Intestinal Transplantation in Pediatric Patients

2002 ◽  
Vol 12 (2) ◽  
pp. 97-115 ◽  
Author(s):  
Beverly Kosmach Park

Intestine transplantation has evolved into a feasible alternative for children with permanent intestinal failure and life-threatening complications related to total parenteral nutrition. Although the first transplantations were done nearly 40 years ago, long-term survival has only been achieved in the last decade. Nearly 700 intestinal transplantations have been performed internationally since 1985, with an overall patient survival of greater than 50%. Improvements in patient selection, medical management, and assessment and treatment for rejection and infection have contributed to the increased survival. This article will discuss current results and medical management strategies for this innovative type of transplantation for children with end-stage short gut syndrome.

2005 ◽  
Vol 15 (1) ◽  
pp. 60-64 ◽  
Author(s):  
Bridget Flynn ◽  
Beverly Kosmach Park ◽  
Geoffrey Bond ◽  
William McGhee ◽  
George Mazariegos ◽  
...  

Intestinal, combined liver-intestinal, and multivisceral transplantation are now considered the standard of care for children and adults with permanent intestinal failure. Early attempts at intestinal transplantation were discouraging because of the high incidence of technical complications, rejection, and infection. Advances in the field of transplantation, including the introduction of tacrolimus, improved surgical techniques, and improvements in postoperative care, have led to a renewed interest in intestinal transplantation since 1990. The most significant achievement, however, has been the effective control of rejection and life-threatening infections. This article focuses on the experience to date of innovative strategies that induce lymphocyte depletion and reduction in the incidence of rejection. In this setting, low-maintenance immunosuppression is clinically achievable with an acceptable rate of allograft rejection. Subsequently, the long-term complications of immunosuppression are significantly reduced with achievement of better long-term survival, and an overall improvement in the quality of life.


2017 ◽  
Vol 2017 ◽  
pp. 1-10 ◽  
Author(s):  
Genevieve Huard ◽  
Thomas Schiano ◽  
Jang Moon ◽  
Kishore Iyer

Intestinal transplantation (ITx) is indicated in patients with irreversible intestinal failure (IF) and life-threatening complications related to total parenteral nutrition (TPN). ITx can be classified into three main types. Isolated intestinal transplantation (IITx), that is, transplantation of the jejunoileum, is indicated in patients with preserved liver function. Combined liver-intestine transplantation (L-ITx), that is, transplantation of the liver and the jejunoileum, is indicated in patients with liver failure related to TPN. Thus, patients with cirrhosis or advanced fibrosis should receive a combined allograft, while patients with lower grades of liver fibrosis can usually safely undergo ITx. Reflecting their degree of sickness, the waitlist mortality rate and the early posttransplant outcomes of patients receiving L-ITx are worse than IITx. However, L-ITx is associated with better long-term graft and patient survival. Multivisceral transplantation (MVTx), that is, transplantation of the organs dependent on the celiac axis and superior mesenteric artery, can be classified into full MVTx if it includes the liver and modified MVTx if it does not. The most common indications for MVTx are extensive portomesenteric thrombosis and diffuse gastrointestinal pathology such as motility disorders and polyposis syndrome. Every patient with IF should undergo a multidisciplinary evaluation by an experienced ITx team.


2019 ◽  
Vol 90 (9) ◽  
pp. 1039-1045 ◽  
Author(s):  
Emma Foster ◽  
Patrick Carney ◽  
Danny Liew ◽  
Zanfina Ademi ◽  
Terry O’Brien ◽  
...  

Almost 10% of people will experience at least one seizure over a lifetime. Although common, first seizures are serious events and warrant careful assessment and management. First seizures may be provoked by acute or remote symptomatic factors including life-threatening metabolic derangements, drug toxicity or structural brain lesions. An unprovoked first seizure may herald the onset of epilepsy and may be accompanied by medical and psychiatric illnesses. Accidents, injuries and death associated with first seizures are likely under-reported. The cognitive and emotional impact of first seizures is often neglected. Evaluation of a patient presenting with a first seizure requires careful history-taking and early specialist assessment, however optimal management strategies have not been extensively investigated. Further, advances in technology and the role of eHealth interventions such as telemedicine may be of value in the care of patients who have experienced a first seizure. This article reviews the impact and implications of first seizures beyond the scope provided in current guidelines which tend to focus on assessment and management. It examines the effect of first seizures on the well-being of patients; assesses morbidity and premature mortality in first seizures and discusses current and future directions to optimise safety and health of people with first seizures, with a focus on adult patients. Recognition of these issues is essential to provide adequate care for people with first seizures.


2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
Bassam Abu-Wasel ◽  
Michele Molinari

IFALD is a common and potentially life-threatening condition for patients with SBS requiring long-term PN. There exists the potential for decreasing its incidence by optimizing the composition and the rate of infusion of parenteral solutions, by advocating a multidisciplinary approach, and by early referral for intestinal-liver transplantation to ensure long-term survival of patients with SBS.


2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Daniella Levy Erez ◽  
Irit Krause ◽  
Amit Dagan ◽  
Roxana Cleper ◽  
Yafa Falush ◽  
...  

