The discovery of hepatitis C virus (HCV) has enabled the diagnosis of type C chronic liver disease, which had in the past been diagnosed as part of non-A, non-B chronic liver disease. Although most cases with chronic liver disease are caused by hepatitis B virus (HBV) or HCV infection, there are still cases of non-B, non-C chronic liver disease. Forty patients with chronic liver disease, who were seronegative for hepatitis B surface antigen and antibody to HCV, were followed for a mean period of 72 months. The clinical manifestations in these patients were compared with those reported for type B and type C chronic liver disease. Of the 40 patients, 22 were diagnosed with chronic hepatitis, 14 with liver cirrhosis and 4 with hepatocellular carcinoma (HCC). Twenty-seven (67.5%) patients showed mild alanine aminotransferase activity profiles, and the natural clinical course of most patients showed a slow progression compared with that reported for type B and type C patients. The yearly incidence of HCC was 9.7% in patients with liver cirrhosis and 3.9% in chronic hepatitis. These rates were similar to those in type B or type C patients. This suggests that our population sample contained a number of patients with type B, type C or other etiologic agent(s), because 66.7% of the patients who developed HCC had some evidence of exposure to HBV or HCV. Our results suggest that more detailed and accurate tests for detecting HBV and HCV should be considered before making the diagnosis of non-B, non-C chronic liver disease, and that there is the need to reveal unknown etiologic agent(s).
(Internal Medicine 35: 600-604, 1996)

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To determine whether or not myocardial fibrosis in the left ventricle increases after exposure to asbestos fiber, we studied myocardial fibrosis in 42 autopsied hearts: 17 of asbestosis (AS) and 18 of chronic pulmonary disease (CP) without pneumoconiosis. Seven patients with extra-cardiopulmonary disease were used as a control group (CONT). The extent of myocardial fibrosis in the left ventricle was significantly greater in the AS group compared to the other groups (AS 3.6±1.3% vs. CP 2.4±1.0% vs. CONT 1.6±0.8%). There was a significant difference in the appearance ratio of focal fibrosis between the asbestosis groim and the other groups. Focal fibrosis was observed in none of 7 control patients, in 3 of 18 patients with chronic pulmonary disease and in 9 of 17 patients with asbestosis. We concluded that characteristic myocardial fibrosis was observed in humans after long-term asbestos exposure.
(Internal Medicine 35: 605-610, 1996)

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The purpose of the study was to assess the quality of life (QOL) and anxiety in 50 inpatients with primary lung cancer and examine the influence of their personalities on the QOL assessment. We used a psychological personality test to evaluate the patient's personality, then followed the course of QOL and anxiety before and after chemotherapy. To measure QOL, we used Holmes's QOL checklist, and the State-Trait Anxiety Inventory was adopted to estimate the patient's anxiety. Eighty courses of chemotherapy were administered, and QOL evaluations were performed 235 times. By using factor analysis, the somatic, social and psychological factors were extracted which confirmed the reliability and validity of the QOL checklist. The psychological QOL score showed a correlation with A (Adult) and AC (Adapted Child) of the five ego states in the Egogram. Therefore, it is important to survey the patients' personalities in order to grasp their QOL accurately.
(Internal Medicine 35: 611-616, 1996)

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In immotile cilia syndrome (ICS), respiratory symptoms show a wide range of severity. Various types of axonemal abnormalities related to ICS are known, but it is unclear whether the symptom severity is related to the type of abnormality. We sent a questionnaire concerning respiratory symptoms to ICS patients. Another type of the questionnaire regarding the clinical features and axonemal abnormality of ICS was sent to the physicians in charge. Analysis of the results showed that in the 48 patients responding, those lacking outer dynein arms (LO) in cilia were more likely to be associated with situs inversus (SI) than those lacking inner dynein arms (LI). Sputum symptoms were more serious in those associated with SI than those unassociated with SI, as was the case in those associated with LO compared to those with LI. Based on these results, it may be concluded that the type of dynein arm defect in cilia is related to the clinical features in ICS.
(Internal Medicine 35: 617-623, 1996)

