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1.
Khirurgiia (Mosk) ; (3): 5-13, 2024.
Artigo em Russo | MEDLINE | ID: mdl-38477238

RESUMO

OBJECTIVE: To improve postoperative outcomes in newborns and infants with choledochal cysts and to determine the indications for surgery. MATERIAL AND METHODS: There were 13 children aged 0-3 months with choledochal cyst who underwent reconstructive surgery between 2019 and 2023. In all children, choledochal cyst was associated with cholestasis. Acholic stool was observed in almost half of the group (n=7). All children underwent cyst resection and Roux-en-Y hepaticoenterostomy. RESULTS: Symptoms of cholestasis regressed in all patients. Mean surgery time was 128±27 min. There were no complications. Enteral feeding was started after 1-2 postoperative days, abdominal drainage was removed after 6.2±1.6 days. Mean length of hospital-stay was 16±3.7 days. Adequate bile outflow is one of the main principles. For this purpose, anastomosis with intact tissues of hepatic duct should be as wide as possible. Roux-en-Y loop should be at least 40-60 cm to prevent postoperative cholangitis. CONCLUSION: Drug-resistant cholestasis syndrome and complicated choledochal cysts (cyst rupture, bile peritonitis) are indications for surgical treatment in newborns and infants. When forming Roux-en-Y hepaticoenterostomy, surgeon should totally excise abnormal tissues of the biliary tract to prevent delayed malignant transformation.


Assuntos
Cisto do Colédoco , Colestase , Laparoscopia , Criança , Lactente , Humanos , Recém-Nascido , Cisto do Colédoco/diagnóstico , Cisto do Colédoco/cirurgia , Portoenterostomia Hepática , Colestase/cirurgia , Ducto Hepático Comum/cirurgia , Bile , Anastomose em-Y de Roux
2.
Sci Rep ; 14(1): 2492, 2024 01 30.
Artigo em Inglês | MEDLINE | ID: mdl-38291117

RESUMO

Bile acids have received increasing attention as a marker of the long-term prognosis and a potential therapeutic target in patients with biliary atresia, which is a progressive disease of the hepatobiliary system. A detailed analysis of serum and urinary bile acid compositions was conducted to assess the characteristics of bile acid profiles and the correlation between bile acid profiles and liver fibrosis markers in adult patients with biliary atresia who achieved bilirubin normalization. Serum total bile acids and glucuronide-conjugated (glyco- and tauro-) cholic acids (GCA and TCA) and chenodeoxycholic acids (GCDCA and TCDCA) were significantly higher in patients with biliary atresia than in healthy controls, whereas unconjugated CA and CDCA showed no significant difference. There were no significant differences in CA to CDCA ratios and glycine-to-taurine-conjugated ratios. Urinary glycocholic acid 3-sulfate (GCA-3S) was significantly higher in patients with biliary atresia. Serum GCDCA showed a strong positive correlation with Mac-2 binding protein glycosylation isomer (M2BPGi). These results demonstrate that bile acid congestion persists into adulthood in patients with biliary atresia, even after cholestasis has completely improved after Kasai portoenterostomy. These fundamental data on bile acid profiles also suggest the potential value of investigating bile acid profiles in patients with biliary atresia.


Assuntos
Atresia Biliar , Colestase , Humanos , Atresia Biliar/cirurgia , Ácidos e Sais Biliares , Fígado , Portoenterostomia Hepática/métodos , Colestase/cirurgia , Ácido Quenodesoxicólico
3.
Langenbecks Arch Surg ; 409(1): 30, 2024 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-38189999

