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

2019Journal article Open access
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dc.contributor.authorPorta, Gilda
dc.contributor.authorCarvalho, Elisa
dc.contributor.authorSantos, Jorge Luiz Dos
dc.contributor.authorGama, Jorge
dc.contributor.authorBorges, Cristian V.
dc.contributor.authorSeixas, Renata
dc.contributor.authorFerreira, Alexandre
dc.contributor.authorMiura, Irene
dc.contributor.authorSilveira, Themis Reverbel
dc.contributor.authorSilva, Luciana
dc.contributor.authorFagundes, Eleonora
dc.contributor.authorBrandao, MA Bellomo
dc.contributor.authorSawamura, Regina
dc.contributor.authorVieira, Sandra
dc.contributor.authorMelere, Melina
dc.contributor.authorMarques, Cibele D F
dc.contributor.authorPugliese, Renata P.
dc.contributor.authorDanesi, Vera
dc.contributor.authorPorta, Adriana
dc.contributor.authorMarsillac, Marise
dc.contributor.authorValladares, Marcia Angélica
dc.contributor.authorMenezes, Daniela G.
dc.contributor.authorKieling, Carlos
dc.contributor.authorPaula, Mariana N. de
dc.contributor.authorVasconcelos, Juliana R.
dc.contributor.authorFerreira, Cristina T.
dc.contributor.authorPerin, Nilza
dc.contributor.authorResende, Leonardo R.
dc.contributor.authorMaia, Jussara
dc.contributor.authorTommaso, Adriana M.A. De
dc.contributor.authorHessel, Gabriel
dc.date.accessioned2020-02-05T16:45:38Z
dc.date.available2020-02-05T16:45:38Z
dc.date.issued2019
dc.descriptionIn this large clinical series of Brazilian children and adolescents, autoimmunehepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higherdisease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.pt_PT
dc.description.abstractsentation, laboratory findings, histological profile, treatments, and outcomes of children andadolescents with autoimmune hepatitis.Methods: The medical records of 828 children and adolescents with autoimmune hepatitiswere reviewed. A questionnaire was used to collect anonymous data on clinical presentation,biochemical and histological findings, and treatments.Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmunehepatitis-2. The female sex was predominant in both groups. The median age at symptomonset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher forautoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patientswith autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-foldhigher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were sig-nificantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels werelower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochem-ical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6%underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmunehepatitis-2: 2.4%).Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmunehepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higherdisease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.doi10.1016/j.jped.2018.04.007pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.6/9045
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/pt_PT
dc.subjectAutoimmune hepatitispt_PT
dc.subjectClinicalpt_PT
dc.subjectLaboratorypt_PT
dc.subjectTreatmentspt_PT
dc.subjectOutcomespt_PT
dc.subjectBrazilpt_PT
dc.titleAutoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomespt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceRio de Janeiropt_PT
oaire.citation.endPage427pt_PT
oaire.citation.issue4pt_PT
oaire.citation.startPage419pt_PT
oaire.citation.titleJornal de Pediatriapt_PT
oaire.citation.volume95pt_PT
person.familyNamePorta
person.familyNameCarvalho
person.familyNamedos Santos
person.familyNameReis Gama
person.familyNameSeixas
person.familyNameFerreira
person.familyNameMiura
person.familyNameReverbel Silveira
person.familyNameSilva
person.familyNameFagundes
person.familyNameBellomo-Brandao
person.familyNameSawamura
person.familyNameGonçalves Vieira
person.familyNameMelere
person.familyNameDANTAS FERREIRA MARQUES
person.familyNameDanesi
person.familyNameElia de Marsillac
person.familyNameValladares
person.familyNameKieling
person.familyNameRamalho
person.familyNameTarga Ferreira
person.familyNameDE TOMMASO
person.familyNameHessel
person.givenNameGilda
person.givenNameElisa
person.givenNameJorge Luiz
person.givenNameJorge Manuel
person.givenNameRenata
person.givenNameAlexandre
person.givenNameIrene
person.givenNameThemis
person.givenNameLuciana
person.givenNameEleonora
person.givenNameMaria Angela
person.givenNameRegina
person.givenNameSandra Maria
person.givenNameMelina
person.givenNameCIBELE
person.givenNameVera
person.givenNameMarise
person.givenNameMarcia Angélica
person.givenNameCarlos
person.givenNameJuliana
person.givenNameCristina
person.givenNameADRIANA
person.givenNameGabriel
person.identifier.ciencia-idE81F-1C44-F08D
person.identifier.ciencia-idDF1D-C70A-9AD5
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person.identifier.scopus-author-id6507189055
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person.identifier.scopus-author-id7102450018
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
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