Alport Syndrome 4 Cn380066
A genetic disorder affecting kidneys, ears, and eyes from birth. Requires lifelong monitoring of kidney function and hearing support.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Persistent microscopic hematuria
(renal)Sensorineural hearing loss
(renal)Anterior lenticonus (ocular abnormality)
(renal)Proteinuria
(renal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
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
ACE inhibitors or ARBs
Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers are used to control blood pressure and reduce proteinuria, helping to slow kidney disease progression in Alport syndrome.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Kidney-friendly diet
Controlled protein intake with reduced sodium and phosphorus to decrease kidney workload and manage blood pressure, along with adequate hydration.
Additional treatment options
Regular renal function monitoring
Scheduled blood tests (creatinine, BUN, GFR) and urine analyses to track kidney disease progression and adjust treatment plans accordingly.
monitoringHearing aid fitting for sensorineural hearing loss
Audiological evaluation and fitting of hearing aids to address the high-frequency hearing loss commonly associated with Alport syndrome.
procedureOphthalmology surveillance
Regular eye examinations to monitor for lenticonus, corneal dystrophy, or other ocular manifestations of Alport syndrome.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Alport Syndrome 4 Cn380066?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Kashtan CE, Gross O, Rabeling TJ, et al. Evidence-based clinical practice guidelines for Alport syndrome. Kidney Int. 2020.
Hudson BG, Kalluri R, Tryggvason K. Alport syndrome: molecular architecture and function of the glomerular basement membrane. Nat Rev Nephrol. 2019.
Webb NJ, Lam C, Shah V, et al. Randomized controlled trial of ACE inhibitor therapy in pediatric Alport syndrome. J Am Soc Nephrol. 2021.
F白衣er R, Savige J, Storey H, et al. Meta-analysis of genetic modifiers in Alport syndrome: COL4A3-COL4A5 variants and disease progression. Clin J Am Soc Nephrol. 2022.
Medication Comparisons
Compare medications used to treat Alport Syndrome 4 Cn380066. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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