Addressing extreme size mismatch in pediatric intestinal transplantation: Outcomes of intestinal length reduction.

Background: Bench liver reduction, with or without intestinal length reduction (LR) (coupled with delayed closure and abdominal wall prostheses), has been a strategy adopted by our program for small children due to the limited availability of size-matched donors. This report describes the short, medium, and long-term outcomes of this graft reduction strategy.

Methods: A single-center, retrospective analysis of children that underwent intestinal transplantation (April 1993 to December 2020) was performed.

An international multicenter validation study of the Toronto listing criteria for pediatric intestinal transplantation.

Deciding which patients would benefit from intestinal transplantation (IT) remains an ethical/clinical dilemma. New criteria* were proposed in 2015: ≥2 intensive care unit (ICU) admissions, loss of ≥3 central venous catheter (CVC) sites, and persistently elevated conjugated bilirubin (CB ≥ 75 μmol/L) despite 6 weeks of lipid modification strategies. We performed a retrospective, international, multicenter validation study of 443 children (61% male, median gestational age 34 weeks [IQR 29-37]), diagnosed with IF between 2010 and 2015.

Long-Term Cognitive Outcomes and Associated Quality of Life of Young Adults Who Experienced Liver Transplantation in Early Childhood.

We evaluated long term outcomes in infants born between 1992 and 2002 with cholestatic liver disease (CLD) who underwent successful liver transplantation (LT). A total of 160 children with CLD were identified: 68 had developmental assessments before and after LT of whom 32 were excluded because they were followed up elsewhere; 16/36 consented to complete measures of IQ, anxiety, depression, health related quality of life (HRQoL), and a habits/employment survey. Illness severity and developmental attainment prior to LT were comparable with the 32 excluded and 20 patients who declined to take part.

New Insights Into the Indications for Intestinal Transplantation: Consensus in the Year 2019.

In 2001, a Statement was published that described indications for intestinal transplantation in patients with intestinal failure expected to require parenteral nutrition indefinitely. Since 2001, advances in the management of intestinal failure including transplantation and patient survival, both on extended parenteral nutrition and after transplantation, have improved, leading to a reduction in the number of intestinal transplants worldwide from a peak of 270 per year in 2008 to 149 per year in 2017. These changes suggest that the original 2001 Statement requires reassessment.

Hypogammaglobulinemia and bacterial infections following pediatric post-transplant lymphoproliferative disorder in the rituximab era.

Introduction: Treatment of PTLD using immune-depleting agents such as RTX may be associated with increased risk of infections. The aim of this report was to describe the incidence of hypogammaglobulinemia and bacterial infections in children with PTLD after SOT at a single center since the introduction of RTX.

Methods: A retrospective review was conducted over a study period of 2000-2016 in pediatric patients diagnosed with biopsy-proven PTLD based on the WHO histologic criteria.

Long-term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry.

Objectives: Children with liver disease have increased risk of long-term cognitive deficits. We differentiated between the effects of chronic liver disease from that associated with transplantation by recruiting children with cholestatic liver disease (CLD) with and without transplantation.

Methods: Psychometric measures and magnetic resonance spectroscopy were obtained for 3 groups of children: stable liver disease without transplantation; CLD from birth with transplantation; and individuals healthy to 18 months of age, before transplantation for acute liver failure.

Hepatopulmonary Syndrome in Children: A 20-Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome.

Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. Twenty patients were diagnosed with HPS between 1996 and 2016.

Cytotoxic T-lymphocyte therapy for post-transplant lymphoproliferative disorder after solid organ transplantation in children.

EBV-CTL immunotherapy targets EBV antigens expressed by tumor cells in PTLD. Data on outcome of EBV-CTL in pSOT patients are limited. The aim of the study is to describe our experience with allogeneic, third-party EBV-CTL for the treatment of PTLD in pSOT patients in a single tertiary center.

Current practices and experience of transition of young people on long term home parenteral nutrition (PN) to adult services - A perspective from specialist centres.

Background: There has been an estimated fivefold increase in the number of children receiving parenteral nutrition (PN) at home in the past 10 years with some children approaching the age when they should be referred to adult services whilst still on treatment. Models of care for moving young people onto adult providers of PN at home are not yet well developed, and transition is a potentially dangerous time for young people with complex health needs.

Methods: A questionnaire to ascertain current experience and practices of transition in the context of home PN services was dispatched to 170 consultant gastroenterologists who were members of the British Association of Parenteral and Enteral Nutrition (BAPEN) and also to all 40 members of the Nutrition and IF working group of the British Society of Paediatric Gastroenterology and Nutrition (BSPGHAN).

Total Parenteral Nutrition-Induced Cholestasis: Prevention and Management.

When cholestasis occurs in patients receiving total parenteral nutrition, it is the result of many pathogenic pathways converging on the hepatic acinus. The result may be a temporary rise in liver function tests. The resulting fibrosis, portal hypertension, and jaundice are hallmarks of type 3 intestinal-associated liver disease to which children are more susceptible than adults.

Selenium homocholic acid taurocholate scanning, selenium-75-labeled bile acid, a novel method for testing the function of the terminal ileum in small bowel transplant recipients: a pilot study.

Introduction: The terminal ileum (TI) is important for the active reabsorption of bile salts and is the site of allograft rejection; disruption of enterohepatic circulation (EHC) may give insights to inflammatory and other physiologic processes at the TI.

Subjects And Methods: Four children aged 5 to 12 years who had received small bowel transplantation (SBTx), 3 recovering from post-transplant lymphoproliferative disease (PTLD) and 1 with acute rejection, were studied. Two of the 4 had stoma reversal.

Social circumstances and medical complications in children with intestinal failure.

Unlabelled: Although most children discharged on home parenteral nutrition (HPN) will achieve enteral autonomy, some remain parenteral nutrition dependent; those who develop life-threatening complications may undergo small bowel transplantation (SBTx). The aim of this study was to investigate the relationship between social circumstances, compliance and complications.

Subjects And Methods: An observational study in 2008-2012 on 64 children (34 HPN, 30 SBTx) from three units (two regional gastroenterology; one transplant).

A comparison of two validated scores for estimating risk of mortality of children with intestinal failure associated liver disease and those with liver disease awaiting transplantation.

Background And Aims: To evaluate risk of mortality in children with intestinal failure associated liver disease (IFALD) compared with other liver disease using two validated scores.

Methods: Sixty-seven children listed for transplant were studied: cholestatic liver disease (CLDn23); liver disease secondary to other processes (LDsec n11); (IFALDn22), acute liver failure (ALFn11). Paediatric Hepatology Score (PHD) score and Pediatric end-stage liver disease score (PELD) were evaluated by Receiver Operating Curves (ROC), proportional hazards regression.

Combined liver-intestine grafts compared with isolated intestinal transplantation in children: a single-center experience.

Background: Intestinal transplantation is known to be associated with a high risk of early complications and mortality.

Methods: We analyzed prospective data of 51 primary small bowel transplantations from December 1999 to August 2009 and identified perioperative factors that impact on early mortality (≤6 months after transplantation) after isolated intestinal (IITx; n=12) and combined liver-intestinal transplantation (CLITx group; n=39).

Results: Ten patients died during the first 6 months after transplantation, all of them in CLITx group (n=10/51, 19%).

Trends in paediatric home parenteral nutrition and implications for service development.

Background & Aims: The demand for home parenteral nutrition (HPN) services for children has risen over the past 20 years. To plan a strategy for delivery of care at regional level, we carried out a national point prevalence survey of HPN during February 2010.

Methods: Paediatric gastroenterologists in 33 hospitals known to have provided HPN services were identified from their membership of the British Society of Paediatric Gastroenterology, Hepatology and Nutrition and participation in the British Intestinal Failure Survey (BIFS).

Surgical complications after intestinal transplantation in infants and children--UK experience.

Unlabelled: Surgical complications have a significant impact on morbidity and mortality following intestinal transplantation (ITx). Birmingham Children's Hospital commenced intestinal transplantation in 1993 and the following surgical strategies evolved: (a) pretransplant abdominal tissue expanders, 1998; (b) combined en-bloc reduced liver and intestinal transplantation (CRLITx), 1998; (c) staged abdominal closure, 2001; (d) preservation of graft duodenal artery, 2005.

Aim: An internal audit was performed to document the surgical complications after ITx and to evaluate strategies in the management and prevention of complications.

Pseudomonoenergetic x-ray diffraction measurements using balanced filters for coherent-scatter computed tomography.

Coherent-scatter computed tomography (CSCT) is a method of "composition" imaging based on measurements of diffraction patterns from tissues. Use of an x-ray tube degrades scatter pattern angular resolution due to the x-ray spectral width, making it difficult to uniquely identify some materials. The use of two transmission filters with similar atomic numbers (balanced "Ross filters") to generate pseudomonoenergetic scatter patterns is described as it applies to CSCT.

Impact of change in the United Kingdom pediatric donor organ allocation policy for intestinal transplantation.

Background: Graft availability remains a problem in pediatric intestinal transplantation (IT), with most children waiting being less than 10 kg weight. In November 2004, wait-listed children in the United Kingdom were prioritized nationally to receive pediatric donor organs to improve donor availability for IT. We aimed to evaluate the impact of this change on the recipient population.

Update on intestinal rehabilitation after intestinal transplantation.

Purpose Of Review: The past decade has seen improvements in patient selection and a greater range of surgical procedures available to patients with intestinal failure, and this, combined with more effective immunosuppressive regimens, has transformed survival after intestinal transplantation (ITx). Much more is known about optimizing rehabilitation of the engrafted intestine in the first 12 months after transplant, but it is also becoming apparent that there are some long-term health issues to consider.

Recent Findings: The key issues in rehabilitation of ITx are the immunogenic nature of the intestinal graft, which means a higher risk of rejection and necessitates relatively high levels of immune suppression that promotes infections, including Epstein-Barr virus-driven lymphoma, viral gastroenteritis and renal impairment; and those related to the specifics of an intestinal allograft (nutritional support, chylous ascites, growth, food allergy, fat soluble vitamin deficiencies, metabolic bone disease).