TY - JOUR
T1 - Precocious puberty in children with neurofibromatosis type 1
AU - Habiby, Reema
AU - Silverman, Bernard
AU - Listernick, Robert
AU - Charrow, Joel
N1 - Funding Information:
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.
Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.
PY - 1995/3
Y1 - 1995/3
N2 - We undertook a comprehensive study of children with neurofibromatosis type 1 (NF-1) cared for in a large multidisciplinary clinic to determine the prevalence of precocious puberty and its relationship to optic pathway tumors (OPTs). Precocious puberty was diagnosed in 7 of 219 children with NF-1 (5 boys and 2 girls) examined between Jan. 1, 1985, and April 20, 1993. All seven children had OPTs involving the optic chiasm; they represented 39% of children with NF-1 and chiasmal tumors (95% confidence interval, 17% to 64%). Eleven prepubertal children (aged 2 to 10 years) with NF-1 and OPTs, and age- and sex-matched NF-1 control subjects without OPTs, underwent luteinizing hormone-releasing hormone (LH-RH) stimulation tests. Two boys with OPTs had pubertal luteinizing hormone (LH) responses, and testosterone levels >10 ng/dl. Basal LH levels were also elevated in these two boys when tested with a very sensitive immunochemiluminometric assay. None of the children without an OPT had either a pubertal response to LH-RH or an elevated basal LH level. We conclude that precocious puberty in children with NF-1 is found exclusively in those who have OPTs involving the optic chiasm; it is a common complication in those children. With the use of a highly sensitive LH assay, biochemical evidence of hypothalamic- pituitary-gonadal axis activation may be demonstrated, even without provocative testing. (J PEDIATR 1995;126:364-7).
AB - We undertook a comprehensive study of children with neurofibromatosis type 1 (NF-1) cared for in a large multidisciplinary clinic to determine the prevalence of precocious puberty and its relationship to optic pathway tumors (OPTs). Precocious puberty was diagnosed in 7 of 219 children with NF-1 (5 boys and 2 girls) examined between Jan. 1, 1985, and April 20, 1993. All seven children had OPTs involving the optic chiasm; they represented 39% of children with NF-1 and chiasmal tumors (95% confidence interval, 17% to 64%). Eleven prepubertal children (aged 2 to 10 years) with NF-1 and OPTs, and age- and sex-matched NF-1 control subjects without OPTs, underwent luteinizing hormone-releasing hormone (LH-RH) stimulation tests. Two boys with OPTs had pubertal luteinizing hormone (LH) responses, and testosterone levels >10 ng/dl. Basal LH levels were also elevated in these two boys when tested with a very sensitive immunochemiluminometric assay. None of the children without an OPT had either a pubertal response to LH-RH or an elevated basal LH level. We conclude that precocious puberty in children with NF-1 is found exclusively in those who have OPTs involving the optic chiasm; it is a common complication in those children. With the use of a highly sensitive LH assay, biochemical evidence of hypothalamic- pituitary-gonadal axis activation may be demonstrated, even without provocative testing. (J PEDIATR 1995;126:364-7).
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U2 - 10.1016/S0022-3476(95)70449-3
DO - 10.1016/S0022-3476(95)70449-3
M3 - Article
C2 - 7869193
AN - SCOPUS:0028915191
SN - 0022-3476
VL - 126
SP - 364
EP - 367
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 3
ER -