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At least 19 recordsLinked to original sources
PubMed1991

Selective IgA deficiency, IgG subclass deficiency, and the major histocompatibility complex.

Here we have examined the connection between IgA deficiency, IgG subclass deficiency, and the absence of alleles of complement C4, and show that IgA deficient subjects who have IgG subclass deficiencies may also have an increased frequency of C4 null alleles. In our group, we found an increased incidence of HLA B38 which might reflect the ethnic composition of the patients tested. While family studies are of primary importance to assess the relationships between histocompatibility antigens and immune deficiency, these studies are complicated by the observation that C4 null alleles are not always inherited with the humoral defect.

Adult↗
PubMed1999

Immediate hypersensitivity in adults with IgG deficiency and recurrent respiratory infections.

BACKGROUND: Little is known about the prevalence of atopy in adults with recurrent respiratory infections and IgG deficiency. OBJECTIVE AND METHODS: To elucidate this aspect, we skin-tested 95 consecutive adults with respiratory infections, subnormal levels of IgG subclasses or common variable immunodeficiency and usually poor response to vaccination. In 50 subjects we also measured total IgE. RESULTS: We found 67 subjects with IgG subclass deficiency, 21 subjects with mild (partial) and 5 with usual common variable immunodeficiency, and 2 subjects with functional IgG deficiency. Atopy was encountered in 42/95 subjects, 33/44 (75%) with asthma, 7/19 (38%) with isolated rhinosinusitis, 1/27 (4%) with chronic obstructive lung disease, and 1/5 (20%) with both the latter disease and asthma, respectively. Atopy was preferentially clustered in subjects with asthma (P < .05) who were less than 40 years of age (P < .05) and nonsmoking. Atopy was not affected by the type of IgG deficiency, unless it was usual common variable immunodeficiency, in which case the skin tests tended to be negative (4/5). Total IgE was within normal range but less elevated than usually seen in asthma or chronic obstructive lung disease. Total IgE was independent of the type of IgG deficiency, except for usual common variable immunodeficiency in which it remained < 10 IU/mL. CONCLUSIONS: In adults with symptomatic IgG deficiency, the prevalence of immediate hypersensitivity and its modulation by age and smoking are similar to the referred, non-IgG deficient population; however, total IgE may be lower in the former than in the latter. In common variable immunodeficiency, consistent with the literature data, both the prevalence of atopy and serum total IgE are decreased.

Adult↗
PubMed1988

IgG2 subclass deficiency: IgG subclass assays and IgG2 concentrations among 8015 blood donors.

Among the four IgG subclasses in humans, IgG2 is preferentially expressed in antibodies to carbohydrate antigens whereas IgG1 subclass is commonly associated with antibodies to protein antigens. Because of this association with carbohydrate antigens, values for IgG2 in serum are often used as an index of immunocompetence against carbohydrate antigens. To investigate the value of IgG2 measurements in a general population, we have developed a convenient IgG subclass assay, using monoclonal antibodies and particle concentration fluorescence immunoassay. Our assay is specific, precise, convenient, and accurate. When IgG2 concentrations were determined in the serum samples from 8015 adult blood donors, there were more individuals with low IgG2 concentrations than predicted by the log-normal distribution. The observed distribution suggested the presence of a subpopulation with low IgG2 concentration. Because apparently healthy individuals in a general population have low IgG2 concentrations, IgG2 measurements alone may have a limited clinical usefulness as an index of immune function against carbohydrate antigens.

Adolescent↗
PubMed1993

IgG deficiency in adults with recurrent respiratory infections.

Total IgA, IgM, IgG, and IgG subclasses were measured in 136 consecutive adults with recurrent respiratory infections. Sinus and chest radiographs were also obtained. IgG antibodies to H. influenzae and tetanus toxoid were determined in 27 immunodeficient subjects. Fifty-eight of the 136 were deficient in some immunoglobulin isotype, three in IgA, three in IgM, and 52 in total IgG or one or more IgG subclasses. The most common IgG deficiencies were single IgG subclass deficiencies, particularly IgG3 or IgG4, and a mild decrease in total IgG (between 450 and 650 mg/dL). In 21/27 subjects with IgG deficiency, the response to booster immunization was blunted, even when IgG1 and IgG2 were normal. Thirty-eight patients were smokers, 37 being > 40 years of age at the onset of respiratory infections. Twenty patients were nonsmokers, 19 being < 40 years of age at the onset. In conclusion, adults with recurrent respiratory infections frequently have some variant of IgG deficiency, often associated with a functional impairment of specific antibody response. Smoking may represent a risk factor for IgG deficiency in adults.

Adult↗
PubMed1994

Airway obstruction in adults with recurrent respiratory infections and IgG deficiency.

In 42 adults with recurrent respiratory infections (RRI) and common variable immunodeficiency or immunoglobulin G (IgG) subclass deficiency, the results of pulmonary function tests were related to factors apt to produce airway obstruction: serum concentration of IgG and IgG subclasses, various features of acute RRI (number/year, time from onset to diagnosis, episodes of pneumonia, etc) and type of chronic lung disease (smoking and nonsmoking related chronic bronchitis, episodic wheezing, and bronchiectasis). Compared with nonsmokers, usually less than 40 years of age, the patients above 40 had smoking-related chronic bronchitis and had obstruction (%FEV1/forced vital capacity [FVC] 55.3 +/- 8.1 vs 80.1 +/- 4.5), hyperinflation (residual volume 182.7 +/- 22.7 percent vs 109.7 +/- 8.8 percent of pred) hypoxemia (66.6 +/- 5.8 vs 83.4 +/- 4.2 mm Hg) and impaired carbon monoxide transfer (65.5 +/- 9.1 percent vs 93.3 +/- 5.8 percent). The features of acute or chronic RRI, the time from onset to diagnosis (< 10 yr in the entire group), the type of IgG deficiency or the serum concentration of the deficient protein did not correlate with substantial obstruction (FEV1/FVC < 70%). In conclusion, in adults with IgG deficiency and RRI for less than 10 yr, smokers with chronic bronchitis rather than nonsmokers develop substantial airway obstruction.

Adult↗
PubMed1987

IgG subclass deficiencies.

IgG subclasses represent distinct immunoglobulin types, each defined by unique primary structures of the constant region of the heavy-chain molecule, and each with characteristic biological and functional properties. New methods and reagents to measure serum concentrations have contributed to the study of the ontogeny, antigen restriction, and deficiencies of IgG subclasses. Distinct clinical entities of single or multiple IgG subclass deficiencies have been identified and the efficacy of immunoglobulin replacement therapy has been demonstrated in several instances.

Adolescent↗
PubMed1997

Lupus vulgaris in a patient with systemic lupus erythematosus and persistent IgG deficiency.

We present the case of a patient with juvenile onset systemic lupus erythematosus (SLE) who developed a persistent, acquired hypogammaglobulinaemia with IgG deficiency. The hypogammaglobulinaemia was probably a complication of high dose corticosteroid treatment. The serum IgG level remained subnormal despite intravenous immunoglobulin therapy. Lupus vulgaris, which developed on the nasal cartilage in this patient with SLE, is not an expected finding. This patient is probably the first reported case of SLE associated with lupus vulgaris.

Adult↗
PubMedDate not supplied

IgG subclass deficiency.

IgG subclasses have been recognized since the early 1960s. Four such subclasses, designated IgG1, IgG2, IgG3, and IgG4, are known to exist. Approximately 65 to 70% of the total circulating IgG in normal persons is of the IgG1 subclass. IgG2 constitutes 20 to 25% of circulating IgG, and IgG3 and IgG4 each represent less than 10%. Deficiencies in the various IgG subclasses have been detected in adults and children with common variable hypogammaglobulinemia as well as in those with relatively normal total IgG levels. An important issue facing clinicians today is to determine what, if any, therapeutic implications are associated with demonstration of an IgG subclass deficiency.

Humans↗
PubMed2000

Molecular basis of IgG subclass deficiency.

IgG subclass deficiency was recognized as a separate disease entity in the early seventies and was shown to be associated with an increased susceptibility to infections. Although deletions of the corresponding gamma genes have been demonstrated in a few cases, a majority of patients suffer from a regulatory dysfunction, and the deficiencies are most often relative rather than absolute. Some of the molecular mechanisms underlying the disorder have been unraveled recently. In this review we will touch upon the deletions described within the IGHC locus but mainly concentrate on the regulatory aberrations involved in IgG subclass deficiency.

Gene Deletion↗
PubMed2002

Localization of extrapulmonary tuberculosis in the synovial membrane, skin, and meninges in a patient with systemic lupus erythematosus and IgG deficiency.

We report on a 31-year-old female patient with systemic lupus erythematosus (SLE) for 24 years who had a past history of skin tuberculosis (lupus vulgaris), long-term corticosteroid therapy, and IgG deficiency. She presented with monoarthritis and concomitant meningitis from skin tuberculosis after 5 years. The diagnosis of joint and meningeal tuberculosis was defined with clinical symptoms--signs and typical histopathological findings of involved synovium. Clinical improvement was achieved with antituberculous therapy. Cutaneous, articular, and cerebral manifestations of tuberculosis might have been confused with some of the lupus manifestations or lupus activation. It should be kept in mind that tuberculosis may be encountered in SLE due to the nature of the underlying disease and/or its therapy. It is also worth mentioning that, in this patient, tissues involved with extrapulmonary tuberculosis were the primary areas of involvement with SLE.

Adrenal Cortex Hormones↗
PubMed1986

IgG subclass deficiencies.

IgG subclasses differ not only in their biochemical and biologic properties, but also in their occurrence as selective deficiencies, alone and in combination. The normal range of IgG subclass concentrations in children and adults is compared with deficiencies found in individuals with repeated respiratory tract infections. Concomitant IgA deficiency is often noted. It is suggested that decreased IgG2 and IgG3 levels in IgA-deficient individuals may increase the risk of recurrent respiratory infection and lung damage. Finally, the efficacy immunoglobulin. prophylaxis in reducing of infection is anecdotally related.

Adult↗
PubMed1991

IgG subclass deficiency with or without IgA deficiency.

IgG subclass deficiency (IgGSD) is difficult to define since reference materials vary between laboratories and the clinically relevant cut off levels for the various subclasses are not well known. The diagnosis of IgGSD should be based on more than one determination since the levels vary, due to various factors such as infections, operations, etc. There is a relation between IgGSD and occurrence of frequent infections, but at the same time even total lack of a subclass can be seen in healthy individuals. Infections in the upper and lower respiratory tract predominate. Gm allotypes influence IgG subclass levels. Most IgGSD patients are homozygous in the Gm system. Immunocytes producing IgG3 in the nasal mucosa correlate with serum IgG3 levels, whereas rectal IgG1 producing cells relate to serum IgG1 levels. This may reflect differences in antigen and/or mitogen exposure at the two mucosal sites. IgG2SD is the predominant form of IgGSD among children, whereas after puberty IgG3SD is most common. At the same age period there is a switch in sex distribution from three boys/one girl to one male/three females. Various abnormalities in B and T lymphocyte numbers and function are often found. In a double blind crossover study of Ig prophylaxis over 2 years in 43 IgGSD adult patients a significant decrease in the number of days with infections was seen both in the whole group and among the IgG1 deficient. Days with bronchial constriction were also significantly decreased among the 22 patients who had asthma as well. Acute bronchitis became significantly less frequent in the IgG3 deficient patients during Ig prophylaxis. This was seen in those under the lower range of 0.41 g/liter of Oxelius' early normal material, although many recent reference materials suggest 0.14-0.15 g/liter of IgG3 as the cut off. In a group of 25 consecutive patients with IgGSD + IgA deficiency recurrent respiratory infections were the major problem and lung function impairment was found in 12, and bronchiectasies in 5. Still many individuals with this combined deficiency have been reported to be healthy, again illustrating our limited understanding of these conditions.

Disease↗
PubMed2005

Increased levels of galactose-deficient IgG in sera of HIV-1-infected individuals.

BACKGROUND: The IgG from sera of patients with chronic inflammatory diseases of autoimmune character or some chronic microbial infections is frequently deficient in galactose on N-linked glycans. However, this phenomenon has not been investigated at length in human viral infections. OBJECTIVES: To evaluate the glycosylation of serum IgG in HIV-1-positive patients. METHODS: Psathyrella velutina lectin was used in enzyme-linked immunosorbent and Western blot assays to determine glycosylation. In addition, gas-liquid chromatography and mass spectrometry were utilized to confirm the galactose deficiency observed in the lectin-binding assays. RESULTS: HIV-1-infected individuals had significantly higher levels of galactose-deficient IgG than healthy controls. In fact, the galactose deficiency of the N-linked glycans observed in other diseases was even more profound in HIV-1 infection. This deficiency was primarily restricted to IgG when total serum glycoproteins were evaluated and IgG1 was the subclass most affected in all patients. Also, a significant increase in lectin binding was observed on IgG2 and IgG4 from HIV-1-positive females compared with HIV-1-negative females. CONCLUSIONS: Identification of deficient galactosylation of serum IgG from HIV-1-infected patients extended the spectrum of diseases in which this phenomenon has been observed. In addition, the results suggest yet another aspect of immune dysfunction as a result of HIV-1 infection.

Adult↗