PMID- 24490128
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160813
LR  - 20211021
IS  - 2195-5840 (Print)
IS  - 2190-9164 (Electronic)
IS  - 2190-9164 (Linking)
VI  - 2
IP  - 1
DP  - 2012
TI  - Human Plasma-Derived, Nanofiltered, C1-Inhibitor Concentrate (Cinryze(R)), a Novel 
      Therapeutic Alternative for the Management of Hereditary Angioedema Resulting 
      from C1-Inhibitor Deficiency.
PG  - 2
LID - 10.1007/s13554-012-0002-5 [doi]
LID - 2
AB  - Hereditary angioedema resulting from the deficiency of the C1 inhibitor 
      (HAE-C1-INH) is a rare, but potentially life-threatening disorder characterized 
      by paroxysmal episodes of subcutaneous or submucosal edema. Early diagnosis is 
      essential. Management is aimed at the prompt elimination of full-fledged attacks, 
      as well as at the prevention of edematous episodes. The most straightforward 
      means for therapy is supplementation with the deficient C1-INH protein. 
      Placebo-controlled and open clinical studies have established that nanofiltered, 
      human C1-INH concentrate, Cinryze(R) (ViroPharma Inc., Exton, PA, USA) (C1-INHCi), 
      administered in 1,000 U doses is an effective and safe remedy for edematous 
      episodes of HAE-C1-INH, regardless of the localization of the attack. Clinical 
      manifestations rapidly improve and then resolve completely following treatment 
      with this medicinal product. Additionally, C1-INHCi is also appropriate for 
      pre-procedural or for routine prophylaxis. The administration of 1,000 U C1-INHCi 
      before the (dental, surgical, or interventional diagnostic) procedure reduced the 
      incidence of edematous episodes compared with placebo, and this reduction proved 
      significant during routine prophylaxis with the administration of this dose every 
      3-4 days. Relapses did not occur, and repeated dosing had no influence on the 
      efficacy of the preparation. Patients also tolerated treatment with C1-INHCi 
      well. The safety of this preparation was confirmed by the absence of viral 
      transmission as well as by the lack of antibody formation against C1-INH during 
      treatment. Nowadays, C1-INHCi for intravenous use is the only medicinal product 
      indicated both for the prevention and management of edematous attacks.
FAU - Farkas, Henriette
AU  - Farkas H
AD  - 3rd Department of Internal Medicine, Semmelweis University, Kutvolgyi ut 4, 
      H-1225 Budapest, Hungary.
FAU - Varga, Lilian
AU  - Varga L
AD  - 3rd Department of Internal Medicine, Semmelweis University, Kutvolgyi ut 4, 
      H-1225 Budapest, Hungary.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20120509
PL  - England
TA  - Biol Ther
JT  - Biologics in therapy
JID - 101617621
EIN - Biol Ther. 2012 Jan;2(1):4. PMID: 27520465
PMC - PMC3906706
OTO - NOTNLM
OT  - C1-inhibitor deficiency
OT  - Hereditary angioedema
OT  - Human C1 inhibitor concentrate
OT  - Nanofiltered
OT  - Plasma derived
OT  - Prophylaxis
OT  - Safety
OT  - Tolerability
OT  - Treatment
EDAT- 2012/01/01 00:00
MHDA- 2012/01/01 00:01
PMCR- 2012/05/09
CRDT- 2014/02/04 06:00
PHST- 2012/02/16 00:00 [received]
PHST- 2014/02/04 06:00 [entrez]
PHST- 2012/01/01 00:00 [pubmed]
PHST- 2012/01/01 00:01 [medline]
PHST- 2012/05/09 00:00 [pmc-release]
AID - 2 [pii]
AID - 10.1007/s13554-012-0002-5 [doi]
PST - epublish
SO  - Biol Ther. 2012 May 9;2(1):2. doi: 10.1007/s13554-012-0002-5. eCollection 2012.