Vascular endothelial cells play an important role in modulating cardiovascular function, by liberating several vasoactive substances, such as prostacyclin, endothelium-derived relaxing factor/nitric oxide, a hyperpolarizing substance, and vasoconstrictor substances. Their roles in the cardiovascular system include modulation of the contraction of the underlying vascular smooth muscle, interaction between blood components and blood vessel wall, vascular growth, as well as regulation of the cardiovascular system at several levels beyond the vascular wall. Alterations in the endothelial functions and the related processes are now known to be involved in the pathogenesis of many cardiovascular diseases, including hypertension and atherosclerosis. Thus, the impaired endothelial regulation of the cardiovascular system may have an important implication for the development and progression of many cardiovascular diseases.
(Internal Medicine 34: 939-946, 1995)

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Adult T-cell leukemia (ATL) was first reported in Japan, where it has a high incidence in the southwestern region. The retrovirus, human T-lymphotropic virus type I (HTLV-I), is the causative agent of ATL. In ATL-endemic areas, the rate of HTLV-I carriers is high. A definite diagnosis of ATL is based on the presence of HTLV-I proviral DNA in the tumor cell DNA. ATL cells originate from the CD4 subset of peripheral T cells. ATL shows diverse clinical features but can be divided into four subtypes: acute, chronic, smoldering, and lymphoma type. Chemotherapy is not effective; the acute and lymphoma types have a poor prognosis. Familial occurrence of ATL is common. HTLV-I infection is caused by transmission of live infected lymphocytes from mother to child, from man to woman, or by blood transfusion. Infection with HTLV-I can lead to other diseases, including HTLV-I-associated myelopathy/tropical spastic paraparesis and HTLV-I uveitis.
(Internal Medicine 34: 947-952 1995)

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Left ventricular function and morphology were assessed using M-mode echocardiography in 3 patients with diabetes mellitus associated with mitochondrial tRNA^Leu(UUR) gene mutation, who were free of clinical, electrocardiographic, or thallium scan evidence of ischemic heart disease. Echocardiograms revealed hypertrophic cardiomyopathy in all 3 patients. Hypertrophy of the interventricular septum was mild in Cases 1 and 3 (12 and 13 mm, respectively) and severe in Case 2 (22 mm) (normal 7-10 mm). When they had neither signs nor symptoms suggestive of congestive heart failure, percentage fractional shortening (%FS), an index of wall motion of the left ventricle (normal >28%), was normal in Cases 2 and 3 (28 and 32%, respectively) whereas it was slightly decreased in Case 1 (22%). In Case 1 with mild hypertrophy, the development of congestive heart failure was associated with a marked decrease in %FS to 13%; this patient responded well to diuretics and captopril and %FS rose to 22%. However, a mild decrease in %FS to 21% caused congestive heart failure in Case 2 with severe hypertrophy. His response to treatment was marginal. The present study indicates that mitochondrial DNA analysis should be done in patients with diabetic cardiomyopathy, and that sequential echocardiography is invaluable for the detection of hypertrophic cardiomyopathy and the management of subsequent myocardial dysfunction in patients with mitochondrial diabetes mellitus and cardiomyopathy.
(Internal Medicine 34: 953-958, 1995)

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To investigate the influence of thoracic irradiation on immunological competence in patients with lung cancer, we examined the changes in peripheral blood lymphocyte subsets in 15 patients before and after radiation therapy by two-color flow cytometry techniques. After radiation therapy, the percentage and the absolute number of CD4+CD45RA+ cells (naive T cells) and CD56+ and/or CD16+ cells (NK cells) decreased. The percentage of CD4+human leukocyte antigen-DR(HLA-DR)+ cells (activated CD4T cells) and CD8+HLA-DR+ cells (activated CD8T cells) increased, although the absolute number did not change significantly. Naive T cells may be more selectively damaged than memory T cells by thoracic irradiation, through their recirculation behavior. The reduction of natural killer (NK) cells is disadvantageous for anti-tumor immunity. The percentage of HLA-DR positive T lymphocytes was significantly increased, and thus the possibility of HLA-DR enhancement by irradiation cannot be excluded. Therefore, thoracic irradiation has numerous varied effects on the immunological system of lung cancer patients.
(Internal Medicine 34: 959-965, 1995)

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Although the prevalence of serum precipitating antibodies for farmer's lung disease (FLD) is lower in smokers than in nonsmokers and FLD predominates in nonsmokers, the affects of smoking on the clinical course of the disease is not known. We compared the clinical findings and the prognosis between 12 smokers (SM-FLD) and 31 non-smokers with FLD (NS-FLD). There was no difference in age, sex, working years on farm, clinical symptoms, laboratory findings, radiographic findings, between the two groups. However, for the type of onset on the first visit for FLD, "acute single episode" type was less common, and "recurrent" and "insidious onset" types were more common in SM-FLD than in NS-FLD (8.3 vs 58.1, 91.7 vs 41.9%, respectively, p<0.05). Although working status and mask wearing status were not significantly different between the two groups after the diagnosis of FLD, patients with symptoms and/or radiographic abnormalities of FLD of more than 6 months were found more frequently in SM-FLD than in NS-FLD (66.7 vs 19.4%, p<0.005). And also SM-FLD had more recurrences of FLD than NS-FLD after the initial diagnosis of FLD (1.58±1.56 vs 0.471±1.07, p<0.05). SM-FLD tended to have lower %VC than NS-FLD (73.6±7.4 vs 88.5±3.9%, respectively, p=0.06). Regarding the prognosis, the 10-year survival rates were 70.7% in SM-FLD, and 91.5% in NS-FLD (p<0.05). These results suggest that smoking may make FLD insidious and chronic, and deteriorates the clinical outcome.
(Internal Medicine 34: 966-971, 1995)

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Autonomic functions were studied in 10 respiratory-support patients with motor neuron disease (MND) at its most advanced stage by means of hemodynamic function tests. The mean duration of disease was 83.4 months (SD 28.0, range 50-140 months). AH the patients in this study had lost their ability to breathe spontaneously and were ventilated by respirator. Hemodynamic function tests were performed in 8 out of 10 patients. These patients showed exaggerated sympathetic vasomotor reflexes, increased reflex hypertension and tachycardia in response to the cold pressor test and increased response to administration of adrenaline. However, the responses to both the Aschner eyeball pressure test and injection of atropine were normal, indicating normal parasympathetic nervous system function. Plasma and urine levels of norepinephrine, epinephrine and their metabolites were almost normal in all 10 patients, as were serum dopamine-β-hydroxylase levels. In patients with the most advanced stage of MND, hyperfunction of the adrenergic sympathetic nervous system was observed, and this was considered to be a symptom characteristic of MND.
(Internal Medicine 34: 972-975, 1995)

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The purpose of this study was to diagnose the initial stage of acute pulmonary infections due to Pneumocystis carinii (P. carinii), Mycobacterium tuberculosis (M. tuberculosis), and cytomegalovirus (CMV) by polymerase chain reaction (PCR) combined with the induced sputum method in hemophilia patients infected with human immunodeficiency virus (HIV). Induced sputum and PCR were obtained at each outpatient visit or when any respiratory symptoms were present during a one-year period. All patients with a CD4+ count of less than 200×10⁶/l were given prophylactics against P. carinii pneumonia (PCP). PCR was conducted 111 times in 29 patients. M. tuberculosis DNA was detected in 1 patient and CMV DNA in 4 patients. P. carinii DNA was not detected. PCR on induced sputum samples is sensitive for the rapid detection of the initial stage of respiratory events. PCP prophylaxis changes the clinical manifestations of HIV infection.
(Internal Medicine 34: 976-981, 1995)

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We retrospectively evaluated the morphologic change of 28 Follow up biopsies from 24 cases of lupus nephritis according to the classification of the World Health Organization and determined the activity index (AI) and the chronicity index (CI). In the cases with biopsies repeated within 6 months, the AI decreased significantly from 6.7±1.3 to 3.5±0.8, while the CI showed no significant change. In cases which were rebiopsied after longer intervals, AI increased significantly from 3.2±0.7 to 7.5±1.2; the CI did not change significantly. When AI and CI changes in the cases biopsied again beyond 6 months were compared with respect to therapy, AI showed no significant difference in the methylprednisolone pulse therapy group but was significantly increased in the oral steroid therapy group. The CI tended to be increased in both groups, but not significantly. Steroid pulse therapy was effective in improving active lesions with a high AI. Steroid therapy for lupus nephritis prevented short-term progression of glomerulosclerosis and did not accelerate glomerulosclerosis.
(Internal Medicine 34: 982-987, 1995)

