RESUMEN

BACKGROUND: In inflammatory bowel disease (IBD) patients there are reports of the occurrence of hepatobiliary manifestations, so the aim of this study was to evaluate the hepatobiliary manifestations in patients with Crohn's disease (CD) and ulcerative colitis (UC) from an IBD reference center. METHODS: Cross-sectional study in an IBD reference center, with interviews and review of medical charts, between July 2015 and August 2016. A questionnaire addressing epidemiological and clinical characteristics was used. RESULTS: We interviewed 306 patients, and the majority had UC (53.9%) and were female (61.8%). Hepatobiliary manifestations were observed in 60 (19.6%) patients with IBD. In the greater part of the patients (56.7%) hepatobiliary disorders were detected after the diagnosis of IBD. In UC (18.2%) patients, the hepatobiliary disorders identified were 11 (6.7%) non-alcoholic fatty liver disease, 9 (5.5%) cholelithiasis, 6 (3.6%) primary sclerosing cholangitis (PSC), 3 (1.8%) hepatotoxicity associated with azathioprine, 1 (0.6%) hepatitis B, and 1 (0.6%) hepatic fibrosis. In CD (21.3%) patients, 11 (7.8%) had cholelithiasis, 11 (7.8%) non-alcoholic fatty liver disease, 4 (2.8%) PSC, 3 (2.1%) hepatotoxicity, 1 (0.7%) hepatitis B, (0.7%) hepatitis C, 1 (0.7%) alcoholic liver disease, and 1 (0.7%) autoimmune hepatitis (AIH). There was one case of PSC/AIH overlap syndrome. CONCLUSION: The frequency of hepatobiliary disorders was similar in both forms of IBD in patients evaluated. The most common nonspecific hepatobiliary manifestations in IBD patients were non-alcoholic liver disease and cholelithiasis. The most common specific hepatobiliary disorder was PSC in patients with extensive UC or ileocolonic CD involvement; this was seen more frequently in male patients.

Asunto(s)

Eliminación Hepatobiliar , Enfermedades Inflamatorias del Intestino/diagnóstico , Hígado/fisiopatología , Adulto , Azatioprina/efectos adversos , Colelitiasis/diagnóstico , Colelitiasis/fisiopatología , Colitis Ulcerosa/diagnóstico , Colitis Ulcerosa/fisiopatología , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/fisiopatología , Estudios Transversales , Femenino , Hepatitis B/diagnóstico , Hepatitis B/fisiopatología , Hepatitis C/diagnóstico , Hepatitis C/fisiopatología , Hepatitis Autoinmune/diagnóstico , Hepatitis Autoinmune/fisiopatología , Humanos , Enfermedades Inflamatorias del Intestino/clasificación , Enfermedades Inflamatorias del Intestino/fisiopatología , Hepatopatías/clasificación , Hepatopatías/patología , Hepatopatías Alcohólicas/diagnóstico , Hepatopatías Alcohólicas/fisiopatología , Masculino , Persona de Mediana Edad , Enfermedad del Hígado Graso no Alcohólico/diagnóstico , Enfermedad del Hígado Graso no Alcohólico/fisiopatología , Adulto Joven

RESUMEN

Glycan-binding proteins, which include galectins, are involved at all stages of immunity and inflammation, from initiation through resolution. Galectin-9 (Gal-9) is highly expressed in the liver and has a wide variety of biological functions in innate and adaptive immunity that are instrumental in the maintenance of hepatic homeostasis. In the setting of viral hepatitis, increased expression of Gal-9 drives the expansion of regulatory T cells and contraction of effector T cells, thereby favoring viral persistence. The dichotomous nature of Gal-9 is evident in hepatocellular carcinoma, where loss of expression in hepatocytes promotes tumor growth and metastasis, whereas overexpression by Kupffer cells and endothelial cells inhibits the antitumor immune response. In nonalcoholic fatty liver disease, Gal-9 is involved indirectly in the expansion of protective natural killer T-cell populations. In ischemic liver injury, hepatocyte-derived Gal-9 is both diagnostic and cytoprotective. In drug-induced acute liver failure, plasma levels correlate with outcome. Here, we offer a synthesis of recent and emerging findings on Gal-9 in the regulation of hepatic inflammation. Ongoing studies are warranted to better elucidate the pathophysiology of hepatic immune-mediated diseases and to develop new therapeutic interventions using glycan-binding proteins. (Hepatology 2017;66:271-279).

