TY  - CHAP
M1  - Book, Section
TI  - Erdheim-Chester Disease (ECD)
A1  - Bissonnette, Bruno
A1  - Luginbuehl, Igor
A1  - Engelhardt, Thomas
PY  - 2019
T2  - Syndromes: Rapid Recognition and Perioperative Implications, 2e
AB  - Systemic  non-Langerhans  cell  histiocytic  disorder  characterized  by  the  abnormal  accumulation  of  histiocytes  (macrophages)  most  often  called  the  non-Langerhans-cell  histiocytosis.  This  leads  to  the  development  of  lipoid  granulomas  in  many  organs  and  tissues  of  the  body,  especially  the  bone  marrow,  leading  to  generalized  sclerosis  of  the  long  bones.  The  onset  of  the  disease  is  the  middle  age.  The  clinical  features  also  include  in  more  than  50%  of  affected  individuals  cases  extraskeletal  involvement  such  as  kidney,  skin,  brain,  and  lung  disease,  and  less  frequently  retroorbital  tissue,  pituitary  gland,  and  heart.  The  clinical  manifestations  range  from  asymptomatic  to  fatal  multisystem  derangement.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accessanesthesiology.mhmedical.com/content.aspx?aid=1164069100
ER  -