Pancreas Transplantation Is Feasible in Donors With Shprintzen-Goldberg Syndrome A. Zanetti-Yabur*, T. Butler, J.P. Rocca, and J.A. Graham Montefiore-Einstein Center for Transplantation, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York ABSTRACT Shprintzen-Goldberg syndrome (SGS) is an autosomal dominant connective tissue disor- der. To date, this report is the first account of a successful pancreas transplantation from an SGS donor. The similarity of the outcomes from previous year-on-year pancreas trans- plantations at the same center demonstrates promising results. Increasing awareness of the utilization of donors with SGS may promote expansion of center-specific criteria for organ acceptance. Therefore, every consideration should be given for use of organs from donors with this genetic abnormality because there is no evidence to suggest poorer allograft viability. W E report here a case of pancreas after kidney trans- plantation (PAK) for a 33-year-old woman with type 1 diabetes with glucose lability and failure of insulin therapy to mitigate metabolic complications only worsened by her diabetogenic steroid-based immunosuppressive regimen. It is noteworthy that the patient underwent an uncomplicated living unrelated kidney transplantation 1 year before pancreas transplantation due to diabetic nephropathy. A medically suitable pancreas donor became available after confirmation of brain death. The donor was an 18- year-old male subject (body mass index, 18.7 kg/m 2 ) who experienced cardiac arrest 2 days after surgical debridement for a left elbow abscess. Importantly, his medical history was significant for Shprintzen-Goldberg syndrome (SGS), autism, Hirschsprung’s syndrome with a pull-through procedure, and an orchiectomy. At the time of recovery, amylase (77 U/L) and lipase (74 U/L) levels were normal, glycosylated hemoglobin level was 5.4%, and the gland had a normal texture. Implantation of the graft was accomplished by using a Y graft to the superior mesenteric artery and splenic artery to the left external iliac artery. Systemic venous drainage was provided with an extra-vessel iliac vein interposition graft between the portal vein and left external iliac vein (cold ischemia time, 6:04 hours; warm ischemia time, 32 minutes). Enteric drainage was accomplished with anastomosis of the C-loop of the donor duodenum and recipient jejunum 25 cm distal to the ligament of Treitz. Notably, the patient was discharged on postoperative day 6, and at her 1-month follow-up, she is euglycemic and does not require antidia- betic agents for glucose control. SGS is an autosomal dominant connective tissue disorder that affects the protein transforming growth factor be signaling pathway, frequently with a missense mutation in the Fibrillin gene that accounts for many of the clinical features resembling Marfan syndrome [1]. However, pa- tients with SGS have a combination of findings that are distinct from Marfan syndrome, including craniosynostosis, hypotonia, obstructive apnea, congenital weakness of the abdominal wall, foot deformity, and neurodevelopmental abnormalities. To date, the present report is the first ac- count of a successful pancreas transplantation from an SGS donor. Although a variant of Ehlers-Danlos syndrome has been associated with poor outcomes after transplantation, the SGS phenotype does not connote visceral connective tissue abnormalities [2]. Moreover, liver transplantation using an allograft from a donor with Marfan syndrome showed promising results, suggesting that donors with the phenotypical expression shared by SGS and Marfan syn- drome may be suitable for organ allocation [3]. Therefore, every consideration should be given for use of organs from donors with this genetic abnormality because there is no evidence to suggest poorer allograft viability. Importantly, when comparing this patient versus the previous year-on-year pancreas transplants, there is no dif- ference in outcomes. Assessing for postoperative peak *Address correspondence to Alana Zanetti-Yabur, MD, Montefiore-Einstein Center for Transplantation, Albert Einstein College of Medicine, 111 E. 210th St, Rosenthal 2, Bronx, NY 10467. E-mail: alana.zanetti-yabur@einstein.yu.edu ª 2017 Elsevier Inc. All rights reserved. 230 Park Avenue, New York, NY 10169 0041-1345/17 http://dx.doi.org/10.1016/j.transproceed.2017.06.024 Transplantation Proceedings, 49, 1883e1884 (2017) 1883