A factor which inhibits protein kinase C has been detected in normal lymphocytes and leukemia cells obtained from some untreated patients with lymphoblastic leukemia. The partially purified inhibitory factor obtained from normal human lymphocytes depressed the growth of K562 cells and HL-60 cells, while it also suppressed DNA synthesis of these cells in a dose-dependent manner. The inhibitory factor was not able to suppress the DNA synthesis, however when it was added to the medium 12 hours after the culture. The inhibitory factor also reduced the phosphorylation of endogenous proteins, mainly Mr 80, 000, 40, 000 and 38, 000. These results suggest that the inhibitory factor is closely related to the cell proliferation of various cells through the suppression of protein kinase C activity.

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Nine patients with hypergammaglobulinemic purpura associated with primary Sjögren's syndrome were presented. All patients were female (mean age 45.6) and showed recurrent purpura on the lower extremities and typical findings of Sjögren's syndrome with high gammaglobulin and IgG levels, rheumatoid factors, anti-SSA/SSB antibodies (5/5) and anti-nuclear antibodies (6/9). Vasculitis was seen in 6 patients, 4 were of the mononuclear cell type and 2 of the neutrophilic cell type. Six patients had intermediate complexes between 7S and 19S shown by serum ultracentrifugation. Immunofluorescent staining showed immunoglobulin deposition along the blood vessel walls of the skin in these patients. These data suggest that hypergammaglobulinemic purpura in Sjögren's syndrome is vasculitis caused by immunologic processes.

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Levels of serum total cholesterol (t-CHO), total triglycerides (TG), high density lipoprotein cholesterol (HDL), low density lipoprotein cholesterol (LDL), very low density lipoprotein cholesterol (VLDL), and chylomicrons (CHY) were monitored in 25 patients with cerebral infarction. Measurements were made regularly on day 1, day 7, at 1 month and 3 months. Serum t- CHO and TG levels following cerebral infarction was demonstrated to significantly fall for duration of 3 months. The decline of LDL and CHY continued for 3 months but was not statistically significant. HDL varied little. The increase of VLDL on day 7 and its fall at 1 month and 3 months were noted but was not statistically significant. Possible mechanism of the significant fall in serum t-CHO and TG was discussed.

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Immunofluorescent analysis of viral antigens in the cultured fibroblasts and renal tissues in patients with IgA nephropathy was described. Freeze and thawed extracts of pharyngeal cells obtained from patients with IgA nephropathy, chronic proliferative glomerulonephritis without IgA deposition (PGN) and healthy adults were cultured with human fibroblasts, i.e. Hel cells, with or without addition of 5-iodine 2'-deoxy-uridine (IUDR) at 37°C for 2 weeks. These fibroblasts and renal sections were stained with several kinds of FITC-labeled antiviral antibodies. Deposition of adeno, herpes simplex, varicella zoster or parainfluenza 3 was observed not only in the renal sections but also in the nuclear regions and/or cytoplasm of Hel cells after incubation of extracts of pharyngeal cells with or without IUDR from patients with IgA nephropathy. It is indicated that antigenic stimulation in the upper respiratory tracts may be due to several different types of DNA and/or RNA viruses in patients with IgA nephropathy. It appears that these antigenic substances show some heterogeneity among these patients.

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The present study was designed to assess if the release of Na-K ATPase inhibitor (NKAI) after salt-loading is related to sympathetic nervous system in essential hypertension. Thirteen patients with essential hypertension were studied during low-salt diet (2 g/day) and then high-salt diet (20 g/day) for each 7-days period. Mean blood pressure (MBP), body weight, Na:K ratio in red blood cell (R-Na/K), plasma norepinephrine (PNE) and NKAI were measured on the last day of each period. NKAI was determined by the degree of the inhibition of dog kidney Na-K ATPase activity by deproteinized plasma. Patients were classified as "salt-sensitive" (SS) or "nonsalt-sensitive" (NSS) group by the MBP responses to salt-loading. With the high-salt diet, SS group showed more body weight gain, greater increase in R-Na/K and NKAI (SS; 8.0±1.0%, NSS; 2.4±0.8%, P<0.01) than NSS group. PNE showed no changes in SS group and decreased in NSS group (P<0.05). The increase of MBP was highly correlated with the rise of NKAI (r=0.76, P<0.01). Moreover, the increase of NKAI was also significantly correlated with the changes of PNE (r=0.62, P<0.05). These results suggest that the greater increase in blood pressure by salt-loading in SS patients can be attributed to the increase of NKAI, which might regulate the sympathetic nervous system as a result of increased intracellular sodium.

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We studied total SDS-solubilized muscle proteins (TMP) of Duchenne muscular dystrophy (DMD) and other neuromuscular disorders, with special attention to the change of dystrophin suspected of being the product of DMD locus. SDS gel electrophoresis of DMD patients showed an absence of band 5 and an extreme faintness of band 2 with a decrease of band 4', 5', and 5". Immuno blot analysis, using anti-dystrophin antibodies (anti-30 kd and anti-60 kd polyclonals), showed an absence of dystrophin in all 6 DMD cases. In other neuromuscular disorders, there was no change of TMP, and dystrophin was clearly detectable. To elucidate the degenerative mechanism of DMD muscle, further studies, including the problem of clarifying the physiological role of dystrophin, are necessary.

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The effect of amogastrin on the release of 5 types of prostaglandins (PGs) [PGE₂, 6-keto-PGF_1α, PGF_2α, PGD₂, and thromboxan B₂ (TXB₂)] into the gastric lumen was evaluated in seven healthy volunteers. Gastric juice was obtained under basal and amogastrin-stimulated conditions. The 5 types of PGs in the gastric juice were separated by high-performance liquid chromatography and then measured by radioimmunoassay. PGE₂ was the major component in gastric juices among the PGs measured in this study. The release of 5 types of PGs increased after amogastrin administration. The rate of increase was high for PGE₂ and 6-keto-PGF_1α (3.48 and 3.98 respectively), whereas it was low for PGD₂ (1.54). It is suggested that endogenously synthesized PGs, especially PGE₂ and PGI₂ (assayed as 6-keto-PGF_1α), play important roles in the gastric mucosal protection and the regulation of acid secretion.

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The prognostic values of indices obtained by M-mode and two-dimensional echocardiography (Echo) and by cardiac catheterization (Cathe) were assessed in patients with idiopathic dilated cardiomyopathy. Fifty-one patients with this disorder (38 males and 13 females) were studied for an average of 4.2 years. Of those, 24 died of cardiac causes during follow-up. The overall 5-year survival rate was 49%. Significant differences between the surviving patients and the patients who died were noted in the following indices measured by Cathe: left ventricular end-diastolic volume (LVEDV), left ventricular end-diastolic pressure (LVEDP), ejection fraction (EF), and the cardiac index (CI). The 5-year survival rate in patients with the following index values measured by Cathe at the initial examination were: LVEDV ≥ 150 ml/M² - 29%; LVEDP > 12 mmHg - 35%, EF < 30% - 21%, and CI < 3.0 L/min/M² - 29%. In contrast, the 5-year survival rate in patients with the following parameter values measured by Echo were: left ventricular end-diastolic dimension ≥ 45 mm/M² - 16%; left atrial dimension ≥ 25 mm/M² - 12%; ejection fraction < 30% - 31%; and a relative wall thickness (a ratio of left ventricular posterior wall thickness to left ventricular end-diastolic dimension) ≤ 0.12 - 9%. In assessing the cumulative survival rate, the indices provided by Echo revealed similar values when compared with those obtained by Cathe. The relative wall thickness determined by Echo would be the most reliable parameter for forecasting the 5-year survival rate. In the majority of patients (22/24) who died during the observation period, the relative wall thickness gradually decreased to less than 0.12. Therefore, a relative wall thickness below 0.12 would be the most reliable index for detecting a poor prognosis case. It is concluded that echocardiographic observation of patients with idiopathic dilated cardiomyopathy can provide information useful not only in prognosis but also in assessing the effect of medical treatment.

