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[PMID]:28746031
[Au] Autor:Hopkins PC; Yazigi N; Nylund CM
[Ad] Endereço:Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD. Electronic address: perri.c.hopkins.mil@mail.mil.
[Ti] Título:Incidence of Biliary Atresia and Timing of Hepatoportoenterostomy in the United States.
[So] Source:J Pediatr;187:253-257, 2017 Aug.
[Is] ISSN:1097-6833
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To evaluate the incidence, trends, seasonality, and age at the time of hepatoportoenterostomy (Kasai procedure) for biliary atresia in the US. STUDY DESIGN: The triennial Health Cost and Utilization Project-Kids' Inpatient Database for 1997-2012 was used to perform a retrospective analysis of biliary atresia in the US. Infants aged <1 year of age with a diagnosis of biliary atresia who underwent a Kasai procedure were included. Nationwide infant population data were used to calculate incidence and evaluate trends. Age at the time of the Kasai procedure and the seasonality of biliary atresia were evaluated as well. RESULTS: The incidence of biliary atresia in the US was 4.47 per 100 000 and was higher in females (risk ratio [RR], 1.43; 95% CI, 1.27-1.62), Asian/Pacific Islanders (RR, 1.89; 95% CI, 1.44-2.47), and blacks (RR, 1.30; 95% CI, 1.06-1.58) compared with whites. The incidence of biliary atresia increased by an average of 7.9% per year from 1997 to 2012 (P <.001). The median age at the time of the Kasai procedure was 63 days, with no improvement over the study period (P = .64). There was no evidence of seasonality (P = .69). CONCLUSION: The incidence of biliary atresia has increased over the past 15 years, with the median age at the time of the Kasai procedure now outside the optimal window. Implementation of systematic screening measures for biliary atresia in the US are needed.
[Mh] Termos MeSH primário: Atresia Biliar/epidemiologia
Portoenterostomia Hepática/métodos
[Mh] Termos MeSH secundário: Atresia Biliar/cirurgia
Bases de Dados Factuais
Feminino
Seres Humanos
Incidência
Lactente
Masculino
Estudos Retrospectivos
Fatores de Tempo
Estados Unidos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:180129
[Lr] Data última revisão:
180129
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170727
[St] Status:MEDLINE


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[PMID]:28457406
[Au] Autor:Éboli L; Tannuri AC; Gibelli N; Silva T; Braga P; Tannuri U
[Ad] Endereço:Pediatric Liver Transplantation Unit at Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
[Ti] Título:Comparison of the Results of Living Donor Liver Transplantation Due to Acute Liver Failure and Biliary Atresia in a Quaternary Center.
[So] Source:Transplant Proc;49(4):832-835, 2017 May.
[Is] ISSN:1873-2623
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: The objective of this study was to compare the complications, outcomes, and survival prevalence in patients undergoing living donor liver transplantation due to biliary atresia (BA) or acute liver failure (ALF). RESULTS: In the period of June 1998-July 2016, 199 children underwent living transplantation due to BA or ALF. Of these 199, 184 were included in the analysis. The average age, weight, and body mass index of BA patients were lower than those of ALF (P < .001). The chi-square test showed a higher prevalence of infection in transplant recipients due to BA (P = .0001) and a higher prevalence of hepatic artery stenosis in those who underwent transplantation due to ALF (P = .001). In the multivariate analysis, the infection remains statistically more prevalent in the BA group (95% confidence interval [CI], 0.20-0.60), while hepatic artery stenosis loses significance. The mortality rate was similar in both groups and the survival in 5 years also. The prevalence of hepatic artery thrombosis, portal vein thrombosis/stenosis, biliary stenosis, and acute and chronic cellular rejection showed no statistical difference between the two groups. CONCLUSION: Living donor liver transplantation should be a valid option in cases of fulminant hepatitis with an indication for liver transplantation, especially in places where the number of cadaverous donors is low and the length of time on the waiting list is high.
[Mh] Termos MeSH primário: Atresia Biliar/cirurgia
Falência Hepática Aguda/cirurgia
Transplante de Fígado/métodos
Doadores Vivos
[Mh] Termos MeSH secundário: Criança
Pré-Escolar
Feminino
Seres Humanos
Transplante de Fígado/efeitos adversos
Masculino
Análise Multivariada
Resultado do Tratamento
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171220
[Lr] Data última revisão:
171220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170502
[St] Status:MEDLINE


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[PMID]:27776008
[Au] Autor:Russo P; Magee JC; Anders RA; Bove KE; Chung C; Cummings OW; Finegold MJ; Finn LS; Kim GE; Lovell MA; Magid MS; Melin-Aldana H; Ranganathan S; Shehata BM; Wang LL; White FV; Chen Z; Spino C; Childhood Liver Disease Research Network (ChiLDReN)
[Ad] Endereço:*Department of Pathology and Laboratory Medicine, the Children's Hospital of Philadelphia, Philadelphia, PA ¶¶Department of Pathology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA Departments of †Surgery §§§Biostatistics, University of Michigan, Ann Arbor, MI ‡Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD §Division of Pediatric Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH ¶Department of Pathology, Indiana University School of Medicine, Indianapolis, IN #Department of Pathology, Texas Children's Hospital, Houston, TX **Department of Pathology, Seattle Children's Hospital, Seattle, WA ††Department of Pathology, University of California San Francisco, San Francisco, CA ***Department of Pathology, Children's Hospital Los Angeles, Los Angeles, CA ‡‡Department of Pathology, Children's Hospital Colorado, Aurora, CO §§Department of Pathology, Kravis Children's Hospital, Mount Sinai Health System, New York, NY ∥∥Department of Pathology, Ann & Robert H. Lurie Children's Hospital, Chicago, IL ##Department of Pathology, Children's Healthcare of Atlanta, Atlanta, GA †††Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO ‡‡‡Quest Diagnostics, Health Informatics, Madison, NJ ∥Division of Pathology, The Hospital of Sick Children, Toronto, ON, Canada.
[Ti] Título:Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome: A Multicenter Study.
[So] Source:Am J Surg Pathol;40(12):1601-1615, 2016 Dec.
[Is] ISSN:1532-0979
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The liver biopsy guides diagnostic investigation and therapy in infants with undiagnosed cholestasis. Histologic features in the liver may also have prognostic value in the patient with biliary atresia (BA). We assessed the relative value of histologic features in 227 liver needle biopsies in discriminating between BA and other cholestatic disorders in infants enrolled in a prospective Childhood Liver Disease Research Network (ChiLDReN) cohort study by correlating histology with clinical findings in infants with and without BA. In addition, we reviewed 316 liver biopsies from clinically proven BA cases and correlated histologic features with total serum bilirubin 6 months after hepatoportoenterostomy (the Kasai procedure, HPE) and transplant-free survival up to 6 years. Review pathologists were blinded to clinical information except age. Semiquantitative scoring of 26 discrete histologic features was based on consensus. Bile plugs in portal bile ducts/ductules, moderate to marked ductular reaction, and portal stromal edema had the largest odds ratio for predicting BA versus non-BA by logistic regression analysis. The diagnostic accuracy of the needle biopsy was estimated to be 90.1% (95% confidence interval [CI]: 85.2%, 94.9%), whereas sensitivity and specificity for a diagnosis of BA are 88.4% (95% CI: 81.4, 93.5) and 92.7% (95% CI: 84.8, 97.3), respectively. No histologic features were associated with an elevated serum bilirubin 6 months after HPE, although it (an elevated serum bilirubin) was associated with an older age at HPE. Higher stages of fibrosis, a ductal plate configuration, moderate to marked bile duct injury, an older age at HPE, and an elevated international normalized ratio were independently associated with a higher risk of transplantation.
[Mh] Termos MeSH primário: Atresia Biliar/diagnóstico
Colestase/etiologia
Fígado/patologia
[Mh] Termos MeSH secundário: Atresia Biliar/complicações
Atresia Biliar/patologia
Atresia Biliar/cirurgia
Bilirrubina/sangue
Biomarcadores/sangue
Biópsia por Agulha
Colestase/sangue
Diagnóstico Diferencial
Feminino
Seres Humanos
Lactente
Recém-Nascido
Estimativa de Kaplan-Meier
Modelos Logísticos
Estudos Longitudinais
Masculino
Portoenterostomia Hepática
Modelos de Riscos Proporcionais
Estudos Prospectivos
Sensibilidade e Especificidade
Método Simples-Cego
[Pt] Tipo de publicação:CLINICAL TRIAL; JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Nome de substância:
0 (Biomarkers); RFM9X3LJ49 (Bilirubin)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:171202
[Lr] Data última revisão:
171202
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161025
[St] Status:MEDLINE


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[PMID]:28902846
[Au] Autor:Ye Y; Li Z; Feng Q; Chen Z; Wu Z; Wang J; Ye X; Zhang D; Liu L; Gao W; Zhang L; Wang B
[Ad] Endereço:Shantou University Medical College, Shantou, Guangdong, China.
[Ti] Título:Downregulation of microRNA-145 may contribute to liver fibrosis in biliary atresia by targeting ADD3.
[So] Source:PLoS One;12(9):e0180896, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA. METHODS: In this study, 14 infants with BA and 14 infants with choledochal cyst (CC) were enrolled as experimental group and control group, respectively. ADD3 and microRNA-145 (miR-145) expression profiles in liver tissues of BA and CC were determined using qPCR. Luciferase reporter assay was performed to verify the direct interaction between miR-145-5p and ADD3 3' Untranslated Regions (3'UTR). The Lentiviral vectors containing miR-145, miR-145-3p inhibitor, miR-145-5p inhibitor, empty vector were transfected into human hepatic stellate cell line (LX-2) to determine the functional effect of miR-145 on ADD3 expression at both mRNA and protein level. RESULTS: MiR-145 was shown to be down-regulated in liver tissues of infants with BA compared to CC (p = 0.0267). ADD3, verified as a target of miR-145-5p, was shown to be overexpressed in infants with BA at the mRNA level (p = 0.0118). Transfection of lentiviruses containing miR-145 into LX-2 cells decreased the expression of ADD3 at both mRNA and protein level compared to negative control group, and suppressed the expression of p-Akt at protein level. CONCLUSIONS: Our study has shown that overexpressed ADD3 and downregulated miR-145 were detected in BA liver tissues. MiR-145-5p was confirmed to target ADD3 by luciferase reporter assay. The downregulation of miR-145 may contribute to liver fibrosis in BA by upregulating the expression of ADD3.
[Mh] Termos MeSH primário: Atresia Biliar/genética
Proteínas de Ligação a Calmodulina/genética
Cirrose Hepática/genética
MicroRNAs/genética
[Mh] Termos MeSH secundário: Estudos de Casos e Controles
Células Cultivadas
Regulação para Baixo/genética
Feminino
Regulação da Expressão Gênica
Seres Humanos
Lactente
Masculino
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (ADD3 protein, human); 0 (Calmodulin-Binding Proteins); 0 (MIRN145 microRNA, human); 0 (MicroRNAs)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171031
[Lr] Data última revisão:
171031
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170914
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0180896


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[PMID]:28763485
[Au] Autor:Jee J; Mourya R; Shivakumar P; Fei L; Wagner M; Bezerra JA
[Ad] Endereço:Divisions of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States of America.
[Ti] Título:Cxcr2 signaling and the microbiome suppress inflammation, bile duct injury, and the phenotype of experimental biliary atresia.
[So] Source:PLoS One;12(8):e0182089, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dependent bile duct injury and obstruction. Adult wild-type (WT) and Cxcr2-/- mice were fed a diet supplemented with sulfamethoxazole/trimethoprim (SMZ/TMP) during pregnancy and lactation, and their pups were injected intraperitoneally with rhesus rotavirus (RRV) within 24 hours of life to induce experimental biliary atresia. The maternal exposure to SMZ/TMP significantly lowered the incidence of jaundice and bile duct obstruction and resulted in improved survival, especially in Cxcr2-/- mice. Analyses of the microbiome by deep sequencing of 16S rRNA of the neonatal colon showed a delay in bacterial colonization of WT mice induced by SMZ/TMP, with a notable switch from Proteobacteria to Firmicutes. Interestingly, the genetic inactivation of Cxcr2 alone produced a similar bacterial shift. When treated with SMZ/TMP, Cxcr2-/- mice infected with RRV to induce experimental biliary atresia showed further enrichment of Corynebacterium, Anaerococcus and Streptococcus. Among these, Anaerococcus lactolyticus was significantly associated with a suppression of biliary injury, cholestasis, and survivability. These results suggest that the postnatal development of the intestinal microbiota is an important susceptibility factor for experimental biliary atresia.
[Mh] Termos MeSH primário: Ductos Biliares/lesões
Atresia Biliar/metabolismo
Inflamação/metabolismo
Microbiota
Receptores de Interleucina-8B/metabolismo
[Mh] Termos MeSH secundário: Animais
Atresia Biliar/microbiologia
Modelos Animais de Doenças
Feminino
Perfilação da Expressão Gênica
Lactação
Modelos Lineares
Macaca mulatta
Camundongos
Camundongos Endogâmicos BALB C
Camundongos Endogâmicos C57BL
Camundongos Transgênicos
Fenótipo
Reação em Cadeia da Polimerase
Gravidez
Prenhez
RNA Ribossômico 16S/genética
Receptores de Interleucina-8B/genética
Rotavirus
Transdução de Sinais
Sulfametoxazol/administração & dosagem
Trimetoprima/administração & dosagem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (RNA, Ribosomal, 16S); 0 (Receptors, Interleukin-8B); AN164J8Y0X (Trimethoprim); JE42381TNV (Sulfamethoxazole)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170802
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0182089


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[PMID]:28710035
[Au] Autor:Liu B; Wei J; Li M; Jiang J; Zhang H; Yang L; Wu H; Zhou Q
[Ad] Endereço:Department of Ultrasound, the Second Affiliated Hospital, Xi'an Jiaotong University, Xi'an 710004, China; Department of Ultrasound, Children's Hospital of Xi'an, Xi'an 710010, China.
[Ti] Título:Association of common genetic variants in VEGFA with biliary atresia susceptibility in Northwestern Han Chinese.
[So] Source:Gene;628:87-92, 2017 Sep 10.
[Is] ISSN:1879-0038
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Biliary atresia (BA) is a major neonatal obliterative cholangiopathy, resulting in progressive cirrhosis. The gene VEGFA encodes a heparin-binding protein that is a regulator of angiogenesis and a mediator of inflammatory reactions, and accumulating evidence have indicated that VEGFA may play a possible role in the pathogenesis of BA. Our study aim was to evaluate the association of common variants within the VEGFA gene with BA susceptibility in Northwestern Han Chinese population. Forty tag SNPs within the VEGFA gene were selected in the study, and then subsequently genotyped in 1336 Northwestern Han Chinese individuals, consisting of 311 BA patients and 1025 healthy controls. The SNP rs3025039 was found to be strongly associated with BA risk (additive P=0.000264) in our sample, and the CC genotype of rs3025039 had higher prevalence than the other two genotypes, indicating the C allele is a risk allele with an odds ratio (OR) of 1.56 and 95% confidence interval (CI) of 1.23-1.99. Haplotype analyses showed that a LD block containing rs3025039 significantly correlated with BA risk (global P<0.001). Moreover, bioinformatics analysis indicated that hsa-mir-591 and VEGFA formed miRNA/SNP target duplexes if the rs3025039 allele was in the T form, suggesting that rs3025039 may alter VEGFA expression by affecting hsa-miR-591/single-nucleotide polymorphism target duplexes. Our results indicate additional evidence supporting that there is an important role of the VEGFA gene in the increased susceptibility of BA.
[Mh] Termos MeSH primário: Atresia Biliar/genética
Polimorfismo de Nucleotídeo Único
Fator A de Crescimento do Endotélio Vascular/genética
[Mh] Termos MeSH secundário: Grupo com Ancestrais do Continente Asiático/genética
China
Feminino
Estudos de Associação Genética
Predisposição Genética para Doença
Seres Humanos
Masculino
Gravidez
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Vascular Endothelial Growth Factor A)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171004
[Lr] Data última revisão:
171004
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170716
[St] Status:MEDLINE


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[PMID]:28688656
[Au] Autor:Zagory JA; Dietz W; Park A; Fenlon M; Xu J; Utley S; Mavila N; Wang KS
[Ad] Endereço:Department of Surgery, Children's Hospital Los Angeles, Los Angeles, California.
[Ti] Título:Notch signaling promotes ductular reactions in biliary atresia.
[So] Source:J Surg Res;215:250-256, 2017 Jul.
[Is] ISSN:1095-8673
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Biliary atresia (BA) is a congenital, progressive, fibro-obliterative disease of the extrahepatic biliary tree and the most common cause of end-stage liver disease in children. BA is characterized by extensive intrahepatic proliferating ductular reactions that may contribute to biliary fibrosis. Lineage tracing during experimental cholestasis indicates that cells within ductular reactions derive from PROM1-expressing hepatic progenitor cells. Given the role of Notch signaling in normal biliary development, we hypothesize that activated Notch signaling promotes the formation of ductular reactions in BA. METHODS: Liver samples collected from BA infants at Kasai portoenterostomy and age-matched controls, as well as from wild-type and Prom1 knockout mice with 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC)-induced experimental cholestasis were analyzed histologically using immunofluorescence and by quantitative polymerase chain reaction. RESULTS: Increased expression of genes encoding Notch ligand JAG1 and its receptor NOTCH2 was observed in BA livers compared with control by quantitative polymerase chain reaction analyses. Livers of DDC-treated mice, which exhibit cytokeratin-19-positive ductular reactions typical of BA livers, demonstrated significant increases in the expression level of the gene encoding Notch2, as well as downstream Notch target gene Hes1 compared with control. Prom1 knockout mice exhibit diminished ductular reactions and decreased levels of Jag1 and Hes1 compared with littermate controls. CONCLUSIONS: Human BA and cholestasis induced by DDC are associated with Notch signaling activation. Null mutation of Prom1 is associated with decreased ductular reactions and decreased Notch signaling activation during DDC treatment. These data are consistent with Notch signaling promoting ductular reactions of Prom1 expressing progenitor cells in BA.
[Mh] Termos MeSH primário: Atresia Biliar/metabolismo
Atresia Biliar/patologia
Receptor Notch2/metabolismo
Transdução de Sinais
[Mh] Termos MeSH secundário: Antígeno AC133/genética
Antígeno AC133/metabolismo
Animais
Biomarcadores/metabolismo
Estudos de Casos e Controles
Seres Humanos
Imuno-Histoquímica
Proteína Jagged-1/metabolismo
Camundongos
Camundongos Knockout
Reação em Cadeia da Polimerase Via Transcriptase Reversa
Fatores de Transcrição HES-1/metabolismo
Regulação para Cima
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (AC133 Antigen); 0 (Biomarkers); 0 (Hes1 protein, mouse); 0 (JAG1 protein, human); 0 (Jag1 protein, mouse); 0 (Jagged-1 Protein); 0 (NOTCH2 protein, human); 0 (Notch2 protein, mouse); 0 (Prom1 protein, mouse); 0 (Receptor, Notch2); 0 (Transcription Factor HES-1)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171003
[Lr] Data última revisão:
171003
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170710
[St] Status:MEDLINE


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[PMID]:28658122
[Au] Autor:Song Z; Dong R; Shen Z; Chen G; Yang Y; Zheng S
[Ad] Endereço:Department of Pediatric Surgery, Children's Hospital of Fudan University, and Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai, China.
[Ti] Título:Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received Kasai operation less than 60 days after birth: A retrospective study.
[So] Source:Medicine (Baltimore);96(26):e7267, 2017 Jun.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:This study aimed to analyze the impact of etiologic heterogeneity and operation age on prognosis of infants with biliary atresia (BA) who received Kasai operation prior to 60 days of age.From 2004 to 2010, 158 infants received Kasai operation before turning 60 days old. According to Davenport 2012 classifications, 4 groups of BA were defined: cystic BA, syndrome BA, and associated malformation, cytomegalovirus (CMV)-associated BA, and isolated BA. Native (autologous) liver survival rates and incidence of cholangitis 2 years after operation, as well as jaundice clearance rates 3 months after operation, were recorded.Although infants who received the operation between 51 and 60 days of age had a better jaundice clearance 3 months after operation and lower incidence of cholangitis as compared with those under 40 or between 41 and 50 days of age, there was no significant difference in survival rates. Among types of BA, infants with cystic BA had the best prognosis. In the syndrome BA and associated malformations group, as well as CMV-associated group, infants who received the operation early (<40 days of age) had a worse outcome as compared with those who received the operation between 41 and 50 days or 51 and 60 days of age.Both clinical etiologic heterogeneity and operation age may influence BA prognosis.
[Mh] Termos MeSH primário: Atresia Biliar/etiologia
Atresia Biliar/cirurgia
[Mh] Termos MeSH secundário: Fatores Etários
Atresia Biliar/diagnóstico
Seres Humanos
Lactente
Portoenterostomia Hepática
Prognóstico
Estudos Retrospectivos
Análise de Sobrevida
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170718
[Lr] Data última revisão:
170718
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170629
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007267


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[PMID]:28655415
[Au] Autor:Kerola A; Lampela H; Lohi J; Heikkilä P; Mutanen A; Jalanko H; Pakarinen MP
[Ad] Endereço:Pediatric Surgery and Pediatric Transplantation Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland; Department of Surgery, North Karelia Central Hospital, Joensuu, Finland. Electronic address: anna.kerola@h
[Ti] Título:Molecular signature of active fibrogenesis prevails in biliary atresia after successful portoenterostomy.
[So] Source:Surgery;162(3):548-556, 2017 Sep.
[Is] ISSN:1532-7361
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: In biliary atresia mechanisms of progressive liver injury leading to need of liver transplantation after successful portoenterostomy remain unknown. A better understanding is a prerequisite for development of novel therapies to extend native liver survival, and we aimed to unravel molecular characteristics of liver injury after successful portoenterostomy. METHODS: Liver biopsies obtained from 28 biliary atresia children during successful portoenterostomy and at median age 3.0 years were studied. Biopsies were analyzed for histology and immunohistochemical expression of collagen 1, myofibroblast marker α-smooth muscle actin, and cytokeratin-7 positive ductal reactions. Hepatic ribonucleic acid (RNA) expression of growth factors and inflammatory cytokines was evaluated. Intestinal failure patients with comparable liver fibrosis and nonfibrotic gallstone patients and donor livers were controls. RESULTS: After successful portoenterostomy, histologic cholestasis resolved and portal inflammation reduced, while fibrosis along with ductal reactions and overexpression of collagen and α-smooth muscle actin persisted. At follow-up, liver RNA expression of collagen and platelet-derived growth factor was increased, whereas RNA expression of various inflammatory cytokines remained low. Disappearance of periductal α-smooth muscle actin expression after successful portoenterostomy (36% of patients) associated with contracted ductal reactions and reduced progression of fibrosis, collagen accumulation, platelet-derived growth factor RNA expression, and serum levels of bile acids and bilirubin. Fibrosis progressed less rapidly in syndromic than in isolated biliary atresia patients. CONCLUSION: These findings suggest that instead of inflammation, molecular signature of active fibrogenesis in association with ductal reactions prevails in long-term native liver survivors with biliary atresia. Patients should be stratified for isolated and syndromic disease forms in interventional studies.
