To determine the changes in bone metabolism in response to combined chemotherapy in patients with bone metastases (BM), we examined osteocalcin (BGP), alkalinephosphatase (ALP), hydroxyproline (HYP), pyridinoline (PYR), and/or deoxypyridinoline (D-PYR) in 25 cancer patients. In patients without BM, serum BGP was normal and not affected by chemotherapy. In patients with BM, however, BGP was often abnormally high or low, and some patients reacted to chemotherapy with a BGP increase at 4 weeks after initiation of therapy. Such an increase was observed in the group of patients who responded favorably to therapy as judged by a decrease in bone pain and tumor-associated biochemical markers. Urine HYP, PYR, and D-PYR were high in patients with BM before therapy; D-PYR decreased transiently at 2 weeks and increased thereafter. We assume that increased bone-resorption markers along with increased bone formation markers after therapy would indicate recovery of coupled bone metabolism, as the deranged bone remodeling is improved by tumor-regression. This study suggests that BGP and D-PYR can be useful early markers to predict favorable bone response to chemotherapy in patients with BM.
(Internal Medicine 32: 611-618, 1993)

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The effects of spa bathing on blood coagulation and fibrino lysis were studied in 20 patients with chronic cerebral infarction. Blood was obtained before and after a 10-minute period of spa bathing at 41°C. Prothrombin time, activated partial thromboplastin time, fibrinogen, factor VIII activity, von Willebrand factor activity, and antithrombin III activity did not show significant changes after bathing, but euglobulin lysis time was significantly reduced (p<0.01) and fibrin lysis activity was increased (p<0.05). These findings suggest that spa bathing activates fibrinolysis without markedly changing blood coagulation in patients with chronic cerebral infarction. It is thought that the activation of fibrinolysis without the activation of coagulation has a favorable effect on blood circulation. The results of fibrin-plate assays using C1 inactivator indicated that tissue-type plasminogen activator was the major contributor to the activation of fibrinolysis during spa
bathing.
(Internal Medicine 32: 619-622, 1993)

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The health effects of human T-lymphotropic virus type I (HTLV-I) were studied in HTLV-I endemic areas in Japan during the period from September 1990 to October 1991. The study subjects were 2, 847 men living in the southern part of Kyushu and engaged in light work. Ages ranged from 19 to 79 years (47.8±7.2; mean±standard deviation). The prevalence of each disturbance was analyzed with a logistic model. The disturbances in which the revalence showed a significant association with HTLV-I were old tuberculosis, ulcerative colitis and ossification of the posterior longitudinal ligament of the cervical spine (OPLL). The results of the present study suggest that HTLV-I may be a risk factor for these diseases and may indicate the necessity for a synthetic study on HTLV-I.
(Internal Medicine 32: 623-628, 1993)

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We determined whether, in 15 diabetic patients, a conventional low-protein diet containing a high proportion of mono- and disaccharides would lead to a deterioration of postprandial glucose metabolism. Three different test meals were given on 3 different days. We found that a high proportion of simple carbohydrates, when consumed as a low-protein meal, aggravated the postprandial hyperglycemia in diabetic patients. The substitution of complex carbohydrates for simple sugars in the meal suppressed postprandial hyperglycemia in diabetic patients.
(Internal Medicine 32: 629-632, 1993)

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We herein describe a 41-year-old female patient with an association of myasthenia gravis (MG) with anti-acetylcholine receptor (AcR) antibody, mixed connective tissue disease (MCTD) and Sjögren's syndrome (SjS). We reviewed the reported association of MG and MCTD, systemic lupus erythematosus, progressive systemic sclerosis, polymyositis and dermatomyositis, and SjS. Since we could find only two patients who fulfilled the diagnostic criteria for MCTD in the previous literature, we concluded that the association of MG and MCTD is rare. We also discuss the coexistence of SjS as one of the underlying pathological conditions for the association of MG and various connective tissue diseases including MCTD.
(Internal Medicine 32: 633-637, 1993)

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A 17-year-old Japanese woman with rigid spine syndrome (RSS) presented with respiratory failure leading to CO₂ narcosis. The clinical symptoms were drowsiness, asterixis and cardiac arrhythmias. Tracheostomy and temporary ventilatory support abolished these symptoms. However, polygraphic sleep studies without a ventilator revealed Cheyne-Stokes respiration and profound arterial oxygen desaturation during rapid eye movement sleep. Nocturnal ventilator support improved not only nocturnal hypoxemia, but daytime blood gas values during spontaneous breathing. These findings indicate that the onset of respiratory failure is preceded by severe nocturnal hypoxemia and that the evaluation and control of nocturnal respiratory insufficiency is essential for RSS patients.
(Internal Medicine 32: 638-640, 1993)

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We report a 38-year-old woman with a mild form of hyperglycinemia complicated with optic nerve atrophy and convulsion. She was normal at birth and showed normal mental and physical development. After the age of 13, her visual acuity rapidly decreased. At the age of 33, she had numerous episodes of tonic seizures lasting for 1-2 minutes. She had optic atrophy, but no intellectual impairment. Glycine levels of the plasma, urine and cerebrospinal fluid were markedly increased, but the CSF/serum glycine ratio was slightly higher than the normal range. Although there is one case of retinal impairment of hyperglycinemia in the literature, this is the first report of blindness with hyperglycinemia in a 38-year-old woman.
(Internal Medicine 32: 641-642, 1993)

