Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency

Bork, Konrad; Witzke, Günther

doi:10.1016/0091-6749(89)90082-1

Cited by 165 publications

(89 citation statements)

References 21 publications

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“…C′1 inhibitor concentrate has been used successfully for long term replacement in selected adult patients, 74 and more recently it has been shown to be superior to a placebo in a double blind controlled study. 60 Based on the clinical benefit seen in these patients, a role for C′1 inhibitor concentrate in long term prophylaxis for children has been suggested, 60 supporting the few earlier proposals.…”

Section: Long Term Prophylaxis Of Attacks In Childrenmentioning

confidence: 99%

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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Section: Long Term Prophylaxis Of Attacks In Childrenmentioning

confidence: 99%

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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“…C1 inhibitor concentrate has been used successfully for longterm replacement in selected adult patients [124], and more recently it has been shown to be superior to a placebo in a double-blind controlled study [76]. In an uncontrolled trial during long-term follow-up of 14 children with C1 INH deficiency, acute attacks in six children were treated with a single dose of 500 U of C1 INH concentrate (Immuno AG, Vienna, Austria) on 30 separate administrations.…”

Section: C1 Inhibitor Concentrate In Childrenmentioning

confidence: 99%

C1 inhibitor deficiency: consensus document

Gompels

Lock

Abinun

et al. 2005

Clinical and Experimental Immunology

379

460

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SummaryWe present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein. The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants. Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid. There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist. Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification.

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“…Other conditions are self limiting but danazol, stanolol have been tried for acute condition, or for prevention of attacks [5,7]. Aminoepsolic acid has been tried but failed [4]. There is no role of steroids, antihistamines, adrenaline locally or nor epinephrine.…”

Section: Discussionmentioning

confidence: 99%

Hereditary Angioedema-Presenting as Recurrent Abdominal Pain

Killedar¹,

Malani²

2011

Indian J Surg

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We report a very rare case of HEREDITARY ANGIOEDEMA, presenting as recurrent acute abdomen. A 22 yr old Maharastrian male, law college student, got admitted for about fifteen times in previous three years for severe, acute onset, upper abdominal pain, vomiting, distention and acute exudative inflammatory ascites .The whole episode used to subside spontaneously within 2-3 days with or without conservative general management .He underwent various investigations from far basic type, to advanced and invasive type with each recurrence but without definitive diagnosis. He also underwent unnecessary appendectomy. The authors did a review of his previous records, but didn't find any definite surgical or medical cause for his acute abdomen. Obviously it was something rare. Authors did search for it in various surgical and medical literature and searched extensively on internet for rare causes of abdominal pain which guided them for further appropriate investigations and diagnose him as a case of HEREDITARYANGIOEDEMA ,as his clinical features and C1-INH,C3-C4 levels were strongly in favour of it . It goes without saying that the internet has become a standard accessory to conventional literature for cases with diagnostic dilemmas and for treatment options as well.

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Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency

Cited by 165 publications

References 21 publications

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

C1 inhibitor deficiency: consensus document

Hereditary Angioedema-Presenting as Recurrent Abdominal Pain

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