O tipo político fascista e o esporte: uma abordagem epistemológica / The fascist political type and sport: an epistemological approach / El tipo político fascista y el deporte: un enfoque epistemológico

RESUMO O presente artigo objetivou investigar à luz da teoria de Adorno como o esporte é utilizado na propaganda bolsonarista. O trabalho tem um caráter teórico-empírico, cujos aspectos do pensamento de Adorno tornam-se instrumento para análise do fenômeno esportivo. Metodologicamente, foram analisadas as publicações do perfil oficial do Instagram de Jair Bolsonaro que fizessem alguma menção ao esporte. Verificou-se que há uma vinculação entre o esporte de alto rendimento e a propaganda fascista difundida pelo perfil de Bolsonaro. Concluiu-se que a propaganda fascista incorpora estratégias de cunho psicológico e anti-intelectual visando atingir às massas na propagação de fatos desvinculados de seu contexto e pseudoverdades, com o propósito de penetração das ideias fascistas.

ABSTRACT This article aimed to investigate, in the light of Adorno's theory, how sport is used in Bolsonarist propaganda. The work has a theoretical-empirical character, whose aspects of Adorno's thought become an instrument for analyzing the sporting phenomenon. Methodologically, publications from Jair Bolsonaro's official Instagram profile that made any mention of sport were analyzed. It was found that there is a link between high-performance sport and the fascist propaganda spread by Bolsonaro's profile. It was concluded that fascist propaganda incorporates psychological and anti-intellectual strategies aimed at reaching the masses in the propagation of facts disconnected from their context and pseudo-truths, with the purpose of penetrating fascist ideas.

RESUMEN Este artículo tuvo como objetivo investigar a la luz de la teoría de Adorno cómo el deporte es utilizado en la propaganda bolsonarista. La obra tiene un carácter teórico-empírico cuyos aspectos del pensamiento de Adorno convertirse en análisis del fenómeno deportivo. Metodológicamente se analizaron las publicaciones del perfil oficial de Instagram de Jair Bolsonaro que hicieran mención al deporte. Se constató que existe un vínculo entre el deporte de alto rendimiento y la propaganda fascista difundida por el perfil de Bolsonaro. Se concluyó que la propaganda fascista incorpora estrategias psicológicas y antiintelectuales encaminadas a llegar a las masas en la propagación de hechos desconectados de su contexto y pseudoverdades con el propósito de penetrar ideas fascistas.

Antibiotic indication in endodontics by Colombian dentists with different levels of training: a survey / Indicação dos antibióticos em Endodontia por dentistas colombianos com diferentes níveis de formação: uma pesquisa por questionário

ABSTRACT Aim: This study investigated how Colombian dentists with different academic levels indicate antibiotics with therapeutic purposes in endodontics. Materials and method: A cross-sectional survey was conducted among 559 dentists in the form of an online questionnaire. Results: Three hundred and twenty questionnaires were answered (57.2%). There were significant differences among respondents. For irreversible pulpitis, 140 dentists (43.7%) said they prescribe antibiotics (57.5% of general practitioners, 20.1% of specialists and 38.9% of those with Master's and/or PhD degrees), while for symptomatic apical periodontitis, 183 (57.2%) did so (74.1% of general practitioners, 28.4% of specialists and 50.0% of those with Master's and/or PhD degrees) (p < 0.05). Amoxicillin was the most frequently prescribed antibiotic, and its association with clavulanic acid was the most often cited for acute periradicular abscess with systemic involvement. Conclusions: The greatest misunderstandings in prescribing antibiotics occurred among general practitioners. Considering all clinical conditions that do not require antibiotics, 60% of general practitioners and 34% of specialists, on average, indicated antibiotics.

RESUMO Objetivo: Este estudo investigou como dentistas colombianos com diferentes níveis acadêmicos indicaram antibióticos com fins terapêuticos em Endodontia. Materiais e método: Realizou-se um levantamento transversal com 559 dentistas. Foi enviado um questionário online. Resultados: Foram respondidos 320 questionários (57,2%). Houve diferenças significativas entre os profissionais com diferentes níveis de formação. Para pulpite irreversível, 140 (43,7%) dentistas afirmaram indicar antibióticos (57,5% clínicos gerais, 20,1% especialistas e 38,9% com mestrado e/ou doutorado), enquanto para periodontite apical sintomática, 183 (57,2%) prescrevem estes medicamentos (74,1% clínicos, 28,4% especialistas e 50,0% com mestrado e doutorado) (p < 0,05). A amoxicilina foi a mais indicada entre os profissionais, e sua associação com ácido clavulânico foi a mais referida para abscesso perirradicular agudo com acometimento sistêmico. Conclusões: Os maiores equívocos na prescrição de antibióticos ocorreram com os clínicos gerais. Considerando todas as condições clínicas que não requerem antibióticos, 60% dos clínicos gerais e 34% dos especialistas, em média, indicaram estes medicamentos.

