Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe

Louise Winding; Maria Loane; Diana Wellesley; Marie-Claude Addor; Larraitz Arriola; Marian K. Bakker; Fabrizio Bianchi; Elisa Calzolari; Miriam Gatt; Martin Haeusler; Nathalie Lelong; Carmel Mullaney; Gioacchino Scarano; David Tucker; Awi Wiesel; Ester Garne

doi:10.1002/pd.4433

Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe

Louise Winding, Maria Loane, Diana Wellesley, Marie-Claude Addor, Larraitz Arriola, Marian K. Bakker, Fabrizio Bianchi, Elisa Calzolari, Miriam Gatt, Martin Haeusler, Nathalie Lelong, Carmel Mullaney, Gioacchino Scarano, David Tucker, Awi Wiesel, Ester Garne^*

^*Corresponding author for this work

Reproductive Origins of Adult Health and Disease (ROAHD)

Research output: Contribution to journal › Article › Academic › peer-review

25 Citations (Scopus)

Abstract

ObjectivesThe aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).

MethodsThe 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 1458552 births.

ResultsThere were 601 MCKD cases giving an overall prevalence of 4.12 per 10000 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

ConclusionsCases 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. (c) 2014 John Wiley & Sons, Ltd.

Original language	English
Pages (from-to)	1093-1098
Number of pages	6
Journal	Prenatal Diagnosis
Volume	34
Issue number	11
DOIs	https://doi.org/10.1002/pd.4433
Publication status	Published - Nov-2014

Keywords

URINARY-TRACT ABNORMALITIES
MANAGEMENT
DISEASE
MALFORMATIONS
ANOMALIES
CHILDREN

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Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe
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@article{a9749201732e414c8d03a6108b4e0949,

title = "Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe",

abstract = "ObjectivesThe aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).MethodsThe 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 1458552 births.ResultsThere were 601 MCKD cases giving an overall prevalence of 4.12 per 10000 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 (pConclusionsCases 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. (c) 2014 John Wiley & Sons, Ltd.",

keywords = "URINARY-TRACT ABNORMALITIES, MANAGEMENT, DISEASE, MALFORMATIONS, ANOMALIES, CHILDREN",

author = "Louise Winding and Maria Loane and Diana Wellesley and Marie-Claude Addor and Larraitz Arriola and Bakker, {Marian K.} and Fabrizio Bianchi and Elisa Calzolari and Miriam Gatt and Martin Haeusler and Nathalie Lelong and Carmel Mullaney and Gioacchino Scarano and David Tucker and Awi Wiesel and Ester Garne",

year = "2014",

month = nov,

doi = "10.1002/pd.4433",

language = "English",

volume = "34",

pages = "1093--1098",

journal = "Prenatal Diagnosis",

issn = "0197-3851",

publisher = "Wiley-Blackwell",

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TY - JOUR

T1 - Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe

AU - Winding, Louise

AU - Loane, Maria

AU - Wellesley, Diana

AU - Addor, Marie-Claude

AU - Arriola, Larraitz

AU - Bakker, Marian K.

AU - Bianchi, Fabrizio

AU - Calzolari, Elisa

AU - Gatt, Miriam

AU - Haeusler, Martin

AU - Lelong, Nathalie

AU - Mullaney, Carmel

AU - Scarano, Gioacchino

AU - Tucker, David

AU - Wiesel, Awi

AU - Garne, Ester

PY - 2014/11

Y1 - 2014/11

N2 - ObjectivesThe aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).MethodsThe 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 1458552 births.ResultsThere were 601 MCKD cases giving an overall prevalence of 4.12 per 10000 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 (pConclusionsCases 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. (c) 2014 John Wiley & Sons, Ltd.

AB - ObjectivesThe aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).MethodsThe 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 1458552 births.ResultsThere were 601 MCKD cases giving an overall prevalence of 4.12 per 10000 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 (pConclusionsCases 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. (c) 2014 John Wiley & Sons, Ltd.

KW - URINARY-TRACT ABNORMALITIES

KW - MANAGEMENT

KW - DISEASE

KW - MALFORMATIONS

KW - ANOMALIES

KW - CHILDREN

U2 - 10.1002/pd.4433

DO - 10.1002/pd.4433

M3 - Article

SN - 0197-3851

VL - 34

SP - 1093

EP - 1098

JO - Prenatal Diagnosis

JF - Prenatal Diagnosis

IS - 11

ER -

Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe

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