Two cases of XY dysgenesis are reported. In one case, that of a 13 year-old girl presenting with impuberism, a gonadoblastoma was detected by histologic examination of the streak. In the second case, that of a 6 month-old girl, short stature and dysplasia of the nails suggested the diagnosis.
Combined Glucagon-Propranolol test used for study of growth hormone is advantages. The combined administration of TRH and LHRH is possible. In 53 children, the hormone responses (GH, TSH, FSH, LH and prolactin) were studied. This combined test allows the rapid assessment of anterior pituitary function.
Three cases of septo-optic dysplasia are related in infants. A neurogenic diabetes insipidus and an central adrenocortical insufficiency is proved. An growth hormone deficiency is founded in one case. The other anterior pituitary functions are normal. The pneumo-encephalography with congenital absence of septum lucidum and the ophtalmologic anomalies are typical. The treatment is envisaged. In one case an autopsy sustains the radiologic aspect.
