Total hip arthroplasty for a woman with hemophilia A -case report-

Publication date: July 2019Source: Annals of Medicine and Surgery, Volume 43Author(s): Akio Kanda, Kazuo Kaneko, Osamu Obayashi, Atsuhiko Mogami, Itaru MorohashiAbstractHemophilia A is a congenital bleeding disorder caused by an X-linked hereditary pattern. Female hemophilia A carriers are usually asymptomatic, although some have far lower levels of clotting factor because more X chromosomes with the normal gene are switched off, a phenomenon referred to as "lyonization.” During a medical checkup at our hospital, a 56-year-old Japanese woman with coxalgia was also diagnosed as an obligate hemophilia A carrier based on World Federation of Hemophilia criteria. She underwent total hip arthroplasty using blood product coagulation factor VIII to address her hemophilia. Immediate female relatives (mother, sisters, daughters) of a person with hemophilia should have their clotting factor levels checked, especially prior to any invasive intervention or childbirth, or if any symptoms occur.
Source: Annals of Medicine and Surgery - Category: General Medicine Source Type: research

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AbstractEmicizumab (Hemlibra®), a recombinant, humanized, bispecific monoclonal antibody, restores the function of missing activated factor VIII (FVIII) by bridging FIXa and FX to facilitate effective haemostasis in patients with haemophilia A. Subcutaneous emicizumab is approved in several countries, including in the USA and Japan, for the routine prophylaxis of bleeding episodes in patients with haemophilia A with or without FVIII inhibitors. It is also approved in the EU for the routine prophylaxis of bleeding episodes in patients with haemophilia A with inhibitors or severe haemophilia A without inhibitors. In phas...
Source: Drugs - Category: Drugs & Pharmacology Source Type: research
Conclusions: This study suggests that prophylaxis with emicizumab results in cost savings compared to FVIII prophylaxis in HA. PMID: 31530050 [PubMed - as supplied by publisher]
Source: Journal of Medical Economics - Category: Health Management Tags: J Med Econ Source Type: research
CONCLUSION: As hemophilia is an orphan disease, patients are mainly treated in specialized centers. For patients who live far from these centers or have limited access to a training there for other reasons, the physical medicine consultation hour and the implementation of online exercise instructions offer individually adapted exercise information for a regular home-based training to benefit from increased physical fitness and joint stability. PMID: 31535221 [PubMed - as supplied by publisher]
Source: Wiener Klinische Wochenschrift - Category: General Medicine Authors: Tags: Wien Klin Wochenschr Source Type: research
Hemophilia A is a genetic disorder through which patients suffer from recurrent bleeding. This can be caused by a defect in human plasma coagulation Factor VIII. High incidence of FVIII inhibitors in some severe hemophilia A patients after FVIII therapy is a considerable complication. Determination of good predictive factors can improve the safety of this treatment. HLA-II have been shown as a predictive element for inhibitor development. The goal of this study is to determine the association between HLA-DRB1*15:03, HLA-DRB1*11 and HLA-DRB1*01:01 alleles and FVIII inhibitors in severe hemophilia A patients in Iran.
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
This study reports the clinical characteristics and treatment outcomes of a relatively sizable cohort of patients with AHA. Methods: We retrospectively analyzed the characteristics and outcomes of 42 patients with AHA diagnosed in our center from January 2014 through December 2018. Results: The FVIII activity (FVIII: C) was significantly suppressed (median 1.5%; interquartile range [IQR]: 0.9-3.5) by FVIII inhibitor (median 8 BU/mL; IQR: 4.0-16.0). Bypassing agents, PCC or FVIIa, were used in 14 patients for bleeding control without any adverse reaction; and most patients (90.5%, 38/42) were placed on immunosuppressive reg...
Source: Hematology - Category: Hematology Tags: Hematology Source Type: research
This article describes the most common pathological coagulation disorders and provides information to identify them preoperatively. Furthermore, surgical strategy considerations for the use of certain implant types in this patient group are discussed. PMID: 31505698 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Orthopadie und Unfallchirurgie - Category: Orthopaedics Tags: Z Orthop Unfall Source Type: research
J Knee Surg DOI: 10.1055/s-0039-1696691Operations on patients with hemophilia A and B are complex. Studies evaluating postoperative outcomes and costs following total knee arthroplasty (TKA) in patients with hemophilia are limited. A retrospective review of the entire Medicare dataset from 2005 to 2014 was performed. International Classification of Disease 9th revision codes were used to identify patients with hemophilia A and B and they were matched to controls using a 1:1 random matching process based on age, gender, Charlson Comorbidity Index (CCI), and select comorbidity burden. The 90-day preoperative period was evalu...
Source: Journal of Knee Surgery - Category: Orthopaedics Authors: Tags: Original Article Source Type: research
Haemophilia A is a hereditary bleeding disorder, which has been considered rare and chronic. The burden of this disease in Portugal remains unknown. The aim of this study was to estimate the annualized cost an...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
CONCLUSION: rIX-FP was associated with substantial reductions in bleeding rates and consumption of FIX compared with standard half-life products that require more frequent administration. PMID: 31469321 [PubMed - as supplied by publisher]
Source: Current Medical Research and Opinion - Category: Research Tags: Curr Med Res Opin Source Type: research
In high-income countries persons with severe hemophilia (PWH) A and B are aging, like their age-matched peers without hemophilia from the general population. Aging is associated not only with the comorbidities stemming from their inherited bleeding disorder (arthropathy, chronic viral infections such as hepatitis and AIDS) but also with the multiple chronic ailments associated with aging (cancer, cardiovascular disease, COPD).  Multimorbidity is inevitably associated with polypharmacy, i.e., the chronic daily intake of at least five drugs, and with the related risk of severe adverse events associated with the use of i...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
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