Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort.

Background: The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort.

Aims: To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP.

Methods: We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP.

U2AF - Hypoxia-induced fas alternative splicing regulator.

The splicing machinery heavily contributes to biological complexity and especially to the ability of cells to adapt to altered cellular conditions. Hypoxia also plays a key role in the pathophysiology of many disease states. Recent studies have revealed that tumorigenesis and hypoxia are involved in large-scale alterations in alternative pre-mRNA splicing.

B-Mode Ultrasonography versus Contrast-Enhanced Ultrasonography for Surveillance of Hepatocellular Carcinoma: A Prospective Multicenter Randomized Controlled Trial.

Background: Current practice guidelines recommend the use of ultrasound (US) as an initial surveillance tool for hepatocellular carcinoma (HCC) in patients with liver cirrhosis. Patients with liver cirrhosis, however, frequently have coarse liver parenchyma, masking the presence of tiny nodules during B-mode US. Contrast-enhanced US (CEUS) with Sonazoid has a long-lasting, stable Kupffer phase, which makes it possible to scan the entire liver to depict small lesions.

Absence of Adverse Neurological Outcomes in a Non-Neurologically Injured Polytrauma Patient Despite Extreme and Prolonged Treatment-Resistant Hypotension: A Case Report.

Temporary hypotension after severe trauma might help achieve hemostasis and increase the chances of survival. However, excessive hypotension can lead to adverse neurological sequelae or be fatal. The relationship between the degree of hypotension and neurological prognosis after trauma is not fully understood.

A novel mutation in SLC2A1 gene causing GLUT-1 deficiency syndrome in a young adult patient.

Üstyol A, Takahashi S, Hatipoğlu HU, Duman MA, Elevli M, Selçuk Duru HN. A novel mutation in SLC2A1 gene causing GLUT-1 deficiency syndrome in a young adult patient. Turk J Pediatr 2019; 61: 946-948.

Proteomic analysis of a urinary stone with two layers composed of calcium oxalate monohydrate and uric acid.

We examined the mechanism of urinary stone formation by analyzing the matrix proteins in a urinary stone with two layers composed of different crystals. Micro-area X-ray spectrometry and infrared spectroscopy revealed calcium oxalate monohydrate in the outside and uric acid in the inside. We also examined the interface.

Hepatocellular carcinoma as a leading cause of cancer-related deaths in Japanese type 2 diabetes mellitus patients.

Background: We reported a cross-sectional study on causes of liver injury in Japanese type 2 diabetes mellitus (T2D) patients (JG 2013). We assessed overall and cause-specific mortality risk during follow-up of patients enrolled in JG 2013.

Methods: This was a longitudinal, multicenter cohort study.

MicroRNAs as Potential Mediators for Cigarette Smoking Induced Atherosclerosis.

Smoking increases the risk of atherosclerosis-related events, such as myocardial infarction and ischemic stroke. Recent studies have examined the expression levels of altered microRNAs (miRNAs) in various diseases. The profiles of tissue miRNAs can be potentially used in diagnosis or prognosis.

Ischemic or Nonischemic Functional Mitral Regurgitation and Outcomes in Patients With Acute Decompensated Heart Failure With Preserved or Reduced Ejection Fraction.

The aim of this study was to evaluate the association of functional mitral regurgitation (FMR), preserved or reduced ejection fraction (EF), and ischemic or nonischemic origin with outcomes in patients discharged alive after hospitalization for acute decompensated heart failure (HF). Of the 4,842 patients enrolled in the Acute Decompensated Heart Failure Syndromes (ATTEND) registry, 3,357 patients were evaluated to assess the association of FMR, preserved or reduced EF, and ischemic or nonischemic origin with the primary end point (all-cause death and readmission for HF after discharge). At the time of discharge, FMR was assessed semiquantitatively (classified as none, mild, or moderate to severe) by color Doppler analysis of the regurgitant jet area.

Long-term Results of the Risk-adapted Treatment for Childhood B-Cell Acute Lymphoblastic Leukemia: Report From the Japan Association of Childhood Leukemia Study ALL-97 Trial.

Purpose: This study was conducted as the first clinical trial by Japan Association of Childhood Leukemia Study to improve the outcome of B-cell acute lymphoblastic leukemia and explore a less toxic reinduction block.

Patients And Methods: From 1997 to 2002, 563 patients with B-cell acute lymphoblastic leukemia aged 1 to 15 years were enrolled. The patients were assigned into 4 risk groups (standard, intermediate, high, or extremely high risk) and treated with regimens intensified according to the risk.

Acute and late toxicities of pirarubicin in the treatment of childhood acute lymphoblastic leukemia: results from a clinical trial by the Japan Association of Childhood Leukemia Study.

Background: Anthracyclines are used to treat childhood acute lymphoblastic leukemia (ALL). Even when administered at low doses, these agents are reported to cause progressive cardiac dysfunction. We conducted a clinical trial comparing the toxicities of two anthracyclines, pirarubicin (THP) and daunorubicin (DNR), in the treatment of childhood ALL.

