Development of alternative gene transfer techniques for and gene therapy in a canine model.

Introduction: Gene therapy have recently attracted much attention as a curative therapeutic option for inherited single gene disorders such as hemophilia. Hemophilia is a hereditary bleeding disorder caused by the deficiency of clotting activity of factor VIII (FVIII) or factor IX (FIX), and gene therapy for hemophilia using viral vector have been vigorously investigated worldwide. Toward further advancement of gene therapy for hemophilia, we have previously developed and validated the efficacy of novel two types of gene transfer technologies using a mouse model of hemophilia A.

Use of preoperative haemostasis and ABO blood typing tests in children: a retrospective observational study using a nationwide claims database in Japan.

Objectives: To describe the prevalence and factors associated with preoperative haemostasis and ABO blood typing tests for children because these tests might represent low-value care.

Design: A retrospective observational study.

Setting: Nationwide insurance claims database in Japan.

Prevalence and associated factors of preoperative functional disability in elective surgical patients over 55 years old: a prospective cohort study.

Purpose: In preoperative settings, patients may have functional disabilities due to the disease for which surgery is being performed or comorbidities, but the associated and predictive factors remain unknown. This study examined the prevalence of preoperative functional disability and clarified the associated factors.

Methods: Individuals aged ≥ 55 years who were scheduled to undergo surgery in a tertiary-care hospital in Japan between April 2016 and September 2016 were eligible for enrolment in the study.

Feasibility, reliability, and validity of the Japanese version of the 12-item World Health Organization Disability Assessment Schedule-2 in preoperative patients.

Purpose: The avoidance of postoperative functional disability is one of the most important concerns of patients facing surgery, but methods to evaluate disability have not been definitively established. The aim of our study was to evaluate the feasibility, reliability, and validity of the Japanese version of the 12-item World Health Organization Disability Assessment Schedule-2 (WHODAS 2.0-J) in preoperative patients.

Functional characterization of tissue factor in von Willebrand factor-dependent thrombus formation under whole blood flow conditions.

Von Willebrand factor (VWF) plays an important role in mediating platelet adhesion and aggregation under high shear rate conditions. Such platelet aggregates are strengthened by fibrin-network formation triggered by tissue factor (TF). However, little is known about the role of TF in VWF-dependent thrombus formation under blood flow conditions.

Coagulation potential of immobilised factor VIII in flow-dependent fibrin generation on platelet surfaces.

Coagulation factor VIII (FVIII) plays an essential role in haemostasis. To date, physiologic activity of FVIII circulating in the bloodstream (S-FVIII) is evaluated by classic coagulation assays. However, the functional relevance of FVIII (-von Willebrand factor complex) immobilised on thrombogenic surfaces (I-FVIII) remains unclear.

[Epidural blood patch for postdural puncture headache in a five-year-old child].

A case was presented of a 5-year-old girl who suffered an accidental dural puncture during placement of an epidural catheter under general anesthesia for orthopedic surgery. She complained of headache 4 days after the operation, which was relieved on supine position but became worse on sitting position. Her symptoms failed to respond to conservative management.

[Comparison of thoracoscopic and open repair of esophageal atresia with tracheoesophageal fistula].

Background: With the increasing use of endoscopic surgery in children, several papers report the comparison between the thoracoscopic and open repair of the neonatal esophageal atresia with tracheoesophageal fistula (EA/TEF). Most of them focus on the duration and outcome of the surgery with few focusing on the neonatal tolerance to the thoracoscopic procedure and intraoperative anesthetic management.

Methods: We retrospectively reviewed the repair surgery of EA/TEF performed during 2001 and 2006 in our institution and compared thoracoscopic repair (thoracoscopy group, n=5) with open repair (open group, n=7).

[General anesthesia for a girl with Joubert syndrome].

Joubert syndrome is a rare autosomal recessive disorder, which is characterized by absence or underdevelopment of the cerebellar vermis and severe developmental delay. The other common features include ataxia, an abnormal breathing pattern, abnormal eye movements and hypotonia. We report the anesthetic management in a 13-year-old girl with Joubert syndrome, scheduled for cauterization of nasal mucosa under general anesthesia.

[Recovery from fatal ventricular fibrillation after immediate application of percutaneous cardiopulmonary support].

We present a patient who recovered from refractory ventricular fibrillation after immediate application of percutaneous cardiopulmonary support (PCPS). On the postoperative day (POD) 3 after the Y-grafting surgery for abdominal aortic aneurysm, circulation collapsed due to sudden onset of ventricular fibrillation. Because ventricular fibrillation had persisted in spite of medical treatment and defibrillation, we established PCPS and his circulation recovered.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine pretreatment attenuates methamphetamine-induced dopamine toxicity.

The effects of pretreatment with MPTP (1-methyl4-phenyl-1,2,3,6-tetrahydropyridine) on the acute and long-term effects of methamphetamine on striatal dopamine were evaluated in BALB/c mice. Four subcutaneous injections of a non-toxic dose of MPTP (8 mg/kg, at 2 hr intervals) were followed three days later by a toxic regimen of methamphetamine (four injections of 4 mg/kg, at 2 hr intervals) and mice were sacrificed immediately or three days later. Control mice received saline in place of the MPTP or methamphetamine and mice were observed for acute changes in body temperature, self-injurious behaviour, and striatal dopamine metabolites, or long-term changes in striatal dopamine levels, tyrosine hydroxylase immunoreactivity and glial fibrillary acidic protein.