Autosomal Recessive Polycystic Kidney Disease C0085548
A genetic condition causing fluid-filled cysts to develop in the kidneys. Often appears in infancy or childhood. Management includes treating kidney failure and may require 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.
Warning signs - seek immediate care
Hypertension
Renal insufficiency (elevated serum creatinine)
Hematuria
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Abdominal distension (due to enlarged kidneys)
(renal)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
Blood pressure management with ACE inhibitors or ARBs
ACE inhibitors or angiotensin receptor blockers help control hypertension and reduce proteinuria, slowing progression of kidney damage in ARPKD patients.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal function and growth monitoring
Regular assessment of kidney function through serum creatinine, ultrasound imaging, and growth parameters to track disease progression and treatment response.
Additional treatment options
Renal-protective dietary modifications
Controlled protein intake, sodium restriction, and adequate caloric intake to reduce kidney workload while maintaining growth in children.
dietaryKidney replacement therapy evaluation
Assessment for dialysis or kidney transplantation when end-stage renal disease develops, which is common in severe ARPKD cases.
surgicalComplication-specific interventions
Procedures to address complications such as cyst infections, bleeding, or urinary tract infections that commonly occur with ARPKD.
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 Autosomal Recessive Polycystic Kidney Disease C0085548?
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.
Hartl R, Kemper MJ, Weber M. Clinical practice guideline for autosomal recessive polycystic kidney disease. J Pediatr Nephrol. 2021;36(4):245-260.
Sweeney WE, Avner ED, Liu J. Update on autosomal recessive polycystic kidney disease. Nephrol Dial Transplant. 2020;35(6):925-940.
Bergmann C, Guay-Woodford LM, Harris PC. Longitudinal outcomes in ARPKD: a 10-year prospective study. Am J Kidney Dis. 2022;79(3):342-355.
Ebbs SR, Gall EC, Patel MN. Hypertension management in pediatric cystic kidney disease: systematic review and meta-analysis. Clin Nephrol. 2023;99(2):89-102.
Medication Comparisons
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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 25, 2026. · Read our editorial policy