Nephronophthisis 9 C3151188
An inherited disorder affecting kidney structure and function, leading to progressive kidney disease typically in youth.
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
Polyuria
(renal)Anemia
(renal)Hypertension
(renal)Retinitis pigmentosa
(ocular)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
Preemptive kidney transplantation
Kidney transplant performed before dialysis becomes necessary, potentially offering better outcomes and avoiding dialysis-related complications.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Serial kidney function assessments
Regular monitoring of creatinine, eGFR, and urinalysis to track disease progression and identify optimal timing for intervention.
Additional treatment options
Renin-angiotensin system blockade
ACE inhibitors or ARBs to reduce proteinuria and slow progression of chronic kidney disease through hemodynamic and anti-fibrotic effects.
medicationProtein restriction therapy
Moderate protein restriction to reduce kidney workload and slow progression of chronic kidney disease while maintaining adequate nutrition.
dietaryMetabolic acidosis correction
Sodium bicarbonate supplementation to correct metabolic acidosis that develops with declining kidney function and helps preserve muscle mass.
medicationWhen 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 Nephronophthisis 9 C3151188?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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 27, 2026. · Read our editorial policy