Successful identical-twin living donor small bowel transplant for necrotizing enterovasculitis secondary to Churg-Strauss syndrome. Transpl Int 2006 Jul;19(7):594-7
Date
06/13/2006Pubmed ID
16764639DOI
10.1111/j.1432-2277.2006.00316.xScopus ID
2-s2.0-33744485402 (requires institutional sign-in at Scopus site) 17 CitationsAbstract
Churg-Strauss syndrome (CSS) is a granulomatous small-vessel vasculitis with unknown etiology. Extra-pulmonary manifestations of CSS are currently treated with a combination of steroids and Cyclophosphamide. Its gastrointestinal complications may be devastating, occasionally requiring extensive bowel resection resulting in short-gut syndrome. Living-related small bowel transplantation (LRSBTx) is a relatively standardized procedure that, not only represents a valid alternative to cadaver bowel transplant in selected cases, but also portraits excellent results when performed in experienced centers. The availability of an identical twin as a donor, which allows avoidance of immunosuppressive therapy, is a major indication for this procedure. We present the case of a young individual affected by gastrointestinal necrotizing vasculitis that lost almost his entire small bowel requiring the immediate institution of total parenteral nutrition (TPN). However, within few weeks a significant hepatic dysfunction ensued. An identical twin-brother, not affected with CSS, became an immediate, optimal donor-candidate for LRSBTx, the first of this kind in a patient affected with CSS. Following the procedure, two main concerns were addressed: the recipient's ability to recover a regular intestinal function without immunosuppression and the possible recurrence of the primary disease. Twenty-seven months post-transplant, the patient enjoys a regular lifestyle without any clinical, endoscopic and histologic evidences of recurrent disease in the transplanted graft.
Author List
Schena S, Testa G, Setty S, Abcarian H, Benedetti EAuthor
Stefano Schena MD, PhD Associate Professor in the Surgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultDiseases in Twins
Graft Survival
Humans
Intestines
Living Donors
Male
Necrosis
Short Bowel Syndrome
Syndrome
Tissue Transplantation
Tissue and Organ Harvesting
Transplantation
Twins, Monozygotic
Vasculitis