Confidential
Page 1
27/09/2006
C1 Inhibitor deficiency: Consensus Document
1
TERMS OF REFERENCE ..........................................................................................................3
2
BACKGROUND ...........................................................................................................................3
2.1 GENETICS & PREVALENCE ..........................................................................................................3
2.2
IMMUNOLOGY.............................................................................................................................4
3
DIAGNOSIS ..................................................................................................................................5
3.1 CLINICAL ....................................................................................................................................5
3.2 LABORATORY .............................................................................................................................6
4
MANAGEMENT ..........................................................................................................................7
4.1 PRIMARY PREVENTION................................................................................................................7
4.2 LONG TERM PROPHYLAXIS ..........................................................................................................8
4.3 LONG TERM C1 INH PROPHYLAXIS...........................................................................................10
4.4 SHORT TERM PROPHYLAXIS ......................................................................................................11
PATIENT POSSESSION OF C1 INH CONCENTRATE................................................................................12
4.6 MONITORING OF TREATMENT ...................................................................................................13
4.7 EMERGENCY CARE .................................