Budd-Chiari syndrome and extrahepatic portal obstruction associated with congenital antithrombin III deficiency

2001 ◽  
Vol 36 (5) ◽  
pp. 341-345 ◽  
Author(s):  
Hiroshi Nakase ◽  
Toshihiko Kawasaki ◽  
Toshinao Itani ◽  
Jun Mimura ◽  
Hideshi Komori ◽  
...  
2011 ◽  
Vol 106 ◽  
pp. S288
Author(s):  
Alpaslan Tanoglu ◽  
Yusuf Yazgan ◽  
Kemal Oncu ◽  
Mustafa Kaplan ◽  
Irfan Kucuk ◽  
...  

2003 ◽  
Vol 70 (12) ◽  
pp. 1003-1005 ◽  
Author(s):  
Amit Jagtap ◽  
Preeti Shanbag ◽  
Mamta Vaidya

1982 ◽  
Vol 78 (2) ◽  
pp. 236-241 ◽  
Author(s):  
Stephen Mcclure ◽  
Hosoon P. Dincsoy ◽  
Helen Glueck

Swiss Surgery ◽  
2001 ◽  
Vol 7 (3) ◽  
pp. 141-144
Author(s):  
Gygax ◽  
Berdat ◽  
Carrel

Wir berichten über einen Patienten mit Budd-Chiari Syndrom welcher unter intravenöser Antikoagulation eine Heparin induzierte Thrombozytopenie entwickelte. Die chirurgische Behandlung bestand aus einer dorsocranialen Leberresektion mit anschliessender hepato-atrialer Anastomose unter Verwendung der extrakorporellen Zirkulation. Die perioperative Antikoagulation wurde mittels Hirudin durchgeführt. Erstaunlicherweise wurde während der Operation eine Thrombusbildung im Kardiotomie-Reservoir der Herz-Lungenmaschine beobachtet, obwohl die intraoperativ gemessene Antikoagulationsparameter (ACT und aPTT) im therapeutischen Bereich waren. Mit einem zusätzlichen Bolus Hirudin in das extrakorporelles Circuit und dank Spülung des Reservoirs konnte die Operation ohne weitere thrombotische Ereignisse zu Ende geführt werden.


1990 ◽  
Vol 63 (01) ◽  
pp. 013-015 ◽  
Author(s):  
E J Johnson ◽  
C R M Prentice ◽  
L A Parapia

SummaryAntithrombin III (ATIII) deficiency is one of the few known abnormalities of the coagulation system known to predispose to venous thromboembolism but its relation to arterial disease is not established. We describe two related patients with this disorder, both of whom suffered arterial thrombotic events, at an early age. Both patients had other potential risk factors, though these would normally be considered unlikely to lead to such catastrophic events at such an age. Thrombosis due to ATIII deficiency is potentially preventable, and this diagnosis should be sought more frequently in patients with arterial thromboembolism, particularly if occurring at a young age. In addition, in patients with known ATIII deficiency, other risk factors for arterial disease should be eliminated, if possible. In particular, these patients should be counselled against smoking.


1985 ◽  
Vol 54 (04) ◽  
pp. 744-745 ◽  
Author(s):  
R Vikydal ◽  
C Korninger ◽  
P A Kyrle ◽  
H Niessner ◽  
I Pabinger ◽  
...  

SummaryAntithrombin-III activity was determined in 752 patients with a history of venous thrombosis and/or pulmonary embolism. 54 patients (7.18%) had an antithrombin-III activity below the normal range. Among these were 13 patients (1.73%) with proven hereditary deficiency. 14 patients were judged to have probable hereditary antithrombin-III deficiency, because they had a positive family history, but antithrombin-III deficiency could not be verified in other members of the family. In the 27 remaining patients (most of them with only slight deficiency) hereditary antithrombin-III deficiency was unlikely. The prevalence of hereditary antithrombin-III deficiency was higher in patients with recurrent venous thrombosis.


1979 ◽  
Author(s):  
J. Conard ◽  
M. Samama ◽  
M. H. Horellou ◽  
B. Cazenave ◽  
P. Griguer ◽  
...  

A congenital Antithrombin III (AT III) deficiency affecting 7 members of 3 families is reported.The first throrabo-embolic accidents were observed between the age of 22 and 35 : they were spontaneous or occured after delivery or oral contraception. in one patient, a deep vein thrombosis was observed during heparin treatment. in 2 cases, recurrent pulmonary embolic episodes required vena cava ligation. No thromboembolic accident was observed during oral anticoagulation.AT III was measured by an amidolytic method and by the Mancini method on plasma and serum ; the antithrombin activity was determined on serum by the von Kaulla method. in 7 patients, a decreased AT III was found by all the methods performed. The AT III level was around 50 % in patients treated or not by oral anticoagulants One patient was studied during heparin treatment and then under oral anticoagulants : AT III levels were lower under heparin.


MedPharmRes ◽  
2018 ◽  
Vol 2 (3) ◽  
pp. 22-26
Author(s):  
Uyen Vo ◽  
Duc Quach ◽  
Luan Dang ◽  
Thao Luu ◽  
Luan Nguyen

Budd–Chiari syndrome (BCS), a rare and life-threatening disorder due to hepatic venous outflow obstruction, is occasionally associated with hypoproteinemia. We herein report the first case of BCS with segmental obstruction of the intrahepatic portion of inferior vena cava (IVC) and hepatic veins (HVs) successfully treated by endovascular stenting in Vietnam. A 32-year-old female patient presented with a 2-month history of massive ascites and leg swelling. She refused history of oral contraceptives use. Hepatosplenomegaly without tenderness was noted. Laboratory data showed polycythemia, mild hypoalbuminemia and hypoproteinemia, slightly high total bilirubin and normal transaminase level. The serum ascites albumin gradient was 1.9 g/dL and ascitic protein level was 1.1 g/dL. The other data were normal. BCS was suspected because of the discrepancy between mild liver failure and massive ascites; and the presence of hepatosplenomegaly and polycythemia. On abdominal magnetic resonance imaging, the segmental obstruction of three HVs and IVC was 2-3 cm long without thrombus. Cavogram revealed the severe segmental stenosis of intrahepatic portion of IVC with no visualized HV and extensive collateral veins. A Protégé stent was deployed to IVC. Leg swelling and ascites were completely resolved within 3 days after stenting. During 1-year follow-up, edema was not recurred and repeated laboratory results were all normal.


1985 ◽  
Vol 21 (3) ◽  
pp. 473
Author(s):  
J H Lee ◽  
E K Kim ◽  
Y T Ko ◽  
Y Yoon ◽  
S W Lee ◽  
...  

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