In the past 5 years approximately 500 patients worldwide suffering from severe autoimmune disease (AD) have received an autologous hematopoietic stem cell transplantation (HSCT) as treatment following high-dose chemotherapy. The EBMT and EULAR data base contains 370 registrations, the most frequently transplanted ADs being multiple sclerosis (MS), systemic sclerosis (SSc), rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and idiopathic thrombocytopenic purpura (ITP). Around 70% responded initially well, with durable remission/stabilization seen more frequently in MS and SSc than in RA and SLE, the latter having around 2/3 relapses, the majority of which respond to simple agents. Overall 8% transplant-related mortality was seen with large inter AD differences (12.5% in SSc and only one patient in RA) probably reflecting the degree of vital organ involvement at the time of transplant. This phase I/II data has led to a running phase III randomized trial in SSc called the Autologous Stem cell Transplantation International Scleroderma (ASTIS) trial, and it will soon begin in MS (ASTIMS) and RA (ASTIRA). The concept of immunological "re-setting" has evolved, and needs to be confirmed by longer follow-up and the multicentre, international phase III randomized studies.
(Internal Medicine 41: 608-612, 2002)

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Objective We investigated the influence of feeding tube placement on survival in hospitalized elderly patients.
Methods To assess long-term mortality in an inception cohort and the influence of feeding tube placement on survival, one hundred six hospitalized elderly patients from a nursing home were followed up through and after the index hospitalization for placement of a feeding tube and mortality. Cox regression hazards model was constructed for both univariate and multivariate analyses.
Results A feeding tube was placed in 15% (16/106) of the study patients during the index hospitalization. Median survival of the 106 patients was 381 days. A total of 92 patients (87%) survived the index hospitalization, and 52 (49%) were still alive at the last follow-up. In the multivariate survival model which included older age, hip fracture history, admitting diagnosis of pneumonia, and tube feeding placement, only feeding tube placement (hazard ratio, 2.29; 95% confidence interval, 1.22-4.33) was significantly associated with higher mortality.
Conclusion In hospitalized elderly patients from nursing home, feeding tube placement may be a risk factor for mortality.
(Internal Medicine 41: 613-616, 2002)

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Objective The present study was designed to evaluate the process of cavity formation in Mycobacterium avium intracellulare complex (MAC) lung infection, pathologically and clinically.
Methods Using resected lung specimens, we first evaluated the distribution of MAC as well as the distribution of myofibroblasts in MAC lung infection according to several pathological findings classified as bronchiectasis, centrilobular nodules, cavity, nodules, bronchiolitis, or consolidation. Resected lung specimens (9 cases) were evaluated by special staining: Ziehl-Neelsen's method and immunohistochemically for CD68 (stain for monocytes and macrophages) and α-smooth muscle actin (stain for myofibroblasts). Chest CT findings were also examined in these 9 patients. In addition, the serial chest CT scans were reviewed in another 3 patients to evaluate the process of cavity formation, radiologically.
Results Although extensive granuloma formations were observed in every pathological classification, MAC was demonstrated only in the necrotic tissue of the inner surface of the cavitary wall, which was connected to the airway. Myofibroblasts which expressed α-smooth muscle actin were intensely demonstrated in the cavitary wall compared with other pathological classifications. In the cavitary wall, the layer of epithelioid cells and multinucleated giant cells surrounded necrosis, and the layer of myofibroblasts surrounded the layer of epithelioid cells. Chest CT findings demonstrated that the cavitary walls were relatively thick. The evaluation of serial chest CT scans demonstrated that cavities were formed from previously existing nodules.
Conclusions Detection of mycobacteria in the cavitary wall, massive infiltration of myofibroblasts compared with other pathological classifications, and connection to the drainage bronchus, were believed to be important in the process of cavity formation in MAC pulmonary infection.
(Internal Medicine 41: 617-621, 2002)

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Objective To evaluate the adverse events possibly caused by sumatriptan injection and explore the relationship between chest symptoms along with sumatriptan injection and coronary ischemia among Japanese patients with migraine.
Methods A cumulative total of 112 subcutaneous injections in 62 patients were evaluated. ECG was continuously monitored before and until 5 minutes after injection on 92 occasions.
Patients Sixteen men and 46 women aged from 16 to 60 (mean 39±12) years. Their clinical diagnoses were migraine with aura, migraine without aura, cluster headache, and others.
Results Chest symptoms occurred following 17% of all injections and in 15% of all patients. None of these chest symptoms was accompanied by ECG changes.
Conclusions Although the risk of coronary ischemia with sumatriptan treatment is commonly stated, our data suggest that chest symptoms following sumatriptan injection are not strongly associated with coronary ischemia in the Japanese population. The mechanism of chest symptoms following sumatriptan administration should be further elucidated.
(Internal Medicine 41: 622-625, 2002)

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Objective To investigate the degree of Japan's contribution in basic and clinical research in the last decade.
Methods Original articles published in 1990-2000 in highly reputed basic and clinical journals were accessed through MEDLINE database. The number of articles having affiliation with a Japanese institution was counted separately for basic and clinical journals.
Results Of total articles, Japan's contributions in basic and clinical research were 3.1% and 0.7%, respectively (p=0.0001). The recent increase in the contribution was significant for basic research (p=0.01), but not for clinical research (p=0.91).
Conclusion The barriers to boosting high quality clinical research in Japan should be determined and accordingly appropriate measures should be taken forthwith.
(Internal Medicine 41: 626-628, 2002)

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We present a rare occurrence of woman monozygotic twins with ulcerative colitis (UC). A 21-year-old woman came to our hospital because of diarrhea, abdominal pain and hematochezia. We diagnosed this case as proctitis type UC by endoscopic and histological findings. Six months later, her twin sister developed total colitis type UC. Both twins had HLA-A24, B52, DR2, and DQ1 serological types, and had DRB1*1502 DNA type, previously shown to be associated with UC. This case report suggested an association of genetic factor together with environmental factors in the etiology for UC.
(Internal Medicine 41: 629-632, 2002)

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A 32-year-old man was admitted to our hospital complaining of abdominal pain in the left upper quadrant. A mass was palpable on the left side of the umbilicus. Laboratory data revealed anemia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and prolonged prothrombin time. Computed tomography demonstrated a soft tissue mass in the mesentery of the jejunum, portal venous thrombosis, and cavernomatous transformation in the porta hepatis. The patient was eventually diagnosed by laparoscopic partial resection as having inflammatory pseudotumor of the mesentery. Four months later, all of his symptoms and abnormal laboratory findings completely disappeared without any therapy. Inflammatory pseudotumor should be kept in mind as a cause of portal venous thrombosis, and/or cavernomatous transformation although it is rare.
(Internal Medicine 41: 633-637, 2002)

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A 64-year-old woman was admitted with systemic edema and exertional dyspnea. High-output heart failure was diagnosed by right heart catheterization and she was treated with diuretics. After 3 weeks, her symptoms disappeared but a high cardiac output state persisted. A diagnosis of Crow-Fukase syndrome was made based on the presence of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Her serum vascular endothelial growth factor (VEGF) level was markedly elevated after recovery from heart failure. We suspect that an elevated VEGF level and a high cardiac output state may play a role in the pathogenesis of heart failure in Crow-Fukase syndrome.
(Internal Medicine 41: 638-641, 2002)

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Isolated ventricular noncompaction (IVNC) is an unusual congenital heart disease. The morphological features are trabeculations and intertrabecular recesses within the left ventricle. Important clinical considerations include the high mortality due to depressed cardiac function, and the instance of ventricular arrhythmia and systemic embolism. One of reasons for the high mortality is that there is still no effective therapy with the exception of the recommendation for anticoagulation therapy to prevent embolisms. Hence, we describe a 29-year-old man with IVNC presenting with chronic heart failure. Careful up-titration of carvedilol in this patient changed the parameters of cardiac diastolic performance
(Internal Medicine 41: 642-647, 2002)

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A 30-year-old man, who had a repeated history of relapsed Hodgkin's lymphoma over 7 years, developed bilateral pleural effusion and chest wall involvement. He was treated with weekly irinotecan hydrochloride (CPT-11; 80 mg/m²/week). Partial response was observed after two cycles of irinotecan. Neutropenia and diarrhea were tolerable. This case demonstrated that irinotecan has a therapeutic effect in patients with relapsed Hodgkin's lymphoma.
(Internal Medicine 41: 648-650, 2002)

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We report the case of a young female patient with chronic active Epstein-Barr virus infection (CAEBV) and severe hypersensitivity to mosquito bites (SHMB). She showed a marked increase of NK cell population in peripheral blood. The NK cell population was suggested to be infected with EBV, and to be oligoclonal by Southern blotting using an EBV genome terminal-repeat probe. The NK cells aberrantly expressed CD25, a high affinity receptor for IL-2, and showed an augmented in vitro proliferative response to IL-2. Moreover, they also showed enhanced expression of both Fas-ligand and Bcl-2, and resistance to in vitro Fasinduced apoptotic cell death (Fas-ACD). Taken together, these observations suggested that both the augmentation of proliferative response to IL-2 and the decrease in Fas-ACD may cause NK cell lineage granular lymphocyte proliferative disorder (NK-GLPD) in patients with CAEBV and SHMB.
(Internal Medicine 41: 651-656, 2002)

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We report herein the case of a 70-year-old patient with elderly onset rheumatoid arthritis associated with severe muscle pain in shoulder and pelvic girdle. The patient revealed erosive polyarthritis with high titers of rheumatoid factor. Muscle pain started one month after the onset of rheumatoid arthritis followed by muscle weakness and muscle atrophy. Synovial effusion and edema in the soft tissue outside of the articular capsule in the knee joint were confirmed ultrasonographically. Administration of prednisolone at 20 mg/day dramatically abolished the muscular manifestations. The coexistence of an early stage of elderly onset rheumatoid arthritis and polymyalgia rheumatica was considered due to the presence of seropositive erosive arthritis and severe muscle manifestations at the same time.
(Internal Medicine 41: 657-660, 2002)

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Paragonimiasis is an important re-emerging parasitosis in Japan. Although the lungs and pleural cavity are the principal sites affected with the parasite, ectopic infection can occur in unexpected sites such as skin and brain. This case report describes a patient with active hepatic capsulitis due to Paragonimus westermani infection. The patient was successfully treated with praziquantel at the dose of 75 mg/kg/day for 3 days.
(Internal Medicine 41: 661-663, 2002)

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Tonsillar tuberculosis is one of the uncommon forms of extrapulmonary tuberculosis. We report a case of tonsillar tuberculosis associated with pulmonary and laryngeal foci. A 23-year-old female was admitted for evaluation of hoarseness and difficulty in swallowing. Bilateral palatine tonsils were enlarged, and a tonsillectomy was performed. Since a histological study revealed tonsillar tuberculosis, antituberculous agents were administered. After the treatment the pulmonary lesions detected with chest computed tomography were improved, and her symptoms were relieved. The possibility of tonsillar tuberculosis should be considered when unexplained enlarged tonsil is observed in patients with pulmonary tuberculosis.
(Internal Medicine 41: 664-666, 2002)

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A 74-year-old Japanese man with scrub typhus presented without the typical symptom of high fever and subsequently developed the complication of acute respiratory distress syndrome. It was suspected that exposure occurred at the river side of Kinugawa, Tochigi Prefecture, Japan. His body temperature was below 38.0°C. After intensive supportive care and minocycline therapy, he dramatically recovered. With the increase in popularity of outdoor recreation, scrub typhus can be found in clinics all over Japan. Physicians should therefore be aware of the manifestations of the disease and the necessity of early treatment in suspected cases.
(Internal Medicine 41: 667-670, 2002)

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Musculoskeletal and central nervous system infections caused by Bacillus cereus are very rare. Only a few cases have been reported, whose clinical courses strongly suggested that surgical procedures combined with appropriate antimicrobial therapy are necessary to cure these infections. A 60-year-old man with severe neutropenia due to myelodysplastic syndrome, developing necrotizing fasciitis and brain abscess caused by Bacillus cereus is reported. Without performing any surgical procedures, the patient was successfully treated with systemic antimicrobial therapy combined with granulocyte colony stimulating factor, which contributed to the increase in the neutrophil count.
(Internal Medicine 41: 671-673, 2002)

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