Because of the rapid progress in genetic research, only a small part of what is known about clinical genetics is taught in medical schools. At Nippon Medical School a clinical genetics course for fourth-year students started in October 2002. In the present study, we used a questionnaire to investigate how well second-year medical school students understand clinical genetics. The results of the questionnaire suggested that many students are confused about basic concepts in clinical genetics, such as the differences between inherited disease and diseased caused by genetic disorders and between hereditary and chromosomal disorders and also suggested that education in clinical genetics is needed in medical schools. In the United States, guidelines for teaching genetics in medical schools have been established. However, in Japan, considering the lack of consensus about medical genetics terminology, to disseminate correct knowledge about clinical genetics, the present situation of medical genetics education in medical schools must be clarified, and various other measures, such as establishing what information should be taught, should be carried out.

During past 15 years 78 patients were operated for thoracic aortic aneurysm. Patients operated in emergency or dead within 24 hours after operation or with preoperative renal failure were excluded and remaining 65 patients were studied for factors affecting postoperative renal dysfunction. Postoperative renal dysfunction was based on the serum creatinine value which was within normal limit before operation and exceeded 1.5mg/dl after operation, or which increased by 1mg/dl and more from preoperative value. 23 patiens developed postoperative renal dysfunction and the incidence was 35.4%. As preoperative factors, old age, male and high value of preoperative serum creatinine were significantly (p<0.01) related with postoperative renal dysfunction. As intraoperative factor, decreased urine output per operative hour was significantly (0.01<p<0.05) related. Other preoperative factors; hypertension, diabetes, location of aneurysm, dissecting and nondissecting, intraoperative factors; operation time, volume of operative bleeding, minimum systolic blood pressure during operation, clamping time of aorta, minimum temperature of rectum, difference of adjuncts (temporary shunt or extracorporeal circulation), postoperative factors; systolic blood pressure at arriving ICU, urine output of first postoperative day were not significantly related. Between the operative procedures of graft replacement and extraanatomic bypass, no significant difference was recognized in occurrence of postoperative renal dysfunction, but patients with patch angioplasty etc. developed no renal dysfunction. In the complications within one week after operation, central nervous system dysfunction, infection and hemorrhage had a tendency to occur together with renal dysfunction. For prevention of postoperative renal dysfunction it is important to minimize the renal ischemia, to protect the kidney and to maintain urine output during operation, particularly in patients of preoperative decreased function of kidney and of old male with advanced arteriosclerosis. Also it is necessary to choose the less invasive procedure of operation for patients of severely decreased function of kidney and to consider about organ system relations in patients of postoperative renal dysfunction.

Isolated left-side inferior vena cava is rare, there being only four cases associated with abdominal aortic aneurysm reported so far in the Japanese literature. A 72-year-old man was admitted to our hospital for the evaluation of an abdominal pulsatile mass. CT scan revealed abdominal aortic aneurysm with isolated left-sided inferior vena cava. Aneurysmectomy and bifurcated graft replacement was performed with retracting inferior vena cava. The postoperative course was uneventful.

Objective: Acanthopanax senticosus Harms extract (ASE) is an ingredient of functional foods, such as health supplements, in Japan. We investigated the effects of ASE on CYP2C9 activity.
Methods and Results: CYP2C9-catalyzed diclofenac 4′-hydroxylase activities in human intestinal and liver microsomes (abbreviated as HIM and HLM, respectively) were significantly decreased by the addition of ASE in a concentration-dependent manner. Kinetic studies of diclofenac 4′-hydroxylase in HLM revealed that ASE addition significantly decreased V_max but had no effect on K_m. These results suggest that diclofenac 4′-hydroxylase activity is suppressed by ASE addition in a non-competitive manner. Then, we investigated the time courses of diclofenac 4′-hydroxylase activity in rat liver microsomes after ASE oral administration (50 to 400 mg/kg). Diclofenac 4′-hydroxylase activities were significantly lowered by the administration of 200 and 400 mg/kg ASE at 0.5 to 4 hr compared with control (0 hr). Furthermore, we investigated the effects of ASE oral administration on the pharmacokinetics of tolbutamide (substrate for CYP2C9) in rats. The area under the concentration-time curve of tolbutamide after ASE oral administration (400 mg/kg) was enhanced by approximately 1.6 times compared with that without ASE oral administration.
Conclusion: These findings indicated that ASE inhibits human intestinal and hepatic CYP2C9 activities.

