An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.
Inborn errors of carbohydrate metabolism are genetic disorders that result from enzyme deficiencies or transport defects in the metabolic pathways responsible for breaking down and processing carbohydrates, leading to accumulation of toxic intermediates or energy deficits, and typically presenting with multisystem clinical manifestations.
Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.
A monosaccharide in sweet fruits and honey that is soluble in water, alcohol, or ether. It is used as a preservative and an intravenous infusion in parenteral feeding.
A group of inherited kidney disorders characterized by the abnormally elevated levels of AMINO ACIDS in URINE. Genetic mutations of transport proteins result in the defective reabsorption of free amino acids at the PROXIMAL RENAL TUBULES. Renal aminoaciduria are classified by the specific amino acid or acids involved.
A pathological state in which BLOOD GLUCOSE level is less than approximately 140 mg/100 ml of PLASMA at fasting, and above approximately 200 mg/100 ml plasma at 30-, 60-, or 90-minute during a GLUCOSE TOLERANCE TEST. This condition is seen frequently in DIABETES MELLITUS, but also occurs with other diseases and MALNUTRITION.
The condition resulting from the absence or deficiency of LACTASE in the MUCOSA cells of the GASTROINTESTINAL TRACT, and the inability to break down LACTOSE in milk for ABSORPTION. Bacterial fermentation of the unabsorbed lactose leads to symptoms that range from a mild indigestion (DYSPEPSIA) to severe DIARRHEA. Lactose intolerance may be an inborn error or acquired.
D-Galactose:NAD(P)+ 1-oxidoreductases. Catalyzes the oxidation of D-galactose in the presence of NAD+ or NADP+ to D-galactono-gamma-lactone and NADH or NADPH. Includes EC 1.1.1.48 and EC 1.1.1.120.
An enzyme which catalyzes the hydrolysis of LACTOSE to D-GALACTOSE and D-GLUCOSE. Defects in the enzyme cause LACTOSE INTOLERANCE.
A group of inherited enzyme deficiencies which feature elevations of GALACTOSE in the blood. This condition may be associated with deficiencies of GALACTOKINASE; UDPGLUCOSE-HEXOSE-1-PHOSPHATE URIDYLYLTRANSFERASE; or UDPGLUCOSE 4-EPIMERASE. The classic form is caused by UDPglucose-Hexose-1-Phosphate Uridylyltransferase deficiency, and presents in infancy with FAILURE TO THRIVE; VOMITING; and INTRACRANIAL HYPERTENSION. Affected individuals also may develop MENTAL RETARDATION; JAUNDICE; hepatosplenomegaly; ovarian failure (PRIMARY OVARIAN INSUFFICIENCY); and cataracts. (From Menkes, Textbook of Child Neurology, 5th ed, pp61-3)
I'm sorry for any confusion, but "Colorado" is a place, specifically a state in the United States, and does not have a medical definition. If you have any questions about medical conditions or terminology, I would be happy to help with those!
Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.
FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.
FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.
Services designed for HEALTH PROMOTION and prevention of disease.
Groups set up to advise governmental bodies, societies, or other institutions on policy. (Bioethics Thesaurus)
INFLAMMATION of the LIVER in humans caused by HEPATITIS C VIRUS, a single-stranded RNA virus. Its incubation period is 30-90 days. Hepatitis C is transmitted primarily by contaminated blood parenterally, and is often associated with transfusion and intravenous drug abuse. However, in a significant number of cases, the source of hepatitis C infection is unknown.

Alteration of substrate specificity by a naturally-occurring aldolase B mutation (Ala337-->Val) in fructose intolerance. (1/35)

A molecular analysis of human aldolase B genes in two newborn infants and a 4-year-old child with hereditary fructose intolerance, the offspring of a consanguineous union, has identified the novel mutation Ala337-->Val in homozygous form. This mutation was also detected independently in two other affected individuals who were compound heterozygotes for the prevalent aldolase B allele, Ala149-->Pro, indicating that the mutation causes aldolase B deficiency. To test for the effect of the mutation, catalytically active wild-type human aldolase B and the Val337 variant enzyme were expressed in Escherichia coli. The specific activities of the wild-type recombinant enzyme were 4.8 units/mg and 4.5 units/mg towards fructose 1,6-bisphosphate (FBP) and fructose 1-phosphate (F-1-P) as substrates with Michaelis constants of 4 microM and 2.4 mM respectively. The specific activities of purified tetrameric Val337 aldolase B, which affects an invariant residue in the C-terminal region, were 4.2 units/mg and 2.6 units/mg towards FBP and F-1-P as substrates respectively; the corresponding Michaelis constants were 22 microM and 24 mM. The FBP-to-F-1-P substrate activity ratios were 0.98 and 1.63 for wild-type and Val337 variant enzymes respectively. The Val337 mutant aldolase had an increased susceptibility to proteolytic cleavage in E. coli and rapidly lost activity on storage. Comparative CD determinations showed that the Val337 protein had a distinct thermal denaturation profile with markedly decreased enthalpy, indicating that the mutant protein is partly unfolded. The undegraded mutant had preferentially decreased affinity and activity towards its specific F-1-P substrate and maintained appreciable activity towards FBP. In contrast, fluorescence studies of the mutant showed an increased binding affinity for products of the aldolase reaction, indicating a role for the C-terminus in mediating product release. These findings in a rare but widespread naturally occurring mutant implicate the C-terminus in the activity of human aldolase B towards its specific substrates and demonstrate its role in maintaining the overall stability of the enzyme tetramer.  (+info)

Novel six-nucleotide deletion in the hepatic fructose-1,6-bisphosphate aldolase gene in a patient with hereditary fructose intolerance and enzyme structure-function implications. (2/35)

Hereditary fructose intolerance (HFI) is an autosomal recessive human disease that results from the deficiency of the hepatic aldolase isoenzyme. Affected individuals will succumb to the disease unless it is readily diagnosed and fructose eliminated from the diet. Simple and non-invasive diagnosis is now possible by direct DNA analysis that scans for known and unknown mutations. Using a combination of several PCR-based methods (restriction enzyme digestion, allele specific oligonucleotide hybridisation, single strand conformation analysis and direct sequencing) we identified a novel six-nucleotide deletion in exon 6 of the aldolase B gene (delta 6ex6) that leads to the elimination of two amino acid residues (Leu182 and Val183) leaving the message inframe. The three-dimensional structural alterations induced in the enzyme by delta 6ex6 have been elucidated by molecular graphics analysis using the crystal structure of the rabbit muscle aldolase as reference model. These studies showed that the elimination of Leu182 and Val183 perturbs the correct orientation of adjacent catalytic residues such as Lys146 and Glu187.  (+info)

Hereditary fructose intolerance and alpha(1) antitrypsin deficiency. (3/35)

A patient with coexisting hereditary fructose intolerance (HFI) and alpha(1) antitrypsin deficiency (alpha(1)ATD) is described. Protease inhibitor typing was not conclusive, presumably because of impaired N-glycosylation secondary to HFI. The case underlines the diagnostic role of molecular genetic techniques in inborn errors of metabolism.  (+info)

Functional and molecular modelling studies of two hereditary fructose intolerance-causing mutations at arginine 303 in human liver aldolase. (4/35)

We have identified a novel hereditary fructose intolerance mutation in the aldolase B gene (i.e. liver aldolase) that causes an arginine-to-glutamine substitution at residue 303 (Arg(303)-->Gln). We previously described another mutation (Arg(303)-->Trp) at the same residue. We have expressed the wild-type protein and the two mutated proteins and characterized their kinetic properties. The catalytic efficiency of protein Gln(303) is approx. 1/100 that of the wild-type for substrates fructose 1,6-bisphosphate and fructose 1-phosphate. The Trp(303) enzyme has a catalytic efficiency approx. 1/4800 that of the wild-type for fructose 1,6-bisphosphate; no activity was detected with fructose 1-phosphate. The mutation Arg(303)-->Trp thus substitution impairs enzyme activity more than Arg(303)-->Gln. Three-dimensional models of wild-type, Trp(303) and Gln(303) aldolase B generated by homology-modelling techniques suggest that, because of its larger size, tryptophan exerts a greater deranging effect than glutamine on the enzyme's three-dimensional structure. Our results show that the Arg(303)-->Gln substitution is a novel mutation causing hereditary fructose intolerance and provide a functional demonstration that Arg(303), a conserved residue in all vertebrate aldolases, has a dominant role in substrate binding during enzyme catalysis.  (+info)

Carbohydrate malabsorption and the effect of dietary restriction on symptoms of irritable bowel syndrome and functional bowel complaints. (5/35)

