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1.
Rev. medica electron ; 43(2): 3074-3090, mar.-abr. 2021. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1251927

ABSTRACT

RESUMEN Introducción: la cirrosis hepática de etiología viral representa un impactante problema de salud a nivel mundial, no solo por su elevada tasa de prevalencia, sino por los costos generados en la atención médica. Objetivos: determinar el comportamiento de los pacientes cirróticos, de etiología viral, en la provincia de Matanzas. Materiales y métodos: se realizó un estudio descriptivo-retrospectivo en 47 pacientes con cirrosis hepática de etiología viral, atendidos en el Servicio de Gastroenterología del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, de Matanzas, de enero de 2016 a enero de 2018. Los resultados de las variables analizadas se expusieron en tablas de doble entrada. Resultados: el 68,1 % de los pacientes correspondió a cirrosis por virus C. Predominaron los mayores de 50 años, con carga viral entre 4-6,9 log10, y atendidos en régimen ambulatorio. En el 57,4 % se detectaron signos endoscópicos de hipertensión portal, que se corroboraron en el doppler hepático. La ascitis asociada a diferentes sepsis fueron las complicaciones más registradas. El 55,4 % fue clasificado como Child-Pugh A, y el 76,6 % en etapa clínica compensada. Conclusiones: el diagnóstico y seguimiento de la cirrosis hepática viral sigue siendo un verdadero reto para la comunidad médica. De ahí los esfuerzos que han de realizarse para su control desde las fases compensadas, para retardar la aparición de complicaciones (AU).


ABSTRACT Introduction: viral etiology liver cirrhosis is an impacting health problem around the world, not only because of its high prevalence rate but also because of the costs generated by its medical care. Objective: to determine the behavior of the patients with viral etiology liver cirrhosis in the province of Matanzas. Materials and methods: a descriptive-retrospective study was carried out in 47 patients with viral etiology liver cirrhosis treated in the service of Gastroenterology of the Hospital "Comandante Faustino Perez" of Matanzas, from January 2016 to January 2018. The results of the analyzed variables were shown in double-entry tables. Results: 68.1% of the patients presented cirrhosis caused by C virus, Patients elder 50 years old predominated, with 4-6.9 log10, treated in ambulatory regimen. Endoscopic signs of portal hypertension were found in 57.4%. It was corroborated with liver Doppler. Ascites associated to different sepsis were the most frequently registered complications. 55.4% were classified as Child-Pugh A, and 76.6% were in compensated clinical stage. Conclusions: viral liver cirrhosis diagnosis and follow-up is still a true challenge for the medical community, and hence the efforts that should be made to control it from the compensated stages to delay the appearance of complications (AU).


Subject(s)
Humans , Male , Female , Virus Diseases/etiology , Liver Cirrhosis/etiology , Global Health/standards , Chronic Disease/epidemiology , Liver Cirrhosis/diagnosis , Liver Cirrhosis/epidemiology , Liver Diseases/complications , Liver Diseases/diagnosis
2.
Rev. medica electron ; 42(3): 1815-1825, mayo.-jun. 2020. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1127043

ABSTRACT

RESUMEN Introducción: el embarazo supone cambios en la fisiología de la mujer. Estos cambios pueden llevar a la aparición de enfermedades que afectan el hígado como: síndrome de HELLP, colestasis gravídica intrahepática, esteatosis hepática aguda del embarazo, entre otras, que pueden repercutir en el curso de la gestación. Material y métodos: se realizó un estudio observacional, descriptivo, retrospectivo de corte transversal en 52 gestantes que fueron valoradas en gastroenterología por sospecha de hepatopatías durante el embarazo en el año 2018, que constituyeron el universo de estudio, con el objetivo de determinar el comportamiento de las hepatopatías durante la gestación en las embarazadas valoradas por el servicio de Gastroenterología del Hospital Universitario "Comandante Faustino Pérez Hernández" de Matanzas. Se estudiaron las variables: trimestre gestacional, síntomas y signos, resultados analíticos, entidad nosológica, tipo de parto y complicación neonatal. Se elaboró una planilla para la recolección de los datos. Los resultados se analizaron en frecuencias absolutas y porcientos y se expusieron en tablas. Resultados: predominaron las gestantes del tercer trimestre con 61.6 %. El síntoma más frecuente fue el prurito en 33 gestantes (63.6%). La hipertransaminasemia se manifestó en el 100% de las gestantes. La entidad más frecuente fue la hepatitis crónica por virus B en 19 gestantes (36.5%) seguida de la colestasis intrahepática del embarazo con un 25 %. La mayoría de los partos fueron realizados por cesárea (94.2 %). La principal complicación neonatal fue el bajo peso al nacer en 26 (39.4 %). Conclusiones: las hepatopatías propias de la gestación se comportaron con igual frecuencia descrita en la literatura de acuerdo al trimestre que predominó, aunque fue significativa la incidencia de gestantes valoradas con infección por virus de la hepatitis b cuyo diagnóstico se hizo durante el embarazo siendo la principal causa de las complicaciones neonatales observadas (AU).


SUMMARY Introduction: Pregnancy supposes changes in the woman's physiology. These changes can lead to the appearance of illnesses affecting the liver, such as Hellp syndrome, intrahepatic cholestasis gravidarum, acute hepatic steatosis of pregnancy, among others, that can rebound in the course of the gestation. Materials and methods: a cross-sectional retrospective, descriptive, observational study was carried out in 52 pregnant women that were valued in Gastroenterology due to the suspicion of liver diseases during pregnancy in 2018; they were the universe of the study and the aim was determining the behavior of liver diseases during pregnancy in pregnant women valued in the service of Gastroenterology of the university hospital "Comandante Faustino Pérez Hernández" of the province of Matanzas: The studied variables were: gestational trimester, symptoms and signs, analytical results; nosological entity, type of delivery and neonatal complications. A form was draw up to collect data. The results were analyzed in absolute frequencies and percentages and showed in tables. Results: The third semester pregnant women predominated with 61.7 %. Pruritus was the most frequent symptom in 33 pregnant women (63.6 %). High levels of serum transaminases were present in 100 % of the studied women. The most frequent disease was chronic hepatitis caused by virus B in 19 pregnant women (36.5 %), followed by intrahepatic cholestasis of pregnancy with 25 %. Most of deliveries were performed by cesarean (94.2 %). The main neonatal complication was low weight at birth in 26 (39.4 %). Conclusions: liver diseases that are proper of gestation behaved in the same frequency as they are described in literature in relation to the predominating semester, although it was significant the incidence of assessed pregnant women with infection caused by the virus of hepatitis B diagnosed during pregnancy; it was the main cause of the observed neonatal complications (AU).


