Application value of single-port laparoscopic left lateral donor liver acquisition in pediatric living donor liver transplantation / 中华消化外科杂志

Objective:To investigate the application value of single-port laparoscopic left lateral donor liver acquisition in pediatric living donor liver transplantation (PLDLT).Methods:The retrospective and descriptive study was conducted. The clinical data of the donor and recipient who were admitted to Beijing Friendship Hospital of Capital Medical University for PLDLT in January 2020 were collected. The donor was a male, aged 28 years with body mass as 62 kg, height as 174 cm and body mass index (BMI) as 20.5 kg/m 2. The recipient was the daughter of the donor, aged 1 year with body mass as 9 kg, height as 75 cm and BMI as 16.0 kg/m 2. The donor underwent single-port laparoscopic left lateral donor liver acquisition. The recipient underwent living donor liver trans-plantation by the same operation team. Observation indicators: (1) intraoperative conditions; (2) postoperative conditions; (3) follow-up. Results:(1) Intraoperative conditions. The donor under-went single-port laparoscopic left lateral donor liver acquisition successfully, with the single-port access system being placed through a transumbilical incision. The operation time, the warm ischemia time of the donor liver and volume of intraoperative blood loss were 240 minutes, 3 minutes and 40 mL, respectively, of the donor. The weight of the donor liver was 233.6 g, and the corrected graft-to-recipient body weight ratio was 2.60%. The recipient underwent living donor liver transplantation successfully. (2) Postoperative conditions. The donor began to take liquid diet at postoperative day 1, and results of laboratory examination showed that the alanine aminotransferase (ALT), aspartate aminotransferase (AST), glutamyltransferase (GGT) and total bilirubin (TBil) of the donor was 239 U/L, 116 U/L, 53 U/L and 22.57 μmol/L. The donor began to take diet with high quality proteins at postoperative day 2, and to get for out-of-bed activities moderately. The donor′s peritoneal drainage fluid was light red at postoperative day 3, and no fluid accumulation was found in the operation area after abdominal B-ultrasound examination, so the peritoneal drainage tube was removed. The donor was discharged at postoperative day 4. The liver function of the recipient recovered to normal level 2 weeks after the operation. (3) Follow-up. The donor was followed up by outpatient examination 2 weeks after discharged, and results of laboratory examination showed that the ALT, AST, GGT and TBil was 44 U/L, 25 U/L, 53 U/L and 9.22 μmol/L, respectively. Neither the donor nor the recipient had complication ≥Ⅱ grade of the Clavien-Dino classification, such as biliary fistula and vascular complication during the 6 months after operation.Conclusion:Single-port laparoscopic left lateral donor liver acquisition can be used into the PLDLT.

Risk factors of acute kidney injury in children with biliary atresia after liver transplantation / 国际外科学杂志

Objective:To analyze the incidence and risk factors of acute kidney injury in children with biliary atresia after liver transplantation.Methods:The retrospective case-control study was conducted.The clinical data of 115 children with biliary atresia who received liver transplantation for the first time in Beijing Friendship Hospital Affiliated to Capital Medical University from December 2018 to November 2020 were collected.The patients were divided into AKI group ( n=39) and non-AKI group ( n=76) according to the diagnostic criteria of the Kidney Disease Improving Global Outcomes(KDIGO). The differences of clinical indicators between the two groups were compared, and multivariate logistic regression analysis was performed for statistically significant variables ( P<0.05) to further determine the independent risk factors for AKI after liver transplantation. The measurement data of normal distribution were expressed as mean±standard deviation ( ± s), and t-test was used for comparison between groups.Measurement data with non-normal distribution were represented by M( Q1, Q3), and Mann-Whitney U test was used for comparison between groups.Count data were expressed as cases and percentage, and comparisons between groups were made using Chi-square test or Fisher′s exact test. Results:The incidence of AKI in biliary atresia patients after liver transplantation was 33.9%. Univariate analysis showed that there were statistically significant differences in age ( OR=0.721, 95% CI: 0.553-0.938, P=0.014), preoperative infection ( OR=3.307, 95% CI: 1.294-8.468, P=0.013), PELD score ( OR=1.065, 95% CI: 1.031-1.101, P<0.001), serum creatinine numerical value ( OR=0.745, 95% CI: 0.657-0.858, P<0.001), intraoperative red blood cell transfusion ( OR=1.034, 95% CI: 1.028-1.051, P<0.001) and intraoperative plasma transfusion ( OR=1.055, 95% CI: 1.025-1.086, P=0.002) between the AKI group and the non-AKI group ( P< 0.05). Multivariate logistic regression analysis was performed on the selected indicators by univariate analysis, and the results showed that preoperative infection ( OR=3.763, 95% CI: 1.185-11.945, P=0.025) and low serum creatinine ( OR=0.685, 95% CI: 0.570-0.823, P<0.001), intraoperative red blood cell transfusion ( OR=1.033, 95% CI: 1.015-1.056, P=0.028) was independently associated with postoperative AKI ( P<0.05). The inpatient treatment time in ICU and in hospital between the two groups were statistically significant ( P<0.05). Conclusions:Preoperative infection, low creatinine numerical value and intraoperative red blood cell transfusion are independent risk factors for postoperative AKI in children with biliary atresia. AKI may prolong the time in ICU and in hospital.

The correlation between the expression levels of vitamin D receptor in bile duct epithelial cells and the prognosis of biliary atresia / 国际儿科学杂志

Objective:To investigate the expression of vitamin D receptor(VDR)in biliary epithelial cells of children with biliary atresia(BA)and explore the correlation between VDR epression levels and clinical pathological prognosis.Methods:A total of 48 BA patients who underwent Kasai surgery in the Pediatric Surgery Department of the Second Affiliated Hospital of Xi′an Jiaotong University from January 2017 to December 2020 with confirmed pathological results were selected as the study subjects.Immunohistochemistry was used to determine the expression of VDR in biliary epithelial cells, and Masson staining was used to determine the degree of liver tissue fibrosis.Based on the VDR expression levels, the 48 BA patients were divided into the significantly low VDR expression group(30 cases)and the normal/high expression group(18 cases).Laboratory testing results within 1 week before Kasai surgery and liver shear wave elastography(SWE)data were collected for all patients.Follow-up was conducted for a period of 0 to 60 months after Kasai surgery or liver transplantation, meanwhile, the occurrence of refractory cholangitis and auto-liver survival time were collected.Results:There was a negative correlation between the degree of liver fibrosis and SWE value in children with BA( r=-0.805, P<0.01).In comparison between the two groups, the significantly low VDR expression group had higher SWE values[(20.57±1.28)kPa vs.(18.02±1.41)kPa, P<0.05], higher liver injury biochemical indicators[ALT(215.8±24.7)U/L vs.(182.6±21.2)U/L, P=0.021; AST(165.4±22.3)U/L vs.(139.6±21.4)U/L, P=0.014], a higher frequency of post-Kasai surgery refractory cholangitis(60.00% vs.22.22%, P=0.037), and a shorter median autologous liver survival time(27.00 months vs.36.00 months, P=0.032)than those in the normal/high expression group. Conclusion:The significant decrease in VDR expression in biliary epithelial cells may serve as an indicator of poor prognosis in BA.

Atresia biliar e seus impactos para a criança acometida e sua família: revisão integrativa

Objetivo: Identificar através da literatura impactos que a atresia biliar provoca em crianças acometidas e em sua família. Método: Trata-se de uma revisão integrativa da literatura realizada entre julho e setembro de 2022, utilizando bases de dados e bibliotecas SCOPUS, PUBMED e EBSCO. Realizou-se adequação dos descritores para busca de acordo com a base ou biblioteca, sendo estes: caregivers, "biliary atresia", "child care", family, child, "child care subject", combinados com o operador booleano AND. Os estudos foram selecionados de forma criteriosa e sistematizada, avaliados quanto ao nível de evidência. Resultados: Na busca primária, identificou-se 1441 artigos e após criteriosa seleção, 7 estudos foram selecionados para compor a análise qualitativa. Obteve-se como principais resultados dos impactos da atresia biliar para as crianças, dizem respeito as consequências oriundas da doença no que se refere a necessidade de procedimento cirúrgico que impactam negativamente na qualidade de vida. Já os impactos para a família dizem respeito a consequências para a saúde mental e baixa qualidade de vida aliados ao desconhecimento sobre cuidados com a criança e excesso de preocupação. Conclusão: Este estudo possibilitou a identificação dos impactos que a atresia biliar provoca em crianças acometidas e em sua família, apontando contribuições significativas para o cuidado em enfermagem.

Objetivo: Identificar a través de la literatura los impactos que la atresia biliar provoca en los niños afectados y en su familia. Método: Se trata de una revisión integradora de la literatura, realizada entre julio y septiembre de 2022, utilizando como bases de datos y bibliotecas SCOPUS, PUBMED y EBSCO. Se realizó la adecuación de los descriptores en busca de concordancia con la base o biblioteca, siendo estos: caregivers, "biliary atresia", "child care", family, child, "child care subject", combinados con el operador booleano AND. Los estudios fueron seleccionados de forma juiciosa y sistematizada, evaluados en cuanto al nivel de evidencia. Resultados: En la búsqueda primaria fueron identificados 1441 artículos y, después de una cuidadosa selección, 7 estudios fueron seleccionados para componer el análisis cualitativo. Los principales resultados de los impactos de la atresia biliar para los niños son las consecuencias de la enfermedad con relación a la necesidad de cirugía, que impactan negativamente en la calidad de vida. Por otro lado, los impactos para la familia están relacionados a las consecuencias para la salud mental y baja calidad de vida, asociados a la falta de conocimiento sobre el cuidado del niño y preocupación excesiva. Conclusión: Este estudio permitió identificar los impactos que la atresia biliar provoca en los niños afectados y su familia, y aporta contribuciones significativas para el cuidado en enfermería.

