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PURPOSE: To report two cases of non-granulomatous unilateral anterior uveitis in two female patients associated with autoimmune liver diseases (ALD), emphasizing the possibility of this rare coexistence as a polyautoimmunity phenomenon. CASE DESCRIPTIONS: Case 1: An 18-year-old female with a history of congenital renal hypoplasia and metabolic syndrome presented with anterior uveitis in OS and a history of jaundice, blood elevated hepatic enzymes, and cholangioresonance compatible with primary sclerosing cholangitis (PSC). Laboratory work-up for additional autoimmune and infective causes were within normal limits. Case 2: An 58-year-old female presented an episode of anterior uveitis in OD and a history of Sjögren syndrome diagnosed at the age of 53, primary biliary cholangitis (PBC), systemic sclerosis, Raynaud's phenomenon, bilateral sacroiliitis, and vitiligo, consistent with polyautoimmunity and multiple autoimmune syndrome. CONCLUSIONS: Uveitis rarely coexists with ALD. However, it is essential to recognize the possibility of polyautoimmunity in patients presenting with ophthalmic manifestations and a previous diagnosis of ALD, such as PSC or PBC.

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Parasitic infections continue to be a burden for developing countries-the lack of hygiene measures and clean water results in dangerous scenarios that can lead to challenging problems. Most Ascaris lumbricoides infections are usually mild; nonetheless, in rare cases in endemic regions, they can migrate to the bile duct and cause severe complications requiring surgery. We present the case of a 43-year-old woman who had severe cholangitis due to A. lumbricoides. The worm migrated from the bowel and obstructed the bile duct; after surgery, the patient fully recovered.

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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.

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OBJECTIVE: To investigate the value of endoscopic duodenal papillary sphincterotomy combined with balloon dilatation in the treatment of duodenal papilloplasty with titanium clip after choledocholithiasis in post-operative complications. MATERIALS AND METHODS: One hundred and twenty-five patients (69 males and 56 females) with a median age of 65 (32-81) years were included. The treatment plan was randomly divided into Group A (n = 59) and Group B (n = 66) according to the random number table. Patients in Group A were treated with endoscopic sphincterotomy (EST) combined with endoscopic papillary large balloon dilation (EPLBD), followed by a titanium clip for duodenal papilloplasty and then indwelling nasobiliary drainage, whereas those in Group B were treated with EST combined EPLBD to remove stones and then indwelling nasobiliary drainage. RESULTS: In patients with choledocholithiasis or with anatomical changes that make stone extraction difficult, this prospective study attempted to perform duodenal papilloplasty with titanium clips after EST and EPLBD lithotripsy to compare and observe post-operative papillary healing, biliary reflux, and complication rates. CONCLUSIONS: The use of endoscopic duodenal papilloplasty with a titanium clip can improve biliary reflux after lithotripsy and reduce the incidence of post-operative cholangitis complications.

OBJETIVO: Investigar el valor de la esfinterotomía papilar duodenal endoscópica combinada con dilatación con balón en el tratamiento de la papiloplastia duodenal con clip de titanio después de coledocolitiasis en complicaciones postoperatorias. MATERIALES Y MÉTODOS: Se incluyeron un total de 125 pacientes (69 hombres y 56 mujeres) con una mediana de edad de 65 (32-81) años. Los pacientes del Grupo A se trataron con esfinterotomía endoscópica (EST) combinada con dilatación papilar endoscópica con balón grande (EPLBD), seguida de clip de titanio para papiloplastia duodenal y luego drenaje nasobiliar permanente, mientras que los del Grupo B se trataron con EPLBD combinado con EST para eliminar cálculos y luego drenaje nasobiliar permanente. RESULTADOS: En pacientes con coledocolitiasis o con cambios anatómicos que dificultan la extracción de cálculos, este estudio prospectivo intentó realizar papiloplastia duodenal con clips de titanio después de litotricia EST y EPLBD para comparar y observar la cicatrización papilar postoperatoria, el reflujo biliar y las tasas de complicaciones. CONCLUSIÓN: El uso de papiloplastia duodenal endoscópica con clips de titanio puede mejorar el reflujo biliar después de la litotricia y reducir la incidencia de complicaciones de colangitis postoperatorias.

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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 immunosuppression, 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 abdomen 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 producing extended spectrum beta-lactamase (ESBL) and Candida 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.

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 etiológico 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. Presentamos el caso de una mujer de 67 años, que no presentaba 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.

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INTRODUCTION AND OBJECTIVES: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. MATERIALS AND METHODS: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. RESULTS: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). CONCLUSIONS: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.

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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.

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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.

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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.

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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.

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BACKGROUND: Primary sclerosing cholangitis (PSC) manifests within a broad ethnic and racial spectrum, reflecting different levels of access to health care. AIM: To evaluate the clinical profile, complications and survival rates of patients with PSC undergoing liver transplantation (LTx) at a Brazilian reference center. METHODS: All patients diagnosed with PSC before or after LTx were included. The medical records were reviewed for demographic and clinical variables, including outcomes and survival. The level of statistical significance was set at P < 0.05. RESULTS: Our cohort represented 1.6% (n = 34) of the 2113 patients receiving liver grafts at our service over the past two decades. Most were male (n = 19; 56%). The average age (40 ± 14 years) was similar for men and women (P = 0.347). The mean follow-up time from diagnosis to LTx was 68 mo. Most patients had the classic form of PSC. Three women had PSC/autoimmune hepatitis overlap syndrome, and one patient had small-duct PSC. Alkaline phosphatase levels at diagnosis and pre-LTx model for end-stage liver disease. scores were significantly higher in males. Inflammatory bowel research (IBD) was investigated by colonoscopy in 26/34 (76%) and was present in most cases (18/26; 69%). IBD was less common in women than in men (44.4% vs. 55.6%) (P = 0.692). Cholangiocarcinoma (CCA) was diagnosed in 2/34 (5.9%) patients by histopathology of the explant (survival: 3 years 6 mo, and 4 years 11 mo). Two patients had complications requiring a second LTx (one after 7 d due to hepatic artery thrombosis and one after 17 d due to primary graft dysfunction). Five patients (14.7%) developed biliary stricture. The overall median post-LTx survival was 66 mo. Most deaths occurred in the first year (infection n = 2, primary liver graft dysfunction n = 3, unknown cause n = 1). The 1-year and 5-year survival rates of this cohort were 82.3% and 70.6%, respectively, matching the mean overall survival rates of LTx patients at our center (87.1% and 69.43%, respectively) (P = 0.83). CONCLUSION: Survival after 1 and 5 years was similar to that of other LTx indications. The observed CCA survival rate suggests CCA may be an indication for LTx in selected cases.

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Platynosomum sp. is a parasite that infects the liver and biliary tract of domestic and wild cats, causing platynosomiasis. A systematic review with meta-analysis was performed to determine the global prevalence and distribution of Platynosomum sp. based on epidemiological studies and case reports. A total of 73 articles met the inclusion criteria, including 46 studies of an epidemiological nature and 27 case report articles, comprising 35 affected animals. Meta-analysis of epidemiological studies revealed an overall prevalence of 17.8%, with high heterogeneity. Central America had the highest prevalence at 64.1%, followed by South America at 15.1%. Necropsy was the primary diagnostic method, followed by morphological detection of eggs in faeces at 29.3% and 12.5%, respectively. The analysis of case reports highlighted a greater number in South America and confirmed the presence of the parasite in countries where no epidemiological studies were obtained. The most infected animals were those that met the criteria of being mixed-breed, over 4 years old, and having unrestricted outdoor access, but no gender predilection was observed. General comorbidities were the most observed, followed by icterus. In addition, pathological changes were observed in the necropsied animals of the case reports, and the presence of thickening of the ducts and gallbladder was mainly identified, followed by fibrosis and hyperplasia, linked to the presence of an adult parasite. In conclusion, this systematic review with meta-analysis revealed a significant prevalence of Platynosomum sp. at a global level, mainly in the Americas, and that comprehensive studies are necessary for a more reliable prevalence, considering that this is a parasite of great importance for feline medicine.

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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.

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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.

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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

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La apendicitis aguda es la patología quirúrgica abdominal más común alrededor del mundo. Presentamos un caso de un paciente de 78 años que se presentó con un cuadro de apendicitis aguda en el servicio de urgencias. La tomografía computada de abdomen y pelvis mostró una apendicitis aguda secundaria a la obstrucción del orificio apendicular por una prótesis biliar migrada. Se realizó un manejo exitoso mediante el retiro de la prótesis por colonoscopía, permitiendo el alta hospitalaria del paciente 72 horas posteriores al ingreso.

