PubMed Health⌕ Search

Biomedical subjects

E C Rodriguez-Merchan

Publications and source records attributed to E C Rodriguez-Merchan.

At least 19 recordsLinked to original sources

HIV and HCV coinfected haemophilia patients: what are the best options of orthopaedic treatment?

In the era of contemporary orthopaedics, haematology and internal medicine, it is obvious that surgery can be indicated in human immunodeficiency virus (HIV) and hepatitis C virus (HCV) coinfected haemophilia patients suffering from severe and painful musculoskeletal problems. However, the expected high risk of infection and other postoperative complications is a concern due to the poor health status that many of these patients present. In a HIV and HCV coinfected haemophilia patient, the orthopaedic surgeon together with the multidisciplinary haemophilia team should weigh the risks and benefits carefully. Clinical and immunological status should be considered before suggesting a surgical procedure (specially a joint arthroplasty) in this group of patients. If a surgical procedure is contraindicated, conservative treatment could be an alternative, although many times with inferior results. Usually, surgical procedures can better relieve pain for several years and improve the quality of life in this cohort of patients. Regarding anaesthetics, drugs metabolized by the liver or the kidney should be avoided depending on every particular case. Rachianaesthesia is more recommendable than epidural anaesthesia in elderly patients in whom general anaesthesia sometimes could be dangerous, although in all ages we prefer general anaesthesia. The size of the needle should be small (size G-27), and we never use spinal catheters.

Arthroplasty, Replacement↗

The haemophilic ankle.

Radiation synoviorthesis is a very effective procedure that decreases both the frequency and the intensity of recurrent ankle bleeds related to ankle synovitis. The procedure should be performed as soon as possible to minimize the degree of articular cartilage damage. It can also be used in patients with inhibitors with minimal risk of complications. On average, the efficacy of the procedure ranges from 76% to 80%, and can be performed at any age. The procedure slows the cartilaginous damage which intra-articular blood tends to produce in the long term. After 30 years of using radiation synovectomy worldwide, no damage has been reported in relation to the radioactive materials. Radiation synovectomy is currently the preferred procedure when radioactive materials are available; however, chemical synoviorthesis is an effective alternative method if radioactive materials are not available. Personal experience and the general recommendation among orthopaedic surgeons and haematologists is that when three early consecutive synoviorthesis (repeated every 3 months) fail to halt synovitis, a surgical synovectomy (open or by arthroscopy) should be immediately considered. For advanced haemophilic arthropathy of the ankle, the best solution is an ankle arthrodesis. Primary prophylaxis and radioactive synoviorthesis are the best ways that we have today of protecting against haemophilic synovitis and arthropathy of the ankle joint.

Ankle Joint↗

Literature review of surgery management in inhibitor patients.

A careful review of the available literature has shown with a moderate degree of evidence that surgery can be safely performed in haemophilic patients with either low-responding or high-responding inhibitors. Until recently, it was generally advised that surgery should be carried out in high responding haemophilic patients only if absolutely necessary, i.e. in emergency situations. Elective surgery was usually discouraged, with the exception of the placing of an indwelling catheter to carry out immune tolerance protocols. However, in reality elective surgery is often advisable in haemophilic patients with inhibitors because it may lead to an improved quality of life. At the present time, several options of treatment exist which appear to be sufficiently safe to assure haemostasis in different surgical procedures However, a more informed choice between different treatment options, based on estimation of advantages and risks linked to each treatment modality is desirable. Furthermore, the choice between different options has to be made taking into consideration costs of treatment. Ideally, randomised prospective studies are needed in order to provide a higher level of evidence of safety and efficacy of different treatment options and specifically comparing the two main bypassing options:activated prothrombin complex concentrates (aPCCs) and recombinant factor (rF)VIIa. In addition, such studies should be done with an adequate protocol, providing sufficient haematological data and an appropriate prolonged postoperative assessment.

Blood Coagulation Factors↗

Consensus perspectives on surgery in haemophilia patients with inhibitors: summary statement.

Summary. Participants in an international workshop on surgery in haemophilia patients with inhibitors developed a consensus summary of the findings and conclusions of the meeting. In the consensus, participants agreed upon revised definitions for minor and major surgery, including an intermediate degree of surgery. An evaluation system of intraoperative and postoperative bleeding was developed. Recommended doses of FEIBA((R)) and rFVIIa (both in bolus injections and in continuous infusion) for surgery were agreed. Participants also agreed on the main blood tests to be performed peri-operatively. They also suggested the need of a prospective evaluation in the future. Finally, the approximate number of surgical procedures and costs performed on haemophilia patients with inhibitors were analysed.

Adult↗

Historical and political implications of haemophilia in the Spanish royal family.

The political implications of haemophilia in the marriage of King Alfonso XIII of Spain and Princess Victoria Eugenie Battenberg of England have been reviewed in recent books on history. However, the fact that they had haemophilic sons also affected their personal relationship. In this article, we review the consequences haemophilia bore on their lives. We feel great compassion for families who suffer the illness, be it ordinary people or members of royalty; however, in this case, it can be said that when the disease affected a royal couple, the political consequences were great.

England↗

Elective orthopaedic surgery for inhibitor patients.

