PMID- 26142065 OWN - NLM STAT- MEDLINE DCOM- 20170206 LR - 20220316 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 51 IP - 3 DP - 2016 Dec TI - Impact of Immunoglobulin Therapy in Pediatric Disease: a Review of Immune Mechanisms. PG - 303-314 AB - Intravenous immunoglobulin (IVIG) provides replacement therapy in immunodeficiency and immunomodulatory therapy in inflammatory and autoimmune diseases. This paper describes the immune mechanisms underlying six major non-primary immunodeficiency pediatric diseases and the diverse immunomodulatory functions of IVIG therapy. In Kawasaki disease, IVIG plays a major, proven, and effective role in decreasing aneurysm formation, which represents an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. In immune thrombocytopenia, IVIG targets the underlying increased platelet destruction and decreased platelet production. Although theoretically promising, IVIG shows no clear clinical benefit in the prophylaxis and treatment of neonatal sepsis. Limitations in research design combined with the unique neonatal immunologic environment offer explanations for this finding. Inflammation from aberrant immune activation underlies the myelinotoxic effects of Guillain-Barre syndrome. HIV-1 exerts a broad range of immunologic effects and was found to decrease serious bacterial infections in the pre-highly active anti-retroviral therapy (HAART) era, although its practical relevance in the post-HAART era has waned. Clinical and experimental data support the role of immune mechanisms in the pathogenesis of childhood epilepsy. IVIG exerts anti-epileptic effects through targeting upregulated cytokine pathways and antibodies thought to contribute to epilepsy. Applications in six additional pediatric diseases including pediatric asthma, atopic dermatitis, cystic fibrosis, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS), autism, and transplantation will also be briefly reviewed. From autoimmunity to immunodeficiency, a dynamic immunologic basis underlies major pediatric diseases and highlights the broad potential of IVIG therapy. FAU - Wong, Priscilla H AU - Wong PH AD - Department of Allergy-Immunology, Wilford Hall Ambulatory Surgical Center, San Antonio, TX, USA. priscilla.wong@us.af.mil. AD - , 2200 Bergquist Drive, Suite 1, Lackland AFB, TX, 78236, USA. priscilla.wong@us.af.mil. FAU - White, Kevin M AU - White KM AD - Department of Allergy-Immunology, Wilford Hall Ambulatory Surgical Center, San Antonio, TX, USA. LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Adaptive Immunity MH - Adolescent MH - Age Factors MH - Autoimmune Diseases/diagnosis/*drug therapy/*immunology/mortality MH - Child MH - Child, Preschool MH - Clinical Trials as Topic MH - Humans MH - Immunity, Innate MH - Immunoglobulins, Intravenous/administration & dosage/adverse effects/*therapeutic use MH - Infant MH - Infant, Newborn MH - Inflammation/diagnosis/*drug therapy/*immunology/mortality MH - Treatment Outcome OTO - NOTNLM OT - Autoimmune OT - Immunomodulation OT - Inflammatory diseases OT - Intravenous immunoglobulin therapy OT - Pediatric diseases EDAT- 2015/07/05 06:00 MHDA- 2017/02/07 06:00 CRDT- 2015/07/05 06:00 PHST- 2015/07/05 06:00 [pubmed] PHST- 2017/02/07 06:00 [medline] PHST- 2015/07/05 06:00 [entrez] AID - 10.1007/s12016-015-8499-2 [pii] AID - 10.1007/s12016-015-8499-2 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2016 Dec;51(3):303-314. doi: 10.1007/s12016-015-8499-2.