Bleeding phenotype according to factor level in 825 children with nonsevere hemophilia: data from the PedNet cohort.

Background: Information on bleeding phenotype in nonsevere hemophilia may be used to determine target factor levels for prophylaxis or gene therapy in severe hemophilia.

Objectives: To assess the association between endogenous factor level and bleeding phenotype in children with nonsevere (factor [F]VIII/FIX activity 1%-25%) hemophilia A (HA) and B without prophylaxis.

Methods: Data on annualized bleeding rate (ABR), annualized joint bleeding rate (AJBR), and onset of bleeding were extracted from the international PedNet cohort including children born since 2000.

Long-term results of total hip arthroplasty in severe congenital hip disease: A ten- to 20-year follow-up study.

Introduction: The impact of bone deformities, previous surgeries, and the surgical technique in total hip arthroplasty (THA) for congenital dislocation of the hip (CDH) at a long-term has not been clearly defined yet. In this single-centre observational study we sought to assess patients undergoing THA due to osteoarthritis secondary to severe CDH with low or high dislocation ten- to 20-years after surgery. To determine this purpose, we assessed: (1) THA-related complications and reoperations; (2) the clinical outcome, patients' satisfaction and radiological results; and (3) the possible risk factors for reoperation with particular attention to the surgical technique and the influence of prior surgeries.

1st EFORT European Consensus "Medical & Scientific Research Requirements for the Clinical Introduction of Artificial Joint Arthroplasty Devices": Background, Delphi Methodology & Consensus process.

The objectives of the 1st EFORT European Consensus on 'Medical and Scientific Research Requirements for the Clinical Introduction of Artificial Joint Arthroplasty Devices' were foremost to focus on patient safety by establishing performance requirements for medical devices. The 1st EFORT European Consensus applied an a priori-defined, modified Delphi methodology to produce unbiased, high-quality recommendation statements, confirmed by consensus voting of a European expert panel. Intended key outcomes are practical guidelines justified by the current stage of knowledge and based on a broad European Expert Consensus, to maintain innovation and optimisation of orthopaedic devices within the boundaries of MDR 2017/745.

Clinical and Epidemiological Correlates of Low IFN-Gamma Responses in Mitogen Tube of QuantiFERON Assay in Tuberculosis Infection Screening During the COVID-19 Pandemic: A Population-Based Marker of COVID-19 Mortality?

Background: The clinical and epidemiological implications of abnormal immune responses in COVID-19 for latent tuberculosis infection (LTBI) screening are unclear.

Methods: We reviewed QuantiFERON TB Gold Plus (QFT-Plus) results (36,709 patients) from July 2016 until October 2021 in Asturias (Spain). We also studied a cohort of ninety hospitalized patients with suspected/confirmed COVID-19 pneumonia and a group of elderly hospitalized patients with COVID-19 who underwent serial QFT-Plus and immune profiling testing.

The experiences of early career geriatricians throughout Europe during the COVID-19 pandemic.

The COVID-19 pandemic has severely affected older adults and brought about unprecedented challenges to geriatricians. We aimed to evaluate the experiences of early career geriatricians (residents or consultants with up to 10 years of experience) throughout Europe using an online survey. We obtained 721 responses.

Ceramic-on-ceramic cementless total hip arthroplasty in patients aged 40 years or under: Do preoperative conditions affect long-term results?

Introduction: Underlying diseases, bone deformities and polyethylene wear affect outcome in young patients undergoing total hip arthroplasty (THA). These issues are not widely confirmed for ceramic - on-ceramic THA, particularly regarding cup fixation. Therefore, we did a retrospective long-term investigation on a large population of cementless ceramic-on-ceramic THA in patients aged 40 years or younger aiming to analyze: 1) the complication rate; 2) clinical results; and 3) survival for cup loosening with regard to their preoperative conditions.

Multiple myeloma and SARS-CoV-2 infection: clinical characteristics and prognostic factors of inpatient mortality.

There is limited information on the characteristics, prognostic factors, and outcomes of patients with multiple myeloma (MM) hospitalized with COVID-19. This retrospective case series investigated 167 patients reported from 73 hospitals within the Spanish Myeloma Collaborative Group network in March and April, 2020. Outcomes were compared with 167 randomly selected, contemporary, age-/sex-matched noncancer patients with COVID-19 admitted at six participating hospitals.

First-line immune checkpoint blockade for advanced non-small-cell lung cancer: Travelling at the speed of light.

The development of PD-1, PD-L1 and CTLA-4 immune checkpoint inhibitors (CPI) has revolutionised the treatment of advanced non-small cell lung cancer (NSCLC). The potential of immunotherapy (IO) to induce durable responses for a subset of patients represents a therapeutic milestone. After the approval of front-line single agent pembrolizumab, IO-based combinations are rapidly entering clinical practice resulting in a fast change of treatment algorithms for advanced NSCLC.

Ileal ulcers due to gastrointestinal amyloidosis as an unusual cause of severe lower gastrointestinal bleeding.

