High prevalence of response to PPI treatment in children and adolescents with eosinophilic esophagitis in southern Brazil.

Introduction: Eosinophilic esophagitis is a newly recognized entity, in which there is significant evidence available that clearly demonstrates the positive impact of PPIs on reducing esophageal eosinophilia in individuals across different age groups, including children, adolescents, and adults. Multiple mechanisms have been proposed to explain how this treatment effect occurs. In Brazil, there seems to be a lack of studies that have prospectively assessed the clinical and therapeutic response rate in pediatric patients with EoE. The objective of this study was to prospectively evaluate the clinical and therapeutic response of pediatric patients with EoE in a medical center located in southern Brazil, by investigating the effectiveness of PPI treatment. Methods: This study is a clinical, prospective, open trial that took place in a pediatric hospital located in southern Brazil. The focus of the study was on patients diagnosed with Eosinophilic Esophagitis (EoE) who were given treatment using omeprazole/esomeprazole at a dosage of 1 mg.kg per dose, twice daily, for a period of 8-12 weeks. Following the treatment period, the patients underwent another endoscopy. Patients who exhibited 15 or less eosinophils in the biopsy conducted after the treatment were considered as responders. Results: A total of 27 patients was evaluated (74.1% boys). The average age (± standard deviation) was 8 years (±4). Nineteen patients (70.3%) were considered as responders to PPI treatment: 6 patients-22.2%-exhibited a complete response (defined as having 5 or fewer eosinophil per high power field. Additionally, 13 patients-48.1%-demonstrated a partial response, characterized by eosinophil counts exceeding 5 but less than 15 eos/hpf. When comparing the responder and non-responder groups at presentation, a statistical difference was observed in the prevalence of food refusal as a presenting symptom. Food refusal was found to be more prevalent in the non-responder group (87.5% vs. 26.3%, P = 0.008). No differences were observed in terms of atopy history and endoscopic scores. Upon comparing the histological findings from the post-treatment endoscopy of the two groups, it was observed that PPI responders exhibited a greater tendency to decrease basal cell hyperplasia (P = 0.06) and intercellular edema (P = 0.08). Conclusion: In this group of pediatric patients with EoE in Southern Brazil most patients showed a high prevalence of histological, endoscopic, and clinical response to PPI treatment. PPIs showed efficacy in Brazilian patients with EoE, most of whom would probably not be able to adequately undergo other treatments. Clinical Trial Registration: https://ensaiosclinicos.gov.br/rg/RBR-2ntbth9, identifier (U1111-1301-1842).

Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort.

BACKGROUND: Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. AIM: To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. METHODS: This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. RESULTS: Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. CONCLUSION: In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

Early hepatic arterial thrombosis in liver transplantation: Systemic intravenous alteplase as a potential rescue treatment after failed surgical revascularization.

eHAT is one of the most dreaded post-LT complication. Treatment approaches include retransplantation, revascularization, or observation. Systemic thrombolytic therapy is used in pediatric patients with thromboembolic events. However, there is no previous study reporting on the use of systemic r-tPA to treat eHAT. The treatment strategies used in patients with eHAT are described, focusing on two children who failed SR and were treated with systemic heparinization plus systemic r-tPA infusion. r-tPA-RP consists of intravenous systemic infusion at a dose of 0.3 mg/kg/h during 6 hours, for 5 days. First case (3-year) was transplanted with a whole liver, and second case (6-year) received a LLS from a living donor. HAT was diagnosed by doppler US and confirmed by angioCT scan in both patients in the first day after LT. They underwent SR and were clinically stable. Re-thrombosis occurred in both patients the day after, and r-TPA-RP was started-one patient required two r-TPA-RP for HAT recurrence. They presented minor bleeding, without repercussion. Hepatic artery recanalized after 10 and 3 days in the first and second patient, respectively. Retransplant was avoided, and one developed biliary strictures, successfully managed in the follow-up. r-TPA-RP avoided retransplantation after eHAT in these cases. To our knowledge, this is the first report of the use of systemic r-TPA to treat eHAT in children. This strategy may compose an algorithm to treat eHAT that failed SR in stable patients.

Erosive Tooth Wear and Erosive Esophagitis in Children: An Observational Study in Porto Alegre, Brazil.

The aim of this study was to establish and compare the prevalence and severity of erosive tooth wear (ETW) in children with and without erosive esophagitis. Children aged 5-12 years, scheduled for upper digestive endoscopy at the Pediatric Gastroenterology Service of the Children's Hospital Santo Antonio, Porto Alegre, Brazil, were eligible to participate in this study. Patients who presented erosive esophagitis at endoscopy were defined as gastroesophageal reflux disease (GERD) carriers, and the severity was described according to the Los Angeles classification. The oral cavity examination was performed by a trained and calibrated dentist and ETW was classified using the Basic Erosive Wear Examination (BEWE) index. Parents/guardians answered a questionnaire about the patients' diets and frequency of consumption of acidic foods and beverages. A total of 110 children were included in the study. Erosive esophagitis was observed in 24 patients (21.8%) and all of them (100%) presented ETW, showing a statistically significant association between these 2 conditions (p < 0.05). Among children who did not present with erosive esophagitis (n = 86), 54 (64.3%) had an ETW risk level of none according to their BEWE scores (0-2). The results of this study showed a statistically significant association between erosive esophagitis and ETW, thus it can be concluded that it is important to recognize groups at risk of ETW and act together with medical professionals to ensure adequate oral health for these patients.