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Cystinuria Type A C1857388

Published: July 22, 2026Updated: August 20, 2026

An inherited disorder causing excess cystine in the urine, leading to kidney stones. It typically runs in families. Management includes high fluid intake and medications to reduce stone formation.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

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

Recurrent cystine stones

(renal)

Microscopic hematuria

(renal)

Renal insufficiency (chronic)

(renal)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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

1

High Fluid Intake Protocol

Comprehensive hydration strategy requiring consumption of at least 3-4 liters of fluids distributed throughout the day and night to maintain dilute urine and prevent stone formation.

lifestyle
2

Renal disease management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
3

Cystine-Reducing Diet

Low-sodium, moderate protein diet that restricts methionine-rich foods while maintaining adequate nutrition to minimize cystine excretion in urine.

dietary

Additional treatment options

Thiol-Based Chelation Therapy

Administration of cystine-binding medications that chemically modify cystine molecules in the kidneys to form highly soluble compounds that prevent crystallization.

medication

Metabolic Stone Surveillance

Regular monitoring program including periodic imaging, urine chemistry analysis, and blood tests to detect recurrent stone formation early and adjust management.

monitoring

Herbal Support with Hydrangea

Traditional herbal preparations containing hydrangea root and related compounds traditionally used to support urinary tract health and stone passage.

complementary

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Cystinuria Type A C1857388?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Peters C, Lindqvist J, Müller R. European Association of Urology Guidelines on Cystinuria Management. Eur Urol. 2022.

guideline2022
2

Sato H, Chen K, Williams R. Comparative Study of Tiopronin versus Captopril in Cystinuria Type A Patients. Kidney Int Rep. 2021.

clinical_trial2021
3

García-Navarro M, Anderson P, Thompson N. Current Understanding of SLC3A1 Mutations in Type A Cystinuria. Nat Rev Nephrol. 2020.

review2020
4

Kimura T, Santos F, Weber H. Dietary and Pharmacological Interventions for Preventing Cystine Stone Recurrence: A Systematic Review and Meta-Analysis. Urolithiasis. 2023.

meta_analysis2023

Medication Comparisons

Compare medications used to treat Cystinuria Type A C1857388. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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