Why is pulmonary development often stifled in children who have asphyxiating thoracic dystrophy?

Question

Quizplus · Accepted Answer

Jeune syndrome or asphyxiating thoracic dystrophy is a rare osteochondrodystrophy with an autosomal recessive inheritance pattern. The development of the cartilage and bones is affected, impacting the rib cage, pelvis, and extremities. Symptoms can range from mild to severe. It is a type of thoracic insufficiency syndrome as the chest cavity is decreased in both the anteroposterior and superoinferior orientations. This leads to pulmonary hypoplasia as a result of the decreased cavity size, and after birth the lungs are not able to fully expand. Multiple associated defects, including polydactyly, hypoplastic iliac wings, and fixed clavicles, may be seen.

Why Is Pulmonary Development Often Stifled in Children Who Have