Neurofibromatosis 1 (NF1) is a common autosomal dominant disorder in which affected individuals develop benign and malignant tumors [ ll. Because of the association between NF 1 and tumor development, the NFI gene has been classified as a tumor suppressor gene [2]. The NFI gene was identified in 1990 and found to code for a large cytoplasmic protein of 2,818 acids [3-51. The NFI gene product, neurofibromin, contains a small region in the central part of the protein that bears sequence similarity with a family of proteins that regulate the protooncogene p21-ras [6][7][8][9]. These p2 1 -ras regulatory proteins are collectively termed guanosine triphosphatase (GTPase)-activating proteins (GAPS). Since p21-ras can transform cells, neurofibromin (as an inhibitor of p21-ras activation) would therefore suppress the growth of cells. This association between NFl GAP function and p21-ras regulation has been demonstrated for some tumors in patients with NF1 [lo-121. One of the most common tumors in patients with NF1 is the optic pathway glioma. Although optic pathway tumors account for only 2 to 5% of all brain tumors in childhood, as many as 70% are associated with NF1. While these tumors generally behave in a benign fashion, optic pathway gliomas can lead to visual loss or extend to nearby brain regions and result in other signs and symptoms. In October 1995, a steering committee was designated by the National Neurofibromatosis Foundation and the Cold Spring Harbor Laboratory to address common clinical problems in NF1. Its goals were to create task forces composed of both basic science and clinical researchers who had worked extensively in various areas of research in NF1. These task forces were to review and critique the known information on these specific targeted areas, to provide rational guidelines for the care of NFl patients, and to establish the basis for future clinical trials. This consensus statement, the result of work performed by the Optic Glioma Task Force, outlines recommendations for the screening, follow-up, and treatment of optic pathway gliomas in children with NF 1. TerminologyOptic glioma has been used widely as a diagnostic term for low-grade astrocytic gliomas arising in the optic nerves. More recently, in recognition of the involvement of other regions of the optic system, the terms optic pathway glioma and visual pathway glioma have gained acceptance. Nonetheless, these terms are imprecise because the term glioma encompasses a wide variety of biologically and clinically distinct lesions. The vast majority of optic pathway gliomas, including the tumors that arise in NF1 patients, are pilocytic astrocytomas. Indeed, the World Health Organization (WHO) classification of central nervous system tumors and the major brain tumor reference texts do not recognize optic pathway glioma as a separate pathological entity, but include these lesions under the rubric of pilocytic astrocytoma [ 13-1 61. Although not well entrenched in the literature, the most accurate term for these tumors is pilocytic artrocy...

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Identification of new optic pathway tumors (OPTs) and progression of pre-existing OPTs in children with neurofibromatosis 1 (NF1) have been reported infrequently after age 6. The authors present eight children with NF1 (mean age 12.2 years) seen in three NF1 centers who had either late-onset (four of eight) or late-progressive (seven of eight) OPT. Continued monitoring of individuals with NF1 into adulthood for the development of OPTs and for progression of known OPTs is warranted.

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Abstract. Objective: To describe the rates of serious bacterial illness (SBI) in children presenting to emergency departments (EDs) with first-time uncomplicated febrile seizures. Methods: The ED visits from seven Chicago metropolitan area hospitals (two tertiary pediatric EDs, five community general EDs) for all pediatric patients seen between July 1995 and December 1997 with a discharge diagnosis including the term ''seizure'' were retrospectively identified. Records of patients who met criteria for simple, firsttime febrile seizure were reviewed (age 6-60 months; temperature Ն38.0ЊC; single, generalized, tonicclonic seizure <20 minutes; absence of known central nervous system disease). Rates of bacteremia, urinary tract infection, bacterial meningitis, and pneumonia were determined. Results: Four hundred fiftyfive children were identified who had first-time simple febrile seizures. The study participants had a mean age of 21 months and a mean temperature of 39.6ЊC, and 64% were male. Seventy-three percent were seen in a community hospital setting. Blood cultures were obtained for 315 children (69%). Four children (1.3% [95% CI = 0.1% to 2.5%]) were bacteremic, all with Streptococcus pneumoniae; the rate of bacteremia did not differ in the subset at highest risk for bacteremia (6-36 months, temperature >39ЊC). No demographic or laboratory data distinguished the bacteremic children from those with negative blood cultures. One hundred seventy-one children (38%) had urine cultures obtained; 5.9% [95% CI = 2.4% to 9.4%] of the cultures grew >100,000 colony-forming units/mL of a single pathogenic organism. One hundred thirty-five children (30%) had cerebrospinal fluid cultures performed. None of these cultures grew a bacterial pathogen [95% CI = 0% to 2.2%]. Two hundred eight children (45.7%) had chest x-rays performed; 12.5% [95% CI = 10.2% to 14.8%] (n = 26) of the x-rays were read as consistent with pneumonia by the radiologist at the treating institution. None of the blood cultures performed on children with abnormal radiographs were positive (cultures drawn on 23 of 26 patients, 88%). Stool cultures were performed on 14 children (3.1%); two cultures (14.3% [95% CI = 0% to 32.6%]) grew a bacterial pathogen, both Shigella. Conclusions: Rates of SBI in this multi-institution population of children with first-time simple febrile seizures were low and are consistent with those published in the literature for febrile children without seizures. Key words: febrile seizure; bacteremia; urinary tract infection; meningitis. ACA-DEMIC EMERGENCY MEDICINE 2001; 8:781-787 S IMPLE febrile seizures are the most common convulsive disorder of childhood and a frequent cause of visits to the emergency department (ED) in both community and tertiary care pediatric institutions. Historically, many emergency medicine providers feared that children who experienced a simple febrile seizure were more ''ill'' than equally febrile children without seizures and evaluated the children with simple febrile seizures more rigorously.1 Althoug...

Low grade gliomas affecting the visual pathway, commonly referred to as optic pathway gliomas (OPGs), have a relatively high survival rate but can cause significant vision loss. While previous treatment outcomes for tumors of the central nervous system have focused primarily on changes in tumor size or patient survival, more recently preservation of vision has also become a primary objective when treating these tumors. Visual acuity (VA) is the most testable and reliable visual parameter in young children with OPGs. Unfortunately, standardized VA assessments have neither been employed to make treatment decisions nor used as primary outcomes in clinical trials. The lack of a standardized VA assessment has also hindered the ability to interpret and compare results between studies. It is essential that all members of the multidisciplinary care team (i.e., pediatric neuro-oncologist, neurologist, neurosurgeon, and ophthalmologist) can accurately interpret VA results and properly use them to guide management decisions. Specifically, determining what constitutes a significant change in VA and the factors that may influence these results should be incorporated into collective team recommendations. This review describes the VA assessment in children with OPGs and proposes a standardized VA testing protocol for future pediatric OPG clinical treatment trials.

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