Literature DB >> 1642809

A syndrome of mental retardation, short stature, craniofacial anomalies with palpebral ptosis and pulmonary stenosis in three siblings with normal parents. An example of autosomal recessive inheritance of the Noonan phenotype?

C Maximilian1, D M Ioan, J P Fryns.   

Abstract

We present a family with four children in which three, a girl and two boys, present a similar MR/MCA syndrome with slight to moderate mental retardation, short stature, peculiar facies with palpebral ptosis, pectus excavatum and pulmonary stenosis. As both parents are mentally and physically normal, autosomal recessive inheritance of this Noonan-like phenotype is most likely. The findings in the present family confirm that the Noonan phenotype may be caused by different etiologies with different types of genetic transmission.

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Year:  1992        PMID: 1642809

Source DB:  PubMed          Journal:  Genet Couns        ISSN: 1015-8146


  3 in total

1.  Autosomal recessive Noonan syndrome associated with biallelic LZTR1 variants.

Authors:  Jennifer J Johnston; Jasper J van der Smagt; Jill A Rosenfeld; Alistair T Pagnamenta; Abdulrahman Alswaid; Eva H Baker; Edward Blair; Guntram Borck; Julia Brinkmann; William Craigen; Vu Chi Dung; Lisa Emrick; David B Everman; Koen L van Gassen; Suleyman Gulsuner; Margaret H Harr; Mahim Jain; Alma Kuechler; Kathleen A Leppig; Donna M McDonald-McGinn; Ngoc Thi Bich Can; Amir Peleg; Elizabeth R Roeder; R Curtis Rogers; Lena Sagi-Dain; Julie C Sapp; Alejandro A Schäffer; Denny Schanze; Helen Stewart; Jenny C Taylor; Nienke E Verbeek; Magdalena A Walkiewicz; Elaine H Zackai; Christiane Zweier; Martin Zenker; Brendan Lee; Leslie G Biesecker
Journal:  Genet Med       Date:  2018-02-22       Impact factor: 8.822

2.  Delineation of dominant and recessive forms of LZTR1-associated Noonan syndrome.

Authors:  Alistair T Pagnamenta; Pamela J Kaisaki; Fenella Bennett; Emma Burkitt-Wright; Hilary C Martin; Matteo P Ferla; John M Taylor; Lianne Gompertz; Nayana Lahiri; Katrina Tatton-Brown; Ruth Newbury-Ecob; Alex Henderson; Shelagh Joss; Astrid Weber; Jenny Carmichael; Peter D Turnpenny; Shane McKee; Francesca Forzano; Tazeen Ashraf; Kimberley Bradbury; Deborah Shears; Usha Kini; Anna de Burca; Edward Blair; Jenny C Taylor; Helen Stewart
Journal:  Clin Genet       Date:  2019-04-03       Impact factor: 4.438

3.  Noonan Syndrome in South Africa: Clinical and Molecular Profiles.

Authors:  Cedrik Tekendo-Ngongang; Gloudi Agenbag; Christian Domilongo Bope; Alina Izabela Esterhuizen; Ambroise Wonkam
Journal:  Front Genet       Date:  2019-04-16       Impact factor: 4.599

  3 in total

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