الفهرس 
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Abstract
he current work was designed to study the frequency and the pattern of IVC involvement in Egyptian patients with BCS. Also to demonstrate its relation to etiology and impact on clinical presentation in these patients.
To fulfill such purpose, we studied 50 patients with confirmed BCS. All patients were subjected to detailed history taking, clinical examination, radiological assessment with duplex U/S, MRV or multislice CT scan, and laboratory investigations including: liver profile tests, kidney function tests, complete blood picture, thrombophilia work up.
In the current study, male and female patients were equally presented (50% for each). Mean age of the studied patients at first visit was 25.28±6.08 years for males and 29.24±8.09 years for females.
Most of the patients in our study were diagnosed during the chronic phase of the disease (76%), the rest of patients were diagnosed during acute (10%) and fulminant (14%) stages.
In the current study, it was found that the relevant symptoms in 50 patients with BCS at the time of diagnosis were abdominal enlargement in 84%, abdominal pain in 74%, lower limb swelling in 30% and history of DVT in 12%, and bouts of encephalopathy in 4% of patients.
As regards the signs, hepatomegaly was present in 86% and the liver was tender in 20%. Ascites was evident in 84%, splenomegaly in 70%, lower limb oedema in 30%, jaundice in 42% and dilated veins over the trunk and body in 34% of the cases. In the current study 1% of patients were asymptomatic.
Regarding the pattern of vascular involvement in the current series we found isolated HVs obstruction in 34%, isolated IVC occlusion in 2%, combined HVs and IVC obstruction in 36% while combined HVs and IVC compression was reported in 40% of the cases.
IVC compression in the current study was documented in 40% of the recruited patients and it was the most common pattern of IVC involvement followed by IVC stenosis, IVC webs and IVC occlusion (14%, 12%, and 10%) of patients respectively.
As regards HVs Color Doppler findings in the current study we identified aphasic or flat flow waveform in all patients with HVs occlusion group, but in patients with HVs stenosis 22% had biphasic flow and 6% showed monophasic flow waveform.
As regards IVC Color Doppler findings in the current study we identified aphasic or flat flow waveform in all patients with IVC occlusion group, however, in patients with IVC stenosis, 57% had monophasic flow and 43% showed biphasic flow. In patients with IVC compression 45% show monophasic and 55% biphasic. Fnially, in patients IVC webs 50% had monophasic and 50% showed biphasic flow waveform.
In the current study we identified that 72% had occlusion in all three hepatic veins (right, middle, and left), 18% had occlusion in two veins, and 10% had single HV vein involvement.
Regarding the impact of the pattern of vascular involvement on the clinical presentation of BCS patients, we have tried to find any correlation between the Clinical presentations and the pattern of vascular involvement specially IVC involvement in BCS (after adding patients with isolated IVC involvement to patients with combined occlusion, due to the small number of of patients in the former). There was only a statistically significant association between Dilated Vs. over body and trunk and the IVC involvement (either obstructed or compressed).
In the current study, Factor V Leiden mutation (FVLM) was the most common cause and this was documented in 68.4% patients (42.1% of patients were heterozygous and 26.3% of patients were homozygous). Another frequently observed etiology in our study was (MTHFR) gene mutation being documented in 55.5% of patients (50% of them was heterozygous and 5.5% of them were homozygous), it was considered the second common cause in our study.
Antiphopholipid Ab syndrome in our series was found in 28.5% of the cases, where anticardiolipin IgM antibodies were found in 16.6% and anticardiolpin IgG antibodies were found in 9.7% of cases but lupus anticoagulant was present in 28.5% of cases. In the current study we identified myeloproliferative disease in 28.5% of patients. We confirmed these disorders by JAK2 V617f mutation. None of the currently studied patients with BCS had prothrombin gene mutation,
In the current study, Behcet’s disease was documented in 4% of the studied patients. We identified protein C deficiency, protein S deficiency and antithrombin III deficiency in (40.6%, 6% and 35.4%) of the cases respectively.
Regarding the relation between the etiology and the pattern of vascular involvement in BCS patients, there was no statistically significant association in the current study, although all patients with Behcets disease have Combined HVS and IVC obstruction.