Síndrome biliar obstructivo no litiásico o de Lemmel. ¿Es tan infrecuente como parece? / Non-lithiasic obstructive biliary syndrome or Lemmel's syndrome. Is it as rare as it seems?

Introducción. Aproximadamente el 5 % de los divertículos duodenales pueden causar síntomas y el 1 % presentar complicaciones, siendo la colangitis la más frecuente. El síndrome de Lemmel corresponde a un tipo de ictericia obstructiva intermitente, asociado a la presencia de divertículos periampulares y disfunción del esfínter de Oddi, sin presencia de coledocolitiasis. Método. Se realizó una revisión sistemática de la literatura en Pubmed, Google Académico y ProQuest, con los términos: síndrome de Lemmel, divertículo duodenal sintomático e ictericia obstructiva intermitente. Resultados. Se encontraron 38 casos, siendo España el país con mayor número, seguido de México, Japón y Colombia. No hay diferencias de distribución con respecto al género. El tratamiento más frecuentemente empleado fue la colangio pancreatografia retrógrada endoscópica. Conclusión. El síndrome de Lemmel es poco frecuente, sin un cuadro clínico especifico, con un incremento en los casos informados en los últimos años, posiblemente debido a la mejor disponibilidad de métodos diagnósticos. Es más frecuente en pacientes en la octava década de la vida y su tratamiento generalmente es endoscópico

Introduction. Approximately 5% of duodenal diverticula can cause symptoms and 1% have complications, cholangitis being the most common. Lemmel syndrome corresponds to a type of intermittent obstructive jaundice, associated with the presence of peri-ampullary diverticula and sphincter of Oddi dysfunction, without choledocholithiasis. Method. A systematic review of the literature was carried out in Pubmed, Google Scholar, ProQuest, with the terms: Lemmel syndrome, symptomatic duodenal diverticulum, and intermittent obstructive jaundice.Results. 38 cases were found, Spain being the country with the highest number, followed by Mexico, Japan and Colombia. There are no differences in distribution with respect to gender. The most frequently used treatment was endoscopic retrograde cholangiopancreatography.Conclusion. Lemmel syndrome is a rare disease, without a specific clinical presentation, with an increase in reported cases in recent years possibly due to the better availability of diagnostic methods. It is more frequent in patients in the eighth decade of life and its treatment is generally endoscopic

Case Report of a Metastatic Biliary Tract Neoplasm as an Unusual Cause of Appendicitis / Reporte de caso de neoplasia biliar metastásica como causa inusual de apendicitis

Abstract Introduction: We describe the case of a patient with appendiceal metastasis as the first manifestation of a cholangiocarcinoma. Main symptoms: Abdominal pain, jaundice, hyporexia, and choluria. Methods and results: We documented an appendiceal plastron histologically compatible with metastatic appendiceal adenocarcinoma, common hepatic duct stricture, and a suspected cholangiocarcinoma, later corroborated by endoscopic retrograde cholangiopancreatography. Conclusions: Metastatic appendiceal tumors are an infrequent and poorly studied manifestation, whereas those secondary to bile duct neoplasia have rarely been reported in the literature.

Resumen Introducción: se describe el caso de una paciente con una metástasis apendicular como primera manifestación encontrada de un colangiocarcinoma. Síntomas principales: expresado con dolor abdominal, ictericia, hiporexia y coluria. Métodos y resultados: se documentó un plastrón apendicular histológicamente compatible con adenocarcinoma apendicular metastásico, estrechez del conducto hepático común, con alta sospecha de colangiocarcinoma, corroborado luego con la realización de una colangiopancreatografía retrógrada endoscópica. Conclusiones: los tumores apendiculares metastásicos son una presentación infrecuente y poco estudiada, donde los secundarios a neoplasia de vía biliar se han reportados de forma muy escasa en la literatura.

Guía para el seguimiento de pacientes pediátricos con enfermedad hepática crónica / Guidelines for the follow-up of pediatric patients with chronic liver disease

La historia natural de la enfermedad hepática crónica (EHC) se caracteriza por una fase de cirrosis compensada asintomática seguida de una fase descompensada, que se acompaña de signos clínicos evidentes, de los cuales los más frecuentes son la ascitis, las hemorragias, la encefalopatía y la ictericia. Esta guía actualizada sobre el manejo de pacientes con EHC en la edad pediátrica fue confeccionada con el propósito de mejorar la práctica clínica de estos pacientes complejos y darle herramientas al pediatra de cabecera para un seguimiento adecuado. Para ello, un grupo de expertos subrayó la importancia del inicio temprano del tratamiento etiológico en cualquier grado de enfermedad hepática y ampliaron su labor jerarquizando las complicaciones de la cirrosis: ascitis, hemorragia digestiva, infecciones, malnutrición; aspectos endocrinológicos, neurológicos, oftalmológicos y gastrointestinales; y complicaciones vasculares pulmonares y renales. Se incluyeron, además, aspectos psicosociales, así como el cuidado del adolescente en su transición a la vida adulta.

The natural history of chronic liver disease (CLD) is characterized by a phase of asymptomatic compensated cirrhosis followed by a decompensated phase, accompanied by the development of evident clinical signs, the most frequent being ascites, hemorrhages, encephalopathy and jaundice. This updated guideline on the management of pediatric patients with CLD was developed with the purpose of improving the clinical practice of these complex patients and to provide the pediatrician with tools for an adequate follow-up. To this end, a group of experts, after stressing the importance of early initiation of etiologic treatment in any degree of liver disease, expanded their work to include a hierarchy of complications of cirrhosis: ascites, gastrointestinal bleeding, infections, malnutrition, endocrinological, neurological, ophthalmological, gastrointestinal, pulmonary vascular and renal complications. Psychosocial aspects including the care of the adolescent in their transition to adult life were also included.

