title: [Infant respiratory distress revealing Jeune syndrome].
abstract: Jeune syndrome (asphyxiating thoracic dystrophy) is a rare autosomal recessive osteochondrodysplasia with a variable degree of severity, clinically characterized by respiratory distress with a narrow chest and limb shortness. The reported incidence is one to five in 500,000 live births. Most patients develop severe respiratory failure during the first 2 years of life, leading to death in 60-80 % of cases. Survivors may suffer from renal, hepatic, or pancreatic complications. Expanding thoracic surgery can be used for severe cases. We describe the case of an 18-month-old boy who developed mild respiratory distress. The patient showed typical radiological features of Jeune syndrome: narrow thorax with short ribs and trident appearance of the pelvis. This case underscores the value of the right interpretation of the chest radiograph of the infant with a respiratory distress syndrome.
authors.affiliation: Service de pédiatrie, clinique Notre-Dame de Grâce, 212, chaussée de Nivelles, 6041 Gosselies, Belgique.
authors.forename: J
authors.initials: J
authors.lastname: Page
authors.affiliation: Service de pédiatrie, CHU UCL Namur site Godinne, 1, avenue du Docteur-Gaston-Therasse, 5530 Yvoir, Belgique.
authors.forename: E
authors.initials: E
authors.lastname: Bodart
authors.affiliation: Service de pédiatrie, clinique Notre-Dame de Grâce, 212, chaussée de Nivelles, 6041 Gosselies, Belgique. Electronic address: jeanluchennecker@skynet.be.
authors.forename: J-L
authors.initials: JL
authors.lastname: Hennecker
mesh_terms: D004613:Ellis-Van Creveld Syndrome / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008297:Male; D012131:Respiratory Insufficiency / Q000209:etiology*