Disease · MONDO_0019623

hereditary angioedema

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. hereditary angioedema. Diseases, record MONDO_0019623. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0019623

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: hereditary angioedema; Open Targets
Open Targets ID: MONDO_0019623; Open Targets
Other names: HAE, familial angioneurotic edema, familial angioneurotic oedema, hereditary angioneurotic edema, hereditary angioneurotic oedema, hereditary bradykinine-induced angioedema, hereditary non histamine-induced angioedema; Open Targets
Disease Ontology: DOID:14735; Disease Ontology
MeSH: D054179; MeSH
NCI Thesaurus: C84758; NCI Thesaurus
Orphanet: ORPHA:91378; Orphanet
Highest stage of its drugs: Approval, for 11 of its 19 drugs, from ATC, DailyMed, EMA Human Drugs, FDA and TTD; Open Targets

Drugs19

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
CONESTAT ALFA	CHEMBL2108350	Protein	Approval	ATC, EMA Human Drugs and TTD	21	18	18	Drugs and targets
ICATIBANT	CHEMBL2028850	Protein	Approval	ATC, EMA Human Drugs and TTD	13	9	9	Drugs and targets
ECALLANTIDE	CHEMBL1201837	Protein	Approval	ATC and DailyMed	9	7	7	Drugs and targets
SEBETRALSTAT	CHEMBL5095248	Small molecule	Approval	ATC and EMA Human Drugs	9	6	6	Drugs and targets
DONIDALORSEN	CHEMBL4650235	Oligonucleotide	Approval	EMA Human Drugs	8	5	5	Drugs and targets
LANADELUMAB	CHEMBL3545189	Antibody	Approval	ATC, EMA Human Drugs, FDA and TTD	7	3	3	Drugs and targets
BEROTRALSTAT	CHEMBL5189739	Small molecule	Approval	ATC, EMA Human Drugs and TTD	6	2	2	Drugs and targets
HUMAN C1-ESTERASE INHIBITOR	CHEMBL4297549	Protein	Approval	ATC, EMA Human Drugs and TTD	5	2	2	Drugs and targets
ICATIBANT ACETATE	CHEMBL2028852	Protein	Approval	DailyMed	2	1	1	Drugs and targets
BEROTRALSTAT HYDROCHLORIDE	CHEMBL6067995	Small molecule	Approval	FDA	2	0	0	Drugs and targets

1 to 10 of 19

See all 19 drugs

Targets9

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
KLKB1	Plasma kallikrein	6	Plasma kallikrein inhibitor	Approval	https://conductscience.org/library/genes/3818	Drugs and targets
BDKRB2	B2 bradykinin receptor	2	Bradykinin B2 receptor antagonist	Approval	https://conductscience.org/library/genes/624	Drugs and targets
AR	Androgen receptor	1	Androgen Receptor agonist	Approval	https://conductscience.org/library/genes/367	Drugs and targets
C1S	Complement C1s subcomponent	1	Complement C1s inhibitor	Approval	https://conductscience.org/library/genes/716	Drugs and targets
F11	Coagulation factor XI	1	Coagulation factor XI inhibitor	Approval	https://conductscience.org/library/genes/2160	Drugs and targets
F12	Coagulation factor XII	1	Coagulation factor XII inhibitor	Approval	https://conductscience.org/library/genes/2161	Drugs and targets
PLG	Plasminogen	1	Plasminogen inhibitor	Phase 3	https://conductscience.org/library/genes/5340	Drugs and targets
SERPINC1	Antithrombin-III	1	Antithrombin-III activator	Unknown	https://conductscience.org/library/genes/462	Drugs and targets
SERPING1	Plasma protease C1 inhibitor	1	Plasma protease C1 inhibitor exogenous protein	Approval	https://conductscience.org/library/genes/710	Drugs and targets

Trials60

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT04057131	FIRAZYR General Drug Use-Results Survey (Japan)		179	2025-02-21	Hereditary Angioedema (HAE)	ICATIBANT ACETATE	Its record names the disease	ClinicalTrials.gov
NCT00997204	EASSI - Evaluation of the Safety of Self-Administration With Icatibant	Phase 3	151	2014-07-28	Hereditary Angioedema	ICATIBANT	Its record names the disease	ClinicalTrials.gov
NCT00456508	Safety and Efficacy Study of Repeated Doses of DX-88 (Ecallantide) to Treat Attacks of Hereditary Angioedema (HAE)	Phase 3	147	2012-12-28	Hereditary Angioedema (HAE)	ECALLANTIDE	Its record names the disease	ClinicalTrials.gov
NCT05259917	A Phase III, Crossover Trial Evaluating the Efficacy and Safety of KVD900 (Sebetralstat) for On-Demand Treatment of Angioedema Attacks in Adolescent and Adult Patients With Hereditary Angioedema (HAE)	Phase 3	136	2024-07-19	Hereditary Angioedema	SEBETRALSTAT	Its record names the disease	ClinicalTrials.gov
NCT00168103	Human C1 Esterase Inhibitor (C1-INH) in Subjects With Acute Abdominal or Facial Hereditary Angioedema (HAE) Attacks	Phase 2/3	126	2010-08-24	Hereditary Angioedema	CONESTAT ALFA	Its record names the disease	ClinicalTrials.gov
NCT00438815	Open-Label C1 Esterase Inhibitor (C1INH-nf) for the Treatment of Acute Hereditary Angioedema (HAE) Attacks	Phase 3	113	2010-06-09	Hereditary Angioedema	CONESTAT ALFA	Its record names the disease	ClinicalTrials.gov
NCT00912093	A Study of Icatibant in Patients With Acute Attacks of Hereditary Angioedema (FAST-3)	Phase 3	98	2014-08-06	Hereditary Angioedema	ICATIBANT	Its record names the disease	ClinicalTrials.gov
NCT00457015	Efficacy Study of DX-88 (Ecallantide) to Treat Acute Attacks of Hereditary Angioedema (HAE)	Phase 3	96	2010-05-04	Hereditary Angioedema (HAE)	ECALLANTIDE	Its record names the disease	ClinicalTrials.gov
NCT00262080	Efficacy and Safety Study of DX-88 to Treat Acute Attacks of Hereditary Angioedema (HAE)	Phase 3	91	2010-05-11	Hereditary Angioedema (HAE)	ECALLANTIDE	Its record names the disease	ClinicalTrials.gov
NCT05139810	OASIS-HAE: A Study to Evaluate the Safety and Efficacy of Donidalorsen (ISIS 721744 or IONIS-PKK-LRx) in Participants With Hereditary Angioedema (HAE)	Phase 3	91	2025-03-06	Hereditary Angioedema	DONIDALORSEN	Its record names the disease	ClinicalTrials.gov

1 to 10 of 60

See all 60 trials

Comparisons in its trials14

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
KALBITOR (Ecallantide) vs Placebo: Patients With Significant Improvement in Overall Response	22 of 48	12 of 48	96	NCT00457015	Trial comparisons
KALBITOR (Ecallantide) vs Placebo: serious adverse events	0 of 48	3 of 48	96	NCT00457015	Trial comparisons
KALBITOR (Ecallantide) vs Placebo: Proportion of Patients Maintaining a Significant Improvement in Overall Response Through 24 Hours	21 of 48	10 of 47	95	NCT00457015	Trial comparisons
KALBITOR (Ecallantide) vs Placebo: Patients With a Successful Response at 4 Hours Post-dosing, Based on the Change From Baseline in the MSCS Score	45 of 48	28 of 47	95	NCT00457015	Trial comparisons
C1INH-nf vs Placebo: serious adverse events	0 of 71	0 of 12	83	NCT00289211	Trial comparisons
rhC1INH vs Placebo (Saline): serious adverse events	1 of 56	0 of 18	74	NCT01188564	Trial comparisons
C1INH-nf vs Placebo: Number of Subjects With Beginning of Substantial Relief of the Defining Symptom	21 of 35	14 of 33	68	NCT00289211	Trial comparisons
Prepubertal vs Pubertal/Postpubertal: Number of Participants With Adverse Events (AEs)	2 of 11	11 of 21	32	NCT01386658	Trial comparisons
Prepubertal vs Pubertal/Postpubertal: Number of Participants With Clinically Significant Changes in Clinical Laboratory Evaluations	0 of 11	0 of 21	32	NCT01386658	Trial comparisons
Prepubertal vs Pubertal/Postpubertal: Number of Participants With Clinically Significant Changes in Reproductive Hormones	0 of 11	0 of 21	32	NCT01386658	Trial comparisons

1 to 10 of 14

See all 14 comparisons in its trials

Papers from its trials53

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
41794053	RESULT	1	PubMed
38819395	RESULT	1	PubMed
30993784	RESULT	1	PubMed
27788880	RESULT	1	PubMed
26016741	RESULT	1	PubMed
22909164	RESULT	1	PubMed
21884533	RESULT	1	PubMed
21195947	RESULT	1	PubMed
20920772	RESULT	2	PubMed
19767078	RESULT	1	PubMed

1 to 10 of 53

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Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0019623

GET https://conductscience.org/api/diseases/v1/MONDO_0019623/trials?page=2

API reference For AI assistants