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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.

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.

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.