Bus yunhii - sammy08

[ArchivalUser]

If you remove deafness out of Jervell Lange Neilson, what will it become ?

[ArchivalUser]

Romano ward

[ArchivalUser]

yup you are right !!

[ArchivalUser]

just like - if you remove fever and CNS symptoms from TTP it becomes HUS...

not a great one...but still...

:-)

[ArchivalUser]

well, not a bad example but it's not absolute removal of CNS symptoms. just that in HUS renal is more predominant than TTP

[ArchivalUser]

Romano ward is commoner, Autosomal Dominant,
TTP and HUS are spectrum
Defective protein in TTP is ADAMTs: a protenase that normally cleaves vWF and prevent platelet aggregation in otherwise normal situation. that is why TTp is a procoagulative state

[ArchivalUser]

hmmm...ok

thanks