NPHS1两种新突变蛋白分子的细胞内分布

目的：研究NPHS1两种新突变编码蛋白在细胞内的分布与先天性肾病综合征发病机制的关系。方法：构建野生型和两种突变型NPHS1克隆，并转染至COS7细胞内，应用免疫荧光双标记的方法，分别进行细胞内及细胞表面的荧光标记，通过共聚焦显微镜对裂隙膜分子Nephrin在细胞内的分布进行研究。结果：野生型Nephrin表现为细胞内和细胞膜染色模式；V822M和C265R则主要为细胞内内质网染色模式，细胞膜着色几乎缺失。结论：突变的Nephrin蛋白由于错误折叠，不能由内质网被输送至细胞表面，这可能是先天性肾病综合征发病的机制之一。     

Successful surgical repair of delayed chronic type a dissection after previous coronary artery bypass grafting.

This reports a case of a 68-year-old woman who had undergone coronary artery bypass 5 years previously. Magnetic resonance angiography (MRA) revealed that the ascending aorta was dilated to about 8 cm in diameter, with type A dissection, and with a patent left internal thoracic artery (LITA) graft to the left anterior descending artery (LAD). Angiography at the ascending aorta did not reveal a coronary artery, nor did it show the sequential saphenous vein graft (SVG) to the obtuse marginal and posterolateral branches. Although the risk of surgical treatment via repeat median sternotomy was very high, we successfully performed the reoperation using profound hypothermic circulatory arrest. The dissection in the mediastinum was facilitated by a sternum retractor for ITA-graft dissection, intraoperative surface echocardiography, and ultrasonic scalpel, with a widely opened bilateral pleural cavity. Furthermore, assuming that most of the myocardium was maintained by perfusion from the in-situ, patent, ITA graft, it was thought that cardioplegia was not necessary during profound hypothermic circulation.     

MANAGEMENT TO PREVENT BLEEDING DURING ENDOSCOPIC SUBMUCOSAL DISSECTION USING THE FLUSH KNIFE FOR GASTRIC TUMORS

The gastric vasculature responsible for intraoperative bleeding in endosocpic submucosal dissection (ESD) is the ramified vascular network occupying the middle of the submucosal layer and large vessels penetrating the muscle layer. Appropriate management for these vessels must be addressed. The trimming of the ramified vascular network can be safely performed with coagulation mode following shallow mucosal cutting. A large penetrating vessel usually requires precoagulation prior to dissection. These procedures are effectively performed with the water jet short needle knife (Flush knife).     

Eosinophilic gastroenteritis in the esophagus, stomach, and small intestine in a patient with a choking feeling in the esophagus]

A 75-year-old man was admitted to our hospital with the chief complaint of a choking feeling around the esophagus. Laboratory examinations revealed eosinophilia, and high levels of serum immunoglobulin (Ig) E. A computed tomography scan (CT) showed wall thickening of the esophagus and terminal ileum, and ascites around the liver. An endoscopic examination revealed mild mucosal edema in the esophagus, stomach, and small intestine. Biopsy specimens showed diffuse eosinophilic infiltration in the mucosa. We therefore diagnosed eosinophilic gastroenteritis. Oral prednisolone relieved clinical conditions and the CT image improved. This case was considered valuable, because there have been few reports of eosinophilic esophagitis in Japan.     

Differential effects of the pyrethroid tetramethrin on tetrodotoxin-sensitive and tetrodotoxin-resistant single sodium channels

The differential effects of the pyrethroid tetramethrin on tetrodotoxin-sensitive (TTX-S) and tetrodotoxin-resistant (TTX-R) single sodium channel currents in rat dorsal root ganglion (DRG) neurons were investigated using the outside-out configuration of patch-clamp technique. Channel conductances were 10.7 and 6.3 pS for TTX-S and TTX-R sodium channels, respectively, at a room temperature of 24–26°C. The single-channel current of TTX-S sodium channels at the test potential of −30 mV was −1.27 ± 0.25 pA, and was not changed after exposure to 10 μM tetramethrin (−1.28 ± 0.23 pA). The open time histogram of TTX-S single-channel currents could be fitted by a single exponential function with a time constant of 1.27 ms. After exposure to 10 μM tetramethrin, the open time histogram could be fitted by the sum of two exponential functions with time constants of 1.36 ms (τ_fast) and 5.73 ms (τ_low). The percentage of contribution of each component to the population was 62% for the fast component representing the normal channels and 38% for the slow component representing the tetramethrin modified channels. The amplitudc of TTX-R single-channel currents was slightly changed from −0.72 ± 0.14 to −0.83 ± 0.07 pA by 10 μM tetramethrin. The open time histogram of TTX-R single-channel currents could be fitted by a single exponential function with a time constant of 1.92 ms. In the presence of 10 μM tetramethrin, the open time histogram could be fitted by the sum of two exponential functions with time constants of 2.07 ms (τ_fast) and 9.75 ms (τ_slow). The percentage of contribution of each component was 15% for the fast, unmodified component and 85% for the slow, modified component. Differential effects of tetramethrin on the open time distribution of single sodium channel currents explains the differential sensitivity of TTX-S and TTX-R sodium channels.     

