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.

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.

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.

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.

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.

Gastrointestinal and Hepatic Involvement in Primary Sjögren's Syndrome: Case Report and Literature Review

Sjögren's syndrome is a systemic autoimmune disease characterized by dry eyes and mouth due to the involvement of exocrine glands. However, it can manifest with GI symptoms that cover a broad spectrum from esophageal and intestinal dysmotility, achalasia, hypochlorhydria, and chronic atrophic gastritis to pancreatic enzyme deficiency, biliary dysfunction, and liver cirrhosis, which varies in its clinical manifestations and is often associated with erroneous approaches. This article reviews the GI manifestations of Sjögren's syndrome. It presents the case of a woman in her eighth decade of life with this syndrome. She showed asymptomatic hepatobiliary disease, documented abnormalities in liver profile tests, and a subsequent diagnosis of primary sclerosing cholangitis, for which she received initial treatment with ursodeoxycholic acid. During her condition, the patient has had three episodes of cholangitis, requiring endoscopic retrograde cholangiopancreatography with no findings of stones, with scant biliary sludge and discharge of purulent bile precipitated by her underlying liver disease. The association between Sjögren's syndrome and primary sclerosing cholangitis is rare and calls for special consideration.

El síndrome de Sjögren es una enfermedad autoinmune sistémica que se caracteriza por la sequedad ocular y bucal debido a la afección de glándulas exocrinas; sin embargo, puede manifestarse con síntomas gastrointestinales que abarcan un espectro amplio desde la dismotilidad esofágica e intestinal, acalasia, hipoclorhidria y gastritis crónica atrófica hasta enzimodeficiencia pancreática, disfunción biliar y cirrosis hepática, que tiene variación en sus manifestaciones clínicas y se asocia con abordajes erróneos en muchas ocasiones. En este artículo se hace una revisión acerca de las manifestaciones gastrointestinales de síndrome de Sjögren y se presenta el caso de una mujer en la octava década de la vida con este síndrome, que cursa con enfermedad hepatobiliar asintomática, documentación de alteración en pruebas de perfil hepático y diagnóstico ulterior de colangitis esclerosante primaria, por lo que recibió un tratamiento inicial con ácido ursodesoxicólico. Durante el curso de su enfermedad ha presentado 3 episodios de colangitis, con requerimiento de colangiopancreatografía retrógrada endoscópica sin hallazgos de cálculos, con escaso barro biliar y salida de bilis purulenta, precipitada por su enfermedad hepática de base. La asociación entre el síndrome de Sjögren y la colangitis esclerosante primaria es infrecuente y justifica una consideración especial.

Intestinal Obstruction Secondary to Gallstone Ileus in a Patient with Cholangitis: Case Report

Gallstone ileus manifests as intestinal obstruction. It occurs due to the passage of a stone and its subsequent lodging in the lumen of the digestive tract. The diagnosis is confirmed by imaging; the gold standard is abdominal tomography. Management is based on the extraction of the intraluminal calculus in one or more surgical times, depending on the patient's condition. We present the case of a patient with multiple comorbidities who showed a picture of cholangitis complicated by gallstone ileus and successfully treated with enterolithotomy. Surgical management is controversial since the optimal approach for these patients has not been established.

El íleo biliar se manifiesta como una obstrucción intestinal, se presenta por el paso de un lito y su posterior alojamiento en el lumen del tubo digestivo. El diagnóstico se confirma mediante imagenología, el patrón de oro es la tomografía abdominal. El manejo se fundamenta en la extracción del cálculo intraluminal en uno o más tiempos quirúrgicos, según el estado del paciente. Se presenta el caso de un paciente con múltiples comorbilidades, que debuta con un cuadro de colangitis complicada por íleo biliar, tratado exitosamente con enterolitotomía. El manejo quirúrgico es controversial, ya que no se ha establecido el abordaje óptimo para estos pacientes.

Carcinoma de vías biliares intrahepáticas: análisis de defunciones según sexo, grupo etario y regiones en Chile entre 2017-2021 / Intrahepatic bile duct carcinoma: analysis of deaths according to sex, age group, and regions in Chile between 2017-2021

Introducción: el colangiocarcinoma intrahepático es un cáncer agresivo de células epiteliales de los conductos biliares intrahepáticos y su desarrollo se asocia a inflamación crónica del árbol biliar. En Chile, su epidemiología es limitada y el presente estudio tiene por objetivo describir su tasa de mortalidad. Métodos: se realizó un estudio descriptivo observacional transversal y ecológico de las defunciones por carcinoma de vías biliares en Chile durante 2017 y 2021 según sexo, grupo etario y región de residencia. Resultados: la tasa de mortalidad nacional de personas mayores a 20 años durante el periodo estudiado fue de 1,56 por cada 100.000 habitantes. La tasa de mortalidad más alta del sexo masculino se observó en 2020, siendo de 2,61. La mayor mortalidad se encontró en personas mayores a 80 años en el sexo masculino con una tasa de 24,38. A nivel regional, en Magallanes se observó la mayor tasa de mortalidad con 5,66, mientras que Tarapacá presentó la menor tasa con un valor de 0,96. Finalmente, el índice de Swaroop fue igual o mayor al 92% en todas las regiones del país. Conclusión: la mayor mortalidad por colangiocarcinoma intrahepático se presenta en personas de edad avanzada y de sexo masculino. Interesantemente la mayor mortalidad por esta causa se concentra en la zona sur de Chile. Dada la magnitud del problema que representa esta enfermedad en la salud pública nacional es que futuros estudios son necesarios para establecer medidas de prevención y/o tratamiento de esta enfermedad.

Introduction: intrahepatic cholangiocarcinoma is an aggressive cancer of epithelial cells of the intrahepatic bile ducts, and its deve-lopment is associated with chronic inflammation of the biliary tree. In Chile, its epidemiology is limited, and the present study aims to describe its mortality rate. Methods: a descriptive, cross-sectional, observational, and ecological study of deaths from bile duct carcinoma in Chile between 2017 and 2021 was performed according to sex, age group, and region of residence. Results: the national mortality rate of people over 20 years old during the study period was 1.56 per 100,000 inhabitants. The highest mortality rate for the male sex was observed in 2020, with a value of 2.61. In turn, the highest mortality rate was found in people over 80 years old in the male sex, with a rate value of 24.38. On a regional level, Magallanes had the highest mortality rate, with a rate value of 5.66, while Tarapacá had the lowest rate, with a value of 0.96. Finally, Swaroop's index was equal to or greater than 92% in all regions of the country. Conclusion: the highest mortality from intrahepatic cholangiocarcinoma occurs in older people and males. Interestingly, the highest mortality from this cause is concentrated in the southern zone of Chile. Given the magnitude of the problem that this disease represents for national public health, future studies are necessary to establish both prevention measures and treatments

Emerging Disease: A Case Series of post-COVID-19 Cholangiopathy

Introduction: Post-COVID-19 cholangiopathy is a novel condition characterized by biliary tract sclerosis and elevated alkaline phosphatase levels in critically ill patients. This case series aims to describe the experience of a Latin American reference hospital in managing this condition. Methods: This case series includes patients with confirmed coronavirus disease 2019 (COVID-19) who exhibited subsequent elevation of alkaline phosphatase levels exceeding three times the normal value. The patients also had documented bile duct abnormalities observed through cholangioresonance or endoscopic retrograde cholangiopancreatography (ERCP). The clinical presentation, imaging findings, complications, and treatment approaches are described. Results: Eight patients (56.5 ± 9.2 years old, 62.5% male) were included in the study. All patients had previously experienced severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) pneumonia and required mechanical ventilation. Four patients (50%) received sedoanalgesia with ketamine, and all eight patients (100%) received propofol. All patients developed infections, such as cholangitis or liver abscesses, caused by gram-negative bacteria. The peak alkaline phosphatase level during follow-up averaged 1646.12 ± 611.3. Imaging findings revealed intrahepatic (100%) and extrahepatic (87.5%) bile duct dilation. In 75% of cases, bile molds with a black appearance were extracted. Seven patients experienced recurrent cholangitis, and three patients were referred for pre-liver transplant consultation. Conclusions: Post-COVID-19 cholangiopathy is characterized by severe cholestasis, intra- and extrahepatic bile duct dilation, formation of bile molds, and recurrent cholangitis. In our study, a possible association between sepsis caused by gram-negative bacteria and the use of sedative medications is hypothesized. Further studies are necessary to establish the most appropriate management strategies for these patients, as they currently face unfavorable long-term morbidity and mortality outcomes.

Introducción: la colangiopatía pos-COVID-19 es una entidad nueva caracterizada por esclerosis del tracto biliar y elevación de fosfatasa alcalina en el paciente críticamente enfermo. Esta serie de casos describe la experiencia de un hospital de referencia en Latinoamérica. Métodos: serie de casos que incluye a pacientes con enfermedad por coronavirus de 2019 (COVID-19) confirmada y elevación posterior de fosfatasa alcalina > 3 veces del valor normal, asociados a anormalidades en las vías biliares documentada por colangiorresonancia o colangiopancreatografía retrógrada endoscópica (CPRE). Se describe la presentación clínica, hallazgos imagenológicos, complicaciones y tratamiento. Resultados: se incluyeron a 8 pacientes (56,5 ± 9,2 años, 62,5% hombres). Todos presentaron neumonía por coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) previo a desarrollo de colangiopatía, y requirieron ventilación mecánica. Cuatro pacientes (50%) recibieron sedoanalgesia con ketamina y ocho (100%) con propofol. Todos presentaron infecciones (colangitis/abscesos hepáticos) por bacterias gramnegativas. El nivel pico de fosfatasa alcalina durante el seguimiento fue en promedio 1646,12 ± 611,3. Los hallazgos imagenológicos incluyeron dilatación de la vía biliar intrahepática (100%) y extrahepática (87,5%). En el 75% se extrajo el molde biliar de apariencia negra. Siete pacientes presentaron recurrencia de colangitis y tres pacientes fueron referidos a consulta de pretrasplante hepático. Conclusiones: la colangiopatía pos-COVID-19 está caracterizada por colestasis grave con dilatación de la vía biliar intra- y extrahepática, formación de moldes biliares y colangitis recurrente. En nuestro estudio se plantea como hipótesis una posible relación con sepsis por bacterias gramnegativas y uso de medicamentos sedativos. Se requieren nuevos estudios para establecer el manejo más adecuado para estos pacientes, que hasta el momento presentan una morbimortalidad desfavorable a largo plazo.

Concurrencia de síndrome de Guillain-Barré y colangitis biliar primaria no vinculado a SARS-CoV-2: reporte de caso / Concurrence of Guillain-Barré syndrome and primary biliary cholangitis not related to SARS-CoV-2: Case report

Introducción El síndrome de Guillain-Barré es una polirradiculoneuropatia de origen autoinmune, considerada la causa más frecuente de parálisis flácida aguda. Se han reportado diversas asociaciones del síndrome de Guillain-Barré con otras enfermedades autoinmunes no neurológicas, algunas de ellas extremadamente raras, como la que ocurre con la colangitis biliar primaria, una enfermedad crónica de etiología autoinmune cuyo diagnóstico se sustenta, además del cuadro clínico, en la alteración de las enzimas hepáticas y la presencia de anticuerpos anti-mitocondriales. Caso clínico Paciente varón de 38 años, sin antecedente de comorbilidades previas, quien luego de presentar enfermedad diarreica dos semanas antes, desarrolló debilidad ascendente de inicio subagudo asociado a parestesias en cuatro extremidades que progresó hasta generar cuadriplejia y dificultad respiratoria. Se le realizó examen citoquímico de líquido cefalorraquídeo que evidenció disociación albumino-citológica y electromiografía que mostró hallazgos compatibles con neuropatía axonal motora aguda. Recibió tratamiento con inmunoglobulina intravenosa a dosis de 0,4 gramos por kilogramo al día, logrando mejoría del cuadro neurológico. Desde su ingreso y durante la hospitalización, presentó alteración persistente de las enzimas hepáticas que seguía un patrón colestásico. Además, se agregó dolor abdominal de leve intensidad y prurito generalizado, por lo cual fue evaluado por gastroenterología, quienes solicitaron anticuerpos anti-mitocondriales que resultaron positivos. Con esta prueba, se comprobó el diagnóstico de colangitis biliar primaria. Conclusión El presente caso muestra una asociación extremadamente rara de dos enfermedades autoinmunes; síndrome de Guillain-Barré y colangitis biliar primaria, tanto así que representa el primer caso reportado, no vinculado a SARS-CoV-2.

Introduction Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies. Clinical case A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis. Conclusion The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.

Microabscesos hepáticos colangiolares secundarios a coledocolitiasis

Los abscesos hepáticos son colecciones supurativas encapsuladas dentro del parénquima hepático, las cuales generalmente se encuentran infectadas por bacterias. Se forman por diferentes mecanismos, una de ellas es la secundaria a la infección de las vías biliares, colangitis aguda, la misma que en su evolución natural puede progresar a una infección severa e incluso a sepsis o choque séptico y es por ello que requiere un diagnóstico y tratamiento adecuados. La litiasis biliar puede complicarse con colangitis aguda tras la obstrucción al flujo de salida de la bilis con posterior infección, generalmente bacteriana, no solo del árbol biliar sino también del parénquima hepático. Se presenta el caso clínico de una paciente mujer joven que cursó con microabscesos hepáticos colangiolares tras un evento de coledocolitiasis no tratado oportunamente.

