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

Author: Luiza Salgado Nader, Matias Epifanio, Mariana Guimarães Coelho, Cintia Steinhaus, Melina Melere, Carolina Soares da Silva, and Cristina Targa Ferreira
Subjects: proton-pump inhibitor responsive esophageal eosinophilia, eosinophilic esophagitis, children, proton-pump inhibitor, histological remission, Immunologic diseases. Allergy, RC581-607
Abstract: IntroductionEosinophilic 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.MethodsThis 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.ResultsA 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).ConclusionIn 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 Registrationhttps://ensaiosclinicos.gov.br/rg/RBR-2ntbth9, identifier (U1111-1301-1842).
Published: 2024
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2. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency

Author: Antonia Felzen, Daan B.E. van Wessel, Emmanuel Gonzales, Richard J. Thompson, Irena Jankowska, Benjamin L. Shneider, Etienne Sokal, Tassos Grammatikopoulos, Agustina Kadaristiana, Emmanuel Jacquemin, Anne Spraul, Patryk Lipiński, Piotr Czubkowski, Nathalie Rock, Mohammad Shagrani, Dieter Broering, Emanuele Nicastro, Deirdre Kelly, Gabriella Nebbia, Henrik Arnell, Björn Fischler, Jan B.F. Hulscher, Daniele Serranti, Cigdem Arikan, Esra Polat, Dominique Debray, Florence Lacaille, Cristina Goncalves, Loreto Hierro, Gema Muñoz Bartolo, Yael Mozer-Glassberg, Amer Azaz, Jernej Brecelj, Antal Dezsőfi, Pier Luigi Calvo, Enke Grabhorn, Steffen Hartleif, Wendy J. van der Woerd, Binita M. Kamath, Jian-She Wang, Liting Li, Özlem Durmaz, Nanda Kerkar, Marianne Hørby Jørgensen, Ryan Fischer, Carolina Jimenez-Rivera, Seema Alam, Mara Cananzi, Noemie Laverdure, Cristina Targa Ferreira, Felipe Ordoñez Guerrero, Heng Wang, Valerie Sency, Kyung Mo Kim, Huey-Ling Chen, Elisa de Carvalho, Alexandre Fabre, Jesus Quintero Bernabeu, Aglaia Zellos, Estella M. Alonso, Ronald J. Sokol, Frederick J. Suchy, Kathleen M. Loomes, Patrick J. McKiernan, Philip Rosenthal, Yumirle Turmelle, Simon Horslen, Kathleen Schwarz, Jorge A. Bezerra, Kasper Wang, Bettina E. Hansen, and Henkjan J. Verkade
Subjects: BSEP, PFIC2, compound heterozygosity, interruption of the enterohepatic circulation, genotype, phenotype, Diseases of the digestive system. Gastroenterology, RC799-869
Abstract: 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.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p
Published: 2023
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3. P-125 REX SHUNT IN A DEVELOPING COUNTRY – IS IT POSSIBLE?

Author: Caroline Montagner Dias, Melina Utz Melere, Carolina Soares da Silva, Luiza Salgado Nader, Cristine Suzana Trein, Alex Finger Horbe, Cristina Targa Ferreira, and Riccardo Superina
Subjects: Specialties of internal medicine, RC581-951
Abstract: Introduction: Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. Objective: Describe the experience in the surgical treatment of EHPVO in children, in a developing country. Methods: Retrospective case series study, with medical records review of patients with EHPVO, who underwent surgical treatment, by an experienced surgeon, between July 2016 and May 2019. Patient profile, laboratory test, images, liver histology, surgery performed, postoperative complications and shunt patency were analysed. Results: 12 patients, median age of 4 years, umbilical catheterization was present in 8 patients (66,6%). Ten patients performed portography, and 60% had type A by Baveno VI criteria. Despite normal liver tests, liver biopsy revealed ductular proliferation in 83,3% of patients and mild portal fibrosis in 66,7%. Splenomegaly was present in 91,7% and thrombocytopenia in 83,3%. All patients had oesophageal varices and gastrointestinal bleeding occurred in 83,3%. Among the coagulation tests, the deficiency of C and S proteins is noteworthy in most patients, with 72,3% and 63,6% respectively.It was possible to perform meso-Rex bypass in 10 patients (83,3%); in the other 2 distal splenorenal shunt was performed. Early post-operative complications occurred in 58,3% of patients, the most common was ascites in 4 (33,3%), which resolved in less than 1 month. One patient developed shunt thrombosis in the first 7 days after surgery, not resolved with thrombectomy. In outpatient follow-up one patient developed thrombosis in the Rex shunt and another 4 had stenosis. All of them underwent to interventional radiology. Currently 8 of 10 meso-Rex patients (80%) have patent shunt. Conclusion: Rex shunt is possible in developing countries with an experienced surgeon and multidisciplinary team.
Published: 2021
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4. Eosinophilic esophagitis – Where are we today?

Author: Cristina Targa Ferreira, Mario Cesar Vieira, Glenn T. Furuta, Fernando Celso Lopes Fernandes de Barros, and Mirna Chehade
Subjects: Pediatrics, RJ1-570
Abstract: 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. It is important to rule out other diseases associated with esophageal eosinophil‐predominant inflammation. It is not yet clear whether the increased prevalence is due to a real increase in incidence or a result of increased awareness of the disease. Various options for management have been used in pediatric patients, including proton pump inhibitors, dietary restriction therapies, swallowed topical steroids, and endoscopic dilations. More recently, proton pump inhibitor‐responsive esophageal eosinophilia and eosinophilic esophagitis have been contemplated on the same spectrum, and proton pump inhibitors should be considered the initial step in the treatment of these patients. Conclusions: Eosinophilic esophagitis is a relatively new disease with a remarkable progression of its incidence and prevalence in the past two to three decades, and diagnostic criteria that are constantly evolving. It is important to better understand the pathogenesis of the disease, the predisposing factors, the natural history, and the categorization of varying phenotypes to develop diagnostic and therapeutic strategies that meet the clinical needs of patients. Resumo: Objetivo: Fornecer uma visão geral do diagnóstico e do tratamento da esofagite eosinofílica na prática clínica e aumentar a visibilidade da doença entre os pediatras. Fontes dos dados: Foi feita uma busca na literatura relevante nos bancos de dados Medline, Embase, CINAHL e consensos e diretrizes recentes foram revisados. Síntese dos dados: A definição de esofagite eosinofílica é baseada nos sintomas e na histologia. É importante excluir outras doenças associadas com inflamação esofágica predominantemente eosinofílica. Ainda não está claro se o aumento na prevalência é devido a um real aumento da incidência ou se é o resultado da maior suspeição diagnóstica. Várias opções para tratamento, inclusive inibidores de bomba de prótons, restrições dietéticas, esteroides tópicos deglutidos e dilatações endoscópicas têm sido usadas em pacientes pediátricos. Mais recentemente a eosinofilia esofágica responsiva a inibidores de bomba de prótons e a esofagite eosinofílica têm sido contempladas no mesmo espectro e os inibidores de bomba de prótons devem ser considerados como opção inicial no tratamento desses pacientes. Conclusões: A esofagite eosinofílica é uma doença relativamente nova com uma notável progressão da incidência e prevalência nas últimas 2‐3 décadas e critérios diagnósticos estão em evolução constante. É importante entender melhor a patogênese dessa doença, os fatores predisponentes, a história natural e a categorização dos diferentes fenótipos para desenvolver estratégias diagnósticas e terapêuticas que vão ao encontro das necessidades clínicas dos pacientes. Keywords: Eosinophilic esophagitis, Esophageal eosinophilia, Eosinophilic, Proton pump inhibitors, Palavras‐chave: Esofagite eosinofílica, Eosinofilia esofágica, Eosinofílico, Inibidores de bomba de prótons
Published: 2019
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5. Tufting enteropathy with EpCAM mutation: case report

Author: Karla Lais Pêgas, Eduardo Cambruzzi, Regis Schander Ferrelli, Carolina Soares da Silva, Renata Rostirola Guedes, Marina Adami, Eduardo Montagner Dias, Melina Utz Melere, Marilia Rosso Ceza, Cintia Steinhaus, Matias Epifanio, Julie Salomon, and Cristina Targa Ferreira
Subjects: enteropatia com tufos epiteliais, diarreia intratável, mucosa intestinal, molécula de adesão das células epiteliais, gene EPCAM, Pathology, RB1-214
Abstract: Tufting enteropathy (TE), also known as intestinal epithelial dysplasia (IED), is a rare congenital enteropathy related to an earlyonset of severe intractable diarrhea due to specific abnormalities of the intestinal epithelium and mutations of the EpCAM gene. TE is characterized by clinical and histological heterogeneity, such as with low or without mononuclear cell infiltration of the lamina propria, and abnormalities of basement membrane. TE can be associated with malformations, other epithelial diseases, or to abnormal enterocytes development and/or differentiation. The authors report a case of a Brazilian child with TE associated with c.556-14A>G mutation in the EpCAM gene (NM_002354.2).
Published: 2014
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6. Gastroesophageal reflux disease: exaggerations, evidence and clinical practice

Author: Cristina Targa Ferreira, Elisa de Carvalho, Vera Lucia Sdepanian, Mauro Batista de Morais, Mário César Vieira, and Luciana Rodrigues Silva
Subjects: Doença do refluxo gastroesofágico, Refluxo gastroesofágico, Inibidores de bomba de prótons, Inibidores da bombade prótons/uso terapêutico, Lactente, Criança, Pediatrics, RJ1-570
Abstract: 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. DATA SOURCE: a search was conducted in the MEDLINE, PubMed, LILACS, SciELO, and Cochrane Library electronic databases, using the following keywords: gastroesophageal reflux; gastroesophageal reflux disease; proton-pump inhibitors; and prokinetics; in different age groups of the pediatric age range; up to May of 2013. DATA SYNTHESIS: abdominal ultrasonography should not be recommended to investigate gastroesophageal reflux (GER). Simultaneous treatment of GERD and CMPA often results in unnecessary use of medication or elimination diet. There is insufficient evidence for the prescription of prokinetics to all patients with GER/GERD. There is little evidence to support acid suppression in the first year of life, to treat nonspecific symptoms suggestive of GERD. Conservative treatment has many benefits and with low cost and no side-effects. CONCLUSIONS: there have been few randomized controlled trials that assessed the management of GERD in children and no examination can be considered the gold standard for GERD diagnosis. For these reasons, there are exaggerations in the diagnosis and treatment of this disease, which need to be corrected.
Published: 2014
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7. Esofagite eosinofílica em 29 pacientes pediátricos Eosinophilic esophagitis in 29 pediatric patients

