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Cohen R.,Schneider Childrens Medical Center
Harefuah | Year: 2010

Neurofibromatosis type 1 (NF1) is an autosomal dominant condition that primarily involves the skin and the nervous system. It affects about 1 in 4000 individuals. NF1 is caused by a mutation in the nfl gene located on chromosome 17q11.2. Neurofibromin, the protein products of the normal nf1 gene, acts as a tumor suppressor and limits cell growth. Mutation in this gene leads to cell overgrowth and an increased risk of developing benign and malignant tumor. The diagnosis of NF1 is made in an individual with any two of the following clinical features: café-au-lait spots, intertriginous freckling, Lisch nodules, neurofibromas, optic glioma, distinctive bone lesions and first degree family relative with NF1. Learning and developmental disorders are the most common neurologic complication of neurofibromatosis type 1 and can be responsible for significant lifetime morbidity. This report provides a review on cognitive and developmental manifestation of children with NF1 and the importance of early diagnosis and treatment. Source

Steinberg T.,Schneider Childrens Medical Center
The Israel journal of psychiatry and related sciences | Year: 2010

The object of this review is to summarize some of the recent developments in the understanding of Tourette's Syndrome which can be regarded as the prototype of a developmental psychopathological entity. The review covers the following topics: tics and their developmental course; sensory phenomena related to tics including measurement of these phenomena; pathophysiology of tics and compensatory phenomena and the parallel development of the various psychiatric comorbidities as they emerge over the life span. Finally there is an attempt to summarize the major points and future directions. Source

Ambrose C.S.,Med Immune Ltd. | Dubovsky F.,Med Immune Ltd. | Yi T.,Med Immune Ltd. | Belshe R.B.,Washington University in St. Louis | And 2 more authors.
European Journal of Clinical Microbiology and Infectious Diseases | Year: 2012

In the European Union and Canada, an Ann Arbor strain live attenuated influenza vaccine (LAIV) is approved for use in children aged 2-17 years, including those with mild to moderate asthma or prior wheezing. The safety and efficacy of LAIV versus trivalent inactivated influenza vaccine (TIV) in children with asthma aged 6-17 years have been demonstrated. However, few data are available for children younger than 6 years of age with asthma or prior wheezing. Safety and efficacy data were collected for children aged 2-5 years with asthma or prior wheezing fromtwo randomized, multinational trials of LAIV and TIV (N01,940). Wheezing, lower respiratory illness, and hospitalization were not significantly increased among children receiving LAIV compared with TIV. Increased upper respiratory symptoms and irritability were observed among LAIV recipients (p©0.05). Relative efficacies were consistent with the results observed in the overall study populations, which demonstrated fewer cases of cultureconfirmed influenza illness in LAIV compared with TIV recipients. Study results support the safety and efficacy of LAIVamong children aged 2-17 years with mild to moderate asthma or a history of wheezing. Data regarding LAIVuse are limited among individuals with severe asthma or active wheezing within the 7 days before vaccination. © The Author(s) 2012. Source

Kiratli P.O.,Hacettepe University | Tuncel M.,Hacettepe University | Bar-Sever Z.,Schneider Childrens Medical Center
Seminars in Nuclear Medicine | Year: 2016

