http://hdl.handle.net/10564/1575 2006-06 Fri, 10 Apr 2026 15:38:28 GMT 2026-04-10T15:38:28Z http://hdl.handle.net/10564/167 タイトル: 孤発性Blau症候群の長期経過観察例 著者: 櫻井, 嘉彦; 福田, 和由; 中島, 充; 吉岡, 章; 森本, 勝彦; 宮川, 幸子; 竹谷, 太; 原, 嘉昭 抄録: We report a female patient with sporadic Blau syndrome who was followed up for more than 10 years before being diagnosed at the age of 19. Recurrent skin, eye, and bone involvement started in infancy. Her initial clinical diagnosis was SLE and juvenile idiopathic arthritis (JIA) later. At 9 years old, the diagnosis of early-onset sarcoidosis (EOS) was confirmed with histopathologic diagnosis from skin biopsy. Since clinical manifestations of EOS are very similar to those of JIA, skin biopsy is essential for differential diagnosis. Underlying etiology of EOS had been unraveled to that date. DNA analysis revealed that the patient had a heterozygous missense mutation for CARD15/NOD2 (caspase recruitment domain-containing protein 15), the gene for an intracellular receptor for bacterial products in monocytes that transduces signals leading to NF-κB activation. This case showed an arg334trp mutation that had been reported in Blau syndrome that shows phenotypic overlap with EOS. As her parents showed no mutation in CARD15, she was diagnosed as having a sporadic Blau syndrome. She had been treated with oral and/or intravenous steroids for a long time. Since she had intraocular lens implantation due to steroid cataract at the age of 12, intravenous liposteroid, a lipid emulsion containing a water-soluble dexamethasone in lipid vesicles, was started with oral prednisolone. This treatment lessened the side effects of the steroid and allowed us to control the manifestation of EOS/Blau syndrome. Thu, 29 Jun 2006 15:00:00 GMT http://hdl.handle.net/10564/167 2006-06-29T15:00:00Z http://hdl.handle.net/10564/166 タイトル: 若年成人における粟粒結核による突然死の1剖検例 著者: 粕田, 承吾; 森村, 佳史; 工藤, 利彩; 福留, 昭人; 実藤, 信之; 正武, 孝規; 石谷, 昭子; 羽竹, 勝彦 抄録: A 29-year-old man was found dead in his bed. He had been treated with pus clearing at the hospital because of anal abscess 2 months before his death. Thereafter, he had been complaining of fever and abdominal pain immediately prior to his death. The cause of death was diagnosed as miliary tuberculosis from the findings of an autopsy. No other lesions were found. The incidence of miliary tuberculosis in the young without underlying diseases is considered to be quite rare. Nowadays concerns about tuberculosis have declined among health-care professionals. This report suggests that all doctors, both clinicians and forensic pathologists, should always keep tuberculosis in mind. Thu, 29 Jun 2006 15:00:00 GMT http://hdl.handle.net/10564/166 2006-06-29T15:00:00Z http://hdl.handle.net/10564/165 タイトル: 悪性症候群後に小脳性運動失調をきたした1例 著者: 永嶌, 朋久; 宮本, 敏雄; 森川, 将行; 根來, 秀樹; 岸本, 年史 抄録: A 17-year-old female with adjustment disorder repeated suicide attempts (self poisoning and cutting) several times due to unstable mental states such as impulsive behaviors. She developed neuroleptic malignant syndrome (NMS) after taking large amounts of drugs (analgesics and antipyretics) and was admitted to the emergency center at Nara Medical University. She was treated intensively, but cerebellar ataxia such as intention tremor and vertical nystagmus occurred subsequently after physical improvement. She understood spoken words, but did not speak any words herself. Some mental influences on her symptoms were suspected and she was moved to a psychiatric ward. We speculated that exposure to abnormal high temperature damaged Purkinje cells in the cerebellum and started to administer tartaric acid protirelin intravenously. Several days later explosive speech appeared and three months later atrophic changes were found in the cerebellar hemisphere by brain MRI. Her cerebellar ataxia was gradually improved by medication and physical therapy, and control of her impulsiveness was also improved by intrafamilial adjustment. Thu, 29 Jun 2006 15:00:00 GMT http://hdl.handle.net/10564/165 2006-06-29T15:00:00Z http://hdl.handle.net/10564/164 タイトル: 奈良県立医科大学精神科における平成16年度の時間外電話相談,時間外受診患者の臨床的検討 著者: 長内, 清行; 森川, 将行; 永嶌, 朋久; 紀本, 創兵; 中川, 恵樹; 太田, 豊作; 鳥塚, 通弘; 岡田, 光司; 澤田, 将幸; 山内, 崇平; 一岩, 智明; 木内, 邦明; 岸野, 加苗; 牧之段, 学; 芳野, 浩樹; 洪, 基朝; 宮本, 敏雄; 根來, 秀樹; 高橋, 良斉; 井上, 眞; 中川, 康司; 岸本, 年史 抄録: Characteristics of emergency telephone counseling and medical service for outpatients in the Psychiatric Service of Nara Medical University in fiscal 2004 were investigated, and those clinical features of emergency psychiatric service of the core general hospitals in Nara Prefecture were analyzed. The total of emergency telephone counseling was 1,049 cases/year and the details consisted of mild psychiatric deterioration (31.8%), questions conceming medication (15%), and distress (of patient or caregiver) (12.8%). The majority of our responses needed for telephone counseling was brief supportive counseling (87.2%). According to the diagnostic classification of ICD-10, F2 (schizophrenia, schizotypal and delusional disorder) were most frequent (35.6%), followed by F4 (neurotic, stress-related and somatoform disorder; 23.5%), F3 (mood disorder; 19.2%), and others. The total of emergency medical services was 846 cases/year and the details consisted of psychiatric deterioration (anxiety, agitation, etc.) (36.8%), complaints related to physical symptoms (21.3%), and suicide attempts (11.7%). About 22% patients were referred for psychiatric examination from other departments in our hospital. Complaints related to physical symptoms might be one of psychiatric symptoms, but additional physical examinations (blood test, brain CT, etc.) were needed according to the circumstances for differential diagnosis of physical disease. The rate of suicide attempts was 11.7%, which may reflect the feature of a general hospital with psychiatric service. Also the availability of psychiatric service in general hospitals (21.7%) is more useful for them and consultation-liaison psychiatry is much important for one another. Thu, 29 Jun 2006 15:00:00 GMT http://hdl.handle.net/10564/164 2006-06-29T15:00:00Z