Author: Cristina Targa Ferreira, Mário C. Vieira, Sandra Maria Gonçalves Vieira, Giovana Stival da Silva, Danielle Reis Yamamoto, and Themis Reverbel da Silveira
Subjects: Esofagite, Eosinofilia, Criança, Esophagitis, Eosinophilia, Child, Diseases of the digestive system. Gastroenterology, RC799-869
Abstract: RACIONAL: A esofagite eosinofílica é uma entidade recentemente descrita, caracterizada por sintomas esofágicos, semelhantes aos da doença por refluxo gastroesofágico e importante eosinofilia esofágica. OBJETIVO: Apresentação de 29 pacientes com esofagite eosinofílica, discutindo as características clínicas, diagnóstico, tratamento e evolução. MÉTODOS: Foram identificados 29 pacientes (22 do sexo masculino) com idade entre 1 e 18 anos, nos quais as biopsias de esôfago demonstraram contagem de 20 ou mais eosinófilos/campo de grande aumento, sem infiltração eosinofílica em antro e/ou duodeno. Avaliaram-se as manifestações clínicas, achados endoscópicos e histológicos, tratamento e evolução. RESULTADOS: Os sintomas mais freqüentes foram vômitos em 15 pacientes (52%) e dor abdominal em 11 (38%). Os pacientes com idade inferior a 4 anos apresentavam recusa alimentar e baixo peso. Os com idades entre 5 e 8 anos apresentavam predominantemente dor abdominal e/ou pirose e/ou vômitos. Os pacientes com mais de 8 anos apresentavam dor abdominal, disfagia e/ou impactação alimentar eventual. Os achados endoscópicos incluíram estrias verticais em 14 pacientes (48%), pontilhado branco em 12 (41%), anéis circulares em 2 (7%) e esofagite erosiva em 3 (10%). Em sete pacientes a endoscopia foi normal (24%). O tratamento incluiu fluticasona tópica em 19 pacientes e restrição dietética em 7. Os pacientes acompanhados apresentaram resposta favorável ao tratamento, com melhora ou remissão dos sintomas. Onze pacientes que foram submetidos a endoscopia de controle pós-tratamento apresentaram diminuição significativa do número de eosinófilos no esôfago. CONCLUSÕES: A esofagite eosinofílica deve ser considerada quando há sintomas de refluxo, que não respondem ao tratamento habitual. Os exames endoscópicos devem ser acompanhados de biopsias com análise detalhada do número de eosinófilos.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. Evaluation of the clinical, endoscopic and histologic findings, treatment and outcome was undertaken. RESULTS: The most common presenting symptoms included vomiting in 15 patients (52%) and abdominal pain in 11 patients (38%). Children under the age of 4 years presented with feeding disorder and failure to thrive. Patients between 5 and 8 years of age presented commonly with abdominal pain or symptoms that may be associated with reflux (heartburn and/or vomiting). Patients over the age of 8 presented most often with abdominal pain, dysphagia and occasional food impaction. Endoscopic features included vertical furrowing in 14 patients (48%), whitish papules in 12 (41%), corrugated rings in 2 patients (7%) and esophageal erosions in 3 patients (10%). In seven patients endoscopy was normal (24%). Treatment included swallowed fluticasone propionate in 19 patients and restriction diet in 7 patients. Patients who returned for follow-up had either improvement or remission of symptoms. After treatment, endoscopic biopsies were repeated in 11 patients, and a significant decrease in esophageal eosinophil counts was observed. CONCLUSIONS: The diagnosis of eosinophilic esophagitis must be considered when symptoms of reflux do not respond to conventional treatment. Upper gastrointestinal endoscopy must be complemented by a detailed analysis of histologic findings and eosinophil counts.
Published: 2008
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8. Prevalence of the serpin peptidase inhibitor (alpha-1-antitrypsin) PIS and PIZ alleles in Brazilian children with liver disease

Author: Guilherme Baldo, Ana Ayala, Matias Melendez, Karina Nonnemacher, Luciane Lima, Sandra Leistner Segal, Carlos Kieling, Sandra Gonçalves Vieira, Cristina Targa Ferreira, Themis Reverbel da Silveira, Roberto Giugliani, and Ursula Matte
Subjects: alpha-1-antitrypsin deficiency, liver disease, pediatric patients, SERPINA1, Genetics, QH426-470
Abstract: Serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1 (SERPINA1) deficiency is one of the main genetic causes related to liver disease in children. In SERPINA1 deficiency the most frequent SERPINA1 alleles found are the PI*S and PI*Z alleles. We used the polymerase chain reaction and the amplification created restriction site (ACRS) technique to investigate the prevalence of the PI*S and PI*Z alleles in a group of Brazilian children (n = 200) with liver disease and established the general frequency of the PI*S allele in our population. We found a significant association of the PI*Z allele and liver disease, but no such relationship was found for the PI*S allele. Our results show that SERPINA1 deficiency due to the PI*Z allele, even when heterozygous, is a frequent cause of liver disease in our group of Brazilian children but that the PI*S allele does not confer an increased risk of hepatic disorders in our group of Brazilian children.
Published: 2008
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9. Alergia alimentar: atualização prática do ponto de vista gastroenterológico Food allergy: a practical update from the gastroenterological viewpoint

Author: Cristina Targa Ferreira and Ernest Seidman
Subjects: Alergia alimentar, alergia mediada por IgE, alergia não-mediada por IgE, alergia mediada por células, prevenção, tratamento, Food allergy, IgE-mediated allergy, non-IgE-mediated allergy, cell-mediated allergy, prevention, treatment, Pediatrics, RJ1-570
Abstract: OBJETIVOS: Apresentar uma revisão atualizada e crítica sobre alergias alimentares, focando principalmente em tratamento e prevenção. FONTES DOS DADOS: Revisão da literatura publicada obtida através do banco de dados MEDLINE, sendo selecionados os mais atuais e representativos do tema (2000-2006). A pesquisa incluiu os sites da European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) e American Academy of Pediatrics (AAP). SÍNTESE DOS DADOS: A prevalência de doenças alérgicas tem aumentado nas últimas décadas e alergia alimentar parece fazer parte desse aumento. Alergia alimentar é muito mais comum em pediatria e apresenta impacto médico, financeiro e social significativos em crianças menores e suas famílias. Tratamento e prevenção da alergia alimentar são desafios maiores do ponto de vista da saúde pública e para as comunidades médica e científica. Há muita informação incorreta e condutas médicas discutíveis nessa área. Apresentamos e discutimos as recomendações publicadas pelos Comitês de Nutrição da ESPGHAN juntamente com a Sociedade Européia Pediátrica de Alergologia e Imunologia Clínica (ESPACI) e AAP. CONCLUSÃO: Excesso de diagnósticos de alergia alimentar é bastante prevalente. Há necessidade de uniformização de definições e procedimentos diagnósticos. O objetivo primário do manejo deve ser o de instituir medidas efetivas de prevenção das alergias alimentares. Há necessidade de métodos precisos para confirmar ou excluir o diagnóstico. Os pacientes necessitam tratamento apropriado através da eliminação de alimentos que causam sintomas, ao mesmo tempo evitando os efeitos adversos nutricionais e o custo de dietas inadequadas.OBJECTIVE: To present an up-to-date and critical review regarding food allergies, focusing mainly on treatment and prevention. SOURCES: Review of published literature searched on MEDLINE database; those data which were the most up-to-date and representative were selected (2000-2006). The search included the European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the American Academy of Pediatrics (AAP). SUMMARY OF THE FINDINGS: The prevalence of allergic diseases has increased over the last decades, and food allergy seems to be part of this increase. Food allergy is much more common in pediatrics and has a significant medical, financial and social impact on young children and their families. Treatment and prevention of food allergy is a major challenge for public health, scientific and medical communities. There is a lot of misinformation and the medical management of this condition is still discussable. We present and discuss the guidelines regarding criteria for the prevention of food allergy and atopic diseases published by the Nutrition Committees of ESPGHAN jointly with the European Society for Pediatric Allergy and Clinical Immunology (ESPACI) and AAP. CONCLUSION: The overdiagnosis of food allergy is quite prevalent. There is a need for standardization of definitions and diagnostic procedures. The primary goal of therapy should be to first establish effective means of preventing food allergies. There is a need for accurate diagnostic methods to confirm or rule out the diagnosis. Patients need appropriate treatment by eliminating foods that cause symptoms, while avoiding the nutritional side effects and the cost of inappropriate diets.
Published: 2007
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10. Hepatites virais: aspectos da epidemiologia e da prevenção Viral Hepatitis: epidemiological and preventive aspects

Author: Cristina Targa Ferreira and Themis Reverbel da Silveira
Subjects: Hepatites virais, Epidemiologia, Hepatite A, Hepatite B, Hepatite C, Prevenção, Viral hepatitis, Epidemiology, Hepatitis A, Hepatitis B, Hepatitis C, Prevention, Public aspects of medicine, RA1-1270
Abstract: As hepatites virais são doenças causadas por diferentes agentes etiológicos, de distribuição universal, que têm em comum o hepatotropismo. Possuem semelhanças do ponto de vista clínico-laboratorial, mas apresentam importantes diferenças epidemiológicas e quanto à sua evolução. As últimas décadas foram de notáveis conquistas no que se refere à prevenção e ao controle das hepatites virais. Entre as doenças endêmico-epidêmicas, que representam problemas importantes de saúde pública no Brasil, salientam-se as Hepatites Virais, cujo comportamento epidemiológico, no nosso país e no mundo, tem sofrido grandes mudanças nos últimos anos. A melhoria das condições de higiene e de saneamento das populações, a vacinação contra a Hepatite B e as novas técnicas moleculares de diagnóstico do vírus da Hepatite C estão entre esses avanços importantes. As condições do nosso país: sua heterogeneidade socioeconômica, a distribuição irregular dos serviços de saúde, a incorporação desigual de tecnologia avançada para diagnóstico e tratamento de enfermidades, são elementos importantes que devem ser considerados na avaliação do processo endemo-epidêmico das hepatites virais. O números de pacientes infectados é incerto, relacionado geralmente a alguns Estados e municípios brasileiros, e o esclarecimento dos agentes causadores das hepatites, cuja identificação requer técnicas laboratoriais complexas de biologia molecular, é realizado de maneira insuficiente. Por outro lado, "a progressiva integração entre as instâncias gestoras dos programas de vigilância e controle das doenças com grupos de pesquisa e desses com os serviços" e a disponibilização de bancos de dados nacionais mais confiáveis apontam para novos e melhores caminhos. No presente artigo é feita uma revisão sucinta das hepatites A, B e C, as mais freqüentes no nosso país, assim como de sua epidemiologia e das estratégias preferenciais para a prevenção dessas doenças.Viral hepatitis is a disease caused by different etiological agents with universal distribution and that have hepatotropism as a common characteristic. They are similar from a clinical-laboratorial point-of-view, but present significant differences in their epidemiology and outcome. The past few decades have brought remarkable victories in relation to the prevention and control of viral hepatitis. Viral hepatitis is very important among the endemic-epidemic diseases that are major public health problems in Brazil, and its epidemiological behavior has undergone major changes over the past few years, both in our country and worldwide. The expansion of substantial improvement in sanitary conditions, the increase in the coverage of hepatitis B vaccination, and the new molecular diagnostic assays of Hepatitis C virus were all decisive factors that contributed to these changes. Various important conditions in our country (socio-economic heterogeneity, irregular distribution of health services, unequal incorporation of advanced techniques for diagnosis and treatment of diseases) must be taken into account when assessing the endemic-epidemic process of viral hepatitis. The number of infected patients is uncertain, especially in some Brazilian states and cities, and the elucidation of the causal agents of hepatitis, whose identification requires complex molecular biology laboratory techniques, is insufficiently performed. On the other hand, "the progressive integration of agencies that manage disease surveillance and control programs and research groups, and between the latter and services," and the availability of more reliable national databases, suggest new and better possibilities. In the present paper, we have briefly reviewed hepatitis A, B and C, the most frequent forms in our country, and the epidemiology and the preferred strategies for preventing this disease.
Published: 2004
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11. Prevenção das hepatites virais através de imunização Viral hepatitis prevention by immunization

Author: Cristina Targa Ferreira and Themis Reverbel da Silveira
Subjects: Hepatite A, hepatite B, vacinas, imunização, Hepatitis A, hepatitis B, vaccines, immunization, Pediatrics, RJ1-570
Abstract: OBJETIVO: Apresentar uma revisão atualizada e crítica da prevenção das hepatites virais A e B, através de imunização. FONTE DOS DADOS: Revisão de artigos médicos obtidos através do banco de dados MEDLINE, sendo selecionados os mais atuais e representativos do tema (2000-2006). Foram também pesquisados os sites do Centers for Disease Control and Prevention (CDC) e American Academy of Pediatrics (AAP), da Sociedade Brasileira de Pediatria (SBP) e do Ministério da Saúde do Brasil. SÍNTESE DOS DADOS: A prevenção das hepatites virais é um enorme desafio para o sistema de saúde pública dos países e das comunidades médica e científica. Os vírus das hepatites ocasionam importante morbimortalidade no mundo, causando doença hepática aguda e crônica. Vacinas altamente eficazes estão disponíveis no mercado para prevenir novas infecções pelos vírus A e B. Entretanto, as hepatites virais A e B continuam a estar entre as doenças preveníveis por vacinas mais comumente notificadas. Neste artigo, revisamos as vacinas usadas para prevenir essas infecções com o objetivo de expandir o conhecimento e o uso da prevenção dessas doenças infecciosas. CONCLUSÃO: Embora as vacinas contra as hepatites A e B sejam recomendadas para vários grupos de risco, a cobertura vacinal estimada ainda é modesta e existem muitas oportunidades perdidas de vacinação. Para que haja diminuição na incidência das hepatites A e B, duas doenças preveníveis por vacinas, é necessário que os médicos incentivem seus pacientes a receber as vacinas.OBJECTIVE: To present an updated review and criticism of viral hepatitis A and B prevention by immunization. SOURCES OF DATA: Review of medical articles obtained from the MEDLINE database. The most recent and representative articles on the subject (2000-2006) were selected. The Centers for Disease Control and Prevention (CDC), American Academy of Pediatrics (AAP), Brazilian Society of Pediatrics and Brazilian Ministry of Health websites were also researched. SUMMARY OF THE FINDINGS: Viral hepatitis prevention is an enormous challenge to the public health systems of countries and the medical and scientific communities. Hepatitis viruses produce important morbidity and mortality in the world, causing acute and chronic hepatic disease. There are highly efficient vaccines available on the market to prevent new infections by the A and B viruses. However, A and B viruses continue to be among the most commonly notified diseases preventable by vaccines. In this article, we discuss the vaccines used to prevent these infections, with the aim of expanding knowledge and the practice of prevention of these infectious diseases. CONCLUSIONS: Although the vaccines against A and B hepatitis are recommended for various risk groups, estimated vaccine coverage is still modest and many vaccination opportunities are lost. In order to reduce the incidence of A and B hepatitis, which are preventable by vaccines, it is necessary for physicians to encourage their patients to be vaccinated.
Published: 2006
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12. Seroprevalence of hepatitis A antibodies in a group of normal and Down Syndrome children in Porto Alegre, Southern Brazil

Author: Cristina Targa Ferreira, Júlio César Leite, Adriano Nori R. Taniguchi, Sandra Maria G. Vieira, Jorge Pereira-Lima, and Themis Reverbel da Silveira
Subjects: Down syndrome, hepatitis A, seroprevalence, Infectious and parasitic diseases, RC109-216, Microbiology, QR1-502
Abstract: The high incidence of Hepatitis A and B in institutionalized patients with Down Syndrome (DS) is not fully understood. Under poor hygienic conditions, immunological alterations might predispose individuals to these infections. Sixty three DS children between 1 and 12 years old living at home with their families were examined for anti-HAV and compared to age-matched controls (64 healthy children). This cross-sectional study was carried out from May, 1999, to April, 2000, at the Hospital de Clínicas of Porto Alegre, southern Brazil. Groups were compared in terms of age, sex, skin color, and family income (> R$ 500 and < R$ 500/ month) by the chi-square test, with Yates' correction and for the prevalence of anti-HAV (Fisher's exact test). In the DS group (n=63), the mean age was 4.4 ± 3.3 years, 94% of the patients were white and 51% were female. Family income was
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13. Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study

Author: Shannon M, Vandriel, Li-Ting, Li, Huiyu, She, Jian-She, Wang, Melissa A, Gilbert, Irena, Jankowska, Piotr, Czubkowski, Dorota, Gliwicz-Miedzińska, Emmanuel M, Gonzales, Emmanuel, Jacquemin, Jérôme, Bouligand, Nancy B, Spinner, Kathleen M, Loomes, David A, Piccoli, Lorenzo, D'Antiga, Emanuele, Nicastro, Étienne, Sokal, Tanguy, Demaret, Noelle H, Ebel, Jeffrey A, Feinstein, Rima, Fawaz, Silvia, Nastasio, Florence, Lacaille, Dominique, Debray, Henrik, Arnell, Björn, Fischler, Susan, Siew, Michael, Stormon, Saul J, Karpen, Rene, Romero, Kyung Mo, Kim, Woo Yim, Baek, Winita, Hardikar, Sahana, Shankar, Amin J, Roberts, Helen M, Evans, M Kyle, Jensen, Marianne, Kavan, Shikha S, Sundaram, Alexander, Chaidez, Palaniswamy, Karthikeyan, Maria Camila, Sanchez, Maria Lorena, Cavalieri, Henkjan J, Verkade, Way Seah, Lee, James E, Squires, Christina, Hajinicolaou, Chatmanee, Lertudomphonwanit, Ryan T, Fischer, Catherine, Larson-Nath, Yael, Mozer-Glassberg, Cigdem, Arikan, Henry C, Lin, Jesus Quintero, Bernabeu, Seema, Alam, Deirdre A, Kelly, Elisa, Carvalho, Cristina Targa, Ferreira, Giuseppe, Indolfi, Ruben E, Quiros-Tejeira, Pinar, Bulut, Pier Luigi, Calvo, Zerrin, Önal, Pamela L, Valentino, Dev M, Desai, John, Eshun, Maria, Rogalidou, Antal, Dezsőfi, Sabina, Wiecek, Gabriella, Nebbia, Raquel Borges, Pinto, Victorien M, Wolters, María Legarda, Tamara, Andréanne N, Zizzo, Jennifer, Garcia, Kathleen, Schwarz, Marisa, Beretta, Thomas Damgaard, Sandahl, Carolina, Jimenez-Rivera, Nanda, Kerkar, Jernej, Brecelj, Quais, Mujawar, Nathalie, Rock, Cristina Molera, Busoms, Wikrom, Karnsakul, Eberhard, Lurz, Ermelinda, Santos-Silva, Niviann, Blondet, Luis, Bujanda, Uzma, Shah, Richard J, Thompson, Bettina E, Hansen, Binita M, Kamath, Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), Vandriel, S.M., Li, L.T., She, H., Wang, J.S., Gilbert, M.A., Jankowska, I., Czubkowski, P., Gliwicz-Miedzi?ska, D., Gonzales, E.M., Jacquemin, E., Bouligand, J., Spinner, N.B., Loomes, K.M., Piccoli, D.A., D'Antiga, L., Nicastro, E., Sokal, É., Demaret, T., Ebel, N.H., Feinstein, J.A., Fawaz, R., Nastasio, S., Lacaille, F., Debray, D., Arnell, H., Fischler, B., Siew, S., Stormon, M., Karpen, S.J., Romero, R., Kim, K.M., Baek, W.Y., Hardikar, W., Shankar, S., Roberts, A.J., Evans, H.M., Jensen, M.K., Kavan, M., Sundaram, S.S., Chaidez, A., Karthikeyan, P., Sanchez, M.C., Cavalieri, M.L., Verkade, H.J., Lee, W.S., Squires, J.E., Hajinicolaou, C., Lertudomphonwanit, C., Fischer, R.T., Larson-Nath, C., Mozer-Glassberg, Y., Lin, H.C., Quintero, Bernabeu J., Alam, S., Kelly, D., Carvalho, E., Ferreira, C.T., Indolfi, G., Quiros-Tejeira, R.E., Bulut, P., Calvo, P.L., Önal, Z., Valentino, P.L., Desai, D.M., Eshun, J., Rogalidou, M., Dezs?fi, A., Wiecek, S., Nebbia, G., Borges Pinto, R., Wolters, V.M., Tamara, M.L., Zizzo, A.N., Garcia, J., Schwarz, K., Beretta, M., Sandahl, T.D., Jimenez-Rivera, C., Kerkar, N., Brecelj, J., Mujawar, Q., Rock, N., Busoms, C.M., Karnsakul, W., Lurz, E., Santos-Silva, E., Blondet, N., Bujanda, L., Shah, U., Thompson, R.J., Hansen, B.E., Kamath, B.M., Global ALagille Alliance (GALA) Study Group, Koç University Hospital, School of Medicine, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique
Subjects: Male, Cholestasis, Alagille syndrome, Bile duct atresia, Intrahepatic cholestasis, Hepatology, Hypertension, Portal/etiology, Gastroenterology and hepatology, Alagille Syndrome/epidemiology, Humans, Female, Child, Retrospective Studies
Abstract: Background and aims: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international cohort of children with ALGS. Approach and results: This was a multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born between January 1997 and August 2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS. In total, 1433 children (57% male) from 67 centers in 29 countries were included. The 10 and 18-year NLS rates were 54.4% and 40.3%. By 10 and 18 years, 51.5% and 66.0% of children with ALGS experienced >= 1 adverse liver-related event (CEPH, transplant, or death). Children (>6 and = 5.0 and = 10.0 mg/dl had an 8.0-fold (95% CI, 3.4-18.4) increased risk of developing CEPH compared with those 10.0 mg/dl were associated with a 4.8 (95% CI, 2.4-9.7) and 15.6 (95% CI, 8.7-28.2) increased risk of transplantation relative to = 5.0 mg/dl, with 79% reaching adulthood with native liver (p < 0.001). Conclusions: in this large international cohort of ALGS, only 40.3% of children reach adulthood with their native liver. A TB, This study received funding support from the following agencies: The Alagille Syndrome Alliance, Mirum Pharmaceuticals Inc. and Albireo Pharma, Inc. who provided unrestricted educational grants to the Hospital for Sick Children (SickKids Foundation). The study sponsors were not involved in the conduct of the research study or preparation of the manuscript.
Published: 2022

