RESUMO
PURPOSE: To investigate the clinical characteristics of cystic biliary atresia (CBA) and evaluate the midterm follow-up outcomes after laparoscopic treatment. METHODS: We analyzed and compared data retrospectively on CBA patients (group A) and nonsyndromic type III biliary atresia (BA) patients (group B), who underwent laparoscopic Kasai portoenterostomy (LKPE) during the same period. RESULTS: There were no significant differences in operative time, conversion rate, or the incidence of any postoperative complications between groups A and B (P > 0.05). The mean age at surgery (P < 0.01), rates of clearance of jaundice (CJ), cholangitis (P < 0.05), and 5-year survival with a native liver (SNL) were significantly lower in group A than in group B. Among the 35 patients with CBA, the CJ and 5-year SNL rates were significantly better in those with type I (n = 27) than in those with type IIId (n = 8) (P < 0.05). CONCLUSIONS: LKPE is a feasible and safe procedure for CBA. The 5-year SNL after LKPE was better in patients with CBA than in those with nonsyndromic type III BA. The 5-year SNL after LKPE for type I CBA was better than that for type IIId CBA.
Assuntos
Atresia Biliar/cirurgia , Laparoscopia/métodos , Portoenterostomia Hepática/métodos , Fatores Etários , Atresia Biliar/classificação , Atresia Biliar/mortalidade , Criança , Pré-Escolar , Estudos de Viabilidade , Seguimentos , Humanos , Lactente , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: To identify changes in demographics, outcomes, and risk factors for patient and graft loss in patients with biliary atresia undergoing liver transplantation since Pediatric End-Stage Liver Disease implementation (2002). STUDY DESIGN: Demographics and outcomes were compared between patients enrolled in the Society of Pediatric Liver Transplantation registry before (n = 547) and after (n = 1477) 2002. Kruskal-and χ2 Wallis tests identified significant differences between eras. Risk factors for patient and graft loss after 2002 were determined by Cox regression model analysis of time to event data. RESULTS: Significant patient differences after 2002 support increasing disease severity including more status 1 patients and those with a derived Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease score of greater than 30 awaiting transplant. Both patient and graft survival improved after 2002 from 90% to 97% and 81% to 90%, respectively (primary transplant; P < .0001). Significant differences in complications within 30 days included reduced relisting for transplant, rejection, culture-positive infection, repeat operation, hepatic artery thrombosis, portal vein thrombosis, and death/transplant before discharge. Multivariable analysis identified deceased technical variant vs whole graft and retransplantation predictive for patient death, hazard ratios of 4.041 and 8.308, respectively. Deceased technical variant vs whole graft (hazard ratio, 1.963) and donor age 0-5 months vs 1-17 years (hazard ratio, 5.525) were risk factors for graft loss. CONCLUSIONS: The overall outcomes of patients receiving liver transplantation for patients with biliary atresia have improved since 2002 despite evidence of increased disease severity at the time of transplant. Risk factors impacting post-transplant morbidity and mortality in patients with biliary atresia are now mainly surgical including donor variables.
Assuntos
Atresia Biliar/classificação , Transplante de Fígado/mortalidade , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Adolescente , Atresia Biliar/cirurgia , Criança , Pré-Escolar , Doença Hepática Terminal/classificação , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Recém-Nascido , Transplante de Fígado/efeitos adversos , Estudos Longitudinais , Masculino , Sistema de Registros , Reoperação/estatística & dados numéricos , Fatores de Risco , Índice de Gravidade de DoençaRESUMO
OBJECTIVE: Biliary atresia (BA) is the most common cause of neonatal jaundice, characterized by progressive and rapid liver fibrosis. Recent studies have shown that microRNAs (miRNAs) contribute to the liver fibrogenesis. We investigated the miRNA-21 impact in liver fibrogenesis in Indonesian BA patients. RESULTS: There were 5, 4, and 7 BA patients with type 2A, 2B, and 3, respectively. Quantitative real-time polymerase chain reaction (qPCR) showed that the miRNA-21 expression was significantly increased (18-fold) in BA patients compared to controls (- 4.4 ± 4.0 vs. - 0.2 ± 4.8; p = 0.041). Furthermore, the phosphatase and tensin homolog deleted on chromosome ten (PTEN) expression was significantly down-regulated (3.1-fold) in BA group compared to control group (0.2 ± 1.4 vs. - 1.4 ± 1.7; p = 0.036). The α-smooth muscle actin (α-SMA) expression was not statistically significantly different between groups (13.7 ± 3.8 vs. 15.0 ± 4.8; p = 0.87). Interestingly, the miRNA-21 expression was significantly lower (25-fold) in cirrhosis than non-cirrhosis BA patients (- 0.8 ± 2.2 vs. - 5.3 ± 3.9; p = 0.004). In conclusions, our study provides support for the association between miRNA-21 expression and liver cirrhosis in BA patients. Further study with a larger sample size of patients is important to confirm our results.
