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 Niermeijer^b

^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

OBJECTIVETo 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.
DESIGNA 10 year prospective multidisciplinary follow up study.
PATIENTSOne hundred and fifty children diagnosed with NF1 according to criteria set by the National Institutes of Health.
RESULTSIn 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.
CONCLUSIONThis 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
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Suenobu, S.-i., Akiyoshi, K., Maeda, T., Korematsu, S., Izumi, T. (2008). Clinical Presentation of Patients With Neurofibromatosis Type 1 in Infancy and Childhood: Genetic Traits and Gender Effects. J Child Neurol 23: 1282-1287 [Abstract]  
• Chung, E. M., Specht, C. S., Schroeder, J. W. (2007). From the Archives of the AFIP: Pediatric Orbit Tumors and Tumorlike Lesions: Neuroepithelial Lesions of the Ocular Globe and Optic Nerve. RadioGraphics 27: 1159-1186 [Abstract] [Full Text]  
• Ramanjam, V., Adnams, C., Ndondo, A., Fieggen, G., Fieggen, K., Wilmshurst, J. (2006). Clinical Phenotype of South African Children With Neurofibromatosis 1. J Child Neurol 21: 63-70 [Abstract]  
• Laithier, V., Grill, J., Le Deley, M.-C., Ruchoux, M.-M., Couanet, D., Doz, F., Pichon, F., Rubie, H., Frappaz, D., Vannier, J.-P., Babin-Boilletot, A., Sariban, E., Chastagner, P., Zerah, M., Raquin, M.-A., Hartmann, O., Kalifa, C. (2003). Progression-Free Survival in Children With Optic Pathway Tumors: Dependence on Age and the Quality of the Response to Chemotherapy--Results of the First French Prospective Study for the French Society of Pediatric Oncology. JCO 21: 4572-4578 [Abstract] [Full Text]  
• Czyzyk, E., Jozwiak, S., Roszkowski, M., Schwartz, R. A. (2003). Optic Pathway Gliomas in Children With and Without Neurofibromatosis 1. J Child Neurol 18: 471-478 [Abstract]  
• Khong, P.-L., Goh, W. H. S., Wong, V. C. N., Fung, C.-W., Ooi, G.-C. (2003). MR Imaging of Spinal Tumors in Children with Neurofibromatosis 1. Am. J. Roentgenol. 180: 413-417 [Abstract] [Full Text]  
• Rosser, T., Packer, R. J. (2002). Review Article : Neurofibromas in Children With Neurofibromatosis 1. J Child Neurol 17: 585-591 [Abstract]  
• Singhal, S, Birch, J M, Kerr, B, Lashford, L, Evans, D G R (2002). Neurofibromatosis type 1 and sporadic optic gliomas. Arch. Dis. Child. 87: 65-70 [Abstract] [Full Text]  
• POYHONEN, M (2000). A clinical assessment of neurofibromatosis type 1 (NF1) and segmental NF in northern Finland. J. Med. Genet. 37: 43e-43 [Full Text]