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Biomedical subjects

A J Ammann

Publications and source records attributed to A J Ammann.

At least 109 records · Page 6Linked to original sources
PubMed1982

Current status of pneumococcal polysaccharide immunization in patients with sickle cell disease or impaired splenic function.

It is now possible to prevent effectively the occurrence of overwhelming pneumococcal infection from the majority of pneumococcal organisms in patients with increased susceptibility to infection. Immunization of patients with sickle cell disease and patients with splenectomy results in a significant increase in antibody amounts which persist for long periods of time. Patients under 2 years of age and patients with Hodgkin's disease and splenectomy respond less well. Most "vaccine failures" which have been described, have been in immunosuppressed patients, or a result of infection with S. pneumoniae type 6A. Future directions in the development of a more effective vaccine for patients under 2 years of age and immunosuppressed patients will include research efforts to formulate a new immunogenic vaccine. Definitive recommendations regarding booster immunization must await more definitive studies, but current data suggests that booster immunization is not necessary for at least 5-6 years following primary immunization.

Anemia, Sickle Cell↗
PubMed1982

Posttransfusion syndrome. Rash, eosinophilia, and thrombocytopenia following intrauterine and exchange transfusions.

A clinical syndrome of transient maculopapular rash with associated eosinophilia, thrombocytopenia, and mild lymphopenia developed in 21 of 35 (60%) neonates receiving both intrauterine transfusions and postnatal exchange transfusions, and six of 17 (35%) neonates receiving multiple exchange transfusions for erythroblastosis fetalis. This syndrome did not develop in infants who received intrauterine transfusions without exchange transfusions or single exchange transfusions without prior intrauterine ones. Patients with this rash and associated laboratory abnormalities did not progress to acute or chronic graft-vs-host disease, and on follow-up examinations were alive and well.

Blood Transfusion, Intrauterine↗
PubMed1981

Effect of thymosin on cellular immunity in old age.

Cell-mediated immunity (CMI) in a population of 28 well-nourished, disease-free old-age individuals (65--103 years) was compared to that of healthy young adult controls (22--41 years) and the effects in vitro of thymosin fraction 5 (TF5) evaluated. The number of T cells (T cell rosettes, TCR) in the peripheral blood was the same as controls. However, there was a significant depression in lymphocyte response to phytohemagglutinin (PHA) (p Less Than 0.005). Limiting dilution of the PHA response revealed an inherent defect in the function of those cells that are capable of responding to PHA. The formation of TCR from old-age subjects was similar to controls when incubated with TF5, i.e. there was no enhancement. When TF5 was incubated with old-age and normal control lymphocytes in the mixed lymphocyte reaction (MLR), there were significant increases in the responses in both (71 +/- 39% and 64 +/- 52%, respectively; p Greater Than 0.2). There was also a mitogenic effect of TF5 on resting lymphocytes which was significantly greater in the control than in the old-age population (p Less Than 0.002). Our results suggest that in old age: (1) there is no loss in absolute numbers of T lymphocytes; (2) there is a defect in the ability of individual lymphocytes to function; and (3) the response of lymphocytes in vitro to TF5 in forming TCR and in the MLR is statistically similar to young adult controls, and suggests that the decline in CMI with age is not due to a loss of thymosin-responsive cells.

Adult↗
PubMed1981

Spectrum of Sjögren syndrome in children.

Sjögren syndrome, consisting of keratoconjunctivitis sicca and xerostomia with or without another autoimmune disease, is uncommon in children. We describe our retrospective experience with eight pediatric patients with SS. All had recurrent parotid enlargement and abnormal salivary gland biopsies, six had keratoconjunctivitis sicca, and five had other autoimmune manifestations, although only two of these had other clearly defined autoimmune disorders (mixed connective tissue disease and hypergammaglobulinemic purpura). Our patients had a higher incidence of primary SS, parotid enlargement, and hematologic abnormalities than did children previously reported with SS. Children with SS demonstrate a clinical heterogeneity comparable to that seen in adults.

Adolescent↗
PubMed1981

Evaluation of the opsonic requirements for phagocytosis of Streptococcus pneumoniae serotypes VII, XIV, and XIX by chemiluminescence assay.

A luminol-enhanced chemiluminescence assay was used to investigate opsonic requirements for phagocytosis of STreptococcus pneumoniae serotypes VII, XIV, and XIX. After opsonization with whole immune sera (with antibody and total complement pathway), heat-inactivated immune sera (with antibody alone), or magnesium dichloride-ethylene glycol tetraacetic acid-chelated immune sera (with antibody and alternative complement pathway), live S. pneumoniae cells were incubated at 37 degrees C with normal polymorphonuclear leukocytes while serial chemiluminescence measurements were recorded. The amount of chemiluminescence observed correlated closely with evidence of phagocytosis as observed by microscopy. Complement was required for efficient opsonization, since all three serotypes showed a slower rise and less integral chemiluminescence after opsonization with heat-inactivated serum as compared with whole serum. The alternative pathway provided opsonic activity equal to that of the total complement pathway for type XIX, but only intermediate activity for types VII and XIV. Type-specific antibody was also required for effective opsonization of all three serotypes since chemiluminescence was markedly reduced when bacteria were opsonized with antibody-depleted serum (serum absorbed with type-specific S. pneumoniae cells at 4 degrees C). Thus, chemiluminescence proved to be an effective means of defining the requirement for both antibody and complement in the opsonization and phagocytosis of S. pneumoniae.

Adult↗
PubMed1981

Clinical and immunologic spectrum of the DiGeorge syndrome.

