FOCUS QUESTIONS 1. What is our understanding of the pathophysiology of Henoch. Schoenlein purpura (HSP)? 2. What are the cardinal clinical features of HSP? 3. Which clinical features offer substantial threat to survival or full recovery from HSP? 4. What clinical or laboratory observations are required for or appropilate to monitoring the course of HSP? 5. What therapeutic measures are available for HSP, and what clinical features direct their choice and use?

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We reviewed the records of 127 consecutive pediatric patients with acute lymphoblastic leukemia (ALL) to determine the incidence, timing, etiologies, and recurrence rate of seizures in this population. Patients with ALL and seizures were identified retrospectively by review of the records of all pediatric ALL patients who were diagnosed and treated during the years 1983 through March 1993 in a large tertiary-care hospital. Seventeen patients (13%) developed one or more seizures. In 16 patients, seizures occurred during antileukemic treatment, and in almost all of them seizures were related to intrathecal methotrexate (IT MTX) or subcutaneous L-asparaginase treatment. One patient who developed a seizure while not receiving chemotherapy had a history of cerebral infarctions. In 8 patients, (47%), the initial seizure episode was associated with a cerebral lesion. One or more seizures recurred in 6 patients. Four of these patients had an isolated recurrence, in 3 patients < or = 3 months and in 1 patient < or = 6 months after the initial event. Two patients (12%) with static encephalopathy and neurological deficits developed a chronic seizure disorder. There is a significant risk of acute symptomatic seizures in pediatric ALL patients. Most seizures in these patients occur during the acute treatment phase and are most frequently related to side effects of chemotherapy. The long-term recurrence risk is low; recurrence occurs most often in patients with evidence of cerebral structural lesions and neurological deficits. Long-term antiepileptic drug (AED) therapy should be restricted to such patients.

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Tumor specimens from 33 patients with neuroblastoma were assayed for amplification of the N-myc oncogene and RNA expression to determine whether N-myc RNA expression levels correlated with N-myc gene amplification and clinical outcome. N-myc gene amplification was detected in one stage II tumor, one stage IV-S tumor, and seven stage III or IV tumors. In each case, N-myc RNA expression roughly paralleled N-myc gene amplification. However, enhanced N-myc RNA expression was not confined to tumors with N-myc gene amplification: all of the early (stage I and II) tumors, five stage IV-S tumors, and 12 advanced (stage III and IV) tumors had levels of N-myc RNA that were elevated up to 50-fold. While N-myc gene amplification correlated with prognosis, there was no such correlation with levels of N-myc RNA expression. The precise role of the N-myc gene in the pathogenesis of neuroblastoma remains unclear.

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The efficacy of antibiotic treatment of port-associated bloodstream infection without device removal has not been systematically studied. We analyzed the outcome of 43 consecutive portassociated bloodstream infections in pediatric hematology-oncology patients. Etiologies included Staphylococcus epidermidis (30) and Staphylococcus aureus (6). Antibiotics were given through the port for a median of 11 days. Four ports were removed within 72 hours. In 36 (92%) of the remaining 39 episodes, there was a response to antibiotic therapy (defervescence and negative blood culture). In 78% of episodes in which there was a response (excluding two in which the catheters were removed because of mechanical problems), the infections were cured without port removal. Two of the four relapses were cured with a second course of antibiotics. The cure rate was 92% for S. epidermidis infections and 67% for S. aureus infections. Thus, the majority of port-associated bloodstream infections in pediatric hematology-oncology patients can be cured without device removal.There are two types of surgically placed, subcutaneously tunneled Silastic (Dow Corning, Midland, MI) central venous catheters designed for long-term use. Broviac-or Hickmantype catheters exit through the skin after being passed through a subcutaneous tunnel, and totally implantable venous access ports ("ports") end in a subcutaneous reservoir after being passed through a subcutaneous tunnel (as, for example, with Mediport [Norfolk, Medina, NY] and Port-A-Cath [Pharmacia Deltec, St. Paul] ports). Ports are accessed by means of a special needle through intact skin. In pediatric oncology patients, ports are associated with a lower rate of catheterrelated bloodstream infection than are Broviac/Hickman catheters [1][2][3][4].Although cure of bloodstream infections related to nontunneled vascular catheters generally requires catheter removal, we and others have found that ϳ75% of Broviac/Hickman catheter-related bloodstream infections are cured without catheter removal [3][4][5][6]. In contrast, there are limited data concerning the efficacy of antibiotic treatment of port-associated bloodstream infection without port removal. To determine the advisability of initial antibiotic therapy without device removal, we studied the outcome of consecutive catheter-related bloodstream infections occurring in pediatric hematologyoncology patients with totally implantable venous access ports. MethodsPatient population. All pediatric hematology-oncology patients cared for at the Schneider Children's Hospital (New Hyde Park, NY) between 1 January 1991 and 31 December 1993 were identified through a log book of all patients seen by the division of pediatric hematology/oncology. Patients with a port were identified by review of the comprehensive flow sheets maintained for each patient by that division, on which data on all surgically placed vascular catheters, hospital admissions, infections, courses of antibiotic therapy, and vascular catheter removal are tabulated prospectively.To ensure that a...