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Combined renal tubular acidosis and diabetes insipidus in hematological disease

Nature Clinical Practice Nephrology volume 3, pages 171–175 (2007)Cite this article

Abstract

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

References

  1. Rose BD and Post TW (2001) Clinical Physiology of Acid–Base and Electrolyte Disorders, edn 5. New York: McGraw-Hill

    Google Scholar 

  2. Stinebaugh BJ et al. (1981) Pathogenesis of distal renal tubular acidosis. Kidney Int 19: 1–7

    Article  CAS  Google Scholar 

  3. Messiaen T et al. (2000) Adult Fanconi syndrome secondary to light chain gammopathy: clinicopathologic heterogeneity and unusual features in 11 patients. Medicine (Baltimore) 79: 135–154

    Article  CAS  Google Scholar 

  4. Smithline N et al. (1976) Light-chain nephropathy: renal tubular dysfunction associated with light-chain proteinuria. N Engl J Med 294: 71–74

    Article  CAS  Google Scholar 

  5. Elisaf M and Siamopoulos KC (1995) Fractional excretion of potassium in normal subjects and in patients with hypokalaemia. Postgrad Med J 71: 211–212

    Article  CAS  Google Scholar 

  6. Sanders PW et al. (1990) Mechanisms of intranephronal proteinaceous cast formation by low molecular weight proteins. J Clin Invest 85: 570–576

    Article  CAS  Google Scholar 

  7. Matsumura Y et al. (1999) Overt nephrogenic diabetes insipidus in mice lacking the CLC-K1 chloride channel. Nat Genet 21: 95–98

    Article  CAS  Google Scholar 

  8. Bachmann S et al. (2005) Renal effects of Tamm–Horsfall protein (uromodulin) deficiency in mice. Am J Physiol Renal Physiol 288: F559–F567

    Article  CAS  Google Scholar 

  9. Kim SW et al. (2001) Amphotericin B decreases adenylyl cyclase activity and aquaporin-2 expression in rat kidney. J Lab Clin Med 138: 243–249

    Article  CAS  Google Scholar 

  10. Héliès-Toussaint C et al. (2000) Cellular localization of type 5 and type 6 ACs in collecting duct and regulation of cAMP synthesis. Am J Physiol Renal Physiol 279: F185–F194

    Article  Google Scholar 

  11. Marples D et al. (1996) Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. J Clin Invest 97: 1960–1968

    Article  CAS  Google Scholar 

  12. Elkjær M-L et al. (2002) Altered expression of renal NHE3, TSC, BSC-1, and ENaC subunits in potassium-depleted rats. Am J Physiol Renal Physiol 283: F1376–F1388

    Article  Google Scholar 

  13. Bedford JJ et al. (2003) Aquaporin expression in normal human kidney and in renal disease. J Am Soc Nephrol 14: 2581–2587

    Article  CAS  Google Scholar 

  14. Reynolds ES et al. (1963) The renal lesion related to amphotericin B treatment for coccidioidomycosis. Med Clin North Am 47: 1149–1154

    Article  CAS  Google Scholar 

  15. Carlotti AP et al. (2001) Tonicity balance, and not electrolyte-free water calculations, more accurately guides therapy for acute changes in natremia. Intensive Care Med 27: 921–924

    Article  CAS  Google Scholar 

  16. Minemura K et al. (2001) IgA-kappa type multiple myeloma affecting proximal and distal renal tubules. Intern Med 40: 931–935

    Article  CAS  Google Scholar 

  17. Spruce BA et al. (1984) Idiopathic hypergammaglobulinaemia associated with nephrogenic diabetes insipidus and distal renal tubular acidosis. Postgrad Med J 60: 493–494

    Article  CAS  Google Scholar 

  18. Folami AO et al. (1978) Coexistence of distal renal tubular acidosis and nephrogenic diabetes insipidus in two patients: implications for the pathogenesis of distal renal tubular acidosis. Clin Invest Med 1: 105–109

    CAS  PubMed  Google Scholar 

  19. Nagayama Y et al. (1994) Acquired nephrogenic diabetes insipidus secondary to distal renal tubular acidosis and nephrocalcinosis associated with Sjögren's syndrome. J Endocrinol Invest 17: 659–663

    Article  CAS  Google Scholar 

  20. Vigeral P et al. (1987) Nephrogenic diabetes insipidus and distal tubular acidosis in methicillin-induced interstitial nephritis. Adv Exp Med Biol 212: 129–134

    Article  CAS  Google Scholar 

  21. Navarro JF et al. (1996) Nephrogenic diabetes insipidus and renal tubular acidosis secondary to foscarnet therapy. Am J Kidney Dis 27: 431–434

    Article  CAS  Google Scholar 

  22. Negro A et al. (1998) Ifosfamide-induced renal Fanconi syndrome with associated nephrogenic diabetes insipidus in an adult patient. Nephrol Dial Transplant 13: 1547–1549

    Article  CAS  Google Scholar 

  23. Karras A et al. (2003) Tenofovir-related nephrotoxicity in human immunodeficiency virus-infected patients: three cases of renal failure, Fanconi syndrome, and nephrogenic diabetes insipidus. Clin Infect Dis 36: 1070–1073

    Article  Google Scholar 

  24. Rollot F et al. (2003) Tenofovir-related Fanconi syndrome with nephrogenic diabetes insipidus in a patient with acquired immunodeficiency syndrome: the role of lopinavir-ritonavir-didanosine. Clin Infect Dis 37: e174–e176

    Article  Google Scholar 

Download references

Acknowledgements

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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Authors and Affiliations

  1. EJ Hoorn is an Internal Medicine Resident and a Nephrology Research Fellow, and R Zietse is a Consultant Nephrologist and Program Director of Nephrology, at the Erasmus Medical Center, Rotterdam, The Netherlands.,

    Ewout J Hoorn & Robert Zietse

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  1. Ewout J Hoorn
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  2. Robert Zietse
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Correspondence to Ewout J Hoorn.

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The authors declare no competing financial interests.

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Hoorn, E., Zietse, R. Combined renal tubular acidosis and diabetes insipidus in hematological disease. Nat Rev Nephrol 3, 171–175 (2007). https://doi.org/10.1038/ncpneph0424

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