RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164074245
T1 Job Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164074245
RD 2024/04/23
AB A  genetic  immunologic  disorder  characterized  by  recurrent  skin  (eczema-like)  and  severe  lung  infections,  mainly  caused  by  Staphylococcus  aureus,  multiple  fractures,  eczematous  dermatitis,  coarse  facies,  and  elevated  immunoglobulin(Ig)E.  The  presence  of  pneumatoceles  in  association  with  the  skin  lesions  is  a  characteristic  of  this  medical  condition.  In  the  recent  years,  other  features  have  been  described  with  this  condition  and  include  vascular  abnormalities,  including  coronary  aneurysm  without  atherosclerosis,  focal  cerebral  hyperintensities,  and  Chiari  Type  I  malformations.  Mortality  is  usually  associated  to  recurrent  lung  infections  in  adulthood  period.