[Neurofibromatosis type 1 complications in the pediatric age: follow-up of a hundred cases].
Neurofibromatosis 1 (NF1) is a frequent genetic disease. Diagnostic criterias were established in 1988. The patients can exhibit various and unpredictable complications.


OBJECTIVE
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.


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.


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).


CONCLUSIONS
An early detection of these difficulties is a priority for the appropriate management of these children.
Bonnemaison
E
E
Service de neurologie et neurochirurgie pédiatrique, centre de pédiatrie Gatien-de-Clocheville, CHRU de Tours, 49, boulevard Béranger, 37044 Tours cedex 01, France.
Roze-Abert
B
B
Lorette
G
G
Sirinelli
D
D
Boscq
M
M
Mazjoub
S
S
De Courtivron
B
B
Bonnard
C
C
Despert
F
F
Toutain
A
A
Maheut-Lourmière
J
J
Barthez
M-A
MA
Castelnau
P
P
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007859:Learning Disabilities / Q000209:etiology; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis*