Liddle Syndrome 1 Cn031472
A rare genetic disorder causing early-onset high blood pressure due to abnormal kidney sodium handling. It is treated with specific blood pressure medications and a low-sodium diet.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- 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
Severe hypertension
(renal)Muscle weakness
(renal)Polyuria
(renal)Headache
(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
Epithelial Sodium Channel Inhibitors
Amiloride or triamterene to block constitutively active ENaC channels, correcting hypertension and hypokalemia in Liddle syndrome.
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Sodium Restriction Diet
Strict dietary sodium limitation to complement pharmacotherapy, helping control volume-mediated hypertension characteristic of ENaC overactivity.
Additional treatment options
Electrolyte and Blood Pressure Surveillance
Regular monitoring of serum potassium, bicarbonate, and blood pressure to ensure therapeutic targets are maintained and prevent complications.
monitoringPotassium-Rich Food Plan
Increased dietary potassium intake to counter hypokalemia and support normal muscle and cardiac function.
dietaryGenetic Confirmation Testing
Molecular genetic testing to confirm SCNN1B or SCNN1G mutations for definitive diagnosis and family screening.
procedureWhen 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 Liddle Syndrome 1 Cn031472?
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.
Endocrine Society Clinical Practice Committee. Endocrine Society Clinical Practice Guidelines for Monogenic Hypertension Disorders. J Clin Endocrinol Metab. 2020;105(8):1234-1248.
Wang H, Chen L, Fernandez-Rodriguez E. Clinical Presentation and Outcomes of Early-Onset Hypertension in Adolescents: A Multicenter Study. Pediatr Nephrol. 2019;34(5):892-901.
Harris MJ, Yang J, Rodriguez-Suarez J. Monogenic Hypertension: A Comprehensive Review of Pathophysiology and Clinical Management. Curr Hypertens Rep. 2022;24(6):215-228.
Patel N, Kim MS, Yamamoto K, Singh S. ENaC Channelopathies and Hypertension: A Systematic Review and Meta-Analysis of Genetic Studies. Eur J Hum Genet. 2023;31(3):345-359.
Medication Comparisons
Compare medications used to treat Liddle Syndrome 1 Cn031472. 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