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1.
Arch. argent. pediatr ; 120(1): S19-S61, feb 2022. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1353860

ABSTRACT

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.


Subject(s)
Humans , Child , Adolescent , Adult , Ascites/etiology , Jaundice , Follow-Up Studies , Gastrointestinal Hemorrhage/etiology , Liver Cirrhosis/therapy
2.
Medicina UPB ; 40(2): 75-79, 13 oct. 2021. tab
Article in Spanish | LILACS, COLNAL | ID: biblio-1342235

ABSTRACT

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


Subject(s)
Humans , Stanozolol , Anabolic Agents , Cholestasis , Testosterone Congeners , Jaundice , Liver
3.
Revista Digital de Postgrado ; 10(1): 275, abr. 2021. tab
Article in Spanish | LILACS, LIVECS | ID: biblio-1147596

ABSTRACT

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


Subject(s)
Humans , Female , Pregnancy , Pregnancy Complications/diagnosis , Fatty Liver/diagnosis , Pregnancy Complications/surgery , Pregnancy Trimester, Third , Cesarean Section , Acute Disease , HELLP Syndrome/diagnosis , Diagnosis, Differential , Fatty Liver/surgery , Fatty Liver/complications , Hypoglycemia/diagnosis , Jaundice/complications , Jaundice/diagnosis
4.
Rev. colomb. gastroenterol ; 36(supl.1): 63-66, abr. 2021. graf
Article in Spanish | LILACS | ID: biblio-1251549

ABSTRACT

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.


Subject(s)
Humans , Female , Infant , Biliary Atresia , Cytomegalovirus , Jaundice
5.
Article in Chinese | WPRIM | ID: wpr-888627

ABSTRACT

The uniformity of blue LED array in jaundice treatment box is improved. The mathematical model of illumination uniformity algorithm for inner and outer LED arrays layout is established. Taking the actual size of blue light board in jaundice treatment box as an example, the optimal illumination uniformity with best LED arrays layout are obtained through programming iteration and simulation verification. The uniformity of blue light LED improved 42.9 % comparing with tradition LED arrays.


Subject(s)
Computer Simulation , Humans , Jaundice , Light , Lighting
6.
An. Fac. Cienc. Méd. (Asunción) ; 53(3): 25-32, 20201201.
Article in Spanish | LILACS | ID: biblio-1177367

ABSTRACT

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.


Subject(s)
Vomiting , Gallstones , Lithiasis , Overweight , Jaundice , Obesity , Population , Women , Disease , Gallbladder Diseases
7.
Arq. gastroenterol ; 57(4): 361-365, Oct.-Dec. 2020. tab
Article in English | LILACS | ID: biblio-1142340

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Adult , Hepatitis, Autoimmune/diagnosis , Liver/pathology , Azathioprine/therapeutic use , Brazil/epidemiology , Prednisone/therapeutic use , Retrospective Studies , Cohort Studies , Hepatitis, Autoimmune/drug therapy , Hepatitis, Autoimmune/epidemiology , Ambulatory Care Facilities , Immunosuppressive Agents/therapeutic use , Jaundice/epidemiology , Liver Cirrhosis/epidemiology , Middle Aged
8.
Rev. chil. obstet. ginecol. (En línea) ; 85(supl.1): S101-S105, set. 2020. tab
Article in Spanish | LILACS | ID: biblio-1138654

ABSTRACT

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.


Subject(s)
Humans , Female , Pregnancy , Adult , Pneumonia, Viral/complications , Pregnancy Complications, Infectious/enzymology , Pregnancy Complications, Infectious/etiology , Coronavirus Infections/complications , Betacoronavirus , Pneumonia, Viral/enzymology , Transferases/analysis , Coronavirus Infections/enzymology , Alkaline Phosphatase/analysis , Pandemics , Jaundice , Liver Diseases/enzymology , Liver Diseases/etiology
9.
Rev. colomb. gastroenterol ; 35(3): 390-393, jul.-set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138799

ABSTRACT

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.


Subject(s)
Humans , Male , Adult , Caroli Disease , Bile Ducts, Intrahepatic , Cholangiopancreatography, Magnetic Resonance , Jaundice
10.
Pesqui. vet. bras ; 40(6): 451-465, June 2020. tab, ilus
Article in English | LILACS, VETINDEX | ID: biblio-1135646

ABSTRACT

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)


Subject(s)
Animals , Dogs , Dog Diseases , Jaundice/diagnosis , Jaundice/etiology , Jaundice/pathology , Jaundice/blood , Jaundice/veterinary , Polymerase Chain Reaction/veterinary , Ehrlichiosis/veterinary , Sepsis/veterinary , Diagnosis, Differential , Leptospirosis/veterinary , Neoplasms/veterinary
11.
Rev. Hosp. Ital. B. Aires (2004) ; 40(1): 25-28, mar. 2020. ilus
Article in Spanish | LILACS | ID: biblio-1102210

ABSTRACT

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)


Subject(s)
Humans , Male , Adult , Zygomycosis/diagnosis , Lymphohistiocytosis, Hemophagocytic/diagnosis , Pancytopenia/blood , Psychomotor Agitation , Vancomycin/therapeutic use , Norepinephrine/administration & dosage , Norepinephrine/therapeutic use , Amphotericin B/therapeutic use , Exophthalmos/diagnostic imaging , Immunocompromised Host/immunology , Colistin/therapeutic use , Amoxicillin-Potassium Clavulanate Combination/administration & dosage , Amoxicillin-Potassium Clavulanate Combination/therapeutic use , Zygomycosis/etiology , Zygomycosis/mortality , Zygomycosis/epidemiology , Delirium , Lymphohistiocytosis, Hemophagocytic/etiology , Lymphohistiocytosis, Hemophagocytic/mortality , Fever , Meropenem/therapeutic use , Immunocompetence/immunology , Jaundice , Mucormycosis/complications , Multiple Organ Failure/diagnosis
12.
Rev. méd. Minas Gerais ; 30(supl.1): S10-S12, 2020.
Article in Portuguese | LILACS | ID: biblio-1120195

ABSTRACT

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)


Subject(s)
Humans , Female , Adult , Adenocarcinoma , General Surgery , Ampulla of Vater , Carcinoma , Hospitals, University , Jaundice , Neoplasms
13.
Rev. colomb. gastroenterol ; 35(1): 76-86, 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1115602

ABSTRACT

Resumen La colestasis es uno de los motivos de consulta más frecuentes en hepatología. Se genera por una alteración en la síntesis, la secreción o el flujo de la bilis, a través del tracto biliar. Esta se define por una elevación de enzimas como la fosfatasa alcalina (Alkaline Phosphatase, ALP) y la gamma-glutamil transferasa, y en estadios tardíos con la hiperbilirrubinemia, al igual que con otras manifestaciones clínicas, tales como el prurito y la ictericia. El enfoque diagnóstico implica establecer el origen de dicha elevación, determinando si es intrahepática o extrahepática. Si es intrahepática, se debe esclarecer si proviene de los hepatocitos o de la vía biliar de pequeño y de gran calibre. El tratamiento dependerá de la etiología, por lo cual es importante un diagnóstico preciso. En esta revisión se presenta la fisiopatología y un enfoque diagnóstico y terapéutico.


Abstract Cholestasis is one of the most frequent reasons for hepatology consultation. It is generated by altered synthesis, secretion or flow of bile through the biliary tract and is defined by elevated levels of enzymes such as alkaline phosphatase and gamma glutamyl transferase. In late stages, hyperbilirubinemia and clinical manifestations such as pruritus and jaundice develop. The diagnostic approach involves establishment of the reasons for elevated enzyme levels and determination of whether it is intrahepatic or extrahepatic. If it is intrahepatic, the source must be determined (hepatocytes, small bile ducts, or large caliber bile ducts). Treatment depends on the etiology, so accurate diagnosis is important. This review presents the pathophysiology and a diagnostic and therapeutic approach.


Subject(s)
Humans , Therapeutics , Cholestasis , Diagnosis , Pruritus , Lifting , Alkaline Phosphatase , Hyperbilirubinemia , Jaundice
14.
Article in English | WPRIM | ID: wpr-811412

ABSTRACT

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.


