Papel del ácido ursodesoxicólico en 40 años de tratamiento para la colangitis biliar primaria / Role of ursodeoxycholic acid in 40 years of treatment for primary biliary cholangitis

La colangitis biliar primaria es una enfermedad hepática autoinmune que conduce a la destrucción progresiva de los conductos biliares intrahepáticos, lo que aumenta el riesgo de desarrollar cirrosis e hipertensión portal. Actualmente, el ácido ursodesoxicólico es el medicamento de primera línea para el tratamiento de esta entidad. Este medicamento desplaza los ácidos biliares hidrofóbicos y aumenta las concentraciones de ácidos biliares hidrofílicos en la bilis, lo cual favorece la integridad de los conductos biliares, adicionalmente, tiene efectos antiinflamatorios y propiedades inmunomo-duladoras y antiapoptóticas. En los últimos 40 años, numerosos ensayos clínicos han respaldado la eficacia clínica del ácido ursodesoxicólico y su seguridad cuando se utiliza en pacientes con colan-gitis biliar primaria. Se realiza una revisión del ácido ursodesoxicólico en el contexto de colangitis biliar primaria, se describe su historia, mecanismos de acción, efectos secundarios y dosificación. Finalmente, se menciona su uso en situaciones especiales como son el embarazo y la lactancia

Primary biliary cholangitis is an autoimmune liver disease that leads to progressive destruction of intrahepatic bile ducts, increasing the risk of developing cirrhosis and portal hypertension. Currently, ursodeoxycholic acid is the first-line drug for the treatment of this condition. This drug displaces hy-drophobic bile acids and increases concentrations of hydrophilic bile acids in the bile, which favors the integrity of the bile ducts, additionally, it has anti-inflammatory effects and immunoprotective and antiapoptotic properties. Over the past 40 years numerous clinical trials have supported the clinical efficacy of ursodeoxycholic acid and its safety when used in patients with primary biliary cholangitis. A review of ursodeoxycholic acid in the context of primary biliary cholangitis is carried out, and its history, mechanisms of action, side effects and dosage are described. Finally, its use in special situations such as pregnancy and lactation are discussed.

Innovación en técnica quirúrgica: Dos técnicas no convencionales para el tratamiento de la coledocolitiasis / Innovative surgical techniques. Two non-conventional techniques for the treatment of choledocholithiasis

RESUMEN La litiasis vesicular asociada a coledocolitiasis puede tener distintos tratamientos, endoscópicos o quirúrgicos, dependiendo del paciente, el entrenamiento del personal médico y de la disponibilidad de instrumental. Ninguno ha demostrado estar exento de complicaciones. Presentamos dos técnicas tendientes a mejorar los resultados del tratamiento quirúrgico de la coledocolitiasis. Una es la extracción transcística de coledocolitiasis y stent, en pacientes que fueron tratados endoscópicamente por colangitis, tendiente a resolver el problema (la litiasis vesicular, la coledocolitiasis y el stent) en un solo tiempo por cirugía laparoscópica. La segunda es una nueva indicación de una técnica ya descripta, la dilatación papilar anterógrada con balón, utilizada en este caso para disminuir las fugas biliares tras un cierre primario de colédoco.

ABSTRACT Cholelithiasis associated with choledocholithiasis may have different treatments, either by endoscopy or surgery, depending on the patient, level of training of the medical staff and availability of instruments. None of them is free of complications. We report two non-conventional techniques aimed at improving the results of the management of choledocholithiasis. Transcystic removal of common bile duct stones and stent in patients who underwent endoscopic treatment for cholangitis is one of these new techniques to manage cholelithiasis, choledocholithiasis and stent removal in a single procedure through laparoscopy. The second technique is a new indication of a previously described procedure, antegrade balloon papillary dilation to reduce biliary leaks after primary closure of the common bile duct.

Pseudotumor inflamatorio hepático, una entidad de difícil diagnóstico / Inflammatory pseudotumor of the liver: a condition hard to diagnose

RESUMEN El pseudotumor inflamatorio hepático es una lesión muy infrecuente, sin una etiología ni patogenia claras. Su diagnóstico preoperatorio no es habitual pero, en caso de realizarse, puede evitar la cirugía. Presentamos el caso de un paciente joven, con antecedente de lupus cutáneo que, tras debutar con una pancreatitis aguda, presenta episodios de colangitis y cuyos hallazgos radiológicos no permiten descartar la presencia de un colangiocarcinoma, por lo que se realiza hepatectomía izquierda, siendo el diagnóstico histológico final de pseudotumor inflamatorio hepático.

ABSTRACT Inflammatory pseudotumors of the liver are rare and lack clear etiology and pathogenesis. The preoperative diagnosis is seldom made but it avoids unnecessary surgery. We report the case of a young male patient with a history of cutaneous lupus and episodes cholangitis after an acute pancreatitis. As the imaging tests could not rule out cholangiocarcinoma, a left liver resection was performed, and the final histologic diagnosis was inflammatory pseudotumor of the liver.

Incidencia de litiasis vesicular asintomática en pacientes con complicaciones biliares / Incidence of asymptomatic gallstones in patients with biliary complications / Incidência de cálculos biliares asintomáticos em pacientes com complicações biliares

Introducción: Las complicaciones de la litiasis biliar (LB) son una causa importante de morbilidad en nuestro país y en el mundo entero y generan elevados costos en salud. Objetivo: El objetivo de este trabajo fue determinar, que pacientes con una complicación de su patología litiásica de la vía biliar (colecistitis, colangitis aguda, pancreatitis aguda), fueron previamente asintomáticos, resultando dicha complicación el debut de la enfermedad. Lugar: Sanatorio Asociación Española de Socorros Mutuos, Montevideo Uruguay. Diseño: Estudio observacional descriptivo, retrospectivo, análisis de historias clínicas. Materiales y Métodos: Se analizaron 234 casos clínicos. Se constató en este grupo de pacientes, características epidemiológicas, metodología diagnóstica, tratamientos recibidos y complicaciones. Resultados: Del total de pacientes (n=234), 109 (46.6%) tenían una litiasis vesicular asintomática (LVA) y la complicación biliar, fue el debut de su enfermedad. La colecistitis aguda fue la complicación más frecuente (68%), en segundo lugar, la colangitis aguda (22%) y en tercer lugar la pancreatitis aguda (10%). La edad promedio de presentación de la enfermedad fue los 59 años. Conclusiones: Casi la mitad de los pacientes (46.6%) que presentaron una complicación de su litiasis biliar eran asintomáticos. Este sería un argumento importante para indicar la colecistectomía laparoscópica con un criterio profiláctico en pacientes con una LVA.

Introduction : Complications of gallstones are an important cause of morbidity in our country and throughout the world and generate high health costs. Objective: The objective of this study was to determine which patients with a complication of their bile duct stone pathology (cholecystitis, acute cholangitis, acute pancreatitis) were previously asymptomatic, and this complication resulted in the onset of the disease. Place: Sanatorium Asociación Española de Socorros Mutuos, Montevideo Uruguay. Design: Descriptive and retrospective observational study with an analysis of medical records. Materials and Methods: 234 clinical cases were analyzed. Epidemiological characteristics, diagnostic methodology, treatments received, and complications were assessed in this group of patients. Results: Of the total number of patients (n=234), 109 (46.6%) had an asymptomatic gallbladder lithiasis and the biliary complication was the debut of their disease. Acute cholecystitis was the most frequent complication (68%), followed by acute cholangitis (22%) and third by acute pancreatitis (10%). The average age of presentation of the disease was 59 years. Conclusions: Almost half of the patients (46.6%) who presented a complication of their gallstones were asymptomatic. This would be an important argument to indicate laparoscopic cholecystectomy with a prophylactic criterion in patients with asymptomatic gallbladder lithiasis.

Introdução: As complicações dos cálculos biliares são uma importante causa de morbidade em nosso país e em todo o mundo e geram altos custos de saúde. Objetivo: O objetivo deste estudo foi determinar quais pacientes com uma complicação de sua patologia de cálculo do ducto biliar (colecistite, colangite aguda, pancreatite aguda) eram previamente assintomáticos, e essa complicação resultou no aparecimento da doença. Local: Sanatório Asociación Española de Socorros Mutuos, Montevidéu - Uruguai. Desenho: Estudo observacional descritivo, retrospectivo, análise de histórias clínicas. Materiais e Métodos: Foram analisados ​​234 casos clínicos. Características epidemiológicas, metodologia diagnóstica, tratamentos recebidos e complicações foram avaliadas neste grupo de pacientes. Resultados: Do total de pacientes (n=234), 109 (46,6%) apresentavam litíase vesicular assintomática e a complicação biliar foi o início da doença. A colecistite aguda foi a complicação mais frequente (68%), seguida da colangite aguda (22%) e a terceira da pancreatite aguda (10%). A idade média de apresentação da doença foi de 59 anos. Conclusões: Quase metade dos pacientes (46,6%) que apresentaram complicação de seus cálculos biliares eram assintomáticos. Esse seria um argumento importante para indicar a colecistectomia laparoscópica com critério profilático em pacientes com litíase vesicular assintomática.

