Reduction of the six-minute walk distance in children with sickle cell disease is correlated with silent infarct: results from a cross-sectional evaluation in a single center in Belgium.
Reduction of the six-minute walk distance in children with sickle cell disease is correlated with silent infarct: results from a cross-sectional evaluation in a single center in Belgium.
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DOI：
10.1371/journal.pone.0108922
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发表时间：
2014
期刊：
影响因子：
3.7
通讯作者：
Ferster A
中科院分区：
文献类型：
--
作者：
Dedeken L;Chapusette R;Lê PQ;Heijmans C;Devalck C;Huybrechts S;Ziereisen F;Hanssens L;Rozen L;Noubouossie D;Mujinga MN;Ferster A
6分钟步行试验(6MWT)被用来评估成人和儿童受各种慢性病影响的运动能力。它反映了在模拟日常生活活动的情况下，各种生理系统的整体反应。对46例镰状细胞病患儿6MWT的影响因素进行了分析。羟基脲(HU)治疗组42例。将检测正常的患者(年龄标准化预测值的80%)与检测异常的患者(≤的80%)进行比较。根据测试的表现，比较基线血液学数值、临床事件、脑血管疾病、心肺参数和疾病调整治疗。46例患者中有14例6MWT异常。在单变量分析中，两组的生物学和临床数据相似。6 MWT异常的14例患者中有6例出现无症状性脑梗塞(SI)，而试验正常的患者有6例(P = 0.0 9)。排除长期输血的患者后，6 MWT异常的11例患者中有4例出现SI，而26例患者中有1例出现SI(P = 0.02)。基础脉搏血氧饱和度在两组均正常，但6MWT异常患者的基础脉搏血氧饱和度略低(P = 0.02)。没有患者表现出运动诱导的减饱和度。在多因素分析中，6 MWT异常的唯一相关因素是SI的存在(P = 0.045)。在我们的队列中，46名患者的特点是高暴露于HU，且没有严重的心肺疾病，唯一与6MWT独立相关的因素是SI的存在。SI儿童的运动能力较低可能反映了一些亚临床神经损害，因为他们在血红蛋白水平或心肺参数方面没有区别。
The 6-minute walk test (6MWT) is used in adults and children affected by a wide range of chronic diseases to evaluate their sub-maximal exercise capacity. It reflects the global response of various physiological systems in a situation simulating a daily life activity. We analyzed factors affecting the 6MWT in 46 Sickle Cell Disease children. Forty-two were treated with hydroxyurea (HU). Patients with normal test (>80% of the age-standardized predicted value) were compared to patients with abnormal test (≤80%). Baseline hematological values, clinical events, cerebrovascular disease, cardio-pulmonary parameters and disease-modifying treatment were compared according to the performance of the test. Among the 46 patients, 14 had an abnormal 6MWT. In univariate analysis, both groups were similar for biological and clinical data. Six of the 14 patients with an abnormal 6MWT had silent infarct (SI) compared to 6/32 with a normal test (P = 0.09). When excluding chronically transfused patients, 4 of the 11 patients with an abnormal 6MWT had SI compared to 1/26 (P = 0.02). Baseline pulse oximetry was normal in both groups but slightly lower in patients with abnormal 6MWT (P = 0.02). No patient presented exercise-induced desaturation. In multivariate analysis, the only factor associated with abnormal 6MWT was the presence of SI (P = 0.045). In our cohort of 46 patients characterized by high exposure rate to HU and by the absence of severe cardiopulmonary disease, the sole factor independently associated with 6MWT was the presence of SI. The lower exercise capacity of children with SI may reflect some subclinical neurological impairment as they do not differ by hemoglobin level or cardiopulmonary parameters.
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影响因子：
168.9
作者：
Setty, BNY;Stuart, MJ;Allen, JL
通讯作者：
Allen, JL
影响因子：
20.3
作者：
Kato, GJ;McGcwan, V;Gladwin, MT
通讯作者：
Gladwin, MT
影响因子：
6.5
作者：
Desai, Payal C.;May, Ryan C.;Ataga, Kenneth I.
通讯作者：
Ataga, Kenneth I.
影响因子：
6.5
作者：
Quinn, CT;Ahmad, N
通讯作者：
Ahmad, N
DOI：
10.3324/haematol.13812
发表时间：
2009-03-01
期刊：
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
影响因子：
--
作者：
Minniti, Caterina R.;Sable, Craig;Gordeuk, Victor R.
通讯作者：
Gordeuk, Victor R.