Efrati Ori, Toren Amos, Duskin Hadar, Modan-Moses Dalit, Bielorai Bella, Goldstein Gal, Churi Chaim, Vilozni Daphna
Pediatric Pulmonary Unit, The Edmond and Lili Safra Children's Hospital, Sheba Medical Center (affiliated with Sackler School of Medicine), Tel-Aviv University, Ramat-Gan, Israel.
Pediatr Blood Cancer. 2008 Nov;51(5):684-8. doi: 10.1002/pbc.21722.
Management of pediatric patients with malignant and hematological diseases is frequently associated with pulmonary complications. We assessed pulmonary function at diagnosis and during a 5-year follow-up to identify risk factors associated with pulmonary deterioration.
Ninety patients (age range 3-20) who were treated at the Pediatric Hematology-Oncology Department, Sheba Medical Center, Israel, were entered into the study. Pulmonary function testing was performed at diagnosis and at least twice during the study period.
At diagnosis and thereafter values of spirometry, total lung capacity, functional residual capacity and diffusion capacity were significantly lower than predicted (P < 0.002 for all indices). The ratio between residual volume and total lung capacity (RV/TLC) was significantly higher than normal at diagnosis and throughout the study (P < 0.001). Age and treatment modalities did not show any effect on lung-function during the study. A subgroup of seven patients (8%) developed Bronchiolitis obliterans (BO) after stem cell transplantation and development of graft versus host disease (GVHD). These patients' baseline FEF25-75 values (small airway disease) were significantly lower than FEF25-75 values of controls and other patients while all other parameters were similar. The RV/TLC in the BO patients gradually increased relative to other patients during the 5-year follow-up.
Lung-function in pediatric hemato-oncological patients at diagnosis is lower than predicted. Abnormal baseline FEF25-75 may be a risk factor for the development of BO in the setting of GVHD after treatment. Careful monitoring, especially of FEF25-75 and RV/TLC at baseline and in the first period after diagnosis.
小儿恶性和血液系统疾病的治疗常伴有肺部并发症。我们评估了诊断时及5年随访期间的肺功能,以确定与肺功能恶化相关的危险因素。
纳入以色列谢巴医疗中心儿科血液肿瘤科治疗的90例患者(年龄3 - 20岁)。在诊断时及研究期间至少进行两次肺功能测试。
诊断时及之后,肺活量测定、肺总量、功能残气量和弥散量的值均显著低于预测值(所有指标P < 0.002)。诊断时及整个研究期间,残气量与肺总量之比(RV/TLC)显著高于正常水平(P < 0.001)。研究期间,年龄和治疗方式对肺功能无任何影响。7例患者(8%)在干细胞移植及移植物抗宿主病(GVHD)发生后发展为闭塞性细支气管炎(BO)。这些患者的基线FEF25 - 75值(小气道疾病)显著低于对照组和其他患者,而所有其他参数相似。在5年随访期间,BO患者的RV/TLC相对于其他患者逐渐增加。
小儿血液肿瘤患者诊断时的肺功能低于预测值。基线FEF25 - 75异常可能是治疗后GVHD情况下发生BO的危险因素。需仔细监测,尤其是基线及诊断后第一阶段的FEF25 - 75和RV/TLC。