Zinc monotherapy for young children with presymptomatic Wilson disease: A multicenter study in Japan.

Background And Aim: Few studies of zinc monotherapy for presymptomatic Wilson disease have focused on young children. We therefore evaluated long-term efficacy and safety of zinc monotherapy for such children and established benchmarks for maintenance therapy.

Methods: We retrospectively and prospectively examined children under 10 years old with presymptomatic Wilson disease who received zinc monotherapy from time of diagnosis at 12 participating pediatric centers in Japan.

Variations of placental migration in patients with early third trimester malposition.

Aim: This study aimed to investigate the impact of placental migration on the definitive prepartum diagnosis of patients with placenta previa (PP) and low-lying placenta (LLP) after late preterm.

Methods: This was a retrospective cohort study of singleton pregnancies with PP and LLP diagnosed at 30-33 weeks of gestation. We assessed the rate of changes in transvaginal ultrasonographic measurements of placental position during the period from 34 to 38 weeks of gestation.

Costal cartilage transplantation for treatment of growth plate injury in a rabbit model.

Objectives: The purpose of this study is to evaluate the effects of allogenic costal cartilage transplantation on preventing bony bridge formation and angular deformities for the treatment of partial growth plate injury using a rabbit model.

Methods: An experimental model of partial growth injury was created by resecting the medial part of the proximal tibial growth plate in male six-week-old New Zealand White rabbits. The rabbits were divided into four groups: no surgery; no transplantation; bone wax transplantation; and allogenic costal cartilage transplantation.

Safety Evaluation of Trastuzumab Emtansine in Japanese Patients with HER2-Positive Advanced Breast Cancer.

Background/aim: Tolerability and safety of trastuzumab emtansine (T-DM1) was investigated in Japanese patients with HER2-positive advanced breast cancer who were previously treated with chemotherapy and trastuzumab.

Patients And Methods: Patients with inoperable or recurrent breast cancer who were previously treated with chemotherapy and trastuzumab in adjuvant and/or metastatic disease were included. T-DM1 3.

Standardization of endoscopic resection for colorectal tumors larger than 10 mm in diameter.

Currently, several endoscopic resection (ER) methods for colorectal tumors are available, including polypectomy, conventional endoscopic mucosal resection (EMR), endoscopic submucosal dissection (ESD), and modified ER. In the present review, we mainly focus on the current status of ER for colorectal tumors as well as the report from the Endoscopic Forum Japan (EFJ) 2016, which was held in Tokyo in August 2016. The proposed ER methods for colorectal tumors larger than 10 mm in diameter are as follows.

Different time trend and management of esophagogastric junction adenocarcinoma in three Asian countries.

Esophagogastric junction (EGJ) adenocarcinoma has been on the increase in Western countries. However, in Asian countries, data on the incidence of EGJ adenocarcinoma are evidently lacking. In the present review, we focus on the current clinical situation of EGJ adenocarcinoma in three Asian countries: Japan, Hong Kong, and Malaysia.

Considering the esophagogastric junction as a 'zone'.

Siewert's classification of adenocarcinoma of the esophagogastric junction (EGJ) classifies tumors anatomically for determining the appropriate surgical technique. According to this classification, a type II tumor, true carcinoma of the cardia, is defined as a cancer within 1 cm proximal to 2 cm distal of the EGJ. Histological analysis indicates that the cardiac gland is present with a high degree of frequency between 1-2 cm to the gastric side and 1-2 cm to the esophageal side of the EGJ, which means that this zone can be considered as neither the stomach nor the esophagus but rather as a third zone known as the 'EGJ zone'.

Endoscopic gastric mucosal atrophy distinguishes the characteristics of superficial esophagogastric junction adenocarcinoma.

Background And Aim: Western studies have suggested two distinct etiologies of esophagogastric junction (EGJ) cancer: Helicobacter pylori-associated atrophic gastritis and non-atrophic gastric mucosa resembling esophageal adenocarcinoma. The present study investigated whether endoscopic gastric mucosal atrophy can distinguish between these two types of EGJ adenocarcinoma.

Methods: Data were collected from patients with Siewert type II, T1 EGJ adenocarcinoma who underwent endoscopic or surgical resection at eight Japanese institutions in 2010-2015.

Endoscopic findings of esophagogastric junction in children.

Esophagogastric landmarks are recognizable in the same way both in children and in adults, and palisade-shaped vessels can be observed at the distal position of esophageal mucosa, even in infants. Few studies have been done in respect to Barrett's esophagus (BE) in children. Incidence of endoscopically suspected BE among all children undergoing esophagogastroduodenoscopy (EGD) is approximately 0.

