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E71.11 ICD-10-CM Code: Branched-chain organic acidurias

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Billing Status: NO. This is a non-billable ICD-10-CM code: report a more specific billable code beneath it.

Coding at a Glance

Tabular directives
5 Excludes1 · 1 Excludes2

Not billable · FY2027A non-billable heading in the tabular list: report a more specific code beneath it.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too; the Instructions section marks which is which.

Excludes1Never report with this code
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)

+1 more in Instructions

Excludes2Not included here; may be reported together
  • Ehlers-Danlos syndromes (Q79.6-)

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for E71.11 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on E71.11 itself; “inherited from” names the category or block whose note applies here.

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).

Source: inherited from E70-E88

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from E70-E88

Coder workflow for E71.11

MedCoder structured workflow — derived from this code’s own official record

Before you code E71.11

  1. E71.11 is not reportable as written. Select the more specific code beneath it that the documentation supports. Codes are reported to the highest level of specificity the classification provides (Guidelines I.B.2).

    ReviewE71.110, E71.111, E71.118

    See the relationships section · Guide: How to choose an ICD-10-CM code →

  2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E71.11. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support one of the more specific codes beneath E71.11?
    Yes → Select that code and continue the checks below on its own page.
    No → E71.11 cannot be reported as written; query for the specificity its subcategory needs.

    ReviewE71.110, E71.111, E71.118

  2. Does the documentation support a condition named in E71.11’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.

    ReviewE34.5, E25.0, D55, Q87.4, E29.1

Consider E71.11. 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 E71.11(5 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E71.11: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE34.5, E25.0, D55, Q87.4, E29.1

    See the official tabular notes · Guidelines I.A.12.a

  • Excludes2 — not part of E71.11(1 note)

    Coding workflow: The conditions named in this note are not included in E71.11. 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 E71.11 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).

ReviewE34.5, E25.0, D55, Q87.4, E29.1

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.

Don’t confuse E71.11 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of E71.11 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • E34.5— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of androgen insensitivity syndrome moves the case to E34.5; the two are not reported together unless documented as unrelated.

    Full comparison: E71.11 vs E34.5 →

  • E25.0— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of congenital adrenal hyperplasia moves the case to E25.0; the two are not reported together unless documented as unrelated.

    Full comparison: E71.11 vs E25.0 →

  • D55— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of hemolytic anemias attributable to enzyme disorders moves the case to D55; the two are not reported together unless documented as unrelated.

    Full comparison: E71.11 vs D55 →

  • Q87.4— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Marfan syndrome moves the case to Q87.4; the two are not reported together unless documented as unrelated.

    Full comparison: E71.11 vs Q87.4 →

  • E29.1— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of 5-alpha-reductase deficiency moves the case to E29.1; the two are not reported together unless documented as unrelated.

    Full comparison: E71.11 vs E29.1 →

  • Q79.6— Excludes2 relationship (Informational)

    Distinct conditions — both may be reported.

    A distinct condition — both may be reported when both are documented.

    What moves you there: Documentation of Ehlers-Danlos syndromes adds Q79.6; it is not part of this code and both may be reported when both are documented.

    Full comparison: E71.11 vs Q79.6 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Branched-chain organic acidurias is a non-billable ICD-10-CM category code (E71.11). A more specific billable subcode must be selected for claims submission.

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

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-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name E71.11 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 8 Excludes1 notes across 3 chapters: E72 — Other disorders of amino-acid metabolism, P59 — Neonatal jaundice from other and unspecified causes (via E71.-), R79 — Other abnormal findings of blood chemistry (via E71.-), R79.83 — Abnormal findings of blood amino-acid level (via E71.-), R80 — Proteinuria (via E71.-), R80-R82 — Abnormal findings on examination of urine, without diagnosis (R80-R82) (via E71.-), R81 — Glycosuria (via E71.-), R82 — Other and unspecified abnormal findings in urine (via E71.-).

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 E71.-), G71 — Primary disorders of muscles (via E71.-), N25.0 — Renal osteodystrophy (via E71.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E71.-).

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 E71.-), H42 — Glaucoma in diseases classified elsewhere (via E71.-).

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.

Contextual Map

Every relationship of E71.11 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 E71.11 with these 14 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Referenced by Excludes2 notes

Referenced by Code First instructions

Nearest codes (40)

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Common coding questions

MedCoder editorial

Can E71.11 be billed directly?

No. E71.11 (Branched-chain organic acidurias) is a non-billable ICD-10-CM category code. A more specific billable subcode must be selected based on clinical documentation.

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 FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) 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-derived 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 editorial 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.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 20, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

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. "E71.11 — Branched-chain organic acidurias." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/e71.11-branched-chain-organic-acidurias

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Branched-chain organic acidurias

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027, 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 E71.11 in its code family, with their registry titles.

View all codes in the E71 family