A case report on choledochoduodenal fistula: how to suspect this unusual entity?

A choledochoduodenal fistula is an abnormal connection between the common bile duct and the duodenum, which are associated with a history of problems in the common bile duct. It has appeared in 0.74% of patients submitted for biliary tract surgery. The most frequent symptoms of non-obstructive enteric biliary fistulas are: epigastric pain, cholangitis (80.91%), jaundice (54,.26%), fever (50.69%), nausea and/or vomiting (10.30%), abdominal distension (0.39%), asymptomatic (0.11%), and diarrhea (0.11%). Diagnostic imaging methods provide the data of greatest interest in revealing the presence of air in the bile duct. This method, as well as barium reflux under the biliary tree in contrasted studies and in ERCP, reveal the fistulous tract and its location. Neither the prevalence, nor the clinical characteristics that pertain to its presentation, are well known among our population. Possible treatments for this illness include conservative treatment with medication, endoscopic sphincterotomy, and surgical therapy.

Colangitis aguda secundaria a hidatidosis biliar resuelta por colangiopancreatografía retrógrada endoscópica. Reporte de un caso / Acute cholangitis secondary to biliary hydatidosis resolved by endoscopic retrograde cholangiopancreatography. Reporte of a case

Introducción. La hidatidosis biliar es la complicación más frecuente de la hidatidosis hepática. La colangiopancreatografía retrógrada endoscópica desempeña un papel clave en el tratamiento. Caso clínico. Femenina de 57 años acude por dolor abdominal, vómito, diarrea, leucocitosis, hiperbilirrubinemia, en colangioresonancia magnética presenta vía biliar dilatada, defecto de señal en tercio proximal y distal. La colangiopancreatografía retrógrada endoscópica evidencia presencia de cuerpo extraño de aspecto de membranas, vía biliar dilatada, se extrae quistes de aspecto parasitario. Conclusión. El tratamiento de elección es quirúrgico y farmacológico, la colangiopancreatografía retrógrada endoscópica antes de la cirugía, asegura la extracción del material hidatídico y trata la obstrucción biliar, identifica el trayecto fistuloso y facilita su cierre mediante colocación de prótesis y esfinterotomía, por lo que constituye un tratamiento no quirúrgico efectivo y con margen amplio de seguridad.

Introduction: Biliary hydatid disease is the most common complication of hepatic hydatid disease. Endoscopic retrograde cholangiopancreatography plays a key role in treatment. Clinical case: A 57-year-old female presented with abdominal pain, vomiting, diarrhea, leukocytosis, mixed hyperbilirubinemia, and magnetic resonance cholangiography showed a dilated bile duct and a signal defect in the proximal and distal third. Endoscopic retrograde cholangiopancreatography shows the presence of a foreign body with a membrane appearance, a dilated bile duct, and cysts with a parasitic appearance. Conclusion: The treatment of choice is surgical and pharmacological, endoscopic retrograde cholangiopancreatography before surgery ensures the extraction of hydatid material and treats biliary obstruction, identifies the fistulous tract and facilitates its closure by placing a prosthesis and sphincterotomy, which is why it constitutes a Effective non-surgical treatment with a wide margin of safety.

Diagnóstico y tratamiento de abscesos hepáticos, a propósito de tres casos

El absceso hepático tiene baja incidencia, pero alta mortalidad. Su diagnóstico suele ser tardío dada la variabilidad de presentaciones clínicas. Presentamos tres casos en mujeres, de los cuales dos se manifestaron con síndrome febril con microbiología no precisada, mientras que el tercero con shock séptico secundario a colangitis, aislándose Escherichia coli. Todos fueron manejados con antibioticoterapia endovenosa empírica y drenaje quirúrgico, uno vía laparoscópica, dos percutáneos y uno asociado a una colangio-pancreatografía retrógrada endoscópica. La elección terapéutica se determina de acuerdo a la presencia de rotura, tamaño y/o loculaciones del absceso, combinando antibioticoterapia con métodos de drenaje quirúrgico mínimamente invasivos. Se contrasta y discute la bibliografía disponible, destacando la necesidad de investigaciones actualizadas en Chile.

