Interventions for the long-term prevention of hereditary angioedema attacks

Beard, Nicole A.; Frese, Michael; Smertina, Elena; Mere, Peter; Katelaris, Constance H.; Mills, Kerry

doi:10.1002/14651858.cd013403.pub2

Cited by 6 publications

(5 citation statements)

References 135 publications

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“…HA patients have a mortality rate of 15-34% due to airway edema during an attack [5]. In the present study, only one patient among all patients was followed up as intubated.…”

Section: Discussionmentioning

confidence: 74%

“…Patients have been admitted to the emergency department (ED) an average of 5 times a year [4]. Airway edema in C1 inhibitor defciency (C1INH-HA) has been reported to have a 15-34% mortality rate [5].…”

Section: Introductionmentioning

confidence: 99%

“…Angioedema in HA attacks does not respond to allergic angioedema treatment, therefore making a correct diagnosis is critical [11]. Te mortality rate due to laryngeal edema in undiagnosed cases is approximately 30% [5].…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of the Retrospective LACE Index in Predicting the Risk of Readmission in Patients with Hereditary Angioedema in an Emergency Department

Songur Kodik,

Inci,

Çetin

et al. 2023

Emergency Medicine International

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This study aimed to calculate the LACE index in patients who admitted to the emergency department (ED) with hereditary angioedema (HA) diagnosed and to predict recurrent admissions of patients. In this single-center study, patients aged 18 or higher who were admitted to the ED diagnosed with HA were included over a 12-year period. 35 patients diagnosed with code E88.0 were evaluated according to electronic file records. The number of admissions to the ED in the last 6 months was 2. The LACE index was 4, and risk was 71.4%. The patients admitted to the hospital in the last 30 days had a higher rate of admission to the hospital in the last 6 months ( p < 0.001). The LACE index at admission predicted 30 days admission with (AUC = 0.75, 95% CI (0.56–0.91)) acceptable discrimination. The LACE index and the number of admissions in the last 6 months included in the evaluation can be considered predictive in recurrent ED admissions of HA patients. However, the distribution of LACE-risk groups is no priority. Therefore, the low-, medium-, or high-risk level of LACE index values should be not taken into consideration in readmission of such patients.

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“…HA patients have a mortality rate of 15-34% due to airway edema during an attack [5]. In the present study, only one patient among all patients was followed up as intubated.…”

Section: Discussionmentioning

confidence: 74%

Section: Introductionmentioning

confidence: 99%

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Evaluation of the Retrospective LACE Index in Predicting the Risk of Readmission in Patients with Hereditary Angioedema in an Emergency Department

Songur Kodik,

Inci,

Çetin

et al. 2023

Emergency Medicine International

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“…However, it is worth noting that Watt et al stated definite conclusions about the relative efficacy of compared treatments whereas a number of other recent studies concluded that high levels of between-trial heterogeneity do not allow drawing any such conclusions. For instance, the Cochrane review “Interventions for the long-term prevention of hereditary angioedema attacks.” [ 11 ] states that the rarity of HAE and current evidence base “ does not allow conclusions on the comparative efficacy of the various drugs for people with HAE ”. Similarly, both lanadelumab (Takhzyro ® ) and berotralstat (Orladeyo ® ) have been evaluated through rigorous processes by several national HTA agencies who commented on the appropriateness of ITCs of HAE LTP therapies to inform decision-making.…”

Section: Issue #4mentioning

confidence: 99%

Letter to the editor: network meta-analysis for indirect comparison of lanadelumab and berotralstat for the treatment of hereditary angioedema

Schlueter,

Nestler-Parr

2024

J. Comp. Eff. Res.

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“…Hereditary angioedema is an extremely serious and perhaps fatal medical disorder [ 1 ]. As an autosomal dominant condition, it causes frequent episodes of discomfort, swelling in the oropharynx, gastrointestinal tract, and subcutaneous tissue, and a lower quality of life [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Hereditary Angioedema With a Normal Complement Level

Muna,

Ahmed,

Madaka

et al. 2024

Cureus

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Hereditary angioedema (HAE) is an uncommon autosomal dominant disorder, characterized by episodes of oropharyngeal, gastrointestinal, and subcutaneous tissue swelling, often accompanied by discomfort. HAE is primarily associated with mutations in the SERPING1 gene, resulting in insufficient levels or impaired function of C1 esterase inhibitor (C1-INH), an important regulatory protein of the complement system. While types 1 and 2 HAE are well-established entities caused by quantitative and qualitative defects in C1-INH, respectively, the emergence of type 3 HAE, also known as estrogen-dependent HAE, has expanded our understanding of this complex disorder. In this case, a 2-year-old girl with Down syndrome visited the ER after experiencing lip and tongue swelling following the ingestion of ground pepper. Her laboratory results showed that her complement levels were within normal limits despite clinical symptoms. This situation leads to the specific variant of hereditary angioedema called hereditary angioedema with a normal C1 esterase inhibitor (HAE-NI-C1-INH). Although there are currently no approved treatments, positive responses have been seen to her use of C1-INH concentrate and tranexamic acid to alleviate both immediate and delayed symptoms.

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Interventions for the long-term prevention of hereditary angioedema attacks

Cited by 6 publications

References 135 publications

Evaluation of the Retrospective LACE Index in Predicting the Risk of Readmission in Patients with Hereditary Angioedema in an Emergency Department

Evaluation of the Retrospective LACE Index in Predicting the Risk of Readmission in Patients with Hereditary Angioedema in an Emergency Department

Letter to the editor: network meta-analysis for indirect comparison of lanadelumab and berotralstat for the treatment of hereditary angioedema

Hereditary Angioedema With a Normal Complement Level

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