Abnormal maturation of non-dysmorphic neurons in focal cortical dysplasia: immunohistochemical considerations.

Aim: Dysmorphic neurons and balloon cells in focal cortical dysplasia (FCD) reportedly show immaturity and abnormal differentiation with neuronal and glial components. Although normal-looking neurons (NL-neurons) in FCD are major constituent elements, their biological characteristics have never been identified. The aim of this study was to investigate maturation of NL-neurons with the focus on neuronal developmental lineage.

[Two cases of microscopic polyangitis with preceding interstitial pneumonia diagnosed by surgical lung biopsy].

We encountered 2 cases of microscopic polyangitis (MPA) with preceding interstitial pneumonia. The patients were elderly men, and radiological findings showed a UIP pattern in both cases. Furthermore, ANCA-associated vasculitis had been suspected since the first admission in case 1, because MPO-ANCA tests were positive, and the surgical lung biopsy revealed vasculitis in a honeycomb lesion.

Clonal evolution with double Ph followed by tetraploidy in imatinib-treated chronic myeloid leukemia with e19a2 transcript in transformation.

Chronic myeloid leukemia (CML) with the e19a2 transcript coding for p230 is typically associated with a benign clinical course unless accompanied at presentation with additional chromosomal abnormalities. We report here a case of CML with e19a2 who did not show additional chromosomal abnormalities at diagnosis, but progressed to the fatal advanced stage in approximately 2 years. The patient was initially treated with imatinib, which, however, could be administered only intermittently at reduced doses because of recurrent thrombocytopenia and fluid retention.

Myocardial Fat Imaging.

The presence of intramyocardial fat may form a substrate for arrhythmias, and fibrofatty infiltration of the myocardium has been shown to be associated with sudden death. Therefore, noninvasive detection could have high prognostic value. Fat-water-separated imaging in the heart by MRI is a sensitive means of detecting intramyocardial fat and characterizing fibrofatty infiltration.

Spontaneous cerebrospinal fluid rhinorrhea associated with a far lateral temporal encephalocele--case report.

A 35-year-old female complained of right-sided watery nasal discharge persisting for 2 weeks. Neuroimaging investigations revealed a defect in the lateral side of middle cranial fossa, temporal lobe encephalocele protruding into the lateral extension of the sphenoid sinus, and cerebrospinal fluid (CSF) collection on the right side of the sphenoid sinus. The transcranial approach was performed for resection of the encephalocele and obliteration of the cranial base defect anterolateral to the foramen spinosum with transcranial multilayered closure of the defect using autologous fat, cranial bone graft, and vascularized split temporal muscle.

Rosette-forming glioneuronal tumor of the fourth ventricle--case report.

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a rare tumor included as a novel glioneuronal neoplasm in the 2007 World Health Organization classification of brain tumors. A 15-year-old female patient presented with RGNT of the fourth ventricle manifesting as headache persisting for one month. Magnetic resonance imaging disclosed a nonenhanced solid mass in the fourth ventricle and vermis.

The impact of obesity on the left ventricle: the Multi-Ethnic Study of Atherosclerosis (MESA).

Objectives: The purpose of this study was to evaluate the relationship of left ventricular (LV) remodeling assessed by cardiac magnetic resonance to various measures of obesity in a large population-based study.

Background: Obesity is a well-known risk factor for cardiovascular disease, yet its relationship with LV size and function is poorly understood.

Methods: A total of 5,098 participants (age 45 to 84 years; 48% men) in the Multi-Ethnic Study of Atherosclerosis who were free of clinically apparent cardiovascular disease underwent cardiac magnetic resonance to assess LV size and function as well as measures of obesity, including body mass index, waist-to-hip ratio and waist circumference, and cardiovascular risk factors.

Solitary metastatic tumor within the optic chiasm--case report.

A 61-year-old female, previously treated for lung cancer, presented with a rare case of metastasis directly to the optic chiasm manifesting as visual deficits. Magnetic resonance imaging revealed a suprasellar mass similar to an optic glioma. At surgery, the optic chiasm appeared swollen with hypervascularity over the surface.

Radiation dose from single-heartbeat coronary CT angiography performed with a 320-detector row volume scanner.

Purpose: To determine radiation doses from coronary computed tomographic (CT) angiography performed by using a 320-detector row volume scanner and evaluate how the effective dose depends on scan mode and the calculation method used.

Materials And Methods: Radiation doses from coronary CT angiography performed by using a volume scanner were determined by using metal-oxide-semiconductor field-effect transistor detectors positioned in an anthropomorphic phantom physically and radiographically simulating a male or female human. Organ and effective doses were determined for six scan modes, including both 64-row helical and 280-row volume scans.

Autoimmune hemolytic anemia accompanied by reactivation of an Epstein-Barr virus infection with suppressed CTL response to EBV-infected cells in an elderly man.

An 88-year-old man with autoimmune hemolytic anemia (AIHA) who had been treated with low dose prednisolone developed a sudden worsening of his anemia accompanied by reactivation of Epstein-Barr virus (EBV). We established EBV-infected spontaneous lymphoblastoid cell lines (LCL), performed an enzyme-linked immunosorbent spot assay, and confirmed a significantly suppressed EBV-specific cytotoxic T-cell (CTL) response to the LCL. EBV reactivation might have been brought about by suppressed CTL activity which could have been due to low dose PSL administration or aging.

Expression of Toll-like receptor 9 in bone marrow cells of myelodysplastic syndromes is down-regulated during transformation to overt leukemia.

