Dent’s disease may also be associated with kaliuresis, phosphaturia, uricosuria.
Dent disease is a genetic kind of liberal renal bankruptcy. It is one reason of Fanconi syndrome, and is characterized by tubular proteinuria, hypercalciuria, calcium nephrolithiasis, nephrocalcinosis and chronic renal bankruptcy. Because of its rather rare occurrence, Dent’s disease is often diagnosed as idiopathic hypercalciuria, i.e. excess calcium in urine with undetermined causes. It is due to mutations that inactivate a voltage-gated chloride channel, named CLC-5, which is expressed in the kidney and is encoded by a gene at Xp11.22. The condition is familial, affecting both males and females in equal numbers, but males are more severely affected than females. In males, it tends to present in childhood or early adult life with symptoms of renal calculi, rickets or even with renal failure.