Survivals of young children with malignant brain tumors are worse than any other age group and they also suffer the brunt of radiation-induced neurotoxicity. As such, the Pediatric Oncology Group (POG) in 1986 mounted a group-wide study in which children <3 years of age with malignant brain tumors were treated with prolonged postoperative chemotherapy (vincristine, cyclophosphamide, cisplatinum and VP16) in an effort to delay radiation for two years in children <2 years of age at diagnosis and for one year in children 2–3 years. “Baby POG” became the basis of all U.S. cooperative group infant brain tumor studies over the following 20 years. By 2006, over 1000 infants had been treated. Prognostic factors identified by Baby POG 1 were: 1) supratentorial PNETs had the worst prognosis, 2) age did not significantly impact survival, 3) delay in radiation from 1 to 2 years only affected survival in ependymomas, 4) groß total resection (GTR) significantly improved survival, 5) metastases did not significantly worsen outcome, 6) those with GTR and no metastases had the best outcome.

Objectives: Many case series have documented prothrombotic abnormalities in children with presumed perinatal stroke. These studies, however, have not included controls. We set out to document the association between genetic polymorphisms for thrombophilia and perinatal stroke using a case-control methodology.

PURPOSE: To evaluate prospectively the effects on survival, relapse-free survival, and patterns of relapse of reduced-dose (23.4 Gy in 13 fractions) compared with standard-dose (36 Gy in 20 fractions) neuraxis irradiation in patients 3 to 21 years of age with low-stage medulloblastoma, minimal postoperative residual disease, and no evidence of neuraxis disease. PATIENTS AND METHODS: The Pediatric Oncology Group and Children’s Cancer Group randomized 126 patients to the study. All patients received posterior fossa irradiation to a total dose of 54 Gy in addition to the neuraxis treatment. Patients were staged postoperatively with contrast-enhanced cranial computed tomography, myelography, and CSF cytology. Of the registered patients, 38 were ineligible. RESULTS: The planned interim analysis that resulted in closure of the protocol showed that patients randomized to the reduced neuraxis treatment had increased frequency of relapse. In the final analysis, eligible patients receiving standard-dose neuraxis irradiation had 67% event-free survival (EFS) at 5 years (SE = 7.4%), whereas eligible patients receiving reduced-dose neuraxis irradiation had 52% event-free survival at 5 years (SE = 7.7%) (P = .080). At 8 years, the respective EFS proportions were also 67% (SE = 8.8%) and 52% (SE = 11%) (P = .141). These data confirm the original one-sided conclusions but suggest that differences are less marked with time. CONCLUSION: Reduced-dose neuraxis irradiation (23.4 Gy) is associated with increased risk of early relapse, early isolated neuraxis relapse, and lower 5-year EFS and overall survival than standard irradiation (36 Gy). The 5-year EFS for patients receiving standard-dose irradiation is suboptimal, and improved techniques and/or therapies are needed to improve ultimate outcome. Chemotherapy may contribute to this improvement.

We tested the precision and accuracy of the AccuLevel Phenobarbital Test, which reports whole-blood (fingerstick) results in plasma equivalent values, and compared these values with plasma results obtained using established methods. We conclude that the assay is precise, reliable, accurate for single tests, and is appropriate for use in offices, outpatient settings, and emergency rooms.

Multicore disease in identical twin boys presented in infancy as generalized weakness and torticollis. Motor milestones such as sitting, standing, and running were delayed, although by the age of 6 years marked improvement in muscle strength had occurred. Serum enzymes were normal. Muscle biopsy revealed multifocal areas of decreased oxidative enzyme activity. Ultrastructurally, these areas were characterized by myofilament disruption and Z-band streaming.