Background: Children with short bowel syndrome starting in the neonatal period (NB- SBS) are exposed to many live-threatening complications that can affect their outcome before and after transplantation. Our aim was to assess the outcome of this group of children in a single transplant center. Material and methods: Retrospective review of pediatric intestinal transplants performed between 1994 and 2010 at our center. All patients with short bowel syndrome of neonatal onset were included. Those with motility disorders or intestinal epithelium disease but normal bowel length were not included in spite of the congenital character of these diseases. Pre-transplant status, weight, time on the waiting list, type of graft, patient survival, graft survival and immunological complications were compared between patients with NB-SBS and the rest of the children included. T-Student test, Chi-square tests and log-rank were used to compare quantitative, qualitative and survival data respectively. Results: A total of 180 pediatric intestinal transplants were performed in children between 1994 and 2010. Of them, 104 (57%) suffered from short bowel syndrome of neonatal onset, 96 (53%) were male and 85 (46%) females. Patient survival at 1, 5 and 10y were similar between patients with NB-SGS and other children (61, 42, 32% vs 62, 42, 34%; respectively). Graft survival was also similar in the three periods for the two groups (61, 39, 33% vs 51, 35, 32%, respectively). Waiting time was lower but not significantly in the NB-SBS group (77.3 vs 98.8 days) Type of graft distribution was similar in both groups except for the absence of MMV in the group of NB-SBS that contrast to 11% of these grafts in other children. Weight at transplantation was significantly lower in the NB-SGS group (10.2±16.8 vs 17.5±6.8 Kg). Regarding pre-transplant status patients in the NB-SGS were more likely to be in the ICU (24. 2 vs 10.9%) and less at home (44.4 vs 62.2%) compared to patients in the other group. Pre transplant creatinin level was similar the two groups but pre transplant albumin, bilirubin, INR and PT were more seriously impaired in patients in the NB-SGS group compared to other children. Recipient/donor weight rate was similar in both groups (1.2 vs 1.1) in NB-SGS vs others. PTLD was most frequent in children with NB-SBS compared to others (18.1 vs 8.5%). Rejection, GVHD and infectious complications were similar in the two groups. Conclusions: Patients suffering from NB-SGS had similar patient and graft survival compared to the rest of children that received an intestinal transplant. Liver function tests were more impaired in the NB-SGS group, and they were more prone to be in the ICU at the moment of transplant than the rest of children. The rest of pre and post-transplant variables examined did not show any difference.
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