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Hereditary Fructose Intolerance

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1. Are heterozygous carriers for hereditary fructose intolerance predisposed to metabolic disturbances when exposed to fructose? (PubMed)

Are heterozygous carriers for hereditary fructose intolerance predisposed to metabolic disturbances when exposed to fructose? High fructose intake causes hepatic insulin resistance and increases postprandial blood glucose, lactate, triglyceride, and uric acid concentrations. Uric acid may contribute to insulin resistance and dyslipidemia in the general population. In patients with hereditary fructose intolerance, fructose consumption is associated with acute hypoglycemia, renal tubular acidosis (...) , and hyperuricemia.We investigated whether asymptomatic carriers for hereditary fructose intolerance (HFI) would have a higher sensitivity to adverse effects of fructose than would the general population.Eight subjects heterozygous for HFI (hHFI; 4 men, 4 women) and 8 control subjects received a low-fructose diet for 7 d and on the eighth day ingested a test meal, calculated to provide 25% of the basal energy requirement, containing 13C-labeled fructose (0.35 g/kg), glucose (0.35 g/kg), protein (0.21 g/kg

2018 American Journal of Clinical Nutrition

2. Fructose Supplementation in Carriers for Hereditary Fructose Intolerance

Fructose Supplementation in Carriers for Hereditary Fructose Intolerance Fructose Supplementation in Carriers for Hereditary Fructose Intolerance - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Fructose (...) Supplementation in Carriers for Hereditary Fructose Intolerance The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier: NCT03545581 Recruitment Status : Recruiting First Posted : June 4, 2018 Last Update Posted : November 14, 2018 See Sponsor

2018 Clinical Trials

3. Non-alcoholic fatty liver in hereditary fructose intolerance. (PubMed)

Non-alcoholic fatty liver in hereditary fructose intolerance. Non-alcoholic fatty liver disease (NAFLD) is characterized by fat accumulation affecting >5% of the liver volume that is not explained by alcohol abuse. It is known that fructose gives rise to NAFLD and it has been recently described that the ingestion of fructose in low amounts in aldolase B deficient mice is associated with the development of fatty liver. Therefore, it is reasonable that patients with HFI (Hereditary Fructose (...) Intolerance) present fatty liver at diagnosis, but its prevalence in patients treated and with adequate follow-up is not well documented in the literature. The aim of this study is to analyze the association between HFI and NAFLD in treated patients.A cross-sectional observational study was conducted. The population comprised 16 genetically diagnosed HFI patients aged from 3 years to 48 and in dietary treatment of fructose, sorbitol and sacarose exclusion at least for two years. Blood samples were

2019 Clinical nutrition (Edinburgh, Scotland)

4. Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance

Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance Metabolic Consequences of Heterozygous Hereditary Fructose Intolerance - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Metabolic (...) Consequences of Heterozygous Hereditary Fructose Intolerance The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02979106 Recruitment Status : Completed First Posted : December 1, 2016 Last Update Posted : December 29, 2017 Sponsor: University of Lausanne Information provided by (Responsible Party): Luc Tappy

2016 Clinical Trials

5. Hereditary Fructose Intolerance

Hereditary Fructose Intolerance Hereditary Fructose Intolerance Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Hereditary Fructose (...) Intolerance Hereditary Fructose Intolerance Aka: Hereditary Fructose Intolerance II. Pathophysiology Intolerance to fructose or sucrose III. Symptoms (follows sucrose or fructose exposure) s (see ) (Direct ) IV. Labs Hepatic fructose-1-phosphatase aldolase activity V. Management Fructose and sucrose eliminated from diet Images: Related links to external sites (from Bing) These images are a random sampling from a Bing search on the term "Hereditary Fructose Intolerance." Click on the image (or right click

2018 FP Notebook

6. Molecular Diagnosis of Hereditary Fructose Intolerance: Founder Mutation in a Community from India (PubMed)

Molecular Diagnosis of Hereditary Fructose Intolerance: Founder Mutation in a Community from India Hereditary fructose intolerance (HFI) is a difficult-to-confirm diagnosis, requiring either invasive liver biopsy-enzyme assay or potentially hazardous fructose challenge test or expensive molecular genetic analysis. Therefore, worldwide there has been a trend towards finding "common mutations" in distinct ethnic groups to simplify the process of diagnosis. The nonspecific presentation