Acute appendicitis is the most common surgical abdominal pathology worldwide that requires immediate intervention. We report a 78-year-old patient who presented with acute appendicitis. A computed tomography (CT) of the abdomen and pelvis showed acute appendicitis due to appendiceal orifice obstruction from a migrated biliary stent. The condition was successfully treated nonoperatively with endoscopic stent removal, allowing his discharge 72 hours after his admission.

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Anti-gp210 is the disease-specific anti-nuclear antibody (ANA) of primary biliary cholangitis (PBC). Anti-gp210-positive PBC patients have worse responses to ursodeoxycholic acid (UDCA) as compared with anti-gp210-negative patients. Moreover, anti-gp210-positive patients always present with more severe histopathologic features including lobular inflammation, interfacial hepatitis, and bile duct injury, and have a worse prognosis than their anti-gp210-negative counterparts. Previous studies have identified two antigenic epitopes recognized by anti-gp210. Although the pathogenetic mechanism of anti-gp210 production remains unclear, evidence suggests that the autoimmune response to anti-gp210 production might be due to molecular mimicry induced by bacteria or endogenous peptides. T cells and related cytokines play a critical role in the pathogenesis of PBC, however, the mechanism hasn't been fully understood. Thus, this review focuses on the clinicopathological characteristics of anti-gp210-positive PBC patients, the fundamental research of gp210 antigen, and the possible mechanism of anti-gp210 production to clarify the mechanism of anti-gp210-positive PBC and provide potential molecular targets for disease prevention and treatment in the future.

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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.

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Background: Periampullary duodenal diverticula are rare and pancreaticobiliary complications infrequent, however, when they are diagnosed and associated with symptoms, they warrant urgent intervention. The aim of this article is to present a clinical case of severe cholangitis secondary to the presence of a periampullary diverticulum successfully treated endoscopically. Clinical case: A 68-year-old man with a history of diabetes and hypertension, was admitted to the emergency room with symptoms of abdominal pain, fever, and tachycardia. With acute kidney injury and alterations in liver function tests, ultrasound with dilated common bile duct and gallstones. Magnetic resonance cholangiography is performed, showing duodenal diverticulum and choledocholithiasis. Antibiotic management is given, and endoscopic retrograde cholangiopancreatography is decided, finding a duodenal diverticulum with stones and pus inside, sphincterotomy, transpapillary dilation and multiple sweeps are performed. Cholecystectomy was performed 7 days later, and the patient was discharged without complications. Conclusions: In patients with signs of severe cholangitis, it is important not to delay endoscopic retrograde cholangiopancreatography, even when infrequent associated pathologies are evidenced, such as a periampullary duodenal diverticulum, since this represents the diagnostic and therapeutic method of choice with high rates of resolution in the case of an obstructive pathology of the bile duct.

Introducción: los divertículos duodenales periampulares son raros y las complicaciones pancreaticobiliares infrecuentes; sin embargo, cuando se diagnostican y se asocian a sintomatología ameritan intervención urgente. El objetivo de este trabajo es presentar un caso clínico de colangitis severa secundaria a la presencia de un divertículo periampular tratado de manera exitosa por vía endoscópica. Caso clínico: hombre de 68 años con antecedentes de diabetes e hipertensión, quien acude al área de Urgencias con cuadro de dolor abdominal, fiebre y taquicardia. Se identifica lesión renal aguda y alteraciones en las pruebas de función hepática, ultrasonido con colédoco dilatado y litiasis vesicular. Se realiza colangioresonancia magnética que evidencia divertículo duodenal y coledocolitiasis. Se otorga manejo antibiótico y se decide colangiopancreatografía retrógrada endoscópica, encontrándose divertículo duodenal con litos y pus en su interior, se realiza esfinterotomía, dilatación transpapilar y múltiples barridos. Se realiza colecistectomía a los siete días y se egresa a domicilio por mejoría sin complicaciones. Conclusiones: en pacientes con datos de colangitis grave es importante no retrasar la colangiopancreatografia retrógrada endoscópica, aun cuando se evidencien patologías asociadas infrecuentes como un divertículo duodenal periampular, ya que esta representa el método diagnóstico y terapéutico de elección con tasas altas de resolución ante una patología obstructiva de la vía biliar.

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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.

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.

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