RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164072374
T1 Hemolytic Anemia, Congenital, with Emphysema and Cutis Laxa
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164072374
RD 2024/04/25
AB Cutis  laxa  is  an  acquired  or  inherited  skin  disorder  characterized  by  wrinkled,  inelastic  skin.  It  is  a  very  rare  syndrome  that  is  characterized  by  hemolytic  anemia  with  early-onset  emphysema  and  cutis  laxa.  The  presence  of  excessive  skinfolds  and  loose,  redundant  skin  leads  to  early  diagnosis.  Associated  features  include  cardiac  valve  anomalies  and  hernias.  Aortic  root  dilatation  might  result  in  life-threatening  aortic  dissection.  Poor  prognosis.