Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA.

Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease.

Results: The study population included 157 patients (mean age 49.

Sitagliptin and risk of fractures in type 2 diabetes: Results from the TECOS trial.

Aim: To examine fracture incidence among participants in the Trial Evaluating Cardiovascular Outcomes with Sitagliptin (TECOS).

Research Design And Methods: We used data from 14 671 participants in the TECOS study who were randomized double-blind to sitagliptin (n = 7332) or placebo (n = 7339). Cumulative fracture incidence rates were calculated and their association with study treatment assignment was examined using multivariable Cox proportional hazards regression.

Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents.

Children and adolescents with pre-existing conditions such as DNA repair defects or other primary immunodeficiencies have an increased risk of non-Hodgkin lymphoma. However, large-scale data on patients with non-Hodgkin lymphoma and their entire spectrum of pre-existing conditions are scarce. A retrospective multinational study was conducted by means of questionnaires sent out to the national study groups or centers, by the two largest consortia in childhood non-Hodgkin lymphoma, the European Intergroup for Childhood non-Hodgkin Lymphoma, and the international Berlin-Frankfurt-Münster Study Group.

Applications of imaging flow cytometry in the diagnostic assessment of acute leukaemia.

Automated imaging flow cytometry integrates flow cytometry with digital microscopy to produce high-resolution digital imaging with quantitative analysis. This enables cell identification based on morphology (cell size, shape), antigen expression, quantification of fluorescence signal intensity and localisation of detected signals (i.e.

Non-anaplastic peripheral T cell lymphoma in children and adolescents-an international review of 143 cases.

Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.

The McKittrick-Wheelock syndrome: a rare cause of curable diabetes.

Unlabelled: McKittrick-Wheelock syndrome (MWS) is a rare consequence of severe dehydration and electrolyte depletion due to mucinous diarrhoea secondary to a rectosigmoid villous adenoma. Reported cases of MWS commonly describe hypersecretion of mucinous diarrhoea in association with dehydration, hypokalaemia, hyponatraemia, hypochloraemia and pre-renal azotemia. Hyperglycaemia and diabetes are rarely reported manifestations of MWS.

Comparison of the International Crowding Measure in Emergency Departments (ICMED) and the National Emergency Department Overcrowding Score (NEDOCS) to measure emergency department crowding: pilot study.

Introduction: There is uncertainty about the best way to measure emergency department crowding. We have previously developed a consensus-based measure of crowding, the International Crowding Measure in Emergency Departments (ICMED). We aimed to obtain pilot data to evaluate the ability of a shortened form of the ICMED, the sICMED, to predict senior emergency department clinicians' concerns about crowding and danger compared with a very well-studied measure of emergency department crowding, the National Emergency Department Overcrowding Score (NEDOCS).

Pediatric and Adolescent Gynecology in Europe: Clinical Services, Standards of Care, and Training.

Study Objective: To identify current clinical services and training available across Europe within pediatric and adolescent gynecology (PAG) and establish the extent to which PAG services meet current European Board and College of Obstetrics and Gynecology (EBCOG) standards.

Design: Quantitative and qualitative questionnaire.

Setting: European countries that are members of the EBCOG and the European Association of Pediatric and Adolescent Gynecology.

Comparison of brucellar and tuberculous spondylodiscitis patients: results of the multicenter "Backbone-1 Study".

Background Context: No direct comparison between brucellar spondylodiscitis (BSD) and tuberculous spondylodiscitis (TSD) exists in the literature.

Purpose: This study aimed to compare directly the clinical features, laboratory and radiological aspects, treatment, and outcome data of patients diagnosed as BSD and TSD.

Study Design: A retrospective, multinational, and multicenter study was used.

Seizure induced polytrauma; not just posterior dislocation of the shoulder.

A 61-year-old woman sustained multiple fractures secondary to the tonic clonic muscular contractions of a seizure. Her injuries included: bilateral proximal humerus posterior fracture dislocations, manubrium fracture, unstable sixth thoracic vertebrae crush fracture, bilateral acetabular fractures and a left femoral neck fracture. Seizures are a rare but recognised cause of fracture.

Non-Hodgkin Lymphoma in Children and Adolescents: Progress Through Effective Collaboration, Current Knowledge, and Challenges Ahead.

Non-Hodgkin lymphoma is the fourth most common malignancy in children, has an even higher incidence in adolescents, and is primarily represented by only a few histologic subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%, in large part because of a better understanding of the biology of the different subtypes and national and international collaborations. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short intensive pulse chemotherapy containing cyclophosphamide, cytarabine, and high-dose methotrexate.

Follow-up after gastrectomy for cancer: the Charter Scaligero Consensus Conference.

Purpose: Presently, there is no scientific evidence supporting a definite role for follow-up after gastrectomy for cancer, and clinical practices are quite different around the globe. The aim of this consensus conference was to present an ideal prototype of follow-up after gastrectomy for cancer, based on shared experiences and taking into account the need to rationalize the diagnostic course without losing the possibility of detecting local recurrence at a potentially curable stage.

