Coexistence of Cervical Leiomyosarcoma and Gastric-Type Adenocarcinoma In Situ with Extensive Extension to the Endometrium and Fallopian Tube.

Cervical leiomyosarcoma is known to be rare from the previous reviews of a large number of malignant cervical tumors. The patient was a 66-year-old woman with irregular vaginal bleeding. She underwent modified radical hysterectomy and bilateral salpingooophorectomy.

Unfolded protein response is activated in Krabbe disease in a manner dependent on the mutation type.

Krabbe disease, one of the autosomal-recessive lysosomal storage disorders (LSDs), is caused by a deficiency of galactocerebrosidase (GALC) activity, resulting in the intracellular accumulation of psychosine, which is cytotoxic for neuronal cells. Genetically pathogenic mutations result in conformational changes in GALC and disrupt the lysosmal trafficking of cargos, which subsequently accumulate in the endoplasmic reticulum (ER). Recently, ER stress together with the activation of the unfolded protein response (UPR) has been suggested to play a key role in the pathogenesis of LSDs.

Cervical Periosteal Chondroma Causing Spinal Cord or Nerve Compression: 2 Case Reports and Literature Review.

Background: Chondroma is a rare benign cartilaginous tumor that originates in chondrocytes and is commonly seen in the long bones; however, it infrequently occurs in the cervical spine.

Case Descriptions: We report 2 patients with cervical periosteal chondroma that originated in the C2 vertebral body and C1 lamina as extramedullary tumors. The first patient was a 28-year-old man who presented with right upper extremity weakness and numbness.

CyberKnife Stereotactic Radiosurgery for Primary and Metastatic Cancer in the Cervix.

Standard radiation therapy for cervical cancer consists of external beam radiation therapy followed by an intracavitary brachytherapy boost. When brachytherapy cannot be performed due to unfavorable anatomy or coexisting medical conditions, stereotactic body radiotherapy with the CyberKnife is another treatment option which is less invasive and can be performed in a shorter treatment time. We performed non-invasive therapy using the CyberKnife for five cases of urogenital cancer located in the cervix.

Interferon sensitivity-determining region of hepatitis C virus influences virus production and interferon signaling.

The number of amino acid substitutions in the interferon (IFN) sensitivity-determining region (ISDR) of hepatitis C virus (HCV) NS5A is a strong predictor for the outcome of IFN-based treatment. To assess the involvement of ISDR in the HCV life cycle and to clarify the molecular mechanisms influencing IFN susceptibility, we used recombinant JFH-1 viruses with NS5A of the genotype 1b Con1 strain (JFH1/5ACon1) and with NS5A ISDR containing 7 amino acid substitutions (JFH1/5ACon1/i-7mut), and compared the virus propagation and the induction of interferon-stimulated genes (ISGs). By transfecting RNAs of these strains into HuH-7-derived cells, we found that the efficiency of infectious virus production of JFH1/5ACon1/i-7mut was attenuated compared with JFH1/5ACon1.

Ten-year-long enzyme replacement therapy shows a poor effect in alleviating giant leg ulcers in a male with Fabry disease.

Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A (α-gal A), leading to the progressive accumulation of glycosphingolipids. Classical hemizygous males usually present symptoms, including pain and paresthesia in the extremities, angiokeratoma, hypo- or anhidrosis, abdominal pain, cornea verticillata, early stroke, tinnitus, and/or hearing loss, during early childhood or adolescence. Moreover, proteinuria, renal impairment, and cardiac hypertrophy can appear with age.

Multilocular cystic leiomyoma of the anterolateral abdominal wall: A case report and literature review.

Rationale: Leiomyomas arising from the anterolateral abdominal wall are uncommon, and their pathogenesis remains unknown. We present the 15th case of such a tumor, having this unique tumor morphology, followed by a detailed discussion on disease pathogenesis.

Patient Concerns: A 48-year-old, asymptomatic perimenopausal, multiparous Japanese woman presented with a left-sided pelvic mass.

CyberKnife Stereotactic Radiosurgery for Cholangiocarcinoma.

Cholangiocarcinoma is a devastating cancer with a poor prognosis. Patients are often diagnosed at the advanced stage, and curative surgery is impossible. Radiation therapy is used for cases of advanced cancer.

Magnetic Resonance Imaging-guided Focused Ultrasound Thalamotomy for Parkinson's Disease.

Thalamotomy is effective in treating refractory tremor in Parkinson's disease (PD). We herein report a PD patient who underwent left ventral intermediate nucleus and ventro oralis posterior nucleus thalamotomy using magnetic resonance imaging-guided focused ultrasound (MRgFUS). Right-side resting tremor and rigidity were abolished immediately following the ultrasound energy delivery.

Radiologic Evaluation of Ossification of the Posterior Longitudinal Ligament with Dural Ossification.

There are 3 basic radiological patterns of dural ossification (DO). Although double-layer DO is most common, when examining neuroimaging of ossification of the posterior longitudinal ligament (OPLL), isolated DO or masse DO should be kept in mind. Bone window computed tomography (CT) is most sufficient in identifying any type of DO associated with OPLL.

History and Evolution of Laminoplasty.

Techniques of expansive laminoplasty for degenerative cervical myelopathy and ossified posterior longitudinal ligament are described, focusing on the history of the surgical procedure. Laminectomy was the only approach for posterior decompression before Japanese orthopedic surgeons introduced laminoplasty from the 1970s to the 1980s to overcome the poor outcomes of laminectomy. Recent laminoplasty techniques offer less invasive maneuvers to the posterior cervical muscle structures to reduce axial neck pain and to obtain better functional outcome, but every operation is carried out based on the unchanged initial concept.

Influence of the revision of surgical fee schedule on surgeons' productivity in Japan: A cohort analysis of 7602 surgical procedures in 2013-2016.

The goal of this study is to evaluate the pure impact of the revision of surgical fee schedule on surgeons' productivity. We collected data from the surgical procedures performed by the surgeons working in Teikyo University Hospital from 1 April through 30 September in 2013-2016. We employed non-radial and non-oriented Malmquist model.

No clinical significance of the time interval between biopsy and robotic-assisted radical prostatectomy for patients with clinically localized prostate cancer on biochemical recurrence: a propensity score matching analysis.

Purpose: To investigate the impact of the time interval (TI) between prostate biopsy and robot-assisted radical prostatectomy (RARP) on the risk of biochemical recurrence (BCR).

Methods: We retrospectively reviewed the medical records of 793 consecutive patients who were treated with RARP at our institution. Patients were divided into three groups, according to TI, to compare BCR-free survival (BCRFS) rates: Group 1 (n = 196), TI < 3 months; Group 2 (n = 513), 3 ≤ TI < 6 months; Group 3 (n = 84), TI ≥ 6 months.

Application of a diagnostic methodology by quantification of 26:0 lysophosphatidylcholine in dried blood spots for Japanese newborn screening of X-linked adrenoleukodystrophy.

X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease that results in the accumulation of very long chain fatty acids (VLCFA) in plasma and all tissues. Recent studies regarding cerebral X-ALD (CALD) treatment emphasize the importance of its early diagnosis. 26:0 lysophosphatidylcholine (LysoPC) is a sensitive biomarker for newborn screening of X-ALD, while its application for Japanese DBS is unclear.

Efficiency of inpatient orthopedic surgery in Japan: a medical claims database analysis.

Purpose The purpose of this paper is to determine the characteristics of healthcare facilities that produce the most efficient inpatient orthopedic surgery using a large-scale medical claims database in Japan. Design/methodology/approach Reimbursement claims data were obtained from April 1 through September 30, 2014. Input-oriented Banker-Charnes-Cooper model of data envelopment analysis (DEA) was employed.

Prospective, randomized, controlled study of the efficacy of transcatheter arterial chemoembolization with miriplatin for hepatocellular carcinoma.

Aim: Transcatheter arterial chemoembolization (TACE) has been recognized as a treatment option for patients with intermediate hepatocellular carcinoma (HCC). This randomized, controlled study compared the local control efficacy of TACE with miriplatin (platinum monohydrate) or with epirubicin.

Methods: The study group consisted of 200 Japanese patients with unresectable HCC treated at the Kitasato University East Hospital (Sagamihara, Japan) between July 2010 and June 2013.

ZEB1 expression is a potential indicator of invasive endometriosis.

Introduction: Although endometriosis is a benign disease, it shares some features with cancers, such as invasiveness and the potential to metastasize. This study sought to investigate the epithelial-mesenchymal transition status in human endometriotic lesions.

Material And Methods: Thirteen endometriosis patients and 10 control women without endometriosis undergoing surgery for benign indications were recruited.

Early onset of Fazio-Londe syndrome: the first case report from the Arabian Peninsula.

Fazio-Londe syndrome is a rare neurological disorder presenting with sensorineural deafness, bulbar palsy and respiratory compromise that is caused by mutation in the gene, which encodes the intestinal (hRFT2) riboflavin transporter. We report a patient with early onset of Fazio-Londe syndrome as the first case report in Saudi Arabia with rapid regression to death at 24 months of age.

Thymic Carcinoma Treated by CyberKnife Stereotactic Body Radiotherapy.

The standard treatment for advanced thymic carcinoma has not yet been established. Most patients have no symptoms until the advanced stage. Radiation therapy has been used for advanced stage cancer, usually in combination with surgery or chemotherapy; however, the survival rates are 30%-50%.

Predictors of surgeons' efficiency in the operating rooms.

The sustainability of the Japanese healthcare system is questionable because of a huge fiscal debt. One of the solutions is to improve the efficiency of healthcare. The purpose of this study is to determine what factors are predictive of surgeons' efficiency scores.

A randomised, phase III trial of once-daily fluticasone furoate/vilanterol 100/25 μg versus once-daily vilanterol 25 μg to evaluate the contribution on lung function of fluticasone furoate in the combination in patients with COPD.

Background: The contribution of fluticasone furoate (FF) on lung function in the FF/vilanterol (VI) 100/25 μg combination has been demonstrated numerically, but not statistically.

Methods: This multicentre, randomised, double-blind, controlled trial (GlaxoSmithKline study number 200820; clinicaltrials.gov NCT02105974) enrolled ≥40-year-old patients with chronic obstructive pulmonary disease (COPD), a ≥10-pack-year smoking history, a post-bronchodilator forced expiratory volume in 1 s (FEV) 30-70% of the predicted value, a FEV/forced vital capacity ratio of ≤0.

The severe clinical phenotype for a heterozygous Fabry female patient correlates to the methylation of non-mutated allele associated with chromosome 10q26 deletion syndrome.

Heterozygous Fabry females usually have an attenuated form of Fabry disease, causing them to be symptomatic; however, in rare cases, they can present with a severe phenotype. In this study, we report on a 37-year-old woman with acroparesthesia, a dysmorphic face, left ventricular hypertrophy, and intellectual disability. Her father had Fabry disease and died due to chronic renal and congestive cardiac failure.