Asunto(s)

Inmunidad Adaptativa/fisiología , Galectinas/metabolismo , Homeostasis/inmunología , Hepatopatías/inmunología , Hepatopatías/fisiopatología , Animales , Carcinoma Hepatocelular/inmunología , Carcinoma Hepatocelular/fisiopatología , Hepatitis/inmunología , Hepatitis/fisiopatología , Hepatitis Autoinmune/inmunología , Hepatitis Autoinmune/fisiopatología , Humanos , Inmunidad Innata/fisiología , Hepatopatías Alcohólicas/inmunología , Hepatopatías Alcohólicas/fisiopatología , Fallo Hepático Agudo/inmunología , Fallo Hepático Agudo/fisiopatología , Neoplasias Hepáticas/inmunología , Neoplasias Hepáticas/fisiopatología , Sensibilidad y Especificidad

RESUMEN

UNLABELLED: Reactivity and titers of autoantibodies vary during the course of autoimmune hepatitis (AIH), and some autoantibodies have been associated with disease activity and adverse outcomes after treatment. The aim of this study was to assess the autoantibody behavior in AIH and its significance as predictors of biochemical and histological remission. A total of 117 patients with AIH (mean age 18.6 [4-69] years) were evaluated and tested for autoantibodies at disease onset and successively (mean 3.2 [2-6] times) after a mean follow-up evaluation of 70 [20-185] months. Antismooth muscle (ASMA), antiliver kidney microsome type 1 (anti-LKM1), antiliver cytosol type 1 (anti-LC1), antimitochondrial, antinuclear (ANA), and antiactin antibodies (AAA) were determined at disease onset and 379 other times during the follow-up evaluation through indirect immunofluorescence in rodent tissues, HEp-2 cells, and human fibroblasts. Anti-SLA/LP were assessed 45 times in the follow-up evaluation of 19 patients using enzyme-linked immunosorbent assay (ELISA). Upon admission, AIH types 1 and 2 were observed in 95 and 17 patients, respectively. Five subjects had AIH with anti-SLA/LP as the sole markers. Patients initially negative for AAA did not develop these antibodies thereafter. ANA were detected de novo in six and three subjects with AIH types 1 and 2, respectively. After treatment, only ASMA (>1:80) and AAA (>1:40) were significantly associated with biochemical (76.9% and 79.8%) and histological features (100% and 100%) of disease activity (P < 0.001). CONCLUSION: With the exception of ANA, the autoantibody profile does not markedly vary in the course of AIH. The persistence of high titers of ASMA and/or AAA in patients with AIH is associated with disease activity.

Asunto(s)

Actinas/inmunología , Anticuerpos Antiidiotipos/sangre , Hepatitis Autoinmune/diagnóstico , Músculo Liso/inmunología , Adolescente , Adulto , Anciano , Biomarcadores/sangre , Niño , Preescolar , Femenino , Estudios de Seguimiento , Hepatitis Autoinmune/metabolismo , Hepatitis Autoinmune/fisiopatología , Humanos , Hígado/enzimología , Hígado/fisiopatología , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pronóstico , Índice de Severidad de la Enfermedad , Adulto Joven

