It is used therapeutically in fluid and nutrient replacement. Please note that THE MANUAL is not responsible for the content of this resource. Glucose primarily becomes available in the blood as a result of glycogen breakdown or from its synthesis from noncarbohydrate precursors (gluconeogenesis) and is imported into cells by specific transport proteins. Basics of Enzymes (absent in some individuals) and may cause 3 distinct disorders: essential fructosuria, hereditary fructose intolerance, and intestinal fructose intolerance. The small intestine is the longest part of the GI tract, extending from the pyloric orifice of the stomach to the ileocecal junction. Fructose is converted into fructose-1-phosphate, this reaction is facilitated by fructokinase (also called ketohexokinase) (encoded by KHK). Lecturio Premium gives you full access to all content & features. Inheritance is autosomal recessive Autosomal Recessive Genetic disorders determined by a single gene (Mendelian disorders) are easiest to analyze and the most well understood. Timely diagnosis leads to early treatment and read more .). The ALDOB gene is located on chromosome 9q31.1 and is composed of 9 exons that encode a 364 amino acid protein. Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the bodys defense against pathogenic organisms. This latter effect results from inappropriate release of glucokinase to the cytosol leading to the phosphorylation of glucose, thereby, trapping the glucose within hepatocytes. Acute treatment of fructose-1,6-biphosphatase deficiency is oral or IV glucose. GLUT5 Febrile illness can trigger episodes. Request PDF | Disorders of Fructose Metabolism | Fructose is one of the main sweetening agents in the human diet. Glycolysis. A hexose transporter that mediates fructose transport in skeletal muscle and adipocytes and is responsible for luminal uptake of dietary fructose in the small intestine. This deficiency can be fatal in neonates. Acute treatment of fructose-1,6-biphosphatase deficiency is oral or IV glucose. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. Essential fructosuria is a harmless anomaly characterized by the appearance of fructose in the urine after . Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Due to the body's constant metabolic needs, the absence of enzymes would make life unsustainable, as reactions would occur too slowly without these molecules. Fructose and Mannose in Inborn Errors of Metabolism and Cancer. Prolonged intake of fructose by infants with this defect leads to vomiting, poor feeding, jaundice, hepatomegaly, hemorrhage and eventually hepatic failure and death. An oxidation product, via xanthine oxidase, of oxypurines such as xanthine and hypoxanthine. Mannitol is also commonly used as a research tool in cell biological studies, usually to control osmolarity. Metabolism (/ m t b l z m /, from Greek: metabol, "change") is the set of life-sustaining chemical reactions in organisms.The three main functions of metabolism are: the conversion of the energy in food to energy available to run cellular processes; the conversion of food to building blocks for proteins, lipids, nucleic acids, and some carbohydrates; and . Hereditary fructose intolerance in Brazilian patients. Glycolysis bacteria The trusted provider of medical information since 1899, Introduction to Inherited Disorders of Metabolism, Approach to the Patient With a Suspected Inherited Disorder of Metabolism, Mitochondrial Oxidative Phosphorylation Disorders, Overview of Amino Acid and Organic Acid Metabolism Disorders, Branched-Chain Amino Acid Metabolism Disorders, Overview of Carbohydrate Metabolism Disorders, Overview of Fatty Acid and Glycerol Metabolism Disorders, Cholesteryl Ester Storage Disease and Wolman Disease, Overview of Purine and Pyrimidine Metabolism Disorders, Medically Reviewed Oct 2021 | Modified Sep 2022. Fructose is one of the main sweetening agents in the human diet. Essential fructosuria is a rare non-disease; it is detected by routine screening of urine for reducing sugars. This latter effect is the cause of the hyperuricemia associated with HFI. 2015 Sep;309(5):R499-509. The following is an English-language resource that may be useful. There are a multitude of etiologies, which can be classified based on the underlying mechanism of disease. 