Liver abscess has low incidence but high mortality. Its diagnosis is often delayed due to the variability of clinical presentations. We present three cases in women, two of which manifested with a febrile syndrome with unspecified microbiology, while the third presented with septic shock secondary to cholangitis, with Escherichia coli isolated. All cases were managed with empirical intravenous antibiotic therapy and surgical drainage, one through laparoscopy, two through percutaneous methods, and one associated with endoscopic retrograde cholangiopancreatography. The therapeutic approach is determined based on the presence of rupture, size, and/or loculations of the abscess, combining antibiotic therapy with minimally invasive surgical drainage methods. We discuss the available literature, emphasizing the need for updated research in Chile.

She Medicine （畲药） Diren Zishen Formula （地稔滋肾方） Combined with Acupuncture as Adjunctive Treatment for 32 Cases of Primary Biliary Cholangitis with Liver and Kidney Yin Deficiency Syndrome / 中医杂志

ObjectiveTo observe the clinical effectiveness and safety of She medicine （畲药） Diren Zishen Formula（地稔滋肾方） combined with acupuncture as adjunctive treatment for primary biliary cholangitis with liver and kidney yin deficiency syndrome. MethodsSeventy patients of primary biliary cholangitis with liver and kidney yin deficiency syndrome were randomly divided into a control group and a treatment group， with 35 patients in each group. The control group received oral ursodeoxycholic acid capsules （250 mg per dose， three times daily）. The treatment group received She medicine Diren Zishen Formula oral decoction （one dose daily， 200 ml per dose in the morning and evening， served warm） and acupuncture ［bilateral Sanyingjiao （SP6）， Taichong （LR3）， Ganshu （BL18）， Zusanli （ST36）， Fenglong （ST17）， once daily， 5 consecutive days per week］ in addition to the same treatment as the control group. The treatment duration was three months for both groups. Comparisons were made between the two groups before and after treatment for the following parameters， which were four traditional Chinese medicine （TCM） symptoms scores （skin itching， fatigue， jaundice， and flank pain）， TCM syndrome scores， liver function indicators including aspartate aminotransferase （AST）， alanine aminotransferase （ALT）， alkaline phosphatase （ALP）， gamma-glutamyl transferase （GGT） and total bilirubin （TBiL）， liver fibrosis markers including serum laminin （LN）， serum hyaluronic acid （HA）， serum type Ⅳ collagen （Ⅳ-C） and serum type Ⅲ procollagen （PC-Ⅲ）， and inflammatory factor indicators including serum interleukin-6 （IL-6） and tumor necrosis factor-alpha （TNF-α）. The effectiveness of TCM syndrome between the two groups was compared and safety evaluations were also conducted after treatment. ResultsA total of 32 cases were finally analyzed in the treatment group， while the control group had 31 cases. The total effective rate of TCM syndrome in the treatment group （87.50%， 28/32） was higher than that in the control group （67.74%， 20/31） （P＜0.05）. After treatment， the TCM symptom scores， syndrome scores， liver function， and liver fibrosis markers in both groups signi-ficantly decreased， while in the treatment group， the inflammatory factor indicators decreased after treatment， and more decreases were found than those in the control group （P＜0.05 or P＜0.01）. Both groups had good safety， and no adverse reactions were observed. ConclusionThe combination of She medicine Diren Zishen Formula and acupuncture as an adjunctive treatment for primary biliary cholangitis can significantly improve the clinical effectiveness， improve liver function， reduce inflammatory response， and alleviate liver fibrosis， with good safety.

Research advances in primary biliary cholangitis with dyslipidemia / 临床肝胆病杂志

Primary biliary cholangitis （PBC） is an autoimmune liver disease characterized by progressive and non-purulent inflammation of small- and medium-sized bile ducts in the liver. Recent studies have shown that abnormal lipid metabolism is relatively common in patients with PBC， and 76% of PBC patients have dyslipidemia. The effects and harms of dyslipidemia have attracted much attention. Lipid metabolism disorders play an important role in the progression of PBC. This article mainly reviews the research advances in the manifestation， role， diagnosis， and treatment of lipid metabolism disorders in PBC， so as to provide new ideas for the treatment of PBC.

The role and impact of macrophages in the pathogenesis of primary biliary cholangitis / 临床肝胆病杂志

Primary biliary cholangitis （PBC） is a chronic autoimmune disease of cholestasis in which immune factors lead to progressive small bile duct destruction， cholestasis， and eventually liver fibrosis， liver cirrhosis， and even liver failure. Macrophages， as a group with functional heterogeneity， play different roles in the whole disease process of PBC. This article summarizes the possible ways by which macrophages are involved in the pathogenesis of PBC and discusses their impact on the disease and the potential therapeutic targets of macrophages. It is pointed out that macrophages are mainly involved in innate immunity in PBC injury and are associated with gut microbiota dysbiosis， and they are also associated with cholestasis， liver fibrosis， and liver cirrhosis in the later stages of the disease.

