An inferior mesenteric-caval shunt via the internal iliac vein with portosystemic encephalopathy.

We report here a case of an unusual extrahepatic portosystemic venous shunt in a 37-year-old woman without liver cirrhosis or portal hypertension, who developed portal systemic encephalopathy. Angiography demonstrated an inferior mesenteric-caval shunt characterized by the presence of direct communication of the inferior mesenteric vein with the left internal iliac vein. After the treatment with percutaneous transcatheter embolization of the shunt via a femoral vein approach using coils, she had no episode of portal systemic encephalopathy.

Successful interferon therapy reverses enhanced hepatic iron accumulation and lipid peroxidation in chronic hepatitis C.

OBJECTIVES: Hepatic iron deposition has been reported in chronic hepatitis C (CH-C), and iron-induced lipid peroxidation may be involved in the pathogenesis of CH-C. The aims of the present study were: 1) to determine whether patients with CH-C have evidence of enhanced hepatic lipid peroxidation and to evaluate its relation to iron status, compared with that in patients with chronic hepatitis B (CH-B); and 2) to assess the effect of interferon (IFN) therapy on hepatic iron and lipid peroxidation. METHODS: In the liver biopsies of 40 patients with CH-C and 26 patients with CH-B, immunohistochemical detection of 4-hydroxy-2-nonenal (HNE)-protein adducts for evaluation of lipid peroxidation was performed, and hepatic iron status was biochemically and histologically assessed. In 16 CH-C patients with normal serum transaminases and undetectable serum HCV-RNA >6 months after the end of IFN treatment (responders) and in 11 nonresponders, hepatic HNE-protein adducts and siderosis were evaluated in pre- and posttreatment liver biopsies. RESULTS: Hepatocytic HNE-protein adducts and iron deposits were more abundant in the patients with CH-C than in those with CH-B. No correlation was found between the levels of hepatocytic HNE-protein adducts and hepatic iron status in either of the two groups. In the responders to IFN treatment for CH-C, hepatocytic HNE-protein adducts disappeared or attenuated with improvement of hepatic siderosis after the treatment, whereas IFN treatment did not improve hepatocytic expression of HNE-protein adducts and hepatic siderosis in the nonresponders. CONCLUSIONS: Patients with CH-C have evidence of enhanced hepatic iron accumulation and lipid peroxidation compared to those with CH-B. In CH-C, hepatic siderosis and lipid peroxidation are improved with successful IFN treatment. These results suggest that hepatic lipid peroxidation and iron may potentially play contributory roles in the pathogenesis of CH-C.

Enhanced hepatic lipid peroxidation in patients with primary biliary cirrhosis.

OBJECTIVE: The mechanisms responsible for hepatic injury have not been fully clarified in primary biliary cirrhosis (PBC). It has recently been suggested that hepatic lipid peroxidation may be involved in the pathogenesis of PBC. The aims of the current study were to determine whether patients with PBC have evidence of enhanced hepatic lipid peroxidation and to evaluate its relationship to clinicopathological features. METHODS: Immunohistochemical detection of 4-hydroxynonenal (HNE) protein adducts was performed in the liver biopsies of 20 patients with PBC. Histological stages of PBC were evaluated. Orcein or Victoria blue staining was performed for detection of copper-associated proteins. The size of bile ducts was defined as the smallest diameter between the subepithelial basement membranes. RESULTS: All 20 patients had immunodetectable HNE protein adducts in the cytoplasm of damaged, but also intact, biliary cells. The mean diameter of bile ducts with HNE protein adducts was smaller than those without the adducts (61.0 +/- 1.9 vs 122.5 +/- 24.4 microm, respectively, p < 0.01). Out of 20 patients, 6 (30%) also had immunodetectable HNE protein adducts in hepatocytes preferentially located around the portal tracts. Most of the patients with hepatocytic HNE protein adducts had copper-associated protein granules in hepatocytes around the portal tracts and were classified as histological stage 3, whereas all of the patients without the adducts lacked copper-associated protein granules and were classified as histological stage 1 or 2. The patients with hepatocytic HNE protein adducts had higher levels of serum total bilirubin than did those without the adducts (2.9 +/- 0.9 vs 0.7 +/- 0.1 mg/dl, respectively, p < 0.01). CONCLUSIONS: Hepatic lipid peroxidation can occur in PBC and may be an early event in bile duct destruction. At advanced stages of PBC, hepatocellular lipid peroxidation may play a role in hepatocyte injury during cholestasis.