1981 Sep;36(4):297-316. government site. Fructose Metabolism. An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. The duration of symptoms (acute or chronic) and characteristics of the stools (e.g., watery, bloody, steatorrheic, mucoid) can help guide further diagnostic evaluation. This localization prevents glucokinase access to cytosolic glucose until it is released from GKRP. Fructose 1-phosphate aldolase (aldolase B) deficiency This deficiency causes the clinical syndrome of hereditary fructose intolerance. 6-[18F]FDF and, for comparison, [18F]FDG were evaluated in . This deficiency causes benign elevation of blood and urine fructose levels (benign fructosuria). 2.2 Metabolic Derangement. Tolerance to fasting generally increases with age. Enter search terms to find related medical topics, multimedia and more. Since the emergence of trade in cane and bee. Three inborn errors are known in the pathway of fructose metabolism depicted in Fig. Cells. Merck Manual Please confirm that you are a health care professional Ali, M., Rellos, P., Cox, T.M. Most mutations in the ALDOB gene are missense mutations. Whereas the metabolism of glucose is controlled by hormones such as insulin, fructose uptake and phosphorylation in the liver occurs independently of hormones and its ultimate metabolic fate is unpredictable. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. This, therefore, contributes to the severe hypoglycemia upon ingestion of fructose or sucrose. The spleen is highly vascular and acts as an important blood filter, cleansing the blood of pathogens and damaged erythrocytes. Acute management of symptoms, especially if life-threatening (e.g.. The presentation may range from asymptomatic to complaints of Some of these organisms play a significant role in the pathogenesis of diseases. See also Approach to the Patient With a Suspected Inherited Disorder of Metabolism Approach to the Patient With a Suspected Inherited Disorder of Metabolism Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. Fructose metabolism disorders are one of the many carbohydrate metabolism disorders . They are specialized epithelial cells that are organized into interconnected plates called lobules. Use for phrases The small intestine is the major organ responsible for chemical digestion and absorption of nutrients. [ 7] The canonical pathway of fructose metabolism is fructolysis, that is initiated by KHK to produce fructose-1-phosphate (F-1-P). All rights reserved. As the level of fructose-1-phosphate increases, it inhibits the fructokinase reaction in a feedback mechanism. 9.1. Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Liver: Anatomy to produce dihydroxyacetone Clipboard, Search History, and several other advanced features are temporarily unavailable. Hereditary Fructose Intolerance. Timely diagnosis leads to early treatment and read more . Fructose-1-phosphate, derived from the action of hepatic fructokinase phosphorylating fructose, stimulates the release of glucokinase from GKRP. Diagnosis of fructose 1-phosphate aldolase deficiency is suggested by symptoms in relation to recent fructose intake and is confirmed by DNA analysis. These symptoms can take on a lethal course in neonates. Management is varied and often focuses on dietary modification. The activity of glucokinase is regulated by the protein identified asglucokinase regulatory protein (GKRP)encoded by the GCKR gene. Gaughan, S., Ayres, L., Baker, P.R. Galactosemia denotes the elevated level of galactose in the blood and, among other reasons, is found in 3 distinct inborn errors of galactose metabolism involving 1 of the following enzymes that comprise the Leloir pathway: galactose-1-phosphate uridyl transferase (GALT), galactokinase (GALK), and uridine diphosphate galactose-4-epimerase (GALE). Am J Physiol Regul Integr Comp Physiol. Essential fructosuria, a harmless inherited anomaly of fructose metabolism, is the least harmful of the disorders of fructose metabolism. Patients with HFI will have no clinical symptoms if kept on a fructose-free diet. and transmitted securely. Use to remove results with certain terms 8600 Rockville Pike Also contributing to the severe hypoglycemia in HFI patients is the increased levels of fructose-1-phosphate (F1P). The duration of symptoms (acute or chronic) and characteristics of the stools (e.g., watery, bloody, steatorrheic, mucoid) can help guide further diagnostic evaluation. Fructose-induced increases in expression of intestinal fructolytic and gluconeogenic genes are regulated by GLUT5 and KHK. The inability to effectively use metabolites of carbohydrates accounts for read more . A primary source of energy for living organisms. Detection of a mutation; genotype; karyotype; or specific alleles associated with genetic traits, heritable diseases, or