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Precocious puberty, growth hormone deficiency, and neurofibromatosis
166
Editorial correspondence
The Journal of Pediatrics January 1996
Reply
REFERENCES
1. Schibler A, Casaulta C, Kraemer R. Rationale of oscillatory breathing as chest physiotherapy performed by the Flutter in patients with cystic fibrosis (CF) [Abstract]. Pediatr Pulmonol 1992;suppl 8:301. 2. Casanlta Aebischer C, Frey U, Schibler A, Kraemer R. Efficacy of chest physiotherapy (CPT) (PEP mask versus Flutter) in patients with cystic fibrosis (CF) [Abstract]. Eur Respir J 1993;6(suppl 17):P0459.
Precocious puberty, growth hormone deficiency, and neurofibromatosis To the Editor: The article on the frequent coexistence of precocious puberty and neurofibromatosis by Habiby et al.1 highlights an underemphasized aspect of hypothalamic-pituitary pathology with important implications for the growth and development of children with this condition. Evaluation for the detection of potential growth hormone deficiency (GHD) should be carried out in all such children before therapeutic strategies are designed. Especially for children with optic pathway tumors, the hypothalamic-pituitary interruption that places them at risk of precocious puberty also increases their propensity for development of GHD. Among the children followed at the M. D. Anderson Cancer Center, 36 of 108 patients had optic gliomas or hamartoma; in this group, three of the four children with documented GHD also had precocious puberty. 2 Because precocious puberty is manifested as accelerated growth velocity and advancing bone age, the characteristic growth delay associated with GHD may be masked, causing a diagnostic delay of both conditions. Shortness is very common in both children and adults with neurofibromatosis,3 and although it rarely causes physiologic illness, it carries psychologic morbidity. Early detection and therapy of both precocious puberty and GHD is feasible and should optimize the genetic growth potential of children with neurofibromatosis. Rena Vassilopoulou-Sellin, MD Section of Endocrinology Unh,ersity of Texas M. D. Anderson Cancer Center Houston, TX 77030 9/35/69687
REFERENCES
1. Habiby R, Silverman B, Listeruick R, Charrow J. Precocious puberty in children with neurofibromatosis type 1. J PEDIA~ 1995; 126:364-7. 2. Vassilopoulou-Sellin R, Quintos MT, Woods D, Needle M, Klein MJ. Short stature in children and adults with neurofibromatosis. Pediatr Nurs 1995;21:149-53. 3. Riceardi VM. Neurofibromatosis. N Engl J Med 1981;305: 1617-27.
To the Editor: We agree that the diagnosis of growth hormone deficiency (GHD) in the face of precocious puberty (PP) can be challenging. In children who do not have neurofibromatosis type 1 (NF-1), the association between PP and GHD has been reported by Attie et al. 1 and Cara et al. 2, 3 With the exception of one patient with empty-sella syndrome2, 3 and one patient with severe head trauma and an extensive tear of the hypothalamus, 1 all of the patients had intracranial tumors previously treated with cranial irradiation and surgery. Vassilopoulou-Sellin et al.4 reported GHD in four children with NF- 1 who had received cranial irradiation for optic gliomas; in three of these children the diagnosis of GHD was complicated by the coexistence of PP. Brauner et al. 5 also reported PP and GHD in patients with optic pathway tumors (some of whom had NF-1). Only one of their patients had GHD before cranial irradiation, and it is not clear whether this patient also had NF- 1, PP, or both conditions. They concluded that optic gliomas may induce PP, but GHD is almost always the result of irradiation. In our study, none of the patients with NF-1 and optic pathway tumors received prior therapy with radiation or surgery. Neurofibromatosis type 1 with optic chiasm glioma is clearly a risk factor for the development of PP. In the absence of previous radiation therapy or surgery, however, it has not yet been established that these children have an increased frequency of GHD. Reema Habiby, MD Bernard Silverman, MD Division of Endocrinology Robert Listernick, MD Division of General Academic Pediatrics Joel Charrow, MD Division of Genetics Department of Pediatrics Northwestern University Medical School Children's Memorial Hospital Chicago, IL 60614 9/35/69688 REFERENCES
1. Attie KM, Nelson RR, Conte FA, Kaplan SL, Grumbach MM. The pubertal growth spurt in eight patients with true precocious puberty and growth hormone deficiency: evidence for a direct role of sex steroids. J Clin Endocrinol Metab 1990;71:975-83. 2. Cara JF, Burstein S, Cuttler L, Moll GW Jr, Rosenfield RL. Growth hormone deficiency impedes the rise in plasma insulin-like growth factor 1 levels associated with precocious puberty. J PEDIATR 1989;115:64-8. 3. Cara JF, Kreiter ML, Rosenfield RL. Height prognosis of children with true precocious puberty and growth hormone deficiency: effect of combination therapy with gonadotropinreleasing hormone agonist and growth hormone. J PEDtATR 1992;120:709-15. 4. Vassilopoulou-Sellin R, Quintos MT, Woods D, Needle M, Klein MJ. Short stature in children and adults with neurofihromatosis. Pediatr Nurs 1995;21:149-53. 5. Brauner R, Malandry F, Rappaport R, et al. Growth and endocrine disorders in optic glioma. Eur J Pediatr 1990;149:825-8.
Editorial correspondence
The Journal of Pediatrics January 1996
Reply
REFERENCES
1. Schibler A, Casaulta C, Kraemer R. Rationale of oscillatory breathing as chest physiotherapy performed by the Flutter in patients with cystic fibrosis (CF) [Abstract]. Pediatr Pulmonol 1992;suppl 8:301. 2. Casanlta Aebischer C, Frey U, Schibler A, Kraemer R. Efficacy of chest physiotherapy (CPT) (PEP mask versus Flutter) in patients with cystic fibrosis (CF) [Abstract]. Eur Respir J 1993;6(suppl 17):P0459.
Precocious puberty, growth hormone deficiency, and neurofibromatosis To the Editor: The article on the frequent coexistence of precocious puberty and neurofibromatosis by Habiby et al.1 highlights an underemphasized aspect of hypothalamic-pituitary pathology with important implications for the growth and development of children with this condition. Evaluation for the detection of potential growth hormone deficiency (GHD) should be carried out in all such children before therapeutic strategies are designed. Especially for children with optic pathway tumors, the hypothalamic-pituitary interruption that places them at risk of precocious puberty also increases their propensity for development of GHD. Among the children followed at the M. D. Anderson Cancer Center, 36 of 108 patients had optic gliomas or hamartoma; in this group, three of the four children with documented GHD also had precocious puberty. 2 Because precocious puberty is manifested as accelerated growth velocity and advancing bone age, the characteristic growth delay associated with GHD may be masked, causing a diagnostic delay of both conditions. Shortness is very common in both children and adults with neurofibromatosis,3 and although it rarely causes physiologic illness, it carries psychologic morbidity. Early detection and therapy of both precocious puberty and GHD is feasible and should optimize the genetic growth potential of children with neurofibromatosis. Rena Vassilopoulou-Sellin, MD Section of Endocrinology Unh,ersity of Texas M. D. Anderson Cancer Center Houston, TX 77030 9/35/69687
REFERENCES
1. Habiby R, Silverman B, Listeruick R, Charrow J. Precocious puberty in children with neurofibromatosis type 1. J PEDIA~ 1995; 126:364-7. 2. Vassilopoulou-Sellin R, Quintos MT, Woods D, Needle M, Klein MJ. Short stature in children and adults with neurofibromatosis. Pediatr Nurs 1995;21:149-53. 3. Riceardi VM. Neurofibromatosis. N Engl J Med 1981;305: 1617-27.
To the Editor: We agree that the diagnosis of growth hormone deficiency (GHD) in the face of precocious puberty (PP) can be challenging. In children who do not have neurofibromatosis type 1 (NF-1), the association between PP and GHD has been reported by Attie et al. 1 and Cara et al. 2, 3 With the exception of one patient with empty-sella syndrome2, 3 and one patient with severe head trauma and an extensive tear of the hypothalamus, 1 all of the patients had intracranial tumors previously treated with cranial irradiation and surgery. Vassilopoulou-Sellin et al.4 reported GHD in four children with NF- 1 who had received cranial irradiation for optic gliomas; in three of these children the diagnosis of GHD was complicated by the coexistence of PP. Brauner et al. 5 also reported PP and GHD in patients with optic pathway tumors (some of whom had NF-1). Only one of their patients had GHD before cranial irradiation, and it is not clear whether this patient also had NF- 1, PP, or both conditions. They concluded that optic gliomas may induce PP, but GHD is almost always the result of irradiation. In our study, none of the patients with NF-1 and optic pathway tumors received prior therapy with radiation or surgery. Neurofibromatosis type 1 with optic chiasm glioma is clearly a risk factor for the development of PP. In the absence of previous radiation therapy or surgery, however, it has not yet been established that these children have an increased frequency of GHD. Reema Habiby, MD Bernard Silverman, MD Division of Endocrinology Robert Listernick, MD Division of General Academic Pediatrics Joel Charrow, MD Division of Genetics Department of Pediatrics Northwestern University Medical School Children's Memorial Hospital Chicago, IL 60614 9/35/69688 REFERENCES
1. Attie KM, Nelson RR, Conte FA, Kaplan SL, Grumbach MM. The pubertal growth spurt in eight patients with true precocious puberty and growth hormone deficiency: evidence for a direct role of sex steroids. J Clin Endocrinol Metab 1990;71:975-83. 2. Cara JF, Burstein S, Cuttler L, Moll GW Jr, Rosenfield RL. Growth hormone deficiency impedes the rise in plasma insulin-like growth factor 1 levels associated with precocious puberty. J PEDIATR 1989;115:64-8. 3. Cara JF, Kreiter ML, Rosenfield RL. Height prognosis of children with true precocious puberty and growth hormone deficiency: effect of combination therapy with gonadotropinreleasing hormone agonist and growth hormone. J PEDtATR 1992;120:709-15. 4. Vassilopoulou-Sellin R, Quintos MT, Woods D, Needle M, Klein MJ. Short stature in children and adults with neurofihromatosis. Pediatr Nurs 1995;21:149-53. 5. Brauner R, Malandry F, Rappaport R, et al. Growth and endocrine disorders in optic glioma. Eur J Pediatr 1990;149:825-8.