Nephronophthisis 8 Cn119610
A rare inherited kidney disease causing cysts and progressive kidney failure, often affecting children and young adults.
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
Increased thirst sensation
(renal)Progressive renal insufficiency
(renal)Lethargy
(neurologic)Hypertension
(cardiovascular)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
Dialysis preparation and initiation
Preparation for and initiation of either hemodialysis or peritoneal dialysis when kidney function falls below critical thresholds.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Progressive disease surveillance
Comprehensive monitoring of kidney function, electrolyte levels, growth metrics, and early detection of extrarenal manifestations.
Additional treatment options
Phosphate binders
Medications that bind dietary phosphate in the gut to prevent hyperphosphatemia and associated bone and vascular complications.
medicationFluid and hydration management
Careful fluid intake monitoring and management appropriate to kidney function stage to prevent dehydration and electrolyte disturbances.
lifestyleMultidisciplinary supportive care
Coordinated care involving nephrologists, dietitians, social workers, and genetic counselors to address comprehensive patient needs.
complementaryWhen 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 8 Cn119610?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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