Literature DB >> 19471182

A case of mesomelic dysplasia Kantaputra type--new findings and a new diagnostic approach.

Karolina Anna Siwicka1, Hiroshi Kitoh, Masaki Nishiyama, Naoki Ishiguro.   

Abstract

Mesomelic dysplasia is a heterogeneous group of rare bone diseases characterized by disproportionate shortness of middle segments of limbs and short stature. This study reports a male patient with an early diagnosed mesomelic dysplasia Kantaputra type (MIM*156232), characterized by symmetric, bilateral forearm and lower leg shortening with feet malformations and ankle-tarsal synostoses. As a result of several diagnostic biases in defining the mesomelic syndromes, we attempted to categorize symptoms and to clarify hitherto difficult discrimination between mesomeliae. Given that specific ankle joint alignment distortions seem to be pathognomonic for Kantaputra type (especially a fibulo-calcanear and, to a lesser extent, a tibio-talar synostosis), but not detectable with plane radiography, we encouraged use of MRI evaluation as an indispensable modality for proper diagnosis and further preoperative planning.

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Year:  2008        PMID: 19471182     DOI: 10.1097/BPB.0b013e32830cc3c8

Source DB:  PubMed          Journal:  J Pediatr Orthop B        ISSN: 1060-152X            Impact factor:   1.041


  1 in total

1.  Mesomelia-synostoses syndrome results from deletion of SULF1 and SLCO5A1 genes at 8q13.

Authors:  Bertrand Isidor; Olivier Pichon; Richard Redon; Debra Day-Salvatore; Antoine Hamel; Karolina A Siwicka; Maria Bitner-Glindzicz; Dominique Heymann; Lena Kjellén; Cornelia Kraus; Jules G Leroy; Geert R Mortier; Anita Rauch; Alain Verloes; Albert David; Cédric Le Caignec
Journal:  Am J Hum Genet       Date:  2010-06-17       Impact factor: 11.025

  1 in total

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