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Biomedical subjects

H H Fudenberg

Publications and source records attributed to H H Fudenberg.

At least 235 records · Page 13Linked to original sources
PubMed1978

X-linked immunodeficiency with increased IgM: clinical, ethnic, and immunologic heterogeneity.

Two cases of immunodeficiency with increased IgM are reported. Patient 1 was a black male 3.5 years old who had recurrent pyogenic infections, failure to thrive, oral thrush, and systemic cryptococcal infection. Patient 2 was a 9-year-old white female who had recurrent cervical abscesses. Serum immunoglobulin determinations by radial immunodiffusion in both patients showed marked depression of IgG and IgA and marked elevation of IgM. A low molecular weight circulating monomeric IgM was demonstrated by immunoelectrophoresis and gel filtration in the second patient; this was not present in the first case. In vitro impairment of cellular immunity was observed in both patients. Administration of dialyzable leukocyte extracts (transfer factor) led to improvement of cell-mediated immunity in patient 1. The etiology of this syndrome apparently has several different genetic bases. These patients demonstrate heterogeneity in genetic, ethnic, immunologic, and other features of the syndrome.

Child↗
PubMed1978

Abnormal T cell subpopulations and circulating immune complexes in the Guillain-Barré syndrome and multiple sclerosis.

Immunologic studies were performed in 21 patients with multiple sclerosis (MS) and 16 with the Guillain-Barré syndrome (GBS). Levels of thymus-derived (T) cells measured by "total" and "active" rosette formation between sheep erythrocytes and peripheral blood mononuclear cells (TEt, TEa) were within normal limits in all the patients, with the exception of four GBS patients, including one who also had received chemotherapy for lymphoma and three who were receiving steroids. When lymphocytes from the 21 patients were incubated with the bone-marrow-derived (B) lymphoblastoid cell line PGLC-33H, there were, for 12 of 18 MS patients and 11 of 16 GBS patients, significant decreases in a subpopulation of peripheral blood T lymphocytes that form "PGLC rosettes" (PGR) with the PGLC-33H cells. (Peripheral blood T cells from normal individuals formed PGR with 23.9 +/- 3.8 percent of PGLC-33H cells.) Using the 125l-C1q binding assay, immune complexes were detected in the serum of 14 of 19 MS patients and 15 of 16 GBS patients. An association between increased C1q binding and decreased PGR values was found in 10 of 18 MS patients and 12 of 17 GBS patients. The results suggest that in both diseases the etiology may involve a decrease in the subset of T cells that bind to the IgM-producing cell line PGLC-33H, in association with the appearance of circulating immune complexes containing the infectious viral agent.

Antigen-Antibody Complex↗
PubMed1978

Genetic polymorphism of serum alpha-1-protease inhibitor (alpha-1-antitrypsin): Pi i, a deficient allele of the Pi system.

The microheterogeneity of the I allele of the Pi system of APi (alpha-1-antitrypsin) was studied in 43 individuals with the new PAGIF technique. The unique aspect of the I allele product (unequal distribution of bands 4 and 6), previously demonstrated with acid-starch gel, was confirmed. In addition, two subtypes of the Pi I allele--I1 and I2--were clearly distinguished. Serum concentrations of APi associated with the expression of the I allele were significantly decreased (68% of normal values) and thus very similar to those associated with the expression of the S allele. This indicates that the I allele can be considered as a "deficient" allele of the Pi system.

Alleles↗
PubMed1978

A new allele of human alpha1-antitrypsin: PiNhampton.

A new allele of alpha1AT is described. By isoelectric focusing, the microheterogeneous pattern of the variant was similar to but more cathodal than that of Pi N. This allele has therefore been tentatively designated PiNhampton(Nham). Further examination revealed that the minor bands of Nham are indistinguishable from the major bands of Z by isoelectric focusing, and a careful family study was necessary to clearly define the proband's phenotype. Pi Nham was found in association with M1, S, and Z, but to date its possession is not apparently related to clinical disorders or reduced serum levels of alpha1AT.

Adult↗
PubMed1978

Sex steroid hormones and antibodies to Candida albicans.