Assuntos
Atresia Biliar/genética , Regulação da Expressão Gênica , Cirrose Hepática/genética , MicroRNAs/genética , Atresia Biliar/classificação , Atresia Biliar/complicações , Criança , Pré-Escolar , Regulação para Baixo , Feminino , Humanos , Indonésia , Lactente , Cirrose Hepática/complicações , Masculino , PTEN Fosfo-Hidrolase/genética , PTEN Fosfo-Hidrolase/metabolismoRESUMO
Hyperbilirubinemia is a common occurrence in neonates; it may be physiological or pathological. Conjugated hyperbilirubinemia may result from medical or surgical causes, and can result in irreversible liver damage if untreated. The aim of imaging is the timely diagnosis of surgical conditions like biliary atresia and choledochal cysts. Abdominal ultrasound is the first line imaging modality, and Magnetic resonance cholangiopancreatography (MRCP) also has a role, especially in pre-operative assessment of choledochal cysts (CDCs). For biliary atresia, the triangular cord sign and gallbladder abnormalities are the two most useful ultrasound features, with a combined sensitivity of 95%. Liver biopsy has an important role in pre-operative evaluation; however, the gold standard for diagnosis of biliary atresia remains an intra-operative cholangiogram. Choledochal cysts are classified into types according to the number, location, extent and morphology of the areas of cystic dilatation. They are often associated with an abnormal pancreaticobiliary junction, which is best assessed on MRCP. Caroli's disease or type 5 CDC comprises of multiple intrahepatic cysts. CDCs, though benign, require surgery as they may be associated with complications like cholelithiasis, cholangitis and development of malignancy. Severe unconjugated hyperbilirubinemia puts neonates at high risk of developing bilirubin induced brain injury, which may be acute or chronic. Magnetic resonance imaging of the brain is the preferred modality for evaluation, and shows characteristic involvement of the globus pallidi, subthalamic nuclei and cerebellum - in acute cases, these areas show T1 hyperintensity, while chronic cases typically show hyperintensity on T2 weighted images.
Assuntos
Diagnóstico por Imagem/métodos , Icterícia Neonatal/diagnóstico por imagem , Síndrome de Alagille/diagnóstico por imagem , Atresia Biliar/classificação , Atresia Biliar/diagnóstico , Atresia Biliar/diagnóstico por imagem , Bilirrubina , Biópsia , Lesões Encefálicas/induzido quimicamente , Lesões Encefálicas/diagnóstico por imagem , Colangiografia/métodos , Colangiopancreatografia por Ressonância Magnética/métodos , Colangite/diagnóstico por imagem , Cisto do Colédoco/classificação , Cisto do Colédoco/diagnóstico por imagem , Cisto do Colédoco/cirurgia , Hepatite/diagnóstico por imagem , Humanos , Recém-Nascido , Icterícia Neonatal/classificação , Icterícia Neonatal/cirurgia , Fígado/patologia , Imageamento por Ressonância Magnética/métodos , Ultrassonografia/métodosRESUMO
OBJECTIVES: Approximately 20% of cases of biliary atresia do not involve the gallbladder, the cystic duct, and the common bile duct. In these cases, a hepatoportocholecystostomy (HPC) may be performed instead of the classical hepatoportoenterostomy (HPE). METHODS: We reviewed our cohort to investigate the efficacy of HPC and the associated surgical complications and clinical problems. RESULTS: From 1984 to 2009, 97 patients underwent HPC in our institution. In the first 6 months of the postoperative period no patient presented with bacterial cholangitis. Nine patients had bile leakage, and 1 patient had a gallbladder obstruction. For these 10 patients, HPC was modified in HPE. Jaundice clearance was maintained after HPE, but 4 of the patients presented 1 or more cholangitis episodes. After 6 months, there were no cases of cholangitis recorded, 2 patients presented bile leakage and 4 patients experienced gallbladder obstruction. After 5 years of follow-up, 46% of the patients were jaundice free with their native liver and 29.4% were transplanted. CONCLUSIONS: In patients with biliary atresia with patent extrahepatic bile ducts, hepatoportocholecystomy is a good surgical technique that can prevent cholangitis. These results are counterbalanced by specific surgical complications that need to be known and looked for in the postoperative period.
