Contributions to the Optimal Use of Human Blood

Ef, Vogelaar; Hg, Brummelhuis; Hw, Krijnen

doi:10.1111/j.1423-0410.1974.tb02676.x

Cited by 41 publications

(3 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The first large-scale attempt to purify C1-esterase inhibitor was documented in 1974[84]. The development of improved manufacturing processes resulting in purified human plasma-derived C1-esterase inhibitor formulations has enabled their use for the treatment of HAE attacks since the 1980s[85].…”

Section: Experience With C1-esterase Inhibitor In the Long-term Prophmentioning

confidence: 99%

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower¹,

Busse²,

Aygören‐Pürsün³

et al. 2011

World Allergy Organization Journal

View full text Add to dashboard Cite

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-esterase inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.

show abstract

Section: Experience With C1-esterase Inhibitor In the Long-term Prophmentioning

confidence: 99%

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower¹,

Busse²,

Aygören‐Pürsün³

et al. 2011

World Allergy Organization Journal

View full text Add to dashboard Cite

show abstract

“…Although it is derived from human plasma with risk of infectious disease, beginning in the 1970s, efforts started in Europe to manufacture a more purified preparation of missing protein 60. The blood-borne contagious risk has been reduced by multiple viral inactivation and removal steps including cryoprecipitation, chromatography, and pasteurization; nanofiltration is added to a recently US Food and Drug Administration (FDA)-approved C1-INH concentrate.…”

Section: Replacement Therapy: Ffp and C1-inh Concentrates (Viral Tranmentioning

confidence: 99%

Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor

Cardona

Bellfill²,

Caus³

2010

TACG

View full text Add to dashboard Cite

Hereditary angioedema (HAE) is a potentially fatal genetic disorder typified by a deficiency (type I) or dysfunction (type II) of the C1-inhibitor (C1-INH) and characterized by swelling of the extremities, face, trunk, abdominal viscera, and upper airway. Type III is normal estrogen-sensitive C1-INH HAE. Bradykinin, the main mediator of HAE, binds to endothelial B2 receptors, increasing vascular permeability and resulting in edema. HAE management includes short- and long-term prophylaxis. For treating acute episodes, C1-INH concentrate is recommended with regression of symptoms achieved in 30–90 min. Infusions of 500–1000 U have been used in Europe for years. Two plasma-derived C1-INH concentrates have been licensed recently in the United States: Berinert® for treating acute attacks and Cinryze® for prophylaxis in adolescent/adult patients. A recombinant C1-INH that is being considered for approval (conestat alfa) exhibited significant superiority versus placebo. Ecallantide (Kalbitor®) is a selective kallikrein inhibitor recently licensed in the United States for treating acute attacks in patients aged >16 years. It is administered in three 10-mg subcutaneous injections with the risk of anaphylactic reactions. Icatibant (Firazyr®) is a bradykinin B2 receptor competitor. It is administered subcutaneously as a 30-mg injection and approved in Europe but not in the United States.

show abstract

“…This specific plasma has to contain about one tenth of the wanted strength of the final specific immunoglobulin. In the fractionation of specific plasma the division of the immunoglobulin over fraction II and III is mostly analogous with the division of the specific antibodies with exception of anti-D which possesses a lower recovery in fraction II (14).…”

mentioning

confidence: 98%

Ethanol Fractionation of Human Plasma: An Overview

Brummelhuis¹

1985

Plasma Fractionation and Blood Transfusion

Self Cite

View full text Add to dashboard Cite

Human plasma is an extraordinarily complex aqueous solution of proteins, hormones, lipoids, carbohydrates etc., from which certain proteins are of interest in therapy. At the moment a small but increasing number of the hundreds of plasma proteins are used in clinical medicine and have to be prepared by fractionation. The methods applied in fractionation are more or less the same as for purification of proteins, but the aim is quite different; fractionation is meant to make the most out of plasma in terms of the clinical efficacy of the wanted protein(s). The safety of the preparations must be guaranteed by avoiding possible changes ill the native structure of the protein(s) or contamination that causes side effects. The economics of fractionation also have to be considered. To reach this aim, good recovery and efficacy of the protein(s) are necessary and are mainly dependent on controlled methods and technology. The safety is dependent mainly on a correct interpretation of "Good Manufacturing Practices", which primarily is to be found in well instructed and well educated personnel, adequate housing, sanitation and equipment. Continuous attention is needed to control the costs.During the last forty years a number of methods have been tried and performed in the fractionation of human plasma based on: differential solubility differential interaction with solid media differential interaction with physical fields. For the preparation of the bulk plasma proteins, i.e. fibrinogen, immunoglobulin and albumin, the cold ethanol method, based on different solubility is generally practised although other methods based on different solubility are or have been used like fractionation with ammonium sulphate (1) and ether(2).The separation between the proteins is performed under conditions where either the solubility of the desired protein is maximized and the solubility of the other proteins is minimized or reversed. The precipitate is separated from the supernatant by semi-continuous centrifugation and if needed, clarification.Variables determining the precipitation of the protein(s) are: ethanol concentration pH temperature ionic strength protein concentration from which the last two are less critical than the other variables and therefore mostly left out of the fractionation schemes. The first large scale batch wise fractionation of human plasma using acetate buffers was described by Cohn and coworkers who developed a number of methods from which the method 5 and 6 (3) and the immunoglobulin method 9 according to Oncley et aI. (4) are the best known.In the flowsheet ( fig. 1) other products like plasminogen, prothrombin complex etc., which can be prepared from fraction II+III and a further purification of fraction II has been left out. By this combined method five preparations can be derived from human plasma: C. T. Smit Sibinga et al. (eds.), Plasma Fractionation and Blood Transfusion

show abstract

Contributions to the Optimal Use of Human Blood

Cited by 41 publications

References 14 publications

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor

Ethanol Fractionation of Human Plasma: An Overview

Contact Info

Product

Resources

About