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The goal of this observational study is to assess the main clinical and anamnestic characteristics, and frailty syndrome in an adult Cystic Fibrosis population. The main question it aims to answer is the possible association of the frailty status with the main clinical, therapeutical characteristics, including the genotyping classification of Cystic Fibrosis patients.
Participants will be assessed for the pulmonary status by spirometry (for Forced Expiratory Volume in 1 second [FEV1 - L]; Forced Expiratory Volume in 1 second percent predicted [ppFEV1%]; Forced Vital Capacity [FVC - L]; Forced Vital Capacity percent predicted [FVC %]; Maximal Mid-Expiratory Flow [MMEF]); for the functional status by evaluating the activities of daily living [ADLs], and the instrumental ADLs [IADLs]; and for frailty by the Study of Osteoporotic Fractures [SOF] Index. The height and weight will be also recorded, and Body Mass Index [BMI] calculated as body weight divided by height squared [Kg/m2]. Body weight will be measured in a fasting state in the morning with a mechanical balance.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Adults with Cystic Fibrosis | All patients who met the diagnostic criteria for CF, over 18 years old, with pathological sweat chloride levels (chloride >60 mEq/L) and two CFTR mutations were recruited. Sweat chloride levels have been tested, and a panel of CFTR mutations screened. The CFTR genotype has been defined through the screening of the most frequent mutations and rearrangements. |
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| Measure | Description | Time Frame |
|---|---|---|
| Pre-Frail/Frail Status | Frailty has been assessed by the SOF Index | through study completion, an average of 1 year |
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Inclusion Criteria:
Exclusion Criteria:
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The study population consists of 139 CF patients referred to the Cystic Fibrosis for the Adults Centre of the University Federico II of Naples.
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| Name | Affiliation | Role |
|---|---|---|
| Graziamaria Corbi, MD, PhD | Federico II University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Cystic Fibrosis for the Adults Centre | Naples | 80131 | Italy |
the data will be treated as pooled
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| ID | Term |
|---|---|
| D000073496 | Frailty |
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
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| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |