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E78.89 ICD-10-CM Code: Other lipoprotein metabolism disorders

Compare with another codeCheck this code on a claim

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
6 Excludes1 · 1 Excludes2
Risk adjustment
RxHCC V08 category 47

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

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
  • sphingolipidosis (E75.0-E75.3)
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)

+2 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

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 v44, Appendix B.

  • MS-DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)

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 E78.89 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 E78.89 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).

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 E78.89

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

Before you code E78.89

  1. “Other” (NEC) means the condition is specified in the record but no dedicated code captures it. Confirm the documented form is not one a sibling code names before settling on E78.89; if the record states no specifics at all, the unspecified sibling applies instead. “Other” codes are for documented conditions the classification gives no specific code; “unspecified” codes are for records lacking the detail (Guidelines I.A.9.a, I.A.9.b).

    ReviewE78.81

    See the relationships section · Guide: Other vs unspecified (NEC vs NOS) →

  2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E78.89. 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 a condition named in E78.89’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 E78.89. 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).
Any detail beyond this code’s title
What the record states that a more specific sibling code would capture — or its absence, which itself supports the unspecified code.

Official instructions as workflow

  • Excludes1 — check before selecting E78.89(6 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E78.89: 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 E78.89(1 note)

    Coding workflow: The conditions named in this note are not included in E78.89. 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 E78.89 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 E78.89 with…

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

The codes most likely to be picked instead of E78.89 — 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.

  • E75.0— 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 sphingolipidosis moves the case to E75.0; the two are not reported together unless documented as unrelated.

    Full comparison: E78.89 vs E75.0 →

  • E75.3— 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 sphingolipidosis moves the case to E75.3; the two are not reported together unless documented as unrelated.

    Full comparison: E78.89 vs E75.3 →

  • 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: E78.89 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: E78.89 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: E78.89 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: E78.89 vs Q87.4 →

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

Other lipoprotein metabolism disorders is a billable ICD-10-CM diagnosis code (E78.89).

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

  • 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-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name E78.89 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 2 Excludes1 notes across 2 chapters: L98.6 — Other infiltrative disorders of the skin and subcutaneous tissue, P59 — Neonatal jaundice from other and unspecified causes (via E78.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 15 Excludes2 notes across 4 chapters: E88.0 — Disorders of plasma-protein metabolism, not elsewhere classified (via E78.-), G11 — Hereditary ataxia (via E78.-), G71 — Primary disorders of muscles (via E78.-), N25.0 — Renal osteodystrophy (via E78.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E78.-), R70 — Elevated erythrocyte sedimentation rate and abnormality of plasma viscosity (via E78.-), R70-R79 — Abnormal findings on examination of blood, without diagnosis (R70-R79) (via E78.-), R71 — Abnormality of red blood cells (via E78.-), R73 — Elevated blood glucose level (via E78.-), R74 — Abnormal serum enzyme levels (via E78.-), R75 — Inconclusive laboratory evidence of human immunodeficiency virus [HIV] (via E78.-), R76 — Other abnormal immunological findings in serum (via E78.-), R77 — Other abnormalities of plasma proteins (via E78.-), R78 — Findings of drugs and other substances, not normally found in blood (via E78.-), R79 — Other abnormal findings of blood chemistry (via E78.-).

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 E78.-), H42 — Glaucoma in diseases classified elsewhere (via E78.-).

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 (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 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 1 MS-DRG: DRG 642 (MDC 10).

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).

E77.0 — Defects in post-translational modification of lysosomal enzymes, E77.1 — Defects in glycoprotein degradation, E77.8 — Other disorders of glycoprotein metabolism, E77.9 — Disorder of glycoprotein metabolism, unspecified, E78.6 — Lipoprotein deficiency, E78.70 — Disorder of bile acid and cholesterol metabolism, unspecified, E78.71 — Barth syndrome, E78.72 — Smith-Lemli-Opitz syndrome, E78.79 — Other disorders of bile acid and cholesterol metabolism, E78.81 — Lipoid dermatoarthritis, E78.9 — Disorder of lipoprotein metabolism, unspecified, E79.0 — Hyperuricemia without signs of inflammatory arthritis and tophaceous disease, E79.1 — Lesch-Nyhan syndrome, E79.2 — Myoadenylate deaminase deficiency, E79.8 — Other disorders of purine and pyrimidine metabolism, E79.81 — Aicardi-Goutières syndrome, E79.82 — Hereditary xanthinuria, E79.89 — Other specified disorders of purine and pyrimidine metabolism, E79.9 — Disorder of purine and pyrimidine metabolism, unspecified, E80.0 — Hereditary erythropoietic porphyria, +258 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Proteinosis”, “Lipoid”, “Hyalinosis”; these codes share that main term but sit in a different category of the Tabular List.

D76.3 — Other histiocytosis syndromes (histiocytosis), E75.29 — Other sphingolipidosis (histiocytosis, essential), J84.01 — Alveolar proteinosis (alveolar), N04.9 — Nephrotic syndrome with unspecified morphologic changes (nephrosis), N05.1 — Unspecified nephritic syndrome with focal and segmental glomerular lesions (focal and segmental)

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.

Lipid Panel

Contextual Map

Every relationship of E78.89 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 E78.89 with these 12 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Referenced by Excludes2 notes (15)

Referenced by Code First instructions

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 10 — Endocrine, Nutritional and Metabolic Diseases and Disorders[MDC crossing]: “Endocrine, Nutritional and Metabolic Diseases and Disorders — 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. 5,013 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Disorder (of), lipoprotein, metabolism, specified NEC[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Disorder (of), metabolism NOS, lipoprotein, specified NEC[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Hyalinosis, cutis (et mucosae)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Lipogranulomatosis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Lipoid, proteinosis of Urbach[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Proteinosis, lipid or lipoid (of Urbach)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Urbach-Wiethe disease[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Urbach's lipoid proteinosis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (23)

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.

Indexed Clinical Terms (8)

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.

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
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 — v44 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 28, 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. "E78.89 — Other lipoprotein metabolism disorders." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/e78.89-other-lipoprotein-metabolism-disorders

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Other lipoprotein metabolism disorders

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 E78.89 in its code family, with their registry titles.

View all codes in the E78 family