Case report on failed Kasai-portoenterostomy for extrahepatic biliary atresia with neonatal cholestasis in one-month-old infant

Biliary atresia is a neonatal onset, obstructive cholangiopathy of the intrahepatic or extrahepatic biliary system, leading to the build-up of bile in the liver. This case discusses a one-month-old infant who was previously diagnosed with jaundice, presenting the symptoms of yellowish eyes and skin, pale stools, and palpable liver. The infant and her mother抯 liver enzyme levels were found to be higher and her histopathology studies evidenced atretic gall bladder. The liver biopsy revealed mild periportal ductular reaction and diffuse hepatocanalicular cholestasis. She was diagnosed with neonatal cholestasis- extrahepatic biliary atresia (EHBA) and underwent Kasai-portoenterostomy. She was stable and discharged with gallstone dissolution agents, antibiotics, vitamin supplements, and a barbiturates-liver enzyme inducer. After six months, she presented hepatosplenomegaly with ascites and was found to have transaminitis. She was then diagnosed with EHBA- failed Kasai, probable cholangitis, and planned for living donor liver transplantation. Antibiotics, antiviral, antifungal, anticoagulants, and immunosuppressants were prescribed on discharge. The Doppler study of allograft was performed to check the blood flow after transplantation. Acute graft rejection was monitored on day 5 with liver transplant pack reports. One year later, the infant's condition had shown improvement, evidenced by enhanced food intake, absence of symptoms, and the return of liver function tests to normal levels.

Lesiones de la vía biliar

La finalidad de esta revisión es mostrar el estado actual de las lesiones de vía biliar (LVB), producidas especialmente durante la colecistectomía laparoscópica. Revisaremos la etiopatogenia, los grados de compromiso biliar, las lesiones vasculares asociadas, las clasificaciones utilizadas y los factores de riesgo. Además la forma como evaluar oportunamente la magnitud de la lesión, tanto en la misma cirugía como en el postoperatorio. Igualmente se muestran las diversas alternativas terapéuticas empleadas. Todo con la finalidad de evitar graves complicaciones.

The purpose of this review is to show the current status of bile duct injury, specially produced during laparoscopic cholecystectomy. We will review etiopathogenesis, degrees of bile commitment, associated vascular lesions, classifications used and risk factors. In addition, how to assess the extent of the injury in a timely manner, both in the same surgery and in the postoperative surgery. The various therapeutic alternatives used are also shown. All in order to avoid serious complications.

Mejoría de la calidad de vida después de la reparación definitiva de la lesión de la vía biliar, evaluada con el cuestionario SF-36 / Improvement on quality of life after bile duct injury repair using the SF-36 Health Survey

RESUMEN Antecedentes: diferentes publicaciones han estudiado la fisiopatología de la lesión de la vía biliar (LVB), pero pocas han investigado los efectos psicosociales y de calidad de vida relacionados con su reparación. Objetivo: comparar la calidad de vida de pacientes antes y después de la reparación definitiva de una LVB. Material y métodos: se usó como instrumento para evaluar la calidad de vida relacionada con la salud (CVRS) el cuestionario de salud SF-36 en una serie de pacientes operados entre diciembre de 2015 y junio de 2019 para la reparación de LVB. Resultados: sobre 48 casos de reparaciones de LVB, contestaron la encuesta 22 (46%). Se compararon los ítems del formulario SF 36: diferencia en la función física, el rol físico, el dolor corporal, la salud general, la vitalidad, la función social, el rol emocional, la salud mental y el ítem de transición de salud, y todos mostraron una mejoría estadísticamente significativa (p < 0,001) después de la reparación. En el análisis univariado se observó que la salud mental se vio afectada por la presencia de una lesión compleja (p = 0,019), el rol físico y el rol emocional antes de la reparación, y mostró relación con el tipo de lesión según Strasberg (p = 0,001 y p = 0,032). Aquellos que asociaron lesión vascular presentaron una asociación negativa con la función física (p = 0,019), la vitalidad (p = 0,033), la salud mental (p = 0,005) y el dolor (p = 0,026) antes de la reparación. Conclusión: la resolución definitiva de la LVB en un centro especializado en patología hepatobiliopancreática mostró producir una significativa mejoría en la calidad de vida.

ABSTRACT Background: Different publications have examined the pathophysiology of bile duct injury (BDI), bur few studies have investigated the effects of BDIs and their subsequent repair on psychosocial and health-related quality of life Objective: The aim of this study was to compare the quality of life of patients before and after definitive BDI repair. Material and methods: The SF-36 Health Survey was used as an instrument to assess health-related quality of life (HRQL) in a series of patients operated on between December 2015 and June 2019 for BDI repair. Results: Of 48 patients who underwent BDI repair, 22 (46%) responded to the survey. The SF-36 items, which includes different domains (physical functioning, physical role functioning, bodily pain, general health, vitality, social functioning, emotional role functioning, mental health, and health transition) were compared. All domains demonstrated statistically significant improvement (p < 0.001) following repair. On univariate analysis, mental health was affected by the presence of a complex injury (p = 0.019), and physical and emotional role functioning before BDI repair were associated with the type of injury of the Strasberg classification (p = 0.001 and p = 0.032, respectively). An associated vascular injury had a negative correlation with physical functioning (p = 0.019), vitality (p = 0.033), mental health (p = 0.005), and pain (p = 0.026) prior to repair. Conclusion: The definitive resolution of BDIs at a center specialized in hepatobiliary and pancreatic surgery resulted in a significant improvement of patients' quality of life.

Todani Type I Common Bile Duct Cyst in an Adult Patient: Clinical Case and Literature Review / Quiste de colédoco Todani I en una paciente adulta: caso clínico y revisión de la literatura

Abstract Bile duct cysts are characterized by intrahepatic or extrahepatic bile duct dilation. It is a rare congenital pathology, diagnosed mainly in children. The clinical manifestation in adults is usually nonspecific but essential due to their increased risk of developing carcinoma. We present the case of a 37-year-old female patient who consulted for pain in the epigastrium radiating to the right hypochondrium, associated with emesis and choluria. The hepatobiliary ultrasound was normal, but due to the risk of choledocholithiasis, a magnetic resonance cholangiopancreatography was performed, revealing a cystic dilation of the proximal common bile duct of approximately 2 cm, classified as Todani type I. Bile duct cysts have been associated with several complications, and cholangiocarcinoma is the most important. The probability of malignancy is higher in adults and Todani type I cysts.

Resumen Los quistes de vía biliar se caracterizan por la dilatación de los conductos biliares intrahepáticos o extrahepáticos. Es una patología congénita poco frecuente, diagnosticada principalmente en niños. La presentación clínica en adultos suele ser inespecífica, pero importante debido a su riesgo aumentado de desarrollar carcinoma. Se presenta el caso de una paciente de 37 años que consulta por dolor en epigastrio irradiado al hipocondrio derecho, asociado a emesis y coluria. La ecografía hepatobiliar resultó normal, pero debido al riesgo de coledocolitiasis se realizó una colangiopancreatografía por resonancia magnética en la que se evidenció una dilatación quística del colédoco proximal de aproximadamente 2 cm, clasificada como Todani tipo I. Los quistes de vía biliar se han asociado a varias complicaciones, y el colangiocarcinoma es la más importante. La probabilidad de malignización es mayor en adultos y en los quistes Todani tipo I.

Duplicación del colédoco asociado a unión biliopancreática anómala: Reporte de caso / Common bile duct duplication associated with anomalous biliopancreatic junction: A case report

Introducción. La duplicación del colédoco es una anomalía congénita poco frecuente. En la mayoría de los casos este defecto se asocia a cálculos en la vía biliar, unión pancreatobiliar anómala, pancreatitis, cáncer gástrico o colangiocarcinoma. Por esta razón, el diagnóstico y el tratamiento temprano son importantes para evitar las complicaciones descritas a futuro. Métodos. Se presenta el caso de una paciente de 30 años, con antecedente de pancreatitis aguda, con cuadro de dolor abdominal crónico, a quien se le realizaron varios estudios imagenológicos sin claro diagnóstico. Fue llevada a manejo quirúrgico en donde se documentó duplicación del colédoco tipo II con unión pancreatobiliar anómala. Resultados. Se hizo reconstrucción de las vías biliares y hepatico-yeyunostomía, con adecuada evolución postoperatoria y reporte final de patología sin evidencia de tumor. Conclusión. El diagnóstico se hace mediante ecografía endoscópica biliopancreática, colangiorresonancia o colangiopancreatografía retrógrada endoscópica. El tratamiento depende de si está asociado o no a la presencia de unión biliopancreática anómala o cáncer. Si el paciente no presenta patología neoplásica, el tratamiento quirúrgico recomendado es la resección del conducto con reconstrucción de las vías biliares.

Introduction. Double common bile duct is an extremely rare congenital anomaly. This anomaly may be associated with bile duct stones, anomalous biliopancreatic junction, pancreatitis, bile duct cancer, or gastric cancers. Thus, early diagnosis and treatment is important to avoid complications. Clinical case. We report a rare case of double common bile duct associated with an anomalous biliopancreatic junction in a 30-year-old female, with prior history of acute pancreatitis, who presented with chronic abdominal pain. She underwent several imaging studies, without clear diagnosis. She was taken to surgical management where duplication of the type II common bile duct was documented with anomalous pancreatobiliary junction. Results. Reconstruction of the bile ducts and hepatico-jejunostomy were performed, with adequate postoperative evolution and final pathology report without evidence of tumor. Conclusion. Diagnosis is usually performed by an endoscopic ultrasound, magnetic resonance cholangiopancrea-tography, or endoscopic retrograde cholangiopancreatography. Treatment depends on the presence of anomalus biliopancreatic junction or concomitant cancer. In cases without associated malignancy, resection of bile duct and biliary reconstruction is the recommended surgical treatment.

Colangiocarcinoma tubulopapilar, una entidad emergente poco frecuente, a propósito de un caso

Objetivo: realizar una revisión bibliográfica y presentación de caso de colangiocarcinoma tubulopapilar. Material y Método: Se revisó la ficha médica y las características clínicas, radiológicas y patológicas del tumor, y la literatura científica respecto al carcinoma tubulopapilar. Caso Clínico: Paciente con ictericia progresiva asociado a baja de peso. El estudio imagenológico muestra amputación del tercio distal del colédoco por tejido de partes blandas, sugerente de colangiocarcinoma. Se realiza endosonografía, arrojando "fragmentos superficiales de adenocarcinoma tubulopapilar". Se realiza duodenopancreatectomía cefálica y, posteriormente, se inicia quimioterapia. Discusión: El colangiocarcinoma es una neoplasia de la vía biliar. Existen diferentes variantes histológicas, entre ellas el colangiocarcinoma tubulopapilar. Su diagnóstico se basa en estudios imagenológicos y anatomopatológicos. El principal hallazgo imagenológico va a depender del patrón de crecimiento tumoral; masiforme, periductal o intraductal. Dentro de los intraductales, se describe un carcinoma biliar con crecimiento tubulopapilar, con fenotipo pancreatobiliar epitelial. En los últimos años han sido de interés por tener mejor pronóstico. Conclusión: El colangiocarcinoma tubulopapilar es una variante histológica poco frecuente del colangiocarcinoma, que se asocia a un mejor pronóstico que otras variantes.

Objective: To conduct a literature review and present a case study of tubulopapillary cholangiocarcinoma. Material and Method: The clinical record and the clinical, radiological, and pathological characteristics of the tumor were reviewed, along with the scientific literature regarding tubulopapillary carcinoma. Case Report: Patient with progressive jaundice associated with weight loss. Imaging studies show amputation of the distal third of the common bile duct by soft tissue, suggestive of cholangiocarcinoma. Endosonography was performed, yielding "superficial fragments of tubulopapillary adenocarcinoma." Subsequently, a cephalic duodenopancreatectomy is performed, followed by chemotherapy. Discussion: Cholangiocarcinoma is a neoplasm of the biliary tract. There are different histological variants, including tubulopapillary cholangiocarcinoma. Its diagnosis is based on imaging and pathological studies. The main imaging finding will depend on the pattern of tumor growth: mass-forming, periductal, or intraductal. Among the intraductal types, a biliary carcinoma with tubulopapillary growth and an epithelial pancreatobiliary phenotype has been described. In recent years, they have been of interest due to their better prognosis. Conclusion: Tubulopapillary cholangiocarcinoma is a rare histological variant of cholangiocarcinoma that is associated with a better prognosis than other variants.

Íleo biliar como causa de obstrucción intestinal en una paciente de 38 años con antecedentes de tres embarazos y cesárea: reporte de caso / Gallstone ileus-induced intestinal obstruction in a 38-year-old patient with a history of three pregnancies and cesarean section: a case report

RESUMEN La obstrucción intestinal inducida por íleo biliar es una condición rara pero significativa que se produce cuando una piedra en la vesícula se aloja en el tracto gastrointestinal. Entre los principales factores de riesgo para la obstrucción intestinal inducida por íleo biliar se encuentran la edad avanzada, el sexo femenino, la diabetes, los antecedentes de enfermedad biliar como cálculos biliares, así como procedimientos quirúrgicos previos relacionados con la vesícula biliar, como la colecistectomía. También el embarazo es un factor de riesgo conocido para la enfermedad de los cálculos biliares. En este artículo, se presenta el caso de una paciente de 38 años con antecedentes de tres cesáreas previas y sin comorbilidades aparentes. Antes de acudir al departamento de emergencias, la paciente experimentó dolor abdominal con cólico moderado en la parte inferior del abdomen durante dos días. El examen físico reveló sensibilidad abdominal difusa, predominantemente en la fosa ilíaca derecha, junto con signos de irritación peritoneal. Los análisis de laboratorio mostraron un recuento de leucocitos de 11 490 células/μl y una neutrofilia del 85,6 %. Después de una laparotomía exploratoria, se confirmó el diagnóstico sospechado de íleo biliar por cálculo. Se realizó una enterolitotomía y la paciente mostró un progreso posoperatorio favorable. En conclusión, es importante considerar la obstrucción intestinal inducida por íleo biliar en pacientes con síntomas relevantes y antecedentes médicos. Este reporte de caso subraya la importancia de considerar la obstrucción intestinal inducida por íleo biliar en pacientes con síntomas gastrointestinales y antecedentes de embarazo. El diagnóstico temprano y la intervención son cruciales para prevenir complicaciones graves.

ABSTRACT Gallstone ileus-induced intestinal obstruction is a rare but significant condition that occurs when a gallstone becomes lodged in the gastrointestinal tract. Major risk factors for gallstone ileus-induced intestinal obstruction include older age, female sex, diabetes, history of gallbladder disease such as gallstones, as well as previous gallbladder-related surgical procedures such as cholecystectomy. Pregnancy is also a known risk factor for gallstone disease. We present the case of a 38-year-old patient with a history of three cesarean sections and no apparent comorbidities. Prior to her visit to the emergency department, the patient experienced moderate colicky abdominal pain in the lower abdomen for two days. The physical examination revealed diffuse abdominal tenderness, predominantly in the right iliac fossa, along with signs of irritation of the peritoneum. Laboratory tests showed a leukocyte count of 11,490 cells/µl and neutrophilia of 85.6 %. Following an exploratory laparotomy, the suspected diagnosis of gallstone ileus was confirmed. An enterolithotomy was performed, and the patient experienced good post-surgical progress. In conclusion, it is important to consider gallstone ileus-induced intestinal obstruction among patients with relevant symptoms and medical history. This case report highlights the importance of considering gallstone ileus-induced intestinal obstruction among patients with gastrointestinal symptoms and a history of pregnancy. Early diagnosis and intervention are crucial to prevent serious complications.

Research progress on anatomic vascular reconstruction in rat models of orthotopic liver transplantation / 器官移植

Establishment of rat models of liver transplantation provides an ideal animal model for resolving the problems of postoperative complications and perioperative treatment of liver transplantation. With in-depth study of the establishment of rat models of liver transplantation, classic "two-cuff" technique has been gradually employed. However, poor surgical field, vascular torsion, biliary tract injury and long anhepatic phase remain unresolved in the process of liver transplantation using traditional techniques. At present, the rat models of liver transplantation at home and abroad are modified mainly from the reconstruction of four vital anatomic structures including the suprahepatic inferior vena cava, portal vein, infrahepatic inferior vena cava and bile duct. Therefore, the latest progress in the reconstruction of the suprahepatic inferior vena cava, portal vein, infrahepatic inferior vena cava and bile duct was reviewed, aiming to provide reference for the establishment of rat models of liver transplantation and promote further development of liver transplantation techniques.

Anatomic classification and reconstruction of right intrahepatic bile duct in the donor liver of split liver trans-plantation / 中华消化外科杂志

Objective:To investigate the anatomic classification and reconstruction of right intrahepatic bile duct in the donor liver of split liver transplantation (SLT).Methods:The retrospective and descriptive study was constructed. The clinical data of 85 patients who underwent SLT in the Third Affiliated Hospital of Sun Yat-sen University from July 2014 to January 2022 were collected. There were 65 males and 20 females, aged 45(range, 1-82)years. Observation indicators: (1) surgical conditions; (2) anatomy of right intrahepatic bile duct; (3) bile duct reconstruction; (4) postoperative biliary complications; (5) follow-up. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range) or M( Q1, Q3).Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test or Fisher exact probability. Results:(1) Surgical conditions. Of the 85 donor livers, 11 donor livers were split between the left and right hemilivers, and 74 donor livers were split between the classic right trilobe and left lateral lobe. The cold ischemia time of 85 donor livers was 291(273, 354)minutes, and the operation time, anhepatic phase time and volume of intraoperative blood transfusion of 85 recipients were (497±97)minutes, 51(40, 80)minutes and 8(7, 12)U. (2) Anatomy of right intrahepatic bile duct. Of the 85 donor livers, there were 47 donor livers with classic bile duct anatomical model (type 1), of the ratio as 55.3%(47/85), and 38 donor livers with anatomical variants, of the ratio as 44.7%(38/85). Of the 38 donor livers with anatomical variants, 7 donor livers were type 2, 16 donor livers were type 3a, 2 donor livers were type 3b, 2 donor livers were type 3c, 1 donor liver was type 4, 3 donor livers were type 5a, 4 donor livers were type 5b, 3 donor livers were type 6. For bile duct splitting patterns of the 85 donor livers, 84 donor livers were split with the main trunk of common hepatic duct preserving in the right hemiliver or right trilobe, and 1 donor liver were treated with complete left and right hemiliver splitting to preserve the main trunk of the common hepatic duct in the left hemiliver and the right hemiliver in the right hepatic duct (type 1 bile duct anatomical model). There were 84 donor livers with only one bile duct opening, and 1 donor liver with two bile duct openings (type 3c bile duct anatomical model). (3) Bile duct reconstruction. Of the 85 recipients, there were 69 recipients with common bile duct end-to-end anastomosis to common bile duct of donor liver (38 donor livers with type 1 bile duct anatomical model, 5 donor livers with type 2 bile duct anatomical model, 14 donor livers with type 3a bile duct anatomical model, 2 donor livers with type 3b bile duct anatomical model, 1 donor liver with type 4 bile duct anatomical model, 3 donor livers with type 5a bile duct anatomical model, 4 donor livers with type 5b bile duct anatomical model, 2 donor livers with type 6 bile duct anatomical model), 11 recipients with jejunum anastomosis to common bile duct of donor liver (7 donor livers with type 1 bile duct anatomical model, 2 donor livers with type 2 bile duct anatomical model, 1 donor liver with type 3c bile duct anatomical model, 1 donor liver with type 6 bile duct anatomical model), 3 recipients with jejunum anastomosis to common hepatic duct of donor liver (1 donor liver with type 1 bile duct anatomical model, 2 donor livers with type 3a bile duct anatomical model), 1 recipient with jejunum anastomosis to right hepatic duct of donor liver (type 1 bile duct anatomical model), 1 recipient with common hepatic duct end-to-end anastomosis to right posterior branch of donor liver combined with jejunum of the recipient Roux-en-y anastomosis to common hepatic duct of donor liver (type 3c bile duct anatomical model). (4) Postoperative biliary complications. Of the 85 recipients, 6 cases had postoperative biliary complications, with an incidence of 7.1% (6/85). Of the 6 recipients with postoperative biliary complications, there were 5 recipients with donor liver with type 1 bile duct anatomical model, including 3 cases undergoing postoperative biliary stricture with biliary leakage and 2 cases undergoing postoperative biliary anastomotic stricture, 1 recipient with donor liver with type 3b bile duct anatomical model and undergoing postoperative biliary anastomotic stricture and bile leakage in the liver section. Cases with biliary complications were 5 in the 47 recipients with donor liver with classic bile duct anatomical model and 1 in the 38 recipients with donor liver with anato-mical variants, showing no significant difference between them ( P>0.05). (5) Follow-up. There were 83 recipients receiving followed up for 52(12,96)months. During the follow-up period, 2 recipients died due to non-biliary complication factors (1 donor liver with type 1 bile duct anatomical model and 1 donor liver with 3a bile duct anatomical model). Conclusion:The anatomical classification of right intrahepatic bile duct of donor liver in SLT is mainly classical bile duct anatomical model, and the bile duct reconstruction scheme is mainly common bile duct of donor liver end-to-end anasto-mosis to common bile duct of recipient.

Prognosis of congenital bile duct cysts after cyst resection and risk factors analysis of postoperative biliary calculus / 中华肝胆外科杂志

Objective:To study the prognosis of congenital bile duct cysts following cyst resection, and to analyze the risk factors associated with the development of postoperative biliary calculus.Methods:Clinical data of 149 patients with congenital bile duct cysts undergoing surgery in the First Affiliated Hospital of Nanjing Medical University from May 2004 to January 2022 were retrospectively analyzed, including 59 males and 90 females, with a median age of 32 (21, 47) years old. Patients were divided into two groups: the stone group ( n=51, biliary calculus occurred during the follow-ups after surgery) and non-stone group ( n=98). Clinical data such as gender, age, medical history, cyst type, biliary calculus, anastomotic stenosis and occurrence of cancer were compared. All patients were followed up via telephone consultations. A logistic regression analysis was used to identify the risk factors associated with the occurrence of biliary calculus after surgery. Results:The duration of the follow-ups was 120 (24, 211) months. The observed incidence of postoperative biliary calculus, anastomotic stricture, and cancer in the patients were 34.2% (51/149), 8.7% (13/149), and 4.7% (7/149), respectively. The logistic regression analysis indicated that incomplete cyst resection ( OR=3.332, 95% CI: 1.221-9.094) and postoperative anastomotic stenosis ( OR=13.300, 95% CI: 2.586-68.401) were associated with a higher risk of biliary calculus formation after cystectomy (all P<0.05). Conclusion:Patients with congenital bile duct cysts suffer a high risk of biliary calculus formation after cystectomy. The residual cyst and postoperative anastomotic stenosis are independent risk factors for biliary calculus after surgery.

Synchronous laparoscopic cholecystectomy combined with laparoscopic common bile duct exploration or combined with endoscopic sphincterotomy for gallstones with common bile duct stones / 中华普通外科杂志

Objective:To compare the recurrence of common bile duct stones (CBDS) in patients with gallstones and concurrent CBDS treated by two surgical approaches: synchronous laparoscopic cholecystectomy (LC) combined with laparoscopic common bile duct exploration (LCBDE) (LC+LCBDE) and synchronous LC combined with intraoperative endoscopic sphincterotomy (IO-EST) (LC+IO-EST).Methods:From Apr 2013 to Apr 2020, the clinical data of 903 patients with gallstones with CBDS who were admitted to the Clinical Medical College, Yangzhou University were retrospectively analyzed.Results:Based on the chosen surgical method, we categorized 389 cases into group A (LC+LCBDE) and 514 cases into group B (LC+IO-EST). Our findings revealed that group A had a significantly lower rate of CBDS recurrence and re-recurrent CBDS compared to group B (4.4% vs. 8.4%, P=0.024; 0.8% vs. 3.1%, P=0.010). Moreover, Logistic regression analysis after inverse probability of treatment weighting, revealed that the surgical approach implemented in group A was an independent protective factor of recurrent CBDS and second recurrence of CBDS ( OR=0.482, 95% CI: 0.365-0.637, P<0.001; OR=0.118, 95% CI:0.080-0.173, P<0.001). Conclusion:LC+LCBDE is an optimal treatment option to LC+IO-EST for patients with gallstones combined with CBDS and common bile duct ≥8 mm.

Association of TP53 germline variant and choledochal cyst among clinically diagnosed Filipino pediatric patients

@#Background and Objective. Choledochal cysts (CC) are rare congenital, cystic dilations of the biliary tree occurring predominantly in Asian populations and in females. Patients are usually children presenting with any of the following: abdominal pain, palpable abdominal mass, and jaundice. Its congenital nature hints at a potential genetic cause. A possible causal gene is TP53, a tumor suppressor with a germline variant called rs201753350 (c.91G>A) that changed from a G allele to an A allele, decreasing the cell proliferation suppressing activity of its functional protein. Currently, there is no information on the TP53 rs201753350 germline variant available for the Filipino population. This study determined the prevalence of rs201753350 and the association between the functional G allele, the rs201753350 germline variant A allele, and the occurrence of CCs in Filipino pediatric patients in a tertiary government hospital. Methods. Genomic DNA was extracted from blood samples of pediatric patients clinically diagnosed with CC. Controls were DNA samples collected from a previous study. The samples underwent PCR, electrophoresis, and sequencing. Results. A total of 109 participants (22 cases and 87 controls) were included in the study. The A allele (22.94%) occurs at a lower frequency than the G allele (77.06%) among both cases and controls. More individuals have a homozygous G/G genotype (54.13%) than a heterozygous A/G genotype (45.87%) while the homozygous A/A genotype was not observed. The estimated risk of choledochal cyst occurrence is significantly lower in individuals with the A allele (PR: 0.08, 95% CI: 0.01 – 0.55) and the A/G genotype (PR: 0.06, 95% CI: 0.01 – 0.40). Conclusion. There is no significant evidence to suggest an association between the TP53 rs201753350 germline variant and the occurrence of choledochal cysts in Filipinos. It is recommended that other mutations within and beyond the TP53 gene be investigated for possible associations with choledochal cyst occurrence.

Clinical significance of determining the level of biliary calprotectin in patients with cholangiocarcinoma or choledocholithiasis / 临床肝胆病杂志

ObjectiveTo investigate the difference in the level of biliary calprotectin between patients with cholangiocarcinoma and those with choledocholithiasis. MethodsClinical data and bile samples were collected from 34 patients with cholangiocarcinoma and 78 patients with choledocholithiasis who were diagnosed and treated with endoscopic retrograde cholangiopancreatography in The First Affiliated Hospital of Anhui Medical University from May 2021 to September 2022. Fluorescence lateral flow immunoassay was used to measure the levels of calprotectin， hemoglobin， and lactoferrin in bile. The Mann-Whitney U test was used for comparison of continuous data between two groups， and the chi-square test was used for comparison of categorical data between two groups； the Spearman correlation test was used for correlation analysis； the DeLong test was used for comparison of the area under the ROC curve （AUC）. ResultsCompared with the choledocholithiasis group， the cholangiocarcinoma group had significant increases in the levels of calprotectin ［4 795.50 （2 286.79‍ ‍—‍ ‍20 179.73） ng/mL vs 411.16 （67.03‍ ‍—‍ ‍1 991.88） ng/mL， Z=5.572， P<0.001］ and fluoride ［115.70 （109.10‍ — ‍125.50） mmol/L vs 106.60 （98.60‍ ‍—‍ ‍114.40） mmol/L， Z=2.702， P=0.007］. The patients with cholangiocarcinoma were further divided into high cholangiocarcinoma group and low cholangiocarcinoma group， and there was no significant difference between the two groups in the level of calprotectin ［3 867.71 （2 235.66‍ — ‍26 407.40） ng/mL vs 4 795.50 （2 361.15‍ — ‍13 070.53） ng/mL， Z=0.129， P>0.05］. Biliary calprotectin level was correlated with white blood cell count， hemoglobin concentration， and lactoferrin concentration in bile （r=0.316， 0.353， and 0.464， all P<0.05）. The ROC curve analysis showed that biliary calprotectin （with a sensitivity of 79.4% and a specificity of 75.6%）， blood CA19-9 （with a sensitivity of 82.4% and a specificity of 78.2%）， and their combination （with a sensitivity of 88.2% and a specificity of 73.1%） had good sensitivity and specificity in the diagnosis of cholangiocarcinoma. ConclusionThere is an increase in the level of biliary calprotectin in patients with cholangiocarcinoma， and therefore， it might become a biomarker for the diagnosis of cholangiocarcinoma.

Myricetin attenuates renal fibrosis by activating Nrf2/HO-1 pathway to inhibit oxidative stress / 药学学报

This paper investigates the effect of myricetin (MYR) on renal fibrosis induced by unilateral ureteral obstruction (UUO) and common bile duct ligation (CBDL) in mice and its mechanism. The animal experiment has been approved by the Ethics Committee of China Pharmaceutical University (NO: 2022-10-020). Thirty-five ICR mice were divided into control, UUO, UUO+MYR, CBDL and CBDL+MYR groups. H&E and Masson staining were used to detect pathological changes in kidney tissues. Western blot (WB) was used to detect the expression of fibrosis-related proteins in renal tissue, and total superoxide dismutase (SOD) activity detection kit (WST-8) was used to detect the changes of total SOD in renal tissue of CBDL mice. In vitro, HK-2 cells and transforming growth factor beta 1 (TGF-β1, 10 ng·mL-1) were used to induce fibrotic model, and high glucose (30 mmol·L-1) was used to induce oxidative stress model, and then treated with different concentrations of MYR, WB was used to detect the expression of fibrosis and oxidative stress-related proteins, while NIH/3T3 cells were treated with different concentrations of MYR, and their effects on cell proliferation were detected by 5-bromo-2′-deoxyuridine (Brdu). The results showed that the renal lesions in UUO group and CBDL group were severe, collagen deposition was obvious, the expression of collagen-Ⅰ (COL-Ⅰ), α-smooth muscle actin (α-SMA), fibronectin (FN), vimentin and plasminogen activator inhibitor-1 (PAI-1) protein was up-regulated, and the activity of SOD enzyme in CBDL group was significantly decreased. MYR partly reversed the above changes after treatment. MYR inhibited the proliferation of NIH/3T3 cells but had no effect on the proliferation of HK-2 cells, and decreased the upregulation of PAI-1, FN and vimentin in HK-2 cells stimulated by TGF-β1. MYR can also up-regulate the down-regulation of nuclear factor erythroid 2-related factor 2 (Nrf2) and heme oxygenase-1 (HO-1) in HK-2 cells stimulated by high glucose. To sum up, MYR can improve renal fibrosis in vivo and in vitro, probably by inhibiting the proliferation of fibroblasts and activating Nrf2/HO-1 signal pathway to inhibit oxidative stress.

