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Complications of cirrhotic portal hypertension (PHTN) in children are broad and include clinical manifestations ranging from variceal hemorrhage, hepatic encephalopathy (HE), ascites, spontaneous bacterial peritonitis (SBP), and hepatorenal syndrome (HRS) to less common conditions such as hepatopulmonary syndrome, portopulmonary hypertension, and cirrhotic cardiomyopathy. The approaches to the diagnosis and management of these complications have become standard of practice in adults with cirrhosis with many guidance statements available. However, there is limited literature on the diagnosis and management of these complications of PHTN in children with much of the current guidance available focused on variceal hemorrhage. The aim of this review is to summarize the current literature in adults who experience these complications of cirrhotic PHTN beyond variceal hemorrhage and present the available literature in children, with a focus on diagnosis, management, and liver transplant decision making in children with cirrhosis who develop ascites, SBP, HRS, HE, and cardiopulmonary complications.

Human adenoviruses typically cause self-limited respiratory, gastrointestinal, and conjunctival infections in healthy children. In late 2021 and early 2022, several previously healthy children were identified with acute hepatitis and human adenovirus viremia.We usedA total of 15 children were identified with acute hepatitis - 6 (40%) who had hepatitis with an identified cause and 9 (60%) who had hepatitis without a known cause. Eight (89%) of the patients with hepatitis of unknown cause tested positive for human adenovirus. These 8 patients plus 1 additional patient referred to this facility for follow-up were included in this case series (median age, 2 years 11 months; age range, 1 year 1 month to 6 years 5 months). Liver biopsies indicated mild-to-moderate active hepatitis in 6 children, some with and some without cholestasis, but did not show evidence of human adenovirus on immunohistochemical examination or electron microscopy. PCR testing of liver tissue for human adenovirus was positive in 3 children (50%). Sequencing of specimens from 5 children showed three distinct human adenovirus type 41 hexon variants. Two children underwent liver transplantation; all the others recovered with supportive care.Human adenovirus viremia was present in the majority of children with acute hepatitis of unknown cause admitted to Children's of Alabama from October 1, 2021, to February 28, 2022, but whether human adenovirus was causative remains unclear. Sequencing results suggest that if human adenovirus was causative, this was not an outbreak driven by a single strain. (Funded in part by the Centers for Disease Control and Prevention.).

Objective Neonatal acute liver failure (ALF) is a rare disease with high mortality for which no standard age-specific definition exists. To advance the understanding of neonatal ALF, we characterize the etiology, presenting features, treatment, and outcomes in infants within 1 month of life. Methods We performed a single-center 11-year retrospective chart review of neonates ≤30 days of life with ALF as defined by an INR of ≥2.0. Comparisons were made by etiology and survival with native liver (SNL). Estimated survival was performed using the Kaplan-Meier method. Results Forty-three patients met inclusion criteria for neonatal ALF. Etiologies included viral infection (23%), gestational alloimmune liver disease with neonatal hemochromatosis (GALD-NH) (21%), cardiac-associated ischemia (16%), other ischemia (14%), genetic etiologies (9%), Trisomy 21-associated myelodysplasia (TAM) (7%), hemophagocytic lymphohistiocytosis (HLH) (2%), and not identified (7%). Infants with viral etiologies had the highest alanine aminotransferase (ALT) at presentation (1179 IU/L, interquartile range [IQR] 683-1585 IU/L) in contrast to low levels in GALD-NH (23 IU/L, IQR 18-64 IU/L). Across all etiologies, only 33% were alive at 1 year. Overall median survival was 74 days; 17 days for viral infection and 74 days for GALD-NH. Among laboratory values at presentation, alpha-fetoprotein (AFP) was significantly higher in patients that survived with their native liver (P = 0.04). Conclusions Overall, outcome for neonatal ALF is poor. Although initial laboratory values can differentiate viral infection or GALD-NH, further studies are needed to identify laboratory parameters that predict SNL by etiology to ultimately improve patient outcomes.

