Familial Juvenile Hyperuricemic Nephropathy Type 2 C2751310
A hereditary form of kidney disease with elevated uric acid beginning in adolescence. Early treatment helps slow kidney damage and control gout symptoms.
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
Hyperuricemia
(renal)Gout
(renal)Proteinuria
(renal)Renal failure
(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
Active treatment with regular monitoring by a healthcare provider is recommended.
Uricosuric Therapy
Medications that enhance renal uric acid excretion, addressing the transport defect characteristic of this condition. This approach differs from production-inhibiting therapies and may be used alone or in combination.
Kidney Ultrasound Screening
Regular imaging to detect medullary nephrocalcinosis or renal cysts associated with hyperuricemic nephropathy. Early structural changes may precede functional decline and guide treatment intensification.
Additional treatment options
Alkaline Diet Supplementation
Dietary approaches and supplements to alkalinize urine, improving uric acid solubility and reducing crystal precipitation in renal tubules. This complements pharmacological urate management.
dietaryFebuxostat for Xanthine Oxidase Inhibition
Selective xanthine oxidase inhibitor particularly useful in patients who cannot tolerate allopurinol. This provides an alternative mechanism for reducing uric acid production.
medicationHigh Fluid Intake Protocol
Aggressive hydration strategy of at least 3 liters daily to maintain dilute urine and prevent uric acid crystal formation in the collecting system. This is particularly important given the inherent renal concentrating defect.
lifestyleWhen 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 Familial Juvenile Hyperuricemic Nephropathy Type 2 C2751310?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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 20, 2026. · Read our editorial policy