E74.11 ICD-10-CM Code: Essential fructosuria
Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.
Coding at a Glance
- Tabular directives
- 1 inclusion term · 10 Excludes1 · 1 Excludes2
Inpatient Payment Groups (MS-DRG)
Potential MS-DRG participation — not a DRG assignment.
MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.
- MS-DRG 391 — ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITH MCC (MDC 06)
- MS-DRG 392 — ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITHOUT MCC (MDC 06)
A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.
Coding instructions
Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for E74.11 in the official ICD-10-CM tabular list, quoted as published.
Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025
Notes without a marker are published on E74.11 itself; “inherited from” names the category or block whose note applies here.
Inclusion Terms
Alternative terms the tabular list files under this code.
- Fructokinase deficiency
Excludes1 — Not Coded Here
Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).
- muscle phosphofructokinase deficiency (E74.09) inherited from E74.1Compare E74.11 vs E74.09 →
- diabetes mellitus (E08-E13) inherited from E74Compare E74.11 vs E08 →
- hypoglycemia NOS (E16.2) inherited from E74Compare E74.11 vs E16.2 →
- increased secretion of glucagon (E16.3) inherited from E74Compare E74.11 vs E16.3 →
- mucopolysaccharidosis (E76.0-E76.3) inherited from E74Compare E74.11 vs E76.0 →
- androgen insensitivity syndrome (E34.5-) inherited from E70-E88Compare E74.11 vs E34.5 →
- congenital adrenal hyperplasia (E25.0) inherited from E70-E88Compare E74.11 vs E25.0 →
- hemolytic anemias attributable to enzyme disorders (D55.-) inherited from E70-E88Compare E74.11 vs D55 →
- Marfan syndrome (Q87.4-) inherited from E70-E88Compare E74.11 vs Q87.4 →
- 5-alpha-reductase deficiency (E29.1) inherited from E70-E88Compare E74.11 vs E29.1 →
Excludes2 — Not Included Here
Conditions not covered by this code, but which may be reported alongside it when both are present.
- Ehlers-Danlos syndromes (Q79.6-) Compare E74.11 vs Q79.6 →
Source: inherited from E70-E88
Coder workflow for E74.11
MedCoder structured workflow — derived from this code’s own official record
Before you code E74.11
- Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E74.11. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).
Choose the right path
- Does the documentation support a condition named in E74.11’s Excludes1 note?
Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
No → Continue.
Consider E74.11. Then confirm the code is valid for the date of service in the Verify section.
Documentation check
- The provider’s diagnostic statement
- Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
Official instructions as workflow
Excludes1 — check before selecting E74.11(10 notes)
Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E74.11: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.
CompareE74.09, E16.2, E16.3, E34.5, E25.0, D55
See the official tabular notes · Guidelines I.A.12.a
Excludes2 — not part of E74.11(1 note)
Coding workflow: The conditions named in this note are not included in E74.11. When the record documents both, both may be reported; the note is a boundary, not a prohibition.
CompareQ79.6
See the official tabular notes · Guidelines I.A.12.b
Coding decision scenarios
Pattern scenarios for this code’s structure — decision rules, not clinical cases
Documentation: Both the condition E74.11 describes and a condition named in its Excludes1 note are documented for the same encounter.
Coding question: Can both codes be reported?
Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.
Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).
Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.
Code Overview
MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.
Indexed Clinical Terms (5)
Official source data — entries quoted as published, in the Index’s own lookup phrasing
Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.
Verify Before Coding
- No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.
From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.
Relationships & Classification
MedCoder structured relationships — computed from published CMS and AHRQ datasets
Other codes that name E74.11 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.
Referenced by 6 Excludes1 notes across 2 chapters: P59 — Neonatal jaundice from other and unspecified causes (via E74.-), R79 — Other abnormal findings of blood chemistry (via E74.-), R80 — Proteinuria (via E74.-), R80-R82 — Abnormal findings on examination of urine, without diagnosis (R80-R82) (via E74.-), R81 — Glycosuria (via E74.-), R82 — Other and unspecified abnormal findings in urine (via E74.-).
These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.
Referenced by 4 Excludes2 notes across 2 chapters: G11 — Hereditary ataxia (via E74.-), G71 — Primary disorders of muscles (via E74.-), N25.0 — Renal osteodystrophy (via E74.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E74.-).
These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.
Referenced by 2 Code First instructions across 2 chapters: G63 — Polyneuropathy in diseases classified elsewhere (via E74.-), H42 — Glaucoma in diseases classified elsewhere (via E74.-).
Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.
MS-DRG Grouper Relationships (FY2026)
Potential MS-DRG participation — not a DRG assignment.
FY2026 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.
Named in the grouper logic of 2 MS-DRGs: DRG 391 (MDC 06), DRG 392 (MDC 06).
From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.
Clinical classification (AHRQ CCSR):END016 — Other specified and unspecified nutritional and metabolic disorders (default).
Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.
Related Codes
Same clinical category (CCSR)
AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified nutritional and metabolic disorders).
E73.8 — Other lactose intolerance, E73.9 — Lactose intolerance, unspecified, E74.00 — Glycogen storage disease, unspecified, E74.01 — von Gierke disease, E74.02 — Pompe disease, E74.03 — Cori disease, E74.04 — McArdle disease, E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency, E74.09 — Other glycogen storage disease, E74.10 — Disorder of fructose metabolism, unspecified, E74.12 — Hereditary fructose intolerance, E74.19 — Other disorders of fructose metabolism, E74.20 — Disorders of galactose metabolism, unspecified, E74.21 — Galactosemia, E74.29 — Other disorders of galactose metabolism, E74.31 — Sucrase-isomaltase deficiency, E74.39 — Other disorders of intestinal carbohydrate absorption, E74.4 — Disorders of pyruvate metabolism and gluconeogenesis, E74.8 — Other specified disorders of carbohydrate metabolism, E74.810 — Glucose transporter protein type 1 deficiency, +258 more
Same Index main term, other category
The ICD-10-CM Index to Diseases and Injuries files this code under the main term “Deficiency, deficient”; these codes share that main term but sit in a different category of the Tabular List.
E71.43 — Iatrogenic carnitine deficiency (carnitine, iatrogenic), E71.448 — Other secondary carnitine deficiency (carnitine, secondary), E72.12 — Methylenetetrahydrofolate reductase deficiency (methylenetetrahydrofolate reductase), E72.19 — Other disorders of sulfur-bearing amino-acid metabolism (sulfite oxidase), E72.4 — Disorders of ornithine metabolism (ornithine transcarbamylase), E72.81 — Disorders of gamma aminobutyric acid metabolism (GABA-T), E72.9 — Disorder of amino-acid metabolism, unspecified (amino-acids), E73.0 — Congenital lactase deficiency (lactase, congenital), E73.1 — Secondary lactase deficiency (lactase, secondary), E73.9 — Lactose intolerance, unspecified (disaccharidase), E75.240 — Niemann-Pick disease type A (acid sphingomyelinase, type, A), E75.241 — Niemann-Pick disease type B (acid sphingomyelinase, type, B), E75.244 — Niemann-Pick disease type A/B (acid sphingomyelinase, type, A/B), E75.249 — Niemann-Pick disease, unspecified (acid sphingomyelinase), E75.26 — Sulfatase deficiency (sulfatase), E76.29 — Other mucopolysaccharidoses (beta-glucuronidase), E78.6 — Lipoprotein deficiency (lipoprotein), E79.1 — Lesch-Nyhan syndrome (hypoxanthine-), E79.2 — Myoadenylate deaminase deficiency (myoadenylate deaminase), E80.5 — Crigler-Najjar syndrome (glucuronyl transferase), +162 more
Lab tests where this diagnosis supports Medicare coverage (NCD)
Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.
Contextual Map
Every relationship of E74.11 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.
