RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164079370 T1 Muckle-Wells 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=1164079370 RD 2024/10/11 AB It is a very rare genetic disorder with an onset in infancy and characterized by deafness (adolescence), nonpruritic urticaria, and renal amyloidosis type AA. The most common presentation of AA amyloidosis is renal in nature, including proteinuria, nephrotic syndrome and progressive development of renal insufficiency leading to End Stage Renal Disease. Other clinical features include arthralgias and/or conjunctivitis.