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Jeune Syndrome

Jeune Syndrome is a rare autosomal recessive,multisystem skeletal structural disorder which has a 1/100.000-130.000 frequency.
It is mostly characterized by narrow thorax with small ribs and dwarfism with short extremities.
The prognosis of disease depends on the severity of rib cage shape.

In Most of the cases,patient loses life with respiratory failure in early infancy.If the patient survives in this period,hepatic failure or failure of pancreas later.

Jeune Syndrome is also seen acquiredly in patients who had a surgery from ribs before because of another reason at a young age.

In the surgical treatment of jeune syndrome,it is applied augmentation surgery which lengtened the ribs with metalic prothesis and enlarges the chest.