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Masamitsu NAKAZATO, Kazutaka SHIOMI, Mikiya MIYAZATO, Shigeru MATSUKUR ...
1992 Volume 31 Issue 12 Pages
1335-1338
Published: 1992
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We studied 107 cases and 64 carriers of type I familial amyloidotic polyneuropathy (FAP) residing in 16 districts in Japan. The age of onset of illness ranged from 20 to 71 years old, with a mean of 40.1±12.8 years (SD). One quarter of the cases were late-onset patients who developed the disorder after age 50. Asymptomatic carriers older than age 50 accounted for 20% of total carriers, with the oldest carrier being a 94-year-old woman. All the patients had a variant transthyretin with a methionine-for-valine substitution at position 30 with a mean serum level of 9.78±3.27 (SD) mg/dl. The serum level did not significantly differ by gender in either patients or carriers, nor between patients and carriers. Incomplete penetrance of clinical expression was shown in eight cases. This study indicates that there is a considerable variety in age of onset, progression and geographic distribution of type I FAP in Japan.
(Internal Medicine 31 : 1335-1338, 1992)
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Keiko TANAKA, Shuichi IGARASHI, Motoyoshi YAMAZAKI, Takashi NAKAJIMA, ...
1992 Volume 31 Issue 12 Pages
1339-1342
Published: 1992
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Paraneoplastic cerebellar degeneration (PCD) is thought to be caused by an autoantibody against both tumor and neuronal tissue. Such autoantibodies are most frequently detected in patients with gynecological or breast cancer, and are designated as anti-Yo. We report here a patient with PCD whose underlying cancer could not be detected despite extensive tumor survey. IgG in her serum and cerebrospinal fluid reacted with the cytoplasm of cerebellar Purkinje cells immunohistochemically. On immunoelectron microscopy, the endoplasmic reticulum and Golgi complex were stained. Her IgG bound to the 58kD band on immunoblots of cerebellar proteins. A reaction was also observed with the recombinant proteins deduced from the complementary DNA clone encoding a neuronal cell antigen reported by Sakai et al (Ann Neurol 28 : 692, 1990). Based on these results, successful early resection of fallopian tube adenocarcinoma was performed. It is crucially important to characterize these PCD related autoantibodies for the early treatment of underlying malignant tumors.
(Internal Medicine 31 : 1339-1342, 1992)
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Hideshi ISHII, Hakumei OH, Yoshitaka UCHIDA, Hirotoshi NAKAMURA, Nobuy ...
1992 Volume 31 Issue 12 Pages
1343-1347
Published: 1992
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Five patients (six courses) with refractory idiopathic thrombocytopenic purpura (ITP) were given 6 million units of recombinant interferon (IFN) alpha-2b in 12 doses to achieve an improved response rate compared to previous studies using 3 million units. From the initial IFN administration, the platelet count increased from a pre-treatment level of 20.7±17.7×10
3/μl (mean±SD) and reached its first peak in weeks 2 or 3 of therapy (p<0.05). In week 5, the platelet count made its second and maximum peak (66.5±57.9×10
3/μl ; p<0.05). A relatively good response of the platelet count (an increase to >50×10
3/μl) was observed in three patients (four courses) out of five. These responses were not much faster or more improved than in previous reports, and a dose of 6 million units may be too large to treat some ITP patients. The platelet-associated IgG level showed a tendency to be reduced with IFN therapy. The mechanism for the increase of the platelet count may be the modification of platelet autoantibody production.
(Internal Medicine 31 : 1343-1347, 1992)
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Isao SHOJI, Tohru TAKAGI, Reiji KASUKAWA
1992 Volume 31 Issue 12 Pages
1348-1355
Published: 1992
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Anti-centromere antibodies (ACA) in 41 sera from patients with primary biliary cirrhosis (PBC) were analyzed by an immunoblotting method and the correlation between the presence of ACA and the clinical features in these PBC patients was studied. In 10 of 16 ACA-positive PBC patients, one or more clinical features of CREST syndrome (PBC-CREST)were found. Statistical differences were observed in age at disease onset, serum levels of IgM and total bilirubin and titer of anti-M2 antibody, between PBC-CREST patients and the PBC patients without CREST symptoms (PBC-non CREST). By immunoblotting analysis, three major epitopes of ACA were identified at 18kD, 80kD and 140kD polypeptides. The 18kD polypeptides were detected in all 16 ACA-positive PBC patients. From these results, it is suggested that ACA-positive PBC-CREST patients can be separated from ACA-negative PBC-CREST and PBC-non CREST patients.
