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Quantitative Determination of Phenylketonuria

Quantitative Determination of Phenylketonuria
CE-IVD certified and suitable for any kind of LC-MS/MS instrument

 

The Zivak Phenylketonuria (PKU) LC-MS/MS analysis kit can be used in combination with the Zivak-Multitasker as well as manually. Analysis run time for the manual version is 2 minutes. Due to the efficient and quite smart procedure of the Zivak-Multitasker, the automated version guarantees to receive quantitative results in just 2,5 minutes per test, including sample preparation-, sample injection- and analysis run time. Additionally, high accuracy is being rectified through external quality tests.

 

LC-MS/MS analysis kit for the in-vitro diagnostic quantitative determination of phenylketonuria in human serum & dried blood samples. 

Phenylketonuria (PKU) is an autosomal recessive genetic disorder characterized by a deficiency in the enzyme phenylalanine hydroxylase (PAH). This enzyme is necessary to metabolize the aminoacid phenylalanine to the amino acid tyrosine. When PAH is deficient, phenylalanine accumulates on the brain tissue and is converted into phenylpyruvate (also known as phenylketone), which is detected in the urine. PAH is found on chromosome number 12. If left untreated, this condition can cause problems with brain development, leading to progressive mental retardation and seizures. However, PKU can be controlled by a special diet with low in phenylalanine and high in tyrosine can be a very effective treatment. There is no cure. The disorder is only detectable after irreversible damage is done hence early diagnosis is crucial. Most babies in developed countries are screened for PKU soon after birth. PKU is usually detected using the HPLC test, but some clinics still use the Guthrie test, as a part of national biochemical screening programs.

Zivak PKU LC-MS/MS kit was developed for quantitative detection of Phenylketonuria in human serum, plasma and dried blood samples. LC-MS/MS is a effective tool for the study of metabolic disorders (e.g. PKU). LC-MS/MS analysis gives results in 2 minutes with minimal sample preparation. Main methods and procedures that is selected by Zivak are based on EN ISO 14971 .

Quantitative Determination of Phenylketonuria 0

LC-MS/MS parameters : contact for more details

Storage & Stability:

  • This analysis kit can be shipped at room temperature.
  • After arrival, calibrator and controls, Reagent 1, 2, 3, and 4 can be stored at 2-8 °C.
  • MP, WB can be stored at room temperature.
  • Reagent 2, 3 and 4 are volatile, please make sure that the lids of reagents are closed tightly after use.
  • All components are guaranteed until expiry date when stored at recommended temperatures and used as described in these instructions.

Preparation of Calibrators & Controls (Serum): contact for more details

 

Materials Supplied (for serum samples)

Order No.

Volume

Component

ZV-3003-02R1-10

1 x 20 ml

Reagent 1, Contains deuterated internal standards

ZV-3003-02R2-10

1 x 40 ml

Reagent 2, Contains acid

ZV-3003-02R3-10

1 x 5 ml

Reagent 3, Contains derivatisation reagent

ZV-3003-02R4-10

4 x 40 ml

Reagent 4, Contains dilution reagent

ZV-3003-02MP-10

1 x 0,5 L

Mobile Phase, Contains organic solvent

ZV-3003-02WB-10

  1 x 0,5 L

Washing solution, Contains organic solvent

ZV-3003-KK 10

1 x 1 pc

User Guide

 
 
Materials Required But Not Supplied (for serum samples)

Order No.

Volume

Component

ZV-3003-02S1-10

1 x 3 ml

Lyophilized Serum Calibrator Level 1

ZV-3003-02K1-10

1 x 3 ml

Lyophilized Serum Control Level 1

ZV-3003-02K2-10

1 x 3 ml

Lyophilized Serum Control Level 2

ZV-3003-02C1-10

1 pcs

Zivak PKU Serum LC-MS/MS Analytical Column

 
 
Materials Supplied (for dried blood samples)

Order No.

Volume

Component

ZV-3003-02R1-15

1 x 20 ml

Reagent 1, Contains deuterated internal standards

ZV-3003-02R2-15

1 x 40 ml

Reagent 2, Contains acid

ZV-3003-02R3-15

1 x 5 ml

Reagent 3 Contains derivatisation reagent

ZV-3003-02R4-15

4 x 40 ml

Reagent 4, Contains dilution reagent

ZV-3003-02MP-15

1 x 0,5 L

Mobile Phase, Contains organic solvent

ZV-3003-02WB-15

1 x 0,5 L

Washing solution, Contains organic solvent

ZV-3003-KK 10

1 x 1 pc

User Guide

,
 
Materials Required But Not Supplied (for dried blood samples)

Order No.

Volume

Component

ZV-3003-02S1-15

1 x 1 spot

Dried Blood Spot Calibrator Level 1

ZV-3003-02K1-15

1 x 1 spot

Dried Blood Spot Control Level 1

ZV-3003-02K2-15

  1 x 1 spot

Dried Blood Spot Control Level 2

ZV-3003-02C1-15

  1 pcs

Zivak PKU Dried Blood Spots LC-MS/MS Analytical Column

 

Zivak General Catalog

Download Zivak General Catalog PDF