Reviewing clinical considerations and guideline recommendations of C1 inhibitor prophylaxis for hereditary angioedema
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DOI: 10.1002/clt2.12092
Abstract: Abstract Background Hereditary angioedema (HAE), a rare disease that is characterized by painful and recurring non‐allergic swelling episodes, is caused by the deficiency or dysfunction of C1 inhibitor (C1INH) protein. A comprehensive HAE management plan… read more here.
Keywords: hae; hereditary angioedema; clinical considerations; guideline recommendations ... See more keywords
Current challenges and future opportunities in patient‐focused management of hereditary angioedema: A narrative review
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DOI: 10.1002/clt2.12243
Abstract: Abstract Patients with hereditary angioedema (HAE) experience a high burden of disease due to unpredictable, painful, disfiguring, and potentially life‐threatening HAE attacks. Multiple HAE‐specific medications for the on‐demand treatment, short‐term and long‐term prophylaxis of HAE… read more here.
Keywords: hae; hereditary angioedema; hae management; challenges future ... See more keywords
Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy
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DOI: 10.1007/s10875-018-0553-4
Abstract: PurposeHereditary angioedema (HAE) is a rare disease caused by a C1 inhibitor (C1-INH) deficit. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal oedema attacks. Managing HAE patients in pregnancy is challenging,… read more here.
Keywords: inh; pregnancy; inhibitor; treatment ... See more keywords
Recognition and Management of Hereditary Angioedema: Best Practices for Dermatologists
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DOI: 10.1007/s13555-021-00593-x
Abstract: The goal of this article is to discuss the importance of differentiating hereditary angioedema (HAE) from other types of angioedema, describe advances in HAE management, especially long-term prophylaxis (LTP), and offer practical recommendations for dermatologists.… read more here.
Keywords: management; hereditary angioedema; management hereditary; recognition management ... See more keywords
Complement factor C4 activation in patients with hereditary angioedema.
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DOI: 10.1016/j.clinbiochem.2017.04.007
Abstract: OBJECTIVES Low complement factor C4 is usually considered a valuable screening tool for patients with the potentially life-threatening hereditary angioedema with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE). However, there are patients with C1-INH-HAE presenting with normal C4… read more here.
Keywords: total antigenic; hae patients; non functional; hae ... See more keywords
Refractory Hyperammonemic encephalopathy in Fibrolamellar hepatocellular carcinoma, a case report and literature review.
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DOI: 10.1016/j.currproblcancer.2022.100847
Abstract: Fibrolamellar hepatocellular carcinoma is a rare type of hepatocellular carcinoma with unclear etiology. Its prevalence ranges from 0.6%-5%. One of the rare manifestations of FHCC includes hyperammonemic hepatic encephalopathy (HAE). Data regarding HAE in FHCC… read more here.
Keywords: fibrolamellar hepatocellular; hepatocellular carcinoma; literature review; carcinoma ... See more keywords
Changes of coagulation parameters during erythema marginatum in patients with hereditary angioedema.
Sign Up to like & getrecommendations! Published in 2020 at "International immunopharmacology"
DOI: 10.1016/j.intimp.2020.106293
Abstract: BACKGROUND Hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent episodes of subcutaneous/submucosal edema, which may be preceded by erythema marginatum (EM) as a prodromal symptom. Our aim was to analyze the changes… read more here.
Keywords: erythema marginatum; symptom free; hae attacks; hereditary angioedema ... See more keywords
A first of its kind quantitative functional C1-esterase inhibitor lateral flow assay for hereditary angioedema point-of-care diagnostic testing.
Sign Up to like & getrecommendations! Published in 2020 at "International immunopharmacology"
DOI: 10.1016/j.intimp.2020.106526
Abstract: Hereditary Angioedema (HAE) is a rare, autosomal dominant disease caused by mutations in SERPING1 gene leading to dysfunction/deficiency of C1-esterase inhibitor (C1-INH) protein and subsequent dysregulation of the contact system and bradykinin overproduction. As functional… read more here.
Keywords: point care; plasma; fc1 inh; hae ... See more keywords
Icatibant for the treatment of hereditary angioedema with C1-inhibitor deficiency in adolescents and in children aged over 2 years
Sign Up to like & getrecommendations! Published in 2018 at "Expert Review of Clinical Immunology"
DOI: 10.1080/1744666x.2018.1476851
Abstract: ABSTRACT Introduction: Hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) is a rare disorder with life-threatening complications if untreated. It begins during childhood, and reduces the patient’s quality of life. Therefore, the availability of an… read more here.
Keywords: aged years; treatment; hereditary angioedema; inhibitor deficiency ... See more keywords
Recombinant human C1 esterase inhibitor (Conestat alfa) for prophylaxis to prevent attacks in adult and adolescent patients with hereditary angioedema
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DOI: 10.1080/1744666x.2018.1503055
Abstract: ABSTRACT Introduction: Hereditary angioedema (HAE) due to C1 inhibitor (C1-INH) deficiency is a debilitating and potentially lethal disease. Management includes on-demand treatment of angioedema and their prophylaxis. Plasma derived C1-INH is an established treatment for… read more here.
Keywords: treatment; prophylaxis; conestat alfa; rhc1 inh ... See more keywords
Characteristics of Patients Who Underwent a Diagnostic Test for Hereditary Angioedema Admitted Due to Angioedema.
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DOI: 10.1093/tropej/fmac058
Abstract: OBJECTIVE Hereditary angioedema (HAE) is clinically characterized by recurrent attacks of angioedema. This study evaluated the clinical findings and examination results of patients admitted due to angioedema who then underwent a diagnostic test for HAE.… read more here.
Keywords: hae; hereditary angioedema; angioedema; underwent diagnostic ... See more keywords