Inbon Errors of Immunity Attending Assiut University Children&Amp;#39;s Hospital: a Single Center Study

NCT06649643 | Not Yet Recruiting

• 1 other identifier: IEIs of Assuit hospital
• Study Type: observational
• Target: 60
• Locations: 0 countries
• Sites: N/A

Brief Summary

Aim of Study The aim of this study to describe the clinical profile of children diagnosed as IEIs who were admitted to in Assiut university children\'s Hospital.

Conditions

Primary Immunodeficiency Diseases (PID)

Outcome Measures

Primary Outcomes (1)

• Profile of Patients with Inborn Errors of Immunity in Assiut University Children Hospital: Single Center Study

  Warning signs for early diagnosis * Weight in kilogram * Height in meters investigation --Routine * CBC with blood film. * Liver Function Test. * Kidney Function Test. * Inflammatory marker ESR,CRP --Immunology * Immunoglobulins IgG, IgM, IgA, IgE * Flow cytometric assessment: B Cell (CD19), T cells (CD3,CD4, CD8) and NK Cells (CD16+56) and other markers whenever needed (e.g. CD40, CD40L for cases of hyper IGM) * Chest x-ray to assess thymus and any abnormality * DHR123 whenever needed (e.g. chronic granulomatous disease). * LAD panel ( CD11b CD15- CD18) whenever needed. .Complement assay (CH 50,C3,C4) whenever needed .

  Baseline

Interventions

Inborn errors of immunity DIAGNOSTIC_TEST

A. Routine .1CBC with blood film. .2Liver Function Test. .3Kidney Function Test. 4\. Inflamatory marker ESR, CRP B.Immunology 1. Immunoglobulins IgG, IgM, IgA, IgE 2. Flow cytometric assessment: B Cell (CD19), T cells (CD3,CD4, CD8) anc NK Cells (CD16+56) and other markers whenever needed (e.g. CD40, CD40L for cases of hyper IGM) 3. Chest x-ray to assess thymus and any abnormality 4. DHR123 whenever needed (e.g. chronic granulomatous disease). 5. LAD panel (CD11b CD15-CD18) whenever needed. 6\. Complement assay (CH

A. Routine .1CBC with blood film. .2Liver Function Test. .3Kidney Function Test. 4. Inflamatory marker ESR, CRP B.Immunology 1-Immunoglobulins IgG, IgM, IgA, IgE 2-Flow cytometric assessment: B DIAGNOSTIC_TEST

A. Routine .1CBC with blood film. .2Liver Function Test. .3Kidney Function Test. 4\. Inflamatory marker ESR, CRP B.Immunology 1. Immunoglobulins IgG, IgM, IgA, IgE 2. Flow cytometric assessment: B Cell (CD19), T cells (CD3,CD4, CD8) anc NK Cells (CD16+56) and other markers whenever needed (e.g. CD40, CD40L for cases of hyper IGM) 3. Chest x-ray to assess thymus and any abnormality 4. DHR123 whenever needed (e.g. chronic granulomatous disease). 5. LAD panel (CD11b CD15-CD18) whenever needed. 6\. Complement assay (CH

Eligibility Criteria

• Age: Up to 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

Sex- all Age - maximum age 18 y

You may qualify if:

• infections within 1 year,two or more serious sinus infections within 1 year,two or more months on antibiotics with little effect,two or more pneumonias within 1 year,failure of an infant to gain weight or grow normally,recurrent, deep skin or organ abscesses, persistent thrush in mouth or fungal infection on skin, need for intravenous antibiotics to clear infections,Two or more deep-seated infections including septicemia and family history of IEIs -

You may not qualify if:

• patients without any criteria of ten warning signs

Sponsors & Collaborators

• Assiut University: lead

Related Publications (2)

• Tangye SG, Al-Herz W, Bousfiha A, Chatila T, Cunningham-Rundles C, Etzioni A, Franco JL, Holland SM, Klein C, Morio T, Ochs HD, Oksenhendler E, Picard C, Puck J, Torgerson TR, Casanova JL, Sullivan KE. Correction to: Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2020 Jan;40(1):65. doi: 10.1007/s10875-020-00763-0.

  PMID: 32086639 RESULT

• Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, Klein C, Morio T, Oksenhendler E, Picard C, Puel A, Puck J, Seppanen MRJ, Somech R, Su HC, Sullivan KE, Torgerson TR, Meyts I. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022 Oct;42(7):1473-1507. doi: 10.1007/s10875-022-01289-3. Epub 2022 Jun 24.

  PMID: 35748970 RESULT

MeSH Terms

Conditions

Primary Immunodeficiency Diseases

Condition Hierarchy (Ancestors)

Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Immunologic Deficiency Syndromes; Immune System Diseases

Central Study Contacts

Christeena Goda Hakeem, Resident of Pediatrics

CONTACT

01271711547; madonaj985@gmail.com

Ismail Lotfy Mohamad, Professor of Pediatrics

CONTACT

01063398967; ismail231@aun.edu.eg

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Inbon errors of immunity attending Assiut University Children's Hospital: a single center study

Study Record Dates

• First Submitted: September 27, 2024
• First Posted: October 21, 2024
• Study Start: January 5, 2025
• Primary Completion: January 5, 2026
• Study Completion: March 5, 2026
• Last Updated: October 21, 2024

Record last verified: 2024-10

Data Sharing

• IPD Sharing: Will not share