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Bilateral multicystic dysplastic kidney(MCRD)

MedGen UID:
333563
•Concept ID:
C1840451
•
Disease or Syndrome
Synonym: Multicystic renal dysplasia, bilateral
SNOMED CT: Bilateral multicystic renal dysplasia (717749002); Multicystic renal dysplasia, bilateral (717749002)
 
Monarch Initiative: MONDO:0019982
OMIM®: 143400
Orphanet: ORPHA97364

Definition

A rare lethal form of multicystic dysplastic kidney (MCDK), a congenital anomaly of the kidney and urinary tract (CAKUT), in which both kidneys are large, distended by non-communicating multiple cysts and non-functional. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBilateral multicystic dysplastic kidney

Professional guidelines

PubMed

Contemporary diagnosis and management of pelvi-ureteric junction obstruction.
Rai A, Hsieh A, Smith A
BJU Int 2022 Sep;130(3):285-290. Epub 2022 Feb 5 doi: 10.1111/bju.15689. PMID: 35044033
Diagnosis and Management of Renal Cystic Disease of the Newborn: Core Curriculum 2021.
Raina R, Chakraborty R, Sethi SK, Kumar D, Gibson K, Bergmann C
Am J Kidney Dis 2021 Jul;78(1):125-141. Epub 2021 Jan 6 doi: 10.1053/j.ajkd.2020.10.021. PMID: 33418012
Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe.
Winding L, Loane M, Wellesley D, Addor MC, Arriola L, Bakker MK, Bianchi F, Calzolari E, Gatt M, Haeusler M, Lelong N, Mullaney C, Scarano G, Tucker D, Wiesel A, Garne E
Prenat Diagn 2014 Nov;34(11):1093-8. Epub 2014 Jul 2 doi: 10.1002/pd.4433. PMID: 24916896

Recent clinical studies

Etiology

Diagnostic accuracy of midtrimester antenatal ultrasound for multicystic dysplastic kidneys.
Scala C, McDonnell S, Murphy F, Leone Roberti Maggiore U, Khalil A, Bhide A, Thilaganathan B, Papageorghiou AT
Ultrasound Obstet Gynecol 2017 Oct;50(4):464-469. doi: 10.1002/uog.17305. PMID: 27643400
Magnetic resonance imaging diagnosis of severe fetal renal anomalies.
Hawkins JS, Dashe JS, Twickler DM
Am J Obstet Gynecol 2008 Mar;198(3):328.e1-5. Epub 2007 Nov 26 doi: 10.1016/j.ajog.2007.09.032. PMID: 18036507
Doppler waveforms from the pulmonary arterial system in normal fetuses and those with pulmonary hypoplasia.
Mitchell JM, Roberts AB, Lee A
Ultrasound Obstet Gynecol 1998 Mar;11(3):167-72. doi: 10.1046/j.1469-0705.1998.11030167.x. PMID: 9589138

Diagnosis

Diagnostic accuracy of midtrimester antenatal ultrasound for multicystic dysplastic kidneys.
Scala C, McDonnell S, Murphy F, Leone Roberti Maggiore U, Khalil A, Bhide A, Thilaganathan B, Papageorghiou AT
Ultrasound Obstet Gynecol 2017 Oct;50(4):464-469. doi: 10.1002/uog.17305. PMID: 27643400
Magnetic resonance imaging diagnosis of severe fetal renal anomalies.
Hawkins JS, Dashe JS, Twickler DM
Am J Obstet Gynecol 2008 Mar;198(3):328.e1-5. Epub 2007 Nov 26 doi: 10.1016/j.ajog.2007.09.032. PMID: 18036507
Doppler waveforms from the pulmonary arterial system in normal fetuses and those with pulmonary hypoplasia.
Mitchell JM, Roberts AB, Lee A
Ultrasound Obstet Gynecol 1998 Mar;11(3):167-72. doi: 10.1046/j.1469-0705.1998.11030167.x. PMID: 9589138

Prognosis

Diagnostic accuracy of midtrimester antenatal ultrasound for multicystic dysplastic kidneys.
Scala C, McDonnell S, Murphy F, Leone Roberti Maggiore U, Khalil A, Bhide A, Thilaganathan B, Papageorghiou AT
Ultrasound Obstet Gynecol 2017 Oct;50(4):464-469. doi: 10.1002/uog.17305. PMID: 27643400

Clinical prediction guides

Diagnostic accuracy of midtrimester antenatal ultrasound for multicystic dysplastic kidneys.
Scala C, McDonnell S, Murphy F, Leone Roberti Maggiore U, Khalil A, Bhide A, Thilaganathan B, Papageorghiou AT
Ultrasound Obstet Gynecol 2017 Oct;50(4):464-469. doi: 10.1002/uog.17305. PMID: 27643400

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