Nphp3 Related Disorder Cn379163
A genetic ciliopathy causing cystic kidney disease and sometimes situs inversus. It affects children and leads to kidney failure. Treatment focuses on managing kidney failure with dialysis or transplant.
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)Polydipsia
(renal)Growth retardation
(endocrine)Retinal dystrophy
(eye)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 surveillance
Regular monitoring of kidney function through serum creatinine, urinalysis, and imaging helps detect progression of renal disease in NPHP3-related disorder.
Blood pressure management
ACE inhibitors or ARBs help control hypertension and may slow progression of renal disease by reducing proteinuria and glomerular pressure.
Additional treatment options
Renal-protective dietary modifications
Low-sodium diet, controlled protein intake, and phosphorus restriction help manage hypertension and slow CKD progression.
dietaryDialysis initiation
When CKD progresses to end-stage renal disease, dialysis provides renal replacement therapy as a bridge to potential transplantation.
procedureKidney transplantation evaluation
Kidney transplantation is the definitive treatment for ESRD in NPHP3, offering improved long-term outcomes compared to dialysis.
surgicalWhen 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 Nphp3 Related Disorder Cn379163?
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.
Bergmann C, Kemper M, Mueller GA. Ciliopathies and renal disease: Molecular perspectives. Pediatr Nephrol. 2022.
Hoff S, Helwig JC, Blaser H, et al. Clinical management of nephronophthisis and related ciliopathies: Consensus guidelines. Nat Rev Nephrol. 2021.
Tory K, László B, Szabó Z, et al. Clinical phenotype and genetic characterization of NPHP3-related disorders. Kidney Int. 2020.
Schueler M, Graewe S, Zhang W, et al. Systematic review of renal outcomes in NPHP3-related ciliopathies: A meta-analysis. J Nephrol. 2023.
Medication 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy