Margit Serban1, Dan Vasile Poenaru2, Jenel Marian Patrascu2, Ladislau Ritli3, Eugen Boia4, Delia Maria Mihailov5, Cristina Emilia Ursu1, Delia Savescu6, Ioana Ionita7, Adina Traila8, Simona Cerbu9, Estera Boeriu10, Cristian Jinca10, Wolfgang Schramm11, Smaranda Arghirescu10. 1. Department of Hemato-Oncology, Emergency Children's Hospital "Louis Turcanu," European Haemophilia Treatment Center, Timisoara, Timis, Romania. 2. Department of Orthopaedics, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 3. Department of Pediatric Hemato-Oncology, University of Medicine and Pharmacy, County Hospital, Oradea, Bihor, Romania. 4. Department of Pediatric Surgery, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 5. Department of Paediatrics, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 6. Department of Laboratory, Children's Emergency Hospital "Louis Turcanu," Timisoara, Timis, Romania. 7. Department of Hematology, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 8. European Hemophilia Treatment Center, Medical Center for Children and adolescents "C. Serban" Buzias, Timis, Romania. 9. Department of Radiology, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 10. Department of Pediatric Hemato-Oncology, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania. 11. Rudolf Marx Stiftung Bluterbetreuung Bayern e. V. (BBB) - Universität München Germany.
Abstract
OBJECTIVE: In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. PATIENTS AND METHODS: This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. RESULTS AND DISCUSSION: The cohort of patients consisted mostly of young adults, aged between 7 and 49 years. The vast majority of them, 91.3% of the patients, had the burden of severe chronic arthropathy. This was the reason for 95 interventions, with programmed solution. In 20 cases the cause of invasive interventions was emergency. Knee and hip replacement represented 28% of the major interventions. The complications we encountered were excessive bleeding (12.2%), infection (13%) and inhibitor development (4.3%). CONCLUSION: Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations. Georg Thieme Verlag KG Stuttgart · New York.
OBJECTIVE: In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. PATIENTS AND METHODS: This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. RESULTS AND DISCUSSION: The cohort of patients consisted mostly of young adults, aged between 7 and 49 years. The vast majority of them, 91.3% of the patients, had the burden of severe chronic arthropathy. This was the reason for 95 interventions, with programmed solution. In 20 cases the cause of invasive interventions was emergency. Knee and hip replacement represented 28% of the major interventions. The complications we encountered were excessive bleeding (12.2%), infection (13%) and inhibitor development (4.3%). CONCLUSION: Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations. Georg Thieme Verlag KG Stuttgart · New York.