Serum thrombopoietin levels in patients with chronic hepatitis and liver cirrhosis.

OBJECTIVE: Thrombocytopenia is a common manifestation of cirrhosis. The aim of this study was to examine the relationship between serum thrombopoietin concentrations, circulating platelet levels, and the stage of hepatic fibrosis in patients with chronic viral hepatitis. METHODS: The study included 48 patients with chronic viral hepatitis (14 with stage 1 fibrosis; five with stage 2 fibrosis; three with stage 3 fibrosis; 26 with cirrhosis) and 30 healthy volunteers. Serum thrombopoietin levels were measured using an enzyme-linked immunosorbent assay. Spleen size, platelet counts, and prothrombin time were measured. RESULTS: Thrombopoietin levels of patients with fibrosis stage 1 (2.50 +/- 1.60 fmol/ml) or stage 2 (1.89 +/- 0.65) were significantly higher than those in patients with cirrhosis (1.21 +/- 0.55) or healthy volunteers (1.26 +/- 0.74). Mean platelet counts of patients with cirrhosis (8.0 +/- 4.6 x 10(4)/microl) were significantly lower than those with fibrosis stage 1 (18.6 +/- 3.9) or stage 2 (16.0 +/- 5.8), or healthy volunteers (24.5 +/- 7.3). Patients with cirrhosis had larger spleens (30.9 +/- 18.4 cm2) than those with fibrosis stage 1 (18.2 +/- 6.4). Platelet counts showed a significant inverse relationship to spleen size (p = -0.51, p < 0.0005) and a significant positive relationship with thrombopoietin levels (p = 0.34, p < 0.02). Thrombopoietin levels were significantly correlated to prothrombin time (p = 0.45, p < 0.005). CONCLUSIONS: Serum thrombopoietin levels are elevated in patients with an early stage of chronic viral hepatitis. As the disease progresses from mild fibrosis to cirrhosis, decreased production of thrombopoietin may contribute to the further development of thrombocytopenia in cirrhosis.

An unusual hyperplastic hepatocellular nodule in a patient with hepatitis C virus-related liver cirrhosis.

Recent advances in diagnostic imaging techniques have increased the likelihood of detecting novel nodular lesions of the liver. We report here a case of unusual hyperplastic hepatocellular tumor found in a 70-yr-old woman with hepatitis C virus-related cirrhosis. A mass was incidentally detected in the right lobe by abdominal ultrasonography and confirmed by computed axial tomography (CT). Magnetic resonance imaging demonstrated that the tumor had hyperintense signal with a small hypointense region in the center and a thin, hypointense rim on T1-weighted image and a hypointense signal on T2-weighted image. CT during hepatic arteriography showed that the tumor was hypodense with a central hyperdense region, whereas CT during arterial portography revealed that the tumor was isodense and surrounded by a thin circular hypodense band with a central hypodense region. These radiographic findings suggested a diagnosis of dysplastic nodule with malignant foci of hepatocellular carcinoma. The patient underwent tumor resection. Macroscopically, the tumor, 45 x 45 x 30 mm in size, was encapsulated and had a central stellate-like scar with radiating septa. Histological examination showed a hyperplastic hepatocellular tumor without cellular, nuclear or structural atypia. The central fibrous scar contained abundant small, artery-like and vein-like vessels, whereas there were no normal portal triads but rather several portal tract-like structures lacking bile ducts in the parenchyma of the tumor. Some of the portal tract-like structures were composed of artery-like and vein-like vessels, and the others possessed vein-like vessels only. There were no bile ducts in the tumor. The nontumorous liver tissue had evidence of macronodular cirrhosis. Finally, this tumor was regarded as an unusual type of hyperplastic hepatocellular nodule encountered in cirrhotic liver, characterized by the presence of central stellate-like fibrosis and the lack of bile ducts. Although the pathogenesis of the hyperplastic lesion is unclear, it may represent a focal regenerative hepatocellular response to localized circulatory disorder.

