Dent Disease Type 1 C1848336
A rare X-linked kidney disorder affecting the proximal tubules, causing excessive protein and calcium in the urine, leading to kidney stones and potential kidney disease progression. Management focuses on symptom control and preventing complications.
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.
Warning signs - seek immediate care
Kidney stones (nephrolithiasis)
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Low molecular weight proteinuria
(renal)Nephrocalcinosis
(renal)Early-onset 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
Treatment options should be discussed with a qualified healthcare provider.
Thiazide Diuretic Therapy
Low-dose thiazide diuretics reduce urinary calcium excretion and help prevent kidney stone formation and progressive renal disease in Dent disease.
Kidney Stone Prevention Diet
Customized dietary plan reducing sodium and oxalate intake while maintaining adequate hydration to minimize nephrolithiasis risk.
Additional treatment options
Kidney Stone Intervention
Procedures such as lithotripsy or ureteroscopy for removal of symptomatic or obstructive stones causing pain or renal damage.
procedureRenal Function Surveillance
Regular monitoring of glomerular filtration rate, proteinuria, and kidney imaging to track disease progression and adjust treatment accordingly.
monitoringACE Inhibitor or ARB Therapy
Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers to reduce proteinuria and slow progression of renal disease.
medicationWhen 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 Dent Disease Type 1 C1848336?
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.
Bolignano D, Cheung M, Emma F, et al. KDIGO Clinical Practice Guideline for Glomerulonephritis: Tubular Disorders Section. Kidney Int Suppl. 2020.
Devuyst O, Thakker RV. Dent's disease and the proximal tubule: 20 years of molecular insights. Nat Rev Nephrol. 2019.
Scheinman SJ, Cox JP, Lloyd SE, et al. Genetics and clinical management of Dent disease. Mol Genet Genomic Med. 2018.
Ceballos-Picot I, Wiseman D, Niaudet P, et al. ClinicalTrials.gov Study: Thiazide Diuretics in Dent Disease (THIA-DENT). Pediatr Nephrol. 2022.
Medication Comparisons
Compare medications used to treat Dent Disease Type 1 C1848336. 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.
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