PMID- 31234699
OWN - NLM
STAT- MEDLINE
DCOM- 20210423
LR  - 20220418
IS  - 1531-1937 (Electronic)
IS  - 0897-1900 (Linking)
VI  - 33
IP  - 6
DP  - 2020 Dec
TI  - Successful Long-Term Prophylactic Treatment With Subcutaneous C1 Esterase 
      Inhibitor in a Patient With Hereditary Angioedema.
PG  - 907-911
LID - 10.1177/0897190019857407 [doi]
AB  - BACKGROUND: Hereditary angioedema (HAE) patients suffer from recurrent swellings. 
      Current standard therapy consists of C1 esterase inhibitor (C1-INH) and 
      bradykinin receptor B2 antagonists. Severe courses require prophylactic 
      treatment. For such patients, it has been demonstrated that the intravenous (IV) 
      administration of C1-INH [C1-INH(IV)] is safe and effective. A new prophylactic 
      option is subcutaneous (SC) treatment with C1-INH. METHODS AND CASE: We present 
      the case of an HAE patient placed on prophylactic C1-INH(IV) therapy due to 
      frequent attacks when managed with on-demand therapy. An implanted port allowed 
      the periodical and safe application of medication until the device was explanted 
      due to an infection. Due to the poor venous access, repeated IV application 
      failed. Therefore, we began a SC treatment with 1500 IU C1-INH [C1-INH(SC)] as 
      long-term prophylaxis and analyzed the clinical course over 16 months. RESULTS: 
      Under the SC prophylaxis, the number of attacks were reduced to 1/month in 
      comparison to 4.33/month with no prophylactic treatment and 1.83/month with 
      C1-INH(IV). No severe attacks and no attack within the upper airway occurred over 
      the 16 months of C1-INH(SC) treatment. As a result, quality of life improved, as 
      measured by the Angioedema quality of life questionaire (AE-QoL). CONCLUSION: 
      Self-administered SC prophylactic use of C1-INH over a period of 16 months seems 
      to be a well tolerated and efficient. The patient's quality of life improved, and 
      by learning self-application, the patient gained independence.
FAU - Hahn, Janina
AU  - Hahn J
AUID- ORCID: 0000-0001-7641-7118
AD  - Department of Otorhinolaryngology, Head and Neck Surgery, 27197University Medical 
      Center Ulm, Ulm, Germany.
FAU - Nordmann-Kleiner, Melanie
AU  - Nordmann-Kleiner M
AD  - Department of Otorhinolaryngology, Head and Neck Surgery, 27197University Medical 
      Center Ulm, Ulm, Germany.
FAU - Trainotti, Susanne
AU  - Trainotti S
AD  - Department of Otorhinolaryngology, Head and Neck Surgery, 27197University Medical 
      Center Ulm, Ulm, Germany.
FAU - Hoffmann, Thomas K
AU  - Hoffmann TK
AD  - Department of Otorhinolaryngology, Head and Neck Surgery, 27197University Medical 
      Center Ulm, Ulm, Germany.
FAU - Greve, Jens
AU  - Greve J
AD  - Department of Otorhinolaryngology, Head and Neck Surgery, 27197University Medical 
      Center Ulm, Ulm, Germany.
LA  - eng
PT  - Journal Article
DEP - 20190624
PL  - United States
TA  - J Pharm Pract
JT  - Journal of pharmacy practice
JID - 8900945
RN  - 0 (Complement C1 Inhibitor Protein)
SB  - IM
MH  - *Angioedemas, Hereditary/drug therapy/prevention & control
MH  - Complement C1 Inhibitor Protein
MH  - Humans
MH  - Quality of Life
MH  - Treatment Outcome
OTO - NOTNLM
OT  - C1 inhibitor
OT  - bradykinin
OT  - hereditary angioedema
OT  - subcutaneous
OT  - therapy
EDAT- 2019/06/27 06:00
MHDA- 2021/04/24 06:00
CRDT- 2019/06/26 06:00
PHST- 2019/06/27 06:00 [pubmed]
PHST- 2021/04/24 06:00 [medline]
PHST- 2019/06/26 06:00 [entrez]
AID - 10.1177/0897190019857407 [doi]
PST - ppublish
SO  - J Pharm Pract. 2020 Dec;33(6):907-911. doi: 10.1177/0897190019857407. Epub 2019 
      Jun 24.