Run E74.11 with these 12 related codes in Claim Check
Hierarchy
- E00-E89 — Chapter 4: Endocrine, Nutritional and Metabolic Diseases (E00-E89) (E00-E89)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E70-E88 — Metabolic disorders[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
Referenced by Excludes1 notes
- P59 — Neonatal jaundice from other and unspecified causes[Excludes1](via E74.-): “jaundice due to inborn errors of metabolism (E70-E88)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- R79 — Other abnormal findings of blood chemistry[Excludes1](via E74.-): “specific findings indicating disorder of carbohydrate metabolism (E73-E74)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- R80 — Proteinuria[Excludes1](via E74.-): “specific findings indicating disorder of carbohydrate metabolism (E73-E74)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- R80-R82 — Abnormal findings on examination of urine, without diagnosis (R80-R82)[Excludes1](via E74.-): “specific findings indicating disorder of carbohydrate metabolism (E73-E74)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- R81 — Glycosuria[Excludes1](via E74.-): “specific findings indicating disorder of carbohydrate metabolism (E73-E74)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- R82 — Other and unspecified abnormal findings in urine[Excludes1](via E74.-): “specific findings indicating disorder of carbohydrate metabolism (E73-E74)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
Referenced by Excludes2 notes
- G11 — Hereditary ataxia[Excludes2](via E74.-): “metabolic disorders (E70-E88)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- G71 — Primary disorders of muscles[Excludes2](via E74.-): “metabolic disorders (E70-E88)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- N25.0 — Renal osteodystrophy[Excludes2](via E74.-): “metabolic disorders classifiable to E70-E88”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- N25.81 — Secondary hyperparathyroidism of renal origin[Excludes2](via E74.-): “metabolic disorders classifiable to E70-E88”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
Referenced by Code First instructions
- G63 — Polyneuropathy in diseases classified elsewhere[Code First](via E74.-): “metabolic diseases (E70-E88)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
- H42 — Glaucoma in diseases classified elsewhere[Code First](via E74.-): “specified metabolic disorder (E70-E88)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
Clinical classification (CCSR)
- END016 — Other specified and unspecified nutritional and metabolic disorders[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)
MS-DRG Grouper
- DRG 391 — ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITH MCC[MS-DRG]: “ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITH MCC (MDC 06)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
- DRG 392 — ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITHOUT MCC[MS-DRG]: “ESOPHAGITIS, GASTROENTERITIS AND MISCELLANEOUS DIGESTIVE DISORDERS WITHOUT MCC (MDC 06)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
MDC crossing
- MDC 06 — Diseases and Disorders of the Digestive System[MDC crossing]: “Diseases and Disorders of the Digestive System — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 5,744 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026
Index entries
- Deficiency, deficient, fructokinase[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
- Disorder (of), fructose metabolism, essential fructosuria[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
- Disorder (of), fructose metabolism, fructokinase deficiency[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
- Fructokinase deficiency[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
- Fructosuria (benign) (essential)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
Nearest codes (31)
- E74 — Other disorders of carbohydrate metabolism[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.0 — Glycogen storage disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.00 — Glycogen storage disease, unspecified[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.01 — von Gierke disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.02 — Pompe disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.03 — Cori disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.04 — McArdle disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
- and 23 more
Change history
- FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016
Sources for this page
Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.
- Code, title, tabular notes and index terms Official source data
- CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
- Claim edits Official source data
- CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
- Inpatient payment groups Official source data
- CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
- Change history and date-of-service validity Official source data
- CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
- Comparisons, relationships and the contextual map MedCoder structured relationship
- Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
- Summary and FAQ answers MedCoder explanation
- Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.
Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates
Cite this page
Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.
MedCoder.ai. "E74.11 — Essential fructosuria." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/e74.11-essential-fructosuria
Change history
- FY2016 — October 1, 2015In the code set at ICD-10-CM adoptionEssential fructosuria
No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027 (effective October 1, 2026), and none are recorded for this code. Note changes are tracked from FY2027 only.
Nearest Codes in This Family
Official ICD-10-CM classifications closest to E74.11 in its code family, with their registry titles.
- E74.04 — McArdle disease
- E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency
- E74.09 — Other glycogen storage disease
- E74.1 — Disorders of fructose metabolism
- E74.10 — Disorder of fructose metabolism, unspecified
- E74.12 — Hereditary fructose intolerance
- E74.19 — Other disorders of fructose metabolism
- E74.2 — Disorders of galactose metabolism
- E74.20 — Disorders of galactose metabolism, unspecified
- E74.21 — Galactosemia