Amyloidosis is a chronic multisystem disease that could show multitude of nonspecific symptoms. Gastrointestinal amyloidosis is a very unusual cause of lower gastrointestinal bleeding and it does not usually lead the patient to death. We report a case of a 73-year-old woman who presented a severe, refractory lower gastrointestinal bleeding secondary to ileal ulcers due to amyloid deposit.

ACO: Time to move from the description of different phenotypes to the treatable traits.

Background: Asthma-COPD overlap (ACO) is a term that encompasses patients with characteristics of two conditions, smoking asthmatics or COPD patients with asthma-like features such as high bronchodilator response or blood eosinophil count ≥300 cells/μL. The aim of this study was to compare the different phenotypes inside the ACO definition in a real-life population cohort.

Methods: We analyzed patients from the MAJORICA cohort who had a diagnosis of asthma and/or COPD based on current guidelines, laboratory data in 2014 and follow-up until 2015.

Duodenal villous atrophy with a negative serology induced by mycophenolate mofetil: not everything is celiac disease.

Mycophenolate mofetil (MMF) is an immunosuppressive agent that is used in transplanted patients, with frequent gastrointestinal adverse effects. We report the case of a patient, under chronic therapy with mycophenolate mofetil , during a diagnostic workup for a chronic diarrhea, which presents a duodenal villous atrophy ( VA) with negative celiac serology, which is a diagnostic challenge. VA secondary to MMF is a very unusual adverse effect.

Chilaiditi syndrome complicated by a sigmoid volvulus and hepatic displacement.

Chilaiditi's sign is an anatomical alteration consisting of the transposition of the small intestine or colon between the liver and diaphragm that is asymptomatic and is usually an accidental radiological finding. The onset of Chilaiditi syndrome is accompanied by clinical symptoms and is even rarer. Moreover, the combination of sigmoid volvulus and Chilaiditi syndrome is extremely rare, with only 17 previous published cases, one of which occurred in a female patient.

Latin America validation of FACED score in patients with bronchiectasis: an analysis of six cohorts.

Background: The FACED score is an easy-to-use multidimensional grading system that has demonstrated an excellent prognostic value for mortality in patients with bronchiectasis. A Spanish group developed the score but no multicenter international validation has yet been published.

Methods: Retrospective and multicenter study conducted in six historical cohorts of patients from Latin America including 651 patients with bronchiectasis.

Intracellular activity of tedizolid phosphate and ACH-702 versus Mycobacterium tuberculosis infected macrophages.

Background: Due to the emergency of multidrug-resistant strains of Mycobacterium tuberculosis, is necessary the evaluation of new compounds.

Findings: Tedizolid, a novel oxazolidinone, and ACH-702, a new isothiazoloquinolone, were tested against M. tuberculosis infected THP-1 macrophages.

Influence of polyvascular disease on cardiovascular event rates. Insights from the REACH Registry.

Cardiovascular event rates have been shown to increase substantially with the number of symptomatic disease locations. We sought to assess the risk profile, management and subsequent event rates of polyvascular disease patients. Consecutive outpatients were assessed for atherosclerotic risk factors and medications in the REACH Registry.

Experience with t-PA treatment in a large South American city.

Background And Purpose: Early thrombolysis with intravenous recombinant tissue plasminogen activator (t-PA) reduces disability following ischemic stroke. However, only a small fraction of the potential candidates receive therapy. We studied the proportion of eligible patients who did not receive t-PA and the reasons therapy was withheld from patients admitted to a Brazilian university hospital.

The difficult access to morphology of the heart: clinical implications.

The literature on the morphology of the heart is reviewed within the context of recent histological findings. There is strong evidence for a dualistic myocardial function, whereby both ventricular constricting and expanding forces are supposed to act synchronously although with variable effect over the heart cycle.The morphological basis of this dualistic myocardial function is the contorted rope-like structure worn into the bulk of the heart muscle.

Effect of cyclosporin on plasma lipoprotein lipase activity in rats.

The effects of cyclosporin on plasma lipoproteins and lipoprotein lipase (LPL) activity were studied in rats treated with different doses of the drug for periods ranging between 7 and 30 days. The treatment with cyclosporin resulted in an increase in plasma triglycerides and non-HDL-cholesterol, and a dose and time-dependent decrease of LPL activity and HDL-cholesterol, mainly because of a fall in the HDL2-cholesterol subfraction. The decrease of LPL activity was positively correlated (p < 0.

Effect of cyclosporin on plasma lipoproteins in bone marrow transplantation patients.

The effects of cyclosporin and prednisone on plasma lipid and lipoprotein levels were studied in 20 allogeneic bone-marrow transplantation patients receiving cyclosporin plus prednisone therapy, and in 14 allogeneic patients treated only with cyclosporin during 100 days. Eighteen autologous bone-marrow patients not requiring cyclosporin were used as a control group. Patients were studied 5 days prior to transplantation, and on days 30, 60, and 100 after transplantation.