Research Projects Funded 2005

2005 Research Grants Funded and Completed

Mechanism of Disease: $50,000 Research Grant

Carmelo Bernabeu, PhD., Madrid, Spain, for the project: Primary
Cultures of Endothelial and Monocytic Cells Derived from HHT Patients:
An Open Window to Unravel the Pathogeniticy of HHT

Findings to date reported in 4 articles in 3 journals:

  1. Journal Human Mutation
  2. Journal Cardiovascular Research (2)
  3. Journal Molecular Diagnostics and Genetics

Treatment of Disease: Laser Treatment for Nosebleeds: When Is It Likely to Work?

By Elizabeth J. Mahoney and Stanley Shapshay. New classification of nasal vasculature patterns in hereditary hemorrhagic telangiectasia. American Journal of Rhinology. Volume 20, Number 1. January-February 2006.

Over the years, a wide variety of treatments have been used to help
manage nosebleeds in HHT patients including electro-cauterization,
nasal packing, septodermoplasty (see article herein), arterial
embolization, arterial ligation, and hormone therapy. While
many of these interventions have achieved moderate success in the
management of the HHT patient�s nosebleeds, recent attention has
focused on the use of lasers for the treatment of intranasal
telangiectases in patients with HHT.

Finally, patients responses to therapy were evaluated. Patients
were identified as a responder to the Nd-YAG laser treatment if they
noted a 50% decrease in the severity and frequency of their nosebleeds
for a period of greater than 6 months.

Interestingly, vascular pattern I was most common, while vascular pattern III was least common. Patients with vascular pattern I uniformly demonstrated a good response to the Nd-YAG laser. Conversely, nearly all of the patients with vascular pattern II were poor responders to the Nd-YAG laser treatments. Finally, 80% of patients with pattern III demonstrated a good response to the Nd-YAG laser photocoagulation treatment.

In addition to correlating vascular pattern with response to Nd-YAG
laser photocoagulation, vascular pattern classification was also
correlated with disease severity. Patients with vascular
pattern I tended to be most mildly impacted by HHT, while patients with
vascular pattern II were more severely impacted. Those with pattern III were more broadly distributed over the range of disease severity.

These findings are important for physicians caring for HHT patients
as they may help to predict response to laser therapy. For example,
when a patient with HHT is noted to have isolated intranasal
telangiectases, he or she can be counseled to anticipate a good
response to Nd-YAG laser photocoagulation, while patients with a more
diffuse vascular pattern almost uniformly have a sub-optimal response
to their Nd-YAG laser treatments. For patients with
patterns I and III, treatment with the Nd-YAG laser will typically
provide an improvement in the intensity and frequency of epistaxis for
a minimum of six months. The procedure is simple, requires only local anesthesia with sedation, and has few risks or side-effects. To
conclude, the findings in this study will allow otolaryngologists to
better counsel HHT patients and improve stratification for therapy.