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Jeune syndrome
  1. M B O’Connor1,
  2. D P Gallagher1,
  3. E Mulloy2
  1. 1
    Department of Medicine, Mid-Western Regional Hospital, Limerick, Ireland
  2. 2
    Department of Respiratory Medicine, St Johns Hospital, Limerick, Ireland

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    Jeune syndrome was first described as familial asphyxiating thoracic dystrophy in a pair of siblings with severely narrow thoracic cage by Jeune et al in 1955.1 It is a rare genetic disorder, with a poor survival rate beyond the neonatal period,2 known to be genetically heterogeneous and characterised by short limbs, underdeveloped iliac wings and a narrow rigid thoracic cage, which often results in asphyxiation.1 Survival has been reported to the fourth decade of life,3 with no race …

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