Up-front carfilzomib, lenalidomide, and dexamethasone with transplant for patients with multiple myeloma: the IFM KRd final results.

Bortezomib, lenalidomide, and dexamethasone plus transplant is a standard of care for eligible patients with multiple myeloma. Because responses can deepen with time, regimens with longer and more potent induction/consolidation phases are needed. In this phase 2 study, patients received eight 28-day cycles of carfilzomib (K) 20/36 mg/m2 (days 1-2, 8-9, 15-16), lenalidomide (R) 25 mg (days 1-21), and dexamethasone (d) 20 mg (days 1-2, 8-9, 15-16, 22-23).

Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study.

The optimal duration of eculizumab treatment in patients with atypical hemolytic uremic syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter open-label study to assess eculizumab discontinuation in children and adults with aHUS. Fifty-five patients (including 19 children) discontinued eculizumab (mean treatment duration, 16.

Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.

Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill defined. This report, by an international multidisciplinary working group of obstetricians, nephrologists, hematologists, intensivists, neonatologists, and complement biologists, summarizes the current knowledge of these potentially severe disorders and proposes a practical clinical approach to diagnose and manage an episode of pregnancy-associated TMA.

Heterogeneity of PD-L1 expression and CD8 tumor-infiltrating lymphocytes among subtypes of cutaneous adnexal carcinomas.

Background: Adnexal carcinomas are rare and heterogeneous skin tumors, for which no standard treatments exist for locally advanced or metastatic tumors.

Aim Of The Study: To evaluate the expression of PD-L1 and CD8 in adnexal carcinomas, and to study the association between PD-L1 expression, intra-tumoral T cell CD8 infiltrate, and metastatic evolution.

Materials And Methods: Eighty-three adnexal carcinomas were included.

Enhancer of Zeste Homologue 2 Inhibition Attenuates TGF-β Dependent Hepatic Stellate Cell Activation and Liver Fibrosis.

Background & Aims: Transdifferentiation of hepatic stellate cells (HSCs) into myofibroblasts is a key event in the pathogenesis of liver fibrosis. Transforming growth factor β (TGF-β) and platelet-derived growth factor (PDGF) are canonical HSC activators after liver injury. The aim of this study was to analyze the epigenetic modulators that differentially control TGF-β and PDGF signaling pathways.

MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease.

Background: Given the phenotypic similarities between rheumatoid arthritis (RA)-associated interstitial lung disease (ILD) (hereafter, RA-ILD) and idiopathic pulmonary fibrosis, we hypothesized that the strongest risk factor for the development of idiopathic pulmonary fibrosis, the gain-of-function MUC5B promoter variant rs35705950, would also contribute to the risk of ILD among patients with RA.

Methods: Using a discovery population and multiple validation populations, we tested the association of the MUC5B promoter variant rs35705950 in 620 patients with RA-ILD, 614 patients with RA without ILD, and 5448 unaffected controls.

Results: Analysis of the discovery population revealed an association of the minor allele of the MUC5B promoter variant with RA-ILD when patients with RA-ILD were compared with unaffected controls (adjusted odds ratio, 3.

Myelodysplastic syndrome (MDS) with isolated trisomy 8: a type of MDS frequently associated with myeloproliferative features? A report by the Groupe Francophone des Myélodysplasies.

Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (MP) features were defined by the repeated presence of one of the following: white blood cell count >10 × 10 /l, myelemia (presence of circulating immature granulocytes with a predominance of more mature forms) >2%, palpable splenomegaly.

Case series of aortic arch disease treated with branched stent-grafts.

Background: Surgical repair of aortic arch pathology is complex and associated with significant morbidity and mortality. Alternative approaches have been developed to reduce these risks, including the use of thoracic stent-grafts with fenestrations or in combination with bypass procedures to maintain supra-aortic trunk blood flow. Branched stent-grafts are a novel approach to treat aortic arch pathology.

Extended-spectrum β-lactamase-producing Enterobacteriaceae, national study of antimicrobial treatment for pediatric urinary tract infection.

Objective: To evaluate clinical practices for ESBL-producing urinary tract infection (UTI) in France.

Methods: We performed an observational, retrospective, cross-sectional, hospital-based study in 22 pediatric departments of university or secondary care hospitals. We collected data of the last five patients presenting with ESBL-producing UTI in 2012 and the physicians' therapeutic approach to two case vignettes of acute non-septic ESBL-producing pyelonephritis (7-month-old girl) and cystitis (30-month-old girl).

Radiosurgery in trochlear and abducens nerve schwannomas: case series and systematic review.

Introduction: Schwannomas involving the occulomotor cranial nerves  (CNs; III, IV and VI), can be disabling, due to the associated diplopia and decreased quality of life and are extremely rare. We evaluated the role of Gamma Knife surgery (GKS) in these cases.

Methods: Five patients with CN IV and VI schwannomas (three and two, respectively) were treated in Lausanne University Hospital between 2010 and 2015.

Radiological assessment of the placement of Essure in order to reduce hysterosalpingography: Pelvic X-ray versus combined pelvic X-ray and ultrasound.