RESUMO

PURPOSE: Many patients with biliary atresia (BA) after the Kasai procedure (KP) progress to death or require liver transplantation to achieve long-term survival; however, most cases of death/liver transplantation (D/LT) occur in the early period after KP (usually within 1 year). This study was designed to construct a convenient nomogram for predicting early D/LT in patients with BA after KP. METHODS: A BA cohort was established in May 2017, and up to May 2023, 112 patients with 1-5 years of follow-up were enrolled in the study and randomly (ratio, 3:1) divided into a training cohort for constructing a nomogram (n = 84) and a validation cohort (n = 28) for externally validating the discrimination and calibration. The training cohort was divided into two groups: the early D/LT group (patients who died or had undergone LT within 1 year after KP [n = 35]) and the control group (patients who survived through the native liver more than 1 year after KP [n = 49]). Multivariate logistic regression and stepwise regression were applied to detect variables with the best predictive ability for the construction of the nomogram. The discrimination and calibration of the nomogram were internally and externally validated. RESULTS: The Kaplan-Meier (K-M) curve showed an actual 1-year native liver transplantation (NLS) rate of 57.1% and an estimated 2-year NLS rate of 55.2%. By multivariate regression and stepwise regression, age at KP, jaundice clearance (JC) speed 1 month after KP, early-onset PC (initial time < 36.5 days) after KP, sex, aspartate aminotransferase-to-platelet ratio index (APRI), and weight at KP were identified as the independent variables with the best ability to predict early D/LT and were used to construct a nomogram. The developed nomogram based on these independent variables showed relatively good discrimination and calibration according to internal and external validation. CONCLUSION: Most D/LTs were early D/LTs that occurred within 1 year after KP. The established nomogram based on predictors, including sex, weight at the KP, the APRI, age at the KP, JC speed 1 month after the KP, and early PC, may be useful for predicting early D/LT and may be helpful for counseling BA patients about patient prognosis after KP. This study was retrospectively registered at ClinicalTrials.gov (NCT05909033) in June 2023.


Assuntos
Atresia Biliar , Transplante de Fígado , Portoenterostomia Hepática , Humanos , Atresia Biliar/cirurgia , Fígado , Nomogramas
4.
Eur Radiol ; 34(3): 1493-1501, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37646810

RESUMO

OBJECTIVES: To investigate the feasibility of using preoperative imaging indices to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia (BA). MATERIALS AND METHODS: The retrospective review included 190 BA patients who underwent the Kasai procedure between 2000 and 2020, with preoperative US and/or MRI, excluding cases with less than 2-year follow-up period. Multivariable logistic regression analysis was performed to identify imaging indices to predict 2-year native liver survival. Kasai failure was defined as the need for liver transplantation or death within 2 years of the Kasai procedure. RESULTS: Of the 90 patients included, all had preoperative US, and 61 also had MRI. Kasai failure occurred in 52% (47/90). Preoperative US identified gallbladder length (OR 0.40, 95% CI 0.17-0.95, p = 0.039; cutoff 1.6 cm, AUC 67.66) and biliary cysts (OR 24.64, 95% CI 1.97-308.08, p = 0.013) as significant Kasai failure predictors, with a combined accuracy of 73% (60/82). For patients having both preoperative US and MRI, significant predictors were hepatic artery diameter (OR 6.75, 95% CI 1.31-34.88, p = 0.023; cutoff 2 mm, AUC 73.83) and biliary cysts (OR 23.89, 95% CI 1.43-398.82, p = 0.027) on US, and gallbladder length (OR 0.25, 95% CI 0.08-0.76, p = 0.014; cutoff 1.2 cm, AUC 74.72) and spleen size (OR 2.53, 95% CI 1.02-6.29, p = 0.045; cutoff 6.9 cm, AUC 73.72) on MRI, with a combined accuracy of 85% (52/61). CONCLUSION: Preoperative US and/or MRI enhance the 2-year native liver survival prediction in BA patients after the Kasai procedure. CLINICAL RELEVANCE STATEMENT: BA patients with hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and absence of biliary cysts (US/MRI) have a decreased likelihood of 2-year native liver survival. KEY POINTS: • Preoperative US and/or MRI can predict the probability of achieving 2-year native liver survival following the Kasai procedure. • Combining US and MRI improved the accuracy to 85% for predicting 2-year native liver survival in BA patients. • The hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and no biliary cysts (US/MRI) are significant predictors of Kasai failure in patients with biliary atresia.


Assuntos
Doenças dos Ductos Biliares , Atresia Biliar , Cistos , Transplante de Fígado , Humanos , Lactente , Atresia Biliar/diagnóstico por imagem , Atresia Biliar/cirurgia , Portoenterostomia Hepática/métodos , Fígado/diagnóstico por imagem , Fígado/cirurgia , Transplante de Fígado/métodos , Estudos Retrospectivos , Resultado do Tratamento
5.
Hepatol Commun ; 7(12)2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38051554

RESUMO

BACKGROUND: We aimed to quantify ductular reaction (DR) in biliary atresia using a neural network in relation to underlying pathophysiology and prognosis. METHODS: Image-processing neural network model was applied to 259 cytokeratin-7-stained native liver biopsies of patients with biliary atresia and 43 controls. The model quantified total proportional DR (DR%) composed of portal biliary epithelium (BE%) and parenchymal intermediate hepatocytes (PIH%). The results were related to clinical data, Sirius Red-quantified liver fibrosis, serum biomarkers, and bile acids. RESULTS: In total, 2 biliary atresia biopsies were obtained preoperatively, 116 at Kasai portoenterostomy (KPE) and 141 during post-KPE follow-up. DR% (8.3% vs. 5.9%, p=0.045) and PIH% (1.3% vs. 0.6%, p=0.004) were increased at KPE in patients remaining cholestatic postoperatively. After KPE, patients with subsequent liver transplantation or death showed an increase in DR% (7.9%-9.9%, p = 0.04) and PIH% (1.6%-2.4%, p = 0.009), whereas patients with native liver survival (NLS) showed decreasing BE% (5.5%-3.0%, p = 0.03) and persistently low PIH% (0.9% vs. 1.3%, p = 0.11). In Cox regression, high DR predicted inferior NLS both at KPE [DR% (HR = 1.05, p = 0.01), BE% (HR = 1.05, p = 0.03), and PIH% (HR = 1.13, p = 0.005)] and during follow-up [DR% (HR = 1.08, p<0.0001), BE% (HR = 1.58, p = 0.001), and PIH% (HR = 1.04, p = 0.008)]. DR% correlated with Sirius red-quantified liver fibrosis at KPE (R = 0.47, p<0.0001) and follow-up (R = 0.27, p = 0.004). A close association between DR% and serum bile acids was observed at follow-up (R = 0.61, p<0.001). Liver fibrosis was not prognostic for NLS at KPE (HR = 1.00, p = 0.96) or follow-up (HR = 1.01, p = 0.29). CONCLUSIONS: DR predicted NLS in different disease stages before transplantation while associating with serum bile acids after KPE.


Assuntos
Atresia Biliar , Aprendizado Profundo , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Prognóstico , Portoenterostomia Hepática/métodos , Cirrose Hepática/diagnóstico , Cirrose Hepática/cirurgia , Cirrose Hepática/complicações , Ácidos e Sais Biliares
7.
BMC Pediatr ; 23(1): 589, 2023 11 22.
Artigo em Inglês | MEDLINE | ID: mdl-37993876

RESUMO

OBJECTIVES: To validate an appropriate evaluation method of liver fibrosis assessment based on the unique pathological features of biliary atresia (BA) that could well predict its prognosis. METHODS: A total of 68 patients with BA who underwent Kasai procedure (KP) and an intraoperative liver biopsy, followed up from January 2019 to December 2021, were recruited in a retrospective analysis. Ishak, Metavir, and BA-specific staging systems in relation to outcomes were analyzed using logistic regression, COX proportional hazard regression, Kaplan-Meier analysis, etc. RESULTS: Kaplan-Meier analysis determined a significant difference in native liver survival according to the BA-specific stage (p = 0.002). The ROC curve analysis for predicting prognosis showed that the AUC of BA-specific staging combined with iBALF and severe bile duct proliferation (BDP) (0.811, 95% CI: 0.710-0.913, p < 0.0001) was higher than BA-specific staging alone (0.755, 95% CI: 0.639-0.872, p < 0.001). CONCLUSIONS: The BA-specific staging system reflects the condition of the liver fibrosis, and its combination with iBALF and severe BDP helps to better evaluate the prognosis of patients with BA.


