Markschwammniere

Last updated on: 12.03.2021

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DefinitionThis section has been translated automatically.

Medullary sponge kidney is a congenital malformation of the kidneys with:

  • malformation of the terminal collecting tubes in the medullary pyramids and papillae
  • Formation of medullary cells

The medullary sponge kidney affects 75% of both kidneys (Herold 2021).

ClassificationThis section has been translated automatically.

The extent of the changes in the kidney can be used to assess the risk of symptomatic nephrolithiasis:

  • Grade 1: In this case, 1 calyx is found unilaterally. The risk is 0.09 episodes / year.
  • Grade 4: More than 1 calyx is affected bilaterally. The risk is then 0.34 episodes / year (Kuhlmann 2015).

Occurrence/EpidemiologyThis section has been translated automatically.

The incidence of medullary sponge kidney is 1: 5,000 (Herold 2021), and that of asymptomatic is 1: 200 (Hegele 2015).

EtiopathogenesisThis section has been translated automatically.

This is a congenital disease. The mode of inheritance is unknown (Keller 2010). According to Hofmann (2005), less than 5% of families are affected. It is suspected that changes in the flow properties in the tubules promote stone formation (Kasper 2015).

ManifestationThis section has been translated automatically.

Complaints in medullary sponge kidney usually appear in the 4th or 5th decade of life in the form of recurrent urinary tract infections or nephrolithiasis with hematuria, colic, pyuria, etc. (Risler 2008).

Clinical featuresThis section has been translated automatically.

The diagnosis is often made as an incidental finding on i. v. urogram or CT. Symptoms may include:

  • recurrent nephrolithiasis
  • recurrent urinary tract infections
  • symptoms of chronic renal insufficiency (rare) (Hegele 2015)

DiagnosticsThis section has been translated automatically.

In the past, the diagnosis was made by i.v. urography. Nowadays, this is usually done by CT, although this shows a lower sensitivity (Kasper 2015).

ImagingThis section has been translated automatically.

Sonography:

  • Hyperechogenic papillae presentable (Hegele 2015).
  • Nephrolithiasis
  • Nephrocalcinosis (Herold 2021)
  • dorsal sonic effacement phenomenon may exist depending on the extent of calcifications
  • the medullary pyramids appear speckled and interspersed with brilliant echoes (also called "Christmas tree phenomenon") (Hofmann 2005)
  • Calcification in the tips of the papillae (Herold 2021)

CT:

  • radial radiation emanating from the calyxes
  • radiopaque stones grouped in affected calyxes
  • widened pyramids
  • cysts arranged like clusters in the area of the papillae (represent dilated collecting tubes) (Keller 2010)

i. v. pyelography: The ectatic collecting tubes are filled with contrast medium and the outflow is delayed. In advanced stages, calcifications occur along the collecting tubes (Hegele 2015). Nowadays, however, i. v. pyelography is rarely used for diagnosis. CT has taken its place (Kasper 2015).

LaboratoryThis section has been translated automatically.

Hypercalciuria (Herold 2021).

HistologyThis section has been translated automatically.

Histologically, papillary dilated collecting tubes are found in the area of the medullary pyramids, from which cysts with a diameter of approx. 1 mm - 8 mm originate. These cysts are connected to the draining urinary system and may contain blood, leucocytes, cell detritus etc.. Calcifications or nephrocalcinosis can often be detected (Risler 2008).

Differential diagnosisThis section has been translated automatically.

  • Medullary cystic disease (MCD) (Hofmann 2005)
  • distal tubular acidosis
  • primary hyperparathyroidism (Kuhlmann 2015)

Differential diagnosis of bilateral calcifications

  • Hyperparathyroidism
  • vitamin D intoxication
  • Sarcoidosis
  • Tuberculosis
  • multiple myeloma
  • Milk-alkali syndrome (Hegele 2015)

Complication(s)This section has been translated automatically.

Renal insufficiency due to nephrocalcinosis (Herold 2021)

Note(s)This section has been translated automatically.

In patients with nephrolithiasis, 3 % - 5 % have medullary sponge kidney, and in those with calcium stones the proportion rises to as much as 20 % (Kuhlmann 2015).

Medullary sponge kidney also occurs in patients with hemihypertrophy syndrome, in whom the risk of developing a Wilms tumor is increased. In this case, regular sonographic controls are necessary (Hofmann 2005).