14. Global multi-stakeholder endorsement of the MAFLD definition

Author: Nahum Méndez-Sánchez, Elisabetta Bugianesi, Robert G Gish, Frank Lammert, Herbert Tilg, Mindie H Nguyen, Shiv K Sarin, Núria Fabrellas, Shira Zelber-Sagi, Jian-Gao Fan, Gamal Shiha, Giovanni Targher, Ming-Hua Zheng, Wah-Kheong Chan, Shlomo Vinker, Takumi Kawaguchi, Laurent Castera, Yusuf Yilmaz, Marko Korenjak, C Wendy Spearman, Mehmet Ungan, Melissa Palmer, Mortada El-Shabrawi, Hans-Juergen Gruss, Jean-François Dufour, Anil Dhawan, Heiner Wedemeyer, Jacob George, Luca Valenti, Yasser Fouad, Manuel Romero‐Gomez, Mohammed Eslam, Maria Lorena Abate, Bahaa Abbas, Ahmed Amr Abbassy, Waleed Abd El Ghany, Amira Abd Elkhalek, Emad Abd ElMajeed, Mohammad Abdalgaber, Mohamed AbdAllah, Marwa Abdallah, Nourhan Abdallah, Shereen Abdelaleem, Yasser Abdelghani, Wael Abdelghany, Safaa Mohamed Abdelhalim, Wafaa Abdelhamid, Nehal Abdelhamid, Nadia A. Abdelkader, Elsayed Abdelkreem, Aly Mohamed Abdelmohsen, Awny Ali Abdelrahman, Sherief M Abd-elsalam, Doaa Abdeltawab, Abdulbaset Abduh, Nada Abdulhakam, Maheeba Abdulla, Navid Abedpoor, Ludovico Abenavoli, Fredrik Åberg, Omala Ablack, Mostafa Abo elftouh, Yousry Esam-Eldin Abo-Amer, Ashraf Aboubkr, Alaa Aboud, Amr M. Abouelnaga, Galal A. Aboufarrag, Ashraf Aboutaleb, Leticia Abundis, Gupse Adalı, Enrique Adames, Leon Adams, Danjuma Adda, Noor Adel, Nada Adel, Muhammad Adel Sayed, Taiba Jibril Afaa, Nawal Afredj, Gulnara Aghayeva, Alessio Aghemo, Carlos A. Aguilar-Salinas, Golo Ahlenstiel, Walid Ahmady, Wafaa Ahmed, Amira Ahmed, Samah Nasser Ahmed, Heba Mostafa Ahmed, Rasha Ahmed, Elmar Aigner, Mesut Akarsu, Maisam Akroush, Umit Akyuz, Mamun Al Mahtab, Tahani Al Qadiri, Yusriya Al Rawahi, Razzaq AL rubaee, Muna Al Saffar, Shahinul Alam, Zaid Al-Ani, Agustín Albillos, Mohamed Alboraie, Said Al-Busafi, Mohamed Al-Emam, Jawaher Alharthi, Kareem Ali, Basma Abdelmoez Ali, Mohammad Ali, Raja Affendi Raja Ali, Anna Alisi, Ali Raad AL-Khafaji, Maryam Alkhatry, Rocio Aller, Yahya Almansoury, Khalid Al-Naamani, Alaa Alnakeeb, Anna Alonso, Saleh A. Alqahtani, Leina Alrabadi, Khalid Alswat, Mahir Altaher, Turki Altamimi, Jose Altamirano, Mario R. Alvares-da-Silva, Elsragy Adel M. Aly, Amgad Alzahaby, Ahmed Alzamzamy, Keisuke Amano, Maysa A. Amer, Mona A. Amin, Sayed A. Amin, Ashraf A. Amir, Javier Ampuero, Noha Anas, Pietro Andreone, Soa Fy Andriamandimby, Mahmoud Anees, Peltec Angela, Manal Antonios, Wael Arafat, Jose Moreno Araya, Juan Armendariz-Borunda, Matthew J. Armstrong, Hassan Ashktorab, Patricia Aspichueta, Fathia Assal, Mira Atef, Dina Attia, Hoda Atwa, Reham Awad, Mohyeldeen Abd Elaziz Awad, Sally Awny, Obafemi Awolowo, Yaw Asante Awuku, Ibrahim Ayada, Than Than Aye, Sherif Ayman, Hedy Ayman, Hesham Ayoub, Hosny M. Azmy, Romiro P. Babaran, Omneya Badreldin, Ahmed Badry, İbrahim Halil Bahçecioğlu, Amira Bahour, Jiajia Bai, Yasemin Balaban, Muthuswamy Balasubramanyam, Khaled Bamakhrama, Jesus M Banales, Babu Bangaru, Jianfeng Bao, Jorge Suazo Barahona, Salma Barakat, Sandra Maria Barbalho, Bikwa Barbra, Beatriz Barranco, Francisco Barrera, Ulrich Baumann, Shamardan Bazeed, Eva Bech, Aourarh Benayad, Andreas Benesic, David Bernstein, Fernando Bessone, Susie Birney, Cyrille Bisseye, Martin Blake, Bilal Bobat, Leonilde Bonfrate, Dmitry S Bordin, Francisco Bosques-Padilla, Jerome Boursier, Boushab Mohamed Boushab, David Bowen, Patricia Medina Bravo, Paul N Brennan, Bisi Bright, Ilse Broekaert, Xabier Buque, Diego Burgos-Santamaría, Julio Burman, Luca Busetto, Chris D. Byrne, Patricia Anne I. Cabral-Prodigalidad, Guillermo Cabrera-Alvarez, Wei Cai, Francesca Cainelli, Ali Riza Caliskan, Ali Canbay, Ana Cano-Contreras, Hai-Xia Cao, Zhujun Cao, Andres Carrion, Francesca Carubbi, Teresa Casanovas, Marlen Ivón Castellanos Fernández, Jin Chai, Siew Pheng Chan, Phunchai Charatcharoenwitthaya, Norberto Chavez-Tapia, Kazuaki Chayama, Jinjun Chen, Lin Chen, Zhong-Wei Chen, Huiting Chen, Sui-Dan Chen, Qiang Chen, Yaxi Chen, Gang Chen, En-Quang Chen, Fei Chen, Pei-Jer Chen, Robert Cheng, Wendy Cheng, Jack Tan Wei Chieh, Imad Chokr, Evangelos Cholongitas, Ashok Choudhury, Abhijit Chowdhury, Evaristus Sunday Chukwudike, Stefano Ciardullo, Michelle Clayton, Karine Clement, Marie Michelle Cloa, Cecilia Coccia, Cristina Collazos, Massimo Colombo, Arif Mansur Cosar, Helma Pinchemel Cotrim, Joris Couillerot, Alioune Coulibaly, Gonzalo Crespo, Javier Crespo, Maria Cruells, Ian Homer Y. Cua, Hesham K. Dabbous, George N Dalekos, Patricia D'Alia, Li Dan, Viet Hang Dao, Mostafa Darwish, Christian Datz, Milagros B Davalos-Moscol, Heba Dawoud, Blanca Olaechea de Careaga, Robert de Knegt, Victor de Ledinghen, Janaka de Silva, Nabil Debzi, Marie Decraecker, Elvira Del Pozo, Teresa C Delgado, Manuel Delgado-Blanco, Łukasz Dembiński, Adilson Depina, Moutaz Derbala, Hailemichael Desalegn, Christèle Desbois-Mouthon, Mahmoud Desoky, Anouk Dev, Agostino Di Ciaula, Moisés Diago, Ibrahima Diallo, Luis Antonio Díaz, Melisa Dirchwolf, Paola Dongiovanni, Andrriy Dorofeyev, Xiaoguang Dou, Mark W. Douglas, Michael Doulberis, Cecil K. Dovia, Adam Doyle, Ivana Dragojević, Joost PH Drenth, Xuefei Duan, Audrius Dulskas, Dan L Dumitrascu, Oliver Duncan, Vincent Dusabejambo, Rev. Shem N.A. Dwawhi, Sho Eiketsu, Doaa El Amrousy, Ahmed El Deeb, Ghada El Deriny, Hesham Salah El Din, Salwa El Kamshishy, Mohamed El Kassas, Maissa El Raziky, Osama A Elagamy, Wafaa Elakel, Dina Elalfy, Hanaa Elaraby, Heba ElAwady, Reda Elbadawy, Hanaa Hassan Eldash, Manal S. Eldefrawy, Carol Lezama Elecharri, Amel Elfaramawy, Mohammed Elfatih, Mahmoud Elfiky, Mohamed Elgamsy, Mohamed Elgendy, Mohamed A. El-Guindi, Nagi Elhussieny, Ahmed Maher Eliwa, Zeineb Elkabbany, Hesham El-Khayat, Nehal M. El-Koofy, Alaa Elmetwalli, Amr Elrabat, Fathiya El-Raey, Fatma Elrashdy, Medhat Elsahhar, Esraa M. Elsaid, Shimaa Elsayed, Hany Elsayed, Aly Elsayed, Amr M. Elsayed, Hamdy Elsayed, Magdy El-Serafy, Ahmed M. Elsharkawy, Reem Yehia Elsheemy, Eman Elsayed Elshemy, Sara Elsherbini, Naglaa Eltoukhy, Reda Elwakil, Ola Emad, Shaimaa Emad, Mohamed Embabi, Ilkay Ergenç, Tatiana Ermolova, Gamal Esmat, Doaa M. Esmat, Enrique Carrera Estupiñan, Said Ettair, Tcaciuc Eugen, Mohammed Ezz-Eldin, Lidia Patricia Valdivieso Falcón, Yu-Chen Fan, Samah Fandari, Mahmoud Farag, Taghreed Mohamed Farahat, Eman M. Fares, Michael Fares, Eduardo Fassio, Hayam Fathy, Dina Fathy, Wael Fathy, Soheir Fayed, Dan Feng, Gong Feng, Miguel Fernández-Bermejo, Cristina Targa Ferreira, Javier Díaz Ferrer, Alastair Forbes, Rabab Fouad, Hanan M. Fouad, Tove Frisch, Hideki Fujii, Shuhei Fukunaga, Shinya Fukunishi, Hacer Fulya, Masato Furuhashi, Yasmine Gaber, Augusto Jose G. Galang, Jacqueline Cordova Gallardo, Rocío Galloso, Mahmoud Gamal, Reham Gamal, Hadeel Gamal, Jian Gan, Anar Ganbold, Xin Gao, George Garas, Tony Garba, Miren García-Cortes, Carmelo García-Monzón, Javier García-Samaniego, Amalia Gastaldelli, Manuel Gatica, Elizabeth Gatley, Tamar Gegeshidze, Bin Geng, Hasmik Ghazinyan, Salma Ghoneem, Luca Giacomelli, Gianluigi Giannelli, Edoardo G. Giannini, Matthew Giefer, Pere Ginès, Marcos Girala, Pablo J Giraudi, George Boon-Bee Goh, Ahmed Ali Gomaa, Benbingdi Gong, Dina Hilda C. Gonzales, Humberto C. Gonzalez, Maria Saraí Gonzalez-Huezo, Isabel Graupera, Ivica Grgurevic, Henning Grønbæk, Xuelian Gu, Lin Guan, Ibrahima Gueye, Alice Nanelin Guingané, Ozen Oz Gul, Cuma Bulent Gul, Qing Guo, Pramendra Prasad Gupta, Ahmet Gurakar, Juan Carlos Restrepo Gutierrez, Ghada Habib, Azaa Hafez, Emilia Hagman, Eman Halawa, Osama Hamdy, Abd Elkhalek Hamed, Dina H. Hamed, Saeed Hamid, Waseem Hamoudi, Yu Han, James Haridy, Hanan Haridy, David C H Harris Harris, Michael Hart, Fuad Hasan, Almoutaz Hashim, Israa Hassan, Ayman Hassan, Essam Ali Hassan, Adel Ahmed Hassan, Magda Shehata Hassan, Fetouh Hassanin, Alshymaa Hassnine, John Willy Haukeland, Amr Ismael M. Hawal, Jinfan He, Qiong He, Yong He, Fang-Ping He, Mona Hegazy, Adham Hegazy, Osama Henegil, Nelia Hernández, Manuel Hernández-Guerra, Fatima Higuera-de-la-Tijera, Ibrahim Hindy, Keisuke Hirota, Lee Chi Ho, Alexander Hodge, Mohamed Hosny, Xin Hou, Jiao-Feng Huang, Yan Huang, Zhifeng Huang, Yuan Huang, Ang Huang, Xiao-Ping Huang, Sheng Hui-ping, Bela Hunyady, Mennatallah A. Hussein, Osama Hussein, Shahinaz Mahmoud Hussien, Luis Ibáñez-Samaniego, Jamal Ibdah, Luqman Ibrahim, Miada Ibrahim, Ibrahim Ibrahim, Maria E. Icaza-Chávez, Sahar Idelbi, Ramazan Idilman Idilman, Mayumi Ikeda, Giuseppe Indolfi, Federica Invernizzi, Iram Irshad, Hasan Mohamed Ali Isa, Natacha Jreige Iskandar, Abdulrahman Ismaiel, Mariam Ismail, Zulkifli Ismail, Faisal Ismail, Hideki Iwamoto, Kathryn Jack, Rachael Jacob, Fuad Jafarov, Wasim Jafri, Helen Jahshan, Prasun K Jalal, Ligita Jancoriene, Martin Janicko, Hiruni Jayasena, Meryem Jefferies, Vivekanand Jha, Fanpu Ji, Yaqiu Ji, Jidong Jia, Changtao Jiang, Ni Jiang, Zong-zhe Jiang, Xing Jin, Yi Jin, Xu Jing, Qian Jingyu, Maia Jinjolava, FX Himawan Haryanto Jong, Alina Jucov, Ibecheole Julius, Mona Kaddah, Yoshihiro Kamada, Abobakr kamal, Enas Mohamed Kamal, Ashraf Sayed Kamel, Jia-Horng Kao, Maja Karin, Thomas Karlas, Mohammad Kashwaa, Leolin Katsidzira, Eda Kaya, M.Azzam Kayasseh, Bernadette Keenan, Caglayan Keklikkiran, William Keml, Deia K. Khalaf, Rofida Khalefa, Sherin Khamis, Doaa Khater, Hamed khattab, Anatoly Khavkin, Olga Khlynova, Nabil Khmis, Nazarii Kobyliak, Apostolos Koffas, Kazuhiko Koike, Kenneth Y.Y. Kok, Tomas Koller, Narcisse Patrice Komas, Nataliya V. Korochanskaya, Yannoula Koulla, Shunji Koya, Colleen Kraft, Bledar Kraja, Marcin Krawczyk, Mohammad Shafi Kuchay, Anand V Kulkarni, Ashish Kumar, Manoj Kumar, Sulaiman Lakoh, Philip Lam, Ling Lan, Naomi F. Lange, Kamran Bagheri Lankarani, Nicolas Lanthier, Kateryna Lapshyna, Sameh A. Lashen, Konang Nguieguia Justine Laure, Leonid Lazebnik, Didier Lebrec, Samuel S. Lee, Way Seah Lee, Yeong Yeh Lee, Diana Julie Leeming, Nathalie Carvalho Leite, Roberto Leon, Cosmas Rinaldi Adithya Lesmana, Junfeng Li, Qiong Li, Jun Li, Yang-Yang Li, Yufang Li, Lei Li, Min Li, Yiling li, Huiqing Liang, Tang Lijuan, Seng Gee Lim, Lee-Ling Lim, Shumei Lin, Han-Chieh Lin, Rita Lin, Rania Lithy, Yaru Liu, Yuanyuan Liu, Xin Liu, Wen-Yue Liu, Shourong Liu, Ken Liu, Tian Liu, Amedeo Lonardo, Mariana Bravo López, Eva López-Benages, Patricio Lopez-Jaramillo, Huimin Lu, Lun Gen Lu, Yan Lu, John Lubel, Rashid Lui, Iulianna Lupasco, Elena Luzina, Xiao-Hui Lv, Kate Lynch, Hong-Lei Ma, Mariana Verdelho Machado, Nonso Maduka, Katerina Madzharova, Russellini Magdaong, Sanjiv Mahadeva, Amel Mahfouz, Nik Ritza Kosai Nik Mahmood, Eman Mahmoud, Mohamed Mahrous, Rakhi Maiwall, Ammar Majeed, Avik Majumdar, Loey Mak, Madiha M Maklouf, Reza Malekzadeh, Claudia Mandato, Alessandra Mangia, Jake Mann, Hala Hussien Mansour, Abdellah Mansouri, Alessandro Mantovani, Jun qian Mao, Flor Maramag, Giulio Marchesini, Claude Marcus, Rui António Rocha Tato Marinho, Maria L Martinez-Chantar, Antonieta A. Soares Martins, Rana Marwan, Karen Frances Mason, Ghadeer Masoud, Mohamed Naguib Massoud, Maria Amalia Matamoros, Rosa Martín Mateos, Asmaa Mawed, Jean Claude Mbanya, Charles Mbendi, Lone McColaugh, Duncan McLeod, Juan Francisco Rivera Medina, Ahmed Megahed, Mai Mehrez, Iqbal Memon, Shahin Merat, Randy Mercado, Ahmed Mesbah, Taoufik Meskini, Mayada Metwally, Rasha Metwaly, Lei Miao, Eileen Micah, Luca Miele, Vladimir Milivojevic, Tamara Milovanovic, Yvonne L. Mina, Milan Mishkovik, Amal Mishriki, Tim Mitchell, Alshaimaa Mohamed, Mona Mohamed, Sofain Mohamed, Shady Mohammed, Ahmed Mohammed, Viswanathan Mohan, Sara Mohie, Aalaa Mokhtar, Reham Moniem, Mabel Segura Montilla, Jose Antonio Orozco Morales, María María Sánchez Morata, Jose Maria Moreno-Planas, Silvia Morise, Sherif Mosaad, Mohamed Moselhy, Alaa Mohamed Mostafa, Ebraheem Mostafa, Nezha Mouane, Nasser Mousa, Hamdy Mahfouz Moustafa, Abeer Msherif, Kate Muller, Christopher Munoz, Ana Beatriz Muñoz-Urribarri, Omar Alfaro Murillo, Feisul Idzwan Mustapha, Emir Muzurović, Yehia Nabil, Shaymaa Nafady, Ayu Nagamatsu, Atsushi Nakajima, Dan Nakano, Yuemin Nan, Fabio Nascimbeni, Mirella S. Naseef, Nagwa Nashat, Taran Natalia, Francesco Negro, Alexander V. Nersesov, Manuela Neuman, Masolwa Ng'wanasayi, Yan Ni, Amanda Nicoll, Takashi Niizeki, Dafina Nikolova, Wang Ningning, Madunil Niriella, K.A Nogoibaeva, Rozeena Nordien, Catherine O Sullivan, James O'Beirne, Solomon Obekpa, Ponsiano Ocama, Missiani Ochwoto, Michael Promise Ogolodom, Olusegun Ojo, Nana Okrostsvaridze, Claudia P. Oliveira, Raul Contreras Omaña, Omneya M. Omar, Hanaa Omar, Mabroka Omar, Salma Omran, Reham Omran, Marian Muse Osman, Nevin Owise, Theobald Owusu-Ansah, P. Martín Padilla- Machaca, Sirish Palle, Ziyan Pan, Xiao-Yan Pan, Qiuwei Pan, Apostolis Papaefthymiou, Feliciano Chanana Paquissi, Gabriella Par, Arit Parkash, Diana Payawal, Kevork M. 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Xu, A, Xu, C, Yan, H, Yang, J, Yang, R, Yang, Y, Yang, Q, Yang, N, Yao, J, Yara, J, Yaras, S, Yilmaz, N, Younes, R, Younes, H, Young, S, Youssef, F, Yu, Y, Yu, M, Yuan, J, Yue, Z, Yuen, M, Yun, W, Yurukova, N, Zakaria, S, Zaky, S, Zaldastanishvili, M, Zapata, R, Zare, N, Zerem, E, Zeriban, N, Zeshuai, X, Zhang, H, Zhang, X, Zhang, Y, Zhang, W, Zhang, Z, Zhao, J, Zhao, R, Zhao, H, Zheng, C, Zheng, Y, Zheng, R, Zheng, T, Zheng, K, Zhou, X, Zhou, Y, Zhou, H, Zhou, L, Zhu, L, Zhu, Y, Zhu, P, Ziada, E, Ziring, D, Ziyi, L, Zou, S, Zou, Z, Zou, H, Zuart Ruiz, R, and Global Multi-Stakeholder Consensus on the Redefinition of Fatty Liver Disease
Subjects: Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Hepatology, Non-alcoholic Fatty Liver Disease, NAFLD, consensu, Gastroenterology, MAFLD, definition, Humans, MAFLD, NAFLD, Human medicine
Abstract: Contains fulltext : 252162.pdf (Publisher’s version ) (Closed access)
Published: 2022

15. Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency

Author: Liting Li, Jian-She Wang, Ozlem Durmaz, Felipe Ordonez, Wikrom Karnsakul, Seema Alam, Cristina Targa Ferreira, Natural Course, Bettina E. Hansen, Kyung Mo Kim, Etienne Sokal, Ryan T. Fischer, Valerie Sency, Estella M. Alonso, Simon Horslen, Elisa de Carvalho, Piotr Czubkowski, Emanuele Nicastro, Nanda Kerkar, Dieter C. Broering, Björn Fischler, Dorothee Krebs-Schmitt, Kathleen M. Loomes, Dominique Debray, Marianne Hørby Jørgensen, Benjamin L. Shneider, Emmanuel Gonzales, Cigdem Arikan, Nathalie Rock, Antal Dezsőfi, Tassos Grammatikopoulos, Steffen Hartleif, Mara Cananzi, Talal Algoufi, Ronald J. Sokol, Jan B F Hulscher, Carolina Jimenez-Rivera, Emmanuel Jacquemin, Anne Spraul, Henkjan J. Verkade, Patryk Lipiński, Daan B E van Wessel, Nejat Mazhar, Maria Rogalidou, Deirdre Kelly, Alexandre Fabre, Daniele Serranti, Pier Luigi Calvo, Yael Mozer-Glassberg, Richard J. Thompson, Cristina Goncalves, Yumirle P. Turmelle, Jesus Quintero Bernabeu, Agustina Kadaristiana, Florence Lacaille, Loreto Hierro, Mohammad Shagrani, Patrick J. McKiernan, Girish S. Rao, Gema Muñoz Bartolo, Huey-Ling Chen, Wendy L. van der Woerd, Irena Jankowska, Amer Azaz, Philip J. Rosenthal, Frederick J. Suchy, Jernej Brecelj, Gabriella Nebbia, Noemie Laverdure, Henrik Arnell, Binita M. Kamath, Heng Wang, Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), van Wessel, D. B. E., Thompson, R. J., Gonzales, E., Jankowska, I., Shneider, B. L., Sokal, E., Grammatikopoulos, T., Kadaristiana, A., Jacquemin, E., Spraul, A., Lipiński, P., Czubkowski, P., Rock, N., Shagrani, M., Broering, D., Algoufi, T., Mazhar, N., Nicastro, E., Kelly, D., Nebbia, G., Arnell, H., Fischler, B., Hulscher, J. B. F., Serranti, D., Debray, D., Lacaille, F., Goncalves, C., Hierro, L., Muñoz Bartolo, G., Mozer-Glassberg, Y., Azaz, A., Brecelj, J., Dezsőfi, A., Luigi Calvo, P., Krebs-Schmitt, D., Hartleif, S., van der Woerd, W. L., Wang, J. S., Li, L. T., Durmaz, Ö., Kerkar, N., Hørby Jørgensen, M., Fischer, R., Jimenez-Rivera, C., Alam, S., Cananzi, M., Laverdure, N., Ferreira, C. T., Ordonez, F., Wang, H., Sency, V., Kim, K. M., Chen, H. L., Carvalho, E., Fabre, A., Quintero Bernabeu, J., Alonso, E. M., Sokol, R. J., Suchy, F. J., Loomes, K. M., McKiernan, P. J., Rosenthal, P., Turmelle, Y., Rao, G. S., Horslen, S., Kamath, B. M., Rogalidou, M., Karnsakul, W. W., Hansen, B., Verkade, H. J., Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM), School of Medicine, Center for Liver, Digestive and Metabolic Diseases (CLDM), UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique
Subjects: 0301 basic medicine, Male, [SDV]Life Sciences [q-bio], CHILDREN, Compound heterozygosity, Gastroenterology, PFIC1, DISEASE, 0302 clinical medicine, Genotype, Medicine, Prospective Studies, Prospective cohort study, Child, ComputingMilieux_MISCELLANEOUS, Adenosine Triphosphatases, ATP8B1 GENE, Bile acid, Progressive familial intrahepatic cholestasis, Prognosis, Treatment Outcome, Codon, Nonsense, Child, Preschool, Cohort, 030211 gastroenterology & hepatology, Female, Original Article, EXPRESSION, medicine.medical_specialty, Adolescent, medicine.drug_class, Cholestasis, Intrahepatic, Risk Assessment, Frameshift mutation, Bile Acids and Salts, 03 medical and health sciences, Young Adult, FAMILIAL INTRAHEPATIC CHOLESTASIS, Internal medicine, Humans, ABCB11 MUTATIONS, Survival analysis, TYPE-1, ATP8B1, FIC1 deficiency, Serum bile acids, Surgical biliary diversion, Retrospective Studies, [SDV.GEN]Life Sciences [q-bio]/Genetics, Hepatology, business.industry, Infant, Original Articles, medicine.disease, Survival Analysis, Liver Transplantation, SALT EXPORT PUMP, 030104 developmental biology, Bile Ducts, Intrahepatic, Autoimmune, Cholestatic and Biliary Disease, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Follow-Up Studies
Abstract: Mutations in ATP8B1 can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive familial intrahepatic cholestasis type 1 (PFIC1). The rarity of FIC1 deficiency has largely prevented a detailed analysis of its natural history, effects of predicted protein truncating mutations (PPTMs), and possible associations of serum bile acid (sBA) concentrations and surgical biliary diversion (SBD) with long-term outcome. We aimed to provide novel insights by using the largest genetically defined cohort of FIC1 deficiency patients to date. This multicenter, combined retrospective and prospective study included 130 patients with compound heterozygous or homozygous predicted pathogenic ATP8B1 variants. Patients were categorized according to the number of PPTMs (i.e., splice site, frameshift due to deletion or insertion, nonsense, duplication); FIC1-A (n=67; no PPTM), FIC1-B (n=29; one PPTM) or FIC1-C (n=34; two PPTMs). Survival analysis showed an overall native liver survival (NLS) of 44% at age 18y. NLS was comparable between FIC1-A, FIC1-B, and FIC1-C (%NLS at age 10y: 67%, 41%, and 59%, respectively; P=0.12), despite FIC1-C undergoing SBD less often (%SBD at age 10y: 65%, 57%, and 45%, respectively; P=0.03). sBAs at presentation were negatively associated with NLS (NLS at age 10y; sBAs, University of Colorado; Baylor College of Medicine; Children’s Hospital of Philadelphia; Children’s Hospital of Pittsburgh; St Louis Children’s Hospital; Riley Hospital for Children; Seattle Children’s Hospital; M.D./Ph.D. Scholarship from the University of Groningen; European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Networking Grant 2019; ChilDReN National Institutes of Health Grants; Ann & Robert H. Lurie Children’s Hospital; Albireo and Mirum Pharmaceuticals
Published: 2021

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

Author: Melina Utz Melere, Flávia Heinz Feier, Alex Finger Horbe, Fabio Tonet, Cristine Suzana Trein, Antonio Nocchi Kalil, Angélica Maria Lucchese, Marcio F. Chedid, Claudia Pires Ricachinevsky, Carolina Soares da Silva, and Cristina Targa Ferreira
Subjects: Transplantation, medicine.medical_specialty, business.industry, medicine.medical_treatment, 030232 urology & nephrology, 030230 surgery, Liver transplantation, Revascularization, medicine.disease, Thrombosis, Rescue treatment, Surgery, 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, Pediatrics, Perinatology and Child Health, medicine, In patient, Complication, business, Surgical revascularization, Artery
Abstract: 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.
Published: 2020

17. Non-Alcoholic Fatty Liver Disease in Children and Adolescents: Lifestyle Change - a Systematic Review and Meta-Analysis

Author: Melina Utz-Melere, Matias Epifanio, Marialena Mouzaki, Cristina Targa-Ferreira, Bernardo Lessa-Horta, and Angelo Alves de Mattos
Subjects: Male, Health Knowledge, Attitudes, Practice, Pediatric Obesity, medicine.medical_specialty, Hepatic steatosis, Adolescent, Health Status, Health Behavior, MEDLINE, Child Behavior, Specialties of internal medicine, Disease, Body Mass Index, 03 medical and health sciences, 0302 clinical medicine, Non-alcoholic Fatty Liver Disease, Risk Factors, Internal medicine, Nonalcoholic fatty liver disease, Humans, Medicine, Healthy Lifestyle, 030212 general & internal medicine, Obesity, Child, Exercise, Transaminases, Pediatric, Nutritional intervention, Hepatology, business.industry, Physical activity, Fatty liver, General Medicine, medicine.disease, Treatment Outcome, RC581-951, Adolescent Behavior, Meta-analysis, Female, 030211 gastroenterology & hepatology, Diet, Healthy, Steatosis, business, Risk Reduction Behavior, Body mass index, Biomarkers
Abstract: Introduction and aim. This manuscript seeks to analyze the impact of lifestyle changes on body mass index (BMI), aminotransferases and steatosis in children and adolescents with nonalcoholic fatty liver disease (NAFLD). Material and methods. A review of PubMed, BIREME, Scopus, EMBASE, Medline and Web of Science databases 2015 was performed seeking studies addressing the impact of lifestyle interventions on children and/or adolescents with NAFLD. Inclusion were manuscripts written in Portuguese, English and Spanish, as well as age less than 18 years. Two reviewers performed the data extraction independently and differences were resolved by consensus. Outcome measures were BMI, serum aminotransferase levels and the presence of hepatic steatosis. Results. The literature search identified 71,012 articles. After excluding 46,397 duplicates and other clearly irrelevant studies, 89 publications were reviewed in detail. Another 55 studies were excluded at this stage. Subsequently, 18 were excluded due to lack of data and three new articles were found in the review of the references of previously identified manuscripts. Therefore, 19 studies that had evaluated 923 subjects (477 boys and 446 girls) aged 6-18 years were included in the review. In most studies, the intervention included aerobic exercise and diet. In nine studies, BMI improved significantly following the intervention. The vast majority of studies reported a benefit from the intervention on aminotransferase levels. Lifestyle changes also had a significant impact on steatosis, reducing the risk by 61%. Conclusion. In conclusion lifestyle changes lead to significant improvements in BMI, aminotransferase levels and hepatic steatosis in children and adolescents with NAFLD.
Published: 2018

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

Author: Jonas de Almeida Rodrigues, Cristiane Meira Assunção, Matias Epifânio, Carolina Soares da Silva, Fernanda Coradini Noal, Luciano Casagrande, Amanda Rodrigues Sari Quoos, Fernando Borba de Araujo, and Cristina Targa Ferreira
Subjects: medicine.medical_specialty, Risk level, business.industry, Reflux, Dentistry, medicine.disease, Tooth wear, Child, Preschool, GERD, Prevalence, Medicine, Esophagitis, Humans, Observational study, Tooth Wear, business, Child, General Dentistry, Erosive esophagitis, Pediatric gastroenterology, Brazil
Abstract: 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.
Published: 2019

19. Consenso Brasileiro sobre Alergia Alimentar: 2018 - Parte 1 - Etiopatogenia, clínica e diagnóstico. Documento conjunto elaborado pela Sociedade Brasileira de Pediatria e Associação Brasileira de Alergia e Imunologia

Author: Norma P. M. Rubini, Roseli Oselka Sacardo Sarni, Neusa Falbo Wandalsen, Cristina Targa Ferreira, Luciana Rodrigues Silva, Hélcio de Sousa Maranhão, Lucila Camargo Oliveira, Maria Luisa Oliva Alonso, Matias Epifanio, José Carlison Oliveira, Ariana Yang, Dirceu Solé, Mauro Sérgio Toporovski, Bruno Paes Barreto, Renata Rodrigues Cocco, Antonio Carlos Pastorino, Virgínia Weffort, Mauro Batista de Morais, Nelson Rosario, Ana Paula Beltran Moschione Castro, Jackeline Motta Franco, Emanuel Cavalcanti Sarinho, and Herberto José Chong Neto
Subjects: General Earth and Planetary Sciences, General Environmental Science
Published: 2018

20. Consenso Brasileiro sobre Alergia Alimentar: 2018 - Parte 2 - Diagnóstico, tratamento e prevenção. Documento conjunto elaborado pela Sociedade Brasileira de Pediatria e Associação Brasileira de Alergia e Imunologia

Author: Maria Luisa Oliva Alonso, Emanuel Cavalcanti Sarinho, Ana Paula Beltran Moschione Castro, Mauro Sérgio Toporovski, Dirceu Solé, Hélcio de Sousa Maranhão, Herberto José Chong Neto, Luciana Rodrigues Silva, Roseli Oselka Sacardo Sarni, Renata Rodrigues Cocco, Ariana Yang, Cristina Targa Ferreira, José Carlison Oliveira, Lucila Camargo Oliveira, Matias Epifanio, Neusa Falbo Wandalsen, Bruno Paes Barreto, Nelson Rosario, Jackeline Motta Franco, Antonio Carlos Pastorino, Mauro Batista de Morais, Virgínia Weffort, and Norma P. M. Rubini
Subjects: General Earth and Planetary Sciences, General Environmental Science
Published: 2018

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

Author: Ellyn Kodroff, Alain M. Schoepfer, Yoshikazu Kinoshita, Philip E. Putnam, David C. Metz, Alex Straumann, Kathryn A. Peterson, Carine Blanchard, Jeffrey A. Alexander, Kelley E. Capocelli, Edaire Cheng, Calies Menard-Katcher, Simon Murch, Michael F. Vaezi, Wendy Book, Vincent A. Mukkada, Seema S. Aceves, Joel E. Richter, Stephen Attwood, Carla M. Davis, Nirmala Gonsalves, Rhonda F. Souza, Alexandra Papadopoulou, Dan Atkins, Hamish Philpott, Christophe Dupont, Mario C. Vieira, Nicholas J. Talley, Peter A Bonis, Noam Zevit, Ranjan Dohil, Jonathan M. Spergel, Guang Yu Yang, Margaret H. Collins, David A. Katzka, Amanda B. Muir, Stephan Miehlke, Marjorie M. Walker, Cristina Targa Ferreira, Carlo Di Lorenzo, Stuart J. Spechler, Yoshikazu Ohtsuka, Mirna Chehade, Joshua B. Wechsler, Glenn T. Furuta, Melissa Scott, Albert J. Bredenoord, Samuel Nurko, Marc E. Rothenberg, Sandeep K. Gupta, Evan S. Dellon, Javed Sheikh, Rok Orel, Jorge Amil Dias, Ting Wen, Gary W. Falk, Chris A. Liacouras, Ikuo Hirano, Barry K. Wershil, Rachel Rosen, Yvan Vandenplas, Nicoleta C. Arva, Adam T. Fox, Javier Molina-Infante, Neil Shah, Mary Jo Strobel, AGEM - Digestive immunity, AGEM - Endocrinology, metabolism and nutrition, Gastroenterology and Hepatology, Growth and Development, Clinical sciences, and Pediatrics
Subjects: medicine.medical_specialty, Consensus, Proton Pump Inhibitor, medicine.drug_class, diagnosis, proton pump inhibitor, Clinical Sciences, MEDLINE, Food Allergies, Proton-pump inhibitor, gastroenterology, Article, Paediatrics and Reproductive Medicine, 03 medical and health sciences, eosinophilic esophagitis, 0302 clinical medicine, Esophageal Eosinophilia, Predictive Value of Tests, Clinical Research, Diagnosis, medicine, Eosinophilia, Humans, Eosinophilic esophagitis, Intensive care medicine, Gastroenterology & Hepatology, business.industry, Gastroenterology, Neurosciences, Proton Pump Inhibitors, Eosinophilic Esophagitis, medicine.disease, Diagnostic strategy, Prognosis, Diagnostic Techniques, 030220 oncology & carcinogenesis, Predictive value of tests, esophageal eosinophilia, hepatology, Research studies, GERD, 030211 gastroenterology & hepatology, medicine.symptom, business, Digestive Diseases, Digestive System, Algorithms
Abstract: BACKGROUND AND 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 in order 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. Pediatric and adult physicians and researchers from gastroenterology, allergy, and pathology subspecialties representing 14 countries utilized on-line communications, teleconferences, and a face-to-face meeting to review the literature and clinical experiences. RESULTS: Substantial evidence documented that PPIs reduce esophageal eosinophilia in children, adolescents and adults, with several mechanisms potentially explaining the treatment effect. Based on these findings, an updated diagnostic algorithm for EoE was developed, with removal of the PPI trial requirement. CONCLUSIONS: EoE should be diagnosed when there are symptoms of esophageal dysfunction and at least 15 eosinophils per high-power field (or ~60 eosinophils per mm(2)) on esophageal biopsy, and after a comprehensive assessment of non-EoE disorders that could cause or potentially contribute to esophageal eosinophilia. The evidence suggests that PPIs are better classified as a treatment for esophageal eosinophilia that may be due to EoE than as a diagnostic criterion, and we have developed updated consensus criteria for EoE that reflect this change.
Published: 2018

22. A Two-Dose Combined Vaccine Against Hepatitis A and Hepatitis B in Healthy Children and Adolescents Compared to the Corresponding Monovalent Vaccines

Author: Yolanda Cervantes, Margarita Ramonet, Eduardo Pernambuco, Ricardo Rüttimann, Mauricio Lisker-Melman, Tania Azevedo, María Cristina Cañero-Velasco, Cristina Targa-Ferreira, Sue Ann Costa Clemens, Themis Reverbel da Silveira, and Luiza Elena Falleiros-Carvalho
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, Vaccination schedule, medicine.medical_treatment, Hepatitis A Antibodies, Internal medicine, Humans, Medicine, Hepatitis B Vaccines, Hepatitis Antibodies, Vaccines, Combined, Hepatitis B Antibodies, Child, Immunization Schedule, Hepatitis, Hepatitis A Vaccines, Reactogenicity, business.industry, Immunogenicity, Hepatitis A, General Medicine, Hepatitis B, medicine.disease, Vaccination, Child, Preschool, Immunology, Female, business, Adjuvant
Abstract: Background Immunization against hepatitis A and B has been available for some time, protecting against both infections. With a view to achieving further reduction in the number of interventions and increasing convenience of the vaccinee, we investigated the reactogenicity and immunogenicity of a combined hepatitis A and B vaccine in healthy 4- to 20-year-old subjects at a 0, 6-month schedule. Methods Two hundred forty-eight study subjects were allocated to two study groups and received either two doses of the combined hepatitis A and B vaccine (68% of subjects) or the corresponding monovalent hepatitis A and hepatitis B vaccines (32% of subjects) concomitantly in opposite arms. Reactogenicity was assessed via diary cards after each vaccination. Serum samples were analyzed at months 1, 2, 6, and 7. Results All vaccines were well tolerated and very few symptoms were scored as severe. All but one subject seroconverted for anti-hepatitis A virus (anti-HAV) antibodies (98.6%) and 100% of subjects seroconverted for anti-hepatitis B (HBs) antibodies, with respective seroprotection rates of 98.7% for the combined vaccine group and 95.9% for the concomitant vaccine group ( p >0.05), respectively. Geometric mean titers were higher in the group receiving the combined vaccine: 6,635 mIU/mL vs. 2,728 mIU/mL ( p = 0.0001) for anti-HAV and 3,362 mIU/mL vs. 1,724 mIU/mL ( p = 0.0205) for anti-HBs, respectively. Younger subjects had a stronger immune response compared to older subjects. Conclusions The combined hepatitis A and B vaccine was well tolerated at this two-dose schedule. The combined vaccine had higher immunogenicity, probably explained by a adjuvant effect of the antigens. Vaccination programs requiring fewer injections will most likely have a positive impact on compliance rate and comfort of the vaccinee.
Published: 2002

23. [Eosinophilic esophagitis in 29 pediatric patients]

Author: Cristina Targa, Ferreira, Mário C, Vieira, Sandra Maria Gonçalves, Vieira, Giovana Stival da, Silva, Danielle Reis, Yamamoto, and Themis Reverbel da, Silveira
Subjects: Androstadienes, Male, Adolescent, Child, Preschool, Eosinophilia, Esophagitis, Fluticasone, Humans, Infant, Female, Enzyme Inhibitors, Child, Omeprazole
Abstract: Eosinophilic esophagitis is a recently described entity with esophageal symptoms like gastroesophageal reflux disease and significant esophageal eosinophilic infiltration.To present our clinical series of 29 children with eosinophilic esophagitis, describing the clinical and diagnostic features, treatment and outcome.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. Evaluation of the clinical, endoscopic and histologic findings, treatment and outcome was undertaken.The most common presenting symptoms included vomiting in 15 patients (52%) and abdominal pain in 11 patients (38%). Children under the age of 4 years presented with feeding disorder and failure to thrive. Patients between 5 and 8 years of age presented commonly with abdominal pain or symptoms that may be associated with reflux (heartburn and/or vomiting). Patients over the age of 8 presented most often with abdominal pain, dysphagia and occasional food impaction. Endoscopic features included vertical furrowing in 14 patients (48%), whitish papules in 12 (41%), corrugated rings in 2 patients (7%) and esophageal erosions in 3 patients (10%). In seven patients endoscopy was normal (24%). Treatment included swallowed fluticasone propionate in 19 patients and restriction diet in 7 patients. Patients who returned for follow-up had either improvement or remission of symptoms. After treatment, endoscopic biopsies were repeated in 11 patients, and a significant decrease in esophageal eosinophil counts was observed.The diagnosis of eosinophilic esophagitis must be considered when symptoms of reflux do not respond to conventional treatment. Upper gastrointestinal endoscopy must be complemented by a detailed analysis of histologic findings and eosinophil counts.
Published: 2007