BACKGROUND: Carbohydrate malabsorption of lactose, fructose and sorbitol has already been described in normal volunteers and in patients with functional bowel complaints including irritable bowel syndrome. Elimination of the offending sugar(s) should result in clinical improvement. OBJECTIVE: To examine the importance of carbohydrate malabsorption in outpatients previously diagnosed as having functional bowel disorders, and to estimate the degree of clinical improvement following dietary restriction of the malabsorbed sugar(s). METHODS: A cohort of 239 patients defined as functional bowel complaints was divided into a group of 94 patients who met the Rome criteria for irritable bowel syndrome and a second group of 145 patients who did not fulfill these criteria and were defined as functional complaints. Lactose (18 g), fructose (25 g) and a mixture of fructose (25 g) plus sorbitol (5 g) solutions were administered at weekly intervals. End-expiratory hydrogen and methane breath samples were collected at 30 minute intervals for 4 hours. Incomplete absorption was defined as an increment in breath hydrogen of at least 20 ppm, or its equivalent in methane of at least 5 ppm. All patients received a diet without the offending sugar(s) for one month. RESULTS: Only 7% of patients with IBS and 8% of patients with FC absorbed all three sugars normally. The frequency of isolated lactose malabsorption was 16% and 12% respectively. The association of lactose and fructose-sorbitol malabsorption occurred in 61% of both patient groups. The frequency of sugar malabsorption among patients in both groups was 78% for lactose malabsorption (IBS 82%, FC 75%), 44% for fructose malabsorption and 73% for fructose-sorbitol malabsorption (IBS 70%, FC 75%). A marked improvement occurred in 56% of IBS and 60% of FC patients following dietary restriction. The number of symptoms decreased significantly in both groups (P < 0.01) and correlated with the improvement index (IBS P < 0.05, FC P < 0.025). CONCLUSIONS: Combined sugar malabsorption patterns are common in functional bowel disorders and may contribute to symptomatology in most patients. Dietary restriction of the offending sugar(s) should be implemented before the institution of drug therapy.  (+info)

Inherited risks for susceptibility to dental caries. (6/35)

Dental caries incidence is affected by host factors that may be related to the structure of dental enamel, immunologic response to cariogenic bacteria, or the composition of saliva. Genetic variation of the host factors may contribute to increased risks for dental caries. This systematic review examined the literature to address the question, "Is the risk for dental decay related to patterns of genetic inheritance?" Numerous reports have described a potential genetic contribution to the risk for dental caries. Studies on twins have provided strong evidence for the role of inheritance. Establishing a basis for a genetic contribution to dental caries will provide a foundation for future studies utilizing the human genome sequence to improve understanding of the disease process. Inherited disorders of tooth development with altered enamel structure increase the incidence of dental caries. Specific genetic linkage has not been determined for all of the syndromes of altered tooth development. Consequently, genetic screens of large populations for genes or mutations associated with increased caries susceptibility have not been done. Altered immune response to the cariogenic bacteria may also increase the incidence of caries. Association between specific patterns of HLA genetic inheritance and dental caries risk is weak and does not provide a predictable basis for predicting future decay rates. The evidence supporting an inherited susceptibility to dental caries is limited. Genetic linkage approaches on well-characterized populations with clearly defined dental caries incidence will be required to further analyze the relationship between inheritance and dental caries.  (+info)

Structural and functional analysis of aldolase B mutants related to hereditary fructose intolerance. (7/35)

Hereditary fructose intolerance (HFI) is a recessively inherited disorder of carbohydrate metabolism caused by impaired function of human liver aldolase (B isoform). 25 enzyme-impairing mutations have been identified in the aldolase B gene. We have studied the HFI-related mutant recombinant proteins W147R, A149P, A174D, L256P, N334K and delta6ex6 in relation to aldolase B function and structure using kinetic assays and molecular graphics analysis. We found that these mutations affect aldolase B function by decreasing substrate affinity, maximal velocity and/or enzyme stability. Finally, the functional and structural analyses of the non-natural mutant Q354E provide insight into the catalytic role of Arg(303), whose natural mutants are associated to HFI.  (+info)

Comparison of breath testing with fructose and high fructose corn syrups in health and IBS. (8/35)

 (+info)

Fructose intolerance, also known as hereditary fructose intolerance (HFI), is a genetic disorder that affects the body's ability to metabolize the sugar called fructose, which is found in fruits, vegetables, and processed foods. It is caused by a deficiency of an enzyme called aldolase B, which is necessary for the breakdown and absorption of fructose in the liver.

When individuals with fructose intolerance consume food or drinks containing fructose, the undigested fructose accumulates in the bloodstream and gets absorbed by other organs, leading to a range of symptoms such as abdominal pain, bloating, diarrhea, vomiting, and low blood sugar. Prolonged exposure to high levels of fructose can also cause liver damage, kidney failure, and growth retardation in children.

The diagnosis of fructose intolerance is usually made through a combination of clinical symptoms, genetic testing, and a fructose tolerance test. The treatment for fructose intolerance involves avoiding foods and drinks that contain fructose or limiting their consumption to very small amounts. In some cases, supplementation with enzyme replacement therapy may be recommended.

Fructose-bisphosphate aldolase is a crucial enzyme in the glycolytic pathway, which is a metabolic process that breaks down glucose to produce energy. This enzyme catalyzes the conversion of fructose-1,6-bisphosphate into two triose sugars: dihydroxyacetone phosphate and glyceraldehyde-3-phosphate.

There are two main types of aldolase isoenzymes in humans, classified as aldolase A (or muscle type) and aldolase B (or liver type). Fructose-bisphosphate aldolase refers specifically to aldolase A, which is primarily found in the muscles, brain, and red blood cells. Aldolase B, on the other hand, is predominantly found in the liver, kidney, and small intestine.

Deficiency or dysfunction of fructose-bisphosphate aldolase can lead to metabolic disorders, such as hereditary fructose intolerance, which results from a deficiency in another enzyme called aldolase B. However, it is essential to note that the term "fructose-bisphosphate aldolase" typically refers to aldolase A and not aldolase B.

Inborn errors of carbohydrate metabolism refer to genetic disorders that affect the body's ability to break down and process carbohydrates, which are sugars and starches that provide energy for the body. These disorders are caused by defects in enzymes or transport proteins that play a critical role in the metabolic pathways involved in carbohydrate metabolism.

There are several types of inborn errors of carbohydrate metabolism, including:

1. Galactosemia: This disorder affects the body's ability to metabolize the sugar galactose, which is found in milk and other dairy products. It is caused by a deficiency of the enzyme galactose-1-phosphate uridylyltransferase.
2. Glycogen storage diseases: These disorders affect the body's ability to store and break down glycogen, which is a complex carbohydrate that serves as a source of energy for the body. There are several types of glycogen storage diseases, each caused by a deficiency in a different enzyme involved in glycogen metabolism.
3. Hereditary fructose intolerance: This disorder affects the body's ability to metabolize the sugar fructose, which is found in fruits and sweeteners. It is caused by a deficiency of the enzyme aldolase B.
4. Pentose phosphate pathway disorders: These disorders affect the body's ability to metabolize certain sugars and generate energy through the pentose phosphate pathway. They are caused by defects in enzymes involved in this pathway.

Symptoms of inborn errors of carbohydrate metabolism can vary widely depending on the specific disorder and its severity. Treatment typically involves dietary restrictions, supplementation with necessary enzymes or cofactors, and management of complications. In some cases, enzyme replacement therapy or even organ transplantation may be considered.

Inborn errors of fructose metabolism refer to genetic disorders that affect the body's ability to break down and process fructose, a simple sugar found in fruits, vegetables, and honey. These disorders are caused by mutations in genes responsible for encoding enzymes involved in fructose metabolism.

The two main types of inborn errors of fructose metabolism are:

1. Hereditary Fructose Intolerance (HFI): This is a rare genetic disorder caused by a deficiency of the enzyme aldolase B, which is necessary for the breakdown of fructose in the liver. When individuals with HFI consume fructose or sucrose (a disaccharide that contains fructose and glucose), they experience a buildup of toxic metabolites, leading to symptoms such as vomiting, abdominal pain, hypoglycemia, and in severe cases, liver damage and failure.
2. Fructose-1,6-bisphosphatase Deficiency (FBPase Deficiency): This is a rare autosomal recessive disorder caused by a deficiency of the enzyme fructose-1,6-bisphosphatase, which is essential for gluconeogenesis (the process of generating glucose from non-carbohydrate sources). Individuals with FBPase Deficiency experience symptoms such as hypoglycemia, lactic acidosis, and hyperventilation, particularly during periods of fasting or illness.

Both disorders can be managed through dietary restrictions and close monitoring of blood sugar levels. In severe cases, enzyme replacement therapy or liver transplantation may be considered.

Fructose is a simple monosaccharide, also known as "fruit sugar." It is a naturally occurring carbohydrate that is found in fruits, vegetables, and honey. Fructose has the chemical formula C6H12O6 and is a hexose, or six-carbon sugar.