RESUMEN

Primary biliary cirrhosis (PBC) is a cholestatic liver disease characterised by the immune-mediated destruction of biliary epithelial cells in small intrahepatic bile ducts. The disease is characterised by circulating anti-mitochondrial antibodies (AMA) as well as disease specific anti-nuclear antibodies (ANA), cholestatic liver biochemistry, and characteristic histology. The disease primarily affects middle-aged females, and its incidence is apparently increasing worldwide. Epidemiological studies have indicated several risk factors for the development of PBC, with family history of PBC, recurrent urinary tract infection, and smoking being the most widely cited. Smoking has been implicated as a risk factor in several autoimmune diseases, including the liver, by complex mechanisms involving the endocrine and immunological systems to name a few. Studies of smoking in liver disease have also shown that smoking may progress the disease towards fibrosis and subsequent cirrhosis. This review will examine the literature surrounding smoking as a risk factor for PBC, as well as a potential factor in the progression of fibrosis in PBC patients.

Asunto(s)

Hepatitis Autoinmune/epidemiología , Cirrosis Hepática Biliar/epidemiología , Fumar/efectos adversos , Progresión de la Enfermedad , Hepatitis Autoinmune/inmunología , Hepatitis Autoinmune/fisiopatología , Humanos , Cirrosis Hepática/epidemiología , Cirrosis Hepática/inmunología , Cirrosis Hepática/fisiopatología , Cirrosis Hepática Biliar/inmunología , Cirrosis Hepática Biliar/fisiopatología , Factores de Riesgo

RESUMEN

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account.

Asunto(s)

Encefalopatía Hepática/diagnóstico , Hepatitis Autoinmune/diagnóstico , Degeneración Hepatolenticular/diagnóstico , Cirrosis Hepática/diagnóstico , Antidiscinéticos/uso terapéutico , Diagnóstico Diferencial , Progresión de la Enfermedad , Femenino , Haloperidol/uso terapéutico , Encefalopatía Hepática/tratamiento farmacológico , Encefalopatía Hepática/fisiopatología , Hepatitis Autoinmune/fisiopatología , Degeneración Hepatolenticular/tratamiento farmacológico , Degeneración Hepatolenticular/fisiopatología , Humanos , Cirrosis Hepática/fisiopatología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Pronóstico , Índice de Severidad de la Enfermedad

RESUMEN

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account.

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A degeneração hepatocerebral adquirida (AHD) e a degeneração hepatolenticular podem ter apresentações clínicas semelhantes, mas quando uma doença hepática crônica e achados motores atípicos coexistem, a distinção entre AHD e encefalopatia hepática (HE) pode ser ainda mais complicada. Descrevemos três casos de AHD (dois tendo HE) com diferentes achados em neuroimagem, doenças hepáticas distintas e apresentações motoras semelhantes, todos com hipertensão arterial e perda de peso antes das manifestações motoras. O diagnóstico e a fisiopatologia são comentados e comparados com relatos prévios. Concluímos que existem muitas correlações entre HE, degeneração hepatolenticular e AHD, mas a sobreposição de HE e AHD pode ser mais comum dependendo do conhecimento clínico e da acurácia dos critérios diagnósticos adotados para cada enfermidade. Como a AHD não é considerada prioridade na lista de transplante hepático, o prognóstico dos pacientes com AHD permanece ruim, e a interrupção do fluxo nos shunts portossistêmicos deve ser sempre considerada.

Asunto(s)

Femenino , Humanos , Masculino , Persona de Mediana Edad , Encefalopatía Hepática/diagnóstico , Hepatitis Autoinmune/diagnóstico , Degeneración Hepatolenticular/diagnóstico , Cirrosis Hepática/diagnóstico , Antidiscinéticos/uso terapéutico , Diagnóstico Diferencial , Progresión de la Enfermedad , Haloperidol/uso terapéutico , Encefalopatía Hepática/tratamiento farmacológico , Encefalopatía Hepática/fisiopatología , Hepatitis Autoinmune/fisiopatología , Degeneración Hepatolenticular/tratamiento farmacológico , Degeneración Hepatolenticular/fisiopatología , Cirrosis Hepática/fisiopatología , Imagen por Resonancia Magnética , Pronóstico , Índice de Severidad de la Enfermedad