Hepatic abscesses are suppurative collections encapsulated within the liver parenchyma, which are generally infected by bacteria. They are formed by different mechanisms, one of them is secondary to bile duct infection, acute cholangitis, which in its natural history can progress to severe infection and even sepsis or septic shock and that is why it requires a proper diagnosis and treatment. Gallstones can be complicated by acute cholangitis after bile outflow obstruction with subsequent infection, usually bacterial, not only of the biliary tree but also of the liver parenchyma. We present the clinical case of a young female patient who presented with cholangiolar hepatic microabscesses after an event of choledocholithiasis not treated in a timely manner.

Liver transplantation in patients with primary sclerosing cholangitis: a multicentric study / Transplante hepático em pacientes com colangite esclerosante primária: estudo multicêntrico

ABSTRACT BACKGROUND: The prevalence of primary sclerosing cholangitis (PSC) in the general population has not yet been clearly established. The management of PSC should focus on delaying the progression of the disease and restraining its complications. The only curative therapy for the disease remains liver transplantation (LT). PSC is currently the fifth most common indication for LT and corresponds to 5% of all LT indications in adults. AIMS: Our objective is to evaluate the indications and outcomes of PSC patients undergoing LT in three liver transplantation centers in southern Brazil - Hospital Santa Isabel in Blumenau, Santa Catarina state, and Hospital das Clínicas and Hospital Nossa Senhora das Graças, in Curitiba, Parana state). METHODS: This is a longitudinal observational study of patients with PSC who underwent LT in three major Brazilian medical centers. Electronic medical records and study protocols of all patients subjected to LT from January 2011 to December 2021 were retrospectively reviewed. RESULTS: Of the 1,362 transplants performed in the three medical centers, 37 were due to PSC. Recurrence of PSC occurred in three patients (8.1%) in 3.0±2.4 years (range, 1-4 years). The 1-year and 5-year survival rates after the first LT were 83.8 and 80.6%, respectively. The 1-year and 5-year graft survival rates were, respectively, 83.8 and 74.8%. CONCLUSIONS: Our experience with LT in patients with PSC demonstrated good patient and graft survival results. Most deaths were due to common factors in patients undergoing LT.

RESUMO RACIONAL: A prevalência de colangite esclerosante primária (CEP) na população em geral ainda não foi claramente estabelecida. O manejo da CEP deve se concentrar em retardar a progressão da doença e gerenciar suas complicações. A única terapia curativa para a doença continua sendo o transplante hepático (TH). A CEP é atualmente a 5ª indicação mais comum de TH e corresponde a 5% de todas as indicações de TH em adultos OBJETIVOS: O nosso objetivo é avaliar as indicações e os resultados do transplante de fígado em pacientes com CEP submetidos a TH em três centros de transplante de fígado no sul do Brasil (Hospital Santa Isabel, Blumenau, Santa Catarina; Hospital das Clínicas, Curitiba, Paraná; e Hospital Nossa Senhora das Graças, Curitiba, Paraná). MÉTODOS: Este é um estudo observacional longitudinal de pacientes com colangite esclerosante primária que foram submetidos a transplante hepático em três grandes centros médicos brasileiros. Os prontuários eletrônicos de todos os pacientes submetidos a TH de Janeiro de 2011 a Dezembro de 2021, foram avaliados retrospectivamente. RESULTADOS: De um total de 1.362 transplantes realizados nos três centros médicos, 37 eram devidos à CEP. A recorrência de CEP ocorreu em 3 pacientes (8,1%) em 3±2,4 anos (intervalo, 1-4 anos). A taxa de sobrevida de 1 ano e 5 anos após o 1° TH foi respectivamente de 83,8 e 80,6%. As taxas de sobrevida dos enxertos em 1 ano e 5 anos foram, respectivamente, 83,8 e 74,8%. CONCLUSÕES: A nossa experiência com TH em pacientes com CEP demonstrou bons resultados de sobrevida dos pacientes e dos enxertos. A maioria dos óbitos ocorreu devido a fatores comuns em pacientes submetidos a TH.

Urgent liver retransplantation due to transmission of intrahepatic cholangiocarcinoma by donor: the first report in the literature / Retransplante de fígado urgente devido à transmissão de colangiocarcinoma intra-hepático pelo doador: o primeiro relato da literatura

ABSTRACT BACKGROUND: Liver transplantation represents the best therapeutic modality in end-stage chronic liver disease, severe acute hepatitis, and selected cases of liver tumors. AIMS: To describe a double retransplant in a male patient diagnosed with Crohn's disease and complicated with primary sclerosing cholangitis, severe portal hypertension, and cholangiocarcinoma diagnosed in the transplanted liver. METHODS: A 48-year-old male patient diagnosed with Crohn's disease 25 years ago, complicated with primary sclerosing cholangitis and severe portal hypertension. He underwent a liver transplantation in 2018 due to secondary biliary cirrhosis. In 2021, a primary sclerosing cholangitis recurrence was diagnosed and a liver retransplantation was indicated. Recipient's hepatectomy was very difficult by reason of complex portal vein thrombosis requiring extensive thromboendovenectomy. Intraoperative ultrasound with liver doppler evaluation was performed. Two suspicious nodules were incidentally diagnosed in the donor's liver and immediately removed for anatomopathological evaluation. RESULTS: After pathological confirmation of carcinoma, probable cholangiocarcinoma, at frozen section, the patient was re-listed as national priority and a new liver transplantation was performed within 24 hours. The patient was discharged after 2 weeks. CONCLUSIONS: The screening for neoplasms in donated organs should be part of our strict daily diagnostic arsenal. Moreover, we argue that, for the benefit of an adequate diagnosis and the feasibility of a safer procedure, the adoption of imaging tests routine for the liver donor is essential, allowing a reduction of the costs and some potential risks of liver transplant procedure.

RESUMO RACIONAL: O transplante de fígado representa a melhor modalidade terapêutica na doença hepática crônica terminal, hepatite aguda grave e casos selecionados de tumores hepáticos. OBJETIVOS: Descrever um retransplante duplo em paciente do sexo masculino, diagnosticado com doença de Crohn e complicado com colangite esclerosante primária, hipertensão portal grave e colangiocarcinoma diagnosticado no fígado transplantado. MÉTODOS: Paciente do sexo masculino, 48 anos, diagnosticado com doença de Crohn há 25 anos e complicado com colangite esclerosante primária e hipertensão portal grave. Foi submetido a um transplante de fígado em 2018 devido a cirrose biliar secundária. Em 2021, foi diagnosticada recidiva de colangite esclerosante primária e indicado retransplante hepático. A hepatectomia do receptor foi de alta complexidade devido à trombose complexa da veia porta, exigindo extensa tromboendovenectomia. Foi realizada ultrassonografia intraoperatória com doppler hepático. Dois nódulos suspeitos foram diagnosticados incidentalmente no fígado do doador e imediatamente removidos para avaliação anatomopatológica. RESULTADOS: Após confirmação patológica de carcinoma, provável colangiocarcinoma, pela congelação, o paciente foi relistado como prioridade nacional, e novo transplante hepático foi realizado em 24 horas. O paciente teve alta após 2 semanas. CONCLUSÕES: O rastreamento de neoplasias em órgãos doados deve fazer parte de nosso estrito arsenal diagnóstico diário. Além disso, defendemos que, em benefício de um diagnóstico correto e da viabilidade de um procedimento mais seguro, a adoção de uma rotina de exames de imagem é essencial em doadores hepáticos, permitindo a redução dos custos e alguns riscos potenciais do procedimento de transplante hepático.

Aplicación de guías tokio 2018 para diagnóstico y tratamiento de colangitis aguda en hospital escuela, honduras

Antecedentes: Las Guías Tokio 2018 son un conjunto de directrices médicas actualizadas y reconocidas que ofrecen recomendaciones detalladas para el diagnóstico y tratamiento de la colangitis aguda, una inflamación aguda de los conductos biliares que puede ser potencialmente grave. Objetivo: Examinar la utilidad de la aplicación de las Guías Tokio 2018 para el diagnóstico y tratamiento en pacientes con Colangitis Aguda ingresados al Hospital Escuela durante 2018-2019. Métodos: Estudio descriptivo transversal, se revisaron 54 expedientes clínicos que registraron datos sociodemográficos, antecedentes personales y patológicos, laboratoriales e imagenológicas. Los resultados se presentaron como frecuencias, porcentajes, promedios y rangos. Resultados: Las Guías de Tokio 2018 (TG18) fueron implementadas en un 94%. La colangitis aguda leve fue más frecuente en 48.1%. La concordancia en la estatificación de los casos según TG18 erró con mayor proporción en las colangitis grado 2 (15% No, 7% Si); la lesión renal siendo la falla orgánica más frecuente 25.9% (14). El manejo antibiótico concordó en el 70.0%. El drenaje biliar se realizó de forma oportuna en 14.0%. La causa más frecuente de colangitis fue la litiasis en un 48.2%. El promedio de hospitalización fue de 5-9 días en 31.5%. El 63.0% (34) de los pacientes egresó mejorado y el 24.0% (13) fallecieron. Conclusión: La utilización de lasTG18 por parte del Hospital Escuela demostró un enfoque en mejorar la precisión del diagnóstico y optimizar la administración de antibióticos. Sin embargo, su eficacia parece comparativamente disminuida cuando se aplica al aspecto fundamental del drenaje de los conductos biliares.

Background: The Tokyo 2018 Guidelines are a set of recognized and up-to-date medical guidelines that provide detailed recommendations for the diagnosis and treatment of acute cholangitis, an acute inflammation of the bile ducts that can be potentially serious. Objective: To examine the usefulness of the application of the Tokyo 2018 Guidelines for the diagnosis and treatment of patients with Acute Cholangitis admitted to the Hospital Escuela during 2018-2019. Methods: Cross-sectional descriptive study, reviewing 54 clinical records that recorded sociodemographic data, personal and pathological, laboratory and imaging history. Results were presented as frequencies, percentages, means, and ranges. Results: The Tokyo 2018 Guidelines (TG18) were implemented by 94%. Mild acute cholangitis was more frequent in 48.1%. The concordance in the staging of the cases according to TG18 erred with a greater proportion in grade 2 cholangitis (15% No, 7% Yes); kidney injury being the most frequent organic failure 25.9% (14). The antibiotic management agreed in 70.0%. Biliary drainage was performed in a timely manner in 14.0%. The most frequent cause of cholangitis was lithiasis in 48.2%. The average hospitalization was 5-9 days in 31.5%. 63.0% (34) of the patients discharged improved and 24.0% (13) died. Conclusion: The use of TG18 by the Hospital Escuela demonstrated a focus on improving diagnostic accuracy and optimizing the administration of antibiotics. However, its efficacy appears comparatively diminished when applied to the fundamental aspect of bile duct drainage.

Manifestaciones hepatobiliares asociadas a la enfermedad inflamatoria intestinal / Hepatobiliary manifestations associated with inflammatory bowel disease

La enfermedad inflamatoria intestinal (EII) engloba dos entidades, la enfermedad de Crohn (EC) y la colitis ulcerativa (CU), las cuales son enfermedades inmunomediadas, crónicas y recurrentes que, aunque afectan al intestino, pueden ir acompañadas de manifestaciones extraintestinales de tipo hepatobiliar en el 5 % de los casos. Entre ellas, las más frecuentes son la enfermedad por hígado graso no alcohólico (EHGNA), la colelitiasis, la colangitis esclerosante primaria (CEP), la colangitis relacionada con IgG4, la hepatitis autoinmune (HAI), el síndrome de superposición HAI/CEP, así como la lesión hepática inducida por fármacos (DILI); y otras menos frecuentes como la colangitis biliar primaria (CBP), la trombosis de la vena porta, los abscesos hepáticos, la hepatitis granulomatosa, las hepatitis B y C, la reactivación de la hepatitis B por terapia inmunosupresora, y la amiloidosis. Estas manifestaciones hepatobiliares cursan con una fisiopatología similar o inclusive la misma de la EII, en la que participan el sistema inmune innato y adaptativo, alteración de la microbiota (disbiosis), permeabilidad intestinal, factores de riesgo genéticos (comunes para EII y manifestaciones hepatobiliares) y desencadenantes ambientales. La primera manifestación de un trastorno hepatobiliar es la alteración del perfil de función hepática, por lo que el abordaje diagnóstico se debe dirigir a evaluar y monitorizar las enzimas hepáticas y su asociación a algún patrón diferencial de alteración hepatocelular o colestásico, con el fin de tomar decisiones oportunas con respecto a la suspensión, indicación o modificación de algún medicamento, o cualquier otro abordaje que impida o retrase la evolución de la enfermedad hepatobiliar, y al mismo tiempo garantice el control de la EII, mejorando potencialmente el pronóstico de estos pacientes.

Inflammatory bowel disease (IBD) encompasses two entities, Crohn's disease (CD) and ulcerative colitis (UC), which are chronic, recurrent, immune-mediated inflammatory diseases that, although affect the gut, may be accompanied by extraintestinal hepatobiliary manifestations in 5% of the cases. Among them, the most frequent are non-alcoholic fatty liver disease (NAFLD), cholelithiasis, primary sclerosing cholangitis (PSC), IgG4-related cholangitis, autoimmune hepatitis (AIH), AIH/PSC overlap syndrome, as well as drug-induced liver injury (DILI); and other less frequent such as primary biliary cholangitis (PBC), portal vein thrombosis, liver abscesses, granulomatous hepatitis, hepatitis B and C, reactivation of hepatitis B due to different drugs, and amyloidosis. These hepatobiliary manifestations present with a pathophysiology similar or even the same as that of IBD, where several factors participate, including the innate and adaptive immune system, an interaction with the components of the microbiota, leaky gut, genetic risk factors (common for both IBD and hepatobiliary manifestations) and environmental triggers. The first manifestation of a hepatobiliary disorder is the alteration of the liver profile; therefore, the diagnostic approach should be aimed at evaluating and monitoring liver enzymes and their association with some differential pattern of hepatocellular or cholestatic changes, in order to make appropriate decisions regarding the suspension or modification of any medication, or any other approach that prevents or delays the evolution of hepatobiliary disease, and at the same time guarantees control of IBD, improving the prognosis of these patients.

