Cystic Fibrosis (CF)

Science and Research

The Cystic Fibrosis (CF) core dataset represents the most important lung research parameters for the disease area.

Kategorie	Parameter	Value Set	Description
Biometric Data	Gender	Diverse Female Male
Biometric Data	Age at visit
Biometric Data	Weight
Biometric Data	Height
Biometric Data	BMI
Smoking History	Smoking Status	Ex Smoker Passive Smoker Active Smoker Never Smoker
Smoking History	Amount	Packyears Zigaretten/Tag
Diagnosis	Cystic Fibrosis (CF)
Diagnosis	PI-CF (Pancreas insufficient)	PI-CF > 80% FEV1 pred. PI-CF < 40% FEV1 pred. PI-CF, 40-60% FEV1 pred. PI-CF, 60-80% FEV1 pred.
Diagnosis	Cystic fibrosis-related disorder		CFTR-RDs refer to a group of diseases associated with CF mutations; do not meet diagnostic criteria for CF
Diagnosis	PS-CF (Pancreas sufficient)	PS-CF > 80% FEV1 pred. PS-CF < 40% FEV1 pred. PS-CF, 40-60% FEV1 pred. PS-CF, 60-80% FEV1 pred.
Diagnosis	Molecular Alteration	?F508 R347P R553X G551D 1717 1G?A G542X 3905insT 2789 +5G?A N1303K
Comorbidities	Comorbidities
Comorbidities	Pseudomonas		Pseudomonas is a clinically notable and aggressive pathogen, often causing nosocomial infections.
Therapy	Lung transplantation
Therapy	Liver transplantation
Therapy	Oxygen longterm therapy
Therapy	CFTR-Modulator-Therapy		These therapies are designed to correct the malfunctioning protein made by the CFTR gene
Lung Function	sRaw		Specific Airway Resistance
Lung Function	sRaw%		Specific airway resistance as percentage of predicted value (sRaw%)
Lung Function	TLC		Total Lung Capacity: the volume of air in the lungs at maximum inflation
Lung Function	TLC%		Total Lung Capacity as percentage of the predicted value
Lung Function	Rtot%		Total airway resistance as percentage of predicted value
Lung Function	Rtot		Total airway resistance
Lung Function	RV		Residual Volume: the amount of air remaining in the lungs after maximum exhalation
Lung Function	RV%		Residual Volume as percentage of predicted value
Lung Function	FVC		Forced Vital Capacity: the volume of air that can forcibly be blown out after full inspiration
Lung Function	Vcmax		The largest volume a patient can exhale after a full inhalation
Lung Function	FEV1		Forced Expiratory Volume in 1 second: the volume of air that has been exhaled at the end of the first second of forced expiration
Lung Function	Multiple Breath Washout	LCI	Lung Clearance Index (LCI) measured by multiple breath washout is a sensititve measure of ventilation inhomogeneity
Biobank Samples	Blood		The availability of blood samples for a certain patient
Biobank Samples	Bronchoalveolar Lavage Fluid		The availability of Bronchoalveolar Lavage Fluid samples for a certain patient
Biobank Samples	Nose swab (NS)		The availability of nose swab samples for a certain patient
Biobank Samples	Sputum (SP)		The availability of sputum samples for a certain patient
Laboratory values	IgG		A protein produced by plasma cells and lymphocytes and characteristics of these types of cells
Laboratory values	Total IgE		Total IgE plays an important role in causing allergic reactions.
Laboratory values	PsA Alkaline Protease Antibody		P. aeruginosa alkaline protease is produced during keratitis, otitis media, CF, and bacteraemia.
Laboratory values	PsA Elastase Antibody		Elastase is a major virulence factor in P. aeruginosa; it cause tissue damage during infection in the human host.
Laboratory values	PsA Exotoxin A Antibody		Exotoxin A is the most toxic of the numerous extracellular proteins produced by P. aeruginosa.
Laboratory values	Oral glucose tolerance test (oGTT)		oGGT are used to measure how well the body can process a larger amount of sugar.
Imaging	Chest X-ray performed
Imaging	MRT NNA performed
Imaging	Ultrasound

Overview: Core Datasets

DZL Core Dataset
Acute Lung Injury
Asthma and Allergy
Chronic Obstructive Pulmonary Disease (COPD)
Cystic Fibrosis (CF)
Diffuse Parenchymal Lung Disease (DPLD)
Lung Cancer (LC)
Lung Transplant (LTx)
Pneumonia
Pulmonary Hypertension (PH)

Contact

Authors
PD Dr. Anna-Maria Dittrich, BREATH
PD Dr. Lutz Nährlich, UGMLC
PD Dr. Olaf Sommerburg, TLRC
Central Data Management

Science and Research

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