Updated 05 December 2016

Registry Variables and Definitions

The Registry collects demographic and clinical data on all CF patients meeting the ECFS Patient Registry criteria. For a detailed list of variables, their definition and the inclusion criteria, please click here (pdf). The summary of the information collected is given hereunder.

Demographic

  • CF centre code
  • Patient code
  • Year of follow up
  • Date of birth
  • Gender
  • Status of patient
  • Cause of death
  • Date of death

 

Genotype

  • First mutation
  • Second mutation

 

Therapy

  • Inhaled continuous hypertonic NaCI this year
  • Inhaled continuous antibiotic this year
  • Inhaled continuous bronchodilators this year
  • In oxygen therapy this year
  • Use of rhDNase this year
  • Use of continuous Azithromycin (or other macrolide) this year
  • Use of ursodeoxycholic acid this year
  • Use of pancreatic enzymes this year

 

Diagnosis

  • Diagnosis confirmed
  • Age at diagnosis
  • Type of sweat test
  • Electrolytes
  • Chloride value
  • Meconium Ileus
  • Neonatal screening

 

Follow Up

  • Date of best FEV1 registered this year
  • Value of best FEV1 registered this year
  • Value of best FVC registered this year
  • Height measured at date of best FEV1 or, if no lung function test, last height of the year of follow-up
  • Weight measured at date of best FEV1
  • Weight measured at date of best FEV1 or, if no lung function test, last weight of the year of follow-up


Complications

  • Allergic broncho-pulmonary aspergillosis this year
  • Diabetes: daily insulin treated this year
  • Pneumotheroax requiring chest drain this year
  • Liver disease this year
  • Hemoptysis major over 250 ml this year
  • Pancreatic status: faecal elastase
  • Pancreatic status: faecal fat
  • Occurrence of malignancy this year

 

Microbiology

  • Chronic Burkholderia cepacia complex
  • Nontuberculous mycobacteria this year
  • Chronic Pseudomonas aeruginosa
  • Chronic Staphylococcus aureus
  • Stenotrophomonas maltophilia this year

 

Transplant

  • Liver transplant
  • Year of latest liver transplant (if occurred before or during this year)
  • Lung transplant
  • Year of latest lung transplant (if occurred before or during this year)