Subject(s)
Anemia, Hemolytic , Anemia, Hemolytic, Autoimmune , Biopsy , Giant Cells , Hepatitis , Hepatocytes , Humans , Infant , Inflammation , Jaundice , Liver , Liver Diseases , Male , Pallor , Physical Examination , Rare Diseases , Recurrence , Rituximab , Splenomegaly
15.
Acta Medica Philippina ; : 632-637, 2020.
Article in English | WPRIM | ID: wpr-877361

ABSTRACT

@#We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.


Subject(s)
Butterflies , Bile Ducts, Intrahepatic , Jaundice , Musculoskeletal Abnormalities , Spine
16.
Rev. colomb. gastroenterol ; 34(4): 364-369, oct.-dic. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1092963

ABSTRACT

Resumen La hepatitis autoinmune (HAI) es una enfermedad hepática inflamatoria progresiva poco frecuente en niños y adolescentes, la cual es un reto diagnóstico para clínicos y patólogos. Describimos las características clínicas, bioquímicas e histopatológicas de 21 pacientes pediátricos con HAI diagnosticados en los últimos 14 años. Las biopsias hepáticas se reevaluaron para analizar detalladamente los hallazgos histopatológicos. De los 21 casos evaluados, 12 (57,1%) fueron mujeres, la mediana de edad fue 14 años, y 17 (80,9%) tenían HAI tipo 1. Los signos clínicos más frecuentes fueron ictericia (66,7%) y coluria (44,4%); también hubo evidencia de hipertensión portal con várices esofágicas (47,1%) y esplenomegalia (41,2%). El 11,8% de los pacientes tenía antecedentes de otras enfermedades autoinmunes. El 89,5%, 88,9% y 60,0% de los casos tenía elevación de aminotransferasas, hiperbilirrubinemia y bajos niveles de albúmina sérica, respectivamente. Las biopsias reevaluadas mostraron infiltrado linfoplasmocitario portal (94,4%), hepatitis de interfase (77,8%) y formación de rosetas (50,0%). En el 42,9% de las biopsias se hallaron inclusiones hialinas en las células de Kupffer. Cerca del 33,5% de los casos mostró cirrosis en la biopsia inicial. A pesar del tratamiento inmunosupresor, 4 pacientes requirieron trasplante hepático y 2 están en lista de espera. La HAI en niños puede manifestarse con ictericia y coluria, signos de hipertensión portal, aminotransferasas elevadas, hiperbilirrubinemia y anticuerpos circulantes. Las inclusiones hialinas en las células de Kupffer pueden ser un hallazgo útil en el diagnóstico histopatológico de la HAI en niños.


Abstract Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease. It is uncommon in children and adolescents, and is a diagnostic challenge for clinicians and pathologists. We describe the clinical, biochemical and histopathological characteristics of 21 pediatric patients with AIH diagnosed in the last 14 years. Liver biopsies were reassessed to analyze histopathological findings in detail. Of the 21 cases evaluated, 12 (57.1%) were girls and young women, the median age was 14 years old, and 17 (80.9%) had type 1 AIH. The most frequent clinical signs were jaundice (66.7%), choluria (44.4%), evidence of portal hypertension with esophageal varices (47.1%), and splenomegaly (41.2%). Histories of other autoimmune diseases were found in 11.8% of these patients. Elevated levels of aminotransferases were found in 89.5% of the patients, hyperbilirubinemia was found in 88.9%, and 60.0% of the cases had low levels of serum albumin. Reassessed biopsies showed portal lymphoplasmocytic infiltrate (94.4%), interface hepatitis (77.8%) and rosette formation (50.0%). Hyaline inclusions were found in Kupffer cells in 42.9% of the biopsies. About 33.5% of the cases showed cirrhosis at the initial biopsy. Despite immunosuppressive treatment, four patients required liver transplantation and two are on the waiting list. AIH in children can manifest with jaundice, choluria, signs of portal hypertension, elevated aminotransferases, hyperbilirubinemia and circulating antibodies. Hyaline inclusions in Kupffer cells may be a useful finding in the histopathological diagnosis of AIH in children.