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The association of human T-lymphotropic virus type I (HTLV-I) with autoimmune disorders was investigated on the basis of prevalence of antinuclear antibody (ANA), rheumatoid factor and anti-thyroglobulin antibody as well as immunoglobulin (Ig) serum level (IgG, IgA, and IgM). The subjects, all atomic bomb survivors, were 59 HTLV-I-seropositive people without HTLV-I-associated myelopathy or adult T-cell leukemia and 149 HTLV-I-seronegative persons. The mean serum level of IgM was higher in HTLV-I-seropositive subjects than in HTLV-I-seronegative subjects, and a significant association with HTLV-I and sex was indicated in the IgM serum level. No association with HTLV-I was indicated in the prevalence of the autoantibodies except for ANA. These results suggest some clear humoral immunity differences between HTLV-I-infected and noninfected subjects, but whether HTLV-I infection can lead to autoimmune disorders remains uncertain.
(Internal Medicine 35: 624-628, 1996)

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To identify the risk factors for infection and to develop a model for the prediction of infection in hospitalized patients with active systemic lupus erythematosus (SLE), we carried out a retrospective cohort study with clinical data collected from 121 consecutive patients with active SLE. Seventeen patients had infection within 6 months of beginning steroid therapy for active SLE. Independent multivariate predictors of infection were a decrease in the serum albumin value, an increase in the serum creatinine value, and prednisolone use in a dose of ≥60 mg/day without methylprednisolone pulse therapy. The error rate of the model by 10-fold cross-validation method was 12%, sensitivity was 65%, specificity was 91%, and positive predictive value was 55%. Four nonsurvivors were correctly discriminated. Use of this model could contribute to earlier diagnosis of infection and may assist decisions regarding empiric antimicrobial administration in patients with SLE.
(Internal Medicine 35: 629-636, 1996)

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Electrocardiographic abnormalities were pointed out in a 51-year-old Japanese male whose major complaint was dizziness. His electrocardiogram showed a complete right bundle branch block, and a prolonged His bundle-ventricle (HV) interval of 100 msec. Two members of his family died of heart disease and 3 members, including a case of sudden death, presented an abnormal electrocardiogram of the Brugada-type with persistent ST segment elevation in the right precordial leads and right bundle branch block. The signal-averaged examination was made in the children of cases that died with the diagnosis of sudden death. Four cases showed a tendency of delay in the HV interval and a positive finding in the late potential. Further studies are necessary to clarify the relationship between electrocardiographic abnormalities of the Brugada-type and atrioventricular conduction disorder as well as to clarify the genetic basis of this disorder.
(Internal Medicine 35: 637-640, 1996)

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Aceruloplasminemia is a systemic degenerative disorder characterized by mutations in the ceruloplasmin gene, the absence of serum ceruloplasmin, and iron accumulation in the brain, liver, and other tissues. Iron is an important catalyst of oxyradical-mediated cellular and tissue injury, and (β-cells in the pancreatic islets are susceptible to the cytotoxic effects of oxidative stress. We report three patients with aceruloplasminemia who have late-onset diabetes mellitus (DM) and impaired glucose tolerance (IGT) as well as neurologic symptoms. Their basal lipid peroxide levels, measured as thiobarbituric acid-reactive products, in plasma samples were three times the values for the controls. This increased susceptibility to lipid peroxidation in patients with aceruloplasminemia suggests that free-radical-mediated tissue injury plays a role in the occurrence of DM and IGT.
(Internal Medicine 35: 641-645, 1996)

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A 59-year-old man visited Kyoto University Hospital because of general malaise, polyuria, and polydipsia. The diagnosis of primary hyperparathyroidism was made based on hypercalcemia and an elevated circulating PTH level. A nodule was palpable in the left anterior neck. Two weeks later, the serum calcium level was normalized and his symptoms subsided. A temporary expansion, followed by reduction of the tumor size was observed by serial ultrasonography. Histology of the resected tumor showed central necrotic tissue, with some peripherally remaining glandular tissue. We report here a rare case of primary hyperparathyroidism with spontaneous remission due to hemorrhagic infarction in the adenoma.
(Internal Medicine 35: 646-649, 1996)

