We studied 18 sera of Sjögren's syndrome (SS) with monoclonal gammopathy. Monoclonality was established by typical immunoelectrophoretic findings in all patients and confirmed by idiotypic (Id) studies in 12 patients. Four of the monoclonal gammopathies were of the IgG class, 8 were of the IgA class and 4 were of the IgM class, and 2 patients had 2 M proteins (IgMK/IgGK and IgAK/IgGK). The monoclonal rheumatoid factor (RF) was found in 6 patients (4 IgA and 2 IgM). A review of the literature revealed additional 19 monoclonal gammopathies (2 IgG, 9 IgA, 7 IgM and one Bence Jones protein) in Japanese SS patients. In non-Japanese SS patients, 27 monoclonal gammopathies (4 IgG, 2 IgA, 20 IgM and once Bence Jones protein) were reported. Both Japanese and non-Japanese patients showed a higher incidence of monoclonal gammopathies in primary than in secondary SS. The non-IgM class monoclonal gammopathies were predominant in Japanese SS patients, whereas monoclonal gammopathies were mostly confined to the IgM class in non-Japanese SS patients. These results indicate that monoclonal gammopathy is another significant complication of SS.

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To assess the role of catecholamines in the pathogenesis of high altitude pulmonary edema (HAPE), 24-hour urinary catecholamine excretions and plasma catecholamine concentrations were measured. We also performed brain computed tomography (CT) in the same 8 serial patients with HAPE. 24-hour urinary norepinephrine excretion showed a statistically high value on admission compared with that after recovery (396.9±92.9 ug/24 hr, decreasing to 93.0±31.1 ug/24 hr, means±S.E., p <0.01). 24-hour urinary epinephrine excretion on admission was also remarkably high, and returned later normal (62.9±25.8 ug/24 hr, decreasing to 12.7±4.2 ug/24 hr, p < 0.05). Plasma norepinephrine concentration was also high, but returned to normal (0.260±0.073 ng/ml, to 0.11±0.043 ng/ml) by the time of discharge. Brain CT scans revealed diffuse low density of the entire cerebrum, small ventricles, disappearance of sulci, and small cistern, suggesting cerebral edema. These findings may suggest that the heightened sympathoadrenal activity that occurred in the patients concomitant with cerebral edema was related to the high altitude illness rather than to simple exposure to high altitude.

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Mediastinal lymph nodes metastasis of lung cancer was analysed by endoscopic ultrasonography in 96 patients in whom histological diagnosis of the lymph nodes could be proven by thoractomy. A Receiver Operating Characteristic (ROC) curve was drawn up for determining the criteria for metastatic lymph nodes by endoscopic ultrasonography. The ROC curve was evaluated by using four parameters: The long diameter, the short diameter, the long diameter plus the short diameter, and the product of the long diameter times the short diameter. The long plus short diameter and the long-times-short diameter showed the highest detectability of the metastasis of all histological types on the ROC curve. The reasonable criteria levels were found to be 18mm and 75 mm² by means of a moderate threshold. In this level, sensitivity were 77%, 76%; specificity 84%, 84%; and accuracy 82%, 82%, respectively. When examinations were done by histological types, the criteria level for the epidermoid carcinoma should be increased to more than that for the adenocarcinoma. And, sensitivity and specificity were better in the epidermoid carcinoma. Thus, these results suggest that endoscoptic ultrasonography is clinically useful for the detection of lymph nodes metastasis in lung cancer.

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The sacral segments of the spinal cord in 15 cases of amyotrophic lateral sclerosis and 7 control cases were investigated using peptides immunocytochemistry. A rich supply of somatostatin-, enkephalin- and substance P-immunoreactive fibers and terminals were found in common with the intermediolateral cell column in Onuf's nucleus, but not with the motor neurons in ventral horn. Furthermore, there was no significant difference with peptides innervation between ALS and control cases. Onuf's nucleus, though located in the ventral horn of the upper sacral segment, has a rich supply of peptidergic innervation which suggests that this nucleus does not represent merely somatic motor neurons, but also represents autonomic neurons. This may be related to sparing of Onuf's nucleus along with the autonomic sacral intermediolateral nucleus in ALS.

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A highly sensitive and specific quantitative assay for inositol in serum has been developed. Uremic serum, to which hexahydroxybenzene as an internal standard was added, was deproteined with trichloroacetate. The supernatent aqueous phase was taken after lipids in serum were removed with chloroform, and was lyophilized. The dried powder was converted to butylboronate derivatives by reaction with 5% n-butylboronate (pyridine solution). The reaction mixture was gas chromatographed on fused silica megabore column DB-1, and assayed by electron impact mass spectrometry using selected ion monitoring. Inositol was quantified by the internal standard method. It was possible to determine concentrations as low as 0.1 μg/ml in 0.5 ml of serum sample with a relative standard deviation of less than 5%. The serum inositol concentration from fifteen fasting patients with chronic renal failure was 39.1 ± 18.2 μg/ml, significantly higher than that of control subjects (5.1 ± 2.1 μg/ml). In the fifteen fasting patients, serum inositol was linearly related to blood urea nitrogen (r = 0.96).

