Pediatric Liver Disease Patients and Secondary Glycosylation Abnormalities

Background: Isoelectric focusing (IEF) of serum transferrin (Tf) is still the method of choice for diagnosis of congenital disorders of glycosylation (CDG). An abnormal glycosylation is also a known phenomenon in adult liver disease patients. The aim of this study was to characterize glycosylation disturbances in pediatric patients with primary liver disease. However, there are no reports of this phenomenon in children.Materials and Methods: Between 1995 and 2019, circa 2,000 serum Tf isoform analyses have been performed in children with primary liver diseases; some of them underwent subsequent analyses. We enrolled in this study 19 patients who developed an acute liver injury (ALI)/failure (ALF) or exhibited a chronic liver disease (CLD) and were evaluated and listed for liver transplantation (LTx) or had just undergone this procedure, and secondary abnormal serum Tf isoform profile.Results: Among 12 patients with ALI/ALF, 10 had an increased percentage of asialo-, monosialo-, and disialo-Tf isoforms. All patients with CLD had an increased percentage of asialo- and monosialo-Tf isoform. Two patients diagnosed with recurrent ALF had very specific serum Tf profile with a huge increase in the asialo- and monosialo-Tf isoform. On follow-up analyses (available in some patients), serum Tf IEF profile normalized in parallel to normalization of liver function tests, spontaneously or during treatment, including glucocorticosteroids in AIH, LTx in CLD.Conclusions: All pediatric patients with primary liver disease had increased asialo-Tf as well as monosialo-Tf isoforms. None of them had elevated percentage of trisialo-Tf isoform.

Liver Elastography in Healthy Children Using Three Different Systems – How Many Measurements Are Necessary?

Purpose Liver elastography is increasingly being applied in screening for and follow-up of pediatric liver disease, and has been shown to correlate well with fibrosis staging through liver biopsy. Because time is of the essence when examining children, we wanted to evaluate if a reliable result can be achieved with fewer acquisitions. Materials and Methods 243 healthy children aged 4–17 years were examined after three hours of fasting. Participants were divided into four age groups: 4–7 years; 8–11 years; 12–14 years and 15–17 years. Both two-dimensional shear wave elastography (2D-SWE; GE Logiq E9) and point shear wave elastography (pSWE; Samsung RS80A with Prestige) were performed in all participants, while transient elastography (TE, Fibroscan) was performed in a subset of 87 children aged 8–17 years. Median liver stiffness measurement (LSM) values of 3, 4, 5, 6, 7, and 8 acquisitions were compared with the median value of 10 acquisitions (reference standard). Comparison was performed for all participants together as well as within every specific age group. We investigated both the intraclass correlation coefficient (ICC) with absolute agreement and all outliers more than 10 %, 20 % or ≥ 0.5 or 1.0 kPa from the median of 10 acquisitions. Results For all three systems there was no significant difference between three and ten acquisitions, with ICCs ≥ 0.97. All systems needed 4 acquisitions to achieve no LSM deviating ≥ 1.0 kPa of a median of ten. To achieve no LSM deviating ≥ 20 % of a median of ten acquisitions, pSWE and TE needed 4 acquisitions, while 2D-SWE required 6 acquisitions. Conclusion Our results contradict recommendations of 10 acquisitions for pSWE and TE and only 3 for 2D-SWE.

Retrospective Review of Interventional Radiology Treatment and Outcomes in Pediatric Liver Disease

Background: Interventional radiology (IR) procedures offer less invasive and low risk options for the treatment of pediatric liver abnormalities, as well as life sustaining measures to bridge patients to transplant. Literature regarding IR interventions for pediatric liver disease is limited. Common conditions that can be treated with IR procedures include vascular anomalies: congenital portosystemic shunts, arteriovenous malformations (AVM), and Budd-Chiari malformation. Transjugular intrahepatic portosystemic shunt (TIPS) can be used to prevent recalcitrant variceal bleeding in portal HTN. Liver biopsy (percutaneous and transjugular) is used to sample the liver and can be targeted to tumors and other lesions. Percutaneous approaches to image biliary abnormalities and treat strictures are useful in children after liver transplant and also without., Objective: describe liver IR procedures and their outcomes in a single-center cohort study Methods: Patients will be identified through retrospective chart review at Riley Hospital for Children to identify and record the diagnoses, interventions, and relevant outcomes for eligible patients. Descriptive statistics can then be performed. This data set can be used to help inform clinicians on indications and expected outcomes for relevant hepatic IR procedures. Conclusion: It is anticipated that data collected for this project will show increased utilization of these procedures at the end of the study period compared to the beginning, and the complication rate for the IR procedure will be lower when compared to equivalent traditional procedures to treat the same condition.