De novo cholangiocarcinoma after liver transplantation in a pediatric patient

Channabasappa N, Johnson‐Welch S, Mittal N. De novo cholangiocarcinoma after liver transplantation in a pediatric patient
Pediatr Transplantation 2010: 14:E110–E114. © 2009 John Wiley & Sons A/S. Abstract: To date, no child has been reported to develop de novo CCA after liver transplantation although patients with transplants have a significantly higher risk of malignancy than the general population. CCA is extremely rare in the pediatric age group, seen mostly in patients with a history of choledochal cysts, Caroli’s disease, or PSC. We report the first case of pediatric de novo CCA in the liver allograft 12 yr after liver transplantation.     

Towards minimizing immunosuppression in pediatric liver transplant recipients

Abstract:  Immunosuppression regimens after liver transplantation focus mainly on preventing rejection and subsequent graft loss. However, in children, morbidity and mortality rates from infections exceed those from rejection after transplant, and immunosuppression can hinder growth, renal function, and graft tolerance. We hypothesized that early steroid withdrawal, with a primary aim of TAC monotherapy would yield no penalty in terms of rejection and graft loss, while reducing risks of infection and maximizing growth. We prospectively evaluated 64 consecutive pediatric liver transplant recipients. One yr patient/graft survival was 93/90%, respectively. At one yr post-transplant, 75.4% of patients were on TAC monotherapy. No deaths or graft losses were caused by infection. Sixty-one percent of patients had at least one episode of rejection, most within three months following transplant and 3.8% were treated for chronic rejection. One non-compliant adolescent died from chronic rejection. CMV, EBV, and lymphoproliferative disease rates were 3.1%, 5.3%, 1.8%, respectively. Pretransplant and one yr post-transplant glomerular filtration rates were unchanged. One yr improved catch-up growth was observed. We conclude that immunosuppression minimization after pediatric liver transplant yields no serious complications from rejection, and might confer advantages with respect to infection, renal function, growth, and is deserving of wider application and study.     

Fluid balance in pediatric postoperative liver transplant recipients

Background

Positive fluid balance (FB) is associated with poor outcomes in critically ill children but has not been studied in pediatric liver transplant (LT) recipients. Our goal is to investigate the relationship between postoperative FB and outcomes in pediatric LT recipients.

Methods

We performed a retrospective cohort study of first-time pediatric LT recipients at a quaternary care children's hospital. Patients were stratified into three groups based on their FB in the first 72 h postoperatively: <10%, 10–20%, and > 20%. Outcomes were pediatric intensive care unit (PICU) and hospital length of stay, ventilator-free days (VFD) at 28 days, day 3 severe acute kidney injury, and postoperative complications. Multivariate analyses were adjusted for age, preoperative admission status, and Pediatric Risk of Mortality (PRISM)-III score.

Results

We included 129 patients with median PRISM-III score of 9 (interquartile range, IQR 7–15) and calculated Pediatric End-stage Liver Disease score of 15 (IQR 2–23). A total of 37 patients (28.7%) had 10–20% FB, and 26 (20.2%) had >20% FB. Greater than 20% FB was associated with an increased likelihood of an additional PICU day (adjusted incident rate ratio [aIRR] 1.62, 95% CI: 1.18–2.24), an additional hospital day (aIRR 1.39, 95% CI: 1.10–1.77), and lower likelihood of a VFD at 28 days (aIRR 0.85, 95% CI: 0.74–0.97). There were no differences between groups in the likelihood of postoperative complications.

Conclusions

In pediatric LT recipients, >20% FB at 72 h postoperatively is associated with increased morbidities, independent of age and severity of illness. Additional studies are needed to explore the impact of fluid management strategies on outcomes.     

Gastroenteritis caused by Edwardsiella tarda in a pediatric renal transplant recipient

Abstract:  Edwardsiella tarda , a member of the family Enterobacteriaceae , is a Gram-negative bacillus that is most often pathogenic in aquatic environments. Human infections with Edwardsiella are rare, with most occurring in immunocompromised or immunosuppressed hosts. Reported infections include meningitis, cholecystitis, endocarditis, osteomyelitis, soft tissue infections, bacteremia and septicemia, dysentery, and gastroenteritis. This report describes a case of E. tarda gastroenteritis in a renal transplant patient receiving immunosuppressive therapy. The epidemiology, diagnosis, clinical presentation, and treatment options pertaining to E. tarda infections are examined.     

