Literature DB >> 11665997

3-M syndrome: description of six new patients with review of the literature.

G van der Wal1, B J Otten, H G Brunner, I van der Burgt.   

Abstract

3-M syndrome combines pre- and postnatal growth retardation and dysmorphic facial features with autosomal recessive inheritance. Six new patients with 3-M syndrome are described and compared with 28 cases from the literature. Our six patients have a growth pattern, which parallels that of Silver-Russell syndrome (SRS). Final height is ISD less in 3-M syndrome than in SRS. Growth hormone treatment significantly increased final height in two of our patients. 3-M syndrome can be differentiated from other types of dwarfism by clinical criteria and by the demonstration of characteristically slender long bones and foreshortened vertebral bodies. We propose that calculating the metacarpal and vertebral indices can be used to measure and document this important diagnostic feature. While the gonadal status of female patients with 3-M syndrome is completely normal, male patients have a gonadal dysfunction and sub- or infertility.

Entities:  

Mesh:

Year:  2001        PMID: 11665997     DOI: 10.1097/00019605-200110000-00002

Source DB:  PubMed          Journal:  Clin Dysmorphol        ISSN: 0962-8827            Impact factor:   0.816


  16 in total

1.  Clinical, molecular and histopathological features of short stature syndrome with novel CUL7 mutation in Yakuts: new population isolate in Asia.

Authors:  N Maksimova; K Hara; A Miyashia; I Nikolaeva; A Shiga; A Nogovicina; A Sukhomyasova; V Argunov; A Shvedova; T Ikeuchi; M Nishizawa; R Kuwano; O Onodera
Journal:  J Med Genet       Date:  2007-08-03       Impact factor: 6.318

2.  A large-scale mutation search reveals genetic heterogeneity in 3M syndrome.

Authors:  Céline Huber; Anee-Lise Delezoide; Fabien Guimiot; Clarisse Baumann; Valérie Malan; Martine Le Merrer; Daniela Bezerra Da Silva; Dominique Bonneau; Pierre Chatelain; Carol Chu; Robin Clark; Helen Cox; Patrick Edery; Thomas Edouard; Virginia Fano; Kate Gibson; Gabriele Gillessen-Kaesbach; Maria-Luisa Giovannucci-Uzielli; Luitgard Margarete Graul-Neumann; Johana-Maria van Hagen; Liselot van Hest; Dafne Horovitz; Judith Melki; Carl-Joachim Partsch; Henry Plauchu; Anna Rajab; Massimiliano Rossi; David Sillence; Elisabeth Steichen-Gersdorf; Helen Stewart; Sheila Unger; Martin Zenker; Arnold Munnich; Valérie Cormier-Daire
Journal:  Eur J Hum Genet       Date:  2008-10-29       Impact factor: 4.246

3.  Clinical utility gene card for: 3-M syndrome - update 2013.

Authors:  Muriel Holder-Espinasse; Melita Irving; Valérie Cormier-Daire
Journal:  Eur J Hum Genet       Date:  2013-07-31       Impact factor: 4.246

4.  Clinical utility gene card for: 3M syndrome.

Authors:  Muriel Holder-Espinasse; Melita Irving; Valérie Cormier-Daire
Journal:  Eur J Hum Genet       Date:  2011-03-02       Impact factor: 4.246

5.  Whole exome sequencing reveals a novel mutation in CUL7 in a patient with an undiagnosed growth disorder.

Authors:  Andrew Dauber; Joan Stoler; Eliana Hechter; Jason Safer; Joel N Hirschhorn
Journal:  J Pediatr       Date:  2012-09-10       Impact factor: 4.406

6.  2D and 3D Ultrasonographic Evaluation of Fetal Midface Hypoplasia in Two Cases with 3-M Syndrome.

Authors:  A Vimercati; A Chincoli; A C de Gennaro; V DʼAddario; E Cicinelli
Journal:  Geburtshilfe Frauenheilkd       Date:  2016-07       Impact factor: 2.915

7.  The primordial growth disorder 3-M syndrome connects ubiquitination to the cytoskeletal adaptor OBSL1.

Authors:  Dan Hanson; Philip G Murray; Amit Sud; Samia A Temtamy; Mona Aglan; Andrea Superti-Furga; Sue E Holder; Jill Urquhart; Emma Hilton; Forbes D C Manson; Peter Scambler; Graeme C M Black; Peter E Clayton
Journal:  Am J Hum Genet       Date:  2009-05-28       Impact factor: 11.025

8.  Two Siblings with a Mutation in CCDC8 Presenting with Mild Short Stature: A Case of 3-M Syndrome.

Authors:  Lihong Liao; Hoong-Wei Gan; Vivian Hwa; Mehul Dattani; Andrew Dauber
Journal:  Horm Res Paediatr       Date:  2017-07-04       Impact factor: 2.852

9.  Exome sequencing identifies CCDC8 mutations in 3-M syndrome, suggesting that CCDC8 contributes in a pathway with CUL7 and OBSL1 to control human growth.

Authors:  Dan Hanson; Philip G Murray; James O'Sullivan; Jill Urquhart; Sarah Daly; Sanjeev S Bhaskar; Leslie G Biesecker; Mars Skae; Claire Smith; Trevor Cole; Jeremy Kirk; Kate Chandler; Helen Kingston; Dian Donnai; Peter E Clayton; Graeme C M Black
Journal:  Am J Hum Genet       Date:  2011-07-07       Impact factor: 11.025

10.  3-M syndrome associated with growth hormone deficiency: 18 year follow-up of a patient.

Authors:  Cristina Meazza; Ekkehard Lausch; Sara Pagani; Elena Bozzola; Valeria Calcaterra; Andrea Superti-Furga; Margherita Silengo; Mauro Bozzola
Journal:  Ital J Pediatr       Date:  2013-03-21       Impact factor: 2.638

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.