Home therapy with intravenous human C1‐inhibitor in children and adolescents with hereditary angioedema

Kreuz, W.; Rusicke, E.; Martinez‐Saguer, Inmaculada; Aygören‐Pürsün, Emel; Heller, Christine; Klingebiel, Thomas

doi:10.1111/j.1537-2995.2011.03240.x

Cited by 73 publications

(81 citation statements)

References 26 publications

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“…A detailed individual action and treatment plan should be provided to the families. Self‐ or assisted treatment techniques should be discussed and training programs for these offered 7, 22, 81.…”

Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

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BackgroundThe consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE.MethodsDuring an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting.ResultsThe symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center.ConclusionsThe pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.

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Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

View full text Add to dashboard Cite

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“…Early C1-INH treatment during an attack shortens its duration and reduces its severity more effectively. [76][77][78][79][80] In addition, this might facilitate prophylactic treatment in patients with very frequent angioedema attacks. [81][82][83][84][85] In life-threatening bradykinin-mediated edema of the pharynx or larynx, if the threat of asphyxion is present, conicotomy is necessary.…”

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

Bradykinin-mediated angioedema

Obtułowicz¹

2016

Polish Archives of Internal Medicine

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“…Пациентам с этим заболеванием необходимо лечение в течение всей жизни, они также должны быть информи-рованы о вероятности внезапного возникновения отека, который может затрагивать верхние дыхательные пути и приводить к жизнеугрожающим последствиям [13,14].…”

Section: Discussionunclassified

Hereditary Angioedema in Children — A Rare Clinical Case in the Practice of the Abdominal Surgeon

Дьяконова¹,

Поддубный²,

Никитина³

et al. 2014

Vopr. sovr. pediatr.

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ВВЕДЕНИЕБоль в животе -одна из наиболее частых причин обращения за помощью к врачу во всем мире [1,2]. Причины боли в животе многочисленны, но в первую очередь необходимо исключить хирургическую патоло-гию, что особенно актуально у детей. В хирургической практике принято проводить дифференциальный поиск «от простого к сложному», поэтому, стремясь не пропу-стить острую хирургическую патологию, врачи не всегда вспоминают о редко встречающихся педиатрических заболеваниях.Наследственный ангионевротический отек -хро-ническое заболевание, относящееся к группе пер-вичных иммунодефицитов с аутосомно-доминантным типом наследования и неполной пенетрантностью, связанное с качественным или количественным гене-тически детерминированным дефектом генов, коди-рующих синтез ингибитора эстеразы компонента комплемента C1 (С1 inh ), которое проявляется в виде рецидивирующих отеков кожи и слизистых оболочек дыхательных путей, желудочно-кишечного и урогени-тального трактов [3,4].

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Home therapy with intravenous human C1‐inhibitor in children and adolescents with hereditary angioedema

Abstract: As in adults, home therapy with pC1-INH is effective and safe in the treatment of HAE attacks in pediatric patients; a larger, randomized study should ideally confirm our findings before this approach can be considered the standard of care for pediatric patients.

Cited by 73 publications

References 26 publications

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Bradykinin-mediated angioedema

Hereditary Angioedema in Children — A Rare Clinical Case in the Practice of the Abdominal Surgeon

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