Literature DB >> 7700712

Bone dysplasias of infancy in the Vienna collection.

P Beighton1, E Sujansky, B Patzak, K A Portele.   

Abstract

The Vienna Museum of Pathological Anatomy contains a vast collection of abnormal skeletons. We have appraised this material and attempted to establish firm diagnoses of specific genetic disorders in terms of modern syndromic concepts. A number of the skeletons in the museum are those of deceased neonates; in many instances it was impossible to reach a diagnosis on the basis of the outward appearance but radiographic investigations confirmed diagnoses including osteogenesis imperfecta type II, thanatophoric dysplasia, achondroplasia and achondrogenesis. The Vienna collection represents a priceless resource for the investigation of genetic skeletal disorders of this type.

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Year:  1994        PMID: 7700712     DOI: 10.1007/bf02011901

Source DB:  PubMed          Journal:  Pediatr Radiol        ISSN: 0301-0449


  2 in total

Review 1.  International classification of osteochondrodysplasias. International Working Group on Constitutional Diseases of Bone.

Authors:  P Beighton; Z A Giedion; R Gorlin; J Hall; B Horton; K Kozlowski; R Lachman; L O Langer; P Maroteaux; A Poznanski
Journal:  Am J Med Genet       Date:  1992-09-15

2.  Genetic skeletal dysplasias in the Museum of Pathological Anatomy, Vienna.

Authors:  P Beighton; E Sujansky; B Patzak; K A Portele
Journal:  Am J Med Genet       Date:  1993-11-01
  2 in total
  3 in total

1.  [Karl Alfons Portele, Pathologist and first director of the Federal Pathologic-anatomical Museum Vienna].

Authors:  Beatrix Patzak; Eduard Winter
Journal:  Wien Med Wochenschr       Date:  2013-07-16

Review 2.  Radiological imaging of teratological fetuses: what can we learn?

Authors:  Lucas L Boer; A N Schepens-Franke; J J A van Asten; D G H Bosboom; K Kamphuis-van Ulzen; T L Kozicz; D J Ruiter; R-J Oostra; W M Klein
Journal:  Insights Imaging       Date:  2017-04-24

3.  Detection of G1138A Mutation of the FGFR3 Gene in Tooth Material from a 180-Year-Old Museological Achondroplastic Skeleton.

Authors:  Lucas L Boer; Jana Naue; Laurens de Rooy; Roelof-Jan Oostra
Journal:  Genes (Basel)       Date:  2017-08-29       Impact factor: 4.096

  3 in total

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