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J Med Genet. 2000 Dec;37(12):933-8.

Growth in North American white children with neurofibromatosis 1 (NF1).

Author information

1
Department of Medical Genetics, The University of British Columbia, 222-6174 University Boulevard, Vancouver, BC V6T 1Z3, Canada. szudek@unixg.ubc.ca

Abstract

OBJECTIVE:

To analyse the distributions of and generate growth charts for stature and occipitofrontal circumference (OFC) in neurofibromatosis 1 (NF1) patients.

DESIGN:

Cross sectional database survey.

SETTING:

The National Neurofibromatosis Foundation International Database (NFDB) includes clinical information on NF1 patients from 14 participating centres in North America.

SUBJECTS:

A total of 569 white, North American, NF1 patients, 55% female and 45% male.

MAIN OUTCOME MEASURES:

Stature and OFC measurements of NF1 patients were compared to age and sex matched population norms using z score standardisation and centile curves.

RESULTS:

The distributions of stature and OFC are shifted and unimodal among NF1 patients; 13% of patients have short stature (>/=2 standard deviations below the population mean) and 24% have macrocephaly (OFC >/=2 standard deviations above the population mean).

CONCLUSIONS:

Alterations of stature and OFC are not limited to NF1 patients with frank short stature or macrocephaly.

PMID:
11106357
PMCID:
PMC1734506
[Indexed for MEDLINE]
Free PMC Article
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