Subject(s)
Humans , Female , Pregnancy , Pregnancy Complications , Gastroenterology , Liver Diseases/epidemiology , Maternal Mortality , Epidemiology, Descriptive , Retrospective Studies , Morbidity , Fetal Mortality , Observational Study , Liver Diseases/complications , Liver Diseases/diagnosis , Liver Diseases/etiology
3.
Gastroenterol. latinoam ; 31(1): 28-34, mayo 2020. tab
Article in Spanish | LILACS | ID: biblio-1103373

ABSTRACT

The SARS-CoV-2 virus and the associated disease COVID-19 has quickly become a pandemic. People with underlying chronic diseases or in an immunosupressed state are at risk of having a worse outcome. Cirrhotic patients and liver transplant recipients are considered to be in this higher risk group due to their immunosuppressed state. The aim of this article is to present recommendations based on expert opinion regarding the management of patients with compensated and decompensated liver pathologies who take medication for their immunosuppressed state in medical check-ups and basic treatment management both of patients with and without the COVID-19 disease.


El virus SARS-CoV-2 asociado a la enfermedad COVID-19, se han instalado a nivel de pandemia mundial. Las personas portadoras de enfermedades crónicas o estados de inmunosupresión se encuentran en riesgo de desarrollar un curso más grave. Se considera que los pacientes con cirrosis hepática, patología autoinmune o trasplante hepático se encontrarían dentro de este grupo de mayor riesgo por su estado de inmunosupresión. Presentamos recomendaciones de manejo basadas en opinión de experto, en pacientes con patología hepática compensada y descompensada e inmunosuprimidos farmacológicos, en relación a controles médicos y manejo de terapia de base tanto en pacientes sin COVID-19 como en pacientes infectados.


Subject(s)
Humans , Pneumonia, Viral/epidemiology , Liver Transplantation , Coronavirus Infections/epidemiology , Betacoronavirus , Liver Diseases/therapy , Pneumonia, Viral/therapy , Chronic Disease , Coronavirus Infections/therapy , Pandemics , Liver Diseases/complications
4.
Medicina (B.Aires) ; 79(5): 391-396, oct. 2019. tab
Article in Spanish | LILACS | ID: biblio-1056736

ABSTRACT

Los altos niveles de vitamina B12 o cobalamina, también denominado hipervitaminosis B12 es una anormalidad analítica frecuentemente subestimada. De acuerdo con la literatura algunas de las entidades relacionadas con este hallazgo son las neoplasias sólidas (primarias o metastásicas) y las enfermedades hematológicas agudas o crónicas. Otras causas incluyen la afección hepática, la gammapatía monoclonal de significación indeterminada, la insuficiencia renal y, con menor frecuencia, un exceso de consumo de vitamina B12, enfermedades inflamatorias o autoinmunes y los trastornos hematológicos transitorios (neutrofilia y eosinofilia secundaria). Este artículo informa sobre causas de hipervitaminosis B12, nuestra experiencia y hace una revisión de la literatura.


High serum levels of vitamin B12 or cobalamin, also called hypervitaminemia B12, is a frequently underestimated biological abnormality. According to the literature, some of the entities related to this finding are solid neoplasia (primary or metastatic) and acute or chronic hematological diseases. Other causes include liver disorders, monoclonal gammapathy of undetermined significance, renal failure and, less frequently, excess of vitamin B12 intake, inflammatory or autoimmune diseases, and transient hematological disorders (neutrophilia and secondary eosinophilia). This article reports on causes of hypervitaminosis B12, our experience and a review of the literature.


Subject(s)
Humans , Vitamin B 12/blood , Nutrition Disorders/etiology , Nutrition Disorders/blood , Vitamin B 12/adverse effects , Acute Kidney Injury/complications , Acute Kidney Injury/blood , Hematologic Diseases/complications , Hematologic Diseases/blood , Liver Diseases/complications , Liver Diseases/blood , Neoplasms/complications , Neoplasms/blood
5.
Brasília; CONITEC; jan. 2018. ilus, tab.
Non-conventional in Portuguese | LILACS, BRISA | ID: biblio-905607

ABSTRACT

INTRODUÇÃO: O câncer colorretal (CCR) acomete o intestino grosso, o cólon e o reto, sendo mais frequente aos 60 anos de idade. No Brasil, para 2016, foram estimados 16.660 casos novos de câncer de cólon e reto em homens e de 17.620 em mulheres. O fígado é considerado o local mais comum de metástases com estimativa de afetar aproximadamente metado dos pacientes com CCR em todo mundo a cada ano. As mutações no gene RAS, presentes em 44% destes pacientes, foram associadas a diminuição nas taxas de repostas ao tratamento com anticorpos anti-EGFR, como o cetuximabe. Dessa forma sua atividade é restrita aos tumores RAS selvagem. Os tratamentos disponíveis no Sistema Único de Saúde (SUS) para os pacientes com CCRm com metástases hepáticas irressecáveis são o tratamento quimioterápico baseado em 5- fluorouracil e leucovorin (5-fluorouracil (5-FU) infusional, leucovorin e oxaliplatina ­ FOLFOX ou irinotecano - FOLFIRI) para a redução tumoral. TECNOLOGIA: cetuximabe (Erbitux®). INDICAÇÃO: Tratamento do câncer colorretal metastático RAS selvagem com doença limitada ao fígado em primeira linha. PERGUNTA: O uso de cetuximabe em associação com a quimioterapia citotóxica é eficaz, seguro e custo-efetivo em pacientes com CCR metastático RAS selvagem com doença limitada ao fígado na primeira linha de tratamento quando comparado ao uso da quimioterapia citotóxica isolada? EVIDÊNCIAS CIENTÍFICAS: A evidência é proveniente de um ensaio clínico randomizado de baixa qualidade metodológica que demonstrou que o tratamento com cetuximabe em adição ao esquema quimioterápico FOLFIRI ou FOLFOX proporcionou que um número maior de pacientes fossem submetidos à ressecção com intenção curativa, e aumento na sobrevida global e na sobrevida livre de progressão, quando avaliada a população por intenção de tratar. No entanto quando considerados apenas os pacientes submetidos à cirurgia, que alcançaram a ressecção R0, não houve significância estatística entre os grupos. AVALIAÇÃO ECONÔMICA: A avaliação de custo-efetividade da adição de cetuximabe à quimioterapia em pacientes com CCRm RAS selvagem com doença limitada ao fígado, em primeira linha de tratamento apresentou RCEI de R$ 56.750 por ano de vida ganho quando comparado à quimioterapia isolada. AVALIAÇÃO DE IMPACTO ORÇAMENTÁRIO: A análise de impacto orçamentário estimou um valor acumulado superior à R$ 326 milhões em cinco anos, após a incorporação de cetuximabe. A análise de sensibilidade demonstrou que esse valor porde variar de R$ 153 milhões a R$ 491 milhões em cinco anos. MONITORAMENTO DO HORIZONTE TECNOLÓGICO: existe um grande número de tecnologias sendo estudadas para o tratamento do CCR, com diferentes mecanismos de ação e diferentes vias de administração, inclusive oral. RECOMENDAÇÃO PRELIMINAR DA CONITEC: o procedimento APAC de tratamento do câncer de cólon e reto não inviabiliza o uso do medicamento cetuximabe no âmbito do SUS, que deve ser definido pelo médico em conjunto com o paciente, cujo reembolso será o valor proposto para as APACs disponíveis. A matéria será disponibilizada em consulta pública com recomendação preliminar de não incorporação do cetuximabe para pacientes com CCRm RAS selvagem com doença limitada ao fígado em primeira linha de tratamento. CONSULTA PÚBLICA: Foram recebidas 209 contribuições técnico-científicas e 323 contribuições de experiência e opinião, sendo 92% discordante com a recomendação preliminar da CONITEC. As contribuições se referiram principalmente à existência de evidências clínicas e científicas comprovadas e melhoria da qualidade de vida e sobrevida dos pacientes. A evidência citada foi descrita e discutida no relatório técnico que subsidiou a recomendação da CONITEC. Evidências adicionais apresentadas não foram consideradas uma vez que não respondem à pergunta PICO proposta pelo demandante. A CONITEC entendeu que não houve argumentação suficiente para alterar sua recomendação preliminar. RECOMENDAÇÃO FINAL: Os membros da CONITEC em 09 de novembro de 2017 deliberaram por não recomendar o cetuximabe para o tratamento do câncer colorretal metastático RAS selvagem com doença limitada ao fígado em primeira linha. Foi assinado o Registro de Deliberação nº 306/2017. DECISÃO: Não incorporar o cetuximabe para primeira linha para pacientes com câncer colorretal metastático (CCRm) RAS selvagem, com doença limitada ao fígado, em combinação com os regimes quimioterápicos Folfiri ou Folfox, no âmbito do Sistema Único de Saúde ­ SUS, dada pela Portaria nº 4, publicada no DOU nº 18, do dia 25 de janeiro de 2018, seção 1, pág. 123.(AU)


