Skip to content
knowrare
HealthSignal
Your condition community

Living with Dent disease

A long-term kidney condition that mostly affects males, causing protein in the urine and kidney stones.

Understanding Dent disease

Dent disease is a chronic kidney condition that occurs almost only in males and is caused by damage to parts of the kidney called proximal tubules.

Signs appear in early childhood and worsen over time. The most common sign is a large amount of protein in the urine. Other signs include extra calcium in the urine, calcium deposits in the kidneys, and kidney stones. Some people develop rickets, a bone condition.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Large amounts of protein in the urine
  • Kidney stones
  • Blood in the urine
  • Abdominal pain
  • Bone pain
  • Long-term kidney disease

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include bone and joint specialists, genetic specialists, hormone specialists and kidney specialists.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

Care & treatment

Care team
Bone and joint specialists (orthopedists or orthopedic surgeons); Genetic specialists (geneticists); Hormone specialists (endocrinologists); Kidney specialists (nephrologists)
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Dent Disease Foundation and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Can genetic testing confirm Dent disease?
How often should kidney function and urine be checked?
How can we lower the chance of kidney stones?

Quick facts

Symptoms usually startChild
InheritanceX-Linked

GARD doesn't name a gene. It lists X-linked inheritance, meaning the gene is on the X chromosome.

GeneticKidneyEndocrine

Also known as: dent syndrome; dent's disease; low-molecular-weight proteinuria with hypercalciuria and nephrocalcinosis; renal fanconi syndrome with nephrocalcinosis and renal stones; x-linked recessive hypercalciuric hypophosphatemic rickets; x-linked recessive hypophosphatemic rickets

Patient organizations

Source: NIH GARD · Dent diseaseGARD ID 13105 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Dent disease.

Explore the Dent disease blog

Explore research for Dent disease

Explore actively recruiting Dent disease studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Dent disease with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • Eligibility is confirmed by the study team

Powered by HealthSpark, our AI chat helper

I can help you find recruiting Dent disease studies. Enter a ZIP code, address or city below and I'll look within 50 miles.

Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

Get Signals for Dent disease

Stay in the loop with community stories and updates from medical researchers.

HealthStoryfor Dent disease

Create a living journal that talks back

HealthStory evolves private journaling with a living AI journal that talks back and asks thoughtful questions, helping you capture your Dent disease experiences more deeply and prepare for better conversations with your doctors and care team.

Start your Dent disease journal

Your journal is private. Sharing your story publicly is a separate choice, made with Share your story above.