RESUMEN

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease. Characteristic liver-infiltrating immune cells in portal and periportal areas, hypergammaglobulinemia and typical autoantibodies indicate an ongoing autoimmune reaction against liver self antigens, which lead to irreversible cellular damage and ultimately to severe hepatic failure. A significant part of adult, but not pediatric AIH patients, exhibit concurrent autoimmune diseases, further strengthening the immunological etiology of the disease. Genetic susceptibility to autoimmune hepatitis is strongly associated with HLA-DRB1 alleles. In Caucasian European and North American patients, AIH-1 is associated with the presence of DRB1*0301, DRB3*0101 and DRB1*0401 alleles, while AIH-2 is associated with DRB1*0301 or DRB1*07. In Brazil, the primary susceptibility allele for AIH-1 is DRB1*1301, but a secondary association with DRB1*0301 has also been identified. We looked for additional susceptibility factors in the extended MHC region. We genotyped 107 AIH-1 children and up to 326 healthy subjects for TNFA G-308A, TNFA G-238A, LTA A+252G, LTA A+80C, NFKBIL1 T-63A, BAT1 C-348T, BAT1 G-22C, MICA, and HLA-B polymorphisms. The TNFA-308 A allele was significantly increased in AIH-1 when compared with healthy controls, confirming data from other studies. Linkage disequilibrium analysis was carried out. The ancestral haplotype comprising TNFA-308A, TNFA-238G, LTA+252G, LTA+80C, NFKBIL1-63A, BAT1-348C, BAT1-22C, HLA-B*08, MICA*08 was more common in DRB1*03 positive patients than in controls (40% vs. 14%), showing a seven-fold increased risk for the disease [OR=7.8 (95%CI 2.04-29.9.2, p=0.0021). In contrast, the remaining patients carrying DRB1*03 exhibited varied haplotypes. Finally, a variety of class III haplotypes was also present in HLA-DRB1*13 patients, without a predominant pattern. The most common of the 98 haplotypes present in patients were completely absent in controls. The extended haplotype analysis in this sample of AIH-1 patients highlights not only the genetic diversity present in the Brazilian population, but is also in accordance with the previously documented microdiversity within the MHC region. The present knowledge of AIH suggests that the same or a very similar disease can be induced by yet unknown, but different, triggers followed by presentation on different HLA-DR molecules of the epitopes derived from the corresponding autoantigens, characterizing a much more complex disease than previously thought.

Asunto(s)

Antígenos HLA/genética , Antígenos HLA/inmunología , Haplotipos , Hepatitis Autoinmune/genética , Hepatitis Autoinmune/inmunología , Predisposición Genética a la Enfermedad , Hepatitis Autoinmune/fisiopatología , Humanos

RESUMEN

AIM: To evaluate the overlap of autoimmune hepatitis in hepatitis C virus (HCV)-infected patients with intense interface hepatitis. METHODS: Among 1759 patients with hepatitis C submitted to liver biopsy, 92 (5.2%) presented intense interface hepatitis. These patients were evaluated regarding the presence of antinuclear antibody (ANA), anti-smooth muscle antibody (SMA) and anti-liver/kidney microsomal antibody (LKM-1), levels of gamma-globulin and histological findings related to autoimmune hepatitis (plasma cell infiltrate and presence of rosettes). RESULTS: Among patients with hepatitis C and intense interface hepatitis there was a low prevalence of autoantibodies (ANA = 12%, SMA = 5%, LKM-1 = 0%) and the median gamma-globulin level was within the normal range. Typical histological findings of autoimmune disease were observed in only two cases (2%). After applying the score for diagnosis of autoimmune hepatitis, only one patient was classified with a definitive diagnosis of autoimmune hepatitis. Since overlap with autoimmune hepatitis was not the explanation for the intense necroinflammatory activity in patients with chronic hepatitis C we sought to identify the variables associated with this finding. The presence of intense interface hepatitis was associated with more advanced age, both at the time of infection and at the time of the biopsy, and higher prevalence of blood transfusion and alcohol abuse. CONCLUSION: Although possible, overlap with autoimmune hepatitis is a very rare association in HCV-infected patients with intense interface hepatitis, an unusual presentation which seems to be related to other host variables.

