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Medullary Sponge Kidney: Current Perspectives

Authors Imam TH, Patail H, Patail H

Received 14 February 2019

Accepted for publication 17 September 2019

Published 26 September 2019 Volume 2019:12 Pages 213—218


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Pravin Singhal

Talha H Imam,1 Haris Patail,2 Hassan Patail3

1Division of Nephrology, Kaiser Permanente, Fontana, CA, USA; 2St. George’s University School of Medicine, West Indies, Grenada; 3Department of Medicine, Division of Pulmonary Critical Care and Sleep Medicine, Stony Brook University Hospital, Stony Brook, NY, USA

Correspondence: Talha H Imam
Division of Nephrology, Kaiser Permanente, 9985 Sierra Ave, Fontana, CA 92335, USA
Tel +1 909-427-7521

Abstract: Medullary Sponge Kidney (MSK) disease is a rare congenital malformation of the distal nephron where cystic dilatation is appreciable in the collecting ducts and renal papillae. Most cases of the malformation are thought to arise from a malfunction within neurotrophic factor and tyrosine kinase interactions. Presentation and prognosis are usually indolent; however, they include urinary tract infections (UTI), nephrolithiasis and nephrocalcinosis, distal renal tubular acidosis (dRTA) and hypocitraturia. With an insidious and asymptomatic onset, MSK is a difficult renal manifestation to both diagnose and treat. Difficulty diagnosing MSK today arises from clinical settings deviating from the usage of contrast methods when assessing the urogenital tract. Many healthcare standards for kidney disorders center diagnosis around imaging techniques rather than contrast methods. This ultimately leads to a decrease in the total number of confirmed cases of MSK. Though intra-venous urogram (IVU) remains as the current gold standard to diagnose MSK, other methods such as endoscopy and Multi-detector computed tomography (MDCT) are being put into place. Endoscopic examination and renal biopsy may allow definitive diagnosis; however, such invasive methods may be considered excessive. Moving forward, differential diagnoses for MSK can be made more precisely when patients present with other renal manifestations, especially in groups at risk. These groups include patients between the age of 20 and 30, patients with other renal malformations, high sodium diet patients, hyperparathyroid patients, and patients with family history of MSK. Basic treatment is aimed at controlling stone formation by stabilizing urinary pH. Treatment for patients, especially those prone to forming stones, includes the application of potassium citrate compounds, prophylactic water and diet control, surgical intervention or lithotripsy for removal of symptomatic kidney stones.

Keywords: medullary sponge kidney, nephron, nephrocalcinosis, renal stones

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