Diagnostic delay in neurofibromatosis type 1

Eur J Pediatr. 1997 Jun;156(6):482-7. doi: 10.1007/s004310050644.

Abstract

Since 1985 a multidisciplinary team in the Sophia Children's University Hospital in Rotterdam provides diagnostic follow up and genetic counseling services for neurofibromatosis type 1 (NF1) patients and their families. Parents of 68 affected children as well as 24 affected parents were interviewed. Of the affected children, 50% and 33% of the affected adults were treated for symptoms related to NF1 before a specific diagnosis was made. Although the disease is fully penetrant by the age of 5 years, 35% of the affected children had not been diagnosed by this age. Parents stated a preference for early diagnosis of NF1. Diagnosis of NF1 did not seem to be a reason to refrain from having children. The general attitude towards prenatal diagnosis was positive; however few parents would actually terminate an affected pregnancy.

Conclusion: Overall delay in diagnosis of NF1 is significant. Knowledge of symptoms should make an early diagnosis possible with beneficial effects for the patient and family members.

MeSH terms

  • Adolescent
  • Adult
  • Attitude to Health*
  • Child
  • Child, Preschool
  • Consumer Behavior
  • Family Health*
  • Family Planning Services
  • Female
  • Genetic Counseling*
  • Humans
  • Infant
  • Male
  • Neurofibromatosis 1 / diagnosis*
  • Prenatal Diagnosis*
  • Time Factors