Objective.Owing to a shortage of kidney donors in Israel, children with end-stage renal disease (ESRD) may stay on maintenance dialysis for a considerable time, placing them at a significant risk. The aim of this study was to understand the causes of mortality.Study Design. Clinical data were collected retrospectively from the files of children on chronic dialysis (>3 months) during the years 1995–2013 at a single pediatric medical center.Results.110 patients were enrolled in the study. Mean age was10.7±5.27 yrs. (range: 1 month–24 yrs). Forty-five children (42%) had dysplastic kidneys and 19 (17.5%) had focal segmental glomerulosclerosis. Twenty-five (22.7%) received peritoneal dialysis, 59 (53.6%) hemodialysis, and 6 (23.6%) both modalities sequentially. Median dialysis duration was 1.46 years (range: 0.25–17.54 years). Mean follow-up was13.5±5.84 yrs. Seventy-nine patients (71.8%) underwent successful transplantation, 10 (11.2%) had graft failure, and 8 (7.3%) continued dialysis without transplantation. Twelve patients (10.9%) died: 8 of dialysis-associated complications and 4 of their primary illness. The 5-year survival rate was 84%: 90%for patients older than 5 years and 61%for younger patients.Conclusions.Chronic dialysis is a suitable temporary option for children awaiting renal transplantation. Although overall long-term survival rate is high, very young children are at high risk for life-threatening dialysis-associated complications.


2003 ◽  
Vol 62 (3) ◽  
pp. 727-737 ◽  
Author(s):  
Kareem Abu-Elmagd ◽  
Geoffrey Bond

Despite the reported high survival with total parenteral nutrition (TPN) therapy for patients with intestinal failure, a considerable number of patients do not escape the potential risks of TPN-associated complications, including hepatic failure, vanishing of central venous access and line sepsis. Thus, intestinal, liver-intestinal and multivisceral transplantation have recently emerged to rescue those who can no longer be maintained on TPN. Before this development, and for nearly three decades, small-bowel transplantation was plagued with uncontrolled rejection, graft v. host disease and fatal infection. These barriers stemmed from the large gut lymphoid mass and heavy microbial load contained in the intestinal lumen. The recent improvement in survival after the clinical introduction of tacrolimus with achievement of full enteric nutritional autonomy qualified the procedure by the US Health Care Financing Administration as the standard of care for patients with intestinal and TPN failure. The decision was supported by a decade of clinical experience with cumulative improvement in patient and graft survival. In addition, the introduction of new effective immunoprophylactic agents and novel therapeutic approaches has contributed to a further increase in the therapeutic advantages of the procedure. The present review article outlines the current clinical practice of intestinal transplantation and defines new management strategies with the aim of raising the level of the procedure to be a better alternative therapy for TPN-dependent patients.


2020 ◽  
pp. 112972982096197
Author(s):  
Fungai Dengu ◽  
James Hunter ◽  
Georgios Vrakas ◽  
James Gilbert

Intestinal failure (IF) patients are dependent on central venous access to receive parenteral nutrition. Longstanding central venous catheters are associated with life-threatening complications including infections and thromboses resulting in multiple line exchanges and the development ofprogressive central venous stenosis or occlusion. The Haemodialysis Reliable Outflow (HeRO) graft is an arterio-venous device that has been successfully used in haemodialysis patients with ‘end-stage vascular access’. We describe a case series of HeRO graft use in patients with IF and end-stage vascular access. Four HeRO grafts were inserted into IF patients with end-stage vascular access to facilitate or support intestinal transplantation. In all patients the HeRO facilitated immediate vascular access, supporting different combinations of parenteral nutrition, intravenous medications, fluids or renal replacement therapy with no bloodstream infections. In a highly complex group of IF patients with central venous stenosis/occlusion limiting conventional venous access or at risk of life-threatening catheter-related complications, a HeRO® graft can be a feasible alternative.


2013 ◽  
Vol 29 (11) ◽  
pp. 1115-1118 ◽  
Author(s):  
Motoshi Wada ◽  
Kotaro Nishi ◽  
Megumi Nakamura ◽  
Hironori Kudo ◽  
Satoshi Yamaki ◽  
...  

2019 ◽  
Vol 11 (1) ◽  
pp. 34-39 ◽  
Author(s):  
Jeremy Mark Woodward ◽  
Dunecan Massey ◽  
Lisa Sharkey

Intestinal failure-associated liver disease (IFALD) often presents in adults unexpectedly with advanced disease. Non-invasive tests can be falsely reassuring. Patients with ‘ultrashort’ intestine (<20 cm) ending in a stoma are at particular risk of developing IFALD, which may occur rapidly. Recent experience and studies suggest that IFALD can be reversed by isolated intestine transplant occurring before the development of high grade fibrosis or cirrhosis. Post-transplant survival is superior for isolated intestinal grafts compared with liver containing intestinal grafts; waiting time and waiting list mortality is higher for a combined graft, and donor liver supply is limited. Therefore, the aim of clinicians treating patients with intestinal failure should be to identify IFALD early and refer to an intestinal transplant centre while isolated intestine transplantation can be contemplated and before the liver disease has progressed to a stage requiring consideration of combined liver and intestinal transplantation.


Children ◽  
2018 ◽  
Vol 5 (7) ◽  
pp. 100 ◽  
Author(s):  
Nisha Mangalat ◽  
Jeffrey Teckman

The term, ‘intestinal failure’, signifies the inability of the body to meet the digestive, absorptive and nutritive needs of the body. As such, these individuals require parenteral nutrition (PN) for survival. The subsequent nutritional, medical and surgical facets to the care are complex. Improved care has resulted in decreased need for intestinal transplantation. This review will examine the unique etiologies and management strategies in pediatric patients with intestinal failure.


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