Immunosuppression in the pediatric transplant recipient

The field of pediatric solid-organ transplantation has significantly evolved since its beginnings in the early 20^th century. As advancements have led to the development of innovative surgical techniques and novel medication regimens, transplantation has now become a routine practice leading to an increase in the rates of organ recipients worldwide.The care of pediatric solid-organ transplant recipients differs from adults in several areas not only due to technically challenging surgeries, but mostly due to the complexity of their immunosuppression management. Although there is large variation of pediatric immunosuppression regimens worldwide, the use of calcineurin inhibitors, either tacrolimus or cyclosporine, still forms the backbone of immunosuppression regimens after solid-organ transplantation. Both medications are relatively well tolerated but are known to have long-term side effects, especially nephrotoxicity and neurotoxicity. The goal of care in long-term pediatric survivors of solid-organ transplant now aims to safely minimize exposure to immunosuppression and to achieve long-term graft tolerance.     

Transition from hospital to home following pediatric solid organ transplant: Qualitative findings of parent experience

Transplant providers are challenged to determine appropriate interventions for patients and families due to limited published research regarding the context of the post‐discharge experience from the perspective of parents of transplanted children. The purpose of this study is to describe the parent perspective of the transition from hospital to home following their child's solid organ transplant. Within a mixed‐methods design, 37 parents of pediatric heart, kidney, and liver transplant recipients from three pediatric hospitals responded to qualitative interview questions on the day of hospital discharge and three wk following hospital discharge. Insight to the discharge preparation process revealed necessary education components. Post‐discharge themes were identified for coping, knowledge, and adherence. The parents' responses provide awareness as to specific stressors and concerns parents are faced with when their child is discharged from the hospital after solid organ transplant and opportunities for ways the transplant team can provide support.     

Medical management of pediatric inflammatory bowel disease

Inflammatory bowel diseases (IBD) are chronic autoimmune conditions of the gut affecting both pediatric and adult patients. Medical therapy is often successful at inducing and maintaining remission and preventing disease complications. The mainstays of treatment are medications and other therapies that reduce inflammation and suppress the overactive immune system. Here we review current medical therapies for pediatric IBD, discuss future therapeutics, and present current treatment goals and approaches.     

Herpes simplex colitis in a child with combined liver and small bowel transplant

Herpes simplex virus (HSV) has been a rare cause of gastrointestinal (GI) infection, especially in immunocompromised patients. A variety of GI sites may be involved; however, only three reported cases of HSV colitis have been documented in the literature. To our knowledge, this is the first report of HSV colitis in a small bowel transplant recipient.     

An update on immunizations before and after transplantation in the pediatric solid organ transplant recipient

Vaccination offers a unique opportunity to decrease the burden of infectious complications following solid organ transplantation. In this paper we review the current guidelines for routine immunizations before and after solid organ transplantation, including the recent updates and changes to recommendations for certain vaccines. We also address the issue of waning immunity in solid organ transplant recipients and discuss the current data on vaccinating this patient population with live vaccines after transplantation.     

De novo phyllodes tumor in an adolescent female after liver transplantation

Cheng F, Qin J‐J, Yu M‐N, Zhang F, Li X‐C, Sun B‐C, Kong L‐B, Wang X‐H. De novo phyllodes tumor in an adolescent female after liver transplantation.
Pediatr Transplantation 2011: 15:E12–E14. © 2009 John Wiley & Sons A/S. Abstract: Phyllodes tumor of the breast is a rare disease constituting 0.3–0.9% of all breast neoplasms. Occurring mainly in females aged 35 to 55 yr, the disease is especially rare among adolescent females. There is no published literature about de novo phyllodes tumor after liver transplantation. Here we describe a case of de novo phyllodes tumors in an adolescent female after liver transplantation from a living donor for Wilson disease.     

Characteristics of diabetes after pediatric liver transplant

Greig F, Rapaport R, Klein G, Akler G, Annunziato R, Miloh T, Arnon R, Florman S, Kerkar N. Characteristics of diabetes after pediatric liver transplant. Abstract: Studies of DALT in pediatric recipients describe incidence and risk factors, but diagnostic criteria varied. This study reports characteristics and course of pediatric DALT by established diabetes criteria. Retrospective chart review of pediatric LT recipients evaluated for hyperglycemia (1/1/1997–12/30/2009) and matched, non‐diabetic controls. DALT: random blood glucose >11.1 mm , ≥2 times, with insulin treatment. DALT diagnosed in 8.0% (24/300) included 7/24 (29.2%) with severe hyperglycemia (>27.7 mm ), ketoacidosis in 2/24 (8.3%). At diagnosis, age was ≥11 yr old in 22/24 (91.7%); body mass was lean (BMIz ?0.2 ± 1.5). Mean blood glucose was 24.6 mm with negative diabetes autoantibodies (19/19) and elevated C‐peptide (2.3 nm ). DALT onset median 5.0 months included 29.1% >12 months. Insulin duration median 4.6 months included 41.7% >6 months. DALT resolved in 83.3% over 4.9 (0.9–9.1) yr. DALT differed from controls by increased preceding rejections, prednisolone dose, tacrolimus level, and triple immunosuppression (all p < 0.01). In conclusion, pediatric DALT occurred in non‐obese adolescents with insulin resistance, distinct from diabetes types 1 or 2. DALT was associated with preceding rejection and increased immunosuppression. Blood glucose monitoring, especially during increased immunosuppression following LT, could allow early diagnosis and reduce morbidity.     

