RESUMO
Endocrine evaluations were performed prospectively in 22 patients with medulloblastoma (ages 2 1/2 to 23 1/2 years at diagnosis), after craniospinal radiation with or without adjuvant chemotherapy. The mean craniospinal hypothalamic-pituitary). and thyroid radiation doses were 3600 and 2400 rads, respectively. Fourteen (73%) of 19 patients who had not yet completed their growth experienced a decrease in growth velocity. However, only three of 10 of these children, who underwent growth hormone stimulation tests, had evidence of deficient growth hormone responses, suggesting that growth hormone secretory or regulatory dysfunction, rather than absolute growth hormone deficiency, is present in the majority of these children. Elevated thyroid-stimulating hormone levels were noted in 15 of 22 patients; one patient had hypothalamic hypothyroidism. Thus, the late effects of therapy for medulloblastoma include frequent endocrine morbidity involving hypothalamic-pituitary and thyroid dysfunction.
Assuntos
Neoplasias Cerebelares/radioterapia , Transtornos do Crescimento/etiologia , Sistema Hipotálamo-Hipofisário/efeitos da radiação , Meduloblastoma/radioterapia , Glândula Tireoide/efeitos da radiação , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Cerebelares/tratamento farmacológico , Criança , Pré-Escolar , Terapia Combinada , Feminino , Hormônio do Crescimento/metabolismo , Humanos , Masculino , Meduloblastoma/tratamento farmacológico , Estudos Prospectivos , Doses de Radiação , Risco , Testes de Função Tireóidea , Glândula Tireoide/fisiopatologia , Fatores de TempoAssuntos
Aldosterona/metabolismo , Fibrose Cística/diagnóstico , Renina/metabolismo , Cloretos/sangue , Fibrose Cística/metabolismo , Diagnóstico Diferencial , Eletrólitos/sangue , Humanos , Hiperaldosteronismo/etiologia , Hiponatremia/etiologia , Lactente , Recém-Nascido , Masculino , Potássio/sangue , Sódio/sangueRESUMO
We have found bioassayable somatomedin activity to be subnormal in 20 of 32 children and adults with beta-thalassemia. The levels were comparable to values reported in growth hormone-deficient subjects. Since patients with thalassemia are not growth hormone deficient, the data suggest the possibility of defective hepatic biosynthesis of somatomedin. Increased iron stores in these patients, who have secondary hemosiderosis of many organs, including the liver, may depress somatomedin activity. Therapy for one year with daily subcutaneous infusions of the iron-chelating agent deferoxamine had no effect on mean bioassayable serum somatomedin activity.