TY - CHAP M1 - Book, Section TI - Shwachman Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A rare congenital defect characterized by exocrine pancreatic insufficiency with neutropenia (60% of cases) and growth retardation. Other features may include recurrent and fatal infections, aplastic anemia, leukemia, skeletal abnormalities (metaphyseal dysostosis, thoracic dystrophy), hepatic failure, and ichthyotic skin changes. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164085089 ER -