Connective tissue disorders research
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Abstract
Abstract
Asphyxiating thoracic dystrophy, also known as Jeune syndrome, is a rare skeletal dysplasia characterized by a constricted thoracic cage and variable skeletal abnormalities that may result in severe respiratory insufficiency in the neonatal period. We report the case of a term male neonate admitted on the first day of life for acute respiratory distress. Imaging revealed a markedly narrow thorax with short ribs and elevated clavicles, associated with characteristic pelvic abnormalities including trident-shaped acetabula and short iliac bones, consistent with Jeune syndrome. Despite intensive supportive care and mechanical ventilation, the clinical course was complicated by progressive respiratory failure, resulting in death after 7 days. This case illustrates the characteristic imaging findings of Jeune syndrome and emphasizes the role of imaging in establishing the diagnosis.
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@article{Aouadi2026rare,
title = {A rare cause of neonatal respiratory distress: Jeune syndrome},
author = {Salma El Aouadi and Soukaina Bahha and Ouiam Taibi and Rania Bouanane and Nazik Allali and Latifa Chat and Siham El Haddad},
journal = {Radiology Case Reports},
year = {2026},
doi = {10.1016/j.radcr.2026.05.059},
url = {https://doi.org/10.1016/j.radcr.2026.05.059}
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