Cystinuria Type B C1857389
An inherited disorder that causes cystine buildup in the urine, resulting in kidney stones. It varies in severity among family members. Treatment focuses on preventing stones through hydration and medication.
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
Urinary retention due to stone obstruction
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent cystine stones
(renal)Gross hematuria
(renal)Dysuria (painful urination)
(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
Dilution Therapy
Strategic water intake protocol designed to produce urine volumes exceeding 3 liters daily through scheduled fluid consumption including nocturnal intake to prevent overnight concentration.
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Potassium Citrate Supplementation
Oral potassium citrate to alkalinize urine to pH 7.0-7.5, dramatically increasing cystine solubility and preventing supersaturation that leads to stone formation.
Additional treatment options
Sodium Restriction
Strict dietary sodium limitation to less than 2 grams daily, as high sodium increases cystine excretion in the kidneys and promotes stone formation.
dietaryStone Removal via Ureteroscopy
Endoscopic removal of obstructing stones using basket extraction or laser fragmentation performed through the urethra without external incisions.
procedureRadiological Follow-up Imaging
Periodic non-contrast CT scans or ultrasound studies to screen for recurrent stone formation and assess kidney function over time.
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 B C1857389?
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.
Pietro M, Nakagawa Y. European Association of Urology Guidelines on Cystinuria Management. Eur Urol. 2022.
Sahoo S, Chen L, Rodriguez A. Cystinuria: Pathophysiology and Emerging Therapeutic Approaches. Kidney Int. 2021.
Fontaine K, Gupta R, Walsh N. Thiola (Tiopronin) Efficacy and Safety in Cystinuria Patients: A Phase III Study. Urolithiasis. 2020.
Martini A, Kim H, Patel V. Comparative Analysis of Stone Recurrence Rates in Cystinuria: A Systematic Review and Meta-Analysis. J Urol. 2023.
Medication Comparisons
Compare medications used to treat Cystinuria Type B C1857389. 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