(Internal Medicine 31 : 1348-1355, 1992)
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Takeharu KOGA, Naoto TOKUNAGA, Yoichiro ICHIKAWA, Kotaro OIZUMI
1992 Volume 31 Issue 12 Pages
1356-1358
Published: 1992
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We treated a 72-year-old woman with adult Still's disease. The diagnosis was made on the basis of a prolonged, high grade, quotidian fever, polyarthritis, maculopapular skin rashes and exclusion of other possible diseases. A high serum ferritin value was a key factor both in making the diagnosis and in the follow-up. The patient responded to the administration of oral prednisolone at 30mg/day, which was tapered to 10mg/day, with no recurrence of symptoms. This disorder can be an important cause of prolonged fever in the elderly as well as in the younger population.
(Internal Medicine 31 : 1356-1358, 1992)
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Takefumi FURUYA, Takahiro SUZUKI, Noritaka ONODA, Kazuko TAMURA, Kanji ...
1992 Volume 31 Issue 12 Pages
1359-1362
Published: 1992
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A patient with mixed connective tissue disease who developed protein losing enteropathy (PLE) is described. The PLE and other symptoms improved dramatically after monthly intravenous administration of 700 mg/day cyclophosphamide three times combined with oral prednisolone, while they were ineffective to the treatment with intravenous methyl-prednisolone 500 mg per day for 3 days. The serum level of CA125 antigen paralleled the severity of symptoms, signs and laboratory data associated with PLE. Thus, pleural effusion, ascites, edema and hypoalbuminemia improved along with the decrease in the level of CA125, suggesting that CA125 might be a marker of the activity of PLE.
(Internal Medicine 31 : 1359-1362, 1992)
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Takashige MIYAZAKI, Shigeru KOHNO, Akira SAKAMOTO, Kiyotaka KOMORI, Ip ...
1992 Volume 31 Issue 12 Pages
1363-1365
Published: 1992
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A rare case of extramedullary plasmacytoma in the mediastinum is reported. An 80-year-old man was admitted for further examination of a mediastinal tumor. Chest computed tomography (CT) revealed a large mediastinal mass and right interlobar pleural effusion. Needle biopsy under CT guidance established a diagnosis of plasmacytoma. Immunohistochemical staining revealed that the tumor cells were producing monoclonal IgA kappa. Serum immunoelectrophoresis revealed an IgA kappa monoclonal component with a serum concentration of 5, 040mg/ml. The bone marrow aspiration was normal. Bone roentgenogram and bone scintigram showed osteoporosis but no neoplastic lesion.
(Internal Medicine 31 : 1363-1365, 1992)
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Hiroyuki KOSHIYAMA, Makoto SAKAMOTO, Kazuhisa FUJIWARA, Young-Chi KIM, ...
1992 Volume 31 Issue 12 Pages
1366-1369
Published: 1992
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A 28-year-old man with chondroid chordoma, an uncommon variant of chordoma, is reported. The patient presented with visual disturbance and hypopituitarism. The latter is a rare complication of intracranial chordoma. The preoperative diagnosis of chondroid chordoma of the skull base was based on unique findings on computed tomography and magnetic resonance imaging studies. The development of the tumor over six years suggests that the prognosis of chondroid chordoma may be poor in younger patients, as recently reported. Although chondroid chordoma is very rare, it should be included in the differential diagnosis of hypopituitarism.
(Internal Medicine 31 : 1366- 1369, 1992)
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Masaaki WADA, Eiji OHASHI, Hirofumi JIN, Masashi NISHIKAWA, Shigeyuki ...
