Literature DB >> 2748234

A new type of lethal short-limbed dwarfism.

E R Nairn1, S Chapman.   

Abstract

Details are presented of a most unusual osteo-chondrodysplasia which presents with lethal neonatal short-limbed dwarfism, defective ossification and nodular calcification within cartilage. The features resemble one case previously described in the literature. We wish to present a new type of lethal short-limbed dwarfism which has presented in one family. Three affected infants have been born to unaffected parents who are first cousins. The third affected infant is reported in detail and the radiological signs of all three sibs are summarised in Table 1. There are also two healthy children, a boy and a girl, born at term.

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Year:  1989        PMID: 2748234     DOI: 10.1007/bf02386847

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


  5 in total

1.  Intracytoplasmic inclusion bodies in the chondrocytes of type I lethal achondrogenesis.

Authors:  S S Yang; K P Heidelberger; J Bernstein
Journal:  Hum Pathol       Date:  1976-11       Impact factor: 3.466

2.  Heterogeneity of Chondrodysplasia punctata.

Authors:  J W Spranger; J M Opitz; U Bidder
Journal:  Humangenetik       Date:  1971

3.  A bone dysplasia for diagnosis.

Authors:  R Astley; A C Kendall
Journal:  Ann Radiol (Paris)       Date:  1980-02

4.  Rib-gap defect with micrognathia, malformed tracheal cartilages, and redundant skin: a new pattern of defective development.

Authors:  D W Smith; K Theiler; G Schachenmann
Journal:  J Pediatr       Date:  1966-11       Impact factor: 4.406

5.  Lethal short-limbed chondrodysplasia in early infancy.

Authors:  S S Yang; K P Heidelberger; A J Brough; D P Corbett; J Bernstein
Journal:  Perspect Pediatr Pathol       Date:  1976
  5 in total
  3 in total

1.  Case report 693: Schneckenbecken dysplasia.

Authors:  A Giedion; K Biedermann; J Briner; R Soler; M Spycher
Journal:  Skeletal Radiol       Date:  1991       Impact factor: 2.199

2.  A lethal skeletal dysplasia with features of chondrodysplasia punctata and osteogenesis imperfecta: an example of Astley-Kendall dysplasia. Further delineation of a rare genetic disorder.

Authors:  N Elçioglu; C M Hall
Journal:  J Med Genet       Date:  1998-06       Impact factor: 6.318

Review 3.  A new case of Greenberg dysplasia and literature review suggest that Greenberg dysplasia, dappled diaphyseal dysplasia, and Astley-Kendall dysplasia are allelic disorders.

Authors:  Pernille A Gregersen; Victoria McKay; Maie Walsh; Erica Brown; George McGillivray; Ravi Savarirayan
Journal:  Mol Genet Genomic Med       Date:  2020-04-18       Impact factor: 2.183

  3 in total

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