Clinical significance of Autotaxin in primary biliary cholangitis and primary Sjogren′s syndrome / 中华风湿病学杂志

Objective:Primary biliary cholangitis (PBC) and Primary Sj?gren′s syndrome (pSS) are autoimmune epithelial inflammatory diseases that share many common clinical symptoms. The aim of this study was to investigate the differences and diagnostic value of Autotaxin (ATX) in PBC and SS.Methods:The clinical data of 237 cases diagnosed with PBC, PBC secondary to SS, pSS and healthy individuals(HC) between September 2020 and September 2021 were retrospectively analyzed. The levels of ATX in each group were measured by enzyme-linked immunosorbent assay (ELISA), and the corresponding sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the curve ( AUC), etc were analyzed. Normally distributed data were expressed as mean ±SD and non-normally distributed as median (IQR). The differences and correlations between ATX and the biochemical tests in each group were assessed by applying the Mann-Whitney U test, Spearman correlation analysis, etc. P<0.05 was considered statistically significant difference. Results:The results showed that ATX was positive in 33.9%, 33.3% and 53.3% for PBC, PBC secondary SS, and pSS, respectively, with the specificities of 93.1%, 100% and 93.2%, respectively. The highest accuracy was achieved in pSS and the sensitivity and specificity were 86.5% and 93.2%, which were higher than those in PBC group(56.8%, 93.1%), respectively. Compared with HC [32.6(21.8, 60.5)ng/ml], ATX levels in PBC[59.3(48.6, 86.3)ng/ml, U=1 750.50, P<0.001], PBC-SS [73.6 (53.3,102.4)ng/ml; U=199.00, P<0.001], and pSS [152.6 (97.4,192.1)ng/ml, U=264.00, P<0.001] were elevated with significant difference ( P<0.05). ATX levels showed a decreasing trend from the pSS group to the HC group. ATX in PBC group[AUC(95% CI)= 0.73(0.651,0.812), P<0.001], PBC secondary SS group [AUC(95% CI)=0.82(0.730, 0.912), P<0.001], and pSS group [AUC(95% CI)=0.94(0.898, 0.984), P<0.001] had prediction accuracy. ATX was associated with total protein ( r=-0.31, P=0.041) level and glutaminase (r=-0.26, P=0.024) level. Conclusion:ATX has diagnostic value in both PBC and SS, and with higher sensitivity and specificity for the latter.

New progress in the treatment of primary biliary cholangitis combined with osteoporosis / 中国综合临床

Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is an autoimmune-mediated chronic intrahepatic cholestatic disease, which is more common in middle-aged and elderly women. Cholestasis can be secondary to hepatic osteodystrophy, with osteoporosis (OP) being the most prominent. OP in PBC patients can increase the risk of fracture and seriously affect the survival and quality of life of patients, thus affecting the prognosis of the disease. In recent years, a large number of studies have shown that with the increase of the incidence and prevalence of PBC, the incidence and prevalence of OP are also on the rise. Therefore, in order to improve the survival and quality of life of patients with PBC combined with OP, how to effectively treat it has become the focus of more and more attention and research.

Correlation of neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and D-dimer with the severity of acute cholangitis / 中国基层医药

Objective:To correlate neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and D-dimer (DD) with the severity of acute cholangitis.Methods:The clinical data of 96 patients with acute cholangitis who received treatment in Panjin Central Hospital from September 2019 to March 2021 were retrospectively analyzed. These patients were divided into three groups according to the severity of acute cholangitis: 36 patients with mild acute cholangitis (group A), 35 patients with moderate acute cholangitis (group B), and 25 patients with severe acute cholangitis (group C). The correlation between age, sex, NLR, PLR, DD, and the severity of acute cholangitis was compared among the three groups.Results:In groups A and B, the area under the receiver operating characteristic curve (AUC) showing the performance of DD, NLR, and PLR levels in predicting acute cholangitis was 0.800, 0.838, and 0.721, respectively, with the optimal cut-off value of 1.985 mg/L, 9.589, and 154.410, respectively. Among them, NLR had the largest AUC, and the highest sensitivity (82.9%), and had a high diagnostic value. In groups B and C, the AUC for DD, NLR, and PLR was 0.967, 0.915, and 0.543, respectively, with the optimal cut-off value of 6.000 mg/L, 22.390, and 264.220, respectively. DD and NLR had a diagnostic significance (both P < 0.05), but PLR had no diagnostic significance ( P > 0.05). The AUC for DD was the largest, and therefore DD had a great diagnostic significance. When NLR, PLR, and DD were jointly detected, the AUC was the highest and the diagnostic value was the highest. The AUC in groups A and B was 0.866, and that in groups B and C was 0.977. Conclusion:The levels of DD, NLR, and PLR increase in patients with acute cholangitis, which are related to the severity of the disease. DD, NLR, and PRL can be used as indicators to evaluate mild and moderate acute cholangitis, and NLR has the highest diagnostic value. DD and NLR can be used as indicators to evaluate moderate to severe acute cholangitis, and the effect of DD is superior to that of NLR. The combined detection of the three indicators can increase the value to evaluate the severity of acute cholangitis, and its effect is superior to that of a single detection. The combined detection of NLR, PLR, and DD is helpful for the clinical diagnosis and treatment of acute cholangitis.

