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| 1 | Embolization of splenorenal shunt associated to portal vein thrombosis and hepatic encephalopathy显示文摘Hepatic encephalopathy(HE)is a cognitive disturbance characterized by neuropsychiatric alterations.It occurs in acute and chronic hepatic disease and also in patients with portosystemic shunts.The presence of these portosystemic shunts allows the passage of nitrogenous substances from the intestines through systemic veins without liver depuration.Therefore,the embolization of these shunts has been performed tocontrol HE manifestations,but the presence of portal vein thrombosis is considered a contraindication.In this presentation we show a cirrhotic patient with severe HE and portal vein thrombosis who was submitted to embolization of a large portosystemic shunt.Case report:a 57 years-old cirrhotic patient who had been hospitalized many times for persistent HE and hepatic coma,even without precipitant factors.She had a wide portosystemic shunt and also portal vein thrombosis.The abdominal angiography confirmed the splenorenal shunt and showed other shunts.The larger shunt was embolized through placement of microcoils,and the patient had no recurrence of overt HE.There was a little increase of esophageal and gastric varices,but no endoscopic treatment was needed.Since portosystemic shunts are frequent causes of recurrent HE in cirrhotic patients,portal vein thrombosis should be considered a relative contraindication to perform a shunt embolization.However,in particular cases with many shunts and severe HE,we found that one of these shunts can be safely embolized and this procedure can be sufficient to obtain a good HE recovery.In conclusion,we reported a case of persistent HE due to a wide portosystemic shunt associated with portal vein thrombosis.As the patient had other shunts,she was successfully treated by embolization of the larger shunt. | Letícia de Campos Franzoni Fábio Cardoso de Carvalho Rafael Gomes de Almeida Garzon Fábio da Silva Yamashiro Laís Augusti Lívia Alves Amaral Santos Mariana de Souza Dorna Júlio Pinheiro Baima Talles Bazeia Lima Carlos Antonio Caramori Giovanni Faria Silva Fernando Gomes Romeiro | 2014 | World Journal of Gastroenterology2014,20,42: | 6 |
| 2 | Juvenile hemochromatosis:HAMP mutation and severe iron overload treated with phlebotomies and deferasirox显示文摘Juvenile hemochromatosis(JH) is a rare condition classified as an autosomal recessive disorder that leads to severe iron absorption. JH usually affects people under the age of 30 and presents symptoms such as chronic liver damage,hypogonadotropic hypogonadism,cardiac diseases and endocrine dysfunctions. The present case reports a 29-year-old Brazilian woman with JH condition due to HAMP mutation(g.47G>A),treated with phlebotomies and deferasirox. She presented symptoms such as weakness,skin hyperpigmentation,joint pain in the shoulders and hands and amenorrhea. First laboratory tests showed altered biochemical parameters [serum ferritin(SF): 5696 ng/mL,transferrin saturation(TS): 85%]. After sessions of phlebotomies(450 mL every 15 d),the patient presented partial symptomatic improvements and biochemical parameters(SF: 1000 ng/mL,Hb: 11 g/dL). One year later,deferasirox(15 mg/kg per day) was introduced to the treatment,and the patient showed total symptomatic improvement,with significant clearing of the skin,SF: 169 ng/mL,and TS: 50%. Furthermore,after the combined deferasirox-phlebotomy therapy,magnetic resonance imaging measurements revealed normalized level for liver iron(30 μmol/g; reference value < 36 μmol/g). In conclusion,combined deferasirox-phlebotomy treatment was able to normalize iron levels and improve symptoms. | Manuel A Lescano Letícia C Tavares Paulo C J L Santos | 2017 | World Journal of Clinical Cases2017,5,10: | 2 |
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