title: Plasma-derived C1-INH for managing hereditary angioedema in pediatric patients: A systematic review.
abstract: Presently, medications approved for children with Hereditary Angioedema (HAE) are extremely limited. This is especially the case for children under 12 years of age. For this reason we reviewed and summarized the data on treatment of children with HAE. Available data indicate that plasma derived C1-inhibitor is a safe, effective treatment option for HAE in pediatric patients, including those below 12 years of age. Other therapies are also appear safe for the under 12 year of age, but less data are available. Importantly, home-based treatment of HAE in this age group appears to be safe and effective and can improve quality of life. These findings support current HAE consensus guidelines which strongly recommend the use of plasma derived C1-inhibitor as a first-line treatment in children and encourage home and self-treatment.
authors.affiliation: Department of Medicine and Pediatrics, Penn State University, Hershey, PA, USA.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Craig
authors.affiliation: Department of Medicine and Pediatrics, Penn State University, Hershey, PA, USA.|Division of Immunology, Boston Children's Hospital, Boston, USA.|Department of Pediatrics, Harvard Medical School, Boston, USA.
authors.forename: Lynda C
authors.initials: LC
authors.lastname: Schneider
authors.affiliation: Department of Medicine and Pediatrics, Penn State University, Hershey, PA, USA.|Division of Immunology, Boston Children's Hospital, Boston, USA.|Department of Pediatrics, Harvard Medical School, Boston, USA.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: MacGinnitie
mesh_terms: D000293:Adolescent; D000367:Age Factors; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D050718:Complement C1 Inhibitor Protein / Q000009:adverse effects / Q000302:isolation & purification / Q000627:therapeutic use*; D006699:Home Care Services; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000302:isolation & purification / Q000627:therapeutic use*; D010949:Plasma / Q000737:chemistry*; D017410:Practice Guidelines as Topic; D012648:Self Care; D016896:Treatment Outcome
chemical_list: D050718:Complement C1 Inhibitor Protein; D007155:Immunologic Factors
keywords: Berinert; Cinryze; children; efficacy; hereditary angioedema; pediatric; plasma-derived C1-inhibitor; safety