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Coexisting or Underlying Risk Factors of Hepatic Veno-Occlusive Disease in Pediatric Hematopoietic Stem Cell Transplant Recipients Receiving Prophylaxis

机译:接受预防的小儿造血干细胞移植受者肝静脉阻塞性疾病并存或潜在危险因素

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Objectives: To evaluate the characteristics of veno-occlusive disease in pediatric hematopoietic stem cell transplant recipients, and their effect as a prophylactic regimen on severity and outcome. Materials and Methods: This study included 204 allogeneic hematopoietic stem cell transplants performed on 187 children whose data retrospectively described the risk factors, prophylaxis, and treatment modalities of veno-occlusive disease. A prophylactic regimen composed of enoxaparin versus ursodeoxycholic acid and vitamin E was given to 167 of 204 patients. Results: Veno-occlusive disease developed in 22 patients (10.8%). Nineteen patients experienced veno-occlusive disease despite this prophylactic regimen. The prophylaxis seemed ineffective in preventing veno-occlusive disease ( P = .657). Regarding risk factors, oral busulphan use, liposomal amphotericin B vancomycin treatment, and total parenteral nutrition were associated with an increased risk of veno-occlusive disease. Conversely, renal impairment also was associated with increased mortality in patients with veno-occlusive disease. The mortality rate in the first 100 days after a hematopoietic stem cell transplant was higher in the patients with veno-occlusive disease than it was in those without the disease. Conclusions: Our prophylactic regimen may have played a role in the fairly low incidence of veno-occlusive disease in a pediatric population with high-risk features.
机译:目的:评估小儿造血干细胞移植受者静脉闭塞性疾病的特征,以及它们作为预防方案对严重程度和预后的影响。资料和方法:这项研究包括对187名儿童进行的204次同种异体造血干细胞移植,其数据回顾性地描述了静脉闭塞性疾病的危险因素,预防和治疗方式。对204例患者中的167例患者采取了由依诺肝素与熊去氧胆酸和维生素E组成的预防方案。结果:22例患者发生了闭塞性静脉疾病(10.8%)。尽管采取了这种预防性治疗方案,但仍有19名患者发生了静脉阻塞性疾病。预防似乎对预防静脉阻塞性疾病无效(P = .657)。关于危险因素,口服氟尿嘧啶,口服脂质体两性霉素B万古霉素治疗和全胃肠外营养与静脉闭塞性疾病风险增加有关。相反,肾功能不全也与静脉闭塞性疾病患者的死亡率增加有关。静脉闭塞性疾病患者造血干细胞移植后前100天的死亡率要高于无此疾病的患者。结论:我们的预防方案可能在具有高风险特征的儿童人群中,静脉闭塞性疾病的发生率相对较低。

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