Nephronophthisis Like Nephropathy 2 C5561953
A hereditary kidney disease similar to nephronophthisis, leading to gradual loss of 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
Polyuria
(renal)Anemia
(renal)Hypertension
(renal)Skeletal dysplasia
(musculoskeletal)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
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal function and growth monitoring
Tracking kidney function parameters along with growth and development parameters, particularly important in pediatric patients.
Protein restriction therapy
Carefully managed protein intake to reduce kidney workload while maintaining adequate nutrition for growth and tissue maintenance.
Additional treatment options
Renal protective pharmacotherapy
Use of renin-angiotensin-aldosterone system inhibitors to reduce hyperfiltration stress on remaining nephrons.
medicationGenetic counseling services
Family genetic counseling to understand inheritance patterns, family planning options, and potential screening for at-risk relatives.
complementaryDialysis preparation and initiation
Education and preparation for eventual dialysis, including vascular access planning and modality selection.
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 Nephronophthisis Like Nephropathy 2 C5561953?
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 Like Nephropathy 2 C5561953. 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