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| Name | Class |
|---|---|
| Cystic Fibrosis Foundation | OTHER |
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The investigators hypothesize that measurement of beta adrenergic induced sweat rate using an evaporimeter can accurately and reliably determine different levels of CFTR dysfunction within a spectrum of patients expressing various degrees of cystic fibrosis disease.
The investigators overall goal is to determine whether the evaporimeter technique of measuring beta-adrenergic induced sweat rate is capable of accurately and reliably identifying different levels of CFTR dysfunction, as a prerequisite before advancing this technique as biomarker assay into clinical trials.
Preliminary data show that following β-adrenergic stimulation, evaporimetry can reliably measure sweat secretion that is: highly reproducible in healthy controls; reduced by 50% in CF obligate heterozygotes; and absent in CF patients carrying severe mutations on both alleles. Further, test- retest experiments suggest good intra-individual reliability. All these features satisfy the required criteria for a biomarker assay that is capable of assessing small increments in CFTR function in vivo in clinical trials designed to assess the effectiveness of correctors and potentiators of CFTR channel activity. Therefore, to provide additional evidence of the value of this novel technique the investigators will determine the accuracy and reliability of evaporimetry to measure Beta-adrenergic induced sweating in subjects with a range of CFTR channel activity.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Sweat Evaporimeter measurement | Other |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Sweat Evaporimeter measurement | Other |
The procedure would take about 45 minutes. |
| Measure | Description | Time Frame |
|---|---|---|
| Measurement of beta-adrenergic induced sweat rate using an evaporimeter can accurately and reliably determine different levels of CFTR dysfunction within a spectrum of patients expressing various degrees of CF disease. | The response patterns will be interpreted and classified by the study PI, as consistent or inconsistent to the published standards. The obtained measurements will be compared to the results obtained from a recently finished validation trial. According to the result of this trial the ranges were as followed: cholinergic response for all subjects 60±20 Evaporimeter units, beta-adrenergic response: Healthy control 51 ± 20 heterozygote -22 ±20, CF -1.4 ±2. | Up to 2 hours |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Frank J Accurso, MD | Children's Hospital Colorado | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital Colorado | Aurora | Colorado | 80045 | United States |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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|
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |