RT Book, Section
A1 Carey, John C.
A2 Rudolph, Colin D.
A2 Rudolph, Abraham M.
A2 Lister, George E.
A2 First, Lewis R.
A2 Gershon, Anne A.
SR Print(0)
ID 7018082
T1 Chapter 184. Disorders of Accelerated Aging
T2 Rudolph's Pediatrics, 22e
YR 2011
FD 2011
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-149723-7
LK accesspediatrics.mhmedical.com/content.aspx?aid=7018082
RD 2024/04/19
AB The  syndromes  associated  with  an  accelerated  or  premature  aging  (often  called  progeroid)  phenotype  represent  an  uncommon  but  medically  and  biologically  important  class  of  disorders.  These  conditions  are  characterized  by  physical  manifestations  that  produce  a  prematurely  aged  appearance  or  symptoms  suggesting  accelerated  aging  unexpected  for  a  child  or  adolescent.  Although  one  of  these  conditions,  Hutchinson-Gilford  progeria  (HGP),  is  the  prototype  for  this  community  of  diseases,  there  are  at  least  20  human  syndromes  that  have  been  labeled  as  progeroid.  It  is  a  common  mistake  for  both  the  clinician  and  the  media  to  incorrectly  lump  all  of  these  disorders  under  the  heading  of  progeria,  even  though  there  are  several  discrete  entities.  Making  an  accurate  diagnosis  of  the  correct  accelerated/premature  aging  syndrome  is  crucial  because  all  of  these  conditions  vary  in  inheritance  pattern,  natural  history  and  prognosis.  The  plan  of  management  and  health  care  supervision  is  informed  by  the  diagnosis  here,  as  is  the  case  of  all  genetic  syndromes.