ICD10 code of Alport Syndrome and ICD9 code

Alport Syndrome is primarily classified under the ICD-10-CM code Q87.89 (Other specified congenital malformations syndromes, not elsewhere classified) or N07 (Hereditary nephropathy, not elsewhere classified), while the legacy ICD-9-CM code is 753.8. These codes are essential for medical billing, insurance authorization, and clinical documentation for patients managing this condition.

What exactly is Alport Syndrome?

Alport Syndrome is a rare genetic disorder characterized by progressive kidney disease, sensorineural hearing loss, and ocular abnormalities. It is caused by mutations in the COL4A3, COL4A4, or COL4A5 genes, which provide instructions for making type IV collagen, a critical structural protein in the basement membranes of the kidneys, inner ears, and eyes. Understanding the specific genetic variant of Alport Syndrome is vital for determining the inheritance pattern and long-term prognosis.

How is Alport Syndrome classified in medical coding?

While coding can be complex, healthcare providers typically use the following classifications to track Alport Syndrome within the electronic health record:

• ICD-10-CM Q87.89: Often used to denote the syndromic nature of the condition.


• ICD-10-CM N07: Specifically identifies hereditary nephropathy, which is the hallmark clinical feature of Alport Syndrome.


• ICD-9-CM 753.8: The historical code used prior to the transition to ICD-10.


• OMIM #104200: The clinical reference code used for X-linked Alport Syndrome in genetic databases.

Is Alport Syndrome hereditary?

Yes, Alport Syndrome is an inherited condition. Approximately 80% of cases are X-linked, meaning the mutation is on the X chromosome, while the remaining cases follow autosomal recessive or, more rarely, autosomal dominant inheritance patterns. Because Alport Syndrome is a multisystem disorder, family genetic counseling is strongly recommended for all newly diagnosed individuals.

How do patients manage their journey?

Living with a chronic condition like Alport Syndrome can be isolating, but you are not alone. Currently, 115 people with Alport Syndrome have joined the DiseaseMaps.org community to share their experiences and support one another through the challenges of renal monitoring and treatment.

Next steps

• Consult with a nephrologist to discuss your specific ICD coding and long-term renal care plan.


• Request a referral to a clinical geneticist to confirm your specific gene mutation.


• Connect with the 115 members of the Alport Syndrome community on DiseaseMaps.org for peer support.


• Monitor the National Kidney Foundation for updates on clinical trials targeting Alport Syndrome.

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 regarding a medical condition.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Alport Syndrome.


• Orphanet: Rare Disease Database (ORPHA:647).


• Online Mendelian Inheritance in Man (OMIM): Entry #104200.


• Alport Syndrome Foundation: Patient Resources and Clinical Guidance.