Stem Cell Surgery: A New Hope for Patients with RDEB

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Friday, August 13th, 2010 |
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Stem Cell Surgery: A New Hope for Patients with RDEB: a; q" l. D5 ~
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' k: L8 L+ u9 w! u- PSource: www.rso.cornell.edu
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A medical breakthrough – for the very first time in surgical procedure history, a medical team was able to successfully use bone marrow stem cells as treatment for recessive dystrophic epidermolysis bullosa or RDEB, a rare and fatal kind of skin disease.' L$ r+ w; A: d" @; F% r  Y
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On the record, the treatment was performed on 10 minor patients who have been diagnosed with an aggressive form of the disease and 8 were successfully treated, 2 died from complications.
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The medical team was headed by Dr. John E. Wagner and Dr. Jakub Tolar, fellows form the University of Minnesota Medical School. The team consists of several doctors from the United States, UK and Japan. Their research is currently on print in the New England Journal of Medicine out last August 12, 2010.' e, Q" C! W7 {; R5 o
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RDEB is a rare skin disease, genetic in origin which leaves the skin extremely vulnerable to scraping and injury. Aside from the epidermis, the disease also affects the esophagus and skin lining the oral cavities./ D4 @4 f2 v" \! b* F0 I

; K' O& A& F4 D; eRBED can vary in severity, the most severe of which can lead to fatalities. This disease is an extremely painful experience, especially for those who are in the advanced stages. Most patients who acquire the disease at a young age barely make it to adulthood and can are usually deformed and severely scarred.
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8 n/ ]$ i( l+ v& [/ V" aPatients with RBED have an extremely vulnerable epidermis and very prone to injury because of the absence of supporting structures, such as collagen 7 to hold each of its layers together. Because of the very visible symptoms of the condition, patients are usually diagnosed young.  S, [- s1 `0 g6 R8 y
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The treatment consists of triggering the expression of collagen 7 which can be detected through immunofluorescence staining and transmission electron microscopy. After the bone marrow transplantation, blister formations in patients were significantly reduced – a sign of great hope for patients with RBED.