TY  - CHAP
M1  - Book, Section
TI  - Inherited Bleeding Disorders
A1  - Diaz, Rosa
A1  - Yee, Donald L.
A2  - Kline, Mark W.
PY  - 2018
T2  - Rudolph's Pediatrics, 23e
AB  - Inherited  bleeding  disorders  are  a  heterogeneous  group  of  conditions  that  include  disorders  of  primary  and  secondary  hemostasis.  During  primary  hemostasis,  platelets  adhere  and  aggregate  at  the  site  of  vascular  injury  to  form  a  platelet  plug.  Bleeding  into  the  skin  (eg,  petechiae  and/or  ecchymosis)  and  mucous  membranes  typifies  disorders  of  primary  hemostasis  such  as  von  Willebrand  disease  (VWD).  Secondary  hemostasis  leads  to  stabilization  of  the  platelet  plug  through  activation  of  the  coagulation  cascade  and  production  of  cross-linked  fibrin.  Patients  affected  by  a  disorder  of  secondary  hemostasis  such  as  hemophilia  or  1  of  the  rare  bleeding  disorders  (RBDs)  classically  bleed  into  soft  tissues,  muscles,  and  joints.  In  general,  children  with  a  bleeding  disorder  may  present  with  severe  or  life-threatening  bleeding  episodes  early  in  life,  or  in  a  more  indolent  manner,  with  symptoms  that  may  not  seem  very  unusual  (eg,  frequent  nosebleeds  or  heavy  menstrual  bleeding).  Recognizing  the  clinical  and  laboratory  features  that  suggest  the  presence  of  an  inherited  bleeding  disorder  is  paramount  to  optimizing  treatment  and  long-term  outcomes.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/20
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1182910120
ER  -