We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT level...We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.展开更多
Background and Aims: Psychometric hepatic encephalop-athy score(PHES)is used widely for diagnosis of minimal hepatic encephalopathy(MHE).This prospective study aimed to determine the utility of the inhibitory control ...Background and Aims: Psychometric hepatic encephalop-athy score(PHES)is used widely for diagnosis of minimal hepatic encephalopathy(MHE).This prospective study aimed to determine the utility of the inhibitory control test(ICT)for the diagnosis of MHE.Additionally,the efficacy of rifaximin and lactulose for reversal of MHE was evaluated.Methods:A total of 180 eligible cirrhotic patients underwent testing for MHE.When PHES was ≤ ?5 and ICT lures were ≥ 14,MHE was diagnosed.The 108 patients with MHE were randomized to three groups for treatment with either lactulose,rifaximin,or placebo.Treatment outcomes were measured at the end of 3 months.Results: The 108 patients with MHE diagnosed by PHES and/or ICT accounted for 60%.The diagnosis of MHE was made by both ICT and PHES positivity in 56 patients,by abnormal ICT and normal PHES in 37 patients,and by abnor-mal PHES and normal ICT in 15 patients.For diagnosis of MHE,ICT had sensitivity of 78.87%,specificity of 66.06%with 60.22%positive predictive value and 82.76%negative predictive value.An area under the curve value of 0.724(95%CI: 0.653–0.788)was obtained for diagnosis of MHE.Reversal of MHE was seen in 71.42%,70.27%and 11.11%of patients in the rifaximin,lactulose and placebo arms(p < 0.001).Rifaximin showed better tolerability compared to lactulose.Conclusions: For the diagnosis of MHE,ICT is a simple tool but has lower sensitivity and better specificity than PHES.Rifaximin is as efficacious as lactulose in the treat-ment of MHE and better tolerated.展开更多
文摘We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.
文摘Background and Aims: Psychometric hepatic encephalop-athy score(PHES)is used widely for diagnosis of minimal hepatic encephalopathy(MHE).This prospective study aimed to determine the utility of the inhibitory control test(ICT)for the diagnosis of MHE.Additionally,the efficacy of rifaximin and lactulose for reversal of MHE was evaluated.Methods:A total of 180 eligible cirrhotic patients underwent testing for MHE.When PHES was ≤ ?5 and ICT lures were ≥ 14,MHE was diagnosed.The 108 patients with MHE were randomized to three groups for treatment with either lactulose,rifaximin,or placebo.Treatment outcomes were measured at the end of 3 months.Results: The 108 patients with MHE diagnosed by PHES and/or ICT accounted for 60%.The diagnosis of MHE was made by both ICT and PHES positivity in 56 patients,by abnormal ICT and normal PHES in 37 patients,and by abnor-mal PHES and normal ICT in 15 patients.For diagnosis of MHE,ICT had sensitivity of 78.87%,specificity of 66.06%with 60.22%positive predictive value and 82.76%negative predictive value.An area under the curve value of 0.724(95%CI: 0.653–0.788)was obtained for diagnosis of MHE.Reversal of MHE was seen in 71.42%,70.27%and 11.11%of patients in the rifaximin,lactulose and placebo arms(p < 0.001).Rifaximin showed better tolerability compared to lactulose.Conclusions: For the diagnosis of MHE,ICT is a simple tool but has lower sensitivity and better specificity than PHES.Rifaximin is as efficacious as lactulose in the treat-ment of MHE and better tolerated.
