Ammann Aj

Polyvalent pneumococcal-polysaccharide immunization of patients with sickle cell anemia and patients with splenectomy.

To reduce the risk of infection from Streptococcus pneumoniae in hyposplenic patients we administered octavalent pneumococcal vaccine to 77 patients with sickle-cell disease and 19 asplenic persons and compared their response with 82 controls (38 age-matched normal persons and 44 normal black African children). Fifty micrograms each of pneumococcal-polysaccharide Types 1, 3, 6, 7, 14, 18, 19, and 23 were administered subcutaneously. Post-immunization serums (three to four weeks) were available from 52 of 77 patients with sickle-cell disease; the percent responding and the magnitude of the indirect hemagglutination response were comparable to those of the controls. Within two years after immunization we observed eight Str. pneumoniae infections in 106 age-matched unimmunized patients with sickle-cell disease, but none in the 77 immunized (P less than 0.025). We conclude that pneumococcal polysaccharides are immunogenic in hyposplenic patients and may protect against systemic Str. pneumoniae infection.

COMPLICATIONS OF CO-TRIMOXAZOLE IN TREATMENT OF AIDS-ASSOCIATED PNEUMOCYSTIS CARINII PNEUMONIA IN HOMOSEXUAL MEN

In 8 of 18 homosexual men with the acquired immunodeficiency syndrome (AIDS) and Pneumocystis carinii pneumonia (PCP) treated with intravenous co-trimoxazole (trimethoprim-sulphamethoxazole) apparent drug-related complications developed during the course of acute therapy. A symptom complex of fevers and increasing malaise, often with nausea and headaches, developed usually after 9 days of therapy at a daily dosage of 20 mg/kg of trimethoprim and 100 mg/kg of sulphamethoxazole. These symptoms were associated with a diffuse erythematous maculopapular eruption and peripheral cytopenias. A similar picture was noted in two children with suspected AIDS-associated PCP. The high frequency of adverse reactions to co-trimoxazole therapy for PCP seems to be characteristic of AIDS patients.

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.

GROWTH RETARDATION IN SICKLE-CELL DISEASE TREATED BY NUTRITIONAL SUPPORT

The effect of increased nutritional intake was evaluated in 5 growth-retarded children with sickle-cell disease. Growth on recommended daily calorie and protein intakes had been inadequate in all 5. Fat absorption and intestinal mucosal morphology were normal in all 5. 2 children were given nutritional supplementation by nasogastric intubation, 1 received nightly oral formula supplements, and 2 were supplemented with zinc, iron, folate, and vitamin E only. Nutritional supplementation by the nasogastric route produced a rapid sustained increase in growth rate, associated with striking reductions in pain crises and infections which had previously necessitated many hospital admissions. Oral supplementation improved the clinical course but had no effect on growth rate. Mineral and vitamin supplements influenced neither the growth rate nor the clinical course. The observations indicate that nasogastric nutritional supplementation may accelerate growth and reduce the incidence and severity of complications in growth-retarded children with sickle-cell disease.

THYMIC TRANSPLANTATION IN A CASE OF CHRONIC MUCOCUTANEOUS CANDIDIASIS

Abstract A 17-year-old male with chronic mucocutaneous candidiasis, Addisons disease, pernicious anaemia, and malabsorption complicated by multiple B-vitamin deficiencies was transplanted twice with fetal thymic tissue. Previously negative in-vitro blastogenic response to candida became positive within one week of the first transplant and was restored to normal by the second transplant. A positive candida skin test was seen for the first time in 4 years. The patients clinical condition has improved and there has been clearing of oral candidal lesions and increased ability to mount a vigorous inflammatory response.

RESPONSE TO PNEUMOCOCCAL POLYSACCHARIDE VACCINE IN PATIENTS WITH UNTREATED HODGKIN'S DISEASE: Children's Cancer Study Group Report

Patients with Hodgkins 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.