Evaluation value of RDW-SD,RPR and RLR on decompensation stage of cirrhosis in primary biliary cholangitis / 重庆医学

Objective To analyze the evaluation value of the standard deviation of erythrocyte volume distribution width(RDW-SD),erythrocyte volume distribution width standard deviation and platelet ratio(RPR)and erythrocyte volume distribution width standard deviation and lymphocyte ratio(RLR)in the de-compensation stage of cirrhosis in primary biliary cholangitis(PBC).Methods The blood routine indexes of 68 patients with PBC admitted and treated in this hospital from January 2019 to June 2021 were retrospective-ly analyzed and divided into the compensation stage(n=36)and decompensation stage(n=32)according to the diagnostic standard.2 mL venous blood was extracted from the patient on an empty stomach in the early morning.The red blood cell(RBC),mean corpuscular volume(MCV),hemoglobin(Hb),hematocrit(HCT),mean erythrocyte hemoglobin content(MCHC),RDW-SD,white blood cell(WBC),neutrophil absolute value(N#),lymphocyte absolute value(L#),platelet count(PLT),mean platelet volume(MPV),platelet volume distribution width(PDW),etc.were detected.The platelet to lymphocyte ratio(PLR),RPR and RLR were calculated.The influencing factors of decompensation stage of PBC cirrhosis were analyzed by binary logistic regression,and the receiver operating characteristic(ROC)curve was used to analyze the diagnostic values of different indicators in the decompensation stage of PBC cirrhosis.Results There were statistically significant differences in age,RBC,Hb,HCT,RDW-SD,L #,PLT,RPR and RLR between the compensation group and decompensation group in PBC cirrhosis(P＜0.05).The binary logistic regression analysis showed that the age[odds ratios(OR)=1.087,95％confidence intervals(CI):1.015-1.165,P＜0.05],RDW-SD(OR=1.144,95％CI:1.030-1.270,P＜0.05)and RLR(OR=1.041,95％CI:1.007-1.075,P＜0.05)were the independent risk factors for progressing to the decompensation stage in the patients with PBC cirrhosis com-pensation stage.The ROC curve analysis showed that the areas under ROC curve(AUC)of RDW-SD,RPR and RLR for the diagnosis alone of decompensation stage of PBC cirrhosis were 0.726,0.778 and 0.798,re-spectively,and the differences were not statistically significant(P＞0.05).Conclusion Combined with the age factor,regular monitoring of RDW-SD,RPR and RLR levels has a high predictive value for the develop-ment of PBC cirrhosis compensation stage to decompensation stage.

Effect of ANAs on hormone response in patients with AIH-PBC overlap syndrome and AIH-only / 解放军医学杂志

Objective To investigate the effect of antinuclear antibodies(ANAs)on hormone response in patients with autoimmune hepatitis(AIH)-primary biliary cholangitis(PBC)overlap syndrome(AIH-PBC OS)and AIH-only within half a year.Methods A retrospective analysis of 77 patients with autoimmune liver disease(AILD)admitted to First Clinical Medical College of Lanzhou University from January 2018 to December 2021,all of whom were confirmed by liver biopsy and receiving glucocorticoid treatment.Among them,46 patients were in AIH-PBC OS group and 31 were in AIH-only group.The general clinical characteristics,liver puncture-related indexes,autoantibodies and immunoglobulin indexes of patients in each group at the time of diagnosis were collected and compared,and the biochemical and immunoglobulin indexes of patients at the time of hormone use and at the time of review within 6 months were also collected,and the hormone response within 6 months was evaluated according to the levels of glutamic transaminase(AST),glutamic alanine transaminase(ALT)and immunoglobulin G(IgG),and the effect of ANAs on hormone response outcomes in both groups over a six-month period was analyzed.Multifactorial ordered logistic analysis was performed to evaluate the effect of ANAs on hormone response between two groups.Results There was no statistically significant difference in the percentage of AIH-PBC OS and AIH-only patients among both ANAs-positive and-negative AILD patients(55.6%vs.44.4%and 65.6%vs.34.4%,P>0.05).Among 46 patients with AIH-PBC OS,there were 25 in ANAs-positive group and 21 in ANAs-negative group.The rate of complete hormone response within 6 months was lower than that of ANAs-negative group(44.0%vs.76.2%),while the rate of hormone non-response was higher than that of ANAs-negative group(20.0%vs.0),and the difference was statistically significant(P<0.05).There were 20 cases of ANAs-positive and 11 cases of ANAs-negative in the 31 AIH-only patients.There was no statistically significant difference in the results of hormone response within 6 months between the two groups(P>0.05).Multifactorial ordered logistic analysis showed that AIH-PBC OS patients were more likely to have a higher likelihood of 6-month hormone non-response rate in ANAs-positive patients,and the difference was statistically different(P<0.05).And there was no significant effect of ANAs type on hormone response outcome in AIH-only patients(P>0.05).Conclusion AIH-PBC OS ANAs-positive patients have a poor hormone response within half a year.In AIH-only patients,ANAs have no significant effect on hormone response results.

The efficacy of artificial(nonbiological)extracorporeal liver support system for primary biliary cholangitis and the effect of this therapy on prognosis / 实用医学杂志

Objective To evaluate the clinical efficacy of plasma exchange(PE)and double plasma molecular absorption system(DPMAS)in the treatment of primary biliary cholangitis(PBC)and the effect of this therapy on prognosis.Methods The clinical data on 526 PBC patients in our hospital from December 2013 to January 2022 were retrospectively analyzed.The patients were divided into different groups according to different therapies and then matched with propensity.The changes in symptoms,laboratory indexes and MELD scores were compared between two groups before and after treatment,and the clinical efficacy of artificial liver treatment for PBC patients was assessed.The effect of this treatment on the survival outcomes in these patients via comparing the cumulative survival rates at 3,6 and 12 months between the two groups.Results The efficiency was better in the group with artificial liver treatment in addition medical therapy than the group with medical treatment alone,the difference was statistically significant(76.7%vs.55.8%,χ2 = 4.214,and P = 0.040).Cox proportional risk regression showed that TBIL was an independent risk factor affecting the 3-,6-,or 12-month survival in PBC patients.Conclusions Artificial liver support system can effectively relieve symptoms,reduce levels of ALT,AST and TBIL,improve blood coagula-tion function,and lower MELD scores in PBC patients.This therapy revealed a trend of improvement in 3-,6-,or 12-month survival outcomes.

Advances in drug therapy for primary sclerosing cholangitis / 临床肝胆病杂志

Primary sclerosing cholangitis （PSC） is a cholestatic disease characterized by chronic progressive bile duct inflammation and has a low incidence rate and poor prognosis in China. There is still no drug therapy that can change the course of PSC， and liver transplantation is the only effective treatment for PSC， with a 5-year survival rate of 85% after transplantation. Drug therapy for PSC is facing great challenges based on the current status of PSC. At present， drugs for the treatment of PSC are in the stage of clinical trials and have shown certain application prospect， among which ursodeoxycholic acid is the most widely studied and commonly used drug. In addition， there are many emerging drugs in the pipeline. This article summarizes the latest advances in drug therapy for PSC.

Pathological diagnosis of primary biliary cholangitis / 临床肝胆病杂志

In recent years， new advances have been achieved for the research on the pathogenesis， diagnosis， and treatment of primary biliary cholangitis （PBC）. Accurate diagnosis， prognosis assessment， and risk stratification based on the clinicopathological features of patients are important for the treatment of PBC. This article summarizes the advances in the clinicopathological features and treatment of PBC.

Effect of ursodeoxycholic acid on symptoms after severe acute respiratory syndrome coronavirus 2 infection in patients with primary biliary cholangitis and their family members / 临床肝胆病杂志

ObjectiveTo investigate the effect of ursodeoxycholic acid （UDCA） on the symptoms after severe acute respiratory syndrome coronavirus 2 （SARS-CoV-2） infection in patients with primary biliary cholangitis （PBC） and their family member. MethodsA questionnaire survey was conducted to collect related information from 171 PBC patients who attended The First Affiliated Hospital of Air Force Medical University before March 22， 2023 and 128 family members， including demographic information， comorbidities， UDCA administration， SARS-CoV-2 infection， vaccination， symptoms， therapeutic medication， and the changes in liver disease-related symptoms. The independent-samples t test or the Mann-Whitney U test was used for comparison of continuous data between two groups， and the chi-square test or the Fisher’s exact test was used for comparison of categorical data between two groups. ResultsThe median age was 51 years in the PBC patients and 49 years in the family members， with no significant difference between the two groups （P>0.05）. Compared with the family member group， the PBC group had significantly lower body mass index （22.2±2.4 kg/m2 vs 23.3±2.9 kg/m2， P<0.001） and proportion of male individuals （10% vs 55%， P<0.001）. All PBC patients received UDCA at a dose of 13‍ ‍—‍ ‍15 mg/kg， and SARS-CoV-2 infection rate was 100% in both groups. The family members had a significantly higher SARS-CoV-2 vaccination rate than the PBC patients （91% vs 57%， P<0.001）. Compared with the family members， the PBC patients had significantly milder symptoms of sneezing， nasal obstruction， chest pain， and abnormal taste （P<0.05）. Compared with the family members， the PBC patients had significantly lower rates of use of compound cold medicine （11% vs 20%， P<0.05） and Lianhua Qingwen capsules （12% vs 21%， P<0.05）. For the PBC patients after SARS-CoV-2 infection， the liver disease-related symptoms such as fatigue， abdominal distension， dry mouth and dry eyes， pruritus， and yellow skin were aggravated by 37%， 2%， 27%， 10%， and 3%， respectively. ConclusionCompared with the immediate family members of PBC patients who do not take UDCA， the PBC patients receiving UDCA do not show a reduction in SARS-CoV-2 infection rate， but UDCA may have a certain effect on alleviating infection-related symptoms in such patients. PBC patients may still experience the aggravation of liver disease-related symptoms after SARS-CoV-2 infection， and the long-term effect on PBC patients after SARS-CoV-2 infection should be taken seriously in clinical practice.

Value of alkaline phosphatase level after ursodeoxycholic acid treatment for one month and baseline red blood cell distribution width in predicting the treatment response of primary biliary cholangitis / 临床肝胆病杂志

ObjectiveTo investigate the value of baseline red cell distribution width （RDW） and alkaline phosphatase （ALP） level after ursodeoxycholic acid （UDCA） treatment for one month in predicting the response to UDCA treatment in patients with primary biliary cholangitis （PBC）. MethodsA retrospective analysis was performed for the data of 127 patients with PBC who were diagnosed in Department of Hepatology， The Third People’s Hospital of Jiangsu University， from January 2015 to July 2022， with data collected at baseline， after one month of treatment， and after one year of follow-up. Based on the Paris-I criteria， the patients were divided into good response group and poor response group， and the two groups were analyzed in terms of clinical and laboratory features and their association with response to UDCA. The Logistic regression method was used to investigate the independent risk factors for response to UDCA treatment. The area under the ROC curve （AUC） was used to determine the optimal cut-off values of related indicators； the patients were divided into two groups based on such values， and the two groups were compared in terms of baseline indicators and response. The independent-samples t test was used for comparison of normally distributed continuous data between two groups， and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups； the chi-square test was used for comparison of categorical data between two groups. ResultsCompared with the good response group， the poor response group had significantly higher levels of total bilirubin， aspartate aminotransferase/alanine aminotransferase， ALP， RDW， and RDW-CV at baseline and a significantly higher level of ALP after one month of UDCA treatment （Z=-4.792， -3.697， -2.399， -4.102， -3.220， and -4.236， all P<0.05）. Compared with the good response group， the poor response group had significantly lower levels of albumin， hemoglobin， lymphocytes， hematocrit， and body mass index at baseline （Z=-3.592， -3.603， -2.602， -3.829， -2.432， all P<0.05）， as well as significantly lower levels of prealbumin， albumin/globulin ratio， apolipoprotein A， and free triiodothyronine at baseline （t=4.530， 3.402， 3.485， and 3.639， all P<0.001）. Compared with the poor response group， the good response group had a significantly lower proportion of patients with liver cirrhosis， gallstones/cholecystitis， or anemia （χ2=20.815， 3.892， and 12.283， all P<0.05）. Baseline RDW （odds ratio ［OR］=1.157， 95% confidence interval ［CI］： 1.028‍ — ‍1.301， P=0.015） and ALP level after one month of treatment （OR=1.012， 95%CI： 1.005‍ — ‍1.020， P=0.002） were independent risk factors for response to UDCA， with an AUC of 0.713 and 0.720， respectively. The patients with baseline RDW≥upper limit of normal （ULN） and ALP≥2.2×ULN after one month of UDCA treatment had a lower UDCA response rate （42.6% vs 8.2%， χ2=20.813， P<0.001）. ConclusionPatients with baseline RDW≥ULN and ALP≥2.2×ULN after one month of UDCA treatment tend to have a low biochemical response rate to UDCA.

Current status of research on the genetic susceptibility of primary biliary cholangitis / 临床肝胆病杂志

Primary biliary cholangitis （PBC） is a liver autoimmune disease with a strong genetic tendency characterized by the degeneration and necrosis of bile duct epithelial cells， and it is often observed in middle-aged and elderly women. With the continuous development of genome-wide association studies， the genetic susceptibility of PBC has attracted more and more attention. This article elaborates on the research advances in the genetic susceptibility genes closely associated with PBC， in order to provide effective targets for the treatment of PBC.

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.

Prognostic role of magnetic resonance imaging of the abdomen with intravenous contrast and magnetic resonance cholangiopancreatography in primary sclerosing cholangitis / Papel prognóstico da ressonância magnética do abdome com contraste intravenoso e colangiopancreatografia por ressonância magnética na colangite esclerosante primária

Abstract Objective: To evaluate the usefulness of Anali scores, determined by magnetic resonance imaging, for predicting the prognosis of primary sclerosing cholangitis (PSC) and to analyze interobserver variability, as well as to assess the impact of periportal edema and heterogeneous signal intensity on diffusion-weighted imaging of the liver. Materials and Methods: This was a retrospective cohort study of 29 patients with PSC and baseline magnetic resonance imaging. Anali scores, without gadolinium (0-5 points) and with gadolinium (0-2 points), were calculated by two radiologists. Clinical end-points included liver transplantation, cirrhotic decompensation, and death. We calculated intraclass correlation coefficients (ICCs) for interobserver agreement on the Anali scores, performed Kaplan-Meier survival analysis comparing event-free survival among the score strata, and calculated the areas under receiver operating characteristic curves to determine sensitivity and specificity. Results: Among the patients with a clinical event, the median Anali score was 4 (interquartile range [IQR], 2-5) without gadolinium and 2 (IQR, 1-2) with gadolinium, compared with 1 (IQR, 1.0-2.5) and 1 (IQR, 0.25-1.0), respectively, among those without a clinical event. The ICC was 0.79 (95% confidence interval: 0.57-0.91) for the Anali score with gadolinium and 0.99 (95% confidence interval: 0.98-0.99) for the Anali score without gadolinium. Periportal edema and heterogeneous signal intensity in the liver on diffusion-weighted imaging showed no statistical impact on clinical events (p = 0.65 and p = 0.5, respectively). Conclusion: Anali scores correlate with clinical events in PSC, with a high level of interobserver agreement.

Resumo Objetivo: Avaliar a utilidade dos escores Anali determinados por ressonância magnética para prever o prognóstico da colangite esclerosante primária (CEP), analisar a variabilidade interobservador e avaliar o impacto do edema periportal e do sinal heterogêneo do fígado em imagens ponderadas por difusão. Materiais e Métodos: Estudo retrospectivo de coorte de 29 pacientes com CEP e ressonância magnética de base. Os escores Anali sem gadolínio (0 a 5 pontos) e com gadolínio (0 a 2 pontos) foram calculados por dois radiologistas. Os desfechos clínicos incluíram transplante de fígado, descompensação cirrótica ou morte. Foram realizados coeficiente de correlação intraclasse (CCI) para a concordância interobservador com relação ao escore Anali, análise de sobrevivência de Kaplan-Meier comparando o tempo livre de eventos de acordo com o escore, e área sob a curva característica de operação do receptor para sensibilidade e especificidade. Resultados: Nos pacientes com evento clínico, a mediana do escore Anali sem gadolínio foi 4 (intervalo interquartil [IIQ]: 2-5) e com gadolínio foi 2 (IIQ: 1-2), enquanto nos pacientes sem evento clínico o escore sem gadolínio foi 1 (IIQ:1-2,5) e com gadolínio foi 1 (IIQ: 0,25-1). A concordância interobservador com gadolínio foi CCI = 0,79 (intervalo de confiança 95%: 0,57-0,91) e sem gadolínio foi CCI = 0,99 (intervalo de confiança 95%: 0,98-0,99). O edema periportal (p = 0,65) e o sinal heterogêneo do fígado nas imagens ponderadas por difusão (p = 0,5) não apresentaram impacto nos eventos clínicos. Conclusão: Os escores Anali se correlacionam com eventos clínicos na CEP, com alto grau de concordância interobservador.

