In 13 patients with an implanted dual-chamber atrioventricular (AV) demand pacemaker, left ventricular performance was elicited by pacing mode manipulation for study using gated cardiac pool scintigraphy at rest and during exercise. There was no significant difference between DDD and VVI at 70 and 90 beats/min with respect to cardiac output, peak ejection rate or peak filling rate. At 110 beats/min, the cardiac output was greater with DDD as compared to VVI. The peak filling rate was also significantly greater with DDD as compared to VVI (DDD : 3.6 vs VVI : 2.8 EDV/s, p<0.05). During exercise the cardiac output was greater with DDD as compared to VVI at the same rate. The peak filling rate during exercise was significantly greater with DDD as compared to VVI (DDD : 3.0 vs VVI : 2.5 EDV/s, p<0.01). We conclude that DDD is more beneficial than VVI in maintaining cardiac performance during exercise.
(Internal Medicine 31 : 1-5, 1992)

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The effect of a new immunosuppressant, FK506, on experimental allergic neuritis (EAN) was examined in Lewis rats. EAN was induced by inoculation with bovine peripheral myelin. The EAN rats were divided into two groups. FK506-treated EAN rats were prophylactically administered FK506 by injection into the peritoneal cavity at a dosage of 5.0 mg/kg/day for 13 days beginning the day after inoculation. The control EAN rats were injected with only saline solution. FK506 prevented the development of EAN, histologically and clinically. In FK506-treated EAN rats, flow cytometric analysis of T cell subsets of the lymph nodes showed a significant decrease in W3/13 positive T cells on the 14th and 21st day and a decrease in W3/25 positive T cells on the 21st day after inoculation when compared with the control EAN rats. The percentage of OX-8 positive T cells were not significantly different between the two groups. Our results suggest that FK506 prevented the development of EAN by decreasing W3/13 and W3/25 positive T cells.
(Internal Medicine 31 : 6-10, 1992)

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Evidence of an acquired T cell-specific deficiency distinct from acquired immunodeficiency syndrome (AIDS) in a 63-yr-old Japanese female is provided. Recently, this patient suffered from primary invasive pulmonary aspergillosis. Skin tests to purified protein derivative of tuberculin (PPD) and Aspergillus antigens were negative. Upon admission to our hospital, her lymphocytes were exclusively unresponsive to T cell mitogens (concanavalin A, phytohemagglutinin, and OKT3). The level of cells defined by monoclonal antibodies (CD1, CD2, CD3, CD4, WT31, and CD5) was less than 3%. In contrast, no decrease in the number of red blood cells, platelets, neutrophils or B cells was apparent. Five years ago, the patient had a normal white blood cell and lymphocyte count. However, over the following 4yr, she developed lymphopenia. With medication, her pulmonary disease recovered, while lymphopenia still continued. The levels of immunoglobulins, complements and enzyme activities (adenosine deaminase and purine nucleoside phosphorylase) were normal. Moreover, several tests for HIV (ELISA and Western bolt) were negative suggesting that the T cell-specific deficiency was not a congenital immunodeficiency or AIDS but rather a new type of acquired immunodeficiency.
(Internal Medicine 31 : 11-16, 1992)

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To evaluate the effect of chronic airway inflammation on cough sensitivity and bronchial responsiveness, we measured the cough threshold to tartaric acid and bronchial responsiveness to methacholine (PC₂₀-FEV₁) in 13 asthmatic, 13 bronchitic (sino-bronchial syndrome) and 49 healthy non-atopic subjects. All subjects were non-smokers. The geometric mean value of the cough threshold was 9.55, 5.62 and 12.3% in asthmatic, bronchitic and normal subjects, respectively. The value in bronchitic subjects was significantly (p<0.02) lower than that in normal subjects. The geometric mean value of PC₂₀-FEV₁ in asthmatic subjects (0.63 mg/ml) was significantly lower than those in bronchitic (8.7mg/ml) (p<0.01) and normal subjects (21.4mg/ml) (p<0.01). There was no correlation between cough threshold and PC₂₀-FEV₁ values [correlation coefficient (r)= 0.06, p>0.1]. These results indicate that cough sensitivity is potentiated by chronic airway inflammation in bronchitis but not in asthma and suggest that cough sensitivity and bronchial responsiveness may be independently potentiated by different mechanisms resulting from chronic airway inflammation.
(Internal Medicine 31 : 17-21, 1992)

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Coronary vasoreactivity of patients with chest pain syndrome (CPS, 18 patients) was examined with intracoronary acetylcholine infusion test (ACh). For comparison, 10 patients with vasospastic angina (VSA) and 17 patients without chest pain (control group) were used. The luminal diameters of coronary arteries were measured before and after ACh, and the Maximal value of constriction rate of each segment (MCR) was used as index of vasoreactivity in each patient. By the Ach test, an average MCR of 42±23% was observed in CPS, 84±17% in VSA, and 26±12% in the control group. In CPS, chest pain was induced by ACh in 7 patients (group I), but was not induced in the other 11 patients (group II). The average MCR of group I (66±18%) was significantly higher than group II (28±9%, P<0.01) and the control group (p<0.01), though lower than VSA (p<0.05). These findings suggest that increased coronary vasoreactivity may play an important role in the chest pain development in CPS.
(Internal Medicine 31 : 22-27, 1992)

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The gap between the tips of the anterior and posterior mitral leaflets was studied to assess the significance of this gap in the diagnosis of mitral valve prolapse. The subjects were 39 patients in whom the gap was seen and the mitral valve did not exceed the mitral annular line in systole on two-dimensional echocardiography. Forty eight healthy subjects, in whom phonocardiography disclosed no abnormalities, served as controls. The site of the gap as well as the site and severity of mitral regurgitation were assessed with two-dimensional echocardiography and Doppler flow imaging. The incidence of mitral regurgitation was 82%, which was comparable to that in the controls (67%). The site of the gap was consistent with that of regurgitation. The gap was also seen in a low percentage of healthy subjects, but clinically significant mitral regurgitation did not accompany the gap in healthy subjects including the gapcarrying controls. On the other hand, many of the gap-carrying patients showed clinically significant mitral regurgitation. Also the fact that a gap between the tip of the anterior and posterior mitral leaflets was found to be frequently accompanied by phonocardiographical features corresponding to mitral valve prolapse indicates that the presence of a gap is a significant finding.
(Internal Medicine 31 : 28-32, 1992)

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Forty Japanese patients with alcoholic liver disease (nonspecific change, 9 ; fatty liver, 5 ; hepatic fibrosis, 4 ; chronic hepatitis, 12 ; alcoholic hepatitis, 5 ; liver cirrhosis, 5) were followed for 3-17 yr (average 8 yr) with repeated liver biopsies (2-5 times ; average 2.5 times) at intervals of more than 3 yr. All of the patients continued to consume alcohol during this observation period. Five out of 12 patients with chronic hepatitis and 2 of 5 patients with alcoholic hepatitis eventually progressed to cirrhosis, while none of the 4 patients with hepatic fibrosis became cirrhotic. Anti-hepatitis C virus antibody was positive in 2 patients with liver cirrhosis among 12 patients whose sera were available. Two patients with cirrhosis died of hepatic failure and one patient died of hepatocellular carcinoma. These data suggest that the long-term prognosis of alcoholic liver disease is not necessarily poor, but patients with chronic hepatitis or alcoholic hepatitis can be at risk of progression to cirrhosis.
(Internal Medicine 31 : 33-38, 1992)

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The patient, an 18-yr-old male (admission ht 153 cm, wt 30 kg), had been suffering from growth arrest and intermittent abdominal pain since he was 13yr old, which was left untreated. Examinations on admission disclosed almost normal pituitary function, while levels of testosterone and somatomedin C were low. Roentgenological examination revealed extensive skipstenotic lesions and longitudinal ulcers in the ileum, diagnostic of Crohn's disease. Therapy involving high-caloric parenteral and enteral alimentation resulted in a marked increase in both ht and wt, and improvement in roentgenological and colonoscopical findings. The interrelation between Crohn's disease and malnutrition with reference to some reports in the literature is discussed.
(Internal Medicine 31 : 39-43, 1992)

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This is a report of a case of glomerular lipidosis with familial combined hyperlipidemia and panhypopituitarism. A 60-yr-old woman was admitted for evaluation of hyponatremia. Administration of hydrocortisone normalized the level of serum Na. A pituitary hormone-stimulating test and brain computed tomography revealed panhypopituitarism with an empty sella. Glucocorticoid deficiency due to secondary hypoaldosteronism was thought to have caused the Na loss. She had been treated for thyroid dysfunction and hyperlipidemia with replacement of thyroid hormone and a lipid-lowering drug. Hyperlipidemia changed from type V into II_a in 4 yr. Furthermore, one of her brothers and one of her sons were suspected to have type IV hyperlipidemia. Familial combined hyperlipidemia accompanied by secondary hypothyroidism was thought to have increased the levels of both total cholesterol and triglyceride. Two renal biopsies in 3 yr showed lipid deposits in the mesangial cells and indicated a positive correlation between the levels of serum lipids and lipid deposits in glomeruli, which suggested an important role of abnormal lipid metabolism in the progression of glomerular lipidosis.
(Internal Medicine 31 : 44-49, 1992)

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A 57-yr-old woman developed Crow-Fukase syndrome 18 yr after resection of plasmacytoma of the rib. Irradiation applied to the relapsed plasmacytoma and systemic chemotherapy alleviated symptoms and signs, but the tumor relapsed in the unirradiated cervical lymph node and she died of Pseudomonas pneumonia during chemotherapy 3 yr after diagnosis. Biopsy of the lymph node revealed proliferation of lgG-λ-positive atypical plasma cells while autopsy revealed plasmacytoma remnant in the pleura of the affected side 21 yr before. No amyloid was found on autopsy. Crow-Fukase syndrome can develop long after the origination of plasmacytoma.
(Internal Medicine 31 : 50-54, 1992)

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A 35-yr-old woman, who suffered from relapsing coma with hyperammonemia for 17 yr, was diagnosed to have lysinuric protein intolerance (LPI). Increased urinary dibasic amino acids (lysine, arginine and ornithine) and impaired absorption of orally administered lysine and arginine proved the defects of renal tubular and intestinal transport of dibasic amino acids. These defects are the primary cause of impaired urea cycle metabolism in LPI. Further, the level of argininosuccinate synthetase (ASS), a urea cycle enzyme, was analyzed and it was found to be below the normal level. This is the second reported case of LPI presenting ASS deficiency.
(Internal Medicine 31 : 55-59, 1992)

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A case of lung cancer with hyperamylasemia was studied. Small cell carcinoma was diagnosed histologically. The salivary gland and pancreas had no clinical involvement in the hyperamylasemia. Saliva-type amylase was dominant as observed from amylase isozyme patterns in the serum and tumorous tissue. Levels of amylase were higher in tumorous tissue than in normal lung tissue. Immunohistochemical study showed amylase localized in tumor cells. Observation of the ultrastructure revealed electron-dense granules in the cytoplasm of the tumor cells. Findings suggested that amylase was being produced by the lung cancer in this case.
(Internal Medicine 31 : 60-63, 1992)

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Two cases of Hashimoto's thyroiditis are presented : a woman who suffered twice from transient hypothyroidism (Case 1), and a woman with polycystic ovary syndrome who had transient hypothyroidism which was inferred to have been caused by exacerbation of Hashimoto's disease (Case 2). In both cases, fluctuation in titers of both anti-thyroglobulin (TGHA) and antimicrosomal antibodies (MCHA)was observed. Although an increased serum thyroid stimulating hormone (TSH) concentration in Case 2 was associated with the increased titer of MCHA, this was not true of Case 1. Measurement of serum iodine concentration in Case 1 disclosed no correlation between serum TSH and iodine concentrations, suggesting that episodes of hypothyroidism in this patient are not due to iodine-induced hypothyroidism. The transient hypothyroidism in Case 2 was considered to be due to fluctuations in immune mechanism (s), but the reason in Case 1 was not clear in the present study.
(Internal Medicine 31 : 64-68, 1992)

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A patient with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who had severe jaundice (total bilirubin 29.1 mg/dl) and rapidly progressive liver damage is reported. The liver damage findings matched the criteria of autoimmune chronic active hepatitis (CAH). There have been no prior roports of a case of CREST syndrome with autoimmune CAH in Japan. Anticentromere antibody (ACA) was detected in the serum ; ACA seemed to be related to the pathogenesis of these two diseases.
(Internal Medicine 31 : 69-73, 1992)

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A 34-yr-old woman with mixed connective tissue disease (MCTD) who died of heart failure due to pulmonary hypertension (PH) is reported. She showed various symptoms and signs over the previous 8 yr, including lymphadenopathy, Raynaud's phenomenon, polyarthralgia, sclerodactylia, esophageal disturbance, PH, Sjören's syndrome and renal tubular acidosis. Autopsy findings revealed severe intimal proliferation with marked luminal narrowing of the small arteries in the lung. Echocardiographic and mechanocardiographic methods were very useful for assessment of the degree of right ventricular dysfunction associated with PH on the early stage of MCTD.
(Internal Medicine 31 : 74-77, 1992)

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A 40-yr-old woman with alveolar soft-part sarcoma and multiple hypervascular cardiac tumors involving both left and right ventricles is reported. Papillary muscle dysfunction and mitral valve prolapse with mitral regurgitation were caused by the largest tumor in the left ventricle.
(Internal Medicine 31 : 78-81, 1992)

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A rare case of retroperitoneal ganglioneuroma incidentally found during an annual health examination is presented. The patient was an asymptomatic female, age 26. Physical examination and routine laboratory studies gave normal results. The plasma concentrations and 24-h urinary excretion of catecholamine and its metabolites were within the normal range. The patient was diagnosed as having a left supra-renal mass (6.3×3.6 cm) by abdominal ultrasonography, although by image diagnosis the paraadrenal mass could not be discriminated from an adrenal tumor. Operation was performed under the suspicion of a nonfunctioning adrenal tumor. The tumor removed was 8×6×4 cm, 98 g and was encapsulated. Histopathological diagnosis of retroperitoneal ganglioneuroma was confirmed. Forty-one cases of retroperitoneal ganglioneuroma in the Japanese literature including the present case are reviewed.
(Internal Medicine 31 : 82-85, 1992)

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We report a case of malignant lymphoma presenting as the superior vena cava syndrome. This case is very interesting since both adenocarcinoma mucocellulare scirrhosum, Borrman type IV, and lymphoma were present in the stomach, forming colliding neoplasms. Rather than systemically disseminated, the lymphoma cells were localized in the stomach, the intestine, a few perigastric lymph nodes and in a mediastinal mass, which may have constituted a neoplastic thymus invaded by lymphoma cells from the stomach. The unique occurrence of the histological combination as gastric colliding neoplasms is discussed.
(Internal Medicine 31 : 86-90, 1992)

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The sudden death of a diabetic patient (NIDDM) during Holter monitoring is described. The patient was a 64-yr-old male with an 11-yr history of diabetic nephropathy, retinopathy, autonomic nervous dysfunction, and old myocardial infarction. In spite of all these complications, he remained asymptomatic and eventually resumed his normal activities, including a daily 1-h walk, until his sudden death. The cause of death was considered to be cardiac disease, but was not confirmed by autopsy. A detailed analysis of his Holter monitoring is given.
(Internal Medicine 31 : 91-93, 1992)

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Hereditary angioedema (HAE) is known as a deficiency state of C1 inhibitor (C1 INH), an important protease inhibitor protein involved in the complement system. As with other components of the classical pathway of the complement system, a state of its deficiency often causes clinical immunoregulatory disorders. A 45-yr-old brother and a 63-yr-old sister with HAE both developed chronic renal failure, probably due to chronic glomerulonephritis, and required regular hemodialysis. This is, to our knowledge, the first report of sibling cases of HAE associated with chronic renal failure.
(Internal Medicine 31: 94-97, 1992)

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We report a 57-yr-old woman with severe cardiac failure secondary to postnephrectomy arteriovenous fistula which was successfully removed. A review of the literature shows the rarity of this complication ; only 72 cases have been reported in the world literature including 6 cases (including the present case) from Japan. Surgical intervention is generally the treatment of choice from a fistula and provides satisfactory results for cardiac failure which is the major complication of this fistula. A characteristic bruit was observed in all reported cases. Therefore, nonsurgical closure by percutaneous embolization for a small sized shunt may be possible if early diagnosis is obtained by careful auscultation of the loin.
(Internal Medicine 31 : 98-101, 1992)

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A 75-yr-old man was admitted because of acute renal failure. On the 9th hospital day, pulmonary hemorrhage occurred. He was treated with a bolus dose of methylprednisolone and plasma exchange, but died from respiratory failure on the 16th hospital day. Autopsy findings demonstrated marked intraalveolar hemorrhage and crescentic glomerulonephritis. Linear depositions of IgG along both alveolar and glomerular basement membranes (GBM) were shown by direct immunofluorescent studies. Circulating anti-GBM antibodies were demonstrated by indirect immunofluorescent techniques. This is a confirmatory case of Goodpasture's syndrome, which is rare in Japan. A review of the Japanese literature revealed some characteristics of Japanese cases.
(Internal Medicine 31 : 102-107, 1992)

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We report a 52-yr-old man with ulcerative colitis who developed sulfasalazine-induced pulmonary infiltration with eosinophilia (PIE syndrome), which resolved completely after withdrawal of this drug. Desensitization to sulfasalazine was successful, and allowed the patient to receive this drug without recurrence of the pulmonary toxicity. This is the first case of the sulfasalazine-induced PIE syndrome in Japan ; a review of the world literature found no previous cases of successful desensitization following sulfasalazine-induced PIE syndrome.
(Internal Medicine 31 : 108-113, 1992)

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A 70-yr-old woman was admitted to our hospital with duodenal ulcer and anemia. The result of liver function test was abnormal and showed persistent elevated alkaline phosphatase levels. Thus, after recovery from duodenal ulcer, endoscopic retrograde cholangiopancreatography (ERCP) was performed ; the characteristic "beaded" appearance with band-like strictures and saccular outpouchings affecting the intrahepatic biliary system were found. The diagnosis of primary intrahepatic sclerosing cholangitis (PISC) was made on the basis of the generally accepted diagnostic criteria of primary sclerosing cholangitis (PSC). However, the histological finding from a liver biopsy specimen revealed highly atypical epithelial proliferation of bile ducts. This case of PISC complicated with atypical biliary glandular changes is described, and the distinction between PISC and carcinoma of the bile duct is discussed.
(Internal Medicine 31 : 114-121, 1992)

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A case of transiently increased wall thickness in the left ventricular apex after stunned myocardiumdue to severe attack of vasospastic angina is described. "Ace of spades" configuration, documented by left ventriculogram and increased apical wall thickness of the left ventricle, as revealed by two-dimensional echocardiograms returned to normal in 2 months. This transiently increased left ventricular mass may have been due to myocardial edema, which is occasionally seen in the recovery course of active myocarditis.
(Internal Medicine 31 : 122-124, 1992)

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A patient with post-gastrectomy megaloblastic anemia is described. Initially, the CD4/CD8 ratio of blood cells was abnormally high and the level of natural killer cell activity was markedly low. These abnormalities were, however, restored by vitamin B12 treatment. Taken together with our prior report on a patient with pernicious anemia in whom the decreased level of CD8-positive blood cells was corrected by vitamin B12 therapy, it may be considered that the reversible CD4/CD8 imbalance of blood cells noted in some cases of pernicious anemia is not pathognomonic for the disease but secondary to vitamin B12 deficiency.
(Internal Medicine 31 : 125-126, 1992)

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A 68-yr-old male with severe psoriasis developed megaloblastic anemia due to folate deficiency 3 months after the cessation of low-dose methotrexate therapy. The mechanism of megaloblastic anemia in this case was suggested to be multifactorial. The case report and a review of magaloblastic anemia associated with psoriasis are presented.
(Internal Medicine 31 : 127-130, 1992)

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A 32-yr-old Japanese female who was suffering from Turner's syndrome associated with Hashimoto's thyroiditis and sarcoidosis is reported. It is known that Hashimoto's thyroiditis is not infrequently complicated with Turner's syndrome on the basis of an abnormal X chromosome. In this case, sarcoidosis occurred 4 months after Hashimoto's thyroiditis. This extremely interesting coincidence of Hashimoto's thyroiditis and sarcoidosis in Turner's syndrome has not been reported to date to our knowledge.
(Internal Medicine 31 : 131-133, 1992)

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In a 39-yr-old female patient with Bartter's syndrome, vascular responsiveness to angiotensin II and phenylephrine was studied. Pressor responses to angiotensin II and to phenylephrine were markedly decreased prior to treatment and were improved by administration of indomethacin, dextran, KCI, captopril, propranolol or pindolol. Moreover, the responses of total peripheral vascular resistence to angiotensin II and phenylephrine were markedly decreased before treatment and were improved by the treatment. A relatively low fractional distal chloride reabsorption was found, and that was not changed after the administration of KCI or indomethacin. The results of this patient were compatible with the primary cause of Bartter's syndrome, defective chloride reabsorption at the loop of Henle, but the possibility of an abnormality in the vascular wall could not be denied.
(Internal Medicine 31 : 134-139, 1992)

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A case of allergic bronchopulmonary aspergillosis (ABPA) is described which presents left upper lobe collapse caused by Aspergillus terreus, an organism rarely found in ABPA. It is unusual for initial radiographic findings of ABPA to include extensive collapse.
(Internal Medicine 31 : 140-142, 1992)

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A 56-yr-old man with hypogonadism, gynecomastia, and mental retardation was evaluated for chromosome constitution and thrombocytopenia. Chromosomal analysis demonstrated the mosaicism of 48, XXYY and 47, XXY in the peripheral lymphocytes. Twenty out of twenty-five cells were 48, XXYY karyotype and the remaining five were 47, XXY karyotype. Thrombocytopenia was the EDTA-dependent pseudothrombocytopenia type 1 (platelet agglutination). Serological examination suggests that the platelet agglutinin belongs to IgM-kappa type. The present case exhibited both EDTA-dependent pseudothrombocytopenia and the 48, XXYY syndrome. Although this combination may have occurred purely by chance, the possibility of whether or not the mosaicism of lymphocytes produces platelet agglutinin remains to be clarified.
(Internal Medicine 31 : 143-146, 1992)

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