Postoperative hypoalbuminemia and outcomes of pediatric liver transplantation.

BACKGROUND: Hypoalbuminemia after liver transplantation (LT) is associated with acute kidney injury (AKI) and poor outcomes in adult LT recipients. This study was performed to examine the association between the postoperative serum albumin level and early postoperative outcomes of LT in children. METHODS: This single-center retrospective review involved pediatric LT recipients (0-18 years old) treated from January 2013 to June 2020. All patients were admitted to PICU and received standard post-LT care protocol. We divided patients into low (< 30 g/L) and normal (> 30 g/L) groups based on postoperative albumin day 1 to 3. RESULTS: Among 108 LT recipients, most had biliary atresia. The median age at the time of LT was 1.8 years [interquartile range (IQR), 1.5-5.7]. There were 18 patients in low albumin group [median albumin level, 27.9 g/L (IQR, 25.8-29.6) and 90 patients in normal albumin group [median albumin level, 34.5 g/L (IQR, 32.4-36.9). The low albumin group had significantly higher incidence of AKI, occurring in 20% of patients with a median onset of 2.5 days following LT (IQR, 1-5). Postoperative hypoalbuminemia (OR, 4.94; 95% CI, 1.32-18.47; p = 0.01) and a longer operative time (OR, 1.37; 95% CI, 1.01-1.47; p = 0.02) were independent risk factors for AKI by multivariable analysis. No significant differences between the two groups were found in other early postoperative outcomes. CONCLUSION: Postoperative hypoalbuminemia was associated with early postoperative AKI following LT in children but not with other worsening outcomes.

Prediction of refractory gastroesophageal reflux disease in young children.

BACKGROUND: Many children respond to medical treatment for gastroesophageal reflux disease (GERD). However, some may require invasive intervention for refractory disease. Due to the lack of prognostic tools in children, this study aimed to develop a predictive model for refractory GERD. METHODS: A retrospective review was performed in children with symptoms of GERD at a university hospital. Refractory GERD was defined as an unresponsive disease after optimal treatment with medication for >8 weeks. The predictive model was constructed based on clinical features and 24-h multichannel intraluminal impedance-pH (MII-pH) monitoring results. RESULTS: A total of 205 children were included with a median (IQR) age of 0.6 (0.3, 2.0) years. Over half of the patients (59.5%) had motor disabilities. Forty-four children (21.5%) were diagnosed with refractory GERD and subsequently underwent fundoplication. Multivariable analysis suggested that the refractory disease was associated with motor disabilities (OR: 5.35; 95% CI: 2.06-13.91), recurrent aspiration pneumonia (OR: 2.78; 95% CI: 1.24-6.26), prematurity with an onset of GERD at a post-conceptual age <40 weeks (OR: 6.76; 95% CI: 1.96-23.33), and abnormal total reflux episodes according to age (OR: 2.78; 95% CI: 1.24-6.19), but not the acid exposure time or symptom association analysis. The predictive model for refractory GERD based on associated factors revealed an area under the ROC curve of 76.8% (95% CI: 69.2%-84.3%) with a sensitivity of 77.3% and a specificity of 64% when applying a cutoff score of ≥2.5. CONCLUSIONS: The predictive model, using clinical features and MII-pH, may be an additional tool to predict refractory GERD in young children.

Cost-effectiveness analysis of liver transplantation in biliary atresia according to the severity of end-stage liver disease.

BACKGROUND: Timing for liver transplantation (LT) in biliary atresia (BA) children with end-stage liver disease (ESLD) is associated with all-cause mortality. The cut-off value of pediatric end-stage liver disease (PELD) score for LT consideration varies across institutions. We aimed to determine the cost-effectiveness of LT to prevent death among BA children registered on the waiting list with different severities of ESLD. METHODS: Subjects were BA children aged < 12 years at a transplant center between 2010 and 2021. A decision tree was developed for cost-effectiveness analysis from a hospital perspective to compare all-cause death between patients initially registered with a low PELD score (< 15) and a high PELD score (≥ 15). Each patient's direct medical cost was retrieved from the beginning of registration until 5 years after LT, adjusted with an inflation rate to 2022 Thai Baht (THB). RESULTS: Among 176 children, 138 (78.4%) were initially registered with the high PELD score. The cost and mortality rate of the low PELD score group (THB1,413,424 or USD41,904 per patient and 31.6% mortality) were less than the high PELD score group (THB1,781,180 or USD52,807 per patient and 47.9% mortality), demonstrating the incremental cost-effectiveness ratio (ICER) of THB2,259,717 or USD66,994 per death prevented. The cost of early post-operative admission had the highest effect on the ICER. Considering the break-even analysis, cost among children initially registered at the low PELD score was also less expensive over time. CONCLUSIONS: Registration for LT at PELD score < 15 was more cost-effective to prevent death among BA children with ESLD.

