Literature DB >> 9788553

Bilaterally cleft lip, limb defects, and haematological manifestations: Roberts syndrome versus TAR syndrome.

M Urban1, C Opitz, C Bommer, H Enders, S Tinschert, R Witkowski.   

Abstract

We report on a 13-year-old patient followed since birth. He is the only offspring of young, non-consanguineous German parents. His mother has an isolated left cleft of lip and a cleft palate. At birth, our patient presented with bilaterally cleft lip/cleft palate, phocomelia of upper limbs with normal hands, and mild symmetrical deficiencies of the long bones of the lower limbs. Haematological evaluation demonstrated a leukaemoid reaction during a urinary tract infection as well as intermittent thrombocytopenia and episodes of marked eosinophilia during the first two years of life. Intellectual development has been normal. Comparison with two similar cases from the literature suggests a non-random phenotypic overlap of Roberts syndrome (MIM 268300) and TAR syndrome (MIM 274000). Such clinical constellations may be key observations to understand the genetic relationship of Roberts syndrome and TAR syndrome in future phenotype-genotype correlations.

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Mesh:

Year:  1998        PMID: 9788553     DOI: 10.1002/(sici)1096-8628(19980923)79:3<155::aid-ajmg1>3.0.co;2-m

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  4 in total

1.  Thrombocytopenia-absent radius syndrome: a clinical genetic study.

Authors:  K L Greenhalgh; R T Howell; A Bottani; P J Ancliff; H G Brunner; C C Verschuuren-Bemelmans; E Vernon; K W Brown; R A Newbury-Ecob
Journal:  J Med Genet       Date:  2002-12       Impact factor: 6.318

2.  Complex inheritance pattern resembling autosomal recessive inheritance involving a microdeletion in thrombocytopenia-absent radius syndrome.

Authors:  Eva Klopocki; Harald Schulze; Gabriele Strauss; Claus-Eric Ott; Judith Hall; Fabienne Trotier; Silke Fleischhauer; Lynn Greenhalgh; Ruth A Newbury-Ecob; Luitgard M Neumann; Rolf Habenicht; Rainer König; Eva Seemanova; André Megarbane; Hans-Hilger Ropers; Reinhard Ullmann; Denise Horn; Stefan Mundlos
Journal:  Am J Hum Genet       Date:  2006-12-21       Impact factor: 11.025

3.  Isolated lower limb phocomelia - a rare limb malformation.

Authors:  Priyanka Bansal; Akhil Bansal; Shitalmala Devi
Journal:  Iran J Pediatr       Date:  2012-09       Impact factor: 0.364

4.  Femoral-tibial-synostosis in a child with Roberts syndrome (Pseudothalidomide): a case report.

Authors:  Ali Al Kaissi; Robert Csepan; Klaus Klaushofer; Franz Grill
Journal:  Cases J       Date:  2008-08-18
  4 in total

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