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PubMed1977

IgG deficiency in association with placental oedema.

Deficiency of the immunoglobulin C (IgG) in the human newborn is rare in the absence of maternal hypogammaglobulinaemia. Low concentrations of IgG in cord blood were found in 3 conditions--the donor twin in the fetofetal transfusion syndrome, hydrops fetalis and congenital hepatic disease. These were all associated with placental oedema. It is suggested that the oedema may be responsible for a disturbance in maternofetal placental transfer.

Dysgammaglobulinemia↗
PubMed1990

Selective IgG deficiency with a transcriptional disorder of the gamma switching region gene and the IL-4 gene.

A primary immunodeficiency patient was analysed whose serum IgG and IgE were extremely low but whose IgM and IgA levels were within the normal range or elevated. Southern blot analysis indicated no deletion of structural genes coding for C gamma, C epsilon, or C alpha. The majority of the patient's peripheral B cells expressed IgM and IgD on the surface yet IgG-positive B cells were not detected, suggesting that the defect is in a switch-recombination process from IgM to IgG. The RFLP pattern detected with the S mu and S gamma DNA regions revealed that there was no deletion or large mutation in the switch region DNA. An in vitro IgG production system with pokeweed mitogen showed an abnormality at the transcriptional level and the defects were in both the patient's T and B cells. Addition of recombinant IL-4 (rIL-4) to the normal B cells enhanced IgG production but the patient's B cells did not respond to rIL-4, although the IL-4 receptor was present at the normal level. Messenger RNA and IL-4 protein were not produced in the patient's T cells upon stimulation with phorbol ester and calcium ionophore, whereas IL-2 was normally produced. The patient's lymphocytes showed a proliferative response to various mitogens, including phorbol ester. The transcripts of unrearranged C gamma region genes were not detected in the patient's lymphocytes, suggesting that the chromatin structure of the S gamma region may not be open. These results suggest that the transcriptional defects at the S gamma region gene in B cells and at the IL-4 gene in the T cells may be responsible for the present IgG immunodeficiency. There might be a common transcriptional system operating in a certain step in the activation of both genes.

Adult↗
PubMedDate not supplied

Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency.

FIRST REPORT: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as IgA deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. Several immunologic evaluations were made between the ages of 4 months and 8 years. At 8 years and 9 months, the diagnosis of IgA deficiency was confirmed, and associated IgG2 and IgG4 deficiency (29.0 mg/dl y 0.01 mg/dl) with normal total IgG serum level was found. With the administration of intravenous gammaglobulin, the lung infections remitted and the subsequent clinical course has been uneventful up to now. SECOND REPORT: a boy with repeated infections since the age of 2 months. IgA deficiency was diagnosed at 1 year 7 months (undetectable serum IgA levels). At age 51/2 years, his clinical course worsened and more serious infections appeared. A new immunologic study revealed IgA deficiency associated with CD4 cell deficiency (432 cells/mm3) and normal CD3, CD19, and CD8 levels. Despite intensive antibiotic treatment and care, the child died. The findings suggest an association of IgA deficiency and common variable immunodeficiency.

Common Variable Immunodeficiency↗
PubMed1988

Immunoglobulin subclass deficiency.

IgG subclass deficiency was first noted in 1968. Subnormal levels of one or two, occasionally three IgG subclasses may be relatively common. It has not been determined, however, at what level below the normal range the IgG subclass deficiency is of clinical relevance. It remains important to clarify this point because certain subclass deficiencies may be without relevance of their own. Because patients with decreases of various IgG subclasses often present with a number of diseases, the low immunoglobulin levels may signify the presence of other abnormalities of more biologic significance. IgG subclass deficiency has been noted in about 25% of patients with well-defined food allergy and in patients with asthma, diabetes mellitus, Henoch-Schönlein's purpura, Bechterew's disease, intractable epilepsy of childhood, Friedreich's ataxia and autoimmune cytopenias. Most commonly they have increased frequency of infections especially in the respiratory tract, including sinusitis, otitis media and bronchopneumonia, but also osteomyelitis, meningitis, septicemia and various skin infections. Low levels of various subclasses have been noted in connection with other immunodeficiencies such as ataxia-telangiectasia. In common variable immunodeficiency there is an obvious imbalance in the IgG subclasses. Furthermore IgG subclass deficiency can be seen in relatives of patients with common variable immunodeficiency and in IgA deficiency. They also occur in relatives of patients with systemic lupus erythematosus, diabetes mellitus type 1 and C2 deficiency. In a few cases of subclass deficiency gene deletions have been shown. Subnormal levels of IgG subclasses make a remarkable change in sex distribution around puberty from 3/1 in boys and girls to the reverse sex ratio among adults.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

IgG subclass levels and southern analysis of DNA in primary immunodeficiency diseases including IgG subclass deficiency.

Serum IgG subclass levels are reported for twenty patients with primary immunodeficiency diseases, including four patients with IgG subclass deficiency, using an enzyme-linked immunosorbent assay with monoclonal antibodies. The disorder of each patient with IgG subclass deficiency seemed to be heterogeneous immunologically and clinically. One had complete IgG2-IgG4 deficiency, another had IgG2-IgG4-IgA deficiency and the other two had IgG2-IgA deficiencies. We did not find any structural-gene deletions in the constant region of the human immunoglobulin heavy-chain locus. Although the possibility of defects in genes controlling immunoglobulin expression, or small mutations, remains, these results suggest that IgG subclass deficiency is not always linked to particular structural-gene deletions.

Adolescent↗
PubMed1992

The association of IgA deficiency but not IgG or IgM deficiency with a reduced patient and graft survival following liver transplantation.

