HCC Prevalence and Histopathological Findings in Liver Explants of Patients With Hereditary Tyrosinemia Type 1

Página do item simplificado
dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorNETO, Joao Seda
dc.contributor.authorLEITE, Katia M. R.
dc.contributor.authorPORTA, Adriana
dc.contributor.authorFONSECA, Eduardo A.
dc.contributor.authorFEIER, Flavia H.
dc.contributor.authorPUGLIESE, Renata
dc.contributor.authorMIURA, Irene K.
dc.contributor.authorCHAPCHAP, Paulo
dc.contributor.authorPORTA, Gilda
dc.date.accessioned2015-02-06T19:09:40Z
dc.date.available2015-02-06T19:09:40Z
dc.date.issued2014
dc.description.abstractBackground. Untreated tyrosinemia type 1 (HT1) is manifested by liver failure associated with renal tubular dysfunction, growth failure, and rickets. The indication for liver transplantation (LT) is restricted to non-responders to 2-(2-nitro-4-trifluoromethylbenzoyl)-1, 3-cyclohexanedione (NTBC) treatment, patients not treated with NTBC or for patients with HCC. The aim of this study is to report on a series of NTBC naive HT1 patients submitted to LT along with the prevalence of HCC in their liver explants. Procedure. This is a retrospective study of 16 children with HT1 who underwent liver transplantation between January 1993 and December 2012. Results. Clinical features: liver failure in 12 (75%), growth failure in 4 (25%), rickets in 5 (31.2%), hypertrophic cardiomyopathy in three (18.7%), and renal tubulopathy in seven patients (43.7%). Median AFP level was 64,335 ng/ml. Abdominal CT scans showed multiple nodules in most patients. Histopathology of the explants showed cirrhosis in all patients and HCC in 12 (75%), 3 with microvascular invasion. The majority of the tumors were well differentiated. Patient survival rate was 86% at a median follow-up of 6.6 years. All survivors were tumor-free with no adjuvant chemotherapy. Conclusion. In countries where neonatal screening programs are not effective and NTBC treatment is not widely available, LT still plays an important role in the treatment of children with HT1. An early indication in patients who present with multinodular livers can also serve to treat an otherwise underdiagnosed HCC condition. (c) 2014 Wiley Periodicals, Inc.
dc.description.indexMEDLINE
dc.identifier.citationPEDIATRIC BLOOD & CANCER, v.61, n.9, p.1584-1589, 2014
dc.identifier.doi10.1002/pbc.25094
dc.identifier.eissn1545-5017
dc.identifier.issn1545-5009
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/8277
dc.language.isoeng
dc.publisherWILEY-BLACKWELL
dc.relation.ispartofPediatric Blood & Cancer
dc.rightsrestrictedAccess
dc.rights.holderCopyright WILEY-BLACKWELL
dc.subjectchildren
dc.subjecthepatocellular carcinoma
dc.subjectliver transplantation
dc.subjectoutcomes
dc.subjecttyrosinemia
dc.subject.otherhepatocellular-carcinoma
dc.subject.otherntbc treatment
dc.subject.othertransplantation
dc.subject.otherchildren
dc.subject.othertumors
dc.subject.otherchildhood
dc.subject.otherdisease
dc.subject.othercancer
dc.subject.otherrecommendations
dc.subject.otherexperience
dc.subject.wosOncology
dc.subject.wosHematology
dc.subject.wosPediatrics
dc.titleHCC Prevalence and Histopathological Findings in Liver Explants of Patients With Hereditary Tyrosinemia Type 1
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.author.externalNETO, Joao Seda:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalPORTA, Adriana:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalFONSECA, Eduardo A.:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalFEIER, Flavia H.:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalPUGLIESE, Renata:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalMIURA, Irene K.:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalCHAPCHAP, Paulo:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.author.externalPORTA, Gilda:Hosp Sirio Libanes Hosp AC Camargo, BR-01308000 Sao Paulo, Brazil
hcfmusp.citation.scopus32
hcfmusp.contributor.author-fmusphcKATIA RAMOS MOREIRA LEITE
hcfmusp.description.beginpage1584
hcfmusp.description.endpage1589
hcfmusp.description.issue9
hcfmusp.description.volume61
hcfmusp.origemWOS
hcfmusp.origem.scopus2-s2.0-84904431183
hcfmusp.origem.wosWOS:000340540600011
hcfmusp.publisher.cityHOBOKEN
hcfmusp.publisher.countryUSA
hcfmusp.relation.referenceArikan C, 2006, PEDIATR TRANSPLANT, V10, P42, DOI 10.1111/j.1399-3046.2005.00395.x