Assuntos
Ductos Biliares/cirurgia , Atresia Biliar/cirurgia , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Vesícula Biliar/cirurgia , Anastomose Cirúrgica , Atresia Biliar/classificação , Atresia Biliar/diagnóstico , Atresia Biliar/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Portoenterostomia Hepática , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/terapia , Resultado do TratamentoRESUMO
Purpose To evaluate the diagnostic performance of ultrasonography (US) in the identification and exclusion of biliary atresia with a modified triangular cord thickness metric together with a gallbladder classification scheme, as well as hepatic artery (HA) diameter and liver and spleen size, in a large sample of jaundiced infants. Materials and Methods The ethics committee approved this study, and written informed parental consent was obtained. In 273 infants with conjugated hyperbilirubinemia (total bilirubin level ≥ 31.2 µmol/L, with direct bilirubin level > indirect bilirubin level), detailed abdominal US was performed to exclude biliary atresia. Biliary atresia was found in 129 infants and ruled out in 144. A modified triangular cord thickness was measured at the anterior branch of the right portal vein, and a gallbladder classification scheme was identified that incorporated the appearance of the gallbladder and a gallbladder length-to-width ratio of up to 5.2 when the lumen was visualized, as well as HA diameter and liver and spleen size. Reference standard diagnosis was based on results of one or more of the following: surgery, liver biopsy, cholangiography, and clinical follow-up. Area under the receiver operating characteristic curve (AUC) analysis, binary logistic regression analysis, Fisher exact test, and unpaired t test were performed. Results Triangular cord thickness, HA diameter, ratio of gallbladder length to gallbladder width, liver size, and spleen size exhibited statistically significant differences (all P < .05) between the group with biliary atresia and the group without. AUCs of triangular cord thickness, ratio of gallbladder length to width, and HA diameter were 0.952, 0.844, and 0.838, respectively. Logistic regression analysis demonstrated that these three US parameters were significantly associated (all P < .05) with biliary atresia. The combination of triangular cord thickness and gallbladder classification could yield comparable AUCs (0.915 vs 0.933, P = .400) and a higher sensitivity (96.9% vs 92.2%), compared with triangular cord thickness alone. Conclusion By using the combination of modified triangular cord thickness and gallbladder classification scheme, most infants with biliary atresia could be identified. (©) RSNA, 2015.
