A prospective 10 year follow up study of patients with neurofibromatosis type 1.
OBJECTIVE
To establish the prevalence and incidence of symptoms and complications in children with neurofibromatosis type 1 (NF1) and to assess possible risk factors for the development of complications.


METHODS
A 10 year prospective multidisciplinary follow up study.


METHODS
One hundred and fifty children diagnosed with NF1 according to criteria set by the National Institutes of Health.


RESULTS
In 62 of 150 children (41.3%) complications were present, including 42 (28.0%) children with one complication, 18 (12.0%) with two complications, and two (1.3%) with three complications (mean (SD) duration of follow up 4.9 (3.8) years). Ninety five of the 150 children presented without complications (follow up, 340.8 person-years). The incidence of complications was 2.4/100 person-years in this group. An association was found between behavioural problems and the presence of complications.


CONCLUSIONS
This is the largest single centre case series of NF1 affected children followed until 18 years of age. Children with NF1, including those initially presenting without complications, should have regular clinical examinations.
Cnossen
M H
MH
Department of Paediatrics, University Hospital Sophia/Dijkzigt, Rotterdam, Netherlands. cnossen@alkg.azr.nl
de Goede-Bolder
A
A
van den Broek
K M
KM
Waasdorp
C M
CM
Oranje
A P
AP
Stroink
H
H
Simonsz
H J
HJ
van den Ouweland
A M
AM
Halley
D J
DJ
Niermeijer
M F
MF
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000209:etiology; D006801:Humans; D015994:Incidence; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D018318:Neurofibroma, Plexiform / Q000209:etiology; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D019574:Optic Nerve Neoplasms / Q000209:etiology; D015995:Prevalence; D011446:Prospective Studies