Objective The present study was performed to clarify the ability of ultrasonography (US) and computed tomography (CT) to detect steatosis and advanced fibrosis in nonalcoholic steatohepatitis (NASH) patients, and to assess the influence of steatosis, fibrosis, and obesity on the radiological detection of steatosis and advanced fibrosis.
Methods One hundred and eighteen biopsy proven NASH patients underwent US and CT within 6 months before or after biopsy. The ability of US and CT to detect histological steatosis and advanced fibrosis was assessed. To evaluate whether fibrosis and obesity interfered with the detection of moderate to severe histological steatosis by US and CT, we analyzed 88 NASH patients with moderate to severe steatosis. To evaluate interference with the detection of advanced fibrosis by steatosis and obesity, we analyzed 59 NASH patients with advanced fibrosis.
Results The sensitivity of US for detecting moderate to severe histological steatosis in patients with mild histological fibrosis was 100%, but this was reduced to 77.8% in patients with advanced histological fibrosis (p=0.001). The sensitivity of CT was 69.8% in patients with mild histological fibrosis and 48.9% in those with advanced histological fibrosis (p=0.047). The sensitivity of US and CT for moderate to severe histological steatosis was similar in each body mass index group. The sensitivity for detecting advanced fibrosis was markedly decreased by severe steatosis and obesity in the case of both US and CT.
Conclusion If we are aware of these disadvantages of US and CT, it is useful for diagnosing steatosis and fibrosis in NAFLD patients.

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Background It is well known that patients with liver cirrhosis often develop insulin resistance and diabetes mellitus. Recently, we encountered a liver cirrhosis patient in whom partial splenic embolization (PSE) improved insulin sensitivity. Therefore, we conducted further investigation about PSE and insulin resistance.
Methods Thirty-seven consecutive patients with liver cirrhosis underwent PSE. Hemodynamic changes, blood counts, and homeostasis model assessment of insulin resistance (HOMA-IR) were assessed before and 2 weeks after PSE.
Results PSE resulted in decreased splenic venous flow and increased intestinal venous flow to the liver. Platelet counts before and after PSE were 7.7±0.5×10⁴ /μL, 15.0±1.4×10⁴ /μL, respectively (p<0.01). HOMA-IR before and after PSE were 6.5±2.1, 3.3±0.6, respectively (p<0.05). HCV core antigen before and after PSE were 6,340±1,296 fmol/L, 4,112±873 fmol/L, respectively (p<0.05).
Conclusion PSE significantly reverses insulin resistance in patients with liver cirrhosis. The increase in intestinal venous flow to the liver and reduced HCV viral load were thought to be mechanisms of improvement in insulin sensitivity after PSE.

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Objective The purpose of this study was to analyze the CT findings of interstitial lung diseases that are associated with collagen vascular disease (CVD), with particular attention to nonspecific interstitial pneumonia (NSIP), and to examine whether it is possible to predict the clinical diagnosis of CVDs based on the CT findings alone.
Methods CT scans of 49 patients with NSIP associated with CVD (15 males, 34 females; mean age, 55±10 years; age range, 25-76 years) were included in this retrospective study. All patients underwent a surgical biopsy. The clinical diagnosis comprised rheumatoid arthritis (RA) (n=15), systemic sclerosis (SSc) (n=8), polymyositis and dermatomyositis (PM/DM) (n=18), Sjögren's syndrome (SjS) (n=4), and mixed connective tissue disease (MCTD) (n=4). Each CT was reviewed by two independent observers who made a clinical diagnosis based on the CT findings alone.
Results The observers made a correct diagnosis for 22 (45%) of the 49 patients. A correct diagnosis was made for: RA in 7 (47%) of 15 patients; SSc in 3 (38%) of 8 patients; PM/DM in 11 (61%) of 18 patients; SjS in 1 (25%) of 4 patients. None of the 4 MCTD cases was diagnosed.
Conclusion It is difficult to make a correct clinical diagnosis of the various types of CVDs based solely on CT findings. However, it is probable to make a reasonably accurate clinical diagnosis in cases that show the typical CT findings, especially for PM/DM patients.