The effect of changes in progesterone (P) and estradiol (E2) on titres of antibodies to Candida albicans was studied by measurement of these three parameters in the following endocrinologically diverse human groups: normal females, gonadal dysgenetics, users of a sequential oral contraceptive (Oracon) and normal males. In females, C. albicans titres (mean +/- s.e.m.) were significantly higher (P less than 0.05) in the luteal (74 +/- 14) than in the follicular phase (34 +/- 19) of the cycle, and there were similar significant increases in P and E2. In the gonadal dysgenetic group (n = 29), with E2 levels comparable with males, the antibody titres were also equivalent to those in normal males (40 +/- 0.5), but were significantly lower than those of normal females in the follicular phase (P less than 0.05). In contrast, Oracon users, with high blood progestin levels, had C. albicans titres (118 +/- 15) significantly higher (P less than 0.001) than those of control subjects during the follicular phase. A significant correlation (P less than 0.05) was observed between P and C. albicans titres (mainly IgA) in randomly selected samples (n = 112) from normal females during the follicular and luteal phases, and in two subjects from whom blood samples were drawn daily for the entire cycle. In the latter, an increase in E2 but not P in the late follicular phase was accompanied by a marked decrease in C. albicans titres. No changes were observed in total immunoglobulin levels or antibodies to SRBC or Herpes virus in response to the marked changes in hormones. These results indicate that the production of antibodies to C. albicans may be specifically influenced by sex steroid hormones, being enhanced by P and E2 at low levels but depressed by E2 at high levels.

Antibodies, Fungal↗
PubMed1978

A practical method for quantitation of antibody responses to tetanus and diphtheria toxoids in normal young children.

Antibodies to diphtheria and tetanus toxoids were assayed in 46 children from 16 to 33 months of age following vaccination. A reversed rocket immunoelectrophoretic technique was used for antibody determination. This method is simple and consistent, and although the limit of detection for antibodies of both types (about 1 U/ml) is above the normal range for primary immune responses, antibody levels in immunized children five weeks after a second injection of vaccine were within measureable range in all but two of the children studied. Levels more than one standard deviation above the mean were found in one child for both antitetanus and anti-diphtheria and in four children for anti-tetanus toxoid antibody only. All four of the high responders to tetanus toxoid were Black, carrying both Black and Caucasian Gm haplotypes. The two apparent nonresponders were Caucasian. All children had normal immunoglobulin levels, except for one with low IgA, who showed normal antibody responses to both toxoids. The results suggest that measurements of precipitating antibodies to diphtheria and tetanus toxoids provide a practical index of humoral immune function in small children.

Antibodies, Viral↗
PubMed1977

Lymphocyte culture: induction of colonies by conditioned medium from human lymphoid cell lines.

The presence of phytohemagglutinin or pokeweed mitogen in cultures of human peripheral blood mononuclear cells in agar is known to stimulate the formation of lymphoid colonies. We now report that similar colonies can be induced in the absence of plant lectins upon addition of filtered and ultracentrifuged conditioned medium (CM) obtained from certain human lymphoblastoid cell lines. Colony formation required at least 6 X 10(5) mononuclear cells per milliliter, and optimum results were obtained at concentrations of 1 X 10(6) cells/ml in the presence of 20% CM (50-500 colonies per 10(6) cells cultured). Individual cells within colonies displayed uniform morphological characteristics of lymphoid cells, and the majority formed rosettes with sheep erythrocytes, suggesting that they were of T-cell type.

Cell Count↗
PubMed1977

Urinary lysozyme phenotypes in monocytic leukemia.

A method of two-dimensional electrophoresis has been devised to allow the study of the electrophoretic mobility of urinary lysozyme. Three different phenotypes have been defined in a study of thirteen purified lysozymes obtained from different patients with monocytic leukemia.

Electrophoresis↗
PubMed1977

Treatment of Bechçet's syndrome with transfer factor.

Six patients with Behçet's syndrome were treated with transfer factor (TF) from randomly selected donors. Mucocutaneous symptoms and signs were predominant at the time that TF injections were started. Three patients showed great improvement, one moderate improvement, and one was unresponsive to multiple injections of TF from different donors. One case was uninterpretable because of concomitant administration of high doses of prednisone and chlorambucil and brief treatment with TF. These results indicate that TF therapy may be beneficial in some patients with Behçet's syndrome and that a trial of TF is warranted at least in the absence of severe ocular or neurologic manifestations.

Adult↗
PubMed1977

Asynchronous development of two monoclonal proteins (IgM lambda and delta 1 chains) in a patient with abdominal lymphoma.

An IgM paraprotein was detected in a patient with abdominal lymphoma. Due to some doubts about the malignant nature of the situation, no cytostatic drugs were administered. When seen two years later the patient had considerably deteriorated in clinical status, with evidence of extra-abdominal involvement. At this time he presented a second paraprotein of low of molecular weight (74,000 to 80,000), consisting of two covalently linked delta1-chain fragments.

Abdominal Neoplasms↗