Subject(s)
Humans , Cetuximab/therapeutic use , Colorectal Neoplasms/drug therapy , Brazil , Cost-Benefit Analysis/economics , Liver Diseases/complications , Technology Assessment, Biomedical , Unified Health System
6.
Arq. gastroenterol ; 54(3): 225-231, July-Sept. 2017. tab, graf
Article in English | LILACS | ID: biblio-888212

ABSTRACT

ABSTRACT BACKGROUND: Protein-calorie malnutrition is common in chronic liver disease (CLD) but adequate clinical tools for nutritional assessment are not defined. OBJECTIVE: In CLD patients, it was aimed: 1. Characterize protein-calorie malnutrition; 2. Compare several clinical, anthropometric and functional tools; 3. Study the association malnutrition/CLD severity and malnutrition/outcome. METHODS: Observational, prospective study. Consecutive CLD ambulatory/hospitalised patients were recruited from 01-03-2012 to 31-08-2012, studied according with age, gender, etiology, alcohol consumption and CLD severity defined by Child-Turcotte-Pugh. Nutritional assessment used subjective global assessment, anthropometry, namely body-mass index (BMI), triceps skinfold, mid upper arm circumference, mid arm muscular circumference and handgrip strength. Patients were followed during two years and survival data was recorded. RESULTS: A total of 130 CLD patients (80 men), aged 22-89 years (mean 60 years) were included. Most suffered from alcoholic cirrhosis (45%). Hospitalised patients presented more severe disease ( P <0.001) and worst nutritional status defined by BMI ( P =0.002), mid upper arm circumference ( P <0.001), mid arm muscular circumference ( P <0.001), triceps skinfold ( P =0.07) and subjective global assessment ( P <0.001). A third presented deficient/low handgrip strength. Alcohol consumption ( P =0.03) and malnutrition detected by BMI ( P =0.03), mid upper arm circumference ( P =0.001), triceps skinfold ( P =0.06), mid arm muscular circumference ( P =0.02) and subjective global assessment ( P <0.001) were associated with CLD severity. From 25 patients deceased during follow-up, 17 patients were severely malnourished according with triceps skinfold. Malnutrition defined by triceps skinfold predicted mortality ( P <0.001). CONCLUSION: Protein-calorie malnutrition is common in CLD patients and alcohol plays an important role. Triceps skinfold is the most efficient anthropometric parameter and is associated with mortality. Nutritional assessment should be considered mandatory in the routine care of CLD patients.


RESUMO CONTEXTO: A desnutrição calórico-proteica é frequente na doença hepática crônica (DHC), no entanto, ferramentas clínicas adequadas para avaliação nutricional destes doentes não estão definidas. OBJETIVO: Em doentes com DHC, pretendeu-se: 1. Caracterizar a desnutrição calórico-proteica; 2. Comparar diferentes ferramentas clínicas, antropométricas e funcionais de avaliação nutricional; 3. Estudar a associação desnutrição/gravidade da DHC e desnutrição/prognóstico. MÉTODOS: Estudo observacional e prospetivo. Foram recrutados doentes ambulatórios/hospitalizados de 01-03-2012 a 31-08-2012 e estudados tendo em conta a idade, gênero, etiologia, consumo alcoólico e gravidade da DHC definida pelo score Child-Turcotte-Pugh. A avaliação nutricional incluiu a utilização da avaliação global subjetiva, antropometria nomeadamente índice de massa corporal (IMC), perímetro braquial, circunferência muscular do braço, prega cutânea tricipital e dinamometria. Os doentes foram seguidos durante 2 anos e foi registada a respectiva sobrevida. RESULTADOS: Foram incluídos 130 doentes com DHC (80 homens) com idade 22-89 anos (média 60 anos). A maioria apresentava cirrose alcoólica (45%). Os doentes hospitalizados apresentaram doença hepática mais severa ( P <0,001) e pior estado nutricional, definido pelo IMC ( P =0,002), perímetro braquial ( P <0,001), circunferência muscular do braço ( P <0,001), prega cutânea tricipital ( P =0,07) e avaliação global subjetiva ( P <0,001). Um terço apresentava força de preensão manual deficiente/baixa. O consumo alcoólico ( P =0,03) e a desnutrição detetada pelo IMC ( P =0,03), perímetro braquial ( P =0,001), prega cutânea tricipital ( P =0,06), circunferência muscular do braço ( P =0,02) e avaliação global subjetiva ( P <0,001) encontraram-se associados à gravidade da DHC. Dos 25 doentes que faleceram durante o seguimento, 17 apresentavam desnutrição severa definida pela prega cutânea tricipital. A desnutrição definida pela prega cutânea tricipital revelou ser um fator preditivo de mortalidade ( P <0,001). CONCLUSÃO: A desnutrição calórico-proteica é comum na DHC para a qual o álcool desempenha um papel importante. A prega cutânea tricipital é o parâmetro antropométrico mais eficiente e encontra-se associado à mortalidade. A avaliação nutricional deve ser considerada mandatória na abordagem rotineira de doentes com DHC.


