Literature DB >> 20633866

Mutations in the hepatocyte nuclear factor-1β (HNF1B) gene are common with combined uterine and renal malformations but are not found with isolated uterine malformations.

Richard A Oram1, Emma L Edghill, Jenny Blackman, Miles J O Taylor, Tracey Kay, Sarah E Flanagan, Ida Ismail-Pratt, Sarah M Creighton, Sian Ellard, Andrew T Hattersley, Coralie Bingham.   

Abstract

OBJECTIVE: Congenital uterine abnormalities are common and may be associated with developmental renal abnormalities. Mutations of the hepatocyte nuclear factor-1β (HNF1B) gene are associated with renal and uterine abnormalities. We aimed to study the role of HNF1B mutations in a cohort with congenital uterine abnormalities. STUDY
DESIGN: We tested 108 probands with uterine abnormalities for HNF1B mutations. We collected clinical information from patient records.
RESULTS: Nine of 108 women (8%) had a mutation or deletion in the HNF1B gene. Abnormal HNF1B was found in 18% of the 50 probands who had both uterine and renal abnormalities but in none of the 58 women with isolated uterine abnormalities.
CONCLUSION: Mutations of the HNF1B gene are found in women with both uterine and renal abnormalities but are rare in isolated uterine abnormalities. We suggest that HNF1B testing should be performed in patients with both renal and uterine abnormalities, but not in patients with isolated uterine abnormalities.
Copyright © 2010 Mosby, Inc. All rights reserved.

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Year:  2010        PMID: 20633866     DOI: 10.1016/j.ajog.2010.05.022

Source DB:  PubMed          Journal:  Am J Obstet Gynecol        ISSN: 0002-9378            Impact factor:   8.661


  22 in total

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Journal:  Ther Adv Endocrinol Metab       Date:  2010-10       Impact factor: 3.565

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4.  HNF1B and PAX2 mutations are a common cause of renal hypodysplasia in the CKiD cohort.

Authors:  Rosemary Thomas; Simone Sanna-Cherchi; Bradley A Warady; Susan L Furth; Frederick J Kaskel; Ali G Gharavi
Journal:  Pediatr Nephrol       Date:  2011-03-05       Impact factor: 3.714

Review 5.  HNF1B-associated clinical phenotypes: the kidney and beyond.

Authors:  Detlef Bockenhauer; Graciana Jaureguiberry
Journal:  Pediatr Nephrol       Date:  2015-07-08       Impact factor: 3.714

6.  Severe prenatal renal anomalies associated with mutations in HNF1B or PAX2 genes.

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Journal:  Clin J Am Soc Nephrol       Date:  2013-03-28       Impact factor: 8.237

7.  The spectrum of renal involvement in male patients with infertility related to excretory-system abnormalities: phenotypes, genotypes, and genetic counseling.

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Journal:  J Nephrol       Date:  2016-03-05       Impact factor: 3.902

8.  Mutations in GREB1L Cause Bilateral Kidney Agenesis in Humans and Mice.

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Journal:  Am J Hum Genet       Date:  2017-11-02       Impact factor: 11.025

9.  HNF1B nephropathy has a slow-progressive phenotype in childhood-with the exception of very early onset cases: results of the German Multicenter HNF1B Childhood Registry.

Authors:  Christine Okorn; Anne Goertz; Udo Vester; Bodo B Beck; Carsten Bergmann; Sandra Habbig; Jens König; Martin Konrad; Dominik Müller; Jun Oh; Nadina Ortiz-Brüchle; Ludwig Patzer; Raphael Schild; Tomas Seeman; Hagen Staude; Julia Thumfart; Burkhard Tönshoff; Ulrike Walden; Lutz Weber; Marcin Zaniew; Hildegard Zappel; Peter F Hoyer; Stefanie Weber
Journal:  Pediatr Nephrol       Date:  2019-01-21       Impact factor: 3.714

Review 10.  Monogenic diabetes and pregnancy.

Authors:  Rinki Murphy
Journal:  Obstet Med       Date:  2015-06-29
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