Markers of Severe Vaso-Occlusive Painful Episode Frequency in Children and Adolescents with Sickle Cell Anemia
Markers of Severe Vaso-Occlusive Painful Episode Frequency in Children and Adolescents with Sickle Cell Anemia
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DOI：
10.1016/j.jpeds.2011.07.018
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发表时间：
2012-02-01
影响因子：
5.1
通讯作者：
Gordeuk, Victor
中科院分区：
文献类型：
--
作者：
Darbari, Deepika S.;Onyekwere, Onyinye;Gordeuk, Victor
目的：在一项镰状细胞病肺动脉高压和缺氧反应的前瞻性研究中，研究当代儿童镰状细胞性贫血（SCA）患者中频繁出现严重血管闭塞性疼痛危像的相关因素。研究设计将前一年有>= 3次严重疼痛发作需要医疗保健的SCA患儿的临床和实验室特征与前一年有= 3次严重疼痛发作的患儿进行比较，其中232例（61%）没有发生过严重疼痛发作。频繁疼痛发作与年龄较大（OR, 1.2; 95% CI, 1.1-1.3; P < 0.0001）、α -地中海贫血特征（OR 3.5; 1.6-6.7; P = 0.002）、较高的中位血红蛋白（OR 1.7; 95% CI: 1.2-2.4; P < 0.003）和较低的乳酸脱氢酶浓度（OR 1.82; 95% CI: 1.07-3.11; P = 0.027）相关。高疼痛频率患儿的铁负荷也增加（血清铁蛋白480 vs 198 μ g/L， P = 0.006），三尖瓣反流喷射速度中位数较高（2.41 vs 2.31 m/s, P = 0.001）。羟基脲的使用和胎儿血红蛋白水平均无明显差异。结论：在我们的SCA患儿队列中，年龄的增加与严重疼痛发作的频率增加有关，如地中海贫血、铁超载、高血红蛋白和低乳酸脱氢酶浓度以及高三尖瓣反流速度。[J] .中华儿科杂志；2012;32(3):391 - 391。
Objective To identify factors associated with frequent severe vaso-occlusive pain crises in a contemporary pediatric cohort of patients with sickle cell anemia (SCA) enrolled in a prospective study of pulmonary hypertension and the hypoxic response in sickle cell disease.Study design Clinical and laboratory characteristics of children with SCA who had >= 3 severe pain crises requiring health care in the preceding year were compared with those of subjects with = 3 severe pain episodes in the preceding year, and 232 (61%) had none. Frequent pain episodes were associated with older age (OR, 1.2; 95% CI, 1.1-1.3; P < .0001), alpha-thalassemia trait (OR 3.5; 1.6-6.7; P = .002), higher median hemoglobin (OR 1.7; 95% CI: 1.2-2.4; P < .003), and lower lactate dehydrogenase concentration (OR 1.82; 95% CI: 1.07-3.11; P = .027). Children with high pain frequency also had an increased iron burden (serum ferritin, 480 vs 198 mu g/L; P = .006) and higher median tricuspid regurgitation jet velocity (2.41 vs 2.31 m/s; P = .001). Neither hydroxyurea use nor fetal hemoglobin levels were significantly different according to severe pain history.Conclusions In our cohort of children with SCA, increasing age was associated with higher frequency of severe pain episodes as were a-thalassemia, iron overload, higher hemoglobin and lower lactate dehydrogenase concentration, and higher tricuspid regurgitation velocity. (J Pediatr 2012; 160: 286-90).