Toll-like receptors (TLRs) play a crucial role in the host defense against invading microorganisms by recognizing pathogen-associated molecular patterns. Recently, a number of endogenous molecules have been reported to be ligands of TLRs. Some of these molecules are known to be expressed in cancer tissue and activate intracellular signal pathways via TLRs during cancer progression.

Quality of life after esophagectomy and endoscopic therapy for Barrett's esophagus with dysplasia.

Esophagectomy (EG) and endoscopic therapy (ET) can eradicate Barrett's esophagus with early neoplasia. Their relative effect on quality of life is unknown. The 36-item Short Form Health Survey (SF-36) and Gastrointestinal Quality of Life Index (GIQLI) questionnaires were sent to all patients who underwent either EG or ET at our institution over the last 9 years.

Diorganotelluride-catalyzed oxidation of silanes to silanols under atmospheric oxygen.

Diorganotellurides efficiently catalyze the aerobic oxidation of organosilanes under photosensitized conditions to afford the corresponding silanols in excellent yield.

[A case of CNS metastasis from gastric MALT lymphoma].

Recently, the incidence of primary CNS lymphoma (PCNSL) is increasing. Metastatic CNS lymphoma occurs much less than PCNSL. We report the case of a 53-year-old man who presented with CNS metastasis from gastric mucosa-associated lymphoid tissue (MALT) lymphoma.

Identification of cell cycle-arrested quiescent osteoclast precursors in vivo.

How are sites suitable for osteoclastogenesis determined? We addressed this issue using in vivo and in vitro experimental systems. We first examined the formation of osteoclasts in ectopic bone induced by BMP-2. When collagen disks which contained BMP-2 (BMP-2-disks) or vehicle (control-disks) were implanted into wild-type mice, osteoclasts and osteoblasts appeared in the BMP-2-disks, but not in the control disks.

Extranodal NK/T cell lymphoma, nasal type, of the small intestine diagnosed by double-balloon endoscopy.

Extranodal NK/T-cell lymphoma (ENKL), nasal type, is rare and the small intestine is quite extraordinary as a primary lesion site. We report a 47-year-old man with ENKL of the small intestine. He was referred to our hospital because of bloody stool and the diagnosis was made by double-balloon endoscopy (DBE) of the small intestine without surgical procedure.

The phenotypic spectrum of contiguous deletion of CYP21A2 and tenascin XB: quadricuspid aortic valve and other midline defects.

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is an autosomal recessive disorder and is the most common cause of ambiguous genitalia in the newborn. The genes encoding 21-hydroxylase, CYP21A2, and tenascin-X (TNX), TNXB, are located within the HLA complex, in a region of high gene density termed the RCCX module. The module has multiple pseudogenes as well as tandem repeat sequences that promote misalignment during meiosis leading to complex gene rearrangements, deletions and gene conversion events.

The association between cholecystectomy and gastroesophageal reflux symptoms: a prospective controlled study.

Objective: A large controlled prospective observational study to compare pre- and postsurgery changes in reflux symptoms between cholecystectomy and hernia repair surgery patients.

Summary Background Data: Six studies have suggested that gastroesophageal reflux worsens after cholecystectomy. However, all these studies had design limitations.

Direct injection of autologous mesenchymal stromal cells improves myocardial function.

Cell-based therapies have been employed with conflicting results. Whether direct injection of ex-vivo expanded autologous marrow stromal cells (MSCs) would improve the function of ischemic myocardium and enhance angiogenesis is not well defined. In a porcine model of chronic ischemia, MSCs were isolated and cultured for 4 weeks.

Cripto-1 is required for hypoxia to induce cardiac differentiation of mouse embryonic stem cells.

Cripto-1 is a membrane-bound protein that is highly expressed in embryonic stem cells and in human tumors. In the present study, we investigated the effect of low levels of oxygen, which occurs naturally in rapidly growing tissues, on Cripto-1 expression in mouse embryonic stem (mES) cells and in human embryonal carcinoma cells. During hypoxia, Cripto-1 expression levels were significantly elevated in mES cells and in Ntera-2 or NCCIT human embryonal carcinoma cells, as compared with cells growing with normal oxygen levels.

Modulation of agonist binding to AMPA receptors by 1-(1,4-benzodioxan-6-ylcarbonyl)piperidine (CX546): differential effects across brain regions and GluA1-4/transmembrane AMPA receptor regulatory protein combinations.

Ampakines are cognitive enhancers that potentiate alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor currents and synaptic responses by slowing receptor deactivation. Their efficacy varies greatly between classes of neurons and brain regions, but the factor responsible for this effect remains unclear. Ampakines also increase agonist affinity in binding tests in ways that are related to their physiological action.

Papillectomy for ampullary neoplasm: results of a single referral center over a 10-year period.

Background: Tumors arising from the duodenal papilla account for approximately 5% of GI neoplasms, but are increasingly identified.

Objective: To describe the clinical characteristics and outcomes in a large single-center experience with patients referred for ampullary lesions.

Design: A retrospective review of the Virginia Mason Medical Center endoscopy and hospital service database.

The ClinSeq Project: piloting large-scale genome sequencing for research in genomic medicine.

ClinSeq is a pilot project to investigate the use of whole-genome sequencing as a tool for clinical research. By piloting the acquisition of large amounts of DNA sequence data from individual human subjects, we are fostering the development of hypothesis-generating approaches for performing research in genomic medicine, including the exploration of issues related to the genetic architecture of disease, implementation of genomic technology, informed consent, disclosure of genetic information, and archiving, analyzing, and displaying sequence data. In the initial phase of ClinSeq, we are enrolling roughly 1000 participants; the evaluation of each includes obtaining a detailed family and medical history, as well as a clinical evaluation.