RESUMO
We studied nine children who had received cranial irradiation for various malignancies and subsequently experienced decreased growth velocity. Their response to standard growth hormone stimulation and release tests were compared with that in seven children with classic GH deficiency and in 24 short normal control subjects. With arginine and L-dopa stimulation, six of nine patients who received radiation had a normal GH response (greater than 7 ng/ml), whereas by design none of the GH deficient and all of the normal children had a positive response. Only two of nine patients had a normal response to insulin hypoglycemia, with no significant differences in the mean maximal response of the radiation and the GH-deficient groups. Pulsatile secretion was not significantly different in the radiation and GH-deficient groups, but was different in the radiation and normal groups. All subjects in the GH-deficient and radiation groups were given human growth hormone for 1 year. Growth velocity increased in all, with no significant difference in the response of the two groups when comparing the z scores for growth velocity of each subject's bone age. We recommend a 6-month trial of hGH in children who have had cranial radiation and are in prolonged remission with a decreased growth velocity, as there is no completely reliable combination of GH stimulation or release tests to determine their response.
Assuntos
Encéfalo/efeitos da radiação , Transtornos do Crescimento/tratamento farmacológico , Hormônio do Crescimento/uso terapêutico , Lesões por Radiação/tratamento farmacológico , Adolescente , Neoplasias Encefálicas/radioterapia , Criança , Pré-Escolar , Feminino , Transtornos do Crescimento/etiologia , Transtornos do Crescimento/fisiopatologia , Hormônio do Crescimento/metabolismo , Humanos , Leucemia Linfoide/radioterapia , Masculino , Lesões por Radiação/fisiopatologiaRESUMO
Twenty patients with growth hormone deficiency ranging in age from 5 5/12 to 15 8/12 years were treated for 12 months with a combination of human growth hormone and oxandrolone, followed by a period of six months off both medications. Eight of the patients received the combined therapy during the first year of hGH treatment, and 12 during either the second or fourth years of hGH administration. In considering growth velocity alone, the addition of anabolic steroid was beneficial. The bone age advanced rapidly when oxandrolone was added during the first year of hGH treatment, and less rapidly in subsequent years. The increased growth velocity, however, compensated for the acceleration of bone maturation and the overall effect of the combined treatment was beneficial, particularly when used after the first year of hGH treatment. We conclude that there is no advantage to using oxandrolone during the first year of hGH therapy, that oxandrolone in the appropriate dose is of benefit in subsequent years of hGH treatment, and that because of the individual variation in bone maturation, bone age should be frequently assessed.
Assuntos
Transtornos do Crescimento/tratamento farmacológico , Hormônio do Crescimento/uso terapêutico , Oxandrolona/uso terapêutico , Adolescente , Determinação da Idade pelo Esqueleto , Estatura/efeitos dos fármacos , Criança , Pré-Escolar , Esquema de Medicação , Quimioterapia Combinada , Crescimento/efeitos dos fármacos , Hormônio do Crescimento/deficiência , Hormônio do Crescimento/farmacologia , Humanos , Oxandrolona/farmacologiaRESUMO
Reye syndrome in siblings was seen in three of 85 families; the incidence of RS in these family groups appears to exceed that of the general population. The interval between development of RS in the first and second siblings was two to 11 days and related to the incubation period of the initial viral infection. In five of the children this infection was chickenpox and in two, an unspecified upper respiratory illness. To assess the role of genetic factors, HLA typing was performed on these siblings; a common genetic marker indicating susceptibility to RS was not identified. All families resided in rural and suburban areas; exposure to a common environmental toxin was not identified.
Assuntos
Síndrome de Reye/genética , Adolescente , Biópsia , Varicela/complicações , Criança , Pré-Escolar , Feminino , Antígenos HLA , Humanos , Fígado/patologia , Masculino , Infecções Respiratórias/complicações , Síndrome de Reye/etiologia , Síndrome de Reye/patologia , Fatores de TempoRESUMO
Cerebral gigantism is a syndrome consisting of characteristic dysmorphic features, accelerated growth in early childhood, and variable degrees of mental retardation. Its etiology and pathogenesis have not been defined. Three families are presented with multiple affected members. The vertical transmission of the trait and equal expression in both sexes in these families indicates a genetic etiology with a dominant pattern of inheritance, probably autosomal. As in previously reported cases, extensive endocrine evaluation failed to define the pathogenesis of the accelerated growth present in this disorder.