Infusion Reactions to Infliximab in Pediatric Patients with Inflammatory Bowel Disease.

Infliximab (IFX) is a recombinant DNA-derived chimeric IgG monoclonal antibody protein that inhibits tumor necrosis factor alpha (TNF-α). IFX, like other agents derived from foreign proteins, can cause infusion reactions both during and after the infusion. The incidence of infusion reactions ranges between 0% and 15% in pediatric patients.

Prenatal treatment of ornithine transcarbamylase deficiency.

Purpose Of Study: Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death. Long-term prognosis despite optimal pharmacological and dietary therapy is still poor. The combination of intravenous sodium phenylacetate and sodium benzoate (Ammonul®) can eliminate nitrogen waste independent of the urea cycle.

Mass cytometry reveals a distinct immunoprofile of operational tolerance in pediatric liver transplantation.

Long-term IS in transplant patients has significant morbidity, poorer quality of life, and substantial economic costs. TOL, defined as graft acceptance without functional impairment in the absence of IS, has been achieved in some pediatric LT recipients. Using mass cytometry, peripheral blood immunotyping was performed to characterize differences between tolerant patients and patients who are stable on single-agent IS.

Treatment of recurrent posttransplant lymphoproliferative disorder of the central nervous system with high-dose methotrexate.

Posttransplant lymphoproliferative disorder (PTLD) is a frequent complication of intestinal transplantation and is associated with a poor prognosis. There is currently no consensus on optimal therapy. Recurrent PTLD involving the central nervous system (CNS) represents a particularly difficult therapeutic challenge.

Successful treatment of recurrent primary sclerosing cholangitis after orthotopic liver transplantation with oral vancomycin.

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60-70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%.

Toll-like receptor 4 contributes to small intestine allograft rejection.

Background: Although outcomes for small intestine transplantation (SIT) have improved in recent years, allograft rejection rates remain among the highest of solid organ grafts. The high load of commensal bacteria in the small intestine may contribute through activation of the toll-like receptor (TLR) pathway. In this study, we examine the participation of TLR4 in acute allograft rejection in an orthotopic mouse model of SIT.

Factors affecting survival to intestinal transplantation in the very young pediatric patient.

Background: Very young pediatric patients awaiting intestinal transplantation have a high mortality rate due to long waiting times, scarcity of appropriate size donor organs, and mortality due to sepsis and liver failure. To investigate specific risk factors impacting survival to intestinal transplantation, we performed a 4-year institutional retrospective study comparing children who received grafts by age 18 months with children 18 months or younger who died while on the waiting list.

Patients And Methods: Twelve children comprised the transplanted group and had the underlying diagnoses: necrotizing enterocolitis, gastroschisis, Hirschsprung's disease, and omphalocele.

Outcomes of transplantation in children with primary hepatic malignancy.

HBL and HCC are the most common hepatic malignancies in children. The role of OLT in children with HCC is still a matter of debate. The aim of this study was to review our experience of OLT for HCC.

Risk factors for small bowel bacterial overgrowth in cystic fibrosis.

Objectives: The purpose of this study was to determine the prevalence of small bowel bacterial overgrowth in patients with pancreatic-insufficient cystic fibrosis (CF) compared with age-matched controls and to identify potential risk factors for small bowel bacterial overgrowth.

Patients And Methods: Fifty patients, 25 pancreatic-insufficient CF study patients (mean age, 17 y) and 25 gastrointestinal clinic control patients (mean age, 15 y), completed a glucose-hydrogen breath test after an overnight fast. Study patients completed a quality-of-life questionnaire modified from the Cystic Fibrosis Questionnaire.

Management of esophageal strictures in children with recessive dystrophic epidermolysis bullosa.

Background: Recessive dystrophic epidermolysis bullosa is a rare, genetically transmitted skin disorder characterized by blister formation and scarring in response to minor trauma. One of the most debilitating features of the disease is the development of esophageal strictures, which produces profound dysphagia, exacerbating an already highly compromised nutritional status common to these patients. Due to the extreme fragility of epithelial surfaces, the optimal therapeutic approach to esophageal strictures in this setting has not been established.