TY - CHAP M1 - Book, Section TI - Griscelli Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Griscelli Syndrome is a rare autosomal recessive disorder characterized by albinism with immunodeficiency that usually causes death by early childhood. It is characterized by partial pigmentary dilution of the skin and hair (silvery gray hair), frequent infections, neurologic abnormalities, and fatal outcome caused by uncontrolled T lymphocyte and macrophage activation. Clinical features include the presence of large clumps of pigment in hair shafts and a pathological accumulation of melanosomes in melanocytes. Two types are described: Type I with severe neurologic impairment and Type II with immunologic deficiency. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/11/03 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164071658 ER -