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

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.

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.

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.

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.

Sialoadenitis esclerosante asociada a enfermedad por IgG4: reporte de un caso / Sclerosing sialadenitis associated with IgG4 disease: case report

Las enfermedades relacionadas con IgG4 (ER-IgG4) son entidades fibroinflamatorias e inmunomediadas, caracterizadas por la afección multiorgánica, con la formación de pseudotumores que provocan lesión tisular y daño orgánico subsecuente. Se describe el caso de un paciente de 43 años que presentó sialoadenitis esclerosante y cumplió todos los criterios diagnósticos de enfermedad relacionada con IgG4.

IgG4-related diseases (IgG4-RD) are fibroinflammatory immune-mediated entities characterized by multiorgan involvement with the development of pseudotumors that cause tissue injury and subsequent organ damage. We describe the case of a 43-year-old man who presented sclerosing sialadenitis and fulfilled the diagnostic criteria for IgG4-related disease.

Peritonitis encapsulante esclerosante (síndrome de cocoon): una causa extremadamente rara de obstrucción intestinal. Reporte de un caso y revisión de la literatura / Cocoon syndrome: an extremely rare cause of bowel obstruction. Case report and literature review

La peritonitis esclerosante encapsulante o síndrome de cocoon es una causa rara de obstrucción intestinal, que puede dividirse en pri-maria o secundaria, producida por una membrana fibrocolágena que envuelve el intestino delgado. El diagnóstico es particularmente desafiante, al tratarse de una entidad cuya clínica es inespecífica. El conocimiento de los hallazgos imagenológicos, sumados a una correcta anamnesis, permite una adecuada valoración preoperatoria. Presentamos un caso de interés y de difícil diagnóstico, junto con una revisión de la literatura sobre el tema.

Sclerosing encapsulating peritonitis or cocoon syndrome is a rare cause of intestinal obstruction, that can be divided into primary or secondary. It is produced by a fibro collagenous membrane that surrounds the small bowel. The diagnosis is particularly challenging since it is an entity whose symptoms are nonspecific. Knowledge of the imaging findings, in addition to a well performed anamnesis allows an adequate preoperative assessment. We present a clinical case and a review of the literature.

Sclerosing odontogenic carcinoma — review of all published cases: is it a justifiable addition as a malignancy? / Carcinoma odontogênico esclerosante - revisão de todos os casos publicados: sua classificação como tumor maligno é justificável?

Abstract Introduction Sclerosing odontogenic carcinoma was a new addition to the list of head and neck tumors by World Health Organization in 2017. This lesion has scarcely been reported and a lack of pathognomonic markers for diagnosis exists. Objective The aim of the study was to summarize findings from the available literature to provide up-to-date information on sclerosing odontogenic carcinoma and to analyse clinical, radiological, and histopathological features to obtain information for and against as an odontogenic malignancy. Methods We conducted a comprehensive review of literature by searching Pubmed, EBSCO and Web of Science databases, according to PRISMA guidelines. All the cases reported as sclerosing odontogenic carcinoma in English were included. Data retrieved from the articles were gender, age, clinical features, site, relevant medical history, radiographical findings, histopathological findings, immunohistochemical findings, treatments provided and prognosis. Results Mean age at diagnosis of sclerosing odontogenic carcinoma was 54.4 years with a very slight female predilection. Sclerosing odontogenic carcinoma was commonly reported in the mandible as an expansile swelling which can be asymptomatic or associated with pain or paraesthesia. They appeared radiolucent with cortical resorption in radiograph evaluation. Histologically, sclerosing odontogenic carcinoma was composed of epithelioid cells in dense, fibrous, or sclerotic stroma with equivocal perineural invasion. Mild cellular atypia and inconspicuous mitotic activity were observed. There is no specific immunohistochemical marker for sclerosing odontogenic carcinoma. AE1/AE3, CK 5/6, CK 14, CK19, p63 and E-cadherin were the widely expressed markers for sclerosing odontogenic carcinoma. Surgical resection was the main treatment provided with no recurrence in most cases. No cases of metastasis were reported. Conclusion From the literature available, sclerosing odontogenic carcinoma is justifiable as a malignant tumor with no or unknown metastatic potential which can be adequately treated with surgical resection. However, there is insufficient evidence for histological grading or degree of malignancy of this tumor.

