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Hereditary fructose intolerance is a rare autosomal recessive inherited disorder that is related to the mutation of enzyme aldolase B found on chromosome 9q22. 3. A fructose 1-phosphate accumulation that occurs immediately and manifests as a result of downstream effects. Renal involvement is most noticeable in the form of proximal renal tubular acidosis, which can lead to chronic renal insufficiency. For confirmation, a genetic test is preferred over aldolase B activity in the liver biopsy sample. Patients with HFI who strictly adhere to a FSS-free diet have an excellent prognosis with a normal lifespan.
Source link: https://doi.org/10.5409/wjcp.v11.i4.321
Nevertheless, HFI patients must be closely monitored after parenteral administration of sucrose-containing drugs, particularly when the amount exceeds the maximum acceptable thresholds.
Source link: https://doi.org/10.1080/21645515.2022.2051414
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