Subject(s)
Humans , Male , Female , Adult , Aged , Aged, 80 and over , Young Adult , Protein-Energy Malnutrition/etiology , Liver Diseases/complications , Severity of Illness Index , Body Mass Index , Nutrition Assessment , Chronic Disease , Prospective Studies , Follow-Up Studies , Protein-Energy Malnutrition/mortality , Liver Diseases/mortality , Middle Aged
7.
Brasília; CONITEC; jun. 2017. graf, tab.
Non-conventional in Portuguese | LILACS, BRISA | ID: biblio-906980

ABSTRACT

CONTEXTO: A hiperamonemia é caracterizada pelo elevado nível de amônia na corrente sanguínea, decorrente de falhas no catabolismo de aminoácidos ou amônia para uréia. As doenças hepáticas são as causas mais comuns de hiperamonemia em adultos. Podem ser agudas, como por exemplo, hepatites virais, isquemia, ou hepatotoxinas, ou crônicas, como cirrose hepática, hepatites, atresia biliar, deficiência de alfa1-antitripsina; doença de Wilson; fibrose cística; galactosemia e tirosinemia. A encefalopatia hepática (EH) é uma complicação neuropsiquiátrica frequente nos portadores de doenças hepáticas e caracteriza-se por distúrbios da atenção, alterações do sono e distúrbios motores que progridem desde simples letargia a estupor ou coma. É um distúrbio metabólico, portanto potencialmente reversível, sendo a hiperamonemia, o principal fator dessa condição. São alternativas terapêuticas, os dissacarídeos não absorvíveis (lactulose e lactitol), antibióticos orais não absorvíveis, BCAA (aminoácidos de cadeia ramificada) e probióticos. No SUS, a lactulose é a alternativa disponível para tratar essa condição. TECNOLOGIA: Aspartato de ornitina (Hepa-Merz®). INDICAÇÃO: Hiperamonemia produzida por doenças hepáticas agudas e crônicas. PERGUNTA: O uso do aspartato de ornitina é eficaz e seguro no tratamento da hiperamonemia produzida por doenças hepáticas agudas e crônicas? EVIDÊNCIAS CIENTÍFICAS: Foram encontrados 6 estudos, sendo 2 revisões sistemáticas, 3 ensaios clínicos e 1 parecer técnico-científico. As evidências demonstraram um benefício provável no uso do aspartato de ornitina em comparação à não intervenção ou placebo no tratamento da hiperamonemia decorrente de doenças hepáticas, predominantemente crônicas e presença de cirrose. Entretanto, comparado à lactulose, alternativa disponível no SUS e demais intervenções, não foi observado benefício adicional. AVALIAÇÃO ECONÔMICA: Foi realizada uma avaliação econômica com um modelo de custominimização. Para a realização dos cálculos, foram considerados somente os custos de aquisição dos medicamentos. Os resultados demonstram que a lactulose pode ser até 76% menos custosa que o aspartato de ornitina oral. AVALIAÇÃO DE IMPACTO ORÇAMENTÁRIO: A Secretaria-Executiva da CONITEC calculou o impacto orçamentário em um cenário base que considera uma divisão de mercado (Market-share) gradual de até 70% em 2021, considerando uma perspectiva de horizonte temporal de 5 anos. A quantidade de pacientes elegíveis foi estimada utilizando dados prevalência encontrados na literatura. Os custos de tratamento considerados foram aqueles já utilizados nas compras do Ministério da Saúde ou encontrados em busca no Banco de Preços em Saúde. O impacto orçamentário resultante da incorporação do aspartato de ornitina seria de um incremento de aproximadamente 15,3 milhões de reais em 5 anos após a incorporação. EXPERIÊNCIA INTERNACIONAL: Não foram encontradas avaliações sobre o aspartato de ornitina para tratamento da encefalopatia hepática ou hiperamonemia nas agências NICE (Inglaterra), CADTH (Canadá), SMC (Escócia) e PBAC (Austrália). DISCUSSÃO: Os resultados não demonstraram superioridade do aspartato de ornitina em relação á lactulose, alternativa disponível no SUS. A despeito dos resultados favoráveis ao aspartato de ornitina quando comparado com placebo/não intervenção, evidências robustas apontam efeito benéfico também da lactulose/lactitol sobre resultados clinicamente relevantes quando comparados à placebo/nenhuma intervenção, com destaque para o desfecho de mortalidade. Por fim, em um cenário de incorporação do aspartato de ornitina, haverá incremento importante no impacto orçamentário. RECOMENDAÇÃO DA CONITEC: Os membros da CONITEC, presentes na 54ª reunião ordinária, realizada nos dias 5 e 6 de abril de 2017, apreciaram a proposta e decidiram que a matéria seria disponibilizada em Consulta Pública com recomendação preliminar desfavorável à incorporação do aspartato de ornitina para o tratamento hiperamonemia produzida por doenças hepáticas agudas e crônicas. CONSULTA PÚBLICA: Foi realizada a Consulta Pública nº 16/2017 sobre o relatório de recomendação da CONITEC "Aspartato de ornitina para o tratamento da hiperamonemia produzida por doenças hepáticas agudas e crônicas" entre os dias 13/04/2017 e 02/05/2017. Foram recebidas 10 contribuições, sendo 8 pelo formulário para contribuições sobre experiência ou opinião e 2 pelo formulário para contribuições técnico-científicas. A maioria das contribuições foram contrárias à recomendação inicial da CONITEC. DELIBERAÇÃO FINAL: Os membros da CONITEC presentes na 56ª reunião do plenário do dia 07/06/2017 deliberaram por unanimidade recomendar a não incorporação do aspartato de ornitina para o tratamento da hiperamonemia produzida por doenças hepáticas agudas e crônicas, com a ressalva de que o Ministério da Saúde reformulará a demanda, especificando a população. Foi assinado o registro de deliberação nº 261/2017.(AU)


Subject(s)
Humans , Hyperammonemia/drug therapy , Liver Diseases/complications , Ornithine/therapeutic use , Brazil , Health Evaluation/economics , Technology Assessment, Biomedical , Unified Health System
8.
Clinics ; 72(6): 351-357, June 2017. tab
Article in English | LILACS | ID: biblio-840092

ABSTRACT

OBJECTIVE: The aim of this research was to compare language development (expressive and receptive skills) in children awaiting liver transplantation with that of children who have already undergone the surgical procedure. METHODS: An observational, descriptive, cross-sectional study was conducted with 76 children divided into groups, as follows: 31 children who were candidates for liver transplantation (Group 1; G1), 45 children who had already undergone liver transplantation (Group 2; G2), and a control group (CG) of 60 healthy, normally developing children. Health status information was gathered, and the Test of Early Language Development (TELD)-3 was used to assess language skills. Family household monthly income data were also gathered using a specific questionnaire. RESULTS: G1 had poorer language performance compared with G2 and the CG. G2 had lower language performance when compared with the CG. However, when considering the TELD-3 standard scores, G2 had scores within normal limits. The regression analysis indicated age as a risk factor for language deficits in Group 1 and family income as a risk factor for language deficits in G2. CONCLUSIONS: The results suggested that children with chronic liver disease have delays in language development. Transplanted children have linguistic performance within normal limits, but their scores tended to be lower than the CG.


Subject(s)
Humans , Male , Female , Child , Language Disorders/complications , Liver Diseases/complications , Brazil , Case-Control Studies , Cross-Sectional Studies , Language Disorders/diagnosis , Language Tests , Liver Diseases/surgery , Liver Transplantation , Risk Factors , Severity of Illness Index , Socioeconomic Factors
9.
Clinics ; 72(4): 231-237, Apr. 2017. tab, graf
Article in English | LILACS | ID: biblio-840064

ABSTRACT

OBJECTIVES: The present study was designed to evaluate the bone phenotypes and mechanisms involved in bone disorders associated with hepatic osteodystrophy. Hepatocellular disease was induced by carbon tetrachloride (CCl4). In addition, the effects of disodium pamidronate on bone tissue were evaluated. METHODS: The study included 4 groups of 15 mice: a) C = mice subjected to vehicle injections; b) C+P = mice subjected to vehicle and pamidronate injections; c) CCl4+V = mice subjected to CCl4 and vehicle injections; and d) CCl4+P = mice subjected to CCl4 and pamidronate injections. CCl4 or vehicle was administered for 8 weeks, while pamidronate or vehicle was injected at the end of the fourth week. Bone histomorphometry and biomechanical analysis were performed in tibiae, while femora were used for micro-computed tomography and gene expression. RESULTS: CCl4 mice exhibited decreased bone volume/trabecular volume and trabecular numbers, as well as increased trabecular separation, as determined by bone histomorphometry and micro-computed tomography, but these changes were not detected in the group treated with pamidronate. CCl4 mice showed increased numbers of osteoclasts and resorption surface. High serum levels of receptor activator of nuclear factor-κB ligand and the increased expression of tartrate-resistant acid phosphatase in the bones of CCl4 mice supported the enhancement of bone resorption in these mice. CONCLUSION: Taken together, these results suggest that bone resorption is the main mechanism of bone loss in chronic hepatocellular disease in mice.


Subject(s)
Animals , Male , Bone Diseases, Metabolic/etiology , Bone Diseases, Metabolic/drug therapy , Bone Remodeling/drug effects , Diphosphonates/pharmacology , Bone Density Conservation Agents/pharmacology , Liver Diseases/complications , Phosphorus/administration & dosage , Bone and Bones/drug effects , Bone and Bones/metabolism , Bone and Bones/diagnostic imaging , Bone Diseases, Metabolic/metabolism , Bone Resorption/metabolism , Carbon Tetrachloride , Disease Models, Animal , Core Binding Factor Alpha 1 Subunit/genetics , RANK Ligand/genetics , Osteoprotegerin/genetics , X-Ray Microtomography , Tartrate-Resistant Acid Phosphatase/genetics , Liver Cirrhosis/chemically induced , Liver Cirrhosis/metabolism , Liver Diseases/metabolism , Mice, Inbred C57BL
10.
Gastroenterol. latinoam ; 27(supl.1): S69-S71, 2016.
Article in Spanish | LILACS | ID: biblio-907658

ABSTRACT

Cirrhotic patients frequently exhibit abnormal coagulation tests such as prothrombin time (PT) and activated partial thromboplastin time (APTT) due to impairment in hepatic synthesis of coagulation factors. Due to these abnormalities and also to the numerous bleeding episodes that characterize advanced or decompensated liver cirrhosis, these patients were traditionally considered as “anticoagulated”. More recently, this paradigm has been challenged due to the increased occurrence of thrombotic complications among this population. This can only be understood when analyzed under the prism of the cellular theory of coagulation, which describes the complex interactions between endothelial, platelets and inflammatory cells that determine the status of coagulation, anticoagulation and fibrinolysis. The liver participates actively in this process contributing to the maintenance of a dynamic equilibrium in healthy patients. During liver failure there is evidence of impairment of synthesis of factors involved in the coagulation process, but also in anticoagulation and fibrinolysis. However, stable cirrhotic patients tend to maintain a delicate dynamic equilibrium. This equilibrium can be altered in acute decompensations leading to hemorrhagic or thrombotic complications.


Los pacientes con cirrosis presentan disminución en la producción de factores de coagulación de síntesis hepática, esto determina alteración del tiempo de protrombina (TP) y tiempo de tromboplastina parcial activada (TTPa). Esta característica, junto con los episodios de sangrado, que se presentan frecuentemente en pacientes descompensados y en etapa avanzada de la enfermedad, significó que por muchos años se considerara a los pacientes con daño hepático crónico como “anticoagulados”. En los últimos años este paradigma ha sido desafiado por estudios que muestran una mayor frecuencia de trombosis en esta población. Este fenómeno se comprende mejor con la teoría celular de la coagulación que integra a la ecuación las membranas celulares, especialmente de endotelio, plaquetas y células inflamatorias, y que permite visualizar las complejas interacciones entre factores coagulantes, anticoagulantes y fibrinolisis. El hígado participa en forma activa en este proceso que determina un amplio equilibrio dinámico en individuos sanos. El daño hepático claramente altera la coagulación, sin embargo, la evidencia actual demuestra que en la mayoría de los pacientes se produce un delicado rebalance hemostático, que determina una coagulación efectiva. Si este frágil equilibrio se altera, se produce un desbalance que puede generar un estado hemorrágico o trombótico.


