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E74.31 ICD-10-CM Code: Sucrase-isomaltase deficiency

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E74.31

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Sucrase-isomaltase deficiency

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.

Buddy the Bee presenting code insight

Buddy Insight

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.

CMS-HCC V28

0

0

RAF 0

CMS-HCC V24

0

0

RAF 0

ACA/HHS

0

0

RAF 0

ESRD/PACE

0

0

RAF 0

RXHCC

HCC 42

RAF 0.0

Code Trumping

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Code Book Path

Official
E74Other disorders of carbohydrate metabolism
E74.3Other disorders of intestinal carbohydrate absorption
E74.31Sucrase-isomaltase deficiency

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E74.31 in this effective period.

Excludes 2

Official
  • lactose intolerance (E73.-)

Related Child Codes

Official
E74.39Other disorders of intestinal carbohydrate absorption

Includes

Official

ICD-10-CM does not list Includes notes for E74.31 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E74.31 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E74.31 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E74.31 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E74.31 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
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

MEAT Support

HCC Buddy guidance
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

Audit Caution

HCC Buddy guidance
Misdiagnosing CSID as irritable bowel syndrome or functional diarrhea in older children and adults
Confusing sucrase-isomaltase deficiency with lactose intolerance — they involve different enzymes and sugars
Not recognizing that this condition may present later in childhood or adulthood despite being congenital
Using a general malabsorption code when the specific enzyme deficiency is documented

Common Mistakes

HCC Buddy guidance
E73.0 — Congenital lactase deficiency: different disaccharidase enzyme; involves lactose, not sucrose
E73.9 — Lactose intolerance, unspecified: different enzyme and sugar pathway
K90.49 — Malabsorption due to intolerance, not elsewhere classified: more general malabsorption code
K58.0 — Irritable bowel syndrome with diarrhea: functional disorder often confused with CSID

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E74.31 an HCC code?

No. E74.31 is a billable ICD-10-CM code but does not map to any HCC category in V28, V24, ESRD, or RxHCC.

HCC Category Mapping

RxHCCHCC 42, Endocrine Disorders and Metabolic Conditions
0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

MEAT Criteria for E74.31

For E74.31to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E74.31 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E74.31 is the ICD-10-CM diagnosis code for sucrase-isomaltase deficiency. 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. E74.31 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E74.31 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.

Does not map to any HCC in V24, V28, or ESRD models. Maps only to RxHCC 42 (Systemic Lupus Erythematosus and Other Autoimmune Disorders). This code has minimal direct RAF impact, making it a non-HCC condition for Medicare Advantage risk adjustment purposes.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E74.31 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

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

  • E73.0: Congenital lactase deficiency: different disaccharidase enzyme; involves lactose, not sucrose
  • E73.9: Lactose intolerance, unspecified: different enzyme and sugar pathway
  • K90.49: Malabsorption due to intolerance, not elsewhere classified: more general malabsorption code
  • K58.0: Irritable bowel syndrome with diarrhea: functional disorder often confused with CSID
  • E74.39: Other disorders of intestinal carbohydrate absorption: for other absorption defects

Child Codes

Code Hierarchy

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