Title: Sickle Cell Nephropathy Presenting as Type 4 Renal Tubular Acidosis and Hyperkalemia

Authors: Jahnabi Bhagawati, Sourya Acharya, Samarth Shukla, Abhijeet K. Agrawal

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i6.134

Abstract

Sickle cell nephropathy (SCN) is a well known clinical entity. Various pathophysiological mechanisms and risk factors alter the renal functions in sickle cell disease (SCD). The renal manifestations ranges from glomerular insults, vasculopathy, tubular dysfunctions and even malignancies like renal medullary carcinoma. We present a case of a 20 year male who presented to us with sickle cell crisis and severe hyperkalemia due to rare type 4 distal renal tubular acidosis (RTA).

Key Words: SCN, SCD, vasculopathy, glomerular, hyperkalemia, RTA.

References

  1.      Scheinman JI: Sickle cell nephropathy. In: Pediatric Nephrology,edited by Holliday M, Barratt TM, Avner ED, Baltimore, Williams & Wilkins. 1994; 908–919.

2.      Powars DR, Elliot-Mills DD, Chan L, Hiti AL, Opas LM, Johnson C. Chronic renal failure in sickle cell disease: Risk factors, clinical course, and mortality. Ann Intern Med 1991; 115: 614–620.

3.      Platt RS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP. Mortality in sickle cell disease. N Engl J Med 1994; 330: 1639–1644.

4.      Scheinman JI.  Sickle cell disease and the kidney. Nat Clin Pract Nephrol 2009; 5:78-88.

5.      Silva Junior GB, Libório AB, Daher EF: New insights on pathophysiology, clinical manifestations, diagnosis,and treatment of sickle cell nephropathy. Ann Hematol 2011;90:1371-1379.

6.      Sharpe CC, Thein SL: Sickle cell nephropathy – a practical approach. Br J Haematol 2011; 155:287-297.

7.      de Jong PE, Statius van Eps LW. Sickle cell nephropathy: new insights into its pathophysiology.  Kidney Int. 1985; 27:711–717.

8.      Allon M. Renal abnormalities in sickle cell disease. Arch. Intern. Med. 1990; 150:501–504.

9.      Maurel S, et al. Prevalence and correlates of metabolic acidosis among patients with homozygous sickle cell disease. Clin. J. Am. Soc. Nephrol. 2014; 9:648–653.  

10.  DeFronzo RA, Taufield PA, Black H, McPhedran P, Cooke CR. Impaired renal tubular potassium secretion in sickle cell disease. Ann. Intern. Med. 1979; 90:310–316.

11.  Batlle D, Itsarayoungyuen K, Arruda JA, Kurtzman NA. Hyperkalemic hyperchl-oremic metabolic acidosis in sickle cell hemoglobinopathies. Am. J. Med. 1982; 72:188–192. 

Corresponding Author

Jahnabi Bhagawati

Resident, Dept. of Medicine, DMIMS Univ.,

JN Medical College, Sawangi (M), Wardha- 442004 Maharashtra