Chronic Kidney Disease in Children (CKiD)

这项申请是由NAPRTCS的26个中心共同努力的，题为“慢性肾功能不全 (CRI)在NAPRTCS患者。我们将招募300名儿童(1-16岁)进行测量肾小球滤过 速率(GFR)为25-75毫升/分钟/1.73平方米。随访每年一次的GFR和6个月的体检 以及血红蛋白、电解质、血清、白蛋白、血清钙、血清磷、 甲状旁腺激素和尿液指标将检验第一个假设，即这个队列最准确 确定CRI进展的比率和风险因素，并且这一进展将是相关的 有蛋白尿、白蛋白、血压、营养状况、生长发育和甲状旁腺功能亢进症。为了测试 第二个假设是轻度CRI儿童会发生心血管疾病，其 随着CRI的进展，病情加重，我们将执行基线 和年度24小时动态血压监测，超声心动图评估 测定左室壁和左心功能，并对颈动脉进行B超检查 测定颈动脉内中膜厚度和顺应性。来检验第三个假设，即神经认知 CRI儿童的预后受肾功能不全进展的影响，一系列 验证的神经认知测试将在研究开始时以及在6、12和24个月时进行，以评估 大脑功能的许多皮质和皮质下区域。来检验第四个假设：慢性 炎症会导致恶病质、生长激素抵抗和生长迟缓，我们将 检查循环细胞因子和神经肽浓度对膳食摄入量、营养成分的影响 和生长参数以及生长激素轴的扰动和对生长的反应 激素疗法。最后，为了检验第五个假设，即骨组织学之间存在相关性， 血清甲状旁腺素浓度和测定的GFR，我们将测量和表征生化和 肾性骨营养不良的组织学特征及血清甲状旁腺素浓度的测定 与正常的骨形成率相关。

This application, a joint effort of 26 centers of the NAPRTCS, is entitled "Chronic Renal Insufficiency (CRI) in NAPRTCS Patients. We will enroll 300 children (1-16 years) with measured glomerular filtration rates (GFR) of 25-75 mL/min/1.73m2. Follow-up with annual GFR and 6 month physical examinations and determinations of hemoglobin, electrolytes, serum, albumin, serum calcium, serum phosphorus, parathyroid hormone and urinary indices will test the 1st hypothesis that this cohort will most accurately define the rate of and the risk factors for progression of CRI, and that this progression will be correlated with proteinuria, albumin, blood pressure, nutritional status, growth and hyperparathyroidism. To test the 2nd hypothesis that cardiovascular disease develops in children with mild CRI and that its orevalence and severity increase in association with the progression of CRI, we will perform baseline and annual 24-hour ambulatory blood pressure monitoring, echocardiographic assessments to determine left-ventricularmass and LV function, and B-mode ultrasound of the carotid artery to determine the IMT and carotid artery compliance. To test the 3rd hypothesis that the neurocognitive outcome of children with CRI is influenced by the progression of renal insufficiency, a battery of validated neurocognitive tests will be conducted at study entry and at 6, 12 and 24 months to assess many cortical and subcortical areas of brain function. To test the 4th hypothesis that chronic inflammation contributes to cachexia, growth hormone resistance and growth retardation, we will examine the impact of circulating cytokine and neuropeptide concentrations on dietary intake, nutritional and growth parameters as well as growth hormone axis pertubations and responsiveness to growth hormone therapy. Finally, to test the 5th hypothesis that a correlation exist between bone histology, serum concentration of PTH and measured GFR, we will measure and characterize the biochemical and histologic features of renal osteodystrophy and determine the serum concentrations of PTH that are associated with normal rates of bone formation.