C1-esterase inhibitor

SCOPE — BERINERT ONLY. §4.1 verbatim: 'Hereditary angioedema type I and II (HAE) Treatment and pre-procedure prevention of acute episodes.' That is exactly this page's indication, route and population, so the dose above is published. §4.2 verbatim (Adults): 'Treatment of acute angioedema attacks: 20 IU per kilogram body weight (20 IU/kg b.w.) Pre-procedure prevention of angioedema attacks: 1000 IU less than 6 hours prior to a medical, dental, or surgical procedure.' || THE PAGE NAMES TWO BRANDS AND ONLY ONE WAS SOURCED. Brand names on this record are Berinert AND Cinryze; only Berinert 1500 IU (eMC product 7043) was fetched. The two concentrates dose differently — Berinert doses an acute attack by weight (20 IU/kg), whereas Cinryze's regimen is not in this bundle at all. Do not apply the figures above to Cinryze without its own SPC. || NO MAXIMUM IS PUBLISHED BECAUSE THE LABEL PRINTS NONE: §4.2 states no ceiling, no maximum daily total and no maximum number of doses for the 20 IU/kg attack dose, so maxDose is deliberately left empty rather than derived. §4.8 does record thrombosis — in single cases fatal — in treatment attempts with HIGH doses of Berinert for prophylaxis or therapy of Capillary Leak Syndrome, which §4.4 states is an unlicensed indication and dose and is not advised. || §4.2 also requires that treatment be initiated under the supervision of a physician experienced in the treatment of C1-esterase inhibitor deficiency.

§4.2 Paediatric population, verbatim: 'Treatment of acute angioedema attacks: 20 IU per kilogram body weight (20 IU/kg b.w.). Pre-procedure prevention of angioedema attacks: 15 to 30 IU per kilogram body weight (15-30 IU/kg b.w.) less than 6 hours prior to a medical, dental, or surgical procedure. Dose should be selected taking into account clinical circumstances (e.g. type of procedure and disease severity).' || DELIBERATELY NOT WIRED TO THE WEIGHT CALCULATOR. dosePerKg is left null on purpose: the label prints no ceiling for 20 IU/kg, so a mounted calculator would render an uncapped 20 IU/kg x weight on a page that also names Cinryze, whose paediatric regimen is different and unsourced, with no brand selector to distinguish them. The label's own paediatric figures are stated above as text instead — they are Berinert's, are not scaled, and must not be applied to Cinryze. || The pre-procedure paediatric dose is a 15-30 IU/kg BAND that the label says must be selected on clinical circumstances; it is not a fixed figure and is not calculable.