HemaBlog™

Factor XIII Patients Get New Treatment

This is going to be quite a year with potentially new products.
Here’s one to start the year off right!
The FDA has approved Novo Nordisk’s Tretten® (coagulation
factor XIII A-subunit [recombinant]) for routine prophylaxis of bleeding in
people with congenital factor XIII (FXIII) A-subunit deficiency. This is one of
the rarest inherited bleeding disorders, with an incidence of 1 in 1 million to
1 in 5 million. 
FXIII is composed of two subunits, A and B, with genes on two
different chromosomes. FXIII deficiency is usually caused by a deficiency of
the A-subunit. In the absence of FXIII, loosely formed clots are developed,
leading to bleeding complications similar to those in severe hemophilia A. Tretten
is the only recombinant treatment for the disorder 
Why does this matter? Patients with
congenital FXIII A-subunit deficiency have a lifelong susceptibility to
bleeding, including intracranial hemorrhage (spontaneous bleeding into the
brain), which could be life-threatening if untreated.
For more info: TRETTEN-US.com

Great Book I Just Read


Rebecca by Daphne du Maurier [Kindle]

Rebecca is a gothic romance/psychological
thriller, much along the lines of Jane Eyre, that skillfully transports the reader
into the early 1900s: a young bride (never named) narrates
the tale, about coming to the famed estate of Manderley, as the new bride of middle-aged
Maxim de Winter, whose first wife drowned in a mysterious boating accident only
a year ago. Rich in character, thought and detail, transfixed on the
lush countryside, landscaped grounds and endless ocean as the story progresses.
The mansion seems haunted by the first wife—Rebecca—and every act, every word
by everyone, including Max, seems to scream how missed the charming and
stunning Rebecca is, how hopelessly inadequate the unsophisticated, young new
bride is, until the bride considers ending her life. But then …Things are not
at all what they appear. Through subtle hints and plot twists, the real story
emerges bit by bit to an amazing and gripping ending. The Hitchcock movie by the same name is also fantastic.  Four/five stars.

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