Asunto(s)

Hepatitis C/fisiopatología , Hepatitis Autoinmune/fisiopatología , Adulto , Autoanticuerpos/sangre , Biopsia , Femenino , Hepatitis C/sangre , Hepatitis C/inmunología , Hepatitis C/patología , Hepatitis Autoinmune/sangre , Hepatitis Autoinmune/inmunología , Hepatitis Autoinmune/patología , Humanos , Masculino , Persona de Mediana Edad

RESUMEN

El diagnóstico de la hepatitis autoinmune (HAI), se basa en una serie de criterios definidos por elGrupo Internacional de HAI (IAIHG) que permite clasificarla como HAI probable o definitiva. Un criterio clave para el diagnóstico de la HAI es la detección de ANA, SMA, y anti- LKM-1 por inmunofluorescencia indirecta. Otros anticuerpos menos frecuentes probados, pero de importancia diagnóstica en HAI pediátrica incluyen los anticuerpos tipo: citosol 1 hígado (LC-1), anti- citoplasma de los neutrófilos (ANCA) y el antígeno soluble hepático (SLA). La Ig G está usualmente elevada en ambos tipos de HAI, cerca del 15% de niños con HAI I y el 25% de niños con HAI tipo II tienen valores normales. La biopsia hepática es necesaria para establecer el diagnóstico de HAI.

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The diagnosis of autoimmune hepatitis (HAI), is based on a set of criteria defined by the International HAI Group (IAIHG) that allows classified as a probable or definite HAI. A key criterion for the diagnosis of HAI is the detection of ANA, SMA, and anti-LKM-1 by indirectimmunofluorescence. Other less common antibodies tested, but important diagnostic tool in pediatric HAI include antibodies such as: liver cytosol 1 (LC-1), anti-neutrophil cytoplasmic (ANCA) and soluble liver antigen (SLA). The Ig G is usually high in both types of HAI, about 15% of children with HAI I and 25% of children with HAI type II are normal. Liver biopsy is necessary to establish the diagnosis of HAI.

Asunto(s)

Humanos , Masculino , Femenino , Niño , Hepatitis Autoinmune/epidemiología , Hepatitis Autoinmune/fisiopatología , Hepatitis Autoinmune/inmunología , Hepatitis Autoinmune/microbiología , Hepatitis Autoinmune/prevención & control , Hepatitis Autoinmune/psicología , Hepatitis Autoinmune/sangre , Hepatitis Autoinmune/virología , Hepatitis Autoinmune/clasificación , Hepatitis Autoinmune/complicaciones , Hepatitis Autoinmune/diagnóstico

RESUMEN

OBJECTIVE: To evaluate the food intake, anthropometry, body composition, and sexual maturity of children and adolescents with autoimmune hepatitis. METHODS: Thirty-seven children and adolescents with autoimmune hepatitis were studied. A questionnaire was given to evaluate food intake over a 24-hour period. Weight, height, and skin-fold thickness were measured. Electric impedance and skin-fold using Slaughter formula were used to evaluate body composition. Sexual maturity was evaluated using the Tanner stage method. Cumulative intake of corticosteroids was determined based on medical records. RESULTS: Most of the subjects were females (83.3%). Food intake did not meet recommended dietary intakes for energy, calcium, and vitamin A for 43.2%, 94.6%, and 59.4% of the patients, respectively. All subjects were in their respective pubertal developmental stage. A lower Z score for height-for-age (

