NON-INVASIVE BLOOD PHENYLALANINE MONITOR

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。苯丙酮尿症是一种罕见的代谢性疾病，其特征是血液中苯丙氨酸浓度增高。这会导致脑损伤、智力迟钝和神经功能障碍。用苯丙氨酸限制饮食治疗可以防止脑损伤。苯丙氨酸血液水平必须维持在一个狭窄的范围内，需要经常监测。本提案的目的是开发一种非侵入性系统来测量苯丙氨酸。我们将比较用类似于血糖表的装置收集的间质液中的苯丙氨酸浓度与血浆苯丙氨酸水平。假设这两种测量将具有良好的相关性，并且可以开发一种pku手表来精确和频繁地监测苯丙酮尿症患者的苯丙氨酸水平。本研究的目的是研究一种能够无创测量苯丙酮尿患者苯丙氨酸水平的新设备。一种类似于血糖表的特殊装置将被应用于患者的皮肤以收集间质液。将在收集的液体中测量苯丙氨酸。还将测定血液中苯丙氨酸的浓度，以确定间质苯丙氨酸与血液水平的关系。为了比较，葡萄糖水平将同时测量。如果成功，该项目将大大提高苯丙酮尿患者的饮食依从性，改善其预后。血液样本将在分析过程中被完全利用。不考虑标本的储存。这是一个试点项目，只有15名患者，为期一年。

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 lead 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. This is a pilot project with only 15 patients for a one year time frame.