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Inborn fructose intolerance symptoms, diagnosis and dietary recommendations

Homepage Articles Inborn fructose intolerance symptoms, diagnosis and dietary recommendations

Inborn fructose intolerance symptoms, diagnosis and dietary recommendations

For most people, it is legal and safe to consume it, but there are diseases in which it must be limited or eliminated from the diet. How does this disease develop and what are the main assumptions of the diet used in its course? It gives a sweet taste to fruits, some vegetables and honey. One of these is the genetically determined metabolic defect congenital intolerance to fructose. Fructose is a simple sugar found in many everyday food products.

Table of Contents

1. The dietary procedure

It is also often difficult to estimate the exact content of fructose in a product and its actual consumption.The basic premise of the diet is to eliminate fructase and some other sweeteners (mainly sorbitol and sucrose) from the diet.The approach should therefore aim at maximizing the amount of this sugar in the diet, especially in the first two years of life.

2. Gaughan, L. Baker 2015) Sykut-Cegielska 2017; S. Ayres, P.R. Products allowed and prohibited in the diet (J

Therefore, it is very important to read the labels carefully, especially for ready-made and processed vegetables (e.g. sausages), sauces (ketchup, mustard, mayonnaise, etc.), mixtures of spices, medicines and dietary supplements (many of them, especially syrups and unleavened tablets, contain sugar or sorbitol) and toothpaste. Thermal processing is also important for vegetables. However, this process is also likely to lead to a decrease in the amount of saturated fat, so it is preferable to have a white variety rather than a full-fledged variety.

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Dziedziczna nietolerancja fruktozy, orpha.net/consor/cgi-bin/Disease_Search.php?lng=PL&data_id=517&MISSING%20CONTENT=Dziedziczna-nietolerancja-fruktozy&search=Disease_Search_Simple&title=Dziedziczna%20nietolerancja%20fruktozy (31.01.2023).
Gaughan S., Ayres L., Baker P.R., Hereditary Fructose Intolerance, GeneReviews® [Internet], pod red. Adam M.P. et al., Seattle 2015.
Li H. et al., Acute liver failure in neonates with undiagnosed hereditary fructose intolerance due to exposure from widely available infant formulas, „Molecular Genetics and Metabolism” 2018, 123(4), 428–432.
Singh S.K., Sarma M.S., Hereditary fructose intolerance: A comprehensive review, „World Journal of Clinical Pediatrics” 2022, 11(4), 321–329.
Sykut-Cegielska J., Wrodzona nietolerancja fruktozy, Żywienie i leczenie żywieniowe dzieci i młodzieży, pod red. Szajewskiej H., Horvath A., Kraków 2017, 271–273