TY - CHAP M1 - Book, Section TI - Jeune Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A very rare form of thoracic chondrodysplasia that affects the fetus and usually leads to death in 70% of infants as a consequence of respiratory insufficiency caused by severe thoracic restrictive deformation. Other features include multiple skeletal malformations (especially the rib cage, pelvis, arms and legs), liver, renal, and retinal dysfunctions. The severity of respiratory problems is thought to decrease with age. However, the presence of severe kidney failure and heart problems is known to increase as the child ages, usually by the age of 2 years. Also, with aging, complications associated to an increase in transaminase levels, leads to hepatomegaly, and pancreatic insufficiency, sometimes resulting in portal hypertension. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164074218 ER -