Assuntos
Atresia Biliar/diagnóstico por imagem , Vesícula Biliar/diagnóstico por imagem , Atresia Biliar/classificação , Feminino , Vesícula Biliar/patologia , Artéria Hepática/diagnóstico por imagem , Artéria Hepática/patologia , Humanos , Lactente , Recém-Nascido , Fígado/diagnóstico por imagem , Fígado/patologia , Masculino , Tamanho do Órgão , Baço/diagnóstico por imagem , Baço/patologia , UltrassonografiaRESUMO
PURPOSE: We reevaluated the impact of age at Kasai operation on the short- and long-term outcomes of type III biliary atresia (BA). PATIENTS AND METHODS: From 1953 to 2009, 242 patients with type III BA underwent Kasai operation at ages ranging between 12 and 421 days (average, 79.7 days). The relationship between age at Kasai operation and jaundice disappearance rates (JDRs), and 10-, 20-, and 30-year native liver survival rates (NLSRs) were assessed retrospectively (JDR [%] = the number of patients in whom jaundice disappeared/the number of patients in each group × 100). RESULTS: Age at Kasai operation had a significant impact on the JDRs (P < .001). However, there was no statistical relationship between long-term NLSR of the patients in whom jaundice disappeared after Kasai operation and operative age. From the results of the cumulative NLSRs estimated by Kaplan-Meier method, each survival rate was quite dependent on the age at operation until 30 years after Kasai operation, but the difference became much smaller in the later period provided age at operation was 4 months or younger. CONCLUSION: The operative age as a prognostic factor might be less significant in the long-term outcome than in the short-term outcome.
Assuntos
Atresia Biliar/cirurgia , Enterostomia/estatística & dados numéricos , Hospitais Universitários/estatística & dados numéricos , Fígado/cirurgia , Fatores Etários , Atresia Biliar/classificação , Atresia Biliar/complicações , Atresia Biliar/epidemiologia , Seguimentos , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Icterícia Obstrutiva/etiologia , Icterícia Obstrutiva/cirurgia , Estimativa de Kaplan-Meier , Transplante de Fígado/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: To identify the epidemiological characteristics of infants with biliary atresia in England and Wales, since centralisation of its management in 1999. METHODS: The care of infants with biliary atresia (BA) in England and Wales is centralised to only three centres. All infants (treated from January 1999 to December 2006) were identified from a prospective national database; demographic details were ascertained from medical records and compared between two groups based on presumed aetiology (isolated biliary atresia (IBA) and developmental biliary atresia (DBA) (for example, syndromic infants, biliary atresia splenic malformation, cystic biliary atresia)). RESULTS: There were 302 (133 male (44%)) infants with BA that could be divided into IBA (n = 219, 73%) and DBA (n = 76, 25%). The overall incidence was 0.58/10 000 (1 in 17,049) live births with marked regional differences along a north-west/south-east axis varying from 0.38 (north-west England) to 0.78 (south-east England)/10,000 live births (OR 2.05 (95% CI 1.26-3.41); p = 0.002). The commonest month of birth was September with December being the least common, although there was no evidence for significant seasonal variation (p = 0.2). Infants with DBA were more likely to be female (p<0.001), of white background (p = 0.01), first-born (p = 0.04) and to be formula-fed (p = 0.07). Infants of south Asian origin came to surgery at an older age (59 (IQ 45-75) versus 52 (IQ 42-65) days; p = 0.03). CONCLUSIONS: There is a remarkable variation of incidence of biliary atresia within England and Wales, some of which may have been caused by factors related to a different aetiological and racial background.
Assuntos
Atresia Biliar/epidemiologia , Atresia Biliar/classificação , Peso ao Nascer , Inglaterra/epidemiologia , Etnicidade , Feminino , Idade Gestacional , Humanos , Incidência , Lactente , Masculino , Vigilância da População , Estudos Prospectivos , Características de Residência , Fatores de Risco , Estações do Ano , País de Gales/epidemiologiaRESUMO
INTRODUCTION: Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA. Because some cases can be detected antenatally, CBA in general may have its origins in utero life. We assessed our experience with CBA. METHODS: Single-center retrospective review of infants with CBA over a 13-year period (January 1994 to December 2006) was done. Data are given as medians (range). RESULTS: Of 270 infants with BA, 29 (9 male) were identified as CBA. Antenatal ultrasonography had detected an abnormality in 12 (41%) infants at a median of 22 weeks (17-34 weeks) of gestation. All infants underwent postnatal excision and Kasai portoenterostomy (KP). Those with antenatally detected CBA came to surgery younger (36 [14-67] vs 48 days [35-147 days], P = .004). Twenty cysts (69%) had a fibroinflammatory wall with no biliary epithelial lining and 6 (26%) contained bile. Age at KP was significantly and positively correlated (r = 0.46, P = .01) with liver fibrosis, as assessed in liver biopsy materials obtained at KP, but not with grade of "hepatocyte disarray" (P = .74). Twenty infants (69%) cleared their jaundice (bilirubin <20 mumol/L) within 6 months after KP. Age at KP markedly affected outcome. CONCLUSION: Cystic BA is a clinically distinct variant of BA. Despite onset in prenatal life, earlier than presumed for isolated BA, it has a better prognosis, particularly with early surgery.