Antibiotic indication in endodontics by Colombian dentists with different levels of training: a survey.

AIM: This study investigated how Colombian dentists with different academic levels indicate antibiotics with therapeutic purposes in endodontics. MATERIALS AND METHOD: A cross-sectional survey was conducted among 559 dentists in the form of an online questionnaire. RESULTS: Three hundred and twenty questionnaires were answered (57.2%). There were significant differences among respondents. For irreversible pulpitis, 140 dentists (43.7%) said they prescribe antibiotics (57.5% of general practitioners, 20.1% of specialists and 38.9% of those with Master's and/or PhD degrees), while for symptomatic apical periodontitis, 183 (57.2%) did so (74.1% of general practitioners, 28.4% of specialists and 50.0% of those with Master's and/or PhD degrees) (p<0.05). Amoxicillin was the most frequently prescribed antibiotic, and its association with clavulanic acid was the most often cited for acute periradicular abscess with systemic involvement. CONCLUSIONS: The greatest misunderstandings in prescribing antibiotics occurred among general practitioners. Considering all clinical conditions that do not require antibiotics, 60% of general practitioners and 34% of specialists, on average, indicated antibiotics.

OBJETIVO: Este estudo investigou como dentistas colombianos com diferentes níveis acadêmicos indicaram antibióticos com fins terapêuticos em Endodontia. Materiais e método: Realizou-se um levantamento transversal com 559 dentistas. Foi enviado um questionário online. RESULTADOS: Foram respondidos 320 questionários (57,2%). Houve diferenças significativas entre os profissionais com diferentes níveis de formação. Para pulpite irreversível, 140 (43,7%) dentistas afirmaram indicar antibióticos (57,5% clínicos gerais, 20,1% especialistas e 38,9% com mestrado e/ou doutorado), enquanto para periodontite apical sintomática, 183 (57,2%) prescrevem estes medicamentos (74,1% clínicos, 28,4% especialistas e 50,0% com mestrado e doutorado) (p < 0,05). A amoxicilina foi a mais indicada entre os profissionais, e sua associação com ácido clavulânico foi a mais referida para abscesso perirradicular agudo com acometimento sistêmico. Conclusões: Os maiores equívocos na prescrição de antibióticos ocorreram com os clínicos gerais. Considerando todas as condições clínicas que não requerem antibióticos, 60% dos clínicos gerais e 34% dos especialistas, em média, indicaram estes medicamentos.

Depressão em médicos da Estratégia de Saúde da Família no município de Itajaí/SC / Depression in physicians of the Family Health Strategy in the municipality of Itajaí/SC / Depresión en médicos de la Estrategia de Salud de la Familia en el municipio de Itajaí/SC

Objetivo: Conhecer a prevalência de depressão em médicos da Estratégia de Saúde da Família (ESF) da cidade de Itajaí/SC. Métodos: Foram utilizados o Inventário de Depressão de Beck para rastreamento dos sintomas depressivos e um questionário elaborado pelos pesquisadores para coletar informações da vida pessoal e profissional. Os dados coletados foram armazenados em tabela de Microsoft Excel, estratificados conforme intensidade dos sintomas e as variáveis relacionáveis. Para apresentação, foram calculadas frequências absolutas e percentuais. Resultados: Em uma amostra de 45 médicos, apenas sintomas depressivos leves foram identificados, o que representou somente 13% dos participantes, porém nenhuma das variáveis analisadas neste estudo apresentou associação com depressão. A grande maioria dos resultados se mostrou satisfatoriamente positiva para prevenção e fatores estressores: horas de sono suficientes, número adequado de consultas, satisfação com o trabalho, prática de atividade física e não relatar antecedentes psiquiátricos. Conclusões: São percebidos, no presente estudo, poucos médicos na ESF de Itajaí/SC com sintomas depressivos, contudo, considera-se, ainda, fundamental a adoção de estratégias na melhoria da saúde mental destes profissionais. (AU)