hcfmusp.relation.referenceArnon R, 2011, PEDIATR TRANSPLANT, V15, P400, DOI 10.1111/j.1399-3046.2011.01497.x
hcfmusp.relation.referenceBeaunoyer M, 2007, PEDIATR TRANSPLANT, V11, P655, DOI 10.1111/j.1399-3046.2007.00751.x
hcfmusp.relation.referenceBuyukpamukcu M, 2006, PEDIATR TRANSPLANT, V10, P517, DOI 10.1111/j.1399-3046.2006.00511.x
hcfmusp.relation.referenceCrone J, 2003, ACTA PAEDIATR, V92, P625
hcfmusp.relation.referencede Laet C, 2013, ORPHANET J RARE DIS, V8, DOI 10.1186/1750-1172-8-8
hcfmusp.relation.referenceEDMONDSON HA, 1954, CANCER, V7, P462, DOI 10.1002/1097-0142(195405)7:3<462::AID-CNCR2820070308>3.0.CO;2-E
hcfmusp.relation.referenceEl-Karaksy H, 2011, WORLD J PEDIATR, V7, P224, DOI 10.1007/s12519-011-0287-3
hcfmusp.relation.referenceForget S, 1999, PEDIATR RADIOL, V29, P104, DOI 10.1007/s002470050551
hcfmusp.relation.referenceGambarin-Gelwan M, 2000, AM J GASTROENTEROL, V95, P1535
hcfmusp.relation.referenceHadzic N, 2011, CLIN LIVER DIS, V15, P443, DOI 10.1016/j.cld.2011.03.011
hcfmusp.relation.referenceHolme E, 1998, J INHERIT METAB DIS, V21, P507, DOI 10.1023/A:1005410820201
hcfmusp.relation.referenceHolme E, 2000, Clin Liver Dis, V4, P805, DOI 10.1016/S1089-3261(05)70142-2
hcfmusp.relation.referenceJacobs SMM, 2006, PEDIATR RES, V59, P365, DOI 10.1203/01.pdr.0000198810.57642.b4
hcfmusp.relation.referenceJorquera R, 2001, HUM MOL GENET, V10, P1741, DOI 10.1093/hmg/10.17.1741
hcfmusp.relation.referenceKalicinski P, 2009, PEDIATR TRANSPLANT, V13, P657, DOI 10.1111/j.1399-3046.2009.01210.x
hcfmusp.relation.referenceKatzenstein BH, 2013, J CLIN ONCOL, V20, P2789
hcfmusp.relation.referenceKim H, 2000, LIVER, V20, P173, DOI 10.1034/j.1600-0676.2000.020002173.x
hcfmusp.relation.referenceKoelink CJL, 2006, MOL GENET METAB, V89, P310, DOI 10.1016/j.ymgme.2006.07.009
hcfmusp.relation.referenceLee JM, 2012, SEMIN ONCOL, V39, P399, DOI 10.1053/j.seminoncol.2012.05.010
hcfmusp.relation.referenceMasurel-Paulet A, 2008, J INHERIT METAB DIS, V31, P81, DOI 10.1007/s10545-008-0793-1
hcfmusp.relation.referenceMazzaferro V, 1996, NEW ENGL J MED, V334, P693, DOI 10.1056/NEJM199603143341104
hcfmusp.relation.referenceMcDiarmid SV, 2002, TRANSPLANTATION, V74, P173, DOI 10.1097/00007890-200207270-00006
hcfmusp.relation.referenceMcKiernan PJ, 2006, DRUGS, V66, P743, DOI 10.2165/00003495-200666060-00002
hcfmusp.relation.referenceMohan N, 1999, EUR J PEDIATR, V158, pS49, DOI 10.1007/PL00014321
hcfmusp.relation.referenceMoore SW, 2004, CANCER, V101, P642, DOI 10.1002/cncr.20398
hcfmusp.relation.referenceMorioka D, 2005, AM J TRANSPLANT, V5, P2754, DOI 10.1111/j.1600-6143.2005.01084.x
hcfmusp.relation.referenceOtte JB, 2008, PEDIATR TRANSPLANT, V12, P1, DOI 10.1111/j.1399-3046.2007.00852.x
hcfmusp.relation.referenceOzcay F, 2006, CLIN TRANSPLANT, V20, P776, DOI 10.1111/j.1399-0012.2006.00571.x
hcfmusp.relation.referencePham TH, 2007, J PEDIATR SURG, V42, P834, DOI 10.1016/j.jpedsurg.2006.12.065
hcfmusp.relation.referenceReyes JD, 2000, J PEDIATR, V136, P795, DOI 10.1067/mpd.2000.106223
hcfmusp.relation.referenceRUSSO P, 1990, AM J HUM GENET, V47, P317
hcfmusp.relation.referenceRusso PA, 2001, PEDIATR DEVEL PATHOL, V4, P212, DOI 10.1007/s100240010146
hcfmusp.relation.referenceShneider BL, 2002, PEDIATR TRANSPLANT, V6, P25, DOI 10.1034/j.1399-3046.2002.1p057.x
hcfmusp.relation.referenceSpronsen FJ, 2005, J PEDIAT GASTROENTER, V40, P90
hcfmusp.relation.referenceSuperina R, 1996, J PEDIATR SURG, V31, P835, DOI 10.1016/S0022-3468(96)90147-5
hcfmusp.relation.referenceTORTAJADA JFI, 2008, AN PEDIATR, V68, P377
hcfmusp.relation.referenceWeeda VB, 2013, EUR J CANCER, V49, P2698, DOI 10.1016/j.ejca.2013.04.012
hcfmusp.scopus.lastupdate2024-05-10
relation.isAuthorOfPublication23772d3b-0da7-472f-8d56-e6312dbf95c1
relation.isAuthorOfPublication.latestForDiscovery23772d3b-0da7-472f-8d56-e6312dbf95c1
Arquivos
Pacote Original
Agora exibindo 1 - 1 de 1
Nenhuma Miniatura disponível
Nome:
art_NETO_HCC_Prevalence_and_Histopathological_Findings_in_Liver_Explants_2014.PDF
Tamanho:
1019.62 KB
Formato:
Adobe Portable Document Format
Descrição:
publishedVersion (English)
Baixar
Coleções
Artigos e Materiais de Revistas Científicas - FM/MCG
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/55
Artigos e Materiais de Revistas Científicas - ODS/03