Abstract | BACKGROUND: Management of patients with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is evolving worldwide. Evaluating the Israeli experience may provide valuable insights. OBJECTIVES: To compare demographics and icatibant treatment patterns and outcomes in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey (IOS) in Israel with those in other countries. METHODS: The IOS is an ongoing observational study that prospectively monitors real-world icatibant safety/tolerability and treatment outcomes. RESULTS: By July 2016, 58 patients from Israel and 594 patients from other countries were enrolled. Median age at diagnosis (16.7 vs. 21.3 years, P = 0.036) and median delay between symptom onset and diagnosis (0.8 vs. 6.6 years, P = 0.025) were lower in Israel compared with other countries, respectively. Differences in attack severity were not significant (P = 0.156); however, during follow-up, Israeli patients were less likely to miss > 7 days of work/school due to C1-INH-HAE-related complications (P = 0.007). A trend was also shown in Israel for earlier time to treatment (median 0.5 vs. 1.3 hours, P = 0.076), attack duration was shorter (median 5.0 vs. 9.0 hours, P = 0.026), and patients more often self-administered icatibant (97.2% vs. 87.5%, P = 0.003), respectively. However, Israeli patients were less likely to treat attacks (P = 0.036). Whereas patients in Israel reported exclusive use of danazol for long-term prophylaxis, those in other countries used various agents, including C1-INH. CONCLUSIONS: Recognition of C1-INH-HAE and timeliness of icatibant treatment appear more favorable, and attack duration shorter, in Israel compared with other countries.
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Authors | Elias Toubi, Shmuel Kivity, Yael Graif, Avner Reshef, Jaco Botha, Irmgard Andresen |
Journal | The Israel Medical Association journal : IMAJ
(Isr Med Assoc J)
Vol. 20
Issue 4
Pg. 227-232
(Apr 2018)
ISSN: 1565-1088 [Print] Israel |
PMID | 29629730
(Publication Type: Journal Article, Observational Study)
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Chemical References |
- Anti-Inflammatory Agents, Non-Steroidal
- icatibant
- Danazol
- Bradykinin
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Topics |
- Adult
- Aged
- Aged, 80 and over
- Angioedemas, Hereditary
(diagnosis, drug therapy, physiopathology)
- Anti-Inflammatory Agents, Non-Steroidal
(administration & dosage, adverse effects, therapeutic use)
- Bradykinin
(administration & dosage, adverse effects, analogs & derivatives, therapeutic use)
- Danazol
(administration & dosage)
- Delayed Diagnosis
- Female
- Follow-Up Studies
- Humans
- Israel
- Male
- Middle Aged
- Prospective Studies
- Registries
- Self Administration
(statistics & numerical data)
- Severity of Illness Index
- Surveys and Questionnaires
- Time Factors
- Treatment Outcome
- Young Adult
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