Resumo Introdução O carcinoma odontogênico esclerosante é a nova adição à lista de tumores de cabeça e pescoço da Organização Mundial da Saúde em 2017. Essa lesão é pouco relatada e não há marcadores patognomônicos para o diagnóstico. Objetivo Resumir os achados da literatura disponível para fornecer informações atualizadas sobre o carcinoma odontogênico esclerosante e analisar as características clínicas, radiológicas e histopatológicas a favor e contra sua classificação como uma lesão odontogênica maligna. Método Uma revisão abrangente da literatura foi feita nos bancos de dados Pubmed, Ebsco e Web of Science, de acordo com as diretrizes do Prisma. Todos os casos relatados em inglês como carcinoma odontogênico esclerosante foram incluídos. Os dados recuperados dos artigos foram sexo, idade, características clínicas, sítio do tumor, histórico médico relevante, achados radiográficos, achados histopatológicos, achados imuno-histoquímico, tratamentos instituídos e prognóstico. Resultados A média de idade ao diagnóstico de carcinoma odontogênico esclerosante foi de 54,4 anos, com uma predileção muito leve pelo sexo feminino. Tumores do tipo carcinoma odontogênico esclerosante foram comumente relatados na mandíbula como um edema expansivo, que pode ser assintomático ou associado a dor ou parestesia. Eles têm aparência radiolucente com reabsorção cortical na radiografia. Histologicamente, o carcinoma odontogênico esclerosante é composto por células epitelioides em estroma denso, fibroso ou esclerótico com invasão perineural ambígua. Atipia celular leve e atividade mitótica imperceptível foram observadas. Não há um marcador imuno-histoquímico específico para SOC. AE1/AE3, CK 5/6, CK 14, CK19, p63 e E-caderina foram os marcadores amplamente expressos para carcinoma odontogênico esclerosante. A ressecção foi o principal tratamento feito sem recorrência na maioria dos casos. Nenhum caso de metástase foi relatado. Conclusão De acordo com a literatura disponível, é justificável classificar o carcinoma odontogênico esclerosante como um tumor maligno com nenhum ou desconhecido potencial metastático, que pode ser tratado adequadamente com ressecção cirúrgica. Entretanto, não há evidências suficientes para a graduação histológica ou de malignidade desse tumor.

Colangiopatía post-COVID-19 grave: reporte de 6 casos / Post-COVID-19 severe cholangiopathy: report of 6 cases

RESUMEN La enfermedad por coronavirus de 2019 (COVID-19) es una infección predominantemente del tracto respiratorio con la capacidad de afectar otros órganos. Las alteraciones en las pruebas hepáticas son una manifestación frecuente de la COVID-19 pero suelen ser transitorias. Describimos el curso clínico y los hallazgos más relevantes de 6 pacientes que desarrollaron una colangiopatía tras una COVID-19 grave. La edad promedio de los pacientes, 4 hombres y dos mujeres, fue de 56 años y el tiempo promedio desde el diagnóstico de COVID-19 hasta el diagnóstico de la colangiopatía fue de 138 días. Las características más importantes fueron la elevación de la fosfatasa alcalina y la desestructuración y el arrosariamiento de la vía biliar intrahepática en las imágenes de resonancia magnética. La colangiopatía tras una COVID-19 grave constituye una nueva entidad con características únicas con el potencial para la lesión progresiva biliar y la cirrosis biliar secundaria. Se requieren más estudios para entender esta enfermedad.

ABSTRACT Coronavirus disease 2019 (COVID-19) is a predominantly respiratory tract infection with the capacity to affect other organs. Liver chemistry abnormalities are a frequent manifestation of COVID-19 but are usually transient. We describe the clinical course and most relevant findings of 6 patients who developed a cholangiopathy after severe COVID-19. The mean age of the patients, 4 men and 2 women, was 56 years and the mean time from COVID-19 diagnosis to diagnosis of cholangiopathy was 138 days. The features most important were the increase of alkaline phosphatase and destructuring and beading of the intrahepatic bile duct in magnetic resonance imaging. Cholangiopathy after severe COVID-19 constitutes a novel entity with unique features and potential for progressive biliary injury and secondary biliary cirrhosis. Further studies are required to understand this disease.

Displasia ósea esclerosante en paciente adulto sin diagnóstico previo: presentación de un caso / Bone sclerosing dysplasia in an adult patient without previous diagnosis: a case report

La displasia ósea esclerosante es una afectación en el desarrollo intrínseco del esqueleto, por alteración en la formación y modelado del hueso, que lleva a una excesiva acumulación ósea con un aumento de la densidad (esclero-sis). Existen varios tipos y todos ellos son de origen genético. Presentamos el caso de una paciente de 37 años que llega a la consulta sin diagnóstico previo, por dolor en miembros inferiores de larga evolución con reagudizaciones, asociado a deformidad e impotencia funcional, que cedía parcialmente con analgésicos comunes. (AU)

Bone sclerosing dysplasia is an affectation of the intrinsic development of the skeleton by an alteration in bone formation and modeling. It causes excessive bone accumulation with an increase in density (sclerosis). There are several types of bone sclerosing dysplasia. They are of genetic origin. We report here a 37 year-old patient without a previous diagnosis of sclerosing bone dysplasia who was seen in the clinic for pain in the lower limbs associated with bone deformity with only partial response to analgesics. (AU)

Características epidemiológicas y clínicas de pacientes con cirrosis hepática

RESUMEN Introducción: La cirrosis hepática constituye la etapa final de muchas enfermedades del hígado. Objetivo: Determinar las características epidemiológicas y clínicas de pacientes con cirrosis hepática. Métodos: Se realizó una investigación descriptiva, retrospectiva, con datos de archivo, de 57 pacientes cirróticos. Se excluyeron aquellos con datos insuficientes en la historia clínica. Las variables analizadas fueron: edad, sexo, etiología, modo de diagnóstico, comorbilidades, manifestaciones endoscópicas de la hipertensión portal, complicaciones, estadios de la enfermedad según D'Amico y clasificación de Child-Pugh. Para el análisis de los resultados se emplearon técnicas de la estadística descriptiva. Resultados: Hubo 19 pacientes (33,3 %) en el grupo de edad entre 60 y 69 años; 31 (54,4 %) mujeres y 26 (45,6 %) hombres. El virus de la hepatitis C fue encontrado en 21 pacientes (36,8 %). El 96,4 % de los enfermos se diagnosticaron mediante ecografía abdominal; 12 (21,1 %) presentaron ascitis y 38 (66,6 %) manifestaciones endoscópicas de hipertensión portal. En estadio 4 de D'Amico estaban 20 (35 %) enfermos y 26 (45,6 %) en estadio de Child-Pugh A; 24 (42,1 %) en Child-Pugh B y 7 (12,3 %) en Child-Pugh C. Conclusiones: La cirrosis hepática predomina en la séptima década de la vida, en el sexo femenino. Prevalece el ultrasonido abdominal como modo de diagnóstico. Las causas más frecuentes son el virus de hepatitis C y el alcoholismo. La ascitis es la complicación que más se presenta. La mayoría de los pacientes muestran signos de hipertensión portal. Predominan las formas no compensadas de la enfermedad.

ABSTRACT Introduction: Liver cirrhosis is the end stage of many liver diseases. Objective: To determine the epidemiological and clinical characteristics of patients with liver cirrhosis. Results: There were 19 patients (33.3%) in the age group between 60 and 69 years; 31 (54,4 %) women and 26 (45,6 %) men. Hepatitis C virus was found in 21 patients (36,8 %). 96,4 % of the patients were diagnosed by abdominal ultrasound; 12 (21,1 %) presented ascites and 38 (66,6 %) endoscopic manifestations of portal hypertension. In D'Amico stage 4 there were 20 (35 %) patients and 26 (45,6 %) in Child-Pugh A stage; 24 (42,1 %) in Child-Pugh B and 7 (12,3 %) in Child-Pugh C. Conclusions: Liver cirrhosis predominates in the seventh decade of life, in females. Abdominal ultrasound prevails as a diagnostic mode. The most common causes are hepatitis C virus and alcoholism. Ascites is the most common complication. Most patients present with signs of portal hypertension. Uncompensated forms of the disease predominate.

Lipogranuloma Esclerosante Escrotal Primario. Reporte de caso y revisión de la literatura / Primary Scrotal Sclerosing Lipogranuloma. Case report and literature review

RESUMEN El lipogranuloma esclerosante es una condición extraña y benigna que puede afectar cualquier órgano, especialmente los genitales externos masculinos. Se suele presentar como masas subcutáneas en escroto, periné y pene. Aunque la mayoría de los casos son secundarios a aplicación de cuerpos extraños como parafina, vaselina o silicona con propósitos estéticos para aumentar el tamaño del pene, también puede deberse a degeneración lipídica endógena, secundaria a trauma, infecciones o reacciones alérgicas. No existe consenso en cuanto a su manejo ni datos sobre su prevalencia al ser una entidad poco reportada; se ha descrito el manejo con ciclos cortos de corticoides sistémicos, y cirugía en los casos recidivantes. El objetivo de este trabajo es reportar el caso de un paciente que acudió con induración y eritema en pene y escroto, quien negaba la aplicación de sustancias exógenas y que fue llevado a biopsia de la lesión, con diagnóstico de lipogranuloma esclerosante. MÉD.UIS.2021;34(2): 97-102.

ABSTRACT Sclerosing lipogranuloma is a noncommon and benign disease that could affect any system in the body, especially the male external genitalia. It is usually presented as a subcutaneous mass in scrotum, perineum and penis. Although, most cases are secondary to the injection of foreign bodies such as paraffin, petrolatum or silicone for cosmetic purposes to increase penis size, it could also be due to endogenous lipid degeneration, secondary to trauma, infections or allergic reactions. There is no unanimity regarding its management or data on its prevalence as it is a poorly reported entity. Management with short cycles of systemic corticosteroids and surgery in relapsing cases have been described. The purpose of this article is to present a case of a patient with induration and erythema in penis and scrotum, who denied the application of exogenous substances and has a reported biopsy of the lesion with diagnosis of sclerosing lipogranuloma. MÉD.UIS.2021;34(2): 97-102.

Colelitíase sintomática como apresentação de colangite esclerosante primária na infância - série de casos e revisão de literatura / Symptomatic cholelithiasis as the presentation of pediatric primary sclerosing cholangitis - case series and literature review

ABSTRACT BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.

