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Autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangitis are chronic autoimmune liver disease, usually present separate, the cases where characteristics of two of the above is observed liver disease is commonly referred to as Overlap Syndromes (OS). Although there is no consensus on specific criteria for the diagnosis of OS identification of this association is important for initiating appropriate treatment and prevent its progression to cirrhosis or at least the complications of cirrhosis and death. We report the case of awoman aged 22 cirrhotic which debuted are edematous ascites, severe asthenia and jaundice compliant diagnostics SS criteria and initially present any response to treatment with ursodeoxycholic acid and oral corticosteroids, but ultimately finished performing a transplant orthotopic liver.

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La hepatitis autoinmune, cirrosis biliar primaria, colangitis esclerosante primaria y colangitis autoinmune son hepatopatías crónicas de origen autoinmune, habitualmente se presentan por separado, los cuadros donde se observa características de dos de las mencionadas hepatopatías se designan comúnmente como síndromes de superposición (SS). Aunque no existe consenso sobre criterios específicos para el diagnóstico de SS la identificación de esta asociación es importante para iniciar un tratamiento adecuado y así evitar su evolución hacia la cirrosis o en todo caso las complicaciones de la cirrosis y muerte. Presentamos el caso de una mujer de 22 años cirrótica que debuto son síndrome ascítico edematosa, intensa astenia e ictérica que cumple los criterios diagnósticos del SS y que inicialmente presentó alguna respuesta al tratamiento con ácido ursodexosicólico y corticoides orales, pero que finalmente terminó realizándose un trasplante hepático ortotópico...

Autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangitis are chronic autoimmune liver disease, usually present separate, the cases where characteristics of two of the above is observed liver disease is commonly referred to as Overlap Syndromes (OS). Although there is no consensus on specific criteria for the diagnosis of OS identification of this association is important for initiating appropriate treatment and prevent its progression to cirrhosis or at least the complications of cirrhosis and death. We report the case of a woman aged 22 cirrhotic which debuted are edematous ascites, severe asthenia and jaundice compliant diagnostics SS criteria and initially present any response to treatment with ursodeoxycholic acid and oral corticosteroids, but ultimately finished performing a transplant orthotopic liver...

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La enfermedad de Wilson (EW) es un trastorno del metabolismo del cobre que se hereda de forma autosómica recesiva, lo cual produce acumulación tóxica del cobre principalmente en el hígado y el cerebro, en general tiene dos formas de presentación, la hepática en edades tempranas y la neurológica en edades más tardías. Se presenta el caso de una paciente mujer de 21 años diagnosticada de EW en su forma hepática en estadio cirrosis que debutó con un síndrome ascítico edematoso sin ninguna manifestación neurológica a pesar de su edad. En sus estudios de laboratorio presentó descenso de la ceruloplasmina sérica y cupruria elevada en 24 horas, datos característicos de esta enfermedad. Aunque la EW no es una enfermedad común debe ser sospechada en toda hepatopatía crónica de etiología no determinada con marcadores virales y de autoinmunidad negativos con o sin manifestaciones neurológicas ya que su reconocimiento temprano e inicio del tratamiento con quelantes del cobre principalmente conlleva a una mejora sustancial del pronóstico de vida de estos pacientes...

Wilson disease (WD) is a disorder of copper metabolism that is inherited as an autosomal recessive, which produces toxic copper accumulation mainly in the liver and brain, in general has two ways presentation, liver at early ages and neurological in later ages. We present the case of a female patient of 21 years diagnosed of WD in liver cirrhosis that started with an edematous ascites without any neurological symptoms despite the age. Their laboratory studies showed decrease in serum ceruloplasmin and high cupruria within 24 hours of the disease , characteristic data of WD. Although WD is not a common disease should be suspected in all chronic liver disease of unknown etiology with negative viral markers and autoimmunity with or without neurological manifestations as soon as posible and starting treatment with copper chelating mainly leads to a substantial improvement the prognosis of these patients...

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Wilson disease (WD) is a disorder of copper metabolism that is inherited as an autosomal recessive, which produces toxic copper accumulation mainly in the liver and brain, in general has two ways presentation, liver at early ages and neurological in later ages. We present the case of a female patient of 21 years diagnosed of WD in liver cirrhosis that started with an edematous ascites without any neurological symptoms despite the age. Their laboratory studies showed decrease in serum ceruloplasmin and high cupruria within 24 hours of the disease , characteristic data of WD. Although WD is not a common disease should be suspected in all chronic liver disease of unknown etiology with negative viral markers and autoimmunity with or without neurological manifestations as soon as posible and starting treatment with copper chelating mainly leads to a substantial improvement the prognosis of these patients.

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OBJECTIVE: To determine the clinical characteristics of patients with thromboembolic events (TEE) included in the National Register of Inflammatory Bowel Diseases of Uruguay (RNEII). MATERIAL AND METHODS: From the RNEII database of 515 patients, an analysis of case series was made. Medical records of 23 patients with diagnosis of ETE were reviewed Image studies were required for diagnosis of deep thrombosis, while superficial thromboflebitis diagnosis was clinical. RESULTS: Eight patients who did not meet the inclusion criteria were excluded. Of the remaining fifteen patients (2.9%), 11 had ulcerative colitis (CUC) and 4 Crohn's disease (CD). The mean age was 42.1 years (range 12 to 64 years). 40% presented this complication before the 40 years old and the mortality caused by TEE was 6.7%. 53.3 of cases had active disease when the TEE presented. 63.6% of CUC patients developed an extensive colitis, while 100% of patients with CD had colonic involvement. Another extraintestinal manifestation was seen in 46.7% of patients, specially in the CUC group. Deep vein thrombosis was the most frequent TEE (66.7%). 40% of patients repeated more than one TEE episode. CONCLUSIONS: TEE in inflammatory bowel disease are an important morbimortality factor because they can occur in young people, be recurrent and affect unusual vascular sites. TEE may be observed in both active and quiescent disease. They have been more frequently found in patients with extensive disease, colonic involvement and other extraintestinal manifestations.

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