HTLV-I, HIV-I, and Hepatitis B and C Viruses in Western Province, Papua New Guinea: A Serological Survey

Seven hundred and twenty-three serum samples from individuals in 13 Gidra-speaking villages in Western Province, Papua New Guinea were tested for evidence of infection with human T- lymphotropic virus type I (HTLV-I), human immunodeficiency virus type I (HIV-I), hepatitis B virus (HBV) and hepatitis C virus (HCV). No samples were positive for antibodies to HIV-I. Antibodies to HTLV-I were found in 13 samples (1.8%), HBV surface antigens (HBsAg) were found in 86 samples (11.9%), and antibodies to HCV were found in 30 samples (4.1%). Six (46.2%) of 13 HTLV-I positive samples were positive for HCV or HBsAg. The seropositive rate varied in different villages and the incidence of HTLV-I and HCV was higher in coastal and riverine areas than inland.     

The diagnosis and treatment of esophageal perforations resulting from nonmalignant causes

Esophageal perforations are extremely difficult to diagnose and treat. We report herein our results of a review of 26 patients with esophageal perforation which were spontaneous in 11, iatrogenic in 11, and caused by a foreign body in 4. Surgical treatment was performed in 7 of the patients with spontaneous rupture, but the remaining 19 patients were treated conservatively. The abnormality was found by plain radiography (X-ray) in 22 (85%) of the 26 patients, and by computed tomography (CT) in all 13 patients who underwent this procedure. The detection rates by esophagography and esophagoscopy were 100%, or all of 25 patients examined, and 60%, or 9 of 15 patients examined, respectively. Of 12 patients with underlying diseases, 4 (33%) died after the perforation, whereas only 1 (7%) of 14 patients without any underlying disease died. Postoperative empyema developed in all of 3 patients treated by intraoperative unfixed intrathoracic drainage (UID), but in none of the 4 treated by fixed intrathoracic drainage (FID). Conservative treatment achieved satisfactory results for spontaneous esophageal ruptures confined to the mediastinum, and for iatrogenic perforations and esophageal perforations caused by foreign bodies, provided there was no serious underlying disease such as advanced cirrhosis. Moreover, intraoperative FID proved useful in helping to prevent postoperative empyema.     

Our Distal Aortic Perfusion System in Descending Thoracic and Thoracoabdominal Aortic Aneurysm Repairs

Abstract: We have used heparin-bonded partial cardio-pulmonary bypass to support distal aortic circulation during aortic cross-clamping. However, there were no cardiotomy reservoirs with fully reliable thromboresistance. To resolve this problem, a short-acting anticoagulant (nafamostat mesilate) was added into a cardiotomy reservoir. The present study was designed to evaluate the efficacy of our distal perfusion system. From May 1995 through the end of May 1996, 27 patients underwent descending thoracic and thoracoabdominal aortic aneurysm repairs with this adjunct, 4 being excluded from the experiment. Twenty patients who had undergone conventional partial cardiopulmonary bypass were defined as the control group. There were no significant differences between the 2 groups in the morbidity, mortality, gas transfer, or transfusion requirements despite the fact that more complicated surgical procedures (shown by a two-fold increase in the prevalence of reoperation) were required in the group that had received the current distal perfusion adjunct. the heparin-bonded group. In conclusion, our perfusion system is very effective for descending thoracic and thoracoabdominal aortic aneurysm repairs.     

Ross procedure in an infant weighing 4.5 kg

A 6 month-old male infant (weight: 4.5 kg) with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft (Ross procedure). The right ventricular outflow tract (RVOT) was reconstructed with a polytetrafluoroethylene (PTFE)-valved equine pericardial conduit. At the age of 5, re-RVOT reconstruction with an equine pericardial patch bearing a PTFE monocusp was required because of severe pulmonary stenosis resistant to 2 attempts of percutaneous transluminal pulmonary valvotomy. Currently, at the age of 8, the degree of aortic regurgitation is trivial and the pulmonary autograft is free of functional deterioration despite somatic growth.