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Brain gliomas, hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F19%3A10398622" target="_blank" >RIV/00064203:_____/19:10398622 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/19:10398622

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=IIucwY_TM" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=IIucwY_TM</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1016/j.braindev.2019.04.003" target="_blank" >10.1016/j.braindev.2019.04.003</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Brain gliomas, hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1

  • Original language description

    Purpose: To evaluate the incidence and clinical importance of brain gliomas - optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis. Subjects and methods: We analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery. Results: We have found OPGs in 77/285 (27%) children and GOOPs in 29/285 (10,2%) of NF1 children, of who 19 had OPG and GOOP together, so the total number of brain glioma was 87/285 (30,5%). GOOPs were significantly more often treated than OPGs (p &gt; 0.01). OPGs contain clinically important subgroup of 14/285 (4.9%) spreading to hypothalamus. Spontaneous regression was documented in 4/285 (1.4%) gliomas and the same number of NF1 children died due to gliomas. Obstructive hydrocephalus was found in 22/285 (7.7%) patients and 14/22 cases were due to glioma. Idiopathic aqueduct stenosis caused hydrocephalus in 6/22 cases and was found in 2.1% of NF1 children. Two had other cause. Conclusions: The total brain glioma number (OPGs and only GOOPs together) better reflected the overall brain tumour risk for NFI children. However, GOOPs occur less frequently than OPGs, they are more clinically relevant. The obstructive hydrocephalus was severe and featuring frequent complication, especially those with GOOP. Idiopathic aqueduct stenosis shows an unpredictable cause of hydrocephalus in comparison with glioma and is another reason for careful neurologic follow up. (C) 2019 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30103 - Neurosciences (including psychophysiology)

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2019

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Brain &amp; Development

  • ISSN

    0387-7604

  • e-ISSN

  • Volume of the periodical

    41

  • Issue of the periodical within the volume

    8

  • Country of publishing house

    NL - THE KINGDOM OF THE NETHERLANDS

  • Number of pages

    13

  • Pages from-to

    678-690

  • UT code for WoS article

    000488144000004

  • EID of the result in the Scopus database

    2-s2.0-85070900855