Nuclear medicine has an important role in the management of many cancers in pediatric age group with multiple imaging modalities and radiopharmaceuticals targeting various biological uptake mechanisms. 18-Flourodeoxyglucose is the radiotracer of choice especially in patients with sarcoma and lymphoma. 18FDG-PET, for sarcoma and lymphomas, is proved to be superior to conventional imaging in staging and therapy response. Although studies are limited in pediatric population, 18FDG-PET/CT has found its way through international guidelines. Limitations and strengths of PET imaging must be noticed before adapting PET imaging in clinical protocols. Established new response criteria using multiple parameters derived from 18FDG-PET would increase the accuracy and repeatability of response evaluation. Current data suggest that I-123 metaiodobenzylguanidine (MIBG) remains the tracer of choice in the evaluation of neuroblastoma (NB) because of its high sensitivity, specificity, diagnostic accuracy, and prognostic value. It is valuable in determining the response to therapy, surveillance for disease recurrence, and in selecting patients for I-131 therapy. SPECT/CT improves the diagnostic accuracy and the interpretation confidence of MIBG scans. 18FDG-PET/CT is an important complementary to MIBG imaging despite its lack of specificity to NB. It is valuable in cases of negative or inconclusive MIBG scans and when MIBG findings underestimate the disease status as determined from clinical and radiological findings. F-18 DOPA is promising tracer that reflects catecholamine metabolism and is both sensitive and specific. F-18 DOPA scintigraphy provides the advantages of PET/CT imaging with early and short imaging times, high spatial resolution, inherent morphologic correlation with CT, and quantitation. Regulatory and production issues currently limit the tracer's availability. PET/CT with Ga-68 DOTA appears to be useful in NB imaging and may have a unique role in selecting patients for peptide receptor radionuclide therapy with somatostatin analogues. C-11 hydroxyephedrine PET/CT is a specific PET tracer for NB, but the C-11 label that requires an on-site cyclotron production and the high physiologic uptake in the liver and kidneys limit its use. I-124 MIBG is useful for I-131 MIBG pretherapeutic dosimetry planning. Its use for diagnostic imaging as well as the use of F-18 labeled MIBG analogues is currently experimental. PET/MR imaging is emerging and is likely to become an important tool in the evaluation. It provides metabolic and superior morphological data in one imaging session, expediting the diagnosis and lowering the radiation exposure. Radioactive iodines not only detect residual tissue and metastatic disease but also are used in the treatment of differentiated thyroid cancer. However, these are not well documented in pediatric age group like adult patients. Use of radioactivity in pediatric population is very important and strictly controlled because of the possibility of secondary malignities; therefore, management of oncological cases requires detailed literature knowledge. This article aims to review the literature on the use of radionuclide imaging and therapy in pediatric population with thyroid cancer, sarcomas, lymphoma, and NB. © 2016 Elsevier Inc. All rights reserved. Source

Tovia E.,Pediatric Neurology Unit | Goldberg-Stern H.,Schneider Childrens Medical Center | Ben Zeev B.,Sheba Medical Center | Heyman E.,Assaf Harofe Medical Center | And 3 more authors.
Epilepsia | Year: 2011

Purpose: Benign childhood epilepsy with centrotemporal spikes (BCECTS) is the most common epileptic syndrome in childhood. The outcome is usually excellent, but there are some atypical forms of BCECTS with less favorable outcomes. The aim of this study was to delineate the frequency of these atypical features among patients with BCECTS. Methods: We conducted a retrospective chart study by retrieving the medical records of all consecutive patients with BCECTS who were evaluated in four pediatric neurology outpatient clinics in Israel between the years 1991 and 2008. Key Findings: A total of 196 patients with BCECTS were identified (78 female and 118 male; mean age at time of diagnosis 7.64 years, range 1.5-14). The mean duration of follow-up was 4.43 years (range 2-11). Nine patients (4.6%) developed electrical status epilepticus in slow waves sleep (ESES) during follow-up, four (2%) had Landau-Kleffner syndrome, three (1.5%) had BCECTS with frequent refractory seizures, two (1%) had BCECTS with falls at presentation, one (0.5%) had a "classic" atypical variant, and one (0.5%) had oromotor dysfunction. None had rolandic status epilepticus. Sixty-one patients (31%) had attention deficit hyperactivity disorder (ADHD), 43 (21.9%) had specific cognitive deficits, and 23 (11.7%) had behavioral abnormalities, including aggressiveness, anxiety disorders, depression, and pervasive developmental disorder (PDD). Significance: The prevalence of most atypical forms of BCECTS other than ESES is low. There is, however, a high prevalence of ADHD and specific cognitive deficits among patients with BCECTS. © 2011 International League Against Epilepsy. Source

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