Fructose is absorbed directly into the bloodstream during digestion and is metabolized primarily in the liver. It is sweeter than other sugars such as glucose and sucrose (table sugar), which makes it a popular sweetener in many processed foods and beverages. However, consuming large amounts of fructose can have negative health effects, including increasing the risk of obesity, diabetes, and heart disease.

Renal aminoacidurias are a group of inherited kidney disorders characterized by the abnormal excretion of amino acids in the urine (aminoaciduria). This condition results from defects in the renal tubular transport systems that are responsible for the reabsorption of amino acids from the filtrate in the kidneys.

There are several types of renal aminoacidurias, each associated with a specific genetic mutation affecting different transporter proteins in the proximal renal tubules. The most common type is cystinuria, which is caused by a defect in the transport system for four amino acids: cystine, ornithine, lysine, and arginine. Other types of renal aminoacidurias include Hartnup disorder, Lowe syndrome, and Dent disease, among others.

The clinical manifestations of renal aminoacidurias vary depending on the specific type and severity of the disorder. Some individuals may be asymptomatic or have only mild symptoms, while others may experience severe complications such as kidney stones, urinary tract infections, neurological symptoms, or growth retardation.

Treatment for renal aminoacidurias typically involves dietary modifications, increased fluid intake, and medications to reduce the risk of kidney stone formation and other complications. In some cases, surgery may be necessary to remove large kidney stones.

Glucose intolerance is a condition in which the body has difficulty processing and using glucose, or blood sugar, effectively. This results in higher than normal levels of glucose in the blood after eating, particularly after meals that are high in carbohydrates. Glucose intolerance can be an early sign of developing diabetes, specifically type 2 diabetes, and it may also indicate other metabolic disorders such as prediabetes or insulin resistance.

In a healthy individual, the pancreas produces insulin to help regulate blood sugar levels by facilitating glucose uptake in muscles, fat tissue, and the liver. When someone has glucose intolerance, their body may not produce enough insulin, or their cells may have become less responsive to insulin (insulin resistance), leading to impaired glucose metabolism.

Glucose intolerance can be diagnosed through various tests, including the oral glucose tolerance test (OGTT) and hemoglobin A1c (HbA1c) test. Treatment for glucose intolerance often involves lifestyle modifications such as weight loss, increased physical activity, and a balanced diet with reduced sugar and refined carbohydrate intake. In some cases, medication may be prescribed to help manage blood sugar levels more effectively.

Lactose intolerance is a digestive condition in which the body has difficulty digesting lactose, a sugar found in milk and dairy products. This occurs due to a deficiency or insufficiency of lactase, an enzyme produced by the small intestine that breaks down lactose into simpler sugars (glucose and galactose) for absorption. When there is not enough lactase to digest the consumed lactose, it passes undigested into the large intestine, where it is fermented by bacteria, leading to various gastrointestinal symptoms.

The symptoms of lactose intolerance may include bloating, cramps, diarrhea, nausea, and gas, usually occurring within 30 minutes to two hours after consuming dairy products. The severity of these symptoms can vary depending on the amount of lactose consumed and an individual's level of lactase deficiency or insufficiency.

Lactose intolerance is not life-threatening but can cause discomfort and may affect a person's quality of life. It is essential to manage the condition through dietary modifications, such as consuming smaller amounts of dairy products, choosing lactose-free or reduced-lactose options, or using lactase enzyme supplements before eating dairy products. In some cases, a healthcare professional may recommend additional management strategies based on an individual's specific needs and medical history.

Galactose dehydrogenases (GDH) are a group of enzymes that play a role in the metabolism of galactose, a simple sugar that is a component of lactose and other complex carbohydrates. These enzymes catalyze the oxidation of galactose to galactonate, using NAD+ as an electron acceptor. This reaction is part of the pathway that converts galactose to glucose in the body.

There are several different isoforms of galactose dehydrogenases found in various tissues and organisms, including:

1. GDH1 (also known as GALT): This is the primary form of galactose dehydrogenase found in humans and other mammals. It is located in the cytosol of cells and is responsible for the majority of galactose metabolism. Mutations in this gene can lead to a genetic disorder called classic galactosemia, which is characterized by an inability to metabolize galactose properly.
2. GDH2 (also known as G Aldo): This form of galactose dehydrogenase is found in the endoplasmic reticulum and is involved in the quality control of glycoproteins. It catalyzes the reverse reaction, reducing galactonate to galactose.
3. GDH3 (also known as G AldoX): This form of galactose dehydrogenase is found in the mitochondria and is involved in the metabolism of ascorbic acid (vitamin C). It also catalyzes the reverse reaction, reducing galactonate to galactose.
4. BGDH: This form of galactose dehydrogenase is found in bacteria and some plants. It is involved in the metabolism of both galactose and glucose.

Deficiencies or mutations in these enzymes can lead to various metabolic disorders, including galactosemia, which can cause a range of symptoms such as cataracts, developmental delays, and liver damage.

Lactase is a specific enzyme that is produced by the cells lining the small intestine in humans and other mammals. Its primary function is to break down lactose, a sugar found in milk and dairy products, into simpler sugars called glucose and galactose, which can then be absorbed into the bloodstream.

Lactase is most active during infancy and early childhood, when breast milk or formula is the primary source of nutrition. However, in some individuals, lactase production decreases after weaning, leading to a condition called lactose intolerance. Lactose intolerant individuals have difficulty digesting lactose, which can result in various gastrointestinal symptoms such as bloating, cramps, diarrhea, and gas.

Supplemental lactase enzymes are available over the counter to help lactose-intolerant individuals digest dairy products more comfortably.

Galactosemia is a rare metabolic disorder that affects the body's ability to metabolize the simple sugar galactose, which is found in milk and other dairy products. It is caused by deficiency or complete absence of one of the three enzymes needed to convert galactose into glucose:

1. Galactokinase (GALK) deficiency - also known as Galactokinase galactosemia, is a milder form of the disorder.
2. Galactose-1-phosphate uridylyltransferase (GALT) deficiency - the most common and severe form of classic galactosemia.
3. Galactose epimerase (GALE) deficiency - also known as Epimerase deficiency galactosemia, is a rare and milder form of the disorder.

The most severe form of the disorder, GALT deficiency, can lead to serious health problems such as cataracts, liver damage, mental retardation, and sepsis if left untreated. Treatment typically involves removing galactose from the diet, which requires avoiding all milk and dairy products. Early diagnosis and treatment are crucial for improving outcomes in individuals with galactosemia.

I believe you are looking for a medical condition or term related to the state of Colorado, but there is no specific medical definition for "Colorado." However, Colorado is known for its high altitude and lower oxygen levels, which can sometimes affect visitors who are not acclimated to the elevation. This can result in symptoms such as shortness of breath, fatigue, and headaches, a condition sometimes referred to as "altitude sickness" or "mountain sickness." But again, this is not a medical definition for Colorado itself.

Liver cirrhosis is a chronic, progressive disease characterized by the replacement of normal liver tissue with scarred (fibrotic) tissue, leading to loss of function. The scarring is caused by long-term damage from various sources such as hepatitis, alcohol abuse, nonalcoholic fatty liver disease, and other causes. As the disease advances, it can lead to complications like portal hypertension, fluid accumulation in the abdomen (ascites), impaired brain function (hepatic encephalopathy), and increased risk of liver cancer. It is generally irreversible, but early detection and treatment of underlying causes may help slow down its progression.

Biliary cirrhosis is a specific type of liver cirrhosis that results from chronic inflammation and scarring of the bile ducts, leading to impaired bile flow, liver damage, and fibrosis. It can be further classified into primary biliary cholangitis (PBC) and secondary biliary cirrhosis. PBC is an autoimmune disease, while secondary biliary cirrhosis is often associated with chronic gallstones, biliary tract obstruction, or recurrent pyogenic cholangitis. Symptoms may include fatigue, itching, jaundice, and abdominal discomfort. Diagnosis typically involves blood tests, imaging studies, and sometimes liver biopsy. Treatment focuses on managing symptoms, slowing disease progression, and preventing complications.

Alcoholic Liver Cirrhosis is a medical condition characterized by irreversible scarring (fibrosis) and damage to the liver caused by excessive consumption of alcohol over an extended period. The liver's normal structure and function are progressively impaired as healthy liver tissue is replaced by scarred tissue, leading to the formation of nodules (regenerative noduli).

The condition typically develops after years of heavy drinking, with a higher risk for those who consume more than 60 grams of pure alcohol daily. The damage caused by alcoholic liver cirrhosis can be life-threatening and may result in complications such as:

1. Ascites (accumulation of fluid in the abdomen)
2. Encephalopathy (neurological dysfunction due to liver failure)
3. Esophageal varices (dilated veins in the esophagus that can rupture and bleed)
4. Hepatorenal syndrome (kidney failure caused by liver disease)
5. Increased susceptibility to infections
6. Liver cancer (hepatocellular carcinoma)
7. Portal hypertension (increased blood pressure in the portal vein that supplies blood to the liver)

Abstaining from alcohol and managing underlying medical conditions are crucial for slowing down or halting disease progression. Treatment may involve medications, dietary changes, and supportive care to address complications. In severe cases, a liver transplant might be necessary.

