We investigated the role of cell-mediated immunity in minimal change nephrotic syndrome (MCNS) by measuring interleukin 2 (IL-2) production and the responsiveness to IL-2 of peripheral blood lymphocytes (PBL). PBL from patients with MCNS, who were in the nephrotic stage prior to initiation of prednisolone (PSL) treatment or who were in remission for less than 1 yr, exhibited significantly lower levels of IL-2 production. In contrast, PBL from patients with MCNS, who were in remission for more than 1 yr or who could remit from the PSL regimen, showed normal IL-2 production. IL-2 production by CD4⁺ cells from patients with MCNS in the nephrotic stage was normal, but that by CD8⁺ cells was markedly reduced, however returned to normal when the disease was in remission. The responsiveness to exogenous IL-2 of concanavalin A-induced lymphoblasts from patients with MCNS was significantly lower, although the proportion of Tac antigen-positive cells did not differ from that of healthy volunteers. These findings suggest that defective IL-2 production and IL-2 responsiveness of PBL in patients with MCNS contribute to the pathogenesis of MCNS.

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To assess the degree of the differential ability of serum angiotensin-converting enzyme (ACE) activity as compared with 13 conventional biochemical tests, we studied 76 healthy subjects and 107 patients with chronic liver diseases. It was found that the mean values of serum ACE activity were significantly different between the healthy group and groups with liver disease. According to discriminant function analysis, the diagnostic accuracy reached 82.2% in 14 tests. In order to analyze the extent of contribution of each test to the entire diagnostic accuracy, we made an indicator of the relative decrease rate expressed as a percentage, which is 100-100 × (13-test diagnostic accuracy less one test/14-test diagnostic accuracy). The relative decrease rate of serum ACE activity was 11.4%, the largest in value. In conclusion, serum ACE activity may be one of the best discriminators to characterize chronic liver disease.

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In 45 patients with diabetes mellitus (DM) without cerebro-cardiovascular diseases (CCVD) the modified method of the tissue thromboplastin inhibition test (TTIT) was studied. TTIT is the method of detection of the lupus anticoagulant (LA). LA, first recognized in patients with systemic lupus erythematosus, is presented by a prolonged activated partial thromboplastin time (APTT), a slightly to moderately prolonged prothrombin time (PT), and high incidence of biological false-positive seroreactions for syphilis (BFP). In patients with LA, thrombotic events have been reported. Six of the 45 diabetic patients were TTIT-positive (13.3%). All control subjects were TTIT-negative. In the TTIT-positive diabetics APTT and PT were normal. BFP also were not observed. The difference between LA and these results in TTIT-positive diabetics remains unclear. Clinical profiles except for duration of DM between the TTIT-negative and TTIT-positive diabetics did not differ. Follow-up studies may resolve an association between the results of TTIT and DM.

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The goal of chemotherapy for acute myeloblastic leukemia (AML) may only be achieved by eradicating the self-renewal capacity of the blast progenitors. In this regard, chemotherapeutic drugs should be screened according to their effects against self-renewal. The present report was aimed to study the effect of the vinca alkaloid, vincristine (VCR), on the terminal divisions and self-renewal of the blast progenitors. A primary colony assay was utilized to reflect the former and a secondary colony assay and suspension culture to reflect the latter in AML patients. The results indicated that in many of the cases studied, VCR was considered to be almost equally toxic to terminal divisions and self-renewal of the blast progenitors. An almost equivalent cytotoxicity of VCR was also noted against normal hematopoietic cells, assessed by colony-forming unit granulocyte-macrophage (CFU-GM) assay. This study suggested that successful chemotherapy with VCR may be more easily achieved in combination with other drugs that are highly inhibitory to the self-renewal of the blast progenitors.

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Imipenem-cilastatin (imipenem 1 to 4 g/day) was administered to patients with severe infections accompanying granulocytopenia in hematological malignancies. Monotherapy with imipenem-cilastatin was effective in 62.1% of the patients, and also effective even they were severely neutropenic. When imipenemcilastatin was administered as the first-choice therapy, the efficacy was much better, at 73.3%. Both Grampositive and Gram-negative infections were curable by the monotherapy with imipenem-cilastatin.

