Immunodeficiency Evaluation for Chronic Infections in Infants and Children Testing Algorithm

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Immunodeficiency Evaluation for Chronic Infections in Infants and Children Testing Click here for topics associated with this algorithm

Severe viral infections; Candida spp or other fungal Abscesses; pneumonia; delayed separation of umbilical cord; Recurrent respiratory infections with or without chronic diarrhea infections; recurrent sinopulmonary infections recurrent respiratory infections with or without diarrhea

CONSIDER ORDER ORDER  Immunoglobulins (IgA, IgG, IgM), Quantitative  HIV NAAT – test of choice in children ≤18 months (antibodies  Immunoglobulins (IgA, IgG, IgM), Quantitative  Lymphocyte Subset Panel 6 - Total Lymphocyte Enumeration with do not function in infants)  Complement Activity Total, (CH50) and Complement Activity CD45RA and CD45RO or Lymphocyte Subset Panel 7 – Congenital  CD4+ T-Cell Recent Thymic Emigrants (RTEs) Alternate Pathway (AH50) Immunodeficiencies  Lymphocyte Subset Panel 6 - Total Lymphocyte Enumeration  CBC with Platelet Count and Automated Differential  Response to polyvalent pneumococcal vaccine if >2 years with CD45RA and CD45RO or Lymphocyte Subset Panel 7 –  Neutrophil Oxidative Burst Assay (DHR)  Response to diphtheria and tetanus (DT) vaccine Congenital Immunodeficiencies  Leukocyte Adhesion Deficiency Panel  Sweat chloride testing (at accredited cystic fibrosis center)  Lymphocyte Mitogen Proliferation (LMP)  Myeloperoxidase Stain  Lymphocyte Subset Panel 7 – Congenital Immunodeficiencies

Immunoglobulin disorder evaluation T-cell disorder evaluation Abnormal Possible complement complement Common variable activity deficiency B cells immune deficiency present (CVID) or other immune deficiency If all normal, Low IgG, IgM, IgA ORDER contact OR ORDER Low T cells Possible severe Individual complement immunology poor antibody B Cell Subset Immunology consult Low LMP combined testing based on results of director for response to Analysis Abnormal RTEs immunodeficiency CH50, AH50 further May need vaccination evaluation immunoglobulin replacement See ARUP Consult’s Human Low IgG or HIV positive Hypogammaglobulinemia Immunodeficiency IgM Virus topic No B cells Agammaglobulinemia Chronic present Abnormal granulomatous ORDER DHR Low T cells disease Chromosome Low LMP Positive Genetic FISH, DiGeorge Abnormal RTEs for testing Metaphase syndrome Decreased Leukocyte Abnormal facies deletion (specify 22q11.2 CD11b/CD adhesion High IgM Hyper IgM syndrome Consider genetic Hypocalcemia low IgG, IgA likely testing deletion) 18 deficiency, type 1

Leukocyte Decreased adhesion CD15 ORDER ORDER Possible deficiency, type 2 Positive Decreased Cystic Fibrosis (CFTR) 165 Toll-Like chronic sweat Cystic fibrosis response to Candida spp- Pathogenic Variants with Receptor mucocutaneous Possible hyper IgE chloride Candida spp Increased specific IgE Reflex to Sequencing Function candidiasis syndrome IgE neutrophil (Job syndrome) chemotaxis IgA absent Immunology consult Hematology consult Low IgG 2,4 May need immunoglobulin Kostmann Poor pneumococcal replacement with low IgA- May need ORDER agranulocytosis vaccine response containing preparation granulocyte colony Immunoglobulin Low stimulating factor Low IgA G Subclasses neutrophil (1, 2, 3, 4) count Neutrophil IgA absent Warn of possible reaction Autoimmune Symptomatic antibody Normal IgG to IgA-containing blood neutropenia treatment positive subclasses products Absence of Myeloperoxidase myeloperoxidase deficiency IgA low ORDER Consider celiac Normal IgG Innate immune IRAK-4 testing Abnormal All normal subclasses deficiency and toll receptor