[Puberty and growth in children and adolescents with neuro fibromatosis type 1].

BACKGROUND

This study evaluates anthropometric and pubertal development parameters in children and adolescents with sporadic and familial occurrence of neurofibromatosis type 1 (NF-1).

METHODS

The study involved 23 patients (15 boys, 8 girls) with sporadic (n=12) and familial NF-1(7 affected mothers and 4 fathers), with average age of 12 years (range 5-20 years). All patients answered to a standard questionnaire including family and personal data and, following, they underwent systematized physical examination, with special attention to anthropometric data, dermatologic and pubertal stage according to Tanner's criteria evaluation.

RESULTS

Short stature was detected in a 17 year-old patient, 148.5cm height, with score Z of -4.16 and being the even outside of the family conduit. Three male patients had head circumference above of + 2DP from on average for the age. It was not found no case of precocious or delayed puberty.

CONCLUSIONS

In our study, do not we find significant alterations regarding the stature and pubertal development.