Objective: To identify through literature the impacts that biliary atresia causes in affected children and their families. Method: This is an integrative review of literature carried out between July and September 2022, using databases and libraries: SCOPUS, PUBMED, and EBSCO. The descriptors for the search were adjusted according to the database or library, being caregivers, "biliary atresia", "child care", family, child, "child care subject", combined with the boolean operator AND. The studies were selected in a careful and systematic way and evaluated for the level of evidence. Results: In the primary search, 1441 articles were identified, and after careful selection, 7 studies were selected to compose the qualitative analysis. The main results of the impacts of biliary atresia for children refer to the consequences arising from the disease regarding the need for surgical procedures that negatively impact quality of life. The impacts for the family relate to consequences for mental health and low quality of life, combined with lack of knowledge about caring for the child and excessive worry. Conclusion: This study made it possible to identify the impacts that biliary atresia causes in affected children and their families, pointing to significant contributions to nursing care.

In vitro fertilization: an unexpected finding in a cohort of patients with biliary atresia

In biliary atresia (BA), efforts to prevent premature liver transplantation (LT) are aimed at early diagnosis, timing of Kasai-portoenterostomy (KPE), and centralization of care. This report presents the clinical picture, treatment strategies, and outcomes of BA patients with no previous treatment. A retrospective cohort study (Jan/2001 to Jan/2021) was conducted to evaluate the outcome of patients with BA referred to a single team. Study groups were: 1) Kasai-only group (K-only) n=9), 2) LT-only group (n=7), and 3) Kasai+LT group (K+LT) (n=23). Survival with native liver and overall survival were 22.9 and 94.8%, respectively, at 120 months of follow-up. There was no difference in age at KPE in the K-only group (46.8±21.8 days) vs K+LT (52.1±22 days), P=0.4. Ten (25.6%) patients were babies conceived through in vitro fertilization (IVF). Four IVF patients (40%) presented associated congenital heart disease vs 5 patients (17%) in the remaining group (P=0.14). Two of the IVF patients were premature (<37 weeks). Median maternal age at birth was 35 years (33 to 41 years). Excellent patient survival is expected for patients with BA with the available treatment strategies. IVF+BA was an unexpected prevalent association in this cohort, and further studies are required to better understand these findings.

Alagille syndrome misdiagnosed as biliary atresia a case report and literature review / 中国热带医学

@#Abstract: By report a case in which the main symptom was cholestasis in an infant and the diagnosis of Alagille syndrome (ALGS) was made after a tortuous treatment process, so as to provide clinicians with experience in diagnosing this type of patient. The patient was a 1-year and 11-month-old male who was admitted to the hospital with "abnormal liver function found for more than 1 year". Physical examination showed a wide forehead, sunken eye sockets, wide eye spacing, a sharp chin, and a grade II systolic murmur in the pulmonary valve region. Biochemical findings showed abnormal liver function accompanied by significant elevation of total bile acids and γ-glutamyl transpeptidase. CT scan of the thoracic vertebrae showed sagittal vertebral fractures in the thoracic 3-7 vertebrae, and pulmonary arteriography showed pulmonary stenosis and genetic testing indicated a JAG1 mutation. Combining the patient's specific facial features, heart defects, spinal deformities, and bile stasis clinical symptoms, along with the genetic analysis results, the final diagnosis was confirmed as Alagille syndrome. Alagille syndrome is the most common cause of chronic cholestasis with phenotypic features and is a dominant inherited disease involving multiple systems. Most patients present with bile stasis as the main symptom within the first three months after birth. Alagille syndrome needs to be distinguished from various forms of cholestasis in infancy, and since biliary atresia requires early surgical treatment, most children with cholestasis as the main clinical manifestation are considered to have biliary atresia at an early stage and undergo a caesarean section. If Alagille syndrome is misdiagnosed as biliary atresia, and surgery may worsen the prognosis. Therefore, the biggest challenge in the early diagnosis of Alagille syndrome is how to distinguish it from biliary atresia. Therefore, physicians need to improve their knowledge of rare cholestatic liver disease in clinical practice to accurately identify rare cholestatic liver disease in the early stages of the disease, and improve improve their diagnosis and treatment levels.

A Rare Case of Cystic Biliary Atresia

@#Biliary atresia is one of the commonest causes of prolonged jaundice in neonate, whereas cystic biliary atresia is a rare form of biliary atresia with similar presentation, but a slightly different sonographic finding. Being able to differentiate them radiologically is important as the surgical management is different for these two diagnoses. An ultrasound examination was done for a 2-month-old baby girl with prolonged jaundice with the finding of a cyst along the portal triad, which raised the suspicion for cystic biliary atresia. The diagnosis was confirmed with intraoperative cholangiography (IOC) and Kasai procedure was done. The patient was well after the surgery with resolution of jaundice.

Variation of amniotic fluid γ-glutamyl transferase level at 19-23 +6 gestational weeks in normal pregnancies / 中华围产医学杂志

Objective:To detect the levels of γ-glutamyl transferase (GGT) in the amniotic fluid of normal pregnancies at 19-23 +6 gestational weeks and to analyze the changes in GGT level with gestational age. Methods:This study retrospectively collected the amniotic fluid supernatant from 383 singleton pregnant women (102, 103, 82, 68 and 28 cases at 19-19 +6, 20-20 +6, 21-21 +6, 22-22 +6, 23-23 +6 weeks of gestation, respectively) who underwent amniocentesis for prenatal diagnosis but had normal genetic diagnosis results in Cheeloo Hospital of Shandong University from January 2021 to September 2022. The levels of GGT in the amniotic fluid supernatant were tested and the statistical parameters including xˉ± s, min-max, median ( M), P1, P2.5, P5, P95, P97.5 and P99 values of GGT levels at each gestational week were calculated. GGT were non-normal data and converted into natural logarithms (lnGGT), and a least square linear regression equation was established to analyze the relationship between lnGGT and gestational week. Results:At 19-19 +6, 20-20 +6, 21-21 +6, 22-22 +6, and 23-23 +6 gestational weeks, the xˉ± s of amniotic fluid GGT were (385.8±235.7), (331.8±219.4), (253.7±197.9), (226.7±166.4), and (155.3±96.8) U/L, and the weekly declines were 14.0%, 23.5%, 10.6%, and 31.5%, respectively; the M values were 311.0, 288.0, 199.0, 160.5, and 105.5 U/L, and the weekly declines were 7.4%, 30.9%, 19.3%, and 34.3%, respectively; the P1- P99 were 67.1-1 404.5, 63.2-1 189.1, 36.0-849.8, 44.0-787.3, and 32.0-375.6 U/L, respectively. lnGGT was negatively correlated with gestational age ( R 2=0.148, P<0.001). Conclusions:In normal pregnancies at 19-23 +6 gestational weeks, GGT levels in amniotic fluid decrease with gestational age. Therefore, gestational age should be considered when establishing the reference value for amniotic fluid GGT in normal pregnancies.

To compare the different hepatic portal exposure techniques in Kasai surgery during their different perioperative periods / 中华肝胆外科杂志

Objective:To compare the impact of different portal exposure techniques in the Kasai surgery on children with type Ⅲ. biliary atresia during their different perioperative periods.Methods:A retrospective study was performed on the data of children with type Ⅲ. biliary atresia who underwent Kasai surgery at Fujian Children's Hospital from January 2017 to October 2020. Of 45 children enrolled in this study, there were 24 males and 21 females, aged (71.3±21.0) days. Patients who had left and right branches of the portal vein and the left and right hepatic arteries in the portal area being completely freed and elastically stretched during the Kasai operation were included into the free group ( n=22) and the remaining patients were included in the control group ( n=23). Postoperative hospital stay, postoperative direct bilirubin levels, postoperative complications and transplant-free survival after the Kasai operation were compared between the 2 groups. Results:Postoperative hospital stay of (17.1±4.4) d in the free group was significantly lower than that in the control group (20.1±5.4) d, ( t=2.07, P=0.044). The direct bilirubin level at 3 months after surgery for the control group was 30.0 (109, 108.0)μmol/L, which was significantly higher than that of 14.5 (4.0, 37.5) μmol/L in the free group ( Z=-2.16, P=0.031). Twenty-one patients (91.3%) in the control group had frequent attacks of postoperative cholangitis, compared with 13 patients (59.1%) in the free group. The difference was statistically significant (χ 2=4.69, P=0.030). Eleven surviving patients (47.8%) in the control group did not undergo liver transplantation at one year after surgery, compared with 15 patients (68.2%) in the free group. At two years after surgery, 7 surviving patients (30.4%) in the control group did not undergo liver transplantation compared with 10 patients (45.5%) in the free group. Conclusion:For children with type Ⅲ. biliary atresia, completely freeing the left and right branches of portal vein, and left and right hepatic arteries in the liver portal area, and elastically stretching these vessels to expose the portal area of the liver during Kasai surgery increased surgical safety and reduced hospital stay.

¿Reconocemos adecuadamente la acolia/ hipocolia en los lactantes?

Objetivo: El objetivo de este estudio fue valorar el nivel de acierto del personal de salud en la identificación de hipocolia/acolia en un escenario simulado. Con respecto a esto afirmamos que la hipocolia/acolia son signos clínicos que indican obstrucción biliar, relacionándose con enfermedades colestásicas obstructivas, siendo la atresia de vías biliares una de las principales causas en los primeros meses de vida; en esta, el pronóstico del manejo quirúrgico depende del diagnóstico temprano. Métodos: Estudio descriptivo/ prospectivo basado en la realización de una prueba virtual al personal de salud de Medellín, dividiéndose según sitio de trabajo, edad, sexo y profesión. Fueron utilizadas cinco imágenes de la tabla de colores de materia fecal, utilizada en Taiwán, previa autorización de sus autores. Se consideró reconocimiento adecuado cuando el participante identificaba como anormales las tres imágenes que representaban la acolia/hipocolia. Resultados: Se obtuvo respuesta de 442 participantes (se excluyeron 53) y solo 253 (65 %) lograron reconocimiento adecuado. En el análisis por subgrupos se encontró que hubo un mayor acierto en los participantes del Hospital Pablo Tobón Uribe con respecto a otras instituciones (69 y 54.6 %, respectivamente - p. 0.005). Los participantes con menor porcentaje de acierto fueron los médicos generales en un (45 %). Conclusión: Durante la valoración simulada sobre la identificación adecuada de acolia/hipocolia se encontró que solo el 65 % de la población participante acertó en la identificación correcta de este signo clínico, lo que sugiere una mayor atención en cuanto al entrenamiento para la identificación de acolia/hipocolia.

Objetive: The objective of this study was to assess the level of success of healthcare worker in the identification of hypocholia / acholia in a simulated scenario. With respect to this, we affirm that hypocholia/acholia are clinical signs indicative of biliary obstruction, related to obstructive cholestatic diseases, being bile duct atresia one of the main causes in the first months of life. In this, the prognosis of surgical management depends on the early diagnosis. Methods: Descriptive and prospective study, based on the analysis of a virtual survey to Medellín's healthcare workers, subgroup analysis was done according to workplace, age, sex and profession. Five images were used from the Taiwan Stool Color Card, with the authorization from their authors. Adequate recognition was considered when the participant identified the three images representing acholia / hypocholia as abnormal. Results: a response was obtained from 442 participants (53 were excluded), and only 253 (65 %) achieved adequate recognition. In the analysis by subgroups, it was found that there was a greater success in the Pablo Tobón Uribe Hospital participants with respect to other institutions (69 % and 54.6 %, respectively - p. 0.005). The participants with the lowest percentage of correct answers were general practitioners (45%). Conclusion: During the simulated assessment on the adequate identification of acholia / hypocolia, it was found that only 65% of the participating population was correct in the right identification of this clinical sign, which suggests greater attention in training for the identification of acholia / hypocolia.

Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia

Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.

Ultra-structural and histopathological features of liver biopsy taken during laparotomy to confirm the diagnosis of biliary atresia

Background: Neonatal cholestasis is caused by a group of diseases that cause jaundice, which can be encountered in the neonatal period. Biliary atresia (BA) and idiopathic neonatal hepatitis (INH) are among neonatal cholestasis diseases. Aims: The aim of this study was to perform histopathological and ultra-structural examinations of liver biopsy tissue samples from BA and INH patients with liver biopsies taken during laparotomy to confirm the diagnosis of biliary atresia. Settings and Design: A total of patients undergoing Kasai surgery before the age of 60 days were included in an “early” group (n = 7), whereas patients undergoing surgery after the age of 60 days were included in a “late” group (n = 11). The control group (n = 11) included INH patients. Materials and Methods: For histopathological examinations, liver tissue samples obtained intra-operatively were subjected to routine histopathological procedures after being stained with caspase-3 and cytokeratin-7 antibodies. Ultra-structural evaluations were also performed. Statistical analysis used: For comparisons between the groups, a one-way analysis of variance (ANOVA) test and the Mann–Whitney U test were used for continuous variables. Results: Histopathological findings reflected the specific liver pathologic findings seen in biliary atresia. Although there was no significant difference between the BA groups, these parameters were not detected in the control group. The histopathological evaluations revealed no significant differences in the findings of liver parenchyma damage between the early, late, and control groups. Electron microscopic examinations showed that the patients in the late group had more severe signs of intra-cellular damage to the liver. Conclusions: Although the histopathological examination revealed no significant differences in liver damage between the three groups, in ultra-structural evaluation, intra-cellular damage was found to be less in groups with better prognosis. Electron microscopy evaluations of intra-cellular damage may be more useful in this respect.