Asunto(s)

Composición Corporal , Dieta , Hepatitis Autoinmune , Maduración Sexual , Adolescente , Antropometría , Calcio/administración & dosificación , Niño , Estudios Transversales , Ingestión de Alimentos , Impedancia Eléctrica , Ingestión de Energía , Femenino , Estudios de Seguimiento , Glucocorticoides/administración & dosificación , Glucocorticoides/efectos adversos , Glucocorticoides/uso terapéutico , Hepatitis Autoinmune/tratamiento farmacológico , Hepatitis Autoinmune/fisiopatología , Humanos , Masculino , Política Nutricional , Encuestas y Cuestionarios , Vitamina A/administración & dosificación , Adulto Joven

RESUMEN

Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneously evaluated the prevalence of JSLE-AIH in a large JLSE and AIH population in groups of Hepatology and Rheumatology of a tertiary Paediatric University Hospital. In a 24-year period, 228 patients were diagnosed with JSLE (ACR criteria). In the same period, 252 patients were diagnosed with AIH according to the International Autoimmune Hepatitis Group. In this article, we present the demographic data, clinical features, laboratory exams and treatment of four children with both the diseases. The prevalence was 1.8% in JSLE population and was 1.6% in AIH population. The current median age was 15.5 years and three were females. In three of them, the diagnosis of AIH preceded JSLE. All of them had increased liver enzymes with a characteristic liver biopsy of AIH and responded to the combination of prednisone, azathioprine and antimalarial drugs. In conclusion, the presence of AIH-JSLE associated disorder was rarely observed. The liver biopsy could be necessary in patients with JLSE with a persistent increase of liver enzymes.

Asunto(s)

Hepatitis Autoinmune , Lupus Eritematoso Sistémico , Adolescente , Niño , Femenino , Hepatitis Autoinmune/inmunología , Hepatitis Autoinmune/fisiopatología , Humanos , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Adulto Joven

RESUMEN

CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA) were utilized for data treatment, regarding significant difference if P<0.05. RESULTS: The highest nutritional deficit was observed in patients with biliary atresia, mainly with cholestasis. The serum levels of vitamins A and E for the reference group changed as a function of age. The serum levels of vitamins A, D and E were higher in reference group than in patients with biliary atresia and autoimmune hepatitis together or separately. There were not difference in the serum levels of vitamins A, D and E between biliary atresia groups with cholestasis and without cholestasis. It was verified correlation between weight/age, triceps skinfold thickness, subscapular skinfold thickness, midarm circumference, midarm fat area values and vitamin A serum levels, as well as between all anthropometric indicators and vitamin E in patients with autoimmune hepatitis and biliary atresia. CONCLUSION: The patients with biliary atresia and cholestasis presented the highest nutritional injury. The patients with biliary atresia and autoimmune hepatitis presented lower serum levels of vitamins A, D and E that in control group. There is a directly proportional correlation between vitamin serum levels, mainly vitamin E, and all anthropometric variables of biliary atresia and autoimmune hepatitis groups.

Asunto(s)

Atresia Biliar/sangre , Hepatitis Autoinmune/sangre , Estado Nutricional/fisiología , Vitamina A/sangre , Vitamina D/sangre , Vitamina E/sangre , Adolescente , Antropometría , Atresia Biliar/fisiopatología , Niño , Preescolar , Métodos Epidemiológicos , Femenino , Hepatitis Autoinmune/fisiopatología , Humanos , Masculino

