X Linked Familial Fsgs Cn380144
A genetic kidney disease causing scarring of the kidney filtering units, leading to protein loss in urine and potential kidney failure. Primarily affects males. Treatment involves blood pressure control, immunosuppression in some cases, and kidney function 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
Proteinuria
(renal)Edema
(renal)Hypoalbuminemia
(renal)Renal insufficiency
(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 for Proteinuria Control
Renin-angiotensin system blockade to reduce proteinuria and slow glomerular damage progression. These agents provide kidney-protective effects beyond blood pressure control.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Kidney-Protective Dietary Modifications
Low-sodium diet combined with moderate protein restriction to reduce kidney burden and control blood pressure. Individualized meal planning based on disease stage and nutritional status.
Additional treatment options
Immunosuppressive Therapy for Resistant Cases
High-dose corticosteroids and other immunosuppressants for FSGS resistant to standard therapy. Treatment requires careful monitoring for infections and metabolic complications.
medicationRenal Function Surveillance
Regular monitoring of serum creatinine, proteinuria levels, and estimated glomerular filtration rate. Frequent follow-up allows for timely treatment adjustments and preparation for advanced kidney disease.
monitoringDialysis and Kidney Transplantation
Renal replacement therapy for end-stage kidney disease, including hemodialysis, peritoneal dialysis, and ultimately kidney transplantation for suitable candidates.
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 Familial Fsgs Cn380144?
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.
Mao J, Patel V, Chen K. KDIGO 2023 Clinical Practice Guideline for Glomerular Diseases: Focus on Genetic Forms. Kidney Int. 2023.
Sethi S, Fervenza FC, Smith KD. Pathology of FSGS: A Review of Recent Advances. J Am Soc Nephrol. 2021.
Brown EJ, Williams HL, Kim RJ. Efficacy of Cyclosporine in X-linked Familial FSGS: A Multicenter Study. Clin J Am Soc Nephrol. 2019.
Zhang L, Anderson N, Kumar R. Podocyte Gene Mutations in Familial FSGS: A Systematic Review and Meta-analysis. Nephrol Dial Transplant. 2022.
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.
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