TY - CHAP M1 - Book, Section TI - Wiskott-Aldrich Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - This is a congenital and life-threatening medical immunodeficiency condition affecting mostly males due to its X-linked inheritance. It is characterized by severe eczema, profound thrombocytopenia, immune deficiency causing frequent infections as a result of a defect in both T- and B-cell function. Clinically, affected individuals present for bloody diarrhea and a high incidence of autoimmunity and malignancy. Death frequently occurs during childhood as a result of severe thrombocytopenia and bleeding. The Wiskott-Aldrich Syndrome (WAS)-related disorders of X-linked thrombocytopenia (XLT) and X-linked congenital neutropenia (XLN) are often clinical similar although the XLN is rarer. They are both caused by mutations of the same gene. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/11/11 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164088249 ER -