Trasplante hepático en paciente con cirrosis secundaria a glucogenosis tipo IIIa y carcinoma hepatocelular / Liver transplantation in patients with type IIIa glycogen storage disease, cirrhosis and hepatocellular carcinoma

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Liver transplantation in patients with type IIIa glycogen storage disease, cirrhosis and hepatocellular carcinoma.

Type III glycogen storage disease (GSD-III) is an autosomal recessive disorder due to the deficiency of the glycogen debrancher enzyme. 80% of the patients have hepatic and muscular involvement (IIIa), compared to 15% with only liver involvement (IIIb). As the life expectancy improves in these patients, the possible liver complications are better understood.

Gastric schwannoma presenting as a casual ultrasonographic findings.

We present the case of a patient under study due to ascites in which a mass located on the gastric wall was observed during ultrasonography. Further studies (upper endoscopy and computed tomography) confirmed this finding. After an ultrasound-guided percutaneous biopsy, diagnosis of gastric schwannoma was made as intense S-100 expression was found. Surgery was rejected due to the bad clinical situation of the patient and because the mass was an asymptomatic benign tumor.

Schwannoma gástrico como hallazgo ecográfico casual / Gastric schwannoma presenting as a casual ultrasonographic findings

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Hipertensión portal e ictericia colestásica como forma de presentación de una amiloidosis sistémica / Portal hypertension and cholestatic jaundice as a form of presentation of systemic amyloidosis

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Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review.

Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis.We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature.

Esclerosis hepatoportal en estadios clínicos evolutivos diferentes: presentación de 3 casos y revisión de la literatura / Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review

La esclerosis hepatoportal se caracteriza por hipertensión portal intrahepática presinusoidal asociada a esplenomegalia y anemia en pacientes con un hígado no cirrótico. La biopsia hepática es fundamental, sobre todo para descartar otros procesos. Se trata de un cuadro de etiología desconocida cuya gran mayoría de los casos se ha descrito en países orientales, si bien podría estar infradiagnosticada en Occidente. Se manifiesta con síntomas asociados a la hipertensión portal y el espectro clínico es muy amplio: desde anemia con pruebas de función hepática normales hasta hemorragia por varices esofagogástricas. Su tratamiento es el de las complicaciones y el pronóstico es mejor que en los pacientes con cirrosis. Presentamos tres casos de EHP que presentan estadios clínicos diferentes, así como los hallazgos de las biopsias hepáticas, su evolución clínica posterior y una revisión de la literatura científica(AU)

Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis. We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature(AU)

Abscesos esplénicos múltiples / Multiple splenic abscesses

Los abscesos esplénicos son una entidad clínica infrecuente en la práctica clínica, encontrándose mayormente en pacientes inmunocomprometidos. Presentamos el caso de un hombre de 81 años que ingresó en el hospital por fiebre, dolor abdominal e ictericia. La ecografía abdominal y la tomografía computerizada demostraron abscesos esplénicos múltiples. La esplenectomíay los antibióticos fueron un tratamiento efectivo. El drenaje percutáneo como alternativa podría ser apropiado solamente en pacientes seleccionados, con el objeto de preservar la función esplénica.(AU)

Multiple splenic abscesses is a rare condition in the clinical practice encountered mostly in immunocompromised patients. We report the case of a 81 year old male admitted to the hospital for fever, abdominal pain and jaundice. An abdominal ultrasound and computed tomography demonstrated multiple splenic abscesses. The splenectomy and antibiotics were a very effective therapy. The percutaneous drainage as an alternative may be appropriate only in selected patients, in order to preserve splenic function.(AU)

Abscesos esplénicos múltiples / Multiple splenic abscesses

Los abscesos esplénicos son una entidad clínica infrecuente en la práctica clínica, encontrándose mayormente en pacientes inmunocomprometidos. Presentamos el caso de un hombre de 81 años que ingresó en el hospital por fiebre, dolor abdominal e ictericia. La ecografía abdominal y la tomografía computerizada demostraron abscesos esplénicos múltiples. La esplenectomíay los antibióticos fueron un tratamiento efectivo. El drenaje percutáneo como alternativa podría ser apropiado solamente en pacientes seleccionados, con el objeto de preservar la función esplénica.

Multiple splenic abscesses is a rare condition in the clinical practice encountered mostly in immunocompromised patients. We report the case of a 81 year old male admitted to the hospital for fever, abdominal pain and jaundice. An abdominal ultrasound and computed tomography demonstrated multiple splenic abscesses. The splenectomy and antibiotics were a very effective therapy. The percutaneous drainage as an alternative may be appropriate only in selected patients, in order to preserve splenic function.

Hemorragia digestiva alta secundaria a divertículo duodenal ulcerado / Gastrointestinal bleeding secondary to ulcer in duodenal diverticulosis

Las causas más frecuentes de hemorragia digestiva alta son la úlcera péptica gástrica y duodenal, seguida por las erosiones agudas en mucosa gástrica y en tercer lugar las várices esofagogástricas. La diverticulosis de intestino delgado es una causa muy rara de hemorragia digestiva alta, aunque debe tenerse en cuenta en pacientes con hemorragia digestiva sin causa evidente a nivel esofagogástrico; lo habitual es diagnosticar esta entidad de forma accidental en el curso de estudios endoscópicos, radiológicos o quirúrgicos. La escasa incidencia de complicaciones asociada a la diverticulosis duodenal justifica el mantener una actitud no quirúrgica en principio

[Multiple splenic abscesses]. / Abscesos esplénicos múltiples.

Multiple splenic abscesses is a rare condition in the clinical practice encountered mostly in immunocompromised patients. We report the case of a 81 year old male admitted to the hospital for fever, abdominal pain and jaundice. An abdominal ultrasound and computed tomography demonstrated multiple splenic abscesses. The splenectomy and antibiotics were a very effective therapy. The percutaneous drainage as an alternative may be appropriate only in selected patients, in order to preserve splenic function.