Lack of suppression by concanavalin A-activated neonatal mononuclear cells.

We studied the mixed leukocyte culture suppression generated by cord and newborn mononuclear cells stimulated by concanavalin A (Con A) compared to normal adult cells and cells from patients with systemic lupus erythematosus. Also we studied the effect of supernatants from cord or adult cells stimulated with Con A linked to sepharose (Con A sepharose). Peripheral blood mononuclear cells from 15 adults, 10 normal newborns, 23 cord blood samples, and 11 patients with systemic lupus erythematosus were preincubated with Con A for 48 hr, irradiated, and added to a one-way mixed leukocyte culture. Adult Con A-activated cells suppressed the mixed leukocyte culture by 30 +/- 6.5%. By contrast cord cells and newborn cells had no suppressive activity; these cells resulted in stimulation of 18 +/- 12.1 and 18 +/- 7%, respectively. This lack of suppression also was present in systemic lupus erythematosus cells (11.2 +/- 13.3). The supernatants of both Con A sepharose-stimulated cord and adult cells showed significant suppressive activity and there was some suppressive activity of sepharose-stimulated cells alone. These results suggest that the mixed leukocyte cultures suppressive activity observed previously by newborn cells is radiosensitive and dependent on ongoing cell division for its expression. It also is independent of prior mitogenic stimulation.

Neutrophil and T lymphocyte characteristics of two patients with hyper-IgE syndrome.

Immunologic parameters including quantitative and qualitative immunoglobulin studies, various T cell functions and neutrophil chemotaxis were evaluated in two patients with the Hyper-IgE syndrome. Both exhibited pruritic dermatitis in locations atypical for atopic dermatitis, marked elevations in serum IgE levels (to 40,000 IU/ml), recurrent staphylococcal abscesses, coarse facial features and variable chemotactic defects characteristic of this syndrome. Both patients responded favorably to courses of trimethoprim-sulfamethoxazole, particularly in helping control the cutaneous infections. We believe that this is a useful therapeutic alternative to anti-staphylococcal antibiotics and prophylactic treatment has permitted therapeutic response. Serum IgG, IgG subclasses, IgM, and IgA were normal for age. Serum IgD was markedly deficient in one patient. Functional IgM was normal with positive isohemagglutinin titers. IgG poliovirus titers were present in both patients; however, tetanus titers were not detectable in either patient, despite repeated immunizations. Despite normal E rosette numbers, subtle T cell abnormalities were noted with variable responses to both in vivo SK-SD, candida, and mumps skin tests and in vitro PHA-, Con A-tetanus-induced lymphocyte proliferation. Lymphocyte production of macrophage inhibitory factor and interferon and responsiveness in a mixed lymphocyte culture were normal in both patients. Considerable Con-A-induced suppressor cell activity was present in one patient, but diminished in the other. In vivo chemotaxis determined by a Rebuck skin window, revealed a markedly delayed PMN migration in both patients during a time when both patients were clinically free of furunculosis or dermatitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Reversal of enterocolitis-associated combined immunodeficiency by plasma therapy.

Two 6-month-old male infants with diarrhea, malabsorption, and hypoproteinemia, who were initially diagnosed as having combined immunodeficiency syndrome, recovered with intensive plasma therapy. Prior to the onset of diarrhea, they had normal serum protein and lymphocyte values. Immunologic features of combined immunodeficiency included lymphopenia, diminished B and T cells, cutaneous anergy, low immunoglobulin levels, and poor lymphocyte proliferative responses in vitro. Prior to therapy, both children had rectal ulcerations by proctosigmoidoscopy, colitis by rectal biopsy, and moderate to severe intestinal villus abnormalities by small bowel biopsy; plasma cells were absent Both had generalized malabsorption of all nutrients. Both infants were given irradiated fresh-frozen plasma for one to two months at 11 to 20 ml/kg/day to replace intestinal protein losses. During this time, diarrhea slowed, biopsy morphology improved, and immunoglobulin levels and T-cell function became normal. After discontinuance of plasma therapy, normal immune function and a normal stool pattern with reversal of malabsorption continued. Since intensive plasma therapy may have contributed to the reversal of the immunodeficiency state, a trial of such therapy is recommended in similar patients.

Scanning and transmission electron microscopic aspects of the nasal acilia syndrome.

A 3-month-old boy was admitted with failure to thrive and persistent fevers. During a 4 month hospitalization for treatment of suspected sepsis, persistent purulent nasal discharge developed. Biopsies of his nasal mucosa on 3 separate occasions disclosed thinned respiratory epithelium and a complete absence of cilia when examined by electron microscopy (EM). Despite an initial granulocytopenia and a wide range in T-cell numbers, he did not show any evidence of lower respiratory tract infection. A tracheal biopsy process for EM demonstrated normal ciliated epithelium. This patient appears to have an unrecognized syndrome of normal tracheal cilia but absent nasal cilia.