ICD10 code of Dent Disease and ICD9 code

Dent disease is classified under the ICD-10 code E72.03 (Low-molecular-weight proteinuria) and the ICD-9 code 270.8 (Other specified disorders of amino-acid transport). These codes are used by healthcare providers to document this rare X-linked renal tubulopathy in medical records and billing systems.

What is the clinical classification of Dent disease?

Dent disease is a rare genetic disorder characterized by proximal renal tubular dysfunction. Because it leads to the excretion of low-molecular-weight proteins in the urine, it is clinically categorized under the ICD-10 code E72.03. While Dent disease presents with complex symptoms like nephrocalcinosis and kidney stones, these specific diagnostic codes are essential for tracking the condition in clinical databases and helping our community of 10 members at DiseaseMaps.org coordinate their care.

Is Dent disease hereditary?

Yes, Dent disease is an X-linked recessive disorder, primarily caused by mutations in the CLCN5 gene (Dent disease 1) or the OCRL gene (Dent disease 2). Because it is X-linked, it predominantly affects males. Genetic counseling is highly recommended for families to understand the inheritance pattern and the potential for carrier status in female relatives.

What are the primary clinical features of Dent disease?

The progression of Dent disease typically involves chronic kidney issues that require long-term monitoring. Common clinical manifestations include:

• Hypercalciuria (excess calcium in the urine)


• Nephrocalcinosis (calcium deposits in the kidneys)


• Nephrolithiasis (kidney stones)


• Hypophosphatemia (low levels of phosphate in the blood)


• Progressive renal failure in adulthood

How is Dent disease managed?

Management of Dent disease focuses on symptom control and slowing the progression of renal damage. This often involves high fluid intake to prevent stones, the use of thiazide diuretics to manage hypercalciuria, and phosphate supplementation to address hypophosphatemia. Regular monitoring by a nephrologist is critical for all patients living with Dent disease.

Next steps

• Consult with a pediatric or adult nephrologist experienced in rare tubulopathies.


• Connect with the 10 community members at DiseaseMaps.org to share experiences and coping strategies.


• Request a referral to a clinical geneticist to discuss CLCN5 or OCRL gene testing.

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 with any questions regarding a medical condition.

References

• NIH GARD: Dent Disease (https://rarediseases.info.nih.gov/)


• Orphanet: Dent Disease (https://www.orpha.net/)


• OMIM: Dent Disease 1 (Entry #300009)


• The Dent Disease Foundation: Patient resources and research updates.