RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58078609
T1 Griscelli Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58078609
RD 2024/04/16
AB Albinism  with  immunodeficiency  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.