Mixed connective tissue disease deserves to be a distinct disease entity due to the persistent citation of this disease in the literature since the original description by Sharp in 1972, in spite of the presence of several criticisms against the independency of this disease. The characteristic features of mixed connective tissue disease are: 1) the presence of anti-U₁snRNP antibody with high titers in sera, 2) an increased frequency of HLA-DR4 in the leukocytes, and 3) death due to pulmonary hypertension.
(Internal Medicine 38: 386-393, 1999)

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Object. In hypercholesterolemic patients, acetylcholine- and substance P-mediated endothelium-dependent dilation of the coronary resistance vessels is impaired due to decreased nitric oxide production. However, it is not clear if bradykinin-induced coronary vasodilation is impaired in these patients. We investigated whether the endothelium-dependent dilation of coronary resistance vessels mediated by bradykinin is impaired in patients with hypercholesterolemia and, if so, whether this impairment is caused by a decreased production of nitric oxide. Methods. We examined the coronary vascular responses to acetylcholine and bradykinin. The vascular responses to bradykinin were also assessed after N^G-monomethyl-L-arginine was infused to inhibit nitric oxide production. Drugs were infused into the left coronary ostium and coronary blood flow (CBF) and coronary vascular resistance were evaluated by quantitative angiography and Doppler flow velocity measurements. Patients. Twelve hypercholesterolemic patients and 11 control patients with angiographically normal coronary arteries were studied. Results. The vasodilator responses to acetylcholine and bradykinin were reduced in hypercholesterolemic patients compared with control patients (p<0.005 and p<0.04, respectively, by two-way analysis of variance (ANOVA)). The CBF responses to acetylcholine and bradykinin were significantly correlated (r=0.56; p<0.01). Bradykinin-induced dilation was similar in hypercholesterolemic patients and control patients after inhibition of nitric oxide. Conclusion. These results suggest that the bradykinin-mediated endothelium-dependent dilation of coronary resistance vessels may be impaired due to depressed nitric oxide production in patients with hypercholesterolemia.
(Internal Medicine 38: 394-400, 1999)

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Object: The potential effects of branched-chain ammo acids (BCAAs) on spinocerebellar degeneration (SCD) were explored in eleven patients. Methods: The patients received 200 ml of BCAA-rich solution, 2 mg of thyrotropin-releasing hormone (TRH; protirelin), or a placebo daily for 7 days each in a random order. An SCD score was used to quantify the severity of symptoms. Patients: Eleven patients with SCD (7 male, 4 female; mean age 60±11; mean disease duration 5.5 years) participated in this study. Results: The mean SCD score of the eleven patients improved significantly by the BCAA treatment compared with the baseline. The conditions of five of the eleven patients (45%) were clearly improved by the BCAA treatment. All of the responders manifested predominantly cerebellar symptoms, but no prominent parkinsonian symptoms. Two patients with marked rigidity and akinesia did not respond to the treatment. Conclusion: We concluded that BCAAs do have a beneficial effect on functional improvement in patients with SCD, and that further large scale studies are needed.
(Internal Medicine 38: 401-406, 1999)

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This study was performed to confirm 1) the difference in the trinucleotide CAG repeat number among tissues, 2) somatic mosaicism in each tissue, 3) the correlation of the repeat number with pathological severity in Huntington's disease. The CAG repeat number was determined by analysis of the polymerase chain reaction (PCR) product in various tissues, including central nervous system (CNS) tissues and non-CNS tissues. We also determined the pathological severity grade in each brain section and compared this with the results of CAG repeat analyses. The patient was a Japanese male with Huntington's disease who died at 62 years of age. Genomic DNA was extracted from 10 parts of the central nervous system and 6 parts of other tissues from the patient. Each part of the formalin-fixed brain was subjected to gross and microscopic pathological assessment. The main peaks of CAG repeat in all tissues were 22 and 44. In analysis of somatic mosaicism, high degrees of mosaicism were obtained in the caudate nucleus, putamen and cerebral cortex, in which more severe degeneration was observed by pathological examination. These results, although this is a single case study, indicated that pathological severity did not correlate with the CAG repeat number, but it did relate to the degree of somatic mosaicism. Somatic mosaicism might reflect region-specific neuronal degeneration in Huntington's disease.
(Internal Medicine 38: 407-411, 1999)

