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| Dr. James G. Colson, Research Director |
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PLE Research Update
Dr. James G. Colson
March 2005
In the time since our last report, progress on our major funded research in Fontan-based cardiac blood flow control at the Children?s Hospital of Philadelphia and intestinal mucosa integrity studies in glycosylation at the Burnham Institute have continued to meet milestones and are on target for 2005 completion. We have increased staffing at the Burnham Institute by $32,000 with the addition of Fangrong Zhang, MD, PhD. most recently from the Dana-Farber Cancer Institute in the Harvard Medical School.
We continue to carefully follow these projects and search the literature and our many contacts for new ?leads.? Every month reports occur of some new cure of Fontan-based PLE. Recently a PLE cure happened on adding calcium to the patient?s diet. Another cure happened on moving a patient to a higher climate in Denver. We just don?t know how to treat these anecdotal cases. So we put them in a file and hope for finding new similar cases.
Heart transplantation has many problems as a method for solving heart problems, but for unknown reasons, the procedure has cured PLE in most reported cases. We have found in the literature fourteen instances of such success. The studies have limitations! PLE often occurs many years after the operation so that longer term follow up is critical. Such long term follow up records are not easily found since surgeons are more interested in the shorter success of the operation and leave the longer term follow-up to the attending cardiologist. An example is a study from the Indiana Medical School wherein 129 Fontan procedures were completed with no PLE. I have not found a representative willing to discuss these findings. Mortality rates during follow up are equally hard to find. This investigational area is continuing with cardiologist.
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Dr. Attila Tarnok of the Klinik fur Kinderkardiologie in Leipzig, one of our recent Symposium Speakers, has submitted a Grant request for $45,000 to study PLE risk factors that a child faces before the Fontan operation. Such a prediction might lead a parent to undertake other surgeries, such as heart transplantation, rather than the Fontan. This will not be a trivial decision as heart transplantation has many of its’ own problems! This Institute is particularly valuable in that they have over 100 patients with univentricular heart problems available for some level of investigation.
The above Institute at Leipzig is continuing to acquire data relating Fontan related PLE and childhood infections. The case, based on ten patients, appears solid and has been published extensively. The fact that the data is from retrospective studies causes me some significant concern. Retrospective studies rely on a memory. Memories are often flawed. More prospective data is needed and will be integrated as it becomes available! All agree that PLE is not a simple “one hit” disease. The ongoing thesis is that infection may be necessary, but not sufficient for PLE to occur.
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We have added Aamir Jeewa, MD. to our effort on PLE with special emphasis on gastrointestinal causes. Dr. Jeewa is a University of Toronto graduate with a Biology degree. His Medical degree is from the University College in Dublin. He is currently a second year Pediatric Resident at Women and Children’s Hospital of Buffalo. He will work with us and Dr. Rychik at the Children’s Hospital of Philadelphia to bring this data to a valid conclusion.
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