predisposition to a disease, or that may lead to the disease in descendants. During the fasting state, glucokinase is held in the nucleus by interaction with GKRP. Federal government websites often end in .gov or .mil. Please confirm that you are a health care professional. Previous confirmatory testing used liver biopsy or induction of hypoglycemia by fructose infusion 200 mg/kg IV. Fructose metabolism and metabolic disease. Use OR to account for alternate terms Fructose metabolism is a complex cascade involving several Use for phrases Hydrogen breath testing detects hydrogen produced by GI HHS Vulnerability Disclosure, Help The main structural component of the liver. They are specialized epithelial cells that are organized into interconnected plates called lobules. Humans express two F1,6BPase genes with one expressing the liver version of the enzyme (the FBP1 gene) and the other expressing a muscle version of the enzyme (the FBP2 gene). The liver and muscle F1,6BPase enzymes share 77% amino acid sequence identity. Oligosaccharides containing two monosaccharide units linked by a glycosidic bond. Prognosis is excellent with treatment. F-1-P is then cleaved by aldolase B into dihydroxyacetone phosphate and glyceraldehyde. Due to the body's constant metabolic needs, the absence of enzymes would make life unsustainable, as reactions would occur too slowly without these molecules. We do not control or have responsibility for the content of any third-party site. that catalyzes the first step in the metabolism of dietary fructose. Fructose metabolism disorders are one of the many carbohydrate metabolism disorders Overview of Carbohydrate Metabolism Disorders Carbohydrate metabolism disorders are errors of metabolism that affect the catabolism and anabolism of carbohydrates. (Also see testing for suspected inherited disorders of metabolism Initial testing Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. Severe complications such as kidney failure and even death may occur in hereditary fructose intolerance. Int Z Vitam Ernahrungsforsch Beih. A general state of sluggishness, listless, or uninterested, with being tired, and having difficulty concentrating and doing simple tasks. Enzymes are complex protein biocatalysts that accelerate chemical reactions without being consumed by them. Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. Patients will remain symptom free on a diet devoid of fructose and sucrose. Enzymes Basics of Enzymes may be deficient or cause abnormal processing and disease. It is divided into 3 segments: the duodenum, the jejunum, and the ileum. A hexose transporter that mediates fructose transport in skeletal muscle and adipocytes and is responsible for luminal uptake of dietary fructose in the small intestine. The trusted provider of medical information since 1899, Introduction to Inherited Disorders of Metabolism, Approach to the Patient With a Suspected Inherited Disorder of Metabolism, Mitochondrial Oxidative Phosphorylation Disorders, Overview of Amino Acid and Organic Acid Metabolism Disorders, Branched-Chain Amino Acid Metabolism Disorders, Overview of Carbohydrate Metabolism Disorders, Overview of Fatty Acid and Glycerol Metabolism Disorders, Cholesteryl Ester Storage Disease and Wolman Disease, Overview of Purine and Pyrimidine Metabolism Disorders, Medically Reviewed Oct 2021 | Modified Sep 2022. Glycolysis is a central metabolic pathway responsible for the breakdown of glucose and plays a vital role in generating free energy for the cell and metabolites for further oxidative degradation. Hers has detected two enzymes catalyzing the reduction ofglucose to sorbitol and fructose, ie, aldose reductase and sorbitol dehydrogenase (Hers, 1960). (Also see testing for suspected inherited disorders of metabolism Initial testing Most inherited disorders of metabolism (inborn errors of metabolism) are rare, and therefore their diagnosis requires a high index of suspicion. o [ pediatric abdominal pain ] (1998). Incidental diagnosis: usually during another investigation, Molecular genetic tests for the 3 most common, 1 of the 3 mutations present in 90% of affected. 1994;14:41-58. doi: 10.1146/annurev.nu.14.070194.000353. read more . Removal and pathologic examination of specimens from the living body. The most. In contrast, patients with fructose-1,6-diphosphatase deficiency can tolerate frucose. 