Research advances on the relationship between age at Kasai portoenterostomy and prognosis for biliary atresia / 中华肝胆外科杂志

Kasai portoenterostomy is the preferred treatment for biliary atresia. Age at Kasai portoenterostomy is an important factor affecting the prognosis of children with biliary atresia and avoiding liver transplantation. Choosing the appropriate surgical age, restoring good bile drainage, improving the native liver survival rate, and avoiding early liver transplantation are the items that clinicians have always been working on. The age at Kasai portoenterostomy was correlated with the jaundice clearance rate, native liver survival rate, and the incidence of postoperative cholangitis. This article systematically reviewed the research advances on the relationship between age at Kasai portoenterostomy and prognosis for biliary atresia, aiming to provide the basis for the ideal surgical age of Kasai.

Pre-operative Non-Invasive Imaging for Neonatal Cholestasis in a Child with Extrahepatic Biliary Atresia

@#Hepatobiliary iminodiacetic acid (HIDA) scintigraphy is a non-invasive, functional imaging of the hepatobiliary system that serves as an adjunct imaging modality for neonatal cholestasis work-up. In view of the urgency to diagnose biliary atresia and restore bile flow through surgery, HIDA scintigraphy could help to distinguish between neonatal cholestasis due to biliary atresia and neonatal hepatitis of various causes. We describe a full-term male infant with jaundice beyond the physiological period in which HIDA scintigraphy showed absent tracer excretion from the biliary system into the intestines up to 5 hours on follow-up imaging. The intraoperative diagnosis confirmed the diagnosis of biliary atresia. The prognosis of the patient with biliary atresia depends on early surgical planning and intervention. Therefore, non-invasive diagnostic tools play an important role in the evaluation of a child with neonatal cholestasis.

Omphalocele and biliary atresia: chance or causality. A case report

ABSTRACT To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.

Metabolic and nutritional repercussions of liver disease on children: how to minimize them? / Repercussões metabólicas e nutricionais da doença hepática em crianças: como minimizá-las?

ABSTRACT Objective: To describe the metabolic and nutritional repercussions of chronic liver disease (CLD), proposing strategies that optimize nutritional therapy in the pre- and post-liver transplantation (LT) period, in order to promote favorable clinical outcomes and adequate growth and development, respectively. Data sources: Bibliographic search in the PubMed, Lilacs and SciELO databases of the last 12 years, in English and Portuguese; target population: children from early childhood to adolescence; keywords in Portuguese and their correlates in English: "Liver Transplant," "Biliary Atresia," "Nutrition Therapy," "Nutritional Status," and "Child"; in addition to Boolean logics "and" and "or," and the manual search of articles. Data synthesis: Malnutrition in children with CLD is a very common condition and an important risk factor for morbidity and mortality. There is an increase in energy and protein demand, as well as difficulties in the absorption of carbohydrates, lipids and micronutrients such as fat-soluble vitamins and some minerals. An increase in the supply of energy, carbohydrates and proteins and micronutrients, especially fat-soluble vitamins, iron, zinc and calcium, is suggested, except in cases of hepatic encephalopathy (this restriction is indicated for a short period). Conclusions: Based on metabolic changes and anthropometric and body composition monitoring, a treatment plan should be developed, following the nutritional recommendations available, in order to minimize the negative impact of malnutrition on clinical outcomes during and after LT.

RESUMO Objetivo: Revisar de forma descritiva as repercussões metabólicas e nutricionais da doença hepática crônica, propondo estratégias que aperfeiçoem a terapia nutricional nos períodos pré e pós transplante hepático (TxH), para promover desfechos clínicos favoráveis e crescimento e desenvolvimento adequados, respectivamente. Fontes de dados: Pesquisa bibliográfica nas bases de dados PubMed, Lilacs e SciELO dos últimos 30 anos em língua inglesa e portuguesa; população-alvo: crianças da primeira infância até a adolescência; palavras-chave em português e seus correlatos em inglês: "transplante de fígado", "atresia biliar," "terapia nutricional", "estado nutricional" e "criança"; além das lógicas booleanas and e or e da busca manual de artigos. Síntese dos dados: A subnutrição em crianças com doença hepática crônica é uma condição muito comum e um importante fator de risco para a morbimortalidade. Ocorre aumento das demandas de energia e proteínas, bem como dificuldades na absorção de carboidratos, lipídeos e de micronutrientes como vitaminas lipossolúveis e alguns minerais. Sugere-se incremento no aporte de energia, carboidratos, proteínas e micronutrientes, sobretudo de vitaminas lipossolúveis, ferro, zinco e cálcio, exceto em casos de encefalopatia hepática (a restrição é indicada por um curto período). Conclusões: Com base nas alterações metabólicas, no monitoramento antropométrico e na composição corporal, um plano terapêutico deve ser elaborado, seguindo as recomendações nutricionais disponíveis, com o objetivo de minimizar o impacto negativo da subnutrição nos desfechos clínicos durante e após o transplante hepático.

Pediatric liver transplantation for Alagille syndrome: a single-center experience / 器官移植

Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.

Clinical significance of expression of leptin in patients with biliary atresia and hepatic fibrosis / 中华肝胆外科杂志

Objective:To study the relationship and the role of leptin in children with biliary atresia and hepatic fibrosis to provide a treatment basis for these patients.Methods:The clinical data of children with biliary atresia or congenital biliary dilatation (CBD) who underwent surgical treatment at the Department of General Surgery of Tianjin Children's Hospital from August 2019 to August 2021 were retrospectively analyzed. Of 31 children included in this study, there were 14 males and 17 females, with age of 60 (30, 63) d. Children with biliary atresia served as the study group ( n=26) and children with CBD served as the control group ( n=5). Leptin protein, α-smooth muscleactin (α-SMA) and phosphorylation of extracellular-regulated protein kinase 1/2 (p-ERK1/2) in liver tissues were detectd by immunohistochemistry (IHC). The expression level of leptin mRNA in liver tissues were detected by quantitative real-time reverse transcription polymerase chain reaction (qRT-PCR). Results:The average optical density values of leptin protein, α-SMA protein and p-ERK1/2 protein in the liver tissues of children in the study group were significantly higher than the control group ( P<0.05). The expression levels of leptin, α-SMA and p-ERK1/2 in liver tissues of children with biliary atresia significantly increased with increase in fibrosis degree ( P<0.05). The expression level of leptin in liver tissues of children with biliary atresia was positively correlated with the liver fibrosis grade ( rs=0.876), α-SMA ( r=0.723) and p-ERK1/2 ( r=0.725) ( P<0.01). The results of qRT-PCR showed that the content of leptin mRNA in liver tissues of children with biliary atresia was significantly higher than that of children with CBD ( P<0.05). Conclusion:Expressions of leptin increased with aggravation of degrees of hepatic fibrosis in biliary atresia. Leptin may be involved in activation of HSCs through the ERK1/2 signaling pathway in the process of hepatic fibrosis due to biliary atresia.

The role of nuclear transcription factor Gli in the regulation of hepatic epithelial mesenchymal transition in biliary atresia mice caused by Rhesus monkey rotavirus infection / 中华实用儿科临床杂志