Role of T lymphocytes in primary sclerosing cholangitis / 临床肝胆病杂志

Primary sclerosing cholangitis （PSC） is an immune-mediated chronic cholestatic liver disease and can progress to end-stage liver diseases such as liver cirrhosis and liver failure， and there are still no effective treatment methods at present. Studies have found that T lymphocytes are closely associated with the development and progression of PSC. This article reviews the role of T lymphocytes in PSC， so as to provide new ideas for research on the pathogenesis of PSC and the clinical diagnosis and treatment of PSC.

Effect of depression on response to ursodeoxycholic acid and the occurrence of liver cirrhosis in patients with primary biliary cholangitis / 临床肝胆病杂志

ObjectiveDepression is common in patients with primary biliary cholangitis （PBC）， but the role of depression in disease progression remains unclear. This study aims to investigate the association between depression and treatment response and the impact of depression on liver cirrhosis in PBC patients. MethodsA retrospective analysis was performed for the clinical data of 141 patients with PBC who attended the outpatient service of autoimmune liver diseases in General Hospital of Tianjin Medical University from January 2018 to December 2020 and received standard ursodeoxycholic acid （UDCA） monotherapy for 1 year， and 170 healthy controls， matched for age and sex， who underwent physical examination in Physical Examination Center were enrolled as healthy control group. Patient Health Questionnaire-9 （PHQ-9） was used to evaluate depressive state in the patients with PBC and the healthy controls. 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. The binary logistic regression model and the decision tree model were used to analyze the influencing factors for liver cirrhosis in patients with PBC， as well as the influence of depression and the HLA-DRB1 gene on liver cirrhosis. The receiver operating characteristic （ROC） curve， the area under the ROC curve （AUC）， and goodness of fit were used to evaluate model performance. All 13 variables were used to establish a classification and regression tree （CART） model， i.e.， age， sex， PHQ-9 score， the DRB1*03∶01 gene， and the serum levels of alanine aminotransferase， aspartate aminotransferase， alkaline phosphatase （ALP）， gamma-glutamyl transpeptidase （GGT）， total bilirubin， immunoglobulin G， immunoglobulin M （IgM）， C3， and C4. The indications including AUC， sensitivity， and specificity were used to evaluate the performance of CART model in the model cohort. ResultsCompared with the normal control group， the PBC group had a significantly higher proportion of the patients with depression （53.9% vs 15.3%， χ2=57.836， P<0.001）. Compared with the PBC patients without depression， the PBC patients with depression had a significantly poorer response to UDCA treatment （χ2=7.549， P=0.006） and significant increases in the serum levels of ALP （Z=-2.157， P=0.031）， GGT （Z=-2.180， P=0.029）， and IgM （Z=-2.000， P=0.046）. Compared with the PBC patients without depression， the PBC patients carrying the HLA-DRB1*03∶01 allele had a significant increase in the risk of liver cirrhosis （P<0.001）. The binary logistic regression model analysis showed that PHQ-9 score （OR=1.148， 95%CI： 1.050 — 1.255， P=0.002）， the HLA-DRB1*03∶01 gene （OR=5.150， 95%CI： 1.362 — 19.478， P=0.016）， age （OR=1.057， 95%CI： 1.009 — 1.106， P=0.018）， and serum ALP level （OR=1.009， 95%CI： 1.001 — 1.017， P=0.020） were independent risk factors for liver cirrhosis in patients with PBC. The decision tree analysis showed that PHQ-9 score ≥3.5 was also a risk factor for liver cirrhosis in PBC patients. ConclusionDepression is associated with poor treatment response in patients with PBC， and it is an independent risk factor for liver cirrhosis in patients with PBC. This study highlights the important clinical significance of the identification and early management of depressive state in patients with PBC.