SUCROSE INTOLERANCE

SUCROSE INTOLERANCE

Sucrose intolerance, otherwise known as a congenital sucrase-isomaltase deficiency, is a rare hereditary metabolic disorder. It is marked by the absence or deficiency of the enzymes sucrase and isomaltase. These enzymes form an enzyme complex (sucrase-isomaltase) that assists in the hydrolysis of specific sugars such as sucrose and certain products of starch digestion (dextrins). Inside the tiny, finger-like projections lining the small intestine (microvilli), the sucrase-isomaltase enzyme complex is usually found. Intolerance to starch usually disappears as the years go by, specifically during the first few years of life. Also, symptoms of sucrose intolerance improve as the affected child gets older.

SYMPTOMS

Sucrose intolerance is a disorder marked by the deficiency or absence of the sucrase-isomaltase enzyme complex. When this occurs, hydrolysis of sucrose into fructose and glucose cease to happen. This impedes the absorption of micronutrients in your gut.

Symptoms of sucrose intolerance in infants include:

  • Watery diarrhea
  • Dehydration
  • Distention
  • Abdominal discomfort
  • Malnutrition
  • Stunting
  • Colic
  • Irritation of the skin of the buttocks resulting from prolonged diarrhea

DIAGNOSIS

This may be observed in any neonate showing signs of severe watery diarrhea after intake of milk-modified or glucose-polymer formula. For example, blood and urine samples may reveal an elevated presence of the disaccharide sucrose, isomaltose or palatinose, its substitute. Fecalysis may also show sucrose, glucose and fructose, and a pH of below 5.0 or 6.0.

Diagnostics may include sucrase-isomaltase enzyme assay that measures the activity of the enzyme complex in the small intestine. This is usually done through a biopsy.

TREATMENT

Treatment of sucrose-intolerance focuses on dietary changes by avoiding sucrose or following a low sucrose diet. Certain individuals may exhibit signs of sucrose tolerance as they reach their 20’s, however, some individuals also exhibit a life-long sucrose intolerance. Ingesting fresh baker’s yeast may benefit individuals affected by this disorder as it mimics sucrase activity after sucrose ingestion. Clinical studies have shown that yeast should be administered on a full stomach, as this is the time when it exhibits its maximum effectiveness.

The FDA has approved the drug sarcosinate as a treatment for sucrose intolerance. It may help ease the symptoms of sucrose intolerance associated with sucrose ingestion. Its mechanism of action is through the introduction of the sucrase enzyme so that it could aid your intestine’s digestion and in the hydrolysis of sucrose.

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