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Recombinant human C1 esterase inhibitor treatment for hereditary angioedema attacks in children

Pediatric Allergy and Immunology Aug 07, 2019

Reshef A, Grivcheva-Panovska V, Kessel A, et al. - Given episodes of hereditary angioedema (HAE) because of C1 esterase inhibitor deficiency (C1-INH-HAE) often start during childhood or adolescence, researchers assessed recombinant human C1-INH (rhC1-INH) for HAE attacks in children (aged 2-13 years) in this open-label, phase 2 study. To treat eligible HAE attacks, intravenous administration of rhC1-INH 50 IU/kg body weight (maximum, 4200 IU) was done. Time to the start of symptom relief ( ≥ 20 mm less from baseline in visual analog scale score, persisting for two consecutive evaluations) was assessed as the primary endpoint. Findings revealed the efficacy, safety, and good tolerability of recombinant human C1-INH in children. For HAE attacks in children, the use of same dosing regimen (50 IU/kg; up to 4200 IU, followed by an additional dose, if required) was supported as is currently advised for adolescents and adults.
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