الفهرس 
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Abstract
B
udd-Chiari syndrome (BCS) is a rare but potentially life threatening hepatic disorder that results from obstruction of the hepatic venous outflow tract. Obstruction can occur at any level from the hepatic venules to the right atrium
According to the etiology, BCS can be classified as primary (due to intrinsic intraluminal thrombosis or webs) or secondary (due to intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, cyst or solid tumor)
The goals of treatment are to prevent extension of thrombosis in the hepatic veins and to alleviate venous obstruction in order to decrease hepatic congestion. Few patients respond to medical treatment (anticoagulation with or without thrombolytic therapy, diuretics). However, most of patients need more invasive procedures to restore the hepatic blood flow including percutaneous angioplasty with or without stenting, transjugular intrahepatic portosystemic shunt (TIPS) or shunt surgery.
Antiphospholipid syndrome (APS) is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. Some patients with APS have no evidence of any definable associated disease, while, in other patients, APS occurs in association with SLE or another rheumatic or autoimmune disorder. Traditionally, these have been referred to as primary or secondary APS, respectively.
This retrospective study aims to assess the prevalence of Antiphospholipid syndrome (APS) in primary Budd Chiari syndrome (BCS) patients and their management outcome.
Patients presented to BCS outpatient clinic, Tropical medicine department, Ain Shams University Hospitals were enrolled in the study from 2010 to 2020.
The included patients were adult patients with confirmed APS and primary BCS of both genders.
History, clinical examination, pre and post intervention laboratory data were collected .Duplex ultrasound testing the stent patency was done after intervention at 1, 3 and 6 months.
The included patients were seventy-two BCS patients with APS as a single etiology. The mean age of the patients was (29.26 ± 8.16) with female predominance, Females were 53% in the studied population. The main presentations were ascites (61.1%) and abdominal pain (15.3%).
Regarding laboratory data, Lupus Anticoagulant, ACL IgG antibodies and ACL IgM antibodies were positive in 60 (83.3%), 49 (68.1%) and 27 (37.5%) patients respectively.
For 42 patients (58.3%), intervention was performed. TIPSS was performed on 33 (45.8%) patients, while Angioplasty with Stent was performed on 9 (12.5%) patients. There was no intervention in 30 (41.7%) of the patients.
In the studied patients, rate of mortality of the total 72 patients was 48.6%.
The mean cause of death was Hepatorenal syndrome (31.4%). The mean survival time was 65.68 months.
Rate of mortality in the intervention group and medical treatment group was 73 % and 31% respectively with a highly significant statistical difference.
The mean survival time for the intervention group and medical treatment group was 90.03 months and 17.597 months respectively. That shows a highly significant statistical difference.
Rate of stent occlusion after 6 months in APS intervention group was 47.5 % while it was 35.5% in the other etiologies group.
This means that rate of stent occlusion is higher in patients with BCS due to APS than in patients with BCS due to other etiologies.