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Background: Advances in blood conservation have reduced the need for allogeneic transfusions in total knee and hip arthroplasty (TKA/THA). This study aimed to assess whether perioperative bleeding complications, including hemorrhage/hematoma, allogeneic transfusions, and postoperative anemia, occurred at similar rates between patients with hereditary bleeding disorders (BDs) and controls. Using a national health care database, we assessed the use of clotting factor concentrates (CFCs), perioperative outcomes, and resource utilization.
Methods: A retrospective cohort study was conducted using the Premier Health Database (2017-2021) to analyze differences in outcomes and costs between 1528 patients with hereditary BDs and 20,509 non-BD controls undergoing elective TKA and THA. Summary statistics, bivariate analyses, and odds ratios (ORs) were used to evaluate perioperative outcomes and resource use.
Results: Patients with hereditary BDs were slightly younger, predominantly female, and more often treated at larger, urban hospitals compared to controls. Bleeding complications, including hemorrhage and hematoma, were infrequent but higher in the BD group (1.1% vs 0.2%; P < .0001). Transfusion rates were higher in THA than TKA, with significantly increased odds for patients with hereditary BD: for THA, OR 2.7 (95% confidence interval [CI], 2.0-3.7; P < .0001); and for TKA, OR 2.6 (95% CI, 1.9-3.8; P < .0001). CFC exposures occurred in 16.4% of patients with hereditary BD compared to 0.03% in controls. Of 270 reported CFC exposures, factor VIII (FVIII) and von Willebrand factor (VWF) were most commonly used (49.6% and 23.0%, respectively), followed by FIX concentrate (12.6%) and bypassing agents, including FVIIa (8.5%) and anti-inhibitor coagulant complex (AICC; 3.7%). Antifibrinolytic therapy was administered in most cases. Pharmacy costs for patients with hereditary BD were significantly higher, with a mean of $23,792 (95% CI, $8722-$39,312), being over 30 times the mean cost in controls ($750; 95% CI, $739-$762). Other outcomes were not different, except for a higher incidence of venous thromboembolism in the BD group (OR 3.9, 95% CI, 2.4-6.1; P < .0001).
Conclusions: THA and TKA in patients with hereditary BDs are relatively safe, with most outcomes comparable to controls. However, higher rates of bleeding, transfusion, and VTE underscore the need for optimizing anemia management and targeted use of CFCs along with antifibrinolytic therapy.
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http://dx.doi.org/10.1213/ANE.0000000000007478 | DOI Listing |
Front Allergy
August 2025
Internal Medicine, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Targu Mures, Romania.
Introduction: In the majority of patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (HAE-C1INH), effective long-term prophylactic (LTP) treatment can achieve complete disease control. Lanadelumab is one of the first-line option recommended for this purpose. Our study aimed to evaluate changes in disease control, quality of life, and attack frequency among Romanian HAE-C1INH patients, during the first year of treatment with lanadelumab.
View Article and Find Full Text PDFFront Med (Lausanne)
August 2025
Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Chronic intestinal pseudo-obstruction (CIPO) is a rare and severe intestinal motility disorder with poor long-term prognosis and high mortality rate, especially when the small intestine is involved. Due to the non-specificity of clinical symptoms, CIPO has long faced diagnostic challenges. With the advancements of sequencing technology, many hereditary CIPOs have been identified.
View Article and Find Full Text PDFBiomed Eng Lett
September 2025
Department of Cardiovascular Ultrasound, The First Hospital of China Medical University, Shenyang, China.
Abstract: Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease and is the leading cause of sudden cardiac death in adolescents. Septal hypertrophy (SH) and apical hypertrophy (AH) are two common types. The former is characterized by abnormal septal myocardial thickening and the latter by left ventricular apical hypertrophy, both of which significantly increase the risk of heart failure, arrhythmias, and other serious complications.
View Article and Find Full Text PDFHematology
December 2025
Thalassaemia International Federation (TIF), Nicosia, Cyprus.
Objective: The complexity of β-thalassaemia with associated morbidity, lifelong daily expensive treatment, and multidisciplinary care results in a considerable disease burden. Our study aimed to revisit the β-thalassaemia burden using epidemiological, clinical, and financial indicators related to patients, families, and healthcare systems.
Methods: Patient density measures, transfusion indices, complication rates, universal health coverage, and other indicators were tabulated by country and region.
Korean J Clin Oncol
August 2025
Department of Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea.
Purpose: Multiple primary tumors arising in the same individual pose challenges for precision oncology, particularly in the context of hereditary cancer syndromes such as Lynch syndrome. While these tumors may originate from a shared germline predisposition, it remains unclear whether they also share somatic alterations that could be therapeutically exploited. This study aimed to characterize the extent of somatic genomic overlap between synchronous or metachronous gastric and colorectal cancers within young Korean patients.
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