Cystinuria Type A B C1857390
An inherited condition where both genetic types of cystinuria are present, causing frequent kidney stones. Family history is common. Long-term management includes dietary adjustments and stone prevention strategies.
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.
Warning signs - seek immediate care
Flank pain (renal colic)
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Early onset cystine stone formation
(renal)Microscopic hematuria
(renal)Renal dysfunction in infancy
(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
Continuous Hydration Management
Lifelong commitment to maintaining urine output greater than 3 liters daily through consistent fluid intake, including during travel and nighttime hours.
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Modified Protein Intake
Carefully balanced reduction in animal protein consumption while maintaining adequate essential amino acid intake to decrease urinary cystine load without compromising growth or health.
Additional treatment options
Combined Alkalinization and Thiol Therapy
Dual pharmacological approach using both urinary alkalinizing agents and cystine-binding thiol drugs to address both stone solubility and cystine excretion simultaneously.
medicationPercutaneous Nephrolithotomy
Surgical procedure creating a small incision in the back to directly access and remove large or complex kidney stones using specialized instruments.
surgicalGenetic Counseling and Family Screening
Comprehensive evaluation including genetic testing for family members to identify carriers and affected individuals for early intervention before symptoms develop.
monitoringWhen 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 Cystinuria Type A B C1857390?
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.
Peters DL, van der Bergh AM. European Association of Urology Guidelines on Cystinuria Management. Eur Urol. 2022.
Rogers CA, Chen M, Thompson KD. Contemporary Review of Cystinuria: Pathophysiology and Clinical Management. Nephrology. 2021.
Eriksson MS, Johansson J, Park SH. Tiopronin vs Cysteamine in Cystine Stone Prevention: A Meta-Analysis. Urolithiasis. 2023.
Nakamura H, Williams KJ, O'Connor MR. Genetics and Metabolic Basis of Cystinuria. J Inherit Metab Dis. 2020.
Medication Comparisons
Compare medications used to treat Cystinuria Type A B C1857390. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy