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PubMedDate not supplied

Severe steroid-dependent asthma with IgG-2 deficiency and recurrent sinusitis: response to treatment with high-dose intravenous immunoglobulin.

Patients with severe asthma pose a dilemma to the physician since the treatment they need, namely high doses of oral steroids, has serious side effects, especially among the pediatric population. Deficiency in one or more of the IgG subclasses has been associated with abnormal pulmonary function, as well as with recurrent sinopulmonary infections in adults and children. In the last years attention has been focused on alternative therapies for these patients. One of these alternatives is the treatment with intravenous immunoglobulin (IVIG). We report an 11-year-old boy with severe asthma since the age of two years and multiple hospital admissions due to asthmatic crisis even more frequent and severe, to the point of needing, in the last year, daily treatment with high doses of oral steroids (20 mg). During six months the patient was given high doses of intravenous immunoglobulin. After one month of treatment a clinical and spirometric improvement was apparent allowing to taper down the oral steroids until their complete substitution by inhaled budesonide (1,600 microg/day). The only side effects noted were severe headaches after gammaglobulin infusions which responded well to oral paracetamol. This improvement was sustained throughout the treatment period, but few weeks after the IVIG was suspended the clinical and spirometric parameters started to worsen again.

Asthma↗
PubMed1990

Lack of the G2m(n) allotype in IgG subclass deficiency, in IgG2 deficiency together with lack of G1m(a) and G3m(g), and in IgG3 deficiency together with lack of G1m(f) and G3m(b).

Lack of G2m(n) was demonstrated in both IgG2-deficient and IgG3-deficient Caucasian patients. Lack of G2m(n) or G2m(",") was found together with homozygosity for both G1m and G3m allotypes as the dominant finding, i.e. for IgG2-deficient patients together with G1m (f,f) and G3m(b,b), constituting the Gm(f,",b) phenotype, and for IgG3-deficient patients together with G1m(a,a) and G3m(g,g), constituting the Gm(a,",g) phenotype. The group with IgG2 deficiency and the selected patients with the Gm(f,",b) phenotype expressed characteristically very low or undetectable IgG4, significantly increased IgG3, and normal IgG1. The group with IgG3 deficiency and the selected patients with the phenotype Gm(a,",g) expressed instead normal IgG4 and nearly normal IgG2 and IgG1 levels. The lack of G2m(n) together with lack of one or the other of the alternative G1m genes and corresponding G3m genes give different IgG2 levels and different IgG subclass patterns. The frequency of G1m allotypes and corresponding G3m allotypes also deviated significantly when the IgG2 deficiency and IgG3 deficiency groups were compared with each other. Most IgG subclass-deficient patients are homozygous in the Gm system and lack genetic variants in the three IgG subclasses, IgG1, IgG2, and IgG3.

Antibodies, Monoclonal↗
PubMed1977

Effect of protein A on staphylococcal opsonization.

To study the effect of wall protein A on bacterial opsonization, phagocytosis of 10 strains of Staphylococcus aureus with high and low protein A contents was measured. Those strains that contained the highest concentrations of protein A were phagocytized by human neutrophils at a slower rate than strains with little or no protein A when normal human serum and purified immunoglobulin G (IgG) were used as opsonic sources. When IgG-deficient serum was used as an source, however, protein A-rich strains were phagocytized more rapidly than protein A-deficient strains. Extracellular (purified) protein A decrease the opsonic activity of all sera tested including IgG-deficient serum. It is proposed that when IgG is not present in the opsonic medium, cell wall protein A is capable of activating complement at the bacterial surface and thereby opsonization is promoted.

Bacterial Proteins↗
PubMed2006

Immunoglobulin induction therapy in renal transplant recipients: Effects on immunoglobulin and regulatory antibody levels.

We have previously shown that high pretransplant regulatory autoantibodies are associated with better kidney graft outcome. To analyze the effect of intravenous immunoglobulin (IVIG) induction therapy on these regulatory antibodies, we performed a prospective randomized study in 50 renal transplant recipients who were randomly assigned to receive 7 x 10 g IVIG or 7 x 10 g IV albumin infusions. Basic immunosuppressive therapy consisted of tacrolimus/azathioprine (n = 24) and tacrolimus/mycophenolate mofetil (n = 26), respectively. ELISA was used to assess IgG-/IgA-anti-Fab, -anti-F(ab)2 and -anti-hinge regulatory antibodies. IVIG induction therapy resulted in upregulation of serum IgG and IgA levels within the first 20 days posttransplant (P = .001, IgG; P = .04, IgA), so that a significant IgG deficiency was found only in non-IVIG patients (day 10: IgG <6 g/L: 7/25 (28%) non-IVIG versus 0/25 IVIG patients; P = .005). As the IVIG charges contained all of the regulatory antibodies tested, intravenous administration of these antibodies explain the elevated IgG- and IgA-anti-F(ab)2 antibody levels found in IVIG compared to non-IVIG patients on day 10 (P = .005 and P = .04, respectively). Our data indicated that IVIG induction prevented severe IgG deficiency in the early posttransplant period but had no impact on severe infectious complications. IVIG induction enhanced immunoregulatory antibody levels early posttransplant, which might provide graft protective effects.

Azathioprine↗
PubMed1994

Influence of iron deficiency on serum IgG subclass and pneumococcal polysaccharides specific IgG subclass antibodies.

The serum IgG subclass concentrations in 47 cases and specific IgG subclass antibodies against pneumococcal polysaccharides (PnPs) were measured in 18 cases with iron deficiency. IgG subclass deficiencies were found in 28 (59.6%) cases with the frequency in order as IgG4 (27.7%, 13/47), IgG1 (21.3%, 10/47), IgG3 (14.9%, 7/47), and IgG2 (2.1%, 1/47). Compared with age-matched healthy children, the mean concentration of serum IgG4 and IgG1, and PnPs specific IgG1, IgG2 antibodies were decreased in children with iron deficiency. Decreased CD4+ cells and CD4+/CD8+ ratio in peripheral blood, low interleukin-6 (IL-6) activity, reduced lymphocyte proliferative responsiveness and increased recurrent respiratory tract infections (RRTI) were found in iron deficiency children. These results suggested that serum IgG subclass and PnPs specific IgG subclass antibody deficiencies caused by dysfunction of the regulation of T lymphocyte on B lymphocyte may be related to the susceptibility to RRTI in children with iron deficiency.

Anemia, Iron-Deficiency↗
PubMed2004

Search for poliovirus carriers among people with primary immune deficiency diseases in the United States, Mexico, Brazil, and the United Kingdom.

OBJECTIVE: To estimate the rate of long-term poliovirus excretors in people known to have B-cell immune deficiency disorders. METHODS: An active search for chronic excretors was conducted among 306 persons known to have immunoglobulin G (IgG) deficiency in the United States, Mexico, Brazil, and the United Kingdom, and 40 people with IgA deficiency in the United States. Written informed consent or assent was obtained from the participants or their legal guardians, and the studies were formally approved. Stool samples were collected from participants and cultured for polioviruses. Calculation of the confidence interval for the proportion of participants with persistent poliovirus excretion was based on the binomial distribution. FINDINGS: No individuals with long-term excretion of polioviruses were identified. Most participants had received oral poliovirus vaccine (OPV) and almost all had been exposed to household contacts who had received OPV. Polioviruses of recent vaccine origin were transiently found in four individuals in Mexico and Brazil, where OPV is recommended for all children. CONCLUSION: Although chronic poliovirus excretion can occur in immunodeficient persons, it appears to be rare.

Adolescent↗
PubMed1998

Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia.

Epidemiological studies have shown wide geographical and racial variations in the prevalence and pattern of immunodeficiency diseases. As there is no national registry, very little is known of the prevalence and nature of humoral immunodeficiency in the Arabian peninsula. We report here for the first time the analysis of serum immunoglobulin (Ig) levels in 2000 consecutive patients (age, 1-80 years). They were seen over a period of 6 years and were referred to us from six district hospitals for suspected immunodeficiency, autoimmunity, allergy, or immunoglobulin dyscrasia. Forty-six were found to be immunodeficient, in whom at least one of the Ig class was low; 15 had secondary immunodeficiency. The remaining 31 cases, representing 1.5% of the population studied (giving a prevalence of 1550/100,000 hospital registered patients), were categorized into four primary humoral immunodeficiency groups: these included, in order of frequency, (i) selective IgA deficiency (45%; 700/100,000) (ii) common variable immunodeficiency (CVID) (29%; 450/100,000), (iii) agammaglobulinemia (16%; 250/100,000), and (iv) selective IgG deficiency (10%; 150/100,000). Compared with similar hospital-based surveys in the west the prevalence of humoral immunodeficiency seems to be higher in Arabs; this in part may be related to race and higher rate of consanguinity. Most patients with IgA deficiency had either infection, atopy or autoimmunity. Compared with some other races, agammaglobulinemia (X- and non-X-linked) seems to be more prevalent.

Adolescent↗
PubMed1980

Profile of multiple lymphocyte functional defects in acquired hypogrammaglobulinemia, derived from in vitro cell recombination analysis.

Immunoregulatory defects in patients can be assigned to B cells, T helper cells, or T suppressor cells by means of a modification of the in vitro PWM-stimulated Ig biosynthesis assay. When lymphocyte subpopulations from patient and normal are recombined, multiple internal comparisons within a single experiment reveal the functional activity of each subpopulation. By this technique we studied nine adult patients with acquired panhypogammaglobulinemia and one with isolated IgG deficiency. Low total Ig production by the B cell fraction was demonstrated in seven of the nine panhypogammaglobulinemic patients. In four of these seven, IgG and IgA secretion was markedly reduced compared with IgM. In the two patients with normal total Ig production, elevated IgM compensated for lower IgA and IgG. The one patient with no surface Ig+ cells had a B cell defect, but no T cell defect. Reduced T help and excessive T suppression characterized two of the four patients with the most severe B cell defects. Three patients were anergic by delayed hypersensitivity skin testing and failed to sensitize to DNCB, but the patient with the most severe T cell defects in vitro was not among them. One of these three patients showed a mild T cell help defect and suppressor excess and the other two had pure B cell defects. Thus, anergy and T regulator function were not correlated. In three instances where hypersuppression was more evident by adding patient unfractionated cells than by adding patient T cells, suppression by the T depleted fraction could be demonstrated. No cases of radiation-resistant T suppression were revealed among the seven patients tested. Subnormal total protein synthesis, noted in six of seven patients with low Ig production, was invariably less marked than the Ig defect and often affected both T and B cells. One additional patient with pure IgG deficiency of 2 yr duration was essentially normal in her in vitro lymphocyte function. The general applicability of this experimental design for analysis of positive or negative immunoregulatory abnormalities is emphasized.

Adult↗
PubMed1999

Recurrent multifocal demyelinating neuropathy with febrile illness and IgG subset deficiency.

We describe a unique syndrome of recurrent multifocal demyelinating motor greater than sensory deficits in cranial and peripheral nerve distributions with rapid, spontaneous improvement. Three patients presented with episodes over a period of 7 to 24 years, largely accompanied by febrile illness. Variably decreased IgG1 and IgG3 subclass levels were found. We postulate an immune-mediated process based upon the clinical presentation and presence of decreased IgG subclass levels.

Aged↗
PubMed1977

Opsonic requirements for phagocytosis of Streptococcus pneumoniae types VI, XVIII, XXIII, and XXV.

An assay system employing radiolabeled, heat-killed Streptococcus pneumoniae and human polymorphonuclear leukocytes was utilized to study serum pneumococcal opsonic requirements. Comparing the kinetics of phagocytosis in normal serum, heat-inactivated serum, immunoglobulin G (IgG)-deficient serum, C2-deficient serum, and magnesium dichloride ethyleneglycol-tetraacetic acid (MgEGTA)-chelated serum allowed definition of the opsonic requirements for four pneumococcal serotypes: VI XVIII, XXIII, and XXV. All four serotypes were efficiently opsonized in 10% normal serum. Only type XVIII was opsonized in heat-inactivated serum. All four were also opsonized in IgG-deficient serum but not as efficiently as in normal serum. Opsonization via the alternative pathway was diminished for all four serotypes in 10% MgEGTA-chelated and C2-deficient serum. Furthermore, by varying the concentration of MgEGTA-chelated serum, it was found that type XXV was least efficiently opsonized via the alternative pathway. The quantitative nature of this assay system will permit measurement of bacterial and host factors that may contribute to host susceptibility to pneumococcal infection.

Blood↗
PubMed1990

Raised stool and serum IgA levels in undernourished infants with chronic diarrhoea and associated parasitic infestations.

Sixty children with chronic diarrhoea, age ranging from 9 months to 3 years and 15 normal healthy children of same age group, all belonging to the low socio-economic families formed the basis of this study. Fifty-six out of these 60 children were undernourished and were marasmic. Stool examination showed enteropathogenic E. coli in 24 (40 per cent), Ascaria lumbricoides in 12 (20 per cent) and Giardia lamblia in 6 (10 per cent). Coeliac disease was detected in 2 (3 per cent) and combined IgA-IgG deficiencies were found in one case (2 per cent). No cause could be found in 15 (25 per cent) cases. Multiple aetiological factors were found in 7 (12 per cent) cases. Stool IgA levels were significantly elevated in the patients than in the controls and more so in the patients with giardiasis and also in patients with coeliac disease. Serum IgA levels were remarkably raised in the patients with diarrhoea due to enteropathogenic E. coli, indicating probable spilling of gut-associated IgA into the circulation. No IgA was detected in the stool of a dysgammaglobulimic patient, who had both serum IgA and IgG deficiencies.

Child, Preschool↗
PubMed1992

The role of the immunologist in sinus disease.

The clinical immunologist is playing an increasingly important role in the evaluation and management of sinus disease. Although most patients with sinus disease are not immunodeficient, a significant proportion of patients with chronic sinusitis unresponsive to medical and/or surgical therapy may have an immunodeficiency. Most immunodeficient patients for whom sinusitis is a major clinical problem tend to be those with humoral immunodeficiency diseases. The role of immunoglobulin replacement therapy is well established for patients with global immunoglobulin and antibody deficiencies (e.g., X-linked agammaglobulinemia and common variable immunodeficiency) and may be helpful in controlling refractory sinusitis in patients with more selective immunoglobulin deficiencies (e.g., IgG subclass deficiency and selective antibody deficiencies), but efficacy in these conditions remains to be established by controlled studies. Many immunodeficient patients have a history of repeated sinus surgery before the recognition of their immune defect. Even in immunodeficient patients treated with antibiotics and immunoglobulin replacement therapy, functional endoscopic sinus surgery is successful in only half of the patients.

Agammaglobulinemia↗
PubMed1979

Effect of normal human serum on the binding of specific antibodies and platelet-unrelated immune complexes to human platelets.

Radiolabelled staphylococcal protein A was used to quantitate the binding of IgG on stored human platelets from human sera containing specific antibodies reactive with platelets and rabbit serum containing immune complexes (IC). Normal human serum (NHS) inhibited the binding of IC onto platelets and to various extents also the binding of specific antibodies. The attachment of inhibitors to platelets seemed to be reversible. The considerable difference in the inhibitory capacities of IgG-deficient sera and monomeric IgG indicates that IgG is the major inhibitory component of NHS. The binding of IgG from NHS onto platelets evidently hampers the detection of weak platelet antibodies even with the most sensitive tests. Purified Clq, known to modify the reactions of IC with fresh platelets did not alter the binding of IC onto stored platelets. A monoclonal, antiglobulin-active rheumatoid factor of IgM class displayed only moderate inhibition. Therefore, the application of RF or Clq for the differentiation of the binding induced by IC or antibodies is not useful in this assay system. The heterogeneity of immunologic receptors of platelets provides an explanation of the inhibitory inefficiency of Clq.

Adult↗
PubMed2003

[The hyper-IgM syndrome: 13 observations].

INTRODUCTION: The hyper-IgM syndrome (HIGM) is a rare hereditary immune deficiency, characterised by a low or nil level of IgG and IgA and a normal or increased level of IgM, predominately affecting boys. Its clinical manifestations are dominated by recurrent infection, notably of the digestive tube, the ears nose and throat and the lungs. METHODS: We conducted a retrospective study of the medical files of 13 patients with 10 boys and 3 girls from 12 different families. In all the patients, the diagnosis was established on measuring the serum immunoglobulines IgG, IgA and IgM using Mancini's technique. RESULTS: The mean age at the onset of the disease was of 20 months (6 - 42 months). The clinical manifestations were dominated by recurrent and persisting infections, notably digestive (10 cases), respiratory (9 cases), cutaneous (5 cases) and glandular (5 cases). No pulmonary Pneumocystis carinii infection or sclerosing Cryptosporidium cholangitis was observed. Two boys exhibited neurological manifestations, unrelated to an infection. The IgG deficiency was constant and IgA deficieny encountered in 12 patients. Ten patients exhibited a high level of IgM (4.6 - 29,9 g/l) whereas this was normal in the 3 others. The expression of the CD(40) ligand was absent in the 6 boys in whom it was assessed and normal in the only girl studied. Eleven patients exhibited concomitant cellular immune deficiency. Three patients presented moderate neutropenia. Nine patients received replacement therapy with IV immunoglobulines with a residual level of IgG at 8,27 g/l (4 - 14,45 g/l) associated with the regression in the manifestations of infection. One patient died from a Burkitt lymphoma before any treatment was initiated. CONCLUSION: The primary hyper-IgM syndrome in Tunisia is characterised by a relative frequency of autosomal recessive forms (3 girls), the absence of Pneumocystis carinii pulmonary infections and sclerosing cholangitis and the occurrence of neurological manifestations (2 cases).

Adolescent↗