RESUMO
The authors describe the case of a 18-year-old man with short stature, epilepsy, mental deficiency and basal ganglia and central nervous system calcifications. The clinical and laboratorial findings have suggested pseudohypoparathyroidism which is a rare pathology with a peripheral resistance to parathormone, neuromuscular hyperexcitability, short stature and various clinical findings. This paper reviews the clinical form and treatment of pseudohypoparathyroidism and the neuroradiologic aspects of calcifications.
Assuntos
Pseudo-Hipoparatireoidismo/diagnóstico , Adolescente , Anticonvulsivantes/uso terapêutico , Doenças dos Gânglios da Base/diagnóstico por imagem , Doenças dos Gânglios da Base/tratamento farmacológico , Calcinose/diagnóstico por imagem , Calcinose/tratamento farmacológico , Epilepsia/tratamento farmacológico , Humanos , Masculino , Fenobarbital/uso terapêutico , Pseudo-Hipoparatireoidismo/tratamento farmacológico , Tomografia Computadorizada por Raios XRESUMO
1. The role of testosterone (T) in growth was evaluated in 11 prepubertal hypopituitary males during two 15-day periods separated by a 4-week interval, i.e., before (PRE-T period) and during T ester treatment (50 mg every 5 days, 3 im doses-T period). 2. T increased growth hormone (GH) secretion, assessed by 4-h rhythm (mean +/- SEM = 1.90 +/- 0.27 vs 1.77 +/- 0.21 ng/ml; P < 0.05) and after a GHRH stimulus (3.42 +/- 0.54 vs 3.08 +/- 0.43 ng/ml; P < 0.05) as compared to the PRE-T period. 3. T also increased basal somatomedin-C (SM-C) levels (0.20 +/- 0.03 vs 0.15 +/- 0.02 U/ml; P < 0.001) and SM-C generation. After GH was administered in 4 im doses (0.01, 0.02, 0.05 and 0.1 U/kg), SM-C levels were 0.31 +/- 0.08 vs 0.24 +/- 0.07 U/ml, P < 0.001. T did not change incremental (absolute minus basal) SM-C levels (0.15 +/- 0.08 vs 0.12 +/- 0.07 U/ml; P > 0.05). 4. The results suggest that T increased plasma SM-C levels by stimulating residual GH secretion in hypopituitary males.
Assuntos
Hormônio do Crescimento/efeitos dos fármacos , Hormônio do Crescimento/deficiência , Hormônio do Crescimento/metabolismo , Fator de Crescimento Insulin-Like I/efeitos dos fármacos , Puberdade/efeitos dos fármacos , Testosterona/uso terapêutico , Adolescente , Adulto , Análise de Variância , Criança , Hormônio do Crescimento/sangue , Humanos , Hipopituitarismo/sangue , Hipopituitarismo/tratamento farmacológico , Hipopituitarismo/epidemiologia , Fator de Crescimento Insulin-Like I/análise , Masculino , Puberdade/sangue , Fatores de TempoRESUMO
1. The role of testosterone (T) in growth was evaluated in 11 prepubertal hypopituitary males during two 15-day periods separated by a 4-week interval, i.e., before (PRE-T period) and during T ester treatment (50 mg every 5 days, 3 im doses-T period). 2. T increased growth hormone (GH) secretion, assessed by 4-h rhythm (mean +/- SEM = 1.90 +/- 0.27 vs 1.77 +/- 0.21 ng/ml; P < 0.05) and after a GHRH stimulus (3.42 +/- 0.54 vs 3.08 +/- 0.43 ng/ml; P < 0.05) as compared to the PRE-T period. 3. T also increased basal somatomedin-C (SM-C) levels (0.20 +/- 0.03 vs 0.15 +/- 0.02 U/ml; P < 0.001) and SM-C generation. After GH was administered in 4 im doses (0.01, 0.02, 0.05 and 0.1 U/kg), SM-C levels were 0.31 +/- 0.08 vs 0.24 +/- 0.07 U/ml, P < 0.001. T did not change incremental (absolute minus basal) SM-C levels (0.15 +/- 0.08 vs 0.12 +/- 0.07 U/ml; P > 0.05). 4. The results suggest that T increased plasma SM-C levels by stimulating residual GH secretion in hypopituitary males