Introduction to Cystic Fibrosis
Cystic fibrosis is a genetic disorder that causes abnormal changes in the exocrine glands, causing the accumulation of thick mucus in the lungs and digestive system, which can have adverse effects on the lungs and intestines.
Cause: Cystic fibrosis is a genetic disease caused by a recessive mutation of a gene on chromosome 7 that encodes a membrane-bound protein called CFTR that functions as a chloride ion transport channel across the membrane. Inactivation of CFTR results in chloride ions being retained in the cell, thereby disrupting the movement of water into the epithelium (which is necessary for the production of thin, free-flowing mucus) resulting in an abnormally thick and thick mucus layer.
Incidence: Cystic fibrosis (CF) is a common genetic disease, with an incidence of: 1/3,000 (in white children), 1/9,000 (in Hispanic children), 1/11,000 (in Native Americans), 1/15,000 (in African Americans), 1/30,000 (in Asian Americans).
Semi-automatic solution
- Product name: DELFIA® Neonatal IRT kit
- Product code: A005-210 / A005-204
- Manufacturer: Wallac Oy – Finland
- Packing: 960 / 384 tests
- Test principle:
The DELFIA Neonatal IRT test is a solid-phase, double-sided time-release immunofluorescence assay based on the direct sandwich technique, in which two monoclonal antibodies (derived from mice) are directed against two distinct antigenic markers on the IRT molecule. Controls, calibrators and test samples containing IRT react simultaneously with monoclonal antibodies immobilized at the specific antigenic site on the IRT and europium-labeled monoclonal antibodies (at the other antigenic site) in a buffer solution. The buffer solution elutes the IRT from dried blood drops on blotting paper discs. The complete test requires only one incubation step. The Enhancement Solution separates europium ions from the labeled antibody and forms highly fluorescent ligands with the components in the Enhancement Solution. The fluorescence signal in each well is then measured again. The fluorescence signal in each sample is proportional to the concentration of IRT in that sample.
Application: The kit is used to quantify IRT (immunoreactive trypsin(ogen) – immunoreactive trypsin and trysinogen) in dried blood samples on dried blood blotting paper of newborns from 2 to 4 days old on the VICTORTM 2D fluorescence meter for screening of cystic fibrosis in newborns. Suitable for hospitals with small and medium sample volumes.
Other equipment required for the test:
- VICTORTM 2D Fluorometer
- Wallac DBS Puncher or Panthera-Puncher™ 9 Dry Blood Puncher
- DELFIA® Plateshake
- DELFIA Trio Plate Washer
Automated Solution
- Product Name: GSP® Neonatal IRT kit
- Product Code: 3306-0010
- Manufacturer: Wallac Oy – Finland
- Packing: 1152 tests
- Test Principle:
The GSP Neonatal IRT test is a two-site, solid-phase fluorescence test based on a direct clamping technique in which two monoclonal antibodies (derived from mice) are directly bound to two distinct antigenic determinants on the IRT molecule. The calibrator, control or test sample containing IRT is reacted simultaneously with a monoclonal antibody at a specific antigenic site on the IRT molecule and a europium-labeled monoclonal antibody (at a different antigenic site) in assay buffer. The assay buffer elutes the IRT from the dried blood on the blotting paper disc. The complete assay requires only one incubation step. The DELFIA Enhancement Solution dissociates the europium ions from the labeled antibody into a solution where they form highly fluorescent complexes with the components of the DELFIA Enhancement Solution. The fluorescence in each well is then measured. The fluorescence of each sample is proportional to the concentration of IRT in the sample.
Application: The kit is used to quantitatively determine trypsin(ogen) (IRT) on dried blood spots of 2- to 4-day-old newborns on the GSP® system for screening for cystic fibrosis in newborns. Suitable for hospitals and testing centers with medium and large sample volumes.
Other equipment required for testing:
- GSP® System
- Wallac DBS Puncher or Panthera-Puncher™ 9