Assuntos
Atresia Biliar , Humanos , Lactente , Atresia Biliar/cirurgia , Portoenterostomia Hepática , Prognóstico , Estudos Retrospectivos , Cirrose Hepática
9.
Pediatr Surg Int ; 39(1): 299, 2023 Nov 20.
Artigo em Inglês | MEDLINE | ID: mdl-37985521

RESUMO

PURPOSE: Stool color (SC) for monitoring prednisolone use in biliary atresia (BA) patients after laparoscopic portoenterostomy (LPE) was reviewed. METHODS: Subjects were 47 post-LPE BA patients given a reducing dose course of intravenous prednisolone. The course started at 4 mg/kg/day and gradually reduced, ultimately reaching a final total dose (TD) of 31.5 mg/kg. Normal SC indicated a course could progress until finished and was repeated until jaundice clearance (JC) was achieved. Abnormal SC persisting for two consecutive courses was the absolute indication for redo or liver transplantation (LTx). RESULTS: JC was achieved in 38/47 (80.9%) LPE cases and 4/6 redos to give an overall JC rate (JCR) of 42/47 (89.4%). Outcomes after one course (n = 5; JCR: 80.0%; median TD: 30.0 mg/kg, interquartile range [IQR: 26.0-31.5]), two courses (n = 10; JCR: 90.0%; median TD: 62.5 mg/kg [IQR: 60.8-66.0]), three courses (n = 13; JCR: 92.3%; median TD: 90.0 mg/kg [IQR: 86.0-90.0]), four courses (n = 10; JCR: 80.0%; median TD: 120.0 mg/kg [IQR: 116.7-123.3]), five courses (n = 7; JCR: 100%; median TD: 156.0 mg/kg [IQR: 154.3-157.5]), six courses (n = 1; JCR: 100%; TD: 189.0 mg/kg), ten courses (n = 1; JCR: 100%; TD: 308 mg/kg). CONCLUSION: Indications for repeat prednisolone and timing of redo/LTx based on SC monitoring appeared effective based on high JCR and successful redo/LTx. LEVELS OF EVIDENCE: III.


Assuntos
Atresia Biliar , Icterícia , Laparoscopia , Humanos , Lactente , Atresia Biliar/cirurgia , Atresia Biliar/tratamento farmacológico , Prednisolona/uso terapêutico , Portoenterostomia Hepática , Resultado do Tratamento , Estudos Retrospectivos
10.
Saudi J Gastroenterol ; 29(5): 286-294, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37787348

RESUMO

Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. Here, we analyzed the data from the Saudi national BA study (204 BA cases diagnosed between 2000 and 2018) to identify the prognostic factors of BA outcomes. Methods: One hundred and forty-three cases underwent KPE. Several prognostic factors (center case load, congenital anomalies, serum gamma-glutamyl transferase, use of steroids, ascending cholangitis post-operatively, and degree of portal fibrosis at time of KPE) were investigated and correlated with the primary outcomes of interest: 1) success of KPE (clearance of jaundice and total serum bilirubin <20 mmol/l after KPE), 2) survival with native liver (SNL), and 3) overall survival. Results: Use of steroids after KPE was associated with clearance of jaundice, 68% vs. 36.8% in the BA cases that did not receive steroids (P = 0.013; odds ratio 2.5) and a significantly better SNL rate at 2 - and 10-year of 62.22% and 57.77% vs. 39.47% and 31.57%, respectively (P = 0.01). A better 10-year SNL was observed in centers with caseload <1/year (group 1) as compared to centers that performed ≥1/year (group 2) [45.34% vs. 26.66%, respectively; P = 0.047]. On comparison of the 2 groups, cases in group 1 had KPE at significantly earlier age (median 59.5 vs. 75 days, P = 0.006) and received steroids after KPE more frequently than group 2 (69% vs. 31%, P < 0.001). None of the remaining prognostic variables were identified as being significantly related to BA outcome. Conclusion: Steroids use post-KPE predicted clearance of jaundice and better short- and long-term SNL. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize the pre- and post-operative clinical practices and facilitate clinical and basic research to evaluate factors that influence BA outcome.


