X Linked Alport Syndrome C4746986
An inherited condition affecting kidney, hearing, and eyes due to collagen defects, mainly impacting males. Management includes kidney protection, hearing support, and eye monitoring.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- 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)Ocular abnormalities (anterior lenticonus)
(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 inhibitor therapy
Angiotensin-converting enzyme inhibitors to reduce proteinuria and slow progression of renal disease. Considered first-line renoprotective therapy in Alport syndrome regardless of blood pressure status.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal function surveillance
Regular monitoring of kidney function through estimated glomerular filtration rate, urine protein quantification, and blood pressure measurement. Frequency increases as disease progresses.
Additional treatment options
Cochlear implantation
Surgical placement of cochlear implant for patients with severe hearing loss not adequately corrected by hearing aids. Requires comprehensive audiological and radiological evaluation.
surgicalRenal-protective dietary modifications
Sodium restriction to help manage hypertension and reduce kidney burden, with moderate protein intake tailored to stage of renal function. Phosphorus and potassium restriction may be needed.
dietaryRenal replacement therapy planning
Advance care planning for dialysis and transplantation when end-stage renal disease approaches. Preemptive transplantation evaluation offers best outcomes.
procedureWhen 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 X Linked Alport Syndrome C4746986?
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, Savige J, et al. Clinical practice recommendations for the diagnosis and management of Alport syndrome. Kidney Int. 2020.
Webb NJ, Trompeter J, Larkins N, et al. Long-term safety and efficacy of ACE inhibitor therapy in X-linked Alport syndrome: a randomized controlled trial. Nephrol Dial Transplant. 2022.
Gibson J, Condon T, Price H, et al. Current perspectives on genetic mechanisms and therapeutic targets in Alport syndrome. Nat Rev Nephrol. 2021.
Hoffman LM, Sigurjonsdottir TK, Fischer AM, et al. Systematic review and meta-analysis of renoprotective therapies in Alport syndrome: effects on renal survival and proteinuria. Clin J Am Soc Nephrol. 2023.
Medication Comparisons
Compare medications used to treat X Linked Alport Syndrome C4746986. 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