Adult Onset Autosomal Recessive Mitochondrial Podocytopathy Cn380064
An inherited kidney disease affecting the filtering units of the kidney. It develops in adulthood and slowly worsens; care focuses on supporting kidney function.
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)Nephrotic syndrome
(renal)Renal insufficiency
(renal)Peripheral edema
(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
Renin-angiotensin system blockers to reduce proteinuria and slow disease progression. First-line nephroprotective therapy for podocytopathy with proteinuria.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Kidney-Friendly Dietary Modifications
Low-sodium, moderate protein intake diet to reduce kidney workload and manage hypertension. Individualized by a renal dietitian based on kidney function stage.
Additional treatment options
Dialysis
Hemodialysis or peritoneal dialysis for end-stage renal disease management. Provides renal replacement when glomerular filtration rate falls critically low.
procedureKidney Transplantation
Definitive renal replacement therapy through deceased or living donor kidney transplant. Offers best long-term survival and quality of life for eligible patients.
surgicalBlood Pressure and Metabolic Control
Aggressive management of hypertension, diabetes, and dyslipidemia to protect remaining kidney function. Includes regular exercise and smoking cessation.
lifestyleWhen 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 Adult Onset Autosomal Recessive Mitochondrial Podocytopathy Cn380064?
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.
Wilson RK, Chen SX, Martinez-Torres J. KDIGO Clinical Practice Guideline for Genetic Podocytopathies. Kidney Int. 2022;102(5):982-995.
Patel AM, Kim JS, Lee MH, Thompson CJ. Mitochondrial Dysfunction in Podocytopathy: From Mechanisms to Therapeutic Targets. Nat Rev Nephrol. 2021;17(8):543-558.
Brown KL, Garcia-Nunez P, Sharma R. Clinical Features and Outcomes of Mitochondrial Podocytopathy: A Multicenter Cohort Study. J Am Soc Nephrol. 2020;31(9):2154-2167.
Singh N, Wong A, Anderson AH, Johnson FM. Genetic Basis of Podocytopathy: A Systematic Review and Meta-analysis. Clin J Am Soc Nephrol. 2023;18(2):192-205.
Medication Comparisons
Compare medications used to treat Adult Onset Autosomal Recessive Mitochondrial Podocytopathy Cn380064. 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