Assuntos
Atresia Biliar , Icterícia , Portoenterostomia Hepática , Humanos , Lactente , Atresia Biliar/cirurgia , Atresia Biliar/complicações , Icterícia/diagnóstico , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Esteroides , Resultado do Tratamento , Transplante de Fígado
11.
Int J Surg ; 109(12): 4009-4017, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-37678274

RESUMO

BACKGROUND: Cholangitis is common in patients with biliary atresia following Kasai portoenterostomy (KPE). The prompt use of empiric antibiotics is essential due to the lack of identified microorganisms. The authors aimed to validate a severity grading system to guide empiric antibiotic therapy in the management of post-KPE cholangitis. MATERIALS AND METHODS: This multicenter, prospective, randomized, open-label study recruited patients with post-KPE cholangitis and was conducted from January 2018 to December 2019. On admission, patients were categorized into mild, moderate, and severe cholangitis according to the severity grading system. Patients in the mild cholangitis group were randomized to receive cefoperazone sodium tazobactam sodium (CSTS) or meropenem (MEPM). Patients with severe cholangitis were randomized to treatment with MEPM or a combination of MEPM plus immunoglobulin (MEPM+IVIG). Patients with moderate cholangitis received MEPM. RESULTS: The primary endpoint was duration of fever (DOF). Secondary outcomes included blood culture, length of hospital stay, incidence of recurrent cholangitis, jaundice clearance rate, and native liver survival (NLS). For mild cholangitis, DOF, and length of hospital stay were similar between those treated with CSTS or MEPM (all P >0.05). In addition, no significant difference in recurrence rate, jaundice clearance rate, and NLS was observed between patients treated with CSTS and MEPM at 1-month, 3-month, and 6-month follow-up. In patients with moderate cholangitis, the DOF was 36.00 (interquartile range: 24.00-48.00) h. In severe cholangitis, compared with MEPM, MEPM+IVIG decreased DOF and improved liver function by reducing alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and direct bilirubin at 1-month follow-up. However, recurrence rate, jaundice clearance rate, and NLS did not differ significantly between MEPM+IVIG and MEPM at 1-month, 3-month, and 6-month follow-up. CONCLUSIONS: In patients with post-KPE cholangitis, MEPM is not superior to CSTS for the treatment of mild cholangitis. However, MEPM+IVIG treatment was associated with better short-term clinical outcomes in patients with severe cholangitis.


Assuntos
Atresia Biliar , Colangite , Icterícia , Criança , Humanos , Lactente , Portoenterostomia Hepática/efeitos adversos , Estudos Prospectivos , Imunoglobulinas Intravenosas , Atresia Biliar/cirurgia , Atresia Biliar/complicações , Colangite/tratamento farmacológico , Colangite/etiologia , Icterícia/complicações , Antibacterianos/uso terapêutico , Meropeném , Estudos Retrospectivos , Resultado do Tratamento
12.
J Pediatr Gastroenterol Nutr ; 77(5): 648-654, 2023 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-37705401