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Object: The aim of this study was to reveal variations in the patterns of expression of the cell surface proteins in regenerating fibers and those in the number of satellite cells to gain an understanding of the pathological processes involved in sarcoglycanopathy. Methods: We have reported that there is a reduction of the beta-1 subunit of laminin, heparan sulfate proteoglycan (HSPG), and HCAM (CD44) in Japanese patients with sarcoglycanopathy. Here, we investigated immunohistocheniically the expression of the neural cell adhesion molecule (NCAM), which is a marker for human regenerating muscle and satellite cell, and CD24, which appears to be expressed in the early stages of the regeneration process. Patients: We investigated six Japanese patients with sarcoglycanopathy, and compared to age-matched Becker muscular dystrophy. Results: We found that the incidences of muscle fibers with increased NCAM were not statistically different between the two groups. However, the incidences of muscle fibers with increased CD24 and those of NCAM positive satellite cells were very low in sarcoglycanopathy and were statistically different between sarcoglycanopathy and age-matched Becker muscular dystrophies. Conclusion: The poor expression of CD24 and the fewer satellite cells in sarcoglycanopathy without significant difference in the number of total regenerating fibers suggest that a different regeneration process is involved in sarcoglycanopathy compared to that in other types of muscular dystrophy.
(Interal Medicine 38: 412-415, 1999)

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A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the CREST syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the CREST syndrome.
(Internal Medicine 38: 416-421, 1999)

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An 82-year-old male patient was admitted for liver dysfunction. Laboratory test showed the following data; aspartate aminotransferase (AST) 79 IU/ι, alanine aminotransferase (ALT) 28 IU/ι, total bilirubin (T. Bil) 0.9 U, zinc sulfate turbidity test (ZTT) 48.9 U, γ-globulin 4.9 g/dl, immunoglobulin G (IgG) 5, 046 mg/dl, anti-nuclear antibodies×320, anti-niitochondrial antibodies (-), hepatitis B virus surface antigen (HBsAg) (-), HBcAb (-), anti-hepatitis C virus (anti-HCV) (-), hepatitis C virus (HCV-RNA) (-), anti-hepatitis G virus (anti-HGV) (-), α-fetoprotein 306.8 ng/ml, carcinoembryonic antigen (CEA) 2.3 ng/nil, carbohydrate antigen (CA) 19-9 77.2 U/ml. Abdominal ultrasonography and computed tomography showed a large mass occupying most of the right lobe and portal thrombosis in the liver. Liver biopsy revealed cirrhosis with inactive hepatitis in the nontumorous lesion and well-differentiated hepatoceliular carcinoma in the tumorous lesion. We report a rare case of an aged male patient with autoimmune hepatitis complicated by hepatoceliular carcinoma.
(Internal Medicine 38: 422-425, 1999)

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A 60-year-old woman was admitted with severe hyponatremia. Basal values of adrenocorticotropic hormone (ACTH), thyroid hormone and cortisol were normal on admission. Impairment of water diuresis was observed by water loading test. Initially, we diagnosed her condition as the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). By provocation test, we finally confirmed that the hyponatremia was caused by hypothalamic adrenal insufficiency. The basal values of ACTH and cortisol might not be sufficient to exclude the possibility of adrenal insufficiency. Therefore, it is necessary to evaluate adrenal function by provocation test or to re-evaluate it after recovery from hyponatremia.
(Internal Medicine 38: 426-432, 1999)

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Malignant pheochromocytoma is a rare tumor with a poor prognosis because excess production of catecholamines leads to potentially lethal complications. Several chemotherapy regimens have been reported to be effective against this tumor, but a standard form of chemotherapy has not been established. We treated two patients with histologically confirmed pheochromocytoma after surgical removal of the primary lesion. Non-cardiogenic pulmonary edema was resolved and bone metastases were controlled by individualized chemotherapy that decreased the catecholamine levels, and the performance status was improved in both cases. Palliative chemotherapy should be designed to improve the quality of life of cancer patients.
(Internal Medicine 38: 433-435, 1999)

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Vasospastic angina is rarely observed during cancer treatment. The present report describes two males with lung cancer, aged 73 and 61, who developed Vasospastic angina during combination treatment of cisplatin-containing chemotherapy and thoracic irradiation. As both patients have smoked and their ages are typical for patients with coronary artery disease, such events may be incidental. However, oncologists should be aware of the possible development of myocardial ischemia during or following administration of antineoplastic agents, especially in elderly patients with pre-existing coronary risk factors or a history of thoracic radiotherapy.
(Internal Medicine 38: 436-438, 1999)

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Spontaneous regression of a bulla in the lung is rare. We describe a case of spontaneous regression associated with the development of adenocarcinoma of the lung in a 59-year-old male smoker. The bulla had begun to regress spontaneously at least six months before lung cancer was detected on a chest radiograph. He underwent left upper lobe lobectomy with mediastinal node dissection. The tumor arose within the bulla, extending along the bulla wall. He has been alive for more than eight years with no evidence of recurrence. This case suggests that spontaneous regression of a bulla should be recognized as one of the early radiographic signs of the development of lung cancer in patients with bullous lung disease.
(Internal Medicine 38: 439-141, 1999)

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We describe a patient with mucosa-associated lymphoid tissue (MALT) type lymphoma of the gallbladder who developed concurrent acute myeloid leukemia (M2). She was admitted because of progressive jaundice and underwent cholecystectomy. Histologic examination of the gallbladder showed diffuse proliferation of atypical lymphoid cells and a formed lymphoepithelial lesion. Because of progressive thrombocytopenia, a bone marrow tap was performed 25 days after the operation. Bone marrow contained 65.5% blasts, and was positive for peroxidase, CD33 and HLA-DR, and negative for lymphoid markers. We discuss the rare association of these disorders.
(Internal Medicine 38: 442-444, 1999)

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Hypertrophic nerves have occasionally been seen in chronic inflammatory demyelinating polyneuropathy (CIDP), but most are in the cauda equina. We report a case with CIDP in whom magnetic resonance imaging (MRI) with gadolinium diethylene triamine penta-acetic acid (Gd-DTPA) enhancement demonstrated hypertrophy of various peripheral nerves including multiple cranial nerves. Interestingly, none showed neurological signs corresponding to the lesions, except for clinical signs consistent with CIDP. MRI can be useful for the detection of silent, but abnormal nerve involvement in CIDP.
(Internal Medicine 38: 445-449, 1999)

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A 48-year-old man was admitted to our hospital with cough, fever and dysphagia. He had a past history of bronchial asthma and surgery for nasal polyp. Chest radiograph and computed tomography showed atelectasis in the right lower field and infiltrative shadow in the left lower field and overall thickening of the esophageal wall. Transbronchial lung biopsy (TBLB) specimens revealed infiltration of eosinophils and lymphocytes under the bronchial mucosa. Gastrointestinal tract biopsy specimens showed submucosal infiltration of eosinophils. These findings led to a definite diagnosis of eosinophilic pneumonia associated with eosinophilic gastroenteritis, a disease which has been rarely reported.
(Internal Medicine 38: 450-453, 1999)

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We analyzed the clinical course of eight patients with liver dysfunction in measles. All of the patients showed an elevation of aspartate aminotransferase (AST), alanine aminotransferase (ALT) and lactate dehydrogenase (LDH), but no jaundice. These levels returned to normal about 3 weeks after the onset of the rash. A percutaneous liver biopsy was done in two cases. Histological examination showed slight necrosis of liver cells but no significant changes in portal area. On electron microscopy, virus particles were not detected. We detected measles virus RNA in the liver specimen by RT-PCR, which suggests that the measles virus affects liver cells directly in measles.
(Internal Medicine 38: 454-457, 1999)

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