Early postoperative duplex ultrasound findings of the hepatic artery in postoperative vascular complications from paediatric liver transplantation.

PURPOSE: To evaluate duplex US findings of the HA in all three postoperative vascular (HA, PV, HV and IVC) complications of paediatric LT for early detection and some helpful secondary signs to determine these vascular complications. MATERIALS AND METHODS: We collected data from 44 post-LT paediatric patients who underwent daily duplex US for seven consecutive days and three months after LT during January 2017-June 2020. Four duplex US parameters of the HA (extrahepatic PSV, intrahepatic PSV, RI and AT) were compared in patients with and without complications. RESULTS: The PSV of the extrahepatic HA in patients with HA complications was higher than that in patients without complications (P value = 0.019). The PSV at 107.7 cm/s is the optimal cut-off parameter associated with HA complications [a sensitivity of 88.9% and a specificity of 80.0% (ROC area is 0.84)]. The intrahepatic RI was higher on the first day than on the last day and gradually decreased in patients without vascular complications (P value = 0.000). The intrahepatic PSV significantly decreased with time when comparing the first and last days in patients without PV and HV-IVC complications (P value = 0.014 and 0.038). In contrast, patients with vascular complications showed no significant decrease. CONCLUSION: The extrahepatic PSV relates to HA complications after paediatric LT but not PV and HV-IVC complications. Non-significantly decreased intrahepatic RI and PSV from the first day to the day of complication diagnosis may correlate with the occurrence of vascular complications.

Etiologies, Prognostic Factors, and Outcomes of Pediatric Acute Liver Failure in Thailand.

PURPOSE: Pediatric acute liver failure (PALF) is a serious condition; however, data on PALF in developing countries are sparse, particularly concerning molecular diagnosis and liver transplantation (LT). This study aimed to determine the causes, outcomes, and prognostic factors of PALF. METHODS: We retrospectively reviewed the medical records of children (age <15 years) with PALF diagnosed using the American Association for the Study of Liver Diseases criteria at our center from 2011 to 2016. The collected data included laboratory results, complications, outcomes, and potential factors associated with death and LT. RESULTS: We included a total of 27 patients, with a median age of 2 years (interquartile range, 3 months to 4 years). Viral infection was the most common etiology (n=8, 30%), predominantly dengue infection (n=4). A total of 16 patients (59%) died and 11 patients survived (3 patients with LT). The prognostic factors associated with death or LT requirement were grade IV hepatic encephalopathy (p<0.01), hypotension (p=0.02), gastrointestinal bleeding (p=0.03), increased intracranial pressure (p=0.04), and higher peak serum lactate level (p=0.01). Peak serum lactate ≥6 mmoL/L had a sensitivity of 79% and a specificity of 88% for predicting mortality or the necessity of LT. CONCLUSION: Viral infection was the most common cause of PALF. The mortality rate remained high, and a considerable number of patients required LT. In addition to several clinical factors, peak serum lactate could be a potential marker for predicting poor outcomes in PALF.

High prevalence of multidrug-resistant gram-negative bacterial infection following pediatric liver transplantation.