Failure to respond to interferon-alpha 2a therapy is associated with increased hepatic iron levels in patients with chronic hepatitis C.

Recent reports suggest the hepatic iron concentration (HIC) may influence the activity of hepatitis and the response to interferon (IFN) therapy in patients with chronic hepatitis C (CH-C). We have evaluated iron status in 28 patients with CH-C and determined if pretreatment iron status can predict the response to IFN-alpha therapy in these patients. Increased serum iron, transferrin saturation, and ferritin levels were observed in 3 (11%), 11 (39%), and 5 (18%) patients, respectively. Hepatic iron deposits were histologically detected in 17 (61%) patients, and 14 of them had stainable hepatocytic iron. However, all HIC values were within the normal range (203-1279 microg/g). Seven of 17 patients treated with IFN-alpha for 6 mo had normalization of serum transaminases and disappearance of serum HCV-RNA (responders). Nonresponders had a significantly higher median HIC compared with responders (710 vs 343 microg/g, respectively; p < 0.05). There was no significant difference in other pretreatment iron parameters, serum HCV-RNA level, or HCV-genotype between responders and nonresponders. In conclusion, mild hepatic iron accumulation occurs in patients with CH-C. Increased hepatic iron stores are associated with poor response to IFN therapy. Pretreatment HIC may be an additional host-specific parameter with a predictive value for responsiveness to IFN therapy, in addition to well-known predictive viral factors.

[A case of hypertrophic obstructive cardiomyopathy with marked T wave changes during the short-term].

A 72-years-old woman was admitted to our hospital for evaluation of giant negative T waves, which appeared for only two days. Chest X-p revealed a cardiomegaly of slight degree and UCG showed ASH (IVS = 21 mm). Coronary arteriography presented no significant stenosis and the left ventricle was spade-shaped. There was a pressure gradient of 65 mmHg between the aorta and the left ventricle during isoproterenol infusion. Furthermore, endomyocardial biopsy showed disarray and fibrosis to a slight degree and fatty degeneration of myocytes with contraction bands. Based on these findings, calcium blocker was administrated under the diagnosis of HOCM. One month after the initiation of this drug, negative T waves gradually became shallow and finally upright with thinning of IVS (12 mm) four month later. We swimise that this T-wave change is primarily based on myocardial hypertrophy as well as being due to the abnormality of myocardial depolarization. We presented a case of HOCM with negative T-wave change of very short duration, which was improved by calcium-blocker and beta-blocker.

[A case of myxedema heart with serial endomyocardial biopsy].

We report a case of middle-aged woman with myxedema heart who presented congestive heart failure due to myocardial damage caused by myxedema, and showed reversible and irreversible myocardial change proven by serial endomyocardial biopsy. She was admitted to our hospital because of facial, peripheral edema and dyspnea. On admission, chest X-P revealed severe cardiomegaly (CTR 70%) and bilateral pleural effusion. Electrocardiogram showed low voltage. Echocardiogram and chest CT revealed a large amount of pericardial effusion. No significant stenosis was observed on a coronary arteriogram. Laboratory data showed elevated TC, CPK, LDH values and immunological test indicated high titer of thyroid test and microsome test. Thyroid function test revealed primary hypothyroidism with low T3, low T4 and high TSH levels. Replacement therapy for hypothyroidism in addition to digitalis and diuretics++ has been started. Seven months after initial evaluation, this therapy has resulted in dramatic clinical improvement. Transvenous right ventricular endomyocardial biopsy demonstrated vacuolated degeneration on admission and improvement of vacuolated degeneration with a slight degree of fibrosis after therapy. This pathological finding suggests that myxedema heart is able to produce both reversible and irreversible myocardial damage.