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Objective Previous studies have reported that serum IL-18 levels are increased in some cancers. We investigated whether IL-18 production is increased in sera and cancer cells of patients with non-small cell lung cancer (NSCLC).
Patients or Materials Serum levels of IFN-γ and IL-18 and thioredoxin 1 (TRX1) were measured in 79 patients (51 males, 28 females, median age 67 years) with advanced NSCLC (57 adenocarcinoma, 22 squamous cell carcinoma; TNM stages IIIA [n=11], IIIB [n=24], and IV [n=44]) and 75 healthy age-matched controls (44 males, 31 females, median age 65 years) by enzyme-linked immunosorbent assay. We examined IL-18 production in the lungs and sites of bone metastasis of adenocarcinoma by immunohistochemistry.
Results Serum IL-18, IFN-γ, and TRX1 levels in NSCLC patients were significantly (p<0.0001, p=0.0031, and p<0.0001, respectively) higher than in control subjects, while serum IFN-γ levels in NSCLC were slightly increased. Serum IL-18, but not IFN-γ or TRX1, levels were significantly (p=0.0102) and negatively associated with overall survival in NSCLC. The serum IL-18 level was identified as an independent prognostic factor for overall survival in multivariate survival analysis. Moreover, serum IL-18 levels were significantly (p=0.049) higher in NSCLC with bone metastasis than in NSCLC without bone metastasis. Based on immunohistochemistry, we observed that cancer cells in the lungs and bone metastases markedly produced IL-18.
Conclusion Our results suggest that elevated serum IL-18 levels may be associated with IL-18 producing cancer cells in advanced NSCLC.

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Background/Aims Secondary hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) follows viral infection, malignant disorders, and autoimmune disease. Criteria for HLH diagnosis, which were proposed in 2004, include hypertriglyceridemia. However, some studies reported the absence of hypertriglyceridemia in patients with secondary HLH, differing from those with primary HLH.
Subjects and Methods In this study, we investigated the presence or absence of hypertriglyceridemia in 28 patients who were diagnosed with secondary HLH between 1997 and 2007 retrospectively. There were no patients undergoing treatment for those with a history of hyperlipidemia.
Results The subjects consisted of 14 patients with lymphoma-associated HLH, 11 with virus-associated HLH, 2 with autoimmune disease-associated HLH, and 1 with post transplantation HLH. In 19 patients (68%), hypertriglyceridemia was noted on diagnosis or during the disease period (mean: 242 mg/dL). Furthermore, the triglyceride (TG) level decreased with the treatment-related amelioration of HLH (mean level before and after treatment: 297 and 136 mg/dL, respectively, p=0.0001).
Conclusion These results suggest that the TG level is useful for diagnosing HLH and evaluating the treatment response. TG measurement is simple and inexpensive; therefore, this parameter can be determined several times to evaluate the treatment response.

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Objective It has been reported that autoimmune cerebellar ataxias, such as anti-glutamic acid decarboxylase (GAD)-antibody-positive cerebellar ataxia and gluten ataxia, are treatable. Here, we examined the therapeutic efficacy of intravenous immunoglobulin (IVIg) on autoantibody-positive cerebellar ataxia.
Patients and Methods IVIg therapy was administered in seven autoantibody-positive cerebellar ataxia patients. Therapeutic efficacy was examined in terms of its effects on clinical symptoms and changes in brain perfusion using single photon emission computed tomography (SPECT).
Results Treatment was effective in four cerebellar cortical atrophy patients (two anti-GAD antibody-positive and two anti-gliadin antibody-positive) and in one anti-thyroid antibody-positive spinocerebellar ataxia type 3 (SCA3) patient, but not in two multiple system atrophy (MSA) patients. All four IVIg effective patients who underwent SPECT showed apparent increases in cerebellar perfusion.
Conclusion If cerebellar ataxia with an autoimmune mechanism is suspected and radiological findings do not reveal MSA, it is worth considering immunotherapy including IVIg.

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Objective Bacterial biofilms cause serious problems, such as antibiotic resistance and medical device-related infections. Recent reports indicate that Bacillus species potentially form biofilms and cause nosocomial bacteremia via catheter infection. Our objective was to investigate the relationship between nosocomial bacteremia caused by Bacillus species and biofilm formations.
Methods Between 2001 and 2006, Bacillus cereus and Bacillus thuringiensis were isolated from blood samples of 21 patients with nosocomial bacteremia in two hospitals. The patients had underlying diseases such as cerebrovascular damage, malignant disease, or chronic obstructive lung disease and had high fever at the onset of bacteremia. After investigation, B. cereus and B. thuringiensis were isolated from patient's catheter tip, gauze, and hospital environment. Pulsed-field gel electrophoresis (PFGE) on 32 B. cereus and 7 B. thuringiensis isolates, microtiter biofilm assay and scanning electron microscopy (SEM) on 22 B. cereus isolates from patient's blood were performed.
Results Molecular analysis by PFGE showed that 32 B. cereus strains had 21 patterns and 7 B. thuringiensis strains had 3 patterns. The PFGE patterns of B. thuringiensis and B. cereus in blood samples from 2 patients blood were similar to those from the same patient's catheter tip. The PFGE pattern of B. cereus from a hospital environment was similar to that from 2 patients' blood samples, and the PFGE pattern of B. thuringiensis from 2 hospital environments was similar to that from 2 patients' blood. The biofilm formations by 22 B. cereus isolates from patients' blood were confirmed by microtiter biofilm assay and SEM even at 24 hours.
Conclusion Our data indicate that various types of Bacillus species exist in hospital environments and the biofilm-forming strains potentially cause nosocomial bacteremia by catheter infection.

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Background Moraxella catarrhalis, occasionally, plays the essential role in nosocomial respiratory infection (NRI). Few studies have reported the route by which this organism spreads in a nosocomial infection outbreak. We identified characteristics of the strains isolated from NRI and attempted to reveal the potential nosocomial transmission routes.
Methods A follow-up study has been performed in a Japanese community hospital between July 2002 and January 2003. M. catarrhalis clinical isolates were identified and β-lactamase production test as well as the minimal inhibitory concentrations (MICs) have been examined. Pulsed-field gel electrophoresis (PFGE) and the multi locus sequence typing method (MLST) have been introduced as the effective "fingerprinting" methods.
Results A total of 29 strains were isolated from 17 participants; 7 independent DNA fragment patterns were detected by PFGE. Pattern B (defined in this study) was dominant, and was detected both in strains from a health care worker (HCW) and inpatients. In the 9 selected strains analyzed by MLST, 7 unique MLST types were identified, which showed the congruence with the results of PFGE results.
Conclusion Epidemiological analysis proved the transmission route from patient to patient, and suggested that more studies should be focused on identifying the possible transmission route between HCWs and inpatients.

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We describe a case of diffuse large B-cell lymphoma with massive portal vein tumor thrombosis in a patient with alcoholic cirrhosis. The tumor was detected only in the intrahepatic portal vein and the spermatic cord by FDG-PET/CT. Percutaneous liver biopsy and orchiectomy were performed and histological examination revealed diffuse large B-cell lymphoma. The tumor showed complete response after six courses of the combination chemotherapy. Portal vein tumor thrombosis of malignant lymphoma is extremely rare; moreover, it is possible that this is the first case of malignant lymphoma originating from the spermatic cord producing portal vein tumor thrombosis.

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We report a very rare case of benign biliary stricture with calcification and porcelain gallbladder, causing difficulty in differential diagnosis. A 64-year-old man was referred for further examination of jaundice. Computed tomography showed calcifications in the gallbladder wall and the common bile duct. Endoscopic retrograde cholangiopancreatography revealed narrowing and a filling defect in the distal common bile duct. Peroral cholangioscopy showed a protruded lesion and stricture, and pathological examinations revealed no evidence of malignancy. The stricture was resolved after temporary insertion of progressively larger of plastic stents. Patients with benign biliary stricture and/or porcelain gallbladder should be followed carefully, because malignancy can occur as a complication, although infrequent.

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Hepatocellular carcinoma (HCC) is usually known to develop in patients with underlying high-risk liver diseases such as viral hepatitis, cirrhosis and alcohol abuse, whereas reports dealing with HCC in Crohn's disease (CD) are limited. We present a case of HCC, which developed sequentially within a short period in a 52-year-old Japanese man with a 36-year history of CD without risky conditions for HCC. He also had not taken immunosuppressants such as azathioprine. Although the definitive etiological factors contributing to hepatocarcinogenesis in the present case could not be elucidated, further close surveillance is required.

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A 73-year-old woman was admitted due to exertional dyspnea. It was considered that a large amount of pericardial effusion caused diastolic heart failure; pericardial paracentesis showed bloody effusion. There were no findings of malignancy or other abnormal findings in the examination. Further examinations were planned but she died of ventricular tachycardia attack. Pathological autopsy revealed primary systemic amyloidosis. Pathologically it was possible that the local inflammation (epicarditis) due to the deposition of amyloid in the epicardium and perivascular tissue caused the bloody effusion. There are no reports of primary systemic amyloidosis with hemorrhagic pericardial effusion. We report this rare case with pathological consideration.

