The Four Most Common Pediatric Immunodeficiencies

Stiehm, Richard

doi:10.1007/978-0-387-72005-0_2

Cited by 52 publications

(36 citation statements)

References 22 publications

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“…A ntibody production defects are the most common category of pediatric primary immunodeficiencies (PIDs) (1,2). The hallmark of these immunodeficiency conditions is the defective production of antigen-specific antibodies that are an indispensable element of the adaptive immune response to pathogens (3,4).…”

mentioning

confidence: 99%

Impaired Antigen-Specific Immune Response to Vaccines in Children with Antibody Production Defects

Szczawińska-Popłonyk

Bręborowicz

Samara

et al. 2015

Clin Vaccine Immunol

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The impaired synthesis of antigen-specific antibodies, which is indispensable for an adaptive immune response to infections, is a fundamental pathomechanism that leads to clinical manifestations in children with antibody production defects. The aim of this study was to evaluate the synthesis of antigen-specific antibodies following immunization in relation to peripheral blood B cell subsets in young children with hypogammaglobulinemia. Twenty-two children, aged from 8 to 61 months, with a deficiency in one or more major immunoglobulin classes participated in the study. Postvaccination antibodies against tetanus and diphtheria toxoids, the surface antigen of the hepatitis B virus, and the capsular Haemophilus influenzae type b polysaccharide antigen were assessed along with an immunophenotypic evaluation of peripheral blood B lymph cell maturation. A deficiency of antibodies against the tetanus toxoid was assessed in 73% of cases and that against the diphtheria toxoid was assessed in 68% of cases, whereas a deficiency of antibodies against the surface antigen of the hepatitis B virus was revealed in 59% of the children included in the study. A defective response to immunization with a conjugate vaccine with the Haemophilus influenzae type b polysaccharide antigen was demonstrated in 55% of hypogammaglobulinemic patients. Increased proportions of transitional B lymph cells and an accumulation of plasmablasts accompanied antibody deficiencies. The defective response to vaccine protein and polysaccharide antigens is a predominating disorder of humoral immunity in children with hypogammaglobulinemia and may result from a dysfunctional state of the cellular elements of the immune system. A ntibody production defects are the most common category of pediatric primary immunodeficiencies (PIDs) (1, 2). The hallmark of these immunodeficiency conditions is the defective production of antigen-specific antibodies that are an indispensable element of the adaptive immune response to pathogens (3, 4). While the poor response to vaccines is another feature of humoral PIDs, the ability to synthesize postvaccination antibodies against toxoids and polysaccharides is the most specific expression of the immune response to antigens. In the evaluation of the immune response associated with antibody production, the response to vaccination against hepatitis B is not routinely recommended because of the large proportion of adults, up to between 1% and 3% of vaccinated individuals, who do not effectively synthesize antibodies against hepatitis B virus surface antigen (HBs). In infants and children, the efficiency of recombinant vaccines against hepatitis B, assessed on the basis of postvaccination anti-HBs antibody concentration over 10 mIU/ml, is estimated at 85% to 100% (5, 6). The minimal protective level of neutralizing antibodies against diphtheria and tetanus toxoids has been estimated at 0.01 to 0.1 IU/ml, whereas to achieve long-term immunity, a concentration of specific antibodies, up to 1.0 IU/ml, may be required. The synthesis of...

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confidence: 99%

Impaired Antigen-Specific Immune Response to Vaccines in Children with Antibody Production Defects

Szczawińska-Popłonyk

Bręborowicz

Samara

et al. 2015

Clin Vaccine Immunol

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“…Brazil reported 50 cases of deficiency complement, followed by 13 reports of Argentina (Leiva et al, 2007). Like others studies, predominantly antibody deficiency was the principal PID observed in Latin America, Australia (Baumgart et al, 1997;Kirkpatrick et al, 2007), China (Zhao et al, 2006;Wang et al, 2011), Egypt (Reda et al, 2009), French (CEREDIH, 2010, Hong Kong (Lam et al, 2005), Iran (Aghamohammadi et al, 2002;Farhoudi et al, 2005;Rezaei et al, 2006), Italy (Luzi et al, 1983), Kuwait (AlHerz, 2008), Netherland (Zegers et al, 1994), Norway (Stray-Pedersen et al, 2000), Poland (Bernatowska et al, 1998), Republic of Ireland (Abuzakouk et al, 2005), Spain (Matamoros et al, 1997;Milá et al, 2001), Swiss (Ryser et al, 1998), Taiwan (Lee et al, 2011), Thailand (Benjasupattananan et al, 2009), Tunisia (Bejaoui et al, 1997) and USA (Javier et al, 2000;Stiehm, 2007), Studies in other countries reveled major number of granulocyte dysfunction in India (Verma et al, 2008), and Combined T-and B-cell Immunodeficiency in Turk (Shabestari et al, 2007).…”

Section: Latin American Group Of Immunodeficiencymentioning

confidence: 99%

Primary Immunodeficiency Diseases in Latin America: Epidemiology and Perspectives

Errante¹,

Condino‐Neto²

2012

Epidemiology Insights

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“…In addition, IgG subclass deficiency is among the more common immunodeficiencies in pediatric populations, resulting in increased rates of particular infections (18,19).…”

Section: Introductionmentioning

confidence: 99%