Literature DB >> 23175007

A child with bone fractures and dysmorphic features: remember of pycnodysostosis and craniosynostosis.

Alberto Berenguer1, António Pedro Freitas, Gomes Ferreira, José Luis Nunes.   

Abstract

Accidental bony injuries are common in children. Children may also present with bony injuries following non-accidental injuries. Pathological fractures, though extremely rare, are an important entity and constitute fractures that occur in abnormal bones, usually after minor trauma. Pycnodysostosis is a rare skeletal dysplasia characterised by a clinical phenotype that includes short stature, skull deformities, osteosclerosis, acroosteolysis and bone fragility. Often the disease is diagnosed at an early age as a result of the investigation of short stature. However, the diagnosis is sometimes delayed and must be considered in any child with a history of recurrent or multiple bone fractures and dysmorphic features. The purpose of this report is to describe the clinical, radiological and genetic issues of a 9-year-old girl with a long history of multiple bone fractures. She had been subjected to safeguarding investigations previously and was identified to have dysmorphic features diagnosed as pycnodysostosis associated with craniosynostosis.

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Year:  2012        PMID: 23175007      PMCID: PMC4544943          DOI: 10.1136/bcr-2012-006930

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  21 in total

1.  High bone mineral density in pycnodysostotic patients with a novel mutation in the propeptide of cathepsin K.

Authors:  A F Schilling; C Mülhausen; W Lehmann; R Santer; T Schinke; J M Rueger; M Amling
Journal:  Osteoporos Int       Date:  2007-01-06       Impact factor: 4.507

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Review 3.  Pyknodysostosis--a report of two cases with a brief review of the literature.

Authors:  R J Bathi; V N Masur
Journal:  Int J Oral Maxillofac Surg       Date:  2000-12       Impact factor: 2.789

4.  Pycnodysostosis with extreme sleep apnea: a possible alternative to tracheotomy.

Authors:  Giacomo Della Marca; Emanuele Scarano; Chiara Leoni; Serena Dittoni; Anna Losurdo; Elisa Testani; Salvatore Colicchio; Valentina Gnoni; Catello Vollono; Giuseppe Zampino
Journal:  Sleep Breath       Date:  2011-01-14       Impact factor: 2.816

5.  Pycnodysostosis, a lysosomal disease caused by cathepsin K deficiency.

Authors:  B D Gelb; G P Shi; H A Chapman; R J Desnick
Journal:  Science       Date:  1996-08-30       Impact factor: 47.728

6.  Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.

Authors:  A T Soliman; A Rajab; I AlSalmi; A Darwish; M Asfour
Journal:  Arch Dis Child       Date:  1996-09       Impact factor: 3.791

7.  Molecular analysis and characterization of nine novel CTSK mutations in twelve patients affected by pycnodysostosis. Mutation in brief #961. Online.

Authors:  Michela Donnarumma; Stefano Regis; Barbara Tappino; Camillo Rosano; Stefania Assereto; Fabio Corsolini; Maja Di Rocco; Mirella Filocamo
Journal:  Hum Mutat       Date:  2007-05       Impact factor: 4.878

Review 8.  Pycnodysostosis. A report of 3 clinical cases.

Authors:  Daniela Alves Pereira; Leonardo Berini Aytés; Cosme Gay Escoda
Journal:  Med Oral Patol Oral Cir Bucal       Date:  2008-10-01

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Authors:  B D Gelb; J G Edelson; R J Desnick
Journal:  Nat Genet       Date:  1995-06       Impact factor: 38.330

10.  Craniosynostosis: A rare complication of pycnodysostosis.

Authors:  Sara Osimani; Isabelle Husson; Sandrine Passemard; Monique Elmaleh; Laurence Perrin; Chloé Quelin; Isabelle Marey; Olivier Delalande; Mirella Filocamo; Alain Verloes
Journal:  Eur J Med Genet       Date:  2010-01-05       Impact factor: 2.708

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  2 in total

1.  Genetic study of eight Egyptian patients with pycnodysostosis: identification of novel CTSK mutations and founder effect.

Authors:  G A Otaify; M S Abdel-Hamid; M I Mehrez; E Aboul-Ezz; M S Zaki; M S Aglan; S A Temtamy
Journal:  Osteoporos Int       Date:  2018-05-23       Impact factor: 4.507

2.  Cathepsin K analysis in a pycnodysostosis cohort: demographic, genotypic and phenotypic features.

Authors:  Ahmet Arman; Abdullah Bereket; Ajda Coker; Pelin Özlem Simşek Kiper; Tülay Güran; Behzat Ozkan; Zeynep Atay; Teoman Akçay; Belma Haliloglu; Koray Boduroglu; Yasemin Alanay; Serap Turan
Journal:  Orphanet J Rare Dis       Date:  2014-04-26       Impact factor: 4.123

  2 in total

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