Efficacy of VRD(Bortezomib, Lenalidomide, and Dexamethasone) Consolidation Therapy and Maintenance Therapy with Immunomodulatory Drugs(Thalidomide or Lenalidomide) after Autologous Peripheral Blood Stem Cell Transplantation in the Era of Bortezomib-Containing Induction Therapy-A Single Institution Experience.

Autologous stem cell transplantation(ASCT)for newly-diagnosed multiple myeloma(NDMM)has underwent recent improvements in combination with novel agents-containing induction and post-ASCT therapy. Since the approval of bortezomib for NDMM in Japan, we conducted the following regimen(BD arm)in transplant-eligible patients with NDMM: BD (bortezomib and dexamethasone)induction, ASCT, VRD consolidation, and maintenance therapy with immunomodulatory drugs(IMIDs). The efficacy and safety of the BD arm were compared to those of patients treated with vincristine, doxorubicin, and dexamethasone(VAD)induction followed by ASCT(VAD arm)retrospectively.

[A Case Involving Successful Resection of Advanced Lower Rectal Cancer with Perineum Reconstruction after Preoperative Chemotherapy and Chemoradiotherapy].

A 58-year-old woman visited our hospital for diagnosis and treatment of rectal tumor. The tumor was diagnosed as adenocarcinoma metastasizing to the uterus and vagina. Using CT, metastases were detected in the lung, liver, and right inguinal lymph node.

Performance of Tc-aprotinin scintigraphy for diagnosing light chain (AL) cardiac amyloidosis confirmed by endomyocardial biopsy.

Background: Light chain (AL) cardiac amyloidosis is associated with a poor prognosis. Diagnosing at an early stage is critical for treatment and the management of cardiac complication.

Purpose: We aimed to evaluate the diagnostic performance of Tc-aprotinin images in patients with AL cardiac amyloidosis.

Pitfalls in diagnosing geriatric general pain: coexistence of polymyalgia rheumatism and fibromyalgia.

Polymyalgia rheumatism and fibromyalgia are clinically similar. As there are no specific diagnostic markers, they are difficult to distinguish. A 92-year-old woman with a 3-month history of general pain, including left hip pain, was referred to our hospital.

Long-Term Evaluation of Low-Dose Betamethasone for Ataxia Telangiectasia.

Background: Ataxia telangiectasia is an autosomal recessive disorder characterized by cerebellar ataxia, telangiectases, immune defects, and a predisposition to malignancy. Quality of life is severely impaired by neurological symptoms. However, curative options for the neurological symptoms are limited.

Catalase expression of Propionibacterium acnes may contribute to intracellular persistence of the bacterium in sinus macrophages of lymph nodes affected by sarcoidosis.

Bacterial catalase is important for intracellular survival of the bacteria. This protein of Propionibacterium acnes, one of possible causes of sarcoidosis, induces hypersensitive Th1 immune responses in sarcoidosis patients. We examined catalase expression in cultured P.

Comprehensive analysis of coding variants highlights genetic complexity in developmental and epileptic encephalopathy.

Although there are many known Mendelian genes linked to epileptic or developmental and epileptic encephalopathy (EE/DEE), its genetic architecture is not fully explained. Here, we address this incompleteness by analyzing exomes of 743 EE/DEE cases and 2366 controls. We observe that damaging ultra-rare variants (dURVs) unique to an individual are significantly overrepresented in EE/DEE, both in known EE/DEE genes and the other non-EE/DEE genes.

Intra- and inter-operator reproducibility of US point shear-wave elastography in various organs: evaluation in phantoms and healthy volunteers.

Purpose: This study was conducted in order to assess the intra- and interoperator reproducibility of shear-wave speed (SWS) measurement on elasticity phantoms and healthy volunteers using ultrasound-based point shear-wave elastography.

Materials And Methods: This study was approved by the institutional review board. Two operators measured the SWS of five elasticity phantoms and seven organs (thyroid, lymph node, muscle, spleen, kidney, pancreas, and liver) of 30 healthy volunteers with 1.

A novel splicing mutation in in a boy with Christianson syndrome.

A loss of function mutation in (Xq26.3) is responsible for Christianson syndrome in males. We identified a novel splicing mutation (NM_006359.

Muscular-stage Dissection during Far Lateral Approach and Its Transcondylar Extension.

 The far lateral approach includes exposure of the C1 transverse process, vertebral artery, posterior arch of the atlas, and occipital condyle. We designed a method for systematic muscular-stage dissection and present our experience with this approach.  We used a horseshoe scalp flap that was reflected downward and medially.

Optimal duration of prone positioning in patients with acute respiratory distress syndrome: a protocol for a systematic review and meta-regression analysis.

Introduction: Several systematic reviews and meta-analyses have demonstrated that prolonged (≥16 hours) prone positioning can reduce the mortality associated with acute respiratory distress syndrome (ARDS). However, the effectiveness and optimal duration of prone positioning was not fully evaluated. To fill these gaps, we will first investigate the effectiveness of prone positioning compared with the conventional management of patients with ARDS, regarding outcomes using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system.

Clinical efficacy of anti-IL-5 monoclonal antibody mepolizumab in the treatment of eosinophilic otitis media.

Objective: Eosinophilic otitis media (EOM) is an intractable otitis media characterized by a highly viscous effusion containing eosinophils, and it is mostly associated with bronchial asthma. Recently, anti-IL-5 therapy using mepolizumab has been reported to be effective for patients with severe and refractory eosinophilic bronchial asthma. EOM shows accumulation of eiosinophils in the middle ear effusion and most EOM patients have high numbers of peripheral blood eosinophils.

The Prognostic Impact of Dose-attenuated R-CHOP Therapy for Elderly Patients with Diffuse Large B-cell Lymphoma.

Objective Although R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone) is a standard therapy for diffuse large B-cell lymphoma (DLBCL), the optimal dose for elderly patients remains unclear. Methods and Patients We retrospectively verified our R-CHOP dose-attenuation system implemented from 2005 for DLBCL patients. Among the 115 DLBCL patients treated during 2001-2010, 33 patients treated during 2001-2005 received R-CHOP doses adjusted according to physicians' decisions (PHY group).

2017 Clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis.

Objective: The Japan Research Committee for Intractable Vasculitis has fully revised the clinical practice guidelines (CPG) for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) to improve and standardize the medical treatment of the disease in Japan.

Methods: The previous CPG was published in a classical review style in Japanese in 2011 and 2014. We adopted the Grading of Recommendations Assessment, Development and Evaluation system for this revision, and various stakeholders, including patients, participated in it.

Systematic review and meta-analysis for 2017 clinical practice guidelines of the Japan research committee of the ministry of health, labour, and welfare for intractable vasculitis for the management of ANCA-associated vasculitis.

Objectives: To provide evidence for the revision of clinical practice guideline (CPG) for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by the Japan Research Committee for Intractable Vasculitis.

Methods: PubMed, CENTRAL, and the Japan Medical Abstracts Society were searched for articles published between January 1994 and January 2015 to conduct systematic review (SR), and the quality of evidence was assessed with GRADE approach.

Results: Nine randomized controlled trials (RCTs) and two non-RCTs were adopted for remission induction therapy, three RCTs and two non-RCTs for plasma exchange, and five RCTs and one non-RCT for remission maintenance therapy.

Atraumatic tension hemothorax associated with ruptured aneurysm of intercostal artery-azygous fistula: a case report.

Atraumatic tension hemothorax is extremely rare. We report a case of 62-year-old woman presented with obstructive and hypovolemic shock status by tension hemothorax due to ruptured aneurysm of congenital intercostal artery-azygous fistula. Contrast-enhanced computed tomography and aortography revealed an aberrant 12th intercostal artery flowing into the aneurysm with a fistula draining into the azygous vein and the rupture of aneurysm.

Significance of C-PIB PET/CT in cardiac amyloidosis compared with Tc-aprotinin scintigraphy: A pilot study.

Background: This study was to investigate the significance of C-Pittsburgh B (PIB) PET/CT in patients with suspected cardiac amyloidosis compared with Tc-aprotinin scintigraphy.

Methods: Thirteen consecutive patients with suspected cardiac amyloidosis were considered for enrolment in this prospective pilot study. Participants were scheduled to undergo a series of C-PIB PET/CT and Tc-aprotinin within a 2-month period.

Background factors and short-term health-related quality of life in patients who initially underwent radical prostatectomy or androgen deprivation therapy for localized prostate cancer in a Japanese prospective observational study (J-CaP Innovative Study-1).

Background: This prospective observational study elucidated the usefulness of hormonal therapy for localized prostate cancer. Background factors and the health-related quality of life in patients who initially underwent radical prostatectomy (RP) or primary androgen deprivation therapy (PADT) for localized prostate cancer are summarized.

Materials And Methods: Patients aged 67-76 years with clinical stage T1c or T2 localized prostate cancer treated with PADT or RP, a prostate-specific antigen concentration of <20 ng/mL, and Gleason score of ≤7 were included.

Propionibacterium acnes-derived insoluble immune complexes in sinus macrophages of lymph nodes affected by sarcoidosis.

Background: Propionibacterium acnes is thought to be a causative agent of sarcoidosis. Patients with sarcoidosis have circulating immune complexes. We attempted to detect P.

Improved progression-free and event-free survival in myeloma patients undergoing PBSCH receiving a cyclophosphamide + G-CSF regimen than G-CSF alone.

Two regimens are commonly used for peripheral blood hematopoietic stem cell harvesting (PBSCH) in multiple myeloma: high-dose cyclophosphamide (HD-CY) + granulocyte-colony stimulating factor (G-CSF), and G-CSF alone. The objective of the present study was to evaluate the anti-myeloma effect of the PBSCH regimen including HD-CY. We retrospectively assessed harvesting efficiency, complications, and anti-myeloma effects in 115 patients receiving HD-CY + G-CSF (HD-CY group) and 32 patients receiving G-CSF alone (G-alone group).

Report of the Second Asian Prostate Cancer (A-CaP) Study Meeting.

The Asian Prostate Cancer (A-CaP) Study is an Asia-wide initiative that has been developed over the course of 2 years. The study was launched in December 2015 in Tokyo, Japan, and the participating countries and regions engaged in preparations for the study during the course of 2016, including patient registration and creation of databases for the purpose of the study. The Second A-CaP Meeting was held on September 8, 2016 in Seoul, Korea, with the participation of members and collaborators from 12 countries and regions.

Cause of acute encephalitis/encephalopathy in Japanese children diagnosed by a rapid and comprehensive virological detection system and differences in their clinical presentations.

Background: Acute encephalitis/encephalopathy (AE/E) is a rare and severe complication of common childhood infections; however, a treatment strategy based on clinical and pathological evidence has not been established.

Methods: The clinical data and aetiological results using a rapid and comprehensive virological detection system of 62 Japanese children diagnosed with AE/E from 2010 to 2014 were collected. We assessed clinical differences between causes and effectiveness of our multiplex PCR system to establish a pathogen-based treatment strategy for AE/E.

Effects of non-invasive ventilation in patients with acute respiratory failure excluding post-extubation respiratory failure, cardiogenic pulmonary edema and exacerbation of COPD: a systematic review and meta-analysis.

Background: This meta-analysis compared the effects of non-invasive ventilation (NIV) with invasive mechanical ventilation (InMV) and standard oxygen (O) therapy on mortality and rate of tracheal intubation in patients presenting acute respiratory failure (ARF).

Methods: We searched the MEDLINE, EMBASE and Cochrane Central Register of clinical trials databases between 1949 and May 2015 to identify randomized trials of NIV for ARF. We excluded the ARF caused by extubation, cardiogenic pulmonary edema, and COPD.