Literature DB >> 33210483

[Genetic analysis and clinical phenotype of a family with lymphedema-distichiasis syndrome].

Gang Hu1,2, Bei Liu1, Min Chen1, Yeqing Qian1, Minyue Dong1.   

Abstract

OBJECTIVE: To identify the genetic causes of a family with lymphedema-distichiasis syndrome (LDS).
METHODS: The whole exome sequencing was performed in a aborted fetus as the proband, and a candidate gene was identified. Peripheral blood of 8 family members were collected. Genotypic-phenotypic analysis were carried out through PCR amplification and Sanger sequencing.
RESULTS: The proband, and the mother, grandmother, uncle, granduncle of the proband all had distichiasis or varix of lower limb carried a FOXC2:c.595dupC frame shift mutation, and other subjects without any significant phenotypes did not present the mutation.
CONCLUSIONS: The FOXC2:c.595dupC frame shift mutation is the genetic cause of this family, which can lead to autosomal dominantly LDS, presenting nuchal translucency thickening and hydrops fetal during pregnancy, and the prognosis is usually good.

Entities:  

Keywords:  Autosomal dominant; Genetic diseases, inborn; Hydrops fetalis; Lymphedema-distichiasis syndrome; Nuchal translucency; Whole exome sequencing

Mesh:

Substances:

Year:  2020        PMID: 33210483      PMCID: PMC8800725          DOI: 10.3785/j.issn.1008-9292.2020.10.05

Source DB:  PubMed          Journal:  Zhejiang Da Xue Xue Bao Yi Xue Ban        ISSN: 1008-9292


  19 in total

1.  Re: outcome of pregnancy in chromosomally normal fetuses with increased nuchal translucency in the first trimester.

Authors:  S Yagel; T Imbar; D V Valsky
Journal:  Ultrasound Obstet Gynecol       Date:  2002-04       Impact factor: 7.299

2.  A case of lymphedema-distichiasis syndrome carrying a new de novo frameshift FOXC2 mutation.

Authors:  Antonella Fabretto; Alison Shardlow; Flavio Faletra; Loredana Lepore; Uros Hladnik; Paolo Gasparini
Journal:  Ophthalmic Genet       Date:  2010-06       Impact factor: 1.803

3.  Novel FOXC2 Mutation and Distichiasis in a Patient With Lymphedema-Distichiasis Syndrome.

Authors:  Matthew A De Niear; Mark P Breazzano; Louise A Mawn
Journal:  Ophthalmic Plast Reconstr Surg       Date:  2018 May/Jun       Impact factor: 1.746

4.  Mutations in FOXC2 are strongly associated with primary valve failure in veins of the lower limb.

Authors:  Russell H Mellor; Glen Brice; Anthony W B Stanton; Jane French; Alberto Smith; Steve Jeffery; J Rodney Levick; Kevin G Burnand; Peter S Mortimer
Journal:  Circulation       Date:  2007-03-19       Impact factor: 29.690

5.  c. 595-596 insC of FOXC2 underlies lymphedema, distichiasis, ptosis, ankyloglossia, and Robin sequence in a Thai patient.

Authors:  Pranoot Tanpaiboon; Piranit Kantaputra; Karn Wejathikul; Wirawit Piyamongkol
Journal:  Am J Med Genet A       Date:  2010-03       Impact factor: 2.802

6.  Analysis of lymphoedema-distichiasis families for FOXC2 mutations reveals small insertions and deletions throughout the gene.

Authors:  R Bell; G Brice; A H Child; V A Murday; S Mansour; C J Sandy; J R Collin; A F Brady; D F Callen; K Burnand; P Mortimer; S Jeffery
Journal:  Hum Genet       Date:  2001-06       Impact factor: 4.132

7.  Defective valves and abnormal mural cell recruitment underlie lymphatic vascular failure in lymphedema distichiasis.

Authors:  Tatiana V Petrova; Terhi Karpanen; Camilla Norrmén; Russell Mellor; Tomoki Tamakoshi; David Finegold; Robert Ferrell; Dontscho Kerjaschki; Peter Mortimer; Seppo Ylä-Herttuala; Naoyuki Miura; Kari Alitalo
Journal:  Nat Med       Date:  2004-08-22       Impact factor: 53.440

Review 8.  Novel FOXC2 missense mutation identified in patient with lymphedema-distichiasis syndrome and review.

Authors:  M T Dellinger; K Thome; M J Bernas; R P Erickson; M H Witte
Journal:  Lymphology       Date:  2008-09       Impact factor: 1.286

9.  Functional analysis of FLT4 mutations associated with Nonne-Milroy lymphedema.

Authors:  Valerie L R M Verstraeten; Wolfgang Holnthoner; Maurice A M van Steensel; Joep C J M Veraart; Reno S Bladergroen; Caroline A Heckman; Salla Keskitalo; Jorge Frank; Kari Alitalo; Michel van Geel; Peter M Steijlen
Journal:  J Invest Dermatol       Date:  2008-08-21       Impact factor: 8.551

Review 10.  Nuchal translucency and other first-trimester sonographic markers of chromosomal abnormalities.

Authors:  Kypros H Nicolaides
Journal:  Am J Obstet Gynecol       Date:  2004-07       Impact factor: 8.661

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