Hiperbilirrubinemia: visión del patólogo / Hyperbilirubinemia: pathologist's view

Los niveles de bilirrubina sérica normal en el adulto varían entre 0,3 mg/dL y 1,2 mg/dL, y su valor está determinado por la tasa de captación hepática, conjugación y excreción. La ictericia se hace evidente cuando los niveles de bilirrubina sérica se elevan por encima de 2,5 mg/dL a 3 mg/dL, siendo un indicador de enfermedad subyacente. La bilis es producida por los hepatocitos y fluye desde los canalículos, canales de Hering, conductos biliares intrahepáticos, conductos hepáticos derechos e izquierdos hasta llegar al duodeno. A nivel histopatológico, cualquier entidad que lleve a la acumulación intrahepática de bilis por disfunción hepatocelular u obstrucción biliar genera colestasis, que se observa en la biopsia hepática como la acumulación de tapones de color marrón verdoso de pigmento biliar en los hepatocitos, y secundariamente se observan los canalículos dilatados. Las causas de colestasis intrahepática son diversas e incluyen enfermedades como colangitis biliar primaria, colangitis esclerosante primaria, hepatitis autoinmune, hepatitis virales y toxicidad medicamentosa. Esta revisión tiene como objetivo analizar algunos tipos de hiperbilirrubinemia, resaltando sus características histopatológicas.

Normal serum bilirubin levels in adults range from 0.3 mg/dL to 1.2 mg/dL, and its value is determined by the rate of hepatic uptake, conjugation, and excretion. Jaundice becomes apparent when serum bilirubin levels rise above 2.5 mg/dL to 3.5 mg/dL and is an indicator of underlying disease. Bile is produced by hepatocytes and flows from the canaliculi, Hering's canals, intrahepatic bile ducts, and right and left hepatic ducts to the duodenum. Pathologically, any condition that leadsto intrahepatic accumulation of bile due to hepatocellular dysfunction or biliary obstruction, generates cholestasis, which is observed in liver biopsy as the accumulation of greenish-brown deposits of bile pigment in hepatocytes, with dilated canaliculi. The causes of intrahepatic cholestasis are diverse and include diseases such as primary biliary cholangitis and primary sclerosing cholangitis, autoimmune hepatitis, viral hepatitis, and drug toxicity. This review aims to analyze some types of hyperbilirubinemia, highlighting their histopathological characteristics.

Hepatotoxicidad por talidomida: caso clínico y revisión de la literatura / Hepatotoxicity by thalidomide: case report and literature review

La talidomida fue desarrollada e introducida al mercado por los laboratorios Grünenthal en 1953, siendo usada principalmente como sedante y también para el tratamiento de las náuseas durante el embarazo. Los informes dan cuenta de aproximadamente 10.000 niños que nacieron con focomelia, dando lugar a la denominada "tragedia de la talidomida", que obligó a su retiro del mercado en 1962. Luego de casi 60 años, es nuevamente utilizada en otros campos de la medicina, entre ellos, para el tratamiento de la lepra y del mieloma múltiple, debido a sus propiedades antinflamatorias, inmunomoduladoras y antiangiogénicas, con expresas advertencias sobre su utilización durante el embarazo; no obstante, con su nuevo uso han sido reportados múltiples efectos adversos, entre los que se encuentra la hepatitis aguda o crónica inducida por este fármaco. Se presenta el caso de una paciente de 34 años con lepra, que estaba en tratamiento con talidomida desde hacía 4 años para combatir las lesiones de piel asociadas a esta enfermedad. Presentó malestar general, vómito, pérdida de peso, artralgias, ictericia, edemas de miembros inferiores, ascitis, coluria y acolia. Se sospechó toxicidad por talidomida, por lo que se suspendió su uso, y se trató con ácido ursodesoxicólico y N-acetilcisteína con mejoría sintomática y de laboratorio, desde la primera semana hasta los 41 días de seguimiento. Las entidades clínicas para las cuales se aprobó talidomida en 1998, pueden traer nuevos problemas y desafíos clínicos. Este caso muestra hepatotoxicidad crónica por talidomida, situación que hasta el momento no se había reportado en la literatura.

Thalidomide was developed and introduced to the market by Grünenthal laboratories in 1953, being used mainly as a sedative and also for the treatment of nausea during pregnancy. Reports give account of approximately 10,000 children who were born with phocomelia, giving rise to the so-called "thalidomide tragedy", which forced its withdrawal from the market in 1962. After almost 60 years, it is usedagain in other fields of medicine, including the treatment of leprosy and multiple myeloma, due to its anti-inflammatory, immunomodulatory and anti-angiogenic properties, with clear warnings about its use during pregnancy; however, multiple adverse effects have been reported in patients with leprosy and multiple myeloma, including acute or chronic hepatitis. We present the case of a 34-year-old patient with leprosy, who had been on thalidomide therapy for 4 years to treat skin lesions associated with this disease. She presented general malaise, vomiting, weight loss, arthralgia, jaundice, lower limb edema, ascites, choluria and acholia. Thalidomide toxicity was suspected, so its use was suspended, and treatment with ursodeoxycholic acid and N-acetylcysteine was initiated, with symptomatic and laboratory improvement from the first week up until 41 days of follow-up. The new range of medical conditions for which thalidomide was approved for in 1998 may bring clinical challenges. This case shows chronic hepatotoxicity due to thalidomide, a situation that had not been reported previously in the literature.

Estanozolol como anabólico: una causa de hepatotoxicidad / Estanozol as anabolic: a cause of hepatotoxicity / Estanozolol Como Anabólico: Uma Causa De Hepatotoxicidade

Los antibióticos y analgésicos han sido descritos frecuentemente como las principales causas de toxicidad hepática. Los esteroides anabólicos se han relacionado también con alteraciones en sistemas como el cardiovascular o el hepático; en este último causan colestasis, carcinoma hepatocelular, hiperplasia regenerativa nodular y sangrado de varices, secundario a hipertensión portal. Es importante entonces considerar los esteroides anabólicos como factores de riesgo para hepatotoxicidad. Se presenta el primer caso en Colombia y uno de los pocos en Latinoamérica, de colestasis asociada únicamente al uso de estanozolol. Se trata de un paciente de 21 años, en tratamiento con el medicamento para incrementar la masa muscular, que presentó compromiso hepático de tipo colestásico. Se descartaron otras posibles causas de ictericia, mediante la escala CIOMS/RUCAM se llegó a establecer causalidad entre el consumo de estanozolol y la colestasis. El objetivo de este reporte es hacer una descripción no reportada en la literatura colombiana y poco común en la literatura mundial.

Antibiotics and pain relievers have been frequently described as the main causes of liver toxicity. Anabolic steroids have also been linked to alterations in systems such as cardio-vascular or liver. In the latter, they seem to cause cholestasis, hepatocellular carcinoma, nodular regenerative hyperplasia and variceal bleeding secondary to portal hypertension. It is important to consider them as factors associated with hepatotoxicity. The first case in Colombia and one of the few in Latin America of cholestasis associated only to the use of Stanozolol is presented in a 21-year-old patient under treatment with the drug to increase muscle mass. The patient presented with cholestatic liver involvement. Other possible causes of jaundice were ruled out. From the CIOMS / RUCAM scale, causality was established between the consumption of Stanozolol and cholestasis. The objective of this case is to report a case not found in Colombian literature and little reported in world literature.

Antibióticos e analgésicos têm sido frequentemente descritos como as principais causas de toxicidade hepática. Os esteroides anabolizantes também têm sido relacionados a alterações em sistemas como cardiovasculares ou hepáticos; neste último, causam colestase, carcinoma hepatocelular, hiperplasia nodular regenerativa e sangramento varicoso, secundário à hipertensão portal. Portanto, é importante considerar os este-roides anabolizantes como fatores de risco para hepatotoxicidade. O primeiro caso é apresentado na Colômbia e um dos poucos na América Latina, de colestase associada apenas ao uso de estanozolol. Paciente de 21 anos, em tratamento com fármaco para aumento de massa muscular, apresentou acometimento hepático colestático. Outras possíveis causas de icterícia foram descartadas, a escala CIOMS / RUCAM estabeleceu causalidade entre o consumo de estanozolol e colestase. O objetivo deste relatório é fazer uma descrição não relatada na literatura colombiana e rara na literatura mundial

Hematúria macroscópica em equinos associada à infecção por Leptospira interrogans / Macroscopic haematuria in horses associated with Leptospira interrogans infection

Hematúria é uma grave manifestação clínica de doença do sistema urinário, ocorrendo sob as formas micro ou macroscópica. Neste artigo relatam-se dois casos de hematúria macroscópica associada à infecção por Leptospira interrogans sorogrupo Canicola. O exame clínico inicial revelou hematúria macroscópica, taquicardia, taquipneia, febre, elevação do tempo de perfusão capilar, hipomotilidade intestinal, além de icterícia da mucosa oral. Leucocitose, proteinúria, glicosúria, piúria e azotemia foram achados comuns aos dois casos. Teste de Soroaglutinação Microscópica foi realizado para titulação de anticorpos contra Leptospira interrogans. Tratamento incluiu medidas terapêuticas de suporte (fluidoterapia), controle da hematúria e antibioticoterapia. Sete dias após manifestação dos sinais clínicos iniciais, ambos animais receberam alta hospitalar após remissão dos sinais clínicos.

Haematuria is a serious clinical manifestation of urinary system disease, occurring in micro or macroscopic forms. In this article two cases of macroscopic haematuria associated with Leptospira interrogans serogroup Canicolainfection are related. The initial clinical examination revealed macroscopic haematuria, tachycardia, tachypnea, fever, increased capillary perfusion time, intestinal hypomotility, in addition to jaundice of the oral mucosa. Leukocytosis, proteinuria, glycosuria, pyuria and azotemia were common findings in both cases. Microscopic serum agglutination test was performed for titration of antibodies against Leptospira interrogans. Treatment included supportive therapeutic measures (fluid therapy), hematuria control and antibiotic therapy. Seven days after the manifestation of the initial clinical signs, both animals were discharged from the hospital without complications.

Hemobilia, una causa infrecuente de hemorragia del tracto digestivo superior / Hemobilia, a rare cause of upper gastrointestinal bleeding

Resumen La hemobilia es una causa poco frecuente de hemorragia del tracto gastrointestinal superior. La principal etiología es de origen iatrogénico y la posibilidad de hemobilia debe considerarse en cualquier paciente con hemorragia gastrointestinal y un historial reciente de procedimientos hepatobiliares. Otras causas menos frecuentes incluyen el trauma de abdomen, la enfermedad oncológica de la vía biliar o las enfermedades inflamatorias del páncreas o la vía biliar. La presentación clínica varía según la gravedad del sangrado; generalmente se presenta con dolor abdominal, ictericia y melenas, aunque puede cursar al ingreso con rectorragia e hipotensión. Un alto porcentaje de estas presenta resolución espontánea, sin requerir procedimientos adicionales. La angiografía es el estándar de oro para el diagnóstico de la hemobilia, pero los avances en la angiotomografía permiten que esta sea una opción menos invasiva y con mayor disponibilidad. La angioembolización es el tratamiento principal para estos pacientes, pero existen otras alternativas como la colocación de stent vascular o de stent en el conducto biliar.

Abstract Hemobilia is a rare cause of upper gastrointestinal (GI) tract bleeding. Its main etiology is iatrogenic, and the possibility of hemobilia should be considered in any patient with GI bleeding and a recent history of hepatobiliary surgery. Other less frequent causes include abdominal trauma, oncologic disease of the biliary tract, or inflammatory diseases of the pancreas or bile duct. Its clinical presentation varies depending on the severity of the bleeding. It usually presents with abdominal pain, jaundice, and tarry stools, although patients may also present with rectorrhagia and hypotension on admission. A high percentage of these symptoms have a spontaneous resolution, without requiring additional procedures. Angiography is the gold standard for the diagnosis of hemobilia, but advances in computed tomography angiography make it a less invasive and more widely available option. Endovascular embolization is the main treatment for these patients, but there are other alternatives such as vascular or bile duct stent placement.

Atresia biliar por citomegalovirus: un reporte de caso / Cytomegalovirus-associated biliary atresia: Case report

Resumen La atresia biliar asociada con inmunoglobulina M (IgM) positiva para citomegalovirus (CMV) es una entidad infrecuente que se caracteriza por la obliteración inflamatoria progresiva de los conductos intra- o extrahepáticos producida por una reacción autoinmune perinatal contra el CMV. El diagnóstico se realiza con IgM positiva para CMV y biopsia hepática con evidencia de atresia de las vías biliares. El conocimiento y la identificación temprana de esta patología conduce a un manejo quirúrgico temprano, mejorando considerablemente el pronóstico de estos pacientes. Se presenta un caso clínico de una paciente de 82 días de edad con un cuadro de acolia, coluria e ictericia de inicio tardío, asociado con hiperbilirrubinemia a expensas de la directa, elevación de perfil hepático e IgM positiva para CMV. La colangiorresonancia intraoperatoria confirmó el cuadro de atresia de las vías biliares. Se realizó una derivación biliodigestiva tipo Kasai y la toma de biopsia hepática que confirmó el cuadro clínico.

Abstract Biliary atresia associated with positive cytomegalovirus IgM results is a rare condition characterized by progressive inflammatory obliteration of the intra- or extrahepatic ducts. It is caused by a perinatal autoimmune reaction against cytomegalovirus (CMV). Diagnosis is made based on positive IgM for CMV and liver biopsy with evidence of bile duct atresia. Knowledge and timely identification of this disease leads to early surgical management, considerably improving the prognosis of these patients. This is the clinical case of an 82-day-old female patient with late-onset acholia, choluria, and jaundice, associated with conjugated hyperbilirubinemia, elevated liver function tests and positive CMV IgM results. Intraoperative cholangioresonance confirmed bile duct atresia. The Kasai procedure was performed, and a liver biopsy was taken, confirming the diagnosis.

Hígado agudo graso del embarazo: reporte de caso / Acute fatty liver of pregnancy: case report

El hígado graso del embarazo es una patología poco frecuente en la especialidad obstétrica, cuyo diagnóstico se realiza basado en los criterios de Swansea, muchas veces es un diagnóstico que se realiza por exclusión; usualmente se presenta entre las semanas 30 y 35 del embarazo, y la cura definitiva se realiza con la interrupción expedita del mismo; con una tasa de recuperación casi del 100% si se realiza la interrupción oportuna y una tasa de mortalidad materno fetal actual del 10%. Es importante estar atentos a la ganancia ponderal de la embarazada durante el control prenatal, la epigastralgia, y los signos clínicos asociados a hipoglicemia(AU)

Fatty liver of pregnancy is a rare pathology in obstetrics, whose diagnosis is made based on the Swansea criteria, many times it is a diagnosis that is made by exclusion; It usually occurs between weeks 30 and 35, and the definitive cure is carried out with the expeditious interruption of pregnancy; with a recovery rate of almost 100% if timely interruption is made and a current maternal-fetal mortality rate of 10%. It is important to be attentive to the weight gain of the pregnant woman during prenatal control, epigastric pain, and clinical signs associated with hypoglycemia

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.

Variante "no clásica" o "atípica" del síndrome de Stauffer en paciente con carcinoma renal de células claras: reporte de caso / "Non-classical" or "atypical" variant of Stauffer syndrome in a patient with clear cell renal carcinoma: a case report

La manifestación paraneoplásica conocida como síndrome de Stauffer tiene una presentación atípica, caracterizada por ictericia y colestasis intrahepática. Presentamos el caso de un paciente de 53 años de edad, con antecedente de una masa renal derecha en plan de resección quirúrgica programada, con cuadro de evolución de dolor abdominal en hipocondrio derecho e ictericia. A su ingreso se documentó hepatoesplenomegalia, elevación de bilirrubinas a expensas de la directa, y de fosfatasa alcalina junto con elevación de transaminasas. Se descartaron causas obstructivas a nivel de vía biliar intra y extrahepática. No se documentaron metástasis o lesiones focales a nivel de parénquima, ni lesiones de etiología vascular que explicaran el cuadro. También se descartó hepatitis B, C e infección por VIH, por lo cual se consideró un probable síndrome de Stauffer. Fue llevado a nefrectomía intrahospitalaria, con posterior diagnóstico patológico compatible con carcinoma de células claras. Luego del procedimiento se normalizó la bioquímica hepática y se corrigió la ictericia. Es importante reconocer que la afectación hepática en el contexto de neoplasias, no es solo atribuida a metástasis a distancia, sino también a la existencia de síndromes paraneoplásicos como condicionantes.

The paraneoplastic manifestation known as Stauffer syndrome has an atypical presentation, characterized by jaundice and intrahepatic cholestasis. We present the case of a 53-year-old patient, with a history of a right renal mass with a planned surgical resection, who developed abdominal pain in the right upper quadrant and jaundice. Upon admission, hepatosplenomegaly, elevated bilirubin, at the expense of direct bilirubin, alkaline phosphatase and elevated transaminases were documented. Intra- and extrahepatic bile ducts obstruction were ruled out. There were no documented metastases or focal lesions at the level of the parenchyma, or lesions of vascular etiology that could explain the condition. Hepatitis B, C and HIV infection were also ruled out, and a probable Stauffer syndrome was considered. In-hospital nephrectomy was performed, with subsequent pathology compatible with clear cell carcinoma. After the procedure, liver biochemistry was normalized and jaundice was corrected. It is important to recognize that liver involvement in the context of neoplasms is not only attributed to distant metastases but to the existence of paraneoplastic syndromes as determining factors.

Anormalidades de la vesícula biliar en hepatitis A: a propósito de un caso / Gallbladder abnormalities in hepatitis A: a case report

El virus de hepatitis A es la causa más común de hepatitis viral aguda en el mundo. La infección en adultos produce un cuadro sintomático, agudo y autolimitado, pero se pueden presentar alteraciones extrahepáticas, como anormalidades en la vesícula biliar y pancreatitis aguda. Se presenta el caso de un hombre de 49 años con engrosamiento de las paredes de la vesícula biliar, atribuido en un principio a una neoplasia de la vesícula, cuya etiología ulterior fue la hepatitis A. Las anormalidades de la vesícula son un hallazgo frecuente en las hepatitis virales agudas, en especial por virus de hepatitis A. Los médicos deben estar familiarizados con esta manifestación extrahepática de la infección para no incurrir en exámenes innecesarios o tratamientos invasivos como la colecistectomía.

Hepatitis A virus is the most common cause of acute viral hepatitis in the world. Infection in adults produces an acute, self-limited symptomatic disease, but extrahepatic manifestations may occur, such as gallbladder abnormalities and acute pancreatitis. The case of a 49-year-old man with gallbladder wall thickening, initially attributed to gallbladder cancer, whose subsequent etiology was hepatitis A, is presented. Abnormalities of the gallbladder are common in patients with acute viral hepatitis, especially due to hepatitis A. Physicians caring for adults with hepatitis A virus infection should be aware that such disease manifestations may occur, in order to avoid unnecessary clinical investigations or invasive treatments such as cholecystectomy.

Síntomas prevalentes y factores de riesgo de litiasis biliar en pacientes pediátricos en un hospital de referencia / Prevalent symptoms and risk factors of gallstones in pediatric patients at a reference hospital

Introducción: La litiasis biliar es una patología considerada como poco frecuente en la infancia; sin embargo, el 50% de los adultos jóvenes con enfermedad vesicular presentaron sus primeros síntomas a edades tempranas. Existen pocos estudios en población pediátrica con esta patología en el Paraguay. Objetivos: Determinar los síntomas más frecuentes y la presencia de factores asociados a litiasis biliar en niños y adolescentes en un hospital de referencia. Materiales y métodos: Estudio transversal, observacional, descriptivo, retrospectivo, realizado con datos secundarios de fichas clínicas de pacientes internados entre los meses de enero del 2014 y enero del 2019. Muestreo no probabilístico de casos consecutivos. Resultados: Se incluyeron 58 pacientes con diagnóstico de litiasis biliar, 3 (5,2%) en edad pre-escolar, 9 (15,5%) escolares y 46 (79,3%) adolescentes. Fueron del sexo femenino 39/58 (67,2%). En cuanto a las manifestaciones clínicas, 57 (98%) pacientes refirieron dolor abdominal, 30 (51%) presentaron vómitos y el resto en menor proporción cursaron con ictericia, coluria o hipocolia. Con respecto a la situación nutricional, 1 (1,7%) paciente presentó desnutrición y 29 (50%) pacientes sobrepeso u obesidad. En relación con la evaluación de la talla, 5 (8,6%) tenían talla baja. En 4 (6,8%) pacientes hubo antecedentes familiares de litiasis biliar. Ninguno presentó complicaciones. No se observaron casos asociados a uso de nutrición parenteral. 2 pacientes habían recibido antibiótico de amplio espectro. Conclusión: La litiasis biliar fue más frecuente en niñas y en la etapa de la adolescencia, en la mayoría fue idiopática y el dolor abdominal fue la manifestación más frecuente. Conclusión: La litiasis biliar fue más frecuente en niñas y en la etapa de la adolescencia, en la mayoría fue idiopática y el dolor abdominal fue la manifestación más frecuente.

Introduction: Gallstones are considered infrequent in childhood; however, 50% of young adults with gallbladder disease had their first symptoms during this period. There are few studies in the pediatric population about this pathology in Paraguay. Objectives: To determine the prevalent symptoms and the presence of predisposing factors in children and adolescents with gallstones at a referral hospital. Materials and methods: it is a cross-sectional, retrospective, descriptive, observational study; based on clinical records of patients hospitalized between January 2014 and January 2019, non-probabilistic sampling of consecutive cases of patients who meet the inclusion criteria. Results: 58 patients with the diagnosis of gallstones were found, of which 3 (5.2%) were preschool children, 9 (15.5%) children and 46 (79.3%) adolescents. There was a predominance of females 67.2% (39/58) over males 33% (19/58). As for clinical manifestations, 57 patients (98%) reported abdominal pain, 30 (51%) vomiting and a lesser extent coursed with jaundice, dark urine or hypocolia. Regarding the nutritional profile, 1 patient (8,6%) presented malnutrition and 29 (50%) were overweight or obese. In relation to height, 5 (8.6%) were short stature. Only 4 patients (6.8%) had a family history of gallstones. None of them had complications or were associated to parenteral nutrition. 2 patients had received broad spectrum antibiotics. Conclusion: Gallstones occurred mainly in female adolescents. In most cases was idiopathic and, abdominal pain was the most frequent symptom.

Avaliação de pacientes com hepatite autoimune atendidos em ambulatório de referência do sul do Brasil / Evaluation of patients with autoimmune hepatitis in a specialized outpatient clinic in southern brazil

ABSTRACT BACKGROUND: Autoimmune hepatitis (AIH) is a chronic liver disease, characterized by necroinflammation and autoimmune etiology. Studies evaluating the characteristics of patients with AIH are scarce in Brazil. OBJECTIVE: Our objective was to evaluate the profile of patients with AIH in a specialized center in Southern Brazil and to verify factors related to treatment response. METHODS: this was a retrospective cohort study, which analyzed demographic, epidemiological, clinical, laboratory, and histologic data. Patients with AIH diagnosed according to the criteria of the International Autoimmune Hepatitis Group (IAIHG) were included. In liver biopsies, the degree of fibrosis, histological activity, presence of hepatocyte rosettes, plasma cell infiltrates, and confluent necrosis were evaluated. In the statistical analysis, the significance level was 5%. RESULTS: Forty adults patients diagnosed with AIH were included. The evaluated population predominantly consisted of women (75.0%) and the average age at diagnosis was 44.2 years. The association with extrahepatic autoimmune diseases occurred in 20.0% of cases. Clinically, 35.0% of patients presented with acute onset hepatitis, 37.5% with cirrhosis, and 27.5% with other forms of presentation. The most common clinical manifestation was jaundice (47.5%). Thirty-five patients were treated, and of these, 97.1% used prednisone combined with azathioprine. The average treatment time was 2.7 years. Response to treatment was complete or partial in 30 (85.7%) and absent in 5 (14.3%) patients. There was no statistically significant difference when evaluating response to treatment in relation to forms of presentation, histological findings, and the presence of autoantibodies. Regarding fibrosis, regression was observed in 18.75% of the cases. CONCLUSION: Most patients with AIH were young at presentation and of female sex. The association with extrahepatic autoimmune diseases and cirrhosis at presentation was seen in a considerable proportion of patients. Treatment was effective, but there were no clinical, histological or serological parameters capable of predicting treatment response.

RESUMO CONTEXTO: A hepatite autoimune (HAI) é uma doença hepática crônica, de caráter necroinflamatório e etiologia autoimune. Os estudos que avaliam as características de pacientes com HAI são escassos no Brasil. OBJETIVO: Nosso objetivo foi avaliar o perfil dos pacientes com HAI atendidos em um centro de referência do sul do Brasil e verificar fatores relacionados à resposta ao tratamento. MÉTODOS: Este foi um estudo de coorte retrospectivo, que analisou dados demográficos, epidemiológicos e clínicos. Nas biópsias hepáticas, foram avaliados o grau de fibrose, a atividade histológica, a presença de rosetas, de infiltrado plasmocitário e de necrose confluente. Na análise estatística, o nível de significância foi de 5%. RESULTADOS: Foram incluídos 40 pacientes adultos com diagnóstico de HAI. Houve predomínio do sexo feminino (75,0%), e a média de idade no diagnóstico foi de 44,2 anos. A associação com doenças autoimunes extra-hepáticas ocorreu em 20,0% dos casos. Clinicamente, 35,0% dos pacientes se apresentaram sob forma de hepatite aguda, 37,5% com cirrose e 27,5% com outras formas de apresentação. A manifestação clínica mais comum na apresentação foi a icterícia (47,5%). Trinta e cinco pacientes foram tratados, sendo que destes, 97,1% utilizaram prednisona associada com azatioprina. A média do tempo de tratamento foi 2,7 anos. A resposta ao tratamento foi completa ou parcial em 30 (85,7%) e ausente em 5 (14,3%) pacientes. Não houve diferença estatisticamente significativa quando avaliada a resposta ao tratamento em relação à forma de apresentação, aos achados histológicos e à presença de autoanticorpos. Em relação à fibrose, foi observada regressão em 18,75% dos casos. CONCLUSÃO: A maioria dos pacientes era jovem no momento do diagnóstico e do sexo feminino. A associação com doenças autoimunes extra-hepáticas e com cirrose na apresentação foi vista em uma parcela considerável dos casos. O tratamento foi eficaz, mas não houve parâmetros clínicos, histológicos ou sorológicos capazes de prever a resposta ao tratamento.

Elevación de enzimas hepáticas inducida por COVID-19 en embarazada / Elevated liver enzimes induced by COVID-19 in pregnancy

INTRODUCCIÓN: Las alteraciones del perfil hepático durante el embarazo ocurren en 3-5% de las gestantes. Una nueva etiología que se ha presentado en el contexto de pandemia actual es el síndrome respiratorio agudo severo relacionado con el nuevo coronavirus (SARS-CoV-2). Éste es responsable de alteraciones hepáticas en 2 a 11% de la población general infectada por este virus, y de hasta un 30% en las embarazadas que se infectan con SARS-CoV-2. Con el objetivo de mostrar una presentación poco frecuente del SARS-CoV-2 se expone un caso clínico de elevación de transaminasas en embarazada inducida por este nuevo virus. CASO CLÍNICO: Paciente de 36 años, cursando embarazo de 20+6 semanas, consulta por dolor abdominal asociado a ictericia y coluria. Se solicita estudio donde destaca elevación de transaminasas. Ecografía abdominal con vía biliar fina. Se descartan diferentes etiologías de hepatitis aguda y crónica (dada la falta de antecedentes). Finalmente se solicita PCR para COVID-19 que resulta positiva. CONCLUSIÓN: Luego de un estudio exhaustivo de diferentes etiologías de elevación de transaminasas, se atribuye esta alteración enzimática a SARS-CoV-2. Se decide seguimiento ambulatorio estricto con pruebas hepáticas cada dos semanas. La paciente evoluciona estable con exámenes normales luego de un mes desde que se indica el alta hospitalaria. Después de descartar etiologías frecuentes de elevación de transaminasas durante el embarazo, sugerimos solicitar el estudio de este virus con PCR para COVID-19, ya que podría ser una presentación poco frecuente de SARS-CoV-2.

INTRODUCTION: Approximately 3-5% of women present alterations of hepatic enzymes during pregnancy. Under the new circumstances that the world is facing with the SARS-COV2 pandemic, a new etiology for hepatic enzyme alterations has risen. The severe acute respiratory syndrome that the novel coronavirus causes is responsible for hepatic enzyme alterations in 2 to 11% of the sick population that did not have a previous underlying hepatic condition. Furthermore, hepatic enzyme alterations in pregnant women infected with SARS-COV2 presents in up to 30% of the cases. An infrequent presentation of SARS-COV2 is presented as our clinical case. CLINICAL CASE: A 36-year-old patient with a 20+6 week pregnancy presents abdominal pain, jaundice and choluria. General blood workup shows elevated transaminases. The abdominal ultrasound revealed a thin bile duct. Acute and chronic hepatitis etiologies were discarded. Finally, a PCR of COVID-19 was solicited, which turned out to be positive. CONCLUSIÓN: After an exhaustive study to determine the etiology of the elevated transaminases, the hepatic alterations were attributed to SARS-COV2 infection. A conservative management was adopted, with outpatient follow-up with liver testing every two weeks. The patient progresses with a stable steady decline in hepatic enzyme levels, and one-month post hospital discharge, her transaminases had reached normal values. Based on this clinical case, after ruling out frequent etiologies for elevated transaminases during pregnancy, it seems reasonable to request a PCR for COVID-19, since it could be a rare presentation of SARS-CoV-2.

Enfermedad de Caroli: a propósito de un caso / Carolis disease: report of one case

Resumen La enfermedad de Caroli es una rara patología caracterizada por la existencia de dilataciones saculares segmentarias del árbol biliar intrahepático, que, por lo general, afectan a todo el parénquima hepático, aunque es poco frecuente el compromiso biliar extrahepático. Presentamos el caso de un varón que ingresó con un cuadro clínico de 45 días de evolución, consistente en ictericia colestásica, baja ponderal y prurito. La colangiorresonancia informó múltiples dilataciones saculares en las vías biliares intrahepáticas.

Abstract Caroli disease is a condition characterized by cystic dilatation of the intrahepatic biliary tree that usually affects the entire liver parenchyma, although extrahepatic biliary involvement is rare. The following is the case of a male patient who was admitted due to cholestatic jaundice, low weight, and pruritus for 45 days. Magnetic resonance cholangiopancreatography reported multiple cystic dilatations in the intrahepatic bile ducts.

Differential diagnoses in 83 dogs with icterus / Diagnósticos diferenciais em 83 cães com icterícia

Icterus (jaundice) is a yellowish pigmentation resulting from the depositing of bilirubin in tissues due to its high plasmatic concentration. The pathogenesis of icterus includes metabolic changes or obstructed bilirubin excretion and it is classified as pre-hepatic, hepatic and post-hepatic. This study aimed to evaluate and classify different causes of icterus in dogs during post mortem examination. These dogs were examined from 2014 to 2017, using macroscopic and histologic exams as well as ancillary tests. Eighty-three dogs were examined macroscopically and microscopically. They were separated into groups of icterus types: 24 (28.9%) dogs had pre-hepatic icterus, 45 (54.2%) had hepatic, 13 (15.7%) pre-hepatic and hepatic and one (1.2%) had post-hepatic icterus. Many factors were identified as a cause of icterus, including infectious agents (51/83), neoplasms (13/83), hepatic degeneration (11/83), chronic hepatic diseases (6/83), and obstructive causes (1/87). Among the infectious causes, leptospirosis, ehrlichiosis and disorders suggestive of septicemia were diagnosed. Neoplasms associated with icterus were cholangiocarcinoma, hemangiosarcoma and lymphoma. Other causes of icterus included degenerative diseases, such as lipidosis and glycogen degeneration. Hepatic fibrosis (cirrhosis) as a chronic disease and cholelithiasis also produced icterus. PCR was performed to confirm leptospirosis and ehrlichiosis. Samples of total DNA were used to amplify a fragment of a gene from Leptospira interrogans and Ehrlichia canis. In some dogs, co-infection of these agents was detected. The classification and identification of icterus etiologies in dogs is very important due to the number of diseases with this alteration, where ante mortem diagnosis is not always easily performed when some of these conditions are present.(AU)

