Letter to the editor: Humoral immunodeficiencies in Down syndrome: Serum IgG subclass and antibody response to hepatitis B vaccine

Avanzini, Maria Antonietta; Monafò, V.; Amici, Mara De; Burgio, Giuseppe; Plebani, Alessandro; Ugazio, Alberto G.; Hanson, Lars Å.

doi:10.1002/ajmg.1320370746

Cited by 24 publications

(22 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The latter has indeed been described (3,15). However, the T-cell-independent antibody response to pneumococcal polysaccharide antigen is also decreased in DS (4), suggesting an intrinsic B-lymphocyte defect is also present.…”

Section: Own Syndrome (Ds) Is Associated With Recurrent-mainlymentioning

confidence: 56%

“…Deficient T-lymphocyte help could lead to disturbed B-lymphocyte activation and proliferation. Despite the B lymphocytopenia, a considerable hypergammaglobulinemia of IgA and IgG after the age of 5 y, with high levels of IgG 1 and IgG 3 and low levels of IgG 2 and IgG 4 , is described (3,15,16).…”

Section: Own Syndrome (Ds) Is Associated With Recurrent-mainlymentioning

confidence: 99%

“…respiratory-infections (1,2), decreased responses to vaccination (3)(4)(5)(6)(7)(8), a higher frequency of hepatitis B surface antigen carriers (3), and autoimmune diseases such as celiac disease and hypothyroidism (9 -11). These features are suggestive of immunodeficiency.…”

Section: Own Syndrome (Ds) Is Associated With Recurrent-mainlymentioning

confidence: 99%

See 2 more Smart Citations

Down Syndrome B-Lymphocyte Subpopulations, Intrinsic Defect or Decreased T-Lymphocyte Help

et al. 2010

View full text Add to dashboard Cite

Down syndrome (DS) is known for increased incidence of respiratory infections and autoimmune diseases, indicating impaired immunity. Until now, attention has been mainly focused on T lymphocytes. Therefore, we determined B-lymphocyte subpopulations in 95 children with DS compared with 33 age-matched control (AMC) children. DS serum immunoglobulin levels were compared with 962 non-DS children with recurrent infections. The results were combined with clinical data. Transitional and naive B lymphocytes are profoundly decreased in the children with DS. This could be caused by an intrinsic B-lymphocyte defect resulting in (partial) failure of B-lymphocyte generation, decreased antigen-induced proliferation and/or increased apoptosis, or by decreased proliferation due to deficient T-lymphocyte help, or a combination of these. The decreased CD27ϩ , CD21 high , and CD23 ϩ cells are reminiscent of common variable immunodeficiency and suggestive of disturbed peripheral B-lymphocyte maturation. Immunoglobulin levels in DS are abnormal-as has been described before-and different from non-DS children with recurrent infections. We conclude that the humoral immune system is abnormal in DS, but could not find a relation between B-lymphocyte subsets, immunoglobulins and clinical features of the children with DS in our cohort, nor could we answer the question whether DS lymphocytes are truly intrinsically deficient, or could all findings be explained by deficient Tlymphocyte help. (Pediatr Res 67: 563-569, 2010)

show abstract

Section: Own Syndrome (Ds) Is Associated With Recurrent-mainlymentioning

confidence: 56%

Section: Own Syndrome (Ds) Is Associated With Recurrent-mainlymentioning

confidence: 99%

See 1 more Smart Citation

Down Syndrome B-Lymphocyte Subpopulations, Intrinsic Defect or Decreased T-Lymphocyte Help

et al. 2010

View full text Add to dashboard Cite

show abstract

“…+ monocytes (14), changes in Tand B-lymphocyte counts (15)(16)(17), early aging of the immune system (18,19), an intrinsic defect of T and B lymphocytes (16,20,21), IgG2 and IgG4 subclass deficiencies (16,17,(21)(22)(23)(24), impaired antibody response to pneumococcal vaccine (25), and diminished invariant natural killer T cells (14,17) and regulatory T cells (17). These lower RTIs in DS children are most often caused by viral pathogens, such as respiratory syncytial virus.…”

mentioning

confidence: 99%

Increased production of interleukin-10 in children with Down syndrome upon ex vivo stimulation with Streptococcus pneumoniae

et al. 2013

View full text Add to dashboard Cite

“…Diferentes estudos têm demonstrado que pacientes com SD apresentam várias disfunções imunológicas 2,3 , que os predispõem a uma maior freqüência de infecções recorrentes, assim como de doenças autoimunes. Vários estudos já foram publicados relatando alto grau de associação entre SD e doenças autoimunes [4][5][6] , principalmente doença celía-ca (DC) [7][8][9][10][11] e tireoidite autoimune 12,13 .…”

