Endocrine Abstracts

JOINT881

Introduction: Empty Sella Syndrome (ESS) is a rare condition in children, often associated with growth hormone (GH) deficiency. We present two pediatric cases of ESS diagnosed with GH deficiency who demonstrated significant growth improvement after receiving recombinant GH (rGH) therapy. Both patients were transitioned to weekly GH therapy at some point through their treatment, showing sustained positive outcomes.

Methods: Case 1 involves a 12-year and 6-month-old male who presented with short stature and rapid decline in height velocity, showing a growth rate of 2.3 cm/year. Medical and birth history were unremarkable. His height was 138.5 cm (≤2nd percentile), and he was in Tanner stage 2-3, with a testicular volume of 7 ml. GH stimulation tests revealed peak GH levels of 6.8 ng/ml (glucagon) and 7.6 ng/ml (L-Dopa), with an IGF-1 of 182 ng/ml. MRI confirmed ESS. GH treatment was started, and the results are shown in table 1. Case 2 involved a 12-year and 10-month-old male referred due to short stature. His height was 140 cm (3rd percentile), weight was 29 kg, and height velocity was 3.1 cm/year. He had a 2-year history of well-managed hypothyroidism, and unremarkable birth history. GH stimulation tests revealed peak GH levels of 6.6 ng/ml (glucagon) and 6.0 ng/ml (L-Dopa). MRI confirmed ESS. GH treatment was started, and the results are shown in table 1.

Results: Both patients showed significant growth improvement with rGH. In Case 1, height increased by 13 cm in 18 months. In Case 2, height increased by 26 cm in 2 years and 7 months. Transitioning to weekly GH therapy midway through treatment maintained sustained or growth improvements in both cases. Interestingly, case 2 benefited more from the weekly GH treatment due to improved compliance.

Conclusion: These cases show the effectiveness of recombinant GH therapy in promoting significant growth in pediatric ESS patients with GH deficiency, with sustained or even improved benefits following the transition to weekly GH.