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ABSTRACT: The Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction involving the hepatic veins, inferior vena cava, or both. BCS has occasionally been reported in the literature as a very rare complication of ulcerative colitis. However, association of Crohn's disease (CD) and BCS is extremely rare with only a single case reported in the world literature to date. We report a case of a young woman with chronically active, therapy-resistant CD who developed massive ascites, elevation of liver enzymes, and coagulopathy in the course of her disease. She was subsequently diagnosed with BCS for which a successful liver transplantation was performed. Chronically active therapy resistant CD and methylenetetrahydrofolate reductase gene mutation have been identified as possible risk factors for development of BCS in this patient.
European journal of gastroenterology & hepatology 07/2009; 22(6):761-4. · 1.66 Impact Factor
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ABSTRACT: Hemophilia A is an inherited bleeding disorder characterized by factor VIII deficiency. The basis for insufficient hemostasis lies within inadequate amplification of factor Xa production with the undersupplied factor VIII. We report on a young patient with critical aortic stenosis bearing all the clinical stigmata of severe hemophilia, in whom aortic valve replacement was performed with a tissue valve in order to avoid the need for long term anticoagulation.
Collegium antropologicum 04/2007; 31(1):355-7. · 0.61 Impact Factor
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Igor Aurer,
Zdravko Mitrović,
Jasminka Kovacević-Metelko,
Ivo Radman,
Sandra Basić-Kinda,
Koraljka Gjadrov-Kuvezdić,
Jasminka Jakić-Razumović,
Klara Dubravcić,
Suncica Ries,
Marin Nola, Silva Zupancic-Salek,
Boris Labar
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ABSTRACT: Hairy cell leukemia is a chronic B-cell lymphoproliferative disorder characterized by clonal proliferation of hairy cells. Treatments of choice are purine analogues, particularly cladribine. We treated thirty patients with cladribine either by continuous 7-day infusion at a daily dose of 0.1 mg/kg or by 2-h infusion for 5 consecutive days at a daily dose of 0.14 mg/kg. Remission was achieved in 90% of the patients. After a median follow-up of 44 months overall survival is 93% and time to treatment failure more than 6 years. Two patients did not respond, one patient died of infection shortly after the treatment. Side-effects resulted mainly from hematological toxicity, 23% of the patients had neutropenic fever while 20% required platelets or packed red cell transfusions. Our results show that cladribine is safe and effective in the treatment of hairy cell leukemia. There were no significant differences in toxicity and response between 7-day continuous infusion and 5-day intermittent infusions of the drug.
Lijec̆nic̆ki vjesnik 129(3-4):80-3.
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ABSTRACT: Although non-melanoma skin cancers are the most predominant malignancies in the Caucasian population and hemophilia A is one of the most frequent hereditary bleeding disorders, medical literature data about the management of non-melanoma skin cancers in patients with hemophilia are surprisingly scarce. In this case report we describe the treatment of a patient with multiple recurrent non-melanoma skin cancers and severe hemophilia A. The management of such patients could be very challenging, with possible significant bleeding complications, and requires a multidisciplinary approach.
Tumori 95(1):115-8. · 0.86 Impact Factor
