Acute presentation of autoimmune hepatitis: a multicentre study with detailed histological evaluation in a large cohort of patients.

Aims: Although liver biopsy is crucial to diagnose and guide treatment decisions, a detailed histological analysis of autoimmune hepatitis (AIH) with clinically acute presentations has not yet been performed. This study aimed to characterise the histological features and explore potential histological hallmarks to diagnose the acute presentation of AIH.

Methods: We systematically evaluated liver specimens of 87 adult patients with acute presentation of AIH retrospectively enrolled from Japanese multicentre facilities.

Autoimmune Hepatitis Associated with Immune Thrombocytopenic Purpura.

Although autoimmune hepatitis (AIH) is frequently complicated with chronic thyroiditis or other autoimmune disorders, reports on its association with immune thrombocytopenic purpura (ITP) are scarce. We herein describe a case of AIH associated with ITP. A 75-year-old Japanese woman was admitted to our hospital due to increased aminotransferase levels and severe thrombocytopenia.

Genome-wide methylation profiles in primary intracranial germ cell tumors indicate a primordial germ cell origin for germinomas.

Intracranial germ cell tumors (iGCTs) are the second most common brain tumors among children under 14 in Japan. The World Health Organization classification recognizes several subtypes of iGCTs, which are conventionally subclassified into pure germinoma or non-germinomatous GCTs. Recent exhaustive genomic studies showed that mutations of the genes involved in the MAPK and/or PI3K pathways are common in iGCTs; however, the mechanisms of how different subtypes develop, often as a mixed-GCT, are unknown.

Clinical analysis of patients treated with afatinib for advanced non-small cell lung cancer: A Nagano Lung Cancer Research Group observational study.

Background: Afatinib has been available in Japan for the treatment of epidermal growth factor receptor (EGFR)-mutated non-small cell lung cancer (NSCLC) since May 2014. We conducted an observational study in patients treated with afatinib in Nagano prefecture, focusing on response and associated toxicities.

Methods: We analyzed the clinical records of NSCLC patients treated with afatinib between May 2014 and February 2015.

Efficacy and Safety of Percutaneous Coronary Intervention for Elderly Patients in the Second-Generation Drug-Eluting Stent Era: The SHINANO Registry.

Background: We evaluated the 1-year outcomes of percutaneous coronary intervention (PCI) for elderly patients (aged ≥ 80 years) in the second-generation drug-eluting stent (DES) era.

Methods And Results: Between August 2012 and July 2013, 1923 consecutive patients (mean age, 71 ± 11 years; ≥80 years, 23%; men, 77%) who underwent 2250 elective/urgent PCI procedures were enrolled in the Shinshu Prospective Multicenter Analysis for Elderly Patients with Coronary Artery Disease Undergoing Percutaneous Coronary Intervention registry. The primary end point was major adverse cardiovascular events (MACEs; cardiovascular death, myocardial infarction, and stroke) at 1 year.

Autoimmune hepatitis in Japan: trends in a nationwide survey.

Background: A nationwide survey of autoimmune hepatitis (AIH) patients was performed in Japan in 2015. The aims of this study were to elucidate the trends and characteristics of AIH in Japan, in addition to identifying differences in AIH between acute hepatitis and chronic hepatitis.

Methods: Questionnaires about patients with AIH diagnosed from 2009 to 2013 were sent to 437 hospitals or clinics with hepatology specialists.

Prediction of 1-year clinical outcomes using the SYNTAX score in patients with prior heart failure undergoing percutaneous coronary intervention: sub-analysis of the SHINANO registry.

Although coronary artery disease (CAD) is common in patients with heart failure (HF), little is known about the prognostic significance of coronary lesion complexity in patients with prior HF undergoing percutaneous coronary intervention (PCI). The aim of this study was to investigate whether the coronary Synergy between Percutaneous Coronary Intervention with TAXus and Cardiac Surgery (SYNTAX) score could improve risk stratification in HF patients with CAD. Two hundred patients (mean age 73 ± 11 years, left ventricular ejection fraction 49 ± 15 %) with prior HF who underwent PCI were divided into two groups stratified by SYNTAX score (median value 12) and tracked prospectively for 1 year.

