RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164075347 T1 Kinky Hair Syndrome T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164075347 RD 2021/01/18 AB It is a genetic disorder of copper metabolism that is usually diagnosed prenatally which subsequently evolves progressively to a degenerative mechanisms involving several organs of the body and especially the brain. It is characterized by seizures, mental retardation, stunted growth, failure to thrive, unstable body temperature, and very unusual color and texture of hair. Caused by defective intestinal copper absorption that results in severe mental retardation, failure to thrive, sparse hair, and osteoporosis.