Ho Doreen T, Rodig Nancy M, Kim Heung B, Lidov Hart G W, Shapiro Frederic D, Raju G Praveen, Kang Peter B
Department of Neurology, Lahey Clinic Medical Center, Burlington, MA, USA.
Pediatr Transplant. 2012 Nov;16(7):E296-300. doi: 10.1111/j.1399-3046.2011.01630.x. Epub 2011 Dec 21.
Clinical and pathologic studies on adults with uremic neuropathy are numerous, but less is known about this disorder in children and adolescents. We report the clinical, electrophysiologic, and pathologic findings in an adolescent female with uremic neuropathy. Electrophysiologic findings were consistent with a primarily axonal sensorimotor polyneuropathy. Sural nerve biopsy revealed areas of focal depletion in myelin sheaths and loss of axons. Axonal degeneration with secondary myelin changes appears to be the characteristic pathology in this case, one of the youngest to our knowledge for which nerve biopsy data are available. Our patient experienced dramatic recovery after renal transplantation, similar to the reports of older patients.
关于成年尿毒症神经病变患者的临床和病理研究众多,但对于儿童和青少年的这种疾病了解较少。我们报告了一名患有尿毒症神经病变的青春期女性的临床、电生理和病理检查结果。电生理检查结果与主要为轴索性感觉运动性多发性神经病变一致。腓肠神经活检显示髓鞘有局灶性脱失区域以及轴突丧失。轴突变性伴继发性髓鞘改变似乎是该病例的特征性病理表现,据我们所知,这是有神经活检数据的最年轻病例之一。我们的患者在肾移植后经历了显著恢复,这与老年患者的报道相似。