Genetic Association of PTPN22 Polymorphisms with Autoimmune Hepatitis and Primary Biliary Cholangitis in Japan.

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are liver-specific autoimmune conditions that are characterized by chronic hepatic damage and often lead to cirrhosis and hepatic failure. Specifically, the protein tyrosine phosphatase N22 (PTPN22) gene encodes the lymphoid protein tyrosine phosphatase, which acts as a negative regulator of T-cell receptor signaling. A missense single nucleotide polymorphism (SNP) (rs2476601) in PTPN22 has been linked to numerous autoimmune diseases in Caucasians.

Variants associated with autoimmune Type 1 diabetes in Japanese children: implications for age-specific effects of cis-regulatory haplotypes at 17q12-q21.

Aims: The aim of this study was to clarify the significance of previously reported susceptibility variants in the development of autoimmune Type 1 diabetes in non-white children. Tested variants included rs2290400, which has been linked to Type 1 diabetes only in one study on white people. Haplotypes at 17q12-q21 encompassing rs2290400 are known to determine the susceptibility of early-onset asthma by affecting the expression of flanking genes.

[Bilateral Testicular Tumor after Bilateral Orchiopexy : A Case Report].

We report a case of bilateral testicular tumor after bilateral orchiopexy. A 42-year-old man who underwent bilateral orchiopexy in early childhood consulted our hospital due to right testicular enlargement in February 2012. Blood tests revealed elevation of human chorionic gonadotropin β, and a right testicular tumor was suspected.

Comparison of mid-term outcomes between patients with and without atrial fibrillation undergoing coronary stenting in the second-generation drug-eluting stent era: from the SHINANO registry.

Little is known about the mid-term outcomes of patients with atrial fibrillation (AF) who undergo coronary stenting in the second-generation drug-eluting stent (DES) era. We evaluated the 1-year outcomes of AF patients undergoing percutaneous coronary intervention (PCI) with second-generation DES. This retrospective cohort analysis used integrated data from the SHINANO registry, a prospective observational multicenter cohort study, which enrolled 1923 consecutive patients undergoing PCI for any coronary artery disease.

Markedly elevated CD64 expression on neutrophils and monocytes as a biomarker for diagnosis and therapy assessment in Kawasaki disease.

Objective: Kawasaki disease (KD) is the most commonly encountered inflammatory disease in children. However, its pathogenesis and diagnostic biomarkers have not been fully investigated. We examined the activation of neutrophils and monocytes in KD.

Recurrent neomorphic mutations of MTOR in central nervous system and testicular germ cell tumors may be targeted for therapy.

Germ cell tumors constitute a heterogeneous group that displays a broad spectrum of morphology. They often arise in testes; however, extragonadal occurrence, in particular brain, is not uncommon, and whether they share a common pathogenesis is unknown. We performed whole exome sequencing in 41 pairs of central nervous system germ cell tumors (CNS GCTs) of various histology and their matched normal tissues.

Incidence and prevalence of autoimmune hepatitis in the Ueda area, Japan.

Aim: Although autoimmune hepatitis (AIH) is considered to be rare in Japan, precise data on the incidence and prevalence of this disease are scarce due to the lack of a nationwide registry. We therefore conducted a study of these factors over a secondary medical care area.

Methods: We retrospectively investigated the medical records of AIH patients seen during 2004-2009 and prospectively recruited subjects from 2010 to 2014 at our hospital.

Dendritic cell-based immunotherapy targeting Wilms' tumor 1 in patients with recurrent malignant glioma.

Object: Dendritic cell (DC)-based vaccination is considered a potentially effective therapy against advanced cancer. The authors conducted a Phase I study to investigate the safety and immunomonitoring of Wilms' tumor 1 (WT1)-pulsed DC vaccination therapy for patients with relapsed malignant glioma.

Methods: WT1-pulsed and/or autologous tumor lysate-pulsed DC vaccination therapy was performed in patients with relapsed malignant gliomas.

Postoperative complications in elderly patients with gastric cancer.