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We investigated the prevalence of gallstones after gastrectomy by abdominal ultrasonography in 289 gastrectomized patients selected from 51, 621 Automated Multiphasic Health Testing and Services (AMHTS) examinees after barium meal studies. The results were compared with those of another group of 3, 193 AMHTS patients who routinely underwent ultrasonography (control). Among the gastrectomized patients, the overall prevalence of gallstones was 16.6%, and in the control group, 4.7%, showing a statistically significant difference (p<0.001). The prevalence of gallstones increased with age in both the control and gastrectomized groups, but was greater in gastrectomized subjects in each (10-yr) age group. The characteristics of the gallstones were compared between gastrectomized patients and the control group according to the ultrasonographic classification proposed by Tsuchiya (Yazawa et al, Jpn J Gastroenterol 85: 708, 1988). In the control group, the percentages of types I, II, III and small stone of Tsuchiya's classification were 30.2, 14.5, 21.9 and 34.3, respectively. On the other hand, in the gastrectomized group, the percentages were 8.3, 6.3, 16.7 and 68.8, respectively. This difference was statistically significant (p<0.001). The differences in the prevalence of gallstones between groups with Billroth I and II anastomoses, and between diseases necessitating gastrectomy, were not statistically significant.

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A 74-year-old female was diagnosed as having xanthinuria by measurement of the uric acid level in plasma, purine bases in urine and activity of xanthine oxidase in the duodenal mucosa. The determination of the urinary excretion of purine bases in her family demonstrated a slightly increased urinary excretion of oxypurines in her younger brother, suggesting that he was a heterozygote. The pyrazinamide-loading test and allopurinolloading test demonstrated that she could neither metabolize pyrazinoic acid into 5-hydroxypyrazinoic acid nor allopurinol into oxypurinol, although there was a slight metabolizing of prazinamide into 5-hydroxypyrazinamide. This suggested that she belonged to the subgroup which can neither metabolize pyrazinamide into 5-hydroxypyrazinamide, pyrazinoic acid into 5-hydroxypyrazinoic acid nor allopurinol into oxypurinol.

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We report a case of a 61-year-old male without any predisposing factors. His X-ray film showed multiple nodular lesions in bilateral lung fields. Open lung biopsy revealed Zygomycetes in the granuloma. The patient was treated with amphotericin B and miconazole, and remains alive more than 32 months later.

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A 64-year-old man developed non-Hodgkin's lymphoma of the chest wall, 37 yr after left plombage thoracoplasty. Late complications of plombage thoracoplasty are discussed as well as the pathogenesis of chest wall neoplasms.

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A young male patient with myositis associated with Crohn's disease is reported. His serum creatine phospliokinase (CPK) level was markedly elevated, but he had no muscle symptoms. The serum CPK level was not correlated with the activity of Crohn's disease. Muscle biopsy showed myositis with only degeneration of the muscle fibers and infiltration of inflammatory cells. The etiology of myositis in this case was not clear. Diagnosis of myositis based on a muscle biopsy in patients with Crohn's disease showing an elevated serum CPK level without any discernible cause has not been reported previously. Careful attention to the serum CPK and muscle symptoms in patients with Crohn's disease is suggested.

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A patient with orthostatic hypertension of neurogenic origin is reported. The Schellong and head-up tilt tests produced a sudden moderate to marked increase of the blood pressure, but the plasma renin activity and plasma aldosterone level showed a normal response. The cold pressor test also produced a moderate to marked increase of the blood pressure. Pharmacological cardiovascular autonomic nervous function tests showed a decrease of parasympathetic tone and denervation hypersensitivity of the sympathetic nerve terminals. These findings suggest that the orthostatic hypertension in this patient was mainly due to sympathetic denervation hypersensitivity.

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Two cases of Sjögren's syndrome accompanied by peripheral neuropathy are reported. The level of antiendothelial cell antibody was increased in both patients. Immunofluorescent deposits of immunoglobulin and C3 component were detected in the vasa nervorum of both cases. The pathological findings showed damage to the endothelial cells in the same vessels. The findings suggest that injury from immune complex and antiendothelial cell antibody may be the immunological factor in the induction of peripheral neuropathy.

