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Arch Dis Child 1998;78:408-412 ( May )

A prospective 10 year follow up study of patients with neurofibromatosis type 1

M H Cnossen,a A de Goede-Bolder,a K M van den Broek,a C M E Waasdorp,a A P Oranje,c H Stroink,d H J Simonsz,e A M W van den Ouweland,b D J J Halley,b M F Niermeijerb

a Department of Paediatrics, University Hospital Sophia/Dijkzigt and Erasmus University Rotterdam, 3015 GJ Rotterdam, Netherlands, b Department of Clinical Genetics, University Hospital Sophia/Dijkzigt, c Department of Dermatology and Venerology, University Hospital Sophia/Dijkzigt, d Department of Paediatric Neurology, University Hospital Sophia/Dijkzigt, e Department of Ophthalmology, University Hospital Sophia/Dijkzigt

Correspondence to: Dr Cnossen. email: cnossen{at}alkg.azr.nl


Accepted 12 January 1998

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.
DESIGN---A 10 year prospective multidisciplinary follow up study.
PATIENTS---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.
CONCLUSION---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.

Keywords: neurofibromatosis type 1; genetic disorders

© 1998 by Archives of Disease in Childhood


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