Enfermedad pulmonar intersticial en colangitis biliar primaria / Interstitial Lung Disease in Primary Biliary Cholangitis

Durante el transcurso de la colangitis biliar primaria se puede desarrollar compromiso intersticial pulmonar: neumonía organizada, fibrosis intersticial, neumonía intersticial linfoide, neumonía intersticial no específica. A pesar de que el diagnóstico de colangitis biliar primaria usualmente precede a las manifestaciones pulmonares, puede ocurrir lo inverso. La frecuencia de enfermedad intersticial en pacientes con colangitis biliar primaria no es conocida con exactitud. Puede estar o no asociada a otras enfermedades del tejido conectivo; por lo tanto, es necesario realizar una búsqueda sistemática de estas y de las manifestaciones pulmonares de dicha entidad. Presentamos el caso de una paciente con diagnóstico previo de colangitis biliar primaria, la cual desarrolla durante el curso de su enfermedad, afectación pulmonar intersticial.

During the course of PBC, interstitial lung involvement may develop: organizing pneu monia, interstitial fibrosis, lymphoid interstitial pneumonia, or non-specific interstitial pneumonia. Although the diagnosis of PBC usually precedes pulmonary manifestations, the opposite can occur. The frequency of interstitial disease in patients with PBC is not exactly known. It may or may not be associated with other connective tissue diseases; therefore, it is necessary to carry out a systematic search of these diseases and the pulmonary manifestations of this entity. We present the case of a patient with a previ ous diagnosis of PBC, who developed interstitial lung involvement during the course of the disease.

Bacteriological profile of patients with biliary obstruction in tertiary care center

Background: Bile ducts are usually kept sterile by bacteriostatic and flushing effects of bile. Patients presenting with biliary obstruction especially due to benign etiology have either single or multiple bacterial organisms. Acute cholangitis carries significant morbidity with variable mortality rate. The serious presentation of such toxic patients signifies the requirement of appropriate antibiotic treatment. Choledocholithiasis followed by neoplasm and benign biliary strictures are the common predisposing factors for obstruction. Widespread use of antibiotics over years lead to change in sensitivity pattern of organisms which necessitates change in empiric antibiotic usage. Methods: This retrospective study was conducted in department of medical gastroenterology. We studied 50 patients with biliary obstruction (clinical and demographic data were recorded). The diagnosis of cholangitis was made according to TG2018. While undergoing therapeutic ERCP, bile aspirate was collected by biliary cannulation and sent for microbiological analysis. The antibiotic susceptibility pattern and rest of the data were analyzed by appropriate statistical tests. Results: Mean age of the study group was 49 years. Majority were female in study (60%). Overall, 74% had benign etiology, 32% had cholangitis. Bile cultures were positive in 64% patients 75% of them had benign etiology (gallstone being most common; 56%) and 25% had malignant etiology. Bacterobilia in cholangitis patients was statistically significant in comparison to patients without cholangitis (p-0.01). Organisms grown are mainly aerobic gram negative, most common being E. coli, Klebsiella and Pseudomonas species. Patients having bacterobilia had mortality of 6.25% during hospital stay. Conclusions: In this study we found higher sensitivity to colistin (90.6%), tigecycline (81.25%), amikacin (75%) and least sensitivity was noted for ampicillin (6.25%) followed by cefixime (12.5%). Sensitivity to previously commonly used ciprofloxacin antibiotic was 31.25%. Study confirms the significance of obtaining routine bile sample during ERCP in obstructed biliary system to prevent dreaded complications of cholangitis.

Isolated IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma: a case report of a challenging disease

IgG4-related disease (IgG4-RD) has recently attracted attention worldwide; however, its diagnosis still remains challenging. It is an immune-mediated multisystem disease affecting a wide range of organs in the human body. Even though it's uncommon, IgG4-sclerosing cholangitis, which is a biliary manifestation of IgG4-RD, can happen without pancreatic involvement. Here, we report a case of a patient with imaging features typical of hilar cholangiocarcinoma, which eventually turned out to be IgG4 sclerosing cholangitis.