Medullary sponge kidney

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Cafer Zorkun M.D., PhD.

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Medullary sponge kidney is a congenital disorder of the kidneys characterized by a cystic dilatation of the collecting tubules in one or both kidneys.

References

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Historical Perspective

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Classification

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Pathophysiology

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Causes

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Differentiating Medullary sponge kidney from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

Medullary sponge kidney must be differentiated from other diseases that may cause renal cysts, such as Autosomal dominant and autosomal recessive polycystic kidney disease, simple renal cysts, tuberous sclerosis complex, von Hippel-Lindau disease.

Differentiating medullary sponge kidney from other Diseases

Medullary sponge kidney must be differentiated from other diseases that may cause renal cysts, such as Autosomal dominant and autosomal recessive polycystic kidney disease, simple renal cysts, tuberous sclerosis complex, von Hippel-Lindau disease.

	Symptoms			Physical examination			Lab Findings	Imaging		Histopathology
Diseases	Clinical manifestations						Para–clinical findings				Gold standard	Additional findings
	Symptoms			Physical examination			Para–clinical findings
	Flank/abdominal pain	Polyuria	Hematuria	Hepatomegaly	Bilateral flank masses	Hypertension	Urinalysis	Ultrasound	CT scan	Histopathology
Medullary sponge kidney^[1]^[2]	+	+/–	+	–	+	–	Microalbuminuria Proteinuria Hematuria	Malformation of distal collecting tubules Nephrolithiasis	Nephrolithiasis Renal cortex is spared Medullary nephrocalcinosis	Segmental or diffused papillary malformation Enlarged medullary collecting ducts	–	Tubular acidosis Recurrent urinary tract infections
Autosomal dominant polycystic kidney disease (ADPKD)^[3]	+	+	+	+	+	+	Microalbuminuria Proteinuria Hypocitraturia Hyperoxaluria Low urine pH Hematuria	Bilateral fluid filled cysts Ultrasonography of parents will show cysts	Numerous renal cysts Scant intervening parenchyma with water attenuation and a very thin wall Multiple homogenous cystic lesions in the liver and pancreas	Segmental sclerosis Vascular sclerosis Interstitial fibrosis Tubular atrophy	Ultrasound	Extrarenal manifestations: Hepatic cysts Pancreatic cysts
Autosomal recessive polycystic kidney disease (ARPKD)^[4]	+	+	+	+	+	+	Microalbuminuria Proteinuria Hypocitraturia Hyperoxaluria Low urine pH Hematuria	Bilateral fluid filled cysts Ultrasonography of parents will not show cysts	Numerous renal cysts Scant intervening parenchyma with water attenuation and very thin wall	Segmental sclerosis Vascular sclerosis Interstitial fibrosis Tubular atrophy	Ultrasound	Extrarenal manifestations: Portal fibrosis Portal hypertension Ascending cholangitis Biliary dysgenesis Pulmonary hypoplasia in neonates
Simple renal cyst^[5]	–	–	–	–	+	–	–	Normal sized kidneys Smooth contour	–	Thin walled cortical cysts Filled with clear yellow fluid Cuboidal, flattened or atrophic epithelium	–	Negative family history of renal cystic disease Age ≥ 30 yrs
Tuberous sclerosis complex^[6]^[7]	+/–	+/–	+/–	–	+	–	–	Renal cysts Renal angiomyolipomas	CT kidneys will differentiate cystic and solid lesions, as well as renal angiomyolipomas CT brain shows: Cortical hemartomas White matter lesions	Epithelioid tissue Epithelial cysts Angiomyolipoma	–	Seizures Mental retardation Angiofibroma (face) Shagreen patch
Von Hippel-Lindau disease^[8]^[9]	+	+/–	+	–	+	–	–	Renal cysts	Differentiates simple from complex cysts	Clear cell abnormalities Simple cysts Malignant cysts	CT scan	Retinal hemangioma Clear cell renal cell carcinoma Cerebellar and spinal hemangioblastoma Pheochromocytoma Pancreatic tumor