We report on a series of 108 elective orthopaedic surgical procedures. It includes 88 radiosynoviortheses and 20 major orthopaedic procedures, performed on 51 patients at nine centres worldwide. The average age of patients was 28.5 years (range 5-40 years), and the average follow-up time was 2 years (range 1-5 years). There were 82 good results, 15 fair and 11 poor. In the synoviorthesis group (41 patients, 88 synoviortheses) the average age was 14.3 years (range 5-40 years) and the average follow-up was 6.5 years (range 1-10 years). There were 66 good results, 14 fair and eight poor. There were no complications. In the group of major orthopaedic procedures, the average age of the 10 patients was 32.5 years (range 27-40 years), and the average follow-up was 2.3 years (range 1-5 years). There were 16 good results, one fair and three poor. Postoperative bleeding complications occurred in three of the 20 major orthopaedic procedures performed (15% complications rate). They occurred in three patients treated with insufficient doses of recombinant activated factor VII. Despite such complications, the study has shown that haemophilic patients with inhibitors requiring elective orthopaedic surgery (EOS) can undergo such procedures with a high expectation of success. In other words, EOS is now possible in haemophilic patients with inhibitors, leading to an improved quality of life for these patients. Thorough analysis of each case as part of a multidisciplinary team will allow us to perform elective orthopaedic procedures in patients with inhibitors.

Adolescent↗

Consensus perspectives on prophylactic therapy for haemophilia: summary statement.

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.

Consensus Statements as Topic↗

Orthopaedic assessment in haemophilia.

Outcomes may be measured in terms of both objective and subjective variables and also on the basis of cost-efficiency. Most tools currently used to quantify outcomes, especially in orthopaedics, involve measurements of general health and of specific body part or organ system function. Patients' expectations of medical care are linked to their requests for treatment and to their assessments of outcome and satisfaction. Rating results are fundamental to evaluation and comparison of different methods of treatment. The discrepancies existing among rating scales have been an impediment in the evaluation of haemophilic patients from the orthopaedic point of view. Progress depends on international cooperation to improve communication and on the evaluation of new surgical techniques. The World Federation of Haemophilia (WFH) physical joint examination instrument and the Colorado physical examination (PE) instruments made important contributions by developing forms that will serve as a foundation for future evaluation systems. Some authors have suggested that the Colorado PE instruments are more indicative of early joint dysfunction than the WFH instrument. Future goals of the Colorado PE instruments are to refine the standard forms, identify additional important reproducible criteria and develop a comprehensive method of evaluation. It is paramount to develop valid and reliable surveys that can be used preoperatively to direct patient education and shared decision making and to provide a framework for setting reasonable goals. Re-examining patients' responses postoperatively could provide a way to assess fulfilment of expectations, which is a crucial patient-derived measure of outcome and satisfaction.

Costs and Cost Analysis↗

Orthopaedic surgery in persons with haemophilia.

The orthopaedic problems of haemophilia and the surgical techniques most frequently performed by orthopaedic surgeons on haemophilia patients are summarised in this review article. Sometimes it can be recommendable to carry out two or three orthopaedic surgical procedures in a single operative session, with the aim of solving the functional problem in a more global way, which usually is polyarticular. There is no doubt that such an undertaking implies a greater anaesthetic risk, but also avoids the repetition of surgical procedures and saves factor consumption. Close cooperation between haematologists, orthopaedic surgeons, rehabilitation physicians, paediatricians, psychologists, physiotherapists and nurses is paramount for the satisfactory result of any orthopaedic surgical procedure. There is no doubt that continuous prophylactic clotting factor replacement (prophylactic therapy) is the optimal way to avoid the orthopaedic problems of haemophilia that can still be seen today. Until such a goal can be reached, the orthopaedic surgeon will continue performing arthrocentheses, synoviortheses, synovectomies, tendon lengthenings, débridements, osteotomies, joint replacements, osteosynthesis of fractures and other less frequent surgical procedures on persons with haemophilia.

Hemarthrosis↗

Orthopaedic surgery of haemophilia in the 21st century: an overview.

Close co-operation between haematologists, orthopaedic surgeons, rehabilitation physicians and physiotherapists is essential for obtaining satisfactory results after orthopaedic procedures that are performed on haemophilic patients. Although continuous prophylaxis could avoid the development of the orthopaedic complications of haemophilia that we still see in the 21st century, such a goal has not been achieved so far, not even in developed countries. Therefore, orthopaedic surgeons are still required to carry out many different surgical procedures, such as arthrocentesis, synoviorthesis, synovectomies, tendon lengthening, articular debridements, alignment osteotomies, joint arthroplasties, nerve releases, opening of compartment syndromes, removal of pseudotumours and osteosynthesis of fractures. Furthermore, the emergence of human immunodeficiency virus has meant that immunosuppressed patients in developed countries sometimes require an arthrotomy for the treatment of spontaneous septic arthritis, or the surgical drainage of a spontaneously infected haematoma (abscess). In addition, they have a high risk of postoperative infection after any surgical procedure, particularly a joint arthroplasty.

Hematoma↗

The haemophilic pseudotumour.

The management of the patient with a haemophilic pseudotumour is complex and carries a high rate of potential complications. There are a number of therapeutic alternatives for this dangerous condition: embolization, radiation, percutaneous management, surgical removal and exeresis, and filling of the dead cavity. It is hoped that with the advent of widespread maintenance therapy, pseudotumours will be less common in the future. It is important that they are diagnosed early, and prevention of muscular haematomas is key to reducing their incidence. Untreated, proximal pseudotumours will ultimately destroy soft tissues, erode bone and may produce neurovascular complications. Surgical excision is the treatment of choice but should only be carried out in major haemophilia centres by a multidisciplinary surgical team.

Bone Diseases↗