Selective Gamma-G Globulin Deficiencies in Patients with Recurrent Pyogenic Infections

Schur, Peter H.; Borel, Halina; Gelfand, Erwin W.; Alper, Chester A.; Rosen, Fred S.

doi:10.1056/nejm197009172831205

Cited by 233 publications

(53 citation statements)

References 10 publications

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“…No long-term sequelae have been identified. Schur et al (1970) first described IgG subclass deficiencies in three adult patients. Since then numerous publications have identified subclass deficiencies in many patients, particularly children.…”

Section: Prognosismentioning

confidence: 99%

The Four Most Common Pediatric Immunodeficiencies

Stiehm

2008

Journal of Immunotoxicology

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The four most common immunodeficiencies in pediatric patients are transient hypogamma-globulinemia of infancy, IgG subclass deficiency, impaired polysaccharide responsiveness (partial antibody deficiency) and selective IgA deficiency. Most of these patients have normal cellular immune systems, phagocyte function and complement levels. All four illnesses are characterized by recurrent bacterial respiratory infections such as purulent rhinitis, sinusitis, otitis and bronchitis. Except for some IgA-deficient patients, the molecular basis for these illnesses is not known, and indeed each syndrome is heterogeneous, with multiple causes, including genetic factors, drug/environmental toxicant exposure, and/or prenatal physiological events. This paper describes the clinical and laboratory features, postulated causes, current management and prognosis. Only a few of these cases require the use of intravenous IgG (IVIG) and the outlook for long life is excellent.

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Section: Prognosismentioning

confidence: 99%

The Four Most Common Pediatric Immunodeficiencies

Stiehm

2008

Journal of Immunotoxicology

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“…Similarly, not all laboratories that can quantify IgG subclasses achieve consistent standards of quality. There is no international standard for IgG subclasses quantitation and inter-laboratory coe¤cients of variation of up to 62% for IgG 4 and up to 32% for IgG 3 have been observed by the UK National External Quality Assessment Scheme.…”

Section: Measurement Of Speci C Antibodies To Bacterial Proteins and mentioning

confidence: 99%

Are there any clinical indications for measuring IgG subclasses?

2002

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It is questionable as to whether a low serum concentration of one of the IgG subclasses identi es a disease state. A low IgG 1 concentration is found in primary or secondary immunode ciency states but does not occur in isolation. Low IgG 2 concentration is associated with an increased risk of bacterial infections but only in some individuals and not in others. Isolated IgG 3 and IgG 4 de ciency have not been convincingly demonstrated. Therefore, the isolated nding of low concentrations of one or more IgG subclass does not identify individuals at risk. In contrast, the nding of low serum concentrations of antibodies to speci c bacterial antigens (Haemophilus in uenzae type B, pneumococcus, tetanus and diphtheria) does identify individuals at risk and these measurements should be used in preference to IgG subclass measurement.

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“…Deficiency of IgG2 has been reported in association with Haemophilus influenzae infection (Schur et al 1970), meningococcal infection (Bass et al 1983), the cartilage-hair syndrome (Oxelius 1979), ataxia telangiectasia (Oxelius et al 1982) and children with suspected acquired immunodeficiency syndrome (Church et al 1982) (Siber et al 1980). Recurrences of eczema herpeticum are sometimes seen in children with atopic eczema, but the severity of the recurrences in this patient is atypical.…”

Section: Discussionmentioning

confidence: 78%