Literature DB >> 849647

A second case of human C3b inhibitor (KAF) deficiency.

R A Thompson, P J Lachmann.   

Abstract

The second case of C3b inhibitor deficiency is described in an 11-year-old girl who presented with recurrent attacks of meningitis, in between which she was well. Her serum showed all of the complement component changes noted in the first described case, although showing only a relatively slight defect in its ability to opsonize bacteria for phagocytosis and killing by polymorphonuclear leucocytes. This correlated with the patient's freedom from other infections.

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Year:  1977        PMID： 849647      PMCID： PMC1540911     

Source DB:  PubMed          Journal:  Clin Exp Immunol        ISSN： 0009-9104            Impact factor:   4.330


  15 in total

1.  A BIOCHEMICAL ABNORMALITY IN HEREDIATRY ANGIONEUROTIC EDEMA: ABSENCE OF SERUM INHIBITOR OF C' 1-ESTERASE.

Authors:  V H DONALDSON; R R EVANS
Journal:  Am J Med       Date:  1963-07       Impact factor: 4.965

2.  [Acute lupus erythematosus with total absence of the C4 fraction of complement].

Authors:  G Hauptmann; E Grosshans; E Heid; S Mayer; A Basset
Journal:  Nouv Presse Med       Date:  1974-04-06

3.  Hereditary C2 deficiency with some manifestations of systemic lupus erythematosus.

Authors:  V Agnello; M M De Bracco; H G Kunkel
Journal:  J Immunol       Date:  1972-03       Impact factor: 5.422

4.  Homozygous deficiency of C3 in a patient with repeated infections.

Authors:  C A Alper; H R Colten; F S Rosen; A R Rabson; G M Macnab; J S Gear
Journal:  Lancet       Date:  1972-12-02       Impact factor: 79.321

5.  The alternate pathway of complement activation. The role of C3 and its inactivator (KAF).

Authors:  P A Nicol; P J Lachmann
Journal:  Immunology       Date:  1973-02       Impact factor: 7.397

6.  Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3.

Authors:  C A Alper; K J Bloch; F S Rosen
Journal:  N Engl J Med       Date:  1973-03-22       Impact factor: 91.245

7.  Studies on eosinophil leucocyte migration. II. Factors specifically chemotactic for eosinophils and neutrophils generated from guinea-pig serum by antigen-antibody complexes.

Authors:  A B Kay
Journal:  Clin Exp Immunol       Date:  1970-11       Impact factor: 4.330

8.  Hereditary deficiency of the second component of complement (C2) in man: correlation of C2 haemolytic activity with immunochemical measurements of C2 protein.

Authors:  S Ruddy; M R Klemperer; F S Rosen; K F Austen; J Kumate
Journal:  Immunology       Date:  1970-06       Impact factor: 7.397

9.  Inactivator of the third component of complement as an inhibitor in the properdin pathway.

Authors:  C A Alper; F S Rosen; P J Lachmann
Journal:  Proc Natl Acad Sci U S A       Date:  1972-10       Impact factor: 11.205

10.  Escherichia coli antibody: a screening test for immunodeficiency.

Authors:  A D Webster; T Efter; G L Asherson
Journal:  Br Med J       Date:  1974-07-06
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  20 in total

1.  Complement activation profiles in disease.

Authors:  D L Brown
Journal:  J Clin Pathol Suppl (R Coll Pathol)       Date:  1979

2.  Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family.

Authors:  A S Grumach; M F Leitão; V G Arruk; M Kirschfink; A Condino-Neto
Journal:  Clin Exp Immunol       Date:  2006-02       Impact factor: 4.330

3.  Complement-activating ability of leucocytes from patients with complement factor I deficiency.

Authors:  H V Marquart; J M Rasmussen; R G Leslie
Journal:  Immunology       Date:  1997-07       Impact factor: 7.397

4.  Genetic polymorphism of human factor I (C3b inactivator).

Authors:  S Nakamura; K Abe
Journal:  Hum Genet       Date:  1985       Impact factor: 4.132

5.  The molecular basis of hereditary complement factor I deficiency.

Authors:  T J Vyse; B J Morley; I Bartok; E L Theodoridis; K A Davies; A D Webster; M J Walport
Journal:  J Clin Invest       Date:  1996-02-15       Impact factor: 14.808

Review 6.  The role of complement in the induction and regulation of immune responses.

Authors:  T G Egwang; A D Befus
Journal:  Immunology       Date:  1984-02       Impact factor: 7.397

7.  Hypocomplementaemia due to a genetic deficiency of beta 1H globulin.

Authors:  R A Thompson; M H Winterborn
Journal:  Clin Exp Immunol       Date:  1981-10       Impact factor: 4.330

8.  C3b inactivator deficiency with immune complex manifestations.

Authors:  P Solal-Celigny; M Laviolette; J Hebert; P C Atkins; M Sirois; G Brun; G Lehner-Netsch; J M Delâge
Journal:  Clin Exp Immunol       Date:  1982-01       Impact factor: 4.330

9.  Inherited deficiency of the seventh component of complement associated with nephritis. Propensity to formation of C56 and related C7-consuming activity.

Authors:  G R Nemerow; H Gewurz; S G Osofsky; T F Lint
Journal:  J Clin Invest       Date:  1978-06       Impact factor: 14.808

10.  Complement activation in chronic liver disease.

Authors:  L E Munoz; D De Villiers; D Markham; K Whaley; H C Thomas
Journal:  Clin Exp Immunol       Date:  1982-03       Impact factor: 4.330
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