Fructose intolerance
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| ICD-10 | E74.1 |
|---|---|
| ICD-9 | 271.2 |
| OMIM | 229600 |
| DiseasesDB | 5003 |
| MedlinePlus | 000359 |
| eMedicine | ped/988 |
Fructose intolerance (Hereditary Fructose Intolerance, or HFI) is a hereditary condition due to a deficiency of liver enzymes that metabolise fructose. The deficient enzyme is Fructose-1-phosphate aldolase-B, this means that the fructose cannot be further metabolised beyond fructose-1-phosphate. This traps phosphates; which are needed to phosphorolyse glycogen phosphorolase to carry on to make glucose. Therefore glucose cannot be made through the breakdown of glycogen nor from gluconeogenesis, resulting in severe hypoglycaemia. If fructose is ingested, vomiting, hypoglycaemia and eventually kidney failure will follow.
Hereditary Fructose Intolerance should not be confused with fructose malabsorption or Dietary Fructose Intolerance (DFI), a deficiency of fructose transporter enzyme in the enterocytes, which leads to abdominal bloating, diarrhea and/or constipation.
[edit] External links
- GPnotebook -389021656
- http://www.uihealthcare.com/topics/medicaldepartments/foodandnutrition/dfi/whatisdfi.html
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