RESUMEN
Between 1979 and 1983, 129 children (95 girls) with precocious puberty were referred to the National Institutes of Health and received treatment for at least 6 months with the long-acting LHRH analogue D-Trp6-Pro9-NEt-LHRH. The majority (107 of 129) of the children had central precocious puberty mediated by activation of the hypothalamic-pituitary-gonadal axis in association with hypothalamic hamartomas (24 of 107) or other central nervous system lesions (21 of 107), or idiopathic precocious puberty (62 of 107). Hypothalamic hamartomas or other central nervous system lesions were a frequent cause of central precocious puberty in girls (27 of 87), but idiopathic precocious puberty was still the most frequent diagnosis (63%). Idiopathic precocious puberty was uncommon in boys (6%). The patients with peripheral precocious puberty included six girls with McCune-Albright syndrome and six boys with familial male precocious puberty. These children had peripheral sex steroid secretion in the absence of hypothalamic-pituitary-gonadal axis maturation. The children with combined peripheral and central precocious puberty included nine children with congenital adrenal hyperplasia and one girl with a virilizing adrenal tumor. In the patients with central precocious puberty or combined peripheral and central precocious puberty, LHRHa therapy caused suppression of gonadotropin and sex steroid levels (P less than 0.001), stabilization or regression of secondary sexual characteristics, and decreases in growth rate and in the rate of bone age maturation (P less than 0.005). Patients with peripheral precocious puberty, however, had no significant change in gonadotropin or sex steroid levels, growth rate, or the rate of bone age maturation, and no improvement in secondary sexual characteristics. Thus, LHRHa is an effective treatment of central precocious puberty and combined peripheral and central precocious puberty, but is ineffective in the therapy of peripheral precocious puberty.
Asunto(s)
Hormona Liberadora de Gonadotropina/análogos & derivados , Pubertad Precoz/clasificación , Pubertad Precoz/tratamiento farmacológico , Pamoato de Triptorelina/análogos & derivados , Hiperplasia Suprarrenal Congénita/complicaciones , Niño , Preescolar , Preparaciones de Acción Retardada , Estradiol/sangre , Femenino , Displasia Fibrosa Poliostótica/complicaciones , Hormona Folículo Estimulante/sangre , Hormona Liberadora de Gonadotropina/administración & dosificación , Hormona Liberadora de Gonadotropina/uso terapéutico , Crecimiento , Hamartoma/complicaciones , Humanos , Neoplasias Hipotalámicas/complicaciones , Hormona Luteinizante/sangre , Masculino , National Institutes of Health (U.S.) , Pubertad Precoz/etiología , Caracteres Sexuales , Factores Sexuales , Testosterona/sangre , Estados UnidosRESUMEN
To assess the role of somatomedin-C as a possible mediator of the growth spurt in children with central precocious puberty, we compared Sm-C levels in 40 children with central precocious puberty, 87 age-matched normal children, and 110 normal pubertal controls. Somatomedin C levels were significantly elevated for age in the children with precocious puberty (P less than 0.01), and were similar to the levels observed during normal puberty. The patients with precocious puberty were given the luteinizing hormone releasing hormone analogue D-Trp6-Pro9-NEt-LHRH (LHRHa) for 6 months. Treatment caused a significant decrease in secondary sexual characteristics, growth rate, plasma gonadotropins, sex steroids (estradiol in the girls and testosterone in the boys), and Sm-C levels. Growth during LHRHa treatment returned to the age-appropriate rate, whereas plasma Sm-C levels, although lower than pretreatment levels, remained significantly elevated for age (P less than 0.002). In addition, growth rates before and during treatment did not correlate with the plasma somatomedin C levels, nor did the decreases in growth rate during LHRHa therapy correlate with the decreases in somatomedin C levels. Growth rates did correlate significantly, however, with plasma estradiol levels in the girls (P less than 0.0005) and with plasma testosterone levels in the boys (P less than 0.025). We conclude that the growth spurt in children with precocious puberty cannot be explained by the plasma level of somatomedin C.
Asunto(s)
Trastornos del Crecimiento/sangre , Pubertad Precoz/sangre , Somatomedinas/sangre , Niño , Preescolar , Estradiol/sangre , Femenino , Hormona Liberadora de Gonadotropina/uso terapéutico , Crecimiento/efectos de los fármacos , Trastornos del Crecimiento/etiología , Humanos , Lactante , Insulina/sangre , Factor I del Crecimiento Similar a la Insulina , Masculino , Péptidos/sangre , Pubertad Precoz/complicaciones , Pubertad Precoz/tratamiento farmacológico , Caracteres Sexuales , Testosterona/sangreAsunto(s)
Neoplasias de las Glándulas Suprarrenales/complicaciones , Hormona Liberadora de Gonadotropina/análogos & derivados , Pubertad Precoz/tratamiento farmacológico , Pamoato de Triptorelina/análogos & derivados , Virilismo/etiología , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Preescolar , Femenino , Hormona Liberadora de Gonadotropina/uso terapéutico , Humanos , Hormonas Hipofisarias/sangre , Pubertad Precoz/etiología , Virilismo/cirugíaRESUMEN
We report a controlled standardized behavioral assessment of 33 girls with true precocious puberty using the Child Behavior Checklist. Although a majority of the girls were reported not to have behavior problems, many were reported to have a dysphoric adjustment to their condition. Twenty-seven percent of the girls with true precocious puberty scored greater than 2 SD above the mean on the Total Behavior Problem scale 10 times the expected prevalence rate. They also scored significantly higher (P less than 0.01) than matched controls on both the internalizing or "overcontrolled symptom" and externalizing or "undercontrolled symptom" scales. Forty-eight percent scored greater than 2 SD above the mean on the Social Withdrawal scale. The high prevalence of reported problem behaviors in this sample may be related directly or indirectly to the precocious maturation mediated by biologic, psychologic, social, and environmental variables. Although elevated levels of sex steroids may directly contribute to increased aggressive and hyperactive behaviors, they may also be modified by social and environmental factors.