Assuntos
Atresia Biliar , Cistos , Atresia Biliar/classificação , Atresia Biliar/diagnóstico , Atresia Biliar/etiologia , Atresia Biliar/cirurgia , Cistos/classificação , Cistos/diagnóstico , Cistos/etiologia , Cistos/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos RetrospectivosRESUMO
The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.
Assuntos
Atresia Biliar/classificação , Atresia Biliar/cirurgia , Cistos/diagnóstico por imagem , Cistos/cirurgia , Portoenterostomia Hepática/métodos , Atresia Biliar/diagnóstico por imagem , Colangiografia , Feminino , Humanos , Lactente , Recém-Nascido , MasculinoRESUMO
PURPOSE: This retrospective study reviews the long-term outcome of type I biliary atresia (BA). METHODS: Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated. RESULTS: The overall survival rate of the nontransplant type I patients was better than that of the type II/III patients (52% vs 33%, P = .0009). Cholangiograms of 32 patients were classified into 3 types: cloudy (48%), treelike (13%), and mixed (39%). Of 26 patients who underwent corrective surgery in 1972 or later, 7 (50%), 7 (78%), and 3 (100%) patients of the cloudy type, mixed type, and treelike type, respectively, have survived without liver transplantation (LTx). Of 18 type I patients who survived more than 20 years without LTx, 7 developed severe late complications. Two of them eventually required LTx after 20 years old. CONCLUSIONS: Use of cholangiograms during corrective surgery might have a long-term prognostic value. The overall survival rate of type I BA was better than that of type II/III. The incidence of late complications was, however, considerably high in the type I survivors. All patients required careful long-term follow-up.
Assuntos
Atresia Biliar/diagnóstico por imagem , Atresia Biliar/cirurgia , Colangiografia , Portoenterostomia Hepática , Anastomose Cirúrgica , Atresia Biliar/classificação , Humanos , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND & AIMS: To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature. METHODS: Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations." RESULTS: A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8). CONCLUSIONS: BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required.
Assuntos
Atresia Biliar/classificação , Atresia Biliar/epidemiologia , Cisto do Colédoco/epidemiologia , Atresia Biliar/cirurgia , Comorbidade , Feminino , Humanos , Lactente , Transplante de FígadoRESUMO
BACKGROUND/PURPOSE: The aim of this study was to determine simple predictors for quality of life (QOL) in long-term jaundice-free survivors after the Kasai operation. METHODS: Kasai's original portoenterostomy was performed on 55 patients with biliary atresia. Among them, records were reviewed retrospectively of 35 long-term (at least 5 years) and jaundice-free (clearance in bilirubin level less than 1.0 mg/dL after Kasai operation) survivors. The patients were divided into 2 groups based on QOL, and the differences in clinical and laboratory data were analyzed statistically. RESULTS: The ages at Kasai operation, histologic, fibrosis grade of liver biopsy specimen at operation, the first onset and frequency of postoperative cholangitis, and postoperative clearance speed of jaundice after Kasai operation were not significantly different between the 2 groups. The aspartate aminotransferase (AST) level at 1 year was significantly correlated with the serum concentration of hyaluronic acid and an independent predictor for QOL in long-term jaundice-free survivors of the Kasai operation. CONCLUSIONS: The serum AST level at 1 year was a simple, strong predicting factor of QOL and liver dysfunction in long-term jaundice-free survivors after Kasai operation and may prove useful in planning liver transplantation.
