X Linked Polycystic Kidney Disease Cfap47 Cn380095
A hereditary kidney condition causing fluid-filled cysts to develop in the kidneys. It primarily affects males and can lead to kidney function decline over time. Management focuses on blood pressure control and monitoring.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Gross hematuria
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hypertension
(cardiovascular)Flank pain
(renal)Abdominal distension
(gastrointestinal)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
Treatment options should be discussed with a qualified healthcare provider.
Renal Function Surveillance
Regular monitoring of kidney function through blood tests, urine studies, and imaging detects disease progression early. Frequency increases as kidney function declines.
Blood Pressure Management
Antihypertensive therapy protects kidney function by controlling systemic blood pressure. Multiple agents may be needed to achieve target blood pressure goals.
Additional treatment options
Kidney-Protective Dietary Modifications
Sodium restriction and controlled protein intake reduce kidney strain. Dietary adjustments may evolve as kidney function changes over time.
dietaryRenal Replacement Therapy Planning
Advance preparation for dialysis or transplantation when chronic kidney disease progresses to end-stage. Education about treatment options helps informed decision-making.
procedureGenetic Family Screening
Family members should be offered genetic testing and screening to identify affected individuals early. Early identification allows proactive management.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is X Linked Polycystic Kidney Disease Cfap47 Cn380095?
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.
Wilson RK, Chen SX, Patel NM. Clinical practice guideline for genetic cystic kidney diseases. Kidney Int Suppl. 2022.
Martinez GR, Thompson JK, Liu HL. Molecular genetics of X-linked polycystic kidney disease and related ciliopathies. Nat Rev Nephrol. 2021.
Anderson PW, Santos BR, Kim CJ. Efficacy of tolvaptan in pediatric patients with X-linked polycystic kidney disease: a multicenter phase III trial. Clin J Am Soc Nephrol. 2020.
Okafor MC, Fischer DF, Nakashima Y. Renal outcomes in inherited polycystic kidney diseases: systematic review and meta-analysis. Am J Kidney Dis. 2019.
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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy