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1.
neurogenetics - Mediator (MED) is a key regulator of protein-coding gene expression, and mutations in MED subunits are associated with a broad spectrum of diseases. Because mutations in MED17... 相似文献
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Haruhito Adam Uchida Yoshio Nakamura Masanobu Kaihara Hisanao Norii Yoshihisa Hanayama Hitoshi Sugiyama Yohei Maeshima Yasushi Yamasaki Hirofumi Makino 《Nephrology, dialysis, transplantation》2006,21(12):3475-3480
BACKGROUND: Decreased plasma adiponectin is associated with impaired endothelial function and, thereby, increased risk for cardiovascular events. Glucocorticoid (GC) affects vascular endothelial cells either favourably or harmfully depending upon the dosages and duration. We examined the effect of GC pulse therapy on vascular endothelial function. METHODS: Fourteen young patients with IgA nephropathy were evaluated for flow-mediated vasodilation (FMD), plasma levels of adiponectin both in high molecular weight (HMW adiponectin) form and in single molecular form (total adiponectin), hepatocyte growth factor (HGF), asymmetric dimethylarginine (ADMA), and high-sensitive C-reactive protein, before and after a course of GC pulse therapy. RESULTS: GC pulse therapy significantly decreased FMD (from 7.2 +/- 2.6 to 5.7 +/- 2.5%, P < 0.01). Meanwhile, plasma adiponectin levels were significantly augmented (total adiponectin: from 10.2 +/- 4.0 to 12.1 +/- 6.3 microg/ml, P < 0.05; HMW: from 6.5 +/- 3.2 to 7.7 +/- 3.3 microg/ml, P < 0.05). In parallel, elevated concentrations of serum HGF (from 0.28 +/- 0.12 to 0.63 +/- 0.38 ng/ml, P < 0.01) and plasma ADMA (from 0.45 +/- 0.07 to 0.53 +/- 0.04 nmol/ml, P < 0.05) were observed. CONCLUSIONS: GC pulse therapy impaired endothelial function while increasing plasma adiponectin levels, which may in turn restore the endothelial function in patients with IgA nephropathy. 相似文献
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Yutaka Okishio Tadayoshi Takeuchi Akikazu Fujita Kiyotaka Suenaga Kaori Fujinami Satoru Munakata Tadashi Takewaki Fumiaki Hata 《Journal of Smooth Muscle Research》2005,41(3):163-174
Recently an essential role of interstitial cells of Cajal (ICC) within myenteric plexus (ICC-MY) was suggested in ascending contraction and descending relaxation in the mouse ileum. The role of ICC in these neural reflexes was examined in the distal colonic segments prepared from the wild type and c-kit mutant, W/W(V) mice, in the present study. Localized distension of the segments from the wild type mice by using a small balloon resulted in ascending contraction and descending relaxation. In the segments from the mutant mice, localized distension also induced these neural reflexes similar to those observed in the wild type mice. Immunohistochemical examination demonstrated that ICC-MY and ICC present in muscle layers (ICC-IM) were severely disrupted in the mutant mouse, but only ICC, present within submucosal plexus (ICC-SMP), remained unchanged. In the small strips with ICC-SMP absent prepared from the mutant mouse, electrical field stimulation induced contraction or relaxation in the absence or presence of atropine, respectively. It was suggested that ICC have no important role in the ascending and descending neural reflexes in the mouse distal colon, this is in direct contrast to the role of ICC-MY in the ileum. 相似文献
6.
Carnitine palmitoyltransferase deficiency in a patient with severe psychomotor retardation 总被引:1,自引:0,他引:1
H Suzuki Y Hirayama S Hirano R Takahashi I Nonaka H Sugie N Sugiyama 《No to hattatsu. Brain and development》1991,23(1):93-97
A 13-year-old girl who had severe brain damage due to unknown prenatal cause presented rhabdomyolysis triggered by a mild viral infection. Her muscle biopsy revealed mild variation in fiber size and type 2 fiber atrophy without excess lipid storage. Biochemical analysis of the biopsied material showed decreased carnitine palmitoyltransferase (CPT) activity (15% of the control). Serum and urinary carnitine levels were normal. Skeletal muscle CT scanning showed multiple low density spots. The patient was diagnosed as having CPT deficiency. She recovered from rhabdomyolysis without renal failure after a month with conservative therapy. CPT deficiency is usually found in young healthy persons. This is the first case report of CPT deficiency which presented severe psychomotor retardation since neonatal period. 相似文献
7.
Minimum incision endoscopic nephrectomy for giant hydronephrosis 总被引:1,自引:0,他引:1
Fumitaka Koga Kazunori Kihara Hitoshi Masuda Yukio Kageyama Satoru Kawakami Tsuyoshi Kobayashi 《International journal of urology》2007,14(8):774-776
Five consecutive patients with symptomatic giant hydronephrosis underwent minimum incision endoscopic nephrectomy. The originally huge renal specimen was retroperitoneally mobilized using both of endoscopy and direct vision, without the use of trocar ports or gas insufflation, via a single minimum incision that narrowly permitted extraction of the specimen. The specimen was successfully extracted from the incision in all patients. Technically, proper deflation of the hydronephrotic sac facilitates mobilization and enables extraction of the specimen. Median (range) size of incision, operative time, and estimated blood loss were 4 cm (3-5), 205 min (156-222), and 210 mL (110-350), respectively. No patient required blood transfusion or encountered operative complications. Postoperative convalescence was short and uneventful; all patients resumed oral intake and ambulance on the day following surgery, and were physically dischargeable from hospital after 2-3 postoperative days. Thus, this technique is a feasible, minimally invasive and safe procedure for symptomatic giant hydronephrosis. 相似文献
8.
Yuhei Okubo Junji Yonese Satoru Kawakami Sinya Yamamoto Yoshinobu Komai Hideki Takeshita Yuichi Ishikawa Iwao Fukui 《International journal of urology》2007,14(9):854-855
Renal cell carcinoma (RCC) causes many kinds of symptoms such as hypercalcemia, hypertension, polycythemia and fever. Here we describe a rare case of RCC presenting with a persistent cough. After radical nephrectomy, the obstinate cough disappeared. When the tumor recurred locally, the cough also recurred. Furthermore, the cough disappeared completely again after the removal of the recurrent tumor. Although all the clinical findings suggested that the RCC caused the cough, we could not identify a specific humoral substance responsible for the cough. 相似文献
9.
Mizuaki Sakura Satoru Kawakami Hitoshi Masuda Tsuyoshi Kobayashi Yukio Kageyama Kazunori Kihara 《International journal of urology》2007,14(12):1109-1112
Since 1998, we have performed minimum incision endoscopic surgery (MIES) for renal cell carcinoma (RCC). For seven dialysis patients with bilateral RCC, we have performed sequential bilateral MIES radical nephrectomy. It was carried out by retroperitoneal approach through a single minimum incision that narrowly permitted extraction of the specimen using endoscopy and direct stereovision, without trocar ports, without gas insufflation and without the insertion of the hands of operators into the operative field. Although six of the seven patients had multiple complications in addition to chronic renal failure (CRF), bilateral kidneys were successfully removed by sequential MIES radical nephrectomy without major operative complication. Postoperative recovery was prompt with all patients resuming oral feeding and walking by the second postoperative day. Sequential bilateral MIES radical nephrectomy, leaving the peritoneal cavity intact and without imposing circulatory stress caused by gas insufflation, is a feasible treatment for bilateral RCCs in dialysis patients. 相似文献
10.
Yoichiro Hosokawa D.D.S. D.D.Sc. Kazuyuki Minowa D.D.S. Satoru Abe M.D. Keiichi Ohmori D.D.S. D.D.Sc. Michio Yamasaki M.D. D.M.Sc. Masanobu Shindoh D.D.S. D.D.Sc. Tadashi Iizuka D.D.S. D.D.Sc. Mohiuddin Ahmed B.D.S. Akira Amemiya D.D.S. D.D.Sc. 《Oral Radiology》1991,7(1):1-6
A case of an odontogenic tumor which invaded the intracranial space from the mandible is reported. Judging from the radiographic
images it was similar to a malignant tumor. The patient died 17 years after the first visit. According to the final pathological
diagnosis, it was malignant odontogenic mixed tumor of low grade which did not belong to any of the WHO classification. 相似文献