Objective: Three months after hysteroscopic sterilisation with Essure, a confirmation test is required to evaluate the correct location of the inserts. The test may be conducted using a pelvic ultrasound (2D or 3D) or an abdominal X-ray. Should the location not look satisfactory on these tests, a follow-up hysterosalpingography (HSG) would be required.

New Alcohol and Onyx Mixture for Embolization: Feasibility and Proof of Concept in Both In Vitro and In Vivo Models.

Introduction: Onyx and ethanol are well-known embolic and sclerotic agents that are frequently used in embolization. These agents present advantages and disadvantages regarding visibility, injection control and penetration depth. Mixing both products might yield a new product with different characteristics.

Integration of Mitochondrial Targeting for Molecular Cancer Therapeutics.

Mitochondrial metabolism greatly influences cancer cell survival, invasion, metastasis, and resistance to many anticancer drugs. Furthermore, molecular-targeted therapies (e.g.

Defining the Effect of the 16p11.2 Duplication on Cognition, Behavior, and Medical Comorbidities.

Importance: The 16p11.2 BP4-BP5 duplication is the copy number variant most frequently associated with autism spectrum disorder (ASD), schizophrenia, and comorbidities such as decreased body mass index (BMI).

Objectives: To characterize the effects of the 16p11.

A phase 1 clinical trial of the selective BTK inhibitor ONO/GS-4059 in relapsed and refractory mature B-cell malignancies.

We report the results of a multicenter phase 1 dose-escalation study of the selective Bruton tyrosine kinase (BTK) inhibitor ONO/GS-4059 in 90 patients with relapsed/refractory B-cell malignancies. There were 9 dose-escalation cohorts ranging from 20 mg to 600 mg once daily with twice-daily regimens of 240 mg and 300 mg. Twenty-four of 25 evaluable chronic lymphocytic leukemia (CLL) patients (96%) responded to ONO/GS-4059, with a median treatment duration of 80 weeks; 21 CLL patients remain on treatment.

A targeted next-generation sequencing assay for the molecular diagnosis of genetic disorders with orodental involvement.

Background: Orodental diseases include several clinically and genetically heterogeneous disorders that can present in isolation or as part of a genetic syndrome. Due to the vast number of genes implicated in these disorders, establishing a molecular diagnosis can be challenging. We aimed to develop a targeted next-generation sequencing (NGS) assay to diagnose mutations and potentially identify novel genes mutated in this group of disorders.

[Current situation of the education in gynaecology-obstetrics in France].

Objectives: To get an overview of the education offer for obstetrics trainees in France and to assess their satisfaction and requests regarding these classes and their access.

Material And Methods: Two questionnaires were sent over the country between September 2013 and July 2014. The first one was intended to the person in charge of teaching in each university hospital.

Assessing early-onset hallucinations in the touch-screen generation.

The increasing development of apps for digital devices provides an opportunity for new instruments to assess hallucinations in young individuals. Here we present the Multisensory HAllucinations Scale for Children (MHASC), dedicated to assessing complex early-onset hallucinations. The MHASC will soon be translated into multilanguage versions with the support of the International Consortium of Hallucination Research.

An Open-Label, Multicenter, Phase I/II Study of JNJ-40346527, a CSF-1R Inhibitor, in Patients with Relapsed or Refractory Hodgkin Lymphoma.

Purpose: This phase I/II study investigated JNJ-40346527, a selective inhibitor of the colony-stimulating factor-1 receptor (CSF-1R) tyrosine kinase as treatment for relapsed or refractory classical Hodgkin lymphoma (cHL).

Experimental Design: Patients ≥18 years with histopathologically confirmed initial diagnosis of cHL that had relapsed or was refractory after ≥1 appropriate therapies were assigned to sequential cohorts of oral daily doses of JNJ-40346527 (150, 300, 450, 600 mg every day, and 150 mg twice a day). For the dose-escalation phase, the primary endpoint was to establish the recommended phase II dose.

Femoral head diameter considerations for primary total hip arthroplasty.

The configuration of total hip arthroplasty (THA) implants has constantly evolved since they were first introduced. One of the key components of THA design is the diameter of the prosthetic femoral head. It has been well established that the risk of dislocation is lower as the head diameter increases.

[Genital prolapse surgery: state of the art of the vaginal approach].

Treatment of genital prolapse is mainly surgical. The vaginal approach is a shorter procedure than the abdominal approach with a quicker resumption of activity for the patients. We describe different techniques which are most often performed in our daily practice.

[Sleep complaints and disorders in residential home patients taking hypnotic drugs].

Objective: To analyze sleep of residential home patients taking hypnotic drugs.

Patients And Method: This prospective, observational and multicentric study was performed a given day in nursing homes. Residents over than 65, having MMSE ≥ 15 and coherence A or B (for the AGGIR scale) were included.

[Study of surplus cost of robotic assistance for radical hysterectomy, versus laparotomy and standard laparoscopy].

Objectives: The study purpose was to compare the costs among robotic, laparoscopic and open radical hysterectomy for cervical cancer.

Patients And Methods: Thirty-seven patients underwent robotic radical hysterectomy for cervical cancer. Cases were performed by three surgeons, at two institutions, and were retrospectively reviewed to perform a cost comparison between all three modalities.