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[Neurofibromatosis type 1 complications in the pediatric age: follow-up of a hundred cases].

Authors :
Bonnemaison E
Roze-Abert B
Lorette G
Sirinelli D
Boscq M
Mazjoub S
De Courtivron B
Bonnard C
Despert F
Toutain A
Maheut-Lourmière J
Barthez MA
Castelnau P
Source :
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie [Arch Pediatr] 2006 Jul; Vol. 13 (7), pp. 1009-14. Date of Electronic Publication: 2006 May 22.
Publication Year :
2006

Abstract

Unlabelled: Neurofibromatosis 1 (NF1) is a frequent genetic disease. Diagnostic criterias were established in 1988. The patients can exhibit various and unpredictable complications.<br />Objectives: To check the efficiency of a coordinated follow-up in specialized multidisciplinary centers providing a higher quality of management and to have a better knowledge of the complications including their true frequencies.<br />Population and Methods: We report a serie of 100 NF1 children who were followed-up during 4 years in a specialized center at the Tours University Hospital. Three hospital check-up at 2-5, 6-7, 14-15 years of age were performed as well as an annual physical examination.<br />Results: In our serie, the mean age was 7.8 years old with a sex ratio of 1. The mean age at diagnosis was 3.8 years old and the main diagnosis criteria were the café-au-lait spots and the family history for 80% of the patients. The optic nerve glioma has a low frequency of 5%. Learning disabilities clearly represent the most frequent complication (46% of the patients).<br />Conclusion: An early detection of these difficulties is a priority for the appropriate management of these children.

Details

Language :
French
ISSN :
0929-693X
Volume :
13
Issue :
7
Database :
MEDLINE
Journal :
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
Publication Type :
Academic Journal
Accession number :
16716577
Full Text :
https://doi.org/10.1016/j.arcped.2006.03.149