Sucrose intolerance

An interesting phenomenon is seen in (he far reaches of the northern hemisphere, where sucrose intolerance occurs among Eskimos. About 10% of the native population of Greenland is sucrose intolerant. The occurrence of sucrose intolerance among these populations is eonsi.stent with the lack of availability of sucrose-containing staples. People in Greenland traditionally have consumed meat as the major food source, and not fruits or sugar cane. 

Individuals with genetic deficiencies of the sucrase-isomaltase complex show symptoms of sucrose intolerance but are able to digest normal amounts of starch in a meal, without problems. The maltase activity in the glucoamylase complex, and residual activity in the sucrase-isomaltase complex (which is normally present in excess of need) is apparently sufficient to digest normal amounts of dietary starch. 

Sucrase deficiency comes about either by profound damage to the small intestine or more usually from an inherited defect. The gene for sucrase resides on chromosome 3, and homozygous individuals for the defective gene have sucrose intolerance. The total world incidence of the inherited intolerance is 1100, so it is very rare. What do you think would be the signs and symptoms of this enzyme defect ... 

Malcamey, H.L., Martinez del Rio, C. Apanius, V. (1994). Sucrose intolerance in birds simple nonlethal diagnostic methods and consequences for assimilation of complex carbohydrates. Auk, 111, 170-7. 

Hereditary fructose intolerance is due to aldolase B deficiency and is often diagnosed when babies are switched from formula or mother s milk to a diet containing fructose-based sweetening, such as sucrose or honey. 

Isomaltase-sucrase deficiency This enzyme deficiency results in an intolerance of ingested sucrose. This disorder is found in about ten percent of Greenland s Eskimos, whereas two percent of North Americans are heterozygous for the deficiency. Treatment is to withhold dietary sucrose. 

Cause and treatment of hereditary fructose intolerance A deficiency of fructokinase causes a benign condition, but a deficiency of aldolase B causes hereditary fructose intolerance, in which severe hypoglycemia and liver damage can lead to death if the amount of fructose (and, therefore, sucrose) in the diet are not severely limited. 

Sucrose (saccharose) Unsuitable in hereditary fructose intolerance, glucose/ galactose malabsorption syndrome, or sucrase-isomaltase deficiency... 

Excessive oral fructose consumption (>75g daily) in the absence of dietary dextrose in any form (e.g., sucrose, starch, dextrin, etc.) may cause malabsorption in susceptible individuals, which may result in flatulence, abdominal pain, and diarrhea. Except in patients with hereditary fructose intolerance, there is no evidence to indicate that oral fructose intake at current levels is a risk factor in any particular disease, other than dental caries. ... 

Although sucrose is very widely used in foods and pharmaceutical formulations, sucrose consumption is a cause of concern and should he monitored in patients with diabetes mellitus or other metabolic sugar intolerance. ... 

Sucrose Oral, parenteral Hereditary fructose intolerance... 

Individuals with a deficiency in aldolase B have the condition known as fructose intolerance. As with most enzyme deficiencies, this is an autosomal recessive disease it does not cause difficulty as long as the patient does not consume any foods with fructose or sucrose. Frequently, children with fructose intolerance avoid candy and fruit, which should raise some eyebrows Likewise, they usually do not have many dental caries. However, if chronically exposed to fractose-containing foods, infants and small children may have poor weight gain and abdominal cramping or vomiting. 

Do you crave sweets, but worry about the empty calories in sugary treats If so, you are not alone. Research tells us that, even as babies, we demonstrate preference for sweet tastes over all others. But there are many reasons to reduce our intake of refined sugars, in particular sucrose or table sugar. Too many people eat high-calorie, low-nutrition snacks rather than more nutritious foods. This can lead to obesity, a problem that is very common in our society. In addition, sucrose is responsible for tooth decay. Lactic acid, one of the products of the metabolism of sucrose by bacteria on our teeth, dissolves the tooth enamel, which results in a cavity. For those with diabetes, glucose intolerance, or hypoglycemia, sucrose in the diet makes it difficult to maintain a constant blood sugar level. 

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