Ignite Creation Date:
2024-05-06 @ 7:03 PM
Last Modification Date:
2024-10-26 @ 2:59 PM
Study NCT ID:
NCT05870410
Status:
NOT_YET_RECRUITING
Last Update Posted:
2023-05-23
First Post:
2023-04-30
Brief Title:
Evaluation Of Non Infectious Presentation of Inborn Errors Of Immunity
Sponsor:
Sohag University
Organization:
Sohag University
Study Overview
Official Title:
Evaluation Of Non Infectious Warning Signs Of Inborn Errors Of Immunity In Children At Sohag University Hospital
Status:
NOT_YET_RECRUITING
Status Verified Date:
2023-05
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:
None
Brief Summary:
Inborn errors of immunity IEI are a wide number of conditions featured by impaired immune response and their classification is in a continuous update with the discovery of new clinical entities Historically the warning signs to identify children at risk of IEI have been defined according to the susceptibility to multiple or severe infectious diseases High recurrence of infections severe infections with need for hospitalization use of intravenous antibiotics and delayed resolution and recently infections by unusual pathogens or restricted pathogen susceptibility are universally recognized as red flags for IEI Recent advances in the clinical comprehension of IEI
the immune dysregulation observed in patients with IEI is clinically expressed with autoimmunity atopy and lymphoproliferation and these manifestations represent the first sign of the disease in about 10 of the patients
the immune dysregulation observed in patients with IEI is clinically expressed with autoimmunity atopy and lymphoproliferation and these manifestations represent the first sign of the disease in about 10 of the patients
Detailed Description:
Inborn errors of immunity IEI are a wide number of conditions featured by impaired immune response and their classification is in a continuous update with the discovery of new clinical entities 1 Historically the warning signs to identify children at risk of IEI have been defined according to the susceptibility to multiple or severe infectious diseases High recurrence of infections severe infections with need for hospitalization use of intravenous antibiotics and delayed resolution and recently infections by unusual pathogens or restricted pathogen susceptibility are universally recognized as red flags for IEI Recent advances in the clinical comprehension of IEI together with the expansion of their genetic background and the formulation of specific genotype-phenotype correlations allowed the better characterization of the non-infectious manifestations of IEI Specifically the immune dysregulation observed in patients with IEI is clinically expressed with autoimmunity atopy and lymphoproliferation and these manifestations represent the first sign of the disease in about 10 of the patients Additionally new diseases with predominant immune dysregulation phenotype in absence of a significant increase of infections have been identified leading to an extension of the paradigm of immunodeficiency During the diagnostic work-up patients with non-infectious onset of IEI are frequently addressed to different specialists including hematologists endocrinologists rheumatologists and allergologists often resulting in a delayed diagnosis In this paper we provide a brief review on the non-infectious manifestations of IEI potentially evidenced at disease onset focusing on the specific items to be considered for the different clinical specialists
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None