Subject(s)
Humans , Male , Female , Hepatitis, Autoimmune , Splenomegaly , Biopsy , Esophageal and Gastric Varices , Hypertension, Portal , Jaundice
17.
Arch. argent. pediatr ; 117(5): 502-504, oct. 2019. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1054971

ABSTRACT

La carotenemia es, en general, benigna y suele ser causada por la ingesta excesiva de alimentos ricos en betacaroteno. Su presentación clínica puede ocurrir a cualquier edad, pero es frecuente en los niños. En este artículo, describimos un caso raro de carotenemia con afectación nasal aislada con el objetivo de recordar la presencia de carotenemia en el diagnóstico diferencial de ictericia. A menudo, la carotenemia se reconoce fácilmente y la manejan los médicos de familia y los pediatras, que podrían participar potencialmente en el tratamiento de estos casos. También es importante conocer y reconocer la carotenemia para informar a las familias y evitar la realización de pruebas innecesarias.


Carotenemia is generally benign and usually caused by excessive intake of beta-carotene rich food. This clinical presentation can occur in any age group but frequently occurs in children. We report a rare case of carotenemia with an isolated involvement of the nose and presented in order to remind carotenemia in the differential diagnosis of jaundice. Carotenemia is often easily recognized and dealt with by family medicine and pediatricians who may potentially get involved in the management of these cases. It is also important to know and recognize carotenemia in terms of informing families and preventing unnecessary tests.


Subject(s)
Humans , Female , Child, Preschool , beta Carotene , Vegetables , Vitamin A , Nose/abnormalities , Diagnosis, Differential , Fruit , Jaundice
18.
Med. leg. Costa Rica ; 36(1): 73-83, ene.-mar. 2019.
Article in Spanish | LILACS | ID: biblio-1002560

ABSTRACT

Resumen La bilirrubina es el producto final de la degradación del grupo hem. La bilirrubina no conjugada (BNC) se forma en las células retículoendoteliales, transportada al hígado, donde es conjugada a glucurónidos y secretada a los canalículos. La BNC se solubiliza en el suero por medio de su fuerte unión con la albúmina. La unión bilirrubina-albúmina es una función de las concentraciones de la albúmina y de la bilirrubina y de la afinidad de unión por la bilirrubina. La fracción de bilirrubina no unida o bilirrubina libre plasmática (Bf) se incrementa significativamente conforme el nivel de bilirrubina sérica total (BST) alcanza la capacidad de unión de la albúmina. La Bf es considerada un mejor indicador de neurotoxicidad que la BST, a causa de que solamente la bilirrubina libre puede cruzar la barrera hematoencefálica. En la práctica médica la bilirrubina es un marcador de disfunción hepática, colestasis o enfermedad hemolítica. Una variedad de factores limita la sensibilidad y la especificidad de la medición de la bilirrubina para detectar anormalidades: lipemia, hemólisis, exposición a la luz visible y el estado de ayuno. La hiperbilirrubinemia puede ser clasificada como prehepática, hepática y poshepática, y esto brinda un marco útil para identificar la causa subyacente. Además, hay bilirrubina conjugada y no conjugada. La hiperbilirrubinemia y la ictericia neonatales se presentan en casi todos los recién nacidos y puede ser benigna si su progresión a hiperbilirrubinemia es reconocida, monitoreada y prevenida o tratada en una manera oportuna.


Abstract Bilirubin is the end product of heme breakdown. Unconjugated bilirubin (UB) is formed in reticuloendothelial cells, transported to the liver where it is conjugated to glucuronides, and then secreted into the canaliculi. UB is solubilized in serum via very tight linkage to albumin. Bilirubin-albumin binding is a function of the concentration of bilirubin and albumin and the binding affinity for bilirubin. The fraction of unbound bilirubin or plasma free bilirubin (Bf) increases significantly as the total serum bilirubin (TSB) level approaches the binding capacity of albumin. Bf is thought to be better indicator of neurotoxicity than TSB, because only plasma free bilirubin can cross the blood-brain barrier. In medical practice bilirubin is a marker of liver dysfunction, cholestasis or hemolytic disease. A variety of factors limit both the sensitivity and the specificity of bilirubin measurement to detect the abnormalities: lipemia, hemolysis, exposure of visible light and fasting state. Hyperbilirubinemia can be categorised as prehepatic, hepatic or poshepatic, and this provides a useful framework for identifying the underlying cause. In addition, there are conjugated and unconjugated bilirubin. Neonatal hyperbilirubinemia and jaundice occur in almost all newborns and may be benign if its progression to extreme hyperbilirubinemia is recognized, monitored and prevented or managed in a timely manner.