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Desquamative interstitial pneumonia (DIP) is a rare pattern of diffuse parenchymal lung disease known as one of the idiopathic interstitial pneumonias and is considered to be a smoking- or dust inhalation-related interstitial pneumonia in the majority of cases. This report presents the first case of DIP in which the pulmonary manifestation preceded the onset of rheumatoid arthritis. This case and our review of twenty-four DIP cases (nineteen cases previously-reported from Japan, plus five cases in our departments) indicate the possibility that the DIP pattern is an additional form of diffuse interstitial pneumonia that may develop in association with autoimmune diseases.

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Hepatic veno-occlusive disease (VOD) is a typical complication occurring soon after allogeneic hematopoietic stem cell transplantation (HST), characterized by jaundice, painful liver enlargement, and weight gain due to fluid retention. The study reported here concerns a patient with VOD after allogeneic HST. Hemodynamic evaluation using ultrasonography revealed reversed portal venous flow before elevation of serum bilirubin, in addition to gallbladder wall thickening, ascites, and hepatomegaly. Quantitative evaluation using abdominal ultrasonography showed improvement in the reversed portal venous flow before the peaking of the serum bilirubin level and coagulopathy. This analysis was useful for both early diagnosis and clinical follow-up of VOD.

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Systemic lupus erythematosis (SLE) is a potentially fatal, autoimmune disease, which can affect different organs and can present with protean clinical manifestations. It may be associated with many other autoimmune conditions and two rare such conditions are myelofibrosis and acquired haemophilia. Autoimmune myelofibrosis is a bone marrow disorder characterized by pancytopenia, which can occur in conjunction with the presenting features, or an exacerbation of previously established SLE. Acquired haemophilia is another rare disorder of haemostasis, which can be life threatening without prompt and appropriate treatment. The management of these different conditions in itself poses a difficult problem but when the three conditions present simultaneously in the same individual, the accurate diagnosis and indeed the appropriate management becomes extremely challenging. This report describes a young woman who presented with pancytopenia secondary to myelofibrosis and panserositis with no identifiable precipitating factors. Her condition deteriorated rapidly and she required intensive care support for respiratory failure and renal impairment. A presumed diagnosis of SLE was considered and treatment was initiated which improved and stabilised her condition. However, she developed bleeding complications from acquired haemophilia which required further specialist intervention. Multidisciplinary management of the patient helped in the resolution of the complications and stabilisation of her autoimmune conditions. This report should make physicians aware of the rare presentations of SLE and its complex management.

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Acute autonomic, sensory and motor neuropathy (AASMN) is a rare peripheral nerve disorder characterized by prominent dysautonomia with somatic sensory and motor impairment. Dysautonomia in AASMN is intractable even with corticosteroid therapy or plasmapheresis. Here we report a case of AASMN with severe orthostatic hypotension. Although the effectiveness of corticosteroid was insufficient, high dose intravenous immunoglobulin therapy (IVIg) was effective for not only sensorimotor symptoms but also autonomic symptoms. This is the first case of AASMN showing favorable responses to IVIg treatment, suggesting that IVIg should be considered when corticosteroid therapy or plasmapheresis is ineffective or insufficient.

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Schönlein-Henoch purpura (SHP) is a systemic vasculitis, primarily involving the skin, gastrointestinal (GI) tract, joints, and kidneys. A wide variety of different conditions may be implicated in the pathogenesis of SHP. We report a 33-year-old man who presented with SHP accompanied by gastric Helicobacter pylori (Hp) infection. The GI manifestations and purpuric rashes were dramatically resolved after Hp eradication therapy. To date, very few publications have focused on the possible pathogenetic relationship between Hp infection and SHP.

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Haemophilus aphrophilus is one of the normal oropharyngeal flora and rarely implicated as a pathogen of spinal infection. A case of H. aphrophilus bacteremia complicated with epidural abscess, psoas muscle abscess, and spondylodiscitis is described in this report. The pathogen was mis-identified as Pasteurella spp. at the very start, and was confirmed by the molecular method. He was successfully treated with adequate antibiotics and surgery. The clinical features of sixteen previously reported cases of spinal infection caused by H. aphrophilus are reviewed.

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