ABSTRACT
INTRODUÇÃO: A síndrome hepatopulmonar é complicação das doenças hepáticas que afeta a vascularização pulmonar, causando comprometimento do sistema respiratório e acompanha-se de elevada morbidade. O transplante hepático é o tratamento ideal para esses casos. O objetivo desse relato é descrever o caso de um paciente que apresentava síndrome hepatopulmonar avançada, devida à síndrome da obstrução sinusoidal.RELATO DO CASO: Homem de 23 anos foi encaminhado com o diagnóstico de síndrome hepatopulmonar avançada e cirrose hepática sem etiologia idefinida. Exames de imagem foram compatíveis com shunt intrapulmonar, porém sem alterações da vascularização pulmonar factíveis de serem embolizadas. A endoscopia digestiva alta demonstrou varizes esofágicas de pequeno calibre. Exames laboratoriais para função hepática estavam alterados. O paciente foi submetido a transplante hepático falecendo 14 dias após o procedimento devido a complicações relacionadas à sepse e insuficiência ventilatória refratária.CONCLUSÃO: Na vigência de síndrome hepatopulmonar o único tratamento efetivo é o transplante hepático, para os casos em que não houver área de shunt factível de ser realizada a embolização seletiva.
INTRODUCTION: The hepatopulmonary syndrome is a complication of liver diseases that affects the pulmonary vascular system compromising respiratory function. High morbidity rates are associated with this syndrome. Liver transplantation is the treatment of choice. The aim of this report is to present the case of a patient who sustained advanced hepatopulmonary syndrome resulting from the sinusoidal obstruction syndrome.CASE REPORT: A 23-year-old male was referred with the diagnosis of advanced hepatopulmonary syndrome and liver cirrhosis of undefined etiology. Imaging studies were consistent with intrapulmonary shunt yet without alterations in pulmonary vascularization amenable to embolization. Upper digestive endoscopy demonstrated small-caliber esophagian varices. Laboratory tests for liver function showed alterations. The patient underwent liver transplantation and died 14 days after, due to sepsis-related complications and refractory ventilatory failure.CONCLUSION: In cases when it is not feasible to perform selective embolization in the shunt area, the only effective treatment for hepatopulmonary syndrome is liver transplantation.
Subject(s)
Humans , Male , Young Adult , Liver Cirrhosis/etiology , Endoscopy, Digestive System , Hepatic Veno-Occlusive Disease/complications , Hepatopulmonary Syndrome/complications , Liver Transplantation/mortalityABSTRACT
We report a previously healthy 29 years old man, presenting with a sudden episode of abdominal pain, mild jaundice, hepatomegaly and ascites. Magnetic resonance imaging study and liver biopsy were compatible with veno-occlusive disease. Incidentally, an ulcerative colitis and portal vein thrombosis were diagnosed. Anticoagulant treatment was started, with good clinical and radiological response. Veno-occlusive disease of the liver must be suspected In cases of liver failure and ascites associated to procoagulant conditions.
Subject(s)
Adult , Humans , Male , Colitis, Ulcerative/diagnosis , Hepatic Veno-Occlusive Disease/diagnosis , Venous Thrombosis/diagnosis , Portal Vein , Incidental Findings , Biopsy , Diagnostic Imaging , Liver/pathology , Hepatic Veno-Occlusive Disease/complications , Hypertension, Portal/diagnosisABSTRACT
The relationship between essential fatty acid [EFA] status and degree of hyperbilirubinaemia and oxidant stress in infants and children with chronic liver diseases was evaluated. Thirty patients with chronic cholestasis and 30 with liver cirrhosis were examined; 30 healthy subjects served as controls. Patient groups had significant decreases in EFAs and significant elevation of total bilirubin. Levels of thiobarbituric acid reactive substances were significantly raised and were significantly inversely correlated to decreased EFA levels. There were also significant decreases in retinol, alpha-tocopherol and alpha-tocopherol/total lipids ratio, which had significant positive correlations with decreased EFA levels. Infants and children with chronic liver diseases have a high risk of EFA deficiency correlated with progressive elevation of serum bilirubin and progressive deterioration of oxidant status
Subject(s)
Adolescent , Child , Female , Humans , Male , Biliary Atresia/complications , Bilirubin/blood , Case-Control Studies , Child, Preschool , Chronic Disease , Fatty Acids, Essential/blood , Glycogen Storage Disease Type III/complications , Hepatic Veno-Occlusive Disease/complications , Hyperbilirubinemia/etiology , Oxidative Stress/physiologyABSTRACT
Objetivo: Alertar os pediatras para uma doença decorrente do uso de ervas consideradas inócuas. Métodos: Descriçä de um caso de hipertensäo porta de início súbito, secundária à Doença Veno-Oclusiva Hepátiaca em um paciente de 2 anos e 5 meses, proveniente de Cruz Alta, RS, após uso crônico de chás de "maria-mole" (Senecio brasiliensis), que contém alcalóides pirrolizidínicos, sabidamente hepatotóxicos. após tratamento adequado, apresentou recuperaçäo clínica e laboratorial completa e hoje encontra-se assintomático. Conclusöes: A prevençäo dessa doença depende da sua divulgaçäo entre médicos e populaçäo em geral, bem como do conhecimento dos efeitos tóxicos de muitos chás.
Subject(s)
Humans , Male , Child, Preschool , Hepatic Veno-Occlusive Disease/chemically induced , Hepatic Veno-Occlusive Disease/complications , Hypertension, Portal/etiology , Pyrrolizidine Alkaloids/adverse effects , Ascites/etiology , Hepatomegaly/etiology , Venules/pathologyABSTRACT
Introducción. La enfermedad venooclusiva del hígado es un padecimiento raro en niños y de manera ocasional se ha descrito en lactantes en los que se confunde con otras causas más comunes de colestasis. Caso clínico. Se presenta un caso de enfermedad venooclusiva en un lactante de 36 días de vida portador de síndrome de Down, producto de gesta IV, para III, de madre de 26 años de edad. Sin antecedentes de ingesta de medicamentos, drogas o infusiones durante el embarazo, que se sabe están asociadas al desarrollo de esta enfermedad. Clínicamente se caracterizó por la presencia de ascitis, hepatomegalia y de ictericia que en pacientes adultos con factores de riesgo orientan al diagnóstico; en el período neonatal no es un padecimiento que se considere en el diagnóstico diferencial de colestasis. Conclusión. Las alteraciones hepáticas en niños con síndrome de Down son variadas, pero la enfermedad venooclusiva del hígado no ha sido previamente informada