References

↑ Gambaro G, Feltrin GP, Lupo A, Bonfante L, D’Angelo A, Antonello A (February 2006). “Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s”. Kidney Int. 69 (4): 663–70. doi:10.1038/sj.ki.5000035. PMID 16395272.
↑ Levine E, Hartman DS, Meilstrup JW, Van Slyke MA, Edgar KA, Barth JC (August 1997). “Current concepts and controversies in imaging of renal cystic diseases”. Urol. Clin. North Am. 24 (3): 523–43. PMID 9275977.
↑ Gabow PA (July 1993). “Autosomal dominant polycystic kidney disease”. N. Engl. J. Med. 329 (5): 332–42. doi:10.1056/NEJM199307293290508. PMID 8321262.
↑ Adeva M, El-Youssef M, Rossetti S, Kamath PS, Kubly V, Consugar MB, Milliner DM, King BF, Torres VE, Harris PC (January 2006). “Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD)”. Medicine (Baltimore). 85 (1): 1–21. doi:10.1097/01.md.0000200165.90373.9a. PMID 16523049.
↑ Ravine D, Gibson RN, Donlan J, Sheffield LJ (December 1993). “An ultrasound renal cyst prevalence survey: specificity data for inherited renal cystic diseases”. Am. J. Kidney Dis. 22 (6): 803–7. PMID 8250026.
↑ Umeoka S, Koyama T, Miki Y, Akai M, Tsutsui K, Togashi K (2008). “Pictorial review of tuberous sclerosis in various organs”. Radiographics. 28 (7): e32. doi:10.1148/rg.e32. PMID 18772274.
↑ Manoukian SB, Kowal DJ (May 2015). “Comprehensive imaging manifestations of tuberous sclerosis”. AJR Am J Roentgenol. 204 (5): 933–43. doi:10.2214/AJR.13.12235. PMID 25905927.
↑ Maher ER, Yates JR, Harries R, Benjamin C, Harris R, Moore AT, Ferguson-Smith MA (November 1990). “Clinical features and natural history of von Hippel-Lindau disease”. Q. J. Med. 77 (283): 1151–63. PMID 2274658.
↑ Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH (June 2003). “von Hippel-Lindau disease”. Lancet. 361 (9374): 2059–67. doi:10.1016/S0140-6736(03)13643-4. PMID 12814730.

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Epidemiology and Demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Epidemiolgoy and Demographics

It has been estimated to occur with a prevalence of between 1 in every 5,000-10,000 individuals in a population.

References

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Risk Factors

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Screening

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Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Natural History,Complications and Prognosis

Complications such as infection, hematuria, and nephrolithiasis may be the presenting complaint in approximately 10% of patients.

References

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Diagnosis

Treatment

Medical Therapy | Surgery | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Template:Nephrology Template:WH Template:WikiDoc Sources

[pmid16395272-1] Gambaro G, Feltrin GP, Lupo A, Bonfante L, D’Angelo A, Antonello A (February 2006). “Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s”. Kidney Int. 69 (4): 663–70. doi:10.1038/sj.ki.5000035. PMID 16395272.

[pmid9275977-2] Levine E, Hartman DS, Meilstrup JW, Van Slyke MA, Edgar KA, Barth JC (August 1997). “Current concepts and controversies in imaging of renal cystic diseases”. Urol. Clin. North Am. 24 (3): 523–43. PMID 9275977.

[pmid8321262-3] Gabow PA (July 1993). “Autosomal dominant polycystic kidney disease”. N. Engl. J. Med. 329 (5): 332–42. doi:10.1056/NEJM199307293290508. PMID 8321262.

[pmid16523049-4] Adeva M, El-Youssef M, Rossetti S, Kamath PS, Kubly V, Consugar MB, Milliner DM, King BF, Torres VE, Harris PC (January 2006). “Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD)”. Medicine (Baltimore). 85 (1): 1–21. doi:10.1097/01.md.0000200165.90373.9a. PMID 16523049.

[pmid8250026-5] Ravine D, Gibson RN, Donlan J, Sheffield LJ (December 1993). “An ultrasound renal cyst prevalence survey: specificity data for inherited renal cystic diseases”. Am. J. Kidney Dis. 22 (6): 803–7. PMID 8250026.

[pmid18772274-6] Umeoka S, Koyama T, Miki Y, Akai M, Tsutsui K, Togashi K (2008). “Pictorial review of tuberous sclerosis in various organs”. Radiographics. 28 (7): e32. doi:10.1148/rg.e32. PMID 18772274.

[pmid25905927-7] Manoukian SB, Kowal DJ (May 2015). “Comprehensive imaging manifestations of tuberous sclerosis”. AJR Am J Roentgenol. 204 (5): 933–43. doi:10.2214/AJR.13.12235. PMID 25905927.

[pmid2274658-8] Maher ER, Yates JR, Harries R, Benjamin C, Harris R, Moore AT, Ferguson-Smith MA (November 1990). “Clinical features and natural history of von Hippel-Lindau disease”. Q. J. Med. 77 (283): 1151–63. PMID 2274658.

[pmid12814730-9] Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH (June 2003). “von Hippel-Lindau disease”. Lancet. 361 (9374): 2059–67. doi:10.1016/S0140-6736(03)13643-4. PMID 12814730.

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Medullary sponge kidney

Overview

References

References

References

References

References

Overview

Differentiating medullary sponge kidney from other Diseases

References

Overview

Epidemiolgoy and Demographics

References

References

References

Overview

Natural History,Complications and Prognosis

References

Diagnosis

Treatment

Case Studies

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