The value of CT three-dimensional reconstruction combined with serum carbohydrate antigen 19-9 and carcinoembryonic antigen detection in the diagnosis and resectability evaluation of hilar cholangiocarcinoma / 中国基层医药

Objective:To investigate the value of three-dimensional reconstruction combined with serum carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) detection in the diagnosis and resectability evaluation of hilar cholangiocarcinoma (HCCA) before resectable lymph node metastasis.Methods:A total of 65 patients with suspected HCCA who were treated at Yiwu Central Hospital from June 2019 to June 2022 were included in the observation group. Thirty healthy people who concurrently underwent physical examinations in the same hospital were included in the control group. All participants underwent a CT three-dimensional reconstruction examination. Simultaneously, the automatic electrochemiluminescence immunoassay analyzer was used to measure serum levels of CA19-9 and CEA. The outcomes of percutaneous transhepatic cholangiography were used as the "gold standard". The consistency between CT three-dimensional reconstruction, CA19-9 detection, and CEA detection, either individually or combined, and the "gold standard" in the diagnosis of HCCA was evaluated.Results:Serum levels of CA19-9 and CEA in the observation group were (62.71 ± 10.63) U/mL and (62.71 ± 10.63) ng/mL, respectively, which were significantly higher than those in the control group [(12.37 ± 7.39) U/mL, (1.31 ± 0.97) ng/mL, t = 23.43, 11.59, both P < 0.05). The levels of CA19-9 [(71.69 ± 12.37) U/mL] and CEA [(8.89 ± 3.51) ng/mL] in patients with HCCA who had lymph node metastasis were significantly higher than those in patients with HCCA who had no lymph node metastasis [CA19-9 (56.78 ± 10.16) U/mL, CEA (6.45 ± 2.11) ng/mL, t = 4.14, 2.76, both P < 0.05].Compared with histopathological examination, the accuracy of CT three-dimensional reconstruction in typing was 85.00%. According to the "gold standard" diagnosis, CT three-dimensional reconstruction, CA19-9 detection, and CEA detection, alone and their combination, successfully detected HCCA in 22 cases (55.00%), 26 cases (65.00%), 31 cases (77.50%), and 38 cases (95.00%), respectively. The detection rate of HCCA was the highest when CT three-dimensional reconstruction, CA19-9 detection, and CEA detection were combined, and the difference was statistically significant ( χ2 = 18.15, P < 0.05). Compared with CT three-dimensional reconstruction (AUC: 0.808), CA19-9 detection (AUC: 0.721), and CEA detection (AUC: 0.703) individually, their combination (AUC: 0.913) had the highest value in the diagnosis of HCCA (all P < 0.05). Conclusion:CT three-dimensional reconstruction, CA19-9 detection, and CEA detection have a certain diagnostic value for HCCA, but the combination of CT three-dimensional reconstruction with the detection of serum levels of CA19-9 and CEA has a higher diagnostic value for HCCA, providing an effective reference for the preoperative evaluation of the resectability of HCCA in the clinic.

Bile duct injury repair in a patient with situs inversus totalis / Tratamento de lesão de via biliar em paciente com situs inversus totalis

ABSTRACT BACKGROUND: Bile duct injury (BDI) causes significant sequelae for the patient in terms of morbidity, mortality, and long-term quality of life, and should be managed in centers with expertise. Anatomical variants may contribute to a higher risk of BDI during cholecystectomy. AIMS: To report a case of bile duct injury in a patient with situs inversus totalis. METHODS: A 42-year-old female patient with a previous history of situs inversus totalis and a BDI was initially operated on simultaneously to the lesion ten years ago by a non-specialized surgeon. She was referred to a specialized center due to recurrent episodes of cholangitis and a cholestatic laboratory pattern. Cholangioresonance revealed a severe anastomotic stricture. Due to her young age and recurrent cholangitis, she was submitted to a redo hepaticojejunostomy with the Hepp-Couinaud technique. To the best of our knowledge, this is the first report of BDI repair in a patient with situs inversus totalis. RESULTS: The previous hepaticojejunostomy was undone and remade with the Hepp-Couinaud technique high in the hilar plate with a wide opening in the hepatic confluence of the bile ducts towards the left hepatic duct. The previous Roux limb was maintained. Postoperative recovery was uneventful, the drain was removed on the seventh post-operative day, and the patient is now asymptomatic, with normal bilirubin and canalicular enzymes, and no further episodes of cholestasis or cholangitis. CONCLUSIONS: Anatomical variants may increase the difficulty of both cholecystectomy and BDI repair. BDI repair should be performed in a specialized center by formal hepato-pancreato-biliary surgeons to assure a safe perioperative management and a good long-term outcome.

RESUMO RACIONAL: As lesões de via biliar (LVB) impõem sequelas significativas ao paciente em termos de morbidade, mortalidade e qualidade de vida a longo prazo, devendo ser manejadas em centros especializados. Variantes anatômicas podem contribuir para um maior risco de LVB durante colecistectomia. OBJETIVOS: Relatar paciente com lesão de via biliar associado a situs inversus totalis. MÉTODOS: Paciente do sexo feminino, 42 anos, com histórico prévio de situs inversus totalis e LVB inicialmente reparada simultaneamente à lesão, há 10 anos, por um cirurgião não especializado. Ela foi encaminhada a um centro especializado devido a episódios recorrentes de colangite e um padrão laboratorial colestático. Colangiressonância revelou uma grave estenose anastomótica. Devido à sua idade jovem e colangites recorrentes, foi submetida a uma revisão cirúrgica da hepaticojejunostomia com técnica de Hepp-Couinaud. Até onde sabemos, este é o primeiro relato de reparo de LVB em um paciente com situs inversus totalis. RESULTADOS: A hepaticojejunostomia realizado prèviamente foi desfeita e refeita empregando a técnica de Hepp-Couinaud, alta na placa hilar, com uma ampla abertura na confluência dos ductos biliares em direção ao ducto hepático esquerdo. A alça de roux anterior foi mantida. A recuperação pós-operatória transcorreu sem intercorrências, o dreno foi removido no sétimo dia pós-operatório, e a paciente está agora assintomática, com bilirrubina e enzimas canalículares normais, e sem mais episódios de colestase ou colangite. CONCLUSÕES: Variantes anatômicas podem aumentar a dificuldade tanto da colecistectomia quanto do reparo de LVB, o qual deve ser realizado em um centro especializado por cirurgiões hepatobiliares para garantir um manejo perioperatório seguro e um bom resultado a longo prazo.

Colangitis aguda por Candida glabrata y Klebsiella pneumoniae / Acute cholangitis due to Candida glabrata and Kleb siella pneumoniae

Resumen La colangitis aguda es una infección de la vía biliar, asociada a la obstrucción de esta. El cultivo de la bilis es positivo en la mayoría de los casos y el agente etio lógico más frecuente es Escherichia coli. La colangitis aguda por Candida sp es un hallazgo poco común, que es más frecuente en pacientes con inmunocompromiso, uso de corticoides, tratamiento antibiótico prolongado o procedimientos quirúrgicos de la vía biliar. Presenta mos el caso de una mujer de 67 años, que no presen taba ninguno de los antecedentes mencionados, y que consultó por fiebre, dolor abdominal e ictericia. En la resonancia magnética nuclear de abdomen se constató imagen litiásica en el colédoco con dilatación de la vía biliar. Requirió drenaje endoscópico del tracto biliar. En el examen microscópico directo del líquido biliar se evidenciaron levaduras y bacilos Gram negativos, y en el cultivo se aisló Klebsiella pneumoniae productora de betalactamasa de espectro extendido (BLEE) y Candida glabrata. La paciente completó el tratamiento antibiótico con piperacilina tazobactam y anidulafungina con buena evolución. La infección de la vía biliar por la asociación de bacilos Gram negativos y Candida sp es una entidad poco frecuente, más en pacientes sin enfermedades subyacentes.

Abstract Acute cholangitis is a bile duct infection associated with bile duct obstruction. Bile culture is positive in most cases, and the most frequent etiological agent is Escherichia coli. Candida sp acute cholangitis is a rare finding, which is more common in patients with im munosuppression, use of corticosteroids, prolonged antibiotic treatment or surgical procedures of the bile duct. We present the case of a 67-year-old woman with none of the above-mentioned history who consulted for fever, abdominal pain and jaundice. MRI of the ab domen revealed a lithiasic image in the common bile duct with dilation. It required endoscopic drainage of the biliary tract. Direct microscopic examination of the bile fluid revealed gram-negative bacilli and yeast, and in the culture of bile fluid Klebsiella pneumoniae produc ing extended spectrum beta-lactamase (ESBL) and Can dida glabrata were isolated. The patient completed the antibiotic treatment with piperacillin tazobactam and anidulafungin with good evolution. Bile duct infection by association of Gram-negative bacilli and Candida sp is a rare entity, more in patients without underlying diseases.

Previsores de falha da colangiopancreatografia retrógrada endoscópica na limpeza de cálculos do ducto biliar durante o procedimento inicial - Um estudo prospectivo / Predictors of failure of endoscopic retrograde cholangiopancreatography in clearing bile duct stones during index procedure - a prospective study

ABSTRACT Background: Common bile duct (CBD) stones are known to complicate 10-15% of gallstone diseases. Endoscopic retrograde cholangiopancreatography (ERCP) is the therapeutic modality of choice for bile duct clearance in CBD stones but may fail to achieve stone clearance. This prospective study was done to identify the predictors of failure of CBD clearance with ERCP. Objective: This prospective study was done to identify the predictors of failure of CBD clearance with ERCP. Methods: All consecutive patients with bile duct stones undergoing ERCP at a tertiary care center were prospectively included from October 2020 to October 2021. The study's primary outcome was to identify and analyze factors that could predict the failure of complete CBD clearance. Results: A total of 120 patients (50.8% males, median age: 53.5 years) were included in the final analysis. Successful clearance of CBD stones during the index procedure was achieved in 70% of patients. At a cut-off stone diameter of >10.5 mm and CBD diameter of >12.5 mm, the AUC was 0.890 and 0.884, respectively, to predict failed clearance of CBD. On multivariate analysis, stone diameter ≥15 mm [odds ratio (OR) 16.97, 95% confidence interval (CI): 1.629-176.785], location of stones in hepatic ducts (OR 7.74, 95%CI: 2.041-29.332), presence of stricture distal to stone (OR 6.99, 95%CI: 1.402-34.726) and impacted stone (OR 21.61, 95%CI: 1.84-253.058) were independent predictors of failed bile duct clearance. Conclusion: Stone size and location are independent predictors of failed bile duct clearance. The endoscopist should consider these factors while subjecting a patient to biliary ductal clearance to plan additional intervention.

RESUMO Contexto: Cálculos do ducto biliar comum (CDC) são conhecidos por complicar 10-15% das doenças de cálculos biliares. A colangiopancreatografia retrógrada endoscópica (CPRE) é a modalidade terapêutica de escolha para a limpeza do CDC, mas pode falhar na sua remoção. Objetivo: Este estudo prospectivo foi realizado para identificar os previsores de falha na limpeza do CDC com CPRE. Métodos: Pacientes consecutivos com cálculos no ducto biliar submetidos a CPRE em um centro de atendimento terciário foram incluídos prospectivamente de outubro de 2020 a outubro de 2021. O principal resultado do estudo foi identificar e analisar fatores que poderiam prever a falha na limpeza completa do CDC. Resultados: Um total de 120 pacientes (50,8% homens, idade média: 53,5 anos) foram incluídos na análise final. A limpeza bem-sucedida dos cálculos de CDC durante o procedimento inicial foi alcançada em 70% dos pacientes. Com um diâmetro de corte de cálculos >10,5 mm e de diâmetro de CDC de >12,5 mm, a AUC foi de 0,890 e 0,884, respectivamente, para prever a falha na limpeza do CDC. Na análise multivariada, diâmetro da cálculos ≥15 mm [razão de chances (OR) 16,97, intervalo de confiança de 95% (IC): 1,629-176,785], localização dos cálculos nos ductos hepáticos (OR 7,74, IC95%: 2,041-29,332), presença de estreitamento distal ao cálculo (OR 6,99, IC95%: 1,402-34,726) e cálculo impactado (OR 21,61, IC95%: 1,84-253,058) foram previsores independentes de falha na limpeza do ducto biliar. Conclusão: O tamanho e a localização dos cálculos são previsores independentes de falha na limpeza do ducto biliar. O endoscopista deve considerar esses fatores ao submeter um paciente à limpeza ductal biliar para planejar intervenção adicional.