RESUMO

OBJECTIVES: A connection between the bowel and bile ducts after the Kasai hepatoportoenterostomy (HPE) procedure poses a risk of ascending cholangitis. There were only a few evidence-based consensuses on the benefits of prophylactic antibiotics. This study aims to assess the value of prophylactic antibiotics in reducing the risk of cholangitis following the Kasai HPE procedure. METHODS: Meta-analysis is performed using random-effects model from the search result of 5 online databases (PubMed, Google Scholar, EBSCO MEDLINE, ClinicalTrials.gov , and EuropePMC) from inception to October 27, 2021. The keywords used were "antibiotic," "antimicrobial," "Kasai," "portoenterostomy," "biliary atresia," and "bile duct atresia." Cochrane Risk of Bias tool and Newcastle-Ottawa Scale is used to assess the risk of bias. The outcomes are incidence of cholangitis and native liver survival. RESULTS: Six studies consisting of 4 cohorts and 2 cross-sectional studies were extracted. A total of 714 patients reported different cholangitis incidence after prophylactic antibiotics administration post-Kasai HPE. The incidence of cholangitis following Kasai HPE was not statistically significant among participants. There is conflicting evidence on the efficacy of antibiotics in prolonging native liver survival. CONCLUSIONS: The existing evidence does not support the administration of prophylactic antibiotics in preventing cholangitis after Kasai HPE among biliary atresia patients. Additionally, their roles in native liver survival are still inconclusive. The fact that there were heterogeneous method and antibiotic usage between existing studies must also be highlighted for better design in future studies.


Assuntos
Atresia Biliar , Colangite , Humanos , Criança , Lactente , Atresia Biliar/complicações , Estudos Transversais , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Portoenterostomia Hepática/efeitos adversos , Colangite/etiologia , Colangite/prevenção & controle , Antibacterianos/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento
13.
J Pediatr Gastroenterol Nutr ; 77(6): e93-e98, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-37697468

RESUMO

PURPOSE: Recent studies demonstrate the success of Kasai portoenterostomy for biliary atresia (BA) is linearly related to infant age at time of Kasai. We sought to review the feasibility and safety of laparoscopic needle micropuncture cholangiogram with concurrent core liver biopsy (if needed) for expedited exclusion of BA in patients with direct conjugated hyperbilirubinemia. METHODS: Expedited laparoscopic cholangiogram and liver biopsy were instituted at our facility for infants with direct hyperbilirubinemia for whom clinical exam and laboratory workup failed to diagnose. A retrospective chart review was performed in infants <1 year with hyperbilirubinemia from 2016 to 2021. Demographics, preoperative evaluation, procedure details, and complications were reviewed. RESULTS: Two hundred ninety-seven infants with unspecified jaundice were identified, of which, 86 (29%) required liver biopsy. Forty-seven percutaneous liver biopsies were obtained including 8 (17%) in whom BA could not be excluded. Laparoscopic cholangiogram was attempted in 47 infants following basic workup; BA was diagnosed in 22 infants (47%) of which 3 were <18 days old. Biliary patency was demonstrated laparoscopically in 22 of 25 (88%); 3 (12%) required conversion to open cholangiogram. Infants with percutaneous liver biopsy had an average delay of 3 days (range: 2-36) to cholangiogram. Preoperative studies and liver biopsy alone did not reliably exclude the diagnosis of BA. CONCLUSION: Laparoscopic cholangiogram with liver biopsy is a safe procedure resulting in the confirmation or exclusion of BA in infants. Forty-seven percent of infants who underwent laparoscopic cholangiogram were found to have BA; those who were surgical candidates underwent Kasai during the same operation.


Assuntos
Atresia Biliar , Laparoscopia , Humanos , Lactente , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Atresia Biliar/complicações , Biópsia/efeitos adversos , Hiperbilirrubinemia/diagnóstico , Laparoscopia/métodos , Fígado/patologia , Portoenterostomia Hepática/métodos , Estudos Retrospectivos , Resultado do Tratamento , Estudos de Viabilidade
14.
J Laparoendosc Adv Surg Tech A ; 33(9): 904-908, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37417990

