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Rối loạn trội NST

DupuytrenÕs là sự co fibroproliferative Một rối loạn trội NST thường thừa kế đó ảnh hưởng đến nam giới thường NHẤT trên 60 tuổi của Ai là Scandinavian, Ai Len, hoặc Đông Âu gốc. Thiếu máu cục bộ địa phương các mạch máu trong bàn tay và yếu tố tăng trưởng tiểu cầu cụ thể có nguồn gốc từ nguyên bào sợi | Dupuytren s Contracture Leon S. Benson MD Craig S. Williams MD and Marjorie Kahle OTR L Abstract Dupuytren s contracture is a fibroproliferative disorder of autosomal dominant inheritance that most commonly affects men over age 60 who are of Scandinavian Irish or eastern European descent. Local microvessel ischemia in the hand and specific platelet-derived and fibroblast growth factors act at the cellular level to promote the dense myofibroblast population and altered collagen profiles seen in affected tissue. Surgical treatment depends to some degree on patient preference and a clear understanding of the possible complications and considerable postoperative therapy commitment. Operative management is appropriate when metacarpophalangeal or proximal interphalangeal joint contracture exceeds 30 degrees. A volar zigzag Brunner incision in the digit and palm provides reliable exposure and leads to predictable healing in most cases. The mainstay of postoperative hand therapy is early active-flexion range-of-motion exercises to restore grip strength. A nighttime extension splint is often used for several months postoperatively to maintain the correction achieved in the operating room. Early recurrence of disease is most common in individuals with Dupuytren s diathesis use of full-thickness skin grafts may be helpful for these patients. J Am Acad Orthop Surg 1998 6 24-35 The first description of Dupuytren s disease has been credited not to Guillaume Dupuytren but to Felix Plater of Basel Switzerland 1536-1614 .1 Plater s description of palmar fibromatosis was published in his book cataloguing autopsy findings Observationum in Hominis Affectibus published in 1614 . Plater described the case of a stonecutter who sustained a severe traction injury to the small finger and subsequently presented with a fixed flexion contracture of the digit. This case has been cited as the first record of Dupuytren s disease probably because Plater s description focused on the progressive .