Colangitis biliar primaria: caracterización de una cohorte retrospectiva / Primary biliary cholangitis: characterization of a retrospective cohort

Introducción. La colangitis biliar primaria (CBP) es una enfermedad hepática crónica de origen autoinmune, caracterizada por inflamación y destrucción progresiva de las células epiteliales de los conductos biliares intralobulillares, que causa de manera secundaria colestasis, fibrosis, cirrosis e insuficiencia hepática. La historia natural de la enfermedad ha cambiado en los últimos años debido a la mejoría en los métodos diagnósticos y terapéuticos. Metodología. Estudio observacional descriptivo de cohorte retrospectivo, en el cual se efectuó la revisión y análisis de las historias clínicas de los pacientes mayores de 16 años con diagnóstico de CBP, atendidos en la Unidad de Hepatología y Trasplante Hepático del Hospital Pablo Tobón Uribe, entre los años 2013 a 2021, con el fin de obtener información sobre las características de esta patología a nivel local. Resultados. Se evaluó un total de 239 pacientes, con un promedio de edad de 61,6±12,31 años, el 97,07% fue del sexo femenino, con criterios serológicos como anticuerpos antimitocondriales (AMA) positivos en un 76,89%, el 66,95% de los pacientes presentaban alguna enfermedad autoinmune concomitante y el 31,60% tuvieron sobreposición con hepatitis autoinmune. La manifestación clínica más frecuente fue el prurito en un 61,92% de los pacientes, seguido por la astenia en un 51,88%. La presencia de hipertensión portal al diagnóstico fue del 29,29%. La colangitis no supurativa y la ductopenia en la biopsia de hígado se documentó en un 43,79% de los casos. El ácido ursodesoxicólico (UDCA) fue la terapia de primera línea en el 100% de los pacientes, se identificó refractariedad del 16,36% según criterios de París II y del 31,79% con los criterios de Toronto. La no respuesta al UDCA, se asoció de manera significativa con mayor mortalidad (p=0,039) y presencia de hepatocarcinoma (p=0,042). Conclusión. Se caracterizó la CBP en nuestra población. El diagnóstico serológico por AMA fue bajo, con altos requerimientos de biopsia hepática en el contexto de síndromes de sobreposición. Los signos de hipertensión portal al momento del diagnóstico fueron prevalentes. La refractariedad bioquímica a la terapiafue descrita en relación con mayor progresión de fibrosis, aumento de mortalidad y presencia de hepatocarcinoma.

ntroduction. Primary biliary cholangitis (PBC) is a chronic liver disease of autoimmune origin, characterized by inflammation and progressive destruction of the epithelial cells of the intralobular bile ducts, causing secondary cholestasis, fibrosis, cirrhosis, and liver failure. The natural history of the disease has changed in recent years due to the improvement in diagnostic and therapeutic methods. Methodology. Cross-sectional descriptive observational study, where the medical records of patients older than 16 years with a diagnosis of PBC, treated at the Hepatology and Liver Transplant Unit of the Pablo Tobón Uribe Hospital, between the years 2013 to 2021, were reviewed and analyzed in order to obtain information on the characteristics of this pathology at a local level. Results. A total of 239 patients were evaluated, with a mean age of 61.6±12.31 years, 97.07% were females, with serological criteria such as positive antimitochondrial antibodies (AMA) in 76.89%. Of all included patients, 66.95% had some concomitant autoimmune disease and 31.60% had an overlap with autoimmune hepatitis. The most frequent clinical manifestation was pruritus in 61.92% of the patients, followed by asthenia in 51.88%. The presence of portal hypertension at diagnosis was 29.29%. Non-suppurative cholangitis and ductopenia on liver biopsy were documented in 43.79% of the cases. Ursodeoxycholic acid (UDCA) was the first line therapy in 100% of patients, 16.36% were refractory to treatment according to the Paris II criteria and 31.79% according to the Toronto criteria. Non-response to UDCA was significantly associated with higher mortality (p=0.039) and presence of hepatocarcinoma (p=0.042). Conclusion. PBC was characterized in our population. Serological diagnosis by AMA was low, with high requirements for liver biopsy in the context of overlap syndromes. Signs of portal hypertension at diagnosis were prevalent. Biochemical refractoriness to therapy was described in relation to greater progression of fibrosis, increased mortality, and the presence of hepatocarcinoma.

Dilataciones quísticas congénitas de las vías biliares (primera parte). Vías biliares intrahepáticas / Congenital bile duct cystic dilatation (part 1). Intrahepatic bile ducts

RESUMEN La enfermedad de Caroli es una enfermedad infrecuente que requiere un alto índice de sospecha para su diagnóstico. Puede afectar un segmento hepático, un lóbulo o todo el hígado; suele generar episodios repetidos de colangitis. Existe una amplia gama de propuestas terapéuticas que oscilan desde el tratamiento médico hasta el trasplante de hígado. En este trabajo presentamos 3 casos, realizamos una revisión de la literatura y proponemos una ampliación de la clasificación de Alonso-Lej modificada por Todani que, a nuestra manera de ver, tiene implicaciones a la hora de seleccionar un tratamiento.

ABSTRACT Caroli's disease is a rare condition, and its diagnosis requires high level of suspicion. The disease may affect one segment, one lobe or the entire liver, and may result in repeated episodes of cholangitis. The disease can be managed using different therapeutic approaches ranging from medical treatment to liver transplantation. In this paper we report 3 cases with review of the literature and propose a modification of the classification by Alonso-Lej modified by Todani which we believe may be useful to guide treatment.

Quiste de colédoco con sintomatología obstructiva en embarazada / Common bile duct cyst with obstructive symptoms in a pregnant woman

Los quistes de colédoco son poco comunes, en los países occidentales se ha reportado una incidencia de 1 por cada 100 mil nacidos vivos. En países asiáticos, esta incidencia es mayor, 1 por 100 nacidos vivos. Son usualmente diagnosticados durante la infancia y en el 25 % de los pacientes se presentan durante su vida adulta. Es inusual que se presenten durante el embarazo, sobre todo, que se manifieste durante el primer trimestre. Se realizó un informe de caso con presentación inusual del cuadro clínico no causal, de una embarazada en su primer trimestre con sintomatología obstructiva, evolucionando a colangitis. se realizan exámenes de gabinete como ultrasonografía abdominal y exámenes de laboratorio revelando obstrucción, además de realizarse colangioresonancia y ultrasonografía endoscópica dando como resultado quiste de colédoco tipo 1C, se realiza drenaje. Posteriormente al alta hospitalaria, la paciente presenta nuevamente cuadro de colangitis, se realiza drenaje endoscópico, sin embargo, presenta aborto

Choledochal cysts are rare, in western countries an incidence of 1 per 100,000 live births has been reported. In Asian countries, this incidence is higher 1 per 100 live births. They are usually diagnosed during childhood and in 25 % of patients they present during their adult life. It is unusual for them to occur during pregnancy, especially during the first trimester. A case report was made with an unusual presentation of the non-causal clinical picture, of a pregnant woman in her first trimester with obstructive symptoms, evolving to cholangitis. Cabinet examinations such as abdominal ultrasonography are performed, resulting in type 1C common bile duct cyst. Drainage is performed After discharge from the hospital, the patient presents again with cholangitis, endoscopic drainage is performed, however, she has abortion

Colangite aguda: da tríade de Charcot aos critérios de Tóquio / Acute cholangitis: from Charcot's triad to Tokyo guidelines

O objetivo deste estudo foi revisar as ferramentas diagnósticas e de manejo da colangite aguda, da tríade de Charcot aos critérios de Tóquio. Foi realizada revisão da literatura no PubMed® utili- zando a combinação dos descritores "Tokyo Guideline", "Char- cot's triad" e "Acute cholangitis". A tríade de Charcot foi a pri- meira ferramenta diagnóstica da colangite aguda. Apesar de sua contribuição, foram os critérios de Tóquio a primeira diretriz com evidência clínica para diagnóstico, classificação de severidade e estratégia para o manejo da colangite aguda. A literatura atual busca identificar marcadores de mau prognóstico do paciente, como a procalcitonina, assim como o tempo ideal para a terapia antibiótica e a drenagem biliar. É bem estabelecido o quanto a tríade de Charcot, juntamente dos critérios de Tóquios, contri- buiu para o melhor diagnóstico e manejo da colangite aguda. Todavia, mais estudos são necessários para validação científica dessas diretrizes na prática clínica-cirúrgica.

The objective of this study was to review acute cholangitis diag- nostic and management tools, from Charcot's triad to Tokyo guidelines. The keywords "Tokyo guideline", "Charcot's triad", and "Acute cholangitis" were searched in PUBMED® and used to review the literature. Charcot's triad was the first diagnostic tool in acute cholangitis. Despite its contribution, Tokyo guidelines were the first evidence-based parameters for diagnosis, severity assessment, and strategy for acute cholangitis management. The current literature searches for biomarkers of patient's bad prognostic, such as procalcitonin, as well the optimal timing of antibiotic therapy and biliary drainage. The good contribution Charcot's triad and Tokyo guidelines has made to improved diagnosis and management of acute cholangitis is well-esta- blished. However, further studies are required for scientific vali- dation of these guidelines in clinical-surgical practice.

Rendimiento diagnóstico de las punciones de lesiones biliopancreáticas guiadas por ultrasonido endoscópico con patólogo en sala / Diagnostic performance of endoscopic ultrasound-guided fine needle aspiration of pancreatobiliary lesions with rapid on-site evaluation

Resumen Introducción y objetivos: El ultrasonido endoscópico con punción-aspiración con aguja fina (USE-PAAF) en lesiones neoplásicas biliopancreáticas suele tener un rendimiento alto, que depende de características de la lesión; aspectos técnicos de la USE-PAAF y la experiencia del endoscopista. De los factores menos estudiados es la presencia de patólogo en sala. Se plantea la realización de USE-PAAF con patólogo en sala para disminuir el número de pases, la tasa de muestras inadecuadas y la necesidad de repetir el procedimiento. Material y métodos: Estudio observacional, retrospectivo, con recolección prospectiva de enero de 2018 a junio de 2019, en pacientes adultos sometidos a USE-PAAF. Las muestras obtenidas fueron extendidas y evaluadas en salas de endoscopia por médico patólogo con coloración Diff-Quick y cuando se obtenía una muestra suficiente se enviaba en frasco con formol para bloque celular o biopsias. Resultados: Se realizaron 48 USE-PAAF biliopancreáticas en individuos con una edad mediana de 64 años. Las indicaciones más frecuentes fueron punciones por masa o pseudomasa pancreática (71 % de casos); Se diagnosticaron 35 malignidades (77 % correspondientes a adenocarcinoma, y 14 % a tumores neuroendocrinos). La mediana de tamaño de lesiones fue de 28 mm; el número de pases promedio fue de 3. Se obtuvieron resultados diagnósticos en 89 % frente a 11 % de falsos negativos. Se presentó 1 complicación menor (2,1 %), que fue dolor abdominal. Conclusiones: La USE-PAAF con patólogo en sala tiene alto rendimiento diagnóstico, con escasos resultados falsos negativos. Se requiere una mediana de pases menor, que podría minimizar los riesgos del procedimiento y la necesidad de repetir la prueba.

Abstract Introduction: Endoscopic ultrasound with fine-needle aspiration (EUS-FNA) of pancreatobiliary neoplastic lesions usually has a high performance that depends on the characteristics of the lesion, technical aspects, and expertise of the endoscopist. One of the least studied factors is rapid on-site evaluation with a pathologist in the room. Objective: To perform EUS-FNA with a pathologist in the endoscopy room to reduce the number of passes, the rate of inadequate samples and the need to repeat the procedure. Material and methods: Observational retrospective study with a prospective data collection approach from January 2018 to June 2019 of adult patients undergoing EUS-FNA. The samples obtained were spread and evaluated in endoscopy rooms by a pathologist with Diff-Quick stain, and when a sufficient sample was obtained, it was sent in a vial with formalin for cell block and/or biopsy. Results: 48 pancreatobiliary EUS-FNA were performed in individuals with a median age of 64 years. The most frequent indication was aspiration due to pancreatic mass (71%); 35 malignancies were diagnosed (77% were adenocarcinomas and 14% were neuroendocrine tumors). The median size of the lesions was 28mm, and the average number of passes was 3. Diagnostic results were obtained in 89% vs. 11% of false negatives. There was 1 minor complication (2.1%), which was abdominal pain. Conclusions: EUS-FNA with an in-room pathologist has a high diagnostic performance, with few false negative results. Also, a lower median number of passes is required, minimizing the risks of the procedure and the need for repeating it.

Obstruccion intestinal por Íleo biliar: reporte de un caso / Biliary Ileus intestinal obstruction. a case report

El ileo biliar es una rara complicación de la colelitiasis no tratada. Presentamos el cuadro clínico y el manejo de un paciente masculino de 72 años (AU)

The biliary or Gallstone ileus is a rare complication of the non-treated Cholelithiasis. We present the clinical features and the treatment of a 72 years old patient

Litiasis del hepatocolédoco / Common bile duct stones

RESUMEN Anteriormente, cuando se diagnosticaba litiasis en la vía biliar, el procedimiento consistía en una co lecistectomía, coledocotomía, extracción de los cálculos y colocación de un drenaje de Kehr. En otros casos se podía hacer papiloesfinteroplastia o una derivación biliodigestiva. Actualmente tenemos mu chas herramientas diagnósticas y terapéuticas como la colangiorresonancia, la pancreatocolangio grafía retrógrada endoscópica, la cirugía laparoscópica de la vía biliar, la ecoendoscopia y la ecografía intraoperatoria. Los procesos de decisiones son más complejos y sin un sustento con evidencia con cluyente. Tenemos estudios que enfocan parceladamente el tema, por lo que, dependiendo de si el diagnóstico se hace antes o durante la colecistectomía laparoscópica, el cirujano empleará su sentido común individualizando cada caso. El manejo ideal de la litiasis de la vía biliar sigue siendo motivo de controversia. Decidir por un manejo endoscópico, laparoscópico o convencional requiere logística, entrenamiento y juicio clínico adecua dos. La cirugía convencional sigue siendo una opción vigente.

ABSTRACT Previously, when a surgeon diagnosed bile duct lithiasis, he/she performed cholecystectomy, chole docotomy, stone removal and placement of a Kehr's "T" tube. Some cases might require sphinctero plasty or bilio-digestive bypass. Nowadays, magnetic resonance cholangiopancreatography, endosco pic retrograde cholangiopancreatography, endoscopic ultrasound and intraoperative ultrasound have emerged as diagnostic and therapeutic tools. Decision-making processes are complex and there is no conclusive evidence supporting them. Many studies have focused on the matter with a non-compre hensive approach so that each surgeon will use his/her common sense for each individual case. The optimal management of the common bile duct is still controversial. Deciding on endoscopic, lapa roscopic or conventional management requires adequate training and clinical judgment. Conventional surgery is still in valid option.

Ascaridiosis en la vía biliar intrahepática: a propósito de un caso pediátrico / Ascariasis in the intrahepatic biliary duct: about a case in pediatrics

El 25 % de la población mundial se encuentra infectada por Ascaris lumbricoides. La ascaridiosis hepatobiliar ocurre en zonas con alta endemicidad y gran carga parasitaria, y genera desde intensa inflamación hasta fibrosis. Se presenta a un paciente de 2 años, que consultó por distensión abdominal y tos de 1 mes de evolución asociada a fiebre en las últimas 72 h. Se realizó una ecografía abdominal que evidenció áscaris en la vía biliar, en el estómago y en el intestino delgado, y una radiografía de tórax con infiltrado inflamatorio intersticial, asociado a hiperleucocitosis con hipereosinofilia y gamma-glutamiltranspeptidasa elevada. Se administró un tratamiento antibiótico, antihelmíntico, sin lograr la eliminación de los parásitos de la vía biliar, por lo que se requirió su extracción mediante colangiografía percutánea

Twenty five percent of the world population is affected by Ascaris lumbricoides. Hepatobiliary ascariasis occurs in areas with high endemicity and great amount of parasitic load, generating intense inflammation to fibrosis. We report a two-year-old patient that consults about abdominal distension and cough of one month of evolution associated with 72 hours of fever. Abdominal ultrasound is performed, which shows bile duct, stomach, small intestine with ascaris and chest x-ray with interstitial inflammatory infiltrate, associated with hyperleukocytosis with hypereosinophilia and elevated gamma-glutamyl transpeptidase. Antibiotic, anthelminthic treatment is administered, without achieving the elimination of the bile duct parasites, requiring their removal by percutaneous cholangiography.

Epidemiological profile, referral routes and diagnostic accuracy of cases of acute cholangitis among individuals with obstructive jaundice admitted to a tertiary-level university hospital: a cross-sectional study

ABSTRACT BACKGROUND: Obstructive jaundice may lead to ominous complications and requires complex diagnostic evaluations and therapies that are not widely available. OBJECTIVE: To analyze the epidemiological profile, referral routes and diagnostic accuracy at admittance of cases of acute cholangitis among patients with obstructive jaundice treated at a referral unit. DESIGN AND SETTING: Cross-sectional study at a tertiary-level university hospital. METHODS: Patients with obstructive jaundice who were treated by means of endoscopic retrograde cholangiopancreatography, resection and/or surgical biliary drainage were evaluated. The main variables analyzed were epidemiological data, referral route, bilirubin levels and time elapsed between symptom onset and admittance and diagnosing of acute cholangitis at the referral unit. The accuracy of the clinical diagnosis of acute cholangitis was compared with a retrospective analysis on the medical records in accordance with the Tokyo criteria. RESULTS: Female patients predominated (58%), with an average age of 56 years. Acute cholangitis was detected in 9.9% of the individuals; application of the Tokyo criteria showed that the real prevalence was approximately 43%. The main referral route was direct contact (31.8%) and emergency care (29.7%); routing via official referral through the public healthcare system accounted for 17.6%, and internal referral from other specialties, 20%. The direct route with unofficial referral was the most important route for cases of neoplastic etiology (P < 0.01) and was the fastest route (P < 0.01). CONCLUSIONS: There is a deficiency in the official referral routes for patients with obstructive jaundice. The accuracy of the clinical diagnosis of acute cholangitis was poor. Wider dissemination of the Tokyo criteria is essential.

Biliary tract infections and their Microbiological Spectrum- A study from coastal region of Southern India / Infecciones de vías biliares y su espectro microbiologico- Un estudio en región de la costa del sur de la India

Objective: Biliary tract infections include cholangitis and cholecystitis. They are associated with high morbidity and mortality in elderly patients with co-morbid disease. The present study was undertaken to determine the microbial aetiology causing biliary tract infections and also to study their antimicrobial resistance profile. Materials & methods: A retrospective study was conducted from January 2011 to December 2016 at the Enteric Diseases Division, Kasturba Medical College Hospital, Manipal. Patients with biliary tract infections admitted in tertiary referral health care hospital, Manipal were included for the study. Aerobic and anaerobic bacteriological and fungal aetiology of biliary tract infections were recorded along with their antimicrobial resistance profile. Results: Out of 307 bile samples sent for aerobic culture and susceptibly testing 187 (60.91%) were positive for culture, of which Escherichia coli (44.4%) was the predominant aetiology followed by Klebsiella pneumoniae (27.3%). Among the 14 samples sent for anaerobic culture, 5 (35.75%) specimens showed growth, of which Bacteroides fragilis group was found to be the predominant anaerobe. Among the 201 bacterial pathogens tested for their antimicrobial susceptibility, 108 (53.73%) isolates were resistant, out of which 9 were PDR Enterobacteriaceae with 12 ESBL strains. All the Candida species were susceptible to fluconazole with the exception of C. glabrata and C. krusei. All the anaerobic isolates were found to be susceptible to Metronidazole. Conclusions: The high rate of bacterial infection particularly gram-negative bacteria was recorded. It is necessary that antimicrobial therapy be initiated when culture or the clinical conditions reports caution. Routine aerobic and anaerobic culturing of bile samples with biliary tract infections are imperatively necessary. With the emergence of multidrug resistant pathogens and change in the microbiological spectrum of biliary tract infections, there is a need for the empirical antimicrobial therapy in every clinical setting.

Objectivo: Las infecciones del tracto biliar incluyen colangitis y colecistitis. Se asocian a gran mortalidad y morbildiad en pacientes ancianos y con comorbilidad. El presente studio se hizo para detemrianr la etiologia microbiana que produce infecciones biliares y para estudiar su perfil de resistencia antimicrobiana. Materiales & metodos: Se hizo un studio retrospectivo entre los meses de Enero 2011 a Diciembre de 2016 en la "Enteric Diseases Division, Kasturba Medical College Hospital, Manipal" en India. Los pacientes con infección de vías biliares admitidos al centro de atención de tercer nivel se incluyeron en el estudio. Se buscaron bacterias aerobicas y anaerobicas y etiologia fungica y se analizó su perfil de resistencia antibiotica. Resultados: De 307 muestras de bilis enviadas para cultivo aerobico y antibiograma, 187 (60.91%) crecieron en el medio de cultivo, predominando Escherichia coli (44.4%) seguida por Klebsiella pneumoniae (27.3%). Entre las 14 muestras analizadas en medio anaerobio, 5 (35.75%) mostraron crecimiento de Bacteroides fragilis. Entre 201 bacterias probadas por antibiograma, 108 (53.73%) tuvieron perfil de resistencia, de los cuales 9 fueron PDR Enterobacteriaceae con 12 cepas ESBL. Todas las especies de Candida fueron susceptibles al fluconazol con la excepción de C. glabrata y C. krusei. Todos los aislados anaerobios fueron susceptibles al Metronidazol. Conclusiones: Se encontró una alta tasa de infección bacteriana con predominio de gram-negativos. Se hace necesario iniciar terapia antimicrobiana cuando lo sugieren las condiciones clínicas o el resultado del cultivo. El cultivo rutinario de bilis es imperioso. Dado el aumento de patógenos multirresistentes se requiere inicio empírico inmediato

Polimorfismos do gene HLA-DRB1 em pacientes pediátricos com hepatite autoimune tipo 1 e hepatite autoimune associada à colangite autoimune / Hla-drb1 gene polymorphisms in pediatric patients with type 1 autoimmune hepatitis and type 1 autoimmune hepatitis overlap syndrome with autoimmune cholangitis

ABSTRACT BACKGROUND: Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease associated with a loss of immunological tolerance to self-antigens. Susceptibility to AIH is partially determined by the presence of genes related to human leukocyte antigen (HLA), mainly allelic variants of DRB1. OBJECTIVE: The purpose of this study was to investigate the frequencies of the polymorphisms in HLA-DRB1 gene in children and adolescents with type 1 AIH and type 1 AIH overlap syndrome with autoimmune cholangitis (overlap syndrome, OS) in comparison to healthy sex and age-matched individuals (control group). METHODS: This is a cross-sectional study of 25 pediatric patients diagnosed with type 1 AIH and 18 with OS. Fifty-seven healthy individuals were included as controls. The polymorphisms of the HLA-DRB1 gene were evaluated by PCR and included HLA-DRB1*03, HLA-DRB1*04, HLA-DRB1*07, and HLA-DRB1*13. RESULTS: Our results showed that the presence of the allele HLA-DRB1*13 increased the chance of autoimmune cholangitis (OR=3.96, CI 1.07 to 14.61, P=0.04). The HLA-DRB1*04 and HLA- DRB1*07 have no association with the AIH and autoimmune cholangitis in a young sample. CONCLUSION: This work demonstrates an association of the main polymorphisms in the HLA-DRB1 gene to AIH with or without cholangitis in a Brazilian sample.

