Background: This study investigated the prognostic parameters and beneficial effects of repeat plasma exchange in children with acute liver failure (ALF). Methods: Twenty-three patients under 18 years of age admitted to National Taiwan University Hospital due to ALF from 2003 to 2016 were included in this retrospective analysis. Results: Among the patients, 11 (48%) had native liver recovery (NLR), 9 (39.1%) died without liver transplant, and 3 (12.9%) received liver transplantation. The NLR group showed a lower proportion of idiopathic cases, lower peak ammonia level, higher peak alpha fetoprotein (AFP) level, and they had plasma exchange fewer times than the other groups. Receiver operating characteristic curve analyses yielded optimal cutoff values of plasma exchange (6 times), peak ammonia level (<190 mmol/L), and peak AFP level for predicting NLR in children with ALF. Conclusion: Pediatric ALF with idiopathic etiology, high peak ammonia level, and low peak AFP level are associated with fewer cases of NLR. Plasma exchange for more than six times probably offers little benefit with regard to patient survival if liver transplantation is not performed promptly.

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Abstracts Background Maternal tenofovir disoproxil fumarate (TDF) therapy during late pregnancy can reduce mother-to-infant transmission of hepatitis B virus (HBV). We investigated HBV mutations associated with maternal TDF therapy and their role in infant immunonophylaxis failure (IPF). Methods Serum samples from untreated (n = 89) and TDF-treated (n = 68), highly-viremic, chronically infected mothers and their infants were analyzed for HBV DNA by nested-PCR and direct sequencing. Results At delivery, compared with untreated mothers, TDF-treated mothers had a lower HBV DNA titer and a higher frequency of basal core promoter (BCP) gene mutations, but they had similar frequencies in pre-S/S and pre-core/core mutations. The 14 mothers harboring surface “a” determinant mutants did not transmit the mutants to their immunized infants. Such mutants were found in 3 of 13 IPF infants; the 13 mothers had wild-type HBsAg. In univariable analysis, maternal HBV DNA titer (odds ratio [OR]: 1.54; 95% confidence intervals [CI]:1.02∼2.33; P = 0.039), genotype C (OR: 4.18; 95% CI: 1.28∼13.62; P = 0.018) and pre-S1 wild-type sequence (OR: 6.33; 95% CI: 1.85∼21.68; P = 0.003) at delivery were associated with infant IPF. Multivariable analyses showed that maternal genotype C (OR: 3.71; 95% CI: 1.11-12.36; P = 0.033) and pre-S1 wild-type (OR: 6.34; 95% CI: 1.79-22.44; P = 0.004) were associated with infant IPF independently of maternal viremia. Conclusions Along with high maternal HBV DNA titer at delivery, maternal genotype C and pre-S1 wild-type sequence were potential risk factors for infant IPF, while BCP mutations were not. The offspring of pregnant women harboring “a” determinant mutants as major strains seemed to be protected by immunoprophylaxis.

Background: Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC. Methods: We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary center in Taiwan. The clinical data of the patients, including clinical and endoscopic UC severity scores, medications, and laboratory parameters, were analyzed. Results: We recruited five children with PSC-UC (PSC-UC group), and 26 with UC alone (non-PSC UC group) in this retrospective analysis. Among the patients with PSC-UC, four (80%) were compatible with definite or probable autoimmune sclerosing cholangitis (ASC). The UC Endoscopic Index of Severity (5.00 vs. 9.00, P Z 0.003) and Mayo score (4.00 vs. 8.00, P Z 0.014) were significantly lower in the PSC-UC group than the non-PSC UC group. The prevalence of immunomodulator use was significantly higher in the PSC-UC than the non-PSC UC group (100% vs. 42.3%, P Z 0.043), but there was no difference regarding steroids, mesalamine, or biologics. At the end of the study, significantly fewer patients were steroid-free in the PSC-UC than the non-PSC UC group (20.0% vs. 84.6%, P Z 0.010). Conclusions: Pediatric patients with PSC-UC had less severe colitis than those with UC alone in