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Dent disease

(dent)
[Charles Enrique Dent, Brit. biochemist, 1911-1976]
A rare, X-linked disease that causes renal tubular dysfunction and is characterized by secretion of abnormal amounts of calcium in the urine, kidney stone formation, rickets, and, eventually, renal failure.

SYMPTOMS AND SIGNS
Affected children usually report urinary frequency or symptoms of kidney stones (pain in the flank, urethra, or scrotum; and/or bloody urine).

DIAGNOSIS
The disease is suggested by the presence of abnormal calcium, phosphate, and protein concentrations in urine (hypercalciuria and proteinuria) in young patients with a history of kidney stones or chronic kidney disease. It can be confirmed with genetic testing.

TREATMENT
Treatment for hypercalciuria, such as restriction of dietary sodium, is often recommended.

PATIENT CARE
Dietary calcium should not be restricted. Oral citrates may reduce urinary calcium excretion. Patients with bone disease (rickets or osteomalacia) benefit from vitamin D supplements.

SYN: SEE: X-linked recessive nephrolithiasis

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Citation

Venes, Donald, editor. "Dent Disease." Taber's Medical Dictionary, 23rd ed., F.A. Davis Company, 2017. Taber's Online, www.tabers.com/tabersonline/view/Tabers-Dictionary/771513/all/Dent_disease.
Dent disease. In: Venes D, ed. Taber's Medical Dictionary. 23rd ed. F.A. Davis Company; 2017. https://www.tabers.com/tabersonline/view/Tabers-Dictionary/771513/all/Dent_disease. Accessed June 18, 2019.
Dent disease. (2017). In Venes, D. (Ed.), Taber's Medical Dictionary. Available from https://www.tabers.com/tabersonline/view/Tabers-Dictionary/771513/all/Dent_disease
Dent Disease [Internet]. In: Venes D, editors. Taber's Medical Dictionary. F.A. Davis Company; 2017. [cited 2019 June 18]. Available from: https://www.tabers.com/tabersonline/view/Tabers-Dictionary/771513/all/Dent_disease.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Dent disease ID - 771513 ED - Venes,Donald, BT - Taber's Medical Dictionary UR - https://www.tabers.com/tabersonline/view/Tabers-Dictionary/771513/all/Dent_disease PB - F.A. Davis Company ET - 23 DB - Taber's Online DP - Unbound Medicine ER -