Alpha-1 Proteinase Inhibitor

Source: UK SPC (eMC) for Respreeza 1,000 mg powder and solvent for solution for infusion, §4.2 (https://www.medicines.org.uk/emc/product/7026/smpc). SCOPE: the figures above are Respreeza's. If any other alpha-1-proteinase inhibitor product is used, dose it from that product's own SPC — a per-product check, not an assumption that all alpha-1-proteinase inhibitors share a regimen; no other product was fetched in this bundle. NO MAXIMUM DOSE is stated in §4.2 — 60 mg/kg once weekly is the single recommended dose, so no maxDose field is given here rather than inventing a ceiling. SUPERVISION (§4.2): 'First infusions should be administered under the supervision of a healthcare professional experienced in the treatment of alpha 1-proteinase inhibitor deficiency. Subsequent infusions can be administered by a caregiver or by the patient.' There are limited data on home-treatment/self-administration; the treating doctor decides suitability and must ensure appropriate training (reconstitution, use of the Mix2Vial set, assembly of intravenous tubing, infusion technique, maintenance of a treatment diary, identification of adverse reactions and the measures to take), reviewed at regular intervals. INFUSION REACTIONS: if any reaction occurs that might be related to administration, decrease the rate or stop the administration as the patient's clinical condition requires; if symptoms subside promptly after stopping, the infusion may be resumed at a lower rate that is comfortable for the patient. PAEDIATRIC: paedDose is null because the label carries no paediatric figure — VERBATIM §4.2: 'The safety and efficacy of Respreeza in the paediatric population (below 18 years) have not been established. No data are available.' Do not scale the adult 60 mg/kg for a child from this bundle. ELDERLY: safety and efficacy in patients 65 years of age or older have not been established in specific clinical trials. RENAL/HEPATIC IMPAIRMENT: no special investigations have been performed and no alternative dose regimen can be recommended in those patients. One vial is for single use only. TRACEABILITY: record the name and batch number of the administered product each time. SOURCE NOTE: §4.4 in this bundle is truncated at the source-fetch limit (it ends mid-sentence in the transmissible-agents paragraph) and §4.5 was not retrieved — verify the full warnings and interactions sections before use. §4.2 was retrieved complete and is the basis of every figure above.