Hartmann Hans, Hawellek Nicola, Wedekin Mirja, Vogel Charlotte, Das Anibh M, Balonwu Katharina, Ehrich Jochen H H, Haffner Dieter, Pape Lars
Clinic for Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany.
Transpl Int. 2015 Apr;28(4):429-36. doi: 10.1111/tri.12510. Epub 2015 Jan 13.
Renal replacement therapy has become available for the majority of patients suffering from severe congenital chronic kidney disease (CKD). Data on the long-term neurocognitive outcome and the impact of early kidney transplantation (KTx) in this setting is unclear. Neurocognitive outcomes in 15 patients (11 male) with isolated congenital CKD (stage 3-5) requiring KTx at a mean age of 2.8 ± 1.3 were assessed at a mean age of 8.3 ± 1.4 years. Patients underwent neurological examination and testing for neuromotor and neurocognitive function using three independent tests. Pre-emptive KTx was performed in six patients, and nine patients were dialyzed prior to KTx for a mean period of 11.1 ± 8.6 months. Neuromotor function was abnormal in 8/15 patients. HAWIK-III showed a global intelligence quotient (IQ) of 93.5 ± 11.4 (P = 0.05) due to a significantly reduced performance IQ of 89.1 ± 11.3 (P < 0.01). In three patients, the global IQ was clinically significantly reduced by >1 SD to <85. In patients with neuromotor dysfunction, performance IQ was lower than in patients with normal neuromotor function (83.8 ± 10.2 vs. 96.2 ± 9.0, P = 0.04). Time on dialysis was inversely correlated to verbal IQ (r = 0.78, P = 0.02). Pre-emptive KTx and duration of dialysis treatment <3 months was associated with superior neurocognitive outcome. Early (pre-emptive) KTx results in superior long-term neurocognitive outcome in children with severe congenital CKD.
对于大多数患有严重先天性慢性肾脏病（CKD）的患者来说，肾脏替代治疗已成为可行的选择。在此背景下，关于长期神经认知结局以及早期肾移植（KTx）影响的数据尚不清楚。对15例（11例男性）孤立性先天性CKD（3 - 5期）且平均年龄为2.8±1.3岁需要进行KTx的患者，在平均年龄8.3±1.4岁时评估其神经认知结局。患者接受了神经系统检查，并使用三项独立测试对神经运动和神经认知功能进行检测。6例患者接受了抢先性KTx，9例患者在KTx前接受了平均11.1±8.6个月的透析。15例患者中有8例神经运动功能异常。由于操作智商显著降低至89.1±11.3（P < 0.01），HAWIK - III显示总体智商（IQ）为93.5±11.4（P = 0.05）。3例患者的总体智商在临床上显著降低>1个标准差至<85。在神经运动功能障碍患者中，操作智商低于神经运动功能正常的患者（83.8±10.2 vs. 96.2±9.0，P = 0.04）。透析时间与言语智商呈负相关（r = 0.78，P = 0.02）。抢先性KTx和透析治疗时间<3个月与较好的神经认知结局相关。早期（抢先性）KTx可使患有严重先天性CKD的儿童获得较好的长期神经认知结局。
