Department of Hemato-Oncology, Emergency Children's Hospital "Louis Turcanu," European Haemophilia Treatment Center, Timisoara, Timis, Romania.
Department of Orthopaedics, University of Medicine and Pharmacy "Victor Babes," Timisoara, Timis, Romania.
Hamostaseologie. 2019 Nov;39(4):377-382. doi: 10.1055/s-0039-1677884. Epub 2019 Feb 13.
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.
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.
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%).
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.
在因子浓缩物使用率较低的国家，致残性关节疾病仍然是血友病患者的主要疾病负担。本分析的目的是表达我国在缺乏预防方案的情况下，因子替代治疗使用率低的情况下，侵袭性矫形干预的趋势和特征。
这是一项在蒂米什瓦拉大学中心进行的回顾性描述性研究，该中心在血友病护理方面拥有长期经验，并成功发展了一种非常有价值的真正全面的机构间合作。本研究涉及 2001 年至 2017 年间在 97 名患者中进行的 115 次侵袭性干预：83 名血友病 A 患者，10 名血友病 B 患者和 4 名血管性血友病患者；其中 17 名患者有抑制剂，5 名患者在干预后产生抑制剂。
患者队列主要由 7 至 49 岁的年轻成年人组成。他们中的绝大多数，91.3%的患者，患有严重慢性关节炎病。这是 95 次有计划解决方案的侵袭性干预的原因。在 20 例中，侵袭性干预的原因是急诊。膝关节和髋关节置换占主要干预的 28%。我们遇到的并发症包括过度出血（12.2%）、感染（13%）和抑制剂发展（4.3%）。
手术是血友病患者的一项高要求干预措施，不幸的是，在我国这种情况无法被忽视。希望，改善替代治疗的可及性和可及性将消除这些干预的必要性，为下一代优化结果。
