Literature DB >> 8026105

Cerebro-facio-articular syndrome of Van Maldergem: confirmation of a new MR/MCA syndrome.

G Zampino1, C Colosimo, F Balducci, P Mariotti, F Serra, G Scarano, P Mastroiacovo.   

Abstract

Van Maldergem et al. (1992) described a new syndrome in an 11-year-old girl, characterized by: mental retardation, hypotonia, dysmorphic facies with telecanthus, epicanthus, broad flattened nose, large inverted W-shaped mouth, malformed ears, finger camptodactyly, and joint hyperlaxity. In this report we present a 5-year-old girl with very similar clinical findings. We confirm the existence of this condition as an independent clinical entity, and we propose that, based on the major clinical manifestations, it should be defined as "cerebro-facio-articular" syndrome.

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Year:  1994        PMID: 8026105     DOI: 10.1111/j.1399-0004.1994.tb04011.x

Source DB:  PubMed          Journal:  Clin Genet        ISSN: 0009-9163            Impact factor:   4.438


  4 in total

1.  Van Maldergem syndrome: further characterisation and evidence for neuronal migration abnormalities and autosomal recessive inheritance.

Authors:  Sahar Mansour; Marielle Swinkels; Paulien A Terhal; Louise C Wilson; Philip Rich; Lionel Van Maldergem; Petra J G Zwijnenburg; Christine M Hall; Stephen P Robertson; Ruth Newbury-Ecob
Journal:  Eur J Hum Genet       Date:  2012-04-04       Impact factor: 4.246

2.  Whole-Exome Sequencing Reveals FAT4 Mutations in a Clinically Unrecognizable Patient with Syndromic CAKUT: A Case Report.

Authors:  Amelie T van der Ven; Shirlee Shril; Hadas Ityel; Asaf Vivante; Jing Chen; Daw-Yang Hwang; Kristen M Laricchia; Monkol Lek; Velibor Tasic; Friedhelm Hildebrandt
Journal:  Mol Syndromol       Date:  2017-07-01

3.  A patient with van Maldergem syndrome with endocrine abnormalities, hypogonadotropic hypogonadism, and breast aplasia/hypoplasia.

Authors:  Juan Sotos; Katherine Miller; Donald Corsmeier; Naomi Tokar; Benjamin Kelly; Vijay Nadella; Huachun Zhong; Amy Wetzel; Brent Adler; Chack-Yung Yu; Peter White
Journal:  Int J Pediatr Endocrinol       Date:  2017-10-13

4.  Requirement of FAT and DCHS protocadherins during hypothalamic-pituitary development.

Authors:  Emily J Lodge; Paraskevi Xekouki; Tatiane S Silva; Cristiane Kochi; Carlos A Longui; Fabio R Faucz; Alice Santambrogio; James L Mills; Nathan Pankratz; John Lane; Dominika Sosnowska; Tina Hodgson; Amanda L Patist; Philippa Francis-West; Francoise Helmbacher; Constantine Stratakis; Cynthia L Andoniadou
Journal:  JCI Insight       Date:  2020-10-27
  4 in total

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