Version 2024

2024 ICD-10-CM Diagnosis Code Q44.71

Alagille syndrome

ICD-10-CM Code:: Q44.71
ICD-10 Code for:: Alagille syndrome
Is Billable?: Yes - Valid for Submission
Code Navigator:

Code Information
Approximate Synonyms
Clinical Information
2024 ICD-10 Code
Tabular List of Diseases and Injuries
Index to Diseases and Injuries References
Diagnostic Related Groups Mapping
Present on Admission (POA)
Replacement Code
Patient Education
Other Codes Used Similar Conditions
Code History

Code Classification

Congenital malformations, deformations and chromosomal abnormalities
(Q00-Q99)
- Other congenital malformations of the digestive system
  (Q38-Q45)
  - Congenital malformations of gallbladder, bile ducts and liver
    (Q44)

Q44.71 is a billable diagnosis code used to specify a medical diagnosis of alagille syndrome. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Arteriohepatic dysplasia
Biliary tract dysplasia

Clinical Information

Alagille Syndrome
a multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (bile ducts, intrahepatic), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. major clinical features include jaundice, and congenital heart disease with peripheral pulmonary stenosis. alagille syndrome may result from heterogeneous gene mutations, including mutations in jag1 on chromosome 20 (type 1) and notch2 on chromosome 1 (type 2).
Jagged-1 Protein
a serrate-jagged protein that functions as a ligand for notch receptors. it may regulate cell differentiation in hematopoiesis and physiologic angiogenesis. mutations in the jagged-1 gene are associated with alagille syndrome 1.

New 2024 ICD-10-CM Code

Q44.71 is new to ICD-10-CM code set for the FY 2024, effective October 1, 2023. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2023. This is a new and revised code for the FY 2024 (October 1, 2023 - September 30, 2024).

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Inclusion Terms

Alagille-Watson syndrome

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

- Alagille (-Watson) syndrome - Q44.71

- Syndrome - See Also: Disease;
- - Alagille (-Watson) - Q44.71

Q44.71 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
441	DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITH MCC	07	1.8282
442	DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITH CC	07	0.9515
443	DISORDERS OF LIVER EXCEPT MALIGNANCY, CIRRHOSIS OR ALCOHOLIC HEPATITIS WITHOUT CC/MCC	07	0.7147

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Present on Admission (POA)

Q44.71 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA Indicator	Reason for Code	CMS will pay the CC/MCC DRG?
Y	Diagnosis was present at time of inpatient admission.	YES
N	Diagnosis was not present at time of inpatient admission.	NO
U	Documentation insufficient to determine if the condition was present at the time of inpatient admission.	NO
W	Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.	YES
1	Unreported/Not used - Exempt from POA reporting.	NO

Replacement Code

Q4471 replaces the following previously assigned ICD-10-CM code(s):

Q44.7 - Other congenital malformations of liver

Code History

FY 2024 - Code Added, effective from 10/1/2023 through 9/30/2024