Methods: On June 19-22, 2013 in Verona (Italy), during the 10th International Gastric Cancer Congress (IGCC) of the International Gastric Cancer Association, a consensus meeting was held, concluding a 6-month, Web-based, consensus conference entitled "Rationale of oncological follow-up after gastrectomy for cancer.

LGR5 Activates Noncanonical Wnt Signaling and Inhibits Aldosterone Production in the Human Adrenal.

Context: Aldosterone synthesis and cellularity in the human adrenal zona glomerulosa (ZG) is sparse and patchy, presumably due to salt excess. The frequency of somatic mutations causing aldosterone-producing adenomas (APAs) may be a consequence of protection from cell loss by constitutive aldosterone production.

Objective: The objective of the study was to delineate a process in human ZG, which may regulate both aldosterone production and cell turnover.

Validation of viscoelastic coagulation tests during cardiopulmonary bypass.

Background: Viscoelastic point-of-care tests such as thromboelastography (TEG) and rotational thromboelastometry (ROTEM) are increasingly used to guide hemostatic therapy after cardiac surgery. The aim of this study was to assess their clinical utility during cardiopulmonary bypass to predict postbypass coagulation status and to guide therapy.

Methods: In this prospective study, TEG and ROTEM tests were performed in 52 adult patients undergoing elective cardiac surgery at two time points: near the end of cardiopulmonary bypass and after heparin reversal with protamine.

An exploratory cohort study comparing prothrombin complex concentrate and fresh frozen plasma for the treatment of coagulopathy after complex cardiac surgery.

Background: Administration of coagulation factor concentrates to treat bleeding after cardiac surgery with cardiopulmonary bypass might be a strategy for reducing allogeneic blood transfusions, particularly for patients treated with warfarin preoperatively. We performed an exploratory analysis on whether the use of prothrombin complex concentrate (PCC) is safe and effective compared with fresh frozen plasma (FFP) to treat coagulopathy after pulmonary endarterectomy surgery with deep hypothermic circulatory arrest.

Methods: Consecutive adult patients who underwent pulmonary endarterectomy surgery between January 2010 and September 2012 and received PCC or FFP to treat coagulopathy were studied.

Genetic diagnosis of developmental disorders in the DDD study: a scalable analysis of genome-wide research data.

Background: Human genome sequencing has transformed our understanding of genomic variation and its relevance to health and disease, and is now starting to enter clinical practice for the diagnosis of rare diseases. The question of whether and how some categories of genomic findings should be shared with individual research participants is currently a topic of international debate, and development of robust analytical workflows to identify and communicate clinically relevant variants is paramount.

Methods: The Deciphering Developmental Disorders (DDD) study has developed a UK-wide patient recruitment network involving over 180 clinicians across all 24 regional genetics services, and has performed genome-wide microarray and whole exome sequencing on children with undiagnosed developmental disorders and their parents.

Germline mutation in BRCA1 or BRCA2 and ten-year survival for women diagnosed with epithelial ovarian cancer.

Purpose: To analyze the effect of germline mutations in BRCA1 and BRCA2 on mortality in patients with ovarian cancer up to 10 years after diagnosis.

Experimental Design: We used unpublished survival time data for 2,242 patients from two case-control studies and extended survival time data for 4,314 patients from previously reported studies. All participants had been screened for deleterious germline mutations in BRCA1 and BRCA2.

Repeatability of quantitative FDG-PET/CT and contrast-enhanced CT in recurrent ovarian carcinoma: test-retest measurements for tumor FDG uptake, diameter, and volume.

Purpose: Repeatability of baseline FDG-PET/CT measurements has not been tested in ovarian cancer. This dual-center, prospective study assessed variation in tumor 2[18F]fluoro-2-deoxy-D-glucose (FDG) uptake, tumor diameter, and tumor volume from sequential FDG-PET/CT and contrast-enhanced computed tomography (CECT) in patients with recurrent platinum-sensitive ovarian cancer.

Experimental Design: Patients underwent two pretreatment baseline FDG-PET/CT (n = 21) and CECT (n = 20) at two clinical sites with different PET/CT instruments.

The feasibility of prospectively studying maternal cardiovascular changes from before conception.

There is compelling evidence that factors before pregnancy and around implantation may have a bearing on maternal cardiovascular adaptation to pregnancy and subsequent pregnancy outcome. Prospective studies from before pregnancy are associated with difficulties in recruitment, low conception rates, early pregnancy loss and low retention of participants during pregnancy and postpartum follow-up. The objective of this study was to establish the feasibility of recruiting to; conducting and completing a prospective cohort study from before pregnancy to the postpartum period.

Glioblastomas with oligodendroglial component have the same clinical phenotype as classical glioblastomas.

Introduction: Glioblastomas are the commonest primary brain tumour and are considered one of the most heterogeneous tumour types. The introduction of a glioblastoma with oligodendroglial component (GBM + O) in the latest WHO Classification of Tumours of the Central Nervous System (1) was to help with this. There has been conflicting evidence as to whether this tumour conferred a better prognosis than classical glioblastoma (GBM).