Assuntos
Atresia Biliar/cirurgia , Portoenterostomia Hepática , Aspartato Aminotransferases/sangue , Atresia Biliar/classificação , Atresia Biliar/complicações , Bilirrubina/sangue , Colangite/sangue , Colangite/etiologia , Humanos , Ácido Hialurônico/sangue , Lactente , Recém-Nascido , Icterícia/complicações , Portoenterostomia Hepática/efeitos adversos , Qualidade de Vida , Recidiva , Estudos RetrospectivosRESUMO
BACKGROUND: There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis. METHODS: All infants who had undergone surgery for BA during the period 1980 through 2000 aged > or =100 days were reviewed. Actuarial survival was calculated using 2 end-points (death and transplantation). A retrospective review of their ultrasonography (n = 12) and preoperative liver histology (n = 22) was also undertaken to ascertain possible predictive criteria. RESULTS: A total of 422 infants had BA diagnosed during this period, of which 35 (8.2%) were > or =100 days at surgery (median [interquartile range], 133 [range, 108 to 180] days). Surgery included portoenterostomy (n = 26), hepaticojejunostomy (n = 7), and a resection and end-to-end anastomosis (n = 1). A laparotomy only was performed in 1. Five- and 10-year actuarial survival rate with native liver was 45% and 40%, respectively. Currently, 12 (35%) patients are alive with their native liver (8 are anicteric), 9 (28%) have undergone transplantation, and 13 have died. Although there were some survival advantages for types 1 or 2 BA and "noncirrhosis" at time of surgery, neither reached statistical significance. Individual histologic features (eg, degrees of fibrosis, giant cell transformation, bile duct destruction) in the retrospective review of available material were not discriminatory. The finding of a "heterogeneous" parenchyma on ultrasonography was predictive of poor outcome but lacked sensitivity. CONCLUSIONS: The potential for reasonable medium-term survival is present in about one third of infants 100 days or older coming to primary corrective surgery. In the absence of accurate discrimination, the authors continue to favor this option rather than subject all to transplant simply on the basis of age.
Assuntos
Atresia Biliar/cirurgia , Portoenterostomia Hepática , Anormalidades Múltiplas , Atresia Biliar/classificação , Atresia Biliar/complicações , Atresia Biliar/mortalidade , Atresia Biliar/patologia , Seguimentos , Artéria Hepática/diagnóstico por imagem , Humanos , Hiperbilirrubinemia/etiologia , Hipertensão Portal/diagnóstico por imagem , Hipertensão Portal/etiologia , Lactente , Jejuno/cirurgia , Tábuas de Vida , Fígado/cirurgia , Cirrose Hepática Biliar/etiologia , Cirrose Hepática Biliar/mortalidade , Transplante de Fígado , Portoenterostomia Hepática/estatística & dados numéricos , Valor Preditivo dos Testes , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , UltrassonografiaRESUMO
BACKGROUND/PURPOSE: Antiinflammatory cytokine (interleukin [IL]-1ra) and proinflammatory cytokines (tumor necrosis factor [TNF]- alpha and IL-6) appear to play important roles in the pathophysiology of liver disease. In this study, the authors attempt to identify the roles of IL-1ra, IL-6, and TNF-alpha at different stages in postoperative biliary atresia (POBA) patients. METHODS: Serum concentrations of IL-1ra, IL-6, and TNF-alpha were measured in 34 POBA patients and 25 healthy controls using specific enzyme-linked immunosorbent assays (ELISA). POBA patients were classified into 5 groups: group I (n = 21), less than 3 years old; Ia, stable; Ib, mild liver dysfunction; Ic, severe liver dysfunction. Group II (n = 13), (more than 3 years old); IIa, stable; IIb, variable liver dysfunction. RESULTS: For IL-1ra differences between controls and groups Ib, Ic, and IIa were statistically significant (P <.01), and for IL-6, differences between controls and groups Ia, Ib, Ic, and IIb were statistically significant (P <.01). Groups Ib and Ic (less than 3 years old subjects) had the highest levels in this series. Serum TNF-alpha was not detected in any groups. CONCLUSION: The relationship between serum levels of IL-6 and IL-1ra may be predictive of prognosis with respect to the progression of fibrosis in POBA patients.
