What Is Dent Disease?

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  • Written By: Meshell Powell
  • Edited By: O. Wallace
  • Last Modified Date: 07 September 2019
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Dent disease, also known as Dent's disease, is a rare type of inherited kidney disease which leads to damage involving the tubules of the kidneys. Some of the most common symptoms associated with this disease include kidney stones, extreme thirst, and dehydration. There may be higher than normal levels of calcium, potassium, and other substances in the urine of those with Dent disease, sometimes leading to kidney failure. There is no standard treatment for this disease, and the primary focus is on managing individual symptoms. Prescription medications and dietary changes may be used to help control some of the symptoms. Any questions or concerns about Dent disease or the most appropriate treatment options available for an individual situation should be discussed with a doctor or other medical professional.

The term Dent disease is often used to describe any number of health issues which stem from gene mutation involving the X chromosome. Most of these issues involve the kidneys and may become so severe that the kidneys are not able to function well enough to support the life of the patient. As is the case with any condition caused by mutations of the X chromosome, women tend to only be carriers for this disease, while the vast majority of patients are male.


Nephrolithiasis is a medical term used to describe the presence of kidney stones, which is common among those with Dent disease. Nephrocalcinosis is a medical condition that causes an abnormal buildup of calcium salts within the kidneys, often leading to a progressive loss of kidney function. Proteinuria, or the presence of protein in the urine, is frequently found among patients with this disease as well.

An accumulation of substances such as glucose, potassium, and phosphates in the urine may occur in those with Dent disease. A condition known as rickets is also relatively common with this disease. Rickets is a condition which causes the bones to become abnormally softened due to a deficiency of Vitamin D, phosphorous, calcium, or magnesium. Symptoms may include bone pain, muscle weakness, or the development of dental problems.

While there is no specifically approved treatment protocol available for those with Dent disease, the individual symptoms can sometimes be managed with a combination of dietary changes and the use of prescription medications. If the kidney damage becomes severe, dialysis or a kidney transplant may become necessary in order to save the life of the patient. The supervising physician can help the patient devise the most appropriate treatment plan for an individual situation.



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