A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema

Kunschak, M; Engl, Werner; Maritsch, Friedrich; Rosen, Fred S.; Eder, Gerald; Zerlauth, Gerold; Schwarz, Hans

doi:10.1046/j.1537-2995.1998.38698326333.x

Cited by 137 publications

(112 citation statements)

References 16 publications

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“…This both shortens the duration of attacks by about a third and also halves the time to the beginning of the relief of symptoms. 28 For acute attacks of abdominal oedema, pain relief should be given at an appropriate level and C′1 inhibitor concentrate should be infused at the same dose as above (vide infra). The patient should be closely observed because the median time, on average, to the beginning of the relief of symptoms after concentrate infusion is about six hours, with resolution after 24 hours.…”

Section: Treatment Of Acute Attacksmentioning

confidence: 99%

See 1 more Smart Citation

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

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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Section: Treatment Of Acute Attacksmentioning

confidence: 99%

“…The patient should be closely observed because the median time, on average, to the beginning of the relief of symptoms after concentrate infusion is about six hours, with resolution after 24 hours. 28 If symptoms persist at a high intensity after this, an alternative diagnosis should be considered.…”

Section: Treatment Of Acute Attacksmentioning

confidence: 99%

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

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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“…Blood samples were collected before administration of the loading dose and at 1,3,6,9,12,15,18,24,36,48, and 72 to 96 hours after the start of loading dose administration. Clotting was prevented with ethylenediaminetetraacetic acid (EDTA), and plasma samples obtained by immediate routine centrifugation were stored at -70°C until further analysis.…”

Section: Methodsmentioning

confidence: 99%

“…Apart from this study, all studies used single intravenous bolus injections of Cl-INH. With the exception of the study of Kunschak et al, 15 in which the total observation time was limited to 24 hours, Cl-INH concentrations were measured for at least 72 hours.…”

Section: Ter and Err Valuesmentioning

confidence: 99%

Pharmacokinetics of C1‐inhibitor protein in patients with acute myocardial infarction

Diris

2002

Clin Pharma and Therapeutics

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Objectives: Cl-inhibitor protein (Cl-INH) purified from pooled human plasma is used for the treatment of patients with hereditary angioedema. Recently, the beneficial effects of high-dose Cl-INH treatment on myocardial ischemia or reperfusion injury have been reported in various animal models and in humans. We investigated the pharmacokinetic behavior of Cl-INH in patients with acute myocardial infarction to calculate the amount ofCI-INH required for optimal efficacy. Methods: Twenty-two patients received an intravenous loading dose, followed by 48 hours of continuous infusion of Cl-INH. Changes in the endogenous production of Cl-INH were evaluated in 16 control patients with acute myocardial infarction. A 2-compartment model was used to estimate the fractional catabolic rate constant (FCR), transcapillary escape rate constant (TER), and extravascular return rate constant (ERR) of Cl -INH. Software designed to analyze and fit measured data to unknown parameters in a system of differential equations was used to fit the experimental data against the 3-parameter model. Maastricht and Amsterdam, The NetherlandsCl-inhibitor protein (Cl-INH), a protein of approximately 104 kd, regulates the classical activation pathway of the complement system. 1 Patients with hereditary angioedema (HAE), in whom there is a congenital lack of Cl-INH, have attacks of uncontrolled complement activation that may lead to life-threatening laryngeal obstruction or swelling of the gastrointestinal mucosa.2 Depending on the severity of the edematous attack, treatment of patients with HAE usually consists of one or more bolus injections of CI-INH purified from pooled human plasma.

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“…This has indeed been observed in clinical practice; no side effects have been reported. C1-INH prepared from normal plasma in concentrated form is used as short term replacement therapy for the treatment of attacks of HANE (11). This therapy is life saving in laryngeal edema.…”

Section: Treatment Of Hereditary Angioneurotic Edema (Hane)mentioning

confidence: 99%

Therapeutic inhibition of the complement system Y2K update

Asghar¹

2000

Front Biosci

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A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema

Abstract: C1 inhibitor concentrate is a safe, effective treatment for acute attacks of HAE.

Cited by 137 publications

References 16 publications

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

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

Pharmacokinetics of C1‐inhibitor protein in patients with acute myocardial infarction

Therapeutic inhibition of the complement system Y2K update

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