NON-INVASIVE BLOOD PHENYLALANINE MONITOR

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 苯丙酮尿症是一种罕见的代谢紊乱，其特征是血液中苯丙氨酸浓度升高。这会导致脑损伤、智力低下和神经功能障碍。限制苯丙氨酸饮食可以预防脑损伤。苯丙氨酸的血液水平必须维持在一个较窄的范围内，需要经常监测。这项提议的目的是开发一种非侵入性的苯丙氨酸测量系统。我们将比较用类似于葡萄糖手表的装置收集的间质性液体中的苯丙氨酸浓度和血浆苯丙氨酸水平。假设这两项测量将具有良好的相关性，可以开发一种PKU-Watch，以允许对苯丙酮尿症患者的苯丙氨酸水平进行准确和频繁的监测。 本研究的目的是研究一种新的设备，能够非侵入性地测量苯丙酮尿症患者的苯丙氨酸水平。一种类似于血糖手表的特殊装置将被应用于患者的皮肤上，以收集间质液体。将采集的液体中的苯丙氨酸进行测量。还将测定血液中苯丙氨酸的浓度，以确定间质苯丙氨酸如何与血液水平相关。为了进行比较，将同时测量血糖水平。如果成功，该项目将极大地改善苯丙酮尿症患者的饮食依从性，改善他们的结局。在分析过程中将完全利用血液样本。没有考虑过保存标本。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Phenylketonuria is a rare metabolic disorder characterized by increased concentrations of phenylalanine in blood. This leads to brain damage, mental retardation, and neurological dysfunction. Treatment with a phenylalanine restricted diet can prevent brain damage. Phenylalanine blood levels must be maintained in a narrow range and require frequent monitoring. The objective of this proposal is to develop a non-invasive system to measure phenylalanine. We will compare phenylalanine concentration in interstitial fluid collected with a device similar to a Gluco-watch to plasma phenylalanine levels. The hypothesis is that the two measurements will have a good correlation and that a PKU-watch can be developed to allow precise and frequent monitoring of phenylalanine levels in patients with phenylketonuria. The objective of this research is to study a new device capable of non-invasively measuring phenylalanine levels in patients with phenylketonuria. A special device, similar to a gluco-watch, will be applied to the patients' skin to collect interstitial fluid. Phenylalanine will be measured in the fluid collected. The concentration of phenylalanine will be also determined in blood to determine how interstitial phenylalanine correlates with blood levels. For comparison, glucose levels will be measured simultaneously. If successful this project will greatly improve dietary compliance in patients with phenylketonuria, improving their outcome. Blood samples will be completely utilized during the analysis. No banking of specimen is contemplated.