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An Open-Label study to Evaluate the Long-Term Safety of Daily Oral BCX7353 in subjects with Type I and II Hereditary Angioedema

Phase 2
Completed
Conditions
HAE
Hereditary Angioedema
10018849
Registration Number
NL-OMON48865
Lead Sponsor
BioCryst Pharmaceuticals Inc.
Brief Summary

Trial is onging in other countries

Detailed Description

Not available

Recruitment & Eligibility

Status
Completed
Sex
Not specified
Target Recruitment
3
Inclusion Criteria

1. Males and nonpregnant, nonlactating females >=18 years of age (main study) ;
2. Subjects with Type I or II HAE who have either participated in a BCX7353
efficacy study OR
expected to derive benefit from oral treatment for the prevention of angioedema
attacks and have a clinical diagnosis of HAE Type 1 or Type2, defined as C1-INH
functional level below 50% and C4 level below lower limit of normal OR a known
SERPING-1 gene mutation known or likely to be associated with HAE Type 1 or 2
OR confirmed family history OR low C4 value from a sample drawn during a HAE
attack.
3. Subject weight >= 40kg
4. Access to appropriate medication for the treatment of acute HAE attacks
5. Females only - Agreement to use acceptable effective contraception
6. Able to provide written, informed consent
7. In the opinion of the Investigator, the subject is able to adequately comply
with all required study procedures for the duration of the study. The subject
must demonstrate adequate compliance with all study procedures required
including diary recording of HAE attacks

Exclusion Criteria

1. Pregnancy or breast feeding or planned pregnancy during the study period
2. Any clinically significant medical condition or medical history that, in the
opinion of the Investigator or Sponsor, would interfere with the subject*s
safety or ability to participate in the study
3. Discontinuation of study drug due to a hypersensitivity reaction to BCX7353
in a prior study. This includes subjects who had a rash of any severity
identified as possibly, probably, or definitely related to active BCX7353 in
the previous study
4. Dementia, altered mental status or any psychiatric condition, that would
prohibit the understanding or rendering of informed consent or participation in
the study
5. Clinically significant abnormal ECG including but not limited to, a QTcF >
470 msec for women, a QTcF > 450 msec for men, a PR > 220 msec (both sexes), or
ventricular and/or atrial premature contractions that are more frequent than
occasional, and/or as couplets or higher in grouping
6. Unacceptable noncompliance in the previous BCX7353 efficacy study as
assessed by the Sponsor or Investigator
7. Any clinically significant history of angina, myocardial infarction,
syncope, clinically significant cardiac arrhythmias, left ventricular
hypertrophy, cardiomyopathy, or any other cardiovascular disease
8. Known family history of sudden cardiac death. Family history of sudden death
from HAE is not exclusionary
9. History of or current implanted defibrillator or pacemaker
10. Use of concomitant medications that are metabolized by CYP2D6, CYP2C9,
CYP2C19, or CYP3A4 and have a narrow therapeutic range, within 7 days of the
baseline visit or planned initiation during the study, including those known to
prolong the QT interval
11. Use of a medication that is transported by P-gp and has a narrow
therapeutic range, within 7 days of the baseline visit or planned initiation
during the study
12. Any laboratory parameter abnormality that, in the opinion of the
Investigator, is clinically significant and relevant for this study
13. Calculated creatinine clearance (CLcr) of <= 60 mL/min or AST or ALT value >=
2 times the ULN reference range value at screening or at the last available
visit prior to enrollment
14. Investigational drug exposure, other than BCX7353, within 30 days prior to
the screening visit (or baseline if no screening visit)
15. History of severe hypersensitivity to any medicinal product, which was
associated with non HAE-related swelling, a severe rash requiring treatment /
hospitalization, or anaphylaxis
16. History of alcohol or drug abuse within the previous year, or current
evidence of substance dependence or abuse (self-reported alcoholic intake > 3
units of alcohol/day)
17. For subjects undergoing a screening visit, a positive drugs of abuse screen
(unless used as a medical treatment [e.g., with a prescription])
18. For subjects undergoing a screening visit, current infection with hepatitis
B virus (HBV), hepatitis C virus (HCV), or human immunodeficiency virus (HIV)
19. Subjects with an immediate family relationship to either Sponsor employees,
the Investigator or employees of the study site who are named on the delegation
log
20. Subjects who are held in an institution by a government or judicial order

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>To evaluate the long-term safety and tolerability of daily dosing of oral<br /><br>BCX7353 in subjects with hereditary angioedema (HAE)</p><br>
Secondary Outcome Measures
NameTimeMethod
<p>To assess the effectiveness (i.e., HAE attack frequency, severity and disease<br /><br>activity over time) of BCX7353 during long-term administration<br /><br>To evaluate quality of life during long-term administration of BCX7353<br /><br>To evaluate subject*s satisfaction with medication during long term<br /><br>administration of BCX7353</p><br>
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