Ignite Creation Date:
2024-05-05 @ 11:55 AM
Last Modification Date:
2024-10-26 @ 9:16 AM
Study NCT ID:
NCT00179439
Status:
COMPLETED
Last Update Posted:
2017-12-13
First Post:
2005-09-12
Brief Title:
NPDT Evaluation in Children With CFTR and PSC
Sponsor:
Beth Israel Deaconess Medical Center
Organization:
Beth Israel Deaconess Medical Center
Study Overview
Official Title:
Nasal Potential Difference Testing Evaluation of Cystic Fibrosis Transmembrane Conductance Regulator CFTR Function in Children With Primary Sclerosing Cholangitis PSC
Status:
COMPLETED
Status Verified Date:
2017-12
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
NPD
Brief Summary:
The investigators hypothesize that PSC in children is associated with mutations and functional changes of the cystic fibrosis CF gene
Detailed Description:
The purpose of this protocol is to perform Nasal Transepithelial Potential Difference NTPD testing to assess the function of the cystic fibrosis gene product a chloride channel referred to as CFTR in patients diagnosed with PSC andor inflammatory bowel disease in childhood and currently 12 years of age and greater
Dr Freedmans laboratory has shown that there is an increased prevalence of CFTR abnormalities in adults with PSC as demonstrated by genotype and phenotype analysis We hypothesize that abnormalities in CFTR based on exhaustive genotype and phenotype assessments are associated with the presence of PSC in children We would like to enroll patients with inflammatory bowel disease and no PSC to use as a control group
Subjects with PSC andor inflammatory bowel disease diagnosed in childhood currently aged 12 years and above will be enrolled in study protocols at Childrens Hospital in Boston which will have received their local IRB approval The only role for BIDMC will be to perform NTPD testing on these subjects No other assessment or testing will be performed at our site We will not be involved in any other aspect of care for these subjects
Dr Freedmans laboratory has shown that there is an increased prevalence of CFTR abnormalities in adults with PSC as demonstrated by genotype and phenotype analysis We hypothesize that abnormalities in CFTR based on exhaustive genotype and phenotype assessments are associated with the presence of PSC in children We would like to enroll patients with inflammatory bowel disease and no PSC to use as a control group
Subjects with PSC andor inflammatory bowel disease diagnosed in childhood currently aged 12 years and above will be enrolled in study protocols at Childrens Hospital in Boston which will have received their local IRB approval The only role for BIDMC will be to perform NTPD testing on these subjects No other assessment or testing will be performed at our site We will not be involved in any other aspect of care for these subjects
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| 02820-4 | None | None | None |