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Dent disease is an X-linked hereditary disorder characterized by impaired reabsorption in the proximal kidney tubules. Clinical manifestations include low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, and nephrolithiasis. There is currently no specific treatment for Dent disease, and its management is largely supportive. Preventive strategies against stone formation include adequate fluid intake, and in cases of hypercalciuria or nephrocalcinosis, dietary salt restriction, thiazide diuretics, or citrate supplementation are indicated. We report a case of Dent disease presenting with nephrocalcinosis without hypercalciuria.
The patient was a 5-year-old boy with no significant past medical history, and a family history of urolithiasis on his maternal side. At 6 months of age, he presented with fever, and laboratory tests revealed marked proteinuria (urine protein/creatinine ratio [UP/Cr] 3.25 g/gCr). Persistent proteinuria and an elevated urinary β2-microglobulin/creatinine ratio (316.5 μg/mgCr) raised suspicion of Dent disease, and kidney ultrasonography at the previous hospital was normal. He was referred to our hospital at 1 year and 1 month. His height was 76.2 cm (+0.08 SD) and weight 11.2 kg (+1.88 SD). Physical examination revealed no abnormalities. Laboratory tests showed mild proteinuria (UP/Cr of 0.60 g/gCr), normal calcium excretion (urine calcium/creatinine ratio 0.19 mg/mgCr), and preserved kidney function (estimated glomerular filtration rate [Cr-eGFR] 93.0 mL/min/1.73 m²). Genetic analysis identified a missense variant in CLCN5 (NM_000084.4:c.512C>T [p.Pro171Leu]), confirming Dent disease 1. Kidney ultrasonography performed at 3 years and 11 months demonstrated fine calcifications in both kidneys. At 4 years and 5 months, additional fine calcifications were noted in the right kidney, and citrate therapy was consequently initiated at 4 years and 10 months. After 3 months of treatment, calcifications persisted, while kidney function remained stable (Cr-eGFR 93.01 mL/min/1.73 m²).
In our case, the patient maintained adequate fluid and salt intake and did not demonstrate hypercalciuria; therefore, conservative management without pharmacological intervention was initially adopted. However, fine nephrocalcinosis appeared during follow-up. These findings suggest the importance of regular kidney ultrasonography, in addition to urine and blood testing, for the early detection and ongoing monitoring of nephrocalcinosis, even in patients without hypercalciuria. Thiazide diuretics enhance calcium reabsorption in the distal tubules and are used in the management of hypercalciuria. Citrate has been shown in ClC-5 knockout mice to exert kidney-protective effects through calcium-chelating action, while in humans, there is no clinical evidence that it can suppress the progression of kidney function decline. In the present case, considering the potential adverse effects of thiazide diuretics, including dehydration, hypotension, and hypercalcemia, citrate supplementation was selected, and careful follow-up of kidney calcification will be continued.
In patients with Dent disease, even in the absence of hypercalciuria, regular kidney ultrasonography is essential for the screening of nephrocalcinosis.