RESUMEN

CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA) were utilized for data treatment, regarding significant difference if P<0.05. RESULTS: The highest nutritional deficit was observed in patients with biliary atresia, mainly with cholestasis. The serum levels of vitamins A and E for the reference group changed as a function of age. The serum levels of vitamins A, D and E were higher in reference group than in patients with biliary atresia and autoimmune hepatitis together or separately. There were not difference in the serum levels of vitamins A, D and E between biliary atresia groups with cholestasis and without cholestasis. It was verified correlation between weight/age, triceps skinfold thickness, subscapular skinfold thickness, midarm circumference, midarm fat area values and vitamin A serum levels, as well as between all anthropometric indicators and vitamin E in patients with autoimmune hepatitis and biliary atresia. CONCLUSION: The patients with biliary atresia and cholestasis presented the highest nutritional injury. The patients with biliary atresia and autoimmune hepatitis presented lower serum levels of vitamins A, D and E that in control group. There is a directly proportional correlation between vitamin serum levels, mainly vitamin E, and all anthropometric...

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CONTEXTO: As doenças hepáticas crônicas podem induzir à má absorção de lipídios e vitaminas lipossolúveis e levar ao comprometimento do estado nutricional. OBJETIVOS: Avaliar o estado nutricional e relacionar com os níveis séricos de vitaminas (A, D e E) e a gravidade da doença em pacientes com atresia biliar e hepatite autoimune na faixa etária pediátrica. MÉTODOS: O estudo foi transversal controlado e foram avaliados os pacientes com hepatite autoimune e atresia biliar e um grupo controle pareado por sexo e idade. Foi realizada avaliação antropométrica, aplicação do inquérito alimentar e determinação dos níveis séricos das vitaminas A, D e E pela técnica de cromatografia líquida de alta eficiência. Foram empregados os testes de Mann-Whitney, o coeficiente de correlação de Spearman e análise de variância (ANOVA), sendo considerada diferença significativa se P<0,05. RESULTADOS: O déficit nutricional mais grave foi observado nos pacientes com atresia biliar, principalmente com colestase. Em relação às vitaminas, no grupo controle, constatou-se que os níveis séricos das vitaminas A e E variaram com a idade. Os níveis séricos das vitaminas A, D e E foram maiores no grupo controle em relação aos pacientes com atresia biliar e hepatite autoimune em conjunto ou separadamente. Verificou-se a correlação do peso/idade, prega cutânea tricipital, prega cutânea subescapular, circunferência braquial, área adiposa braquial com a vitamina A e de todos os indicadores antropométricos com a vitamina E nos pacientes com hepatite autoimune e atresia biliar em conjunto. CONCLUSÕES: Os pacientes com atresia biliar e colestase apresentaram o maior comprometimento nutricional. Os pacientes com atresia biliar e hepatite autoimune possuíram menores níveis séricos das vitaminas A, D e E do que o grupo controle. Existe uma correlação diretamente proporcional, principalmente da vitamina E com todos as variáveis antropométricas do grupo de AB e HAI em conjunto.

Asunto(s)

Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Atresia Biliar/sangre , Hepatitis Autoinmune/sangre , Estado Nutricional/fisiología , Vitamina A/sangre , Vitamina D/sangre , Vitamina E/sangre , Antropometría , Atresia Biliar/fisiopatología , Métodos Epidemiológicos , Hepatitis Autoinmune/fisiopatología

RESUMEN

Autoimmune hepatitis is a rare condition that is more common among women than men. An association between pregnancy and autoimmune hepatitis is rare. This clinical scenario requires the gastroenterologist and hepatologist to have a profound knowledge of clinical course counseling and medical management. The prognosis in well-controlled and closely monitored patients is good. In this review, we discuss the most important aspects of autoimmune hepatitis and pregnancy as part of the Symposium on Liver and Pregnancy, co-sponsored by the Mexican Association of Hepatology and the Mexican Association of Gynecologists and Obstetrics.