Background: Elderly patients undergoing gastrectomy are expected to be at high risk of postoperative complications. This retrospective multicenter cohort study assessed complications and long-term outcomes after gastrectomy for gastric cancer (GC).

Methods: A total of 993 patients with GC who had undergone gastrectomy were included, comprising 186 elderly patients (age ≥ 80 y, E group) and 807 nonelderly patients (age ≤ 79 y, NE group).

Myositis ossificans occupying the thenar region: a case report.

Introduction: Myositis ossificans is a benign, self-limiting, tumor-like lesion that usually affects the elbow and thigh; occurrence in the hand is uncommon. We report a rare case of a patient with myositis ossificans in the thenar region.

Case Presentation: A 15-year-old Japanese girl presented to our hospital with a 2-month history of a painful mass in the right thenar region without previous trauma.

Clinical outcome of deep-seated atypical lipomatous tumor of the extremities with median-term follow-up study.

Aims: There is no consensus on the best surgical treatment for deep-seated atypical lipomatous tumor (ALT) of the extremities; furthermore, the appropriate duration for follow-up observation remains unclear. We investigated clinical and functional median-term outcomes in the primary operations for ALT of the extremities in order to find its best treatment methods and observation periods.

Methods: From 1996 to 2009, we diagnosed 41 patients with deep-seated ALT of the extremities.

Giant cell tumor of the tendon sheath extending around the patellar tendon and invading the knee joint and tibia: A case report.

The current report presents the case of a 41-year-old male exhibiting a giant cell tumor of the tendon sheath (GCT-TS) arising from the patellar tendon sheath. Plain radiography and magnetic resonance imaging revealed a well-localized mass that wrapped around the patellar tendon, and extended from the subcutis into the infrapatellar fat pad and tibia. Following histopathological determination of the diagnosis, a piecemeal resection was performed.

A case of adult Dent disease in Japan with advanced chronic kidney disease.

Dent disease is an inherited tubulopathy caused by a mutation in the CLCN5 chloride channel gene. In cases of Dent disease in Japan (Japanese Dent, J-Dent), renal function is generally preserved and rarely progresses to advanced kidney dysfunction. However, the long-term prognosis of J-Dent remains unknown.

In vitro expansion of CD34(+)CD38(-) cells under stimulation with hematopoietic growth factors on AGM-S3 cells in juvenile myelomonocytic leukemia.

Using serum-containing culture, we examined whether AGM-S3 stromal cells, alone or in combination with hematopoietic growth factor(s), stimulated the proliferation of CD34(+) cells from patients with juvenile myelomonocytic leukemia (JMML). AGM-S3 cells in concert with stem cell factor plus thrombopoietin increased the numbers of peripheral blood CD34(+) cells to approximately 20-fold of the input value after 2 weeks in nine JMML patients with either PTPN11 mutations or RAS mutations, who received allogeneic hematopoietic transplantation. Granulocyte-macrophage colony-stimulating factor (GM-CSF) also augmented the proliferation of JMML CD34(+) cells on AGM-S3 cells.

[Two cases of lower abdominal tumors difficult to differentiate from urachal tumors].

Case 1: A 28-year-old woman visited a local medical doctor, complaining of abdominal pain, urinary frequency and a sense of residual urine. Magnetic resonance imaging revealed a lower abdominal extraperitoneal tumor, approximately 5 cm in diameter, adjacent to the bladder dome. It was thought to be a urachal tumor, and she was referred to our hospital.

Colonic diverticular hemorrhage: the hood method for detecting responsible diverticula and endoscopic band ligation for hemostasis.

Background And Study Aims: Although colonic diverticular hemorrhage is a common cause of lower gastrointestinal bleeding, the low rate of detection of the diverticula responsible for bleeding, together with inadequate evaluation of endoscopic hemostasis, remain unsatisfactory.

Patients And Methods: Over 3 years, we employed the hood method to diagnose diverticular hemorrhage in 53 patients and applied endoscopic band ligation (EBL) for hemostasis in 27 patients with responsible diverticula.

Results: The hood method revealed active bleeding in 13 patients (24.