Medullary Sponge Kidney with Distal Renal Tubular Acidosis: A Case Report and Review of the Literature

Jamshidian M,Coombs Rj,Ratnam S,Malhotra D

doi:10.18875/2375-7043.5.204

Abstract

Medullary sponge kidney (MSK) is a rare disease, with a prevalence of 0.0002 – 0.0005%, characterized by cystic dilations of the collecting ducts. Patients with MSK have the propensity for recurrent formation of kidney stones, nephrocalcinosis and urinary tract infections (UTIs). Unfamiliar to many practitioners and commonly asymptomatic, MSK patients often remain undiagnosed.

Highlights

Medullary sponge kidney (MSK) is a rare disorder, usually congenital, defined by cystic dilations of the precalyceal collecting ducts within the medullary pyramids of the kidney [1,2,3]
The disease is usually discovered during evaluation of a urinary tract infection (UTI), renal colic or hematuria [4]
The pathophysiology of MSK is unknown, but it is associated with impaired acidification of the urine and most commonly distal renal tubular acidosis

Summary

Introduction

Medullary sponge kidney (MSK) is a rare disorder, usually congenital, defined by cystic dilations of the precalyceal collecting ducts within the medullary pyramids of the kidney [1,2,3]. We report a case of MSK in an adult patient, with no history of kidney stones or UTI, who presented with severe asymptomatic hypokalemia due distal renal tubular acidosis, nephrocalcinosis and a UTI. A previously healthy 37-year-old Caucasian female, with a past medical history of mild asthma and controlled epilepsy on levetiracetam, presented for hospital admission from her primary care facility due to hypokalemia with a potassium level of 2.5 mEq/L Her home medications included levetiracetam and montelucast. Review of systems was positive only for intermittent, non-radiating, substernal chest pain lasting less than 30 minutes She denied associated shortness of breath, nausea, vomiting or recent diarrhea. Her laboratory investigations revealed hypokalemia and hyperchloremic metabolic acidosis Due to reported chest pain, her EKG and cardiac enzymes were trended and deemed not indicative of cardiac ischemia

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