Objective: Discover the prevalence of depression in physicians of the Family Health Strategy (FHS) of the city of Itajaí/SC. Methods: The Beck Depression Inventory was used to track depressive symptoms and a questionnaire developed by the researchers was used to collect information about personal and professional life. Data collected were stored in a Microsoft Excel table, stratified according to symptom intensity and relatable variables. Absolute and percentage frequencies were calculated for presentation. Results: In a sample of 45 physicians, only mild depressive symptoms were identified, which represented only 13% of the participants, but none of the variables analyzed in this study was associated with depression. The great majority of the results were satisfactorily positive for prevention and stressors: sufficient sleep hours, adequate number of consultations, satisfaction with work, practice of physical activity and no report of psychiatric records. Conclusions: In the present study, few physicians in the FHS01 of Itajaí/SC were found with depressive symptoms, however, the adoption of strategies to improve the mental health of these professionals is still considered essential.(AU)

Objetivo: Conocer la prevalencia de depresión en médicos de la Estrategia de Salud de la Familia (ESF) de la ciudad de Itajaí/SC. Métodos: Se utilizaron el Inventario de Depresión de Beck para el rastreo de los síntomas depresivos y un cuestionario elaborado por los investigadores para recoger informaciones de la vida personal y profesional. Los datos recolectados fueron almacenados en una tabla de Microsoft Excel, estratificados según la intensidad de los síntomas y las variables relacionables. Para la presentación se calcularon frecuencias absolutas y porcentuales. Resultados: En una muestra de 45 médicos, sólo se identificaron síntomas depresivos ligeros, lo que representó sólo el 13% de los participantes, pero ninguna de las variables analizadas en este estudio presentó asociación con depresión. La gran mayoría de los resultados se mostró satisfactoriamente positiva para prevención y factores estresores: horas de sueño suficientes, número adecuado de consultas, satisfacción con el trabajo, práctica de actividad física y no relatar antecedentes psiquiátricos. Conclusiones: Se percibe en el presente estudio, pocos médicos en la ESF de Itajaí/SC con síntomas depresivos, sin embargo, se considera todavía fundamental la adopción de estrategias en la mejora de la salud mental de estos profesionales.

Histopathological and clinical evaluation of chronic spontaneous urticaria patients with neutrophilic and non-neutrophilic cutaneous infiltrate.

BACKGROUND: Chronic urticaria has an expressive prevalence in general population, especially in adults, and is defined by the presence of intermittent hives for six weeks or longer. Our study aims to characterize the histological patterns of chronic spontaneous urticaria, based on the inflammatory cell infiltrate, and correlate them to laboratory exams. METHODS: It was performed a retrospective analysis of laboratory, histopathology and direct immunofluorescence data of 93 patients with chronic urticaria. For histopathological analysis, cell count was performed in four fields at high magnification (×400) for each specimen. The resulting cell count medians were submitted to statistical analysis and, then, were correlated to laboratorial findings. RESULTS: We found a female predominance (76.34%) of chronic urticaria cases, and an average age of 42.5 years (SD ± 15). Two histological groups were distinctive: 1) chronic urticaria with predominance of neutrophils or eosinophils - N (%) = 39 (42.4%) - and 2) chronic urticaria with predominance of lymphocytes - N (%) = 53 (57.6%). There was not significant correlation between histological groups and laboratorial tests. Moreover, direct immunofluorescence was positive in 21 (33,87%) from 62 patients. CONCLUSIONS: There is not enough scientific evidence to support neutrophilic urticaria as a solid, separate entity.

Sweet Syndrome: Clinical Features, Histopathology, and Associations of 83 Cases.

