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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