Preventive health services refer to measures taken to prevent diseases or injuries rather than curing them or treating their symptoms. These services include screenings, vaccinations, and counseling aimed at preventing or identifying illnesses in their earliest stages. Examples of preventive health services include:

1. Screenings for various types of cancer (e.g., breast, cervical, colorectal)
2. Vaccinations against infectious diseases (e.g., influenza, pneumococcal pneumonia, human papillomavirus)
3. Counseling on lifestyle modifications to reduce the risk of chronic diseases (e.g., smoking cessation, diet and exercise counseling, alcohol misuse screening and intervention)
4. Screenings for cardiovascular disease risk factors (e.g., cholesterol levels, blood pressure, body mass index)
5. Screenings for mental health conditions (e.g., depression)
6. Preventive medications (e.g., aspirin for primary prevention of cardiovascular disease in certain individuals)

Preventive health services are an essential component of overall healthcare and play a critical role in improving health outcomes, reducing healthcare costs, and enhancing quality of life.

Advisory committees, in the context of medicine and healthcare, are groups of experts that provide guidance and recommendations to organizations or governmental bodies on medical and health-related matters. These committees typically consist of physicians, researchers, scientists, and other healthcare professionals who have expertise in a specific area.

Their roles can include:

1. Providing expert advice on clinical guidelines, treatment protocols, and diagnostic criteria.
2. Evaluating the safety and efficacy of medical products, such as drugs and devices.
3. Making recommendations on public health policies and regulations.
4. Assessing the impact of new research findings on clinical practice.
5. Providing education and training to healthcare professionals.

Advisory committees can be found at various levels, including within hospitals and medical institutions, as well as at the state and federal level. Their recommendations are intended to help inform decision-making and improve the quality of care delivered to patients. However, it's important to note that these committees do not have legislative or regulatory authority, and their recommendations are non-binding.

Hepatitis C is a liver infection caused by the hepatitis C virus (HCV). It's primarily spread through contact with contaminated blood, often through sharing needles or other equipment to inject drugs. For some people, hepatitis C is a short-term illness but for most — about 75-85% — it becomes a long-term, chronic infection that can lead to serious health problems like liver damage, liver failure, and even liver cancer. The virus can infect and inflame the liver, causing symptoms like jaundice (yellowing of the skin and eyes), abdominal pain, fatigue, and dark urine. Many people with hepatitis C don't have any symptoms, so they might not know they have the infection until they experience complications. There are effective treatments available for hepatitis C, including antiviral medications that can cure the infection in most people. Regular testing is important to diagnose and treat hepatitis C early, before it causes serious health problems.