We report the initial results of thoracic endovascular repair using the Gore TAG device (TAG) used in treatment of thoracic aortic aneurysms (TAA), and evaluate initial outcome based on the Japan SCORE (JS) system. From August 2008 to July 2009, thoracic aortic endovascular repair (TEVAR) for TAA was applied in 27 cases (men/women, 22/5, 53-88 years old, mean age 70.5). Locations included the distal arch in 7 cases, proximal descending TAA (dTAA) in 12 cases and middle or distal dTAA in 8 cases. Deployment of a stent-graft (SG) was successful in 27 cases (100%) and complete thrombosis of the aneurysm or complete entry closure was achieved in 26 cases (96.3%). There was 1 type 2 endoleak (3.7%), 2 iliac arterial injuries (7.4%) and 2 cases of temporary hemodialysis (7.4%). There was no occurrence of paraplegia or hospital death. The 30-day mortality rate and major complication rate examined by the Japan SCORE (JS) system did not show any statistical differences between the TEVAR group and the open repair (OR) group, however the data were higher in the TEVAR group, although not statisfically in the OR group. The OR group had a high complication incidence in comparison with the TEVAR group. Based on evaluation by the JS system, the initial results suggest that TAG for the treatment of TAA is superior to conventional open surgery.

Between August 2008 and June 2012, 17 TEVAR procedures for thoracic aortic aneurysms (TAA) requiring Z2 coverage were performed at our institution. Patient age ranged from 46 to 82 years old (mean 69.4), 16 were male. Criteria for LSA revascularization at our institution are defined as either : 1) dominant left vertebral artery (VA), 2) absent or diminutive or occluded right VA, 3) no communication of bilateral VA, 4) bilateral carotid artery disease, 5) patent LIMA-coronary bypass, 6) if a long length of the thoracic aorta is covered. Devices utilized were Gore TAG (n=12) and TX2 (n=5). Deployment of the stent-graft (SG) was successful in 17 cases (100%) and complete thrombosis of the aneurysm or complete entry closure was achieved in 16 cases (94.1%). Axillo-axillar cross over bypass (Ax-Ax B) was performed in 5 cases (29.4%). There was no instance of cerebrospinal ischemia or hospital death and the mean follow-up was 22.9 month (range 5 to 46). One case was converted to open surgery due to secondary type 1 endoleak. There was no instance of Ax-Ax B graft occlusion or aneurysmal rupture. The initial and mid-term results of TEVAR requiring Z2 coverage were satisfactory, and we believe that our criteria for LSA revascularization played an important role in providing the satisfactory results.

A 54-year-old male with sudden back pain was diagnosed as having acute aortic dissection of Stanford type A. He underwent an aortic arch replacement under the deep hypothermic circulatory arrest and retrograde cerebral perfusion. During retrograde cerebral perfusion, the central venous pressure was maintained at 20mmHg, the perfusion flow rate was 400ml/min and the lowest rectal temperature was 19°C. The duration of retrograde cerebral perfusion was 135 min, but the patient recovered successfully without any evidence of neurological complications. This report suggests that retrograde cerebral perfusion associated with deep hypothermic circulatory arrest has the possibility to prolong the safety time limit of antegrade cerebral circulatory arrest up to 135min.

A 62-year-old woman presented with acute chest pain. An enchanced CT scan showed type A closing aortic dissection. An ulcer-like projection (ULP) was observed in the abdominal aorta above the superior mesenteric artery on aortography. At 3 months after onset, recurrent chest pain appeared. An enchanced CT scan showed a false lumen in the ascending aorta and a new ULP and localized false lumen were opacified in the distal ascending aorta on aortography. The graft replacement of the ascending aorta was performed using open distal anastomosis under circulatory arrest and retrograde cerebral perfusion. Two intimal tears were found in the aortic root and distal ascending aorta. The patient recovered without complications. Postoperative CT scan and aortography revealed no residual false lumen.