C1 esterase inhibitor deficiency in X-linked hypogammaglobulinaemia: An anomaly fostering anaphylactoid reactions following intramuscular gammaglobulin administration Journal Article


Authors: Pollack, S.; Cunningham-Rundles, C.; Good, R. A.; Day, N. K.
Article Title: C1 esterase inhibitor deficiency in X-linked hypogammaglobulinaemia: An anomaly fostering anaphylactoid reactions following intramuscular gammaglobulin administration
Abstract: A patient with apparent X-linked agammaglobulinaemia was found to be inordinately susceptible to anaphylactoid reactions to intramuscular injections of gammaglobulin. The patient was found also to have low levels of Cl esterase inhibitor (Cl INH). The possibility that the Cl INH deficiency in this patient, whether genetic or acquired, fostered the susceptibility to the production of anaphylactoid reactions after gammaglobulin injections urges further studies of the association ofCl INH deficiency and anaphylactoid reactions to gammaglobulin injections. The possibility that Cl INH levels like Clq levels may be low in hypogammaglobulinaemic patients as a consequence of increased catabolism of this regulator of the complement system when IgG levels are low is considered. © The Fellowship of Postgraduate Medicine, 1986.
Keywords: adult; case report; immunoglobulin; x chromosome; drug therapy; anaphylaxis; adverse drug reaction; therapy; linkage (genetics); complement; intramuscular drug administration; injections, intramuscular; immunization, passive; hypogammaglobulinemia; human; male; priority journal; complement component c1s inhibitor; agammaglobulinemia; support, non-u.s. gov't; support, u.s. gov't, p.h.s.; gamma-globulins; complement deficiency; congenital disorder; complement 1 inactivators
Journal Title: Postgraduate Medical Journal
Volume: 62
Issue: 732
ISSN: 0032-5473
Publisher: BMJ Publishing Group Ltd.  
Date Published: 1986-10-01
Start Page: 939
End Page: 942
Language: English
DOI: 10.1136/pgmj.62.732.939
PUBMED: 2430274
PROVIDER: scopus
PMCID: PMC2419044
DOI/URL:
Notes: Article -- Export Date: 18 August 2021 -- Source: Scopus
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