... may refer to: Fructose malabsorption, a digestive disorder of the small intestine in which the fructose ... liver enzymes that metabolise fructose This disambiguation page lists articles associated with the title Fructose intolerance. ... carrier in enterocytes is deficient Hereditary fructose intolerance, a hereditary condition caused by a deficiency of ...
... (HFI) is an inborn error of fructose metabolism caused by a deficiency of the enzyme aldolase B ... Fructose malabsorption "Hereditary fructose intolerance". Genetic and Rare Disorders Information Center (GARD). National ... If fructose is ingested, the enzymatic block at aldolase B causes an accumulation of fructose-1-phosphate which, over time, ... Gaughan S, Ayres L, Baker PR II (2021). "Hereditary Fructose Intolerance". (GeneReviews® [Internet]). Seattle: University of ...
Fructose Intolerance. An under-recognized problem. Am J Gastroenterol 2003;98:1348-53. Rao SSC. Diagnosis and management of ... Choi, Young K.; Johlin, Fredrick C.; Summers, Robert W.; Jackson, Michelle; Rao, Satish S. C. (2003). "Fructose intolerance: an ... food intolerance notably fructose and fructan, gas and bloating and small intestinal bacterial and fungal overgrowth (SIBO/SIFO ... fructose and fructan breath tests, and treatments such as home biofeedback for dyssynergia and translumbosacral neuromodulation ...
TARDBP Fructose intolerance; 229600; ALDOB Fructose-1,6-bisphosphatase deficiency; 229700; FBP1 Fucosidosis; 230000; FUCA1 ... SH2D1A Lysinuric protein intolerance; 222700; SLC7A7 Lysosomal acid phosphatase deficiency; 200950; ACP2 Lysyl hydroxylase 3 ... TP63 Orthostatic intolerance; 604715; SLC6A2 Osseous heteroplasia, progressive; 166350; GNAS Ossification of posterior ...
Fructose malabsorption and lactose intolerance; deficient fructose transport by the duodenum, or by the deficiency of the ... "Fructose malabsorption may play a role in the development of depressed mood. Fructose malabsorption should be considered in ... "Fructose and sorbitol reduced diet in subjects with fructose malabsorption does not only reduce gastrointestinal symptoms but ... Ledochowski M, Widner B, Bair H, Probst T, Fuchs D (October 2000). "Fructose- and Sorbitol-reduced Diet Improves Mood and ...
The specific strain known as blue agave syrup is not recommended for people with fructose intolerance. Agave syrups are sold in ... Having fructose as its primary sugar, blue-agave syrup (56% fructose) is similar in fructose content to high-fructose corn ... "Fructose intolerance: Which foods to avoid?". Mayo Clinic. 29 November 2016. Retrieved 18 February 2018. Getty, Anna (2010). ... fructose, having an effect on blood sugar comparable to fructose itself, as measured by its low glycemic index (GI) of between ...
Hereditary fructose intolerance Inverted sugar syrup "Fructose". m-w.com. Merriam-Webster. Archived from the original on 5 June ... In addition, fructose transfer activity increases with dietary fructose intake. The presence of fructose in the lumen causes ... The first step in the metabolism of fructose is the phosphorylation of fructose to fructose 1-phosphate by fructokinase, thus ... Fructose exists in foods either as a monosaccharide (free fructose) or as a unit of a disaccharide (sucrose). Free fructose is ...
Fedewa A, Rao SS (January 2014). "Dietary fructose intolerance, fructan intolerance and FODMAPs". Current Gastroenterology ... Reduction of fructose and fructan has been shown to reduce IBS symptoms in a dose-dependent manner in people with fructose ... The diet restricts various carbohydrates which are poorly absorbed in the small intestine, as well as fructose and lactose, ... They are globally trimmed, rather than individually, which is more successful than for example restricting only fructose and ...
"Dietary fructose intolerance, fructan intolerance and FODMAPs". Current Gastroenterology Reports (Review). 16 (1): 370. doi: ... Fructose malabsorption and lactose intolerance may produce IBS symptoms through the same mechanism, but unlike other FODMAPs, ... poor absorption of fructose is found in only a minority of people. Lactose intolerance is found in most adults, except for ... Fructan intolerance does not explain the extra-digestive symptoms that people with non-celiac gluten sensitivity may develop, ...
Some defects in aldolase B cause hereditary fructose intolerance. The metabolism of free fructose in liver exploits the ability ... fructose 1,6-bisphosphate + 2(NADP+ + ADP + Pi) fructose 1,6-bisphosphate + H2O → fructose 6-phosphate + Pi In gluconeogenesis ... "Determination of fructose metabolic pathways in normal and fructose-intolerant children: a C-13 NMR study using C-13 fructose ... Archaeal fructose-bisphosphate aldolase/phosphatase is presumably involved in gluconeogenesis because its product is fructose 6 ...
Acute Hypoglycemia Fructose 1-Phosphate Aldolase Deficiency (Hereditary fructose intolerance). There are two types of glycogen ... fructose intolerance). Serum lactate is in reference ranges in fasting patients with glycogen-storage disease type 0. Liver ... Oral loading of glucose, galactose, or fructose results in a marked rise in blood lactate levels. Liver biopsy for microscopic ... However, urine-reducing substance findings are positive (fructosuria) in those with fructose 1-phosphate aldolase deficiency ( ...
... is not to be confused with hereditary fructose intolerance, a potentially fatal condition in which the ... Foods with high fructose-to-glucose ratio. Glucose enhances absorption of fructose, so fructose from foods with fructose-to- ... Intolerance to fructose was first identified and reported in 1956. Similarity in symptoms means that patients with fructose ... Hereditary fructose intolerance is quite rare, affecting up to 1 in 20,000 to 30,000 people.[citation needed] Fructose ...
Another carbohydrate intolerance caused by enzyme deficiency is hereditary fructose intolerance. Celiac disease, an autoimmune ... Diagnosis of food intolerance can include hydrogen breath testing for lactose intolerance and fructose malabsorption, ... Drug intolerance Egg intolerance Elimination diet Favism Fructose malabsorption Gluten sensitivity Gluten-sensitive enteropathy ... Fructose intolerance Histamine intolerance, also related to biogenic amine intolerance (BAI) Lactose intolerance Orthorexia ...
Morris RC (1968). "An experimental renal acidification defect in patients with hereditary fructose intolerance. I. Its ... Hereditary fructose intolerance Lowe syndrome Tyrosinemia Wilson's disease Acquired disorders Amyloidosis Multiple myeloma ...
Fructose malabsorption Lactose intolerance Wright EM, Turk E, Martin MG (2002). "Molecular basis for glucose-galactose ... Sucrose is broken down into glucose and another simple sugar called fructose, and lactose is broken down into glucose and ... However, they are able to digest fructose-based formulas that do not contain glucose or galactose. Some affected children are ... Treatment mainly consists of introducing formulas that are based on fructose and a regular diet deficient in glucose and ...
In hereditary fructose intolerance caused by defects in aldolase B, fructose 1-phosphate accumulates in the liver and causes a ... Symptoms of hereditary fructose intolerance are apathy, drowsiness, sweatiness and tremulousness. "Marks A. Basic Medical ... Fructose-1-phosphate is a derivative of fructose. It is generated mainly by hepatic fructokinase but is also generated in ... Metabolism of fructose thus essentially results in intermediates of glycolysis. This means that fructose has the same fate as ...
Breath hydrogen tests are utilized to determine lactose, fructose, and/or sucrose intolerance. Small intestinal bacterial ... "Lactose intolerance". Genetics Home Reference. Retrieved 2020-08-03. "Glucose-galactose malabsorption , Genetic and Rare ... fructose, and sucrose). Dietary fiber and fat can be increased and fluid intake, especially fruit juice intake, decreased. With ... lactose intolerance) Medications, especially antibiotics Infection exposure (e.g. travel) Evaluation of past medical history ...
Conditions that can present similarly include galactosaemia, hereditary fructose intolerance, cystic fibrosis, and biliary ...
Dietary fructose intolerance occurs when there is a deficiency in the amount of fructose carrier. Lactose intolerance is the ... Fructose, on the other hand, crosses the apical membrane of the enterocyte, using GLUT5. It is thought to cross into the blood ...
Defects in this gene have been identified as the cause of hereditary fructose intolerance (HFI). The generic fructose ... Patient prognosis, however, is good in cases of hereditary fructose intolerance. By avoiding foods containing fructose, sucrose ... Cross NC, Stojanov LM, Cox TM (1990). "A new aldolase B variant, N334K, is a common cause of hereditary fructose intolerance in ... Cross NC, Tolan DR, Cox TM (1988). "Catalytic deficiency of human aldolase B in hereditary fructose intolerance caused by a ...
Simultaneously, food allergy, cross-reactions with pollen, fructose malabsorption, lactose intolerance, and celiac disease ... Histamine intolerance is sometimes informally called an allergy; however, the intolerance is technically caused by the gradual ... Roughly 1% of the population has histamine intolerance; of those, 80% are middle-aged. The imbalance in histamine intolerance ... Histamine intolerance, sometimes called histaminosis, is an over-accumulation of dietary histamine in the human body. ...
Stomach bypass surgery or hereditary fructose intolerance are believed to be causes, albeit uncommon, of reactive hypoglycemia ... hereditary fructose intolerance, galactosemia, and leucine sensitivity of childhood) Late hypoglycemia (occult diabetes; ...
Patients who are allergic to peanut, soya or with rare hereditary fructose intolerance should not take this medicine. It is ...
Hereditary fructose intolerance (HFI) results in poor feeding, failure to thrive, chronic liver disease and chronic kidney ... Fructose malabsorption is a digestive disorder in which absorption of fructose is impaired by deficient fructose carriers in ... Infants and adults are asymptomatic unless they ingest fructose or sucrose. Deficiency of hepatic fructose 1,6-biphosphate ( ... Three autosomal recessive disorders impair fructose metabolism in liver cells. The most common is caused by mutations in the ...
Additional contraindications for the children's formulation are hereditary fructose intolerance, glucose-galactose ...
Fructose malabsorption Digestive system diseases Lactose intolerance Baudon JJ, Veinberg F, Thioulouse E, Morgant G, Aymard P, ... secondary sucrose intolerance). There are specific tests used to help determine if a person has sucrose intolerance. The most ... so-called primary sucrose intolerance). Sucrose intolerance can also be caused by irritable bowel syndrome, aging, or small ... Sucrose intolerance or genetic sucrase-isomaltase deficiency (GSID) is the condition in which sucrase-isomaltase, an enzyme ...
