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Abstract B udd-Chiari syndrome (BCS)is a condition result from partial or complete obstruction of the hepatic venous outflow system which can lead to liver cirrhosis secondary to centrilobular congestion and hepatic necrosis (Saleh et al 2013). Liver fibrosis expresses the severity of liver diseases, regardless of etiology; it predicts the evolution of liver diseases towards cirrhosis with subsequent complications, cirrhosis is the seventh death cause within general mortality (Braticevici et al., 2011). Liver biopsy is a method neither ideal nor sufficient to diagnose and determine the stage of liver fibrosis because fibrosis is a dynamic process.The identification of a noninvasive method for the evaluation of liver fibrosis and evaluate the efficiency of antifibrotic treatments is indicated (Braticevici et al., 2011). Angiotensin converting enzyme is a zinc dependent dipeptidase that catalyzes the conversion of the decapeptide angiotensin I to the potent vasopressor octapeptide angiotensin II. Following liver injury, ACE is markedly expressied and increased and in localized to areas of active fibrogenesis. It involved in the pathogenesis of chronic tissue injury and fibrosis by increasing AngII, which induces contraction and proliferation of HSCs via the AT1 receptor and increase TGF-β1 expression (Grace et al., 2012). In this regard, the present study aimed to evaluate the clinical utility of ACE as a marker of liver fibrosis in Budd-Chiari syndrome patients. Moreover, the association between Liver fibrosis& serum levels of ACE were also investigated. This study was conducted at the department of Tropical Medicine and Infectious Diseases at Ain Shams University hospital on 50 Budd–Chiari syndrome patients with liver fibrosis stage III assessed by fibroscan (group I), 50 HCV liver fibrosis patients stage III determined by METAVIR scoring system (group II), in addition to 30 healthy control subjects (group III) with matched age and sex. All subjects included in this study were subjected to full history taking, clinical examination, radiological investigations including CT scan and abdominal ultrasound. Assessment of liver fibrosis by liver biopsy or fibroscan for patients. laboratory investigations included CBC, FBS, liver profile (total and conjugated bilirubin, serum ALT, AST, ALP, total proteins and albumin),AFP, liver fibrosis indices (AAR, APRI and FIB4) were calculated as well as assay of ACE level by enzyme linked immunosorbent assay (ELISA). In conclusion, ACE significantly increased in stage III liver fibrosis of Budd–Chiari syndrome patients and HCV patients compared to healthy control. In addition, lower ACE serum levels were associated with BCS patient’s related fibrosis in respect to HCV related fibrosis.Moreover,a significant positive correlation was detected between ACE serum levels andboth APRI and FIB4 in the two groups Therefore, ACE serum level could be a promising non-invasive marker of liver fibrosis in Budd–Chiari syndrome patients |