BACKGROUND: Sweet syndrome (SS) is an infrequent skin disease characterised by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous plaques infiltrated by neutrophils. Multiple conditions have been associated with this syndrome. OBJECTIVES: The aim of this study was to evaluate the clinical, epidemiological, laboratory, and histopathological findings and associations of patients with SS. METHODS: We conducted a retrospective study of 83 patients with SS followed between January 1, 2006, and January 31, 2015. RESULTS: Of the patients, 82% were female; the mean age at onset was 48 years. Clinical presentation was mainly characterised by erythematous and edematous plaques, mostly on upper extremities and trunk. Fever was observed in 32%; 60% presented leukocytosis and 39% neutrophilia. On histopathological examination, neutrophilic and lymphohistiocytic infiltrate and edema were the most frequent findings. Fourteen percent of patients had malignancy or hematologic disorders, 26% were classified as having drug-induced SS, and 24% noted recent infection. Only 2 cases occurred during pregnancy. Systemic corticosteroid was the most common choice of treatment, with excellent response. In malignancy-associated SS, the mean hemoglobin level was lower ( P = .01) and the erythrocyte sedimentation rate (ESR) was higher ( P = .04) in comparison to classic and drug-induced SS. Leukocytoclasia was associated with higher risk of recurrence ( P = .01). CONCLUSION: All patients with SS deserve careful investigation of possible underlying conditions. Higher ESR and lower hemoglobin levels might reinforce the need of malignancy screening. Also, leukocytoclasia appears to be a potential marker of higher recurrence rate, demanding closer and longer follow-up.

Steroid sulfatase in the human MG-63 preosteoblastic cell line: Antagonistic regulation by glucocorticoids and NFκB.

Steroid sulfatase (STS) converts sulfated steroids into active forms in cells. Preosteoblastic cells possess STS, but its role and regulation in bone are unclear. We examined STS activity and gene expression during differentiation of human MG-63 preosteoblasts. STS activity and gene expression were decreased during differentiation in cells treated with osteogenic supplement containing dexamethasone (DEX). DEX also inhibited STS activity and expression in undifferentiated cells, and the glucocorticoid antagonist RU486 reversed DEX inhibition of STS. These data may have implications for glucocorticoid-induced osteoporosis. The NFκB activators lipopolysaccharide and phorbol myristate acetate increased STS expression in undifferentiated and differentiated MG-63 cells, while the NFκB inhibitor BAY-11-7082 partially blocked these responses. The antagonistic actions of glucocorticoids and NFkB on STS expression are similar to the regulation of inflammatory response proteins. We propose a model of STS regulation whereby inflammation leads to increased STS, resulting in increased estrogen, which modulates the inflammatory response.

Inflammatory regulation of steroid sulfatase: A novel mechanism to control estrogen homeostasis and inflammation in chronic liver disease.

BACKGROUND & AIMS: Chronic inflammatory liver diseases are associated with estrogen excess and feminization in men, which is thought to be due to compromised liver function to break down estrogens. The goal of this study is to determine whether the inflammatory induction of steroid sulfatase (STS), which converts inactive estrogen sulfates to active estrogens, may have contributed to the estrogen excess in chronic liver disease. METHODS: We performed bioinformatic analysis, real-time PCR, immunohistochemistry, and UPLC/MS-MS to analyze hepatic STS expression and serum estrogen levels in patients with chronic liver diseases. The crosstalk between NF-κB pathway and STS-regulated estrogen signaling was investigated by electrophoretic mobility shift assay, chromatin immunoprecipitation, luciferase assay and gene knockdown experiments in human hepatocytes. RESULTS: Hepatic STS was induced in patients with chronic inflammatory liver diseases, which was accompanied by increased circulating estrogen levels. The human STS gene, but not the mouse Sts gene, was induced by inflammatory stimuli in hepatic cells. Mechanistically, STS was established as a novel NF-κB target gene, whose induction facilitated the conversion of inactive estrogen sulfates to active estrogens, and consequently attenuated the inflammatory response. In contrast, genetic or pharmacological inhibition of STS or a direct blockade of estrogen signaling sensitized liver cells to the transcriptional activation of NF-κB and inflammatory response, possibly through the inhibition of IκB kinase activation. CONCLUSIONS: Our results suggest a negative feedback loop in chronic inflammatory liver diseases, in which the inflammatory activation of NF-κB induces STS gene expression. The induced STS facilitates the conversion of inactive estrogen sulfates to active estrogens, which in return attenuates the NF-κB-mediated inflammation.

Lack of mutations in the leptin receptor gene in severely obese children.

