CEDIE   05498
CENTRO DE INVESTIGACIONES ENDOCRINOLOGICAS "DR. CESAR BERGADA"
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Idiopatic GH Neurosecretory Dysfunction (GHNSD): A Possible Cause of GH Deficiency (GHD)
Autor/es:
FREIRE A; KESELMAN A; CHIESA A; ROPELATO G; BERGADÁ I
Lugar:
Lima , Perú
Reunión:
Congreso; XX REUNION ANUAL SOCIEDAD LATINOAMERICANA DE ENDOCRINOLOGIA PEDIATRICA; 2008
Institución organizadora:
SLEP
Resumen:
GHNSD was described by Spiliotis et al in 1984.Since then,this entity was reported in patients after cranial radiation therapy.However, the diagnosis of idiopathic GHNSD has been questioned. We describe 3 prepubertal patients aged 12,7.8 and 6.3 years with height of .4.2, .3.3 and .4.3 SDS,respectively and delay in bone age from 3 to 5 years.Target height was normal,without a history of constitutional delay.Psychosocial deprivation was included.Arginine-clonidine tests showed a maximum GH peak of 10.8,16.8 and 17.1 ng/ml,and serum IGF1 (SDS)levels were .4.64,.2.3 and .2.8 respectively. An increase of IGF-1 >60%in the GH generation tests excluded GH insensitivity.A twelve-hour 20 minutes sampling nocturnal pulsatile GH secretion was performed and compared with normal prepubertal controls (C). Spontaneous mean ±SD GH levels (ng/ml)were 0.69 ±0.3, 1.3 ±0.8 and 1.4 ±0.8 vs 4.4 ±1.4 in C.Pulse amplitude were 0.6 ±0.39,1.9 ±2.1 and 3.4 ±0.37 vs 8.5 ±4.3 in C.Max GH were 1.21,4.2 and 4.6 vs 18.2 ±9.5 in C.All these values were significantly lower than C,while the number of pulses were not. GH treatment at a mean dose of 0.23 mg/k/wk resulted in growth acceleration at year one from a baseline of 4.1,4.0,and 3.2 cm/yr,to 10.1,10.2 and 7.6 cm/yr,respectively. These data show that idiopatic GHNSD should be considered when other causes of GHD are excluded even without a history of cranial irradiation.The 12-hour spontaneous nocturnal GH sampling is an alternative test to delineate who might benefit from GH treatment.