Pediatric Medullary Sponge Kidney Follow-up

Author: Howard Trachtman, MD; Chief Editor: Craig B Langman, MD more...

Updated: Oct 27, 2010

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Further Inpatient Care
Further Outpatient Care
Inpatient & Outpatient Medications
Complications
Prognosis
Patient Education
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Further Inpatient Care

Further inpatient care is needed only for patients with medullary sponge kidney (MSK) who have renal stones or a severe urinary tract infection (UTI).

Serial screening for malignancies is required in patients with medullary sponge kidney and hemihypertrophy.

Further Outpatient Care

Periodic screening for hematuria, bacteriuria, and kidney stones is indicated.

Inpatient & Outpatient Medications

Medications are needed only for patients with UTIs or kidney stones.

Complications

Complications include the following:

Gross hematuria
Renal stones
UTIs

Prognosis

Medullary sponge kidney is a nonprogressive disease and has no adverse impact on renal or patient survival.

Patient Education

Inform the patient of possible complications.

For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Blood in the Urine.

Contributor Information and Disclosures

Author

Howard Trachtman, MD Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Uri S Alon, MD Director of Bone and Mineral Disorders Clinic and Renal Research Laboratory, Children's Mercy Hospital of Kansas City; Professor, Department of Pediatrics, Division of Pediatric Nephrology, University of Missouri-Kansas City School of Medicine

Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Frederick J Kaskel, MD, PhD Director of the Division and Training Program in Pediatric Nephrology, Vice Chair, Department of Pediatrics, Montefiore Medical Center and Albert Einstein School of Medicine

Frederick J Kaskel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Eastern Society for Pediatric Research, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Renal Physicians Association, Sigma Xi, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

References

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. Feb 2006;69(4):663-70. [Medline].
Carboni I, Andreucci E, Caruso MR, et al. Medullary sponge kidney associated with primary distal renal tubular acidosis and mutations of the H+-ATPase genes. Nephrol Dial Transplant. Sep 2009;24(9):2734-8. [Medline].
Torregrossa R, Anglani F, Fabris A, et al. Identification of GDNF gene sequence variations in patients with medullary sponge kidney disease. Clin J Am Soc Nephrol. Jul 2010;5(7):1205-10. [Medline].
Harris AM, Hall B, Kriss VM, Fowlkes JL, Kiessling SG. Rabson-Mendenhall syndrome: medullary sponge kidney, a new component. Pediatr Nephrol. Dec 2007;22(12):2141-4. [Medline].
Fabris A, Bernich P, Abaterusso C, Marchionna N, Canciani C, Nouvenne A. Bone disease in medullary sponge kidney and effect of potassium citrate treatment. Clin J Am Soc Nephrol. Dec 2009;4(12):1974-9. [Medline].
Fabris A, Lupo A, Bernich P, et al. Long-term treatment with potassium citrate and renal stones in medullary sponge kidney. Clin J Am Soc Nephrol. Sep 2010;5(9):1663-8. [Medline].
Abeshouse BS, Abeshouse GA. Sponge kidney: a review of the litrature and a report of five cases. J Urol. Aug 1960;84:252-67. [Medline].
Avner ED. Medullary sponge kidney. In: Greenber A, Cheung AK, Coffman TM, et al, eds. NKF Primer on Kidney Disease. 1997.
Osther PJ, Mathiasen H, Hansen AB, et al. Urinary acidification and urinary excretion of calcium and citrate in women with bilateral medullary sponge kidney. Urol Int. 1994;52(3):126-30. [Medline].
Patriquin HB, O'Regan S. Medullary sponge kidney in childhood. AJR Am J Roentgenol. Aug 1985;145(2):315-9. [Medline].
[Guideline] Tiselius HG, Alken P, Buck C, Gallucci M, Knoll T, Sarica K, Turk C. Guidelines on urolithiasis. Arnhem, The Netherlands: European Association of Urology (EAU); 2008 Mar. [Full Text].

Unenhanced coronal volume-rendered (VR) CT image of the kidneys demonstrates 2 small calculi in the mid portion of the right kidney and 2 small calculi in the lower pole of the left kidney (arrowheads). A large low-density lesion in the lower pole of the right kidney and a small low-density lesion in the upper pole of the left kidney (short arrows) were shown to represent benign simple renal cysts on the contrast enhanced CT images. Image courtesy of Dr. Terri J. Vrtiska, Consultant, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

CT image of both kidneys demonstrates brushlike densities throughout multiple papillae of both kidneys consistent with renal tubular ectasia. Correlation of the stone disease with the ectatic tubules is diagnostic of medullary sponge kidney. Image courtesy of Dr. Terri J. Vrtiska, Consultant, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

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