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Last reviewed: 21 Apr 2025
Last updated: 05 Jul 2022

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors

Other diagnostic factors

  • previous nephrolithiasis
  • flank pain
  • haematuria
  • costovertebral angle tenderness
  • dysuria
  • urinary urgency
  • increased frequency of urination
  • groin pain

Risk factors

  • hemi-hypertrophy
  • Beckwith-Wiedemann's syndrome
  • Caroli's disease
  • congenital hepatic fibrosis
  • Ehlers-Danlos' syndrome
  • family history of polycystic kidney disease
  • chronic kidney disease
  • hypercalciuria
  • female sex
  • hyperparathyroidism

Diagnostic investigations

1st investigations to order

  • urinalysis
  • urine culture
  • urea and creatinine
  • serum electrolytes
  • abdominal radiography
  • renal ultrasound
  • intravenous urography
  • non-contrast CT scan
  • CT urogram

Investigations to consider

  • 24-hour urine monitoring
  • stone analysis

Treatment algorithm

Contributors

Authors

Michael J. Choi, MD

Associate Professor of Medicine

Clinical Director of Nephrology

Johns Hopkins University School of Medicine

Baltimore

MD

Disclosures

MJC declares that he has no competing interests.

David S. Goldfarb, MD

Clinical Chief

Division of Nephrology

New York University School of Medicine

New York VA Medical Center

New York

NY

Disclosures

DSG is an author of several references cited in this topic.

Acknowledgements

Dr Michael J. Choi and Dr David S. Goldfarb would like to gratefully acknowledge Dr Amaka Edeani, a previous contributor to this topic.

Disclosures

AE declares that she has no competing interests.

Peer reviewers

Khashayar Sakhaee, MD

Internal Medicine Physician

UT Southwestern Medical Center

Dallas

TX

Disclosures

KS declares that he has no competing interests.

Eric N. Taylor, MD, MSc

Assistant Professor of Medicine

Harvard Medical School

Renal Division

Brigham and Women's Hospital

Channing Laboratory

Boston

MA

Disclosures

ENT declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Forster JA, Taylor J, Browning AJ, et al. A review of the natural progression of medullary sponge kidney and a novel grading system based on intravenous urography findings. Urol Int. 2007;78(3):264-9. Abstract

Imam TH, Patail H, Patail H. Medullary sponge kidney: current perspectives. Int J Nephrol Renovasc Dis. 2019;12:213-8.Full text  Abstract

Gambaro G, Feltrin GP, Lupo A, et al. Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s. Kidney Int. 2006 Feb;69(4):663-70. Abstract

Maw AM, Megibow AJ, Grasso M, et al. Diagnosis of medullary sponge kidney by computed tomographic urography. Am J Kidney Dis. 2007 Jul;50(1):146-50. Abstract

Reference articles

A full list of sources referenced in this topic is available here.

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