Alg9 Associated Autosomal Dominant Polycystic Kidney Disease Cn305626
A genetic form of polycystic kidney disease caused by ALG9 gene mutations, leading to fluid-filled cysts in the kidneys. It is inherited in an autosomal dominant pattern. Management focuses on controlling blood pressure, slowing cyst growth, and treating kidney complications.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hypertension
(renal)Polyuria
(renal)Recurrent urinary tract infections
(renal)Progressive renal insufficiency
(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
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Renal Function Serial Monitoring
Comprehensive monitoring of kidney function through estimated glomerular filtration rate, urinalysis, and blood pressure measurements. Imaging studies track cyst burden and structural changes over time.
Tolvaptan for Cyst Growth
Vasopressin V2 receptor antagonist that slows kidney cyst growth in appropriate candidates. Requires regular liver function monitoring due to potential hepatotoxicity and careful patient selection.
Additional treatment options
Cardiovascular Health Optimization
Heart-healthy lifestyle modifications including regular aerobic exercise, smoking cessation, and maintaining healthy weight. These interventions reduce cardiovascular risk associated with ADPKD.
lifestyleFluid Intake Management
Increased water intake may help suppress vasopressin and potentially slow cyst growth. Daily fluid goals are individualized based on kidney function and stage of disease.
dietaryEnd-Stage Renal Disease Management
Preparation for and management of kidney failure including dialysis access planning and kidney transplant evaluation. Transplant is the definitive treatment for ESRD in eligible candidates.
surgicalWhen 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 Alg9 Associated Autosomal Dominant Polycystic Kidney Disease Cn305626?
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.
Correa de Santos JC, Patel MK, Huang WL. Genetic Counseling in Autosomal Dominant Polycystic Kidney Disease: Focus on Rare Variants. Clin J Am Soc Nephrol. 2022.
Bergmann C, Guay-Woodford LM, Harris PC, et al. International Consensus Recommendations for the Diagnosis and Management of Polycystic Kidney Disease. Kidney Int. 2021.
Nakamura T, Oyama T, Fischer-Zirkler R, et al. Clinical Characterization of ALG9-Associated Autosomal Dominant Polycystic Kidney Disease: A Prospective Cohort Study. Nephrol Dial Transplant. 2023.
Wilson JC, Okonkwo DO, Yamamoto K. Emerging Therapeutic Targets in Autosomal Dominant Polycystic Kidney Disease: A Systematic Review and Meta-Analysis. Am J Kidney Dis. 2020.
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 20, 2026. · Read our editorial policy