OBJECTIVE: To analyze the LEPR gene in obese children and to investigate the associations between molecular findings and anthropometric and metabolic features. SUBJECTS AND METHODS: Thirty-two patients were evaluated regarding anthropometric characteristics, blood pressure, heart rate, serum glucose, insulin, leptin levels, and lipid profile. The molecular study consisted of the amplification and automatic sequencing of the coding region of LEPR in order to investigate new mutations. RESULTS: We identified a high prevalence of metabolic disorders: impaired fasting glucose in 12.5% of the patients, elevated HOMA-IR in 85.7%, low HDL-cholesterol levels in 46.9%, high triglyceride levels in 40.6%, and hypertension in 58.6% of the patients. The molecular study identified 6 already described allelic variants: rs1137100 (exon-2), rs1137101 (exon-4), rs1805134 (exon-7), rs8179183 (exon-12), rs1805096 (exon-18), and the deletion/insertion of the pentanucleotide CTTTA at 3'untranslated region. CONCLUSIONS: The frequency of alleles observed in this cohort is similar to that described in the literature, and was not correlated with any clinical feature. The molecular findings in the analysis of the LEPR did not seem to be implicated in the etiology of obesity in these patients.

Lack of mutations in the leptin receptor gene in severely obese children / Ausência de mutação no gene receptor de leptina em crianças gravemente obesas

OBJECTIVE: To analyze the LEPR gene in obese children and to investigate the associations between molecular findings and anthropometric and metabolic features. SUBJECTS AND METHODS: Thirty-two patients were evaluated regarding anthropometric characteristics, blood pressure, heart rate, serum glucose, insulin, leptin levels, and lipid profile. The molecular study consisted of the amplification and automatic sequencing of the coding region of LEPR in order to investigate new mutations. RESULTS: We identified a high prevalence of metabolic disorders: impaired fasting glucose in 12.5% of the patients, elevated HOMA-IR in 85.7%, low HDL-cholesterol levels in 46.9%, high triglyceride levels in 40.6%, and hypertension in 58.6% of the patients. The molecular study identified 6 already described allelic variants: rs1137100 (exon-2), rs1137101 (exon-4), rs1805134 (exon-7), rs8179183 (exon-12), rs1805096 (exon-18), and the deletion/insertion of the pentanucleotide CTTTA at 3'untranslated region. CONCLUSIONS: The frequency of alleles observed in this cohort is similar to that described in the literature, and was not correlated with any clinical feature. The molecular findings in the analysis of the LEPR did not seem to be implicated in the etiology of obesity in these patients.

OBJETIVO: Analisar o LEPR em crianças obesas e investigar associações entre achados moleculares e características antropométricas e metabólicas. SUJEITOS E MÉTODOS: Foram avaliados 32 pacientes quanto às características antropométricas, à pressão arterial, à frequência cardíaca, às dosagens séricas de glicemia, à insulina, à leptina e ao perfil lipídico. O estudo molecular consistiu na amplificação e no sequenciamento automático da região codificadora do LEPR para pesquisar mutações. RESULTADOS: Identificou-se uma alta prevalência de distúrbios metabólicos: glicemia de jejum alterada em 12,5%, HOMA-IR elevado em 85,7%, níveis de HDL-colesterol baixos em 46,9%, níveis de triglicérides elevados em 40,6% e hipertensão arterial em 58,6%. O estudo molecular identificou 6 variações alélicas já descritas na literatura: rs1137100 (éxon-2), rs1137101 (éxon-4), rs1805134 (éxon-7), rs8179183 (éxon-12), rs1805096 (éxon-18) e deleção/inserção do pentanucleotídeo CTTTA na região 3' não traduzida. CONCLUSÕES: A frequência das variações alélicas observada é semelhante à descrita na literatura e não se correlacionou com nenhuma característica clínica. Os resultados da análise molecular do LEPR não parecem estar implicados na etiologia da obesidade desses pacientes.

Clinical aspects of autosomal recessive polycystic kidney disease.

INTRODUCTION: Autosomal Recessive Polycystic Kidney Disease (ARPKD) is an important pediatric cause of morbidity and mortality, with a variable clinical spectrum. METHODS: The clinical presentation and evolution of 25 patients (Pts) were analyzed by clinical record review, according to the forms proposed by Guay-Woodford et al. Morbidities associated with the disease were evaluated with respect to their frequencies and age of onset. RESULTS: The median age at the diagnosis was 61.45 months (0 to 336.5 months), with similar gender distribution (52% of the patients were female). A family ARPKD history was found in 20% of the cases (5/25), two of them associated with consanguinity. On arrival, arterial hypertension (SAH) was diagnosed in 56% of the Pts (14/25); chronic kidney disease stage ≥ 2 (CKD ≥ 2) in 24% (6/25); urinary tract infection (UTI) in 40% (10/25); and portal hypertension (PH) in 32% of the cases (8/25). Eighty percent of the initial abdominal ultrasonograms detected echogenic kidneys with gross cysts and 64% demonstrated normal liver and biliary ducts. ACE inhibitors were used in 36% of the analyzed patients, beta-blockers in 20%, calcium channel blockers in 28%, and diuretics in 36% of them. In the final evaluation, after an average follow-up time of 152.2 months (29.8 to 274.9 months), SAH was detected in 76% of the cases, CKD ≥ 2 in 44%, UTI in 52% and PH in 68%. CONCLUSION: The high morbidity and mortality associated with ARPKD justify the assembly of an international database, with the aim of establishing an early therapeutic support.

