Role of neutrophil antigen NA1 in an infant with autoimmune neutropenia.

A nontransfused 14-month-old female infant was investigated for persistent neutropenia of eight months' duration, with absolute neutrophil counts ranging from 410 to 935 cu mm. The patient's sera reacted with neutrophils from her own peripheral blood, from normal donors, and from her mother, all these having the neutrophil antigen NA 1, but not with neutrophils from NA 1-negative donors, including the father. The autoantibody was detectable by capillary agglutination and by indirect immunofluorescence techniques but not by complement-dependent cytotoxicity. No antibody was found in the mother's serum. Studies on three occasions showed good correlation between the appearance of circulating autoantibody and the peripheral neutrophil counts. Our observations, together with previously published reports, suggest a possible relationship of NA 1 antigen and the disease susceptibility of NA 1-positive infants to autoimmune neutropenia.

Chemoprophylaxis of recurrent otitis media using trimethoprim/sulfamethoxazole.

This study was undertaken during the late fall, winter, and early spring months to determine the efficacy of daily trimethoprim/sulfamethoxazole (TMP/SMX) administration in the prevention of recurrent acute otitis media (RAOM) in a specific high-risk pediatric population. Twenty-one ambulatory patients, 10 in the study group and 11 in the control group, were selected from a medical university clinic and a local private practice. The study group receiving prophylactic therapy for six months suffered no recurrences, as compared with eight children suffering one recurrence each in the control group that was treated only acutely. The results were significant at p less than 0.005 with df = 1, determined by the Fischer's exact test and the chi-square method using Yates's correction factor. These data indicated that a mean dose of TMP/SMX 6.8/34 mg/kg/d divided into twice daily doses and given for six months was safe and effective in controlling RAOM infections in a high-risk pediatric population.

Bactericidal capacity of polymorphonuclear leukocytes from patients receiving theophylline therapy.

The capacity of polymorphonuclear leukocytes (PMNs) to kill Staphylococcus aureus strains 502 A was studied in 25 patients receiving theophylline therapy and in normal healthy controls. A significant difference in PMN bactericidal capacity was found between controls and patients with serum theophylline levels higher than 8 micrograms/ml, as determined by high-pressure liquid chromatography. The bactericidal capacity of PMNs from both the patient and control populations was reduced in the presence of theophylline levels above 8 micrograms/ml. In addition, a transient but significant drug-independent reduction was found in the bactericidal capacity of transient but significant drug-independent reduction was found in the bactericidal capacity of PMNs from patients receiving theophylline.

Chorioretinitis with a combined defect in T and B lymphocytes and granulocytes. A new syndrome successfully treated with dialyzable leukocyte extracts (transfer factor).

A patient with immune deficiency, recurrent pyogenic infections and active chorioretinitis is described; in addition to agammaglobulinemia, both quantitative and qualitative T-cell deficiencies were documented. Furthermore, the patient's granulocytes (polymorphonuclear leukocytes), although normal in their bactericidal capacity for Staphylococcus, responded poorly to both leukocyte migration inhibition factor and neutrophil immobilizing factor obtained from normal cells. The immunologic features of this patient appear to comprise a new syndrome. Remarkable diminution of the ocular lesions and increased visual acuity occurred within two months after the initiation of therapy with dialyzable leukocyte extracts (transfer factor). Concurrent testing of the patient's cell-mediated immunity showed increased numbers of circulating T lymphocytes and improved T-cell function following dialyzable leukocyte extract [DLE] therapy. The dramatic clinical results indicate that similar therapy may prove to be beneficial in other patients with chorioretinitis and T-cell deficiency.

Association between immunoglobulin allotypes and immune responses to Haemophilus influenzae and Meningococcus polysaccharides.

Serum samples were collected from 20 healthy White and 33 Black infants before and after immunisation with three doses of diphtheria-pertussis-tetanus vaccine and with one dose of Haemophilus influenzae type b polyribose phosphate vaccine and meningococcal group A and group C polysaccharide vaccines. Antibodies to these immunogens were measured and sera were allotyped for several Gm, A2m, and Km antigens. A highly significant association was found between the Km(1) allotype and the immune responses (difference between post-immunisation and pre-immunisation antibody levels) to H. influenzae and meningococcus C polysaccharides in the White children.

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.

Proteus mirabilis brain abscess in a neonate.

Occasionally a brain abscess has been observed in a neonate. This report presents a unique case of a septic infant who developed a proteus mirabilis brain abscess shortly after birth, which persisted undetected until 21/2 months of age.

Antibodies to Candida albicans in IgA-deficient humans.

Titers of serum antibody to Candida albicans were studied in 111 IgA-deficient subjects by the passive hemagglutination method. Circulating antibodies to C. albicans (mean titer, 1:7) were found in only 7.3% of the 111 subjects, as compared with 80% of 137 controls (mean titer, 1:16). However, two IgA-deficient patients with systemic candidiasis were positive for these antibodies (mean titer, 1:64). Indirect immunofluorescence tests indicated that the antibody response in IgA-deficient subjects to superficial or deep-seated infection with C. albicans consists mainly of immunoglobulins of the IgG rather than of the IgM class.

Mucocutaneous lymph node syndrome.

Mucocutaneous lymph node syndrome represents a series of clinical findings that has been observed primarily in Japanese children. The disease now appears to be migrating to this country. It involves the cervical lymph nodes, the skin, and mucus membranes. Although the course is usually benign and self-limiting, a number of deaths have resulted from coronary artery disease.