Shoeshine maneuver for cystic duct dissection: a simple technique to make Calot-triangle dissection smooth

ABSTRACT Purpose: Laparoscopic cholecystectomy, introduced in 1985 by Prof. Dr. Erich Mühe, has become the gold standard for treating chronic symptomatic calculous cholecystopathy and acute cholecystitis, with an estimated 750,000 procedures performed annually in the United States of America. The risk of iatrogenic bile duct injury persists, ranging from 0.2 to 1.3%. Risk factors include male gender, obesity, acute cholecystitis, previous hepatobiliary surgery, and anatomical variations in Calot's triangle. Strategies to mitigate bile duct injury include the Critical View of Safety and fundus-first dissection, along with intraoperative cholangiography and alternative approaches like subtotal cholecystectomy. Methods: This paper introduces the shoeshine technique, a maneuver designed to achieve atraumatic exposure of anatomical structures, local hemostatic control, and ease of infundibulum mobilization. This technique involves the use of a blunt dissection tool and gauze to create traction and enhance visibility in Calot's triangle, particularly beneficial in cases of severe inflammation. Steps include using the critical view of safety and Rouviere's sulcus line for orientation, followed by careful dissection and traction with gauze to maintain stability and reduce the risk of instrument slippage. Results: The technique, routinely used by the authors in over 2000 cases, has shown to enhance patient safety and reduce bile duct injury risks. Conclusion: The shoeshine technique represents a simple and easy way to apply maneuver that can help surgeon during laparoscopic cholecystectomies exposing the hepatocystic area and promote blunt dissection.

Desarrollo de una Unidad de Cirugía Hepatobiliar Compleja y Trasplante Hepático en un hospital público: a una década de su formación / Development of a complex hepatobiliary surgery and liver transplantation unit in a public hospital: a decade after its creation

RESUMEN Antecedentes: concentrar esta patología hepatobiliar en centros especializados puede permitir bajas cifras de morbimortalidad y mejores resultados alejados. Objetivo: describir las características clínico-patológicas y los resultados de una serie de pacientes operados en los primeros 10 años de una Unidad de Cirugía Hepatobiliar Compleja y Trasplante Hepático (TH), así como la actividad científica y docente de sus integrantes. Materiales y métodos: estudio retrospectivo descriptivo. Se recopiló información de una base de datos prospectiva. Se clasificaron los procedimientos según su complejidad y se analizaron los TH, las hepatectomías y los procedimientos biliares complejos, realizados entre 2013 y 2022. Resultados: sobre 1440 procedimientos quirúrgicos, 832 fueron de alta complejidad (58%). Se realizaron 452 trasplantes hepáticos (TH), 14 (3%) de ellos hepatorrenales; la sobrevida global del trasplante fue de 83%, 77% y 75% a 1, 3 y 5 años, respectivamente. La falla primaria del injerto se observó en 5 casos (1,1%). Se efectuaron 254 hepatectomías: 58 (23%) por patología benigna, 155 (61%) por patología maligna y 41 (16%) en donante vivo relacionado para TH. La morbilidad total fue del 27% y la mortalidad intrahospitalaria de 2%. Se efectuaron 106 derivaciones biliodigestivas: 53 en pacientes con lesión quirúrgica de la vía biliar. La morbilidad a 30 días fue del 42%, en su mayoría complicaciones menores (28%). Además, se realizaron 17 duodenopancreatectomías cefálicas y 3 esplenopancreatectomías. Se realizaron múltiples publicaciones, presentaciones y cursos, y desde 2015 se implementó una residencia posbásica de la especialidad. Conclusión: la concentración de patología hepatobiliar en un centro de alta complejidad permitió alcanzar resultados satisfactorios, comparables con series extranjeras.

ABSTRACT Background: Centralizing hepatobiliary disease in specialized centers can reduce morbidity and mortality rates and improve long-term outcomes. Objective: The aim of the present study was to describe the clinical and pathological features and surgical outcomes of a series of patients operated on in the Hepatobiliary Surgery and Liver Transplantation Unit of Hospital El Cruce during its first 10 years, as well as the scientific and educational activity of its members. Material and methods: We conducted a descriptive and retrospective study. The information was retrieved from a prospective database. The procedures were classified according to their complexity. An analysis was conducted on liver transplantations (LTs), liver resections and complex biliary procedures, performed from 2013 to 2022. Results: Of 1449 surgical procedures, 832 were high-complexity interventions (58%). A total of 452 LTs were performed, 14 (3%) were combined liver and kidney transplantations; overall transplant survival was 83%, 77% and 75% at 1, 3 and 5 years, respectively. Primary graft dysfunction was observed in 5 cases (1.1%). There were 254 liver resections; 58 cases (23%) were related to benign liver diseases, 155 (61%) to malignancies, and 41 (16%) were associated with living donor transplants. Overall morbidity was 27% and in-hospital mortality was 2%. A total of 106 bilio-digestive bypass procedures were carried out, 53 in patients with bile duct injury. Thirty-day morbidity was 42%, and most cases were minor complications (28%). Other procedures included 17 cephalic pancreaticoduodenectomies and 3 splenectomies and distal pancreatectomies. We have published numerous papers, delivered presentations and courses, and implemented a fellowship program in the specialty since 2015. Conclusion: Centralizing hepatobiliary diseases at a high-complexity center led to comparable outcomes with those reported in international series.