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A 73-year-old woman was diagnosed as primary hyperparathyroidism. Hypercalcemia recurred after parathyroidectomy, though serum calcium concentration temporarily resumed to normal. Hypercalcemia was eventually shown to be due to multiple myeloma. In spite of the frequent association of hypercalcemia with malignant disease and the relative frequency of primary hyperparathyroidism as a cause of hypercalcemia, primary hyperparathyroidism accompanied by multiple myeloma has been considered in only a limited number of reports. Here, the cases in the literature are reviewed and discussed.
(Internal Medicine 34: 988-991, 1995)

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A 46-year-old woman presented with sudden severe pain in the interscapular region. Physical examination, including detailed neurological evaluation, did not disclose any abnormalities. However, magnetic resonance imaging revealed an epidural hematoma anterior to the thoracic spinal cord and its spontaneous resolution thirty days after onset. Her hospital course was uneventful. To our knowledge, this report documents the first case of a spontaneous spinal spidural hematoma without neurological deficits. Spinal epidural hematoma may be more common than previously thought because some cases have probably been misdiagnosed as transient back pain of unknown etiology.
(Internal Medicine 34: 992-994, 1995)

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We report a 29-year-old male hemophiliac with human immunodeficiency virus (HIV)-1-associated dementia complex, who died 2.5 months after the onset of dementia. The patient's cognitive abnormalities including forgetfulness, loss of concentration and slowing of thought appeared about 7 years after HIV infection. His neurological symptoms were characterized as progressive dementia, episodic consciousness loss, transverse myelopathy and peripheral neuropathy. He had generalized slow waves in electroencephalogram (EEG), progressive cerebral atrophy and a diffuse high intensity lesion in the white matter as shown by T2-weighted brain magnetic resonance imaging (MRI). We emphasize the significance of neurological complications, especially acute progressive dementia, in Japanese patients with acquired immunodeficiency syndrome (AIDS).
(Internal Medicine 34: 995-999, 1995)

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A 58-year-old woman with kidney-limited recurrence of microscopic polyarteritis (MPA) is described. The patient had a history of histologically-confirmed MPA 7 years previously, which had been in remission with corticosteroid treatment for 30 months followed by no medication thereafter. However, in February 1994, clinical manifestations including leg edema and proteinuria developed, followed by rapidly progressive renal insufficiency. Renal biopsy revealed crescentic glomerulonephritis with necrotizing vasculitis. Furthermore, at the same time antimyeloperoxidase antibody (MPO-ANCA) was detected in plasma. She was diagnosed as having kidney-limited recurrence of MPA without systemic presentation. Corticosteroid therapy was reinstituted, and the renal function improved, with a decrease in the titer of MPO-ANCA.
(Internal Medicine 34: 1000-1004, 1995)

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A 50-year-old man with Churg-Strauss syndrome showed granulocytes (GNLs) which generated more superoxide anion (O₂^-) than GNLs from healthy subjects following in vitro stimulation with C5a or C3a. Production of O₂^- subsided as the clinical symptoms improved with steroid treatment. A hyperresponsiveness of GNLs may be involved in this disorder.
(Internal Medicine 34: 1005-1008, 1995)

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We report two Guillain-Barré Syndrome (GBS) patients with culture-proven Campylobacter jejuni (Penner19/Lior 7). Elevated anti-C.jejuni IgG antibody declined to the normal range within one month, elevation of anti-C.jejuni IgM antibody lasted for 3 months and the liters of anti-C.jejuni IgG antibody were still elevated at one year after the neurologic onset. Serological criteria for antecedent C.jejuni infection in GBS patients should require 1) high titer of serum IgA and/or IgM antibody against C.jejuni followed by seroconversion of the IgA and/or IgM and 2) a 4-fold decline of IgG serum dilution against C.jejuni. Anti-C.jejuni antibodies in cerebrospinal fluid (CSF) reached the maximum value at one month after their neurologic onset which may not be responsible for the main pathological mechanism in the development of GBS. Anti-GM1 antibodies in serum and CSF were parallel with those of anti-C.jejuni antibodies during the course of the disease.
(Internal Medicine 34: 1009-1014, 1995)

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The clinical profiles of 11 Japanese patients with recurrent episodes of Guillain-Barré syndrome (GBS), including 3 of our cases out of a total of 76 patients (48 males and 28 females), are reviewed. The recurrence rate was estimated as 4 %. Among the 11 Japanese patients, 10 were male. Various viral infections had been confirmed in 1 case. No patients showed recurrent ophthalmoplegia. Sensory involvement varied from one episode to the next. These findings suggest the changing distribution of the demyelinating lesions and possibly a different reaction to the unknown pathogens in the recurrence of GBS.
(Internal Medicine 34: 1015-1018, 1995)

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A 60-year-old woman was admitted to our hospital with shortness of breath and was diagnosed to have pulmonary arteriovenous fistula with unequal branching of the pulmonary artery system, as assessed by pulmonary angiography. The concomitant occurrence has not been reported previously. Although the etiology of these abnormalities is unknown, these abnormalities are considered to have occurred during an early stage of fetal development. The analysis of a concomitant occurrence of these abnormalities may be useful in elucidating the etiology of pulmonary arteriovenous fistula.
(Internal Medicine 34: 1019-1022, 1995)

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A 65-year-old man was hospitalized with IgG κ-type multiple myeloma (MM) and enormous subcutaneous plasmacytomas. Two different combination chemotherapy regimens (MMCP and AVPP) and α-interferon therapy were ineffectual. Oral administration of etoposide at 50 mg/day was subsequently started, the tumors completely disappeared after 5 months. The blood level of monoclonal protein became undetectable after 8 months of continuous treatment. The side effect noted was loss of hair. The course in this patient suggests that long-term daily low-dose administration of etoposide should be attempted in patients with refractory MM and extramedullary plasmacytoma.
(Internal Medicine 34: 1023-1026, 1995)

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A 74-year-old man developed proximal muscular weakness and wasting of the left upper extremity without sensory disturbance or myelopathic symptoms. The muscle atrophy had not progressed for a few years. Radiological examination of the spine showed cervical disc herniation. These findings and electrophysiological studies excluded motor neuron diseases, permitting the diagnosis of dissociated motor loss syndrome. Interestingly, delayed computerized tomographic myelography disclosed cavities in the anterior horns of the spinal cord, which coincided with the clinical symptoms. Previous radiological and pathological examinations showed formation of such cavities within the spinal cord resulting from chronic compression, which was followed by ischemic change. In this context, the present case supports ischemia as a cause of dissociated motor loss syndrome.
(Internal Medicine 34: 1027-1029, 1995)

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We report a systemic lupus erythematosus patient with lupus anticoagulant accompanied by pulmonary thromboembolic hypertension possibly due to large vegetation of the tricuspid valve of the heart. Histopathological analysis of localization and distribution of thromboemboli in pulmonary vasculature revealed that the organized thromboembolic occlusion of multiple blood vessels (99 out of 222 arteries), might be responsible for the pulmonary hypertension both in quality and quantity. The contribution of lupus anticoagulant to the pathogenesis of Libman-Sacks endocarditis in systemic lupus erythematosus, and its possible relationship to pulmonary thromboembolic hypertension are discussed.
(Internal Medicine 34: 1030-1034, 1995)

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We report CD34-positive "blastema" which developed in a patient with idiopathic myelofibrosis (IMF). A 48-year-old woman was diagnosed as having essential thrombocythemia, then the disease had transformed into IMF 41 months later. Finally, a subcutaneous tumor, composed of blastic cells positive for CD34 but negative for lineage-specific antigens, developed without any evidence of systemic leukemia transformation.
(Internal Medicine 34: 1035-1037, 1995)

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Gastric tumors with calcification have been considered to be relatively rare. We treated 8 cases of tumors involving calcification, 4 cases of gastric carcinomas and 4 other cases of submucosal tumors with ulceration. These cases are reported along with a review of the cases reported in Japan.
(Internal Medicine 34:1038-1042, 1995)

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