RESUMO

Background: An aberrant vascular anatomy might present a technical pitfall for biliary atresia (BA) surgery. The purpose of this study was to report the rare cases and discuss the significance and management strategy for BA with an aberrant right hepatic artery (ARHA) by laparoscopic Kasai procedure in children. Methods: The subjects for this study were 10 consecutive type III BA patients with an ARHA who had laparoscopic Kasai procedure at our institute between January 2012 and August 2021. The common bile duct was mobilized between the right hepatic artery and the right branch of portal vein, and then lifted to the liver hilum. The fibrous cord was transected and then the laparoscopic Kasai was carried out. Results: All patients survived the laparoscopic Kasai without any intraoperative complications. The mean operative time was 235 minutes for each laparoscopic Kasai. The mean follow-up time was 32.6 months. The total and direct bilirubin dropped to normal within 4 months of surgery in 7 patients. One patient died of repeated cholangitis and liver failure 1 year after surgery. In the additional 2 patients the bilirubin levels dropped significantly after the surgery but elevated again because of repeated cholangitis and requiring ongoing observation and intermittent treatment. Conclusions: With the perfect laparoscopic skills, the common bile duct could be safely mobilized between the right hepatic artery and right branch of portal vein for the infants with type III BA associated with an ARHA, and laparoscopic Kasai could be carried out safely and successfully.


Assuntos
Atresia Biliar , Colangite , Laparoscopia , Lactente , Criança , Humanos , Atresia Biliar/cirurgia , Portoenterostomia Hepática/efeitos adversos , Artéria Hepática/cirurgia , Resultado do Tratamento , Laparoscopia/métodos , Colangite/etiologia , Bilirrubina , Estudos Retrospectivos
17.
J Pediatr Surg ; 58(12): 2347-2351, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37468346

RESUMO

AIMS: The outcomes following surgical treatment of infants with biliary atresia (BA) varies across the world with many possible confounding factors. APRi (AST-to-platelet ratio index) is a simple surrogate marker of liver fibrosis and we sought to determine its long-term relationship (if any) with outcome post-Kasai portoenterostomy (KPE). METHODS: Prospectively acquired database (Jan 1998-Dec 2021). Clearance of jaundice was defined as achieving <20 umol/L post-KPE. Categorical and survival data were tested using Chi2 tests and a log rank test respectively. P ≤ 0.05 was regarded as significant. Data are quoted as median (interquartile range) unless otherwise stated. RESULTS: There were 473 infants with a calculated APRi at time of KPE [0.70 (IQR 0.45-1.2)] and known outcomes. There was significant but moderate correlation with age at KPE (rS = 0.43; P < 0.0001). APRi was divided into quartiles (1st 0.11-0.44, n = 120; 2nd 0.45-0.69, n = 120; 3rd 0.70-1.18, n = 115 and 4th 1.2-15.1; n = 118). There was a clear distinction in APRi levels between CMV + ve BA and the other groups (Syndromic BA, Cystic BA, Isolated BA), with an overrepresentation of CMV IgM + ve BA in the higher APRi quartiles (Χ2 = 26.6; P = 0.0002). Clearance of jaundice showed a stepwise decrease across the quartiles (67%; 58%; 55%; 49%; overall Χ2 = 7.8, P = 0.049 and P = 0.005 for trend). Decreasing native liver survival also showed a significant trend (P = 0.01). CONCLUSION: APRi appears to be of fundamental prognostic value in stratifying the BA population. In our series, CMV status was associated with higher APRi score and appears to be different. This simple variable offers an objective method of assessing the biological status of BA at presentation and variability between different series. LEVEL OF EVIDENCE: II (prospective comparison).


