E. C. Rodriguez-Merchan

Bio: E. C. Rodriguez-Merchan is an academic researcher from Hospital Universitario La Paz. The author has contributed to research in topics: Haemophilia & Medicine. The author has an hindex of 38, co-authored 165 publications receiving 4054 citations.

Consensus perspectives on prophylactic therapy for haemophilia: summary statement.

Abstract: Participants in an international conference on prophylactic therapy for severe haemophilia developed a consensus summary of the findings and conclusions of the conference. In the consensus, participants agreed upon revised definitions for primary and secondary prophylaxis and also made recommendations concerning the need for an international system of pharmacovigilance. Considerations on starting prophylaxis, monitoring outcomes, and individualizing treatment regimens were discussed. Several research questions were identified as needing further investigation, including when to start and when to stop prophylaxis, optimal dosing and dose interval, and methods for assessment of long-term treatment effects. Such studies should include carefully defined cohorts, validated orthopaedic and quality-of-life assessment instruments, and cost-benefit analyses.

Pediatric fractures of the forearm.

Abstract: Forearm fractures are common injuries in childhood. There are a number of important principles that should be followed to achieve the ideal goal of fracture healing without deformity or dysfunction. I will review the general principles, classifications, diagnosis, treatment, and complications of pediatric forearm fractures, including some specific injuries such as Monteggia fractures, Galeazzi injuries, and open fractures. The basic principle is to accurately align the fracture fragments and to maintain this position until the fracture is united. Forearm fractures in children can be treated differently from adult fractures because of continuing growth in both bones (radius and ulna) after the fracture has healed. As long as the physes are open, remodeling can occur. However, generally it is thought that rotational deformity does not remodel. Undisplaced fractures may be treated in a cast until the fracture site is no longer painful. Most displaced fractures of the forearm are best maintained in a long arm cast. However, redisplacement occurs in 7 to 13% of cases, usually within 2 weeks of injury. Unstable metaphyseal fractures should be percutaneously pinned. Unstable diaphyseal fractures can be stabilized by intramedullary fixation of the radius and ulna. If none of these techniques is helpful, plate and screw fixation is the best choice.

Joint protection in haemophilia

Abstract: Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in haemophilia, although magnetic resonance imaging remains the gold standard as far as imaging techniques are concerned. Some patients experience subclinical haemarthroses, which eventually tend to result in some degree of arthropathy, especially in the ankles. Nowadays, the most effective way of protecting these patients is primary prophylaxis, which in practice changes severe haemophilia into moderate haemophilia, preventing or at least minimizing the occurrence of haemarthrosis. If primary prophylaxis is, for whatever reason not an option, secondary prophylaxis and enhanced on demand treatment should be considered. Two alternatives are available for inhibitor patients: (i) control of haemostasis using by-passing agents (rFVIIa or aPCCs) either as enhanced on demand treatment or secondary prophylaxis, as appropriate, following the same basic principles used for non-inhibitor patients and (ii) immune tolerance induction (ITI) to eradicate the inhibitor.

Aspects of current management: orthopaedic surgery in haemophilia.

Abstract: If continuous prophylaxis is not feasible due to expense or lack of venous access, we must aggressively treat major haemarthroses (including arthrocentesis) to prevent progression to synovitis, recurrent joint bleeds, and ultimately end-stage osteoarthritis (haemophilic arthropathy). For the treatment of chronic haemophilic synovitis, radiosynovectomy should always be indicated as the first procedure. If, after three procedures with 6-month interval, radiosynovectomy fails, an arthroscopic synovectomy must be indicated. Between the second and fourth decades, many haemophilic patients develop joint destruction (arthropathy). At this stage possible treatments include alignment osteotomy, arthroscopic joint debridement, arthrodesis (joint fusion) and total joint arthroplasty. For the hip press-fit uncemented components (hemispherical acetabulum, flanged femoral stem, metal-to-polyethylene) are recommended whilst for the knee a posterior-stabilized (PS) cemented design is advised. Muscular problems must not be underestimated in haemophilia due to their risk of developing compartment syndromes (which will require surgical decompression) and pseudotumours (which will require surgical removal or percutaneous treatment). Regarding patients with inhibitors, the advent of APCCs and rFVIIa has made major orthopaedic surgery possible, leading to an improved quality of life for haemophilia patients. Concerning local fibrin seal, it is not always necessary to achieve haemostasis in all surgical procedures performed in persons with haemophilia. However, it could be a good adjunct therapy, mainly when a surgical field potentially will bleed more than expected (i.e. patients with inhibitors), and also in some orthopaedic procedures (mainly the surgical removal of pseudotumours).

Total knee replacement in haemophilic arthropathy

Abstract: The results of primary total knee replacement performed on a group of haemophiliac patients in a single institution by the same surgeon using the same surgical technique and prosthesis are reported. A total of 35 primary replacements in 30 patients were carried out between 1996 and 2005 and were reviewed retrospectively. The mean age of the patients was 31 years (24 to 42) and the mean follow-up was for 7.5 years (1 to 10). There were 25 patients with haemophilia A and five with haemophilia B. The HIV status and CD4 count were recorded, and Knee Society scores determined. Two patients had inhibitors to the deficient coagulation factor. There were no early wound infections and only one late deep infection which required a two-stage revision arthroplasty, with a good final result. The incidence of infection in HIV-positive and negative patients was thus similar. One knee in a patient with inhibitor had excessive bleeding due to a pseudoaneurysm which required embolisation. The results were excellent in 27 knees (77%), good in six (17%) and fair in two (6%). The survival rate at 7.5 years taking removal of the prosthesis for loosening or infection as the end-point was 97%. The mechanical survival of total knee replacements in haemophiliacs is very good. Our results confirm that this is a reproducible procedure in haemophilia, even in HIV-positive patients with a CD4 count > 200 mm(3) and those with inhibitors. Our rate of infection was lower than previously reported. This could be due to better control of the HIV status with highly active anti-retroviral therapy and the use of antibiotic-loaded cement.

