Nephronophthisis 4 C1847013
An inherited condition leading to cysts in the kidneys and gradual loss of kidney function. It usually develops during childhood or adolescence. Dialysis or transplant may eventually be needed.
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.
Warning signs - seek immediate care
Renal failure
Portal hypertension
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Nocturia
(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
Kidney transplantation
Definitive treatment for end-stage renal disease caused by nephronophthisis. Involves surgical replacement of failed kidneys with a healthy donor kidney.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular renal function monitoring
Frequent blood tests, urine studies, and imaging to track disease progression and guide timing of intervention. Helps detect complications early.
Additional treatment options
Angiotensin-converting enzyme (ACE) inhibitors
Blood pressure medications that reduce proteinuria and slow kidney disease progression by decreasing intraglomerular pressure.
medicationRenal-friendly dietary modifications
Sodium, potassium, and phosphorus-restricted diet to manage electrolyte imbalances and reduce kidney workload as function declines.
dietaryErythropoiesis-stimulating agents
Hormone therapy to treat anemia associated with chronic kidney disease by stimulating red blood cell production.
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 4 C1847013?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Nephronophthisis 4 C1847013. 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 27, 2026. · Read our editorial policy