Assuntos
Atresia Biliar , Infecções por Citomegalovirus , Icterícia , Lactente , Humanos , Portoenterostomia Hepática , Atresia Biliar/cirurgia , Fígado/cirurgia , Icterícia/cirurgia , Infecções por Citomegalovirus/complicações , Resultado do Tratamento , Estudos Retrospectivos
19.
Hepatol Commun ; 7(8)2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-37471052

RESUMO

BACKGROUND: Biliary atresia (BA) is likely caused by a common phenotypic response to various triggers; one proposed trigger, cytomegalovirus (CMV), may lead to worse outcomes. The aim of this study was to determine the severity of disease and pretransplant outcomes of infants with BA, who have evidence of CMV (CMV+) at diagnosis compared with CMV-negative (CMV-) infants. METHODS: The study used data and biospecimens from the Childhood Liver Disease Research Network PROBE study of cholestatic infants. Plasma obtained at the time of hepatic portoenterostomy (HPE) of 249 infants with BA was tested for CMV by DNA-PCR and CMV-IgM. Comparisons between CMV+ and CMV- infants were made using Wilcoxon rank sum, Student t test, chi-square, or Fisher exact test. Native liver survival (NLS) outcomes were analyzed using Kaplan-Meier and Cox regression adjusting for age at HPE; pretransplant patient survival outcomes were analyzed using a competing risk model and adjusting for age at HPE. RESULTS: CMV+ infants (n = 29, 12%) underwent HPE later (67.8±13.6 d vs. 55.1±18.5 d, p = 0.0005) and had higher baseline alkaline phosphatase and aminotransferases. There was no difference between groups in jaundice clearance or NLS. The subdistribution HR of pretransplant death for CMV+ infants adjusted for age at HPE was 3.8 (p = 0.034). CONCLUSIONS: CMV infection at the time of HPE in infants with BA is not associated with worse NLS despite the association with worse liver injury, older age at HPE, and increased risk of pretransplant death adjusted for age at HPE. Continued evaluation of the consequences of CMV infection and the effects of antiviral treatment should be explored.


Assuntos
Atresia Biliar , Infecções por Citomegalovirus , Lactente , Humanos , Criança , Atresia Biliar/cirurgia , Citomegalovirus , Fígado/cirurgia , Portoenterostomia Hepática , Infecções por Citomegalovirus/complicações
20.
Transplant Proc ; 55(5): 1214-1222, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37302864

RESUMO

BACKGROUND: Liver transplantation is a life-saving treatment for end-stage pediatric liver failure. We aimed to present the results of pediatric liver transplants performed in our center in the last 11 years (between 2012 and March 2022) in association with prognostic factors affecting survival. METHODS: Demographic characteristics, etiologic reasons, previous operations (Kasai procedure), morbidity, mortality, survival, and bilio-vascular complication rates were determined, and outcomes were evaluated. In the postoperative period, the duration of mechanical ventilation and intensive care unit stay and surgical and other complications were evaluated. Graft and patient survival rates were determined, and univariate and multivariate factors affecting these rates were evaluated. RESULTS: In the last 10 years, 229 pediatric liver transplantaion (Pe-LT)/1513 adult liver taransplantation (Ad-LT) (21.35%) were performed in our center. This ratio (Pe-LT/Ad-LT ratio) is 1741/15,886 (10.95%) for our country. A total of 229 liver transplants were performed in 214 pediatric patients. Retransplantation was performed in 15 patients (6.55%). Cadaveric liver transplantation was performed in 9 patients. Graft survival rates were 87%, 83%, 78%, 78%, 78%, and 78% at <30 days, 30 to 90 days, 91 to 364 days, 1 to 3 years, and >3 years, respectively. Patient survival rates for <30 days, 30 to 90 days, 91 to 364 days, 1 to 3 years, and >3 years were 91.5%, 85.7%, 82%, 81.5%, and 81.5%, respectively. Our 5-year survival rates in metabolic diseases and the acute fulminant failure group are 93.8% and 100%, respectively. CONCLUSIONS: The fact that the 1- and 5-year survival rates are the same shows that when patients overcome biliary vascular and infectious problems, their survival is prolonged.


Assuntos
Falência Hepática , Transplante de Fígado , Adulto , Humanos , Criança , Transplante de Fígado/métodos , Falência Hepática/cirurgia , Portoenterostomia Hepática , Reoperação/efeitos adversos , Taxa de Sobrevida , Sobrevivência de Enxerto , Resultado do Tratamento , Estudos Retrospectivos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia
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