Guidelines for the management of hemophilia.

Abstract: Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.

Management of severe perioperative bleeding Guidelines from the European Society of Anaesthesiology

Abstract: The aims of severe perioperative bleeding management are three-fold. First, preoperative identification by anamesis and laboratory testing of those patients for whom the perioperative bleeding risk may be increased. Second, implementation of strategies for correcting preoperative anaemia and stabilisation of the macro- and microcirculations in order to optimise the patient’s tolerance to bleeding. Third, targeted procoagulant interventions to reduce the amount of bleeding, morbidity, mortality and costs. The purpose of these guidelines is to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthetists throughout Europe to integrate this knowledge into daily patient care wherever possible. The Guidelines Committee of the European Society of Anaesthesiology (ESA) formed a task force with members of scientific subcommittees and individual expert members of the ESA. Electronic databases were searched without language restrictions from the year 2000 until 2012. These searches produced 20 664 abstracts. Relevant systematic reviews with meta-analyses, randomised controlled trials, cohort studies, case-control studies and cross-sectional surveys were selected. At the suggestion of the ESA Guideline Committee, the Scottish Intercollegiate Guidelines Network (SIGN) grading system was initially used to assess the level of evidence and to grade recommendations. During the process of guideline development, the official position of the ESA changed to favour the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. This report includes general recommendations as well as specific recommendations in various fields of surgical interventions. The final draft guideline was posted on the ESA website for four weeks and the link was sent to all ESA members. Comments were collated and the guidelines amended as appropriate. When the final draft was complete, the Guidelines Committee and ESA Board ratified the guidelines.

Current concepts review

Abstract: umeroscapular arthrodesis is a well-established operative procedure that involves fusion of the humeral head to the glenoid. In some procedures, the fusion also includes an acromiohumeral arthrodesis. Humeroscapular arthrodesis is commonly called “shoulder arthrodesis,” and we use that term in this paper for simplicity. Indications for this procedure early in the twentieth century included the treatment of residual glenohumeral destruction resulting from tuberculosis and the treatment of upper-extremity paralysis resulting from poliomyelitis. Additional historical indications included osteoarthritis, rheumatoid arthritis, irreparable injury of the rotator cuff, and severely comminuted fracture of the proximal aspect of the humerus. The advent of shoulder arthroplasty has resulted in a marked reduction in the number of shoulder arthrodeses performed, although there are instances when arthrodesis is favored over joint-replacement arthroplasty. This article reviews the indications for shoulder arthrodesis, the pertinent features of the preoperative evaluation, the controversial issue of the desirable position of the arthrodesis, the various techniques for shoulder arthrodesis, and the management of complications.

Guidelines for the use of fresh-frozen plasma, cryoprecipitate and cryosupernatant.

Abstract: The indications for transfusing fresh-frozen plasma (FFP), cryoprecipitate and cryosupernatant plasma are very limited. When transfused they can have unpredictable adverse effects. The risks of transmitting infection are similar to those of other blood components unless a pathogen-reduced plasma (PRP) is used. Of particular concern are allergic reactions and anaphylaxis, transfusion-related acute lung injury, and haemolysis from transfused antibodies to blood group antigens, especially A and B. FFP is not indicated in disseminated intravascular coagulation without bleeding, is only recommended as a plasma exchange medium for thrombotic thrombocytopenic purpura (for which cryosupernatant is a possible alternative), should never be used to reverse warfarin anticoagulation in the absence of severe bleeding, and has only a very limited place in prophylaxis prior to liver biopsy. When used for surgical or traumatic bleeding, FFP and cryoprecipitate doses should be guided by coagulation studies, which may include near-patient testing. FFP is not indicated to reverse vitamin K deficiency for neonates or patients in intensive care units. PRP may be used as an alternative to FFP. In the UK, PRP from countries with a low bovine spongiform encephalopathy incidence is recommended by the Departments of Health for children born after 1 January 1996. Arrangements for limited supplies of single donor PRP of non-UK origin are expected to be completed in 2004. Batched pooled commercially prepared PRP from donors in the USA (Octaplas) is licensed and available in the UK. FFP must be thawed using a technique that avoids risk of bacterial contamination. Plastic packs containing any of these plasma products are brittle in the frozen state and must be handled with care.

WFH Guidelines for the Management of Hemophilia, 3rd edition

Abstract: Alok Srivastava 1 | Elena Santagostino 2 | Alison Dougall 3 | Steve Kitchen 4 | Megan Sutherland 5 | Steven W. Pipe 6 | Manuel Carcao 7 | Johnny Mahlangu 8 | Margaret V. Ragni 9 | Jerzy Windyga 10 | Adolfo Llinás 11 | Nicholas J. Goddard 12 | Richa Mohan 13 | Pradeep M. Poonnoose 14 | Brian M. Feldman 15 | Sandra Zelman Lewis 16 | H. Marijke van den Berg 17 | Glenn F. Pierce 18 | on behalf of the WFH Guidelines for the Management of Hemophilia panelists and co-authors*