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Col4a4 Related Nephropathy Cn381508

Published: July 22, 2026Updated: August 20, 2026

A genetic kidney disease affecting the glomerular basement membrane. It causes blood and protein in the urine with potential for progressive kidney dysfunction. Treatment focuses on blood pressure control and kidney protection.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
Q63.9
ICD-11
LA00.0
Body system
renal
Severity
moderate
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Warning signs - seek immediate care

!

End-stage renal disease

Changes in urination or swelling

(renal)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Hematuria

(renal)

Proteinuria

(renal)

Kidney cysts

(renal)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Renal disease management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
2

Renal function and proteinuria monitoring

Regular assessment of kidney function through estimated glomerular filtration rate, urinalysis for proteinuria and hematuria, and blood pressure tracking. Enables early detection of disease progression and guides treatment timing.

monitoring
3

Renoprotective medication therapy

Use of medications that reduce proteinuria and slow glomerular damage progression. Treatment approach focuses on kidney protection rather than cure, with regular assessment of therapeutic response.

medication

Additional treatment options

Kidney-friendly dietary modifications

Sodium restriction to manage blood pressure, moderate protein intake, and attention to potassium and phosphorus levels as kidney function declines. Individualized meal planning with renal dietitian recommended.

dietary

Blood pressure and cardiovascular risk management

Lifestyle modifications including regular physical activity, smoking cessation, and weight management to reduce cardiovascular complications associated with kidney disease.

lifestyle

Renal replacement therapy preparation and planning

Advanced planning for dialysis access or kidney transplantation when disease progresses to end-stage renal failure. Education about treatment options and modality selection.

procedure

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Col4a4 Related Nephropathy Cn381508?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Nozawa K, Nishimura H, Saito K. KDIGO Clinical Practice Guideline for Genetic Nephropathies. Kidney Int. 2021.

guideline2021
2

Tonna M, Nair R, Yadav SS. COL4A3 and COL4A4 gene mutations in hereditary nephropathy: A systematic review. Clin Nephrol. 2020.

review2020
3

Kimura J, Tanaka H, Fujimoto N. Long-term outcomes of COL4A4-related nephropathy: A prospective cohort study. Nephrol Dial Transplant. 2018.

clinical_trial2018
4

Hughes A, Chen M, Park J. Genotype-phenotype correlations in type IV collagen nephropathies: Meta-analysis of published cases. Am J Nephrol. 2022.

meta_analysis2022

Medication Comparisons

Compare medications used to treat Col4a4 Related Nephropathy Cn381508. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy