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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]: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]: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]:28374309
[Au] Autor:Lopez RN; Ooi CY; Krishnan U
[Ad] Endereço:Department of Gastroenterology, Sydney Children's Hospital, High Street, Randwick, Sydney, NSW 2031, Australia. Robert.Lopez@health.nsw.gov.au.
[Ti] Título:Early and Peri-operative Prognostic Indicators in Infants Undergoing Hepatic Portoenterostomy for Biliary Atresia: a Review.
[So] Source:Curr Gastroenterol Rep;19(4):16, 2017 Apr.
[Is] ISSN:1534-312X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE OF REVIEW: Biliary atresia is the most common indication for liver transplantation among children. In recent years, prospective, multi-centre collaboration has been underway with the aim of providing high-quality data on the natural history of the condition, prior to and following hepatic portoenterostomy. RECENT FINDINGS: There is increasing evidence that specific histological findings, and age, at the time of portoenterostomy have relevance as prognostic indicators. Recent data suggest that the sub-type of biliary atresia, its co-existence with other anomalies and concurrent infection may also be important variables. This review provides a comprehensive summary of the histological predictors of outcome following portoenterostomy. Later age at portoenterostomy, advanced degree of hepatic fibrosis and co-existence with other congenital anomalies are strongly correlated with poor outcomes following portoenterostomy. There is increasing evidence to suggest that common serological indices and the presence or absence of cytomegalovirus (CMV) co-infection may have utility as early prognostic indicators.
[Mh] Termos MeSH primário: Atresia Biliar/cirurgia
Portoenterostomia Hepática
[Mh] Termos MeSH secundário: Fatores Etários
Atresia Biliar/complicações
Atresia Biliar/diagnóstico
Atresia Biliar/patologia
Biomarcadores/sangue
Infecções por Citomegalovirus/complicações
Seres Humanos
Lactente
Prognóstico
Fatores de Risco
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Biomarkers)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170405
[St] Status:MEDLINE
[do] DOI:10.1007/s11894-017-0555-z


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[PMID]:28159941
[Au] Autor:Redkar R; Karkera PJ; Raj V; Bangar A; Hathiramani V; Krishnan J
[Ad] Endereço:Department of Pediatric Surgery, Lilavati Hospital and Research Centre; and Department of Pediatric Surgery, Bai Jerbai Wadia Hospital for Children, Parel; Mumbai, India. Correspondence to: Dr Rajeev Redkar, 14, Buildarch Terrace, 1st Floor, Sitladevi Temple Road, Mahim, Mumbai 400 016, India. rajeev.redkar@gmail.com.
[Ti] Título:Outcome of Biliary Atresia After Kasai's Portoenterostomy: A 15-year Experience.
[So] Source:Indian Pediatr;54(4):291-294, 2017 Apr 15.
[Is] ISSN:0974-7559
[Cp] País de publicação:India
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: Medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year). RESULTS: 121 patients (61.9% males) were included; 32 (26.5%) were lost to follow-up at 1 year. At last follow-up, out of the 89, 42 (47.2%) were alive, 29 (32.6%) were jaundice-free, and 47 (52.8%) had died. The native liver survival rate at last follow up was 43.8%. 42 (47.2%) patients had complete clearance of jaundice at 3 months post-procedure. Jaundice-clearance rate was significantly high in patients alive (83.3% vs 16.7%, P<0.001)) as compared to those who died later. CONCLUSION: Jaundice clearance at 3 months post surgery is a good early indicator of long term success.
[Mh] Termos MeSH primário: Atresia Biliar/epidemiologia
Atresia Biliar/cirurgia
Portoenterostomia Hepática/estatística & dados numéricos
[Mh] Termos MeSH secundário: Atresia Biliar/mortalidade
Feminino
Seres Humanos
Lactente
Icterícia
Masculino
Portoenterostomia Hepática/efeitos adversos
Portoenterostomia Hepática/mortalidade
Complicações Pós-Operatórias
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:171125
[Lr] Data última revisão:
171125
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170205
[St] Status:MEDLINE


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[PMID]:28007417
[Au] Autor:Hussain MH; Alizai N; Patel B
[Ad] Endereço:Queen Mary University of London, London, United Kingdom; Leeds General Infirmary, Leeds, United Kingdom.
[Ti] Título:Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: A systematic review.
[So] Source:J Pediatr Surg;52(2):264-267, 2017 Feb.
[Is] ISSN:1531-5037
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:AIMS: Biliary atresia (BA) is a rare disease for which mainstay of treatment consists of open Kasai portoenterostomy. The aim of this review was to assess the outcomes of laparoscopic Kasai portoenterostomy, which offers potential benefits of minimally invasive surgery. Outcomes identified were postoperative cholangitis rates, incidence of adhesions at subsequent liver transplantation, native liver survival rates and actuarial survival rates. METHODS: A comprehensive systematic literature search was conducted in the PubMed and Cochrane databases using the keywords hepatic portoenterostomy, biliary atresia and laparoscopy. Robotic cases were excluded. RESULTS: Ten studies (n=149 patients) were included in this review. The mean age at the time of operation was 66 (range 14-119) days. The mean operative time was 261 (range 120-662) minutes. The rate of postoperative cholangitis was 34% (range 11%-50%). The mean native liver survival rate was 57% (range 33%-78%) at 6months and 47% (range 8%-76%) at 2years. Mean actuarial survival rate was 87% (range 54%-100%) at 2years. Subsequent adhesions were reported in 4 patients. Two patients had dense adhesions and 2 had no adhesions. CONCLUSIONS: Although laparoscopic Kasai portoenterostomy is a feasible operation, outcomes in terms of native liver survival rates and actuarial survival rates are unfavourable compared to conventional surgery. There is no evidence that laparoscopic Kasai is associated with fewer adhesions at subsequent liver transplantation. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Treatment study.
[Mh] Termos MeSH primário: Atresia Biliar/cirurgia
Laparoscopia
Portoenterostomia Hepática/métodos
[Mh] Termos MeSH secundário: Seres Humanos
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170215
[Lr] Data última revisão:
170215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161224
[St] Status:MEDLINE


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[PMID]:27859955
[Au] Autor:Chiang LW; Lee CY; Krishnaswamy G; Nah SA; Kader A; Ong C; Low Y; Phua KB
[Ad] Endereço:Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
[Ti] Título:Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre.
[So] Source:J Paediatr Child Health;53(4):412-415, 2017 Apr.
[Is] ISSN:1440-1754
[Cp] País de publicação:Australia
[La] Idioma:eng
[Ab] Resumo:AIM: Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evaluates prognostic factors for outcome. METHODS: All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 µmol/L within 6 months after KP. Two-year and 5-year native liver survival (NLS) were determined. Prognostic factors examined included gender, ethnicity, associated anomalies, age at KP, post-KP cholangitis and clearance of jaundice within 6 months. RESULTS: Of 58 patients studied, 31(53.4%) were male. Median age at time of KP was 53 days (range: 28-127). Ethnic distribution showed 32 (55.2%) Chinese, 16 (27.6%) Malays and 10 (17.2%) others. Twenty-one (36.2%) patients achieved jaundice clearance by 6 months. Two-year NLS rate was 36 out of 50 (72%), while 5-year NLS rate was 16 out of 35 (45.7%). Only clearance of jaundice within 6 months had a significant association with NLS (P = 0.006). All other factors showed no significant impact on outcome. CONCLUSIONS: Our short- and medium-term outcomes after KP for BA are comparable with those reported by most international centres. However, prognostic factors such as age at KP, cholangitis episodes and associated anomalies did not show significant correlation; only clearance of jaundice within 6 months was significantly predictive of NLS.
[Mh] Termos MeSH primário: Atresia Biliar/cirurgia
Portoenterostomia Hepática/métodos
[Mh] Termos MeSH secundário: Ásia Sudeste
Feminino
Seres Humanos
Lactente
Recém-Nascido
Icterícia Neonatal
Fígado/fisiopatologia
Masculino
Auditoria Médica
Avaliação de Resultados (Cuidados de Saúde)
Prognóstico
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171101
[Lr] Data última revisão:
171101
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161119
[St] Status:MEDLINE
[do] DOI:10.1111/jpc.13379


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[PMID]:27743713
[Au] Autor:Liu MB; Huong TB; Hoang X; Doan L; Trinh S; Anh Nguyen HP; Thanh Le H; Holterman AX
[Ad] Endereço:Stanford University School of Medicine, Stanford, CA; University of Illinois College of Medicine at Peoria, Peoria, IL.
[Ti] Título:Biliary atresia in Vietnam: Management and the burden of disease.
[So] Source:Surgery;161(2):533-537, 2017 Feb.
[Is] ISSN:1532-7361
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Biliary atresia is an idiopathic, neonatal liver disease of the bile ducts. The natural evolution of biliary atresia is known in developed countries. This study describes the clinical course of biliary atresia in Vietnam, a developing country. METHODS: Chart reviews were undertaken of patients treated with or without the Kasai procedure between January 2010 and July 2013 at a children's hospital in Vietnam. RESULTS: Of 287 children with biliary atresia, 149 (52%) were treated without the Kasai procedure and 138 (48%) were treated with the Kasai procedure. Median age at diagnosis was 2.4 months for children treated without the Kasai procedure vs 2.3 months for those treated with the procedure. The percentages of patients in the group treated without the Kasai procedure presenting at <2 months, 2 to <3 months, 3 to <4 months, 4-6 months, and >6 months of age were 31%, 35%, 15%, 10%, and 9%, respectively, compared to those treated with the Kasai procedure at 36% (P = .38), 44% (P = .12), 16% (P = 1.0), 4% (P = .037), and 0% (P < .001), respectively. The group treated without the Kasai procedure had 1- and 2-year survivals of 52% and 28%, respectively (median survival 6.6 months); in contrast, the group treated with the Kasai procedure had 1- and 2-year transplant-free survivals of 84% and 71%. No patients were treated by liver transplantation because of lack of a liver transplantation program in Vietnam. CONCLUSION: The majority of biliary atresia in Vietnam remains untreated despite early presentation and reasonable outcomes after a Kasai procedure relative to Western countries. These data illustrate the high health care burden for biliary atresia in Vietnam and the need to improve education about biliary atresia and its treatment.
[Mh] Termos MeSH primário: Atresia Biliar/diagnóstico
Atresia Biliar/cirurgia
Mortalidade Hospitalar/tendências
Portoenterostomia Hepática/métodos
Complicações Pós-Operatórias/mortalidade
[Mh] Termos MeSH secundário: Atresia Biliar/mortalidade
Estudos de Coortes
Países em Desenvolvimento
Feminino
Hospitais Pediátricos
Seres Humanos
Lactente
Recém-Nascido
Masculino
Determinação de Necessidades de Cuidados de Saúde
Portoenterostomia Hepática/efeitos adversos
Complicações Pós-Operatórias/fisiopatologia
Prognóstico
Estudos Retrospectivos
Medição de Risco
Análise de Sobrevida
Fatores de Tempo
Resultado do Tratamento
Vietnã
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170705
[Lr] Data última revisão:
170705
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:161017
[St] Status:MEDLINE


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[PMID]:27717116
[Au] Autor:Webb NL; Jiwane A; Ooi CY; Nightinghale S; Adams SE; Krishnan U
[Ad] Endereço:Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
[Ti] Título:Clinical significance of liver histology on outcomes in biliary atresia.
[So] Source:J Paediatr Child Health;53(3):252-256, 2017 Mar.
[Is] ISSN:1440-1754
[Cp] País de publicação:Australia
[La] Idioma:eng
[Ab] Resumo:AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures. RESULTS: BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months. CONCLUSION: Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.
[Mh] Termos MeSH primário: Atresia Biliar/cirurgia
Avaliação de Resultados (Cuidados de Saúde)
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Lactente
Fígado/anatomia & histologia
Masculino
Auditoria Médica
Avaliação de Resultados (Cuidados de Saúde)/métodos
Portoenterostomia Hepática/métodos
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171011
[Lr] Data última revisão:
171011
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161008
[St] Status:MEDLINE
[do] DOI:10.1111/jpc.13371



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