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The patient, a 51-year-old man, was receiving immunosuppresants for 2 yr after renal allotransplantation. He had heart failure with aortic regurgitation, fever, anemia and a history of odontectomy on admission. He was resistant to medical treatments and died from cerebral emboli. On autopsy, vegetation of the aortic valve was identified. Progression of atherosclerosis, which may have been due to steroids and chronic rejection, was prominent. This report is the first case of infective endocarditis following organ transplantation in Japan. Such complications as infective endocarditis and atherosclerosis will be on the rise with the increase of numbers of organ transplantations.

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We report a case of hypocalcemic heart failure without underlying myocardial disease. Two-dimensional and Doppler echocardiography revealed dilatation and impaired contraction of the left ventricle, but did not show any valvular dysfunction. Cardiac catheterization showed a normal coronary artery, and cardiac muscle biopsy showed morphological changes in mitochondoria and endoplasmic reticulum, which may be due to metabolic changes. This patient was asymptomatic after the serum calcium concentration was normalized.

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A 50-year-old man developed myoglobinuric renal failure after taking pyrazinamide. Both serum and urine myoglobin levels were elevated and tubulo-interstitial nephropathy was demonstrated on renal biopsy. After pyrazinamide was discontinued, the myoglobin concentrations were normalized and his renal function ameliorated. The rhabdomyolysis was considered to be caused by pyrazinamide. To our knowledge, this is the first reported case of rhabdomyolysis associated with pyrazinamide.

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We report a fatal case of alcoholic hepatitis with hyperleukocytosis mainly consisting of mature granulocytes in a 43-year-old woman. White blood cell count was increased in parallel with clinical deterioration to 54, 800/mm3 with no immature neutrophils on a differential count. The bone marrow aspirate revealed normal maturation and no evidence of hematological malignancy. It has been postulated that severe leukocytosis accompanied by alcoholic hepatitis may be provoked by release of high levels of colony stimulating factor from damaged hepatic cells. However, the present patient showed a normal level of serum granulocyte colony stimulating factor, and could not prove the above assumption.

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A 71-year-old woman was clinically suspected of allergic granulomatous angiitis (AGA) because of preceding allergic diseases including bronchial asthma, remarkable eosinophilia (14, 300/mm³), mononeuritis multiplex, positive rheumatoid factor, elevated serum immunoglobulin E, and eosinophilic inflammation of the kidney. Autopsy findings, however, were characteristic of polyarteritis nodosa (PAN). Necrotizing angiitis was present in several organs except for the lung; focal and segmental glomerular lesions with crescent formation were observed in the kidney, and granuloma formation was not found. This case may be an intermediate type between PAN and AGA (an overlap syndrome) and provide useful information on the clinical entities of systemic necrotizing vasculitis.

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We describe a case of multiple myeloma associated with myelofibrosis. This patient had hepatosplenomegaly, moderate anemia with anisocytosis and nucleated red blood cells, and Bence-Jones protein (κ) in the urine. A bone marrow biopsy showed extensive marrow fibrosis and proliferation of numerous immature plasma cells containing κ light chain in the cytoplasm. Melphalan-prednisolone therapy not only facilitated the disappearance of the immature plasma cells but also resulted in an improvement of myelofibrosis in the bone marrow. The immature plasma cell proliferation and marrow fibrosis in the bone marrow were seen again after interruption of chemotherapy. Therefore, this myelofibrosis may be secondary to the coexistent multiple myeloma.

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A case of cellular schwannoma originating from the retroperitoneum is reported. The histological features of the tumor resembled those of a smooth muscle tumor. However, the tumor cells were positive for S-100 protein, which is almost exclusively identified within nerve sheath tumor cells. This finding confirmed the diagnosis of cellular schwannoma. This type of tumor is generally considered to be benign but is sometimes mistaken for a malignant tumor. These findings indicate that it is important to prove whether or not S-100 protein is present in the cells of a tumor of unknown origin especially arising in the mediastinum or retroperitoneum.

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