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2015 JIMD reports

7. Fruit-Induced FPIES Masquerading as Hereditary Fructose Intolerance. (PubMed)

Fruit-Induced FPIES Masquerading as Hereditary Fructose Intolerance. Hereditary fructose intolerance (HFI) symptoms develop at first introduction of fruit during weaning. We report on an infant with suspected HFI who presented with repeated episodes of vomiting and hypotension after ingestion of fruit-containing meals. The first episode occurred at age 4 months. Despite negative genetic testing for HFI, strict avoidance of fruit ingestion resulted in lack of recurrence of symptoms. Oral (...) -fructose-tolerance testing conducted with an apple mousse did not determine hypoglycemia or fructosuria but caused severe hypotension. Allergy evaluations were negative, and the history was diagnostic for fruit-induced food protein-induced enterocolitis syndrome. Because this non-immunoglobulin E-mediated gastrointestinal food hypersensitivity manifests as profuse, repetitive vomiting, often with diarrhea, leading to acute dehydration and lethargy, it may be misinterpreted as HFI. We advise

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2014 Pediatrics

8. Ketohexokinase C blockade ameliorates fructose-induced metabolic dysfunction in fructose-sensitive mice (PubMed)

Ketohexokinase C blockade ameliorates fructose-induced metabolic dysfunction in fructose-sensitive mice Increasing evidence suggests a role for excessive intake of fructose in the Western diet as a contributor to the current epidemics of metabolic syndrome and obesity. Hereditary fructose intolerance (HFI) is a difficult and potentially lethal orphan disease associated with impaired fructose metabolism. In HFI, the deficiency of aldolase B results in the accumulation of intracellular (...) phosphorylated fructose, leading to phosphate sequestration and depletion, increased adenosine triphosphate (ATP) turnover, and a plethora of conditions that lead to clinical manifestations such as fatty liver, hyperuricemia, Fanconi syndrome, and severe hypoglycemia. Unfortunately, there is currently no treatment for HFI, and avoiding sugar and fructose has become challenging in our society. In this report, through use of genetically modified mice and pharmacological inhibitors, we demonstrate

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2018 The Journal of clinical investigation

9. Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Diagnosis)

Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Diagnosis) Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency): Background, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9yZWZlcmVuY2UubWVkc2NhcGUuY29tL2FydGljbGUvOTQ0NTQ4LW92ZXJ2aWV3 processing > Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Updated: Aug 10, 2017 Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD Share Email Print Feedback Close Sections Sections Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Overview Background Clinical intolerance to fructose was initially described in 1956. The following year, researchers reported a familial incidence of the disorder in several family members

2014 eMedicine Pediatrics

10. Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Treatment)

Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Treatment) Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Treatment & Management: Medical Care, Consultations, Diet Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9yZWZlcmVuY2UubWVkc2NhcGUuY29tL2FydGljbGUvOTQ0NTQ4LXRyZWF0bWVudA== processing > Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Treatment & Management Updated: Aug 10, 2017 Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD Share Email Print Feedback Close Sections Sections Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Treatment Medical Care Definitive treatment simply consists of eliminating fructose from the diet. Eliminating fructose early in the disease course totally restores

2014 eMedicine Pediatrics

11. Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Overview)

Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Overview) Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency): Background, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9yZWZlcmVuY2UubWVkc2NhcGUuY29tL2FydGljbGUvOTQ0NTQ4LW92ZXJ2aWV3 processing > Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Updated: Aug 10, 2017 Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD Share Email Print Feedback Close Sections Sections Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Overview Background Clinical intolerance to fructose was initially described in 1956. The following year, researchers reported a familial incidence of the disorder in several family members

2014 eMedicine Pediatrics

12. Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Follow-up)