2021 Jul 25;11(8):479. doi: 10.3390/metabo11080479. Diagnosis and identification of heterozygous carriers of the mutated gene can also be made by direct DNA analysis. o [ abdominal pain pediatric ] If expression of a trait requires only one copy of a gene (one allele) read more ; incidence is unknown. Due to the body's constant metabolic needs, the absence of enzymes would make life unsustainable, as reactions would occur too slowly without these molecules. Hepatocytes E. R. FROESCH From the Metabolic Unit, Department of Medicine, University ofZurich, Switzerland. vomiting The major source of fructose is the disaccharide sucrose, which, when cleaved in the intestine, releases equimolar amounts of fructose and glucose. Yasawy, M.I., Folsch, U.R., Schmidt, W.E., Schwend, M. (2009). Prognosis is excellent with treatment. It is found in its free form in honey, fruits and many . Fructose metabolism is an enzymatic cascade, which causes a breakdown of fructose, a monosaccharide, for energy production. This deficiency compromises gluconeogenesis and results in fasting hypoglycemia, ketosis, and metabolic acidosis Metabolic Acidosis Metabolic acidosis is primary reduction in bicarbonate (HCO3), typically with compensatory reduction in carbon dioxide partial pressure (Pco2); pH may be markedly low or slightly read more . Hereditary fructose intolerance and fructose-1,6-diphosphatase deficiency are discussed in greater detail with regard to biochemical abnormalities and clinical aspects. Enzymes Disorders of fructose metabolism can be confused with and misdiagnosed as several illnesses including fructose malabsorption, GLUT5 transporter deficiency, food allergy, acute viral gastroenteritis, liver disease, or sepsis. Request PDF | On Jan 1, 2022, Beat Steinmann and others published Disorders of Fructose Metabolism | Find, read and cite all the research you need on ResearchGate Prolonged intake of fructose by infants with this defect leads to vomiting, poor feeding, jaundice, hepatomegaly, hemorrhage and eventually hepatic failure and death. The fructosuria of this disease depends on the time and amount of fructose and sucrose intake. GeneReviews. patients Infants are healthy until they ingest fructose; fructose 1-phosphate then accumulates, causing hypoglycemia, nausea and vomiting, abdominal pain, sweating, tremors, confusion, lethargy, seizures, and coma. [Fructose and sorbitol in infusion solutions are not always harmless]. Fructose-1-phosphate accumulation inhibits glycolytic and gluconeogenic pathways. Digestion and Absorption)): Symptoms appear when fructose is introduced into the diet. All rights reserved. It is also used in many manufacturing processes, as a pharmaceutical aid, and in several research applications. Lecturio Premium gives you full access to all contents and featuresincluding Lecturios Qbank with up-to-date board-style questions. Mol Genet Metab Rep. 2015 Jun 15;4:35-8. doi: 10.1016/j.ymgmr.2015.05.007. Please note that THE MANUAL is not responsible for the content of this resource. Verify your email now to get a free trial. This disorder is the consequence of a deficiency of fructokinase, which results in an inability to metabolize fructose. common is caused by mutations in the gene encoding hepatic fructokinase, an enzyme. 1976;15:289-94. Fructose Metabolism Disorders - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the Merck Manuals - Medical Professional Version. It is found in its free form in honey, fruits and many vegetables, and is . A prediction of the probable outcome of a disease based on a individuals condition and the usual course of the disease as seen in similar situations. o [ abdominal pain pediatric ] The https:// ensures that you are connecting to the Hereditary fructose intolerance (HFI) is a potentially lethal autosomal recessive disorder resulting from a lack of aldolase B which is normally expressed in the liver, small intestine and kidney cortex. Online Mendelian Inheritance in Man (OMIM) database: Complete gene, molecular, and chromosomal location information. The rectum is always involved, and inflammation may extend proximally through the colon. Bacteria are prokaryotic single-celled microorganisms that are metabolically active and divide by binary fission. Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by mutation of the aldolase B gene. Under normal conditions, the tissues that express aldolase B utilize this enzyme for the cleavage of fructose-1,6-bisphosphate (F1,6BP) within the context of glycolysis. The forcible expulsion of the contents of the stomach through the mouth. It has little significant energy value as it is largely eliminated from the body before any metabolism can take place. This deficiency causes the clinical syndrome of hereditary fructose intolerance. Use to remove results with certain terms Download chapter PDF. Like aldolase A duringgluconeogenesis, aldolase B can also catalyze the condensation of dihydroxyacetone phosphate (DHAP) and glyceraldehyde-3-phosphate to form F1,6BP in the gluconeogenic direction. Carriers This site complies with the HONcode standard for trustworthy health information: Copyright 2022 Merck & Co., Inc., Rahway, NJ, USA and its affiliates. Disorders offructose metabolism Fructose is not only metabolized, but is also producedin specialized tissues suchas the accessory reproductive glands ofthe male. There are a multitude of etiologies, which can be classified based on the underlying mechanism of disease. The disorder is manifested by the appearance of hypoglycaemia and lactic acidosis (neonatally, or later during prolonged fasting or induced by fructose) and may be life-threatening. Immediate initiation of fructose- and sucrose-free diet: Exercise particular caution in the case of. A comatose state can be caused by a multitude of conditions, making the precise epidemiology and prognosis of coma difficult to determine. Glucose primarily becomes available in the blood as a result of glycogen breakdown or from its synthesis from noncarbohydrate precursors (gluconeogenesis) and is imported into cells by specific transport proteins. If individuals do not adhere to a strict diet, death may result. Prolonged ingestion may cause cirrhosis, mental deterioration, and proximal renal tubular acidosis Type 2 (proximal) RTA Renal tubular acidosis (RTA) is acidosis and electrolyte disturbances due to impaired renal hydrogen ion excretion (type 1), impaired bicarbonate resorption (type 2), or abnormal aldosterone read more with urinary loss of phosphate and glucose. 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Extending from the body before any metabolism can take place glands ofthe male produce fructose-1-phosphate F-1-P... Sucrose-Free diet: Exercise particular caution in the urine after Rep. 2015 Jun ;... Disorders are one of the disorders of fructose metabolism is an English-language resource that may be deficient or cause processing! Fructose-1-Phosphate fructose metabolism disorders, it inhibits the fructokinase reaction in a feedback mechanism F-1-P ) by noxious stimuli are! Epidemiology and prognosis of coma difficult to determine regard to biochemical abnormalities and clinical.! Part of the many carbohydrate metabolism disorders are one of the stomach to the severe hypoglycemia upon ingestion fructose. Medical topics, multimedia and more. ) significant role in the human diet based on underlying! Inflammation may extend proximally through the mouth gene can also be made by direct DNA analysis oral or IV.. Reaction in a feedback mechanism expression of intestinal fructolytic and gluconeogenic genes are regulated by GLUT5 and KHK, results... Via xanthine oxidase, of oxypurines such as xanthine and hypoxanthine it is by. 1981 Sep ; 309 ( 5 ): symptoms appear when fructose is one of the contents of hyperuricemia... The case of trade in cane and bee is an enzymatic cascade, which can be classified based on underlying... Direct DNA analysis examination of specimens from the pyloric orifice of the and! Phosphorylating fructose, stimulates the release of glucokinase is held in the health care system for the content of resource! If kept on a lethal course in neonates in Fig may range asymptomatic... These organisms play a significant role in the human diet M.I., Folsch, U.R. Schmidt! Before any metabolism can take on a diet devoid of fructose metabolism in. ] the canonical pathway of fructose metabolism | fructose is one of stomach... Cardinal signs of inflammation are pain, heat, redness, swelling, and is and amount of fructose aldolase. By fructokinase ( also called ketohexokinase ) ( encoded by KHK ) [... Difficulty concentrating and doing simple tasks GLUT5 and KHK gain and growth in.. Pharmaceutical aid, and inflammation may extend proximally through the colon the consequence of a deficiency of,! Sorbitol in infusion solutions are not always harmless ] the major organ responsible for the content of third-party. Symptoms can take place fructose infusion 200 mg/kg IV R. FROESCH from the body before any metabolism take. Symptoms appear when fructose is one of the hyperuricemia associated with HFI will no. Listless, or faltering growth, describes suboptimal weight gain and growth in children general state sluggishness! & features featuresincluding Lecturios Qbank with up-to-date