RESUMO CONTEXTO: A colangite esclerosante primária (CEP) é uma doença hepatobiliar rara, cuja etiologia ainda não está totalmente elucidada. Dada a raridade do CEP na infância, até a recente publicação de uma colaboração multicêntrica internacional, mesmo dados sobre suas características e história natural eram escassos. A colelitíase sintomática não foi relatada anteriormente como a apresentação inicial de CEP na infância. OBJETIVO: O objetivo deste estudo foi o diagnóstico de CEP após a apresentação inicial incomum com colelitíase sintomática, que seguiu um curso clínico atípico que não poderia ser explicado apenas pela colelitíase. Também foi realizada uma revisão da literatura. MÉTODOS: Foi realizada uma revisão retrospectiva dos prontuários de três pacientes, que foram diagnosticados e/ou acompanhados no Hospital das Clínicas da Universidade Estadual de Campinas - São Paulo / Brasil, entre 2014 e 2020. Os dados analisados incluíram sexo, idade de apresentação, história médica pregressa, achados de imagem, resultados laboratoriais, avaliação endoscópica, resposta à terapia médica e acompanhamento. RESULTADOS: A idade no momento da apresentação da colelitíase variou de 10 a 12 anos. Em dois dos casos relatados, um início mais subagudo dos sintomas precedeu o episódio de colelitíase. Dois pacientes foram tratados com colecistectomia, não seguida de qualquer complicação cirúrgica, e um paciente foi tratado de forma conservadora. Biópsia hepática percutânea foi realizada em todos os três casos, mostrando achados histológicos compatíveis com CEP. Doença inflamatória intestinal associada não foi observada em nenhum dos pacientes. Os pacientes foram acompanhados por um tempo médio de 3,4 anos. CONCLUSÃO: CEP e colelitíase são raras na população pediátrica. Este estudo relata a colelitíase sintomática como uma apresentação de CEP e levanta a importância da suspeita de doença hepatobiliar subjacente em crianças com colelitíase sem quaisquer fatores predisponentes conhecidos e/ou que seguem um curso clínico atípico.

Enfermedad de Crohn con compromiso extenso y manifestaciones extradigestivas inusuales: a propósito de un caso / Crohn's disease with extensive involvement and rare extraintestinal manifestations: A case report

Resumen La enfermedad de Crohn (EC) es considerada una entidad inmunológicamente mediada que compromete el tracto digestivo. Su compromiso suele ser transmural y puede afectar cualquier parte del tubo digestivo, desde la cavidad oral hasta el ano. Aunque se sabe que su extensión es variable, es poco habitual ver un compromiso extenso y multiorgánico. Se presenta el caso de un paciente joven, quien debutó con síntomas pulmonares asociados con la EC y años más tarde se presentaron los síntomas digestivos y de la vía biliar. El tratamiento se basó en la terapia con anticuerpos contra el factor de necrosis tumoral alfa (TNF-α), con lo cual se obtuvo una respuesta clínica satisfactoria. La relevancia clínica de este caso es la forma de presentación clínica tan florida, tanto por el compromiso gastrointestinal extenso como por las manifestaciones extradigestivas tan infrecuentes.

Abstract Crohn's disease (CD) is considered an immunologically mediated entity that involves the digestive tract. It is characterized by transmural inflammation and can affect any part of the digestive tract, from the oral cavity to the anus. Although it is recognized that its severity varies, extensive and multiple organ failure is unusual. We present the case of a young patient, who initially presented with pulmonary symptoms associated with CD. Years later, digestive and bile duct symptoms appeared. Treatment was based on anti-tumor necrosis factor-alpha antibody therapy, resulting in a satisfactory clinical response. The clinical relevance of this case is its full-blown presentation, which includes extensive gastrointestinal involvement and rare extraintestinal manifestations.

Mesenteritis esclerosante: diagnóstico y tratamiento / Sclerosing mesenteritis: Diagnosis and treatment

Introducción. El objetivo de este artículo es dar a conocer el caso de un paciente con diagnóstico de mesenteritis esclerosante quien cursó con cuadro de obstrucción intestinal. Descripción del caso. Paciente masculino de 28 años de edad, quien se presenta con cuadro clínico sugestivo de obstrucción intestinal, por lo que se decide resolución quirúrgica. Durante la cirugía se observa una zona fibrótica y adherente del intestino delgado, asociada a un mesenterio engrosado. El examen histopatológico de la pieza quirúrgica confirmó el diagnóstico de mesenteritis esclerosante. Discusión. La mesenteritis esclerosante es una patología de baja incidencia, y su forma de presentación es inespecífica, por lo que el diagnóstico definitivo es histopatológico. La tomografía es útil para el diagnóstico cuando se tiene la sospecha clínica. Puede optarse por el tratamiento quirúrgico para los casos que se presenten con clínica de obstrucción intestinal, o en caso contrario, el tratamiento médico a base de fármacos inmunosupresores e inmunomoduladores ha demostrado ser efectivo. De acuerdo con los diferentes estudios publicados hasta el momento, se observa una adecuada respuesta, independientemente del tratamiento empleado

Introduction. The objective of this article is to present the case of a patient diagnosed with sclerosing mesenteritis who presented with intestinal obstruction.Case description. A 28-year-old male patient, who presented with a clinical picture suggestive of intestinal obstruction, for which a surgical resolution was decided. During surgery, a fibrotic and adherent area of the small intestine is observed, associated with a thickened mesentery. The histopathological examination of the surgical specimen confirmed the diagnosis of sclerosing mesenteritis. Discussion. Sclerosing mesenteritis is a low incidence pathology, and its presentation is nonspecific, so the definitive diagnosis is histopathological. Tomography is useful for diagnosis when there is clinical suspicion. Surgical treatment can be chosen for cases that present with symptoms of intestinal obstruction, or otherwise medical treatment based on immunosuppressive and immunomodulatory drugs has proven to be effective. According to the different studies published so far, an adequate response is observed, regardless of the treatment used

Colangitis esclerosante primaria / Primary sclerosing cholangitis

La colangitis esclerosante primaria (CEP) se define por la inflamación, fibrosis y estenosis de los conductos biliares intra o extrahepáticos que no pueden ser explicadas por otras causas. La prevalencia de CEP está estimada entre 0 a 16,2 por 100.000 habitantes, mientras que la incidencia está entre 0 y 1,3 casos por cada 100.000 personas por año. Las causas siguen siendo difíciles de dilucidar y en muchos casos se establece como de origen idiopático. Sin embargo, se han propuesto factores genéticos, ambientales e isquémicos asociados, además de un componente autoinmune. Existe además una fuerte asociación entre la enfermedad inflamatoria intestinal y la CEP. Los síntomas suelen ser inespecíficos, 50% de los pacientes son asintomáticos, presentando únicamente alteración en el perfil hepático de patrón colestásico, con predominio de elevación de la fosfatasa alcalina. La ictericia es un signo de mal pronóstico que con frecuencia se asocia a colangiocarcinoma. La confirmación diagnóstica se hace por colangiopancreatografía retrógrada endoscópica (CPRE) e imágenes por resonancia magnética. Aún no existe un tratamiento establecido, y en la mayoría de los casos coexiste con otras patologías. El tratamiento es multimodal con fármacos, terapia endoscópica y trasplante hepático.

Primary sclerosing cholangitis (PSC) is defined by inflammation, fibrosis, and stenosis of the intra or extrahepatic bile ducts that cannot be explained by other causes. The prevalence of PSC is estimated between 0 to 16.2 per 100,000 inhabitants, while the incidence is between 0 and 1.3 cases per 100,000 persons-year. The causes remain elusive and, in many cases, it is established as idiopathic in origin. However, genetic, environmental and ischemic factors have been proposed in addition to an autoimmune component. There is also a strong association between inflammatory bowel disease and PSC. Symptoms are usually nonspecific, 50% of the patients are asymptomatic, presenting only an alteration in the liver profile with a cholestatic pattern, and predominance of elevated alkaline phosphatase. Jaundice is a poor prognostic sign and is frequently associated with cholangiocarcinoma. Diagnostic confirmation is made by endoscopic retrograde cholangiopancreatography and magnetic resonance imaging. There is still no established treatment, and in most cases, the disease coexists with other pathologies. Treatment is multimodal with drugs, endoscopic therapy and liver transplantation.

Hepatopatías autoinmunes. Hallazgos clínicos y de laboratorio en pacientes de un hospital de referencia nacional / Autoimmune liver diseases. Clinical and laboratory findings in patients in a national reference hospital

Introducción. Las enfermedades autoinmunes del hígado son un grupo de patologías caracterizadas por una respuesta autoinmune contra los hepatocitos y/o el epitelio biliar. Sus manifestaciones clínicas son variadas, con alteraciones en las pruebas de función hepática y presencia de autoanticuerpos. Metodología. Estudio observacional descriptivo con 101 pacientes atendidos en el Hospital Universitario de La Samaritana de Bogotá D.C., entre enero a diciembre de 2019, con los diagnósticos de hepatitis autoinmune, colangitis biliar primaria, colangitis esclerosante primaria y síndrome de sobreposición. Se evaluaron los parámetros clínicos y de laboratorio, con el fin de caracterizar su frecuencia en estas patologías, debido a la importancia de un diagnóstico precoz. Resultados. Se encontraron 54 casos de hepatitis autoinmune, 19 casos de colangitis biliar primaria, 4 casos de colangitis esclerosante primaria y 24 casos de síndrome de sobreposición. El 81% fueron mujeres y la edad promedio fue de 55 años. El 39% de los pacientes tenían cirrosis. En general, los resultados se ajustaron a lo descrito internacionalmente, como es el predominio en mujeres y la comorbilidad autoinmune. Conclusión. Los hallazgos indican que cualquier alteración del perfil bioquímico hepático debe ser considerado, y se debe descartar la presencia de hepatopatías autoinmunes para diagnosticarlas de manera precoz, evitando que lleguen a cirrosis y sus complicaciones, con la necesidad de un trasplante hepático como única alternativa terapéutica.

Introduction. Autoimmune liver diseases are a group of pathologies characterized by an autoimmune response against hepatocytes and/or the biliary epithelium. Their clinical manifestations are varied, with alterations in liver function tests and the presence of autoantibodies. Methodology. Descriptive study with 101 patients who attended at the Hospital Universitario de La Samaritana in Bogota D.C., between January and December 2019, with the diagnoses of autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and overlap syndrome. Clinical and laboratory parameters were evaluated in order to characterize their frequency in these pathologies, due to the importance of an early diagnosis. Results. There were 54 cases of autoimmune hepatitis, 19 cases of primary biliary cholangitis, 4 cases of primary sclerosing cholangitis, and 24 cases of overlap syndrome. Of all patients, 81% were women, the average age was 55 years, and 39% had cirrhosis. In general, the findings were consistent with what has been described worldwide, such as a higher prevalence in women and autoimmune comorbidity. Conclusion. The findings indicate that any alteration in the liver biochemical profile should be considered to rule out an autoimmune liver disease for an early diagnosis, avoiding the possibility of cirrhosis and its complications, with the need for a liver transplant as the only therapeutic alternative.

Tratamiento exitoso de lipodermatoesclerosis aguda con doxiciclina: reporte de diez casos / Successful treatment of acute lipodermatosclerosis with doxycycline: report of ten cases

Resumen La doxiciclina, una tetraciclina semisintética de segunda generación, ampliamente usada para el tratamiento de patologías dermatológicas por sus propiedades antimicrobianas, ha demostrado en varios estudios experimentales sus acciones como un potente fármaco antiinflamatorio, aprobado por estas características por la FDA como una terapia complementaria en la periodontitis del adulto, así como en el tratamiento de la forma papulopustulosa de la rosácea. Se describen además en la literatura otras indicaciones para procesos inflamatorios, tales como, calcinosis cutis, vasculitis livedoide, patologías ampollares, entre otras. Se presenta una serie de 10 pacientes con lipodermatoesclerosis que presentaron una respuesta eficaz a su patología al recibir tratamiento con doxiciclina durante 3 meses, destacando de esta forma su eficacia terapéutica, accesibilidad y baja asociación a efectos adversos.