Fructose malabsorption or Dietary Fructose Intolerance is a dietary disability of the small intestine, where the amount of ... GLUT5 is a fructose transporter expressed on the apical border of enterocytes in the small intestine. GLUT5 allows for fructose ... The absorption of fructose in the simultaneous presence of glucose is improved, while sorbitol is inhibitory. Fructose ... Fructose uptake rate by GLUT5 is significantly affected by diabetes mellitus, hypertension, obesity, fructose malabsorption, ...
Among the public health concerns cited were those individuals with hereditary fructose intolerance or fructose malabsorption, ... Another enzyme is then used to convert varying fractions of glucose into fructose...High-fructose corn syrup just doesn't exist ... who have been advised to avoid ingredients that contain fructose. While individuals with fructose allergies have associated " ... Nutritionally, high-fructose corn syrup and sucrose may be identical." Since HFCS is present in a "staggering" amount of food ...
... and hereditary fructose intolerance. Two forms, Dent's disease and Lowe syndrome, are X linked. A recently described form of ...
... hereditary fructose intolerance, with R. Froesh, A. Labhart et al. (1957) pseudo-vitamin D deficiency with R. Illig et al. ( ...
Fructose intolerance may refer to: Fructose malabsorption, a digestive disorder of the small intestine in which the fructose ... liver enzymes that metabolise fructose This disambiguation page lists articles associated with the title Fructose intolerance. ... carrier in enterocytes is deficient Hereditary fructose intolerance, a hereditary condition caused by a deficiency of ...
Hereditary fructose intolerance is a condition that affects a persons ability to digest the sugar fructose. Explore symptoms, ... Hereditary fructose intolerance is a condition that affects a persons ability to digest the sugar fructose. Fructose is a ... Hereditary fructose intolerance should not be confused with a condition called fructose malabsorption. In people with fructose ... medlineplus.gov/genetics/condition/hereditary-fructose-intolerance/ Hereditary fructose intolerance. ...
I. An appeal was lodged by the opponent (appellant) against the decision of the opposition division rejecting the opposition against European patent No. 2 289 352 (the patent).. II. With their statement of grounds of appeal, the appellant requested that the decision under appeal be set aside and that the patent be revoked. Auxiliarly, oral proceedings were requested.. III. With their reply to the statement of grounds of appeal the patent proprietor (respondent) requested that the appeal be dismissed, i.e. the patent be maintained as granted, or, alternatively, that the patent be maintained in amended form on the basis of one of the sets of claims of auxiliary request 1 to 20, all filed with the reply to the statement of grounds of appeal. Auxiliarly, oral proceedings were requested.. IV. The board scheduled oral proceedings to be held on 17 May 2021 and issued a communication pursuant to Article 15(1) RPBA setting out its preliminary assessment of the appeal.. V. By letter dated 12 April 2021, ...
... postulating that the defect was a deficiency of hepatic fructose 1-aldolase. ... Clinical intolerance to fructose was initially described in 1956. The following year, researchers reported a familial incidence ... Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) * Sections Hereditary Fructose Intolerance ( ... Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency)) and Hereditary Fructose Intolerance (HFI) ( ...
The symptoms of fructose intolerance are very similar to another sugar intolerance - lactose, the sugar in milk. Many people ... Answer: ructose intolerance is common, but it is seldom diagnosed. One cause, hereditary fructose intolerance, is a genetic ... it is absorbed better by people with fructose intolerance.. Therefore, the goal in someone with inability to absorb fructose is ... Unlike lactose intolerance, which has a simple diagnostic breath test, there is no commonly available test for fructose ...
Hereditary Fructose Intolerance - Learn about the causes, symptoms, diagnosis & treatment from the MSD Manuals - Medical ... Symptoms of Hereditary Fructose Intolerance Ingesting more than tiny amounts of fructose or sucrose causes low blood sugar ... Treatment of Hereditary Fructose Intolerance *. Eliminating fructose, sucrose, and sorbitol from the diet ... Treatment of hereditary fructose intolerance involves excluding fructose (generally present in sweet fruits), sucrose, and ...
title = "The biochemical basis of hereditary fructose intolerance",. abstract = "Hereditary fructose intolerance is a rare, but ... N2 - Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by ... AB - Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by ... Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by ...
"Fructose Intolerance" by people in this website by year, and whether "Fructose Intolerance" was a major or minor topic of these ... "Fructose Intolerance" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical ... Below are the most recent publications written about "Fructose Intolerance" by people in Profiles. ... An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, ...
Concerned about the possibility of Fructose intolerance? Discover the indicators here. Explore our available tests today for a ... you may have a fructose intolerance, also known as fructose malabsorption. Often mistaken for an allergy, fructose intolerance ... What is fructose intolerance?. Fructose is a natural sugar found in many fruits and vegetables. The body converts fructose into ... What to eat and what not to eat with a fructose intolerance. If you have fructose intolerance, youll need to avoid food and ...
... either due limits in the transport system or too much fructose in the diet. ... Fructose Intolerance refers to the limited absorption of fructose, ... Fructose intolerance Fructose intolerance happens when the fruit sugar fructose is poorly absorbed in the small intestine ... Fructose Intolerance refers to the limited absorption of fructose, either due to limits in the transport system or too much ...
Fructose Intolerance - Fructose-free nutrition - Products, information and advice! ... Your manufacturer for fructose-free foods and ingredients - ... Fructose Intolerance * Types of Fructose Intolerance * Fructose ... Our company was founded to provide good snack foods for people with hereditary fructose intolerance, so our fructose levels are ... Our food is made specially for people who suffer from hereditary fructose intolerance (HFI), fructose malabsorption, or are ...
... such as fructose intolerance. Check which of our products to suit you best. ... You can still follow the Optifast program if you have allergies or intolerances, ... Fructose Malabsorption. You can still follow the OPTIFAST VLCD program if you have allergies or intolerances. We recommend ... Intolerance. You can still follow the OPTIFAST VLCD program if you have allergies or intolerances. We recommend working with ...
... postulating that the defect was a deficiency of hepatic fructose 1-aldolase. ... Clinical intolerance to fructose was initially described in 1956. The following year, researchers reported a familial incidence ... Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) * Sections Fructose 1-Phosphate Aldolase Deficiency (Fructose ... Fructose Intolerance)) and Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) What to Read Next on Medscape ...
Thanks for sharing your story!! Im recently diagnosed with fructose intolerance (in addition to gluten and lactose intolerance ... Then in 2006 I was diagnosed with fructose intolerance (no fruit across the board, and very limited veggies in my diet). Even ... Then after another Mayo Clinic quest, I was tested & diagnosed with fructose intolerance. I am feeling so much better following ... was wondering if anyone else with fructose intolerance had found this a problem. thanks again ...
An Equal Eats Russian Fructose Intolerance Card helps communicate your allergy in foreign languages so you are understood when ... Travel with a fructose intolerance can be difficult due to language barriers. Keep a list of which foods to avoid with fructose ... "Fructose Intolerance in Russian. Fructose Intolerant Card for Russia","width":1200,"height":1200,"src":"\/\/equaleats.com\/cdn ... "Fructose Intolerance in Russian. Fructose Intolerant Card for Russia","id":20888522784942,"position":1,"preview_image":{"aspect ...
Fructose intolerance. Glycogen storage diseases. Glycogen storage disease type 1. Pompe disease ...
Hereditary Fructose Intolerance and our test Hereditary fructose intolerance is a rare genetic disorder. It is characterized by ... Hereditary Fructose Intolerance Herlitz Junctional Epidermolysis Bullosa (LAMB3-Related) Leigh Syndrome, French Canadian Type ... low blood sugar levels, stomach pain, and vomiting after eating fructose. A person must have two variants in the ALDOB gene in ...
Fructose intolerance. If your child is fructose intolerant, his or her doctor will recommend reducing or avoiding foods and ... Food allergies and intolerances. Food allergies. If your child has a food allergy, his or her doctor will recommend avoiding ... Lactose intolerance. If your child is lactose intolerant, his or her doctor will recommend reducing or avoiding foods and ... Sucrose intolerance. If your child is sucrose intolerant, his or her doctor will recommend reducing or avoiding foods and ...
Fructose [pharmacokinetics] *Fructose Intolerance [complications] [diet therapy] *Humans. *Irritable Bowel Syndrome [diet ... of patients had fructose, lactose malabsorption or small intestinal bacterial overgrowth respectively. Fructose malabsorption ... METHODS: Irritable bowel syndrome patients, who had performed hydrogen/methane breath testing for fructose and lactose ...
Intolerance to carbohydrates is the most common type of non-immune-mediated AFR. Carbohydrate intolerances with early onset and ... The Panel also includes the gene for hereditary fructose intolerance (ALDOB), which is characterized by metabolic disturbances ... following dietary exposure to fructose, sucrose, or sorbitol. Also genes for genetic hypergalactosemias are included (GALT, ...
Test For Lactose Intolerance. *● Test For Fructose Intolerance. *● 5 Days Mon - Fri ...
Lactose intolerance and malabsorption. 25 or 50 g lactose. Fructose intolerance and malabsorption. 25-50 g fructose. ... 2020) investigated lactose malabsorption and intolerance in 84 patients before undergoing RYGB and 84 patients after undergoing ... 13C-lactose for lactose intolerance, 13C-lactose ureide for OCTT, and 13C-xylose for bacterial overgrowth in the intestine ( ...
leaf extract in fructose-induced diabetic rats. Pharmacognosy Magazine, 14 (59). pp. 649-657. ISSN 0976-4062 ... Naveen, Y. P. and Asna Urooj (2018) Normalization of insulin resistance, glucose intolerance, and lipid profile by Swietenia ... Normalization of insulin resistance, glucose intolerance, and lipid profile by Swietenia Mahagoni (l.) jacq. leaf extract in ... in fructose-induced diabetic rats with insulin resistance. MAE was analyzed using high-performance liquid chromatography (HPLC ...
A food intolerance. • Bacterial overgrowth. • Irritable bowel syndrome (IBS). • Fructose intolerance. • Lactose intolerance. • ...
Fructose intolerance. *Tyrosinemia. *Wilson disease. *Alpha1-antitrypsin deficiency. *Nonalcoholic fatty liver disease (NAFLD) ...
Oral suspension and hereditary fructose intolerance. *Fructose can be harmful to patients with hereditary fructose intolerance ... this is above the daily maximum limit of sorbitol for patients with hereditary fructose intolerance, and may cause dyspepsia ...
The threshold of fructose intolerance can vary widely. For some people, intolerance can be triggered by just a few grams and ... 20 g fructose.. The decision regarding the consumption of Complete products by those with a fructose intolerance must ... 5. Do Complete by Juice Plus+ products contain fructose? What does this mean for people with fructose intolerance?. ... The types of sugar provided are sucrose, glucose and fructose.. - A ready to eat portion of Complete Vegetable Soup prepared ...
What Is Fructose Intolerance?. Fructose intolerance is when you cant properly digest fructose, which can create digestive ... What Are the Symptoms of Fructose Intolerance?. Symptoms of fructose intolerance can include gas, bloating and loose stools, ... How is fructose intolerance diagnosed?. Integrative medicine expert Aviva Romm, MD, discusses which main culprits to eliminate ...
After 20 g fructose, patients with history of fruit intolerance produce more breath hydrogen, pass flatus more frequently, and ... We recorded breath hydrogen, flatus passage and bloating after 20 g fructose in 8 patients with history of fruit intolerance ... and hydrogen production after oral fructose in patients with history of fruit intolerance and compare these parameters with ... Reproducibility of lactulose and fructose breath hydrogen testing and impact on clinical utility. June 2016 · Journal of ...
2018). Fructose intolerance: Cause or cure of chronic functional constipation.. https://www.ncbi.nlm.nih.gov/pmc/articles/ ... Pears are high in sorbitol and fructose, which may have laxative properties. Fructose is a type of sugar that slowly gets ... This means that unabsorbed fructose may bring water into your intestines and loosen up. your stools. ...
  • Fructose intolerance may refer to: Fructose malabsorption, a digestive disorder of the small intestine in which the fructose carrier in enterocytes is deficient Hereditary fructose intolerance, a hereditary condition caused by a deficiency of liver enzymes that metabolise fructose This disambiguation page lists articles associated with the title Fructose intolerance. (wikipedia.org)
  • Hereditary fructose intolerance should not be confused with a condition called fructose malabsorption. (medlineplus.gov)
  • In people with fructose malabsorption, the cells of the intestine cannot absorb fructose normally, leading to bloating, diarrhea or constipation, flatulence, and stomach pain. (medlineplus.gov)
  • Dear Dr. Roach hat can you tell me about fructose intolerance and/or malabsorption? (signalscv.com)
  • If you experience unpleasant or uncomfortable symptoms after eating food that contains fructose, such as fruit, sweets or bread, you may have a fructose intolerance, also known as fructose malabsorption. (checkmybodyhealth.com)
  • When the body struggles to do this it doesn't absorb all the fructose, and this is known as a fructose intolerance, or fructose malabsorption. (checkmybodyhealth.com)
  • Fructose malabsorption is relatively common, and it affects around one in three people . (checkmybodyhealth.com)
  • However, fructose malabsorption can develop at any stage of your life. (checkmybodyhealth.com)
  • However, fructose malabsorption isn't the same as an allergy. (checkmybodyhealth.com)
  • Our food is made specially for people who suffer from hereditary fructose intolerance (HFI), fructose malabsorption, or are following a low FODMAP diet. (frusano.com)
  • Fructose malabsorption and intolerance: effects of fructose with and without simultaneous glucose ingestion. (medscape.com)
  • Molecular analysis of the fructose transporter gene(GLUT5) in isolated fructose malabsorption. (medscape.com)
  • Developmental changes and fructose absoprtion in children: effect on malabsorption testing and dietary management. (medscape.com)
  • Fructose intolerance/malabsorption and recurrent abdominal pain in children. (medscape.com)
  • METHODS: Irritable bowel syndrome patients, who had performed hydrogen/methane breath testing for fructose and lactose malabsorption and had received dietary advice regarding the low FODMAP diet, were included. (medscape.com)
  • 75.6%, 37.8% and 13.3% of patients had fructose, lactose malabsorption or small intestinal bacterial overgrowth respectively. (medscape.com)
  • Carbohydrate intolerances with early onset and genetic cause include congenital sucrase-isomaltase deficiency (CSID), glucose-galactose malabsorption (GGM) and congenital lactase deficiency (CLD). (blueprintgenetics.com)
  • This blog is to share the recipes I have found that work for me through many hours of experimentation in the kitchen that are suitable for people with fructose intolerances or fructose malabsorption. (blogger.com)
  • Individuals with fructose malabsorption or fructose intolerance may experience gastrointestinal symptoms such as bloating, abdominal pain, or diarrhea after consuming honeydew melon. (kobmel.com)
  • As the tablet coat contains sucrose (41.2 mg), patients with rare hereditary problems of fructose intolerance, glucose-galactose malabsorption or sucrase- isomaltase insufficiency should not take Buscopan Tablets. (janusinfo.se)
  • Although lactose is the carbohydrate base in most infant formulas, some (eg, soy formulas) contain sucrose, a fructose-glucose disaccharide that may cause symptoms. (medscape.com)
  • Fructose is also found in honey and is part of the disaccharide sucrose (table sugar), where it is combined with glucose. (signalscv.com)
  • Fructose is a sugar present in table sugar (sucrose), honey, and many fruits. (msdmanuals.com)
  • Ingesting more than tiny amounts of fructose or sucrose causes low blood sugar levels ( hypoglycemia ), with sweating, confusion, and sometimes seizures and coma. (msdmanuals.com)
  • Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet. (ouhsc.edu)
  • Everything we make is extremely low in fructose, sucrose and sorbitol, making it appropriate for a low FODMAP diet. (frusano.com)
  • Full transparency: We list all ingredients and provide full nutritional information about all contained sugars, including the unavoidable, low residual content of fructose, sucrose, sorbitol and lactose in every product description. (frusano.com)
  • In children and some sensitive people, too much fructose and sucrose from high intakes of certain fruits produces these unpleasant symptoms. (paediatricnutrition.com)
  • Sucrose is made of one fructose unit and one glucose unit and so consuming excess sugar itself may give rise to similar symptoms. (paediatricnutrition.com)
  • Most potatoes contain over 1000 mg of fructose and are also composed of sucrose, which breaks down into equal parts glucose and fructose. (blogspot.com)
  • chronic growth restriction/failure to thrive) following dietary exposure to fructose, sucrose, or sorbitol. (blueprintgenetics.com)
  • The type of sugar present is predominantly sucrose, with small amounts of glucose and fructose. (juiceplus.com)
  • The types of sugar provided are fructose, glucose and sucrose. (juiceplus.com)
  • The biochemical basis of hereditary fructose intolerance. (medlineplus.gov)
  • Timson, David J. / The biochemical basis of hereditary fructose intolerance . (brighton.ac.uk)
  • Tolan DR. Molecular basis of hereditary fructose intolerance: mutations and polymorphisms in the human aldolase B gene. (medscape.com)
  • The combination of decreased cellular energy, low blood sugar, and liver cell death leads to the features of hereditary fructose intolerance. (medlineplus.gov)
  • This review gives a general overview of the features of hereditary fructose intolerance, then concentrates on the biochemistry of the AP variant (Ala149Pro variant of aldolase B) and molecular pathological consequences of mutation of the aldolase B gene. (brighton.ac.uk)
  • The absence of the latter function readily explains the clinical hypoglycemia in individuals with hereditary fructose intolerance. (medscape.com)
  • The rapid early progress in the understanding of this disorder may have occurred because of the fairly dramatic and difficult-to-miss symptoms associated with fructose ingestion. (medscape.com)
  • Thus, in an infant who is homozygous for fructose 1-aldolase deficiency, fructose ingestion triggers a cascade of biochemical events that result in severe clinical disease. (medscape.com)
  • The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. (ouhsc.edu)
  • Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. (ouhsc.edu)
  • Positive result of the aCPQ test after 25 g fructose ingestion. (who.int)
  • Fructose intolerance symptoms can show in babies as soon as they're introduced to food or baby formula, which often contains fructose. (checkmybodyhealth.com)
  • Honeydew melon contains fructose, a natural sugar. (kobmel.com)
  • The following year, researchers reported a familial incidence of the disorder in several family members, postulating that the defect was a deficiency of hepatic fructose 1-aldolase. (medscape.com)
  • Fructokinase deficiency, which is a type of hereditary fructose intolerance, does not cause any symptoms. (msdmanuals.com)
  • Other causes include vitamin D deficiency, fructose intolerance, some medicines, and some metabolic conditions that run in families. (teenshealth.org)
  • Milk intolerance is more frequently due to milk-protein allergy than primary lactase deficiency. (medscape.com)
  • Unlike lactose intolerance, which has a simple diagnostic breath test, there is no commonly available test for fructose intolerance. (signalscv.com)
  • How do you test for fructose intolerance? (checkmybodyhealth.com)
  • Mutations in the ALDOB gene cause hereditary fructose intolerance. (medlineplus.gov)
  • ALDOB gene mutations reduce the function of the enzyme, impairing its ability to metabolize fructose. (medlineplus.gov)
  • The spectrum of aldolase B (ALDOB) mutations and the prevalence of hereditary fructose intolerance in Central Europe. (medscape.com)
  • A Novel Frameshift Mutation of the ALDOB Gene in a Korean Girl Presenting with Recurrent Hepatitis Diagnosed as Hereditary Fructose Intolerance. (medscape.com)
  • Continued exposure to fructose may result in seizures, coma, and ultimately death from liver and kidney failure. (medlineplus.gov)
  • A lack of functional aldolase B results in an accumulation of fructose-1-phosphate in liver cells. (medlineplus.gov)
  • Within the next 4-5 years, the enzyme defect in aldolase B isozyme in the liver was demonstrated, and hereditary fructose intolerance (HFI) became recognized as a distinct clinical entity. (medscape.com)
  • Very small amounts of fructose cause low blood sugar levels and can lead to kidney and liver damage. (msdmanuals.com)
  • Doctors base the diagnosis of hereditary fructose intolerance on the person's symptoms after consuming fructose and on a chemical analysis of a sample of liver tissue. (msdmanuals.com)
  • In adults, fructose intolerance symptoms have a lot in common with liver disease. (checkmybodyhealth.com)
