DiGeorge anomaly associated with 10p deletion.

DiGeorge anomaly (DGA) represents a heterogeneous entity, which is often sporadic, although familial cases and the association with monosomy 22q11 have been reported. Recently, a few patients with 10p deletion syndrome and immunological and other laboratory findings similar to DGA have been described. We report on an additional case of partial DGA associated with 10p deletion.

Jejunal bacterial overgrowth and intestinal permeability in children with immunodeficiency syndromes.

Seventeen paediatric patients with immunodeficiency syndromes (10 with selective IgA deficiency, four with panhypogammaglobulinaemia, and three with selective T cell deficiency) were investigated for bacterial overgrowth of the small intestine and gut permeability to macromolecules. Five of 12 patients showed viable bacterial counts of more than 2 x 10(5)/ml in jejunal fluid. Bacterial overgrowth was also confirmed indirectly by breath hydrogen determination, which was higher than 10 ppm in four of the five patients with positive jejunal culture.

Effect of prednisone on DR-positive T cells in children with chronic active hepatitis B.

The effect of short-term immunosuppressive treatment on the percentage of circulating DR-bearing T cells was investigated in 16 children with HBsAg-positive chronic active hepatitis. DR-positive T cells, thought to represent activated T cells, were significantly increased in all patients as compared to 10 age-matched controls [14.5 +/- 4.

Immunoregulatory functional abnormalities in children affected by HBsAg-positive chronic active hepatitis: role of prostaglandins in T-mediated suppression.

Suppressor cell function was evaluated in children affected by HBsAg-positive chronic active hepatitis. Circulating concanavalin A- (ConA) precultured lymphocytes failed to suppress the proliferative response of autologous responder cells to a mitogen. In four of eight patients with a failure of ConA-induced suppressor activity, indomethacin added during the induction phase of T suppressor cells abolished the defect, indicating that prostaglandin-producing adherent cells may influence ConA-induced suppressor activity.

Impaired suppressor activity in children affected by coeliac disease.

Immunoregulatory cells were enumerated in 19 coeliac disease children on a gluten free diet by means of monoclonal antibodies that define total T lymphocytes (T3), helper/inducer T cells (T4), suppressor/cytotoxic T cells (T8) and monocytes (M1), as well as by means of surface receptors for Fc fragments of IgM and IgG (T mu and T gamma, respectively). In addition, suppressor cell function was assessed in 17 coeliac disease patients by examining the ability of concanavalin-A (Con-A)-activated suppressor cells to inhibit autologous cell response to mitogenic stimulus as compared with age-matched controls. No statistically significant differences were found in the percentages of subsets defined by monoclonal antibodies between coeliac disease patients and age-matched controls, whereas coeliac disease patients had a significant decrease of the subpopulation bearing membrane receptor for Fc fragment of IgG.

Chronic diarrhea and failure to thrive in an infant with Campylobacter jejuni.

We report the case of an infant with chronic diarrhea and failure to thrive. Campylobacter jejuni was isolated from stools and treatment with erythromycin resulted in eradication of infection and prompt resolution of symptoms. A 22-month-old girl was referred to our University Hospital because of weight loss and chronic diarrhea, which did not respond to repeated dietetic trials that excluded milk, gluten, and other foodstuffs.

Prolonged Q-T interval syndrome presenting as idiopathic epilepsy.

We report the case of a 4 1/2-year-old girl admitted to our Hospital because of repeated tonic convulsions. These attacks were triggered by noxious stimuli as well as by emotional stress. Since patient's history was not typical of idiopathic epilepsy, and several electroencephalograms failed to reveal any abnormality, a cardiac basis for the clinical picture was suspected.

A solid-phase radioimmunoassay for IgG gliadin antibodies using 125I-labelled staphylococcal protein A.

A sensitive radioimmunoassay for IgG gliadin antibodies is described. Serum specimens were added to wells of plastic microtitre plates coated with gliadin. After removal of the unbound material, gliadin antibodies were detected by adding 125I-labelled staphylococcal protein A (125I-SpA).

Immunoregulatory T subsets in chronic active viral hepatitis: characterization by monoclonal antibodies.

Immunoregulatory T subsets, defined by monoclonal antibodies, were enumerated in children affected by HBsAg-positive chronic active hepatitis. The helper to suppressor/cytotoxic cells ratio was lower in patients than in age-matched controls. The lower ratio was mainly due to an increase of lymphocytes of the suppressor/cytotoxic phenotype.

Colostral T lymphocytes detected by intracytoplasmic and membrane markers.

Colostral lymphocytes were studied using two established T-cell markers: intracytoplasmic alpha-naphtyl-acetate esterase (ANAE) staining and membrane receptors for sheep erythrocytes (E rosettes). ANAE staining allowed counting and identification of T-cell subsets independently of the status of membrane structures and receptors frequently altered in colostral cells. The fact that a sizeable number of colostral lymphocytes had the same phenotype as the majority of mature circulating peripheral blood lymphocytes supports the hypothesis that colostral lymphocytes may play a role in protecting neonates against infections, in transferring immune information to the newborn, or in modulating the immune response via release of soluble factors.