David L. Becton

Serotype-specific immunoglobulin G antibody responses to pneumococcal polysaccharide vaccine in children with sickle cell anemia: Effects of continued penicillin prophylaxis

OBJECTIVES (1) To determine serotype-specific IgG antibody responses to reimmunization with pneumococcal polysaccharide vaccine at age 5 years in children with sickle cell anemia and (2) to determine whether continued penicillin prophylaxis had any adverse effects on these responses. STUDY DESIGN Children with sickle cell anemia, who had been treated with prophylactic penicillin for at least 2 years before their fifth birthday, were randomly selected at age 5 years to continue penicillin prophylaxis or to receive placebo treatment. These children had been immunized once or twice in early childhood with pneumococcal polysaccharide vaccine and were reimmunized at the time of randomization. RESULTS Serotype-specific IgG antibody responses to reimmunization varied according to pneumococcal serotype but in general were mediocre or poor; the poorest response was to serotype 6B. The antibody responses were similar in subjects with continued penicillin prophylaxis or placebo treatment, and in subjects who received one or two pneumococcal vaccinations before reimmunization. The occurrence of pneumococcal bacteremia was associated with low IgG antibody concentrations to the infecting serotype. CONCLUSIONS Reimmunization of children with sickle cell anemia who received pneumococcal polysaccharide vaccine at age 5 years induces limited production of serotype-specific IgG antibodies, regardless of previous pneumococcal vaccine history. Continued penicillin prophylaxis does not interfere with serotype-specific IgG antibody responses to reimmunization.

Severe neutropenia caused by copper deficiency in a child receiving continuous ambulatory peritoneal dialysis

Changes in trace element concentrations in patients with uremia receiving dialysis have been investigated extensively in recent years. ~-~ The most consistently observed abnormality is an accumulation of aluminum; serum zinc and copper levels have been found to be normal, increased, or decreased. A recent report described low levels of red cell copper in adult patients receiving continuous ambulatory peritoneal dialysis? We report an adolescent with cystinosis who became copper deficient, with neutropenia and an increased red cell transfusion requirement, several months after beginning CAPD.

9p− in a girl with acute lymphocytic leukemia and sickle cell disease☆

A 4 year old girl with sickle cell disease developed acute lymphocytic leukemia null cell type. The bone marrow karyotype was 46,XX,del(9)(p13). This girl is among the few patients with acute lymphocytic leukemia and abnormalities of #9 to have an isolated 9p-.

Severe Mycoplasma Pneumonia in Three Sisters with Sickle Cell Disease

Mycoplasma pneumoniae, which usually causes mild infections in normal children, has been shown to cause isolated cases of severe pneumonia in children with sickle cell disease. We recently observed an outbreak of Mycoplasma pneumonia in three sisters with homozygous sickle cell disease. Two of them required hospitalization, and one progressed to respiratory failure requiring prolonged ventilatory assistance. All eventually recovered without long-term pulmonary complications. Familial outbreaks are not uncommon in Mycoplasma infection, but they have not been described previously in siblings with sickle cell disease. It is assumed that local or systemic host defense abnormalities predispose patients with sickling syndromes to more severe courses of Mycoplasma infections.

Bacterial endocarditis in a child with a Broviac catheter.

Broviac and Hickman catheters facilitate the care of children with cancer but provide a source of potential infection. We describe a child with a Broviac catheter who developed left-sided bacterial endocarditis in whom right-to-left atrial shunting was documented following catheter flushing. Following removal of the catheter and administration of prolonged intravenous antibiotics, recovery was complete and cardiac function returned to normal.

An infant girl with severe autoimmune hemolytic anemia: apparent anti-Vel specificity

Abstract. Autoantibodies directed against the common red‐cell antigen Vel are unusual, with only 2 previous cases reported. This report describes an infant girl with steroid‐resistant autoimmune hemolytic anemia whose serum contained an antibody with apparent specificity for Vel antigen. Transfusion with Vel‐positive cells resulted in a massive hemolytic transfusion reaction. Survival of labelled Vel‐negative cells was normal up to 8 h following transfusion, and no transfusion reaction followed subsequent use of Vel‐negative cells. There was no evidence of a collagen vascular disease, an immunodeficiency syndrome, or a malignancy. Although rare, anti‐Vel should be considered in unexplained autoimmune hemolytic anemia.