Dr. William Mentzer
is the principal investigator
for the UCSF branch of the Northern California Comprehensive Sickle Cell
Center, one of the 10 NIH funded national comprehensive Sickle Cell Centers
in the country that provide research into Sickle Cell Anemia. Dr. Mentzer
also heads a variety of national collaborative studies that currently
address problems of low bone density in thalassemia, treatment of hepatitis
C, and new approaches to the management of painful crises in sickle cell
anemia.
Under Dr. Mentzer’s direction, UCSF participates in the Thalassemia
Clinical Research Network which is a collaboration among five North American
clinical centers to examine the blood disorder thalassemia and determine
its prevalence and complications in North America. A Registry collects
routine clinical care baseline data on patients with thalassemia. Using
the Registry data, the investigators will be able to determine how many
patients are potentially eligible for other Thalassemia Clinical Research
protocols.
Dr. Mentzer also directs the Natural History Study of Iron Overload.
The assessment and treatment of iron overload are critical to the outcomes
of ß-thalassemia and sickle cell disease (SCD) requiring red blood
cell transfusions. It has been observed that iron overload produces greater
organ toxicity and earlier death in ß-thalassemia than in SCD, despite
the fact that chelation therapy protocols are identical for both diseases.
It is proposed that the mechanisms and consequences of iron deposition
differ between SCD and ß-thalassemia . This multi-center study will
test if the incidence and rate of development of organ dysfunction differe
between iron overloaded patients with SCD and ß-thalassemia.
The Arginine Supplementation study, headed by Dr. Mentzer, assesses the
physiological effects (both beneficial and deleterious) of the administration
of oral arginine in patients with SCD. It also evaluates the effects of
daily oral arginine on clinical vaso-occlusive events in SCD patients.
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