http://hdl.handle.net/10564/1523 1998-04 2026-04-10T15:42:01Z http://hdl.handle.net/10564/440 タイトル: A CASE OF AN ISOLATED ADRENOCORTICOTROPIN (ACTH) DEFICIENCY : RESOLVED AND DELIVERED A CHILD AFTER 8 YEARS OF TREATMENT 著者: Okamoto, Shingo; Ueda, Shigehiko; Tsukamoto, Noboru; Sugimoto, Yumi; Tanaka, Yasuyo; Ueshima, Masakazu; Matsuo, Takeshi; Izumi, Yukiko; Hosokawa, Akiko; Ikenaka, Yasuhide; Kuriyama, Hideki; Fukui, Hiroshi 抄録: We describe a 19-year-old Japanese woman with isolated ACTH deficiency who improved and delivered a child after 8 years hydrocortisone replacement therapy. Menarche was at the age of 13 y, and menstruation continued regularly. At the age of 16 y general fatigue and amenorrhea developed, and she was referred to our hospital. Her plasma ACTH and cortisol levels were low, and ACTH provocation tests revealed no response. Although the plasma ACTH and cortisol levels were low, dexamethasone suppressed them. Results of other pituitary function tests were normal. Autoantibodies against ACTH-producing cells were identified. She was diagnosed with isolated ACTH deficiency, and we initiated hydrocortisone replacement therapy. Her general 6ondition and anemia were improved, but the amenorrhea persisted. Seven years later, the hydrocortisone was tapered off and menstruation started again. At that time, the test for pituitary autoantibody was negative. One year after fisishl'ng hydrocortisone replacement therapy, she gave birth to a healthy child by natural delivery. This was an atypical case of isolated ACTH deficiency because the ACTH levels were not responsive to provocation tests but were suppressed by dexamethasone. We suggest that the deficiency of ACTH secretion was functional and transient due to the autoimmune mechanism. 1998-04-29T15:00:00Z http://hdl.handle.net/10564/439 タイトル: AN ADULT CASE OF CONGENITAL ADRENAL HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY ASSOCIATED WITH BILATERAL ADRENAL MYELOLIPOMA : AN 8-YEAR OBSERVATION OF CLINICAL CHARACTERISTICS DURING STEROID REPLACEMENT THERAPY 著者: Okamoto, Shingo; Izumi, Yukiko; Hosokawa, Akiko; Matsuo, Takeshi; Sugimoto, Yumi; Tanaka, Yasuyo; Ueshima, Masakazu; Ikenaka, Yasuhide; Kuriyama, Shigeki; Samma, Shoji; Kyumon, Masayuki; Okada, Hideto; Hirao, Yoshihiko; Okajima, Eigoro; Fukui, Hiroshi 抄録: We describe the case of a 44-year-old man with congenital adrenal hyper- plasia (CAH ; 21-hydroxylase deficiency) associated with bilateral adrenal myelolipoma. While undergoing computed tomogaraphy(CT) for the evaluation of persisting flank pain in May 1988, he was diagnosed incidentally as having a bilateral adrenal tumor. Histology of the right extirpated tumor revealed myelolipoma of the adrenal gland, and the other side of the tumor also was diagnosed as myelolipoma by the image diagnostic characteristics. The left side tumor was not extirpated. During examination of hyper-reninism, plasma renin activity (PRA) at 10.5ng/ml/h, he was diagnosed as having congenital adrenal hyperplasia due to elevation of adrenocorticotropin (ACTH), progesterone, and 17 α- hydroxyprogesterone (17 α-OHP). Hydrocortisone with dexamethasone supplemented therapy was started, and the ACTH, progesterone, and 17 α-OHP were normalized, but plasma PRA persisted at high levels. The PRA in this case did not show the degree of sodium loss. During 8 years of observation of the left side myelolipoma by CT, the tumor size did not increase. This case provides an interesting natural history of the clinical characteristics of CAH that went undiagnosed for a long period and an interesting compli- cation of bilateral adrenal myelolipoma. 1998-04-29T15:00:00Z http://hdl.handle.net/10564/438 タイトル: Haemphilus influenzae呼吸器感染症に関する臨床的および基礎的検討 著者: 辻本, 正之 抄録: The present study was performed to analyze the clinical features of respira- tory tract infection due to Hemophilus influenzae (H. influenzae) based on the study of bacteriology using the method of transtracheal aspiration (TTA) and that of outer membrane protein (OMP) profiles by SDS-PAGE. This study included an attempt to isolate the active moiety of cytotoxity against bronchial epithelial cells from H. influenzae. The following results were obtained : (1) In acute respiratory tract infection due to H. influenzae, polymicrobial infection with Streptococcus pneumonae (S. Pneumonae) and Neisseria sp., as well as immunological status of patients were involved in contraction of pneumonia. In bronchitis cases, although many cases were monomicrobial infection, polymicrobial with α-streptococcus was found in some cases. (2) In patients with chronic lower respiratory tract infection (CLRTI) due to H. influen- zae, changes in OMP of this pathogen were closely associated with acute exacerbation of CLRTI. (3) In patients with CLRTI due to H. influenzae, the administration of clarithromycin (CAM) resulted in decrease in serum IL-6 levels during the early phase of treatment. This effect lasted as long as treatment of CAM was continued. (4) The protein with a molecular weight of 14 kDa, as determined by SDS-PAGE, was purified by ion-exchange chromatography, gel-filtration and chromatofocusing, and was found to exhibit cytotoxity against cultured normal human bronchial epithelial cells. These results suggest that respiratory infection caused by H. influenzae includes a variety of clinicopathological features, and that this pathogen may directly cause bronchial epith- elial damage by releasing a cytotoxic protein. Moreover, treatment of CAM may relieve CLRTI patients from sustained inflammation in the airway by supressing IL-6 production. 1998-04-29T15:00:00Z http://hdl.handle.net/10564/433 タイトル: 高度の流出路圧較差を呈したsigmoid septumの1症例 著者: 木村, 麻子; 藤本, 眞一; 水野, 麗子; 中野, 博; 白井, 利彦; 土肥, 和紘 抄録: An 83-year-old woman was referred to our hospital for the treatment of epidermoid pemphigus. She had a history of hypertension since the age of fifty years and had been receiving an anti-hypertensive medication from a private clinic for the last few years. Physical examination showed a systolic ejection murmur (Levine 3/6) at the apex. An electrocardiogram showed high voltage in the left lateral chest leads. A chest roent- genogram showed that the thoracic aorta was severely tortuous. Laboratory data on admission revealed mild anemia and elevation of C-reactive protein. An echocardiogram revealed that the base of the interventricular septum protruded toward the left ventricular outflow tract and showed a sigmoid appearance. The angle between the midline of the interventricular septum and that of the aortic root was 70°. Left ventricular outflow blood velocity was 3.5 m/s, and the outflow pressure gradient appeared to be 50 mmHg. Although a sigmoid septum usually does not give rise to serious clinical problems, a significant left ventricular outflow pressure gradient may develop. 1998-04-29T15:00:00Z