This report summarizes the spectrum of clinical and immunologic findings gathered prospectively in 13 patients with the DiGeorge syndrome. Our patients demonstrated marked variability in both the clinical manifestations and the degree of immunodeficiency, confirming the findings of earlier individual case reports and retrospective autopsy reviews. Ages at the time of presentation ranged from one day to 4 months. Congenital heart defects including truncus arteriosus, ventricular septal defect, interrupted aortic arch, and tetralogy of Fallot commonly brought these infants to medical attention within the first two weeks of life. Abnormal calcium homeostasis was found in all patients. Those patients presenting after the first month of life often had hypocalcemic seizures as the initial clinical manifestation. Parathyroid hormone levels and the number and location of parathyroid glands varied considerably. Immunologic evaluation revealed that total lymphocyte counts, percent T-cells, total T-cells, and T-lymphocyte function ranged from normal to severely depressed. The most consistent immunologic abnormality, found in 11 of the 13 patients, was a decrease in total T-cells. Sequential studies in five patients demonstrate that spontaneous resolution of immunodeficiency may occur in some, yet progressive loss of immune function may be observed in others. Complete immunologic evaluation and careful followup is mandatory in infants with persistent hypocalcemia and congenital heart disease who are suspected to have DiGeorge syndrome.

B-Lymphocytes↗
PubMed1980

Response to pneumococcal polysaccharide vaccine in patients with untreated Hodgkin's disease. Children's Cancer Study Group Report.

Patients with Hodgkin's disease (HD) have a high risk of overwhelming pneumococcal infections after splenectomy. Previous studies have shown that HD patients given polyvalent pneumococcal polysaccharide (PPS) vaccine after immunosuppressive therapy have a suboptimum antibody response. This study shows significant antibody response in HD patients to PPS vaccine given before radiation and chemotherapy. The same response was obtained whether the vaccine was given before or after splenectomy.

Adolescent↗
PubMed1980

Ocular microbial flora in immunodeficient patients.

The eyes of 20 patients with primary immunodeficiency syndromes were examined, and microbial cultures of their lids and conjunctivae were made. Nine patients had a history of recurrent external ocular infections, and six of these had active blepharitis or blepharoconjunctivitis at the time of examination. A single pathogenic bacterium was isolated from the lids and conjunctivae of six patients with a history of infection; a mixture of two possible pathogens was cultured from eyes of three other patients. Three of the nine patients with infections had impaired B-cell and intact T-cell immunity, whereas three patients had impaired B cell and T-cell immunity. Two patients had chronic granulomatous disease in which a defect in phagocytosis was the predominant immunologic abnormality, and one patient (with mucocutaneous candidiasis) had intact B- and T-cell function at the time of testing. Although the immunodeficient patients had a higher incidence of lid or conjunctival infection than 20 age-matched controls, the microbial flora of the lids and conjunctivae did not differ between the two groups.

Adolescent↗
PubMed1980

Thymosin therapy in the DiGeorge syndrome.

Reconstruction of the T-cell immune defect in patients with the DiGeorge syndrome has been accomplished in the past by fetal thymus transplantation. Because of the risk of fatal graft-versus-host reaction with fetal thymus transplantation in patients with abnormal T-cell immunity, we have examined the effects of a thymus tissue extract, thymosin fraction 5, on the in vitro and in vivo immune function in children with the DiGeorge syndrome. T-cell numbers were increased with thymosin F5 in vitro in three of five patients. T-cell number and function was improved in three of four patients treated with thymosin F5 in vivo. Spontaneous improvement in the immune function of these patients cannot be excluded. These results suggest, however, that further trials with thymosin F5 therapy may be indicated in patients with the DiGeorge syndrome.

DiGeorge Syndrome↗
PubMed1980

Immunoglobulin G and M antibodies to pneumococcal polysaccharides detected by enzyme-linked immunosorbent assay.

An enzyme-linked immunosorbent assay has been developed to detect serum immunoglobulin G and M antibodies against pneumococcal polysaccharide antigens. Parameters affecting the specificity and sensitivity of the assay are described. A vigorous antibody response involving both the immunoglobulin G and M classes was demonstrated after pneumococcal polysaccharide immunization in normal adults. Studies with this enzyme-linked immunosorbent assay technique will allow further understanding of the biology of the primary and secondary immune response to Streptococcus pneumoniae in normals as well as in those persons most susceptible to infection with that organism.

Adult↗
PubMed1979

Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity.

Three siblings presented in early childhood with central-nervous-system (CNS) dysfunction, candida dermatitis, keratoconjunctivitis, and alopecia. Two were studied immunologically and had absent delayed-hypersensitivity skin-test responses and absent in-vitro lymphocyte responses to candida antigen. One of them had selective IgA deficiency and no antibody response to pneumococcal polysaccharide immunisation, and the other had a subnormal percentage of T lymphocytes in peripheral blood. The first two siblings died with progressive CNS deterioration and overwhelming infection. The third child, who presented with a periorificial candida dermatitis, alopecia, keratoconjunctivitis, and intermittent ataxia at eighteen months of age, had intermittent lactic acidosis and raised excretion of beta-hydroxyproprionate, methylcitrate, beta-methylcrotonylglycine, and beta-hydroxyisovalerate in urine. After four days of oral biotin, 10 mg/per day, the metabolites in her urine were significantly reduced, suggesting a biotin-responsive multiple carboxylase deficiency. These findings, taken with previous reports of immune defects in patients with disorders of branched-chain aminoacid catabolism, suggest a new biochemical basis for primary immunodeficiency disease.

Alopecia↗