Immunoglobulin a Deficiency 1

Last update 01 Nov 2024

Basic Info

Synonyms

GAMMA-A-GLOBULIN, SELECTIVE DEFICIENCY OF, Gamma-a-globulin, selective deficiency of, IGAD1

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Introduction

Decreased or absent levels of serum immunoglobulin A, with normal serum levels of immunoglobulin G and immunoglobulin M in a patient who is older than 4 years of age and in whom all other causes of hypogammaglobulinemia have been excluded. Affected individuals may be asymptomatic or have frequent infections, allergic reactions, or autoimmune disorders.

100 Clinical Results associated with Immunoglobulin a Deficiency 1

100 Translational Medicine associated with Immunoglobulin a Deficiency 1

0 Patents (Medical) associated with Immunoglobulin a Deficiency 1

Literatures (Medical) associated with Immunoglobulin a Deficiency 1

15 Aug 1999·Journal of immunology (Baltimore, Md. : 1950)

A putative susceptibility locus on chromosome 18 is not a major contributor to human selective IgA deficiency: evidence from meiotic mapping of 83 multiple-case families.

Article

Author: Blennow, E ; Vorechovský, I ; Hammarström, L ; Nordenskjöld, M ; Webster, A D

Previous reports of an association between constitutional chromosome 18 abnormalities and low levels of IgA suggested that this chromosome contains a susceptibility locus for selective IgA deficiency (IgAD), the most frequent Ig deficiency in humans. IgAD is genetically related to common variable immunodeficiency (CVID), characterized by a lack of additional isotypes. Our previous linkage analysis of 83 multiple-case IgAD/CVID families containing 449 informative pedigree members showed a significantly increased allele sharing in the chromosome region 6p21 consistent with allelic associations in family-based and case-control studies and provided the evidence for a predisposing locus, termed IGAD1, in the proximal part of the MHC. We have typed the same family material at 17 chromosome 18 marker loci with the average intermarker distance of 7 cM. A total of 7633 genotypes were analyzed in a nonparametric linkage analysis, but none of the marker loci exhibited a significantly increased allele sharing in affected family members. In addition, reverse painting and deletion mapping of a panel of constitutional chromosome 18 deletions/translocations showed the presence of IgA-deficient and IgA-proficient patients with the same abnormality and did not reveal a region commonly deleted. The linkage analysis of chromosome 8 and 21 regions involved in reciprocal translocations t(8;18) and t(18;21), which were identified in two patients lacking IgA, did not disclose a significant allele sharing. Although these results do not exclude the presence of a minor predisposing locus on this chromosome, such a putative locus would confer a population risk of developing IgAD/CVID much lower than IGAD1.

01 Jan 1998·Terapevticheskii arkhivQ4 · MEDICINE

[Common variable immunodeficiency in adults and problems of its immunotherapy].

Q4 · MEDICINE

ArticleOA

Author: Malashenkova, E V ; Varfolomeeva, M I ; Borisova, A M ; Setdikova, N Kh ; Kaliazina, V A

AIMClinicoimmunological study of adult patients with prevailing defect of humoral immunity, development of diagnostic criteria and treatment of this disease.MATERIALS AND METHODSClinical, immunological and microbiological examinations were made in 68 patients with defects of antibody formation.RESULTSTotal variable immunodeficiency (TVID), selective deficiency of IgA, congenital agammaglobulinemia, hyper-IgM-syndrome were detected in 74, 13, 10 and 3% of patients, respectively. TVID was frequently associated with respiratory, chronic ENT and gastrointestinal diseases, low CD4+ and high CD8+ levels. The response was achieved with combined therapy: antibacterial treatment + immunocorrection + differentiated replacement with plasma or immunoglobulins.CONCLUSIONIn choice of immunotherapy of TVID patients it is necessary to consider parameters of immunogram. Intravenous administration of immunoglobulin preparations provided the highest effect in good safety.

01 Mar 1993·Boletin medico del Hospital Infantil de Mexico

[Selective IgA deficiency and malabsorption. Review of the literature apropos of a case].

Review

Author: García-Aranda, J A ; García-Tamayo, F ; Faure-Fontenla, M A ; Torres-Alcántara, S

One of the most common primary immunodeficiencies is the selective deficiency of IgA, which is present in very variable clinical conditions. This situation had conducted to different criteria with respect to the clinical meaning in a particular patient. The association of immunodeficiency of IgA and intestinal malabsorption is reported as statistically significant in the international literature. Nevertheless, the precise pathogenic connection between them is unknown. The relationship between bowel and immune system is reviewed here, and illustrated with the clinical case of a six-year old girl who presented with malabsorption and selective deficiency of IgA.

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Immunoglobulin a Deficiency 1

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