RESUMO CONTEXTO: Hepatite autoimune (HAI) é uma doença hepática inflamatória crônica, rara, associada à perda da tolerância imunológica aos auto-antígenos. A susceptibilidade à HAI é parcialmente determinada pela presença de genes relacionados ao antígeno leucocitário humano (HLA), principalmente variantes alélicas do DRB1. OBJETIVO: O objetivo deste estudo foi investigar a frequência de polimorfismos no gene HLA-DRB1 em crianças e adolescentes com HAI tipo 1 e HAI tipo 1 associada à colangite autoimune, em comparação com indivíduos saudáveis pareados por sexo e idade (grupo controle). MÉTODOS: Este é um estudo transversal de 25 pacientes pediátricos com diagnóstico de HAI tipo 1 e 18 com HAI associada à colangite autoimune. Cinquenta e sete indivíduos saudáveis foram incluídos como controles. Os polimorfismos do gene HLA-DRB1 foram avaliados por PCR e incluíram HLA-DRB1*03, HLA-DRB1*04, HLA-DRB1*07 e HLA-DRB1*13. RESULTADOS: Nossos resultados mostraram que a presença do alelo HLA-DRB1*13 aumentou a chance de colangite autoimune (OR=3,96; IC 1,07 a 14,61; P=0,04). O HLA-DRB1*04 e o HLA-DRB1*07 não apresentam associação com a HAI e colangite autoimune no grupo de pacientes mais jovens. CONCLUSÃO: Este trabalho demonstra uma associação dos principais polimorfismos no gene HLA-DRB1 à HAI com ou sem colangite na população brasileira.

Artritis reumatoidea asociada a colangitis biliar primaria bajo tratamiento con medicamentos biológicos. Reporte de 4 casos y puesta al día / Rheumatoid arthritis associated with primary biliary cholangitis under treatment with biological drugs. Report of 4 cases and update

El abordaje terapéutico de pacientes con dos o más enfermedades autoinmunes es un verdadero desafío, especialmente cuando el tratamiento enfocado en una de ellas podría precipitar la progresión de la otra. Si bien la asociación de artritis reumatoidea (AR) con colangitis biliar primaria (CBP) no es tan frecuente, cuando coexisten, la utilización de metotrexato u otras drogas hepatotóxicas debe decidirse con cautela. Con la indicación más generalizada de las drogas biológicas modificadoras del curso de la AR (DMARb) han aparecido algunos reportes de pacientes con AR y CBP tratados con etanercept, infliximab, rituximab, tocilizumab y abatacept. Presentamos una serie de casos de 4 pacientes con AR y CBP que fueron tratados con DMARb. Nuestro reporte sería el primero en describir dos casos con golimumab para controlar la AR y el segundo en reportar un caso con adalimumab y otro con abatacept. Con rituximab, ya existen tres casos publicados. En ninguno de los pacientes de nuestra serie empeoraron los síntomas de CBP y, al contrario, en dos de ellos hubo mejoría de los parámetros bioquímicos. En conclusión, según lo observado y lo reportado en la literatura, el uso DMARb podría ser considerado en el caso de pacientes con AR activa que además padecen CBP

The therapeutic approach of patients with two or more autoimmune diseases is quite a challenge, especially when the treatment of one of them, can precipitate the progression of the other. Even though the association of rheumatoid arthritis (RA) and primary biliary cholangitis (PBC) is rare; when both coexist, the use of methotrexate and other hepatotoxic drugs should be used with caution. With a most widespread indication of biologic diseasemodifying antirheumatic drugs (bDMARDs) some reports of patients with RA and PBC treated with etanercept, infliximab, rituximab, tocilizumab and abatacept have been published. We report a case series that includes 4 patients with RA and PBC treated with bDMARDs. This is the first report to describe two cases in which golimumab was used to control RA and the second to report patients who received adalimumab and abatacept. Three cases of patients treated with rituximab have been published to date. None of the patients of our report suffered a progression of their PBC; matter in fact, two of them showed an improvement in their biochemical parameters. PBC symptoms did not get worse in any of the patients. On the contrary, laboratory parameters improved in two of the four patients. In conclusion, according to the literature reviewed and to our findings, the use of bDMARDs could be considered in RA patients with concomitant PBC

Hidrotórax hepático en paciente con cirrosis por colangitis esclerosante primaria / Hepatic hydrothorax in a patient with cirrhosis due to primary sclerosing cholangitis

Se presenta el caso de un paciente de sexo masculino de 39 años, portador de colangitis esclerosante primaria desde hace 9 años, complicado con síndrome de hipertensión portal más insuficiencia hepática, en plan de trasplante hepático, en seguimiento por el Servicio de Gastroenterología. Ingresa por dificultad respiratoria, constatándose derrame pleural izquierdo extenso con características de trasudado con ascitis moderada, por lo que se procede al drenaje del líquido pleural y ascítico con goteo concomitante de albúmina endovenosa. El paciente presenta buena tolerancia y disminución importante tanto del derrame pleural como del líquido ascítico. Es dado de alta en mejores condiciones y con aumento de dosis de fármacos para el tratamiento de la ascitis

We present the case of a male patient 39 years old, with a history of primary sclerosing cholangitis for 9 years, complicated with portal hypertension and liver failure, he is in liver transplant program, followed up by Gastroenterology Service. He was admitted for respiratory failure, and massive left pleural effusion, with of features transudate and moderate ascites was noted aswell, we performes pleural and ascitic fluid drainage with concomitant infusion of intravenous albumin. The patient shows good tolerance and significant decrease in both pleural effusion and ascitic fluid. He was discharged in better condition and with increased doses of drugs for the treatment of ascites