Characteristics and prognosis of rectal gastrointestinal stromal tumors: an analysis of registry data.

Purpose: Rectal gastrointestinal stromal tumors (GISTs) are rare. Accordingly, their clinical features are not well-documented and optimal treatment has not been established. The objective of this study is to clarify the rates and patterns of recurrence after surgical resection of rectal GISTs, with a focus on outcomes and therapeutic modalities.

Five-year survival analysis of surgically resected gastric cancer cases in Japan: a retrospective analysis of more than 100,000 patients from the nationwide registry of the Japanese Gastric Cancer Association (2001-2007).

Background: The aim of this retrospective study was to investigate the tumor characteristics, surgical details, and survival distribution of surgically resected cases of gastric cancer from the nationwide registry of the Japanese Gastric Cancer Association.

Methods: Data from 118,367 patients with primary gastric carcinoma who underwent resection between 2001 and 2007 were included in the survival analyses. The 5-year survival rates were calculated for various subsets of prognostic factors.

Global Cardiovascular and Renal Outcomes of Reduced GFR.

The burden of premature death and health loss from ESRD is well described. Less is known regarding the burden of cardiovascular disease attributable to reduced GFR. We estimated the prevalence of reduced GFR categories 3, 4, and 5 (not on RRT) for 188 countries at six time points from 1990 to 2013.

Decreased cohesin in the brain leads to defective synapse development and anxiety-related behavior.

Abnormal epigenetic regulation can cause the nervous system to develop abnormally. Here, we sought to understand the mechanism by which this occurs by investigating the protein complex cohesin, which is considered to regulate gene expression and, when defective, is associated with higher-level brain dysfunction and the developmental disorder Cornelia de Lange syndrome (CdLS). We generated conditional -knockout mice and observed greater dendritic complexity and larger numbers of immature synapses in the cerebral cortex of mice.

Laparoscopic and luminal endoscopic cooperative surgery can be a standard treatment for submucosal tumors of the stomach: a retrospective multicenter study.

 We have developed a combined laparoscopic and luminal endoscopic surgery technique for resection of gastric submucosal tumors (SMTs) that can be performed without excessive resection of the stomach. In a multicenter retrospective study we aimed to evaluate the feasibility and safety of laparoscopic and endoscopic cooperative surgery (LECS) for gastric SMT resection. Between October 2007 and December 2011, 126 patients with gastric SMTs underwent LECS at 8 institutions.

Novel MCA/ID syndrome with ASH1L mutation.

We identified a novel mutation in ASH1L in a patient with severe intellectual disability, growth failure, microcephaly, facial dysmorphism, myelination delay, and skeletal abnormalities. ASH1L is a histone methyltransferase that associates with the transcribed region of all active genes examined, including Hox genes. It catalyzes H3K36 methylation and plays important roles in development.

Multiple therapeutic peptide vaccines for patients with advanced gastric cancer.

We performed a clinical trial using HLA-A24-binding peptide vaccines containing a combination of novel cancer-testis antigens and anti-angiogenic peptides for advanced gastric cancer (GC). Thirty-five GC patients who had shown resistance to the standard therapy were enrolled in this clinical trial using vaccinations with a mixture of multiple peptides derived from DEPDC1, URLC10, FoxM1, Kif20A and VEGFR1. The safety, the overall survival (OS), and the immunological responses based on an ELISPOT assay were determined to assess differences in patients who were HLA-A24-positive [24(+)] and HLA-A24-negative [24(-)].

Outcome of hematopoietic cell transplantation for DNA double-strand break repair disorders.

Background: Rare DNA breakage repair disorders predispose to infection and lymphoreticular malignancies. Hematopoietic cell transplantation (HCT) is curative, but coadministered chemotherapy or radiotherapy is damaging because of systemic radiosensitivity. We collected HCT outcome data for Nijmegen breakage syndrome, DNA ligase IV deficiency, Cernunnos-XRCC4-like factor (Cernunnos-XLF) deficiency, and ataxia-telangiectasia (AT).

FDG-PET predicts treatment efficacy and surgical outcome of pre-operative chemoradiation therapy for resectable and borderline resectable pancreatic cancer.

Background: The efficacy of neoadjuvant chemoradiotherapy (NACRT) for resectable and borderline resectable pancreatic cancer is important for predicting outcomes after radical surgery, but few clinical indicators predict outcome before resection. This study examined the utility of FDG-PET in predicting the efficacy of NACRT and outcome after radical surgery.

Methods: Eighty-three pancreatic cancer patients who underwent FDG-PET before and after NACRT and had positive standard uptake values (SUVs) before NACRT were enrolled in this study.