Early Graft Loss With Suspected Seventh-Day Syndrome Following Pediatric Liver Transplantation.

INTRODUCTION: Allograft dysfunction within the first week posttransplant is an uncommon but known complication following liver transplantation. Seventh-Day Syndrome (7DS) is a rare complication of allograft dysfunction following liver transplantation characterized by the rapid clinical deterioration of a formerly well-functioning allograft within the first week posttransplant. The etiology of 7DS is unknown, and treatment options remain limited. While cases of graft survival have been reported, the risk of mortality remains exceedingly high without urgent retransplantation. METHODS: Patient data was retrospectively analyzed and a literature review performed. RESULTS: We present a unique case of split liver transplantation into two pediatric recipients in which one recipient developed rapidly progressive graft failure approximately 1 week postoperatively requiring urgent retransplantation while the other recipient had an unremarkable postoperative course. Upon clinical manifestation of progressive graft failure, the patient was treated with thymoglobulin, rituximab, intravenous immunoglobulin, and plasmapheresis. Despite this, the patient's clinical status continued to decline and she underwent retransplantation 11 days following her initial liver transplant. CONCLUSION: Seventh-Day Syndrome is a rare complication following liver transplantation that is associated with a high risk of morbidity and mortality. Our case adds to the limited literature on 7DS in children and is the first to report a comparative posttransplant clinical course in two recipients who received split grafts from the same donor.

Inflammatory Bowel Disease Presenting With Concurrent COVID-19 Multisystem Inflammatory Syndrome.

Coronavirus disease 2019 is associated with a postinfectious multisystem inflammatory syndrome in children (MIS-C). This syndrome is marked by cytokine storm and multiorgan dysfunction, often affecting the gastrointestinal tract, the heart, and the hematopoietic system. We describe the case of a 16-year-old boy with an initial presentation of severe inflammatory bowel disease and concurrent MIS-C. He presented with abdominal pain, diarrhea, and hematochezia and met criteria for the systemic inflammatory response syndrome. Laboratory inflammatory profiling revealed markedly elevated ferritin, D-dimer, C-reactive protein, soluble interleukin 2, and interleukin 6 levels. Endoscopy and colonoscopy revealed severe active gastroduodenitis, patchy colitis, and a normal-appearing terminal ileum. The patient was treated with a combination of steroids, intravenous immunoglobulin, and infliximab, and his symptoms slowly resolved over a 3-week period. In this case, we describe coincident MIS-C with a remarkably severe and difficult-to-treat initial presentation of inflammatory bowel disease and highlight the need to investigate the effect of coronavirus disease 2019 and MIS-C on inflammatory disorders.

Nonalcoholic Fatty Liver Disease in Children.

Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease worldwide. It represents a spectrum of disease from simple hepatic steatosis to steatohepatitis that may develop into progressive hepatic fibrosis and even cirrhosis. NAFLD is the most rapidly increasing indication for liver transplantation in adults. In children, the incidence of NAFLD has also increased over the past decade. Although the majority of children with NAFLD are overweight or obese, there is an increasing subset of children with normal body mass index with so-called lean NAFLD. NAFLD in children is associated with several extrahepatic manifestations, including hyperlipidemia, insulin resistance, and obstructive sleep apnea. The pathogenesis of NAFLD in children involves a multifactorial interaction among genetics, in utero exposures, early childhood exposures, and ongoing nutritional exposures. Although there are some similarities between pediatric NAFLD and adult NAFLD, liver biopsies in children show histologic differences between the two. The current standard-of-care treatment of NAFLD in children is lifestyle change to decrease caloric intake and increase physical activity. There are no medications currently approved for the treatment of NAFLD in children. This article aims to summarize the current understanding of pediatric NAFLD and future directions for intervention and therapeutic aims.

Rapid Progression of Acute Pancreatitis to Acute Recurrent Pancreatitis in Children.

OBJECTIVE: Research is lacking on the natural history of acute pancreatitis (AP) progression to acute recurrent pancreatitis (ARP). The aim of this project was to study the progression from AP to ARP among pediatric patients with pancreatitis to better understand the presentation and natural history of pancreatitis. METHODS: Patients presenting with AP were included in a prospective database in Research Electronic Data Capture. We enrolled 115 patients with AP from March 2013 to November 2016. Physicians completed surveys regarding clinical data for patients with first attack of AP. Patients were followed prospectively, with data on progression entered when patients presented with ARP. RESULTS: The most common etiologies for the first attack of AP were idiopathic (31%), toxic/drug-related (23%), and biliary/gallstone (18%). Twenty of the 115 patients (17%) developed ARP during the follow-up period. Seventy percent (14/20) of patients with ARP progressed from AP to ARP within 5 months from first diagnosis. A comparison of patients who rapidly progressed to ARP within 3 months (n = 12) to those followed for >3 months without progression in 3 months (n = 97) revealed associations with a higher weight percentile for age (P = 0.045), male sex (P = 0.03), and presence of pancreatic necrosis during first AP attack (P = 0.004). Progression to ARP significantly differed by etiology group with genetics having the highest risk for ARP progression over time and patients with gallstone/biliary, viral/systemic, and obstructive (nongallstone) having the lowest risk for ARP progression over time (P = 0.02). CONCLUSIONS: Most patients who progressed from AP to ARP progressed within 5 months. The presence of a higher weight percentile for age, male sex, and pancreatic necrosis during the first AP attack are associated with rapid progression to ARP.