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Biomedical subjects

H Herrod

Publications and source records attributed to H Herrod.

7 recordsLinked to original sources

Prediction of persistent immunodeficiency in the DiGeorge anomaly.

To assess the natural history of the immune defect in DiGeorge anomaly, we reviewed serial immunologic studies in 18 patients. The diagnosis was made with criteria based on the concept of the DiGeorge anomaly as a field defect. Initial or early follow-up laboratory examination suggested moderate to normal T cell function in 14 patients. None of these patients have lost T cell capability; they have never had infections characteristic of T cell deficiency. Four patients had clinical and laboratory evidence of profound immunodeficiency. A decreased number of CD4+ cells (less than 400/microliters) and a decrease in phytohemagglutinin responsiveness (stimulation index less than 10) may be useful in discriminating patients with immunodeficiency; absolute lymphocyte count and immunoglobulin values were not informative. At the time of surgery, the thymus was not found in 11 of 14 patients; however, only two of these patients had immunodeficiency. Patients with a persistently low number of CD4+ cells and decreased phytohemagglutinin response are candidates for immunologic reconstitution.

Aging↗

The use of methacholine inhalation, methacholine skin testing, distilled water inhalation challenge and eosinophil counts in the evaluation of patients presenting with cough and/or nonwheezing dyspnea.

Twenty-four patients presenting with cough and/or nonwheezing dyspnea were evaluated with methacholine inhalation challenge (MC), distilled water inhalation challenge (DC), intracutaneous tests to varying concentrations of methacholine, total eosinophil counts (TEC), sinus and chest x-rays. We found a statistically significant difference (P less than .005) in the mean TEC in those patients with a positive MC test and those with a negative test. Hyperreactivity of the airways to methacholine in asthmatics is not found in the skin. Distilled water inhalation did not serve to substitute for MC as a test of hyperreactive airways. The TEC is an excellent screening test as a predictor of patients with cough or dyspnea who have hyperreactive airways.

Adolescent↗

Lymphocyte phenotype and function in chronically transfused children with sickle cell disease.

Immunologic studies were performed on mononuclear cells from ten chronically transfused children with sickle cell disease, and the results were compared with those from five other groups: 21 sickle cell patients who were not receiving regular transfusions, 6 chronically transfused children with other forms of refractory anemia, 10 previously splenectomized children, 5 boys with hemophilia A, and 27 normal adult controls. The helper:suppressor T cell ratios (T4:T8) in all groups except hemophiliacs were normal, were unrelated to the number of units transfused, and were not suggestive of findings reported for patients with the acquired immune deficiency syndrome (AIDS) or groups at risk for the syndrome. Percentages of T3, T4, and T8 cells were low in sickle cell and splenectomized patients, but not in chronically transfused patients with other anemias. Serum IgG was frequently elevated, and IgG synthesis in vitro was increased relative to IgM synthesis in sickle cell patients. Coculture experiments indicated that such findings may stem from a selective increase in IgG synthesis by B cells. Thus, transfused sickle cell patients have a particular pattern of immunologic abnormalities that is distinct from that seen in AIDS.

Acquired Immunodeficiency Syndrome↗

Immunoregulatory abnormalities in Evans syndrome.

Immune function in six patients with Evans syndrome (Coombs-positive hemolytic anemia and immune thrombocytopenia) was compared to that in seven with chronic ITP. The two groups differed in measurements of T-cell subsets and immunoglobulin production. Evans syndrome patients had decreased T4 (T-helper) (P = 0.025), increased T8 (T-suppressor) (P = 0.008), and a decreased ratio of T4:T8 cells (P = 0.0009) when compared to controls. Results in chronic ITP patients were similar to those in controls. Serum IgG, IgM, and IgA levels and in vitro synthesis of IgG and/or IgM were decreased in most Evans syndrome patients. Diminished in vivo and in vitro immunoglobulin synthesis in Evans syndrome is consistent with the decreased T4:T8 ratio in these patients. The altered T4:T8 ratio may represent an unsuccessful response to an autoimmune process in which the trigger is unknown.

Adolescent↗

Side effects associated with intravesical mitomycin.

The nature and severity of side effects accompanying intravesical chemotherapy with mitomycin C were studied in 29 patients. No patient experienced systemic toxicity. Local side effects developed in 7 patients (24 per cent), and consisted of moderate cystitis in 3 and drug-related palmar desquamation, with or without generalized rash, in 4. Based on skin tests 3 of these patients were believed to have a contact dermatitis. The fourth patient had a generalized rash, in addition to palmar desquamation, that appeared after subsequent instillations of mitomycin C and the drug had to be discontinued. Careful cleansing of the hands and perineum on the day of treatment can prevent most skin reactions.

Aged↗

High anti-TNP plaque-forming cell potential of residual mIg+ cells in a T cell population.

In the course of experiments designed to study the immune response of purified populations of B lymphocytes to thymus-independent (TI) antigens, a variety of cell purification procedures were followed. In using anti-immunoglobulin-coated dishes to separate lymphocytes bearing membrane immunoglobulin (mIg) from mIg- lymphocytes, it was found that the nonadherent fraction, which was predominantly mIg-, complement receptor negative, and nonresponsive to the B cell mitogen lipopolysaccharide, gave very substantial anti-TNP plaque-forming cell responses to 2 TI antigens. These responses could be inhibited by incubation of such cells in the presence of anti-mu and thus appeared to be attributable to mIg+ cells. The evidence suggests the existence of a population of B lymphocytes that constitute a minor component of mIg+ cells having a high potential to make in vitro antibody responses. Users of techniques that utilize anti-Ig as a tool for separating B and T lymphocytes should carefully assess the extent to which residual B lymphocytes in the mIg- population contribute to antibody responses being studied.

Animals↗

The Stickler syndrome presenting as a dominantly inherited cleft palate and blindness.

The Stickler syndrome is a newly recognized, but probably relatively frequent inherited generalized connective tissue disorder involving skeleton, eye, and oro-facial structures. A family with three affected generations is discussed. Severe myopia leading to blindness, cleft palate, or subnucous cleft, Pierre Robin anomaly, premature degenerative arthritis, or a family history of any of these indicates further evaluation.

Adult↗