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{{Medullary sponge kidney}}
{{CMG}}; {{AE}} {{MKA}}
 
==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|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|von Hippel-Lindau disease]].
 
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para–clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
|-
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Flank/abdominal pain
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Polyuria
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hematuria
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hepatomegaly
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Bilateral flank masses
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hypertension
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Urinalysis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Ultrasound
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CT scan
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Medullary sponge kidney]]<ref name="pmid16395272">{{cite journal |vauthors=Gambaro G, Feltrin GP, Lupo A, Bonfante L, D'Angelo A, Antonello A |title=Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s |journal=Kidney Int. |volume=69 |issue=4 |pages=663–70 |date=February 2006 |pmid=16395272 |doi=10.1038/sj.ki.5000035 |url=}}</ref><ref name="pmid9275977">{{cite journal |vauthors=Levine E, Hartman DS, Meilstrup JW, Van Slyke MA, Edgar KA, Barth JC |title=Current concepts and controversies in imaging of renal cystic diseases |journal=Urol. Clin. North Am. |volume=24 |issue=3 |pages=523–43 |date=August 1997 |pmid=9275977 |doi= |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/–
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* [[Microalbuminuria]]
* [[Proteinuria]]
* [[Hematuria]]
| style="background: #F5F5F5; padding: 5px;" |
* Malformation of [[distal]] collecting [[tubules]]
* [[Nephrolithiasis]]
| style="background: #F5F5F5; padding: 5px;" |
* [[Nephrolithiasis]]
* [[Renal cortex]] is spared
* [[Medullary]] [[nephrocalcinosis]]
| style="background: #F5F5F5; padding: 5px;" |
* Segmental or diffused [[papillary]] malformation
* Enlarged [[medullary]] collecting [[ducts]]
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* [[Tubular acidosis]]
* Recurrent [[urinary tract infections]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Autosomal dominant polycystic kidney disease|Autosomal dominant polycystic kidney disease (ADPKD)]]<ref name="pmid8321262">{{cite journal |vauthors=Gabow PA |title=Autosomal dominant polycystic kidney disease |journal=N. Engl. J. Med. |volume=329 |issue=5 |pages=332–42 |date=July 1993 |pmid=8321262 |doi=10.1056/NEJM199307293290508 |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
* [[Microalbuminuria]]
* [[Proteinuria]]
* [[Hypocitraturia]]
* [[Hyperoxaluria]]
* Low [[urine]] [[pH]]
* [[Hematuria]]
| style="background: #F5F5F5; padding: 5px;" |
* [[Bilateral]] [[fluid]] filled [[cysts]]
* [[Ultrasonography]] of parents will show [[cysts]]
| style="background: #F5F5F5; padding: 5px;" |
* Numerous [[renal cysts]]
* Scant intervening [[parenchyma]] with water [[attenuation]] and a very thin wall
* Multiple [[homogenous]] [[cystic]] lesions in the [[liver]] and [[pancreas]]
| style="background: #F5F5F5; padding: 5px;" |
* Segmental [[sclerosis]]
* [[Vascular]] [[sclerosis]]
* [[Interstitial]] [[fibrosis]]
* [[Tubular]] [[atrophy]]
| style="background: #F5F5F5; padding: 5px;" |[[Ultrasound]]
| style="background: #F5F5F5; padding: 5px;" |Extrarenal manifestations:
* [[Hepatic]] [[cysts]]
* [[Pancreatic]] [[cysts]]
 
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Autosomal recessive polycystic kidney disease|Autosomal recessive polycystic kidney disease (ARPKD)]]<ref name="pmid16523049">{{cite journal |vauthors=Adeva M, El-Youssef M, Rossetti S, Kamath PS, Kubly V, Consugar MB, Milliner DM, King BF, Torres VE, Harris PC |title=Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD) |journal=Medicine (Baltimore) |volume=85 |issue=1 |pages=1–21 |date=January 2006 |pmid=16523049 |doi=10.1097/01.md.0000200165.90373.9a |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
* [[Microalbuminuria]]
* [[Proteinuria]]
* [[Hypocitraturia]]
* [[Hyperoxaluria]]
* Low [[urine]] [[pH]]
* [[Hematuria]]
| style="background: #F5F5F5; padding: 5px;" |
* [[Bilateral]] [[fluid]] filled [[cysts]]
* [[Ultrasonography]] of parents will not show [[cysts]]
| style="background: #F5F5F5; padding: 5px;" |
* Numerous [[renal]] [[cysts]]
* Scant intervening [[parenchyma]] with water [[attenuation]] and very thin wall
| style="background: #F5F5F5; padding: 5px;" |
* Segmental [[sclerosis]]
* [[Vascular]] [[sclerosis]]
* [[Interstitial]] [[fibrosis]]
* [[Tubular]] [[atrophy]]
| style="background: #F5F5F5; padding: 5px;" |[[Ultrasound]]
| style="background: #F5F5F5; padding: 5px;" |Extrarenal manifestations:
* [[Portal]] [[fibrosis]]
* [[Portal]] [[hypertension]]
* [[Ascending cholangitis]]
* [[Biliary]] [[dysgenesis]]
* [[Pulmonary]] [[hypoplasia]] in [[neonates]]
 