Visinin-Like Protein-3 Modulates the Interaction Between Cytochrome b and NADH-Cytochrome b Reductase in a Ca-Dependent Manner.

Visinin-like proteins (VILIPs) belong to the calcium sensor protein family. VILIP-1 has been examined as a cerebrospinal fluid biomarker and as a potential indicator for cognitive decline in Alzheimer's disease (AD). However, little is known about VILIP-3 protein biochemistry.

Retrospective Analysis of Growth Speed of 54 Lesions of Colitis-associated Colorectal Neoplasia.

Aim: This study used a multicenter questionnaire survey to evaluate the morphology and progression of the initial lesion in cases of colitis-associated colorectal neoplasia (CRN).

Patients And Methods: Endoscopic images of lesions that had been definitively diagnosed as CRN by pathological examination were retrospectively reviewed.

Results: This resulted in the identification of 54 initial lesions in 49 patients.

Direct evidence for activated CD8+ T cell transmigration across portal vein endothelial cells in liver graft rejection.

Background: Lymphocyte recruitment into the portal tract is crucial not only for homeostatic immune surveillance but also for many liver diseases. However, the exact route of entry for lymphocytes into portal tract is still obscure. We investigated this question using a rat hepatic allograft rejection model.

Sudden death prediction by C-reactive protein, electrocardiographic findings, and myocardial fatty acid uptake in haemodialysis patients: analysis of a multicentre prospective cohort sub-study.

Aims: The purpose of this study was to identify determinants of sudden death among clinical information in combination with cardiac fatty acid metabolism for better risk-stratification of haemodialysis patients.

Methods And Results: Clinical and imaging data from 677 haemodialysis patients enrolled in the beta-methyl-p-iodophenyl pentadecanoic acid (BMIPP) SPECT Analysis for Decreasing Cardiac Events in Hemodialysis Patients (B-SAFE) study were analysed in this study. During a 3-year prospective follow-up interval, 20 sudden deaths were observed.

Urinary Potassium Excretion and Renal and Cardiovascular Complications in Patients with Type 2 Diabetes and Normal Renal Function.

Background And Objectives: We investigated the association of urinary potassium and sodium excretion with the incidence of renal failure and cardiovascular disease in patients with type 2 diabetes.

Design, Setting, Participants, & Measurements: A total of 623 Japanese type 2 diabetic patients with eGFR≥60 ml/min per 1.73 m(2) were enrolled in this observational follow-up study between 1996 and 2003 and followed-up until 2013.

Cardiac event risk stratification in patients with end-stage renal disease: Sub-analysis of the B-SAFE study.

Background: The aim of this study was to investigate whether 123I-labelled β-methyl iodophenyl-pentadecanoic acid (BMIPP) imaging as an abnormal myocardial fatty acid metabolism indicator better predicted fatal and non-fatal cardiac events than conventional predictors [e.g. peripheral artery disease (PAD) and diabetes mellitus (DM)] in haemodialysis patients.

Increased Serine-Arginine (SR) Protein Phosphorylation Changes Pre-mRNA Splicing in Hypoxia.

The removal of introns from mRNA precursors (pre-mRNAs) is an essential step in eukaryotic gene expression. The splicing machinery heavily contributes to biological complexity and especially to the ability of cells to adapt to altered cellular conditions. Inhibitory PAS domain protein (IPAS), a dominant negative regulator of hypoxia-inducible gene expression, is generated from hypoxia inducible transcription factor-3α (HIF-3α) pre-mRNA by an alternative splicing mechanism.

Expression of Secretogranin III in Chicken Endocrine Cells: Its Relevance to the Secretory Granule Properties of Peptide Prohormone Processing and Bioactive Amine Content.

The expression of secretogranin III (SgIII) in chicken endocrine cells has not been investigated. There is limited data available for the immunohistochemical localization of SgIII in the brain, pituitary, and pancreatic islets of humans and rodents. In the present study, we used immunoblotting to reveal the similarities between the expression patterns of SgIII in the common endocrine glands of chickens and rats.

Transplanted bone marrow-derived circulating PDGFRα+ cells restore type VII collagen in recessive dystrophic epidermolysis bullosa mouse skin graft.

Recessive dystrophic epidermolysis bullosa (RDEB) is an intractable genetic blistering skin disease in which the epithelial structure easily separates from the underlying dermis because of genetic loss of functional type VII collagen (Col7) in the cutaneous basement membrane zone. Recent studies have demonstrated that allogeneic bone marrow transplantation (BMT) ameliorates the skin blistering phenotype of RDEB patients by restoring Col7. However, the exact therapeutic mechanism of BMT in RDEB remains unclear.

Laboratory and clinical features of abnormal macroenzymes found in human sera.

We report the analysis of unusual macroenzymes, performed in our laboratory, and review the relevant literature. In particular, we focused on macro AST, macroamylase, macro LD and macro CK. Macroenzymes are seen in healthy subjects, but can also be related to disease; thus, accurate detection is useful in day-to-day clinical practice.