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Biomedical subjects

F Kueppers

Publications and source records attributed to F Kueppers.

At least 73 records · Page 4Linked to original sources

Serum concentrations of C3 and C4 of the complement system in patients with chronic obstructive pulmonary disease.

In a search for host factors for the complex multifactoral disease, chronic obstructive pulmonary disease, we compared some major components of the complement system in 111 subjects with forced expiratory volumes in 1 sec less than 70% of predicted (index subjects) to those in 111 subjects matched as pairs for age, sex, smoking, and occupation having forced expiratory volumes in 1 sec more than 85% of predicted normal. Significantly lower blood levels of C3 and C4 were found in the index subjects (p less than 0.001). Among the index subjects there was a significant correlation between chronic cough and expectoration and low C3 and C4. There was not a significant correlation between low values and the frequency and severity of past respiratory infections. Levels of serum B globulin fraction, in which C3 and C4 migrate, were not different between the index and control subjects.

Complement C3↗

Group-specific component (Gc) 'subtypes' of Gc1 by isoelectric focusing in US blacks and whites.

Isoelectric focusing was applied to the Gc polymorphism. In agreement with Constans et al., we found two common 'subtypes' of Gc1 that could not be identified by conventional electrophoretic procedures. They are labeled Gc1F and Gc1S. Gc1F has a slightly lower isoelectric point than Gc1S. In groups of US blacks the allele frequencies were for Gc1F; 0.732 and for Gc1S; 0.147. In whites these figures were 0.149 and 0.572. We also found GcAb in blacks with a frequency of 0.015. The concentrations in serum of Gc protein as measured by radial immunodiffusion did not differ according to phenotype.

Adolescent↗

Alpha-1 antitrypsin elevation in healthy neonates.

alpha 1-Antitrypsin concentrations and phenotypes were determined in plasma of 257 healthy newborns. Samples were taken during the first 3 days after birth (time 1) and approximately after 30 days (time 2). We found the alpha1-antitrypsin levels immediately after birth elevated in the three Pi phenotypes M, M1M2, and MS as compared to the concentrations in cord blood and those at the age of 30 days. The concentrations in cord blood were within the normal range for adults. The concentrations (in mg/ml) at times 1 and 2 (+/- S.D.) for the different phenotypes were M: 2.94 +/- 0.58, 1.99 +/- 0.35; M1M2: 2.81 +/- 0.48, 2.01 +/- 0.37; MS: 2.45 +/- 0.5, 1.55 +/- 0.33. These differences were highly significant (p less than 0.001). Samples with the PiMZ phenotype had the characteristically reduced levels and did not show a significant difference in concentration between the two samples: 1.54 +/- 0.25 mg/ml (time 1) and 1.42 +/- 0.34 (time 2). We conclude that the alpha 1-antitrypsin level in plasma of normal infants rises in response to stimuli in the immediate neonatal period, or that perhaps the rise is due to the process of delivery itself. Heterozygotes for Piz can apparently not respond, or respond only minimally, to the same factor(s).

Adult↗

HLA-A antigens of patients with Wegener's granulomatosis.

The frequency of HL-A antigens was determined in 31 patients with biopsy-confirmed Wegener's granulomatosis and compared with their frequency in healthy Caucasian control population. There was no significant difference between the two groups for any of the 24 HL-A antigens tested.

Adult↗

alpha1-Antitrypsin deficiency in nonsmokers.

The clinical symptoms, measurements of pulmonary function, and interpretations of thoracic roentgenograms in 18 patients with alpha1-antitrypsin deficiency associated with the PiZ phenotype are reported. The patients had never smoked and had little or no exposure to occupational and urban air pollution. The findings were compared with those in a group of patients who also had the PiZ phenotype, but who were smokers. The results showed that the clinical course, rate of pulmonary-function deterioration, and appearance of the thoracic roentgenograms in persons who had never smoked are variable and suggested that other factors, in addition to phenotype and environmental pollutants, are determinants of the chronic obstructive pulmonary disease that develops in these patients. Many of these patients lived into their sixth and seventh decades, suggesting that those patients who avoid respiratory irritants do not necessarily have an ominous prognosis. These are important considerations in the diagnosis and treatment of patients who have this deficiency.

Adolescent↗

Alpha1-antitrypsin: further genetic heterogeneity revealed by isoelectric focusing.

Alpha1-antitrypsin is a major human serum protein that shows an extensive polymorphism. Genetic heterogeneity has previously been demonstrated by starch gel electrophoresis. By applying analytical isoelectric focusing (pH 3.5--5.0) to this system, we found a common variant, Pi M3, with an isoelectric point between those of Pi M1 and Pi M2. The gene frequency of this variant was .11 in U.S. whites and .054 in blacks. When PiM3 and PiM1 are included in the Pi system, the heterozygosity at the Pi locus is five times greater in whites and 10 times greater in blacks than that detected by earlier electrophoretic techniques.

Black People↗

alpha1-Antitrypsin polymorphism in Malaysian Macaca irus.

alpha1-Antitrypsin types were determined in 200 individual specimens of Malaysian Macaca irus. We found the pattern B in 76 samples, BC in 116, and C in 8. Assuming that these patterns are determined by codominant alleles at one locus, this distribution constitutes a significant (P less than 0.001) deviation from Hardy-Weinberg equilibrium, because of an excess of BC and low prevalence of C. We found no clear evidence for the presence of alpha1-antitrypsin deficiency comparable to the situation in man.

Alleles↗

Alpha1-antitrypsin deficiency with M-like phenotype.

A patient with a low serum concentration of alpha1-antitrypsin (0-1 g/l) but with an M-like phenotype is described. Her parents and 2 sibs have a PIM phenotype, but all except the father have approximately half-normal levels of alpha1-antitrypsin: The M-like variant apparently cannot be distinguished from M-alpha1-antitrypsin, when it occurs with M in heterozygotes. The proposita has severe airways obstruction and emphysema, and her father has moderate chronic obstructive pulmonary disease. The mother and 2 sibs are healthy.

Adult↗

Exclusion of the HLA locus from a large portion of the long arm of chromosome 6.

HLA antigens were determined in two infants with multiple congenital anomalies and in their healthy parents and one sibling. One infant had a deletion of a major portion of the long arm of chromosome 6. The other child had a translocation of a similar piece of chromosome 6 to the short arm of chromosome 3. The mother and the maternal grandmother showed this translocation in a balanced state. The HLA types of both children and their parents exclude the localization of the major histocompatibility locus from the deleted or translocated portion of the long arm of chromosome 6.

Abnormalities, Multiple↗

Prevalence of fungal complement-fixing antibodies in sarcoidosis.

The prevalence of fungal complement-fixing antibodies in sera from 58 patients with sarcoidosis was determined and compared to complement-fixing antibody titers in 50 sera from a normal control group and 50 antinuclear antibody-positive sera. Sera from 9 patients with sarcoidosis had complement-fixing antibody titers greater than 1:8 to Histoplasma yeast antigen; serum from one normal control subject had a titer greater than 1:8; and none of the antinuclear antibody-positive sera demonstrated titers greater than 1:8. There were no significant complement-fixing antibody concentrations observed against histoplasmin, blastomycin, and coccidioidin antigens in any serum from the 3 groups studied. The increase in antibody titers to the Histoplasma yeast antigen might have been related to the generalized increase in immunoglobulin concentrations noted in patients with sarcoidosis.

Antibodies, Fungal↗

Pulmonary and extrapulmonary sarcoidosis in relation to circulating immune complexes: a quantification of immune complexes by two radioimmunoassays.

Serum specimens from 53 patients with pulmonary sarcoidosis were examined for the presence of immune complexes by 2 methods, the Raji cell and the monoclonal rheumatoid factor radioimmunoassays. We found increased concentrations of immune complexes in the sera of 27 patients by one or both techniques. A significant association was found between increased concentrations of immune complexes and stage III sarcoidosis. Seventeen of 23 patients with stage III sarcoidosis and 10 of 50 with stage I or II disease had increased concentrations of immune complexes. Eight of the 10 patients with stage I or II sarcoidosis and increased concentrations of immune complexes had extrapulmonary sarcoid features, such as erythema nodosum, synovitis, or salivary gland enlargements. The size of the immune complex was 15S in one of the patients examined. Concentrations of C4 were normal. The data suggest a possible role of immune complexes in the pathogenesis of pulmonary and extrapulmonary features of sarcoidosis.

Adult↗

Alpha1-antitrypsin phenotypes in chronic active liver disease and primary biliary cirrhosis.

Alpha1-antitrypsin concentrations and phenotypes were determined in groups of patients with chronic active liver disease and primary biliary cirrhosis. The concentrations of alpha1-antitrypsin were above normal values in both groups; the patients with primary biliary cirrhosis had higher concentrations than those with chronic active liver disease. The prevalence of common phenotypes in these two groups did not differ from that in a sample of healthy blood donors from this institution of from a large Norwegian sample. We interpret our data as disputing the view that alpha1-antitrypsin phenotypes, other than Z. significantly predispose adults to hepatic cirrhosis.

Adolescent↗