E74.31

HCC Category Mapping

What This Code Means

This is a rare genetic condition where the body cannot properly digest certain sugars (sucrose and maltose) because it lacks specific enzymes in the small intestine. People with this condition experience digestive problems like diarrhea, bloating, and abdominal pain when they consume foods containing these sugars.

Coding Tips

• •This is a congenital enzyme deficiency disorder; verify documentation confirms sucrase-isomaltase deficiency specifically, as other disaccharidase deficiencies have different codes (e.g., E73 for lactase deficiency)
• •Look for associated symptoms like chronic diarrhea, failure to thrive in infants, or malabsorption that may warrant additional coding; this condition is often diagnosed in childhood but can present at any age

Clinical Significance

Sucrase-isomaltase deficiency (congenital sucrase-isomaltase deficiency or CSID) is an inherited disorder of disaccharide digestion where the small intestine cannot properly break down sucrose and starch. This causes chronic osmotic diarrhea, bloating, and failure to thrive, particularly in infants starting solid foods. While not life-threatening, it significantly impacts quality of life and nutritional status if undiagnosed.

Documentation Requirements

• ✓Confirmed diagnosis of sucrase-isomaltase deficiency
• ✓Disaccharide enzyme assay from small bowel biopsy or breath hydrogen testing results
• ✓Genetic testing for SI gene mutations if performed
• ✓Documentation of gastrointestinal symptoms: chronic diarrhea, bloating, abdominal pain
• ✓Current dietary management including sucrose restriction and enzyme replacement therapy (sacrosidase)

Commonly Confused Codes