Prognostic factors in children with PRES and hematologic diseases

Prognostic factors in children with PRES and hematologic diseases
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DOI:
10.1111/ane.12570
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发表时间:
2016-12-01
影响因子:
3.5
通讯作者:
Verrotti, A.
Verrotti, A.
中科院分区:
医学3区
文献类型:
--
作者:
Tambasco, N.;Mastrodicasa, E.;Verrotti, A.

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后部可逆性脑病综合征(PRES)是一种临床和放射学特征,以局灶性神经症状、头痛、意识不清和癫痫发作为特征,与神经影像学可检测到的脑后部一过性病变相关。在儿童中,导致PRES最常见的原因之一是癌症。材料和方法在这篇综述中,我们报告了5例因血液病而进行干细胞移植后发生PRES的儿童病例,并回顾了所有英文文献报道的病例,以调查结果和相关危险因素。结果共报告111例。高血压非常常见(80%)。临床特征包括癫痫发作(80.1%)、头痛(44.1%)、视力障碍(26.1%)和精神改变(48.6%)。32例患者中27例脑电图异常。MRI显示所有患者的特征性病变,甚至在早期阶段。晚期MRI异常表现与神经系统后遗症有关。死亡19例(17.1%),其中2例为PRES,存活患者中17例有神经系统后遗症。报告了4例PRES复发病例。结论所有符合PRES症状的移植受者应及时识别,避免长期并发症甚至死亡。
ObjectivesPosterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by focal neurological signs, headache, confusion, and seizure, associated with transitory lesions in the posterior areas of the brain detectable with neuroimaging. Among children, one of the most common causes of PRES is cancer.Materials and MethodsIn this review, we present the cases of 5 children developing PRES after stem cell transplantation for hematological disease and review all the cases reported in English literature to investigate outcomes and associated risk factors.ResultsOne hundred and eleven cases were reported. Hypertension was very frequent (80%). Clinical features included seizures (80.1%), headache (44.1%), visual disturbance (26.1%), and mental change (48.6%). EEG was abnormal in 27 of 32 patients. MRI revealed characteristic lesions in all patients even in early stages. Abnormal MRI findings in late stages were associated with neurological sequelae. Nineteen patients died (17.1%) of which 2 of PRES. Among alive patients, 17 had neurological sequelae. Four cases of PRES relapse were described.ConclusionsThus, all transplant recipients with symptoms consistent with PRES should be promptly recognized to avoid long-term complications or even death.