Bacterial infection has been identified as one of the most significant complications of liver transplantation (LT). Multidrug-resistant (MDR) gram-negative bacteria (GNB) infection remains problematic issue following LT in the adults. However, data in children are scarce. We aimed to examine the prevalence and associated factors of MDR-GNB infection among pediatric LT recipients.We performed a single-center retrospectively study of 118 children who underwent LT between January 2010 and December 2018. Data on the prevalence, clinical characteristics, types, and sites of MDR-GNB infection within 3 months after LT as well as the treatment outcomes were collected. Multidrug resistance was defined as acquired non-susceptibility to at least 1 agent in 3 or more antibiotic classes.In total, 64 (53.7%) patients developed 96 episodes of culture-proven bacterial infection with 93 GNB isolates. Moreover, there were 58 (62.4%) MDR-GNB isolates, with a predominance of Klebsiella pneumoniae (32.7%), Escherichia coli (31%), and Pseudomonas aeruginosa (10.3%). Interestingly, 10 (17.2%) isolates were determined to be carbapenem-resistant Enterobacteriaceae. The median time to MDR-GNB infection was 9 (interquartile range: 5-33) days. The most common type of infection was intra-abdominal infection (47.9%). In the multivariate analysis, the significant variables associated with post-LT MDR-GNB infection include exposure to third-generation cephalosporins (hazard ratio [HR]: 2.16, P = .023), operative time (hazard ratio [HR] 1.20, P = .009), and length of intensive care unit stay (HR 1.03, P = .049). With a focus on carbapenem-resistant Enterobacteriaceae infection, a pediatric end-stage liver disease score >21 was the only significant 6 variable in the multivariate analysis (HR 11.48, P = .024). The overall 3-month mortality rate was 6.8%.This study has highlighted the high prevalence rate of MDR-GNB infection after pediatric LT. Therefore, caution on the emergence of MDR-GNB infection should be paid in at-risk children. Moreover, knowledge regarding the prevalence of MDR-GNB infection and resistant patterns is essential for guideline development to prevent and minimize the risk of MDR-GNB infection in this group of patients.

Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated with surgical shunt ligation: A case report and literature review.

INTRODUCTION: Congenital extrahepatic portosystemic shunt is also known as Abernethy malformation. It is a rare anomaly of the portal venous system where partial or complete portal blood flow drains directly into the systemic vein via this abnormal shunt, bypassing the liver. Occasionally, this condition is incidentally detected on imaging studies performed for other indications. An early diagnosis is challenging due to the lack of specific symptoms-often leading to delayed diagnosis and development of subsequent complications. PRESENTATION OF CASE: A 4-year-old boy presented with multiple cutaneous hemangiomas with concomitant splenomegaly. While the diagnostic work-up for the definite cause of splenomegaly was in progress, an Abernethy malformation was incidentally noted by abdominal ultrasonography. After computerized tomography (CT) imaging confirmed the diagnosis, the patient underwent a successful surgical shunt ligation without any complications through a one-year follow-up period. DISCUSSION: Various imaging studies, including Doppler ultrasonography, CT imaging, and Magnetic Resonance Imaging (MRI), are useful tools to diagnose an Abernethy malformation. Preoperative angiography with a balloon occlusion of the shunt is highly recommended in order to opt for the most appropriate intervention-including one-or-two-step surgical closure, endovascular occlusion, and liver transplantation. CONCLUSION: An Abernethy malformation should be considered in the differential diagnosis of unexplained portal hypertension or any of its associated complications in pediatric patients. Early diagnosis and proper management of this condition can lead to a favorable prognosis, as in this case. Accordingly, a pre-operative evaluation is important to determine the proper therapeutic method.

Anaplastic Large Cell Lymphoma of the Duodenum in a Teenage Girl: Misdiagnosed as an Intramural Duodenal Hematoma.

We report a case of a 13-year-old girl who presented with a 2-month history of intermittent abdominal pain. Laboratory examination showed hepatitis and pancreatitis. Because of persistent vomiting, computed tomography (CT) was performed, which revealed a circumferential soft tissue density in the duodenal wall, causing partial obstruction. Supportive therapy failed. Repeat CT showed no significant change from the initial study. The patient underwent upper endoscopy, which revealed a mass in the second portion of the duodenum, which occluded most parts of the lumen. The histopathological finding was consistent with an anaplastic large cell lymphoma, a rare form of small bowel neoplasm. After the third course of chemotherapy, complete resolution of the mass was noted, and her symptoms were relieved.

Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation.

BACKGROUND AND STUDY AIMS: Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT). MATERIAL AND METHODS: All pediatric patients with CLD listed for LT were studied. Pulse oxygen saturation (SpO(2)), technetium-99m-labeled macroaggregated albumin ((99m)Tc- MAA) perfusión scan (positive test: uptake of the isotope ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed. SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20 bubbles), and grade III (> 20 bubbles). RESULTS: Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary atresia (77.8%). Pre-LT, all patients had SpO(2) of 100% and none had positive (99)mTc- MAA perfusion scan. Two patients (11%) had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT became negative in all survivors (n = 16), (p = 0.0001). CONCLUSIONS: Most cirrhotic children in this cohort study had evidence of IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.