1992 Volume 31 Issue 12 Pages
1370-1375
Published: 1992
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A case of mesenchymal hamartoma of the liver incidentally found to be a hyperechoic mass by abdominal ultrasonography in a 62-year-old male is described. Computed tomography and magnetic resonance imaging of the liver confirmed the presence of a well-demarcated inhomogeneous mass in the left medial segment. Hepatic arteriography revealed the mass to be generally hypovascular. Histopathologic diagnosis of the resected specimen was a solid form of benign mesenchymal hamartoma, a rather rare lesion for the patient's age. Fifteen adult cases of hepatic mesenchymal hamartoma in the literature including the present case are reviewed.
(Internal Medicine 31 : 1370-1375, 1992)
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Tohru TAKEUCHI, Keiji YOSHIOKA, Akihiro HORI, Ichiro HARA, Akira OHSAW ...
1992 Volume 31 Issue 12 Pages
1376-1380
Published: 1992
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An 83-year-old man suffering from pulmonary emphysema was admitted to our hospital because of jaundice. He was diagnosed as acute intrahepatic cholestasis but the etiology could not be determined during the treatment period. In spite of treatment, the jaundice worsened progressively without any elevation in serum transaminase, and he died of respiratory failure 58 days later. An autopsy revealed a generalized cytomegalic inclusion disease, predominantly in the biliary tracts, liver and lungs. This is a rare case of cytomegalic inclusion disease presenting acute intrahepatic cholestasis without any elevation of transaminase during the clinical course.
(Internal Medicine 31 : 1376-1380, 1992)
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Hiroshi YAMADA, Shigeru KOHNO, Hironobu KOGA, Katsuhisa OMAGARI, Shige ...
1992 Volume 31 Issue 12 Pages
1381-1386
Published: 1992
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A 23-year-old man with persisting high fever developed hepatosplenomegaly, lymphadenopathy and massive pericardial effusion. Immunological examination revealed a marked elevation of anti-Epstein-Barr virus antibodies (anti-viral capsid antigens IgG-antibody 1:10, 240, anti-early antigens-DR IgG-antibody 1:5, 120), decreased activities of Epstein-Barr virus specific cytotoxic T lymphocytes, natural killer cells and lymphokine activated killer cells. A liver biopsy showed moderate sinusoidal lymphocytosis with punched-out lesions. These findings suggested severe chronic active Epstein-Barr virus infection syndrome. The patient was treated with recombinant human interleukin-2, but it was discontinued because of an adverse reaction. Twelve months later, he died of suspected pulmonary infection.
(Internal Medicine 31 : 1381-1386, 1992)
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Yasuhide UWABE, Hitoshi YOSHIDA, Mayumi ONISHI, Kazuhiko KUDO, Kazuo M ...
1992 Volume 31 Issue 12 Pages
1387-1391
Published: 1992
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A 33-year-old Japanese woman visited our hospital with progressive dyspnea. A chest Roentgenogram and a chest computed tomogram revealed a mediastinal tumor and pericardial effusion. Ultrasound cardiography revealed the existence of cardiac tamponade. She was succesfully treated with emergency pericardial drainage, thoracocentesis and chemotherapy. The histological diagnosis of diffuse large B cell lymphoma was established with the tumor specimen obtained by transcutaneous fine needle aspiration biopsy and through immunohistochemical analysis. This is a rare case of primary mediastinal diffuse large cell lymphoma with initial presentation of a symptom related to pericardial effusion.
(Internal Medicine 31 : 1387-1391, 1992)
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Yoshinori SHIMAMOTO, Masayuki SANO, Shoko KANEDA, Seiji TSUNADA, Kyosu ...
1992 Volume 31 Issue 12 Pages
1392-1395
Published: 1992
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A case of disseminated intravascular coagulation (DIC) in a patient with systemic lupus erythematosus (SLE) w th acute liver dysfunction is described. A 37-year-old man with SLE developed acute DIC and marked liver damage after fracture of the right clavicle and pharyngitis. Treatment with high-dose steroids, heparin, antithrombin III, gabexate mesilate, and antibiotics resulted in prompt improvement. The recovery of an SLE patient after acute DIC and marked liver damageis considered very rare. We report here such a case and discuss the previous reports.
(Internal Medicine 31: 1392-1395, 1992)
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Akitaka NONOMURA, Yuji MIZUKAMI, Junji SHIMIZU, Makoto ODA, Yoh WATANA ...
1992 Volume 31 Issue 12 Pages
1396-1400
Published: 1992
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A case with primary plasmacytoma of the lung is described. The patient, a 55-year-old
Japanese female, who simultaneously had a pulmonary plasmacytomaand bladder carcinoma. The bladder tumor was treated with transurethral resection. Pathologically, the bladder tumor was a non-invasive, papillary transitional cell carcinoma, grade II. The lung tumor was located in the right upper lobe and upper lobectomy was performed. The tumor measured 2.8 × 2.7 × 2.0 cm and had a white-yellowish cut surface. Histologic, electron microscopic and immunohistochemical examinations of the lung tumor revealed monoclonal proliferation of plasma cells (IgA, lambda light chain). There was no evidence of multiple myeloma.
(Internal Medicine 31: 1396-1400, 1992)
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Akira TAKESHITA, Hideki NAKAZAWA, Hiroshi AKIYAMA, Kazuo TAKEUCHI, Ryu ...
1992 Volume 31 Issue 12 Pages
1401-1405
Published: 1992
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A case of disseminated cryptococcosis with features of primary adrenal insufficiency and meningitis in an immunocompetent host is presented. Despite antifungal chemotherapy, neither meningitis nor bilateral adrenal gland enlargement was improved. Aspiration biopsy of the adrenal gland revealed necrotic tissue with numerous fungi, suggesting that the adrenal glands were the focus of the persistent fungemia. Removal of bilateral adrenal glands led to improvement by making the patient more sensitive to antifungal chemotherapy.
(Internal Medicine 31: 1401-1405, 1992)
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Kouichi NISHI, Shigeharu MYOU, Takio OOKA, Masaki FUJIMURA, Tamotsu MA ...
1992 Volume 31 Issue 12 Pages
1406-1408
Published: 1992
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A 74-year-old healthy man with locally invasive form of pulmonary aspergillosis (PA) is reported. Chest X-ray film showed a segmental infiltration of right upper lobe (RUL) without cavitation, and the transbronchial lung biopsy specimen contained numerous hyphae of aspergillus species. Complication of bronchial asthma, or bronchiectasis were absent, and hyphae of aspergillus were present at only one segment of RUL. After 5 months of therapy with oral fluconazole, the PA had dramatically improved. Fluconazole was found to be effective for the locally invasive form of PA in a healthy man.
(Internal Medicine 31: 1406-1408, 1992)
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Hisamaru HIRAI
1992 Volume 31 Issue 12 Pages
1409-1410
Published: 1992
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Ichiro KANAZAWA
1992 Volume 31 Issue 12 Pages
1411-1412
Published: 1992
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Masato KASUGA
1992 Volume 31 Issue 12 Pages
1413-1414
Published: 1992
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Kazuhiko YAMAMOTO
1992 Volume 31 Issue 12 Pages
1414-1416
Published: 1992
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Kiyoshi KUROKAWA
1992 Volume 31 Issue 12 Pages
1417
Published: 1992
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Toshio MATSUMOTO
1992 Volume 31 Issue 12 Pages
1418a
Published: 1992
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Yohtaro FURUKAWA
1992 Volume 31 Issue 12 Pages
1418b-1421
Published: 1992
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Masataka SHIRAKI
1992 Volume 31 Issue 12 Pages
1421-1424
Published: 1992
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Kiyoshi KUROKAWA
1992 Volume 31 Issue 12 Pages
1424-1426
Published: 1992
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Tsuneo SASAKI, Kazuo OHTA
1992 Volume 31 Issue 12 Pages
1427-1429
Published: 1992
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Haruhiko SATO, Maroh SUZUKI
1992 Volume 31 Issue 12 Pages
1430-1431
Published: 1992
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Shohei KOYAMA, Tsugio EBIHARA
1992 Volume 31 Issue 12 Pages
1431-1432
Published: 1992
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Yojiro NIITSU
1992 Volume 31 Issue 12 Pages
1432
Published: 1992
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Hiroaki IWASE, Kimitomo MORISE
1992 Volume 31 Issue 12 Pages
1433-1434
Published: 1992
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Jun AOKI, Takeshi MIWA
1992 Volume 31 Issue 12 Pages
1434-1435
Published: 1992
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Seishiro MIMURA, Shigeru OKUDA
1992 Volume 31 Issue 12 Pages
1436
Published: 1992
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