Brain abscesses due to Streptococcus milleri occurring in 2 previously healthy children are reported. Both children had evidence of liver involvement and both responded well to antibiotic treatment.
A majority of haemophiliacs who have received large-pool plasma products within the past 5 years have been exposed to the putative agent of the acquired immunodeficiency syndrome (AIDS)--HIV. It is not known what the risk of infection is among patients in South Africa. A study was made of 39 children with congenital coagulation disorders attending the Red Cross War Memorial Children's Hospital Haemophilia Clinic. All but 3 had been treated exclusively with small-pool lyophilised cryoprecipitate or a factor IX concentrate prepared by local blood transfusion services. Three patients had also received imported non-heat-treated commercial products FEIBA (Immuno), Autoplex, Proplex (Hyland) or Factorate (Armour). Absolute lymphocyte counts were normal in all patients but the OKT4/OKT8 ratio was reduced below 1.0 in 9 children including 2 of the 3 who had received commercial plasma concentrates. A high titre of HIV antibody was present in 2 of the 38 patients tested. Both of these children had received imported plasma concentrates and 1 shows some features of the AIDS-related complex. These results suggest that haemophiliacs who receive non-heat-treated commercial concentrates may be at greater risk of HIV infection than patients treated with locally produced plasma products.
Three children with ataxia-telangiectasia have been followed up since their early childhood. Sequential immunological, biochemical and chromosome studies have been performed over the last 7 years. All the children showed progressive cerebellar ataxia and inexorable neurological deterioration. Further evidence for the progressive nature of this condition is the fall in lymphocyte counts, deterioration of lymphocyte transformation responses to mitogens, and an increase in chromosomal translocations and breakage. Elevated serum alpha-fetoprotein levels are a highly characteristic and useful diagnostic finding in this condition. Two of the patients had an X,14 translocation. In vitro studies of immunoglobulin synthesis suggest an intrinsic defect in B-cell synthesis as well as decreased helper T-cell activity. In spite of moderately severe and progressive abnormalities in the immune system, sinopulmonary infections have not been prominent in our patients.
The synthesis of intestinal secretory IgA was studied in in vitro cultures of duodenal mucosal biopsies from children with Kwashiorkor. Production of secretory IgA was measured by the incorporation of radioactive label and visualized following PAGE and autoradiography. Results obtained before and after nutritional rehabilitation demonstrate an enhanced synthesis of sIgA in children with acute Kwashiorkor. Histological examination of plasma cells in the biopsy tissue confirms a twofold increase in IgA staining plasma cells in acute Kwashiorkor. Peripheral blood B lymphocytes in acute Kwashiorkor however, showed a reduction in IgA synthesis in the acute stage. These results suggest an effective mucosal sIgA response to the increased intestinal antigen load in Kwashiorkor.
Cord blood samples from 11 term neonates whose placentas showed histological changes typical of the amniotic fluid infection syndrome were analysed in order to determine haemolytic activity of the classic and alternative complement pathways and serum levels of complement proteins and immunoglobulins. Although the mean values of all these parameters were higher in this group than in an age-related control group, only classic haemolytic pathway activity was significantly elevated (P less than 0,025).
To describe the spectrum of primary immunodeficiency diseases (PIDs) diagnosed at Red Cross War Memorial Children's Hospital.Retrospective, descriptive study.Tertiary, referral hospital.All patients investigated by the immunology service because of suspected PIDs, between January 1983 and December 1996.Review of immunology service database and hospital case records.During the 14-year review period, 515 patients were investigated, a mean of 36.8 new patients per annum. Ninety-three patients with PIDs were diagnosed, a mean of 6.6 new patients per annum. The spectrum of PIDs was similar to that reported in developed countries. As in other series, antibody deficiencies predominated, accounting for 56% (52/93) of diagnoses. The male/female ratio was 1.5:1; 73% (62/85) came from the Western Cape, the remaining 27% (23/85) resided in five other provinces. Eighty per cent (70/87) presented with recurrent or atypical infection, with or without failure to thrive. Sinopulmonary infections (80%), diarrhoeal disease (19%) and candidiasis (18%) were the most common preceding infections. By the age of 5 years, only 60% had been diagnosed, compared with about 80% in developed countries. During the study period, 20% (19/93) were known to have died.The results show a pattern of PIDs incidence similar to that in developed countries. Diagnosis was delayed in many patients, which probably contributed to morbidity. To facilitate earlier diagnosis and to improve outcome, children should be considered for an immunological assessment if they exhibit increased susceptibility to infection.
This report describes a fourteen year old girl with an unusual immunodeficiency characterized by persistent lymphadenopathy and associated with hypogammaglobulinaemia, excessive IgM production and a severe T cell defect. Total T cell and T helper cell numbers were reduced and T cell proliferative responses to mitogens were poor. Serum IgM levels showed marked fluctuations and peaks correlated with acute tender lymphadenopathy. She was treated with intravenous gammaglobulin and prophylactic antibiotics. Although defective isotype switching of B cells into IgA and IgG producing cells has been accepted as the mechanism of the hyper IgM syndrome, it is becoming increasingly evident that T cell function is not uncommonly involved and may be responsible for impaired isotype switching.
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