Clinical aspects of autosomal recessive polycystic kidney disease / Aspectos clínicos da doença renal policística autossômica recessiva DRPAR

INTRODUÇÃO: A Doença Renal Policística Autossômica Recessiva (DRPAR) é uma causa importante de morbidade e mortalidade pediátricas, com um espectro variável de manifestações clínicas. MÉTODOS: A apresentação e evolução clínica de 25 pacientes (Pts) foram analisadas através da revisão de prontuários, aplicando-se os formulários propostos por Guay-Woodford et al. As morbidades associadas à doença foram avaliadas quanto à frequência e à idade de manifestação. RESULTADOS: A idade média de diagnóstico foi de 61,45 meses (0 a 336,5 meses), com distribuição similar entre os sexos (52 por cento dos pts do sexo feminino). Houve histórico familiar da doença em 20 por cento dos casos (5/25), com dois casos de consanguinidade. Na análise inicial, diagnosticou-se hipertensão arterial (HAS) em 56 por cento dos Pts (14/25); doença renal crônica estágio > 2 (DRC > 2) em 24 por cento (6/25); infecções do trato urinário (ITU) em 40 por cento (10/25) e hipertensão portal (HP) em 32 por cento dos casos (8/25). Das ultrassonografias abdominais iniciais, 80 por cento demonstraram rins ecogênicos com cistos grosseiros e 64 por cento detectaram fígado e vias biliares normais. Inibidores da ECA foram utilizados em 36 por cento dos Pts, betabloqueadores em 20 por cento, bloqueadores de canais de cálcio em 28 por cento e diuréticos em 36 por cento dos casos. Na análise final, após um tempo de acompanhamento médio de 152,2 meses (29,8 a 274,9 meses), HAS foi diagnosticada em 76 por cento dos Pts, DRC > 2 em 44 por cento, ITU em 52 por cento e HP em 68 por cento. CONCLUSÃO: As altas morbidade e mortalidade associadas à DRPAR justificam a construção de um banco de dados internacional, visando ao estabelecimento de um tratamento de suporte precoce.

INTRODUCTION: Autosomal Recessive Polycystic Kidney Disease (ARPKD) is an important pediatric cause of morbidity and mortality, with a variable clinical spectrum. METHODS: The clinical presentation and evolution of 25 patients (Pts) were analyzed by clinical record review, according to the forms proposed by Guay-Woodford et al. Morbidities associated with the disease were evaluated with respect to their frequencies and age of onset. RESULTS: The median age at the diagnosis was 61.45 months (0 to 336.5 months), with similar gender distribution (52 percent of the patients were female). A family ARPKD history was found in 20 percent of the cases (5/25), two of them associated with consanguinity. On arrival, arterial hypertension (SAH) was diagnosed in 56 percent of the Pts (14/25); chronic kidney disease stage > 2 (CKD > 2) in 24 percent (6/25); urinary tract infection (UTI) in 40 percent (10/25); and portal hypertension (PH) in 32 percent of the cases (8/25). Eighty percent of the initial abdominal ultrasonograms detected echogenic kidneys with gross cysts and 64 percent demonstrated normal liver and biliary ducts. ACE inhibitors were used in 36 percent of the analyzed patients, beta-blockers in 20 percent, calcium channel blockers in 28 percent, and diuretics in 36 percent of them. In the final evaluation, after an average follow-up time of 152.2 months (29.8 to 274.9 months), SAH was detected in 76 percent of the cases, CKD > 2 in 44 percent, UTI in 52 percent and PH in 68 percent. CONCLUSION: The high morbidity and mortality associated with ARPKD justify the assembly of an international database, with the aim of establishing an early therapeutic support.