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Simple [[renal cyst]]<ref name="pmid8250026">{{cite journal |vauthors=Ravine D, Gibson RN, Donlan J, Sheffield LJ |title=An ultrasound renal cyst prevalence survey: specificity data for inherited renal cystic diseases |journal=Am. J. Kidney Dis. |volume=22 |issue=6 |pages=803–7 |date=December 1993 |pmid=8250026 |doi= |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* Normal sized [[kidneys]]
* Smooth contour
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* Thin walled cortical [[cysts]]
* Filled with clear yellow [[fluid]]
* [[Cuboidal]], flattened or [[atrophic]] [[epithelium]]
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* Negative family history of renal [[cystic]] disease
* Age ≥ 30 yrs
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Tuberous sclerosis complex]]<ref name="pmid18772274">{{cite journal |vauthors=Umeoka S, Koyama T, Miki Y, Akai M, Tsutsui K, Togashi K |title=Pictorial review of tuberous sclerosis in various organs |journal=Radiographics |volume=28 |issue=7 |pages=e32 |date=2008 |pmid=18772274 |doi=10.1148/rg.e32 |url=}}</ref><ref name="pmid25905927">{{cite journal |vauthors=Manoukian SB, Kowal DJ |title=Comprehensive imaging manifestations of tuberous sclerosis |journal=AJR Am J Roentgenol |volume=204 |issue=5 |pages=933–43 |date=May 2015 |pmid=25905927 |doi=10.2214/AJR.13.12235 |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | +/–
| style="background: #F5F5F5; padding: 5px;" | +/–
| style="background: #F5F5F5; padding: 5px;" | +/–
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* [[Renal cysts]]
* [[Renal]] angiomyolipomas
| style="background: #F5F5F5; padding: 5px;" |
* CT [[kidneys]] will differentiate [[cystic]] and [[solid]] lesions, as well as [[renal]] angiomyolipomas
* CT [[brain]] shows:
* Cortical hemartomas
* White matter lesions
| style="background: #F5F5F5; padding: 5px;" |
* [[Epithelioid]] tissue
* [[Epithelial]] [[cysts]]
* [[Angiomyolipoma]]
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* [[Seizures]]
* Mental [[retardation]]
* [[Angiofibroma]] (face)
* Shagreen patch
 
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Von Hippel-Lindau disease|Von Hippel-Lindau disease]]<ref name="pmid2274658">{{cite journal |vauthors=Maher ER, Yates JR, Harries R, Benjamin C, Harris R, Moore AT, Ferguson-Smith MA |title=Clinical features and natural history of von Hippel-Lindau disease |journal=Q. J. Med. |volume=77 |issue=283 |pages=1151–63 |date=November 1990 |pmid=2274658 |doi= |url=}}</ref><ref name="pmid12814730">{{cite journal |vauthors=Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH |title=von Hippel-Lindau disease |journal=Lancet |volume=361 |issue=9374 |pages=2059–67 |date=June 2003 |pmid=12814730 |doi=10.1016/S0140-6736(03)13643-4 |url=}}</ref>'''
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/–
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" | –
| style="background: #F5F5F5; padding: 5px;" |
* [[Renal cysts]]
 
| style="background: #F5F5F5; padding: 5px;" |
* Differentiates simple from complex [[cysts]]
| style="background: #F5F5F5; padding: 5px;" |
* [[Clear cell]] abnormalities
* Simple [[cysts]]
* [[Malignant]] [[cysts]]
| style="background: #F5F5F5; padding: 5px;" | [[CT scan]]
| style="background: #F5F5F5; padding: 5px;" |
* [[Retinal]] [[hemangioma]]
* [[Clear cell]] [[renal cell carcinoma]]
* [[Cerebellar]] and spinal [[hemangioblastoma]]
* [[Pheochromocytoma]]
* [[Pancreatic]] [[tumor]]
|}


{{Medullary sponge kidney}}
==References==
==References==
{{reflist|2}}
{{reflist|2}}
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Nephrology]]
[[Category:Nephrology]]


[[Category:Needs content]]
[[Category:Needs content]]

Latest revision as of 16:25, 30 July 2018

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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.

Diseases Clinical manifestations Para–clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Flank/abdominal pain Polyuria Hematuria Hepatomegaly Bilateral flank masses Hypertension Urinalysis Ultrasound CT scan
Medullary sponge kidney[1][2] + +/– + +
Autosomal dominant polycystic kidney disease (ADPKD)[3] + + + + + + Ultrasound Extrarenal manifestations:
Autosomal recessive polycystic kidney disease (ARPKD)[4] + + + + + + Ultrasound Extrarenal manifestations:
Simple renal cyst[5] +
  • Normal sized kidneys
  • Smooth contour
  • Negative family history of renal cystic disease
  • Age ≥ 30 yrs
Tuberous sclerosis complex[6][7] +/– +/– +/– +
  • CT kidneys will differentiate cystic and solid lesions, as well as renal angiomyolipomas
  • CT brain shows:
  • Cortical hemartomas
  • White matter lesions
Von Hippel-Lindau disease[8][9] + +/– + +
  • Differentiates simple from complex cysts
CT scan

References

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