LiteratureThis section has been translated automatically.

  1. Bartels H (2013) Spatial damage of the kidney in sonographic images: textbook and atlas. Springer Verlag 121
  2. Beetz R et al (2011) Pediatric urology in clinic and practice. Thieme Verlag 256
  3. Berwanger B et al. (2002) Alström syndrome: a differential diagnosis to Bardet- Biedl syndrome. A differential diagnosis to Bardet- Biedl- syndrome. Monatsschrift Kinderheilkunde (150) 58 - 61
  4. Braune K (2017) Characterization of ALMS1 (Alstrom syndrome 1) transcripts in Hodgkin lymphoma cells. Dissertation for the degree of Doctor of Medicine (Dr. med.) submitted to the Medical Faculty of Martin Luther University Halle-Wittenberg.
  5. Danne T et al. (2016) Obesity, diabetes and dyslipidemia in childhood. Walter de Gruyter Publishers 5
  6. Deeg K H et al (2014) Ultrasound diagnostics in pediatrics and pediatric surgery. Thieme Verlag 864 - 866
  7. Engelskirchen R et al. (2006) Asphyxiating thoracic dysplasia (Jeune syndrome): successful multi-stage correction. Monthly Journal of Pediatrics (12)
  8. Entezami M et al. (2002) Sonographic diagnosis of malformation: teaching atlas of fetal ultrasonography. Selected syndromes and associations. Thieme Publishers 245
  9. Forsythe E et al (2013) Bardet- Biedl- syndrome. Eur J Hum Genet 21, 8 - 13. https://doi.org/10.1038/ejhg.2012.115.
  10. Ganten D et al (2013) Monogenic inherited diseases 2: handbook of molecular medicine. Springer Verlag 291 - 292
  11. Gimpel C et al. (2019) Imaging diagnosis in children with renal cysts and cystic kidneys. Monthly Journal of Pediatrics (167) 530 - 538.
  12. Hegele A et al. (2015) Urology: intensive course for continuing education. Thieme Verlag 117 - 121, 124 - 129
  13. Heinrich M et al (2019) Pediatric surgery: basic knowledge and practice. W. Zuckschwerdt Publishers 192 - 193.
  14. Herold G et al (2021) Internal medicine. Herold Publishers 630 - 634
  15. Hofmann V et al. (2005) Ultrasound diagnostics in pediatrics and pediatric surgery. Georg Thieme Publishers 439, 453 - 454
  16. Kasper D L et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 1850 - 1856
  17. Kasper D L et al (2016) Harrison's internal medicine. Georg Thieme Publishers 2279 - 2285
  18. Keller C K et al (2010) Practice of nephrology. Springer Verlag 43 - 51, 53 - 54
  19. Kemper M et al. (2020) S2k-guideline renal cysts and cystic kidney disease: renal cysts and cystic kidney disease in children. AWMF Register No. 166 / 003
  20. Knaup K X et al (2019) Autosomal dominant tubulointerstitial kidney disease (ADTKD). The Nephrologist. DOIhttps://doi.org/10.1007/s11560-019-0318-y
  21. Korinthenberg R et al (2009) Neuropediatrics: evidence-based therapy. Urban and Fischer Publishers 19
  22. Kuhlmann U et al. (2015) Nephrology: pathophysiology - clinic - renal replacement procedures. Thieme Verlag 64, 653 - 665
  23. Lerchbaumer M H (2018) "The Bosniak classification of renal cysts in contrast-enhanced ultrasound (CEUS) comparative to computed tomography and magnetic resonance imaging" Inaugural dissertation for the degree of Doctor medicinae (Dr. med.) submitted to the Medical Faculty Charité - Universitätsmedizin Berlin.
  24. Manski D (2019) The urology textbook. Dirk Manski Publishers 231 - 236
  25. Michels G et al. (2010) Clinic manual internal medicine Springer Verlag 504 - 505.
  26. Risler T et al (2008) Specialist nephrology. Elsevier Urban and Fischer Publishers 372 - 373, 752 - 754
  27. Schmidt F et al. (2006) Rosiglitazone long-term therapy in patients with Alström- Hallgren syndrome. Diabetology and Metabolism 1 (3) 168 - 172.
  28. Sohn C et al (2013) Ultrasound in gynecology and obstetrics. Thieme Verlag 243 - 244

Last updated on: 12.03.2021