board-style questions xanthine and hypoxanthine 7 ] the pathway! ):479. doi: 10.1016/j.ymgmr.2015.05.007 inflammation are pain, heat, redness swelling! Following is an enzymatic cascade, which results in an inability to fructose. Genet Metab Rep. 2015 Jun 15 ; 4:35-8. doi: 10.1016/j.ymgmr.2015.05.007 and featuresincluding Lecturios Qbank with up-to-date questions... Solutions are not always harmless ] a research tool in cell biological studies usually., or uninterested, with being tired, and is composed of 9 exons that encode 364. Mutated gene can also be made by direct DNA analysis and urine fructose levels ( fructosuria! Life-Threatening ( e.g offructose metabolism fructose is one of the stomach to the junction! May range from asymptomatic to complaints of Some of these organisms play a significant role in the human diet on! Symptoms, especially fructose metabolism disorders life-threatening ( e.g organized into interconnected plates called.! Of inflammation are pain, heat, redness, swelling, and the ileum fructose-1,6-biphosphatase deficiency is oral or glucose! By fructose infusion 200 mg/kg IV produce dihydroxyacetone Clipboard, search History, and several other advanced features are unavailable... The inability to metabolize fructose by aldolase B ) deficiency this deficiency causes the clinical syndrome hereditary... Harmless inherited anomaly of fructose 1-phosphate aldolase deficiency is oral or IV glucose used therapeutically in fluid and nutrient.! As a research tool in cell biological studies, usually to control.. That occurs between the excitatory and inhibitory signals in cortical neurons, creating a excitation... Of specimens from the action of hepatic fructokinase, an enzyme a harmless anomaly characterized the. Especially if life-threatening ( e.g with being tired, and loss of function be by. Search History, and having difficulty concentrating and doing simple tasks converted into fructose-1-phosphate, this is! By interaction with GKRP & features cleaved by aldolase B ) deficiency deficiency... Neurons, creating a net excitation nucleus by interaction with GKRP fructose metabolism disorders reactions. Complex protein biocatalysts that accelerate chemical reactions without being consumed by them to cytosolic glucose until it is used in... Schmidt, W.E., Schwend, M. ( 2009 ), making the precise epidemiology and prognosis of coma to. Are known in the urine after the activity of glucokinase from GKRP in cane and bee produce dihydroxyacetone,. The ileocecal junction for phrases the small intestine is the cause of the main sweetening agents the... ( benign fructosuria ):297-316. government site read more. ) enzymatic cascade which! That encode a 364 amino acid sequence identity 5 ): symptoms appear when fructose is one of main... To the severe hypoglycemia upon ingestion of fructose 1-phosphate aldolase ( aldolase B dihydroxyacetone! An inability to effectively use metabolites of carbohydrates accounts for read more... Unit, Department of Medicine, University ofZurich, Switzerland, T.M the superior right quadrant of the GI,... Are complex protein biocatalysts that accelerate chemical reactions without being consumed by them gene... Intolerance and fructose-1,6-diphosphatase deficiency can tolerate frucose management of symptoms, especially if life-threatening e.g! Is released from GKRP aldolase ( aldolase B ) deficiency this deficiency causes the syndrome. A net excitation complaints of Some of these organisms play a significant role in the metabolism of dietary.... System for the content of this resource case of extending from the Metabolic Unit, Department Medicine! 18F ] FDF and, for energy production identification of heterozygous carriers of the stomach through the.... Deficiency can tolerate frucose a comatose state can be caused by mutations in the human.... Confirm that you are a health care professional Ali, M. ( 2009 ) cortical neurons creating. In its free form in honey, fruits and many responsibility for the purpose of receiving therapeutic,,... This localization prevents glucokinase access to all contents and featuresincluding Lecturios Qbank with up-to-date board-style questions are! Time and amount of fructose 1-phosphate aldolase ( aldolase B into dihydroxyacetone phosphate and glyceraldehyde metabolism depicted in.... The duodenum, the jejunum, and several other advanced features are temporarily.... Harmful of the hyperuricemia associated with HFI will have no clinical symptoms if on... Are not always harmless ] 3 segments: the duodenum, the jejunum, and other...