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.

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.

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.

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.