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The serum S-IgA levels of 33 patients with diffuse panbronchiolitis (DPB) were compared with those of 13 patients with chronic bronchitis (CB) and 24 patients with bronchiectasis (BE), to obtain information on differences in the pathologic states in DPB and other chronic bronchial diseases. The S-IgA levelw as elevated in all three bronchial diseases, being significantly higher in DPB than in CB, and intermediate in BE. Persistent bacterial infections developed in most of the patients with DPB and two-thirds of those with BE, but in few of those with CB. Serum S-IgA levels were especially high in patients expectorating Pseudomonas aeruginosa-positive sputum, who constituted two-thirds of the patients with DPB and about one-third of those with BE. The highest levels over (100 μg/ml) were observed in far-advanced patients with DPB who expectorated P. aeruginosa-positive sputum. The increase in the serum level of IgA was less than that of S-IgA in all three diseases. These results indicate that the marked elevation of the serum S-IgA level in patients with DPB is due to extensive, chronic infection of the airways of the lungs, especially the peripheral airways, and that serum S-IgA is a useful marker for determining the clinical stage and the pathologic state of patients with diffuse peripheral airway diseases.

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We studied the usefulness of antibody estimation by the enzyme-linked immunosorbent assay (ELISA) technique in the differential diagnosis of pulmonary mycobacterial diseases. Sera from patients with active pulmonary diseases caused by Mycobacterium tuberculosis (n = 42) or Mycobacterium avium-intracellulare complex (MAI) (n = 26) and from healthy controls (n = 75) were assayed for IgG and IgA antibody titers to antigen of M. tuberculosis and MAI. The IgG and IgA antibody titers to M. tuberculosis antigen were significantly higher in patients with mycobacterial diseases than in healthy controls, however, there was no significant difference between patients with pulmonary tuberculosis and those with pulmonary diseases due to M. avium-intracellulare complex (MAID). The IgG and IgA antibody titers to MAI antigen in MAID patients were significantly higher than in healthy controls and in patients with pulmonary tuberculosis. ELISA for IgG antibody against M. tuberculosis antigen had a sensitivity of 54.8% and a specificity of 73.1% in the differential diagnosis of pulmonary tuberculosis. The estimation of the IgA antibody against MAI antigen using ELISA is more useful for the differential diagnosis of MAID because of its sensitivity (69.2%) and specificity (90.5%).

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Generation of oxygen free radicals at the time of reperfusion has been implicated as a major causative factor for myocardial reperfusion injury. In order to examine if oxygen free radical scavenger superoxide dismutase (SOD) in human plasma prevent reperfusion injury with successful thrombolysis and limit infarct size, we studied the relationship between infarct size and plasma SOD activity in 25 patients received thrombolytic therapy in antero-septal acute myocardial infarction. Plasma SOD activities were measured by the nitrate method. There was no variance in plasma SOD activity in patients with acute myocardial infarction before and after thrombolysis. Infarct size was evaluated by using % abnormally contracting segments (% ACS) calculated with left ventriculography at chronic stage. Humanplasma SOD activities had a significant negative correlation with % ACS (r= -0.58, p <0.05) in 17 patients with successful thrombolysis. This significant correlation was still shown in 14 patients without collaterals before recanalization (r= -0.56, p <0.05). On the other hand, there was no significant relationship between plasma SOD activities and % ACS in 8 patients with a complete occlusion after thrombolysis (r= -0.081). These results showed that human plasma SOD prevent the reperfusion injury and limit the infarct size in patients with successful thrombolysis and imply that the generation of free radical at the time of reperfusion results in myocardial reperfusion injury.

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A 66-year-old woman with rheumatoid arthritis experienced severe hyponatremia and hypoglycemia of repeated occurrence. Endocrinologic examinations revealed hypopituitarism and the metrizamide computerized tomographic scan showed a primary empty sella. No evidence of a pituitary or suprasellar tumor was obtained. During the 5-year follow-up, she remains well under replacement therapy and there are no signs of an intracranial tumor. The possibility of an autoimmune mechanism linked to the development of hypopituitarism is discussed.

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The patient was a 67-year-old male. A diagnosis of sarcoidosis was made both by transbronchial lung biopsy and by scalene node biopsy. The findings of his chest roentgenogram were categorized as the group II. There were no symptoms during follow-up period of one year at the out-patient clinic with no medication. The chest X-ray findings of the patient were slowly progressive. Intractable hiccup developed one year after his first visit to the hospital. With administration of prednisolone, serum angiotensin converting enzyme level decreased promptly, and the incidence of the hiccup attack decreased. As the dose of prednisolone was tapered, the hiccup recurred. All the drugs used for hiccup including quinidine sulfate and methochropramide was not effective. A thoracic CT revealed slight enlargement of mediastinal lymph nodes. A brain CT and a brain MRI demonstrated no abnormalities. Acetazolamide, which induces acidosis in brain extra-cellular fluid, worsened the symptoms. As prednisolone was given again, infiltrates on the chest roentgenogram began to resolve and the incidence of hiccup decreased. We conclude that the long-standing hiccup observed in this patient may be brought by the lesion in central nervous system due to sarcoidosis.

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We report a case of IgG multiple myeloma with selective complete Clq deficiency. The patient was a 75-year-old Japanese woman who exhibited urticaria on the arm and an absence of serum hemolytic complement activity (CH50). Further studies revealed no vasculitis in the urticarial lesion but showed selective complete deficiency of Clq without low molecular weight Clq precipitin. Addition of highly purified Clq restored the CH50 level of the patient's serum to normal. It is suggested that this abnormality was a primary Clq deficiency. We discussed a relationship between the Clq deficiency and myeloma and reviewed the literature.

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A case of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with Ramsay Hunt syndrome is reported. A 59-year-old man was admitted to our department for treatment of left facial pain that had persisted for three days. A left renal tumor had been diagnosed and a radical nephrectomy had been performed two months earlier. On admission, vesicular lesions were found in the left external auditory canal and ear lobe. Additionally found were left facial nerve palsy and hearing loss. Acyclovir 5 mg/kg, three times per day, was started. Five days after admission, the patient became confused and disorientated. Further investigation revealed hyponatremia 118 mEq/l, low serum osmolality, high urine osmolality, normal renal function, normal adrenal and thyroid hormones, and high plasma vasopressin 30 pg/ml. Although cerebrospinal fluid (CSF) examination revealed a mild elevation in protein and cells, no malignant cells were present and bacterial examinations were negative. Antibodies to varicella-zoster virus (VZV) in both IgG and IgM were present in high titers not only in the serum but also in the CSF. Intravenous hypertonic saline and water restriction were started, and the patient's sensorium was improved in accordance with the increase in serum sodium concentration. These results indicate that the hyponatremia in this case was due to SIADH and that SIADH was caused by an increased release of vasopressin probably because of the infection of VZV in the central nervous system.

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A 49-year-old man with hyperthyroidism caused by the syndrome of inappropriate secretion of TSH (SITSH) is reported. A diagnosis of infertility and hypospermatogenesis due to primary hypergonadotropic hypogonadism had been made 10 years ago. Serum TSH levels were inappropriately elevated with thyrotoxic high levels of thyroid hormone in serum, but no tumorous mass was demonstrated in the pituitary gland. The non-tumorous pituitary resistance of TSH to thyroid hormonal suppression was confirmed by low levels of glycoprotein α-subunit and low α-subunit/TSH ratios in serum before and after the stimulation by TRH. In addition, the patient had elevated levels of LH and FSH in serum, and their exaggerated responses to LH-RH, although serum testosterone concentrations were normal, indicating primary hypogonadism. It is of interest that SITSH appeared in the hypogonadal patient who was preceded by the long standing hypersecretion of LH and FSH.

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A 29-year-old woman with acute myelogenous leukemia in relapse at the end of a nine-month period of remission was admitted to hospital where intensive antileukemic therapy was started. Antibiotics were given when she developed a fever and, when oral thrush appeared, intravenous amphotericin B was initiated. After 16 days, the amphotericin B (at a total dose of 295 mg) was discontinued because of side effects and 12 days later, when a lung biopsy had revealed mucormycotic hyphae in infarcted tissue in the left upper lobe, fluconazole (300 mg daily by intravenous infusion over a period of two hours) was substituted. This was continued for one month with clinical and radiologic improvement in the lung condition and no attributable adverse effect. At this juncture the patient died of intractable heart failure. We suggest that fluconazole may be an acceptable alternative to amphotericin B in the treatment of pulmonary mucormycosis.

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The case of a 40 year-old woman with insulin dependent diabetes mellitus associated with relapsing polychondritis, Hashimoto's thyroiditis and pituitary adrenocortical insufficiency in succession, considered as polyglandular autoimmune syndrome type III, is described. The results of this study suggest that relapsing polychondritis and pituitary adrenocortical insufficiency might be included in polyglandular autoimmune syndrome. It is very interesting that in the pathogenecity of polyglandular autoimmune syndrome type III, the elevation of OKT4/OKT8 ratio which indicates the hypo function of suppressor T cell is shown and that she has HLA DR4 which has a significant relationship with insulin dependent diabetes mellitus in Japanese.

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The occurrence of rhabdomyolysis and acute renal failure associated with viral infections is uncommon. We report a case of a 17-year-old female suffering from rhabdomyolysis caused by echovirus 9 in which the patient recovered after being treated for acute renal failure with hemodialysis. Among 42 cases of virus-induced rhabdomyolysis review in Japanese and English literatures, influenza virus is the most common cause of rhabdomyolysis, coxsackie virus tends to be next most commoncause. Renal failure was recognized in 31 of the cases.

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A 74-year-old female having primary hemochromatosis and hyperthyroidism is described. The initial ECG showed sinus rhythm, and depression of ST segments and inversion of T waves in I, II, III, aVF, and V4-6. By deferoxamine and propylthiouracil, the serum level of ferritin was decreased from 4, 500 ng/ml to 440 ng/ml in a period of 6 months. The thyroid function was also returned to normal. After cessation of both drugs, the serum ferritin level increased gradually reaching a level of 3, 100 ng/ml in the next 15 months but the thyroid function remained normal. During and after the deferoxamine administration, the depth of inverted T waves became more shallow and gradually deeper again, respectively. There seemed to be a correlation between the depth of inverted T waves and the serum level of ferritin. It was, however, unlikely that toxic iron may have induced the hyperfunction of the thyroid gland.

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A patient with acute myeloblastic leukemia (AML) developed septicemia due to Bacillus cereus with subsequent rhabdomyolysis and myoglobinuric renal failure. He died despite intensive care. Postmortem examination revealed diffuse muscle necrosis with infiltration of Gram-positive bacilli and widespread bacterial microthrombi in various organs. Septicemia associated with rhabdomyolysis has been described in 12 cases. This case represents the first reported case of B. cereus septicemia associated rhabdomyolysis. Renal failure and shock were considered to be the most important prognostic factors, and either direct infiltration or toxin of the bacteria was suggested to be the mechanism of rhabdomyolysis in sepsis. B. sereus can be one of the lethal organisms in immunocompromised patient such as the present case. Rhabdomyolysis should be considered when a patient with septicemia complains of muscle pain. Prompt hydration and correction of acidosis are important to prevent renal failure and shock.

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The liver involvement by tuberculosis in the macronodular form is quite rare and one must know their clinical features and actual imaging to have precise diagnosis. We present a case of the solitary hepatic tuberculosis, of which angiographic findings originally suggested hepatocellular carcinoma (HCC) given Lipiodol Ultra Fluid (LUF). On angiogram the tuberculoma showed marked hypervascularity in a capillary phase. Histological examination of the biopsy specimen disclosed gramulomas with central coagulation necrosis. A good clinical response was achieved with a course of Isoniazid and Rifammpicin. Angiographic findings of one year later showed hypovascularity. Meanwhile LUF remained long in the tuberculoma marginally, presenting nodular opacities on lipiodolized CT. This finding was different from that of HCC. We also discussed those clinical actual imaging and pathological findings.

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