醫學突破 — — 外科手術醫療團隊第一次成功地用骨髓幹細胞治療隱性營養大皰致或 RDEB ,一種致命性的皮膚疾病。10個被診斷患有這種疾病病人接受移植治療,其中8 人成功治療,2 因併發症而死亡。
由明尼蘇達大學醫學院博士約翰 E.瓦格納和博士 Jakub Tolar 為首,和來自美國、英國和日本的幾個醫生組成的醫療團隊,在2010 年 8 月 12發表最新研究於新英格蘭醫學雜誌。RDEB 是遺傳性,極易受損傷的罕見皮膚疾病,除了在表皮也會影響食道和口腔的上皮組織。
RBED 可依不同的嚴重程度導致死亡的可能性。大多數病人幾乎在年輕時就獲得這種疾病,導致成年後身體變形和嚴重傷痕。RBED 患者有一個極易受傷害的表皮和因膠原蛋白缺少而沒有支撐力。治療後可以透過免疫螢光染色和電子顯微鏡觀察膠原蛋白的表達。骨髓移植患者被發現水泡有大微減少的跡象 — — 提供RBED 患者很大的希望。
Stem Cell Surgery: A New Hope for Patients with RDEB
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.
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.
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.
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.
RBED 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.
Patients 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.
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.
