Short answer · Medically reviewed summary · Last updated: 2026-04-06
The ICD-10-CM code for Alagille syndrome is Q44.7, and the corresponding ICD-9-CM code is 751.61. As a physician who has supported many families navigating the complexities of Alagille syndrome, I understand that these diagnostic codes are often the first step in unlocking the insurance coverage and specialized care your loved one requires. While these codes are essential for administrative and billing purposes, they represent only a tiny fraction of the clinical reality of living with this multisystem disorder. Understanding the Clinical Scope Alagille syndrome is a complex genetic condition that primarily involves the liver—specifically due to a paucity of bile ducts—but it is truly a multisystemic disorder.
ICD10 code of Alagille Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Alagille Syndrome, with classification details for clinicians, coders and patients.
The ICD-10-CM code for Alagille syndrome is Q44.7, and the corresponding ICD-9-CM code is 751.61.
As a physician who has supported many families navigating the complexities of Alagille syndrome, I understand that these diagnostic codes are often the first step in unlocking the insurance coverage and specialized care your loved one requires. While these codes are essential for administrative and billing purposes, they represent only a tiny fraction of the clinical reality of living with this multisystem disorder.
Understanding the Clinical Scope
Alagille syndrome is a complex genetic condition that primarily involves the liver—specifically due to a paucity of bile ducts—but it is truly a multisystemic disorder. Because the underlying genetic mutations (typically in the JAG1 or NOTCH2 genes) affect signaling pathways involved in embryonic development, the disease can impact the heart, skeleton, eyes, and kidneys in addition to the liver. When using the Q44.7 code, physicians are often documenting a clinical picture that requires a multidisciplinary team, including hepatologists, cardiologists, and genetic counselors, to manage the unique needs of a patient with Alagille syndrome.
Navigating the Diagnostic Journey
Receiving a formal diagnosis of Alagille syndrome can be an overwhelming experience for parents and patients alike. It is important to remember that these codes are tools for medical record-keeping, not a definition of a patient’s potential. Because this condition is rare and variable, the clinical management is highly individualized. Whether you are managing chronic cholestasis or monitoring for peripheral pulmonic stenosis, having the correct ICD-10 code ensures that your medical team can better coordinate care and access the specific resources available for this community.
Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
References
- NIH Genetic and Rare Diseases (GARD) Information Center - Alagille syndrome
- Orphanet (ORPHA:51) - Alagille syndrome
- Online Mendelian Inheritance in Man (OMIM #118450) - Alagille Syndrome; ALGS
