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Q44.71 ICD-10-CM Code: Alagille syndrome

Compare with another code for Q44.71Check 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
1 inclusion term · 1 code-also instruction
Risk adjustment
CMS-HCC V28: 1 category

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, and is marked with where it comes from.

Code alsofrom Q44.7May be needed with this code
  • Code also, if applicable, associated malformations affecting other systems
IncludesWhat this code covers
  • Alagille-Watson syndrome

Where it sits Source: CMS/NCHS

Under:Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-QA1) (Q00-QA1)Q38-Q45 Other congenital malformations of the digestive systemQ44 Congenital malformations of gallbladder, bile ducts and liverQ44.7 Other congenital malformations of liver

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 441 — DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITH MCC (MDC 07)
  • MS-DRG 442 — DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITH CC (MDC 07)
  • MS-DRG 443 — DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITHOUT CC/MCC (MDC 07)

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.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 68 — Cholangitis and Obstruction of Bile Duct Without Gallstones

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q44.71 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 Q44.71 itself; “inherited from” names the category or block whose note applies here.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Alagille-Watson syndrome

Code Also

Additional codes that may be required to fully describe the encounter.

  • Code also, if applicable, associated malformations affecting other systems

Source: inherited from Q44.7

Coder workflow for Q44.71

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

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

  • Code Also — related condition(1 note)

    Coding workflow: Review the related condition when both are documented and the instruction applies. A Code Also note does not fix sequencing; the order follows the circumstances of the encounter.

    See the official tabular notes · Guidelines I.A.17

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 Q44.71 with…

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

The codes most likely to be picked instead of Q44.71 — 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.

  • Q44.70— Specificity relationship (Review)

    Overlapping diagnoses in the same category.

    Overlapping codes in the same category — the documentation decides which is the more specific fit.

    What moves you there: A record that documents no more than “Other congenital malformation of liver, unspecified” takes Q44.70; specificity in the record moves it back here.

    Full comparison: Q44.71 vs Q44.70 →

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

Alagille syndrome is a billable ICD-10-CM diagnosis code (Q44.71).

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

  • CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.

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

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 10 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 441 (MDC 07), DRG 442 (MDC 07), DRG 443 (MDC 07).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):MAL002 — Digestive congenital anomalies (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 process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

Q44.0 — Agenesis, aplasia and hypoplasia of gallbladder, Q44.1 — Other congenital malformations of gallbladder, Q44.2 — Atresia of bile ducts, Q44.3 — Congenital stenosis and stricture of bile ducts, Q44.4 — Choledochal cyst, Q44.5 — Other congenital malformations of bile ducts, Q44.6 — Cystic disease of liver, Q44.70 — Other congenital malformation of liver, unspecified, Q44.79 — Other congenital malformations of liver

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Cholangitis and Obstruction of Bile Duct Without Gallstones) for risk-adjusted payment.

K74.3 — Primary biliary cirrhosis, K74.5 — Biliary cirrhosis, unspecified, K83.01 — Primary sclerosing cholangitis, K83.09 — Other cholangitis, K83.1 — Obstruction of bile duct, Q44.2 — Atresia of bile ducts, Q44.3 — Congenital stenosis and stricture of bile ducts

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Liver Transplant Status/Complications, Chronic Liver Failure/End-Stage Liver Disorders, Cirrhosis of Liver

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Digestive congenital anomalies).

Q43.6 — Congenital fistula of rectum and anus, Q43.7 — Persistent cloaca, Q43.8 — Other specified congenital malformations of intestine, Q43.9 — Congenital malformation of intestine, unspecified, Q44.0 — Agenesis, aplasia and hypoplasia of gallbladder, Q44.1 — Other congenital malformations of gallbladder, Q44.2 — Atresia of bile ducts, Q44.3 — Congenital stenosis and stricture of bile ducts, Q44.4 — Choledochal cyst, Q44.5 — Other congenital malformations of bile ducts, Q44.6 — Cystic disease of liver, Q44.7 — Other congenital malformations of liver, Q44.70 — Other congenital malformation of liver, unspecified, Q44.79 — Other congenital malformations of liver, Q45.0 — Agenesis, aplasia and hypoplasia of pancreas, Q45.1 — Annular pancreas, Q45.2 — Congenital pancreatic cyst, Q45.3 — Other congenital malformations of pancreas and pancreatic duct, Q45.8 — Other specified congenital malformations of digestive system, Q45.9 — Congenital malformation of digestive system, unspecified, +41 more

Contextual Map

Every relationship of Q44.71 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.

Hierarchy

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 68 — Cholangitis and Obstruction of Bile Duct Without Gallstones [CMS-HCC]— CMS-HCC V28 · 2026

MS-DRG Grouper

MDC crossing

  • MDC 07 — Diseases and Disorders of the Hepatobiliary System and Pancreas[MDC crossing]: “Diseases and Disorders of the Hepatobiliary System and Pancreas — 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. 2,488 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Alagillesyndrome (-Watson)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Syndrome, Alagille (-Watson)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (11)

Change history

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 (2)

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
Risk adjustment Official source data
2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release 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. "Q44.71 — Alagille syndrome." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q44.71-alagille-syndrome

Change history

  • FY2024 — October 1, 2023
    Added to the code set
    Alagille syndrome
    FY2024 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q44.71 in its code family, with their registry titles.

View all codes in the Q44 family