Assuntos
Atresia Biliar/sangue , Interleucina-6/sangue , Receptores de Interleucina-1/antagonistas & inibidores , Sialoglicoproteínas/sangue , Fator de Necrose Tumoral alfa/análise , Adolescente , Atresia Biliar/classificação , Criança , Pré-Escolar , Progressão da Doença , Humanos , Proteína Antagonista do Receptor de Interleucina 1RESUMO
In 3 (9 %) of 34 children with biliary atresia, US revealed gallbladder contraction following an oral feed, given on admission, but not with subsequent feeds. Surgery revealed a Kasai type IIIa biliary atresia with a patent communication between the gallbladder and duodenum. We propose that the bile ducts may initially have been patent, but then gradually became obliterated secondary to inflammation. These cases may explain the development of one type of biliary atresia.
Assuntos
Atresia Biliar/diagnóstico por imagem , Esvaziamento da Vesícula Biliar/fisiologia , Atresia Biliar/classificação , Atresia Biliar/etiologia , Feminino , Vesícula Biliar/diagnóstico por imagem , Vesícula Biliar/fisiologia , Hepatite/diagnóstico por imagem , Humanos , Recém-Nascido , Icterícia Neonatal/diagnóstico por imagem , Masculino , UltrassonografiaRESUMO
BACKGROUND: It is not easy to discriminate between infantile hepatitis and biliary atresia in spite of several diagnostic tests including laboratory analyses, ultrasound, and hepatobiliary scans. ERCP is the most useful procedure for visualization of the extrahepatic biliary system, but ERCP is still an uncommon procedure in children. METHODS: ERCP examination was performed in 52 infants with biliary atresia (10 with infantile hepatitis, 5 with congenital biliary dilatation, 3 with paucity of intrahepatic bile duct, 2 with duodenal atresia, and 1 with postoperative jaundice of hepatoblastoma) aged from 8 days to 300 days (mean, 71 days). RESULTS: ERCP was successful in 47 with biliary atresia, in 9 with infantile hepatitis, and 10 with another disease. Liver biopsy was performed in 1 infant with hepatitis in whom the cannulation failed; in 9 with hepatitis in whom the cannulation was successful, exploratory laparotomy could be avoided. The ERCP findings in 46 patients with biliary atresia (excluding 1 in whom evaluation could not be performed because of poor x-ray quality) were classified into four patterns. CONCLUSIONS: A success rate of ERCP examinations in infants was 88%, so ERCP is recommended to make a correct decision regarding the need for surgery in cholestatic disorders.
Assuntos
Atresia Biliar/diagnóstico por imagem , Colangiopancreatografia Retrógrada Endoscópica , Atresia Biliar/classificação , Colangiopancreatografia Retrógrada Endoscópica/instrumentação , Colangiopancreatografia Retrógrada Endoscópica/métodos , Ducto Colédoco/diagnóstico por imagem , Meios de Contraste , Diagnóstico Diferencial , Diatrizoato de Meglumina , Feminino , Hepatite/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , MasculinoRESUMO
The outcome of 21 survivors (8 males, 13 females; age range, 11 to 29 years) followed for > 10 years after surgery for biliary atresia are discussed. Of the 21 patients, 18 with type III disease had hepatic portoenterostomy (Kasai operation), and 3 with type I disease had hepaticoenterostomy. Twenty patients are alive leading almost normal lives; however, 13 (61.9%) patients did have a history of complications, including hemorrhage from esophageal varices in 10, from a gastric ulcer or erosion in 3, and from a duodenal ulcer in 2; biliary reobstruction in 3; and multiple pulmonary arteriovenous fistulae in 2. Of those with complications, 7 required surgery. One died suddenly at the age of 19 years of a bleeding gastric ulcer. Liver function is normal in 9 (45.0%) of the 20 alive patients, 2 (10.0%) have slight hepatic dysfunction, and 9 (45.0%) have mild-to-moderate hepatic dysfunction. Liver function is almost normal in 3 patients who had a history of variceal hemorrhage.