ESPE Abstracts

Introduction: Turner Syndrom (TS) is one of the most common chromosomic abnormalities. It associates almost constantly an ovarian dysgenesis and a growth delay (GD). The GD is often secondary to a peripheral resistance to the growth hormone (GH). The GH deficiency (GHD) is a rare cause of growth delay, the association between these two condition is even rarer.

Patients and methods: We describe here the cases of 4 patients, seen in our consultation for growth delay and delayed puberty. The mean age was 13 years, the mean height was 124 cm (- 6 SD in 1 patient, -3SD in 2 patients and -2SD in 3 patients). The mean bone age : 9 years. The dysmorphic syndrom was present in 3 patients. Biological assessment: Low IGF1 in the 4 patients, positive anti TPO antibodies without thyroïd dysfunction in 1 case. All the other investigation were normal. The Avlocardyl-Glucagon and insulinic hypoglycemia tests, showed an isolated GHD in the 4 patients. (GH peak < 7ng/ml) Pituitary MRI: Pituitaty hypoplasia in 2 patients. GH treatment was conducted in the 4 patients, although a poor response was noted. (The mean height gain 04 cm). The caryotype was done one year after the treatment initiation: 3 cases monosomy, and one case mosaïc.

Conclusion: Turner Syndrom must be evoked in every girl wih growth delay. In our series GHD was searched before the establishement of the TS diagnosis, due to the caryotype unavailability.