Asunto(s)

Hepatitis Autoinmune/fisiopatología , Complicaciones del Embarazo/fisiopatología , Femenino , Hepatitis Autoinmune/terapia , Humanos , Masculino , Embarazo , Complicaciones del Embarazo/terapia , Resultado del Embarazo , Resultado del Tratamiento

Asunto(s)

Humanos , Adulto , Femenino , Estudio Comparativo , Hepatitis Autoinmune/diagnóstico , Hepatitis Autoinmune/etiología , Hepatitis Autoinmune/epidemiología , Hepatitis Autoinmune/fisiopatología , Hepatitis Autoinmune/tratamiento farmacológico , Hepatitis Autoinmune/terapia , Hepatitis Autoinmune/diagnóstico por imagen , Hepatitis Autoinmune/sangre , Colestasis Intrahepática/diagnóstico , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/terapia , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/fisiopatología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/etiología , Lupus Eritematoso Sistémico/genética , Biopsia con Aguja/estadística & datos numéricos , Técnicas de Laboratorio Clínico , Diagnóstico Diferencial , Glucocorticoides/uso terapéutico

Asunto(s)

Humanos , Femenino , Hepatitis Autoinmune/fisiopatología , Biopsia/estadística & datos numéricos , Diagnóstico Diferencial , Hepatitis Autoinmune/tratamiento farmacológico , Hepatitis Autoinmune/patología

RESUMEN

The aim of this study was to compare major histocompatibility complex (MHC) class II susceptibility to type 1 autoimmune hepatitis (AH) between children and adults of the same ethnic group. HLA-DRB1, HLA-DRB3, HLA-DQA1, and HLA-DQB1 gene subtypes were examined by high resolution oligonucleotide typing in 122 pediatric (PAH) and 84 adult (AAH) patients and in 208 controls. In children, HLA-DRB1*1301 was the primary susceptibility allele (66.4% patients vs. 10.6% controls, relative risk [RR] = 16.3, Pc < 10(-24)) whereas HLA-DRB1*1302, which differs from HLA-DRB1*1301 by only 1 amino acid, appeared to be protective. The exclusion of individuals with HLA-DRB1*1301 from control and pediatric patients allowed us to find a secondary association of PAH with HLA-DRB1*0301. Possession of HLA-DRB1*1301, however, was associated with a lower therapeutic response rate. Analysis of peptide binding pocket residues indicated that Tyr 10, Ser 11, Ser 13, and Val 86 in the class II beta chain were present in 85% of patients compared with 37% of controls, suggesting that a high proportion of AH susceptibility is attributable to these residues (etiologic fraction [EF] = 76%). In contrast to the class II associations in children, AAH was associated with HLA-DRB1*0405 (RR = 10.4, Pc <.005) but not with HLA-DRB1*1301 or HLA-DRB1*0301. In addition, HLA-DR4 with the class I gene, HLA-A11, appeared synergistic in predisposing AAH patients to develop extra-hepatic autoimmune (AI) manifestations (odds ratio [OR] = 104.9, Pc < 10(-4)). Concomitant differences in autoantibody profiles were also observed in PAH versus AAH: smooth muscle antibodies (SMA) were most prevalent in PAH but antinuclear antibodies were most prevalent in AAH (P =.003). This study therefore reveals that different HLA-DRB1 allotypes confer susceptibility to AH in children and adults and raises the possibility that PAH and AAH may be triggered by different factors.

Asunto(s)

Genes MHC Clase II , Predisposición Genética a la Enfermedad/genética , Hepatitis Autoinmune/genética , Hepatitis Autoinmune/inmunología , Adulto , Edad de Inicio , Alelos , Secuencias de Aminoácidos , Argentina , Autoanticuerpos/inmunología , Niño , Etnicidad/genética , Antígenos HLA-DQ/genética , Antígenos HLA-DQ/inmunología , Antígenos HLA-DR/química , Antígenos HLA-DR/genética , Antígenos HLA-DR/inmunología , Haplotipos , Hepatitis Autoinmune/fisiopatología , Hepatitis Autoinmune/terapia , Humanos , Desequilibrio de Ligamiento/genética , Recurrencia , Tasa de Supervivencia , Resultado del Tratamiento