Short answer · Medically reviewed summary · Last updated: 2026-05-08
Bartter's syndrome is primarily coded as E26.89 in ICD-10 (other hyperaldosteronism), while the older ICD-9-CM system classified it under 255.14. Because Bartter's syndrome is a group of rare renal tubular disorders, specific clinical documentation is essential for accurate medical billing and diagnostic clarity. What exactly is Bartter's syndrome? Bartter's syndrome is a rare, inherited salt-wasting tubulopathy that affects the kidneys' ability to reabsorb sodium and chloride.
ICD10 code of Bartter's Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Bartter's Syndrome, with classification details for clinicians, coders and patients.
Bartter's syndrome is primarily coded as E26.89 in ICD-10 (other hyperaldosteronism), while the older ICD-9-CM system classified it under 255.14. Because Bartter's syndrome is a group of rare renal tubular disorders, specific clinical documentation is essential for accurate medical billing and diagnostic clarity.
What exactly is Bartter's syndrome?
Bartter's syndrome is a rare, inherited salt-wasting tubulopathy that affects the kidneys' ability to reabsorb sodium and chloride. Patients with Bartter's syndrome experience significant electrolyte imbalances, including hypokalemia (low potassium), metabolic alkalosis, and often hypercalciuria. With 61 people currently sharing their experiences on DiseaseMaps.org, we understand that living with the chronic fluid and electrolyte management required by Bartter's syndrome can be both physically and emotionally taxing.
How is Bartter's syndrome classified and coded?
While the ICD-10 code E26.89 is the most common designation, clinicians must often specify the subtype of Bartter's syndrome to ensure appropriate care. The condition is categorized into several types based on the genetic defect, which helps guide long-term management strategies.
- Type I and II: Often present with neonatal onset and severe salt wasting.
- Type III: Known as classic Bartter's syndrome, which may present later in childhood.
- Type IV: Characterized by sensorineural deafness alongside renal symptoms.
- Type V: Associated with CASR gene mutations and hypocalcemia.
Is Bartter's syndrome hereditary?
Yes, Bartter's syndrome is a genetic condition typically inherited in an autosomal recessive pattern, meaning both parents must carry a mutation in the same gene. Genetic counseling is highly recommended for families affected by Bartter's syndrome to understand the recurrence risks and the implications for future generations.
Next steps
- Consult a pediatric or adult nephrologist who specializes in tubulopathies to manage electrolyte replacement therapy.
- Engage with the 61 community members on DiseaseMaps.org to share coping strategies for managing chronic medication regimens.
- Discuss genetic testing options with a clinical geneticist to confirm the specific subtype of Bartter's syndrome.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding any medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Bartter syndrome.
- Orphanet: Rare disease database, Bartter syndrome entry (ORPHA:120).
- OMIM (Online Mendelian Inheritance in Man): Clinical synopsis for Bartter syndrome.
- The Bartter Site: Patient-focused resources for rare renal disorders.