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A 41-year-old woman developed fatal pneumomediastinum in the course of dermatomyositis. One characteristic feature of this patient was the relatively mild myositis with slight elevation of serum creatine kinase. In a literature review, some of the patients with dermatomyositis, but not polymyositis, with a very slight elevation of serum creatine kinase tended to develop spontaneous pneumomediastinum during corticosteroid therapy. Since pneumomediastinum is a highly fatal complication in patients with dermatomyositis, only slight elevation of creatine kinase could be a marker for poor prognosis. Thus, mild/minimal myositis in patients with dermatomyositis should be carefully observed irrespective of corticosteroid therapy.
(Internal Medicine 32: 643-647, 1993)

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A 61-year-old male with acute promyelocytic leukemia (APL) had been in complete remission for the previous 15 months, but his APL relapsed with neutropenia. Although promyelocytes in bone marrow were reduced after administration of 60 mg all-trans retinoic acid (ATRA) daily, myelocytes were predominant on the myelogram and neutropenia did not recover. By adding 75 μg of granulocyte colony-stimulating factor (G-CSF) daily, neutrophils accounted for 35.0-55.5 % of the myelogram, and the peripheral neutrophil count rose dramatically. Such morphological differentiation of myeloid series was also ascertained in terms of their functions of both neutrophil alkaline phosphatase activity and active oxygen producing capacity. This case supports the concept that G-CSF accelerates ATRA-induced neutrophilic differentiation of blast cells in APL.
(Internal Medicine 32: 648-650, 1993)

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We describe acute mononeuropathy multiplex in a patient with chronic ulcerative colitis. The symptoms of neuropathy were well correlated with the disease activity of colitis. Both electrophysiological study and sural nerve biopsy revealed axonal degeneration. Mononeuropathy multiplex maybe an extraintestinal manifestation of ulcerative colitis.
(Internal Medicine 32: 651-654, 1993)

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We describe a 53-year-old woman with portal-systemic encephalopathy and altered thyroid function. Endocrinological studies revealed low levels offree thyroid hormone with an inappropriately low level of thyroid-stimulating hormone that responded to bolus injection of thyrotropinreleasing hormone with a normal but somewhat delayed pattern. On the diagnosis of hypothalamic hypothyroidism, she was treated with levothyroxine sodium. Thyroid hormone replacement improved not only the symptoms of hypothyroidism but the hyperammonemiaand consciousnessdisturbance, which suggested a hitherto undescribed possibility that hypothyroidism may be an exacerbation factor of hyperammonemia and portal-systemic encephalopathy.
(Internal Medicine 32: 655-658, 1993)

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A 48-year-old male developed acute respiratory failure owing to Legionnaires' disease (LD). Antibiotic treatment including erythromycin and riphampicin was not effective, thus transbronchial lung biopsy was performed. The histological examination of the lung showed intra-alveolar fibrosis. Corticosteroid therapy was begun and he responded well with definite clinical improvement. Bronchoalveolar lavage (BAL) was performed three times in the following year. The first BAL showed an increase of lymphocytes which then decreased significantly in the following studies. This case report thus demonstrates the importance of lung biopsy of protracted LD and theusefulness of BAL in the assessment of corticosteroid therapy.
(Internal Medicine: 32: 659-662, 1993)

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The first case of primary gastric lymphoma associated with Behcet's disease is reported. A 43- year-old woman, who had been treated for Behçet's disease for 15 years, was diagnosed as lowgrade and small lymphocytic malignant lymphomaof the stomach. Although the patient had been treated for Behcet's disease, chemotherapy was performed and a complete remission of gastric lymphomawas achieved. The literature regarding the association of malignant diseases with Behcet's disease is briefly reviewed.
(Internal Medicine 32: 663-667, 1993)

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Three cases of primary myelodysplastic syndrome (MDS) associated with myelofibrosis were initially diagnosed as refractory anemia by the presence of bicytopenia or pancytopenia and having normo- or hypercellular marrow with dysplastic features. The bone marrow aspiration of these patients showed dry tap a few months after admission, or on admission. Their bone marrow biopsy specimens revealed various grades of increased formation of reticulin fibers. One patient entered into complete remission in response to metenolone, while the other two patients died of cerebral hemorrhage several months after admission. These results indicate that this disease should be classified as a distinct subgroup of MDS.
(Internal Medicine 32: 668-671, 1993)

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A 29-year-old female was admitted because of thrombocytopenia and an abdominal mass which was palpated 5 finger breadths below the left costal margin. A computed tomographic scan of the abdomen revealed splenomegaly with a large calcified cystic lesion. The spleen was removed and the pathological examination disclosed that the cyst was monolocular and the wall was of mesothelial origin.
(Internal Medicine 32: 672-674, 1993)

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We present a case of tuberculous pericarditis that was diagnosed early by a high titer of adenosine deaminase activity in the pericardial fluid and by a strongly positive tuberculin test. Within 2 weeks of initiation of treatment, pericardial effusion gradually decreased while clinical symptoms improved markedly. Culture from sputum, gastric juice, urine, and pericardial fluid were negative for tubercle bacillus. Measurement of adenosine deaminase activity in the pericardial fluid is a supplementary diagnostic test which is as important as for tuberculous pericarditis as it is for tuberculous pleuritis, because negative Ziehl Neelsen staining and culture for tubercle bacillus are common in tuberculous pericarditis.
(Internal Medicine 32: 675-677, 1993)

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A 23-year-old man was recognized as a sporadic case of facioscapulohumeral syndrome (FSH syndrome). He was a case of early onset, and there was severe atrophy of skeletal muscles, although muscle biopsy was normal. Electromyogram revealed neurogenic changes. This patient also had severe sensorineural hearing loss and a mild abnormality of retinal vessels. It is known that some FSH dystrophy or FSH spinal muscular atrophy patients have such coincidental complications. The cause of these coincidences is still unclear, but their occurrence suggests the pleiotropy of the FSH syndrome.
(Internal Medicine 32: 678-680, 1993)

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