Objective The efficacy of a new chemotherapeutic combination consisting of Cis-diammineglycolatoplatinum (Nedaplatin), a derivative of cisplatin (CDDP), and 5-fluorouracil (5FU) was evaluated in patients with advanced esophageal carcinomas. Methods Nedaplatin was administered at a dose of 80 or 100 mg/m² with 500 ml of saline by slow drip infusion for 120 minutes on day 1.5FU at a dose of 350 or 500 mg/m² was mixed with 1, 000 ml of saline and administered by continuous infusion for 24 hours on days 1 to 5. Patients or Materials This combination chemotherapy was tried in 17 patients with metastatic, recurrent, or bulky unresectable esophageal cancers. Of these, 15 evaluable patients received at least two courses of chemotherapy. Results The response rates in assessable and all patients were 60% and 52.9%, respectively. Cases with lymph node and liver metastases, as well as primary lesions, showed excellent response to the therapy with positive response rates of 54.5% (6/11), 100% (5/5) and 58.4% (7/12), respectively. The median response duration was 7 (range 3 to 37+) months for patients who achieved a partial response. Adverse drug reactions were limited to three cases of grade 3 toxicity, including allergy, and decreased hemoglobin and platelets, which were well tolerated by the patients. Conclusion The present study thus indicated the combination chemotherapy of Nedaplatin and 5FU to be safe and efficacious for advanced esophageal cancer. Further investigations are clearly warranted.
(Internal Medicine 38: 844-848, 1999)

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Objective The culprit lesion morphology at acute myocardial infarction (AMI) and unstable angina pectoris (UAP) was investigated by observing the responsible vessels through intravascular ultrasound (IVUS) during the acute stage. Methods As the subjects of study, 54 lesions of 54 ACS patients (26 in AMI patients, 28 in UAP patients) were enrolled prospectively from June 1994 to June 1998. The appearance of plaque in the lesion, the distal and proximal sites, extent of calcification, eccentricity, remodeling and shrinkage were observed through IVUS before the intervention. Results At lesion and distal site, significantly more soft plaques were observed in AMI than UAP. As to the extent of calcification in the former, mild calcification was noted significantly more in distal site as well as a tendency of more mild calcification in the lesion and proximal site. Conclusion These results suggested that the condition of responsible coronary artery is involved in the onset mechanism of AMI and UAP.
(Internal Medicine 38: 849-855, 1999)

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Objective We studied familial cases of skeletal myopathy with atrial fibrillation (Af) and atrioventricular (AV) block to compare the clinical features to other myopathies associated with cardiac abnormalities. Methods Neurologic, cardiologic, electrophysiologic, muscle pathology, and genetic studies were performed on the patients showing muscle weakness. Patients Four patients (a 63-year-old mother, 30 and 32-year-old sisters, and their maternal grandmother) and three healthy family members from three generations were studied. The mode of inheritance was suspected as autosomal dominant. Results Two sisters with congenital myopathy without rigid spine developed Af and AV block at the age of 28 and 18, respectively. The mother showed AV block, and underwent pacemaker implantation at the age of 63. The maternal grandmother had dilated cardiomyopathy, Af and severe lordosis. She died of stroke attacks and congestive heart failure at the age of 78. Muscle biopsy obtained from the mother and sisters showed myopathic changes without characteristic abnormalities. No mitochondrial DNA mutations were found. Other inherited myopathies with cardiac complications were not suspected in this family. Conclusion This Japanese family appears to belong to a new genetically heterogeneous group of autosomal dominant skeletal myopathy with severe AV block and Af.
(Internal Medicine 38: 856-860, 1999)

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A 66-year-old man whose renal function had progressively deteriorated had an elevated blood pressure and also was found to have an inflammatory abdominal aortic aneurysm (AAA). Blood examination revealed that he had eosinophilia. Livedo reticularis of the toes developed, and a skin biopsy specimen showed embolization of atheromatous plaques in the arterioles of the subcutaneous tissue. Progressive enlargement of inflammatory AAA may have dislodged the atheromatous plaques, resulting in cholesterol embolism.
(Internal Medicine 38: 861-864, 1999)

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A pituitary mass compressing the optic nerve was revealed by magnetic resonance imaging (MRI) in a 35-year-old woman complaining of visual disturbance in the post-partum period. Responses of plasma gonadotropin and corticotropin-cortisol levels to respective hypothalamic hormones were delayed or blunted, but the response of plasma prolactin to thyrotopin-releasing hormone was exaggerated. Diabetes insipidus was not associated. Biopsy revealed lymphocytic adenohypophysitis, and no hypophysectomy was performed. Only five weeks later, the pituitary mass spontaneously disappeared on MRI. The pituitary function was normalized. Anti-thyroidal and anti-pituitary antibodies were negative throughout the clinical course. Pituitary masses developing during late pregnancy or the post-partum period should be carefully observed.
(Internal Medicine 38: 865-870, 1999)

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A 59-year-old woman was hospitalized in hypoglycemic coma. Although hypoglycemia was promptly reversed, she was in a somnolent, restless state with tachycardia, tremor, profuse sweating, and high body temperature. Thyrotoxic storm was highly suspected and vigorous antithyroid regimens gradually brought her up to normal mental and cardiovascular states in several days. However, profound generalized myopathy necessitated the maintenance with a respirator. One month later, an episode of angina pectoris was followed by generalized convulsion, coma, and death in a few days. Neuroimaging study disclosed posterior leukoencephalopathy syndrome. This case is instructive in that hypoglycemic coma may masquerade the major symptomatology of thyrotoxic storm, and that profound myopathy and angiopathic or angiospastic processes of the brain and the heart may interfere with the outcome.
(Internal Medicine 38: 871-874, 1999)

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We report a rare case of Klinefelter's syndrome (KS) with mixed connective tissue disease (MCTD), diabetes mellitus (DM) and several endocrine disorders. A 57-year-old man presented with polyarthritis and tapering fingers with Raynaud's phenomenon on admission. In addition to a karyotype of 47, XXY, a marked restrictive change in respiratory functional test, a myogenic pattern in electromyogram, the positive tests for anti-RNP antibody indicated that this was a case of KS complicated with MCTD. The patients also presented DM with insulin resistance, hyperprolactinemia, slight primary hypothyroidism and hypoadrenocorticism. The mechanism for these coincidences remains to be elucidated.
(Internal Medicine 38: 875-881, 1999)

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A 49-year-old woman, who had been diagnosed as sarcoidosis based on bilateral hilar lymphadenopathy and lung biopsy, presented increased serum creatinine and calcium concentrations. Renal biopsy showed the presence of interstitial nephritis with non-caseating epithelioid granuloma and focal membranous transformation. Therapy with prednisolone was effective in normalizing serum creatinine, serum calcium, serum angiotensin converting enzyme, and urine β2 microglobulin, but these abnormalities reappeared after rapid withdrawal of prednisolone. This is a rare case of sarcoidosis manifested by both membranous nephropathy and granulomatous interstitial nephritis, and indicates the necessity of long-term treatment of corticosteroid.
(Internal Medicine 38: 882-886, 1999)

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CA 19-9 is a widely used tumor marker. However, an elevation in serum CA 19-9 can occur in some patients with benign disorders such as cholecystolithiasis in the absence of tumor. We treated a case of acquired ureteral stone-induced giant hydronephrosis with markedly elevated serum CA 19-9 values. After nephrectomy, the serum CA 19-9 level returned to normal. No malignant cells were found in the tissues of the resected kidney. Localization of CA 19-9 was confirmed by immunohistochemical staining of the renal pelvic mucosa. A detailed case report is presented with a review of the literature.
(Internal Medicine 38: 887-891, 1999)

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We report a 62-year-old male with extensive disease small-cell lung cancer (SCLC) who was successfully treated with double high-dose chemotherapy supported by autologous peripheral blood stem cell transplantation (auto-PBSCT). This patient achieved a partial response with 3 cycles of induction chemotherapy. After the peripheral blood stem cell mobilization, two cycles of high-dose ICE regimen (ifosfamide 3, 000 mg/m² at days 1 to 5, carboplatin 400 mg/m² at days 1, 3, 5, and etoposide 500 mg/m² at days 1, 3, 5) could be given with further regression of the tumor and acceptable toxicities. This successful case suggests the feasibility of double high-dose ICE with auto-PBSCT in elderly patients with SCLC.
(Internal Medicine 38: 892-895, 1999)

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A 65-year-old pigeon breeder who presented with acute hypersensitivity pneumonitis refused to give up contact with pigeons and her lung disease, which had initially improved in hospital in response to removal from pigeons, progressed to chronic interstitial flbrosis. Bronchoalveolar lavage lymphocytes fell from 50.0% of total cells in December 1986 to 27.1% in February 1990. The ratio of CD4+/CD8+ lymphocytes shifted from 0.43 to 1.47. Furthermore, IL-6 and TNF-α were elevated initially and were much higher at the second time point. These data pointed to the importance of CD4+ lymphocytes, IL-6 and TNF-α in the development of pulmonary fibrosis.
(Internal Medicine 38: 896-899, 1999)

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The chest radiographs of an asymptomatic 58-year-old Japanese man with pulmonary corpora aniylacea (PCA) revealed bilateral patchy and nodular infiltrates. Lung specimens obtained by open lung biopsy were histochemically and immunohistochemically analyzed. In all sections of dissected lung tissue, hematoxylin and eosin staining revealed homogeneous eosinophilic acellular round bodies (50-100 μm in diameter) containing granular black pigments in the alveolar spaces. Some round bodies were surrounded and phagocytized by alveolar macrophages. The laminated round bodies stained positively with PAS and Congo red. In addition, many of the rounded bodies contained particles which stained positively with Berlin blue. Immunohistochemical staining for epithelial membrane antigen (EMA) as well as PE-10 was distinctively positive. This is a very rare case of diffuse PCA found by open lung biopsy. Immunohistochemical examination suggested that PCA consisted of pulmonary surfactant protein and epithelial membrane antigen.
(Internal Medicine 38: 900-903, 1999)

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A case of a 66-year-old Japanese man developed therapy-related megakaryoblastic leukemia with pituitary involvement after chemotherapy for non-Hodgkin's lymphoma. Alkylating agents had been administered for the treatment of non-Hodgkin's lymphoma and 6 years later, megakaryoblastic leukemia with myelofibrosis and myelodysplasia developed. The blast cells expressed CD41, and immature antigens also. These findings were compatible with therapy-related megakaryoblastic leukemia. An autopsy revealed blast-cell infiltration into multiple organs including the posterior pituitary lobe. Therapy-related megakaryoblastic leukemia is very rare, and pituitary involvement may be associated with immaturity of blast cells.
(Internal Medicine 38: 904-909, 1999)

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We report two cases of polymyositis (PM) complicated with nosocomial pneumonia probably caused by Stenotrophomonas maltophilia, which was resistant to multiple antimicrobials. In the first case, the chest CT findings and high serum endotoxin level as well as sputum culture results were helpful for the proper diagnosis and the therapy was successful. However the second patient died of a lung abscess in spite of the intensive antibiotic therapy. When PM patients develop pneumonia unresponsive to various antibiotics, a multi-drug-resistant bacteria such as Stenotrophomonas maltophilia should be considered as the pathogen.
(Internal Medicine 38: 910-916, 1999)

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