Recurrent Sepsis With Deficiencies of C2 and Galactokinase

Michael S. Borzy, Lawrence Wolff, Anita Gewurz, Neil R M Buist, Everett Lovrien

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

A 4-year-old girl with recurrent, severe bacterial infections and absence of both the second component of complement and galactokinase was investigated for immunodeficiency. The C2 deficiency (C2D) was diagnosed after four major pyogenic infections. Results of studies of cellular and humoral immunity were normal, as were polymorphonuclear leukocyte chemotaxis and bactericidal activities and alternative-pathway hemolytic activity. Serum chemotactic and opsonic activities were deficient in this patient and in an older, asymptomatic sibling with C2D. Fresh-frozen plasma, administered during an episode of Streptococcus pneumoniae meningitis, enhanced serum opsonic activity at 12 hours after infusion. To our knowledge, this is the first description of C2D in a patient with a documented second, unusual genetic defect.

Original languageEnglish (US)
Pages (from-to)186-191
Number of pages6
JournalAmerican Journal of Diseases of Children
Volume138
Issue number2
DOIs
StatePublished - 1984
Externally publishedYes

Fingerprint

Galactosemias
Sepsis
Galactokinase
Leukocyte Chemotaxis
Pneumococcal Meningitis
Humoral Immunity
Serum
Bacterial Infections
Cellular Immunity
Siblings
Neutrophils
Infection

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Recurrent Sepsis With Deficiencies of C2 and Galactokinase. / Borzy, Michael S.; Wolff, Lawrence; Gewurz, Anita; Buist, Neil R M; Lovrien, Everett.

In: American Journal of Diseases of Children, Vol. 138, No. 2, 1984, p. 186-191.

Research output: Contribution to journalArticle

Borzy, Michael S. ; Wolff, Lawrence ; Gewurz, Anita ; Buist, Neil R M ; Lovrien, Everett. / Recurrent Sepsis With Deficiencies of C2 and Galactokinase. In: American Journal of Diseases of Children. 1984 ; Vol. 138, No. 2. pp. 186-191.
@article{3491b9d056644f27b24a155965e11bf8,
title = "Recurrent Sepsis With Deficiencies of C2 and Galactokinase",
abstract = "A 4-year-old girl with recurrent, severe bacterial infections and absence of both the second component of complement and galactokinase was investigated for immunodeficiency. The C2 deficiency (C2D) was diagnosed after four major pyogenic infections. Results of studies of cellular and humoral immunity were normal, as were polymorphonuclear leukocyte chemotaxis and bactericidal activities and alternative-pathway hemolytic activity. Serum chemotactic and opsonic activities were deficient in this patient and in an older, asymptomatic sibling with C2D. Fresh-frozen plasma, administered during an episode of Streptococcus pneumoniae meningitis, enhanced serum opsonic activity at 12 hours after infusion. To our knowledge, this is the first description of C2D in a patient with a documented second, unusual genetic defect.",
author = "Borzy, {Michael S.} and Lawrence Wolff and Anita Gewurz and Buist, {Neil R M} and Everett Lovrien",
year = "1984",
doi = "10.1001/archpedi.1984.02140400068017",
language = "English (US)",
volume = "138",
pages = "186--191",
journal = "JAMA Pediatrics",
issn = "2168-6203",
publisher = "American Medical Association",
number = "2",

}

TY - JOUR

T1 - Recurrent Sepsis With Deficiencies of C2 and Galactokinase

AU - Borzy, Michael S.

AU - Wolff, Lawrence

AU - Gewurz, Anita

AU - Buist, Neil R M

AU - Lovrien, Everett

PY - 1984

Y1 - 1984

N2 - A 4-year-old girl with recurrent, severe bacterial infections and absence of both the second component of complement and galactokinase was investigated for immunodeficiency. The C2 deficiency (C2D) was diagnosed after four major pyogenic infections. Results of studies of cellular and humoral immunity were normal, as were polymorphonuclear leukocyte chemotaxis and bactericidal activities and alternative-pathway hemolytic activity. Serum chemotactic and opsonic activities were deficient in this patient and in an older, asymptomatic sibling with C2D. Fresh-frozen plasma, administered during an episode of Streptococcus pneumoniae meningitis, enhanced serum opsonic activity at 12 hours after infusion. To our knowledge, this is the first description of C2D in a patient with a documented second, unusual genetic defect.

AB - A 4-year-old girl with recurrent, severe bacterial infections and absence of both the second component of complement and galactokinase was investigated for immunodeficiency. The C2 deficiency (C2D) was diagnosed after four major pyogenic infections. Results of studies of cellular and humoral immunity were normal, as were polymorphonuclear leukocyte chemotaxis and bactericidal activities and alternative-pathway hemolytic activity. Serum chemotactic and opsonic activities were deficient in this patient and in an older, asymptomatic sibling with C2D. Fresh-frozen plasma, administered during an episode of Streptococcus pneumoniae meningitis, enhanced serum opsonic activity at 12 hours after infusion. To our knowledge, this is the first description of C2D in a patient with a documented second, unusual genetic defect.

UR - http://www.scopus.com/inward/record.url?scp=0021357686&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0021357686&partnerID=8YFLogxK

U2 - 10.1001/archpedi.1984.02140400068017

DO - 10.1001/archpedi.1984.02140400068017

M3 - Article

VL - 138

SP - 186

EP - 191

JO - JAMA Pediatrics

JF - JAMA Pediatrics

SN - 2168-6203

IS - 2

ER -