Which Patients should be Reported to the Registry?
The ECFS Patient Registry is a longitudinal study. It recruits all the prevalent cases on 1st January 2008 and all the subsequent incident cases.
![](https://www.ecfs.eu/sites/default/files/general-content-images/working-groups/ecfs-patient-registry/Study_graphic1.jpg?fid=991)
What are the inclusion criteria?
Only patients who fulfil the diagnostic criteria below should be included in the registry:
a. Two sweat tests >60 mmol/L chloride.
b. One sweat test >60 mmol/L chloride AND DNA Analysis/Genotyping - two identified disease causing CF mutations.
If the sweat value is less than or equal to 60 mmol/L, then at least 2 of these should be fulfilled:
a. DNA Analysis/Genotyping - two identified desease causing CF mutations.
b. Transepithelial (Nasal) Potential Difference - study consistent with a diagnosis of CF.
c. Clinical Presentation - typical features of CF.
Diagnosis reversal: if the patient's CF diagnosis reversed during the year, identify the reason from the options listed:
i. DNA Analysis - unable to identify two disease causing CF mutations.
ii. Transepithelial (Nasal) Potential Difference - study not consistent with a diagnosis of CF.
iii. Repeat normal sweat testing - confirm with clinical team.
In case of certain diagnosis reversal you should inform the registry in order to have this patient's records deleted from the registry.
References:
Gibson LE, Cook RE. A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics 1959;23:545-49
Farrell PM, Koscik RE. Sweat chloride concentrations in infants homozygous or heterozygous for F508del cystic fibrosis. Pediatrics 1996;97:524-8
De Boeck et al. Cystic fibrosis: terminology and diagnostic algorithms. Thorax 2006; 61(7):627-35.