24. Immunogenicity and Safety of Hepatitis A Vaccine in Children With Chronic Liver Disease.

Author: Cristina Targa Ferreira, Themis Reverbel da Silveira, Sandra Maria Vieira, Adriano Taniguchi, and Jorge Pereira-Lima
Abstract: SUMMARY: BACKGROUND Acute hepatitis A superimposed on chronic liver disease has been associated with a more severe course of disease and development of fulminant hepatitis. The aim of this study was to evaluate the immunogenicity and safety of an inactivated hepatitis A virus vaccine in children with chronic liver disease.PATIENTS AND METHODS This was an open, prospective, and controlled trial with 89 anti-HAV negative children between 1 and 16 years of age studied at a pediatric liver disease and transplantation referral center. Inactivated HAV vaccine (Havrix), from GlaxoSmithKline Biologicals containing 720 Elisa units of alum-adsorbed hepatitis A antigen per 0.5 ml dose was used. Thirty-four pediatric patients with chronic liver disease (mean age: 7.0 ± 4.86 years) and 55 healthy controls (mean age: 4.8 ± 2.7 years) received two doses of Havrix vaccine in months zero and six. Seroconversion and anti-HAV titers expressed as geometric mean titers (GMT) in mIU/ml were measured at months one and seven, by a modified Hepatitis A virus andibodies (HAVAB) assay.RESULTS Seroconversion rates at four weeks after primary immunization were 76% and 94% and the GMT 107.77 and 160.77 mIU/ml in the patient and control groups, respectively. One month after second dose the seroconversion rates were 97% and 100% in the groups with GMT of 812.40 and 2344.90 mIU/ml. Both doses were well tolerated with no significant adverse events observed. Local injection-site symptoms were the most common reactions reported in both groups.CONCLUSION Although GMTs were significantly lower in children with chronic liver disease compared to healthy controls, the overall seroconversion rates were not different. Hepatitis A virus vaccine was safe, well-tolerated, and immunogenic in children with chronic liver disease. [ABSTRACT FROM AUTHOR]
Published: 2003
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25. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency

Author: Antonia Felzen, Daan B.E. van Wessel, Emmanuel Gonzales, Richard J. Thompson, Irena Jankowska, Benjamin L. Shneider, Etienne Sokal, Tassos Grammatikopoulos, Agustina Kadaristiana, Emmanuel Jacquemin, Anne Spraul, Patryk Lipiński, Piotr Czubkowski, Nathalie Rock, Mohammad Shagrani, Dieter Broering, Emanuele Nicastro, Deirdre Kelly, Gabriella Nebbia, Henrik Arnell, Björn Fischler, Jan B.F. Hulscher, Daniele Serranti, Cigdem Arikan, Esra Polat, Dominique Debray, Florence Lacaille, Cristina Goncalves, Loreto Hierro, Gema Muñoz Bartolo, Yael Mozer-Glassberg, Amer Azaz, Jernej Brecelj, Antal Dezsőfi, Pier Luigi Calvo, Enke Grabhorn, Steffen Hartleif, Wendy J. van der Woerd, Binita M. Kamath, Jian-She Wang, Liting Li, Özlem Durmaz, Nanda Kerkar, Marianne Hørby Jørgensen, Ryan Fischer, Carolina Jimenez-Rivera, Seema Alam, Mara Cananzi, Noemie Laverdure, Cristina Targa Ferreira, Felipe Ordoñez Guerrero, Heng Wang, Valerie Sency, Kyung Mo Kim, Huey-Ling Chen, Elisa de Carvalho, Alexandre Fabre, Jesus Quintero Bernabeu, Aglaia Zellos, Estella M. Alonso, Ronald J. Sokol, Frederick J. Suchy, Kathleen M. Loomes, Patrick J. McKiernan, Philip Rosenthal, Yumirle Turmelle, Simon Horslen, Kathleen Schwarz, Jorge A. Bezerra, Kasper Wang, Bettina E. Hansen, Henkjan J. Verkade, Gastroenterology & Hepatology, Center for Liver, Digestive and Metabolic Diseases (CLDM), Institut Català de la Salut, [Felzen A, van Wessel DBE] Pediatric Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, the Netherlands. [Gonzales E] Pediatric Hepatology & Pediatric Liver Transplant Department, Centre de Référence de l'Atrésie des Voies Biliaires et des Cholestases Génétiques, Filière de Santé des Maladies Rares du Foie de l'enfant et de l'adulte, Assistance Publique-Hôpitaux de Paris, Faculté de Médecine Paris-Saclay, CHU Bicêtre, Paris, France. European Reference Network on Hepatological Diseases (ERN RARE-LIVER). INSERM, Hepatinov, Université Paris-Saclay, Orsay, France. [Thompson RJ] Institute of Liver Studies, King's College London, London, United Kingdom. [Jankowska I] European Reference Network on Hepatological Diseases (ERN RARE-LIVER). Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute, Warsaw, Poland. [Shneider BL] Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA. Childhood Liver Disease Research Network (ChiLDReN). [Quintero Bernabeu J] European Reference Network on Hepatological Diseases (ERN RARE-LIVER). Unitat de Gastroenterologia, Hepatologia, Suport Nutricional i Trasplantaments Hepàtics Pediàtrics, Vall d'Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique
Subjects: Fetge - Malalties - Aspectes genètics, Surgical Procedures, Operative::Digestive System Surgical Procedures::Surgical Procedures, Operative::Surgical Procedures, Operative::Liver Transplantation [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT], Hepatology, Otros calificadores::Otros calificadores::/genética [Otros calificadores], phenotype, genotype, Genetic Phenomena::Genetic Variation::Mutation [PHENOMENA AND PROCESSES], Gastroenterology, Fetge - Trasplantació, Polycyclic Compounds::Fused-Ring Compounds::Steroids::Bile Acids and Salts [CHEMICALS AND DRUGS], interruption of the enterohepatic circulation, compuestos policíclicos::compuestos con anillos de fusión::esteroides::ácidos y sales biliares [COMPUESTOS QUÍMICOS Y DROGAS], PFIC2, enfermedades del sistema digestivo::enfermedades hepáticas [ENFERMEDADES], Anomalies cromosòmiques, Digestive System Diseases::Liver Diseases [DISEASES], Àcids biliars - Secrecions, BSEP, COMPOUND HETEROZYGOSITY, Other subheadings::Other subheadings::/genetics [Other subheadings], Internal Medicine, Immunology and Allergy, intervenciones quirúrgicas::procedimientos quirúrgicos del sistema digestivo::intervenciones quirúrgicas::intervenciones quirúrgicas::trasplante de hígado [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS], fenómenos genéticos::variación genética::mutación [FENÓMENOS Y PROCESOS]
Abstract: Compound heterozygosity; Genotype; Phenotype Heterocigosidad compuesta; Genotipo; Fenotipo Heterozigositat composta; Genotip; Fenotip 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.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p

26. Contribution of endoscopy in the management of eosinophilic esophagitis.

Author: Ferreira CT and Goldani HA
Abstract: 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. It is unclear whether EoE is a new disease or a new classification of an old esophageal disorder. Esophagogastroduodenoscopy (EGD) and biopsies with histological examination of esophageal mucosa are required to establish the diagnosis of EoE, verify response to therapy, assess disease remission, document and dilate strictures and evaluate symptom recurrence of EoE. Repeated endoscopies with biopsies are necessary for monitoring of disease progression and treatment efficacy. EGD has a fundamental role in the diagnosis and management of EoE, forming an essential part of the investigation and follow-up of this condition. EoE is now considered a systemic disorder and not only a local condition with an important immunological background. One of the aims of research in EoE is to study non-invasive markers, such as immune indicators found in plasma, that correlate with local presence of EoE in esophageal tissues. Studies over the next few years will provide new information about diagnosis, pathogenesis, endoscopic/histologic criteria, non-invasive markers, novel and more efficacious treatments, as well as establishing natural history. Randomized clinical trials are urgently called for to inform non-invasive diagnostic tests, hallmarks of natural history and more efficacious treatment approaches for patients with EoE. The collaboration between pediatric and adult clinical and experimental studies will be paramount in the understanding and management of this disease.
Published: 2012
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26 results on '"Cristina Targa Ferreira"'

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

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

3. P-125 REX SHUNT IN A DEVELOPING COUNTRY – IS IT POSSIBLE?

4. Eosinophilic esophagitis – Where are we today?

5. Tufting enteropathy with EpCAM mutation: case report

6. Gastroesophageal reflux disease: exaggerations, evidence and clinical practice

7. Esofagite eosinofílica em 29 pacientes pediátricos Eosinophilic esophagitis in 29 pediatric patients

8. Prevalence of the serpin peptidase inhibitor (alpha-1-antitrypsin) PIS and PIZ alleles in Brazilian children with liver disease

9. Alergia alimentar: atualização prática do ponto de vista gastroenterológico Food allergy: a practical update from the gastroenterological viewpoint

10. Hepatites virais: aspectos da epidemiologia e da prevenção Viral Hepatitis: epidemiological and preventive aspects

11. Prevenção das hepatites virais através de imunização Viral hepatitis prevention by immunization

12. Seroprevalence of hepatitis A antibodies in a group of normal and Down Syndrome children in Porto Alegre, Southern Brazil

13. Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study

14. Global multi-stakeholder endorsement of the MAFLD definition

15. Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency

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

17. Non-Alcoholic Fatty Liver Disease in Children and Adolescents: Lifestyle Change - a Systematic Review and Meta-Analysis

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

19. Consenso Brasileiro sobre Alergia Alimentar: 2018 - Parte 1 - Etiopatogenia, clínica e diagnóstico. Documento conjunto elaborado pela Sociedade Brasileira de Pediatria e Associação Brasileira de Alergia e Imunologia

20. Consenso Brasileiro sobre Alergia Alimentar: 2018 - Parte 2 - Diagnóstico, tratamento e prevenção. Documento conjunto elaborado pela Sociedade Brasileira de Pediatria e Associação Brasileira de Alergia e Imunologia

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

22. A Two-Dose Combined Vaccine Against Hepatitis A and Hepatitis B in Healthy Children and Adolescents Compared to the Corresponding Monovalent Vaccines

23. [Eosinophilic esophagitis in 29 pediatric patients]

24. Immunogenicity and Safety of Hepatitis A Vaccine in Children With Chronic Liver Disease.

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

26. Contribution of endoscopy in the management of eosinophilic esophagitis.

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