:e 2757:t 2750:v 2473:/ 2146:/ 1965:/ 1961:/ 1875:) 1871:/ 1867:( 1769:) 1765:/ 1761:/ 1757:( 1464:e 1457:t 1450:v 1389:- 1374:- 1364:D 1327:. 1305:: 1278:. 1264:: 1219:. 1205:: 1175:. 1161:: 1134:. 1112:: 1082:. 1068:: 1038:. 1016:: 989:. 975:: 933:. 908:. 896:: 890:4 862:. 838:: 811:. 797:: 791:1 740:: 705:. 681:: 675:3 643:. 619:: 613:3 585:. 571:: 535:. 513:: 479:. 465:: 438:. 424:: 45:( 37:(

Index


Sucrose
Specialty
Endocrinology
Edit this on Wikidata
sucrase-isomaltase
enzyme
metabolism
sucrose
small intestine
Diarrhea
Hypoglycemia
Upper respiratory tract
viral diseases
Anxiety
palpitations
genetic mutations
irritable bowel syndrome
Sucrose
disaccharide
glucose
fructose
autosomal recessive
heterozygous
deficiency
enzyme
sucrase-isomaltase
malabsorption
Dietary supplements
Fructose malabsorption

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