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.

Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency.

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.

International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature.

Background & Aims: Substantial heterogeneity in terminology used for eosinophilic gastrointestinal diseases (EGIDs), particularly the catchall term "eosinophilic gastroenteritis," limits clinical and research advances. We aimed to achieve an international consensus for standardized EGID nomenclature.

Methods: This consensus process utilized Delphi methodology.

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.

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.

Eosinophilic esophagitis-Where are we today?

Objective: The objective of this review is to provide an overview of the practical diagnostic and therapeutic approaches to eosinophilic esophagitis and to increase the visibility of the disease among pediatricians.

Sources: A search of the MEDLINE, Embase, and CINAHL databases and recent consensus statements and guidelines were performed.

Summary Of The Findings: The definition of eosinophilic esophagitis is based on symptoms and histology.

Prevalence and characteristics of hepatopulmonary syndrome in children with cirrhosis in southern Brazil.

Background: Hepatopulmonary syndrome (HPS) is defined as a triad characterized by arterial deoxygenation, intrapulmonary vascular dilatations (IPVDs), and liver disorder. The aims of this study were to assess the prevalence of HPS in children with cirrhosis, the clinical characteristics of patients with HPS, and the tests used for the diagnosis of IPVD.

Patients And Methods: This was a prospective, cross-sectional study of 40 children with cirrhosis (median age: 44 months).

Updated International Consensus Diagnostic Criteria for Eosinophilic Esophagitis: Proceedings of the AGREE Conference.

Background & Aims: Over the last decade, clinical experiences and research studies raised concerns regarding use of proton pump inhibitors (PPIs) as part of the diagnostic strategy for eosinophilic esophagitis (EoE). We aimed to clarify the use of PPIs in the evaluation and treatment of children and adults with suspected EoE to develop updated international consensus criteria for EoE diagnosis.

Methods: A consensus conference was convened to address the issue of PPI use for esophageal eosinophilia using a process consistent with standards described in the Appraisal of Guidelines for Research and Evaluation II.

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes.

Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis.

Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments.

Gastroesophageal reflux disease: exaggerations, evidence and clinical practice.

Objective: there are many questions and little evidence regarding the diagnosis and treatment of gastroesophageal reflux disease (GERD) in children. The association between GERD and cow's milk protein allergy (CMPA), overuse of abdominal ultrasonography for the diagnosis of GERD, and excessive pharmacological treatment, especially proton-pump inhibitors (PPIs) are some aspects that need clarification. This review aimed to establish the current scientific evidence for the diagnosis and treatment of GERD in children.

Noninvasive methods for prediction of esophageal varices in pediatric patients with portal hypertension.

Aim: To evaluate clinical and laboratory parameters for prediction of bleeding from esophageal varices (EV) in children with portal hypertension.

Methods: Retrospective study of 103 children (mean age: 10.1 ± 7.

Ghrelin, leptin and insulin in cirrhotic children and adolescents: relationship with cirrhosis severity and nutritional status.

Objective: Ghrelin, leptin, and insulin concentrations are involved in the control of food intake and they seem to be associated with anorexia-cachexia in cirrhotic patients. The present study aimed to investigate the relationship between the nutritional status and fasting ghrelin, leptin and insulin concentrations in pediatric cirrhotic patients.

Methods: Thirty-nine patients with cirrhosis and 39 healthy controls aged 0-15 years matched by sex and age were enrolled.

Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience.

Purpose: Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period.

Methods: Patients with achalasia were identified from a database built during the period of January 2000-January 2012 from a Pediatric Gastroenterology reference center.

Contribution of endoscopy in the management of eosinophilic esophagitis.

Eosinophilic esophagitis (EoE) is a clinicopathological entity characterized by a set of symptoms similar to gastroesophageal reflux disease and eosinophilic infiltration of the esophageal epithelium. EoE is an emerging worldwide disease as documented in many countries. Recent reports indicate that EoE is increasingly diagnosed in both pediatric and adult patients although the epidemiology of this new disease entity remains unclear.

Managing gastroesophageal reflux disease in children: The role of endoscopy.

Gastroesophageal reflux disease (GERD) is a growing problem in the pediatric population and recent advances in diagnostics and therapeutics have improved their management, particularly the use of esophago-gastroduodenoscopy (EGD). Most of the current knowledge is derived from studies in adults; however there are distinct features between infant onset and adult onset GERD. Children are not just little adults and attention must be given to the stages of growth and development and how these stages impact the disease management.

Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome.

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy.

Shifting susceptibility to hepatitis A among children and adolescents over the past decade.

Objectives: To estimate the prevalence of anti-hepatitis A virus (anti-HAV) antibodies in serum samples from children and adolescents obtained at two clinical pathology laboratories in the city of Porto Alegre, south of Brazil, and to compare findings to those of a study carried out in the 1990s.

Methods: In this cross-sectional study conducted between 2007 and 2008, 465 serum samples obtained from subjects aged 1-19 years were consecutively tested to determine the prevalence of total anti-HAV antibodies. Samples were provided by a public laboratory (group 1) that serves the Unified Health System exclusively, meant to represent the lowest socioeconomic strata, and by a private laboratory (group 2), meant to represent the higher socioeconomic classes.

The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis.

Background/purpose: In biliary atresia (BA), a derangement in the biliary system remains, despite portoenterostomy performance. Many factors can influence the disease progression rate. This study aimed to analyze the association between biliary proliferation extent in biopsies from BA patients and postoperative prognosis.

Biliary atresia: we still operate too late.

Objective: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil.

Methods: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed.

[Eosinophilic esophagitis in 29 pediatric patients].

Background: Eosinophilic esophagitis is a recently described entity with esophageal symptoms like gastroesophageal reflux disease and significant esophageal eosinophilic infiltration.

Aim: To present our clinical series of 29 children with eosinophilic esophagitis, describing the clinical and diagnostic features, treatment and outcome.

Methods: We describe 29 patients (22 boys), 1-18 years-old, with 20 eosinophils per high-power field in esophageal biopsy specimens and absence of eosinophilic inflammation in the stomach and duodenum.