Axiell Internet Server 6 | Details | Jeune's syndrome

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Actions	Jeune's syndrome - Asphyxiating thoracic dystrophy Remove from selection Add to selection Object numberGCX.613 TitleJeune's syndrome - Asphyxiating thoracic dystrophy DescriptionInfant who died 70 minuted after birth. X-ray reveals a typical example of Jeune's Syndrome (asphyxiating thoracic dystrophy), an autosomal recessive abnormality causing short limb dwarfism which may be confused with achondroplasia. The distal, rather than the proximal limb bones are affected. There are irregular metaphyses which show spurring. The skull and spine are normal but there is premature ossification of the proximal femoral epiphyses. Because of the short ribs, the chest is narrow both in the antero-posterior and lateral dimensions. The clavicles are long, high and thin. Associated abnormalities are polydactyl and cystic disease of the kidneys and liver. Similar radiological appearances are seen in the Ellis-van Creveld Syndrome, where ectodermal changes also occur. Production date 1975 - 1975 Production periodTwentieth century, late Object nameCHEST Object categoryX-ray Dimensions Width: 16.5 cm Height: 21.5 cm

Object numberGCX.613

TitleJeune's syndrome - Asphyxiating thoracic dystrophy

DescriptionInfant who died 70 minuted after birth. X-ray reveals a typical example of Jeune's Syndrome (asphyxiating thoracic dystrophy), an autosomal recessive abnormality causing short limb dwarfism which may be confused with achondroplasia. The distal, rather than the proximal limb bones are affected. There are irregular metaphyses which show spurring. The skull and spine are normal but there is premature ossification of the proximal femoral epiphyses. Because of the short ribs, the chest is narrow both in the antero-posterior and lateral dimensions. The clavicles are long, high and thin. Associated abnormalities are polydactyl and cystic disease of the kidneys and liver. Similar radiological appearances are seen in the Ellis-van Creveld Syndrome, where ectodermal changes also occur.

Production date 1975 - 1975

Production periodTwentieth century, late

Object nameCHEST

Object categoryX-ray

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