OBJECTIVES Acute-on-chronic liver failure (ACLF) is well-studied in adults and characterized by decompensated cirrhosis, multi-organ failure, and early mortality. Studies of ACLF in children are limited. We sought to characterize the prevalence and clinical factors associated with pediatric ACLF (PACLF). METHODS A retrospective review of children 3 months to 18 years listed for liver transplantation and hospitalized for decompensated cirrhosis between January 2007 and December 2017 at a single pediatric hospital. Primary outcome was the development of PACLF, characterized as failure of at least 1 extrahepatic organ (mechanical ventilation, renal replacement therapy, vasoactive medications, grade III/IV hepatic encephalopathy). Characteristics were recorded for each hospitalization. RESULTS Sixty-six patients had 186 hospitalizations with mean age at admission 4.0 ± 5.6 years and diagnosis of biliary atresia (BA) in 65%. PACLF developed in 20 patients during 23 hospitalizations (12%) and respiratory failure was most common (17/23, 74%). Duration of intensive care unit stay, 13.1 ± 1.2 days versus 0.6 ± 0.6 days (P

In pediatric liver transplant recipients, hepatic artery thrombosis and portal vein thrombosis are major causes of acute graft failure and mortality within 30 days of transplantation. There is, however, a strong possibility of graft salvage if flow can be re-established to reduce ischemic injury. The current standard treatment is surgical revascularization, and if unsuccessful, retransplantation. Due to our success in treating these complications with catheter-directed therapies, we sought to summarize and publish the outcomes of all patients who experienced hepatic artery thrombosis or portal vein thrombosis within 30 days of liver transplantation.We conducted a retrospective cohort analysis of 27 pediatric liver transplant recipients who experienced hepatic artery thrombosis (n = 13), portal vein thrombosis (n = 9), or both (n = 5) between September 2012 and March 2021. We collected and tabulated data on the patients and therapies performed to treat them, including success rates, primary and secondary patency, and clinical outcomes.Among these patients, 6 were managed with anticoagulation and relisting for transplant and 21 had a primary revascularization attempt. Surgical recanalization was attempted in 7 patients of which 3 had successful recanalization (43%) and catheter-directed recanalization was attempted in 14 patients with 100% success in re-establishing blood flow to the graft. Additionally, patency was increased, and mortality was decreased in patients treated with catheter-directed recanalization compared to surgical revascularization or anticoagulation alone.This data illustrates the need to further investigate catheter-directed thrombolysis as a potential first-line treatment for postoperative HAT and PVT in pediatric liver transplant recipients.

Background Early hepatic artery thrombosis (HAT) after liver transplantation is a serious complication that frequently results in graft loss and the need for retransplantation. Although studies have reported on various operative and endovascular treatment approaches, pharmacologic strategies for the prevention or management of HAT are not well defined. Patients with blood clotting disorders, those with a contraindication to heparin, and those who have previously developed HAT represent unique challenges in management. Methods We present the case of a 9-month-old male with a hypercoagulable state who developed early HAT after two liver transplants, despite the use of postoperative therapeutic heparin infusion. Results and conclusion The patient successfully underwent a third liver transplant using intraoperative and postoperative bivalirudin infusion, a direct thrombin inhibitor. Rotational thromboelastometry (ROTEM) was used to guide anticoagulation and blood product administration in the perioperative period. At 1.5 years post-transplant, the patient has good graft function with patent hepatic vasculature. This case demonstrates the innovative use of bivalirudin anticoagulant therapy and viscoelastic methodologies to improve outcomes in hypercoagulable liver transplant recipients.

Transient elastography is an imaging technique utilizing shear wave technology to measure liver stiffness. Recent studies have shown success in utilizing this technique in children. Transient elastography is useful in estimating degree of fibrosis in various pediatric liver diseases, including biliary atresia, alpha-1-antitrypsin deficiency, Alagille syndrome, cystic fibrosis-related liver disease, and non-alcoholic steatohepatitis among others. Confounding factors, however, may affect elastography measurements, such as obesity, severe inflammation, nonfasting state, and hepatic congestion, and should be considered whenever interpreting these measurements. Future studies will correlate liver stiffness on transient elastography and severity of disease.

