Precocious puberty in children with neurofibromatosis type 1☆,☆☆,★,★★
Section snippets
METHODS
The Children's Memorial Hospital Neurofibromatosis Clinic is a multidisciplinary clinic that provides consultation services to assist in the diagnosis and management of neurofibromatosis in children. All children are seen at least annually and are examined by a general academic pediatrician, a geneticist, and a pediatric ophthalmologist at each visit. Patients seen in this clinic between Jan. 1, 1985, and April 20, 1993, were the subjects of this study. NF-1 was diagnosed in each subject on the
RESULTS
Two hundred nineteen children with NF-1 were examined during the period of study, and precocious puberty was diagnosed in seven of them (3%). All seven children who had precocious puberty had abnormal LH-RH test results. Of the 219 children, 171 underwent neuroimaging. All the children with precocious puberty had tumors involving the optic chiasm (Table I); they represented 39% of children with chiasmal tumors (95% confidence interval, 17% to 64%). Fifty-seven percent of the children without
DISCUSSION
The main finding of this study is that precocious puberty was found exclusively in those children with NF-1 who had OPTs involving the optic chiasm. Previous reports of this association in the absence of OPTs may have erroneously included children with McCune-Albright syndrome, in whom café au lait spots and precocious puberty may also occur. Saxena3 reported two examples of precocious puberty occurring in NF-1 without tumors of the optic chiasm, but neither computed tomography nor magnetic
References (19)
- et al.
Neurofibromatosis type 1 in childhood
J PEDIATR
(1990) Type 1 neurofibromatosis and the pediatric patient
Curr Probl Pediatr
(1992)- et al.
The national history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study
J PEDIATR
(1994) - et al.
Optic gliomas in children with neurofibromatosis type 1
J PEDIATR
(1989) - et al.
Emergence of optic pathway gliomas in children with neurofibromatosis type 1 after normal neuroimaging results
J PEDIATR
(1992) Southwestern Internal Medicine Conference. New developments in the diagnosis and treatment of sexual precocity
Am J Med Sci
(1992)Endocrine manifestations of neurofibromatosis in children
Am J Dis Child
(1970)- et al.
Precocious puberty associated with neurofibromatosis and optic gliomas
Am J Dis Child
(1985) - et al.
Pubertas praecox in neurofibromatosis of the optic chiasm
Acta Neurochir Suppl (Wien)
(1979)
Cited by (0)
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From the Department of Pediatrics, Northwestern University Medical School, and the Divisions of Endocrinology, General Academic and Emergency Pediatrics, and Genetics, Children's Memorial Hospital, Chicago, Illinois
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Supported in part by the National Center of Research Resources and General Clinical Research Centers Program, RR00078, and a grant from the Children's Memorial Institute for Education and Research.
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Reprint requests: Joel Charrow, MD, Division of Genetics-59, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.
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0022-3476/95/$3.00 + 0 9/20/60907