title: Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe.
abstract: OBJECTIVE
  The aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).
  
  
  METHODS
  The study is based on routinely collected data from a European database of major congenital anomalies including 13 registries with cases born in 1997-2006 and covering 1 458 552 births.
  
  
  RESULTS
  There were 601 MCKD cases giving an overall prevalence of 4.12 per 10 000 births with regional variation. In live births, 87% of cases had an isolated renal anomaly and 13% had associated major nonrenal anomalies (chromosomal, syndrome or other major anomalies). For the cases with isolated renal anomalies, 51/386 (11%) and 7/386 (2%) choose to terminate the pregnancy or resulted in an intrauterine fetal death, respectively. The prenatal detection rate was 88% in both unilateral and bilateral cases. Birth outcome differed with 92% of unilateral MCKD cases being liveborn compared with 33% of bilateral MCKD cases. For unilateral MCKD cases, 84% had an isolated renal anomaly compared with 51% of bilateral MCKD cases (p < 0.001).
  
  
  CONCLUSIONS
  Cases with unilateral MCKD are mainly liveborn, and only 16% have associated major malformations or a syndrome. Cases with bilateral MCKD are often associated with nonrenal major congenital anomalies or part of a syndrome, and only one third of bilateral MCKD cases in this study were liveborn. Prenatal detection rate of MCKD was high for both unilateral and bilateral cases. © 2014 John Wiley & Sons, Ltd.
authors.affiliation: Paediatric Department, Hospital Lillebaelt, Kolding, Denmark.
authors.forename: Louise
authors.initials: L
authors.lastname: Winding
authors.forename: Maria
authors.initials: M
authors.lastname: Loane
authors.forename: Diana
authors.initials: D
authors.lastname: Wellesley
authors.forename: Marie-Claude
authors.initials: MC
authors.lastname: Addor
authors.forename: Larraitz
authors.initials: L
authors.lastname: Arriola
authors.forename: Marian K
authors.initials: MK
authors.lastname: Bakker
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Bianchi
authors.forename: Elisa
authors.initials: E
authors.lastname: Calzolari
authors.forename: Miriam
authors.initials: M
authors.lastname: Gatt
authors.forename: Martin
authors.initials: M
authors.lastname: Haeusler
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lelong
authors.forename: Carmel
authors.initials: C
authors.lastname: Mullaney
authors.forename: Gioacchino
authors.initials: G
authors.lastname: Scarano
authors.forename: David
authors.initials: D
authors.lastname: Tucker
authors.forename: Awi
authors.initials: A
authors.lastname: Wiesel
authors.forename: Ester
authors.initials: E
authors.lastname: Garne
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D005060:Europe / Q000453:epidemiology; D005260:Female; D005313:Fetal Death; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D050498:Live Birth / Q000453:epidemiology; D008297:Male; D021782:Multicystic Dysplastic Kidney / Q000175:diagnosis* / Q000453:epidemiology*; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000706:statistics & numerical data; D015995:Prevalence; D012042:Registries / Q000706:statistics & numerical data; D050497:Stillbirth / Q000453:epidemiology