Literature DB >> 26333564

RBBP8 syndrome with microcephaly, intellectual disability, short stature and brachydactyly.

Sara Mumtaz1, Esra Yıldız2, Saliha Jabeen1, Amjad Khan1, Aslıhan Tolun2, Sajid Malik1.   

Abstract

Primary microcephaly is clinically variable and genetically heterogeneous. Four phenotypically distinct types of autosomal recessive microcephaly syndromes are due to different RBBP8 mutations. We report on a consanguineous Pakistani family with homozygous RBBP8 mutation c.1808_1809delTA (p.Ile603Lysfs*7) manifesting microcephaly and a distinct combination of skeletal, limb and ectodermal defects, mild intellectual disability, minor facial anomalies, anonychia, disproportionate short stature and brachydactyly, and additionally talipes in one patient.
© 2015 Wiley Periodicals, Inc.

Entities:  

Keywords:  Pakistani family; RBBP8; anonychia; consanguineous; inbreeding coefficient; intellectual disability; mental retardation; microcephaly; mutation; short stature

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Year:  2015        PMID: 26333564     DOI: 10.1002/ajmg.a.37299

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  2 in total

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Journal:  BMC Genomics       Date:  2021-05-08       Impact factor: 3.969

2.  Clinical relevance of systematic phenotyping and exome sequencing in patients with short stature.

Authors:  Nadine N Hauer; Bernt Popp; Eva Schoeller; Sarah Schuhmann; Karen E Heath; Alfonso Hisado-Oliva; Patricia Klinger; Cornelia Kraus; Udo Trautmann; Martin Zenker; Christiane Zweier; Antje Wiesener; Rami Abou Jamra; Erdmute Kunstmann; Dagmar Wieczorek; Steffen Uebe; Fulvia Ferrazzi; Christian Büttner; Arif B Ekici; Anita Rauch; Heinrich Sticht; Helmuth-Günther Dörr; André Reis; Christian T Thiel
Journal:  Genet Med       Date:  2017-10-12       Impact factor: 8.822

  2 in total

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