Recipients of solid organ allografts require lifelong immunosuppression in order to prevent graft rejection and to maintain graft function. In general, such immunosuppression greatly impairs the cellular immune system, as this level of the immune system is principally responsible for self and non-self recognition. The consequences of allograft transplantation in terms of patient and graft survival when transplants are given to individuals who have a preexisting humoral immune deficiency characterized by a deficiency of the serum levels of one or more of the major Ig classes have not yet been reported. From February 1, 1981 through December 31, 1990, a total of 43 adult patients with a deficiency of 1 or more Ig classes received a ABO-matched liver allograft at this institution. This sample represents 2.5% of a total of 1684 adults transplanted during this interval. These 43 liver graft recipients could be divided into 3 major groups based upon the presence of an IgG, IgM, or IgA deficiency. IgG deficiencies were defined as levels less than 50 mg/dl. Patient and graft survival for the IgA-deficient group was significantly reduced (P less than 0.04 and P less than 0.009, respectively) compared with both the IgG- and IgM-deficient groups. The latter two groups did not differ from controls without an Ig deficiency for these same two endpoints. The major causes of death in the IgA-deficient group were sepsis and opportunistic infection. A third of the deaths in the IgA-deficient group occurred in the perioperative period (first 30 days) while greater than 50% of the deaths occurred within the first 3 months, and all deaths occurred before the first year. Based upon these data, the following conclusions can be made: (1) serum IgA deficiency but not IgG or IgM deficiency is associated with an increased post-OLTx death and graft loss rate; (2) the majority of these deaths are due to sepsis or an opportunistic infection; and (3) most of the deaths occur early. These data suggest that recognition of a deficiency of IgA prior to organ grafting necessitates meticulous attention to the prevention of infection in the immediate perioperative period if patient and graft survival of these patients is to be improved.

Adult↗
PubMed1988

[Clinico-immunologic aspects of IgG subclass deficiency].

Serum IgG contains 4 subclasses, IgG1 (60-66%), IgG2 (20-30%), IgG3 (less than or equal to 5%) and IgG4. Individual subclasses vary with respect to their physicochemical and biological properties. IgG subclass concentrations in serum are age dependent. IgG1 and IgG3 reach near to adult levels around the age of 3, IgG2 and IgG4 after the age of 6. Antibodies of certain specificities generally belong to a certain isotype (subclass) due to the isotype restriction. Patients with subclass deficiencies often suffer from recurrent infections. Those with IgG2 deficiency (often occurring with IgA and IgG4 deficiency) develop recurrent infection of the upper and lower respiratory tract often caused by pyogenic microorganisms (Haemophilus, Pneumococcus). Since early initiation of IVIG substitution therapy has a beneficial effect on long term prognosis the importance of early diagnosis is apparent.

Child↗
PubMed1989

[Clinico-immunologic aspects of IgG subclass deficiency].

Serum IgG contains 4 subclasses, IgG1 (60-66%), IgG2 (20-30%), IgG3 (less than or equal to 5%) and IgG4. Individual subclasses vary with respect to their physicochemical and biological properties. IgG subclass concentrations in serum are age dependent. IgG1 and IgG3 reach near to adult levels around the age of 3, IgG2 and IgG4 after the age of 6. Antibodies of certain specificities generally belong to a certain isotype (subclass) due to the isotype restriction. Patients with subclass deficiencies often suffer from recurrent infections. Those with IgG2 deficiency coften occurring with IgA and IgG4 deficiency) develop recurrent infection of the upper and lower respiratory tract often caused by pyogenic microorganisms (Haemophilus, Pneumo-(occus). Since early initiation of IVIG substitution therapy has a beneficial effect on long term prognosis the importance of early diagnosis is apparent.

Adolescent↗
PubMed1993

Duodenal intraepithelial gamma/delta T cells and soluble CD8, neopterin, and beta 2-microglobulin in serum of IgA-deficient subjects with or without IgG subclass deficiency.

Expression of the gamma/delta T cell receptor (TCR) on CD3+ intraepithelial lymphocytes (IELs) was studied by two-colour immunofluorescence in duodenal tissue sections from healthy (n = 6) or infection-prone (n = 7) subjects with selective IgA deficiency (IgAD), and subjects (n = 4) with combined IgAD and IgG subclass deficiency. TCR gamma/delta+ IEL proportions in selective IgAD subjects (median 6.3%, range 1.0-41%) and in those with combined deficiency (median 4.5%, range 1.2-33%) were well within the range (0.3-38%) for histologically normal controls (n = 11), but the healthy IgAD subgroup tended to show raised TCR gamma/delta+ IEL proportions (median 13.6%) compared with the other two subgroups. Also the number of TCR gamma/delta+ IELs per intestinal length unit was relatively high (median 13.9/mm) in the healthy IgAD subjects, and significantly raised (P < 0.03) compared with controls (median 3.2/mm). Paired staining revealed that most TCR gamma/delta+ IELs in both selective IgAD (98%) and combined deficiency (99%) were CD8-, and a large fraction (median 84% and 63%, respectively) expressed the V delta 1/J delta 1-encoded epitope. The total number of CD3+ IELs (mostly CD8+) was similar to controls. IgAD subjects, and especially the healthy subgroup, had significantly increased serum concentrations of soluble CD8 (P < 0.0002), neopterin (P < 0.005), and beta 2-microglobulin (P < 0.007), which was similar to our previous observations in common variable immunodeficiency, and probably reflected stimulation of cell-mediated immunity. In addition, the increased TCR gamma/delta+ IELs might reflect a component of compensatory surface protection in the healthy IgAD subgroup.

Adolescent↗