Section: Introductionunclassified

Celiac disease in children and adolescents with Down syndrome

2005

J Pediatr (Rio J)

View full text Add to dashboard Cite

Objectives: High prevalence rates of celiac disease in patients with Down syndrome have been reported in several countries. However, in Brazil there are no data regarding this association. In this study we report the prevalence of celiac disease in Down syndrome children and adolescents from southern Brazil.Methods: Seventy-one patients (32 female and 39 male, 2-18 years) from Curitiba, Brazil, were studied. Eighty young people (42 male and 38 female, 2-19 years) were used as controls. All subjects were screened for the IgA-antiendomysium antibody (EmA) and IgA anti-tecidual transglutaminase (anti-tTG). EmA was measured by an immunofluorescence assay using umbilical cord as the substrate and anti-tTG by ELISA with tecidual transglutaminase as the antigen. The total IgA serum level was determined by turbidimetry. ResumoObjetivos: Alta prevalência de doença celíaca em pacientes com síndrome de Down tem sido descrita em vários países. No entanto, no Brasil ainda não há relatos mostrando essa associação. O presente estudo teve como objetivo avaliar a prevalência de doença celíaca em crianças e adolescentes com síndrome de Down no sul do Brasil.Métodos: Setenta e um pacientes (32 do sexo feminino e 39 masculino, 2-18 anos) provenientes de Curitiba, Brasil, foram estudados. Oitenta indivíduos (42 do sexo masculino e 38 feminino, 2-19 anos) foram utilizados como controles do estudo. Todas as amostras foram investigadas para anticorpo anti-endomísio (EmA) e anti-transglutaminase tecidual (anti-tTG). O EmA foi pesquisado através de imunofluorescência indireta usando cordão umbilical como substrato e o anti-tTG através da técnica de ELISA, utilizando transglutaminase tecidual como antígeno. As dosagens de IgA foram realizadas por turbidimetria.Resultados: Cinco pacientes com síndrome de Down (7%) foram positivos para EmA-IgA, com títulos entre 1/5 to 1/80 e catorze (17,5%) para anti-tTG (21-340 units). Todos os pacientes positivos para EmA apresentaram simultaneamente positividade para o anti-tTG. Os achados clínicos e histológicos na mucosa intestinal confimaram doença celíaca em quatro pacientes. O outro paciente EmA positivo não foi submetido a biópsia duodenal. Os pacientes positivos apenas para anti-tTG apresentaram valores limítrofes (< 25 unidades) e eram assintomáticos. Nenhum indivíduo do grupo controle foi positivo para EmA ou anti-tTG. Nenhuma amostra do estudo foi deficiente para IgA.Conclusões: Os dados do presente estudo mostram alta prevalên-cia (5,6%) de doença celíaca confirmada em crianças e adolescentes com síndrome de Down da região sul do Brasil.J Pediatr (Rio J). 2005;81(5):373-6: Anticorpos anti-endomísio, anticorpos anti-transglutaminase, doença celíaca, síndrome de Down.

show abstract

Letter to the editor: Humoral immunodeficiencies in Down syndrome: Serum IgG subclass and antibody response to hepatitis B vaccine

Cited by 24 publications

References 7 publications

Down Syndrome B-Lymphocyte Subpopulations, Intrinsic Defect or Decreased T-Lymphocyte Help

Down Syndrome B-Lymphocyte Subpopulations, Intrinsic Defect or Decreased T-Lymphocyte Help

Increased production of interleukin-10 in children with Down syndrome upon ex vivo stimulation with Streptococcus pneumoniae

Celiac disease in children and adolescents with Down syndrome

Contact Info

Product

Resources

About