  • Fructose is a type of sugar that slowly gets absorbed because your liver metabolizes the bulk of it. (healthline.com)
  • Sorbitol, a sugar alcohol used as a sweetener in "diet" or "sugarless" foods, tends to worsen fructose intolerance and should be avoided. (signalscv.com)
  • Fructose is also present in sorbitol (a sugar substitute). (msdmanuals.com)
  • We do not use refined sugar nor artificial sweeteners nor alditols (e.g. sorbitol) nor, this goes without saying, high-fructose corn syrups (hfcs). (frusano.com)
  • Pears are high in sorbitol and fructose, which may have laxative properties. (healthline.com)
  • The cause of severe hepatic dysfunction remains unknown but may be a manifestation of focal cytoplasmic degeneration and cellular fructose toxicity. (medscape.com)
  • An inborn defect of hepatic fructose-1-phosphate splitting aldolase. (medscape.com)
  • Affected individuals develop signs and symptoms of the disorder in infancy when fruits, juices, or other foods containing fructose are introduced into the diet. (medlineplus.gov)
  • Due to the severity of symptoms experienced when fructose is ingested, most people with hereditary fructose intolerance develop a dislike for fruits, juices, and other foods containing fructose. (medlineplus.gov)
  • Because I was misdiagnosed for most of my life, Gluten & Fructose took a toll on my system and caused other health problems. (blogspot.com)
  • She has lactose, fructose and gluten intolerance. (britishcouncil.org)
  • and is involved in the breakdown (metabolism) of fructose so this sugar can be used as energy. (medlineplus.gov)
  • Aldolase B is responsible for the second step in the metabolism of fructose, which breaks down the molecule fructose-1-phosphate into other molecules called glyceraldehyde and dihydroxyacetone phosphate. (medlineplus.gov)
  • Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose metabolism, caused by mutation of the aldolase B gene. (brighton.ac.uk)
  • An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. (ouhsc.edu)
  • An inborn error of fructose metabolism. (medscape.com)
  • Fructose intolerance in children presenting with abdominal pain. (medscape.com)
  • In this post, we're going to explore what a fructose intolerance is, including the symptoms, which foods contain fructose and what you can do to find out for sure whether or not you have one. (checkmybodyhealth.com)
  • If your child is fructose intolerant , his or her doctor will recommend reducing or avoiding foods and beverages that contain fructose . (nih.gov)
  • 5. Do Complete by Juice Plus+ products contain fructose? (juiceplus.com)
  • Thus, homozygous neonates remain clinically well until confronted with dietary sources of fructose. (medscape.com)
  • Dietary fructose intolerance (DFI) - when the intestine finds it difficult to process fructose. (checkmybodyhealth.com)
  • Tsampalieros A, Beauchamp J, Boland M, Mack DR. Dietary fructose intolerance in children and adolescents. (medscape.com)
  • Coffee EM, Yerkes L, Ewen EP, Zee T, Tolan DR. Increased prevalence of mutant null alleles that cause hereditary fructose intolerance in the American population. (medlineplus.gov)
  • On average, both African Americans and Hispanic Americans consume less than the recommended levels of dairy foods, and perceived or actual lactose intolerance can be a primary reason for limiting or avoiding dairy intake, while true lactose intolerance prevalence is not known in these populations. (medscape.com)
  • that is caused by a lack of the enzyme needed to metabolize fructose. (msdmanuals.com)
  • Children who have one of these disorders are missing one of the enzymes needed to break down (metabolize) fructose. (msdmanuals.com)
  • and condensation of the triose phosphates, glyceraldehyde phosphate, and dihydroxyacetone phosphate to form fructose 1,6-diphosphate). (medscape.com)
  • Aldolase is an enzyme responsible for breaking down glucose products into energy, specifically converting fructose 1,6-bisphosphate into the triose phosphates dihydroxyacetone phosphate (DHAP) and glyceraldehydes 3-phosphate. (medscape.com)
  • Aldolase is a cytoplasmic enzyme responsible for converting sugar into energy, specifically splitting aldol, fructose 1,6-bisphosphate, into the triose phosphates dihydroxyacetone phosphate (DHAP) and glyceraldehydes 3-phosphate (a reversible reaction). (medscape.com)
  • One cause, hereditary fructose intolerance, is a genetic disorder that can cause serious symptoms in infants and children, but it is usually mild in adults. (signalscv.com)
  • A food intolerance is a negative reaction to a food, drink, or compound within a food. (healthspan.co.uk)
  • A food intolerance is usually caused by an inability to digest and absorb a food properly. (healthspan.co.uk)
  • How does a food intolerance develop? (healthspan.co.uk)
  • Unlike allergies, which generally occur from an early age, a food intolerance can develop at any time. (healthspan.co.uk)
  • Fructose is a simple sugar found primarily in fruits. (medlineplus.gov)
  • Fructose is a natural sugar found in many fruits and vegetables. (checkmybodyhealth.com)
  • It is not recommended or easy to exclude fructose from the diet completely, because fruits and vegetables are part of a healthy diet. (paediatricnutrition.com)
  • We take a closer look at the gut bacteria that help to maintain a healthy gut, their role in minimising food intolerances, and the steps you can take to try to heal your digestive tract. (healthspan.co.uk)
  • If intolerances are caused by a digestive issue, healing the gut could be an important step in reducing or even eliminating an intolerance. (healthspan.co.uk)
  • Therefore, the goal in someone with inability to absorb fructose is to reduce or avoid fructose by itself. (signalscv.com)
  • Someone who has a fructose intolerance, for example, cannot absorb every bit of fructose and some therefore remains in the gut. (healthspan.co.uk)
  • Reduced cleavage of F-1-P leads to its cellular accumulation and fructokinase inhibition, causing free fructose accumulation in the blood. (medscape.com)
  • Kim Y, Park SC, Wolf BW, Hertzler SR. Combination of erythritol and fructose increases gastrointestinal symptoms in healthy adults. (medscape.com)
  • Coffee EM, Tolan DR. Mutations in the promoter region of the aldolase B gene that cause hereditary fructose intolerance. (medscape.com)
  • A fructose intolerant card in Russian helps bridge the communication gap, ensuring your request is noted, remembered, and understood. (equaleats.com)
  • The main way to test whether you've developed fructose intolerance is with a breath test. (checkmybodyhealth.com)
  • Fructose breath hydrogen test - is it really a harmless diagnostic procedure? (medscape.com)
  • Hereditary fructose intolerance is a condition that affects a person's ability to digest the sugar fructose. (medlineplus.gov)
  • Clinical intolerance to fructose was initially described in 1956. (medscape.com)
  • Fructose intolerance happens when the fruit sugar fructose is poorly absorbed in the small intestine causing symptoms of diarrhoea, bloating and wind. (paediatricnutrition.com)
  • Fructose that passes into the large intestine unabsorbed in the small intestine, provides food for the intestinal bacteria, which ferment the sugar into gases and acids. (paediatricnutrition.com)
  • What is fascinating yet poorly understood is that when fructose is consumed with glucose, it is absorbed better by people with fructose intolerance. (signalscv.com)
  • This graph shows the total number of publications written about "Fructose Intolerance" by people in this website by year, and whether "Fructose Intolerance" was a major or minor topic of these publications. (ouhsc.edu)
  • Below are the most recent publications written about "Fructose Intolerance" by people in Profiles. (ouhsc.edu)
  • Between 1970 and 1990, the consumption of fructose grew by over 1,000% , which is thought to be the reason so many people experience symptoms when eating fructose-containing foods today. (checkmybodyhealth.com)
  • Because the symptoms of fructose intolerance have crossover with symptoms of an allergy, many people confuse the two. (checkmybodyhealth.com)
  • Our company was founded to provide good snack foods for people with hereditary fructose intolerance, so our fructose levels are extremely low. (frusano.com)
  • Fructose is not fully absorbed in most people, but does not usually give rise to symptoms. (paediatricnutrition.com)
  • What does this mean for people with fructose intolerance? (juiceplus.com)
  • For some people, intolerance can be triggered by just a few grams and for others, only after taking daily amounts well in excess of 10 g. (juiceplus.com)
  • However, symptoms of lactose intolerance rarely develop in people younger than 6 years. (medscape.com)
  • The general consensus is that a healthy gut supports a healthy body, and for some people an unhappy gut might even lead to the development of food intolerances. (healthspan.co.uk)
  • Some people may even experience intolerances for years before ever being diagnosed. (healthspan.co.uk)
  • Keep a list of which foods to avoid with fructose intolerance with an Equal Eats card. (equaleats.com)
  • Hereditary fructose intolerance (HF) - when the body amasses sugars that it cannot process, which can lead to severe symptoms and complications. (checkmybodyhealth.com)
  • Steinmann B, Gitzelmann R. The diagnosis of hereditary fructose intolerance. (medscape.com)
  • Treatment includes avoiding fructose in the diet and, when needed, taking glucose tablets. (msdmanuals.com)
  • Since 2006, Frusano has been developing and curating food specifically designed for those on a low fructose diet. (frusano.com)
  • Fructose Intolerance refers to the limited absorption of fructose, either due to limits in the transport system or too much fructose in the diet. (paediatricnutrition.com)
  • The treatment is a diet low in fructose, achieved by reducing fruit and vegetable intakes, especially fruit juices, dried fruit and honey. (paediatricnutrition.com)
  • Following a diet low in fructose for several weeks, once symptoms have completely resolved, fructose may be reintroduced in a controlled way. (paediatricnutrition.com)
  • Then in 2006 I was diagnosed with fructose intolerance (no fruit across the board, and very limited veggies in my diet). (blogspot.com)
  • Although transient lactose intolerance may occur during acute gastroenteritis and as part of any process that leads to reduction of the small intestinal absorptive surface (such as untreated celiac disease ), it is rarely clinically significant and, when present, can be easily treated with a short course of a lactose-free diet. (medscape.com)
  • The decision regarding the consumption of Complete products by those with a fructose intolerance must ultimately be made by the customer or their medical practitioner. (juiceplus.com)
  • Lactose intolerance in adulthood is very common and is the result of a genetically programmed progressive loss of the activity of the small intestinal enzyme lactase. (medscape.com)
  • Most children with lactose intolerance can tolerate some amount of lactose in what they eat or drink. (nih.gov)