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In order to elucidate active oxygen in liver diseases, activities, electrophoretic profiles and immunolocalization of superoxide dismutase (SOD) in human liver specimens were investigated. Activities and electrophoresis were studied using liver homogenates in 41 cases and immunolocalization of Cu, Zn SOD was observed in 87 cases. Total SOD activity in acute viral hepatitis (AVH) and fatty liver (FL) groups was significantly lower than that in non-specific reactive hepatitis (NSRH) group. Cu, Zn SOD activity in AVH, FL and chronic active hepatitis (CAH) groups was also significantly lower than that in NSRH group. However, no difference of Mn SOD activity, was found between NSRH group and others. Decreased activity of superoxide dismutase in liver tissues suggests the release of this enzyme from the injured hepatocytes. In electrophoretic patterns of superoxide dismutase, 3 bands of Cu, Zn SOD isozymes and 8 to 10 bands of Mn SOD isozymes were recognized. Immunocytochemical investigation revealed the localization of Cu, Zn SOD in the cytoplasm of hepatocytes. Two different distribution of Cu, Zn SOD was observed in the lobules: a diffuse localization pattern and a focal one. The latter was found in the cases of liver diseases with severe parenchymal lesion. These findings suggest that superoxide radical anion and its scavenger, superoxide dismutase, may play an important role in the pathogenesis of liver cell necrosis.

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Thyroid function was studied in 40 patients with chronic heart failure. Thyroid antibodies and microsome antibodies were negative in all cases. Serum T₄, and T₃ concentrations showed significant inverse correlation with cardiothoracic ratio, mean right atrial pressure, pulmonary artery systolic pressure, and peripheral venous pressure. Serum T₄, T₃ concentrations showed significant correlation with PaO₂, serum albumin, and serum cholinesterase. Serum TSH concentrations increased with increasing cardiothoracic ratio. Histological examinations showed fibrosis and atrophy of the thyroid gland in 2 cases. These findings suggest the possible development of primary hypothyroidism as a result of chronic heart failure.

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Peptide YY concentrations in both rectal mucosa and fasting plasma, which were produced by endocrine cells in the lower gastrointestinal tract, were measured in normal subjects and patients with ulcerative colitis by specific radioimmunoassay and were examined in the relation to histopathological changes and extent of lesions of ulcerative colitis. Peptide YY levels of rectal mucosa (1 1.0 ± 1.4 pmol/mg protein) and fasting plasma (53.3 ± 7.9 fmol/ml) of patients with ulcerative colitis were significantly lower (p < 0.001) than those of normal subjects (31.5 ± 3.4 pmol/mg protein and 160.7 ± 19.7 fmol/ml). It appears that the decreased PYY level in rectal mucosa and fasting plasma is related to crypt destruction and reduction of mucosal cell. These changes of PYY levels in rectal mucosa and fasting plasma suggest a presence of relationship between concentrations of peptide YY and severity of lesions in patients with ulcerative colitis.

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A Japanese group comprising 54 hyperlipidemic and 73 normolipidemic subjects were genotyped for DNA restriction fragment length polymorphisms (RFLPs) at the apoAI-CIII gene locus. The polymorphisms with Sstl were present at allelic frequencies of 0.63 (SI allele) and 0.37 (S2 allele). Unlike the previously reported associations of the S2 allele with hypertriglycedemia found in Caucasians there was no difference in the frequency of S2 allele between normolipidemic and hyperlipidemic Japanese subjects. No significant association of genotypes were observed with serum levels of lipids and apolipoproteins, except for the association of the S2 allele with the higher mean level of HDL-cholesterol. In addition, among 26 patients who had coronary artery disease (CAD), as documented by angiography, there was no significant difference in the distribution of polymorphic alleles between CAD patients and normolipidemic control subjects.

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The purpose of the present study is to determine polymorphonucleocytal elastase-α₁-protease inhibitor complex in bronchoalveolar lavage fluid (BALF) in order to investigate the clinical singificance of the increased complex in BALF. BALF was collected by the usual method using a bronchial fiberscope and concentrated 5-10 folds by Amicon UM-2 ultra filtration. Determination of the complex in BALF and plasma was made in accordance with the method of Neumann et al. The level of complex (μg/mg albumin, mean value) in BALF was 0.38 in healthy non-smokers (n=5), 3.14 in healthy smokers (n=6), 5.61 in the patients with chronic obstructive pulmonary disease (COPD) (n = 9) and 1.78 in the patients with idiopathic pulmonary fibrosis (IPF) (n=15), that is, higher in healthy smokers and COPD and IPF papitents. The ratio of the complex in BALF vs plasma was higher in healthy smokers and in COPD patients than in healthy non-smokers. Furthermore, there was a positive correlation between the level of this complex in BALF and the number of PMN in BLAF (r=0.68, p <0.01). From the above it was assumed that the release of elastase from PMN is progressed in the lower airway of smokers and COPD patients.

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Serum transferrin receptor concentration in patients with anemias and polycythemias was determined. The mean normal value was 258±82 ng/ml. Comparing with normal values, the higher values were obtained in patients with iron deficiency anemia, autoimmune hemolytic anemia, and polycythemias, while the lower values were obtained in patients with aplsatic anemia. During treatments of patients with iron deficiency anemia and autoimmune hemolytic anemia, serial change of serum receptor was pararelled with that of peripheral reticulocyte counts, suggesting that the circulating receptor values may reflect the activity of bone marrow erythropoiesis. The immunoreactive receptor was migrated at the position of α₁-globulin by agarose gel electrophoresis and the isoelectric point(pl) was 3.57 by isoelectric focusing. SDS-polyacrylamide gel electrophorogram of various patients' sera revealed that the autoradiographed band was migrated at the molecular weight of 110, 000 daltons in the non-reducing condition and 46, 000 and 23, 000 daltons in the reducing condition. These bands were also capable of binding to ¹³¹I-labelled diferric transferrin. The proposed model of circulating transferrin receptor may be the nicked dimers of 55, 000 daltons in which inter- and intra-disulfide bridges were present.

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We describe a case of a 29-year-old Japanese woman with toxic shock syndrome which occurred 6 days after a normal vaginal delivery. This is the second case of postpartum toxic shock syndrome in Japan, so far as we know. Staphylococcus aureus was isolated from the uterine cavity. The isolate was coagulase type II and it produced both enterotoxin C and toxic shock syndrome toxin-1. The clinical course and laboratory data for this patient were in conformance with previously reported typical postpartum toxic shock syndrome, except for the onset time after delivery, because the previously reported postpartum TSS cases were clearly divided into two groups, early onset within 3 days of delivery and late onset 2 or more weeks after delivery.

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A 36-year-old man who was diagnosed to have the Mar fan syndrome with an XYY chromosone pattern is reported. He was tall with long limbs and arachnodactylia, and had severe aortic regurgitation (AR). The chromosome pattern studied in specimens of blood and bone marrow revealed an XYY chromosome pattern. The relationship between the Mar fan syndrome and an XYY chromosome pattern is discussed.

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We have identified two cases in which anomalous papilla of Vater was found emptying into the duodenal bulb. In both instances, bile outflow from the papilla was confirmed endoscopically and endoscopic retrograde cholangiopancreaticography (ERCP) was performed.

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We report a male patient with lupoid hepatitis who developed primary liver cell cancer 8 years after the onset of lupoid hepatitis. Primary liver cell cancer is thought to be a rare complication of lupoid hepatitis. To our konwledge, this complication has been previously reported in only 6 patients. The etiology of chronic active hepatitis is not known, but is strongly suspected to be associated with an autoimmune mechanism. There is evidence of an increased incidence of malignancy in other forms of autoimmune disease, and this may represent a further contributing factor to the development of primary liver cell cancer in cases of chronic active hepatitis.

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Bronchoalveolar lavage (BAL) was performed in order to reveal inflammatory immune processes during inhalation provocation test in a patient with farmer's lung disease. Three hours after inhalation challenge the patient showed dyspnea and an increase in body temperature to 38.9°C, restrictive and obstructive impairment, and a decrease in peripheral lymphocytes from, 2.8 × 10³/mm³ to 1.8 × 10³/mm³, although peripheral granulocytes increased from 4.4 × 10³/mm³ to 10.3 × 10³/mm. BAL fluid at that time showed increases in lymphocytes, granulocytes and macrophage, as well as proteins and complements, compared with that before the inhalation test. These findings indicate that with inhalation challenge in a patient with farmer's lung disease, inflammation increases vascular permeability while protein loss is followed by an increase in lymphocytes, granulocytes and macrophage; the lymphocytes migrate to the lung from peripheral blood.

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We reported three patients who developed disseminated intravascular coagulation (DIC) accompanying thoracic and abdominal aortic aneurysm. The first case, a 26-year-old man with dissecting aortic aneurysm developed DIC with clinical bleeding after operating on glaucoma. The administration of fibrinogen concentrates and antifibrinolytic agent made his DIC improve. The second case, a 70-year-old man with abdominal aortic aneurysm developed DIC showing large ecchymosis after angiography. His DIC disappeared after operation on aneurysm. The third case, a 73-year-old woman with thoracic and abdominal aneurysm developed laboratory-DIC without severe hemorrhagic diathesis. During antifibrinolytic therapy, platelet count, fibrinogen and fibringen degradation product (FDP) level improved. Since the treatment of the coagulaopathy might be varied in the situation of the cause and clinical course, it is noted that anti-fibrinolytic therapy was effective in our two cases.

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