Alport Syndrome 3b Autosomal Recessive C5882699
A form of inherited kidney disease affecting multiple organs. Both parents must carry the gene. Care includes kidney support and monitoring.
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
Hematuria
(renal)Proteinuria
(renal)Hearing loss
(renal)Corneal dystrophy
(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 inhibitor or ARB therapy
Aggressive renoprotective therapy with ACE inhibitors or angiotensin receptor blockers to significantly reduce proteinuria and delay end-stage renal disease progression. Often requires higher intensity treatment than autosomal dominant forms.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal replacement therapy planning
Advance care planning for dialysis or kidney transplantation when renal function declines. Autosomal recessive disease typically progresses more rapidly to end-stage renal disease.
Additional treatment options
Comprehensive multi-system surveillance
Regular monitoring of kidney function, hearing, and ocular manifestations. Requires coordination among nephrology, otolaryngology, and ophthalmology specialists.
monitoringCochlear implantation
Surgical intervention for severe hearing loss that does not respond to conventional hearing aids. Cochlear implants can significantly improve communication outcomes in affected individuals.
surgicalFamily-centered psychological support
Comprehensive psychological support addressing the significant burden of severe autosomal recessive disease. Includes coping strategies, family counseling, and connection to support networks.
psychologicalWhen 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 3b Autosomal Recessive C5882699?
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.
Knebelmann B, Gross O, Kashtan CE. KDIGO Clinical Practice Recommendations for Alport Syndrome. Kidney Int Suppl. 2022.
Hudson BG, Kalluri R, Miner JH. The Pathobiology of Alport Syndrome: Advances in Basic Science and Therapeutic Targets. Nat Rev Nephrol. 2021.
Storey H, Flinter F, Trompeter R. Clinical and Molecular Characterization of Autosomal Recessive Alport Syndrome. Clin J Am Soc Nephrol. 2020.
Gross O, Friede T, Hilgers R. Safety and Efficacy of ACE Inhibitors in Autosomal Recessive Alport Syndrome: A Randomized Controlled Trial. J Clin Invest. 2023.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy