Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
49 巻, 9 号
選択された号の論文の18件中1~18を表示しています
EDITORIAL
ORIGINAL ARTICLES
  • Yasuaki Tatsumi, Ai Hattori, Hisao Hayashi, Jiro Ikoma, Masahiko Kaito ...
    2010 年 49 巻 9 号 p. 809-815
    発行日: 2010年
    公開日: 2010/04/30
    ジャーナル オープンアクセス
    Objective This study evaluated the current state of patients with Wilson disease in central Japan.
    Patients and Methods Between 1999 and 2007, 30 patients were diagnosed as having Wilson disease with an International Diagnostic Score of 4 or more. The phenotypes, genotypes and post-diagnostic courses of these patients were analyzed.
    Results Twenty-six patients had ATP7B mutations responsible for Wilson disease. Four patients had a single mutant chromosome. There were 2 major mutations of 2333 G>T and 2871 delC (40%), and 6 novel mutations (13%) in our patients. The first clinical manifestation was the hepatic form in 22, neurological form in 5, and hemolysis in 3 patients. The hepatic form was diagnosed around the age of 13 years, followed by neurological complication with a time lag of 9 years. Thus, some patients, especially patients with the neurological form, did not undergo early diagnostic tests including ATP7B analysis. During the post-diagnosis period, 3 patients were hospitalized for recurrent liver disease, and 2 patients committed suicide. One female patient died from acute hepatic failure associated with encephalopathy after fertilization therapy, while 2 male patients recovered from encephalopathy-free, prolonged hepatic failure after noncompliance with drug therapy. The King's Scores for liver transplantation were below the cut-off in both cases.
    Conclusion To minimize delayed diagnosis, ceruloplasmin determination and ATP7B analysis may be recommended to patients showing hepatic damage of unknown etiology. At gene diagnosis, appropriate management of patients including compliance education and emotional care to prevent suicide might be important.
  • Kaori Miwa, Taku Hoshi, Hidetaka Hougaku, Makiko Tanaka, Shigetaka Fur ...
    2010 年 49 巻 9 号 p. 817-822
    発行日: 2010年
    公開日: 2010/04/30
    ジャーナル オープンアクセス
    Background Both silent cerebral infarction (SCI) and carotid intima-media thickness (IMT) are associated with future stroke. We evaluated whether SCI could be a predictor for incident stroke independent of carotid IMT in high-risk patients.
    Methods We performed a prospective cohort study among 282 outpatients who had one or more atherosclerotic risk factors but without a history of cardiovascular disease. We conducted cranial MRI and measured carotid IMT at baseline, and then evaluated the risks of incident stroke and transient ischemic attacks (TIA) using Cox proportional hazards models.
    Results SCI was present in 67 patients (23.7%) at baseline. During 4.1 years of follow-up, stroke and TIA occurred in 8 patients (2.8%). The incidence of stroke/TIA was 22.3 per 1,000 person-years in those with SCI compared with 2.2 per 1,000 person-years in those without SCI. Both SCI and carotid IMT at baseline were associated with incident stroke/TIA events after adjustment for age, sex, and traditional vascular risk factors. The predictive value of SCI remained significant even after adjustment for carotid IMT (HR 8.56; 1.72-42.55).
    Conclusion SCI, similar to carotid IMT, is an independent predictor of stroke and TIA in high-risk patients.
  • Naomi Matsuyama, Shinji Morimoto, Yoshiaki Tokano, Hirofumi Amano, Kaz ...
    2010 年 49 巻 9 号 p. 823-828
    発行日: 2010年
    公開日: 2010/04/30
    ジャーナル オープンアクセス
    Objective Patients with lupus nephritis receiving intravenous cyclophosphamide (IVCY) therapy were divided into groups according to their clinical course, and the long-term prognosis was evaluated.
    Patients and Methods A total of 67 patients with lupus nephritis were enrolled and divided as follow into the following groups: Group A: patients with fresh nephritis, Group B: patients with relapse nephritis, Group C: patients with nephritis as a transition of the main clinical manifestation. IVCY (500 mg or 750 mg) was administered every month, and continued for two to more than six months.
    Results The rate of remission was 78%; group A revealed a significantly higher rate of remission as compared with the other groups. Although long-term remission was revealed in most patients, some patients in Group B demonstrated a decreased rate of remission. Concerning the total dose administered, there was no relation to prognosis; a high dose was not required, especially for patients in Group A. On the other hand, the combination of steroid pulse therapy with IVCY revealed a moderate relation to the increased rate of remission in Group A. However, this combination therapy was not related to the maintenance of remission. There was no adverse effect at late onset.
    Conclusion The long-term prognosis of IVCY differed according to the patient's clinical course, and the result differed from those reported in other countries. Therefore, we should consider the clinical course and race specificity for the Japanese subject.
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