Combined renal tubular acidosis and diabetes insipidus in hematological disease

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Background A 39-year-old male with multiple myeloma was admitted for treatment with melphalan and autologous stem cell reinfusion. He presented with hypokalemia and hyperchloremic non-anion-gap metabolic acidosis with a high urinary pH. He also had hypomagnesemia, hypophosphatemia, hypouricemia, proteinuria and glucosuria. The patient subsequently developed polyuria with a low urine osmolality, hypernatremia and, finally, acute renal failure.

Investigations Physical examination, blood and urine analyses, kidney biopsy and tonicity balance.

Diagnosis Fanconi syndrome with proximal (type II) renal tubular acidosis caused by myeloma kidney. Renal tubular acidosis was complicated by probable nephrogenic diabetes insipidus and acute renal failure.

Management Potassium supplementation, sodium bicarbonate therapy, intravenous fluid therapy and dialysis.

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Figure 1: Tonicity balances showing two different mechanisms of hypernatremia
Figure 2: Kidney biopsy of the main case


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We thank Dr IM Bajema, who analyzed the kidney biopsy, Dr B van den Berg and Dr MR Korte, who were also involved in the treatment of these patients, and Dr ML Halperin for critical reading of this manuscript.

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Correspondence to Ewout J Hoorn.

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The authors declare no competing financial interests.

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