Search

Defective chemotaxis and phagocytosis of Staphylococcus aureus was identified in a female infant with marked hepatosplenomegaly and recurrent infections. Neutrophil mobilization from the marrow and marginal pool; random mobility; phagocytosis of yeast particles, latex beads and carbon particles; phagocytosis and intracellular killing capacity of Streptococcus pyogenes and E. coli; and NBT dye reduction were normal. Infiltration of neutrophils was depressed in the skin window test. The complement system and cellular immunity were intact. The patient also had a persistent EB virus infection. Defective phagocytosis limited to Staphylococcus aureus seems to be caused by an intrinsic cellular defect, such as congenital defect in neutrophil receptors for Staphylococcus aureus.

A case ofde novo 18/21 translocation resulting in 18q trisomy was described. Based on both G-and C-bandings, the patient's karyotype was identified as 46,XX, t(18;21) (p11;q11). Her clinical feature fullfilled almost all criteria of the 18 trisomy syndrome reported.

The number of patients with the first attack of rheumatic fever reached a peak in 1960, decreased remarkably until 1968, then has remained unchanged for the last nine years. Approximately 10% of rheumatic patients still develop recurrences. we would like to express our thanks for the cooperation of the seven pediatric departments which made this report possible.

This report showed the comparative study of the rheumatic fever and rheumatic heart disease in Japan and Thailand. At first, there is remarkable difference about the incidence of the rheumatic heart disease. The incidence of the rheumatic heart disease in Thailand was one hundred times that in Japan. Secondly, there is particular difference about the course of mitral stenosis in childhood. In Japan common pattern of rheumatic heart disease was slight mitral insufficiency and mitral stenosis was seldom. In Thailand mitral stenosis was observed in 5% on rheumatic heart disease, and mitral stenosis developed early following an attack of rheumatic fever. These differences were supposed due to the socio-economic state in each country.

The effect of the IgE-mediated reaction on the passive cutaneous anaphylaxis (PCA) reaction was studied at local skin sites of monkeys, and we found that the IgE-mediated reaction appeared to enhance the PCA reaction. Interactions among IgE-mediated reaction, PCA reaction and delayed hypersensitivity reaction were also determined. Contact dermatitis induced with DNCB was utilized as the delayed hypersensitivity reaction. The IgE-mediated reaction or PCA reaction, as well as simple serum irritation, enhanced the delayed hypersensitivity reaction. It is thus assumed that the IgE-mediated reaction enhances the PCA reaction and that this in turn accelerates the delayed hypersensitivity reaction.

Early clinical manifestations and radiologic features of tuberous sclerosis were studied in 18 consecutive patients encountered in the past two years, including two patients harboring an associated intraventricular tumor (giant-cell astrocytoma). Depigmented naevi rather than adenoma sebaceum, infantile spasms and intracranial calcifications were the cardinal early features in this present series, and CT scanning proved to be the single most useful diagnostic technique in the early detection of intracranial calcifications and therefore for the early diagnosis of this disorder. CT scanning was also useful in detecting the intraventricular tumors in the early stage before they became clinically manifest.

To clarify the immunological mechanisms in poststreptococcal acute glomeulonephritis (PSAGN) and anaphylactoid purpura (AP), anti-streptococcal cell membrane (anti-SCM) and anti-human glomerular basement membrane (anti-GBM) titers in the sera of patients with PSAGN and AP were determined by passive hemagglutination with chromic chloride-treated sheep erythrocytes. Sodium lauryl sulfate (SLS) soluble SCM and collagenase soluble GBM were used as soluble antigens. Positive anti-SCM titers (greater than 1:8) were demonstrated in 10 of 14 patients (71.4%) with PSAGN and in 4 of 9 patients (44.4%) with AP with evidence of antecedent streptococcal infection. Two of 4 patients with AP without evidence of antecedent streptococcal infection had positive anti-SCM titers. No correlation was noted between anti-streptolysin O (ASO) titers and anti-SCM titers in patients with PSAGN or AP, but many patients with high ASO titers also had high anti-SCM titers. No positive anti-GBM reactions were detected in patients with PSAGN or AP. No cross-reactions were noted between SLS soluble SCM and collagenase soluble GBM.

Five children who have evidence of residual nephritis after an episode of acute poststreptococcal glomerulonephritis have been investigated to determine the relationship of their disease to membranoproliferative nephritis. Measurements of serum β 1 C-globulin and study of late renal biopsies showed no changes suggestive of membranoproliferative disease. Serum β 1 levels were normal, and silver staining of renal biopsies gave no evidence of deposition of large quantities of nonargyrophilic material typically observed in membranoproliferative nephritis.