RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58075324
T1 Maroteaux-Lamy 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=58075324
RD 2023/10/03
AB The  three  variants  of  Maroteaux-Lamy  syndrome  (mucopolysaccharidosis  type  VI)  are  severe,  intermediate,  and  mild.  The  severe  form  of  this  condition  is  similar  to  the  severe  form  of  Hurler  syndrome,  except  for  the  preservation  of  intelligence  in  these  patients.  Other  clinical  features  include  macrocephaly,  coarse  facial  features,  and  short  stature.  Corneal  opacities  are  often  present.  Stubby  fingers,  joint  restrictions,  clawhands,  lumbar  lordosis,  and  hip  pain  occur  after  age  3  or  4  years.