[Mh] Termos MeSH primário: Atresia Biliar/genética
Atresia Biliar/cirurgia
Cirrose Hepática/patologia
Transplante de Fígado/métodos
Portoenterostomia Hepática/métodos
[Mh] Termos MeSH secundário: Atresia Biliar/patologia
Biomarcadores/metabolismo
Biópsia por Agulha
Estudos de Casos e Controles
Pré-Escolar
Feminino
Seguimentos
Seres Humanos
Imuno-Histoquímica
Lactente
Queratinas/genética
Cirrose Hepática/cirurgia
Testes de Função Hepática
Transplante de Fígado/mortalidade
Masculino
Portoenterostomia Hepática/efeitos adversos
Portoenterostomia Hepática/mortalidade
Quinazolinas/metabolismo
RNA/genética
Reoperação
Estudos Retrospectivos
Medição de Risco
Índice de Gravidade de Doença
Estatísticas não Paramétricas
Taxa de Sobrevida
Fatores de Tempo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (Quinazolines); 0 (SMA-41); 63231-63-0 (RNA); 68238-35-7 (Keratins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170913
[Lr] Data última revisão:
170913
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170629
[St] Status:MEDLINE


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[PMID]:28515290
[Au] Autor:Mohanty SK; Donnelly B; Dupree P; Lobeck I; Mowery S; Meller J; McNeal M; Tiao G
[Ad] Endereço:Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
[Ti] Título:A Point Mutation in the Rhesus Rotavirus VP4 Protein Generated through a Rotavirus Reverse Genetics System Attenuates Biliary Atresia in the Murine Model.
[So] Source:J Virol;91(15), 2017 Aug 01.
[Is] ISSN:1098-5514
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Rotavirus infection is one of the most common causes of diarrheal illness in humans. In neonatal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstruction of the extrahepatic biliary tract and intrahepatic bile ducts. We previously showed that the amino acid arginine (R) within the sequence SRL (amino acids 445 to 447) in the RRV VP4 protein is required for viral binding and entry into biliary epithelial cells. To determine if this single amino acid (R) influences the pathogenicity of the virus, we generated a recombinant virus with a single amino acid mutation at this site through a reverse genetics system. We demonstrated that the RRV mutant (RRV ) produced less symptomatology and replicated to lower titers both and than those seen with wild-type RRV, with reduced binding in cholangiocytes. Our results demonstrate that a single amino acid change in the RRV VP4 gene influences cholangiocyte tropism and reduces pathogenicity in mice. Rotavirus is the leading cause of diarrhea in humans. Rhesus rotavirus (RRV) can also lead to biliary atresia (a neonatal human disease) in mice. We developed a reverse genetics system to create a mutant of RRV (RRV ) with a single amino acid change in the VP4 protein compared to that of wild-type RRV. , the mutant virus had reduced binding and infectivity in cholangiocytes. , it produced fewer symptoms and lower mortality in neonatal mice, resulting in an attenuated form of biliary atresia.
[Mh] Termos MeSH primário: Atresia Biliar/patologia
Proteínas do Capsídeo/genética
Proteínas Mutantes/genética
Mutação Puntual
Rotavirus/patogenicidade
[Mh] Termos MeSH secundário: Animais
Animais Recém-Nascidos
Atresia Biliar/virologia
Proteínas do Capsídeo/metabolismo
Modelos Animais de Doenças
Camundongos
Proteínas Mutantes/metabolismo
Genética Reversa
Rotavirus/genética
Tropismo Viral
Replicação Viral
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Capsid Proteins); 0 (Mutant Proteins); 0 (VP4 protein, Rotavirus)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170727
[Lr] Data última revisão:
170727
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170519
[St] Status:MEDLINE



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