Fructose 1-Phosphate Aldolase Deficiency (Fructose Intolerance) (Follow-up) Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile (...) &urlCache=aHR0cHM6Ly9yZWZlcmVuY2UubWVkc2NhcGUuY29tL2FydGljbGUvOTQ0NTQ4LWZvbGxvd3Vw processing > Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Follow-up Updated: Aug 10, 2017 Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD Share Email Print Feedback Close Sections Sections Hereditary Fructose Intolerance (HFI) (Fructose 1-Phosphate Aldolase Deficiency) Follow-up Further Outpatient Care Close dietary monitoring is important for a good outcome and should

2014 eMedicine Pediatrics

13. Effect of administration of the fructose on the glycogenolytic action of glucagon. An investigation of the pathogeny of hereditary fructose intolerance (PubMed)

Effect of administration of the fructose on the glycogenolytic action of glucagon. An investigation of the pathogeny of hereditary fructose intolerance 1. The mechanism by which the administration of fructose to patients with hereditary fructose intolerance makes them unresponsive to the hyperglycaemic action of glucagon was studied. In four patients, a 10-fold increase in the urinary excretion of cyclic AMP was induced by glucagon, but this effect was drastically decreased by the previous (...) of patients with hereditary fructose intolerance.

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1973 Biochemical Journal

14. Hereditary Fructose Intolerance

Hereditary Fructose Intolerance Hereditary Fructose Intolerance Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Hereditary Fructose (...) Intolerance Hereditary Fructose Intolerance Aka: Hereditary Fructose Intolerance II. Pathophysiology Intolerance to fructose or sucrose III. Symptoms (follows sucrose or fructose exposure) s (see ) (Direct ) IV. Labs Hepatic fructose-1-phosphatase aldolase activity V. Management Fructose and sucrose eliminated from diet Images: Related links to external sites (from Bing) These images are a random sampling from a Bing search on the term "Hereditary Fructose Intolerance." Click on the image (or right click

2015 FP Notebook

15. An experimental renal acidification defect in patients with hereditary fructose intolerance: I. Its resemblance to renal tubular acidosis (PubMed)

An experimental renal acidification defect in patients with hereditary fructose intolerance: I. Its resemblance to renal tubular acidosis In three unrelated patients with hereditary fructose intolerance (HFI), but in none of five normal subjects, the experimental administration of fructose invariably induced a reversible dysfunction of the renal tubule with biochemical and physiological characteristics of renal tubular acidosis. During a state of ammonium chloride-induced acidosis, (a) urinary (...) of fructose and disappeared afterward. The tubular dysfunction was not causally dependent on hypoglucosemia, ammonium chloride-induced acidosis or osmotic diuresis. Rather, it appeared causally related to the fructose-induced metabolic abnormality of patients with HFI. The causal enzymatic defect, the virtual absence of fructose-1-phosphate aldolase, occurs in the kidney as well as in the liver of patients with HFI.

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1968 Journal of Clinical Investigation

16. An experimental renal acidification defect in patients with hereditary fructose intolerance: II. Its distinction from classic renal tubular acidosis; its resemblance to the renal acidification defect associated with the fanconi syndrome of children with c (PubMed)

An experimental renal acidification defect in patients with hereditary fructose intolerance: II. Its distinction from classic renal tubular acidosis; its resemblance to the renal acidification defect associated with the fanconi syndrome of children with c In adult patients with hereditary fructose intolerance (HFI) fructose induces a renal acidification defect characterized by (a) a 20-30% reduction in tubular reabsorption of bicarbonate (T HCO(3) (-)) at plasma bicarbonate concentrations (...) renal tubular acidosis is characterized by (a) just less than complete tubular reabsorption of bicarbonate at plasma bicarbonate concentrations of 26 mEq/liter or less, (b) a normal Tm HCO(3) (-) of approximately 2.8 mEq/100 ml of glomerular filtrate, and (c) during acidosis of an even severe degree, a quantitatively trivial bicarbonaturia, as well as (d) a urinary pH of greater than 6. That the fructose-induced renal acidification defect involves a reduced H(+) secretory capacity of the proximal

