Fructose intolerance.html


Fructose intolerance Classification and external resources
Fructose
ICD-10	E 74.1
ICD-9	271.2
OMIM	229600
DiseasesDB	5003
MedlinePlus	000359
eMedicine	ped/988
MeSH	D005633

Hereditary fructose intolerance (HFI) or fructose poisoning is a hereditary condition caused by a deficiency of liver enzymes that metabolise fructose. It is also known as hereditary fructosemia, or fructose in the blood (-emia means in the blood)

1 Causes
2 Presentation
3 Treatment
4 Related conditions
5 See also
6 References
7 External links

Causes

The deficient enzyme is aldolase-B, which converts fructose-1-phosphate to DHAP and glyceraldehyde. This means that the fructose cannot be further metabolised beyond fructose-1-phosphate. This traps phosphates; which are needed to phosphorylate glycogen phosphorylase which carries on to release units of glucose-1-phosphate from glycogen. (Glucose-1-phosphate gets converted to glucose-6-phosphate and then dephosphorylated to form glucose).

In addition, Aldolase A plays an important role in gluconeogenesis, producing fructose-1,6-bisphosphate from glyceraldehyde-3-phosphate and DHAP. But, glucose may still be released through the breakdown of glycogen. Although, it cannot be synthesized from gluconeogenesis, resulting in severe hypoglycaemia.

Presentation

If fructose is ingested, other symptoms such as vomiting, hypoglycemia, jaundice, hemorrhage, hepatomegaly, hyperuricemia and eventually kidney failure will follow.

Treatment

Treatment is with a fructose free diet, which if adhered to, is concordant with a good prognosis. ¹

Fructose and sucrose eliminated from diet.

Related conditions

Hereditary fructose intolerance should not be confused with fructose malabsorption. The latter is the same as dietary fructose intolerance (DFI), a deficiency of fructose transporter enzyme in the enterocytes, which leads to abdominal bloating, diarrhea and/or constipation.

References

^ -389021656 at GPnotebook

External links

v • d • e Inborn error of carbohydrate metabolism (including glycogen storage diseases) (E73-74, 271)

Disaccharide catabolism	Lactose intolerance - Sucrose intolerance

Monosaccharide catabolism	fructose: Essential fructosuria - Fructose intolerance galactose/galactosemia : Galactokinase deficiency - Galactose-1-phosphate uridylyltransferase galactosemia - Galactose epimerase deficiency

Monosaccharide transport	Glucose-galactose malabsorption - Inborn errors of renal tubular transport (Renal glycosuria)

Glycolysis	GSD type VII, Tarui's, phosphofructokinase - Triosephosphate isomerase deficiency - Pyruvate kinase deficiency

Pyruvate catabolism	PDHA - Fumarase deficiency

Gluconeogenesis	PCD - Fructose bisphosphatase deficiency - GSD type I, von Gierke, glucose 6-phosphatase

Glycogenesis	GSD type 0, glycogen synthase - GSD type IV, Andersen's, branching

Glycogenolysis	GSD type II, Pompe's, glucosidase - GSD type III, Cori's, debranching - GSD type V, McArdle, glycogen phosphorylase/GSD type VI, Hers', glycogen phosphorylase - GSD type I, von Gierke, glucose 6-phosphatase

Pentose phosphate pathway	Glucose-6-phosphate dehydrogenase deficiency - Pentosuria

Other	Hyperoxaluria (Primary hyperoxaluria)

see also glycolysis enzymes, pentose phosphate pathway enzymes, fructose and galactose metabolism enzymes

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