Microsoft word - hae elective and emergency procedures_flowchart_pinuk.doc

(C1-esterase inhibitor deficiency)
Guidance for managing patients presenting for
surgery, bronchoscopy, endoscopy, or dental treatment.
A condition characterised by intermittent attacks of non-pruritic cutaneous, abdominal, or
laryngeal swelling.1-3
– Hereditary: usually family history and commonly presents 1st/2nd decade.1,2,5
– Acquired: autoimmune/low grade lymphoproliferative disorders; usually adult /elderly patients.1,5
Precipitants of acute attacks:1,3,4

– Trauma (especially surgery or dental work). Clinical history:
Identify frequency, severity, and features of attacks.
– Has the patient ever required any treatment for exacerbations? – Is the patient on long-term prophylaxis? – Does the patient have their own supply of C1 inhibitor concentrate? – Does patient carry an advice card? For advice regarding procedure prophylaxis contact on-call Immunologist

Elective procedures:
 Possible agents:3,5
Oral (starting 5 days before the procedure and continued for 2 days thereafter)
- Anti-fibrinolytic agents e.g. tranexamic acid or attenuated androgens e.g. danazol.
- Replacement C1-esterase inhibitor concentrate
– Should be requested from Blood Bank or Pharmacy.
– Dose: 20 units/kg (to nearest 500 units). 1 vial= 500 units. Usual dose 1000 – 1500 units.6
– Reconstitute vial with 10mL of sterile water for injection. Administer slowly via a peripheral IV line at
– During infusion document observations and watch for adverse blood-product related transfusion reactions.6

Emergency procedures:3,5
 Requires intravenous replacement C1-esterase inhibitor concentrate (above), a minimum of 1 hour prior
to surgery.

Post-procedure management:
– Attacks of swelling may occur up to 36 hours after precipitant.5 Patient should be observed for minimum of 12
hours post-procedure and discharged with advice to promptly re-attend an Emergency Department if oral
swelling occurs.
– Some patients keep a home supply of C1-esterase inhibitor concentrate. If this has been used please arrange for
its replenishment.
Developed by Dr Caroline Hawe and Dr David Edgar, Royal Group of Hospitals, Belfast Health and Social Care Trust, Belfast, Northern Ireland. References
1. Agostoni A, Aygoren-Pursun E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004;114(3):S51–S131.
2. Bork K, Meng G, Staubach P, et al. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Clin Med 2006;119:267–274.
3. Bowen T, Cicardi M, Bork K, et al. Hereditary andgioedema: a current state-of-the-art review, VII: Canadian Hungarian 2007 consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. Ann Allergy Asthma Immunol 2008;100(Suppl. 2):S30–
4. Frank MM. Hereditary angioedema: the clinical syndrome and its management in the United States. Immunol Allergy Clin N Am 2006;26:653–668.
5. Gompels MM, Lock RJ, Abinun M, et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol 2005;139:379–394.
6. Berinert Summary of Product Characteristics. CSL Behring. 2009.


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