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Clinical Practice Guidelines for the Diagnosis and Management of Hereditary Fructose Intolerance. [PDF]

Diseases
Introduction: Hereditary fructose intolerance or hereditary fructosemia is an autosomal recessive metabolic disorder caused by a loss of function in the aldolase B gene.
Úbeda F, Santander S, Luesma MJ.
europepmc   +5 more sources

Vitamin C and folate status in hereditary fructose intolerance. [PDF]

Eur J Clin Nutr, 2022
Hereditary fructose intolerance (HFI) is a rare inborn error of fructose metabolism caused by the deficiency of aldolase B. Since treatment consists of a fructose-, sucrose- and sorbitol-restrictive diet for life, patients are at risk of presenting ...
Cano A, Alcalde C, Belanger-Quintana A, Cañedo-Villarroya E, Ceberio L, Chumillas-Calzada S, Correcher P, Couce ML, García-Arenas D, Gómez I, Hernández T, Izquierdo-García E, Chicano DM, Morales M, Pedrón-Giner C, Jáuregui EP, Peña-Quintana L, Sánchez-Pintos P, Serrano-Nieto J, Suarez MU, Miñana IV, de Las Heras J.   +21 more
europepmc   +5 more sources

Descriptive Analysis of Carrier and Affected Hereditary Fructose Intolerance in Women during Pregnancy. [PDF]

Healthcare (Basel)
(1) Background: Hereditary fructose intolerance (HFI) is a rare autosomal recessive metabolic disorder resulting from aldolase B deficiency, requiring a fructose, sorbitol and sucrose (FSS)-free diet.
Zuriaga E, Santander S, Lomba L, Izquierdo-García E, Luesma MJ.   +4 more
europepmc   +5 more sources

Effect of a high fructose diet on metabolic parameters in carriers for hereditary fructose intolerance [PDF]

Clinical Nutrition, 2021
BACKGROUND & AIMS Hyperuricemia is an independent risk factor for the metabolic syndrome and cardiovascular disease. We hypothesized that asymptomatic carriers for hereditary fructose intolerance (OMIM 22960) would have increased uric acid and altered ...
Luc Tappy, Kevin Seyssel, Christel Tran   +2 more
exaly   +4 more sources

Reply letter to the editor concerning the article 'Safety of Sars-Cov-2 vaccines administration for adult patients with hereditary fructose intolerance'. [PDF]

Hum Vaccin Immunother, 2022
According to the current European medicines legislation, on the labeling is mandatory a warning contraindicating for hereditary fructose intolerance (HFI) patients medicines with oral or parenteral fructose and sorbitol, and oral sucrose, invert sugar ...
Izquierdo-Garcia E, Alvaro Alonso EA, Montero Pastor B, Such Díaz A, Escobar Rodríguez I.   +4 more
europepmc   +4 more sources

Celiac Disease in Conjunction with Hereditary Fructose Intolerance as a Rare Cause of Liver Steatosis with Mild Hypertransaminasemia-A Case Report. [PDF]

Pediatr Rep, 2021
Celiac disease (CD) has been associated with several genetic and autoimmune disorders, but its association with hereditary fructose intolerance (HFI) is very rare.
Bobrus-Chociej A, Pollak A, Kopiczko N, Flisiak-Jackiewicz M, Płoski R, Lebensztejn DM.   +5 more
europepmc   +4 more sources

Non-alcoholic fatty liver in hereditary fructose intolerance [PDF]

Clinical Nutrition, 2020
BACKGROUND Non-alcoholic fatty liver disease (NAFLD) is characterized by fat accumulation affecting >5% of the liver volume that is not explained by alcohol abuse.
Javier Adolfo De Las Heras Montero, Fernando Andrade, Maria Luz Couce   +2 more
exaly   +4 more sources

Hereditary Fructose Intolerance [PDF]

Baillière's Clinical Gastroenterology, 1990
Hereditary fructose intolerance (HFI) is an inborn error of carbohydrate metabolism that is inherited as an autosomal recessive condition. The disease is caused by a catalytic deficiency of aldolase B and is characterized by severe abdominal symptoms and hypoglycaemia which follow the ingestion of fructose, sucrose or sorbitol.
N C, Cross, T M, Cox
core   +12 more sources

Daily Fructose Traces Intake and Liver Injury in Children with Hereditary Fructose Intolerance [PDF]

Nutrients, 2019
Hereditary fructose intolerance (HFI) is a rare genetic disorder of fructose metabolism due to aldolase B enzyme deficiency. Treatment consists of fructose, sorbitol, and sucrose (FSS)-free diet.
Maria Immacolata Spagnuolo, Fabiola Di Dato, Gabriella Esposito   +2 more
exaly   +4 more sources

Estimation of hereditary fructose intolerance prevalence in the Chinese population. [PDF]

Orphanet J Rare Dis, 2022
Background Hereditary fructose intolerance (HFI) caused by aldolase B reduction or deficiency that results in fructose metabolism disorder. The disease prevalence in the Chinese population is unknown, which impedes the formulation of HFI screening and ...
Tang M, Chen X, Ni Q, Lu Y, Wu B, Wang H, Yin Z, Zhou W, Dong X.   +8 more
europepmc   +2 more sources