Abstract Doxycycline, a second generation semi-synthetic tetracycline, widely used for the treatment of dermatological diseases for its antimicrobial effects, has shown in many clinical studies its actions as a powerful anti-inflammatory drug. Approved by the FDA as a complementary therapy in adult periodontitis, and of the papulopustular form of rosacea. In addition, other indications for inflammatory processes are described in the literature, such as calcinosis cutis, livedoid vasculitis, blistering pathologies, among others. We present a series of 10 patients with lipodermatosclerosis who presented an effective response to their pathology when receiving treatment with doxycycline for 3 months. Its therapeutic efficacy, accessibility and low association with adverse effects are highlighted.

Colangitis esclerosante primaria. Una revisión narrativa / Primary Sclerosing Cholangitis. A narrative review

RESUMEN La colangitis esclerosante primaria (CEP) es una patología hepática crónica y rara que se caracteriza por la inflamación y fibrosis de los conductos biliares, cuya evolución puede llevar a la cirrosis, hipertensión portal y enfermedad hepática en etapa terminal. Su etiología es desconocida, pero se ha relacionado con factores genéticos y autoinflamatorios. Además, tiene una relación muy estrecha con la enfermedad inflamatoria intestinal (EII). Su presentación clínica es muy inespecífica, sus principales síntomas son el prurito y la fatiga. La prueba estándar para su diagnóstico es la colangiopancreatografía por resonancia magnética (CPRM), donde se observa un aspecto anular ocasionado por estenosis multifocales cortas con segmentos alternos normales o dilatados. Actualmente, no existe ningún tratamiento farmacológico que logre prolongar la supervivencia sin un trasplante de hígado en la CEP. Sólo se puede hacer tratamiento sintomático, especialmente del prurito. El único manejo curativo con el que se cuenta hoy en día es el trasplante hepático, aunque existe un riesgo de recurrencia de la enfermedad. Es muy importante la vigilancia de los trastornos inflamatorios intestinales, la malignidad y la enfermedad metabólica ósea en estos pacientes. Se ha visto que algunos factores, como el diagnóstico temprano, son de buen pronóstico para la enfermedad.

SUMMARY Primary sclerosing cholangitis (PSC) is a rare, chronic liver pathology characterized by inflammation and fibrosis of the bile ducts, whose evolution can lead to cirrhosis, portal hypertension and end-stage liver disease. Its etiology is unknown, but it has been related to genetic and autoinflammatory factors. In addition, it has a very close relationship with inflammatory bowel disease. Its clinical presentation is nonspecific, the main symptoms are pruritus and fatigue. The standard test for diagnosis is magnetic resonance cholangiopancreatography (MRCP), where an annular aspect is observed caused by short multifocal stenoses with alternating normal or dilated segments. Currently, there is no pharmacological treatment that can prolong the survival without liver transplantation in PSC. Symptomatic treatment is warranted, especially for pruritus. The only curative treatment that is currently available is liver transplantation, although there is a risk of recurrence of the disease. The monitoring of intestinal inflammatory disorders (IID), malignancy and metabolic bone disease in these patients is very important. It has been seen that some factors, such as early diagnosis, are good prognosis for the disease.

Rehabilitación mediante implante dental tras un caso de osteomielitis focal esclerosante. A propósito de un caso / Rehabilitation by dental implant following a case of focal sclerosing osteomyelitis. A case report

La osteomielitis focal esclerosante es un cuadro de poca frecuencia ante el cual no existen demasiadas opciones terapéuticas, al margen del tratamiento de conductos del diente afectado. En caso de no encontrarse el diente vital, los tratamientos se reducen y, respecto del hueso afectado, no existen evidencias científicas sobre la colocación de implantes. Presentamos un caso clínico en el que se maneja este cuadro eliminando el hueso y regenerándolo con PRGF-Endoret®, para colocar posteriormente un implante dental. (AU)

Focal sclerosing osteomyelitis is a rare condition with few therapeutic options other than root canal treatment of the affected tooth. If the tooth is not vital, the treatments are reduced, and there is little scientific evidence of the benefits of the placement of implants in the affected bone. We present a clinical case in which this condition is managed by removing the affected bone, regenerating it with PRGF-Endoret® and subsequently placing a dental implant in the treated area. (AU)