Plasma 18-hydroxycorticosterone (18-OHB) and aldosterone responses to angiotensin II (AH) and ACTH were examined in 2 patients with a 18-OHB-producing tumor (18-OHBPT) versus those in 8 patients with a aldosterone-producing adenoma (APA), 7 patients with low renin essential hypertension (LREH) and 10 normal subjects. Plasma 18-OHB and aldosterone levels and the 18-OHB: aldosterone ratio were high in patients with an APA and normal in patients with LREH. In patients with a 18-OHBPT, plasma 18-OHB and aldosterone levels were high and normal, respectively, resulting in a 2-fold greater 18-OHB: aldosterone ratio than that in patients with an APA. Patients with an APA had a blunted response of plasma 18-OHB and aldosterone to All and a supranormal response of these corticoids to ACTH. Patients with LREH had a supranormal response of plasma 18-OHB and aldosterone to AH and a normal response of these corticoids to ACTH. In patients with a 18-OHBPT the responses of both plasma 18-OHB and aldosterone to AH and ACTH closely resembled those in patients with an APA but not in patients with LREH.
These data suggest that 18-OHBPT may be a variant of aldosteronomas, producing a large amount of 18-OHB and a small amount of aldosterone.

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A differential diagnosis of polymyalgia rheumatica (PMR) and rheumatoid arthritis (RA) can be difficult in elderly patients, especially at the prodromal phase of RA. Furthermore, it was felt that there might be a diagnostic criteria for PMR specific to the Japanese. Accordingly, we have retrospectively studied the PMR patients who had come to our hospital, for making a diagnostic criteria. We especially stressed the distinguishing of PMR from RA in elderly patients. The criteria proposed are as follows: 1. Bilateral muscular pain persisting for 2 weeks at least in more than two of the following areas; neck, shoulders or shoulder girdle, upper arms, hips or pelvic girdle and thighs. 2. Normal serum myogenic enzymes. 3. ESR of more than 40 mm/h. 4. No swelling in the hand joints. PMR is defined by the presence of all of the 4 items. The criteria demonstrated a 93.1% sensitivity and 98.3% specificity for PMR.

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To determine the effect of diabetic treatment on plasma high density lipoprotein (HDL) composition and lecithin: cholesterol acyltransferase (LCAT) activity, 196 diabetic patients admitted to our hospital to receive diabetic control were divided into three groups according to the type of treatments; insulin treated group (91 patients), sulfonylurea group (56) and diet alone group (49). Plasma lipids, lipoproteins, apolipoproteins and LCAT activity were measured before and after the admission for 2-3 weeks. As compared with the pre-admission, the following results were obtained at the time of discharge in each group. 1) Body weight, fasting blood glucose, triglyceride, total cholesterol, apolipoproteins (A-I, A-II, C-II, C-III, E) were significantly decreased in each group. 2) Apoliprotein A-I/A-II ratio was significantly increased from 4.9 to 5.3 in insulin treated group (p<0.001). 3) Total HDL-cholesterol was significantly decreased in diet alone and sulfonylurea groups, however HDL 3-cholesterol was significantly decreased in sulfonylurea and insulin group. Then HDL 2-cholesterol/HDL 3-cholesterol ratio was significantly increased from 4.8 to 5.1 (p<0.001) in insulin group. 4) LCAT activity were significantly decreased from 790 to 637 (nmol/ml•h) (p<0.05) in insulin group, but did not change in other two groups. In conclusion, compared to diet alone and sulfonylurea groups, insulin group made significant reduction of HDL 3-cholesterol and LCAT activity during hospitalization.

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Sixty-eight patients with various malignancy was examined for their natural killer (NK) cell activity against 14 target cell lines. The group consisted of 10 patients with gastric cancer, 10 patients with lung cancer, 8 patients with hepatoma, 11 patients with cancer of female genital organs, 14 patients with malignant lymphoma and 15 patients with acute myelogenous luekemia (AML). The target cells from a variety of lineage were selected to examine the disease-related specificity in NK cell activity. The peripheral mononuclear cells from patients with gastric cancer did not show a decrease in NK activity against 14 targets including gastric cancer cell lines. Other patients except for AML demonstrated low NK activity against one or two target cells out of 14 targets. Whereas, NK activity in patients with AML was remarkably depressed against 10 target cells out of 14. At single cell assay, killing ability rather than binding activity to target was markedly impaired in AML. Comprehensively, the data demonstrated the marked difference in the NK level between the patients with solid tumor and the patients with hematopoietic malignancy. There existed neither disease-related specificity in NK cytolysis, nor correlation in NK levels and clinical severity in the patients with malignancy. These results suggested that it was very difficult to evaluate the anti-cancer capacity in patients with malignancy by NK activity alone.

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To study the risk of death and causes of death among female patients with tuberculosis (TB), a total of 1, 083 female patients who diagnosed as having active pulmonary TB and newly registered into the Nagoya TB registry between 1979-1981 were followed up till the end of 1983. During follow-up period 138 deaths (12.7%) were observed, a significantly higher rate than expected from the general population. Observed deaths (O) from all types of cancer (O/E ratio &equal 2.5), lung (6.4) and colorectal cancers (5.0), were significantly higher than that which was expected (E). The number of deaths from TB was naturally high (O/E ratio &equal 40.0, p<0.001). Malignant lymphoma and multiple myeloma also showed high relative risk. The smoking rate among the patients was 14%, about the same as with the general population. Excessive high rates of death from lung cancer were observed both among smoking and non-smoking cancer patients. Non-smokers showed a high proportion (60%) of adenocarcinoma. The proportion of the use of antituberculous drugs during the past five years was not different between the lung cancer group and the non-lung cancer group. Patients who died from cirrhosis of the liver showed more frequent use of antituberculous drugs than the others. Factors causing excess incidence of colorectal cancer remain unknown. It was suggested that some immunologic disorders in TB patients may play some role in the development of cancer.

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We measured adenosine deaminase (ADA), lysozyme, fibronectin and carcinoembryonic antigen (CEA) in the pleural fluid of tuberculous and carcinomatous pleural effusion in order to discriminate these two groups. Tuberculous pleural effusion had significantly higher levels of ADA and lysozyme than did carcinomatous effusion. When ADA activity of more than 33 IU/ι is considered, diagnostic tests of tuberculous effusions showed a sensitivity of 100%, specificity of 95% and accuracy of 96%. A pleural fluid/serum ADA ratio (pl-ADA/s-ADA) above 1.1 was found in 100% of tuberculous and in 53% of carcinomatous effusions (sensitivity 100%, specificity 47%, diagnostic accuracy 70%). A lysozyme level above 12 μg/ml, selected as the discriminating limit, was found in 100% of tuberculous and in 17% of carcinomatous effusions (sensitivity 100%, specificity 83%, diagnostic accuracy 88%). Pleural fluid/serum lysozyme ratio (PL/SL) was also valuable in the discrimination of these two groups. When the cut-off level of 1.2 was considered, diagnostic tests of tuberculous effusions showed a sensitivity of 100%, specificity of 88% and accuracy of 93%, respectively. The mean fibronectin concentration in pleural fluid with tuberculous effusion was significantly higher than that in carcinomatous effusion, but there was a marked overlap between these two groups. On the other hand, CEA was significantly higher in carcinomatous effusions than in tuberculous effusions. At a cut-off level of 5 ng/ml, 53% of patients with carcinomatous effusion showed elevated pleural fluid CEA levels, while none of the tuberculous effusion did (sensitivity 53%, specificity 100%, diagnostic accuracy 65%). The combined assay of ADA, lysozyme, pl-ADA/s-ADA, PL/SL and CEA provide valuable information in the differential diagnosis between tuberculous and carcinomatous effusion, while fibronectin concentration can not be considered to have a definite diagnostic value.

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The natural killer (NK) cell activity of the blood and marrow was studied in patients with acute myelogenous leukemia (AML). NK activity of the cells from blood and marrow was significantly decreased in AML to three target cell lines. Both binding and killing capacities of the effector cells were deeply depressed in the blood as well as in the marrow at single cell assay. Surface phenotypic analysis showed a significant decrease in CD 11⁺ cell subsets, but not in CD 16⁺ or Leu-7⁺ cells, in both blood and marrow cells from AML. A significant decrease of large granular lymphocytes (LGL) was also displayed in these samples at morphological examination. The effector cells from AML patients poorly responded to interferon stimulation in NK cytolysis. Taken together, a decrease in CD 11⁺ cell population with LGL morphology appeared to be responsible for the impaired NK activity in patients with AML.

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The aim of this study of 225 duodenal ulcer patients was to determine the association of cigarette smoking to ulcer recurrence. In this study, smokers were mostly men whose smoking habit was positively associated with a past history of duodenal ulcer, stress and alcohol consumption. These characteristics are regarded to favor ulcer recurrence. In 161 patients not on maintenance therapy, smokers had a higher recurrence rate than nonsmokers, even when foregoing patients characteristics were taken into consideration. No difference in recurrence rates could be observed between nonsmokers and smokers in 64 patients under maintenance therapy. In conclusion, our findings suggest that smoking plays a major role on recurrence of duodenal ulcer and that maintenance therapy can overcome the adverse effect of cigarette smoking on ulcer recurrence.

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To clarify magnesium metabolism in thyroid dysfunction, plasma and erythrocyte magnesium concentrations (P-Mg, E-Mg) were determined in patients with untreated thyroid diseases and their relations to thyroid hormone levels and the duration of illness were examined. P-Mg was significantly lower in hyperthyroid patients than in euthyroid or hypothyroid individuals. E-Mg was significantly higher in hypothyroid patients than in hyperthyroid or euthyroid individuals. In the entire series of 84 subjects, both P-Mg and E-Mg showed significant negative correlations with thyroid hormone levels, but the correlations were greater in P-Mg than E-Mg. In hyperthyroid patients, both P-Mg and E-Mg were negatively correlated with the duration of illness, but this correlation was greater in E-Mg than P-Mg. Also, both P-Mg and E-Mg were significantly higher in patients with destructive thyroiditis with a short duration (0.5 months) such as subacute or painless thyroiditis than in patients with Graves' disease (4.9 months). These results suggest that magnesium metabolism in thyroid dysfunction is affected not only by thyroid hormone levels but also by the duration of illness.

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A case of diffuse panbronchiolitis (DPB), associated with benign monoclonal IgA gammopathy and IgA nephropathy is described. The IgA deposition in the glomeruli of the patient was identified as consisting mainly of the monoclonal IgA having the same idiotype as that of serum monoclonal IgA. Recurrent infections of Hemophilus influenzae and Pseudomonas aeruginosa in the respiratory tract might have enhanced IgA-mediated immunity at the mucosal surface of the bronchiole, and ultimately induced monoclonal IgA gammopathy and IgA nephropathy.

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To determine whether pulmonary blood flow is altered by cold exposure to the hand, cold exposure was induced by immersion of the left hand in 7°C water in 8 patients with progressive systemic sclerosis (PSS). Lung perfusion was measured by continuous infusion of ⁸¹M krypton into the right antecubital vein. Exposing the left hand to cold caused an 11 to 32% decrease in the pulmonary blood flow in all lung fields in 4 of 8 patients. After release of cold exposure, in one of the 4 patients demonstrating a decrease during cold exposure, pulmonary blood flow recovered, while it kept on a decrease in the remaining 3 patients. Physiological abnormalities of pulmonary blood flow in PSS have been shown as a quantitative change and a image.

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Since the natural history of a coronary artery to left ventricle fistula is not well known, a case of such a fistula in 36-year-old female patient, followed for 20 years is presented. Two-dimensional echocardiography performed as a follow-up examination revealed enlargement of the right coronary artery and the right Valsalva sinus. Two-dimensional color flow mapping showed the jet flow to the apex from the base of the left ventricle only during the diastolic phase. An aortogram confirmed that enlargement of both the right coronary artery and the right Valsalva sinus had occurred during the 20 years. The shunt flow ratio was about 50%. The Symbas procedure, closing of the coronary artery fistula by arteriotomy directly over the site of the fistula, was performed with the aid of extracorporeal circulation. Our findings indicate that enlargement of the shunted coronary artery and its Valsalva sinus can occur gradually where there is a relatively large shunt flow. Therefore, early surgical treatment is recommended in cases like this one.

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We report a case of amyotrophic lateral sclerosis (ALS) in which the ability to close the eyes on command or voluntarily, was lost in spite of retention of reflex activity. A electrophysiological study of the blink reflex revealed a prominent R₁ component with normal latency, which confirmed that the blink reflex was exactly preserved and also suggested a hemispherical lesion. Postmortem examination disclosed prominent cortical and subcortical lesions of the precentral areas on both sides. These lesions seem to be very closely related to the inability to initiate lid closing.

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Pulmonary lymphangiomyomatosis (LAM) is characterized by proliferation of smooth muscles around lymphatics and bronchovascular bundles, presenting clinical manifestations of obstructive or restrictive lung disease with greatly impaired gas exchange. To date, little attention has been paid in the literature to the pathophysiology of pulmonary hypertension related to this disorder. A case of a 39-year-old woman with the characteristic clinical and histological features of pulmonary LAM is presented. She was found to have moderate pulmonary hypertension. Possible mechanisms for the development of pulmonary hypertension are discussed.

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A 57-year-old woman who had had renal amyloidosis with nephrotic syndrome for five years was found to have the Fanconi syndrome and monoclonal lambda light-chain proteinuria. The amyloidosis of this patient was of primary type on the basis of the permanganate-resistent amyloid, the presence of monoclonal light-chain proteinuria, and the absence of clinical and histologic evidence of multiple myeloma. To best of our knowledge, this is the first case report in which primary amyloidosis with monoclonal lambda light-chain proteinuria was accompanied by adult Fanconi syndrome.

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Three cases of bronchial asthma after middle ages associated with pets were presented. Case 1 was a 39-year-old man who was admitted due to exacerbation of asthma after having fed a cat and a dog for 2 years. IgE-RAST was positive for cat fur and house dust. Inhalation test of cat hair antigen was positive. His symptoms improved by avoidance of the cat. Case 2 was 54-year-old woman suffering from asthma for 6 years after having fed a cat. IgE-RAST was positive for dog hair and cat fur. Symptoms did not improve by any treatment because she could not give up pets. Case 3 was a 45-year-old womansuffering from asthma attacks for two months. She had kept a dog for 1 year. IgE-RAST was positive for dog hair. Her symptoms disappeared by avoidance of the dog. Patients with pet allergy have increased in recent years because of the pet boom, but it is not so common to have asthma attacks in aged people. Bronchial asthma caused by allergens of pets easily improves by avoidance of them and it is important to establish early diagnosis of this disorder.

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At the age of 29, a woman developed central nervous system manifestations of incontinence, psychosis and a grand mal seizure in February 1982. She was diagnosed as having systemic lupus erythematosus (SLE) based on photosensitivity, oral ulcers and elevated antinuclear and anti-DNA antibodies titers. Three years and one month later the patient had episodes of severe headache and vomiting during the course of maintenance treatment. CT examination of the head revealed blood within subarachnoid cisterns, and a small berry aneurysm was found at the distal portion of the basilar artery by cerebral angiography. The possible role of SLE-associated cerebral vascular changes in the development of this aneurysm is discussed.

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A 38-year-old female was admitted to our hospital because of dyspnea. The diagnosis of total lipodystrophy was made by following findings: (1) gaunt appearance; (2) insulin-resistant diabetes mellitus; (3) hyperlipidemia; (4) fatty liver. Chest X-ray demonstrated cardiomegaly, pulmonary edema and pleural effusion. Echocardiogram was characterized by left ventricular hypertrophy with asymmetrical septal hypertrophy and left ventricular dysfunction. Renal biopsy revealed focal glomerulosclerosis. We reported a patient with total lipodystrophy combined with heart failure and renal failure, which have been rarely associated with the disease.

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