First case report of multivisceral transplant from a deceased cardiac death donor.

The current shortage of pediatric multivisceral donors accounts for the long time and mortality on the waiting list of pediatric patients. The use of donors after cardiac death, especially after the outbreak of normothermic regional perfusion, has increased in recent years for all solid organs except the intestine, mainly because of its higher susceptibility to ischemia-reperfusion injury. We present the first literature case of multivisceral donors after cardiac death transplantation in a 13-month-old recipient from a 2.

Delayed introduction of sirolimus in paediatric intestinal transplant recipients: indications and long-term benefits.

To review our experience using sirolimus in a single centre paediatric intestinal transplantation cohort. Intestinal transplant patients with more than 3 months follow-up were divided into two groups according to their immunosuppression regimen: tacrolimus, (TAC group, n = 45 grafts) or sirolimus (SRL group, n = 38 grafts), which included those partially or completely converted from tacrolimus to sirolimus. The indications to switch were tacrolimus side effects and immunological complications.

Native Spleen Preservation During Visceral Transplantation Inhibits Graft-Versus-Host-Disease Development: Clinical and Experimental Study.

Objective: We aimed to assess whether native spleen preservation during visceral transplantation (VT) affects graft-versus-host-disease (GVHD) incidence.

Summary Background Data: GVHD is one of the most severe and frequently lethal hematological complications after VT procedures. Because there is no specific treatment for GVHD, it is imperative to develop a strategy to reduce donor lymphocyte engraftment and proliferation.

New mutations associated with Hirschsprung disease.

Introduction: Hirschsprung Disease is caused by an impairment in cell migration from the neural crest to the gastrointestinal tract, resulting in an absence of neurons in the myenteric plexus. Many mutations in several genes have been associated to Hirschsprung disease; most of them affecting the RET proto-oncogen pathway. The purpose of this study is the description of novel and known mutations in genes associated to Hirschsprung disease and their prognostic implications.

Graft infusion of adipose-derived mesenchymal stromal cells to prevent rejection in experimental intestinal transplantation: A feasibility study.

Background: Mesenchymal stromal cells (MSC) have been proposed as a promising complement to standard immunosuppression in solid organ transplantation because of their immunomodulatory properties. The present work addresses the role of adipose-derived MSC (Ad-MSC) in an experimental model of acute rejection in small bowel transplantation (SBT).

Material/methods: Heterotopic allogeneic SBT was performed.

Donor's graft ex vivo T-cell depletion with fludarabine reduces graft-versus-host disease signs and improves survival after intestinal transplantation - an experimental study.

Intestinal passenger T leukocytes are responsible for graft-versus-host disease (GvHD) in intestinal transplantation (ITx). We hypothesized that ex vivo fludarabine treatment of the bowel graft would diminish the risk of GvHD and improve overall survival post-transplant. We performed isolated heterotopic small bowel transplantations from Lewis (LEW) to Brown Norway (BN) rat strains, which generated GvHD signs from the fourth day post-transplant.

Current status of pediatric intestinal transplant in Europe.

Purpose Of Review: This review features articles published during 2018 and 2019 regarding pediatric visceral transplantation in Europe. In this biannual review, the authors identify and summarize key articles pertinent to clinical and research areas.

Recent Findings: There is a trend to a lower use of intestinal transplantation in pediatric population in Europe.

[New mutations associated with Hirschsprung disease].

Introduction: Hirschsprung disease is caused by an impairment in cell migration from the neural crest to the gastrointestinal tract, resulting in an absence of neurons in the myenteric plexus. Many mutations in several genes have been associated to Hirschsprung disease; most of them affecting the RET proto-oncogen pathway. The purpose of this study is the description of novel and known mutations in genes associated to Hirschsprung disease and their prognostic implications.

Long-term results of surgery for bowel lengthening: how many transplants are avoided, for which patients?

Purpose Of Review: One of the biggest successes of intestinal rehabilitation programs is that more patients achieve enteral autonomy without transplantation. Many factors are responsible of this accomplishment including new parenteral formulas, better catheter management, surgical management, and the experience of the teams. The purpose of this review is to analyze recent published papers regarding intestinal lengthening procedures trying to find out how many transplantations are avoided and for which patients.

Difficulties, guidelines and review of developing an acute rejection model after rat intestinal transplantation.

Experimental small bowel transplantation (SBT) in rats has been proven to be a useful tool for the study of ischemia-reperfusion and immunological aspects related to solid organ transplantation. However, the model is not completely refined, specialized literature is scarce and complex technical details are typically omitted or confusing. Most studies related to acute rejection (AR) use the orthotopic standard, with small sample sizes due to its high mortality, whereas those studying chronic rejection (CR) use the heterotopic standard, which allows longer term survival but does not exactly reflect the human clinical scenario.

Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome.

Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT.

Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient.

Introduction: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.

Material And Methods: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted.

Ulcerative ileitis after proctocolectomy in children: a complication of ulcerative colitis or a disease in itself after ileoanal pullthrough?

Ulcerative ileitis (UI) after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) is a rare condition described as inflammation of the terminal ileum proximal to the anastomosis. It is mostly observed in ulcerative colitis (UC) and is designated as prepouch ileitis; sometimes with pouchitis, but not necessarily all the time. Its incidence in adults is less than 5%, but the information in children is limited.

Ischemic preconditioning of the graft for intestinal transplantation in rats.

To test the hypothesis that two modalities of IPC should decrease acute rejection and BT after SBTx in rats. Orthotopic allogenic SBTx was performed from Wistar to BN. IPC was performed by 2 ' and 5 ' superior mesenteric artery clamping, following 2-min and 5-min reperfusion before graft cooling and retrieving.

The use of sirolimus as a rescue therapy in pediatric intestinal transplant recipients.

To review our experience with SRL as a second-line therapy in our series of 45 SBTx recipients (1997-2009). Retrospective review of five children converted to SRL: 3 M/2 F; median of three yr old (range 20 months-18 yr); rescue indications, adverse events with SRL, resolution of tacrolimus-related side effects, incidence of rejection, PTLD, or GVHD were summarized. Tacrolimus was discontinued (average 13 months after transplant) because of refractory hemolytic anemia in four patients with decreased renal function and because of advanced renal failure and unclear neutropenia in one.

Autoimmune cytopaenia after paediatric intestinal transplantation: a case series.

Autoimmune cytopaenia is a rare, but severe complication after solid organ transplantation. We retrospectively analysed 57 paediatric intestinal transplants performed in 49 patients between 1999 and 2009. Autoimmune cytopaenia was observed in six patients; it appeared after an average of 10 months post-transplant.

Colonic interposition for esophageal replacement in children remains a good choice: 33-year median follow-up of 65 patients.

Introduction: Gastric pull-up has become the predominant technique for esophageal replacement because of allegedly deficient results of colon grafts. This retrospective study examines the long-term results in a large series of colonic interpositions.

Patients And Method: One hundred six children (median, 2.

Graft-vs-host disease after small bowel transplantation in children.

Purpose: Graft-vs-host disease (GVHD) is a rare complication of transplantation of organs rich in immunocompetent cells. The goal of this study was to report the features of GVHD after small bowel transplantation (SBTx) in children.

Methods: The study involved a retrospective review of patients undergoing SBTx between 1999 and 2009 who had GVHD.

Repeat surgical bowel lengthening with the STEP procedure.

Background: Feasibility of repeat lengthening has been demonstrated in animals, but in humans, none of the three clinical case reports have achieved enteral autonomy after secondary lengthening with serial transverse enteroplasty (STEP).

Patients And Methods: Institutional Review Board approved retrospective review of a single center series of repeat intestinal lengthening after prior Bianchi lengthening or STEP. Outcome measures examined include patient survival, weaning from parenteral nutrition, and need for intestinal transplantation.