Subject(s)
Humans , Blood Coagulation Disorders/etiology , Liver Cirrhosis/physiopathology , Liver Diseases/complications , Chronic Disease , Hemorrhage/etiology , Liver Diseases/physiopathology , Thrombosis/etiology
11.
Femina ; 43(5): 225-234, set.-out. 2015. tab
Article in Portuguese | LILACS | ID: lil-771218

ABSTRACT

A gestação é um período de significativas modificações no organismo materno, que objetivam promover a homeostase do binômio materno-fetal. Sob o ponto de vista hepático, demais das alterações conspícuas à gravidez, deve o obstetra detectar precocemente anomalias envolvendo o fígado, que complicam até 3% das gestações e são responsáveis por elevada mortalidade materna e perinatal. Por outro lado, certas doenças hepáticas têm sua história natural modificada quando ocorrem durante a gestação, demandando cuidados especiais de uma equipe multidisciplinar que envolva o obstetra e o hepatologista. Este artigo revisa as modificações fisiológicas do sistema hepático na gravidez, assim como suas alterações hepáticas mais prevalentes no Brasil. O objetivo é auxiliar e fornecer orientações ao obstetra e guiar o melhor cuidado das pacientes a fim de prevenir e reduzir as complicações hepáticas na gravidez.(AU)


Pregnancy is a period of significant changes in the mother's organism aimed at promoting the mother-fetus homeostasis. From the hepatic standpoint, the obstetrician should detect early the abnormalities attacking the liver, which complicates up to 3% of pregnancies and are responsible for high rates of maternal and perinatal mortality. On the other hand, some liver diseases have their natural evolution changed when they occur during the pregnancy, requiring special care of a multidisciplinary team involving obstetrician and hepatologist specialists. This study presents the physiological changes of the hepatic system during pregnancy, as well as the most prevalent pregnancy hepatic disorders occurring in Brazil. It aims to help the obstetrician and guide the best patient care to prevent and reduce hepatic complications in pregnancy.(AU)


Subject(s)
Humans , Female , Pregnancy , Pregnancy Complications/etiology , Liver/physiopathology , Liver Diseases/complications , Liver Diseases/diagnosis , Pre-Eclampsia/etiology , Pregnancy, Abdominal/physiopathology , Cholestasis, Intrahepatic/complications , Databases, Bibliographic , HELLP Syndrome/etiology , Fatty Liver/complications , Hyperemesis Gravidarum/complications
12.
Clinics ; 70(7): 486-492, 2015. tab, graf
Article in English | LILACS | ID: lil-752391

ABSTRACT

OBJECTIVE: To determine the associations of liver lobe-based magnetic resonance diffusion-weighted imaging findings using multiple b values with the presence and Child-Pugh class of cirrhosis in patients with hepatitis B. METHODS: Seventy-four cirrhotic patients with hepatitis B and 25 healthy volunteers underwent diffusion-weighted imaging using b values of 0, 500, 800 and 1000 sec/mm2. The apparent diffusion coefficients of individual liver lobes for b(0,500), b(0,800) and b(0,1000) were derived from the signal intensity averaged across images obtained using b values of 0 and 500 sec/mm2, 0 and 800 sec/mm2, or 0 and 1000 sec/mm2, respectively, and were statistically analyzed to evaluate cirrhosis. RESULTS: The apparent diffusion coefficients for b(0,500), b(0,800) and b(0,1000) inversely correlated with the Child-Pugh class in the left lateral liver lobe, the left medial liver lobe, the right liver lobe and the caudate lobe (r=-0.35 to -0.60, all p<0.05), except for the apparent diffusion coefficient for b(0,1000) in the left medial liver lobe (r=-0.17, p>0.05). Among these parameters, the apparent diffusion coefficient for b(0,500) in the left lateral liver lobe best differentiated normal from cirrhotic liver, with an area under the receiver operating characteristic curve of 0.989. The apparent diffusion coefficient for b(0,800) in the right liver lobe best distinguished Child-Pugh class A from B-C and A-B from C, with areas under the receiver operating characteristic curve of 0.732 and 0.747, respectively. CONCLUSION: Liver lobe-based apparent diffusion coefficients for b(0,500) and b(0,800) appear to be associated with the presence and Child-Pugh class of liver cirrhosis. .


Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young Adult , Diffusion Magnetic Resonance Imaging , Hepatitis B/diagnosis , Liver Cirrhosis/diagnosis , Case-Control Studies , Hepatitis B/complications , Liver Cirrhosis/complications , Liver Diseases/complications , Liver Diseases/diagnosis , Prospective Studies
13.
Arab Journal of Gastroenterology. 2015; 16 (3-4): 90-93
in English | IMEMR | ID: emr-174959

ABSTRACT

Background and study aims: Liver biopsy is a well-established procedure in the diagnosis and follow-up of liver diseases. Complications of liver biopsy are rare but potentially lethal. The aim of this study was to evaluate the complications of percutaneous liver biopsy and to compare the complications of blind and ultrasound-guided percutaneous liver biopsy in paediatric wards of Nemazee Hospital of Shiraz in the south of Iran


Patient and method: To complete the questionnaire, registered information of liver biopsies due to different causes in paediatric patients between 2008 and 2012 was retrospectively reviewed. All children aged between 0 and 18 years, who underwent liver biopsy [due to any indication], participated in this study


Results: Liver biopsies were obtained from 210 patients. Seven of 210 cases were excluded due to unreliable data. A total of 209 liver biopsies were done in the rest of the cases [n = 203]. Of all cases of liver biopsies, 22 [10.5%] experienced complications after biopsy. Pain [n = 7] was the most frequent complication in 22 cases of liver biopsy. Mortality rate was one [0.5%] due to rupture of subcapsular haematoma. In terms of complication [p = 0.592], there was no significant difference statistically between patients with blind liver biopsy [n = 16] and patients with ultrasound-guided liver biopsy [n = 6]


Conclusion: In terms of complications, there was no significant difference when the patients were evaluated with and without ultrasound-guided biopsy


Subject(s)
Humans , Infant , Infant, Newborn , Child , Child, Preschool , Adolescent , Liver/pathology , Biopsy , Liver Diseases/diagnostic imaging , Liver Diseases/complications , Surveys and Questionnaires , Child
14.
JABHS-Journal of the Arab Board of Health Specializations. 2014; 15 (1): 40-47
in English | IMEMR | ID: emr-157622

ABSTRACT

Assessing hepatobiliary function among critically ill patients remains challenging, and neither static nor dynamic tests can be considered a gold standard. This issue could contribute to an underestimation of the frequency and importance of this system dysfunction during the course of sepsis. Because hepatic and biliary dysfunction is strongly associated with high morbidity and mortality in patients with sepsis, the ability to accurately assess these functions is of critical interest. During sepsis, the liver plays a key role in the pathophysiology of this disorder. It is implicated in the host response, participating in the clearance of the infectious agents and their products. Sepsis also can causes hepatobiliary damage through hemodynamic alterations or through direct or indirect assault on the hepatocytes or through both. Accordingly, hepatobiliary dysfunction induced by sepsis is recognized as one of the components that contribute to the severity of the disease. In this review article, we discuss the epidemiology, diagnostic tools, and some impact on outcome as well as the pathophysiological aspects, including the cellular events and clinical picture leading to hepatobiliary dysfunction. Finally, therapeutic considerations with regard to the weakness of the pertinent specific approach are examined


Subject(s)
Humans , Liver Diseases/complications , Hemodynamics , Critical Illness , Liver Function Tests , Hepatocytes
15.
Article in English | WPRIM | ID: wpr-112007

ABSTRACT

Tetrahydrobiopterin (BH4) is an essential cofactor in NO synthesis by endothelial nitric oxide synthase (eNOS) enzymes. It has been previously suggested that reduced intrahepatic BH4 results in a decrease in intrahepatic NO and contributes to increased hepatic vascular resistance and portal pressure in animal models of cirrhosis. The main aim of the present study was to evaluate the relationship between BH4 and portal hypertension (PHT). One hundred ninety-three consecutive patients with chronic liver disease were included in the study. Liver biopsy, measurement of BH4 and hepatic venous pressure gradient (HVPG) were performed. Hepatic fibrosis was classified using the Laennec fibrosis scoring system. BH4 levels were determined in homogenized liver tissues of patients using a high performance liquid chromatography (HPLC) system. Statistical analysis was performed to evaluate the relationship between BH4 and HVPG, grade of hepatic fibrosis, clinical stage of cirrhosis, Child-Pugh class. A positive relationship between HVPG and hepatic fibrosis grade, clinical stage of cirrhosis and Child-Pugh class was observed. However, the BH4 level showed no significant correlation with HVPG or clinical features of cirrhosis. BH4 concentration in liver tissue has little relation to the severity of portal hypertension in patients with chronic liver disease.


Subject(s)
Adult , Aged , Biopterin/analogs & derivatives , Chromatography, High Pressure Liquid , Chronic Disease , Elasticity Imaging Techniques , Female , Hepatic Veins/physiology , Humans , Hypertension, Portal/complications , Liver/pathology , Liver Cirrhosis/diagnostic imaging , Liver Diseases/complications , Male , Middle Aged , Nitric Oxide/metabolism , Portal Pressure , Regression Analysis , Severity of Illness Index
16.
Article in English | WPRIM | ID: wpr-18381

ABSTRACT

Portal hypertension is a severe consequence of chronic liver diseases and is responsible for the main clinical complications of liver cirrhosis. Hepatic venous pressure gradient (HVPG) measurement is the best available method to evaluate the presence and severity of portal hypertension. Clinically significant portal hypertension is defined as an increase in HVPG to >10 mmHg. In this condition, the complications of portal hypertension might begin to appear. HVPG measurement is increasingly used in the clinical fields, and the HVPG is a robust surrogate marker in many clinical applications such as diagnosis, risk stratification, identification of patients with hepatocellular carcinoma who are candidates for liver resection, monitoring of the efficacy of medical treatment, and assessment of progression of portal hypertension. Patients who had a reduction in HVPG of > or =20% or to < or =12 mmHg in response to drug therapy are defined as responders. Responders have a markedly decreased risk of bleeding/rebleeding, ascites, and spontaneous bacterial peritonitis, which results in improved survival. This review provides clinical use of HVPG measurement in the field of liver disease.


Subject(s)
Chronic Disease , Hemodynamics , Hemorrhage/etiology , Hepatic Veins/physiology , Humans , Hypertension, Portal/complications , Liver Cirrhosis/diagnosis , Liver Diseases/complications , Portal Pressure
17.
Article in English | WPRIM | ID: wpr-190506

ABSTRACT

Variceal bleeding is common in chronic liver disease and is a frequent cause of acute upper gastrointestinal bleeding. The most common site of varices is the lower oesophagus but they may occur at any location where there are portosystemic anastomoses and collateral vascular formation. Location of ectopic varices at the site of enterocutaneous stomas is rare. We report on three cases of recurrent and severe bleeding from parastomal varices, requiring hospital admission. The patients had chronic liver disease but of different aetiological factors. Variceal formation results from portal hypertension due to chronic liver disease. There are various treatment options for parastomal variceal bleeding, including local, medical, and surgical interventions. Management of parastomal variceal bleeding presents a recurring and difficult problem. Bleeding may be considerable and sometimes life threatening. This diagnosis must be considered in patients with chronic liver disease presenting with stomal bleeding, even where the variceal formation may not be readily visible.


Subject(s)
Aged, 80 and over , Chronic Disease , Female , Gastrointestinal Hemorrhage , Humans , Liver Diseases/complications , Male , Middle Aged , Recurrence , Severity of Illness Index , Tomography, X-Ray Computed , Varicose Veins/complications
18.
GEN ; 67(2): 91-95, jun. 2013. ilus, graf, tab
Article in Spanish | LILACS | ID: lil-690968

ABSTRACT

Las lesiones focales del hígado comprenden un grupo heterogéneo de entidades como tumores benignos y malignos, sólidos o quistes, vasculares, masas inflamatorias y metástasis, entre otras. Reportar lesiones focales hepáticas en niños, características clínicas, estudios por imágenes y conducta terapéutica. Estudio descriptivo, retrospectivo, basado en la revisión de 16 historias clínicas de niños con lesiones focales hepáticas en el periodo comprendido entre enero 2007 a enero 2012. Se excluyeron los abscesos hepáticos. El 68,75%11 eran del sexo masculino y 31,25%5 del sexo femenino; con edad promedio de 9,5 años (rango: 10 días - 14 años). Las lesiones focales benignas fueron: hemangioma 4/16 (25%), hematoma hepático 4/16 (25%), quiste hepático 3/16 (18,75%), hiperplasia nodular focal 1/16 (6,25%) adenoma 1/16 (6,25%) y las malignas: hepatocarcinoma 2/16 (12,50%) y hepatoblastoma 1/16 (6,25%). La mayoría de los pacientes (56,25%) se encontraban asintomáticos, 3/16 (18,75%) presentaron dolor abdominal, 3/16 (18,75%) masa palpable y 2/16 (12,5%) ictericia. La lesión fue identificada por ultrasonido abdominal en todos los pacientes y sólo el 56,25% (9/16) ameritaron tomografía con contraste. Al 43,75% (7/16) se les realizó marcadores tumorales y de estos el 42,85% (3/7) reportaron neoplasias. El funcionalismo hepático reportó normal en el 75% de los casos. El ultrasonido abdominal es el método de elección para el diagnóstico y seguimiento de las lesiones focales hepáticas, mientras el origen de las mismas, guía la conducta terapéutica


Focal liver lesions comprise a heterogeneous group of entities such as benign and malignant, solid or cysts, vascular, inflammatory masses and metastasis among others. To report focal liver lesions in children, clinical features, imaging and therapeutic behavior. A descriptive, retrospective study based on a review of 16 case histories of children with focal liver lesions in the period from January 2007 to January 2012. Liver abscesses were excluded. 68.75%11 were male and 31.25%5 females, mean age 9.5 years (range: 10 days - 14 years). Focal lesions were benign: Hemangioma 4/16 (25%), liver hematoma 4/16 (25%), hepatic cyst 3/16 (18.75%), nodular focal hyperplasia 1/16 (6.25%) and adenomas 1/16 (6.25%) and malignant: Hepatocellular carcinoma 2/16 (12.50%) and hepatoblastoma 1/16 (6.25%). Most patients (56.25%) were asymptomatic, 3/16 (18.75%) had abdominal pain, 3/16 (18.75%) and palpable mass 2/16 (12.5%) jaundice. The lesion was identified by abdominal ultrasound in all patients and only 56.25% (9/16) needed tomography with contrast. To 43.75% (7/16) underwent tumor markers and of these 42.85% (3/7) reported neoplasm. Normal hepatic function reported in 75% of cases. An abdominal ultrasound is the method of choice for the diagnosis and management of focal liver lesions, while the origin of them, guide therapeutic conduct


Subject(s)
Female , Child , Adenoma, Liver Cell/pathology , Carcinoma/pathology , Hemangioma/pathology , Hematoma/pathology , Hepatoblastoma/pathology , Liver Diseases/complications , Liver/injuries , Gastroenterology , Pediatrics
19.
Rev. méd. Minas Gerais ; 23(1)jan.-mar. 2013.
Article in Portuguese, English | LILACS | ID: lil-702862

ABSTRACT

Objetivo: este trabalho analisa as alterações ultrassonográficas que caracterizam a síndrome de Cruveilhier-Baumgarten (SCB), com suas alterações clínicas. A SCB caracteriza-se pela veia paraumbilical proeminente, com dilatação das veias superficiais da parede abdominal de aspecto radiado provenientes do umbigo dos pacientes com hepatopatia crônica e hipertensão portal. Casuística e métodos: em avaliação ultrassonográfica de 60 pacientes com diagnóstico de hepatopatia crônica com hipertensão portal foram encontrados quatro casos com a SCB em que foi realizado o estudo por meio do doppler colorido. Resultados: todos os pacientes avaliados eram do gênero masculino, estavam na terceira década de vida, tinham história de esplenomegalia, varizes na parede abdominal e eram portadores de esquistossomose e aspecto histopatológico de cirrose micronodular. Foram analisados também dois pacientes com varizes do esôfago sem clinica de hematêmese. Ultrassonograficamente, todos os pacientes tinham sinais de hepatopatia crônica com esplenomegalia, com fluxo hepatopetal em todos os segmentos da veia porta e fluxo hepatofugal nas veias que deixam o fígado (veia paraumbilical no ligamento falciforme e nas veias superficiais da parede abdominal anterior). Conclusão: a SCB caracteriza-se pela associação de sinais de hepatopatia crônica com esplenomegalia, veias paraumbilicais com aumento de calibre (hipertensão porta) e com fluxo para as veias superficiais da parede abdominal, que se acham dilatadas na região umbilical e com sopro à ausculta pelo ultrassom e ao exame físico.


Objective: The purpose of this paper is to analyze the sonographic changes that characterize the Cruveilhier-Baumgarten syndrome and its clinical charactheristics. Cruveilhier- Baumgartem syndrome is characterized by a prominent paraumbilical vein, with dilatated superficial veins of the abdominal wall radiating from the umbilicus of patients with chronic liver disease and portal hypertension. Patients and methods: In a sonographic evaluation of 60 patients diagnosed with chronic liver disease with portal hypertension we found 4 cases of Cruveilhier-Baumgarten syndrome, which we studied with color doppler sonography.Results: All patients were male, in their third decade of life, with a clinical history of splenomegaly and varicose abdominal wall veins. Patients had schistosomiasis and micronodular cirrhosis on hystopathological examination. We also evaluated two patients with esophageal varices without hemathemesis. All patients had ultrasound signs of splenomegaly and chronic liver disease, with hepatopetal flow in all segments of the portal vein and hepatofugal flow in the efferent veins of the liver (paraumbilical vein in the falciform ligament and the anterior abdominal wall surface veins). Conclusion: CBS is charactherized by the associationof signs of chronic liver damage with splenomegaly, distention of paraumbilical veins (portal hypertension) and blood flow towards the superficial abdominal wall veins, which are dilated in the umbilical region, associated with an audible bruit in both clinical and sonographic examination.


Subject(s)
Humans , Liver Diseases/complications , Hypertension, Portal , Liver Cirrhosis , Splenomegaly , Ultrasonography, Doppler, Color
20.
Arch. pediatr. Urug ; 84(1): 32-34, mar. 2013.
Article in Spanish | LILACS | ID: lil-722860

ABSTRACT

Introducción: la hipertensión portal (HTP) es unaentidad poco frecuente en pediatría, peropotencialmente grave cuando ocurre el sangradodigestivo debido a várices esofágicas, con unamortalidad de hasta el 25%. Las enfermedades que ladeterminan evolucionan a menudo de forma silente,entorpeciendo el diagnóstico oportuno.Objetivo: conocer la presentación y causas quedeterminaron la HTP en los pacientes admitidos en unservicio de pediatría polivalente.Metodología: estudio descriptivo retrospectivoanalizando las historias clínicas de los pacientesingresados en el Departamento de Pediatría delSanatorio Americano entre enero de 2007 y diciembrede 2010, con diagnóstico de hipertensión portal,hepatopatía, hepatitis o patología digestiva.Resultados: ingresaron 3.581 niños, 173 presentaronlos diagnósticos mencionados. Se confirmó HTP en 6pacientes. Las patologías que provocaron la HTP.


Subject(s)
Humans , Male , Adolescent , Female , Child, Preschool , Child , Liver Diseases/complications , Hypertension, Portal/etiology
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