Subject(s)
Humans , Bilirubin , Biomarkers , Hyperbilirubinemia , Jaundice , Liver Function Tests
19.
Article in English | WPRIM | ID: wpr-741823

ABSTRACT

Benign recurrent intrahepatic cholestasis (BRIC), a rare cause of cholestasis, is characterized by recurrent episodes of cholestasis without permanent liver damage. BRIC type 2 (BRIC2) is an autosomal recessive disorder caused by ABCB11 mutations. A 6-year-old girl had recurrent episodes of jaundice. At two months of age, jaundice and hepatosplenomegaly developed. Liver function tests showed cholestatic hepatitis. A liver biopsy revealed diffuse giant cell transformation, bile duct paucity, intracytoplasmic cholestasis, and periportal fibrosis. An ABCB11 gene study revealed novel compound heterozygous mutations, including c.2075+3A>G in IVS17 and p.R1221K. Liver function test results were normal at 12 months of age. At six years of age, steatorrhea, jaundice, and pruritus developed. Liver function tests improved following administration of phenylbutyrate and rifampicin. Her younger brother developed jaundice at two months of age and his genetic tests revealed the same mutations as his sister. This is the first report of BRIC2 confirmed by ABCB11 mutations in Korean siblings.


Subject(s)
Bile Ducts , Biopsy , Child , Cholestasis , Cholestasis, Intrahepatic , Female , Fibrosis , Giant Cells , Hepatitis , Humans , Jaundice , Liver , Liver Function Tests , Pruritus , Rifampin , Siblings , Steatorrhea
20.
Article in English | WPRIM | ID: wpr-766026

ABSTRACT

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive liver diseases that present as neonatal cholestasis. Little is known of this disease in Korea. METHODS: The records of five patients histologically diagnosed with PFIC, one with PFIC1 and four with PFIC2, by liver biopsy or transplant were reviewed, and ATP8B1 and ABCB11 mutation status was analyzed by direct DNA sequencing. Clinicopathological characteristics were correlated with genetic mutations. RESULTS: The first symptom in all patients was jaundice. Histologically, lobular cholestasis with bile plugs was the main finding in all patients, whereas diffuse or periportal cholestasis was identified only in patients with PFIC2. Giant cells and ballooning of hepatocytes were observed in three and three patients with PFIC2, respectively, but not in the patient with PFIC1. Immunostaining showed total loss of bile salt export pump in two patients with PFIC2 and focal loss in two. Lobular and portal based fibrosis were more advanced in PFIC2 than in PFIC1. ATP8B1 and ABCB11 mutations were identified in one PFIC1 and two PFIC2 patients, respectively. One PFIC1 and three PFIC2 patients underwent liver transplantation (LT). At age 7 months, one PFIC2 patient was diagnosed with concurrent hepatocellular carcinoma and infantile hemangioma in an explanted liver. The patient with PFIC1 developed steatohepatitis after LT. One patient showed recurrence of PFIC2 after 10 years and underwent LT. CONCLUSIONS: PFIC is not rare in patients with neonatal cholestasis of unknown origin. Proper clinicopathologic correlation and genetic testing can enable early detection and management.


Subject(s)
Bile , Biopsy , Carcinoma, Hepatocellular , Cholestasis , Cholestasis, Intrahepatic , Fatty Liver , Fibrosis , Genetic Testing , Giant Cells , Hemangioma , Hepatocytes , Humans , Jaundice , Korea , Liver , Liver Diseases , Liver Transplantation , Recurrence , Sequence Analysis, DNA
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