Icterícia é a pigmentação amarelada decorrente da deposição de bilirrubina em tecidos devido à elevada concentração plasmática. A patogênese da icterícia inclui alterações no metabolismo ou na excreção de bilirrubina, sendo classificada em pré-hepática, hepática ou pós-hepática. O objetivo desse estudo foi identificar, avaliar e classificar as causas de icterícia em cães necropsiados de 2014 a 2017, associando as lesões macroscópicas, histológicas e exames complementares. Foram avaliados macro- e microscopicamente 83 cães com diferentes intensidades de icterícia. Os cães foram separados em grupos de acordo com o tipo de icterícia: 24 (28,9%) cães com icterícia pré-hepática, 45 (54,2%) cães com icterícia hepática, 13 (15,7%) com icterícia pré-hepática e hepática e um (1,2%) com icterícia pós-hepática. Foram identificadas várias etiologias associadas à icterícia, dentre elas pode-se destacar, agentes infecciosos (51/83), neoplasmas (13/83), processos degenerativos (11/83), crônicos (6/83) e obstrutivos (1/83). Dentre as causas infecciosas, destacam-se a leptospirose, a erliquiose e as lesões sugestivas de septicemia. Entre os neoplasmas associados com icterícia destacaram-se o colangiocarcinoma, hemangiossarcoma e linfoma. Outras causas de icterícia incluiriam os processos degenerativos como as degenerações gordurosa e glicogênica. Fibrose hepática (cirrose) e colelitíase foram também diagnosticados como causa de icterícia. A PCR foi utilizada para o diagnóstico confirmatório de leptospirose e erliquiose. Amostras de DNA total foram utilizadas para amplificar um fragmento dos genes de Leptospira interrogans e de Ehrlichia canis. Em alguns cães foi detectada co-infecção por estes agentes. A classificação e a identificação das causas de icterícia em cães são relevantes devido ao grande número de doenças que apresentam essa alteração, muitas vezes sem diagnóstico ante mortem.(AU)

Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

Adenocarcinoma de papila duodenal maior - Um relato de caso / Adenocarcinoma of the major duodenal papilla - A case report

Tumores periampulares (TP) são originados a partir de estruturas próximas a Ampola de Vater e correspondem a um grupo heterogêneo de neoplasias. O adenocarcinoma da papila duodenal maior faz parte desse grupo de neoplasias. As principais manifestações são a icterícia do tipo obstrutiva, prurido, colúria, acolia fecal, além de perda ponderal e dor abdominal, associado a elevação dos níveis séricos de bilirrubina e enzimas hepáticas. O prognóstico é obscuro, sendo os carcinomas do tipo pancreaticobiliar aqueles com pior prognóstico. A ecografia endoscópica é o método mais sensível para diagnóstico e estadiamento e o tratamento de escolha é pancreaticoduodenectomia, conhecida como cirurgia de Whipple, frequentemente associada à quimioterapia adjuvante ou terapia de quimiorradiação. O presente trabalho propõe descrever um caso de adenocarcinoma da ampola de Vater em paciente de 38 anos atendida no Hospital Universitário da Universidade Federal de Juiz de Fora (HU-UFJF), abrangendo desde a sintomatologia inicial, processo diagnóstico até a conduta terapêutica. Sua relevância está em alertar os profissionais da saúde sobre a importância de elencar os tumores periampulares entre os possíveis diagnósticos diferenciais para pacientes com síndrome colestática, dado que o diagnóstico e a ressecção cirúrgica precoce são os fatores decisivos para um melhor prognóstico. (AU)

Periampular tumors (PT) originate from structures close to Vater's Ampulla and correspond to a heterogeneous group of cancers. The adenocarcinoma of the major duodenal papilla is part of this group of cancers. The main manifestations are obstructive jaundice, pruritus, choluria, fecal acholia, weight loss and abdominal pain, associated with elevated serum levels of bilirubin and liver enzymes. The prognosis is poor, with pancreaticobiliar type carcinomas having the worst prognosis. Endoscopic ultrasound is the most sensitive method for diagnosis and staging, and the best treatment is pancreaticoduodenectomy, known as Whipple surgery, often associated with adjuvant chemotherapy or chemoradiation therapy. The article proposes to describe a case of adenocarcinoma of the Vater's ampulla in a 38-year-old patient treated at the University Hospital of the Federal University of Juiz de Fora (HU-UFJF), covering from the initial symptoms, the diagnostic process and the therapeutic conduct. Its relevance is to alert health professionals about the importance of listing periampular tumors among the possible differential diagnoses for patients with cholestatic syndrome, considering that the diagnosis and early surgical resection are decisive factors for a better prognosis. (AU)