Clinicopathologic characteristics of de novo nodular regenerative hyperplasia in pediatric liver transplant

Liver NRH is seen in all patients age; however, more frequently in those over the age of 60 years and associated with multiple systemic diseases. In liver allograft recipients, the development of DnNRH has been linked with the use of azathioprine or vascular abnormalities. We present the clinicopathologic characteristics of 17 pediatric patients who underwent liver transplantation and subsequently developed DnNRH. The patients were divided into early and late onset depending if DnNRH was diagnosed within or beyond 4 years after transplant. Eight patients (47%) presented as early onset, of which two had normal ultrasound at time of diagnosis. One patient (12.5%) with early onset lost the graft secondary to DnNRH. Nine patients (53%) presented as late onset, of which two (22%) had normal ultrasound at time of diagnosis. Two patients (25%) of the late onset lost their graft secondary to chronic rejection and DnNRH. Two patients (12%) died secondary to cytomegalovirus pneumonitis and pancolitis. Furthermore, both groups presented with symptoms differing from the adult population in prior studies and were not associated with the use of azathioprine or vascular abnormalities. Interestingly, episodes of acute cellular rejection were more common in the early‐onset group compared to the late‐onset group. In conclusion, DnNRH in the pediatric age group has a different clinical presentation, possibly reflecting a different pathogenesis compared to the adult population.     

Adenoviral disease in pediatric solid organ transplant recipients

Adenoviral disease in pediatric SOT recipients is emerging as an important viral pathogen, with serious consequences impacting morbidity, mortality and graft survival. The optimal diagnostic techniques, as well as therapy have yet to be established. This article reviews the current epidemiology of AdV in orthotopic liver, intestinal, cardiothoracic and renal transplant recipients. Issues related to diagnosis, notably the use of newer non-culture based viral detection methods and therapy, including anti-adenoviral agents and adoptive immunotherapy are discussed.     

De novo amyloidosis in a renal transplant patient

Systemic AA amyloidosis is a serious complication of many chronic inflammatory disorders and chronic infections. Renal involvement is seen in the majority of the patients and can lead to end‐stage renal disease. Renal transplantation can be performed in these patients; however, amyloidosis can recur in the transplanted kidneys. On the other hand, de novo AA amyloidosis in renal transplant patients has been rarely reported. We report a 17‐yr‐old patient with end‐stage renal disease due to genitourinary anomalies who developed recurrent pyelonephritis after transplantation. Three yr after transplantation, renal biopsy was performed for proteinuria and AA amyloidosis was identified in the renal allograft. Although rare, chronic infections might cause de novo amyloidosis in renal transplant patients. Therefore, amyloidosis should be kept in mind in those types of patients who present with proteinuria.     

Strategies for the prevention of cytomegalovirus infection and disease in pediatric liver transplantation recipients

Cytomegalovirus (CMV) is the most common opportunistic infection following solid organ transplantation. Prevention and management of CMV infection has assumed a higher priority as transplantation has become a frequent treatment for many congenital and acquired disorders, as more potent immunosuppressive agents have become available, new molecular and virologic assays to detect CMV have made their way from research to clinical laboratories and new antiviral medications and biologics have been developed. Management strategies are diverse; however, there are little or no data from large controlled pediatric trials demonstrating the superiority of any particular approach. This review outlines the current strategies employed to prevent CMV infection and disease and summarizes the strengths and limitations of each regimen to guide clinicians in the selection of the optimal preventative approach.     

Post‐transplant malignancies in pediatric organ transplant recipients

The majority of cancer diagnoses in pediatric solid organ transplant recipients (SOTRs) are post‐transplantation lymphoproliferative disorders (PTLD) or skin cancers. However, pediatric SOTRs are also at significantly elevated risk for multiple other solid and hematological cancers. The risks of specific cancers vary by transplanted organ, underlying disease, and immunosuppression factors. More than one‐quarter of pediatric SOTRs develop cancer within 30 years of transplantation and their risk of solid cancer is 14 times greater than the general population. Pediatric SOTRs are at significantly higher risk of cancer‐associated death. Improving patient survival among pediatric SOTRs puts them at risk of adult epithelial cancers associated with environmental carcinogenic exposures. Vaccination against oncogenic viruses and avoidance of excessive immunosuppression may reduce the risk of solid cancers following transplantation. Patient and family education regarding photoprotection is an essential component of skin cancer prevention. There is significant variability in cancer screening recommendations for SOTRs and general population approaches are typically not validated for transplant populations. An individualized approach to cancer screening should be developed based on estimated cancer risk, patient life expectancy, and screening test performance.