Batinić Danica, Milošević Danko, Filipović-Grčić Boris, Topalović-Grković Marija, Barišić Nina, Turudić Daniel
University Zagreb School of Medicine, Department of Pediatrics, University Hospital Center Zagreb, Kišpatićeva 12, 10000, Zagreb, Croatia.
University Zagreb School of Medicine, Department of Anesthesiology and Intensive Care, University Hospital Center Zagreb, Zagreb, Croatia.
Wien Klin Wochenschr. 2017 Aug;129(15-16):579-582. doi: 10.1007/s00508-017-1221-z. Epub 2017 Jun 9.
Cyclosporine A-associated neurotoxicity has been reported mainly after organ transplantation. Only a small number of children with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity have been reported.
We report three children, aged 4, 11, and 15, with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity. In two of the patients, primary diagnosis was idiopathic nephrotic syndrome, and in one it was IgA nephropathy. Magnetic resonance with diffusion-weighted imaging, combined with quantification of apparent diffusion coefficient values, showed lesions caused by cytotoxic edema indicating irreversible brain damage. Nonetheless, the patients fully recovered clinically and radiologically after prompt discontinuation of cyclosporine A.
Neurotoxic effects should be suspected in any child with nephrotic syndrome treated with cyclosporine A in whom sudden neurological symptoms occur. Cytotoxic edema is a rare finding in pediatric patients. However, even in such cases with seemingly irreversible brain damage, full recovery without permanent neurological sequels is possible with prompt cyclosporine A discontinuation and supportive therapy.
环孢素A相关神经毒性主要在器官移植后被报道。仅有少数患有类固醇抵抗型肾病综合征且出现环孢素A相关神经毒性的儿童病例被报道。
我们报告了三名年龄分别为4岁、11岁和15岁的患有类固醇抵抗型肾病综合征且出现环孢素A相关神经毒性的儿童。其中两名患者的初步诊断为特发性肾病综合征，一名为IgA肾病。磁共振扩散加权成像结合表观扩散系数值定量分析显示，细胞毒性水肿导致的病变提示存在不可逆的脑损伤。尽管如此，在迅速停用环孢素A后，这些患者在临床和影像学上均完全康复。
对于任何接受环孢素A治疗的肾病综合征患儿，若突然出现神经症状，均应怀疑存在神经毒性作用。细胞毒性水肿在儿科患者中是一种罕见的表现。然而，即使在这些看似存在不可逆脑损伤的病例中，通过迅速停用环孢素A并给予支持治疗，仍有可能实现完全康复且不遗留永久性神经后遗症。
