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Properdin deficiency in a boy with fulminant meningococcal septic shock

Genel, Ferah; Atlihan, Fusun; Gulez, Nesrin; Sjöholm, Anders LU ; Melander Skattum, Lillemor LU and Truedsson, Lennart LU (2006) In Acta Pædiatrica 95(11). p.1498-1500
Abstract
Bacterial meningitis is a rare presentation for congenital immunodeficiency, but meningococcal invasive diseases and meningitis have been associated with late complement component deficiencies and properdin deficiency. A 5(1)/(2)-y-old boy of non-consanguineous parents was admitted to our hospital with meningococcal septic shock. He had previously been suffering from recurrent respiratory infections. His 13-y-old brother had also been treated for meningococcal meningitis when he was 7 y old. Immunological studies, done after recovery, on the patient and his two brothers revealed normal immunoglobulin, IgG subclasses, C3, C4 and CH50 levels. Haemolytic activity of the alternative complement pathway could not be detected, and properdin... (More)
Bacterial meningitis is a rare presentation for congenital immunodeficiency, but meningococcal invasive diseases and meningitis have been associated with late complement component deficiencies and properdin deficiency. A 5(1)/(2)-y-old boy of non-consanguineous parents was admitted to our hospital with meningococcal septic shock. He had previously been suffering from recurrent respiratory infections. His 13-y-old brother had also been treated for meningococcal meningitis when he was 7 y old. Immunological studies, done after recovery, on the patient and his two brothers revealed normal immunoglobulin, IgG subclasses, C3, C4 and CH50 levels. Haemolytic activity of the alternative complement pathway could not be detected, and properdin concentrations were < 0.01 mg/l in serum samples from the patient and his brothers. The patient and family members received quadrivalent polysaccharide meningococcal vaccine. The patient was discharged on penicillin prophylaxis, and he remained healthy during the ensuing year. Conclusion: Our findings stress that measurement of the haemolytic activity of the alternative complement pathway in addition to classical pathway haemolytic complement activity should be performed in patients with meningococcal disease to reveal various forms of complement deficiency. This is particularly important when there is a family history, or recurrences or infection due to uncommon serogroups. Deficient individuals and affected family members might be protected from infection by vaccination. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
meningococcal disease, complement, family history
in
Acta Pædiatrica
volume
95
issue
11
pages
1498 - 1500
publisher
Wiley-Blackwell Publishing Ltd
external identifiers
  • wos:000241520400030
  • scopus:33750408201
ISSN
1651-2227
DOI
10.1080/08035250600603008
language
English
LU publication?
yes
id
c05ea1d2-b56a-4601-afc3-72ea09dfcce5 (old id 387184)
date added to LUP
2007-10-03 08:43:08
date last changed
2019-01-06 10:41:26
@article{c05ea1d2-b56a-4601-afc3-72ea09dfcce5,
  abstract     = {Bacterial meningitis is a rare presentation for congenital immunodeficiency, but meningococcal invasive diseases and meningitis have been associated with late complement component deficiencies and properdin deficiency. A 5(1)/(2)-y-old boy of non-consanguineous parents was admitted to our hospital with meningococcal septic shock. He had previously been suffering from recurrent respiratory infections. His 13-y-old brother had also been treated for meningococcal meningitis when he was 7 y old. Immunological studies, done after recovery, on the patient and his two brothers revealed normal immunoglobulin, IgG subclasses, C3, C4 and CH50 levels. Haemolytic activity of the alternative complement pathway could not be detected, and properdin concentrations were &lt; 0.01 mg/l in serum samples from the patient and his brothers. The patient and family members received quadrivalent polysaccharide meningococcal vaccine. The patient was discharged on penicillin prophylaxis, and he remained healthy during the ensuing year. Conclusion: Our findings stress that measurement of the haemolytic activity of the alternative complement pathway in addition to classical pathway haemolytic complement activity should be performed in patients with meningococcal disease to reveal various forms of complement deficiency. This is particularly important when there is a family history, or recurrences or infection due to uncommon serogroups. Deficient individuals and affected family members might be protected from infection by vaccination.},
  author       = {Genel, Ferah and Atlihan, Fusun and Gulez, Nesrin and Sjöholm, Anders and Melander Skattum, Lillemor and Truedsson, Lennart},
  issn         = {1651-2227},
  keyword      = {meningococcal disease,complement,family history},
  language     = {eng},
  number       = {11},
  pages        = {1498--1500},
  publisher    = {Wiley-Blackwell Publishing Ltd},
  series       = {Acta Pædiatrica},
  title        = {Properdin deficiency in a boy with fulminant meningococcal septic shock},
  url          = {http://dx.doi.org/10.1080/08035250600603008},
  volume       = {95},
  year         = {2006},
}