TY  - CHAP
M1  - Book, Section
TI  - Periodic Fever Syndromes
A1  - Akoghlanian, Shoghik
A1  - Zeft, Andrew
A2  - Usatine, Richard P.
A2  - Sabella, Camille
A2  - Smith, Mindy Ann
A2  - Mayeaux, E.J.
A2  - Chumley, Heidi S.
A2  - Appachi, Elumalai
PY  - 2015
T2  - The Color Atlas of Pediatrics
AB  - A  6-year-old  Caucasian  boy  is  seen  by  his  pediatrician  for  a  14-month  history  of  fever  episodes  lasting  up  to  10  days.  During  these  episodes,  he  develops  a  red  rash,  nonspecific  joint  pains,  and  abdominal  pain  variably  accompanied  by  diarrhea.  Between  episodes  he  is  asymptomatic.  He  has  had  serial  evaluations  in  the  primary  care  clinic  and  has  been  admitted  to  a  children’s  hospital  for  work  up  to  rule  out  potential  infectious,  gastrointestinal,  and  oncologic  etiologies.  The  work-up  is  only  positive  for  nonspecific  elevations  in  inflammatory  markers  and  a  mild  leukocytosis.  The  pediatrician  suspects  a  periodic  fever  syndrome  and  refers  the  child  to  a  pediatric  rheumatologist.  During  an  episode  of  fever,  the  child  is  evaluated  by  the  pediatric  rheumatologist.  The  only  clinical  finding  is  a  rash  on  the  back  and  trunk  (Figure  176-1).  Work-up  for  a  periodic  fever  syndrome  reveals  a  heterozygous  missense  mutation  in  the  gene  encoding  the  cell  surface  receptor  for  tumor  necrosis  factor  (TNF)  TNFRSF1A,  and  the  diagnosis  of  tumor  necrosis  factor  receptor-associated  periodic  syndrome  (TRAPS)  is  made.  The  boy  is  treated  with  etanercept,  a  soluble  TNF-α  receptor  fusion  protein,  after  which  the  frequency  and  severity  of  his  episodes  lessen.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/23
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1114878306
ER  -