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- W3153894827 abstract "Introduction. Management of patients with maple syrup urine disease(MSUD) includes a low protein diet, supplemented with special formulasand constant monitoring of branched chain amino acids (BCAA). Thegold standard for monitoring BCAA is plasma amino acid analysis usingHigh Performance Liquid Chromatography (HPLC). In a developingcountry like the Philippines, however, the cost of this test is prohibitiveto the majority of the patients. In our center, dried blood spot leucine/isoleucine (leu/ile) levels analysed by thin layer chromatography (TLC)is often used to diagnose and monitor these patients.Objective. This study was done to determine the correlation of leu/ilelevels using the two methods (TLC and HPLC).Methods. A total of 46 MSUD patients were referred to the BiochemicalGenetics Laboratory of the Institute of Human Genetics (IHG) from July2001 to January 2004. Thirty five samples were obtained from 18 ofthese patients [some patients were seen at IHG more than once], andpaired determinations of plasma amino acid using TLC and HPLC weremade. The remaining samples were either hemolyzed or were notanalyzed. The correlation coefficient [rho denoted as “ρ”] was estimatedat a 95% confidence level using the Fisher’s Z transformation.Results and Conclusion. Of the 18 patients, 12 were males. The youngestwas 1 day old and the oldest was 5 years old. The majority had theclassical type of MSUD and dietary protein was restricted to between0.6 gram/kg/day to 1 gram/kg/day of natural protein. Using the firstpairs of observation for these 18 patients, the correlation coefficientwas 0.76 {95% CI:0.462 to 0.907). This suggests a strong correlationbetween the two methods. It is recommended that further studies bedone to determine the potential of the dried blood spot leu/ile levelby TLC as an alternative method that can be used in the diagnosis andmonitoring of MSUD patients especially in a developing country." @default.
- W3153894827 created "2021-04-26" @default.
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- W3153894827 date "2008-12-03" @default.
- W3153894827 modified "2023-09-23" @default.
- W3153894827 title "Correlation between Dried Blood Spot Thin Layer Chromatography and Plasma High Performance Liquid Chromatography of Leucine/Isoleucine Levels Among Filipino Patients with Maple Syrup Urine Disease (MSUD) seen at the Institute of Human Genetics" @default.
- W3153894827 doi "https://doi.org/10.47895/amp.v42i2.2413" @default.
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