Dexmedetomidine, an α2 -agonist, is used in the PICU for its sedative properties as it minimally affects respiratory status. However, hemodynamic instability is one of its known side effects. There is limited published experience with its use in pediatric liver transplant. We present a case of a 9-month-old infant who received a deceased donor liver transplantation for biliary atresia and received an IV dexmedetomidine infusion for sedation starting at 20 hours post-operatively. The patient received an IV bolus of 0.08 mcg/kg followed by an increase to 1 mcg/kg/hour. She was also receiving a fentanyl infusion for sedation at the time of dexmedetomidine initiation. Approximately 3 hours after initiation, she developed bradycardia as low as 30 beats-per-minute with an associated sinus pause of 7 seconds. She was given chest compressions by the bedside nurse briefly before arousing and becoming agitated. Evaluation of other etiologies for the patient's bradycardia was unrevealing. Thus, bradycardia was attributed to dexmedetomidine therapy which was discontinued without recurrence. Hemodynamic instability, specifically bradycardia, is known to occur with dexmedetomidine administration. As this medication is primarily metabolized by the liver, its use immediately after transplantation, when liver function is still recovering, may be associated with an increased risk of side effects. Understanding risk factors for bradycardia and hemodynamic instability early after liver transplantation, particularly with dexmedetomidine, is critical to allow clinicians to identify the patients for higher risk for dexmedetomidine side effects.

OBJECTIVES Gastrointestinal bleeding is one of the most common indications for urgent endoscopy in the pediatric setting. The majority of these procedures are performed for control of variceal bleeding, with few performed for nonvariceal upper gastrointestinal (NVUGI) bleeding. The data on therapeutic endoscopy for NVUGI are sparse. The aims of our study were to review our experience with NVUGI bleeding, describe technical aspects and outcomes of therapeutic endoscopy, and determine gastroenterology fellows' training opportunities according to the national training guidelines. METHODS We performed a retrospective review of endoscopy database (Endoworks, Olympus Inc, Center Valley, PA) from January 2009 to December 2014. The search used the following keywords: bleeding, hematemesis, melena, injection, epinephrine, cautery, clip, and argon plasma coagulation. The collected data included demographics, description of bleeding lesion and medical/endoscopic therapy, rate of rebleeding, relevant laboratories, physical examination, and need for transfusion and surgery. The study was approved by the institutional review board. RESULTS During the study period 12,737 upper endoscopies (esophagogastroduodenoscopies) were performed. A total of 15 patients underwent 17 esophagogastroduodenoscopies that required therapeutic intervention to control bleeding (1:750 procedures). The mean ± standard deviation (median) age of patients who required endoscopic intervention was 11.6 ± 6.0 years (14.0 years). Seven out of 17 patients received dual therapy to control the bleeding lesions. All but 3 patients received medical therapy with intravenous proton pump inhibitor, and 3 received octreotide infusions. Six of the patients experienced rebleeding (40%), with 4 out of 6 initially only receiving single modality therapy. Two of these patients eventually required surgical intervention to control bleeding and both patients presented with bleeding duodenal ulcers. There were no cases of aspiration, perforation, or deaths. There were a total of 24 fellows trained in our program during the study period. Less than 1 therapeutic endoscopy per fellow for NVUGI bleeding was performed. CONCLUSIONS NVUGI bleeding requiring therapeutic endoscopic intervention is rare in pediatrics. A high rate (40%) of rebleeding was noted with a large proportion (66%) of patients receiving single modality therapy. Two patients required surgical intervention to control bleeding and both presented with bleeding duodenal ulcers. An insufficient number of therapeutic procedures is available for adequate fellow training requiring supplemental simulator and hands-on animal model, or adult endoscopy unit training.

Hepatic artery thrombosis (HAT) is a serious complication after liver transplantation. This is the first report of spontaneous resolution of HAT in pediatric liver transplant patients on low molecular weight heparin therapy. A total of 2 patients, a 26-month-old boy who presented with acute liver failure and required emergent liver transplantation and a 15-year-old boy with ulcerative colitis and autoimmune hepatitis-primary sclerosing cholangitis overlap underwent liver transplantation for progressive cirrhosis; both developed HAT during the postoperative period. They were both treated with low molecular weight heparin. Follow-up imaging for both patients showed resolution of HAT without evidence of collateral flow.