JBCGenetics |

Original Article

Volume: 1 | Issue: 1 | Published: Jan 01, 2018 | Pages: 26 - 30 | DOI: 10.24911/JBCGenetics/183-1530358447

Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia

Authors: Abdul Rafiq Khan , Ali Al-Othaim , Ahmed Al-Fares , Najla Al Hussain

Authors

Abdul Rafiq Khan

National Guard Health Affairs, Department of Pathology & Laboratory Medicine, Biochemical Metabolic Laboratory, Riyadh, Kingdom of Saudi Arabia

Ali Al-Othaim

National Guard Health Affairs, Department of Pathology & Laboratory Medicine, Biochemical Metabolic Laboratory, Riyadh, Kingdom of Saudi Arabia

Ahmed Al-Fares

National Guard Health Affairs, Department of Pathology & Laboratory Medicine, Biochemical Metabolic Laboratory, Riyadh, Kingdom of Saudi Arabia

Najla Al Hussain

National Guard Health Affairs, Department of Pathology & Laboratory Medicine, Biochemical Metabolic Laboratory, Riyadh, Kingdom of Saudi Arabia.

Publication History

Received: October 30, 2017

Accepted: December 15, 2017

Published: January 01, 2018

Abstract

Background: Measurement of branched-chain amino acids (BCAAs) [valine (Val), alloisoleucine (allo-Ile), isoleucine (Ile), and leucine (Leu)] in plasma and dry blood spot samples is important for the diagnosis and monitoring of maple syrup urine disease (MSUD), which is a metabolic disorder that affects metabolism of BCAAs. We used a temporally short program on an High performance liquid chromatography (HPLC) (Biochrom 30) for the first time in Saudi Arabia to analyze plasma samples for the diagnosis of MSUD. Methodology: 500 μl of plasma were mixed with 500 μl of 10% sulphosalicylic acid, followed by shaking for 5-10 seconds on a vortexer. The mixture was incubated at 4 C for 10 minutes followed by centrifugation at 10,000 rpm for 5 minutes. Supernatants were transferred into HPLC vials for injection. Results: For all amino acids, the limit of detection and limit of quantification were 5.0 and 10.0 μmol/l, respectively. The method was linear in the range of 10-2,500 μmol/l. The method was specific and selective for the detection of BCAAs, including alloisoleucine. The correlation coefficient (CV) of method comparison was greater than 0.980 and percent recovery of all amino acids in plasma was 80%-100%, which was evaluated at two amino acid concentrations. Precision was assessed by repeat analysis of spiked plasma samples and percent CV was found to be

Keywords: MSUD HPLC analysis

Pubmed Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain. Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia. JBC Genetics. 2018; 01 (January 2018): 26-30. doi:10.24911/JBCGenetics/183-1530358447

Web Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain. Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia. https://www.jbcgenetics.com/index.php/articles/2015 [Access: April 27, 2025]. doi:10.24911/JBCGenetics/183-1530358447

AMA (American Medical Association) Style

Vancouver/ICMJE Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain. Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia. JBC Genetics. (2018), [cited April 27, 2025]; 01 (January 2018): 26-30. doi:10.24911/JBCGenetics/183-1530358447

Harvard Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain (2018) Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia. JBC Genetics, 01 (January 2018): 26-30. doi:10.24911/JBCGenetics/183-1530358447

Chicago Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain. "Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia." 01 (2018), 26-30. doi:10.24911/JBCGenetics/183-1530358447

MLA (The Modern Language Association) Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain. "Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia." 01.January 2018 (2018), 26-30. Print. doi:10.24911/JBCGenetics/183-1530358447

APA (American Psychological Association) Style

Abdul Rafiq Khan, Ali Al-Othaim, Ahmed Al-Fares, Najla Al Hussain (2018) Use of HPLC-UV method for the analysis of maple syrup urine disease in plasma sample first time in Saudi Arabia. , 01 (January 2018), 26-30. doi:10.24911/JBCGenetics/183-1530358447