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This is the first case report of exacerbation of tracheobronchitis due to nontoxigenic corynebacterium diphtheriae in which tracheal pseudomembrane was identified and oral erythromycine therapy was very successful.
(Internal Medicine 35: 650-652, 1996)

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A case of diffuse alveolar hemorrhage due to lung metastasis of ovarian angiosarcoma is described. A 33-year-old woman developed persistent cough and recurrent hemoptyses with resultant anemia. Chest radiograph and computerized tomography (CT) showed diffuse and patchy shadows in both lungs with nodules in the central area of the shadows. In spite of intensive care and administration of large amounts of glucocorticoids, she died of respiratory failure 5 months after the onset of her symptoms. Histological examination by both thoracoscopic biopsy and autopsy revealed diffuse tumor emboli of disseminated angiosarcoma which originated from the right ovary and diffuse alveolar hemorrhage which appeared to be caused by the tumor emboli with resultant invasion and destruction to vascular walls. This is the first description of diffuse alveolar hemorrhage probably caused by tumor emboli of metastatic angiosarcoma.
(Internal Medicine 35: 653-656, 1996)

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We report two cases of empyema as a complication of the percutaneous instillation of antifungal drugs for pulmonary and pleural aspergillosis. Case 1 underwent percutaneous administration of amphotericin B and fluconazole for 2 months. Six months later, the patient was found to have an Aspergillus empyema with a bronchopleural fistula. Case 2 with pulmonary and pleural aspergillosis underwent percutaneous administration of amphotericin B for one month. Four months later, the patient underwent pleural drainage due to empyema. Pleural biopsy revealed pleural aspergillosis. In both cases, it was suggested that the preceding Aspergillus infection and percutaneous instillation of antifungals resulted in the development of empyema.
(Internal Medicine 35: 657-659, 1996)

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A 42-year-old woman with Crow-Fukase syndrome developed acute myeloid leukemia (M6: FAB classification) following treatment with alkylating agents (a total of 2, 500 mg of melphalan and 9, 800 mg of cyclophosphamide). Chromosome analysis of the bone marrow showed 49, XX, der(1;7)(q10;p10), +8, +19, +21 in therapy-related myelodysplastic syndrome with additional chromosomes 8, and 12 and two additional chromosomes 21 in acute leukemia. Because of the risk of therapy-related leukemia, alky lating agents should be used with caution in the treatment of Crow-Fukase syndrome.
(Internal Medicine 35: 660-662, 1996)

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The efficacy of polyvalent intravenous immunoglobulin therapy (IVIG) was evaluated in three patients with refractory myositis. Patients refractory to conventional therapy, such as corticosteroid administration, corticosteroid or cyclophosphamide pulse therapy [2 with dermatomyositis (DM), 1 with polymyositis (PM)] were treated with IVIG (0.4 g/kg daily) for 5 days. Clinical improvement was apparent within 1-2 months after IVIG and persisted for 19-23 months (DM patients) and 12 months (PM patient). No adverse effects were observed. Thus, IVIG may be considered an effective therapy for refractory myositis.
(Internal Medicine 35: 663-667, 1996)

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A 24-year-old man with livedo racemosa and psychiatric disturbances, manifesting as low intelligence (IQ 80) and delusions, had anti-cardiolipin antibody and showed shortening of the fingers and toes. A skin biopsy of the livedo lesion revealed endoarteritis obliterans, being compatible with Sneddon's syndrome. MRI of the brain demonstrated multiple infarction and moderate cortical atrophy. A single photon emission tomography of the brain showed a marked reduction of the blood flow in the front-temporal lobe. These findings might relate to the psychiatric disturbance. After intravenous administration of cyclophosphamide and the start of oral prednisolone, the anti-cardiolipin antibody level decreased and his physical condition improved. However, a low dose of haloperidol is still necessary to maintain his mental condition.
(Internal Medicine 35: 668-673, 1996)

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