Objective:To investigate the role of nuclear transcription factor Gli1/Gli2 of the sonic hedgehog (Shh) signaling pathway in the hepatic epithelial mesenchymal transition (EMT) of biliary atresia mice caused by Rhesus rotavirus (RRV) infection.Methods:The biliary atresia model in mice was generated by RRV infection.Mice were divided into normal group, model group, Gli1 overexpression group, Gli1 shRNA group, Gli2 overexpression group and Gli2 shRNA group.Real-time fluorescence quantitative polymerase chain reaction and Western blot were used to detect the mRNA and protein expressions of regulatory factors for EMT (Snail/Slug) and characteristic cytokines of EMT [Vimentin, α-smooth muscle actin(α-SMA), E-cadherin] in mouse liver tissues.Additionally, hematoxylin-eosin staining and Masson staining were performed to calculate the percentage of liver fibrous tissue expression area.The data were analyzed by One- Way ANOVA and LSD- t test. Results:The relative mRNA expression of Snail, Slug, Vimentin, α-SMA and E-cadherin in Gli2 overexpression group, Gli2 shRNA group and model group were 15.13±3.40, 5.48±0.46, 8.78±1.06, 12.40±2.18 and 3.06±0.53; 3.73±1.16, 5.62±1.75, 3.56±1.06, 3.88±1.16 and 10.51±1.83; 8.13±1.27, 5.32±0.98, 5.05±0.98, 4.02±0.77 and 5.12±1.60.Compared with those of the model group, mRNA levels of Snail, Vimentin and α-SMA were significantly higher in Gli2 overexpression group, while that of E-cadherin was significantly lower( t=4.53, 5.29, 8.12, -2.13; all P<0.05); compared with those of the model group, mRNA levels of Snail and Vimentin in Gli2 shRNA group significantly decreased, while that of E-cadherin significantly increased( t=-2.86, -2.12, 5.62; all P<0.05). In Gli2 overexpression group, Gli2 shRNA group and model group, the protein levels of Snail, Slug, Vimentin, α-SMA and E-cadherin were 2.02±0.39, 0.31±0.08, 0.95±0.17, 1.07±0.17 and 0.42±0.06; 0.53±0.13, 0.40±0.18, 0.20±0.04, 0.28±0.07 and 1.09±0.31; 0.70±0.15, 0.42±0.22, 0.64±0.13, 0.81±0.11 and 0.42±0.09.Compared with those of the model group, protein levels of Snail, Vimentin and α-SMA were significantly higher in Gli2 overexpression group( t=12.71, 4.28, 3.70; all P<0.05); compared with those of the model group, protein levels of Vimentin and α-SMA in Gli2 shRNA group significantly decreased, while that of E-cadherin significantly increased( t=-6.14, -7.57, 5.96; all P<0.05). However, no significant change trend were detected in expression levels of characteristic cytokines of EMT between Gli1 overexpression group and Gli1 shRNA group.The area percentage of liver fiber expression in normal group, model group, Gli1 overexpression group, Gli1 shRNA group, Gli2 overexpression group and Gli2 shRNA group were (1.03±0.58)%, (33.02±11.39)%, (39.81±5.67)%, (26.06±1.29)%, (49.81±8.57)% and (17.55±0.66)%, respectively.Besides, in terms of percentage of area expressed in liver fiber tissue, the Gli2 overexpression group and Gli2 shRNA group were statistically significant compared with the model group( t=3.21, -2.96; all P<0.05), while the Gli1 overexpression group and Gli1 shRNA group were not statistically significant compared with the model group (all P>0.05). Conclusions:The Shh signaling pathway plays an important role in liver fibrosis in mice with biliary atresia.Gli2, a key transcription factor of Shh signaling pathway, can significantly regulate liver EMT process in mice with biliary atresi.

Expression level of vitamin D receptor in intrahepatic bile duct epithelial cells and its clinical significance in children with biliary atresia / 中华实用儿科临床杂志

Objective:To explore the possible role and clinical significance of vitamin D receptor (VDR) in intrahepatic bile duct epithelial cells (IBDECs) in biliary atresia (BA).Methods:A retrospective analysis was performed on expression level of VDR in IBDECs of 38 BA children who underwent Kasai surgery in the Second Affiliated Hospital of Xi′an Jiaotong University and the Children′s Hospital of Xi′an Jiaotong University from January 2015 to December 2019.Expression level of VDR in IBDECs of 38 children with BA was detected by immunohistochemical staining, and that in children with choledochal cysts was detected as negative control.Masson staining was performed to examine the degree of liver fibrosis.The correlation between the expression level of VDR in IBDECs of children with BA, and the degree of liver fibrosis during operation, the incidence of refractory cholangitis after Kasai portoenterostomy and the survival time of autologous liver was analyzed.Human intrahepatic bile duct epithelial cells (HiBECs) were induced with dsRNA virus infection by polyinosinic acid-polycytidylic acid [Poly(I∶C)] in vitro, followed by detection of cell activity, apoptosis and VDR level.The differences between 2 independent groups were analyzed using Student t test.The relationship between the expression of VDR and clinicopathologic characteristics was conducted with χ2 test or Fisher′ s test.The Kaplan- Meier survival curve was used to analyze the differences in the survival time of autologous liver after Kasai in BA children with different VDR expression levels. Results:A total of 38 children with BA were included in this study.Among them, 23 cases showed no significant decrease of VDR protein level in IBDECs, and 15 cases showed a significant decrease in IBDECs.Compared with BA children without a significant decrease in VDR level in IBDECs, much severer liver fibrosis ( P<0.001) and significantly higher incidence of refractory cholangitis after Kasai procedure ( P=0.017) were detected in those with a significant decrease in VDR level.Compared with the control group, BA children with significantly lower VDR expression levels in HiBECs had a shorter autologous liver survival time ( P=0.030). Poly (I∶C) increased the apoptotic rate of HiBECs ( P<0.000 1) and decreased cell activity of HiBECs ( P<0.05), which significantly stimulated the secretion of inflammatory factors (interferon, tumor necrosis factor-α, interleukin-6) in the culture medium of HiBECs ( P<0.001). Poly (I∶C) significantly decreased the expression level of VDR protein in HiBECs ( P<0.001). Conclusions:Poly (I∶C) causes HiBECs damage and decreases VDR expression level in HiBECs of BA children, and the significantly decreased VDR expression level in IBDECs may be a marker of poor prognosis of BA.