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1968 Journal of Clinical Investigation

17. Manifestation of hereditary fructose intolerance. (PubMed)

Manifestation of hereditary fructose intolerance. 5576008 1971 07 06 2018 11 13 0007-1447 2 5759 1971 May 22 British medical journal Br Med J Manifestation of hereditary fructose intolerance. 446-7 Raju L L Chessells J M JM Kemball M M eng Case Reports Journal Article England Br Med J 0372673 0007-1447 30237-26-4 Fructose AIM IM Carbohydrate Metabolism, Inborn Errors complications pathology Female Fructose metabolism Hemorrhagic Disorders etiology Humans Infant, Newborn Infant, Newborn

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1971 British medical journal

18. Modulation of Experimental Renal Dysfunction of Hereditary Fructose Intolerance by Circulating Parathyroid Hormone (PubMed)

Modulation of Experimental Renal Dysfunction of Hereditary Fructose Intolerance by Circulating Parathyroid Hormone In a woman with hereditary fructose intolerance and intact parathyroid function, the experimental administration of fructose at different dosage schedules invariably induced the dose-dependent, complex dysfunction of the proximal renal tubule now recognized as characteristic. But in a woman with hereditary fructose intolerance and hypoparathyroidism given similar amounts (...) of fructose, the experimental dysfunction was strikingly attenuated or nondemonstrable unless or until fructose and parathyroid hormone were administered in sustained combination. Thereupon, a renal dysfunction of characteristic type and severity occurred invariably and almost immediately. Thus, the concentration of circulating parathyroid hormone can modulate the functional expression of the experimental renal disorder. This effect of parathyroid hormone, which appears to involve more than simple

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1971 Proceedings of the National Academy of Sciences of the United States of America

19. High liver glycogen in hereditary fructose intolerance (PubMed)

High liver glycogen in hereditary fructose intolerance A case of hereditary fructose intolerance is reported in a girl aged 2 years at the time of her death. She had apparently progressed normally until the age of 14 months. At 19 months she was admitted to hospital with failure to thrive, hepatomegaly, and superficial infections. Investigations revealed hypoglycaemia, persistent acidosis, aminoaciduria, and a high liver glycogen level which suggested that she had glycogen storage disease (...) . There was also some evidence of malabsorption. At necropsy the liver enzyme estimations showed that fructose 1-phosphate aldolase activity was absent and that fructose 1,6-diphosphate aldolase activity was reduced. Hereditary fructose intolerance and glycogen storage disease have been confused in the past on clinical grounds, but a high liver glycogen level has not previously been reported in hereditary fructose intolerance.

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1971 Gut

20. Animal model of human disease: hereditary fructose intolerance. (PubMed)

Animal model of human disease: hereditary fructose intolerance. 4835994 1974 08 26 2018 11 13 0002-9440 75 3 1974 Jun The American journal of pathology Am. J. Pathol. Animal model of human disease: hereditary fructose intolerance. 591-4 Phillips M J MJ Yu D T DT eng Journal Article United States Am J Pathol 0370502 0002-9440 30237-26-4 Fructose 8L70Q75FXE Adenosine Triphosphate IY9XDZ35W2 Glucose AIM IM Adenosine Triphosphate metabolism Animals Carbohydrate Metabolism, Inborn Errors genetics (...) pathology Disease Models, Animal Fructose metabolism Glucose metabolism Liver metabolism pathology Male Rats 1974 6 1 1974 6 1 0 1 1974 6 1 0 0 ppublish 4835994 PMC1910841 Nature. 1967 May 27;214(5091):920-1 6054986 Scand J Gastroenterol. 1968;3(1):80-2 5655259 Am J Med. 1968 Jun;44(6):910-21 5656202 Science. 1968 Sep 20;161(3847):1253-4 5673437 Biochem Biophys Res Commun. 1969 Mar 10;34(5):619-26 5777779 J Ultrastruct Res. 1971 Jul;36(1):222-36 5568356 Lab Invest. 1974 Jan;30(1):85-92 4812811 Enzymol

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1974 The American journal of pathology

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