This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor 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.
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