TY - CHAP M1 - Book, Section TI - Job Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A genetic immunologic disorder characterized by recurrent skin (eczema-like) and severe lung infections, mainly caused by Staphylococcus aureus, multiple fractures, eczematous dermatitis, coarse facies, and elevated immunoglobulin(Ig)E. The presence of pneumatoceles in association with the skin lesions is a characteristic of this medical condition. In the recent years, other features have been described with this condition and include vascular abnormalities, including coronary aneurysm without atherosclerosis, focal cerebral hyperintensities, and Chiari Type I malformations. Mortality is usually associated to recurrent lung infections in adulthood period. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/07 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164074245 ER -