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 C1 esterase inhibitor (C1 INH). The possibility that the C1 INH deficiency and 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 of C1 INH deficiency and anaphylactoid reactions to gammaglobulin injections. The possibility that C1 INH levels like C1q 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.
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000201:enzymology / Q000235:genetics* / Q000628:therapy; D000707:Anaphylaxis / Q000209:etiology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003165:Complement System Proteins / Q000032:analysis; D008040:Genetic Linkage; D006801:Humans; D007116:Immunization, Passive; D007273:Injections, Intramuscular; D008297:Male; D014960:X Chromosome; D005719:gamma-Globulins / Q000008:administration & dosage* / Q000009:adverse effects*
chemical_list: D003174:Complement C1 Inactivator Proteins; D005719:gamma-Globulins; D003165:Complement System Proteins